{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[0, 193]], "word_ranges": [[0, 30]], "text": "Although other hemorrhagic diseases can have a prolonged thromboplastin time, due to the intensity of the lesion and the child's sex and family history, the most likely diagnosis is hemophilia."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Although other hemorrhagic diseases can have a prolonged thromboplastin time, due to the intensity of the lesion and the child's sex and family history, the most likely diagnosis is hemophilia.", "full_answer_no_ref": "Although other hemorrhagic diseases can have a prolonged thromboplastin time, due to the intensity of the lesion and the child's sex and family history, the most likely diagnosis is hemophilia.", "full_question": "An 18-month-old boy, with complete immunization schedule to date, who consults the Emergency Department for right knee swelling after playing in the park, without obvious trauma. In the directed anamnesis, the mother refers that an uncle of hers had similar problems. The ultrasound examination is compatible with hemarthrosis and in the analytical analysis only an APTT lengthening of 52'' (normal 25-35'') stands out. What is the most probable diagnostic hypothesis?", "id": 190, "lang": "en", "options": {"1": "Marfan syndrome.", "2": "Von-Willebrand's disease.", "3": "Ehlers-Danlos disease.", "4": "Hemophilia A.", "5": "Bernard-Soulier disease."}, "question_id_specific": 94, "type": "PEDIATRICS", "year": 2013, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0052_9437", "title": "Further evidence for recessive inheritance of von Willebrand disease with abnormal binding of von Willebrand factor to factor VIII.", "score": 0.01852010631912887, "content": "A new family with a bleeding diathesis and FVIII deficiency secondary to abnormal binding of von Willebrand factor (vWF) to factor VIII (FVIII) is described. Two propositi of this family, an 18-year-old male and a 33-year-old female, both with a history of epistaxis, bruising, bleeding from the gums, epistaxis, hemarthrosis, and hematoma, were analyzed. Also additional members of the same family with no bleeding history were also studied. The propositi showed normal vWF activities, low FVIII activity; one of them had been diagnosed as having hemophilia A and the other was a hemophilia A carrier. Both showed a very poor response to treatment with FVIII concentrates and desmopressin (DDAVP) but a good clinical response to cryoprecipitate. APTT was prolonged and no inhibitory activity was noticeable in their plasmas. Thirty-five units per kilogram body weight of Hemofil M was infused to both propositi and FVIII reached basal level within 60 minutes of the infusion. No FVIII response at all was observed in the female after intravenous DDAVP administration. However, the male who received the infusion of 35 U/kg body weight of Humate-P achieved a normal FVIII level that was maintained for 12 hours. Multimeric analysis of vWF was normal in all the members studied. Von Willebrand factor domain for FVIII binding was assayed in the two propositi and in six other members of the same family by using a non-isotopic and sensitive method, a modification of the one previously described, using the Hemofil M concentrate as exogenous FVIII. The data obtained showed that both propositi had similar binding to that observed by using plasma of a patient with severe von Willebrand disease. Furthermore, five siblings had a decreased binding of vWF to FVIII, when compared with plasma from normal individuals or patients with hemophilia A. We also observed that, for screening purpose, the ratio of bound FVIII/immobilized vWF (at saturation of the anti-vWF and offering of 1 U/ml of exogenous FVIII) distinguished two levels of abnormality (normal range 0.70-1.15, propositi 0.004-0.007, and remaining members affected 0.25-0.42). The most probable explanation is that the propositi are homozygous or double heterozygous, the other five siblings affected being heterozygous for a recessive vWF defect. This more accessible assay presented here may be of help in routine analysis for diagnosing this type of von Willebrand disease, which has important implications for therapy and genetic counseling."}, {"id": "pubmed23n1089_3373", "title": "Diagnostic Challenges in Children With Congenital Bleeding Disorders: A Developing Country Perspective.", "score": 0.017704661182922053, "content": "To assess the frequency and characteristics of children with inherited bleeding disorders that were initially misdiagnosed, leading to inappropriate disease management. This study was conducted at the Haematology/Pathology Department of Fauji Foundation Hospital, Rawalpindi, Pakistan, from August 2014 to August 2018. Children who were diagnosed with an inherited bleeding disorder but did not respond to initial therapy were reevaluated. In total, 62 children were diagnosed with a bleeding disorder. Of these, 27 were diagnosed with an inherited bleeding disorder and 35 with an acquired bleeding disorder. Of the 27 children with inherited bleeding disorders, 18% (n = 5) were misdiagnosed and treated inappropriately. The median age of the misdiagnosed patients was 9 years (range, 5-13 years). Three patients with Bernard-Soulier syndrome had been misdiagnosed as having immune thrombocytopenic purpura, 1 patient with von Willebrand disease had been misdiagnosed as having hemophilia A, and 1 patient with haemophilia B had been misdiagnosed as having hemophilia A. There are chances of misdiagnosis and improper or invasive management if comprehensive laboratory evaluation and a thorough clinical evaluation are not performed in children with congenital bleeding disorders."}, {"id": "pubmed23n0596_19559", "title": "The dental patient with a congenital bleeding disorder.", "score": 0.017392619479733817, "content": "Congenital bleeding disorders account for approximately one in 10,000 births. Dentists are often anxious about delivering treatment to this special group of patients. In the Irish Republic, patients with inherited bleeding disorders have their dental care co-ordinated centrally at the National Centre for Hereditary Coagulation Disorders (NCHCD), St James's Hospital, Dublin. Dental care is normally integrated with routine outpatient haematological appointments. This ensures regular monitoring of oral health and the early treatment of any hard/soft tissue pathology. This article describes, in simple diagrammatic form, the normal coagulation mechanism (Figures 1 and 2), explains common coagulation terms (Appendix 1), and examines the three most common congenital bleeding disorders: haemophilia A, haemophilia B, and von Willebrand disease. General recommendations based on the current literature are provided with respect to procedures that are appropriate to perform in a general dental practice setting. Although not discussed in this article, it is important to note that non-coagulation bleeding disorders also exist. These include: hereditary haemorrhagic telangiectasia; blood vessel wall defects resulting from connective tissue disorders such as Marfan syndrome and Ehlers-Danlos syndrome; and, platelet disorders such as Bernard-Soulier syndrome, resulting in defective platelet adhesion."}, {"id": "pubmed23n0780_13321", "title": "Screening bleeding disorders in adolescents and young women with menorrhagia.", "score": 0.015617875056999544, "content": "Chronic menorrhagia causes anemia and impairment of life quality. In this study the aim was the screening of bleeding disorders in adolescents and young women with menorrhagia. The study was performed prospectively by pediatric hematologists. A form including demographic characteristics of the patients, bleedings other than menorrhagia, familial bleeding history, characteristics of the menorrhagia, and impairment of life quality due to menorrhagia was filled out by the researcher during a face-to-face interview with the patient. A pictorial blood assessment chart was also used for evaluation of blood loss. All patients underwent pelvic ultrasound sonography testing and women also received pelvic examination by gynecologists. Whole blood count, peripheral blood smear, blood group, serum transaminases, urea, creatinine, ferritin, PFA-100, PT, aPTT, INR, TT, fibrinogen, VWF:Ag, VWF:RCo, FVIII, and platelet aggregation assays were performed. Platelet aggregations were studied by lumiaggregometer. Out of 75 patients enrolled, 60 patients completed the study. The mean age was 20.68±10.34 (range: 10-48) years and 65% (n=39) of the patients were younger than 18 years. In 18 (46%) of the adolescents, menorrhagia subsided spontaneously. In 20% (n=12) of the patients, a bleeding disorder was detected (1 case of type 3 von Willebrand disease, 2 patients with low VWF:Ag, 1 case of probable von Willebrand disease, 3 cases of Bernard-Soulier syndrome, 2 cases of Glanzmann thrombasthenia, 2 cases of immune thrombocytopenic purpura, 1 case of congenital factor VII deficiency). In patients with menorrhagia, at least complete blood count, peripheral smear, aPTT, PT, VWF:Ag, VWF:RCo, FVIII, and fibrinogen assays must be performed. When there is history of nose and gum bleeding, platelet function assay by lumiaggregometer must also be performed. In nearly 50% of adolescents, menorrhagia is dysfunctional and transient. Detailed coagulation assays can be postponed in adolescents if bleeding history other than menorrhagia and/or family history of bleeding and/or parental consanguinity is absent. All subjects with menorrhagia must consult with gynecologists and hematologists. None declared."}, {"id": "pubmed23n0083_20904", "title": "Ehlers-Danlos syndrome, clotting disorders and muscular dystrophy.", "score": 0.015548567435359888, "content": "Ehlers-Danlos syndrome includes 11 distinct entities. The diversity of this collagen dysplasia and its combination with other abnormalities make it difficult to understand physiopathologically. A case of Ehlers-Danlos syndrome is reported, which is novel owing to its combination with clotting abnormalities and especially with muscular dystrophy. To our knowledge this has not previously been reported. The patient was a young man aged 16 years who presented with Ehlers-Danlos syndrome satisfying Perelman's diagnostic criteria. His father and two brothers had comparable clinical symptoms, but his mother and sister were healthy. The four male subjects had an increased cephalin-kaolin time, reduced levels of factor VIII and Willebrand's factor (but without haemophilia A or Willebrand's disease), and, especially, an abnormal platelet ATP secretion. The proband alone had muscular disease with bilateral quadriceps fatigability and amyotrophy. The muscle enzyme levels were greatly increased, the electromyographic trace was myogenic, and the biopsy showed severe muscular dystrophy. This new observation poses the problem of the relation between clotting abnormalities and collagen abnormalities in the Ehlers-Danlos syndrome. It is difficult to classify this case within any of the 11 known types because of its muscular manifestations. It may perhaps be a fortuitous combination or an extension of the nosological framework of this syndrome."}, {"id": "wiki20220301en081_52264", "title": "Bernard–Soulier syndrome", "score": 0.015040515040515042, "content": "Differential diagnosis The differential diagnosis for Bernard–Soulier syndrome includes both Glanzmann thrombasthenia and pediatric Von Willebrand disease. BSS platelets do not aggregate to ristocetin, and this defect is not corrected by the addition of normal plasma, distinguishing it from von Willebrand disease. Following is a table comparing its result with other platelet aggregation disorders: Treatment Bleeding events can be controlled by platelet transfusion. Most heterozygotes, with few exceptions, do not have a bleeding diathesis. BSS presents as a bleeding disorder due to the inability of platelets to bind and aggregate at sites of vascular endothelial injury. In the event of an individual with mucosal bleeding tranexamic acid can be given. The affected individual may need to avoid contact sports and medications such as aspirin, which can increase the possibility of bleeding. A potential complication is the possibility of the individual producing anti-platelet antibodies."}, {"id": "wiki20220301en071_25146", "title": "Erik Adolf von Willebrand", "score": 0.013859020310633213, "content": "He published a Swedish-language article in 1926 about the disease, titled Hereditär pseudohemofili (\"Hereditary pseudohemophilia\"). He referenced six previous publications from the years of 1876 to 1922, totalling 19 cases on families with bleeding diatheses. The earlier authors attributed the condition to hemophilia (even in the cases of females) or to thrombopathy, which was discovered shortly before as the cause of what had previously been known as purpura hemorrhagica or Werlhof's disease. Von Willebrand also conducted hematological examinations on Hjördis and some of her family members. He recorded a normal or slightly reduced number of platelets and an undisturbed clot retraction, unlike Glanzmann's thrombasthenia. The bleeding time (Duke) was greatly prolonged, extending to more than 2 hours in some cases, while the clotting time was within the normal range. He concluded that the disease was either a new form of thrombopathy or a condition of the capillary endothelium."}, {"id": "wiki20220301en100_5603", "title": "List of MeSH codes (C15)", "score": 0.013703271467831193, "content": "– hemorrhagic disorders – afibrinogenemia – bernard-soulier syndrome – disseminated intravascular coagulation – factor v deficiency – factor vii deficiency – factor x deficiency – factor xi deficiency – factor xii deficiency – factor xiii deficiency – hemophilia a – hemophilia b – hypoprothrombinemias – platelet storage pool deficiency – hermanski-pudlak syndrome – purpura, thrombocytopenic, idiopathic – thrombasthenia – thrombocythemia, hemorrhagic – vascular hemostatic disorders – cryoglobulinemia – ehlers-danlos syndrome – hemangioma, cavernous – hemangioma, cavernous, central nervous system – multiple myeloma – pseudoxanthoma elasticum – purpura, hyperglobulinemic – purpura, schoenlein-henoch – scurvy – shwartzman phenomenon – telangiectasia, hereditary hemorrhagic – waldenstrom macroglobulinemia – vitamin k deficiency – hemorrhagic disease of newborn – von willebrand disease – waterhouse-friderichsen syndrome"}, {"id": "pubmed23n0703_24426", "title": "Rare and unusual bleeding manifestations in congenital bleeding disorders: an annotated review.", "score": 0.012926108374384238, "content": "Epistaxis, superficial and deep hematomas, hemarthrosis, gastrointestinal bleeding, hematuria represent the most frequent hemorrhagic events in congenital coagulation disorders. Occasionally, bleeding manifestations occur in unusual sites or are peculiar. A clotting defect may alter the clinical aspect of skin conditions or infections (hemorrhagic scabies or varicella). Hemobilia may occur as a complication of transjugular liver biopsy in hemophilia or Bernard-Soulier syndrome. Hemarthrosis of small joints of feet and hands occur in patients with hemophilia treated with protease inhibitors. Intramedullary hematomas of long bones have been described in α2-plasmin inhibitor or fibrinogen deficiencies. Spleen fracture with consequent hemoperitoneum has been reported in patients with fibrinogen deficiency. Rectus muscle sheath hematoma may occur in patients with factor VII (FVII)or FX deficiency. Acute or subacute intestinal obstruction may be caused by intramural wall hematomas in hemophilia and von Willebrand (vW)-disease. Physicians should always keep in mind that a congenital hemorrhagic disorder may cause bleeding in any tissue of the body and therefore alter the normal clinical features of a given disease."}, {"id": "article-31270_27", "title": "Von Willebrand Disease -- Differential Diagnosis", "score": 0.012883358471593767, "content": "Factor X deficiency Factor XI deficiency Hemophilia A Hemophilia B Bernard-Soulier syndrome Platelet function defects Antiplatelet drug ingestion Fibrinolytic defects"}, {"id": "pubmed23n0637_1860", "title": "Spectrum of inherited bleeding disorders in southern Iran, before and after the establishment of comprehensive coagulation laboratory.", "score": 0.012847884102173085, "content": "The objective of the present study was to determine the pattern of inherited bleeding disorders in southern Iran and evaluate the effect of a comprehensive coagulation laboratory and related efforts. A total of 545 patients with inherited bleeding disorders were evaluated during 1992-2007 by a cross-sectional study. Data were collected by a data-gathering form. Statistical analysis was done using Statistical Package for the Social Sciences version 15. A P value less than 0.05 was considered statistically significant. Overall 411 patients had common bleeding disorders including 326 hemophilia A, 46 hemophilia B, and 39 von Willebrand disease. Seventy-nine patients had rare coagulation disorders including deficiency of factor VII (n = 26), factor X (n = 18), factor XIII (n = 9), factor I (n = 9), factor XI (n = 7), factor V (n = 4), combined factor VIII and factor V (n = 4), and combined factor X and factor VII (n = 2). Fifty-five patients had platelet disorders including 23 with Glanzmann's thrombasthenia, 15 with Bernard-Soulier syndrome, and 17 with other platelet disorders, most of which (45) were diagnosed after the establishment of the comprehensive coagulation laboratory. Annual mean number of new diagnosed patients with common and rare bleeding disorders increased from 29 +/- 4 to 38 +/- 17. The ratio of the patients diagnosed with rare bleeding disorders to common bleeding disorders significantly increased after the establishment of the comprehensive diagnosis laboratory (P &lt; 0.001).It seems that implementation of collaborative projects by the Shiraz Hemophilia Society and the establishment of the comprehensive coagulation laboratory and treatment centers have been successful in increasing diagnosis of the inherited bleeding disorders and consequently better management of the patients."}, {"id": "wiki20220301en100_5598", "title": "List of MeSH codes (C15)", "score": 0.012756221400363567, "content": "– blood coagulation disorders – coagulation protein disorders – activated protein c resistance – afibrinogenemia – factor v deficiency – factor vii deficiency – factor x deficiency – factor xi deficiency – factor xii deficiency – factor xiii deficiency – hemophilia a – hemophilia b – hypoprothrombinemias – von willebrand disease – disseminated intravascular coagulation – blood coagulation disorders, inherited – activated protein c resistance – afibrinogenemia – antithrombin iii deficiency – bernard-soulier syndrome – factor v deficiency – factor vii deficiency – factor x deficiency – factor xi deficiency – factor xii deficiency – factor xiii deficiency – hemophilia a – hemophilia b – hermanski-pudlak syndrome – hypoprothrombinemias – protein c deficiency – thrombasthenia – von willebrand disease – wiskott-aldrich syndrome – platelet storage pool deficiency – hermanski-pudlak syndrome – protein s deficiency"}, {"id": "wiki20220301en014_34439", "title": "Von Willebrand disease", "score": 0.012316715542521993, "content": "Diagnosis Basic tests performed in any patient with bleeding problems are a complete blood count-CBC (especially platelet counts), activated partial thromboplastin time-APTT, prothrombin time with International Normalized Ratio-PTINR, thrombin time-TT, and fibrinogen level. Patients with abnormal tests typically undergo further testing for hemophilias. Other coagulation factor assays may be performed depending on the results of a coagulation screen. Patients with von Willebrand disease typically display a normal prothrombin time and a variable prolongation of partial thromboplastin time."}, {"id": "pubmed23n0684_10587", "title": "Congenital bleeding disorders in Karachi, Pakistan.", "score": 0.011854657687991021, "content": "To determine the frequency of inherited bleeding disorders, its complications, and treatment modalities available for its treatment. Cross-sectional study. Patients with a history of bleeding tendency were tested for confirmation of the diagnosis. History and clinical findings were recorded. Laboratory analysis included prothrombin time (PT), activated partial thromboplastin time (APTT), bleeding time (BT), and fibrinogen assay. Patients with prolonged APTT were tested for factors VIII (FVIII) and IX (FIX). If FVIII was low, von Willebrand factor: antigen (vWF:Ag) and von Willebrand factor:ristocetin cofactor (vWF:RCo) were performed. When PT and APTT both were prolonged, FV, FX, and FII were tested. Platelet aggregation studies were done when there was isolated prolonged BT. Urea clot solubility test was done when all coagulation tests were normal. All patients with hemophilia A and B were evaluated for inhibitors. Of the 376 patients, inherited bleeding disorder was diagnosed in 318 (85%) cases. Median age of patients was 16.4 years. Hemophilia A was the commonest inherited bleeding disorder that was observed in 140 (37.2%) followed by vWD 68 (18.0%), platelet function disorders 48 (12.8%), and hemophilia B in 33 (8.8%) cases. We also found rare congenital factor deficiencies in 13 (3.4%), low VWF in 11 (3.0%) participants and 5 (1.3%) in female hemophilia carriers. Hemarthrosis was the most frequent symptom in hemophilia A and B (79.7%) involving knee joint. Inhibitor was detected in 21 (15%) cases. Fresh frozen plasma/cryoprecipitate were the most common modality of treatment. In 58 patients, no abnormality was detected in coagulation profile. Hemophilia A and vWD are the most common congenital bleeding disorders in this study. Hemarthrosis involving knee joint was the most common complication. Inhibitor was detected in a significant number of patients. Plasma is still the most common modality of treatment."}, {"id": "pubmed23n0862_14626", "title": "Clinical profile of hemophilia patients in Jodhpur Region.", "score": 0.011667345000678335, "content": "Hemophilia is widely distributed all over the world, but little is known about its clinical profile in resource-limited regions. An insight into its clinical spectrum will help in the formulation of policies to improve the situation in these areas. To study the clinical profile of hemophiliacs (age &lt;18 years) in Jodhpur region and screen them for transfusion-transmitted infections. A cross-sectional study conducted in the Department of Pediatrics, Umaid Hospital, Dr. S. N. Medical College, Jodhpur, over a period of 12 months. Out of a total of 56 cases enrolled, 51 (91%) cases were diagnosed as hemophilia A while 5 (9%) were diagnosed as hemophilia B. Positive family history was found in 26 (46%) cases. According to their factor levels, 25 (44%) cases had severe disease, 20 (36%) had moderate disease, and 11 (20%) had mild disease. The mean age of onset of symptoms and diagnosis was 1.73 ± 1.43 and 3.87 ± 3.84 years, respectively. First clinical presentation was posttraumatic bleed in 20 (36%), gum bleeds in 17 (30%), epistaxis in 4 (7%), joint bleeds in 4 (7%), skin bleeds in 4 (7%), and circumcision bleed in 3 (5%) cases. Knee joint was the predominant joint affected by hemarthrosis in 38 (68%), followed by ankle in 29 (52%), elbow in 20 (36%), and hip joint in 7 (13%) cases. All patients had a negative screening test for transfusion-transmitted infections. Occurrence of posttraumatic bleeds and gum bleeds in an otherwise normal child should warn the clinician for evaluation of hemophilia."}, {"id": "article-22743_12", "title": "Hemophilia A -- Differential Diagnosis", "score": 0.011639607299230371, "content": "Acquired hemophilia Ehlers-Danlos syndrome Factor XI deficiency Glanzmann thrombasthenia Haemophilia C Haemophilia type B Physical child abuse Platelet disorders Von Willebrand disease"}, {"id": "wiki20220301en011_69738", "title": "Bleeding time", "score": 0.011267006802721087, "content": "Normal values fall between 3 – 10 minutes depending on the method used. A disadvantage of Ivy's method is closure of puncture wound before stoppage of bleeding. Duke's method With the Duke;s method, the patient is pricked with a special needle or lancet, preferably on the earlobe or fingertip, after having been swabbed with alcohol. The prick is about 3–4 mm deep. The patient then wipes the blood every 30 seconds with a filter paper. The test ceases when bleeding ceases. The usual time is about 2–5 minutes. This method is not recommended and cannot be standardized because it can cause a large local hematoma. Interpretation Bleeding time is affected by platelet function, certain vascular disorders and von Willebrand Disease—not by other coagulation factors such as haemophilia. Diseases that cause prolonged bleeding time include thrombocytopenia, disseminated intravascular coagulation (DIC), Bernard-Soulier disease, and Glanzmann's thrombasthenia."}, {"id": "pubmed23n0726_15808", "title": "A longitudinal prospective study of bleeding diathesis in Egyptian pediatric patients:  single-center experience.", "score": 0.011224489795918367, "content": "Keeping an updated registry of bleeding disorders is crucial for planning care and documenting prevalence. We aimed to assess the prevalence of various bleeding disorders including rare inherited coagulation and platelet disorders concerning their clinico-epidemiological, diagnostic data and bleeding manifestations severity. Patients suffering from manifestations of bleeding or coagulation disorders presented to Hematology Clinic during 16 years were included and prospectively followed up. Demographics, clinical characteristics, complete blood count, bleeding, prothrombin and activated partial thromboplastin times, platelet aggregation tests and bone marrow aspiration were recorded. Overall 687 patients with bleeding disorders from total 2949 patients were identified. Inherited coagulation defects were found in 27.2%; hemophilia A (70.6%), hemophilia B (13.9%), factor I deficiency (2.3%), factor V deficiency (1.6%), factor X deficiency (4.2%), factor VII deficiency (2.6%), factor XIII deficiency (1.1%), combined factor deficiency (2.1%) and unclassified coagulation disorders in 1.6% of studied patients. Overall 72.7% had diagnosed with platelet disorders; immune thrombocytopenia was the commonest (74.8%), and inherited conditions represent (25.2%) in the following order: Glanzman's thrombasthenia (11.2%), von Willebrand disease (6.6%), Bernard-Soulier syndrome (1%) and Chediak Higashi in 0.4% and unclassified in 6%. Median age of diagnosis of coagulation and platelet disorders were 33 and 72 months. Presenting symptoms of coagulation disorders were: 25.1% post circumcision bleeding, 22.5% ecchymosis, 20.9% hemoarthrosis and 15% epistaxis. Symptoms of rare coagulation disorders were postcircumcision bleeding (20%), bleeding umbilical stump (20%), epistaxis (12%), hemoarthrosis (8%) and hematomas (4%). Presenting symptoms in rare inherited platelet disorders were purpura, ecchymosis, epistaxis and bleeding gums, respectively. Analysis of the clinico-epidemiological data of patients with bleeding disorders is a useful tool for monitoring and improving their quality of care."}, {"id": "wiki20220301en026_78760", "title": "Bleeding diathesis", "score": 0.01085003144936652, "content": "Causes other than coagulation Bleeding diathesis may also be caused by impaired wound healing (as in scurvy), or by thinning of the skin, such as in Cushing's syndrome. Genetic Some people lack genes that typically produce the protein coagulation factors that allow normal clotting. Various types of hemophilia and von Willebrand disease are the major genetic disorders associated with coagulopathy. Rare examples are Bernard–Soulier syndrome, Wiskott–Aldrich syndrome and Glanzmann's thrombasthenia. Gene therapy treatments may be a solution as they involve in the insertion of normal genes to replace defective genes causing for the genetic disorder. Gene therapy is a source of active research that hold promise for the future. Diagnosis Comparing coagulation tests"}, {"id": "article-22748_37", "title": "Hemophilia -- Differential Diagnosis", "score": 0.010722125288456126, "content": "Other conditions can also present similarly with bleeding after minor trauma or spontaneous bleeds and require exclusion before confirming the diagnosis of hemophilia. Some of these conditions include von Willebrand disease, scurvy, diseases of platelet dysfunction, deficiency of other coagulation factors like V, VII, X, or fibrinogen, Ehlers-Danlos syndrome, Fabry disease, disseminated intravascular coagulation, and child abuse. In von Willebrand disease, bleeding symptoms can be similar to mild hemophilia, but patients with von Willebrand disease have more mucosal bleeding compared to musculoskeletal bleeding seen in hemophilia. Von Willebrand disease is diagnosed by checking for von Willebrand factor antigen or von Willebrand factor multimers. [40] Similarly, in scurvy, Ehlers-Danlos syndrome, and Fabry disease; also, the bleeding is usually mucosal, unlike hemophilia, where it is musculoskeletal. In scurvy, there is a deficiency of vitamin C. [41] In Ehlers-Danlos syndrome, the skin is hyperextensible, and joints are hypermobile. The diagnosis is usually through clinical features, genetic testing, and tissue biopsy. [42] Similarly, in Fabry disease, patients may also have other organs being affected, including kidneys and heart, and have skin lesions called angiokeratomas. They also have pain in the extremities. Fabry disease is usually diagnosed with clinical findings and genetic testing. [43] In cases of platelet dysfunction disorders, bleeding is usually mucocutaneous, unlike hemophilia. Usually, these disorders are diagnosed by platelet aggregation studies or platelet electron microscopy. [44] In the deficiency of other coagulation factors, musculoskeletal bleeding is uncommon. In fact, sometimes thrombosis can occur, especially in patients with factor VII or fibrinogen deficiency or in patients with combined factor V and VIII deficiency. Specific coagulation factor assays usually confirm the diagnosis. Disseminated intravascular coagulation (DIC) that mimics hemophilia is hard to differentiate, but usually, there is an underlying condition in DIC, for example, acute promyelocytic leukemia. Diagnosis is usually carried out by blood tests that show decreased platelet count and the absence of factor VIII autoantibodies. Child abuse can sometimes be misidentified and confused with hemophilia, and it is essential to find inconsistencies in the history of how trauma has occurred. Other signs of malnourishment require vigilance, and x-rays may reveal evidence of fractures of different ages. [45] [46]"}, {"id": "wiki20220301en100_5572", "title": "List of MeSH codes (C16)", "score": 0.010553058432675396, "content": "– anemia, hypoplastic, congenital – anemia, Diamond–Blackfan – fanconi anemia – ataxia telangiectasia – blood coagulation disorders, inherited – activated protein C resistance – afibrinogenemia – antithrombin III deficiency – Bernard–Soulier syndrome – factor V deficiency – factor VII deficiency – factor X deficiency – factor XI deficiency – factor XII deficiency – factor XIII deficiency – hemophilia A – hemophilia B – Hermansky–Pudlak syndrome – hypoprothrombinemias – protein C deficiency – thrombasthenia – Von Willebrand disease – Wiskott–Aldrich syndrome – CADASIL – cardiomyopathy, hypertrophic, familial – cherubism"}, {"id": "article-18225_15", "title": "Bernard-Soulier Syndrome -- History and Physical", "score": 0.010514704156593987, "content": "The International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH-BAT) is useful for assessing bleeding disorders. Its utility was tested in a small study, including patients with known inherited platelet disorders. The study demonstrated a specificity of 100%, a positive predictive value of 90%, and a negative predictive value of 100% using this assessment tool. [17] In the setting of von Willebrand disease, a BAT score >6 repeatedly correlates to a 99% probability of an inherited platelet defect such as BSS. [8] ] Similarly, other bleeding assessment tools like Molecular and Clinical Markers for the Diagnosis and Management (MCMDM) of type 1 von Willebrand's disease (VWD) and the World Health Organization Bleeding Assessment Tool are similar tools. An electronic version of MCMDM-type-1 VWD was developed in 2010. [17]"}, {"id": "InternalMed_Harrison_26188", "title": "InternalMed_Harrison", "score": 0.01045157359454456, "content": "Hemophilia is a sex-linked recessive genetic disorder characterized by the absence or deficiency of factor VIII (hemophilia A, orclassic hemophilia) or factor IX (hemophilia B, or Christmas disease) (Chap. 141). Hemophilia A constitutes 85% of cases. Spontaneous hemarthrosis is a common problem with both types of hemophilia and can lead to a deforming arthritis. The frequency and severity of hemarthrosis are related to the degree of clotting factor deficiency. Hemarthrosis is not common in other disorders of coagulation such as von Willebrand disease, factor V deficiency, warfarin therapy, or thrombocytopenia. Hemarthrosis occurs after 1 year of age, when a child begins to walk 2241 and run. In order of frequency, the joints most commonly affected are theknees,ankles,elbows,shoulders,andhips.Smalljointsofthehands and feet are occasionally involved."}, {"id": "pubmed23n0597_16253", "title": "Hemarthrosis due to a rare cause of hemorrhagic diathesis: Ehlers-Danlos syndrome.", "score": 0.01044450504499591, "content": "The authors report a case of hemarthrosis complicated by severe anemia related to a congenital connective tissue disease: Ehlers-Danlos syndrome. A boy fell down and suffered tumefaction of both knees with bilateral rupture of the rotula tendon. He underwent surgical reinsertion of each tendon on the rotula. He later showed an unexpected ongoing hematic effusion, with severe anemia. He was screened for coagulation disorders with no results. On taking a more detailed history and investigating the patient's phenotypical features, the authors diagnosed Ehlers-Danlos syndrome, hypermobile variant. The hemarthrosis and anemia were thus concluded to be consequences of excessive tissue fragility due to a congenital connective tissue disease."}, {"id": "pubmed23n0709_6992", "title": "The role of ultrasonography in the diagnosis of the musculo-skeletal problems of haemophilia.", "score": 0.009900990099009901, "content": "Recurrent haemarthrosis is the final cause of haemophilic arthrosic disease in haemophilia patients. Therefore, it is essential to diagnose it early, both clinically and by imaging. In addition, haemophilia patients experience chronic synovitis, joint degeneration, muscle haematoma and pseudotumours. The objective of this article is to highlight the value of ultrasounds in the diagnosis and control of the evolution of musculo-skeletal problems in haemophilia patients. To this end, we have performed a literature search in the PubMed, Web of Science(®) (WOS) and SciVerse bases, using the following keywords: hemophilia or haemophilia and ultrasonography (US), ultrasound, echography and sonography. The search was limited to studies published in English between the years 1991 and 2011, finding a total of 221 references. After reviewing the title or abstract for evidence of the use of US for the diagnosis of musculo-skeletal lesions in haemophilia, we selected 24 of these references. We added data collected from our experience to the most important data found in the references. Our main conclusion is that US is highly valuable for the diagnosis of musculo-skeletal diseases in haemophilia. It is a fast, effective, safe, available, comparative, real-time technique that can help us confirm the clinical examination. It is particularly important in acute haemarthrosis, as it can be used to objectively identify the presence of blood in the joints, measure its size, pinpoint its location, assess its evolution and confirm its complete disappearance."}, {"id": "pubmed23n1125_14898", "title": "The value of ultrasonography in detecting early arthropathic changes and contribution to the clinical approach in patients of hemophilia.", "score": 0.00980392156862745, "content": "PURPOSE\\AIM: Hemophilia affects the blood clotting process, is a genetic disease characterized by recurrent bleeding. The hemophilia early arthropathy detection with ultrasound (HEAD-US) procedure and scoring method were designed for the detection of early changes in affected joints of patients. In this article, it was aimed to detect early arthropathic changes in the joints of hemophilia patients with the HEAD US scoring system and to investigate its clinical contribution. It was aimed to investigate the effectiveness of HEAD-US scoring in showing early joint damage in subclinical hemophilia cases and its contribution to treatment. The present study included 50 hemophilia patients who were admitted to Departments of Pediatric and Adult Hematology for routine follow-up. During routine follow-up controls, patients were scored by physical examination and HJHS 2.1 and by ultrasonography and HEAD US. Statistical tests were used to analyze joint health status and the results of US examination in the patient group. A total of 294 joints (elbow n = 100, knee n = 94, ankle n = 100) were evaluated by ultrasonography. The mean HJHS and HEAD-US scores of the patients were 14.94 ± 15.18 and 15.6 ± 12.6, respectively. HEAD-US is accepted to be more sensitive than HJHS in detecting early signs of arthropathy. Detection of early abnormalities by ultrasonography will enable the development of individualized treatment protocols and to the prevention of arthropathy development."}, {"id": "pubmed23n0318_20292", "title": "Nonsurgical synovectomy in the treatment of arthropathy in Von Willebrand's Disease.", "score": 0.009708737864077669, "content": "Von Willebrand's disease is the most common inherited bleeding disorder, with an overall prevalence in the general population of 0.8% to 1.3%. Hemarthrosis occurs mainly in the severest forms of the disease (type III), with a frequency of 3.5% to 11%, and can cause severe arthropathy similar to that seen in hemophilia. We retrospectively reviewed our experience with nonsurgical synovectomy in the treatment of recurrent hemarthrosis with arthropathy in patients with von Willebrand's disease. Four of our six patients had type III disease and the remaining two had type II disease. The age range was 13 to 63 years. The frequency of hemarthrosis prior to synovectomy was one to four per month. One (n = 2) or both (n = 1) knees were treated in 4 cases, one (n = 1) or both (n = 1) ankles in 3 cases and an elbow in one case. We used yttrium 90 in a dose of 5 mCi for one knee, rhenium 186 in a dose of 2 mCi for two ankles and the elbow and osmic acid for two knees and one ankle. Clinical and radiological results were evaluated six months after synovectomy using the World Federation of Hemophilia score. Radiologic lesions remained stable and clinical manifestations improved in every case (p &lt; 0.05). Five patients achieved a complete remission. Safety was satisfactory. The clinical efficacy of synovectomy done, using radiocolloids or osmic acid in arthropathy due to von Willebrand's disease, seems similar to that in hemophilia."}, {"id": "pubmed23n0905_24616", "title": "Joint assessment in von Willebrand disease. Validation of the Haemophilia Joint Health score and Haemophilia Activities List.", "score": 0.009708737864077669, "content": "Assessment of clinical outcome after joint bleeding is essential to identify joint damage and optimise treatment, to prevent disability. However, disease-specific tools to assess the musculoskeletal status in patients with von Willebrand disease (VWD) are lacking. We aimed to determine validity and reliability of the Haemophilia Joint Health Score (HJHS) and Haemophilia Activities List (HAL) in patients with Von Willebrand disease (VWD). Ninety-six patients with VWD were included (mean age 46 years) of whom 27 had more than five documented joint bleeds. The HJHS was performed in all patients and all patients completed the HAL and Impact on Participation and Autonomy (IPA) questionnaires. Health-related quality of life (SF36) results were obtained from the prior 'Willebrand in the Netherlands' study. Joint X-rays of knees, elbows and ankles were scored according to Pettersson (PS). Internal consistency of the HJHS (Cronbach's α (α)=0.75) and HAL (α=0.89) were good. Inter-observer agreement of the HJHS was good (ICC 0.84; Limits of Agreement ± 10.3). The HJHS showed acceptable correlation with the X-ray PS (Spearman's r (r<subs</sub)&gt;0.60 all joints) and HAL (r<subs</sub=0.71). The HAL also showed acceptable correlation with the SF36 physical functioning (r<subs</sub=0.65) and IPA (r<subs</sub=0.69). Hypothesis testing showed adequate discriminative power of both instruments: in patients with a history of &gt;5 versus ≤ 5 joint bleeds (median HJHS 10 vs 2 (p&lt;0.01); median HAL 77 vs 98 (p&lt;0.01)), independent from age. In conclusion, both the HJHS and HAL are feasible to assess clinical outcome after joint bleeds in VWD."}, {"id": "wiki20220301en023_76222", "title": "Von Willebrand factor", "score": 0.009615384615384616, "content": "Interactions von Willebrand Factor has been shown to interact with Collagen, type I, alpha 1. Recently, It has been reported that the cooperation and interactions within the Von Willebrand Factors enhances the adsorption probability in the primary haemostasis. Such cooperation is proven by calculating the adsorption probability of flowing VWF once it crosses another adsorbed one. Such cooperation is held within a wide range of shear rates. See also von Willebrand disease Bernard–Soulier syndrome References External links GeneReviews/NCBI/NIH/UW entry on von Willebrand Factor Deficiency. Includes: Type 1 von Willebrand Disease, Type 2A von Willebrand Disease, Type 2B von Willebrand Disease, Type 2M von Willebrand Disease, Type 2N von Willebrand Disease, Type 3 von Willebrand Disease Blood proteins Coagulation system Glycoproteins"}, {"id": "pubmed23n0902_15061", "title": "Autosomal recessive inherited bleeding disorders in Pakistan: a cross-sectional study from selected regions.", "score": 0.009523809523809525, "content": "Autosomal recessive bleeding disorders (ARBDs) include deficiencies of clotting factors I, II, V, VII, X, XI, XIII, vitamin K dependent clotting factors, combined factor V &amp; VIII, Von Willebrand Disease (vWD) type 3, Glanzmann's thrombasthenia (GT) and Bernard-Soulier syndrome. Patients with primary bleeding disorders from all the major provincial capitals of Pakistan were screened for ARBDs. Prothrombin (PT), activated partial thromboplastin time (APTT), bleeding time (BT) and fibrinogen levels were measured. Cases with isolated prolonged APTT were tested for factors VIII and IX using factor assays This was followed by FXI:C level assessment in cases with normal FVIII and FIX levels. vWD was screened in patients with low FVIII levels. Factors II, V and X were tested in patients with simultaneous prolongation of PT and APTT. Peripheral blood film examination and platelet aggregation studies were performed to assess platelet disorders. Urea clot solubility testing was done to detect Factor XIII levels where platelet function tests were normal. Descriptive analysis was done using SPSS version 16. Of the 429 suspected bleeding disorder patients, 148 (35%) were diagnosed with hemophilia A and 211 (49.1%) patients had ARBDs. 70 patients (16.3%) remained undiagnosed. Out of 211 patients with ARBD; 95 (33.8%) had vWD type 3. Fibrinogen deficiency was found in 34 patients (12%), GT in 27 (9.6%), factor XIII deficiency in 13 (4.6%), factor VII deficiency in 12 (4.3%), factor V deficiency in 9 (3.2%). Eight patients (2.8%) had vitamin K-dependent clotting factor deficiency, Bernard-Soulier syndrome was diagnosed in seven patients (2.5%), factor X deficiency in 2 (0.7%), factor II deficiency in 2 (0.7%), factor XI deficiency and combined factor V and VIII deficiency in 1 (0.4%) patient each. vWD type 3 was the most common ARBD found in our sample of patients in Pakistan, followed by fibrinogen deficiency and GT in respective order."}, {"id": "pubmed23n0820_14481", "title": "Spectrum of Von Willebrand's disease in Punjab: clinical features and types.", "score": 0.009523809523809525, "content": "Von Willebrand's disease (VWD) is a common inherited bleeding disorder caused by quantitative deficiency (Type-1 &amp; Type-3 VWD) or qualitative defect of Von Willebrand's Factor (Type-2 VWD). Regarding VWD limited studies are available in Pakistan. The current study was aimed to determine the clinical presentation and frequency of types of VWD. A cross sectional study was carried out from 16th December 2012 to 15th December 2013 on fifty one patients of VWD. Patients were diagnosed on the basis of prolonged bleeding time, abnormal APTT, reduced level of VWF: Ag, FVIII, VWF: RCo and ratio of VWF: RCo/VWF Ag. Among them 26 (50.98%) were male and 25 (49.02%) were female. Type3 VWD (94.12%) was found to be the commonest type. Two (3.92%) cases of type-2 VWD and only one (1.96%) case of type-1 VWD were identified. Easy bruising was the most commonly observed clinical presentation, 21 (41.18%) patients, followed by epistaxis 7 (13.73%), gum bleed 4(7.84%) menorrhagia 5(9.80%), haemarthosis 2(3.92%), haematoma formation 5 (9.80%), bleeding after circumcision 2 (3.92%), bleeding after surgery 2 (3.92%) and umbilical cord bleeding 3 (5.88%). Consanguineous marriages were reported in parents of 42 (82.4%) patients. Family history of bleeding disorder was reported in 44 (86.27%) of cases. Type-3 VWD was found to be the commonest type which can be attributed to the fact that type-3 VWD is transmitted through autosomal recessive pattern of inheritance and consanguineous marriages are highly practiced in our society leading to high frequency of this form of VWD. Easy bruising and epistaxis were concluded to be the most common clinical presentation. Menorrhagia was found to be common in the females of child bearing age."}, {"id": "pubmed23n0868_20021", "title": "Abstracts of papers on haemophilia from other journals.", "score": 0.009433962264150943, "content": "Surgical synovectomy in haemophilic arthropathy of the knee. Rodriguez Merchan EC, Galindo E, Ladreda JMM, Pardo JA Definition of the bleeding tendency in factor XI deficient kindreds: a clinical and laboratory study. Bolton-Maggs PHB, Patteson DA, Wensley RT, Tuddenham EGD. Rapid genotype analysis in type 2B von Willebrand's disease using a universal heteroduplex generator. Wood N, Standen G, Murray EW, Lillicrap D, Holmberg L, Peake IR, Bidwell J Biological effect of desmopressin in eight patients with type 2N ('Normandy') von Willebrand disease. Mazurier C, Gaucher C, Jorieux S, Goudemand M. Heterogeneity of hepatitis C virus genotypes in haemophilia: relationship with chronic liver disease. Preston FE, Jarvis LM, Makris M, Philp L, Underwood JCE, Ludlam CA, Simonds P. "}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[174, 314]], "word_ranges": [[31, 60]], "text": "the less harmful answer is the 2. Because it is the one with the shortest half-life and he does not want the lady to fall down the next day."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Knowing that we have taken measures of sleep hygiene, we have explored what provokes him not to sleep, and he does not sleep nine hours and still wants to sleep more,...then the less harmful answer is the 2. Because it is the one with the shortest half-life and he does not want the lady to fall down the next day.", "full_answer_no_ref": "Knowing that we have taken measures of sleep hygiene, we have explored what provokes him not to sleep, and he does not sleep nine hours and still wants to sleep more,...then [HIDDEN]. [HIDDEN] and he does not want the lady to fall down the next day.", "full_question": "We are consulted by an 84-year-old woman for insomnia of conciliation. After failing sleep hygiene measures, it is decided to initiate pharmacological treatment. Which of the following drugs would you select for the patient?", "id": 150, "lang": "en", "options": {"1": "Diacepam.", "2": "Lormetacepam.", "3": "Phenobarbital.", "4": "Chlordiazepoxide.", "5": "Chloracepate."}, "question_id_specific": 201, "type": "PSYCHIATRY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0209_16856", "title": "[Insomnia therapy and withdrawal of hypnotics].", "score": 0.01526374859708193, "content": "The aim of this study is to evaluate the efficiency of a treatment prescribed, in the course of an hospital consultation for sleep pathology, to patients suffering from chronic insomnia not improved by longstanding and sustained medication with hypnotic drugs. The basis of the treatment is a progressive but total withdrawal of hypnotics in so far taken regularly. The withdrawal of hypnotics was prescribed to 79 patients: 33 aged 17 to 39 years (group 1, mean age 30) and 46 aged 40 to 70 years (group 2, mean age 51). 41 showed primary psychophysiological insomnia and 28 showed insomnia associated with psychiatric disorders. In patients of group 1, the average durations were 8 years for insomnia and 3 years for sustained hypnotic use; these durations were 15 and 5 years respectively in patients of group 2. Hypnotic drug withdrawal was achieved without placebos in 3 months in group 1 patients and 5 months in group 2 patients. 65 patients completely stopped the continual use of hypnotics. Subjective improvement of insomnia was reported by 51 of these patients (as well as by 6 patients who were given simultaneous antidepressant therapy). 16 of the 51 improved patients have resorted to hypnotics occasionally (at intervals of 10 days or more). After complete withdrawal, patients went on consulting for various lengths of time: 5 months average for group 1, 14 months average for group 2. This study of a fairly large group of insomniacs shows the frequent ineffectiveness of a sustained use of currently available hypnotics. It also shows that two times out of three the complete stop of sustained hypnotic medication proved beneficial to the patient."}, {"id": "pubmed23n0546_22837", "title": "Studio Morfeo 2: survey on the management of insomnia by Italian general practitioners.", "score": 0.015219907407407408, "content": "To carry out an observational epidemiological survey (Studio Morfeo 2) in order to define the management procedures of insomnia in a large Italian population presenting directly to the general practitioner (GP). Each GP recruited five insomniac subjects in the course of 1 week or 5 consecutive office days over a period of 2 weeks. On each office day, a brief questionnaire (Q1) including five questions investigating insomnia symptoms and current use of treatment was administered to the first 10 patients who referred to the GP office for reasons associated with their own health. The first patient of each day classified as insomniac underwent a second investigation based on a more detailed questionnaire (Q2) including demographic variables, socio-economic status, general medical conditions, severity, duration and clinical features of insomnia, daytime dysfunction, sleep satisfaction and therapeutic management. In a primary care setting, insomnia symptoms are often persistent (&gt;1 year), recurrent (&gt;1/week) and accompanied by daytime consequences. Two out of three patients with insomnia symptoms are dissatisfied with their sleep. In most cases, insomnia symptoms are underrated both by the patients, who cover the problem or reject treatment, and by the GP, who limits intervention on the sleep disorder (scarcely modifying ongoing therapy both in responders and in non-responders). In responders, treatment was confirmed in 91% of cases and discontinued in only 2%. When there was no improvement, or if insomnia symptoms became worse (non-responders), treatment was nevertheless continued in 74.5% of cases, either maintaining the same ineffective dose, increasing the dose, or adding another drug or a non-pharmacological procedure. Regardless of specific medication, the Italian GP privileges the pharmacological approach, which is fourfold more frequent than non-pharmacological therapy (78.6 versus 18.2%). Non-benzodiazepine hypnotic drugs are mostly prescribed when the GP decides to apply medication in previously untreated patients with insomnia symptoms. Self-administration is not unusual among the patients with insomnia symptoms and is more common among non-responders. Italian GPs tend to confirm the ongoing therapy and avoid re-evaluation of the treatment regimen. Limited use of non-pharmacological treatment in the Italian primary care setting is in line with this conservative approach of the GPs who tend to be problem-solvers rather than problem-seekers."}, {"id": "pubmed23n1022_10550", "title": "Perampanel in chronic insomnia.", "score": 0.01465923291358262, "content": "Insomnia is the most prevalent sleep disorder in the general population, and one of the most frequent reasons for consultation in the Sleep Units. Perampanel is an antiepileptic also effective on the structure of sleep, and in restless legs syndrome. We describe the first study that evaluates perampanel in patients with chronic insomnia. Observational retrospective cohorts study of 66 patients with chronic resistant insomnia, 33 exposed to perampanel, other 33 as non-exposed group. All patients attended in Neurology or Psychiatry Consultation, from November 2017 to November 2018. Patients included had been treated with more than 4 different drugs in the previous 4 years. We reviewed age, sex, insomnia etiology, years of evolution, number of previously used drugs, and the results of perampanel for insomnia after 3 months of treatment in the exposed cohort, measured by the improvement of 3 or more points in the ISI and Pittsburgh scales, as well as the average of hours of sleep gained. Non-exposed patients were matched with this variables, but never treated with perampanel. We have included 66 patients. In the exposed cohort: we describe 33 patients with chronic resistant insomnia, 20 women (60 %), 13 men (40 %). Average age 53.48 years, average time of evolution: 11.25 years. Main etiology: depression 13 cases (40 %). After the combination of perampanel 2-4 mg (100 %) with antidepressants (17 cases, 51.5 %) or anxiolytics (12 cases, 36.36 %) along 3 months: the total number of hours of sleep improves in 2.5 h, the scale ISI improves by 6 points (± 2.1 SD, p = 0.02), and Pittsburgh scale improves in 4 points (± 1.7, p = 0.04). In non-exposed cohort, the improvement of the ISI scale was 2.2 points (±0.8, p = 0.06), on the Pittsburgh scale was 1.6 points (± 0.5, p = 0.01). The main adverse effect was irritability in 3 patients, without withdrawal perampanel. The treatment was abandoned by 4 patients (12.12%): 1 due to persistent irritability (3%), 2 due to lack of efficacy (6 %), 1 due to pregnancy wish (3 %). The combination of Perampanel with an antidepressant, or an anxiolytic, improves the quality of sleep measured by ISI and Pittsburgh scales (statistically significant), probably due to its antagonistic action on glutamate. A clinical trial compared with placebo would be necessary to corroborate these results."}, {"id": "wiki20220301en115_9133", "title": "Chlordiazepoxide", "score": 0.012640716126407162, "content": "Tolerance Chronic use of benzodiazepines, such as chlordiazepoxide, leads to the development of tolerance, with a decrease in number of benzodiazepine binding sites in mouse forebrain. The Committee of Review of Medicines, who carried out an extensive review of benzodiazepines including chlordiazepoxide, found—and were in agreement with the Institute of Medicine (USA) and the conclusions of a study carried out by the White House Office of Drug Policy and the National Institute on Drug Abuse (USA)—that there was little evidence that long-term use of benzodiazepines were beneficial in the treatment of insomnia due to the development of tolerance. Benzodiazepines tended to lose their sleep-promoting properties within 3–14 days of continuous use, and in the treatment of anxiety the committee found that there was little convincing evidence that benzodiazepines retained efficacy in the treatment of anxiety after 4 months' continuous use due to the development of tolerance."}, {"id": "wiki20220301en426_11111", "title": "GABAA receptor positive allosteric modulator", "score": 0.01245511030040472, "content": "Insomnia Barbiturates were introduced as hypnotics for patients with schizophrenia. It induced a state of deep and prolonged sleep. But this was not used for long because of adverse side effects. Anticonvulsant Phenobarbital was the first truly effective drug against epilepsy. It was discovered by accident when given to epileptic patients to help them sleep. The positive side effects were anticonvulsant properties that reduced seizure number and intensity. Sedation Pentobarbital is used as a hypnotic when analgesia is not required. It´s often used in CT imaging when sedation is needed. It is efficient, safe and the recovery time is short. In 2013 the barbiturates phenobarbital and butabarbital are still used as sedatives in certain cases as well as to antagonize the effects of drugs as ephedrine and theophylline. Phenobarbital is used in cases of drug withdrawal syndromes. It is used as normal and emergency treatment in some cases of epilepsy."}, {"id": "wiki20220301en053_35214", "title": "Somnology", "score": 0.011975432374784777, "content": "Generally, these treatments are given after the behavioral treatment has failed. Drugs such as tranquilizers, though they may work well in treating insomnia, have a risk of abuse which is why these treatments are not the first resort. Some sleep disorders such as narcolepsy do require pharmacological treatment. See also Sleep disorder Sleep medicine Snoring References External links Sleep disorders sv:Somnologi"}, {"id": "wiki20220301en053_35213", "title": "Somnology", "score": 0.010995410813706991, "content": "Pharmacological treatments Pharmacological treatments are used to chemically treat sleep disturbances such as insomnia or excessive daytime sleepiness. The kinds of drugs used to treat sleep disorders include: anticonvulsants, anti-narcoleptics, anti-Parkinsonian drugs, benzodiazepines, non-benzodiazepine hypnotics, and opiates as well as the hormone melatonin and melatonin receptor stimulators. Anticonvulsants, opiates, and anti-Parkinsonian drugs are often used to treat restless legs syndrome. Furthermore, melatonin, benzodiazepines hypnotics, and non-benzodiazepine hypnotics may be used to treat insomnia. Finally, anti-narcoleptics help treat narcolepsy and excessive daytime sleepiness. Of particular interest are the benzodiazepine drugs which reduce insomnia by increasing the efficiency of GABA. GABA decreases the excitability of neurons by increasing the firing threshold. Benzodiazepine causes the GABA receptor to better bind to GABA, allowing the medication to induce sleep."}, {"id": "wiki20220301en057_237", "title": "Clobazam", "score": 0.010418009747543774, "content": "In India, clobazam is approved for use as an adjunctive therapy in epilepsy, and in acute and chronic anxiety. In Japan, clobazam is approved for adjunctive therapy in treatment-resistant epilepsy featuring complex partial seizures. In New Zealand, clobazam is marketed as Frisium In the United Kingdom clobazam (Frisium) is approved for short-term (2–4 weeks) relief of acute anxiety in patients who have not responded to other drugs, with or without insomnia and without uncontrolled clinical depression. It was not approved in the United States until October 25, 2011, when it was approved for the adjunctive treatment of seizures associated with Lennox-Gastaut syndrome in patients 2 years of age or older. Contraindications Clobazam should be used with great care in patients with the following disorders: Myasthenia gravis. Sleep apnea. Severe liver diseases such as cirrhosis and hepatitis. Severe respiratory failure."}, {"id": "pubmed23n0562_13117", "title": "Effectiveness and safety of hypnotic drugs in the treatment of insomnia in over 70-year old people.", "score": 0.009900990099009901, "content": "Good sleep is an important index of the quality of life in people and above all in old subjects. Among all the symptoms reported to general practitioner, insomnia is at the 3(rd) place and this is present in particular in the elderly. In elderly people high comorbidity and polytreatment are often present. We have studied 60 elderly people with history of insomnia and concomitant diseases: depression, dementia and behavioral disturbances. All the patients of the present study were visited in our outpatients' department. Three hypnotic drugs were used for the treatment of insomnia: zolpidem, or triazolam, or oxazepam, respectively at doses of 10mg/day, 0.125-0.25mg/day and 15.0mg/day. All the three drugs showed to be effective and safe; no paradoxical effects were observed."}, {"id": "pubmed23n0956_4804", "title": "Autism Spectrum Disorder and Mental Health Comorbidity Leading to Prolonged Inpatient Admission.", "score": 0.00980392156862745, "content": "Sam is a 6-year-old boy with a diagnosis of autism spectrum disorder (ASD) who recently relocated and has an appointment with you, his new pediatric clinician, to establish care. He was previously followed by a psychiatrist for 2 years for additional diagnoses of insomnia, bipolar disorder, anxiety, attention deficit hyperactivity disorder, and intellectual disability. He has tried and (apparently) failed multiple psychotropic trials including stimulants, nonstimulants, mood stabilizers, atypical antipsychotics, and nonbenzodiazepine hypnotics. He has a delayed sleep onset and frequent night awakenings each night for the past 3 months, during which he \"screams, cries, and thrashes and can stay up for over an hour.\" His behaviors are described as irritable, self-injurious, and aggressive with no clear pattern of triggers according to his mother. He is nonverbal and communicates by leading and rarely pointing. The patient's current medication regimen includes clonidine 0.2 mg at night, lorazepam 1.5 mg as needed at night, olanzapine 5 mg twice daily, and diphenhydramine as needed for sleep/agitation. His mother is concerned that he is developing \"tolerance\" to the regimen and wants to wean him off some of the medications. His mother is struggling to take care of the patient given his worsening behavior and body habitus (body mass index &gt;99%; z = 3.41).There is a family history of depression, anxiety, bipolar disorder, and autism. He has a 3-year-old sister, who is also diagnosed with ASD, though she is not as severely impacted. His mother's partner recently moved in along with 2 children of his own, aged 3 and 4 years. Sam attends a specialized school, where he receives behavior therapy and occupational therapy. He has undergone inpatient pediatric hospitalization twice, 1 time for 3 weeks and the other for 6 days, for aggressive behavior, and in both instances, he was discharged before inpatient psychiatric placement because of a lack of available beds.After urgent consultation with your local developmental and behavioral pediatrician, a slight reduction was made in the lorazepam because of concerns about tolerance and side effects. However, within a week of this, he was brought to the emergency department for continued self-injurious behavior and increased trouble with sleeping. His mother voiced concerns about his safety in the home, which were particularly related to aggression toward his younger sister. He was admitted to the pediatric inpatient floor for observation, and medication adjustment (increasing olanzapine), which was initially helpful in improving behavior, but mostly behavioral/environmental strategies were used to soothe him, including frequent wagon rides through the hospital corridors.Despite the patient being stable from the medical standpoint, Sam's mother did not feel comfortable taking him home. Social work contacted local community mental health services to pursue outpatient resources and respite care options and sought inpatient pediatric psychiatry. After several failed attempts to find placement, he remained in pediatric inpatient care for 1 and a half months with no acute medical interventions other than his oral medications.He was finally accepted to the in-state pediatric psychiatric facility when a bed was available. During his week-long stay, he had further medication adjustments with a decrease in olanzapine and optimization of his clonidine dose. During his psychiatric hospital stay, care coordination succeeded in arranging center-based applied behavior analysis interventions and respite care and parent training for his family. Sam began to show improvement in his overall agitation and aggression, requiring less clonazepam, and his mother then maintained outpatient follow-up.The day before discharge, you visit him in the hospital, and a medical student asks you why he was in the hospital for so long. How would you answer the question?"}, {"id": "pubmed23n0002_2549", "title": "[Clinical picture and therapy of sleep in aged, internal-medicine patients].", "score": 0.00980392156862745, "content": "The management of sleep disorders in elderly patients with internal diseases consists in the first line in rectifying pathophysiological disregulations. Only in the second line, proper hypnotics are to be prescribed. When considered as indispensable, these medicaments are selected according to their toxicity and side effects. In present time, Benzodiazepines are definitely preferred, whereas neuroleptic, anti-depressant and the older drugs are to be taken secondarly in account."}, {"id": "pubmed23n0253_13", "title": "[Sleep disorders--what can be done when hypnotics no longer help? Overview and case report].", "score": 0.009708737864077669, "content": "We report on the case of a 45-year old female with chronic insomnia and refractory to hypnotics, who also has a - polygraphically documented - tolerance to the imidazopyridine \"zolpidem\". We discuss the main differential diagnosis and demonstrate a therapeutic regimen which allows a step-by-step replacement of hypnotics by sedative antidepressants. This interval replacement treatment reduces on the one hand the risk of developing a severe withdrawal syndrome. On the other hand the replacement by sedative antidepressants improves insomnia and insomnia-associated depressive symptoms. Finally, the clinical implications and rationale of a therapeutic approach with sedative antidepressants in chronic insomnia are discussed."}, {"id": "wiki20220301en115_9126", "title": "Chlordiazepoxide", "score": 0.009686649779529037, "content": "Chlordiazepoxide, trade name Librium among others, is a sedative and hypnotic medication of the benzodiazepine class; it is used to treat anxiety, insomnia and symptoms of withdrawal from alcohol and other drugs. Chlordiazepoxide has a medium to long half-life but its active metabolite has a very long half-life. The drug has amnesic, anticonvulsant, anxiolytic, hypnotic, sedative and skeletal muscle relaxant properties. Chlordiazepoxide was patented in 1958 and approved for medical use in 1960. It was the first benzodiazepine to be synthesized and the discovery of chlordiazepoxide was by pure chance. Chlordiazepoxide and other benzodiazepines were initially accepted with widespread public approval but were followed with widespread public disapproval and recommendations for more restrictive medical guidelines for its use."}, {"id": "pubmed23n0329_5531", "title": "Behavioral treatment of chronic insomnia in psychiatrically ill patients.", "score": 0.009615384615384616, "content": "Psychiatric patients often have residual intractable insomnia as a serious problem. Forty-eight psychiatrically ill patients (DSM-IV diagnoses) who had failed to respond to medicinal treatment for chronic insomnia were referred for and completed behavioral therapy as an adjunct to the pharmacologic treatment of their insomnia. The behavioral treatments included structured sleep hygiene, progressive muscle relaxation, stimulus control, and sleep restriction. The treatment program was accomplished in 6 sessions over 2 months. Follow-up evaluations were completed at 2, 6, and 12 months from the beginning of the treatment program. The outcome of the treatment program was evaluated in terms of the change in (1) self-reported specific sleep parameters, (2) self-ratings of sleep-related day-time state, (3) self-rating of quality of sleep, (4) the use of sleep medication, and (5) the therapist's global rating of improvement. There was a statistically significant change from the baseline in all self-reported specific sleep parameters after 2 months that was sustained after 6 and 12 months. Sleep-related characteristics of daytime state showed statistically significant changes after 2 and 6 months that were maintained after 12 months. Sleep quality had a statistically significant change after 2 months, continued to improve statistically after 6 months, and was maximum after 12 months. Over half the patients (52.7%; 20 of 38) either reduced their sleep medication by half or stopped it completely. The therapist's global rating showed an improvement in 29.2% (N = 14) of patients after 2 months, 56.2% (N = 27) after 6 months, and 68.7% (N = 33) after 12 months. The use of concomitant behavioral and pharmacologic treatment of chronic insomnia in psychiatrically ill patients results in improving sleep and sleep-related state and reduces the risk of return of insomnia for 10 months after finishing active treatment."}, {"id": "pubmed23n0204_4159", "title": "[Therapeutic trials in outpatients. Apropos of triazolam trials].", "score": 0.009615384615384616, "content": "Clinical trials conducted in general practice are more especially interesting as they enable to test a drug in the real conditions of use. On the other hand, these trials are beneficial to the G.P. (new image, rupture of his loneliness, change in his prescription habits, contact with hospitals). The methodology, as for hospital studies, must be rigorous. As a matter of fact, these two types of studies are additional and the cooperation between the G.P.'s and the pharmaceutical industry can conduct to the solution of specific problems: drug interaction--long-term therapeutic effect--new indications. This double blind cross over study comparing triazolam and nitrazepam conducted by G.P.'s on insomniacs is the first French clinical study intended for the registration application and done according to this methodology. This work is an exemple of the new opportunity offered to G.P.'s in future. The results have shown that: on 54 patients (23 male and 31 female) of an average age: 48, 32 have preferred triazolam, 13 have preferred nitrazepam, 9 have had no preference."}, {"id": "wiki20220301en619_15476", "title": "Cabotegravir/rilpivirine", "score": 0.009523809523809525, "content": "Contraindications and interactions Cabotegravir/rilpivirine must not be combined with drugs that induce the liver enzyme CYP3A4, because they accelerate the inactivation of rilpivirine, and/or the enzmye UGT1A1, because they accelerate the inactivation of cabotegravir. These mechanisms potentially result in loss of effectiveness. Examples for such drugs are rifampicin, rifapentine, carbamazepine, oxcarbazepine, phenytoin and phenobarbital. Adverse effects The most common side effects of the injectable combination therapy with rilpivirine are reactions at the injection site (in up to 84% of patients) such as pain and swelling, as well as headache (up to 12%) and fever or feeling hot (in 10%). Less common side effects (under 10%) are depressive disorders, insomnia, rashes, fatigue, musculoskeletal pain, nausea, sleep disorders, and dizziness. Pharmacology"}, {"id": "pubmed23n0038_1959", "title": "Insomnia: often a therapeutic challenge.", "score": 0.009523809523809525, "content": "Insomnia, a more or less chronic sleep disturbance, is a very common symptom in psychiatric patients but also relatively freguent in the general population to a lesser degree. Two broad types of insomnia may often be distinguished: (1) difficulty falling asleep and frequent wakening, characteristic of anxiety states or obsessive worrying; and (2) early morning wakening, sometimes in a panic, suggestive of endogenous depression. The first group of patients respond well to minor tranquilizers and psychotherapy, whereas the second do well with tricyclic anti-depressants. Many studies in sleep laboratories have declineated the stages and cycles of sleep physiology and pathology, especially the importance of REM or dreaming sleep. The clinician should be cautious in the use of hypnotics like barbiturates which suppress REM sleep and produce a rebound increase on withdrawal, as well as problems of dependence of habituation. Flurazepam and chloral hydrate are considerably safer in this respect. Understanding sleep neurophysiology and biochemistry permits appropriate individual clinical management for both psychiatric patients and medical patients with conditions like peptic ulcer and nocturnal angina pectoris."}, {"id": "wiki20220301en262_7415", "title": "Barbiturate", "score": 0.009500653808434128, "content": "for anesthetic purposes, and are also sometimes prescribed for anxiety or insomnia. This is not a common practice anymore, however, owing to the dangers of long-term use of barbiturates; they have been replaced by the benzodiazepines and Z-drugs such as zolpidem, zaleplon and eszopiclone for sleep. The final class of barbiturates are known as long-acting barbiturates (the most notable one being phenobarbital, which has a half-life of roughly 92 hours). This class of barbiturates is used almost exclusively as anticonvulsants, although on rare occasions they are prescribed for daytime sedation. Barbiturates in this class are not used for insomnia, because, owing to their extremely long half-life, patients would awake with a residual \"hang-over\" effect and feel groggy."}, {"id": "pubmed23n0495_8799", "title": "[The use of olanzapine in sleep disorders. An open trial with nine patients].", "score": 0.009433962264150943, "content": "The impossibility of treating patients with sleep disorders adequately means that, as specialists, we have to look for new pharmacological treatments and for this reason we examined the information in the paper by Salin Pascual (1999) about the increase in deep sleep when olanzapine is used as an antipsychotic drug. We decided to use this medication in six females and three males who were suffering from different sleep disorders that conditioned their chronic insomnia. The dosages of olanzapine used ranged from 2.5 and 10 mg in a single dose. The clinical history and progress were used to elaborate the results and conclusions. The result was positive in eight of the nine patients, five who were administered the medication as monotherapy and three as polytherapy. The population studied is insufficient to prove the effectiveness of the drug, but the fact that in eight of our patients the treatment clearly improved their symptoms leads us to think that this line of research must be continued."}, {"id": "wiki20220301en002_127704", "title": "Insomnia", "score": 0.009345794392523364, "content": "Non medication based strategies provide long lasting improvements to insomnia and are recommended as a first line and long-term strategy of management. Behavioral sleep medicine (BSM) tries to address insomnia with non-pharmacological treatments. The BSM strategies used to address chronic insomnia include attention to sleep hygiene, stimulus control, behavioral interventions, sleep-restriction therapy, paradoxical intention, patient education, and relaxation therapy. Some examples are keeping a journal, restricting the time spent awake in bed, practicing relaxation techniques, and maintaining a regular sleep schedule and a wake-up time. Behavioral therapy can assist a patient in developing new sleep behaviors to improve sleep quality and consolidation. Behavioral therapy may include, learning healthy sleep habits to promote sleep relaxation, undergoing light therapy to help with worry-reduction strategies and regulating the circadian clock."}, {"id": "pubmed23n0265_4571", "title": "[Ambulatory treatment of sleep disorders in the aged].", "score": 0.009345794392523364, "content": "An enquiry into the handling by medical practitioners of sleeping problems among elderly patients was conducted in southern Lower Saxony by personal interview, combined with a standard questionnaire. A typical case report had been drafted concerning a 70-year-old, previously healthy widow: her complaints were \"nonspecific\" and could be classified as an example of either depression, of the onset of senile dementia or as within normal limits for age. This case report was presented by two interviewers to 145 general practitioners (GPs) and 14 neurologists in private practice (response rate of 83.2%) who were asked how they would have treated the patient's sleeping disorder. 30.3% of the GPs and 14.3% of the neurologists would initially not have prescribed medication. Only GPs (19.5%) mentioned possible herbal medication. Sedative neuroleptics were preferred by 57.1% of neurologists and 26.2% of GPs, while benzodiazepines would have been given by 14% of both groups. Antidepressive drugs and chloral hydrate were chosen less often (5.7% and 2.5%, respectively). These data support the finding of a high frequency of neuroleptic prescriptions given to the elderly. They also make clear that the possibility of treatment without drugs is usually not sufficiently explored."}, {"id": "wiki20220301en038_67126", "title": "Sleep hygiene", "score": 0.009259259259259259, "content": "There is support showing positive sleep outcomes for people who follow more than one sleep hygiene recommendation. There is however no evidence that poor sleep hygiene can contribute to insomnia. While there is inconclusive evidence that sleep hygiene alone is effective as a treatment for insomnia, some research studies have shown improvement in insomnia for patients who receive sleep hygiene education in combination with cognitive behavioral therapy practices."}, {"id": "pubmed23n0271_17119", "title": "[Pharmacotherapy of sleep disorders].", "score": 0.009259259259259259, "content": "Benzodiazepines and related drugs are the hypnotics of first choice. They shorten sleep latency, enhance sleep continuity and may prolong sleep duration. Their undesired effects include a persistent day-time sedation and ataxia when getting up at night. There is some risk of habit formation and dependence. For treating an acute insomnia, the prescription of hypnotics should be limited to a short duration (smallest package size), for treating chronic forms of insomnia they should have only an adjuvant role in therapy."}, {"id": "pubmed23n0169_87", "title": "Factors leading to dependence on hypnotic drugs.", "score": 0.009174311926605505, "content": "Patients in general practice complaining of insomnia of recent origin have been studied in order to ascertain which factors may be of value in the detection of those more susceptible to drug dependence. The type of sleep disturbance was found to be of importance and a personal disturbance scale was found useful as a screening test in two-thirds of the patients. No difference was found in the development of dependence on amylobarbitone and nitrazepam. One of the most important factors in the prevention of drug dependence seems to be frequent review by the doctor after the first prescription and his cautionary advice to the patient."}, {"id": "wiki20220301en024_22838", "title": "Paradoxical reaction", "score": 0.009137529137529138, "content": "Antipsychotics Chlorpromazine, an antipsychotic and antiemetic drug which is classed as a \"major\" tranquilizer, may cause paradoxical effects such as agitation, excitement, insomnia, bizarre dreams, aggravation of psychotic symptoms and toxic confusional states. Barbiturates Phenobarbital can cause hyperactivity in children. This may follow after a small dose of 20 mg, on condition of no phenobarbital administered in previous days. Prerequisity for this reaction is a continued sense of tension. The mechanism of action is not known, but it may be started by the anxiolytic action of the phenobarbital. Barbiturates such as pentobarbital have been shown to cause paradoxical hyperactivity in an estimated 1% of children, who display symptoms similar to the hyperactive-impulsive subtype of attention deficit hyperactivity disorder. Intravenous caffeine administration can return these patients' behaviour to baseline levels."}, {"id": "pubmed23n0810_134", "title": "Cognitive-behavioral therapy for chronic insomnia.", "score": 0.00909090909090909, "content": "Psychological and behavioral therapies should be considered the first line treatment for chronic insomnia. Although cognitive behavioral therapy for insomnia (CBT-I) is considered the standard of care [1], several monotherapies, including sleep restriction therapy, stimulus control therapy, and relaxation training are also recommended in the treatment of chronic insomnia [2]. CBT-I is a multimodal intervention comprised of a combination of behavioral (eg, sleep restriction, stimulus control) and cognitive therapy strategies, and psychoeducation delivered in 4 to 10 weekly or biweekly sessions [3]. Given that insomnia is thought to be maintained by an interaction between unhelpful sleep-related beliefs and behaviors, the goal of CBT-I is to modify the maladaptive cognitions (eg, worry about the consequences of poor sleep), behaviors (eg, extended time in bed), and arousal (ie, physiological and mental hyperarousal) perpetuating the insomnia. CBT-I is efficacious when implemented alone or in combination with a pharmacologic agent. However, because of the potential for relapse upon discontinuation, CBT-I should be extended throughout drug tapering [4]. Although the treatment options should be guided by the available evidence supporting both psychological therapies and short-term hypnotic treatment, as well as treatment feasibility and availability, treatment selection should ultimately be guided by patient preference [5]. Despite its widespread use among treatment providers [6], the use of sleep hygiene education as a primary intervention for insomnia should be avoided. Sleep hygiene may be a necessary, but insufficient condition for promoting good sleep and should be considered an adjunct to another empirically supported treatment."}, {"id": "wiki20220301en045_8393", "title": "Phenobarbital", "score": 0.009009009009009009, "content": "The first-line drugs for treatment of status epilepticus are benzodiazepines, such as lorazepam or diazepam. If these fail, then phenytoin may be used, with phenobarbital being an alternative in the US, but used only third-line in the UK. Failing that, the only treatment is anaesthesia in intensive care. The World Health Organization (WHO) gives phenobarbital a first-line recommendation in the developing world and it is commonly used there. Phenobarbital is the first-line choice for the treatment of neonatal seizures. Concerns that neonatal seizures in themselves could be harmful make most physicians treat them aggressively. No reliable evidence, though, supports this approach. Phenobarbital is sometimes used for alcohol detoxification and benzodiazepine detoxification for its sedative and anti-convulsant properties. The benzodiazepines chlordiazepoxide (Librium) and oxazepam (Serax) have largely replaced phenobarbital for detoxification."}, {"id": "pubmed23n0260_6985", "title": "Drug treatment of insomnia: indications and newer agents.", "score": 0.009009009009009009, "content": "Insomnia is a symptom that should be treated according to the underlying etiology. It is more common in elderly individuals and in women. Common causes of insomnia include acute situational factors, psychiatric disorders, use of various medications and illicit drugs, and medical disorders that cause pain, dyspnea or nausea. Pharmacotherapy should be generally restricted to use of the benzodiazepines, imidazopyridines (zolpidem) and occasionally tricyclic antidepressants. As a rule, hypnotic drugs should be used for less than two weeks to one month."}, {"id": "pubmed23n0406_12691", "title": "[A connection between insomnia and psychiatric disorders in the French general population].", "score": 0.008928571428571428, "content": "Untreated insomnia often has repercussions on socio-professional or cognitive functioning of insomniacs. In industrialized countries, the prevalence of insomnia ranges between 10% and 48%, depending on the methodology and the measured time interval. However, few studies have examined the relationship between insomnia and mental disorder diagnoses. This epidemiological study on insomnia complaints was conducted on 5 622 subjects representative of the non-institutionalized French population aged 15 years or over. Sixteen interviewers using the Sleep-EVAL expert system performed telephone interviews. Insomnia complaints (defined as difficulty initiating or maintaining sleep, feeling unrefreshed at awakening accompanied by dissatisfaction with sleep quality or quantity, or use of sleep-promoting medication) were observed in 18.6% (95% confidence interval: 17.6% to 19.6%) of the sample. The median duration of insomnia complaints was five years. Regional variations in the prevalence of insomnia complaints were observed in France. In North 2 and Center 4 regions, the prevalence of insomnia complaints was higher compared to the rest of France with a relative risk of 1.4 (95% confidence interval: 1.1-1.6) time superior for the North region and 1.3 (95% CI: 1.0-1.6) for the Center 4 region. The lowest prevalence was registered in the Mediterranean area. In most regions, the prevalence of insomnia complaints was higher in women than in men with the exception of the South and West regions where the prevalence was similar. Subjects with insomnia complaints consulted more frequently compared to the rest of sample with an odds ratio of 3 to 1 [95% CI: 2.8 to 4.1]. Close to 20% of subjects were being treated for a physical disease at the time of the survey; subjects with insomnia complaints being twice more numerous (34.3%) than the rest of the sample (15.9%; p&lt;0.001). To identify the main factors associated with insomnia complaints, socio-demographic and health variables were introduced in a multivariate model. Separated or divorced individuals (OR: 1.6); widowers (OR: 1.5); subjects aged between 45 and 65 years (OR: 1.4) or older than 65 (OR: 1.5); women (OR: 1.3); those with little or no education (OR: 1.4); and subjects living in the North region had higher reported insomnia complaints. Living in the East region (Mediterranean) was a protective factor (OR: 0.6). Furthermore, subjects with vascular diseases (OR: 2.0), musculo-skeletal diseases (OR: 2.0) or cardiac diseases (OR: 1.9) and those who had consulted a physician in the previous six months (OR: 2.7) had higher a probability of insomnia complaints. Subsequently, DSM IV insomnia diagnoses were examined in subjects who complained of insomnia. A diagnosis of primary insomnia was found in 7% of these subjects. A diagnosis of insomnia related to another mental disorder was found in 15.6% of insomnia complainers. A depressive disorder diagnosis was given in 10.8% of cases (mainly a major depressive disorder). This diagnosis was made more often among women and subjects of less than 65 years. An anxiety disorder diagnosis was given for 33.1% of insomnia complainers (an anxiety generalized disorder in about half the cases). About a quarter of insomnia complainers did not receive a diagnosis. This was the case more often for men and the subjects 65 years or older. If demographic and medical factors are relatively well documented at the epidemiological level, it is otherwise for psychiatric diagnosis associated with insomnia complaint. Very few studies in the general population have been done and still fewer of them have applied a positive and differential diagnosis process. In this study, we used the DSM IV classification to establish positive and differential diagnoses among subjects with insomnia complaints. Compared to other epidemiological studies, our study is distinguished by several aspects: 1) insomnia complaint had a narrower definition. It did not suffice that the subject reported insomnia symptoms, it was also necessary that the subject said s/he was dissatisfied with her/hr/his sleep or that s/he took measures to improve it (medication or sleep hygiene). This choice was motivated essentially by the fact that it is difficult, from a point of clinical point of view, to consider that an individual has insomnia solely based on the presence of symptoms, that, appreciated by a clinician, would resemble insomnia without that they make problem for the subject. 2) Several sleep habits were systematically collected. The majority of epidemiological studies are not centered on sleep problems, with the consequence that results do not allow a global view of factors that are associated with insomnia. 3) The various diagnostic categories of insomnia as well as elements of the differential diagnosis were applied. Thus, we can conclude that insomnia, as a diagnostic entity, including all its forms, is found in 5.6% of the French population. In the majority of cases, the insomnia complaint is part of the symptomatology of a mental disorder, mainly an anxiety disorder. This distinction is important since it helps the physician to determine therapeutic choices. To conclude, it is worthwhile to consider the number of insomnia complainers who had consulted a physician, mainly a general practitioner, in the six months prior to the study. This designates physicians as the first-line resource in the treatment and the prevention of sleep disorders."}, {"id": "pubmed23n0326_11643", "title": "What happens when doctors stop prescribing temazepam? Use of alternative therapies.", "score": 0.008928571428571428, "content": "We investigated the withdrawal of temazepam in a single general practice using two alternative prescribing policies: an alternative benzodiazepine; or an alternative group of drugs recommended for short-term management of insomnia, including sedative antihistamines and chloral hydrate. The study showed that temazepam prescribing in general practice can be reduced or stopped by using a simple intervention. An alternative benzodiazepine is useful in helping patients to stop their use of hypnotic agents. The use of antihistamines as substitute hypnotics is not advocated on the basis of our findings."}, {"id": "pubmed23n0890_19702", "title": "Changes in insomnia subtypes in early Parkinson disease.", "score": 0.008849557522123894, "content": "To examine the development of factors associated with insomnia in a cohort of originally drug-naive patients with incident Parkinson disease (PD) during the first 5 years after diagnosis. One hundred eighty-two drug-naive patients with PD derived from a population-based incident cohort and 202 control participants were assessed for insomnia before treatment initiation and were repeatedly examined after 1, 3, and 5 years. Insomnia was diagnosed according to the Stavanger Sleepiness Questionnaire. The Parkinson's Disease Sleep Scale was used to differentiate sleep initiation problems from problems of sleep maintenance. Generalized estimating equation models were applied for statistical measures. The prevalence of insomnia in general was not higher in patients with PD compared to controls at the 5-year follow-up. There were changes in the prevalence of the different insomnia subtypes over the 5-year follow-up. The prevalence of solitary problems in sleep maintenance increased from 31% (n = 18) in the drug-naive patients at baseline to 49% (n = 29) after 1 year and were associated with the use of dopamine agonists and higher Montgomery-Åsberg Depression Rating Scale scores. The prevalence of solitary sleep initiation problems decreased continuously from 21% (n = 12) at baseline to 7.4% (n = 4) after 5 years; these were associated with less daytime sleepiness. The prevalence rates of the different insomnia subtypes changed notably in patients with early PD. The frequency of sleep maintenance problems increased, and these problems were associated with dopamine agonist use and depressive symptoms, while the total number of patients with insomnia remained stable. Our findings reflect the need for early individual assessments of insomnia subtypes and raise the possibility of intervention to reduce these symptoms in patients with early PD."}, {"id": "Pharmacology_Katzung_2325", "title": "Pharmacology_Katzung", "score": 0.008849557522123894, "content": "Benzodiazepines can cause a dose-dependent decrease in both REM and slow-wave sleep, though to a lesser extent than the barbiturates. The newer hypnotics, zolpidem, zaleplon, and eszopiclone, are less likely than the benzodiazepines to change sleep patterns. However, so little is known about the clinical impact of these effects that statements about the desirability of a particular drug based on its effects on sleep architecture have more theoretical than practical significance. Clinical criteria of efficacy in alleviating a particular sleeping problem are more useful. The drug selected should be one that provides sleep of fairly rapid onset (decreased sleep latency) and sufficient duration, with minimal “hangover” effects such as drowsiness, dysphoria, and mental or motor depression the following day. Older drugs such as chloral hydrate, secobarbital, and pentobarbital continue to be used, but benzodiazepines, zolpidem, zaleplon, or eszopiclone are generally preferred. Daytime"}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": true, "char_ranges": [[104, 213]], "word_ranges": [[19, 39]], "text": "the imaging tests both CT, but especially MRI would help us to rule out, but would not confirm the diagnosis."}, "2": {"exist": true, "char_ranges": [[0, 103]], "word_ranges": [[0, 19]], "text": "The answer is 2, an EMG, which would be the diagnostic test to confirm the diagnostic suspicion of ALS,"}, "3": {"exist": true, "char_ranges": [[104, 213]], "word_ranges": [[19, 39]], "text": "the imaging tests both CT, but especially MRI would help us to rule out, but would not confirm the diagnosis."}, "4": {"exist": true, "char_ranges": [[104, 213]], "word_ranges": [[19, 39]], "text": "the imaging tests both CT, but especially MRI would help us to rule out, but would not confirm the diagnosis."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The answer is 2, an EMG, which would be the diagnostic test to confirm the diagnostic suspicion of ALS, the imaging tests both CT, but especially MRI would help us to rule out, but would not confirm the diagnosis.", "full_answer_no_ref": "The answer is [HIDDEN], an EMG, which would be the diagnostic test to confirm the diagnostic suspicion of ALS, the imaging tests both CT, but especially MRI would help us to rule out, but would not confirm the diagnosis.", "full_question": "A 65-year-old woman consults for weakness in the right hand that has spread in a few months to other muscle territories of both arms and legs, with distal predominance. On examination there is atrophy and fasciculations in different metameric territories with preserved sensitivity. There is a bilateral Babinski's sign, what is the diagnostic test that would confirm the suspected diagnosis?", "id": 454, "lang": "en", "options": {"1": "Cerebral CT.", "2": "Electromyographic study.", "3": "Cerebral MRI.", "4": "Multimodal evoked potentials.", "5": NaN}, "question_id_specific": 153, "type": "NEUROLOGY", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0091_19675", "title": "[A case presenting manifestations of bulbospinal muscular atrophy with senile onset, rapid progression and marked asymmetry].", "score": 0.01686176142697882, "content": "A 64-year-old man was admitted to our department because of muscle cramp, atrophy and weakness of the limbs together with difficulty in walking, which had gradually progressed from age 60. About 1 year prior to admission, he had noticed hand tremor and gynecomastia. On admission, neurological examination revealed diffuse muscle atrophy and weakness of the extremities, which were more obvious on the right side with preponderance in the right leg. Bilateral postural hand tremor was also more prominent on the right hand. Fasciculations were observed both in the extremities and tongue. The remaining cranial nerves and cerebellar functions were intact. Sensation was normal except for slightly decreased vibratory sense in the distal part of the legs. Deep tendon reflexes including jaw jerk were increased with the exception of hyporeflexia of the right leg. Babinski sign was negative bilaterally. Blood examination disclosed slight elevation of CK and fasting glucose level of 110 mg/dl. Glucose tolerance test showed a diabetic pattern. CSF examination showed total protein of 74 mg/dl and IgG of 12 mg/dl. On a series of endocrinological studies, there was no significant elevation of androgen and estrogen both in serum and urine except for slight elevation of serum E1 level. Serum LH and FSH, however, were markedly high, which responded far beyond the normal range following to 0.1 mg injection of LH-RH. These results suggested that gynecomastia might be caused by dysfunction of the hypothalamus-hypophysis system. Brain CT and spine MRI showed no abnormality. Muscle biopsy obtained from the right quadriceps femoris revealed neurogenic abnormalities.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en106_36413", "title": "Progressive muscular atrophy", "score": 0.015648116535791512, "content": "Signs and symptoms As a result of lower motor neuron degeneration, the symptoms of PMA include: muscle weakness muscle atrophy fasciculations Some patients have symptoms restricted only to the arms or legs (or in some cases just one of either). These cases are referred to as flail limb (either flail arm or flail leg) and are associated with a better prognosis. Diagnosis PMA is a diagnosis of exclusion, there is no specific test which can conclusively establish whether a patient has the condition. Instead, a number of other possibilities have to be ruled out, such as multifocal motor neuropathy or spinal muscular atrophy. Tests used in the diagnostic process include MRI, clinical examination, and EMG. EMG tests in patients who do have PMA usually show denervation (neuron death) in most affected body parts, and in some unaffected parts too. It typically takes longer to be diagnosed with PMA than ALS, an average of 20 months for PMA vs 15 months in ALS."}, {"id": "pubmed23n0306_14025", "title": "[The diagnosis of amyotrophic lateral sclerosis supported by motor evoked potential and brain MRI studies].", "score": 0.015306716331044117, "content": "A 57-year-old man developed severe muscle weakness and atrophy of the upper extremities within a five-month period. Neurological examination revealed severe weakness and atrophy in the scapular muscles and proximal and distal muscles of the upper extremities. Fasciculations were also observed in the various muscles of the upper extremities. There was neither muscle weakness, atrophy nor fasciculation in either his face, neck muscles or lower extremities. He had no pseudobulbar or bulbar signs. Tendon reflexes were mildly hyperactive in the jaw and lower extremities, and normal in the upper extremities. There were no pathological reflexes, spasticity or sensory disturbances. The needle EMG study revealed denervation potentials in all muscles of the upper extremities examined. The nerve conduction study revealed no findings of the conduction block. Cervical spine X-rays revealed the narrowing of the spinal foramens at the left C3/C4 and bilateral C4/C5, C5/C6, and C6/C7 intervertebral levels. In addition, magnetic resonance imaging (MRI) revealed compressions of the cervical cord at C4/C5 and C5/C6 intervertebral levels. These clinical and neuroradiological findings resembled those of the cervical spondylotic amyotrophy (CSA). However, the motor evoked potential (MEP) study revealed the pyramidal tract dysfunction above the levels of the pyramidal decussation. Furthermore, brain MRI revealed abnormal foci in both internal capsules which were characterized by hyperintense relative to cortical gray matter on T2-weighted images and still hyperintense to white matter on proton-density-weighted images. In addition, T2-weighted images demonstrated a low signal within the motor cortex and hyperintense lesions in the white matter of the precentral gyri. These MRI findings indicated the degeneration of the pyramidal tract and corresponded to those found in the patients with amyotrophic lateral sclerosis (ALS) which have been recently reported. It has been difficult to distinguish ALS from CSA. However, MEP and brain MRI studies were useful for distinguishing these two diseases in this patient. In addition, this patient showed typical MRI findings suggesting the degeneration of the pyramidal tract, although this patient had a relatively short course of illness and did not show obvious physical findings suggesting pyramidal tract dysfunction."}, {"id": "pubmed23n0309_14143", "title": "[Neurological CPC.57. An 80-year-old woman with four years history of muscle atrophy involving lower extremities predominantly on the right side].", "score": 0.015220189133232611, "content": "We report an 80-year-old woman with progressive muscular atrophy predominantly involving her right lower extremity. She was well until 1992 (75 years of age) when she noted an onset of weakness in her right leg which had got progressively worse. She was admitted to our service in July 1994. On admission, general physical examination was unremarkable. She was alert and well oriented without dementia. Higher cerebral functions were normal. Cranial nerves also appeared intact. She dragged her right leg in walking. Mild to moderate weakness (2/5 to 4/5) was noted in muscles in her right lower extremity more in the distal part. Deep tendon reflexes were within normal limits, and the plantar response was flexor bilaterally. Sensation was intact. Laboratory examinations were also unremarkable except for slight increase in CK which was 470 IU/l. CSF was also normal. EMG revealed neurogenic changes in the lower extremities. She was admitted to Aoki Hospital on October 21, 1994, by that time, her weakness in the right lower extremity had gotten worse in that the muscle strength of the right extensor hallucis longus was 0 and tibialis anterior 2; muscle atrophy was also prominent in her right leg; the right ankle jerk could not be elicited. In the subsequent course, weakness and atrophy appeared in her left lower extremity, however, upper extremities and cranial nerves had never been affected. Babinski sign was always negative. In February 1996, she developed delusional ideation of self persecution, and showed difficulty in communication with medical staffs. She developed fever of 38.7 degrees C on June 13, 1996 expired on the next day. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that the patient had a form of spinal muscular atrophy. Opinions were divided between ALS and spinal muscular atrophy. Post-mortem examination revealed marked loss of anterior horn neurons in the lumbar area with astrogliosis. Bunina bodies were seen in some of the remaining neurons. No myelin pallor was noted in the pyramidal tracts, however, atrophy and loss of Betz cells were noted in the motor cortex. Other cortical areas were unremarkable. The neuropathologist arrived at the conclusion that the patient had ALS. This patient was unique in that she had asymmetric atrophy and weakness limited to the lower extremities. This is quite unusual as ALS of four years duration. In addition, the patient developed some mental change which was thought to represent dementia by some participants. But no clear morphologic changes were seen to account for her mental change."}, {"id": "pubmed23n0377_18350", "title": "[Spinal meningioma as differential diagnosis of diabetic polyneuropathy].", "score": 0.015148247978436658, "content": "A 70 year old woman had suffered from diabetes mellitus type 2 since she was 52. Three years before the surgery she had begun to experience weakness together with altered sensitivity in the right leg, which was regarded as having been caused by diabetic polyneuropathy. During the admission examination the level for algesia on the right-hand side was at about D 11, a distal paraparesis of the leg (3-4 degrees, Janda's classification), more intense on the right, hyperactive deep tendon reflexes, Babinski's reflex on both sides, and depressed abdominal cutaneous reflexes. The sensitivity to vibrations on the Malleolus medialis on both sides was 0/8. The patient could walk only with the help of a Rollator. Over the three-year period following onset of symptoms the following tests were carried out: motor nerve conduction speeds of the N. tibialis and N. peronaeus, electromyogram of the N. tibialis anterior and the M. gastrocnemius, somatosensory evoked potentials (SSEP) of the N. tibialis, which indicated a lesion in the peripheral nerves or nerve roots. Cranial computed tomography (CCT), CT scan of the lumbar spine (L3-S1) and angiological investigation elicited no significant pathological findings. An MRI of the thoracal spine showed a vertebra-sized dorsal tumor pressing on the spinal cord from left to right. By means of microsurgery the spinal tumor was completely removed. Suspected meningeoma was confirmed by histological analysis. During the post-surgical period, the incomplete paraplegia quickly regressed, and 7 weeks after the removal of the spinal meningeoma the patient was able to climb stairs. In case of slowly-developing paresis of the legs in diabetic patients, diabetic polyneuropathy should not be diagnosed without careful consideration, and rare spinal tumors should be considered as part of the differential diagnosis, especially if the blood glucose level is normal, and intensive physiotherapy brings no improvement in the patient's condition."}, {"id": "pubmed23n0566_18451", "title": "[Familial spastic paraplegia with severe amyotrophy of the hands. (Silver syndrome?)].", "score": 0.012490450725744843, "content": "Familial spastic paraplegia (FSP) with severe muscular atrophy of hands and feet is exceptional. Autosomal dominant forms were initially described by Silver in 1966. We report two cases, from the same Tunisian family, presenting FSP with severe amyotrophy of the hands. A brother and his sister, aged respectively 37 and 36 years old, presented practically the same clinical picture. Their parents were cousins. The female patient was hospitalized. Both patients developed gait disorders around the age of three years. Muscular atrophy of the hands arose much later, around the age of 20 years. The neurological examination disclosed a spastic gait with distal amyotrophy, severe in the hands and moderate in the feet. Sensitivity was preserved and there was no fasciculation. The spinal cord and cerebral MRI was normal. Electromyography (EMG) showed a neurogenic pattern in the distal muscles. Stimulation of the median, ulnar and sciatica nerves was ineffective. The somatosensory evoked potentials (EP) were delayed (upper limb) or desynchronised (lower limb). The auditory and visual EP were normal. The cerebrospinal fluid contained 1 mononuclear cell/mm3 and 10 mg protein/100 ml. Abnormalities of the cranio-vertebral junction, Arnold-Chiari malformation, syringomyelia and familial juvenile amyotrophic lateral sclerosis (ALS) were excluded and the diagnosis of Silver's syndrome was evoked."}, {"id": "pubmed23n0375_12867", "title": "[A patient with motor neuron syndrome clinically similar to amyotrophic lateral sclerosis, presenting spontaneous recovery].", "score": 0.012398280362352219, "content": "We report a patient with motor neuron syndrome similar to amyotrophic lateral sclerosis (ALS) and with spontaneous recovery. At the age 40, the woman developed progressive muscular weakness, atrophy and fasciculation in extremities. She also noted a dyspnea, tongue atrophy and dysphagia. A neurological examination 6 months after onset revealed i) a tongue atrophy and fasciculation, ii) diffuse muscule weakness and atrophy in face, neck and extremities, and iii) marked hyperreflexia in the four limbs and bilateral Babinski reflex, but iv) neither sensory disturbance nor ophthalmoplegia. Electromyogram (EMG) detected such denervation potentials as fibrillation potentials, fasciculation potentials, positive sharp waves and polyphasic or giant MUPs diffusely in the limb muscles. Peripheral nerve conduction study detected neither conduction block nor delay. Thus, she was diagnosed as suffering from ALS. However, since approximate 1 year after onset, her muscle weakness has gradually been getting better. Simultaneously, the dyspnea and dysphagia gradually improved. Two years after onset, an EMG examination detected chronic denervation potentials in the left musculus sternocleidomastoideus and a few on-going denervation potentials in the left musculus extensor carpi radialis, but no denervation potentials in other limb muscles. Fasciculation potentials were found in tongue muscles. Thus, the present case was thought to have a reversible motor neuron syndrome clinically quite similar to ALS. A mild increase in IgE (346 U/ml) and a low-titer IgM-class anti-GM1 antibody were found in her serum though its pathological significance was uncertain. Any immunological aberrance may account for the pathogenesis. It should be noted that clinically diagnosed cases of ALS may rarely recover spontaneously."}, {"id": "pubmed23n0701_4715", "title": "An unusual cause of dementia: essential diagnostic elements of corticobasal degeneration-a case report and review of the literature.", "score": 0.011223795683631192, "content": "Corticobasal degeneration (CBD) is an uncommon, sporadic, neurodegenerative disorder of mid- to late-adult life. We describe a further example of the pathologic heterogeneity of this condition. A 71-year-old woman initially presented dysarthria, clumsiness, progressive asymmetric bradykinesia, and rigidity in left arm. Rigidity gradually involved ipsilateral leg; postural instability with falls, blepharospasm, and dysphagia subsequently developed. She has been previously diagnosed as unresponsive Parkinson's Disease. At our clinical examination, she presented left upper-arm-fixed-dystonia, spasticity in left lower limb and pyramidal signs (Babinski and Hoffmann). Brain MRI showed asymmetric cortical atrophy in the right frontotemporal cortex. Neuropsychological examination showed an impairment in visuospatial functioning, frontal-executive dysfunction, and hemineglect. This case demonstrates that association of asymmetrical focal cortical and subcortical features remains the clinical hallmark of this condition. There are no absolute markers for the clinical diagnosis that is complicated by the variability of presentation involving also cognitive symptoms that are reviewed in the paper. Despite the difficulty of diagnosing CBD, somatosensory evoked potentials, motor evoked potentials, long latency reflexes, and correlations between results on electroencephalography (EEG) and electromyography (EMG) provide further support for a CBD diagnosis. These techniques are also used to identify neurophysiological correlates of the neurological signs of the disease."}, {"id": "article-16985_11", "title": "La Belle Indifference -- History and Physical", "score": 0.011097768825534551, "content": "Hoover's sign (63% Sensitivity & 100% specificity): This test is commonly used to separate organic from the nonorganic cause of weakness or paralysis. An examiner's hand is placed below the heel of the affected leg, and the patient is asked to flex the hip of the normal leg against resistance. In organic disorders, there should not be any pressure on the examiner's hand on the affected side, while pressure is felt in patients with FNSD/CD. [20] Variable Strength (63% sensitivity and 97% specificity): The weakness is inconsistent with variable force at different locations."}, {"id": "Neurology_Adams_1712", "title": "Neurology_Adams", "score": 0.01102958250127711, "content": "In patients with neurologic signs, nerve conduction studies disclose reduced amplitude of the ulnar sensory potentials. There may be decreased amplitude of the median motor evoked potentials as well, a mild but uniform slowing of the median motor conduction velocity, and a prolongation of the F-wave latency. Concentric needle examination of affected hand muscles reveals large-amplitude motor units, suggesting collateral reinnervation. Somatosensory evoked potentials may be a useful adjunct to the conventional nerve conduction and EMG studies (Yiannikas and Walsh). Brachial artery MR angiography is usually reserved for patients with a suspected arterial occlusion, an aneurysm, or an obvious cervical rib. The place of venography in the diagnostic workup is uncertain, for a number of otherwise normal individuals can occlude the subclavian vein by fully abducting the arm."}, {"id": "Neurology_Adams_10161", "title": "Neurology_Adams", "score": 0.010686095931997572, "content": "The biceps and brachioradial reflexes on one or both sides may be depressed, sometimes in association with an increase in the triceps and finger reflexes. The hand or forearm muscles may undergo atrophy; in a few cases, the atrophy of hand muscles is severe. In such cases, the spondylotic compression, as judged by MRI or CT myelography, may be confined to the high cervical cord, well above the levels of the motor neurons that innervate these muscles. In patients with sensory loss, pain and thermal sensation often appear to be affected more than tactile sense. An unexpected Babinski sign has already been mentioned and a few fasciculations may be seen, especially in proximal arm muscles. Another unusual feature in advanced stages of cervical cord compression is the appearance of mirror movements of the hands, in which effortful attempts to make refined movements of the fingers of one hand, causes the opposite hand to move similarly."}, {"id": "Neurology_Adams_452", "title": "Neurology_Adams", "score": 0.010252348579851715, "content": "Monoplegia with Muscular Atrophy This is more frequent than monoplegia without muscular atrophy. Long-continued disuse of one limb may lead to atrophy, but it is usually of lesser degree than atrophy caused by lower motor neuron disease (denervation atrophy). In disuse atrophy, the tendon reflexes are retained and nerve conduction studies are normal. With denervation of muscles, there may be visible fasciculations and reduced or abolished tendon reflexes in addition to paralysis. The location of the lesion (in nerves, spinal roots, or spinal cord) can usually be determined by the pattern of weakness, by the associated neurologic symptoms and signs, and by special tests—MRI of the spine, examination of the cerebrospinal fluid (CSF), and electrical studies of nerve and muscle. If the limb is partially denervated, the EMG shows reduced numbers of motor unit potentials (often of large size) as well as fasciculations and fibrillations."}, {"id": "pubmed23n0408_22841", "title": "Tests of motor function in patients suspected of having mild unilateral cerebral lesions.", "score": 0.009900990099009901, "content": "Though various textbooks describe clinical manoeuvres that help detect subtle motor deficits, their sensitivity, specificity and predictive values have not been determined. We investigated the sensitivity, specificity and predictive values of various manoeuvres in order to determine the most sensitive and reliable test or combination thereof. Straight arm raising (Barré), pronator drift, Mingazzini's manoeuvre, finger tap, forearm roll, segmental strength and deep tendon reflexes were tested in 170 patients with (86) and without (84) a proven lesion in the motor areas confirmed by computed tomography. Segmental motor strength bad good specificity (97.5%) but poor sensitivity (38.9%) and negative predictive value (NPV) (58.7%). The forearm roll had a similar profile. Finger tap had a sensitivity of 73.3% and a specificity of 87.5%. Barré and pronator testing had a sensitivity and specificity of 92.2% and 90.0% respectively. Hyperreflexia had a sensitivity of 68.9% and a specificity of 87.5%. An abnormality of pronator, reflexes or finger tap had a sensitivity of 97%, and when these three tests were positive, specificity was 97%. When all six tests were positive, the positive predictive value was 100%, when all six tests were negative the NPV was 100%. The detailed segmental examination has very good specificity for detecting motor deficits, but the sensitivity and NPV are unacceptably low. Pronator drift with finger tap and reflexes is the most reliable and time-effective combination of tests for the detection of subtle motor lesions, and could replace the segmental motor examination as a screening for motor lesions."}, {"id": "article-40985_10", "title": "How to Localize Neurologic Lesions by Physical Examination -- Clinical Significance", "score": 0.009846205634217668, "content": "Examination of the motor system of a limb includes checking for muscle bulk and fasciculation, muscle tone at joints, the power of muscle groups, deep tendon reflexes, clonus, plantar response, and coordination. In cases of a lower motor neuron type weakness, there is early muscle wasting, fasciculations, hypotonia, hyporeflexia, and a normal plantar response. On the other hand, the upper motor neuron type of weakness is characterized by normal muscle bulk, hypertonia, hyperreflexia, clonus, and an extensor plantar response (positive Babinski’s sign). Furthermore, the preservation of deep tendon reflexes distinguishes myopathy from neuropathy."}, {"id": "pubmed23n0883_13259", "title": "Hirayama's disease: an Italian single center experience and review of the literature.", "score": 0.00980392156862745, "content": "Hirayama's disease (HD), is a benign, self-limited, motor neuron disease, characterized by asymmetric weakness and atrophy of one or both distal upper extremities. In the present study we report the clinical, electrophysiological and MRI features of a group of Italian patients, with review of the literature. Moreover we propose an optimized MRI protocol for patients with suspected or diagnosed HD in order to make an early diagnosis and a standardized follow up. Eight patients with clinical suspicion of Hirayama disease underwent evaluation between January 2007 and November 2013. All patients underwent standard nerve conduction studies (NCS), electromyography (EMG) and motor/sensory evoked potentials (MEP/SEP). Cervical spine MRI studies were conducted with a 1.5 Tesla MRI scanner in neutral and flexion position, including sagittal T1-weighted sequences and sagittal and axial T2-weighted sequences. The following diagnostic features were evaluated: abnormal cervical curvature, localized cervical cord atrophy in the lower tract (C4-C7), presence of cord flattening (CF), intramedullary signal hyperintensity on T2 weighted sequences, anterior shifting of the posterior wall of the cervical dural sac (ASD) and presence of flow voids (EFV) in the posterior epidural space during flexion. All patients complained of weakness in hand muscles as initial symptoms, associated with hand tremor in three of them and abnormal sweating of the hand palm in two of them. No sensory deficits and weakness at lower limbs were reported by any patients. Distal deep tendon reflexes at upper limbs were absent in all patients with the absence of the right tricipital reflex in one of them. Deep tendon reflexes at lower limbs were normal and no signs of pyramidal tract involvement were present. The clinical involvement at onset was unilateral in six patients (three left-sided and three right-sided) and bilateral asymmetric in two of them, with the right side more affected. With the progression of the disease all patients but one experienced weakness and wasting of hand muscles and forearm bilaterally, but still asymmetric. The duration of the progression phase of the disease ranged from eight months to three years. In all patients, NCS and EMG findings were consistent with a spinal metameric disorder involving the C7-T1 myotomes bilaterally; sensory conduction and electrophysiologic features at lower limbs were normal. MEP and SEP were normal and we did not observe the disappearance of the spinal potential during the neck flexion in any of the patients. MRI is the best diagnostic tool in the diagnosis of HD; it can confirm clinical diagnosis and exclude other conditions responsible for the neurological deficits leading to a correct patient management and therapy, limiting arm impairment. On MRI all patients had loss of the normal cervical lordosis (100%). Five patients had loss of attachment of posterior dural sac and anterior dural shift on flexion MRI with presence of flow voids from venous plexus congestion (62.5%); three patients had no anterior dislocation of the dural sac and no epidural vein congestion. Two patients showed localized cord atrophy, one at C5-C6 and the other at C6-C7 level (25%). Three patients had T2 intramedullary hyperintensities (37.5%) and cord flattening (CF) was present in 5 patients of 8 (62.5%). HD is a rare entity and a self-limited condition, but it has to be early differentiated from other diseases that may determine myelopathy and amyotrophy to establish a correct therapy and limit arm impairment. MRI is very important to confirm the clinical suspect of HD and a standardized MRI protocol using axial and sagittal images in both neutral and flexing position is needed, in order to diagnose and follow up affected patients."}, {"id": "pubmed23n0389_20579", "title": "[Regarding the clinical diagnosis of the  monotopical  spinal forms of multiple sclerosis. The value of the fan sign in the adult].", "score": 0.00980392156862745, "content": "We wish to discuss the value of the clinical history and examination in orientation of the diagnosis of probable multiple sclerosis (MS). We report the two year study of a woman who over the previous ten years had had three episodes of paraesthesia, with pins and needles in her left leg and other parts of the left side of her body, although never affecting head or neck. She also complained of tiring more than usual. In an outpatient clinic she was found to have a syndrome affecting the upper segments of the spinal cord, mainly involving the right side and resembling an incomplete Brown Sequard type syndrome. There were increased clinical muscle and deep reflexes. The most marked was that of the right deltoid (C5), bilateral fanning of the toes when the Babinski reflex was tested, Barré positive in the right leg, pins and needles and dysaesthesia on the left to an undetermined level. Function was well preserved when compared with the clinical signs found. The case was considered to be of monotopical MS. Spinal magnetic resonance findings confirmed the clinical diagnosis. We emphasise the value of careful clinical investigation directed towards the diagnosis of probable MS. We draw attention to the diagnostic value of the dissociation between the severe clinical alterations and the functional performance, which was surprisingly well maintained. Also we report the originality of the presence of bilateral fanning sign supporting the diagnosis of MS, occurring in a disease of adult life."}, {"id": "pubmed23n0255_13290", "title": "[A 54-year-old man with progressive proximal muscle atrophy and gynecomastia].", "score": 0.009708737864077669, "content": "We report a 54-year-old man with progressive proximal muscle atrophy and gynecomastia. The patient had an insidious onset of weakness in his lower extremities at age 14, in that he noted a difficulty in standing up from a chair. Soon after he noted some difficulty in climbing up stairs. At age 35, he noted weakness in his arms; his weakness slowly progressed in that he became unable to walk or stand alone before 40 years of age. He also noted gynecomastia at that age. He was admitted to our hospital for the work up on September 16, 1993, when he was 54-year-old. On admission, he was alert and oriented; his BP was 150/70 mmHg; he had bilateral gynecomastia, however, no other skeletal deformities were found. On neurologic examination, he was mentally sound without dementia, and his higher cerebral functions were normal. Cranial nerves also appeared intact without facial atrophy, dysarthria, or dysphagia; no atrophy was noted in the tongue. He had marked muscle atrophy in both upper and lower extremities more marked in the proximal portions; muscle strength was approximately in the range of 2/5 to 3/5 in the proximal parts, and 4/5 in the distal parts in both upper and lower extremities. No fasciculation was noted; muscle tone was flaccid; no ataxia was present. Deep reflexes were either lost or markedly diminished. No Babinski sign was noted. Sensation was intact. Laboratory examination revealed normal blood counts; serum CK was slightly increased to 131 IU/l; ECG showed complete right bundle branch block; EMG revealed no active units in the right biceps brachii, deltoid, quadriceps femoris, and triceps surae muscles; in other muscles tested, motor unit potentials of low amplitude and short duration were seen; in the right tibialis anterior muscle, however, motor unit potentials with an amplitude up to 6 m V were also seen. Nerve conduction velocities were normal. A diagnostic procedure was performed. He was discussed in the neurological CPC, and the chief discussant arrived at the conclusion that this patient had Becker type of progressive muscular dystrophy. In her differential diagnosis, the possibility of Kennedy-Alter-Sung syndrome was discussed because this patient had gynecomastia. However, the discussant excluded that possibility because of absence of both bulbar symptoms and typical neurogenic changes in his EMG. The diagnostic procedure was a muscle biopsy on the left tibialis anterior muscle. Histologic observation on HE stained specimens revealed marked inequality in the muscle fiber diameters, increase in endomysial nuclei, proliferation of connective tissue, and fiber splitting.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n1126_11880", "title": "Distal Acquired Demyelinating Symmetric Neuropathy Associated with Decreased Electrical Excitability of the Femoral Nerves.", "score": 0.009708737864077669, "content": "<bIntroduction:</b There are many phenotypic variants of chronic inflammatory demyelinating polyneuropathy. <bMethods:</bAn Ancient Greek aryvallos painted c. 480-450 BC, now on display at the Louvre museum, was meticulously studied regarding its painted surface, which presents an outpatient clinic in Ancient Greece. Other Ancient Greek works of art presenting medical activities have been also evaluated in order to reach informed conclusions regarding medical practice of that period. <bCase report:</b We report a rare case of the distal phenotype of chronic inflammatory demyelinating polyneuropathy with a subacute onset and rapidly progressive course. A 58-year-old male had distal, symmetric, predominantly motor impairment without ataxia and tremor. After a three-month duration of the disease, the patient had already complete paresis of the feet with absence of compound muscle action potentials (CMAPs) over the feet and lower leg muscles, but preserved proprioception and sural sensory nerve action potential. Cerebrospinal fluid protein level was elevated to 3.4 g/L. Demyelinating neuropathy was predominantly in the proximal segment of the nerves. Low amplitude of CMAPs was recorded hardly over the vastus medialis and rectus femoris muscles, while weakness and atrophy in these muscles were not. The patient was refractory to treatment. He died three years after disease onset. <bConclusion:</bWe described a new clinical-electrophysiological phenomenon, which was characterized as a decrease in the evoked electrical excitability at the femoral nerve stimulation site (decreased CMAP), while the natural physiological conduction of the impulse from the motor neuron to the muscle was not blocked (preserved muscle strength)."}, {"id": "pubmed23n0848_24134", "title": "Characteristics of C6-7 myelopathy: assessment of clinical symptoms and electrophysiological findings.", "score": 0.009615384615384616, "content": "This is a single-center retrospective study. The objective of this study was to study the clinical symptoms and electrophysiological features of C6-7 myelopathy. This study was conducted at the Department of Orthopedic surgery, Yamaguchi University Graduate school of medicine, Japan. A total of 20 patients with cervical compressive myelopathy were determined by spinal cord-evoked potentials or a single level of obvious magnetic resonance imaging (MRI)-documented cervical spinal cord compression. Neurological examinations included manual muscle testing and investigation of deep tendon reflex, including Hoffmann sign, and of sensory disturbance areas. Motor-evoked potentials (MEPs), compound muscle action potentials (CMAPs) and F-wave were recorded from bilateral abductor digit minim and abductor halluces muscles. Central motor conduction time was calculated as follows: MEPs latency-(CMAPs latency+F latency-1)/2 (ms). Eighteen patients (90%) had negative Hoffmann sign. Eight patients (40%) had no sensory disturbance in the upper limbs and 8 patients (40%) had no muscle weakness in the upper limbs. We determined that patients had cervical myelopathy when their central motor conduction time measured in abductor digit minim was longer than 6.76 ms (+2 s.d.). Using this definition, the sensitivity for myelopathy was 42.8%. Patients with C6-7 myelopathy may lack clinical symptoms in their hands and central motor conduction time measured in abductor digit minim tended to be less prolonged, and it only showed symptoms in their lower limbs as gait disturbance. Surgeons should bear in mind the possibility of disorders of caudal C6-7 when they encounter patients with no or few symptoms in their hands and with leg weakness or numbness."}, {"id": "pubmed23n0259_1319", "title": "[A 65-year-old woman with dysarthria, dysphagia, weakness, and gait disturbance].", "score": 0.009523809523809525, "content": "We report a 65-year-old woman with progressive dysarthria, dysphagia, weakness, and gait disturbance. The patient was well until 59 years of age (January of 1986) when she noted bilateral ptosis. One year later, she noted a gradual onset of difficulty in speech (articulation). Her speech slowly deteriorated and she noted weakness in chewing power and difficulty in swallowing in addition. In October 1987, she developed emotional incontinence. In January of 1988, she started to drag her left foot. She was admitted to our hospital on June 13 of 1988. On admission, she was alert and general physical examination was unremarkable. Neurologic examination revealed no dementia; her higher cerebral functions appeared intact. Ptosis was present bilaterally more on the right. She showed difficulty in opening her eyes on command; no contraction of the frontal muscles was seen upon attempted eye opening. There was a moderate limitation in the vertical gaze. Forced laughing and crying were seen. Facial muscles were moderately weak without apparent atrophy. The movement of the soft palate was very weak, and swallowing disturbance was more prominent for liquid staff. The tongue appeared somewhat small, however, no fasciculation was noted. Her step was small and the posture was stooped. Retropulsion was present, however, Romberg's sign was absent. No muscle atrophy was apparent, however, diffuse mile to moderate muscle weakness was noted in all four limbs. Cerebellar sign was absent. Deep tendon reflexes were exaggerated bilaterally, and Babinski sign was present on the left side. Sensation was intact. Routine blood tests were unremarkable as was a cranial CT scan. Her ptosis did not improve after 10 mg of edrophonium injection. CSF was also normal. She was transferred to another hospital but her neurological disabilities further progressed. In 1989, she was totally unable to move her limbs; she could only move her eyes; still consciousness was clear without dementia. She developed respiratory difficulty and expired on July 25, 1992. She was discussed in a neurological CPC, and the opinions were divided into ALS and primary lateral sclerosis (PLS). The chief discussant arrived at the conclusion that the patient might have had the pyramidal form of ALS. Postmorten examination revealed marked myelin pallor in the anterior as well as lateral corticospinal tracts. Pyramidal tract degeneration was prominent starting at the level of the cerebral peduncle and was continued to be seen until the level of lumbar cord. The number of anterior horn cells showed only slight decrease in the cervical level, however, it was normal in the lumbar cord.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0018_10473", "title": "[Value of preoperative investigations in the so-called \"cervical myelopathy\" (author's transl)].", "score": 0.009523809523809525, "content": "The particular value of clinical, radiological and electromyographical features is compared in 42 patients with motor deficit related to cervicarthrosic myelopathy or amyotrophic lateral sclerosis. The initial onset of the disease was identical (motor deficit and long tracts pathways involvement). Three different groups were identifyed according to the evolution: -- Group I: (13 cases): true lateral amyotrophic sclerosis which were not operated on. -- Group II (10 cases): myelopathy called \"cervicarthrosic\" because of radiological findings which were operated on but had the same steady worsened course as a lateral amyotrophic sclerosis. -- Group III (19 cases): cervical myelopathy which had surgery. The operation brought about stabilization or fairly good recovery over the 18 months following at least. From a clinical aspect, the \"Lhermitte sign\" or objective sensitive deficit are strongly significant for cervical myelopathy. On the contrary, diffuse fasciculations specially in the tongue seem to be mostly found in lateral amyotrophic sclerosis, whereas they are restricted into the paralysed area in cervical myelopathy. Electromyographic examination is decisive: simple activity with high frequency motor units (increased amplitude and polyphasic waves) or \"preponderant potentials\" into a cranial nerve territory or three segments of the lower limbs are frequently found in lateral amyotrophic sclerosis. These electromyographic features are less significant in the upper limbs. The neuroradiological findings lonely cannot assert definitely the cervicarthrosic origin of the myelopathy but visualize the conflicting situation between the spinal cord and the cervical canal and allow to choose the surgical procedure."}, {"id": "pubmed23n0345_20471", "title": "Clinical utility of reflex studies in assessing cervical radiculopathy.", "score": 0.009433962264150943, "content": "We prospectively studied the diagnostic utility of upper limb segmental reflexes in patients with suspected cervical radiculopathy (CR). Fifty-three patients (29 men and 24 women), referred for electrodiagnostic testing, were positive for at least one of four clinical criteria for CR: abnormal (1) history, (2) motor (myotomal) examination, (3) sensory (dermatomal) examination, and (4) changes in deep tendon reflexes (DTR). All underwent electrodiagnostic assessment, needle electrode examination (NEE), specialized segmental reflexes (heteronymous and Hoffman's reflexes [H reflexes]), and neuroimaging. The clinical diagnosis was supported in all 32 patients who entered the study with two or more clinical signs for CR. Abnormal NEE was found in 90% of subjects with three clinical signs, 59% with two signs, and only 10% of those with one sign. H reflexes demonstrated a sensitivity of 72% and specificity of 85% for detection of CR and were particularly helpful when forming conclusions in the 21 subjects with only one clinical sign for CR. Specialized segmental H-reflex studies of the upper limb were as sensitive and specific as neuroimaging (magnetic resonance imaging)."}, {"id": "pubmed23n0295_9154", "title": "[A 63-year-old woman with muscle weakness, myotonia, and parkinsonism].", "score": 0.009345794392523364, "content": "We report a 63-year-old woman who presented myotonia and parkinsonism. The patient was well until 15 years of the age when she noted that the ring finger of her left hand at times flexed when she did not intend to do so. She noted weakness in her left upper extremity at the age of 40, and difficulty in relaxing her hand grip at 45. She had an onset of tremor in her right foot at age 50, which was followed by difficulty in gait and hand writing. She was admitted to Juntendo University Urayasu Hospital when she was 63-year-old. Her mother, two sisters, and a son were affected with similar muscle weakness and myotonia. Although some of them developed stooped posture in the late stage of the disease, none of them had overt parkinsonism. General physical examination was unremarkable. Neurologic examination revealed an alert and oriented woman with some recent memory loss. She had bilateral ptosis, facial weakness, and a masked face. Myerson's sign was present. Her speech was small and monotonous. The sternocleidomastoid muscles were markedly atrophic and weak. The remaining of the cranial nerves were intact. She walked in small steps with freezing with support. She showed bradykinesia, retropulsion, and resting tremor in her right leg. Slight distal dominant weakness was noted in both upper and lower extremities more on the left. No cerebellar signs were noted. Muscle stretch reflexes were within normal limits in the upper extremities and diminished in the lower limbs. Sensation was intact. Routine laboratory findings were unremarkable. Cranial CT scan and MRI revealed slight cortical atrophy and leukoaraiosis. She responded to levodopa and she became able to walk by herself. She was transferred to another hospital one month after her admission. She had several bouts of airway obstruction with one episode of respiratory arrest. She expired six month after the transfer. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that this patient suffered from myotonic dystrophy and Parkinson's disease which set in later years. Postmortem examination on the iliopsoas muscle revealed uneven muscle fiber diameters, central nuclei, and type 1 fiber predominance; the pathologic finding was consistent with myotonic dystrophy. The substantia nigra showed marked cell loss and Lewy bodies in the remaining neurons. The finding was consistent with Parkinson's disease. In myelin stain, diffuse myelin pallor was noted in the cerebral white matter which was the pathologic substrate of leukoaraiosis in this patient. Combination of these two disorders have never been reported in the literature to our knowledge. It appears to be that the coincidence is just a by-chance phenomenon, but it seems interesting to note that accelerated aging process appears to be present in both myotonic dystrophy and Parkinson's disease."}, {"id": "pubmed23n0338_15048", "title": "[A case of subacute necrotizing lymphadenitis complicated with brachial plexus neuritis].", "score": 0.009345794392523364, "content": "A 22-year-old female noted a low grade fever and swelling of the cervical lymph nodes in May 1997, and later developed a dry cough. She was diagnosed to have interstitial pneumonitis, and then administration of corticosteroids alleviated her symptoms. On February 6, 1998, however, a high fever recurred and her swollen cervical lymph node on the right side was biopsied on February 9, 1998. A histological examination revealed an increased number of histiocytes and karyorrhexis of the lymphocytes in the paracortical areas, and she was therefore diagnosed to have histiocytic necrotizing lymphadenitis. She could not fully elevate her arm on February 16, 1998. On admission, her cervical lymph node was swollen on the left side. A neurological examination revealed a marked weakness of the right deltoid muscle, moderate weakness of the right latissimus dorsi, triceps and brachioradialis muscles and also a mild weakness of the serratus anterior, supra- and infra-spinatus, and biceps brachii muscles. The muscle power of the other muscles were normal and no muscle atrophy was evident. Winging of the right scapula was observed. The deep tendon reflexes were normal in all four limbs, and her sensation was also normal. No cerebellar sign was found. The Jackson, Spurling, Allen, Morley and Adson tests were all negative. ESR was mildly elevated to 18 mm/hr, but CRP was negative. RF, ANA and anti-SS-A and SS-B antibodies were positive, whereas LE-test, direct and indirect Coombs tests and other autoantibodies were negative. Needle EMG disclosed fasciculation potentials in the right triceps muscle and polyphasic waves in the right deltoid muscle. MRI showed gadolinium-enhancement of the right brachial plexus. Although an abnormal accumulation of gallium was detected in the right parotid and bilateral submandibular glands, no sicca symptoms were found and the Schirmer test findings were normal. Oral prednisolone (50 mg/day with gradual tapering) alleviated both her symptoms and the gadolinium-enhancement of the right brachial plexus. As a result, her right upper limb paresis was thus considered to have been caused by right brachial plexus neuritis, which was probably associated with histocytic necrotizing lymphadentis. Although acute cerebellar ataxia and meningitis have previously been reported to be complicated with histiocytic necrotizing lymphadenitis, this is the first report to describe the complication of peripheral neuritis with this condition."}, {"id": "pubmed23n0272_4893", "title": "[A 64-year-old man with recurrent blurred vision and an abdominal mass].", "score": 0.009259259259259259, "content": "We report a 64-year-old man with recurrent bouts of blurred vision who died after developing an abdominal mass. He was well until June of 1985 when he was 59-years-old when he had an acute onset of loss of vision in his right eye. He was treated by prednisolone with a complete remission. In August of 1986, he had another bout of blurring of vision in his left eye. Once he lost his left vision completely, from which he showed slow recovery. In January of 1987, he developed blurring of his right eye and loss of pain and touch sensation in his right leg. Since then he repeated loss of vision in his right or left eye five times, and he was admitted to our hospital in May of 1990. On admission, he was alert and oriented. General physical examination was unremarkable. Neurologic examination revealed bilateral optic nerve atrophy. He could not discriminate light or dark by either eye. Other cranial nerves were unremarkable. He could walk in a wide-base only with support; spasticity was noted in his left leg. Muscle strength was preserved. Deep reflexes were exaggerated in both legs with extensor plantar reflex bilaterally. Pain and touch sensation was decreased in the left leg by 30%, and vibration was diminished in both feet. Position sense was preserved. Routine blood counts and chemistries were unremarkable. Cranial MRI scans revealed multiple high-signal intensity lesions in both pontine bases, basal ganglia, thalami, and in the deep cerebral white matters. He was treated with oral prednisolone, plasmapheresis, lymphocytapheresis, and then immuran. His vision showed only slight recovery to discriminate light and dark. In October of 1990, slight weakness appeared in his both legs. In December of that year, he developed nausea, and a fiber colonoscopic study revealed a stenosis in the transverse colon. In March of 1991, he developed anemia and liver dysfunction. In July of that year, jaundice appeared, and his serum bilirubin was increased. In October, his leg weakness became more prominent, and his cranial CT scans at that time revealed a low density change in the right cerebellum in the right superior cerebellar artery territory; in addition, multiple low density spots were scattered to be seen in both cerebral hemispheres including the basal ganglia and thalamic areas with ventricular dilatation and cortical atrophy.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0281_16132", "title": "[Motor neuropathy with conduction blocks].", "score": 0.009259259259259259, "content": "A series of 4 patients with pure, chronic and progressive motor neuropathy whose main clinical characteristics were asymmetric and distal weakness of the upper limbs, myokymia and fasciculations is presented. There were no sensory impairment and amyotrophy was observed in only one case. This picture suggested the diagnosis of motor neuron disease (MND). However, neurophysiologic examination demonstrated the presence of multifocal conduction blocks (CB) of the motor axons which were preferentially located in the proximal nerve segments and always at points atypical to nerve compression. The peripheral sensitive conductions and the somatosensory evoked potentials were normal, even through the nerve segments where the CB were located. Since this is a treatable potentially reversible syndrome, this motor neuropathy with CB should be included in the differential diagnosis of MND."}, {"id": "pubmed23n0701_9548", "title": "Cerebral palsy masking spinal muscular atrophy.", "score": 0.009174311926605505, "content": "Spinal muscular atrophy (SMA) is an autosomal recessive anterior horn cell disease that results in progressive muscular weakness and atrophy without sensory involvement. A wide clinical spectrum that ranges from early death to essentially normal adult live exists. We describe a case of two 12 years olds, who represent two of three surviving non-identical quadruplets, born at 25 weeks gestational age. A diagnosis of hypotonic cerebral palsy (CP) was made in early childhood and early intervention services were initiated. At 3 years of age, MRI's showed white matter changes. Both briefly attained Gross Motor Functional Classification Scale (GMFCS) 3 status, but by 12 years of age their ambulatory abilities had decreased to Level 4. Physical Medicine and Rehabilitation (PM&amp;R) physicians were consulted. On exam, distal lower extremities atrophy, hypotonia, hyporeflexia, and muscle weakness were noted. Neither child had upper motor neuron signs or spasticity. Cognition was normal. Neuromuscular disorder was suspected and genetic testing confirmed spinal muscular atrophy in both patients. While prior MRI/CT demonstrated static encephalopathy, recognition of symptoms and signs consistent with neuromuscular disease should have led to a secondary diagnosis. Therapeutic and surgical treatment decisions may have differed. Fragmentation of care and lack of a comprehensive team approach also contributed to the delay in recognition of their dual diagnosis."}, {"id": "pubmed23n0803_8071", "title": "Hoover's sign: Clinical relevance in Neurology.", "score": 0.009174311926605505, "content": "Hoover's sign was described by Dr. Charles Franklin Hoover more than 100 years back to differentiate between the organic and functional weakness of pyramidal origin. This test is usually performed in the lower limbs and is valuable when on bedside one is not sure about the nature of hemiparesis. A subject with hemiparesis of organic cause while asked to flex the hip of normal leg against resistance will not exert pressure on the hand of examiner placed under the heel on the affected side while in hysterical weakness heightened pressure will be felt on the examiner's hand. The presumed genesis of this sign could be the crossed extensor reflex or the principle of synergistic contraction. It is a useful clinical test in differentiating functional and organic paresis with moderate sensitivity (63%) and high specificity (100%), but there are some limitations which should be kept in mind while evaluating a patient. "}, {"id": "pubmed23n1054_1778", "title": "Coronavirus Disease 2019-Related Acute Ischemic Stroke: A Case Report.", "score": 0.00909090909090909, "content": "Coronavirus disease 2019 (COVID-19) is an active worldwide pandemic with diverse presentations and complications. Most patients present with constitutional and respiratory symptoms. Acute ischemic stroke remains a medical emergency even during the COVID-19 pandemic. Here we present a case of a patient with COVID-19 who presented with acute ischemic stroke in the absence of common risk factors for cerebrovascular accidents. A 70-year-old male patient, with no prior comorbidities, presented to the emergency department (ED) with fever, cough, and shortness of breath for four days, and altered level of consciousness and right-sided weakness with the sensory loss for one day. On examination, the patient had a score of 8/15 on the Glasgow coma scale (GCS). There was a right-sided sensory loss and weakness in both upper and lower limbs with a positive Babinski's sign. The pulmonary examination was remarkable for bilateral crepitation. On blood workup, there was leukocytosis and raised c-reactive protein (CRP). D-dimer, ferritin, thyroid-stimulating hormone (TSH), vitamin B12, and hypercoagulability workup were normal. Transthoracic echocardiography was also normal. COVID-19 polymerase chain reaction (PCR) detected the virus. Chest x-ray showed infiltrations in the left middle and both lower zones of the lungs in the peripheral distribution. Computed tomography (CT) scan of the chest showed peripheral and mid to basal predominant multilobar ground-glass opacities. CT scan of the head showed a large hypodense area, with a loss of gray and white matter differentiation, in the left middle cerebral artery territory. Magnetic resonance imaging (MRI) of the head showed abnormal signal intensity area in the left parietal region. It appeared isointense on T1 image and hyperintense on T2 image. It also showed diffusion restriction on the diffusion-weighted 1 (DW1) image with corresponding low signals on the apparent diffusion coefficient (ADC) map. These findings were consistent with left middle cerebral artery territory infarct due to COVID-19. The patient was intubated in the ED. He was deemed unfit for thrombolysis and started on aspirin, anti-coagulation, and other supportive measures. Patients with COVID-19 should be evaluated early for neurological signs. Timely workup and interventions should be performed in any patient suspected of having a stroke to reduce morbidity and mortality."}, {"id": "pubmed23n0481_4860", "title": "Abductor sign: a reliable new sign to detect unilateral non-organic paresis of the lower limb.", "score": 0.00909090909090909, "content": "To test a new neurological sign, the \"abductor sign,\" which can distinguish between organic and non-organic leg paresis using synergic movements of the bilateral hip abductors. The subjects were 33 patients presenting with paresis of one leg, 17 of organic origin and 16 of non-organic origin (hysteria). To test the abductor sign, the examiner told the patient to abduct each leg, and opposed this movement with his hands placed on the lateral surfaces of the patient's legs. The leg contralateral to the abducted one showed opposite actions for organic paresis and non-organic paresis: for example, when the paretic leg was abducted, the sound leg stayed fixed in organic paresis, but moved in the hyperadducting direction in non-organic paresis. Hoover's sign was used for comparison in the same patients. The abductor sign gave the correct classification for all 33 cases. Hoover's sign was reliable if the results were carefully interpreted, but it was non-diagnostic for 16 patients because of strong hip extensors and in two because of strong hip flexors. Two patients with non-organic paresis succeeded in tricking the examiner by pretending full effort to lift the paretic leg. The abductor sign is a useful test to detect non-organic paresis, because (1) it is difficult for a hysterical patient to deceive the examiner, (2) the hip abductor is one of the most commonly involved muscles in pyramidal weakness, and (3) the results are easily visible as movement or non-movement of the unabducted leg."}, {"id": "pubmed23n1058_8240", "title": "Hirayama Disease Presenting as 4-Limb Paresthesia.", "score": 0.009009009009009009, "content": "Hirayama disease is a rare clinical entity that presents typically as a unilateral, slowly progressive arms weakness, mostly occurring in young men. We report a case of Hirayama disease in a 20-year-old man presenting with a 4-year history of progressive paresthesia starting in his left arm, progressing to the right arm 1 year later. Four months before the presentation, he experienced bilateral foot paresthesias. Examination revealed weakness of the abductor digiti minimi, hallux extension weakness, and postural tremor bilaterally. He had hypersensitivity to pinprick in both hands with ulnar and median distribution. Sensory examination in the legs was normal. He had a postural tremor in both hands, which worsened on neck flexion. Spinal fluid analysis, including oligoclonal band testing, was normal. Electromyography demonstrated bilateral chronic C7 and C8 radiculopathies. Laboratory tests were normal. Flexion-extension magnetic resonance imaging demonstrated laxity of the dura and ligamentum flavum, with compression of cervical cord, maximal at C5-C6 in neck flexion. Laxity of the posterior dura during neck flexion has been postulated to lead to asymmetric lower cervical cord atrophy. Involvement of all 4 limbs is rare, and the condition can be mistaken for progressive multiple sclerosis."}, {"id": "pubmed23n0708_12296", "title": "[A case of amyotrophic lateral sclerosis with bilateral vocal cord paralysis necessitating tracheotomy].", "score": 0.008928571428571428, "content": "Vocal cord movement disorders are increasingly recognized in patients with amyotrophic lateral sclerosis (ALS). We describe a patient with limb-onset ALS who developed vocal cord paralysis. A 74-year-old Japanese male consulted our clinic with a 6-month history of weakness in both arms. His family history was unremarkable. There were fasciculations and mild atrophy of the tongue and both arms. In the legs, muscle strength was almost normal but widespread fasciculations were present. All tendon reflexes were hypoactive and pathological reflexes were absent. Thereafter, he developed weakness of the legs and showed increased eating time. Babinski sign was positive bilaterally at this stage. The forced vital capacity dropped from 90% at the initial evaluation to 62% of the predicted value 14 months later. Two years after disease onset, the patient developed aspiration pneumonia with hoarseness and had difficulty clearing his throat of phlegm. Laryngoscopy demonstrated severe vocal cord paresis on both sides, particularly in the abductor muscles possibly leading to obstruction. Tracheotomy was performed because of the risk that the patient could choke to death. A review of the literature suggests that severe impairment of vocal cord abduction could be a prelude to sudden death in ALS. Follow up by laryngoscopic examination is necessary."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 163]], "word_ranges": [[0, 28]], "text": "The management of displaced intracapsular hip fracture in a young patient is surgical and should be done early to reduce the risk of avascular necrosis of the hip."}, "2": {"exist": true, "char_ranges": [[355, 486]], "word_ranges": [[58, 82]], "text": "This is a 25 year old patient so we have to pursue reduction and fixation avoiding replacement options (we discard option 2 and 4)."}, "3": {"exist": true, "char_ranges": [[85, 354]], "word_ranges": [[12, 58]], "text": "surgical and should be done early to reduce the risk of avascular necrosis of the hip. They are not talking about life threatening enough to postpone a necessary surgical procedure or to consider an external fixator following a damage containment policy so we discard option 3."}, "4": {"exist": true, "char_ranges": [[355, 486]], "word_ranges": [[58, 82]], "text": "This is a 25 year old patient so we have to pursue reduction and fixation avoiding replacement options (we discard option 2 and 4)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The management of displaced intracapsular hip fracture in a young patient is surgical and should be done early to reduce the risk of avascular necrosis of the hip. They are not talking about life threatening enough to postpone a necessary surgical procedure or to consider an external fixator following a damage containment policy so we discard option 3. This is a 25 year old patient so we have to pursue reduction and fixation avoiding replacement options (we discard option 2 and 4).", "full_answer_no_ref": "The management of displaced intracapsular hip fracture in a young patient is surgical and should be done early to reduce the risk of avascular necrosis of the hip. They are not talking about life threatening enough to postpone a necessary surgical procedure or to consider an external fixator following a damage containment policy so we [HIDDEN]. This is a 25 year old patient so we have to pursue reduction and fixation avoiding replacement options (we [HIDDEN]).", "full_question": "25-year-old patient, who suffers a motorcycle accident on a Friday night. He is taken to the emergency room and diagnosed with abdominal trauma (negative echo-fast), mild head trauma (Glasgow = 14) and a displaced intracapsular fracture of the right hip. Hemodynamically she is stable, what would be the treatment of choice?", "id": 541, "lang": "en", "options": {"1": "Reduction, open if necessary, and osteosynthesis of the fracture in the first 24-36 hours.", "2": "Total hip resurfacing arthroplasty on Monday on a scheduled basis.", "3": "Wait for improvement of the cranial trauma and schedule the following week a scheduled surgery consisting of reduction and osteosynthesis of the fracture.", "4": "Given the risk of non-union of these types of fracture, I would perform an emergency bipolar hip hemiarthroplasty.", "5": NaN}, "question_id_specific": 5, "type": "TRAUMATOLOGY", "year": 2021, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0613_1717", "title": "[Femoral neck fractures in patients over 50 years old].", "score": 0.016892373485388454, "content": "Despite many papers and instructional course lectures, therapeutic guidelines are not clearly defined about treatment of femoral neck fractures. The aim of this multicentric French symposium was to prospectively study the results of current therapeutic options in order to propose scientifically proven options. Three prospective studies were carried out in order to answer to these questions: (1) is it possible with anatomical reduction and stable fixation to lower the non union and osteonecrosis rate? (2) is functional treatment of Garden 1 fractures successful in more than 65 years patients? (3) what criteria are useful to choose the kind of arthroplasty for more than 65 years patients? For the 64 patients between 50 and 65 years old included in the first study, 44 ORIF and 17 prostheses were performed. No open reduction was performed in this series despite a 34% malreduction rate. The risk for displacement after functional treatment of Garden 1 fractures is 31%. For patients over 65 years old, almost fractures are treated in this series by an arthroplasty. The one-year mortality rate after displaced femoral neck fracture was 17%. Functional results were better in total hip prosthesis group than in bipolar or unipolar group. Non cemented stems were not safer than cemented ones in frail patients. For young patients, ORIF should be the treatment of choice: the initial displacement and its effects on the femoral head vascularisation, the quality of reduction and fixation are the two most significant factors for good outcome. For Garden 1, fractures in patients 65 years old or more, it is proposed to performed an internal fixation despite in two thirds of the cases, it should be unnecessary because non identification of predictive factors of failure. For patients over 65 years old, the type of arthroplasty to perform in displaced fractures is to be chosen according to the preoperative mobility and comorbidities. Because of acetabular erosion with long-term follow-up, it is clearly indicated to perform total hip replacement for patients with life expectancy of 10 years or more. For frail patients, unipolar arthroplasty is the best option. The place for bipolar or uncemented implants is not yet well-defined and more prospective trials are needed. In this multicentric study, results appear quite different in terms of mortality, or functional status. These differences seem to be related to technical choice, geriatric care, nutritional consideration or surgical organisation, all factors that may be of major importance for prognostic."}, {"id": "pubmed23n0729_5355", "title": "Surgical treatment outcome evaluation of hip joint acetabular fracture.", "score": 0.015635102568623993, "content": "Since November 1989 till the end of 2007 year we have been operatively treating 752 of acetabular hip fractures. We have analised 750 operated patients, two patients bilateral fractures. The follow up span lasts from 2 till 20 years. The age of treated patients is from 14 till 79 years old. These fractures were divided into those operated to 21 days after trauma--569 operated acetabulum. And those operated after 21 days after trauma--183 patients with the delayed reconstruction of acetabular fractures from 22 till 229 days. Both groups were provided detailed assesement. And next we compared fracture reduction of broken acetabulum and assesement of clinical treatment. The types of fractures were defined according to classification of Judet-Letournel. The criteria of fracture reduction due to Letournel. The clinical result was based on Merle d'Aubigne-Matta scale. In the group of treated patients till 3 weeks after trauma, we have received 83.8% of very good and good results, 6.9% fair results and 9.3% poor results. During fracture reduction: 75% very good, 4% in secondary joint congruence, 17.8% fair and 3.2% poor. After fair reduction there was always the right congrugence between the head and acetabulum. The displacements to 3 mm were left in more cases as a part after weight-bearing area. In trauma fracture trated after 3 weeks, the result was very good and good 66.1%, fair 14.8% and poor 19.1%. During fracture reduction: 49.7% very good, 4.4% secondary joint congruence, 32.8% fair and 13.1% poor. We have noticed the crucial corelation between fracture reduction and the final result of treatment. Additional traumae, especially head with long time loss of consciousness and the traumae of chest with insufficient breathing have indirect infulence on treatment results causing the longer operation waiting. The complexity fractures also indirectly influence on the result of treatment decreasing the chances on anathomical fracture reduction."}, {"id": "pubmed23n0527_18258", "title": "Displaced intracapsular hip fractures in fit, older people: a randomised comparison of reduction and fixation, bipolar hemiarthroplasty and total hip arthroplasty.", "score": 0.014377470355731226, "content": "To compare internal fixation, bipolar hemiarthroplasty and total hip arthroplasty for the management of displaced subcapital fracture of the hip in previously fit patients of 60 years or older. A prospective randomised clinical trial. This multicentre trial was carried out in 11 Scottish hospitals with acute orthopaedic trauma units. The participants were 298 previously fit patients of 60 years or older with displaced subcapital hip fractures. The three surgical interventions for comparison were reduction and fixation, bipolar hemiarthroplasty and total arthroplasty (total hip replacement). Participating surgeons elected to randomise patients either among all three types of operation (three-way randomisation) or just between fixation and hemiarthroplasty (two-way randomisation). Clinical outcomes were mortality rates, reoperation rates and the complication rates associated with each procedure. Functional outcome was measured using a hip specific questionnaire [Johanson Hip Rating Questionnaire (HRQ)] and a general health status questionnaire [EuroQol 5 Dimensions (EQ-5D)]. Economic analysis compared the costs in the randomised groups of hospital treatment for the initial and subsequent admissions for up to 2 years. Altogether, 207 patients were randomised among all three trial operations, and 91 between just fixation and bipolar hemiarthroplasty. There were no statistically significant differences in clinical outcomes, but confidence intervals (CIs) were wide. At 2 years fixation failure reached 37% among those allocated fixation and 39% had undergone further surgery. Further surgery rates after hemiarthroplasty and total hip replacement were 5% and 9%, respectively. The group allocated fixation had significantly worse HRQ and EQ-5D scores than both arthroplasty groups at 4 and 12 months. At 24 months the results still favoured arthroplasty, but the overall HRQ and EQ-5D scores were no longer statistically significant. Total hip replacement had the best patient-assessed outcome scores. At 24 months the overall HRQ and EQ-5D scores for total hip replacement were significantly better than for hemiarthroplasty. The mean costs for the initial episode ranged from 6384 pounds Sterling for fixation to 7633 pounds Sterling for total hip replacement. The cost differences were largely due to differences in theatre costs and the cost of prostheses and hardware. The cumulative cost over 2 years of hemiarthroplasty was around 3000 pounds Sterling lower than for fixation (95% CI 1227 pounds Sterling to 7192 pounds Sterling). Compared with total hip replacement, both fixation and hemiarthroplasty were characterised by increased costs arising from hip-replacement admissions. When total (initial episode and subsequent hip-related admissions) hip-related costs are compared, total hip replacement conferred a cost advantage of around 3000 pounds Sterling per patient (versus hemiarthroplasty, 95% CI -pounds Sterling 1400 to 7420 pounds Sterling). In fit, older patients the results of the study show a clear advantage for arthroplasty over fixation; arthroplasty was more clinically effective and probably less costly over a 2-year period postsurgery. The results suggest that total hip replacement has long-term advantages over bipolar hemiarthroplasty, but these findings are less definite. This study provided support for the use of total hip replacement to treat displaced intracapsular hip fractures in fit, older patients. A larger trial comparing total versus hemiarthroplasty for these fractures could help to verify these findings. It would also be useful to know whether the findings of this study apply to patients aged 60 years or less who are usually treated with reduction and fixation. A clinical trial comparing arthroplasty versus fixation in patients older than 40 years would be a logical extension of the current study."}, {"id": "wiki20220301en047_45311", "title": "Hip fracture", "score": 0.013837997973025649, "content": "Surgery on the same day or day following the break is estimated to reduce postoperative mortality in people who are medically stable. Intracapsular fractures For low-grade fractures (Garden types 1 and 2), standard treatment is fixation of the fracture in situ with screws or a sliding screw/plate device. This treatment can also be offered for displaced fractures after the fracture has been reduced. Fractures managed by closed reduction can possibly be treated by percutaneously inserted screws. In elderly patients with displaced or intracapsular fractures many surgeons prefer to undertake a hemiarthroplasty, replacing the broken part of the bone with a metal implant. However, in elderly people who are medically well and still active, a total hip replacement may be indicated. Independently mobile older adults with hip fractures may benefit from a total hip replacement instead of hemiarthroplasty."}, {"id": "pubmed23n0810_19631", "title": "Changing the consultant on calls from a daily to weekly rotation system reduces time to theater for patients with hip fracture to improve quality of care: a retrospective study of 2 cohorts of patients presenting with hip fracture.", "score": 0.013450710519259987, "content": "To determine whether changing the consultant on-call schedule resulted in a reduction in time to theater for patients presenting with a hip fracture. Guidelines in the United Kingdom state that patients presenting with a neck of femur fracture should ideally be operated on the day of or the day after admission. However, there is a best practice tariff in the United Kingdom persuading trusts to operate on elderly patients with hip fracture within 36 hours of admission. Differing formats of daily trauma operating lists and varying consultant on-call schedules have the potential to affect a trusts ability to successfully meet such demands. This study retrospectively analyzed whether changing the on-call schedule from a system where the on-call consultant is changed on a daily basis to one which changes weekly resulted in a reduction in time to theater for such patients and an increase in best practice tariffs paid. With the initial rotation system, the average time to theater for a fractured neck of femur was 44 hours 46 minutes, with 44.7% of patients having a time to surgery of less than 36 hours. Patients in the modified system underwent surgery with an average time to theater of 32 hours 19 minutes. In 71.7% of these patients, time to surgery was less than 36 hours. This study demonstrates that changing the schedule to permit a consultant to have a 7-day period of trauma on call at a time instead of only 1 day dramatically reduced the time to theater for patients with hip fracture. This significantly reduced the number of these cases done outside 36 hours and increased trust financial reward."}, {"id": "pubmed23n0519_758", "title": "[Femoral neck fracture complicating orthopedic reposition of a dislocated hip: four cases].", "score": 0.012403446226975638, "content": "Posterior dislocation of the femoral head with fracture is an exceptional hip injury. Emergency reduction is required. Reposition into the acetabular cavity of the dislocated femoral head may not be feasible. Irreducibility, instability, and more rarely accidental fracture of the femoral neck may also occur. We encountered this latter complication in four patients and report here its frequency and mechanism and propose preventive therapeutic measures. Seventy dislocations and fracture-dislocations of the hip were treated in our unit from March 1997 to February 2003. Among these cases, fourteen hip dislocations were complicated by femoral head fractures. Fracture of the femoral neck occurred during reduction in four. All four cases occurred in men, mean age 49.7 years, who were traffic accident victims (drivers or passengers). There were two Pipkin IV fracture-dislocations and two Pipkin II. The first reduction, achieved under general anesthesia in an emergency setting, was performed by an orthopedic surgeon in one patient and a general surgeon in three patients. Arthroplasty was used to treat the femoral neck fracture in three patients and pinning in one. We reviewed retrospectively the clinical and imaging data before and after reduction. Sub-capital fracture situated 4.0 cm (mean, range 3.5-4.5 cm) from the lesser trochanter occurred in all four cases. The head remained attached above and posteriorly to the acetabulum and was rotated less than 90 degrees . The fragment remaining in the acetabulum was displaced in two cases. In one patient, the fracture-dislocation of the head was associated with a fracture of the posterior rim of the acetabulum. This complication appears to result from an abrupt inappropriate reduction movement. The neck fracture would occur when capsulomuscular retention of the femoral head is associated with a head defect which catches on the rim of the acetabulum during the reduction movement. Neck fracture during reduction of traumatic hip dislocation is a serious complication. Prevention of this iatrogenic event requires a slow, progressive reduction limiting the trauma to a minimum; first intention open surgery may be required in selected cases."}, {"id": "pubmed23n0817_12964", "title": "[Hip fracture prosthetics in German trauma surgery. State of the art].", "score": 0.012125220458553791, "content": "To improve patient safety and quality in joint arthroplasty a certification of arthroplasty centers (EndoCert©) and a German arthroplasty register (EPRD) have been implemented. This should guarantee a long-term improvement in documentation of arthroplasty in the future. Although the stages of operations in elective and trauma-associated joint arthroplasty are comparable, the surgical preconditions are often quite different. As required by the German Society of Trauma Surgery (DGU) this study analyzed the current situation with respect to the proportion of fracture-associated joint arthroplasties among the total volume carried out in Germany. A uniform internet-based questionnaire was sent to all listed trauma centers in Germany by the central office of the DGU. In addition any information regarding hip joint arthroplasty in 2011 was collected from the Federal Statistical Office of Germany. The questionnaire was returned by 324 (47 %) out of 690 of the trauma centers contacted. A total of 34,135 total hip arthroplasties (THA) and 26,753 total knee arthroplasties (TKA) were carried out in 2011 by these clinics. The absolute numbers for total endoprosthesis replacement cited by the trauma centers were 5718 THAs and 3829 TKAs. According to the data from the Federal Statistical Office of Germany 131,966 hip fractures were registered in 2011, including 69,582 femoral neck fractures (patient age &gt;19 years) and 62,384 pertrochanteric fractures. From a total number of THAs of approximately 200,000 in Germany 47,695 (approximately 25 %) of these were associated with trauma. The data analyzed in this study and the results obtained from known literature sources demonstrate that THA is the most frequently performed procedure in trauma management of femoral neck fractures. Because of the ubiquitous and high incidence and the need for emergency treatment due to correlated risks and complications of delayed treatment, a high and standardized around the clock (24 h and 7 days a week) treatment option is mandatory. Long waiting times to treatment or transfer are inacceptable due to the increased mortality and complications."}, {"id": "pubmed23n0931_22527", "title": "[Case-control study on retaining articular capsule in the total hip replacement for old patients with unstable femoral neck fractures].", "score": 0.009900990099009901, "content": "To study the effect of reserving articular capsule and traditional total hip replacement for old patients with unstable femoral neck fractures. From January 2010 to January 2015, one hundred and twenty patients with femoral neck fracture were retrospective review. Among them, sixty patients were treated with total hip arthroplasty reserving articular capsule, the others were treated with traditional total hip arthroplasty without reserving articular capsule. The gender of reserving articular capsule group and traditional group were (male/female) 34/26, 31/29 respectively; the age were (73.4±4.4), (72.3±4.1) years old, respectively. All patients were followed up for six months, the operation time, blood loss, length of stay, incidence of postoperative complications, incidence of postoperative hip dislocation and hip Harris score of the two groups were observed. The operative time of the reserving articular capsule group and traditional group were (95.68±6.90), (93.39±7.90) min (<iP</i&gt;0.05), and the blood loss were (998.78±15.20), (1 000.25±16.80) ml (<iP</i&gt;0.05). The time for hospital stay were (10.74±2.90), (13.25±2.20) days(<iP</i&lt;0.05). The Harris scores were 58.53±5.10, 49.38±4.90(<iP</i&lt;0.05) at 1 month after operation;91.08±7.50, 90.74±7.10(<iP</i&gt;0.05) at 6 months after operation. The method of reserving auricular capsule can not increase the operative time and the blood loss. But it has a favorable effect on decreasing the hospital stay, complication, the rate of early dislocation of the hip joint, improving the function of hip joint."}, {"id": "pubmed23n0657_22527", "title": "[Periprosthetic intertrochanteric fracture of the femur following articular resurfacing of the hip joint: treatment with lag screw osteosynthesis].", "score": 0.00980392156862745, "content": "Improvements in implant design, material combination and operating instruments have led to an increased number of resurfacing arthroplasties of the hip joint especially in younger patients. Therefore, there is generally a higher risk of periprosthetic fractures even with this type of prosthesis. These fractures are divided into mainly iatrogenic fractures of the head/neck part of the femur and trochanteric fractures of the femur caused by trauma. Especially in the second group preservation of the prosthesis is much desired since the patient cohort is often very young and active. We report on a 31-year-old male patient who suffered an intertrochanteric fracture (classification AO 31 A2.1) after resurfacing arthroplasty of the hip joint (McMinn BHR prosthesis). The patient was treated with 3 AO lag screws by the percutaneous technique following closed reduction. During follow-up 22 months after the operation the reduction was preserved and the fracture fully consolidated with a good range of motion of the hip joint. The Harris hip score gave a result of 97 points."}, {"id": "pubmed23n0482_1529", "title": "Post-operative mortality related to waiting time for hip fracture surgery.", "score": 0.00980392156862745, "content": "In this retrospective study, we looked at the difference in 1 year mortality between two groups of patients who were operated for fracture of the hip. In cohort 1, 72% of the patients underwent surgery on the same day of admission, 15% of the patients were operated on the next day, the remaining 13% of the patients waited more than 1 day for surgery. The mean waiting time was 0.47 day. The percentage of patients who were operated on the same day of admission in cohort 2 was 18%. Sixty-nine percent of the patients had to wait 1 day before they were operated and 13% waited 2 days or more. The average waiting time was 1.01 days. The date of death for both the 166 patients in cohort 1 and the 197 patients in cohort 2, was obtained from the national mortality register and the 1 year mortality was calculated. These two groups of patients were from separate 12 month periods and the operations performed were either Dynamic Hip Screw (DHS) or hemiarthroplasty. The two groups were comparable in gender, age distribution and the types of operations. There was an increase of 10.1% (P&lt;0.025, chi(2), 1 d.f.; 95% CI 1.7-18.5) in the mortality of patients in cohort 2. The mortality data of the two cohorts was also analysed after dividing the patients into three groups according to their age. A statistically significant increase in mortality of 16.9% in patients over 80 years of age in cohort 2 was found. The difference in mortality was still statistically significant when only patients over 80 years of age and having a DHS operation were compared. Total mortality at 2 years after the operation was the same in the two cohorts. Mortality rate for patients in cohort 2 was less than that for cohort 1 patients during the second post-operative year. This study shows that survival at 1 year is better when patients who are medically fit for surgery are operated on the same day of admission. This survival advantage is more pronounced for patients who are over 80 years of age."}, {"id": "pubmed23n0638_10549", "title": "[Osteosynthesis of intracapsular femoral neck fractures by dynamic hip screw (DHS) fixation].", "score": 0.009708737864077669, "content": "The treatment of femoral neck fractures shows a relatively high number of poor outcomes, usually due to late complications, such as avascular necrosis of the femoral head or pseudoarthrosis. The latter may develop when the osteosynthesis of osteoporotic bone fails. The aim of this retrospective study was to evaluate a group of patients treated by osteo- synthesis for intra-capsular femoral neck fractures at our department, and to verify indication criteria and identify the therapeutic procedures that are best suited to our conditions. In the 1997-2001 period, a total of 81 patients with intra-capsular femoral neck fractures were operated on. Of these, 64 treated by dynamic hip screw (DHS) fixation were followed up for at least 5 years. There were 33 women and 31 men; the average age was 21.5 years (range, 21 to 74 years). The Garden classification was used to evaluate the displacement of femoral neck fractures. Preferably, osteosynthesis was carried out by closed reduction; only exceptionally was an extension device for the operating table used. A 135-degree sliding hip screw, with a short thread, directed to the head centre and a two-hole side plate were used most often.The average follow-up was 6.9 years. Evaluated were: the occurrence of late complications in relation to the length of time between injury and surgery, quality of fracture reduction, use of an anti-rotation screw and necessity of repeat surgery. Garden I or II fractures were diagnosed in 13 patients, 51 had Garden III or Garden IV fractures. Bone union without complications was achieved in 73.4 % of the patients within 12 months of surgery. Late complications were found in 26.6 %; of these, only one had Garden I fracture and the rest were Garden III and IV fractures. An anti-rotation screw was used in 39 patients (60.9 %) and its use had no effect on the development of late complications. Of the seven patients who developed pseudoarthrosis, the screw was used in four (57.1%); out of the nine patients with avascular necrosis, it was used in six (66.7 %). In the whole group, an unsatisfactory outcome of post-operative reduction was recorded in 29.7 %. In the patients with late complications this was found in 52.9 %, which was a statistically significant difference. Of the 17 patients with poor outcomes, 14 underwent total hip arthroplasty; in the patients with necrosis, arthroplasty was carried out at an average of 26 months post-operatively, in those with pseudoarthrosis it was at 7 months post-operatively. For the treatment of intra-capsular fractures of the femoral neck, surgery is the most frequent approach, but there are controversial views on various relevant issues. An important factor affecting the treatment outcome is the patient's bone quality. Our results show a direct relationship between the extent of fracture displacement and late complications, i.e., avascular necrosis and non-union. The quality of fracture reduction had a greater effect on fracture non-union than on the development of femoral head necrosis. The length of time between injury and surgery played a lesser role than it is believed. The use of an anti-rotation screw was not significantly related to the occurrence of late complications. The DHS method is economical and available, and provided sufficient results whose comparisons with the literature data show that this therapeutic approach is correct."}, {"id": "pubmed23n0096_15193", "title": "[Para-articular fracture of the hip joint in the aged--an indication for immediate operation?].", "score": 0.009708737864077669, "content": "177 patients with fractures near the hip joint were operated on between January 1983 and December 1985. The fractures were treated with Ender pinnings, total endoprostheses and dynamic hip screws (compression screw fixation). Up to June 1984 surgery was performed after the patients had been allowed a stabilisation phase of 4 days on the average, but from July 1984 onwards we aimed at performing immediate surgery within 12 hours. The second group was compared with the first group in a retrospective study in respect of recumbent period and mortality (hospital mortality and mortality after 8 months). The period during which the patients had to remain recumbent in the hospital was found to be considerably shorter, but there were no statistically significant differences in respect of mortality. The causes and consequences are discussed."}, {"id": "pubmed23n0695_12065", "title": "Avascular necrosis of the femoral head after osteosynthesis of femoral neck fracture.", "score": 0.009615384615384616, "content": "The reported incidence of avascular necrosis after femoral neck fracture fixation varies widely, and there is no consensus regarding its risk factors. We evaluated the incidence of avascular necrosis of the femoral head with the use of contemporary techniques for femoral neck fracture fixation. We then sought to determine what potential risk factors influenced the development of avascular necrosis.Between 1990 and 2005, one hundred sixty-three intracapsular femoral neck fractures in 163 patients were treated with internal fixation at our level-I trauma center. All patients were monitored until conversion to total hip arthroplasty or for a minimum of 2 years postoperatively. Ten patients (10 hips) died and 7 patients (7 hips) were lost to follow-up. The remaining 146 patients (146 hips) had a mean 5.2 years of follow-up (range, 3 months to 17 years). The incidence of avascular necrosis was 25.3% (37 hips). The average time to diagnosis of avascular necrosis was 18.8 months (range, 3-47 months). Patient sex, age, interval from injury to surgery, and mechanism of injury were statistically not associated with the development of avascular necrosis. The quality of fracture reduction, adequacy of fixation, degree of displacement, and comminution of the posterior cortex were significantly associated. After we controlled for patient and radiographic characteristics, multivariate analyses indicated that the important predictors for avascular necrosis are poor reduction (odds ratio=13.889) and initial displacement of the fracture (odds ratio=4.693)."}, {"id": "pubmed23n0976_9527", "title": "[WAITING TIME FOR SURGICAL FIXATION OF FEMORAL NECK FRACTURES: DOES A DIAGNOSIS-RELATED GROUP PAYMENT METHOD MATTER?]", "score": 0.009615384615384616, "content": "Early surgical fixation of femoral neck factures in elderly patients has been suggested to decrease morbidity and mortality and to improve treatment outcome. This study evaluates the effect of the implementation of a diagnosis-related group payment method in our hospital on waiting time for surgery and the short-term outcomes of elderly patients following surgical fixation of hip fractures. Demographic and clinical characteristics of 75 consecutive patients, who underwent surgery for hip fracture in our hospital, before the implementation of a diagnosis-related group payment method, were compared with those of 75 consecutive patients, who were operated on after the implementation of the payment system. Demographic characteristics were similar for both groups. Before the implementation of a diagnosis-related group payment method, 84% of the patients waited longer than 48 hours for surgery, compared to only 24% of patients after the implementation (p&lt;0.001). Medical considerations and operation room availability were the main reasons for delaying surgery in both groups. Mortality and morbidity rates during the hospital stay remained similar, regardless of the implementation of the payment method. The implementation of a diagnosis-related group payment method shortened the waiting time for surgical hip fixation in elderly patients treated in our hospital, with no effect on the mortality and complication rate during the hospital stay."}, {"id": "pubmed23n0900_15081", "title": "Bilateral atypical femoral fracture and end-stage arthritis of the hip, treated with total hip arthroplasty.", "score": 0.009523809523809525, "content": "The prolonged use of bisphosphonates has been associated with an increased rate of atypical femoral fracture. A 77-year-old woman with prolonged bisphosphonate use presented to our office with groin pain and end-stage arthritis, She was scheduled for a total hip replacement. Before the surgery and with minimal trauma, the patient then suffered a displaced atypical femoral fracture. She underwent a total hip replacement as a treatment for her fracture and her arthritis. Subsequently, the patient presented with pain in the contralateral thigh with an incomplete atypical femoral fracture. That side was also treated with a total hip arthroplasty. An uncemented stem with open reduction internal fixation and a long cemented stem were used on the complete fracture and incomplete fracture sides, respectively. At a follow-up of 2 years, the patient had no pain and had excellent function demonstrating the short-term success of both cemented and uncemented stems in total hip arthroplasty after atypical femoral fractures."}, {"id": "pubmed23n0530_5041", "title": "[Emergency room consultation policy prevents surgery delay in elderly patients with hip fractures].", "score": 0.009523809523809525, "content": "Clinical guidelines for the management of hip fractures strongly emphasize the critical role of early surgery as a major factor affecting better health outcomes. Shortening the time from admission to pre-operative consultation by bringing the consultants to the emergency room, before patient admission to the orthopedic ward, is am efficient cost effective hospital policy that enables adherence to guideline recommendations. Early pre-operative specialist consultation is an efficient cost effective policy for preventing delay of surgery following hip fracture in the elderly."}, {"id": "pubmed23n0998_20529", "title": "Bilateral posterior hip dislocation associated with right Pipkin II fracture: A case report.", "score": 0.009433962264150943, "content": "Bilateral posterior hip dislocations are very rare injury, requiring a very hight trauma energy. We present a case of 40-year-old male who sustained bilateral posterior hip dislocation with associated right femoral head fracture Pipkin type II following a hight energy trauma without neurovascular deficit. A prompt closed reduction was made, it was successful in lift hip but incomplete in right one, therefore, an open reduction was indicated, performed through a modified Hardinge approach permitting internal osteosynthesis with two Herbert screws. Posterior hip dislocations are an orthopaedic emergency that must be reduced within 6 h to avoid sciatic nerve compression and avascular necrosis (AVN). They are most often associated with femoral head fractures commonly known as Pipkin's fractures, that need anatomic reduction and osteosynthesis through posterior approaches, rarely external or anterior approaches. Anatomic and functional results were good at two years follow up excepting a right hip non-bridging heterotopic ossification; the patient returned to his work normally without any functional sequelae."}, {"id": "pubmed23n0889_4669", "title": "When is the ideal time to operate on a patient with a fracture of the hip? : a review of the available literature.", "score": 0.009433962264150943, "content": "Fractures of the hip are common, often occurring in frail elderly patients, but also in younger fit healthy patients following trauma. They have a significant associated mortality and major social and financial implications to patients and health care providers. Many guidelines are available for the management of these patients, mostly recommending early surgery for the best outcomes. As a result, healthcare authorities now put pressure on surgical teams to 'fast track' patients with a fracture of the hip, often misquoting the available literature, which in itself can be confusing and even conflicting. This paper has been written following an extensive review of the available literature. An attempt is made to clarify what is meant by early surgery (expeditious versus emergency), and we conclude with a personal view for the practical management of these patients of variable age, fitness and type of surgery performed within services that are often under considerable pressure of finance and available operating theatres and qualified staff. Cite this article: Bone Joint J 2016;98-B:1573-81."}, {"id": "pubmed23n0675_15422", "title": "Initial promising results of the dynamic locking blade plate, a new implant for the fixation of intracapsular hip fractures: results of a pilot study.", "score": 0.009345794392523364, "content": "The osteosynthesis of intracapsular hip fractures results in a 19-48% failure rate. Only when the anatomical reduction is secured by stable fixation, revascularisation of the femoral head can take place and the fracture can heal by primary osteonal reconstruction. The common implants lack rotational and/or angular stability. Also the relative large volume of the implants within the femoral head compromises the (re)vascularisation. The combination of an anatomical reduction and a low volume, dynamic implant, providing angular and rotational stability seem to be crucial factors in the treatment of intracapsular hip fractures. This assumption formed the starting point for the development of the dynamic locking blade plate (DLBP), a new implant for the internal fixation of intracapsular hip fractures. This report describes the first clinical results of the new implant. Internal fixation with the DLBP was performed in 25 consecutive patients with an intracapsular hip fracture within 24 h from admission. Failure of fixation, due to non-union, avascular necrosis, implant failure or secondary displacement of the fracture, was the primary outcome measurer. Functional outcome was assessed by the Harris Hip Score. Following internal fixation of intracapsular hip fractures with the DLBP, a failure rate of 2 out of 25 patients and excellent functional results were seen after a follow-up of more than 2 years. The initial clinical results of the DLBP are promising and justify the start of a randomised controlled trial."}, {"id": "pubmed23n1095_19071", "title": "\"Acute Primary Total Hip Arthroplasty for Combined Posterior Acetabulum Fracture with Ipsilateral Associated Posteriorly Dislocated Femoral Head with Femoral Neck Fracture. Using of Femoral Head as an Autograft Would be an Advantage\".", "score": 0.009259259259259259, "content": "Traumatic posterior hip dislocation with comminuted fracture of the ipsilateral acetabulum and femoral neck is a rare fracture pattern. These injuries are associated with high energy trauma and pose challenges during management. Controversy exists between hip preservation and replacement surgeries in middle-age patients. Open reduction and internal fixation (ORIF) have a high risk of non-union, avascular necrosis, and post-traumatic osteoarthritis of hip requiring total hip arthroplasty hip replacement (THA) as a secondary procedure later. A 56-year-old male presented with posterior hip dislocation and comminuted fracture of ipsilateral wall and column of the acetabulum, and femoral neck following a high energy trauma. He was managed by acetabular reconstruction using femoral head structural autograft combined with acute primary uncemented THA. At 2-year follow-up, the patient had good functional outcome with a satisfactory range of motion without any difficulty in weight-bearing and doing his daily activities. Although uncommon, acetabular reconstruction using femoral head structural autograft and acute primary uncemented THA is a viable alternative treatment option compared to ORIF in middle-age patients with fracture of ipsilateral neck and acetabulum. This facilitates post-operative rehabilitation and avoids further operations for possible developing AVN or secondary arthritis."}, {"id": "pubmed23n0603_17230", "title": "[Treatment of intertrochanteric comminuted fracture in aged patients by replacement of artificial long-stem bipolar femoral head].", "score": 0.009259259259259259, "content": "To discuss the clinical effect of hip arthroplasty for the treatment of intertrochanteric comminuted fracture in aged patients. From February 2004 to March 2006, 89 cases with intertrochanteric comminuted fractures were treated by replacement of artificial long-stem bipolar hip hemiarthroplasty with bone cement, including 34 males and 55 females, with the average age of 82.6 years (ranging from 70 years to 92 years). There were fresh fractures in 76 cases and old fractures in 13 cases. According to improving-classification of Evans-Jensen, there were 32 cases with type III fracture and 57 with type IV. Osteoporosis was graded by Singh-index, and there were 24 cases with level IV, 46 with level III and 19 with level II. The operation was delayed from 4 days to 36 days (6.5 days on average) after the injury. The surgery time lasted from 50 minutes to 70 minutes (62 minutes on average). Bleeding volume was from 100 mL to 250 mL (150 mL on average). The time in hospital was from 16 days to 25 days (18.6 days on average). All incision primarily healed except 1 case which delayed union by revision with infection. Steel wire rupture occurred in 1 case, invalidation in 1 case. They were not treated because there were no uncomfortable symptoms. The consciousness disability happened in 2 cases 2 to 3 days after the operation, 2 case with the infection for the system of urine and breath 1 week after operation, were healed through the treatment. Eighty-five cases were followed up for 14 months to 39 months (26.5 months on average). The function of the hip was graded by the system of Harris and the choiceness rate was 84.7% (excellent in 16 cases, good in 56 cases, fair in 12 cases and poor in 1 case). The treatment of intertrochanteric comminuted fracture in the aged patients by replacement of long-stem bipolar femoral head prosthesis is effective, but indications and techniques of the operation must be paid much attention to."}, {"id": "wiki20220301en079_43609", "title": "Hip dislocation", "score": 0.009236823029926478, "content": "Management Hip dislocations are a medical emergency, requiring timely placement of the femoral head back into the acetabulum (reduction) in order to reduce the risk of osteonecrosis of the femoral head. Most professionals recommend closed reduction (nonoperative) barring operative indications such as irreducible dislocation, delayed presentation, non-concentric reduction, fracture requiring excision and/or open reduction internal fixation (ORIF) among other operative indications. Prognosis is worsened if reduction is delayed more than 6 hours. If the reduction is stable, the patient can proceed to protective weight bearing which includes crutch-assisted walking (ambulation) with weight bearing as tolerated for 4-6 weeks succeeding a short period of bed rest. If reduction is unstable, 4-6 weeks of skeletal traction is necessary before protective weight bearing."}, {"id": "pubmed23n0371_10220", "title": "Inability to obtain formal informed consent in the face of a standard surgical indication.", "score": 0.009174311926605505, "content": "A thirty-eight-year-old intoxicated man was admitted to the surgical trauma service following a single motor-vehicle accident. He had a severe closed head injury, bilateral pulmonary contusions, a fracture-dislocation of the right acetabulum, and an open injury of the right knee joint. The acetabular fracture pattern was an associated both-column fracture with the femoral head dislocated into a widely displaced posterior-column fracture line. The treating physicians agreed that it would be in the patient's best interest to take him to the operating room for emergent debridement and irrigation of his knee wound. At surgery, the patient also underwent attempted closed reduction of the acetabular fracture and placement of a skeletal traction pin. Radiographs obtained with the patient in traction showed reduction of the femoral head beneath a displaced superior dome fragment, but there remained a 12-mm gap in the posterior column, greater than 3 mm of step incongruity, and a large articular fragment entrapped in the anterior aspect of the hip joint. The patient remained intubated and sedated for several days. Upon weaning from the ventilator, it became evident that his head injury would prevent him from being able to give informed consent in the foreseeable future. The patient's family members refused to become involved with his care or medical decision-making, as he had become completely estranged from them as a result of his chronic alcohol abuse. Further delay in surgical treatment for the acetabular fracture would be associated with greater difficulty in obtaining an anatomic reduction, the potential for additional articular damage to the femoral head, and an increased risk of surgical complications. The question that arises is whether it is in the patient's best interest for the surgeon to proceed with open reduction and internal fixation of the acetabular fracture without having had the opportunity to fully inform him of the treatment options or the risks associated with an extensive surgical exposure."}, {"id": "pubmed23n0525_4407", "title": "[The hip head-conserving management of traumatic medial cervical hip fractures with big-fragment screws: a biomechanical examination].", "score": 0.009174311926605505, "content": "With the growing number of elderly people in the population and the increasing incidence of proximal hip fractures the question of how to manage the medial hip head fracture is of increasing importance. Especially in Hungary and the Scandinavian countries surgeons prefer hip head-conserving therapy although the redislocation of this fracture and necrosis of the hip head opposes this point of view. Encouraged by two theoretical and mathematical calculations, we tested two different possibilities to screw hip head fractures. Our results show that the hip head-conserving therapy with two cranial screws and a three-point-supported screw at Adam's arc has essential biomechanical advantages compared with the situation after conventional osteosynthesis. This result encourages us to prefer the minimally invasive head-conserving therapy of medial hip head fractures, especially for treatment of Pauwell's I and II injuries."}, {"id": "pubmed23n0898_5369", "title": "[Periprosthetic Femoral Fractures after Total Hip Replacement: Our Results and Treatment Complications].", "score": 0.00909090909090909, "content": "PURPOSE OF THE STUDY The study consists of a retroactive evaluation of results of surgical treatment in patients with periprosthetic femoral fracture after total hip replacement and a comparison with results reported in the literature. MATERIAL AND METHODS In the period from 2003 to 2013, a total of 83 patients with periprosthetic femoral fracture after total hip replacement were treated at our clinic, namely 69 women and 14 men. The mean age in the cohort was 74 years (range 47-87). The Vancouver classification was used to grade the fractures. The cohort included 31 patients with type B1 fracture, 25 patients with type B2 fracture, 8 patients with type B3 fracture, and 19 patients with type C fracture. Altogether 80 patients underwent a surgery, 3 patients with non-displaced type B1 fracture were treated conservatively. The mechanism of injury was a simple fall in 75 % of primary endoprostheses and in 56% of revision endoprostheses. The average time to fracture was 7.6 years in primary implant and 3.6 years in revision endoprosthesis. In fractures with a well-fixed stem (type B1 and C) plate osteosynthesis was used. In case of a comminution zone, osteosynthesis was followed by spongioplasty. In patients with a loose stem (type B2 and B3), the fracture was treated with a revision uncemented stem. In two cases a combination of a revision stem and a massive corticocancellous bone graft was used. The evaluation was performed using the Harris Hip Score and the minimum follow-up from the surgery was 3 years. RESULTS In the group of patients with type B1 fracture, 28 patients were treated surgically. An excellent result was achieved in 22 patients (84%), in 4 patients (16%) the result was very good. The remaining 2 patients failed to meet the requirement of the minimum follow-up of 3 years. In the group of patients with type B2 fractures, composed of 25 patients, the femoral component was replaced with a revision uncemented stem with cerclage wires or titanium tapes or cables. Osseointegration of the stem was recorded in 24 patients, one female patient died 4 months after the surgery. An excellent result was achieved in 16 patients (64%), a very good result in 4 patients (16%). The remaining 5 patients (20%) failed to meet the minimum follow-up of 3 years. In 8 patients with type B3 trauma, the reimplant of a revision stem was supplemented by spongioplasty, in 2 cases by solid corticocancellous bone grafts with cerclage. In this group osseointegration occurred in all the cases within 6-9 months. The follow-up was affected by the older age of patients and 6 patients died during the follow-up period. The requirement of a follow-up longer than 3 years was met in 2 patients (25%) only and the result was considered very good. In the group of 19 patients with type C fracture, plate osteosynthesis was performed, which was in 12 cases complemented with spongioplasty. Healing occurred within 6 months in 13 patients (72%), within 9 months in 3 patients (17%) and in 2 patients (11%) reoperation was carried out due to fixation failure. One female patient died 16 days after the surgery. An excellent result was achieved in 15 patients (83%), in the remaining three patients the follow-up was shorter than three years due to their death. DISCUSSION Periprosthetic femoral fractures after total hip replacement is a rare but feared complication. Its incidence ranges from 0.1 to 4%. It occurs most frequently 7 to 8 years after the primary implant and 3 to 4 years after the revision of endoprosthesis implantation. The main risk factor is the loosening of stem of endoprosthesis. Another risk factor is osteoporosis. Age, sex and obesity do not constitute significant risk factors. Stem stability and presence of bone defects are the main criteria in favour of surgical treatment. If the stem remains well fixed, the osteosynthesis is opted for, whereas if the stem is loose, its replacement has to be performed. The management of bone defects is an integral part of femoral reconstruction and restoration of endoprosthesis stability. CONCLUSIONS Surgical treatment of periprosthetic fractures, thanks to the introduction of new implants for osteosynthesis and development of new stems for revision endoprostheses, helps achieve ever better results. Of major importance for choosing the treatment method is correct classification of fracture and stem stability. Poor bone quality is a common feature, therefore a perfect mechanical fixation is necessary. The long-term results are affected primarily by the patient s age. Key words: periprosthetic femoral fractures, surgical treatment, results, complications."}, {"id": "pubmed23n0962_3922", "title": "[Analysis of the prognostic factors influencing the time elapsing until the contralateral hip fracture].", "score": 0.00909090909090909, "content": "Although several national studies reported on the risk factors for contralateral hip fracture, there are no data about the prognostic factors of the time until contralateral hip fractures. The aim of the study was to analyse the impact of different prognostic factors on the time until the development of contralateral fracture and to determine the incidence of contralateral hip fractures after femoral neck fractures. Patients aged 60 years and over with contralateral hip fracture between 01 Jan 2000 and 31 Dec 2008 were identified among those who suffered their femoral neck fracture in Hungary in 2000. Risk factors as age, sex, comorbidities, type of fracture and surgery, place of living and hospitals providing treatment for primary fracture were analysed by one way ANOVA focusing on the time until the development of contralateral hip fracture. 312 patients met the inclusion criteria. The incidence of contralateral hip fracture after femoral neck fracture ranged between 1.5% and 2.1%, the cumulative incidence was 8.24%. The mean time until the development of contralateral hip fracture was 1159.8 days. The incidence of contralateral hip fracture showed no significant deviation. Significantly shorter time (p = 0.010) was detected until the contralateral hip fracture in older patients with femoral neck fracture. The yearly incidence of contralateral hip fracture showed no significant difference by patients with femoral neck fracture over 60 years. The shorter time until the contralateral hip fracture by the older age groups highlights the need of elaboration of prevention strategies. Orv Hetil. 2018; 159(38): 1543-1547."}, {"id": "pubmed23n0847_7078", "title": "[Fracture Type and Injury-to-Surgery Interval as Risk Factors for Avascular Necrosis of the Femoral Head after Internal Fixation of Intracapsular Femoral Neck Fracture].", "score": 0.009009009009009009, "content": "The aim of the study was to investigate the occurrence of avascular necrosis (AVN) of the femoral head following the osteosynthesis of intracapsular fracture of the femoral neck in relation to the time interval between injury and surgery and the type of fracture. The data of patients with intracapsular fractures of the femoral neck surgically treated in the period from 2001 to 2011 were reviewed. Of 1555 patients treated for this fracture, 125 (7%) underwent osteosynthesis. The evaluated group included 115 patients who came for examination at one-year follow-up. There were 59 (52%) women and 56 (48%) men. Dynamic hip screw (DHS) osteosynthesis with an anti-rotation screw was performed in 103 patients and lag-screw osteosynthesis involving three parallel cannulated cancellous screws was employed in 12 patients. The patients were allocated to groups according to the injury-to-surgery interval and to sub-groups on the basis of the Garden classification of femoral fracture stage. In the group of 58 patients treated within 6 h of injury, AVN developed in 10 (17%). When the type of fracture was considered, 4% of the non-displaced fractures and 30% of the displaced fractures developed AVN. The patients with Garden stage I and II (non-displaced) fractures treated within 6 h of injury had a significantly lower risk of AVN development than those with Garden stage III or IV (displaced) fractures. The group treated between 6 and 24 post-injury hours comprised 21 patients, of whom four (19%) had AVN. In non-displaced and displaced fracture sub-groups, 25% of the patients in the former and 16% in the latter had AVN. The stage of displacement had no effect on AVN development. The two groups together (patients treated by 24 h) had a significantly lower AVN incidence than the patients treated after 24 h (p = 0.0025). In this group of 36 patients, 16 had AVN (44%) and the fracture stage made no significant difference (p = 0.6985; nondisplacement sub-group, 41%; displacement sub-group, 55%). The study showed a significantly lower AVN occurrence in the patients surgically treated within 24 h of injury. In the patients treated within 6 h of injury, AVN incidence was significantly lower in the patients with non-displaced fractures, as compared with those who had displaced fractures. This was not true for the two patient groups treated later (6-24 and later than 24 h) in which the differences between AVN development after non-displaced fractures and that after displaced fractures were similar."}, {"id": "pubmed23n0856_16770", "title": "The World Hip Trauma Evaluation Study 3: Hemiarthroplasty Evaluation by Multicentre Investigation - WHITE 3: HEMI - An Abridged Protocol.", "score": 0.009009009009009009, "content": "Approximately half of all hip fractures are displaced intracapsular fractures. The standard treatment for these fractures is either hemiarthroplasty or total hip arthroplasty. The recent National Institute for Health and Care Excellence (NICE) guidance on hip fracture management recommends the use of 'proven' cemented stem arthroplasty with an Orthopaedic Device Evaluation Panel (ODEP) rating of at least 3B (97% survival at three years). The Thompsons prosthesis is currently lacking an ODEP rating despite over 50 years of clinical use, likely due to the paucity of implant survival data. Nationally, adherence to these guidelines is varied as there is debate as to which prosthesis optimises patient outcomes. This study design is a multi-centre, multi-surgeon, parallel, two arm, standard-of-care pragmatic randomised controlled trial. It will be embedded within the WHiTE Comprehensive Cohort Study (ISRCTN63982700). The main analysis is a two-way equivalence comparison between Hemi-Thompson and Hemi-Exeter polished taper with Unitrax head. Secondary outcomes will include radiological leg length discrepancy measured as per Bidwai and Willett, mortality, re-operation rate and indication for re-operation, length of index hospital stay and revision at four months. This study will be supplemented by the NHFD (National Hip Fracture Database) dataset. Evidence on the optimum choice of prosthesis for hemiarthroplasty of the hip is lacking. National guidance is currently based on expert opinion rather than empirical evidence. The incidence of hip fracture is likely to continue to increase and providing high quality evidence on the optimumCite this article: A. L. Sims. The World Hip Trauma Evaluation Study 3: Hemiarthroplasty Evaluation by Multicentre Investigation - WHITE 3: HEMI - An Abridged Protocol. Bone Joint Res 2016;5:18-25. doi: 10.1302/2046-3758.51.2000473."}, {"id": "pubmed23n1000_5875", "title": "A Periprosthetic Femoral Fracture with Characteristics of Atypical Femoral Fracture.", "score": 0.008928571428571428, "content": "Although the definition of atypical femoral fracture (AFF) excludes periprosthetic femoral fracture (PFF), the number of reports about PFF with characteristics of AFF is increasing. We present the case of such a fracture in this report. An 87-year-old woman who underwent bipolar hip arthroplasty for a femoral neck fracture 38 months prior reported left thigh pain with no history of trauma. Radiographs showed a simple transverse fracture at the level of the stem distal end with features of AFF: periosteal thickening of the lateral cortex, a medial spike, and a noncomminuted fracture. She presented other features resembling AFF: history of bisphosphonate use, prodromal symptoms, no associated trauma, and lateral bowing of the contralateral femur. The fracture showed nonunion after the initial osteosynthesis, and a revision surgery of the arthroplasty and osteosynthesis was performed. Nine months after the surgery, bony union was achieved and she regained the ability to walk. It is supposed that the fracture was influenced by a stress force related to implants and lateral bowing concentrating on the fracture site as a mechanical factor in addition to bisphosphonates as a biological factor. It would be important to recognize that AFF could occur at the peri-implant location, and early detection and treatment are essential."}, {"id": "pubmed23n0772_9476", "title": "Unipolar hemiarthroplasty versus bipolar hemiarthroplasty in patients with displaced femoral neck fractures: a four-year follow-up of a randomised controlled trial.", "score": 0.008928571428571428, "content": "The treatment of choice for a displaced femoral neck fracture in the most elderly patients is a cemented hemiarthroplasty (HA). The optimal design, unipolar or bipolar head, remains unclear. The possible advantages of a bipolar HA are a better range of motion and less acetabular wear. The aim of this study was to evaluate hip function, health related quality of life (HRQoL), surgical outcome and acetabular erosion in a medium-term follow-up. One hundred and twenty patients aged 80 or more with a displaced fracture of the femoral neck (Garden III and IV) were randomised to treatment with a cemented Exeter HA using a unipolar or a bipolar head. All patients were able to walk independently, with or without aids, before surgery. Follow-ups were performed at four, 12, 24 and 48 months postoperatively. Assessments included HRQoL (EQ-5D index score), hip function (Harris hip score [HHS]) and radiological acetabular erosion. The mean EQ-5D index score was generally higher among the patients with bipolar hemiarthroplasties at the follow-ups with a significant difference at 48 months: unipolar HAs 0.59 and bipolar HAs 0.70 (p = 0.04). There was an increased rate of acetabular erosion among the patients with unipolar hemiarthroplasties at the early follow-ups with a significant difference at 12 months (unipolar HAs 20% and bipolar HAs 5%, p = 0.03). At the later follow-ups the incidence of acetabular erosion accelerated in the bipolar group, and there were no significant differences between the groups at the 24- and 48-month follow-ups. There was no difference in HHS or reoperation rate between the groups at any of the follow-ups. The bipolar HAs seem to result in better HRQoL beyond the first two years after surgery compared to unipolar HAs. Bipolar HAs displayed a later onset of acetabular erosion compared to unipolar HAs."}, {"id": "pubmed23n1125_15969", "title": "Osteosynthesis and outcomes of traumatic periprosthetic femoral fractures after total hip arthroplasty.", "score": 0.008849557522123894, "content": "The aim of this retrospective study was to investigate the treatment of traumatic periprosthetic femoral fractures with open reduction and internal fixation. The outcomes with the use of the surgical techniques were also reported. Between September 2017 and September 2019, 25 patients with traumatic periprosthetic femoral fractures were managed by open reduction and internal fixation in Ain Shams University Hospital, Egypt. The fixation methods were selected based on the surgeon's preference. Outcomes were assessed using the Harris Hip Score, visual analogue score (VAS) for pain, and EuroQol 5 Dimensions - 5 Level (EQ5D-5L) prior to and after surgery. Patients were regularly followed up for one year. A P value &lt; 0.05 was considered to be statistically significant. The mean age at surgery was 77 years (range, 51 to 95 years), 64% (n = 16) were females. According to the Vancouver classification, there were 1 type AG, 15 type B1, and 9 type C fractures. Postoperative complications included wound site infection (n = 2) and non-union (n = 1). The mean pre-trauma Harris Hip Score was 77.44 ± 8.63 (range, 65 to 90), and the mean Harris Hip Score collected at the final follow-up was 72.47 ± 8.85 (range, 60 to 86) (P &lt; 0.05). The mean pre-trauma VAS was 2.20 ± 1.21 (range, 0 to 4), and the mean VAS recorded at the final follow-up was 3.00 ± 1.41 (range, 0 to 5) (P &lt; 0.05). According to the EQ5D-DL assessed at the final follow-up, no patient felt that their daily life and activities became more problematic. This study provided added validation of the current management of periprosthetic femoral fractures after total hip arthroplasty. Using the proper fixation and implant can achieve a reliable fixation and good functional recovery. IVa."}, {"id": "pubmed23n0684_19319", "title": "[Comparison of effectiveness of three operations in treatment of displaced femoral neck fractures in the elderly patients].", "score": 0.008849557522123894, "content": "To compare the effectiveness of internal fixation, hemiarthroplasty, and total hip arthroplasty in the treatment of displaced femoral neck fractures in elderly patients so as to provide the evidence for the selection of therapeutic methods. Between May 2005 and April 2008, 108 elderly patients with displaced femoral neck fractures were treated by internal fixation with compression screw (IF group, n = 31), hemiarthroplasty (HA group, n = 37), and total hip arthroplasty (THA group, n = 40). In IF group, there were 8 males and 23 females with an average age of 73 years (range, 65-80 years); fractures were caused by tumbling (25 cases) and traffic accident (6 cases), including 17 cases of Garden type III and 14 cases of Garden type IV; and the time from injury to operation ranged from 8 hours to 13 days with an average of 4.2 days. In HA group, there were 10 males and 27 females with an average age of 74 years (range, 65-80 years); fractures were caused by tumbling (29 cases) and traffic accident (8 cases), including 21 cases of Garden type III and 16 cases of Garden type IV; and the time from injury to operation ranged from 1 to 14 days with an average of 4.4 days. In THA group, there were 11 males and 29 females with an average age of 73 years (range, 66-80 years); fractures were caused by tumbling (32 cases) and traffic accident (8 cases), including 23 cases of Garden type III and 17 cases of Garden type IV; and the time from injury to operation ranged from 2 to 14 days with an average of 5.6 days. There was no significant difference in general data among 3 groups (P &gt; 0.05). There were significant differences in operation time and blood loss among 3 groups (P &lt; 0.05), and IF group was less than other 2 groups. All patients were followed up 1 year and 4 months to 2 years and 3 months with an average of 1 year and 8 months. In IF group, HA group, and THA group, the rates of early postoperative complications were 19.4% (6/31), 8.1% (3/37), and 7.5% (3/40), respectively; the rates of late postoperative complications were 29.0% (9/31), 13.5% (5/37), and 7.5% (3/40), respectively; and the reoperation rates were 29.0% (9/31), 10.8% (4/37), and 5.0% (2/40), respectively. The rates of the early postoperative complication, late postoperative complication, and reoperation rate were significantly higher in IF group than in HA group and THA group (P &lt; 0.05), but there was no significant difference between HA group and THA group (P &gt; 0.05). The mortality rates were 16.1% (5/31), 13.5% (5/37), and 15.0% (6/40) in IF group, HA group, and THA group, respectively; showing no significant difference (P &gt; 0.05). According to Harris hip score, the excellent and good rates were 65.4% (17/26), 81.3% (26/32), and 85.3% (29/34) in IF group, HA group, and THA group, respectively; showing significant differences among 3 groups (P &lt; 0.05). According to patient's age, life expectancy, and general conditions, THA is a reasonable choice for the patients aged 65-80 years with displaced femoral neck fracture."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[303, 410]], "word_ranges": [[51, 70]], "text": "The rest of the pathologies do not have a positive Lasègue maneuver, so these options would not be correct."}, "2": {"exist": true, "char_ranges": [[303, 410]], "word_ranges": [[51, 70]], "text": "The rest of the pathologies do not have a positive Lasègue maneuver, so these options would not be correct."}, "3": {"exist": true, "char_ranges": [[0, 302]], "word_ranges": [[0, 51]], "text": "In this case, when speaking of \"reappearance of the symptoms when we lift the lower limb with the knee extended\" we are describing the Lasègue Maneuver. This maneuver is positive in cases of lumbosciatic pain, since it causes a stretching of the sciatic nerve, therefore the correct answer is number 3."}, "4": {"exist": true, "char_ranges": [[303, 410]], "word_ranges": [[51, 70]], "text": "The rest of the pathologies do not have a positive Lasègue maneuver, so these options would not be correct."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "In this case, when speaking of \"reappearance of the symptoms when we lift the lower limb with the knee extended\" we are describing the Lasègue Maneuver. This maneuver is positive in cases of lumbosciatic pain, since it causes a stretching of the sciatic nerve, therefore the correct answer is number 3. The rest of the pathologies do not have a positive Lasègue maneuver, so these options would not be correct.", "full_answer_no_ref": "In this case, when speaking of \"reappearance of the symptoms when we lift the lower limb with the knee extended\" we are describing the Lasègue Maneuver. This maneuver is positive in cases of lumbosciatic pain, since it causes a stretching of the sciatic nerve, therefore [HIDDEN]. The rest of the pathologies do not have a positive Lasègue maneuver, so these options would [HIDDEN].", "full_question": "A 61-year-old woman, administrative, with a history of overweight, hypertension, dyslipidemia and metabolic syndrome, who consults for pain in both buttocks, left trochanteric region, lateral aspect of the left thigh up to the knee and left leg up to the middle third. The pain appears when the lower limb is lifted with the knee extended, but is relieved when the knee is flexed. What is the first clinical suspicion?", "id": 602, "lang": "en", "options": {"1": "Gouty arthritis of left hip.", "2": "Left coxofemoral arthrosis.", "3": "Radiated low back pain / lumbosciatica.", "4": "Claudication due to canal stenosis.", "5": NaN}, "question_id_specific": 112, "type": "TRAUMATOLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en029_55324", "title": "Sacroiliac joint", "score": 0.015040515040515042, "content": "Signs and symptoms The following are signs and symptoms that may be associated with an SI joint (SIJ) problem: Mechanical SIJ dysfunction usually causes a dull unilateral low back pain. The pain is often a mild to moderate ache around the dimple or posterior superior iliac spine (PSIS) region. The pain may become worse and sharp while doing activities such as standing up from a seated position or lifting the knee towards the chest during stair climbing. Pain is typically on one side or the other (unilateral PSIS pain), but the pain can occasionally be bilateral. When the pain of SIJ dysfunction is severe (which is infrequent), there can be referred pain into the hip, groin, and occasionally down the leg, but rarely does the pain radiate below the knee. Pain can be referred from the SIJ down into the buttock or back of the thigh, and rarely to the foot. Low back pain and stiffness, often unilateral, that often increases with prolonged sitting or prolonged walking."}, {"id": "article-28772_10", "title": "Sciatica -- History and Physical", "score": 0.012498392696412498, "content": "Further, a straight leg raise (SLR) test is a neurological maneuver performed while examining a patient presenting with lower back pain. This test is conducted with the patient lying supine while keeping the symptomatic leg straight by flexing the quadriceps. The examiner elevates the leg progressively at a slow pace. The test is deemed positive if it reproduces the patient's symptoms (pain and paresthesia) at an angle lower than 70° with radiation below the knee (Lasegue sign). This test is most helpful in diagnosing L4, L5, and S1 radiculopathies. The patient is asked to dorsiflex the foot while the examiner raises the leg (Bragaad sign) to increase the test's sensitivity. When executing the straight leg raise test, the examiner will slightly bend the patient's knee by 20° to 30°, which will lessen the pain. Then, manual pressure is applied in the popliteal fossa. The Bowstring sign is considered positive if it causes the same level of discomfort that the patient feels during a straight leg raise. [8] The Naffziger test involves reproducing pain via coughing. [9]"}, {"id": "article-28772_9", "title": "Sciatica -- History and Physical", "score": 0.012235449735449735, "content": "A straight-leg raise has variable sensitivity and specificity and may or may not be present depending on the underlying cause. The straight-leg test is a passive examination where the patient first lies in a relaxed, supine position. The examiner then lifts the leg from the posterior aspect, flexing at the hip joint and keeping the knee in full extension or keeping the leg straight. Typically, pain that is reproduced between 30° to 70° of hip flexion and experienced primarily in the back is likely due to a lumbar disc herniation. Pain and parenthesis felt in the leg are possible due to lateralizing compression of a peripheral nerve. While not absolute, musculoskeletal causes of the pain usually reproduce pain above 70° of flexion and below 30° of flexion."}, {"id": "wiki20220301en132_2144", "title": "Greater trochanteric pain syndrome", "score": 0.011970110139179091, "content": "More often the lateral hip pain is caused by disease of the gluteal tendons that secondarily inflames the bursa. This is most common in middle-aged women and is associated with a chronic and debilitating pain which does not respond to conservative treatment. Other causes of trochanteric bursitis include uneven leg length, iliotibial band syndrome, and weakness of the hip abductor muscles. Greater trochanteric pain syndrome can remain incorrectly diagnosed for years, because it shares the same pattern of pain with many other musculoskeletal conditions. Thus people with this condition may be labeled malingerers, or may undergo many ineffective treatments due to misdiagnosis. It may also coexist with low back pain, arthritis, and obesity. Signs and symptoms The primary symptom is hip pain, especially hip pain on the outer (lateral) side of the joint. This pain may appear when the affected person is walking or lying down on that side. Diagnosis"}, {"id": "article-24453_19", "title": "Lumbar Disc Herniation -- History and Physical", "score": 0.011927655677655679, "content": "A straight leg raise test is a neurological maneuver performed while examining a patient presenting with lower back pain. It is conducted with the patient lying supine while keeping the symptomatic leg straight by flexing the quadriceps. The examiner slowly elevates the leg progressively at a slow pace. The test is positive when it reproduces the patient's symptoms (pain and paresthesia) at an angle lower than 45 degrees with radiation below the knee (Lasegue sign). It is most helpful in diagnosing L4, L5, and S1 radiculopathies. The patient is asked to dorsiflex the foot while the examiner is raising the leg (Bragaad's sign) to increase the sensitivity of the test."}, {"id": "pubmed23n0414_16669", "title": "[Diagnosis and treatment of lumbar spinal canal stenosis].", "score": 0.011708371949335805, "content": "Lumbar spinal canal stenosis (LSCS) was first described in 1954 by Verbiest, followed by the currently accepted international classification of LSCS in 1976 by Arnoldi. Briefly, LSCS is a nervous system syndrome that is characterized by neural symptoms in the lower extremities due to tightened cauda equina and spinal nerve root involvement. LSCS international classification consists of: (1) degenerative, (2) congenital developmental, (3) combined, (4) spondylolytic spondylolisthesis, (5) iatrogenic and (6) post traumatic stenosis. Degenerative stenosis-the most common type of LSCS-is caused by disc degeneration, osteoarthritis of the facet joint and hypertrophy of the ligamentum flavum. LSCS may also be the result of intervertebral disc degeneration, protruded intervertebral disc and/or bony spur compress cauda equina and spinal nerve root anteriorally, while degenerated facet joint and hypertrophied the ligamentum flavum compress cauda equina and spinal nerve root posteriorally? Most often, spondylolytic spondylolisthesis occurs at the fourth lumbar vertebrae in middle-aged women. As a result of a slipping forward of the vertebra, cauda equina and spinal nerve roots can be tightened between the edge behind the top of lower vertebra and frontal edge of the lower part of upper lamina. Typical clinical symptoms of LSCS are low back pain, leg pain and intermittent claudication. Low back pain is chronic with secondary radiating pain in the buttock. The leg pain is called \"sciatica\", which tends to appear on the back of thigh, in the lateral aspect of lower leg and calf muscles, and which intensifies when the patient is fatigued. Intermittent claudication is a symptom associated with this syndrome. Often, patients with LSCS find it impossible to walk because of increased numbness and pain in their leg. Many patients report that after squatting for a few minutes they are able to resume walking. LSCS patients may also report dysaesthesia in the perineum area, and may also report urinary dysfunction ranging from extreme urgency to urinary delay. Patients who present with symptoms of LSCS should be seen by an orthopedic surgeon. Correct diagnosis by imaging and clinical examination, with appropriate conservative or operative treatment in a timely fashion should be encouraged in order to prevent irreversible nerve damage."}, {"id": "article-20584_16", "title": "Disc Herniation -- History and Physical -- Physical Examination", "score": 0.011553936494127882, "content": "L5 Nerve - back, radiating into buttock, lateral thigh, lateral calf, and dorsum foot, great toe; sensory loss on the lateral calf, dorsum of the foot, webspace between first and second toe; weakness on hip abduction, knee flexion, foot dorsiflexion, toe extension and flexion, foot inversion and eversion; decreased semitendinosus/semimembranosus reflex. S1 Nerve - back, radiating into buttock, lateral or posterior thigh, posterior calf, lateral or plantar foot; sensory loss on the posterior calf, lateral or plantar aspect of foot;  weakness on hip extension, knee flexion, plantar flexion of the foot; Achilles tendon; Medial buttock, perineal, and perianal region; weakness may be minimal, with urinary and fecal incontinence as well as sexual dysfunction. S2-S4 Nerves - sacral or buttock pain radiating into the posterior aspect of the leg or the perineum; sensory deficit on the medial buttock, perineal, and perianal region; absent bulbocavernosus, anal wink reflex."}, {"id": "wiki20220301en023_60316", "title": "Low back pain", "score": 0.011486398542236105, "content": "Low back pain may be classified based on the signs and symptoms. Diffuse pain that does not change in response to particular movements, and is localized to the lower back without radiating beyond the buttocks, is classified as nonspecific, the most common classification. Pain that radiates down the leg below the knee, is located on one side (in the case of disc herniation), or is on both sides (in spinal stenosis), and changes in severity in response to certain positions or maneuvers is radicular, making up 7% of cases. Pain that is accompanied by red flags such as trauma, fever, a history of cancer or significant muscle weakness may indicate a more serious underlying problem and is classified as needing urgent or specialized attention."}, {"id": "article-24477_15", "title": "Lumbosacral Facet Syndrome -- History and Physical", "score": 0.01124031007751938, "content": "In most presentations, facetogenic lumbosacral pain often presents secondary to chronic pain alone. It should, however, be noted that facet joint pain may be referred distally into the lower limb, thus mimicking sciatica. In these cases of \"pseudo-radicular\" lumbar pain, patients typically experience radiation into the unilateral or bilateral buttocks and the trochanteric region (from L4 and L5 levels), the groin and thighs (from L2 to L5), ending above the knee, and without any neurologic deficits. At times, pain may radiate further down the lower extremity reaching the foot, thus mimicking sciatic pain. This may occur especially in the setting of rather large synovial cysts causing direct mechanical compression and creating ensuing inflammation to further irritate the surrounding nerve roots. [15] Facet joint pain is typically worse in the mornings and following periods of inactivity. Stress exercise, lumbar spinal extension or rotary motions, standing or sitting positions, and facet joint palpation may also elicit lumbar facetogenic pain. [2]"}, {"id": "wiki20220301en191_24071", "title": "GALS screen", "score": 0.011189417759947955, "content": "Now ask the patient to do the following noting any painful, restricted or asymmetrical movements: Test rotation of the thoracic and lumbar spine. Gently hold the patient's hips still and ask them to: \"Turn your shoulders round as far as you can to the left, then do the same to the right.\" Test lateral flexion of the thoracic and lumbar spine: \"Stand up straight and then slide the palm of your right hand down your thigh towards your knee, bending your shoulder down to the side.\" \"Now do the same with your left hand down your left leg.\" \"Bend your left ear down towards your left shoulder and then your right ear down towards your right shoulder\" to test for pain free cervical spine lateral flexion. Now test for stiffness or pain flexing or extending the cervical spine: \"bend your neck forwards to try to touch your chin against your chest.\" \"bend your neck back to lift your chin.\""}, {"id": "Surgery_Schwartz_6384", "title": "Surgery_Schwartz", "score": 0.011091533387548567, "content": "heavy-muscled athletesVenous claudicationEntire leg, but usually worse in thigh and groinTight, bursting painAfter walkingSubsides slowlyRelief speeded by elevationHistory of iliofemoral deep venous thrombosis, signs of venous congestion, edemaNerve root compression (e.g., herniated disk)Radiates down leg, usually posteriorlySharp lancinating painSoon, if not immediately after onsetNot quickly relieved (also often present at rest)Relief may be aided by adjusting back positionHistory of back problemsSymptomatic Baker’s cystBehind knee, down calfSwelling, soreness, tendernessWith exercisePresent at restNoneNot intermittentIntermittent claudication (hip, thigh, buttock)Hip, thigh, buttocksAching discomfort, weaknessAfter same degree of exerciseQuickly relievedNoneReproducibleHip arthritisHip, thigh, buttocksAching discomfortAfter variable degree of exerciseNot quickly relieved (and may be present at rest)More comfortable sitting, weight taken off legsVariable, may relate to activity"}, {"id": "article-28194_14", "title": "Radicular Back Pain -- History and Physical", "score": 0.010895163784285272, "content": "L2, L3, and L4 lumbar radiculopathies are considered a group. This group has a marked overlap of the innervation of the anterior thigh muscles. [1] An acute injury in the distribution of L2, L3, and L4 will most commonly present with the patient experiencing radiating back pain to the anterior aspect of the thigh, which may progress into their knee, and possibly radiate to the medial aspect of the lower leg, into the foot. On examination, patients can have weakness during knee extension, hip adduction, and or hip flexion. There is often a loss of sensation over the anterior thigh along the area of pain. The patient may show a reduced patellar reflex (L4). [1] Activities that can make the symptoms worse include coughing, leg straightening, or sneezing."}, {"id": "pubmed23n0626_20486", "title": "Greater trochanteric pain syndrome: a review of anatomy, diagnosis and treatment.", "score": 0.010795902285263987, "content": "Greater trochanteric pain syndrome (GTPS) is a term used to describe chronic pain overlying the lateral aspect of the hip. This regional pain syndrome, once described as trochanteric bursitis, often mimics pain generated from other sources, including, but not limited to myofascial pain, degenerative joint disease, and spinal pathology. The incidence of greater trochanteric pain is reported to be approximately 1.8 patients per 1000 per year with the prevalence being higher in women, and patients with coexisting low back pain, osteoarthritis, iliotibial band tenderness, and obesity. Symptoms of GTPS consist of persistent pain in the lateral hip radiating along the lateral aspect of the thigh to the knee and occasionally below the knee and/or buttock. Physical examination reveals point tenderness in the posterolateral area of the greater trochanter. Most cases of GTPS are self-limited with conservative measures, such as physical therapy, weight loss, nonsteroidal antiinflammatory drugs and behavior modification, providing resolution of symptoms. Other treatment modalities include bursa or lateral hip injections performed with corticosteroid and local anesthetic. More invasive surgical interventions have anecdotally been reported to provide pain relief when conservative treatment modalities fail."}, {"id": "InternalMed_Harrison_31210", "title": "InternalMed_Harrison", "score": 0.010729804025893411, "content": "2692 LATERAL FEMORAL CUTANEOUS NEUROPATHY (MERALGIA PARESTHETICA) The lateral femoral cutaneous nerve arises from the upper lumbar plexus (spinal levels L2/3), crosses through the inguinal ligament near its attachment to the iliac bone, and supplies sensation to the anterior lateral thigh. The neuropathy affecting this nerve is also known as meralgia paresthetica. Symptoms and signs consist of paresthesias, numbness, and occasionally pain in the lateral thigh. Symptoms are increased by standing or walking and are relieved by sitting. There is normal strength, and knee reflexes are intact. The diagnosis is clinical, and further tests usually are not performed. EDx is only needed to rule out lumbar plexopathy, radiculopathy, or femoral neuropathy. If the symptoms and signs are classic, EMG is not necessary. Symptoms often resolve spontaneously over weeks or months, but the patient may be left with permanent numbness. Treatment consists of weight loss and avoiding tight belts. Analgesics"}, {"id": "article-29356_9", "title": "Spinal Stenosis -- History and Physical", "score": 0.010447761194029851, "content": "Stenosis in the lumbar spine can lead to neurogenic claudication, myeloradiculopathy symptoms, sensory disturbances, motor weakness, and pathologic reflexes. Patients will present with complaints of cramping pain in the leg, calf, and or buttocks. They might report an increase in pain with walking or standing for prolonged periods and relief when sitting or leaning forward while using a shopping cart. [9] Disk herniation is most common at the L4-5 and L5-S1 levels. A herniated disk at L5-S1 can lead to plantarflexion weakness, decrease sensation in the lateral foot, and cause pain in the posterior leg. A disk herniation at L4-5 can lead to a foot drop and numbness in the large toe web and dorsal aspect of the foot. Lastly, an L3-4 disk herniation can lead to knee extension weakness, numbness in the medial foot, and pain in the anterior thigh."}, {"id": "article-22895_7", "title": "Greater Trochanteric Pain Syndrome -- History and Physical", "score": 0.010383244206773619, "content": "Patients with greater trochanteric pain syndrome usually have lateral hip pain, tenderness over and around the greater trochanter, pain at end-range hip rotation, abduction or adduction, pain with resisted hip abduction, and a positive Patrick-FABER (flexion abduction external rotation) test. Patients usually suffer from chronic back pain radiating to the posterolateral aspect of the thigh, leg paresthesias, and tenderness over the iliotibial tract. [6] They will often complain of the inability to lay on the affected hip. The mean duration of symptoms before treatment ranges from 7.1 weeks to 4.4 years. Young adults (18 to 35 years old) with hip pain often present with non-specific symptoms and vague findings from the history and physical examination, which may lead to a misdiagnosis of GTPS or trochanteric bursitis. [1] [4]"}, {"id": "Neurology_Adams_10553", "title": "Neurology_Adams", "score": 0.010271408923939348, "content": "The most characteristic syndrome affects the lumbar roots. Pain, which can be severe, begins in the low back or hip and spreads to the thigh and knee on one side; the discomfort has a deep, aching character with superimposed lancinating jabs and there is a propensity for pain to be most severe at night. Weakness and later atrophy are evident in the pelvic girdle and thigh muscles, although the distal muscles of the leg may also be affected. The weakness can progress for days or weeks (rarely, months). The patellar reflex is lost on the affected side. Curiously, we have found the opposite patellar reflex to be absent in some patients, without explanation. Deep and superficial sensation may be intact or mildly impaired, conforming to either a multiple nerve or multiple adjacent root distribution (i.e., L2 and L3, or L4 and L5). The pain lasts for several days and then gradually abates. Motor recovery is the rule although months and even years may elapse before it is complete. The same"}, {"id": "pubmed23n1089_19591", "title": "Spinal gouty tophus presenting as an epidural mass lesion - A case report.", "score": 0.010071977199169121, "content": "Gout is a metabolic disease secondary to an increased body pool of urate with hyperuricemia. Gout typically affects the peripheral joints and rarely involves the intra-spinal area. A 43-year-old man, who had metabolic syndrome s/p bariatric surgery and gout suffered from severe left low back pain with radiation to the lateral side of his left thigh and anterior side of his left leg for more than 7 days. His L-spine MRI showed an abnormal posterior epidural space occupying lesion at L4-L5 level. For tissue diagnosis and neural structures decompression, he underwent surgical removal of the epidural mass lesion. The surgical specimen showed a picture of gout and he got a good recovery after operation. The differential diagnoses of an epidural mass includes synovial cysts, ligament cyst, cystic neuromas, tumors, hematomas and abscesses. Gout in the spinal canal is difficult to diagnosis before surgery because it is rare and its clinical presentation and radiologic findings mimic tumor, abscess, tuberculosis, and degenerative spinal diseases. Patients with spinal gout may present with axial pain and a variety of neurological symptoms. Spinal gouty tophus should be considered in the different diagnoses of spinal epidural masses especially in patients with systemic gout. Surgery is needed for final diagnosis. If spinal gouty tophus is highly suspected during the surgery, the specimen should not be preserved with Formalin because birefringent crystals under polarized light is a unique feature for gouty tophus but they dissolve in Formalin."}, {"id": "article-28194_19", "title": "Radicular Back Pain -- History and Physical", "score": 0.009977002164502164, "content": "A straight leg raising can be helpful in lumbosacral radiculopathy. The mechanism of pain during a straight leg raise is increased dural tension placed upon the lumbosacral spine during the test. Patients lay supine during the test. The physician will flex the patient's quadriceps with the leg in extension as well as dorsiflex the patient's foot on the symptomatic side. Pain or reproduction of paresthesias is considered a positive test (Lasegue's sign). A Bowstring sign relieves this underlying radicular pain with flexion of the patient's knee on the affected side. The straight leg raising test is most helpful in the diagnosis of L4 and S1 radiculopathies. [11] [3]"}, {"id": "pubmed23n0222_2576", "title": "[Acromegaly and spinal canal stenosis].", "score": 0.009900990099009901, "content": "A rare case of acromegaly with radiculomyelopathy due to spinal canal stenosis is reported. A long history of acromegalic deformity was seen on this 55 years old acupuncture therapist for the last 18 years, while he had developed unusually increased appetite, profuse perspiration and gained weight. Fifteen years ago, acromegalic tendency became prominent and was accompanied by low back and knee pain. In 1974 CB-154 was administered in several occasions beside 4000 rads irradiation to the sella. After radiation therapy was completed his outlook was somewhat improved, although low back pain was aggravated and associated with atrophy of the left lower extremity. The neurological examination at admission in 1980 revealed acromegalic feature, increased DTRs on both upper and lower extremities, dysesthesia of feet and atrophy of the leg muscles in general, mainly due to diffuse atrophy. Patient bended knees due to pain and unable to walk. He had radiating pain in the postero-lateral aspect of the left lower extremity and some dysesthesia of feet, although no specific root lesion was identifiable. Spinal roentgenograms disclosed diffuse ossification of anterior and posterior longitudinal ligaments, and marked spondylotic changes. Although the low back pain was impending complaint of the patient, the hypophyseal tumor was advised to remove and transsphenoidal hypophyseal adenomectomy was performed. A good amount of softend tumor tissue was removed however, adenoma found markedly fibrotic. Low back pain and knee were decreased gradually, but intermittent claudication was evident. Acromegaly in association with spinal canal stenosis were reviewed in literature, and the relationship of growth hormone and therapeutic problems were discussed."}, {"id": "pubmed23n1017_12062", "title": "Diabetic amyotrophy, not your typical back pain.", "score": 0.00980392156862745, "content": "A 49-year-old man presented to the hospital for spinal cord decompression surgery with left buttock and left leg pain. The patient described an acute burning pain radiating down from his left buttock to left lateral leg. He also noted a 13.6 kg weight loss in recent months. Physical examination showed decreased muscle bulk of the left thigh, decreased strength of the left hip, left knee flexors and extensors. Recent MRI spine showed mild canal narrowing and cord flattening in the lower thoracic spine. Serologic testing showed an elevated glucose of 17.9 mmol/L and haemoglobin A1c of 9.8%. Electromyography showed denervation of scattered muscles of the left knee flexors, hip flexors and adductors. In the setting of newly diagnosed diabetes mellitus, he was diagnosed with diabetic amyotrophy, started on insulin therapy, and his surgery was cancelled."}, {"id": "pubmed23n0977_21644", "title": "Comparison of the history and physical examination for hip osteoarthritis and lumbar spinal stenosis.", "score": 0.00980392156862745, "content": "Leg pain associated with walking is sometimes incorrectly attributed to hip osteoarthritis (OA) or lumbar spinal stenosis (LSS). This study compared physicians' values of signs and symptoms for diagnosing and differentiating hip OA and LSS to their clinical utility. Musculoskeletal physicians were surveyed with online questionnaires. Patients were recruited from hip and spine specialty practices. Seventy-seven hip OA and 79 LSS patients. Signs and symptoms of hip OA and LSS. Fifty-one of 66 invited musculoskeletal physicians completed online surveys about the values of 83 signs and symptoms for diagnosing hip OA and LSS. Of these, the most valued 32 symptoms and 13 physical examination items were applied to patients with symptomatic hip OA or LSS. Positive likelihood ratios (+LR) were calculated for each items' ability to differentiate hip OA from LSS, with a +LR&gt;2 set as indicating usefulness for favoring either diagnosis. Positive LRs were compared with surveyed physicians' values for each test. All symptoms were reported by some patients with each diagnosis. Only 11 of 32 physician-valued symptoms were useful for discriminating hip OA from LSS. Eight symptoms favored hip OA over LSS: groin pain (+LR=4.9); knee pain (+LR=2.2); pain that decreased with continued walking (+LR=3.9); pain that occurs immediately with walking (+LR=2.4); pain that occurs immediately with standing (+LR=2.1); pain getting in/out of a car (+LR=3.3); pain with dressing the symptomatic leg (+LR=3.1); and difficulty reaching the foot of the symptomatic leg while dressing (+LR=2.3). Three symptoms favored LSS over hip OA: pain below the knee (+LR=2.3); leg tingling and/or numbness (+LR=2.7); and some pain in both legs (+LR=2.5). Notable symptoms that did not discriminate hip OA from LSS included: pain is less while pushing a shopping cart (+LR=1.0); back pain (+LR=1.1); weakness and/or heaviness of leg (+LR=1.1); buttocks pain (+LR=1.2); poor balance or unsteadiness (+LR=1.2); pain that increased with weight-bearing on the painful leg (+LR=1.3), and step to gait on stairs (+LR=1.7). Consistent with physicians' expectations, 7 of 13 physical examination items strongly favored hip OA over LSS: limited weight-bearing on painful leg when standing (+LR=10); observed limp (+LR=9); and painful and restricted range-of-motion with any of five hip maneuvers (+LR range 21-99). Four of five tested neurological deficits (+LR range 3-8) favored the diagnosis of LSS over hip OA. There is substantial crossover of symptoms between hip OA and LSS, with some physician-valued symptoms useful for differentiating these disorders whereas others were not. Physicians recognize the value of the examination of gait, the hip, and lower extremity neurological function for differentiating hip OA from LSS. These tests should be routinely performed on all patients for which either diagnosis is considered. Awareness of these findings might reduce diagnostic errors."}, {"id": "pubmed23n1147_26703", "title": "Pudendal tumor mimicking cauda equina syndrome and acute radiculopathy: case report.", "score": 0.009708737864077669, "content": "Cauda equina syndrome (CES) is most caused by lumbar disc herniation, and the associated treatment involves prompt surgical decompression. Rarer causes of CES include perineural (Tarlov) cysts. A 62-year-old female with history of rheumatoid arthritis, hip and knee replacements, and chronic low back pain presented with worsening back pain, left leg weakness and pain for 6 weeks, and bowel/bladder incontinence with diminished sensation in the perianal region for 24 h prior to presentation. MRI demonstrated severe spinal stenosis at L4-S1, central disc herniation at L5-S1, and compression of the cauda equina, consistent with CES. A lumbar decompression was performed. Patient did well at 2-week follow up, but presented 5 weeks post-discharge with increased left leg pain/weakness and genitalia anesthesia. Imaging was unremarkable. Two months later, the patient presented with diminished sensation in the buttocks and bilateral lower extremities and bowel/bladder incontinence. Imaging demonstrated a large cystic presacral mass with involvement of the left sciatic foramen and S3 neural foramen. A team of plastic, orthopedic, and neurological surgeons performed an S3 sacral laminectomy, foraminotomy, partial sacrectomy, and S3 rhizotomy, and excision of the large left hemorrhagic pudendal mass. Final pathology demonstrated a perineural cyst with organizing hemorrhage. On follow-up, the patient's pain and weakness improved. CES-like symptoms were initially attributed to a herniated disk. However, lumbar decompression did not resolve symptoms, prompting further radiographic evaluation at two separate presentations. This represents the first reported case of a pudendal tumor causing symptoms initially attributed to a herniated disc."}, {"id": "pubmed23n0681_16103", "title": "Does this older adult with lower extremity pain have the clinical syndrome of lumbar spinal stenosis?", "score": 0.009708737864077669, "content": "The clinical syndrome of lumbar spinal stenosis (LSS) is a common diagnosis in older adults presenting with lower extremity pain. To systematically review the accuracy of the clinical examination for the diagnosis of the clinical syndrome of LSS. MEDLINE, EMBASE, and CINAHL searches of articles published from January 1966 to September 2010. Studies were included if they contained adequate data on the accuracy of the history and physical examination for diagnosing the clinical syndrome of LSS, using a reference standard of expert opinion with radiographic or anatomic confirmation. Two authors independently reviewed each study to determine eligibility, extract data, and appraise levels of evidence. Four studies evaluating 741 patients were identified. Among patients with lower extremity pain, the likelihood of the clinical syndrome of LSS was increased for individuals older than 70 years (likelihood ratio [LR], 2.0; 95% confidence interval [CI], 1.6-2.5), and was decreased for those younger than 60 years (LR, 0.40; 95% CI, 0.29-0.57). The most useful symptoms for increasing the likelihood of the clinical syndrome of LSS were having no pain when seated (LR, 7.4; 95% CI, 1.9-30), improvement of symptoms when bending forward (LR, 6.4; 95% CI, 4.1-9.9), the presence of bilateral buttock or leg pain (LR, 6.3; 95% CI, 3.1-13), and neurogenic claudication (LR, 3.7; 95% CI, 2.9-4.8). Absence of neurogenic claudication (LR, 0.23; 95% CI, 0.17-0.31) decreased the likelihood of the diagnosis. A wide-based gait (LR, 13; 95% CI, 1.9-95) and abnormal Romberg test result (LR, 4.2; 95% CI, 1.4-13) increased the likelihood of the clinical syndrome of LSS. A score of 7 or higher on a diagnostic support tool including history and examination findings increased the likelihood of the clinical syndrome of LSS (LR, 3.3; 95% CI, 2.7-4.0), while a score lower than 7 made the diagnosis much less likely (LR, 0.10; 95% CI, 0.06-0.16). The diagnosis of the clinical syndrome of LSS requires the appropriate clinical picture and radiographic findings. Absence of pain when seated and improvement of symptoms when bending forward are the most useful individual findings. Combinations of findings are most useful for identifying patients who are unlikely to have the diagnosis."}, {"id": "pubmed23n0621_10286", "title": "Bilateral periprosthetic stress fractures in a juvenile chronic arthritis patient secondary to bilateral genu valgum.", "score": 0.009615384615384616, "content": "Lateral insufficiency fractures following total hip replacement have been reported with the femoral stems positioned in varus, together with osteopenia of the lateral femoral cortex. Any abnormal alignment of the lower limbs, such as genu valgum, will alter the load distribution across the femoral cortices, and repetitive loading during walking will predispose the bones to stress fractures at any stress riser point, such as the tip of a femoral component. Bilateral femoral stress fractures post total hip replacements have not been previously described. We present a 55-year-old woman, diagnosed with juvenile idiopathic arthritis, who had undergone bilateral total hip replacements and bilateral knee replacements. The knees 15 years postoperatively were in valgus and the left knee was extremely stiff, flexing to just 5. The patient presented with bilateral thigh pain, with plain radiographs confirming bilateral periprosthetic fractures of the femur at the tip of well-fixed femoral components. There was no history of injury and her hips were functioning well up to this time. The patient required revision of both hips to long-stem uncemented components, bypassing the fractures, and revision of both knees to stemmed semi-constrained implants, thereby correcting the alignment of both lower limbs. Both fractures healed and the patient is currently pain-free and mobile with walking aids. Surgeons must remain aware that when implants are in situ, abnormal alignments will lead to abnormal forces, and stress fractures are likely to occur at any stress riser around the implant. Avoiding malalignment will avoid this complication."}, {"id": "article-27291_20", "title": "Plantar Heel Pain -- History and Physical -- S1 Radiculopathy", "score": 0.009615384615384616, "content": "Patients with heel pain may have a history of chronic lower back pain. Clinically, radicular involvement will present as pain and numbness radiating from the lower back down to the heel of the foot. Patients may have decreased sensation over the skin of the sole, heel, or lateral foot and weakness of the gastrocnemius, gluteus maximus, hamstring, peroneal, and foot muscles with a diminished ankle reflex. Perform the positive straight leg raise test (Lasègue test) with the patient in the supine position. Pain with passive hip flexion while lifting the leg suggests stretching the lower lumbar and sacral roots. [21] Perform a crossed straight leg raise test, eliciting pain on the contralateral side with the same motion. Results from new studies suggest that these exams may not be as reliable as we once thought. [22]"}, {"id": "wiki20220301en175_10188", "title": "Neurogenic claudication", "score": 0.009540427846265409, "content": "Knee to chest stretch - Laying down on the back, bring one leg up and pull it towards the chest and hold for 30–45 seconds. Posterior pelvic tilt (bridges) - Laying on the back, bend both legs and place your feet on the floor. Raise stomach from the ground, lifting the back and pelvis, until the back is straight. Hold for 5–10 seconds and relax. Neural Stretching of the legs - Laying on the back, bring one leg up with a stretching band until a stretch is felt in the legs. Ensure your legs are straight. Once the stretch is felt, hold for 30–45 seconds and relax. Hip-flexor stretch - To stretch the right hip-flexor, bring the left leg forward, and kneel on the right knee. Push the pelvis forward (lean forward), whilst keeping the upper body straight. Hold the position for 30–45 seconds and relax. To stretch the left hip-flexor, bring swap the positions of the legs."}, {"id": "pubmed23n0553_3822", "title": "Deep vein thrombosis in an athletic military cadet.", "score": 0.009523809523809525, "content": "Resident's case problem. A 21-year-old healthy athletic male military cadet with complaint of worsening diffuse left knee pain was evaluated 4 days after onset. The knee pain began 2 hours after completing a long car trip, worsened over the subsequent 3 days, and became almost unbearable during the return trip. The patient reported constant pain, limited knee motion, and difficulty ambulating. In addition, he was unable to perform physical military training or attend academic classes due to the severe left knee pain. Past medical history revealed a mild left lateral calf strain 21/2 weeks prior, which completely resolved within 24 hours of onset. Our physical examination led us to either monoarticular arthritis, pseudothrombophlebitis (ruptured Baker's cyst), or a lower leg deep vein thrombosis (DVT) as the cause of knee pain. Diagnostic imaging of this patient revealed a left superficial femoral vein thrombosis and popliteal DVT, with bilateral pulmonary emboli (PE). A systematic differential diagnosis was undertaken to rule out a potentially fatal DVT diagnosis as the cause of knee pain, despite minimal DVT risk factors. The physical therapist in a direct-access setting must ensure timely evaluation and referral of a suspected DVT, even when patient demographics cause the practitioner to question the likelihood of this diagnosis. The physical examination findings, clinical suspicion, and established clinical prediction rules can accurately dictate the appropriate referral action necessary."}, {"id": "pubmed23n0839_300", "title": "Multiple Venous Thromboses Presenting as Mechanical Low Back Pain in an 18-Year-Old Woman.", "score": 0.009433962264150943, "content": "The purpose of this case report is to describe a patient who presented with acute musculoskeletal symptoms but was later diagnosed with multiple deep vein thrombosis (DVT). An 18-year-old female presented to a chiropractic clinic with left lumbosacral pain with referral into the posterior left thigh. A provisional diagnosis was made of acute myofascial syndrome of the left piriformis and gluteus medius muscles. The patient received 3 chiropractic treatments over 1 week resulting in 80% improvement in pain intensity. Two days later, a sudden onset of severe abdominal pain caused the patient to seek urgent medical attention. A diagnostic ultrasound of the abdomen and pelvis were performed and interpreted as normal. Following this, the patient reported increased pain in her left leg. Evaluation revealed edema of the left calf and decreased left lower limb sensation. A venous Doppler ultrasound was ordered. Doppler ultrasound revealed reduction of the venous flow in the femoral vein area. An additional ultrasonography evaluation revealed an extensive DVTs affecting the left femoral vein and iliac axis extending towards the vena cava. Upon follow-up with a hematologist, the potential diagnosis of May-Thurner syndrome was considered based on the absence of blood dyscrasias and sustained anatomical changes found in the left common iliac vein at its junction with the right common iliac artery. A week following discharge, she presented with chest pain and was diagnosed with venous thromboembolism. The patient was successfully treated with anticoagulation therapy and insertion of a vena cava filter. Although DVTs are common in the general population, presence in low-risk individuals may be overlooked. In the presence of subtle initial clinical signs such as those described in this case report, clinicians should keep a high index of suspicion for a DVT. Rapid identification of such clinical signs in association with a lack of objective examination findings warrants further evaluation due to potentially negative outcomes."}, {"id": "pubmed23n0374_2491", "title": "What was the disease of the legs that afflicted King Asa?", "score": 0.009433962264150943, "content": "The elderly have suffered from pain in their legs, which may be associated with various diseases, for thousands of years. This report analyzes the disease that afflicted the biblical King Asa (the third king of the house of Judah who reigned between 867 and 906 BCE). The sentence 'Nevertheless in the time of his old age he was diseased in his legs' indicates that King Asa suffered from disease in his legs. Among numerous diseases, peripheral vascular disease, gout, and degenerative osteoarthritis were most likely to affect the King's legs. And among these diseases, the diagnosis of peripheral vascular disease is the most acceptable. This report shows that the roots of contemporary modern gerontology can be traced back to biblical times."}, {"id": "article-24453_17", "title": "Lumbar Disc Herniation -- History and Physical", "score": 0.00942019145902641, "content": "L5 nerve root exits at the L5-S1 foramina. When compressed by a herniated disc, it causes back pain that radiates into the buttock, lateral thigh, lateral calf, the dorsum of the foot, and the great toe. Sensory loss is present on the web space between the big toe and second toe, the dorsum of the foot, and lateral calf. There is a weakness in hip abduction, knee flexion, foot dorsiflexion, big toe dorsiflexion, foot inversion, and eversion. Patients present with decreased semitendinosus/semimembranosus reflex. Weakness in foot dorsiflexion makes it challenging to walk on the heels. Chronic L5 radiculopathy may cause atrophy of the extensor digitorum brevis and the tibialis anterior of the anterior leg."}, {"id": "pubmed23n0948_124", "title": "Repetitive nerve block for neuropathic pain management: a case report.", "score": 0.009345794392523364, "content": "Schwannoma is a common neoplasm in the peripheral and central nervous systems. Sciatic nerve schwanommas are rare. We report the case of a 50-year-old woman who was referred for treatment of persistent neuropathic pain in the left lower limb after resection of a schwannoma on the left S1 nerve root. The patient's history goes back when she was 27 years old and started to have electric-like pain in her lower left limb upon intercourse. Examination revealed a left ovarian cyst which was surgically removed. Her pain persisted despite taking nonsteroidal anti inflammatory drugs (NSAIDs). Several years later a schwannoma on the left S1 nerve root was detected. The patient had surgical excision of the left S1 nerve root at the plexus along with the schwannoma. Following the surgery, she experienced pain upon sitting and touch, and had a limp in her left leg. She was prescribed NSAIDs, antidepressant and pregabalin. Despite the pharmacological treatment, the patient had persistent mild pain. Upon physical examination, the incision from her previous surgery was 4 cm away from the sacral midline and parallel to S1 and S2. The length of the incision was 3 cm. The patient had severe allodynia upon palpation at the area between S1 and L5 and the visual analog scale (VAS) score increased from 3 to 10. She had severe pain at rest and movement. Her neurologic exam revealed that the left lower extremity motor power showed mild weakness in the leg abduction, foot eversion, plantar and toes flexion, and in the hip extension. The sensory exam showed severe reduction in pinprick and temperature sensation in the lateral aspect of foot, lower leg and dorsolateral thigh and buttocks. Nerve stimulator guided injection was performed at the pain trigger point being 1 cm above the midline of the incision. Upon nerve stimulation the contraction of the gluteal muscle was observed. Then, 20 mL of the anesthetic mixture were injected. The patient had immediate pain relief after the block (VAS 1/10). She remained pain free for 15 days after which pain reappeared but with less severity (3/10). Repetitive sciatic nerve block was performed in a progressive manner and was shown to be effective in managing neuropathic pain."}]}}}}
{"correct_option": 5, "explanations": {"1": {"exist": true, "char_ranges": [[1000, 1109]], "word_ranges": [[154, 168]], "text": "although there are forms of peripheral polyneuropathy \"pseudosyringomyelic\" they are usually of lumbar onset."}, "2": {"exist": true, "char_ranges": [[788, 900]], "word_ranges": [[119, 137]], "text": "MS and other spinal cord lesions would be accompanied by other exploratory signs such as exaltation of reflexes,"}, "3": {"exist": true, "char_ranges": [[901, 978]], "word_ranges": [[137, 149]], "text": "for carpal tunnel the exploration exceeds the territory distal to the carpus,"}, "4": {"exist": true, "char_ranges": [[788, 900]], "word_ranges": [[119, 137]], "text": "MS and other spinal cord lesions would be accompanied by other exploratory signs such as exaltation of reflexes,"}, "5": {"exist": true, "char_ranges": [[579, 787]], "word_ranges": [[88, 119]], "text": "I believe that the correct answer is 5, that is a syringomyelic lesion, whose initial characteristic is the sensitive dissociation with anesthesia for the thermoalgesic and conservation of the posterior cord."}}, "full_answer": "This year's Neurology section's burning question. He presents a diabetic patient on insulin treatment with good control (although diabetic neuropathies do not only develop in patients with poor control) with a sensory picture limited to the upper limbs and of short onset time, without motor symptoms or at any other level. In the examination there is a clear dissociation with thermoalgesic anesthesia and preservation of arthrokinetic and vibratory. Reflexes are normal, neither abolished nor exalted. In addition, the rest of the examination is strictly normal. With all this I believe that the correct answer is 5, that is a syringomyelic lesion, whose initial characteristic is the sensitive dissociation with anesthesia for the thermoalgesic and conservation of the posterior cord. MS and other spinal cord lesions would be accompanied by other exploratory signs such as exaltation of reflexes, for carpal tunnel the exploration exceeds the territory distal to the carpus, and as for answer 1, although there are forms of peripheral polyneuropathy \"pseudosyringomyelic\" they are usually of lumbar onset. The short evolution time (2 weeks), the respect of the lower limbs and the preservation of muscle reflexes make me rule out this response despite the distribution being \"glove-like\".", "full_answer_no_ref": "This year's Neurology section's burning question. He presents a diabetic patient on insulin treatment with good control (although diabetic neuropathies do not only develop in patients with poor control) with a sensory picture limited to the upper limbs and of short onset time, without motor symptoms or at any other level. In the examination there is a clear dissociation with thermoalgesic anesthesia and preservation of arthrokinetic and vibratory. Reflexes are normal, neither abolished nor exalted. In addition, the rest of the examination is strictly normal. With all this I believe that [HIDDEN], that is a syringomyelic lesion, whose initial characteristic is the sensitive dissociation with anesthesia for the thermoalgesic and conservation of the posterior cord. MS and other spinal cord lesions would be accompanied by other exploratory signs such as exaltation of reflexes, for carpal tunnel the exploration exceeds the territory distal to the carpus, and as for answer 1, although there are forms of peripheral polyneuropathy \"pseudosyringomyelic\" they are usually of lumbar onset. The short evolution time (2 weeks), the respect of the lower limbs and the preservation of muscle reflexes make me rule out this response despite the distribution being \"glove-like\".", "full_question": "A 32-year-old diabetic patient on insulin therapy with good control of his blood glucose levels comes to your office with tingling in both hands, with a sensation of corking and thermal insensitivity of progressive onset over the course of 2 weeks. She does not report visual disturbances, strength deficit, motor clumsiness or other symptoms. On examination she found anesthesia to pain and temperature in both hands and distal forearms; positional and vibratory sensitivity were preserved. There is no muscle atrophy or strength deficit. Muscle reflexes are normal and symmetrical. There is no dysmetria, dysdiadochokinesia or intention tremor. The rest of the neurological examination is strictly normal. Indicate the most likely diagnosis in this case:", "id": 127, "lang": "en", "options": {"1": "Peripheral sensitvo symmetric distal peripheral neuropathy of diabetic cause.", "2": "Compressive cervical spinal cord injury.", "3": "Bilateral carpal tunnel syndrome.", "4": "Multiple sclerosis type demyelinating disease.", "5": "Central cervical spinal cord injury."}, "question_id_specific": 77, "type": "NEUROLOGY AND NEUROSURGERY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en094_13074", "title": "Intention tremor", "score": 0.017653890824622535, "content": "Intention tremors can be a first sign of multiple sclerosis, since loss or deterioration of motor function and sensitivity are often one of the first symptoms of cerebellar lesions. Intention tremors have a variety of other recorded causes as well. These include a variety of neurological disorders, such as stroke, alcoholism, alcohol withdrawal, peripheral neuropathy, Wilson's disease, Creutzfeldt–Jakob disease, Guillain–Barré syndrome and fragile X syndrome, as well as brain tumors, low blood sugar, hyperthyroidism, hypoparathyroidism, insulinoma, normal aging, and traumatic brain injury. Holmes tremor, a rubral or midbrain tremor, is another form of tremor that includes intention tremors, among other symptoms. This disease affects the proximal muscles of the head, shoulders, and neck. Tremors of this disease occur at frequencies of 2–4 Hz or more."}, {"id": "pubmed23n1157_5293", "title": "[A Case of Hirayama Disease Presenting Horn Hand: Spinal Horn Hand].", "score": 0.017125292740046837, "content": "A 28-year-old man with Hirayama disease presented with a peculiar hand posture called \"horn hand\" (\"main en corne\"). The patient noticed that he could not extend his 3rd and 4th fingers when extending the fingers of his right hand 1 year prior to presentation. On neurological examination, the strength of the finger extension is severely weak in the 1st, 3rd and 4th fingers, causing a drop finger posture, but it is only moderately weak in the 2nd and 5th fingers, enabling him to sustain the extension finger posture. The finger posture is like a bull's horns and is named \"horn hand\". Amyotrophy of the ulnar side of the right forearm, so-called \"oblique amyotrophy\", and amyotrophy of the right hand are observed. MRI examination reveals atrophy of the lower cervical spinal cord and compression of it in the neck flexion posture. Diagnosis of Hirayama disease (juvenile muscular atrophy of the distal upper extremity) is confirmed. Horn hand has been reported so far in some cases of lead neuropathy, chronic inflammatory demyelinating polyneuropathy, and distal myopathy; a \"peripheral\" horn hand is characterized by partial and selective lesion of the radial nerve or of the finger extensor muscles. Meanwhile, a \"spinal\" horn hand in this patient with Hirayama disease represents the partial and selective lesion of the spinal anterior horn neurons."}, {"id": "wiki20220301en120_27085", "title": "Amyotrophy", "score": 0.014264880138547077, "content": "Amyotrophy is progressive wasting of muscle tissues. Muscle pain is also a symptom. It can occur in middle-aged males with type 2 diabetes. It also occurs with motor neuron disease. Differential diagnosis The following are considered differential diagnosis for Amyotrophy: compressive and infective causes of polyradiculopathy structural disc diseases chronic demyelinating neuropathies See also Diabetic amyotrophy Monomelic amyotrophy Amyotrophic lateral sclerosis References External links Muscular disorders"}, {"id": "pubmed23n0345_14736", "title": "[An elderly case of juvenile muscular atrophy in the unilateral upper extremity with tremor in both hands].", "score": 0.014200163324562198, "content": "A 75-year-old man had noticed muscle atrophy and weakness of his right hand and forearm at the age of 25. The symptoms slowly progressed and then stopped. Right hand tremor appeared at about age 40. There was no symptom in his left upper extremity, and his gait was normal. He now shows severe muscle atrophy in his right hand and forearm. There was distally dominant weakness of the right upper extremity and his hand grip was 0 kg on the right and 25 kg on the left. On admission there was no weakness in the bilateral lower extremities. He had postural tremor in both hands and fingers. The tendon reflexes were hypoactive in the upper extremities and normal in the lower extremities. Abnormalities in the superficial sensation were unremarkable, whereas vibration sensation in both the upper and lower extremities was mildly disturbed. Electromyography revealed chronic denervation, especially in the right upper extremity. The sensory nerve conduction study results and somatosensory evoked potentials in the upper extremities were normal. Cervical MRI demonstrated spondylotic changes, canal stenosis from the C5 to C7 levels, and compression of the spinal cord. His hand tremor was dominant on the right with a peak frequency of about 7 Hz. Tremor frequency and power were decreased by mechanical load. Hirayama's disease (juvenile muscular atrophy of unilateral upper extremity) was the most probable diagnosis, although aging might have produced various additional abnormalities. The tremor seen in this patient showed characteristics of enhanced physiological tremor."}, {"id": "wiki20220301en508_25644", "title": "Monomelic amyotrophy", "score": 0.013725490196078431, "content": "The disease is rare and several cited cases deviate from the expected norm, making diagnosis more difficult. Proposed diagnostic criteria: Distal predominant muscle weakness and atrophy in forearm and hand Involvement of the unilateral upper extremity almost always all the time Onset between the ages of 10 to early 20s Insidious onset with gradual progression for the first several years, followed by stabilization No lower extremity involvement No sensory disturbance and tendon reflex abnormalities Exclusion of other diseases (e.g., motor neuron disease, multifocal motor neuropathy, brachial plexopathy, spinal cord tumors, syringomyelia, cervical vertebral abnormalities, anterior interosseous, or deep ulnar neuropathy)"}, {"id": "article-22550_11", "title": "Hand Nerve Compression Syndromes -- History and Physical -- Median Nerve", "score": 0.012548732943469785, "content": "Median nerve compression at the hand and wrist is called carpal tunnel syndrome. It is the most common type of nerve injury and results from compression of the median nerve at the wrist as it passes between the carpal bones and the flexor retinaculum. It is typically the result of repetitive use of the hands but can be the result of other factors such as obesity, diabetes, pregnancy, and hypothyroidism. Patients often report numbness, tingling, and pain that worsens at night. These symptoms can be elicited from activities that involve prolonged wrist flexion and/or extension. Shaking the hand may alleviate the symptoms. They may also be weakness and clumsiness of the hand with activities such as gripping and grasping. The physical exam is an important component in the diagnosis of carpal tunnel as it can help distinguish carpal tunnel from other diagnoses such as proximal median neuropathy (pronator syndrome) and cervical radiculopathy. Sensory symptoms involve the thumb, index, long, and lateral half of the ring finger. There will be no sensory loss at the thenar eminence with carpal tunnel syndrome when compared to more proximal lesions. If motor weakness is present, it is typically evident with weakness of thumb abduction. There may also be atrophy of the thenar eminence as the median nerve innervates many muscles in this region. Signs that will be absent in carpal tunnel syndrome include weakness in forearm pronation, finger abduction, and finger extension. There are several provocative tests used to diagnose carpal tunnel syndrome [8] [10] :"}, {"id": "article-24839_21", "title": "Median Nerve Palsy -- History and Physical", "score": 0.012511956156451155, "content": "Diagnosticians must always evaluate the neck in patients with presumed median nerve injury or carpal tunnel syndrome. Double crush syndrome is defined as an injury to a nerve at both a distal site of compression as well as proximally such as in the case of a coexistent cervical disk herniation or foraminal stenosis. This condition is most commonly identified when patients have an unsatisfactory resolution of symptoms after a carpal tunnel release. [10] Osterman et al. found in a prospective study that patients who suffered from double crush syndrome reported more \"paresthesias\" rather than \"numbness\" compared to patients who had isolated carpal tunnel syndrome. He also found that grip strength was decreased more with double crush syndrome compared to carpal tunnel syndrome. [11] Of note, radiography of the cervical spine is not currently recommended for evaluation of double crush syndrome by current literature, particularly in the older patient population as there is a very high incidence of asymptomatic degenerative changes of the spine. MRI may be useful but is cost-prohibitive and not necessary in most cases. Therefore, history and physical examination with documentation of such examination techniques as Spurling's cervical spine maneuver to identify cervical nerve root compression, are important tools to identify double crush syndromes. [10] [12] [13]"}, {"id": "wiki20220301en023_25278", "title": "Peripheral neuropathy", "score": 0.012370125901479036, "content": "Most types of polyneuropathy progress fairly slowly, over months or years, but rapidly progressive polyneuropathy also occurs. It is important to recognize that at one time it was thought that many of the cases of small fiber peripheral neuropathy with typical symptoms of tingling, pain, and loss of sensation in the feet and hands were due to glucose intolerance before a diagnosis of diabetes or pre-diabetes. However, in August 2015, the Mayo Clinic published a scientific study in the Journal of the Neurological Sciences showing \"no significant increase in...symptoms...in the prediabetes group\", and stated that \"A search for alternate neuropathy causes is needed in patients with prediabetes.\" The treatment of polyneuropathies is aimed firstly at eliminating or controlling the cause, secondly at maintaining muscle strength and physical function, and thirdly at controlling symptoms such as neuropathic pain."}, {"id": "wiki20220301en508_25646", "title": "Monomelic amyotrophy", "score": 0.012353490614360179, "content": "In early stages of the disease MMA may be confused for amyotrophic lateral sclerosis (ALS), cervical spondylotic amyotrophy (CSA), and other challenging neurological diseases, as well as conditions that are minor but that call for very different treatments, such as advanced carpal tunnel syndrome (CTS). Symptoms somewhat differ. Pain and tingling in the hand is typically present in CTS and absent from MMA; loss of function presents differently; with careful electrophysiological study and neurological exams the two are distinguished. In early stages, ALS, SCA, and MMA, presentation may be similar. Both CSA and ALS ultimately have more extensive symptoms. MMA is more prevalent in young people while ALS and CSA are more common in older populations. With ALS, hand symptoms usually more commonly both proximal and distal vs in MMA mostly distal only, and with ALS fasciculations (twitching) are often present in upper extremities, but rarely in MMA. MMA is usually eliminated from"}, {"id": "Neurology_Adams_8895", "title": "Neurology_Adams", "score": 0.012253267126164183, "content": "Over the years, the authors have encountered young men with localized and asymmetrical amyotrophy of the forearm that became arrested and did not advance over a decade or two, typical of Hirayama disease. Reports of such a partial cervical spinal amyotrophy have appeared (Hirayama et al; Moreno Martinez et al). In the type described by Hirayama and associates, young men are affected with progressive and asymmetrical amyotrophy of the forearm and hand that has been traced to ligamentous hypertrophy and buckling in the ventral spinal canal. This causes a compression of the cervical spinal cord gray matter, presumably by a chronic ischemic effect as discussed in detail in Chap. 42. In a familial variety of pure restricted amyotrophy, only the vocal cords became paralyzed over a period of years in adult life; only later were the hands affected."}, {"id": "Neurology_Adams_1699", "title": "Neurology_Adams", "score": 0.01207059003285034, "content": "Unlike herniated lumbar discs, cervical ones, if large and centrally situated, result in compression of the spinal cord (Fig. 10-6). The centrally situated disc is often painless, and the cord syndrome may simulate multiple sclerosis or a degenerative neurologic disease. Bilateral hand numbness, paresthesia, or similar altered sensation is common. Failure to consider a protruded cervical disc in patients with obscure symptoms in the legs, including stiffness and falling, is a common error. A vague sensory change can often be detected on the thorax, the rostral margin of which is several dermatomes below the level of compression. The diagnosis and the level of disc protrusion can be confirmed by MRI or by CT myelography. Nerve conduction studies, F responses, and EMG are helpful in confirming the level of root compression and distinguishing pain of radicular origin from that originating in the brachial plexus or in individual nerves of the arm (see “Brachial Neuritis” in Chap. 43)."}, {"id": "InternalMed_Harrison_1903", "title": "InternalMed_Harrison", "score": 0.010654160654160656, "content": "Isolated mononeuropathies may cause symptoms beyond the territory supplied by the affected nerve, but abnormalities on examination typically are confined to appropriate anatomic boundaries. In multiple mononeuropathies, symptoms and signs occur in discrete territories supplied by different individual nerves and—as more nerves are affected—may simulate a polyneuropathy if deficits become confluent. With polyneuropathies, sensory deficits are generally graded, distal, and symmetric in distribution (Chap. 459). Dysesthesias, followed by numbness, begin in the toes and ascend symmetrically. When dysesthesias reach the knees, they usually also have appeared in the fingertips. The process is nerve length–dependent, and the deficit is often described as “stocking-glove” in type. Involvement of both hands and feet also occurs with lesions of the upper cervical cord or the brainstem, but an upper level of the sensory disturbance may then be found on the trunk and other evidence of a central"}, {"id": "wiki20220301en297_7787", "title": "Hand injury", "score": 0.010401446296342157, "content": "Carpal tunnel syndrome is a common disorder of the hand. This disorder results from compression of an important nerve in the wrist. Disorders like diabetes mellitus, thyroid or rheumatoid arthritis can narrow the tunnel and cause impingement of the nerve. Carpal tunnel syndrome also occurs in people who overuse their hand or perform repetitive actions like using a computer key board, a cashiers machine or a musical instrument. When the nerve is compressed, it can result in disabling symptoms like numbness, tingling, or pain in the middle three fingers. As the condition progresses, it can lead to muscle weakness and inability to hold objects. The pain frequently occurs at night and can even radiate to the shoulder. Even though the diagnosis is straightforward, the treatment is not satisfactory."}, {"id": "InternalMed_Harrison_1904", "title": "InternalMed_Harrison", "score": 0.010327570518653321, "content": "both hands and feet also occurs with lesions of the upper cervical cord or the brainstem, but an upper level of the sensory disturbance may then be found on the trunk and other evidence of a central lesion may be present, such as sphincter involvement or signs of an upper motor neuron lesion (Chap. 30). Although most polyneuropathies are pansensory and affect all modalities of sensation, selective sensory dysfunction according to nerve fiber size may occur. Small-fiber polyneuropa-161 thies are characterized by burning, painful dysesthesias with reduced pinprick and thermal sensation but with sparing of proprioception, motor function, and deep tendon reflexes. Touch is involved variably; when it is spared, the sensory pattern is referred to as exhibiting sensory dissociation. Sensory dissociation may occur also with spinal cord lesions as well as small-fiber neuropathies. Large-fiber polyneuropathies are characterized by vibration and position sense deficits, imbalance, absent tendon"}, {"id": "pubmed23n0348_21382", "title": "[Compressive ischemic neuropathies of the upper extremities and work].", "score": 0.00980392156862745, "content": "The objective of the presented study was to motile that cumulative trauma disorders of the upper extremities in recent years leads to arising morbidity and applications for compensation for occupational diseases. This rise is probably not associated with an increase of this disorder but a different awareness of workers and doctors as regards the possible adverse effect of forceful and frequently repeated movements of the upper extremities on their function, extended diagnostics and notification. It is important to master the diagnosis of these diseases and interpretation of auxiliary examinations to prevent inadequate payment of damages to the affected subjects and on the other hand to prevent breakdown or in adequate function of the financial provisions in this area. A number of syndromes of the mentioned conditions has very few objective symptoms and abnormal results of auxiliary examinations. The diagnosis of stenotic syndromes has a compared with other disorders great support in electrophysiological techniques, their high sensitivity and specificity (1, 15). The authors discuss therefore their problems and suggest electrophysiological criteria of medium grade affections in the carpal tunnel syndrome which is the condition for compensation for occupational disease and which so far was defined only roughly in this country."}, {"id": "wiki20220301en219_14750", "title": "Central nervous system disease", "score": 0.009708737864077669, "content": "Multiple sclerosis Multiple sclerosis (MS) is a chronic, inflammatory demyelinating disease, meaning that the myelin sheath of neurons is damaged. Symptoms of MS include visual and sensation problems, muscle weakness, numbness and tingling all over, muscle spasms, poor coordination, and depression. Also, patients with MS have reported extreme fatigue and dizziness, tremors, and bladder leakage. Myelopathy Myelopathy is an injury to the spinal cord due to severe compression that may result from trauma, congenital stenosis, degenerative disease or disc herniation. The spinal cord is a group of nerves housed inside the spine that runs almost its entire length. Tourette's"}, {"id": "pubmed23n0401_14141", "title": "[An autopsy case with subacute spinal cord disease showing progressive paraplegia].", "score": 0.009615384615384616, "content": "We report a 72-year-old woman who died of respitory failure. History included onset of diabetes mellitus at the age of 67 years and hypertension at the age of 72 years. The patient had been in good health otherwise until 2000, when she had onset of numbness or tingling of the bilateral lower limbs. On December 3, 2000, she was admitted to a hospital in the vicinity of her home because of the above-mentioned complaints. Neurological examinations revealed progressive paraplegia. Symptoms and signs suggested Guillain-Barré syndrome. Examinations of cerebrospinal fluids revealed cell count of 338/3 (mono 72%, poly 18%) and protein value of 100 mg/dl. Later the patient course deteriorated. On December 15, 2000, she was admitted to Hakujikai Memorial Hospital for the second time. Ten days later, MRI examination showed diffuse swelling of the spinal cord from the cervical (C 3/4) level to the thoracic level. Gd-enhanced T 1-weighted MRI performed 22 days later showed a partially enhanced lesion at the thoracic (Th 5/6) level of the spinal cord. The patient was treated with steroid therapy (methylprednisolone 500 mg/dl). She died of respiratory failure on January 6, 2001. The patient was presented in a neurological CPC. Neurological and imaging findings suggested a transverse myelopathy. However, there were several points in this case that were unusual for a typical transverse myelopathy, such as total sensory loss below spinal segments of thoracic level (Th 5) and motor weakness of the upper limbs of upper segment of the same level. A clinical neurologist concluded that the patient had subacute transverse myelopathy with fused multiple pathy pathologic lesions. We discussed whether this case was a transverse myelopathy or multiple sclerosis. Post mortem examination revealed acute necrotic myelopathy affecting the spinal cord from the second cervical to the tenth thoracic vertebrae, with conspicuous infiltration of CD 68-positive macrophages involving both gray and white matter, partially necrotic associated with scattered UCHL-1 dominants lymphocytic infiltration of T cells around vessels. There were relatively older lesions with demyelinating features in the spinal roots that were particularly dominant in the anterior roots. No demyelinated plaques in the optic chiasm, tracts and nerves, or in the cerebero-cerebellar white matter were found. Systemic pathological diagnosis was lung edema with fresh hemorrhage, pancreatic atrophy consistent with diabetes mellitus and choleductlithiasis."}, {"id": "wiki20220301en050_74511", "title": "Nerve conduction study", "score": 0.009615384615384616, "content": "Repetitive nerve stimulation Interpretation of nerve conductions The interpretation of nerve conduction studies is complex and requires the expertise of health care practitioners such as clinical neurophysiologists, medical neurologists, physical therapists, chiropractic neurologists or physiatrists. In general, different pathological processes result in changes in latencies, motor, and/or sensory amplitudes, or slowing of the conduction velocities to differing degrees. For example, slowing of the NCV usually indicates there is damage to the myelin. Another example, slowing across the wrist for the motor and sensory latencies of the median nerve indicates focal compression of the median nerve at the wrist, called carpal tunnel syndrome. On the other hand, slowing of all nerve conductions in more than one limb indicates generalized diseased nerves, or generalized peripheral neuropathy. People with diabetes mellitus often develop generalized peripheral neuropathy."}, {"id": "wiki20220301en060_57304", "title": "Neuropathic arthropathy", "score": 0.009523809523809525, "content": "Roughly 75% of patients experience pain, but it is less than what would be expected based on the severity of the clinical and radiographic findings. Pathogenesis Any condition resulting in decreased peripheral sensation, proprioception, and fine motor control: Diabetes mellitus neuropathy (the most common in the U.S. today, resulting in destruction of foot and ankle joints), with Charcot joints in 1/600-700 diabetics; related to long-term high blood glucose levels. Alcoholic neuropathy Cerebral palsy Leprosy Syphilis (tabes dorsalis), caused by the organism Treponema pallidum Spinal cord injury Myelomeningocele Syringomyelia Intra-articular steroid injections Congenital insensitivity to pain Peroneal muscular atrophy"}, {"id": "pubmed23n0307_14080", "title": "[Left hand clumsiness due to disturbance of kinesthesia after damage to the dorsal column of the high cervical cord].", "score": 0.009433962264150943, "content": "We described a 48-year-old, right-handed woman who manifested left hand clumsiness after damage to the dorsal column of the high cervical cord due to probable multiple sclerosis. On February 29, 1996, she developed a weakness in the right limbs. Subsequently, she suffered numbness and clumsiness in the left limbs, even though muscle strength of the left limbs was preserved. Seventeen days later, she was referred to our hospital. A T2-weighted MRI after admission demonstrated high signal intensities in the left dorsal column and the right antero-lateral part of the cervical cord at the C1 to C3 vertebral level. Under the diagnosis of probable multiple sclerosis, steroid pulse therapy was applied twice and she gradually regained muscle strength in the right limbs and sensation in the left limbs. One month later, elemental sensations such as pain, touch, temperature, vibration, and position, as well as discriminative sensations such as localization sensation, two-point discrimination, barognosis, pinch-press discrimination, and graphesthesia in the left limbs returned to normal. However, her left hand remained clumsy, especially when she tried to manipulate objects. She also showed a great difficulty in sustaining a constant level of pinching force by the left thumb and index finger, and in localizing her right thumb placed in space with the left hand with her eyes closed. She stated herself that she could not sense at all how her left hand and fingers were moving. Somatosensory evoked potentials recorded from the right scalp showed that the NI was poorly organized and the patency of subsequent peaks was delayed. Transcranial magnetic stimulation revealed that the pyramidal tract from the right motor cortex to the left cervical cord was functionally intact. These observations lead us to conclude as follows: (1) the patient's left hand clumsiness is probably due to the disturbance of kinesthesia, which is crucial to activate temporo-spatial patterns of complex hand and finger movements as well as to maintain long sequences of simple motor execution without vision; and (2) kinesthesia is a specific sensation that is presumably conveyed by the dorsal columns and could be selectively affected by a cervical cord lesion."}, {"id": "wiki20220301en510_541", "title": "Distal hereditary motor neuropathy type V", "score": 0.009259259259259259, "content": "Distal hereditary motor neuropathy type V is a particular type of neuropathic disorder. In general, distal hereditary motor neuropathies affect the axons of distal motor neurons and are characterized by progressive weakness and atrophy of muscles of the extremities. It is common for them to be called \"spinal forms of Charcot-Marie-Tooth disease (CMT)\", because the diseases are closely related in symptoms and genetic cause. The diagnostic difference in these diseases is the presence of sensory loss in the extremities. There are seven classifications of dHMNs, each defined by patterns of inheritance, age of onset, severity, and muscle groups involved. Type V (sometimes notated as Type 5) is a disorder characterized by autosomal dominance, weakness of the upper limbs that is progressive and symmetrical, and atrophy of the small muscles of the hands."}, {"id": "wiki20220301en013_91213", "title": "Multiple myeloma", "score": 0.009174311926605505, "content": "Neurological symptoms Some symptoms (e.g., weakness, confusion, and fatigue) may be due to anemia or hypercalcemia. Headache, visual changes, and retinopathy may be the result of hyperviscosity of the blood depending on the properties of the paraprotein. Finally, radicular pain, loss of bowel or bladder control (due to involvement of spinal cord leading to cord compression) or carpal tunnel syndrome, and other neuropathies (due to infiltration of peripheral nerves by amyloid) may occur. It may give rise to paraplegia in late-presenting cases. When the disease is well-controlled, neurological symptoms may result from current treatments, some of which may cause peripheral neuropathy, manifesting itself as numbness or pain in the hands, feet, and lower legs."}, {"id": "pubmed23n0688_8544", "title": "[Natural history of carpal tunnel syndrome--a review].", "score": 0.009174311926605505, "content": "The review of the literature on the natural history of the carpal tunnel syndrome is presented. It is shown that the condition is characterised by non-uniform and unpredictable clinical course, in which besides the progressive type of evolution, a regressive one (characterised by spontaneous resolution of symptoms) and type of stable clinical picture (with episodes of exacerbation and resolution of symptoms) exist. Proportion of prevalence of particular types of clinical courses is not precisely estimated, but it appears that at least a half of the cases is of non-progressive type. Non-operative treatment of the condition may be effective in those particular cases, without risk of the development of severe neurological complications as a consequence of impairment of the median nerve. The evidence form analysed studies shows that in carpal tunnel syndrome, the clinical symptoms and signs and nerve conduction disturbances have different natural histories. Clinical features are subjected to greater temporal fluctuations than electrophysiological findings, and they frequently do not correlate one with another. There is not common opinion about efficacy of intervention in extreme carpal tunnel syndrome, characterised by severe conduction disturbances in electrophysiological tests and fixed neurological deficits, however surgical decompression of the carpal tunnel appears to be more promising than decline of the treatment. The natural history of the syndrome occurred in the course of other diseases (or conditions, e.g. pregnancy) is different, depending on the type of the disease itself. In the commonest systemic diseases associated with carpal tunnel syndrome, such as diabetes, hypothyroidism and rheumatoid arthritis, there is not common opinion about their prognostic effect on the natural course of the syndrome."}, {"id": "pubmed23n0883_13259", "title": "Hirayama's disease: an Italian single center experience and review of the literature.", "score": 0.00909090909090909, "content": "Hirayama's disease (HD), is a benign, self-limited, motor neuron disease, characterized by asymmetric weakness and atrophy of one or both distal upper extremities. In the present study we report the clinical, electrophysiological and MRI features of a group of Italian patients, with review of the literature. Moreover we propose an optimized MRI protocol for patients with suspected or diagnosed HD in order to make an early diagnosis and a standardized follow up. Eight patients with clinical suspicion of Hirayama disease underwent evaluation between January 2007 and November 2013. All patients underwent standard nerve conduction studies (NCS), electromyography (EMG) and motor/sensory evoked potentials (MEP/SEP). Cervical spine MRI studies were conducted with a 1.5 Tesla MRI scanner in neutral and flexion position, including sagittal T1-weighted sequences and sagittal and axial T2-weighted sequences. The following diagnostic features were evaluated: abnormal cervical curvature, localized cervical cord atrophy in the lower tract (C4-C7), presence of cord flattening (CF), intramedullary signal hyperintensity on T2 weighted sequences, anterior shifting of the posterior wall of the cervical dural sac (ASD) and presence of flow voids (EFV) in the posterior epidural space during flexion. All patients complained of weakness in hand muscles as initial symptoms, associated with hand tremor in three of them and abnormal sweating of the hand palm in two of them. No sensory deficits and weakness at lower limbs were reported by any patients. Distal deep tendon reflexes at upper limbs were absent in all patients with the absence of the right tricipital reflex in one of them. Deep tendon reflexes at lower limbs were normal and no signs of pyramidal tract involvement were present. The clinical involvement at onset was unilateral in six patients (three left-sided and three right-sided) and bilateral asymmetric in two of them, with the right side more affected. With the progression of the disease all patients but one experienced weakness and wasting of hand muscles and forearm bilaterally, but still asymmetric. The duration of the progression phase of the disease ranged from eight months to three years. In all patients, NCS and EMG findings were consistent with a spinal metameric disorder involving the C7-T1 myotomes bilaterally; sensory conduction and electrophysiologic features at lower limbs were normal. MEP and SEP were normal and we did not observe the disappearance of the spinal potential during the neck flexion in any of the patients. MRI is the best diagnostic tool in the diagnosis of HD; it can confirm clinical diagnosis and exclude other conditions responsible for the neurological deficits leading to a correct patient management and therapy, limiting arm impairment. On MRI all patients had loss of the normal cervical lordosis (100%). Five patients had loss of attachment of posterior dural sac and anterior dural shift on flexion MRI with presence of flow voids from venous plexus congestion (62.5%); three patients had no anterior dislocation of the dural sac and no epidural vein congestion. Two patients showed localized cord atrophy, one at C5-C6 and the other at C6-C7 level (25%). Three patients had T2 intramedullary hyperintensities (37.5%) and cord flattening (CF) was present in 5 patients of 8 (62.5%). HD is a rare entity and a self-limited condition, but it has to be early differentiated from other diseases that may determine myelopathy and amyotrophy to establish a correct therapy and limit arm impairment. MRI is very important to confirm the clinical suspect of HD and a standardized MRI protocol using axial and sagittal images in both neutral and flexing position is needed, in order to diagnose and follow up affected patients."}, {"id": "pubmed23n0918_10443", "title": "Characteristics of nerve conduction studies in carpal tunnel syndrome.", "score": 0.00909090909090909, "content": "Numerous nerve conduction tests are used for the electrodiagnosis of carpal tunnel syndrome (CTS), with a wide range of sensitivity and specificity reported for each test in clinical studies. The purpose of this study was to compare the diagnostic accuracy of various nerve conduction tests and determine the properties of the most accurate test. A prospective observational case control study. Eighty patients with clinically confirmed CTS and 80 asymptomatic healthy controls were included in the study. All patients underwent the routine hematological investigations as per the protocol. All cases and controls were subjected to various nerve conduction study protocols for CTS. Results were analyzed statistically. The two-tailed Student's t-test was used for the comparative statistical analysis. The sensitivity of each test was calculated as (the number of hands with an abnormal study result/the number of CTS hands) × 100. Comparison between percentages was performed by the McNemar test. The mean age was 38.19 ± 10.13 years and the female:male ratio was 1.5:1. The mean duration of disease was 0.89 ± 0.61 years. Hypothyroidism was present in 21 (26.25%) patients, whereas 13 (16.25%) and 4 (5%) patients had diabetes mellitus and rheumatoid arthritis, respectively. The median nerve motor latency was 4.73 ± 0.83 ms while the sensory latency was 3.44 ± 0.56 ms. The median nerve orthodomic sensory latency was found to be 2.57 ± 0.31 ms. The conduction velocity across the palm and wrist was 41.37 ± 0.67 ms. The sensitivity was the highest in the inching method (86.25%) and lowest for the conventional median motor and sensory latencies (56.25% and 45%, respectively). Addition of a single test of median and ulnar sensory latency, the median and radial sensory latency or the inching method, in routine protocol will improve the sensitivity for the diagnosis of CTS in all patients."}, {"id": "pubmed23n0294_20891", "title": "[A family of hereditary motor and sensory neuropathy type I with a mutation (Arg98--&gt;His) in myelin Po--report on a second Japanese family].", "score": 0.009009009009009009, "content": "A 46-year-old housewife had complaints of insidiously progressive muscle weakness and paresthesia in the distal lower limbs. On neurological examination, a slight to moderate degree of muscle weakness with slight atrophy was observed in the bilateral intrinsic hand muscles. A severe degree of muscle weakness with moderate atrophy was observed in tibialis anterior, gastrocnemius and soleus muscles. Muscle stretch reflexes were decreased in the upper limbs and absent in the lower limbs, without pathologic reflexes. She had a steppage gait. Vibratory sensation was slightly decreased in the hands and moderately decreased in the feet. Touch, pain and temperature sensations were also moderately decreased only in the feet. On laboratory examination, glycosuria (5.6g/dl) was noted. Fasting blood sugar was 226mg/dl with an elevated hemoglobin A1C level (12.7%). The right median motor and sensory nerve conduction velocities were 14.8 and 20.3 m/sec, respectively, with a markedly prolonged distal latency. No muscle action potential was obtained from stimulation of the right tibial nerve. Also, no nerve action potential was elicited from stimulation of the right sural nerve. A fascicular biopsy of the right sural nerve revealed the presence of both demyelinated and remyelinated axons, and an onion-bulb formation with a marked decrease in the density of the myelinated fibers. Based on the neurological examination and nerve conduction studies of the family members, a younger sister, younger brother and an elder daughter of the proband were found to be affected by demyelinating polyneuropathy. Diabetes mellitus was not found among the family members with laboratory evidences of demyelinating polyneuropathy. Based on the family history, an uncle on the mother's side of the proband, the proband's grandmother and a younger daughter of a proband's brother were considered to be affected. The uncle and grandmother had diabetes mellitus. Therefore, we concluded that this family had HMSN type I with autosomal dominant inheritance. In the studies on fluorescence in situ hybridization, and restriction fragment length polymorphism of the genomic DNA of the proband, a DNA duplication in the 17p11.2-12 region was not observed. However, the direct sequencing analysis of DNA fragments from genomic DNA encoding the Po gene of the proband revealed a substitution of histidine for arginine at the codon 98 in the extramembranous domain of Po. She was heterozygous for the mutant allele and normal allele. Alterations in the tertiary structure of the extramembranous domain of Po may result in an impairment of the peripheral myelin compaction. This is the second Japanese family with the same mutation (Arg98--&gt;His) of myelin Po as reported previously by us, and this type of case is rare in the literature. Therefore, the mutation at the codon 98 may play a critical role in the development of the myelin abnormality in HMSN type IB."}, {"id": "wiki20220301en033_62876", "title": "Myelopathy", "score": 0.008928571428571428, "content": "Presentation Clinical signs and symptoms depend on which spinal cord level (cervical, thoracic, or lumbar) is affected and the extent (anterior, posterior, or lateral) of the pathology, and may include: Upper motor neuron signs—weakness, spasticity, clumsiness, altered tonus, hyperreflexia and pathological reflexes, including Hoffmann's sign and inverted plantar reflex (positive Babinski sign) Lower motor neuron signs—weakness, clumsiness in the muscle group innervated at the level of spinal cord compromise, muscle atrophy, hyporeflexia, muscle hypotonicity or flaccidity, fasciculations Sensory deficits Bowel/bladder symptoms and sexual dysfunction Diagnosis"}, {"id": "article-25766_15", "title": "Neuroanatomy, Neurapraxia -- Clinical Significance", "score": 0.008854223482782444, "content": "In these cases, a good knowledge of anatomy is beneficial. [4] The history should be thorough and the neurological examination, complete and meticulous, to obtain an accurate diagnosis and administer the appropriate management. Test for motor function and deranged sensation should include and follow specific nerve innervations. Depending on the nerve involved, neurologic findings include sensory impairment such as pain, allodynia, hyperesthesia, hypoesthesia, paresthesia, motor impairment such as weakness, atrophy in chronic cases, hyporeflexia. [4] Results of the neurologic examination serve to identify the type of injury, the location of the lesion, and the degree of injury of the sensory, motor, or both sensory-motor impairment, which is of prognostic value. [17] [18] [4] [22] Differential diagnosis includes peripheral neuropathies due to diabetes, hypothyroidism, alcohol, and malnutrition, radiculopathy, myelopathy, spinal cord trauma or infarction, muscle diseases. [18]"}, {"id": "wiki20220301en131_45670", "title": "Neurogenic bladder dysfunction", "score": 0.008849557522123894, "content": "Central nervous system Damage to the brain or spinal cord is the most common cause of neurogenic bladder. Damage to the brain can be caused by stroke, brain tumors, multiple sclerosis, Parkinson's disease or other neurodegenerative conditions. Bladder involvement is more likely if the damage is in the area of the pons. Damage to the spinal cord can be caused by traumatic injury, demyelinating disease, syringomyelia, cauda equina syndrome, or spina bifida. Spinal cord compression from herniated disks, tumor, or spinal stenosis can also result in neurogenic bladder. Peripheral nervous system Damage to the nerves that travel from the spinal cord to the bladder (peripheral nerves) can cause neurogenic bladder, usually the flaccid type. Nerve damage can be caused by diabetes, alcoholism, and vitamin B12 deficiency. Peripheral nerves can also be damaged as a complication of major surgery of the pelvis, such as for removal of tumors. Diagnosis"}, {"id": "pubmed23n0389_20579", "title": "[Regarding the clinical diagnosis of the  monotopical  spinal forms of multiple sclerosis. The value of the fan sign in the adult].", "score": 0.008849557522123894, "content": "We wish to discuss the value of the clinical history and examination in orientation of the diagnosis of probable multiple sclerosis (MS). We report the two year study of a woman who over the previous ten years had had three episodes of paraesthesia, with pins and needles in her left leg and other parts of the left side of her body, although never affecting head or neck. She also complained of tiring more than usual. In an outpatient clinic she was found to have a syndrome affecting the upper segments of the spinal cord, mainly involving the right side and resembling an incomplete Brown Sequard type syndrome. There were increased clinical muscle and deep reflexes. The most marked was that of the right deltoid (C5), bilateral fanning of the toes when the Babinski reflex was tested, Barré positive in the right leg, pins and needles and dysaesthesia on the left to an undetermined level. Function was well preserved when compared with the clinical signs found. The case was considered to be of monotopical MS. Spinal magnetic resonance findings confirmed the clinical diagnosis. We emphasise the value of careful clinical investigation directed towards the diagnosis of probable MS. We draw attention to the diagnostic value of the dissociation between the severe clinical alterations and the functional performance, which was surprisingly well maintained. Also we report the originality of the presence of bilateral fanning sign supporting the diagnosis of MS, occurring in a disease of adult life."}, {"id": "pubmed23n0967_22165", "title": "[Late diagnosed cervical myelomalesia in a case of Fahr disease experiencing a neuropathic pain].", "score": 0.008771929824561403, "content": "Fahr disease is an idiopathic disorder characterized with deposition of calcium and a few other minerals in basal ganglia, cerebellum and subcortical brain area. A 51 years old female with the complaints of pain, numbness, tingling and weakness in both upper extremities for six months was referred to our electromyography laboratory with a suspicion of carpal tunnel syndrome. She got the diagnosis of Fahr disease upon the investigations for the convulsions that she experienced ten years ago. Beside, she had a generalized anxiety disoder. Neurological examination revealed mild to moderate weakness in flexion and extension of forearm, and extension of hand on both sides. She described dysesthesia on C6 &amp; C7 dermatomes, bilaterally. Symmetric calsifications on both cerebellar hemispheres and basala ganglia were present on cranial CT. Median and ulnar nerve conduction studies were normal on both sides. Concentric needle electromyography revealed chronic neurogenic changes on the morphology of motor unit potentials recorded from the muscles of C6 &amp; C7, bilaterally. Cervical magnetic resonance imaging revealed discopathies on C4-5, C5-6 and C6-7 levels causing myelomalacia. Neuropathic pain, paresthesia or muscle weakness on upper extremities are rare in Fahr disease. Presented case got the diagnosis of cervical discopathies in late as those findings were supposed to be related with Fahr disease. Therefore, clinicians should be aware of common findings occured during the course of this disease, and consider the possible coincidental pathologies when the atypical neurological deficits are observed in these patients."}, {"id": "pubmed23n0393_11438", "title": "Neurological diagnosis: aspects of bedside and electrodiagnostic examinations in relation to hand-arm vibration syndrome.", "score": 0.008771929824561403, "content": "The objective of this paper, was to direct attention to the diagnostic strategy and clinical approach necessary in the diagnosis of neuropathy in workers exposed to vibration. The purpose encompassed evaluation of selected aspects of bedside and electrodiagnostic examinations with respect to biological validity and the ability to distinguish between subjects with and without neuropathy. The neurological examinations viewed were restricted to those applicable to the upper extremity and neck system. A MEDLINE search was performed through the clinical queries service of PubMed searching for the following terms: nerve-conduction, Tinel's test, Phalen's test, tendon reflex, two-point discrimination test, abduction external rotation test, and Spurling test. Retrieved articles were discussed both in relation to the test accuracy and the validity aspects of the tests. The evidence in support of the view that neurological tests can accurately distinguish between subjects with and without neuropathy specifically addressing hand-arm vibration syndrome was sparse. The initial number of diagnostic hypotheses could be reduced by progressively ruling out diseases based on negative results of highly sensitive tests. As the possible diagnostic alternatives become fewer, the use of positive results from highly specific tests are more effective. The information value of the various diagnostic tests is determined by the change in pre-test to post-test probability of target disorder, which depends on the prevalence of the disorder and the likelihood ratios of the tests. The review showed that target disease characteristics influence the test outcome as well as the choice of \"gold standard\" and the population domain of the studies. The selection of various bedside examinations and diagnostic electrophysiological tests should be dependent on the clinical context, the history and results from the successive diagnostic tests."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 93]], "word_ranges": [[0, 15]], "text": "It is SLE with joint, skin and serositis involvement. Treatment is EC at moderate-high doses."}, "2": {"exist": true, "char_ranges": [[94, 210]], "word_ranges": [[15, 34]], "text": "Mycophenolate is never a treatment in acute phase but in maintenance, nor has it proved useful in joint involvement."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "It is SLE with joint, skin and serositis involvement. Treatment is EC at moderate-high doses. Mycophenolate is never a treatment in acute phase but in maintenance, nor has it proved useful in joint involvement.", "full_answer_no_ref": "It is SLE with joint, skin and serositis involvement. Treatment is EC at moderate-high doses. Mycophenolate is never a treatment in acute phase but in maintenance, nor has it [HIDDEN].", "full_question": "A 37-year-old man presents with arthritis of the metacarpophalangeal joints of both hands and right pleuritis. Bilateral malar erythema is seen on examination. Positive antinuclear antibodies were detected (titer 1/640), with anti native DNA antibodies also positive; anti-Sm negative What would be the initial treatment of choice for this patient?", "id": 316, "lang": "en", "options": {"1": "Glucocorticoids at high doses.", "2": "Glucocorticoids and mycophenolate.", "3": "Nonsteroidal anti-inflammatory drugs and antimalarials.", "4": "The picture will probably be self-limited and does not require treatment.", "5": NaN}, "question_id_specific": 138, "type": "RHEUMATOLOGY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0422_20674", "title": "[Two cases of rheumatoid arthritis developed after polymyositis].", "score": 0.01677018633540373, "content": "We report two cases of rheumatoid arthritis (RA) who later had developed after polymyositis (PM). The first patient was 64-year old male who experienced muscular weakness of the four limbs in proximity 10 years ago. He was diagnosed as PM because of the elevated serum CK and the myogenic pattern of EMG, and his symptoms were improved by treatment with corticosteroid. He started to complain polyarthralgia 2 years ago, followed by interstitial pneumonia, pleuritis and skin ulcer. He was admitted because of exacerbated polyarthralgia, multiple subcutaneous nodules, skin eruption and fever. The level of serum CK was within normal range but CRP was elevated and CH 50 was decreased. The laboratory examination showed positive cryoglobulin and high titer of rheumatoid factor, but anti-Jo 1 antibody was negative. The hand X-ray showed bone erosions in bilateral wrist joints. Skin biopsy revealed leukocytoclastic vasculitis. Based on these findings, he was diagnosed as malignant RA. He was successfully treated with methylprednisolone pulse therapy, cyclophosphamide and prostaglandin E 1. The second patient was 77-year old male with pneumoconiosis who experienced muscular weakness of the four limbs in proximity 4 years ago. He was diagnosed as PM based on his clinical and laboratory findings and was treated with temporary corticosteroid. He started to have polyarthralgia last year, and he was admitted because of increasing arthralgia after the treatment of pulmonary tuberculosis. The level of serum CK was slightly elevated due to hypothyroidism, and CRP was highly elevated. Rheumatoid factor and cryoglobulin were positive, but anti-Jo 1 antibody was negative. The hand X-ray showed bone erosions in bilateral wrist joints. Crystals of pyrophosphate calcium was observed in knee joints. He was diagnosed as RA associate with pseudogout. His symptoms were relieved with corticosteroid, salazosulfapyridine and anti-tuberculous therapy. These two cases had altered their clinical features from PM to definite RA, and both had pulmonary complications. Previous reports described the cases of RA followed by PM, most of which were induced by such drugs as D-penicillamine, but the cases of PM who later had developed RA are extremely unusual. The overlapped cases of RA and PM tend to highly associate with pulmonary lesions."}, {"id": "pubmed23n1004_25990", "title": "Rowell Syndrome in Nigeria: Systemic Lupus Erythematosus Presenting as Recurrent Erythema Multiforme in a Young Woman.", "score": 0.01642643812491884, "content": "Dear Editor, Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by diverse patterns of auto-antibody production with multi-organ affectation. Cutaneous involvement, either alone or in association with other systemic illnesses, is one of its most common manifestations (1). Dermatologic disorders like malar and discoid rashes are quite suggestive of SLE. However, the occurrence of non-specific skin lesions like erythema multiforme (EM) in patients with SLE (Rowell syndrome) can rarely occur (1). In such patients, a diagnosis of SLE may be missed or delayed in the absence of other overt clinical features of lupus. Herein we report a case of recurring EM-like eruptions as the cardinal cutaneous manifestation of previously undiagnosed, active SLE in a young Nigerian woman. A 26-year-old Nigerian woman presented with a three-day history of non-pruritic, generalized, and target-like, erythematous annular patches and plaques which mostly affected the trunk. A few lesions had presented with crusting and erosions at the time of examination (Figure 1). Associated symptoms included oral painful ulcers, low grade fever, and malaise. The patient had no other systemic symptoms and her prior drug history was not remarkable. Her erythrocyte sedimentation rate (ESR) was 66 mm/hour using the Westergren method. Screening for HIV and hepatitis B and C was negative. Herpes simplex, cytomegalovirus, and Epstein Barr viruses could not be screened for. Other baseline investigations (complete blood count, electrolytes, urea and creatinine as well as urinalysis) were within normal limits. The patient was managed as a case of EM of an unidentified inciting agent and her symptoms resolved with supportive care and antibiotics. However, she developed a recurrence about 5 weeks later, with more extensive and coalescent skin lesions (Figure 2). Additionally, there was a new onset of alopecia and pain in the small joints of the hands as well as both knees and ankles. At this time, the patient's ESR had gone up to 112 mm/h and she had developed significant proteinuria, with a protein creatinine ratio of 1.3 g/g (reference &lt;0.5 g/g). Her antinuclear antibody (ANA) titer was high (1:320) with a speckled pattern. Anti-Smith antibody was also positive. A renal biopsy was declined. A tentative diagnosis of Rowell syndrome was made. The patient was started on high-dose steroids and hydroxychloroquine 200 mg twice daily. Subsequent care included the use of mycophenolate mofetil 1 g twice daily for 6 months. This was then changed to azathioprine at 50 mg twice daily. Follow-up after 6 months showed sustained clearance of skin lesions, resolution of fever and joint pains, as well as improvement in the renal profile, with a urine protein-creatinine ratio of 0.77 g/g. The presence of systemic lupus erythematosus, EM-like lesions, and a speckled pattern of antinuclear antibody in our patient fulfils the revised diagnostic criteria for RS put forward by Zeitouni et al. at the turn of the twenty-first century (2). Considering the rarity of EM-like lesions in SLE and the possibility of constitutional symptoms in EM, a diagnosis of RS may be readily overlooked in patients like the one described, whose major cutaneous manifestation of severe active SLE was EM-like lesions. In contrast to classic EM, where skin lesions are concentrated in the extremities, a predominant truncal distribution of EM-like lesions as found in our patient may favor a clinical consideration of RS (3). However, some authors have challenged the existence of Rowell syndrome as a distinct clinical laboratory entity. Arguments put forward in this regard include the fact that none of the immunological markers that have been described in RS are specific to any disorder. Additionally, the annular polycyclic dermatosis seen in sub-acute cutaneous lupus erythematosus (SCLE) can be difficult to clinically and histologically differentiate from EM (4,5). Patients with SLE also have a higher likelihood of developing adverse drug reactions (6). The inherent complexity of SLE may make for delayed and oftentimes difficult diagnosis, especially in a country where immunologic tests are expensive and rheumatologists are scarce. When patients do occasionally present with recurrences of skin lesions in the spectrum of EM, Steven-Johnson syndrome, and toxic epidermal necrolysis in the absence of a definite inciting agent, undiagnosed lupus may indeed be present in some of these individuals and should be considered in the differential diagnosis. In conclusion, while it is very rare, SLE may present first with recurrent episodes of EM-like rash. Despite the various possibilities which underlie their association, prompt identification and treatment of SLE in patients presenting with EM is important to prevent death or irreversible organ damage."}, {"id": "pubmed23n1115_3216", "title": "[Multicentric reticulohistiocytosis: A case report].", "score": 0.015763076188201577, "content": "A 65-year-old woman developed erythema, papules and nodules over the body. Some nodules of her auricles and hands like string beads. Besides, she suffered from symmetrical swelling and pain of multiple joints, morning stiffness with deformity of joints; She had elevated erythrocyte sedimentation rate and C reactive protein levels; Her rheumatoid factor and antinuclear antibody were positive; Joints destruction was found with X-ray imaging; Skin pathology showed Dermal infiltrate of abundant histiocytes, part of them with a ground-glass appearance; A CD68 immunohistochemical stain was positive and the cells were negative for S100, CD1a. These findings were diagnostic evidences of multicentric reticulohistiocytosis (MRH). The patient received high-dose of glucocorticoids combinated with immunosuppressive agents, and achieved a satisfactory effect. MRH was a rare multisystem disease characterized by papulonodular mucocutaneous and destructive arthritis, and its pathogeny was not yet completely understood. The typical lesions of MRH were hard papules or nodules that usually occured on the hands, face and arms. Classic coral bead appearance from periungual cutaneous nodules that were characteristic of MRH. MRH was an inflammatory joint disease, affecting almost all the appendicular joints and characterized by joint multiple, symmetrical, destructive, progressive disability. Joints destruction of the distal interphalangeal joints was a unique feature of MRH. In addition to skin and joints, it could also involve other systems. There were no diagnostic laboratory markers for MRH. Laboratory examinations had often been found to be non-specific. Imageological examination mainly showed bone and joint destruction. Skin biopsy was the best test to diagnose MRH, the typical histopathological findings included an infiltrate with histiocytes and multinucleated giant cells with a ground-glass appearing in eosinophilic cytoplasm, and the immunohistochemical stain was positive for CD68. The diagnosis was typically made based on the clinical presentation, supportive radiographic findings and skin biopsy. MRH was easily possible to mistake for other more common autoimmune conditions, such as rheumatoid arthritis, psoriatic arthritis, osteoarthritis, and dermatomyositis, but the distinctive clinical, radiographic, and histologic features could aid in differentiating these diseases. MRH could mimic other rheumatic diseases, besides, it could also coexist with cancer or other autoimmune disorders. There was no standardized treatment for MRH. However, Nonsteroidal anti-inflammatory drugs, glucocorticoid, Immunosuppressant, biologic medications, and bisphosphonates had been used with varying degrees of curative effect. Treatment with glucocorticoid combined with immunosuppressants were effective for rash and arthritis, early use of them should be strongly considered, and refractory cases could be treated with biological agents. By reporting a MRH case and reviewing literature, this paper aims to help the clinicians improve the understanding of this rare disease, and suggests that when one diagnosis cannot explain the whole picture of the disease, and further evidence should be sought to confirm the diagnosis."}, {"id": "pubmed23n1151_11646", "title": "[Lack of therapeutic response: Is it really a rheumatoid Arthritis?]", "score": 0.014662894860914663, "content": "A 78-year-old female patient was referred to our hospital with treatment-resistant seronegative anti-citrullinated protein antibodies (ACPA)-negative rheumatoid arthritis. The course was characterized by high inflammatory activity and rapid progression of the erosive changes. Under the required high-dose prednisolone therapy, osteoporosis and a deep venous thrombosis (DVT) with pulmonary embolism developed. A physical examination revealed symmetrical, painful, synovial swelling of the metacarpophalangeal, proximal-interphalangeal and distal-interphalangeal (MCP, PIP, and DIP) joints, finger joint-related violaceous erythema, contractures of the long fingers, and advanced deformities bilaterally. Pain and weakness in the muscles of her proximal extremities led to difficulties in raising her arms and climbing stairs. The results of laboratory tests showed negative RF (rheumatoid factor) and ACPAs, positive ANA (anti-nuclear antibodies) with titer 1:5120, a nuclear fluorescence pattern and a positive anti-Mi-2 Antibodies in the myositis blot. Conventional x-ray showed erosive and advanced mutilating joint changes in both hands. A magnetic resonance imaging (MRI) of the proximal extremities showed a pronounced muscle atrophy without sights of active myositis.This clinical constellation leads to the diagnosis of an amyopathic dermatomyositis. The patient was started on intravenous prednisone 100 mg daily, 4 days, followed by rapid dose tapering in the setting of accompanying risk factors such as Osteoporosis, arterial Hypertension and blurred vision. This treatment leads to improvement of symptoms. The basic therapy was switched to Rituximab. An extended tumor search was recommended on an outpatient basis. A seronegative, ACPA negative, therapy-resistant RA with a rapidly progressive erosive course requires a diagnostic re-evaluation. An erosive, rapidly progressing polyarthritis is commonly seen as manifestation of the subtype inflammatory myopathies associated with anti-Jo 1 antibodies, known as anti-synthetase syndrome. However, associations with the presence of other myositis specific antibodies (MSA) have been also described. The anti-Mi-2 Antibodies are highly specific for dermatomyositis (DM).Amyopathic DM is not common, but the disease course and prognosis do not differ significantly from myopathic DM.As a sudden presentation of DM may be of paraneoplastic origin a further examination in order to exclude malignancy are indicated."}, {"id": "InternalMed_Harrison_25064", "title": "InternalMed_Harrison", "score": 0.013116424182611188, "content": "Diagnosis: Symptom complex suggestive of SLE Order laboratory tests: ANA, CBC, platelets, urinalysis All tests normal symptoms subside All tests normal symptoms persist ANA positive Not SLE Not SLE Treatment Repeat ANA, add anti-dsDNA, anti-Ro All negative Some positive Definite SLE (˜4 criteria, Table 378-3) Possible SLE (<4 criteria, Table 378-3) Not lifeor organ-threatening Lifeor organ-threatening Quality of life: Acceptable Quality of life: Not acceptable High-dose glucocorticoids, usually with addition of second agent Conservative manage-ment (Table 378-5) Conservative treatment plus low-dose glucocorticoids Consider belimumab Mycophenolate mofetil (or myfortic acid) Cyclophosphamide Low or high dose Do not exceed 6 months of Rx After response, d/c cyclophosphamide; maintain with mycophenolate or azathioprine No response Response Taper dose of all agents, especially glucocorticoids Belimumab, Rituximab, calcineurin inhibitors, or experimental therapies"}, {"id": "pubmed23n0998_19836", "title": "Rowell's Syndrome Triggered by Omeprazole.", "score": 0.011834733893557423, "content": "Dear Editor, Rowell's syndrome is a rare disease, characterized by the appearance of erythema multiforme (EM)-like lesions in patients with lupus erythematosus. It was initially reported by Rowell (1) in 1963 and its existence as a separate clinical entity is currently under debate (2,3). A few cases may have been induced by drugs such as systemic antimycotics, antibiotics, anticonvulsants, and more recently proton pump inhibitors (PPIs). CASE REPORT We present the case of a 67-year-old woman with subacute cutaneous lupus erythematosus (SCLE) and EM-like lesions who fulfilled all the criteria for Rowell's syndrome. The patient had lupus arthritis for two years and was treated with oral methylprednisolone 8 mg/day and hydroxychloroquine 200 mg/day. She started receiving 20 mg of omeprazole daily for gastroprotection. The patient also had arterial hypertension with no current treatment, osteoporosis, and an L1 vertebral fracture. The dermatological examination revealed multiple erythematous infiltrated plaques involving mainly the sun-exposed areas (neck, chest, upper back, and shoulders). Cutaneous lesions had an annular or target pattern and a tendency to form hemorrhagic crusts and scales at the margins (Figure 1, A). The mucous membranes were unaffected. Histological examination (hematoxylin and eosin ×200) found epidermal atrophy, vacuolar degeneration of the basal layer, and sparse perivascular lymphocytic infiltrate in the dermis - features corresponding to lupus erythematosus (Figure 2, A). Single eosinophilic necrotic keratinocytes characteristic for erythema multiforme were observed in the epidermis (Figure 2, B). Direct immunofluorescence (IF) from lesional skin showed granular deposits of C3 on the dermo-epidermal junction. Lupus band test from sun-protected, nonlesional skin was negative. On indirect IF a speckled pattern antinuclear antibodies (ANA) with &gt;1:1280 titers were detected. Anti-Ro (&gt;200 U/mL) and anti-La (&gt;200 U/mL) antibodies were also positive. The blood cell count and differential analysis were within reference ranges. The 24-hour urine protein test showed a non-significant proteinuria - 0.36 g/24h. Photo-testing was impossible considering the extent of the skin lesions. The therapeutic approach consisted of increasing the hydroxychloroquine dose to 400 mg/day, substituting PPI with famotidine 20 mg/day p.o. and ceftriaxone 2 g/day for the superinfection with Ps. aeruginosa, which led to a clinical improvement (Figure 1, B). The methylprednisolone dose remained unchanged due to already existing severe adverse effects. DISCUSSION The diagnosis was based on Zeitouni et al.'s classification (4). The three main criteria are as follows: lupus erythematosus, EM-like lesions, and speckled pattern of ANA. Our patient met all three major and one minor criteria, namely the presence of anti-Ro and anti-La antibodies. As for the other minor criteria, RF was negative and no chilblains were found. Although there was a continuous time lapse (more than 1 year) between the initiation of omeprazole intake and the diagnosis of Rowell's syndrome, we suggest that the connection is probable. For instance, the latency differs depending on the incriminated medication in drug induced SCLE. Longer periods are reported for diuretics and calcium blockers, while the time interval is shorter for chemotherapeutic drugs and antimycotics (5). Our suspicions were further confirmed by the fact that the lesions improved promptly within a month after discontinuation of omeprazole and doubling the dose of hydroxychloroquine. PPIs are reported to be a major cause of drug-induced SCLE (6,7). According to Laurinaviciene et al., the most common drugs involved are PPIs, thiazide diuretics, antifungals, chemotherapeutics, statins, and antiepileptics (6). However, very few cases of Rowell's syndrome are found to be drug-related. The culprit drugs include: oral terbinafine (8,9), norfloxacin (10), sodium valproate (11) and esomeprazole (12) (Table 1). CONCLUSION Despite the common clinical and immunological features shared between SCLE, drug-induced SCLE and EM, Rowell's syndrome seems to be a separate entity rather than a coincidental association. Finally, according to our knowledge this case would be the second of Rowell's syndrome due to PPIs."}, {"id": "pubmed23n1014_1022", "title": "A Rare Case of Adult-onset Still's Disease with Anti-Ro Antibody Positive.", "score": 0.011121856866537718, "content": "Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder. The exact pathogenesis is unknown but believed to have multiple etiologies. The Yamaguchi criteria are used to aid in the diagnosis of AOSD. Typical characteristics are spiking fevers, febrile rash, arthritis, and the absence of other serologic markers of rheumatic diseases. We present a case of a 31-year-old Hispanic female who presented with fevers, arthritis, febrile rash, high ferritin levels, and cervical and axillary lymphadenopathies. The unique feature of our case is that the patient was positive for antinuclear antibody (ANA) titers of greater than 1:640 and anti-Ro antibody. She responded with the pulse dose steroids and later prescribed methotrexate and tapered off prednisone with improvement in her symptoms."}, {"id": "pubmed23n1066_5169", "title": "Eosinophilic Fasciitis - Clinical Features and Therapeutic Management.", "score": 0.010942285391454313, "content": "Eosinophilic fasciitis is a rare disease from the group of scleroderma-like connective tissue diseases with unclear etiopathogenesis. It may be occasionally accompanied with other eosinophilic or autoimmune dysfunctions (1,2). Lack of international diagnostic criteria and treatment consensus may lead to diagnostic and therapeutic difficulties. The 61-year-old man with no significant personal or family pathological history was admitted to the Dermatology Department presenting persistent induration for several months as well as erythema and pain of the shins that gradually extended to thighs and forearms, with limited mobility of peripheral joints. (Figure 1) Additional tests showed leukocytosis with 16% eosinophilia, elevated CRP, and hypergammaglobulinemia. Borrelia burgdorferi antibodies (classes IgM and IgG) were negative twice. A biopsy that included deep fascia was taken for histopathological examination. Antinuclear antibody screening was negative, but the direct immunofluorescence showed complexes in the dermo-epidermal junction and around the vessels. The diagnostics conducted toward malignant process showed no disturbing abnormalities (i.e. tumor markers in serum, chest, and abdomen computed tomography imaging, panendoscopy). The treatment was carried out with cephalosporin and nonsteroidal anti-inflammatory drugs (NSAIDs). The condition did not improve much but was stable. Histopathological results were indicative of eosinophilic fasciitis with fibrous thickening of deep fascia and perivascular infiltrations of plasma cells and lymphocytes; oral prednisone was initiated and the condition begin to improve. After 12 weeks, we observed disease progression with fever and very hard and cyanic skin lesions, which presented as an orange peel with linear furrows over the superficial venous vessels (Figure 2). The lesions extended to the trunk and caused troubles in moving. A complex rehabilitative intervention was started to minimize the inflammatory fascial restrictions. The prednisolone dose was increased, and oral methotrexate was added. After two weeks, the patient suffered from abdominal pain and periodic bleeding diarrhea. Methotrexate was suspected of inducing gastrointestinal adverse effects, and antipyretic NSAIDs were completely withdrawn. Colonoscopy showed features of mucosal edema with erythema, and histopathological examination revealed eosinophilic colitis. The patient was referred to a gastroenterologist, and methotrexate was ceased and switched to azathioprine. In summary, the consensus therapy of the rheumatologist, dermatologist, and gastroenterologist consisted of prednisolone and azathioprine. As of this writing, the patient's condition is gradually improving. The most characteristic symptoms of eosinophilic fasciitis is sudden onset with induration, sclerosis, and pain of the skin, with subcutaneous tissue and fascia usually appearing on the upper and lower limbs (3,4). The skin surface forms a characteristic orange peel appearance. The \"groove\" sign refers to the linear furrows over the superficial vessels of the extremities (1). Typical abnormalities are eosinophilia, elevated CRP, and hypergammaglobulinemia. The presence of eosinophilia is the most characteristic feature, occurring in 60-93% cases, but it is not necessary for diagnosis (1,5). Antinuclear antibodies are commonly absent with positive lesional direct immunofluorescence (6). If antinuclear antibodies are positive, it is recommended to broaden the diagnostic process to include other connective tissue diseases. Eosinophilia must be differentiated from hematological disorders and paraneoplastic syndrome. (4,6). Eosinophilic colitis is an eosinophilic gastrointestinal disease (EGID). It is the least frequent manifestation of EGID. It may be associated with connective tissue diseases, mostly systemic sclerosis - to our knowledge, there is no information in the literature about coexisting eosinophilic fasciitis. (7,8). The case described herein demonstrated that such a connection may occur. In treatment, it is important to prevent the patient from contractures and to maintain joint mobility by appropriate physiotherapy (2,9). The fascia forms a functional integral and continuous structure. Inflammation of one part of it changes the elasticity of the whole and produces fascial restrictions with movement limitation and pain. The fascia is profusely innervated, which favors constriction as a result of inflammation, and is also poorly vascularized which disrupts its regeneration (9,10). Myofascial techniques improve fascia elasticity by breaking up the tissue adhesions caused by inflammation (11). Eosinophilic fasciitis is a rare clinical entity, but knowing the possible clinical symptoms and laboratory abnormalities should help in taking the appropriate diagnostic path. It is important to treat the patient with attention to all concomitant diseases in consultation with different specialists."}, {"id": "InternalMed_Harrison_25234", "title": "InternalMed_Harrison", "score": 0.010611237661351556, "content": "Several developments during the past two decades have changed the therapeutic landscape in RA. They include (1) the emergence of methotrexate as the disease-modifying antirheumatic drug (DMARD) of first choice for the treatment of early RA; (2) the development of novel highly efficacious biologicals that can be used alone or in combination with methotrexate; and (3) the proven superiority of combination DMARD regimens over methotrexate alone. The medications used for the treatment of RA may be divided into broad categories: nonsteroidal anti-inflammatory drugs (NSAIDs); glucocorticoids, such as prednisone and methylprednisolone; conventional DMARDs; and biologic DMARDs (Table 380-2). Although disease for some patients with RA is managed adequately with a single DMARD, such as methotrexate, the situation in most cases demands the use of a combination DMARD regimen that may vary in its components over the treatment course depending on fluctuations in disease activity and emergence of"}, {"id": "wiki20220301en111_27506", "title": "Pemphigoid", "score": 0.00980392156862745, "content": "Enzyme-linked immunosorbent assay (ELISA) ELISA for bullous pemphigoid are commercially available to test for circulating Ig against NC16A domain of BP180 and BP230, known as bullous pemphigoid antigen 2 [BPAg2] and bullous pemphigoid antigen 1 [BPAg1] respectively. Antibodies to BP180NC16A domain is useful for the diagnosis of bullous pemphigoid as it has a sensitivity of 89% and specificity of 98%. Detection of BP180 and/or BP230 antibodies in serum does not give a confirmative diagnosis of bullous pemphigoid. A study has reported that 7% were tested positive for one or both autoantibodies in one series of 337 people without bullous pemphigoid. ELISA findings should be correlated with DIF to reduce the risk of misdiagnosis. Treatment The treatment for bullous pemphigoid include: 1. Corticosteroids i. Topical Corticosteroids ii. Systemic corticosteroids 2. Glucocorticoid-sparing drugs i. Immunosuppressive drugs ii. Anti-inflammatory drugs 3. Biologic therapy"}, {"id": "wiki20220301en420_21560", "title": "Systemic-onset juvenile idiopathic arthritis", "score": 0.00973389355742297, "content": "Treatment Treatment with either glucocorticoids, methotrexate, anakinra, or tocilizumab has been examined. Anakinra has been shown to resolve the clinical features of the disease in 87% of patients. It also induces remission in half of corticosteroid-resistant patients. The results of another study were similar, with half of the patients responding to treatment with Anakinra. Canakinumab, an antibody to interleukin-1 beta, is indicated for treatment in patients who respond poorly to other treatments. Prognosis 25% of cases progress to severe destructive arthritis. In the United States, mortality is estimated at about 4% and in Europe, mortality is estimated at 21.7%. History Still's disease is named after English physician Sir George Frederic Still (1861–1941). It was characterized by EG Bywaters in 1971. References External links Arthritis Pediatrics Rheumatology Connective tissue diseases Inflammatory polyarthropathies Idiopathic diseases Rare diseases"}, {"id": "wiki20220301en024_81127", "title": "Autoimmune hepatitis", "score": 0.009708737864077669, "content": "Classification On the basis of detected autoantibodies, autoimmune hepatitis can be classified into three subtypes but have no distinct clinical presentations. Type 1 autoimmune hepatitis. Positive antibodies include: Antinuclear antibody (ANA) Anti-smooth muscle antibody (ASMA) - 65% of people Anti-actin antibodies Anti-mitochondrial antibodies - rare except for overlap syndromes with primary biliary cholangitis Anti-soluble liver antigen/liver pancreas antibody antigen - 20% of people Anti-double stranded DNA - 30% of people Atypical perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) Type 2 autoimmune hepatitis. Positive antibodies include: Liver Kidney Microsomal antibody (LKM-1) Anti-liver cytosol antibody-1 (SLC-1) Autoantibody negative autoimmune hepatitis. Lack positive ANA, ASMA, LKM-1, etc. antibody panels but present with clinical features of autoimmune hepatitis that resolve with standard treatment."}, {"id": "InternalMed_Harrison_25896", "title": "InternalMed_Harrison", "score": 0.009708737864077669, "content": "FIGUrE 390-10 Approach to chronic disease is based on whether glucocorticoid therapy is tolerated or not. Hydroxychloroquine 200–400 mg qd 400 mg qd Eye exam q6–12 mo Ocular B: Some forms of D: Routine eye exam disease Azathioprine 50–150 mg qd 50–200 mg qd CBC, renal q2mo Hematologic, nausea C: Some forms D: Routine hematologic chronic disease monitoring Abbreviations: CBC, complete blood count; PPD, purified protein derivative test for tuberculosis. Source: Adapted from RP Baughman, O Selroos: Evidence-based approach to treatment of sarcoidosis, in PG Gibson et al (eds): Evidence-Based Respiratory Medicine. Oxford, BMJ Books Blackwell, 2005, pp 491–508."}, {"id": "wiki20220301en087_12177", "title": "Löfgren syndrome", "score": 0.009615384615384616, "content": "Löfgren syndrome consists of the triad of erythema nodosum, bilateral hilar lymphadenopathy on chest radiograph, and joint pain. Genetics Recent studies have demonstrated that the HLA-DRB1*03 is strongly associated with Löfgren syndrome. Diagnosis The triad of erythema nodosum, acute arthritis, and bilateral hilar lymphadenopathy is highly specific (>95%) for the diagnosis of Löfgren syndrome. When the triad is present, further testing with additional imaging and laboratory testing is unnecessary. Treatment NSAIDs (nonsteroidal anti-inflammatory drugs) are the usual recommended treatment for Löfgren syndrome. Colchicine or low-dose prednisone may also be used. Prognosis Löfgren syndrome is associated with a good prognosis, with > 90% of patients experiencing disease resolution within 2 years. In contrast, patients with the disfiguring skin condition lupus pernio or cardiac or neurologic involvement rarely experience disease remission."}, {"id": "pubmed23n1003_13224", "title": "Anti-MDA-5 Dermatomyositis Presenting with Rapidly Progressive Interstitial Lung Disease: A Cautionary Tale.", "score": 0.009615384615384616, "content": "A 68-year-old Indian man presented with a pruritic eruption on his neck, back, elbows, knees, and the dorsum of his hands. He was initially treated for possible Lyme's disease by his primary care physician, but without improvement. Then he developed daily chills and fevers up to 101 °F, as well as shortness of breath. A chest radiograph showed patchy airspace opacities suggestive of atypical pneumonia, and the patient was treated with levofloxacin and prednisone. Although prednisone diminished the eruption, the patient continued to experience fever, malaise, and generalized weakness, at which point he was hospitalized. Blood cultures and an antinuclear antibodies (ANA) were negative and extensive lab workup was only notable for an elevated erythrocyte sedimentation rate (ESR) (63 mm/hr, Reference Range 0-22), mild transaminitis (AST 77 U/L, Reference Range 10-40), hyponatremia (131 mEq/L, Reference Range 135-145) and elevated ferritin (440, Reference Range 20-500). The patient was discharged on 20 mg of prednisone, with referral to rheumatology and dermatology for possible autoimmune diseases."}, {"id": "pubmed23n0871_6759", "title": "Systemic Lupus Erythematosus With Acute Inflammatory Demyelinating Polyneuropathy: A Case Report and Review of the Literature.", "score": 0.009523809523809525, "content": "We recently encountered a patient with acute inflammatory demyelinating polyneuropathy (AIDP) that was associated with systemic lupus erythematosus (SLE). A 34-year-old Chinese female with a 3-year history of SLE presented with acute bilateral leg weakness and paraparesis, and lost the ability to walk 1 day after noticing bilateral leg numbness and pain for 12 days. Physical examination revealed bilateral facial muscle paralysis, muscle strength in the legs with graded 1/5 proximally and 2/5 distally bilaterally and absence of deep tendon reflex in both knees and ankles. Paresthesia was observed in distal limbs with glove and stocking distribution. Cerebrospinal fluid analysis demonstrated albuminocytologic dissociation. Electrophysiologic survey also indicated sensory-motor demyelinating polyneuropathy. The diagnosis of SLE was established based on her initial symptoms including intermittent fevers, hair loss, oral ulcers, malar rash and arthritis affecting the elbow, wrist and hand joints; positive immunologic findings for antinuclear antibody (ANA), anti-DNA antibody, anti-Smith (anti-Sm) antibody, low serum complement levels, and the kidney biopsy specimen showed glomerular mesangial proliferation with focal endothelial cell proliferation (ISN/PPS 2004 classification lupus nephritis, class III). Treatment with intravenous immunoglobulin, methylprednisolone and cyclophosphamide resulted in clinical and electrophysiological improvement. "}, {"id": "pubmed23n0702_18357", "title": "[Adult-onset Still's disease].", "score": 0.009433962264150943, "content": "Adult-onset Still's disease is a rare inflammatory systemic disease. Cardinal symptoms/manifestations are fever, arthralgias or arthritis, myalgias, the typical skin rash, sore throat, hepatosplenomegaly, lymphadenopathy and serositis. Several other symptoms and organ involvements are possible. The clinical picture is variable with mild to life-threatening courses. The disease is self-limiting, intermittently active or chronic. Because of the lack of a defined diagnostic test the diagnosis of AOSD can only be made after exclusion of several differential diagnoses in particular of infectious, malignant and autoimmune origin. For therapy non-steroidal anti-inflammatory drugs, glucocorticoids, disease modifying antirheumatic drugs and biologics can be used."}, {"id": "wiki20220301en111_27511", "title": "Pemphigoid", "score": 0.009345794392523364, "content": "Glucocorticoid sparing drugs For patients who require high dose of corticosteroids for clearing or maintenance, glucocorticoid sparing agents such as immunosuppressive drugs and anti-inflammatory drugs can be used as an adjunct therapy to reduce the systemic side effects of corticosteroids. Patients who have comorbidities and contraindications for corticosteroids may also consider these glucocorticoid sparing agents. Immunosuppressant drug Immunosuppressant drugs include azathioprine (1–3 mg/kg/day in two equally divided doses), mycophenolate mofetil (1000–3000 mg/day or 40 mg/kg/day in two divided doses), and methotrexate (10–15 mg/week)."}, {"id": "pubmed23n0688_16038", "title": "Adult onset Still's disease: review of 41 cases.", "score": 0.009345794392523364, "content": "To describe the clinical, laboratory and radiological features, treatment and prognosis of patients with adult onset Still's disease (AOSD). Specific clinical features were retrospectively recorded in 41 patients fulfilling the Yamaguchi criteria. Patients were reviewed in two academic hospitals with a referral area of 700,000-1,000,000 inhabitants. Laboratory tests including haemogram, ferritin, biochemistry and autoimmunity were reviewed. Radiological studies, treatment and ACR functional class were determined. Forty-one patients with AOSD were identified, 25 of whom were female. Mean age at diagnosis: 38.19 years (range 17-68). Feverish polyarthritis was the most common clinical presentation. Acute phase reactants were invariably high in all patients. Serum ferritin levels were elevated in 86% of patients. Anti-cyclic citrullinated peptide antibodies (anti-CCP antibodies) were negative in all patients except one. The course of the disease was monocyclic in 44% of the patients, polycyclic in 26%, and chronic articular in 30%. ACR class was as follows: 29 (72.5%) class I, 7 (17.5%) class II, 2 (5%) class III and 2 (5%) class IV. As for the treatment received, aspirin or NSAIDs controlled the disease in eight patients (19.5%) and high-dose corticosteroids (0.5-1 mg/kg/day) in 32 (78%). Almost half of the patients (49%) required an additional diseasemodifying agent, usually methotrexate. Finally, in seven of them (17%) a biological treatment with TNF-α or specially anti-IL-1 had to be added to control the disease. The clinical and laboratory findings were similar to previous studies. Anti-CCP antibodies were almost always negative. A monocyclic course was associated with a good prognosis. Most of the patients were in ACR functional class I and II. Biological agents were required in 7 patients (17%)."}, {"id": "wiki20220301en129_17765", "title": "Drug-induced lupus erythematosus", "score": 0.009259259259259259, "content": "Diagnosis Antinuclear antibodies are usually positive in drug-induced Lupus. Anti-Neutrophil Cytoplasmic antibodies (ANCA) can also be positive in association with certain drugs. Furthermore, Anti-Histone antibodies can also be positive in drug-induced lupus. Anti-Histone antibodies are positive in up to 95% of patients with drug induced lupus. The most common medications associated with drug induced lupus are hydralazine, procainamide, isoniazid, methyldopa, chlorpromazine, quinidine, and minocycline. Treatment It is important to recognize early that these drugs are causing DIL like symptoms and discontinue use of the drug. Symptoms of drug-induced lupus erythematosus generally disappear days to weeks after medication use is discontinued. Non-steroidal anti-inflammatory drugs (NSAIDs) will quicken the healing process. Corticosteroids may be used if more severe symptoms of DIL are present."}, {"id": "pubmed23n0652_14323", "title": "[Polyarteritis nodosa with a spontaneous recovery].", "score": 0.009259259259259259, "content": "The periarteritis nodosa (PAN) is a serious necrotizing vasculitis. Healing is, classically, obtained after a long-term treatment using corticosteroids and immunosuppressive agents. Reporting the case of a NAP or was spontaneous healing without having recourse to any immunosuppressive treatment. We report an observation of a patient aged 27 monitoring for manic-depressive psychosis hospitalized for a fever during the course associated with high blood pressure (hypertension), a weight loss, asthenia and myalgia. Biology was a biological inflammatory syndrome (SIB), a creatinine 115 micromol/l. The survey infectious and immunological balance were negative. The arteriography showed a strongly evocative of the NAP. We have seen no sign of poor prognosis. The recovery was spontaneous after a short period of evolution with an amendment clinical and biological weapons. The patient was put under anti-HTA and reviewed regularly to the consultation. An analogy is drawn between such developments and already reported in some cases of viral PAN. The actual frequency of self-limitting PAN is unknown. The scarcity of cases reported in the literature may in part be attributed to ignorance."}, {"id": "pubmed23n1132_18000", "title": "Can SARS-CoV-2 infection trigger rheumatoid arthritis? A case report.", "score": 0.009174311926605505, "content": "Inflammatory arthritis has been reported after SARS-COV-2 infection. We present a case of a 38-year-old female patient who developed polyarthralgia 1 month after SARS-COV-2 infection. Musculoskeletal examination was significant for synovitis of hands and wrists. Antinuclear antibody (ANA), rheumatoid factor (RF), and anti-cyclic citrullinated peptide (CCP) antibodies were positive. Magnetic resonance imaging of the hands showed synovitis of the metacarpophalangeal joints and proximal interphalangeal joints of the hands, wrist joints, and tendinitis with tenosynovitis. The patient was diagnosed with seropositive nonerosive rheumatoid arthritis (RA) and initiated on therapy using nonsteroidal anti-inflammatory agents and disease-modifying anti-rheumatic drug methotrexate leading to an improvement in symptoms."}, {"id": "pubmed23n0835_18846", "title": "[Microscopic polyangiitis associated with antineutrophil cytoplasmic antibodies: clinical features].", "score": 0.009174311926605505, "content": "To study the clinical features of early- and extended-stage microscopic polyangiitis (MPA) and its outcomes on the basis of a long-term follow-up in a rheumatologist's practice. The clinical features of early- and extended-stage MPA were studied in detail and the premorbid background and possible precipitating factors were analyzed in 70 patients with MPA and the proven hyperproduction of antineutrophil cytoplasmic antibodies (anti-proteinase-3 (anti-PR3) antibodies in 55% and anti-myeloperoxidase (anti-MPO) antibodies in 45%) who had been followed up for more than a year. There is evidence for the nosological unity of the two immunological types of MPA associated with anti-PR-3 or anti-MPO antibodies. MPA has been demonstrated to be an aggressive, polysyndromic disease prone to recurrences (52%), the typical manifestation of which is glomerulonephritis (94%) that is rapidly progressive in every four cases and accompanied by hemorrhagic alveolitis (69%) and involvement of other organs. ENT organs and lungs have been noted to be commonly involved in early-stage MPA, which was observed in 61% of the patients in the premorbid period, and to become the first manifestation of MPA (63%) concurrent with body temperature rises (64%), arthralgia or arthritis (41%). Respiratory tract involvement in MPA may be asymptomatic. Anti-PR-3-associated MPA may manifest itself more aggressively and in the first 2 years it is characterized by a poorer prognosis than of anti-MPO-associated MPA (survival rates, 82 and 94%, respectively; p = 0.04). With time, the differences were levelled off; recurrences in the patients with anti-PR-3 and anti-MPO develop equally frequently and proceed showing the similar clinical picture; the survival curves converge by age 3. Anti-MPO-associated MPA is characterized by the highest rate of lung involvement in the clinical phase of the disease (61%) and by a propensity to develop hemorrhagic alveolitis, diffuse interstitial (22%) or circumscribed pulmonary fibrosis in the outcome. CONCLUSION. The findings emphasize how important to diagnose MPA early and to prescribe long-term active treatment using the entire current arsenal of medications as soon as possible until severe injury to organs and systems develops. To specify regularities in the development of MPA may be of value for the better diagnosis of the disease and the further elaboration of optimal treatment policy."}, {"id": "wiki20220301en243_17832", "title": "Gero Hütter", "score": 0.00909090909090909, "content": "to express wildtype CCR5 (because they hadn't been replaced yet from bone marrow precursors), also had no detectable virus. After 600 days without antiretroviral drug treatment, the patient's blood, bone marrow and bowel HIV levels were below the limit of detection; the virus was thought to be present in other tissues. However, the patient actually had a brain biopsy, in addition to biopsies of his intestines, liver, lymph nodes, bone marrow—basically, every part of the body that can be biopsied. All were negative for virus. There is no virus in this person's body out to two and a half years off of all anti-HIV drugs. His antibody levels—called titers—are declining just the way expected if the patient was vaccinated against HIV and then the levels of antibodies were examined. They'd be very strong in the beginning, but would weaken if they are not re-exposed to the virus. It is believed this patient has no HIV in his body and therefore there is nothing to re-expose him, so the"}, {"id": "pubmed23n0506_104", "title": "Unusual presentation of lupus nephritis.", "score": 0.00909090909090909, "content": "We report a male patient who presented with pyrexia, generalized lymphadenopathy, hepatosplenomegaly, and pleural effusion with no cutaneous or musculoskeletal symptoms. Despite extensive investigation, no cause was detected. His initial serology was also negative for autoantibodies. The patient was placed on a trial of antitubercular treatment in view of a positive Mantoux test. His disease evolved into the full clinical picture of systemic lupus erythematosus with nephritis (World Health Organisation class IV) and strongly positive antinuclear antibody and dsDNA over a period of months. He was treated successfully with intravenous cyclophosphamide pulses along with oral prednisolone, and the disease was still in remission after 3 years of follow-up."}, {"id": "pubmed23n0293_20390", "title": "[Avellis syndrome in systemic rheumatoid vasculitis].", "score": 0.009009009009009009, "content": "A 74-year-old man presented sudden onset hoarseness and dysphagia. Two months before this event, he had developed arthralgia of the shoulders, elbows, hands and foot and pleuritis which had been alleviated by a treatment with prednisolone. On admission, the patient could not phonate nor swallow at all. His soft palate was elevated at the right side. The uvula moved left when the patient tried to speak. Laryngoscopic examination revealed the paralysis of right vocal cord. The erythrocyte sedimentation rate (79mm/1h), C-reactive protein (5.3mg/dl), rheumatoid factor (310 IU/ml) and Clq-binding immune complex (4.5 micrograms/ml) were elevated. Hepatitis C virus antibody titer was more than 10.8 IU/l. Anti-nuclear antibody was 1:20 (normal &lt; 1:20) and anti-neutrophil cytoplasmic antibody (p-ANCA) was positive. Blood study also revealed the evidences of hemolytic anemia and hypoproteinemia. Hepatitis B virus markers, cryoglobulin, anti-ds DNA, anti-Sm, anti-RNP, anti-SS-A, anti-SS-B antibodies were negative. Magnetic resonance imaging of the brainstem was normal. A sural nerve biopsy revealed patchy demyelination of the fascicles. The teasing of nerve fibers showed segmental demyelination. Chest X-ray showed the interstitial pneumonia and pleuritis in the right lower lung. Otological examination revealed the bilateral secretory otitis media. A treatment with high dose prednisolone, ciclosporin and cyclophosphamide was partially effective. However we could not continue these medication because of the induction of liver damage. The patient died of multi-organ failure around a year after the emergence of aphonia and dysphagia. The autopsy specimen of the right vagus nerve showed the similar patchy damage of nerve fibers as was observed in the biopsied sural nerve. The present case was diagnosed as systemic rheumatoid vasculitis. The syndrome of aphonia and dysphagia due to paralysis of the unilateral soft palate and vocal cord is called Avellis syndrome. This syndrome has been reported mainly in relation with the infarction of lateral medulla. The present case shows that Avellis syndrome can be produced by mononeuritis of the vagus nerve."}, {"id": "pubmed23n0760_16452", "title": "[Anti-synthetase syndrome].", "score": 0.009009009009009009, "content": "Antysynthetase syndrome is considered as a group ofidiopathic inflammatory myositis with charcteristic serologic hallmark--antibodies which recognise the aminoacyl-tRNA synthetasses (ARS). Clinical picture of those patients contains myositis and/or intersticial lung disease (ILD) and/or arthritis and/or fever and/or Raynaud phenomenon and sometimes characteristic look of mechanic's hands. Myositis can be overt, sometimes even absent, while IBP is major cause of morbidity and determines the outcome of the disease. Untill now eight different any-synthetase autoantibodies are recognised, and most frequent are findings of anti-histidyl-tRNa synthetase antibodies. Patients with other ARS autoantibodies usually have severe ILD. Drug of choice are steroids in dosage of 1 mg/kg with immunosupresive agent (azatioprin or methotrexate) while in severe IBP cyclophosphamide is needed. Recently succsesful treatment with rituximab in combination with cyclophosphamide is reported."}, {"id": "wiki20220301en011_6033", "title": "Rheumatology", "score": 0.008928571428571428, "content": "Treatment Most rheumatic diseases are treated with analgesics, NSAIDs (nonsteroidal anti-inflammatory drug), steroids (in serious cases), DMARDs (disease-modifying antirheumatic drugs), monoclonal antibodies, such as infliximab and adalimumab, the TNF inhibitor etanercept, and methotrexate for moderate to severe rheumatoid arthritis. The biologic agent rituximab (anti-B cell therapy) is now licensed for use in refractory rheumatoid arthritis. Physiotherapy is vital in the treatment of many rheumatological disorders. Occupational therapy can help patients find alternative ways for common movements that would otherwise be restricted by their disease. Patients with rheumatoid arthritis often need a long term, coordinated and a multidisciplinary team approach towards management of individual patients. Treatment is often tailored according to the individual needs of each patient which is also dependent on the response and the tolerability of medications."}, {"id": "pubmed23n0853_10391", "title": "A 28-year-old man with chest and joint pains.", "score": 0.008928571428571428, "content": "A 28-year-old man with extensive travel history to developing countries was hospitalised for intermittent sharp chest pains, worst when supine and with inspiration. Two weeks prior to presentation, he had suffered a flu-like illness with a sore throat, which was resolving. Physical examination was notable for mild fever and tachycardia with cervical lymphadenopathy and painful bilateral knee and wrist effusions. Cardiac auscultation was remarkable for a soft early-peaking systolic murmur over the aortic area with a decrescendo early diastolic murmur along the left sternal edge. There was mild leucocytosis, elevation of serum troponin and acute-phase reactants with an ECG showing sinus tachycardia. Echocardiographic windows were extremely limited but suggested the presence of pericardial effusion and aortic regurgitation. Cardiac MRI was performed (figure 1). Viral, microbiological and autoimmune testing was remarkable only for significant elevation of antistreptolysin-O titres (1450 IU rising to 1940 IU, normal &lt;200 IU). Pericardiocentesis revealed an exudative effusion, which was negative by cytology and microbiological analysis, including for tuberculosis and fungi. The most appropriate next step is? Coronary angiographyEndomyocardial biopsyTreatment with colchicine for 3 monthsTreatment with corticosteroidsTreatment with high-dose salicylates and long-term penicillinFor the answer see page 808For the question see page 769."}, {"id": "pubmed23n0913_15886", "title": "Crescentic glomerular nephritis associated with rheumatoid arthritis: a case report.", "score": 0.008849557522123894, "content": "Rheumatoid arthritis is a systemic disorder where clinically significant renal involvement is relatively common. However, crescentic glomerular nephritis is a rarely described entity among the rheumatoid nephropathies. We report a case of a patient with rheumatoid arthritis presenting with antineutrophil cytoplasmic antibody-negative crescentic glomerular nephritis. A 54-year-old Sri Lankan woman who had recently been diagnosed with rheumatoid arthritis was being treated with methotrexate 10 mg weekly and infrequent nonsteroidal anti-inflammatory drugs. She presented to our hospital with worsening generalized body swelling and oliguria of 1 month's duration. Her physical examination revealed that she had bilateral pitting leg edema and periorbital edema. She was not pale or icteric. She had evidence of mild synovitis of the small joints of the hand bilaterally with no deformities. No evidence of systemic vasculitis was seen. Her blood pressure was 170/100 mmHg, and her jugular venous pressure was elevated to 7 cm with an undisplaced cardiac apex. Her urine full report revealed 2+ proteinuria with active sediment (dysmorphic red blood cells [17%] and granular casts). Her 24-hour urinary protein excretion was 2 g. Her serum creatinine level was 388 μmol/L. Abdominal ultrasound revealed normal-sized kidneys with acute parenchymal changes and mild ascites. Her renal biopsy showed renal parenchyma containing 20 glomeruli showing diffuse proliferative glomerular nephritis, with 14 of 20 glomeruli showing cellular crescents, and the result of Congo red staining was negative. Her rheumatoid factor was positive with a high titer (120 IU/ml), but results for antinuclear antibody, double-stranded deoxyribonucleic acid, and antineutrophil cytoplasmic antibody (perinuclear and cytoplasmic) were negative. Antistreptolysin O titer &lt;200 U/ml and cryoglobulins were not detected. The results of her hepatitis serology, retroviral screening, and malignancy screening were negative. Her erythrocyte sedimentation rate was 110 mm in the first hour, and her C-reactive protein level was 45 mg/dl. Her liver profile showed hypoalbuminemia of 28 g/dl. She was treated with immunomodulators and had a good recovery of her renal function. This case illustrates a rare presentation of antineutrophil cytoplasmic antibody-negative crescentic glomerular nephritis in a patient with rheumatoid arthritis, awareness of which would facilitate early appropriate investigations and treatment."}, {"id": "pubmed23n0594_19445", "title": "[Interstitial granulomatous dermatitis with arthritis: four cases].", "score": 0.008849557522123894, "content": "Interstitial granulomatous dermatitis with arthritis, or Ackerman's syndrome, is characterized by inflammatory articular flares of potentially destructive outcome and cutaneous lesions of varied patterns: cord-like lesions on the flanks or violaceous plaques on the trunk and limbs. Histologically, the main features are histiocytes laminated between collagen fibres, sometimes organized in rosettes comprising tiny granulomas surrounding fibres of homogeneous aspect. Four patients fitting this description were investigated. Cutaneous lesions displayed various patterns: cord-like lesions (one patient), brown plaques on the flanks edged with papules (one patient), grossly annular violaceous plaques on the back (one patient), and infiltrated plaques on the palms and thighs (one patient). Joint symptoms were present in all cases (one seropositive rheumatoid arthritis and one seronegative rheumatoid arthritis, both already known; acute rheumatoid arthritis in two cases). The histological pattern was quite similar in all cases, with laminated histiocytes in palisading layers mixed with swollen collagen fibres; \"rosette\" figures were present in two cases. Autoimmune response was seen with antinuclear antibodies (two cases) and/or rheumatoid factor (two cases). The short-term outcome of cutaneous lesions was rapidly favourable in most cases with systemic steroids (one case) or Non Steroidal Anti-Inflammatory Drugs (one case), and spontaneously in one case. This entity, defined by the presence of inflammatory arthritis and cutaneous lesions of various clinical types, either more specific but infrequent (cord-like), or less specific but more frequent (plaque-like), and featuring a peculiar histological pattern, chiefly affects women aged between 30 and 80 years. Pathophysiological mechanisms and nosological borders are debatable and authors consider this condition as either an autonomous disease or else a mere subset of vasculitis with palisading granulomas in collagen vascular disorders, despite the rarity of authentic vasculitis. A setting of autoimmunity is frequently present. Furthermore, a fairly similar histological pattern is sometimes seen in some lesions forming a subset of cutaneous side-effects of drugs. Articular outcome may be unfavourable with joint destruction in more than half of patients, whether or not in a setting of rheumatoid arthritis. Spontaneous resorption can occur. Treatment has not yet been codified and is based primarily on anti-inflammatory drugs."}, {"id": "wiki20220301en400_28934", "title": "Antisynthetase syndrome", "score": 0.008771929824561403, "content": "Diagnosis In the presence of suspicious symptoms a number of test are helpful in the diagnosis: Muscle enzymes are often elevated, i.e. creatine kinase Anti-Jo-1 antibody testing Electromyography Muscle biopsy Pulmonary function testing Lung biopsy Imaging such as High Resolution computed tomography In certain situations, testing of other antibodies, specific imaging (MRI, thoracic high resolution computed tomography), and swallowing evaluation may be needed. Treatment Unfortunately, treatment for the anti-synthetase syndrome is limited, and usually involves immunosuppressive drugs such as glucocorticoids. For patients with pulmonary involvement, the most serious complication of this syndrome is pulmonary fibrosis and subsequent pulmonary hypertension. Additional treatment with azathioprine and/or methotrexate may be required in advanced cases. Prognosis Prognosis is largely determined by the extent of pulmonary damage. References External links"}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 187]], "word_ranges": [[0, 27]], "text": "Given this clinical picture, one possibility is dermatomyositis. Of the proposed tests, the determination of serum aldolase may be useful. Its elevation is characteristic of this disease."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[188, 239]], "word_ranges": [[27, 35]], "text": "The biopsy should be muscular and not subcutaneous."}, "4": {"exist": true, "char_ranges": [[240, 309]], "word_ranges": [[35, 44]], "text": "Anti-smooth muscle antibodies are not characteristic of this disease."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Given this clinical picture, one possibility is dermatomyositis. Of the proposed tests, the determination of serum aldolase may be useful. Its elevation is characteristic of this disease. The biopsy should be muscular and not subcutaneous. Anti-smooth muscle antibodies are not characteristic of this disease.", "full_answer_no_ref": "Given this clinical picture, one possibility is dermatomyositis. Of the proposed tests, the determination of serum aldolase may be useful. Its elevation is characteristic of this disease. The biopsy should be muscular and not subcutaneous. Anti-smooth muscle antibodies are not characteristic of this disease.", "full_question": "A 75-year-old woman consults for violaceous lesions on the hands and neck together with progressive muscle weakness of 3 months of evolution. What diagnostic tests, among those indicated, can be useful for the diagnosis?", "id": 153, "lang": "en", "options": {"1": "Determination of serum aldolase.", "2": "Electroencephalogram.", "3": "Biopsy of subcutaneous cellular tissue.", "4": "Determination of anti-smooth muscle antibodies.", "5": "Genetic study of their descendants."}, "question_id_specific": 74, "type": "RHEUMATOLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en065_36497", "title": "Hereditary inclusion body myopathy", "score": 0.013478025797482042, "content": "Diagnosis The most useful information for accurate diagnosis is the symptoms and weakness pattern. If the quadriceps are spared but the hamstrings and iliopsoas are severely affected in a person between ages of 20 - 40, it is very likely HIBM will be at the top of the differential diagnosis. The doctor may order any or all of the following tests to ascertain if a person has IBM2: Blood test for serum Creatine Kinase (CK or CPK); Nerve Conduction Study (NCS) / Electomyography (EMG); Muscle Biopsy; Magnetic Resonance Imaging (MRI) or Computer Tomography (CT) Scan to determine true sparing of quadriceps; Blood Test or Buccal swab for genetic testing;"}, {"id": "wiki20220301en032_90409", "title": "Becker muscular dystrophy", "score": 0.013436713485426646, "content": "In terms of the diagnosis of Becker muscular dystrophy symptom development resembles that of Duchenne muscular dystrophy. A physical exam indicates lack of pectoral and upper arm muscles, especially when the disease is unnoticed through the early teen years. Muscle wasting begins in the legs and pelvis, then progresses to the muscles of the shoulders and neck. Calf muscle enlargement (pseudohypertrophy) is quite obvious. Among the exams/tests performed are: Muscle biopsy (removes a small piece of muscle tissue, usually from the thigh, to check for dystrophin in muscle cells.) Creatine kinase test (checks the level of Creatine Kinase proteins in the blood. Creatine Kinase proteins are normally found inside of healthy muscle cells, but can be found in the blood when muscle cells are damaged.) Electromyography (shows that weakness is caused by destruction of muscle tissue rather than by damage to nerves.)"}, {"id": "pubmed23n1106_16797", "title": "[Anti-nuclear matrix protein 2 antibody-positive dermatomyositis with the preferential involvement of neck extensors: a case report].", "score": 0.012950971322849215, "content": "A 68-year-old man with a 2-month history of progressive weakness and spontaneous pain in proximal limb muscles presented to our hospital with a dropped head. He started experiencing progressive dysphagia several days before admission. On admission, he had muscle weakness of the limbs and neck extensors with edema and induration in distal extremities. Laboratory tests showed elevation of muscle enzymes. FDG-PET/CT demonstrated multiple hypermetabolic lymph nodes, but the primary site was not identified; thus, metastatic carcinoma of unknown primary origin was considered. The patient was diagnosed with anti-nuclear matrix protein 2 antibody-positive paraneoplastic myopathy based on serum tests. Histological findings of the left biceps brachii muscle biopsy revealed severe variation in fiber size and perifascicular myofiber atrophy. Myofibers exhibited myxovirus resistance protein A expression predominantly in the perifascicular region. Following intravenous methylprednisolone pulse therapy and intravenous immunoglobulin, the patient's muscle strength improved with normalization of muscle enzyme levels. The dropped head was considered to have resulted from the preferential involvement of neck extensors based on the observed FDG-PET/CT uptake in neck extensors."}, {"id": "wiki20220301en000_241100", "title": "Inclusion body myositis", "score": 0.01274876774265278, "content": "A diagnosis of inclusion body myositis was historically dependent on muscle biopsy results. Antibodies to cytoplasmic 5'-nucleotidase (cN1A; NT5C1A) have been strongly associated with the condition. In the clinical context of classic history and positive antibodies, a muscle biopsy might be unnecessary. Differential diagnosis IBM is often initially misdiagnosed as polymyositis. A course of prednisone is typically completed with no improvement and eventually, sIBM is confirmed. sIBM weakness comes on over months or years and progresses steadily, whereas polymyositis has an onset of weeks or months. Other forms of muscular dystrophy (e.g. limb girdle) must be considered as well."}, {"id": "wiki20220301en281_30724", "title": "Multi/minicore myopathy", "score": 0.012167874396135266, "content": "Other features include a long head, low set ears and a short neck. The respiratory muscles can be moderately to severely affected and problems with breathing are common. Genetics The most common causes are mutations in the RYR1 and SEPN1 genes. In these cases the inheritance is autosomal recessive. Less common recessive mutations causing this condition include those in the TTN, MEGF10 and CACNA1S genes. Automsomal dominant mutations associated with this disease include those in MYH7 and CACNA1S. The pathogenesis is not well understood at present. Diagnosis The diagnosis may be suspected on clinical grounds. On blood testing the creatinine kinase may be raised. Imaging with ultrasound or MRI will show abnormalities in the affected muscles but these changes are not diagnostic. The diagnostic test is a muscle biopsy."}, {"id": "wiki20220301en042_31047", "title": "Fukuyama congenital muscular dystrophy", "score": 0.009900990099009901, "content": "Diagnosis In terms of diagnosis of Fukuyama congenital muscular dystrophy, serum creatine kinase concentration and muscle biopsies can be obtained to help determine if the individual has FMCD. FKTN molecular genetic testing is used to determine a mutation in the FKTN gene after a serum creatine kinase concentration, muscle biopsies, and/or MRI imaging have presented abnormalities indicative of FCMD, the presence of the symptoms indicates Fukuyama congenital muscular dystrophy. The available genetic test include: Linkage analysis Deletion analysis Sequence analysis - exons Sequence analysis - entire coding region Treatment"}, {"id": "pubmed23n0093_16117", "title": "[A case of polymyositis with repeated dysphagia and muscle weakness associated with peculiar findings of skin].", "score": 0.009900990099009901, "content": "A case of polymyositis with repeated dysphagia and muscle weakness associated with peculiar findings of skin was reported. The patient was a 67-year-old man. His birth and development was normal. There was no family history of neuromuscular disease. On 26th March 1987 he was admitted to a hospital because of dysarthria and dysphagia after fever and diagnosed as having viral myositis. His conditions improved spontaneously with bed rest and he left hospital on 14th April. On 23rd April he had chill and sore throat with fever. On 27th he was admitted to the same hospital because of dysarthria and muscle weakness of the proximal portion of the upper limbs. These symptoms also improved with bed rest. He had repeated these symptoms several times and then he was admitted to our hospital on 12th June. On examination he showed the skin pigmentation under the right eye and the eruption in the back of hands and the buttocks. Muscle weakness was observed in the proximal portion of the upper limbs and the neck flexor. Laboratory tests in admission were as follows: sGOT 49 mU/ml, sGPT 104 mU/ml, LDH 1064 mU/ml, CPK 565 mM/ml, aldolase 25.2 IU/1/37 degrees C. Electromyography showed the typical myogenic changes and biopsy of left biceps brachii revealed inflammatory cells in the muscle fiber which are specific to polymyositis. Immuno-histochemical study is performed to analyse the subpopulation of mononuclear cells in biopsied muscle and skin. Mononuclear cells infiltrated into perimysium, endomysium and epidermis were positive for T11 and T8, but less positive for T4, B1 and Leu11. On the basis of these findings he was diagnosed as having \"polymyositis syndrome\"."}, {"id": "wiki20220301en093_17532", "title": "Neuromuscular disease", "score": 0.009849310094408134, "content": "Further causes of neuromuscular diseases are : Inflammatory muscle disorders Polymyalgia rheumatica (or \"muscle rheumatism\") is an inflammatory condition that mainly occurs in the elderly; it is associated with giant-cell arteritis(It often responds to prednisolone). Polymyositis is an autoimmune condition in which the muscle is affected. Rhabdomyolysis is the breakdown of muscular tissue due to any cause. Tumors Smooth muscle: leiomyoma (benign) Striated muscle: rhabdomyoma (benign) Diagnosis Diagnostic procedures that may reveal muscular disorders include direct clinical observations. This usually starts with the observation of bulk, possible atrophy or loss of muscle tone. Neuromuscular disease can also be diagnosed by various blood tests and using electrodiagnostic medicine tests including electromyography (measuring electrical activity in muscles) and nerve conduction studies. Genetic testing is an important part of diagnosing inherited neuromuscular conditions."}, {"id": "wiki20220301en601_17481", "title": "Calpainopathy", "score": 0.00980392156862745, "content": "With calpain 3 mutation, proteins typically found at the triad are reduced, including CaMKII (Ca2+/calmodulin-dependent protein kinase II). Decreased CaMKII activity impairs induction of slow oxidative gene expression, which in turn impairs genes involving the mitochondria and lipid metabolism. Diagnosis Genetic testing is the most definitive test. If genetic testing is not available, a muscle biopsy with protein immunoanalysis can be used. Biopsy shows general dystrophic features, such as areas of muscle death, variability in muscle size, nuclei in the center of muscle fibers, and disorganized muscle fibers within muscle cells. Serum creatine kinase, a nonspecific marker of muscle damage, can be elevated early in the disease. Facioscapulohumeral muscular dystrophy (FSHD) can present similarly, although facial weakness and asymetrical weakness is common in FSHD. Management As of 2019, no disease modifying pharmaceuticals are known."}, {"id": "pubmed23n0135_17694", "title": "[Subcutaneous localizations of Castleman's pseudolymphoma. Review of the literature apropos of a case].", "score": 0.00980392156862745, "content": "The angiofollicular lymphoid hyperplasia, first described in 1954 by Castleman in the mediastinum, is a quite rare pseudolymphoma where there are few subcutaneous localizations. Since 1954, more than 300 observations were published including mediastino-pulmonary forms (about 60 p. 100 of the cases) intra-abdominal forms (15 p. 100 of the cases) and superficial forms which represent 25 p. 100 of the cases and associate superficial ganglionic, intra-muscular and subcutaneous localizations. The authors report the observation of a 44-year-old negro who had a subcutaneous tumefaction of the left elbow which appeared recently without a functional sign nor a biological change. The histological findings allowed the diagnosis of Castleman's pseudolymphoma in a hyalino-vascular form or Flendrig's type II. The evolution was marked a few weeks later by a local recurrence of which a second surgery has secured the recovery. The detailed study of the 76 cases of Castleman's superficial pseudo-tumours published in the literature allows us to recall the features of this disease which affects especially the young adult without prevalence of sex at about 25 years old. The circumstances of discovery are univocal, isolated palpable subcutaneous tumefaction in most of the cases. The localizations are distributed by decreasing incidence as following: latero-cervical, axillary, sus-clavicular, inguinal, vulvar, abdominal wall, shoulder, arm, forearm with a few bifocal forms. The histological aspect associated a predominant lymphoid population and vessels with fibro-hyalinous wall which morphological variations have permitted to individualize three forms: a plasmocytic form or Flendrig's type I which should be a stage of beginning often associated with hematological changes, a hyalino-vascular form or Flendrig's type II more frequent and a mixed form or intermediary type. The immunofluorescence, histo-enzymology and immunohistochemistry studies reveal a changeable polyclonal plasmocytosis and a predominance of T-suppressors in the lymphocytic population. The histological differential diagnosis of the superficial forms of the Castleman's pseudolymphoma is rarely set with certain lymphoma in case of ganglionic localization. On the other hand isolated subcutaneous localizations must be distinguished of the Kimura's disease and of the angiolymphoid hyperplasia with eosinophils where the vessels have a different morphology. The evolution is favorable in most of the cases and surgical exeresis insures the recovery.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "wiki20220301en572_13748", "title": "Muscle–eye–brain disease", "score": 0.009708737864077669, "content": "Diagnosis Medical diagnosis for the MEB usually involves the study of family history, measurement of serum CPK level, molecular testing, muscle biopsy and imaging study. Physical examination People with MEB have distinctive facial dysmorphisms. Rounded forehead, thin and drooping lip, micrognathia, midface retrusion, short nasal bridge are the possible indicative evidence for diagnosis. Assessment of motor and mental development, visual ability also provide clues. Genetic test Genetic test can analyze the genome of infants for confirmation of the specific genetic mutation. Mutation in the POMGNT1 is the determinant in the diagnosis of MEB. Several mutations like [c.1539+1G→A], [c.879+5G→T] are the prevalent nucleotide change found in affected people. The commonly used practices collect fetal DNA by chronic villus sampling, followed by linkage analysis and direct sequencing to conclude the POMGNT1 gene sequence."}, {"id": "pubmed23n0621_19682", "title": "[Diagnosis of a myopathic disease in adult].", "score": 0.009708737864077669, "content": "Strategy for the diagnosis of a muscle weakness includes two steps: to rely the weakness to a muscular origin and to find the aetiology. A muscular deficit is purely motor, without sensory signs, involving mainly axial and proximal muscles. The essential informations for aetiological characterization are: 1st) a family history, indicating a genetic origin, 2nd the chronological profile, 3rd) the clinical pattern (deficit topography, modification of muscle volume, fatigability, contractures, myotonia, oculobulbar, respiratory or cardiac involvement), 4th) investigations (CK level, EMG, muscle imaging muscle, biopsy, genetic testing). Two autosomal dominant myopathies begin in adulthood: Steinert's myotonic dystrophy characterized by myotonia facial and distal weakness and atrophy, plurisystemic involvement and facio-scapulo-peroneal dystrophy with asymmetric facial and scapulo-humeral weakness. If the evolution is rapid and family history absent, a curable myopathy (inflammatory, toxic, iatrogenic, and endocrine) is to be looked for. Inclusion body myositis is the most frequent myopathy after the age of 50 years."}, {"id": "pubmed23n0559_9392", "title": "Dermatomyositis with panniculitis.", "score": 0.009615384615384616, "content": "Case 1. A 23-year-old white housewife presented with an erythematous violaceous rash on her face, neck, chest, and limbs, particularly over the dorsum of the hands and fingers; diffuse alopecia; and an inability to climb stairs and get up from a low seat. The clinical examination showed red to violaceous well-demarcated plaques on sun-exposed areas on the dorsum of the fingers and hands, with periungual erythema and telangiectasia; facial erythema; and heliotrope rash. There was also symmetric involvement of proximal muscles of the limbs. Laboratory examination showed hypergammaglobulinemia, elevated serum aspartate aminotransferase, and serum alanine aminotransferase; normal activities of creatinokinase, lactate dehydrogenase, and aldolase; an antinuclear antibody titer of 1:40 with a speckled pattern; negative anti-DNA and anti-Scl70; and normal serum complement levels (C3, C4, and CH50). Urinalysis results were within normal limits. Skin biopsy histopathology showed hyperkeratosis, edema of the upper epidermis, scattered inflammatory infiltrate, and focal accumulation of mucin in the form of acid mucopolysaccharides. Deep asymptomatic nodules on the inner upper limbs appeared later. Histopathology of these lesions showed focal areas of lobular panniculitis in the subcutaneous tissue, with lymphoplasmocytic inflammatory infiltrate without vasculitis (Figure 1 and Figure 2). Case 2. A 29-year-old white housewife presented with an erythematous violaceous rash on her face, neck, chest, and lower extremities. Clinical examination showed red to violaceous well-demarcated aching plaques on the internal surface of the thighs and tips of the fingers; periungual erythema and digital petechiae; Raynaud's phenomenon; and bilateral ulnar and cervical enlarged lymph nodes. Laboratory examination showed elevated serum aspartate aminotransferase, alanine aminotransferase, creatinokinase, lactate dehydrogenase, and aldolase; negative venereal disease research test results; an antinuclear antibody titer of 1:1024 with speckled pattern; negative anti-DNA and anti-Scl70; and normal serum complement levels (C3, C4, and CH50). Urinalysis results were within normal limits. Histopathology of the deep asymptomatic nodule on the inner left thigh showed lobular panniculitis with a scattered inflammatory infiltrate and diffuse fat necrosis, in addition to calcium deposition between the lipocytes and microcysts without vasculitis (Figure 3)."}, {"id": "pubmed23n0060_9591", "title": "[Congenital muscular dystrophy: clinical study of 17 patients].", "score": 0.009615384615384616, "content": "We concur with the idea that congenital muscular dystrophy (CMD) is a distinct clinical entity, and report 17 patients (2 negroes and 15 whites; 12 M and 5 F; median age 6 years, range 1 to 24 years) with genetic, clinical, laboratorial, electrophysiological and histochemical studies. All our cases have an inheritance compatible with an autosomal recessive pattern. A decrease in fetal movements was reported by 57% of the mothers, generalized hypotonia at birth was present in 82%, limb girdle and neck weakness, absent or decreased deep tendon reflexes, and limb contractures were present in all. Severe muscular wasting was found in 41%. Calf pseudo-hypertrophy was observed in one patient. A patient was severely mentally retarded and another was borderline. During a 30-month follow-up, the muscle weakness of the majority remained essentially unchanged but the degree of motor activity deteriorated and was proportional to the worsening of the limb contractures. Serum CK levels were normal or increased to a maximum of 8 times. The electromyogram was myopathic in 74%, neurogenic in 13% and normal in 13%. CT scans showed a symmetrical white matter hypodensity in the hemispheres in 8 cases. All but 5 patients were operated upon to release the limb contractures and all were submitted to physical therapy. The contractures recurred in 4 patients submitted to surgery and were probably related to the cessation of physical therapy."}, {"id": "wiki20220301en244_24488", "title": "Metabolic myopathy", "score": 0.009523809523809525, "content": "Diagnosis The symptoms of a metabolic myopathy can be easily confused with the symptoms of another disease. In most cases, a Muscle biopsy is necessary for an accurate diagnosis of the cause of muscle weakness. A blood test can be done under normal circumstances to test for genetic differences and signs of tissue breakdown, or with an added cardio portion that can indicate if muscle breakdown is occurring. An electromyography is sometimes taken in order to rule out other disorders if the cause of fatigue is unknown. Differentiating between different types of metabolic myopathies can be difficult due to the similar symptoms of each type such as Myoglobinuria and exercise intolerance. It has to be determined whether the patient has fixed or exercise induced manifestations, and if exercise related what kind of exercise, before extensive exercise related lab testing is done to determine the underlying cause."}, {"id": "pubmed23n0043_12307", "title": "[A case of chronic multifocal myositis].", "score": 0.009523809523809525, "content": "A 61-year-old civil engineer began to have slowly progressive muscle atrophy in the right shoulder and the left arm at 56 years of age. Muscle wasting became manifest in the left thigh at 59 years and in the right thigh at 60 years. He had mild difficulty in climbing and descending stairs. On examination, although he had notable muscle atrophy in the right trapezius and proximal muscles in the upper and lower extremities, his muscle strength was relatively well preserved. The muscle atrophy was asymmetrical; the right periscapular region and the left upper and lower extremities were more markedly atrophic. In addition, multiple foci of the striking muscle atrophy were noted in the upper trunk and the proximal limb muscles. Fasciculation was not present. Deep tendon reflexes were normal with no pathologic reflexes. Except for a moderately elevated serum creatine kinase level of 709 Ul/l (normal 40-170) and mildly elevated serum myoglobin level of 100 ng/ml (normal &lt; 60), no laboratory tests showed abnormal values suggesting an inflammatory process. Motor and sensory nerve conduction velocities were within normal limits. Electromyography disclosed myopathic and neuropathic changes. Computed tomography (CT) of skeletal muscles showed asymmetrical muscle atrophy and patchy low-density foci. In biopsied left quadriceps and right gastrocnemius muscles which showed partially low density on CT, there was marked variation in muscle fiber size, with necrotic and regenerating fibers, an increased number of centrally placed nuclei, and interstitial fibrosis. There were numerous foci of mononuclear inflammatory cellular infiltration, especially around the blood vessels.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "Neurology_Adams_11294", "title": "Neurology_Adams", "score": 0.009522438402369297, "content": "In addition to these main issues of distinguishing PM and DM from IBM, currently aided by antibody testing, we call attention to the following problems that we have encountered in connection with diagnosis: 1. The patient with proximal muscle weakness is incorrectly diagnosed as having progressive muscular dystrophy (actually, the opposite pertains more often). Points in favor of myositis are (1) lack of family history (although many dystrophies have recessive inheritance); (2) older age at onset; (3) rapid evolution of weakness; (4) evidence, past or present, of other connective tissue diseases; (5) high serum CK values (again, can be high in certain dystrophies); (6) marked degeneration and regeneration in muscle biopsy; and, finally, if there is still doubt, (7) unmistakable improvement with corticosteroid therapy."}, {"id": "wiki20220301en128_50746", "title": "Equine polysaccharide storage myopathy", "score": 0.009433962264150943, "content": "A muscle biopsy may be taken from the semimembranosis or semitendinosis (hamstring) muscles. The biopsy is stained for glycogen, and the intensity of stain uptake in the muscle, as well as the presence of any inclusions, helps to determine the diagnosis of PSSM. This test is the only method for diagnosing Type 2 PSSM. Horses with Type 1 PSSM will usually have between 1.5-2 times the normal levels of glycogen in their skeletal muscle. While abnormalities indicating muscle damage can be seen on histologic sections of muscle as young as 1 month of age, abnormal polysaccharide accumulation may take up to 3 years to develop. Management"}, {"id": "pubmed23n0318_19140", "title": "[A case of fasciitis associated with Basedow's disease and polymyositis].", "score": 0.009433962264150943, "content": "A 39-year-old female suffered from diffuse goiter, palpitation, finger tremor and body weight loss for about one year. Then she developed acute onset of myalgia and swelling of calves, and muscle weakness of proximal limbs. She could not walk because of myalgia and muscle weakness, and was admitted to our hospital 4 days after the onset of muscle symptoms. On admission, her pulse was 110 per minute and she had finger tremor of 11-12 Hz. The thyroid gland was markedly and diffusely enlarged with an elastic soft surface. She presented muscle weakness of proximal limbs and neck, and had intermittent swelling and myalgia on calves. Deep tendon reflexes were increased in all extremities. The erythrocyte sedimentation rate was 22 mm per hour. Eosinophilia was not recognized. Serum CK level was elevated to 671 IU/l. Serum free T3 was higher than 21.7 pg/ml and free T4 was also elevated to 10.19 ng /dl. Serum TSH was lower than 0.05 microU/ml and thyroid stimulating antibody was 1,302.0%. Muscle biopsy of her left gastrocnemius muscle revealed markedly hypertrophic fascia with inflammatory cellular infiltration on HE staining. Inflammatory change was also recognized in muscle tissue and in perivascular region of perimysium. Variation of fiber size, necrotic fibers, and central nuclei were also seen. From these clinical and laboratory findings she was diagnosed as having Basedow's disease associated with fasciitis and polymyositis. Her thyroid function was improved by anti-thyroid drug, and swelling and myalgia of sural regions and weakness of proximal limbs were also improved by steroid therapy. Only one case of Basedow's disease associated with fasciitis and seven cases of that associated polymyositis have so far been reported. This is the first case report of fasciitis associated with Basedow's disease and polymyositis."}, {"id": "pubmed23n0076_3609", "title": "[A case of senile onset rimmed vacuole myopathy with proximally dominant involvement].", "score": 0.009345794392523364, "content": "A 73-year-old woman with progressive proximal-dominant muscular atrophy and weakness was described. She had been well until 70-year-old, when she found difficulty in standing up from sitting position. At age 72 years, she could not raise her arms. Neurological examination showed muscular wasting and weakness in the proximal parts of extremities, shoulder and pelvic girdle. In the thigh, the flexors and adductors were severely affected. Muscular weakness was also observed in m. tibialis anterior. Serum CK and aldolase were normal. Electromyography showed low voltage short duration motor unit potentials with positive sharp waves and fibrillations. Rimmed vacuoles were observed in 4.8% of muscle fibers in biopsy sample obtained from right m. quadriceps femoris. No inflammatory cells, PAS-positive materials and inclusion bodies were observed in the sample. This case differs from distal myopathy with rimmed vacuoles, because the onset was very late and her muscular weakness and atrophy was proximal dominant. This case also differs from inclusion body myositis, because muscle biopsy revealed no inflammatory cells or inclusion body."}, {"id": "wiki20220301en435_2629", "title": "Schwartz–Jampel syndrome", "score": 0.009259259259259259, "content": "Diagnosis Schwartz–Jampel syndrome is diagnosed on the basis of characteristic facial features, skeletal features and myotonia. Blood tests may show elevated serum creatine kinase or aldolase. X-rays, muscle biopsy or electromyography (EMG) may be useful. Genetic testing for the HSPG2 gene may confirm diagnosis. Treatment There is no cure for Schwartz–Jampel syndrome. Treatment is aimed at reducing muscle stiffness and cramping and may include massage, muscle warming and gradual strengthening exercises. Muscle relaxants or anti-seizure medications, especially carbamazepine, may be used. Eye symptoms such as blepharospasm might be relieved by Botox. Otherwise, a variety of surgical procedures have been found to be effective. Malignant hyperthermia, a potential complication of surgery, is a greater risk for people Schwartz–Jampel syndrome and an important consideration when considering surgery. Prognosis Most people with Schwartz–Jampel syndrome have a nearly normal life expectancy."}, {"id": "pubmed23n0220_14234", "title": "[Apropos of a case of myopathy with histologic and electrophysiologic findings of both a myogenic and neurogenic nature].", "score": 0.009259259259259259, "content": "The authors describe the case of a 36 years old woman suffering from muscular weakness with proximo-distal distribution to legs, and proximal to arms. The disease, appeared during the third decade of life, is slowly becoming more serious. Hematochemical analyses are all within a normal standard; EMG and histopathologic findings prove the existence of both a protopathic and neurogenic trouble in studied muscles. A therapy based on prednisone (50 mg/die) and ACTH (1 mg each 5 days) for a fourty days period doesn't cause essential changes in symptomatology. A cousin (on her mother's side) of our patient suffers from probable \"sporadic distal myopathy\". The authors discuss if the disease, shown by this patient, may be considered as an atypical form of SDM or if, what appears more probable, it must be nosographically framed as \"scapuloperoneal atrophy\"."}, {"id": "wiki20220301en330_16141", "title": "Brody myopathy", "score": 0.009174311926605505, "content": "Blood testing may be used to measure serum creatine kinase, which ranges from normal to slightly elevated in those with BD. Skeletal muscle biopsies are used to examine muscle fibers. Biopsies in individuals with BD often show variation in muscle fiber size, atrophied fast-twitch muscle fibers, and increased nuclei number. Electromyography (EMG) can be used in diagnosis to rule out myotonia, or muscle stiffness that is detected by EMG. Individuals with BD have stiff muscles but normal EMG results (pseudo-myotonia), where no myotonic discharges are detected. Genetic testing may also be used in the diagnosis of BD to look for mutations in ATP2A1. Since only some forms of the disease are associated with ATP2A1, results of genetic testing do not always confirm a diagnosis of BD, but are useful to rule out other similar disorders. Treatment"}, {"id": "pubmed23n0325_15644", "title": "[Diagnostic muscle MRI abnormality in a patient with inclusion body myositis].", "score": 0.009174311926605505, "content": "A 64-year-old woman was admitted to our hospital because of muscle weakness and atrophy in the extremities. Four years before admission, he was noticed to have elevated creatine kinase (CK) level, but had no further evaluation. Two years later, she became difficult in standing up and needed a wheelchair. Six months before admission, she noticed muscle wasting in the buttock, thigh, bilateral forearms, and weakness in the upper limbs. On neurologic examination, she had weakness in sternocleidomastoid and all limb muscles, predominantly in the distal portion of the upper extremities. Laboratory study revealed elevated CK, LDH, and aldolase levels, and myogenic change with fibrillation on needle EMG. Muscle biopsy showed myopathic changes with infiltration of mononuclear cells and rimmed vacuoles. The clinical manifestations as well as poor response to corticosteroids therapy were supportive of the diagnosis of inclusion body myositis. However, the distribution of muscle weakness in her wrist, weaker in the extensors than in the flexors, was not characteristic to IBM. This problem was solved by the right forearm MRI which showed a high signal intensity area in flexor muscles, but not in extensors on T1 and T2 weighted images. Accordingly, the muscle MRI of forearm was a diagnostic aid of IBM in this patient."}, {"id": "wiki20220301en082_3387", "title": "MELAS syndrome", "score": 0.00909090909090909, "content": "Diagnosis MRI: Multifocal infarct-like cortical areas in different stages of ischemic evolution, areas that do not conform to any known vascular territory. Initial lesions often occur in the occipital or parietal lobes with eventual involvement of the cerebellum, cerebral cortex, basal ganglia, and thalamus. Lactate levels are often elevated in serum and cerebrospinal fluid. MR spectroscopy may show an elevated lactate peak in affected and even unaffected brain areas. Muscle biopsy shows ragged red fibers. However, genetic evaluation should be done first, which eliminates the need for muscle biopsy in most cases. Diagnosis may be molecular or clinical: Stroke-like episodes before 40 years old Encephalopathy with seizures or dementia Blood lactic acidosis* or ragged red fibers on muscle biopsy"}, {"id": "pubmed23n0070_6846", "title": "[Hypothyroid myopathy. Clinico-pathologic study of 20 cases].", "score": 0.00909090909090909, "content": "20 patients afflicted with primary hypothyroidism were studied in order to evaluate the association of clinical or sub-clinical myopathy, detected by neurophysiological (electromyography) (EMG) or neuropathological methods (muscular biopsy with enzymatic study). 70% of the patients had muscular weakness (moderate in 30% and severe in 40%) of the scapular and pelvic muscles. 60% of the patients had muscular cramps. There was no myodema nor muscular atrophy or hypertrophy. Seric CPK was high in 70% of the cases. EMG was myopathic in 65%. All cases with weakness registered EMG alterations. The histological findings were import findings were important. The enzymatic techniques showed alterations of the fiber subtypes in 90% of the cases. The type I fibers had sarcolemmal and mitochondrial accumules in 85% and 70% had areas without oxidative activity, similar to \"core\". In this study, we did not find any correlation between the evolution time of hypothyroidism, hormonal levels, CPK increase, and muscular weakness. The EMG was myopathic in cases with severe weakness, however, in patients with moderate weakness it could also prove abnormal. There was no correlation between the electric myopathic pattern, CPK levels and thyroid hormones."}, {"id": "wiki20220301en051_31863", "title": "Nemaline myopathy", "score": 0.009009009009009009, "content": "There are several other identified kinds of mutations that lead to Nemaline Myopathies. One affects slow skeletal muscles, one leads to the formation of both nemaline bodies and other abnormal, core-like, structures forming in the patient's muscles. Diagnosis Electromyography or (EMG). This procedure determines if nerve or muscle cells are damaged. Since a common symptom of Nemaline Myopathy is muscle weakness this allows doctors to determine where and why the weakness is occurring. MRI of the Musculoskeletal System. MRI uses a magnetic field to take pictures of body structures and allows physicians to determine if a patient has a certain disease. Needle biopsy A needle biopsy allows a physician to test specific cells in the body. These cells are sent to a laboratory to undergo testing and can further determine why muscle weakness throughout the body could be occurring. This testing can confirm that muscle cells contain rod like structures."}, {"id": "pubmed23n0041_6300", "title": "Congenital muscular dystrophy: a clinico-pathological and follow-up study of 15 patients.", "score": 0.009009009009009009, "content": "Fifteen patients with a presumptive diagnosis of congenital muscular dystrophy were followed for up to 15 years. The diagnosis was based on clinical, enzyme, histological and neurophysiological examinations. The group formed nine per cent of the 160 children suffering from neuromuscular disorders seen at the same hospital during a period of ten years. The muscle weakness was generalized and also involved respiratory muscles and the face. 60 per cent of the children had congenital contractures; these were well amenable to treatment. However, there was a strong tendency for new contractures to form from the second to third year onwards. There were also other signs indicating that the disease process was changing with time. The deep tendon reflexes were present in the beginning but later were usually lost. The serum creatine kinase was raised even to high levels in the first one to two years and gradually sank to normal or near normal values. The histopathological findings changed with time from relatively slight changes compatible with a muscle destroying process to inactive type lesions characterized by fibrotic and particularly adipose tissue replacing muscle fibres. On the basis of these findings it can be assumed that the active disease process is at its height during intrauterine and early postnatal life and then wanes leaving an outburnt or cicatrical state in which new contractures easily develop causing possible deterioration with time. Active treatment is thus of great importance both to overcome neonatal contractures and to prevent new ones to develop."}, {"id": "wiki20220301en323_9175", "title": "Acquired non-inflammatory myopathy", "score": 0.008928571428571428, "content": "If needed, more advanced equipment can be used to help determine whether a patient is suffering from ANIM. This includes: Measurement of serum levels of muscle enzymes Electromyography (EMG) Magnetic Resonance Imaging (MRI) Muscle biopsy When examining the serum levels of muscle enzymes, the relative levels of creatine kinase, aldolase, aspartate aminotransferase, alanine aminotransferase, and lactate dehydrogenase are closely examined. Abnormal levels of these proteins are indicative of both inflammatory myopathy and ANIM."}, {"id": "pubmed23n0668_6610", "title": "[Inflammatory myopathy with an unusual evolution].", "score": 0.008928571428571428, "content": "Inflammatory myopathies are a heterogeneous group of conditions characterized by proximal muscle weakness, nonsuppurative inflammation of skeletal muscle, with elevated muscle enzyme levels and characteristic electromyography and muscle biopsy findings. The authors describe a clinical case of a young woman, admitted with a four day history of bilateral thigh myalgia. She was afebrile and without skin, mucosal or joint involvement. Thigh muscle palpation was painful. Complete blood count revealed leukopenia and thrombocytopenia. High levels of creatine kinase, serum aminotransferases and myoglobin were detected. Metabolic, toxic and drug-related causes were excluded as well as infectious diseases, malignant tumours and endocrine myopathies. Auto-antibodies for connective diseases were negative. Magnetic resonance imaging and electromyography of lower limbs were suggestive of inflammatory myopathy. Generalized muscle weakness and dysphagia were reported subsequently. Clinical and laboratorial improvement was seen after corticotherapy. Muscle biopsy revealed myopathy signs without inflammatory changes or vasculitis. After prednisolone reduction, presently without treatment, she remains asymptomatic with normal laboratorial findings. The authors emphasize in this case of inflammatory myopathy the unusual clinical and laboratory evolution and the importance of a cautious differential diagnosis."}, {"id": "pubmed23n0997_17604", "title": "[Smooth muscle tumors of uncertain malignant potential].", "score": 0.008849557522123894, "content": "To investigate microsatellite instability in smooth muscle tumors of uncertain malignant potential and to compare the results with clinical and morphological data. Histological and immunohistochemical studies were conducted in 26 patients aged 30-63 years (mean age, 37 years) with leiomyomatosis; which revealed intravenous leiomyomatosis in 20 cases, metastasizing leiomyoma in 2, disseminated peritoneal leiomyomatosis in 3, and smooth muscle tumor of uncertain malignant potential in 1 case. Microsatellite instability was studied by fragment analysis on a genetic analyzer using a test system of six markers: D10S1146, D10S218, D10S24, D10S1213, D3S1295, and D9S942. Microsatellite repeat changes characteristic of leiomyosarcomas (heterozygosity loss and/or microsatellite instability in at least one locus studied) were found in 6 patients; all were clinically and morphologically diagnosed as having intravenous leiomyomatosis. In 3 of these 6 cases, leiomyomatosis was accompanied by metastases to the lungs and spread to the peritoneum; heart damage was noted in 2 cases. The data analysis did not allow identification of any significant clinical and morphological criteria for this group. Leiomyomatosis is not a transitional form from benign leiomyoma to leiomyosarcoma, as evidenced by the difference in the status of molecular markers. Analysis of molecular genetic changes in DNA from tumor tissue samples cannot categorically clarify the nature of the disease by identifying the signs of genetic instability; however, there is a need for further accumulation of experience in studying tumors of this group and in identifying the possible association with disease prognosis."}, {"id": "wiki20220301en041_14148", "title": "Muscle biopsy", "score": 0.008812865707003625, "content": "In medicine, a muscle biopsy is a procedure in which a piece of muscle tissue is removed from an organism and examined microscopically. A muscle biopsy can lead to the discovery of problems with the nervous system, connective tissue, vascular system, or musculoskeletal system. Indications In humans with weakness and low muscle tone, a muscle biopsy can help distinguish between myopathies (where the pathology is in the muscle tissue itself) and neuropathies (where the pathology is at the nerves innervating those muscles). Muscle biopsies can also help to distinguish among various types of myopathies, by microscopic analysis for differing characteristics when exposed to a variety of chemical reactions and stains."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 125]], "word_ranges": [[0, 17]], "text": "Systemic corticosteroids in these patients should be used primarily in acute exacerbations, not for control of exacerbations."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Systemic corticosteroids in these patients should be used primarily in acute exacerbations, not for control of exacerbations.", "full_answer_no_ref": "Systemic corticosteroids in these patients should be used primarily in acute exacerbations, not for control of exacerbations.", "full_question": "A 67-year-old man, ex-smoker, with a diagnosis of severe COPD (multidimensional index BODE 5, FEVl 38%, body mass index 23, dyspnea index according to the mMRC 3 scale, distance covered in the 6-minute walk test 260 m) who has had 3 hospital admissions for exacerbation of his COPD in the last 7 months, comes to the consultation. She also has a history of hypertension, ischemic heart disease with AMI 5 years ago and intermittent claudication. In the clinical examination, there is a decrease in vesicular murmur with expiratory wheezing in both lung fields and an oximetry saturation of 88%. Which of the following therapeutic strategies would NOT be recommended for this patient?", "id": 369, "lang": "en", "options": {"1": "Adjustment of inhaled therapy with long-acting bronchodilators combining anticholinergics and beta-2 adrenergics with inhaled glucocorticoids.", "2": "Start oral glucocorticoids for 6 months to control exacerbations.", "3": "Check that the patient performs the inhalation technique correctly.", "4": "Initiate chronic home oxygen therapy regimen.", "5": NaN}, "question_id_specific": 122, "type": "PNEUMOLOGY AND THORACIC SURGERY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0794_1665", "title": "The clinical and integrated management of COPD.", "score": 0.015942028985507246, "content": "COPD is a chronic pathological condition of the respiratory system characterized by persistent and partially reversible airflow obstruction, to which variably contribute remodeling of bronchi (chronic bronchitis), bronchioles (small airway disease) and lung parenchyma (pulmonary emphysema). COPD can cause important systemic effects and be associated with complications and comorbidities. The diagnosis of COPD is based on the presence of respiratory symptoms and/or a history of exposure to risk factors, and the demonstration of airflow obstruction by spirometry. GARD of WHO has defined COPD \"a preventable and treatable disease\". The integration among general practitioner, chest physician as well as other specialists, whenever required, assures the best management of the COPD person, when specific targets to be achieved are well defined in a diagnostic and therapeutic route, previously designed and shared with appropriateness. The first-line pharmacologic treatment of COPD is represented by inhaled long-acting bronchodilators. In symptomatic patients, with pre-bronchodilator FEV1 &lt; 60%predicted and ≥ 2 exacerbations/year, ICS may be added to LABA. The use of fixed-dose, single-inhaler combination may improve the adherence to treatment. Long term oxygen therapy (LTOT) is indicated in stable patients, at rest while receiving the best possible treatment, and exhibiting a PaO2 ≤ 55 mmHg (SO2&lt;88%) or PaO2 values between 56 and 59 mmHg (SO2 &lt; 89%) associated with pulmonary arterial hypertension, cor pulmonale, or edema of the lower limbs or hematocrit &gt; 55%. Respiratory rehabilitation is addressed to patients with chronic respiratory disease in all stages of severity who report symptoms and limitation of their daily activity. It must be integrated in an individual patient tailored treatment as it improves dyspnea, exercise performance, and quality of life. Acute exacerbation of COPD is a sudden worsening of usual symptoms in a person with COPD, over and beyond normal daily variability that requires treatment modification. The pharmacologic therapy can be applied at home and includes the administration of drugs used during the stable phase by increasing the dose or modifying the route, and adding, whenever required, drugs as antibiotics or systemic corticosteroids. In case of patients who because of COPD severity and/or of exacerbations do not respond promptly to treatment at home hospital admission should be considered. Patients with \"severe or \"very severe COPD who experience exacerbations should be carried out in respiratory unit, based on the severity of acute respiratory failure. An integrated system is required in the community in order to ensure adequate treatments also outside acute care hospital settings and rehabilitation centers. This article is being simultaneusly published in Multidisciplinary Respiratory Medicine 2014; 9:25."}, {"id": "pubmed23n0519_20578", "title": "Contemporary issues in the care of patients with chronic obstructive pulmonary disease.", "score": 0.015476190476190477, "content": "Chronic obstructive pulmonary disease (COPD) is the fourth leading cause of death in the United States and is estimated to be responsible for 119,000 deaths in the year 2000 alone. Additionally, COPD places a tremendous burden on the health care system, with estimated annual costs of US 24 billion dollars in 2000, and it is generally expected that costs will continue to rise as more individuals are diagnosed. COPD was responsible for approximately 8 million physician outpatient visits, 1.5 million emergency department visits and 726,000 hospitalizations, also in the year 2000. The objective of this article is to review current, pertinent clinical issues in the management of patients with COPD, with estimates of their relative utility and efficacy. COPD is a disease characterized by airflow limitation that is not fully reversible. Patients with COPD may frequently experience symptoms of chronic cough with sputum production, dyspnea, and reduced exercise capacity. They may frequently experience exacerbations characterized by increased symptoms that often require medical intervention. The diagnosis of COPD is usually fairly straightforward and made in a cigarette smoker, with the aforementioned symptoms and airflow obstruction measured by spirometry. Spirometry should be performed in all patients in whom COPD is suspected, as it provides useful prognostic information and may be used to stage the disease. The Global Initiative for Chronic Obstructive Lung Disease (GOLD) has provided evidenced-based management guidelines for COPD. GOLD guidelines advocate staging COPD by spirometry and make specific treatment recommendations based on COPD stage. The most important risk factor for the development of COPD is cigarette smoking, and smoking cessation has been shown to reduce all-cause mortality and to alter the natural history of COPD. Smoking cessation strategies that employ both counseling and medications like buproprion and nicotine replacement are most effective, but relapse rates remain high. It has not been shown that medications like bronchodilators or inhaled steroids change the natural history of COPD, nor do they reduce mortality, but they can affect other important outcomes. Long-acting bronchodilators, including beta- 2-adrenergic agonists such as salmeterol and formoterol, and the anticholinergic agent tiotropium, improve lung function and exercise tolerance, reduce symptoms, and modestly reduce exacerbation rates. Long-acting bronchodilators are indicated for all COPD patients with chronic symptoms. Short-acting bronchodilators are indicated for rescue when acute symptoms occur. Inhaled corticosteroids minimally improve lung function, but, importantly, reduce exacerbation rates and are indicated in severe COPD or when exacerbations are frequent. Continuous oxygen therapy has been shown to reduce mortality when severe hypoxemia is present and can improve quality of life when moderate hypoxia is present. Finally, well-designed, multidisciplinary disease management programs and pulmonary rehabilitation can improve important disease outcomes in a cost-effective manner. COPD is a common, preventable disease that affects a significant number of people. It may be managed by utilizing various readily available medical therapies, as well as other nonpharmacologic interventions, such as pulmonary rehabilitation. Proper coordination of care is important in this disease, and efforts should be focused on improving quality of life and reduction of symptoms."}, {"id": "pubmed23n0801_9963", "title": "The clinical and integrated management of COPD. An official document of AIMAR (Interdisciplinary Association for Research in Lung Disease), AIPO (Italian Association of Hospital Pulmonologists), SIMER (Italian Society of Respiratory Medicine), SIMG (Italian Society of General Medicine).", "score": 0.015363786202024958, "content": "COPD is a chronic pathological condition of the respiratory system characterized by persistent and partially reversible airflow obstruction, to which variably contribute remodeling of bronchi (chronic bronchitis), bronchioles (small airway disease) and lung parenchyma (pulmonary emphysema). COPD can cause important systemic effects and be associated with complications and comorbidities. The diagnosis of COPD is based on the presence of respiratory symptoms and/or a history of exposure to risk factors, and the demonstration of airflow obstruction by spirometry. GARD of WHO has defined COPD \"a preventable and treatable disease\". The integration among general practitioner, chest physician as well as other specialists, whenever required, assures the best management of the COPD person, when specific targets to be achieved are well defined in a diagnostic and therapeutic route, previously designed and shared with appropriateness. The first-line pharmacologic treatment of COPD is represented by inhaled long-acting bronchodilators. In symptomatic patients, with pre-bronchodilator FEV1 &lt; 60% predicted and ≥ 2 exacerbations/year, ICS may be added to LABA. The use of fixed-dose, single-inhaler combination may improve the adherence to treatment. Long term oxygen therapy (LTOT) is indicated in stable patients, at rest while receiving the best possible treatment, and exhibiting a PaO2 ≤ 55 mmHg (SO2 &lt; 88%) or PaO2 values between 56 and 59 mmHg (SO2 &lt; 89%) associated with pulmonary arterial hypertension, cor pulmonale, or edema of the lower limbs or hematocrit &gt; 55%. Respiratory rehabilitation is addressed to patients with chronic respiratory disease in all stages of severity who report symptoms and limitation of their daily activity. It must be integrated in an individual patient tailored treatment as it improves dyspnea, exercise performance, and quality of life. Acute exacerbation of COPD is a sudden worsening of usual symptoms in a person with COPD, over and beyond normal daily variability that requires treatment modification. The pharmacologic therapy can be applied at home and includes the administration of drugs used during the stable phase by increasing the dose or modifying the route, and adding, whenever required, drugs as antibiotics or systemic corticosteroids. In case of patients who because of COPD severity and/or of exacerbations do not respond promptly to treatment at home hospital admission should be considered. Patients with \"severe\" or \"very severe\" COPD who experience exacerbations should be carried out in respiratory unit, based on the severity of acute respiratory failure. An integrated system is required in the community in order to ensure adequate treatments also outside acute care hospital settings and rehabilitation centers. This article is being simultaneously published in Sarcoidosis Vasc Diffuse Lung Dis 2014, 31(Suppl. 1);3-21. "}, {"id": "pubmed23n0816_19939", "title": "Three-month treatment response and exacerbation in chronic obstructive pulmonary disease.", "score": 0.015343915343915344, "content": "The aim of this study was to investigate relationships between acute exacerbation and Forced Expiratory Volume 1 second (FEV1) improvement after treatment with combined long-acting beta-agonist (LABA) and inhaled corticosteroid (ICS) in patients with chronic obstructive pulmonary disease (COPD). A total of 137 COPD patients were classified as responders or nonresponders according to FEV1 improvement after 3 months of LABA/ICS treatment in fourteen referral hospitals in Korea. Exacerbation occurrence in these two subgroups was compared over a period of 1 yr. Eighty of the 137 COPD patients (58.4%) were classified as responders and 57 (41.6%) as nonresponders. Acute exacerbations occurred in 25 patients (31.3%) in the responder group and in 26 patients (45.6%) in the nonresponder group (P=0.086). FEV1 improvement after LABA/ICS treatment was a significant prognostic factor for fewer acute exacerbations in a multivariate Cox proportional hazard model adjusted for age, sex, FEV1, smoking history, 6 min walk distance, body mass index, exacerbation history in the previous year, and dyspnea scale.Three-month treatment response to LABA/ICS might be a prognostic factor for the occurrence of acute exacerbation in COPD patients. "}, {"id": "pubmed23n0805_16697", "title": "Withdrawal of inhaled glucocorticoids and exacerbations of COPD.", "score": 0.014823717948717948, "content": "Treatment with inhaled glucocorticoids in combination with long-acting bronchodilators is recommended in patients with frequent exacerbations of severe chronic obstructive pulmonary disease (COPD). However, the benefit of inhaled glucocorticoids in addition to two long-acting bronchodilators has not been fully explored. In this 12-month, double-blind, parallel-group study, 2485 patients with a history of exacerbation of COPD received triple therapy consisting of tiotropium (at a dose of 18 μg once daily), salmeterol (50 μg twice daily), and the inhaled glucocorticoid fluticasone propionate (500 μg twice daily) during a 6-week run-in period. Patients were then randomly assigned to continued triple therapy or withdrawal of fluticasone in three steps over a 12-week period. The primary end point was the time to the first moderate or severe COPD exacerbation. Spirometric findings, health status, and dyspnea were also monitored. As compared with continued glucocorticoid use, glucocorticoid withdrawal met the prespecified noninferiority criterion of 1.20 for the upper limit of the 95% confidence interval (CI) with respect to the first moderate or severe COPD exacerbation (hazard ratio, 1.06; 95% CI, 0.94 to 1.19). At week 18, when glucocorticoid withdrawal was complete, the adjusted mean reduction from baseline in the trough forced expiratory volume in 1 second was 38 ml greater in the glucocorticoid-withdrawal group than in the glucocorticoid-continuation group (P&lt;0.001); a similar between-group difference (43 ml) was seen at week 52 (P=0.001). No change in dyspnea and minor changes in health status occurred in the glucocorticoid-withdrawal group. In patients with severe COPD receiving tiotropium plus salmeterol, the risk of moderate or severe exacerbations was similar among those who discontinued inhaled glucocorticoids and those who continued glucocorticoid therapy. However, there was a greater decrease in lung function during the final step of glucocorticoid withdrawal. (Funded by Boehringer Ingelheim Pharma; WISDOM ClinicalTrials.gov number, NCT00975195.)."}, {"id": "pubmed23n1061_20996", "title": "Clinical features of Japanese patients with exacerbations of chronic obstructive pulmonary disease.", "score": 0.01459931798806479, "content": "Exacerbations are critical events in chronic pulmonary obstructive disease (COPD). The frequency of COPD exacerbations is associated with the prognosis, including mortality, but no useful biomarker has been established. The present retrospective study investigated 481 COPD patients. Clinical features in the stable period were compared between patients who experienced severe exacerbation (n = 88, 18.3%) and those who never experienced severe exacerbation (n = 393, 81.7%). In the patients who experienced exacerbations, clinical features were also compared between frequent exacerbators (exacerbation rate ≥ 2 times/year, n = 27, 30.7%) and infrequent exacerbators (1 time/year, n = 61, 69.3%). Compared to COPD patients who never experienced exacerbations, body mass index (BMI), serum albumin, and pulmonary functions were significantly lower, and the cardiovascular disease comorbidity rate, COPD assessment test score, modified Medical Research Council dyspnea scale, and use of long-term oxygen therapy, long-acting β<sub2</sub adrenergic agonist therapy, inhaled corticosteroid therapy, and macrolide therapy were significantly higher in COPD patients with exacerbations (all p &lt; 0.01). In patients who experienced exacerbations, frequent exacerbators had significantly lower % forced expiratory volume in 1.0 s and a higher risk of critical exacerbations, percentage of blood eosinophils, history of mechanical ventilation use, and use of long-term oxygen therapy and of macrolide therapy than infrequent exacerbators (all p &lt; 0.01). On multivariate analysis, the percentage of blood eosinophils was the parameter most correlated with exacerbation frequency (β value [95% confidence interval] 1.45 [1.12-1.88], p &lt; 0.01). Blood eosinophil in the stable period is the factor most correlated with the frequency of severe exacerbations. The patients in this study was registered retrospectively."}, {"id": "wiki20220301en332_33625", "title": "Chronic obstructive pulmonary disease", "score": 0.014539655738473805, "content": "Management of exacerbations People with COPD can experience exacerbations (flare-ups) that are commonly caused by respiratory tract infections. The symptoms that worsen are not specific to COPD and differential diagnoses need to be considered. Acute exacerbations are typically treated by increasing the use of short-acting bronchodilators including a combination of a short-acting inhaled beta agonist and short-acting anticholinergic. These medications can be given either via a metered-dose inhaler with a spacer or via a nebulizer, with both appearing to be equally effective. Nebulization may be easier for those who are more unwell. Oxygen supplementation can be useful. Excessive oxygen; however, can result in increased levels and a decreased level of consciousness. Corticosteroids given orally can improve lung function and shorten hospital stays but their use is recommended for only five to seven days; longer courses increase the risk of pneumonia and death."}, {"id": "wiki20220301en187_4321", "title": "Obstructive lung disease", "score": 0.013025975998784748, "content": "The diagnosis of COPD is established through spirometry although other pulmonary function tests can be helpful. A chest X-ray is often ordered to look for hyperinflation and rule out other lung conditions but the lung damage of COPD is not always visible on a chest x-ray. Emphysema, for example, can only be seen on CT scan. The main form of long term management involves the use of inhaled bronchodilators (specifically beta agonists and anticholinergics) and inhaled corticosteroids. Many patients eventually require oxygen supplementation at home. In severe cases that are difficult to control, chronic treatment with oral corticosteroids may be necessary, although this is fraught with significant side effects."}, {"id": "pubmed23n0876_20261", "title": "Compare the efficacy and safety of long-acting anticholinergic and a combination of inhaled steroids and long-acting beta-2 agonist in moderate chronic obstructive pulmonary disease.", "score": 0.01086765481281856, "content": "The treatment of COPD (Chronic Obstructive Pulmonary Disease) aims to improve the patients's well-being and to reduce mortality, morbidity and the development of exacerbations. This study was thus designed to compare the efficacy and tolerability of salmeterol/fluticasone combination with tiotropium in patients with moderate COPD. This was an open, prospective, randomized trial in COPD patients whose FEV1 (forced expiratory volume in 1 second) levels were between 80% and 50% predicted. A total of 44 patients who met the inclusion and exlusion criteria and who gave written informed consent were included in the study. At the end of the two week wash-out period, the patients were randomized to receive either salmeterol 50 µg/fluticasone 500 µg combination as dry powder inhaler twice daily (SF Group) or tiotropium dry powder inhaler 18 µg once daily (T Group) for one year. These were equally distributed in the two groups (22 patients in each study group). At follow-up, the patients were required to come to the outpatient clinic at the third, sixth, ninth and twelfth months. There were no statistically significant difference between the two groups with regards to demographic features and baseline measurements. There were 1.2 ± 1.7 exacerbations in SF Group and 2.1 ± 2.2 exacerbations in T Group (p= 0.070). The time to the first exacerbation was 4.2 ± 4.0 and 4.2 ± 3.3 months, respectively (p= 0.697). The number of severe exacerbations that resulted in admission to the emergency department or hospital was 0.6 ± 1.0 and 1.1 ± 1.4, respectively (p= 0.245). Significant improvements were observed in CAT (CPOD Assessment Test) scores in both groups during the treatment period (p&lt; 0.0001); but there was no difference between the two groups. This study has shown that in patients with moderate COPD, treatment with combined corticosteroid and long-acting beta-2 agonist provides similar improvements in pulmonary function tests, patient-reported outcomes and exercise capacity as compared a long-acting anticholinergics."}, {"id": "pubmed23n0316_5698", "title": "Multicentre randomised placebo-controlled trial of inhaled fluticasone propionate in patients with chronic obstructive pulmonary disease. International COPD Study Group.", "score": 0.010681818181818181, "content": "The efficacy of inhaled corticosteroids in the treatment of chronic obstructive pulmonary disease (COPD) remains controversial because of a lack of placebo-controlled studies. We compared the effect of inhaled fluticasone propionate with placebo in the treatment of patients with COPD. We used a randomised, double-blind, placebo-controlled design. We enrolled from 13 European countries, New Zealand, and South Africa, 281 outpatient current or ex-smokers, aged between 50 and 75 years. They had a forced expiratory volume in 1 s (FEV1) of between 35% and 90% of predicted normal values, a ratio of FEV1 to forced vital capacity of 70% or less and bronchodilator reversibility of less than 15%, as well as a history of chronic bronchitis. Patients were randomly assigned fluticasone propionate 500 microg (n=142) or placebo (n=139) twice daily via a metered-dose inhaler for 6 months. The main outcome measures were the number of patients who had at least one exacerbation by the end of treatment, the number and severity of exacerbations, clinic lung function, diary card symptoms and peak expiratory flow and 6 min walking distance. 51 (37%) patients in the placebo group compared with 45 (32%) in the fluticasone propionate group had had at least one exacerbation by the end of treatment (p=0.449). Significantly more patients had moderate or severe exacerbations in the placebo group than in the fluticasone propionate group (86% vs 60%, p&lt;0.001). Diary-card and clinic morning peak expiratory flows improved significantly in the fluticasone propionate group (p&lt;0.001, p=0.048, respectively), as did clinic FEV1 (p&lt;0.001), forced vital capacity (p&lt;0.001), and mid-expiratory flow (p=0.01). Symptom scores for median daily cough and sputum volume were significantly lower with fluticasone propionate treatment than with placebo (p=0.004 and p=0.016, respectively). At the end of treatment, patients on fluticasone propionate had increased their 6 min walking distance significantly more than those on placebo (p=0.032). Fluticasone propionate was tolerated as well as placebo, with few adverse effects and without a clinically important effect on mean serum cortisol concentration. Fluticasone propionate may be of clinical benefit in patients with COPD over at least 6 months. Inhaled corticosteroids may have an important role in the long-term treatment of COPD."}, {"id": "pubmed23n1069_20693", "title": "Correlation of BODE index with quality of life in stable Chronic Obstructive Pulmonary Disease (COPD) patients - A prospective study.", "score": 0.009900990099009901, "content": "Chronic obstructive pulmonary disease (COPD) is characterized by slow progressive deterioration of respiratory function with systemic effects which have a great impact on health-related quality of life (HRQoL). The severity of airflow limitation in COPD, as reflected by forced expiratory volume in one second (FEV1) does not represent the systemic consequences of COPD. Hence, a multidimensional grading system, BODE index (Body mass index, Airflow obstruction, Dyspnea and Exercise capacity) that assessed both the pulmonary and systemic manifestations has recently been proposed to provide useful prognostic information and predict the outcome in COPD patients. The objective of this study was to evaluate the relationship between BODE index and health-related quality of life (HRQOL) in stable COPD patients and its usefulness in predicting the disease exacerbations. Sixty stable COPD patients who presented in the out-patient departments of Medicine and Pulmonology were recruited over one year period. We evaluated them by body-mass index, forced expiratory volume in one second (FEV1), Modified Medical Research Council dyspnea scale and six minute walk test (6MWT). BODE index was calculated using these variables. Disease duration, number of exacerbations and hospitalization in the previous year were recorded. We also administered the St. George's Respiratory Questionnaire (SGRQ) to assess the health related quality of life (HRQoL) in these patients. BODE scores were categorized into four quartiles, quartile one to four with scores of 0-2, 3-4, 5-6 and 7-10, respectively. According to BODE score, (16) 26.7% of patients were BODE 1, (27) 45% BODE 2, (15) 25% BODE 3 and (2) 3.3% were BODE 4. Higher BODE quartiles were associated with higher total SGRQ scores and SGRQ subscale scores (symptom, activity and impact). Very strong correlations were found between BODE quartiles and total SGRQ scores (<iP</i &lt; 0.01). Among the components of BODE index, the decrease in the FEV1 (%predicted) and 6MWD, and the increase of MMRC dyspnea grade were associated with worsening of health status (increase in total SGRQ and SGRQ subscales). BODE index also correlated with the acute exacerbations (<iP</i &lt; 0.0012) during one year follow-up. BODE index strongly correlated with the HRQoL and also reliably predicted acute exacerbations in stable COPD patients."}, {"id": "pubmed23n0673_21841", "title": "Management and survival of patients admitted with an exacerbation of COPD: comparison of two Danish patient cohorts.", "score": 0.009900990099009901, "content": "  The aim of this study was to describe the management and prognosis related to a hospital admission for acute exacerbation of chronic obstructive pulmonary disease and to compare results to an earlier study.   This is a retrospective study of 300 consecutively discharged patients admitted in 2006-2007 with an exacerbation of chronic obstructive pulmonary disease from three respiratory departments. Data were collected from patient charts and compared with a replicate study done in 2001.   The mean age was 72.1years; 61.7% were women. Mean forced expiratory volume in 1s was 37.6% of predicted. On admission, 11.3% were treated with non-invasive ventilation, and 84.3% were given systemic corticosteroids. In-hospital mortality was 4.7%. At discharge, treatment with inhaled corticosteroids or at least one long-acting bronchodilator was given to 86.7% and 89% of patients, respectively, which was significantly higher than for similarly sampled patients in 2001. Mortality in 30days and 1year after discharge was 4.5% and 25.5%, respectively, compared with 5.5% and 30.3% in 2001, the 12-month mortality being significantly lower (P=0.03). Readmission rate in the 12months following discharge was 42.3%. Long-term oxygen treatment, treatment with anti-dysrhythmic drugs and lack of outpatient follow-up were independent predictors of 1-year mortality. Risk of readmission was increased with dependence in self-care activities, previous admissions and treatment with strong analgesics.   Over a period of 6years, a significantly higher number of patients are being treated according to guidelines. Survival following discharge increased over the same period."}, {"id": "pubmed23n0824_8181", "title": "Do frequent moderate exacerbations contribute to progression of chronic obstructive pulmonary disease in patients who are ex-smokers?", "score": 0.00980392156862745, "content": "In addition to smoking, acute exacerbations are considered to be a contributing factor to progression of chronic obstructive pulmonary disease (COPD). However, these findings come from studies including active smokers, while results in ex-smokers are scarce and contradictory. The purpose of this study was to evaluate if frequent acute moderate exacerbations are associated with an accelerated decline in forced expiratory volume in one second (FEV1) and impairment of functional and clinical outcomes in ex-smoking COPD patients. A cohort of 100 ex-smoking patients recruited for a 2-year follow-up study was evaluated at inclusion and at 6-monthly scheduled visits while in a stable condition. Evaluation included anthropometry, spirometry, inspiratory capacity, peripheral capillary oxygen saturation, severity of dyspnea, a 6-minute walking test, BODE (Body mass index, airflow Obstruction, Dyspnea, Exercise performance) index, and quality of life (St George's Respiratory Questionnaire and Chronic Respiratory Disease Questionnaire). Severity of exacerbation was graded as moderate or severe according to health care utilization. Patients were classified as infrequent exacerbators if they had no or one acute exacerbation/year and frequent exacerbators if they had two or more acute exacerbations/year. Random effects modeling, within hierarchical linear modeling, was used for analysis. During follow-up, 419 (96% moderate) acute exacerbations were registered. At baseline, frequent exacerbators had more severe disease than infrequent exacerbators according to their FEV1 and BODE index, and also showed greater impairment in inspiratory capacity, forced vital capacity, peripheral capillary oxygen saturation, 6-minute walking test, and quality of life. However, no significant difference in FEV1 decline over time was found between the two groups (54.7±13 mL/year versus 85.4±15.9 mL/year in frequent exacerbators and infrequent exacerbators, respectively). This was also the case for all other measurements. Our results suggest that frequent moderate exacerbations do not contribute to accelerated clinical and functional decline in COPD patients who are ex-smokers."}, {"id": "pubmed23n0621_575", "title": "[Acute exacerbations of chronic obstructive pulmonary disease].", "score": 0.00980392156862745, "content": "Acute exacerbation of chronic obstructive pulmonary disease (COPD) is defined by modification of the usual COPD symptoms, dyspnea, coughing and sputum, beyond daily variations, with a sudden onset, and requiring modification of the usual treatment. Exacerbations stud the course of COPD. Their frequency is variable, averaging 1-2 per year. Their frequency generally increases with COPD severity. Exacerbations impair patients' quality of life and aggravate disease prognosis by accelerating the decline in FEV1, the primary indicator of respiratory function. The most frequent causes of exacerbations are viral and bacterial respiratory infections and pollution. No cause is identified for nearly one third of all exacerbations. Most exacerbations can be treated at home, if a careful search for signs of clinical severity is negative. Treatment combines inhaled bronchodilator agents (beta-2 agonists, combined if necessary with anticholinergics) and oral corticosteroid therapy (prednisone: 0.5 mg/kg/d for 1 week) when the COPD is severe or signs of severity accompany the exacerbation. Antibiotic therapy is justified when the sputum appears purulent. Severe exacerbation may require oxygen therapy in cases of severe hypoxemia (PaO(2)&lt;60 mm Hg) or mechanically assisted ventilation, essentially by noninvasive ventilation in cases of respiratory acidosis (pH&lt;7.35). Noninvasive ventilation improves dyspnea and respiratory acidosis, diminishes respiratory frequency, intubation, duration of hospitalization, nosocomial infections, and mortality. Pulmonary follow-up is necessary after an exacerbation, especially to prevent the recurrence of exacerbations by measures that have been demonstrated to be effective, including help in smoking cessation, adaptation of COPD treatment, vaccination against influenza and pneumonia (pneumococci), and respiratory rehabilitation. Early diagnosis and rapid treatment of exacerbations can limit their impact, improve quality of life, and reduce the risk of hospitalization."}, {"id": "pubmed23n0573_4597", "title": "[Chronic obstructive pulmonary disease in the setting of hospital at home. Study of 522 episodes].", "score": 0.009708737864077669, "content": "To assess the effectiveness, respiratory status, services of origin and outcome of patient with exacerbated COPD attended in Hospital at Home (HaH) regimen. Study of patients with an exacerbated COPD in HaH from Vitoria-Gasteiz, Spain during the period March 1999-October 2004, in whom hospital admission had been recommended after medical assessment. We studied: age, gender, patient's stay, oxygen-saturation or arterial blood gas analysis, FEV1 (basal), dyspnea status (basal and current), coexisting diseases, exacerbation causes, Services of origin, use of home nebulizers and oxygen therapy, intravenous drugs, course (discharges/admissions/deaths). We analyzed the number of visits to the Emergency Department and hospital admissions 90 days before and after discharge from Hospital at home. A total of 302 patients who generated 522 cases with exacerbated COPD were accepted, 81% of whom are men. Means stay was 11 days (0-111). Three hundred ninety six (76%) of the cases were discharge from HaH, 111 (21%) had to be hospitalized for different reasons, on 13 (2.5%) died. Of these, 43% came from the Respiratory Department and 39% from the Emergency one. Mean FEV1 was 45.4. A total of 89% of the patients had dyspnea 4/4 and 34% 3/4 when seen and 9% of the patients had pneumonia. During the 90 days following discharge from Hospital at Home, the number of visits to the Emergency Department and the rate of hospital admissions decreased significantly (p &lt; 0.001). Our data confirm that Hospital at Home is a good alternative to conventional hospital admission for the management of patients with exaxerbated COPD."}, {"id": "wiki20220301en266_12808", "title": "Acute exacerbation of chronic obstructive pulmonary disease", "score": 0.009688013136289, "content": "Treatment Based on the severity different treatments may be used. Mild exacerbations are treated with short acting bronchodilators (SABDs). Moderate exacerbations are treated with SABDs together with antibiotics or oral corticosteroids, or both. Severe exacerbations need hospital treatment, and the prognosis is poor. Oxygen Oxygen therapy should be initiated if there is significantly low blood oxygen. High flow oxygen may be harmful in those with an acute exacerbation of COPD. In the prehospital environment those given high flow O2 rather than titrating their O2 saturations to 88% to 92% had worse outcomes. Antibiotics and steroids appear useful in mild to severe disease."}, {"id": "pubmed23n0366_10070", "title": "Randomized controlled trial of supported discharge in patients with exacerbations of chronic obstructive pulmonary disease.", "score": 0.009615384615384616, "content": "A randomised trial was performed on patients presenting to hospital with an exacerbation of chronic obstructive pulmonary disease (COPD) to compare outcomes in those managed at home with support with those admitted to hospital in the standard manner. Over an 18 month period all patients presenting to the Royal Infirmary of Edinburgh on weekdays (n=718) with a diagnosis of an exacerbation of COPD were assessed for inclusion in the trial. Patients with impaired level of consciousness, acute confusion, acute changes on radiography, or an arterial pH of &lt;7.35 or with other serious medical or social reasons for admission were excluded. Patients randomised to home support were discharged with an appropriate treatment package (antibiotics, corticosteroids, nebulised bronchodilators and, if necessary, home oxygen). They were visited by a nurse the following day and thereafter at intervals of 2-3 days until recovery when they were discharged from follow up. Parallel observations were made on patients allocated to normal hospital admission up to the point of discharge. Patients in both groups were assessed at home eight weeks after the initial assessment. Among weekday patients 353 (50%) were considered obligatory admissions, 140 (19%) were admitted because of co-morbidity, 17 (2%) because of poor social circumstances, and 24 (3%) did not consent to the trial. The remaining 184 (26%) were randomised (2:1) either to home support or to a standard hospital admission. The median time to discharge was 7 days for the home support group and 5 days for the admitted group (p&lt;0.01); 25% of the home support group and 34% of the admitted group were readmitted before the final assessment at eight weeks (p&gt;0.05). There were no significant differences between the groups in attendances by GPs and carers or in health status measured eight weeks after the initial assessment. Satisfaction with the service was good. The mean total health service cost per patient was estimated as 877 pounds sterling for the home support group and 1753 pounds sterling for the admitted group. This study shows that home supported discharge is a well tolerated, safe, and economic alternative to hospital admission for a proportion of patients referred to hospital for admission for an exacerbation of COPD."}, {"id": "pubmed23n0501_12356", "title": "Tiotropium bromide. A review of its use as maintenance therapy in patients with COPD.", "score": 0.009523809523809525, "content": "Tiotropium bromide (Spiriva) is a long-acting anticholinergic bronchodilator that maintains bronchodilation for at least 24 hours, allowing once-daily administration. The active moiety is the tiotropium cation (tiotropium); tiotropium bromide 22.5 micrograms is equivalent to 18 micrograms of tiotropium cation. Greater improvements in lung function from baseline (primary endpoint mean trough FEV(1)) were observed with inhaled tiotropium 18 micrograms once daily than with placebo in 6-month and 1-year randomized, double-blind trials in patients with COPD. Tiotropium improved lung function (trough FEV(1) response) more effectively than ipratropium bromide (ipratropium) 40 micrograms four times daily in 1-year clinical trials, and was at least as effective as salmeterol 50 micrograms 12-hourly in 6-month trials. Preliminary data suggest that tiotropium alone or in combination with once-daily formoterol has a greater bronchodilator effect than twice-daily formoterol in patients with COPD. Improvements in patients' perception of health-related quality of life (HR-QOL) or dyspnea were greater with tiotropium than with placebo or ipratropium, and were similar to those with salmeterol. Reductions in the frequency and severity of acute exacerbations and in the use of rescue medication were also greater with tiotropium than with ipratropium or placebo. There was no evidence of tachyphylaxis with tiotropium during 1-year clinical trials. Inhaled tiotropium was generally well tolerated in clinical trials. Apart from dry mouth, the type and incidence of adverse events with tiotropium were similar to those with ipratropium, salmeterol or placebo in patients with COPD. In conclusion, inhaled tiotropium 18 micrograms once daily improved lung function, dyspnea, and HR-QOL, and decreased the incidence of acute COPD exacerbations and the use of rescue medication relative to placebo or ipratropium in clinical trials in patients with COPD. Tiotropium was at least as effective as salmeterol in terms of bronchodilator efficacy and improvements in dyspnea or HR-QOL. With the exception of dry mouth, the tolerability profile of tiotropium was similar to that with placebo, ipratropium, or salmeterol. Consequently, inhaled tiotropium is likely to be a valuable option for first-line, long-term maintenance therapy in the management of bronchoconstriction in patients with symptomatic COPD. Tiotropium bromide has a quaternary ammonium structure and acts as an anticholinergic bronchodilator; the active moiety is the tiotropium cation (tiotropium). A 22.5 micrograms dose of tiotropium bromide provides 18 micrograms of tiotropium. Orally inhaled tiotropium bromide antagonizes the muscarinic M(1), M(2), and M(3) receptors located in airway smooth muscle, reversing vagally mediated bronchoconstriction. Receptor binding assays and in vitro tests indicate that tiotropium bromide is kinetically selective for M(1) and M(3) receptors over the M(2) receptor, unlike ipratropium bromide, which is nonselective. Animal and in vitro studies showed that tiotropium bromide was more potent ( approximate, equals 20-fold) than ipratropium bromide in displacing [(3)H]N-methylscopolamine (NMS) from muscarinic receptors, and had a more sustained protective effect (&gt;70% inhibition) against NMS binding. Tiotropium bromide was a more potent inhibitor of bronchial contraction than atropine ( approximate, equals 23-fold), and had a slower onset and markedly longer duration of action than atropine or an equipotent dose of ipratropium bromide. Aerosol particle penetration is improved with tiotropium, without delaying mucus clearance from the lungs. Tiotropium 4.5-36 micrograms once daily for 4 weeks increased mean trough and average FEV(1) and FVC and mean PEFR values from baseline compared with placebo, with no evidence of tachyphylaxis. Improvements in trough FEV(1) from baseline with tiotropium 4.5-36 micrograms were not dose dependent. Based on a lack of dose response, the optimal once-daily tiotropium dosage is 18 micrograms. Steady-state trough FEV(1) values are achieved within 48 hours of commencing tiotrochodilation (for &gt;/=24 hours) and an attenuation of the nocturnal decline in FEV(1) that were unaffected by timing of the daily tiotropium dose were seen in randomized, double-blind, placebo-controlled studies in patients with stable COPD. The drug improved static and dynamic lung hyperinflation (evidenced by reduced trapped air volume and increased tidal volume and end-of-exercise inspiratory capacity), and improved exertional dyspnea (during activities of daily living and exertion) and exercise tolerance compared with placebo in randomized, double-blind studies. In patients with stable COPD, improved sleep-related oxygen desaturation that was unaffected by the timing of the daily dose was seen with tiotropium but not with placebo. Clinically significant treatment-related disorders of conduction or rhythm, or changes in heart rate were not observed with tiotropium in this patient group. Mean maximal plasma concentrations (C(max)) were observed within 5 minutes of inhalation of a single dose of tiotropium 18 micrograms in patients with COPD. Plasma drug levels declined to minimum concentrations (C(min)) within 1 hour of treatment in healthy volunteers. Mean steady-state C(max) concentrations (16 ng/L) were achieved after 2-3 weeks of once-daily inhaled tiotropium 18 micrograms in elderly patients with COPD; tiotropium does not appear to accumulate once steady-state has been achieved.The estimated absolute bioavailability of tiotropium at steady state in healthy volunteers was approximately 20-25%, and approximately 72% of the drug is bound to plasma proteins. Excretion of tiotropium is predominantly renal (through active secretion by the kidneys), although in vitro studies suggest that cytochrome P450 (CYP) oxidation (possibly involving CYP2D6 and CYP3A4 enzymes) may have a minor role. In patients with COPD, renal excretion of the unchanged drug at 24 hours (Ae(24)) was approximately 7%. The mean plasma elimination half-life after single or multiple doses in healthy volunteers and elderly patients with COPD was approximately 5-6 days. The renal clearance and urinary excretion of tiotropium decrease with increasing age; however, these changes are not considered to be clinically significant. Because of altered steady-state C(max), C(min), area under the concentration-time curve, and Ae(24) values, caution is required with tiotropium administration in patients with moderate-to-severe renal impairment. The pharmacokinetics of tiotropium in patients with severe renal or hepatic impairment have not been studied. Tiotropium does not interact with drugs such as cimetidine or ranitidine, which are also eliminated by active renal secretion. Orally inhaled tiotropium bromide has been evaluated as a bronchodilator for the management of patients with COPD in randomized, double-blind 6-month and 1-year trials, and in several shorter studies. In clinical trials, COPD was diagnosed according to the American Thoracic Society guidelines. The bronchodilator effect was expressed as the trough FEV(1) response (the mean change in FEV(1) from baseline measured 1 hour prior to and immediately before a scheduled dose), and was the primary endpoint in all but two clinical trials. The bronchodilator effect with tiotropium 18 micrograms once daily was superior to that with placebo in several well designed trials in patients with COPD. Moreover, greater improvements in mean peak and average FEV(1) responses occurred with tiotropium but not with placebo. Mean trough, peak, and average FVC responses, and weekly mean morning and evening PEFR values were also improved to a greater extent with tiotropium than with placebo. Tiotropium demonstrated a greater bronchodilator effect than ipratropium bromide (hereafter referred to as ipratropium when used at approved dosages) 40 micrograms four times daily in two 1-year trials in patients with COPD. Mean peak and average FEV(1), mean trough FVC responses, and weekly mean morning and evening PEFR values were also increased to a greater extent with tiotropium than with ipratropium. In one of the two 6-month trials that compared the efficacy of tiotropium with that of inhaled salmeterol 50 micrograms twice daily, greater improvements from baseline in mean trough, peak, and average FEV(1) and FVC responses were seen with tiotropium than with salmeterol. Increases in weekly mean evening, but not morning, PEFR values were generally greater with tiotropium than salmeterol. In the second trial, improvement in the primary endpoint (mean trough FEV(1) response from baseline) with tiotropium or salmeterol was similar, although peak and average responses were superior with tiotropium. Preliminary results from a 6-week crossover study in patients with COPD suggested that tiotropium alone or in combination with once-daily formoterol improved mean trough and average FEV(1) and trough FVC values from baseline to a greater extent than twice-daily formoterol. More patients achieved a clinically important improvement (increase of &gt;/=1 unit) in the transitional dyspnea index focal score (a measure of dyspnea-related impairment) with tiotropium than with placebo in the 1-year trials. Tiotropium was superior to ipratropium in 1-year trials, and was at least as effective as salmeterol in 6-month trials, in achieving a clinically important improvement in focal scores. Tiotropium recipients experienced fewer COPD exacerbations than placebo or ipratropium recipients and had fewer and shorter COPD-related hospitalizations compared with placebo recipients. Unlike salmeterol, tiotropium lengthened the time to onset of the first exacerbation and decreased the number of exacerbations compared with placebo in two 6-month trials. Similar proportions of tiotropium, salmeterol, and placebo recipients required COPD-related hospitalizations. (ABSTRACT TRUNCATED)"}, {"id": "pubmed23n0501_9858", "title": "Management of acute exacerbations of chronic obstructive pulmonary disease in the elderly: physician practices in the community hospital setting.", "score": 0.009523809523809525, "content": "Chronic obstructive pulmonary disease (COPD) is the fourth leading cause of death and sixth most common reason that Medicare patients are hospitalized. We performed retrospective chart review on a statewide random sample of 409 Medicare patients discharged from October 1, 2000, through January 31, 2001, with a diagnosis of COPD. The most commonly performed diagnostic tests were chest radiography (96.8%) and oxygenation assessment (94.9%). The most common treatments provided were inhaled short-acting beta-agonist bronchodilators (98.5%) and oxygen (94.4%). Antibiotics (89.0%) and systemic corticosteroids (85.1%) were prescribed less frequently. The median length of stay was 5 days. The readmission rate was 27.4% (CI, 23.1-32.0) at 30 days and 43.0% (CI, 38.2-47.9) at 180 days. The in-hospital mortality rate was 1.7% (CI, 0.7-3.5) and the 180-day mortality rate was 23.7% (CI, 19.7-28.1). The morbidity and mortality associated with acute exacerbations of COPD remain high. There are opportunities to improve quality of care for this condition."}, {"id": "pubmed23n0643_19018", "title": "BODE index and GOLD staging as predictors of 1-year exacerbation risk in chronic obstructive pulmonary disease.", "score": 0.009433962264150943, "content": "The body mass index/airflow obstruction/dyspnea/exercise capacity (BODE) index and global initiative for chronic obstructive lung disease (GOLD) staging system are validated measures to define disease severity and to predict survival in chronic obstructive pulmonary disease (COPD). We aimed to investigate the influence of BODE classes (score: 0-2, 3-4, 5-7, and 7-10) and GOLD stages (I, II, III, and IV) on the moderate/severe exacerbation occurrence risk in a cohort of 120 mild/very severe stable patients with COPD. Demographics, clinical evaluation, spirometry, peripheral oxygen saturation, body composition, 6-minute walking distance, dyspnea, and quality of life measurements were obtained at baseline. Patients were followed up for 1 year or until death, and information on exacerbation was collected. The median annual exacerbation rate was 0.8. Logistic regression showed that the relationship between the risk for moderate/severe exacerbations during a 1-year follow-up for the GOLD stage was odds ratio: 2.01; 95% confidence interval: 1.39-2.98 and for the BODE index was odds ratio: 2.08; 95% confidence interval: 1.27-3.61. The area under the receiver-operator curve to predict exacerbation during the 1-year follow-up was 0.69 for the GOLD stage and 0.62 for the BODE index. Adjusted multiple logistic regression selected only older age and lower peripheral oxygen saturation as risk factors for COPD exacerbation in the 1-year follow-up. In summary, our study shows that unidimensional GOLD classification and multidimensional BODE index staging systems seem to have similar clinical utility in predicting exacerbation in ambulatory COPD patients with COPD. However, variables not included in both systems seem to be the main predictors of the exacerbation risk."}, {"id": "pubmed23n0265_16747", "title": "Management of chronic airflow obstruction: differences in practice between respiratory and general physicians.", "score": 0.009433962264150943, "content": "An audit of inpatient care of diseases characterized by chronic airflow obstruction namely chronic bronchitis, emphysema and chronic obstructive airways disease (ICD Code Nos. 490-2 &amp; 496) was performed and the practice of respiratory and general physicians compared. One hundred cases were sampled at random from 279 cases admitted to hospitals serving the West of Glasgow in 1988. Fifty cases were selected from those admitted under the care of respiratory physicians and 50 from those under general physicians; 89 were suitable for analysis. The main outcome measurements consisted of the use of routine respiratory investigations, comparison of the use of standard therapies during the admission and at discharge, length of stay, inpatient deaths, follow up and readmission rates. The groups were similar in age, smoking history, gender and there was no significant difference in admission arterial blood gas values. The pulse rate on admission was higher in the general group (102 beats per min) in comparison to the respiratory group (91 beats per min) (P &lt; 0.004). A similar use of chest radiograph and arterial blood gas analysis was noted between the groups. Ninety-six per cent of respiratory patients had either spirometry or peak expiratory flow measured compared to 62% in the general group (P = 0.0001). No significant differences were noted in the use of antibiotics, bronchodilators, corticosteroids, oxygen or respiratory stimulants. The mean length of stay was similar. Two patients (4%) in the respiratory group compared with seven (18%) in the general group died during the admission (P = 0.01); there were no further early deaths at 1 month from discharge.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en332_33631", "title": "Chronic obstructive pulmonary disease", "score": 0.009401480320804899, "content": "There are many variables affecting the long-term outcome in COPD, and GOLD recommends the use of a composite test (BODE) that includes the main variables of body-mass index, obstruction of airways, dyspnea (breathlessness), and exercise, and not just spirometry results. NICE recommends against the use of BODE for the prognosis assessment in stable COPD; factors such as exacerbations and frailty need to be considered. Other factors that contribute to a poor outcome include older age, comorbidities such as lung cancer and cardiovascular disease, and the number and severity of exacerbations needing hospital admittance."}, {"id": "wiki20220301en274_18983", "title": "BODE index", "score": 0.009345794392523364, "content": "The BODE index, for Body-mass index, airflow Obstruction, Dyspnea, and Exercise, is a multidimensional scoring system and capacity index used to test patients who have been diagnosed with chronic obstructive pulmonary disease (COPD) and to predict long-term outcomes for them. The index uses the four factors to predict risk of death from the disease. The BODE index will result in a score of zero to ten dependent upon FEV1 or \"forced expiratory volume in one second\" (the greatest volume of air that can be breathed out in the first second of a breath), body-mass index, the distance walked in six minutes, and the modified MRC dyspnea scale. Significant weight loss is a bad sign. Results of spirometry are also good predictors of the future progress of the disease, but they are not as good as the test results of the BODE index. References Further reading Chronic lower respiratory diseases"}, {"id": "pubmed23n0238_7182", "title": "[Probability of survival and prognostic factors in chronic obstructive lung disease].", "score": 0.009345794392523364, "content": "The course of chronic obstructive pulmonary disease (COPD) is described over a 3-7 year period in 201 patients treated by family physicians and in the same hospital. The probability of survival after 3 years is 63% (normal population of same age 85%) and after 5 years 48% (normal 75%) for all patients. These figures are comparable with data in the literature of the last 12 years (42-69% 5-year survival). The patients in our study are 68 years old on average and have an FEV1 of 1.3 liters. The prognosis of COPD is significantly influenced by the following factors: ECG signs of cor pulmonale, clinical signs of heart failure, vital capacity, FEV1 and paCO2. For example, within a 3 year period 63 of 124 patients with heart failure died as compared to only 3 of 57 without. Retrospective analysis shows inhalation therapy by motor-driven nebulizers to have a favourable effect on survival. For IPPB home therapy a favourable effect can only be shown after stratification for the two most significant prognostic factors to exclude therapeutic bias. No effect on survival was noted with long-term steroid therapy. 11% of patients who have never smoked, and those who have stopped smoking, live significantly (p less than 0.002) longer than those who continued to smoke more than 3 cigarettes per day during the observation period."}, {"id": "pubmed23n0741_11358", "title": "Chronic obstructive pulmonary disease: prevalence, characteristics, and pharmacologic treatment in nursing home residents with cognitive impairment.", "score": 0.009259259259259259, "content": "Chronic obstructive pulmonary disease (COPD) is prevalent in nursing home residents. National and international guidelines exist for management of COPD; however, little is known about \"real-world\" management of COPD in this population. Nursing home patients with significant cognitive impairment may have difficulty utilizing handheld device (HHD) formulations of respiratory medications and may be clinically appropriate candidates for nebulized therapy. To determine (a) the prevalence, clinical characteristics, and treatment of patients with a diagnosis of \"emphysema/COPD\" per Minimum Data Set (MDS) version 2.0 records in U.S. nursing homes and (b) the relationship of nebulized versus HHD formulations of medication to prevalence of shortness of breath in a cohort of cognitively impaired nursing home residents. In a descriptive, retrospective analysis of a large data repository of skilled nursing home residents with COPD, prescription claims and MDS data from October 1, 2009, through September 30, 2010, were extracted, linked, and de-identified. Measures included medications, diagnoses, and selected outcome parameters from the MDS. Cognitive impairment was defined as a score of 3-6 on the Cognitive Performance Scale derived from MDS records. A proxy of ≤ 14-day courses of respiratory antibiotics, oral corticosteroids, or both was used to identify COPD exacerbations. Shortness of breath (SOB) in the last 7 days was captured from Section J1.l. of the MDS. The total number of unique patients with at least 1 MDS record during the study period was 126,121. Of those, 27,106 (21.5%) had COPD. The prevalence rates of diagnoses concurrent with COPD were as follows: asthma = 8.6%, Alzheimer's disease or other dementia = 37.2%, congestive heart failure = 37.5%, anxiety disorder = 23%, depression = 50.1%, pneumonia = 21.2%, and respiratory infection = 9%. 58% of patients with COPD were white females aged 75 years or older. According to the MDS, 62% of COPD patients had a short-term memory problem, while 43.3% of patients had moderately or severely impaired cognitive skills for daily decision making. 83% of COPD patients with pharmacy claims (17,395/27,106) received at least 1 medication used to treat COPD; 9,711 (17.1%) received no respiratory medications. Use of beta-agonists (53.9%), anticholinergic medications (41.2%), long-acting beta-agonist/corticosteroid (LABA/ICS) combinations (28%) in HHD, and nebulized beta-agonist/anticholinergic combinations (26.6%) was prevalent. Inhaled LABA/ICS and long-acting anticholinergic therapy was received by 28% and 22% residents, respectively. 22% (n = 5,085) of patients exhibited at least 2 exacerbations of COPD, and 33% were noted to have SOB. Monotherapy with short-acting beta-agonists (SABA) was evident in 48.7% of cognitively impaired COPD patients. SOB within the previous 7 days was noted in 39.1% of cognitively impaired COPD patients treated with nebulized SABA monotherapy. 38% of these patients exhibited 2 or more COPD exacerbations, and 57.9% were hospitalized at least once during the 12-month period. LABA monotherapy or combined LABA/SABA use represented ≤ 1% of beta-agonist use for unique COPD patients with cognitive impairment. In this retrospective analysis of administrative data, 21.5% of nursing home residents had a diagnosis of COPD, and 17% of these residents received no respiratory medications. These residents had significant cognitive and functional impairment and concurrent diagnoses. 22% of residents experienced at least 2 exacerbations of COPD during the 12 months of study. As many as 60% were not receiving inhaled LABA/ICS or inhaled tiotropium, and 33% exhibited SOB. There is significant use of nebulized SABA monotherapy, which may be contributing to SOB and exacerbations or hospitalizations in nursing home residents with COPD."}, {"id": "pubmed23n0732_10627", "title": "Hospital management of patients with exacerbation of severe chronic obstructive pulmonary disease.", "score": 0.009259259259259259, "content": "The article assesses the originally developed criteria of clinical stability and treatment protocol in the hospital management and discharge procedures of patients with exacerbations of severe chronic obstructive pulmonary disease (COPD). The study included 34 patients (26 males, 8 females), aged 58-80 years, hospitalized due to exacerbation of severe (23 patients) and very severe (11 patients) COPD. On admission, the mean FEV1 was 0.78 ± 0.22 L (31.7% ± 8.2% of predicted), FVC 2.52 ± 0.87 L (77.9% ± 9.8% of predicted) and FEV1/FVC 33.17% ± 10.84%. Before hospitalization, 10 out of the 34 patients were diagnosed with chronic respiratory failure. All patients were treated according the same treatment protocol which included the developed criteria of clinical stability. Meeting all these criteria in a 24-h observation period was the basis to slash the dose of systemic glucocorticosteroids by half. The maintenance of the stability criteria through the subsequent 24 h allowed discharging a patient from the hospital. Every patient was supplied with a detailed plan of out-of-hospital treatment. The results show that the mean duration of hospitalization was 6.4 ± 4.8 days. Only one patient required readmission within 4 weeks after discharge. Two patients died; one during the hospitalization time and the other after discharge. In the latter case, death was not directly related to the COPD exacerbation. In conclusion, the protocol of treatment and the criteria of stability used for patients with COPD exacerbation enabled to optimize the hospitalization time. A shortening of hospitalization was not associated with increased risk of readmission within 4 weeks after discharge."}, {"id": "wiki20220301en332_33618", "title": "Chronic obstructive pulmonary disease", "score": 0.009216785592932382, "content": "Mucolytics may help to reduce exacerbations in some people with chronic bronchitis; noticed by less hospitalization and less days of disability in one month. Erdosteine is recommended by NICE. GOLD also supports the use of some mucolytics that are advised against when inhaled corticosteroids are being used, and singles out erdosteine as having good effects regardless of corticosteroid use. Erdosteine also has antioxidant properties but there is not enough evidence to support the general use of antioxidants. Erdosteine has been shown to significantly reduce the risk of exacerbations, shorten their duration, and hospital stays. Cough medicines are not recommended. Beta blockers are not contraindicated for those with COPD, and should only be used where there is concomitant cardiovascular disease. Oxygen therapy"}, {"id": "pubmed23n0760_23293", "title": "A comparison of three multidimensional indices of COPD severity as predictors of future exacerbations.", "score": 0.009174311926605505, "content": "Prediction of future exacerbations of chronic obstructive pulmonary disease (COPD) is a major concern for long-term management of this disease. To determine which of three multidimensional assessment systems (the body mass index, obstruction, dyspnea, and exercise capacity [BODE] index; dyspnea, obstruction, smoking, exacerbations [DOSE] index; or age, dyspnea, obstruction [ADO] index) is superior for predicting exacerbations. This was a 2-year prospective cohort study of COPD patients. Pulmonary function tests, the 6-minute walk distance (6MWD), Modified Medical Respiratory Council (MMRC) dyspnea scores, chest computed-tomography measurements, and body composition were analyzed, and predictions of exacerbation by the three assessment systems were compared. Among 183 patients who completed the study, the mean annual exacerbation rate was 0.57 events per patient year, which correlated significantly with lower predicted forced expiratory volume in 1 second (FEV1) (P &lt; 0.001), lower transfer coefficient of the lung for carbon monoxide (%DLco/VA) (P = 0.021), lesser 6MWD (P = 0.016), higher MMRC dyspnea score (P = 0.001), higher DOSE index (P &lt; 0.001), higher BODE index (P = 0.001), higher ADO index (P = 0.001), and greater extent of emphysema (P = 0.002). For prediction of exacerbation, the areas under the curves were larger for the DOSE index than for the BODE and ADO indices (P &lt; 0.001). Adjusted multiple logistic regression identified the DOSE index as a significant predictor of risk of COPD exacerbation. In this study, the DOSE index was a better predictor of exacerbations of COPD when compared with the BODE and ADO indices."}, {"id": "pubmed23n0560_9827", "title": "The role of nebulised budesonide in the treatment of exacerbations of COPD.", "score": 0.009174311926605505, "content": "The present study was designed to evaluate the hypothesis that nebulised budesonide (NB) might be an alternative to systemic corticosteroids (SC) in the treatment of patients with exacerbations of chronic obstructive pulmonary disease (ECOPD). Patients hospitalised with ECOPD (n = 159) were randomised into three groups. Group 1 received only standard bronchodilator treatment (SBDT), group 2 received SC (40 mg prednisolone) plus SBDT, and group 3 received NB (1,500 microg q.i.d.) plus SBDT. Improvement during 10-day hospitalisation was compared with exacerbation and rehospitalisation rates after discharge. While mean+/-sd age was 64.1+/-8.9 yrs (female/male = 0.1), mean forced expiratory volume in one second (FEV(1)) at admission was found to be 37.2+/-12.2% predicted. Arterial blood gases and spirograms recovered faster in groups 2 and 3. While improvements in arterial oxygen tension (P(a,O(2))) and forced vital capacity (FVC) in group 2, and improvements in P(a,O(2)), FVC and FEV(1) in group 3, became significant at 24-h control, the first significant improvement in group 1 appeared in arterial oxygen saturation at 72-h control. The mean improvement of P(a,O(2)) after 10 days was 1.20 and 1.06 kPa (9 and 8 mmHg) higher in group 2 and 3, respectively, than in group 1. Blood glucose exhibited an upward trend only in group 2. The study demonstrates that nebulised budesonide may be an effective and safe alternative to systemic corticosteroids in the treatment of exacerbations of chronic obstructive pulmonary disease."}, {"id": "pubmed23n0755_23957", "title": "Chronic obstructive pulmonary disease--a treatable disease.", "score": 0.00909090909090909, "content": "Chronic obstructive pulmonary disease (COPD) is a global health challenge and a leading cause of death worldwide. Several risk factors have been identified, with cigarette smoking being the most important. Diagnostic assessment is based on symptoms, risk of exacerbations and results of lung function testing. A fixed post-bronchodilator ratio for forced expiratory volume in one second to forced expiratory volume (FEV1/FVC) of &lt;0.7 is required to make the diagnosis, and the severity of airflow obstruction defines the grade according to GOLD (Global Strategy for the Diagnosis, Management, and Prevention of COPD). The GOLD strategy makes therapeutic recommendations taking into account the grade, symptomatic assessment and future risk of exacerbations. This review focuses on the therapeutic options for COPD, in accordance with the GOLD strategy. Smoking cessation is the most effective treatment option in all COPD stages. Bronchodilators, namely long-acting antimuscarinic drugs and long-acting beta-agonists, form the mainstay of treatment in COPD. Patients with frequent exacerbations also benefited from the addition of inhaled corticosteroids. Roflumilast is an add-on option for patients with severe COPD. Several controversies are the subject of discussion: (1.) whether pharmacotherapy can modify the natural history of COPD; (2.) whether pharmacotherapy should be started in the early stages of COPD; (3.) the impact of therapy on comorbidities; (4.) whether patients benefit from a combination therapy with a long-acting beta-agonist, a long-acting antimuscarinic drug and an inhaled corticosteroid; (5.) step-down therapy. This overview also reviews the evidence for recommended vaccines in COPD, as well as nonpharmacological therapies. Rehabilitation is an essential part of COPD treatment. Oxygen therapy, noninvasive nocturnal ventilation and surgical treatment options only apply to a highly selected group of patients. Disease management programmes and guideline adherence are briefly discussed. In conclusion, although there is debate as to the extent with which pharmacological therapies influence mortality, adherence to the GOLD strategy is recommended."}, {"id": "pubmed23n0347_9924", "title": "Oral corticosteroids in patients admitted to hospital with exacerbations of chronic obstructive pulmonary disease: a prospective randomised controlled trial.", "score": 0.00909090909090909, "content": "The role of oral corticosteroids in treating patients with exacerbations of chronic obstructive pulmonary disease (COPD) remains contentious. We assessed in a prospective, randomised, double-blind, placebo-controlled trial the effects of oral corticosteroid therapy in patients with exacerbations of COPD requiring hospital admission. We recruited patients with non-acidotic exacerbations of COPD who were randomly assigned oral prednisolone 30 mg once daily (n=29) or identical placebo (n=27) for 14 days, in addition to standard treatment with nebulised bronchodilators, antibiotics, and oxygen. We did spirometry and recorded symptom scores daily in inpatients. Time to discharge and withdrawals were noted in each group. We recalled patients at 6 weeks to repeat spirometry and collect data on subsequent exacerbations and treatment. Hospital stay was analysed by intention to treat and forced expiratory volume in 1 s (FEV1) according to protocol. FEV1 after bronchodilation increased more rapidly and to a greater extent in the corticosteroid-treated group: percentage predicted FEV1 after bronchodilation rose from 25.7% (95% CI 21.0-30.4) to 32.2% (27.3-27.1) in the placebo group (p&lt;0.0001) compared with 28.2% (23.5-32.9) to 41.5% (35.8-47.2) in the corticosteroid-treated group (p&lt;0.0001). Up to day 5 of hospital stay, FEV1 after bronchodilation increased by 90 mL daily (50.8-129.2) and by 30 mL daily (10.4-49.6) in the placebo group (p=0.039). Hospital stays were shorter in the corticosteroid-treated group. Groups did not differ at 6-week follow-up. These data provide evidence to support the current practice of prescribing low-dose oral corticosteroids to all patients with non-acidotic exacerbations of COPD requiring hospital admission."}, {"id": "wiki20220301en266_12814", "title": "Acute exacerbation of chronic obstructive pulmonary disease", "score": 0.009068627450980391, "content": "The symptoms of acute exacerbations are treated using short-acting bronchodilators. A course of corticosteroids, usually in tablet or intravenous rather than inhaled form, can speed up recovery. The IV and oral forms of steroids have been found to be equivalent. Antibiotics are often used but will only help if the exacerbation is due to an infection. Antibiotics are indicated when a patient notes increased sputum production, purulent sputum, increased dyspnea, has an elevated white count, or is febrile. Examples of first-line antibiotics are amoxicillin, doxycycline, and co-trimoxazole. Mechanical ventilation Severe exacerbations can require hospital care where treatments such as oxygen and mechanical ventilation may be required. Mechanical ventilation can be invasive (endotracheal intubation) or non-invasive forms of ventilation such as continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP)."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[181, 399]], "word_ranges": [[25, 60]], "text": "the most frequent complications are mitral insufficiency, which would present with a musical or piante murmur, and VSD, which presents with a murmur radiating to the right border (think of the direction of blood flow)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Rupture of the interventricular septum. Mechanical complications are typical in infarctions in elderly women. Likewise, a murmur that was not present indicates abnormal blood flow: the most frequent complications are mitral insufficiency, which would present with a musical or piante murmur, and VSD, which presents with a murmur radiating to the right border (think of the direction of blood flow).", "full_answer_no_ref": "Rupture of the interventricular septum. Mechanical complications are typical in infarctions in elderly women. Likewise, a murmur that was not present indicates abnormal blood flow: the most frequent complications are mitral insufficiency, which would present with a musical or piante murmur, and VSD, which presents with a murmur radiating to the right border (think of the direction of blood flow).", "full_question": "An 87-year-old woman with a history of hypertension was admitted 48 hours ago to the coronary unit for acute myocardial infarction with ST-segment elevation of anterior location. She reported dyspnea. Examination revealed a systolic murmur with fremitus, radiating to the right sternal border, which was not present on admission. What complication do you suspect?", "id": 413, "lang": "en", "options": {"1": "Heart failure due to extensive necrosis.", "2": "Anterior aneurysm.", "3": "Left ventricular free wall rupture.", "4": "Rupture in the interventricular septum.", "5": NaN}, "question_id_specific": 68, "type": "CARDIOLOGY AND CARDIOVASCULAR SURGERY", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0122_844", "title": "[Mid-systolic ejection murmur with thrill caused by right ventricular outflow tract obstruction secondary to septal aneurysm following myocardial infarction: a case report].", "score": 0.019232547387887194, "content": "A 71-year-old woman with a history of previous myocardial infarction was transferred to our hospital for evaluation of chest pain and ventricular tachycardia. On admission, a loud mid-systolic ejection murmur accompanied by a thrill was found at the left sternal border in the third intercostal space, and it was significantly accentuated in the post-extrasystolic beat. Abnormal Q waves and ST elevations were noted in leads I, aVL and V5,6 on electrocardiograms. Echocardiograms, confirmed a septal-to-apical aneurysm, and a thin interventricular septum (IVS) with paradoxical motion. Right ventricular (RV) catheterization showed a pressure gradient of 21 mmHg between the outflow tract (RVOT) and the apex, and a mid-systolic ejection murmur was recorded in the RVOT on an intracardiac phonocardiogram. Coronary arteriograms revealed total occlusion of the left anterior descending artery in its proximal portion, and a 90% stenosis of the circumflex artery. A left ventriculogram demonstrated a septal-to-apical aneurysm with a markedly reduced ejection fraction of 0.16. A right ventriculogram showed obstruction to RVOT caused by systolic ballooning of the IVS. In this patient, the mid-systolic ejection murmur was probably caused by the obstruction of the outflow tract secondary to septal aneurysm following old myocardial infarction."}, {"id": "pubmed23n0972_2406", "title": "Left ventricular free wall rupture associated with a combination of acute myocardial infarction and stress-provoked cardiomyopathy: An autopsy case.", "score": 0.017786561264822136, "content": "A 74-year-old female was admitted to our hospital due to prolonged chest pain that had lasted about 2 h. An electrocardiogram revealed ST-elevation in leads I, aVL, and V3-6, with an increase in myocardial necrosis markers. Emergency coronary angiography was performed, and left ventriculography showed the typical features of apical ballooning, and so a diagnosis of Takotsubo cardiomyopathy (TC) was made. On the 10th day after admission, the patient suddenly went into cardiopulmonary arrest because of a blow-out type left ventricular (LV) free wall rupture. Despite extensive cardiopulmonary resuscitation, the patient died. The autopsy revealed hemopericardium and a perforating wound located in the anterior wall of the LV. It was revealed that the diagonal branch of the coronary artery was occluded, and so a diagnosis of TC coexisting with acute myocardial infarction (AMI) was made. No previous case of TC accompanied by AMI has been reported. We present its clinical course during hospitalization and the result of a histopathologic examination."}, {"id": "pubmed23n0889_21873", "title": "Delayed ventricular septal rupture complicating anterior wall myocardial infarction.", "score": 0.017630683575626353, "content": "A 59-year-old woman was admitted to our hospital with acute pulmonary oedema and cardiogenic shock 35 days after anterior ST elevation myocardial infarction. She developed a new loud pan systolic murmur. Echocardiography revealed a ventricular septal rupture with a significant left to right shunt. She was immediately transferred to the local cardiothoracic unit where she underwent a successful ventricular septal defect (VSD) repair. Ventricular septal rupture often presents within the first 24 hours of acute myocardial infarction and is rare thereafter. It carries a poor mortality (41-80%) even when recognised. Timely recognition of this life-threatening complication can help reduce the resultant morbidity and mortality. Doctors should be aware that this well-recognised complication may present unusually late as in this case."}, {"id": "pubmed23n0960_16888", "title": "Exertional dyspnea after myocardial infarction: thinking beyond the diagnosis of heart failure.", "score": 0.017496229260935144, "content": "We herein present an unusual case of a pseudoaneurysm of the left ventricular myocardium, which is a rare and fatal complication of myocardial infarction. A 64-year-old man with a history of bipolar disorder and arterial hypertension was hospitalized for delayed presentation ST-elevation myocardial infarction. He was admitted to our hospital 24 hours after symptom onset. Diagnostic coronary angiography revealed 95% stenosis at the distal third of the right coronary artery, and he underwent a primary percutaneous coronary intervention to the culprit lesion. Despite administration of a diuretic and optimization of other pharmaceutical treatment, his heart failure deteriorated. Electrocardiography showed a sinus rhythm with Q-wave formation in the inferior wall leads (II, III, aVF), T-wave inversion in the same leads, and borderline QT prolongation (QTc of 490 ms). No ST elevation suggestive of left ventricular aneurysm formation was noticed. Forty days later, cardiac ultrasound revealed a dyskinetic cavity (pseudoaneurysm) in continuity with the posterior-inferior wall of the myocardium, resulting in severe mitral valve regurgitation. Unfortunately, the patient died while awaiting surgical treatment. Although most patients with left ventricular pseudoaneurysm have a relatively benign outcome, those with symptoms of heart failure must be urgently diagnosed and treated."}, {"id": "pubmed23n0061_13645", "title": "[Clinical and anatomical features of acute myocardial infarction associated with double rupture of the interventricular septum and ventricular free wall].", "score": 0.017191142191142192, "content": "Four patients with acute myocardial infarction (MI) complicating double rupture; interventricular septum and ventricular free wall ruptures, were studied. All patients had histories of hypertension, and pre-infarction angina pectoris of short duration less than 8 days without previous MI. The sites of infarction were anteroseptal in 2 patients and inferoposterior in the other 2. Only one case was complicated with mild pump failure (Killip class II). Blood pressure was adequately controlled after the onset of MI in all patients. Interventricular septal rupture occurred between 2 and 10 days after the onset of MI. Free wall rupture occurred between 2 and 22 days after MI. Types of free wall ruptures were oozing in 2 patients and blow-out in the other 2. Surgical repair was performed in 2 patients with the oozing type rupture, who however died soon after surgery. The autopsy findings were as follows: 3 patients had left ventricular free wall ruptures and one had right ventricular free wall rupture. One of the patients with left ventricular free wall rupture showed a secondary rupture of a pseudo-ventricular aneurysm. Postmortem coronary angiograms revealed 3 patients with single-vessel disease and one patient with double-vessel disease, indicating that coronary arterial lesions and complicated heart failure were not severe in these 4 patients.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0074_423", "title": "[A successful surgical repair of ventricular septal perforation following acute myocardial infarction in a 83-year-old man].", "score": 0.015756050802779774, "content": "A 83-year-old man, who experienced a sudden severe malacia 13 days before, was admitted, complaining of dyspnea since 8 hours before. A loud systolic murmur of Levine IV/VI was audible on the left sternal border of the 4th intercostal space. The chest X-ray film demonstrated severe pulmonary congestion. The ECG showed abnormal Q waves in II, III, a VF and V1-5. The right heart catheterization revealed an intraventricular shunt from left to right and thus ventricular septal perforation (VSP) 13 days after acute anteroseptal-inferior myocardial infarction was diagnosed. Continuing an aggressive medical treatment with the intraaortic balloon pumping, an emergency operation for VSP was performed 2 days after the onset. A single Teflon patch was sutured on the left side of the septum around VSP (2.5 x 2.5 cm) and the ventricular free wall was closed including the patch with two felt strips. The patient survived through the operation and is doing well at the 11 months of follow-up. Twenty patients above 70 years old have been surgically treated with success for VSP after acute myocardial infarction in Japan. Our patient was the oldest."}, {"id": "pubmed23n1105_1078", "title": "Acute Dyspnea After Inferior-Wall Myocardial Infarction.", "score": 0.015685328185328185, "content": "A 57-year-old woman presented with acute-onset dyspea with a duration of more than 2 days. Four days earlier, she had been thrombolyzed with streptokinase for inferior wall myocardial infarction in a nearby hospital. On examination, we found that the patient had elevated jugular venous pressure and systolic murmur in left lower parasternal region. In addition, there was a ventricular septal rupture in the posterobasal interventricular septum, with at least 2 exit points into the right ventricle. Timely identification of ventricular septal rupture before PCI is of paramount importance, as it has major implications in management of the patient."}, {"id": "pubmed23n0218_2663", "title": "[Echocardiographic manifestations of postinfarction perforation with extensive dissection of the interventricular septum: report of an autopsied case].", "score": 0.015211104684788895, "content": "This is a report of the echocardiographic features of postinfarction perforation associated with dissection of the interventricular septum (IVS) in a 61-year-old woman. She had acute myocardial infarction and was admitted to a nearby hospital, and later admitted to our CCU because hemodynamic deterioration. On admission, she was somnolent and hypotensive (90/64 mmHg), with sinus tachycardia, marked cyanosis, and peripheral edema. On auscultation there were a harsh holosystolic murmur over the LLSB and moist rales in both lung fields. An ECG revealed extensive anterior and inferior infarctions. Catheterization data confirmed O2 step-up in the right ventricle. Two-dimensional echocardiograms demonstrated extensive dissection of the IVS characterized by an echo-free lumen extending from the apex to the cardiac base in the long-axis and partly reaching the left ventricular free wall in the short-axis with the concomitant paradoxical movement of the IVS. Autopsy disclosed marked hemorrhagic infarction and extensive dissection of the IVS forming a lumen (5 X 5.5 X 2 cm3) corresponding exactly to the echocardiographic free space. In addition, a shunt between the right and left ventricles was confirmed by the presence of two perforations near the apex on the right and left sides of the IVS, whose diameters were 6 and 10 mm, respectively."}, {"id": "pubmed23n0497_21555", "title": "Ventricular septal rupture after early successful thrombolytic therapy in acute myocardial infarction: a case report.", "score": 0.015082337249268968, "content": "Ventricular septal defect (VSD) is a severe complication of acute myocardial infarction and has a high mortality rate. This complication appears to have declined in the reperfusion era. It has mostly been reported in elderly or female patients who suffer from anterior wall infarction, patients with multivessel coronary artery disease (CAD) or occluded infarct-related artery (IRA) without collateral circulation, or patients who have had delayed reperfusion therapy. Here, we report the case of a 60-year-old male patient who presented with persistent chest pain and Killip I ST-segment-elevation myocardial infarction. Thrombolytic therapy was started 3 hours after the onset of chest pain. Based on the subsidence of chest pain, resolution of the elevated ST segment, and early peak of cardiac enzymes, reperfusion was thought to be successful. However, on the third day of admission, the patient complained of dyspnea after defecation and was found to have new-onset grade 3 pansystolic murmur over the left sternal border. Cardiac echography showed an apical VSD. A Swan-Ganz catheter was inserted into the right side of the heart; analysis of blood oxygen saturation revealed a 6% step-up of oxygen in the right ventricle. Coronary angiography showed only one-vessel CAD and TIMI 3 flow in the IRA. The patient received intensive medical management and underwent VSD repair and internal mammary artery bypass grafting to the left anterior descending artery. His recovery was uneventful. This case illustrates that VSD can be found in patients receiving early successful reperfusion therapy, with one-vessel CAD, and TIMI 3 flow in the IRA."}, {"id": "pubmed23n1042_8741", "title": "Serious takotsubo cardiomyopathy: an autopsy case presenting severe irreversible myocardial damage after frequent episodes of recurrence.", "score": 0.014984526847277267, "content": "Takotsubo cardiomyopathy is characterized by transient dysfunction of the medial to apical segment of the left ventricle. Recurrence within a few months or years has been reported and serious complications, including arrhythmia, acute cardiac shock and cardiac rupture, can arise; however, recurrence is rare and takotsubo cardiomyopathy is typically a reversible functional disorder. A 91-year-old Japanese woman with a past medical history of angina pectoris, hypertension and uterine carcinoma noted bilateral axillary pain and presented herself to an emergency room. Although the pain improved and she went home, there were several subsequent episodes of recurrent chest pain. At approximately 1 week after the onset, she was hospitalized as her symptom worsened. Electrocardiography showed low voltage in limb and chest leads, and ST-segment elevation in leads II, III, aVF and V3 to V6. Echocardiography revealed medial to apical dyskinesia and basal hypercontractility of the left ventricle, and cardiac tamponade. Pericardiocentesis improved the symptom, but not her cardiac dysfunction. At 3 days after her admission, cardiopulmonary resuscitation was performed due to ventricular fibrillation. She died on the 5th day of admission (2 weeks after the onset). At autopsy, the left ventricle was dilatated and the apical ventricular wall was thin. On microscopy, remarkable wavy change and thinning of myocardium were diffusely observed, especially at the apex and the anterior to lateral wall of the left ventricle, interventricular septum and right ventricle, intermingled with interstitial fibrosis, hemorrhage and neutrophil infiltration. Contraction band necrosis was mainly observed on the posterior to inferior wall of the left ventricle. Our case showed severe morphological myocardial change after several chest pain episodes that were considered to be takotsubo cardiomyopathy. This notable case suggests that the frequent recurrence of serious takotsubo cardiomyopathy is life threatening and can lead to irreversible serious myocardial degeneration."}, {"id": "pubmed23n0988_10005", "title": "Peculiar mechanical complication of myocardial infarction.", "score": 0.014946182588812671, "content": "A 58-year-old man presented to the chest pain unit with crescendo angina over 24 hours and worsening dyspnoea of 10 hours duration. He was a known diabetic and hypertensive on regular treatment for 10 years and a habitual smoker with over 15 pack-years smoking duration. Examination revealed a profusely diaphoretic and dyspnoeic (respiratory rate of 45/min) individual with a blood pressure of 100/60 mm Hg and heart rate of 124 beats/min. He was hypoxic and his oxygen saturation in the ambient air was 64%. His jugular venous pressure was elevated with a prominent V wave. Cardiovascular examination revealed a harsh grade IV/VI systolic murmur over the lower left parasternal border. There were bilateral extensive crepitations heard over the lung fields. ECG on admission revealed presence of Q wave and ST elevation in leads II and III, aVF with ST depression in I and aVL. X-ray chest showed normal cardiac shadow and features of grade III pulmonary venous hypertension. Transthoracic echocardiography is shown in figure 1.Figure 1Transthoracic echocardiogram short axis view at mid cavity level, 2D (A) and colour Doppler (B) image. <bWhat is the most likely diagnosis</b?A. Left ventricular (LV) true aneurysmB. LV pseudoaneurysmC. LV pseudo-pseudoaneurysmD. Ventricular septal rupture (VSR)E. LV free wall rupture."}, {"id": "pubmed23n0054_21038", "title": "[Myocardial dissection in infarction of the right ventricle. Clinical echocardiographic and pathological aspects].", "score": 0.014908976773383553, "content": "Dissection of the inferior wall of the right ventricle during the acute phase of myocardial infarction with right ventricular involvement is a mechanical complication which has been recently identified, the diagnosis being almost exclusively post-mortem. The authors report the clinical, echocardiographic and pathological features of myocardial dissection in four patients. Between 1985 and 1988, the diagnosis of myocardial dissection was made by echocardiography in 4 patients aged 77 to 80 years, admitted to hospital for an acute inferior wall myocardial infarction. All 4 patients had signs of acute right ventricular failure indicating right ventricular necrosis and a loud systolic murmur at the left sternal border; 2 patients were in shock. The ECG showed signs of inferior wall infarction with, in 2 patients, electrical changes suggestive of right ventricular involvement. Echocardiography showed dissection of the inferior wall of the right ventricle as a pulsatile, echo-free space in the diaphragmatic wall of the right ventricle which appeared to obstruct right ventricular ejection in end systole to a variable degree. The outcome was fatal in all cases with death resulting from refractory myocardial failure. Pathological analysis confirmed biventricular inferior wall infarction also involving the posterior part of the interventricular system, the site of a small tear on the left side which communicated with a neo-cavity dissecting the RV posterior wall. The right coronary artery was totally occluded in all cases. The anatomical lesions were fully concordant with the echocardiographic data: the dissection filled with blood from the left ventricle at each systole creating a pulsatile space in the diaphragmatic wall of the ventricle obstructing ejection."}, {"id": "pubmed23n1093_12453", "title": "Complete Dissection of the Interventricular Septum Following Myocardial Infarction.", "score": 0.014776444929116684, "content": "In this report, we present a case of interventricular septal dissection (IVSD) following inferior wall myocardial infarction (MI) in a 64-year-old patient; the patient ultimately recovered after prompt resuscitation and intervention, despite the high mortality associated with these cases. A 64-year-old male with a history of hypertension and obesity was brought to the hospital following an episode of syncope at home. He had been experiencing chest tightness over the past few days prior to the admission. On physical exam, he had a heart rate of 72 beats per minute and blood pressure of 73/52 mmHg. His electrocardiogram revealed ST-segment elevations in leads II, III, and aVF. Emergent coronary angiography revealed 100% occlusion of the right coronary artery (RCA) with no collateral supply and 95% stenosis of the left anterior descending (LAD) artery. Aspiration thrombectomy and balloon angioplasty and subsequent stenting of the RCA were performed. Transthoracic echocardiogram with color Doppler was performed, which confirmed the presence of a defect in the septum. Color Doppler demonstrated a clear jet entering the ventricular septum from the left ventricle (LV), with the jet traversing the entire length of the septum through a dissection and entering into the right ventricle (RV), consistent with complete IVSD. The patient subsequently underwent a successful bovine pericardial patch repair of the ventricular septum. IVSD is a rare anomaly of the IVS. An echocardiogram is a useful tool to establish the diagnosis. The mortality rate after ventricular septal rupture remains high. Fortunately, our patient had interventricular dissection without rupture. Prompt surgical repair remains the choice of treatment for this condition."}, {"id": "pubmed23n0988_76", "title": "Concurrent true inferoposterior left ventricular aneurysm and ventricular septal rupture secondary to inferior myocardial infarction: a case report.", "score": 0.014609545596339816, "content": "Although left ventricular aneurysm (LVA) is the most common mechanical complication of myocardial infarction (MI), it rarely involves the inferior or posterior left ventricular wall. Ventricular septal rupture (VSR) may be a fatal mechanical complication of MI but rarely occurs in the posterior or inferior portion of the interventricular septum. Thus, LVA and VSR as two mechanical complications of MI in the same patient are extremely rare. A 65-year-old woman, who had inferior ST-segment elevation myocardial infarction 2 months before without reperfusion therapy, was admitted with exertional dyspnoea for 1 month. Echocardiography and computed tomography revealed true inferoposterior LVA and VSR as concurrent complications of MI. These imaging findings were confirmed during cardiac surgery. After successful coronary bypass grafting and ventriculoplasty, the patient recovered quickly and was discharged from the hospital. A rare case of post-infarction inferoposterior LVA with concurrent interventricular septal rupture was reported. Transthoracic and transoesophageal echocardiography and cardiac computed tomography were valuable tools for the diagnosis of this rare condition. Combined coronary bypass grafting and ventriculoplasty were effective in treating this often fatal complication of inferior MI."}, {"id": "pubmed23n0296_20486", "title": "[A successful repair of left ventricular rupture after surgical treatment of ventricular septal perforation complicated due to acute myocardial infarction].", "score": 0.014473999520728493, "content": "A 75-year-old woman was admitted complaining of anterior chest pains, and peripheral coldness. The 6th day after admission, a loud systolic murmur of Levine IV/VI was audible at the apex. The chest X-ray film demonstrated a cardiomegaly and ST elevation in the V1-V5 leads. UCG and right-heart catheterization revealed an intraventricular shunt from left to right, and the diagnosis of ventricular septal perforation after acute antero-septal myocardial infarction was confirmed. Although, we were continuing and aggressive medical treatment with the intraaortic balloon pumping, an emergency operation for ventricular septal perforation was performed, 15 days after onset. The direct closure of the septal perforation and the plication of the left ventricular free wall with two felt strips were performed. The postoperative course seemed well. But in the 3rd postoperative day, massive bleeding was recognized through the drainage tube. The left ventricular rupture was suggested, and a massive blood transfusion was done. An emergency operation was performed. Another left ventricular free wall was lacerated about 6 mm at the apex. The laceration was closed with teflon felt strips. The patient survived through the operation, and is doing well after 7 years since the operation."}, {"id": "pubmed23n1028_9567", "title": "[Giant left ventricular false aneurysme revealing a silent myocardial infarction].", "score": 0.014156394453004623, "content": "Left ventricular false aneurysms are rare. They are secondary to a myocardial rupture which is contained by adherent pericardium and scar tissue. LV pseudoaneurysm contains no endocardium or myocardium unlike left ventricular true aneurysm. Most cases of LV pseudoaneurysm are related to acute myocardial infarction in inferior or posterior wall. We report a case of a 56-year-old man with a medical history of chronic cigarette smoking, dyslipidemia, and obesity. The patient had no myocardial infarction before. He was admitted for evaluation of important shortness of breath at effort without chest pain for 5 months. Physical exam find an enlarged left ventricular. The electrocardiogram revealed Q waves and ST segment elevation in leads V1 to V6. Transthoracic echocardiogram showed a large thrombosed apical left ventricular false aneurysm, severe left ventricular dysfunction, which were confirmed by cardiac magnetic resonance imaging, this exam also showed no viability in the mid left anterior descending coronary artery territory. The coronary angiography showed an occlusion of the mid left anterior descending coronary artery and a stenosis of the first diagonal artery. The patient was offered a surgical aneurysectomy with coronary artery bypass. The surgery was successful with amelioration of symptoms. We present a rare case of a giant false left ventricular aneurysm complicating a silent myocardial infarction in the anterior wall. The diagnosis is made by cardiac echocardiogram and cardiac magnetic resonance imaging. Because of the important risk of rupture, the surgical treatment is required."}, {"id": "pubmed23n0215_8125", "title": "[Rupture of the ventricular septum following acute myocardial infarction].", "score": 0.01391418032404165, "content": "Septal rupture (SR) during acute myocardial infarction (AMI) was found in 23 patients of 4 300 consecutive cases of AMI. In 55% the SR was diagnosed clinically by the appearance of a murmur, in 23% by shock and in 22% by heart failure. Eventually every patient developed a pan-systolic murmur. In 82% of the cases the murmur was best heard at the lower left sternal border. In half of the patients the location of the murmur suggested the possibility of papilary muscle rupture or dysfunction. In 68% of cases the rupture occurred during the first week after the onset of AMI. Cardiac catheterization was performed in 17 patients, pulmonary hypertension was found in all of them. Pulmonary blood flow was twice the systemic blood flow (P less than 0.005). Coronary angiograms performed in 6 patients showed three vessel disease in 5 cases. Five patients underwent surgery. Three of them survived. All of the 17 patients who had medical treatment died. Ten within the first week after AMI, 5 within the first month and 2 late deaths. Autopsy was performed in 15 cases. Eighty percent had coronary narrowing in several vessels. SR measured between 5 and 20 millimeters in 78% and was found in the anterior septum in 61%. In the cases who were in shock, the ventricular myocardium was extensively damaged. We conclude that once the diagnosis of SR is established, the patient should undergo surgical closure of the SR and coronary revascularization if necessary. Early surgical indication is particularly important in patients at risk of developing shock."}, {"id": "pubmed23n0882_21093", "title": "A case of recent myocardial infarction with cardiac failure.", "score": 0.013105205678922462, "content": "A 50-year-old hypertensive smoker presented with a typical angina of 2 days duration. An urgent ECG revealed extensive anterior wall myocardial infarction. In view of the delayed presentation, the patient was conservatively managed with heparin. In-hospital echocardiogram showed akinesia of entire left anterior descending artery (LAD) territory with severe left ventricular (LV) dysfunction. He was discharged with a plan for early coronary intervention. However, he presented a fortnight later with acute pulmonary oedema. General appraisal revealed a restless individual who was dyspnoeic and diaphoretic at rest. On clinical examination, the patient was in hypotension with features of biventricular failure. A 12-lead ECG showed QS pattern with persistent ST segment elevation in precordial leads. The chest radiograph demonstrated features of pulmonary oedema, cardiomegaly and bilateral pleural effusion. Creatine Phosphokinase-MB (CPK-MB) was negative. A preliminary transthoracic echocardiography was done (figure 1 and see online supplementary video 1). What is the most likely diagnosis based on the echocardiogram? LV pseudo-aneurysm with contained ruptureDissecting intramural haematoma of LV apexVentricular apical aneurysm with thrombusLV non-compaction with prominent ventricular trabaculations."}, {"id": "pubmed23n0277_4033", "title": "[Papillary muscle rupture complicating with acute myocardial infarction: a case report].", "score": 0.013049151805132667, "content": "Cardiogenic shock caused by papillary muscle rupture in acute myocardial infarction is potentially reversible by surgical treatment. A case of posterior myocardial infarction in a 79-year-old woman is reported. She was admitted to the hospital in cardiogenic shock. She had been suffered from chest pain for three days before admission. On physical examination diffuse rales and a grade 4/6 holosystolic apical murmur were present. The diagnosis of an acute posterior myocardial infarction was based on the electrocardiographic findings and serum creatine kinase level. Coronary angiography visualized subtotal occlusion of the left circumflex coronary artery. Transesophageal echocardiography demonstrated severe mitral regurgitation and the ruptured anterior papillary muscle connected to normal chordae tendineae and posterior mitral leaflet. In systole, the head of the ruptured papillary muscle moved like a whip in the left atrium. At operation, the ruptured papillary muscle was confirmed. Mitral valve replacement with a 27 mm St. Jude Medical prosthesis and coronary artery bypass grafting to the left circumflex coronary artery was performed. Postoperatively she was weaned intraaortic balloon pumping after 4 days and recovered uneventfully."}, {"id": "pubmed23n0016_1269", "title": "[Acute rupture of the interventricular septum in posterior wall infarction (author's transl)].", "score": 0.012924520798536548, "content": "A rupture of the interventricular septum, as described in a case report, is found in 2% of myocardial infarctions. Clinical symptom loud systolic murmur audible at the left sternal border associated with a thrill and with signs of cardiogenic shock. The diagnosis is made by right heart catheterisation which shows a typical oxygen stepup between right atrium and right ventricle, by which the rupture of the interventricular septum can be differentiated from acute papillary muscle rupture. The therapy should be at first hemodynamic stabilisation by drugs and intraaortic balloon pump for 4 to 6 weeks and then closure of the ventricular septal defect by operation."}, {"id": "pubmed23n0754_4961", "title": "Delayed ventricular septal rupture complicating acute inferior wall myocardial infarction.", "score": 0.012901960784313726, "content": "Ventricular septal rupture is a potentially fatal complication of acute myocardial infarction. Its incidence has declined with modern reperfusion therapy. In the era of percutaneous coronary interventions, it occurs a median of 18-24 hours after myocardial infarction and is most commonly associated with anterior myocardial infarction. We present a case of delayed ventricular septal rupture complicating acute inferior wall myocardial infarction. A 53-year-old Caucasian male presented with epigastric pain for three days and electrocardiographic evidence for an acute inferior wall myocardial infarction. Coronary angiography revealed a total occlusion of the proximal right coronary artery. Reperfusion was achieved by balloon angioplasty followed by placement of a bare metal stent. On hospital day six, the patient developed acute respiratory distress, a new loud pansystolic murmur, and hemodynamic instability. Echocardiography revealed the presence of a large defect in the inferobasal interventricular septum with significant left-to-right shunt consistent with ventricular septal rupture. The patient underwent emergent surgical repair with a bovine pericardial patch. Ventricular septal rupture after myocardial infarction should be suspected in the presence of new physical findings and hemodynamic compromise regardless of revascularization therapy."}, {"id": "pubmed23n1153_4310", "title": "Takostubo syndrome combined with ventricular septal perforation: a case report.", "score": 0.012764227642276423, "content": "The precise clinical features and etiologic basis of Takotsubo syndrome remain unclear, although an association with emotional or stressful triggers has been recognized. Ventricular septal perforation is a very rare life-threatening complication. A 77-year-old female patient presented to the hospital with unrelieved chest tightness and shortness of breath. Three months ago, the patient's electrocardiogram revealed ischemic T wave inversion of the anterior wall, along with an increase in myocardial injury markers. There was no evidence of a ventricular septal defect on echocardiography. The patient was admitted to the respiratory department to treat lung lesions. The electrocardiogram demonstrated dynamic changes following admission, and the myocardial markers returned to normal, but the echocardiography revealed a ventricular septal defect. The initial diagnosis was ventricular septal perforation because of myocardial infarction with acute anterior ST-segment elevation. Coronary angiography revealed no abnormalities, but left ventricular angiography revealed an enlarged apex and VSD, with a right ventricular shunt bundle. Later, cardiac MRI revealed an apical ventricular septal defect. Further inquiry of the patient's medical history revealed that her husband died unexpectedly three months ago, and her daughter was seriously injured in a car accident, causing the patient severe emotional distress. Takotsubo syndrome was then determined in conjunction with the patient's medical history, symptoms, signs, and pertinent examinations. Without using a catheter or a surgical procedure, we managed the patient's medical condition. Two weeks later, the patient was discharged with symptoms improved. Takotsubo syndrome is comparable to an acute myocardial infarction on clinical and electrocardiographic examination in the absence of significant coronary disease. Although ventricular septal perforation is most commonly associated with acute myocardial infarction, it can also happen following Takotsubo syndrome. Takotsubo syndrome complicated by ventricular septal perforation is easily misdiagnosed. The early recognition and management of this condition can avoid or reduce morbidity and mortality."}, {"id": "wiki20220301en563_1338", "title": "List of cardiology mnemonics", "score": 0.012159386553471176, "content": "Renal failure Embolism: pulmonary Complications of Myocardial Infarction Darth Vader Death Arrythmia Rupture(free ventricular wall/ ventricular septum/ papillary muscles) Tamponade Heart failure (acute or chronic) Valve disease Aneurysm of Ventricles Dressler's Syndrome thromboEmbolism (mural thrombus) Recurrence/ mitral Regurgitation Coronary artery bypass graft: indications DUST:p. 31 Depressed ventricular function Unstable angina Stenosis of the left main stem Triple vessel disease ECG: left vs. right bundle block WiLLiaM MaRRoW:p. 31 W pattern in V1-V2 and M pattern in V3-V6 is Left bundle block. M pattern in V1-V2 and W in V3-V6 is Right bundle block. Exercise ramp ECG: contraindications RAMP:p. 31 Recent MI Aortic stenosis MI in the last 7 days Pulmonary hypertension Endocarditis FROM JANE: Fever Roth's spots Osler's nodes Murmur of heart Janeway lesions Anemia Nail hemorrhage Emboli Heart valve sequence Try Puling My Aorta:p. 3 Tricuspid"}, {"id": "InternalMed_Harrison_19088", "title": "InternalMed_Harrison", "score": 0.011786786786786787, "content": "Palpation may reveal cardiac enlargement and abnormal contraction of the cardiac impulse (left ventricular dyskinesia). Auscultation can uncover arterial bruits, a third and/or fourth heart sound, and, if acute ischemia or previous infarction has impaired papillary muscle function, an apical systolic murmur due to mitral regurgitation. These auscultatory signs are best appreciated with the patient in the left lateral decubitus position. Aortic stenosis, aortic regurgitation (Chap. 283), pulmonary hypertension (Chap. 304), and hypertrophic cardiomyopathy (Chap. 287) must be excluded, since these disorders may cause angina in the absence of coronary atherosclerosis. Examination during an anginal attack is useful, since ischemia can cause transient left ventricular failure with the appearance of a third 1582 and/or fourth heart sound, a dyskinetic cardiac apex, mitral regurgitation, and even pulmonary edema. Tenderness of the chest wall, localization of the discomfort with a single"}, {"id": "wiki20220301en133_43049", "title": "Myocardial rupture", "score": 0.011427357689039932, "content": "Classification Myocardial ruptures can be classified as one of three types. Type I myocardial rupture is an abrupt slit-like tear that generally occurs within 24 hours of an acute myocardial infarction. Type II is an erosion of the infarcted myocardium, which is suggestive of a slow tear of the dead myocardium. Type II ruptures typically occur more than 24 hours after the infarction occurred. Type III ruptures are characterized by early aneurysm formation and subsequent rupture of the aneurysm. Another method for classifying myocardial ruptures is by the anatomical portion of the heart that has ruptured. By far the most dramatic is rupture of the free wall of the left or right ventricles, as this is associated with immediate hemodynamic collapse and death secondary to acute pericardial tamponade. Rupture of the interventricular septum will cause a ventricular septal defect. Rupture of a papillary muscle will cause acute mitral regurgitation."}, {"id": "InternalMed_Harrison_20985", "title": "InternalMed_Harrison", "score": 0.01132480504687637, "content": "Diagnosis Due to the unstable condition of these patients, supportive therapy must be initiated simultaneously with diagnostic evaluation (Fig. 326-2). A focused history and physical examination should be performed, blood specimens sent to the laboratory, and an electrocardiogram (ECG) and chest x-ray obtained. Etiologies of Cardiogenic Shock or Pulmonary Edema Acute myocardial infarction/ischemia LV failure Ventricular septal rupture Papillary muscle/chordal rupture–severe MR Ventricular free wall rupture with subacute tamponade Other conditions complicating large MIs Post-cardiac arrest Post-cardiotomy Refractory sustained tachyarrhythmias Acute fulminant myocarditis End-stage cardiomyopathy LV apical ballooning Takotsubo’s cardiomyopathy Hypertrophic cardiomyopathy with severe outflow obstruction Aortic dissection with aortic insufficiency or tamponade Severe valvular heart disease Other Etiologies of Cardiogenic Shockb RV failure due to:"}, {"id": "pubmed23n1162_4215", "title": "Ventricular Septal Rupture Following Acute Myocardial Infarction.", "score": 0.011274852315314743, "content": "ST-segment elevation myocardial infarction (STEMI) is a known medical exigency that has seen considerable advances in medical treatment, dramatically boosting survival rates. Post myocardial infarction ventricular rupture is a major serious mechanical complication following myocardial infarction. We present a case of a 68-year-old male admitted to the emergency department with heaviness in the chest, for which electrocardiography was done and it was suggestive of anterior and lateral wall myocardial infarction. After six hours he experienced breathlessness, jugular venous pressure (JVP) was raised, and auscultation revealed early systolic murmur at apex suggestive of ventricular septal rupture. An urgent echocardiogram was done and it confirmed the diagnosis of ventricular septal rupture (VSR). To enhance the prognosis, early identification and appropriate care are required, which necessitate a thorough clinical evaluation that raises the possibility of mechanical problem, as late presentation is one of the major risk factors for developing VSR. VSR can manifest itself in numerous ways, based on the patient's condition. Right clinical judgement and ECG are required to establish a quick diagnosis, as a result, to determine the most appropriate treatment at the appropriate time."}, {"id": "InternalMed_Harrison_19241", "title": "InternalMed_Harrison", "score": 0.011210971804405912, "content": "The precordium is usually quiet, and the apical impulse may be difficult to palpate. In patients with anterior wall infarction, an abnormal systolic pulsation caused by dyskinetic bulging of infarcted myocardium may develop in the periapical area within the first days of the illness and then may resolve. Other physical signs of ventricular dysfunction include fourth and third heart sounds, decreased intensity of the first heart sound, and paradoxical splitting of the second heart sound (Chap. 267). A transient midsystolic or late systolic apical systolic murmur due to dysfunction of the mitral valve apparatus may be present. A pericardial friction rub may be heard in patients with transmural STEMI at some time in the course of the disease, if they are examined frequently. The carotid pulse is often decreased in volume, reflecting reduced stroke volume. Temperature elevations up to 38°C may be observed during the first week after STEMI. The arterial pressure is variable; in most"}, {"id": "pubmed23n0319_5582", "title": "[Myocardial infarct caused by dissection of the anterior interventricular ramus after blunt thoracic trauma--a case report].", "score": 0.0111261372018687, "content": "A 23 year old man, having experienced sudden retrosternal pain, radiating to both hemithoraces, with dyspnea at rest was admitted to another hospital. The physical examination of heart and lung was unremarkable, but the patient showed discrete signs of a respiratory infection. Because of the young age and the history of a respiratory infection, the differential diagnosis perimyocarditis was favored and an appropriate treatment was begun. Five days later the patient was transferred to our center for cardiac catheterization and further treatment. After admission the patient was reported to be hit by a football shortly before the onset of symptoms. The electrocardiogram and chemical values showed the signs of myocardial damage with extensive myocardial necrosis. In the coronary arteriography a dissection of the proximal left anterior descending artery of a length of about 2 cm was seen; in the levocardiogram anterior akinesis was verified. Because of the already completed myocardial infarction, the short distance of the lesion to the left main coronary artery, and the restored flow in the left anterior descending, a non-invasive treatment was preferred. A control coronary arteriography five days later showed solely an irregularity of the vessel wall, the coronary dissection was not further demonstrable."}, {"id": "pubmed23n0280_12034", "title": "Acute myocardial infarction with papillary muscle rupture.", "score": 0.010050251256281407, "content": "The subject of this report is a 57-year-old obese, hypertensive woman who had been well until the onset of severe chest pain and hypotension. She had to be defibrillated four times on her way to the hospital. The diagnosis of acute inferior-posterior infarction was made by electrocardiogram (ECG) and there was a markedly elevated serum creatine kinase (CK) (including the MB fraction). The patient had a very low cardiac output and ejection fraction. A lung scan revealed possible pulmonary embolism for which she was anticoagulated. She remained hypotensive and hypoxemic and, on Day 17 of her hospital stay, she had a bout of severe dyspnea. A new systolic murmur was heard and the clinical diagnosis of ruptured papillary muscle was made and confirmed by echocardiography, and later at autopsy. All three coronary arteries were severely atherosclerotic and, in addition, the right coronary artery was completely closed by a thrombus. This case clearly illustrates the major pathological changes in the heart that correlate with the clinical findings in patients with a myocardial infarct that is complicated by left ventricular papillary muscle rupture. The pathophysiological effects of this condition, as illustrated in this case report, include the following:1. The posterior papillary muscle wa s almost completely separated from its base, with only a thin strip of muscle intact. The mistral valve thus was insufficient (a \"flail valve''); this markedly reduced the ejection fraction of the left ventricle, increased its end-diastolic volume and pressure, produced a damming of blood in the pulmonary circulation, and this resulted in the pulmonary edema seen on the chest x-ray.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0281_3991", "title": "Acute myocardial infarction with ventricular septal rupture.", "score": 0.009900990099009901, "content": "The interventricular septum is one of the three main sites at which the myocardium can rupture. The features of the interventricular septal rupture that occurred in a 72-year-old woman are characteristic of interventricular septal ruptures in general: (1) they occur most commonly in elderly women; (2) the most common site is the mid-portion of an acute, transmural anteroseptal apical infarct; (3) they are also most common during the patient's first heart attack; (4) the clinical diagnosis of acute myocardial infarct is confirmed by both ECG and by serum enzyme levels; (5) the usual time of the rupture is 3-10 days after the onset of the infarction (it occurred after 3 days in our patient); (6) a new cardiac murmur usually is heard and the patient frequently goes into shock; (7) the diagnosis can be confirmed by a step-up in pO2 levels from right atrium to right ventricle; (8) the usual cause is severe old coronary atherosclerosis with a recent thrombotic occlusion as the final precipitating event."}, {"id": "pubmed23n1012_10681", "title": "Peripartum myocardial infarction associated with coronary spasm and acquired protein S deficiency: A case report.", "score": 0.00980392156862745, "content": "Coronary angiography (CAG) findings of acute myocardial infarction (AMI) in pregnant women are characterized by a high incidence of normal coronary arteries. This is the first report of AMI with normal coronary arteries during pregnancy, showing coronary spasm and pregnancy-related acquired protein S (PS) deficiency. A 30-year-old Japanese woman was admitted to an emergency department. One hour before admission, she developed sudden onset of precordial discomfort, back pain, and dyspnea. She was a primigravida at 39 weeks' gestation and had no abnormality in the pregnancy thus far. She had no history of heart disease, diabetes, hypertension, dyslipidemia, deep vein thrombosis (DVT), smoking, or oral contraceptive use and no family history of ischemic heart disease, hemostasis disorder, or DVT. She did not take any medication. Electrocardiography showed ST-segment elevations in leads II, III, aVF, and V2-V6. Heart-type fatty acid-binding protein was positive. Echocardiography showed hypokinesis of the anterior interventricular septum and inferior wall. Continuous intravenous infusion of isosorbide dinitrate was initiated. Coronary computed tomography angiography revealed diffuse narrowing of the apical segment of the left anterior descending coronary artery. Three hours after admission, troponin T became positive, and the following enzymes reached their peak levels: creatine kinase (CK), 1,886 U/L; CK-muscle/brain, 130 U/L. She was diagnosed with transmural AMI due to severe coronary spasm and administered benidipine hydrochloride. Five hours after admission, premature membrane rupture occurred. Emergency cesarean section was performed. There were no anesthetic or obstetrical complications during the operation. On postpartum day 1, the free PS antigen level was low (29%). On postpartum day 18, she was discharged with no reduction in physical performance. Four months after the infarction, CAG showed normal coronary arteries. Acetylcholine provocation test showed diffuse vasospasm in the coronary artery. She was advised that her next pregnancy should be carefully planned. Two years after delivery, free PS antigen level was within normal range, at 86%. She had not experienced recurrence of angina during the 2-year period. Her child was also developing normally. In addition to coronary spasm, pregnancy-related acquired PS deficiency may be involved in AMI etiology."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": true, "char_ranges": [[765, 907]], "word_ranges": [[128, 154]], "text": "in the meantime we would ask for a CT scan (or abdominal MRI) to subsequently assess the plexus block (answers 1 and 2 for future management)."}, "2": {"exist": true, "char_ranges": [[765, 907]], "word_ranges": [[128, 154]], "text": "in the meantime we would ask for a CT scan (or abdominal MRI) to subsequently assess the plexus block (answers 1 and 2 for future management)."}, "3": {"exist": true, "char_ranges": [[700, 760]], "word_ranges": [[116, 127]], "text": "we would not decrease the dose of morphine (false answer 3),"}, "4": {"exist": true, "char_ranges": [[495, 699]], "word_ranges": [[84, 116]], "text": "although opioids for neuropathic pain are not very effective, we will not reduce the dose but will associate the adjuvants. We would manage the patient with gabapentin and corticosteroids (true answer 4);"}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "We have before us a patient with neuropathic pain, which due to the origin of the tumor, is most likely due to involvement of the celiac plexus. It is important to have a radiological diagnosis in view of a possible local treatment, such as neurolytic treatment or plexus blockade. However, we should not leave the patient without a treatment that can alleviate but rather solve the current situation. He is taking WHO step 3 analgesia, which is clearly insufficient, so he will need adjuvants; although opioids for neuropathic pain are not very effective, we will not reduce the dose but will associate the adjuvants. We would manage the patient with gabapentin and corticosteroids (true answer 4); we would not decrease the dose of morphine (false answer 3), and in the meantime we would ask for a CT scan (or abdominal MRI) to subsequently assess the plexus block (answers 1 and 2 for future management).", "full_answer_no_ref": "We have before us a patient with neuropathic pain, which due to the origin of the tumor, is most likely due to involvement of the celiac plexus. It is important to have a radiological diagnosis in view of a possible local treatment, such as neurolytic treatment or plexus blockade. However, we should not leave the patient without a treatment that can alleviate but rather solve the current situation. He is taking WHO step 3 analgesia, which is clearly insufficient, so he will need adjuvants; although opioids for neuropathic pain are not very effective, we will not reduce the dose but will associate the adjuvants. We would manage the patient with gabapentin and corticosteroids ([HIDDEN]); we would not decrease the dose of morphine ([HIDDEN]), and in the meantime we would ask for a CT scan (or abdominal MRI) to subsequently assess the plexus block ([HIDDEN]).", "full_question": "A 66-year-old patient diagnosed with stage IV pancreatic adenocarcinoma 8 months ago. He follows treatment with delayed release morphine 200 mg/12 hours orally, kerosene and lactulose. For the last 15 days he has reported paresthesias and occasional lancinating pain in the right lumbar and periumbilical area that does not subside with rescue morphine. The neurological examination is normal. Indicate the most appropriate management:", "id": 314, "lang": "en", "options": {"1": "Perform computed axial tomography and evaluate nerve compression since it is neuropathic pain.", "2": "Evaluate neurolytic treatment since neuropathic pain is difficult to control.", "3": "Decrease the dose of morphine as it is ineffective in this type of pain.", "4": "Administer amitriptyline or gabapentin, dexamethasone and increase the dose of morphine.", "5": NaN}, "question_id_specific": 178, "type": "ONCOLOGY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en046_39133", "title": "Neuralgia", "score": 0.013010540184453228, "content": "Treatment Treatment options include medicines and surgery. Neuralgia is more difficult to treat than other types of pain because it does not respond well to normal pain medications. Special medications have become more specific to neuralgia and typically fall under the category of membrane stabilizing drugs or antidepressants such as Cymbalta. The antiepileptic medication(AED) Lyrica (pregabalin) was developed specifically for neuralgia and other neuropathic pain as a successor to Neurontin (gabapentin). High doses of anticonvulsant medicines—used to block nerve firing— and tricyclic antidepressants are generally effective in treating neuralgia. If medication fails to relieve pain or produces intolerable side effects, surgical treatment may be recommended."}, {"id": "pubmed23n0907_3220", "title": "Morphine for chronic neuropathic pain in adults.", "score": 0.012507603406326034, "content": "Neuropathic pain, which is caused by a lesion or disease affecting the somatosensory system, may be central or peripheral in origin. Neuropathic pain often includes symptoms such as burning or shooting sensations, abnormal sensitivity to normally painless stimuli, or an increased sensitivity to normally painful stimuli. Neuropathic pain is a common symptom in many diseases of the nervous system. Opioid drugs, including morphine, are commonly used to treat neuropathic pain. Most reviews have examined all opioids together. This review sought evidence specifically for morphine; other opioids are considered in separate reviews. To assess the analgesic efficacy and adverse events of morphine for chronic neuropathic pain in adults. We searched the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, and Embase for randomised controlled trials from inception to February 2017. We also searched the reference lists of retrieved studies and reviews, and online clinical trial registries. We included randomised, double-blind trials of two weeks' duration or longer, comparing morphine (any route of administration) with placebo or another active treatment for neuropathic pain, with participant-reported pain assessment. Two review authors independently extracted data and assessed trial quality and potential bias. Primary outcomes were participants with substantial pain relief (at least 50% pain relief over baseline or very much improved on Patient Global Impression of Change scale (PGIC)), or moderate pain relief (at least 30% pain relief over baseline or much or very much improved on PGIC). Where pooled analysis was possible, we used dichotomous data to calculate risk ratio (RR) and number needed to treat for an additional beneficial outcome (NNT) or harmful outcome (NNH). We assessed the quality of the evidence using GRADE and created 'Summary of findings' tables. We identified five randomised, double-blind, cross-over studies with treatment periods of four to seven weeks, involving 236 participants in suitably characterised neuropathic pain; 152 (64%) participants completed all treatment periods. Oral morphine was titrated to maximum daily doses of 90 mg to 180 mg or the maximum tolerated dose, and then maintained for the remainder of the study. Participants had experienced moderate or severe neuropathic pain for at least three months. Included studies involved people with painful diabetic neuropathy, chemotherapy-induced peripheral neuropathy, postherpetic neuralgia criteria, phantom limb or postamputation pain, and lumbar radiculopathy. Exclusions were typically people with other significant comorbidity or pain from other causes.Overall, we judged the studies to be at low risk of bias, but there were concerns over small study size and the imputation method used for participants who withdrew from the studies, both of which could lead to overestimation of treatment benefits and underestimation of harm.There was insufficient or no evidence for the primary outcomes of interest for efficacy or harm. Four studies reported an approximation of moderate pain improvement (any pain-related outcome indicating some improvement) comparing morphine with placebo in different types of neuropathic pain. We pooled these data in an exploratory analysis. Moderate improvement was experienced by 63% (87/138) of participants with morphine and 36% (45/125) with placebo; the risk difference (RD) was 0.27 (95% confidence interval (CI) 0.16 to 0.38, fixed-effects analysis) and the NNT 3.7 (2.6 to 6.5). We assessed the quality of the evidence as very low because of the small number of events; available information did not provide a reliable indication of the likely effect, and the likelihood that the effect will be substantially different was very high. A similar exploratory analysis for substantial pain relief on three studies (177 participants) showed no difference between morphine and placebo.All-cause withdrawals in four studies occurred in 16% (24/152) of participants with morphine and 12% (16/137) with placebo. The RD was 0.04 (-0.04 to 0.12, random-effects analysis). Adverse events were inconsistently reported, more common with morphine than with placebo, and typical of opioids. There were two serious adverse events, one with morphine, and one with a combination of morphine and nortriptyline. No deaths were reported. These outcomes were assessed as very low quality because of the limited number of participants and events. There was insufficient evidence to support or refute the suggestion that morphine has any efficacy in any neuropathic pain condition."}, {"id": "wiki20220301en014_36780", "title": "Trigeminal neuralgia", "score": 0.012336182336182337, "content": "Medical The anticonvulsant carbamazepine is the first line treatment; second line medications include baclofen, lamotrigine, oxcarbazepine, phenytoin, gabapentin and pregabalin. Uncontrolled trials have suggested that clonazepam and lidocaine may be effective. Antidepressant medications, such as amitriptyline have shown good efficacy in treating trigeminal neuralgia, especially if combined with an anti-convulsant drug such as pregabalin. There is some evidence that duloxetine can also be used in some cases of neuropathic pain, especially in patients with major depressive disorder as it is an antidepressant. However, it should, by no means, be considered a first line therapy and should only be tried by specialist advice. There is controversy around opiate use such as morphine and oxycodone for treatment of TN, with varying evidence on its effectiveness for neuropathic pain. Generally, opioids are considered ineffective against TN and thus should not be prescribed."}, {"id": "pubmed23n0700_575", "title": "A comparative efficacy of amitriptyline, gabapentin, and pregabalin in neuropathic cancer pain: a prospective randomized double-blind placebo-controlled study.", "score": 0.009900990099009901, "content": "Neuropathic pain is difficult to diagnose and difficult to treat with certainty. So the aim of the study was to evaluate comparative clinical efficacy of pregabaline with amitriptyline and gabapentin in neuropathic cancer pain. A total of 120 patients with cancer having severe neuropathic cancer pain were enrolled in the study after taking approval from Institutional Ethics Committee and divided in to 4 groups: group AT-amitriptyline, group GB-gabapentin, group PG-pregabalin, and group PL-placebo. Oral morphine was used for rescue analgesic for continued pain. Pain score (Visual Analogue scale) and secondary outcome measures such as intensity of lancinating, dysesthesia, and burning on numerical rating scale, Global satisfaction score (GSS), Eastern Co-operative Oncology Group scoring (ECOG), and adverse effects were assessed. At the end of study there was significant decrease in pain score in group PG as compared to the other groups; group AT (P = .003), group GB (P = .042), and group PL (P = .024). Percentage of patients with lancinating pain and dysesthesia were significantly less in group PG as compared to groups GB and PL. All the patients in group PL needed rescue morphine. After 4 visits, maximum improvement in ECOG scoring and GSS scoring was observed in group PG patients. Our results suggested that all antineuropathic drugs are effective in relieving cancer-related neuropathic pain. There was statistically and clinically significant morphine sparing effect of pregabaline in relieving neuropathic cancer pain and neuropathic symptoms as compared to other antineuropathic drugs."}, {"id": "pubmed23n0786_14196", "title": "[Epidural injection shows no advantages over oral medication and physiotherapy in the treatment of sciatica, irrespective of the duration of symptoms].", "score": 0.009900990099009901, "content": "The study presented here investigated the short-term effectiveness of one-off lumbar caudal epidural injection (EI) in sciatica in relationship to the reported duration of pain. This retrospective analysis involved 106 consecutive in-patients who received either conservative treatment (Group I) or an additional EI on the first day of their treatment (Group II). Both groups were divided according to the duration of symptoms at the time of admission (less than three months, or more than six months). Propensity score matching was performed for the whole collective and the resulting subgroups. This incorporated gender, age and pain intensity at the time of admission. The target parameter were changes on a visual analogue scale (VAS) of pain intensity on days D1, D3, and D10 depending on the respective treatment. A routine evaluation of the mental variables anxiety, depression and somatisation was performed as part of the examination upon admission and their relationship to the success of treatment was later assessed. The mean age of the patients was 61.7 (± 11.6) in Group I and 63.6 (± 13.6) in Group II. 59 % of the patients were female (n = 63). The Lasègue sign was prevalent in 45 % of Group I and 51 % of Group II. The intensity of pain on the day of admission was similar in both groups (7.0 ± 1.0 for Group I, 6.7 ± 1.8 for Group II). The length of stay on the ward was also similar in both groups (10.2 ± 3.9 and 9.4 ± 3.7 d, respectively). It was found that, independent of the duration of symptoms, injection treatment was significantly more effective than conservative treatment only in the early stages (D1 and D3, p &lt; 0.001). No differences could be found in the expression of these mental variables between treatment groups, as these factors showed no influence on the results of therapy. In the context of acute treatment a once only lumbar caudal epidural injection represents at most a short-term effectiveness for the therapy of sciatica. The results presented here indicate that neither the duration of symptoms nor the measured psychometric variables show any effect on the success of therapy."}, {"id": "pubmed23n0975_21660", "title": "Gabapentin as add-on to morphine for severe neuropathic or mixed pain in children from age 3 months to 18 years - evaluation of the safety, pharmacokinetics, and efficacy of a new gabapentin liquid formulation: study protocol for a randomized controlled trial.", "score": 0.00980392156862745, "content": "Gabapentin has shown efficacy in the treatment of chronic neuropathic or mixed pain in adults. Although pediatric pain specialists have extensive experience with gabapentin for the treatment of neuropathic pain, its use is off-label. Its efficacy and safety in this context have never been shown. The aim of this trial is to compare gabapentin with placebo as add-on to morphine for the treatment of severe chronic mixed or neuropathic pain in children. This trial is part of the European Union Seventh Framework Programme project Gabapentin in Paediatric Pain (GAPP) to develop a pediatric use marketing authorization for a new gabapentin suspension. The GAPP-2 study is a randomized, double-blind, placebo-controlled, multicenter superiority phase II study in children with severe chronic neuropathic or mixed pain. Its primary objective is to evaluate the efficacy of a gabapentin liquid formulation as adjunctive therapy to morphine. Sixty-six eligible children 3 months to 18 years of age with severe pain (pain scores ≥ 7), stratified in three age groups, will be randomized to receive gabapentin (to an accumulating dose of 45 to 63 mg/kg/day, dependent on age) or placebo, both in addition to morphine, for 12 weeks. Randomization will be preceded by a short washout period, and treatment will be initiated by a titration period of 3 weeks. After the treatment period, medication will be tapered during 4 weeks. The primary endpoint is the average pain scores in the two treatment groups (average of two measures each day for 3 days before the end-of-study visit [V10] assessed by age-appropriate pain scales (Face, Legs, Activity, Cry, Consolability scale; Faces Pain Scale-Revised; Numeric Rating Scale). Secondary outcomes include percentage responders to treatment (subjects with 30% reduction in pain scale), number of episodes of breakthrough pain, number of rescue interventions, number of pain-free days, participant dropouts, quality of life (Pediatric Quality of Life Inventory), and acceptability of treatment. Outcomes will be measured at the end-of-study visit after 12 weeks of treatment at the optimal gabapentin dose. Groups will be compared on an intention-to-treat basis. We hope to provide evidence that the combination of morphine and gabapentin will provide better analgesia than morphine alone and will be safe. We also aim to obtain confirmation of the recommended pediatric dose. EudractCT, 2014-004897-40 . Registered on 7 September 2017. ClinicalTrials.gov, NCT03275012 . Registered on 7 September 2017."}, {"id": "pubmed23n0527_20983", "title": "[Effectiveness and time to onset of pregabalin in patients with neuropathic pain].", "score": 0.00980392156862745, "content": "The data from a previously published 12-week randomised, double-blind, placebo-controlled multicentre study on the efficacy and safety of pregabalin were analyzed for time to onset of analgesic action with neuropathic pain. A total of 338 patients with postherpetic neuralgia or painful diabetic peripheral neuropathy were treated with flexible or fixed regimens of pregabalin at daily doses of up to 600 mg/day (n=141 and 132, respectively) or placebo (n=65). Under fixed dose treatment, a decrease of one full point on the 11-point numerical rating pain scale was reached on day 1, two full points on day 13, and three full points on day 23 (under flexible dose pregabalin: on days 6, 17 and 30). In both treatment arms, pain reduction was statistically significant (P=0.001, P=0.002 vs placebo, respectively). In patients with chronic neuropathic pain, the analgesic effect of both pregabalin treatment regimens was high and associated with a rapid time to onset."}, {"id": "wiki20220301en003_196343", "title": "Porphyria", "score": 0.009708737864077669, "content": "Neurologic and psychiatric disorders Patients who experience frequent attacks can develop chronic neuropathic pain in extremities as well as chronic pain in the abdomen. Intestinal pseudo-obstruction, ileus, intussusception, hypoganglionosis, and encopresis in children have been associated with porphyrias. This is thought to be due to axonal nerve deterioration in affected areas of the nervous system and vagal nerve dysfunction. Pain treatment with long-acting opioids, such as morphine, is often indicated, and, in cases where seizure or neuropathy is present, gabapentin is known to improve outcome."}, {"id": "pubmed23n0597_2596", "title": "[The influence of dexamethasone on pain after lumbar disc surgery. A double-blind study.].", "score": 0.009708737864077669, "content": "Patients operated because of lumbar disc herniations (104 patients) were included in a randomized double-blind study analyzing the influence of dexamethasone versus placebo on postoperative drug requirements and the pain score on the visual analogue scale. High doses of dexamethasone had been administered: 40 mg i.v. on the night before the operation; 8 mg intraoperatively topical perineural application; 8 mg i.v. in the evening of the day of operation; 2x8 mg i.m. on days 1 and 2 postoperatively; 2x4 mg i.m. on days 3 and 4; 4 mg po on day 5 and 6 postoperatively. A significant decrease in the requirement for analgesics was found in the drug-treated group, particularly male patients, and also an impressive reduction in the lumbar pain score. In conclusion, there was good alleviation of sciatic pain in the dexamethasone-treated group of females during the 1st week after operation, but we found no evidence that the agent tested had an influence on the clinical outcome 1 month following the operation."}, {"id": "wiki20220301en001_2684", "title": "Morphine", "score": 0.009615384615384616, "content": "Extended-release There are extended-release formulations of orally administered morphine whose effect last longer, which can be given once per day. Brand names for this formulation of morphine include Avinza, Kadian, MS Contin and Dolcontin. For constant pain, the relieving effect of extended-release morphine given once (for Kadian) or twice (for MS Contin) every 24 hours is roughly the same as multiple administrations of immediate release (or \"regular\") morphine. Extended-release morphine can be administered together with \"rescue doses\" of immediate-release morphine as needed in case of breakthrough pain, each generally consisting of 5% to 15% of the 24-hour extended-release dosage."}, {"id": "pubmed23n0885_1699", "title": "Management of postoperative pain after Lumbar surgery-pregabalin for one day and 14 days-a randomized, triple-blinded, placebo-controlled study.", "score": 0.009615384615384616, "content": "Despite the progress in understanding acute pain physiology during recent decade, eighty percent of patients still suffer from post-operative discomfort. Pregabalin is an anticonvulsant agent that is approved for painful neuropathies in diabetic patients and post herpetic neuralgia. The main objective of the present study was to compare the improvement in post-operative pain management and patient lifestyle in 3 groups, as first group received placebo, second who received Pregabalin for one day and the last group those who received it for 14 days. This was a prospective single center, randomized, triple-blind, 3-arm, parallel group study. In this triple-blind study, patients were randomized to 1 of 3 groups using computer-generated random number table. 1) The first group received placebo for 14 days, the second group received Pregabalin 300mg 8h preoperatively and 150mg 12 and 24h postoperatively and for the rest of 13days received placebo and the third group received Pregabalin 300mg eight hours preoperatively and 15mg every 12h postoperatively for 14 days. Name, age, gender, height, weight, education, duration of pain, past medical history, drug history,total morphine requirement at the time of discharge and MRI findings of all the patients were recorded, also they Numerical scale system (NRS) and Oswestry low back pain disability index (ODI) questionnaire were completed for them. All the patients were operated based on standard surgery techniques, bilateral foramenotomy and interlaminar discectomy. Of the 105 patients who entered the run-in period, 47 patients (44.8%) were female and 58 (55.2%) were male. The Patients radicular pain mean score based on NRS estimated before surgery was 7.22±1.95 in pregabalin14, 7.71±1.84 in pregabalin1 and 7.45±1.9 in control group. There were no statically significant differences between three groups (P-Value&gt;0.05). The Patients back pain mean score based on NRS was 5.2±2.87 in pregabalin14, 5.11±3.23 in pregabalin1 and 6.4±3.06 in control group. This means that there were no significant differences in the overall score among those three groups (P-Value&gt;0.05). In comparison to their preoperative pain, the average radicular pain in each group of patients improved significantly 4, 8, 12 and 24h after the operation (P-Value&lt;0.001), but there were no significant differences in radicular pain improvements comparing three groups. The results of this study indicate that 1day and 2 weeks post-operative 300mg pregabalin administration may not improve acute pain, morphine consumption and quality of life of patients after surgery. It seems that the diseases cause chronic pain that requires long-term treatment with higher doses."}, {"id": "wiki20220301en012_71939", "title": "Tramadol", "score": 0.009523809523809525, "content": "Its analgesic effects take about one hour to come into effect and 2 to 4 h to peak after oral administration with an immediate-release formulation. On a dose-by-dose basis, tramadol has about one-tenth the potency of morphine (thus 100 mg is commensurate with 10 mg morphine but may vary) and is practically equally potent when compared with pethidine and codeine. For pain moderate in severity, its effectiveness is equivalent to that of codeine at low doses, and hydrocodone at very high doses; for severe pain it is less effective than morphine. These painkilling effects last about 6 h. The potency of analgesia varies considerably as it depends on an individual's genetics. People with specific variants of CYP2D6 enzymes may not produce adequate amounts of the active metabolite (desmetramadol) for effective pain control."}, {"id": "pubmed23n1124_3404", "title": "[Treatment of sciatica by lumbar nerve root canal injection under X-ray angiography].", "score": 0.009523809523809525, "content": "To investigate the short-term clinical effect of lumbar nerve root canal injection under X-ray angiography in the treatment of sciatica. The clincal data of 78 patients with sciatica underwent lumbar nerve root canal injection under X-ray angiography from December 2017 to February 2020 was retrospectively analyzed. Including 31 males and 47 females, aged from 22 to 88 years old with a median of 65 years. There were 55 cases of lumbar disc herniation and 23 cases of lumbar spinal stenosis, the course of disease ranged from 1 to 8 weeks with a median of 3 weeks. There were 71 cases of single segment disc herniation or stenosis, including L<sub3,4</sub of 5 cases, L<sub4,5</sub of 61 cases, L<sub5</subS<sub1</sub of 5 cases, and 7 cases of multisegment herniation or stenosis. The pain visual analogue scale (VAS) was recorded and Macnab was used to evaluate the clinical effect. All patients completed standardized treatment without serious adverse reactions. VAS were (3.21±0.76) scores immediately after treatment, (2.89±0.33) scores 1 hour after treatment, (1.80±0.27) scores 6 hours after treatment, (1.10±0.20) scores 24 hours after treatment, (2.53±0.35) scores 1 week after treatment and (4.27±0.36) scores 1 month after treatment. There were significant differences in VAS between before treatment(7.83±0.56) and each time period after treatment(<iP</i&lt;0.05). According to Macnab low back pain evaluation standard, 42 cases were effective, 34 cases were markedly effective and 2 cases were ineffective within 24 hours after treatment, with an effective rate of 97.4%;38 cases were effective, 25 cases were markedly effective, 15 cases were ineffective within one week after treatment, the effective rate was 80.0%;32 cases were effective, 22 cases were markedly effective, 24 cases were ineffective within one month after treatment, the effective rate was 69.2%. The short-term clinical effect of nerve root canal injection under X-ray radiography in the treatment of sciatica is good and it is an effective method to relieve sciatica."}, {"id": "pubmed23n0402_12432", "title": "Amitriptyline in neuropathic cancer pain in patients on morphine therapy: a randomized placebo-controlled, double-blind crossover study.", "score": 0.009433962264150943, "content": "Amitriptyline is the most common analgesic adjuvant used in cancer patients with neuropathic pain, even though no specific studies have demonstrated a benefit. A randomized placebo-controlled, double-blind crossover study was designed to evidence the effects of amitriptyline in patients with neuropathic cancer pain. Sixteen advanced cancer patients with neuropathic pain on systemic morphine therapy, no longer receiving oncologic treatment, presenting moderate pain (about 4 or more, but less than 7, on a numerical scale of 0-10) in the last week, and given a stable morphine dose in the last 2 days were admitted to the study. During the first week of study, patients were administered 25 mg of amitriptyline or equivalent drops of placebo at night for 3 days and 50 mg for the following 4 days. Doses for patients aged more than 65 years were 15 mg (first 3 days) and 30 mg (3 days after). After a week, a crossover took place for the second week, with the other treatment at an inverse sequence. Opioid consumption, pain intensity, symptoms and adverse effects, mood, sleep, patient's preference, quality of life before starting the study, the first week after and the second week after were recorded. No significant benefits in analgesia were found in the global pain intensity of the previous week of treatment, the least pain intensity or the pain evaluated just after a week of treatment, at the moment of the visit, when amitriptyline was compared with placebo. A significant difference was evidenced for the worst pain (P &lt; 0.035). No differences in opioid doses during the period of study were found. Drowsiness, confusion and dry mouth were significantly more intense with amitriptyline than with placebo (P &lt; 0.036, 0.003, and 0.034, respectively). There were no substantial differences between the two treatments in Spitzer's quality of life score and for each item. No differences in patients' preference for the two treatment periods were found. The analgesic effects of amitriptyline were slight and associated with adverse effects. In light of the results obtained in the study, the extensive use of the drug for cancer pain should be questioned."}, {"id": "pubmed23n0648_20017", "title": "[Pharmacological treatment of neuropathic pain].", "score": 0.009433962264150943, "content": "Neuropathic pain (NP), in view of its non-nociceptive component, is not caused by physiological lesions but by problems in the nervous system itself, whether in the central nervous system (CNS) or peripheral nervous system (PNS). This particular action mechanism makes NP a very difficult-to-treat condition, resistant to most of the commonly used analgesic drugs. A recent study stated that NP has an incidence of 1.24% over the general population, and this percentage increases if we consider acute radiculopathies and some recurrent neuropathies, frequently considered not only neuropathic pain but also nociceptive. Thus, the improvement of NP treatment has become a public health necessity. While WHO recommendations include a three-lined scale in pain treatment -including NSAIDs as the first-line drugs, soft opioids (tramadol or codein) as the second-line, and strong opioids (morphine, oxycodone, and phentanyl) as the third-line- some studies have found this rationale not useful in NP treatment. Based on several studies as STEP, Spanish Pain Society recommendations included antidepressant and anticonvulsant drugs as the first line treatment. Pregabalin, a new neuromodulators class drug, provides a pharmacokinetic profile than its predecessors (phenytoin, carbamazepine, gabapentin, topiramate, oxcarbazepine, and lamotrigine), and showed effectiveness controlling peripheral neuropathic pain. Thus, pregabalin opened the door to a new approach to NP. Other pain societies, such as the Canada Pain Society, have also included pregabalin in the first line treatment of NP. In fact, gabapentin and pregabalin are the current standard care in most of NP-associated diseases."}, {"id": "wiki20220301en058_5897", "title": "Extended-release morphine", "score": 0.009345794392523364, "content": "Dosage comparison For constant pain, the relieving effect of extended-release morphine given once (for Kadian) or twice (for MS Contin) every 24 hours is roughly the same as multiple administrations of immediate release (or \"regular\") morphine. Morphine sulfate pentahydrade (trade names including Dolcontin) has a higher molecular mass than morphine base, and therefore 10 mg morphine sulfate pentahydrate contains approximatively 7.5 mg of morphine free base. Extended-release morphine can be administered together with \"rescue doses\" of immediate-release morphine pro re nata in case of breakthrough pain, each generally consisting of 5% to 15% of the 24-hour extended-release dosage."}, {"id": "pubmed23n0934_19011", "title": "Efficacy and Safety of Lidocaine Infusion Treatment for Neuropathic Pain: A Randomized, Double-Blind, and Placebo-Controlled Study.", "score": 0.009345794392523364, "content": "Lidocaine infusion therapy (LIT) is an effective treatment for relieving neuropathic pain (NeP). However, it remains unclear whether pain relief can be sustained through repeated lidocaine infusions. This study aimed to determine whether repeated intravenous administration of low-dose lidocaine could provide prolonged pain relief in patients with specific NeP conditions. This is a prospective, randomized, double-blind, placebo-controlled, parallel study. We compared the efficacy and safety of lidocaine infusions (3 mg/kg) in the LIT group and normal saline infusions in the control group once a week for 4 consecutive weeks in patients with postherpetic neuralgia or complex regional pain syndrome type II. The primary outcome was the difference in the percentage change in the 11-point numerical rating scale (NRS) pain score from baseline to after the final infusion. Secondary outcomes included pain scores during 4 weeks of follow-up and any complications. Forty-two patients completed this study protocol. The percentage reduction in NRS pain scores after the final infusion was significantly greater in the LIT group compared with the control group (P = 0.011). However, this pain reduction was not detectable at the 4-week follow-up. The difference in the percentage change in NRS pain scores was especially prominent in the LIT group after the third and fourth infusions. None of the study participants experienced serious complications from the treatment. Lidocaine infusion therapy (3 mg/kg of lidocaine administered over 1 hour) provided effective short-term pain relief, which was substantially prominent after repeated infusions were administered to patients with refractory NeP. This study was registered at ClinicalTrials.gov, identifier NCT02597257."}, {"id": "pubmed23n0210_13666", "title": "[Non-steroidal anti-inflammatory agents. Evaluation criteria in discal sciatic neuralgia].", "score": 0.009259259259259259, "content": "Few controlled trials have been carried out with non-steroidal antiinflammatory drugs in this indication, mainly as a result of methodological difficulties. A prerequisite for correct evaluation is compliance with certain fundamental principles. The study must be controlled (i.e. comparative, prospective randomized, double-blind, with data quality control and statistical analysis). The sample size must be consistent with the objectives (at least 40 patients per group). The selection criteria must be well specified and the comparability of baseline data assessed. The most appropriate study design for assessing clinical efficacy is to compare two parallel groups of out patients treated during five to fifteen days. The main criterion of efficacy should be based on the judgments of both patients and physician. Time course of pain, improvement in the patient's functional capacity, improvement in physical findings (Schober, Lasègue), dosage of concomitant analgesic treatments when these are allowed, and evaluation at three and six months can also be used as efficacy criteria. Assessment of clinical and biological tolerance is mandatory. Considering the frequent use of non-steroidal antiinflammatory drugs in treating sciatica, there is a strong need for rigorous investigations."}, {"id": "pubmed23n0392_15955", "title": "A combination of gabapentin and morphine mediates enhanced inhibitory effects on dorsal horn neuronal responses in a rat model of neuropathy.", "score": 0.009174311926605505, "content": "Peripheral nerve damage can result in severe, long-lasting pain accompanied by sensory deficits. This neuropathic pain remains a clinical problem, and effective morphine analgesia is often limited by intolerable side effects. The antiepileptic gabapentin has recently emerged as an alternative chronic pain treatment. Improved management of the diverse symptoms and mechanisms of neuropathic pain may arise from combination therapy, based on multiple pharmacologic targets and low drug doses. The authors used the Kim and Chung rodent model of neuropathy to induce mechanical and cold allodynia in the ipsilateral hind paw. In vivo electrophysiologic techniques were subsequently used to record evoked dorsal horn neuronal responses in which the effects of systemic morphine and gabapentin were investigated, both individually and in combination. Morphine (1 and 4 mg/kg) inhibited neuronal responses of control rats but not after neuropathy. Gabapentin (10 and 20 mg/kg) inhibited neuronal responses in nerve injured rats and to a lesser extent in sham rats but not in naive rats. In the presence of gabapentin (ineffective low dose of 10 mg/kg), morphine (1 and 3 mg/kg) mediated significant inhibitory effects in all experimental groups, with the greatest inhibitions observed in spinal nerve-ligated and sham-operated rats. After neuropathy, inhibitions mediated by morphine were significantly increased in the presence of gabapentin compared with morphine alone. After spinal nerve ligation, the inhibitory effects of systemic morphine on evoked dorsal horn neuronal responses are reduced compared with control, whereas the effectiveness of systemic gabapentin is enhanced. In combination with low-dose gabapentin, significant improvement in the effectiveness of morphine is observed, which demonstrates a clinical potential for the use of morphine and gabapentin combinational treatment for neuropathic pain."}, {"id": "Neurology_Adams_1103", "title": "Neurology_Adams", "score": 0.009174311926605505, "content": "Several types of spinal injections, including epidural, root, and facet blocks, have long been used for the treatment of spinal pain. Injections of epidural corticosteroids or mixtures of analgesics and steroids have been helpful in selected cases of lumbar or thoracic nerve root pain, and occasionally in painful peripheral neuropathy, but precise criteria for the use of this measure are not established. Several randomized trials have failed to support a long term beneficial effect of these treatments but a number of our patients have been helped, if only for several days or weeks (see Chap. 10 for a more complete discussion of these approaches). Nerve root blocks with lidocaine or with longer-acting local anesthetics are sometimes helpful in establishing the precise source of radicular pain. Their main therapeutic use in our experience has been for thoracic radiculitis from shingles, chest wall pain after thoracotomy, and diabetic radiculopathy. Similar local injections are used in"}, {"id": "wiki20220301en294_33593", "title": "Active placebo", "score": 0.00913900913900914, "content": "An active placebo is a placebo that produces noticeable side effects that may convince the person being treated that they are receiving a legitimate treatment, rather than an ineffective placebo. Nomenclature According to a 1965 paper, the term \"concealed placebo\" (German: Kaschiertes Placebo) was suggested in a 1959 paper published in German. Example An example of an active placebo is the 1964 work of Shader and colleagues who used a combination of low-dose phenobarbital plus atropine to mimic the sedation and dry mouth produced by phenothiazines. Morphine and gabapentin are painkillers with the common side effects of sleepiness and dizziness. In a 2005 study assessing the effects of these painkillers on neuropathic pain, lorazepam was chosen as an active placebo because it is not a painkiller but it does cause sleepiness and can cause dizziness."}, {"id": "wiki20220301en019_80256", "title": "Gabapentin", "score": 0.00909090909090909, "content": "The oral bioavailability of gabapentin is approximately 80% at 100 mg administered three times daily once every 8 hours, but decreases to 60% at 300 mg, 47% at 400 mg, 34% at 800 mg, 33% at 1,200 mg, and 27% at 1,600 mg, all with the same dosing schedule. Drugs that increase the transit time of gabapentin in the small intestine can increase its oral bioavailability; when gabapentin was co-administered with oral morphine, the oral bioavailability of a 600 mg dose of gabapentin increased by 50%. Gabapentin at a low dose of 100 mg has a Tmax (time to peak levels) of approximately 1.7 hours, while the Tmax increases to 3 to 4 hours at higher doses. Food does not significantly affect the Tmax of gabapentin and increases the Cmax and area-under-curve levels of gabapentin by approximately 10%."}, {"id": "pubmed23n1007_14107", "title": "Intra-Venous Lidocaine to Relieve Neuropathic Pain: A Systematic Review and Meta-Analysis.", "score": 0.00909090909090909, "content": "<bBackground:</b The prevalence of neuropathic pain is estimated to be between 7 and 10% in the general population. The efficacy of intravenous (IV) lidocaine has been studied by numerous clinical trials on patients with neuropathic pain. The aim of this systematic review and meta-analysis was to evaluate the efficacy of IV lidocaine compared with a placebo for neuropathic pain and secondly to assess the safety of its administration. <bMethods:</b A literature search on PubMed, Scopus, CENTRAL (Cochrane Central Register of Controlled Trials), and Google scholar databases was performed for relevant studies published up to February 2019. Randomized controlled trials (RCTs) evaluating IV lidocaine treatment for pain relief in patients with neuropathic pain were included. <bResults:</b 26 articles met the inclusion criteria. Patients with varied etiology of neuropathic pain were among the patient samples of these studies. Fifteen articles were included for quantitative analysis. Lidocaine was superior to a placebo in relieving neuropathic pain in the early post-infusion period [Mean Difference (MD) = -11.9; 95% Confidence interval (CI): -16.8 to -7; <ip</i &lt; 0.00001]. Multiple infusions of lidocaine over a period of 4 weeks, however, had no significant effect on reliving neuropathic pain (MD = -0.96; 95% CI: -2.02 to 0.11; <ip</i = 0.08). IV lidocaine was also associated with a significant number of adverse events compared to a placebo [Odds Ratio (OR) = 7.75; 95% CI: 3.18-18.92; <ip</i &lt; 0.00001]. <bConclusion:</b Our study indicates that while IV lidocaine is effective in pain control among patients with neuropathic pain in the immediate post-infusion period, it does not have a long-lasting, persistent effect. IV infusions of the drug are associated with an increased risk of side effects compared to a placebo. However, the risk of serious adverse events is negligible. Further, well-designed RCTs evaluating the effects of various dosages and infusion periods of IV lidocaine are required to provide clear guidelines on its clinical use."}, {"id": "wiki20220301en001_2677", "title": "Morphine", "score": 0.009009009009009009, "content": "Further studies on the effects of morphine on the immune system have shown that morphine influences the production of neutrophils and other cytokines. Since cytokines are produced as part of the immediate immunological response (inflammation), it has been suggested that they may also influence pain. In this way, cytokines may be a logical target for analgesic development. Recently, one study has used an animal model (hind-paw incision) to observe the effects of morphine administration on the acute immunological response. Following hind-paw incision, pain thresholds and cytokine production were measured. Normally, cytokine production in and around the wounded area increases in order to fight infection and control healing (and, possibly, to control pain), but pre-incisional morphine administration (0.1 mg/kg to 10.0 mg/kg) reduced the number of cytokines found around the wound in a dose-dependent manner. The authors suggest that morphine administration in the acute post-injury period"}, {"id": "pubmed23n1006_216", "title": "Microsurgery versus Medical Treatment for Neuropathic Pain Caused by Foraminal Extraforaminal Lumbar Disc Herniation: An Observational Study.", "score": 0.009009009009009009, "content": "To investigate the incidence of neuropathic pain (NP) in patients with foraminal/extraforaminal lumbar disc herniation (FEFLDH), the prognosis of NP and the effect of microsurgery on patients treatment. Two patient groups with FEFLDH were investigated: the surgery group underwent surgical treatment, and the medical-treated group received medical treatment. Patients were diagnosed with NP when the Self-Administered Leeds Assessment of Neuropathic Symptoms and Signs (S-LANSS) pain scale was ≥ 12 points. The NP scores were evaluated during patient admission and at 1, 6 and 12 months postoperation or during medical treatment. The surgery group included 37 patients (18 women, 19 men); FEFLDHs were localised at the L3-4 (n=9), L4-5 (n=23) and L5-S1 (n=5) levels. NP was detected in 16 patients before surgery (43%). The medical-treated group included 46 patients (19 women, 27 men); FEFLDHs were localised at the L2-3 (n=7), L3-4 (n=12) and L4-5 (n=27) levels. NP was detected in 20 patients before medical treatment (43%). The most common neuropathic symptom for patients was a burning sensation. NP was found more common in patients who were of at advanced age ( &gt; 65 years) (p=0.019), had a longer clinical duration (p=0.007) or had a foraminal disc herniation (p=0.038). Chronic compression of the dorsal root ganglion by FEFLDH is a significant cause of NP. If surgical treatment is delayed for FEFLDH, the risk of persistent NP may increase."}, {"id": "wiki20220301en214_36334", "title": "Dejerine–Roussy syndrome", "score": 0.008928571428571428, "content": "Pharmaceutical treatment Opiates contain the narcotics morphine, codeine, and papaverine which provide pain relief. Opiates activate μ-opioid receptors in the brain which alter the brain's perception of sensory input, alleviating pain and sometimes inducing pleasure for a short time period. When intravenously administered, opiates can relieve neuropathic pain but only for a time between 4 and 24 hours. After this time window, the pain returns and the patient must be treated again. Although this method of treatment has been proven to reduce pain, the repetitive use of opiates has also been linked to the activation of the brain's reward system and therefore poses a threat of addiction. Heavy doses of opiates can also cause constipation, and respiratory depression. More common side effects include light-headedness, dizziness, sedation, itching, nausea, vomiting, and sweating."}, {"id": "pubmed23n0807_18360", "title": "Curative effect research on curing intercostal neuralgia through paravertebral nerve block combined with pregabalin.", "score": 0.008928571428571428, "content": "This paper aimed to discuss the curative effect and safety of curing intercostal neuralgia through paravertebral nerve block combined with pregabalin. 90 cases of patients diagnosed as intercostal neuralgia were taken as research object. Random number method was used to divide the patients that is conforming to the inclusion criteria and exclusion criteria into 3 groups. 30 cases was in group A (oral lyrica), 30 cases was in group B (paravertebral block only) and 30 cases was in group C (paravertebral block combined with pregabalin). The clinical effect and safety of three groups was compared. The result showed that: visual analogue scale (VAS) and quality of sleep (QS) of three groups of patients after treatment all decreased obviously; group A had slow work, large amount of dosage and many adverse effects; group B had quick work, but the improvement on pain and sleep was not satisfactory; the curative effect of group C was higher than group A and B (p&lt;0.05); 3 groups all had adverse effect, among which group C had the least adverse effect. It can be concluded that paravertebral nerve block combined with pregabalin for curing intercostal neuralgia was superior than single use of pregabalin or paravertebral block and that is worth to promote. "}, {"id": "wiki20220301en354_20189", "title": "Cancer pain", "score": 0.008849557522123894, "content": "Analgesics should not be taken \"on demand\" but \"by the clock\" (every 3–6 hours), with each dose delivered before the preceding dose has worn off, in doses sufficiently high to ensure continuous pain relief. People taking slow-release morphine should also be provided with immediate-release (\"rescue\") morphine to use as necessary, for pain spikes (breakthrough pain) that are not suppressed by the regular medication. Oral analgesia is the cheapest and simplest mode of delivery. Other delivery routes such as sublingual, topical, transdermal, parenteral, rectal or spinal should be considered if the need is urgent, or in case of vomiting, impaired swallow, obstruction of the gastrointestinal tract, poor absorption or coma. Current evidence for the effectiveness of fentanyl transdermal patches in controlling chronic cancer pain is weak but they may reduce complaints of constipation compared with oral morphine."}, {"id": "pubmed23n1115_10079", "title": "Neuropathic Pain Frequency in Neurology Outpatients: A Multicenter Study.", "score": 0.008849557522123894, "content": "Neuropathic pain is common, but the frequency of misdiagnosis and irrational treatment is high. The aim of this study is to evaluate the rate of neuropathic pain in neurology outpatient clinics by using valid and reliable scales and review the treatments of patients. The study was conducted for 3 months in eleven tertiary health care facilities. All outpatients were asked about neuropathic pain symptoms. Patients with previous neuropathic pain diagnosis or who have neuropathic pain symptoms were included and asked to fill painDETECT and douleur neuropathic en 4 questions (DN4) questionnaire. Patients whose DN4 score is higher than 3 and/or painDETECT score higher than 13 and/or who are on drugs for neuropathic pain were considered patients with neuropathic pain. The frequency of neuropathic pain was calculated and the treatments of patients with neuropathic pain were recorded. Neuropathic pain frequency was 2.7% (95% CI: 1.5-4.9). The most common cause was diabetic neuropathy. According to painDETECT, the mean overall pain intensity was 5.7±2.4, being lower among patients receiving treatment. Pharmacological neuropathic pain treatment was used by 72.8% of patients and the most common drug was pregabalin. However, 70% of those receiving gabapentinoids were using ineffective doses. Besides, 4.6% of the patients were on medications which are not listed in neuropathic pain treatment guidelines. In our cohort, the neuropathic pain severity was moderate and the frequency was lower than the literature. Although there are many guidelines, high proportion of patients were being treated by ineffective dosages or irrational treatments."}, {"id": "pubmed23n0745_18202", "title": "Transdermal buprenorphine controls central neuropathic pain.", "score": 0.008771929824561403, "content": "A 53-year-old male with peripheral sensorimotor neuropathy suffered an intracerebral hemorrhage resulting in right hemiparesis and hemisensory loss. Three months later, he developed constant and burning pain within the entire right side of his body. He was diagnosed with central pain syndrome and treated with antiepileptics and tricyclic antidepressants. Minimal analgesia was achieved, which was limited by intractable sedation and drowsiness. Patient was then treated with oral opioids (morphine and hydrocodone with acetaminophen) in escalating doses that produced cognitive impairment. After an opioid rotation was attempted, by switching morphine to transdermal fentanyl, there was no pain reduction or improved quality of life. A trial of buprenorphine was initiated, by administering transdermal patches in escalating doses in weekly intervals. Patient's pain was eventually successfully controlled with buprenorphine patch 60 μg/h every 7 days. His self-reported Visual Analogue Scale pain scores decreased from an average of 8/10 to 2/10 or less. Patient's overall function and participation in home activities increased. Buprenorphine is a partial μ-receptor and a κ-δ receptor antagonist known to block NMDA receptors and reduce hyperalgesia secondary to central sensitization.(1) Buprenorphine is also a partial agonist at the opioid receptor-like (ORL-1) receptor, which is found to be analgesic and antinociceptive at the level of the spinal cord.(1,2) The difference in analgesic responses between buprenorphine and other opioids may be due to different receptor G protein interactions and/or selective activation of neuronal K(ATP) channels by buprenorphine.(3) Deficient opening of K(ATP) channels has been shown to mediate neuropathic pain(4); therefore, activation of these channels by buprenorphine may contribute to its analgesic effect in neuropathic pain states wherein other opioids fail. More recently, there have been two case reports in which patients with neuropathic pain of different central etiology were successfully treated with buprenorphine.(5) Despite advances in understanding the pathology related to central pain, effective treatment options are limited. Buprenorphine may be an analgesic option for central pain management when opioids fail to reduce hypersensitivity or when patients exhibit intolerable side effects to other medications."}, {"id": "InternalMed_Harrison_1296", "title": "InternalMed_Harrison", "score": 0.008771929824561403, "content": "These drugs are useful primarily for patients with neuropathic pain. Phenytoin (Dilantin) and carbamazepine (Tegretol) were first shown to relieve the pain of trigeminal neuralgia. This pain has a characteristic brief, shooting, electric shock–like quality. In fact, anticonvulsants seem to be particularly helpful for pains that have such a lancinating quality. Newer anticonvulsants, gabapentin (Neurontin) and pregabalin (Lyrica), are effective for a broad range of neuropathic pains. Furthermore, because of their favorable side effect profile, these newer anticonvulsants are often used as first-line agents."}, {"id": "pubmed23n0360_13808", "title": "Gabapentin enhances the analgesic effect of morphine in healthy volunteers.", "score": 0.008695652173913044, "content": "The most effective group of drugs for the treatment of severe pain is opioid analgesics. Their use, however, is limited by decreased effects in neuropathic and chronic pain as a result of increased pain and development of tolerance. Gabapentin (GBP) is effective in both experimental models of chronic pain and clinical studies of neuropathic pain. Therefore, we investigated, in a randomized, placebo-controlled, double-blinded study, the pharmacodynamic and pharmacokinetic interaction of GBP and morphine in 12 healthy male volunteers. Morphine (60 mg, controlled release) or placebo was administered at 8:00 AM, and GBP (600 mg) or placebo was administered at 10:00 AM, thus comparing the analgesic effect of placebo + GBP (600 mg) with placebo + placebo and morphine (60 mg) + GBP in comparison to morphine plus placebo by using the cold pressor test. The duration and intensity of the side effects were assessed by using visual analog scales. The analgesic effect was evaluated by the change in the area under the curve (h x %; 0% baseline before Medication 1) of pain tolerance. Placebo + GBP (18.9% x h, 95% confidence interval [CI]: -2.5 to 40.3) did not present any significant analgesic effect compared with placebo + placebo (4.7% x h, 95% CI: -16.7 to 26.1). A significant increase in pain tolerance was observed comparing the combination of morphine and GBP (75.5% x h, 95% CI: 54.0-96.9) with morphine + placebo (40.6% x h, 95% CI: 19. 2-62.0). The observed adverse events after placebo + GBP were not significantly different compared with placebo + placebo. Morphine + placebo led to the expected opioid-mediated side effects. They were significantly more pronounced compared with placebo + placebo but did not differ significantly compared with the combination of morphine + GBP. Concerning the pharmacokinetic variables of morphine and its glucuronides, no significant difference between morphine + placebo and morphine + GBP was observed, whereas the area under the curve of GBP (43.9 +/- 5.3 vs 63.4 +/- 16.2 microg. h(-1). mL(-1), P &lt; 0.05) significantly increased, and apparent oral clearance (230.8 +/- 29.4 mL/min vs 178 +/- 97.9 mL/min, P = 0.06) and apparent renal clearance (86.9 +/- 20.6 vs 73.0 +/- 24.2 mL/min, P = 0.067) of GBP decreased when morphine was administered concomitantly. These results suggest two different sites for the pharmacokinetic interaction-one at the level of absorption and the other at the level of elimination. Our study reveals both a pharmacodynamic and pharmacokinetic interaction between morphine and GBP, leading to an increased analgesic effect of morphine + GBP. These results and the good tolerability of GBP should favor clinical trials investigating the clinical relevance of the combination of morphine and GBP for treating severe pain. In a randomized, placebo-controlled, double-blinded trial with 12 healthy volunteers, we studied the interaction of morphine and gabapentin using the cold pressor test. The anticonvulsant gabapentin enhanced the acute analgesic effect of morphine. Furthermore, the plasma concentration of gabapentin was increased when morphine was administered concomitantly. Therefore, the well tolerated combination of gabapentin and morphine may improve pain therapy, especially in pain states, like chronic and neuropathic pain, which respond poorly to opioids."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[41, 114]], "word_ranges": [[7, 21]], "text": "It is a claude bernard horner syndrome and probably the correct one is 1."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The question would be more of neurology. It is a claude bernard horner syndrome and probably the correct one is 1. But for safety's sake it is better to have it checked by a neurologist.", "full_answer_no_ref": "The question would be more of neurology. It is a claude bernard horner syndrome and probably [HIDDEN]. But for safety's sake it is better to have it checked by a neurologist.", "full_question": "Juan is 60 years old, has been smoking 2 packs/day for years and has reported a persistent cough for the last 6 months. He notes that his left eyelid is more droopy and that the pupil of this eye is smaller. John reports that the medial side of his left hand is numb and with less strength. His physician checks for palpebral ptosis and left miosis; he checks that he can close both eyelids symmetrically and that both pupils respond correctly to light. In addition, he checks that there is no sweating from the left hemiface, that he feels less prickling on the inner surface of the left hand and that he has less strength in the grip of the left hand. Regarding the ocular symptomatology, where is the lesion located?", "id": 263, "lang": "en", "options": {"1": "Sympathetic fibers, at some level that would span from the hypothalamus to the interinedio-lateral Clark's column of the dorsal medulla.", "2": "Left common ocular motor nerve in the midbrain.", "3": "Edinger-Westphal nucleus above the left common ocular motor nerve nucleus.", "4": "Parasympathetic fibers, at some level ranging from the Edinger-Westphal nucleus to the constrictor muscle of the left pupil.", "5": "Tarsal muscle exclusively."}, "question_id_specific": 37, "type": "PNEUMOLOGY", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en012_32998", "title": "Oculomotor nerve", "score": 0.015604128718882818, "content": "All these branches enter the muscles on their ocular surfaces, with the exception of the nerve to the inferior oblique, which enters the muscle at its posterior border. Nuclei The oculomotor nerve (CN III) arises from the anterior aspect of mesencephalon (midbrain). There are two nuclei for the oculomotor nerve: The oculomotor nucleus originates at the level of the superior colliculus. The muscles it controls are the striated muscle in levator palpebrae superioris and other extraocular muscles except for the superior oblique muscle and the lateral rectus muscle. The Edinger-Westphal nucleus supplies parasympathetic fibers to the eye via the ciliary ganglion, and thus controls the sphincter pupillae muscle (affecting pupil constriction) and the ciliary muscle (affecting accommodation)."}, {"id": "wiki20220301en024_53582", "title": "Pupillary light reflex", "score": 0.015543738678197862, "content": "Schematic Referring to the neural pathway schematic diagram, the entire pupillary light reflex system can be visualized as having eight neural segments, numbered 1 through 8. Odd-numbered segments 1, 3, 5, and 7 are on the left. Even-numbered segments 2, 4, 6, and 8 are on the right. Segments 1 and 2 each includes both the retina and the optic nerve (cranial Nerve #2). Segments 3 and 4 are nerve fibers that cross from the pretectal nucleus on one side to the Edinger-Westphal nucleus on the contralateral side. Segments 5 and 6 are fibers that connect the pretectal nucleus on one side to the Edinger-Westphal nucleus on the same side. Segments 3, 4, 5, and 6 are all located within a compact region within the midbrain. Segments 7 and 8 each contains parasympathetic fibers that courses from the Edinger-Westphal nucleus, through the ciliary ganglion, along the oculomotor nerve (cranial nerve #3), to the ciliary sphincter, the muscular structure within the iris."}, {"id": "wiki20220301en010_81090", "title": "Parasympathetic nervous system", "score": 0.013714715137851673, "content": "The oculomotor nerve is responsible for a number of parasympathetic functions related to the eye. The oculomotor PNS fibers originate in the Edinger-Westphal nucleus in the central nervous system and travel through the superior orbital fissure to synapse in the ciliary ganglion located just behind the orbit (eye). From the ciliary ganglion the postganglionic parasympathetic fibers leave via short ciliary nerve fibers, a continuation of the nasociliary nerve (a branch of ophthalmic division of the trigeminal nerve (CN V1)). The short ciliary nerves innervate the orbit to control the ciliary muscle (responsible for accommodation) and the iris sphincter muscle, which is responsible for miosis or constriction of the pupil (in response to light or accommodation). There are two motors that are part of the oculomotor nerve known as the somatic motor and visceral motor. The somatic motor is responsible for moving the eye in precise motions and for keeping the eye fixated on an object. The"}, {"id": "article-18545_21", "title": "Neuroanatomy, Brainstem -- Nerves -- Midbrain", "score": 0.01153983778625954, "content": "The Oculomotor nerve (cranial nerve III) – Arises from the oculomotor sulcus on the medial portion of the crus cerebri. It is a motor nerve that receives inputs from two nuclei. The first nucleus is the oculomotor nucleus; it serves as its main motor nucleus and is in the anterior midline of the periaqueductal grey at the level of the superior colliculus. The second nucleus is the Edinger-Westphal nucleus, which provides parasympathetic motor inputs. The somatic motor fibers from the oculomotor nucleus provide innervation to all the extraocular muscles, with the exceptions of the superior oblique and lateral rectus muscles. The parasympathetic motor fibers of the Edinger-Westphal nucleus provide innervation to the ciliary muscles and constrictor pupillae after passing through the ciliary ganglion."}, {"id": "article-31142_15", "title": "Neuroanatomy, Vestibular Pathways -- Nerves -- Vestibulocochlear Nerve", "score": 0.011382113821138212, "content": "CN III, IV, and VI nerves innervate the six extraocular muscles that control eye movements in response to head movements. When the head turns to the left, vestibular information initiates its journey from the left semicircular canal via vestibular nerve fibers and proceeds to the ipsilateral medial and superior vestibular nuclei. Then, excited neurons in the left superior and medial vestibular nuclei send signals to the right CN VI nucleus. The right CN VI innervates the right lateral rectus muscle to contract and direct the gaze to the right. Another nerve fiber projects from the CN VI nucleus and ascends via the MLF toward the left CN III nucleus. When stimulated, the oculomotor nerve results in the medial rectus muscle contraction and allows the left eye to gaze to the right. Inhibitory nerve fibers also proceed from the medial vestibular nucleus to the left CN VI nucleus, rendering the left lateral rectus and right medial rectus muscles to relax. The result of these combined counteracting effects is a smooth, coordinated eye movement toward the opposite direction, and thus a steady visual field with head-turning. The trochlear nerves innervate the contralateral superior oblique muscle and stimulate the muscle to contract and make eye movements via lateral rotation, intorsion, and depression. [8] [24]"}, {"id": "article-43127_9", "title": "Anatomy, Head and Neck: Eye Nerves -- Structure and Function -- Parasympathetic nervous system", "score": 0.0108325956296883, "content": "The parasympathetic nervous system causes pupillary constriction through a process called miosis. The parasympathetic nerve fibers start at the first neuron called the Edinger-Westphal nucleus. This neuron's nerve fibers span to the ciliary ganglion by traveling on the oculomotor nerve. After the ciliary ganglion, the second neuron is from the short ciliary nerves to the sphincter pupillae muscles. When the sphincter pupillae muscle contract, it will cause the constriction of the pupils. [7]"}, {"id": "wiki20220301en033_38417", "title": "Ciliary body", "score": 0.010446343779677113, "content": "Nerve supply The parasympathetic innervation of the ciliary body is the most clearly understood. Presynaptic parasympathetic signals that originate in the Edinger-Westphal nucleus are carried by cranial nerve III (the oculomotor nerve) and travel through the ciliary ganglion. Postsynaptic fibers from the ciliary ganglion form the short ciliary nerves. Parasympathetic activation of the M3 muscarinic receptors causes ciliary muscle contraction, the effect of contraction is to decrease the diameter of the ring of ciliary muscle. The parasympathetic tone is dominant when a higher degree of accommodation of the lens is required, such as reading a book. The ciliary body is also known to receive sympathetic innervation via long ciliary nerves. When test subjects are startled, their eyes automatically adjust for distance vision."}, {"id": "wiki20220301en603_15861", "title": "Roots of the ciliary ganglion", "score": 0.010167310167310168, "content": "Presynaptic parasympathetic fibers originate in the Edinger-Westphal nucleus, the parasympathetic motor nucleus associated with the oculomotor nucleus in the brainstem. Axons from the Edinger-Westphal nucleus and the oculomotor nucleus run together in the brainstem and exit together as the oculomotor nerve. The oculomotor nerve passes through the lateral wall of the cavernous sinus and enters the orbit through the superior orbital fissure. It divides into branches that innervate the levator palpebrae superioris and four of the six extraocular muscles. Parasympathetic fibers initially run in the inferior division of the oculomotor nerve. They exit as one or two short “motor roots” that synapse in the ciliary ganglion."}, {"id": "article-28080_3", "title": "Pupillary Light Reflex -- Anatomy and Physiology", "score": 0.009948785807052366, "content": "Light travels through the cornea, anterior chamber, pupil, lens, and the posterior chamber, eventually reaching the retina. Photoreceptor cells in the outer layers of the retina, which are called rods and cones, convert light stimuli into neuronal impulses. These signals are then relayed to the bipolar cells, which interact with ganglion cells, which in turn coalesce to form the optic disc and optic nerve (CN II). The optic nerve sends impulses to the brain for further processing and image recognition. [1] These are the first steps of the pupillary light reflex afferent pathway. The optic nerve then forms the optic chiasm, which diverges into a left and right optic tract. At the optic chiasm, nasal retinal fibers will cross to the contralateral side of the optic tract, and the temporal retinal fibers continue on the ipsilateral side. Thus, the right optic tract will contain temporal retinal fibers from the right eye, as well as nasal retinal fibers from the left eye. The optic tracts join the brachium of the superior colliculus, and then signals travel to the pretectal area of the midbrain. Each pretectal area sends bilateral signals to the preganglionic parasympathetic nuclei in the midbrain called Edinger-Westphal nuclei. [2] [3] There are a minority of axons that go to the hypothalamus and the olivary pretectal nucleus (OPN). [4] Efferent parasympathetic preganglionic fibers travel on the oculomotor nerve and synapse with the ciliary ganglion, which sends postganglionic axons to directly innervate the iris sphincter muscles. The contraction of the iris sphincter muscles leads to pupillary constriction (miosis). [3] This extensive pathway is being tested when a light is shined in the eyes. And, because of the crossing fibers, there is not only a direct pupillary reflex but also a consensual pupillary light reflex. Of note, the pupillary dark reflex involves a separate pathway, which ends with sympathetic fibers from long ciliary nerves innervating the dilator pupillae muscle."}, {"id": "pubmed23n0968_15434", "title": "Isolated Horner syndrome as a rare initial presentation of nasopharyngeal carcinoma: a case report.", "score": 0.009900990099009901, "content": "Horner syndrome refers to a set of clinical presentations resulting from disruption of sympathetic innervation to the eye and adnexa. Classically, the clinical triad consists of ipsilateral blepharoptosis, pupillary miosis, and facial anhidrosis. Ocular sympathetic denervation may signify life-threatening causes. Timely investigation and accurate diagnosis are essential in patients with oculosympathetic denervation. A 33-year-old Asian man with a heavy smoking habit presented with a 3-week history of left ptosis and no other complaints. His visual acuity was 20/20 bilaterally. An ophthalmic examination was significant for mild ptosis of his left eyelid and anisocoria (smaller left pupil), which was greater in the dark. Both pupils reacted to light briskly without an afferent pupillary defect. Anhidrosis was found on the medial side of the left forehead. A 10% cocaine test was positive. At his first visit, neurologic examination was unremarkable. Comprehensive radiological investigations were scheduled for a left-sided isolated Horner syndrome. Two weeks after his first visit, he experienced a left-sided headache along with ipsilateral Horner syndrome. Neurologic examination revealed hypoesthesia in the left cranial nerve V<sub1-3</sub territories. Emergent computed tomography angiography was suspected for petrous part of the left internal carotid artery (ICA) dissection. Magnetic resonance imaging demonstrated an enhancing infiltrative lesion with its epicenter at the left sphenoid bone. The lesion encased the left ICA and invaded the left Meckel cave. Rhinoscopy with incisional biopsy revealed squamous cell nasopharyngeal carcinoma. This case involved an unusual initial presentation of nasopharyngeal carcinoma: isolated Horner syndrome with clinical progression to adjacent structures. Infiltration involving the Meckel cave and ICA at the foramen lacerum can present as postganglionic Horner syndrome associated with trigeminal pain and hypoesthesia. These clinical findings may mimic carotid artery dissection on computed tomography angiography. Detailed magnetic resonance imaging with careful attention to the skull base should be performed."}, {"id": "article-30060_2", "title": "Cranial Nerve III Palsy -- Introduction", "score": 0.009900990099009901, "content": "The third cranial nerve is also known as oculomotor nerve and has 2 major components: Outer parasympathetic fibers that supply the ciliary muscles and the sphincter pupillae Inner somatic fibers that supply the levator palpebrae superioris in the eyelid (which retracts the upper eyelid) and the 4 extraocular muscles (superior, middle, inferior recti, and inferior oblique). LR6(SO4)3 is a simple mnemonic representing the innervation of the extraocular muscles. It stands for: LR6: Lateral rectus muscle which is supplied by the sixth cranial nerve SO4: Superior oblique muscle which is supplied by the fourth cranial nerve 3: The third cranial nerve supplies other extraocular muscles"}, {"id": "pubmed23n0264_19619", "title": "[A 55-year-old man with prostate cancer, papilledema, and multiple cranial nerve palsies].", "score": 0.00980392156862745, "content": "We report a 55-year-old man with papilledema and multiple cranial nerve palsies. He was well until 52 years of age when there was an onset of progressive difficulty in initiating urination; he visited the urology service of our hospital where a diagnosis of prostate cancer was made; the cancer was invading the bladder and was metastasizing to lymph nodes and bones. He was treated with oochiectomy and estrogen preparations with some improvement in his symptoms. Two years later, he developed difficulty in urination again, and transurethral resection of the tumor was performed in 1991. In December 1991, he noted tingling and numb sensation in his left face, which had become progressive worse within the next one month, and he developed blepharoptosis and deafness all on the left side. He was admitted to the urology service on February 4, 1992, and a neurological consultation was asked. On physical examination, general findings were unremarkable, except for lymph node enlargements of about 0.5 to 1.0 mm in size in cervical and inguinal regions. On neurologic examination, he was alert with normal mental activities; higher cerebral functions were intact. He had normal vision and visual fields, however, papilledema was present bilaterally; pupils and light reactions were normal. Extraocular muscles were intact on the right side, however, moderate restriction was noted in the left eye in that all the extraocular muscles except for the medial rectus were weak; blepharoptosis was noted on the left; no nystagmus was present. The sensation was diminished in the left face, and left facial paresis of the peripheral type was also noted; the taste sensation was also diminished in the left anterior two thirds of the tongue. He had sensorineural deafness on the left side. The other cranial nerves appeared intact. He walked normally; no weakness or muscle atrophy was noted; muscle tone was normal and no ataxia was observed. Deep reflexes were normally elicited and symmetric; the plantar response was flexor. No meningeal signs were present. Laboratory examination revealed following abnormalities: Hb 7.1 g/dl, platelet 47,000/cmm, WBC3,800/cmm, LDH 950IU/l, PAP232ng/ml (normal less than 1.6), PA2.631ng/ml (normal less than 7.4); a small amount of effusion was noted in the left pleural cavity; cytological examination of the fluid was class V. A cranial CT scan as well as MRI were entirely normal, as was the spinal tap. He was treated with glycerol, however, there was progressive increase in the pleural effusion, and he developed dyspnea; moist rale had become audible in the end of February.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "article-43127_20", "title": "Anatomy, Head and Neck: Eye Nerves -- Nerves", "score": 0.00980392156862745, "content": "There are six cranial nerves and nerve fibers from the autonomic nervous system. Optic nerve Oculomotor nerve Short ciliary nerve (parasympathetic nervous system) Trochlear nerve Trigeminal nerve (ophthalmic branch) Abducens nerve Facial nerve Long ciliary nerve (sympathetic nervous system)"}, {"id": "Physiology_Levy_1217", "title": "Physiology_Levy", "score": 0.009802225681831287, "content": "Fig. 11.1 ). Hence, this part of the autonomic nervous system is sometimes called the craniosacral division. The cranial nerve nuclei that contain parasympathetic preganglionic neurons are the Edinger-Westphal nucleus (cranial nerve III), the superior (cranial nerve VII) and inferior (cranial nerve IX) salivatory nuclei, and the dorsal motor nucleus of the vagus and nucleus ambiguus (cranial nerve X). Postganglionic parasympathetic cells are located in cranial ganglia, including the ciliary ganglion (preganglionic input is from the Edinger-Westphal nucleus), the pterygopalatine and submandibular ganglia (input is from the superior salivatory nucleus), and the otic ganglion (input is from the inferior salivatory nucleus). The ciliary ganglion innervates the pupillary sphincter and ciliary muscles in the eye. The pterygopalatine ganglion supplies the lacrimal gland, as well as glands in the nasal and oral pharynx. The submandibular ganglion projects to the submandibular and sublingual"}, {"id": "wiki20220301en024_53577", "title": "Pupillary light reflex", "score": 0.009767170644341705, "content": "The pupillary light reflex neural pathway on each side has an afferent limb and two efferent limbs. The afferent limb has nerve fibers running within the optic nerve (CN II). Each efferent limb has nerve fibers running along the oculomotor nerve (CN III). The afferent limb carries sensory input. Anatomically, the afferent limb consists of the retina, the optic nerve, and the pretectal nucleus in the midbrain, at level of superior colliculus. Ganglion cells of the retina project fibers through the optic nerve to the ipsilateral pretectal nucleus. The efferent limb is the pupillary motor output from the pretectal nucleus to the ciliary sphincter muscle of the iris. The pretectal nucleus projects crossed and uncrossed fibers to the ipsilateral and contralateral Edinger-Westphal nuclei, which are also located in the midbrain. Each Edinger-Westphal nucleus gives rise to preganglionic parasympathetic fibers which exit with CN III and synapse with postganglionic parasympathetic neurons in"}, {"id": "pubmed23n0480_8345", "title": "Permanent paralysis at sites of dermal exposure to chlorpyrifos.", "score": 0.009708737864077669, "content": "Poisoning with organophosphate pesticides can cause sensory and motor neuropathy with permanent paralysis. Paralysis at the site of dermal exposure has not been reported. A 61-year-old carpenter sprayed a nest of termites with an insecticide containing chlorpyrifos without protective equipment and with direct contact of pesticide solution to hands, lower arms, feet, and lower legs, as well as inhalation of vapors from spraying. After 30 min he became ill with nausea, abdominal cramping, arm and leg weakness, bilateral shoulder pain, chest pain, and numbness in the left hand and arm. At a hospital, he was treated with atropine 1 mg IV and pralidoxime Cl 2 g IV There was 0/5 strength in the hands and wrists and 3/5 elsewhere, a left peritoneal palsy, and urinary retention. He was transferred to a tertiary care hospital where paralysis persisted. Electromyogram studies documented widespread peripheral neuropathy. With continued progression of neuropathy, pralidoxime was repeated on the third day. By day 12, motor strength improved except for the hands and left lower leg. Right interosseous muscle strength was 1/5 and left was 0/5. Right-hand grip was 2/5, and left-hand grip was 0/5. He was transferred to a rehabilitation center. He never regained use of his hands and was disabled from employment as a carpenter. There was a disturbed gait, with inability to clear his left foot with walking. Urinary retention persisted and required self-catherization. Dermal exposure of the hands and feet to chlorpyrifos was associated with atrophy and permanent paralysis of exposed areas. The importance of protective equipment is emphasized."}, {"id": "article-43127_6", "title": "Anatomy, Head and Neck: Eye Nerves -- Structure and Function -- Abducens Nerve (CN VI)", "score": 0.009708737864077669, "content": "The abducens nerve innerves only one muscle in the eye. This muscle is the lateral rectus muscle. When this muscle contracts, it causes the eye to abduct. [5]"}, {"id": "pubmed23n0298_11176", "title": "[A 56-year-old man with fever, backache and tetraparesis].", "score": 0.009615384615384616, "content": "We report a 56-year-old man who developed progressive paraparesis. He was apparently well, except for left Bell's palsy which developed on May 9 of 1994, for which he received stellate ganglion block on the left side more than ten times until July 2nd of 1994, when he noted pain in his left shoulder and in his lumbar region. On July 5th, he noted some difficulty in urination. On July 6th, he noted tingling sensation in his four extremities and difficulty in gait. He was admitted to another hospital where he was treated with intravenous infusion of glycerol. After this treatment, his gait and sensory disturbance showed some improvement, however, on July 7th, his shoulder and lumbar pain worsened, and he became unable to stand. His temperature went up to 39 degrees C on the next day. Lumbar CSF on that day contained 119 cells/microliters, 112 mg/dl of protein, and 53 mg/dl of sugar. He was transferred to our hospital on July 14th. His past medical history revealed that he had suffered from frequent bouts of osteomyelitis since the age of 13 years. He was operated on several times on osteomyelitis. He had been treated on his tooth ache until shortly before the onset of the present illness. He also received steroid hormone for his Bell's palsy. On admission, his consciousness varied from alert to stupor. His BP was 150/100 mmHg, HR 98/min and regular, BT 39.4 degrees C. The bulbar conjunctiva appeared somewhat icteric. Otherwise, general physical examination was unremarkable. On neurologic examination, there was no apparent dementia. Higher cerebral functions appeared intact. The optic discs were flat. Pupils were round and isocoric reacting to light and accommodation promptly. Ocular movements were full without nystagmus. Some exophthalmos was noted bilaterally. The sensation of the face and facial muscles were intact. The remaining cranial nerves also appeared intact. Nuchal rigidity was present. He was unable to stand or walk. Muscle strength was markedly diminished in all four limbs; manual muscle testing revealed 1 to 2/5 weakness in both upper and lower extremities bilaterally. Muscle stretch reflexes were decreased or lost in both upper and lower limbs, but the plantar response was extensor on the right. Sensation appeared to be diminished in legs, but detail was not clear because of disturbance of consciousness. Pertinent laboratory findings were as follows: WBC 12,800/microliter, GPT 58 IU/l, total bilirubin 2.65 mg/dl, and CRP 16.8 mg/dl. Cerebrospinal fluid contained 34 cells/microliter (approximately two thirds were neutrophils), RBC 1,110/microliter, 2,949 mg/dl of protein, and 119 mg/dl of glucose; stapylococcus aureus was cultured from the CSF. Myelogram showed a filling defect in the anterior epidural space between the low thoracic and the upper lumbar region. The patient was treated with cephotaxim, aminobenzyl penicillin, and chloramphenicol. On the second hospital day, his BT was still 39 degrees C and he was agitated His weakness was worse than the previous day. Spinal MRI was attempted; as he was agitated 5 mg of diazepam was given intravenously at 4 PM. His respiration was rapid and somewhat shallow. At 6 PM, gadolinium DTPA was injected intravenously; at that time, he was breathing and pupils were 3 mm on both sides. At 6:35 PM, an examiner noted that he stopped breathing; the left pupil was dilated to 5 mm. Cardiopulmonary resuscitation was initiated immediately, and intubation was performed. He was placed on a respirator. His blood pressure did not reach 100 mmHg; he was in deep coma. Cardiac arrest occurred at 8:53 AM on the next morning. The patient was discussed in a neurological CPC. Most of the participants thought that the patient had either spinal epidural empyema or spinal subdural abscess. The question was what might be the original focus of infection. Three possibilities were considered, i.e., stellate ganglion block, teeth infection, and osteomyelitis..."}, {"id": "article-21498_13", "title": "Anatomy, Head and Neck, Eye -- Muscles", "score": 0.009615384615384616, "content": "Six extrinsic eye muscles that move each eye: (1) superior rectus, (2) inferior rectus, (3) lateral rectus, (4) medial rectus, (5) superior oblique, and (6) inferior oblique. Lateral rectus is innervated by trochlear and superior oblique is innervated by abducens nerve; The oculomotor nerve innervates the remaining four muscles. The motor units in these muscles tend to be small with neurons serving only two or three muscle fibers at times. Such small motor units allow precise and rapid eye movement. Portions of the brainstem and cerebellum coordinate eye movements."}, {"id": "pubmed23n1151_14732", "title": "A Case Report of Herpes Zoster-Associated Bickerstaff Brainstem Encephalitis.", "score": 0.009523809523809525, "content": "Bickerstaff brainstem encephalitis (BBE) is a rare demyelinating disease of the central nervous system (CNS) that is caused by a direct viral infection or secondary autoimmune responses. BBE secondary to Herpes zoster has rarely been reported. A 68-year-old man developed a painful vesicular rash and drooping eyelid on the left side of his face for 20 days. Physical examination revealed left-sided blepharoptosis and crusted erythema on the left front side of his face, left upper eyelid, and left nasal tip. Neurological examination showed impaired sensation over the left side of his face and cheek. His left pupil was dilated (4mm compared to 2mm on the right side), and the Pupillary light reflection (PLR) was absent, with an ocular movement disorder (limited adduction) and diplopia. Brain imaging did not reveal abnormalities. Cerebrospinal fluid (CSF) examination showed leukocytosis and increased protein levels. He was treated with intravenous acyclovir for 7 days, but developed disturbance of consciousness and right limb weakness. Neurological examination revealed right lower limb hypoesthesia. The Heel-Knee-Shin test was positive on the left side, and Babinski's sign was present on the right side. He was diagnosed with Bickerstaff brainstem encephalitis caused by herpes zoster. After 2 days of intravenous acyclovir combined with intravenous immune globulin (IVIG), the patient developed acute kidney injury (AKI). Then, his treatment was changed to systemic steroids. At the 3-month follow-up, his pupils were bilaterally equal and reactive to light, and there was a significant improvement in ocular motility and ptosis. At the 6-month follow-up, his diplopia had completely resolved. BBE associated with herpes zoster is very rare and can be overlooked. Dermatologists should be aware of the expanding spectrum of neurological complications caused by varicella zoster virus (VZV) infections to aid early diagnosis and treatment."}, {"id": "article-36670_5", "title": "Anatomy, Head and Neck: Eye Inferior Oblique Muscles -- Nerves", "score": 0.009523809523809525, "content": "The inferior division of cranial nerve III, also known as the oculomotor nerve, runs inferolaterally along the inferior rectus to innervate the inferior oblique muscle. The nerve is approximately 27 mm long, running from orbital apex to the inferior oblique muscle. [6] The nerve to the inferior oblique is the longest branch of the inferior division of the oculomotor nerve. It enters the muscle through its orbital surface. [2] The nerve has been measured to be approximately 1.04mm on the right and 1.07 mm on the left. [2]"}, {"id": "wiki20220301en117_44292", "title": "Ptosis (eyelid)", "score": 0.009473507712944333, "content": "Ptosis caused by oculomotor palsy can be unilateral or bilateral, as the subnucleus to the levator muscle is a shared, midline structure in the brainstem. In cases in which the palsy is caused by the compression of the nerve by a tumor or aneurysm, it is highly likely to result in an abnormal ipsilateral papillary response and a larger pupil. Surgical third nerve palsy is characterized by a sudden onset of unilateral ptosis and an enlarged or sluggish pupil to the light. In this case, imaging tests such as CTs or MRIs should be considered. Medical third nerve palsy, contrary to surgical third nerve palsy, usually does not affect the pupil and it tends to slowly improve in several weeks. Surgery to correct ptosis due to medical third nerve palsy is normally considered only if the improvement of ptosis and ocular motility are unsatisfactory after half a year. Patients with third nerve palsy tend to have diminished or absent function of the levator."}, {"id": "pubmed23n0290_728", "title": "[A 62-year-old man with familial parkinsonism with the onset at 24 years of the age].", "score": 0.009433962264150943, "content": "We report a right-handed 62-year-old man with early onset familial parkinsonism. The patient was well until 24 years of the age when he noted an onset of resting tremor in his right hand. During the next four years, he noted rigidity, bradykinesia, and difficulty in walking. He was seen in another hospital at 28 years of the age, where he received left pallidotomy. Rigidity on the left side showed marked improvement. He received right pallidotomy at age 30 years. He developed right hemiplegia after this surgery. He was admitted to our hospital in March, 1983 when he was 51 years of the age. He was treated with levodopa but improvement was rather of minor degree. He was transferred to another hospital, but his motor disturbance progressed slowly, and was admitted again to our hospital in November 1990. He had 6 siblings 4 of whom including himself suffered from parkinsonism. No consanguinity was noted in parents. On admission, he appeared chronically ill but the general physical examination was unremarkable. Neurologic examination revealed an alert and mentally sound man. Hasegawa dementia scale was 28.5/32.5. Upward gaze was slightly restricted (3/5). Cranial nerve examination revealed oculogyric crisis, apraxia of eyelid opening, masked face, and small voice. He was able to stand with support; his posture showed left-ward leaning. He had right hemiparesis with moderate weakness. He showed marked bradykinesia and moderate rigidity in his left upper extremity. Fine postural tremor was noted in the left hand. Deep tendon reflexes were diminished in the upper extremities. No Babinski sign was noted. Pain sensation was somewhat diminished on the right side. Results of routine laboratory examination were unremarkable. Cranial CT scan revealed atrophy in the frontal lobe, particularly in the prefrontal area. In addition, MRI revealed T1-and-T2-low signal intensity lesions in the right ventral pallidal region and in the left ventrolateral thalamic-hypothalamic areas. He was treated with 600 mg of levodopa with benserazide and 22.5 mg of bromocriptine with mild to moderate improvement in his bradykinesia and rigidity. He was discharged in January 1991. His clinical course was complicated by intestinal obstruction in October, 1994. He was admitted to another hospital where he was operated on the obstruction on November 5, 1994. The sigmoid colon was markedly dilated but no mass was found. Postoperative course was uneventful until November 18, 1994 when he was found dead in his hospital room shortly after 4 am. The patient was discussed in neurological CPC, and the chief discussant arrived at the conclusion that the patient had young-onset familial Lewy body-negative parkinsonism. Opinions were divided between Lewy body-positive familial Parkinson's disease and Lewy body negative young onset parkinsonism. Postmortem examination revealed aspiration pneumonia, which appeared to be the cause of his death, in the right lung. Neuropathologic examination revealed loss of malanized neurons in the substantia nigra and the locus coeruleus. In the substantia nigra, neuronal loss was particularly severe in the ventrolateral area. No Lewy bodies were seen. The dorsal motor nucleus of the vagal nerve was well preserved. Stereotaxic lesions involved bilateral thalamic areas. This patient appears to represent a case of autosomal recessive juvenile parkinsonism (AR-JP). Early onset, superb response to levodopa, sleep effect, and easy development of dyskinesias and motor fluctuations characterize AR-JP. The reason why this patient did not show these clinical features is probably bilateral sterotaxic surgeries. Particularly, the second surgery was complicated by right hemiparesis. His siblings who developed parkinsonism showed typical clinical features of AR-JP."}, {"id": "article-43127_35", "title": "Anatomy, Head and Neck: Eye Nerves -- Clinical Significance -- Oculomotor Nerve", "score": 0.009433962264150943, "content": "Motor : Compromised of the motor function of the oculomotor nerve will manifest as a down and out gaze with ptosis. The reason behind this eye position is the unopposed action of the trochlear and abducens nerve pulling the eye inferiorly and laterally, respectively. Causes of compromise to the motor function of the oculomotor nerve can be from ischemia from vascular disease or due to the build-up of sorbitol from diabetes. This ischemia is due to poor perfusion. The motor nerve fibers of the oculomotor nerve can be affected along with the parasympathetic nerve fibers with severe compression. The compression of the oculomotor nerve first affects the parasympathetic nerve fibers; then, it can progress to compression of the arterial blood supply."}, {"id": "Physiology_Levy_1090", "title": "Physiology_Levy", "score": 0.009392374543426237, "content": "Fig. 8.27 ). The depolarized hair cells cause increased activity in the left vestibular afferent fibers and thereby excite neurons of the left medial vestibular nucleus. These include excitatory neurons that project to the contralateral abducens nucleus and synapse with both motor neurons and internuclear neurons. Excitation of the motor neurons leads to contraction of the right lateral rectus muscle and rotation of the right eye to the right, whereas excitation of the internuclear neurons of the right abducens nucleus leads to excitation of the medial rectus motor neurons in the left oculomotor nucleus, thus causing the left eye to rotate to the right as well."}, {"id": "pubmed23n0898_14607", "title": "A rare presentation of myxofibrosarcoma as a Pancoast tumor: a case report.", "score": 0.009345794392523364, "content": "Myxofibrosarcoma is an aggressive soft tissue neoplasm, classified as a variant of malignant fibrous histiocytoma. Most often, it occurs in middle to late adult life peaking in the seventh decade and involving the lower extremities (77%), trunk (12%), and retroperitoneum or mediastinum (8%). We report the first case of thoracic myxofibrosarcoma presenting as a Pancoast tumor. A 48-year-old non-tobacco smoking African-American man presented with a slow-growing mass in his neck along with 11 kg weight loss over 9 months. A review of his systems was positive for hoarseness and lowgrade intermittent fever without any shortness of breath or cough. A physical examination revealed a mass on the left side of his neck superior to his sternoclavicular joint measuring 3 × 3 × 1 cm. He had ptosis and miosis of his left eye. His breath sounds were decreased and coarse at the left apex. A neurological examination revealed 3/5 strength in his left upper arm. The remainder of the physical examination was unremarkable. Ultrasound of his neck showed an ill-defined heterogeneous mass lateral to his left thyroid lobe. A computed tomography scan of his chest showed a large multiloculated pleural-based mass in his left lung surrounding the adjacent neurovascular structures. A percutaneous biopsy was non-diagnostic. Subsequently, he underwent a left thoracotomy with biopsy. The mass extended from his anterior mediastinum medially at the level of the pulmonary trunk, superiorly into the superior sulcus and posteriorly into his chest wall. Surgical pathology confirmed the diagnosis of myxofibrosarcoma. Here we present a case of Pancoast tumor with myxofibrosarcoma as the underlying etiology. Pancoast syndrome generally entails an infiltrating lesion in the superior sulcus presenting with upper extremity pain, atrophy of the hand muscles, and Horner's syndrome. The differential diagnosis of Pancoast syndrome includes inflammatory and infectious etiologies, as well as neoplasms of benign and malignant nature. Of the neoplasms implicated, the most common are non-small cell lung carcinomas; myxofibrosarcoma presenting as a Pancoast tumor has not been reported in the literature."}, {"id": "article-23495_9", "title": "Anatomy, Head and Neck: Eye Inferior Rectus Muscle -- Nerves", "score": 0.009345794392523364, "content": "Additionally, the parasympathetic innervation of the sphincter pupillae and ciliary muscle travels with the branch of the lower division of cranial nerve III that supplies the inferior oblique muscle, and this bundle passes near the inferior rectus muscle. This anatomy has important surgical considerations that will be explained further. [2]"}, {"id": "pubmed23n0260_19248", "title": "[A 37-year-old man with memory loss, homonymous hemianopsia, and elevation of anti-herpes simplex virus antibody titer].", "score": 0.009259259259259259, "content": "We report a 37-year-old man who presented memory loss, homonymous right hemianopsia, and elevation of anti-herpes simplex antibody titer. He had an auto accident in January 1992 in that the car he was driving slipped down a 3 m slope; his car was severely damaged, however, he himself was not injured. Shortly after this accident, he went out of his house less often than before, and he noted some difficulty in his vision. He changed his glasses twice, but his vision was unchanged. In July of 1992, he had an onset of difficulty in recent memory and disorientation to time. He also noted diplopia, and difficulty in seeing objects in his right visual field. He was admitted to our hospital on August 26 of the same year. General physical examination was unremarkable. On neurologic examination, he was alert but disoriented to time and place; calculation was also impaired. Mini-mental state examination was 18/30. He had no aphasia, apraxia, or agnosia. He showed a tendency to neglect his left side. Optic fundi and visual acuity were normal; right homonymous hemianopsia was present. Ocular movement was moderately restricted to most of the directions; pupils were isocoric and reacted to light promptly. He complained of diplopia in right gaze, and monocular nystagmus was induced in his right eye upon right lateral gaze. Trigeminal nerves appeared intact. Minimum left facial weakness was present. The remaining of the cranial nerves appeared intact. His gait was wide-based and tandem gait was impossible. Muscle strength was normal as was the muscle tone. Finger to nose and heel to knee tests were done normally.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0022_10957", "title": "Sympathetic connections to the fifth and sixth cranial nerves.", "score": 0.009259259259259259, "content": "Thirty additional parasellar gross dissections and light microscopic examinations have been carried out, confirming a previous observation that the sympathetic nerve or nerves running with the carotid artery gives off a multitude of fine branches at irregular intervals on the way up, but the largest residual component joins the sixth cranial nerve and leaves to join the first division of the fifth cranial nerve. No similar fibers can be found by us to the sympathetic in the neck results in a Horner's syndrome and that section of the ophthalmic artery at its point of departure from the carotid does not result in any part of Horner's syndrome nor does secretion of the external, internal or common corotid in the neck, it is assumed that these fibers process the functions the absence of which result in Horner's syndrome."}, {"id": "wiki20220301en024_53578", "title": "Pupillary light reflex", "score": 0.009230965113318054, "content": "are also located in the midbrain. Each Edinger-Westphal nucleus gives rise to preganglionic parasympathetic fibers which exit with CN III and synapse with postganglionic parasympathetic neurons in the ciliary ganglion. Postganglionic nerve fibers leave the ciliary ganglion to innervate the ciliary sphincter. Each afferent limb has two efferent limbs, one ipsilateral and one contralateral. The ipsilateral efferent limb transmits nerve signals for direct light reflex of the ipsilateral pupil. The contralateral efferent limb causes consensual light reflex of the contralateral pupil."}, {"id": "article-36670_7", "title": "Anatomy, Head and Neck: Eye Inferior Oblique Muscles -- Nerves", "score": 0.009174311926605505, "content": "The inferior division of the oculomotor nerve also carries parasympathetic fibers along the optic nerve to the ciliary ganglion. These fibers innervate the sphincter pupillae and ciliary muscles. [2] [7]"}, {"id": "pubmed23n0043_5332", "title": "[A case of hemi-hyperhidrosis and non-paralytic pontine exotropia due to brainstem infarction].", "score": 0.00909090909090909, "content": "A case of hemihyperhidrosis and non-paralytic pontine exotropia due to brainstem infarction is reported. A 55-year-old hypertensive man developed right hemiparesis with slight dysarthria and nausea upon awaking. The right side of his face and right upper limb and trunk to the level of the Th8-9 territory showed hyperhidrosis, which disappeared in a week. Ocular motor examination revealed that during forward gaze with the left eye fixing, the right eye deviated outward. The patient was able to adduct the right eye to midposition with the right eye fixing. Rightward gaze elicited full abduction and right-beating nystagmus of the right eye, but the left eye did not adduct. When he attempted to gaze leftward, both eyes made the full excursion, but saccades were slow in that direction. Convergence was intact. Vertical gaze was full, and he did not show Horner's sign. This ocular sign, non-paralytic pontine exotropia, disappeared three days later. T2-weighted spin echo magnetic resonance imaging disclosed a small lesion with high intensity in the inner side of the left middle pons. This hyperhidrosis was thought to be caused by destruction of inhibitory fibers thermoregulating sweating. These findings suggest that at the level of the middle pons inhibitory fibers descend along the inner side of facilitatory fibers thermoregulating sweating, which are speculated to descend the dorso-lateral part of the pontine tegmentum. These findings also suggest that lesions of non-paralytic pontine exotropia may be located in the paramedian pontine reticular formation rostral to the abducens nucleus with ipsilateral medial longitudinal fasciculus lesion, but further investigation is necessary."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 367]], "word_ranges": [[0, 59]], "text": "She should not become pregnant until she has undergone regular check-ups and one year has passed with negative beta-HCG levels. Patients will be monitored weekly with hCG dosing until it becomes undetectable, for three consecutive times. After that the monitoring will be monthly for six months and then every two months for another six months before a new pregnancy."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "She should not become pregnant until she has undergone regular check-ups and one year has passed with negative beta-HCG levels. Patients will be monitored weekly with hCG dosing until it becomes undetectable, for three consecutive times. After that the monitoring will be monthly for six months and then every two months for another six months before a new pregnancy.", "full_answer_no_ref": "She should not become pregnant until she has undergone regular check-ups and one year has passed with negative beta-HCG levels. Patients will be monitored weekly with hCG dosing until it becomes undetectable, for three consecutive times. After that the monitoring will be monthly for six months and then every two months for another six months before a new pregnancy.", "full_question": "A 24-year-old woman, primigestation, suffers a spontaneous abortion at 7 weeks gestation. The anatomopathological study of the abortive remains indicates molar disease. We should inform you that:", "id": 344, "lang": "en", "options": {"1": "The risk of a new molar gestation in a future pregnancy is 50%.", "2": "She should not become pregnant until periodic check-ups and after one year with negative BHCG levels.", "3": "Subsequent controls are not necessary if the evacuation of the trophoblastic tissue was complete.", "4": "Periodic check-ups are necessary since 40% of cases will develop gestational trophoblastic neoplasia.", "5": NaN}, "question_id_specific": 158, "type": "GYNECOLOGY AND OBSTETRICS", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en036_29141", "title": "Gestational trophoblastic disease", "score": 0.018304351443547716, "content": "In the past, it was seen as important not to get pregnant straight away after a GTD. Specialists recommended a waiting period of 6 months after the hCG levels become normal. Recently, this standpoint has been questioned. New medical data suggest that a significantly shorter waiting period after the hCG levels become normal is reasonable for approximately 97% of the patients with hydatidiform mole. Risk of a repeat GTD The risk of a repeat GTD is approximately 1 in 100, compared with approximately 1 in 1000 risk in the general population. Especially women whose hCG levels remain significantly elevated are at risk of developing a repeat GTD. Persistent trophoblastic disease The term «persistent trophoblastic disease» (PTD) is used when after treatment of a molar pregnancy, some molar tissue is left behind and again starts growing into a tumour. Although PTD can spread within the body like a malignant cancer, the overall cure rate is nearly 100%."}, {"id": "pubmed23n0256_1552", "title": "Persistence of gestational trophoblastic disease for longer than 1 year following evacuation of hydatidiform mole.", "score": 0.018137254901960782, "content": "A spontaneous fall in the radioimmunoassay for the beta subunit of hCG to less than 2 mIU/mL documents regression of hydatidiform mole following evacuation of a molar pregnancy. Continued negative hCG levels for the year after evacuation indicates the absence of risk for persistent gestational trophoblastic disease. This report describes an unusual case of recurrent nonmetastatic gestational trophoblastic disease 16 months after initial evacuation. A 29-year-old woman presented at 19 weeks' gestation with severe preeclampsia and vaginal bleeding. Pelvic ultrasonography demonstrated a molar pregnancy. Pathology following uterine evacuation confirmed a hydatidiform mole. Serial hCG levels fell progressively to less than 2 mIU/mL over the following 25 weeks. She remained compliant with oral contraceptive pills despite having no sexual activity. Sixteen months after uterine evacuation, recurrence of gestational trophoblastic disease was documented by a rising beta-hCG, negative pelvic ultrasound, normal liver function tests, and normal computed tomography of the head. Endometrial curettage showed no chorionic villi or molar tissue. She was treated with five courses of actinomycin D and has remained disease-free for the following 5 years. This late recurrence of gestational trophoblastic disease suggests that those with a molar pregnancy may benefit from surveillance beyond 1 year after uterine evacuation."}, {"id": "pubmed23n0403_4413", "title": "[Gestational trophoblastic tumors and recent clinical information].", "score": 0.018070818070818073, "content": "Recent clinical advances in the field of gestational trophoblastic diseases are described. WHO modified its risk factor scoring system. This change was proposed to combine both the basic FIGO anatomic staging with the modified WHO risk factor scoring system. Patients who score as low-risk are treated with single agent chemotherapy, such as methotrexate (MTX), and patients refractory to MTX are treated with a combination chemotherapy, EMA/CO. Patients who score as high-risk are treated with EMA/CO, and patients refractory to the first line chemotherapy may be successfully treated with EP/EMA. Recent epidemiological data showed that women with complete hydatidiform moles could anticipate normal reproduction in the future. Studies found that pregnancies after treatment of molar pregnancy resulted in 69% full-term, live births; 8% premature deliveries; 1% ectopic pregnancies, and 0.5% stillbirths. First-trimester spontaneous abortions occurred in 17% of pregnancies, and major and minor malformations were detected in 0.4% of infants. Patients with hydatidiform mole were at increased risk of developing molar pregnancy in subsequent conceptions. After having one molar pregnancy, the risk of having molar disease in a future gestation was about 1%. The risk of persistent gestational trophoblastic tumors was increased by long-term oral contraceptive use before conception. In a large, multicenter, case-control study, the risk was shown to be increased in women who had ever used oral contraceptives, but was highest for women taking oral contraceptives during the cycle in which they became pregnant. Partial hydatidiform moles were never previously proven to transform into choriocarcinoma; however, a recent study with molecular techniques clearly showed that partial moles could transform into choriocarcinoma. All patients with suspected partial moles should be reviewed centrally and require hCG follow-up."}, {"id": "pubmed23n0501_5170", "title": "Low risk of relapse after achieving undetectable HCG levels in women with complete molar pregnancy.", "score": 0.0178585665311329, "content": "Complete hydatidiform molar pregnancies occur in approximately 1 of 1,000 conceptions. After uterine evacuation of the trophoblastic tissue, women are followed up with serial serum human chorionic gonadotropin (hCG) measurements. Patients are considered to have attained remission when their hCG level spontaneously declines to an undetectable level and remains there during a 6-month follow-up period. This standard effectively detects all disease recurrence; however, it is resource intensive, delays child bearing, and is subject to significant noncompliance. Our objective was to determine the risk of disease recurrence after hCG spontaneously declines to undetectable levels. We used a database from the New England Trophoblastic Disease Center to analyze hCG levels in patients with complete molar pregnancies. Among 1,029 women with complete molar pregnancy and complete data, 15% developed persistent gestational trophoblastic neoplasia. The rate of persistent neoplasm among those whose hCG level fell spontaneously to undetectable levels was 0.2% (2/876, 95% confidence interval 0-0.8%). No women developed persistent gestational trophoblastic neoplasia after their hCG level fell to undetectable levels using an assay with a sensitivity of 5 mIU/mL (n = 82, 95% confidence interval 0-4.5%). Based on our experience with women with complete hydatidiform molar pregnancies whose hCG values spontaneously fell to undetectable levels after molar evacuation, we conclude that the risk of recurrent neoplasm after hCG levels fall to less than 5 mIU/mL approaches zero."}, {"id": "pubmed23n0482_23918", "title": "Placental site trophoblastic tumor arising from antecedent molar pregnancy.", "score": 0.017704661182922053, "content": "Placental site trophoblastic tumor (PSTT) is a rare form of gestational trophoblastic disease. Little is known about its pathogenesis and natural history. This report describes two cases that arose in patients with documented complete hydatidiform moles and summarizes the antecedent prenatal histories of PSTTs based on a detailed Medline literature analysis. A 28-year-old, G(2)P(2) female had a live, 12-week gestation fetus and a coexisting molar pregnancy. Her hCG levels dropped promptly from 1.5 million to 23,273 IU/ml after termination, but rose shortly thereafter together with the onset of recurrent vaginal bleeding. Curettage revealed persistent mole. Persistently elevated hCG led to hysterectomy disclosing a fundal PSTT. The second case was that of a 48-year-old, G(2) woman who presented with symptoms of preeclampsia, hyperthyroidism, and elevated hCG. Curettage yielded a complete hydatidiform mole. Although the hCG level decreased for a short period, it soon increased despite treatment with methotrexate. A second curettage revealed a PSTT. A Medline literature analysis of PSTT, which consists almost entirely of individual cases and several small series, disclosed that PSTT is preceded in 61% of cases by normal term pregnancy, 12% molar pregnancy, 9% spontaneous abortion, 8% therapeutic abortion, and 3% with ectopic pregnancy, stillbirths or preterm delivery. No information is known in 7%. This report describes two additional cases of PSTT preceded by complete molar pregnancy. PSTT is a well recognized, but uncommon form of gestational trophoblastic disease. Although little is known about its pathogenesis, it is preceded not uncommonly by an abnormal pregnancy, including a molar pregnancy."}, {"id": "pubmed23n0208_14120", "title": "[Studies on the viability of trophoblast after termination of various kinds of pregnancies (author's transl)].", "score": 0.016946778711484593, "content": "Although normal value of hCG (LH level) does not necessarily indicate eradication of viable trophoblast, its confirmation has been demonstrated as a clinically useful guide for the probable prevention of choriocarcinoma after hydatidiform mole by Takeuchi et al. Choriocarcinoma preceded by other pregnancies than hydatidiform mole which has the highest risk for choriocarcinoma has drawn more attention than before in connection with the decrease of postmolar choriocarcinoma. So that I have studied the regression rate of urinary gonadotropin (hCG) after the termination of various kinds of pregnancies. In 2,433 cases of induced abortion, 695 cases of spontaneous abortion, 1,724 cases of term delivery and 43 cases of hydatidiform mole, their urinary hCG were determined to the level of physiological range of LH. The rate of hCG regression was in the order of term delivery, spontaneous abortion, induced abortion and hydatidiform mole. The younger was the gestational age of trophoblast, the slower was the regression of hCG. At one month after the termination of pregnancy, 80.1%, 11%, 0.3%, 8% and 4.1%, and at two month 55.8%, 1.6%, 0.5%, 4% and 0.5% for hydatidiform mole, induced abortion of less than 12 week of gestation, spontaneous abortion of less than 12 week of gestation, spontaneous abortion of between 13 and 20 week of gestation respectively still showed abnormal hCG value. One percent of induced abortion at 5 month, 4% of spontaneous abortion at 3 month, 0.3% of term delivery at 4 month still maintained abnormal titer. No malignant sequelae in patients under the investigation have ever been observed in the follow up period between 3 and 8 years."}, {"id": "pubmed23n0525_17277", "title": "Postevacuation hCG levels and risk of gestational trophoblastic neoplasia in women with complete molar pregnancy.", "score": 0.016124871001031993, "content": "Women diagnosed with complete hydatidiform molar pregnancy are at 15% to 28% risk of developing persistent gestational trophoblastic neoplasia (GTN) requiring further management with chemotherapy. Our objective was to develop human chorionic gonadotropin (hCG) criteria that establish a patient's risk of developing persistent GTN or achieving remission from their baseline risk within a few weeks of molar evacuation. We used a database from the New England Trophoblastic Disease Center to analyze hCG levels from 1,029 women with complete molar pregnancies. We conducted a retrospective cohort study using data from 1973 to 2001. Women whose hCG level declined below 50 mIU/mL during their follow-up were found to be at no more than 1.1% risk for developing persistent GTN, irrespective of when this level was reached. Women whose hCG levels was below 200 mIU/mL in the fourth week after evacuation (59.8% of all women), or below 100 mIU/mL in the sixth week after evacuation (65.8% of all women), had a risk of persistence below 9%. hCG levels above 2,000 mIU/mL in the fourth week after evacuation (13.3% of women) were associated with a 63.8% risk of developing persistent disease. These data may allow clinicians to evaluate the risk of persistence that their patients with complete molar pregnancy have based on early hCG results after molar evacuation. In the fourth week after molar evacuation, 59.8% of women may be counseled that their risk of developing persistent GTN is substantially reduced from their baseline, whereas 13.3% of women may be warned that their risk of developing persistent GTN is greater than 50%. II-2."}, {"id": "pubmed23n0798_17361", "title": "[Reservation of fertility for seventeen patients with placental site trophoblastic tumor].", "score": 0.016076962809917356, "content": "To approach the efficiency and feasibility of preserving the fertility for patients with placental site trophoblastic tumor (PSTT). Totally 2 086 cases of gestational trophoblastic neoplasm (GTN) patients registered in Peking Union Medical College Hospital between 1998 and 2013. Fifty-seven of them were PSTT patients, 40 cases of which suffered hysterectomy, the rest 17 PSTT patients who preserved their fertility were analyzed retrospectively. The computerized database of clinical and pathological reports was reviewed in this cohort. The clinical manifestation of PSTT was not specific compared to other types of GTN. The average age of the 17 patients was 29.5 years old (range 22-39 years). The most common antecedent pregnancy was term birth (8 cases), the others were spontaneous abortion in 4 case, artificial abortion in 3 cases and molar pregnancy in 2 cases. The baseline serum β-hCG was slightly elevated and 12 patients (12/15) were less than 1 000 U/L. In this cohort, nine of the patients were in stage I, while the other eight cases were in stage III . The patients suffered conservative surgery, including dilation and curettage of uterus in 7 cases, open abdomen uterine lesion excision in 4 cases, laparoscopic uterine lesion excision in 3 cases, hysteroscopic uterine lesion excision in 1 case, and pulmonary lobectomy in 2 cases. Two of the patients didn't received chemotherapy, while the other 15 cases suffered combination chemotherapy. Compared with 40 patients who suffered hysterectomy during the same interval, fertility preservation group did not result in poor outcomes or high risk of relapse rate. Six subsequent pregnancies happened after the therapy, two of them were during their second-trimester, while four patients had healthy babies by vaginal delivery in two and cesarean section in two. The scar of the uterus was fairly well during the cesarean sections. Reservation of fertility therapy could be considered in highly-selected patients for young women who strongly desired to preserve their fertility and with localized lesion. Exactitude follow-up after therapy should be recommended. Contraception should also be recommended for at least one year after the chemotherapy. Vaginal delivery could be an option for the future pregnancies."}, {"id": "pubmed23n0383_12290", "title": "Pregnancy outcomes of patients who conceived within 1 year after chemotherapy for gestational trophoblastic tumor: a clinical report of 22 patients.", "score": 0.015362511052166225, "content": "The aim of this study was to explore the risk of pregnancy of patients who conceived within 1 year after successful chemotherapy for gestational trophoblastic tumor (GTT). From 1966 to 1996, 22 patients who conceived within 1 year after chemotherapy were followed up and analyzed retrospectively. Among 22 patients, 9 had term deliveries and 1 had a premature birth, 6 had induced abortion at the patient's request, and 6 had therapeutic abortion because of various indications such as repeated hydatidiform mole (1 case), intrauterine death (1 case), inevitable abortion (1 case), and threatened abortion (3 cases). The fetal loss rate was 27.1% (6/22). The incidence rate of gestational trophoblastic disease (GTD) was 9.1% (2/22). The incidence rate of GTT was 4.5% (1/22). The average interval between completion of chemotherapy and pregnancy was 10.25 months in the group of term pregnancies and 5.86 months in that of fetal loss (P &lt; 0.05), indicating that the longer the interval, the lesser the risk of GTD. The results suggest that contraception for 1 year is necessary in patients with GTT after successful chemotherapy. However, in the case of a patient who conceives within 1 year, it is not necessary to terminate pregnancy, but the pregnancy must be carefully watched."}, {"id": "wiki20220301en036_29137", "title": "Gestational trophoblastic disease", "score": 0.014890085014336737, "content": "The use of a reliable contraception method is very important during the entire follow up period, as patients are strongly advised against pregnancy at that time. If a reliable contraception method is not used during the follow-up, it could be initially unclear to clinicians as to whether a rising hCG level is caused by the patient becoming pregnant again, or by the continued presence of GTD. In women who have a malignant form of GTD, hCG concentrations stay the same (plateau) or they rise. Persistent elevation of serum hCG levels after a non molar pregnancy (i.e., normal pregnancy [term pregnancy], or preterm pregnancy, or ectopic pregnancy [pregnancy taking place in the wrong place, usually in the fallopian tube], or abortion) always indicate persistent GTD (very frequently due to choriocarcinoma or placental site trophoblastic tumour), but this is not common, because treatment mostly is successful."}, {"id": "pubmed23n0847_11859", "title": "Gestational trophoblastic diseases - clinical guidelines for diagnosis, treatment, follow-up, and counselling.", "score": 0.014472455648926238, "content": "Hydatidiform mole is treated with surgical uterine evacuation with suction and blunt curettage (D). Medical uterine evacuation should not be used (C). On clinical suspicion of hydatidiform mole, one representative sample of the evacuated tissue is fixed for histopathologic investigation and one is forwarded unfixed for genetic analysis (D). Serum hCG is measured on suspicion of hydatidiform mole. At the time of the uterine evacuation, the initial hCG is measured (A). After a hydatidiform mole that is both triploid and partial, serum hCG is measured weekly until there are two consecutive undetectable values (&lt; 1 or &lt; 2), after which the patient can be discharged from follow-up (C). After a diploid hydatidiform mole, a complete mole, or a hydatidiform mole without valid ploidy determination, serum hCG is measured weekly until the value is undetectable (&lt; 1 or &lt; 2). If serum hCG is undetectable within 56 days after evacuation, the patient can be discharged from follow-up after an additional four monthly measurements. If serum hCG is first normalised after 56 days, the patient is follow-up with monthly serum hCG measurement for six months. Safe contraception should be used during the follow-up period (A). If hCG stagnates (less than 10% fall over three measurements), increases, or if hCG can be demonstrated for longer than 6 months, the patient by definition has persistent trophoblastic disease (PTD). A chest X-ray should be taken and a gynaecologic ultrasound scanning performed. The patient is referred to oncologic treatment (A). Uterine re-evacuation as a treatment for PTD can, in general, not be recommended because the rate of remission is low, and there is the risk of perforation of the uterus (C). In all following pregnancies, the woman is offered an early ultrasound scan, e.g. in gestational week eight (D). Eight weeks after termination of all future pregnancies, serum hCG is measured (D). In PTD and invasive hydatidiform mole, the primary treatment is MTX, either orally every third week or IV every week (B). In MTX-resistant PTD, IV act D is added (or replaces the MTX) (B). Third line chemotherapy is BEP or EP, alternatively EMA-CO (B). Choriocarcinoma is primarily treated with chemotherapy. Hysterectomy and/or resection of metastases are possible treatments (A). Placental site trophoblastic tumour (PSTT) and epithelioid trophoblastic tumour (ETT) are primarily treated with hysterectomy. In the case of disseminated disease, chemotherapy is considered (A). The risk of reoccurrence after trophoblastic disease treated with chemotherapy is approximately 3%. Most reoccurrences are seen within 12 months, and for this reason monitoring of hCG is recommended for one year, the first third months once or twice a month, thereafter every second to third month. Patients with PSTT and ETT are monitored with measurement of hCG throughout their lifetimes (C). In genetically verified twin pregnancy with hydatidiform mole and a living foetus, the pregnancy can continue if serum hCG is monitored and ultrasound scans regularly performed, and possible obstetric complications dealt with (C). In the case of recurrent hydatidiform mole and/or familial hydatidiform mole, patients should be referred to genetic workup and counselling (C). Women with a hereditary disposition to hydatidiform mole because of a mutation in NLRP7 should be informed of the possibility of becoming pregnant via egg donation (C). "}, {"id": "pubmed23n1164_1142", "title": "Misdiagnosis of gestational trophoblastic neoplasia as ectopic pregnancy: A 15-year retrospective study.", "score": 0.01407199625993455, "content": "Gestational trophoblastic neoplasia is an uncommon disease, whose clinical manifestations are similar to ectopic pregnancy, thus some rare pelvic lesion can be misdiagnosed as ectopic pregnancy. This study was presented to investigate the characteristics of gestational trophoblastic neoplasia misdiagnosed as ectopic pregnancy and reduce the misdiagnosis. The clinicopathological data for 14 cases of gestational trophoblastic neoplasia misdiagnosed as ectopic pregnancy at West China Second Hospital Sichuan University from January 2006 to December 2020 were retrospectively analyzed. The main clinical manifestations were amenorrhea, abnormal vaginal bleeding, and abdominal pain. At initial diagnosis, the serum hCG level was &gt;10,000 mIU/mL in 5 patients and &lt;10,000 mIU/mL in 7 patients, and a positive urine pregnancy test alone was found in 2 patients. Vaginal ultrasonography showed no abnormalities in 7 cases, adnexal mass in 5 cases, and tubal thickening in 2 cases. The patient's previous pregnancy was an abortion in 7 cases, full-term in 4 cases, and a hydatidiform mole in 3 cases. Clinical stage: 3 cases were stage I, 3 were stage II, 7 were stage III, and 1 case was stage IV (liver and spleen metastases). The median FIGO prognostic score was 13.5 points (12-21 points), with 9 cases having a score &gt;13 points (very high risk). From 14 patients, only 3 had molar pregnancy previously. Only 3 patients had no metastasis at GTN diagnosis (from these 3, only one after molar pregnancy). After chemotherapy alone or in combination with surgery, all patients survived, with a median follow-up of 84 months (23-102 months). If we have positive hCG, without a sonographic topic gestation confirmation, associated with metastatic lesions, the GTN diagnosis should be considered instead of ectopic pregnancy, if the patient have had a pregnancy once during her life."}, {"id": "pubmed23n0391_978", "title": "Outcome of subsequent pregnancy after treatment for persistent gestational trophoblastic tumour.", "score": 0.013945278022947925, "content": "This study analysed subsequent pregnancy outcome in patients treated for persistent gestational trophoblastic tumour (GTT). Between 1974 and 1999, a total of 378 patients with GTT (83 patients with high-risk and 295 patients with low-risk GTT) were treated at Chiba University Hospital, Japan. Of these 378 patients, 363 (96.0%) achieved primary remission and 315 survivors have been followed at our hospital. To date, 129 patients have had 243 subsequent conceptions. While pregnancy outcome was comparable with that of the general Japanese population, the incidence of repeat molar pregnancy (2.1%) was approximately seven times higher than that of the general population. During the mandatory HCG follow-up period of 1 year, 15 patients conceived within 6 months of completion of chemotherapy. The incidence of spontaneous abortion in these 15 patients was significantly higher than that in patients who conceived after a waiting period of &gt;6 months (P = 0.0053). Patients treated for GTT may anticipate a normal future reproductive outcome, although it would be better to avoid pregnancy for at least 6 months after completion of chemotherapy."}, {"id": "pubmed23n0080_18466", "title": "Occurrence of molar pregnancy in patients undergoing elective abortion: comparison with other clinical presentations.", "score": 0.013898112133573665, "content": "Clinical data of molar pregnancies found in women undergoing elective abortion (group 1, n = 39) were compared to those of molar pregnancies in women who experienced spontaneous abortions (group 2, n = 157) and women in whom molar pregnancy was discovered before symptoms of spontaneous abortion were evident (group 3, n = 209). Group 1 women were younger and experienced uterine evacuation at an earlier stage of amenorrhea than groups 2 and 3. Group 3 had larger uteri at evacuation and longer intervals of positive tests for the beta-subunit of human chorionic gonadotropin during the postmolar phase as compared with groups 1 and 2. On the basis of available provincial data for the number of elective abortions, the estimated incidence of molar pregnancies in this population was 1:2,699. The presence of malignant gestational trophoblastic neoplasia was documented in a single patient in group 1. The incidence of malignant gestational trophoblastic neoplasia in this group was not significantly different from that in groups 2 and 3. Routine pathologic examination of the products of conception in women undergoing elective abortion coupled with routine assays of the beta-subunit of human chorionic gonadotropin when molar pregnancy is found can identify both noninvasive and invasive trophoblastic disease in these women."}, {"id": "wiki20220301en036_29144", "title": "Gestational trophoblastic disease", "score": 0.013810080106809078, "content": "However, the incidence of rare diseases (such as GTD) is difficult to measure, because epidemiologic data on rare diseases is limited. Not all cases will be reported, and some cases will not be recognised. In addition, in GTD, this is especially difficult, because one would need to know all gestational events in the total population. Yet, it seems very likely that the estimated number of births that occur at home or outside of a hospital has been inflated in some reports. Terminology Gestational trophoblastic disease (GTD) may also be called gestational trophoblastic tumour (GTT). Hydatidiform mole (one type of GTD) may also be called molar pregnancy. Persistent disease; persistent GTD: If there is any evidence of persistence of GTD, usually defined as persistent elevation of beta hCG (see «Diagnosis» below), the condition may also be referred to as gestational trophoblastic neoplasia (GTN). See also Trophoblastic neoplasms References External links"}, {"id": "pubmed23n0789_5549", "title": "Gestational trophoblastic neoplasia: A 6 year retrospective study.", "score": 0.013747165532879819, "content": "To study the clinical presentations of gestational trophoblastic neoplasia and its response to chemotherapy. This is a retrospective study of 28 women of gestational trophoblastic neoplasia evaluated over a period of 6 years from January 2004 to December 2009. Patients were evaluated on the basis of their age, number of deliveries, history of abortion or molar pregnancy, and the treatment received. All patients were scored on the basis of WHO scoring system. Patients with low risk (score &lt;/=6) received single agent chemotherapy with methotrexate or actinomycin D. Patients with high risk (score &gt;/=7) received multiple agent chemotherapy with EMACO regimen. After completion of chemotherapy patients were followed for a minimum of 2 years. The response to treatment was evaluated during follow-up by clinical examination, beta hCG levels and imaging as and when required. Out of 28 women only 27 could be evaluated, because 1 patient was lost to follow-up. Out of 27 patients, 18 patients (66.67%) achieved complete remission with the first-line chemotherapy and additional 25.92% (7/27) achieved complete remission with second line chemotherapy resulting in complete remission of 92.5% (25/27). Gestational trophoblastic neoplasia is curable if patient is properly evaluated and scored. It shows good response to chemotherapy."}, {"id": "pubmed23n0843_545", "title": "Gestational trophoblastic neoplasia after spontaneous human chorionic gonadotropin normalization following molar pregnancy evacuation.", "score": 0.013432323405716248, "content": "To evaluate the risk of gestational trophoblastic neoplasia (GTN) after spontaneous human chorionic gonadotropin normalization in postmolar follow-up. Retrospective chart review of 2284 consecutive cases of hydatidiform mole with spontaneous normalization of hCG following uterine evacuation treated at one of five Brazilian reference centers from January 2002 to June 2013. After hCG normalization, GTN occurred in 10/2284 patients (0.4%; 95% CI 0.2%-0.8%). GTN developed in 9/1424 patients (0.6%; 95% CI 0.3%-1.2%) after a complete hydatidiform mole, in 1/849 patients (0.1%; 95% CI&lt;0.01%-0.7%) after a partial hydatidiform mole, and in 0/13 patients (0%; 95% CI 0%-27%) after a twin molar pregnancy. The median time to GTN diagnosis after hCG normalization was 18months, and no diagnoses were made before six months of postmolar surveillance. Patients who required more than 56days to achieve a normal hCG value had a ten-fold increased risk of developing GTN after hCG normalization (9/1074; 0.8%; 95% CI 0.4%-1.6%) compared to those who reached a normal hCG level in fewer than 56days (1/1210;0.08%; 95% CI&lt;0.01%-0.5%; p=0.008). All patients presented with symptoms at the time of GTN diagnosis. GTN after spontaneous hCG normalization following molar pregnancy is exceedingly rare, and the few patients who do develop GTN after achieving a normal hCG value are likely to be diagnosed after completing the commonly recommended six months of postmolar surveillance. Current recommendations for surveillance after hCG normalization should be revisited."}, {"id": "wiki20220301en073_24425", "title": "Trophoblastic neoplasm", "score": 0.013402216232404911, "content": "Management of GTN requires pathology review, treatment options and monitoring of hCG. Therefore, it can be treated with curettage, hysterectomy and single agent or multi agent chemotherapy. Although this group of diseases are highly susceptible to chemotherapy, prognosis depends on the type of GTN and whether the tumor has spread to other areas of the body. Cause and Risk factors The exact cause of gestational trophoblastic neoplasia (GTN) is unknown. GTN often arises after molar pregnancies but can also occur after any gestation including miscarriages and term pregnancies. Although risk factors may impact on the development of the tumor, most do not directly cause of disease. According to the some studies, the risk of complete molar pregnancy is highest in women over age 35 and younger than 20. The risk is even higher for women over age 45. Signs and Symptoms The symptoms of GTN will vary from person to person. People with the same disease may not have all the symptoms listed."}, {"id": "wiki20220301en036_29140", "title": "Gestational trophoblastic disease", "score": 0.013306543331564183, "content": "In this scoring system, women with a score of 7 or greater are considered at high risk. It is very important for malignant forms of GTD to be discovered in time. In Western countries, women with molar pregnancies are followed carefully; for instance, in the UK, all women who have had a molar pregnancy are registered at the National Trophoblastic Screening Centre. There are efforts in this direction in the developing countries too, and there have been improvements in these countries in the early detection of choriocarcinoma, thereby significantly reducing the mortality rate also in developing countries. Becoming pregnant again Most women with GTD can become pregnant again and can have children again. The risk of a further molar pregnancy is low. More than 98% of women who become pregnant following a molar pregnancy will not have a further hydatidiform mole or be at increased risk of complications."}, {"id": "pubmed23n0834_2417", "title": "Postmolar gestational trophoblastic neoplasia: beyond the traditional risk factors.", "score": 0.012950157880011191, "content": "To investigate the slope of linear regression of postevacuation serum hCG as an independent risk factor for postmolar gestational trophoblastic neoplasia (GTN). Multicenter retrospective cohort study. Academic referral health care centers. All subjects with confirmed hydatidiform mole and at least four measurements of β-hCG titer. None. Type and magnitude of the relationship between the slope of linear regression of β-hCG as a new risk factor and GTN using Bayesian logistic regression with penalized log-likelihood estimation. Among the high-risk and low-risk molar pregnancy cases, 11 (18.6%) and 19 cases (13.3%) had GTN, respectively. No significant relationship was found between the components of a high-risk pregnancy and GTN. The β-hCG return slope was higher in the spontaneous cure group. However, the initial level of this hormone in the first measurement was higher in the GTN group compared with in the spontaneous recovery group. The average time for diagnosing GTN in the high-risk molar pregnancy group was 2 weeks less than that of the low-risk molar pregnancy group. In addition to slope of linear regression of β-hCG (odds ratio [OR], 12.74, confidence interval [CI], 5.42-29.2), abortion history (OR, 2.53; 95% CI, 1.27-5.04) and large uterine height for gestational age (OR, 1.26; CI, 1.04-1.54) had the maximum effects on GTN outcome, respectively. The slope of linear regression of β-hCG was introduced as an independent risk factor, which could be used for clinical decision making based on records of β-hCG titer and subsequent prevention program."}, {"id": "pubmed23n0480_15545", "title": "How long should patients be followed after molar pregnancy? Analysis of serum hCG follow-up data.", "score": 0.012729285262492094, "content": "We analyzed human chorionic gonadotropin (hCG) follow-up data of patients with molar pregnancy. Women often do not complete recommended post-disease screening. Our purpose was to determine if continuing follow up of uncomplicated molar cases beyond attaining undetectable hCG levels is necessary for detecting relapse of gestational trophoblastic disease. One hundred fifty patients treated at Hungarian National Health Center were analyzed. Those who developed persistent disease before hCG had become undetectable were excluded from further analysis (n=24; 16%). Among 126 uncomplicated cases, 72 patients (57%) completed follow up, and 54 (43%) discontinued their protocol before it had been completed. Of 120 patients who achieved at least one undetectable hCG level, none had any evidence of relapse. In uncomplicated hydatidiform mole, our analysis indicates that once undetectable serum hCG levels are attained, relapse is unlikely. Although further monthly checks are advisable, the likelihood of recurrence appears very low."}, {"id": "pubmed23n0705_21494", "title": "Clinical features of early-stage nonhydropic mole for diagnosis of persistent trophoblastic disease.", "score": 0.012593970394481903, "content": "To characterize the clinical features of \"nonhydropic\" hydatidiform mole and to investigate regression of serum human chorionic gonadotropin (hCG) as an aid in detecting persistent trophoblastic disease after nonhydropic hydatidiform mole. Our study included women with histologically diagnosed nonhydropic molar pregnancies. Women did not exhibit macroscopic or characteristic ultrasonographic appearances specific to hydatidiform mole. Regression of serum hCG levels was compared with abortions of nonmolar pregnancies, which were histologically confirmed. Among 34 nonhydropic molar pregnancies, 32 complete hydatidiform moles were analyzed, excluding two partial hydatidiform moles. Compared with nonmolar aborted pregnancies, pre-evacuation hCG levels were significantly higher in the 32 complete hydatidiform moles. The 32 molar pregnancies progressed to 24 cases of spontaneous remission and eight cases of persistent trophoblastic disease. Among patients with spontaneous remission, the time at which serum hCG levels became undetectable and the onset of first postabortion menstruation were similar to those in patients who had nonmolar abortions. In all patients who experienced regression, serum hCG was undetectable after the third postabortion menstruation. In all patients with persistent trophoblastic disease, serum hCG levels exceeded 25 milli-international units/mL 4 weeks after evacuation. Without histological confirmation, it is difficult to diagnose nonhydropic molar pregnancy based solely on clinical presentation. Follow-up studies of serum hCG levels 4 weeks after abortion and after the third postabortion menstruation may aid in detecting impending persistent trophoblastic disease. II."}, {"id": "pubmed23n0557_20206", "title": "Postevacuation hCG levels and risk of gestational trophoblastic neoplasia among women with partial molar pregnancies.", "score": 0.012375591754575616, "content": "To develop human chorionic gonadotropin (hCG) criteria that determine a patient's risk of developing persistent gestational trophoblastic neoplasia (GTN) or achieving remission after partial mole evacuation. We used a database from the New England Trophoblastic Disease Center to analyze hCG levels from 284 women with partial molar pregnancies diagnosed between 1973 and 2003. An hCG level &gt;199 mIU/mL in the third through eighth week following molar evacuation was associated with at least a 35% risk of GTN. Women with partial mole who have elevated hCG levels within the first few weeks after molar evacuation are at increased risk for developing GTN."}, {"id": "pubmed23n0541_9218", "title": "Guidelines following hydatidiform mole: a reappraisal.", "score": 0.012306484982521224, "content": "The aim of this study was to determine how often patients with complete hydatidiform mole (CHM) who spontaneously achieve normal human chorionic gonadotrophin (hCG) levels subsequently develop persistent or recurrent gestational trophoblast disease. Four hundred and fourteen cases of CHM registered at the Hydatidiform Mole Registry of Victoria were reviewed retrospectively after molar evacuation. Maternal age, gestational age, gravidity and parity were determined for each patient, as well as the need for chemotherapy. Among the 414 patients, 55 (13.3%) required chemotherapy for persistent trophoblastic disease. None of the patients whose hCG levels spontaneously fell to normal subsequently developed persistent molar disease. Weekly hCG measurements are recommended for all patients until normal levels are achieved. For patients who attain normal hCG levels within 2 months after evacuation, it seems safe to discontinue monitoring once normal levels are achieved. Patients who fail to achieve normal hCG levels by 2 months after evacuation should be monitored with monthly hCG measurements for 1 year after normalisation to assure sustained remission."}, {"id": "pubmed23n0541_9219", "title": "Persistent trophoblast disease following partial molar pregnancy.", "score": 0.012281494876431584, "content": "Human chorionic gonadotrophin (hCG) follow-up data were analysed retrospectively in all patients registered in the Hydatidiform Mole Registry at the Royal Women's Hospital, Melbourne from January 1992 to January 2001 to determine the risk of persistent trophoblast disease following partial molar pregnancy and to review the present follow-up protocol of patients suffering from partial hydatidiform molar pregnancy (PHM). Demographic factors were determined for all 344 cases with a review diagnosis of PHM, included age, history of previous hydatidiform mole, gestation length, hCG levels and compliance with follow-up. Six of the 344 patients diagnosed with PHM required treatment with single-agent methotrexate and folinic acid rescue. All six patients achieved and maintained a complete biochemical remission after chemotherapy. hCG regression assays were analysed for 235 patients: 225 patients had at least one normal hCG measurement during follow-up, of whom 152 (64.7%) patients obtained normal values within 2 months after evacuation. All patients obtained normal levels within 32 weeks after evacuation of the partial hydatidiform mole. Only 63 (25.6%) patients completed the recommended follow-up program. No patient who achieved normal hCG levels required chemotherapy because of a recurrent gestational trophoblastic tumour. This study indicates that 1.7% of all partial mole pregnancy patients needed treatment for malignant sequelae. In contrast, no patient diagnosed with partial mole had a biochemical or clinical relapse after achieving normal levels of hCG, consistent with previous studies. Patients who have had a partial hydatidiform mole should be followed by hCG assays until normal levels are achieved and then follow-up can be safely discontinued."}, {"id": "pubmed23n0511_9522", "title": "Gestational trophoblastic disease: is intensive follow up essential in all women?", "score": 0.012246273687364242, "content": "To determine the timescale of the registration process for gestational trophoblastic disease and its impact on hCG level at registration and subsequent need for chemotherapy. A prospective observational study using a standardised protocol for registration, assessment and treatment for molar pregnancy. A supra-regional tertiary referral centre for gestational trophoblastic disease. A total of 2046 consecutive women registered between January 1994 and December 1998 with a diagnosis of molar pregnancy. Data at and after registration, collected prospectively on a computerised database, were statistically analysed (by multiple logistic regression and ANOVA). Relationship between length of time to and hCG value at registration; also the subsequent need for chemotherapy. A total of 2046 women with a diagnosis of molar pregnancy were registered in the study period. The mean time interval between first evacuation and registration at the referral centre was 47 days (median 37, range 0-594). One hundred and five out of 2046 (5.1%) women needed chemotherapy. Sixty-three precent of the women (1296 out of 2046) had a normal level of urinary hCG (less than 40 IU/24 hours) at the time of registration and only one (0.08%) needed chemotherapy. Binary logistic regression analysis showed a statistically significant relationship between time to registration, hCG value, histology, pretreatment risk score and decision to administer chemotherapy. Women with gestational trophoblastic disease who were registered late were significantly more likely to have normal levels of hCG and were less likely to need chemotherapy. A less intensive follow up may be justified in women with gestational trophoblastic disease who are registered with a normal hCG level."}, {"id": "pubmed23n0855_2675", "title": "Late spontaneous resolution of persistent molar pregnancy.", "score": 0.012084917162733988, "content": "To determine the outcome of women with persistently raised but falling human chorionic gonadotrophin (hCG) levels 6 months after surgical evacuation of a molar pregnancy. An 11-year retrospective review. The United Kingdom supra-regional trophoblastic disease treatment centres at Weston Park Hospital (Sheffield) and Charing Cross Hospital (London). Women with raised but falling serum human chorionic gonadotrophin (hCG) levels 6 months after evacuation of a molar pregnancy. Retrospective case note review of eligible women identified by the electronic databases held at each supra-regional centre. The proportion of women that attain normal hCG levels spontaneously without chemotherapy. In addition, rates of gestational trophoblastic neoplasia (GTN), drug resistance, disease relapse and overall survival are reported. Thirty-five women with molar pregnancy and raised but falling serum hCG levels continued surveillance 6 months after evacuation. Levels of hCG in 30 of the patients (86%) fell to normal levels spontaneously. One woman defaulted follow up prior to hCG normalisation (3%) and the remaining four women (11%) were treated with chemotherapy due to a plateau or rise in serum hCG levels indicating GTN. All treated women were successfully salvaged by either first (n = 1) or second line (n = 2) chemotherapy or found to have persistently raised low level hCG of uncertain clinical relevance (n = 1). No women developed relapsed disease and overall survival was 100%. Women with a molar pregnancy and a raised but falling hCG level beyond 6 months from uterine evacuation can be safely observed with regular hCG monitoring and can usually avoid potentially toxic chemotherapy. Women with treated molar pregnancy may avoid chemotherapy if 6-month hCG levels are raised but falling."}, {"id": "pubmed23n0263_15299", "title": "Expression of c-erb B-2 oncogene product in persistent gestational trophoblastic disease.", "score": 0.012067379149267464, "content": "Much debate exists on the initiation of chemotherapy for women at risk for persistent gestational trophoblastic disease. This is a result of a lack of early predictors for the development of persistent gestational trophoblastic disease after evacuation of a complete hydatidiform mole, because the only current reliable method of detection and diagnosis lies in persistent or rising postmolar beta-human chorionic gonadotropin values. We used immunocytochemical techniques to retrospectively study the expression of the c-erb B-2 oncogene product in formalin-fixed, paraffin-embedded trophoblastic tissues as a potential indicator of the development of persistent gestational trophoblastic disease. In this retrospective study 56 trophoblastic tumors were examined by means of immunocytochemical techniques to stain for the oncogene product for evidence of c-erb B-2 expression. Our 56 cases included original tissue from 20 cases of complete mole that progressed to persistent gestational trophoblastic disease, seven cases of choriocarcinoma after term pregnancy or abortion, and 29 cases of hydatidiform mole representing postevacuation, spontaneously regressing disease (including one partial mole). We also studied 11 cases of first-trimester trophoblast and 15 cases of term placenta as additional controls. Our results showed positive immunostaining for c-erb B-2 gene product in one case of persistent gestational trophoblastic disease, with negative staining in all other cases in the study groups and controls. Analysis for the significance of c-erb B-2 expression in persistent gestational trophoblastic disease showed that this correlation between c-erb B-2 expression and persistent gestational trophoblastic disease is not significant, suggesting that future efforts should be directed at the involvement of different oncoproteins."}, {"id": "wiki20220301en036_29129", "title": "Gestational trophoblastic disease", "score": 0.011945117029862794, "content": "All five closely related tumours develop in the placenta. All five tumours arise from trophoblastic cells. The trophoblast is the membrane that forms the wall of the blastocyst in the early development of the fetus. In a normal pregnancy, trophoblastic cells aid the implantation of the fertilised egg into the uterine wall. But in GTD, they develop into tumour cells. Cause Two main risk factors increase the likelihood for the development of GTD: 1) The woman being under 20 years of age, or over 35 years of age, and 2) previous GTD. Although molar pregnancies affect women of all ages, women under 16 and over 45 years of age have an increased risk of developing a molar pregnancy.Being from Asia/of Asian ethnicity is an important risk factor. Hydatidiform moles are abnormal conceptions with excessive placental development. Conception takes place, but placental tissue grows very fast, rather than supporting the growth of a fetus."}, {"id": "pubmed23n0344_3977", "title": "Outcome of pregnancies occurring before completion of human chorionic gonadotropin follow-up in patients with persistent gestational trophoblastic tumor.", "score": 0.011854657687991021, "content": "To determine the outcome of pregnancies occurring before completion of human chorionic gonadotropin follow-up in patients treated with chemotherapy for gestational trophoblastic tumor. Retrospective record review of patients with gestational trophoblastic tumor who conceived before standard hCG follow-up was completed during 1973-1998. Forty-three patients treated for gestational trophoblastic tumors conceived before human chorionic gonadotropin follow-up was completed. The antecedent pregnancy was complete mole in 31 (72.1%) and partial mole in 12 (27. 9%) patients. Of the 43 patients, 39 (90.7%) had stage I, 1 had stage II, and 3 had stage III disease. The mean interval from human chorionic gonadotropin remission to new pregnancy was 6.3 months (range 1-11 months). Ten patients underwent elective termination and four patients were lost to follow-up. Of the remaining 29 patients, 22 (75.9%) had term live births, 3 (10.3%) had preterm delivery, 3 had spontaneous abortion, and 1 (3.5%) had a repeat mole. Two cases of fetal anomalies were detected; one was inherited polydactyly and the other was hydronephrosis. One patient developed choriocarcinoma with lung involvement and underwent cesarean section at 28 weeks; a normal fetus was delivered and no choriocarcinoma was detected in the placenta. Pregnancies occurring in patients treated for gestational trophoblastic tumor before standard human chorionic gonadotropin follow-up is completed may continue under close clinical surveillance since the majority have a favorable outcome. However, patients should also be advised of the low but important risk of delayed diagnosis in case tumor relapse develops during early subsequent pregnancy."}, {"id": "article-22233_9", "title": "Gestational Trophoblastic Disease -- Epidemiology", "score": 0.011255411255411256, "content": "Risk factors for molar pregnancy include extremes of age, ethnicity, and a prior history of a HM, suggesting a genetic etiology. The risk of a complete mole is higher for women older than 35 years and younger than 21 years and 7.5 times higher for women older than 40 years. The risk of repeat molar pregnancy in women with a history of molar pregnancy is approximately 1% which is 10 to 20 times the risk in the general population. [1] Interestingly, a history of prior spontaneous abortion has been reported to confer a 2- to 3-fold increased risk of molar pregnancy compared to women without a history of spontaneous abortion. [3] Following 2 molar gestations, the risk of having a third mole is > 10%. [7] It is also important to note that malignant transformation such as choriocarcinoma or placental-site trophoblastic tumor can occur following any pregnancy."}, {"id": "pubmed23n0568_17404", "title": "Shortened duration of human chorionic gonadotrophin surveillance following complete or partial hydatidiform mole: evidence for revised protocol of a UK regional trophoblastic disease unit.", "score": 0.011123136123136123, "content": "Following hydatidiform mole, women are at increased risk of persistent gestational trophoblastic neoplasia (pGTN) and are therefore monitored using serum human chorionic gonadotrophin (hCG) concentration measurements. We retrospectively evaluated the policy of extended (2 year) follow up for women with hCG concentrations returning to normal &gt;56 days after evacuation. Of 6701 women registered for hCG follow up, 422 (6%) developed pGTN, 412 (98%) of these women presented within 6 months after evacuation. Three developed pGTN at 402, 677 and 1267 days after evacuation following spontaneous normalisation of hCG levels. Only one woman was detected by routine extended follow up. Prolonged surveillance after molar pregnancy causes significant anxiety and is not cost-effective. Therefore, the current revised protocol comprises hCG follow up for 6 months after spontaneous return of hCG levels to normal for all women."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[143, 334]], "word_ranges": [[26, 57]], "text": "Microhematuria without associated symptoms may be a finding without pathologic significance and must be confirmed in a subsequent new sediment (although I do not know if at 15 days or later)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The correct answer is 4. What is not very clear to me is \"routine urinalysis\" because it is not usually done in an asymptomatic healthy child. Microhematuria without associated symptoms may be a finding without pathologic significance and must be confirmed in a subsequent new sediment (although I do not know if at 15 days or later).", "full_answer_no_ref": "[HIDDEN] What is not very clear to me is \"routine urinalysis\" because it is not usually done in an asymptomatic healthy child. Microhematuria without associated symptoms may be a finding without pathologic significance and must be confirmed in a subsequent new sediment (although I do not know if at 15 days or later).", "full_question": "A 10-year-old girl comes for a health check-up. Physical examination is normal with weight and height in the 50th percentile and BP 109/65. A routine urinalysis shows a specific gravity of 1035 pH6 blood 2+ with no protein. Urine sediment shows 5-10 red blood cells per field. What would be the most appropriate course of action?", "id": 43, "lang": "en", "options": {"1": "Determination of creatinine and nitrogen in blood.", "2": "Refer the child for cystoscopy.", "3": "Determine antinuclear antibodies and complement.", "4": "Repeat urine sediment in 15 days.", "5": "Abdominal computed axial tomography."}, "question_id_specific": 151, "type": "PEDIATRICS", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0510_13943", "title": "[Acute tubulointerstitial nephritis in children].", "score": 0.017862838915470493, "content": "Acute tubulointerstitial nephritis (ATIN) is a rare renal disorder in children. Patients usually present non-specific symptoms and signs so that the diagnosis of ATIN is often delayed. The disease may be infection- or drug-induced or it may occur without a known cause. Early recognition and appropriated therapy usually lead to an excellent prognosis. The aim of the study was to describe clinical and laboratory findings and the course of ATIN in 21 patients, that are typical enough to enable early recognition of the disease as it is potentially reversible. Between 1986 and 1997 we observed 21 patients, aged 7-16 years (mean, 12.8), with acute tubulointerstitial nephritis, including eight with tubulointerstitial nephritis and uveitis (TINU syndrome). Laboratory studies included urinalysis, complete blood count, erytrocyte sedimentation rate (ESR), plasma creatinine, glomerular filtration rate (GFR), electrolytes, proteins, IgG, C3, C4 antinuclear-antibodies (ANA), antistreptolysin-O and antibodies to hantaviruses. Renal ultrasound was done in all patients. Renal biopsy was performed in 5 children. All children had previously been healthy. The symptoms of ATIN developed within a few days (Table 1). The most common initial symptoms were fatigue, fever, gastrointestinal disturbances, anorexia and weight loss. Less common complaints included headache, arthralgias and maculopapular rash. On addmition no patient had hypertension, oedema or evidence of acute infection. ESR, plasma urea and creatinine concentrations were increased in all, plasma proteins and IgG levels in the majority of patients. ANA were negative in 15 pts in whom this analysis was performed; C3 and C4 levels were normal. In two children recent strepococcal and in the other 6 hantavirus infection was serologicaly proved. All patients had non-oliguric acute renal failure (ARF): GFR was 21.7 +/- 8 9 in 14 pts and 67 +/- 9.7 in 7 pts. Low urine specific gravity (1003-1014), mild proteinuria (0.3-0.4 g/24 h), leukocyturia and/or haematuria were found in all patients; glycosuria, aminoaciduria and decreased tubular reaposrption of phosphate (TRP) were found in 12/21, 9/21 and 9/14 patients, respectively. Urine cultures were negative in all children. Renal US demonstrated enlarged hyperechoic kidneys in 11 pts, in remaining 10 pts no abnormalities were found. Renal biopsy, performed in 5 children, confirmed the diagnosis of ATIN. Eight patients subsequently developed anterior uveitis as part of TINU syndrome. Treatment included supportive therapy in all and six patients received prednisolone for 4-8 weeks (40-60 mg/m2/24 h for 10-14 days with subsequent reduction of dose over several weeks). Anterior uveitis was successfully treated with topical steroids. Renal function completely recovered in all patients: GFR (109 +/- 22.6 ml/min) within a mean interval of 47 +/- 33 days, concentration ability within 2-12 (mean 4.5) months. Common clinical features of ATIN are non-oliguric acute renal failure of various degrees, signs of tubular dysfunction, proteinuria, haematuria, leukocyturia and absence of hypertension. All our patients had normal blood pressure, non-oliguric renal failure, proteinuria, hypostenuria and abnormal urinary sediment; about half of them had glycosuria and/or other signs of proximal tubular dysfunction. The most important causes of ATIN in children reported in literature are systemic infections and drugs. However, the cause of ATIN in our patients was assessed as being related to infection only in 8 patients and to diclofenac in one. No infection, drug, toxin or other cause could be identified in 4, as well as in 8 pts with TINU syndrome. The prognosis of ATIN in children is considered to be favourable, but some patients may develop chronic renal failure. Renal function completely recovered in all our patients; that is consistent with outcome data from the most reports. Acute tubulointerstitial nephritis is an important cause of ARF in children, its aetiology may be different and it carries an excellent prognosis. ATIN should be suspected in a child who presents typical, although non-specific symptoms and signs, associated with lukocyturia and/or microhaematuria, signs of tubular dysfunction and unexplained renal failure. The diagnosis can be verified at renal biopsy. Early recognition of the disease is important to remove possible aetiologic agents and to treat them before chronic lesions are present to avoid long-term renal damage."}, {"id": "pubmed23n0417_5268", "title": "Cases from the Osler Medical Service at Johns Hopkins University. Antiglomerular basement membrane disease.", "score": 0.01609322974472808, "content": "A 47-year-old Taiwanese man with no notable medical history was admitted with low-grade fevers and night sweats that had persisted for 5 to 6 weeks. An extensive investigation at another hospital could not determine the cause of the fevers, but documented acute renal failure with a blood urea nitrogen level of 60 mg/dL and a serum creatinine level of 5.6 mg/dL. He was admitted to the Johns Hopkins Hospital for further evaluation.The patient, who had been living in the United States for the past 20 years, reported no recent travel and no behaviors that are associated with transmission of human immunodeficiency virus. He was not taking any medications, and he denied using herbal or nutritional supplements. He had no recent weight loss. There were no specific complaints on review of systems. On physical examination, he was a thin, middle-aged man in no distress. Vital signs included a temperature of 37.5 degrees C, a blood pressure of 166/86 mm Hg, a pulse of 70 beats per minute, a respiratory rate of 16 breaths per minute, and 99% oxygen saturation on room air. Sclera were anicteric, and he had no palpable adenopathy. His lungs were clear, and his heart rate was regular without extra sounds. His abdomen was thin, nontender, and without masses or organomegaly. There was no edema or signs of embolism in the extremities. Laboratory studies revealed a white blood cell count of 14,200/mL(3), a hematocrit of 23.1%, and a platelet count of 456,000/mL(3). Blood chemistries were notable for a blood urea nitrogen level of 61 mg/dL and a serum creatinine level of 7.6 mg/dL. Levels of aminotransferases, total bilirubin, and alkaline phosphatase were within normal limits. Urinalysis revealed large hemoglobin, 1+ protein, numerous red blood cells, and 3 to 5 white blood cells. Numerous red blood cell casts were seen on microscopic examination of the urine sediment. The patient's erythrocyte sedimentation rate was &gt;130 mm/h, and his C-reactive protein level was elevated at 12.6 mg/dL. Serologies were negative for antinuclear antibodies and antineutrophil cytoplasmic antibodies; serum complement levels were normal. What is the diagnosis?"}, {"id": "pubmed23n0620_809", "title": "Microscopic hematuria is associated with low bone mineral density in aged women and men.", "score": 0.016064516129032258, "content": "Little is known concerning renal or urological risk factors for osteoporosis. The aim of this study was to explore an association between urinalysis and bone mineral density (BMD) in 4,835 Japanese adults. Participants were 4,835 individuals (female 3,683; male 1,152) aged 50 years and over who received a health check-up between January 1995 and March 2006 in Japan. BMD of the distal radius and ulna of the non-dominant forearm was measured by the dual-energy X-ray absorptiometry (DXA) method using a DTX-200 Dexacare osteometer (Osteometer MediTech A/S, Rødovre, Denmark). Urine variables were protein, and red blood cells (RBCs) and white blood cells (WBCs) in urine sediment (categorized as &lt;1, 1-4, 5-9, or &gt;or=10 cells per high-power microscopic field). Average age was 58.9 years (SD 5.6) in women, and 60.5 years (SD 6.2) in men. Simple linear regression analysis showed that urinary RBCs were associated with BMD in women (beta = or-5.88 x 10(-3), R (2) = 0.004, p &lt; 0.0001) and men (beta = or-1.34 x 10(-2), R (2) = 0.013, p = 0.0001). These associations held when possible confounders were adjusted (beta = or-2.05 x 10(-3), R (2) = 0.001, p = 0.0338 for women and beta =or -5.67 x 10(-3), R (2) = 0.006, p = 0.0163 for men). Microscopic hematuria is associated with forearm BMD in women and men aged 50 years and over. Further studies should be conducted to determine the mechanisms underlying this association."}, {"id": "pubmed23n0324_186", "title": "Limited evaluation of microscopic hematuria in pediatrics.", "score": 0.015154994259471873, "content": "The purpose was to determine the value of the standard laboratory and radiologic evaluation of microscopic hematuria in children, and to determine the prevalence of idiopathic hypercalciuria in those children referred for evaluation of unexplained microscopic hematuria. This was a retrospective study of 325 children referred from 1985 to 1994 for the evaluation of asymptomatic microscopic hematuria. The diagnostic studies reviewed included serum creatinine, blood urea nitrogen, serum electrolyte studies, serum complement concentration, antinuclear antibody, urinalysis, urine calcium to creatinine ratios, urinary protein to creatinine ratio and/or 24-hour urinary protein excretion, renal ultrasounds, intravenous pyelograms, voiding cystourethrograms, and historical information. All creatinine and electrolyte values were normal for age, and none of the biochemical tests obtained in the children with hypercalciuria was abnormal. Of the 325 patients with idiopathic microscopic hematuria, only 18 had abnormal renal ultrasound examinations and 9 voiding cystourethrograms showed low-grade reflux. Hypercalciuria was found in 29 patients. The family history was positive for urolithiasis in 16% of patients without hypercalciuria compared with 14% of patients with hypercalciuria. A positive family history of hematuria was reported in 25% of patients; 62 patients did not have hypercalciuria and 4 of the patients had hypercalciuria. Microscopic hematuria in children is a benign finding in the vast majority of children. Our data demonstrate that a renal ultrasound, voiding cystourethrogram, cystoscopy, and renal biopsy are not indicated in the work-up of microscopic hematuria, and microhematuria in the otherwise healthy child is a minimal health threat, rarely indicative of serious illness."}, {"id": "pubmed23n1014_25815", "title": "[Adrenal hemorrhage in a patient with systemic lupus erythematosus].", "score": 0.014421936524503059, "content": "A 58-year-old female was referred to our department with intermittent suffocation for 1.5 years, aggravated for a month. 1.5 years before she developed oral ulcer, raynaud phenomenon, proteinuria, bilateral pleural effusion, ANA and anti-dsDNA positive. This patient was diagnosed with systemic lupus erythematosus (SLE). After given hormones, hydroxychloroquine sulfate (HCQ), her symptom relieved soon. The patient stopped her pills 1 year ago. One month ago, she had chest tightness, increased urine foam, and suffered from oliguria. Her admission medical examination: blood pressure (BP) 130/80 mmHg, conjunctiva pale, and lower lung breath sounds reduced. There was no tenderness, rebound pain and abdominal muscle tension in the abdomen. Liver and spleen rib inferior, mobile dullness negative, and lower extremity edema. Blood routine tests were performed with hemoglobin (HGB) 57 g/L. Urine routine: BLD (3+). 24-hour urinary protein 3.2 g. serum albumin 20.5 g/L, C-reactive protein (CRP) 12.85 mg/L, erythrocyte sedimentation rate (ESR) 140 mm/h. Antinuclear antibody (ANA) (H)1:10 000, anti-dsDNA antibody 1:3 200; anti-Smith antibody, anti-U1-snRNP/Sm antibody were positive, blood complement 3(C3) 0.43 g/L, complement 4(C4) 0.07 g /L. Anticardiolipin antibody (ACL), anti-β2-GP1, lupus anticoagulant (LA) were negative; HRCT suggested bilateral medial pleural cavity product liquid. Admission diagnosis: SLE lupus nephritis, anemia, pleural effusion, and hypoproteinemia. We treated her with methylprednisolone 1 000 mg×3 d, late to 48 mg/d and cyclophosphamide 1.0 g, HCQ 0.2 g bid, gamma globulin 10 g×5 d. Day 2 of treatment, this patient developed acute right upper quadrant pain, not accompanied by nausea, vomiting, blood stool and diarrhea. Antipyretic antispasmodic treatment was invalid, after the morning to ease their own abdominal pain. Day 4 of treatment, daytime blood HGB 77 g/L. Bilateral renal vascular ultrasound: bilateral renal artery blood flow velocity was reduced. The abdominal pain of the above symptoms recurred at night, BP was 120/80 mmHg, and no positive signs were found on abdominal examination. No abnormality was found in the vertical abdominal plain film. Blood routine examination: HGB 53 g/L, Plasma D dimer 2 515 μg/L, amylase in hematuria was normal, the stool occult blood was negative. Abdominal computed tomography (CT): normal structure of right adrenal gland disappeared, irregular mass shadow could be seen in adrenal region, CT value was about 50 HU. Morphological density of left adrenal gland was not abnormal. The retroperitoneum descended along the inferior vena cava to the right iliac blood vessel and showed a bolus shadow. The density of some segments increased. The lesion involved the right renal periphery and reached the left side of abdominal aorta. Most lesions surrounded the inferior vena cava, the right renal vein and part of the small intestine. The boundary between the upper lesion and the vena cava was unclear. Iodinecontaining contrast agent was taken orally. No sign of contrast agent overflowing was found in the abdominal cavity. Hematoma and exudative changes were considered in retroperitoneum. CONCLUSION of contrast-enhanced ultrasound of blood vessels: The retroperitoneal inferior vena cava (volume 3.5 cm×3.5 cm×1.5 cm) was hypoechoic and had no blood flow lesion. The adrenal gland had a high possibility of origin. Left renal vein thrombosis extended to inferior vena cava. According to the above data, it was analyzed that the cause of retroperitoneal hematoma of the patient was left adrenal vein thrombosis caused by hypercoagulable state, which led to vascular rupture and hemorrhage caused by increased vascular pressure in adrenal gland. Therefore, on the basis of continuing to actively treat the primary disease, and on the basis of dynamic observation of no active hemorrhage for 3 days, the anticoagulant therapy was continued with 10 mg/d of apixaban. Clinical symptoms were gradually eased, HGB did not decrease. Two weeks later, the ultrasonic examination showed that the irregular cluster hypoechoic range behind the inferior vena cava was significantly smaller than that before (1.8 cm×1.2 cm×0.7 cm). Abdominal CT examination after 1 month showed that there was no abnormal morphological density of bilateral adrenal glands and basic absorption of retroperitoneal exudation. Adrenal hemorrhage is uncommon. SLE with adrenal hemorrhage is rarer. In SLE patients, especially those complicated with APS, if abdominal pain accompanied by HGB decrease occurs, except after gastrointestinal hemorrhage, the possibility of adrenal hemorrhage should be warned."}, {"id": "pubmed23n0392_8074", "title": "Atherosclerotic Renovascular Disease.", "score": 0.014250493096646943, "content": "The patient, a 78-year-old Asian male, was brought to the hospital because of acute shortness of breath that had progressively worsened over the course of the day. He complained of a nonproductive cough and claudication after walking 1 block. His past medical history was significant for mild renal insufficiency (serum creatinine 1.5--2.0 mg/dl), the etiology of which was never explored. Although there was a recent history of mild to moderate hypertension, at presentation his blood pressure was noted to be 240/118 mm Hg in both arms. His physical exam at the time of admission was remarkable for grade II hypertensive retinopathy, an S4 gallop, periumbilical systolic bruits, audible femoral arterial bruits and absent distal lower extremity pulses. Initial complete blood count, serum electrolytes and cardiac enzymes (including lactate dehydrogenase) were normal. His blood urea nitrogen and serum creatinine concentrations were 51 and 3.6 mg/dl, respectively, and his urinalysis showed 1+ protein (both by dipstick and sulfasalicylic acid) with a \"benign\" sediment (0--1 WBC/HPE, 1--2 RBCs/HPF) with occasional granular casts. His electrocardiogram, apart from demonstrating left ventricular hypertrophy with secondary ST-T wave abnormalities, showed no acute changes; his chest X-ray demonstrated cardiomegaly and pulmonary vascular congestion. He was intubated and subsequently treated with increasing parenteral doses of furosemide (40--240 mg) and a nitroglycerine drip (up to 15 mcg/min). Over the course of the first 48 h, his blood pressure was gradually lowered to 170/100 mm Hg. His urine output increased from 20 ml/h to 125/ml/h, and his respiratory status improved, allowing him to be extubated. In spite of adequate control of his blood pressure in the ensuing days (150--170/80--90 mm Hg), his renal function continued to deteriorate. Renal sonography (without Doppler) demonstrated a right kidney of 9.6 cm and a left kidney of 9.3 cm in length without evidence of hydronephrosis. Both kidneys were noted to be echogenic. Assays for antinuclear antibodies and antineutrophilic cytoplasmic antibodies were negative, and the patient's serum complement levels were normal. For several days after his admission, his serum creatinine gradually rose to 10.7 mg/dl, and hemodialysis was initiated for uremic encephalopathy. Because of the high index of suspicion for renal artery stenosis as the case of both his hypertension and renal failure, a renal angiogram was performed. It revealed a 90% occlusion of the right renal artery with ostial involvement and a 70% occlusion of the left renal artery; both kidneys had poor distal renal vasculature and there was marked atherosclerotic disease of the aorta. After being hemodialyzed for 3 treatments, his renal function began to improve spontaneously. His serum creatinine returned to 3.4 mg/dl, and a subsequent 24-hour urine demonstrated a creatinine clearance of 20 ml/min and an excretion of 1.2 g of protein. Following his discharge from the hospital, his renal function remained unchanged for 3 years, and his blood pressure was easily controlled on monotherapy with a long-acting calcium channel blocker. He recently died from pneumonia."}, {"id": "article-22283_9", "title": "Diffuse Proliferative Glomerulonephritis -- Evaluation", "score": 0.01404869640163758, "content": "A complete blood count showing possible anemia and low platelet count followed by renal function tests with elevated serum creatinine (0.4 mg/dl above the upper limit), blood urea nitrogen levels, and urine analysis positive for urine sediments: red blood cells and casts, white blood cells, granular casts are indicative of a glomerular pathology. For further confirmation, a 24 hours urine protein to creatinine ratio and 24 hours urine sample for protein levels can be done. A protein count of greater than 3.5 g/day is suggestive of nephrotic range proteinuria, which is associated with a worse prognosis. A 24-hour urine sample can be used to calculate creatinine clearance to estimate the eGFR. Renal ultrasound can be done to see the size and confirm the presence of two kidneys and the absence of any obstructive pathology resulting in hydronephrosis. Serum complement (C3 and C4) levels help determine the etiology; low levels are associated with the presence of SLE, cryoglobulinemia, and infectious etiology. [12]"}, {"id": "pubmed23n0064_4794", "title": "Usefulness of scanning procedures for diagnosis of fever of unknown origin in children.", "score": 0.013066349906668929, "content": "During a 5-year study period, 109 patients were referred to a large children's hospital for evaluation of prolonged fever of unknown origin, defined as temperature greater than or equal to 38 degrees C (100.4 degrees F) for 3 weeks or longer and negative findings on initial examination. A two-phase protocol of outpatient followed by inpatient diagnostic studies was instituted for most patients. Confirmed diagnoses were achieved in just 36 of these children (33%) in the following disease categories: infectious, 24 (22%); autoimmune, 7 (6%); and neoplastic, 2 (2%). Scanning or special procedures and the number with positive results (in parentheses) were as follows: abdominal ultrasonography, 43 (8); abdominal computed tomography, 14 (3); indium scan 11 (5); gallium scanning, 4 (1), upper gastrointestinal tract series, 13 (2); technetium bone scanning 15 (2); bone marrow examination, 16 (1); and cranial computed tomography, 7 (0). These studies rarely led to an unsuspected diagnosis. It appears most appropriate in evaluating fever of unknown origin in children to obtain only basic laboratory studies such as a complete blood cell count, urinalysis and culture, chest radiograph, tuberculin skin test, and, in the older child, an antinuclear antibody titer. When these test results are negative, almost all children can be observed clinically for progression of illness or a focus that might then direct specific diagnostic procedures."}, {"id": "pubmed23n0075_21536", "title": "[Laboratory tests in primary care medicine: \"essential laboratory tests\" (1). Urinalysis].", "score": 0.013063266511482471, "content": "Japan Society of Clinical Pathology has formed a committee dealing with \"lab. tests in primary care medicine\". As the first step, they made \"Essential Lab. Tests\" which were composed of simple qualitative bed-side tests such as urinalysis, Complete blood count (CBC: Hb, Ht, WBC, RBC), CRP, or ESR (Erythrocyte sedimental rate), A/G ratio and biochemical tests if necessary (Table 1). We have performed \"Essential Lab. Tests\" on 1,026 outpatients who visited General Medicine Clinic for the first time. They consisted of 456 male (age 13-81), and 526 female (age 10-85). This report is the result of urinalysis from \"Essential Lab. Tests\" of 1,026 patients. 1) The result showed that overall positivity of the urinalysis was 21.3% (when more than one item of the qualitative tests was positive). 2) There was distinctive difference in the positivity of the urinalysis between the sex; i.e. protein and glucose were about twice frequently positive in male, where as occult blood and WBC (Esterase reaction) were 2-3 times more positive in female. 3) Urine protein shows positive in the individual 10-20 yrs old and more than 50 yrs old in both sexes. 4) Glucose was positive in over 40 yrs in male, and occult blood, 40-50 yrs or older in male. 5) WBC shows positive in all age groups in female and 50 yrs or older in male. 6) Positive WBC patients did not necessarily reflect urinary infection in female but nitrites roughly corresponded with urinary WBC in male of 50 yrs or older, meaning probable urinary infection associated with prostatic hypertrophy. 7) Abnormality of urinary sediment corresponded to the positive occult blood and WBC Erastase. 8) Urinalysis is an useful method of screening in primary care medicine."}, {"id": "wiki20220301en003_107194", "title": "Appendicitis", "score": 0.012315806644554401, "content": "Blood and urine test While there is no laboratory test specific for appendicitis, a complete blood count (CBC) is done to check for signs of infection. Although 70–90 percent of people with appendicitis may have an elevated white blood cell (WBC) count, there are many other abdominal and pelvic conditions that can cause the WBC count to be elevated. Due to its low sensitivity and specificity, on its own, WBC is not seen as a good indicator of appendicitis. A urinalysis generally does not show infection, but it is important for determining pregnancy status, especially the possibility of an ectopic pregnancy in women of childbearing age. The urinalysis is also important for ruling out a urinary tract infection as the cause of abdominal pain. The presence of more than 20 WBC per high-power field in the urine is more suggestive of a urinary tract disorder."}, {"id": "wiki20220301en022_61054", "title": "Hematuria", "score": 0.012287697533599172, "content": "The first step in evaluation of red or brown colored urine is to confirm true hematuria with urinalysis and urine microscopy, where hematuria is defined by three of more red blood cells per high power field. Although a urine dipstick test may be used, it can give false positive or false negative results. In gathering information, it is important to inquire about recent trauma, urologic procedures, menses, and culture-documented urinary tract infection. If any of these are present, it is appropriate to repeat a urinalysis with urine microscopy in 1 to 2 weeks or after treatment of the infection. If the results of the urinalysis and urine microscopy reveal a glomerular origin of hematuria (indicated by proteinuria or red blood cell casts), consultation of a nephrologist should be made. If the results of the urinalysis indicate a non-glomerular origin, a microbiological culture of the urine should be performed, if it has not been done already. If the culture is positive, treatment of the"}, {"id": "wiki20220301en022_61059", "title": "Hematuria", "score": 0.012061679040548258, "content": "a female less than 50 years old or a male less than 40 years old; has 3-10 red blood cells per high power field; has not had microscopic hematuria before; and has no other risk factors for urothelial cancer. To be in the intermediate risk category, one must satisfy any of the following criteria: Has smoked 10-30 pack-years; is a female 50-59 years old or a male aged 40-59 years old; has 11-25 red blood cells per high power field; or was previously a low-risk patient with persistent microscopic hematuria and has 3-25 red blood cells per high power field. To be in the high risk category, one must satisfy any of the following criteria: Has smoked more than 30 pack-years; is older than 60 years of age; or has above 25 red blood cells per high power field on any urinalysis. For the low risk category, the next step is to either repeat a urinalysis with urine microscopy in 6 months or perform a cystoscopy and renal ultrasound. For the intermediate risk category, the next step is to perform a"}, {"id": "wiki20220301en057_61758", "title": "Nephritic syndrome", "score": 0.011691002367797947, "content": "Blood urea nitrogen (BUN) - Also measured using a BMP or CMP, blood urea nitrogen is an indicator of how much nitrogen is in the blood at the time of the phlebotomy. The kidney is responsible for excreting nitrogenous substances in the urine, so an elevated BUN usually indicates that the kidney is not functioning appropriately. Urine Analysis (Urinalysis) - After the patient provides a urine specimen, it is sent to the lab for analysis using a variety of methods including urine dipstick testing and microscopic examination. Because the kidney is responsible for making urine, analyzing the urine directly can provide crucial data that can help the physician diagnose nephritic syndrome. Some findings on urinalysis that are consistent with nephritic syndrome include red blood cells (hematuria), red blood cell casts, proteinuria, and possibly white blood cells (pyuria)."}, {"id": "pubmed23n0396_2510", "title": "Clinical utility of a rapid test for uristatin.", "score": 0.011033478893740904, "content": "Uristatin is a trypsin inhibitor present in urine that is increased in most patients with bacterial or viral infections and in many with inflammatory disorders. We included the assay of uristatin as part of a screening program carried out by pediatricians on 4207 Japanese schoolchildren to judge the ability of uristatin to identify those with an infection and (or) inflammation of any cause. We used urine dipsticks for the assay of uristatin, creatinine, albumin, blood, leukocyte esterase, and protein. We also performed quantitative assays for uristatin and creatinine. Another aim was to estimate the reference range for uristatin in schoolchildren, ages 5 to 14 yr. We prepared dipstick pads that were impregnated with a chromogenic substrate for trypsin and measured the uristatin-caused inhibition of trypsin in urine. We measured creatinine so that the ratio of uristatin to creatinine could be calculated to correct for urine concentration. We obtained quantitative uristatin and creatinine results for 4207 children. Of these, 177 had an abnormal urine dipstick for albumin or blood or protein or leukocyte esterase or a combination of these. We used data from 3622 children to establish the reference range for the uristatin dipsticks. The 3622 were diagnosed by their pediatricians as free from an infection or inflammation of any cause and with normal urine dipstick tests. We recommend an upper reference limit for uristatin by dipstick of &lt; or = 7.5 mg uristatin/g creatinine. The leftover 408 children ( [4207-3622-177] = 408) fell into two groups: 205 with diagnoses of no infection, possible infection, or possible inflammatory disorders. The remaining 203 children were renal disease follow-up cases. The diagnoses were based on a physical examination, microscopic urinalysis plus urine dipstick tests for albumin, blood, creatinine, protein, leukocyte esterase and a complete blood count. In the 205 children, 46 had an abnormal uristatin dipstick test, 39 had an abnormal uristatin by immunoassay, 41 had an abnormal erythrocyte sedimentation rate (ESR), 27 had an abnormal serum C-reactive protein (CRP), and one had an abnormal urine microscopic exam. For the first 938 children in the study, the agreement was 93% of negative dipstick uristatin results and immunoassays. The agreement of positive uristatin dipsticks with immunoassays was 85%. We assumed that the immunoassay results were correct. In the evaluation of 189 children with fever, 62 also had an abnormal uristatin by dipstick. A rapid dipstick test for uristatin read on a reflectance photometer gave values that compared well with a quantitative immunoassay method. The uristatin test is sensitive but not specific for any cause of infection or inflammation. Uristatin is easy to determine and appears to be a better indicator than fever, ESR, or CRP for the diagnosis of an infection or inflammation."}, {"id": "wiki20220301en001_26330", "title": "Nephrology", "score": 0.011021580068537557, "content": "Diagnosis History and physical examination are central to the diagnostic workup in nephrology. The history typically includes the present illness, family history, general medical history, diet, medication use, drug use and occupation. The physical examination typically includes an assessment of volume state, blood pressure, heart, lungs, peripheral arteries, joints, abdomen and flank. A rash may be relevant too, especially as an indicator of autoimmune disease. Examination of the urine (urinalysis) allows a direct assessment for possible kidney problems, which may be suggested by appearance of blood in the urine (hematuria), protein in the urine (proteinuria), pus cells in the urine (pyuria) or cancer cells in the urine. A 24-hour urine collection used to be used to quantify daily protein loss (see proteinuria), urine output, creatinine clearance or electrolyte handling by the renal tubules. It is now more common to measure protein loss from a small random sample of urine."}, {"id": "InternalMed_Harrison_1673", "title": "InternalMed_Harrison", "score": 0.01100948509485095, "content": "Fever >38.3°C (101°F) on at least two occasions 2. Illness duration of ≥3 weeks 3. 4. Diagnosis that remains uncertain after a thorough history-taking, physical examination, and the following obligatory investigations: determination of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level; platelet count; leukocyte count and differential; measurement of levels of hemoglobin, electrolytes, creatinine, total protein, alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, creatine kinase, ferritin, antinuclear antibodies, and rheumatoid factor; protein electrophoresis; urinalysis; blood cultures (n = 3); urine culture; chest x-ray; abdominal ultrasonography; and tuberculin skin test (TST). The range of FUO etiologies has evolved over time as a result of changes in the spectrum of diseases causing FUO, the widespread Percentage of Cases Due to Indicated Cause"}, {"id": "wiki20220301en057_61744", "title": "Nephritic syndrome", "score": 0.010579710144927536, "content": "Signs and symptoms that are consistent with nephritic syndrome include: Hematuria (red blood cells in the urine) Proteinuria (protein in the urine) ranging from sub-nephrotic (<3.5 g/day) to >10 g/day, although it is rarely above nephrotic range proteinuria levels. Hypertension resting blood pressure is persistently at or above 130/80 or 140/90 mmHg. Blurred vision Azotemia (increased plasma Urea and Creatinine) Oliguria (low urine output <400 ml/day) Red blood cell casts (seen with urine analysis and microscopy) Pyuria (white blood cells or pus in the urine) Causes"}, {"id": "wiki20220301en012_140488", "title": "Nephrotic syndrome", "score": 0.010557563242127001, "content": "Along with obtaining a complete medical history, a series of biochemical tests are required in order to arrive at an accurate diagnosis that verifies the presence of the illness. In addition, imaging of the kidneys (for structure and presence of two kidneys) is sometimes carried out, and/or a biopsy of the kidneys. The first test will be a urinalysis to test for high levels of proteins, as a healthy subject excretes an insignificant amount of protein in their urine. The test will involve a 24-hour bedside urinary total protein estimation. The urine sample is tested for proteinuria (>3.5 g per 1.73 m2 per 24 hours). It is also examined for urinary casts, which are more a feature of active nephritis. Next a blood screen, comprehensive metabolic panel (CMP) will look for hypoalbuminemia: albumin levels of ≤2.5 g/dL (normal=3.5-5 g/dL). Then a Creatinine Clearance CCr test will evaluate kidney function particularly the glomerular filtration capacity. Creatinine formation is a result of"}, {"id": "InternalMed_Harrison_1704", "title": "InternalMed_Harrison", "score": 0.010181114453396159, "content": "Fever ˜38.3° C (101° F) and illness lasting ˜3 weeks and no known immunocompromised state History and physical examination Stop antibiotic treatment and glucocorticoids Obligatory investigations: ESR and CRP, hemoglobin, platelet count, leukocyte count and differential, electrolytes, creatinine, total protein, protein electrophoresis, alkaline phosphatase, AST, ALT, LDH, creatine kinase, antinuclear antibodies, rheumatoid factor, urinalysis, blood cultures (n=3), urine culture, chest x-ray, abdominal ultrasonography, and tuberculin skin test"}, {"id": "pubmed23n0076_14175", "title": "Routine serologic tests in the differential diagnosis of the adult nephrotic syndrome.", "score": 0.010161779575328614, "content": "From 1980 to 1985, we performed biopsies on 87 adults with nephrotic syndrome (NS). The patients were tested for whether serologic studies obtained routinely at biopsy added to clinical diagnostic accuracy. Using history, physical examination, complete blood cell count (CBC), chemistry panel, urinalysis, and urine creatinine and protein, four nephrologists each predicted whether the patient had primary NS (PNS) or secondary NS (SNS), and the most likely histopathologic entity. Six months later, each nephrologist used this information, with results of tests of sera for fluorescent antinuclear antibody (FANA), rheumatoid factor (RF), complement components, hepatitis B surface antigen (HBsAg), venereal disease research laboratory serology (VDRI), cryoglobulins and protein electrophoresis (SPEP), with an erythrocyte sedimentation rate (ESR) and protein electrophoresis of the urine (UPEP), to make identical predictions. Histopathology was established by renal biopsy. We analyzed the concordance between nephrologists' choices and biopsy results both before and after serologic tests were available with a kappa statistic. Preserology concordance was moderate (kappa = 0.52), and identical to postserology concordance (kappa = 0.51) for both PNS versus SNS and actual histopathology. Serologies were rarely abnormal without clinical suspicion. These results suggest routine serologic testing does not improve diagnostic accuracy in adult NS."}, {"id": "InternalMed_Harrison_3372", "title": "InternalMed_Harrison", "score": 0.010054301713897845, "content": "PART 2 Cardinal Manifestations and Presentation of Diseases HEMATURIA Proteinuria (>500 mg/24 h), Dysmorphic RBCs or RBC casts Pyuria, WBC casts Urine culture Urine eosinophils Hemoglobin electrophoresis Urine cytology UA of family members 24 h urinary calcium/uric acid IVP +/Renal ultrasound As indicated: retrograde pyelography or arteriogram, or cyst aspiration Cystoscopy Urogenital biopsy and evaluation Renal CT scan Renal biopsy of mass/lesion Follow periodic urinalysis Renal biopsy FIguRE 61-2 Approach to the patient with hematuria. ANCA, antineutrophil cytoplasmic antibody; ASLO, antistreptolysin O; CT, computed tomography; GBM, glomerular basement membrane; IVP, intravenous pyelography; RBC, red blood cell; UA, urinalysis; VDRL, Venereal Disease Research Laboratory; WBC, white blood cell."}, {"id": "InternalMed_Harrison_3385", "title": "InternalMed_Harrison", "score": 0.009993002711449313, "content": "FIguRE 61-3 Approach to the patient with proteinuria. Investigation of proteinuria is often initiated by a positive dipstick on routine urinalysis. Conventional dipsticks detect predominantly albumin and provide a semiquantitative assessment (trace, 1+, 2+, or 3+), which is influenced by urinary concentration as reflected by urine specific gravity (minimum, <1.005; maximum, 1.030). However, more exact determination of proteinuria should employ a spot morning protein/creatinine ratio (mg/g) or a 24-h urine collection (mg/24 h). FSGS, focal segmental glomerulosclerosis; RBC, red blood cell; UPEP, urine protein electrophoresis."}, {"id": "wiki20220301en218_35381", "title": "Cholesterol embolism", "score": 0.009900990099009901, "content": "Tests for inflammation (C-reactive protein and the erythrocyte sedimentation rate) are typically elevated, and abnormal liver enzymes may be seen. If the kidneys are involved, tests of kidney function (such as urea and creatinine) are elevated. The complete blood count may show particularly high numbers of a type of white blood cell known as eosinophils (more than 0.5 billion per liter); this occurs in only 60-80% of cases, so normal eosinophil counts do not rule out the diagnosis. Examination of the urine may show red blood cells (occasionally in casts as seen under the microscope) and increased levels of protein; in a third of the cases with kidney involvement, eosinophils can also be detected in the urine. If vasculitis is suspected, complement levels may be determined as reduced levels are often encountered in vasculitis; complement is a group of proteins that forms part of the innate immune system. Complement levels are frequently reduced in cholesterol embolism, limiting the use"}, {"id": "pubmed23n0237_310", "title": "[Asymptomatic microhematuria].", "score": 0.009900990099009901, "content": "Microscopic haematuria is an urinary finding more and more frequently observed in routine analysis in childhood; the diagnostic problems, most of which unresolved, are yet the principal questions of concern. We have studied 123 patients during five years from 1975 to 1980, and followed them for 1-8 years (mean 4 years). Our experience allowed us to consider \"normal\" or without remarkable pathologic significance a urinary finding less than or equal to 5.000 RBC/m' at the Addis count. Such findings were pointed out in 55 cases (44%); 27 patients (22%) had 5-10.000 RBC/m', 34 cases (27,6%) presented 10-50.000 RBC/m' and 7 cases (5,7%) had more then 50.000 RBC/m'. The familiar background, the clinical, biological and immunological data, the roentgenographic investigations and the renal biopsy carried out in the 4 groups of patients, led us to the following conclusions: 1) 26% of the 123 cases had a \"unexplained;; microscopic haematuria with complete lack of anamnestic data, symptomatology and with normal biological findings. 2) in 65,8% of the cases it was possible to discover frequent upper respiratory tract infections (and allergy in 5,6% of them). 3) in 26% of the patients we discovered a previous or actual genito-urinary (10,5% and 15,5% respectively) infection: 9,4% of 96 urography demonstrated a variable degree of nephro-urological abnormalities. 4) 23 children (18,7%) was selected for renal biopsy, primarily by the hypocomplementemia and positive anti-DNA ab. test, and secondly by elevated degree of microhematuria. The histological and immunohistochemical studies demonstrated the presence of mesangial proliferation glomerulonephritis with IgG-IgA-C3 deposits in 7 cases (30% of the cases biopsied and 5,6% of the total), 69,6% of the cases had only minimal charges with negative immunofluorescence. 5) A mean follow-up of 4 years in two groups of patients (less than or greater than 6 years of age) has demonstrated that microscopic haematuria remains unchanged in 18-19% of both groups. A more marked improvement or normalization has been documented in the children more than 6 years aged (p less than 0,001) while a worsening has been observed in the children less than 6 years aged (p less than 0,005), with a statistically significant difference between the two groups considered."}, {"id": "wiki20220301en074_14199", "title": "Microhematuria", "score": 0.009873537566781634, "content": "Microhematuria, also called microscopic hematuria (both usually abbreviated as MH), is a medical condition in which urine contains small amounts of blood; the blood quantity is too low to change the color of the urine (otherwise, it is known as gross hematuria). While not dangerous in itself, it may be a symptom of kidney disease, such as IgA nephropathy or Sickle cell trait, which should be monitored by a doctor. The American Urological Association (AUA) recommends a definition of microscopic hematuria as three or more red blood cells per high-power microscopic field in urinary sediment from two of three properly collected urinalysis specimens. Microhematuria is usually asymptomatic, and there are medical guidelines on how to handle asymptomatic microhematuria (AMH) so as to avoid problems such as overtreatment or misdiagnosis. See also Proteinuria Hematuria Myoglobinuria Hemoglobinuria References External links 2012 AUA Guidelines"}, {"id": "pubmed23n0235_2713", "title": "[Rationalization of urine analysis while maintaining diagnostic accuracy (author's transl)].", "score": 0.00980392156862745, "content": "Screening 720 morning urinary samples for WBC, RBC and protein by test-strip, the number of subsequent microscopic examinations of urinary sediment was reduced to about half, without missing any significant number of clinically significant findings (4.4%). On the other hand, a large number (21.3%) of cases with obviously false-negative sediment findings were revealed. These were largely due to lysis of WBC and RBC, as well as poorly standardized methods of examining urinary sediment. But they could also have been due to differences in subjective criteria employed by the technicians. Our results indicate that using test-strips for screening, clinical routine examination can be rationalized, taking about half the time needed for sediment examination, with more potentially significant findings being discovered than missed."}, {"id": "pubmed23n0043_16875", "title": "[Clinical and laboratory correspondence to outpatients with the extreme value of C-reactive protein].", "score": 0.009708737864077669, "content": "It is the policy of Tenri Hospital to notify the patient promptly whenever an extreme laboratory data value is detected. We investigated the utility of forwarding clinical and laboratory correspondence to outpatients with extreme value of C-reactive protein (CRP). Sixty-eight outpatients with CRP levels more than 20 mg/dl detected during 1986 were studied. CRP was measured by turbidometric method, and a sample with CRP level more than 15 mg/dl was diluted with CRP negative serum (CRP level less than 0.2 mg/dl) and was reanalyzed. Fifty-two of 68 patients (76%) had infectious diseases as the causal disease of high CRP, and eight (12%) had other diseases. In the remaining (12%) the causes were unknown. In most patients the causal diseases were diagnosed within one or two days, but diagnosis required more than 4 days in those with acute pyelonephritis, meningitis, liver abscess or renal abscess, as these diseases were diagnosed after the examination of urine or cerebrospinal fluid, or after ultrasonography. Thirty-seven of 58 patients (64%) who had appointments with their physician on the day of the laboratory examination were admitted the same day, and two of 10 patients (20%) who had appointments on the following day were admitted on that day. Seventeen of 25 patients (68%) with urea-N levels more than 30 mg/dl, cholinesterase levels less than 0.7 delta pH and albumin levels less than 3.5 g/dl required more than 15 days to recover, while 29 of 32 patients (91%) with only 2 or fewer of these laboratory values required less than 14 days. The prompt notification of extreme CRP value is an important aspect of medical care. The examination of urine and cerebrospinal fluid and ultrasonography are necessary screening techniques accompanying examination of blood and plain chest X-ray. Urea-N, cholinesterase and albumin values should be determined at the same time as CRP value to assess prognosis."}, {"id": "wiki20220301en254_18252", "title": "Urine test strip", "score": 0.009615384615384616, "content": "Urinary sediment During routine screening, if a positive test for leukocytes, blood, protein, nitrite, and a pH greater than 7 is identified, the urine sediment be microscopically analysed to further pinpoint a diagnosis. Automated analysers Automatic analysis of urine test strips using automated urine test strip analysers is a well-established practice in modern-day urinalysis. They can measure calcium, blood, glucose, bilirubin, urobilinogen, ketones, leukocytes, creatinine, microalbumin, pH, ascorbic acid and protein. References Further reading Compendium Urinalysis: Urinalysis with Test Strips. Dr E F Hohenberger, Dr H Kimling (2002)http://www.diavant.com/diavant/servlet/MDBOutput?fileId=1392 Urinalysis Strips Instructions Urine tests"}, {"id": "pubmed23n0213_1876", "title": "[Critical study of microscopic hematuria disclosed by screening tests].", "score": 0.009615384615384616, "content": "Asymptomatic microscopic haematuria detected by dipstick in 0.28 p. 100 of 95,200 men was confirmed in only 0.11 p. 100. These results lead to the study of 272 cases to determine the value of dipsticks as a screening test and the benefits of complementary investigations to exclude symptomless diseases. Microscopic haematuria was confirmed in 65 p. 100 but results of other tests reduce the false positive screening results to 25.3 p. 100. Addis counting of at least 5,000 RBC/min is preferable to RBC count/ml which is dilution dependent. 77 p. 100 of microscopic haematuria are intermittent and can only be diagnosed by repeated Addis counts. In the absence of confirmed haematuria, only investigations to exclude proteinuria and urinary infections are justified."}, {"id": "article-25100_41", "title": "Microalbuminuria -- Evaluation", "score": 0.009605144589170148, "content": "In order to determine the cause of albuminuria as the underlying glomerular disease, the following should be done: Urine microscopy – Presence of dysmorphic red blood cells/casts 24-hour urine collection for the quantification of albumin excretion Serum creatinine, blood glucose, albumin, and cholesterol Autoantibody panel - If indicated by the clinical picture, test for antinuclear antibody (ANA), antistreptolysin O titers, anti-DNA antibodies, antineutrophil cytoplasmic antibodies (ANCA), cryoglobulins, anti-glomerular basement membrane (anti-GBM) antibodies, and complement levels (C3 and C4) Imaging studies in albuminuria may include the following: Renal ultrasonography – To establish glomerular disease as the cause of microalbuminuria, it is imperative to look at the echogenicity and size of the kidneys [18] Chest X-ray or computed tomography (CT) scan – If guided by the clinical picture"}, {"id": "wiki20220301en254_18211", "title": "Urine test strip", "score": 0.009523809523809525, "content": "Blood Blood may be present in the urine either in the form of intact red blood cells (hematuria) or as the product of red blood cell destruction, hemoglobin (hemoglobinuria). Blood present in large quantities can be detected visually. Hematuria produces cloudy red urine, and hemoglobinuria appears as a clear red specimen. Any amount of blood greater than five cells per microliter of urine is considered clinically significant; visual examination cannot be relied upon to detect the presence of blood. Microscopic examination of the urinary sediment shows intact red blood cells, but free hemoglobin produced either by hemolytic disorders or lysis of red blood cells is not detected. Therefore, chemical tests for hemoglobin provide the most accurate mean for determining the presence of blood. Once blood has been detected, the microscopic examination can be used to differentiate between hematuria and hemoglobinuria."}, {"id": "pubmed23n0399_3685", "title": "Laboratory and imaging studies used by French rheumatologists to determine the cause of recent onset polyarthritis without extra-articular manifestations.", "score": 0.009433962264150943, "content": "The cause of recent onset polyarthritis can be difficult to identify. To determine which laboratory and imaging studies French rheumatologists recommend, not taking cost into account, for the diagnosis of recent onset polyarthritis without extra-articular manifestations. From the list of the French Society for Rheumatology, a random sample of 210 rheumatologists was selected, who were asked to complete a questionnaire on the laboratory and imaging studies they would recommend in two fictional cases of recent onset polyarthritis (possible rheumatoid arthritis (RA)-case 1 and probable RA-case 2). In case 1, the following were recommended by over 75% of respondents: hand radiographs, rheumatoid factors (RFs), and antinuclear antibodies (ANA) (92%, 98%, and 98%, respectively). 50-74% of respondents recommended radiographs of the feet, knees, and chest (50%, 57%, and 66%, respectively); blood cell counts, erythrocyte sedimentation rate (ESR), serum assays of C reactive protein (CRP), aspartate aminotransferase (AST) and alanine aminotransferase (ALT) (65%, 74%, 67%, and 62%, respectively). 25-49% recommended determination of creatinine and proteinuria, HLA-B27, antikeratin antibody, radiographs of the pelvis, and synovial fluid analysis. Several investigations were recommended less often in case 2 than in case 1. Nevertheless, some laboratory and imaging studies (radiographs of hand, feet, knees, chest x rays, blood cell counts, ANA, RF, antikeratin antibody, CRP, ESR, creatinine, AST and ALT, proteinuria, and joint aspiration) were recommended by more than 25% of respondents in both cases. Wide variations were found among rheumatologists, indicating a need for standardisation. Some laboratory and imaging studies are recommended by at least 25% of respondents in recent onset polyarthritis with or without clues suggesting RA. In contrast, many tests were considered useful by fewer than 25% of the respondents in both cases."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 69]], "word_ranges": [[0, 11]], "text": "Orthosis. Cobb angle between 25º - 45º. Immature skeleton (Risser 0)."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Orthosis. Cobb angle between 25º - 45º. Immature skeleton (Risser 0).", "full_answer_no_ref": "Orthosis. Cobb angle between 25º - 45º. Immature skeleton (Risser 0).", "full_question": "A 13-year-old female, with no relevant history, with menarche 3 months ago, followed since the age of 10 years by idiopathic scoliosis that has worsened. In the physical examination she presents a hump of 7 degrees in the Adams test and in the scoliogram a thoracolumbar curve T4-L1 of 35 degrees of Cobb and a Risser 0. The correct attitude to take will be:", "id": 616, "lang": "en", "options": {"1": "Recommend swimming and revision in three months.", "2": "Prescribe a corset-type orthosis.", "3": "Refer to physiotherapy for spine elastification.", "4": "Review in 6 months with a new X-ray.", "5": NaN}, "question_id_specific": 119, "type": "TRAUMATOLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0573_15298", "title": "The natural history of idiopathic scoliosis.", "score": 0.019334952363160844, "content": "Background. The natural history of idiopathic scoliosis is a crucial issue in the planning and assessment of different treatment methods. This article presents the evaluation of scoliotic deformity in immature patients who have been in observation without any treatment. Material and methods. 159 patients (128 girls, 31 boys) were examined between 1971 and 2002. Skoliosis was diagnosed at a mean age of 6 years 4 months (range 2.1-8.10), and observation was concluded at a mean age of 16 years 11 months (range 14.6-20.3). The mean follow-up was 10 years 5 months. The prognostic factors analyzed were: age, sex, Cobb angle, Mehta angle, apical vertebral rotation, specific rotation, Risser test. The progression and regression of curvature was analyzed in different biological age periods, and was measured by calculating the difference in the Cobb angle on successive x-rays divided by the interval between x-rays. Results. The mean progression of curvature before age 5 was 5.7 degrees per year; in the 6-10 age bracket, 2.3 degrees per year; in the 11-15 age bracket, 7.4 degrees per year; in the &gt;15 age bracket, 0.3 degrees per year. The mean progression for patients with Risser 1 was 8.8 degrees per year; Risser 2, 7.3 degrees per year; Risser 3, 5.1 per year; Risser 4, 2.1 degrees per year; Risser 5, 0.3 degrees per year. Conclusions. The progression of curvature in idiopathic scoliosis is variable, and is influenced by age. Knowledge of the natural history of idiopathic scoliosis is a crucial tool in predicting the development of spinal curvature. The Risser test and biological age are the only effective predictors of progression."}, {"id": "pubmed23n0825_810", "title": "Effectiveness of brace treatment for adolescent idiopathic scoliosis.", "score": 0.01899189918991899, "content": "Effectiveness of brace treatment for adolescent idiopathic scoliosis (AIS) was demonstrated by the BrAIST study in 2013. Objectives of this study were to confirm its effectiveness by analyzing our results and to clarify the factors affecting the results of the treatment. According to the Scoliosis Research Society AIS brace studies standardization criteria, patients with age 10 years or older, Risser 0 to II, less than 1 year post-menarche, curve magnitude 25 to 40 degrees before brace treatment and who received no prior treatment were included in the study. At skeletal maturity, the rate of the patients whose curve was stabilized, exceeded 45 degrees, and who were recommended or underwent surgery were investigated. Additionally, initial correction rate by the brace and factors affecting the results were investigated. A total of 33 patients (27 females and 6 males) could be followed-up until their skeletal maturity and included in the analysis. An average age was 11.9 years, average Cobb angle was 30.8°, and Risser sign was 0 in 13 patients, I in 5, and II in 15 patients before treatment. There were 13 thoracic curves, 14 thoracolumbar or lumbar curves, and 6 double curves. Initial correction rate by the brace was 53.8% for the total curves. In terms of curve pattern, 34.4% for thoracic curve, 73.9% for thoracolumbar or lumbar curve, and 48.8% for double curve. After an average follow-up period of 33 months, 8 patients improved in more than 6 degrees, change of 17 patients were within 6 degrees, and 8 progressed in more than 6 degrees. Therefore, totally, 76% (25/33) of the curves were stabilized by the treatment. Four curves (12%) exceeded 45 degrees and one patient (3%) underwent surgery. Our results were better than the reported natural history. Factors that affected the results were hump degree before treatment and initial correction rate by the brace. 76% of the curve with AIS could be stabilized by brace treatment. Brace treatment was effective for treatment of AIS. Factors affecting the results were hump degrees and initial correction rate."}, {"id": "pubmed23n0258_10528", "title": "[The Schroth scoliosis-specific back school--initial results of a prospective follow-up study].", "score": 0.017496229260935144, "content": "The prospective study reported here was instituted in 1987 to obtain more detailed data on the efficacy of scoliosis-specific spinal rehabilitation after Schroth. Inclusion criteria were 1) idiopathic scoliosis, 2) Risser stage &lt; 4, 3) no treatment with corset or electrical stimulation, 4) first examination between 1 and 3 years postoperatively, 5) usable total X-rays taken with the patient standing not more than 6 months prior to admission. A total of 181 scoliosis patients with an average age of 12.76 years and an average Cobb angle of 27 degrees were included in the study. The average Risser's sign was 1.4 and the average follow-up period was 33 months. No cases of relative progression (annual increase in curvature of 5 degrees or more) were observed. For the purpose of comparison with the spontaneous course, the patients were grouped by age and severity of scoliosis. Both the absence of any relative progression as well as direct comparison of the development of scoliosis under therapy with the spontaneous course confirmed the efficacy of the stationary rehabilitation programme notably in cases with poor prognosis, i.e. with large scoliosis angles and unfavourable curvatures."}, {"id": "pubmed23n0726_2853", "title": "Effectiveness of the Charleston night-time bending brace in the treatment of adolescent idiopathic scoliosis.", "score": 0.01562595373252762, "content": "Part-time or night-time bracing has been introduced to address the poor compliance and psychological burden of full-time bracing. The results of various bracing methods vary, however, due to a lack of consistent inclusion criteria and definitions of brace effectiveness. We have evaluated the effectiveness of the Charleston night-time bending brace in the treatment of adolescent idiopathic scoliosis based on the new standardized criteria proposed by the Scoliosis Research Society. To be included in this study, patients met the following criteria proposed by the Scoliosis Research Society: diagnosis of adolescent idiopathic scoliosis, age 10 years and older when the orthosis was prescribed, Risser 0-2, a primary curve magnitude of 25 to 40 degrees, and no prior treatment. A total of 95 patients (87 girls, 8 boys) were included. At skeletal maturity, 80 patients (84.2%) had 5 degrees or less curve progression and 15 (15.8%) had 6 degrees or more progression. Seven patients (7.8%) were recommended to undergo or underwent surgery before skeletal maturity. Eleven patients (12.6%) progressed beyond 45 degrees. According to these 3 criteria, the Charleston night-time brace was successful in 74 patients (77.9%). Depending on curve type, we observed success rates of 78.3% (47/60) for double, 71.4% (15/21) for thoracic, 83.3% (5/6) for thoracolumbar, and 87.5% (7/8) for lumbar curves. Success rates of 80.0% (36/45) and 76.0% (38/50) were observed in patients with curve magnitudes at bracing of 25 to 30 degrees and 31 to 40 degrees, respectively. Patients with high apex curves had a 67.6% (23/34) success rate, and those with low apex curves had 83.0% (39/47) success rate. Brace success rates among patients with initial Risser signs of 0, 1, and 2 were 68.8% (22/32), 80.6% (25/31), and 84.4% (27/32), respectively. Compared with the results of previous natural history and conventional brace study, the Charleston night-time bending brace is effective for the treatment of adolescent idiopathic scoliosis. Level VI."}, {"id": "pubmed23n0321_19357", "title": "Preliminary results and worst-case analysis of in patient scoliosis rehabilitation.", "score": 0.01552757793764988, "content": "The purpose of this study was to assess the effectiveness of a scoliosis-specific rehabilitation programme as it is carried out in the Katharina Schroth Spinal Deformities Rehabilitation Center. Physiotherapy in the treatment of scoliosis patients is still regarded as ineffective since the study by the American Orthopedic Association in 1941, which showed that general exercises could not influence the natural history of scoliosis. However, specific exercise programmes were not known in the USA at that time. This preliminary study started in 1989 with the following inclusion criteria: (1) diagnosis of idiopathic scoliosis; (2) risser sign &lt; 4; (3) no treatment other than physiotherapy; (4) first control after 1-3 years during repeated in patient treatment; (5) standing AP radiograph taken not more than 6 months before the first in patient treatment. A total of 181 scoliosis patients, with an average age of 12.7 years and an average angle of curvature of 27% according to Cobb, were included in this study. The average risser sign was 1.4, the average follow-up 33 months. The Cobb angle of the major curve was measured in a standardized way. The results of our preliminary study were compared to natural history as known from literature. For the worst-case analysis additionally a questionnaire was sent to the non-repeaters treated at our centre at the same time (1989 and 1990) as the patient sample described above, taking into account the same inclusion criteria for this patient sample except point 4. Results showed that progression as usually defined (increase in curvature of 5 degrees or more per year) has not been found in the preliminary study. The patient sample of this study was divided into different age groups and different groups of curve magnitude, for comparison with other studies. Additional to the patient sample of the preliminary study, 116 of the patients from the years 1989 and 1990 fulfilled the inclusion criteria of the preliminary study with the exception of point 4. These patients formed the questionnaire sample for the worst-case analysis showing that the progression rate of the 181 patients from the preliminary study and the 116 patients of the questionnaire sample together was still better than natural history even if all drop-outs were considered to be failures. The fact that there was no relative progression in our patients sample treated solely by physiotherapy (preliminary study), seems to show the effectiveness of the inpatient rehabilitation programme even in cases with a bad prognosis, severe angles of curvature and unfavourable curvature patterns. A worst-case analysis does not prevent this conclusion, even if all dropouts from the non-repeaters group were considered as failures."}, {"id": "pubmed23n0830_21807", "title": "Mild angle early onset idiopathic scoliosis children avoid progression under FITS method (Functional Individual Therapy of Scoliosis).", "score": 0.015423410743607788, "content": "Physiotherapy for stabilization of idiopathic scoliosis angle in growing children remains controversial. Notably, little data on effectiveness of physiotherapy in children with Early Onset Idiopathic Scoliosis (EOIS) has been published.The aim of this study was to check results of FITS physiotherapy in a group of children with EOIS.The charts of the patients archived in a prospectively collected database were retrospectively reviewed. The inclusion criteria were:diagnosis of EOIS based on spine radiography, age below 10 years, both girls and boys, Cobb angle between 118 and 308, Risser zero, FITS therapy, no other treatment (bracing), and a follow-up at least 2 years from the initiation of the treatment. The criterion for curve progression were as follows: the Cobb angle increase of 68 or more, for curve stabilization; the Cobb angle was 58 comparing to the initial radiograph,for curve correction; and the Cobb angle decrease of 68 or more at the final follow-up radiograph.There were 41 children with EOIS, 36 girls and 5 boys, mean age 7.71.3 years (range 4 to 9 years) who started FITS therapy. The curve pattern was single thoracic (5 children), single thoracolumbar (22 children) or double thoracic/thoracolumbar (14 children), totally 55 structural curvatures. The minimum follow-up was 2 years after initiation of the FITS treatment, maximum was 16 years, mean 4.8 years). At follow-up the mean age was 12.53.4 years. Out of 41 children, 10 passed pubertal growth spurt at the final follow-up and 31 were still immature and continued FITS therapy. Out of 41 children, 27 improved, 13 were stable, and one progressed. Out of 55 structural curves, 32 improved, 22 were stable and one progressed. For the 55 structural curves, the Cobb angle significantly decreased from 18.085.48 at first assessment to 12.586.38 at last evaluation,p&lt;0.0001, paired t-test. The angle of trunk rotation decreased significantly from 4.782.98 to 3.282.58 at last evaluation, p&lt;0.0001,paired t-test.FITS physiotherapy was effective in preventing curve progression in children with EOIS. Final postpubertal follow-up data is needed."}, {"id": "pubmed23n0345_12855", "title": "[Primary correction of scoliosis with the Wilmington corset].", "score": 0.015093273035613341, "content": "As part of our study, the effectiveness and patient's acceptance of the Wilmington-brace is to be evaluated. The effectiveness can be documented with the help of the primary correction achieved, especially in light of the fact, that the primary correction and the long-term results are directly dependant upon one another. We examined a total of 52 patients with an idiopathic scoliosis treated in a thermoplast brace. The group consisted of 38 female and 14 male patients (average age 11.6 years). The angulation was measured with the help of the Cobb-angle and the rotation with the method described by Nash and Moe. The skeletal age was classified according to Risser's-sign. The angle determinations were carried out at three separate points in time--at first presentation, prior to bracing and four to six weeks following bracing. The patients presented with an average angulation of 31 degrees. The average correction achieved in the Wilmington-brace was 41%. This corresponds to a correction of 13 degrees. The best primary correction (45%) was obtained in the thoracolumbar spine. Those patients with the smallest deformity at the onset of treatment showed the best results. The scoliosis with a large primary deformity and a marked rotation of the vertebral bodies responded poorly to correction. Advanced age or skeletal maturity were also limiting factors. Physical therapy had a positive influence on the amount of primary correction obtained. The Wilmington-brace (thermoplast) allows for a good primary correction of idiopathic scoliosis. The simplicity of application and the low production costs are also positive attributes."}, {"id": "pubmed23n0637_18882", "title": "Effectiveness of complete conservative treatment for adolescent idiopathic scoliosis (bracing and exercises) based on SOSORT management criteria: results according to the SRS criteria for bracing studies - SOSORT Award 2009 Winner.", "score": 0.014421936524503059, "content": "The SRS criteria give the methodological reference framework for the presentation of bracing results, while the SOSORT criteria give the clinical reference framework for an appropriate bracing treatment. The two have not been combined in a study until now. Our aim was to verify the efficacy of a complete, conservative treatment of Adolescent Idiopathic Scoliosis (AIS)according to the best methodological and management criteria defined in the literature. Study Design. Retrospective study. Population. We included all AIS patients respecting the SRS inclusion criteria (age 10 years or older; Risser test 0-2; Cobb degrees 25-40 degrees ; no prior treatment; less than one year post-menarchal) who had reached the end of treatment since our institute database start in 2003. Thus we had 44 females and four males, with an age of 12.8 +/- 1.6 at the commencement of the study. Methods. According to individual needs, two patients have been treated with Risser casts followed by Lyon brace, 40 with Lyon or SPoRT braces (14 for 23 hours per day, 23 for 21 h/d, and seven for 18 h/d at start), and two with exercises only (1 male, 1 female): these were excluded from further analysis. Outcome criteria. SRS (unchanged; worsened 6 degrees or more; over 45 degrees at the end of treatment; surgically treated; two years' follow-up); clinical (ATR, Aesthetic Index, plumbline distances); radiographic (Cobb degrees); and ISICO (optimal; minimal). Statistics. Paired ANOVA and t-test, Tukey-Kramer and chi-square test. Median reported compliance during the 4.2 +/- 1.4 treatment years was 90% (range 5-106%). No patient progressed beyond 45 degrees , nor was any patient fused, and this remained true at the two-year follow-up for the 85% that reached it. Only two patients (4%) worsened, both with single thoracic curve, 25-30 degrees Cobb and Risser 0 at the start. We found statistically significant reductions of the scoliosis curvatures (-7.1 degrees ): thoracic (-7.3 degrees ), thoracolumbar (-8.4 degrees ) and lumbar (-7.8 degrees ), but not double major. Statistically significant improvements have also been found for aesthetics and ATR. Respecting also SOSORT management criteria and thus increasing compliance, the results of conservative treatment were much better than what had previously been reported in the literature using SRS criteria only."}, {"id": "pubmed23n0805_1755", "title": "Low rate of surgery in juvenile idiopathic scoliosis treated with a complete and tailored conservative approach: end-growth results from a retrospective cohort.", "score": 0.014164173522812266, "content": "The main distinctive aspect of Juvenile Idiopathic Scoliosis (JIS) with respect to Adolescent Idiopathic Scoliosis (AIS) is the high risk of severe deformity and surgery. Approximately 70% of curves in patients with JIS progress and ultimately require surgery. There are presently very few studies with long-term follow-up of JIS and even fewer looking specifically at bracing Purpose To verify the effectiveness of a complete conservative treatment, including bracing and exercises, for JIS. Retrospective cohort observational study nested in a clinical prospective database of consecutive outpatients. Patient Sample Inclusion criteria: JIS, no previous treatment, all consecutive radiographies available from treatment start to end of growth (Risser sign 3). We found 30 patients, 27 females, 10 JIS type 1; mean age at first diagnosis was 7.8 +/-1.5 and mean treatment lasted 5.8 years. Cobb degrees 24.4+/-10 degrees, with 7 cases &gt;30 degrees, and 2 &gt; 45degrees. Outcome Measures Physiological measures. Radiographic and clinical data. Treatment (exercises alone, or elastic-rigid-highly rigid braces plus exercises) was tailored and continuously changed according to Cobb degrees, individual preferences, anthropometric characteristics, pubertal spurt, remaining growth, rotation, hump, lumbar curve take-off, and imbalance. The SOSORT Guidelines for patients' management have been followed. Funding and Conflict of Interest: no. 33.3% (95% Confidence Interval 16.4-50.2%) of patients worsened over the years. At the end of growth, 6.6% (0-15.5%) had surgical deformities (&gt;45degrees). We observed a good correction in the first years of treatment until pubertal growth spurt, when progression was usually noted and treatment changed increasing corrective forces (hours or rigidity of bracing). 23 cases were followed up until they had two consecutive radiographies showing Risser sign 5 and showed stability. Conservative treatment initiated already in childhood may favorably change the natural history of JIS with the aim of reaching a curve as far as possible from surgical thresholds. Observation, physical exercises, braces can be useful tools in the hand of physicians, but they must be carefully utilized by a deep knowledge of JIS."}, {"id": "pubmed23n0668_24621", "title": "A comparison of thoracolumbosacral orthoses and SpineCor treatment of adolescent idiopathic scoliosis patients using the Scoliosis Research Society standardized criteria.", "score": 0.013440860215053764, "content": "SpineCor is a relatively new bracing system that uses dynamic bracing concepts in the treatment of adolescent idiopathic scoliosis (AIS). Limited data are available regarding its effectiveness. This study compared treatment outcomes of 2 groups of AIS patients treated via either a conventional rigid thoracolumbosacral orthoses (TLSO) or a SpineCor nonrigid orthosis. We identified 2 scoliosis patient cohorts: 35 patients treated with a TLSO and 32 patients treated with a SpineCor orthosis. All patients included in these groups conformed with the Scoliosis Research Society (SRS) standardized criteria for AIS bracing: (1) Risser &lt; or =2, (2) curve magnitude 25 to 40 degrees, (3) age &gt; or =10 years. Outcomes were SRS standardized with failure being defined as curve progression &gt; or =6 degrees, or ever exceeding 45 degrees, or having surgery recommended before skeletal maturity. All patients were followed through the completion of brace treatment or attainment of other treatment end points. The Yates corrected chi test and unpaired t test were used for data analysis. The 35 patients (32 girls, 3 boys) in the TLSO group had an average age of 13 years (range: 11.1-16.8) and an average primary curve magnitude of 33 degrees (range: 25-40 degrees). Follow-up averaged 2 years (range: 8-61 m) from the beginning of brace treatment. The 32 patients (28 girls, 4 boys) in the SpineCor group had an average age of 13 years (range: 11-15.2) and an average primary curve magnitude of 31 degrees (range: 25-40 degrees). Follow-up for this group averaged 2 years and 6 months (range: 13-73 mo) from the beginning of brace treatment. No significant difference (P=0.75) was found using the more strict outcome measure (&lt; or =5-degree curve progression) as the success rates were 60% (21/35) for TLSO and 53% (17/32) for SpineCor. Similarly, no significant difference (P=0.62) was found using the more liberal outcome measure (never reached 45 degrees) as the success rates were 80% (28/35) for TLSO and 72% (23/32) for SpineCor. We were unable to identify any significant differences in brace treatment outcomes when comparing TLSO and SpineCor treated patients."}, {"id": "wiki20220301en506_25267", "title": "Management of scoliosis", "score": 0.013235294117647059, "content": "Surgery is indicated by the Society on Scoliosis Orthopaedic and Rehabilitation Treatment (SOSORT) at 45 degrees to 50 degrees and by the Scoliosis Research Society (SRS) at a Cobb angle of 45 degrees. SOSORT uses the 45-degree to 50-degree threshold as a result of the well-documented, plus or minus five degrees measurement error that can occur while measuring Cobb angles. Scoliosis braces are usually comfortable for the patient, especially when it is well designed and fit; also after the 7- to 10-day break-in period. A well fit and functioning scoliosis brace provides comfort when it is supporting the deformity and redirecting the body into a more corrected and normal physiological position. The Scoliosis Research Society's recommendations for bracing include curves progressing to larger than 25°, curves presenting between 30 and 45°, Risser sign 0, 1, or 2 (an X-ray measurement of a pelvic growth area), and less than six months from the onset of menses in girls."}, {"id": "pubmed23n0239_2151", "title": "[Results of brace treatment in idiopathic scoliosis--evaluation of the patients treated for over 2 years or those who completed the treatment].", "score": 0.012824510797536447, "content": "The results of the brace treatment (Milwaukee brace, Thoraco-Lumbo-Sacral-Orthosis, Boston-Milwaukee brace) were studied in 509 patients with idiopathic scoliosis, who were braces for an average of 3 years and 3 months ranging from 2 to 10 years. Of these patients, 60 were followed up for about 24 months after the brace was discontinued. The distribution of these patients according to curve pattern was as follows: 319 had thoracic curve; 78 had lumbar curve (combining lumbar and thoracolumbar curves together as one group); and 112 had double major curve. The findings are summarized and conclusions were drawn as follows. 1. The best correction was obtained within a year after the initiation of bracing, followed by a gradual loss of correction. 2. There was a significant difference between the final correction rate and the best correction rate in the brace. 3. In the patients with ages ranging from 11 to 14 years and with curves of 30 degrees or less at the beginning of brace treatment, the curves were maintained within 30 degrees at the final stage. 4. Those patients who cooperated well in wearing their braces had a smaller loss of correction at the final stage. 5. In 63 per cent of the cases who showed progression in the brace, iliac apophysis did not appear at the beginning of brace treatment. 6. Moiré topography was used for the analysis of cosmesis and it was shown to be a valuable method of three dimensional evaluation. 7. In 60 patients who were followed up for 24 months on average after the brace was discontinued, the curves had improved an average of 1.6 degrees at the time of their last check up."}, {"id": "pubmed23n0730_6128", "title": "Bracing can reduce high degree curves and improve aesthetics immediately after the end of growth. Final results of a retrospective case series.", "score": 0.012481530445602303, "content": "Recently it has been shown that idiopathic scoliosis (IS) curves can be reduced with bracing, and it has been proposed that this could be useful in non-surgically treated high degree curves even after Risser 3. Moreover, bracing has been shown to be able to improve aesthetics, and this could be another reason to treat some patients with cosmetic needs. Our aim is to preliminary check if results can be obtained in IS patients after Risser 3. Design. Retrospective uncontrolled cohort study. Inclusion criteria. All IS patients treated on a voluntary basis for aesthetic reasons and/or for curve reduction; Risser 4-5 at start; end of treatment reached. Population. 34 females and 2 males, age 16.2±1.6 years, Cobb angle 27.6°±8.9°. Treatment. Lyon or SPoRT braces 18 to 24 hours/day, specific SEAS exercises, rapid weaning (2-3 hours every 6 months). Outcome criteria: SRS (unchanged; worsened over 6°; over 45° at the end of treatment; surgically treated), radiographic and clinical. Statistics. ANOVA and chi-test. The reported compliance during the 2.8 ± 1.1 treatment years was 95.1%, while residual growth was 0.9 ± 1.1 cm. Improvements were found in 39% of this cohort, (46% in curves over 30°). Only 1 patient progressed 6°. We found highly statistically significant reductions of maximal (-4.4°), thoracic (-6.0°) and thoracolumbar (-6.6°) curves. Statistically significant improvements were found for Aesthetic Index. Before 20 years of age, even in skeletally mature patients, it is possible to reach radiographic and aesthetic improvements, although not as good as during growth. Correction is based on bone growth, but ligaments and neuromuscular control of posture can also be involved."}, {"id": "pubmed23n0638_18760", "title": "Treatment of thoraco-lumbar curves in adolescent females affected by idiopathic scoliosis with a progressive action short brace (PASB): assessment of results according to the SRS committee on bracing and nonoperative management standardization criteria.", "score": 0.012043399638336347, "content": "The effectiveness of conservative treatment of scoliosis is controversial. Some studies suggest that brace is effective in stopping curve progression, whilst others did not report such an effect.The purpose of the present study was to effectiveness of Progressive Action Short Brace (PASB) in the correction of thoraco-lumbar curves, in agreement with the Scoliosis Research Society (SRS) Committee on Bracing and Nonoperative Management Standardisation Criteria. Fifty adolescent females (mean age 11.8 +/- 0.5 years) with thoraco-lumbar curve and a pre-treatment Risser score ranging from 0 to 2 have been enrolled. The minimum duration of follow-up was 24 months (mean: 55.4 +/- 44.5 months). Antero-posterior radiographs were used to estimate the curve magnitude (CM) and the torsion of the apical vertebra (TA) at 5 time points: beginning of treatment (t1), one year after the beginning of treatment (t2), intermediate time between t1 and t4 (t3), end of weaning (t4), 2-year minimum follow-up from t4 (t5). Three situations were distinguished: curve correction, curve stabilisation and curve progression.The Kruskal Wallis and Spearman Rank Correlation tests have been used as statistical tests. CM mean value was 29,30 +/- 5,16 SD at t1 and 14,67 +/- 7,65 SD at t5. TA was 12.70 +/- 6,14 SD at t1 and 8,95 +/- 5,82 at t5. The variation between measures of Cobb and Perdriolle degrees at t1,2,3,4,5 and between CM t5-t1 and TA t5-t1 were significantly different.Curve correction was accomplished in 94% of patients, whereas a curve stabilisation was obtained in 6% of patients. The PASB, due to its peculiar biomechanical action on vertebral modelling, is highly effective in correcting thoraco-lumbar curves."}, {"id": "pubmed23n0740_12959", "title": "Scoliosis detection, patient characteristics, referral patterns and treatment in the absence of a screening program in Norway.", "score": 0.011942959001782532, "content": "Early diagnosis of idiopathic scoliosis allows for observation and timely initiation of brace treatment in order to halt progression. School scoliosis screening programs were abolished in Norway in 1994 for lack of evidence that the programs improved outcome and for the costs involved. The consequences of this decision are discussed. To describe the detection, patient characteristics, referral patterns and treatment of idiopathic scoliosis at a scoliosis clinic during the period 2003-2011, when there was no screening and to compare treatment modalities to the period 1976-1988 when screening was performed. Patient demographics, age at detection, family history, clinical and radiological charts of consecutive patients referred for scoliosis evaluation during the period 2003-2011, were prospectively registered. Patients were recruited from a catchment area of about 500000 teenagers. Maturity was estimated according to Risser sign and menarcheal status. Severity of pain was recorded by a verbal 5-point scale from no pain to pain at all times. Physical and neurological examinations were conducted. The detector and patient characteristics were recorded. Referral patterns of orthopedic surgeons at local hospitals and other health care providers were recorded. Patient data was obtained by spine surgeons. Treatment modalities in the current period were compared to the period 1976-1988. We registered 752 patients with late onset juvenile and adolescent idiopathic scoliosis from 2003-2011. There were 644 (86%) girls and 108 (14%) boys. Mean age at detection was 14.6 (7-19) years. Sixty percent had Risser sign ≥ 3, whilst 74% were post menarche with a mean age at menarche of 13.2 years. Thirty-one percent had a family history of scoliosis. The mean major curve at first consultation at our clinic was 38° (10°-95°). About 40% had a major curve &gt;40°. Seventy-one percent were detected by patients, close relatives, and friends. Orthopaedic surgeons referred 61% of the patients. The mean duration from detection to the first consultation was 20(0-27) months. The proportion of the average number of patients braced each year was 68% during the period with screening compared to 38% in the period without screening, while the proportion for those operated was 32% and 62%, respectively ( p=0.002, OR 3.5, (95%CI 1.6 to 7.5). In the absence of scoliosis screening, lay persons most often detect scoliosis. Many patients presented with a mean Cobb angle approaching the upper limit for brace treatment indications. The frequency of brace treatment has been reduced and surgery is increased during the recent period without screening compared with the period in the past when screening was still conducted."}, {"id": "wiki20220301en007_12922", "title": "Scoliosis", "score": 0.011295928500496523, "content": "For example, a person who is still growing with a 17° Cobb angle and significant thoracic rotation or flatback could be considered for nighttime bracing. On the opposite end of the growth spectrum, a 29° Cobb angle and a Risser sign three or four might not need to be braced because the potential for progression is reduced. The Scoliosis Research Society's recommendations for bracing include curves progressing to larger than 25°, curves presenting between 30 and 45°, Risser sign 0, 1, or 2 (an X-ray measurement of a pelvic growth area), and less than six months from the onset of menses in girls. Scoliosis braces are usually comfortable, especially when well designed and well fitted, also after the 7- to 10-day break-in period. A well fitted and functioning scoliosis brace provides comfort when it is supporting the deformity and redirecting the body into a more corrected and normal physiological position."}, {"id": "pubmed23n1138_9600", "title": "Does Risser Casting for Adolescent Idiopathic Scoliosis Still Have a Role in the Treatment of Curves Larger Than 40°? A Case Control Study with Bracing.", "score": 0.010638598873892992, "content": "<bBackground</b: The most common conservative treatment for Adolescent Idiopathic Scoliosis (AIS) is bracing. However, several papers questioned the effectiveness of bracing for curves between 40° and 50° Cobb: the effectiveness in preventing curve progression could be as low as 35%. Seriate casting is considered a standard approach in early onset scoliosis; however, in the setting of AIS, cast treatment is seldom utilized, with only few studies reporting on its effectiveness. <bAim of the study:</b The main aim of the study is to determine whether a seriate casting with Risser casts associated with bracing is more effective in preventing curve progression than bracing alone in curves larger than 40°. Furthermore, the secondary endpoints were: (1) is there a difference in effectiveness of casting between Thoracic (T) and Thoracolumbar/Lumbar (TL/L) curves? (2) Does the 'in cast' correction predicts the treatment outcome? (3) What is the effect on thoracic kyphosis of casting? <bMethods</b: This is a retrospective monocentric case-control study; through an Institutional Database search we identified all the patients treated at our institution between 1 January 2017 and 31 December 2020, with a diagnosis of AIS, Risser grade between 0 and 4 at the beginning of the treatment, at least one curve above 40° Cobb and treatment with either seriate Risser casting and bracing (Study Group, SG) or bracing alone (Control Group, CG). Standing full spine X-rays in AP and LL are obtained before and after the cast treatment; only AP standing full spine X-rays 'in-cast' are obtained for each cast made. Patients were stratified according to the curve behavior at the end of treatment (Risser 5): progression was defined as ≥6° increase in the curve magnitude or fusion needed; stabilization is defined as a change in curve by ±5°; and improvement was defined as ≥6° reduction in the curve. <bResults</b: For the final analysis, 55 compliant patients (12 M, 43 F, mean age 13.5 ± 1.6) were included in the SG and 27 (4 M, 23 F, mean age 13.6 ± 1.6) in the CG. Eight (14.5%) patients in the SG failed the conservative treatment while 14 (51.3%) failed in the CG. Consequently, the Relative Risk for progression in the Efficacy Analysis was 1.8 (95% CI 1, 3-2.6, <ip</i = 0.001), and the Number Needed to Treat was 2,4. No significant difference was found between the T and TL/L curves concerning the 'progressive' endpoint (z-score 0.263, <ip</i = 0.79). The mean percentage of 'in cast' curve reduction was 40.1 ± 15.2%; no significant correlation was found between the percentage of correction and the outcome (Spearman Correlation Coefficient 0.18). Finally, no significant differences between baseline and end of FU TK were found (32° ± 16.2 vs. 29.6 ± 15.8, <ip</i = ns). <bDiscussion:</b Seriate Risser casting for AIS with larger curves (&amp;gt;40° Cobb) is effective in reducing curve progression when compared with full time bracing alone in treatment compliant patients. The treatment is equally effective in controlling T and TL/L curves; furthermore, a slight but non-significant decrease in TK was observed in patients treated with casting. This type of treatment should be considered for AIS patients who present with large curves to potentially reduce the percentage of surgical cases. <bShort Abstract</b: The aim of the study is to determine whether seriate Risser casting associated with bracing is more effective in preventing curve progression than bracing alone in curves larger than 40°. This is a retrospective monocentric case-control study; we identified all the patients treated at our institution with a diagnosis of AIS, Risser grade 0-4 at the beginning of the treatment, at least one curve above 40° Cobb (35° if treated with bracing alone) and treatment with either seriate Risser casting and bracing (Study Group, SG) or bracing alone (Control Group, CG). Fifty-five patients (12 M, 43 F, mean age 13.5 ± 1.6) were included in the SG and 30 (5 M, 25 F, mean age 13.9 ± 1.7) in the CG. Eight (14,5%) patients in the SG failed the conservative treatment while fifteen (50%) failed in the CG. Consequently, the Relative Risk for progression in the Efficacy Analysis was 1.8 (95% CI 1.3-2.6, <ip</i = 0.001), and the Number Needed to Treat was 2,4. Seriate Risser casting for AIS with larger curves (&amp;gt;40°) is effective in reducing curve progression when compared with full time bracing alone. This type of treatment should be considered for AIS patients who present with large curves."}, {"id": "pubmed23n0684_23337", "title": "Effectiveness of Chêneau brace treatment for idiopathic scoliosis: prospective study in 79 patients followed to skeletal maturity.", "score": 0.009975124378109452, "content": "Progressive idiopathic scoliosis can negatively influence the development and functioning of 2-3% of adolescents, with health consequences and economic costs, placing the disease in the centre of interest of the developmental medicine. The aim of this study was to evaluate the effectiveness of Chêneau brace in the management of idiopathic scoliosis. A prospective observational study according to SOSORT and SRS recommendations comprised 79 patients (58 girls and 21 boys) with progressive idiopathic scoliosis, treated with Chêneau brace and physiotherapy, with initial Cobb angle between 20 and 45 degrees, no previous brace treatment, Risser 4 or more at the final evaluation and minimum one year follow-up after weaning the brace. Achieving 50° of Cobb angle was considered surgical recommendation. At follow-up 20 patients (25.3%) improved, 18 patients (22.8%) were stable, 31 patients (39.2%) progressed below 50 degrees and 10 patients (12.7%) progressed beyond 50 degrees (2 of these 10 patients progressed beyond 60 degrees). Progression concerned the younger and less skeletally mature patients. Conservative treatment with Chêneau orthosis and physiotherapy was effective in halting scoliosis progression in 48.1% of patients. The results of this study suggest that bracing is effective in reducing the incidence of surgery in comparison with natural history."}, {"id": "pubmed23n0504_7732", "title": "Three-dimensional action of Chêneau brace on thoracolumbar scoliosis.", "score": 0.00980392156862745, "content": "We treated 18 girls for idiopathic thoracolumbar scoliosis with Chêneau brace. The apex of the curve was at Th12 or at L1 or at the disc Th12/L1. The initial Cobb angle varied from 21 to 42 degrees, mean 28. The follow up period was of 2 years and 6 months on the average. We noted the rib hump height on Adams' forward bending test. We measured Cobb angle, apical vertebra transposition and apical vertebra rotation (according to Perdriolle method) on antero-posterior standing radiograms before the treatment had started and at the moment of the best correction. We digitized antero-posterior and lateral standing radiograms with sonic digitizer GP-9 and we prepared computed reconstruction of the transversal plane of the spine with Hecquet and Graf's software RACHIS 91TM. The best clinical and radiological correction was achieved after 3 to 8 months of treatment (mean 5,5 months). We considered it as the fitting period and we analysed the correction achieved at that moment and at last follow-up. Cobb angle in brace ranged from 0 to 18 degrees, mean 9 degrees. The correction of apical vertebra transposition ranged from 51% to 100%, mean 80%. The correction of apical vertebra rotation ranged from 0% to 100%, mean 52%. The correction of rib hump ranged from 0 to 100%, mean 42%. Normal sagittal contour was established in 15 patients. important correction was present in each of the three planes."}, {"id": "pubmed23n0608_19205", "title": "Anterior spinal fusion versus posterior spinal fusion for moderate lumbar/thoracolumbar adolescent idiopathic scoliosis: a prospective study.", "score": 0.009708737864077669, "content": "A prospective study. Comparison study of radiologic and clinical outcomes, efficiency, and cost between anterior spinal fusion (ASF) and posterior spine fusion (PSF) in surgical treatment of moderate lumbar/thoracolumbar adolescent idiopathic scoliosis (AIS). ASF and PSF indicated for lumbar and thoracolumbar adolescent idiopathic scoliosis surgical treatment have respective advantages and disadvantages. However, up until today, a related prospective AIS comparative study has rarely been reported. Thirty-two cases in this prospective study with patients enrolled in either method A or B alternately in a sequence were divided into 2 groups. Group A underwent ASF with single solid rod and single screw constructs, and group B underwent PSF with segmental total pedicle screw system. Inclusion criteria were: (1) AIS diagnosis; (2) diagnosis classification as Lenke5CN type; (3) Cobb angles 35 degrees-60 degrees on anteroposterior view radiographs. Exclusion criteria were: (1) a history of spinal surgery; (2) age younger than 10 years; (3) Risser sign 0 degree; (4) lumbar/thoracolumbar kyphosis. All patients were observed with 2-year minimum follow-up (24-46 months). Clinical and radiologic outcomes of both groups A and B were analyzed. Statistical t test or Mann-Whitney U test demonstrated no significant difference in preoperative age (P = 0.380), Risser sign (P = 0.733), magnitude (P = 0.936), flexibility (P = 0.815), apical vertebra rotation (AVR, P = 0.756), and apical vertebra translation (AVT, P = 0.355) of the lumbar/thoracolumbar curves, trunk shift (TS, P = 0.448), sagittal kyphosis from T5-T12 (P = 0.792) and sagittal lordosis from L1-L5 (P = 0.299). Average coronal correction of thoracolumbar/lumbar curves was 83% after surgery and 77% at follow-up in group A and 87% after surgery and 82% at follow-up in group B (P = 0.236 and P = 0.138). No significant differences were observed regarding correction of sagittal alignment, TS, AVT, AVR and hospitalization days on last follow-up between both groups (P &gt; 0.05). No pseudarthrosis, reoperation, neurologic complications, infection, and no other problems were observed. Excellent clinical fusion results were present in all patients on their last follow-up. However, significant differences were evident in group A in regards to reduced operative time (P = 0.046), reduced estimated blood loss (P = 0.003), decreased blood transfusion (P = 0.006), reduced implants cost and hospitalization expenses (P = 0.000). Additionally, group A had shorter fusion levels than group B (p50 = 4 vs. p50 = 5, P = 0.003). ASF versus PSF comparison in treating moderate lumbar/thoracolumbar AIS did not show significant differences in regards to safety or efficacy but demonstrated shorter fusion levels, reduced surgical trauma and costs in ASF."}, {"id": "pubmed23n0211_15462", "title": "Progression in scoliosis. A 360 degrees change in 75 years.", "score": 0.009708737864077669, "content": "With the proliferation of school screening programs for spinal deformity, attention has been directed to methods of scoliosis evaluation and follow-up that avoid serial x-ray exposure. An historical review of the scoliosis literature prior to the extensive use of x-ray in scoliosis care has demonstrated much that is now being \"rediscovered.\" By 1900, screening for spinal deformity in the schools had been instituted, pertinent findings of the physical examination in patients with scoliosis had been well described, and early natural history information had been obtained regarding the behavior of small curves and curves associated with thoracic lordosis. Recordings of surface contours and rib humps, using inclinometers, lead rulers, and other measuring devices, were used to assess the progression of curves. Low contour braces were widely available. Although our orthopaedic forefathers had many erroneous ideas, a perusal of the early orthopaedic writings from around the turn of the century has shown how little is \"new\" (other than changes in material and spinal instrumentation techniques) from what was recognized 75 years ago."}, {"id": "pubmed23n0727_10998", "title": "Anterior instrumentation (dual screws single rod system) for the surgical treatment of idiopathic scoliosis in the lumbar area: a prospective study on 33 adolescents and young adults, based on a new system of classification.", "score": 0.009615384615384616, "content": "The choice of anterior instrumentation in the treatment of lumbar scoliosis in adolescents and young adults is not a new topic for the authors. The first results achieved using the Dwyer surgical modality were reported by one of the authors followed by the results achieved using Zielke (VDS) instrumentation. Today, new techniques and new instrumentations have been developed that challenge the instrumentation choices. Here we describe how the new system of classification of scoliotic curves we developed has been used as a basis for treating idiopathic scoliosis in lumbar area in adolescents and young adults using an anterior approach. A prospective study was carried out between 1998 and 2010 at two hospital centers on 33 adolescents and young adult with idiopathic lumbar scoliosis involving curves of three kinds, on whom surgical treatment was performed using a single solid rod. Topography of curves: our system of classification includes curves corresponding to the following three type of scoliosis: Type K I: double thoracic and lumbar curves (lumbar predominant) scoliosis (17 cases) mean age 16 years all female patients. Mean Cobb angle of lumbar curve 41°. Mean Cobb angle of thoracic curve 28°. The lumbar curve was left hand convex in 15 cases and right hand convex in 2 cases. Horizontal tilting of L4 mean value 22°. C7 offset mean value 3 cm. Type K IV A: unbalanced thoracolumbar scoliosis (13 cases) mean age 17 years, ten female patients and three male patients. Mean Cobb angle of thoracolumbar curve 39°. The thoracolumbar curve was left hand convex 4 times and right hand convex 9 times. Horizontal tilting of L4 mean value 18°. C 7 offset mean value 2.5 cm. Type K VI A: real lumbar (three cases). Age: 17, 15 and 13 years; all female patients. Cobb angle of the lumbar curve 66°, 29° and 70° (all LH convex). Horizontal tilting of L4: 40°, 20° and 46°. C 7 offset: 7 cm, 1 cm and 4 cm. Surgical instrumentation: We used the EUROS AZUR anterior instrumentation for all the procedures. Cages have been used on five patients at the lower stages. Number of vertebrae instrumented: mean five vertebrae. The patients did not wear postoperative orthosis. Mean duration of procedure: 3 h 50 min. Mean blood loss: 350 cm(3). Type K I scoliosis (17 cases): Mean follow-up: 6 years. Correction of the lumbar curve Cobb angle: the mean angle has been corrected from 41° to 21°. Number of vertebrae instrumented: 4:6 times and 5:11 times. Correction of the upper thoracic curve Cobb angle: mean angle corrected from 28° to 19°. Correction of L4 horizontal tilting: mean residual was 7°. Correction of C 7 offset: mean 0.7 cm. Type K IV A scoliosis (13 cases): mean follow-up: 4 years. Correction of the lumbar curve Cobb angle: the mean angle has been corrected from 39° to 16°. Mean number of instrumented vertebrae: 5 (4:4 times, 5:6 times and 6:3 times.) Correction of L4 horizontal tilting: mean residual 5°. Correction of C 7 offset: mean 0.7 cm. Type K VI A scoliosis (three cases): mean follow-up: 7, 2 and 4 years; Correction of the lumbar curve Cobb angle: the angles have been corrected from 66° to 15°, from 29° to 11° and from 70° to 28°. Number of instrumented vertebrae: 5, 4 and 6. Correction of L4 horizontal tilting: residual tilting of 8°, 7° and 17°. Correction of C 7 offset: 1 cm, 0 cm and 1 cm. There has been no report early or late septic or vascular or neurological complications. Instrumentation failure: there were three cases of screw breakage, all occurred on the lowest implant. Revision surgery was undertaken in both cases, only the last plate needed to be replaced and the rod could be kept without any other modification of the construct. In both cases, fusion has been achieved without any loss of correction. The mean loss of correction of the main curve was 2.5° for the three series. Anterior instrumentation of lumbar idiopathic scoliosis gives highly satisfactory morphological and functional results, since the lumbar musculature is spared and the instrumentation placed at the apex of the curvature has selective effects. Despite our preference and that of other surgeons throughout the world for anterior instrumentation, we are still a minority in comparison with the users of posterior instrumentation. There are several reasons for this reticence, including surgeons' training and ideas about pedicular screw fixation, but the main reason has been the lack of a sufficiently exact system of classification. Previous comparative studies between the anterior and posterior approaches have been biased by the use of an excessively restrictive mode of classification (lumbar/thoracolumbar) of the curves. Real lumbar scoliosis, unbalanced thoracolumbar scoliosis and thoracic and lumbar double curve (lumbar predominant) scoliosis should be properly defined before being compared."}, {"id": "wiki20220301en506_25265", "title": "Management of scoliosis", "score": 0.009615384615384616, "content": "Indications for Scoliosis Bracing: Scoliosis professionals determine the proper bracing method for a patient after a complete clinical evaluation. The patient’s growth potential, age, maturity, and scoliosis (Cobb angle, rotation, and sagittal profile) are also considered. Immature patients who present with Cobb angles less than 20 degrees should be closely monitored and proactively treated based on their risk of progression as surgery can be prevented with early intervention of conservative treatment. Immature patients who present with Cobb angles of 20 degrees to 29 degrees should be braced according to the risk of progression by considering age, Cobb angle increase over a six-month period, Risser sign, and clinical presentation. Immature patients who present with Cobb angles greater than 30 degrees should be braced. However, these are guidelines and not every patient will fit into this table. For example, an immature patient with a 17-degree Cobb angle and significant thoracic"}, {"id": "pubmed23n0609_6199", "title": "SpineCor vs. natural history - explanation of the results obtained using a simple biomechanical model.", "score": 0.009615384615384616, "content": "In the recent peer reviewed literature the SpineCor is described as an effective method of treatment for patients with scoliosis. However until recently no prospective controlled end-result study is presented comparing the results obtained with this soft brace to natural history. The objective was to determine whether the results obtained by the use of the SpineCor are better than natural history during pubertal growth spurt. The method employed prospective comparison of the survival rates of SpineCor treatment vs. natural history with respect to curve progression during pubertal growth spurt. 12 Patients with Cobb angles between 16 and 32 degrees (at average 21 degrees) during pubertal growth spurt are presented as a case series treated with the SpineCor. Survival rate of this sample is described and compared to natural history (SRS brace study 1995). All girls treated in both studies were at risk for being progressive with the first clinical signs of maturation (Tanner 2-3). During the pubertal growth spurt most of the patients (11/12) with SpineCor progressed clinically and radiologically as well (at least 5 degrees). Progression could be stopped changing SpineCor to the Chêneau brace in most of the sample described (7/10). The avarage Cobb angle at the start of treatment with the SpineCor was 21.3 degrees, after an average observation time of 21.5 months 31 degrees. At 24 months of treatment time 33% of the patients with the SpineCor where still under treatment with their original bracing concept, at 72 months follow-up time 8 % of the patients with the SpineCor survived with respect to curvature progression. Survival proportion in the SpineCor sample, though was 0.08, while in the natural history cohort it was 0.34. The SpineCor treatment during pubertal growth spurt seems to lead to a worse outcome than observation only. The use of a simple biomechanical model explains that in the brace the compression forces exceed the lateral forces used for the corrective movement. Therefore SpineCor does not seem to be indicated as a treatment during pubertal growth spurt."}, {"id": "wiki20220301en506_25262", "title": "Management of scoliosis", "score": 0.009572884461238978, "content": "Bracing is most effective when the patient has bone growth remaining (is skeletally immature) and should aim to both prevent progression of the curve (prevent progression to surgery), as well as reduce the scoliosis curve. Reduction of the curve is important as the natural history of idiopathic scoliosis suggests it can continue to progress at a rate ~1 degree per year in adulthood, while the treatment results of bracing have been shown to hold over >15 years. In some cases with juveniles, bracing has reduced curves significantly, going from a 40 degrees (of the curve, mentioned in length above.) out of the brace to 18 degrees in it. Braces are sometimes prescribed for adults to relieve pain related to scoliosis. Bracing involves fitting the patient with a device that covers the torso; in some cases, it extends to the neck. The most commonly used brace is a TLSO, such as a Cheneau type brace, a corset-like appliance that fits from armpits to hips and is custom-made from fiberglass or"}, {"id": "pubmed23n0885_17329", "title": "Immediate effects of scoliosis-specific corrective exercises on the Cobb angle after one week and after one year of practice.", "score": 0.009523809523809525, "content": "We are unaware of any studies describing the immediate effects of scoliosis-specific exercises on the Cobb angle measured by radiograph. This study aimed to describe the differences between radiographs obtained with and without corrective exercises after initial training and after one year. A female with adolescent idiopathic scoliosis was first seen at age 13 years, 0 months with a Risser 0. She had a 43<supo</sup left lumbar, 15<supo</sup right thoracic curve. She was seen again after 6, 18 and 30 months and performed exercises from 18 to 30 months. She performed Barcelona Scoliosis Physical Therapy School (BSPTS) exercises for a four-curve type (lumbar dominant with pelvis deviation to the lumbar concave side). At 18 and 30 months, x-rays were obtained with and without performing corrective exercises. At 6 months, her lumbar and thoracic curves measured 41<supo</sup and 28<supo</sup, respectively. At 18 months, her lumbar and thoracic curves measured 47 <supo</sup and 30<supo</sup, respectively. Also at 18 months, immediately after her x-ray in the relaxed standing position, she performed her corrective exercises in standing with arms lowered for a second x-ray. Her lumbar and thoracic curves remained similar and measured 43<supo</sup and 32<supo</sup, respectively. At 30 months, she performed unsolicited corrective exercises during the x-ray. Her lumbar and thoracic curves measured 26<supo</sup and 41<supo</sup, respectively. Another x-ray in the relaxed position revealed lumbar and thoracic curves measuring 39<supo</sup and 35<supo</sup, respectively. The immediate effect of corrective exercises after a year of training was a 33 % improvement at the lumbar spine compared to only a 9 % improvement the previous year. After initial training, corrective exercises during a standing x-ray did not significantly improve the Cobb angle for the major lumbar curve compared to the relaxed standing x-ray. However, a year after performing exercises, unsolicited corrective exercises resulted in a significantly improved Cobb angle compared to relaxed standing for the curve primarily targeted by the exercise program. Improved exercise ability and spinal flexibility may have contributed to the improved Cobb angle."}, {"id": "pubmed23n0661_12745", "title": "[Systematic Long-term Screening of Scolioses.].", "score": 0.009523809523809525, "content": "The authors implemented in a defined region for a period of 14 years screening of scolioses in children, primary screening using Adams' test and secondary screening using a scoliometer. They selected as criterion for detection 5 degrees of asymmetry of the back, corresponding to 17 degrees according to Cobb on X-ray pictures. In three growth stages a total of 603 children with scoliosis were detected, incl. 382 girls and 221 boys. The detection rate was highest in the adolescent period (71 %), the majority of detected scolioses (77 %) were idiopathic. On examination with a scoliometer comparable results were achieved with the technique moiré (false positivity 5.2 %). Deformities of the spine were detected at a level (mainly grade Ib), where follow up and further treatment can be effective. Screening which was implemented involved small costs per child. A positive outcome of the screening was a reduced incidence of severe scolioses and this reduced among others the expenditure on treatment by orthesis of the trunk or surgery. Key words: scoliosis, screening, mechanical scoliometer."}, {"id": "wiki20220301en007_12914", "title": "Scoliosis", "score": 0.009478672985781991, "content": "The standard method for assessing the curvature quantitatively is measuring the Cobb angle, which is the angle between two lines, drawn perpendicular to the upper endplate of the uppermost vertebra involved and the lower endplate of the lowest vertebra involved. For people with two curves, Cobb angles are followed for both curves. In some people, lateral-bending X-rays are obtained to assess the flexibility of the curves or the primary and compensatory curves. Congenital and idiopathic scoliosis that develops before the age of 10 is referred to as early-onset scoliosis. Progressive idiopathic early-onset scoliosis can be a life-threatening condition with negative effects on pulmonary function. Scoliosis that develops after 10 is referred to as adolescent idiopathic scoliosis. Screening adolescents without symptoms for scoliosis is of unclear benefit."}, {"id": "wiki20220301en007_12913", "title": "Scoliosis", "score": 0.00946266386291441, "content": "As an alternative, a scoliometer may be used to diagnose the condition. When scoliosis is suspected, weight-bearing, full-spine AP/coronal (front-back view) and lateral/sagittal (side view) X-rays are usually taken to assess the scoliosis curves and the kyphosis and lordosis, as these can also be affected in individuals with scoliosis. Full-length standing spine X-rays are the standard method for evaluating the severity and progression of scoliosis, and whether it is congenital or idiopathic in nature. In growing individuals, serial radiographs are obtained at 3- to 12-month intervals to follow curve progression, and, in some instances, MRI investigation is warranted to look at the spinal cord."}, {"id": "pubmed23n0109_8666", "title": "Natural history and school screening for scoliosis.", "score": 0.009433962264150943, "content": "In light of the questions and controversy regarding school screening for spinal deformities, should the programs be dropped? The natural history is not completely known and the results of nonoperative treatment questioned. The costs are high due to over-referral and numerous physician visits and radiographs. Would it not be best to wait until the epidemiologic questions are answered? The best approach is one in the middle ground. The program needs to be organized and strengthened. With the education of screeners, over-referral can be reduced. The treating physician must confirm the physical finding, take appropriate radiographs, and plan appropriate follow-up. In this way, the costs will be reduced. In addition, with knowledge regarding natural history, only larger curves or progressive curves will be treated. Nonoperative treatment of idiopathic scoliosis is effective. It can control progression and even result in correction of some curves. The overall effectiveness of braces and electrical stimulation needs to be constantly reviewed. How do these forms of nonoperative treatment affect the progressive curve, and do they reduce the need for surgery in idiopathic scoliosis? Only after we have more studies on natural history and on the results of nonoperative treatment can screening for scoliosis be reassessed to determine its role in the overall treatment program of spinal deformities."}, {"id": "pubmed23n0573_23658", "title": "[A clinical follow-up study on treatment of adolescent idiopathic scoliosis with brace].", "score": 0.009345794392523364, "content": "To evaluate clinical outcomes of bracing and analyze related factors that influence curative effects in adolescents with idiopathic scoliosis, and to investigate indications of bracing. Seventy-nine patients with AIS who had no history of prior therapy were treated with a brace. Several parameters were consecutive measured and documented during the period of follow-up including Cobb's angles, curve patterns, menarche status, sitting heights, standing heights, Risser sign, apical vertebral rotation, and so on. The average period of followed-up was 30 months (12 months to 60 months). Twenty-one patients (26.6%) presented curve deterioration, 40 patients have no obvious curve change, 18 patients (22.8%) got a curve improvement. There was significantly lower percentage of curve progression and higher percentage of curve improvement in cases with Cobb's angle less than 35 degrees at the first visit (P &lt; 0.05). The percentage of curve progression was significantly greater in the cases with apical vertebral rotation beyond grade III while the percentage of curve improvement was lower (P &lt; 0.05). Curve patterns, Risser sign and other parameters were found to make their effects on the percentage of curve progression and improvement, which, however, was not statistically significant (P &gt; 0.05). Bracing can limit or improve mild and moderate curve of idiopathic scoliosis effectively, especially in cases with initial curve magnitude ranging from 20 degrees to 35 degrees . Risser sign is not a reliable parameter for measuring the outcome of bracing treatment for idiopathic scoliosis. Surgery is advised as soon as possible for the cases with initial Cobb's angles greater than 45 degrees and initial apical vertebral rotation beyond grade III early while bracing did not work."}, {"id": "pubmed23n1160_1563", "title": "Surgical correction of a previously operated juvenile idiopathic scoliosis with crankshaft phenomenon: an illustrative case report.", "score": 0.009259259259259259, "content": "Crankshaft phenomenon secondary to posterior fusion for scoliotic deformity at a young age has become rare and its management can be very challenging. We report the case of an 11-year-old girl who has been complaining of a progressively increasing hump in her back with waist and shoulders asymmetry during the past 6 months. Three years prior to presentation, she underwent in another institution posterior correction fusion from T3 to L3 for a juvenile idiopathic scoliosis with a Cobb angle of 60°. After the initial correction, follow-up X-rays revealed a progressive increase of the scoliosis angulation with the onset of a coronal malalignment mainly at the cervicothoracic junction. Full spine anteroposterior and lateral X-rays revealed a long right thoracolumbar scoliosis of 70° with a rib-vertebra angle difference of 27° and the proximal right screw pulled out from the rod. CT scan confirmed the posterior fusion between the apical vertebras. MRI did not show any congenital anomaly. The patient underwent a revision surgery with instrumentation from T1 to L4, and posterior column osteotomies at 6 levels between T4 and T10. Coronal Cobb angle corrected to 11° with satisfactory sagittal alignment and a maintained correction at 3 years of follow-up. This is the first case to thoroughly illustrate surgical management in the onset of a crankshaft phenomenon. Through a posterior-only approach, the use of posterior column osteotomies at the apex of the deformity in order to release the previous fusion is a safe and satisfactory option to reestablish proper coronal and sagittal alignment, with satisfactory clinical and radiological long-term results."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[425, 564]], "word_ranges": [[66, 89]], "text": "The bone marrow aspirate or biopsy is only indicated if there are other accompanying cytopenias that are not justified by the B-CLL itself,"}, "3": {"exist": true, "char_ranges": [[565, 738]], "word_ranges": [[89, 121]], "text": "PET-CT is only indicated if Ritcher's syndrome is suspected and we do not have enough data to suspect it, there are no B symptoms, nor is there any mention of LDH elevation."}, "4": {"exist": true, "char_ranges": [[0, 424]], "word_ranges": [[0, 66]], "text": "The patient is already diagnosed with chronic lymphocytic leukemia B (B-CLLL), presents lymphocytosis that is maintained for more than 3 months and by flow cytometry presents a phenotype compatible with it. Therefore, we must first know the risk factors that will indicate with what priority treatment will need to be initiated, their intensity and prognosis; the presence of TP53 mutations or 17p deletions depends on this."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The patient is already diagnosed with chronic lymphocytic leukemia B (B-CLLL), presents lymphocytosis that is maintained for more than 3 months and by flow cytometry presents a phenotype compatible with it. Therefore, we must first know the risk factors that will indicate with what priority treatment will need to be initiated, their intensity and prognosis; the presence of TP53 mutations or 17p deletions depends on this. The bone marrow aspirate or biopsy is only indicated if there are other accompanying cytopenias that are not justified by the B-CLL itself, PET-CT is only indicated if Ritcher's syndrome is suspected and we do not have enough data to suspect it, there are no B symptoms, nor is there any mention of LDH elevation.", "full_answer_no_ref": "The patient is already diagnosed with chronic lymphocytic leukemia B (B-CLLL), presents lymphocytosis that is maintained for more than 3 months and by flow cytometry presents a phenotype compatible with it. Therefore, we must first know the risk factors that will indicate with what priority treatment will need to be initiated, their intensity and prognosis; the presence of TP53 mutations or 17p deletions depends on this. The bone marrow aspirate or biopsy is only indicated if there are other accompanying cytopenias that are not justified by the B-CLL itself, PET-CT is only indicated if Ritcher's syndrome is suspected and we do not have enough data to suspect it, there are no B symptoms, nor is there any mention of LDH elevation.", "full_question": "A 67-year-old patient who in the last 6 months, in two routine analyses, presents progressive lymphocytosis. In the last one, hemoglobin 15.4 g/dL; leukocytes 18.5 x103/μL with 82 % of mature lymphocytes that by flow cytometry express CD5/CD19/CD23 antigens and platelets 240 x103/μL. What do you think is the correct approach?", "id": 489, "lang": "en", "options": {"1": "Study of TP53 mutations to establish prognosis.", "2": "Bone aspirate/biopsy to confirm diagnosis.", "3": "PET/CT to establish the therapeutic attitude.", "4": "New clinical and analytical control in 6 months.", "5": NaN}, "question_id_specific": 105, "type": "HEMATOLOGY", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0771_21251", "title": "[Chronic lymphocytic leukemia].", "score": 0.018447998646018447, "content": "Chronic lymphocytic leukemia (CLL) is a lymphoproliferative disorder that accounts for approximately 30 % of adult leukemias and 25 % of Non-Hodgkin lymphomas (NHL). It is the most common form of leukemia in the western world (incidence 3-5/100 000). Elderly people are mainly affected, median age at diagnosis is around 70 years and there is a slight predominance in men. The etiology of the disease is unknown. The initial symptoms are nonspecific. Cervical lymphadenopathy and splenomegaly followed by general fatigue are seen most commonly. Other possible symptoms include night sweats, fever, loss of weight (so-called B symptoms) and frequent infections. Several patients develop autoimmune complications as autoimmune hemolytic anemia (AIHA) or immune thrombocytopenia (ITP). To confirm the diagnosis more than 5000 B-lymphocytes/µl need to be present. The expression of the typical surface markers CD5, CD19, and CD23 has to be confirmed by flow cytometry. Imaging studies as X-ray of the chest, ultrasound of the abdomen, or CT scan are used to assess the degree of lymphadenopathy or organomegaly. A bone marrow biopsy is not mandatory for the diagnosis. According to the European Binet staging system, CLL is divided into 3 stages (A, B and C). Patients in Binet stage A have 0 to 2 areas of node or organ enlargement with normal levels of hemoglobin and platelets. Binet stage B patients have 3 to 5 areas of node or organ enlargement and normal or slightly decreased levels of hemoglobin and platelets. Binet stage C patients have anemia (hemoglobin &lt; 10 g/dl) and/or thrombocytopenia (platelet counts &lt; 100 000/µl), with or without lymphadenopathy or organomegaly. As there is no survival benefit associated with early intervention, asymptomatic patients with early stage CLL (Binet stage A and B) are usually not treated but are followed on a \"watch and wait\" principle. Treatment indications include stage Binet C or signs of an active disease as rapidly progressive lymphadenopathy or organomegaly together with physical limitation, B symptoms that cannot be tolerated, rapidly deteriorating blood values, or rapidly increasing leukocyte counts. The patient's physical condition has major impact on the treatment decision. Currently immunochemotherapy with fludarabine, cyclophosphamide and the CD20-antibody rituximab (FCR) is the standard of care in previously untreated and physically fit CLL-patients. An alternative regimen is the combination of bendamustine and rituximab (BR). Physically compromised patients can be treated with the oral drug chlorambucil or with bendamustine with or without rituximab. Due to high morbidity and mortality, allogeneic stem cell transplantation is limited to a small group of patients and should be discussed in a high-risk situation, such as 17p deletion, lack of response to standard therapy or early relapse."}, {"id": "pubmed23n0491_6249", "title": "[Chronic lymphocytic leukemia--the old and the new].", "score": 0.01710319164162015, "content": "Chronic Lymphocytic Leukemia (CLL) is the most frequent lymphoproliferative disease and leukaemia in western countries. CLL occurs more frequently in men than women, the median age at diagnosis is 65 years. CLL is defined as a persisting chronic lymphocytosis &gt; 5 G/l with classical morphological features (small lymphocytic cells with round nuclei, dense chromatin and small cytoplasmic rim) and a classical immunophenotype (CD5+, CD19+, CD20+, CD23+); however, deviations from classical morphology are frequent. In cases with classical diagnostic features in the peripheral blood, a bone marrow biopsy is not necessary for diagnosis. Prognostic features comprise the stage of the disease according to the Rai or Binet systems, laboratory markers such as LDH, beta-2-microglobulin, lymphocyte doubling time and CD38 expression by flow cytometry (and ZAP-70 expression if available) as well as the status on IgV(H) hypermutations and cytogenetic analysis. Up to 2/3 of patients do not need treatment at the time of diagnosis and can initially be followed using a watch and wait strategy. If therapy becomes necessary, initial standard treatment still is chlorambucil. Later, purine analogues and Alemtuzumab are treatment options for refractory or relapsing disease. Therapies with antibodies such as Alemtuzumab and Rituximab in combination with purine analogues are currently under clinical investigation. With recurrent or atypical infections, hypogammaglobulinemia should be searched for and immunoglobulins should be substituted if necessary. However, their prophylactic use is not recommended."}, {"id": "pubmed23n0825_9939", "title": "[Chronic lymphatic leukemia].", "score": 0.015764936817568395, "content": "Chronic lymphocytic leukemia (CLL) is the most common form of leukemia in the Western world. Median age at diagnosis is around 70 years. To confirm the diagnosis more than 5000 B-lymphocytes/µl need to be present. The expression of the typical surface markers CD5, CD19, CD20 and CD23 has to be confirmed by flow cytometry. A bone marrow biopsy is not mandatory for the diagnosis. Before start of treatment the assessment of 17 p deletion and/or TP53-mutational status is recommended. Treatment indications include stage Binet C or signs of an active disease as rapidly progressive lymphadenopathy or organomegaly together with physical limitation, B symptoms that cannot be tolerated, rapidly deteriorating blood values, or rapidly increasing leukocyte counts (Lymphocyte doubling time less than 6 months). The patient's physical condition has major impact on the treatment decision. Currently immunochemotherapy with fludarabine, cyclophosphamide and the CD20-antibody rituximab (FCR) is the standard of care in previously untreated and physically fit patients. An alternative regimen is the combination of bendamustine and rituximab (BR) or ofatumumab. Physically compromised patients can be treated with the oral drug chlorambucil in combination with an anti-CD20 antibody. Due to high morbidity and mortality, allogeneic stem cell transplantation is limited to a small group of patients and should be discussed in a high-risk situation, such as 17 p deletion and/or TP53-mutation, lack of response to standard therapy or early relapse. Recently several new chemo-free treatment options have been introduced within clinical trials. Among them are monoclonal antibodies, most of them targeting the CD20 molecule: besides the licensed drugs rituximab and ofatumumab, obinutuzumab, in combination with chemotherapy, has recently shown high clinical efficacy in front-line treatment of elderly patients with CLL. Novel agents have been designed to block aberrant signaling from the B-cell receptor. Ibrutinib acts by inhibiting the Bruton's tyrosine kinase (BTK) while idelalisib represents a first-in-class specific inhibitor of the phosphoinositol-3 kinase (PI3K) delta isoform. Another class of drugs with potential impact for chemo-free treatment strategies in CLL is the BH3-mimetic inhibitor of the Bcl-2 family of pro-survival proteins, ABT-199. Given all these novel agents and targets, chemo-free or at least chemo-reduced concepts may become reality in the near future for our patients suffering from CLL. "}, {"id": "pubmed23n0363_6664", "title": "[CD43 in B-cell chronic lymphocytic leukemia].", "score": 0.015617403986387944, "content": "CD43 (other names: sialophorin, leukosialin, sialoglycoprotein of white blood cells) is an integral cell membrane mucin. In population of peripheral B cells CD43 occurs only on activated B cells and CD5 positive B cells. These last cells create neoplasm population in patients with B-cell chronic lymphocytic leukemia (B-CLL). Anti-CD43 monoclonal antibodies are used routinely in investigations of tissue fragments in cases of non-Hodgkin's lymphoma, whereas we did not find publication on theme of CD43 expression on peripheral blood B cells in patients with B-cell chronic lymphocytic leukemia. Wherefore advisable appeared estimation CD43 expression on B-CLL cells and comparison it with expression of typical B-CLL markers--such as CD5 and CD6. Immunological phenotype of peripheral blood and bone marrow lymphocytes has been evaluated using flow cytometry (Cytoron Absolute Ortho-Diagnostic Systems) and two-color staining. Twenty six untreated patients with B-CLL were studied. Because on well-known correlations between CD43 expression and metastasis potential of tumor, patients were divided on two groups differing score of total tumor mass (score TTM). Score TTM was evaluated according to criterion of Jaksic and Vitale. Twelve patients whose TTM score was equal or lower than 9 and median lymphocytosis was 24.6 x 10(9) in microliter were included in group I. 14 patients whose TTM score was higher than 9 were included in group II. Median lymphocytosis in these patients was 152.6 x 10(9) in microliter. The median percentage of CD43+/CD19+ cells in peripheral blood was 62.6% in the group I, and 75% in the group II (p &lt; 0.05). Median fluorescence intensity (MFI) of CD43 antigen was 87.7 in the I group comparing to 77.4 in the group II. So one observed tendency to lowering MFI during tumor growing but the difference was not significant (p = 0.25). In peripheral blood during progression of disease more clearly than CD43+ cells increased percentage of CD5+ and CD6+ cells. The median percentage of CD19+/CD5+ cells was 62.7% in the group I, 82.4% in the group II and the difference was significant (p &lt; 0.002). The difference in the median percentages CD6+/CD19+ cell 71.8% in group I and 84.3% in the II one were also significant (p &lt; 0.03). MFI of CD5 and also CD6 antigens did not change in course of disease. Moreover, examination of CD43 and CD5 expression in marrow additionally to blood study were performed in 12 cases (6 from group I, 2 from group II and 4 new not included). The median percentage of CD43+/CD19+ cell was 35.1% in blood and 43.7% In marrow, in contrast to these results was the median percentage of CD19+/CD5+ cell, which was higher in peripheral blood (70.4%) than in bone marrow (60.9%). The results of this study indicate that CD43 is present on peripheral blood B-CLL cells. Moreover, percentage of these cell increases during progression of disease however more weakly than percentage of CD5 and CD6 positive cells. Expression of CD43 is independent from expression CD5 and CD6 and diminishes during tumor mass increasing, what can depended from releases exocellular domains of CD43. CD43+ cell from B-CLL patients have a tendency to accumulation in tissues what is illustrated by higher percentage of CD43+ cell in bone marrow than in peripheral blood."}, {"id": "pubmed23n1076_182", "title": "An unknown chromosomal aberration in a patient with chronic lymphocytic leukemia: Extra isochromosome 4q.", "score": 0.015159747880895457, "content": "The genetic characterization of chronic lymphocytic leukemia (CLL) has made significant progress over the past few years. Chromosomal abnormalities are detected in up to 80% of patients. Determination of new chromosomal disorders is important in the pathogenesis and treatment facilities. A patient was diagnosed with CLL Stage 2 on 2012 and followed since then by hematology clinic. She was 63 years old. Mature, small lymphocytes, and smudge cell was found in the patient's peripheral blood smear. Bone marrow (BM) biopsy made and hypercellularity showing infiltration of atypical cells with CD5+, CD20+, and CD23+ were determined. Hypoplasia is detected in myeloid/erythroid series, and Stage 2 reticular fibers proliferation were detected. The patient was followed up without medication. While follow-up of patient's white blood cell: 57300, hemoglobin: 5.36, and PLT: 99700 are determined in May 2014. According to the patient's flow results, CD5+, CD23+, and FMC7+ were detected. Mature, small lymphocytes and smudge cell was found in the patient's peripheral blood smear. In ultrasonography imaging, multiple laps were found in the abdomen and multiple neck lymph nodes were detected. The patient BM aspiration was performed in 2014, and hypercellularity was found to contain 54% of atypical lymphocytes in the BM. Fluorescence in situ hybridization (FISH) analysis made two times in 2014. At first, FISH analysis patient's rate of 18% in RB1/13q14.2/13qter revealed a deletion of the gene regions. Patient's FISH result was reported as normal (for RB1/13q14.2/13qter) after 5 months at second analysis. Cytogenetic analysis is made from the patient's BM at the same time. According to the results of karyotyping and FISH, 47, XX, isochromosome 4q (+i4q) is determined. According to literature, extra isochromosome 4q is reported by our case for the first time in CLL. She was diagnosed with Stage 4 CLL and FISH treatment was initiated. Our patient showed disease progression compared to previous results. Hence, we offer that this evidence can be considered regarding triggering the disease's progression or as a result of disease progression i4q was occurred."}, {"id": "wiki20220301en254_2214", "title": "Oncology", "score": 0.01346813636031827, "content": "Diagnosis and staging Diagnostic and staging investigations depend on the size and type of malignancy. Blood cancer Blood investigations including hemoglobin, total leukocyte count, platelet count, peripheral smear, red cell indices. Bone marrow studies including aspiration, flow cytometry, cytogenetics, fluorescent in situ hybridisation and molecular studies. Lymphoma Excision biopsy of lymph node for histopathological examination, immunohistochemistry, and molecular studies. Blood investigations include lactate dehydrogenase (LDH), serum uric acid, and kidney function tests. Imaging tests such as computerised tomography (CT scan), positron emission tomography (PET CT). Bone marrow biopsy. Solid tumors Biopsy for histopathology and immunohistochemistry. Imaging tests like X-ray, ultrasonography, computerised tomography (CT), magnetic resonance imaging (MRI) and PET CT."}, {"id": "pubmed23n0256_10414", "title": "CD5 negative lymphocytosis mimicking typical B-chronic lymphocytic leukaemia. Description of 26 cases.", "score": 0.01323212211713954, "content": "We report 26 elderly patients (median age 68.3 years) who met diagnostic criteria for B-cell chronic lymphocytic leukaemia (B-CLL) but whose lymphocytes lacked CD5 expression. Haematological and clinical features of this CD5- series were compared with those of 333 CD5+ B-CLL patients from the same institute. No significant differences were observed regarding peripheral blood (PB) and bone marrow (BM) lymphocytosis, Hb level, platelet count, incidence of adenomegaly, hepatomegaly or splenomegaly or diffuse BM pattern. Due to an absence of nodal enlargements or to general clinical condition, lymph node biopsy was performed in only three patients, while spleen histology was examined in two cases following splenectomy. All histological results confirmed the clinical diagnosis of CLL. The distribution of the CD5- subjects according to the different staging categories proposed by Rai, Binet and Mandelli was similar to that of CD5+ subjects. Ten patients received standard chemotherapy with Chlorambucil (CHL) and Prednisone (PDN). All achieved partial remission, although one of these patients later died of disease progression; 80 months after diagnosis. We conclude that rare cases of CD5- lymphocytosis fulfilling all criteria for B-CLL may occur. Haematological and clinical features at presentation and the response to conventional treatment with Chlorambucil support our hypothesis of considering this disease as a less frequent subgroup of B-CLL."}, {"id": "pubmed23n0526_10108", "title": "Blastic transformation after splenectomy in a patient with nonvillous splenic marginal zone lymphoma with p53 overexpression: a case report.", "score": 0.012562421972534332, "content": "A 61-year-old man with no subjective symptom was admitted to our hospital for further examination of the causes of anemia (hemoglobin, 9.5 g/dL) and thrombocytopenia (platelets, 9.2 x 10(4)/microL), which had been pointed out in a medical checkup half a year previously. A bone marrow examination showed 73% lymphoid cells. Immunophenotyping of these cells were CD19+CD20+CD3-CD5-CD10-CD23-, and light chain restriction (kappa) was positive by fluorescence-activated cell sorting analysis. A computed tomography scan showed mild splenomegaly. To confirm the diagnosis histologically, we performed a splenectomy. Finally, we diagnosed the patient's disease as nonvillous splenic marginal zone lymphoma (SMZL). A month after the splenectomy, the white blood cell count was remarkably increased to 7 x 10(4)/microL with the blastic transformation of lymphoid cells. We first treated the patient with fludarabine and then with the CHOP regimen (cyclophosphamide, hydroxydaunomycin, vincristine [Oncovin], and prednisone), but the disease was so refractory that the patient died of the disease 13 months after the splenectomy. Immunohistochemical staining and a molecular examination for p53 were carried out with specimens from the splenectomy. We found overexpression of the p53 protein in lymphoid cells and a point missense mutation in codon 280 at exon 8 that changed AGA (Arg) to AGT (Ser). This case may indicate the existence of a more aggressive subset of SMZL, suggesting a reconsideration of the roles of splenectomy and p53 overexpression in the diagnostic and therapeutic approaches to patients with SMZL."}, {"id": "pubmed23n0311_7388", "title": "Analysis of residual disease in chronic lymphocytic leukemia by flow cytometry.", "score": 0.011869952659426343, "content": "We have investigated the value of both conventional and quantitative flow cytometry to detect minimal residual disease in 21 CLL patients in remission including bone marrow histology: eight in complete remission (CR), 11 in nodular partial remission (nPR) and two in PR. The techniques used were double immunostaining with CD5 and CD19 and quantitative estimation of the number of both antigens with standard microbeads. Reference values were established on normal peripheral blood and bone marrow controls. Patients were considered in 'immunological' remission when the percentage of CD5+ CD19+/total CD19+ cells was &lt;25% in PB and &lt;15% in BM. In six of the eight patients in CR, CLL cells were still detectable by flow cytometry. Only two patients, that underwent allogeneic bone marrow transplant, achieved immunological remission. CLL samples showed significantly higher CD5 and lower CD19 antigen density than normal controls (P &lt; 0.001). Persistence of residual disease was a predictor of time to progression. None of the two patients in immunological remission relapsed within a period of 13 and 33 months, whilst two of the six patients in CR with positive flow cytometry relapsed 3 and 6 months after achieving CR. This study demonstrates that flow cytometry contributes to increase the sensitivity of the clinicohematological criteria to detect residual malignant cells in CLL patients and may be useful to monitor disease status following treatment."}, {"id": "pubmed23n0863_3840", "title": "[Monoclonal B-cell lymphocytosis: from literature to laboratory practice].", "score": 0.011756756756756758, "content": "Monoclonal B-cell lymphocytosis (MBL) is defined as an asymptomatic condition characterized by the presence of less than 5,000 monoclonal B-cells per microliter and the absence of clinical signs or symptoms of a B-cell lymphoproliferative disorder. Most MBL cases involve B cells presenting an identical phenotype to CLL (CLL-like MBL) with a Catovsky-Matutes score of 3 to 5 and share the same chromosomal abnormalities than CLL. Depending on the absolute B cell count, one may distinguish low-count CLL-like MBL (&lt;500 B cells/μL) which have no evidence of progression, no reduction in overall survival, no increase in infection risk and do not require any specific follow-up. Patients with clinical CLL-like MBL (&gt;500 B cells/μL) have a 1% to 2% per year risk of progression to CLL requiring therapy, a higher risk of infectious complications and mortality implicating an annual follow-up by hematologist. MBL may also express other less common phenotypes and are named atypical MBL in case of CD5 antigen expression (Catovsky-Matutes score: 1-2) and non-CLL-like MBL for CD5 negative cases (Catovsky-Matutes score: 0-2). Their poorer prognosis implicates imaging studies, bone marrow biopsy and cytogenetic analysis in addition to physical examination in order to rule out non-hodgkinien lymphoma, and require a more frequent follow-up. This review focuses on key concepts in the classification, diagnosis, monitoring and biology of MBL in laboratory practice. "}, {"id": "pubmed23n1110_4796", "title": "Chronic lymphocytic leukemia presenting as gingival swelling and tooth mobility.", "score": 0.009900990099009901, "content": "Chronic-lymphocytic-leukemia (CLL) is the most prevalent leukemia in developed countries, caused by monoclonal proliferation of CD5+ B-cells and accumulation of mature-appearing-neoplastic lymphocytes in blood, bone marrow, and secondary lymphoid organs. Oral manifestations of CLL are infrequent and rarely reported in literature. We report a new case of a 67-year old man who presented with the complaints of tooth mobility and gingival swelling. Extra-oral examination was remarkable for cutaneous pallor and bilateral cervical lymphadenopathy involving the submandibular, and deep cervical lymph nodes on both sides of the neck. Complete blood count revealed normal red blood cell count (4.25 × 106/μl), normal platelet count (136 × 103/μl) and increased white blood cell count (25.3 × 103/μl). Differential white blood cell count showed marked lymphocytosis (88%), and blood film revealed the presence of leukocytosis, with small mature-looking lymphocytes, and mild thrombocytopenia. A flow cytometry immune-phenotyping revealed that 55% of peripheral blood cells were monoclonal B-lymphocytes expressing CD19, CD20, CD23, CD200, CD22, CD5, CD38, CD11c, sIgD and Kappa light chain confirming the diagnosis of CLL. Oral healthcare professionals should consider systemic causes, such as CLL, in the differential diagnosis of generalized tooth mobility and gingival swelling, particularly in patients with associated symptoms such as lymphadenopathy, fever, weight loss, and general fatigue."}, {"id": "article-24574_30", "title": "Lymphocytosis -- Evaluation", "score": 0.009828543829992755, "content": "Additional testing: Flow Cytometry: Peripheral blood flow cytometry is essential to determine the proliferation of monoclonal cells. It is a costly test and should not be ordered routinely on all patients with lymphocytosis. Certain features on peripheral blood smear or on review of CBC which prompt a physician to order flow cytometry include: The presence of lymphoblasts on the peripheral blood smear, suggesting ALL- This should also prompt a referral to a tertiary care center to obtain further workup. The presence of other abnormal lymphocyte morphology on PBS as detailed above ALC more than 30000 cells/microL Persistent unexplained lymphocytosis for more than one month Abnormalities in other cell lines including anemia and thrombocytopenia Presence of lymphadenopathy and/or hepatosplenomegaly in the right clinical context where reactive causes have been ruled out Flow cytometry patterns are beneficial in delineating clonality and differentiating clonal disorders as follows: CLL: lymphocytes are CD5+, CD23+, CD20 (dim), CD10-, cyclin D1-, weak sIg (surface immunoglobulin), FMC -, CD200 + MCL: lymphocytes are CD5+, CD23-, CD10-, CD20+, sIg +, cyclin D1 +, FMC +, CD200- FL: lymphocytes are CD5-, CD10+, CD20+, sIg+ and often BCL2+, BCL6+ MZL: lymphocytes are CD5-, CD10-, CD20+, sIg+, cyclin D1- HCL: lymphocytes are CD5-, CD10-, CD20+, sIg+, CD11c+, CD25+, CD103+ T-LGL: lymphocytes are CD3+, CD8+, CD16+, CD56+ Fluorescence in situ hybridization (FISH), karyotype, and mutation analysis: FISH, karyotype, and certain mutation analysis help diagnose and risk stratification of hematologic malignancies, especially CLL and lymphomas. The FISH can not only help to ascertain the clonal nature of lymphocytosis but also helps to confirm the diagnosis of certain lymphomas. Examples include: FL: characterized by t(14,18) MCL: characterized by t(11,14) HCL: characterized by the presence of BRAF mutation CLL: different karyotypic abnormalities including del 17p, del 11q, trisomy 12, and del 13q14."}, {"id": "pubmed23n0874_13088", "title": "Concomitant Presence of Two Distinct Clones of Chronic Lymphocytic Leukemia and Plasma Cell Myeloma in a Patient.", "score": 0.00980392156862745, "content": "A 74 years old male patient, presented with history of generalized weakness, fatigue, loss of appetite and breathlessness on exertion for past one and a half months. On examination, he was found to have significant pallor and generalized lymphadenopathy (cervical, axillary and inguinal). The skeletal survey showed punched out lytic lesions in skull and pelvic bones. The peripheral smear examination showed lymphocytosis with absolute lymphocyte count of 25,000/μL. The bone marrow aspirates revealed a hypercellular marrow with 74 % lymphocytes &amp; 14 % plasma cells, suggestive of chronic lymphoplasmacytic disorder. The bone marrow biopsy had two morphologically distinct populations of lymphocytes &amp; plasma cells. The immunohistochemical markers on bone marrow biopsy showed hat plasma cells were positive for CD138 with kappa light chain restriction. Flow cytometry showed B cell population with CD19/CD5 co expression, CD5/CD23 coexpression, were positive for CD22, CD20 and negative for FMC-7 and lambda light chain. In addition, plasma cells were also identified as CD45 negative cells and showed CD38/CD138 co-expression with variable CD19 and CD56 positivity. Serum protein electrophoresis revealed M band, serum immunofixation electrophoresis corresponded to IgA -Kappa. The final diagnosis of chronic lymphocytic leukemia with concomittant presence of plasma cell myeloma was concluded. This case imparts an important message to look for presence of coexisting entities in a single specimen and highlights the benefits of testing both plasma cell and B-cell compartments when the clinical features are not entirely consistent Flow cytometry together with protein electrophoresis can help to clinch difficult and rare dual diagnosis. These cases are rare and pose therapeutic challenge. "}, {"id": "pubmed23n0834_3336", "title": "Antigen Expression on Blast Cells and Hematological Parameters at Presentation in Acute Lymphoblastic Leukemia Patients.", "score": 0.009708737864077669, "content": "To analyze the expression of various antigens on the leukemic blasts and to determine the hematological parameters, in Acute Lymphoblastic Leukemia (ALL) patients at presentation. Observational study. King Edward Medical University, Lahore and Hameed Latif Hospital, Lahore, from February 2013 to March 2014. A total of 50 newly diagnosed and untreated patients of ALL were selected from Mayo Hospital and Hameed Latif Hospital. These patients included both genders and all age groups. Hemoglobin, total leukocyte count and platelet count were determined on hematology analyser-Sysmex-Kx-2I. Blast cell percentage was estimated on Giemsa stained blood smears. Immunophenotyping was done on bone marrow samples by 5 colour flowcytometery on Beckman Coulter Navious Flowcytometer. An acute leukemia panel of 23 antibodies was used. The data was entered and analyzed in SPSS version 22. Of the 50 ALL patients, 36 (72%) were B-ALL and 14 (28%) T-ALL. There were 18 (36%) children and 32 (64%) adults. T-ALL included 22% of the childhood and 31% of the adult cases. Immunophenotypic analysis showed that CD19, CD79a and CD20 were B-lineage specific markers whereas cCD3, CD3 and CD5 were T-lineage specific. CD10 was the most sensitive marker for B-ALL and CD7 was the most sensitive marker of T-ALL. TdT was expressed in 92% B-ALL and 71% T-ALL cases, CD34 in 58% and 43% cases and CD45 in 83% and 100% respectively. High leukocyte count (&gt; 50 x 109/L) was present in 58% cases. Hemoglobin was &lt; 10 g/dl in 74% patients and platelet count was below 20 x 109/Lin 12% patients. Leukocyte count, hemoglobin, platelet count and blast cell % did not show a significant difference in the two ALL immunotypes. The frequency of T-ALL is higher in childhood as well as adult ALL in our population compared to the Western literature. Antigenic expression of the blast cells also shows some interesting differences. A large number of our patients present with high leukocyte count which is a known factor associated with poor prognosis."}, {"id": "pubmed23n1058_1052", "title": "High-Grade Epstein-Barr Virus-Negative Biphenotypic Lymphoma with Expression of B- and T-Cell Markers and Leukemia Presentation: Case Report and Literature Review.", "score": 0.009615384615384616, "content": "Lymphomas are presently categorized according to their origin from B or T lymphocytes. The co-expression of CD3 in B-cell lymphomas or CD20 in T-cell lymphomas has been rarely reported. Immature and less often mature lymphomas may incorporate the rearrangements of both B- and T-cell antigen receptor genes (dual genotype or bigenotype). Lymphoma cells with a sole genotype hardly concurrently express both B- and T-cell markers (biphenotypic lymphomas). We describe a 63-year-old female who was presented with obstructive jaundice and epigastric pain of 10 days. Initial CBC revealed 43×10&lt;sup&gt;3&lt;/sup&gt;/μL white blood cells, 11.2 g/dL hemoglobin, and 88x10&lt;sup&gt;3&lt;/sup&gt;/μL platelets. CT abdomen revealed hepatomegaly and suspected pancreatic mass with large retroperitoneal lymph nodal mass. Peripheral smear showed 56% lymphoid cells with blast morphology. The bone marrow (BM) aspirate smear was infiltrated by 83% immature-looking cells. BM biopsy showed interstitial to diffuse extensive infiltration by primitive-looking cells, positive for pan-B-cell antigens CD20, CD79, and PAX5 as well as the T-cell antigen CD4, CD5, CD3, while negative for all immaturity markers (CD34, TdT, and CD1a). In situ hybridization for Epstein-Barr virus (EBV)-encoded small RNA (EBER) was negative. Flow cytometry on BM aspirate showed an abnormal population (50%) expressing the B-cell antigens (CD19, CD20, CD79, CD22) and CD10, and showed lambda light chain restriction as well as the T-cell antigens cCD3 and CD4 with partial CD5. The analysis showed, also, another abnormal population of lambda restricted monotypic B cells (8%) with dimmer CD45 (blast gate) and showed the same immunophenotype (expressing the B-cell antigens), but negative for CD10, cCD3, CD5, and CD4. Conventional cytogenetic revealed complex karyotype. Molecular studies revealed rearrangements of the immunoglobulin heavy chain region consistent with a clonal B-cell population. TCR gene rearrangement analysis was equivocal concerning clonality but was not conclusive for clonal T-cell disease. Our final diagnosis was peripheral blood and BM involvement by EBV-negative high-grade lymphoid neoplasm (in leukemic phase with blast morphology) and an ambiguous immunophenotype with a differential diagnosis that may include the rare entity of bigenotypic lymphoma or an unusual case of high-grade B-cell lymphoma with aberrant expression of T-cell markers (biphenotypic lymphomas)."}, {"id": "pubmed23n0375_22046", "title": "[Monitoring minimal residual disease in patients with hairy cell leukemia in complete remission after treatment with 2-chlorodioxyadenosine].", "score": 0.009615384615384616, "content": "Treatment of hairy cell leukemia with 2-chlorodeoxyadenosine (2-CdA) induces in 85% patients complete remission. Complete remission is defined as the condition when signs of activity of the disease are absent, splenomegaly and lymphadenopathy are absent, the hemoglobin concentration is &gt; or = 120 g/l, the absolute number of granulocytes is &gt; or = 1.5 x 10(9)/l and the number of thrombocytes is &gt; or = 100 x 10(9)/l. In complete remission in the peripheral blood, bone marrow aspirate and bioptic samples obtained by trephin bone marrow core biopsy, using standard staining (hematoxylin-eosin and May-Grünwald-Giemsa's method), no leukemic cells are present. When more sensitive methods are used (immunophenotyping, immunohistochemistry or molecular genetic methods), a persisting leukemic population can be detected which is described as minimal residual disease (MRD). For detection of MRD the authors used immunohistochemical examination of bone marrow with DBA.44 antibodies. As leukemic cells they described those which produced intense cytoplasmic and membrane positivity with antibody DBA.44 and corresponded morphologically to hairy cells. For evaluation computer analysis of the picture LUCIA-M was used. The infiltration grade was examined on three areas of standard size (3 x 65,265 micron 2) and expressed in percent. A total of 45 trepanobioptic specimens from 21 patients were examined who achieved after treatment with 2-CdA complete remission. In all samples suitable for evaluation the presence of leukemic cells (MRD) was detected with a median of 3% and a range of 1% to 18%. With induction of complete remission correlates also the low serum level of the soluble receptor for IL-2 (sIL-2R). In a female patient after 24 months of treatment with 2-CdA the grade of leukemic infiltration rose from 1% to 12% and during the 36th month to 50% DBA.44+ leukemic cells. The incipient relapse in this patient was not associated, despite marked infiltration of bone marrow, with failure of hematopoiesis and a marked rise of sIL-2R."}, {"id": "pubmed23n0735_5890", "title": "A 61-year-old man presented with myopathy, neuropathy, and inflammatory dermatitis responsive to chronic lymphocytic leukemia treatment.", "score": 0.009523809523809525, "content": "The prevalence of paraneoplastic neurologic syndrome in cancer is 0.01%. Neurological syndromes can be seen in chronic lymphocytic leukemia (CLL) and mostly present as either leukemic infiltration of the central nervous system (CNS) or progressive multifocal leukoencephalopathy. To our knowledge, this is the first reported case of combined sensory-motor neuropathy, myopathy, and dermatitis in a patient with CLL. A 61-year-old African American man presented with acute dysphagia, rapidly progressive proximal limb-girdle weakness, and dermatitis. He had a white blood cell (WBC) count of 14,600/mm(3), hemoglobin of 11.4 mg/dL, and a platelet count of 165,000/mm(3). Lymphocytes comprised 15% of the total WBC with an absolute lymphocyte count of 2100/mm(3). Metabolic profile was unremarkable except for a serum creatine phosphokinase (CPK) level of 1056 mg/dL. Serum protein electrophoresis, serologic studies for autoimmune, genetic diseases, and paraneoplastic syndromes were all negative. Electrodiagnostic studies revealed sensorimotor neuropathy with mixed axonal and demyelinating features. Muscle biopsy revealed discrete areas of interstitial fibrosis juxtaposed to areas of intact muscle without any inflammation. At that point, a bone marrow biopsy was done because of anemia and slightly elevated mean corpuscular volume of 103. Bone marrow biopsy revealed minimal involvement with CD5/CD19-positive CLL. Flow cytometry demonstrated monoclonal CD5/CD19/CD20/CD23-positive cells, with dim kappa expression, and negative FMC-7 and CD3. This case doesn't meet the criteria for CLL/small lymphocytic lymphoma. However, considering the possibility of paraneoplastic phenomenon for his symptoms, it was decided to start the patient on CLL-directed therapy with Rituximab and Cyclophosphamide. After only two cycles, the patient experienced a dramatic improvement in his muscle strength with disappearance of the rash. This case highlights a unique clinical picture of inflammatory dermatitis with electromyography and biopsy findings suggestive of myopathy and combined sensorimotor neuropathy with response to CLL-directed therapy. Also the symptoms started before peripheral lymphocytosis which masked the diagnosis for over a year."}, {"id": "pubmed23n0346_332", "title": "[Mantle cell lymphoma as a diagnostic and therapeutic problem].", "score": 0.009523809523809525, "content": "The authors present the characteristics of a group of 23 patients with mantle cell lymphoma. In the group only a slight predominance of men over women was found (1.1:1), the median age was 63 years. Twenty-one (91%) of the patients were diagnosed in stage IV (Ann Arbor). In all these patients the bone marrow was affected. In 19 of them immunoflowcytometric analysis revealed the typical clone of B lymphocytes (CD5 positive)/CD 23 negative). The majority of patients had at the time of diagnosis a large tumourous mass with massive splenomegaly (61%), hepatomegaly (57%) and bulky disease (52%). The node was excised in 17 patients, but in four patients (24%) during the first session the diagnosis was not assessed correctly. In the laboratory findings an inclination to anaemia, thrombocytopenia, lymphocytosis and in particular to high levels of serological indicators of activity of the disease dominated--lactate dehydrogenase, beta-2-microglobulin and serum thymidine kinase. All patients were treated by chemotherapy. Complete remission was achieved by the date of evaluation in one patient (4%), partial remission in seven patients (30%) but 48% patients did not respond to first line treatment. Nine patients of the group died, their median of survival was 14 months (0-24), the median of the follow up of the remaining patients was 133 months (2-31). Two female patients had large-dose treatment with subsequent administration of autologous stem cells. The first one is after 370 days of treatment in complete remission, the second one developed a relapse 100 days after the procedure. From the results and analysis of the literature ensues that mantle cell lymphoma is one of the aggressive malignant B-lymphoproliferations with a very adverse prognosis and it deserves therefore special diagnostic and intense therapeutic attention."}, {"id": "pubmed23n0657_12622", "title": "A rare occurrence of hairy cell leukemia in the Thai population: a case report.", "score": 0.009433962264150943, "content": "Hairy cell leukemia (HCL) has been mainly reported from the Western countries. Herein we describe a case of HCL diagnosed in a Thai patient. A 36-year-old man presented with abdominal discomfort, frequent gum bleeding and significant weight loss for 2 months. Physical examination revealed moderate anemia, petechial hemorrhage on the extremities and an enlarged spleen down to the umbilicus. No hepatomegaly or lymphadenopathy was detected. Complete blood counts revealed a hemoglobin (Hb) of 6.6 g/dL, a white blood cell (WBC) count of 1.6 x 10(9)/L (neutrophil 16%, lymphocyte 71%, monocyte 11%, atypical lymphocyte 1%), and a platelet (PLT) count of 17 x 10(9)/L. Abnormal large mononuclear cells with villous projections were seen in the blood smear. Although bone marrow (BM) aspiration resulted in a dry tap, abnormal lymphocytes with villous projections could again be identified in the touch preparation. Flow cytometric analysis showed a distinct population above the normal lymphocyte region on CD45/SSC gates with a strong expression of CD19, CD20, CD22, CD25, CD11c, and kappa. CD5, CD23, CD10, CD4, and CD8 were all negative. BM biopsy was consistent with HCL. The patient was treated with splenectomy followed by 8 cycles of fludarabine and cyclophosphamide chemotherapy. At 21 months after diagnosis, the patient was doing well with a Hb of 16.9 g/dl, a WBC count of 6.8 x 10(9)/L, neutrophil 49.9%, lymphocyte 39.6%, monocyte 8.6%, and a PLT count of 329 x 10(9)/L). No abnormal lymphoid cells were detected in the blood smear. This present report represents the first Thai HCL case that was immunophenotypically confirmed by flow cytometry and successfully treated at Siriraj Hospital."}, {"id": "pubmed23n0259_17791", "title": "Atypical chronic lymphocytic leukaemia with t(11;14)(q13;q32): karyotype evolution and prolymphocytic transformation.", "score": 0.009433962264150943, "content": "In order to define better the cytological and clinical features of atypical B-cell chronic lymphocytic leukaemia (B-CLL) with t(11:14)(q13;q32), sequential morphologic immunological and cytogenetic studies were performed in seven patients belonging to a series of 72 consecutive cases presenting with a diagnosis of CLL or atypical CLL according to the FAB criteria. Cytologic diagnosis in these seven patients with t(11;14) was typical CLL in two cases presenting with &lt; 10% large lymphocytes (LL) and prolymphocytes (PL) and atypical CLL in five cases in which LL and PL comprised between 10% and 55%. The diagnosis was supported by histologic findings on bone marrow biopsy (five cases) or splenectomy specimens (two cases). A progressive increase of peripheral LL and PL was observed, resulting in a switch of FAB diagnosis over a 6-60-month period from typical CLL into atypical CLL in two cases and from atypical CLL into prolymphocytic leukaemia in five cases. Immunophenotyping showed a mature B-cell phenotype with CD19, CD22, CD24 positivity and CD10 negativity in all patients. A bright-staining pattern for surface immunoglobulins (SIg) was detected in 6/7 cases, CD5 positivity in 6/7 cases, and CD23 positivity in 1/7 cases. The FMC-7 monoclonal antibody was positive in &gt; 40% cells in 5/6 cases. Chromosome changes in addition to t(11;14) were seen in five cases; in two cases unbalanced translocations involving the 3q21 chromosome region, resulting in partial trisomy for the long arm of chromosome 3, were detected early in the course of the disease. Karyotype evolution that was associated with disease progression occurred in 3/6 assessable patients. Comparison of these findings with similar data from 65 B-CLL patients without t(11:14) showed that atypical morphology, switch of FAB diagnosis during the course of the disease, and karyotype evolution were more frequently seen in cases with t(11;14) (5/7 v 15/65 cases, P = 0.015, 7/7 v 7/65 cases, P &lt; 0.0001, and 3/6 v 5/45 assessable cases, P = 0.04, respectively). The frequency of positivity for CD23 and bright SIg staining differed significantly in the two groups. It is concluded that t(11;14) identifies a cytologically atypical subset of B-CLL, characterized by frequent cytologic and cytogenetic evolution and by a distinct immunological profile, sharing some biological features with mantle cell lymphoma."}, {"id": "pubmed23n0932_6385", "title": "The sorafenib anti-relapse effect after alloHSCT is associated with heightened alloreactivity and accumulation of CD8+PD-1+ (CD279+) lymphocytes in marrow.", "score": 0.009345794392523364, "content": "We studied three FLT3 ITD acute myeloid leukemia (AML) patients who relapsed after allogeneic haematopoietic stem cell transplantation (alloHSCT) and received multikinase inhibitor (MKI) sorafenib as part of salvage therapy. MKI was given to block the effect of FLT3 ITD mutation which powers proliferation of blast cells. However, the known facts that sorafenib is more effective in patents post alloHSCT suggested that this MKI can augment the immune system surveillance on leukaemia. In the present study, we investigated in depth the effect of sorafenib on the alloreactivity seen post-transplant including that on leukaemia. The patients (i) responded to the treatment with cessation of blasts which lasted 1, 17 and 42+ months, (ii) developed skin lesions with CD3+ cell invasion of the epidermis, (iii) had marrow infiltrated with CD8+ lymphocytes which co-expressed PD-1 (programmed cell death protein 1 receptor, CD279) in higher proportions than those in the blood (163±32 x103 cells/μl vs 38±8 x103 cells/μl, p&lt;0.001). The Lymphoprep fraction of marrow cells investigated for the expression of genes involved in lymphocyte activation showed in the patients with long lasting complete remission (CR) a similar pattern characterized by (i) a low expression of nitric oxide synthase 2 (NOS2) and colony stimulating factor 2 (CSF2) as well as that of angiopoietin-like 4 (ANGPTL4) (supporting the immune response and anti-angiogenic) genes, and (ii) higher expression of fibroblast growth factor 1 (FGF1) and collagen type IV alpha 3 chain (COL4A3) as well as toll like receptor 9 (TLR9) and interleukin-12 (IL-12) (pro-inflammatory expression profile) genes as compared with the normal individual. The positive effect in one patient hardly justified the presence of unwanted effects (progressive chronic graft-versus-host disease (cGvHD) and avascular necrosis of the femur), which were in contrast negligible in the other patient. The anti-leukemic and unwanted effects of sorafenib do not rely on each other."}, {"id": "wiki20220301en566_561", "title": "Epstein–Barr virus-associated lymphoproliferative diseases", "score": 0.009259259259259259, "content": "receptor, and, in EBV+ HLH cases, circulating EBV. In the latter cases, histological examination of lymphatic, bone marrow, liver, neuronal, and other involved tissues show infiltrations of small EBV+ T cells, scattered small bystander EBV+ B cells, reactive histiocytes, reactive macrophages, and, in ~70% of cases, hemophagocytosis, i.e. ingestion of erythrocytes, leukocytes, platelets, and/or their precursor cells by histiocytes and macrophages. (Evidence of hemophagocytosis is not critical for the diagnosis of HLH.) The EBV in infected lymphocytes is in its lytic cycle rather than any latent phase. Criteria consistent with the diagnosis of HLH, as developed by the Histiocytic Society (2004), include finding five of the eight following signs or symptoms: fever ≥38.5 °C; splenomegaly; low blood levels of any two of the following, hemoglobin (<10 mg/L), platelets (<100,000/μL), or neutrophils <1,000/μl; either one or both of the following, blood fasting triglyceride levels >265 mg/dL"}, {"id": "pubmed23n0079_1903", "title": "[DBMP-85 was effective at diagnosis and LVP was effective at relapse in a case of acute mixed leukemia].", "score": 0.009259259259259259, "content": "A 16 year-old boy was admitted to our hospital in April 1985, because of bilateral submandibular swellings. Hematological examination revealed Hb was 7.3 g/dl, WBC was 89,000/microliters (76% blast), and platelet was 154,000/microliters. His bone marrow was hypercellular and consisted with 91% blasts. Myeloperoxidase staining was positive for 38% of blasts. Auer rods were seen in some of blasts. Thus, the diagnosis was M1 according to FAB classification. Cytogenetic studies of 20 marrow cells were performed and all cells had 46, XY, -1, -7, 3q-, 7q-, 17q+, +2mar. Eighty five percent of blasts expressed HLA-DR and 43% of blasts expressed CD2 and CD13 simultaneously. Thus, this leukemia was considered as the hybrid type of acute mixed leukemia by surface marker analysis. DBMP-85 regimen, the chemotherapy for AML, was started after admission and complete remission (CR) was attained in June 1985. After 4 courses of post remission chemotherapy, he discharged in December 1985 and was followed at our outpatient clinic without chemotherapy. His disease was relapsed in June 1986, and the combination chemotherapy with mitoxantrone, etoposide and Ara-C was applied to him but failed to attain CR. Then, LVP protocol, the chemotherapy for ALL, was started and CR was achieved. The blasts at relapse had morphologically myeloid features, and expressed HLA-DR, CD2 and CD13 as well as at diagnosis. Cytogenetic studies at relapse showed some karyotype except gaining 12p- anomaly. Therefore, same blasts were considered to emerge at relapse. Our case suggests that LVP therapy may be effective for AML expressing myeloid and lymphoid surface markers."}, {"id": "pubmed23n0792_7780", "title": "[Identification of splenic marginal zone lymphoma from B lymphoproliferative disorders by flow cytometry].", "score": 0.009174311926605505, "content": "The splenic marginal zone lymphoma (SMZL) is a relatively rare chronic B lymphoproliferative disease, which primarily manifest increase of peripheral blood lymphocyte count and/or scale, and splenomegaly, while the peripheral superficial lymph nodes are often not swollen. Therefore, the splenectomy are usually needed to confirm the diagnosis, but the majority of patients could not accept such management, resulting in early difficult diagnosis. This study was purposed to explore the more prior way for diagnosis based flow cytometry (FCM). Six patients with suspected diagnosis of SMZL were used as research objects, 10 healthy bone marrow donors and 10 cases of chronic lymphocytic leukemia (CLL), 3 cases of hairy cell leukemia (HCL), 3 cases of lymphatic plasma cell lymphoma/Waldenströ's macroglobulinemia (LPL/WM) were selected as control. The immunophenotype of bone marrow cells were analyzed and compared by FCM using a panel of antibodies including CD45, CD5, CD10, CD19, CD20, CD22, CD23, CD25, CD103, CD11c, CD123, κ,λ, Cyclin D1, and combined with bone marrow cell morphology. The results indicated that 6 cases of suspected SMZL showed a large increase of lymphocytes and splenomegaly. Because absence of peripheral lymphadenopathy, 6 patients did not suffer from lymph node biopsy, only 1 patient underwent diagnostic splenectomy. The immunophenotypes of bone marrow in patients and controls were analyzed by FCM, as a result, except for the healthy donors, varying degrees of abnormal mature B cell clones were found in bone marrow of all patients, and the further differentiation from other B-cell tumors was performed through CD5, CD10 expression and combination with other B-cell phenotype. All 6 cases of SMZL patients expressed CD19(+) and CD20(+), but CD10 expression was negative, 4 patients expressed CD5(-), 2 patients expressed CD5(+). The expressions of CD23, CD38, ZAP-70, CD11c, CD103, CD123, Cyclin D1 were negative. The morphological examination of bone marrow cells showed velutinous abnormal lymphocytes. Combined with clinical characteristics, 6 patients were diagnosed as SMZL, 1 patient suffered from splenectomy because of concurrent hypersplenism, and this postoperative pathologic examination confirmed the patient with SMZL. Ten cases of CLL mainly expressed CD5, CD23; 3 cases of HCL had more typical morphology of \"hair like\" in addition to CD11c, CD103 and CD123 positive; 3 cases of LPL/WM had significantly increased light chain restriction expression, IgM, plasmacytoid lymphocytes. It is concluded that the FCM immunophenotype analysis can be used as a powerful tools for clinical diagnosis of SMZL."}, {"id": "pubmed23n0347_22507", "title": "[Detection of PNH clones using flow cytometry in aplastic anemia and paroxysmal nocturnal hemoglobinuria].", "score": 0.009174311926605505, "content": "To detect and quantify by flow cytometry (FC) PNH clones in paroxysmal nocturnal haemoglobinuria (PNH) and aplastic anaemia (AA) patients. We have performed a flow cytometric analysis to determine the granulocyte expression of CD55 and CD59 from 29 patients with AA and 11 patients with PNH. In the 11 PNH patients the study showed 58 +/- 34% and 56 +/- 32% (mean +/- SD) CD55(-) y CD59(-) granulocytes. A good correlation was found between the results of FC and haemolysis. The follow-up study showed PNH clone progression in one case and stability in 5 cases. Among 11 AA patients studied at diagnosis, two presented a population of CD55(-) granulocytes (14% and 48%) with CD59 normal, this defect disappeared in both patients after immunosuppressive therapy. The FC study revealed PNH clones in 7 cases among the 26 analyzed after treatment (23 with ATG and/or CyA), in 3 cases with negative Ham's test (in two this became positive 6 and 12 months later). The mean values obtained in these 7 patients with PNH-AA syndrome were 26 +/- 15% y 36 +/- 30% (mean +/- SD) CD55(-) and CD59(-) granulocytes. The median time from diagnosis to detection of PNH phenomenon was 83 months. In the follow-up study, 4 cases had stability, one case had a decrease and one a progression of the abnormal clone. In a retrospective analysis, among the 7 patients with PNH-AA syndrome, 5 had a partial response after the initial treatment. The FC on granulocytes is a useful method to diagnose and characterize PNH. This test is good for early detection of PNH clones in AA patients at initial diagnosis and in long term survivors. In both diseases it permits measuring the extent of the abnormal clone and its follow up. The extent of the defect is more related to haemolysis than the haematopoietic deficiency. PNH development seems to be more frequent in AA patients with incomplete response after immunosuppressive therapy and in some cases the defect could be latent at the time of diagnosis."}, {"id": "wiki20220301en018_98872", "title": "Chronic lymphocytic leukemia", "score": 0.009094575519257915, "content": "Diagnosis The diagnosis of CLL is based on the demonstration of an abnormal population of B lymphocytes in the blood, bone marrow, or tissues that display an unusual but characteristic pattern of molecules on the cell surface. CLL is usually first suspected by a diagnosis of lymphocytosis, an increase in a type of white blood cell, on a complete blood count test. This frequently is an incidental finding on a routine physician visit. Most often the lymphocyte count is greater than 5000 cells per microliter (µl) of blood but can be much higher. The presence of lymphocytosis in an person who is elderly should raise strong suspicion for CLL, and a confirmatory diagnostic test, in particular flow cytometry, should be performed unless clinically unnecessary."}, {"id": "wiki20220301en254_37373", "title": "Monoclonal B-cell lymphocytosis", "score": 0.00909090909090909, "content": "MLB falls into three phenotypes that are distinguished based on the cell surface marker proteins which they express viz., the CLL/SLL, atypical CLL/SLL, and non-CLL/SLL phenotypes. These markers are: CD5, CD19, CD20, CD23, and immunoglobulins (Ig) (either Ig light chains or complete Ig, i.e. light chains bound to Ig heavy chains. Distinguishing between these phenotypes is important because they progress to different lymphocyte malignancies. The following table gives the markers for the three MBL phenotypes with (+) indicating the expression (either dim, moderate, or bright depending or the intensity of their expression), (−) indicating the absence of expression, and na indicating not applicable as determined using fluorescent probes that bind the marker proteins. Detection of fluorescent probe binding by the cells requires the use of flow cytometry preferably employing 6 to 8 different fluorescent probes that bind to different markers on 5 million cells from the patient's blood. The"}, {"id": "pubmed23n0682_19246", "title": "White blood cell count at diagnosis and immunoglobulin variable region gene mutations are independent predictors of treatment-free survival in young patients with stage A chronic lymphocytic leukemia.", "score": 0.00909090909090909, "content": "A comprehensive panel of clinical-biological parameters was prospectively evaluated at presentation in 112 patients with chronic lymphocytic leukemia (&lt;65 years), to predict the risk of progression in early stage disease. Eighty-one percent were in Binet stage A, 19% in stages B/C. Treatment-free survival was evaluated as the time from diagnosis to first treatment, death or last follow up. In univariate analysis, advanced stage, hemoglobin, platelets, white blood cell, leukemic lymphocyte count, raised beta 2-microglobulin and LDH, unmutated immunoglobulin variable region genes, CD38, del(17p), del(11q) and +12, were significantly associated with a short treatment-free survival; the T/leukemic lymphocyte ratio was associated with a better outcome. Multivariate analysis of treatment-free survival in stage A patients selected a high white blood cell count and unmutated immunoglobulin variable region genes as unfavorable prognostic factors and a high T/leukemic lymphocyte ratio as a favorable one. At diagnosis, these parameters independently predict the risk of progression in stage A chronic lymphocytic leukemia patients."}, {"id": "wiki20220301en179_39478", "title": "B-cell prolymphocytic leukemia", "score": 0.009009009009009009, "content": "Immunophenotype This technique is used to study proteins expressed in cells using immunologic markers. In B-PLL patients there is strong expression of surface immunoglobulin – a membrane-bound form of an antibody, b-lymphocyte surface antigens CD19, CD20, CD22, CD79a and FMC7, and weak expression of CD5 and CD23. Due to the similarities among lymphoproliferative disorders, it is often difficult to diagnose patients. Immunophenotyping helps distinguish B-PLL from similar diseases, one of its key identifiers is the absence in expression of the surface antigens CD10, CD11c, CD25, CD103 and cyclin D1 – an important regulator of cell-cycle progression. A case has been described as CD20+, CD22+, and CD5-. It can also be CD5+. Another case was described as CD45+, CD19+, CD20+, CD5+, HLA-DR+, CD10-, CD23+/-, CD38+ and FMC7-."}, {"id": "pubmed23n0673_21307", "title": "[Minimal residual disease monitoring by flow cytometry in children with acute lymphoblastic leukemia].", "score": 0.009009009009009009, "content": "The cells that have avoided the action of antitumor drugs may be retained after remission achievement during induction therapy and consolidation. A combination of these cells is given the name minimal residual disease (MRD). Multicolor flow cytometry has recently attracted considerable interest as the most promising method for measuring the content of residual tumor blasts. This technique is based on the detection of the so-called leukemia-associated immunophenotype (LAIP), i.e., a tumor-specific combination of the expression of membrane and cytoplasmic markers. Flow cytometry may be successfully used to monitor MRD in 90-95% cases of acute lymphoblastic leukemia (ALL) and in 80-85% of patients with acute myelocytic leukemia. The sensitivity of flow cytometry, which is real for routine flow techniques, is a possibility of identifying one cell among 10(4)-10(5) cells. Multicolor flow cytometry (that involves the simultaneous analysis of the expression of a few markers) is the most reasonable tool for MRD monitoring. The monoclonal antibody panels recommended by different groups of investigators for MRD monitoring in B-lineage ALL include antibodies to the pan-B-cell antigen CD19, markers of different stages of differentiation of B-lineage precursors of CD10, CD34, and CD20 and leukemia-associated markers different for each panel, such as CD22, CD38, CD58, CD45, TdT, CD13, CD33. The hyperexpression of CD10, CD34, CD19, TdT, the decreased expression of CD38, CD45, CD22, CD19, the simultaneous expression of markers of different stages of differentiation of B lymphocytes, such as CD10 and CD20, and the lymphoblast coexpression of myeloid markers of CD13, CD33, CDS15 are the most frequently described immunophenotype aberrations in B-lineage ALL. The selection of combinations of markers for MRD monitoring in children with T-ALL is based on the simultaneous expression of combinations of the antigens characteristic for early stages of differentiation of normal T lymphocytes, namely TdT and cytoplasmic CD3. Some authors consider the use of CD99 versus TdT to be most appropriate. There is recent evidence that MRD-positive patients have a higher cumulative risk for recurrences as compared with those without residual blasts. Moreover, the longer the tumor cells are retained during therapy, the worse the prognosis is. Thus, for choice of the adequate intensity of antitumor therapy, it is necessary to qualitatively and quantitatively assess MRD by multicolor flow cytometry at different stages of therapy."}, {"id": "pubmed23n0749_1875", "title": "A case of chronic lymphocytic leukemia with massive ascites.", "score": 0.008928571428571428, "content": "An 81-year old woman with a history of chronic lymphocytic leukemia (CLL) was admitted with night sweats and abdominal distension. A complete blood count showed hemoglobin 5 g/dL, white blood cell (WBC) count 28.5×10(9)/L and platelets 38.4×10(9)/L. Peripheral blood smear examination showed a large number of smudge cells and lymphocytosis composed of mature-looking lymphocytes with clumped nuclear chromatin. Computed tomography scan demonstrated enlarged cervical, axillary, paraaortic, retroperitoneal and mesenteric lymph nodes with concomitant omental thickening and ascites. Also, the liver and the spleen were enlarged in the presence of multiple ill-defined hypoechoic areas in the latter. Histopathological analysis of the cervical lymph node biopsy was consistent with CLL. Bone marrow examination showed diffuse infiltration of the marrow with small lymphocytes. Analysis of the ascitic fluid revealed an exudate with WBC 1220 cells/mL. Cytocentrifuge preparation of the ascitic fluid showed small mature lymphoid cells containing hyperchromatic nuclei with coarsely granular chromatin. On flow cytometric analysis of the ascitic fluid, expression of CD5, CD19, CD20, CD22, CD23, CD45 and HLA-DR was compatible with a diagnosis of CLL, in accordance with the results of the peripheral blood analysis. The patient was treated with chemotherapy consisting of cyclophosphamide, vincristine and prednisolone but died within one month after development of non-chylous ascites."}, {"id": "pubmed23n0694_17017", "title": "[The limited possibility of using a simplified approach to detect minimal residual disease by the flow cytometry technique in children with precursor B-lineage acute lymphoblastic leukemia].", "score": 0.008928571428571428, "content": "Minimal residue disease (MRD) is a state in which the tumor cells remain in the patient in the amounts unrecognizable with the standard cytological techniques. Flow cytometry is one of the basic methods for evaluation of MRD in precursor B-lineage acute lymphoblastic leukemia (PBLALL). The so-called simplified three-color analysis using the combination of CD19/CD10/CD34 antibodies has been proposed to detect MRD in the midcourse of induction therapy. Four-to-nine-color is presently used to identify MRD. One hundred and thirty-four bone marrow samples taken at different stages of therapy in 55 children with PBLALL were examined to estimate the possibility of using the flow cytometry technique using the 3-color simplified approach to determining MRD. The results of the simplified and standard approaches were compared in the samples stained with 6-8 monoclonal antibodies in the combinations that always included CD19, Cd10 and CD34. The comparison revealed that MRD had been incorrectly identified by the simplified method in 8.0, 17.6, and 75.8% of the patients on therapy days 15, 36, and 85, respectively. In addition, the content of residual tumor cells with respect to the threshold values more frequently proposed to stratify patients was found to be incorrectly calculated in some true positive samples. Thus, when the simplified approach was applied using the results of MRD detection to stratify the patients into risk groups, 16.0, 27.4, and 81.8% of the samples would yield incorrect information on therapy days 15, 36, and 85, respectively. Thus, the simplified approach to identifying MRD is most applicable on day 15 of therapy; however, there may be mistakes in this point of observation. This method used on day 36 more frequently yields incorrect results and is inapplicable on day 85."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 351]], "word_ranges": [[0, 50]], "text": "This is a clear clinical case of systemic sclerosis (Raynaud's, esophageal involvement, skin induration) presenting with renal crisis. Apart from anti-proteinase 3 antibodies, which are not related to scleroderma (incorrect answer 3), the most likely ones would be antiRNA polymerase III antibodies, which appear in younger patients with renal crises."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[146, 234]], "word_ranges": [[20, 32]], "text": "anti-proteinase 3 antibodies, which are not related to scleroderma (incorrect answer 3),"}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "This is a clear clinical case of systemic sclerosis (Raynaud's, esophageal involvement, skin induration) presenting with renal crisis. Apart from anti-proteinase 3 antibodies, which are not related to scleroderma (incorrect answer 3), the most likely ones would be antiRNA polymerase III antibodies, which appear in younger patients with renal crises.", "full_answer_no_ref": "This is a clear clinical case of systemic sclerosis (Raynaud's, esophageal involvement, skin induration) presenting with renal crisis. Apart from anti-proteinase 3 antibodies, which are not related to scleroderma ([HIDDEN]), the most likely ones would be antiRNA polymerase III antibodies, which appear in younger patients with renal crises.", "full_question": "A 45-year-old female patient with a history of gastroesophageal reflux has been presenting for the past year with episodes of pallor in some fingers with exposure to cold. She had recently been prescribed prednisone at a dose of 20 mg/day for joint pain and skin induration in the hands and arms. For the last 48 hours, she presented with general malaise and intense headache, for which she went to the emergency department. Examination revealed only a rhythmic tachycardia at 100 bpm, with no neurological focality. Blood pressure was 200/110 mmHg. The blood test shows only a creatinine level of 2.5 mg/dL. Indicate which of the following autoantibodies is best related to the process described:", "id": 534, "lang": "en", "options": {"1": "Anti-RNA polymerase III antibodies.", "2": "Anti-centromere antibodies.", "3": "Anti-proteinase 3 antibodies.", "4": "Anti-PM-Scl antibodies.", "5": NaN}, "question_id_specific": 119, "type": "RHEUMATOLOGY", "year": 2021, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1102_22675", "title": "Case 294: Catastrophic Antiphospholipid Syndrome.", "score": 0.017940466613032984, "content": "History A 50-year-old woman presented to the emergency department of our hospital with a 2-day history of lower limb pain associated with unusual asthenia and diffuse arthralgia over the past 3 weeks. She was a native of Guinea and had lived in France for most of her life, working as a personal care assistant. Her only medical history of note was an occurrence of fetal death at 12 weeks gestation when she was 35 years old. She had bilateral lower limb swelling, without changes in skin temperature or color. All proximal and distal arterial pulses were felt. General physical examination findings were otherwise unremarkable. Her laboratory tests showed a decreased hemoglobin concentration of 8.9 g/dL (normal range, 12-16 g/dL), a decreased platelet count of 45 × 10<sup9</sup/L (normal range, [150-400] × 10<sup9</sup/L), a C-reactive protein level of 158 mg/L (normal range, &lt;5 mg/L), and a d-dimer level of 2000 mg/L (normal range, &lt;500 mg/L]). Compression US of the lower limbs revealed bilateral calf vein thrombosis involving the fibular and posterior tibial veins. Curative anticoagulation using low-molecular-weight heparin (enoxaparin, subcutaneous injection of 100 units per kilogram of body weight twice a day) was started. The day after the start of anticoagulation therapy, the patient reported dyspnea and acute chest and abdominal pain. Her vital signs were assessed, and she had elevated blood pressure and increased heart rate and respiratory rate, but she remained afebrile. Her cardiac auscultation was unremarkable, besides tachycardia. Skin examination revealed small areas of necrosis on the fingertips of her right hand. Laboratory studies were repeated and showed an increase in serum creatinine level from a baseline value of 0.49 mg/dL to a new value of 1.01 mg/dL (normal range, 0.6-1.1 mg/dL), an apparition of low-grade proteinuria of 0.43 g per day (normal range, &lt;0.3 g/ day), and a high serum troponin level of 1066 ng/L (normal range, &lt;14 ng/L), whereas electrocardiography showed no ST segment modification and echocardiography revealed a moderately altered left ventricular ejection fraction (45%). There was no coronary occlusion seen at emergency coronarography. Contrast-enhanced CT of the chest, abdomen, and pelvis was performed (Figs 1, 2) together with cardiac MRI (Figs 3, 4)."}, {"id": "pubmed23n1132_13584", "title": "Systemic Sclerosis (SSc) After COVID-19: A Case Report.", "score": 0.017679900744416874, "content": "Since the start of the global pandemic caused by coronavirus disease 2019 (COVID-19), there have been numerous reports of autoimmune and rheumatological disorders developing after infection with SARS-CoV-2. To date, there has been only one reported case of systemic sclerosis (SSc) developing after SARS-CoV-2 infection. Here, we present another case of SSc developing after infection with SARS-CoV-2. A 48-year-old female with past medical history of anxiety and depression presented to the rheumatology clinic after being referred for further evaluation of abnormal labs, Raynaud's phenomenon, and other concerning symptoms. Shortly after hospitalization for COVID-19 pneumonia, she began experiencing symptoms that included fatigue, xerostomia, dysphagia, bilateral lower extremity weakness, dyspnea with exertion, unintentional weight loss, and diffuse skin hyperpigmentation. Labs ordered shortly before presentation were significant for antinuclear antibody (ANA) titer &gt; 1:1280. Physical exam was remarkable for puffy fingers, sclerodactyly of the fingers, diffuse skin hyperpigmentation, and abnormal nailfold capillaries. Anti-RNA polymerase III, anti-Scl-70, anti-centromere, anti-SSA, anti-SSB, anti-Smith, and anti-Smith/RNP antibodies were all negative. BNP, aldolase, and serum myoglobin levels were within normal limits while creatine phosphokinase level was slightly decreased. Pulmonary function testing showed reduced diffusion capacity with normal lung mechanics and volumes. High-resolution CT scan of the chest showed interstitial lung disease, with findings suggestive of nonspecific interstitial pneumonia. Transthoracic echocardiogram showed mild elevation of right ventricular systolic pressure, but pulmonary hypertension was not found on right heart catheterization. Esophagogastroduodenoscopy (EGD) with biopsy performed for evaluation of esophageal dysphagia showed sliding hiatal hernia, irregular Z-line, and gastric hyperemia. Biopsy of the distal esophagus was consistent with Barrett's esophagus. The patient was diagnosed with SSc according to the 2013 American College of Rheumatology/European League Against Rheumatism (ACR-EULAR) classification criteria for SSc. She is currently being treated with mycophenolate mofetil, amlodipine, methotrexate, and prednisone."}, {"id": "pubmed23n1085_24991", "title": "Case 294.", "score": 0.017193034238488784, "content": "History A 50-year-old woman presented to the emergency department of our hospital with a 2-day history of lower limb pain associated with unusual asthenia and diffuse arthralgia over the past 3 weeks. She was a native of Guinea and had lived in France for most of her life, working as a personal care assistant. Her only medical history of note was an occurrence of fetal death at 12 weeks gestation when she was 35 years old. She had bilateral lower limb swelling, without changes in skin temperature or color. All proximal and distal arterial pulses were felt. General physical examination findings were otherwise unremarkable. Her laboratory tests showed a decreased hemoglobin concentration of 8.9 g/dL (normal range, 12-16 g/dL), a decreased platelet count of 45 × 10<sup9</sup/L (normal range, 150-400 × 10<sup9</sup/L), a C-reactive protein level of 158 mg/L (normal range, &lt;5 mg/L) and a d-dimer level of 2000 mg/L (normal range, &lt;500 mg/L). Compression US of the lower limbs revealed bilateral calf vein thrombosis involving the fibular and posterior tibial veins. Curative anticoagulation using low-molecular-weight heparin (enoxaparin, subcutaneous injection of 100 units per kilogram of body weight twice a day) was started. The day after the start of anticoagulation therapy, the patient reported dyspnea and acute chest and abdominal pain. Her vital signs were assessed, and she had elevated blood pressure and increased heart rate and respiratory rate, but she remained afebrile. Her cardiac auscultation was unremarkable, besides tachycardia. Skin examination revealed small areas of necrosis on the fingertips of her right hand. Laboratory studies were repeated and showed an increase in serum creatinine level from a baseline value of 0.49 mg/dL to a new value of 1.01 mg/dL (normal range, 0.6-1.1 mg/dL), an apparition of low-grade proteinuria of 0.43 g per day (normal range, &lt;0.3 g/day), and a high serum troponin level of 1066 ng/L (normal range, &lt;14 ng/L), whereas electrocardiography showed no ST segment modification and echocardiography revealed a moderately altered left ventricular ejection fraction (45%). There was no coronary occlusion seen at emergency coronarography. Contrast-enhanced CT of the chest, abdomen, and pelvis was performed (Figs 1, 2) together with cardiac MRI (Figs 3, 4)."}, {"id": "pubmed23n0073_4514", "title": "[A case of mixed connective tissue disease complicated with malignant hypertension].", "score": 0.016298946531504672, "content": "This case was a 51-year-old woman, who had been diagnosed as having rheumatoid arthritis at some clinic and had been treated with both non-steroidal anti-inflammatory drugs and steroid 3 years before visiting our clinic. When she noticed a decrease in visual acuity and general fatigue in June 1985, she was referred to an ophthalmologist of our hospital, and found to have blood pressure of 240/150 mmHg and KW grade IV retinal findings. She was admitted in our department to examine and treat malignant hypertension. On admission, remarkable hypergammaglobulinemia (29.3%), arthralgia, arthral deformity and pericardial effusion were present thus, she was suspected to be suffering from malignant rheumatoid arthritis. Anti-nuclear antibody (64X), anti-nuclear ribonucleoprotein antibody (64X) and anti-RNase sensitive antibody of anti-extractable nuclear antigens (ENA) antibody (81920X) were positive, while anti-RNase resistant antibody of anti-ENA antibody was negative. Immunologically, her condition was consistent with mixed connective tissue disease (MCTD). Since urinary protein was positive and creatinine clearance was 46.0 ml/min, renal function was thought to be diminished. Her chest roentgenogram revealed cardiomegaly (CTR 67.5%) and an increase in pulmonary vascular shadow. An echocardiogram demonstrated the presence of pericardial effusion. Plasma renin activity was 3.3 ng/ml/h and it was suspected that an intrarenal ischemic change resulted in increased renin release from the juxta-glomerular apparatus, leading to the marked hypertension. Treatment was started with prednisolone 60 mg/day during 4 weeks.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n1059_23586", "title": "A 45-Year-Old Man with Scleroderma Renal Crisis Associated with a History of Systemic Sclerosis Sine Scleroderma.", "score": 0.015696649029982364, "content": "BACKGROUND The diagnosis of systemic sclerosis sine scleroderma (ssSSc) with renal crisis is difficult because of its unusual presentation and rarity. CASE REPORT A 45-year-old man presented to the Emergency Department with worsening nausea, vomiting, and exertional dyspnea for 3 weeks. Initial examination showed blood pressure 182/108 mmHg without skin thickening or other skin manifestations. Laboratory investigations showed serum creatinine level 21.73 mg/dL and diffuse airspace opacities on chest radiography. He was admitted to the intensive care unit and started on emergent hemodialysis. He was anemic and became gradually hypoxic, requiring supplemental oxygen. Computed tomography of the chest showed bilateral infiltrates. Antinuclear antibodies (ANA) were positive for centromere pattern with titer of 320. Antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibodies were negative. He was started on therapeutic plasmapheresis (TP) and captopril, which resulted in significant improvement of respiratory symptoms. The kidney biopsy revealed thrombotic microangiopathy. Anticentromere, anti-Scl-70, and antiribonucleic acid polymerase III antibodies, drawn after 4 sessions of TP, were not detected. CONCLUSIONS Here we report a rare case of ssSSc with renal crisis in a patient who presented with acute renal failure requiring hemodialysis and suspected pulmonary hemorrhage. Clinical improvement was achieved by TP and angiotensin-converting enzyme inhibitor. The diagnosis of ssSSc was difficult and required an ANA pattern and kidney biopsy."}, {"id": "pubmed23n0053_15137", "title": "[Spontaneous remission of dermatomyositis which developed one month after normal delivery].", "score": 0.0150650789255054, "content": "A case of dermatomyositis which developed one month after normal delivery and subsided spontaneously was reported. A 29-year-old woman gave birth to a healthy child. One month later, she noticed muscular pain and weakness of the upper extremities. On admission, there were diffuse edema of upper eyelids with heliotrope rash. The reddish skin rashes were observed on the extensor surfaces of the PIP and MP joints of fingers. Erythrocyte sedimentation rate was 29 mm/hr. The lactic dehydrogenase (LDH), SGOT, CK levels were 470 (normal 150 to 320 IU/l), 43 (normal 6 to 25 IU/l) and 317 (normal 21 to 110 IU/l) respectively. Autoantibodies to nuclear and cytoplasmic antigens were negative. Rheumatoid factor and anti-DNA antibody were negative. Thyroid function was normal. An electromyogram (EMG) demonstrated small amplitude short-duration polyphasic motor unit potentials. The muscle biopsy specimen from left upper arm showed degenerating muscle fibers and infiltration of inflammatory cells surrounding blood vessels. The skin biopsy revealed the presence of edema and perivascular infiltration of lymphocytes. Based on these clinical features and results of various diagnostic tests, a diagnosis of dermatomyositis was established. After the admission, muscle strength has improved dramatically and the CK returned to normal level without specific drug therapy. She has since been seen as an out patient, and complete remission lasted for two years up to date. Review of the literature disclosed that 13 cases of PM/DM which developed during pregnancy or postpartum have been reported including the present case. Detailed analysis showed that these patients were characterized by mild muscular diseases, rare occurrence of internal organ involvements and good response to steroid therapy. As our case, a spontaneous remission was also observed. Although the mechanism involved in occurrence of inflammatory myositis associated with pregnancy or delivery are not clarified, these patient indicated a presence of subset of PM/DM which do not require intensive drug therapy."}, {"id": "pubmed23n1014_25815", "title": "[Adrenal hemorrhage in a patient with systemic lupus erythematosus].", "score": 0.014492753623188406, "content": "A 58-year-old female was referred to our department with intermittent suffocation for 1.5 years, aggravated for a month. 1.5 years before she developed oral ulcer, raynaud phenomenon, proteinuria, bilateral pleural effusion, ANA and anti-dsDNA positive. This patient was diagnosed with systemic lupus erythematosus (SLE). After given hormones, hydroxychloroquine sulfate (HCQ), her symptom relieved soon. The patient stopped her pills 1 year ago. One month ago, she had chest tightness, increased urine foam, and suffered from oliguria. Her admission medical examination: blood pressure (BP) 130/80 mmHg, conjunctiva pale, and lower lung breath sounds reduced. There was no tenderness, rebound pain and abdominal muscle tension in the abdomen. Liver and spleen rib inferior, mobile dullness negative, and lower extremity edema. Blood routine tests were performed with hemoglobin (HGB) 57 g/L. Urine routine: BLD (3+). 24-hour urinary protein 3.2 g. serum albumin 20.5 g/L, C-reactive protein (CRP) 12.85 mg/L, erythrocyte sedimentation rate (ESR) 140 mm/h. Antinuclear antibody (ANA) (H)1:10 000, anti-dsDNA antibody 1:3 200; anti-Smith antibody, anti-U1-snRNP/Sm antibody were positive, blood complement 3(C3) 0.43 g/L, complement 4(C4) 0.07 g /L. Anticardiolipin antibody (ACL), anti-β2-GP1, lupus anticoagulant (LA) were negative; HRCT suggested bilateral medial pleural cavity product liquid. Admission diagnosis: SLE lupus nephritis, anemia, pleural effusion, and hypoproteinemia. We treated her with methylprednisolone 1 000 mg×3 d, late to 48 mg/d and cyclophosphamide 1.0 g, HCQ 0.2 g bid, gamma globulin 10 g×5 d. Day 2 of treatment, this patient developed acute right upper quadrant pain, not accompanied by nausea, vomiting, blood stool and diarrhea. Antipyretic antispasmodic treatment was invalid, after the morning to ease their own abdominal pain. Day 4 of treatment, daytime blood HGB 77 g/L. Bilateral renal vascular ultrasound: bilateral renal artery blood flow velocity was reduced. The abdominal pain of the above symptoms recurred at night, BP was 120/80 mmHg, and no positive signs were found on abdominal examination. No abnormality was found in the vertical abdominal plain film. Blood routine examination: HGB 53 g/L, Plasma D dimer 2 515 μg/L, amylase in hematuria was normal, the stool occult blood was negative. Abdominal computed tomography (CT): normal structure of right adrenal gland disappeared, irregular mass shadow could be seen in adrenal region, CT value was about 50 HU. Morphological density of left adrenal gland was not abnormal. The retroperitoneum descended along the inferior vena cava to the right iliac blood vessel and showed a bolus shadow. The density of some segments increased. The lesion involved the right renal periphery and reached the left side of abdominal aorta. Most lesions surrounded the inferior vena cava, the right renal vein and part of the small intestine. The boundary between the upper lesion and the vena cava was unclear. Iodinecontaining contrast agent was taken orally. No sign of contrast agent overflowing was found in the abdominal cavity. Hematoma and exudative changes were considered in retroperitoneum. CONCLUSION of contrast-enhanced ultrasound of blood vessels: The retroperitoneal inferior vena cava (volume 3.5 cm×3.5 cm×1.5 cm) was hypoechoic and had no blood flow lesion. The adrenal gland had a high possibility of origin. Left renal vein thrombosis extended to inferior vena cava. According to the above data, it was analyzed that the cause of retroperitoneal hematoma of the patient was left adrenal vein thrombosis caused by hypercoagulable state, which led to vascular rupture and hemorrhage caused by increased vascular pressure in adrenal gland. Therefore, on the basis of continuing to actively treat the primary disease, and on the basis of dynamic observation of no active hemorrhage for 3 days, the anticoagulant therapy was continued with 10 mg/d of apixaban. Clinical symptoms were gradually eased, HGB did not decrease. Two weeks later, the ultrasonic examination showed that the irregular cluster hypoechoic range behind the inferior vena cava was significantly smaller than that before (1.8 cm×1.2 cm×0.7 cm). Abdominal CT examination after 1 month showed that there was no abnormal morphological density of bilateral adrenal glands and basic absorption of retroperitoneal exudation. Adrenal hemorrhage is uncommon. SLE with adrenal hemorrhage is rarer. In SLE patients, especially those complicated with APS, if abdominal pain accompanied by HGB decrease occurs, except after gastrointestinal hemorrhage, the possibility of adrenal hemorrhage should be warned."}, {"id": "pubmed23n0320_15745", "title": "[A case of scleroderma renal crisis with massive pericardial effusion and positivity on antiphospholipid antibody test].", "score": 0.014266435319066899, "content": "A 47-year-old woman was admitted to our hospital for evaluation of general fatigue and dyspnea. She had been diagnosed with progressive systemic sclerosis (PSS) when she was 39 years of age, on the basis of Raynaud's phenomenon, proximal sclerosis, and pigmentation of the skin. On admission, her blood pressure was 206/128 mmHg. Funduscopy revealed grade III (Keith &amp; Wagener) hypertensive retinopathy. Laboratory data showed positivity for anti-nuclear antibody and anticardiolipin beta 2 glycoprotein I antibody, and the plasma level of renin activity (PRA) was abnormally high. Chest X-ray and UCG revealed massive pericardial effusion. On the second hospital day, she was operated on for pericardiodiaphragmatic fenestration. The volume of pericardial effusion amounted to more than 2000 ml. Post operative malignant hypertension persisted. Laboratory data showed thrombocytopenia, hemolytic anemia, and acute renal failure. We diagnosed scleroderma renal crisis (SRC) associated with antiphospholipid syndrome. Following the initiation of angiotensin converting enzyme inhibitor (ACE-I) combined with calcium antagonist and alpha-one blocker, her blood pressure and PRA decreased. She also had been treated with aspirin 81 mg daily. These therapies were effective in recovering the platelet count and stopped the progression of anemia and renal failure. Although either the finding of large pericardial effusion or SRC is associated with poor prognosis in PSS, this case has had a good clinical course. In this case, the findings suggested that anti-phospholipid antibody may have contributed to the pericarditis and SRC."}, {"id": "pubmed23n0301_22172", "title": "[The first manifestations of Schönlein-Henoch purpura in a 74-year-old female patient with hyperthyroidism].", "score": 0.014141613924050632, "content": "A 74-year-old woman had for 3 weeks suffered from watery diarrhoea and diffuse abdominal pain. She felt restless, had a subfebrile temperature (37.8 degrees C), tachycardia and a blood pressure of 190/90 mmHg. Shortly after admission petechiae were found over the lower legs and she complained of joint pains. Laboratory tests established hyperthyroidism. Skin biopsy showed leukocytoclastic vasculitis. Tests for antinuclear antibodies, antistreptolysin titre, rheumatoid factors, cryoglobulins and TSH-receptor antibodies were negative, immunoglobulin A (IgA) was raised. As a drug-induced vasculitis was suspected treatment was started with methylprednisolone, 100 mg daily. Proteinuria (5.6 g daily) indicated renal biopsy, which revealed focal glomerulonephritis with deposits of IgA, fibrin/fibrinogen and complement factor 3. Gastroscopic biopsy, performed after an episode of gastrointestinal bleeding, demonstrated necrotizing vasculitis, confirming the diagnosis of Schönlein-Henoch-purpura (SHP). As the patient's condition rapidly worsened, cyclophosphamide was started additionally (2 mg/kg). She died on the 17th hospital day from acute cardiac failure. The lethal course of the disease in this elderly patient illustrates a previously not reported close temporal and clinical relationship between SHP and hyperthyroidism."}, {"id": "wiki20220301en035_34634", "title": "Polyarteritis nodosa", "score": 0.013959000921820817, "content": "A patient is said to have polyarteritis nodosa if he or she has three of the 10 signs known as the 1990 American College of Rheumatology (ACR) criteria, when a radiographic or pathological diagnosis of vasculitis is made: Weight loss greater than/equal to 4.5 kg Livedo reticularis (a mottled purplish skin discoloration over the extremities or torso) Testicular pain or tenderness (occasionally, a site biopsied for diagnosis) Muscle pain, weakness, or leg tenderness Nerve disease (either single or multiple) Diastolic blood pressure greater than 90 mmHg (high blood pressure) Elevated kidney blood tests (BUN greater than 40 mg/dL or creatinine greater than 1.5 mg/dL) Hepatitis B (not C) virus tests positive (for surface antigen or antibody) Arteriogram (angiogram) showing the arteries that are dilated (aneurysms) or constricted by the blood vessel inflammation"}, {"id": "wiki20220301en022_110925", "title": "Raynaud syndrome", "score": 0.013954089323398939, "content": "A careful medical history will seek to identify or exclude possible secondary causes. Digital artery pressures are measured in the arteries of the fingers before and after the hands have been cooled. A decrease of at least 15 mmHg is diagnostic (positive). Doppler ultrasound to assess blood flow Full blood count may reveal a normocytic anaemia suggesting the anaemia of chronic disease or kidney failure. Blood test for urea and electrolytes may reveal kidney impairment. Thyroid function tests may reveal hypothyroidism. Tests for rheumatoid factor, erythrocyte sedimentation rate, C-reactive protein, and autoantibody screening may reveal specific causative illnesses or an inflammatory process. Anti-centromere antibodies are common in limited systemic sclerosis (CREST syndrome). Nail fold vasculature (capillaroscopy) can be examined under a microscope."}, {"id": "pubmed23n0303_14725", "title": "[A case report of typical scleroderma accompanied with serum abnormalities characteristic of SLE during the course].", "score": 0.01384895533831704, "content": "A 40-year-old woman had complained of cyanosis induced by cold exposure from the age of 26. When she was 32 years old, Raynaud's phenomenon occurred. She developed diffuse cutaneous sclerosis affecting the upper limbs, face and trunk, digital pitting scar, flexion contractures of hands, dilatation of lower esophagus and pulmonary fibrosis, and she was diagnosed as scleroderma. Laboratory findings revealed positive anti-topoisomerase I antibody and hypergammaglobulinemia (IgG 2,782, IgA 632, IgM 146 mg/dl). However, serum complement levels were normal and anti-DNA antibodies measured by radioimmunoassay (RIA) were negative. Initial dose of oral prednisolone was 30 mg/day and afterwards 5 mg/day of prednisolone was maintained. At the age of 36, scleroderma and contraction of hands were progressed, and telangiectasias appeared on her chest at the age of 36. Laboratory tests revealed hypocomplementemia (C3 27, C4 9 mg/dl, CH50 16 U/ml) and high titers, more than 100 U/ml, of anti-DNA antibodies measured by RIA. Clinical evidence suggestive of SLE could not be found. Reexamination of previous sera by enzyme immunoassay, in which anti-DNA antibody could not be detected by RIA, clarified the presence of IgG anti-dsDNA antibodies. It was considered that there existed low avidity/affinity of anti-dsDNA antibodies at first, and afterwards high avidity/affinity of anti-dsDNA antibodies appeared. Increasing of oral prednisolone up to 30 mg/day normalized serum complements and decreased titers of anti-DNA antibodies. She had not developed any clinical evidence that suspected SLE throughout the course."}, {"id": "pubmed23n0060_13580", "title": "[A case of mixed connective tissue disease (MCTD) associated with transverse myelitis responding to pulse therapy].", "score": 0.013770913770913771, "content": "A 42-year-old female was admitted to our hospital on October 1, 1990 because of one week history of back pain, weakness of her right lower extremity and sensory disturbance of her left lower extremity. Physical examination revealed swollen hands, Raynaud's phenomenon, sclerodactyly and heliotrope rash. The body temperature was 37.0 degrees C. Neurological findings included weakness in the right lower extremity, left hypalgesia and thermohypesthesia below Th4, hyperreflexia on the right lower extremity and right extensive plantar response. Laboratory data showed leucopenia (3,700/mm3) and hypergammaglobulinemia. Serological examination revealed antinuclear antibodies with a titer of 1:5120 (speckled pattern) and anti-RNP antibody with a titer of 1:32. Neither anti-DNA antibody nor anti-Sm antibody were detected. Serum C3 and C4 were normal. The cerebrospinal fluid (CSF) contained mononuclear cells of 5/mm3, protein 29 mg/dl and glucose 56 mg/dl. Queckenstedt test was negative. Treatment with prednisolone 60 mg daily was started. On the 8th day of therapy, she complained of a burning sensation in the back, then paraplegia and urinary retention developed. MRI examination showed a high intensity area of the spinal cord at the right Th4 on T2-weighted images. Next day the treatment with 1000-mg intravenous daily pulse of methylprednisolone for 3 days was started, followed by prednisolone 40 mg daily. After this pulse therapy, the CSF contained mononuclear cells of 52/mm3, protein 34 mg/dl, glucose 67 mg/dl and IgG 7.6 mg/dl. Her neurological manifestation gradually improved and at six weeks after the pulse therapy neurological examination revealed no abnormality except for painful tonic spasm. Prednisolone was slowly tapered to 15 mg daily.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n1004_25990", "title": "Rowell Syndrome in Nigeria: Systemic Lupus Erythematosus Presenting as Recurrent Erythema Multiforme in a Young Woman.", "score": 0.012597809076682316, "content": "Dear Editor, Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by diverse patterns of auto-antibody production with multi-organ affectation. Cutaneous involvement, either alone or in association with other systemic illnesses, is one of its most common manifestations (1). Dermatologic disorders like malar and discoid rashes are quite suggestive of SLE. However, the occurrence of non-specific skin lesions like erythema multiforme (EM) in patients with SLE (Rowell syndrome) can rarely occur (1). In such patients, a diagnosis of SLE may be missed or delayed in the absence of other overt clinical features of lupus. Herein we report a case of recurring EM-like eruptions as the cardinal cutaneous manifestation of previously undiagnosed, active SLE in a young Nigerian woman. A 26-year-old Nigerian woman presented with a three-day history of non-pruritic, generalized, and target-like, erythematous annular patches and plaques which mostly affected the trunk. A few lesions had presented with crusting and erosions at the time of examination (Figure 1). Associated symptoms included oral painful ulcers, low grade fever, and malaise. The patient had no other systemic symptoms and her prior drug history was not remarkable. Her erythrocyte sedimentation rate (ESR) was 66 mm/hour using the Westergren method. Screening for HIV and hepatitis B and C was negative. Herpes simplex, cytomegalovirus, and Epstein Barr viruses could not be screened for. Other baseline investigations (complete blood count, electrolytes, urea and creatinine as well as urinalysis) were within normal limits. The patient was managed as a case of EM of an unidentified inciting agent and her symptoms resolved with supportive care and antibiotics. However, she developed a recurrence about 5 weeks later, with more extensive and coalescent skin lesions (Figure 2). Additionally, there was a new onset of alopecia and pain in the small joints of the hands as well as both knees and ankles. At this time, the patient's ESR had gone up to 112 mm/h and she had developed significant proteinuria, with a protein creatinine ratio of 1.3 g/g (reference &lt;0.5 g/g). Her antinuclear antibody (ANA) titer was high (1:320) with a speckled pattern. Anti-Smith antibody was also positive. A renal biopsy was declined. A tentative diagnosis of Rowell syndrome was made. The patient was started on high-dose steroids and hydroxychloroquine 200 mg twice daily. Subsequent care included the use of mycophenolate mofetil 1 g twice daily for 6 months. This was then changed to azathioprine at 50 mg twice daily. Follow-up after 6 months showed sustained clearance of skin lesions, resolution of fever and joint pains, as well as improvement in the renal profile, with a urine protein-creatinine ratio of 0.77 g/g. The presence of systemic lupus erythematosus, EM-like lesions, and a speckled pattern of antinuclear antibody in our patient fulfils the revised diagnostic criteria for RS put forward by Zeitouni et al. at the turn of the twenty-first century (2). Considering the rarity of EM-like lesions in SLE and the possibility of constitutional symptoms in EM, a diagnosis of RS may be readily overlooked in patients like the one described, whose major cutaneous manifestation of severe active SLE was EM-like lesions. In contrast to classic EM, where skin lesions are concentrated in the extremities, a predominant truncal distribution of EM-like lesions as found in our patient may favor a clinical consideration of RS (3). However, some authors have challenged the existence of Rowell syndrome as a distinct clinical laboratory entity. Arguments put forward in this regard include the fact that none of the immunological markers that have been described in RS are specific to any disorder. Additionally, the annular polycyclic dermatosis seen in sub-acute cutaneous lupus erythematosus (SCLE) can be difficult to clinically and histologically differentiate from EM (4,5). Patients with SLE also have a higher likelihood of developing adverse drug reactions (6). The inherent complexity of SLE may make for delayed and oftentimes difficult diagnosis, especially in a country where immunologic tests are expensive and rheumatologists are scarce. When patients do occasionally present with recurrences of skin lesions in the spectrum of EM, Steven-Johnson syndrome, and toxic epidermal necrolysis in the absence of a definite inciting agent, undiagnosed lupus may indeed be present in some of these individuals and should be considered in the differential diagnosis. In conclusion, while it is very rare, SLE may present first with recurrent episodes of EM-like rash. Despite the various possibilities which underlie their association, prompt identification and treatment of SLE in patients presenting with EM is important to prevent death or irreversible organ damage."}, {"id": "pubmed23n0913_15886", "title": "Crescentic glomerular nephritis associated with rheumatoid arthritis: a case report.", "score": 0.009900990099009901, "content": "Rheumatoid arthritis is a systemic disorder where clinically significant renal involvement is relatively common. However, crescentic glomerular nephritis is a rarely described entity among the rheumatoid nephropathies. We report a case of a patient with rheumatoid arthritis presenting with antineutrophil cytoplasmic antibody-negative crescentic glomerular nephritis. A 54-year-old Sri Lankan woman who had recently been diagnosed with rheumatoid arthritis was being treated with methotrexate 10 mg weekly and infrequent nonsteroidal anti-inflammatory drugs. She presented to our hospital with worsening generalized body swelling and oliguria of 1 month's duration. Her physical examination revealed that she had bilateral pitting leg edema and periorbital edema. She was not pale or icteric. She had evidence of mild synovitis of the small joints of the hand bilaterally with no deformities. No evidence of systemic vasculitis was seen. Her blood pressure was 170/100 mmHg, and her jugular venous pressure was elevated to 7 cm with an undisplaced cardiac apex. Her urine full report revealed 2+ proteinuria with active sediment (dysmorphic red blood cells [17%] and granular casts). Her 24-hour urinary protein excretion was 2 g. Her serum creatinine level was 388 μmol/L. Abdominal ultrasound revealed normal-sized kidneys with acute parenchymal changes and mild ascites. Her renal biopsy showed renal parenchyma containing 20 glomeruli showing diffuse proliferative glomerular nephritis, with 14 of 20 glomeruli showing cellular crescents, and the result of Congo red staining was negative. Her rheumatoid factor was positive with a high titer (120 IU/ml), but results for antinuclear antibody, double-stranded deoxyribonucleic acid, and antineutrophil cytoplasmic antibody (perinuclear and cytoplasmic) were negative. Antistreptolysin O titer &lt;200 U/ml and cryoglobulins were not detected. The results of her hepatitis serology, retroviral screening, and malignancy screening were negative. Her erythrocyte sedimentation rate was 110 mm in the first hour, and her C-reactive protein level was 45 mg/dl. Her liver profile showed hypoalbuminemia of 28 g/dl. She was treated with immunomodulators and had a good recovery of her renal function. This case illustrates a rare presentation of antineutrophil cytoplasmic antibody-negative crescentic glomerular nephritis in a patient with rheumatoid arthritis, awareness of which would facilitate early appropriate investigations and treatment."}, {"id": "pubmed23n0064_8788", "title": "[A case of systemic lupus erythematosus complicated with pulmonary hypertension and massive pericardial effusion].", "score": 0.009900990099009901, "content": "A 22 year-old-female had suffered from polyarthralgia and Raynaud's phenomenon since 1984. In 1986, she was diagnosed as systemic lupus erythematosus (SLE). In April 1988, she was admitted to Kawasaki Municipal Hospital because of fever and dyspnea on exertion (DOE). Physical examination showed high fever, butterfly rash, oral ulcer and elevation of heart sound IIp on auscultation. Laboratory findings revealed that erythrocyte sedimentation rate was elevated to 105 mm/hr. The following values were observed, anti DNA antibody 391 IU/ml, serum IgA 5mg/dl, anti IgA antibody weakly positive. Chest X ray showed CTR 65%. Echo cardiogram showed massive pericardial effusion. 201T1 myocardial SPECT revealed right ventricular pressure over loading. PSL 40 mg/day was started to administer for the massive pericardial effusion due to SLE activities. On 6th of June, right heart catheterization confirmed the pulmonary hypertension (PPA 22 mmHg, Pulmonary artery resistance (PAR) 1163 dyne/sec/cm-5/mm2). By the treatment with PSL, massive pericardial effusion was gradually improved but DOE clinically unchanged. Second right heart catheterization was done on 8th of August. PAR was improved to 895 dyne/sec/cm-5/mm2 but PPA was elevated to 26 mmHg. It is very interesting that PPA was elevated although PAR was improved by PSL therapy. It is considered that the increase in venous return which was caused by improvement of massive pericardial effusion induced conversely the elevation of PPA. Additionally she was complicated with IgA deficiency. It may occur not only by the immunogenetical disorder such as HLA or IgG subclass alteration but also by anti IgA antibody or lymphocytes dysfunction complicated with SLE."}, {"id": "pubmed23n0866_23648", "title": "Paraneoplastic Scleroderma: Are There Any Clues?", "score": 0.00980392156862745, "content": "Dear Editor, Scleroderma associated with neoplasia is rare, with only a small number of cases reported. We describe 4 patients with paraneoplastic scleroderma who were treated at the I. Department of Dermatovenereology, St. Anna Hospital, during the period between 2004 and 2014. The patients were diagnosed with cholangiogenic carcinoma, endometrial carcinoma, prostatic adenocarcinoma, and adenoma of the suprarenal gland. In the case of concurrent scleroderma and tumor, four situations may occur: they can develop independently of each other; scleroderma may be induced by the tumor; the tumor can develop in the scleroderma; or the tumor can be induced by immunosuppressive therapy. Sclerotization of the skin was described in association with lung cancer, carcinoid, plasma cell dyscrasia, cancer of the ovary, cervix, breast, esophagus, stomach, nasopharynx, melanoma, and sarcoma (1,2,5,7,10). Symptoms may be induced by substances secreted by the tumor (hormones, cytokines, etc.) (9). Tumorous cells further induce cytotoxic and autoantibody response. Scleroderma is characterized by immunological dysregulation, vasculopathy, and hyperproduction of the extracellular matrix by activated fibroblasts. Endothelial, inflammatory, and mesenchymal cells produce cytokines, chemokines, and growth factors e.g. Interleukin-1 (IL1), Interleukin-6 (IL6), tumor necrosis factor alpha (TNF α), collagen alpha 1, connective tissue growth factor (CTGF) (3), and basic fibroblast growth factor (bFGF). This factor is also produced by lung cancer cells (4). The clinical picture of scleroderma and paraneoplastic scleroderma is similar. Diffuse thickening of the skin and/or sclerodermatous plaques can be seen. The histological picture is consistent with scleroderma. Capillaroscopy changes, antinuclear antibodies (ANA), sclerodactyly, and Raynaud phenomenon suggest the diagnosis of systemic scleroderma (SS) (4). Our patients did not fulfill enough of the criteria for SS. Both diffuse and localized scleroderma was seen in 3 patients and generalized localized scleroderma in one case. All patients had a histological picture consistent with scleroderma, negative ANA and ENA antibodies (Table 1, Figure 1). A 66-year-old woman presented with a 10 months history of sclerodermatous plaques on her neck, trunk, and upper and lower extremities. The skin on her breasts and cheeks was diffusely indurated. Examination showed thrombocytopenia, elevated transaminases, Cancer antigen 19-9 (Ca 19-9), thyroid stimulating hormone (TSH), and anti-thyroid peroxidase antibodies, dysmotility of the lower part of esophagus, hepatosplenomegaly, cholecystolithiasis, and benign polyps of colon. She was given prednisone 40 mg/day but did not return for follow up. After 6 months she was diagnosed with cholangiogenic carcinoma with metastatic disease and died shortly afterwards. A 74-year-old woman had localized scleroderma on the trunk for three years. She was treated with procaine penicillin for positive borrelia Immunoglobulin M (IgM) antibodies. Her condition worsened suddenly with confluent scleroderma plaques on her trunk, extremities, and genital region, and vasoneurosis on her lower extremities; she was started on prednisone 35 mg/day. Examination revealed endometrial cancer. The patient underwent a hysterectomy, adnexectomy, and radiotherapy with curative effect. Scleroderma patches softened with residual hyperpigmentation, and prednisone was stopped two years later. A 80-year-old man had a month-long history of diffuse thickening and toughening of the skin on the forearms and lower legs and scleroderma patches on the thighs and shins. Examination revealed prostate adenocarcinoma, and therapy with antiandrogen bicalutamide and prednisone 15 mg/day was started. Two years after the diagnosis he continues with bicalutamide treatment, prednisone 5 mg q.a.d. and has residual toughening of the skin on his lower legs. A 62-year-old woman with seronegative rheumatoid arthritis presented with diffusely tough skin on her extremities and trunk, present for 2 months. Examination revealed cervicitis with a benign endometric polyp, cholecystolithiasis, borderline pulmonary hypertension, and a hormonally inactive suprarenal adenoma. She was given prednisone 40 mg/day and penicillamine with effect. In the 3rd year of therapy she has residual induration of her lower legs and a scleroderma plaque in the lumbar region. She is monitored for her suprarenal adenoma. Two patients had scleroderma at the same time as a malignant tumor; in one patient the localized scleroderma worsened rapidly at the time of the tumor diagnosis, and in one patient a clinically silent adenoma was found. Adrenal tissue can secrete molecules such as serotonine or bFGF involved in fibroplasia (3,6). One patient died of a metastatic disease, two patients after the successful treatment of the tumor, and the patient with suprarenal adenoma experienced softening of the skin and regression of scleroderma. Although paraneoplastic scleroderma is often classified as a pseudoscleroderma, we regard neoplasia as a distinct triggering impulse for scleroderma. Recently, an association between RNA polymerase I/III antibodies in systemic scleroderma and cancer was suggested (8). Such studies may confirm the true link between scleroderma and malignancy. These patients are characterized by older age, sudden onset, diffuse thickening of the skin, and/or generalized morphea with a concurrent neoplastic process. In the case of a successful tumor treatment, skin changes regress. "}, {"id": "pubmed23n0873_8740", "title": "[The 451(th) case: intermittent rash, fever and headache].", "score": 0.00980392156862745, "content": "A 29-year-old woman was admitted to the Department of Rheumatology, Peking Union Medical College Hospital due to intermittent rashes, fever and headache. Palpable purpura were symmetrically distributed on the extremities and trunk. Other manifestations included headache with nausea and vomiting. Elevated white blood cell (WBC) count, platelet (PLT) count, erythrocyte sedimentation rate (ESR) and C-reactive protein were the main laboratory findings. Antinuclear antibodies and antineutrophil cytoplasmic antibodies were negative. Examination of the cerebrospinal fluid (CSF) revealed high intracranial pressure, while routine cytology and biochemical tests of CSF were normal. Head MRI scan and PET-CT did not detect remarkable findings. A diagnosis of systemic vasculitis was confirmed by the biopsy of skin lesion which showed inflammatory infiltration of the muscular vessel wall. Combination therapy of corticosteroids and cyclophosphamide lead to a rapid improvement in clinical symptoms and laboratory parameters. The patient was in stable remission till 6 month follow-up. "}, {"id": "pubmed23n0540_7733", "title": "Ulcerative paraneoplastic dermatomyositis secondary to metastatic breast cancer.", "score": 0.009708737864077669, "content": "A 40-year-old Chinese-American woman with breast carcinoma metastatic to her lungs presented with a 3-month history of erosions on her inner thighs (Figure 1) and medial left shoulder. Faint livedo reticularis was evident on her legs as well. She had difficulty in walking and raising her shoulders. Her cutaneous examination was also notable for cuticular erythema (Figure 2) and mild periorbital erythema and edema. She had no systemic or rheumatologic complaints other than some difficulty in swallowing. Her blood chemistry values were notable for a creatinine kinase of 564 IU/L (5-200 IU/L), alanine aminotransferase 161 U/L (0-40 U/L) and aspartate aminotransferase 93 U/L (0-40 U/L), and an antinuclear antibody titer of 1:2560. Other blood chemistries and antibody serologies (anti-Jo-1, anti-Mi-2 and other anti-tRNA synthetase, anti-Ro/SSA, anti-U1RNP, anti-PM/Scl, and anti-Ku) were within normal limits. A biopsy specimen was obtained from an area of intact skin close to a right thigh ulceration that showed subtle vacuolar alteration at the dermo-epidermal junction with occasional necrotic keratinocyte (Figure 3). Melanophages and telangiectases were present. Within the subcutis there was fibrin deposition and neutrophils. A diagnosis of dermatomyositis was made. The patient received oral prednisone 20 mg three times a day, and her ulcerations resolved. Her creatinine kinase, alanine aminotransferase, and aspartate aminotransferase values returned to normal over the course of 3 weeks, but her antinuclear antibody was unchanged. Radiographic studies concurrently noted that her breast cancer had recurred in her lungs; plans were made to treat her with chemotherapy. The patient was lost to close follow-up, but it was learned that her erosions had reoccurred while her prednisone was tapered and resolved when her dosage of prednisone was increased."}, {"id": "pubmed23n0352_7387", "title": "[A case of microscopic polyangiitis with severe cardiac and respiratory muscle involvement].", "score": 0.009708737864077669, "content": "A 66-year-old female was admitted to our hospital in January, 1998, complaining of low grade fever and muscle weakness of her legs. Physical examination revealed muscle weakness of her neck (4/5) and proximal skeletal muscles of her bilateral legs (3/5-4/5). She showed proteinuria and microhematuria. Her serum levels of ureanitrogen, creatinine, aspartate aminotransferase, alanine aminotransferase, creatinekinase, aldolase and myoglobin were all within the normal ranges. Antinuclear antibodies were negative, but her serum levels of pANCA (743 EU) and C reactive protein (18.0 mg/dl) were elevated. Neuroconduction velocity of her left common peroneal nerve was decreased to 40.8 m/sec and electric myograph showed neurogenic changes. Magnetic resonance images (MRI) of her bilateral thigh depicted high signal intensity in quadriceps by T 2 weighed images, but the signals were not enhanced by gadolinium injection. Muscle and renal biopsies revealed necrotizing vasculitis of the small arteries. Crescentic glomerulonephritis was also observed by renal biopsy. These findings supported the diagnosis of microscopic PN. On 16 th admission day, she developed acute cardiac and respiratory failures due to cardiac and respiratory muscle involvements with PN, and was assisted by mechanical ventilation. She was treated with methylprednisolone pulse therapy (500 mg/day, three consecutive days) on 18 th admission day, followed by 40 mg of oral prednisolone daily. However, her symptoms deteriorated, and herserum creatinine levels increased to 2.4 mg/dl. On 24 th admission day, intravenous cyclophosphamide pulse therapy (500 mg/day) was instituted. Her cardiac wall motion on echocardiography and serum creatinine levels gradually improved, but her skeletal and respiratory muscle weakness did not improve. On 38 th admission day, she was complicated with respiratory infection by methicillin resistant Staphylococcus aures. On 62 th admission day, she died of endotoxic shock. This is the first report describing respiratory muscle involvement with PN, and the second report describing MRI findings of muscle involvement by PN. Therefore, our case provides important clinical information for the diagnosis and treatment of the disease."}, {"id": "pubmed23n0086_6631", "title": "[A case of malignant rheumatoid arthritis with severe peripheral neuropathy].", "score": 0.009615384615384616, "content": "A 72-year-old woman of definite type of malignant rheumatoid arthritis (MRA) with severe peripheral neuropathy. She has often noted pain of both shoulders or knee joints since some years ago. At the age of 71, she noticed numbness of the feet with pain and swelling of knee joints. She was diagnosed as definite type of rheumatoid arthritis by one podiatrist. Although she took some medications, she subsequently developed general fatigue, appetite loss, exacerbation of arthritis, drop feet and hands with prominent coldness. She was admitted to our hospital on March 22, 1985. On examination, she revealed purpura, decubitis, heart murmur, arthritis of knee joints, and fingers necrosis with skin ulcer. She had severe muscle weakness, and wasting of four limbs. Moderate impairment of all-modality sensations were noted in all extremities. Distal involvement was greater than proximal. Laboratory data during administration of prednisolone (60 mg/day) were as follows: glucose in urine, 2+; occult blood in urine, 1+; white blood cells count, 18600 with 92% polymorphonuclear leukocytes; erythrocyte sedimentation rate, 60 mm in an hour; CRP, 14.62 mg/dl (normal 0.5 greater than); RA test, 2+; RAHA, 10240; CH50, 10 U/ml (normal 32-42); C3, 37 mg/dl (normal 55-75); C4, 9 mg/dl (normal 15-28); immune complex, 4.4 micrograms/ml (normal 3.0): Chest X-ray film showed cardiomegaly (CTR, 57%). ECG disclosed atrial premature contraction, and echo cardiography suggested epicarditis with aortic valve insufficiency. 99mTc RI angiogram revealed impairment of peripheral circulation. SCV on sural nerve was not elicited. Sural nerve biopsy showed obliterans type of endoarteritis and axonal degeneration with loss of myelinated fiber."}, {"id": "pubmed23n0274_11474", "title": "[Two cases of acute progressive interstitial pneumonia associated with dermatomyositis--clinical features and immunological disorders].", "score": 0.009523809523809525, "content": "Some patients of dermatomyositis (DM) with interstitial pneumonia (IP) have common clinical features. Clinical features of these patients are acute onset, very poor prognosis and that patients have fever, arthritis, typical skin rash, mild myositis and show low ratio of CPK/LDH, low incidence of antinuclear antibody (ANA) appearance, low inflammatory signs. We experienced two cases of this category of DM with IP and examined immunological aspects. Case 1. A 52-year-old woman was admitted in June 1, 1990 with a one-month history of arthralgia and a ten-days history of fever, skin rash, myalgia and dyspnea on exertion. On examination she had Gottron's papules on her fingers, erythema on back, bilateral elbows and legs, proximal muscle weakness and arthritis. Fine crackles were audible in the lower lung fields. Laboratory data included CPK 200 IU/l, ALD 3.2 IU/l, LDH 805 IU/l. Analysis of bronchoalveolar lavage fluid (BALF) revealed increased cellularity with lymphocytosis. She was treated with oral corticosteroid (CS), pulse CS, cyclosporin A. Inspite of these therapies, she died of progressive respiratory insufficiency in July 10, 1990. Case 2. A 23-year-old woman was admitted in April 1, 1991, with a two-month history of arthralgia and a one-month history of fever, skin rash, stomatitis, alopecia. On examination she had Gottron's papules on her fingers, erythema on malar, bilateral elbows and legs, arthritis and stomatitis. Laboratory data included CPK 97 IU/l, ALD 8.5 IU/l, LDH 779 IU/l. She began experiencing dry cough and dyspnea on exertion in May 1991. Analysis of BALF revealed increased cellularity with lymphocytosis. She was treated with oral corticosteroid(CS), pulse CS, pulse cyclophosphamide.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en014_21973", "title": "Anti-nuclear antibody", "score": 0.009433962264150943, "content": "Anti-PM-Scl Anti-PM-Scl antibodies are found in up to 50% of polymyositis/systemic sclerosis (PM/SSc) overlap syndrome. Around 80% of individuals with antibodies present in their blood serum will have the disorder. The presence of the antibodies is linked to limited cutaneous involvement of PM/SSc overlap syndrome. The antigenic targets of the antibodies are components of the RNA-processing exosome complex in the nucleolus. There are ten proteins in this complex and antibodies to eight of them are found at varying frequencies; PM/Scl-100 (70–80%), PM/Scl-75 (46–80%), hRrp4 (50%), hRrp42 (21%), hRrp46 (18%), hCs14 (14%), hRrp41 (10%) and hRrp40 (7%)."}, {"id": "pubmed23n0078_16912", "title": "[A case of progressive systemic sclerosis associated with a hemorrhagic infarction of the cerebellum].", "score": 0.009433962264150943, "content": "Central nervous system is rarely involved in progressive systemic sclerosis (PSS) unless there are concomitant abnormalities in renal or lung function or hypertension. A 72-year-old woman with typical PSS developed cerebellar bleeding. Medical history records revealed, she had noted the onset of Raynaud's sign on her upper extremities at the age of 37. This was followed by necrosis and repeated infection, and as a result, shortening of her fingers in her 40's. The disease progressed and involved lower extremities, and then face and body in her 50's. Aortic valve stenosis was diagnosed at 69 year old, cardiac myopathy at 70 and at the age of 71 infectious dermatitis in both inguinal regions. Mild anemia, hypoalbuminemia and the decrease of serum Fe were discovered in June 1988. At the same time, prolonged ESR, positive C-reactive protein, RA, and anti-nuclear-antibody were also noticed. A chest roentgenogram revealed pulmonary fibrosis. Systemic hypertension was not noticed on the clinical course. She developed an onset of vertigo and vomiting in the morning of August 8, 1988. Consequently, she was brought to our hospital. She was alert but a physical examination showed a swallowing disturbance, dysarthria, right cerebellar ataxia, nystagmus and hypertension (192/100 mmHg). A CT examination on admission revealed a slightly low density area in right cerebellar hemisphere without mass effect. She was treated with dextran and mannitol and her condition improved on the 6th day of her admission. She was alert and blood pressure calm down to 120/70 mmHg without the use of anti-hypertension drugs on August 21.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0064_440", "title": "[A case of subacute idiopathic pure pan-dysautonomia--recovery with prednisolone therapy].", "score": 0.009345794392523364, "content": "A 52-year-old man had, after an episode of fever in June 1989, developed orthostatic dizziness followed by sexual impotence, dysuria, decreased sweating and weight-loss, which progressed gradually and reached their maximum seven months after the onset. He was given 400 mg of droxydopa and 8 mg of midodrine HCL per day without apparent benefits, and was admitted to our hospital. His blood pressure (mmHg) and heart rate were 167/102 and 68 in supine position, and 74/41 and 62 in sitting position. Skin was dry. Pupillary reactions were sluggish. Left pupil was slightly irregular. Other cranial nerves, sensory and somatic motor functions were normal. Laboratory tests revealed as follows: slight anemia, ESR 42 mm/hour, serum IgG 2236 mg/dl, CSF protein 64 mg/dl and positive tests for non-specific autoantibodies. Nerve conduction studies and electromyogram were normal. Autonomic function tests showed postganglionic impairments of sympathetic and parasympathetic systems. The sural nerve biopsy disclosed normal myelinated fibers, but decreased unmyelinated fiber density to 60% of the control value. No demyelinating lesions, cell infiltration or amyloid deposits were seen. Under the diagnosis of idiopathic pure pan-dysautonomia, prednisolone, initially 60 mg daily, was added. Within 10 days, he showed marked improvement of general conditions. No exacerbation was seen during reduction or after withdrawal of prednisolone. Repeated tests showed normalizing laboratory data and regression of autonomic deficits. A year after onset he regained normal social life.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0930_17097", "title": "Infliximab-Induced Lupus: A Case Report.", "score": 0.009259259259259259, "content": "We report the case of a 48-year-old, leukodermic female diagnosed with ulcerative proctitis for 4 years and latent tuberculosis. She was allergic to salicylates and had a minor allergic reaction to infliximab (rash, vertigo, and headache). Thereafter, she started azathioprine (2.5 mg/kg/day). She maintained intravenous infliximab, together with prophylaxis with clemastine and hydrocortisone, due to the steroid-dependent proctitis. The therapy was continued every 8 weeks with anti-tumor necrosis factor for about 3 years. The analytical evaluation when she was diagnosed with ulcerative proctitis (February 2011) showed negative antinuclear antibodies (ANA), double-stranded-DNA antibodies (anti-dsDNA), antineutrophil cytoplasmic antibodies and anti-<iSaccharomyces cerevisiae</i antibodies, and a positive outer membrane protein antibody. About 2 years and 6 months after starting infliximab (November 2013), the patient complained of inflammatory symmetrical polyarthralgia (knee, shoulder, elbow, and wrist) without synovitis, which started every week before the administration of infliximab. Resolution of symptoms was observed after each infliximab infusion. In July 2014, the autoantibody re-evaluation showed positive ANA with a homogeneous pattern with a titer of 1:640, weak positive anti-dsDNA (30.2), and positive anti-histone with C3 decreased (80.3). She was then diagnosed with lupus induced by infliximab and initiated hydroxychloroquine 400 mg. Infliximab was suspended. On re-evaluation, the erythrocyte sedimentation rate was 25 mm/h (1st hour), C-reactive protein 0.5 mg/dL (previously erythrocyte sedimentation rate 15 mm/h and C-reactive protein 1.2 mg/dL), and endoscopically, the mucosa was scarred, with some atrophy and scarce mucus in the lower rectum. About 10 months after discontinuation of infliximab, repeated autoantibodies proved all negative, keeping only low C3 (87). The patient also reported complete resolution of the arthralgia."}, {"id": "pubmed23n0323_13890", "title": "[Diffuse panbronchiolitis with myeloperoxidase-specific antineutrophil cytoplasmic antibody-related vasculitis].", "score": 0.009259259259259259, "content": "A 46-year-old woman was referred to our department in July 1996 with complaints of fever and myalgia in her calves. She had a 20-year history of purulent sputum; diffuse panbronchiolitis had been diagnosed in 1983. Physical examination revealed low-pithed rhonchi over the lung fieldis and hypesthesia of the right leg. She had a white blood cell count of 16,100/mm3, including 4% eosinophils, and a platelet count of 80.0 x 10(4)/mm3. The serum IgE level was 2,200 U/ml, and the cold hemagglutinin titer was high. Pulmonary-function tests showed mixed ventilatory dysfunction, and arterial blood gas analysis revealed a PaO2 of 55.8 Torr on room air. Pseudomonas aeruginosa was cultured from her sputum. A chest X-ray film and CT scan showed diffuse nodular shadows and bronchiectatic changes with mild hyperinflation. An infiltrative lesion in right S6 area could also be seen. Administration of broad-spectrum antibiotics did not alleviate her symptoms. The level of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA) in serum was 245 EU/ml, and 67Ga scintigraphy showed marked accumulation in the abdomen. Abdominal angiography demonstrated a bead-like appearance and irregularities in the peripheral branches of the hapatic artery, the splenic artery, the cystic artery, and the superior mesenteric artery. Because of the high MPO-ANCA level and the angiographic abnormalities, MPO-ANCA-related vasculitis was diagnosed. She was treated with 1 g of methylprednisolone daily for 3 days, followed by 60 mg of prednisolone and 50 mg of cyclophosphamide daily. Her condition improved dramatically, and the MPO-ANCA level became almost normal. During treatment, her blood pressure rose markedly with a normal serum creatinine level and normal urinalysis. Plasma renin activity was 13.3 ng/ml/hr. Renal angiography showed stenoses and irregularities in the peripheral branches of renal arteries bilaterally. These findings led to a diagnosis of renovascular hypertension due to vasculitis. Her blood pressure was controlled with an angiotensin-converting enzyme inhibitor and a calcium antagonist. Vasculitis associated with chronic supportive lung disease has occasionally been reported, which suggests a casual relation between chronic respiratory infection and ANCA-related vasculitis. Systemic vasculitis should be taken into account as a potential complication of chronic suppurative lung disease."}, {"id": "pubmed23n0422_20674", "title": "[Two cases of rheumatoid arthritis developed after polymyositis].", "score": 0.009174311926605505, "content": "We report two cases of rheumatoid arthritis (RA) who later had developed after polymyositis (PM). The first patient was 64-year old male who experienced muscular weakness of the four limbs in proximity 10 years ago. He was diagnosed as PM because of the elevated serum CK and the myogenic pattern of EMG, and his symptoms were improved by treatment with corticosteroid. He started to complain polyarthralgia 2 years ago, followed by interstitial pneumonia, pleuritis and skin ulcer. He was admitted because of exacerbated polyarthralgia, multiple subcutaneous nodules, skin eruption and fever. The level of serum CK was within normal range but CRP was elevated and CH 50 was decreased. The laboratory examination showed positive cryoglobulin and high titer of rheumatoid factor, but anti-Jo 1 antibody was negative. The hand X-ray showed bone erosions in bilateral wrist joints. Skin biopsy revealed leukocytoclastic vasculitis. Based on these findings, he was diagnosed as malignant RA. He was successfully treated with methylprednisolone pulse therapy, cyclophosphamide and prostaglandin E 1. The second patient was 77-year old male with pneumoconiosis who experienced muscular weakness of the four limbs in proximity 4 years ago. He was diagnosed as PM based on his clinical and laboratory findings and was treated with temporary corticosteroid. He started to have polyarthralgia last year, and he was admitted because of increasing arthralgia after the treatment of pulmonary tuberculosis. The level of serum CK was slightly elevated due to hypothyroidism, and CRP was highly elevated. Rheumatoid factor and cryoglobulin were positive, but anti-Jo 1 antibody was negative. The hand X-ray showed bone erosions in bilateral wrist joints. Crystals of pyrophosphate calcium was observed in knee joints. He was diagnosed as RA associate with pseudogout. His symptoms were relieved with corticosteroid, salazosulfapyridine and anti-tuberculous therapy. These two cases had altered their clinical features from PM to definite RA, and both had pulmonary complications. Previous reports described the cases of RA followed by PM, most of which were induced by such drugs as D-penicillamine, but the cases of PM who later had developed RA are extremely unusual. The overlapped cases of RA and PM tend to highly associate with pulmonary lesions."}, {"id": "pubmed23n0715_3812", "title": "Systemic lupus erythematosus following HPV immunization or infection?", "score": 0.00909090909090909, "content": "The link between autoimmunity and infectious agents has been strongly suggested by reports of lupus or lupus-like syndromes following immunization. This report describes three patients with either newly diagnosed systemic lupus erythematosus (SLE) or SLE flare, following vaccination for human papilloma virus (HPV). CASE 1: A 17-year-old female completed two doses of HPV vaccine uneventfully. Two months later, she developed arthralgias with pruritic rashes on both lower extremities, later accompanied by livedo reticularis, bipedal edema with proteinuria, anemia, leucopenia, hypocomplementemia and high titers of anti-nuclear antibody (ANA) and anti-double-stranded DNA (anti-dsDNA). Kidney biopsy showed International Society of Nephrology/Renal Pathology Society Class III lupus nephritis. She was started on high dose steroids followed by pulse cyclophosphamide therapy protocol for lupus nephritis, and subsequently went into remission. CASE 2: A 45-year-old housewife, previously managed for 11 years as having rheumatoid arthritis, had been in clinical remission for a year when she received two doses of HPV immunization. Four months later, she developed fever accompanied by arthritis, malar rash, oral ulcers, recurrent ascites with intestinal pseudo-obstruction, and behavioral changes. Cranial MRI showed vasculitic lesions on the frontal and parietal lobes. Laboratory tests showed anemia with leucopenia, hypocomplementemia, proteinuria, ANA positive at 1:320, and antibodies against dsDNA, Ro/SSA, La/SSB and histone. She improved following pulse methylprednisolone with subsequent oral prednisone combined with hydroxychloroquine. CASE 3: A 58-year-old housewife diagnosed with SLE had been in clinical remission for 8 years when she received two doses of HPV immunization. Three months later, she was admitted to emergency because of a 1-week history of fever, malar rash, easy fatigability, cervical lymph nodes, gross hematuria and pallor. Laboratory exams showed severe anemia, thrombocytopenia, active urine sediments, and hypocomplementemia. Despite pulse steroid therapy, blood transfusions, intravenous immunoglobulin and aggressive resuscitative measures, she expired a day after hospital admission. These cases narrate instances of the onset or exacerbation of lupus following HPV immunization suggesting adjuvant-induced autoimmunity. On the other hand, there are reports of higher incidence of HPV infection in SLE, with the infection per se possibly contributing to disease activity. Thus, the benefit of HPV immunization may still outweigh the risk among these individuals."}, {"id": "pubmed23n1018_16235", "title": "Development of anti-centromere antibody-positive autoimmune hepatitis after childbirth.", "score": 0.009009009009009009, "content": "This is the first case involving the development of anti-centromere antibody (ACA)-positive autoimmune hepatitis (AIH) after childbirth that triggered nailfold bleeding. A 32-year-old woman visited a dermatologist presenting with nailfold bleeding 6 months after the delivery of her second baby. Blood tests revealed liver dysfunction, and she was admitted to our hospital. Tests for hepatitis virus, antinuclear antibody, and anti-mitochondrial antibody were negative. She had no history of alcohol consumption or oral medication. Because of nailfold bleeding, we performed tests for ACA, anti-Scl-70 antibody, anti-RNA polymerase III antibody, and anti-U1 RNP antibodies; only ACA was positive. A liver biopsy was performed for the diagnosis of liver dysfunction. Histological examination of the liver biopsy specimen showed moderate infiltration of inflammatory cells, interface hepatitis, bridging fibrosis, and bile duct injury. The AIH international score was 17, and thus, we diagnosed AIH. Oral prednisolone (PSL) 0.6 mg/day/body weight was initiated. Two weeks post-treatment, the liver enzyme levels normalized and the nailfold bleeding disappeared. In case of nailfold bleeding complicated with liver dysfunction post-childbirth, ACA-positive AIH should be considered as a differential diagnosis."}, {"id": "pubmed23n0414_6005", "title": "[Systemic sclerosis associated with microscopic polyangitis presenting with high myeloperoxidase (MPO) titer and necrotizing angitis: a case report].", "score": 0.008928571428571428, "content": "We herein report a case of systemic sclerosis associated with microscopic polyangitis. The patient was a 54-year-old woman, who was diagnosed to have systemic sclerosis at a hospital in 1992, but she did not receive any medical treatment. She had been suffering from pyrexia, paresthesia and muscle weakness of both lower limbs since the beginning of 2001, and was introduced to our hospital. She showed hardened skin extending from her fingers to upper arms, weakness in both lower limbs and livedo reticularis. Her laboratory test showed WBC 11, 600/microliter, CRP 6.63 mg/dl, CH 50 24 U/ml, anti Scl-70 antibody 90.1 index, and MPO-ANCA 281 EU, but no impaired renal function was recognized. Chest computed tomography showed interstitial pneumonia while necrotising vasculitis of the right sural nerve was found in a biopsy specimen. Based on these findings, we diagnosed her to have systemic sclerosis accompanied with microscopic polyangitis (MPA). She received steroid treatment after the diagnosis was made, and her symptoms and the laboratory findings thereafter immediately improved. Many cases have been reported to have ANCA positive systemic sclerosis among patients with systemic sclerosis that are complicated MPO-ANCA-related vasculitis. However, since our patient demonstrated necrotising vasculitis in a sural nerve biopsy and no evidence of an impaired renal function, we diagnosed her to have systemic sclerosis complicated with MPA instead of ANCA positive systemic sclerosis. The pathological state of this patient thus seemed to be different from that of ANCA-positive systemic sclerosis. We concluded that this patient had both systemic sclerosis and MPA. It is therefore important to note that some patients who have been reported to have ANCA-positive systemic sclerosis may also have systemic sclerosis complicated with MPA."}, {"id": "pubmed23n0645_2929", "title": "[Interstitial pneumonia complicating amyopathic dermatomyositis: a case report].", "score": 0.008849557522123894, "content": "Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the absence of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by neoplasm or interstitial pneumonia that shows a rapid progressive course both of them are associated with a poor prognosis. A 56-year-old woman with no medical history was referred to the department of medicine because of arthralgia with a remarkable weight loss. She also complained of rapidly progressive dyspnea, cough and photosensitivity. Physical examination on admission showed scaly erythema on the dorsum of the hands (Gottron sign) and periorbital edema with a purplish appearance (heliotropic rash), arthritis, but no muscle weakness. Auscultation of the chest identified audible fine crackles on the lower aspects of both lungs. Results of laboratory findings on admission revealed a lymphopenia. The serum creatine kinase and serum lactate dehydrogenase concentration were normal. IRM muscle and electromyography were normal. Antinuclear antibody was positive 1:80 and anti-Jo-1 antibody and other autoantibodies to specific antigens were all negative. High resolution computed tomographic chest scans also revealed diffuse ground-glass opacities in both lungs with basilar predominance. Arterial blood gas analysis revealed hypoxia and hypocapnia. LBA was not performed because of the deterioration of respiratory symptoms. There was no neoplasm associated. The diagnosis of ADM complicated with ADM rapidly progressive interstitial pneumonia was made. Despite of IV methylprednisolone pulse therapy (1g*day-1 for 3 days) and cyclophosphamide, she died by respiratory failure."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": true, "char_ranges": [[32, 137]], "word_ranges": [[4, 19]], "text": "Barrett's esophagus does not necessarily imply peptic stricture, but assuming it does, it is progressive."}, "2": {"exist": true, "char_ranges": [[208, 381]], "word_ranges": [[27, 52]], "text": "That leaves distal esophageal ring and eosinophilic esophagitis; both are possible, but the insistence on the patient's atopic burden indicates the likelihood of the latter."}, "3": {"exist": true, "char_ranges": [[138, 207]], "word_ranges": [[19, 27]], "text": "Infectious esophagitis is more typical of immunocompromised patients."}, "4": {"exist": true, "char_ranges": [[208, 381]], "word_ranges": [[27, 52]], "text": "That leaves distal esophageal ring and eosinophilic esophagitis; both are possible, but the insistence on the patient's atopic burden indicates the likelihood of the latter."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "This is intermittent dysphagia. Barrett's esophagus does not necessarily imply peptic stricture, but assuming it does, it is progressive. Infectious esophagitis is more typical of immunocompromised patients. That leaves distal esophageal ring and eosinophilic esophagitis; both are possible, but the insistence on the patient's atopic burden indicates the likelihood of the latter.", "full_answer_no_ref": "This is intermittent dysphagia. Barrett's esophagus does not necessarily imply peptic stricture, but assuming it does, it is progressive. Infectious esophagitis is more typical of immunocompromised patients. That leaves distal esophageal ring and eosinophilic esophagitis; both are possible, but the insistence on the patient's atopic burden indicates the likelihood of the latter.", "full_question": "18-year-old young man with a history of asthma, allergy to pollens, mites and cat hair, comes to the emergency room referring sensation of food detention at retrosternal level with practical inability to swallow his own saliva. He refers similar episodes on other occasions that have subsided spontaneously within a few minutes. Which of the following is the most likely diagnosis?", "id": 273, "lang": "en", "options": {"1": "Barrett's esophagus.", "2": "Distal esophageal ring (Schatzki).", "3": "Infectious esophagitis.", "4": "Eosinophilic esophagitis.", "5": NaN}, "question_id_specific": 71, "type": "DIGESTIVE SYSTEM", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en087_9091", "title": "Eosinophilic esophagitis", "score": 0.017624223602484473, "content": "Esophageal strictures and rings can be safely dilated in EoE. It is recommended to use a graduated balloon catheter for gradual dilation. The patient should be informed that after dilation they might experience chest pain and in addition risk of esophageal perforation and bleeding. Prognosis The long-term prognosis for patients with EoE is unknown. Some patients may follow a “waxing and waning” course characterized by symptomatic episodes followed by periods of remission. There have also been reports of apparent spontaneous disease remission in some patients; however, the risk of recurrence in these patients is unknown. It is possible that long-standing, untreated disease may result in esophageal remodeling, leading to strictures, Schatzki ring and, eventually, achalasia. History The first case of eosinophilic esophagitis was reported in 1978. In the early 1990s, it became recognized as a distinct disease. See also Eosinophilic gastroenteritis References External links"}, {"id": "pubmed23n0542_13782", "title": "Eosinophilic esophagitis: an overlooked entity in chronic dysphagia.", "score": 0.01611875637104995, "content": "A 40-year-old white male with atopy presented to our department in March 2004 with a history of chronic heartburn and solid-food dysphagia since 1994. The patient was taking on-demand salbutamol for asthma and ranitidine for mild heartburn, occurring less than once per week. Eight years previously, he had undergone esophageal dilatation for a Schatzki's ring. Physical examination, laboratory investigations, video esophagram, upper endoscopy with mid-esophageal biopsies, and skin testing for a number of food and environmental allergens. Diagnosis Eosinophilic esophagitis. Topical steroids with a fluticasone 220 microg multiple-dose inhaler, four puffs swallowed twice a day for 6 weeks."}, {"id": "pubmed23n0521_7946", "title": "Eosinophilic esophagitis in adults, an emerging cause of dysphagia. Description of 9 cases.", "score": 0.01595873786407767, "content": "Eosinophilic esophagitis is a rare condition mainly affecting children, although the number of cases reported in adults is on the increase. It is characterized by intense infiltration of eosinophilic leukocytes in the esophageal mucosa, without involvement of other sections of the alimentary canal. Over the past year, following the performance of endoscopies and biopsies, our service identified nine patients who were diagnosed with suffering from this disorder. Each patient sought medical help for episodes of long-term, self-limited dysphagia or food impaction in the alimentary canal. Endoscopy revealed esophageal stenosis in the form of simultaneous contraction rings or regular stenosis. In six cases, the manometric study showed a nonspecific motor disorder of severe intensity affecting the esophageal body, and another patient had a disorder characterized by the presence of simultaneous waves and secondary peristaltic waves in the three thirds of the organ. These disorders are presumably due to eosinophilic infiltration of the muscular layer or ganglionar cells of the esophagus, and account for symptoms in these patients. Although the etiopathogenesis of this illness is uncertain, it is clearly an immunoallergic manifestation. As the number of diagnosed cases is on the increase, eosinophilic esophagitis is in adults a specific entity within the differential diagnosis of dysphagia in young males with a history of allergies. Eosiniphilic esophagitis responds in a different number of ways to therapies used. We successfully used fluticasone propionate, a synthetic corticoid applied topically, which proved to be efficient in the treatment of this illness by acting on the pathophysiological basis of the process. It does not have any adverse effects, thus offering advantages over other therapies such as systematic corticoids or endoscopic dilations."}, {"id": "pubmed23n0490_13884", "title": "Eosinophilic esophagitis in adults: an emerging problem with unique esophageal features.", "score": 0.015176419022572867, "content": "Eosinophilic esophagitis is an inflammatory condition in which there is dense eosinophilic infiltration of the surface lining of the esophagus. Reports of eosinophilic esophagitis pertain almost exclusively to pediatric populations. However, eosinophilic esophagitis is emerging as a clinical affliction of adults. This report describes the clinical and endoscopic findings of eosinophilic esophagitis in the largest cohort of adult patients reported to date. Twenty-nine patients (21 men, 8 women; mean age 35 years) with documented eosinophilic esophagitis (&gt;/=15 eosinophils per high-power field in biopsy specimens) and a significant history of chronic dysphagia for solid food (24 patients) were evaluated clinically and endoscopically during a 3-year period (1999-2002). Fourteen patients (48%) had a history of asthma, environmental allergy, or atopy. In a subset of 15 patients, the diagnostic accuracy of endoscopy was compared with that of barium contrast esophagography. Twenty-seven patients (93%) had abnormal endoscopic findings; 25 (86%) had unique esophageal structural changes, associated with a preserved mucosal surface, that were highly atypical for acid reflux injury. Structural alterations seen in adult patients with eosinophilic esophagitis may occur in combination or as a primary characteristic, e.g., uniform small-caliber esophagus, single or multiple corrugations (rings), proximal esophageal stenosis, or 1 to 2 mm whitish vesicles scattered over the mucosal surface. Barium contrast radiography combined with swallow of a barium-coated marshmallow identified 10 (67%) of the primary features observed endoscopically in 15 patients. However, radiography failed to detect other features noted at endoscopy (e.g., only 3/6 patients with proximal stenosis, 5/9 patients with concentric rings and none of 4 patients with small caliber esophagus). Eight of the 29 patients (20%) had a history of chronic heartburn. Twelve patients had been treated with a proton pump inhibitor and only 3 reported some improvement in the severity of dysphagia. Relatively young age, a history of chronic dysphagia for solid food, and endoscopic detection of unique structural alterations atypical for GERD in an adult patient should prompt a suspicion of EE and subsequent biopsy confirmation. Acid reflux appears to have a secondary role in eosinophilic esophagitis. In an uncontrolled comparison, endoscopy was superior to barium contrast radiography for the diagnosis of eosinophilic esophagitis. The incidence of eosinophilic esophagitis in adults appears to be increasing."}, {"id": "pubmed23n1088_7066", "title": "A case of lymphocytic esophagitis in a woman with multiple allergies.", "score": 0.0150997150997151, "content": "Lymphocytic esophagitis is a newly recognized entity of unknown origin. Dysphagia is defined as difficulty swallowing and represents a common symptom in the general population with a prevalence of approximately 20%. Chronic inflammation of the esophageal wall may manifest itself clinically and endoscopically, mimicking inflammation of another origin. However, little is known about the pathogenesis of the disease, as patients are seldom suspected and rarely diagnosed with lymphocytic esophagitis. Here, we present a rare case of lymphocytic esophagitis in a patient with multiple allergies and suspected eosinophilic esophagitis. A 28-year-old woman with polyvalent sensitization to food and inhalant allergens presented with intermittent dysphagia, a sensation of a foreign body in the throat, itchiness of the oral cavity after ingesting certain foods, heartburn, and prolonged chewing time. A skin prick test showed positive results for birch-tree, alder, hazel, and rye pollen, as well as house dust mites. Apart from obesity (BMI 30 kg/m<sup2</sup), multiple pustules and excoriations on the skin, her physical examination was insignificant. Esophagogastroduodenoscopy (EGD) was performed revealing full-length but discrete trachealization of the esophagus. A barium swallow test showed slowing of esophageal peristalsis in the recumbent position. No esophageal pathology was observed. A histopathological analysis of mucosal samples revealed slight hyperplasia of the basal layer of the esophagus, and the stomach showed changes typical of chronic gastritis. In summary, this clinical case illustrates that lymphocytic esophagitis, as a newly recognized entity, should be considered in the differential diagnosis of chronic dysphagia. Additionally, when treating allergic patients, clinicians should be aware that lymphocytic esophagitis, distinct from eosinophilic esophagitis, should be considered in the diagnosis of patients with atopy and upper gastrointestinal symptoms."}, {"id": "wiki20220301en184_21994", "title": "Esophageal food bolus obstruction", "score": 0.014209207912239785, "content": "Risk factors Food bolus obstruction is most commonly caused by Schatzki rings, which are mucosal rings of unknown cause in the lower esophagus. Foodstuff jams into the esophagus due to the narrowing caused by the ring. An increasingly commonly recognized cause for esophageal food bolus obstruction is eosinophilic esophagitis, which is an inflammatory disorder of the mucosa of the esophagus, of unknown cause. Many alterations caused by eosinophilic esophagitis can predispose to food boluses; these include the presence of multiple rings and narrowing of the lumen. When considering esophageal dilation to treat a patient with food bolus obstruction, care must be made to look for features of eosinophilic esophagitis, as these patients are at a higher risk of dilation-associated complications."}, {"id": "pubmed23n0843_3849", "title": "Eosinophilic Esophagitis with Dysphagia and Food Impaction in a Young Adult.", "score": 0.01414858514148585, "content": "Eosinophilic esophagitis (EoE) is an emerging esophageal disease associated with dysphagia and food impaction. Practice guidelines have only recently been developed. It affects 1/1000 individuals, predominantly young men. As this demographic represents a substantial portion of the military aviation population, aerospace medicine clinicians should be familiar with this diagnosis when evaluating dysphagia or impactions. A 23-yr-old Caucasian man, a U.S. Air Force air traffic controller, presented to Flight Medicine following an episode of food impaction requiring evaluation in the local emergency department. The patient reported a 5-yr history of recurrent episodes of food lodging in his throat, requiring fluid and body repositioning for resolution. Medical history was significant for eczema. Upper endoscopy revealed an abnormal esophagus with macroscopic features of EoE and biopsies were also consistent with EoE. After further work-up, the patient was diagnosed with EoE and treated. Significant symptom improvement was noted after 2 mo of therapy. This case outlines the evaluation of food impaction as well as the diagnostic criteria for EoE, which is a disease that affects patients with demographics common to the military aviation community. As the diagnostic and treatment guidelines for EoE are relatively new, it may easily be overlooked by the primary care physician, causing a delay in subspecialist consultation, thus delaying treatment. EoE is a condition with symptoms that pose high risk to the performance of aircrew duties; therefore, flight surgeons must be familiar with the aeromedical standards that accompany this diagnosis."}, {"id": "pubmed23n0559_6518", "title": "Non stenotic food impaction due to eosinophilic esophagitis: a potential surgical emergency.", "score": 0.014034068912117692, "content": "Eosinophilic esophagitis (EoE) is an emergent condition in which a mucosal infiltrate of &gt; 20 eosinophils per high power microscopic field is accompanied by motor disturbances that may cause food impaction in the absence of esophageal stricture. We report a series of such cases to point out the potential involvement of pediatric surgeons in diagnosis and treatment. Furthermore, data on the motor function of the esophagus investigated manometrically is included. Thirteen patients with EoE were referred to our emergency room for acute food bolus impaction. Their median age at diagnosis was 12 years (range 7.6-14.4). History of allergy, endoscopy with biopsy and esophageal function (24-h combined ambulatory manometry with simultaneous pH-metry) were investigated. In 7 patients emergency endoscopic extraction of the impacted bolus was necessary. Allergic tests were positive in eight patients. The pH probe showed gastroesophageal reflux in two cases. Upon endoscopy, typical features of EoE (esophageal trachealization and whitish papular exudates) were found. Ambulatory 24-h manometry revealed abnormal motility of the distal esophagus with strikingly high amplitudes (&gt; 150 mmHg) and long duration (&gt; 7 sec) of the waves, particularly during the night. Six patients responded rapidly to steroids and/or antiallergic treatment. The remaining patients had a good outcome with dietary treatment alone. EoE is an emergent condition that may involve the pediatric surgeon in both the diagnosis and treatment. Typical endoscopic findings and biopsy are required for proper diagnosis. Ambulatory manometry reveals a marked propulsive dysfunction that explains impaction. This dysfunction is reversible, since the symptoms usually disappear with steroids or antiallergic treatment."}, {"id": "pubmed23n1121_6823", "title": "Eosinophilic Esophagitis After an Allegra-D Bolus: A Case Report.", "score": 0.013897866839043309, "content": "Eosinophilic esophagitis (EoE) is an immune-mediated disorder that may be related to exposure to additive chemicals in crops, air pollutants, or supplements found within livestock. Co-occurring allergic or atopic diseases including atopic dermatitis, food allergies, and asthma are also commonly seen in 70% of cases and help guide diagnosis. Diagnosis of EoE requires eosinophilic infiltration greater than 15 eosinophils per high power field (HPF) with endoscopic evidence of abnormal esophageal changes. Here, we discuss a rare presentation of food bolus impaction secondary to EoE after ingestion of a nasal decongestant and antihistamine pill that has previously never been described in the literature. A 22-year-old male with no significant past medical history presented to the emergency department (ED) with a chief complaint of a sudden onset respiratory distress, regurgitation of clear oral secretions, and globus sensation post ingestion of a fexofenadine-pseudoephedrine tablet. Prior to intake of the capsule, the patient was consuming liquids and solids appropriately. The patient was afebrile, hypertensive at 172/114, and found to have a normal heart rate of 88 bpm and a respiration rate of 18 breaths per minute. An esophagogastroduodenoscopy (EGD) was performed, which revealed a fexofenadine-pseudoephedrine capsule at 23 cm from the incisors along with a superficial ulceration at the corresponding level in the esophagus. The foreign body was successfully removed using raptor forceps. Further visualization demonstrated trachealization of the esophagus and furrowing and severe narrowing (&lt; 10mm), which raised suspicion for EoE. Proximal biopsy indicated 16 intraepithelial eosinophils per HPF within the squamous epithelium, likely compatible with EoE. The patient tolerated the procedure well and was discharged on an eight-week course of proton-pump inhibitors. EoE is defined as an immune-mediated esophageal disease characterized histologically by eosinophil-predominant inflammation. Our patient was reported to have up to 30 eosinophils per HPF from the proximal esophageal biopsy, which satisfies the requirements for an EoE diagnosis. Based on the current literature review, there have been no other reported cases of symptomatic food bolus impaction secondary to EoE after ingestion of antihistamines."}, {"id": "pubmed23n0681_12902", "title": "Eosinophilic esophagitis: clinical features, endoscopic findings and response to treatment.", "score": 0.013699245418613008, "content": "Eosinophilic esophagitis (EE) is a motility disorder of the esophagus that typically presents with dysphagia. The objective of the present study was to explore patient characteristics, clinical and endoscopic features, and response to treatment of patients with EE. Patients were selected retrospectively based on a review of biopsy results from previous endoscopies performed between 2004 and 2008. A total of 54 patients (41 men and 13 women) with biopsy-proven EE were included in the study. Further information regarding the patients' clinical and endoscopic features, and response to treatment were obtained through chart reviews and patient telephone interviews. The mean age of the patients at symptom onset was 30 years. All patients complained of dysphagia, 81% had a history of bolus obstruction, 43% had a history of asthma and 70% had a history of environmental allergies. Thirty-three per cent had a family history of asthma, while 52% had a family history of food or seasonal allergies. The most common endoscopic findings were rings and⁄or corrugations, which were found in 63% of patients. Swallowed fluticasone therapy resulted in symptom resolution in 74% of patients; however, 79% of these patients relapsed after discontinuing fluticasone therapy and required repeat treatments. Esophageal dilation was complication free and resulted in improvement in 80% of patients. However, 83% of those reporting improvement relapsed within one year. The clinical and endoscopic findings were similar to those found in the literature, with most patients requiring ongoing, repeated therapies. Further studies are needed to assess the safety and efficacy of treatment modalities ideally suited to patients with EE."}, {"id": "wiki20220301en010_78283", "title": "Heartburn", "score": 0.013098568372552246, "content": "Esophagus GERD (most common cause of heartburn) occurs when acid refluxes from the stomach and inflames the esophagus. Esophageal spasms typically occur after eating or drinking and may be combined with difficulty swallowing. Esophageal strictures Esophageal cancers Esophagitis GERD Eosinophilic esophagitis - a disease commonly associated with other atopic diseases such as asthma, food allergies, seasonal allergies, and atopic skin disease Mallory-Weis tears - tears of the superficial mucosa of the esophagus that are subsequently exposed to gastric acid commonly due to vomiting and/or retching Chemical esophagitis - related to the intake of caustic substances, excessive amounts of hot liquids, alcohol, or tobacco smoke Infections may explain heartburn symptoms. These especially include CMV and certain fungal infections, most common in immunocompromised persons Stomach"}, {"id": "wiki20220301en184_21992", "title": "Esophageal food bolus obstruction", "score": 0.013027916964924838, "content": "An esophageal food bolus obstruction is a medical emergency caused by the obstruction of the esophagus by an ingested foreign body. It is usually associated with diseases that may narrow the lumen of the esophagus, such as eosinophilic esophagitis, Schatzki rings, peptic strictures, webs, or cancers of the esophagus; rarely it can be seen in disorders of the movement of the esophagus, such as nutcracker esophagus. While some esophageal food boli can pass by themselves or with the assistance of medications, some require the use of endoscopy to push the obstructing food into the stomach, or remove it from the esophagus. The use of glucagon, while common, has not been found to be useful. Eponymous names include 'the steakhouse syndrome' and 'backyard barbeque syndrome'."}, {"id": "wiki20220301en087_9078", "title": "Eosinophilic esophagitis", "score": 0.012969156447417318, "content": "Predominant symptoms in school-aged children and adolescents include difficulty swallowing, food impaction, and choking/gagging with meals- particularly when eating foods with coarse textures. Other symptoms in this age group can include abdominal/chest pain, vomiting, and regurgitation. The predominant symptom in adults is difficulty swallowing; however, intractable heartburn and food avoidance may also be present. Due to the long-standing inflammation and possible resultant scarring that may have gone unrecognized, adults presenting with EoE tend to have more episodes of esophageal food impaction as well as other esophageal abnormalities such as Schatzki ring, esophageal webs, and in some cases, achalasia."}, {"id": "pubmed23n0747_13079", "title": "Endoscopic findings in patients with Schatzki rings: evidence for an association with eosinophilic esophagitis.", "score": 0.012952101661779082, "content": "To investigate endoscopic findings in patients with Schatzki rings (SRs) with a focus on evidence for eosinophilic esophagitis (EoE). We consecutively approached all adult patients scheduled for elective outpatient upper endoscopy for a variety of indications at the German Diagnostic Clinic, Wiesbaden, Germany between July 2007 and July 2010. All patients with endoscopically diagnosed SRs, defined as thin, symmetrical, mucosal structures located at the esophagogastric junction, were prospectively registered. Additional endoscopic findings, clinical information and histopathological findings with a focus on esophageal eosinophilia (≥ 20 eosinophils/high power field) were recorded. The criteria for active EoE were defined as: (1) eosinophilic tissue infiltration ≥ 20 eosinophils/hpf; (2) symptoms of esophageal dysfunction; and (3) exclusion of other causes of esophageal eosinophilia. Gastroesophageal reflux disease was excluded by proton pump inhibitor treatment prior to endoscopy. The presence of ≥ 20 eosinophils/hpf in esophageal biopsies in patients that did not fulfil the criteria of EoE was defined as esophageal hypereosinophilia. A SR was diagnosed in 171 (3.3%; 128 males, 43 females, mean age 66 ± 12.9 years) of the 5163 patients that underwent upper gastrointestinal-endoscopy. Twenty of the 116 patients (17%) from whom esophageal biopsies were obtained showed histological hypereosinophilia (≥ 20 eosinophils/hpf). Nine of these patients (8 males, 1 female, mean age 49 ± 10 years) did not fulfill all diagnostic criteria of EoE, whereas in 11 (9%) patients with ≥ 20 eosinophils/hpf, a definite diagnosis of EoE was made. Three of the 11 patients (27%) with definite EoE had no suspicious endoscopic features of EoE. In contrast, in the 25 patients in whom EoE was suspected by endoscopic features, EoE was only confirmed in 7 (28%) patients. Patients with EoE were younger (mean age 41.5 ± 6.5 vs 50.5 ± 11.5 years, P = 0.012), were more likely to have a history of allergies (73% vs 29%, P = 0.007) and complained more often of dysphagia (91% vs 34%, P = 0.004) and food impaction (36% vs 6%, P = 0.007) than patients without EoE. Endoscopically, additional webs were found significantly more often in patients with EoE than in patients without EoE (36% vs 11%, P = 0.04). Furthermore, the SR had a tendency to be narrower in patients with EoE than in those without EoE (36% vs 18%, P = 0.22). The percentage of males (73% vs 72%, P = 1.0) and frequency of heartburn (27% vs 27%, P = 1.0) were not significantly different in both groups. The 9 patients with esophageal hypereosinophilia that did not fulfil the diagnostic criteria of EoE were younger (mean age 49 ± 10 years vs 58 ± 6 years, P = 0.0008) and were more likely to have a history of allergies (78% vs 24%, P = 0.003) than patients with &lt; 20 eosinophils/hpf. Predictors of EoE were younger age, presence of dysphagia or food impaction and a history of allergies. A significant proportion of patients with SRs also have EoE, which may not always be suspected according to other endoscopic features."}, {"id": "pubmed23n0615_21539", "title": "Ring(s)-related esophageal meat bolus impaction: biopsy first, dilate later.", "score": 0.012933285134755192, "content": "Distal esophageal or Schatzki's rings are a common cause of intermittent solid food dysphagia requiring endoscopic dilation for relief. Similarly, eosinophilic esophagitis (EE) is a rapidly emerging disease in both children and young adults, and manifests as dysphagia to solids and/or episodic food bolus impaction. Endoscopic findings vary considerably among patients with EE, posing significant recognition and management challenges. Esophageal dilation in EE can be painful and risky. This case report describes a patient with acute food bolus impaction due to underlying Schatzki's ring and associated but clinically indolent EE, and highlights some safety aspects of esophageal dilation."}, {"id": "InternalMed_Harrison_22452", "title": "InternalMed_Harrison", "score": 0.012640716126407162, "content": "EoE is diagnosed based on the combination of typical esophageal symptoms and esophageal mucosal biopsies demonstrating squamous epithelial eosinophil-predominant inflammation. Alternative etiologies of esophageal eosinophilia include GERD, drug hypersensitivity, connective tissue disorders, hypereosinophilic syndrome, and infection. Current evidence indicates that EoE is an immunologic disorder induced by antigen sensitization in susceptible individuals. Dietary factors play an important role in both the pathogenesis and treatment of EoE. Aeroallergens may also contribute, but the evidence is weaker. The natural history of EoE is unclear, but an increased risk of esophageal stricture development paralleling the duration of untreated disease has been noted. Diseases of the Esophagus FIGURE 347-11 Endoscopic features of (A) eosinophilic esophagitis (EoE), (B) Candida esophagitis, (C) giant ulcer associated with HIV, (D) and a Schatzki ring."}, {"id": "pubmed23n0634_20531", "title": "Esophageal trachealization: a feature of eosinophilic esophagitis.", "score": 0.012551836492890996, "content": "Eosinophilic esophagitis (EE) is an inflammatory condition characterized by intense eosinophilic infiltration of the esophagus. EE is frequently misdiagnosed as gastroesophageal reflux disease. Here, we present a child with EE and a characteristic endoscopic finding, \"ringed esophagus\". An 11-year-old Saudi boy presented with dysphagia for 1 year. He had experienced an intermittent sensation of solid food sticking in his chest, which was relieved by drinking liquids. A barium swallow excluded anatomical causes of dysphagia, but revealed multiple-ringed esophagus. Endoscopy showed a furrowing and trachealizing appearance of the entire esophagus. Hisologically, extensive eosinophilic infiltration was a feature in biopsies obtained from the esophagus. The child responded well to a 2-month course of inhaled fluticasone. Symptoms recurred 3 months after discontinuation of therapy, which necessitated resumption of inhaled fluticasone. The endoscopic appearance of multiple esophageal rings should raise suspicion of EE and be confirmed by esophageal biopsies."}, {"id": "wiki20220301en010_102256", "title": "Esophagitis", "score": 0.012439261418853256, "content": "For subtypes To treat eosinophilic esophagitis, avoiding any allergens that may be stimulating the eosinophils is recommended. As for medications, proton pump inhibitors and steroids can be prescribed. Steroids that are used to treat asthma can be swallowed to treat eosinophil esophagitis due to nonfood allergens. The removal of food allergens from the diet is included to help treat eosinophilic esophagitis. For infectious esophagitis, medicine is prescribed based on what type of infection is causing the esophagitis. These medicines are prescribed to treat bacterial, fungal, viral, and/or parasitic infections. Procedures An endoscopy can be used to remove ill fragments. Surgery can be done to remove the damaged part of the esophagus. For reflux esophagitis, a fundooplication can be done to help strengthen the lower esophageal sphincter from allowing backflow of the stomach into the esophagus. For esophageal stricture, a gastroenterologist can perform a dilation of the esophagus."}, {"id": "pubmed23n0629_1493", "title": "[Impaction of a \"sausage bread\" in the esophagus--first manifestation of an eosinophilic esophagitis in a 17-year-old patient].", "score": 0.012424277501254005, "content": "A 17-year-old patient was transferred to the emergency room with an impacted food bolus by colleagues from the Department of Otorhinolaryngology. The examination of ear, nose and throat revealed significant amounts of saliva in both recessus piriformis, a radiologic examination of the esophagus showed a foreign body with a diameter of 1.6 cm in the region of the transitional zone of esophagus and stomach with a support level of the contrast medium. Clinical examination and laboratory tests showed no abnormalities. An emergency gastroscopy was performed. The foreign body, already evident in the barium swallow, was found in the distal esophagus. The foreign body was identified as a food bolus and gently advanced into the stomach with the aid of the gastroscope. In the stomach further food residues were detected and the examination was aborted because of increased risk of aspiration. On the next day, an elective gastroscopy was performed. Several biopsies were obtained from the esophagus because eosinophilic esophagitis (EE) was suspected due to clinical symptoms. Histological work-up showed a significant amount of eosinophilic granulocytes (&gt; 15 eosinophils/HPF, 400 x) and reactive changes in the distal esophagus. Therefore, EE was diagnosed. Fluticasone therapy led to amelioration of symptoms and there was no evidence of recurring bolus impaction during follow-up."}, {"id": "wiki20220301en087_9075", "title": "Eosinophilic esophagitis", "score": 0.012369200105519243, "content": "Eosinophilic esophagitis (EoE) is an allergic inflammatory condition of the esophagus that involves eosinophils, a type of white blood cell. In healthy individuals, the esophagus is typically devoid of eosinophils. In EoE, eosinophils migrate to the esophagus in large numbers. When a trigger food is eaten, the eosinophils contribute to tissue damage and inflammation. Symptoms include swallowing difficulty, food impaction, vomiting, and heartburn. Eosinophilic esophagitis was first described in children but also occurs in adults. The condition is not well understood, but food allergy may play a significant role. The treatment may consist of removal of known or suspected triggers and medication to suppress the immune response. In severe cases, it may be necessary to enlarge the esophagus with an endoscopy procedure. While knowledge about EoE has been increasing rapidly, diagnosis of EoE can be challenging because the symptoms and histo-pathologic findings are not specific."}, {"id": "wiki20220301en010_102253", "title": "Esophagitis", "score": 0.012264480665967656, "content": "Lymphocytic esophagitis Lymphocytic esophagitis is a rare and poorly understood entity associated with an increased amount of lymphocytes in the lining of the esophagus. It was first described in 2006. Disease associations may include Crohn's disease, gastroesophageal reflux disease and coeliac disease. It causes similar changes on endoscopy as eosinophilic esophagitis including esophageal rings, narrow-lumen esophagus, and linear furrows. Caustic esophagitis Caustic esophagitis is the damage of tissue via chemical origin. This occasionally occurs through occupational exposure (via breathing of fumes that mix into the saliva which is then swallowed) or through pica. It occurred in some teenagers during the fad of intentionally eating Tide pods. By severity The severity of reflux esophagitis is commonly classified into four grades according to the Los Angeles Classification:"}, {"id": "pubmed23n0665_1477", "title": "[Eosinophilic esophagitis: a rare cause of dysphagia].", "score": 0.012060062508493001, "content": "Eosinophilic esophagitis is an unrecognized and emerging entity. Its incidence increases with allergic disorders. A 29-year-old man presented with a 4-year history of intermittent and paroxysmal dysphagia. The triad including allergy, young age, and impaction of foreign bodies, combined with a chronic dysphagia is almost pathognomonic of eosinophilic esophagitis. Endoscopic esophageal features can be diverse, so systematic esophageal biopsies are required. Diagnosis is established with the demonstration of an eosinophilic infiltrate with a cell count exceeding 15 eosinophils per high power field (×400). First line therapy includes swallowed topical corticosteroids and removal of an allergic cause, when it could be identified."}, {"id": "wiki20220301en114_47912", "title": "Esophageal spasm", "score": 0.011985090932459353, "content": "Signs and symptoms The symptoms may include trouble swallowing, regurgitation, chest pain, heartburn, globus pharyngis (which is a feeling that something is stuck in the throat) or a dry cough. Causes It is not clear what causes esophageal spasms. Sometimes esophageal spasms start when someone eats hot or cold foods or drinks. However, they can also occur without eating or drinking. The increased release of acetylcholine may also be a factor, but the triggering event is not known. Spasms may also be the result of a food intolerance. Diagnosis The diagnosis is generally confirmed by esophageal manometry. DES is present when more than a fifth of swallows results in distal esophageal contractions. NE is present if the average strength of the contractions of the distal esophagus is greater than 180 mmHg but the contraction of the esophagus is otherwise normal."}, {"id": "wiki20220301en009_96992", "title": "Esophagus", "score": 0.011767101898576632, "content": "Inflammation Inflammation of the esophagus is known as esophagitis. Reflux of gastric acids from the stomach, infection, substances ingested (for example, corrosives), some medications (such as bisphosphonates), and food allergies can all lead to esophagitis. Esophageal candidiasis is an infection of the yeast Candida albicans that may occur when a person is immunocompromised. the causes of some forms of esophagitis, such as eosinophilic esophagitis, are not well-characterized, but may include Th2-mediated atopies or genetic factors. There appear to be correlations between eosinophilic esophagitis, asthma (itself with an eosinophilic component), eczema, and allergic rhinitis, though it is not clear whether these conditions contribute to eosinophilic esophagitis or vice versa, or if they are symptoms of mutual underlying factors. Esophagitis can cause painful swallowing and is usually treated by managing the cause of the esophagitis - such as managing reflux or treating infection."}, {"id": "wiki20220301en010_102251", "title": "Esophagitis", "score": 0.011674624432281355, "content": "Some lifestyle indicators for this disease include stress, unhealthy eating, smoking, drinking, family history, allergies, and immunodeficiency. Types Reflux esophagitis Although it usually assumed that inflammation from acid reflux is caused by the irritant action on the mucosa by hydrochloric acid, one study suggests that the pathogenesis of reflux esophagitis may be cytokine-mediated. Infectious esophagitis Esophagitis happens due to a viral, fungal, parasitic or bacterial infection. More likely to happen to people who have an immunodeficiency. Types include: Fungal Candida (Esophageal candidiasis) Viral Herpes simplex (Herpes esophagitis) Cytomegalovirus Drug-induced esophagitis Damage to the esophagus due to medications. If the esophagus is not coated or if the medicine is not taken with enough liquid, it can damage the tissues. Eosinophilic esophagitis"}, {"id": "wiki20220301en087_9085", "title": "Eosinophilic esophagitis", "score": 0.0114467503121841, "content": "Allergy assessment A thorough personal and family history of other atopic conditions is recommended in all patients with EoE. Testing for allergic sensitization may be considered using skin prick testing or blood testing for allergen-specific IgE. This is particularly important for the 10–20% of EoE patients who also have symptoms of immediate IgE-mediated food allergy. Atopy patch testing has been used in some cases for the potential identification of delayed, non-IgE (cell-mediated) reactions. Diagnostic criteria The diagnosis of eosinophilic esophagitis requires all of the following: Symptoms related to esophageal dysfunction. Eosinophil-predominant inflammation on esophageal biopsy, characteristically consisting of a peak value of ≥15 eosinophils per high power field (HPF). Exclusion of other causes that may be responsible for symptoms and esophageal eosinophilia. Treatment"}, {"id": "InternalMed_Harrison_2996", "title": "InternalMed_Harrison", "score": 0.011236245954692557, "content": "is progressive over the course of weeks to months raises concern for neoplasia. Episodic dysphagia to solids that is unchanged over years indicates a benign disease process such as a Schatzki’s ring or eosinophilic esophagitis. Food impaction with a prolonged inability to pass an ingested bolus even with ingestion of liquid is typical of a structural dysphagia. Chest pain frequently accompanies dysphagia whether it is related to motor disorders, structural disorders, or reflux disease. A prolonged history of heartburn preceding the onset of dysphagia is suggestive of peptic stricture and, infrequently, esophageal adenocarcinoma. A history of prolonged nasogastric intubation, esophageal or head and neck surgery, ingestion of caustic to neck, nasal regurgitation, aspiration, neck, food impaction"}, {"id": "pubmed23n0757_25290", "title": "[Eosinophilic esophagitis: an underevaluated condition. Our experience].", "score": 0.010976158344579396, "content": "The aim of this review is to present our experience in this emerging disease and mainly help improve diagnostic suspicion. We reviewed the literature in order to analyze the epidemiology, pathophysiology, diagnosis and management of eosinophilic esophagitis (EE), we describe 4 cases diagnosed in our hospital during 2011 after an emergency admission by food impaction. The age of our patients was 7-11 years, males in all cases. All patients had a history of allergies, and the impaction had been preceded by episodes of dysphagia. In esophagoscopy we observed nonspecific macroscopic findings. The results of biopsies showed the presence of an infiltration of eosinophils in the mucosa over 15 per high power field. All patients were referred to the gastroenterology section of our hospital. Eosinophilic esophagitis is a primary disease of esophagus, defined as the presence of symptoms of esophageal dysfunction (mainly dysphagia and food impaction), associated to at least one esophageal biopsy with more than 15 eosinophils in high-power field and the exclusion of gastroesophageal reflux. The diagnosis is clinical, endoscopic and pathologic. It requires an upper endoscopy to evaluate characteristic findings and biopsies for histology. Current treatments include diet therapy based on avoiding exposure to certain food allergens."}, {"id": "wiki20220301en100_23301", "title": "Schatzki ring", "score": 0.009900990099009901, "content": "Other associations Schatzki rings can be associated with swallow syncope, a rare variety of syncope. Schatzki rings are associated with lesser incidence of Barrett's esophagus, which is considered to be a pre-cancerous condition of the esophagus in some cases. Cause Although many hypotheses have been proffered, the cause of Schatzki rings remains uncertain; both congenital and acquired factors may be involved. Diagnosis A Schatzki ring is usually diagnosed by esophagogastroduodenoscopy or barium swallow. Endoscopy usually shows a ring within the lumen of the esophagus which can be of variable size (see picture). The ring is usually located a few centimetres above the gastro-esophageal junction, where the esophagus joins the stomach. Schatzki rings can often resemble a related entity called an esophageal web. Esophageal webs also contain extra mucosal tissue, but do not completely encircle the esophagus."}, {"id": "wiki20220301en010_102252", "title": "Esophagitis", "score": 0.009822076978939723, "content": "Damage to the esophagus due to medications. If the esophagus is not coated or if the medicine is not taken with enough liquid, it can damage the tissues. Eosinophilic esophagitis Eosinophilic esophagitis is caused by a high concentration of eosinophils in the esophagus. The presence of eosinophils in the esophagus may be due to an allergen and is often correlated with GERD. The direction of cause and effect between inflammation and acid reflux is poorly established, with recent studies (in 2016) hinting that reflux does not cause inflammation. This esophagitis can be triggered by allergies to food or to inhaled allergens. This type is still poorly understood. Lymphocytic esophagitis"}, {"id": "pubmed23n0550_15040", "title": "A Japanese case of eosinophilic esophagitis.", "score": 0.00980392156862745, "content": "Eosinophilic esophagitis (EE) is a rarely diagnosed condition involving eosinophilic infiltration of the esophageal mucosa. Here we present a case of EE in a 69-year-old Japanese man, who presented with abdominal pain, appetite loss, and a history of bronchial asthma. Laboratory findings included peripheral eosinophilia and an increased serum immunoglobulin E level. Computed tomography showed diffuse severe thickening of the esophageal wall, and a barium esophagogram revealed a small caliber of the middle and lower portion of the esophagus, without normal peristaltic contractions. Endoscopy of the esophagus showed a pale mucosa, with adherent whitish exudates resembling fungal infection, and prominent ring-like contractions. Histologic examination of a biopsy specimen revealed marked eosinophil infiltration into the esophageal mucosa. Endoscopic ultrasonography (EUS) demonstrated marked circumferential thickening of the esophageal submucosal layer, and an esophageal manometry study showed a high percentage of ineffective esophageal peristalsis and high-amplitude esophageal body contractions. EUS findings showed no change even after oral corticosteroid therapy, although the histological findings were improved. This is thought to be the first documented Japanese case of EE. EE should be considered in the differential diagnosis in cases of esophageal motility disturbance, even if the patients do not complain of dysphagia."}, {"id": "wiki20220301en100_23099", "title": "Esophageal dilatation", "score": 0.009615384615384616, "content": "Esophageal dilatation is a therapeutic endoscopic procedure that enlarges the lumen of the esophagus. Indications It can be used to treat a number of medical conditions that result in narrowing of the esophageal lumen, or decrease motility in the distal esophagus. These include the following: Peptic stricture Eosinophilic esophagitis Schatzki rings Achalasia Scleroderma esophagus Rarely esophageal cancer"}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 79]], "word_ranges": [[0, 11]], "text": "Answer 2 is false (Turner syndrome: low estradiol and elevated gonadotrophins),"}, "3": {"exist": true, "char_ranges": [[204, 263]], "word_ranges": [[29, 39]], "text": "refractive defects do not give hemianopsia (3 and 4 false)."}, "4": {"exist": true, "char_ranges": [[204, 263]], "word_ranges": [[29, 39]], "text": "refractive defects do not give hemianopsia (3 and 4 false)."}, "5": {"exist": true, "char_ranges": [[80, 177]], "word_ranges": [[11, 24]], "text": "brain tumors affecting the hypothalamus-pituitary gland do not give low gonadotrophins (5 false),"}}, "full_answer": "Answer 2 is false (Turner syndrome: low estradiol and elevated gonadotrophins), brain tumors affecting the hypothalamus-pituitary gland do not give low gonadotrophins (5 false), and it seems obvious that refractive defects do not give hemianopsia (3 and 4 false).", "full_answer_no_ref": "Answer 2 is [HIDDEN] (Turner syndrome: low estradiol and elevated gonadotrophins), brain tumors affecting the hypothalamus-pituitary gland do not give low gonadotrophins ([HIDDEN]), and it seems obvious that refractive defects do not give hemianopsia ([HIDDEN]).", "full_question": "14-year-old girl who consults for decreased growth for 2-3 years previously normal (provides data) and that other girls her age have greater physical and sexual development. Lately she has had headaches and visual problems that she notices in class and when studying. She has not had menarche or polydipsia or polyuria. Parents with normal height. Examination: short stature at -2.1 standard deviations, normal body proportions, little pubic hair and breast development. Campimetry shows left temporal partial hemianopsia. Bone age: delay of 2 years. General laboratory tests were normal. Gonadotrophins (FSH and LH) and estradiol are low. What do you think is the most appropriate response?", "id": 56, "lang": "en", "options": {"1": "Decreased growth and sexual development, delayed bone age, headache and visual alteration suggest hormonal deficit and involvement of the optic chiasm.", "2": "As she is a girl of pubertal age, it is most likely that her decreased growth and sexual retardation are due to Turner syndrome.", "3": "She must not have a hypothalamic tumor because of the absence of polyuria and polydipsia. She probably has constitutional delay and her visual problem is refractive.", "4": "A growth hormone deficiency may explain the developmental delay and low estradiol. To evaluate if she needs glasses, due to her headaches and visual disturbances.", "5": "She could have a craniopharyngioma, but it would be rare if she had not shown symptoms before. Also, it would not justify low gonadotrophins and estradiol."}, "question_id_specific": 76, "type": "ENDOCRINOLOGY", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1132_12218", "title": "Different Cases of Short Stature.", "score": 0.016078164615670028, "content": "This case sieries gives three different cases with three different presentation and different approaches to a patient with short stature. Material: CASE 1: A 13 Year old female brought by her parents with complaints of decreased height appropriate for age. O/E height- 127cm(&amp;lt;-3SD), weight 42kg, MPH- 150cm, arm span to height ratio- 1:1, secondary sexual characters- tanners stage 2. X-ray hand with wrist- bone age 0f 12 years. TFT- within normal limits. MRI pituitary- normal study. Patient suspected to have isolated growth harmone deficiency. Growth harmone stimulation test done after sex steroid priming which showed results of growth harmone deficiency. Patient started on 1.5mg growth harmone s/c per day. CASE 2: A 18 year old boy brought by his parents with complaints of decreased height appropriate for age and absence of facial, axillary and pubic hair. O/E height- 145cm, weight 55kg, MPH- 165cm, arm span to height ratio 1.1:1, external genitalia- male, testes in scrotum, testicular volume- 2.5ml, secondary sexual characters- tanners stage 1. Xray hand with wrist- bone age of 17years. TFT within normal limits. FSH, LH, testosterone levels were low. Growth harmone stimulation test after sex steroid priming showed low growth harmone levels. MRI pituitary showed empty sella. Patient diagnosed to have panhypopititarism and started on inj. Growth harmone 0.24mg/kg/week subcutaneous and inj.testosterone 100mg/month IM. CASE 3: A 17 year old female brought by her parents with complaints of decreased height appropriate for age and absent menstruation till date. O/E height- 143cm(&amp;lt;- 3SD), weight- 45 kg, MPH- 170cm, arm span to height ratio 1.1:1, external genitalia - female, secondary sexual characters - tanners stage 1. Xray hand with wrist - bone age of 16years. Further evaluation showed TSH-21.8. MRI pelvis showed streak gonads. Serum growth harmone and IGF levels within normal limits. Estradiol was low and FSH, LH were high. Karyotyping showed 45XO. Patient diagnosed to have turner's syndrome. Since x-ray showed non-closure of epiphysis, patient started on growth harmone 0.33 mg/kg/week and advised not to take estrogen till epiphyseal closure. Observation: case 1: functonal pituitary pathology Case 2: structural pituitary pathology Case 3: gonadal pathology Conclusion: Short stature can be a manifestation of isolated GH defeciency, Hypogonadotropic Hypogonadism or Hypergonadotropic hypergonadism."}, {"id": "pubmed23n0739_21576", "title": "Precocious puberty in Turner Syndrome: report of a case and review of the literature.", "score": 0.014658290500666285, "content": "Turner Syndrome (TS) is caused by monosomy or structural abnormalities of the X chromosome, with a prevalence of about 1/2000 females live birth. Most important clinical features of TS are short stature and gonadal failure. Approximately one third of girls with TS may undergo spontaneous puberty. Here we report on the case of a girl with a rare 45X0/47XXX mosaic TS exhibiting a precocious puberty. The patient was diagnosed with TS at the age of 4 years, upon a diagnostic work-up for dysmorphic features. Chromosome analysis revealed a mosaic karyotype (45X0/47XXX). She presented with normal height and normal growth velocity so that Growth Hormone (GH) therapy was not started. She was referred to our Department at the age of 7 years and 10 months, because of vaginal bleeding. A physical examination revealed a Tanner stage III for breast and Tanner stage III for pubic hair development. Height and weight were within the normal range for age. Psychological evaluation showed moderate global developmental delay, together with emotional and social immaturity and reading difficulties. The growth rate was accelerated. Her bone age was 10 years. Pelvic ultrasound demonstrated increased size for age of both the uterus and the ovaries, with bilateral ovarian follicles. GnRH stimulation test revealed pubertal response of gonadotropins (peak LH 22.5 mIU/ml). MRI of the brain was normal. These clinical, radiologic and laboratory findings were consistent with a diagnosis of idiopathic central precocious puberty; therefore, GnRH analog therapy was started, in order to slow pubertal progression and to preserve adult stature. Furthermore, GH treatment was added to further improve adult height. Our case highlights the possibility of precocious puberty as an atypical clinical feature of TS. Thus, precocious puberty may occur in TS girls when a dosage compensation by the cell line with more than two X chromosomes allows normal ovarian function. GnRH analog therapy in addition to GH treatment should be recommended in TS girls with precocious puberty in order to slow pubertal progression and to preserve adult stature."}, {"id": "Gynecology_Novak_5163", "title": "Gynecology_Novak", "score": 0.014656931171480727, "content": "Figure 29.13 Right A: A 16-year-old girl with delayed puberty. Breast budding began at 11 years of age, but there was no further development. During the year before presentation, her scholastic performance in school deteriorated, she gained 25 lb, she became increasingly lethargic, and nocturia and polydypsia were noted. Initial evaluation documented low follicle-stimulating hormone, elevated prolactin, and a bone age of 10.5 years. Computed tomography scanning documented a large hypothalamic neoplasm that proved to be an ectopic germinoma. The patient was also documented to be hypothyroid and hypoadrenal and to have diabetes insipidus. Despite the elevated prolactin, she had no galactorrhea because of the minimal breast development. (From Rebar RW. Normal and abnormal sexual differentiation and pubertal development. In: Moore TR, Reiter RC, Rebar RW, et al., eds. Gynecology and obstetrics: a longitudinal approach. New York: Churchill Livingstone, 1993:97–133, with permission.)"}, {"id": "pubmed23n0594_18701", "title": "Precocious puberty in a girl with floating-harbor syndrome.", "score": 0.013696831787152109, "content": "Floating-Harbor syndrome (FHS) is a rare genetic disorder characterized by short stature, delayed bone age, mild to moderate mental retardation, speech problems, and peculiar craniofacial features. In these patients pubertal development has been reported to be normal. In this paper, we describe a girl with FHS who developed precocious puberty. FHS diagnosis was made at 2 years 5 months on the basis of peculiar clinical features. At 7 years 7 months, the girl began pubertal development; her height was 112.5 cm (-2.42 SDS) and pubertal staging was B2 PH2 AH1. LHRH test underlined LH and FSH peak values of 11.7 mIU/ml and 6.2 mIU/ml, respectively. Plasma levels of 17beta-estradiol were normal (8.5 pg/ml). Ophthalmological and neurological examinations, including nuclear magnetic resonance imaging of the brain, were normal. Treatment with gonadotrophin-releasing hormone analogue was begun. At 10 years 1 month, because of reduced height velocity, her growth hormone secretion was evaluated with diagnosis of neurosecretory dysfunction; hGH therapy was begun. The patient showed a good response to hGH treatment, reaching a normal adult height (156.1 cm; -1.20 SDS). This report suggests that, in patients with FHS, precocious puberty should be taken into consideration; in these patients, a careful endocrinological followup for the possible presence of growth and pubertal disorders is needed."}, {"id": "wiki20220301en024_54492", "title": "Delayed puberty", "score": 0.013172541743970315, "content": "Initial workup for delayed puberty not due to a chronic condition involves measuring serum FSH, LH, testosterone/estradiol, as well as bone age radiography. If it becomes clear that there is a permanent defect of the reproductive system, treatment usually involves replacement of the appropriate hormones (testosterone/dihydrotestosterone for boys, estradiol and progesterone for girls). Timing and definitions Puberty is considered delayed when the child has not begun puberty when two standard deviations or about 95% of children from similar backgrounds have. In North American girls, puberty is considered delayed when breast development has not begun by age 13, when they have not started menstruating by age 15, and when there is no increased growth rate. Furthermore, slowed progression through the Tanner scale or lack of menarche within 3 years of breast development may also be considered delayed puberty."}, {"id": "wiki20220301en101_24439", "title": "Adiposogenital dystrophy", "score": 0.012318029115341545, "content": "Adiposogenital dystrophy is a condition that may be caused by tertiary hypogonadism originating from decreased levels in GnRH. Low levels of GnRH has been associated with defects of the feeding centers of the hypothalamus, leading to an increased consumption of food and thus caloric intake. Presentation It is characterized by: Obesity Growth delays and delayed sexual development, atrophy or hypoplasia of the gonads, and altered secondary sex characteristics, Headaches Problems with vision polyuria, polydipsia. It is usually associated with tumors of the hypothalamus, causing increased appetite and depressed secretion of gonadotropin. It seems to affect males mostly.Many overweight children may appear to have the disorder because of the concurrence of obesity and retarded sexual development; these children have no endocrine disturbances, however, and they mature normally after delayed puberty."}, {"id": "wiki20220301en347_35378", "title": "Compensatory growth (organism)", "score": 0.010899256993006992, "content": "Anorexia nervosa can have serious implications if its duration and severity are significant and if onset occurs before the completion of growth, pubertal maturation or prior to attaining peak bone mass. Both height gain and pubertal development are dependent on the release of growth hormone and gonadotrophins (LH and FSH) from the pituitary gland. Suppression of gonadotropins in patients with anorexia nervosa has been frequently documented. In some cases, especially where onset is pre-pubertal, physical consequences such as stunted growth and pubertal delay are usually fully reversible. Height potential is normally preserved if the duration and severity of anorexia nervosa are not significant and/or if the illness is accompanied with delayed bone age (especially prior to a bone age of approximately 15 years), as hypogonadism may negate the deleterious effects of undernutrition on stature by allowing for a longer duration of growth compared to controls. In such cases, appropriate early"}, {"id": "wiki20220301en344_26256", "title": "Signs and symptoms of Graves' disease", "score": 0.0101008295194508, "content": "Children and adolescents Hyperthyroidism has unique effects in children on growth and pubertal development, e.g. causing epiphyseal maturation. In growing children, accelerated bone growth from hyperthyroidism can increase osteogenesis in the short term, but generally results in short-stature adults compared with the predicted heights. Pubertal development tends to be delayed, or slowed. Girls who have undergone menarche may develop secondary amenorrhea. Hyperthyroidism is associated with high sex hormone-binding globulin (SHBG), which may result in high serum estradiol levels in girls and testosterone levels in boys. However, unbound or free levels of these hormones are decreased. Hyperthyroidism before the age of four may cause neurodevelopmental delay. A study by Segni et al. suggests that permanent brain damage can occur as a result of the illness."}, {"id": "pubmed23n0876_17161", "title": "Trichotillomania: Bizzare Patern of Hair Loss at 11-Year-old Girl.", "score": 0.009900990099009901, "content": "Trichotillomania (TTM) is defined by the Diagnostics and Statistic Manual of Mental Disorders, 4th edition (DMS-IV) as hair loss from a patient`s repetitive self-pulling of hair. The disorder is included under anxiety disorders because it shares some obsessive-compulsive features. Patients have the tendency towards feelings of unattractiveness, body dissatisfaction, and low self-esteem (1,2). It is a major psychiatric problem, but many patients with this disorder first present to a dermatologist. An 11-year-old girl came to our department with a 2-month history of diffuse hair loss on the frontoparietal and parietotemporal area (Figure 1). She had originally been examined by a pediatrician with the diagnosis of alopecia areata. The patient`s personal history included hay fever and shortsightedness, and she suffered from varicella and mononucleosis. Nobody in the family history suffered from alopecia areata, but her father has male androgenetic alopecia (Norwood/Hamilton MAGA C3F3). The mother noticed that the child had had changeable mood for about 2 months and did not want to communicate with other persons in the family. The family did not have any pet at home. At school, her favorite subjects were Math and Computer Studies. She did not like Physical Education and did not participate in any sport activities during her free time. This was very strange because she was obese (body-mass index (BMI) 24.69). She was sometimes angry with her 13-year-old sister who had better results at school. The girl had suddenly started to wear a blue scarf. The parents did not notice that she pulled out her hair at home. Dermatological examination of the capillitium found a zone of incomplete alopecia in the frontoparietal and parietotemporal area, without inflammation, desquamation, and scaring. Hairs were of variable length (Figure 1). There was a patch of incomplete alopecia above the forehead between two stripes of hair of variable length (Figure 2). The hair pull test was negative along the edges of the alopecia. Mycological examination from the skin capillitium was negative. The trichoscopy and skin biopsy of the parietotemporal region of the capillitium (Figure 3) confirmed trichotillomania. Laboratory tests (blood count, iron, ferritin, transferrin, selenium, zinc, vitamin B12, folic acid, serology and hormones of thyroid gland) were negative. We referred the girl for ophthalmologic and psychological examination. Ophthalmologic examination proved that there was no need to add any more diopters. The psychological examination provided us with a picture in which she drew her family (Figure 4). The strongest authority in the family was the mother because she looked after the girls for most of the day. She was in the first place in the picture. The father had longer working hours and spent more time outside the home. He worked as a long vehicle driver. He was in the second place in the picture. There was sibling rivalry between the girls, but the parents did not notice this problem and preferred the older daughter. She was successful at school and was prettier (slim, higher, curly brown hair, without spectacles). Our 11-years-old patient noticed all these differences between them, but at her level of mental development was not able to cope with this problem. She wanted to be her sister's equal. The sister is drawn in the picture in the third place next to father, while the patient's own figure was drawn larger and slim even though she was obese. Notably, all three female figures had very nice long brown hair. It seemed that the mother and our patient had better quality of hair and more intense color than the sister in the drawing. The only hairless person in the picture was the father. The girl did not want to talk about her problems and feelings at home. Then it was confirmed that our patient was very sensitive, anxious, willful, and withdrawn. She was interested in her body and very perceptive of her physical appearance. From the psychological point of view, the parents started to pay more interest to their younger daughter and tried to understand and help her. After consultation with the psychiatrist, we did not start psychopharmacologic therapy for trichotillomania; instead, we started treatment with cognitive behavioral therapy, mild shampoo, mild topical steroids (e.g. hydrocortisone butyrate 0.1%) in solution and methionine in capsules. With parents' cooperation, the treatment was successful. The name trichotillomania was first employed by the French dermatologist Francois Henri Hallopeau in 1889, who described a young man pulling his hair out in tufts (3-5). The word is derived from the Greek thrix (hair), tillein (to pull), and mania (madness) (5). The prevalence of TTM in the general adult population ranges from 0.6% to 4%, and 2-4% of the general psychiatric outpatient population meet the criteria for TTM (2-5). The prevalence among children and adolescents has been estimated at less than 1% (5). The disease can occur at any age and in any sex. The age of onset of hair pulling is significantly later for men than for women (3). There are three subsets of age: preschool children, preadolescents to young adults, and adults. The mean age of onset is pre-pubertal. It ranges from 8 to 13 years (on average 11.3 years) (2-5). The occurrence of hair-pulling in the first year of life is a rare event, probably comprising &lt;1% of cases (5). The etiology of TTM is complex and may be triggered by a psychosocial stressor within the family, such as separation from an attachment figure, hospitalization of the child or parent, birth of a younger sibling, sibling rivalry, moving to a new house, or problems with school performance. It has been hypothesized that the habit may begin with \"playing\" with the hair, with later chronic pulling resulting in obvious hair loss (2). Environment is a factor because children usually pull their hair when alone and in relaxed surroundings. The bedroom, bathroom, or family room are \"high-risk\" situations for hair-pulling (5). Men and women also differed in terms of the hair pulling site (men pull hair from the stomach/back and the moustache/beard areas, while women pull from the scalp) (3). Pulling hair from siblings, pets, dolls, and stuffed animals has also been documented, often occurring in the same pattern as in the patient (5). Genetic factors contributing to the development of TTM are mutations of the SLITRK1 gene, which plays a role in cortex development and neuronal growth. The protein SAPAP3 has been present in 4.2% of TTM cases and patients with obsessive-compulsive disorder (OCD). It may be involved in the development of the spectrum of OCD. A significantly different concordance rate for TTM was found in monozygotic (38.1%) compared with dizygotic (0%) twins in 34 pairs (3). The core diagnostic feature is the repetitive pulling of hairs from one`s own body, resulting in hair loss. The targeted hair is mostly on the scalp (75%), but may also be from the eyebrows (42%), eyelashes (53%), beard (10%), and pubic area (17%) (3,5). There are three subtypes of hair pulling - early onset, automatic, and focused. Diagnostic criteria for TTM according to DSM-IV criteria are (2,3,5): 1) recurrent pulling of one`s hair resulting in noticeable hair loss; 2) an increasing sense of tension immediately prior to pulling out the hair or when attempting to resist the behavior; 3) pleasure, gratification, or relief when pulling out the hair; 4) the disturbance is not better accounted for by another mental disorder and is not due to a general medical condition (e.g., a dermatologic condition); 5) the disturbance causes clinically significant distress or impairment in social, occupational, or other important areas of functioning. The differential diagnosis includes alopecia areata (Table 1) (6), tinea capitis, telogen effluvium, secondary syphilis, traction alopecia, loose anagen syndrome, lichen planopilaris, alopecia mucinosa, and scleroderma (2-5). Biopsy of an involved area (ideally from a recent site of hair loss) can help to confirm the diagnosis (5). On histologic examination, there are typically increased numbers of catagen and telogen hairs without evidence of inflammation. Chronic hair pulling induces a catagen phase, and more hairs will be telogen hairs. Pigment casts and empty anagen follicles are often seen. Perifollicular hemorrhage near the hair bulb is an indicator of TTM (2). Complications of TTM are rare, but they comprise secondary bacterial infections with regional lymphadenopathy as a result of picking and scratching at the scalp. Many patients play with and ingest the pulled hairs (e.g. touching the hair to lips, biting, and chewing). Trichophagia (ingestion of the hair) can lead to a rare complication named trichobezoar (a \"hair ball\" in stomach). This habit is present in approximately 5% to 30% of adult patients, but it is less frequent in children. Patient with trichophagia present with pallor, nausea, vomiting, anorexia, and weight loss. Radiologic examination and gastroscopy should not be delayed (2,4,5). The management of the disease is difficult and requires strong cooperation between the physician, patient, and parents. The dermatologist cannot take part in the therapy, strictly speaking, but without the psychological, psychopharmacologic, and topic dermatologic treatment a vicious circle will be perpetuated. "}, {"id": "pubmed23n0349_5715", "title": "Idiopathic growth hormone deficiency: presentation, diagnostic and treatment during childhood.", "score": 0.009900990099009901, "content": "The clinical and biological presentation of idiopathic growth hormone (GH) deficiency (GHD) varies greatly, demonstrating the variety of its pathogenic features and explaining why it is difficult to diagnose. We examined 48 patients (26 males) with certain idiopathic GHD diagnosed at 4.8 +/- 0.7 yr. The symptoms that led to the diagnosis of GHD were low growth rate (33 cases), hypoglycemia (12 cases), microphallus (1 case) and in 2 cases the GHD was diagnosed from magnetic resonance imaging (MRI) performed for delayed mental development (1 case), or congenital blindness (1 case). The 2 other cases were diagnosed from routine GH evaluation performed at birth because of idiopathic GHD in siblings. Thirteen had congenital malformation. Twenty three cases (48%) had features suggesting that the GHD was of antenatal origin. Six of them were born by breech delivery. Twenty one cases (44%) had features suggesting a hypothalamic origin. The decrease in growth rate occurred before 0.5 year in 21 (55%), before 1 year in 27 (71%) and before 2 years in 30 (79%): 8 patients (21%) maintained a normal growth rate after this age. Among these 8 patients, 5 had signs suggesting an antenatal origin and 4 had severe episodes of hypoglycemia from birth. The mean GH peak after the pharmacological stimulation test was 3.6 +/- 0.5 micrograms/l. The mean plasma insulin-like growth factor 1 (IGFI) was 0.1 +/- 0.02 U/ml. The GH deficiency was associated with deficiencies of thyrotropin in 26 (54%) and of adrenocorticotrophic hormone in 17 (35%) patients. Among the 15 patients of pubertal age, 9 (60%) had gonadotrophin deficiency. No patient had diabetes insipidus. The MRI showed pituitary stalk interruption syndrome in 39 patients and normal pituitary anatomy in 6 patients. GH treatment reduced the difference between target and actual heights from 3.5 SD (before) to I SD (after 3 years) in the 39 more recently seen patients given 0.5-0.6 U/kg/w GH in 6 or 7 weekly injections. Height gain during the first year and cumulative height gain over 3 years (SD) was correlated negatively with height (SD) at the start of treatment (p &lt; 0.01). We conclude that most of the patients with GHD have features suggesting an antenatal origin. Despite this early origin, the decreased growth rate may occur after 2 years."}, {"id": "pubmed23n0088_8676", "title": "[Final body height and puberty in idiopathic hypopituitarism].", "score": 0.00980392156862745, "content": "Twenty four children with hypopituitarism were treated with growth hormone from 6-15 years of age until cessation of growth. The height deficit decreased from -4.2SD to -2.2SD. Final height was above the 3rd percentile in half of the patients. There was no difference in final height between patients with and without additional gonadotropin deficiency. Spontaneous puberty started late but at a normal bone age and its course was normal. In girls with gonadotropin deficiency low dose oestrogen given at unchanged growth hormone doses did not accelerate growth. Final height was closely correlated with the degree of growth retardation at the beginning of treatment (r = 0.73, p less than 0.001). In 17 of the 79 patients treated with growth hormone compliance was bad. The main cause of bad compliance was the low educational level of the parents. It is concluded that final height in growth hormone deficiency can be increased with earlier diagnosis and improved compliance."}, {"id": "pubmed23n0047_2561", "title": "[Suprasellar arachnoid cyst associated with precocious puberty: report of an operated case and review of the literature].", "score": 0.009708737864077669, "content": "The pathogenesis remains unknown in the majority of patients with precocious puberty, and yet infrequently such causative cerebral lesions as hypothalamic hamartomas are associated with sexual precocity. We reported a rare case of suprasellar arachnoid cyst in an infant presenting with precocious puberty, which eventually disappeared after a cyst-peritoneal shunt. It was believed that the mass effect of the arachnoid cyst upon the hypothalamus was, at least in part, responsible for development of precocious puberty. The role of surgical decompression of the cyst was also discussed. A one-year-old girl was admitted to the hospital for evaluation of genital bleeding which had persisted on and off for two months. The height, 80cm, and the weight, 12.4kg, exceeded by far the two standard deviations from the mean level of the normal population. In addition she had the development of breast tissue as classified Tanner's Stage II, and both pubic and axillary hair. The bone age by skeletal survey of the hand was rated as 3 years. Endocrinological examination showed that serum levels of luteinizing hormone (LH), follicle-stimulating hormone (FSH) and estradiol had increased for her age, to levels equivalent to those for females at puberty. An LH-RH test revealed an excessive LH reaction. There were no definite neurological deficits. CT and MRI demonstrated the presence of a large arachnoid cyst involving the suprasellar region as well as the right middle and posterior fossa. After the patient underwent a cyst-peritoneal shunt, the cyst decreased in size and such symptoms as genital bleeding and breast growth disappeared. Serum levels of her LH and FSH also significantly decreased.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0381_8298", "title": "Severe hypothyroidism due to autoimmune atrophic thyroiditis--predicted target height and a plausible mechanism for sexual precocity.", "score": 0.009615384615384616, "content": "Autoimmune thyroiditis, the most frequent cause of acquired hypothyroidism in childhood and adolescents, is characterized by raised levels of the specific antibodies to thyroperoxidase (TPOAb) and thyroglobulin (TgAb). We report a girl aged 10 years and 9 months who presented with arrested growth and breast development (thelarche). She also exhibited myxedema of the face and legs, prominent striae on the thighs, dry, cold skin, and hypertrichosis on her back. There was no goiter, no history of thyroid pain and no family history of thyroid disease. She complained occasionally of a transient headache. The patient's height was below the 3rd percentile, while her body weight was at the 50th percentile and bone age was normal. Laboratory tests proved severe hypothyroidism (fT4 0 ng/dl, fT3 0.99 pg/ml, TSH &gt;100 microIU/ml plus an increased titer of TPOAb). Thyroid ultrasound supported the diagnosis of thyroiditis. Pituitary PRL and FSH levels and peripheral estradiol were all elevated. L-Thyroxine therapy, instituted following diagnosis, improved the growth velocity to 11 cm/year and the FSH and E2 levels were normalized to prepubertal values. Complete regression of the breast development was observed within 4 months. However, 4 months later a true (central), isosexual LHRH-dependent puberty started. The pubertal features at the time of the original diagnosis might be explained by: 1. the direct action of elevated TRH on gonadotropes to stimulate gonadotropin secretion and on lactotrophes to stimulate PRL secretion, and 2. TSH action on LH and mostly FSH receptors (homologous to TSH receptors) in the ovary, stimulating the secretion of estradiol."}, {"id": "pubmed23n0243_2715", "title": "Growth hormone treatment in children with craniopharyngioma: final growth status.", "score": 0.009615384615384616, "content": "Twenty-seven out of thirty craniopharyngioma patients treated with human growth hormone (hGH) for 2 years or more (average 4.5 years) reached final adult heights above the population third centile, though none was above the fiftieth centile. However, only twelve of twenty-eight patients had final heights above the lower limits to be expected from their parents' heights. All patient eventually had long legs relative to sitting height (final mean subischial leg length SDS = + 0.2, final mean sitting height SDS = -3.0). Twenty-nine patients were TSH-deficient, twenty-two were ACTH-deficient, thirteen were deficient in ADH and all had total (85%) or partial (15%) gonadotrophin deficiency. Following the administration of testosterone or hCG the boys had, on average, only half the normal adolescent growth spurt. This may have been due to the lateness of starting androgens in these patients and we recommend, when considering height, that testosterone or hCG should be started when a bone age of 13.0 \"years' is reached or when a lower bone age has remained unchanged for a year. The girls showed adolescent height spurt; the average increase after oestrogen treatment commended was 1.7 cm."}, {"id": "pubmed23n0496_3061", "title": "[A case of severe mental retardation with blepharophimosis, ptosis, microphthalmia, microcephalus, hypogonadism and short stature--the difference from Ohdo blepharophimosis syndrome].", "score": 0.009523809523809525, "content": "We report a case of 13-year-old girl with short stature, microcephalus, blepharophimosis, ptosis, bilateral microphthalmia (more prominent in the right), hypogonadism, other minor anomalies, and severe mental retardation. Her mother had two spontaneous abortions. She was born as the second baby of dizygotic twins. The first baby died of diaphragm hernia and heart failure. Her body height, body weight and head circumference were below -3 SD. She did not have epicanthus inversus, hypoplastic teeth, heart anomalies, seizures, muscle weakness, and hearing loss. She was able to handle her wheelchair, but could neither understand nor speak meaningful words. When she looked at something in front of herself, she turned her face up and lifted the left eyelid with her own fingers. She had no somatic change of puberty. Laboratory and radiological examinations demonstrated a normal karyotype, normal bone age, findings of Chilaiditi syndrome, and absence of brain malformation on cranial CT. The serum levels of LH and FSH were high for age and those of estradiol and progesterone were low, suggesting immaturity of ovarian function. These findings suggested the ovarian functions might not get maturations. Hypogonadism has previously been reported in female cases of the blepharophimosis, ptosis and epicanthus inversus syndrome (BPES) type I, but not in those with the Ohdo blepharophimosis syndrome (OBS). Our case's condition differs from BPES because of the presence of mental retardation and the absence of epicanthus inversus. We also discuss the distinction from OBS, a disease entity of unknown etiology presenting with a variety of complications."}, {"id": "pubmed23n0006_6511", "title": "Delayed adolescence.", "score": 0.009523809523809525, "content": "Delayed adolescence has several causes. Most frequent is the physiological or constitutional (hereditary) delay of growth and adolescence. This is a normal variation of growth and development, with growth, bone age and puberty retarded in a harmonious way. It carries a good prognosis with late but normal puberty and late catch-up growth leading to normal adult height. It manifests itself long before puberty by short stature and retarded bone age. True endocrine defects with permanent hypogonadism (hypothalamic-pituitary deficiency of the gonadotropinds and primary gonadal failure) are rare. Differential diagnosis before puberty is not always possible on clinical grounds alone. The most useful laboratory test consists of the LH-RH test. The i.v. injection of the recently introduced hypothalamic LH-releasing hormone, LH-RH, is followed by an age-dependent increase of the plasma gonadotropinds LH and FSH. This test allows differentiation, before puberty, between constitutional delay of growth and adolescence with a normal response for bone age, true hypothalamic-pituitary insufficiency with no response, and primary gonadal failure with an increased response. True hypogonadism requires permanent sex hormone replacement therapy. Constitutional delay of growth and adolescence in boys may present a psychosocial indication for temporary hormone therapy with testosterone."}, {"id": "wiki20220301en023_68542", "title": "Growth hormone deficiency", "score": 0.00948058950395399, "content": "Severe GH deficiency in childhood additionally has the following measurable characteristics: Proportional stature well below that expected for family heights, although this characteristic may not be present in the case of familial-linked GH deficiency Below-normal velocity of growth Delayed physical maturation Delayed bone age Low levels of IGF1, IGF2, IGF binding protein 3 Increased growth velocity after a few months of GH treatment In childhood and adulthood, the diagnosing doctor will look for these features accompanied by corroboratory evidence of hypopituitarism such as deficiency of other pituitary hormones, a structurally abnormal pituitary, or a history of damage to the pituitary. This would confirm the diagnosis; in the absence of pituitary pathology, further testing would be required."}, {"id": "pubmed23n0423_22902", "title": "Association of Turner's syndrome and hypopituitarism: a patient report.", "score": 0.009433962264150943, "content": "Turner's syndrome (TS) is associated with a wide spectrum of clinical features, such as short stature and gonadal dysgenesis. While it is a common chromosomal abnormality, the association of Turner's syndrome and hypopituitarism is an uncommon finding. We describe here a girl with concomitant pituitary insufficiency and gonadal dysgenesis. When she was 7 years old, her mother reported that she suffered from frontal headache, asthenia and delayed growth. Basal laboratory thyroid evaluation suggested hypothyroidism, with no evidence of autoimmune disease association. She began taking L-thyroxine. At age 11 years, short stature and complaints of frontal headache still persisted. She was still prepubertal and her bone age was delayed by 2.2 years. Her karyotype was compatible with 45,X/46,XX (100 cells analyzed by FISH) and a CT scan showed empty sella. At 12 years of age, an anterior pituitary stimulation test with insulin, gonadotropin-releasing hormone (GnRH) and thyrotropin-releasing hormone (TRH) showed gonadotropin, thyrotropin (TSH) and growth hormone (GH) deficiency. Replacement therapy with GH was begun and she grew 12 cm during the first year of treatment. This report illustrates that, despite the high incidence of sinusitis, short stature and primary hypothyroidism in TS, we should consider the presence of hypopituitarism when the patient presents low levels of TSH with negative thyroid antibodies and inappropriately low levels of gonadotropins for patients with gonadal dysgenesis."}, {"id": "pubmed23n0051_753", "title": "Diagnosis of growth hormone deficiency.", "score": 0.009433962264150943, "content": "Many ways of evaluating the physiological state of hGH secretion exist, some of which have been touched upon and none of which has as yet proven infallible. Apart from important clinical features like history, physical data and growth rate, the diagnosis of altered pituitary function is based on tests and their interpretation. The physician responsible has to be informed on their effectiveness and pitfalls. Results should be interpreted in relation to developmental age (bone age) rather than chronological age. Research is under way to try to facilitate the diagnosis of varying degrees of alterations of hGH secretion. Reliability in predicting the effect of therapy with hGH is the ultimate aim in order to prevent unnecessary cost and disappointment for the patients. With the help of doctors involved in child care, such as physicians at kindergarten or school, it should be possible to start the slow process of investigating growth disorders at an early age."}, {"id": "pubmed23n1146_16888", "title": "Case 308.", "score": 0.009345794392523364, "content": "An 11-year-old girl presented to the pediatric gastroenterology outpatient department of our institution with gradually increasing painless abdominal distention. The distention started 2 years earlier and was not associated with any other constitutional symptoms, vomiting, diarrhea, jaundice, hematemesis, or melaena. She reported early satiety and heaviness in the lower abdomen. The abdominal swelling was predominantly in the infraumbilical region and was soft at palpation. She was the first child of nonconsanguineous parents and had an uneventful perinatal course after a normal vaginal delivery. Her developmental milestones were normal. She had an average scholastic performance at school. There was no history of visual problems, seizures, or inappropriate behaviors. She had an early menarche 2 years previously. Her menstrual cycles were regular, and there was no abnormal vaginal discharge. Her breast development was normal (Tanner stage III), while pubic and axillary hair were absent (Tanner stage I). She was short for her age (104 cm; normal range, 120-154 cm). There was no history of short stature among her siblings or parents. Laboratory investigations were performed to measure thyroid-stimulating hormone (1354.34 μIU/mL; normal range, 0.35-5.5 μIU/mL), triiodothyronine (&lt;2.5 ng/dL [0.0385 pmol/L]; normal range, 100-200 ng/dL [1.54-3.08 pmol/L]), thyroxine (1.35 μg/dL [17.37 nmol/L]; normal range, 5-12 μg/dL [64.35-154.44 nmol/L]), β-human chorionic gonadotropin (&lt;1.2 mIU/mL; normal, &lt;5 mIU/mL), luteinizing hormone (0.08 mIU/mL; normal range, 0.1-6.0 mIU/mL), and follicle-stimulating hormone (6.93 mIU/mL; normal range, 0.3-2.0 mIU/mL) levels. Complete blood count was normal. An abdominal mass was suspected, and abdominopelvic CT was performed and followed by US; these examinations revealed multiple large cysts in both ovaries (Figs 1, 2A, 2B). The uterus was pubertal in shape, and endometrial thickness was 9 mm, representing normal follicular phase measurement. Serum CA-125 and inhibin levels were normal. To evaluate short stature, radiographs of the hand (Fig 3) and pelvis (Fig 3B) were obtained as part of a limited skeletal survey, keeping in mind the possible skeletal changes associated with hypothyroidism. In view of the hypothyroidism, US of neck was also performed (Fig 4). Treatment was started based on the clinical and radiologic parameters, and the child's condition improved with medical treatment."}, {"id": "pubmed23n0360_16324", "title": "Growth hormone deficiency as the only identifiable cause for primary amenorrhea", "score": 0.009345794392523364, "content": "Background: There is much evidence that growth hormone plays an important role in the development and function of the reproductive system of both males and females. Growth hormone exerts its effects on the ovarian follicular cycle directly or by local production of insulin-like growth factor 1 (IGF-1). It is known that growth hormone deficiency during childhood may delay pubertal development, but there is limited data about primary amenorrhea in GH-deficient girls with sufficient stimulated gonadotropin levels.Methods: Case series.Results: In the evaluation of primary amenorrhea and delayed puberty, 3 cases of adolescent females aged 17-19 years were identified as isolated GH-deficiency. Among the 3 patients, 2 had history of intracranial surgery due to hydrocephalus (shunt operation) or prolactin-secreting pituitary macro-adenoma (transphenoidal surgery, one year before). 17-year-old patient with shunted hydrocephalus and 19-year-old patient with primary amenorrhea showed short statue (&lt; 5%) and delayed bone maturation. The patient undertaken transphenoidal surgery for prolactinoma showed normal height and bone maturation. There was no familial history of delayed puberty. On physical examination, 3 patients showed variable degree of breast development from Tanner stage II to IV without sex-steroid replacement. In sella MRI, small pituitary gland were identified in 2 patients with short statue and delayed bone maturation. All of the 3 patients underwent combined pituitary function test. After insulin-induced hypoglycemia, peak growth hormone levels of the 3 patients were 0.08, 1.4 and 1.4 ng/ml and were compatible with growth hormone deficiency. Peak LH after intravenous gonadrelin (FACTREL) were 19.0 to 56.1 mIU/ml and LH % responses were 217 to 1100% and were hence defined as not being gonadotropin deficiency. Other anterior pituitary functions were normal in all of the 3 patients.Conclusions: We found isolated growth hormone deficiency as the only identifiable cause for primary amenorrhea in three patients with sufficient gonadotropins secretion. These findings suggest a complementary role of GH to gonadotropins in the occurrence of menarche."}, {"id": "pubmed23n1164_4819", "title": "Case 308: Van Wyk-Grumbach Syndrome.", "score": 0.009259259259259259, "content": "An 11-year-old girl presented to the pediatric gastroenterology outpatient department of our institution with gradually increasing painless abdominal distention. The distention started 2 years earlier and was not associated with any other constitutional symptoms, vomiting, diarrhea, jaundice, hematemesis, or melaena. She reported early satiety and heaviness in the lower abdomen. The abdominal swelling was predominantly in the infraumbilical region and was soft at palpation. She was the first child of nonconsanguineous parents and had an uneventful perinatal course after a normal vaginal delivery. Her developmental milestones were normal. She had an average scholastic performance at school. There was no history of visual problems, seizures, or inappropriate behaviors. She had an early menarche 2 years previously. Her menstrual cycles were regular, and there was no abnormal vaginal discharge. Her breast development was normal (Tanner stage III), while pubic and axillary hair were absent (Tanner stage I). She was short for her age (104 cm; normal range, 120-154 cm). There was no history of short stature among her siblings or parents. Laboratory investigations were performed to measure thyroid-stimulating hormone (1354.34 µIU/mL; normal range, 0.35-5.5 µIU/mL), triiodothyronine (&lt;2.5 ng/dL [0.0385 pmol/L]; normal range, 100-200 ng/dL [1.54-3.08 pmol/L]), thyroxine (1.35 µg/dL [17.37 nmol/L]; normal range, 5-12 µg/dL [64.35-154.44 nmol/L]), β-human chorionic gonadotropin (&lt;1.2 mIU/mL; normal, &lt;5 mIU/mL), luteinizing hormone (0.08 mIU/mL; normal range, 0.1-6.0 mIU/mL), and follicle-stimulating hormone (6.93 mIU/mL; normal range, 0.3-2.0 mIU/mL) levels. Complete blood count was normal. An abdominal mass was suspected, and abdominopelvic CT was performed and followed by US; these examinations revealed multiple large cysts in both ovaries. The uterus was pubertal in shape, and endometrial thickness was 9 mm, representing normal follicular phase measurement. Serum CA-125 and inhibin levels were normal. To evaluate short stature, radiographs of the hand and pelvis were obtained as part of a limited skeletal survey, keeping in mind the possible skeletal changes associated with hypothyroidism. In view of the hypothyroidism, US of the neck was also performed. Treatment was started based on the clinical and radiologic parameters, and the child's condition improved with medical treatment."}, {"id": "pubmed23n0260_5467", "title": "[The status of the gonadotropin releasing hormone test in differential diagnosis of delayed puberty in adolescents over 14 years of age].", "score": 0.009259259259259259, "content": "In patients with delayed puberty with a bone age less than 11 years in girls or 12 years in boys, the clinical and endocrinological examination allows the differentiation of patients with the various forms of hypergonadotropic hypogonadism, but not of patients with hypogonadotropic hypogonadism from more prevalent constitutional delay in puberty. Therefore, watchful waiting is generally recommended for differential diagnosis in patients with delayed puberty. On the other hand, the late onset of sexual hormone replacement in patients with hypogonadism will worsen their outcome. Therefore, we decided to carry out a retrospective study in 105 adolescents who were examined because of short stature or delayed puberty, who were aged 14 to 22 years at first visit and in whom the differential diagnosis of delayed puberty was documented after an at least one-year follow-up in order to find out which endocrinological parameters could have effectively predicted the final diagnosis already at the first visit. Patients with hypogonadotropic hypogonadism differed from patients with constitutional delay in puberty by lower responses of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) levels to gonadotropin-releasing hormone stimulation (GnRH, 100 micrograms iv) (p &lt; 0.01) as well as by smaller testicular volume (p &lt; 0.05) and by lower testosterone levels (p &lt; 0.01). Stimulated LH &lt; 10 mU/ml differentiated patients with hypogonadotropic hypogonadism from constitutional delay in puberty with a sensitivity of 82% and a specificity of 98%. In patients with delayed puberty aged 14 years and older bone age usually exceeds 11 years in girls or 12 years in boys. It thus is in the range, in which normal adult responses of LH to GnRH can be expected. In contrast to patients aged less than 14 years, therefore, measuring GnRH-stimulated LH levels in these patients allows the rapid and effective differential diagnosis of delayed puberty."}, {"id": "pubmed23n0389_5240", "title": "Turner's syndrome mosaicism 45X/47XXX: an interesting natural history.", "score": 0.009174311926605505, "content": "Mosaicism 45X/47XXX is a sporadic form of ovarian dysgenesis. Many of the cases previously described were characterized by a variable phenotype expression. We here report the case of a 33-yr-old woman with recent secondary amenorrhea, weight loss and breast regression. Her menarche had occurred at the age of 11 yr and 6 months and her menstrual cycles had been regular until the age of 28; then, oligomenorrhea and hypertricosis developed. A pelvic ultrasound showed enlarged polycystic-like ovaries and normal uterus. She was treated with ethynil-estradiol and cyproterone acetate for one year. At the age of 31 yr, she underwent a pelvic ultrasound--which revealed normal volume of the ovaries--and hormonal assays including FSH (69 UI/l), LH (113 UI/l), 17beta-estradiol (88 pg/ml), plasma androgens and cortisol levels within normal ranges. No organ-specific autoantibodies toward ovaries, steroid-producing cells or adrenals were found. At the age of 33 yr, there was ultrasound evidence of streak-like ovaries. The patient's height was 145 cm and her weight 45 kg. She had normal female external genitalia, abnormal upper-to-lower body segment ratio, webbed neck, low posterior hair line, cubitus valgus, short and asymmetrical 4th metacarpi, hallux with lateral deviation and moderate scoliosis. No increase in ovarian steroids were found after GnRH-analogue triptorelin (0,1 mg sc) administration. The karyotype analysis on peripheral blood lymphocytes showed a mosaic 45X (90% cells) and 47XXX (10% cells). Diagnostic pelviscopy confirmed streak gonads. Chronic lymphocytic thyroiditis was diagnosed but no cardiovascular or kidney abnormalities were found. A neuro-psychological evaluation revealed emotional and social immaturity, disorders in motorial coordination, visual-spatial organization, as well as reading difficulties and impaired complex phrase construction. The presence of several somatic features of Turner's syndrome, neuro-psychological disorders and an interesting natural history probably depended on the quantitative proportion of 45X to 47XXX cell-lines in different tissues and organs. Estrogen and progestin replacement therapy led to weight gain, re-appearance of secondary sexual characteristics and a mild improvement in mental equilibrium."}, {"id": "pubmed23n0390_20260", "title": "Hormones and handedness.", "score": 0.009174311926605505, "content": "The Wessex Growth Study has monitored the growth and psychological development of short normal (SN) and average height control subjects since they entered school in 1985/1986. During psychometric testing, we found that 25% SN compared to 9% control subjects wrote with their left hand. The short group also attained significantly lower scores on measures of IQ and attainment and displayed less internalisation of control. Laterality, however, is thought to be influenced by the intrauterine environment and has been associated with pubertal delay. At recruitment, short children had a relatively low birth weight, delayed bone age and were more likely than controls to be short for family. To determine if birth conditions were associated with lateral preference and whether laterality could account for the differences found during the psychometric assessment or predict pubertal timing of SN children. Subjects were classified as right- (RH) or left-handed (LH) according to the writing hand and the data were investigated examining the effect of handedness and stature. RH and LH SN children were no more likely to suffer birth complications than those of average height. Psychometric testing did not reveal any significant differences between RH and LH SN children and their patterns of growth appeared to be similar. However, both RH and LH SN children scored less well on tests of cognitive ability and analyses of covariance revealed significant gender/handedness effects for both the timing of puberty and final height. The increase in left-handedness among SN children did not appear to be related to adverse birth conditions, but it may be that the hormones responsible for growth and development also play some part in brain laterality and cognitive development."}, {"id": "pubmed23n0224_19003", "title": "Daytime pulsatile growth hormone secretion during childhood and adolescence.", "score": 0.00909090909090909, "content": "Spontaneous GH secretory patterns were studied in 91 subjects (84 children, 2-18 yr old, at various stages of pubertal development and 7 healthy adults). Plasma GH was determined every 20 min for 6 h (0900-1500 h), and at least 1 spontaneous GH secretory episode (peak, greater than or equal to 5 ng/ml) was evident in 61 children and 5 of 7 adults. There was no significant difference in the mean number of GH secretory episodes or the mean 6-h plasma GH levels in 40 children with short stature compared to those in children of the same sex and pubertal maturation with normal or tall stature. The mean number of GH secretory episodes observed during the sampling period was significantly less in Tanner Stage II males (1.3 +/- 0.15) than in Tanner Stage III males (2.1 +/- 0.20; P less than 0.05). Also, the mean 6-h plasma GH level and the amplitude of the highest GH peak in Tanner Stage III (or greater) boys were greater than those in the prepubertal, early pubertal, or adult male subjects. Among females there was no difference in the number of peaks, mean 6-h plasma GH, or mean peak amplitude in prepubertal, pubertal, or adult subjects. Furthermore, there was no significant difference in overall mean 6-h plasma GH levels between male and female subjects. The frequency of GH secretory bursts was greater between 0830-0930 and 1330-1430 h. The GH secretory profiles were not different in children fed 1 or 2 meals. Children failing to show spontaneous peaks had GH deficiency secondary to central nervous system pathology (n = 10), psychosocial GH deficiency (n = 4), estrogen-dependent GH deficiency (n = 2), and optic nerve hypoplasia (n = 3). There were 2 false negatives and 2 children who were not retested. Pulsatile GH secretion is present during the daytime in children of all ages and stages of puberty. Determination of spontaneous GH secretory bursts is a safe and effective method for assessing GH deficiency."}, {"id": "First_Aid_Step2_807", "title": "First_Aid_Step2", "score": 0.009071460896038178, "content": "Complete androgen insensitivity: Patients present with breast development (aromatization of testosterone to estrogen) but are amenorrheic and lack pubic hair. First step: Get a pregnancy test. Next step: Obtain a radiograph to determine if bone age is consistent with pubertal onset (> 12 years in girls). ■If the patient is of short stature (bone age < 12 years) with normal growth velocity, constitutional growth delay (the most common cause of 1° amenorrhea) is the probable cause. ■If bone age is > 12 years but there are no signs of puberty, obtain LH/ FSH and consider where the problem is on the HPA axis (see Figure 2.12-3). ■↓ GnRH, ↓ LH/FSH, ↓ estrogen/progesterone at prepuberty levels: Points to constitutional growth delay (puberty has not yet started). ■↓ GnRH, ↓ LH/FSH, ↓ estrogen/progesterone: Hypogonadotropic hypogonadism. Suggests a hypothalamic or pituitary problem."}, {"id": "pubmed23n0284_7534", "title": "Aromatase deficiency in male and female siblings caused by a novel mutation and the physiological role of estrogens.", "score": 0.009009009009009009, "content": "The aromatase enzyme complex catalyzes the conversion of androgens to estrogens in a wide variety of tissues, including the ovary, testis, placenta, brain, and adipose tissue. Only a single human gene encoding aromatase P450 (CYP19) has been isolated; tissue-specific regulation is controlled in part by alternative promoters in a tissue-specific manner. We report a novel mutation in the CYP19 gene in a sister and brother. The 28-yr-old XX proband, followed since infancy, exhibited the cardinal features of the aromatase deficiency syndrome as recently defined. She had nonadrenal female pseudohermaphrodism at birth and underwent repair of the external genitalia, including a clitorectomy. At the age of puberty, she developed progressive signs of virilization, pubertal failure with no signs of estrogen action, hypergonadotropic hypogonadism, polycystic ovaries on pelvic sonography, and tall stature. The basal concentrations of plasma testosterone, androstenedione, and 17-hydroxyprogesterone were elevated, whereas plasma estradiol was low. Cyst fluid from the polycystic ovaries had a strikingly abnormal ratio of androstenedione and testosterone to estradiol and estrone. Hormone replacement therapy led to breast development, menses, resolution of ovarian cysts, and suppression of the elevated FSH and LH values. Her adult height is 177.6 cm (+2.5 SD). Her only sibling, an XY male, was studied at 24 yr of age. During both pregnancies, the mother exhibited signs of progressive virilization that regressed postpartum. The height of the brother was 204 cm (+3.7 SD) with eunuchoid skeletal proportions, and the weight was 135.1 kg (+2.1 SD). He was sexually fully mature and had macroorchidism. The plasma concentrations of testosterone (2015 ng/dL), 5 alpha-dihydrotestosterone (125 ng/dL), and androstenedione (335 ng/dL) were elevated; estradiol and estrone levels were less than 7 pg/mL. Plasma FSH and LH concentrations were more than 3 times the mean value. Plasma PRL was low; serum insulin-like growth factor I and GH-binding protein were normal. The bone age was 14 yr at a chronological age of 24 3/12 yr. Striking osteopenia was noted at the wrist. Bone mineral densitometric indexes of the lumbar spine (cancellous bone) and distal radius (cortical bone) were consistent with osteoporosis; the distal radius was -4.7 SD below the mean value for age- and sex-matched normal men; indexes of bone turnover were increased. Hyperinsulinemia, increased serum total and low density lipoprotein cholesterol, and triglycerides and decreased high density lipoprotein cholesterol were detected.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0419_17533", "title": "Growth without growth hormone: growth pattern and final height of five patients with idiopathic combined pituitary hormone deficiency.", "score": 0.009009009009009009, "content": "Growth without GH has been reported in patients with organic combined pituitary hormone deficiency (CPHD) after resection of craniopharyngiomas and hypothalamic tumours or in septo-optic dysplasia. This study describes the growth pattern and final height of five children (four boys, one girl) with idiopathic CPHD (GH, TSH, ACTH, LH and FSH) who maintained normal growth despite persistent GH deficiency throughout the growth period. Presenting findings were borderline small penis in two children diagnosed at ages 3 and 9 years, and absence of pubertal signs in three adolescents diagnosed at age 12.8-13.7 years. The latter three patients also exhibited acromegaloid features. The height of all patients was within the 10-25th percentiles, and weight at the 25-50th percentiles. Although they were moderately overweight, accelerated weight gain was not observed. Prepubertal growth rate was 4-5 cm/year. The pubertal growth period, starting after initiation of sex hormone therapy (chronological age 15.9-16.3 years and bone age 12.5-14.5 years) continued for 4-5.5 years. Total pubertal growth was 6-11.7 cm with reduced growth spurt. Final height, which was attained at an advanced age (19-22 years), was 170-179 cm in the boys and 164 cm in the girl, equal to or exceeding the target height range. Repeated hormonal evaluations revealed undetectable GH and IGF-I levels, and no evidence of hyperprolactinaemia or hyperinsulinism. Final height attainment within or above target height range may occur in patients with idiopathic CPHD despite persistent GHD. As this was not mediated by GH, IGF-I, insulin or prolactin, some other growth factors probably played a growth-promoting role."}, {"id": "Gynecology_Novak_5294", "title": "Gynecology_Novak", "score": 0.008950617283950617, "content": "Evaluation of Women with Amenorrhea Associated with the Absence of Secondary Sexual Characteristics A careful history and physical examination are necessary to appropriately diagnose and treat primary amenorrhea associated with hypogonadism. The physical examination may be particularly helpful in patients with Turner syndrome. A history of short stature but consistent growth rate, a family history of delayed puberty, and normal physical findings (including assessment of smell, optic discs, and visual fields) may suggest physiologic delay. Headaches, visual disturbances, short stature, symptoms of diabetes insipidus, and weakness of one or more limbs suggest central nervous system lesions (38). Galactorrhea may be seen with prolactinomas, a condition more commonly associated with secondary amenorrhea in the presence of normal secondary sexual characteristics. The diagnostic workup is summarized as follows: 1."}, {"id": "pubmed23n1065_5338", "title": "A Case Report Emphasizing the Importance of Early Diagnosis and Management of Intracranial Germinoma.", "score": 0.008928571428571428, "content": "Intracranial germ cell tumors (GCTs) account for 3%-5% of all intracranial tumors. They commonly manifest during first two decades of life. We are reporting a case of a young female, who presented with progressive visual loss, polyuria and polydipsia, harboring an intracranial GCT. She presented initially to a neurosurgery clinic and then to an endocrine clinic, with a history of chronic worsening headache and recent onset visual blurring along with polyuria with polydipsia. On further inquiry, she was found to have primary amenorrhea, easy fatigability, and failure of development of secondary sexual characteristics. On examination the patient had bitemporal hemianopia with breast development at tanner stage II and pubic and axillary hair at tanner stage I. Her initial hormonal workup was suggestive of panhypopituitarism with diabetes insipidus. MRI pituitary showed a sellar mass with suprasellar extension, so an initial impression of a pituitary macroadenoma was made and the patient underwent trans-sphenoidal surgery. The histopathology was suggestive of lymphoid hyperplasia. Follow up MRI showed significant residual tumor and her vision and pituitary function did not recover. Neurosurgery was planned as second surgery, but we requested a second opinion of histopathology report and it was suggestive of a germinoma. She was then started on chemotherapy followed by radiotherapy, after which her tumor size reduced significantly, though she still required pituitary hormone replacement therapy.  Pituitary stalk lesions are rare and their diagnosis is challenging as different etiologies present clinically and radiologically in a similar manner with tissue diagnosis being the gold standard. Germinoma is a radiosensitive tumor. In our patient it took a long time to reach the correct diagnosis and late diagnosis resulted in permanent visual field defect and panhypopituitarism. This case report emphasizes that we should guide and educate our patients to seek medical advice early in the course of disease. We should also keep differential diagnosis in mind before referring the patient for surgery."}, {"id": "pubmed23n0113_6062", "title": "[Long-term fate of pituitary dwarfs treated with growth hormone].", "score": 0.008928571428571428, "content": "A questionnaire having been mailed to 50 hypopituitary patients aged 18 to 36 years (m 21.7 +/- 3.4) previously treated with human growth hormone for at least 3 years, 44 answers have been received. The final height is 2.1 +/- 0.9 standard deviations below the average. However 57% of the patients consider it is sufficient. The smallness is felt as a handicap by 20% only of these adults, though 88% had suffered for it during their adolescence. The treatment is retrospectively considered as useful and acceptable by 68%, heavy but useful by 25%, heavy and useless by 7%, without correlation with the results. Only 41% are satisfied with their school achievements. However, more than two thirds of patients had severe school difficulties at the time of onset of the treatment. Actually 75% of the patients are professionally qualified, among whom 36% have achieved high school, and most have an educational level similar to that of their parents or even higher. But 41% only have an employment, 27% are still students and 32% are unemployed. The way of life of the young hypopituitary adults is severely affected: 6 only are married or living with a mate, 11 only write they have occasional sexual experiences, 16 remain completely alone. In contrast, leisure activities are good in more than 90%. A score taking all these data into consideration to evaluate their way of life shows, among the 43 complete answers to the questionnaire, 16% with excellent results, 49% with a rather good social status and 35% with poor final result.(ABSTRACT TRUNCATED AT 250 WORDS)"}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[25, 189]], "word_ranges": [[5, 33]], "text": "The statement perfectly defines a tolerance phenomenon in which the patient has become \"used to\" the dose and needs more in order to have the same analgesic effect."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The correct answer is 1. The statement perfectly defines a tolerance phenomenon in which the patient has become \"used to\" the dose and needs more in order to have the same analgesic effect.", "full_answer_no_ref": "The [HIDDEN] The statement perfectly defines a tolerance phenomenon in which the patient has become \"used to\" the dose and needs more in order to have the same analgesic effect.", "full_question": "The patient comes to the home of an oncology patient whose pain has not been well controlled lately. On physical examination there appears to be no evidence of tumor progression, and no previously known data of interest is revealed. In the anamnesis, the main caregiver states that the patient has pain 8 hours after receiving the prescribed basal dose of morphine every 12 hours. This clinical situation is referred to as:", "id": 409, "lang": "en", "options": {"1": "Tolerance", "2": "Hyperalgesia", "3": "Dependency", "4": "Ineffectiveness", "5": NaN}, "question_id_specific": 191, "type": "PRIMARY CARE", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en289_33584", "title": "Patient-controlled analgesia", "score": 0.011736001729516044, "content": "With a PCA the patient is protected from overdose by the caregiver programming the PCA to deliver a dose at set intervals. If the patient presses the button sooner than the prescribed intake pressing the button does not operate the PCA. (The PCA can be set to emit a beep telling the patient a dose was NOT delivered). Dosage is also controlled when the patient is too sedated to press the button; preventing the patient from receiving needless doses and keeping the patient safe from overdosing. Epidural Patient-controlled epidural analgesia (PCEA) is a related term describing the patient-controlled administration of analgesic medicine in the epidural space, by way of intermittent boluses or infusion pumps. This can be used by women in labour, terminally ill cancer patients or to manage post-operative pain."}, {"id": "wiki20220301en354_20189", "title": "Cancer pain", "score": 0.010107455524643304, "content": "Analgesics should not be taken \"on demand\" but \"by the clock\" (every 3–6 hours), with each dose delivered before the preceding dose has worn off, in doses sufficiently high to ensure continuous pain relief. People taking slow-release morphine should also be provided with immediate-release (\"rescue\") morphine to use as necessary, for pain spikes (breakthrough pain) that are not suppressed by the regular medication. Oral analgesia is the cheapest and simplest mode of delivery. Other delivery routes such as sublingual, topical, transdermal, parenteral, rectal or spinal should be considered if the need is urgent, or in case of vomiting, impaired swallow, obstruction of the gastrointestinal tract, poor absorption or coma. Current evidence for the effectiveness of fentanyl transdermal patches in controlling chronic cancer pain is weak but they may reduce complaints of constipation compared with oral morphine."}, {"id": "wiki20220301en131_179", "title": "Opioid-induced hyperalgesia", "score": 0.009900990099009901, "content": "activation (desensitization of antinociceptive mechanisms) and opioid receptor down-regulation (internalization of membrane receptors). In opioid-induced hyperalgesia, sensitization of pronociceptive mechanisms occurs, resulting in a decrease in the pain threshold, or allodynia. In addition, what appears to be opioid tolerance can be caused by opioid-induced hyperalgesia lowering the baseline pain level, thus masking the drug's analgesic effects. Identifying the development of hyperalgesia is of great clinical importance since patients receiving opioids to relieve pain may paradoxically experience more pain as a result of treatment. Whereas increasing the dose of opioid can be an effective way to overcome tolerance, doing so to compensate for opioid-induced hyperalgesia may worsen the patient's condition by increasing sensitivity to pain while escalating physical dependence."}, {"id": "pubmed23n0590_13933", "title": "[Outpatient opiate therapy in cancer patients during their last days of life].", "score": 0.009900990099009901, "content": "Pain is one of the symptoms that many tumor patients are especially afraid of in the final phase of their illness. Symptoms can change rapidly, making quick adaptation of the therapy necessary. This poses particular challenges to organizational structures in outpatient treatment if the patients' desire to spend their last days of life in their accustomed surroundings is to be realized. Pain intensity and the associated symptoms in a WHO step III opiate therapy during the last 3 days of life were investigated retrospectively among 601 tumor patients who had received medical care from Home Care Berlin. Differences in gender, age, living conditions/care situation and place of death were evaluated with due consideration for the different pain medications administered and for the different forms and routes of administration. More than 80% of the patients reported freedom from pain or only moderate pain during the opiate therapy. Care provided by the hospice-at-home medical service Home Care Berlin allowed excellent control of tumor patients' symptoms with only moderate side-effects in their final days of life. Among patients receiving opioids by the transdermal route there were significantly more frequent complaints of pain (p=0.004) and nausea (p=0.001). During the last days of life continuous subcutaneous infusions containing opiates facilitated good analgesia within an acceptable spectrum of side-effects. Most problems with controlling symptoms in outpatients were encountered in younger patients. Morphine emerged as the drug of first choice in this investigation, because it can be given parenterally and also because of its price. The use of subcutaneous and intravenous administration systems such as PCA pumps requires trained nursing services and regular house visits by physicians experienced in palliative medicine."}, {"id": "wiki20220301en356_22715", "title": "Racotumomab", "score": 0.00980392156862745, "content": "Treatment administration and side effects Racotumomab is administered in patients who have previously received the oncospecific treatment established in the oncological therapeutic standards (surgery, chemotherapy and radiation therapy). A Racotumomab-alum solution is administered by intradermal injection every 14 days for the first 2 months (5 doses), followed by monthly booster doses. Racotumomab is well tolerated by patients. The overall toxicity of the vaccine has been classified as grade 1 and 2, according to the NCI Common Toxicity Criteria (version 3.0). Treatment is mostly associated with mild to moderate injection-site reactions (local erythema, induration and pain), which disappear within 24–48 hours. Systemic reactions, such as flu-like symptoms and chills are less frequent, reversible, and self-limited. Approvals and indications"}, {"id": "pubmed23n0328_5262", "title": "[Terminal care of the cancer patient at home--patient and family spending the last moments together].", "score": 0.00980392156862745, "content": "Some 129 cancer patients were taken care of at home by the visiting nurse team of our hospital since 1994 to 1997; 116 patients have already died, 50 of them at home (group A), and 66 at the hospital (group B). We analyze the differences the between two groups in nutritional assistance, pain control and manpower in looking after a patient. Twice as many patients in group A took nutritional medication (drip infusion or intravenous hyperalimentation, for example) than group B. More patients in group A used oral and suppository morphine than group B. In group A 70% of patients (56% in group B) had more than two persons looking after at home. The important factors for a good recuperation at home are: 1. Available nutritional assist available. 2. Good control. 3. Less burden and anxiety of the person looked after. To spend pain desirable last moments together, it is important for the visiting nurse team to take proper care and to confirm the wishes of the patient and family, considering the rapidly changing conditions of the individual patient."}, {"id": "wiki20220301en556_14481", "title": "Ubrogepant", "score": 0.009708737864077669, "content": "The effectiveness of ubrogepant for the acute treatment of migraine was demonstrated in two randomized, double-blind, placebo-controlled trials. In these studies, 1,439 adult patients with a history of migraine, with and without aura, received the approved doses of ubrogepant to treat an ongoing migraine. In both studies, the percentages of patients achieving pain freedom two hours after treatment (defined as a reduction in headache severity from moderate or severe pain to no pain) and whose most bothersome migraine symptom (nausea, light sensitivity or sound sensitivity) stopped two hours after treatment were significantly greater among patients receiving ubrogepant (19–21% depending on the dose) compared to those receiving placebo (12%). Patients were allowed to take their usual acute treatment of migraine at least two hours after taking ubrogepant. 23% of patients were taking a preventive medication for migraine."}, {"id": "pubmed23n0502_2110", "title": "Patterns of high-dose morphine use in a home-care hospice service: should we be afraid of it?", "score": 0.009708737864077669, "content": "Management of cancer pain is one of the most important goals of palliative care. Relieving pain is often problematic. High doses of morphine at home may be required to relieve patients' pain, and is therefore feared. The goals of the current study were to assess the feasibility of high-dose morphine use at home, to characterize the patients, and to examine whether the use of high-dose morphine might affect their survival. The authors retrospectively studied the medical charts of 661 outpatients, which were completed by a home-care hospice team. The authors collected data regarding demographic parameters, medical diagnosis, pain type, morphine dosage, use of rescue doses in addition to regular doses, use of coanalgesics and adjuvant treatments, and survival time as associated with morphine dosage. The authors also compared the data of patients receiving high-dose morphine with those of a group of patients receiving regular doses. The authors identified 435 patients (65.8%) who received morphine for pain relief. Of these, 396 patients (91%) received a dose of 5-299 mg of morphine per day), 32 patients (7.4%) received 300-599 mg of morphine per day), and 7 patients (1.6%) received very high doses (&gt; or = 600 mg of morphine per day). Overall, 39 patients (9%) received &gt; 299 mg per day. Morphine dosage was found to be inversely correlated (r) with age (r = -0.254; P &lt; 0.001). Male patients required slightly higher dosages than female patients (62.5% of high-dose and 71% of very high-morphine groups, respectively). Primary gastrointestinal (P = 0.015) and lung (P = 0.027) carcinomas, as well as metastatic bone disease (P = 0.001), ovarian carcinoma (P = 0.037), and brain tumors (P = 0.0053) were associated with higher and very higher morphine dosages. Adverse effects were similar in the groups receiving regular, high, and very high doses of morphine. The median survival of patients treated with high doses of morphine was 27 days and was 37 days for those treated with very high doses. Patients treated with low doses of morphine survived for 18 days. Patients not treated with morphine survived for 22 days (P = 0.001 by Mantel-Cox analysis; P = 0.029 by Breslow analysis). The use of high and very high morphine doses at home proved safe and did not appear to affect the patients' life expectancy adversely. The use of high or very high-dose morphine should not be a barrier to providing palliative terminal care for home-care hospice patients."}, {"id": "wiki20220301en131_194", "title": "Opioid-induced hyperalgesia", "score": 0.009615384615384616, "content": "The use of an NSAID, especially some COX-2 inhibitors, or acetaminophen either as monotherapy or combination therapy is also suggested as a possible treatment option. Research needs It can be difficult to apply research into OIH to average patients, because some research focused on people taking very high doses or in methadone rehabilitation programs. Opioid-induced hyperalgesia has also been criticized as overdiagnosed among chronic pain patients, due to poor differential practice in distinguishing it from the much more common phenomenon of opioid tolerance. The misdiagnosis of common opioid tolerance (OT) as opioid-induced hyperalgesia (OIH) can be problematic as the clinical actions suggested by each condition can be contrary to each other. Patients misdiagnosed with OIH may have their opioid dose mistakenly decreased (in the attempt to counter OIH) at times when it is actually appropriate for their dose to be increased or rotated (as a counter to opioid tolerance)."}, {"id": "pubmed23n0749_24517", "title": "Opioid use and effectiveness of its prescription at discharge in an acute pain relief and palliative care unit.", "score": 0.009615384615384616, "content": "The aim of this study was to present how opioids are used in an acute pain relief and palliative care unit (APRPCU), where many patients with difficult pain conditions are admitted from GPs, home palliative care programs, oncology departments, other hospitals or emergency units, and other regional places. From a consecutive sample of cancer patients admitted to an APRPCU for a period of 6 months, patients who had been administered opioids were included in this survey. Basic information was collected as well as opioid therapy prescribed at admission and, subsequently, during admission and at time of discharge. Patients were discharged once stabilization of pain and symptoms were obtained and the treatment was considered to be optimized. One week after being discharged, patients or relatives were contacted by phone to gather information about the availability of opioids at dosages prescribed at time of discharge. One hundred eighty six of 231 patients were specifically admitted for uncontrolled pain, with a mean pain intensity of 6.8 (SD 2.5). The mean dose of oral morphine equivalents in patients receiving opioids before admission was 45 mg/day (range 10-500 mg). One hundred seventy five patients (75.7 %) were prescribed around the clock opioids at admission. About one third of patients changed treatment (opioid or route). Forty two of 175 (24 %), 27/58 (46.5 %), 10/22 (45.4 %), and 2/4 (50 %) patients were receiving more than 200 mg of oral morphine equivalents, as maximum dose of the first, second, third, and fourth opioid prescriptions, respectively. The pattern of opioids changed, with the highest doses administered with subsequent line options. The mean final dose of opioids, expressed as oral morphine equivalents, for all patients was 318 mg/day (SD 798), that is more than six times the doses of pre-admission opioid doses. One hundred eighty six patients (80.5 %) were prescribed a breakthrough cancer pain (BTcP) medication at admission. Sixty five patients changed their BTcP prescription, and further 27 patients changed again. Finally, eight patients were prescribed a fourth BTcP medication. Of 46 patients available for interview, the majority of them (n = 39, 84 %) did not have problems with their GPs, who facilitated prescription and availability of opioids at the dosages prescribed at discharge. For patients with severe distress, APRPCUs may guarantee a high-level support to optimize pain and symptom intensities providing intensive approach and resolving highly distressing situations in a short time by optimizing the use of opioids."}, {"id": "wiki20220301en422_29695", "title": "Mistresses (American season 1)", "score": 0.009547899006471607, "content": "Karen After her patient, Thomas Grey, reveals that he is dying of lung cancer and that he has been in love with her for a year, Karen begins having an affair with him. To help him end his life when the pain becomes too much, Karen prescribes Thomas fatal doses of morphine, which his wife, Elizabeth, helps to administrate. The insurance company begins to investigate Thomas' death, going so far as to approach Karen for the notes from her sessions with Thomas. However, Karen had destroyed anything relating to Thomas being her patient. While grieving his father's death, Thomas' son Sam turns to Karen for advice as he has discovered that his father was having an affair. Even though Savi advises Karen to cut all ties with the Grey Family, Karen begins helping Sam. Things take a dangerous turn after Sam falls in love with Karen and begins stalking her."}, {"id": "wiki20220301en175_2628", "title": "Agkistrodon contortrix phaeogaster", "score": 0.009523809523809525, "content": "CroFab antivenin has been used successfully to treat Osage copperhead bites, although a lack of complete cross-tolerance requires careful administration and close supervision during the full course of treatment to ensure that the lowest effective dose is administered (a lower dose would not fully treat the envenomation, and a higher dose may be particularly dangerous to children, the elderly, and infirm adults). Not uncommonly, opiate/opioid narcotic analgesics (ex. morphine, fentanyl), muscle relaxerss (ex. diazepam, tizanidine, orphenadrine), and broad-spectrum antibiotics are administered. A few days' supply of weaker analgesics and muscle relaxers may be prescribed for the patient to control pain after he or she returns home as the pain resolves completely within one to three days. Patients also receive a prescription for an intensive antibiotic therapy, which much be taken until the supplies are depleted, giving the drug enough time to fully treat any opportunistic infections"}, {"id": "pubmed23n0659_24409", "title": "[Opioid rotation in the home medical care service].", "score": 0.009523809523809525, "content": "Pain relief is a quite important subject for maintaining the home medical care of patients with terminal cancer. Therefore, the opioid rotation should be made in conjunction with an individual medical condition, which is of growing importance in a proper pain management. We considered what opioid rotation is desirable in the home medical care service by analyzing the cases at our clinic. The most important thing in the opioid rotation at home is to perform a rotation before exacerbation of pain becomes apparent. For this purpose, morphine hydrochloride injection is thought to be the best dosage form because it has advantages of: (1) quickness in varying the amount, (2) immediate rescue efficacy, and (3) usefulness in case of ingestion."}, {"id": "wiki20220301en001_39074", "title": "Oxycodone", "score": 0.009433962264150943, "content": "Oxycodone has a half-life of 4.5 hours. It is available as a generic medication. The manufacturer of OxyContin, a controlled-release preparation of oxycodone, Purdue Pharma, claimed in their 1992 patent application that the duration of action of OxyContin is 12 hours in \"90% of patients.\" It has never performed any clinical studies in which OxyContin was given at more frequent intervals. In a separate filing, Purdue claims that controlled-release oxycodone \"provides pain relief in said patient for at least 12 hours after administration.\" However, in 2016 an investigation by the Los Angeles Times found that \"the drug weans off hours early in many people,\" inducing symptoms of opiate withdrawal and intense cravings for OxyContin. One doctor, Lawrence Robbins, told journalists that over 70% of his patients would report that OxyContin would only provide 4–7 hours of relief. Doctors in the 1990s often would switch their patients to a dosing schedule of once every eight hours when they"}, {"id": "pubmed23n0597_2723", "title": "[Patient-controlled analgesia in outpatients with severe cancer pain.].", "score": 0.009433962264150943, "content": "In this case report, we describe continuous subcutaneous infusion of opiates as PCAO (patient controlled analgesia in outpatients) in one patient with metastatic carcinoma of the rectum (liver and bone metastases, partial bowel obstruction) with severe cancer pain and vomiting in the terminal phase. The parenteral administration of opioids extended over 58 days. The infusion was powered by an external portable clockwork-driven syringe pump (Perfusor M, Braun Medical/Germany). The open-accessible pump has a syringe volume of 10 ml, and its maximal infusion time is 24 h. The 27-G infusion needle (Sub-Q-Set, Baxter/USA) was inserted in the side of the abdomen and was left in the same position for 10 to 20 days. It took the patient and his family only 1.5 h to familiarize themselves with the use of the pump. They were trained in its use in our outpatient pain department. For pain control both the variable continuous infusion and the extra injection doses could be administered by the way of the syringe driver. The patient was given a stock of 120 ampoules of morphine for further treatment at home. For optimal pain control he decided to raise the daily dose of opioid infusion from the initial 60 mg to 240 mg morphine within 48 h. In this way, PCAO-besides rapid titration of the opioid dose to achieve analgesia-allows the use of opioids controlled by the patient himself. In the present case this procedure was also important when an outpatient radiation therapy became urgently necessary to prevent a fracture of the spine because of metastasis. The pain control by the patient himself was the main factor to get free of pain during the transport to the hospital. Even positioning for radiation was possible without pain. When he received outpatient radiation therapy the patient needed extra injection doses of up to 360 mg morphine a day. The PCAO procedure by continuous subcutaneous infusion with opiates is a safe and efficient method of pain management for outpatient patients suffering from severe cancer pain and intractable nausea in the terminal phase. Its validity has also been proven especially for radiation treatment of bone metastases."}, {"id": "wiki20220301en352_34206", "title": "Neutron capture therapy of cancer", "score": 0.009345794392523364, "content": "was well tolerated by the 30 patients who were enrolled in this study. All were treated with 2 fields, and the average whole brain dose was 3.2–6.1 Gy (weighted), and the minimum dose to the tumor ranged from 15.4 to 54.3 Gy (w). There has been some disagreement among the Swedish investigators regarding the evaluation of the results. Based on incomplete survival data, the MeST was reported as 14.2 months and the time to tumor progression was 5.8 months. However, more careful examination of the complete survival data revealed that the MeST was 17.7 months compared to 15.5 months that has been reported for patients who received standard therapy of surgery, followed by radiotherapy (RT) and the drug temozolomide (TMZ). Furthermore, the frequency of adverse events was lower after BNCT (14%) than after radiation therapy (RT) alone (21%) and both of these were lower than those seen following RT in combination with TMZ. If this improved survival data, obtained using the higher dose of BPA"}, {"id": "pubmed23n0349_8700", "title": "Intravenous titration with morphine for severe cancer pain: report of 28 cases.", "score": 0.009345794392523364, "content": "In a multicenter study, 28 patients with cancer pain and insufficient pain relief with analgesic treatment according to step II of the guidelines of the World Health Organization (WHO) were switched to oral slow-release morphine. Patients received intravenous morphine through a patient-controlled pump (PCA) for the first 24 hours (bolus = 1 mg, lockout interval = 5 minutes, maximum dose = 12 mg/hour). From day 2 patients were treated with oral slow-release morphine. Daily doses were calculated from the requirements of the day before. Breakthrough pain was treated with PCA until stable doses were reached (&lt;2 boluses/day) and then with oral immediate-release morphine solution. Pain intensity was reported in a diary four times a day, in addition to mood, activity, and quality of sleep once daily. Mean duration until adequate pain relief reported (&lt;30 on a 101-step numerical scale; NRS) was 5 hours (range = 80-620 minutes). Mean pain intensity was reduced from 67 NRS to 22 NRS. Mean doses of oral morphine were 133 mg/day initially and then 154 mg/day on day 14. Serious adverse events such as respiratory depression were not observed. Two patients terminated the study due to progressive symptoms of gastrointestinal obstruction. Seventy-five percent of the patients evaluated the effectiveness of the analgesic regime as good. Dose finding with intravenous PCA may be appropriate for a small minority of patients with severe pain. Higher treatment costs and the risk of complications are drawbacks of this method compared with conventional oral titration."}, {"id": "wiki20220301en544_13987", "title": "Bland embolization", "score": 0.009259259259259259, "content": "After embolization, patients are observed in the post-anesthesia care unit for several hours. Patients are discharged from the hospital when taking adequate nutrition by mouth, when pain is adequately controlled with oral narcotics and when the temperature is lower than 38.5 for 24 hours. Post-procedure evaluation Follow-up triple phase CT is performed 2 to 4 weeks after treatment is complete and reviewed for any evidence of persistent untreated disease. If there is no evidence of enhancement of the treated tumor, these patients are monitored with triple phase CT every 3 months for the first year and every 6 months thereafter. When there is evidence of untreated disease, recurrent disease, or new disease elsewhere within the liver the patient is scheduled for additional embolization. References Hepatology Diseases of liver"}, {"id": "pubmed23n0045_19047", "title": "Analgesic use in home hospice cancer patients.", "score": 0.009259259259259259, "content": "Pain control in hospice patients in the home may be compromised by concerns about overuse of analgesics, particularly narcotics. A retrospective chart audit of analgesic type and amount was performed on the medical records of 100 cancer patients receiving hospice care in the home. Different types and amounts of analgesics were converted to a common standard, an oral morphine equivalent (OME) relative to 1 mg of oral morphine sulfate. Descriptive statistics were used to characterize patient analgesic use during the entire course of hospice care and the last 5 days of life. Associations between analgesic use and select patient characteristics (age, sex, cancer site, metastases, and pain intensity at admission) were explored. Ninety-one percent of the sample had used analgesics at some time during hospice care. The proportion of patients using analgesics increased as death approached. The mean and median daily analgesic use over the entire period were 114 and 82 OMEs and during the last 5 days 140 and 84 OMEs, respectively. The range of mean daily analgesic use was between 10 and 735 OMEs. Individual variability in analgesic use was demonstrated. Not all patients required analgesics, and among those who did there was remarkable variation in the amount used. Large and even enormous doses of analgesics may sometimes be required to control cancer pain."}, {"id": "pubmed23n0800_14851", "title": "Breathlessness with pulmonary metastases: a multimodal approach.", "score": 0.009174311926605505, "content": "Case Study  Sarah is a 58-year-old breast cancer survivor, social worker, and health-care administrator at a long-term care facility. She lives with her husband and enjoys gardening and reading. She has two grown children and three grandchildren who live approximately 180 miles away. SECOND CANCER DIAGNOSIS  One morning while showering, Sarah detected a painless quarter-sized lump on her inner thigh. While she thought it was unusual, she felt it would probably go away. One month later, she felt the lump again; she thought that it had grown, so she scheduled a visit with her primary care physician. A CT scan revealed a 6.2-cm soft-tissue mass in the left groin. She was referred to an oncologic surgeon and underwent an excision of the groin mass. Pathology revealed a grade 3 malignant melanoma. She was later tested and found to have BRAF-negative status. Following her recovery from surgery, Sarah was further evaluated with an MRI scan of the brain, which was negative, and a PET scan, which revealed two nodules in the left lung. As Sarah had attended a cancer support group during her breast cancer treatment in the past, she decided to go back to the group when she learned of her melanoma diagnosis. While the treatment options for her lung lesions included interleukin-2, ipilimumab (Yervoy), temozolomide, dacarbazine, a clinical trial, or radiosurgery, Sarah's oncologist felt that ipilimumab or radiosurgery would be the best course of action. She shared with her support group that she was ambivalent about this decision, as she had experienced profound fatigue and nausea with chemotherapy during her past treatment for breast cancer. She eventually opted to undergo stereotactic radiosurgery. DISEASE RECURRENCE  After the radiosurgery, Sarah was followed every 2 months. She complained of shortness of breath about 2 weeks prior to each follow-up visit. Each time her chest x-ray was normal, and she eventually believed that her breathlessness was anxiety-related. Unfortunately, Sarah's 1-year follow-up exam revealed a 2 cm × 3 cm mass in her left lung, for which she had a surgical wedge resection. Her complaints of shortness of breath increased following the surgery and occurred most often with anxiety, heat, and gardening activities, especially when she needed to bend over. Sarah also complained of a burning \"pins and needles\" sensation at the surgical chest wall site that was bothersome and would wake her up at night. Sarah met with the nurse practitioner in the symptom management clinic to discuss her concerns. Upon physical examination, observable signs of breathlessness were lacking, and oxygen saturation remained stable at 94%, but Sarah rated her breathlessness as 7 on the 0 to 10 Borg scale. The nurse practitioner prescribed duloxetine to help manage the surgical site neuropathic pain and to assist with anxiety, which in turn could possibly improve Sarah's breathlessness. Several nonpharmacologic modalities for breathlessness were also recommended: using a fan directed toward her face, working in the garden in the early morning when the weather is cooler, gardening in containers that are at eye level to avoid the need to bend down, and performing relaxation exercises with pursed lip breathing to relieve anxiety-provoked breathlessness. One month later, Sarah reported relief of her anxiety; she stated that the fan directed toward her face helped most when she started to feel \"air hungry.\" She rated her breathlessness at 4/10 on the Borg scale. SECOND RECURRENCE: MULTIPLE PULMONARY NODULES  Sarah's chest x-rays remained clear for 6 months, but she developed a chronic cough shortly before the 9-month exam. An x-ray revealed several bilateral lung lesions and growth in the area of the previously resected lung nodule. Systemic therapy was recommended, and she underwent two cycles of ipilimumab. Sarah's cough and breathlessness worsened, she developed colitis, and she decided to stop therapy after the third cycle. In addition, her coughing spells triggered bronchospasms that resulted in severe anxiety, panic attacks, and air hunger. She rated her breathlessness at 10/10 on the Borg scale during these episodes. She found communication difficult due to the cough and began to isolate herself. She continued to attend the support group weekly but had difficulty participating in conversation due to her cough. Sarah was seen in the symptom management clinic every 2 weeks or more often as needed. No acute distress was present at the beginning of each visit, but when Sarah began to talk about her symptoms and fear of dying, her shortness of breath and anxiety increased. The symptom management nurse practitioner treated the suspected underlying cause of the breathlessness and prescribed oral lorazepam (0.5 to 1 mg every 6 hours) for anxiety and codeine cough syrup for the cough. Opioids were initiated for chest wall pain and to control the breathlessness. Controlled-release oxycodone was started at 10 mg every 12 hours with a breakthrough pain (BTP) dose of 5 mg every 2 hours as needed for breathlessness or pain. Sarah noted improvement in her symptoms and reported a Borg scale rating of 5/10. Oxygen therapy was attempted, but subjective improvement in Sarah's breathlessness was lacking. END OF LIFE  Sarah's disease progressed to the liver, and she began experiencing more notable signs of breathlessness: nasal flaring, tachycardia, and restlessness. Opioid doses were titrated over the course of 3 months to oxycodone (40 mg every 12 hours) with a BTP dose of 10 to 15 mg every 2 hours as needed, but her breathlessness caused significant distress, which she rated 8/10. The oxycodone was rotated to IV morphine continuous infusion with patient-controlled analgesia (PCA) that was delivered through her implantable port. This combination allowed Sarah to depress the PCA as needed and achieve immediate control of her dyspneic episodes. Oral lorazepam was also continued as needed. Sarah's daughter moved home to take care of her mother, and hospice became involved for end-of-life care. As Sarah became less responsive, nurses maintained doses of morphine for control of pain and breathlessness and used a respiratory distress observation scale to assess for breathlessness since Sarah could no longer self-report. A bolus PCA dose of morphine was administered by Sarah's daughter if her mother appeared to be in distress. Sarah died peacefully in her home without signs of distress. "}, {"id": "pubmed23n0363_6533", "title": "[Emergency pain treatment--ambulatory intravenous morphine titration in a patient with cancer pain].", "score": 0.009174311926605505, "content": "Even when the guidelines for cancer pain management are followed, acute severe pain requiring immediate treatment will occur in some patients. Titration with intravenous morphine may provide fast and efficient pain relief and give an indication of the amount of opioid necessary for continuous treatment. In cooperation with a general practitioner we performed an intravenous morphine titration in a patient with severe cancer pain at home. Adequate analgesia was reached with 20 mg intravenous morphine. Blood pressure, cardiac frequency and oxygen saturation did not change. No side effects were reported during the titration, and the previous regimen with tramadol 150 mg per day was switched to slow release morphine 300 mg per day. This medication was prescribed by the general practitioner and provided good pain relief until the patient died two weeks later. We conclude that intravenous morphine titration may be performed even in cancer patients at home, adequate monitoring, however, should be available."}, {"id": "wiki20220301en020_61862", "title": "Opioid", "score": 0.00909090909090909, "content": "The initial 24 hours after opioid administration appear to be the most critical with regard to life-threatening OIRD, but may be preventable with a more cautious approach to opioid use. Patients with cardiac, respiratory disease and/or obstructive sleep apnoea are at increased risk for OIRD. Increased pain sensitivity Opioid-induced hyperalgesia – where individuals using opioids to relieve pain paradoxically experience more pain as a result of that medication – has been observed in some people. This phenomenon, although uncommon, is seen in some people receiving palliative care, most often when dose is increased rapidly. If encountered, rotation between several different opioid pain medications may decrease the development of increased pain. Opioid induced hyperalgesia more commonly occurs with chronic use or brief high doses but some research suggests that it may also occur with very low doses."}, {"id": "pubmed23n0582_16576", "title": "Intravenous morphine consumption in outpatients with cancer during their last week of life--an analysis based on patient-controlled analgesia data.", "score": 0.00909090909090909, "content": "Studies on opioid use in terminally ill cancer patients have shown a prefinal dose increase in the majority of patients. Mostly oral opioids were used. Due to the pharmacokinetic properties of opioids, it is rather difficult to get a reliable estimate of the true opioid need from those results. Retrospectively, we analyzed opioid use during the last week of life of 30 consecutive outpatients with cancer on intravenous (i.v.) morphine patient-controlled analgesia (PCA). A dose increase (decrease) was defined as an increase (decrease) of the patient's individual daily dose by at least 30% with respect to their prior daily dose. We also analyzed circadian variations in morphine use. Thirty patients fulfilled the primary study inclusion criteria. Fulfilling the exclusion criteria, seven patients had to be excluded from analysis (n = 3, on PCA for less than 7 days; n = 4, PCA was finished before death). Twenty-three patients with a total of 161 treatment days were analyzed. The patients' median age was 57 years (range, 4 to 72). The median duration of intravenous morphine PCA was 19 days (range, 8 to 58). The median daily intravenous morphine dose during the last week of life was 96 to 115 mg, without significant change over time/from day to day (Friedman test). On 144/161 days (89.2%), morphine dose remained stable. On 9 treatment days (5.6%), the dose increased, and on 8 days (5.0%), it decreased. In three patients, only dose increases, and in four patients, only dose decreases were observed. In four patients, both dose increases and decreases were observed. Twelve patients showed no change in daily morphine dose. Opioid use lacked a diurnal pattern. During their end-of-life phase, cancer patients on i.v. morphine PCA showed a stable daily opioid need."}, {"id": "wiki20220301en027_47835", "title": "Buprenorphine", "score": 0.009009009009009009, "content": "Both buprenorphine and methadone are medications used for detoxification and opioid replacement therapy, and appear to have similar effectiveness based on limited data. Both are safe for pregnant women with opioid use disorder, although preliminary evidence suggests that methadone is more likely to cause neonatal abstinence syndrome. In the US and European Union, only designated clinics can prescribe methadone for opioid use disorder, requiring patients to travel to the clinic daily. If patients are drug free for a period they may be permitted to receive \"take home doses,\" reducing their visits to as lilttle as once a week. Alternatively, up to a month's supply of buprenorphine has been able to be prescribed by clinicians in the US or Europe who have completed a basic training (8–24 hours in the US and received a waiver/licence allowing prescription of the medicine. In France, buprenorphine prescription for opioid use disorder has been permitted without any special training or"}, {"id": "pubmed23n0299_3086", "title": "[How to palliate the symptoms of terminally ill patients at home].", "score": 0.009009009009009009, "content": "We reported the kind of symptoms and how they could be palliated in terminally ill patients at home based on our experience of about 9 years. Cancer pain, which was the most frequent symptom, appeared in 67 among 126 patients receiving home care, and it could be effectively controlled with morphine; no patient returned to the hospital because of aggravation of pain. Very few patients stayed in the hospital and never returned home due to uncontrollable pain. Home parenteral infusion was done for 63 patients who were unable to eat or drink because of peritonitis carcinomatosa or cancer cachexia. High fever in the tumor mass was controlled by glucocorticoid hormone, and ascites was drained continuously when the patients suffered from abdominal distension. From analysis of the cases in which home care was interrupted or those in which patients were unable to transfer to home care, symptoms that were difficult to palliate at home were nausea caused by bowel obstruction, acute symptoms (bleeding, disturbance of consciousness, and so on), and dyspnea. But if the patients and family are eager for home care and an adequate medical support system is in place, home care may be possible despite these symptoms."}, {"id": "wiki20220301en300_9122", "title": "Oxytrex", "score": 0.008928571428571428, "content": "A phase III clinical trial has shown that the combination of ULD (ultra-low dose) naltrexone with oxycodone has been nearly as effective in providing pain relief while causing less physical dependence than oxycodone alone. The trial has been criticized for its confounding and limiting factors which include the huge dropout rate (54%), lack of demographic stratification of pain intensity amongst the study arms, and that patients received varying amounts of oxycodone which would influence their overall dependence. The lack of an on-board break-through-pain agent also hurts their external validity and likely contributed to their high dropout rate. References Webster et al., \"Oxytrex Minimizes Physical Dependence While Providing Effective Analgesia: A Randomized Controlled Trial in Low Back Pain\", The Journal of Pain, Vol 7, No 12, 2006:937-946 Expert Opin. Investig. Drugs (2007) 16(8):1277-1283 Mu-opioid receptor agonists Semisynthetic opioids Combination drugs Experimental drugs"}, {"id": "pubmed23n0252_10171", "title": "[Cancer-pain management in home care].", "score": 0.008928571428571428, "content": "Patient was 58 year old female with severe uterus cancer pain who refused to take the oral morphine in hospital because doctor should be hesitated to discharged patient whose pain is particularly difficult to manage in home. A few days later, she returned back home with her family irritably and recovered from hallucination by morphine-intake in home. The oral morphine is the prepared route of analgesic administration in home care. When patient can not take medications orally, continuous intravenous infusion provides the most consistent level of analgesia. In future, transdermal route offers a practical alternative in the hospice and home."}, {"id": "pubmed23n0090_18219", "title": "Pharmacokinetics and clinical efficacy of oral morphine solution and controlled-release morphine tablets in cancer patients.", "score": 0.008849557522123894, "content": "Twenty-three adult patients with chronic pain due to cancer completed a double-blind, randomized, two-phase crossover trial comparing plasma morphine concentrations and analgesic efficacy of oral morphine sulfate solution (MSS) and controlled-release morphine sulfate tablets (MS Contin [MSC], Purdue Frederick, Inc., Toronto, Ontario, Canada). MS Contin was given every 12 hours to all patients except those whose daily morphine dose could not be equally divided into two 12-hour doses with the tablet strengths available. MSS was given every 4 hours. Patients received both of the test drugs for at least 5 days, and, on the final day of each phase, peripheral venous blood samples for morphine analysis were obtained. Eighteen patients received MSC every 12 hours, and five received it every 8 hours. The same total daily morphine dose was given in both phases. In the 18 patients who received MSC every 12 hours, the daily morphine dose was 183.9 +/- 140.0 mg (mean +/- SD). In this group, the mean area under the curve (AUC) with MSC was 443.6 +/- 348.4 ng/ml/hour, compared with 406.8 +/- 259.7 ng/ml/hour for MSS (P greater than 0.20). Mean maximum morphine concentrations (Cmax) for MSC and MSS were 67.9 +/- 42.1 and 58.8 +/- 30.3 ng/ml, respectively (P greater than 0.05). Mean minimum morphine concentrations (Cmin) were 17.0 +/- 17.7 and 18.3 +/- 15.0, respectively (P greater than 0.30). There was a significant difference (P less than 0.001) between the two drugs in time required to reach maximum morphine concentration (Tmax). Mean Tmax after MSC occurred at 3.6 +/- 2.3 hours. After MSS, it occurred at 1.3 +/- 0.4 hours. In the five patients who received MSC every 8 hours, the findings paralleled those in the principal group, with no significant differences between MSC and MSS in Cmax or Cmin and a highly significant difference between the two in Tmax. However, in this small group of patients, the AUC with MSC was significantly (P = 0.04) greater than that with MSS. All patients had very good pain control throughout the study and both formulations were well tolerated. There were no significant differences between MSC and MSS in pain scores or side effects. Under the conditions of this study there was no clinically significant difference in bioavailability between MSC and oral MSS. When given on a 12-hourly basis in individually titrated doses, the MSC provided therapeutic plasma morphine concentrations throughout the dosing interval."}, {"id": "pubmed23n0299_3083", "title": "[What can we do for cancer patients with pain to go home].", "score": 0.008849557522123894, "content": "When patients suffering from cancer pain wish to stay at home, the most important condition is to relieve the cancer pain. But it is not enough; decreasing the fear of pain is another important problem. From these viewpoints, we have tried to assist these patients to go home. In this paper we report two cases of 61-year-old and 57-year-old women with severe leg pain caused by pelvic recurrence of cancer of the uterus and rectum. At first, we needed high doses of morphine by intravenous administration all day long. To control their mental condition, we talked personally with them to understand their distress and asked them to put in writing anything about their anxiety in a special notebook. After that, we advised them on how to use anodyne, how to deal with side effects of the drugs and how to use various social services in their home. Then, after their cancer pain was controlled by radiotherapy and appropriate anodyne, they overcame their anxiety and at last they could go home."}, {"id": "pubmed23n0478_8599", "title": "Patient-reported utilization patterns of fentanyl transdermal system and oxycodone hydrochloride controlled-release among patients with chronic nonmalignant pain.", "score": 0.008771929824561403, "content": "Although use of long-acting opioid analgesics has increased for chronic nonmalignant pain management, little is known about patient-reported utilization patterns. To assess patient-reported utilization patterns of fentanyl transdermal system and oxycodone hydrochloride (HCl) controlled-release among patients with chronic nonmalignant pain and to compare these patterns to standard dose administration guidelines recommended in the manufacturers. prescribing information (PI). Cross-sectional, observational, multicenter study of English-speaking patients who were seeking chronic nonmalignant pain management from 6 outpatient pain clinics. The inclusion criteria for the study were (1) diagnosis of chronic nonmalignant pain, (2) prescription for and current use of either transdermal fentanyl or oxycodone HCl controlled-release, and (3) duration of use for either transdermal fentanyl or oxycodone HCl controlled-release of at least 6 weeks. Patients completed either an oxycodone HCl controlled-release or transdermal fentanyl utilization questionnaire. A conversion table was used to standardize opioid analgesic doses from transdermal fentanyl or oxycodone HCl controlled-release to daily oral morphine equivalents. The principal outcome measures were the average interval between oxycodone HCl controlled-release administrations, the number of days the current transdermal fentanyl patch would be worn, and the percentage of oxycodone HCl controlled-release and transdermal fentanyl patients whose administration frequency exceeded the standard recommendation in the manufacturer.s PI (every 12 hours for oxycodone HCl controlled-release or every 72 hours for transdermal fentanyl). Other outcome measures included the number of oxycodone HCl controlled-release tablets per administration, the daily dose of long-acting opioid, the duration of adequate pain relief, and the difference in daily oral morphine equivalents between transdermal fentanyl and oxycodone HCl controlled-release patients, after adjusting in a multivariate regression model for demographic and clinical characteristics. A total of 690 patients were enrolled in this study; 437 (63.4%) received oxycodone HCl controlled-release and 253 (36.6%) received transdermal fentanyl. Oxycodone HCl controlled-release patients reported taking a median of 1 tablet 3 times per day or a median of 3 tablets per day. A mean of 1.6 tablets per administration and 4.6 tablets per day were taken. The average interval between administrations of oxycodone HCl controlled-release was 7.8 hours, and the median daily dose was 80.0 mg (mean 155.6 mg). Among oxycodone HCl controlled-release patients, 17.5% had an average interval between administrations of 12 or more hours, whereas 1.9% reported the duration of pain relief as 12 or more hours. Transdermal fentanyl patients reported wearing the patch, on average, for 2.5 days (median 2.5),and 41.2% reported wearing the patch for at least 3 days, whereas 14.1% reported the duration of pain relief as at least 3 days. The median daily dosage strength of transdermal fentanyl was 75.0 mcg/hour. In the multivariate regression analysis, oxycodone HCl controlled-release patients had, on average, roughly 22 mg additional oral morphine equivalents per day relative to transdermal fentanyl patients (not statistically significant); the probability that oxycodone HCl controlled-release patients had higher oral morphine equivalents was 82.6%, which suggests a trend toward higher oral morphine equivalents per day in the oxycodone HCl controlled-release group. Transdermal fentanyl and oxycodone HCl controlled-release both appear to be used by patients in a manner that is inconsistent with the standard recommendation in the manufacturers' PI;however, the difference between patient-reported utilization and the PI recommendation is more pronounced with oxycodone HCl controlled-release."}, {"id": "pubmed23n0287_8829", "title": "[Continuous spinal analgesia in home care of oncologic pain].", "score": 0.008771929824561403, "content": "The authors in this study, after a short survey of the most important therapeutic techniques for cancer pain, report their results in the treatment of 18 patients suffering from incurable disease. It was impossible to dismiss them from hospital care on account of a painful symptomatology not controllable by oral morphine or owing to excessive collateral morphinic consequences. The analgetic technique employed was continuous intrathecal infusion of morphine, clonidine, droperidol and, in 10 cases, bupivacaine. Drug delivery systems, totally internalized, except infusion pump, were always utilized. Adequate pain relief was obtained, within - 5 days, in all the patients. Family membres, in the same period, learnt the infusion circuit action. At this point the patients were dismissed and treated with home care. The average time of assistance was 140 days, and very moderate variations in posology were necessary. Hospital reentrance, really little numerous, happened only when no member of palliative care service was present. Reasons were no bodily pain, but the total suffering of cancer disease. No complication nor collateral consequences were never found."}, {"id": "wiki20220301en522_29798", "title": "Opioid rotation", "score": 0.00873015873015873, "content": "Opioid rotation or opioid switching is the process of changing one opioid to another to improve pain control or reduce unwanted side effects. This technique was introduced in the 1990s to help manage severe chronic pain and improve the opioid response in cancer patients. In order to obtain adequate levels of pain relief, patients requiring chronic opioid therapy may require an increase in the original prescribed dose for a number of reasons, including increased pain or a worsening disease state. Over the course of long term treatment, an increase in dosage cannot be continued indefinitely as unwanted side effects of treatment often become intolerable once a certain dose is reached, even though the pain may still not be properly managed. One strategy used to address this is to switch the patient between different opioid drugs over time, usually every few months. Opioid rotation requires strict monitoring in patients with ongoing levels of high opioid doses for extended periods of time,"}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": true, "char_ranges": [[0, 195]], "word_ranges": [[0, 31]], "text": "In the presence of isolated thrombocytopenia we must not lose sight of the physical examination; thus, the presence of lymphadenopathy forces us to rule out the presence of lymphoma, for example."}, "2": {"exist": true, "char_ranges": [[210, 422]], "word_ranges": [[34, 69]], "text": "When performing a bone marrow biopsy-aspirate it is seen that the number of megakaryocytes is normal or even increased, the failure is not in the marrow but in the peripheral blood, where platelets are destroyed."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "In the presence of isolated thrombocytopenia we must not lose sight of the physical examination; thus, the presence of lymphadenopathy forces us to rule out the presence of lymphoma, for example. 1 is correct. When performing a bone marrow biopsy-aspirate it is seen that the number of megakaryocytes is normal or even increased, the failure is not in the marrow but in the peripheral blood, where platelets are destroyed. So. The false answer is 2.", "full_answer_no_ref": "In the presence of isolated thrombocytopenia we must not lose sight of the physical examination; thus, the presence of lymphadenopathy forces us to rule out the presence of lymphoma, for example. [HIDDEN] When performing a bone marrow biopsy-aspirate it is seen that the number of megakaryocytes is normal or even increased, the failure is not in the marrow but in the peripheral blood, where platelets are destroyed. So. [HIDDEN]", "full_question": "A 33-year-old woman consults for repeated epistaxis, petechiae and ecchymosis. Laboratory tests show thrombocytopenia with a platelet count of 4000 platelets/microliter. The initial presumptive diagnosis is chronic immune thrombocytopenic purpura (ITP). Which of the following statements is FALSE regarding the diagnosis of ITP?", "id": 179, "lang": "en", "options": {"1": "The presence of lymphadenopathy or splenomegaly in the physical examination suggests a different diagnosis of ITP.", "2": "Bone marrow analysis shows a decreased number of megakaryocytes without other alterations.", "3": "Complete blood count shows isolated thrombocytopenia with often large platelets, without anemia unless there is significant bleeding or associated autoimmune hemolysis (Evans syndrome).", "4": "The diagnosis of ITP is established by exclusion of other processes causing thrombocytopenia.", "5": "The determination of antiplatelet antibodies is not accurate to establish the diagnosis."}, "question_id_specific": 98, "type": "HEMATOLOGY", "year": 2013, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en016_42764", "title": "Immune thrombocytopenic purpura", "score": 0.01951265943270512, "content": "Despite the destruction of platelets by splenic macrophages, the spleen is normally not enlarged. In fact, an enlarged spleen should lead to a search for other possible causes for the thrombocytopenia. Bleeding time is usually prolonged in ITP patients. However, the use of bleeding time in diagnosis is discouraged by the American Society of Hematology practice guidelines and a normal bleeding time does not exclude a platelet disorder. Bone marrow examination may be performed on patients over the age of 60 and those who do not respond to treatment, or when the diagnosis is in doubt. On examination of the marrow, an increase in the production of megakaryocytes may be observed and may help in establishing a diagnosis of ITP. An analysis for anti-platelet antibodies is a matter of clinician's preference, as there is disagreement on whether the 80 percent specificity of this test is sufficient to be clinically useful."}, {"id": "wiki20220301en016_42759", "title": "Immune thrombocytopenic purpura", "score": 0.01837336704593342, "content": "ITP is an autoimmune disease with antibodies detectable against several platelet surface structures. ITP is diagnosed by identifying a low platelet count on a complete blood count (a common blood test). However, since the diagnosis depends on the exclusion of other causes of a low platelet count, additional investigations (such as a bone marrow biopsy) may be necessary in some cases. In mild cases, only careful observation may be required but very low counts or significant bleeding may prompt treatment with corticosteroids, intravenous immunoglobulin, anti-D immunoglobulin, or immunosuppressive medications. Refractory ITP (not responsive to conventional treatment or constant relapsing after splenectomy) requires treatment to reduce the risk of clinically significant bleeding. Platelet transfusions may be used in severe cases with very low platelet counts in people who are bleeding. Sometimes the body may compensate by making abnormally large platelets."}, {"id": "pubmed23n0319_6679", "title": "[Idiopathic thrombocytopenic purpura in children].", "score": 0.017158294392523366, "content": "Idiopathic (immune) thrombocytopenic purpura (ITP) is the most frequent hemorrhagic disease in children. It represents the acquired megakaryocytic thrombocytopenia with the shortened life of platelets because of immunologic damage (antibodies absorbed by platelets). In the case of this acquired hemorrhagic disorder, in spite of compensatory increased function of the bone marrow, there is a reduced number of platelets because of their increased destruction by the reticuloendothelial system (destructive thrombocytopenia). There are three forms of ITP: acute, chronic and intermittent. The acute form occurs in 80-90% of cases with bleeding episodes lasting a few days or weeks, but no longer than 6 months. The chronic form occurs in 10-15% of children, while the rarest-intermittent form is characterized by periods of normalization in regard to the number of platelets but also with relapse in intervals of 1-3 months. The disease is caused by an immunological disorder in the sense of an imbalanced immune response. Immunologic damages of platelets cause shortening of the opsonized platelets life span. The most frequent platelet opsonins are the immumoglobulin G (IgG) antibodies directed at the platelet membrane in the form of autoantibodies, alloantibodies or possibly absorbed antigen caused by microorganism infection or drug intake. It is typical for the phenomenon of bleeding that it starts suddenly and without any other sign of illness. The most frequent acute form appears between the second and fourth year, and is characterized by seasonal occurrence usually after acute viral infections. Children older than 10 years of age, like adults, often have the chronic form associated with other immunologic disorders. The disease affects girls more often than boys (about three times more often) with moderate and constant increase of antiplatelet antibodies. Hemorrhagic manifestations include: petechiae, purpura, epistaxis, gastrointestinal and genitourinary bleeding. They depend on the grade of thrombocytopenia, although there is no strict correlation between the number of platelets and volume of bleeding. Low mortality of the acute ITP is almost exclusively due to intracranial hemorrhage. LABORATORY STUDIES: Thrombocytopenia represents a decrease in the number of blood platelets being a basic abnormality of the blood count. The half-life of platelets in ITP is shortened. Detection of antiplatelet antibodies is connected with technical difficulties, so they are established in about 30% of cases. Bleeding time is prolonged and so is the coagulum retraction which may be completely missed. The Rumpel-Leede test is positive. Clinical differentiation of drug-induced thrombocytopenia is not possible. However, other differential diagnostic possibilities are thrombotic-thrombocytopenic purpura and hemolytic-uremic syndrome. A child with aplastic anemia or acute leukemia, beside thrombocytopenia, has a characteristic finding of white and red blood cell count. Thrombocytopenia with absent radii syndrome is associated with skeletal system abnormalities. New knowledge about the role of the immune system in ITP determines the modern therapeutic modalities. In cases of acute ITP in children, there are two therapeutic options or therapies of choice: corticosteroids and high doses of intravenous immunoglobulin. Immunosupressive therapy means anti Rh(D) immunoglobulin, cyclosporine, cytostatics, danazol, loaded platelets. In cases of distinctive hemorrhagic syndrome there are also indications for platelet transfusion. Nowadays splenectomy is more restricted, because one third of cases is unsuccessful, whereas plasmapheresis is rarely used in children because of possible complications. ITP is the most frequent hemorrhagic disease in children. The disease is basically caused by an immunologic disorder with platelet destruction due to increased immunoglobulin on their membrane. (ABSTRACT TRUNCATED)"}, {"id": "pubmed23n0526_14831", "title": "Initial laboratory findings useful for predicting the diagnosis of idiopathic thrombocytopenic purpura.", "score": 0.016695652173913042, "content": "To identify initial laboratory findings useful for the later diagnosis of idiopathic thrombocytopenic purpura (ITP) in adult patients with thrombocytopenia. We studied 62 consecutive adult patients who had thrombocytopenia and whose peripheral blood film was normal except for thrombocytopenia at presentation. Each patient underwent physical examination and routine laboratory tests and was prospectively followed for 22.5 +/- 9.8 months (range, 8 to 41 months). The frequency of antiglycoprotein (GP) IIb/IIIa antibody-producing B cells, the presence of platelet-associated and plasma anti-GPIIb/IIIa antibodies, the percentage of reticulated platelets, and the plasma thrombopoietin level were examined at the first visit. The final diagnosis was based on the clinical history, physical examination, complete blood test, bone marrow findings, and the clinical course at last observation. Forty-six patients were diagnosed as having ITP and 16 as having another disorder, including myelodysplastic syndrome, aplastic anemia, amegakaryocytic thrombocytopenia, and reduced platelet production, with or without other cytopenias, and without dysplasia or evidence for destruction. Six initial laboratory findings discriminated ITP from other diagnoses: the absence of anemia, absence of leukocytopenia, increased frequency of anti-GPIIb/IIIa antibody-producing B cells, increased platelet-associated anti-GPIIb/IIIa antibodies, elevated percentage of reticulated platelets, and a normal or slightly increased plasma thrombopoietin level. Three or more of these ITP-associated findings were found at presentation in 44 patients (96%) with thrombocytopenia later diagnosed as ITP, compared with only 1 patient (6%) whose disorder was non-ITP. Initial laboratory findings can well predict future diagnosis of ITP. Further studies prospectively evaluating these same diagnostic criteria on another, independent set of patients are necessary."}, {"id": "pubmed23n0396_7459", "title": "The role of the bone marrow examination in the diagnosis of immune thrombocytopenic purpura: case series and literature review.", "score": 0.01579740729574917, "content": "The need for a bone marrow examination was assessed in patients with clinical and laboratory features consistent with ITP; the literature was reviewed. The records of all patients undergoing a bone marrow examination between January 1988 to January 1998 were retrospectively reviewed to determine which were motivated by the suspicion of ITP. Data were collected from hospital and outpatient medical and pathology records. Eighty-six patients with isolated thrombocytopenia (i.e., normal white blood cell count, hemoglobin, peripheral smear and clotting studies) were studied. The bone marrow was consistent with ITP in 82 patients, (i.e., normal or increased megakaryocytes and other hemopoietic lineages normal.) Four patients had decreased megakaryocytes, but all patients responded to corticosteroids. All 86 patients were followed up for a median of 22 months after bone marrow aspiration (range, 2-76 months.) During that time, none of the patients developed features to suggest an alternative diagnosis to ITP. The initial clinical and laboratory findings of 99 patients with acute leukemia were also reviewed; all had features atypical of ITP. These data suggest that routine performance of a bone marrow examination for the diagnosis of ITP is not necessary, provided that a thorough history and physical examination are performed and that the complete blood cell count, peripheral blood smear, and routine clotting studies show no abnormalities apart from thrombocytopenia. The findings of seven prior retrospective studies, two in adults and five in children are consistent with the previous findings. However, the value of marrow investigation in ITP remains unresolved and data from a large prospective study would be helpful."}, {"id": "wiki20220301en020_74489", "title": "Thrombocytopenia", "score": 0.015160118101294572, "content": "Immune thrombocytopenic purpura Many cases of immune thrombocytopenic purpura (ITP) also known as idiopathic thrombocytopenic purpura, can be left untreated, and spontaneous remission (especially in children) is not uncommon. However, counts under 50,000 are usually monitored with regular blood tests, and those with counts under 10,000 are usually treated, as the risk of serious spontaneous bleeding is high with such low platelet counts. Any patient experiencing severe bleeding symptoms is also usually treated. The threshold for treating ITP has decreased since the 1990s; hematologists recognize that patients rarely spontaneously bleed with platelet counts greater than 10,000, although exceptions to this observation have been documented."}, {"id": "pubmed23n1121_25608", "title": "Assessment and Management of Immune Thrombocytopenia (ITP) in the Emergency Department: Current Perspectives.", "score": 0.01510676965015902, "content": "Immune thrombocytopenia (ITP) is characterized by a platelet count less than 100 × 10^9/L without anemia or leukopenia. Patients with ITP may be asymptomatic, or they may have mild bleeding like petechiae, purpura, or epistaxis. In rare cases, they may present to the emergency department (ED) with life-threatening bleeding as a result of their thrombocytopenia. The emergency physician should thus be prepared to diagnose ITP and treat the bleeding that can result from it. The diagnosis of ITP requires excluding secondary causes of thrombocytopenia, and in the ED, the bare minimum workup for ITP includes a complete blood count and a peripheral blood smear. The peripheral blood smear should show a small number of large platelets with normal morphology, and there should not be an increased number of schistocytes. Many patients with ITP require no emergent treatment. However, if a patient with suspected ITP presents to the ED with critical hemorrhage, the emergency physician should initiate treatment with a platelet transfusion, corticosteroids, and intravenous immune globulin (IVIG) as soon as possible. For less severe bleeding, platelet transfusions are not recommended, and the treatment consists of corticosteroids by themselves or in conjunction with IVIG."}, {"id": "pubmed23n0416_22915", "title": "[The diagnosis of ITP].", "score": 0.014509285029885476, "content": "Idiopathic thrombocytopenic purpura(ITP) is a hematologic disorder which causes thrombocytopenia. The diagnosis of ITP is based on the history, physical examination and, complete blood count, and examination of the peripheral smear. The diagnostic criteria of ITP established by the Ministry of Health, Welfare, and Labor in Japan requires the bone marrow examination and the measurement of platelet associated IgG, but those tests are not always necessary according to the guidelines developed by the American Society of Hematology. The appropriate strategies for the diagnosis of ITP need to be established. In this paper, some new examinations which may help the diagnosis of ITP are also demonstrated."}, {"id": "wiki20220301en016_42758", "title": "Immune thrombocytopenic purpura", "score": 0.013782051282051282, "content": "Immune thrombocytopenic purpura (ITP), also known as idiopathic thrombocytopenic purpura or immune thrombocytopenia, is a type of thrombocytopenic purpura defined as an isolated low platelet count with a normal bone marrow in the absence of other causes of low platelets. It causes a characteristic red or purple bruise-like rash and an increased tendency to bleed. Two distinct clinical syndromes manifest as an acute condition in children and a chronic condition in adults. The acute form often follows an infection and spontaneously resolves within two months. Chronic immune thrombocytopenia persists longer than six months with a specific cause being unknown. ITP is an autoimmune disease with antibodies detectable against several platelet surface structures."}, {"id": "pubmed23n0980_11344", "title": "Immune thrombocytopenic purpura.", "score": 0.0135122838944495, "content": "Immune thrombocytopenic purpura (ITP) is a bleeding disorder characterized by isolated thrombocytopenia (platelet count &lt;150,000 u/L), which is not associated with a systemic illness. ITP is reported in approximately 2 per 100,000 adults. The mean age of diagnosis is 50 years. ITP is more common in females of childbearing age and in pregnancy. In adults, the course is more chronic although spontaneous remission can also occur within months of initial diagnosis. A thorough and timely workup of thrombocytopenia is imperative to rule out other differentials of ITP as it is considered a diagnosis of exclusion. Primary care physicians encounter patients who exhibit signs of thrombocytopenia such as petechiae or purpura on a regular basis. A high index of clinical suspicion is required to accurately diagnose ITP and commence the appropriate treatment including glucocorticoids to increase the chances of a favorable prognosis as described by the authors."}, {"id": "pubmed23n1009_24869", "title": "Teetering on a liver's edge: a case report highlighting clinical decision-making in thrombocytopenia.", "score": 0.013461369814960975, "content": "This report illustrates the importance of a detailed history and physical exam and careful analysis of hematologic parameters when diagnosing ITP. This case demonstrates that even with subtle deviations from typical ITP findings one must promptly reevaluate the diagnosis. This case also highlights the importance of peripheral smear review by an expert in pediatric hematopathology. A previously healthy 10 year-old Asian boy presented with 2 months of easy bruising. Review of systems was negative for any constitutional symptoms. On examination, he appeared well but had numerous large ecchymoses. He had no appreciable lymphadenopathy or splenomegaly. The liver was palpable 1.5 cm below the costal margin. A complete blood count (CBC) showed: platelets = 17 × 109/L, hemoglobin = 128 g/L, white blood cell count = 5.43 × 109/L, and neutrophils = 1.63 × 109/L. A blood smear was reported as normal. Urate was 370 umol/L and lactate dehydrogenase (LDH) was 803 U/L. The child was admitted with a presumptive diagnosis of immune thrombocytopenic purpura (ITP) and treated with intravenous immunoglobulin. The following day, the blood smear was reviewed by a hematopathologist who identified blasts. A bone marrow aspiration (BMA) confirmed the diagnosis of precursor B-cell acute lymphoblastic leukemia. In children presenting with suspected ITP, leukemia should always be considered. A BMA was historically performed on all patients with presumed ITP to rule out leukemia. In 2011, the American Society of Hematology (ASH) stopped recommending routine BMA in patients suspected of having ITP. ASH advises in cases with unusual findings on history, physical examination or CBC, it is reasonable to perform a BMA. Our patient had mild hepatomegaly, which may have qualified him for a BMA. He also had an elevated LDH and urate, which are not listed as criteria for BMA by ASH but were considered atypical for ITP by the clinical team. A literature search did not reveal any primary data assessing these markers. While corticosteroids are a first line treatment in ITP, they must be reserved for when clinicians are confident that the patient does not have leukemia. Steroid administration prior to diagnosing leukemia results in delayed diagnosis and may increase the risk of complications and decrease survival."}, {"id": "pubmed23n0233_63", "title": "Present-day problems of diagnosis and treatment in the idiopathic thrombocytopenic purpura.", "score": 0.013105389171252153, "content": "A review is made of the data in the literature and of the authors' experience regarding the etiology and pathogenesis, diagnosis and therapy of the chronic form of idiopathic thrombocytopenic purpura (ITP). The mechanisms of production (after McMillan et al.) are presented schematically and the five criteria of ITP diagnosis suggested by Karpatkin are discussed: 1) decreased blood platelet count with direct or indirect signs of thrombocytolysis; 2) increased number of megakaryocytes in the bone marrow and/or signs of intramedullary thrombocytolysis; 3) direct or indirect signs of antiplatelet autoantibody presence in the plasma; 4) exclusion of a primary disorder and 5) absence of splenomegaly. The results of corticotherapy, splenectomy, platelet transfusion and immunosuppression (including \"target\" immunosuppressive therapy) in 188 patients with ITP, admitted to the clinic of Hematology--Bucharest between 1966 and 1978, are presented and analysed."}, {"id": "wiki20220301en016_42781", "title": "Immune thrombocytopenic purpura", "score": 0.012842166569598136, "content": "It is recommended that pregnant women with thrombocytopenia or a previous diagnosis of ITP should be tested for serum antiplatelet antibodies. A woman with symptomatic thrombocytopenia and an identifiable antiplatelet antibody should be started on therapy for their ITP which may include steroids or IVIG. Fetal blood analysis to determine the platelet count is not generally performed as ITP-induced thrombocytopenia in the fetus is generally less severe than NAIT. Platelet transfusions may be performed in newborns, depending on the degree of thrombocytopenia. It is recommended that neonates be followed with serial platelet counts for the first few days after birth."}, {"id": "First_Aid_Step2_374", "title": "First_Aid_Step2", "score": 0.01283068783068783, "content": "ITP is associated with a range of conditions, including lymphoma, leukemia, SLE, HIV, and HCV. The clinical presentation is as follows: Acute: Abrupt onset of hemorrhagic complications following a viral illness. Commonly affects children 2–6 years of age, with males and females affected equally. Chronic: Insidious onset that is unrelated to infection. Most often affects adults 20–40 years of age; women are three times more likely to be affected than men. A diagnosis of exclusion, as the test for platelet-associated antibodies is a poor one. Once other causes of thrombocytopenia have been ruled out, a diagnosis can be made on the basis of the history and physical, a CBC, and a peripheral blood smear showing normal RBC morphology. Most patients do not require bone marrow biopsy, which would show ↑ megakaryocytes as the only abnormality. Most patients with acute childhood ITP spontaneously remit, but this is rarely the case in chronic ITP."}, {"id": "wiki20220301en016_42763", "title": "Immune thrombocytopenic purpura", "score": 0.012776222526654356, "content": "Diagnosis The diagnosis of ITP is a process of exclusion. First, it has to be determined that there are no blood abnormalities other than a low platelet count, and no physical signs other than bleeding. Then, secondary causes (5–10 percent of suspected ITP cases) should be excluded. Such secondary causes include leukemia, medications (e.g., quinine, heparin), lupus erythematosus, cirrhosis, HIV, hepatitis C, congenital causes, antiphospholipid syndrome, von Willebrand factor deficiency, onyalai and others. All patients with presumed ITP should be tested for HIV and hepatitis C virus, as platelet counts may be corrected by treating the underlying disease. In approximately 2.7 to 5 percent of cases, autoimmune hemolytic anemia and ITP coexist, a condition referred to as Evans syndrome."}, {"id": "pubmed23n0950_231", "title": "Immune Thrombocytopenic Purpura Detected with Oral Hemorrhage: a Case Report.", "score": 0.012767425810904072, "content": "Immune thrombocytopenic purpura (ITP) is an immune-mediated acquired disease found in both adults and children. It is characterized by transient or persistent decreases in the platelet count. We report a case of ITP detected based on oral hemorrhagic symptoms. The patient was a 79-year-old female with no significant past medical history. She presented with sudden onset of gingival bleeding and hemorrhagic bullae on the buccal mucosa. Gingival bleeding was difficult to control. Laboratory tests revealed severe thrombocytopenia with a platelet count as low as 2000/μL. Under a provisional diagnosis of a hematological disorder, she was referred to a hematologist. A peripheral smear showed normal-sized platelets. A bone marrow examination revealed increased numbers of megakaryocytes without morphologic abnormalities. The patient was diagnosed with ITP and treated with a combination of pulsed steroid therapy and high-dose immunoglobulin therapy. However, her severe thrombocytopenia was refractory to these treatments. Then, a thrombopoietin receptor agonist was begun as a second-line treatment. Her platelets rapidly increased, and no bleeding complications were reported. Because oral symptoms can be one of the initial manifestations of ITP, dentists should be familiar with the clinical appearance of ITP, and attention must be paid to detect and diagnose unidentified cases."}, {"id": "pubmed23n1154_18040", "title": "Development and internal validation of a clinical prediction model for the diagnosis of immune thrombocytopenia.", "score": 0.012743506493506494, "content": "Immune thrombocytopenia (ITP) is a diagnosis of exclusion that can resemble other thrombocytopenic disorders. To develop a clinical prediction model (CPM) for the diagnosis of ITP to aid hematogists in investigating patients presenting with undifferentiated thrombocytopenia. We designed a CPM for ITP diagnosis at the time of the initial hematology consultation using penalized logistic regression based on data from patients with thrombocytopenia enrolled in the McMaster ITP registry (n = 523) called the Predict-ITP Tool. The case definition for ITP was a platelet count less than 100 × 10<sup9</sup /L and a platelet count response after high-dose corticosteroids or intravenous immune globulin, defined as the achievement of a platelet count above 50 × 10<sup9</sup /L and at least a doubling of baseline. Internal validation was done using bootstrap resampling. Model discrimination was assessed by the c-statistic, and calibration was assessed by the calibration slope, calibration-in-the-large, and calibration plot. The final model included the following variables: (1) platelet count variability (based on three or more platelet count values), (2) lowest platelet count value, (3) maximum mean platelet volume, and (4) history of major bleeding (defined by the ITP bleeding scale). The optimism-corrected c-statistic was 0.83, the calibration slope was 0.88, and calibration-in-the-large for all performance measures was &lt;0.001 with standard error &lt;0.001, indicating good discrimination and excellent calibration. The Predict-ITP Tool can estimate the likelihood of ITP for a given patient with thrombocytopenia at the time of the initial hematology consultation. The tool had high predictive accuracy for the diagnosis of ITP."}, {"id": "InternalMed_Harrison_9145", "title": "InternalMed_Harrison", "score": 0.0126529244176303, "content": "laboratory testing in itp Laboratory testing for antibodies (serologic testing) is usually not helpful due to the low sensitivity and specificity of the current tests. Bone marrow examination can be reserved for those who have other signs or laboratory abnormalities not explained by ITP or in patients who do not respond to initial therapy. The peripheral blood smear may show large platelets, with otherwise normal morphology. Depending on the bleeding history, iron-deficiency anemia may be present."}, {"id": "InternalMed_Harrison_9146", "title": "InternalMed_Harrison", "score": 0.01227698286521816, "content": "Laboratory testing is performed to evaluate for secondary causes of ITP and should include testing for HIV infection and hepatitis C (and other infections if indicated). Serologic testing for SLE, serum protein electrophoresis, immunoglobulin levels to potentially detect hypogammaglobulinemia, selective testing for IgA deficiency or monoclonal gammopathies, and testing for H. pylori infection should be considered, depending on the clinical circumstance. If anemia is present, direct antiglobulin testing (Coombs’ test) should be performed to rule out combined autoimmune hemolytic anemia with ITP (Evans’ syndrome). The treatment of ITP uses drugs that decrease reticuloendothelial uptake of the antibody-bound platelet, decrease antibody production, and/or increase platelet production. The diagnosis of ITP does not necessarily mean that treatment must be instituted. Patients with platelet counts >30,000/μL appear not to have increased mortality related to the thrombocytopenia."}, {"id": "wiki20220301en016_42780", "title": "Immune thrombocytopenic purpura", "score": 0.012015221017514597, "content": "Pregnancy Anti-platelet autoantibodies in a pregnant woman with ITP will attack the patient's own platelets and will also cross the placenta and react against fetal platelets. Therefore, ITP is a significant cause of fetal and neonatal immune thrombocytopenia. Approximately 10% of newborns affected by ITP will have platelet counts <50,000/uL and 1% to 2% will have a risk of intracerebral hemorrhage comparable to infants with neonatal alloimmune thrombocytopenia (NAIT). No lab test can reliably predict if neonatal thrombocytopenia will occur. The risk of neonatal thrombocytopenia is increased with: Mothers with a history of splenectomy for ITP Mothers who had a previous infant affected with ITP Gestational (maternal) platelet count less than 100,000/uL"}, {"id": "wiki20220301en010_97374", "title": "Platelet", "score": 0.011666458930574598, "content": "Disorders Adapted from: The three broad categories of platelet disorders are \"not enough\"; \"dysfunctional\"; and \"too many\". Thrombocytopenia Immune thrombocytopenias (ITP) – formerly known as immune thrombocytopenic purpura and idiopathic thrombocytopenic purpura Splenomegaly Gaucher's disease Familial thrombocytopenia Chemotherapy Babesiosis Dengue fever Onyalai Thrombotic thrombocytopenic purpura HELLP syndrome Hemolytic–uremic syndrome Drug-induced thrombocytopenic purpura (five known drugs – most problematic is heparin-induced thrombocytopenia (HIT) Pregnancy-associated Neonatal alloimmune associated Aplastic anemia Transfusion-associated Pseudothrombocytopenia idiopathic thrombocytopenic purpura Vaccine induced immune thrombocytopenia Gilbert's syndrome"}, {"id": "wiki20220301en046_45191", "title": "Evans syndrome", "score": 0.011654511654511654, "content": "Causes Although Evans syndrome seems to be a disorder of immune regulation, the exact pathophysiology is unknown, but a gradual loss of self-tolerance is postulated. Autoantibodies targeted at different antigenic determinants on red cells and platelets are assumed to cause isolated episodes of hemolytic anemia and thrombocytopenia, respectively. Diagnosis The diagnosis of primary Evans syndrome is made upon blood tests to confirm not only hemolytic anemia and immune thrombocytopenic purpura, but also a positive direct antiglobulin test (DAT) and an absence of any known underlying cause. In 27% to 50% of cases there is an associated malignancy or a predisposing autoimmune disease (e.g. systemic lupus erythematosus), it is then common to denote it as secondary Evans syndrome. Other antibodies may occur directed against neutrophils and lymphocytes, and \"immunopancytopenia\" has been suggested as a term for this syndrome."}, {"id": "pubmed23n0660_1139", "title": "[Myelodysplastic syndrome mimicking idiopathic thrombocytopenic purpura].", "score": 0.011628300037183518, "content": "In patients with isolated thrombocytopenia, but without significant dysplasia, diagnosis of idiopathic thrombocytopenic purpura (ITP) rather than myelodysplastic syndrome (MDS) may be taken into account. It is important to make an accurate diagnosis because different treatments are used for ITP and MDS. The purpose of this study was to investigate the clinical and hematologic features of patients who were initially diagnosed as ITP but had cytogenetic abnormalities. We retrospectively reviewed cytogenetic studies of 100 patients who were diagnosed as ITP from 2004 to 2009 at Mokdong Hospital of Ewha Womans University based on clinical features and hematologic studies. Bone marrow pathology was re-evaluated based on 2008 WHO classification. Cytogenetic analysis was performed by 24-48 hr culture of bone marrow aspirates without using mitogens and 20 metaphases were analyzed. Of the 100 patients diagnosed as ITP initially, three patients (3%) had cytogenetic abnormalities. They had no thrombocytopenia-related symptoms and thrombocytopenia was found accidentally. The numbers of megakaryocytes in bone marrow were increased and dysplasia was not found in megakaryocyte, erythroid, and myeloid cell lineages. The proportion of blasts was within normal limits. Clonal chromosomal abnormalities found were der(1;7)(q10;p10), add(9)(q12), or t(7;11)(p22;q12). Presumptive diagnosis of MDS or diagnosis of idiopathic cytopenia of undetermined significance (ICUS) was made according to 2008 WHO classification. During the follow up, disease progression was not found. In patients with suspected ITP, cytogenetic analysis should be done. If specific clonal chromosomal abnormality is found, presumptive diagnosis of MDS has to be considered and close follow up is needed."}, {"id": "wiki20220301en192_930", "title": "Harrington–Hollingsworth experiment", "score": 0.011321078361302495, "content": "The Harrington–Hollingsworth experiment was an experiment that established the autoimmune nature of the blood disorder immune thrombocytopenic purpura. It was performed in 1950 by the academic staff of Barnes-Jewish Hospital in St. Louis, Missouri. Experiment The experiment was undertaken in 1950 by William J. Harrington and James W. Hollingsworth, who postulated that in patients with idiopathic thrombocytopenic purpura (ITP), it was a blood factor that caused the destruction of platelets. To test this hypothesis, Harrington received 500 ml of blood from a patient with ITP. Within three hours, his platelets dropped to dangerously low levels and he experienced a seizure. His platelet count remained extremely low for four days, finally returning to normal levels by the fifth day. Bone marrow biopsy from Harrington's sternum demonstrated normal megakaryocytes, the cells necessary for platelet production."}, {"id": "Pediatrics_Nelson_3266", "title": "Pediatrics_Nelson", "score": 0.01130952380952381, "content": "Diagnosis. The diagnosis of ITP usually is based on clinical presentation and the platelet count and does not often require a bone marrow examination. If atypical findings are noted, however, marrow examination is indicated to rule out an infiltrative disorder (leukemia) or an aplastic process (aplastic anemia). In ITP, an examination of the bone marrow reveals increased megakaryocytes and normal erythroid and myeloid elements."}, {"id": "wiki20220301en016_42777", "title": "Immune thrombocytopenic purpura", "score": 0.011308439587128112, "content": "Epidemiology A normal platelet count is considered to be in the range of 150,000–450,000 per microlitre (μl) of blood for most healthy individuals. Hence one may be considered thrombocytopenic below that range, although the threshold for a diagnosis of ITP is not tied to any specific number. The incidence of ITP is estimated at 50–100 new cases per million per year, with children accounting for half of that number. At least 70 percent of childhood cases will end up in remission within six months, even without treatment. Moreover, a third of the remaining chronic cases will usually remit during follow-up observation, and another third will end up with only mild thrombocytopenia (defined as a platelet count above 50,000). A number of immune related genes and polymorphisms have been identified as influencing predisposition to ITP, with FCGR3a-V158 allele and KIRDS2/DL2 increasing susceptibility and KIR2DS5 shown to be protective."}, {"id": "wiki20220301en020_74487", "title": "Thrombocytopenia", "score": 0.011154115746807667, "content": "In severe thrombocytopenia, a bone marrow study can determine the number, size, and maturity of the megakaryocytes. This information may identify ineffective platelet production as the cause of thrombocytopenia and rule out a malignant disease process at the same time. Treatment Treatment is guided by the severity and specific cause of the disease. Treatment focuses on eliminating the underlying problem, whether that means discontinuing drugs suspected to cause it or treating underlying sepsis. Diagnosis and treatment of serious thrombocytopenia is usually directed by a hematologist. Corticosteroids may be used to increase platelet production. Lithium carbonate or folate may also be used to stimulate platelet production in the bone marrow. Platelet transfusions Platelet transfusions may be suggested for people who have a low platelet count due to thrombocytopenia."}, {"id": "wiki20220301en435_34729", "title": "Upshaw–Schulman syndrome", "score": 0.011148522259633371, "content": "Diagnosis A diagnosis of TTP is based on the clinical symptoms with the concomitant presence of thrombocytopenia (platelet count below 100×109/L) and microangiopathic hemolytic anemia with schistocytes on the blood smear, a negative direct antiglobulin test (Coombs test), elevated levels of hemolysis markers (such as total bilirubin, LDH, free hemoglobin, and an unmeasurable haptoglobin), after exclusion of any other apparent cause. USS can present similar to the following diseases, which have to be excluded: fulminant infections, disseminated intravascular coagulation, autoimmune hemolytic anemia, Evans syndrome, the typical and atypical form of hemolytic uremic syndrome, HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome, pre-eclampsia, heparin-induced thrombocytopenia, cancer that is often accompanied with metastasis, kidney injury, antiphospholipid antibody syndrome, and side effects from hematopoietic stem cell transplantation."}, {"id": "article-29382_20", "title": "Physiology, Spleen -- Clinical Significance", "score": 0.010940976312001048, "content": "ITP appears to be an autoimmune condition resulting in thrombocytopenia, petechiae/purpura, and bleeding from mucosal surfaces. There have been many antibodies implicated in the pathogenesis of ITP with the two main autoantibodies being against IgG and the glycoprotein (GP) IIb/IIIa complex on platelets. Platelets tagged by these autoantibodies then get broken down by phagocytes in the spleen, resulting in thrombocytopenia. Without platelets to form the platelet plug, which is the first step of hemostasis, the patient becomes more susceptible to bleeding. Most patients with ITP only have minor bleeds, such as nosebleeds or conjunctival bleeds. However, these patients are at risk of severe bleeds, and that risk helps guide management. ITP tends to affect children around five years old or older adults. In children, ITP typically presents with a sudden onset, usually following a microbial illness and is brief. In adults, it typically presents with insidious onset and is a chronic condition. In both patient populations, there must be a high index of suspicion, and ITP is usually a diagnosis of exclusion. A CBC and peripheral blood smear are typically performed in the initial evaluation. In adults, it is essential to rule out ITP secondary to other autoimmune diseases, so an anti-nuclear antibody (ANA) and autoantibodies specific for other conditions may be tested as well. A more specific test for ITP would be autoantibodies against IgG and GP IIb/IIIa. In children, treatment is generally conservative, as ITP is typically transient. Adults usually require treatment. Prednisone is the first-line treatment in adults and can also be used to treat children. IVIG can also be used to reduce or eliminate causative autoantibodies and is typically used before steroids in children and after steroids in adults. In cases refractory to medical management, a splenectomy is an option as most platelet breakdown occurs there. [28]"}, {"id": "pubmed23n0896_11785", "title": "Role of <i>Helicobacter pylori</i> Eradication Therapy on Platelet Recovery in Chronic Immune Thrombocytopenic Purpura.", "score": 0.010840824960338447, "content": "<iBackground</i. Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder in which the immune system destroys native platelets. In this condition an autoantibody is generated against a platelet antigen. ITP affects women more often than men and is more common in children than adults. <iObjective</i. To assess the effect of <iHelicobacter pylori</i eradication therapy (HPET) on platelet count in <iHelicobacter pylori</i associated chronic immune thrombocytopenic purpura (chronic ITP) in adult. <iMaterials and Methods</i. It is an interventional prospective study conducted at Liaquat University of Medical and Health Sciences, Jamshoro, from 2014 to 2015. A set of 85 patients diagnosed with chronic ITP were included in the study via convenient sampling. Patients with platelets count &lt; 100 × 10<sup9</sup/L for &gt;3 months were selected. They were posed to first-line investigations which comprised complete blood count (CBC) and peripheral blood smear examination followed by second-line tests including bone marrow examination and <iHelicobacter pylori</i stool specific antigen (HpSA-EIA). Standard <iH. pylori</i eradication therapy was offered and the patients were assessed at regular intervals for 6 months. <iResults.</i Of the 85 study patients, 32 (37.6%) were male and 53 (62.3%) were female. Mean ages of <iH. pylori</i positive and negative subjects were 43.89 ± 7.06 and 44.75 ± 7.91 years, respectively. Bone marrow examination confirmed the diagnosis and excluded other related BM disorders. <iH. pylori</i stool antigen (HpSA) was detected in 34 (40%) patients and hence regarded as <iH. pylori</i positive; the rest were negative. Treatment with eradication therapy significantly improved the mean platelet counts from 48.56 ± 21.7 × 10<sup9</sup/l to 94.2 ± 26.8 × 10<sup9</sup/l. <iConclusion.</i We concluded that the <ianti-H. pylori</i eradication therapy improves blood platelet counts in chronic immune thrombocytopenia."}, {"id": "InternalMed_Harrison_9121", "title": "InternalMed_Harrison", "score": 0.010612180248673478, "content": "Thrombocytopenia results from one or more of three processes: (1) decreased bone marrow production; (2) sequestration, usually in an enlarged spleen; and/or (3) increased platelet destruction. Disorders of production may be either inherited or acquired. In evaluating a patient with thrombocytopenia, a key step is to review the peripheral blood smear and to first rule out “pseudothrombocytopenia,” particularly in a patient without an apparent cause for the thrombocytopenia. Pseudothrombocytopenia (Fig. 140-1B) is an in vitro artifact resulting from platelet agglutination via antibodies (usually IgG, but also IgM and IgA) when the calcium content is decreased by blood collection in ethylenediamine tetraacetic (EDTA) (the anticoagulant present in tubes [purple top] used to collect blood for complete blood counts [CBCs]). If a low platelet count is obtained in EDTA-anticoagulated blood, a blood smear should be evaluated and a platelet count determined in blood collected into sodium"}, {"id": "article-30098_40", "title": "ITP-Immune Thrombocytopenic Purpura -- Differential Diagnosis", "score": 0.010482734319943622, "content": "One should consider the following in the differential thrombocytopenias due to increased platelet destruction and those due to decreased platelet production. [1] [24] [25]"}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[24, 237]], "word_ranges": [[2, 40]], "text": "It is a typical case of deep endometriosis, with implants in different parts of the pelvis. The definitive diagnosis will be given by the pathological anatomy, so for this we have to do Laparoscopy and send to PA."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Diagnostic laparoscopy. It is a typical case of deep endometriosis, with implants in different parts of the pelvis. The definitive diagnosis will be given by the pathological anatomy, so for this we have to do Laparoscopy and send to PA.", "full_answer_no_ref": "Diagnostic laparoscopy. It is a typical case of deep endometriosis, with implants in different parts of the pelvis. The definitive diagnosis will be given by the pathological anatomy, so for this we have to do Laparoscopy and send to PA.", "full_question": "A 27-year-old woman referred to the gynecology office for evaluation referring dyspareunia for about 8 months, along with dyschezia and occasional rectorrhagia coinciding with menstruation for 3-4 months. She also reports dysmenorrhea for years, which she controls well with ibuprofen. She has been trying to get pregnant for 16 months without success. In the gynecological examination she only has pain when pressing on the posterior vaginal fornix. Which test do you consider would allow you to reach a diagnosis of certainty of her pathology?", "id": 353, "lang": "en", "options": {"1": "Transvaginal ultrasound.", "2": "Diagnostic laparoscopy.", "3": "Magnetic resonance imaging.", "4": "Colonoscopy.", "5": NaN}, "question_id_specific": 161, "type": "GYNECOLOGY AND OBSTETRICS", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1003_10794", "title": "Sonographic Evaluation for Endometriosis in Routine Pelvic Ultrasound.", "score": 0.01204004329004329, "content": "To show how the evaluation for endometriosis can be included in the routine pelvic ultrasound examination. Stepwise narrated video demonstration of the sonographic evaluation for endometriosis in routine pelvic ultrasound following the recommended sonographic approach published in the 2016 consensus paper by the International Deep Endometriosis Analysis (IDEA) group [1]. Endometriosis is a common and often debilitating gynecological disorder that affects 5-10% of women [2]. The prevalence is even higher among women with symptoms of endometriosis [2], which include chronic pelvic pain, acquired dysmenorrhea, dyspareunia, dyschezia, menorrhagia, abnormal bleeding, and infertility. Approximately 80% of women who have endometriosis have superficial lesions, whereas 20% have deep infiltrating endometriosis (DIE; [3]). Laparoscopy is the gold standard for diagnosing endometriosis, because it allows the diagnosis of all forms of endometriosis and often immediate removal of superficial endometriosis. The removal of DIE is considerably more complicated and usually cannot be completed unless it was diagnosed preoperatively. The technique to diagnose DIE with transvaginal ultrasound (TVUS) was first described in detail in 2009 [4]. Since then, the accuracy of TVUS for the prediction of DIE has been well established in the literature [5-7]. TVUS is widely used as a first-line investigation for women with gynecological symptoms. The inclusion of an assessment for endometriosis in the routine pelvic ultrasound allows earlier diagnosis and better surgical outcomes for all women with DIE. The evaluation for endometriosis in routine pelvic ultrasound based on the IDEA consensus promotes a 4-step dynamic ultrasound approach [1]: (1) routine evaluation of uterus and adnexa with particular attention for sonographic signs of adenomyosis and the presence or absence of endometriomas; (2) evaluation of transvaginal sonographic 'soft markers' such as site-specific tenderness and ovarian mobility; (3) assessment of status of pouch of Douglas using the real-time ultrasound-based \"sliding sign;\" and (4) assessment of DIE nodules in the anterior and posterior compartments, which involves assessment of the bladder, vaginal vault, uterosacral ligaments, and bowel, including rectum, rectosigmoid junction, and sigmoid colon. Because 5-10% of women with DIE also have ureteric endometriosis, it is useful to assess the kidneys. Silent hydronephrosis is easily identified in 50-60% of patients with ureteric involvement. Although it is possible to identify DIE involving the ureters more directly, this requires more advanced skills, and further studies are still needed to better define the accuracy of ureteric DIE detection by TVUS [8-10]. Traditionally, only pathologies of the uterus and ovaries are assessed during a routine pelvic ultrasound. Here we demonstrate that the routine ultrasound examination can easily be extended beyond the uterus and ovaries into the posterior and anterior pelvic compartments to evaluate structural mobility and to look for deep infiltrating endometriotic nodules, wherewith women suffering from DIE can benefit from a preoperative diagnosis and subsequently, a single, well-planned procedure in the hands of a well-prepared team."}, {"id": "Gynecology_Novak_91", "title": "Gynecology_Novak", "score": 0.010102776320646578, "content": "Adolescent Patients A pelvic examination may be less revealing in an adolescent than in an older woman, particularly if it is the patient’s first examination or if it takes place on an emergency basis. An adolescent who presents with excessive bleeding should have a pelvic examination if she had intercourse, if the results of a pregnancy test are positive, if she has abdominal pain, if she is markedly anemic, or if she is bleeding heavily enough to compromise hemodynamic stability. The pelvic examination occasionally may be deferred in young teenagers who have a classic history of irregular cycles soon after menarche, who have normal hematocrit levels, who deny sexual activity, and who will reliably return for follow-up. A pelvic examination may be deferred in adolescents who present to the office requesting oral contraceptives before the initiation of intercourse or at the patient’s request, even if she has had intercourse. Newer testing methods using DNA amplification techniques"}, {"id": "pubmed23n1076_23833", "title": "Surgical treatment of deep endometriosis with adenomyosis externa: a challenging case in an infertile woman.", "score": 0.009900990099009901, "content": "To describe the management and the fertility-enhancing potential of surgery in an infertile patient with deep-infiltrating endometriosis and adenomyosis externa. Video case report. Minimally invasive and robotic gynecologic surgery unit of a university hospital. A 31-year-old nulliparous patient with dysmenorrhea, dysuria, dyspareunia, and primary infertility. Bimanual examination, transvaginal ultrasound, and magnetic resonance imaging (MRI) were performed as a comprehensive preoperative workup. The findings were consistent with bladder endometriosis and a 4-cm right pararectal cystic mass suggestive of adenomyosis externa. Laparoscopic excision of all visible endometriosis was performed. A pararectal lesion was found, completely developing in the retroperitoneal spaces, from the right medial pararectal space to the rectovaginal space, reaching the pelvic floor fascia without infiltration of the levator ani muscle. According to Koninckx classification, this kind of lesion corresponds to type III endometriosis or adenomyosis externa. Nerve-sparing eradication of the nodule was performed. The decision to use these techniques was taken with the intention to treat the patient, and not with the aim of testing the procedures performed. Therefore, as a common clinical practice in our institution and for the above reasons, there was no need for consultation of the institutional review board for approval. Improvement of symptoms and spontaneous conception after surgical removal of all endometriotic implants. There were no intraoperative or postoperative complications, and the patient was discharged after 3 days. She discontinued postoperative hormone therapy with gonadotropin-releasing hormone analogue after 3 months because she desired fertility. She conceived spontaneously after 2 months of attempting. She delivered vaginally and had no complications during pregnancy and labor. Neither recurrence of pain symptoms nor voiding or rectal dysfunctions were reported by the patient. In the management of a case of deep endometriosis, the preoperative assessment should be carefully carried out to give the surgeon the most accurate information about the extent of the disease and the patient's main objectives. Imaging techniques such as ultrasound and MRI play a fundamental role along with the clinical evaluation in also detecting lesions that are not visible at first laparoscopic inspection. In this case of a young woman without any detectable fertility issues except for endometriosis, the laparoscopic excision of endometriosis was feasible, safe, and effective in improving the patient's fertility and pain symptoms. The fertility-enhancing potential of complete eradication of pelvic endometriosis, including removal of deep posterior localizations such those presented in this case, has been hypothesized by various investigators. It has been suggested that skilled surgical management for symptomatic deep endometriosis may be followed by a high pregnancy rate, with most pregnancies resulting from postoperative natural conception even in patients with primary infertility."}, {"id": "Gynecology_Novak_2406", "title": "Gynecology_Novak", "score": 0.009900990099009901, "content": "The uterus is typically diffusely enlarged, although usually less than 14 cm in size, and is often soft and tender, particularly at the time of menses. Mobility of the uterus is not restricted, and there is no associated adnexal pathology (48). Adenomyosis is a clinical diagnosis. Imaging studies including pelvic ultrasound or MRI, although helpful, are not definitive. Because of the cost of MRI and negligible improvement in diagnostic accuracy, this study is not recommended routinely. In women with diffuse uterine enlargement and negative pregnancy test results, secondary dysmenorrhea may be attributed to adenomyosis; however, the pathologic confirmation of suspected adenomyosis can be made only at the time of hysterectomy."}, {"id": "pubmed23n0891_9621", "title": "Rectovaginal Splenosis: An Unexpected Cause of Dyspareunia Approached by Laparoscopy.", "score": 0.00980392156862745, "content": "To demonstrate the technique of laparoscopic approach in a rare case of rectovaginal splenosis with severe dyspareunia and dyschesia. A step-by-step explanation of the patient's condition, diagnosis, surgical technique, and postoperative results (Canadian Task Force classification II-3). Splenosis consists of ectopic functioning splenic tissue that can be located anywhere within the abdomen or pelvis. Fragments are often multiple and range in diameter from a few millimeters to a few centimeters. They are reddish-blue and are sessile or pedunculated. Their appearance can mimic that of neoplasms or endometriosis, which are the main differential diagnoses. Trauma and subsequent splenectomy is the cause in most cases. Splenosis is a benign condition usually found incidentally and is usually asymptomatic. The need for therapy is controversial, and treatment is suggested only in symptomatic cases, primarily those related to pelvic or abdominal lesions, as in our patient. The diagnosis of splenosis in a woman complaining of pelvic pain may present diagnostic difficulties. The splenic tissue has the macroscopic appearance of endometriosis, and its position in the pelvis also may suggest this diagnosis. Where excision of splenosis is considered necessary, the approach should be laparoscopic, unless this is considered too risky owing to the proximity of vital structures. A 40-year-old woman was referred to our department for severe dyspareunia and dyschezia. The gynecologic examination revealed a painfull nodularity on the posterior vaginal cul de sac. Further evaluation with 2- and 3-dimensional ultrasound and magnetic resonance imaging revealed several soft tissue nodules in the pouch of Douglas (POD), which were enhanced on contrast administration. She had undergone a splenectomy 15 years earlier after a car accident. A laparoscopic approach to a rectovaginal nodularity was performed. Under general anesthesia, the patient was placed in the dorsal decubitus position with her arms alongside her body and her legs in abduction. Pneumoperitoneum was achieved using a Veres needle placed at the umbilicus. Four trocars were placed: a 10-mm trocar at the umbilicus for the 0-degree laparoscope; a 5-mm trocar at the right anterosuperior iliac spine; a 5-mm trocar in the midline between the umbilicus and the pubic symphysis, approximately 8 to 10 cm inferior to the umbilical trocar; and a 5-mm trocar at the left anterosuperior iliac spine. The entire pelvis was inspected for endometriotic lesions. In the pelvis, hypervascular and bluish nodules were visible with extension from the POD into the deep rectovaginal space. The macroscopic appearance was atypical for endometriotic implants. The nodularities were carefully dissected and excised, and histological assessment revealed splenic tissue. At the time of this report, the patient had been asymptomatic for 6 months after surgery. Rectovaginal splenosis may mimic endometriosis. The laparoscopic approach to rectovaginal splenosis avoids an abdominal incision, with its associated pain and possible adhesion formation. It also provides a better view for dissection. In this patient, the splenosis was removed by laparoscopy, with no postoperative dyspareunia or dyschesia."}, {"id": "pubmed23n0509_1593", "title": "Endometriosis is not only a gynecologic disease.", "score": 0.00980392156862745, "content": "The efficacy of medical and surgical treatment of endometriosis and pelvic pain is a source of questions and controversies. Complete resolution of endometriosis is not yet possible but therapy has essentially three main objectives: 1) to reduce pain; 2) to increase the possibility of pregnancy; 3) to delay recurrence for as long as possible. In case of moderate and severe endometriosis, operative laparoscopy must be considered as first line treatment. The mean pregnancy rate of 50% reported in the literature following surgery provides scientific proof that operative treatment should first be undertaken to give our patients the best chance of conceiving naturally. In case of rectovaginal adenomyotic nodules, surgery must also be considered as first line therapy, medical therapy being relatively inefficacious. Careful preoperative examination is mandatory (transrectal sonography, magnetic resonance imaging, bowel barium enema or intravenous pyelography) to evaluate potentially severe complications of the disease."}, {"id": "pubmed23n0525_18087", "title": "[Endometriosis with massive hemorrhagic ascites: a case report and review of the literature].", "score": 0.009708737864077669, "content": "Endometriosis is defined as the presence of endometrial tissue outside the uterine cavity. It generally involves the peritoneum, ovaries and rectovaginal septum. Its characteristic symptoms include dysmenorrhea, pelvic pain, deep dyspareunia and infertility. It may also involve the gastrointestinal tract, urinary tract or extra abdominal sites, giving rise to a wide variety of clinical symptoms such as bloody stools, renal haemorrhage, hemoptysis and pleural effusion during menstruation. Recurrent hemorrhagic ascites secondary to endometriosis is an unusual occurrence, 41 cases have been reported since 1954. Here we report an additional case, in order to draw attention to this condition. A 28 years-old black nulligravida woman was seen for the first time in april 2000 with a chief complaint of infertility. Her past medical history was unremarkable. She had regular menses but associated with severe dysmenorrhea. She also recalled abdominal and pelvic pain for several years. She underwent an ovulation induction with gonadotrophin, which resulted in a progressive increase of pelvic pain. A first laparoscopy was performed, revealing voluminous ascites (10 I). Two years later the ascites recurred spontaneously. Ultrasound examination revealed suspect \"para uterine masses\". A second exploratory laparoscopy showed a voluminous bloody ascites (71), and extensive adhesions. On histologic examination all specimens (peritoneal biopsies) were compatible with endometriosis and ruled out malignancy. Treatment with Gn RH analog was performed and full remission was obtained after 6 months. One year later the ascites recurred again spontaneously, leading to a third laparoscopy in an other medical institution. Histologic examination showed endometrial stromal tissue and fibrous proliferation. Later she became pregnant after in vitro fertilization. In the first trimester of pregnancy, the pelvic ultrasound showed only a small effusion in the pouch of Douglas. Still, the ascites did not progress during pregnancy. The patient was hospitalized from 27 to 33 weeks of gestational age for threatened labor, but she finally had a normal vaginal delivery at 36 weeks of gestational age. Four months later, she had no complaint, but the pelvic ultrasound showed the recurrence of the ascites. She will have a drainage. The future treatement will consists of GnRH analog for about six months, which will be relayed by a long term progestative therapy. A diagnosis of endometriosis should always be considered in middle-age women who presents with bloody ascites. Long follow-up is advisable for patients who undergo conservative treatment because of thehigh risk of recurrence."}, {"id": "pubmed23n0553_4116", "title": "Surgery for gastrointestinal endometriosis: indications and results.", "score": 0.009708737864077669, "content": "Although gastrointestinal endometriosis is an uncommon and often unexpected finding, the best treatment requires removal of all endometriotic lesions. The purpose of our study was to report our experience with the diagnosis and treatment of bowel endometriosis. From January 1997 to January 2004, 13 patients (mean 35.7y ; range 21-55y) were operated for bowel endometriosis. We noted: age, history of endometriosis, previous pregnancies, preoperative investigations and symptoms, operative procedure and intraoperative findings. Follow-up varied between one month postoperative examination and seven years. Presenting symptoms of the cases were: acute appendicitis (3), dysmenorrhoea (7), constipation (6), pelvic pain (2), rectal bleeding (3) and dyspareunia (2). Operative management was performed in accordance with the anatomical distribution. Seven patients had a history of previous operations and multifocal involvement was present in 61.5% of cases. At a median follow-up of 12.2 months, 83.3% had complete relief of their initial complaints, with only one reoperation needed. The pregnancy rate after surgery was 66.6%. Preoperative tests were: ultrasound for ovarian endometriomas, coloscopy, barium enema, vaginal palpation for detecting rectovaginal involvement, MRI and CT scan. These tests predicted the extension of endometriotic process correctly in 50% of the cases. Endometriosis of the sigmoid and rectum is rare but can give rise to severe gastrointestinal and pelvic symptoms. Preoperative investigations are not infallible in predicting the extent of the disease, sometimes placing the surgeon before a dilemma, because it involves mostly young women in the reproductive phase of life. The colorectal surgeon, therefore, should seek the advice of an experienced gynaecologist and vice versa. Removal of all endometriotic lesions is mandatory for obtaining an optimal relief of symptoms."}, {"id": "pubmed23n0923_8210", "title": "Multidisciplinary Resection of Deeply Infiltrative Endometriosis.", "score": 0.009615384615384616, "content": "To describe a multidisciplinary approach for the resection of deeply infiltrative endometriosis using the robotic platform. A technical video showing a step-by-step approach for the resection of deeply infiltrative endometriosis (Canadian Task Force classification level III). Institutional review board approval was not required for this study. There is considerable involvement of the bowel and bladder with deeply infiltrative endometriosis [1-3]. The need for operative procedures involving multiple organs while performing a complete resection is common. The benefits of minimally invasive surgery for a gynecologic pathology have been documented in numerous studies. Patients had fewer medical and surgical complications postoperatively, better cosmesis, and better quality of life [4-6]. We believe that deeply infiltrative endometriosis does not preclude patients from having a minimally invasive resection procedure. In this video, we describe how the robotic platform was used for a seamless transition between surgical specialties including gynecology, colorectal, and urology to ensure complete resection of endometriosis lesions involving multiple organs. A 47-year-old woman with a 4-year history of severe pelvic pain, dysuria, dyspareunia, dyschezia, and dysmenorrhea failing multiple medical therapies presented to our clinic to discuss surgical options. After thorough counseling, the decision was made to proceed with definitive surgical management. Postoperatively, the patient was admitted for 2 days of postoperative inpatient care. After meeting all immediate postoperative milestones, she was discharged with an indwelling Foley catheter and instructed to follow up in the clinic with all 3 surgical specialties. At the 1-week interval, she was seen by the urology team; her indwelling catheter was removed after a cystoscopy was performed documenting adequate healing. Two weeks postoperatively, the patient was seen by the gynecology and colorectal teams and was noted to be healing adequately from the procedure. Her six-week visit was also unremarkable. She continued to follow up with the gynecology team for her yearly well-woman examinations and has been symptom free for 2 years after the surgery. She takes norethindrone daily to minimize recurrence. Preoperative pelvic magnetic resonance imaging (MRI) showed bladder endometriosis and extensive rectovaginal endometriosis. We describe the multidisciplinary approach used for surgery and the procedures performed by each specialty. The urology team performed a cystoscopy preoperatively to assess for full-thickness erosions and the location of those lesions in that event. The urology team also reviewed the magnetic resonance images with the radiology team, and the endometriosis lesions were suspected to be close to the bladder trigone, keeping in mind that this finding could be overestimated given that the bladder was deflated at the time the imaging was obtained. Accordingly, at the time of surgery, the decision was made to proceed with cystoscopy and the placement of ureteral stents as a prophylactic measure. An intentional cystotomy and resection of the bladder section involved with endometriosis were performed followed by watertight closure. The trigone area of the bladder was not involved, and ureteral reimplantation was not needed in this case. The gynecology team operated second and performed an extensive dissection of the retroperitoneal space with the development of the pararectal and paravesical spaces. They also ligated the uterine artery at its origin followed by dissection of the uterovesical space, effectively reflecting the bladder off of the lower uterine segment. At this point, they proceeded with a total hysterectomy, and the specimen was removed from the pelvis through the vaginal cuff. Preoperatively, the colorectal surgeon ordered a colonoscopy to determine if full-thickness erosions were present and reviewed the magnetic resonance images with the radiology team. Based on the MRI and colonoscopy, all patients are counseled and consented for the possibility of a low anterior resection and loop ileostomy to protect the anastomosis. Based on the understanding that colorectal and gynecologic surgeries have a different approach when dissecting the pararectal space at our institution, a discussion between the 2 teams is initiated at the multidisciplinary session for surgery planning. In the case we present, the colorectal surgeon opted for the removal of the uterus before his dissection was initiated given that he dissects this space presacrally and not retroperitoneally like the gynecology counterpart. He would also benefit from the extra space for dissection with the uterus out of the pelvis. The colorectal part of the case was initiated by mobilization of the rectum and dissecting the obliterated rectovaginal space. The presacral space was then opened followed by mobilization of the rectosigmoid from its attachment. The case was concluded with full transection and reanastomosis of the rectum section involved with endometriosis. The specimen was also removed from the pelvis through the vaginal cuff. Complete resection of deeply infiltrative endometriosis spanning beyond the scope of 1 surgical specialty. No immediate intraoperative, perioperative, or long-term complications from surgery. Complete resolution of endometriosis symptoms. We encourage collaborative care for planning and performing comprehensive and safe resection of deeply infiltrative endometriosis."}, {"id": "Gynecology_Novak_4858", "title": "Gynecology_Novak", "score": 0.009615384615384616, "content": "2. Loose stools are rarely present without the use of laxatives, and there are insufficient criteria for IBS. 3. a Criteria fulfilled for the last 3 months with symptom onset at least 6 months prior to diagnosis. From Drossman DA, Corazziari E, Talley NJ, et al., eds. Rome III: the functional gastrointestinal disorders. 3nd ed. McLean, VA: Degnon Associates, 2006:885–897, Appendix A, with permission. Table 28.6 Functional Defecation Disorders 1. The patient must satisfy diagnostic criteria for functional constipation (Table 28.5) 2. During repeated attempts to defecate must have at least two of the following: a. Evidence of impaired evacuation, based on balloon expulsion test or imaging b. Inappropriate contraction of the pelvic floor muscles (i.e., anal sphincter or puborectalis) or less than 20% relaxation of basal resting sphincter pressure by manometry, imaging, or EMG c."}, {"id": "pubmed23n0350_14102", "title": "Endometriosis.", "score": 0.009523809523809525, "content": "27 year old Melinda presents to you with increasingly severe dysmenorrhoea. She has been using condoms for contraception and is no longer able to control the pain with the anti inflammatory tablets you suggested at the last consultation 6 months ago. She also complains of the recent development of deep dyspareunia. She has been in her current relationship for the last 5 years. Examination illicits similar pain and tenderness to that which she feels during intercourse. A Pap smear and STD screen are normal. You refer her to a gynaecologist who undertakes a laparoscopy on Melinda. What is seen? Ovarian adhesions secondary to endometriosis (Figure 1). Classic endometriosis (Figure 2)."}, {"id": "pubmed23n0517_8369", "title": "[Results of diagnostic hysteroscopy in a 7-year period in the gynecological clinic of \"UMBAL-Pleven\"].", "score": 0.009523809523809525, "content": "The aim of the authors is to show the data for the reception diagnosis, age, histological results and the conduct after the performed diagnostic hysteroscopies in Gynecological clinic of UMBAL-Pleven. For the fulfillment of this aim was made a prospective study for 7 years' period: from 01/01/1997 to 31/01/2003. The objects of observation were 74 women of age from 16 to 65 years, with performed hysteroscopies for gynecologic complaints. There were performed 74 diagnostic hysteroscopies for the studied period. The hysteroscopic findings were 20 cases with endometrial polyposis, 14--submucosal myoamatic nodes, deforming the uterine cavity, 4--cervical polyp, 19--increased endometrium, 9--Asherman syndrome, 1--bicomous uterus, 1--a suspected section for endometrial carcinoma and 6 cases without pathologic findings. There were performed 59 trial abrasions and the removed materials were sent for histological examination The performed comparative analysis between the hysteroscopic presentation and histological findings showed a coincidence of the diagnosis. It was made the conclusion, that the hysteroscopy is an easy, accessible and inexpensive diagnostic method, which must take its place as one of the basic contemporary diagnostic methods in gynecology."}, {"id": "pubmed23n0636_18547", "title": "Patient with pelvic pains: retroperitoneal fibrosis or pelvic endometriosis? A case report and review of literature.", "score": 0.009433962264150943, "content": "To describe how a hydronephrosis can lead to a difficult differential diagnosis between endometriosis and retroperitoneal fibrosis. Case report. Department of Obstetrics and Gynecology, Sacro Cuore Don Calabria General Hospital, Negrar, Verona, Italy. The history of a 34-year-old woman revealed the appearance of hydroureteronephrosis on the right side at the 35th week of pregnancy. She had an magnetic resonance imaging scan and was diagnosed with a spread retroperitoneal fibrosis. After 2 months, the patient reported the occurrence of pelvic pain, dyspareunia and dysmenorrhea. She was treated with corticosteroids and tamoxifen with no results. Laparoscopic surgery. A complete retroperitoneal extirpation was done of an endometriotic nodule of the right broad ligament, near the right ureter (without stenosis). Reduction of pelvic pain. She noticed an important decrease of pain. The cause of hydronephrosis could be a physiologic hydroureteronephrosis, which is the most common cause of dilatation of the urinary tract in pregnancy. The pain symptoms of the patients seemed to be linked to endometriosis and not to retroperitoneal fibrosis. Magnetic resonance imaging sometimes does not enable a correct diagnosis between these two pathologies. Fertile women with suspected fibrosis should undergo a diagnostic laparoscopy by an expert surgeon in retroperitoneal surgery."}, {"id": "Gynecology_Novak_2446", "title": "Gynecology_Novak", "score": 0.009433962264150943, "content": "Pelvic congestion affects women of reproductive age. Typical symptoms include bilateral lower abdominal and back pain that is increased with standing for long periods, secondary dysmenorrhea, dyspareunia, abnormal uterine bleeding, chronic fatigue, and irritable bowel symptoms (97). Pain usually begins with ovulation and lasts until the end of menses. The uterus is often bulky, and the ovaries are enlarged with multiple functional cysts. The uterus, parametria, and uterosacral ligaments are tender. Transuterine venography is the primary method for diagnosis, although other modalities, such as pelvic ultrasound, magnetic resonance imaging, and laparoscopy, may disclose varicosities (93). Because of the cost and possible side effects of treatment, further management should be based on related symptoms and not simply on the presence of varicosities."}, {"id": "pubmed23n1027_22858", "title": "Questioning a Previous Autism Spectrum Disorder Diagnosis: Can You \"Lose\" the Diagnosis?", "score": 0.009345794392523364, "content": "Heidi is an almost 6-year-old girl presenting to your primary care office to establish care because of a change in insurance status. You review her previous medical records before seeing her.She was diagnosed with autism spectrum disorder (ASD) when she was 25 months old. Her parents were initially concerned about language delay. Through a comprehensive evaluation by a developmental-behavioral pediatrician and a child psychologist, including administration of the Bayley Scales of Infant and Toddler Development and the Autism Diagnostic Observation Schedule, she was diagnosed with ASD. Her cognitive skills were reported to be within the average range. Soon after the diagnosis, she began receiving 20 hours of applied behavioral analysis (ABA) per week, as well as music therapy, occupational therapy, and a toddler playgroup through early intervention. Four months after the initial diagnosis, her parents reported that she had started making small improvements in her behavior, used more eye contact, and seemed more socially engaged. Approximately 1 year after the diagnosis, she was receiving 6 hours of ABA per week in addition to starting preschool with an Individualized Education Program. She reportedly continued to show progress with social communication and pretend play skills.At the age of 3 years, 8 months, neuropsychological testing was completed at her parent's request, and her cognitive skills and adaptive skills were reported to be within the average range. She continued to meet the diagnostic criteria for ASD, given her challenges with social awareness, communication, delayed play skills, decreased flexibility, and tendency toward subtle self-direction. She continued to receive speech/language therapy and attended an integrated preschool program within the school district because of her social and communication challenges. She also received ABA 4 hours weekly at home.During your first visit with Heidi, her parents report that she has continued to make progress in all areas, including social skills. She can engage in imaginary play with her friends, ask strangers questions, and comprehend the perspective of others, and she is no longer \"rigid.\" She is not receiving services outside of school and is only receiving once weekly speech/language therapy in school. Her parents no longer believe that she meets the criteria for ASD, and they are interested in further evaluation. Her parents ask if it is possible to \"lose\" the diagnosis of ASD. They also want to know if there are other things to be concerned about for her future. How do you respond?"}, {"id": "Gynecology_Novak_4503", "title": "Gynecology_Novak", "score": 0.009345794392523364, "content": "Imaging Tests The role of imaging techniques in studying female urinary incontinence is not yet established. Researchers are evaluating the potential roles of ultrasonography, fluoroscopy, functional neuroimaging, and magnetic resonance imaging (MRI). These tests should not be done routinely but are useful in certain conditions. If the patient’s symptoms (easily remembered by the three Ds: dysuria, dribbling, and dyspareunia) or examination suggests a urethral diverticulum, MRI is the test of choice (44)."}, {"id": "pubmed23n0828_23796", "title": "Laparoscopic Double Discoid Resection With a Circular Stapler for Bowel Endometriosis.", "score": 0.009259259259259259, "content": "To demonstrate the technique of laparoscopic double discoid resection with a circular stapler for bowel endometriosis. Case report (Canadian Task Force classification III). Private hospital in Curitiba, Paraná, Brazil. A 33-year-old woman was referred to our service complaining about cyclic dysmenorrhea, dyspareunia, chronic pelvic pain, and cyclic dyschezia. Transvaginal ultrasound with bowel preparation showed a 6-cm endometriotic nodule at the retrocervical area, uterosacral ligaments, posterior vaginal fornix, and anterior rectal wall, infiltrating up to the submucosa, 5 cm far from the anal verge. Under general anesthesia, the patient was placed in the dorsal decubitus position with her arms alongside her body and her lower limbs in abduction. Pneumoperitoneum was achieved using a Veres needle placed at the umbilicus. Four trocars were placed: a 10-mm trocar at the umbilicus for the zero-degree laparoscope; a 5-mm trocar at the right anterosuperior iliac spine; a 5-mm trocar in the midline between the umbilicus and the pubic symphysis, approximately 8 to 10 cm inferior to the umbilical trocar; and a 5-mm trocar at the left anterosuperior iliac spine. The entire pelvis was inspected for endometriotic lesions, and all implants in the anterior compartment of the pelvis were resected. The lesions located at the ovarian fossae were completely removed. The ureters were identified bilaterally, and both para-rectal fossae were dissected. The right hypogastric nerve was released from the disease laterally. The lesion was separated from the retrocervical area, and the posterior vaginal fornix was resected (reverse technique), leaving the disease attached to the anterior surface of the rectum. The lesion was shaved off the anterior rectal wall using a harmonic scalpel. A x-shaped stitch was placed at the anterior rectal wall using 2-0 mononylon suture. A 33-mm circular stapler was placed transanally under laparoscopic control, and once it reached the area to be resected, it was opened. A gap was created between the envil and the stapler. The anterior rectal wall was placed inside this gap with the aid of the stitch at the anterior rectal wall. The stapler was fired, and a piece of the anterior rectal wall was resected. The same procedure was performed using a 29-mm circular stapler, which allowed for the complete removal of the lesion. We usually perform the second discoid resection using a 29-mm circular stapler to allow an easy progression of the stapler through the rectum beyond the first stapler line, so not to put too much pressure on it. In our experience, the first discoid resection removes most of the disease, and the second discoid resection is only needed to remove a small amount of residual disease, along with the first staple line. The procedure took 177 min, and the estimated blood loss was 100 mL. The patient started clear liquids 6 hours after the procedure, and was discharged 19 hours after that [1]. Pathological examination of the 2 strips of the anterior rectal wall revealed infiltration of the bowel wall by endometriotic tissue. She had an uneventful postoperative course, and was able to re-start sexual intercourse 50 days after surgery. Between January 2010 and January 2015, 315 women underwent laparoscopic surgery for the treatment of bowel endometriosis in our service. Among them, 16 (5.1%) were operated on by using the double discoid resection technique. Median age of the patients was 34 years, and median body mass index was 25.9 kg/m(2). Median preoperative cancer antigen-125 level was 26.5 U/mL (normal value is &lt;35 U/mL). Median size of the rectosigmoid nodule was 35 mm (range: 30-60), and median distance from the anal verge was 10.5 cm (range: 5-15 cm). Median surgical time was 160 min (range: 54-210 min). Concomitant procedures included hysterectyomy (n = 5), partial cystectomy (n = 3), resection of the posterior vaginal fornix (n = 4), and appendectomy (n = 1). Median estimated intraoperative bleeding was 32.5 mL (range: 30-100), and median time of hospitalization was 19 hours (range: 10-41). Median American Fertility Society score was 46 (10-102). Two minor complications (12.5%) occurred in this initial series: 1 patient required bladder catheterization for urinary retention; and 1 patient developed a urinary tract infection that required oral antibiotic treatment. One major complication (6.2%) was observed; the patient developed fever and abdominal pain on the fourth postoperative day. She was re-operated, and the intraoperative diagnosis was pelviperitonitis. The abdominal cavity was inspected for any dehiscence of the bowel and then washed. She was discharged on the second day after re-operation with oral antibiotic therapy. In our daily practice, we are used to discharging our patients soon in the postoperative setting (19 hours for rectal shaving or discoid resection and 28 hours for segmental bowel resection) [1] because the rate of postoperative fistula seems to be low [2]. Because we still have not seen any fistulas after conservative surgery (rectal shaving, discoid resection, and double discoid resection), we usually prefer to perform this type of surgery compared with segmental bowel resection, when possible. Laparoscopic double discoid resection with circular stapler may be an alternative to segmental bowel resection in selected patients with bowel endometriosis."}, {"id": "pubmed23n0047_19990", "title": "[Role of vaginal echography in the investigation of menorrhagia and metrorrhagia in the reproductive years].", "score": 0.009259259259259259, "content": "Eighty non-menopausal patients who had troubles of menorrhagia or metrorrhagia who were neither pregnant nor had cervical pathology, were investigated first by vaginal ultrasound and then by hysteroscopy. In 43 of the cases a hysterosalpingogram had been carried out before hand. All patients had histological examination of tissues. The main pathological ultrasound features were clearly made out. Vaginal ultrasound, and the conditions under which the study was undertaken, gives much more information than hysterosalpingography and will be able in future to a greater extent, to replace conventional radiography. Vaginal ultrasound manages to achieve information about the endometrium and the uterine cavity almost equal to that obtained by hysteroscopy and furthermore it gives more precise information about the state of the myometrium. Vaginal ultrasound therefore seems to be an excellent first stage examination to investigate menstrual disturbances in reproductive life, and so long as it is carried out under good conditions will limit the indications for hysteroscopy and will be able to indicate when it is necessary to carry out simple exploratory hysteroscopy or operative hysteroscopy."}, {"id": "wiki20220301en017_53103", "title": "Dysmenorrhea", "score": 0.00919179229480737, "content": "Further work-up includes a specific medical history of symptoms and menstrual cycles and a pelvic examination. Based on results from these, additional exams and tests may be motivated, such as: Gynecologic ultrasonography Laparoscopy Management Treatments that target the mechanism of pain include non-steroidal anti-inflammatory drugs (NSAIDs) and hormonal contraceptives. NSAIDs inhibit prostaglandin production. With long-term treatment, hormonal birth control reduces the amount of uterine fluid/tissue expelled from the uterus. Thus resulting in shorter, less painful menstruation. These drugs are typically more effective than treatments that do not target the source of the pain (e.g. acetaminophen). Regular physical activity may limit the severity of uterine cramps."}, {"id": "pubmed23n0557_533", "title": "Delayed diagnosis of partially obstructed longitudinal vaginal septa.", "score": 0.009174311926605505, "content": "To report delayed diagnosis in two cases because of subtle manifestations of partially obstructive müllerian anomalies. Case report. Academic medical center. The first case is a 30-year-old woman who was seen initially with irregular vaginal bleeding, dysmenorrhea, and dyspareunia. On physical examination she was noted to have an anterior vaginal mass with a fistulous tract adjacent to the cervix. Blood and mucus issued from the fistulous tract when the anterior blade of the speculum compressed the vaginal mass. In case 2 a 40-year-old nulligravida was seen with infertility and mild dysmenorrhea. Her history was significant for a Strassman's metroplasty. On examination she was noted to have a bulging at the apex of the vagina adjacent to the cervix. Transvaginal ultrasound, fistulogram, hysterosalpingogram, resection of the longitudinal vaginal septa, and cycle day 3 FSH. Symptoms. In case 1 the subject had resolution of irregular vaginal bleeding, dysmenorrhea, and dyspareunia. In case 2 the patient declined to pursue further therapy. Common gynecologic symptoms resulted from partially obstructed vaginal septa. These cases demonstrate the importance of a high index of suspicion in subjects who do not respond to standard therapies."}, {"id": "pubmed23n1081_12192", "title": "Critical Role of 3D ultrasound in the diagnosis and management of Robert's uterus: a single-centre case series and a review.", "score": 0.009174311926605505, "content": "A septate uterus with a non-communicating hemicavity was first described by Robert in 1969/70 as a specific malformation of the uterus. The condition is commonly associated with a blind uterine hemicavity, unilateral haematometra, a contralateral unicornuate uterine cavity and a normal external uterine fundus. The main symptoms are repetitive attacks of pain at four-weekly intervals around menarche, repeated dysmenorrhea, recurrent pregnancy loss and infertility. In this report, we review the disease, its diagnosis and treatment, and describe five cases of Robert's uterus. Three dimensional (3D) ultrasound (US) imaging was performed by the transvaginal route in four cases. In the fifth case of a 13-year-old girl, we avoided the vaginal route and magnetic resonance imaging (MRI) and 3D transrectal US yielded the correct diagnosis. The following treatment procedures were undertaken: laparoscopic endometrectomy, hysteroscopic septum resection, laparoscopic uterine hemicavity resection and total laparoscopic hysterectomy (TLH). The diagnosis and optimum treatment of Robert's uterus remains difficult for clinicians because of its rarity. A detailed and careful assessment by 3D US should be performed, followed by hysteroscopy in combination with laparoscopy, to confirm the diagnosis."}, {"id": "pubmed23n1109_8001", "title": "Disorders, Disabilities, and Differences: Reconciling the Medical Model with a Neurodiversity Perspective.", "score": 0.00909090909090909, "content": "Zoe is a 25-month-old girl who presented to developmental-behavioral pediatrics with her parents for follow-up after receiving a diagnosis of autism spectrum disorder with global developmental delay and language impairment 3 months ago. Zoe was born by spontaneous vaginal delivery at term after an uncomplicated pregnancy, labor, and delivery. She had a routine newborn course and was discharged home with her parents 2 days after her birth.At 7 months, Zoe was not able to sit independently, had poor weight gain, and had hypertonia on physical examination. Her parents described her to tense her arms and have hand tremors when she held her bottle during feedings and reported that she had resisted their attempts to introduce pureed or other age-appropriate table foods into her diet. The Bayley Scales of Infant and Toddler Development Screening Test was administered and found a cognitive composite score of 70, language composite score of 65, and motor composite score of 67. Chromosomal microarray analysis, testing for fragile X syndrome, laboratory studies for metabolic disorders, magnetic resonance imaging of the brain, and an audiologic examination were normal. Zoe was referred to and received early intervention services including physical therapy, feeding therapy, and infant stimulation services. By 16 months, Zoe was walking independently and was gaining weight well but continued to have sensory aversions to some foods.At 22 months, Zoe was evaluated by a multidisciplinary team because of ongoing developmental concerns and concerning results on standardized screening for autism spectrum disorder completed at her 18-month preventive care visit. Her parents also reported concern about the possibility of autism spectrum disorder (ASD) because they both were diagnosed with ASD as young children. Both parents completed college and were employed full-time. Zoe's mother seemed to be somewhat anxious during the visit and provided fleeting eye contact throughout the evaluation. Zoe's father was assertive, but polite, and was the primary historian regarding parental concerns during the evaluation.Zoe was noted to have occasional hand flapping and squealing vocalizations while she roamed the examination area grabbing various objects and casting them to the floor while watching the trajectory of their movements. She did not use a single-finger point to indicate her wants or needs and did not initiate or follow joint attention. She met criteria for ASD. In discussing the diagnosis with Zoe's parents, they shared that they were not surprised by the diagnosis. They expressed feeling that Zoe was social and playful, although delayed in her language. Hence, they were more concerned about her disinterest in eating. They were not keen on behavioral intervention because they did not want Zoe to be \"trained to be neurotypical.\" Although the mother did not receive applied behavior analysis (ABA), the father had received ABA for 3 years beginning at age 5 years. He believed that ABA negatively changed his personality, and he did not want the same for Zoe.How would you assist Zoe's parents in identification of priorities for her developmental care while ensuring respect for their perspective of neurodiversity?"}, {"id": "pubmed23n0741_21893", "title": "[Diagnosis of pelvic inflammatory disease. Which clinical and paraclinical criteria? Role of imaging and laparoscopy?].", "score": 0.00909090909090909, "content": "Diagnosis of pelvic inflammatory disease is difficult. We focus on a systematic literature review to study diagnostic values of history-taking, clinical examination, laboratory tests and imagery. After this literature review, we build a diagnostic model for pelvic inflammatory disease. This diagnostic model is built on two major criteria: presence of adnexal tenderness or cervical motion tenderness. Additional minor criteria, increasing the likelihood of the diagnosis of pelvic inflammatory disease were added based on their specificity and their positive likelihood ratio. These minor criteria are supported by history-taking, clinical examination, laboratory tests and also on relevant ultrasonographic criteria."}, {"id": "pubmed23n0623_896", "title": "Transvaginal-laparoscopic anterior rectum resection in a hysterectomized woman with deep-infiltrating endometriosis: Description of a gynecologic natural orifice transendoluminal surgery approach.", "score": 0.009009009009009009, "content": "Deep-infiltrating endometriosis may affect the vagina, the rectum, and the cervicoisthmic part of the uterus, resulting in severe pain, particularly dyschezia, dysmenorrhea, dyspareunia, and diminished quality of life. Advanced surgical techniques, such as laparoscopic-assisted anterior rectum resection, are recognized as safe and effective therapeutic approaches. In some cases, a laparotomy or minilaparotomy has to be performed for technical reasons. This can be avoided in some cases by transvaginal-laparoscopic low anterior rectum resection. The technique is a 4-step procedure, which can be described as follows: step 1 (vaginal) - rectovaginal examination, preparation of the rectovaginal septum, opening of the pouch of Douglas, mobilization of the endometriotic nodule and the rectum, temporary vaginal closure; step 2 (laparoscopic) - removal of additional endometriotic lesions, adhesiolysis, final mobilization of the rectum, mobilization of the rectosigmoid, endoscopic resection using an endoscopic stapler step 3 (vaginal) - transvaginal resection of the lesion, preparation of the oral anvil, closure of the vagina; and step 4 (laparoscopic) - endoscopic transanal stapler anastomosis and underwater rectoscopy, prophylaxis of adhesions, drainage. We used this procedure to treat a 46-year-old woman (gravida 2, para 2) who was admitted to our hospital for severe lower abdominal pain, constipation, dyspareunia, dyschezia, and cyclic rectal bleedings. The symptoms were caused by an endometriotic nodule accompanied by a palpable rectum stenosis. In addition, she reported a past abdominal hysterectomy with complications caused by symptomatic myomatous uterus. As a gynecologic natural orifice surgery approach, the transvaginal-laparoscopic anterior rectum resection may be an additional useful surgical technique that could be offered by surgical gynecologists to some women with deep-infiltrating endometriosis."}, {"id": "Gynecology_Novak_61", "title": "Gynecology_Novak", "score": 0.009009009009009009, "content": "Finally, before dismissing the symptom under study, inquire about other symptoms that might reasonably be expected under the clinical circumstances of the case. Symptoms specifically sought but denied are known as negative symptoms. These negative symptoms may confirm or rule out diagnostic possibilities suggested by the positive symptoms. 3. The data secured by the techniques described in the first two phases of the interview should now suggest several diagnostic possibilities. Test these possibilities further by inquiring about other symptoms or events that may form part of the natural history of the suspected disease or group of diseases. 4."}, {"id": "pubmed23n1128_74", "title": "Ultrasound-Guided Transvaginal Aspiration and Sclerotherapy for Uterine Cystic Adenomyosis: Case Report and Literature Review.", "score": 0.008928571428571428, "content": "Uterine cystic adenomyosis is a very rare type of adenomyosis which can be easily misdiagnosed in clinical practice. In the past, cases have been mostly treated with surgical resection of the uterine lesion. We report the case of a 25-year-old woman who presented with severe dysmenorrhea for more than 1 year. Physical examination showed that the uterus was enlarged. The transvaginal ultrasound showed a cystic mass of about 5.0 × 3.6 × 3.6 cm in the posterior myometrium, with dense echo spots and no blood flow signal in the cystic part. Magnetic resonance imaging (MRI) indicated hemorrhages within the cystic mass, suggesting the possibility of uterine cystic adenomyosis. The lower abdominal pain and severe dysmenorrhea were not alleviated after a 6-month trial of oral contraceptives. Subsequently, she underwent ultrasound-guided transvaginal aspiration and sclerotherapy for uterine cystic adenomyosis. Approximately 90 mL of chocolate-colored fluid was aspirated from the mass and 20 mL of lauromacrogol was injected in the cyst. The reduction rates of the mass 3 and 12 months after the procedure were 92.01 and 99.10%, respectively. Her dysmenorrhea completely resolved. One and half year after the operation, she had a successful pregnancy and gave birth to a healthy baby through vagina. The rare entity of uterine cystic adenomyosis can be treated safely and effectively by ultrasound-guided transvaginal aspiration and sclerotherapy."}, {"id": "pubmed23n0344_10189", "title": "[The diagnosis and surgical treatment in rectal endometriosis].", "score": 0.008928571428571428, "content": "From Jun 1975 to Mar 1995, 26 cases of endometriosis in the rectum (RE) were admitted. Local resection was performed in 16 and Dixon's operation in 10. The result showed 21 cases were symptomatically cured and 5 with remission. 2 cases were reoperated because of recurrence in 5 years after the first operation and cured. RE is difficult in distiguishing from rectal cancer. It is characterized by tenesmus, bursting pain, hematochezia in young and middle aged women during periods. The diagnosis can be made by tipycal history and vaginal examination, rectal examination, barium enema, proctoscopy and so on. The indications of operation include severe clinic symptoms and failed conservative therapy. Wedge resection was suitable in cases with small lession in rectum, while large, deep seated lessions in lower rectum were treated with Dixon's operation in order to prevent recurrence."}, {"id": "pubmed23n1035_3786", "title": "Peritoneal Retraction Pocket Defects and Their Important Relationship with Pelvic Pain and Endometriosis.", "score": 0.008849557522123894, "content": "The objective of this video is to demonstrate different clinical presentations of peritoneal defects (peritoneal retraction pockets) and their anatomic relationships with the pelvic innervation, justifying the occurrence of some neurologic symptoms in association with these diseases. Surgical demonstration of complete excision of different types of peritoneal retraction pockets and a comparison with a laparoscopic retroperitoneal cadaveric dissection of the pelvic innervation. Private hospital in Curitiba, Paraná, Brazil. A pelvic peritoneal pocket is a retraction defect in the surface of the peritoneum of variable size and shapes [1]. The origin of defects in the pelvic peritoneum is still unknown [2]. It has been postulated that it is the result of peritoneal irritation or invasion by endometriosis, with resultant scarring and retraction of the peritoneum [3,4]. It has also been suggested that a retraction pocket may be a cause of endometriosis, where the disease presumably settles in a previously altered peritoneal surface [5]. These defects are shown in many studies to be associated with pelvic pain, dyspareunia, and secondary dysmenorrhea [1-4]. Some studies have shown that the excision of these peritoneal defect improves pain symptoms and quality of life [5]. It is important to recognize peritoneal pockets as a potential manifestation of endometriosis because in some cases, the only evidence of endometriosis may be the presence of these peritoneal defects [6]. In this video, we demonstrate different types of peritoneal pockets and their close relationship with pelvic anatomic structures. Case 1 is a 29-year-old woman, gravida 0, with severe dysmenorrhea and catamenial bowel symptoms (bowel distension and diarrhea/constipation) that were unresponsive to medical treatment. Imaging studies were reported as normal, and a laparoscopy showed a posterior cul-de-sac peritoneal pocket infiltrating the pararectal fossa, with extension to the lateral border of the rectum. Case 2 is a cadaveric dissection of a posterior cul-de-sac peritoneal pocket infiltrating the pararectal fossa, with extension to the pelvic sidewall. After dissection of the obturator fossa, we can observe that the pocket is close to the sacrospinous ligament, pudendal nerve, and some sacral roots. Case 3 is a 31-year-old woman, gravida 1, para 1, with severe dysmenorrhea that was unresponsive to medical treatment and catamenial bowel symptoms (catamenial bowel distention and diarrhea). Imaging studies were reported as normal and a laparoscopy showed left uterosacral peritoneal pocket infiltrating the pararectal fossa in close proximity to the rectal wall. Case 4 is a cadaveric dissection of the ovarian fossa and the obturator fossa showing the proximity between these structures. Case 5 is a 35-year-old woman, gravida 0, with severe dysmenorrhea that was unresponsive to medical treatment, referring difficulty, and pain when walking only during menstruation. A neurologic physical examination revealed weakness in thigh adduction, and the magnetic resonance imaging showed no signs of endometriosis. During laparoscopy, we found a peritoneal pocket infiltrating the ovarian fossa, with involvement in the area between the umbilical ligament and the uterine artery. This type of pocket can easily reach the obturator nerve. Because the obturator nerve and its branches supply the muscle and skin of the medial thigh [7,8], patients may present with thigh adduction weakness or difficulty ambulating [9,10]. Case 6 is a cadaveric dissection of the sacrospinous ligament and the pudendal nerve from a medial approach, between the umbilical artery and the iliac vessels. Case 7 is a 34-year-old woman, gravida 1, para 1, with severe dysmenorrhea and catamenial bowel symptoms as well as deep dyspareunia. The transvaginal ultrasound showed focal adenomyosis and a 2-cm nodule, 9-cm apart from the anal verge, affecting 30% of the bowel circumference. In the laparoscopy, we found a posterior cul-de-sac retraction pocket associated with a large deep endometriosis nodule affecting the vagina and the rectum. In all cases, endometriosis was confirmed by histopathology, and in a 6-month follow-up, all patients showed improvement of bowel, pain, and neurologic symptoms. Peritoneal pockets can have different clinical presentations. Depending on the topography and deepness of infiltration, they can be the cause of some neurologic symptoms associated with endometriosis pain. With this video, we try to encourage surgeons to totally excise these lesions and raise awareness about the adjacent key anatomic structures that can be affected."}, {"id": "Gynecology_Novak_57", "title": "Gynecology_Novak", "score": 0.008849557522123894, "content": "Table 1.5 Technique of Taking the History of the Present Illness 1. The technique used in taking the history of the present illness varies with the patient, the patient’s problem, and the physician. Allow the patient to talk about her chief symptom. Although this symptom may or may not represent the real problem (depending on subsequent evaluation), it is usually uppermost in the patient’s mind and most often constitutes the basis for the visit to the physician. During the phase of the interview, establish the temporal relation of the chief symptom to the total duration of the illness. Questions such as, “Then up to the time of this symptom, you felt perfectly well?” may elicit other symptoms that may antedate the chief one by days, months, or years. In this manner, the patient may recall the date of the first appearance of illness. Encourage the patient to talk freely and spontaneously about her illness from the established date of onset. Do not interrupt the patient’s account,"}, {"id": "pubmed23n1009_364", "title": "Herlyn-Werner-Wunderlich syndrome presenting with dysmenorrhea: a case report.", "score": 0.008771929824561403, "content": "Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to hematocolpos. We present the first case report on Herlyn-Werner-Wunderlich syndrome from Bangladesh. A 15-year-old Asian girl presented with lower abdominal pain of 3 months' duration. She had had menarche 3 months earlier and had a regular menstrual cycle with cyclical abdominal pain. Abdominal examination found a tender mass on the right iliac fossa. Further evaluation with ultrasound revealed distended endometrial cavity filled with complex fluid and nonvisualization of the right kidney. Pelvic magnetic resonance imaging showed absent right kidney and two separate endometrial stripes surrounded by endometrium and a muscular layer. The right endometrial cavity and cervix were distended with blood. This magnetic resonance imaging finding is consistent with Herlyn-Werner-Wunderlich syndrome with uterine didelphyis, right-sided hematometra resulting from obstructed hemivagina, and ipsilateral agenesis of the right kidney. The vaginal septum was resected for vaginoplasty. She was discharged 5 days after surgery and came for follow-up after 7 days. Vaginal examination revealed a healthy wound with no adhesion of the vaginal wall. She also informed us that she had started regular menstruation without any pain 30 days after the operation. An unusual presentation of regular menstruation and nonspecific abdominal pain delays the diagnosis, which can lead to complications such as endometriosis and infertility. Awareness is required; otherwise, misdiagnosis clearly can occur."}, {"id": "pubmed23n0046_23599", "title": "Diagnosis of acute pelvic pain.", "score": 0.008771929824561403, "content": "The diagnosis of acute pelvic pain in the woman of reproductive age represents a major clinical challenge. In approaching such a patient, the clinician must differentiate between pregnancy-related causes, gynecologic disorders, and nonreproductive tract causes. A careful history and physical examination, along with selective and knowledgeable use of diagnostic tests and procedures, are essential to the diagnostic process. Diagnostic laparoscopy represents the reference standard for diagnosis of many of its possible causes and can obviate the need for exploratory laparotomy. Once competing diagnoses have been adequately excluded, an empiric trial of antibiotic therapy for acute pelvic inflammatory disease, coupled with close clinical follow-up, should be considered in patients with acute pelvic pain found to have cervical motion tenderness and bilateral adnexal tenderness on examination."}, {"id": "pubmed23n0304_247", "title": "Pseudomembranous enterocolitis after gynecologic endoscopy.", "score": 0.008695652173913044, "content": "A 21-year-old nulligravida underwent diagnostic hysteroscopy and laparoscopic potassium-titanyl-phosphate laser ablation of pelvic peritoneal endometriosis (revised American Fertility score of 10) for dysmenorrhea, dyspareunia, and dyschezia. Preoperatively the patient had an electrolyte bowel preparation but no antibiotic prophylaxis. Six days postoperatively she developed symptoms of nausea, vomiting, and diarrhea, which were not affected by diet and over-the-counter bowel medications. Examination of stool samples for culture, ova, parasites, and Clostridium difficile toxin led to the diagnosis of C. difficile pseudomembranous enterocolitis. The patient was referred to a gastroenterologist. She required 4 months of metronidazole therapy, including two hospitalizations, before her symptoms resolved. The stool assay became negative for C. difficile toxin 6 months after surgery. Pseudomembranous enterocolitis may occur rarely in patients without the usual risk factors of antibiotic therapy. The role of electrolyte bowel preparation is uncertain, but it may have permitted overgrowth of C. difficile."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[257, 502]], "word_ranges": [[34, 74]], "text": "In our case, when attending animal births, the entry is through the respiratory route, so the pulmonary infiltrates and chest pain are due to the bacteria. The treatment varies according to the affectation and mortality is very low, almost null."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "As a possible disease I would consider Brucellosis, typical of cattle (milk, abortion, genital secretions...). It presents with a pseudo-flu-like symptomatology (headache, arthralgias, myalgias, fever...) and other symptoms depending on the route of entry. In our case, when attending animal births, the entry is through the respiratory route, so the pulmonary infiltrates and chest pain are due to the bacteria. The treatment varies according to the affectation and mortality is very low, almost null. Morbidity and involvement in pregnant women are of greater concern. Important: it is very frequent in livestock workers (veterinarians, farmers, slaughterhouses...) if you read something like \"attends births of livestock animals\", \"works on a farm\", \"is a veterinarian\" suspect Brucellosis.", "full_answer_no_ref": "As a possible disease I would consider Brucellosis, typical of cattle (milk, abortion, genital secretions...). It presents with a pseudo-flu-like symptomatology (headache, arthralgias, myalgias, fever...) and other symptoms depending on the route of entry. In our case, when attending animal births, the entry is through the respiratory route, so the pulmonary infiltrates and chest pain are due to the bacteria. The treatment varies according to the affectation and mortality is very low, almost null. Morbidity and involvement in pregnant women are of greater concern. Important: it is very frequent in livestock workers (veterinarians, farmers, slaughterhouses...) if you read something like \"attends births of livestock animals\", \"works on a farm\", \"is a veterinarian\" suspect Brucellosis.", "full_question": "A 38-year-old woman of veterinary profession, in charge of monitoring wild animals and assisting in the delivery of domestic livestock. She starts with a high fever with chills, headache, myalgia and non-productive cough that she interprets as a flu-like process. She presented with chest pain. Chest X-ray showed bilateral pulmonary infiltrates in lower fields. A serologic test was performed with elevated titers of antibodies against phase II antigens. Which of the following statements is TRUE?", "id": 438, "lang": "en", "options": {"1": "This entity is transmitted by ticks.", "2": "Both doxycycline and hydroxychloroquine are effective in treating acute forms of this disease.", "3": "In the acute form, the patient also generally has elevated antibodies to phase I antigens.", "4": "Mortality in acute forms is almost nonexistent.", "5": NaN}, "question_id_specific": 119, "type": "EPIDEMIOLOGY AND PREVENTIVE MEDICINE", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1159_13154", "title": "Importance of clinical history in the diagnosis of psittacosis: A case report.", "score": 0.01889483065953654, "content": "Psittacosis, caused by the bacteria Chlamydia psittaci, is primarily a disease of birds that can be transmitted to humans. The clinical manifestations of the disease are wide, ranging from asymptomatic illness to fulminant psittacosis with multi-organ failure. The organism gets attached to the upper respiratory mucosa after inhalation and the majority remain asymptomatic. However, some people may develop symptoms of atypical pneumonia. Psittacosis usually presents with sudden onset fever with chills and rigor, headache, and myalgia. Here we present a case of a 35 years old female with a history of close contact with parrots who presented to the ER with complaints of high-grade fever and headache for 2 weeks which started 2 days after her parrots died. The disease usually manifests as flu-like symptoms or pneumonia and is included in the differential diagnosis of community-acquired pneumonia. Investigations reveal neutrophilia, raised erythrocyte sedimentation rate, C-reactive protein, and elevated liver enzymes which were consistent with the findings of our patient. Chest X-ray showed ill-defined consolidation in the right middle and lower lobes which were inconclusive. Hence, a CT chest was done which revealed patchy ground glass opacities with surrounding consolidation giving a reverse halo sign. Due to her contact with birds and CT findings which were suggestive of psittacosis, she was started on doxycycline and her condition improved thereafter. We highlight the importance of proper history taking and awareness on zoonotic diseases to the general public to prevent, diagnose and treat the disease effectively."}, {"id": "pubmed23n0063_1826", "title": "[Familial outbreak of psittacosis].", "score": 0.01717142443124185, "content": "Three familial cases of psittacosis are reported. The first case was a 46-year-old woman, the second case, her 18-year-old daughter. Both of them often visited the house of the third case, a 49-year-old women, who was the elder sister of case 1 and who took care of the chick of a budgerigar which she kept in the house. Case 1 came to our hospital with abrupt onset of fever, headache, nausea and general malaise. Because she was suspected to have meningitis, she was admitted to the Department of Neurology. On admission, her chest X-ray film showed bilateral ground glass shadows. She also had hypoxemia and liver dysfunction. On learning of her history of contact with the chick, psittacosis was suspected. Case 2 suffered from fever and headache. Her chest X-ray film revealed opaque infiltration in the right lower lung field. Case 3 complained of fever, headache and vomiting. Her chest X-ray film showed fan-shaped faint shadows in the left upper, middle and lower lung fields. We interpreted these findings as showing psittacosis based on anamnesis. The result of the complement fixation (CF) antibody titer against chlamydia was 1:32 in cases 2 and 3, enabling a serological diagnosis of psittacosis. The corresponding result was 1:16 in case 1. Although the CF antibody titer showed no increase, we diagnosed the case clinically as psittacosis. It is difficult to correctly diagnose psittacosis only from the physical findings and chest X-ray films. Detailed anamnesis, in particular taking a history of exposure to birds, is an important clue for diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0416_4739", "title": "Q fever--still a query and underestimated infectious disease.", "score": 0.016797541823147834, "content": "Coxiella burnetii (C.b.) is a strictly intracellular, Gram-negative bacterium. It causes Q fever in humans and animals worldwide. The animal Q fever is sometimes designated \"coxiellosis\". This infection has many different reservoirs including arthropods, birds and mammals. Domestic animals and pets, are the most frequent source of human infections. Q fever may appear basically in two forms, acute and chronic (persistent). The latter form of Q fever in animals is characteristic by shedding C.b. into the environment during parturition or abortion. Human Q fever results usually from inhalation of contaminated aerosols originating mostly from tissue and body fluids of infected animals. Q fever may appear in humans either in an acute form accompanied mainly by fever (pneumonia, flu-like disease, hepatitis) or in a chronic form (mainly endocarditis). Diagnosis of Q fever is based on isolation of the agent in cell culture, its direct detection, namely by PCR, and serology. Detection of high phase II antibodies titers 1-3 weeks after the onset of symptoms and identification of IgM antibodies are indicative to acute infection. High phase I IgG antibody titers &gt;800 as revealed by microimmunofluorescence offer evidence of chronic C.b. infection. For acute Q fever, a two-weeks-treatment with doxycycline is recommended as the first-line therapy. In the case of Q fever endocarditis a long-term combined antibiotic therapy is necessary to prevent relapses. Application of Q fever vaccines containing or prepared from phase I C.b. corpuscles should be considered at least for professionally exposed groups of the population. Infections caused by C.b. are spread worldwide and may pose serious and often underestimated health problems in human but also in veterinary medicine. Though during the last decades substantial progress in investigation of C.b. has been achieved and many data concerning this pathogen has been accumulated, some questions, namely those related to the pathogenesis of the disease, remain open."}, {"id": "pubmed23n0736_2372", "title": "[Two cases of acute hepatitis associated with Q fever].", "score": 0.014932386571294313, "content": "Q fever which is caused by Coxiella burnetii, is a worldwide zoonosis. Many species of wild and domestic mammals, birds, and arthropods, are reservoirs of C.burnetii in nature, however farm animals are the most frequent sources of human infection. The most frequent way of transmission is by inhalation of contaminated aerosols. The clinical presentation of Q fever is polymorphic and nonspecific. Q fever may present as acute or chronic disease. In acute cases, the most common clinical syndromes are selflimited febrile illness, granulomatous hepatitis, and pneumonia, but it can also be asymptomatic. Fever with hepatitis associated with Q fever has rarely been described in the literature. Herein we report two cases of C.burnetii hepatitis presented with jaundice. In May 2011, two male cases, who inhabited in Malkara village of Tekirdag province (located at Trace region of Turkey), were admitted to the hospital with the complaints of persistent high grade fever, chills and sweats, icterus, disseminated myalgia and headache. Physical examination revealed fever, icterus and the patient appeared to be mildly ill but had no localizing signs of infection. Radiological findings of the patients were in normal limits. Laboratory findings revealed leukocytosis, increased hepatic and cholestatic enzyme levels, and moderate hyperbilirubinemia- mainly direct bilirubin, whereas serum C-reactive protein and erythrocyte sedimentation rate were found normal. Blood and urine cultures of the patients yielded no bacterial growth. Serological markers for acute viral hepatitis, citomegalovirus and Epstein-Barr virus infections, brucellosis, salmonellosis, toxoplasmosis and leptospirosis were found negative. Acute Q fever diagnosis of the cases were based on the positive results obtained by C.burnetii Phase II IgM and IgG ELISA (Vircell SL, Spain) test, and the serological diagnosis were confirmed by Phase I and II immunofluorescence (Vircell SL, Spain) method. Both cases were treated with doxycycline for 14 days and became afebrile within four days. These cases were presented to emphasize that C.burnetii infection should be considered in the differential diagnosis of patients with fever and elevated serum transaminase levels, irrespective of the presence of abdominal pain and exposure to potentially infected animals."}, {"id": "pubmed23n0841_17504", "title": "Pulmonary inflammatory pseudotumor due to Coxiella burnetii. Case report and literature review.", "score": 0.01405236070709331, "content": "A 58-year-old man was admitted because of respiratory failure, episodic fever with chilling, cough, malaise, fatigue, myalgia and weight loss lasting for at least one month. Chest x-rays and CT scan of the chest showed bilateral pulmonary consolidations in upper lobes, the left lower lobe, and mediastinal lymphadenopathy. Bronchoscopy with cytology was unremarkable. A needle CT-guided lung biopsy documented an inflammatory pseudotumor, lymphoplasmacytic type. Serology showed high titer antibodies to phase II Coxiella burnetii infection. Therapy with doxycycline and hydroxychloroquine for three months led to a complete resolution of symptoms and radiological findings, and a marked decrease in titers to Q fever. "}, {"id": "pubmed23n0744_3336", "title": "Q Fever: an old but still a poorly understood disease.", "score": 0.013401377479047382, "content": "Q fever is a bacterial infection affecting mainly the lungs, liver, and heart. It is found around the world and is caused by the bacteria Coxiella burnetii. The bacteria affects sheep, goats, cattle, dogs, cats, birds, rodents, and ticks. Infected animals shed this bacteria in birth products, feces, milk, and urine. Humans usually get Q fever by breathing in contaminated droplets released by infected animals and drinking raw milk. People at highest risk for this infection are farmers, laboratory workers, sheep and dairy workers, and veterinarians. Chronic Q fever develops in people who have been infected for more than 6 months. It usually takes about 20 days after exposure to the bacteria for symptoms to occur. Most cases are mild, yet some severe cases have been reported. Symptoms of acute Q fever may include: chest pain with breathing, cough, fever, headache, jaundice, muscle pains, and shortness of breath. Symptoms of chronic Q fever may include chills, fatigue, night sweats, prolonged fever, and shortness of breath. Q fever is diagnosed with a blood antibody test. The main treatment for the disease is with antibiotics. For acute Q fever, doxycycline is recommended. For chronic Q fever, a combination of doxycycline and hydroxychloroquine is often used long term. Complications are cirrhosis, hepatitis, encephalitis, endocarditis, pericarditis, myocarditis, interstitial pulmonary fibrosis, meningitis, and pneumonia. People at risk should always: carefully dispose of animal products that may be infected, disinfect any contaminated areas, and thoroughly wash their hands. Pasteurizing milk can also help prevent Q fever."}, {"id": "pubmed23n1017_25354", "title": "Imported brucellosis and Q-fever coinfection in Croatia: a case report.", "score": 0.013046064932857386, "content": "The brucellosis and Q-fever coinfection is very rarely reported. To our knowledge, this is the first case report of concomitant brucellosis and Q-fever, most likely imported in Croatia. A 30-year-old male agricultural worker was hospitalized on 22 April 2017 after a ten days fever up to 40°C with chills, shivering, excessive sweating, general weakness, loss of appetite and headache. A month and a half prior to the hospitalization he lost 18 kg of body weight. Three weeks before hospitalization the patient returned from Kupres (Bosnia and Herzegovina) where he was working for the past year on a sheep farm and consumed unpasteurized dairy products of sheep origin. At admission, his condition was moderately severe due to pronounced dehydration. Routine laboratory tests showed slightly elevated erythrocyte sedimentation rate, anemia, thrombocytopenia and elevated liver transaminases. The chest X-ray showed an inhomogeneous infiltrate of the lower right lung. Three sets of blood culture were cultivated. After 48 hours incubation, bacterial growth was detected in aerobic bottles. Gram-stained smear revealed small, gram-negative coccobacilli. Specimens were subcultured on blood and chocolate agar plates. Using a Vitek GN identification card, the isolated organism was identified as Brucella melitensis. 16S rRNA gene sequencing of the isolate confirmed it as a Brucella sp. Rose-Bengal test was positive, while Wright agglutination test showed a significant increase in antibody titer from 80 to 640 in paired sera. Using indirect immunofluorescence assay (IFA), Coxiella burnetii phase II IgM/IgG titers were 50 and 1024, respectively indicating acute Q-fever. The patient was treated with doxycycline and rifampicin. So far, there has been no relapse or signs of chronic infection."}, {"id": "wiki20220301en200_20677", "title": "Acute chest syndrome", "score": 0.012891556992226584, "content": "Diagnosis The diagnosis of acute chest syndrome is made difficult by its similarity in presentation with pneumonia. Both may present with a new opacification of the lung on chest x-ray. The presence of fevers, low oxygen levels in the blood, increased respiratory rate, chest pain, and cough are also common in acute chest syndrome. Diagnostic workup includes chest x-ray, complete cell count, reticulocyte count, ECG, and blood and sputum cultures. Patients may also require additional blood tests or imaging (e.g. a CT scan) to exclude a heart attack or other pulmonary pathology. Prevention Hydroxyurea is a medication that can help to prevent acute chest syndrome. It may cause a low white blood cell count, which can predispose the person to some types of infection. Treatment Broad spectrum antibiotics to cover common infections such as Streptococcus pneumoniae and mycoplasma, pain control, and blood transfusion. Acute chest syndrome is an indication for exchange transfusion."}, {"id": "wiki20220301en415_31130", "title": "Hantavirus pulmonary syndrome", "score": 0.011763829945648127, "content": "Signs and symptoms Initially, HPS has an incubation phase of 2–4 weeks, in which patients remain asymptomatic. Subsequently, patients can experience 3–5 days of flu-like prodromal phase symptoms, including fever, cough, muscle pain, headache, lethargy, shortness of breath, nausea, vomiting and diarrhea. In the following 5–7 day cardiopulmonary phase, the patient's condition rapidly deteriorates into acute respiratory failure, characterized by the sudden onset of shortness of breath with rapidly evolving pulmonary edema, as well as cardiac failure, with hypotension, tachycardia and shock. In this phase, patients may develop acute respiratory distress syndrome. It is often fatal despite mechanical ventilation and intervention with diuretics. After the cardiopulmonary phase, patients can enter a diuretic phase of 2–3 days characterized by symptom improvement and diuresis. Subsequent convalescence can last months to years. As of 2017, patient mortality in the USA from HPS is 36%."}, {"id": "pubmed23n0751_12765", "title": "Pulmonary involvement in brucellosis.", "score": 0.01152977139819245, "content": "Brucellosis is a zoonotic disease caused by a Gram-negative bacillus of the Brucella genus with multisystem involvement, primarily affecting the reticuloendothelial system, joints, heart and kidneys. Although the disease can be spread by inhalation, pulmonary involvement is rare. To report a case of brucellosis with pulmonary involvement. CASE PRESENTAION: A previously healthy 36-year-old woman was admitted with complaints of fever, weakness, night sweats, dry cough and bilateral chest pain. She hed been diagnosed with pneumonia 20 days previously and was started on a course of ampicillin for 14 days, with no response. Her chest auscultation revealed diminished breath sounds and scattered crackles and rhonchi over the inferior zone of the right hemithorax. Wright and Coombs testing resulted in titres of 1:1280 and 1:640, respectively. Chest radiography revealed an area of confluent lobar consolidation in the right lower lobe. Treatment was started with a six-week course of oral doxycycline 200 mg/day and rifampicin 600 mg/day. This treatment regimen rapidly improved the patient's condition. Follow-up after one year showed no residual effects from the infection. Pulmonary involvement is a rare event in the course of brucellosis, but the rate could be higher than currently estimated. In endemic regions, brucellosis should be considered as a causative agent in patients with pulmonary symptoms. Brucellosis is a zoonotic disease caused by a Gram-negative bacillus of the <iBrucella</i genus with multisystem involvement, primarily affecting the reticuloendothelial system, joints, heart and kidneys. Although the disease can be spread by inhalation, pulmonary involvement is rare. To report a case of brucellosis with pulmonary involvement. A previously healthy 36-year-old woman was admitted with complaints of fever, weakness, night sweats, dry cough and bilateral chest pain. She hed been diagnosed with pneumonia 20 days previously and was started on a course of ampicillin for 14 days, with no response. Her chest auscultation revealed diminished breath sounds and scattered crackles and rhonchi over the inferior zone of the right hemithorax. Wright and Coombs testing resulted in titres of 1:1280 and 1:640, respectively. Chest radiography revealed an area of confluent lobar consolidation in the right lower lobe. Treatment was started with a six-week course of oral doxycycline 200 mg/day and rifampicin 600 mg/day. This treatment regimen rapidly improved the patient’s condition. Follow-up after one year showed no residual effects from the infection. Pulmonary involvement is a rare event in the course of brucellosis, but the rate could be higher than currently estimated. In endemic regions, brucellosis should be considered as a causative agent in patients with pulmonary symptoms."}, {"id": "pubmed23n0323_13890", "title": "[Diffuse panbronchiolitis with myeloperoxidase-specific antineutrophil cytoplasmic antibody-related vasculitis].", "score": 0.011211607311098548, "content": "A 46-year-old woman was referred to our department in July 1996 with complaints of fever and myalgia in her calves. She had a 20-year history of purulent sputum; diffuse panbronchiolitis had been diagnosed in 1983. Physical examination revealed low-pithed rhonchi over the lung fieldis and hypesthesia of the right leg. She had a white blood cell count of 16,100/mm3, including 4% eosinophils, and a platelet count of 80.0 x 10(4)/mm3. The serum IgE level was 2,200 U/ml, and the cold hemagglutinin titer was high. Pulmonary-function tests showed mixed ventilatory dysfunction, and arterial blood gas analysis revealed a PaO2 of 55.8 Torr on room air. Pseudomonas aeruginosa was cultured from her sputum. A chest X-ray film and CT scan showed diffuse nodular shadows and bronchiectatic changes with mild hyperinflation. An infiltrative lesion in right S6 area could also be seen. Administration of broad-spectrum antibiotics did not alleviate her symptoms. The level of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA) in serum was 245 EU/ml, and 67Ga scintigraphy showed marked accumulation in the abdomen. Abdominal angiography demonstrated a bead-like appearance and irregularities in the peripheral branches of the hapatic artery, the splenic artery, the cystic artery, and the superior mesenteric artery. Because of the high MPO-ANCA level and the angiographic abnormalities, MPO-ANCA-related vasculitis was diagnosed. She was treated with 1 g of methylprednisolone daily for 3 days, followed by 60 mg of prednisolone and 50 mg of cyclophosphamide daily. Her condition improved dramatically, and the MPO-ANCA level became almost normal. During treatment, her blood pressure rose markedly with a normal serum creatinine level and normal urinalysis. Plasma renin activity was 13.3 ng/ml/hr. Renal angiography showed stenoses and irregularities in the peripheral branches of renal arteries bilaterally. These findings led to a diagnosis of renovascular hypertension due to vasculitis. Her blood pressure was controlled with an angiotensin-converting enzyme inhibitor and a calcium antagonist. Vasculitis associated with chronic supportive lung disease has occasionally been reported, which suggests a casual relation between chronic respiratory infection and ANCA-related vasculitis. Systemic vasculitis should be taken into account as a potential complication of chronic suppurative lung disease."}, {"id": "article-22910_10", "title": "Histoplasmosis -- History and Physical -- Acute Pulmonary Histoplasmosis", "score": 0.010808190960862716, "content": "Primary infections are frequently asymptomatic, or the patients tend to ignore mild flu-like symptoms. The major determinant of symptoms is the inoculum size. The typical incubation period is seven to 21 days. High fever, headache, nonproductive cough, and chest pain are the main symptoms. This chest pain is not pleuritic and is in the anterior chest and is believed to be due to enlargement of mediastinal or hilar lymph nodes. Most of the symptoms resolve in 10 days. Rheumatologic manifestations can occur such as arthralgias, erythema nodosum or erythema multiforme in a small number of patients (6%, mostly women). This syndrome appears to be less frequent in histoplasmosis when compared to coccidioidomycosis. Imaging shows patchy diffuse pneumonitis and hilar lymphadenopathy. There is no granulomatous inflammation as cellular immunity matures by two weeks in primary infection. During the acute pulmonary infections, a skin test may be positive in more than 90% of cases, antibody to H. capsulatum may be present in 25% to 85% of cases, the urinary antigen may be positive in 20% of cases, and sputum culture from lungs may be positive in less than 25% of cases."}, {"id": "article-28128_26", "title": "Q Fever -- Differential Diagnosis", "score": 0.010704149209352615, "content": "Patients with acute viral infections, such as Epstein-Barr virus, cytomegalovirus, influenza, hepatitis A, B, or C virus, have similar presenting complaints, such as febrile illness, hepatitis, and myalgia. Serological tests and PCR assays are used to identify these infections. Atypical pneumonia caused by Legionella and Mycoplasma needs to be considered. They can be ruled out by detecting urine antigen or serum antibody titers. Tick-borne illnesses such as Lyme disease, relapsing fever, and Rocky Mountain spotted fever manifest with fever and headache with or without a rash. Anaplasmosis and ehrlichiosis present with fever, headache, and hepatitis, with a lesser incidence of rash. Tick-borne diseases are detected via PCR and/or serology. Zoonotic diseases such as brucellosis and leptospirosis can manifest as an acute flu-like illness with a history of exposure to animals or animal products."}, {"id": "pubmed23n0068_15725", "title": "An outbreak of cat-associated Q fever in the United States.", "score": 0.010273701957271937, "content": "Q fever is usually acquired by contact with aerosols generated during parturition of domestic ungulates (e.g., sheep, cows, goats). In the maritime provinces of Canada, parturient cats have also been implicated in its transmission. A 66-year-old woman from eastern Maine developed high fever, rigors, headache, myalgias, pulmonary infiltrates, and elevated hepatocellular enzymes, and the diagnosis of acute Q fever was confirmed serologically. She and 14 other family members had attended a family reunion in Maine 2 weeks earlier, when they were exposed to a parturient cat. All 11 adults and older children attending the reunion developed symptoms consistent with acute Q fever. Serum samples were obtained from 10 who attended the reunion and 8 who did not attend. Titers greater than or equal to 1:64 to Coxiella burnetii were present in all who attended the reunion but in none of those who did not. Cat-associated Q fever should be considered when sporadic cases of the disease occur in the United States."}, {"id": "wiki20220301en086_48572", "title": "Farmer's lung", "score": 0.010037878787878788, "content": "Signs and symptoms Acute Stage: After four to eight hours symptoms such as headache, irritating cough, and shortness of breath upon physical exertion, appear. Subacute Stage: Symptoms persist without further exposure, and increase in severity. Symptoms include: shortness of breath upon exertion, chronic coughing, physical weakness, occasional fever and sweating, decrease in appetite, aches and pains. Chronic Stage: Debilitating effects are now considered long-term. Symptoms include: severe shortness of breath, chronic coughing, physical weakness, occasional fever and sweating at night, decrease in appetite, and general aches and pains. These symptoms develop between four and eight hours after exposure to the antigens. In acute attacks, the symptoms mimic pneumonia or flu. In chronic attacks, there is a possibility of the victim going into shock and dying from the attack."}, {"id": "pubmed23n0569_19775", "title": "[A case of psittacosis with wandering infiltrates developing to acute respiratory distress syndrome].", "score": 0.009900990099009901, "content": "A 52-year-old woman visited a local hospital with a high fever, non-productive cough and general fatigue. Her chest X-ray showed infiltrate in the right middle lung field. Computed tomography scans revealed ground-glass opacity and surrounding ring-shaped air-space consolidation, the \"reversed halo sign\". Cefpirom was administered, but her symptoms persisted and the infiltrate migrated to the left upper lobe. As cryptogenic organizing pneumonia was suspected, she was then treated with intravenously pulsed methylprednisolone followed by prednisolone. Despite these therapies, acute respiratory failure occurred and she was therefore transferred to our hospital. On admission, severe hypoxemia and diffuse bilateral infiltrates on chest images suggested acute respiratory distress syndrome. As we obtained information that a parakeet had recently died at her home, minocycline was administered, resulting in prompt improvement of the symptoms, respiratory insufficiency and pulmonary infiltrates. Finally, elevated antibody titers against Chlamydophila psittasi confirmed a diagnosis of Psittacosis. Sequential chest computed tomography scans in this case indicate that absorption of marginal air-space consolidation with extended central ground glass attenuation in concordance with a new infiltrate on another lung field appeared to create wandering infiltrate. Wandering infiltrate on chest X-ray in psittacosis may be a sign of disease progression."}, {"id": "wiki20220301en029_52548", "title": "Blastomycosis", "score": 0.00980392156862745, "content": "a flu-like illness with fever, chills, arthralgia (joint pain), myalgia (muscle pain), headache, and a nonproductive cough which resolves within days. an acute illness resembling bacterial pneumonia, with symptoms of high fever, chills, a productive cough, and pleuritic chest pain. a chronic illness that mimics tuberculosis or lung cancer, with symptoms of low-grade fever, a productive cough, night sweats, and weight loss. a fast, progressive, and severe disease that manifests as ARDS, with fever, shortness of breath, tachypnea, hypoxemia, and diffuse pulmonary infiltrates. skin lesions, usually asymptomatic, can be verrucous (wart-like) or ulcerated with small pustules at the margins. bone lytic lesions can cause bone or joint pain. prostatitis may be asymptomatic or may cause pain on urinating. laryngeal involvement causes hoarseness. 40% immunocompromised individuals have CNS involvement and present as brain abscess, epidural abscess or meningitis."}, {"id": "pubmed23n0593_9434", "title": "Diagnosis and treatment of Q fever: attempts to clarify current problems in Japan.", "score": 0.009708737864077669, "content": "\"Q fever\" is a generic term for infection caused, mostly in the form of pneumonia or bronchitis, by Coxiella burnetii (Q-fever Coxiella), a pathogen closely related to Rickettsia and Legionella. Q fever is an influenza-like, transient febrile infectious disease that is common to humans and animals; it develops after the transmission of the infectious agent from livestock or pet animals, but person-to-person transmission is rare. In Europe and the United States, it is ranked fourth or fifth as an underlying cause of community-acquired pneumonia. Many patients with Q fever have a good prognosis, and their mortality is about 1%-2% when left untreated. However, because some patients may take a long time to be cured or may have a chronic condition with poor prognosis, patients with definitely diagnosed Q fever or those strongly suspected of having Q fever are strongly recommended to receive treatment. The definite diagnosis of Q fever is made based on a significant increase in serum antibody titers, the determination of which often requires considerable time, and therefore patients must be monitored for a certain period. Q-fever Coxiella, an obligate intracellular parasite, is basically not susceptible to beta-lactam antibiotics, which barely permeate into the cells, but the parasite is susceptible to tetracyclines, macrolides, and quinolones, with these agents being sufficiently permeable into the cells. However, there are many cases of spontaneous cure, and it is likely that beta-lactam treatment may have been involved in these cases. Vaccination against Q fever is not common in Japan."}, {"id": "pubmed23n1051_16743", "title": "SARS-CoV-2 Infection and COVID-19 in 5 Patients in Ecuador After Prior Treatment with Hydroxychloroquine for Systemic Lupus Erythematosus.", "score": 0.009615384615384616, "content": "BACKGROUND This case series describes 5 patients with SARS-CoV-2 infection and COVID-19 in Ecuador who had been treated with hydroxychloroquine for systemic lupus erythematosus (SLE) prior to their COVID-19 illness. CASE REPORT Case #1 reports a 29-year-old woman who had been treated with 200 mg of hydroxychloroquine per day for 1 year and presented with flu-like symptoms, chest pain, fever, odynophagia, asthenia, dry cough, and chills. Case #2 was a 34-year-old woman whose treatment for SLE included 200 mg of hydroxychloroquine per day since 2017. She arrived at the clinic with a dry cough, asthenia, and myalgias. Case #3 was a 24-year-old woman who had been using 200 mg of hydroxychloroquine per day since 2010. She presented with asthenia, myalgias, headaches, hypogeusia, and anosmia. Case #4 was a 39-year-old woman taking 200 mg of hydroxychloroquine every day for SLE who presented with dyspnea, chest pain, odynophagia, hypogeusia, anosmia, diarrhea, and fever. Case #5 was a 46-year-old woman who had been taking 200 mg of hydroxychloroquine since 2019. She came to our hospital complaining of chest pain, fever, and dyspnea. In all 5 patients, SARS-CoV-2 infection was confirmed with a nasopharyngeal SARS-CoV-2 reverse transcription-polymerase chain reaction (RT-PCR) test using the Cepheid/GeneXpert system. CONCLUSIONS All 5 of our patients with SLE who were taking hydroxychloroquine presented with SARS-CoV-2 infection and symptoms of COVID-19. This case series provides support for a lack of prevention of COVID-19 by hydroxychloroquine."}, {"id": "pubmed23n0756_22380", "title": "[Investigation of Coxiella burnetii and Brucella seropositivities in patients presenting with acute fever].", "score": 0.009615384615384616, "content": "Tokat province and Kelkit Valley located in the Black Sea region of Turkey are endemic areas for brucellosis and Crimean-Congo hemorrhagic fever. Since the risk factors are similar, the probability of Coxiella burnetii seroposititivity is assumed to be also high in this area. The aim of this study was to investigate Q fever and brucellosis seropositivity in patients with acute fever. A total of 53 patients (37 male, 16 female; age range: 18-65 years, mean age: 47.13 ± 16.40 years) who were admitted to the emergency room and infection diseases outpatient clinics of Gaziosmanpasa University hospital with acute fever between June 2011-June 2012 were included in the study. Symptoms, physical examination findings and laboratory test results of the patients were recorded. In addition, their place of residence, relationship with rural area, and history of contacts with animals were questioned. The presence of C.burnetii phase II lgM and lgG antibodies were investigated by indirect immunofluorescent antibody assay, and Brucella spp. antibodies by Rose Bengal and standard tube agglutination methods in the serum samples of patients. C.burnetii seropositivity was determined in 19 (36%) of the patients, and 2 (4%) of them were diagnosed as acute Q fever with the positivity of both lgG and lgM antibodies. Among the seropositive and seronegative patients, there was no statistically significant differences in terms of age, gender, animal contact, occupation, place of residence and relationship with rural-life (p&gt; 0.05). Acute fever was attributed to pneumonia in 10 patients and of them five were found positive for phase II lgG antibodies. There was no significant difference between C.burnetii seropositive and seronegative patients in terms of the presence of pneumonia (p= 0.30). In two patients diagnosed as acute Q fever no signs of pneumoniae were detected in the chest X-rays; one of these cases was resided in the city and the other in the rural area while both had contact with animals. The most frequently detected symptoms in patients with acute Q fever were malaise, fatigue, chills, cough, sputum, dyspnea, nausea, abdominal pain and diarrhea. Brucella seropositivity was detected in 6 (11%) patients and four of them were diagnosed as acute brucellosis. Four of the Brucella seropositive patients were also found positive for C.burnetii. Sixteen (84%) of C.burnetii seropositive patients were male and 3 (16%) were female. Eleven of them were living in the village and eight in the city, however six out of eight urban patients had a history of relation with rural-life, resulting a total of 17 (89%) rural-contacts. In addition, 79% (15/19) of seropositive cases had the history of animal contact most commonly with cattle and sheep (11/15; 73%). When the laboratory findings were compared, serum ferritin levels were found to be significantly higher in patients with acute Q fever then those seronegative patients (874 ng/ml mean value vs. 150 ng/mL mean value; p= 0.04), however there was no significant difference between the other laboratory parameters (p&gt; 0.05). Our data indicated that Q fever seropositivity was quite high in Tokat region and the reason may be attributed to entwined life between rural and urban areas. In conclusion in the patients presenting with acute fever, brucellosis and Q fever should be considered in differential diagnosis, since both infections are endemic in that area of Turkey."}, {"id": "pubmed23n1120_5120", "title": "A 15-year-old with chest pain: An unexpected etiology.", "score": 0.009523809523809525, "content": "A 15-year-old female with no significant past medical history presented to the emergency department with 1 day of substernal and pleuritic chest pain, chills, cough, and hematuria. She also had swelling of the face and ankles that resolved by presentation. She was found to have elevated troponin and brain natriuretic peptide during initial workup. Electrocardiogram was normal, but there were significant pleural effusions on chest x-ray. She was strep positive and had blood pressure up to 150/90, prompting admission for cardiac monitoring and cardiology consultation. Blood pressure decreased down to 125/72 without intervention. She was afebrile with unlabored breathing and normal saturations. She was clear to auscultation bilaterally, with no abdominal distension or hepatosplenomegaly, and edema was not evident on exam. There was mild erythema to the bilateral tonsillar pillars. Initial considerations included viral myocarditis, pericarditis, and atypical nephritic syndrome. Workup revealed elevated antistreptolysin antibodies, low C3 complement, negative antineutrophil cytoplasmic antibodies, and negative flu testing. Renal sonography was unremarkable. Cardiology recommended echocardiography, which confirmed pleural effusions but revealed no cardiac abnormalities. Urinalysis revealed hematuria and mild proteinuria. Diagnosis was found to be post-streptococcal glomerulonephritis complicated by fluid overload and left ventricular strain secondary to hypertensive emergency. Post-streptococcal glomerulonephritis is the most common cause of acute glomerulonephritis in children. The mechanism of disease is a proliferation and inflammation of the renal glomeruli secondary to immunologic injury, with deposition of immune complexes, neutrophils, macrophages, and C3 after complement activation. This leads to hematuria, proteinuria, and fluid overload. Edema is present in 65%-90% of patients, progressing to pulmonary involvement in severe cases. Cardiac dysfunction secondary to fluid overload is a potentially fatal outcome in the acute setting. Physicians should consider post-streptococcal glomerulonephritis for patients presenting with hypertension, cardiac/pulmonary pathology, or symptoms of acute heart failure in the context of strep infection."}, {"id": "pubmed23n0110_13531", "title": "Q fever: current concepts.", "score": 0.009523809523809525, "content": "Persons with Q fever usually present with severe retrobulbar headache, a fever to 104 degrees F or higher with shaking chills, general malaise, myalgia, chest pain, and sometimes pneumonia and hepatitis. Cattle, sheep, goats, and ticks are the primary reservoirs of the etiologic agent, Coxiella burnetii. Humans are usually infected by inhaling infectious aerosols. Because C. burnetii can survive for long periods in the environment, it poses a continuing health hazard once it is disseminated. Q fever usually occurs sporadically, but large outbreaks are frequently observed throughout the world, particularly among abattoir workers and personnel working in research centers. Q fever endocarditis follows a chronic course and is frequently fatal. Tests for antibodies to C. burnetii are required for confirmation of the diagnosis. Tetracyclines remain the mainstay of treatment for acute Q fever, and tetracyclines in combination with other antibiotics have been advocated for patients with Q fever endocarditis. Vaccines for Q fever have been proven effective in clinical trials."}, {"id": "pubmed23n1070_21180", "title": "A 47-Year Old Woman With Rapidly Progressive Hypoxemic Respiratory Failure.", "score": 0.009433962264150943, "content": "A 47-year-old Hispanic woman presented to a pulmonology clinic with 2 weeks of cough productive of white sputum and worsening dyspnea on exertion, requiring increasing supplemental oxygen. In addition, she reported fatigue, night sweats, diffuse myalgias, and extremity weakness. She denied hemoptysis, fevers, chills, weight loss, or rash. Her medical history is significant for undifferentiated rapidly progressive hypoxemic respiratory failure 2 years before her current presentation. At that time, she presented to the ED with 3 weeks of progressive shortness of breath and cough. Chest CT imaging showed bilateral infiltrates concerning for infection, and she was treated empirically for community-acquired pneumonia. She developed worsening hypoxemic respiratory failure despite broadening of her antibiotics and subsequently required intubation. Her course was further complicated by pulseless electrical activity arrest with return of spontaneous circulation and development of shock requiring multiple vasopressors. Because of difficulty with oxygenation, she was referred to our center for extracorporeal membrane oxygenation evaluation and was ultimately started on venous-arterial extracorporeal membrane oxygenation. Bronchoscopy with BAL was negative for bacterial, viral, and fungal origins, and initial autoimmune evaluation (antinuclear antibody and rheumatoid factor) was negative, except an elevated creatine kinase (CK) to 3,000. Her course was complicated by heparin-induced thrombocytopenia, and as a result she suffered limb ischemia requiring amputation of her left lower extremity. Elevated CK at that time was attributed to compartment syndrome before amputation. The patient recovered clinically with supportive care and was ultimately discharged on 2 L supplemental oxygen, with a diagnosis of acute respiratory failure of unclear origin. The patient had stability in her clinical symptoms until this current presentation."}, {"id": "pubmed23n0291_13396", "title": "Coxiella burnetii (Q fever) pneumonia.", "score": 0.009433962264150943, "content": "Pneumonia is one manifestation of acute Q fever following infection with Coxiella burnetii. Fever, headache, and myalgia dominate the clinical picture of Q fever pneumonia. Cough is nonproductive and may be absent despite the presence of pneumonia. While in most instances pneumonia results in an illness of mild-to-moderate severity, on occasion it is rapidly progressive and results in respiratory failure. Infection occurs as a result of inhalation of contaminated aerosols. Infected cattle, sheep, and goats are the usual reservoirs for this zoonosis. In some areas, infected parturient cats serve as the reservoir, and in such instances, rounded opacities are seen on the chest radiograph. The diagnosis of C. burnetii pneumonia is usually confirmed by demonstration of a fourfold or greater rise in antibody titer. Treatment is usually with a tetracycline or rifampin for 7 to 10 days."}, {"id": "wiki20220301en025_101159", "title": "Nitrofurantoin", "score": 0.009345794392523364, "content": "Pulmonary toxicity The pulmonary toxicity caused by nitrofurantoin can be categorized into acute, subacute, and chronic pulmonary reactions. The acute and subacute reactions are thought to be due to a hypersensitivity reaction and often resolve when the drug is discontinued. Acute reactions have been estimated to occur in about one in 5000 women who take the drug. These reactions usually develop 3–8 days after the first dose of nitrofurantoin, but may occur from a few hours to a few weeks after starting the drug. Symptoms include fever, dyspnea, chills, cough, pleuritic chest pain, headache, back pain, and epigastric pain. Chest radiograph will often show unilateral or bilateral infiltrates similar to pulmonary edema. Treatment includes discontinuation of the nitrofurantoin, which should result in symptom improvement within 24 hours."}, {"id": "pubmed23n0298_20653", "title": "[Acute bronchiolitis due to Mycoplasma pneumoniae and successfully treated with steroids].", "score": 0.009259259259259259, "content": "A high fever, coughing, stridor, and dyspnea developed in a 52-year-old woman on October 19, 1995. She went to a local clinic and was treated with oral penicillin and intravenous cefpirome. The symptoms worsened, and she was admitted to our hospital on October 26. Coarse crackles and wheezing were heard in both lung fields. The white blood cell count was 9000/mm3 and arterial blood gas analysis revealed a PaO2 of 49.8 Torr on room air. A chest roentgenogram obtained on admission showed a few small bibasilar nodular infiltrates, and a chest CT scan showed thickened bronchial walls along with small nodules having a centrilobular distribution. Of the cells in bronchoalveolar lavage fluid, 88% were neutrophils, but tests for bacteria and mycobacteria were negative. The cold-agglutinin titer was 1:512. The Mycoplasma pneumoniae antibody titer (IIIA) was 1:640 and viral serology tests were negative. Acute bronchiolitis due to M. pneumoniae was diagnosed and treatment with intravenous minocycline was started. The symptoms (coughing, fever, and stridor) resolved and the small nodules on chest CT scan disappeared, but hypoxemia remained. At the same time, an obstructive ventilatory defect (FEV1% 62.8%) and abnormal ventilation/perfusion lung scans were noted. Development into bronchiolitis obliterans was suspected, so administration of methyl prednisolone (1 g/day for 3 days) and prednisolone was started. The response to steroids was good. Pulmonary function improved and the arterial PaO2 at the time of discharge was 86 Torr (room air). Use of steroid therapy in the early phase of bronchiolitis obliterans seemed to be effective."}, {"id": "pubmed23n0323_14081", "title": "Acute Q fever pneumonia: a review of 80 hospitalized patients.", "score": 0.009259259259259259, "content": "To emphasize epidemiologic, clinical, or radiologic characteristics whose detection could lead to an early diagnosis and to enhance therapeutic efficacy. Eighty hospitalized patients from 1982 to 1996. The diagnosis of Q fever infection was serologically confirmed in all the patients (phase II Coxiella burnetii antibody) using the complement fixation test and/or the indirect immunofluorescence antibody test. Patients from rural and urban areas were noted in the same proportion; however, the usual epidemiologic factors such as contact with cats or farm animals were found in 40% of the patients. Mean age+/-SD was 49+/-20 years, and there was a higher sex ratio of male to female patients (1:3.44). We found a specific seasonal distribution since 80% of the cases occurred between February and May. Delay before referring to hospital was 8.2+/-7.8 days, while 69.3% of the patients received an antibiotic treatment that was mainly penicillin or cephalosporin. The dominant clinical features were dry cough and high fever, as the maximal temperature reached more then 40 degrees C in 58% of the patients. Digestive symptoms were rare. WBC count remained within normal range in 80% of the cases with a low proportion of lymphocytes in half of the patients, and the sedimentation rate was usually elevated (55+/-34 mm). Altered liver function consisted more frequently in an elevated level of alkaline phosphatase (70% of the cases) than transaminases, while hyponatremia was frequently mentioned (28.2% of the patients). We found radiologic evidence of unique lobar or segmental alveolar opacity involving more likely the lower lobes in 55 patients, and multiple or interstitial opacities in the others. Chest radiographs were considered normal in eight patients. The clinical response was favorable in all the patients with a reduction in fever 4.8+/-3.9 days after the start of treatment with the second antibiotic that included mainly erythromycin or quinolones, and chest radiographs returned to normal in 81% of the patients within the first month."}, {"id": "pubmed23n0551_20930", "title": "[Clinical manifestation of Q fever and tuberculosis, similarly caused by intracellular parasites].", "score": 0.009174311926605505, "content": "Q fever is a generic term for pneumonia, bronchitis, etc. caused by infection with Coxiella burnetii, a rickettsia-related species of bacteria, in humans. Q-fever is a transient and acute febrile illness that takes a course similar to influenza, and its clinical picture greatly differs from that of tuberculosis that takes a chronic course. The reason for this is thought to be because the generation time of C. burnetii is extremely short (several tens of minutes) compared with Mycobacterium tuberculosis, though those are similar intracellular parasites. Q fever is fourth- or fifth-ranked among the community-acquired pneumonias in the United States and Europe but has a good prognosis with 1-2% of mortality even in the cases that follow a natural course without treatment. Meanwhile, there is a chronic type that follows a protracted course or has a poor prognosis. Therefore, cases definitely diagnosed with Q fever or strongly suspected of Q fever should seek aggressive treatment. Q fever is definitely diagnosed by confirming significant increase in serum antibody titer, but the patients should be followed because in many cases it takes a long time before serum antibody titer increases. Beta-lactams are ineffective against C. burnetii, an obligate intracellular parasite. Although tetracyclines, macrolides, quinolones, rifampicin, etc. are used effectively in the treatment of Q fever, many cases appear to improve by beta-lactam administration because the illness often takes a natural course."}, {"id": "Neurology_Adams_5761", "title": "Neurology_Adams", "score": 0.00909090909090909, "content": "Q fever, unlike the other rickettsioses, is not associated with an exanthem. In the few cases with which we are familiar, the main symptoms were those of a low-grade meningitis. Rare instances of encephalitis, cerebellitis, and myelitis are also reported, possibly as postinfectious complications. There is usually a tracheobronchitis or atypical pneumonia (one in which no organism can be cultured from the sputum) and a severe prodromal headache. In these respects, the pulmonary and neurologic illnesses resemble that of the other main cause of “atypical pneumonia,” M. pneumoniae. The Q fever agent (Coxiella) should be suspected if there are concomitant respiratory and meningoencephalitic illnesses and there has been exposure to parturient animals, to livestock (including abattoir workers, who are also exposed to Brucella and anthrax), or to wild deer or rabbits. The diagnosis can be made by the finding of a severalfold increase in specific immunofixation antibodies. Patients who survive"}, {"id": "pubmed23n0774_24797", "title": "[Symptomatic acute Q fever: a series of 87 cases in an area of Mallorca].", "score": 0.009009009009009009, "content": "Q fever is a widespread zoonotic infection caused by Coxiella burnetii (C. burnetii). Acute infection varies from a self-limited flu-like illness to pneumonia or hepatitis. A retrospective case study from March 2003 to December 2011 was conducted in the Hospital Son Llàtzer in Palma de Mallorca. Acute Q-fever was diagnosed in a patient with clinical suspicion and IgM in phase ii positive (≥ 1/40), with a positive IgG (≥1/80), or when IgG seroconversion was observed during convalescence. A total of 87 cases of acute Q fever were diagnosed. The median age was 50 years (range 21-89), and 69 (79.3%) were male. Fever and headache were the most common symptoms. Pneumonia was diagnosed in 39 (44.8%) patients, febrile episode in 21 (24.1%), and acute hepatitis in 23 (25.6%). Increased serum transaminases were observed in 19 (21.8%). Doxycycline was prescribed in 29 cases (33.4%). There were 30 (34.5%) patients lost to follow up after hospital discharge. A favorable outcome was observed in all other cases. Only one new case progressed to chronic Q fever. A total of 87 cases of acute Q fever were diagnosed. The median age was 50 years (range 21-89), and 69 (79.3%) were male. Fever and headache were the most common symptoms. Pneumonia was diagnosed in 39 (44.8%) patients, febrile episode in 21 (24.1%), and acute hepatitis in 23 (25.6%). Increased serum transaminases were observed in 19 (21.8%). Doxycycline was prescribed in 29 cases (33.4%). There were 30 (34.5%) patients lost to follow up after hospital discharge. A favorable outcome was observed in all other cases. Only one new case progressed to chronic Q fever. Acute Q fever acute is common our environment. Pneumonia was the most common clinical presentation. Even although doxycycline was prescribed in a small number of patients, a favorable outcome was observed in all cases."}, {"id": "pubmed23n1135_7507", "title": "Q-uestioning the Diagnosis: An Educational Case Report.", "score": 0.008928571428571428, "content": "Q fever is a zoonotic infection that may lead to acute or long-term renal injury. Given its rare incidence, Q fever is not often considered on the initial differential diagnosis for glomerular disease which can lead to delays in treatment. This case highlights the importance of avoiding early diagnostic closure and revisiting the differential diagnosis in the setting of an atypical clinical presentation or response to treatment. A 52-year-old female was referred for assessment of possible glomerulonephritis. She described a 3-month history of bilateral lower extremity rash, intermittent knee pain with swelling, and a 2-year history of subjective fevers. Urinalysis showed persistent microscopic hematuria, and her creatinine was elevated at 94 umol/L (baseline 59 umol/L). Her initial investigations included an elevated C-reactive protein (CRP) and rheumatoid factor with a weakly positive anti nuclear antibody (ANA). Kidney biopsy was consistent with an immune complex mesangial proliferative glomerulonephritis. Light microscopy showed diffuse global mesangial hypercellularity. Immunofluorescence was positive for trace mesangial IgG and kappa, 1+ IgM, lambda and C1q, and 2+ C3. Electron microscopy showed mesangial electron dense deposits. These findings were felt to be most in keeping with mesangial proliferative lupus nephritis; however, it was acknowledged that clinical and laboratory findings supporting this diagnosis were lacking. Following treatment with oral prednisone her symptoms resolved, and renal function improved. However, she was unable to taper off prednisone completely without her symptoms returning. Additional immunosuppressive therapies were trialed, but she remained steroid dependent with disease flares related to prednisone tapers. Her atypical response to treatment led to consideration of alternative diagnoses, and further investigation revealed positive Q fever serology (phase-I IgG 1:1892, phase II IgG 1:8192, phase-I and -II IgM &lt; 1:16). She was diagnosed with long-term Q fever and was treated with doxycycline and hydroxychloroquine. She remained on treatment for 2 years. During this time, her symptoms resolved, hematuria disappeared, and her creatinine returned to baseline. Following cessation of therapy, her Q fever IgM titres rose, and she was restarted on doxycycline and hydroxychloroquine indefinitely. (1) Keeping a broad differential diagnosis in the setting of atypical clinical features or unexpected response to therapy is important for ensuring accurate diagnosis and appropriate treatment. (2) Clinical improvement in relation to immunosuppressive therapy does not preclude an infectious cause of glomerular disease."}, {"id": "pubmed23n0362_6235", "title": "[Acute and chronic Q fever; epidemiology, symptoms, diagnosis and therapy of infection caused by Coxiella burnetii].", "score": 0.008928571428571428, "content": "Q fever is a zoonosis caused by Coxiella burnetii, an obligate intracellular bacterium. Domestic ungulates and parturient cats are the primary reservoirs of infection. The animals excrete the bacterium in urine, faeces, milk and amniotic fluid. After desiccation the micro-organism spreads via aerosols. After inhalation or ingestion and an incubation period of 2-6 weeks acute Q fever may develop with atypical pneumonia and hepatitis as major clinical symptoms. The infection also may present as a flu-like illness or remain asymptomatic. Generally, the prognosis is favourable. However, endocarditis or another chronic form of Q fever occasionally develops with possibly fatal outcome. Diagnosis relies upon serologic testing with an indirect immunofluorescence method. Doxycycline is the antibiotic of choice in the treatment of Q fever. Endocarditis needs therapy for years with the addition of rifampin or hydroxychloroquine. Q fever is poorly recognised due to the variety of clinical presentations."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": true, "char_ranges": [[87, 152]], "word_ranges": [[10, 20]], "text": "According to the described examination the Glasgow is 4 (O1V1M2)."}, "2": {"exist": true, "char_ranges": [[153, 235]], "word_ranges": [[20, 30]], "text": "Cerebral perfusion pressure is mean arterial pressure minus intracranial pressure."}, "3": {"exist": true, "char_ranges": [[236, 378]], "word_ranges": [[30, 50]], "text": "Cerebral edema in which cellular edema is produced by the introduction of extracellular fluid into the intracellular compartment is cytotoxic."}, "4": {"exist": true, "char_ranges": [[0, 86]], "word_ranges": [[0, 10]], "text": "Hypercapnia and acidosis produce vasodilatation causing increased cerebral blood flow."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Hypercapnia and acidosis produce vasodilatation causing increased cerebral blood flow. According to the described examination the Glasgow is 4 (O1V1M2). Cerebral perfusion pressure is mean arterial pressure minus intracranial pressure. Cerebral edema in which cellular edema is produced by the introduction of extracellular fluid into the intracellular compartment is cytotoxic.", "full_answer_no_ref": "Hypercapnia and acidosis produce vasodilatation causing increased cerebral blood flow. According to the described examination the Glasgow is 4 (O1V1M2). Cerebral perfusion pressure is mean arterial pressure minus intracranial pressure. Cerebral edema in which cellular edema is produced by the introduction of extracellular fluid into the intracellular compartment is cytotoxic.", "full_question": "A 49-year-old man is admitted to the ICU for traumatic brain injury after an accident at work. In the physical examination he does not open his eyes, does not emit sounds before being intubated and presents extension of extremities to nociceptive stimulus. An intracranial pressure sensor is placed and a decompressive craniotomy must be performed due to intraparenchymal hemorrhage. Which of the following statements is correct?", "id": 579, "lang": "en", "options": {"1": "On arrival she is in a Glasgow scale coma of 7.", "2": "Cerebral perfusion pressure is mean arterial pressure plus intracranial pressure.", "3": "Vasogenic cerebral edema is due to cellular edema, membrane rupture and cell death.", "4": "Cerebral vascular flow increases with hypercapnia and acidosis.", "5": NaN}, "question_id_specific": 108, "type": "NEUROLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0576_5783", "title": "Role of decompressive craniectomy in the management of severe head injury with refractory cerebral edema and intractable intracranial pressure. Our experience with 48 cases.", "score": 0.013378485722647267, "content": "The effects of decompressive craniectomy in the treatment of severe head injury remain unclear. Only very few randomized studies relating to this topic exist in the literature, including a very small number of patients with no class I evidence. We rretrospectively reviewed a series of 221 patients operated on for a head injury during a 25-month period. Of these, 48 patients underwent a decompressive craniectomy. All data available on patients' Glasgow Coma Scale score, pupil size and reaction, and intracranial pressure were collected and analyzed. The patients' outcome was evaluated by the Glasgow Outcome Scale (GOS) and the results compared with the data available in the Traumatic Coma Data Bank. Furthermore, the results were analyzed in respect of the time of surgical intervention (early or late), age, and the preoperative Glasgow Coma Score. Decompressive craniectomy reduced the midline shift in all patients with monolateral diffuse brain edema and contusions having a median value of 7 mm; in the remaining, it ameliorated the basal cisterns effacement. At a mean follow-up of 14 months, 6 (12.5%) patients died, 7 (15%) were discharged home with a GOS of 5, 18 (40%) showed a favorable outcome after rehabilitation with a GOS of 4 and 5, 6 (12.5%) had a severe disability (GOS 3), 9 (20%) were in a vegetative state (GOS 2), and 2 were lost to follow-up. The younger age, earlier surgery, and higher preoperative Glasgow Coma Scale score were related to better outcome (P &lt; .001, P &lt; .05, and P &lt; .034, respectively). Our results seem to support the idea that decompressive craniectomy coupled with neurointensive care may be an effective way to reduce intractable raised intracranial pressure, and probably to improve patients outcome. However, it should be obvious that our results and those available in the literature can not be considered conclusive."}, {"id": "wiki20220301en016_48170", "title": "Cerebral edema", "score": 0.012887666277273242, "content": "Additionally, ventilation with use of positive pressure (PEEP) can improve oxygenation with the negative effect of decreasing cerebral venous drainage and increasing intracranial pressure (ICP), and thus, must be used with caution. Fluid management and cerebral perfusion Maintenance of cerebral perfusion pressure using appropriate fluid management is essential in patients with brain injury. Dehydration, or intravascular volume loss, and the use of hypotonic fluids, such as D5W or half normal saline, should be avoided. Blood serum ion concentration, or osmolality, should be maintained in the normo to hyperosmolar range. Judicial use of hypertonic saline can be used to increase serum osmolality and decrease cerebral edema, as discussed below."}, {"id": "wiki20220301en016_48165", "title": "Cerebral edema", "score": 0.01267222854253571, "content": "Intracranial pressure monitoring Intracranial pressure (ICP) and its management is a fundamental concept in traumatic brain injury (TBI). The Brain Trauma Foundation guidelines recommend ICP monitoring in individuals with TBI that have decreased Glasgow-Coma Scale (GCS) scores, abnormal CT scans, or additional risk factors such as older age and elevated blood pressure. However, no such guidelines exist for ICP monitoring in other brain injuries such as ischemic stroke, intracerebral hemorrhage, cerebral neoplasm."}, {"id": "wiki20220301en016_48140", "title": "Cerebral edema", "score": 0.012235967802927988, "content": "The treatment of cerebral edema depends on the cause and includes monitoring of the person's airway and intracranial pressure, proper positioning, controlled hyperventilation, medications, fluid management, steroids. Extensive cerebral edema can also be treated surgically with a decompressive craniectomy. Cerebral edema is a major cause of brain damage and contributes significantly to the mortality of ischemic strokes and traumatic brain injuries. As cerebral edema is present with many common cerebral pathologies, the epidemiology of the disease is not easily defined. The incidence of this disorder should be considered in terms of its potential causes and is present in most cases of traumatic brain injury, central nervous system tumors, brain ischemia, and intracerebral hemorrhage. For example, malignant brain edema was present in roughly 31% of people with ischemic strokes within 30 days after onset."}, {"id": "wiki20220301en610_27922", "title": "Stages of death", "score": 0.01219047619047619, "content": "The heart and lungs are vital organs for human life due to their ability to properly oxygenate human blood (lungs) and distribute this blood to all vital organs (heart). Hence failure of the heart to pump blood or the lungs to obtain oxygen can lead to a cardiopulmonary death where the heart stops pumping and there is no pulse. In the brain, this can be manifested by a hypoxic state which leads to cerebral edema and thus an increase in intracranial pressure. The rise in intracranial pressure can lead to further disruption in cerebral blood flow, leading to necrosis or tissue death. The aforementioned mechanism is the most common cause of brain death, however this increase in intracranial pressure does not always occur due to an arrest in cardiopulmonary function. Traumatic brain injuries and subarachnoid hemorrhages can also increase the intracranial pressure in the brain leading to a cessation of brain function and hence death. While cardiopulmonary death can be easily assessed by"}, {"id": "wiki20220301en114_11032", "title": "Osmotherapy", "score": 0.01197999217801198, "content": "An increase in cerebral water content is called cerebral edema and it usually results from traumatic brain injury (TBI), subarachnoid hemorrhage (SAH), intracerebral hemorrhage (ICH), subdural hematoma, ischemic stroke, brain tumors, infectious disorders and intracranial surgery. Cerebral edema may result in compromised regional cerebral blood flow (CBF) and intracranial pressure (ICP) gradients which could lead to death of the affected. Increased ICP leads to increased intracranial volume. Unmonitored ICP leads to brain damage by global hypoxic ischemic injury due to reduction in cerebral perfusion pressure (CPP) which is found by subtracting the ICP from mean arterial pressure (MAP), cerebral blood flow, and mechanical compression of brain tissue due to compartmentalized ICP gradients. Cerebral edema is mainly classified into cytotoxic edema, vasogenic edema and interstitial edema."}, {"id": "wiki20220301en016_48186", "title": "Cerebral edema", "score": 0.011165597887589588, "content": "It is important to note that prolonged hyperventilation in those with traumatic brain injuries has been shown to worsen outcomes. Barbiturates Induction of a coma via the use of barbiturates, most notably pentobarbital and thiopental, after brain injury is used for secondary treatment of refractory ICP. Yet their use is not without controversy and it is not clear whether barbiturates are favored over surgical decompression. In patients with traumatic brain injuries, barbiturates are effective in reducing ICP but have failed to show benefit to clinical outcomes. Evidence is limited for their use in cerebral disease that include tumor, intracranial hypertension, and ischemic stroke. There are several adverse effects of barbiturates that limit their use, such as lowering of systemic blood pressure and cerebral perfusion pressure, cardiodepression, immunosuppression, and systemic hypothermia."}, {"id": "pubmed23n0778_19177", "title": "Impact of intracranial pressure measurement on survival in patients with severe traumatic brain injury.", "score": 0.010863165332077249, "content": "The aim of the study was to assess (i) a group of patients with established intracranial pressure (ICP) sensor in severe brain injuries scoring 3 points of Glasgow Coma Scale, (ii) mortality and survival of the patients within periods of 7, 30, 180 and 270 days; and (iii) predictive value of intracranial pressure and cerebral perfusion pressure for short and long-term survival of patients with traumatic brain injury. The group consisted of 61 patients with trauma brain injury scoring 3 points of Glasgow Coma Scale, continuously monitored for intracranial pressure cerebral perfusion pressure at the Intensive Care Unit setting in Nové Zámky. Follow-up period was between 7 and 270 days. Measured values and other recorded data were analysed using methods of descriptive and inferential statistics. ICP values below 20 mmHg were associated with a significantly lower risk of death of an individual patient at particular time. Accordingly, cerebral perfusion pressure values above 70 mmHg during 0-2 days were associated with a significantly higher long-term survival. Overall mortality rates within 30 days showed no peaks on survival curves. In the periods of 0-7 days, within 30 days, and between 30 and 180 days we recorded 24, 51 and 2 deaths, respectively. In the period between 180 and 270 days, mortality was zero. The survival of trauma brain injury patients depends on the speed and quality of pre-hospital care and adequate follow-up treatment at specialized intensive care units. High levels of intracranial pressure and low cerebral perfusion pressure values in the early period after brain injury are closely related to mortality of patients within 30 days. Intracranial pressure monitoring may help to avoid problems and allow intervention before they become life-threatening (Tab. 4, Fig. 4, Ref. 23). Text in PDF www.elis.sk."}, {"id": "wiki20220301en556_5317", "title": "Pressure reactivity index", "score": 0.010374167451267567, "content": "PRx and outcome prediction A high PRx indicating disturbed pressure autoregulation predicts poor outcome in traumatic brain injury. PRx as a treatment target PRx varies with the concurrent cerebral perfusion pressure (CPP) in a U-shaped way. It has been suggested that the CPP with the lowest PRx is optimal (CPPopt) and CPP-values close to optimal have been associated with better outcome. CPP values above CPPopt are believed to cause hyperemia, i.e. to high cerebral blood flow that may cause cerebral edema and intracranial hypertension, whereas CPP values below CPPopt are believed to cause hypoperfusion and ischemia resulting in tissue damage. See also Cerebral autoregulation Intracranial pressure Cushing's triad References Intensive care medicine Neurotrauma Neurophysiology"}, {"id": "article-18523_6", "title": "Brain Death Criteria -- Function", "score": 0.010329131652661064, "content": "Brain death occurs as a result of an acute catastrophic brain injury. Abrupt loss of cerebral perfusion occurs if a concomitant elevation of intracranial pressure is more than mean arterial pressure (cerebral perfusion pressure (CPP) = mean arterial pressure (MAP) - intracranial pressure (ICP). This process was studied by monitoring brain tissue oxygenation in patients with brain death and can occur via two different mechanisms:"}, {"id": "wiki20220301en016_48166", "title": "Cerebral edema", "score": 0.010100074128984433, "content": "Clinical researches have recommended ICP and cerebral perfusion pressure (CPP) monitoring in any persons with cerebral injury who are at risk of elevated intracranial pressure based on clinical and neuroimaging features. Early monitoring can be used to guide medical and surgical decision making and to detect potentially life-threatening brain herniation. There was however, conflicting evidence on the threshold values of ICP that indicated the need for intervention. Researches also recommend that medical decisions should be tailored to the specific diagnosis (e.g. subarachnoid hemorrhage, TBI, encephalitis) and that ICP elevation should be used in conjunction with clinical and neuroimaging and not as an isolated prognostic marker."}, {"id": "pubmed23n0306_11519", "title": "[Intracranial pressure monitoring in patients with severe craniocerebral injury].", "score": 0.009900990099009901, "content": "After severe head injury intracranial pressure (ICP) must be measured continuously for management to assess and maintain the cerebral perfusion. Therefore in our hospital epidural transducers are used. To prove the efficiency of this method in a 12-month period the clinical courses of 23 patients with intracranial pressure transducers were analysed retrospectively. Eighteen patients survived, 5 of them without residuals, 13 with residuals and 2 remained in coma. In 14 patients secondary rises of intracranial pressure were observed between days 3 and 6 post injury. The mean ICP value of the survivors revealed 25 mm Hg. whereas the expired showed 60 mm Hg. In 17 patients the measurements were considered as reliable, 6 measurements were not reliable, which included 1 of the 5 patients who died. One transduce was displaced, another one showed a hemorrhage at the drill hole. There was no infection."}, {"id": "wiki20220301en033_70953", "title": "Traumatic brain injury", "score": 0.00980392156862745, "content": "Secondary injury events include damage to the blood–brain barrier, release of factors that cause inflammation, free radical overload, excessive release of the neurotransmitter glutamate (excitotoxicity), influx of calcium and sodium ions into neurons, and dysfunction of mitochondria. Injured axons in the brain's white matter may separate from their cell bodies as a result of secondary injury, potentially killing those neurons. Other factors in secondary injury are changes in the blood flow to the brain; ischemia (insufficient blood flow); cerebral hypoxia (insufficient oxygen in the brain); cerebral edema (swelling of the brain); and raised intracranial pressure (the pressure within the skull). Intracranial pressure may rise due to swelling or a mass effect from a lesion, such as a hemorrhage. As a result, cerebral perfusion pressure (the pressure of blood flow in the brain) is reduced; ischemia results. When the pressure within the skull rises too high, it can cause brain death or"}, {"id": "pubmed23n0306_1310", "title": "Effects of varying levels of positive end-expiratory pressure on intracranial pressure and cerebral perfusion pressure.", "score": 0.00980392156862745, "content": "To determine the influence of positive end-expiratory pressure (PEEP) on intracranial pressure and cerebral perfusion pressure. Neurosurgical intensive care patients requiring intracranial pressure monitoring and mechanical ventilation were studied in a randomized, controlled study. Tertiary care, neurosurgical intensive care unit. Eighteen patients were enrolled in the study. Patients had posttraumatic head injuries (n = 9), subarachnoid hemorrhage (n = 7), obstructive hydrocephalus (n = 1), and intracerebral hemorrhage of unknown cause (n = 1). Patients had PEEP levels of 5, 10, and 15 cm H2O applied to their lungs. Changes in intracranial pressure, mean arterial pressure, and cerebral perfusion pressure were measured. The results were analyzed separately for patients with normal and increased intracranial pressure (&gt; 15 mm Hg). PEEP at 5 cm H2O had no effect on intracranial pressure in the group with normal intracranial pressure. However, PEEP at 10 and 15 cm H2O produced a significant (p &lt; .05) increase in intracranial pressure (1.9 and 1.5 mm Hg, respectively). In the group with increased intracranial pressure, no significant change in intracranial pressure occurred at any of the PEEP levels used. In both groups, cerebral perfusion pressure was unchanged throughout. In patients with normal intracranial pressure, PEEP at 5 cm H2O did not significantly alter intracranial pressure. The clinical relevance of the intracranial pressure increase at PEEP levels of 10 and 15 cm H2O is questionable because cerebral perfusion pressure did not change and remained &gt; 60 mm Hg. In patients with increased intracranial pressure, higher levels of PEEP did not significantly change intracranial pressure or cerebral perfusion pressure."}, {"id": "wiki20220301en082_15099", "title": "Blunt trauma", "score": 0.00973885094415428, "content": "Most patients with more severe traumatic brain injury have of a combination of intracranial injuries, which can include diffuse axonal injury, cerebral contusions, and intracranial bleeding, including subarachnoid hemorrhage, subdural hematoma, epidural hematoma, and intraparenchymal hemorrhage. The recovery of brain function following a traumatic accident is highly variable and depends upon the specific intracranial injuries that occur, however there is significant correlation between the severity of the initial insult as well as the level of neurologic function during the initial assessment and the level of lasting neurologic deficits. Initial treatment may be targeted at reducing the intracranial pressure if there is concern for swelling or bleeding within this skull, which may require surgery such as a hemicraniectomy, in which part of the skull is removed. Blunt trauma to extremities"}, {"id": "wiki20220301en016_48150", "title": "Cerebral edema", "score": 0.009708737864077669, "content": "There are several clinical conditions in which vasogenic edema is present: CNS tumors, like glioblastoma and meningioma Infections like meningitis, abscess, and encephalitis Inflammatory central nervous system disease such as multiple sclerosis Brain hemorrhage Traumatic brain injuries can lead to increased intracranial pressure, local damage, reduced cerebral blood flow, and focal ischemia secondary to vasogenic edema. Late stage of ischemic stroke after rapid recovery from cytotoxic edema Hypertensive encephalopathy Radiation injury Ionic (Osmotic) In ionic edema, the solute concentration (osmolality) of the brain exceeds that of the plasma and the abnormal pressure gradient leads to accumulation of water intake into the brain parenchyma through the process of osmosis. The blood-brain barrier is intact and maintains the osmotic gradient. The solute concentration of the blood plasma can be diluted by several mechanisms:"}, {"id": "pubmed23n0356_2981", "title": "[Craniocerebral injuries: initial treatment and at resuscitation units].", "score": 0.009708737864077669, "content": "Head injury is the main cause of death or disability among under-45-year-olds. This review covers the main pathophysiological aspects of head injury as well as initial treatment and management in the intensive care recovery ward. The chief therapeutic aim is to maintain adequate cerebral perfusion pressure rather than to maintain normal levels of intracranial pressure. An important challenge is to avoid development of secondary lesions, and in this context arterial hypotensive events that affect prognosis and the survival of such patients merit special attention. We reject treatment based on indiscriminate use of hyperventilation and mannitol and underline the importance of providing adequate sedation and analgesia while maintaining normal flow and pressure and adequate monitoring of such patients."}, {"id": "wiki20220301en020_53865", "title": "Intracranial pressure", "score": 0.009615384615384616, "content": "Pathophysiology Cerebral perfusion pressure (CPP), the pressure of blood flowing to the brain, is normally fairly constant due to autoregulation, but for abnormal mean arterial pressure (MAP) or abnormal ICP the cerebral perfusion pressure is calculated by subtracting the intracranial pressure from the mean arterial pressure: CPP = MAP − ICP . One of the main dangers of increased ICP is that it can cause ischemia by decreasing CPP. Once the ICP approaches the level of the mean systemic pressure, cerebral perfusion falls. The body's response to a fall in CPP is to raise systemic blood pressure and dilate cerebral blood vessels. This results in increased cerebral blood volume, which increases ICP, lowering CPP further and causing a vicious cycle. This results in widespread reduction in cerebral flow and perfusion, eventually leading to ischemia and brain infarction. Increased blood pressure can also make intracranial hemorrhages bleed faster, also increasing ICP."}, {"id": "pubmed23n0249_19049", "title": "Intracranial pressure and cerebral perfusion pressure in severe head injury.", "score": 0.009615384615384616, "content": "Monitoring and management of intracranial pressure (ICP) are fundamental to modern neurotraumatology. Although never formally proven to independently improve outcome in prospective, randomized, placebo-controlled trials, there is such a predominance of indirect support for this modality that most neurotrauma protocols are impossible with-out its inclusion and ethical considerations virtually preclude placebo-controlled trials of its efficacy. In addition to the question of improving outcome, ICP monitoring is also useful in guiding the use of potentially harmful treatment modalities such as hyperventilation, mannitol, and barbiturates, and also provides important prognostic data. ICP monitoring provides information on the likelihood of cerebral herniation and allows calculation of the cerebral perfusion pressure (CPP). Although there is no constant threshold for herniation, the most commonly used treatment threshold is 20 to 25 mm Hg. In addition, ICP trends are indispensable in providing the earliest possible indication of critical intracranial mass effects when combined with other clinical indicators. CPP is the difference between mean arterial pressure and ICP. CPP is an important clinical indicator of cerebral blood flow (CBF). Cerebral autoregulation generally remains at least partially preserved after severe head injury, although the CPP value at which it is activated appears to be shifted upward. Therefore, maintaining adequate CBF appears to require using an elevated minimal CPP threshold when treating the injured brain. A generally accepted value of 70 mm Hg is suggested."}, {"id": "wiki20220301en016_48142", "title": "Cerebral edema", "score": 0.009545227386306848, "content": "Increased intracranial pressure (ICP) is a life-threatening surgical emergency marked by symptoms of headache, nausea, vomiting, decreased consciousness. Symptoms are frequently accompanied by visual disturbances such as gaze paresis, reduced vision, and dizziness. Increased pressures within the skull can cause a compensatory elevation of blood pressure to maintain cerebral blood flow, which, when associated with irregular breathing and a decreased heart rate, is called the Cushing reflex. The Cushing reflex often indicates compression of the brain on brain tissue and blood vessels, leading to decreased blood flow to the brain and eventually death. Causes Cerebral edema is frequently encountered in acute brain injuries from a variety of origins, including but not limited to:"}, {"id": "pubmed23n0772_11758", "title": "[Prognostic correlation of intracranial pressure monitoring in patients with severe craniocerebral injury].", "score": 0.009523809523809525, "content": "To explore the clinical application of intracranial pressure (ICP) monitoring and its prognostic correlation in patients with severe craniocerebral injury. A total of 216 severe craniocerebral injury patients with scores of Glasgow coma scale 3-8 underwent craniotomy at Affiliated Qilu Hospital, Shandong University.And 168 cases of ICP monitoring were divided into 3 treatment groups and another 48 cases without ICP monitoring selected as the control group.According to ICP, stepwise treatment was administered to control the level of ICP and maintain the cerebral perfusion pressure to analyze the relationship between ICP monitoring and prognosis. As compared with the control group, there were significant decreases of disability and mortality rate for patients with ICP monitoring (A, B, C group). Especially group C had a better prognosis than the other groups for statistical significance.In addition, the dose and duration of mannitol of group A, B or C were significantly lower than those of the control group (P &lt; 0.05). The application of ICP monitoring is capable of reducing mortality, improving prognosis and enhancing success rate of treating severe craniocerebral injury."}, {"id": "wiki20220301en170_30297", "title": "Middle cerebral artery syndrome", "score": 0.009433962264150943, "content": "Depending upon the location and severity of the occlusion, signs and symptoms may vary within the population affected with MCA syndrome. More distal blockages tend to produce milder deficits due to more extensive branching of the artery and less ischemic response. In contrast, the most proximal occlusions result in widespread effects that can lead to significant cerebral edema, increased intracranial pressure, loss of consciousness and could even be fatal. In such occasions, mannitol (osmotic diuretic) or hypertonic saline are given to draw fluid out of the edematous cerebrum to minimise secondary injury. Hypertonic saline is better than mannitol, as mannitol being a diuretic will decrease the mean arterial pressure and since cerebral perfusion is mean arterial pressure minus intracranial pressure, mannitol will also cause a decrease in cerebral perfusion."}, {"id": "pubmed23n0066_17066", "title": "Intracranial monitoring in patients with head trauma.", "score": 0.009433962264150943, "content": "The treatment of head-injured patients has over the past 15 years become significantly more complex and involved. The rising interest in the treatment of these patients has been driven in large part by the growing body of evidence showing that there is a definite association between elevated ICP and increased mortality and long-term morbidity. The effective treatment of intracranial hypertension with hyperventilation, osmotic agents, and barbiturate therapy has been aided by the regular use of ICP monitoring in most neurosurgical centers. A continuing and ongoing record of ICP allows for the judicial and appropriate use of modern treatment modalities."}, {"id": "pubmed23n0020_7884", "title": "Long-term intracranial pressure monitoring in comatose patients suffering from head injuries. A critical survey.", "score": 0.009345794392523364, "content": "On the basis of a series of 75 patients, the practical use of Icp continuous long-term recording in severe head injuries without mass lesions or remaining deeply comatose after surgical procedures is critically analyzed. ICP monitoring alone seems to be not essential for prognosis. Conversely it is of much greater use as a guide to management (respirator treatment, osmotics, CSF drainage). If the pros and cons of the procedure are carefully weighed, it would appear that, for the time being, ICP long-term monitoring is justified only in comatose patients on intensive care."}, {"id": "pubmed23n0500_4353", "title": "Is there an upper limit of intracranial pressure in patients with severe head injury if cerebral perfusion pressure is maintained?", "score": 0.009259259259259259, "content": "Authors of recent studies have championed the importance of maintaining cerebral perfusion pressure (CPP) to prevent secondary brain injury following traumatic head injury. Data from these studies have provided little information regarding outcome following severe head injury in patients with an intracranial pressure (ICP) greater than 40 mm Hg, however, in July 1997 the authors instituted a protocol for the management of severe head injury in patients with a Glasgow Coma Scale score lower than 9. The protocol was focused on resuscitation from acidosis, maintenance of a CPP greater than 60 mm Hg through whatever means necessary as well as elevation of the head of the bed, mannitol infusion, and ventriculostomy with cerebrospinal fluid drainage for control of ICP. Since the institution of this protocol, nine patients had a sustained ICP greater than 40 mm Hg for 2 or more hours, and five of these had an ICP greater than 75 mm Hg on insertion of the ICP monitor and later experienced herniation and expired within 24 hours. Because of the severe nature of the injuries demonstrated on computerized tomography scans and their physical examinations, these patients were not aggressively treated under this protocol. The authors vigorously attempted to maintain a CPP greater than 60 mm Hg with intensive fluid resuscitation and the administration of pressor agents in the four remaining patients who had developed an ICP higher than 40 mm Hg after placement of the ICP monitor. Two patients had an episodic ICP greater than 40 mm Hg for more than 36 hours, the third patient had an episodic ICP greater than of 50 mm Hg for more than 36 hours, and the fourth patient had an episodic ICP greater than 50 mm Hg for more than 48 hours. On discharge, all four patients were able to perform normal activities of daily living with minimal assistance and experience ongoing improvement. Data from this preliminary study indicate that intense, aggressive management of CPP can lead to good neurological outcomes despite extremely high ICP. Aggressive CPP therapy should be performed and maintained even though apparently lethal ICP levels may be present. Further study is needed to support these encouraging results."}, {"id": "wiki20220301en105_5826", "title": "Decompressive craniectomy", "score": 0.009191919191919192, "content": "Other effects In addition to reducing ICP, studies have found decompressive craniectomy to improve cerebral perfusion pressure and cerebral blood flow in head injured patients. Decompressive craniectomy is also used to manage major strokes, associated with \"malignant\" edema and intracranial hypertension. The pooled evidence from three randomised controlled trials in Europe supports the retrospective observations that early (within 48 hours) application of decompressive craniectomy after \"malignant\" stroke may result in improved survival and functional outcome in patients under the age of 55, compared to conservative management alone. The procedure is recommended especially for young patients in whom ICP is not controllable by other methods. Age of greater than 50 years is associated with a poorer outcome after the surgery. Complications Infections such as meningitis or brain abscess can occur after decompressive craniectomy."}, {"id": "wiki20220301en016_48149", "title": "Cerebral edema", "score": 0.009174311926605505, "content": "Vasogenic Extracellular brain edema, or vasogenic edema, is caused by an increase in the permeability of the blood-brain barrier. The blood-brain barrier consists of astrocytes and pericytes joined together with adhesion proteins producing tight junctions. Return of blood flow to theses cells after an ischemic stroke can cause excitotoxicity and oxidative stress leading to dysfunction of the endothelial cells and disruption of the blood-brain barrier. The breakdown of the tight endothelial junctions that make up the blood–brain barrier causes extravasation of fluid, ions, and plasma proteins, such as albumin, into the brain parenchyma. Accumulation of extracellular fluid increases brain volume and then intracranial pressure causing the symptoms of cerebral edema. There are several clinical conditions in which vasogenic edema is present:"}, {"id": "pubmed23n0217_5778", "title": "[Intracranial pressure in severe brain injuries. 2nd Part: Therapeutic interests and prognosis].", "score": 0.009174311926605505, "content": "Sixty-seven patients with severe head injury underwent intracranial pressure (ICP) monitoring (10 extradural and 57 intraventricular). All patients had Liege coma scale (LCS) score of 12 or less. Ventriculitis (E. Coli) occurred in one patient (1,8%). The intraventricular method allows C.S.F. drainage with lowering of I.C.P. even with slit ventricles. After 8 days, if an intracranial hypertension persists, we perform a ventriculo-atrial drainage. Pressure recording is a useful way of following the patient's evolution and a guide for prognosis of survival. If I.C.P. is constantly below 5 torr, then the probability of a bad outcome is great. The study also confirms the high mortality rate (93%) if I.C.P. is greater than 40 torr."}, {"id": "pubmed23n0069_14926", "title": "[Intracranial pressure and brain death].", "score": 0.00909090909090909, "content": "Cerebral death occurs during reanimation as an isolated destruction of the entire brain. It is the result of a malignant and irreversible increase of the intracranial pressure. Continuous registration of the intracranial and systemic blood pressures which is done as a routine monitoring procedure in the majority of deep coma patients, allows to identify the moment when cerebral perfusion has come to a complete standstill, and also allows to confirm its irreversibility. At the end of an ischaemic period of 8 to 10 minutes, absolutely lethal to brain tissue, cerebral death is completed. To be on the safe side, the expiration of a 15 to 20 minute period of complete circulatory arrest within the cranial cavity is recommended before further diagnostic measures, especially cerebral arteriography, are undertaken as final proof of dissociated brain death, permitting the explantation of vital organs for grafting. At present, due to possible technical difficulties, reliance upon epidural intracranial pressure measurement alone must still be discouraged. Nevertheless, this investigation method can be most useful in the early timing of the so-called terminal angiography in order not to delay the diagnosis of brain death and its medical consequences."}, {"id": "wiki20220301en020_53863", "title": "Intracranial pressure", "score": 0.009065749121101054, "content": "One of the most damaging aspects of brain trauma and other conditions, directly correlated with poor outcome, is an elevated intracranial pressure. ICP is very likely to cause severe harm if it rises too high. Very high intracranial pressures are usually fatal if prolonged, but children can tolerate higher pressures for longer periods. An increase in pressure, most commonly due to head injury leading to intracranial hematoma or cerebral edema, can crush brain tissue, shift brain structures, contribute to hydrocephalus, cause brain herniation, and restrict blood supply to the brain. It is a cause of reflex bradycardia. Low ICP"}, {"id": "wiki20220301en186_21521", "title": "Neurointensive care", "score": 0.009020083102493075, "content": "Basic life support monitoring: Electrocardiography, pulse oximetry, blood pressure, assessment of comatose patients. Neurological monitoring : Serial neurologic examination, assessment of comatose patients (Glasgow Coma Scale plus pupil or four score), ICP (subarachnoid hemorrhages, TBI, Hydrocephalus, Stroke, CNS infection, Hepatic failure), multimodality monitoring to monitor disease and prevent secondary injury in states that are insensitive to neurological exam or conditions confounded by sedation, neuromuscular blockade and coma. Intracranial pressure (ICP) management: Ventricular catheter to monitor Brain oxygen and concentrations of glucose and PH. With treatment options of Hypertonic serum, barbiturates, hypothermia and decompressive hemi-craniotomy Common neurointensive care illnesses and treatments Traumatic brain injury: Sedation, ICP monitoring and management, Decompressive Craniectomy, Hyperosmolar therapy and maintain hemodynamic stability."}, {"id": "pubmed23n0695_12954", "title": "Hypercapnic cerebral edema presenting in a woman with asthma: a case report.", "score": 0.009009009009009009, "content": "Common causes of non-traumatic acute cerebral edema include malignant hypertension, hyponatremia, anoxia, and cerebral vascular accident. The computed tomographic images and data obtained during care of the patient described in this case report provide evidence that hypercarbia can cause increased intracranial pressure and coma without permanent brain injury. Partial pressure of carbon dioxide evaluation for coma is essential to provide faster diagnosis and therapeutic correction in certain common critical disease states. We present the case of a patient in a coma associated with cerebral edema during a typical asthma exacerbation with hypercapnic respiratory failure. An obese 63-year-old African American woman with asthma presented to our hospital with facial swelling and shortness of breath. Immediately following intubation for hypercapnic respiratory failure, she was noted to have a dilated, unresponsive right pupil. An emergent computed tomographic head scan revealed that she had increased intracranial pressure. A neurosurgeon agreed with the computed tomography interpretation and recommended no surgical intervention. The patient's respiratory acidosis was corrected with ventilatory management over several hours in the intensive care unit. Nine and one-half hours later a follow-up head computed tomographic scan was read as normal without cerebral edema. At 12 hours, the patient's right pupil was 5 mm in diameter and reactive. By 24 hours, her pupils were symmetrically equal and reactive. Her symptoms had improved, and she was extubated. A brain magnetic resonance imaging scan revealed no abnormalities. Alteration of consciousness related to hypercapnia during respiratory failure is not generally thought to be related to cerebral edema. Respiratory acidosis resulting from hypercarbia is known to produce carbon dioxide narcosis and coma, but no current treatment algorithm suggests that rapid hypercapnia correction can be critical to neurologic outcome. To the best of our knowledge, our case is a unique example of the physiological changes that may occur in relation to arterial carbon dioxide concentration in the normal brain in the setting of typical hypercapnic respiratory failure. Correction of respiratory acidosis reversed the neurologic symptoms and physiology causing cerebral edema and coma in our patient. Rare similar cases have been sporadically reported in the medical literature, typically in children. Our case is also unusual in that rapid deterioration and clinical status were directly observed on simultaneous computed tomographic scans. Had this patient been found unresponsive, or had she had brief respiratory or cardiac arrest, the scan could have been interpreted as global anoxic injury leading to a different therapeutic course."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": true, "char_ranges": [[0, 183]], "word_ranges": [[0, 30]], "text": "Answers 1, 2 and 5 are appropriate treatments for dumping syndrome or postgastrectomy, but the question is focused on initial management, so the most appropriate answer seems to be 2."}, "2": {"exist": true, "char_ranges": [[0, 183]], "word_ranges": [[0, 30]], "text": "Answers 1, 2 and 5 are appropriate treatments for dumping syndrome or postgastrectomy, but the question is focused on initial management, so the most appropriate answer seems to be 2."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": true, "char_ranges": [[0, 183]], "word_ranges": [[0, 30]], "text": "Answers 1, 2 and 5 are appropriate treatments for dumping syndrome or postgastrectomy, but the question is focused on initial management, so the most appropriate answer seems to be 2."}}, "full_answer": "Answers 1, 2 and 5 are appropriate treatments for dumping syndrome or postgastrectomy, but the question is focused on initial management, so the most appropriate answer seems to be 2.", "full_answer_no_ref": "Answers 1, 2 and 5 are appropriate treatments for dumping syndrome or postgastrectomy, but the question is focused on initial management, so the most appropriate answer seems to be [HIDDEN].", "full_question": "A 45-year-old man undergoes a truncal vagotomy and antrectomy with Billroth II reconstruction for chronic peptic ulcer disease with pyloro-duodenal stricture. Six weeks after the surgery she reports that shortly after (less than half an hour) after ingestions she presents nausea, asthenia and sweating, dizziness and abdominal cramps usually accompanied by diarrhea. Which of the following is the most appropriate approach for her initial management?", "id": 212, "lang": "en", "options": {"1": "Apply treatment with a somatostatin inhibitor (octreotide).", "2": "Follow specific dietary measures.", "3": "Trial treatment with a benzodiazepine.", "4": "Search for a probable neuroendocrine tumor (e.g. carcinoid).", "5": "Indicate surgical treatment to perform an antiperistaltic Roux-en-Y gastrojejunostomy."}, "question_id_specific": 88, "type": "GENERAL SURGERY", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0285_22989", "title": "[Peptic ulcer: late complications of the surgical treatment].", "score": 0.016298946531504672, "content": "The incidence of surgical treatment of peptic ulcer decreased in the last two decades. The majority of procedures for surgical management of peptic disease impairs the ability of the stomach to receive and to store food. The intake of high protein-caloric content diets can improve some nutritional deficits expressed by loss of body weight and anemia. The mechanism responsible for diarrhea is unknown, but truncal vagotomy has the highest incidence. It is usually episodic, lessens over the first year after operation and rarely remains a severe problem. The decreasing levels of colecistokinin response after meal in gastrectomy and the division of hepatic branch of anterior vagus can cause gallbladder sludge and stone formation. Alkaline reflux explains gastritis and esophagitis after partial gastric resection. Surgical duodenal diversion, like a Roux-en-Y limb, have been successful in its control. The mechanism that leads to the dumping syndrome are loss of gastric reservoir function and rapid emptying of hyperosmolar meals into small intestine. Somatostatin analogues improve the symptoms caused by abnormal release of neurohormonal agents responsible of the behaviour of the gastrointestinal tract after meals. Cancer of gastric remanent may be due to increased bacterial overgrowth and nitrosation formation. The endoscopic follow-up is essential for early diagnosis of the stump cancer. In spite of all complications, the surgeon cannot have hesitations by carrying out radical approach meanly during catastrophic emergencies of peptic disease i.e. in elderly aged patients. Nowadays, the control of chronic sequelas is easy with conservative therapeutic."}, {"id": "pubmed23n0583_10985", "title": "Diagnosis and treatment of gastrinoma in the era of proton pump inhibitors.", "score": 0.015271872902215261, "content": "The Zollinger-Ellison syndrome is characterized pathophysiologically by a significant hypergastrinemia derived from a gastrin-secreting neuroendocrine tumor with a primary location in the pancreas or duodenum. Chronic hypergastrinemia in turn triggers gastric acid hypersecretion yielding in chronic or recurrent or refractory peptic ulcer disease and/or chronic diarrhea. One half of patients with ZES will have distant metastases in the liver by the time the diagnosis is established and one half of all patients with ZES will experience chronic diarrhea as chief complaint rather than peptic ulcer-related symptoms and signs. Gastrinomas have been reported to either manifest sporadically or to occur in conjunction with the genetic background of the MEN-I syndrome. Diagnosis is based on the patients history which is typically characterized by recurrent episodes of peptic ulcer disease or by severe reflux esophagitis and/or diarrhea or by acid-related symptoms which fail to respond to standard treatment regimens. Upper gastrointestinal tract endoscopy will provide evidence for peptic ulcer disease in anatomical regions located aborally the duodenal bulb within the descending part of the duodenum or even farther distally within the jejunum. Peptic ulcers frequently occur in groups indicating some substantial acid hypersecretion. A gastric pH &gt; 2 is mutually exclusive for ZES. Increased serum gastrin levels confirm the diagnosis biochemically. Gastrin secretion can be determined in the basal state or following stimulation with secretin or calcium. High sensitivity and specificity for the diagnosis of ZES is provided by determining the ratio of basal versus pentagastrin-stimulated gastric acid secretion: The ratio of BAO / MAO &gt; 0.6 is highly specific for gastrinoma. To localize the gastrin-secreting tumor computer-assisted tomography, endoscopic ultrasound, and somatostatin receptor scintigraphy provide useful help but most recently, endoscopic ultrasound with high resolution transducers appear to improve preoperative site localization. If modern imaging techniques fail to elucidate the site of the tumor, intraoperative diaphany may help to detect gastrinomas within the duodenal wall. Definitive treatment will only be achieved by total surgical resection of the gastrin-producing tumor in the pancreas or duodenum including dissection of the regional lymph nodes. Control of symptoms will have to be achieved by administration of highly potent proton pump inhibitors in up to 2-3-fold increased standard doses to inhibit gastric acid hypersecretion. Elevation of gastric pH &gt; 4 will be the therapeutic target to protect the mucosa of the upper gastrointestinal tract. Basal acid output should be reduced to less than 10 mEq H(+) per hour which requires administration of highly potent proton pump inhibitors with a recommended starting dose of 60 mg omeprazole equivalents per day."}, {"id": "pubmed23n0759_11962", "title": "Octreotide improves early dumping syndrome potentially through incretins: a case report.", "score": 0.011927546138072453, "content": "Dumping syndrome, or rapid gastric emptying, is a frequent complication after gastric surgery. In this case, the patient was a 47-year-old woman who 10 years previously had undergone distal gastrectomy with Billroth I reconstruction for early-stage gastric cancer. She presented with symptoms of weakness, headache, palpitation, sweating, dizziness and significant fatigue between one and two hours after a meal. Because a 75 g oral glucose tolerance test (75 g-OGTT) induced both acute postprandial tachycardia (within 1 hour) and postprandial hypoglycemia, we diagnosed this patient with early and late dumping syndrome. Dietary measures and acarbose improved symptoms of late dumping syndrome but did not prevent the symptoms of early dumping syndrome such as postprandial tachycardia, weakness, headache, palpitation, and dizziness. We therefore used the somatostatin analogue octreotide, which has been reported as an effective therapy for early dumping syndrome. Octreotide prevented the symptoms of early dumping syndrome, especially postprandial tachycardia, but caused postprandial hyperglycemia. Glucagon-like peptide-1 (GLP-1) and glucose-dependent insulinotropic polypeptide (GIP) were completely suppressed during the 75 g-OGTT following subcutaneous injection of octreotide. No change was observed in vasoactive intestinal polypeptide (VIP), which is the gastrointestinal peptide hormone generally responsible for early dumping syndrome, suggesting possible contribution of incretins in early dumping syndrome of this patient."}, {"id": "wiki20220301en461_3354", "title": "Post-vagotomy diarrhea", "score": 0.01150277459504392, "content": "Post-vagotomy diarrhea is a form of diarrhea which occurs in 10% of people after a truncal vagotomy, which can range from severe to debilitating in approximately 2% to 4% of patients. However, the occurrence of post-vagotomy diarrhea is significantly reduced after proximal selective vagotomy, specifically when celiac and hepatic branches of the vagus are retained. Diagnosis Treatment Surgical treatment for refractory post-vagotomy diarrhea is rarely needed and at least one year from the occurrence of symptoms should be allotted to ensure all non-surgical treatments have been appropriately explored. Under severe cases, where surgical intervention does become necessary, a 10 cm reverse jejunal interposition is usually the procedure of choice. References Neurosurgery Diarrhea"}, {"id": "wiki20220301en119_17621", "title": "Gastric outlet obstruction", "score": 0.009900990099009901, "content": "Treatment Treatment of gastric outlet obstruction depends on the cause, but is usually either surgical or medical. Medication In most people with peptic ulcer disease, the oedema will usually settle with conservative management with nasogastric suction, replacement of fluids and electrolytes and proton pump inhibitors. Surgery Surgery is indicated in cases of gastric outlet obstruction in which there is significant obstruction and in cases where medical therapy has failed. Endoscopic balloon therapy may be attempted as an alternative to surgery, with balloon dilation reporting success rates of 76% after repeat dilatons. The operation usually performed is an antrectomy, the removal of the antral portion of the stomach. Other surgical approaches include: vagotomy, the severing of the vagus nerve, the Billroth I, a procedure which involves anastomosing the duodenum to the distal stomach, or a bilateral truncal vagotomy with gastrojejunostomy. References External links"}, {"id": "pubmed23n0058_19433", "title": "Treatment of severe postvagotomy/postgastrectomy symptoms with the somatostatin analogue octreotide.", "score": 0.009900990099009901, "content": "Fourteen patients with severe and persistent postvagotomy/postgastrectomy symptoms were entered into a trial of treatment with the somatostatin analogue octreotide, 50 micrograms twice daily 30 min before meals being self-administered by subcutaneous injection. Six of the seven patients completing the 3-month trial showed sustained overall improvement of symptoms. The remaining patients were unhelped by treatment or developed unwanted effects. Six of eight patients with dumping syndrome showed sustained improvement of dumping symptoms during treatment. Bile vomiting was relieved in three of four patients with this complaint. Diarrhoea accompanying dumping showed a variable response to treatment, with improvement in three patients and no change or worsening of this symptom in five. Two patients with severe postvagotomy diarrhoea alone showed no improvement. Four patients with unwanted effects and three patients who found no benefit stopped the trial medication early. Four further patients reported mild or transient side-effects. For patients with severe postvagotomy/postgastrectomy symptoms, a trial of octreotide seems justified when significant dumping symptoms are present and other treatment options have been exhausted."}, {"id": "wiki20220301en105_42912", "title": "Vagotomy", "score": 0.00980392156862745, "content": "All types of vagotomy can be performed at open surgery (laparotomy) or using minimally invasive surgery (laparoscopy). For the management of PUD, vagotomy is sometimes combined with antrectomy (removal of the distal half of the stomach) to reduce the rate of recurrence. Reconstruction is performed with gastroduodenostomy (Billroth I) or gastrojejunostomy (Billroth II). It is left intact in highly selective vagotomy so the function of gastric emptying remains intact. Applications Truncal vagotomy is a treatment option for chronic duodenal ulcers. It was once considered the gold standard, but is now usually reserved for patients who have failed the first-line \"triple therapy\" against Helicobacter pylori infection: two antibiotics (clarithromycin and amoxicillin or metronidazole) and a proton pump inhibitor (e.g., omeprazole). It is also used in the treatment of gastric outlet obstruction."}, {"id": "pubmed23n0646_12484", "title": "[Diarrhoea, nausea and vomiting].", "score": 0.00980392156862745, "content": "We saw a 54-year-old patient who had been treated for gastrointestinal ulcerations with bleeding complications now presenting with nausea, vomiting and diarrhoea. The patient has been suffering from recurrent diarrhea for years. The suspicion of a neuroendocrine tumor had been made but could not be confirmed intraoperatively in the past. As suspicion was still high with high levels of chromogranin A and high gastrin levels the diagnosis now could be confirmed with a somatostatin scintigraphy. Finally the confirmed spot was identified to be a metastasis of a gastrinoma of still unknown primary localisation."}, {"id": "pubmed23n0124_15010", "title": "Early and late results after antrectomy, selective vagotomy and Roux-en-Y reconstruction for severe peptic ulcer disease.", "score": 0.009708737864077669, "content": "The Roux-en-Y gastrojejunostomy has become an increasingly used method for gastrointestinal tract reconstruction, and is advocated in the treatment of alkaline reflux gastritis. Since 1979 we have used selective vagotomy, antrectomy and Roux-en-Y reconstruction as the primary method in treating gastric ulcer and complicated duodenal ulcer in 25 patients. In a follow-up study we have evaluated the postoperative course and the long term results. All patients were operated on between 1979 and 1984. Nine patients had a gastric ulcer (GU), 11 a prepyloric ulcer (PPU) and 5 patients had a complicated duodenal ulcer (DU). The clinical results showed a grade Visick I in 48%, Visick II in 44%, Visick III in 4% and a grade Visick IV in 4%. There were no postoperative deaths, but other early complications occurred in 9 patients (36%). At follow-up, endoscopy showed signs of delayed emptying in 5 patients, but only 1 had corresponding symptoms. Mild to moderate symptoms of dumping were found in 1 patient. There was no case of severe diarrhea. The overall results were satisfactory, indicating that selective vagotomy and antrectomy with Roux-en-Y reconstruction is a valuable alternative as a primary method in the surgical treatment of severe peptic ulcer disease."}, {"id": "pubmed23n0299_15408", "title": "[Endosonographic demonstration of extrapancreatic and extraintestinal gastrinoma].", "score": 0.009708737864077669, "content": "No cause had been found for chronic diarrhoea in a 57-year-old man. Up to 15 watery stools daily had been without relation to food intake and without blood admixture. But muscular cramps had developed, especially in the legs. The patient had a history of recurrent peptic ulcers for which a selective proximal vagotomy had been performed 13 years ago. Physical examination was unremarkable. Alkaline phosphatase activity (182 U/l) and C-reactive protein (9.3 mg/l) were slightly raised; serum iron was 42 micrograms/dl, while all other routine laboratory tests, including protein electrophoresis, blood picture and differential count were within normal limits. Gastroscopy revealed ulcerative duodenitis, gastritis with erosions and numerous ulcers and reflux oesophagitis, grade III-IV. Endosonography showed enlarged gastric mucosal relief as sign of foveolar hyperplasia and a ca. 4 x 3 cm tumour next to the duodenal bulb. Gastrin level was 7537 pg/ml (normal &lt; 150 pg/ml). Computed tomography and somatostatin receptor scintigraphy confirmed the site and size of the gastrinoma. Treatment with omeprazole (40 mg three times daily) slightly improved the symptoms. The tumour was excised a week after diagnosis. The patient has been symptom-free since then. Chronic diarrhoea of unknown aetiology can be caused by an endocrine tumour; endosonography can often provide information on the diagnosis and location of such a tumour."}, {"id": "wiki20220301en057_38005", "title": "Carcinoid", "score": 0.009624246721020914, "content": "Cause Carcinoid syndrome involves multiple tumors in one out of five males. The incidence of gastric carcinoids is increased in achlorhydria, Hashimoto's thyroiditis, and pernicious anemia. Treatment Surgery, if feasible, is the only curative therapy. If the tumor has metastasized (most commonly, to the liver) and is considered incurable, there are some promising treatment modalities, such as radiolabeled octreotide (e.g. Lutetium (177Lu) DOTA-octreotate) or the radiopharmaceutical 131I-mIBG (meta iodo benzyl guanidine) for arresting the growth of the tumors and prolonging survival in patients with liver metastases, though these are currently experimental. Chemotherapy is of little benefit and is generally not indicated. Octreotide or lanreotide (somatostatin analogues) may decrease the secretory activity of the carcinoid, and may also have an anti-proliferative effect. Interferon treatment is also effective, and usually combined with somatostatin analogues."}, {"id": "pubmed23n0224_836", "title": "Experience with vagotomy--antrectomy and Roux-en-Y gastrojejunostomy in surgical treatment of duodenal, gastric, and stomal ulcers.", "score": 0.009615384615384616, "content": "Gastroduodenostomy (Billroth I) is our reconstruction of choice following gastric resection for gastroduodenal ulcer. Dissatisfaction with a Billroth II anastomosis has led us in recent years to employ a Roux-en-Y diversion in selected cases, particularly those in which the pathologic state of the pyloroduodenal canal would render a Billroth I anastomosis unsafe. During the past 7 years, truncal vagotomy-antrectomy and Roux-en-Y (VARY) has been carried out in 50 selected patients: duodenal ulcer (DU) 13 patients, gastric ulcer (GU) 11 patients, and stomal ulcer (SU) 26 patients. Fourteen patients (28%) developed postoperative complications, of which nine (18%) were of major degree and five (10%) of a lesser degree. No hospital death occurred among the 50 patients. Five patients (10%) developed postoperative delayed gastric emptying and two of the five required revision of the Roux. Forty-five patients had no clinical problems with delayed emptying. Overall results showed a Visick grading of I in 72%, Visick II in 24%, and Visick III in 4%. Further analysis revealed that of the 13 patients with DU who had VARY, 62% were Visick I, 30% Visick II, and 8% Visick III. The 11 GU patients with VARY were graded Visick I 73% and Visick II 27%. Of 26 patients with SU who underwent VARY, 77% were Visick I, 19% Visick II, and 4% Visick III. Mild to moderate dumping took place in 8% of the 50 patients, mild diarrhea 10%, weight loss 10%, and no patient experienced alkaline reflux gastritis. Long-range postoperative gastric emptying studies among nine patients using a radionuclide revealed varying patterns of emptying. Overall clinical results have been satisfactory and we are continuing to use VARY in selected cases, particularly those in which a Billroth I reconstruction appears contraindicated."}, {"id": "pubmed23n0237_7285", "title": "The early diagnosis of gastrinoma.", "score": 0.009615384615384616, "content": "Despite the increasing awareness of gastrinoma and its lethal peptic ulcer sequelae, the diagnosis is often initially missed or made as a terminal event. The authors screened all patients with peptic ulcer symptoms serious enough to warrant hospital admission or those associated with diarrhea, nephrolithiasis, hypercalcemia, or pituitary abnormality. In a one-year period (1979-1980) nine (of 14 suspected) new gastrinoma patients were identified using a sensitive and specific gastrin radioimmunoassay in combination with provocative tests including IV secretin, calcium, and food. Conventional upper GI series, CAT scan, arteriography, and endoscopy provided no additional information other than to confirm the presence of ulcer disease. Basal plasma gastrin levels were more than 200 pmol L-1 in only three of the nine (normal fasting plasma gastrin levels are less than 25 pmol L-1). Three patients presented with acute ulcer perforation, and the diagnosis of gastrinoma was suspected because of multiple ulcers and pancreatic masses. In three other patients, previous duodenal ulcer surgery had failed. One patient with dyspepsia, high basal plasma gastrin, negative secretin and calcium infusion studies, and a positive meal test was diagnosed as having G-cell hyperplasia; this was confirmed by biopsy and antral gastrin extraction. Antrectomy alone resulted in cure. In all patients tested, a positive calcium infusion or secretin bolus (greater than 100% rise over basal) strongly suggested the diagnosis of gastrinoma, which was confirmed at surgery. In the acute perforations, initial management with omental patch and cimetidine therapy allowed survival of two patients, while emergency total gastrectomy in the third resulted in death due to esophagojejunal leak. Elective patients were treated with cimetidine initially for at least two weeks before total gastrectomy. In this group there were no operative mortalities, and postoperative morbidity was minimal. This series illustrates three important points: (1) careful screening of an ulcer population using gastrin radioimmunoassay and provocative tests has enabled a high yield of gastrinomas while conventional investigations are of minimal values; (2) a high index of suspicion in appropriate cases is necessary; and (3) total gastrectomy performed under elective circumstances is safe and allows the patients to resume a normal and healthy life without the sequelae of aggressive peptic ulceration or daily drug administration."}, {"id": "pubmed23n0069_6586", "title": "Surgical treatment of peptic ulcer disease.", "score": 0.009523809523809525, "content": "Elective surgery for peptic ulcer disease has diminished significantly over the past 15 years. However, emergency surgery has not shown a decline. Some series have even reported an increase in hospitalizations and operations for hemorrhage. The appropriate surgical procedure for peptic ulcer disease must be tailored to the specific needs of the individual patient. During emergency operations for hemorrhage from duodenal ulcer, we recommend suture ligature of the bleeding vessel and vagotomy-pyloroplasty for high-risk patients, or vagotomy-antrectomy for the lower-risk patient. Bleeding gastric ulcers should be resected, if possible. For massive hemorrhage from stress ulceration requiring surgery, near-total or total gastrectomy should be performed. Perforated duodenal ulcers are best managed by closure and a definitive ulcer operation, such as vagotomy-pyloroplasty. Perforated gastric ulcers are best excised but may be simply closed if conditions do not favor resection. In these situations, biopsy should be performed. We recommend truncal vagotomy-antrectomy for patients presenting with obstruction. Vagotomy (truncal or proximal gastric) with drainage is an acceptable alternative in this situation. For patients with intractable ulcer disease or for those who are noncompliant, proximal gastric vagotomy is the preferred operation. However, other operations may need to be considered, depending on the specific situation. Recurrent ulceration needs appropriate work-up to determine the possible cause. Although patients with ulcer recurrence initially may be placed on medical treatment, about 50% will require reoperation. The most effective procedure for peptic ulcer disease is truncal vagotomy-antrectomy, which has a recurrence rate of less than 1%. The procedure with the least morbidity and the fewest undesirable side effects is proximal gastric vagotomy. Ulcer recurrence after proximal gastric vagotomy or truncal vagotomy-pyloroplasty is in the range of 10% to 15%."}, {"id": "pubmed23n0095_13329", "title": "[10 years' results following proximal selective vagotomy in duodenal ulcer disease. A prospective study].", "score": 0.009523809523809525, "content": "The results 10 years after proximal gastric vagotomy for chronic duodenal ulcer disease in a prospective trial are presented. Among 76 patients 5 were lost to follow-up, 3 had died from causes unrelated of ulcer disease. 80.3% of the patients remained clinically free from recurrence. 92.2% had a Visick grade I or II. The symptomatic recurrence rate was 19.7%, total recurrence rate including asymptomatic recurrences having been 25.3%. 6 patients (8.4%) had to be reoperated, 12 (16.9%) were treated medically. 3/4 of the medically treated patients only had 1 recurrence throughout 10 years. The rate of mild dumping and diarrhea was 2% each. Patients with recurrence showed no more significant reduction of BAO or pentagastrin stimulated maximal acid output (MAO) 10 years postoperatively. Patients without recurrence had a significant reduction of BAO and MAO of 42%. Based upon the results presented, the indication for proximal gastric vagotomy for chronic duodenal ulcer is still justified."}, {"id": "pubmed23n0032_2873", "title": "Alkaline reflux gastritis.", "score": 0.009433962264150943, "content": "Any surgical procedure that ablates the pyloric sphincter mechanism permits increased reflux of duodenal fluid into the stomach or gastric remnant. Although it is reported as most common with Billroth II gastrectomy, our experience indicates that reflux is nearly as frequent after Billroth I gastroduodenostomy and is not at all infrequent after pyloroplasty. The precise constituents of duodenal fluid which damage the gastric mucosa remain controversial. The best present evidence is that the bile acids are probably essential, but that one or more other constituents of duodenal content are also necessary. The clinical history differs significantly from chronic afferent loop syndrome in that the quality of pain is different, pain tends to be more continuous and less closely related to food-taking, and bile vomiting does not provide dramatic relief, often containing food due to coexistent interference with gastric emptying. Diagnosis is confirmed by gross endoscopic findings and characteristic histopathologic changes in the endoscopic biopsies. Treatment with an interposed isoperistaltic jejunal segment has been disappointing. Only four of ten patients experienced lasting relief, indicating that the relatively short 10 to 12 cm. of jejunum does not adequately prevent duodenogastric reflux. We have, therefore, shifted to the Roux-en-Y duodenal diversion implanting the afferent limb 40 cm. caudad to the gastrojejunostomy. Results have been excellent in 24 of 25 cases with prompt improvement in gastric emptying, absence of bile vomiting, progressive regression in abdominal distress and progressive improvement in nutrition. Endoscopic evaluation at three to four months has indicated marked gross improvement and striking histologic improvement in 23 of 25 cases. The question is raised whether the Roux-en-Y reconstruction should not be used primarily, particularly if both vagotomy and antrectomy are to be performed for peptic ulcer. Both the afferent loop syndrome and alkaline reflux gastritis would be prevented, and it is doubted that the incidence of marginal ulcer would increase appreciably."}, {"id": "pubmed23n0085_12824", "title": "The use of the long-acting somatostatin analogue, octreotide acetate, in patients with islet cell tumors.", "score": 0.009433962264150943, "content": "Octreotide lowers plasma concentrations of the marker peptide in the majority of patients with islet cell tumors. However, as described above the effect of octreotide on plasma concentrations of marker peptides is not necessarily related to the effect on symptoms. Nevertheless octreotide is capable of producing symptomatic relief in a large proportion of patients with islet cell tumor syndromes. The data on the effect of octreotide on the symptoms due to VIPoma and due to the carcinoid syndrome (presumably including some who have islet cell tumors) are strong and the drug has been approved for these indications by the Food and Drug Administration. With respect to the other islet cell tumor syndromes, the published data suggest that the utility of octreotide differs in the different syndromes. Insulinomas are usually single, benign, and can and should be removed surgically, resulting in cure. Octreotide therefore has no role to play in such patients, particularly since the response of insulinomas is variable. However in the 10 per cent of insulinomas that are malignant octreotide is certainly effective in at least a portion of cases, although as yet the true response rate and efficacy compared with diazoxide is not clear. Although octreotide is effective at reducing acid output, and thus improving symptoms in patients with Zollinger-Ellison syndrome, because of the effectiveness of histamine H2-receptor antagonists and omeprazole, there is no need for octreotide in this syndrome. For patients with glucagonoma, GHRHoma, Cushing's syndrome, and other rare islet cell tumor syndromes octreotide may well be of benefit and should be considered. The current data do not support the use of octreotide for an antitumor effect."}, {"id": "pubmed23n0376_13387", "title": "[Laparoscopic truncal vagotomy, antrectomy with Billroth-II reconstruction for complicated duodenal ulcer (Case report and literature review)].", "score": 0.009345794392523364, "content": "Today surgeons are performing fewer elective ulcer surgeries, as H2 receptor blockers and the eradication of Helicobacter pylori represent a major step in treatment of this disease. Nevertheless, patients with complications and those resistant to medical therapy should be offered surgical options. The laparoscopic management of these complications is an alternative to open surgery, if applied with appropriate patient selection. The authors report a case with repeated bleeding and duodenal narrowing due to a duodenal ulcer. The patient was treated by totally intraabdominal laparoscopic truncal vagotomy, antrectomy and Billroth-II reconstruction--this probably being the first performed and publicized operation in Hungarian literature. The technique this operation and common perioperative problems based on existing literature are reviewed. The benefits of the minimally invasive approach were clearly evident. The authors believe that minimally invasive approaches will renew the interest in definitive surgery for the treatment of ulcer disease."}, {"id": "pubmed23n0208_6248", "title": "Primary peptic ulcerations of the jejunum associated with islet cell tumors. Twenty-five-year appraisal.", "score": 0.009345794392523364, "content": "A review of 42 patients with gastrinoma, who either survived five years or longer or who died during this period of evaluation, was carried out to define the surgical principles which might be combined with the recent trend toward cimetidine therapy. Thirty-four (80%) of the patients had total gastrectomy with an operative mortality rate of 2.3%, and eight patients (20%) had less than total gastrectomy. No tumor was found in six patients with hypergastrinemia and an abnormal secretin bolus whose five-year survival rate was 100%. Of the thirty-six patients having tissue proof of gastrinoma, twenty-two (61%) had complete resection of all gross tumor resulting in a 76% five-year survival rate. Fourteen patients had unresectable tumor or partial resection with a five-year survival rate of 21%. Complete gross tumor resection increased mean survival by six years (p &lt; 0.01), but resulted in persistent eugastrinemia in only two patients. Long-term survival was possible with a combination of vagotomy, lesser gastric procedures, tumor resection, and cimetidine, seven of eight patients living more than five years. Surgical management of gastrinoma should be directed toward aggressive tumor resection and vagotomy, with reliance on cimetidine therapy postoperatively to control the gastric hypersecretion. Total gastrectomy should be reserved for cimetidine failures and those who do not wish to take cimetidine for the rest of their lives."}, {"id": "pubmed23n0265_6782", "title": "A comparative study of gastrectomy without vagotomy with either Roux-en-Y or Billroth II anastomosis in peptic ulcer.", "score": 0.009259259259259259, "content": "Since recent small uncontrolled studies have suggested that surgery for peptic ulcer comprising partial gastrectomy with Roux-en-Y anastomosis without vagotomy effectively prevents postoperative enterogastric reflux without increasing ulcer recurrence rate, we have compared mortality, ulcer recurrence rate, and complaints in ulcer patients who had undergone partial gastrectomy with either Roux-en-Y (n = 47) or Billroth II anastomosis (n = 47). The groups were comparable with regard to age, sex, ulcer localisation, indication for surgery and number of emergency procedures. During postoperative follow-up, seven patients with Roux-en-Y have died, compared with nine patients with Billroth II gastrectomy. In two of the seven patients who died after Roux-en-Y gastrectomy, but in none of the nine who died after Billroth II resection, death was unequivocally related to postoperative ulcer recurrences. At 1, 2, 3 and 4 years postoperatively, 90 vs. 100% (not significant), 78 vs. 98% (p &lt; 0.01), 72 vs. 95% (p &lt; 0.01) and 72 vs. 95% (p &lt; 0.01) of the patients were in remission after Roux-en-Y and Billroth II gastrectomy, respectively. All ulcers were localized at or just distal to the anastomosis, and were diagnosed within the first 3 postoperative years. We conclude that in peptic ulcer patients the ulcer recurrence rate after Roux-en-Y gastrectomy without vagotomy is considerably higher than after Billroth II resection. Thus, gastrectomy with Roux-en-Y anastomosis without vagotomy cannot be recommended as the primary procedure in patients undergoing partial gastrectomy for peptic ulcer disease."}, {"id": "pubmed23n0273_14427", "title": "Ten-year follow-up of a prospective, randomized trial of selective proximal vagotomy with ulcer excision and partial gastrectomy with gastroduodenostomy for treating corporeal gastric ulcer.", "score": 0.009259259259259259, "content": "Between 1975 and 1980, 30 patients with type I corporeal gastric ulcer were randomly allocated to undergo selective proximal vagotomy with ulcer excision or partial gastrectomy with gastroduodenostomy. Sixteen patients underwent selective proximal vagotomy (1 was excluded from the follow-up since microscopic examination of the excised ulcer revealed an early gastric cancer) and 14 underwent partial gastrectomy. No significant differences in the clinical results were found 3 years after surgery. During a median follow-up of 10 years, ulcer recurred in 3 patients after selective proximal vagotomy and in 2 after partial gastrectomy. One patient in each group had recurrent ulcer without symptoms and received no treatment. Two selective proximal vagotomy patients and three partial gastrectomy patients had epigastric pain with or without ulcer. One patient with selective proximal vagotomy underwent a second operation because of epigastric pain and recurrent ulcer. Bowel habits remained unchanged in all but one patient in each group, and mild or moderate dumping was recorded for two patients in each group. Very good or good results (modified Visick scale) were recorded for 11 of 15 patients after selective proximal vagotomy and for 10 of 14 patients after partial gastrectomy. Except for one patient in each group who had moderate dumping, patients classified as Visick III or IV had no symptoms during treatment with antacids or H2-blockers, or had asymptomatic ulcers and needed no treatment. Selective proximal vagotomy reduced the median acid response to insulin hypoglycemia and to pentagastrin by 100% and 80%, respectively, for at least 3 to 5 years, and partial gastrectomy reduced the median acid response to pentagastrin by 97%. In our opinion, selective proximal vagotomy with ulcer excision is an alternative to partial gastrectomy for surgically treating type I gastric ulcer."}, {"id": "wiki20220301en163_1363", "title": "Hormonal therapy (oncology)", "score": 0.009253003003003003, "content": "Somatostatin analogs Octreotide is an analog of the peptide hormone somatostatin, which inhibits the production of the growth hormone as well as numerous peptide hormones of the gastrointestinal system, including insulin, glucagon, pancreatic polypeptide, gastric inhibitory polypeptide, and gastrin. Octreotide is used for suppression of the hormonal syndromes which accompany several pancreatic islet cell tumors, including the Zollinger-Ellison syndrome of gastrinoma and the chronic hypoglycemia of insulinoma. It is also effective in suppression of the carcinoid syndrome, caused by advanced or extra-gastrointestinal carcinoid tumors. Octreotide may also be used for treatment of severe diarrhea caused by 5-fluorouracil chemotherapy or radiation therapy."}, {"id": "pubmed23n0372_8204", "title": "Thoracoscopic truncal vagotomy.", "score": 0.009174311926605505, "content": "Nowadays the only indications to truncal vagotomy is recurrent ulceration after previous gastric surgery. Truncal vagotomy allows us to obtain a reduction in acid production and to promote ulcer healing, but this technique causes pylorospasm in about 20% of cases and this requires further synchronous or metachronous pyloric drainage procedure. For this reason, videothoracoscopic truncal vagotomy is reserved to patients with gastroresection. The authors describe 15 patients treated with videothoracoscopic truncal vagotomy. In 12 patients, a gastrojejunostomy was done according to Roux technique in 2 patients, a reconstruction according Billroth II technique and in 1 patient, a gastroduodenostomy according to Billroth I technique. Videothoracoscopic bilateral truncal vagotomy was done in all patients; operation time was 45 minutes. During the postoperative period there were no complications. No patients underwent medical therapy for peptic ulcer. Only in 12 patients was it possible to execute an endoscopic follow-up in a period of 3 to 4 years. In all patients the ulcer was completely healed. Complete vagotomy in patients who present with recurrent gastrointestinal bleeding after previous gastroresection, is associated with significant risks. Videothoracoscopic bilateral truncal vagotomy as a simple and efficient procedure seems to be an alternative treatment for the management of recurrent ulceration after previous gastric surgery for peptic disease."}, {"id": "wiki20220301en041_48293", "title": "Octreotide", "score": 0.009146015332501338, "content": "Octreotide, sold under the brand name Sandostatin (marketed by Novartis) among others, is an octapeptide that mimics natural somatostatin pharmacologically, though it is a more potent inhibitor of growth hormone, glucagon, and insulin than the natural hormone. It was first synthesized in 1979 by the chemist Wilfried Bauer, and binds predominantly to the somatostatin receptors SSTR2 and SSTR5. It was approved for use in the United States in 1988. Medical uses Tumors Octreotide is used for the treatment of growth hormone producing tumors (acromegaly and gigantism), when surgery is contraindicated, pituitary tumors that secrete thyroid-stimulating hormone (thyrotropinoma), diarrhea and flushing episodes associated with carcinoid syndrome, and diarrhea in people with vasoactive intestinal peptide-secreting tumors (VIPomas). Octreotide is also used in mild cases of glucagonoma when surgery is not an option."}, {"id": "pubmed23n0621_21768", "title": "Latest results (12-21 years) of a prospective randomized study comparing Billroth II and Roux-en-Y anastomosis after a partial gastrectomy plus vagotomy in patients with duodenal ulcers.", "score": 0.00909090909090909, "content": "After a partial resection of the stomach, the continuity of the gastrointestinal tract can be restored either by a Billroth II gastrojejunal anastomosis or a Roux-en-Y gastrojejunostomy. Each procedure has its advantages and disadvantages. To determine through a prospective and random clinical trial, the clinical outcome and the endoscopic and histologic alterations of the distal esophagus and the gastric remnant in patients who received a partial distal gastrectomy due to duodenal ulcers and a Billroth II or Roux-en-Y reconstruction. In this prospective random trial, a total of 75 patients with duodenal ulcers were included. A bilateral selective vagotomy and partial distal gastrectomy were performed in all patients. A Billroth II or Roux-en-Y 60-cm-long loop was randomly used for reconstruction of the gastrointestinal tract. During the latest follow-up clinical evaluation, upper endoscopy and biopsy samples from the distal esophagus and gastric remnant were obtained. There was 1 operative mortality and 6 patients had some morbidity. The average follow-up period was 15.5 years (range, 11-21). Patients with Roux-en-Y gastrojejunostomy were significantly more asymptomatic and had greater Visick I grading than patients with Billroth II reconstruction (P &lt; 0.001). In the distal esophagus, endoscopic findings were normal in 90% of the Roux-en-Y group, but only in 51% of the Billroth II group (P &lt; 0.0009). Nearly 25% of the latter group had the appearance of a short-segment Barrett esophagus compared with 3% of the Roux-en-Y group (P &lt; 0.0001). The gastric remnant endoscopic findings were normal in 100% of the Roux-en-Y group and in 18% of the Billroth II group (P &lt; 0.02). Histologic analyses showed similar proportions of normal fundic mucosa and chronic active fundic gastritis. However, chronic atrophic fundic gastritis and intestinal metaplasia were significantly more frequent after Billroth II reconstruction (P &lt; 0.008). Helicobacter pylorus was present in a similar proportion of patients. This prospective and random study showed that Roux-en-Y gastrojejunostomy is significantly better than a Billroth II reconstruction in patients with duodenal ulcers, through subjective and objective endoscopic and histologic evaluations during the latest follow-up evaluation."}, {"id": "pubmed23n0235_13349", "title": "[Changes in the diagnosis and treatment of Zollinger-Ellison syndrome (author's transl)].", "score": 0.00909090909090909, "content": "A gastrinoma was found in 12 of 23 patients with Zollinger-Ellison syndrome. Those with gastrinoma were not different from those without as regarded fasting gastrin level, increased gastrin secretion after secretin and calcium, acid secretion, or survival time. Five of the 23 patients have died, four immediately postoperatively, the fifth of the metastasizing tumour. Treatment with the H2-receptor antagonist cimetidine avoided emergency operation and thus decreased operative mortality, giving time for localization by ultrasound, computed tomography and selective arteriography. The rate of false-negative results was high. Transhepatic selective catheterization of the pancreatic veins with gastrin determination gives localization of the tumour and identification of the secreted hormone. In three patients tumour resection was possible under cover of H2-receptor antagonist administration which normalized gastrin and acid secretion. There is a change in the treatment of the Zollinger-Ellison syndrome, away from total gastrectomy to conservative treatment with H2-receptor antagonists and an attempt of curative treatment by removal of the tumour."}, {"id": "pubmed23n0095_14298", "title": "[Somatostatin in gastroenterological therapy].", "score": 0.009009009009009009, "content": "Somatostatin (SST) has been shown by several controlled studies to be effective in halting acute severe bleeding from ulcerative and erosive lesions of the upper intestinal tract. Its efficacy for the treatment of bleeding esophageal varices is less certain, and more controlled studies are necessary. Intravenous administration of SST or subcutaneous application of the new synthetic SST-analogues produces a decrease in serum hormone levels and abolition of symptoms in patients with endocrine-active tumors such as vipoma, glucagonoma and carcinoid. SST has no effect on the outcome of acute pancreatitis, and experience with SST in treating intestinal fistulas is very limited."}, {"id": "wiki20220301en027_75866", "title": "Gastrectomy", "score": 0.008928571428571428, "content": "All patients lose weight after gastrectomy, although the extent of weight loss is dependent on the extent of surgery (total gastrectomy vs partial gastrectomy) and the pre-operative BMI. Maximum weight loss occurs by 12 months and many patients regain weight afterwards. History The first successful gastrectomy was performed by Theodor Billroth in 1881 for cancer of the stomach. Historically, gastrectomies were used to treat peptic ulcers. These are now usually treated with antibiotics, as it was recognized that they are usually due to Helicobacter pylori infection or chemical imbalances in the gastric juices. In the past a gastrectomy for peptic ulcer disease was often accompanied by a vagotomy, to reduce acid production. This problem is now managed with proton pump inhibitors. See also Finsterer-Hofmeister operation List of surgeries by type Roux-en-Y Sleeve gastrectomy References Digestive system surgery"}, {"id": "pubmed23n0521_22798", "title": "VIPomas: an update in diagnosis and management in a series of 11 patients.", "score": 0.008928571428571428, "content": "VIPoma is a rare pancreatic endocrine tumor (PET) which secretes excessive amounts of VIP (Vasoactive Intestinal Peptide) that causes a special clinical syndrome characterized by secretory diarrhea, hypokalemia and achlorhydria. Among a total number of 76 patients (pts) with PETs, we present in this study 11 pts with VIPoma syndrome focusing on our diagnostic and therapeutic approach, in parallel with a brief review of the literature. Eleven pts (7 males and 4 females), aged from 2 to 83 years (mean age 53.1 years) were included. The diagnosis was based upon compatible clinical features and serum VIP values and was supported by the estimation of other peptides and neuroendocrine markers such as gastrin, pancreatic polypeptide and chromogranin-A (CgA). In 10/11 pts, diagnosis was confirmed histopathologically. The primary or metastatic lesions were located by conventional imaging methods or by OCTREOSCAN or Endoscopic Ultrasound (EUS). The follow-up period ranged from 2.5-13.5 years (mean 4.8 years). Chronic secretory diarrhea, which persisted despite fasting, was the main symptom in all pts of our study. VIP levels at the time of diagnosis were more than 3 or 10 times the upper normal limit in 7/11 (63.6%) or 4/11 (36.4%) pts, respectively. The primary lesion was detected by CT scan or MRI in 6/11 (54.5%), with EUS or abdominal angiography in 4/11 (36.4%). OCTREOSCAN revealed a solitary lesion in the right hepatic lobe, not detected by all the previous studies, while it detected, as a whole, the primary lesion in 10/11 (91%), and the metastases in 3/4 (75%) pts. In 7/11 (63.6%) the primary lesion was located in the pancreas, whereas in the rest it was in the duodenum or retroperitoneum. A surgical resection was possible in 7/11 (63.6%) pts, while pts with metastatic disease already or poorly differentiated tumors also received additional treatment with somatostatin analogues and chemotherapy. Liver metastases and poor differentiation of tumors seemed to be negative prognostic factors. Clinical suspicion, early diagnosis and precise management may affect survival and improve the quality of life of patients. Also, surgical treatment, as extensive as possible, in combination with somatostatin analogues or chemotherapy when necessary, may also result in prolonged survival, also in patients with advanced disease."}, {"id": "pubmed23n0032_2872", "title": "The afferent loop syndrome.", "score": 0.008849557522123894, "content": "The afferent loop syndromes result from obstruction to the afferent jejunal loop. Acute ALS results from complete obstruction, usually occurs early after surgery and runs a devastatingly lethal course unless promptly treated by reoperation. In chronic ALS the obstruction is intermittent and produces a clinical syndrome from which a diagnostic histroy can usually be obtained. Although the exact incidence is unknown, it is certainly not rare, especially in antecolic Billroth II gastrectomies. Treatment consists of doing away with the afferent loop. In gastroenterostomy alone takedown of the anastomosis with a Weinberg pyloroplasty is the treatment of choice. The safest and simplest treatment for patients whose original operation was Billroth II gastrectomy is conversion to a Roux-en-Y procedure. In all cases vagotomy should be added unless previously performed. No medical treatment is available and patients with no other contraindication should have revisional surgery if symptoms are clinically significant. Both acute and chronic afferent loop syndromes should be completely prevented by appropriate choice of the initial operative procedure. The vagotomized stomach should be drained by pyloroplasty, not gastrojejunostomy. Vagotomy and antrectomy should be reconstructed with a Billroth I gastroduodenostomy. The Braun enteroanastomosis should be utilized after subtotal gastrectomy for carcinoma. The wider application of parietal cell vagotomy for duodenal ulcer deserves close observation and further consideration."}, {"id": "Surgery_Schwartz_7510", "title": "Surgery_Schwartz", "score": 0.008849557522123894, "content": "should prompt an evalu-ation for gastrinoma. Gastrinoma also should be considered in the differential diagnosis of recurrent or refractory peptic ulcer, secretory diarrhea, gastric rugal hypertrophy, esophagi-tis with stricture, bleeding or perforated ulcer, familial ulcer, peptic ulcer with hypercalcemia, and gastric neuroendocrine tumor (carcinoid). The majority of patients with ZES have been symptomatic for several years before definitive diagnosis and, 3Table 26-13Differential diagnosis of intractability or nonhealing peptic ulcer diseaseCancer Gastric Pancreatic DuodenalPersistent Helicobacter pylori infection Tests may be false-negative Consider empiric treatmentNoncompliant patient Failure to take prescribed medication Surreptitious use of NSAIDsMotility disorderZollinger-Ellison syndromeBrunicardi_Ch26_p1099-p1166.indd 113601/03/19 7:12 PM 1137STOMACHCHAPTER 26in general, patients with ZES and MEN1 are diagnosed in their 20s and 30s, while those with sporadic ZES more"}, {"id": "pubmed23n0074_11106", "title": "Stasis syndromes following gastric surgery: clinical and motility features of 60 symptomatic patients.", "score": 0.008771929824561403, "content": "We retrospectively reviewed the records of 60 patients who had been referred for gastrointestinal manometry because of stasis after gastric surgery. Nausea, vomiting, bloating, abdominal pain, and weight loss were the most common symptoms. Two thirds of these patients had a well-documented history of peptic ulcer before their initial operations; in others, surgery was performed for other reasons, such as obesity (5%) or reflux esophagitis (8%). Twelve patients had undergone truncal vagotomy and a \"drainage operation\" and 48 had received a partial gastrectomy with a gastroenterostomy: Billroth I (n = 8), Billroth II (n = 11), Roux-en-Y (n = 29). All patients had recordings of gastrointestinal manometry; 16 also had a scintigraphic measurement of gastric emptying. Measurements were compared with data from healthy controls. Gastric manometry, which could be assessed only in the group with an intact antrum, was characterized by antral hypomotility (p less than 0.05). Gastric emptying studies showed rapid early emptying of liquids and delayed emptying of solids (both p less than 0.05). In the whole group, fasting jejunal motility was characterized by absence of phase II in 13, presence of bursts of phasic activity in 18, and abnormal propagation of phase III in 8. A significantly increased frequency of phase III of MMC was noted in the patients after Billroth II and Roux-en-Y operations. Postprandially, 19 patients failed to develop a \"fed pattern.\"(ABSTRACT TRUNCATED AT 250 WORDS)"}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Be careful with lithium. Intoxications have been seen with normal lithium. It is not rare -especially due to interactions-. A very effective drug if everyone is forewarned.", "full_answer_no_ref": "Be careful with lithium. Intoxications have been seen with normal lithium. It is not rare -especially due to interactions-. A very effective drug if everyone is forewarned.", "full_question": "A 46-year-old man with bipolar disorder is brought to the emergency department after an over-ingestion of lithium carbonate. Examination reveals severe tremor, ataxia, dysarthria, myoclonus and fasciculations. Lithemia is 4.1 mEq/L (toxicity > 1.6 mEq/L). Which of the following therapeutic options would be most indicated?", "id": 507, "lang": "en", "options": {"1": "Aminophylline associated with a cathartic.", "2": "Activated charcoal.", "3": "Hemodialysis.", "4": "Forced diuresis.", "5": NaN}, "question_id_specific": 89, "type": "PSYCHIATRY", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0522_8152", "title": "[Acute intoxication with sustained-release lithium carbonate tablets. A propos of a case].", "score": 0.019232547387887194, "content": "To describe the case of a patient who ingested 50 sustained release lithium carbonate 400 mg tablets, and reached a late peak concentration above 3 mEq/L. A 32-year-old male with bipolar mood disorder ingested 50 sustained-release lithium carbonate tablets. Upon admission to the emergency room, a gastric wash was performed,from which several tablet remnants were obtained, as well as an intestinal lavage using activated carbon. good general status, no fever, blood pressure 160/90 mm Hg, no edemas. Neurologic, pulmonary, and cardiac examinations were normal. CBC and the chemistry panel were normal. The patient's psychopathological examination suggested a stable status with no apparent manifestations arising from a decompensated mood disorder. Five hours after his massive lithium ingestion the drug's plasma levels were 0.75 mEq/L. At 22 hours post-ingestion a chemistry panel was obtained, which showed serum creatinin at 1.38 mg/dL and a lithium plasma concentration of 3.15 mEq/L. A hemodyalisis trial was attempted for 4 hours. At 73 hours post-ingestion, lithium plasma levels were 0.6 mEq/L, that is, within therapeutic range. The patient was hemodynamically stable and serial blood tests were normal; he was discharged. Acute lithium intoxication with plasma levels above 3 mEq/l can be fatal or result in irreversible neurologicsequelae in almost one third of cases, with persistent cerebellar dysfunction in association with dementia of variable degree, andrenal, blood, and liver disturbances. Sustained-release tablets may prolong absorption and delay peak plasma concentrations. In such cases, therefore, it is recommended that drug plasma concentrations be monitored during 48-72 hours post-ingestion."}, {"id": "pubmed23n0968_28", "title": "Lithium intoxication presenting as altered consciousness and arrhythmia with cardiogenic shock: A case report.", "score": 0.018350930115636, "content": "Lithium has been used to treat bipolar disorder. Lithium has a narrow therapeutic index, with a therapeutic level between 0.6 and 1.5 mEq/L. The possible complications of lithium overdose include altered mental status, hand tremor, muscle weakness, nausea, vomiting, diarrhea, seizure, syncope, and arrhythmia. Lithium intoxication can be fatal and is difficult to diagnose in patients without a history of lithium intake. The occurrence of serious cardiac arrhythmias is rare in lithium intoxication. An 81-year-old man was brought to the emergency department because of consciousness disturbance for 2 days. According to his daughter, he had a history of hypertension and diabetes. Recently, his family also observed slurring of speech and easy choking. The physical examination findings were unremarkable. Blood examination only revealed impaired renal function. Twelve-lead electrocardiography revealed sinus rhythm with first-degree atrioventricular block. Chest radiography revealed mediastinal widening. The blood pressures obtained from the 4 limbs showed no significant differences. Subsequently, brain computed tomography revealed no obvious intracranial lesion. A neurologist was consulted, and a recent ischemic stroke could not be ruled out. While in the observation area, his systolic blood pressure decreased to &lt;90 mm Hg and he showed bradycardia, and 12-lead electrocardiography revealed an AV block and long pulse. Contrast-enhanced chest computed tomography revealed no evidence of aortic dissection. Another family member reported a history of lithium intake for bipolar disorder for &gt;30 years. Blood examination revealed a lithium concentration of 2.65 mEq/L. A nephrologist was consulted, and emergency hemodialysis was indicated. Dopamine was administered for his shock status via a right neck central venous catheter. His lithium level gradually declined after the hemodialysis, and blood pressure and consciousness level improved subsequently. The patient was discharged 9 days later in a stable condition. If an emergency physician encounters a patient with altered consciousness and arrhythmia with cardiogenic shock, the patient's drug intake history should be carefully reviewed to rule out cardiovascular problems on the basis of the patient's clinical condition."}, {"id": "pubmed23n0660_22478", "title": "Prolonged neurological burden in severe lithium intoxication.", "score": 0.01778827233372688, "content": "A 53-year-old woman was brought to the Emergency Department for a persistent state of stupor, tremors, fever and oliguria. The patient had been under treatment for depression. The electrocardiogram showed a wider QRS complex; laboratory tests were as follows: urea 110 mg/dL, creatinine 3 mg/dL, sodium 135 mEq/L, potassium 4.5 mEq/L, and lithium 8.0 mEq/L. Renal replacement was initiated to normalize plasma lithium levels; both stupor and speech impairment persisted for several days after dialysis. Complete recovery was achieved several days afterwards."}, {"id": "pubmed23n0691_20683", "title": "[Difficulty in determining when to end continuous hemodialysis for lithium intoxication: case report].", "score": 0.016677089847821555, "content": "The patient was a 26-year-old woman who ingested a total of 230 tablets, including 160 lithium carbonate tablets (200 mg), in a suicide attempt, and was brought to our hospital 5 hr later. After arriving at the hospital, her only complaint was mild nausea, and no neurological abnormalities or renal dysfunction was observed. We were unable to learn the blood concentration of lithium immediately. A forced diuresis was performed after admission, but the nausea persisted. Continuous hemodialysis (CHD) was performed for 23.5 hr starting 19 hr after ingesting the tablets because the patient had ingested a large amount of lithium carbonate, 32,000 mg. Since the nausea resolved after the CHD was started and no manifestations of lithium intoxication had developed as of 91 hr after ingestion, the patient was discharged. The blood lithium concentrations (mEq/L) revealed at a later date showed that the concentration 5 hr (at the time of the initial examination), 19 hr (start of CHD), 44.5 hr (end of CHD), and 91 hr after ingestion (at the time of discharge) was 4.08, 3.30, 1.09, and 0.38, respectively. Blood purification is said to be effective in treating serious lithium intoxication, but it is difficult to judge when to stop. A favorable outcome of treatment of acute lithium intoxication in a patient with normal renal function appears to have been achieved by performing CHD guided by the clinical manifestations, intravascular redistribution times, etc."}, {"id": "pubmed23n0743_22851", "title": "Lithium toxicity precipitated by thyrotoxicosis due to silent thyroiditis: cardiac arrest, quadriplegia, and coma.", "score": 0.016196721311475412, "content": "Lithium is widely used to treat bipolar disorders. Lithium toxicity is generally caused by inappropriately high doses of lithium or impaired lithium excretion. Most lithium is eliminated via the kidneys and, since thyroid hormone increases tubular reabsorption of lithium, thyrotoxicosis could contribute to the development of lithium toxicity. We report a case of severe lithium toxicity that was apparently precipitated by the onset of thyrotoxicosis resulting from silent thyroiditis and dehydration. The patient was a 64-year-old woman who was admitted for muscle weakness in the lower extremities, diarrhea, and palpitations. She had bipolar disorder and was being treated with lithium carbonate, which she discontinued one week before admission. Her circulating lithium levels had been monitored yearly. Early in her admission she was dehydrated and had febrile episodes, paroxysmal atrial fibrillation, and muscle weakness. Initially, fluid therapy was started, but she lost consciousness and had a cardiac arrest for 2 minutes due to prolonged sinus arrest. Chest compression and manual artificial ventilation were performed, and body surface pacing was started. Serum lithium was markedly elevated to 3.81 mEq/L (therapeutic range, 0.4-1.0 mEq/L), and thyroid hormone levels were increased (free triiodothyronine, 8.12 pg/mL; free thyroxine, 4.45 ng/dL), while thyrotropin (TSH) was suppressed (&lt;0.01 μIU/mL). Hemodialysis was performed, and a temporary pacemaker was inserted for severe sinus bradycardia. The serum thyroglobulin was 4680 ng/mL (reference range, &lt;32.7 ng/mL). A TSH receptor antibody test was negative. Glucocorticoid therapy and inorganic iodine (100 mg) were administered and continued until day 11. However, her neurological symptoms deteriorated with floppy quadriplegia and deep coma. She gradually recovered. On day 36, she was discharged without any neurological symptoms or thyrotoxicosis. A 64-year-old woman taking lithium for bipolar disorder developed lithium toxicity in the setting of what seemed likely to be a recent onset of thyrotoxicosis due to silent thyroiditis. Thyrotoxicosis may be a contributing cause of lithium toxicity, particularly if it is abrupt in onset and even with cessation of lithium therapy if renal function is compromised. Thyroid function should be assessed immediately in patients with suspected lithium toxicity."}, {"id": "pubmed23n0479_3914", "title": "[Reversible choreoathetosis associated with lithium intoxication].", "score": 0.01606699751861042, "content": "Several reports have been published in the literature of choreoathetosis associated with lithium intoxication, but little is known about choreoathetosis without concurrent antipsychotic treatment. We report a 65-year-old woman with lithium intoxication whose choreoathetosis completely recovered without sequela following decrease of her serum lithium level. She had been treated elsewhere for bipolar II disorder and also for hypertension, chronic hepatitis type C and diabetes mellitus. As she became hypomanic, lithium carbonate at 600 mg/day was commenced, which was increased to 1200 mg/day due to unfavorable therapeutic response. She began to manifest disorientation and abnormal involuntary movement and was therefore referred to our Department of Psychiatry. Her clinical symptoms at admission included consciousness disturbance with marked bilateral symmetrical slow-wave activity in her EEG and choreoathetosis was observed in her face and upper and lower extremities. Cerebellar symptoms were minimal with only mild ataxic gait and finger-to-nose test did not show dysmetria or intention tremor. Her serum lithium level was 3.52 mEq/L, which was clearly in the toxic range. She demonstrated no metabolic abnormalities including hyperglycemia, and was diagnosed with lithium intoxication and treated with water loading and mannitol for forced diuresis. On the 14th day after admission her consciousness disturbance and choreoathetosis resolved, but EEG abnormalities still persisted. On the 23rd day after admission, she was discharged with clinical remission and normal EEG background activity. Although she developed mild renal dysfunction, hemodialysis was not indicated. Hypersensitivity of dopamine receptor in the nigrostriatal pathways may contribute to choreoathetosis in association with the patient's vulnerability. Choreoathetosis can be a sign of lithium intoxication and prompt treatment is required following careful differential diagnosis."}, {"id": "pubmed23n1080_6407", "title": "[Lithium sulfate poisoning treated with hemodialysis in a patient with normal renal function: a case report].", "score": 0.015763076188201577, "content": "Lithium is the milestone of psychiatric patients' therapy, in particular in bipolar disorder. Despite its high therapeutic efficacy, there are several side effects (renal, thyroid, parathyroid, dermatological) and management problems linked to its narrow therapeutic range, which exposes patients to a high risk of toxicity. We describe the case of a male patient with bipolar disorder in therapy with lithium sulfate who developed a severe acute-on-chronic intoxication. He came to our attention in a somnolent state with lithemia &gt;3 mEq/L and therefore underwent hemodialysis. In view of the high toxicity of lithium, a timely and correct therapeutic choice is important to improve the patient's outcome. In this context, considering lithemia, but also kidney function and the patient's clinical status, it is necessary to consider extracorporeal treatments, of which hemodialysis is the most preferable."}, {"id": "wiki20220301en487_12076", "title": "Lithium toxicity", "score": 0.013792040682667489, "content": "Gastric lavage and whole bowel irrigation may be useful if done early. Activated charcoal is not effective. For severe toxicity hemodialysis is recommended. The risk of death is generally low. Acute toxicity generally has better outcomes than chronic toxicity. In the United States about 5,000 cases are reported to poison control centers a year. Lithium toxicity was first described in 1898. Signs and symptoms Symptoms of lithium toxicity can be mild, moderate, or severe. Mild symptoms include nausea, feeling tired, and tremor occur at a level of 1.5 to 2.5 mEq/L. Moderate symptoms include confusion, an increased heart rate, and low muscle tone occur at a level of 2.5 to 3.5 mEq/L. Severe symptoms include coma, seizures, low blood pressure and increased body temperature which occur at a lithium concentration greater than 3.5 mEq/L. When lithium overdoses produce neurological deficits or cardiac toxicity, the symptoms are considered serious and can be fatal."}, {"id": "wiki20220301en096_2387", "title": "Treatment of bipolar disorder", "score": 0.01339437738847478, "content": "Potential side effects from lithium include gastrointestinal upset, tremor, sedation, excessive thirst, frequent urination, cognitive problems, impaired motor coordination, hair loss, and acne. Excessive levels of lithium can be harmful to the kidneys, and increase the risk of side effects in general. As a result, kidney function and blood levels of lithium are monitored in patients being treated with lithium. Therapeutic plasma levels of lithium range from 0.5 to 1.5 mEq/L, with levels of 0.8 or higher being desirable in acute mania. Lithium levels should be above 0.6 mEq/L to reduce both manic and depressive episodes in patients. A recent review concludes that the standard lithium serum level should be 0.60-0.80 mmol/L with optional reduction to 0.40-0.60 mmol/L in case of good response but poor tolerance or an increase to 0.80-1.00 mmol/L in case of insufficient response and good tolerance."}, {"id": "pubmed23n0316_15490", "title": "Antacid-induced hypermagnesemia in a patient with normal renal function and bowel obstruction.", "score": 0.01329004329004329, "content": "To report a case of severe hypermagnesemia caused by magnesium hydroxide in a woman with normal renal function. A 42-year-old Hispanic woman with schizophrenia and bipolar affective disorder was transported from jail to the emergency department with confusion, abdominal pain, vomiting, and constipation. She had been treated in jail with magnesium hydroxide, ordered as milk of magnesia 30 mL po each night and Maalox 30 mL po three times daily. Additional medications included lithium carbonate 300 mg po three times daily, chlorpromazine 150 mg po three times daily, benztropine mesylate 1 mg po twice daily, and docusate sodium 100 mg po each morning. Her temperature was 35.1 degrees C, blood pressure 108/58 mm Hg, heart rate 112 beats/min, and respiratory rate 24 breaths/min. She would respond only briefly to voice or painful stimuli. Her abdomen was distended and diffusely tender. Laboratory tests included serum magnesium concentration 9.1 mEq/L (normal 1.3-2), blood urea nitrogen 16 mg/dL (8-22), creatinine 0.9 mg/dL (0.5-1.1), calcium 3.9 mEq/L (4.2-5.2), and lithium 1.0 mEq/L. A laparotomy was performed, and an adhesive band from a previous oophorectomy was found to be compressing the sigmoid colon. Hypermagnesemia, hypothermia, and hypotension continued in the intensive care unit. Despite successful treatment of the hypermagnesemia with calcium, intravenous fluids, and furosemide, the patient's cardiac rhythm degenerated into fatal, pulseless electrical activity on postoperative day 2. This case of severe hypermagnesemia from magnesium hydroxide ingestion illustrates many of the risk factors for hypermagnesemia in patients with normal renal function. People using magnesium-containing medications for relief of gastrointestinal distress may be at increased risk for hypermagnesemia. A brief review of magnesium physiology, clinical effects, and treatment is provided. Frequent use of the laboratory to identify hypermagnesemia is encouraged because it is often a clinically unexpected finding and responds well to early treatment."}, {"id": "pubmed23n1156_1115", "title": "[Diagnostic trap: Lithium neurotoxicity with normal lithemia].", "score": 0.013289183222958058, "content": "We describe here the case of a 54-year-old bipolar woman, followed in psychiatry and treated with lithium and a selective serotonin reuptake inhibitor (escitalopram) and lamotrigine, presenting a lithium poisoning with an altered state of consciousness caused by a supposed mismanagement of her treatment. Lithium poisoning was suggested based on neurological clinical features, but the blood test brought out a lithium concentration within the therapeutic values at 1,2 mmol/L (N: 0,6-1,2 mmol/L). The classic biological complications related to lithium poisoning (hypercalcemia, diabetes insipidus) confirmed the diagnosis. The patient has been transferred to our nephrology department where she got two hemodialysis sessions conducting to clinical and biological improvement, confirming the diagnosis of lithium poisoning despite the normal blood levels. Later, she was transferred to the psychiatry department for follow-up and for treatment adjustment."}, {"id": "InternalMed_Harrison_3617", "title": "InternalMed_Harrison", "score": 0.013096965927154605, "content": "A 63-year-old man was admitted to the intensive care unit (ICU) with a severe aspiration pneumonia. Past medical history included schizophrenia, for which he required institutional care; treatment had included neuroleptics and intermittent lithium, the latter restarted 6 months before admission. The patient was treated with antibiotics and intubated for several days, with the development of polyuria (3–5 L/d), hypernatremia, and acute renal insufficiency; the peak plasma Na+ concentration was 156 meq/L, and peak creatinine was 2.6 mg/dL. Urine osmolality was measured once and reported as 157 mOsm/kg, with a coincident plasma osmolality of 318 mOsm/kg. Lithium was stopped on admission to the ICU. On physical examination, the patient was alert, extubated, and thirsty. Weight was 97.5 kg. Urine output for the previous 24 h had been 3.4 L, with an IV intake of 2 L/d of D5W."}, {"id": "pubmed23n0590_20860", "title": "Lithium intoxication-induced acute parkinsonism complicated with hyperparathyroidism and nephrogenic diabetes insipidus: report of a case.", "score": 0.012099083619702175, "content": "To describe a patient with lithium intoxication presenting as acute parkinsonism, adverse metabolic effects and nephrogenic diabetes insipidus (DI). We report a case of a 67-year-old woman with a bipolar affective disorder who was treated with lithium for 10 years. Under concomitant renal insufficiency and urinary tract infarction, she experienced progressive hand tremor, bradykinesia, and unsteady gait. Laboratory results revealed hypercalcemia and hypermagnesiemia. A high serum lithium level (3.6 mEq/L) was found; thus lithium was discontinued. She was found to have a high serum level of intact parathyroid hormone: 135.0 pg/ml and a suspicious parathyroid adenoma. Polyuria with hypernatremia was also noted. A water deprivation test confirmed nephrogenic diabetes insipitces. After correction of electrolyte imbalance and reduction of lithium level, her consciousness recovered. Her parkinsonian features were responsive to levodopa 400 mg/day in 2 divided doses. One month later, apart from the residual extrapyramidal symptoms and mania, her condition was otherwise stationary. Tremor is the most frequent movement disorder associated with lithium therapy, while severe parkinsonism has been rarely reported. It should be kept in mind in differential diagnosis of acute parkinsonism especially in elder patients who receive a chronic lithium carbonate therapy."}, {"id": "pubmed23n0917_11038", "title": "[Reversible alterations in the dentate nuclei and rapid-onset cerebral atrophy due to neurotoxicity caused by lithium].", "score": 0.011761914446478205, "content": "Treatment with lithium can cause several neurological side effects, even at therapeutic levels. We report the case of a 49-year-old woman, with bipolar disorder and depression, undergoing treatment with lithium, antidepressants and antipsychotics, who was admitted to hospital due to a clinical picture of visual hallucinations with an elevated lithaemia of 2.1 mEq/L (therapeutic range: 0.6-1.2 mEq/L). The patient developed a severe encephalopathy that required the use of assisted ventilation in the intensive care unit. Initial magnetic resonance imaging showed a reversible bilateral symmetrical hyperintensity in the dentate nuclei in T2 and T2-FLAIR sequences. Over the following months she gradually developed a pancerebellar syndrome with evidence of a marked loss of bilateral volume in the cerebellum, above all at the expense of the vermis, which was accompanied by a permanent and disabling cerebellar syndrome. Although treatment with lithium can cause a variety of neurological side effects, they are usually reversible. However, they occasionally give rise to permanent and disabling sequelae, as in the case of the patient reported here, with a marked and progressive cerebellar atrophy, accompanied by permanent sequelae in the form of a disabling cerebellar syndrome. The cerebellar neurotoxicity of lithium must be taken into account in the broad differential diagnosis of cerebellar ataxia in adults."}, {"id": "pubmed23n0915_18044", "title": "Use of the anion gap and intermittent hemodialysis following continuous hemodiafiltration in extremely high dose acute-on-chronic lithium poisoning: A case report.", "score": 0.011430064334933543, "content": "A 35-year-old woman intentionally took 40,000 mg of lithium carbonate, and she was transferred to our hospital with nausea, vomiting, and diarrhea. She was diagnosed as having bipolar disorder 10 years ago and was receiving oral lithium therapy. Blood test results on arrival were remarkable for a negative anion gap of -2.1 and later, the serum lithium level turned out to be as high as 15.4 mEq/L. Intubation was required because of disrupted consciousness, and continuous hemodiafiltration (CHDF) was immediately started in the intensive care unit to obtain constant removal of lithium. After adding intermittent hemodialysis (IHD) twice during the daytime to accelerate the lithium clearance, CHDF became unnecessary on day 4, and she was extubated on day 6 with complete recovery of consciousness. Close monitoring of the patient data showed recovery of the decreased anion gap as indicator of the serum lithium level reduction. On day 36, she was discharged without any complication and sequela. The current case highlighted the effective use of CHDF between IHD sessions to prevent the rebound elevation of lithium and the role of the anion gap as a surrogate marker of serum lithium concentration during the treatment."}, {"id": "wiki20220301en000_324536", "title": "Mood stabilizer", "score": 0.011349477219784387, "content": "Mineral Lithium – Lithium is the \"classic\" mood stabilizer, the first to be approved by the US FDA, and still popular in treatment. Therapeutic drug monitoring is required to ensure lithium levels remain in the therapeutic range: 0.6 or 0.8-1.2 mEq/L (or millimolar). Signs and symptoms of toxicity include nausea, vomiting, diarrhea, and ataxia. The most common side effects are lethargy and weight gain. The less common side effects of using lithium are blurred vision, a slight tremble in the hands, and a feeling of being mildly ill. In general, these side effects occur in the first few weeks after commencing lithium treatment. These symptoms can often be improved by lowering the dose."}, {"id": "wiki20220301en415_39692", "title": "Lithium (medication)", "score": 0.010789014821272886, "content": "Lithium concentrations in whole blood, plasma, serum or urine may be measured using instrumental techniques as a guide to therapy, to confirm the diagnosis in potential poisoning victims or to assist in the forensic investigation in a case of fatal overdosage. Serum lithium concentrations are usually in the range of 0.5–1.3 mmol/L (0.5–1.3 mEq/L) in well-controlled people, but may increase to 1.8–2.5 mmol/L in those who accumulate the drug over time and to 3–10 mmol/L in acute overdose. Lithium salts have a narrow therapeutic/toxic ratio, so should not be prescribed unless facilities for monitoring plasma concentrations are available. Doses are adjusted to achieve plasma concentrations of 0.4 to 1.2 mmol /L on samples taken 12 hours after the preceding dose. Given the rates of thyroid dysfunction, thyroid parameters should be checked before lithium is instituted and monitored after 3–6 months and then every 6–12 months."}, {"id": "wiki20220301en487_12081", "title": "Lithium toxicity", "score": 0.010607759587351424, "content": "Gastric lavage. A tube is placed through the nose or mouth into the stomach. The tube is used to remove lithium that has not been digested yet. It may also be used to put medicines directly into the stomach to help stop lithium from being absorbed. Use of an artificial kidney to clean the blood (dialysis). This is usually done only in the most severe cases. Diuretic medications such as furosemide and hydration via intravenous normal saline appear to be effective in speeding the removal of lithium and also rehydrate patients who've lost fluids. Hemodialysis. Hemodialysis is widely advocated as a means of reducing the risk of permanent neurological sequelae following lithium poisoning. Although hemodialysis clearly enhances the elimination of lithium, it is unclear whether this translates into improved patient outcomes. People may be sent home once their lithium level is less than 1.5 mEq/L and they have no symptoms. See also"}, {"id": "InternalMed_Harrison_3685", "title": "InternalMed_Harrison", "score": 0.010577236383687997, "content": "A stuporous 22-year-old man was admitted with a history of behaving strangely. His friends indicated he experienced recent emotional problems stemming from a failed relationship and had threatened suicide. There was a history of alcohol abuse, but his friends were unaware of recent alcohol consumption. The patient was obtunded on admission, with no evident focal neurologic deficits. The remainder of the physical examination was unremarkable. Na+ 140 meq/L K+ 5 meq/L Cl− 95 meq/L HCO3− 10 meq/L Glucose 125 mg/dL BUN 15 mg/dL Creatinine 0.9 mg/dL Ionized calcium 4.0 mg/dL Plasma osmolality 325 mOsm kg/H2O Urinalysis revealed crystalluria, with a mixture of envelope-shaped and needle-shaped crystals."}, {"id": "Neurology_Adams_9710", "title": "Neurology_Adams", "score": 0.010146056015667398, "content": "With blood levels of lithium in the upper therapeutic range (therapeutic 0.6 to 1.2 mEq/L), it is not uncommon to observe a fast-frequency action tremor or asterixis, together with nausea, loose stools, fatigue, polydipsia, and polyuria. These symptoms usually subside with time. Above a level of 1.5 to 2 mEq/L, particularly in patients with impaired renal function or in those taking a thiazide diuretic, serious intoxication becomes manifest—clouding of consciousness, confusion, delirium, dizziness, nystagmus, ataxia, stammering, diffuse myoclonic twitching, and nephrogenic diabetes insipidus. Vertical (downbeating) nystagmus and opsoclonus (see Chap. 13) may also be prominent. A variety of skin problems is common including worsening of acne vulgaris. An uncommon toxic effect is the development of goiter but most patients remain euthyroid although the thyroid-stimulating hormone (TSH) levels may increase slightly. The goiter usually requires no treatment but it is possible to"}, {"id": "Neurology_Adams_12202", "title": "Neurology_Adams", "score": 0.01009266720386785, "content": "becomes effective, usually a matter of 4 or 5 days. The usual dosage of lithium is 1,200 to 2,400 mg daily in divided oral doses, which produces a desired serum level of 0.9 to 1.4 mEq/L. The serum level of lithium must be checked frequently, both to ensure that a therapeutic dose is being taken and to guard against toxicity (see later)."}, {"id": "InternalMed_Harrison_31726", "title": "InternalMed_Harrison", "score": 0.010035035035035035, "content": "In the treatment of acute mania, lithium is initiated at 300 mg bid or tid, and the dose is then increased by 300 mg every 2–3 days to achieve blood levels of 0.8–1.2 meq/L. Because the therapeutic effect of lithium may not appear until after 7–10 days of treatment, adjunctive usage of lorazepam (1–2 mg every 4 h) or clonazepam (0.5–1 mg every 4 h) may be beneficial to control agitation. Antipsychotics are indicated in patients with severe agitation who respond only partially to benzodiazepines. Patients using lithium should be monitored closely, since the blood levels required to achieve a therapeutic benefit are close to those associated with toxicity. Valproic acid may be better than lithium for patients who experience rapid cycling (i.e., more than four episodes a year) or who present with a mixed or dysphoric mania. Tremor and weight gain are the most common side effects; hepatotoxicity and pancreatitis are rare toxicities."}, {"id": "InternalMed_Harrison_3656", "title": "InternalMed_Harrison", "score": 0.009956492637215528, "content": "A 32-year-old man was admitted to the hospital with weakness and hypokalemia. The patient had been very healthy until 2 months previously when he developed intermittent leg weakness. His review of systems was otherwise negative. He denied drug or laxative abuse and was on no medications. Past medical history was unremarkable, with no history of neuromuscular disease. Family history was notable for a sister with thyroid disease. Physical examination was notable only for reduced deep tendon reflexes. Sodium 139 143 meq/L Potassium 2.0 3.8 meq/L Chloride 105 107 meq/L Bicarbonate 26 29 meq/L BUN 11 16 mg/dL Creatinine 0.6 1.0 mg/dL Calcium 8.8 8.8 mg/dL Phosphate 1.2 mg/dL Albumin 3.8 meq/L TSH 0.08 μIU/L (normal 0.2–5.39) Free T4 41 pmol/L (normal 10–27)"}, {"id": "pubmed23n0035_294", "title": "[Origin and treatment of the hypokalemic paresis (author's transl)].", "score": 0.009900990099009901, "content": "The abuse of laxatives and a prolonged treatment with diuretics has to be brought into consideration as the most common cause for renal or intestinal loss of potassium. Besides characteristical alterations at the E.C.G. and besides intestinal disturbances there do occur again and again acute, life-threatening aspects of cases connected with tetraplegias and a respiratory failure. By means of 3 cases from our hospital and 27 casuistics in literature symptomatology and dynamic in the development of hypokaliemia is discussed. The mean of potassium in the serum of the 16 patients, those having quadriplegias, ran up to 1,7 mval/l (range 1,4-2,5). Paralysis develops peracutely in 4 of the cases within hours and in 12 of the cases within days. In the anamnesis symptoms of adynamia could be traced with nearly every patient. A functional psychosis (reversible physically founded psychosis) couldn't be detected in any of the cases. With the help of a administration of potassium one could achieve a total retrogression of the symptoms. Besides this a normalisation of the acide-base equilibrium is required because of a metabolic alkalosis, detectable in most of the cases."}, {"id": "pubmed23n0422_8094", "title": "[Aetiologies of lithium overdose: 10-year experience of Marseille poison centre].", "score": 0.00980392156862745, "content": "Lithium is used for control of bipolar disorders. In order to precise the different circumstances at the origin of poisonings, the authors present the cases of lithium intoxication observed in the Marseille poison centre between January 1991 and December 2000. Retrospective study. Three hundred and four cases were observed during the studied period (1 patient a case), concerning 6 different circumstances. For 3 of them, the symptoms were mild: accidental ingestion with children (13 cases); mistakes on the quantities of ingested tablets (43 cases); elevation of lithium blood level due to diuretic therapy (8 cases). For 2 other circumstances, the clinical signs were more severe: treated patients who developed renal failure (15 cases, 6 patients managed in intensive care unit [ICU], 1 death) or dehydration (35 cases, 8 patients treated in ICU and 1 death). Finally, the most severe cases were collected with suicide attempts. Fifty-six percent of the patients were managed in ICU, 5% needed haemodialysis, 10% had cardiac (repolarization disturbances) or neurological (seizures) complications, 2% died. The severity of lithium poisonings depends of the circumstances. Ingestion of high quantities of sustained released tablets is the most dangerous situation. Accidental ingestion, even with children, must be considered as less severe situations."}, {"id": "Neurology_Adams_9712", "title": "Neurology_Adams", "score": 0.009769961808331113, "content": "The myoclonic state, particularly when combined with confusion and sharp waves in the EEG, may mimic Creutzfeldt-Jakob disease (see Chap. 32) but there should be no problem in diagnosis if the setting of the illness and the administration of lithium are known. At blood lithium concentrations above 3.5 mEq/L, these symptoms are replaced by stupor and coma, sometimes with convulsions, and may prove fatal. Discontinuing lithium in the intoxicated patient, which is the initial step in therapy, does not result in immediate disappearance of toxic symptoms. This may be delayed by a week or two, and the diabetes insipidus may persist even longer. Fluids, sodium chloride, aminophylline, and acetazolamide promote the excretion of lithium. Lithium coma may require hemodialysis, which has proved to be the most rapid means of reducing the blood lithium concentration."}, {"id": "wiki20220301en000_287929", "title": "Lithium carbonate", "score": 0.009708737864077669, "content": "Lithium carbonate is used as a psychiatric medication to treat mania, the elevated phase of bipolar disorder. Prescription lithium carbonate from a pharmacy is suitable for use as medicine in humans while industrial lithium carbonate is not since the latter may, for example, contain unsafe levels of toxic heavy metals or other toxicants. After ingestion, lithium carbonate is dissociated into pharmacologically active lithium ions (Li+) and (non-therapeutic) carbonate, with 300 mg of lithium carbonate containing approximately 8 mEq (8 mmol) of lithium ion. According to the Food and Drug Administration (FDA), 300–600 mg of lithium carbonate taken two to three times daily is typical for maintenance of bipolar I disorder in adults, where the exact dose given varies depending on factors such as the patient's serum lithium concentrations, which must be monitored by a physician to avoid lithium toxicity and potential kidney damage (or even failure) from lithium-induced nephrogenic diabetes"}, {"id": "article-24349_30", "title": "Lithium -- Toxicity -- Recommendations", "score": 0.009705540488182875, "content": "The guidelines established by EXtracorporeal TReatments In Poisoning (EXTRIP) are listed below. Initiate extracorporeal treatment for patients with severe lithium poisoning presenting with coma, myoclonus, convulsions, or cardiopulmonary collapse. Initiate extracorporeal treatment when impaired kidney function is evident, and the lithium concentration surpasses 4 mEq/L. Hemodialysis is also indicated in patients with altered consciousness, seizures, or life-threatening dysrhythmias, regardless of the lithium concentration. Consider extracorporeal treatment if the lithium concentration exceeds 5 mEq/L, significant confusion is evident, or the projected duration for reducing the lithium concentration below 1 mEq/L extends beyond 36 hours. Continue extracorporeal treatment until clinical improvement or lithium concentration drops below 1 mEq/L. In cases where lithium concentration measurement is unattainable, maintain extracorporeal therapies for at least 6 hours."}, {"id": "wiki20220301en487_12075", "title": "Lithium toxicity", "score": 0.009641537774530102, "content": "Lithium toxicity, also known as lithium overdose, is the condition of having too much lithium. Symptoms may include a tremor, increased reflexes, trouble walking, kidney problems, and an altered level of consciousness. Some symptoms may last for a year after levels return to normal. Complications may include serotonin syndrome. Lithium toxicity can occur due to excessive intake or decreased excretion. Excessive intake may be either a suicide attempt or accidental. Decreased excretion may occur as a result of dehydration such as from vomiting or diarrhea, a low sodium diet, or from kidney problems. The diagnosis is generally based on symptoms and supported by a lithium level of greater than 1.2 mEq/L."}, {"id": "pubmed23n0802_12127", "title": "Case report on lithium intoxication with normal lithium levels.", "score": 0.009615384615384616, "content": "An 18-year old female was admitted to a psychiatric hospital with an initial episode of mania. Treated with routine dosages of lithium bicarbonate, her symptoms resolved after two weeks; she was discharged on a dosage of 250mg lithium bid. Five days after discharge she was taken to the emergency department of a general hospital with loss of appetite and disturbed consciousness. The general hospital physicians were unable to diagnose the problem so she was transferred back to the psychiatric hospital. At that time she had a lithium blood level of 0.57 mmol/L (i.e., at the lower end of the therapeutic range) but was, nevertheless, clearly experiencing lithium intoxication with anuria, trembling extremities, blurred speech, muscle rigidity and hyperactive tendon reflexes. Treated with intravenous mannitol, her acute symptoms resolved quickly. The case highlights the need to monitor clinical symptoms of intoxication in all patients taking lithium, regardless of their blood level, and to inform patients, family members, and general physicians about the symptoms and management of lithium intoxication."}, {"id": "pubmed23n0607_14684", "title": "[Analysis of the poisonings by lithium in a department of internal medicine].", "score": 0.009345794392523364, "content": "Lithium salts have been mainly used in the treatment of bipolar disorder. Because of its narrow therapeutic range, and several well characterised adverse effects, serum lithium levels must be monitored regularly in patients given lithium treatment in order to prevent intoxication. To describe the clinic and toxic characteristics in inpatients at our Clinic Toxicologic Unit. Descriptive and retrospective study of lithium intoxications in 150 inpatients between 2003 and 2006. Patients were classified based on their neuropsychiathric symptom profile and serum lithium levels. Sixteen of 150 inpatients had lithium intoxication: 58.3% women and 43.8% men; 49.19% +/- 18.49% years old. Lithium was used as treatment of bipolar disorder in 87.5% of cases. The most frequent cause of intoxication was attempted suicide. Using neuropsychiatric parametres, intoxication was moderate in 50% of cases, and mild in 25% and severe or very severe in 25%. Using serum lithium levels, intoxication was very severe in 31.35%, severe in 25%, and slight-moderate in 43.7%. Conservative measures were used as the most frequent treatment (50%), and haemodialfiltration was needed in 37.5%. Mean stay was 4,8 days in acute intoxication, and 11.2 days in chronic. Sequelaes were found in two patients (ataxia). Death was not present. Lithium intoxications can involve severe complications, even death. Narrow control is encouraged in polymedicated and elderly patients, and in concommitant treatment with antidepressant and neuroleptics."}, {"id": "pubmed23n1041_9231", "title": "Lithium toxicity with prolonged neurologic sequelae following sleeve gastrectomy: A case report and review of literature.", "score": 0.009259259259259259, "content": "Lithium is the first-line medication for bipolar disorder, given a narrow therapeutic window of 0.8 to 1.2 mEq/L. Change of lithium pharmacokinetics following bariatric surgery may lead to lithium toxicity, which is particularly concerned. We presented a 39-year-old man with morbid obesity and bipolar affective disorder for 20 years, who was treated with lithium. He developed serious lithium toxicity following sleeve gastrectomy and prolonged neurologic sequelae. He suffered from persistent watery diarrhea, general weakness, and then drowsy consciousness. Lithium level was checked immediately to be 3.42 mEq/L and lithium toxicity was diagnosed. After 3 courses of hemodialysis, his serum lithium level subsequently declined to 0.63 mEq/L, while his consciousness returned normal. Lithium was replaced by lamotrigine. The patient was discharged thirty-five days after admission, while his serum lithium declined to 0.06 mEq/L. Neurologic sequelae were noted by muscle weakness and pain sensation in both feet. The nerve conduction test revealed sensorimotor polyneuropathy with conduction block. He was advised to keep a passive range of motion exercise. Although the consensus guideline remains lacking, our report reviewed cases of relevance in the literature and highlighted the awareness of the potential risk of lithium toxicity following bariatric surgery. We suggest close monitoring of the lithium levels and perhaps a dosage adjustment for the postoperative period."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 221]], "word_ranges": [[0, 33]], "text": "There is no doubt, they are not meeting the diagnostic criteria of Kawasaki disease (fever of several days of evolution, exanthema, conjunctival injection, red lips, raspberry tongue, edema of acral parts and adenopathy)."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "There is no doubt, they are not meeting the diagnostic criteria of Kawasaki disease (fever of several days of evolution, exanthema, conjunctival injection, red lips, raspberry tongue, edema of acral parts and adenopathy).", "full_answer_no_ref": "There is no doubt, they are not meeting the diagnostic criteria of Kawasaki disease (fever of several days of evolution, exanthema, conjunctival injection, red lips, raspberry tongue, edema of acral parts and adenopathy).", "full_question": "A 4-year-old girl presenting with a high fever of 6 days' evolution. On clinical examination she presents an erythematous maculopapular rash on the trunk and genital area, with a tendency to confluence, without becoming scarlatiniform; conjunctival injection without secretions and red lips with raspberry tongue. She also presents erythema with edema in hands and feet and a unilateral cervical adenopathy of 2 cm in diameter. The most likely clinical diagnosis of suspicion is:", "id": 552, "lang": "en", "options": {"1": "Kawasaki disease.", "2": "Measles.", "3": "Rubella.", "4": "Scarlet fever.", "5": NaN}, "question_id_specific": 89, "type": "DERMATOLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0735_10590", "title": "Catastrophic Kawasaki disease unresponsive to IVIG in a 3-month-old infant: a diagnostic and therapeutic challenge.", "score": 0.018596642272922945, "content": "The present report describes the severe evolution of Kawasaki disease in a three-month-old infant. The ailment was initially atypical in its presentation, with the patient exhibiting only persistent fever in association with a progressive lethargy and maculopapular rash on the face, trunk and limbs erroneously diagnosed as roseola infantum. On the 10th day of the condition, mainly due to the unexplained persistence of fever, the infant was admitted to a local hospital. The typical features of KD appeared only on the 14th day of illness with the relapse of the maculopapular rash in association with non-purulent conjunctivitis; dry, reddish and fissured lips; tongue with reddish and hypertrophic papillae; erythema and edema of the palms and soles. During the following days, the ailment rapidly evolved to a catastrophic clinical picture characterized by generalized vasculitis, splenic infarction, pulmonary thrombosis, giant right and left coronary aneurysms, dilatation of common and internal iliac arteries and progressive ischemia of the distal third of the feet resulting in necrotic lesions of both halluces. Appropriate therapy was initiated, but repeated administration of intravenous immunoglobulin G (IVIG) followed by three days of administration of methylprednisolone did not abate the intense inflammatory activity. The remission of inflammation and regression of vascular lesions were only achieved during the following five weeks after the introduction of methotrexate associated with etanercept. The report of this case aims to draw attention to severe forms of KD that exhibit an unfavorable evolution and can be extremely refractory to the conventional therapy."}, {"id": "pubmed23n0985_24440", "title": "Late Treatment and Recurrence of Kawasaki Disease in a Moroccan Infant.", "score": 0.017862838915470493, "content": "While the diagnosis of typical form of Kawasaki disease (KD) is obvious, this multifaceted disease continues to surprise us. We report the case of a recurrent Kawasaki disease in an infant. At the age of 13 months, the infant was diagnosed with complete Kawasaki disease; he presented with prolonged fever, bilateral conjunctivitis, enanthem, exanthema, edema of the lower limb, peeling, and biological inflammatory syndrome. He was treated with intravenous immunoglobulin (IVIG) associated with a high dose of aspirin and then an antiplatelet dose with a good clinical-biological evolution. The echocardiography was normal. Seven months later, the patient was again admitted, in a similar picture: a prolonged fever evolving for 7 days, bilateral conjunctivitis, enanthem, cervical adenopathy of 1.5 cm/1 cm, scarlatiniform erythema, pruriginous of the trunk and limb, and peeling of the toes, with indurated edema of the hands and feet. The rest of the examination was normal except the irritability. The diagnosis of recurrent KD was made according the five criteria of the American Heart Association. The echocardiography was normal again. The infant received IVIG with good outcome. Despite its rarity, the possibility of recurrence of KD should be known by clinicians, so as not to delay the specific management of vasculitis whose stakes in terms of prevention of coronary artery lesions are well known. Our case confirms the possibility of this recurrence."}, {"id": "InternalMed_Harrison_4123", "title": "InternalMed_Harrison", "score": 0.017588325652841783, "content": "The cutaneous eruption in Kawasaki disease (Chap. 385) is polymorphous, but the two most common forms are morbilliform and scarlatiniform. Additional mucocutaneous findings include bilateral conjunctival injection; erythema and edema of the hands and feet followed by desquamation; and diffuse erythema of the oropharynx, red strawberry tongue, and dry fissured lips. This clinical picture can resemble TSS and scarlet fever, but clues to the diagnosis of Kawasaki disease are cervical lymphadenopathy, cheilitis, and thrombocytosis. The most serious associated systemic finding in this disease is coronary aneurysms secondary to arteritis. Scarlatiniform eruptions are also seen in the early phase of SSSS (see “Vesicles/Bullae,” above), in young adults with Arcanobacterium haemolyticum infection, and as reactions to drugs."}, {"id": "pubmed23n0271_3624", "title": "[Acute renal insufficiency in Kawasaki disease].", "score": 0.016865079365079364, "content": "Kawasaki disease is an acute inflammatory condition characterized by various combinations of features but renal involvement is rare. This report is of a case of Kawasaki disease complicated by acute kidney failure. A 10 year-old girl was admitted because of acute renal failure with fever. She developed a high fever, and her general condition was poor; she had developed a macular erythematous rash 10 days earlier for which she was given cefadroxil. At admission, she remained febrile and had strawberry tongue, pharyngitis, dry erythematous lips, bilateral conjunctivitis, cervical lymphadenopathy and desquamation of the skin on her hands. She was anemic (hemoglobin = 9.6 g%), leukocytotic (33,100/mm3), but with no burr, fragmented red blood cells or thrombocytopenia. Her plasma C-reactive protein level was 236 mg/l; her blood urea was 9.5 mmol/l, her creatininemia 288 mumol/l and proteinuria was 0.5 g/l without hematuria. Urine cultures did not grow. Her blood transaminase and gammaglutamyltransferase activities were elevated. Ultrasonography of kidneys and coronary arteries was normal. Kidney biopsy performed one day after admission showed no vascular or glomerular changes, but renal tubular necrosis, indicating urinary excretion of pigments. Tests for myoglobinemia, myoglobinuria and blood muscle enzyme activities were all positive. The renal failure disappeared within 10 days but the fever and inflammatory manifestations persisted for 1 1/2-2 months despite two treatments of intravenous gammaglobulins and continuous salicylate administration. The patient developed arthralgias at the end of the first month of disease, but recovered without renal or vascular complications. Several cases of renal involvement have been reported during the course of Kawasaki disease. They have been rarely documented by histological examination so that the vascular origin of changes has not been demonstrated. Myoglobinuria, as seen in muscular crush injury, and in our case possibly due to malignant hyperthermia, may be responsible for the transient acute renal failure."}, {"id": "article-23847_9", "title": "Kawasaki Disease -- History and Physical", "score": 0.016260162601626018, "content": "In 2014, the American Heart Association (AHA) published the criteria needed to establish a diagnosis. [1] However, it is important to note that children who fall short of the full criteria but have cardiac abnormalities on echocardiogram meet the diagnosis of KD. [1] The patient must have fevers for five or more days, with at least four of the following criteria (either all at once or over a series of days): Bilateral painless bulbar conjunctival injection without exudate Erythematous mouth and pharynx, strawberry tongue or red, cracked lips Polymorphous exanthem (morbilliform, maculopapular, or scarlatiniform) Swelling of hands and feet with erythema of the palms and soles Cervical lymphadenopathy (over 1.5 cm in diameter)"}, {"id": "pubmed23n0482_11745", "title": "An unusual pattern of arthritis in a child with Kawasaki syndrome.", "score": 0.015617403986387944, "content": "Arthritis is reported in one-third of cases with Kawasaki syndrome. It may have an early or a late onset form. We present a 15-month-old-girl who had been referred with complaints of pain and swelling in her left shoulder. Physical examination revealed bulbar conjunctival injection, erythematous lips and pharynx, strawberry tongue, erythematous rash, edema and erythema of the left shoulder, left knee, right elbow and right wrist, and moderate distress in the left shoulder and left hip. She was diagnosed with Kawasaki syndrome, and intravenous immunoglobulin infusion (IVIG) 2 g/kg and aspirin (100 mg/kg/day) were instituted. The patient had two additional episodes of arthritis involving the hip joint on the 8th day, and the shoulder and metacarpophalangeal (MCP) and interphalangeal (IP) joints of her right hand on the 15th day. Turbid material was aspirated in both instances; Gram and Wright's staining of this material showed many leukocytes but no bacteria. A second dose of IVIG (1 g/kg) was given. At the end of the third week all extremities were painless, with a normal range of motion. Arthritis in our patient was the presenting sign, having a 'septic arthritis mimicking' and 'biphasic' pattern. Although the patient presented with severe and recurrent arthritis, which is significantly correlated with severe multisystem disease and the presence or development of coronary artery aneurysm, the response to IVIG was excellent."}, {"id": "pubmed23n1039_17116", "title": "A Case of Kawasaki Disease with Intussusception.", "score": 0.015503875968992248, "content": "Kawasaki disease (KD) is a systemic vasculitis of unknown cause and is associated with various digestive disorders, although only a few cases of intussusception associated with KD have been reported. We describe a case of intussusception followed by KD in a 3-year-old boy. The patient was admitted to our hospital for evaluation of severe abdominal pain. Because the target sign was seen on ultrasonography, intussusception was diagnosed and hydrostatic reduction was performed. On the second day after admission, he developed a high fever (38°C) and an irregular rash over his whole body. On the fourth day after admission, the high fever continued, and bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, strawberry tongue, indurated edema of the dorsa of the hands and feet, and diffuse erythema of the palms and soles appeared, and KD was ultimately diagnosed. He was treated with intravenous immunoglobulin 2 g/kg, aspirin 30 mg/kg/day, and prednisolone 2 mg/kg/day. The high fever and other clinical symptoms resolved immediately after the start of treatment. There was no relapse of KD symptoms after initial treatment, and periungual desquamation was observed on the 10th day after admission. He was discharged on the 15th day, without abnormalities such as coronary dilatation, 3 months after the onset of KD symptoms. Patients with intussusception and KD were older (≥3 years vs &lt;3 years) than those with intussusception alone. In addition, the site of intussusception in KD was mainly colonic rather than ileocolic. If intussusception precedes development of the characteristic clinical symptoms of KD, diagnosis of KD may be delayed. KD should be considered in children older than 3 years with intussusception at a colonic site."}, {"id": "pubmed23n0327_56", "title": "Mediastinal lymphadenopathy: a variant of incomplete Kawasaki disease.", "score": 0.01501052398360474, "content": "A 14-month-old girl presented with a 4-d history of fever and generalized exanthema. Four characteristic symptoms of incomplete Kawasaki disease (KD) were present on admission (fever, rash, non-purulent conjunctival injection, oropharyngeal changes) and then followed by oedema of the hands and feet and mild plantar desquamation. The typical laboratory features of KD, such as elevated erythrocyte sedimentation rate, leukocytosis, thrombocytosis, and positive C-reactive protein were also seen. Ultrasound examination of the mediastinum revealed the presence of a lymph node, 30 mm in diameter, below the tracheal carina. Thoracic CT scan confirmed the mediastinal lymph node. The patient was treated with aspirin and intravenous gamma-globulin. Ultrasound study of the mediastinum, which was carried out 6 weeks after hospital discharge, showed that the lymph node had disappeared. This case illustrates that lymph nodes other than cervical lymphadenopathy should be sought when the diagnosis of classical or atypical KD is suspected."}, {"id": "pubmed23n0723_13682", "title": "Unusual manifestations of Kawasaki disease with retropharyngeal edema and shock syndrome in a Taiwanese child.", "score": 0.014988265414977598, "content": "We report a 3-year-old girl with Kawasaki disease who presented with retropharyngeal edema and shock syndrome. This is the first reported case in Taiwan. The patient initially presented with fever, cough, and pyuria followed by rapidly progressive enlarged bilateral cervical lymphadenopathy. On the third day of the fever, computed tomography for airway compression sign found widening of the retropharyngeal space mimicking a retropharyngeal abscess. Later, an endotracheal tube was inserted for respiratory distress. A skin rash over her trunk was also noted. On the fifth day of the fever, the clinical course progressed to hypotension and shock syndrome. Because of more swelling of bilateral neck lymph nodes, computed tomography was arranged again and revealed partial resolution of the edematous changes in the retropharyngeal space. Edema of the hands and feet, bilateral bulbar conjunctivitis, and fissured lips were subsequently found. The diagnosis of Kawasaki disease was confirmed on the eighth day of fever. There was no evidence of bacterial infection. She was administered intravenous immunoglobulin (2 mg/kg) and high dose aspirin (100 mg/kg/day). One day later, the fever subsided, and her blood pressure gradually became stable. Heart echocardiography on the Day 13 revealed dilated left coronary artery and mitral regurgitation. Follow-up echocardiography six months later showed normal coronary arteries. To date, the patient has not experienced any complications. This case illustrates that retropharyngeal edema and shock syndrome can be present in the same clinical course of Kawasaki disease. Clinicians and those who work in intensive care units should be aware of unusual presentations of Kawasaki disease to decrease rates of cardiovascular complications. "}, {"id": "pubmed23n0631_10241", "title": "[A case report of acute Q fever showing Kawasaki disease-like symptoms in a 9-year-old girl].", "score": 0.014983164983164984, "content": "A 9-year-old girl developing fever and hyperemia of both bulbar conjunctiva 5 days before admission to the Saitama Children's Medical Center after antibiotics proved ineffective was found on admission to have general fatigue and a temperature of 39 degrees C. Physical examination showed hyperemia of the bulbar conjunctiva, fissures of the lips, redness of the pharynx, and swelling of the cervical lymph nodes. Laboratory tests detected neutrophilia (11,200/microL), mild anemia (11.4g/dL), thrombocytopenia (110,000/microL), and elevated serum aspartate aminotransferase (242IU/L), alanine aminotransferase (328IU/L), and C-rective protein (25.2 mg/dL). Autoantibodies such as anti-nuclear, anti-SS-A/Ro, and anti-Jo-1 were also found. Echocardiography showed no abnormality of the coronary arteries. She was diagnosed as having incomplete Kawasaki disease on day 7 of illness, necessitating that a high dose of immunoglobulin be given intravenously. Her temperature dropped temporarily to 37 degrees C, but she developed erythema of the cheek and fever. Intravenous immunoglobulin was restarted, and minocycline introduced because her daily contact with a pet cat indicated richettsial infection such as Q fever. Mild fever, muscle pain, and elevated C-reactive protein did not improve, but clinical signs and symptoms gradually lessened after ibuprofen was given, then disappeared. A definitive diagnosis of Q fever was made through an over 4-fold rise in phase II IgG antibody titers against Coxiella burnetii, titer of less than 1 : 16 on day 14 of illness, and titer of 1 : 256 on day 34. This case study describes on atypical case of Q fever with clinical manifestations mimicking Kawasaki disease."}, {"id": "pubmed23n1010_25280", "title": "Uncommon erythema multiforme in small children: experience of a single Romanian pediatric unit: Two case reports.", "score": 0.014605543710021322, "content": "Erythema multiforme (EM) is an immune-mediated disease with mucocutaneous localization and plurietiologic determinism. The term \"multiforme\" refers to the variety of aspects that the lesions can take from patient to patient and during evolution in a single patient. We have selected 2 cases of small children diagnosed with different etiology of EM to illustrate the importance of a correct and fast diagnosis. Case 1 involves a 2-year-old girl from a rural area who presented with fever and pruritic erythematous papular eruption. The onset of the symptoms was 3 days before presentation with fever and ulcerative lesions on the oral and labial mucosa, followed by the appearance of erythematous macular lesions, with progressive confluence to intense pruritic patches. The 2nd involves a 2-year-old boy with fever, loss of appetite, productive cough, and petechiae. He had corticosensible immune thrombocytopenia from the age of 6 months, with many recurrences. The patient received treatment with ampicillin/sulbactam and symptomatics for an erythemato-pultaceous angina. During the 2nd day of treatment the patient developed an erythematous macular eruption on the face, scalp, trunk, and limbs, with bullae formation. The 1st patient was diagnosed based on biologic findings: positive inflammatory syndrome, elevated level of anti-Mycoplasma pneumoniae immunoglobulin M antibodies and immunoglobulin E. Histopathologic examination described papillary dermal edema, inflammatory infiltrate, and lymphocyte exocytosis. In the 2nd case, the hemoleucogram identified 12,000/mm platelets and the medulogram aspect was normal. Serology for Epstein-Barr virus was negative. The diagnosis was EM secondary to M pneumoniae infection in case 1 and secondary to administration of ampicillin/sulbactam in case 2. In both cases, etiopathogenic treatment consisting of steroidal antiinflammatory drugs, antihistamines was administered. Because of specific etiology, the 1st case received antibiotics. The evolution was favorable in 10 to 14 days; the patients were discharged after etiopathogenic treatment consisting of steroidal antiinflammatory drugs, antihistamines, and/or antibiotics. Performing a detailed clinical examination, medical history of drug use, infection or general diseases can establish a good diagnosis of EM. Histopathologic examination can help. The treatment is etiologic, pathogenic, and symptomatic. EM usually has a self-limited evolution."}, {"id": "pubmed23n0520_17294", "title": "Concomitant dengue infection and Kawasaki disease in an infant: a case report and literature review.", "score": 0.014150487781319424, "content": "A previously healthy 11-month-old girl presented with fever and rash for 6 days. Physical examination revealed an irritable infant with a high fever, injected conjunctivae, red cracked lips, posterior auricular lymphadenopathy, hepatomegaly, generalized erythematous maculopapular rash and petechial hemorrhage on trunk, face and extremities. Complete blood count showed atypical lymphocytosis and thrombocytopenia. Dengue infection was initially diagnosed. The persistent fever and clinical manifestations of Kawasaki disease (KD) were observed on day 8 with high erythrocyte sedimentation rate (56 mm/hr). Treatment of KD included intravenous immunoglobulin on day 9 of the illness. Desquamation of the fingers was found on day 15 of the illness. Ectasia of left coronary artery with small aneurysmal dilatation was detected by echocardiography on day 15 of the illness. Hemagglutination-inhibition test and enzyme-linked immunosorbent assay for dengue virus eventually showed a four-fold rising. According to the literature review, this is the second reported case of dengue infection concomitant with KD. The natural course of each disease may be modified and causes some difficulties in diagnosis and management."}, {"id": "First_Aid_Step2_934", "title": "First_Aid_Step2", "score": 0.014141613924050632, "content": "Cervical lymphadenopathy (often painful and unilateral, with at least one node > 1.5 cm). Diffuse mucous membrane erythema (e.g., “strawberry tongue”); dry, red, chapped lips. Erythema of the palms and soles; indurative edema of the hands and feet; late desquamation of the fingertips (in the subacute phase). Other manifestations include sterile pyuria, gallbladder hydrops, hepatitis, and arthritis. Untreated Kawasaki disease can lead to coronary aneurysms and even myocardial infarction! Conjunctivitis Rash Adenopathy Strawberry tongue Hands and feet (red, swollen, f aky skin) BURN (fever > 40°C for ≥ 5 days) Subacute phase: Begins after the abatement of fever and typically lasts for an additional 2–3 weeks. Manifestations are thrombocytosis and elevated ESR. Untreated children may begin to develop coronary artery aneurysms (40%); all patients should be assessed by echocardiography at diagnosis."}, {"id": "pubmed23n1118_16380", "title": "Nontypical presentation of a common disease: a case report.", "score": 0.014009009009009008, "content": "Kawasaki disease is an idiopathic medium-sized vasculitis that occurs primarily in infants and children younger than 5 years of age. Atypical Kawasaki disease applies to patients who do not fulfill the complete criteria of fever of 5 days or more with at least four of five features: bilateral conjunctival injection, changes in the lips and oral cavity, cervical lymphadenopathy, extremity changes, and polymorphous rash. Acute kidney injury is defined as a sudden decline in kidney function within hours, including structural injuries and loss of function. Acute kidney injury is extremely common in hospitalized pediatric patients. However, it is rarely documented in Kawasaki disease. Acute kidney injury is underestimated in Kawasaki disease due to the lack of a clear definition of age-specific normal serum creatinine levels and routine renal functions. This report describes a case who presented with clinical features suggestive of atypical Kawasaki disease and developed acute kidney injury. A 2-year-old Saudi girl had a history of high-grade fever for 5 days, moderate dehydration, dry cracked lips, poor appetite, and generalized erythematous rash; therefore, she was diagnosed to have incomplete Kawasaki disease. Laboratory investigations revealed normochromic normocytic anemia, leukocytosis, thrombocytosis, high inflammatory markers, and acute kidney injury stage III. An echocardiogram showed a 4-mm dilatation on the left main coronary artery and a 3-mm dilatation on the right. A renal biopsy was not performed to identify the cause of the injury as it showed improvements after the start of the specific therapy for Kawasaki disease; intravenous immune globulin at a dose of 2 g/kg, aspirin at a high dosage of 80 mg/kg/day, and prednisolone at 2 mg/kg. In addition to the acute kidney injury management, normal saline boluses were followed by furosemide at a 2 mg/kg dose. Her urine output increased, and her renal functions normalized. She was discharged in good condition after 10 days. It is valuable to check renal function tests in a confirmed case of Kawasaki disease to reduce the negative consequences of late acute kidney injury discovery. Early detection and intervention make a substantial difference in acute kidney injury management."}, {"id": "article-83816_25", "title": "Rubeola (Measles) -- Differential Diagnosis", "score": 0.014009009009009008, "content": "Measles should be distinguished from similar presenting exanthemic diseases of childhood, autoimmune processes, and adverse drug reactions. Rubella causes a rash similar to measles with head to caudal distribution, mild respiratory symptoms, the absence of conjunctivitis. Still, it is accompanied by the presence of adenopathies - which is characteristic of this disease. Roseola is characterized by an illness beginning with a high fever, which subsides after a few days, accompanied by the appearance of a rash in the central part of the body, without the presence of Koplik's points. Mononucleosis is a febrile viral disease, a characteristic course with few symptoms during childhood, contrary to what happens in more advanced ages.  Mononucleosis manifests itself by pharyngeal compromise, polyadenopathy, and hepatosplenomegaly, and the rash can have different forms of presentation. In Kawasaki disease, there is an ocular compromise with the presence of conjunctivitis without exudate, and the respiratory compromise is not part of this pathology. Group A Streptococcus (particularly Scarlet fever) may present with a similar rash (a coarse, sandpaper-like, blanching, erythematous) to measles in association with pharyngitis. [5] [14]"}, {"id": "pubmed23n0516_19231", "title": "Kawasaki disease.", "score": 0.01399703374119392, "content": "Kawasaki disease (KD) is a common vasculitic disorder usually seen in children below 5 years of age. The disease can present with protean clinical manifestations which include high grade fever (for at least 5 days), rash, redness of the lips and a typical strawberry tongue, cervical lymph node enlargement (often unilateral), swelling over the hands/feet and, later a characteristic peripheral desquamation over the fingers and toes. These clinical features appear sequentially and the findings may change from day-to-day. Thus, all these features may not be seen together at any one point of time. The diagnosis rests on the recognition of this characteristic temporal sequence of clinical events, none of which are, by themselves, pathognomonic. Establishing a diagnosis of KD may be further complicated by the occurrence of several other, seemingly unrelated, clinical features. These include irritability, neck stiffness, sterile pyuria, pneumonitis, hydrops of the gallbladder and hepatitis among many others. There is no laboratory test that can help in confirming a diagnosis of KD. Left untreated, up to 20% of children with KD can develop coronary aneurysms with catastrophic long term sequelae. It is important to diagnose KD in the first 10 days of the illness so that appropriate therapy with intravenous immunoglobulin and aspirin can be Initiated. All paediatricians, and physicians looking after children, need to be aware of this condition which is now being increasingly recognized in India."}, {"id": "pubmed23n0048_5013", "title": "Kawasaki syndrome.", "score": 0.01369047619047619, "content": "Kawasaki syndrome, also known as mucocutaneous lymph node syndrome, is an acute vasculitis of infants and young children. We describe a four-year-old girl who presented with fever, a diffuse erythematous maculopapular rash, bilateral nonpurulent bulbar conjunctivitis, dry, red, fissured lips, a tongue with a strawberry \"appearance\", an erythematous pharynx, indurative erythema, and edema and desquamation of the face, hands and feet. She probably developed mitral valve prolapse during the course of the disease. The diagnosis of Kawasaki syndrome was arrived at by excluding other diseases and by the presence of all the clinical criteria for Kawasaki syndrome. Since this syndrome is rarely encountered in Turkey, this case is presented and the literature regarding the syndrome is reviewed."}, {"id": "wiki20220301en033_29253", "title": "Kawasaki disease", "score": 0.013599788447735257, "content": "Classically, five days of fever plus four of five diagnostic criteria must be met to establish the diagnosis. The criteria are: erythema of the lips or oral cavity or cracking of the lips rash on the trunk swelling or erythema of the hands or feet red eyes (conjunctival injection) swollen lymph node in the neck of at least 15 mm Many children, especially infants, eventually diagnosed with Kawasaki disease, do not exhibit all of the above criteria. In fact, many experts now recommend treating for Kawasaki disease even if only three days of fever have passed and at least three diagnostic criteria are present, especially if other tests reveal abnormalities consistent with Kawasaki disease. In addition, the diagnosis can be made purely by the detection of coronary artery aneurysms in the proper clinical setting. Investigations A physical examination will demonstrate many of the features listed above."}, {"id": "pubmed23n0245_6121", "title": "Kawasaki disease in healthy young adult.", "score": 0.013448969331322273, "content": "This report describes a 26-year-old woman who fulfills the criteria for the diagnosis of Kawasaki disease or mucocutaneous lymph node syndrome, an acute febrile illness that usually afflicts young children. The diagnosis is made in persons with fever lasting 5 or more days when four of the following criteria are met: bilateral injection of ocular conjunctivae; the involvement of the mucous membranes of the upper respiratory tract consisting of any combination of the following--redness and fissuring of lips; \"strawberry tongue,\" or erythema of the pharynx; involvement of the peripheral extremities characterized in the early stages by an indurative erythematous rash of palms and soles followed by membranous desquamation; polymorphous nonvesicular truncal exanthem; and acute nonsuppurative enlargement of cervical lymph nodes. An added stipulation is that the illness must not be attributable to a known disease process."}, {"id": "pubmed23n0118_16510", "title": "Kawasaki disease in a 4-year-old boy.", "score": 0.01331028522039758, "content": "A 4-year-old boy experienced sudden fever, followed by a rash on the trunk and extremities and erythema of the pharynx. Five days later, the fever remained and erythema appeared on the oropharynx, tongue, and lips. The skin of the palms and soles became erythematous and indurated, and both conjunctivae became injected. Desquamation of the skin occurred on both thumbs and one finger, and an anterior cervical lymph node was found to be enlarged. The patient was diagnosed as having Kawasaki disease, and treatment with aspirin was started. The desquamation progressed to involve the entire surface of the palms and soles, and then symptoms resolved. Twenty years after recognition of Kawasaki disease, this enigmatic illness continues to defy attempts to understand its etiology and pathogenesis. Most experts agree that the cause is either an environmental toxin or an infectious agent, but other possible causative agents may need to be proposed and investigated."}, {"id": "pubmed23n1001_25845", "title": "Twenty-year-old woman presenting with typical Kawasaki disease.", "score": 0.013254843865736228, "content": "We describe adult-onset Kawasaki disease (KD) and review clinical manifestations and treatment guidelines. Our patient is a 20-year-old female who initially presented to an outside hospital for fever, cervical lymphadenopathy, malaise, exudative tonsillitis, and skin eruption. She received antibiotics for suspected exudative pharyngitis, but experienced continued fevers and presented to the UCLA emergency room one week later. She had diffuse petechial macules coalescing into reticulated patches, fingertip peeling, conjunctival injection, oral erosions, and tongue swelling. Despite her age, given her constellation of symptoms, a diagnosis of typical KD was favored. She was started on high dose aspirin and IVIG, with improvement of rash and conjunctivitis. She was discharged on 325mg of aspirin daily with close follow-up. This case highlights the challenge of diagnosing KD in adults. Although this patient had classic symptoms, she was likely misdiagnosed because KD is rare in adults and without validated criteria. Our patient met the pediatric criteria, suggesting these should be considered when clinical suspicion for adult-onset KD is high. Adult-onset KD is most commonly misdiagnosed as toxic shock syndrome or drug-induced hypersensitivity syndrome and these are important to rule-out. Treatment with high-dose aspirin and IVIG is well established and should be initiated promptly."}, {"id": "pubmed23n0694_19426", "title": "An adult case of kawasaki disease in a pregnant Japanese woman: a case report.", "score": 0.013141025641025641, "content": "Kawasaki disease is an acute febrile disease predominantly seen in young children. We report a case of Kawasaki disease in a 32-year-old pregnant woman. She developed a generalized erythematous skin rash accompanied by high fever. Bilateral conjunctival congestion, tender cervical lymphadenopathy, an edematous lower lip and peripheral edema followed by desquamation were observed. She was successfully treated with aspirin and intravenous gammaglobulin (1 g/kg/day). Her course was not complicated by coronary artery aneurysm and she delivered a healthy baby. To the best of our knowledge, this is the first case of Kawasaki disease in a pregnant woman. We suggest that Kawasaki disease should be included in the differential diagnosis of a generalized, erythematous skin rash accompanied by high fever in adults."}, {"id": "wiki20220301en249_18856", "title": "Systemic vasculitis", "score": 0.013075592227841364, "content": "Kawasaki disease. Usually in children (age<4), it affects large, medium, and small vessels, prominently the coronary arteries. Associated with a mucocutaneous lymph node syndrome. Diagnosis requires fever lasting five days or more with at least four out of five criteria: Bilateral conjunctival injection Injected or fissured lips, injected pharynx, or strawberry tongue Erythema of palms/soles, edema of hands/feet, periungual desquamation Polymorphous rash Cervical lymphadenopathy (at least one node > 1.5 cm) Isolated cerebral vasculitis. Affects medium and small arteries over a diffuse CNS area, without symptomatic extracranial vessel involvement. Patients have CNS symptoms as well as cerebral vasculitis by angiography and leptomeningeal biopsy. Small vessel vasculitis There are several vasculitides that affect small vessels."}, {"id": "InternalMed_Harrison_11793", "title": "InternalMed_Harrison", "score": 0.012918282821642233, "content": "in recent years, although strains of GAS that produce pyrogenic exotoxins continue to be prevalent in the population. The symptoms of scarlet fever are the same as those of pharyngitis alone. The rash typically begins on the first or second day of illness over the upper trunk, spreading to involve the extremities but sparing the palms and soles. The rash is made up of minute papules, giving a characteristic “sandpaper” feel to the skin. Associated findings include circumoral pallor, “strawberry tongue” (enlarged papillae on a coated tongue, which later may become denuded), and accentuation of the rash in skinfolds (Pastia’s lines). Subsidence of the rash in 6–9 days is followed after several days by desquamation of the palms and soles. The differential diagnosis of scarlet fever includes other causes of fever and generalized rash, such as measles and other viral exanthems, Kawasaki disease, TSS, and systemic allergic reactions (e.g., drug eruptions)."}, {"id": "pubmed23n0076_2863", "title": "[Kawasaki disease complicated with hemorrhagic enteritis mimicking intestinal obstruction: report of one case].", "score": 0.01290853031860226, "content": "A 6-month-old little boy presented with fever and cervical lymphadenopathy for four days. On admission, he was found to have conjunctival congestion of both eyes, reddened and fissured lips, straw-berry tongue, macular rash at the trunk and erythematous change on the BCG injection site. The diagnosis of Kawasaki disease was made and aspirin therapy (100 mg/kg/day) was started. On the 3rd hospital day, he developed abdominal distention, jaundice, poor activity and tachypnea. Bowel sound was silent on auscultation. Nasogastric tube was inserted for decompression and bilious material was drained out continuously. In addition, bloody, mucoid stool passage was noted. An abdominal radiography revealed dilatation of the small bowel and paucity of the colon gases. On the abdominal ultrasound, hydrops of gallbladder, marked ascites and silent and dilated bowel loops were found. Despite of supportive care, abdominal symptoms persisted and condition deteriorated. Laparotomy was done on the following day. At operation, it was found that the patient developed severe petechia on the long segment of small intestine from 15cm distal to the Treitz ligament to ileocecal valve and some fibrin plagues on the terminal ileum at 25cm proximal to the ileocecal valve were also found."}, {"id": "pubmed23n1119_3182", "title": "14-month-old girl with prolonged fever, a desquamative rash, and a new left hemiplegia.", "score": 0.012878787878787878, "content": "A young child with fever, erythematous rash, and conjunctivitis in sub-Saharan Africa is usually a case of measles. We report a-14-month-old girl with a prolonged fever, a desquamating erythematous rash, and a new left hemiplegia. This was initially diagnosed as measles but her correct final diagnosis was Kawasaki disease (KD); she very rapidly defervesced and regained normal function of her limbs after appropriate treatment. We believe this is the first reported case in Liberia of KD in a child younger than two years of age."}, {"id": "pubmed23n0905_1989", "title": "Atypical desquamation in a 2.5-year-old boy with Kawasaki disease: A case report.", "score": 0.012766474831806759, "content": "Kawasaki disease (KD) is a vasculitis that mostly affects children under 5 years of age. This article presents a 2.5-year-old boy who presented with 6 days of fever, generalized maculopapular rash, bilateral non-exudative conjunctivitis, cracked lips, right cervical lymphadenopathy, erythematous extremities, and perianal desquamation. Laboratory studies showed leukocytosis and sterile pyuria. Because diagnosis of KD was proved, oral acetylsalicylic acid with the anti-inflammatory dose and intravenous immunoglobulin were started for him. On the seventh day of admission time, he developed desquamation and erythema on the site of his right cervical lymphadenopathy as well as periungual scaling. About three weeks after starting the treatment, scaling of the cervical lymphadenopathy and periungual area stopped. Echocardiography was performed for him three times: at the time of diagnosis, four weeks, and 6 months later and revealed normal coronary arteries. We report this sign, desquamation on the site of cervical lymphadenopathy, as a new finding."}, {"id": "First_Aid_Step2_953", "title": "First_Aid_Step2", "score": 0.012711716430941156, "content": "Rubella Rubella virus Prodrome: Asymptomatic or tender, generalized lymphadenopathy. Rash: Presents with an erythematous, tender maculopapular rash that also starts on the face and spreads distally. In contrast to measles, children with rubella often have only a low-grade fever and do not appear as ill. Polyarthritis may be seen in adolescents. Encephalitis, thrombocytopenia (a rare complication of postnatal infection). Congenital infection is associated with congenital anomalies. Roseola infantum HHV-6 Prodrome: Acute onset of high fever (> 40°C); no other symptoms for 3–4 days. Rash: A maculopapular rash appears as fever breaks (begins on the trunk and quickly spreads to the face and extremities) and often lasts < 24 hours. Febrile seizures may occur as a result of rapid fever onset. Varicella Varicella-zoster virus (VZV) Prodrome: Mild fever, anorexia, and malaise precede the rash by 24 hours. Rash: Generalized, pruritic, “teardrop” vesicular periphery; lesions are often at"}, {"id": "article-28741_8", "title": "Scarlet Fever -- History and Physical", "score": 0.012658680493473986, "content": "Typically, scarlet fever is associated with acute pharyngitis. As a result, fever, sore throat, pain with swallowing, and cervical adenopathy is present. If there is no pharyngitis, the source of infection can be a wound or burn which is infected with GAS. The two vectors of infection can both cause scarlet fever and are not distinguishable from one another. The rash itself is a blanching, papular rash. It is distinguished from the macular rash found an allergic reaction by its insidious emergence and lack of confluence of the lesions. This lack of confluence is the primary reason it feels like sandpaper. Also of note, there are no vesicles or pustules present. Vesicles are more associated with the “dew on a rose petal” appearance of chickenpox in its initial stages. Pustules are more indicative of a local infection such as impetigo or erysipelas. The rash develops within 2 to 3 days after infection but can be delayed up to 7 days. The trunk, underarms, and groin are affected first, and then it spreads to the extremities. Usually, the palms and soles are spared. The circumoral area is also spared, making it pallor-like. The “strawberry tongue” begins with a white coating of the tongue with hyperplastic papillae. As the white coating resolves, the papules remain, giving the appearance of a strawberry. Pastia lines are found in the folds of the skin such as the neck, antecubital fossa, and groin. This appears as a linear accumulation of papules around pressure points. After the initial rash begins to resolve, a period of desquamation can occur and last up to two weeks in some cases."}, {"id": "wiki20220301en033_29237", "title": "Kawasaki disease", "score": 0.01245511030040472, "content": "The course of the disease can be divided into three clinical phases. The acute febrile phase, which usually lasts for one to two weeks, is characterized by fever, conjunctival injection, erythema of the oral mucosa, erythema and swelling of the hands and feet, rash, cervical adenopathy, aseptic meningitis, diarrhea, and hepatic dysfunction. Myocarditis is common during this time, and a pericardial effusion may be present. Coronary arteritis may be present, but aneurysms are generally not yet visible by echocardiography. The subacute phase begins when fever, rash, and lymphadenopathy resolve at about one to two weeks after the onset of fever, but irritability, anorexia, and conjunctival injection persist. Desquamation of the fingers and toes and thrombocytosis are seen during this stage, which generally lasts until about four weeks after the onset of fever. Coronary artery aneurysms usually develop during this time, and the risk for sudden death is highest."}, {"id": "article-83816_10", "title": "Rubeola (Measles) -- History and Physical", "score": 0.012015221017514597, "content": "The clinical picture of measles can be divided into three stages:  prodromal, eruptive, and convalescent and should be suspected in patients with the classic triad of the three “Cs”:  cough, conjunctivitis, and coryza. The primary or prodromal phase lasts four to six days and is characterized by the presence of high fever, malaise, coryza, conjunctivitis, palpebral edema, and dry cough.  Most cases show the characteristic Koplik spots of the disease, located in the buccal mucosa at the height of the second molar, and appear two to three days before the rash and disappear on the third day. The second phase, the eruptive, is characterized by the appearance of a maculopapular rash, initially fine that subsequently becomes confluent. The rash begins behind the auricle and along the hair implantation line, and extends downward to the face, trunk, and extremities. The third phase or convalescence occurs after three to four days when the rash begins to disappear, in the same order in which it appeared, leaving brown spots and producing a thin peeling of the skin. The fever disappears two to three days after the rash begins, as does the general malaise."}, {"id": "wiki20220301en004_55557", "title": "Scarlet fever", "score": 0.011755432946740231, "content": "Drug eruption: These are potential side effects of taking certain drugs such as penicillin. The reddened maculopapular rash which results can be itchy and be accompanied by a fever. Kawasaki disease: Children with this disease also present a strawberry tongue and undergo a desquamative process on their palms and soles. However, these children tend to be younger than 5 years old, their fever lasts longer (at least five days), and they have additional clinical criteria (including signs such as conjunctival redness and cracked lips), which can help distinguish this from scarlet fever. Toxic shock syndrome: Both streptococcal and staphylococcal bacteria can cause this syndrome. Clinical manifestations include diffuse rash and desquamation of the palms and soles. It can be distinguished from scarlet fever by low blood pressure, lack of sandpaper texture for the rash, and multi-organ system involvement."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 446]], "word_ranges": [[0, 75]], "text": "In this patient, he presents with a granulomatosis with polyangiitis formerly known as Wegener's granulomatosis. In the clinical case we are told about the otorhinolaryngologic involvement that is present in 92% of patients. At the pulmonary level, we were told that mucus with a clot was present in 85% of the patients. Also at the end of the case, they comment us the urinalysis with a glomerulonephritis that is present in 77% of the patients."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "In this patient, he presents with a granulomatosis with polyangiitis formerly known as Wegener's granulomatosis. In the clinical case we are told about the otorhinolaryngologic involvement that is present in 92% of patients. At the pulmonary level, we were told that mucus with a clot was present in 85% of the patients. Also at the end of the case, they comment us the urinalysis with a glomerulonephritis that is present in 77% of the patients.", "full_answer_no_ref": "In this patient, he presents with a granulomatosis with polyangiitis formerly known as Wegener's granulomatosis. In the clinical case we are told about the otorhinolaryngologic involvement that is present in 92% of patients. At the pulmonary level, we were told that mucus with a clot was present in 85% of the patients. Also at the end of the case, they comment us the urinalysis with a glomerulonephritis that is present in 77% of the patients.", "full_question": "A 67-year-old man presents with 3 months of asthenia and febrile fever, with nasal obstruction and mucus emission with some clots in the last month. In the last few days she noticed pain in the right eye and asymmetry with respect to the contralateral eye. Physical examination reveals proptosis of the right eyeball and inspection of the nostrils reveals an erythematous mucosa with serohematic crusts. The rest of the examination was normal. Blood tests (hemogram, renal and hepatic function) are normal, except for an ESR of 65 mm/h; urinalysis shows microhematuria and proteinuria of 520 mg/24h. What is the most probable initial diagnosis?", "id": 598, "lang": "en", "options": {"1": "Eosinophilic granulomatosis with polyangiitis.", "2": "Microscopic polyangiitis.", "3": "Granulomatosis with polyangiitis.", "4": "Polyarteritis nodosa.", "5": NaN}, "question_id_specific": 181, "type": "RHEUMATOLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en249_18855", "title": "Systemic vasculitis", "score": 0.019417475728155338, "content": "Medium vessel vasculitis These conditions are sometimes considered together with the small vessel vasculitides. Polyarteritis nodosa (PAN). Systemic necrotizing vasculitis and aneurysm formation affecting both medium and small arteries. If only small vessels are affected, it is called microscopic polyangiitis, although it is more associated with granulomatosis with polyangiitis than to classic PAN. At least 3 out of 10 criteria yields sensitivity and specificity of 82 and 87%: Unexplained weight loss > 4 kg Livedo reticularis Testicular pain Myalgias, weakness Abdominal pain, diarrhea, and GI bleeding Mononeuropathy or polyneuropathy New onset diastolic blood pressure > 90 mmHg Elevated serum blood urea nitrogen (> 40 mg/dL) or serum creatinine (> 1.5 mg/dL) Hepatitis B infection Arteriographic abnormalities Arterial biopsy showing polymorphonuclear cells"}, {"id": "pubmed23n1147_6293", "title": "Eosinophilic granulomatosis with polyangiitis as a rare cause of the syndrome of inappropriate antidiuretic hormone secretion.", "score": 0.017629698664181424, "content": "Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare multisystem necrotizing vasculitis that involves small- to medium-sized blood vessels. We report a rare case of syndrome of the inappropriate antidiuretic hormone (ADH) secretion (SIADH) secondary to EGPA. A 53-year-old man applied with complaints of pain in the large joints and morning stiffness in knee for 2 months. The patient had the history of impaired fasting glucose, asthma, nasal polyps, and urticaria. Physical examination revealed intrinsic muscle atrophy and weakness in the right hand. Peripheral eosinophil count was 9.78 × 109/L (0.02-0.5), erythrocyte sedimentation rate 39 mm/h (0-20), and C-reactive protein 5.77 mg/dL (0-0.5). Migratory ground-glass pulmonary opacities had been reported in previous chest computed tomography scans. Echocardiography revealed findings compatible with eosinophilic involvement. Electroneuromyographic evaluation showed acute distal axonal neuropathy of right ulnar nerve. EGPA was considered. Oral methylprednisolone treatment was initiated. Intravenous immunoglobulin (IVIG) and cyclophosphamide treatment and gradual tapering of oral steroids were planned. In 24-h urine analysis, sodium was 387 mEq, creatinine was 1156 mg, and volume was 3000 mL. When his medical records were investigated, it was observed that hyponatremia was present for nearly 2 years. While serum osmolality was 270, urine osmolality was 604 mOsm/kg H<sub2</subO. So, SIADH diagnosis was made. Fluid intake was restricted. Although the patient's sodium level did not return to normal, it rose up to 130 mEq/L. After second cycle of EGPA treatment (cyclophosphamide and IVIG), serum sodium was normal. There is only four other documented cases of SIADH associated with EGPA. We hypothesized that blood supply to the hypothalamus and/or posterior hypophysis might be affected from EGPA vasculitis. Here, in this case, with effective treatment of EGPA, SIADH was resolved which implies a causality between two conditions."}, {"id": "pubmed23n1047_6840", "title": "Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).", "score": 0.015639980824544583, "content": "Churg-Strauss syndrome, Eosinophilic granulomatosis with polyangiitis (EGPA), is a systemic vasculitis that affects small- to medium-sized vessels. It is rare and part of the Anti-neutrophil cytoplasm antibody-associated vasculitis (ANCA) group. We present a 37-year-old man, with a previous history of asthma, that was sent to the ED due to 2 weeks of productive cough, occasional dyspnea on exertion, fever (one week), asthenia, and anorexia. Upon physical examination, he was subfebrile and tachycardic. He had leukocytosis (17.00 x10^9/L) and eosinophilia of 20.0 % (3.4 X10^9/L), creatinine level of 1.5 mg/dL, subtle elevation on liver function tests and CRP of 10.82mg/dL. On Chest X-Ray, there was infiltrate on the right pulmonary base. Due to a strong suspicion of EGPA, he was started on 80mg of prednisolone from admission. ANCA MPO was positive, with the remaining auto-immune study negative. He underwent Thorax CT (under corticotherapy) without relevant changes, as well as bronchoalveolar lavage, without macroscopic signs of alveolar hemorrhage. Because of active urinary sediment, nephrotic proteinuria (6.5g/24h), and acute renal failure he underwent a renal biopsy, which revealed pauci-immune crescentic glomerulonephritis, with predominantly acute findings (in the context of ANCA-MPO Vasculitis - EGPA). After the biopsy, he received three 1g methylprednisolone pulses and was started on Cyclophosphamide. He remained asymptomatic and renal function was restored. This case highlights the importance of integrating all findings in one clinical scenario to prevent a more complex disease diagnosis, with a specific treatment, from being missed."}, {"id": "pubmed23n0905_20279", "title": "Granulomatosis with polyangiitis: seeing the diagnosis.", "score": 0.015606801770323782, "content": "A 41-year-old woman presented to her primary doctor with nausea, back pain and lower extremity oedema. Initial labs showed elevated serum creatinine and white blood cell count (WBC), which her doctor attributed to ibuprofen use and a recent upper respiratory infection. Five days later, she presented to the eye clinic with eye pain, redness and blurred vision. She was diagnosed with iritis, conjunctivitis and keratitis. The inflammatory eye disease with decreased renal function prompted the ophthalmologist to initiate systemic autoimmune and infectious disease work-up. Before laboratory testing was complete, she developed severe haemoptysis. Diagnosis of granulomatosis with polyangiitis (GPA) was confirmed using blood testing, radiological imaging and kidney biopsy. She received plasmapheresis, then cyclophosphamide and prednisone with good effect. This case highlights the need to consider GPA in the differential when patients present with inflammatory eye disease with decreased renal function and the need for multispecialty collaboration including ophthalmologists in the diagnosis of GPA."}, {"id": "wiki20220301en249_18865", "title": "Systemic vasculitis", "score": 0.014520902700702921, "content": "Lab tests. Basic lab tests may include a complete blood count, chemiestries (look for creatinine), creatine phosphokinase level, liver function tests, erythrocyte sedimentation rate, hepatitis serologies, urinalysis, chest X-ray, and an electrocardiogram. Additional, more specific tests include: Antinuclear antibody test can detect an underlying connective tissue disorder, especially lupus erythematosus Complement levels that are low can suggest mixed cryoglobulinemia, hepatitis C infection, and lupus erythematosus], but not most other vasculitides. Antineutrophil cytoplasmic antibody may suggest granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, or drug-induced vasculitis, but is not diagnostic. Electromyography. It is useful if a systemic vasculitis is suspected and neuromuscular symptoms are present."}, {"id": "pubmed23n1052_5567", "title": "Granulomatosis with polyangiitis-associated ischemic optic neuropathy in a previously healthy 50-year-old female.", "score": 0.014459087892291646, "content": "To describe a case of anterior ischemic optic neuropathy as a presenting sign of granulomatosis with polyangiitis. A previously healthy 50-year-old female developed right eye, then left eye, redness and pruritis and was diagnosed with allergic versus viral conjunctivitis. Five days later, she noted an acute decline in vision in the right eye, corresponding with a decrease on Snellen testing from 20/30 to 20/100 with correction. She was noted to have a right relative afferent pupillary defect, 2+ pallid disc edema, and OCT (Spectralis, Heidelberg Engineering, Carlsbad, CA) findings of significant retinal nerve fiber thickening. Review of systems revealed a three-month history fatigue, right-sided headaches, jaw claudication, bronchitis, cough without hemoptysis, and epistaxis, as well as interval development of a petechial rash across her body, migratory polyarthralgias, fevers, and tachycardia. ESR and CRP were markedly elevated, and the patient was admitted to the hospital for a systemic vasculitis workup. She was started on IV methylprednisolone. Her vision improved dramatically with steroids, measuring 20/50 with correction in the right eye after 24 hours and returning to baseline after five days. An extensive workup including imaging, bloodwork, and biopsies led to a diagnosis of granulomatosis with polyangiitis, with PR3-positive ANCA. Ocular findings, including anterior ischemic optic neuropathy, may be the presenting signs for patients with granulomatosis with polyangiitis. Prompt recognition and treatment with high-dose steroids and immunomodulatory therapy is important for visual recovery. Prompt recognition of potential vasculitis-related vision loss can lead to timely initiation of vision-saving treatment."}, {"id": "pubmed23n0769_6070", "title": "Vasculitis-like hemorrhagic retinal angiopathy in Wegener's granulomatosis.", "score": 0.013844974609562135, "content": "Granulomatosis with polyangiitis, also known as Wegener's granulomatosis, is a chronic systemic inflammatory disease that can also involve the eyes. We report a case of massive retinal and preretinal hemorrhages with perivascular changes as the initial signs in granulomatosis with polyangiitis (Wegener's granulomatosis). A 39-year-old Caucasian male presented with blurred vision in his right eye, myalgia and arthralgia, recurrent nose bleeds and anosmia. Fundus image of his right eye showed massive retinal hemorrhages and vasculitis-like angiopathy, although no fluorescein extravasation was present in fluorescein angiography. Laboratory investigations revealed an inflammation with increased C-reactive protein, elevated erythrocyte sedimentation rate and neutrophil count. Tests for antineutrophil cytoplasmic antibodies (ANCA) were positive for c-ANCA (cytoplasmatic ANCA) and PR3-ANCA (proteinase 3-ANCA). Renal biopsy demonstrated a focal segmental necrotizing glomerulonephritis. Granulomatosis with polyangiitis (Wegener's granulomatosis) was diagnosed and a combined systemic therapy of cyclophosphamide and corticosteroids was initiated. During 3 months of follow-up, complete resorption of retinal hemorrhages was seen and general complaints as well as visual acuity improved during therapy. Vasculitis-like retinal changes can occur in Wegener's granulomatosis. Despite massive retinal and preretinal hemorrhages that cause visual impairment, immunosuppressive therapy can improve ocular symptoms."}, {"id": "wiki20220301en035_34633", "title": "Polyarteritis nodosa", "score": 0.013646308451069827, "content": "Diagnosis No specific lab tests exist for diagnosing polyarteritis nodosa. Diagnosis is generally based on the physical examination and a few laboratory studies that help confirm the diagnosis: CBC (may demonstrate an elevated white blood count) ESR (elevated) Perinuclear pattern of antineutrophil cytoplasmic antibodies (p-ANCA) - not associated with \"classic\" polyarteritis nodosa, but is present in a form of the disease affecting smaller blood vessels, known as microscopic polyangiitis or leukocytoclastic angiitis Tissue biopsy (reveals inflammation in small arteries, called arteritis) Elevated C-reactive protein"}, {"id": "wiki20220301en058_69678", "title": "Microscopic polyangiitis", "score": 0.013322120917057626, "content": "Diagnosis Laboratory tests may reveal an increased sedimentation rate, elevated CRP, anemia and elevated creatinine due to kidney impairment. An important diagnostic test is the presence of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) with myeloperoxidase specificity (a constituent of neutrophil granules), and protein and red blood cells in the urine. In patients with neuropathy, electromyography may reveal a sensorimotor peripheral neuropathy. Differential diagnosis The signs and symptoms of microscopic polyangiitis may resemble those of granulomatosis with polyangiitis (GPA) (another form of small-vessel vasculitis) but typically lacks the significant upper respiratory tract involvement (e.g., sinusitis) frequently seen in people affected by GPA."}, {"id": "pubmed23n0824_15886", "title": "[Disseminated histoplamosis in adolescent mimicking granulomatosis with polyangiitis].", "score": 0.012923203963666391, "content": "Systemic histoplasmosis is an invasive fungal infection that may mimic primary vasculitis, particularly granulomatosis with polyangiitis (GPA), and was rarely described in adult patients. We reported an immunocompetent patient with disseminated histoplasmosis mimicking GPA who fulfilled European League Against Rheumatism (EULAR)/Pediatric Rheumatology International Trials Organisation (PRINTO)/Pediatric Rheumatology European Society (PRES) validated classification criteria. A 6-year old boy presented acute migratory polyarthritis with spontaneous improvement, sinus inflammation, fever, headache and abdominal pain. Serologic test for hepatitis, cytomegalovirus, human immunodeficiency virus, Epstein-Barr virus, toxoplasmosis, dengue virus and antistreptolysin O were all negative. Magnetic resonance imaging (MRI) showed moderate ascites in pelvis and pansinusitis. Antineutrophil cytoplasmic antibodies (c-ANCA) were positive. He had spontaneous remission of the symptoms including fever. At the age of 11 years and 11 months, he had sinusitis, pneumonia and epididymitis. A month later, he was hospitalized and MRI showed left eye proptosis. Cerebrospinal fluid was normal and indirect tests of fungi were negative. Two months later, he had lumbar pain and computer tomography showed a mass in the right kidney and pulmonary nodule in the right lung. He fulfilled EULAR/PRINTO/PRES criteria for GPA, however the renal biopsy showed a focal granulomatous interstitial nephritis with yeast fungal cells compatible with Histoplasma sp. He was treated with liposomal amphotericin B and itraconazole with improvement of signs and symptoms. We reported a progressive disseminated histoplasmosis case mimicking GPA. Histoplasmosis infection should be considered in immunocompetent subjects with uncommon clinical manifestations, such as arthritis, nephritis and epididymitis."}, {"id": "pubmed23n0819_7243", "title": "The role of conjunctival biopsy in the diagnosis of granulomatosis with polyangiitis.", "score": 0.012917247601082588, "content": "The purpose of this study is to describe a patient who was diagnosed with granulomatosis with polyangiitis based on conjunctival biopsy. This study is a case report and review of the literature. A 48-year-old Caucasian woman presented with a 2-week history of a left eye peripheral corneal ulcer with adjacent conjunctivitis and a 4-month history of a non-resolving productive cough. Given her elevated serum perinuclear antineutrophil cytoplasmic antibody (P-ANCA) and erythrocyte sedimentation rate (ESR) levels as well as a chest computed topography (CT) that showed bilateral patchy infiltrates, suspicion of limited granulomatosis with polyangiitis with lung and ocular involvement was high. Because bronchoalveolar lavage was nondiagnostic for granulomatous disease, conjunctival biopsy was initially attempted in order to avoid a more invasive lung biopsy. The conjunctival biopsy revealed mixed subacute inflammatory mediators and vasculitis consistent with granulomatosis with polyangiitis. Conjunctival biopsy may be a valuable, minimally invasive method for diagnosing systemic granulomatosis with polyangiitis."}, {"id": "pubmed23n0051_17661", "title": "[Spontaneous kidney rupture as an early complication of Wegener's granulomatosis].", "score": 0.011844687412391365, "content": "A 51-year-old woman had been suffering from blood-stained purulent sinusitis and antibiotic-resistant bouts of fever for 4 months. She had microhematuria and serological evidence of inflammation (erythrocyte sedimentation rate [ESR] 92/135 mm, C-reactive protein 5.0 mg/dl). When she was admitted to hospital suspected of having postinfectious glomerulonephritis she complained of spontaneous colic-like pains in the left flank. Within one day the haemoglobin concentration fell from 10 to 6.5 g/dl. Ultrasound and computed tomography demonstrated a large space-occupying lesion around the left kidney. At operation this was found to be a rupture of the kidney with perirenal bleeding which was treated without removing the kidney. No biopsy was taken, but serological tests showed antineutrophil cytoplasmatic antibodies (cANCA), indicating Wegener's granulomatosis as the cause of the compensated renal insufficiency and spontaneous renal rupture. Under immunosuppressive treatment the inflammatory signs (ESR 18/44 mm), fever, chronic maxillary sinusitis, raised serum creatinine concentration and the ANCA titre all regressed, while proteinuria of about 4 g/24 h persisted. There was no recurrence during a follow-up period of 15 months. Serological signs of marked inflammatory activity, urinary sediments of nephritis and spontaneous retroperitoneal bleeding should suggest that, in addition to lupus erythematodes and panarteritis nodosa, Wegener's granulomatosis be included in the differential diagnosis."}, {"id": "wiki20220301en431_14401", "title": "Palpable purpura", "score": 0.0116881743177491, "content": "Palpable purpura is a condition where purpura, which constitutes visible non-blanching hemorrhages, are raised and able to be touched or felt upon palpation. It indicates some sort of vasculitis secondary to a serious disease. Causes Rocky mountain spotted fever Acute meningococcemia Disseminated gonococcal infection Ecthyma gangrenosum Henoch–Schönlein purpura Eosinophilic granulomatosis with polyangiitis Polyarteritis nodosa Leucocytoclastic vasculitis Microscopic polyangiitis Mixed essential cryoglobulinemia Subacute bacterial endocarditis Diagnosis Identification of underlying cause. Treatment Treat the underlying disease. References Further reading Vascular-related cutaneous conditions"}, {"id": "pubmed23n1116_18712", "title": "Optic neuropathy secondary to granulomatosis with polyangiitis in a patient with Graves' disease: a case report.", "score": 0.011417513304305758, "content": "Dysthyroid optic neuropathy is the most commonly suspected diagnosis of optic neuropathy in Graves' patients; however, other causes need to be ruled out. We present a unique case of optic neuropathy secondary to hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be antithyroid drug related. A 79-year-old Japanese male presented with acute visual loss in the left eye. He had a 24-year history of Graves' disease and was taking methimazole. Best-corrected visual acuity was 0.8 in the right eye and light perception in the left eye, and relative afferent pupillary defect in the left eye was seen. Ocular movement was normal, and there were no findings explaining visual loss in intermediate optic media and fundus in the left eye. Contrast-enhanced magnetic resonance imaging demonstrated thickened dura mater. Tests for myeloperoxidase-antineutrophil cytoplasmic antibody, proteinuria, and hematuria were positive; pulmonary nodule lesions and a blood clot in the left lower leg were also found. After excluding the presence of diseases that could lead to hypertrophic pachymeningitis, we diagnosed optic neuropathy due to hypertrophic pachymeningitis with granulomatosis with polyangiitis-a subtype of antineutrophil cytoplasmic antibody-associated vasculitis. Since he had history of using methimazole, antineutrophil cytoplasmic antibody-associated vasculitis was considered as drug related. We started high-dosage steroid pulse therapy followed by 1 mg/kg body weight daily of oral prednisolone, and subsequently tapered. Methimazole was stopped. Best-corrected visual acuity recovered to 0.9, 2 weeks after starting treatment. Though myeloperoxidase-antineutrophil cytoplasmic antibody remained negative, the symptom relapsed 6 months after treatment initiation. We gave a second high-dose steroid pulse therapy followed by prednisolone tapered together with methotrexate. Remission remained, and using 4 mg/week methotrexate without prednisolone, myeloperoxidase-antineutrophil cytoplasmic antibody was kept within the normal limit until now, 4 years after onset. We present a case of optic neuropathy with hypertrophic pachymeningitis related to antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be drug related. The patient had good visual recovery after quitting the drug and receiving immunosuppressive therapy with systemic steroids. Hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis related to antithyroid drugs should be considered as a differential diagnosis for optic neuropathy in Graves' patients in whom optic nerve compression is not obvious."}, {"id": "wiki20220301en035_34638", "title": "Polyarteritis nodosa", "score": 0.011363636363636364, "content": "Differential diagnosis Polyarteritis nodosa rarely affects the blood vessels of the lungs and this feature can help to differentiate it from other vasculitides that may have similar signs and symptoms (e.g., granulomatosis with polyangiitis or microscopic polyangiitis). Treatment Treatment involves medications to suppress the immune system, including prednisone and cyclophosphamide. When present, underlying hepatitis B virus infection should be immediately treated. In some cases, methotrexate or leflunomide may be helpful. Some patients have entered a remission phase when a four-dose infusion of rituximab is used before the leflunomide treatment is begun. Therapy results in remissions or cures in 90% of cases. Untreated, the disease is fatal in most cases. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. A fatal course usually involves gastrointestinal bleeding, infection, myocardial infarction, and/or kidney failure."}, {"id": "wiki20220301en027_70641", "title": "Granulomatosis with polyangiitis", "score": 0.01069169176179471, "content": "Diagnosis Granulomatosis with polyangiitis is usually suspected only when a person has had unexplained symptoms for a long period of time. Determination of anti-neutrophil cytoplasmic antibodies (ANCAs) can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. More than 90% of people who have GPA test positive for ANCA. Cytoplasmic-staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with GPA. Involvement of the ears, nose, and throat is more common in granulomatosis with polyangiitis than in the similar condition microscopic polyangiitis."}, {"id": "pubmed23n0969_6095", "title": "Severe ophthalmic manifestation in pituitary-involved granulomatosis with polyangiitis: a case report and literature review.", "score": 0.010645828024576007, "content": "Granulomatosis with polyangiitis (GPA), a necrotizing granulomatous disease, very rarely involves the central nervous system (CNS), particularly the pituitary. Delayed treatment may cause permanent bilateral blindness. We report an isolated case of pituitary GPA that manifested as a progressive bilateral temporal visual field (VF) defect and was diagnosed via pituitary biopsy. Additionally, we review ocular, chiasmal and cranial nerve involvement in pituitary GPA. A 20-year-old Chinese man was referred for repeated fever, sudden headache, diplopia with a bilateral best-corrected visual acuity (BCVA) of 10/20, ptosis in both eyes and restricted abduction on the right side. VF tests showed bitemporal hemianopsia. Laboratory tests revealed hypothyroidism and were negative for autoimmune markers. Enhanced magnetic resonance imaging (MRI) showed pituitary enlargement. The diagnosis was lymphocytic pituitaritis. After intravenous (IV) dexamethasone treatment, full recovery occurred within 2 months. Two years later, the patient was readmitted for headache recurrence. With oral prednisone, the visual acuity in his right eye rapidly decreased to hand motion (HM) within one month. Enhanced MRI showed pituitary enlargement and a new, invasive suprasellar CNS lesion. All infection- and autoimmune-related tests were negative. The visual acuity in his right and left eye decreased to no light perception (NLP) after 6 days and 2 weeks, respectively. The biopsy results suggested GPA. After IV methylprednisolone treatment, complete remission of the symptoms occurred and was confirmed by MRI. The 15-month follow-up showed no signs of recurrence. GPA typically affects the respiratory tract, lungs and kidneys. To date, 50 cases with pituitary involvement have been reported. Chiasmal and cranial nerve involvement leading to visual acuity impairment are common. We found 2 cases with severe visual loss resembling our case and discuss certain similarities."}, {"id": "wiki20220301en032_35767", "title": "Eosinophilic granulomatosis with polyangiitis", "score": 0.010275676751592357, "content": "Risk stratification The French Vasculitis Study Group has developed a five-point system (\"five-factor score\") that predicts the risk of death in Churg–Strauss syndrome using clinical presentations. These factors are: Reduced renal function (creatinine >1.58 mg/dl or 140 μmol/l) Proteinuria (>1 g/24h) Gastrointestinal hemorrhage, infarction, or pancreatitis Involvement of the central nervous system Cardiomyopathy Having none of these factors indicates milder case, with a five-year mortality rate of 11.9%. The presence of one factor indicates severe disease, with a five-year mortality rate of 26%, and three or more indicate very severe disease: 46% five-year mortality rate."}, {"id": "wiki20220301en249_1086", "title": "Pauci-immune", "score": 0.010096237970253719, "content": "In the setting of systemic vasculitis as described above, proliferative nephritis is associated with antineutrophil cytoplasmic antibodies (ANCA). Because of this, an ANCA test should always follow a negative immunofluorescence result to have the highest accuracy for confirming pauci-immune vasculitis-driven proliferative nephritis. Some cases of pauci-immune proliferative nephritis have no explanation and are thus deemed \"idiopathic.\" Peak incidences in 50- to 60-year-olds symptoms include intermittent fever / weight loss / shortness of breath / joint pain. See also Systemic vasculitis#Pauci-immune Goodpasture Syndrome and Poststrep Glomerulonephritis Microscopic polyangiitis, Eosinophilic granulomatosis with polyangiitis or Granulomatosis with polyangiitis References External links wikt:paucity Vascular-related cutaneous conditions"}, {"id": "article-76416_14", "title": "Granulomatosis With Polyangiitis -- History and Physical -- Organ System Involvement", "score": 0.010012562578516116, "content": "Upon presentation, renal involvement is noted in only 10%-20%, but glomerulonephritis eventually develops in 80% of patients within two years of disease onset. The most common manifestation is rapidly progressive crescentic glomerulonephritis leading to chronic kidney disease or end-stage renal disease. Eye involvement:"}, {"id": "pubmed23n0056_3942", "title": "[A case of polyarteritis nodosa with bilateral hilar lymphadenopathy].", "score": 0.009900990099009901, "content": "A 55-year-old male was admitted with non productive cough and fever which had continued for 6 weeks. The patient had symptoms of peripheral neuralgia. Chest X-ray revealed bilateral hilar lymphadenopathy (BHL) and reticular shadows in both lung fields. Other laboratory abnormalities included hematuria, RBC cast, high BUN, leukocytosis and thrombocytosis. Destruction of the internal membrane of arterioles was observed in a livedo reticularis on the right lower extremity. Renal angiography showed irregularity in the diameter, discontinuation and narrowing of peripheral arteries of both kidneys. These findings suggested the existence of \"angiitis\". These data were compatible with the diagnosis of polyarteritis nodosa (PN). Prednisolone (60 mg/day) administration resulted in the improvement of his symptoms and laboratory findings. A case of PN with lymph node swelling has been reported, however PN with BHL has not yet been reported. This is the first report of PN with BHL."}, {"id": "pubmed23n0302_21179", "title": "Classical polyarteritis nodosa and microscopic polyarteritis with medium vessel involvement--a comparison of the clinical and laboratory features.", "score": 0.00980392156862745, "content": "Microscopic polyarteritis may involve medium-sized and small blood vessels as well as arterioles, venules and capillaries. We have compared the clinical and laboratory features in patients with microscopic polyarteritis and medium vessel involvement, with the features found in patients with polyarteritis nodosa affecting medium vessels alone. In a 9-year period, 21 patients presented to our hospital with a form of polyarteritis. Seven had microscopic polyarteritis demonstrated histologically (6/7, 86%) and associated with dysmorphic urinary red cells (7/7, 100%), as well as medium vessel vasculitis demonstrated histologically (7/7) or by angiography (1/7, 14%). Five patients had polyarteritis nodosa with medium vessel vasculitis demonstrated histologically (3/5, 60%) or by angiography (2/5, 40%); and no evidence of a glomerular vasculitis on biopsy (2/7, 29%) or in the urinary sediment (0/7, 0%). The remaining 9 patients had microscopic polyarteritis but medium vessel involvement was not excluded by angiography. All patients with microscopic polyarteritis and medium vessel involvement had glomerular hematuria (&gt; 100,000 glomerular RBC/ml), proteinuria &gt; 0.5 g/24 hours), and an elevated serum creatinine (0.166 to 0.811 mmol/l). Other symptoms included fever (6/7, 86%), night sweats (5/7, 71%), gastrointestinal bleeding (4/7, 57%), proximal myopathy (3/7, 43%) and peripheral neuropathy (3/7, 43%). One patient (1/7, 14%) had hypertension. Anemia (6/7, 86%), a raised ESR (6/7, 86%), thrombocytosis (6/7, 86%), hypoalbuminemia (6/7, 86%) and abnormal liver function tests (6/7, 86%) were common. Two patients (29%) had an eosinophilia. All 5 individuals who were tested for ANCA were positive (2cANCA, 2pANCA and one pattern not described). In contrast, in patients with polyarteritis nodosa and medium vessel involvement alone, an elevated ESR was common (4/5, 80%) but fever (1/5, 20%), night sweats (0/5, 0%), proximal myopathy (1/5, 20%) and peripheral neuropathy (1/5, 20%) were seen infrequently; hypertension (1/5, 20%) and eosinophilia (1/5, 20%) were also uncommon; and ANCA were not demonstrated (0/3, 0%). Medium-sized vessel involvement is common in patients with microscopic polyarteristis, and these patients are more likely to have renal involvement and systemic symptoms, and be ANCA-positive, than patients with polyarteritis nodosa alone. Gastrointestinal symptoms are often seen in both groups."}, {"id": "wiki20220301en249_18864", "title": "Systemic vasculitis", "score": 0.009740590895182403, "content": "Mononeuritis multiplex. Also known as asymmetric polyneuropathy, in a non-diabetic this is suggestive of vasculitis. Palpable purpura. If patients have this in isolation, it is most likely due to cutaneous leukocytoclastic vasculitis. If the purpura is in combination with systemic organ involvement, it is most likely to be Henoch–Schönlein purpura or microscopic polyangiitis. Pulmonary-renal syndrome. Individuals who are coughing up blood and have kidney involvement are likely to have granulomatosis with polyangiitis, microscopic polyangiitis, or anti-GBM disease (Goodpasture syndrome). Diagnosis A detailed history is important to elicit any recent medications, any risk of hepatitis infection, or any recent diagnosis with a connective tissue disorder such as systemic lupus erythematosus (SLE). A thorough physical exam is needed as usual."}, {"id": "pubmed23n0309_9355", "title": "[Microscopic polyangiitis with eosinophilia--an overlap syndrome or separate disease entity? A case report and review of the literature].", "score": 0.009685286806434969, "content": "Systemic vasculitides are potentially life-threatening diseases. Early and appropriate diagnosis based on case history, clinico-pathological features, and laboratory parameters, such as the presence of anti-neutrophil cytoplasmic antibodies (ANCA), is crucial for starting appropriate and, often, life-saving therapeutic measures. We report a 50-year-old female patient who presented with fever, arthralgias and hemoptysis. Skin signs included disseminated hemorrhagic pustules, ulcerations of oral and genital mucosa, subcutaneous nodules on arms and legs, and a pyoderma gangrenosum-like lesion on the right leg. Laboratory investigations revealed a peripheral eosinophilia and a positive cANCA titer. Histopathologic analysis of various biopsy specimens showed a granulomatous vasculitis in the subcutis, a nongranulomatous vasculitis with massive eosinophil infiltration in the lungs, and a segmental, necrotizing glomerulonephritis in the kidneys. Differential diagnosis included Wegener's granulomatosis, microscopic polyangiitis (MPA) and Churg-Strauss syndrome. MPA was diagnosed based on clinical and histopathological criteria. An interesting feature of this case was marked peripheral and tissue eosinophilia. Therapy consisted of cyclophosphamide and methylprednisolone. The patient went into a long-lasting clinical remission one month after starting therapy."}, {"id": "pubmed23n1079_2973", "title": "Wegener's granulomatosis with orbital involvement: case report and literature review.", "score": 0.009615384615384616, "content": "<bObjective:</b To describe the chronology and the extent of orbital involvement in a case of granulomatosis with polyangiitis. <bMethods:</b Descriptive case report and literature review. <bResults:</b A 45-year-old patient, formerly diagnosed with granulomatosis with polyangiitis due to otorhinolaryngologic manifestations, pulmonary lesions, renal impairment, left knee arthritis and high blood levels of antineutrophil cytoplasmic antibodies, addressed the Ophthalmology Department in November 2020, having the following complaints: left eye mild retro-orbital discomfort, proptosis and epiphora. On examination, Snellen's best corrected visual acuity was 6/ 6 in both eyes. The anterior segment of the left eye displayed significant changes: proptosis, upper lid swelling, ptosis, slightly decreased ocular motility, mild conjunctival hyperemia and chemosis, thinning of sclera in the upper quadrant and mild posterior subcapsular cataract. Left eye funduscopy revealed a slightly elevated optic disc, with indistinct margins in the nasal quadrant. Spectral-domain optical coherence tomography (OCT) of the optic nerve confirmed the clinical findings, illustrating an increase in the retinal nerve fiber layers thickness in the nasal quadrant, with no corresponding visual field defect. The orbit magnetic resonance imaging (MRI) unveiled an intraconal mass surrounding the optic nerve on its entire intra-orbital path, confirming the diagnosis of left orbital granuloma. <bConclusion:</b Considering the relapsing disease and the orbital involvement, the patient is currently a candidate for rituximab, a monoclonal antibody against CD20. <bAbbreviations:</b AAV = ANCA associated vasculitides, ANCA = antineutrophil cytoplasmic antibody, AOM = acute otitis media, BCVA = best corrected visual acuity, CRP = C-reactive protein, CT = computerized tomography, EGPA = eosinophilic granulomatosis with polyangiitis, ENT = otorhinolaryngology/ ear-nose-throat, ESR = erythrocyte sedimentation rate, GPA = granulomatosis with polyangiitis, LE = left eye, MPA = microscopic polyangiitis, MRI = magnetic resonance imaging, OCT = optical coherence tomography, RE = right eye, RNFL = retinal nerve fiber layers, TNF = tumor necrosis factor, WG = Wegener's granulomatosis."}, {"id": "pubmed23n0074_19679", "title": "[Intracranial multiple granuloma preceded by rheumatic disease--a case report].", "score": 0.009615384615384616, "content": "In 1980, a 38-year-old man had remittent fever, swelling and arthralgia of the knee, ankle and wrist joints, as well as visual disturbance due to bilateral iritis. On his admission to our hospital, his laboratory data showed neutrophilia, normocytic normochromic anemia, hepatomegaly, hepatocellular damage, and a strongly positive RA test. All the microbiological examinations were negative. Thirty mg of prednisolone improved his symptoms and abnormal laboratory findings. Due to persistent mild arthralgia, he had continued to take 5-10 mg of prednisolone and analgesics until 1985 when he was readmitted to our hospital. In 1983, he began to complain of a steady pain around his left eye, and he sometimes had double vision. In 1985, he began to complain of decreasing left visual acuity and sensory disturbance in his left face in addition to pain in and around the eye. On his 2nd admission to our hospital, the neurological examination revealed involvement of the 2nd and 3rd cranial nerves and the 1st branch of the 5th cranial nerves of the left side. Laboratory data showed a positive RA test with RAHA titer at 1:320 and IgM at 216 mg/dl, but he had no joint deformities. The computed tomography (CT) of the brain demonstrated a high density mass of his left cavernous sinus extending to the left orbital apex. The prescription of the high dose of prednisolone (100 mg/day) relieved ophthalmic pain and improved visual acuity and neurological involvement within a week. Prednisolone was then gradually decreased to 10 mg. In 1986, he had partial and complex partial seizures.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en011_6030", "title": "Rheumatology", "score": 0.009523809523809525, "content": "Systemic conditions and connective tissue diseases Lupus Ehlers-Danlos syndrome Sjögren's syndrome Scleroderma (systemic sclerosis) Polymyositis Dermatomyositis Polymyalgia rheumatica Mixed connective tissue disease Relapsing polychondritis Adult-onset Still's disease Sarcoidosis Fibromyalgia Myofascial pain syndrome Vasculitis Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis Granulomatosis with polyangiitis Polyarteritis nodosa Henoch–Schönlein purpura Serum sickness Giant cell arteritis, Temporal arteritis Takayasu's arteritis Behçet's disease Kawasaki disease (mucocutaneous lymph node syndrome) Thromboangiitis obliterans Hereditary periodic fever syndromes"}, {"id": "pubmed23n1153_17586", "title": "Conjunctivitis as the important indicator of pediatric granulomatosis with polyangiitis.", "score": 0.009523809523809525, "content": "Granulomatosis with polyangiitis disease is a rare vasculitis characterized by granulomatous inflammation of respiratory tracts and glomerulonephritis along with vasculitis of other organs. In this study, a 14- year-old boy was referred from ophthalmology clinic to the pediatric rheumatology ward due to drug-resistant conjunctivitis. He had a history of chronic rhinorrhea and nighttime coughing, and he was diagnosed with allergic rhinitis. Complete blood count showed leukocytosis and thrombocytosis, and the estimated sedimentation rate was elevated. Laboratory tests showed hematuria, proteinuria, and highly positive antineutrophil cytoplasmic antibody. Moreover, sinus computed tomography demonstrated pansinusitis, and spiral chest computed tomography showed multiple pulmonary nodules in both his lungs. Finally, based on renal biopsy, the patient was confirmed as a case of granulomatosis with polyangiitis. It is notable that acute or chronic conjunctivitis may be a manifestation of rheumatic diseases."}, {"id": "wiki20220301en024_99511", "title": "Vasculitis", "score": 0.009345794392523364, "content": "A small number have been shown to have a genetic basis. These include adenosine deaminase 2 deficiency and haploinsufficiency of A20. According to the size of the vessel affected, vasculitis can be classified into: Large vessel: Takayasu's arteritis, Temporal arteritis Medium vessel: Buerger's disease, Kawasaki disease, Polyarteritis nodosa Small vessel: Behçet's syndrome, Eosinophilic granulomatosis with polyangiitis, Cutaneous vasculitis, granulomatosis with polyangiitis, Henoch–Schönlein purpura, and microscopic polyangiitis. Condition of some disorders have vasculitis as their main feature. The major types are given in the table below: Takayasu's arteritis, polyarteritis nodosa and giant cell arteritis mainly involve arteries and are thus sometimes classed specifically under arteritis."}, {"id": "pubmed23n0368_18062", "title": "Laboratory testing in the evaluation and diagnosis of vasculitis.", "score": 0.009345794392523364, "content": "A multitude of tests are available for the diagnosis and management of the vasculitides. Most of them are nonspecific but provide useful information that, when appropriately used in conjunction with the patient's history and physical examination can be of great assistance in arriving at a final diagnosis. In addition, information gathered may be of great help in monitoring disease activity and clinical response to therapy, in indicating the presence of specific organ system involvement, in monitoring toxicity of medication used, and in assessing prognosis. Serial measurements of acute phase reactants, complete blood cell count with differential, biochemistry profiles, urinalysis, and C3 and C4 levels should be obtained in all patients. Antineutrophil cytoplasmic antibodies (ANCA) determination provides valuable information and is highly specific for the diagnosis of small-vessel vasculitides, particularly Wegener's granulomatosis and microscopic polyangiitis. ANCA levels can be particularly useful to assess disease activity in these disorders. Hepatitis-B and, more importantly, hepatitis-C testing is extremely useful, particularly in the presence of liver involvement and associated risk factors. Angiographic studies may confirm the diagnosis, particularly if there is laboratory and clinical evidence of specific organ involvement. It should be noted, however, that angiography may be normal even when vasculitis is present, or the findings may be nonspecific. A definite diagnosis is provided by a tissue biopsy. This should be performed whenever there is access to clinically affected tissue."}, {"id": "pubmed23n1133_9884", "title": "Eosinophilic granulomatosis with polyangiitis complicated with rapidly progressive glomerulonephritis in a young man who is a healthy cyclist.", "score": 0.009259259259259259, "content": "Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic vasculitis. Rapidly progressive glomerulonephritis (RPGN) is a rare complication of EGPA. We report a case of a 60-year-old man, who is also a skilled cyclist, who was hospitalized to investigate a symptomatology that had arisen over the previous months and worsened in the last few weeks, to the point of limiting normal everyday activities. The physical examination revealed the presence of livedo reticularis of the four limbs, purpura of the lower limbs, arthritis of the ankles, and low-grade fever; the patient showed intense asthenia, loss of appetite, retrosternal heartburn, and a scarcely pharmacologically controlled asthma. He also reported weight loss (about 5 kg in the last 6 months). Rapidly progressing renal failure was observed with hyper-eosinophilia (4.7 thousand/μL eosinophils, 44% of total leukocytes), pulmonary opacities on chest computed tomography (CT), and sinusitis on CT of the facial massif. The search for antibodies directed against neutrophil cytoplasm (ANCA) revealed a high level of pANCA (pANCA ++, ELISA anti-MPO 666 UI/ml), associated with an increment of inflammation indicators. The induction therapy was high-dosage intravenous glucorticoids and cyclophosphamide, to improve the short and long-term prognosis. After 7 months of treatment, the patient reported a considerable improvement of the symptoms, which at that point did not necessitate pharmacological interventions. The eosinophils value was 0 cells/mm³, the inflammation indexes were back to the norm, and the renal function appeared significantly improved."}, {"id": "pubmed23n0297_5457", "title": "[Microscopic polyarteritis].", "score": 0.009259259259259259, "content": "The microscopic polyarteritis is a vasculitis related to necrotizing glomerolunephritis. It always damages at renal and systemic level (a third of the cases presented hemorrhage alveolar). We have showed a case that took place with hemoptysis and renal progressive insufficiency. Among the patient antecedents, we can find arterial hypertension hematuria, rhinitis, epistaxis and artromyalgias. Just before his admittance it showed edemas on lower limbs and eyelids, dysnea, severe hemoptysis, paresthesias and general malaise. The immunologic analysis: Acs. glomerular basal antimembrane: negative, ANCA positive with antimieloperoxidasa specificity. The renal biopsy: focal necrotizing glomerulonephritis with semilunar and negative immunofluorescent. The nasas biopsy: unspecified chronic rhinitis. From the clinic point of view, the patient seemed to have the Wegener granulomatosis apart from the fact that he had hemoptysis which is a rare sign in this cases. However, we could not find any granuloma anatomopatologically, which did not clarify this diagnosis. We diagnosed microscopis Poliarteritis, as a third of the cases presented intrapulmonary haemorrhage. Moreover the renal damage it is identical than in the in Wegener granulomatosis. On the top of that, often we can find p-ANCA, which confirms the diagnosis in 99% of cases. Despite our doubt according to the diagnosis the therapeutical treatment of both illnesses is nowadays identical. This means that we were able to begin a precocious treatment with plasmapheresis, metilprednisolona and ciclofosfamida. After a week treatment there was an evident improvement. Five moth later the illness relapsed."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": true, "char_ranges": [[750, 801]], "word_ranges": [[117, 125]], "text": "An epiretinal membrane usually has a slower course."}, "2": {"exist": true, "char_ranges": [[802, 930]], "word_ranges": [[125, 148]], "text": "In a macular hole, visual loss is more relevant (metamorphopsia may also be reported, but it is less likely in the acute stage)."}, "3": {"exist": true, "char_ranges": [[931, 1088]], "word_ranges": [[148, 174]], "text": "In thrombosis of the central retinal vein, visual loss is more important and does not usually explain metamorphopsia (but may have it, due to macular edema)."}, "4": {"exist": true, "char_ranges": [[222, 480]], "word_ranges": [[37, 78]], "text": "The key to differentiate between the other four is that he had soft drusen in the fundus. That while not a \"diagnosis\" (the question could have been better worded) is a finding related to age-related macular degeneration, or age-related macular degeneration."}, "5": {"exist": true, "char_ranges": [[74, 221]], "word_ranges": [[12, 37]], "text": "We could initially rule out central serous chorioretinopathy because by definition it occurs in young people (up to 55 years of age, more or less)."}}, "full_answer": "Of the five options, four could be compatible with the referred symptoms. We could initially rule out central serous chorioretinopathy because by definition it occurs in young people (up to 55 years of age, more or less). The key to differentiate between the other four is that he had soft drusen in the fundus. That while not a \"diagnosis\" (the question could have been better worded) is a finding related to age-related macular degeneration, or age-related macular degeneration. The dry or atrophic form presents with slowly progressive visual loss. But the wet or exudative form produces acute or rapidly progressive severe visual loss (usually due to subretinal hemorrhage accompanied by metamorphopsia (image distortion due to retinal lifting). An epiretinal membrane usually has a slower course. In a macular hole, visual loss is more relevant (metamorphopsia may also be reported, but it is less likely in the acute stage). In thrombosis of the central retinal vein, visual loss is more important and does not usually explain metamorphopsia (but may have it, due to macular edema).", "full_answer_no_ref": "Of the five options, four could be compatible with the referred symptoms. [HIDDEN] because by definition it occurs in young people (up to 55 years of age, more or less). The key to differentiate between the other four is that he had soft drusen in the fundus. That while not a \"diagnosis\" (the question could have been better worded) is a finding related to age-related macular degeneration, or age-related macular degeneration. The dry or atrophic form presents with slowly progressive visual loss. [HIDDEN] (usually due to subretinal hemorrhage accompanied by metamorphopsia (image distortion due to retinal lifting). An epiretinal membrane usually has a slower course. In a macular hole, visual loss is more relevant (metamorphopsia may also be reported, but it is less likely in the acute stage). In thrombosis of the central retinal vein, visual loss is more important and does not usually explain metamorphopsia (but may have it, due to macular edema).", "full_question": "75-year-old woman diagnosed 3 years ago with soft drusen in the fundus. She reports presenting, since 2 weeks ago, metamorphopsia and significant visual loss in her right eye that prevents her from reading. Indicate the most probable diagnosis:", "id": 133, "lang": "en", "options": {"1": "Macular epiretinal membrane.", "2": "Macular hole.", "3": "Thrombosis of the central retinal vein.", "4": "Senile macular degeneration.", "5": "Central serous chorioretinopathy."}, "question_id_specific": 168, "type": "OPHTHALMOLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0087_4306", "title": "[Macular diseases in the elderly person].", "score": 0.018558295386201563, "content": "Macular diseases in the elderly, such as age-related macular degeneration, idiopathic senile macular hole and epiretinal membrane of the macular area were studied. In 75 normal subjects aged from 20 to 78 years, retinal sensitivity in the central 10 degree visual field were examined using automated static quantitative perimetry. For background luminance of 31.5 asb, a significant reciprocal correlation was demonstrated between individual mean sensitivity and age. The influence of age on the decrease in sensitivity was proved to differ according to different test locations. To enhance contrast, an image processing procedure was applied for fluorescein angiographs of age-related pathologies which resulted in better recognition of age-related RPE pathologies were recognized. The senile disciform macular degeneration (SDMD) study group sponsored by the Ministry of Health and Welfare performed an epidemiological survey to estimate the number of patients with SDMD. The epidemiological estimation was 6,000 to 13,000 patients in the entire Japanese population. 133 eyes of uni- or bi-lateral senile macular degeneration without choroidal neovascularization and 156 opposite eyes of patients with unilateral SDMD were followed-up for choroidal neovascularization development. Choroidal neovascularization development was confirmed in 15 eyes, 5.2%. In 13 of the 15 eyes, choroidal neovascularization was proved to develop through serous RPE detachment. Also, serous drusen were shown to be to predisposed to choroidal neovascularization through serous RPE detachment. Therefore, it was concluded that senile macular degeneration should be classified into the atrophic form, predisciform or intermediate form and disciform form. In the author's previous paper, it was reported that the navel-like lesion would be a macular lesion predisposing to a senile macular hole. 49 opposite eye of patients with one eye affected by a macular hole were follow-up for macular hole development. At the initial examination, the navel-like lesion was observed in 5 of the 49 eyes. During the course of observation, navel-like lesions developed in one of the other 27 eyes with other abnormalities and in 4 of the 17 eyes without any abnormality. Finally, macular holes developed in 11 of the 49 eyes; in 10 eyes with a navel-like lesion and one eye with another abnormality. We found a 17 year old female Japanese monkey with pre-macular holes in both eyes and clinicopathological correlative study was carried out. In her right eye, photoreceptor cell loss at the foveola, circumferential retinal detachment around the area of cell loss, cystoid spaces in the detached retina, and very thin residual tissue covering the foveolar lesion were observed.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0828_16890", "title": "Disappearance of soft drusen and subsequent development of choroidal neovascularization following macular hole surgery: a case report.", "score": 0.01418546365914787, "content": "Drusen are important risk factor for neovascular age-related macular degeneration (AMD) and have a dynamic nature as they can enlarge, newly form, or disappear over time. There have been few reports on drusen regression or choroidal neovascularization (CNV) development after macular hole surgery. We report, to our knowledge, the first case of both drusen regression and subsequent CNV development within 7 months of successful macular hole surgery. A 73-year-old woman presented with a stage 3 full-thickness macular hole and large, confluent soft macular drusen in the right eye and a neovascular age-related macular degeneration (AMD) in the fellow eye. Four months after the successful macular hole surgery, significant regression of drusen was seen, especially in the temporal area to the fovea. Three months later, the patient developed CNV and her best-corrected visual acuity decreased to 20/100, despite further regression of macular drusen. Macular hole patients with macular soft drusen need to be carefully followed up after surgery for possible drusen regression and CNV development."}, {"id": "pubmed23n0744_13003", "title": "Treatment of macular serous neuroretinal detachment in tilted disk syndrome: report of 3 cases.", "score": 0.014109918578830496, "content": "To describe functional and anatomic results obtained by treatment with photodynamic therapy (PDT) or intravitreal bevacizumab (Avastin, Roche) in macular serous retinal detachment associated with tilted disk syndrome. Three eyes of 3 patients with symptomatic macular serous detachment associated with tilted disc syndrome (optic disc with an oblique axis, inferonasal crescent, and inferior staphyloma) were treated. In all patients, best-corrected visual acuity (BCVA) was tested and fluorescein angiography (FA) and optical coherence tomography (OCT) were performed before and about 45 days after treatment. All patients underwent a complete ophthalmologic examination including OCT at least 6 months after treatment. The first patient was treated with one low fluence (300 mW/cm2 for 83 seconds) PDT (6 months follow-up). The second patient was treated with 3 intravitreal injections of bevacizumab 1.25 mg (33 months follow-up) and the third patient was treated with 2 low fluence PDTs at 4 months and, after 1 year, 3 intravitreal injections of bevacizumab 1.25 mg (37 months follow-up). Before treatment, all patients complained of visual loss and metamorphopsia. The OCT showed in the macular area a focal neurosensory detachment with foveal involvement. The FA showed in the macular area multiple focal areas of hyperfluorescence due to pigment epithelium atrophy and in the second and third patient also a hyperfluorescent pinpoint with minimal leakage. After treatment in all eyes, symptoms did not change, BCVA remained stable, and in OCT the foveal neuroretinal detachment was changeless. In FA, no noticeable variation of the hyperfluorescence areas was appreciated. In the second patient, the hyperfluorescent point remained unvaried, and the same occurred in the third patient after the first PDT, while after the second PDT a new leaking dot disappeared. Macular serous retinal detachment was first described in 1998 as an uncommon complication of tilted disc syndrome showing angiographic and OCT features similar to a chronic central serous chorioretinopathy. In contrast to this pathology, in our patients treatment with PDT or intravitreal bevacizumab did not succeed, probably because of a different pathogenesis of macular serous detachment. Further investigations are needed to clarify the proper therapy of this disease."}, {"id": "pubmed23n1024_7657", "title": "Macular hole and serous pigment epithelial detachment in bilateral acquired vitelliform lesions.", "score": 0.013844367015098724, "content": "Acquired vitelliform lesions (AVLs) are associated with age-related macular degeneration and other variable macular disorders. AVLs often lead to outer retinal atrophy, sometimes accompanying a macular hole and choroidal neovascularization. The purpose of this study was to report a rare case with bilateral AVLs, in which one eye had accompanied a macular hole and the second eye a serous pigment epithelial detachment (sPED). A 66-year-old woman complained of bilateral metamorphopsia. AVLs were observed in the right eye and a flat sPED in the left eye. The best-corrected visual acuity (BCVA) was 20/17 in both eyes. Fluorescein angiography revealed local leakage in the right eye and pattern dystrophy-like hypofluorescence in both eyes. The sPED progressed with AVLs in the left eye and was treated with a combination therapy of intravitreal aflibercept, a sub-Tenon's injection of triamcinolone acetonide, and photodynamic therapy (IVA/STTA/PDT), which successfully flattened the sPED and sustained good vision for 4 years. The right eye was treated with intravitreal ranibizumab and tissue plasminogen activator, which enhanced absorption of the vitelliform material. However, 14 months later, a macular hole with typical metamorphopsia formed above a subretinal fibrotic scar at the vitelliruptive stage. Although pars plana vitrectomy closed the macular hole, enlargement of the outer retinal atrophy worsened the BCVA to 20/100. We successfully treated one eye with a sPED with AVLs using the combination therapy of IVA/STTA/PDT, while the second eye with a macular hole secondary to AVLs ultimately developed outer retinal atrophy with visual loss."}, {"id": "wiki20220301en043_34118", "title": "Amsler grid", "score": 0.012491663674139435, "content": "Clinical significance Amsler grid can be used in detecting central visual field defects in following conditions: Age-related macular degeneration: The grid will help detecting the progression of AMD from dry form to wet form. Chance of metamorphopsia is more in wet AMD compared to dry form. Choroidal neovascular membranes: Choroidal neovascular membranes cause scotoma and metamorphopsia. It may be associated with many diseases like macular degeneration, POHS, myopic macular degeneration, trauma etc. Central serous chorioretinopathy: CSCR Causes round or oval scotoma. Macular pucker: Macular pucker also known as an epiretinal membrane cause metamorphopsia and distortions in central field of vision. Cystoid macular edema: Due to macular edema, micropsia may occur. Glaucoma: Amsler grid is useful in detecting central field defects in moderate to severe glaucoma. Macular sparing: Amsler Grid can be used to detect and accurately measure macular sparing. Types"}, {"id": "article-24633_32", "title": "Macular Hole -- Differential Diagnosis", "score": 0.01192746647292102, "content": "Recent advancement in OCT has made it simpler to differentiate a macular hole from other similar types of smaller diameter macular pathologies of old age. From history and physical examination, round central small reddish lesions with dimness of central vision, probable differential diagnosis include: Epiretinal membrane (ERM) with a macular pseudo hole Central foveal dot hemorrhage Lamellar (aborted) macular hole Vitreomacular traction syndrome (VMTS) Foveal drusen Central areolar pigment epitheliopathy Solar retinopathy Small choroidal neovascular membrane involving center Small central serous chorioretinopathy involving center Cystoid macular edema (CME)"}, {"id": "wiki20220301en029_81304", "title": "Macular degeneration", "score": 0.011892029539088363, "content": "Other types There are a few other (rare) kinds of macular degeneration with similar symptoms but unrelated in etiology to Wet or Dry age-related macular degeneration. They are all genetic disorders that may occur in childhood or middle age. Vitelliform macular dystrophy Sorsby's fundus dystrophy is an autosomal dominant, retinal disease characterized by sudden acuity loss resulting from untreatable submacular neovascularisation Stargardt's disease (juvenile macular degeneration, STGD) is an autosomal recessive retinal disorder characterized by juvenile-onset macular dystrophy, alterations of the peripheral retina, and subretinal deposition of lipofuscin-like material. Similar symptoms with a very different etiology and different treatment can be caused by epiretinal membrane or macular pucker or any other condition affecting the macula, such as central serous retinopathy. Notable cases Judi Dench Joan Plowright Peter Sallis June Brown S. Robert Morgan See also"}, {"id": "wiki20220301en067_41616", "title": "Metamorphopsia", "score": 0.011713106295149638, "content": "Treatment and Prognosis Metamorphopsia is a symptom of several common retinal and macular diseases, therefore treating the underlying disorder can improve symptoms. For people who have conditions such as Epiretinal membrane (ERM), Macular Holes and Retinal Detachment, decreased metamorphopsia is associated with an increase in visual acuity. Quantitative evaluation of metamorphopsia is an important step in understanding visual functions of individuals with macular disorders and is an essential tool for physicians in evaluating treatment results. Types Dry (non-exudative, > 80%)—deposition of yellowish extracellular material in and between Bruch's membrane and retinal pigment epithelium (“drusen”) with gradual loss in vision. Wet (exudative, 10–15%)—rapid loss of vision due to bleeding secondary to choroidal neovascularization. Etymology Gk, meta + morphe, form, opsis, sight See also Dysmorphopsia Hallucination References Visual disturbances and blindness"}, {"id": "pubmed23n0791_18770", "title": "Spontaneous closure of a fully developed macular hole in a severely myopic eye.", "score": 0.011612089844397995, "content": "Purpose. Myopic macular holes can be difficult to close with surgery and are frequently associated with retinal detachment. We report on a case of a macular hole in a severely myopic eye that underwent spontaneous closure. Methods. An observational case study. Results. A 55-year-old female was referred to Ophthalmology for a central scotoma and metamorphopsia in the right eye. Visual acuity was 1/20 in both eyes. Fundus examination showed loss of the foveal depression, with a small yellow ring in the center of the fovea in the right eye, and a tilted optic disc and peripapillary staphyloma bilaterally. Spectral domain optical coherence tomography (SD-OCT) revealed a fully developed macular hole with a rim of thickened and slightly elevated retina in the right eye. The patient refused surgery. After 4 years of follow-up, her visual acuity improved to 20/40 in the right eye, and SD-OCT revealed spontaneous sealing of the macular hole without bare retinal pigment epithelium. Conclusions. Myopic macular holes represent a challenge regarding their management, and the prognosis is often poor. "}, {"id": "pubmed23n0325_11826", "title": "Symptomatic age-related macular degeneration in Asian patients.", "score": 0.01158031346710592, "content": "To characterize retinal and fluorescein angiographic findings of Asian patients with symptoms secondary to age-related macular degeneration (ARMD). We retrospectively reviewed 453 consecutive medical records corresponding to fluorescein angiograms performed between November 1992 and November 1995 to identify Asian patients with symptomatic ARMD. Presenting visual symptoms, best-corrected Snellen visual acuities, and retinal examination findings were determined from the medical records. Fundus photos and fluorescein angiograms were reviewed. There were 26 symptomatic eyes in 19 Asian patients with a median age of 73 years. Presenting visual symptoms included decreased visual acuity (19 eyes), metamorphopsia (5 eyes), or scotoma (2 eyes). Retinal findings included occult choroidal neovascularization (CNV) in 5 (19%) eyes, serous pigment epithelial detachment (PED) in 8 (31%) eyes, PED with CNV in 5 (19%) eyes, drusen in 5 (19%) eyes, retinal pigment epithelial atrophy in 1 (4%) eye, vitreous hemorrhage in 1 (4%) eye, and a disciform scar in 1 (4%) eye. In this cohort of Asian patients with ARMD, the majority of symptomatic eyes had either CNV (46%) or serous PED (31%)."}, {"id": "pubmed23n0678_14026", "title": "[Clinical observations of macular hole with and without retinal detachment in high myopic eyes].", "score": 0.009900990099009901, "content": "To study the clinical features and the pathogenesis of macular hole with and without retina detachment (RD) in high myopic eyes. It was a retrospective series case study. The charts of high myopic patients with macular hole at our hospital from June 2006 to February 2007 were retrospectively reviewed and analyzed. Patients were divided into two groups (the RD group and non-RD group) depending on the presence of RD or not. Their clinical data and optic coherence tomography (OCT) results were further analyzed. SPSS 13.0 was used for the statistic analysis. When comparing the quantitative aspects like age, axial length and refraction, t-test was used. Categorical data, such as sex ratio, occurrence of vitreous traction, posterior staphyloma and retinoschisis were compared by using χ(2) test. Fisher's test was used in comparing eye laterality, incidence of white hole, visual acuity and posterior vitreous detachment (PVD). During this period, there were 43 patients fitting the including criteria. Among them, 36 patents (37 eyes) were in the RD group and 7 patients (7 eyes) in the no-RD group. In the RD group, the average age was 56.1, 24.3% of them (9/37) were male; percentage of left and right eyes was (11/37) and 70.3% (26/37), respectively; average refraction was (-8.9 ± 2.2) D; average axial length was (28.7 ± 2.0) mm. Visual acuity was ≤ 0.05 (72.2%) in 26 eyes and 0.05 - 0.2 (27.8%) in 10 patients. The incidence of complete and non-complete PVD was 89.2% (33/37) and 10.8% (4/37), respectively. White hole presented in 35.1% (3/37) patients. Vitreous traction and retinoschisis presented in 27.0% (10/37) and 35.1% (13/37) patients, respectively. In the non-RD group, the average age was 47.6; 16.7% of them (1/7) were male; left and right eyes were involved in 42.9% (3/7) and 57.1% (4/7), respectively. Average refraction was (-9.0 ± 1.9) D; average axial length was (28.9 ± 1.5) mm. Vision acuity was ≤ 0.05 in 3 patients (42.9%); between 0.05 - 0.2 in 3 eyes (42.9%) and ≥ 0.2 in 1 eye (14.3%). Incidence of complete and non-complete PVD was 85.7% (6/7) and 14.3% (1/7), respectively. White hole was observed in 14.3% (1/7) patients; 42.9% (3/7) patients were accompanied with vitreous traction and 71.4% (5/7) with retinoschisis. B-scan ultrasonography showed posterior staphyloma in all 44 eyes. The results of statistical analysis showed that the gender (χ(2) = 0.008) and eye laterality (χ(2) = 0.449) as well as refraction (t = 0.193), axial length (t = -0.25) and visual acuity (χ(2) = 4.509) of these two groups were similar (P &gt; 0.05). The incidences of vitreous traction (χ(2) = 0.709), white hole (χ(2) = 1.179), PVD (χ(2) = 0.071) and retinoschisis (χ(2) = 3.207) were also similar (P &gt; 0.05). But the age of the non-RD group is significantly younger than the RD group (t = 1.66, P &lt; 0.05). Various pathogenesis may involved in the occurrence of retinal detachment in highly myopic eyes with macular hole. Further study is required to improve our understanding of this entity."}, {"id": "pubmed23n1133_19399", "title": "Atypical Chronic Central Serous Chorioretinopathy Mimicking Vogt-Koyanagi-Harada Disease: Full Therapeutic Response to Half-Fluence Photodynamic Therapy", "score": 0.00980392156862745, "content": "The aim of this case report is to describe a case of atypical central serous chorioretinopathy (CSCR) definitively diagnosed after 8 years. A 44-year-old woman presented with reduced visual acuity in her left eye. Her visual acuity was light perception with projection in the right eye and 0.15 in the left. She described a similar decline in vision in her right eye 8 years ago. At that time, she had exudative retinal detachment and was treated with systemic immunosuppressive therapy for a presumed diagnosis of Vogt-Koyanagi-Harada disease. Despite resolution of the exudative retinal detachment, macular scarring developed. Eight years later, she developed inferior exudative retinal detachment in the left eye. A diagnosis of atypical CSCR was made with the help of multimodal imaging and her left eye was successfully treated with eplerenone and half-fluence photodynamic therapy (hf-PDT). In conclusion, early diagnosis and treatment of atypical CSCR may prevent subretinal fibrosis formation and permanent vision loss. Hf-PDT and eplerenone are successful treatment options for atypical CSCR."}, {"id": "pubmed23n1025_4637", "title": "An Unusual Case: Self-separation of an Idiopathic Epiretinal Membrane", "score": 0.00980392156862745, "content": "Self-separation or peeling of an idiopathic epiretinal membrane (ERM) in an eye with partial posterior vitreous detachment (PVD) is a rare event. A 56-year-old woman presented to our clinic with complaints of floaters in her right eye. Best-corrected visual acuity (BCVA) was 9/10 in this eye. Fundus examination and Spectral domain optical coherence tomography (SD-OCT) revealed an idiopathic ERM and Grade 3 PVD in this eye. Four months later, she had complaints of metamorphopsia in her right eye. BCVA was 7/10, while SD-OCT images of the right macula were similar to previous images. One week after the last visit, she presented again due to the sudden disappearance of her metamorphopsia complaints. BCVA had improved to 10/10. Fundus examination demonstrated that the ERM had spontaneously separated from the retinal surface as a flap floating in the vitreous and the foveal contour had returned to normal. The etiologic mechanism may be explained as the contracting forces within an immature ERM being stronger than its adhesion to the retina."}, {"id": "pubmed23n0697_1369", "title": "[Long-term results of the treatment of optic disc pit associated with serous macular detachment: a review of 20 cases].", "score": 0.009708737864077669, "content": "The pathogenesis of the macular serous retinal detachment (SRD) associated with congenital optic disc pit remains controversial. The treatment is also discussed. Through this study, which includes the majority of the techniques available, we report our experiment in the treatment of this pathology. This was a retrospective single-centre study of 20 patients who presented with macular SRD associated with optic disc pit between 1983 and 2009. Various treatments were provided. At the beginning of the study, patients were treated only by juxtapapillary laser photocoagulation. After laser failure then as first-line treatment, laser photocoagulation was associated with intravitreal gas (C3F8) injection with postoperative facedown positioning for 2 weeks. During the past few years, all patients have been systematically treated with vitrectomy with or without internal limiting membrane (ILM) peeling, laser, and gas (C2F6) tamponade. This series consisted of 20 patients: nine men and 11 women. The patients' mean age at presentation was 29 years (range, 9-60 years). The mean time between the onset of the decrease in visual acuity (VA) and treatment was 6.1 months. None of these patients had a posterior vitreous detachment at the time of diagnosis. Six patients were treated by laser photocoagulation alone, which was successful only in two cases. Eleven patients (with laser treatment failure in three) were treated by laser and intravitreal gas injection, with a 72% success rate. We performed vitrectomy with posterior hyaloid dissection, laser, and gas tamponade in eight cases (with laser-gas treatment failure in two) with 87% success rate and no recurrence. Five of these patients had ILM peeling during the vitrectomy. The mean follow-up period was 60 months (range, 2 months to 17 years). This study shows that early treatment of macular SRD associated with optic disc pit by vitrectomy, ILM peeling, juxtapapillary photocoagulation, and gas tamponade is followed by good anatomical and functional results. This treatment is superior to the other less invasive procedures. Optical coherence tomography is an important exam for diagnosis and postoperative follow-up of patients."}, {"id": "pubmed23n0856_18120", "title": "[Atypical presentation of central serous choroidopathy. Case report].", "score": 0.009615384615384616, "content": "Central serous choroidopathy is a macular disease, usually with a self-limited and benign course, and predominantly affects male patients between 20 and 45 years old. A 68 year-old female patient complained of decreased visual acuity of her right eye of approximately 3 weeks of onset. Best corrected visual acuity in her right eye was 20/100. Fundus examination revealed a macular serous detachment involving its centre, as well as the presence of multiple calcified drusen. Fluorescein angiography showed late parafoveal leakage in a \"smokestack\" pattern in the right macular area. Optical coherence tomography showed a dome-shape macular detachment, also in the right eye. The patient was observed every 2 weeks and spontaneous resolution of the macular detachment was seen a month later. Based on these clinical features, a diagnosis was made of central serous choroidopathy of atypical presentation. Atypical presentation cases of serous central choroidopathy might be seen occasionally. Hence, it is an important differential diagnosis of age related macular degeneration in patients older than 60 years."}, {"id": "pubmed23n0348_19038", "title": "Use of a macular buckle in the treatment of exudative age-related macular degeneration.", "score": 0.009615384615384616, "content": "To evaluate a macular buckle for exudative choroidal neovascularization secondary to age-related macular degeneration (ARMD). Forty-two eyes with choroidal neovascular membranes (CNVM) secondary to ARMD underwent surgical placement of a macular buckle. A Gore-Tex strip (2.0-2.5 mm wide) was button-holed through a 5 mm diameter silicone sponge (9 mm long) and placed behind the macula underneath the CNVM by the same surgeon (Dr Peyman) in all cases. Follow-up ranged from 7-76 months (mean, 20.9 months). Of 12 eyes with classic subfoveal CNVM: 4 (33%) gained 2 or more lines of Snellen visual acuity; 3 (25%) gained 1 line, remained the same, or lost 1 line; and 5 (42%) lost 2 or more lines (range + 6 to - 6 lines). Of 22 eyes with ill-defined subfoveal CNVM: 12 (54%) gained 1 line, remained the same, or lost 1 line; and 10 (46%) lost 2 or more lines (range + 1 to - 8 lines). Eight eyes with ill-defined juxtafoveal CNVM had the following visual acuity outcomes: 5 eyes (62%) maintained the same level of Snellen visual acuity (gained 1, 0, or lost 1 line); and 3 (38%) got worse (lost 2 or more lines of Snellen visual acuity, range + 1 to - 6 lines). Ten eyes (24%) bled subretinally during the follow-up period (average 11.5 months, range 14 days to 27 months), all outside the area of indentation of the macular buckle. The macular buckle treatment for exudative subretinal choroidal neovascular membranes in ARMD stabilized visual decline and displaced significant subfoveal hemorrhage."}, {"id": "pubmed23n0888_25831", "title": "CYSTOID MACULAR EDEMA AND CYSTOID MACULAR DEGENERATION AS A RESULT OF MULTIPLE PATHOGENIC FACTORS IN THE SETTING OF CENTRAL SEROUS CHORIORETINOPATHY.", "score": 0.009523809523809525, "content": "To report the pathogenic factors that account for cystoid macular edema and cystoid macular degeneration in chronic central serous chorioretinopathy (CSC). The clinical course and multimodal imaging findings, including fundus color photography, fundus autofluorescence, spectral-domain optical coherence tomography, and fluorescein angiography, of one eye with cystoid macular edema due to chronic CSC was documented. A 44-year old woman with a history of chronic CSC presented with progressive visual decline in the right eye. Best-corrected visual acuity was 20/40. Funduscopic examination revealed diffuse retinal pigment epithelial changes and macular edema. Fluorescein angiography demonstrated perifoveal microaneurysms and leakage in a petaloid configuration. Spectral-domain optical coherence tomography demonstrated cysts at the level of the inner nuclear layer, an epiretinal membrane, vitreomacular traction, and an attenuated retinal pigment epithelial band. Central subfield thickness was 486 μm. Three intravitreal injections of aflibercept were administered over 16 weeks following which there was resolution of leakage, release of vitreomacular traction, and resolution of microaneurysms. Central subfield thickness reduced to 379 μm, but persistent intraretinal cysts were observed. There was subjective improvement in visual symptoms, but Snellen acuity remained at 20/40. Intraretinal cystic changes in chronic CSC may be the result of multifactorial pathogenic factors and may represent the coexistence of cystoid macular edema and cystoid macular degeneration. Anti-vascular endothelial growth factor may play an important role in the treatment of cystoid macular edema caused by CSC."}, {"id": "pubmed23n0723_18591", "title": "[Patient with recurrent central serous chorioretinopathy who developed multiple evanescent white dots and serous retinal detachment immediately following bevacizumab administration].", "score": 0.009523809523809525, "content": "A patient developed choroidal neovascularization (CNV) in one eye during treatment for bilateral recurrent central serous chorioretinopathy (CSC) and was intravitreously injected with bevacizumab; she developed multiple evanescent white dots and serous retinal detachment(SRD). A 50-year-old women had a history of CSC OD at the age of 29 years. On initial examination, CSC OD was noted, and multiple detachments of the retinal pigment epithelium OU were observed. While the CSC in the right eye was successfully treated by laser photocoagulation, it spread to both eyes following this episode. Examination of the right eye by optical coherence tomography (OCT) following the recurrence of the CSC showed slight elevation of the retinal pigment epithelial layer in the central fovea, but this finding disappeared with the resolution of the CSC. However, as the CSC combined with CNV (Gass type 2) recurred within 1 year, the patient was intravitreously injected with bevacizumab. On the day following the injection, SRD OD occurred, and on the 7th day following the injection many white lesions varying in size appeared in the deep layer of the retina, but they healed 3 weeks later, leaving only the CNV. The CNV was cured later by additional photodynamic therapy. Since the lesions of the fundus observed immediately after the bevacizumab administration resolved spontaneously without sequelae, they were retrospectively diagnosed as a white dot syndrome-like disease. The white dot syndrome-like disease is suggested as a rare complication of bevacizumab."}, {"id": "pubmed23n0855_6275", "title": "Unilateral central serous chorioretinopathy in a pregnant Nigerian woman.", "score": 0.009433962264150943, "content": "Central serous chorioretinopathy (CSCR) is an idiopathic condition characterized by serous detachment of the neurosensory retina in the macular region. It is relatively uncommon in Africans and though pregnancy is a known risk factor, there are no previous reports of CSCR in pregnant African women. We report the case of a 35-year-old pregnant woman who presented to our clinic at gestational age of 29 weeks with a 4 months history of blurring of vision in her left eye. Examination revealed visual acuity of 6/4 on the right eye and 6/9 on the left eye. She had normal anterior segments bilaterally and a normal posterior segment on the right. However, she had left macular edema with exudates. There was no significant refractive error. Her blood pressure was normal. Investigations including electrolytes and urea, urinalysis, and blood sugar profile were all normal. She was managed conservatively, and symptoms resolved 2 weeks prior to delivery. This is a case report of CSCR in a pregnant Nigerian woman with spontaneous resolution of symptoms prior to delivery. Pregnant women should be educated about the possibility of visual problems accompanying pregnancy. "}, {"id": "pubmed23n1120_22745", "title": "Three cases of macular hole that occurred in inferior scleral staphyloma associated with tilted disc syndrome: a case series.", "score": 0.009433962264150943, "content": "The objective is to examine the clinical characteristics of three patients with macular hole that occurred in inferior posterior staphyloma associated with tilted disc syndrome. This study involved three eyes of three Japanese female patients (mean age 76.0 years, range 73-84 years) with macular hole occurring in inferior posterior staphyloma associated with tilted disc syndrome. One of the three eyes was slightly myopic, while the other two eyes were highly myopic. In all three eyes, the macular hole was found to be located in or near the margin of the inferior posterior staphyloma. In one eye, the extent of retinoschisis was rather broad in the margin of the macular hole, and another eye had a history of treatment for choroidal neovascularization. As surgical treatment, the internal limiting membrane in areas surrounding the macular hole was detached after producing artificial posterior vitreous detachment, and a gas tamponade was performed. It was found during surgery that the extensibility of the retina in the margin of the MH was decreased in the three eyes as compared with a usual macular hole. Although the macular hole was successfully closed in all three cases post surgery, the layer structure of the central retina was poorly repaired in all three cases and choroidal neovascularization remained in one case. In all three cases, corrected visual acuity remained at 0.3-0.5 post surgery. Our findings showed poor improvement of visual acuity in all three cases post surgery, even if closure of the macular hole is achieved, thus suggesting that in cases of macular hole associated with tilted disc syndrome and inferior posterior staphyloma, the presence of macular dysfunction existing prior to the onset of macular hole affects visual prognosis."}, {"id": "pubmed23n0900_10038", "title": "Cystoid macular edema associated with preservative-free latanoprost after uncomplicated cataract surgery: case report and review of the literature.", "score": 0.009345794392523364, "content": "Cystoid macular edema associated with latanoprost administration has been reported in patients after complicated cataract surgery with coexisting risk factors. We present the first case of preservative free latanoprost associated cystoid macular edema that occurred many months after uncomplicated cataract surgery. A 65-year old Caucasian female presented in the Outpatients Clinic complaining of reduced vision and metamorphopsia in the right eye. She had undergone uneventful phacoemulsification 19 months ago in the right eye and was under treatment with preservative free latanoprost eye drops for the last 7 months for ocular hypertension. Her remaining medical and ocular history were otherwise unremarkable. Cystoid macular edema with serous retinal detachment was diagnosed in the right eye using optical coherence tomography and fluorescein angiography. Latanoprost was discontinued and brinzolamide and nepafenac eye drops were administered in the right eye. Two months later, cystoid macular edema completely resolved with restoration of visual acuity. Nepafenac eye drops were administered for another 2 months. Eight months after latanoprost cessation optical coherence demonstrated no sign of cystoid macular edema whereas a subtle epiretinal membrane was noted. Cystoid macular edema may potentially occur in patients receiving preservative free latanoprost. More interestingly, in our case it was diagnosed in a patient with a long standing pseudophakia after uncomplicated phacoemulsification. No obvious risk factor for macular edema development was recognized. Prompt diagnosis and latanoprost discontinuation resulted in complete resolution of the cystoid macular edema and functional restoration of the eye."}, {"id": "pubmed23n0722_21615", "title": "Vitrectomy for optic disk pit with macular schisis and outer retinal dehiscence.", "score": 0.009345794392523364, "content": "To describe the outcomes of vitrectomy for optic disc pit-related maculopathy with central outer retinal dehiscence. This prospective interventional case series included seven patients with optic disc pit with macular schisis and central outer retinal dehiscence who underwent vitrectomy with internal limiting membrane peeling, barrage laser photocoagulation, and gas tamponade and were followed for at least 6 months. The surgical outcomes in terms of restoration of macular anatomy and visual improvement were recorded at each visit by fundus photography and optical coherence tomography. The mean age of the patients was 21.3 ± 8.6 years (range, 10-35 years), and the mean duration of defective vision was 6.7 ± 8.5 months (range, 1-24 months). Preoperatively, the median best-corrected visual acuity (BCVA) was 20/60 (range, 20/40 to 20/120). Full-thickness macular holes were noticed in 4 patients 1 month postoperatively. Gas tamponade was repeated in two patients with large macular holes. By the final follow-up, macular holes had closed and BCVA improved in all patients except one. Final mean central macular thickness was 176.83 ± 55.74 μ, the range being 109 μ to 256 μ. The median postoperative BCVA was 20/30 (range, 20/20 to 20/80). Six of 7 patients (85.7%) had improvement in BCVA postoperatively (mean, +2 lines; range, 1-4 lines). Five patients (71%) achieved a postoperative BCVA of ≥20/30. Best-corrected visual acuity dropped by one line in the patient with persistent macular hole. Vitrectomy with internal limiting membrane peeling can achieve excellent final surgical outcomes in optic pit maculopathy with outer retinal dehiscence despite the potential for macular hole formation."}, {"id": "wiki20220301en150_35346", "title": "Epiretinal membrane", "score": 0.009266409266409266, "content": "Epidemiology This ocular pathology was first described by Iwanoff in 1865, and it has been shown to occur in about 7% of the population. It can occur more frequently in the older population with postmortem studies showing it in 2% of those aged 50 years and 20% in those aged 75 years. Culture In 1996, Spalding Gray (June 5, 1941 – ca. January 10, 2004), an American actor, screenwriter and playwright, released Gray's Anatomy, a film monologue describing his experiences dealing with a macular pucker and his decision to undergo surgery. In the 2011 film Paul, Ruth had Epiretinal membrane complicated by macular edema in her left vitreous cavity. See also Eye surgery References Additional references External links Macular Pucker Resource Guide from the National Eye Institute (NEI). Disorders of choroid and retina"}, {"id": "wiki20220301en155_35383", "title": "Optic pit", "score": 0.009260053159564435, "content": "Treatment for optic pit-associated macular detachment involves photocoagulation of the retina by use of an ion laser (either krypton or argon). This procedure works by burning one or more rows in between the optic disc and areas of serous retinal detachment. In most cases, macular reattachment results and visual acuity can be restored to about 20/80. This procedure may also be utilized prior to macular detachment in order to help prevent the future development of macular detachment. Other treatments for optic pit-associated macular detachment include macular buckling, gas tamponade, or vitrectomy. Some experts feel that the best results can be attained when the use of any of the above-mentioned modalities (laser photocoagulation, macular buckling, gas tamponade, and vitrectomy) are used in combination. Diagnosis Optic pits should be diagnosed by an eye care professional who can perform a thorough exam of the back of the eye using an ophthalmoscope."}, {"id": "pubmed23n0918_21332", "title": "Microperimetry - A New Tool for Assessing Retinal Sensitivity in Macular Diseases.", "score": 0.009259259259259259, "content": "Macular disease is the leading cause of low vision in the Western world. Drusen and pigmentary irregularities are common among the rural Northern Indian population. The disease process leads to loss of central vision, metamorphopsia, macropsia or micropsia and colour vision defect. To study the retinal sensitivity changes in macular diseases using microperimetry. It was an observational study, conducted in the Department of Ophthalmology at a rural tertiary care hospital. This study was started from December 2014 until June 2016, in all patients with macular disease above the age of 20 years attending the outpatient department. Microperimetry was done for 84 eyes of 52 patients with macular disease. Mean retinal Sensitivity (MS) and fixation stability was evaluated. The statistical analysis of mean retinal sensitivity, central 2° and 4° fixation was done by calculating the mean and standard deviation using 95% confidence interval. The range of age was between 20-81 years. Majority were 32 males (62%) and 20 females (38%). Out of the 84 eyes studied, majority of the macular disease were Age-Related Macular Degeneration (AMD) (50%). Rest 50% were other macular diseases. The mean retinal sensitivity (dB) shown by microperimetry was 10.83 in AMD, 9.12 in Cystoid Macular Oedema (CME), 10.34 in Epiretinal Membrane (ERM), 10.74 in Pigment Epithelial Detachment (PED), 8.96 in Central Serous Chorioretinopathy (CSCR), 6.43 in macular dystrophy, 7.15 in Lamellar Hole (LMH), 9.8 in Pseudomacular Hole (PMH), 3 in geographic atrophy, 11.1 in macular telangiectasia, 5.6 in Berlin oedema, 12.3 in macular scar and 15.2 in haemorrhage in macula. The study showed 64% of the eyes had stable 2° central fixation, 35% had relatively unstable fixation and 1% had unstable fixation. No significant correlation between retinal sensitivity and retinal thickness in AMD was found. This study shows that microperimetry can be a useful tool for objective evaluation of macular function and progression of the disease."}, {"id": "pubmed23n0359_7066", "title": "[Role of vitreoretinal interface in the pathogenesis and therapy of macular disease associated with optic pits].", "score": 0.009259259259259259, "content": "Although the relationship between optic pits and macular lesions was described nearly a century ago, the pathology and pathogenesis of macular detachment remain unclear. Recent OCT studies have shown schisislike spaces in connection with the disc. None of the hypotheses of pathogenesis proposed so far could have been proven. Besides the hypothesis of exudation, the role of the vitreoretinal interface is kept in the background of discussion. We describe a case of macular detachment associated with optic pit that regained full vision after pars plana vitrectomy with laser coagulation and gas tamponade over a follow-up of 26 months. The purpose of our case report is to emphasize the role of the vitreoretinal interface in the pathogenesis of macular detachment associated with optic pits. A 32-year-Caucasian woman developed macular detachment associated with an optic pit on her right eye. The vision deteriorated to 24/60. A standard three-port pars plana vitrectomy was performed. After creating a posterior vitreous detachment, all vitreoretinal adhesions were removed carefully. Peripapillary laser coagulation and gas tamponade with 15% C2F6-air mixture followed. For 26 months after surgery the macula has been flat. The vision is 60/60. Besides the exudative component of macular detachment, the vitreoretinal interface seems to play an underestimated role in the pathogenesis of maculopathy associated with optic pits. Tractional forces could explain the delay of macular detachment in young adulthood and the frequency of treatment failure after laser coagulation and gas tamponade. Pars plana vitrectomy with complete removal of all vitreoretinal adhesions should be a suitable technique in the treatment of macular detachment associated with optic pits."}, {"id": "wiki20220301en067_41615", "title": "Metamorphopsia", "score": 0.00917604966852573, "content": "Age-related macular degeneration Epiretinal membrane and vitreomacular traction Posterior vitreous detachment Macular hole Diagnosis Tests used for diagnosis of Metamorphopsia mostly make use of subjective assessments of how a person views regular patterns. Many of these tests have a poor ability to accurately diagnose or identify a person with the disease (i.e.,poor sensitivity). The use of assessments such as a psychophysical test called preferential hyperacuity perimetry, which assesses a person’s ability to any misalignments of visual objects, may permit a more sensitive diagnosis of Metamorphopsia. Treatment and Prognosis"}, {"id": "pubmed23n0818_11670", "title": "Bilateral macular edema in a patient treated with tamoxifen: a case report and review of the literature.", "score": 0.009174311926605505, "content": "We present a case of a 41-year-old female patient with progressive bilateral visual loss. On examination, her best corrected visual acuity (BCVA) in her right eye was 3/10 and her BCVA in her left eye was 2/10. Fundus and optical coherence tomography examination revealed severe bilateral macular edema. She had been diagnosed with breast cancer 6 years ago and was receiving tamoxifen at a dosage of 20 mg/day ever since. Tamoxifen therapy was discontinued, and the patient received 250 mg of acetazolamide three times a day for a period of 1 month. Both foveae regained their normal contour within 2 months, and her vision was restored to 10/10 BCVA 3 months later. To our knowledge, this is the first case reported where bilateral intraretinal macular edema is the only retinal manifestation in a patient on oral tamoxifen. "}, {"id": "pubmed23n0302_4975", "title": "[Surgical removal of submacular neovascularization membranes].", "score": 0.009174311926605505, "content": "New techniques for vitreoretinal surgery enable the removal of submacular neovascular membranes inaccessible to laser coagulation. The present study describes the anatomical and functional results following membrane removal. Between January 1994 and June 1995, submacular neovascular membranes were removed from 56 eyes with age-related exudative macular degeneration. Thirty-one eyes had classic subfoveal membranes; 19, occult membranes; and 6 eyes, disciform fibrotic changes that had already persisted for more than 6 months. Surgery led to improved vision in 20 of the 56 eyes (36%). In 26 of the 56 eyes (44%) vision at the last follow-up was no different from preoperative vision: in 10 eyes (17%) visual acuity declined. In the group of 6 eyes with a 6-month history of exudative maculopathy, surgery produced a slight improvement in vision in most patient. Complications arose in 8 of the 56 eyes (14%), namely choroidal hemorrhage (2 eyes), a macular hole (1 eye), and peripheral retinal detachment (5 eyes). Recurrence of the membrane was seen in 5 eyes (9%). Surgical removal of submacular membranes can often lead to stabilization or even improvement of vision. Owing to the relatively high rate of complications, however, the indications must be rigorously appraised on a case-by-case basis."}, {"id": "pubmed23n0059_11482", "title": "[Preventive treatment using laser of age-related macular degeneration of the contralateral eye after age-related macular degeneration of the first eye].", "score": 0.00909090909090909, "content": "Since 1982, and with informed patient consent, we have photocoagulated confluent drusen and limited serous pigment epithelium detachment (SPED) in the fellow eye of ten patients suffering from advanced, disciform type, age-related macula degeneration (ARMD). This treatment was only carried out on appearance of metamorphopsia. Photocoagulation was performed with either the green ray of the argon laser, or the yellow ray of a dye laser. Spots of about 200 microns were placed in a grid-like fashion among the drusen. No complications were observed due to the treatment. The follow-up period on these ten patients, eight women and two men, mean age 77 years, was two to eight years, and the three patients have died. The drusen disappeared completely in three patients and partially in one. The functional results seemed favorable in three cases. In one case of confluent drusen associated with SPED and serous retinal detachment, vision improved remarkably from 0.3 to 0.5 with a Parinaud 2, with a follow-up of five years. In another case, the improvement was from 0.4 to 0.7 but the patient died after only a few months. In another case, vision has been stable for five years. The vision of the seven remaining patients deteriorated; three cases showed central areolar sclerosis, and one case a localised new vessel with vision less than 0.1. In three cases vision dropped to 0.2 and Parinaud 6, but they have been stable for at least four years (eight years for one patient).(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n1124_9880", "title": "A case of Nd:YAG laser-induced traumatic macular hole with good visual prognosis after vitrectomy with inverted internal limiting membrane technique.", "score": 0.009009009009009009, "content": "To report an accidental case of traumatic macular hole caused by Nd:YAG laser in a dermatology clinic. A 24-year-old woman sustained a laser injury to her right eye while practicing a dermatologic treatment using a Nd:YAG laser without wearing protective goggles. She noticed sudden-onset and progressing visual loss in her right eye and consulted an ophthalmologist 2 days after injury. The best-corrected visual acuity (BCVA) of her right eye decreased to 20/133. Fundus examination showed white parafoveal flecks with a central retinal hemorrhage and underlying serous retinal detachment. The retinal sensitivity in this lesion deteriorated. Two weeks later, a full-thickness macular hole (FTMH) developed in the affected eye. She was referred to Nagoya City University Hospital where the laser damage described was observed. The BCVA was 20/67. She underwent pars plana vitrectomy performed using the inverted internal limiting membrane (ILM) flap technique and gas tamponade. One week postoperatively, the FTMH closed, the BCVA in her right eye improved to 20/50, and the retinal sensitivity in the macular area mostly improved. The BCVA gradually improved and reached 20/25 9 months after the injury. Protective goggles must be worn when using an Nd:YAG laser in the laboratory or clinical setting. In the unfortunate event of a FTMH, early vitrectomy with an inverted ILM flap technique can be helpful to achieve a good visual prognosis."}, {"id": "pubmed23n0597_21915", "title": "Fellow eye findings of highly myopic subjects operated for retinal detachment associated with a macular hole.", "score": 0.009009009009009009, "content": "To identity anatomic risk factors involved in the onset of retinal complications causing decrease of visual acuity (VA) in the fellow eyes of highly myopic patients operated for retinal detachment with macular hole (RDMH). Cohort study. Ninety-eight patients (mean age, 51.5+/-8.0 years) with bilateral high myopia (mean myopia of the fellow eye, 20.4+/-5.5 diopters) affected by RDMH in the other eye at baseline. Evaluation of the anatomic features at baseline and during 84+/-2.7 months of follow-up by biomicroscopic examination, indirect binocular ophthalmoscopy, B-scan ultrasonography, and optical coherence tomography. Detection of anatomic features associated with onset of retinal complications causing decrease of VA during the follow-up period. The fellow eyes were divided into 2 groups according to the clinical features of the RDMH eyes: Group 1, presence of posterior vitreous detachment (PVD); and Group 2, presence of posterior vitreous schisis (PVS). At baseline, the incidence of PVD in group 1 was 31 of 47 eyes (65.9%) and the incidence of PVS in Group 2 was 42 of 51 eyes (82.3%). At the end of follow-up, group 1 eyes had a lower incidence of retinal complications causing visual decrease than group 2 eyes (group 1, 2/47 eyes; group 2, 9/51 eyes). Fellow eyes of RDMH cases with higher degree of myopia and peculiar vitreoretinal features including PVS, posterior epiretinal membrane, severe posterior staphyloma, and chorioretinal atrophy are more likely to develop retinal complications causing decrease of VA."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[107, 186]], "word_ranges": [[19, 34]], "text": "We all agree that the patient seems to have Cushing's syndrome (we rule out 1)."}, "2": {"exist": true, "char_ranges": [[1119, 1191]], "word_ranges": [[184, 199]], "text": "answer 2 is false (abdominal CT is not a test to rule out s. Cushing's);"}, "3": {"exist": true, "char_ranges": [[1192, 1285]], "word_ranges": [[199, 215]], "text": "an imaging test should only be done once a clear diagnosis of hypercortisolism has been made."}, "4": {"exist": true, "char_ranges": [[323, 620]], "word_ranges": [[54, 100]], "text": "If hypercortisolism is confirmed, the origin should be sought; the ACTH measurement indicates the origin: suppressed in hypercortisolism of adrenal origin or prolonged use of corticoids and elevated or normal if the origin is pituitary or by ectopic ACTH secretion. (Therefore, 4 and 5 are false)."}, "5": {"exist": true, "char_ranges": [[323, 620]], "word_ranges": [[54, 100]], "text": "If hypercortisolism is confirmed, the origin should be sought; the ACTH measurement indicates the origin: suppressed in hypercortisolism of adrenal origin or prolonged use of corticoids and elevated or normal if the origin is pituitary or by ectopic ACTH secretion. (Therefore, 4 and 5 are false)."}}, "full_answer": "This question is a bit convoluted for the diagnosis of Cushing's syndrome but easy if the steps are clear. We all agree that the patient seems to have Cushing's syndrome (we rule out 1). For the diagnosis we have to measure urinary free cortisol, do a dexamethasone suppression test or measure nocturnal salivary cortisol. If hypercortisolism is confirmed, the origin should be sought; the ACTH measurement indicates the origin: suppressed in hypercortisolism of adrenal origin or prolonged use of corticoids and elevated or normal if the origin is pituitary or by ectopic ACTH secretion. (Therefore, 4 and 5 are false). If ACTH is low, a CT scan of the abdomen should be performed to locate the origin. If ACTH is normal or high, a pituitary MRI should be performed (pituitary adenomas responsible for Cushing's syndrome are very small and CT of the pituitary is less sensitive than MRI). It is very important to perform the order well to arrive at a proper diagnosis: 1st: confirm hypercortisolism or s. Cushing's syndrome, 2nd: measure ACTH to orient etiology. 3rd: imaging test according to ACTH levels. Therefore, answer 2 is false (abdominal CT is not a test to rule out s. Cushing's); an imaging test should only be done once a clear diagnosis of hypercortisolism has been made. Therefore, the true one is 3.", "full_answer_no_ref": "This question is a bit convoluted for the diagnosis of Cushing's syndrome but easy if the steps are clear. We all agree that the patient seems to have Cushing's syndrome (we [HIDDEN]). For the diagnosis we have to measure urinary free cortisol, do a dexamethasone suppression test, or measure nocturnal salivary cortisol. If hypercortisolism is confirmed, the origin should be sought; the ACTH measurement indicates the origin: suppressed in hypercortisolism of adrenal origin or prolonged use of corticoids, and elevated or normal if the origin is pituitary or by ectopic ACTH secretion ([HIDDEN]). If ACTH is low, a CT scan of the abdomen should be performed to locate the origin. If ACTH is normal or high, a pituitary MRI should be performed (pituitary adenomas responsible for Cushing's syndrome are very small and CT of the pituitary is less sensitive than MRI). It is very important to perform the order well to arrive at a proper diagnosis: 1st: confirm hypercortisolism or s. Cushing's syndrome, 2nd: measure ACTH to orient etiology. 3rd: imaging test according to ACTH levels. Therefore, answer 2 is [HIDDEN] (abdominal CT is not a test to [HIDDEN]); an imaging test should only be done once a clear diagnosis of hypercortisolism has been made. Therefore, the true one is [HIDDEN].", "full_question": "A 56-year-old female patient consulted for dorso-lumbar spine pain and progressive difficulty in performing usual tasks. In the last 5 years she gained weight, she has ecchymosis easily and arterial hypertension was detected. Physical examination: Obesity of central predominance, rounded facies, increased supraclavicular fat, decreased proximal muscle strength and some reddish striae in the abdomen. She has a blood glucose of 136 mg/dL and the radiological study showed osteoporosis and vertebral crushing. What do you think is the most coherent interpretation and attitude?", "id": 186, "lang": "en", "options": {"1": "Postmenopausal osteoporosis, type 2 diabetes mellitus and essential hypertension, with decreased strength due to diabetic polyneuropathy.", "2": "It is necessary to rule out Cushing's disease by dexamethasone suppression test and perform a cranial CT scan.", "3": "Suggest Cushing's. Determine urinary free cortisol and basal ACTH, which serves to orient its etiology and select the most appropriate imaging technique.", "4": "It looks like Cushing's. If basal ACTH is high, it may be due to corticosteroid use or an adrenal tumor, and an MRI should be performed.", "5": "Probably has Cushing's. If the basal ACTH is low, he probably has a pituitary micro-adenoma, and a cranial CT scan should be performed."}, "question_id_specific": 67, "type": "ENDOCRINOLOGY", "year": 2013, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0356_4633", "title": "Subclinical Cushing's syndrome.", "score": 0.019049346879535557, "content": "Classic Cushing's syndrome is a rare disease with an estimated incidence of 1 case per 100,000 persons. With routine use of imaging techniques such as ultrasound and CT, adrenal masses are being detected with increased frequency. A substantial percentage of these incidentalomas are hormonally active, with 5% to 20% of the tumors producing glucocorticoids. Autonomous glucocorticoid production without specific signs and symptoms of Cushing's syndrome is termed subclinical Cushing's syndrome. With an estimated prevalence of 79 cases per 100,000 persons, subclinical Cushing's syndrome is much more common than classic Cushing's syndrome. Depending on the amounts of glucocorticoids secreted by the tumor, the clinical spectrum ranges from slightly attenuated diurnal cortisol rhythm to complete atrophy of the contralateral adrenal gland with lasting adrenal insufficiency after unilateral adrenalectomy. Patients with subclinical Cushing's syndrome lack the classical stigmata of hypercortisolism but have a high prevalence of obesity, hypertension, and type 2 diabetes. All patients with incidentally detected adrenal masses scheduled for surgery must undergo testing for subclinical Cushing's syndrome to avoid postoperative adrenal crisis. The best screening test to uncover autonomous cortisol secretion is the short dexamethasone suppression test. Because the adrenal origin of a pathologic cortisol secretion is anticipated, the author prefers a higher dexamethasone dose (3 mg instead of 1 mg) to reduce false-positive results. A suppressed serum cortisol level of less than 3 micrograms/dL (80 nmol/L) after dexamethasone excludes significant cortisol secretion by the tumor. A serum cortisol level greater than 3 micrograms/dL requires further investigation, including confirmation by high-dose dexamethasone (8 mg) suppression testing, a CRH test, and analysis of diurnal rhythm. Determination of urinary free cortisol is less useful because increased values are a late finding usually associated with emerging clinical signs of Cushing's syndrome. Patients with suppressed plasma ACTH in response to CRH generally have adrenal insufficiency after surgery and require adequate perioperative and postoperative substitution therapy. Whether patients with subclinical Cushing's syndrome should undergo adrenalectomy is a matter of debate. The author performs surgery in young patients (&lt; 50 years), in patients with suppressed plasma ACTH, and in patients with a recent history of weight gain, substantial obesity, arterial hypertension, diabetes mellitus, and osteopenia. In completely asymptomatic patients with normal plasma ACTH concentrations and in patients older than 75 years, the author recommends a nonsurgical approach. A large prospective randomized study is necessary to evaluate the benefits of surgery versus conservative treatment in patients with subclinical Cushing's syndrome."}, {"id": "pubmed23n0817_15153", "title": "Screening for Cushing's syndrome: is it worthwhile?", "score": 0.017095234703326447, "content": "Cushing's syndrome (CS) is a rare disease characterized by a collection of signs and symptoms, also common in the general population without elevated cortisol secretion. During the last years more patients with CS are identified earlier and with milder disease. Many of these patients are diagnosed during screening efforts performed for certain or isolated complaints like weight gain, diabetes mellitus (DM), hypertension, osteoporosis, elevated white blood cell counts and more. In this review article the most popular screening test performed in the studies cited was the 1-mg dexamethasone suppression test. Cushing is not frequent enough to support the use of routine screening in patients with morbid obesity and type 2 DM. Also only 1% of hypertensive patients have secondary hypertension due to CS. However, screening should be considered in young patients with resistant DM and/or hypertension. Among patients with osteoporosis and vertebral fractures up to 5% were diagnosed with subclinical hypercortisolism; most of these had adrenal adenoma. Screening for CS is important in subjects with adrenal incidentaloma, and many studies show a high prevalence (~10%) of Cushing or subclinical CS in these patients."}, {"id": "pubmed23n0783_14121", "title": "Cushing's disease: establishing the diagnosis and management approach.", "score": 0.015518967627099789, "content": "A 64 year old lady, with a background history of type 2 diabetes mellitus and hypertension, presented with general deterioration of general health, poor glycemic control, difficulty in controlling blood pressure and difficulty in walking. She had past medical history of adenocarcinoma of the oesophagus, treated with surgery and subsequent chemotherapy. General examination revealed high blood glucose and blood pressure and a Cushingoid facies. Overnight dexamethasone suppression test and urinary free cortisol levels confirmed Cushing's syndrome and High dose dexamethasone suppression test showed partial suppression. CT scan of the abdomen showed bilateral hyperplasia of the adrenals with nodularity on the left side, raising the possibility of an adrenal adenoma. ACTH levels were elevated thereby ruling out autonomously functioning adrenal nodule, however increasing the possibility of ectopic ACTH secretion due to the previous medical history. MRI of the pituitary confirmed the presence of an adenoma, thereby pointing to the diagnosis of pituitary dependant Cushing's disease. The patient could not undergo further invasive investigation or surgery due to septicaemia. Medical management of Cushing's syndrome was resorted to in the interim with Ketoconazole, showing excellent response. This case depicts the need for a high index of suspicion for the diagnosis, the importance of organizing specific investigations in the appropriate order to arrive at a diagnosis and an effective management plan."}, {"id": "pubmed23n1115_13389", "title": "[Morbidity and mortality in Cushing's syndrome].", "score": 0.015270734976977327, "content": "Endogenous Cushing's syndrome is a rare endocrine disorder that is fatal if left untreated. It can be distinguished into adrenocorticotropic hormone (ACTH)-dependent (central and ectopic Cushing's syndrome) and ACTH-independent subtypes (unilateral or bilateral adrenal adenomas). The clinical presentation of patients includes typical stigmata of cortisol excess with physical symptoms of catabolic metabolism (myopathy, striae, parchment skin, osteoporosis) and components of metabolic syndrome (diabetes mellitus, obesity, arterial hypertension, hypercholesterolemia). Biochemical diagnosis is performed in three steps: 1. Confirmation of the diagnosis by 1‑mg dexamethasone suppression test, 24‑h urine free cortisol, and measurement of late-night salivary cortisol. 2. Differentiation of ACTH-dependent Cushing's syndrome from ACTH-independent adrenal Cushing's syndrome by measurement of plasma ACTH. 3. Further subtyping by corticotropin-releasing hormone (CRH) test, inferior petrosal sinus sampling, and imaging modalities. Therapeutic decisions are made on an interdisciplinary basis. First-line therapy for all subtypes is surgery when possible; additional options for all forms include drug therapy and bilateral adrenalectomy. Despite adequate treatment, Cushing's syndrome is associated with increased long-term morbidity and mortality. Interdisciplinary and multimodal therapy management is necessary in the long term to positively influence mortality and reduced quality of life."}, {"id": "pubmed23n1121_2204", "title": "Coexistence of Cushing Disease With a Solitary Adrenocorticotrophic Hormone-Dependent Adrenal Adenoma.", "score": 0.015079365079365081, "content": "We report a 49-year-old woman who had minimal features of Cushing syndrome and an incidentally discovered adrenal adenoma. She was subsequently diagnosed with pituitary-dependent Cushing syndrome. Laboratory and imaging studies including serum cortisol, plasma adrenocorticotrophic hormone (ACTH), high dose dexamethasone test, corticotropin-releasing hormone test, computed tomography (CT) scan, and magnetic resonance imaging were performed. A 49-year-old woman was admitted for urosepsis. An abdominal CT scan performed during the urosepsis workup showed a 2.7-cm right adrenal adenoma. She denied any abdominal striae or other symptoms. Physical examination showed normal vital signs, minimal facial fullness without central obesity, and striae. Laboratory results were as follows: 24-hour-urine cortisol 294 μg (reference 4.0-50.0), midnight serum cortisol 23.0 μg/dL (reference &lt; 7.5), and plasma ACTH level 39 pg/mL (reference 5-27). A corticotropin-releasing hormone stimulation test showed &gt;20% rise in serum cortisol and &gt;35% rise in ACTH levels. A pituitary magnetic resonance image showed a 5 mm pituitary lesion. The patient underwent transsphenoidal pituitary surgery, which confirmed an ACTH-secreting lesion. Postoperatively, she required hydrocortisone replacement for the next 10 months. A follow-up adrenal CT performed 6 months later showed a decrease in the size of the adrenal adenoma (1.8 cm). This case highlights the importance of recognizing the coexistence of ACTH-dependent Cushing disease with an adrenal adenoma and partial ACTH dependency of the adrenal adenoma."}, {"id": "pubmed23n0634_13742", "title": "[Prevalence, etiology and clinical findings of Cushing's syndrome].", "score": 0.015040515040515042, "content": "Endogenous Cushing's syndrome is a very rare entity, with an incidence of 2-4 cases per million inhabitants per year. Cases caused by ectopic ACTH secretion are under-diagnosed. Cushing's disease is the most frequent cause of endogenous Cushing's syndrome, which is 5 or 6 times more frequent than adrenal Cushing's syndrome, with an incidence of between 1.2 and 2.4 cases per million inhabitants per year. Cushing's disease is 3-8 times higher in women than in men. The frequency of adrenal tumors is 3 times higher in women, while that of Cushing's syndrome due to adrenal tumors is 3-5 times higher. Age at diagnosis of Cushing's syndrome varies according to the etiology. Most cases of Cushing's disease are due to a pituitary adenoma, although the tumor may not be visible on the available imaging techniques. ACTH-independent Cushing's syndrome is found in 20% of cases and is most frequently due to adenomas (10%) or adrenal carcinomas (8). Bilateral micronodular hyperplasia and macronodular hyperplasia are infrequent entities, representing less than 10% of all cases of ACTH-independent Cushing's syndrome. Both familial and sporadic forms exist: the familial form, or Carney complex, and ACTH-independent bilateral macronodular hyperplasia, in which the size of the adrenal glands is considerably enlarged. The signs and symptoms of Cushing's syndrome are a direct result of long-term exposure to excessive glucocorticoids. Most signs and symptoms are highly prevalent in the general population (hypertension, central obesity, diabetes mellitus or carbohydrate intolerance, osteoporosis, and characteristic phenotypical alterations)."}, {"id": "pubmed23n0270_19530", "title": "[Cushing's syndrome: diagnostic exploration].", "score": 0.01435472739820566, "content": "The diagnosis of Cushing's syndrome is one of the most perplexing and controversial problems in endocrinology. However, significant advances in the diagnosis procedures have been made in the past decade. The diagnostic studies involved in the evaluation of patients with suspected Cushing's syndrome fall into two categories: confirming the presence of true hypercortisolism and establishing the precise aetiology. Diagnosis of Cushing's syndrome: ambulatory screening relies on the overnight 1 mg dexamethasone test. Negative tests are confirmed by measuring cortisol in two 24-hour urine samples. If cortisol excretion is slightly above normal, a 48-hour low-dose dexamethasone suppression test or an intravenous infusion dexamethasone suppression test are required. Diagnosis of the aetiology of Cushing's syndrome: the first step is to establish if the hypercortisolism is ACTH-dependent or not. This step is solved by measuring plasma ACTH and cortisol in the late afternoon. Computed tomography scanning of the adrenal glands is required in ACTH-independent Cushing's syndrome. A unilateral tumour will be demonstrated in most of cases. If bilateral lesions are found, dynamic testing using cortisol releasing factor and/or metyrapone must be performed to confirm the ACTH-independency of the syndrome. In ACTH-dependent Cushing's syndrome, the major difficulty is to distinguish between a pituitary source and an ectopic source of ACTH secretion. Magnetic resonance imaging of the pituitary with gadolinium enhancement must be preferred to computed tomography scanning but its sensitivity is not better than 70-80% and false positives can occur. When no macroscopic pituitary lesion can be detected, bilateral inferior petrosal sinus sampling coupled to CRH injection for ACTH measurement will indicate the source of ACTH secretion. If this test indicates the patient has Cushing's disease, pituitary trans-sphenoidal surgery can be performed. If the test indicates the patient has ectopic ACTH-secretion, a cervico-thoraco-abdominal scanning is necessary to identify the tumour. In the case of occult tumour the hypercortisolism must be controlled by pharmacological agents and the imaging investigations must be repeated at appropriate intervals."}, {"id": "pubmed23n0591_1986", "title": "[Management of hypercortisolism].", "score": 0.01404394825447457, "content": "Cushing's syndrome is a rare but frequently considered disease. Its diagnosis can lead to some difficulties, including confirming the effective endogenous hypercortisolism and determining its cause. The severity of this disease, the diversity of its complications and the multiple therapeutic options make its management challenging. The aim of this review is to present the most recent data about management of Cushing's syndrome, especially diagnostic approaches and therapeutic options. Our references were obtained by screening MEDLINE database from 1996 to 2006. We also included some anterior reviews and consensus statements. We retained the following points: midnight salivary cortisol is a useful tool in the diagnosis of Cushing's syndrome; the desmopressin test can help to distinguish between Cushing's syndrome and \"pseudoCushing's\" due to alcohol consumption or psychiatric disorders; cavernous sinus and inferior petrosal sinus sampling is indicated in the evaluation of ACTH-dependent Cushing's syndromes when pituitary imaging is normal or equivocal or when dynamic tests are contradictory; multislice computed-tomography of the chest and the abdomen and somatostatin analogue scintigraphy, eventually combined, are the best imaging procedures in occult ectopic ACTH syndromes; patients with Cushing's disease should be referred to a neurosurgeon experienced in corticotroph adenomas surgery; metabolic consequences of Cushing's syndrome, such as cardiovascular risk factors and osteoporosis need an aggressive treatment. The incidence of Cushing's syndrome is only 1/100000 per year. However, hypercortisolism is diagnosed by systematic evaluation in 2 to 5% of patients with poorly controlled type 2 diabetes and adrenal incidentalomas. Endocrinological management of the disease improves metabolic disorders in these patients. If these results are confirmed, screening for Cushing's syndrome should be systematically performed in these populations."}, {"id": "wiki20220301en004_55231", "title": "Cushing's syndrome", "score": 0.013584706856325731, "content": "When Cushing's syndrome is suspected, either a dexamethasone suppression test (administration of dexamethasone and frequent determination of cortisol and ACTH level), or a 24-hour urinary measurement for cortisol offers equal detection rates. Dexamethasone is a glucocorticoid and simulates the effects of cortisol, including negative feedback on the pituitary gland. When dexamethasone is administered and a blood sample is tested, cortisol levels >50 nmol/l (1.81 μg/dl) would be indicative of Cushing's syndrome because an ectopic source of cortisol or ACTH (such as adrenal adenoma) exists which is not inhibited by the dexamethasone. A novel approach, recently cleared by the US FDA, is sampling cortisol in saliva over 24 hours, which may be equally sensitive, as late-night levels of salivary cortisol are high in cushingoid patients. Other pituitary hormone levels may need to be ascertained. Performing a physical examination to determine any visual field defect may be necessary if a"}, {"id": "wiki20220301en015_42166", "title": "Cushing's disease", "score": 0.01347210957916554, "content": "ACTH blood test Once Cushing's syndrome has been diagnosed, the first step towards finding the cause is measuring plasma adrenocorticotropic hormone (ACTH) concentration. A concentration consistently below 1.1 pmol/L is classified as corticotropin-independent and does not lead to a diagnosis of Cushing's disease. In such cases, the next step is adrenal imaging with CT. If plasma corticotropin concentrations are consistently above 3.3 pmol/L, then corticotropin-dependent Cushing's syndrome is most likely. Any intermediate values need to be cautiously interpreted and a corticotropin-releasing hormone (CRH) test is advised in order to confirm corticotropin dependency. If corticotropin-dependent Cushing's syndrome is determined then the next step is to distinguish between Cushing's disease and ectopic corticotropin syndrome. This is done via a combination of techniques including CRH, high-dose DST, BIPSS, and pituitary MRI."}, {"id": "pubmed23n0392_16373", "title": "A natural history of adrenocorticotropin-independent bilateral adrenal macronodular hyperplasia (AIMAH) from preclinical to clinically overt Cushing's syndrome.", "score": 0.013290356764394207, "content": "A 49-year-old man was referred to our hospital for the treatment of gallstones in 1993. Bilateral adrenal nodular masses were detected incidentally by abdominal computed tomography. He had no clinical signs of Cushing's syndrome such as central obesity, striae of skin and diabetes mellitus. We performed cholecystectomy and partial adrenalectomy of right adrenal gland as a biopsy, and diagnosed him as preclinical Cushing's syndrome due to adrenocorticotropin-independent bilateral adrenal macronodular hyperplasia (AIMAH) based on endocrinological and histological examinations. We followed him up for 7 years. During the observation period, the sizes of both adrenal glands increased gradually, and finally serum cortisol level increased beyond normal range, and he showed a Cushingoid appearance such as moon face and central obesity. His skin became atrophic and very fragile, and the bone mineral density of his lumbar spine was extremely low. Serum cortisol level was elevated, and plasma ACTH level was always suppressed. Urinary excretion of 17-hydroxycorticosteroid and free cortisol were increased. Diurnal rhythm of cortisol and ACTH was completely lost and high dose (8 mg/day) dexamethasone did not suppress urinary 17-hydroxycorticosteroid excretion. He became clinically overt Cushing's syndrome. We recommended total adrenalectomy, but he refused it. It is important to know the natural history of preclinical Cushing's syndrome due to AIMAH when choosing an adequate treatment."}, {"id": "wiki20220301en000_30619", "title": "Adrenal gland", "score": 0.013189269746646796, "content": "Corticosteroid overproduction Cushing's syndrome Cushing's syndrome is the manifestation of glucocorticoid excess. It can be the result of a prolonged treatment with glucocorticoids or be caused by an underlying disease which produces alterations in the HPA axis or the production of cortisol. Causes can be further classified into ACTH-dependent or ACTH-independent. The most common cause of endogenous Cushing's syndrome is a pituitary adenoma which causes an excessive production of ACTH. The disease produces a wide variety of signs and symptoms which include obesity, diabetes, increased blood pressure, excessive body hair (hirsutism), osteoporosis, depression, and most distinctively, stretch marks in the skin, caused by its progressive thinning."}, {"id": "pubmed23n0750_19523", "title": "Challenges in the diagnostic work-up and management of patients with subclinical Cushing's syndrome and bilateral adrenal masses.", "score": 0.013122829685859646, "content": "To review the challenges encountered in the diagnostic work-up and management of patients with subclinical Cushing's syndrome (SCS) and bilateral adrenal masses to aid in the case description of a patient with SCS and adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH). We describe our experience managing a patient with AIMAH and SCS. This case report is followed by an extensive review of the literature regarding differential diagnoses, work-up including adrenal venous sampling (AVS), and treatment of SCS with bilateral adrenal masses. A 51-year-old female who was diagnosed with recent onset hypertension and diabetes mellitus type 2 was evaluated for adrenal masses discovered incidentally on computed tomography (CT). She did not have any Cushingoid features. Magnetic resonance imaging (MRI) of abdomen was performed for further evaluation. Hormonal evaluation came back consistent with SCS. The AVS results were consistent with bilateral autonomous cortisol hypersecretion without lateralization. Collectively, the findings favored the diagnosis of bilateral AIMAH. A left adrenalectomy was performed, and the patient's clinical response was favorable with improvement in blood pressure (BP) accompanied by significant weight loss. Follow-up hormonal testing for autonomous cortisol hypersecretion was within the target range. AIMAH is a rare cause of SCS. AVS is a useful diagnostic tool that helps localize the source of autonomous cortisol hypersecretion in ACTH-independent SCS with bilateral adrenal masses, especially if radiological features are inconclusive. Patients undergoing unilateral adrenalectomy should be followed for monitoring of clinical response, as well as progression of AIMAH in the contralateral adrenal gland."}, {"id": "wiki20220301en023_25617", "title": "Corticotropic cell", "score": 0.012933710422846106, "content": "Associated diseases Cushing's Disease Corticotropic cells can have detrimental effects on the body if they express too much or too little ACTH. One such example is Cushing's disease, which can result from overproduction of ACTH in the corticotropes due to pituitary tumors known as corticotroph adenomas; this is the cause for roughly two-thirds of those diagnosed with Cushing's disease. It is also possible that this disease can result from production of ACTH in a non-pituitary tumor, known as ectopic production, or the adrenal glands can overproduce cortisol due to an adrenal tumor. This overproduction of ACTH causes an increase in cortisol levels due to increased glucocorticoid synthesis in the adrenal cortex resulting in several associated symptoms. Symptoms of Cushing's disease include: Fatty deposits in the neck or back Stretch marks (striae) Fatigue Osteoporosis Weakened immune system Hypertension"}, {"id": "wiki20220301en004_55233", "title": "Cushing's syndrome", "score": 0.01276003870343493, "content": "When any of these tests is positive, CT scanning of the adrenal gland and MRI of the pituitary gland are performed to detect the presence of any adrenal or pituitary adenomas or incidentalomas (the incidental discovery of harmless lesions). Scintigraphy of the adrenal gland with iodocholesterol scan is occasionally necessary. Occasionally, determining the ACTH levels in various veins in the body by venous catheterization, working towards the pituitary (petrosal sinus sampling) is necessary. In many cases, the tumors causing Cushing's disease are less than 2 mm in size and difficult to detect using MRI or CT imaging. In one study of 261 patients with confirmed pituitary Cushing's disease, only 48% of pituitary lesions were identified using MRI prior to surgery. Plasma CRH levels are inadequate at diagnosis (with the possible exception of tumors secreting CRH) because of peripheral dilution and binding to CRHBP."}, {"id": "pubmed23n0555_1604", "title": "Cushing's disease.", "score": 0.012324395701133403, "content": "Cushing's disease, i.e., pituitary ACTH-secreting adenoma causing excess glucocorticoid secretion, is a rare disease with significant mortality and morbidity. Timely diagnosis and appropriate treatment can alter the course of the disease and are therefore mandatory. First step of the diagnostic work-up is the endogenous glucocorticoid excess by measurement of urinary free cortisol, cortisol circadian rhythmicity or suppression by low doses of dexamethasone. In patients with equivocal results, second line tests, such as the dexamethasone-suppressed CRH test and desmopressin stimulation, usually enable the diagnosis to be confirmed. Measurement of plasma ACTH then allows the distinction between ACTH-dependent (e.g., pituitary or extrapituitary neuroendocrine tumors) and ACTH-independent causes (e.g., adrenal tumors). The last step in the diagnostic algorithm is often the most fraught with problems as the distinction between Cushing's disease and ectopic ACTH secretion relies on judicious interpretation of several diagnostic procedures. Positive responses to stimulation with CRH and inhibition by high doses of dexamethasone, if concurrent, enable a pituitary origin to be established whereas conflicting results call for inferior petrosal sinus sampling, the latter to be performed in experienced centres only. Visualisation of the tumor at pituitary imaging is helpful but not required for the diagnosis, as microadenomas often remain undectected by MRI and/or CT scan and, on the other hand, visualisation of a non-secreting incidentaloma may be misleading. Surgical removal of the pituitary tumor is the optimal treatment choice and should be attempted in every patient. Surgical failures as well as relapses can be treated by radiotherapy, medical therapy or, if necessary, bilateral adrenalectomy. Finally, patients cured of Cushing's disease require long-term monitoring given the risk of relapse and clinical burden of associated ailments."}, {"id": "pubmed23n0648_18952", "title": "The frequency of type 2 diabetic patients who meet the endocrinological screening criteria of subclinical Cushing's disease.", "score": 0.01205913334059787, "content": "Cushing's syndrome, including its mild form/state of adrenal-dependent subset (subclinical Cushing's syndrome; subCS), is known to enhance glucose intolerance, hypertension and obesity. Recently, subclinical Cushing's disease (subCD) has been identified, but its prevalence and the extent of consequent metabolic derangement are unclear. We screened 90 type 2 diabetic patients hospitalized in our department for subCD, according to the diagnostic guideline proposed by the working group of Japanese Ministry of Health, Welfare and Labor in 2006. Plasma ACTH and cortisol levels in the morning and at midnight were determined, and overnight 0.5 mg dexamethasone suppression test (DST) was performed. Those who showed poor cortisol suppression in DST underwent the desmopressin (DDAVP) test. Fifty-seven patients (63.3%) demonstrated abnormally high midnight cortisol levels (&gt;or=2.5 microg/dL), while only nine of them failed to suppress plasma cortisol levels to &lt;3 microg/dL after DST. Although none of the eight patients who underwent the DDAVP test demonstrated the anticipated paradoxical rise in plasma ACTH, these eight patients (8.9%) endocrinologically met the screening criteria of subCD. Since a considerable percentage of pituitary adenomas causing overt Cushing's disease are not identifiable in magnetic resonance imaging, many of those causing subCD may also be unidentifiable. Further follow-up studies including confirmatory testing and pituitary imaging are necessary."}, {"id": "article-20175_17", "title": "Cushing Disease -- Evaluation", "score": 0.01191016333938294, "content": "The most accurate test used to differentiate a pituitary adenoma from ectopic or adrenal Cushing syndrome is inferior petrosal sinus sampling. [12] [18] This invasive method measures the difference in ACTH level found in the inferior petrosal sinus (where the pituitary gland drains) compared to the periphery. [12] [18] A basal central to the peripheral ratio of over 3:1 when CRH is administered confirms the diagnosis of Cushing disease. [18] This test is considered the gold standard in diagnosing Cushing disease because it has a sensitivity and specificity of nearly 94%. Still, it is rarely used in clinical practice due to its high cost, invasiveness, rare but serious complications, and the required special expertise to perform."}, {"id": "Surgery_Schwartz_11118", "title": "Surgery_Schwartz", "score": 0.011906726062805916, "content": "is diagnostic 1) Overnight DST2) 24-hour urinary free cortisol3) 11:00 pm salivary cortisol1) Plasma ACTH2) High-dose DST and urinary cortisolConfirm the diagnosisACTH gradient?Determine source of hypercortisolismDecreased ACTHLack of suppressionCT scan adrenalsIncreased ACTHPositive Increased ACTHLack of suppressionEquivocalresultsFurther testingBilateral petrosalvein samplingAdrenalsourcePituitarysourceEctopic ACTHsourceSTEPS IN DIAGNOSISDIAGNOSTIC STUDIESYesNoFigure 38-44. Diagnosis of Cushing’s syndrome. ACTH = adrenocorticotropic hormone; CT = computed tomography; DST = dexamethasone suppression test.Brunicardi_Ch38_p1625-p1704.indd 169001/03/19 11:22 AM 1691THYROID, PARATHYROID, AND ADRENALCHAPTER 38of a pituitary tumor. In patients suspected of having ectopic ACTH production, CT or MRI scans of the chest and anterior mediastinum are performed first, followed by imaging of the neck, abdomen, and pelvis if the initial studies are negative.Treatment Laparoscopic"}, {"id": "article-20178_12", "title": "Cushing Syndrome -- Evaluation", "score": 0.011817688204669583, "content": "Serum ACTH levels can differentiate ACTH-dependent Cushing syndrome (elevated ACTH or inappropriately normal ACTH) from ACTH-independent (low ACTH level) Cushing syndrome. In patients with ACTH-dependent Cushing syndrome, the high-dose dexamethasone suppression test done by giving 8 mg dexamethasone by mouth at 2300 h and checking cortisol the next day at 0800 h, can differentiate pituitary ACTH from an ectopic ACTH source. A high-dose dexamethasone suppression test will decrease cortisol level by 50 % if the ACTH source is a pituitary adenoma, but not if ACTH is secreted by an ectopic tumor (e.g., oat cell lung carcinoma). Pituitary MRI, unenhanced CT scan of the adrenals, and chest X-ray and CT are also useful to localize the pathology."}, {"id": "wiki20220301en004_55212", "title": "Cushing's syndrome", "score": 0.011465005410780615, "content": "Cushing's syndrome is caused by either excessive cortisol-like medication, such as prednisone, or a tumor that either produces or results in the production of excessive cortisol by the adrenal glands. Cases due to a pituitary adenoma are known as Cushing's disease, which is the second most common cause of Cushing's syndrome after medication. A number of other tumors, often referred to as ectopic due to their placement outside the pituitary, may also cause Cushing's. Some of these are associated with inherited disorders such as multiple endocrine neoplasia type 1 and Carney complex. Diagnosis requires a number of steps. The first step is to check the medications a person takes. The second step is to measure levels of cortisol in the urine, saliva or in the blood after taking dexamethasone. If this test is abnormal, the cortisol may be measured late at night. If the cortisol remains high, a blood test for ACTH may be done."}, {"id": "pubmed23n0737_1554", "title": "Differential diagnosis of adrenocorticotropic hormone-independent Cushing syndrome: role of adrenal venous sampling.", "score": 0.011430208863983698, "content": "To outline the potential role for adrenal venous sampling in the diagnosis and management of adrenocorticotropic hormone (ACTH)-independent Cushing syndrome (CS). We present a case description and discuss the management of a 59-year-old woman with an 8-year history of weight gain, centripetal obesity, a round plethoric face, skin thinning, easy bruising, hirsutism, and progressive muscle weakness. The patient reported a prior personal history of asthma, type 2 diabetes mellitus, hypertension, dyslipidemia, and bilateral leg ulcers, but she denied having any personal or family history of endocrinopathy and was not taking any corticosteroid medication. Elevated midnight serum cortisol, failure to suppress cortisol levels with a low-dose dexamethasone suppression test, and undetectable plasma ACTH all indicated ACTH-independent CS. Additional investigations including dynamic tests and adrenal imaging were supported by adrenal venous sampling in order to make a diagnosis and formulate a management plan. She was ultimately noted to have bilateral functioning adrenal nodules (adenoma and adenolipoma) and underwent successful bilateral laparoscopic adrenalectomy, with postoperative glucocorticoid and mineralocorticoid replacement. Adrenal venous sampling may be an important step in the differential diagnosis of CS and localization of the source of cortisol excess. It may distinguish pheochromocytoma or benign nonfunctioning adrenal nodules from cortisol-secreting adenomas and may avoid unnecessary bilateral adrenalectomy. It can also ensure that the correct operation is completed, if required, and thus avoid the increased morbidity and mortality associated with repeated surgical interventions."}, {"id": "wiki20220301en004_55229", "title": "Cushing's syndrome", "score": 0.011201079622132255, "content": "Strictly, Cushing's syndrome refers to excess cortisol of any etiology (as syndrome means a group of symptoms). One of the causes of Cushing's syndrome is a cortisol-secreting adenoma in the cortex of the adrenal gland (primary hypercortisolism/hypercorticism). The adenoma causes cortisol levels in the blood to be very high, and negative feedback on the pituitary from the high cortisol levels causes ACTH levels to be very low. Cushing's disease refers only to hypercortisolism secondary to excess production of ACTH from a corticotroph pituitary adenoma (secondary hypercortisolism/hypercorticism) or due to excess production of hypothalamus CRH (Corticotropin releasing hormone) (tertiary hypercortisolism/hypercorticism). This causes the blood ACTH levels to be elevated along with cortisol from the adrenal gland. The ACTH levels remain high because the tumor is unresponsive to negative feedback from high cortisol levels."}, {"id": "InternalMed_Harrison_26973", "title": "InternalMed_Harrison", "score": 0.011113296616837135, "content": "Differential Diagnosis The evaluation of patients with confirmed Cushing’s should be carried out by an endocrinologist and begins with the differential diagnosis of ACTH-dependent and ACTH-independent cortisol excess (Fig. 406-10). Generally, plasma ACTH levels are suppressed in cases of autonomous adrenal cortisol excess, as a consequence of enhanced negative feedback to the hypothalamus and pituitary. By contrast, patients with ACTH-dependent Cushing’s have normal or increased plasma ACTH, with very high levels being found in some patients with ectopic ACTH syndrome. Importantly, imaging should only be used after it is established whether the cortisol excess is ACTH-dependent or ACTH-independent, because nodules in the pituitary or the adrenal are a common finding in the general population. In patients with confirmed ACTH-independent excess, adrenal imaging is indicated (Fig. 406-11), preferably using an unenhanced computed tomography (CT) scan. This allows assessment of adrenal"}, {"id": "Neurology_Adams_5297", "title": "Neurology_Adams", "score": 0.01058870207806378, "content": "Cushing disease Described in 1932 by Cushing, this condition is only about one-fourth as frequent as acromegaly. A distinction is made between Cushing disease and Cushing syndrome, as indicated in Chap. 26. The former term is reserved for cases that are caused by the excessive secretion of pituitary ACTH, which, in turn, causes adrenal hyperplasia; the usual basis is a pituitary adenoma. Cushing syndrome refers to the effects of cortisol excess from any one of several sources—excessive administration of steroids (the most common cause), adenoma of the adrenal cortex, ACTH-producing bronchial carcinoma, and, very rarely, other carcinomas that produce ACTH. The clinical effects are the same in all of these disorders and include truncal obesity, hypertension, proximal muscle weakness, amenorrhea, hirsutism, abdominal striae, hyperglycemia, osteoporosis, and in some cases a characteristic mental disorder (see “Cushing Disease and Corticosteroid Psychoses” in Chap. 49)."}, {"id": "wiki20220301en004_55226", "title": "Cushing's syndrome", "score": 0.010537654552266768, "content": "Endogenous Endogenous Cushing's syndrome results from some derangement of the body's own system of cortisol secretion. Normally, ACTH is released from the pituitary gland when necessary to stimulate the release of cortisol from the adrenal glands. In pituitary Cushing's, a benign pituitary adenoma secretes ACTH. This is also known as Cushing's disease and is responsible for 70% of endogenous Cushing's syndrome. In adrenal Cushing's, excess cortisol is produced by adrenal gland tumors, hyperplastic adrenal glands, or adrenal glands with nodular adrenal hyperplasia. Tumors outside the normal pituitary-adrenal system can produce ACTH (occasionally with CRH) that affects the adrenal glands. This etiology is called ectopic or paraneoplastic Cushing's disease and is seen in diseases such as small cell lung cancer. Finally, rare cases of CRH-secreting tumors (without ACTH secretion) have been reported, which stimulates pituitary ACTH production."}, {"id": "pubmed23n0274_14036", "title": "[Cushing syndrome in children].", "score": 0.010519683313700245, "content": "In pediatric patients, endogenous Cushing syndrome is an infrequent condition almost always due to one of two conditions. 1) Adrenal gland tumors account for 70% of Cushing syndromes in young pediatric patients. They cause rapidly progressive hypercorticism not due to increased ACTH production (elevated plasma and urine cortisol levels, very low ACTH and LPH levels unchanged by dexamethasone, metyrapone or CRH). Imaging techniques determine the side and spread of the tumor and look for metastases. Following surgical removal, patients with indicators of malignant disease (tumor weight above 30 g, extracapsular spread or metastases, independently from pathological data) are given op'DDD. 2) Cushing disease occurs in peripubertal patients and causes overweight with delayed statural gain. ACTH production is increased (positive dexamethasone suppression test and provocative metopirone and CRH tests) as a result of a pituitary adenoma which should be looked for by magnetic resonance imaging and whose removal ensures recovery in 50% of cases. Other therapeutic tools include op'DDD, radiation to the pituitary, and bilateral adrenalectomy as the last resort given the high risk of post-adrenalectomy pituitary tumor (50% of pediatric patients). Other causes are exceedingly rare: primary nodular hyperplasia of the adrenal glands and production of ACTH by a nonpituitary tumor. Corticosteroid treatment is the most common cause of Cushing syndrome in children."}, {"id": "InternalMed_Harrison_26975", "title": "InternalMed_Harrison", "score": 0.010276287020473068, "content": "For ACTH-dependent cortisol excess (Chap. 403), a magnetic resonance image (MRI) of the pituitary is the investigation of choice, but it may not show an abnormality in up to 40% of cases because of small tumors below the sensitivity of detection. Characteristically, pituitary corticotrope adenomas fail to enhance following gadolinium administration on T1-weighted MRI images. In all cases of confirmed ACTH-dependent Cushing’s, further tests are required for the differential diagnosis of pituitary Cushing’s disease and ectopic ACTH syndrome."}, {"id": "wiki20220301en015_42168", "title": "Cushing's disease", "score": 0.010272013314609665, "content": "ACTH stimulation test An ACTH stimulation test involving administration of corticotropin-releasing hormone (CRH) or another agent can differentiate this condition from ectopic ACTH secretion. In a patient with Cushing's disease, the tumor cells will be stimulated to release corticotropin and elevated plasma corticotropin levels will be detected. This rarely occurs with ectopic corticotropin syndrome and thus is quite useful for distinguishing between the two conditions. If ectopic, the plasma ACTH and cortisol levels should remain unchanged; if this is pituitary related, levels of both would rise. The CRH test uses recombinant human or bovine-sequence CRH, which is administered via a 100μg intravenous bolus dose. The sensitivity of the CRH test for detecting Cushing's disease is 93% when plasma levels are measured after fifteen and thirty minutes. However, this test is used only as a last resort due to its high cost and complexity."}, {"id": "wiki20220301en015_42170", "title": "Cushing's disease", "score": 0.01016631173308995, "content": "Inferior petrosal sinus sampling IPSS (inferior petrosal sinus sampling) or BIPSS (bilateral IPSS) is a more accurate but invasive test used to differentiate pituitary from ectopic or adrenal Cushing's syndrome. A corticotropin gradient sample via BIPSS is required to confirm diagnosis when pituitary MRI imaging and biochemical diagnostic tests have been inconclusive. A basal central:peripheral ratio of over 2:1, or a ratio over 3:1 when CRH is administered, is indicative of Cushing's disease. This test has been the gold standard for distinguishing between Cushing's disease and ectopic corticotropin syndrome. The BIPSS has a sensitivity and specificity of 94% for Cushing's disease but it is usually used as a last resort due to its invasiveness, rare but serious complications, and the expertise required to perform it."}, {"id": "wiki20220301en086_19076", "title": "Dexamethasone suppression test", "score": 0.010114164904862579, "content": "Test Procedures There are several types of DST procedures: Overnight DST - An oral dose of dexamethasone is given between 11pm and midnight, and the cortisol level is measured at 8 - 9am the next morning Two-day DST - This involves giving an oral dose of dexamethasone at six-hourly intervals for 2 days, with the cortisol level measured 6 hours after the final dose was given Intravenous DST Dexamethasone-CRT test Interpretation Low-dose and high-dose variations of the test exist. The test is given at low (usually 1–2 mg) and high (8 mg) doses of dexamethasone, and the levels of cortisol are measured to obtain the results. A low dose of dexamethasone suppresses cortisol in individuals with no pathology in endogenous cortisol production. A high dose of dexamethasone exerts negative feedback on pituitary neoplastic ACTH-producing cells (Cushing's disease), but not on ectopic ACTH-producing cells or adrenal adenoma (Cushing's syndrome)."}, {"id": "article-20175_14", "title": "Cushing Disease -- Evaluation", "score": 0.010087285902503294, "content": "Two or more positive initial screening tests in a patient with a high pretest probability of Cushing disease confirm the biochemical diagnosis of Cushing syndrome. [12] [17] Once Cushing syndrome has been diagnosed, the first step toward finding the cause is by measuring a baseline plasma ACTH level. [15] A level consistently greater than 3.3 pmol/L is classified as corticotropin-dependent. [15] To differentiate Cushing disease from ectopic corticotropin syndrome, a corticotropin-releasing hormone (CRH) test is needed. [15] In a patient with Cushing disease, the administered CRH stimulates additional corticotropin release, resulting in an elevated plasma corticotropin level. [15] The sensitivity of the CRH test for detecting Cushing disease is 93% when plasma levels are measured at fifteen and thirty minutes. [15] Alternatively, a high-dose 48-hour dexamethasone suppression test or pituitary magnetic resonance imaging (MRI) can be used. [15]"}]}}}}
{"correct_option": 5, "explanations": {"1": {"exist": true, "char_ranges": [[54, 169]], "word_ranges": [[6, 26]], "text": "By rule 1 would be easy to eliminate, the treatment of colon cancer is surgical (with neoadjuvant in rectal cancer)."}, "2": {"exist": true, "char_ranges": [[171, 291]], "word_ranges": [[26, 46]], "text": "If biopsies confirm adenocarcinoma, there is no need to repeat them, since it is necessary to have the complete specimen."}, "3": {"exist": true, "char_ranges": [[293, 364]], "word_ranges": [[46, 58]], "text": "Most colon cancer develops on adenomas, so we could eliminate this one."}, "4": {"exist": true, "char_ranges": [[365, 607]], "word_ranges": [[58, 95]], "text": "Among the other 2, it is important to know that 5 is correct since they almost always present microsatellite instability even without fulfilling the Amsterdam criteria and have a better prognosis than other poorly differentiated colon cancer."}, "5": {"exist": true, "char_ranges": [[365, 607]], "word_ranges": [[58, 95]], "text": "Among the other 2, it is important to know that 5 is correct since they almost always present microsatellite instability even without fulfilling the Amsterdam criteria and have a better prognosis than other poorly differentiated colon cancer."}}, "full_answer": "Complicated question about an unusual adenocarcinoma. By rule 1 would be easy to eliminate, the treatment of colon cancer is surgical (with neoadjuvant in rectal cancer). If biopsies confirm adenocarcinoma, there is no need to repeat them, since it is necessary to have the complete specimen. Most colon cancer develops on adenomas, so we could eliminate this one. Among the other 2, it is important to know that 5 is correct since they almost always present microsatellite instability even without fulfilling the Amsterdam criteria and have a better prognosis than other poorly differentiated colon cancer.", "full_answer_no_ref": "Complicated question about an unusual adenocarcinoma. By rule 1 would be easy to eliminate, the treatment of colon cancer is surgical (with neoadjuvant in rectal cancer). If biopsies confirm adenocarcinoma, there is no need to repeat them, since it is necessary to have the complete specimen. Most colon cancer develops on adenomas, so we could eliminate this one. Among the other 2, it is important to know that [HIDDEN] since they almost always present microsatellite instability even without fulfilling the Amsterdam criteria and have a better prognosis than other poorly differentiated colon cancer.", "full_question": "During a colonoscopy, a 5-cm tumor located in the right colon is detected in a 48-year-old man. No other lesions were found. His maternal grandmother also suffered from colon cancer. The biopsies are superficial and show a poorly differentiated tumor with abundant inflammatory cells in the stroma that is diagnosed as a medullary type carcinoma.", "id": 7, "lang": "en", "options": {"1": "Chemotherapy is the treatment of choice.", "2": "Since the biopsy is superficial, it should be repeated before proceeding with treatment.", "3": "The prognosis of the tumor depends mainly on its high degree of anaplasia.", "4": "It is unlikely that this tumor has developed over a previous adenoma.", "5": "Microsatellite instability and DNA error repair genes should be studied."}, "question_id_specific": 208, "type": "DIGESTIVE", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en190_40247", "title": "Signet ring cell carcinoma", "score": 0.012132231404958678, "content": "Colorectal Primary signet ring cell carcinoma of the colon and rectum (PSRCCR) is rare, with a reported incidence of less than 1 percent. It has a poor prognosis because symptoms often develop late and it is usually diagnosed at an advanced stage. Five-year survival rates in previous studies ranged from nine to 30 percent. Average survival was between 20 and 45 months. It tends to affect younger adults with higher likelihood of lymphovascular invasion. It is worth noting that the overall survival rate of patients with SRCC was significantly poorer than that of patients with mucinous or poorly differentiated adenocarcinoma. In advanced gastric cancers, the prognosis for patients with the SRCCs was significantly worse than for the other histological types, which can be explained by the finding that advanced SRCC gastric cancers have a larger tumor size, more lymph node metastasis, a deeper invasive depth and more Borrmann type 4 lesions than other types. Stomach"}, {"id": "wiki20220301en010_160879", "title": "Colorectal cancer", "score": 0.011086802270577106, "content": "The U.S. National Comprehensive Cancer Network and American Society of Clinical Oncology provide guidelines for the follow-up of colon cancer. A medical history and physical examination are recommended every 3 to 6 months for 2 years, then every 6 months for 5 years. Carcinoembryonic antigen blood level measurements follow the same timing, but are only advised for people with T2 or greater lesions who are candidates for intervention. A CT-scan of the chest, abdomen and pelvis can be considered annually for the first 3 years for people who are at high risk of recurrence (for example, those who had poorly differentiated tumors or venous or lymphatic invasion) and are candidates for curative surgery (with the aim to cure). A colonoscopy can be done after 1 year, except if it could not be done during the initial staging because of an obstructing mass, in which case it should be performed after 3 to 6 months. If a villous polyp, a polyp >1 centimeter or high-grade dysplasia is found, it"}, {"id": "wiki20220301en589_7880", "title": "Histopathology of colorectal adenocarcinoma", "score": 0.01084844213808273, "content": "Microscopy Adenocarcinoma is a malignant epithelial tumor, originating from superficial glandular epithelial cells lining the colon and rectum. It invades the wall, infiltrating the muscularis mucosae layer, the submucosa, and then the muscularis propria. Tumor cells describe irregular tubular structures, harboring pluristratification, multiple lumens, reduced stroma (\"back to back\" aspect). Sometimes, tumor cells are discohesive and secrete mucus, which invades the interstitium producing large pools of mucus. This occurs in mucinous adenocarcinoma, in which cells are poorly differentiated. If the mucus remains inside the tumor cell, it pushes the nucleus at the periphery, this occurs in \"signet-ring cell.\" Depending on glandular architecture, cellular pleomorphism, and mucosecretion of the predominant pattern, adenocarcinoma may present three degrees of differentiation: well, moderately, and poorly differentiated. Micrographs (H&E stain) Microscopic criteria"}, {"id": "pubmed23n0933_23652", "title": "[Clinicopathological characteristics and prognosis analysis of colorectal synchronous multiple primary cancer].", "score": 0.010414163262036369, "content": "To investigate the clinicopathological features and prognosis of colorectal synchronous multiple primary cancer(SMPC). From January 2008 to June 2011, 51 patients diagnosed with colorectal SMPC underwent surgery at Department of General Surgery of Peking University First Hospital. Their clinicopathological features, diagnosis, treatment and prognosis were summarized and analyzed. SMPC was diagnosed according to the following criteria: each tumor must have a definite pathologic picture of malignancy; metastasis or recurrence from another colorectal cancer was excluded; tumors must be distinctly separated by at least 5 cm of all intact bowel wall from each other; SMPC has abnormal cells between tumor and normal mucosa and abnormal gland of transitional zone; each cancer is infiltrating carcinoma except the carcinoma in situ; all the cancers are detected at the same time or within 6 months. Multiple primary colorectal cancer originated from familial colonic polyposis or ulcerative colitis was excluded. These 51 colorectal SMPC patients accounted for 3.5% of 1 452 colorectal cancer patients in the same period at our hospital, with 32 males and 19 females, and mean age of (63±13)(29 to 82) years. Of 51 cases, 46(90.2%) had 2 original carcinoma, 3(5.9%) had 3 original carcinoma and 2(3.9%) had 4 carcinoma; 23(45.1%) complicated with colon polyps, 4(7.8%) complicated with malignancy outside the colorectum. In TNM staging, 7(13.7%), 15(29.4%), 24(47.1%) and 5(9.8%) patients were stage I(, II(, III( and IIII( respectively. Among 51 patients undergoing surgery by different procedures, 16 were subtotal colon resection, 8 were extended right colon resection, 5 were extended left hemicolon resection, 8 were right hemicolon resection plus Dixon procedure, 10 were Dixon, and 4 were right hemicolon resection plus sigmoid colon resection. Adjuvant chemotherapy and support treatment were given according to the condition after operation. A total of 105 tumors were found, including 25(23.8%) tumors in sigmoid colon, 24(22.9%) in rectum, 22(21.0%) in ascending colon and 4 in organs outside the colorectum. Tubular adenocarcinoma (86/105, 81.9%) was the main pathological type in these colorectal SMPC patients. During the follow-up of median 43.5 months, 10 cases presented local recurrence and 6 cases had liver metastasis. Multivariable analysis showed that ≤65 years old (OR=22.757, 95%CI: 1.562-331.543, P=0.002),undifferentiated carcinoma or mucous adenocarcinoma (OR=27.174, 95%CI: 2.834-260.512, P=0.004), stage III(-IIII( (OR=29.626, 95%CI: 3.216-272.884, P=0.003) were independent risk factors of postoperative 5-year recurrence and metastasis, but the number of SMPC lesions and the surgical method were not associated with postoperative 5-year recurrence and metastasis (P=0.564, P=0.513). The 3-year and 5-year survival rates of colorectal SMPC patients were 76.5% and 64.7%. Two-original carcinoma is the most common in colorectal SMPC patients, which mainly distributes in sigmoid colon and rectum. Postoperative monitoring should be strengthened for those patients with younger age, poor pathological types and advanced staging to prevent recurrence and metastasis."}, {"id": "wiki20220301en010_160872", "title": "Colorectal cancer", "score": 0.010253239559986603, "content": "In Stage I colon cancer, no chemotherapy is offered, and surgery is the definitive treatment. The role of chemotherapy in Stage II colon cancer is debatable, and is usually not offered unless risk factors such as T4 tumor, undifferentiated tumor, vascular and perineural invasion or inadequate lymph node sampling is identified. It is also known that the people who carry abnormalities of the mismatch repair genes do not benefit from chemotherapy. For stage III and Stage IV colon cancer, chemotherapy is an integral part of treatment."}, {"id": "wiki20220301en126_27013", "title": "Microsatellite instability", "score": 0.009900990099009901, "content": "Direct and indirect mechanisms contribute to chemotherapy resistance. Direct mechanisms include pathways that metabolize the drug, while indirect mechanisms include pathways that respond to the chemotherapy treatment. The NER DNA repair pathway plays a substantial role in reversing cell damage caused by chemotherapeutic agents such as 5-FU. Discoveries since 2010 In May 2017 the FDA approved an immunotherapeutic called Keytruda (pembrolizumab) (PD-1 inhibitor) for patients with unresectable or metastatic microsatellite instability-high (MSI-H) or mismatch repair deficient (dMMR) solid tumors that have progressed following prior treatment. This indication is independent of PD-L1 expression assessment, tissue type and tumor location. Researchers have found another MSI, called elevated microsatellite alterations at selected tetranucleotide repeats (EMAST). However, EMAST is unique in that it is not derived from MMR, and it is commonly associated with TP53 mutations."}, {"id": "pubmed23n0301_22311", "title": "[The role of colonoscopy in early diagnosis of intraluminal recurrences in patients already treated for colorectal cancer].", "score": 0.009900990099009901, "content": "It is a common opinion that the more often and the more rigorously the colon is examined, the more lesions will be discovered and diagnosed. However it has not been shown which methods of colonic examination and which regimen of surveillance should be used. Chart review was conducted on 481 patients who underwent curative resection for colorectal cancer between 1980 and 1990. Colonoscopy was performed preoperatively, after 12-15 months from surgical treatment, and then at an interval of 12-24 months, or when symptoms appeared. About ten percent of patients developed intraluminal recurrences, and more than 25% adenomatous polyps. More than one half of the metachronous lesions arise within the first 24 months. The median time to diagnosis was 25 months for intraluminal recurrences and 22 months for adenomatous polyps. Patients with left sited tumor at an advanced stage run a higher risk of developing recurrent intraluminal disease, and patients who presented associated polyps at the time of the operation for the index cancer have a higher risk of developing new polyps. About 50% of recurrences were detected when patients were asymptomatic. Colonoscopy must be performed within the first 12-15 months after operation, while an interval of 24 months between each examination seems sufficient to guarantee an early detection of metachronous lesion. Asymptomatic patients are more frequently reoperated for cure and thus have a better survival rate."}, {"id": "wiki20220301en010_160832", "title": "Colorectal cancer", "score": 0.009858336505900976, "content": "Bowel cancer may be diagnosed by obtaining a sample of the colon during a sigmoidoscopy or colonoscopy. This is then followed by medical imaging to determine whether the disease has spread. Screening is effective for preventing and decreasing deaths from colorectal cancer. Screening, by one of a number of methods, is recommended starting from the age of 50 to 75. During colonoscopy, small polyps may be removed if found. If a large polyp or tumor is found, a biopsy may be performed to check if it is cancerous. Aspirin and other non-steroidal anti-inflammatory drugs decrease the risk. Their general use is not recommended for this purpose, however, due to side effects."}, {"id": "wiki20220301en015_13653", "title": "Biopsy", "score": 0.00980392156862745, "content": "There are two types of liquid biopsy (which is not really a biopsy as they are blood tests that do not require a biopsy of tissue): circulating tumor cell assays or cell-free circulating tumor DNA tests. These methods provide a non-invasive alternative to repeat invasive biopsies to monitor cancer treatment, test available drugs against the circulating tumor cells, evaluate the mutations in cancer and plan individualized treatments. In addition, because cancer is a heterogeneous genetic disease, and excisional biopsies provide only a snapshot in time of some of the rapid, dynamic genetic changes occurring in tumors, liquid biopsies provide some advantages over tissue biopsy-based genomic testing. In addition, excisional biopsies are invasive, can’t be used repeatedly, and are ineffective in understanding the dynamics of tumor progression and metastasis. By detecting, quantifying and characterisation of vital circulating tumor cells or genomic alterations in CTCs and cell-free DNA in"}, {"id": "pubmed23n0565_13838", "title": "Localization, clinical and pathological characteristics and survival in sporadic colon cancer patients younger than 40 and over 65 years of age.", "score": 0.00980392156862745, "content": "Colorectal cancer is predominantly a disease of older population, but occasionally it affects younger patients, in whom very often diagnosis is overseen and treatment begins late. The aim of our study was to compare localization, clinical and pathological characteristics and survival of sporadic colorectal cancer patients aged up to 40 and over 65 years. The first group (group I) included 19 patients under 40 years and the second group (group II) 28 patients aged over 65 years, treated during 1997-2001. Patients with family history of colon cancer and inflammatory disease of the colon were not included. Arithmetic mean, standard deviation, Fisher's test, Student 's t test, x(2) test and the Kaplan-Meier method were used in the statistical analysis of the results. There was no difference among the tested groups regarding tumor localization. The most frequent localization was in the rectum and left colon. At presentation, in group I patients, besides the metastases in the liver and lymph nodes, colorectal cancer infiltrated also the duodenum, stomach, right kidney capsule in one patient, and adnexa in two patients. In group II patients we registered only liver and lymph node metastases. Pathologically, tubular and mucinous forms were present in all of the patients up to 40 years of age, while only one patient over 65 had tumor with mucinous component. In group I, Astler-Coller stage B was found in 1.5% of the patients, stage C in 72.5% and stage D in 26%; in group II, stage B was found in 1.5%, stage C in 84.5% and stage D in 14%. Grade III was 36.8% in group I and 17.8% in group II. No statistical differences were found in stage distribution (p=0.36) and grade (p=0.06) between group I and II. Five-year overall survival was 57.8% and 28.5% in younger and older patients, respectively (p=0.053). The results obtained showed no difference in clinical symptomatology and tumor localization in both groups. The incidence of more aggressive tumors was higher in younger persons. However, early detection combined with more aggressive therapeutic approach, could enable significant improvement of the 5-year survival of younger patients with colon cancer."}, {"id": "wiki20220301en126_27010", "title": "Microsatellite instability", "score": 0.009708737864077669, "content": "Microsatellite instability diagnostics MSI is a good marker for determining Lynch syndrome and determining a prognosis for cancer treatments. In 1996, the National Cancer Institute (NCI) hosted an international workshop on Lynch Syndrome, which led to the development of the “Bethesda Guidelines” and loci for MSI testing. During this first workshop the NCI has agreed on five microsatellite markers necessary to determine MSI presence: two mononucleotides, BAT25 and BAT26, and three dinucleotide repeats, D2S123, D5S346, and D17S250. MSI-H tumors result from MSI of greater than 30% of unstable MSI loci (>2 or more of the 5 loci). MSI-L tumors result from less than 30% of unstable MSI biomarkers. MSI-L tumors are classified as tumors of alternative etiologies. Several studies demonstrate that MSI-H patients respond best to surgery alone, rather than chemotherapy and surgery, thus preventing patients from needlessly experiencing chemotherapy."}, {"id": "pubmed23n0039_7596", "title": "Definitive treatment of \"malignant\" polyps of the colon.", "score": 0.009708737864077669, "content": "There has been an unremitting rise in incidence of colonic cancer in this country with no recent improvement in cure rate. As a result the evolution of colorectal cancer has been the focus of considerable attention with an enlarging body of evidence pointing to the common neoplastic polyp as a precursor to malignancy. \"Neoplastic\" polyps include \"adenomatous polyps,\" \"villous adenomas\" and, lately recognized, \"villo-glandular polyps.\" Experience with endoscopic removal of over 2,000 colonic polyps (with no mortality) has introduced two questions of prime concern to the surgeon: (1) What constitutes  clinical malignancy in a polyp? AND, (2) When should laparatomy supplant or follow endoscopic removal? Eight hundred and ninety-two consecutive adenomatous (tubular), villous, villoglandular (villo-tubular) and \"polypoid cancer\" polyps are analyzed, 855 of which have been followed for 6 months to 4 years. Support is offered to the concept that villous and tubular growth patterns are merely variants of a similar base disturbance in cell renewal. Superficial cancer (carcinoma-in-situ) occurred in 6.6% of neoplastic polyps and represents no threat if the polyp is completely removed. Only when the cancer penetrates the muscularis mucosae should it be regarded as \"invasive.\" The term \"malignant polyp\" should be reserved for this form. Invasive cancer was found in 5.0% of neoplastic polyps in this series. Only in this group need the question of further surgical intervention be raised. Major considerations influencing a decision for subsequent laparotomy are polyp size and gross morphology (i.e. sessile or pedunculated), histologic type (of the polyp and of the cancer itself), adequacy of clearance between depth of invasion and plane of polyp resection, and the patient's age and general condition. These are analyzed. Twenty-five of 46 patients with \"malignant polyps\" were subjected to abdominal exploration: 17 showed no residual cancer, whereas 8 (5 with recognized incomplete endoscopic removal) had tumor in the bowel wall. Of the remaining 21 patients, for whom endoscopic polypectomy alone was deemed appropriate, none have shown residual or recurrent cancer on clinical and endoscopic followup. Colonoscopy appears to be a most promising approach in terms of the goals of cancer programs, offering both prophylaxis and opportunity for treatment at a favorable stage of disease."}, {"id": "wiki20220301en189_29036", "title": "Skin cancer in cats and dogs", "score": 0.009615384615384616, "content": "Treatment The specific treatment will depend on the tumor's type, location, size, and whether the cancer has spread to other organs. Surgical removal of the tumor remains the standard treatment of choice, but additional forms of therapy such as radiation therapy, chemotherapy, or immunotherapy exist. When detected early, skin cancer in cats and dogs can often be treated successfully. In many cases, a biopsy can remove the whole tumor, as long as the healthy tissues removed from just outside the tumor area do not contain any cancer cells. References External links Skin Cancer in Cats and Dogs from Pet Cancer Center Skin Cancer in Dogs from CanineCancer.com' Types of animal cancers Cancer in dogs Cancer in cats Integumentary neoplasia"}, {"id": "pubmed23n0387_9310", "title": "[Gastrocolic tumor progression--a possibility or mere supposition?].", "score": 0.009615384615384616, "content": "The authors examined the five-years postoperative survival rate of fifty patients who suffered from colorectal cancer along with the fact that the large bowel one of the neighbouring organs were resected. The subjects were divided into four groups: the colorectal resection was associated with (1) stomach resection (13 patients); (2) liver metastasectomy (14 patients); (3) small bowel resection (10 patients); (4) the resection of other organs (13 patients). In the first two years of the study they were examined once in every three months, in the next two years once in every six months and then yearly. The following tests were carried out: chest X ray, abdominal sonography, irrigography or colonoscopy and CEA. On condition that the colonoscope reached the caecum and the result was negative, the test was repeated only a year later. The patients were operated on between 1985 and 1997. The statistical analysis was made with the help of the Kaplan-Meier method. During this period fifty-six complex resections were performed. Out of fifty-six patients fifty were followed. Compliance 89%. In group 1, where the average age of patients was sixty-two years, one patient died in the forty-first and the other in the fifty-second month after the surgery. Survival rate: 11/13 (83%). The survival rates for the other groups were as follows: group 2 (average age 64) twelve patients died within five years. Survival rate 2/14 (14%). The difference between the survival rates in the first two groups in significant (P = 0.0001). Group 3 (average age 67) seven died and only three survived. Survival rate: 3/10 (30%). The difference between group 1 and group 3 is significant (P = 0.0022). Group 4 (average age 64) seven patients died. Survival rate 6/13 (46%). Comparing this rate to that of the group 1, the difference is not significant (P &gt; 0.01). Having analysed the results of the four groups it can be concluded that the patients of group 1 lived the longest (stomach resection) and those of group 2 (liver metastasectomy) died the earliest after the operation. It is surprising that the patients of group 3 lived significantly shorter than the ones of group 1 in spite of the fact that they belong by far the greatest number to stage Dukes B (group 1: 12/13 = 92%; group 3: 7/10 = 70%). The authors assume that the partial or the total absence of the stomach keeps back the growth of the tumour (gastro-colic tumour growing dependency). They think that in case of colon cancer which infiltrates the stomach surgeons experienced in gastric and colorectal surgery should be encouraged to take the risk of the double resection providing the fact that the operation is accomplishable."}, {"id": "wiki20220301en015_13654", "title": "Biopsy", "score": 0.009523809523809525, "content": "in understanding the dynamics of tumor progression and metastasis. By detecting, quantifying and characterisation of vital circulating tumor cells or genomic alterations in CTCs and cell-free DNA in blood, liquid biopsy can provide real-time information on the stage of tumor progression, treatment effectiveness, and cancer metastasis risk. This technological development could make it possible to diagnose and manage cancer from repeated blood tests rather than from a traditional biopsy."}, {"id": "pubmed23n0239_1422", "title": "[Diagnosis and therapy of colorectal polyps with special reference to adenomas].", "score": 0.009523809523809525, "content": "Colo-rectal adenoms occur more frequently in the elderly and should be considered as precancerous. The structural changes of the glandular epithelium are known as dysplasia or atypia and are classified into three grades of severity; the \"severe epithelial dysplasia\" has all the histological characteristics of a malignant tumor which however has not infiltrated the muscularis mucosa and so has not gained access to the lymphatic system. Whenever these structural changes were present the terms focal carcinoma or carcinoma in situ were used. However in 1976 the WHO accepted to change the nomenclature to \"severe epithelial dysplasia\", as Morson had proposed. Their aim was to avoid superfluous radical surgical intervention. Whenever severe dysplasia is present in an adenoma, the necessary therapy is the local excision of the adenoma together with its pedick. An exact complete histological examination is necessary. Between 1976 and 1980 we saw 201 cases of adenoma of the colon or rectum at the Surgical Clinic, University of Düsseldorf. 27 of these cases showed severe epithelial dysplasia. As described in the literature there was a correlation between the size of the adenoma, the histological picture and the risk of malignancy. The reexamination of 105 patients showed that there was a significant percentage of recurrency at the site of excision or new polyps at a different site. Therefore, regular checkups are a must for all those patients in whom polyps of the large bowel have been removed."}, {"id": "wiki20220301en634_8498", "title": "Lipofibromatosis", "score": 0.009433962264150943, "content": "The diagnosis of LPF depends on its clinical presentation almost exclusively in newborn and young children and, most importantly, its histopathology as determined on biopsied intact tissue or fine-needle aspiration to obtain a sampling of the tumor's cells. Intact tissue samples typical show abundant mature-appearing adipose (i.e. fat) tissue mixed with a minor component of oval-shaped or spindle-shaped fibroblast-like cells some of which have a pseudolipoblast-like morphology. Needle biopsies should show these cells. However, LPF histopathology can vary widely between cases. The cited gene abnormalities in the above section are insufficient to support a diagnosis of LPF although further study of these and discoveries of other gene abnormalities may do so. The histopathology of lipofibromatosis-like neural tumors (LPF-NT) can closely resemble LPF tumors. Unlike LPF tumors, however, LPF-NT tumors have been diagnosed in adults in more than 27% of cases with the remaining cases diagnosed"}, {"id": "pubmed23n0271_12666", "title": "[Synchronous and metachronous tumors of the colon and rectum].", "score": 0.009433962264150943, "content": "Three hundred and seventy-two patients with colorectal tumours treated by curative resection between January 1982 and January 1992 were reviewed in order to determine the role of colonoscopy and the outcome of patients with multiple tumours. Thirty (8.1%) of them with a mean age of 57 (35-79) years (20 males, 10 females) had synchronous (19 cases) or metachronous (11 cases) lesions. Rectum and sigmoid colon were the most frequent site of multiple lesions, accounting for 73% of all lesions. Accurate pre-operative diagnosis was performed in 14 of the 19 patients with synchronous lesions, and in the remaining 5 cases failure to perform an intra-operative colonoscopy was the cause of missing the lesions. Three of them had over-looked lesions on the previous barium enema. Synchronous lesions has the tendency to be less invasive as compared to metachronous ones. Five-year survival rates (Kaplan-Meier method) were 45% and 58% for patients with multiple and single lesions respectively (not significant). For patients with colorectal carcinoma a thorough examination of the whole colon by intra-operative colonoscopy should be accomplished in order to rule out the possibility of associated lesions as well as to decrease the incidence of \"early\" metachronous lesions."}, {"id": "wiki20220301en040_16700", "title": "Benign tumor", "score": 0.009374557803877175, "content": "Tumors are formed by carcinogenesis, a process in which cellular alterations lead to the formation of cancer. Multistage carcinogenesis involves the sequential genetic or epigenetic changes to a cell's DNA, where each step produces a more advanced tumor. It is often broken down into three stages; initiation, promotion and progression, and several mutations may occur at each stage. Initiation is where the first genetic mutation occurs in a cell. Promotion is the clonal expansion (repeated division) of this transformed cell into a visible tumor that is usually benign. Following promotion, progression may take place where more genetic mutations are acquired in a sub-population of tumor cells. Progression changes the benign tumor into a malignant tumor. A prominent and well studied example of this phenomenon is the tubular adenoma, a common type of colon polyp which is an important precursor to colon cancer. The cells in tubular adenomas, like most tumors that frequently progress to"}, {"id": "wiki20220301en459_3656", "title": "Circulating tumor DNA", "score": 0.009345794392523364, "content": "The emergence of drug-resistant tumors due to intra- and inter-tumoral heterogeneity an issue in treatment efficacy. A minor genetic clone within the tumor can expand after treatment if it carries a drug-resistant mutation. Initial biopsies can miss these clones due to low frequency or spatial separation of cells within the tumor. For example, since a biopsy only samples a small part of the tumor, clones that resides in a different location may go unnoticed. This can mislead research that focuses on studying the role of tumor heterogeneity in cancer progression and relapse. The use of ctDNA in research can alleviate these concerns because it could provide a more representative 'screenshot' of the genetic diversity of cancer at both primary and metastatic sites. For example, ctDNA has been shown to be useful in studying the clonal evolution of a patient’s cancer before and after treatment regimens. Early detection of cancer is still challenging but recent progress in the analysis of"}, {"id": "pubmed23n0621_2234", "title": "[A thousand total colonoscopies: what is the relationship between distal and proximal findings?].", "score": 0.009345794392523364, "content": "Flexible sigmoidoscopy is indicated for colorectal cancer screening. The decision about who needs total colonoscopy based on distal findings is still controversial because of the uncertainty of the associations between distal and proximal findings. The purpose of the study was to characterize distal findings in patients with total colonoscopy, to investigate its importance as markers of advanced proximal lesions and to evaluate the usefulness of a clinical Predictive Index, already published in the literature, in the identification of these lesions. Retrospective analysis of the patients submitted to total colonoscopy between January 2006 and February 2007, with selection of 1000 consecutive cases with reference to polyps. We analysed demographic data, indication for the exam and morphological and histological characteristics of the polyps. Advanced lesion was defined as any adenoma larger than 10 mm or any polyp with villous characteristics, high grade dysplasia or cancer. The Predictive Index was obtained through the assignment of points to 3 categories: sex, age and distal findings, which result in 3 groups: low, intermediate and high risk. The mean age of patients was 64,69 years and 65,1% were male. Distal and proximal polyps were identified in 829 (82,9%) and 369 (36,9%) patients, respectively. Advanced distal lesion was found in 342 patients (34,2%) and advanced proximal lesion in 98 (9,8%). 587 patients (58,7%) were in the high risk group. In the group of patients with advanced proximal lesion, a third presented low and intermediate risk, 52% had no distal polyps, 88,7% had less than three distal polyps and 71,4% had no advanced distal lesion. Sensitivity values for these four categories ranged between 11,2% and 66,6%. If the decision to perform total colonoscopy is based on distal colonic findings or on the Predictive Index, the ability to identify advanced proximal lesion is markedly reduced, endangering the aim of a screening program."}, {"id": "wiki20220301en371_14817", "title": "Endoscopic mucosal resection", "score": 0.009259259259259259, "content": "For the esophagous Endoscopic mucosal resection has been advocated for early esophageal cancers (that is, those that are superficial and confined to the mucosa only) and has been shown to be a less invasive, safe, and effective therapy for early squamous cell carcinoma. It has also been shown to be safe and effective for early adenocarcinoma arising in Barrett’s esophagus. The prognosis after treatment with this method is comparable to surgical resection. This technique can be attempted in patients who have no evidence of nodal or distant metastases, with differentiated tumors that are slightly raised and less than 2 cm in diameter, or in differentiated tumors that are ulcerated and less than 1 cm in diameter. The most commonly employed modalities of endoscopic mucosal resection include strip biopsy, double-snare polypectomy, resection with combined use of highly concentrated saline and epinephrine, and resection using a cap."}, {"id": "pubmed23n0215_9020", "title": "A potentially brighter prognosis for colon carcinoma in the third and fourth decades.", "score": 0.009259259259259259, "content": "In contrast to earlier studies that suggested that colon carcinoma is unusually lethal in the young, 69 patients, ages 20 to 39 years, had a relatively good prognosis. Fifty-nine percent lived over 5 years after diagnosis, and 51% were cured. Furthermore, 67% were cured if they did not have distant spread of the carcinoma at the time of the initial operation. Neither age, sex, tumor size, location, mere presence of lymph node metastases, depth of tumor invasion, nor predisposing disease of the colon was a strong prognostic factor. Metastases to six or more lymph nodes and distant spread of the tumor at the time of initial surgery were ominous findings. Mucinous carcinoma was relatively frequent (28%) and was also an ominous feature (only 5 of 20 patients cured as opposed to 26 of 43 with classical adenocarcinoma)."}, {"id": "wiki20220301en189_29035", "title": "Skin cancer in cats and dogs", "score": 0.009174311926605505, "content": "Cytology is an important tool that can help the veterinarian distinguish a tumor from inflammatory lesions. The biopsy technique used will largely depend on the tumor's size and location. Small masses are usually completely excised and sent to the pathology lab to confirm that the surrounding healthy tissues that were excised along with the tumor do not contain any cancer cells. If the tumor is larger, a small sample is removed for analysis and depending on the results, appropriate treatment is chosen. Depending on the tumor type and its level of aggressiveness, additional diagnostic tests can include blood tests to assess the pet’s overall health, chest X-rays to check for lung metastasis, and abdominal ultrasound to check for metastasis to other internal organs."}, {"id": "pubmed23n0418_19398", "title": "[Clinical and macroscopic variables that influence the prognosis of colorectal carcinoma].", "score": 0.009174311926605505, "content": "The paradoxical evolution of approximately one third of patients with neoplasms cataloged in Dukes stages B and C demonstrates the desirability of utilizing other prognostic criteria that are capable of broadening the information provided by these two important variables. Only a small number of investigators have dedicated themselves to the study of the prognostic value of clinical and macroscopic parameters of colorectal neoplasms, and the results obtained have been shown to be controversial. The principal aim of this work was to evaluate the prognostic importance of these parameters. A study was made of 320 patients with colorectal cancer who underwent curative extirpation. They had a median age of 58 years, and there were 199 females (62.2%) and 121 males (37.8%). The patients were divided into three age groups: under 40 years old, between 40 and 60 years old and over 60 years old. The tumors were distributed in three intestinal segments: right colon, left colon and rectum. The neoplasms were classified as small (diameter less than or equal to 35 mm) and large (diameter greater than 35 mm). With regard to their form, they were classified as exophytic, when characterized by luminal growth, and endophytic, when there was intramural growth. The involvement of the intestinal circumference at the site of the neoplasm was considered as partial or total. Of the 320 patients, 22 (6.9%) were aged under 40 years, 159 (49.7%) from 40 to 60 years and 139 (43.4%) presented an age of over 60 years. Seventy-three (22.8%) of the neoplasms were located in the right colon, 130 (40.6%) in the left colon and 117 (36.6%) in the rectum. Regarding the size, 280 (87.5%) were large and 40 (12.5%) small; exophytic lesions predominated over endophytic ones - 173 (54.1%) vs 147 (45.9%). A greater number of tumors presented total involvement of the intestinal circumference - 216 (67.5%) - while 104 (32.5%) presented partial involvement. The 5-year survival of the patients was not influenced by their age and sex, or by the location and size of the neoplasms. Exophytic lesions conferred greater survival on their sufferers (65.9%), in comparison with endophytic lesions (49.0%). The survival of patients with lesions partially involving the intestinal circumference was greater than for those with total involvement - 72.1% vs. 51.4%. Clinical variables had no influence on the patients' prognosis. Among the macroscopic variables, the form of the neoplasia and its involvement in the intestinal circumference did influence the patients' prognosis. These last two variables are important data capable of contributing to the identification of patient subpopulations with greater or lesser prognostic risk."}, {"id": "wiki20220301en454_6872", "title": "Pancreatic neuroendocrine tumor", "score": 0.00909090909090909, "content": "In functioning PanNETs, octreotide is usually recommended prior to biopsy or surgery but is generally avoided in insulinomas to avoid profound hypoglycemia. PanNETs in Multiple endocrine neoplasia type 1 are often multiple, and thus require different treatment and surveillance strategies. Some PanNETs are more responsive to chemotherapy than are gastroenteric carcinoid tumors. Several agents have shown activity. In well differentiated PanNETs, chemotherapy is generally reserved for when there are no other treatment options. Combinations of several medicines have been used, such as doxorubicin with streptozocin and fluorouracil (5-FU) and capecitabine with temozolomide. Although marginally effective in well-differentiated PETs, cisplatin with etoposide has some activity in poorly differentiated neuroendocrine cancers (PDNECs), particularly if the PDNEC has an extremely high Ki-67 score of over 50%."}, {"id": "pubmed23n0220_18004", "title": "[Cancers of the colon. Results of surgical treatment. Presentation of a series of 234 patients].", "score": 0.00909090909090909, "content": "This report is a retrospective analysis of the results of surgical treatment in 234 consecutive cases of adenocarcinoma of the colon; 56.4 p. 100 of patients were male with a mean age of 66. Sixty per cent of the carcinoma were situated in the sigmoid. Carcinoma was complicated in 26.1 p. 100 of cases. The tumor was confined to the bowel wall in 14.3 p. 100 of cases (stage A), involved the serosa in 36.3 p. 100 of cases (stage B), lymph nodes in 25.5 p. 100 of cases (stage C), and distal organs in 23.8 p. 100 of cases (stage D). Global operative mortality was 10 p. 100. Obvious anastomotic leakages occurred in two patients with one death. The overall five year survival rate was 35 p. 100. The stage-by-stage 5-year survival rates depended mainly on the differentiation and on the extension of the tumor: 59.2 p. 100 in patients with stage A lesions, 54.8 p. 100 in those with stage B lesions, 30.2 p. 100 in those with stage C lesions, 3.9 p. 100 in those with stage D lesions. On the other hand, survival was not significantly related to the duration of symptoms. This suggests that early diagnosis of symptomatic disease does not guarantee a better prognosis. This can only be achieved by prevention of the disease."}, {"id": "pubmed23n0516_12328", "title": "[Association between microsatellite instability and clinico-pathological characteristics in sporadic colon cancer].", "score": 0.009057971014492754, "content": "Currently, colon cancer is a leading cause of cancer death world-wide. It progresses according to three molecular pathways, named suppressor, mutador and methylator. Microsatellite instability is a hallmark of the lack of reparation, of DNA mismatches and it characterizes a subset of colon tumors (unstable tumors, MSI). MSI-H patients (high degree of microsatellite instability) seem to share clinico-pathological differences with MSS (microsatellite stable) and MSI-L (low degree of microsatellite instability) patients. In this study, associations between high degree of microsatellite instability and pathological (location, mucinous content, differentiation grade, stages T3N0, stages II and III) and clinical features (response to chemotherapy, disease-free survival and overall survival) were evaluated. 117 patients with sporadic colon cancer were classified into two populations (MSS/MSI-L and MSI-H) by using PCR and electrophoresis of seven microsatellites, according to the National Cancer Institute recommendations. MSI-H tumors tended to be located in the right colon (p = 0.022) and were of mucinous histologic type (p = 0.04). No differences in disease-free survival and overall survival between group of stage II and III patients with MSS/ MSI-L and corresponding ones with MSI-H colon cancer were found (p = 0.54, p = 0.37, respectively). Conversely, MSI-H patients with stage II colon cancer had a favourable prognosis (p = 0.027). Nevertheless, response to 5-fluorouracil (5-FU) and leucovorin was similar in MSS/ MSI-L and MSI-H groups (p = 0.38). MSI-H patients are characterized by certain pathological features; those MSI-H patients with a stage II seem to have a better prognosis than MSS/ MSI-L patients."}, {"id": "wiki20220301en149_24224", "title": "Surgical pathology", "score": 0.009009009009009009, "content": "polyps are very common. The pathologist's interpretation of a biopsy is critical to establishing the diagnosis of a benign or malignant tumor, and can differentiate between different types and grades of cancer, as well as determining the activity of specific molecular pathways in the tumor. This information is important for estimating the patient's prognosis and for choosing the best treatment to administer. Biopsies are also used to diagnose diseases other than cancer, including inflammatory, infectious, or idiopathic diseases of the skin and gastrointestinal tract, to name only a few."}, {"id": "pubmed23n0261_15612", "title": "Synchronous colorectal carcinomas.", "score": 0.009009009009009009, "content": "Eighteen (5.0%) out of 358 patients who underwent resection of a colorectal carcinoma during the period 1978 through 1990 had synchronous colorectal carcinomas, and were 5.6 years younger on average than those with a single carcinoma. The distance between synchronous lesions was less than 10 cm in 69.6% of all the patients in the study. Among the synchronous carcinomas there was a higher incidence of ascending colon involvement, mucinous carcinoma, family history of malignant diseases, multiple malignant neoplasms associated with other organs and benign neoplastic polyps of the colorectum, and it is suggested that hereditary oncogenic factors influence these carcinomas. The synchronous lesions were detected pre-operatively in 14 of 18 patients with synchronous carcinomas, and the most common reason why synchronous lesions were missed was that the lesions on the anal side prevented the lesions on the proximal side from being examined. The prognosis in the synchronous lesion group was worse than in the solitary lesion group. Since it is difficult to predict synchronous colorectal carcinomas, careful pre-operative examination, including that of other organs, is necessary, and intra-operative colonoscopy should be carried out when pre-operative examination was insufficient."}, {"id": "wiki20220301en409_14583", "title": "Pembrolizumab", "score": 0.008928571428571428, "content": "In May 2017, pembrolizumab received an accelerated approval from the US FDA for use in any unresectable or metastatic solid tumor with DNA mismatch repair deficiencies or a microsatellite instability-high state (or, in the case of colon cancer, tumors that have progressed following chemotherapy). This approval marked the first instance in which the FDA approved marketing of a drug based only on the presence of a genetic mutation, with no limitation on the site of the cancer or the kind of tissue in which it originated. The approval was based on a clinical trial of 149 patients with microsatellite instability-high or mismatch repair deficient cancers who enrolled on one of five single-arm trials. Ninety patients had colorectal cancer, and 59 patients had one of 14 other cancer types. The objective response rate for all patients was 39.6%. Response rates were similar across all cancer types, including 36% in colorectal cancer and 46% across the other tumor types. Notably, there were 11"}, {"id": "pubmed23n0122_5726", "title": "Synchronous and 'early' metachronous carcinomas of the colon and rectum.", "score": 0.008928571428571428, "content": "In a review of cases of colorectal cancer presenting to St. Mark's Hospital over the 16-year period 1970-85, 59 patients were found to have a synchronous carcinoma (3.4 per cent). Although 82 per cent of these synchronous tumours were distal to the splenic flexure (and hence within reach of a 60 cm flexible sigmoidoscope) only 42 per cent were detected pre-operatively: the remaining tumours were noted at surgery (24 per cent) or found incidentally on pathological examination of the resected specimen (34 per cent). Histological examination of these synchronous lesions revealed a high proportion with favourable stage (Dukes' A-75 per cent) and grade (well or moderate differentiation-90 per cent). Over the same 16-year period, 10 patients presented with an 'early' metachronous lesion (less than 3 years from initial surgery). Review of these cases noted negative findings on the initial barium studies in four patients and a failure to conduct full examination of the colon at initial presentation in the remaining six. It is concluded that full examination of the colon in all patients presenting with primary colorectal cancer is mandatory and that, in the light of this experience and recent reports in the literature, this should be by pre- or peroperative colonoscopy."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[240, 393]], "word_ranges": [[34, 59]], "text": "No changes should be made in the hypouricemic treatment during this period because the uric acid metabolic chain is altered and the situation may worsen."}, "2": {"exist": true, "char_ranges": [[240, 393]], "word_ranges": [[34, 59]], "text": "No changes should be made in the hypouricemic treatment during this period because the uric acid metabolic chain is altered and the situation may worsen."}, "3": {"exist": true, "char_ranges": [[0, 239]], "word_ranges": [[0, 34]], "text": "In the presence of acute gouty arthritis (the presence of intracellular crystals with negative birefringence confirms this) in a hyperuricemic patient previously treated with allopurinol, an NSAID should be added until the crisis subsides."}, "4": {"exist": true, "char_ranges": [[240, 393]], "word_ranges": [[34, 59]], "text": "No changes should be made in the hypouricemic treatment during this period because the uric acid metabolic chain is altered and the situation may worsen."}, "5": {"exist": true, "char_ranges": [[240, 393]], "word_ranges": [[34, 59]], "text": "No changes should be made in the hypouricemic treatment during this period because the uric acid metabolic chain is altered and the situation may worsen."}}, "full_answer": "In the presence of acute gouty arthritis (the presence of intracellular crystals with negative birefringence confirms this) in a hyperuricemic patient previously treated with allopurinol, an NSAID should be added until the crisis subsides. No changes should be made in the hypouricemic treatment during this period because the uric acid metabolic chain is altered and the situation may worsen.", "full_answer_no_ref": "In the presence of acute gouty arthritis (the presence of intracellular crystals with negative birefringence confirms this) in a hyperuricemic patient previously treated with allopurinol, an NSAID should be added until the crisis subsides. No changes should be made in the hypouricemic treatment during this period because the uric acid metabolic chain is altered and the situation may worsen.", "full_question": "A hyperuricemic patient who usually takes 100 mg of allopurinol daily comes to the ED with acute pain and inflammatory signs in the right knee. Arthrocentesis is performed and polarized light microscopy shows intracellular crystals with negative birefringence. Which of the following therapeutic approaches is the most appropriate in this case?", "id": 152, "lang": "en", "options": {"1": "Discontinue allopurinol and start colchicine treatment.", "2": "Discontinue allopurinol and start NSAIDs.", "3": "Add an NSAID until the crisis remits.", "4": "Increase the dose of allopurinol to 300 mg/day.", "5": "Substitute allopurinol for uricosuric acid."}, "question_id_specific": 72, "type": "RHEUMATOLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0824_19774", "title": "Does starting allopurinol prolong acute treated gout? A randomized clinical trial.", "score": 0.019327731092436976, "content": "Traditionally, allopurinol is not initiated during an acute gout attack to avoid prolonging the painful arthritis. The 2012 American College of Rheumatology Guidelines for the Management of Gout suggest that urate-lowering therapy can be started during an acute attack, based on \"consensus opinion of experts, case studies, or standard of care.\" The aim of this study was to determine whether initiating allopurinol will adversely affect the resolution of acute, treated gout. We conducted a 28-day, placebo-controlled, double-blind study of allopurinol initiation in patients with acute gout. Patients with crystal-proven gout by arthrocentesis were enrolled if they presented to the rheumatology clinic with an acute gout attack within 72 hours from initial therapy. The patients were also required to meet at least 1 additional criterion for urate-lowering therapy including (1) the presence of gouty tophi, (2) more than 1 acute gout attack per year, (3) a history of nephrolithiasis, or (4) urate overproduction (&gt;1000 mg in 24-hour urine collection). Patients were excluded from the study if they had a glomerular filtration rate of less than 50 or liver function test of greater than 1.25 times the upper limit of normal. The treating physician determined therapy for the acute gout attack. Standard prophylaxis, with colchicine or nonsteroidal anti-inflammatory drugs, was prescribed. Allopurinol or placebo was initiated at 100 mg daily for the first 14 days and then increased to 200 mg daily for the next 14 days. The primary end point was protocol defined days to resolution of acute gout, incorporating patient-rated joint pain and physician examination. Secondary measures included Physician Global Assessment, patient-rated pain, adverse effects of therapy, and serum uric acid. Thirty-one patients (17 on placebo, 14 on allopurinol) completed the study. Both intent-to-treat and completer analyses showed only a statistically insignificant difference in days to resolution (15.4 days in the allopurinol group completers vs 13.4 days in the placebo group; P = 0.5). The secondary measures revealed that the acute phase of pain rapidly improved in both groups. We initiated allopurinol at low doses during an acute gout attack in patients who met criteria for starting urate-lowering therapy and did not have abnormal kidney or liver function. In this cohort, allopurinol did not prolong the acute, treated attack."}, {"id": "pubmed23n1047_3671", "title": "[ERRORS IN THE DIAGNOSTICS AND TREATMENT OF PATIENTS WITH GOUT AND THE ALGORITHM OF THERAPEUTIC TACTICS IN DIFFERENT PERIODS OF THE DISEASE (CLINICAL CASE)].", "score": 0.019324122479462285, "content": "Difficulties and errors in the treatment of patients with the gout arise, mainly, during urate-lowering therapy. The article discusses possible medical errors in acute gouty arthritis and during chronic tophaceous gout in the light of the updated international recommendations of the American College of Rheumatology (ACR) and the European Antirheumatic League (EULAR 2018). As an example of inadequate treatment, the authors describe a case of a patient with chronic tophaceous gout. Errors in the diagnosis and treatment of the patient caused various complications and unjustified surgical intervention - amputation of the right finger and removal of a large tophus in the left forearm. Based on the analysis of mistakes made in the diagnosis and treatment of gout, the authors propose an algorithm for therapeutic tactics in different periods of the disease. So, for the relief of exacerbation in acute gouty arthritis, it is recommended to take the following drugs at starting doses: colchicine at a dose of 1.8 mg/day (1.2 mg immediately followed by 0.6 mg 1 hour later during 7-10 days or until complete relief of the gout attack), non-steroidal anti-inflammatory drugs (nimesulide up to 200 mg/day) or glucocorticosteroids (prednisolone at a dose of 30 mg/day for 3-5 days with subsequent withdrawal). The first-line urate-lowering drugs for chronic tofaceous gout are xanthine oxidase inhibitors - allopurinol and febuxostat. Allopurinol is prescribed no earlier than 2 weeks after the arthritis attack has stopped at a starting dose of no more than 100 mg/day, the dose is gradually increased to the minimum effective. The starting dose of febuxostat is 40 mg/day. Also, together with allopurinol or febuxostat, it is recommended to take uricosuric drugs (probenecid 500 mg/day or benzbromarone 50-200 mg/day). At the same time, the authors draw attention to the inadmissibility of the combination of allopurinol and febuxostat. In case of gout that does not respond to the main methods of therapy, treatment with pegloticase is recommended. When prescribing urate-lowering therapy, dose titration is necessary, to avoid the development of toxic effects."}, {"id": "pubmed23n0740_13678", "title": "Initiation of allopurinol at first medical contact for acute attacks of gout: a randomized clinical trial.", "score": 0.01905453225660103, "content": "Streamlining the initiation of allopurinol could result in a cost benefit for a common medical problem and obviate the perception that no treatment is required once acute attacks have resolved. Our objective was to test the hypothesis that there is no difference in patient daily pain or subsequent attacks with early versus delayed initiation of allopurinol for an acute gout attack. A total of 57 men with crystal-proven gout were randomized to allopurinol 300 mg daily or matching placebo for 10 days. All subjects received indomethacin 50 mg 3 times per day for 10 days, a prophylactic dose of colchicine 0.6 mg 2 times per day for 90 days, and open-label allopurinol starting at day 11. Primary outcome measures were pain on visual analogue scale (VAS) for the primary joint on days 1 to 10 and self-reported flares in any joint through day 30. On the basis of 51 evaluable subjects (allopurinol in 26, placebo in 25), mean daily VAS pain scores did not differ significantly between study groups at any point between days 1 and 10. Initial VAS pain scores for allopurinol and placebo arms were 6.72 versus 6.28 (P=.37), declining to 0.18 versus 0.27 (P=.54) at day 10, with neither group consistently having more daily pain. Subsequent flares occurred in 2 subjects taking allopurinol and 3 subjects taking placebo (P=.60). Although urate levels decreased rapidly in the allopurinol group (from 7.8 mg/dL at baseline to 5.9 mg/dL at day 3), sedimentation rates and C-reactive protein levels did not differ between groups at any point. Allopurinol initiation during an acute gout attack caused no significant difference in daily pain, recurrent flares, or inflammatory markers."}, {"id": "pubmed23n0283_18062", "title": "Gout: modern management of an ancient malady.", "score": 0.017412370353546824, "content": "If Dr. Sydenham could have benefited from today's therapy, he likely would not have had to endure thirty years of \"violent ... torture\" that gave birth to his most elegant and classic description of acute gout. The five key points to remember in management of the gouty spectrum are: (1) Establish the diagnosis as clearly as possible or as clearly as seems necessary under the clinical circumstances (i.e. arthrocentesis with crystal analysis to establish diagnosis is not always necessary with reliable patients when septic joint seems highly unlikely). (2) Treat acute attacks with NSAIDs alone or perhaps steroids--or rarely IV colchicine under special circumstances. (3) DO NOT START ALLOPURINOL OR PROBENECID DURING AN ACUTE FLARE OF GOUT--IT MAY MAKE THE EPISODE WORSE. (4) The pattern of disease over time (frequency and severity of attacks) determines whether or not one decides to use an agent such as allopurinol, probenecid, or prophylactic colchicine chronically once a patient is over the acute attack--the mere presence of increased uric acid and a single or rare gouty attack would not usually require any other than the appropriate acute therapy. (5) The presence of visible tophi, uric acid renal calculi and destructive gouty arthritis nearly always warrant uric acid lowering therapy."}, {"id": "pubmed23n0318_11898", "title": "Managing problem gout.", "score": 0.016298946531504672, "content": "For the management of acute gouty arthritis, non-steroidal anti-inflammatory drugs (NSAIDs) are the drugs of choice. In recent years, the use of colchicine has declined because of its frequent adverse reactions, and its reduced efficacy when administered more than 24 hours after onset of an acute attack. Intra-articular corticosteroid therapy (e.g. methylprednisolone acetate) is indicated for the treatment of acute mono or oligoarticular gouty arthritis in aged patients, and in those with co-morbid conditions contraindicating therapy with either NSAIDs or colchicine. Oral corticosteroids (e.g. prednisone), and both parenteral corticotrophin (ACTH) and corticosteroids (e.g. intramuscular triamcinolone acetonide) are valuable, relatively safe alternate treatment modalities in those with polyarticular attacks. For the treatment of hyperuricaemia and chronic gouty arthritis, allopurinol is the preferred urate-lowering drug. Its toxicity in elderly individuals, those with renal impairment, and in cyclosporine-treated transplant patients can be minimised by adjusting the initial dose according to the patient's creatinine clearance. In those experiencing cutaneous reactions to allopurinol, cautious desensitisation to the drug can be achieved using a schedule of gradually increasing doses. The therapeutic usefulness of uricosuric drugs is limited by the presence of renal impairment, occurrence of intolerable side-effects, or concomitant intake of salicylates. They are particularly indicated in patients allergic to allopurinol and in those with massive tophi requiring combined therapy with both allopurinol and a uricosuric."}, {"id": "pubmed23n0897_25218", "title": "Crystal arthritides - gout and calcium pyrophosphate arthritis : Part 3: Treatment.", "score": 0.01571342564720048, "content": "The treatment of gout is based on several principles. Symptom control and termination of the inflammatory process are important early goals, whereas the urate level should be lowered in the long term to prevent further gout attacks and complications. The non-pharmacological approach is based on individually informing the patient on dietary measures and changes of life style. Besides physical measures, such as cold applications on the affected joint, various medications are available for treatment of an acute gout attack. The choice of drug depends on the individual risk profile. If non-steroidal anti-inflammatory drugs (NSAID) and coxibs are chosen it should be taken into account that the use is restricted in patients with renal insufficiency. Moreover, these drugs may have gastrointestinal side effects and are associated with increased cardiovascular morbidity and mortality. Colchicine has gastrointestinal side effects at high dosages but can also be used for differential diagnostics if there is a quick response to treatment. Steroids are an effective alternative and can be given orally or parenterally in patients with dysphagia. Moreover, steroids can be used in cases of renal insufficiency. After symptoms of the acute attack have subsided, urate lowering therapy should be initiated to prevent further attacks. Low-dose urate lowering therapy can be started during an acute gout attack when acute therapy is initiated. Allopurinol is still the medication of choice but its use is restricted in patients with renal insufficiency. A rare but serious side effect is allopurinol hypersensitivity syndrome. Febuxostat can be an alternative in patients who do not tolerate allopurinol. In February 2016, lesinurad, an URAT-1 and OAT-4 inhibitor, was approved in combination with allopurinol or febuxostat. Data on the effectiveness and safety of synthetic uricases and biologicals are still sparse for elderly patients. These substances are reserved for severe cases of gout."}, {"id": "pubmed23n0809_9520", "title": "Allopurinol for chronic gout.", "score": 0.014685756395410043, "content": "Allopurinol, a xanthine oxidase inhibitor, is considered one of the most effective urate-lowering drugs and is frequently used in the treatment of chronic gout. To assess the efficacy and safety of allopurinol compared with placebo and other urate-lowering therapies for treating chronic gout. We searched the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE and EMBASE on 14 January 2014. We also handsearched the 2011 to 2012 American College of Rheumatology (ACR) and European League against Rheumatism (EULAR) abstracts, trial registers and regulatory agency drug safety databases. All randomised controlled trials (RCTs) or quasi-randomised controlled clinical trials (CCTs) that compared allopurinol with a placebo or an active therapy in adults with chronic gout. We extracted and analysed data using standard methods for Cochrane reviews. The major outcomes of interest were frequency of acute gout attacks, serum urate normalisation, pain, function, tophus regression, study participant withdrawal due to adverse events (AE) and serious adverse events (SAE). We assessed the quality of the body of evidence for these outcomes using the GRADE approach. We included 11 trials (4531 participants) that compared allopurinol (various doses) with placebo (two trials); febuxostat (four trials); benzbromarone (two trials); colchicine (one trial); probenecid (one trial); continuous versus intermittent allopurinol (one trial) and different doses of allopurinol (one trial). Only one trial was at low risk of bias in all domains. We deemed allopurinol versus placebo the main comparison, and allopurinol versus febuxostat and versus benzbromarone as the most clinically relevant active comparisons and restricted reporting to these comparisons here.Moderate-quality evidence from one trial (57 participants) indicated allopurinol 300 mg daily probably does not reduce the rate of gout attacks (2/26 with allopurinol versus 3/25 with placebo; risk ratio (RR) 0.64, 95% confidence interval (CI) 0.12 to 3.52) but increases the proportion of participants achieving a target serum urate over 30 days (25/26 with allopurinol versus 0/25 with placebo, RR 49.11, 95% CI 3.15 to 765.58; number needed to treat for an additional beneficial outcome (NNTB) 1). In two studies (453 participants), there was no significant increase in withdrawals due to AE (6% with allopurinol versus 4% with placebo, RR 1.36, 95% CI 0.61 to 3.08) or SAE (2% with allopurinol versus 1% with placebo, RR 1.93, 95% CI 0.48 to 7.80). One trial reported no difference in pain reduction or tophus regression, but did not report outcome data or measures of variance sufficiently and we could not calculate the differences between groups. Neither trial reported function.Low-quality evidence from three trials (1136 participants) indicated there may be no difference in the incidence of acute gout attacks with allopurinol up to 300 mg daily versus febuxostat 80 mg daily over eight to 24 weeks (21% with allopurinol versus 23% with febuxostat, RR 0.89, 95% CI 0.71 to 1.1); however more participants may achieve target serum urate level (four trials; 2618 participants) with febuxostat 80 mg daily versus allopurinol 300 mg daily (38% with allopurinol versus 70% with febuxostat, RR 0.56, 95% CI 0.48 to 0.65, NNTB with febuxostat 4). Two trials reported no difference in tophus regression between allopurinol and febuxostat over a 28- to 52-week period; but as the trialists did not provide variance, we could not calculate the mean difference between groups. The trials did not report pain reduction or function. Moderate-quality evidence from pooled data from three trials (2555 participants) comparing allopurinol up to 300 mg daily versus febuxostat 80 mg daily indicated no difference in the number of withdrawals due to AE (7% with allopurinol versus 8% with febuxostat, RR 0.89, 95% CI 0.62 to 1.26) or SAE (4% with allopurinol versus 4% with febuxostat, RR 1.13, 95% CI 0.71 to 1.82) over a 24- to 52-week period.Low-quality evidence from one trial (65 participants) indicated there may be no difference in the incidence of acute gout attacks with allopurinol up to 600 mg daily compared with benzbromarone up to 200 mg daily over a four-month period (0/30 with allopurinol versus 1/25 with benzbromarone, RR 0.28, 95% CI 0.01 to 6.58). Based on the pooled results of two trials (102 participants), there was moderate-quality evidence of no probable difference in the proportion of participants achieving a target serum urate level with allopurinol versus benzbromarone (58% with allopurinol versus 74% with benzbromarone, RR 0.79, 95% CI 0.56 to 1.11). Low-quality evidence from two studies indicated there may be no difference in the number of participants who withdrew due to AE with allopurinol versus benzbromarone over a four- to nine-month period (6% with allopurinol versus 7% with benzbromarone, pooled RR 0.80, 95% CI 0.18 to 3.58). There were no SAEs. They did not report tophi regression, pain and function.All other comparisons were supported by small, single studies only, limiting conclusions. Our review found low- to moderate-quality evidence indicating similar effects on withdrawals due to AEs and SAEs and incidence of acute gout attacks when allopurinol (100 to 600 mg daily) was compared with placebo, benzbromarone (100 to 200 mg daily) or febuxostat (80 mg daily). There was moderate-quality evidence of little or no difference in the proportion of participants achieving target serum urate when allopurinol was compared with benzbromarone. However, allopurinol seemed more successful than placebo and may be less successful than febuxostat (80 mg daily) in achieving a target serum urate level (6 mg/dL or less; 0.36 mmol/L or less) based on moderate- to low-quality evidence. Single studies reported no difference in pain reduction when allopurinol (300 mg daily) was compared with placebo over 10 days, and no difference in tophus regression when allopurinol (200 to 300 mg daily) was compared with febuxostat (80 mg daily). None of the trials reported on function, health-related quality of life or participant global assessment of treatment success, where further research would be useful."}, {"id": "pubmed23n0617_17753", "title": "Desensitization to allopurinol after allopurinol hypersensitivity syndrome with renal involvement in gout.", "score": 0.014516129032258063, "content": " This is a case report of a 36-year-old male with tophaceous gout for 16 years. He started therapy with 300 mg/day of allopurinol. He had received variable dexamethasone doses by self-prescription for 16 years. When allopurinol was initiated, he had hyperuricemia and normal renal function. Twenty days after starting allopurinol, he presented diffuse maculopapular rash, conjunctivitis, increase in serum creatinine values, leukocytosis and eosinophilia and the diagnosis of allopurinol hypersensitivity (AH) syndrome was made. He completely recovered from the AH and renal function normalized. However, the gout worsened over the following years in spite of treatment with benzobromarone, low doses of prednisone, and colchicine. Allopurinol desensitization was successful beginning with an oral low dose scheme (6.5 mug/day) until we reached 300mg/day. Today the patient receives allopurinol with no side effects. We believe that this is the first reported example of successful desensitization in full-blown AH with renal involvement. Our cautious regimen might be tried in other such patients."}, {"id": "wiki20220301en015_140922", "title": "Colchicine", "score": 0.014192835592572133, "content": "Medical uses Gout Colchicine is an alternative for those unable to tolerate NSAIDs when treating gout. Low doses appear to be well tolerated and may reduce gout symptoms and pain (1.2 mg in one hour, followed by 0.6 mg an hour later). This low dose may have a similar effectiveness to NSAIDS (low quality evidence). At high doses, side effects (primarily diarrhea, nausea, or vomiting) limit its use, however may be effective against pain. In addition, there is preliminary evidence that daily colchicine (0.6 mg twice daily) may be effective as a long-term prophylaxis when used with allopurinol to reduce the risk of increased uric acid levels and acute gout flares, although adverse gastrointestinal effects may occur. For treating gout symptoms, colchicine is used orally with or without food, as symptoms first appear. Subsequent doses may be needed if symptoms worsen."}, {"id": "wiki20220301en002_185653", "title": "Gout", "score": 0.014017017465293328, "content": "Treatment The initial aim of treatment is to settle the symptoms of an acute attack. Repeated attacks can be prevented by medications that reduce serum uric acid levels. Tentative evidence supports the application of ice for 20 to 30 minutes several times a day to decrease pain. Options for acute treatment include nonsteroidal anti-inflammatory drugs (NSAIDs), colchicine, and Glucocorticoids. While glucocorticoids and NSAIDs work equally well, glucocorticoids may be safer. Options for prevention include allopurinol, febuxostat, and probenecid. Lowering uric acid levels can cure the disease. Treatment of associated health problems is also important. Lifestyle interventions have been poorly studied. It is unclear whether dietary supplements have an effect in people with gout."}, {"id": "wiki20220301en002_185649", "title": "Gout", "score": 0.013615384615384616, "content": "While it has been recommended that urate-lowering measures should be increased until serum uric acid levels are below 300–360 µmol/l (5.0–6.0 mg/dl), there is little evidence to support this practice over simple putting people on a standard dose of allopurinol. If these medications are in chronic use at the time of an attack, it is recommended that they be continued. Levels that cannot be brought below 6.0 mg/dl while attacks continue indicates refractory gout. While historically it is not recommended to start allopurinol during an acute attack of gout, this practice appears acceptable. Allopurinol blocks uric acid production, and is the most commonly used agent. Long term therapy is safe and well-tolerated and can be used in people with renal impairment or urate stones, although hypersensitivity occurs in a small number of individuals."}, {"id": "pubmed23n0632_1646", "title": "Ultrasonography shows disappearance of monosodium urate crystal deposition on hyaline cartilage after sustained normouricemia is achieved.", "score": 0.013419101985175087, "content": "This study aimed at determining whether lowering serum urate (SU) to less than 6 mg/dl in patients with gout affects ultrasonographic findings. Seven joints in five patients with monosodium urate (MSU) crystal proven gout and hyperuricemia were examined over time with serial ultrasonography. Four of the five patients were treated with urate lowering drugs (ULDs) (allopurinol, n = 3; probenecid, n = 1). One patient was treated with colchicine alone. Attention was given to changes in a hyperechoic, irregular coating of the hyaline cartilage in the examined joints (double contour sign or \"urate icing\"). This coating was considered to represent precipitate of MSU crystals. Index joints included metacarpophalangeal (MCP) joints (n = 2), knee joints (n = 3), and first metatarsophalangeal (MTP) joints (n = 2). The interval between baseline and follow-up images ranged from 7 to 18 months. Serial SU levels were obtained during the follow-up period. During the follow-up period, three patients treated with ULD (allopurinol, n = 2; probenecid, n = 1) achieved a SU level of &lt;6 mg/dl. In two patients, SU levels remained above 6 mg/dl (treated with allopurinol, n = 1; treated with colchicine, n = 1). At baseline, the double contour sign was seen in all patients. In those patients who achieved SU levels of &lt;6 ml/dl, this sign had disappeared at follow-up. Disappearance of the double contour sign was seen in two knee joints, two first MTP joints, and one MCP joint. In contrast, disappearance of the double contour sign was not seen in patients who maintained a SU level &gt; or =7 mg/dl. In one patient treated with allopurinol, SU levels improved from 13 to 7 mg/dl during the follow-up period. Decrease, but not resolution of the hyperechoic coating was seen in this patient. In the patient treated with colchicine alone, SU levels remained &gt;8 mg/dl, and no sonographic change was observed. In our patients, sonographic signs of deposition of MSU crystals on the surface of hyaline cartilage disappeared completely if sustained normouricemia was achieved. This is the first report showing that characteristic sonographic changes are influenced by ULDs once SU levels remain &lt; or =6 mg/dl for 7 months or more. Sonographic changes of gout correlate with SU levels and may be a non-invasive means to track changes in the uric acid pool. Larger prospective studies are needed to further assess these potentially important findings."}, {"id": "wiki20220301en534_23030", "title": "Lesinurad/allopurinol", "score": 0.013137254901960785, "content": "Lesinurad/allopurinol (trade name Duzallo) is a fixed-dose combination drug for the treatment of gout. It contains 200 mg of lesinurad and 300 mg of allopurinol. In August 2017, the US Food and Drug Administration approved it for the treatment of hyperuricemia associated with gout in patients for whom target serum uric acid levels have not been achieved with allopurinol alone. It was approved for medical use in the European Union in August 2018. In February 2019, it was discontinued by its manufacturer for business reasons and is no longer available. References Antigout agents Combination drugs AstraZeneca brands Withdrawn drugs"}, {"id": "pubmed23n0505_18289", "title": "Management of acute and chronic gouty arthritis: present state-of-the-art.", "score": 0.013131648936170214, "content": "There are three stages in the management of gout: (i) treating the acute attack; (ii) lowering excess stores of uric acid to prevent flares of gouty arthritis and to prevent tissue deposition of urate; and (iii) providing prophylaxis to prevent acute flares. It is important to distinguish between therapy to reduce acute inflammation in acute gout and therapy to manage hyperuricaemia in patients with chronic gouty arthritis. During the acute gouty attack nonpharmacological treatments such as topical ice and rest of the inflamed joint are useful. NSAIDs are the preferred treatment in acute gout. The most important determinant of therapeutic success is not which NSAID is chosen, but rather how soon NSAID therapy is initiated. Other treatments include oral and intravenous colchicine, intra-articular and systemic corticosteroids, and intramuscular corticotropin. Optimal treatment of chronic gout requires long-standing reduction in serum uric acid. The urate-lowering drugs used to treat chronic gout are the uricosuric drugs, the uricostatic drugs, which are xanthine oxidase inhibitors, and the uricolytic drugs. Xanthine oxidase inhibitors such as allopurinol, oxipurinol and febuxastat should be used as first-line treatment in patients with renal calculi, renal insufficiency, concomitant diuretic therapy and ciclosporin (cyclosporine) therapy, and urate overproduction. Uricosuric drugs include probenecid, benzbromarone, micronised fenofibrate and losartan. They are the urate-lowering drugs of choice in allopurinol-allergic patients and underexcretors with normal renal function and no history of urolithiasis. The use of recombinant urate oxidase in patients with chronic gout is limited by the need for parenteral administration, the potential antigenicity and production of anti-urate oxidase antibodies, and declining efficacy. The effectiveness of colchicine prophylaxis as an isolated therapy is still to be confirmed by placebo-controlled trials. Another issue is prophylaxis with NSAIDs. There are no comparative studies with colchicine."}, {"id": "pubmed23n0839_14448", "title": "Adherence to the 2012 American College of Rheumatology (ACR) Guidelines for Management of Gout: A Survey of Brazilian Rheumatologists.", "score": 0.012766474831806759, "content": "To describe the current pharmacological approach to gout treatment reported by rheumatologists in Brazil. We performed a cross-sectional survey study using an online questionnaire e-mailed to 395 rheumatologists, randomly selected, from among the members of the Brazilian Society of Rheumatology. Three hundred and nine rheumatologists (78.2%) responded to the survey. For acute gout attacks, combination therapy (NSAIDs or steroid + colchicine) was often used, even in monoarticular involvement, and colchicine was commonly started as monotherapy after 36 hours or more from onset of attack. During an acute attack, urate-lowering therapy (ULT) was withdrawn by approximately a third of rheumatologists. Anti-inflammatory prophylaxis (98% colchicine) was initiated when ULT was started in most cases (92.4%), but its duration was varied. Most (70%) respondents considered the target serum uric acid level to be less than 6 mg/dl. Approximately 50% of rheumatologists reported starting allopurinol at doses of 100 mg daily or less and 42% reported the initial dose to be 300 mg daily in patients with normal renal function. ULT was maintained indefinitely in 76% of gout patients with tophi whereas in gout patients without tophi its use was kept indefinitely in 39.6%. This is the first study evaluating gout treatment in a representative, random sample of Brazilian rheumatologists describing common treatment practices among these specialists. We identified several gaps in reported gout management, mainly concerning the use of colchicine and ULT and the duration of anti-inflammatory prophylaxis and ULT. Since rheumatologists are considered as opinion leaders in this disease, a program for improving quality of care for gout patients should focus on increasing their knowledge in this common disease."}, {"id": "pubmed23n0886_3759", "title": "Management of Gout: A Systematic Review in Support of an American College of Physicians Clinical Practice Guideline.", "score": 0.012727130325814535, "content": "Gout is a common type of inflammatory arthritis in patients seen by primary care physicians. To review evidence about treatment of acute gout attacks, management of hyperuricemia to prevent attacks, and discontinuation of medications for chronic gout in adults. Multiple electronic databases from January 2010 to March 2016, reference mining, and pharmaceutical manufacturers. Studies of drugs approved by the U.S. Food and Drug Administration and commonly prescribed by primary care physicians, randomized trials for effectiveness, and trials and observational studies for adverse events. Data extraction was performed by one reviewer and checked by a second reviewer. Study quality was assessed by 2 independent reviewers. Strength-of-evidence assessment was done by group discussion. High-strength evidence from 28 trials (only 3 of which were placebo-controlled) shows that colchicine, nonsteroidal anti-inflammatory drugs (NSAIDs), and corticosteroids reduce pain in patients with acute gout. Moderate-strength evidence suggests that low-dose colchicine is as effective as high-dose colchicine and causes fewer gastrointestinal adverse events. Moderate-strength evidence suggests that urate-lowering therapy (allopurinol or febuxostat) reduces long-term risk for acute gout attacks after 1 year or more. High-strength evidence shows that prophylaxis with daily colchicine or NSAIDs reduces the risk for acute gout attacks by at least half in patients starting urate-lowering therapy, and moderate-strength evidence indicates that duration of prophylaxis should be longer than 8 weeks. Although lower urate levels reduce risk for recurrent acute attacks, treatment to a specific target level has not been tested. Few studies of acute gout treatments, no placebo-controlled trials of management of hyperuricemia lasting longer than 6 months, and few studies in primary care populations. Colchicine, NSAIDs, and corticosteroids relieve pain in adults with acute gout. Urate-lowering therapy decreases serum urate levels and reduces risk for acute gout attacks. Agency for Healthcare Research and Quality. (Protocol registration: http://effectivehealth-care.ahrq.gov/ehc/products/564/1992/Gout-managment-protocol-141103.pdf)."}, {"id": "pubmed23n0629_4293", "title": "Febuxostat: a new treatment for hyperuricaemia in gout.", "score": 0.012517602879048663, "content": "Febuxostat is a new non-purine xanthine oxidase inhibitor that is more potent than allopurinol 300 mg daily. In two Phase III trials, significantly more febuxostat-treated gout patients met the primary endpoint [serum urate (sUA) &lt;6 mg/dl (&lt;360 mumol/l) at the last three visits] (48 and 53% with 80 mg; 65 and 62% with 120 mg), compared with those receiving allopurinol 300 mg (22 and 21%; P &lt; 0.001 in both studies). Febuxostat was more effective than allopurinol in the subset with impaired renal function; no dose adjustment is required in mild-to-moderate renal impairment. Long-term extension studies confirmed the efficacy and tolerability of febuxostat. In patients who achieved the sUA target of 6 mg/dl (360 mumol/l), the incidence of gout flares fell steadily and tophi resolved in many patients. The incidence of adverse events such as dizziness, diarrhoea, headache and nausea with febuxostat was similar to allopurinol. The incidence of cardiovascular side-effects (Antiplatelet Trialists Collaboration events) was numerically higher with febuxostat than with allopurinol, but this was not statistically significant. Co-administration of febuxostat with AZA or 6-mercaptopurine is not recommended. Prophylaxis (colchicine and/or NSAIDs) against acute attacks should be used for at least the first 6 months, since early mobilization flares were observed in the clinical trials. In conclusion, febuxostat is more effective than allopurinol 300 mg daily in reducing sUA levels &lt;6 mg/dl (360 mumol/l), the target recommended by EULAR, and offers a new option for the long-term treatment of gout."}, {"id": "wiki20220301en012_92531", "title": "Allopurinol", "score": 0.012499192558620243, "content": "It is also used to treat kidney stones caused by deficient activity of adenine phosphoribosyltransferase. Tumor lysis syndrome Allopurinol was also commonly used to treat tumor lysis syndrome in chemotherapeutic treatments, as these regimens can rapidly produce severe acute hyperuricemia; however, it has gradually been replaced by urate oxidase therapy. Intravenous formulations are used in this indication when people cannot take medicine by mouth. Inflammatory bowel disease Allopurinol cotherapy is used to improve outcomes for people with inflammatory bowel disease and Crohn's disease who do not respond to thiopurine monotherapy. Cotherapy has also been shown to greatly improve hepatoxicity side effects in treatment of IBD. Cotherapy invariably requires dose reduction of the thiopurine, usually to one-third of the standard dose depending upon the patient's genetic status for thiopurine methyltransferase."}, {"id": "pubmed23n0277_19348", "title": "Preventing acute gout when starting allopurinol therapy. Colchicine or NSAIDs?", "score": 0.012043010752688172, "content": "Acute gout is a well known complication of the commencement of allopurinol therapy. Prophylaxis is needed for some months, even after serum urate levels have returned to normal. Colchicine is usually preferable to NSAIDs for this purpose, being cheaper, and better tolerated, especially in patients with peptic ulcer, gastrointestinal bleeding or dyspepsia or who are taking anticoagulants."}, {"id": "wiki20220301en002_185648", "title": "Gout", "score": 0.0118939883645766, "content": "Medications As of 2020, allopurinol is generally the recommended preventative treatment if medications are used. A number of other medications may occasionally be considered to prevent further episodes of gout, including probenecid, febuxostat, benzbromarone, and colchicine. Long term medications are not recommended until a person has had two attacks of gout, unless destructive joint changes, tophi, or urate nephropathy exist. It is not until this point that medications are cost-effective. They are not usually started until one to two weeks after an acute flare has resolved, due to theoretical concerns of worsening the attack. They are often used in combination with either an NSAID or colchicine for the first three to six months."}, {"id": "wiki20220301en002_185633", "title": "Gout", "score": 0.01185693560986497, "content": "Treatment with nonsteroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, or colchicine improves symptoms. Once the acute attack subsides, levels of uric acid can be lowered via lifestyle changes and in those with frequent attacks, allopurinol or probenecid provides long-term prevention. Taking vitamin C and eating a diet high in low-fat dairy products may be preventive. Gout affects about 1 to 2% of the Western population at some point in their lives. It has become more common in recent decades. This is believed to be due to increasing risk factors in the population, such as metabolic syndrome, longer life expectancy, and changes in diet. Older males are most commonly affected. Gout was historically known as \"the disease of kings\" or \"rich man's disease\". It has been recognized at least since the time of the ancient Egyptians. Signs and symptoms"}, {"id": "pubmed23n1063_18839", "title": "[Evaluation of a 12-week allopurinol-lowering therapy in combination with the non-steroidal anti-inflammatory drug meloxicam in patients with gout].", "score": 0.011739699149771092, "content": "To evaluate a 12-week course of combined alloturinol-lowering therapy with a prophylactic anti-inflammatory dose of movalis for the frequency of exacerbations and the quality of life of patients with gout. Allopurinol was administered orally, 1 time per day. Every 3 weeks, the dosage of the drug was increased by 50 mg to 300 mg per day under the control of the level of serum uric acid (sUA). The total daily dose of the drug movalis, used in the form of different dosage forms, was 7.515 mg. The clinical effectiveness of the treatment was evaluated after 3, 6, 9 and 12 weeks according to physical examination, the dynamics of joint pain at rest, during movement and palpation, according to the visual analogue scale (VAS) in millimeters, Likert scale, EuroQol-5D-5L questionnaire, care for oneself, habitual daily activities, the presence of anxiety and depression, assessment of satisfaction with treatment (on a scale of 1 to 5, where 1 is the complete absence of improvement or worsening, and 5 is a very good result); took into account the period of remission, as well as the time before the onset of relapse of gouty arthritis. An adverse event (AE) was recorded. On the background of treatment with movalis 7.5 mg per day more than two-thirds of patients showed no worsening of the articular syndrome with an increase in the dose of allopurinol to 300 mg per day. By the 12th week of observation, a significant difference was found between the severity of gouty arthritis characteristics in the direction of improving mobility, self-care, normal daily activities, reducing soreness, reducing anxiety and depression (p0.05). In addition, the ESR and sUA levels were significantly different initially and at the final observation point (p0.05), which indicates a positive effect on the inflammatory process. A 3-month course of combination therapy was not accompanied by significant increases in blood pressure, changes in creatinine clearance in blood serum. There were no adverse events from the gastrointestinal tract. 90.9% of patients rated the treatment result as very good. AE in the form of a skin allergic rash was observed in one patient; it did not require interruption of treatment and completely stopped without consequences after completion of the course. 12 a week-long combined therapy of the allopurinol-reducing drug with the anti-inflammatory dose movalis prevents the exacerbation of the articular syndrome and improves the quality of life of patients with gout."}, {"id": "wiki20220301en001_271444", "title": "Kidney stone disease", "score": 0.011532738095238094, "content": "Allopurinol For people with hyperuricosuria and calcium stones, allopurinol is one of the few treatments that have been shown to reduce kidney stone recurrences. Allopurinol interferes with the production of uric acid in the liver. The drug is also used in people with gout or hyperuricemia (high serum uric acid levels). Dosage is adjusted to maintain a reduced urinary excretion of uric acid. Serum uric acid level at or below 6 mg/100 ml) is often a therapeutic goal. Hyperuricemia is not necessary for the formation of uric acid stones; hyperuricosuria can occur in the presence of normal or even low serum uric acid. Some practitioners advocate adding allopurinol only in people in whom hyperuricosuria and hyperuricemia persist, despite the use of a urine-alkalinizing agent such as sodium bicarbonate or potassium citrate."}, {"id": "wiki20220301en049_51196", "title": "Mercaptopurine", "score": 0.011516595602374382, "content": "Mercaptopurine causes changes to chromosomes in animals and humans, though a study in 1990 found, \"while the carcinogenic potential of 6-MP cannot be precluded, it can be only very weak or marginal.\" Another study in 1999 noted an increased risk of developing leukemia when taking large doses of 6-MP with other cytotoxic drugs. Drug interactions Allopurinol inhibits xanthine oxidase, the enzyme that breaks down mercaptopurine. Those taking allopurinol (often used to prevent gout) are at risk for mercaptopurine toxicity. The dose should be reduced or allopurinol should be discontinued. Several published studies have demonstrated that the use of allopurinol in combination with low dose 6-MP helps reduce 6-MP levels, which are toxic to liver tissue, whilst increasing the therapeutic levels of 6-MP for some inflammatory conditions. Mechanisms of action"}, {"id": "First_Aid_Step1_515", "title": "First_Aid_Step1", "score": 0.011430477230004244, "content": "tREatmEnt Acute: NSAIDs (eg, indomethacin), glucocorticoids, colchicine. Chronic (preventive): xanthine oxidase inhibitors (eg, allopurinol, febuxostat). Previously called pseudogout. Deposition of The blue P’s—blue (when Parallel), Positive calcium pyrophosphate crystals within the birefringence, calcium Pyrophosphate, joint space. Occurs in patients > 50 years old; Pseudogout both sexes affected equally. Usually idiopathic, sometimes associated with hemochromatosis, hyperparathyroidism, joint trauma. Pain and swelling with acute inflammation (pseudogout) and/or chronic degeneration (pseudo-osteoarthritis). Most commonly affected joint is the knee. Chondrocalcinosis (cartilage calcification) on x-ray. Crystals are rhomboid and weakly ⊕ birefringent under polarized light (blue when parallel to light) A . Acute treatment: NSAIDs, colchicine, glucocorticoids. Prophylaxis: colchicine. MUSCULOSKELETAL, SKIN, AND CONNECTIVE TISSUE ` pathology SECTION III 468"}, {"id": "pubmed23n0651_7031", "title": "Management of hyperuricemia in gout: focus on febuxostat.", "score": 0.011278434362689032, "content": "Gout is the most common inflammatory arthritis in an elderly population, and can be diagnosed with absolute certainty by polarization microscopy. However, diagnosis may be challenging because atypical presentations are more common in the elderly. Management of hyperuricemia in the elderly with gout requires special consideration because of co-medication, contra-indications, and risk of adverse reactions. Urate-lowering agents include allopurinol and uricosuric agents. These also must be used sensibly in the elderly, especially when renal function impairment is present. However, if used at the lowest dose that maintains the serum urate level below 5.0 to 6.0 mg/dL (0.30 to 0.36 mmol/L), the excess urate in the body will eventually be eliminated, acute flares will no longer occur, and tophi will resolve. Febuxostat, a new xanthine oxidase inhibitor, is welcomed, as few alternatives for allopurinol are available. Its pharmacokinetics and pharmacodynamics are not significantly altered in patients with moderate renal function or hepatic impairment. Its antihyperuricemic efficacy at 80 to 120 mg/day is better than \"standard dosage\" allopurinol (300 mg/day). Long-term safety data and efficacy data on tophus diminishment and reduction of gout flares have recently become available. Febuxostat may provide an important option in patients unable to use allopurinol, or refractory to allopurinol."}, {"id": "pubmed23n0022_2964", "title": "Allopurinol-induced granulomatous hepatitis with cholangitis and a sarcoid-like reaction.", "score": 0.010764448537877044, "content": "A 36-year-old man had pain in both knees and an elevated uric acid concentration; his liver function was normal. Allopurinol therapy was started, 100 mg twice daily. After one month fever, lethargy, and severe polyarthralgia developed. On admission to our hospital liver function was abnormal, and a liver biopsy specimen showed granulomas with cholangitis and pericholangitis. He also had lymphopenia with a reduced number of T cells and granulomas in the bone marrow. One month after discontinuation of allopurinol therapy the patient was clinically well with normal liver function and a normal lymphocyte count. A repeated liver biopsy specimen showed normal liver tissue with no granulomas. The onset of the symptoms and findings shortly after the initiation of allopurinol therapy, and their disappearance after the discontinuation of therapy suggest a drug-induced hypersensitivity."}, {"id": "article-17382_13", "title": "Allopurinol -- Adverse Effects", "score": 0.010642931025096631, "content": "Due to the destabilization of intra-articular uric acid microtophi on initiating any urate-lowering therapy, there is an increased incidence of acute gouty flares, especially during the initial few months. To prevent this, patients should start an anti-inflammatory agent such as colchicine, nonsteroidal anti-inflammatory drug (NSAID), or low-dose prednisone (only in patients who cannot take colchicine or NSAIDs) before or at the same time as initiating allopurinol. [10]"}, {"id": "pubmed23n0361_16937", "title": "Clinical manifestations of gout and their management.", "score": 0.010396466278819221, "content": "Gout is an inflammatory response to deposition of monosodium urate crystals in and around joints. It is primarily a disease of adult men. In acute gout, treatment options include non-steroidal anti-inflammatory drugs (NSAIDs), colchicine and corticosteroids, administered either intra-articularly, orally or parenterally. Asymptomatic hyperuricaemia does not require specific treatment, but should prompt screening for atherosclerosis risk factors, and general lifestyle modification to reduce serum urate levels. Gout presents differently in the elderly. Both women and men are affected, attacks are frequently polyarticular and in the upper limbs, and the gout may be associated with diuretic use, hypertension and renal impairment. In patients with peptic ulcer disease, selective COX-2 inhibitors provide another treatment option. In the presence of renal impairment, allopurinol is the treatment of choice for urate lowering therapy, but doses of allopurinol and colchicine must be adjusted. Urate lowering therapy should only be used if recurrent episodes of gout occur despite aggressive attempts to reverse or control the underlying causes. It should not be introduced or discontinued during an acute episode of gout, and gout prophylaxis (NSAIDs or colchicine) should be prescribed during the introduction of urate lowering therapy."}, {"id": "pubmed23n0910_14966", "title": "Images in clinical medicine: Tophi.", "score": 0.009900990099009901, "content": "Tophi (plural of tophus, Latin for \"stone\") are stone-like deposits of monosodium urate in the soft tissues, synovial tissues, or in bones near the joints. They are pathognomonic for gout, the most common inflammatory arthritis in the United States, with an estimated lifetime prevalence of 4%. It is usually the end result of loss of the balance between uric acid production and excretion. It can be found anywhere in the body especially in areas of friction or trauma. It is usually painless and rarely to present as the initial manifestation of gout. It is diagnosed mainly clinically. Imaging is mainly used to assess the complication like bony erosions. The American College of Rheumatology (ACR) guidelines currently indicate that urate-lowering therapy should be initiated in patients with the presence of tophi visible on examination or imaging (ACR Evidence A). First-line therapy for urate lowering remains the xanthine oxidase inhibitor allopurinol. The ACR currently recommends colchicine, 0.6 mg (or 0.5 mg) once or twice daily, or low dose NSAIDs should be continued to reduce gout flare incidence for six months after resolution of the tophus. Daily prednisone ≤10 mg has been endorsed as an acceptable second-line prophylactic agent. <bAbbreviations:</b ACR: American College of Rheumatology; NSAID: non-steroidal anti-inflammatory drug."}, {"id": "wiki20220301en012_92534", "title": "Allopurinol", "score": 0.0098884652389267, "content": "Allopurinol should not be given to people who are allergic to it. Drug interactions Drug interactions are extensive, and are as follows: Azathioprine and 6-mercaptopurine: Azathioprine is metabolised to 6-mercaptopurine which in turn is inactivated by the action of xanthine oxidase - the target of allopurinol. Giving allopurinol with either of these drugs at their normal dose will lead to overdose of either drug; only one-quarter of the usual dose of 6-mercaptopurine or azathioprine should be given; Didanosine: plasma didanosine Cmax and AUC values were approximately doubled with concomitant allopurinol treatment; it should not be co-administered with allopuroinol and if it must be, the dose of should be reduced and the person should be closely monitored."}, {"id": "article-17382_11", "title": "Allopurinol -- Administration", "score": 0.00980481784522558, "content": "To prevent tumor lysis syndrome, allopurinol shall be initiated 2 to 3 days before starting chemotherapy and continued until 3 to 7 days after chemotherapy. Doses for oral allopurinol are 300 mg/m^2/day in three divided doses every 8 hours, a maximum of 800 mg daily, and IV allopurinol is 200 to 400 mg/m2 daily single doses or 2 to 3 divided doses. Allopurinol dosing is 300 mg oral daily for the prevention of recurrent uric acid or calcium nephrolithiasis."}]}}}}
{"correct_option": 5, "explanations": {"1": {"exist": true, "char_ranges": [[651, 887]], "word_ranges": [[121, 163]], "text": "ELISA is a very sensitive technique, so a negative result could leave us quite calm, however, we all know that there is no medical test that rules out anything with total certainty (unless its sensitivity is 100%, which is not the case)"}, "2": {"exist": true, "char_ranges": [[88, 267]], "word_ranges": [[16, 49]], "text": "we could directly rule out answer 2 since we know that mononucleosis can be an expression of the first phase of HIV infection, especially when we have a history of a risk contact."}, "3": {"exist": true, "char_ranges": [[311, 431]], "word_ranges": [[58, 77]], "text": "ELISA is a very sensitive but not very specific technique and confirmation with a Western-blot test is ALWAYS necessary."}, "4": {"exist": true, "char_ranges": [[311, 431]], "word_ranges": [[58, 77]], "text": "ELISA is a very sensitive but not very specific technique and confirmation with a Western-blot test is ALWAYS necessary."}, "5": {"exist": true, "char_ranges": [[1210, 1406]], "word_ranges": [[218, 254]], "text": "the answer that best fits us would be 5, in the window period a viral load may be indicated for diagnosis (positive if there are more than 10,000 copies), although this test is not done routinely."}}, "full_answer": "A simple question if we are clear about the diagnostic process of HIV. At first glance, we could directly rule out answer 2 since we know that mononucleosis can be an expression of the first phase of HIV infection, especially when we have a history of a risk contact. Answers 3 and 4 are also easily ruled out; ELISA is a very sensitive but not very specific technique and confirmation with a Western-blot test is ALWAYS necessary. Answers 1 and 5 contradict each other if you notice, so one of the two is correct. In this case, the false answer is 1, it is a very categorical answer (\"rule out\") that does not quite fit the reality; As we have said, ELISA is a very sensitive technique, so a negative result could leave us quite calm, however, we all know that there is no medical test that rules out anything with total certainty (unless its sensitivity is 100%, which is not the case) and, on the other hand, we could say that in this case we have a high clinical suspicion (the picture is suggestive, and the antecedents are there). In addition, it is only 3 weeks since the contact, so our patient is probably in the \"window period\" during which serological techniques may be unprofitable. In conclusion, the answer that best fits us would be 5, in the window period a viral load may be indicated for diagnosis (positive if there are more than 10,000 copies), although this test is not done routinely.", "full_answer_no_ref": "A simple question if we are clear about the diagnostic process of HIV. At first glance, we could directly rule out answer 2 since we know that mononucleosis can be an expression of the first phase of HIV infection, especially when we have a history of a risk contact. [HIDDEN]; ELISA is a very sensitive but not very specific technique and confirmation with a Western-blot test is ALWAYS necessary. Answers 1 and 5 contradict each other if you notice, so [HIDDEN]. In this case, [HIDDEN]; it is a very categorical answer (\"rule out\") that does not quite fit the reality; As we have said, ELISA is a very sensitive technique, so a negative result could leave us quite calm, however, we all know that there is no medical test that rules out anything with total certainty (unless its sensitivity is 100%, which is not the case) and, on the other hand, we could say that in this case we have a high clinical suspicion (the picture is suggestive, and the antecedents are there). In addition, it is only 3 weeks since the contact, so our patient is probably in the \"window period\" during which serological techniques may be unprofitable. In conclusion, [HIDDEN].", "full_question": "A 20-year-old girl comes to the clinic with an acute fever, cervical lymphadenopathy and skin rash. According to the patient, 3 weeks ago she had a sexual relationship that could be a risk for contracting the HIV virus. Indicate which of the following answers is true:", "id": 243, "lang": "en", "options": {"1": "A negative HIV-1/HIV-2 serology performed by ELISA technique rules out the possibility that the patient has been infected with the HIV virus.", "2": "The clinical process that the patient suffers from is not consistent with acute HIV infection.", "3": "If the patient's ELISA test was positive, nothing further would be necessary for the diagnosis of HIV infection.", "4": "The ELISA technique has a high sensitivity for the diagnosis of HIV infection, but its specificity is even higher.", "5": "If the ELISA test to diagnose HIV in the patient was negative, we could determine by PCR technique the viral load in blood."}, "question_id_specific": 112, "type": "INFECTIOUS DISEASES", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en015_23217", "title": "Diagnosis of HIV/AIDS", "score": 0.01778093883357041, "content": "The ELISA antibody tests were developed to provide a high level of confidence that donated blood was not infected with HIV. It is therefore not possible to conclude that blood rejected for transfusion because of a positive ELISA antibody test is in fact infected with HIV. Sometimes, retesting the donor in several months will produce a negative ELISA antibody test. This is why a confirmatory western blot is always used before reporting a \"positive\" HIV test result."}, {"id": "wiki20220301en015_23216", "title": "Diagnosis of HIV/AIDS", "score": 0.016902982468394216, "content": "If no antibodies to HIV are detected, this does not mean the person has not been infected with HIV. It may take several months after HIV infection for the antibody response to reach detectable levels, during which time rapid testing for antibodies to HIV will not be indicative of true infection status. For most people, HIV antibodies reach a detectable level after two to six weeks. Although these tests have high specificity, false positives do occur. Any positive test result should be confirmed by a lab using the western blot. Interpreting antibody tests ELISA testing alone cannot be used to diagnose HIV, even if the test suggests a high probability that antibody to HIV-1 is present. In the United States, such ELISA results are not reported as \"positive\" unless confirmed by a western blot."}, {"id": "wiki20220301en016_8858", "title": "Misconceptions about HIV/AIDS", "score": 0.0151259559154296, "content": "HIV antibody testing is unreliable Diagnosis of infection using antibody testing is a well-established technique in medicine. HIV antibody tests exceed the performance of most other infectious disease tests in both sensitivity (the ability of the screening test to give a positive finding when the person tested truly has the disease) and specificity (the ability of the test to give a negative finding when the subjects tested are free of the disease under study). Many current HIV antibody tests have sensitivity and specificity in excess of 96% and are therefore extremely reliable. While most patients with HIV show an antibody response after six weeks, window periods vary and may occasionally be as long as three months."}, {"id": "wiki20220301en015_23221", "title": "Diagnosis of HIV/AIDS", "score": 0.014840299887028858, "content": "The specificity rate given here for the inexpensive enzyme immunoassay screening tests indicates that, in 1,000 HIV test results of healthy individuals, about 15 of these results will be a false positive. Confirming the test result (i.e., by repeating the test, if this option is available) could reduce the ultimate likelihood of a false positive to about 1 result in 250,000 tests given. The sensitivity rating, likewise, indicates that, in 1,000 test results of HIV infected people, 3 will actually be a false negative result. However, based upon the HIV prevalence rates at most testing centers within the United States, the negative predictive value of these tests is extremely high, meaning that a negative test result will be correct more than 9,997 times in 10,000 (99.97% of the time). The very high negative predictive value of these tests is why the CDC recommends that a negative test result be considered conclusive evidence that an individual does not have HIV."}, {"id": "wiki20220301en015_23207", "title": "Diagnosis of HIV/AIDS", "score": 0.014550656012627011, "content": "Antibody tests may give false negative (no antibodies were detected despite the presence of HIV) results during the window period, an interval of three weeks to six months between the time of HIV infection and the production of measurable antibodies to HIV seroconversion. Most people develop detectable antibodies approximately 30 days after infection, although some seroconvert later. The vast majority of people (97%) have detectable antibodies by three months after HIV infection; a six-month window is extremely rare with modern antibody testing. During the window period, an infected person can transmit HIV to others although their HIV infection may not be detectable with an antibody test. Antiretroviral therapy during the window period can delay the formation of antibodies and extend the window period beyond 12 months. This was not the case with patients that underwent treatment with post-exposure prophylaxis (PEP). Those patients must take ELISA tests at various intervals after the"}, {"id": "pubmed23n0509_7150", "title": "[Advances in the diagnosis and treatment of acute human immunodeficiency virus type 1 (HIV-1) infection].", "score": 0.014113628191298093, "content": "According the WHO there are about 14,000 new HIV infections a day. However, in a few cases the diagnosis will be made in the acute phase of the disease. Acute HIV infection is the period between infection with the virus and complete seroconversion, defined by a positive Western blot test. This period lasts approximately 30 days and most patients (40-90%) have mild clinical manifestations (fever, rash, pharyngitis, mucosal ulcers, among others) for 2 weeks which, because they are nonspecific, can be confused with other community-acquired infections. Microbiological diagnosis is based on the absence of serum antibodies (negative ELISA test) together with a positive HIV viral load in plasma (&gt; 10,000 copies/ml). Diagnosis of acute HIV infection is important for several reasons: firstly, from the epidemiological point of view, this is the period with the highest rates of HIV transmission and identification of new HIV infections reveals the growth of the epidemic and the transmission rates of resistant HIV strains, which in Spain is about 10%; secondly, from the immunopathological point of view, this period provides a unique opportunity to study the virological, immunological and genetic mechanisms that play a role in the transmission and pathogenesis of this disease; and thirdly, therapeutically, starting antiretroviral therapy during this phase could alter the natural history of the disease. However, this is a controversial issue and currently most guidelines recommend treatment only if these patients can be included in clinical trials or if they show lasting or severe clinical manifestations."}, {"id": "wiki20220301en015_23209", "title": "Diagnosis of HIV/AIDS", "score": 0.013852813852813853, "content": "Three instances of delayed HIV seroconversion occurring in health-care workers have been reported; in these instances, the health-care workers tested negative for HIV antibodies greater than 6 months postexposure but were seropositive within 12 months after the exposure. DNA sequencing confirmed the source of infection in one instance. Two of the delayed seroconversions were associated with simultaneous exposure to hepatitis C virus (HCV). In one case, co-infection was associated with a rapidly fatal HCV disease course; however, it is not known whether HCV directly influences the risk for or course of HIV infection or is a marker for other exposure-related factors. ELISA The enzyme-linked immunosorbent assay (ELISA), or enzyme immunoassay (EIA), was the first screening test commonly employed for HIV. It has a high sensitivity."}, {"id": "wiki20220301en501_11176", "title": "Viral load monitoring for HIV", "score": 0.013834154351395732, "content": "Viral load tests can also be used to diagnose HIV infection, especially in children under 18 months born to mothers with HIV, where the presence of maternal antibodies prevents the use of antibody-based (ELISA) diagnostic tests. Pooled viral RNA testing shortens the window period to a median of 17 days (95% CI, 13-28 Days). Although it is not the standard of care to use this test for diagnosis, in communities with high HIV prevalence, this test has a significantly improved negative predictive value over 3rd and 4th generation tests for detecting acute HIV infections."}, {"id": "wiki20220301en000_219039", "title": "HIV", "score": 0.01348254526010236, "content": "HIV-1 testing is initially done using an enzyme-linked immunosorbent assay (ELISA) to detect antibodies to HIV-1. Specimens with a non-reactive result from the initial ELISA are considered HIV-negative, unless new exposure to an infected partner or partner of unknown HIV status has occurred. Specimens with a reactive ELISA result are retested in duplicate. If the result of either duplicate test is reactive, the specimen is reported as repeatedly reactive and undergoes confirmatory testing with a more specific supplemental test (e.g., a polymerase chain reaction (PCR), western blot or, less commonly, an immunofluorescence assay (IFA)). Only specimens that are repeatedly reactive by ELISA and positive by IFA or PCR or reactive by western blot are considered HIV-positive and indicative of HIV infection. Specimens that are repeatedly ELISA-reactive occasionally provide an indeterminate western blot result, which may be either an incomplete antibody response to HIV in an infected person or"}, {"id": "wiki20220301en217_33420", "title": "Subtypes of HIV", "score": 0.013362640429837414, "content": "Diagnosis HIV-2 diagnosis can be made when a patient has no symptoms but positive blood work indicating the individual has HIV. The Multispot HIV-1/HIV-2 Rapid Test is currently the only FDA approved method for such differentiation between the two viruses. Recommendations for the screening and diagnosis of HIV has always been to use enzyme immunoassays that detect HIV-1, HIV-1 group O, and HIV-2. When screening the combination, if the test is positive followed by an indeterminate HIV-1 western blot, a follow up test, such as amino acid testing, must be performed to distinguish which infection is present. According to the NIH, a differential diagnosis of HIV-2 should be considered when a person is of West African descent or has had sexual contact or shared needles with such a person. West Africa is at the highest risk as it is the origin of the virus."}, {"id": "wiki20220301en140_35317", "title": "HIV set point", "score": 0.013199614006065619, "content": "The HIV set point is the viral load or number of virions in the blood of a person infected with HIV. HIV infections are broken down into three stages: acute infection, asymptomatic infection, and AIDS. The acute infection stage refers to the first weeks after infection, where the majority of infected individuals display severe flu-like symptoms such as fever, myalgia, sore throat, swollen lymph nodes, arthralgia, fatigue, headache, and sometimes rash. At this stage, viral loads reach high levels and the number of CD4 helper T cells in the blood begins to drop. At this point, seroconversion, the development of antibodies, occurs and the CD4 T cell counts begin to recover as the immune system attempts to fight the virus, marking the HIV set point. The higher the viral load at the set point, the faster the virus will progress to AIDS; the lower the viral load at the set point, the longer the patient will remain in clinical latency, the next stage of the infection."}, {"id": "wiki20220301en108_32643", "title": "Hepatitis C and HIV coinfection", "score": 0.013133673180402152, "content": "To diagnose an individual with HIV, a test must be taken to determine if the virus is present in their system. There are several test options including ELISA, at-home, saliva, viral load, and western blot. To establish the presence of the HIV virus, some tests measure the level of HIV antibodies in the blood and/or saliva or the level of both HIV antigens and antibodies in the blood. Other tests can detect the presence of the HIV virus by calculating the amount of actual virus present in the blood. None of the tests available can determine if a person is positive immediately after they believe they have been exposed to the virus. Each test has a window of time after the initial exposure occurred until the test can accurately tell if an individual has been infected or not. One reason for this is because the focus of some of the tests are antibodies. After the initial exposure to the virus, it usually takes 3–4 weeks but it can take up to six months for antibodies to be produced by the"}, {"id": "wiki20220301en448_8278", "title": "HIV and pregnancy", "score": 0.012895002075057806, "content": "The most updated HIV testing protocols recommend using the HIV-1 and HIV-2 antigen/antibody combination immunoassay as the initial screening test for an HIV infection. This blood test assesses whether or not the mother has created antibodies, which are disease-fighting proteins of the immune system, against the HIV-1 and HIV-2 viruses. These antibodies will only be present if the patient has been exposed to HIV, therefore, they act as a marker of infection. This test also detects a protein called p24 in maternal blood, which is a specific component of the HIV virus itself and also acts as an early marker of an HIV infection. If this test is positive, the CDC recommends performing follow-up testing using a test called the HIV-1/HIV-2 antibody differentiation immunoassay that both confirms the diagnosis and determines the specific type of HIV infection the patient has to specifically tailor further management of the patient."}, {"id": "wiki20220301en015_23219", "title": "Diagnosis of HIV/AIDS", "score": 0.01265167209233501, "content": "HIV antibody tests are highly sensitive, meaning they react preferentially with HIV antibodies, but not all positive or inconclusive HIV ELISA tests mean the person is infected by HIV. Risk history, and clinical judgement should be included in the assessment, and a confirmation test (western blot) should be administered. An individual with an inconclusive test should be re-tested at a later date."}, {"id": "wiki20220301en015_23196", "title": "Diagnosis of HIV/AIDS", "score": 0.012136752136752138, "content": "HIV tests are used to detect the presence of the human immunodeficiency virus (HIV), the virus that causes acquired immunodeficiency syndrome (AIDS), in serum, saliva, or urine. Such tests may detect antibodies, antigens, or RNA. AIDS diagnosis AIDS is diagnosed separately from HIV. Terminology The window period is the time from infection until a test can detect any change. The average window period with HIV-1 antibody tests is 25 days for subtype B. Antigen testing cuts the window period to approximately 16 days and nucleic acid testing (NAT) further reduces this period to 12 days. Performance of medical tests is often described in terms of: Sensitivity: The percentage of the results that will be positive when HIV is present Specificity: The percentage of the results that will be negative when HIV is not present."}, {"id": "wiki20220301en015_23199", "title": "Diagnosis of HIV/AIDS", "score": 0.012135614702154626, "content": "Diagnosis of HIV infection Tests used for the diagnosis of HIV infection in a particular person require a high degree of both sensitivity and specificity. In the United States, this is achieved using an algorithm combining two tests for HIV antibodies. If antibodies are detected by an initial test based on the ELISA method, then a second test using the western blot procedure determines the size of the antigens in the test kit binding to the antibodies. The combination of these two methods is highly accurate Human rights The UNAIDS/WHO policy statement on HIV Testing states that conditions under which people undergo HIV testing must be anchored in a human rights approach that pays due respect to ethical principles. According to these principles, the conduct of HIV testing of individuals must be Confidential; Accompanied by counseling (for those who test positive); Conducted with the informed consent of the person being tested. Confidentiality"}, {"id": "pubmed23n0367_22040", "title": "Anti-HIV-1/2 Antibody Detection by Dot-ELISA in Oral Fluid of HIV Positive/AIDS Patients and Voluntary Blood Donors.", "score": 0.012093425030731918, "content": "Serology is the primary means for identifying patients with HIV infection and Acquired Immunodeficiency Syndrome (AIDS). Testing of serum by serologic methods has been extensively used since 1985, not only for clinical diagnosis but also for epidemiological surveillance and donor screening in blood banks. Fast serological diagnostic techniques are now being developed, using urine and oral fluid, as an alternative for anti-HIV antibody screening, and many parallel studies are proving its accuracy. The purpose of this study was to evaluate the sensitivity, specificity, accuracy, positive predictive value (PPV) and negative predictive value (NPV) of the ImmunoComb II HIV 1&amp;2 Saliva((R)) test from Orgenics (Dot-ELISA) compared to the routine exams (ELISA and Western Blot) of HIV positive/AIDS patients, undergoing antiretroviral treatment or not, in different stages of the disease's evolution, and compared to serologic testing of known HIV negative patients by the use of serum ELISA (blood donors). To accomplish this, patients of the Immunogenic Deficiencies Control Center (CCDI) and voluntary blood donors of the Blood Bank Center of the Medical School of S&amp;atilde:o Paulo/Federal University of São Paulo (EPM-UN I FESP) were evaluated. Sensitivity of Dot-ELISA in oral fluid was 100%, specificity 97.08%, PPV 96.66% and NPV 100%. The method used in this case study was shown to be highly sensitive and specific, being useful particularly in epidemiological surveillance and screening."}, {"id": "article-22927_12", "title": "HIV Testing -- Diagnostic Tests -- Screening Procedure", "score": 0.011974557522123893, "content": "In the non-clinical setting, oral swab tests are still primarily enzyme-linked immunosorbent assay (ELISA) antibody tests performed as rapid tests but need to be confirmed with a serum western blot. An advantage of rapid tests is that they can be offered in a non-clinical setting such as community health fairs, places of worship, HIV service centers, and other locations outside healthcare facilities. Results are presented within 20 minutes, which decreases the number of patients who do not know the outcome of their test because they do not follow up for a return appointment, as can occur in the clinical setting. ELISA tests are Ab-only tests and detect HIV as early as 3 weeks after transmission. [5]"}, {"id": "wiki20220301en218_28749", "title": "Blood donation restrictions on men who have sex with men", "score": 0.011965171260523584, "content": "In 1985, early tests using the ELISA method looked for antibodies, which are the immune system's response to the virus. However, there is a window period when using this method in which a person who has been infected with HIV is able to spread the disease but may test negative for the virus. This window period can be as long as three to six months, with an average of 22 days. Tests using the ELISA methods are often still used in developed countries because of their ease-of-use, as well as their fairly high sensitivity, which boasts 100% sensitivity. To cover the window period resultant from the use of these tests, donors are also screened for high risk behaviors, one of which is a history of same-sex sexual activity among male potential donors. Newer tests look for the virus itself, such as the p24 antigen test, which looks for a part of the virus on the surface of infected cells, and Nucleic acid tests (NAT), which look for the genetic material of the virus in HIV-infected cells."}, {"id": "wiki20220301en099_37790", "title": "HIV/AIDS", "score": 0.011888906935195712, "content": "Diagnosis HIV/AIDS is diagnosed via laboratory testing and then staged based on the presence of certain signs or symptoms. HIV screening is recommended by the United States Preventive Services Task Force for all people 15 years to 65 years of age, including all pregnant women. Additionally, testing is recommended for those at high risk, which includes anyone diagnosed with a sexually transmitted illness. In many areas of the world, a third of HIV carriers only discover they are infected at an advanced stage of the disease when AIDS or severe immunodeficiency has become apparent. HIV testing Most people infected with HIV develop specific antibodies (i.e. seroconvert) within three to twelve weeks after the initial infection. Diagnosis of primary HIV before seroconversion is done by measuring HIV-RNA or p24 antigen. Positive results obtained by antibody or PCR testing are confirmed either by a different antibody or by PCR."}, {"id": "wiki20220301en501_11179", "title": "Viral load monitoring for HIV", "score": 0.01170076726342711, "content": "On June 15, 2010, the FDA approved the first diagnostic test capable of detecting HIV antigens and HIV antibodies. The Abbott ARCHITECT HIV Ag/Ab combo test, a fourth-generation test, has an increased sensitivity for detecting infections during the acute phase (when compared to 1st and 3rd generation tests), when the immune system is still developing antibodies and the virus is replicating unchecked, and in one study, was able to detect 83% of such infections. Society and culture A person, who may be unaware of the infection, is highly infectious during this time yet may test negative for HIV using tests that detect anti-HIV antibodies only. Although Nucleic Acid Amplification Testing NAAT is more expensive and can take a week for processing, some have argued that it may still be a preferred way to screen for HIV."}, {"id": "wiki20220301en015_23213", "title": "Diagnosis of HIV/AIDS", "score": 0.011496503496503496, "content": "There are no universal criteria for interpreting the western blot test: The number of viral bands that must be present may vary. If no viral bands are detected, the result is negative. If at least one viral band for each of the GAG, POL, and ENV gene-product groups are present, the result is positive. The three-gene-product approach to western blot interpretation has not been adopted for public health or clinical practice. Tests in which less than the required number of viral bands are detected are reported as indeterminate: a person who has an indeterminate result should be retested, as later tests may be more conclusive. Almost all HIV-infected persons with indeterminate western blot results will develop a positive result when tested in one month; persistently indeterminate results over a period of six months suggests the results are not due to HIV infection. In a generally healthy low-risk population, indeterminate results on western blot occur on the order of 1 in 5,000 patients."}, {"id": "wiki20220301en004_26482", "title": "Blood transfusion", "score": 0.011424428778561072, "content": "Since the advent of HIV testing of donor blood in the mid/later 1980s, ex. 1985's ELISA, the transmission of HIV during transfusion has dropped dramatically. Prior testing of donor blood only included testing for antibodies to HIV. However, because of latent infection (the \"window period\" in which an individual is infectious, but has not had time to develop antibodies) many cases of HIV seropositive blood were missed. The development of a nucleic acid test for the HIV-1 RNA has dramatically lowered the rate of donor blood seropositivity to about 1 in 3 million units. As transmittance of HIV does not necessarily mean HIV infection, the latter could still occur at an even lower rate. The transmission of hepatitis C via transfusion currently stands at a rate of about 1 in 2 million units. As with HIV, this low rate has been attributed to the ability to screen for both antibodies as well as viral RNA nucleic acid testing in donor blood."}, {"id": "pubmed23n0508_9929", "title": "Evaluation of the WHO human immunodeficiency virus (HIV) antibody testing strategy for the diagnosis of HIV infection.", "score": 0.011348175633889918, "content": "To evaluate a WHO testing strategy based on the use of two consecutive enzyme-linked immunosorbent assays (ELISA) as an alternative to ELISA followed by Western blotting (WB) for the serologic diagnosis of HIV infection. The study was of 2069 consecutive serum specimens from patients suspected of HIV infection received for HIV diagnostic testing at the HIV laboratory, Muhimbili Medical Centre, Dar es Salaam. The strategy involved testing all sera with Behring indirect anti-HIV 1 + 2 peptide ELISA, followed by Wellcozyme anti-HIV-1 recombinant competitive ELISA on those sera reactive by the first ELISA. WB was done on a sample of the sera reactive on both ELISAs and on all those giving discordant results on the two ELISAs. Of the 2069 sera tested, 837 (40.5%) were negative on the first ELISA, 1172 (56.6%) were reactive on both ELISAs and 60 (2.9%) were initially reactive on the first test but not on the second assay. Of the 1172 sera reactive on both ELISAs, 329 (28.1%) were tested by WB. The diagnostic accuracy of the WHO alternative testing strategy using WB confirmation as the 'gold' standard was as follows: sensitivity 99.4% (326/328), specificity 99.7%, (893/896), positive predictive value 99.1% (328/331) and negative predictive value 99.8% (893/895). Repeated testing by ELISA of the sera which initially gave discordant results on the two ELISAs increased the sensitivity to 100%. Three sera giving false positive reactions on both ELISAs became negative on both ELISAs after retesting. In order to achieve a specificity and a positive predictive value of 100%, it would have been necessary to subject all sera reacting on both ELISAs to retesting on one ELISA. A second ELISA based on different antigens and a different test principle compared with the first ELISA could be used as an alternative to the WB assay for confirmation of HIV antibodies. However, some modifications of the WHO strategy for diagnostic HIV antibody testing were required in order to maximize the diagnostic accuracy."}, {"id": "wiki20220301en501_11180", "title": "Viral load monitoring for HIV", "score": 0.010961592374544436, "content": "Contagiousness The higher the viral load value, the more viral elements there are in blood and other body fluids. For example, individuals with HIV are most contagious during the earliest (acute) stages of the infection, sometimes with millions of copies of HIV per centiliter of blood. According to one estimate, the majority of transmissions among gay men in the UK occur during primary infection. This is because, at this phase, the immune response is still developing. Antibody levels against the virus during acute infection are often too low to be detected, meaning that an antibody test for a highly infectious individual can come back negative."}, {"id": "wiki20220301en000_219042", "title": "HIV", "score": 0.010935917835961232, "content": "The latest recommendations of the US Centers for Disease Control and Prevention (CDC) show that HIV testing must start with an immunoassay combination test for HIV-1 and HIV-2 antibodies and p24 antigen. A negative result rules out HIV exposure, while a positive one must be followed by an HIV-1/2 antibody differentiation immunoassay to detect which antibodies are present. This gives rise to four possible scenarios: 1. HIV-1 (+) & HIV-2 (−): HIV-1 antibodies detected 2. HIV-1 (−) & HIV-2 (+): HIV-2 antibodies detected 3. HIV-1 (+) & HIV-2 (+): both HIV-1 and HIV-2 antibodies detected 4. HIV-1 (−) or indeterminate & HIV-2 (−): Nucleic acid test must be carried out to detect the acute infection of HIV-1 or its absence. Research HIV/AIDS research includes all medical research that attempts to prevent, treat, or cure HIV/AIDS, as well as fundamental research about the nature of HIV as an infectious agent and AIDS as the disease caused by HIV."}, {"id": "InternalMed_Harrison_15089", "title": "InternalMed_Harrison", "score": 0.010925556663343303, "content": "antibodies to HIV, an series of assay, HIV-1 RNA assay, or HIV-1 DNA PCR and specific serologic point-of-care tests can provide results in 1–60 min. Among the most testing for HIV-2. If the p24 and HIV RNA assays are negative and popular of these is the OraQuick Rapid HIV-1 antibody test that can be run on blood, plasma, or saliva. The sensitivity and specificity of this test is ~99% when run on whole blood. Specificity remains the SEROLOGIC TESTS IN THE DIAGNOSIS OF HIV-1 OR same but sensitivity drops to 98% when the test is run on saliva. While"}, {"id": "InternalMed_Harrison_15079", "title": "InternalMed_Harrison", "score": 0.010740314537782892, "content": "The standard blood screening tests for HIV infection are based on the detection of antibodies to HIV. A common platform is the ELISA, also referred to as an enzyme immunoassay (EIA). This solid-phase assay is an extremely good screening test with a sensitivity of >99.5%. Most diagnostic laboratories use commercial kits that contain antigens from both HIV-1 and HIV-2 and thus are able to detect antibodies to either. These kits use both natural and recombinant antigens and are continuously updated to increase their sensitivity to newly discovered species, such as group O viruses (Fig. 226-1). The fourth-generation EIA tests combine detection of antibodies to HIV with detection of the p24 antigen of HIV. EIA tests are generally scored as positive (highly reactive), negative (nonreactive), or indeterminate (partially reactive). While the EIA is an extremely sensitive test, it is not optimal with regard to specificity. This is particularly true in studies of low-risk individuals, such as"}, {"id": "pubmed23n0619_4406", "title": "RT-PCR detection of HIV in Republic of Macedonia.", "score": 0.010552011969446412, "content": "The aim of the study was to detect HIV RNA in seropositive patients using RT-PCR method and thus, to establish PCR methodology in the routine laboratory works. The total of 33 examined persons were divided in two groups: 1) 13 persons seropositive for HIV; and 2) 20 healthy persons - randomly selected blood donors that made the case control group. The subjects age was between 25 and 52 years (average 38,5). ELFA test for combined detection of HIV p24 antigen and anti HIV-1+2 IgG and ELISA test for detection of antibodies against HIV-1 and HIV-2, were performed for each examined person. RNA from the whole blood was extracted using a commercial kit based on salt precipitation. Detection of HIV RNA was performed using RT-PCR kit. Following nested PCR, the product was separated by electrophoresis in 1,5 % agarose gel. The result was scored positive if the band of 210bp was visible regardless of intensity. Measures of precaution were taken during all the steps of the work and HIV infected materials were disposed of accordingly. In the group of blood donors ELFA, ELISA and RT-PCR were negative. Assuming that prevalence of HIV infection is zero, the clinical specificity of RT-PCR is 100 %. The analytical specificity of RT-PCR method was tested against Hepatitis C and B, Human Papiloma Virus, Cytomegalovirus, Herpes Simplex Virus, Rubella Virus, Mycobacterium tuberculosis, Chlamydia trachomatis. None of these templates yielded amplicon. In the group of 13 seropositive persons, 33 samples were analyzed. HIV RNA was detected in 15 samples. ELISA and ELFA test were positive in all samples. Different aliquots of the samples were tested independently and showed the same results. After different periods of storing the RNA samples at -70 masculineC, RT-PCR reaction was identical to the one performed initially. The obtained amplicons were maintained frozen at -20 masculineC for a week and the subsequently performed electrophoresis was identical to the previous one. The reaction is fast, simple for manipulation; with low detection level of 60 IU/ml. RT-PCR needs a small amount of RNA, as well as a small volume of sample. HIV RNA was detected in different periods of time with different clinical presentations in patients, with or without antiretroviral therapy. RT-PCR method gives the opportunity for reliable determination of HIV-1 RNA with border of detection of 60 IU/ml. The test is reproducible and has high analytical and clinical specificity."}, {"id": "wiki20220301en015_23214", "title": "Diagnosis of HIV/AIDS", "score": 0.010297600659046443, "content": "a period of six months suggests the results are not due to HIV infection. In a generally healthy low-risk population, indeterminate results on western blot occur on the order of 1 in 5,000 patients. However, for those individuals who have had high-risk exposures to individuals where HIV-2 is most prevalent, Western Africa, an inconclusive western blot test may prove infection with HIV-2."}, {"id": "wiki20220301en015_23235", "title": "Diagnosis of HIV/AIDS", "score": 0.010006780295059737, "content": "AIDS denialism HIV tests have been criticized by AIDS denialists (a fringe group whose members believe that HIV either does not exist or is harmless). The accuracy of serologic testing has been verified by isolation and culture of HIV and by detection of HIV RNA by PCR, which are widely accepted \"gold standards\" in microbiology. While AIDS denialists focus on individual components of HIV testing, the combination of ELISA and western blot used for the diagnosis of HIV is remarkably accurate, with very low false-positive and -negative rates as described above. The views of AIDS denialists are based on highly selective analysis of mostly outdated scientific papers; there is broad scientific consensus that HIV is the cause of AIDS."}, {"id": "pubmed23n0606_5568", "title": "Evaluation of Determine HIV-1/2 rapid diagnostic test by 4th generation ELISA using blood donors' serum at Felege Hiwot Referral Hospital, northwest Ethiopia.", "score": 0.009900990099009901, "content": "The study aims to evaluate the HIV-1/2 rapid diagnostic test kit is routinely used to screen HIV infection for safe blood transfusion and VCT services in many parts of Ethiopia. A total of 324 sera were collected from consecutive blood donors from February to May 2006. All samples were screened for HIV infection using Determine HIV-1/2 (Abbott Japan) at hospital blood bank laboratory. Blindly, all serums were retested at Regional Health Research Laboratory using 4th generation ELISA (Vironostika HIV Uni-Form II AG/Ab) and Determine HIV-1/2 (Abbott lab). Discordant samples were repeatedly retested using the same ELISA and Determine HIV-1/2 to avoid technical errors. Finally, discordant results were resolved using Western Blot at the National HIV/AIDS Laboratory. Determine HIV-1/2 and ELISA showed 94.4% concordance in HIV antibody testing with fair Cohen's Kappa statistic value (0.68) among blood donors. The sensitivity, specificity, positive and negative predictive values of Determine HIV-1/2 were 60.5%, 98.9%, 88.5% and 94.9% respectively. As a rapid HIV screening test for blood donors, Determine HIV-1/2 showed poor sensitivity. Further evaluation at multiple centres is recommended to test its validity as a routine HIV screening test in blood donors. Use of a combination of rapid assays is also recommended for screening of HIV infection among the donor population."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[251, 440]], "word_ranges": [[36, 66]], "text": "In our environment the most frequent cause is surgery of the split gland with a percentage of occurrence between 10 and 80%. Conservative treatment with botulinum toxin offers good results."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "You are asking about auriculotemporal syndrome or Frey's syndrome. It is the clinical expression of a vegetative neuropathy caused by mechanical or irritative lesion of the vegetative fibers of the auriculotemporal nerve in its infratemporal pathway. In our environment the most frequent cause is surgery of the split gland with a percentage of occurrence between 10 and 80%. Conservative treatment with botulinum toxin offers good results.", "full_answer_no_ref": "You are asking about auriculotemporal syndrome or Frey's syndrome. It is the clinical expression of a vegetative neuropathy caused by mechanical or irritative lesion of the vegetative fibers of the auriculotemporal nerve in its infratemporal pathway. In our environment the most frequent cause is surgery of the split gland with a percentage of occurrence between 10 and 80%. Conservative treatment with botulinum toxin offers good results.", "full_question": "A 47-year-old man, with a history of a right parotid pleomorphic adenoma, treated with surgery (extrafacial parotidectomy) 6 months ago, who comes to our office for presenting pain with sweating and reddening of the skin in the preauricular region during mastication. What treatment would be the treatment of choice?", "id": 458, "lang": "en", "options": {"1": "Extended total parotidectomy on suspicion of tumor recurrence.", "2": "Pregabalin.", "3": "Intradermal botulinum toxin injection.", "4": "Broad-spectrum antibiotherapy.", "5": NaN}, "question_id_specific": 126, "type": "OTOLARYNGOLOGY AND MAXILLOFACIAL SURGERY", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0680_6897", "title": "[Thirteen years' experience with superficial partial parotidectomy as treatment for benign parotid tumours].", "score": 0.0177750058470414, "content": "Most authors agree that surgery is the treatment of choice for benign tumours of the parotid gland. However, the best surgical technique and the extent of surgery remain controversial. This study attempts to establish whether the implementation of a partial superficial parotidectomy (PSP) is appropriate for the treatment of benign parotid gland tumours. We selected 63 patients with benign parotid gland surgery, of whom 43 had a pleomorphic adenoma and 20, a Warthin tumour. Of this group of 63 patients, 6 could not be included. We consequently studied 57 patients, 41 of them diagnosed as pleomorphic adenoma and 16, as Warthin tumours. In all of them, a PSP was performed without intraoperative monitoring of the facial nerve. Transient facial nerve paralysis, 14 patients (24.5%). Ten cases were resolved within the first month after surgery and 4 before the third month, after indicating facial physiotherapy. One patient (1.7%) had a permanent difficulty in keeping one side of the lower lip aligned under pressure-mobility, without altering commissure mobility. None of the patients studied had a recurrence (control, 3-13 years). Although PSP is a technique with a few complications, it has a recurrence rate comparable to or lower than other techniques used for the treatment of pleomorphic adenomas or Warthin parotid tumours. Intraoperative facial nerve monitoring can be helpful during surgery. The lack of monitoring would not be considered a contraindication for surgery."}, {"id": "pubmed23n0347_17139", "title": "Botulinum toxoid in the management of gustatory sweating (Frey's syndrome) after superficial parotidectomy.", "score": 0.01709901351845086, "content": "Botulinum toxin has been successfully used to treat Frey's syndrome occurring in a 31-year-old patient following superficial parotidectomy for pleomorphic adenoma. An initial injection of 7.5 U (0.3 ml over 6 cm2 of cheek) resulted in 3 months' resolution of gustatory sweating and flushing and a second injection 12 months' symptomatic improvement. The symptoms recurred after further facial surgery."}, {"id": "pubmed23n0585_13388", "title": "[Frey syndrome secondary to submaxillectomy and botulinic treatment].", "score": 0.01661220043572985, "content": "A case of Frey syndrome (FS) secondary to submaxillar gland exeresis is presented and the results of the treatment with botulinum toxin (BTX) type A. FS is a condition of sweating cheek and preauricular area during realtime as a sequel detected in about 20-60% of patients after parotidectomy. The clinical symptoms include swelling, flushing and hyperhidrosis. The treatment choice for this condition is intracutaneous injection of BTX type A which blocks acetylcholine release at the sweat glands. A 30-year-old man, with thyroid medullar carcinoma diagnosed in 2002 received 6 cicles of cisplatin plus doxorubicin previous to the thyroidectomy with anterolateral neck dissection. During surgery the left ramus marginalis mandibulae was damaged. Two years later the patient referred sweating in submaxillar region during meals. CT scan demonstrated the absence of left submaxillar gland. Minor's test disclosed the affected area and BTX type A was injected (2.5 U/cm2/17 points). A twenty-one-day control showed a 95% reduction of the affected skin area. Persistent efficacy was observed up to one year follow-up time when he was reinjected. The FS, also known as \"gustatory hyperhidrosis\", was probably first reported by M. Duphenix in 1757. Lucja Frey considered its physiopathology as a disorder of both sympathetic and parasympathetic innervation. In our case the FS was caused by a misdirected regeneration of postganglionic parasympathetic nerve fibers that arrised from the nervus lingualis rami ganglionares of the nervus trigeminus. After nerve injury the colinergic parasympathetic fibers seek out colinergic receptors--sympathetic receptors of the skin--innervating sweat glands and small skin vessels. All previous cases were located at masseter region post-parotidectomy. We have not found any description of FS in the submaxillary region. The self-assessed efficacy of the treatment with a hyperhidrosis disease severity scale revealed a very satisfied patient at 20 months follow-up after being injected twice with BTX type A."}, {"id": "pubmed23n0055_18124", "title": "[Treatment of recurrence of pleomorphic adenomas of the parotid gland].", "score": 0.016504329004329004, "content": "This is a retrospective study of 10 patients who underwent surgery for a first or multiple recurrence of pleomorphic adenoma (P.A.). Recurrence may come more than 10 years after an initial episode. During initial surgery, the risk of recurrence is related to pre-operative dissemination and to failure to identify tumoral prolongations in the parotid gland. Recurrence is generally multifocal. In 2 cases, carcinoma developed in association with P.A. Treatment of benign recurrence is surgical: it consists of totalizing the previous parotidectomy. If the previous parotidectomy has been total, tumorectomy is carried out. In all cases, surgery preserves the facial nerve trunk."}, {"id": "pubmed23n0376_14673", "title": "[Frey syndrome after lateral parotidectomy. Follow-up and therapeutic outlook].", "score": 0.01647457627118644, "content": "Gustatory sweating is a common complication of parotid surgery. In order to evaluate the incidence of Frey's syndrome following superficial parotidectomy, 69 patients who underwent surgery due to adenoma were studied. Forty-three patients (62%) suffered from gustatory sweating following superficial parotidectomy, and 33 of them requested treatment. Nineteen patients felt that their quality of life had been decreased by the symptoms. Minor's starch iodine test proved that 85% of the patients who did not notice Frey's syndrome after surgery actually had a subclinical manifestation. Eight patients were successfully treated with intracutaneous injections of botulinum toxin A. Within 1 week gustatory sweating disappeared. Frey's syndrome is present in almost all patients following superficial parotidectomy and there is a strong need for treatment. Intracutaneous injection of botulinum toxin A is an effective treatment in severe cases of the syndrome."}, {"id": "wiki20220301en421_8393", "title": "Parotidectomy", "score": 0.01601510169285105, "content": "Complications Complications that may occur due to parotidectomy involve nerve dysfunction, Frey’s syndrome (uncharacteristic sweating near glands), salivation from wound, numbness, facial asymmetry, necrosis (death of skin) near incision, and tumor reappearance. Prognosis There is a 25-50% risk of facial weakness directly after parotidectomy and a 1-2% risk of permanent weakness. Frey’s syndrome may occur in up to 90% of patients. Risk of mortality is very low in reference to the surgery. In a case of benign tumor, such as pleomorphic adenoma, a significant outcome is also the rate of tumor recurrence. Pleomorphic adenomas may recur after a very long time from primary surgery, on average over 7–10 years and up to 24 years. Survival rates due to malignancy depends on the patient and extent of disease. A 10-year survival ranges from 32-83%. Of all cancers, salivary gland tumors account for only 1%. Parotid tumors account for 7% of all head and neck cancers. Etymology"}, {"id": "pubmed23n0297_7224", "title": "[Development and results of surgical treatment of pleomorphic adenoma of the parotid gland in 245 patients, 1974-1994].", "score": 0.015899949723479134, "content": "To describe the results of parotidectomy for pleomorphic adenoma of the parotid gland in 245 patients, 1974-1994. Descriptive. Academic Medical Hospital, Free University, Amsterdam, the Netherlands. Of all patients follow-up data were obtained by yearly physical diagnostic examination during 10 years, and by a questionnaire (1995) from the general practitioners involved. In the period 1974-1994, 246 primary surgical parotid procedures were performed on 245 patients for pleomorphic adenoma. The surgical procedures included: 131 'partial' and 61 'complete' superficial parotidectomies, 30 partial superficial/ deep lobe parotidectomies, 8 total parotidectomies, and 16 'selective' deep lobe parotidectomies. Eleven patients received postoperative radiotherapy for different reasons. The median follow-up was 95 months. Fourteen patients died without recurrent tumour. Two patients (0.8%) developed a local recurrence, both after total parotidectomy for a deep lobe tumour. None of the patients experienced permanent facial nerve paresis or paralysis. The incidence of auriculotemporal sweating for partial superficial parotidectomy proved to be 6.9% (9/I31) as compared with 13.1% (8/61) for complete superficial parotidectomy. In the later years, in the majority of patients, the posterior branch of the greater auricular nerve was preserved. In the hands of the experienced head and neck surgeon partial parotidectomy is an effective treatment for the great majority of pleomorphic adenomas: local recurrence is rare, while in general morbidity is minimal. Rarely there is a need for prolonged follow-up."}, {"id": "pubmed23n0299_15861", "title": "[Reoperation and recurrence of pleomorphic adenoma of the parotid. A propos of 62 cases].", "score": 0.015194244604316547, "content": "Between 1967 and 1994, 344 patients were treated with total conservative parotidectomy for benign pleomorphic adenoma of the parotid gland. Our retrospective study focuses on a sixty-two patients group treated for recurrence after biopsy, enucleation or total parotidectomy. Twenty-two patients underwent a systematic total parotidectomy after biopsy (n = 7) or enucleation (n = 15). Twenty-nine patients were treated with total parotidectomy for local recurrence after enucleation. The mean time before this treatment was 8 years-9 months. In the third group, 11 patients, (7 patients from our institution), were surgically treated for recurrence after total parotidectomy. After enucleation, the recurrence rate was high and insufficient margins were found in 27% of the cases. In this group, a multicentric recurrence was found in 45% of the cases. In our own experience, recurrence after total parotidectomy was noted in 2.4%. The surgical salvage was performed with enucleation after identification of the branches of the facial nerve. The operative microscope was usefull. In 1 case, a second recurrence occured, and in 1 case iterative recurrence was noted. The local control rate after total parotidectomy was 99.6% (292/293). Total conservative parotidectomy is, for us, the treatment of choice for pleomorphic adenoma of the parotid gland."}, {"id": "pubmed23n0416_22248", "title": "[Successful use of botulinum toxin injection in the treatment of salivary fistula following parotidectomy].", "score": 0.015060080106809079, "content": "A twenty-year-old woman underwent right superficial parotidectomy for pleomorphic adenoma. On the 10th postoperative day she presented with a salivary fistula, for which repeated aspirations with pressure dressings were applied for a month. Despite decreases in the salivary fluid volume, reaccumulation persisted. Following aspiration of the salivary fluid, 40 units of botulinum toxin was injected into the pouch. On the second day of injection, the discharge ceased and the pouch disappeared. No side effects were observed and the patient remained symptom-free during four-month follow-up."}, {"id": "pubmed23n0589_14957", "title": "Treatment of Frey's syndrome with botulinum toxin.", "score": 0.014991728701406122, "content": "Frey s syndrome or Gustatory sweating was first described by Baillarger in 1853. Lucie Frey had described a patient as \"auriculotemporal syndrome\" in 1923. The explanation for this symptom has been an aberrant regeneration of postganglionic parasympathetic fibers feeding the parotid gland that are severed during parotidectomy. After parotidectomy, these cholinergic parasympathetic fibers regenerate and anastomosis with postganglionic sympathetic fibers that supply vessel and sweat gland of the skin. According to a recent study, the treatment of Frey's syndrome has no treatment of choice. The authors investigated the effectiveness of botulinum toxin type A in the treatment of Frey's syndrome for the first time in Thai patients. The present study was a prospective non-randomized, exploratory study. Nine patients with a median involvement skin area of 4.2 cm2 (1-16.3) were injected intradermal with botulinum toxin type A 2 unit in every 1 cm2 of involved skin. The mean total dose was 10.6 units (range 2-32 unit). All of the patients showed improvement after 4-7 days. Five patients have no Gustatory sweating. In the same way, four patients present with a dramatic decrease in Gustatory sweating. When comparing the skin involvement area, indicated by Minor's iodine starch test and calculated by program ImageJ 1.34s, between before and after injection of botulinum toxin type A using sign test, the result is statistically significant with p = 0.0039. The result lasted for 9.2 months (7-10 months). Intradermal injection of botulinum toxin type A for patients with Frey's syndrome is not only effective with no side effect but also minimally invasive. The present report supports that intradermal injection of botulinum toxin type A should be the treatment of choice for Frey's syndrome."}, {"id": "pubmed23n0369_8023", "title": "[Post-parotidectomy Frey's syndrome. Treatment with botulinum toxin type A].", "score": 0.014760904105376456, "content": "The Frey's syndrome, manifest after parotid trauma, is characterized by head and neck hyperemia and abundant sweating of the hyperemic skin in response to gustatory stimuli. The use of the botulin toxin to treat the symptoms in patients with Frey's syndrome has been described in numerous studies. For some time up until now our Center has achieved excellent results using the group A botulin toxin to overcome the hypertonus of the cricopharyngeal muscle in patients who had undergone laryngectomy and were rehabilitated with voice button. We have sought to extend the use of this toxin to Frey's syndrome, a relatively frequent complication of parotidectomy. A total of 86 patients participated in the study: 41 males (47.6%) and 45 females (52.4%) ranging in age from 25 to 77 years (average age 51 years). Of these patients 7 (8.1%) had undergone post-operative radiotherapy. Of the 86 patients studied, 18 referred significant symptoms in terms of abundance and frequency. The syndrome was considered severe if the symptoms were present at each meal and if the patient indicated a significant worsening of his quality of life. Intermittent episodes were indicated by 22 patients. The remaining 46 (43.5%) did not complain of any symptoms. The exact extension of the cervicofacial gustatory sweating was evaluated using the Minor test and the involved region was divided into 1 square centimeters sections. The amount of skin surface involved ranged from 10 to 80 square centimeters. The type A neurotoxin was frozen and was reconstituted with a sterile saline solution at a final concentration of 2.5 UI/0.1 ml. The intracutaneous infiltration was performed without anesthesia, infiltrating 0.1 ml of solution, containing 2.5 UI of toxin into the center of each 1 square centimeters section. Statistical analysis was performed to evaluate the potential relationship between how long the treatment was effective, incidence of recurrence, seriousness of the crises and the following variables: age, sex, histology, cutaneous surface involved, injected dose of botulin toxin and post-operative radiotherapy. In the group of 18 patients with severe symptoms (20.9%) the benefit was immediate in all cases although the recurrence rate was 50%. The Frey's syndrome symptoms disappeared within 7 days of infiltration. In the group of 22 patients with less severe involvement (25.5%), the treatment gave positive, definitive results in 16 patients (72.7%). Those patients whose symptoms persisted were treated a second time with an infiltration of 2.5 UI per square centimeters. We feel that the use of the type A botulin toxin is the most appropriate treatment for the Frey's syndrome. In fact, such treatment offers the following advantages: it is effective within 7 days, has limited side effects, can be applied on an outpatient basis, is inexpensive and is positively considered by the patients."}, {"id": "wiki20220301en073_31938", "title": "Pleomorphic adenoma", "score": 0.014067656765676569, "content": "There have been several approaches for surgery of parotid pleomorphic adenoma in the course of time. Enucleation of the tumor (i.e. intracapsular dissection), a procedure that was common in the early 20th century, is nowadays obsolete due to very high incidence of recurrence. After the time of enucleations, pleomorphic adenomas of parotid gland were recommended to be routinely treated with superficial or total parotidectomy. These procedures combine complete tumor removal and identification of the main trunk of facial nerve during surgery to avoid any lesions to the nerve. However, extensive surgery may cause significant morbidity, such as Frey´s syndrome (excessive sweat while eating) and salivary fistula. Also, aesthetic outcome may be compromised. Therefore, less invasive procedures have been preferred in selected cases during the recent years, and introduction of perioperative neuromonitoring enabled the evolution of several different surgical techniques some twenty years ago."}, {"id": "pubmed23n0546_18065", "title": "Treatment of gustatory sweating with low-dose botulinum toxin A: a case report.", "score": 0.013776834158999765, "content": "Frey's syndrome, gustatory sweating in the preauricular area, is an unpleasant phenomenon occurring during meals after surgery on the parotid gland. Recently, botulinum toxin A (BTX) has been shown to reduce the symptoms, but the variation in the reported doses is large. To quantify the effect of treatment with low-dose BTX in a case of Frey's syndrome over a period of 6 months. A 56-year-old woman was treated with 10 U Botox given as 20 single, intracutaneous injections of 0.5 U, one for each cm(2), 3 years after resection of the parotid gland. Before treatment and repeatedly during the 6-month period, the sweating was rated subjectively on a 100-mm visual analog scale (VAS) and by a severity index, and objectively by assessment of the extent of the involved skin area using Minor's iodine-starch test, staining the area of sweating dark. The treatment decreased the involved area from 20 to 5 cm(2) and the VAS ratings from 98 to 8 mm. The index showed that treatment affected the sweating intensity, not the frequency. After the 6-month period the patient was still satisfied, but the involved skin area had increased; however, not entirely to pretreatment values. The effect of BTX injections for gustatory sweating obtained in this case was comparable to results reported using higher doses. Low doses of BTX can therefore be used in the treatment of Frey's syndrome, but studies to clarify the dose-response relationship, in terms of both time-course and obtained effect, are needed."}, {"id": "pubmed23n0559_20248", "title": "Management of Frey syndrome.", "score": 0.013661202185792351, "content": "Almost all patients who undergo parotidectomy will to some extent develop Frey syndrome (auriculotemporal syndrome or gustatory sweating) after surgery, because of aberrant regeneration of cut parasympathetic fibers between otic ganglion and subcutaneous vessels. However, only the minority of these patients needs treatment. The syndrome consists of gustatory sweating, flushing, and warming over the preauricular and temporal areas. Thick skin flap and partial superficial parotidectomy are the most important techniques to minimize the risk of developing symptomatic Frey syndrome. Intracutaneous injection of botulinum toxin A is an effective, long-lasting, and well-tolerated treatment of Frey syndrome. If recurrence occurs, the treatment can be repeated."}, {"id": "article-73061_35", "title": "Parotidectomy -- Complications", "score": 0.013440285204991086, "content": "Frey’s syndrome: more accurately referred to as gustatory sweating. Patients report facial swelling and sweating at the site of the parotidectomy in occurrence with meals. Etiology is believed to be aberrant innervation of the sweat glands with branches emerging from the auriculotemporal nerve after their division during surgery. This provides parasympathetic innervation to the normally sympathetic-innervated sweat glands [38] . Diagnosis is usually based on patient history, however if there is any doubt an iodine-starch test (Minor test) will confirm the diagnosis, where iodine starch placed on the affected area turns blue signaling sweat secretion. The incidence historically has been reported as high as 50 to 100%, though, with modern techniques and the use of SMAS flaps and thicker skin flaps at the time of initial elevation, this is greatly reduced and is now quite rare. Should this develop, surgical treatment options can be disappointing, with the best results obtained using SMAS and superficial temporal artery flaps as a barrier between the surgical site and the skin. Gold standard treatment now is botulinum toxin injection. Relief of symptoms is obtained for 6 to 36 months. It works at the pre-synaptic level of the neuromuscular and neuroglandular junction by blocking the release of acetylcholine. [37] [39]"}, {"id": "wiki20220301en073_31939", "title": "Pleomorphic adenoma", "score": 0.013385208452312964, "content": "Currently, the choice of surgical approach for parotid pleomorphic adenoma is mainly based on the size, location, and mobility of the tumor. The recommended main techniques include extracapsular dissection, partial superficial parotidectomy, and lateral or total parotidectomy. Nevertheless, the experience of surgeon plays a key role in the results of these distinct procedures. An important point of view is that recurrent pleomorphic adenomas may occur after a very long time from primary surgery, on average over 7–10 years but up to 24 years afterwards. Thus, it is of utmost importance to evaluate the ultimate results of these different surgical techniques in the future. The benign tumors of the submandibular gland is treated by simple excision with preservation of mandibular branch of the facial nerve, the hypoglossal nerve, and the lingual nerve. Other benign tumors of minor salivary glands are treated similarly."}, {"id": "pubmed23n0320_7935", "title": "[Surgery in benign parotid tumors: individually adapted or standardized radical interventions?].", "score": 0.013159937888198758, "content": "Several authors demand emphatically that the minimal operative procedure in benign parotid gland tumors has to be a superficial parotidectomy. Of a consecutive series of 372 patients with benign parotid tumors treated in our department between 1973-1996 81% of the patients could be followed up 1-24 years. in 10.9% a total parotidectomy was performed, in 16% a lateral parotidectomy and in 73.1% a simple extirpation of the tumor (often taking away a small margin of surrounding parotid parenchyma). The operating microscope and microsurgical techniques were used in all of these operations. Of all the followed-up patients 2.3% developed a recurrence. There were no recurrences of cystadenolymphomas or of rare types of adenomas. Recurrences of primary treated pleomorphic adenomas occurred in 3.0%. In recurrent pleomorphic adenomas a further recurrence could be seen in 7.4% of the cases. The over-all incidence of permanent facial nerve weakness was 2.1%: 0.7% after extirpation, 3.3% after lateral parotidectomy and 9.7% after total parotidectomy. we observed in 6.3% a gustatory sweating. Our data prove that with simple extirpation similar results compared to lateral parotidectomy can be achieved concerning recurrence, function of the facial nerve and the Frey's syndrome. We suggest a surgical management adapted to the extent, the size and the location of the parotid gland tumors. In our opinion lateral or total parotidectomy should be reserved for tumors of larger amount or deep located tumors."}, {"id": "wiki20220301en421_8390", "title": "Parotidectomy", "score": 0.01309090909090909, "content": "Post-Operation After completion of a parotidectomy, patients can expect postoperative hospitalization ranging from one-to-three days, to help ensure the safest and most effective postoperative management. At this time, patients will be administered antibiotics to minimize risk of infection as well as an assessment of pain management throughout their stay. Duration of hospitalization is subject to change from patient to patient, with most patients being discharged within 24 hours after surgery. If a tumor was malignant, many patients are referred to radiation therapy. For benign tumors and slow growing cancers, surgery typically provides a complete cure or remission (no evidence for disease). Patient Care after Discharge"}, {"id": "wiki20220301en421_8385", "title": "Parotidectomy", "score": 0.01266025641025641, "content": "Throughout history, many different types and techniques have been developed in order to complete a parotidectomy and consequently, many different names have been associated with each type. However, there are really only two main distinctions to be made in parotidectomies: The specific nerve(s) to be dissected or not dissected The amount of gland excised It is important to note that the specific surgery chosen is based on preservation of the facial nerve in order to avoid significant morbidities (diseases). Furthermore, there are still many controversies regarding the choice of surgery and incidence of cancer recurrence. Below indicates the various and main techniques typically associated with a parotidectomy: Extracapsular dissection - excision of the parotid tumor surrounded by some millimetres of healthy tissue, without searching and exposing the main truck of the facial nerve."}, {"id": "pubmed23n0792_11512", "title": "Pleomorphic adenoma and benign parotid tumors: extracapsular dissection vs superficial parotidectomy--review of literature and meta-analysis.", "score": 0.01253930817610063, "content": "This study compared extracapsular dissection (ED) vs superficial parotidectomy (SP) in the treatment of pleomorphic adenoma and benign parotid tumors. The research covered the years 1950-2011 in PubMed, Ovid MEDLINE, the Cochrane Database of Systematic Reviews, and Scopus. Of 1152 articles screened, 123 studies met the inclusion criteria. A review of the nomenclature of the different parotid surgery techniques was done. Recurrence rate, permanent facial nerve paralysis, and Frey syndrome of patients who underwent ED vs those who underwent SP were compared by meta-analysis. Our meta-analysis data comparing ED and SP found that: (1) the recurrence rate is higher in patients treated with SP; (2) SP has a higher incidence of cranial nerve VII paralysis; and (3) Frey syndrome is more common after SP. ED may be a viable option in the treatment of unilateral benign parotid tumors of the superficial lobe, sized less than 4 cm, without involvement of the facial nerve."}, {"id": "wiki20220301en110_5731", "title": "Frey's syndrome", "score": 0.012330648685771366, "content": "Treatments Injection of botulinum toxin A Surgical transection of the nerve fibers (a temporary treatment) Application of an ointment containing an anticholinergic drug such as scopolamine Cochrane reviews of interventions to either prevent or treat Frey’s syndrome have found little or no evidence to support their effectiveness or safety, and conclude that further clinical trials are needed. Epidemiology The condition is rare, although the exact incidence is unknown. The disorder most often occurs as a complication of the surgical removal of a parotid gland (parotidectomy). The percentage of individuals who develop Frey syndrome after a parotidectomy is controversial and reported estimates range from 30–50 percent. In follow-up examinations, approximately 15 percent of affected individuals rated their symptoms as severe. Frey syndrome affects males and females in equal numbers."}, {"id": "pubmed23n0266_6450", "title": "[Pleomorphic adenoma of the parotid gland. Results of surgical treatment].", "score": 0.012303485987696514, "content": "Twenty years experience of lateral parotidectomy as suspical treatment for pleomorphic adenoma are reviewed. All cases were managed at the ORL Clinic of the University of Zürich. 167 patients were followed for the frequency of possible recurrent tumors. Three patients (3/123) operated primarily developed a recurrences. Recurrences appeared after an average of 10 years, ranging from 1-30 years. The follow-up time varied from 1 to 21 years (average, 8 years). 39% (13 of 33) of the patients, who were re-operated for a recurrent tumor, developed another recurrence. The second recurrence appeared after an average of 10 years, ranging from 1-22 years. A persistent partial paresis of the facial nerve was found in 1% of the patients operated primarily and in 9% of the patients operated more than once. No paralysis was seen. We now choose \"en-bloc\" resections of pleomorphic adenomas without intra-operative opening of the tumor capsule as the treatment of choice. This treatment was possible in 83% of all cases, using a lateral parotidectomy. If tumor extends into the medial parotid lobe, total parotidectomy is required."}, {"id": "pubmed23n0598_787", "title": "[Parotid gland's tumors in children].", "score": 0.01148989898989899, "content": "The tumors of the salivary glands are infrequent in children, and parotid gland is involved in 80% of them. When a salivary gland tumor is present, the chance of malignancy is greater in the child than in the adult. We reviewed 8 cases identified in patients aged 14 years and younger in our hospital, analyzing its antecedents, signs and symptoms, histological features, diagnosis, treatment and evolution. All the patients displayed preauricular painless, non-inflammatory and slow-growing masses to an age between 10 months and 14 years. Four or them were pleomorphic adenomas, two haemangiomas, one epidermal cysts and one myoepithelial carcinoma. We emphasize the exceptional nature of the carcinoma for its rareness and for the high degree of malignancy expressed. We made a fine needle aspiration biopsy in four cases but they were conclusive only in three. All were treated by surgical resection of the tumour except for the myoepithelial carcinoma and the recurrent pleomorphic adenoma that were treated by total parotidectomy. The malignant tumours of the parotid gland are clinically indistinguishable of the benign ones, thus when any palpable mass appears in the zone of the parotid gland, an accurate diagnosis should be made without delay. The treatment of choice is the surgical excision with wide margins, being other adjuvant treatments less useful to this age than in the adult age."}, {"id": "pubmed23n1091_11060", "title": "Botulinum toxin for chronic parotid sialadenitis: A case series and systematic review.", "score": 0.011457609999368727, "content": "To evaluate salivary gland chemodenervation with botulinum toxin in chronic parotid sialadenitis. Patients who underwent parotid gland chemodenervation for chronic sialadenitis due to duct stenosis refractory to siaendoscopy were reviewed (case series). Additionally, a systematic review of the literature on botulinum toxin injection for chronic parotid sialadenitis was performed. Inclusion criteria included studies containing original data on botulinum toxin injections in patients with chronic sialadenitis symptoms. Sialadenitis symptoms from 10 patients with 13 affected parotid glands were examined. All had duct stenosis diagnosed on sialendoscopy, refractory sialadenitis symptoms, and received parotid onabotulinum toxin injection(s) (median dose 65U). Of patients with 3-month follow-up, 78% reported significant improvement in symptoms. Mean Chronic Obstructive Sialadenitis Symptoms (COSS) Score improved at 3 months post-injection (47-25.9, <iP</i = .039) with significant reduction in gland pain frequency and gland swelling severity. No patients had a facial nerve paralysis or increased xerostomia. With the systematic review, 518 abstracts were reviewed and 11 studies met inclusion criteria and included case series or case reports with a total of 40 patients treated with botulinum toxin for chronic parotitis. Thirty-four out of a total of 35 patients in the studies (97%) reported complete (9, 26%) or partial (25, 71%) improvement in sialadenitis symptoms with minimal complications. Parotid gland chemodenervation with botulinum toxin is a minimally invasive treatment option for symptomatic chronic sialadenitis refractory to medical treatment or sialendoscopy. Botulinum toxin injections alleviate gland pain and swelling associated with salivary obstruction and provide an alternative to parotidectomy for recurrent sialadenitis.Level of evidence: 4."}, {"id": "pubmed23n0303_6743", "title": "Surgical management of 246 previously untreated pleomorphic adenomas of the parotid gland.", "score": 0.011411665257819104, "content": "Recent modifications of surgical technique may have influenced outcome following parotidectomy. This retrospective study compares the results of the different surgical methods with regard to recurrence rate and the effects on morbidity between 1974 and 1994. A total of 246 primary surgical parotid procedures were performed on 245 patients for pleomorphic adenoma. These included 131 'partial' superficial parotidectomies, 61 'total' superficial parotidectomies, 30 partial superficial/deep lobe parotidectomies, eight total parotidectomies, and 16 'selective' deep lobe parotidectomies. In the recent past, the posterior branch of the greater auricular nerve was preserved in the majority of patients. Eleven patients received postoperative radiotherapy. Median follow-up was 95 months. Fourteen patients died without recurrent tumour. Two patients (0.8 per cent) developed local recurrence, both after total parotidectomy for a deep lobe tumour. No patient experienced permanent facial nerve palsy. The incidence of gustatory sweating for partial superficial parotidectomy was 6.9 per cent (nine of 131) compared with 13.1 per cent (eight of 61) for total superficial parotidectomy. Partial parotidectomy is an effective treatment for the majority of pleomorphic adenomas; local recurrence is rare and morbidity is low. Prolonged follow-up is unnecessary."}, {"id": "article-28568_55", "title": "Rhytidectomy -- Complications", "score": 0.010963822148577742, "content": "First bite syndrome This has been reported with deep plane rhytidectomy and may be a result of damage to postganglionic parasympathetic nerve fibers to the parotid gland, similar to what may occur during deep lobe parotidectomy or parapharyngeal space surgery. [19] Aberrant reinnervation results in a painful hypercontraction of myoepithelial cells within the parotid gland at the beginning of meals; the pain usually subsides with subsequent bites. While it is unpleasant for patients, it can often be relieved with botulinum toxin injections and will generally resolve spontaneously within 6 to 12 months."}, {"id": "article-73061_30", "title": "Parotidectomy -- Technique or Treatment", "score": 0.010919540229885057, "content": "In the recovery room, facial nerve function should be assessed as soon as possible. Some facial weakness is to be expected, particularly in total parotidectomy. This typically resolves with time so long as all branches were definitively identified and preserved, though full recovery can take many months."}, {"id": "wiki20220301en014_135359", "title": "Parotid gland", "score": 0.010759477685449447, "content": "Surgery Surgical treatment of parotid gland tumors is sometimes difficult because of the anatomical relations of the facial nerve parotid lodge, as well as the increased potential for postoperative relapse. Thus, detection of early stages of a parotid tumor is extremely important in terms of postoperative prognosis. Operative technique is laborious, because of relapses and incomplete previous treatment made in other border specialties. Surgical techniques in parotid surgery have evolved in the last years with the use of neuromonitoring of the facial nerve and have become safer and less invasive. After surgical removal of the parotid gland (Parotidectomy), the auriculotemporal nerve is liable to damage and upon recovery it fuses with sweat glands. This can cause sweating on the cheek on the side of the face of the affected gland. This condition is known as Frey's syndrome. Infections Bacterial infections"}, {"id": "article-73061_8", "title": "Parotidectomy -- Indications", "score": 0.010342663486883393, "content": "By far, the most common indication for parotidectomy is the removal of a neoplasm. In 75% to 80% of cases, these neoplasms are benign and of primary parotid origin. Pleomorphic adenoma and Warthin tumors represent the majority of tumors, with pleomorphic adenoma representing the most common benign parotid tumor. Of malignant tumors, mucoepidermoid and adenoid cystic carcinoma are the two most common primary parotid tumors, in that order. Metastasis from a cutaneous primary represents the most common parotid malignancy in some series, notably in Australia [13] . Chronic parotitis and recurrent sialadenitis can be served with a parotidectomy when medical treatment and sialoendoscopy fail or are unavailable. Other indications include caseating granulomas, toxoplasmosis, branchial cleft cyst, symptomatic lymphoepithelial cyst, or tuberculosis. [14] [15] [16] [17] [18]"}, {"id": "article-73061_33", "title": "Parotidectomy -- Complications", "score": 0.009926238145416228, "content": "Facial paralysis: when anatomically identified and branches are known to be intact, the most probable cause of the paralysis is stretching. This is also supported by the significantly more common incidence of paresis/paralysis in total parotidectomy when compared to superficial parotidectomy. The incidence of transient paralysis is somewhere between 16.6% to 34%, and 90% will recover within 1 month; however, it could last as long as 18 months. The ability to close the eye without any corneal exposure needs to be assessed and preventive measures should be taken to prevent exposure keratitis: ophthalmic drops and ointments, ophthalmologist consultation, gold weights, and botulinum toxin are all potential treatment options in this interim. [36] [37] Seroma: managed by needle aspiration and compression dressing. [37]"}, {"id": "article-73061_27", "title": "Parotidectomy -- Technique or Treatment", "score": 0.009805724091438376, "content": "Benign tumors can almost always be dissected free from the facial nerve without problems. Even with pleomorphic adenoma where a certain margin is required to avoid recurrence, preservation of the facial nerve is always the rule and has only a small risk of recurrence when resected via true superficial parotidectomy. However, some malignant tumors might invade the nerve and raise the question of the sacrifice of the invaded branch. If preoperative facial paralysis exists, every attempt should be made to resect the tumor completely, even if it included the resection of a segment of the facial nerve. When no preoperative paralysis is documented, it is acceptable to shave the tumor off the nerve (R1 resection), though many authors advocate resection of the affected branches and primary cable grafting. Isolated midfacial branches might not need to be repaired because the resulting deformity is often unnoticed. [15] [33]"}, {"id": "wiki20220301en421_8382", "title": "Parotidectomy", "score": 0.009719961308891877, "content": "Painless, noticeably felt growths are the most common presentations described in medical literature. Benign parotid gland neoplasms typically present after the age of 40 and have an equal presentation in both genders. Malignant growths predominantly affect women over the age of 60. The most common form of benign parotid neoplasms are pleomorphic adenomas"}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[37, 151]], "word_ranges": [[7, 27]], "text": "The most frequent cause of urinary tract infections is Escherichia coli and in the case of pregnant women as well."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The correct answer is the first one. The most frequent cause of urinary tract infections is Escherichia coli and in the case of pregnant women as well.", "full_answer_no_ref": "[HIDDEN] The most frequent cause of urinary tract infections is Escherichia coli and in the case of pregnant women as well.", "full_question": "Pregnant woman, 27 years old, 30 weeks of gestation. She comes to the emergency room because she noticed pain in the left lumbar region and dysuria since yesterday. She has no febrile sensation. She refers repeated urinary tract infections (UTI). Urinalysis shows Hb 3+, leukocytes 3+, nitrites 2+, sediment: 15-20 leukocytes per field and 5-10 red blood cells per field. Which of the following microorganisms is the most frequent culprit in pregnant women?", "id": 123, "lang": "en", "options": {"1": "Escherichia coli.", "2": "Enterococcus faecalis.", "3": "Streptococcus agalactiae.", "4": "Proteus mirabilis.", "5": "Satphylococcus saprophyticus."}, "question_id_specific": 223, "type": "MICROBIOLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1031_21939", "title": "Urinary Tract Infections among Indonesian Pregnant Women and Its Susceptibility Pattern.", "score": 0.016741071428571428, "content": "Pregnant women are usually at risk of urinary tract infections (UTIs) such as asymptomatic bacteriuria. In the current multidrug-resistance era, appropriate diagnosis and treatment should be provided to avoid complications in pregnant women in developing countries, which have limited facilities, such as Indonesia. The aim of this study was to evaluate in vitro susceptibility tests. Urinary isolates were collected from 715 pregnant women who visited eight Community Health Centers in Jakarta, Indonesia, between 2015 and 2017. We identified bacterial uropathogens from samples that were positive for nitrite/leukocyte esterase (LE), using two types of VITEK cards. Since noncompliance among patients is a major problem, fosfomycin-trometamol 3 g single-dose sachets were given to the patients, and the side effects of the medication and neonatal outcomes were reported. Asymptomatic bacteriuria was found in 10.5% of the 715 pregnant women. <iEscherichia coli</i was the most common etiological factor (26.7%), followed by <iKlebsiella pneumoniae</i (20%), <iStreptococcus agalactiae</i (9.3%), <iEnterobacter cloacae</i (5.3%), <iEnterococcus faecalis</i (5.3%), <iStaphylococcus saprophyticus</i (4%), <iAcinetobacter baumannii</i (4%), and others. Out of 76 pregnant women who took fosfomycin-trometamol, two complained of diarrhea that subsided without medication and fever that responded to paracetamol. Neonatal outcomes showed 100% full-term and normal-weight babies. <iE. coli</i, including extended-spectrum beta-lactamase- (ESBL-) producing <iE. coli</i, was 100% susceptible to fosfomycin. Nitrite/LE test results are often used as evidence for empiric antibiotic administration for treating asymptomatic bacteriuria in pregnancy, but the diagnosis should be confirmed using culture tests. Based on in vitro susceptibility patterns and medication outcomes, fosfomycin-trometamol single dose could be administered to noncompliant UTI patients, including pregnant women."}, {"id": "Pathoma_Husain_318", "title": "Pathoma_Husain", "score": 0.015440969287123133, "content": "II. CYSTITIS A. Infection of the bladder B. Presents as dysuria, urinary frequency, urgency, and suprapubic pain; systemic signs (e.g., fever) are usually absent. C. Laboratory findings 1. Urinalysis-cloudy urine with > 10 WBCs/high power field (hpf) 2. Dipstick-Positive leukocyte esterase (due to pyuria) and nitrites (bacteria convert nitrates to nitrites) 3. Culture-greater than 100,000 colony forming units (gold standard) D. Etiology 1. E coli (80%) 2. Staphylococcus saprophyticus-increased incidence in young, sexually active women (but E coli is still more common in this population) 3. Klebsiella pneumoniae 4. Proteus mirabilis-alkaline urine with ammonia scent Fig. 12.15 Linear IF, Goodpasture syndrome. Fig. 12.16 lgA nephropathy. (Courtesy ofTony (Courtesy ofTony Chang, MD) Chang, MD) 5. Enterococcus faecalis E. Sterile pyuria is the presence of pyuria (> 10 WBCs/hpf and leukocyte esterase) with a negative urine culture."}, {"id": "pubmed23n0778_14939", "title": "Antibiotic sensitivity pattern of uropathogens from pregnant women with urinary tract infection in Abakaliki, Nigeria.", "score": 0.014916435298600905, "content": "Urinary tract infection (UTI) is a common bacterial infection during pregnancy and a significant cause of perinatal and maternal morbidity and mortality. The causative bacteria have remained virtually the same although with variations in individual prevalence. There has been an increasing resistance by these bacteria to the commonly available antibiotics. To determine the prevalence of UTI, the common causative bacteria, and their antibiotic sensitivity pattern among pregnant women with UTI. This is a descriptive study that was carried out at the Obstetrics Department of two tertiary institutions in Abakaliki, Ebonyi State, Nigeria (Federal Medical Center and Ebonyi State University Teaching Hospital) over a period of 12 months. Midstream urine specimens from selected pregnant women with clinical features of UTI were collected for microscopy, culture, and sensitivity. The results were analyzed with the 2008 Epi Info™ software. A total of 542 pregnant women presented with symptoms of UTI and were recruited for the study over the study period. Of the 542 pregnant women, 252 (46.5%) had significant bacteriuria with positive urine culture and varying antibiotic sensitivity pattern. The prevalence of symptomatic UTI was 3%. Escherichia coli was the most common bacteria isolated with a percentage of 50.8%. Other isolated micro organisms included Stapylococcus aereus (52 cultures, 20.6%), Proteus mirabilis (24 cultures, 9.5%), S. saprophyticus (18 cultures, 7.1%), Streptococcus spp. (14 cultures, 5.6%), Citrobacter spp. (5 cultures, 2.0%), Klebsiella spp. (4 cultures, 1.6%), Enterobacter spp. (4 cultures, 1.6%), and Pseudomonas spp. (3 cultures, 1.2%). Levofloxacin had the highest overall antibiotic sensitivity of 92.5%. Others with overall antibiotic sensitivity pattern greater than 50% included cefpodoxime (87.3%), ofloxacin (77.4%), ciprofloxacin (66.7%), ceftriaxone (66.7%), and gentamicin (50.8%). E. coli was the most common etiological agent of UTI in pregnancy with Enterococcus (Staphylococcus) gaining prominence. Cephalosporin and quinolones were shown to be very effective against the organisms causing UTI in these pregnant women."}, {"id": "pubmed23n0601_2316", "title": "Contemporary management of uncomplicated urinary tract infections.", "score": 0.014780577001219016, "content": "Uncomplicated urinary tract infections (uUTIs) are common in adult women across the entire age spectrum, with mean annual incidences of approximately 15% and 10% in those aged 15-39 and 40-79 years, respectively. By definition, UTIs in males or pregnant females and those associated with risk factors known to increase the risk of infection or treatment failure (e.g. acquisition in a hospital setting, presence of an indwelling urinary catheter, urinary tract instrumentation/interventions, diabetes mellitus or immunosuppression) are not considered herein. The majority of uUTIs are caused by Escherichia coli (70-95%), with Proteus mirabilis, Klebsiella spp. and Staphylococcus saprophyticus accounting for 1-2%, 1-2% and 5-10% of infections, respectively. If clinical signs and symptoms consistent with uUTI are present (e.g. dysuria, frequency, back pain or costovertebral angle tenderness) and there is no vaginal discharge or irritation present, the likelihood of uUTI is &gt;90-95%. Laboratory testing (i.e. urinary nitrites, leukocyte esterase, culture) is not necessary in this circumstance and empirical treatment can be initiated. The ever-increasing incidence of antimicrobial resistance of the common uropathogens in uUTI has been and is a continuing focus of intensive study. Resistance to cotrimoxazole (trimethoprim/sulfamethoxazole) has made the empirical use of this drug problematic in many geographical areas. If local uropathogen resistance rates to cotrimoxazole exceed 10-25%, empirical cotrimoxazole therapy should not be utilized (fluoroquinolones become the new first-line agents). In a few countries, uropathogen resistance rates to the fluoroquinolones now exceed 10-25%, rendering empirical use of fluoroquinolones problematic. With the exception of fosfomycin (a second-line therapy), single-dose therapy is not recommended because of suboptimal cure rates and high relapse rates. Cotrimoxazole and the fluoroquinolones can be administered in 3-day regimens. Nitrofurantoin, a second-line therapy, should be given for 7 days. beta-Lactams are not recommended because of suboptimal clinical and bacteriological results compared with those of non-beta-lactams. If a beta-lactam is chosen, it should be given for 7 days. Management of uUTIs can frequently be triaged to non-physician healthcare personnel without adverse clinical consequences, resulting in substantial cost savings. It can be anticipated that the optimal approach to the management of uUTIs will change substantially in the future as a consequence of antimicrobial resistance."}, {"id": "pubmed23n0759_24868", "title": "Symptomatic Shigella sonnei urinary tract infection in pregnancy.", "score": 0.014674719220173766, "content": "This report describes a case of urinary tract infection (UTI) due to Shigella sonnei during pregnancy. A 31-year-old pregnant woman was admitted complaining of left-flank tenderness, dysuria, and fever. Following examination, significant laboratory data were collected including increased leukocyte count (10,800/ul with 86% neutrophils) and C-reactive protein (9.6 mg/dl). Urinalysis revealed 30 to 50 leukocytes per high power field while from the quantitative urine culture Shigella sonnei was recovered after 24 h incubation at 37 degrees C. After a two-week course with 750 mg cefuroxime every 8 h, the patient experienced gradual resolution of all symptoms and urinary cultures were negative two weeks and one month, respectively, after completing the therapy. The gestational course was uneventful and the patient delivered a healthy baby girl at term. Shigella sonnei can be responsible for UTI during pregnancy even when no predisposing factors or an apparent source of infection can be identified."}, {"id": "pubmed23n0771_21526", "title": "Urinary tract infection in outpatient febrile infants younger than 30 days of age: a 10-year evaluation.", "score": 0.014210128495842782, "content": "To determine the prevalence of outpatient-diagnosed urinary tract infection (UTI) in consecutive febrile neonates ≤ 30 days of age and correlate demographic, laboratory and radiographic imaging results with infectious etiology. Review of medical records of consecutive febrile infants ≤ 30 days of age presenting to an urban pediatric emergency department during a 10-year period, whose policy is to perform a sepsis evaluation (urine culture obtained by bladder catheterization) and hospitalize for parenteral antibiotic therapy pending culture results. Of 670 febrile neonates ≤ 30 days of age evaluated for sepsis, urine culture was obtained in 651 cases (97%). Of 100 patients with UTI (15.4%), 73% were male; the most common uropathogens were Escherichia coli (71%), Enterococcus (10%) and Klebsiella sp. (10%). In all, 39% had a maximum documented fever ≥ 102 °F, and 40% had CBC total white blood cells count ≥ 15,000/mm(3). Urine dipstick test was positive for leukocyte esterase or nitrite in 79%. Renal ultrasound performed in 95 patients (95%) showed anatomic abnormalities in 47%; 5/26 (24%) with hydronephrosis had vesicoureteral reflux on voiding cystourethrogram. Four patients had urosepsis; none had bacterial meningitis and no patients died. UTI affects approximately 1 in 6 febrile neonates ≤ 30 days of age. Males are affected 2.5-times greater than females. E. coli continues to be the predominant uropathogen. Clinical parameters like height of fever, CBC total white blood cell count and urine dipstick test lack sensitivity in identifying UTI risk in the outpatient setting. Only 4 infants had urosepsis (4%). Nearly half of neonates with UTI have a radiographically identified anatomic abnormality. All febrile young infants should receive performance of a urine culture; those with UTI require imaging."}, {"id": "Obstentrics_Williams_7288", "title": "Obstentrics_Williams", "score": 0.013614987864077669, "content": "If this infection is suspected, a urine sample obtained by catheterization may be preferred to avoid obscuring contamination from the lower genital tract. he urinary sediment contains many leukocytes, frequently in clumps, and numerous bacteria. Bacteremia is demonstrated in 15 to 20 percent of these women. E coli is isolated from urine or blood in 70 to 80 percent of infections, Klebsiela pneumoniae in 3 to 5 percent, Enterobacter or Proteus species in 3 to 5 percent, and gram-positive organisms, including group B Streptococcus and Staphylococcus aureus, in up to 10 percent of cases (Hill, 2005; Wing, 2000)."}, {"id": "pubmed23n0788_19788", "title": "Can a simple urinalysis predict the causative agent and the antibiotic sensitivities?", "score": 0.013450710519259987, "content": "The objective of this study was (1) to determine the reliability of urinalysis (UA) for predicting urinary tract infection (UTI) in febrile children, (2) to determine whether UA findings can predict Escherichia coli versus non-E. coli urinary tract infection, and (3) to determine if empiric antibiotics should be selected based on E. coli versus non-E. coli infection predictions. This was a retrospective chart review of children from 2 months to 2 years of age who presented to the emergency department with fever (rectal temperature &gt;100.4°F) and had a positive urine culture. This study was conducted between January 2004 and December 2007. Negative UA was defined as urine white blood cell count less than 5 per high-power field, negative leukocyte esterase, and negative nitrites. Urine cultures were classified into E. coli and non-E. coli groups. These groups were compared for sex, race, and UA findings. Multivariate forward logistic regression, using the Wald test, was performed to calculate the likelihood ratio (LR) of each variable (eg, sex, race, UA parameters) in predicting UTI. In addition, antibiotic sensitivities between both groups were compared. Of 749 medical records reviewed, 608 were included; negative UA(-) was present in 183 cases, and positive UA(+) was observed in 425 cases. Furthermore, 424 cases were caused by E. coli, and 184 were due to non-E. coli organisms. Among 425 UA(+) cases, E. coli was identified in 349 (82.1%), whereas non-E. coli organisms were present in 76 (17.9%); in contrast, in 183 UA(-) cases, 108 (59%) were due to non-E. coli organisms versus 75 (41%), which were caused by E. coli. Urinalysis results were shown to be associated with organism group (P &lt; 0.001). Positive leukocytes esterase had an LR of 2.5 (95% confidence interval [CI], 1.5-4.2), positive nitrites had an LR of 2.8 (95% CI, 1.4-5.5), and urine white blood cell count had an LR of 1.8 (95% CI, 1.3-2.4) in predicting E. coli versus non-E. coli infections. Antibiotic sensitivity compared between UA groups demonstrated equivalent superiority of cefazolin (94.7% sensitive in UA(+) vs 84.0% in UA(-) group; P &lt; 0.0001), cefuroxime (98.2% vs 91.7%; P &lt; 0.001), and nitrofurantoin (96.1% vs 82.2%; P &lt; 0.0001) in the UA(+) group. In contrast, the UA(-) group showed significant sensitivity to trimethoprim-sulfamethoxazole (82.2% vs 71.3% in UA(+); P = 0.008). Urinalysis is not an accurate predictor of UTI. A positive urine culture in the presence of negative UA most likely grew non-E. coli organisms, whereas most UA(+) results were associated with E. coli. This study also highlighted local patterns of antibiotic resistance between E. coli and non-E. coli groups. Negative UA results in the presence of strong suspicion of a UTI suggest a non-E. coli organism, which may be best treated with trimethoprim-sulfamethoxazole. Conversely, UA(+) results suggest E. coli, which calls for treatment with cefazolin or cefuroxime."}, {"id": "pubmed23n0948_2044", "title": "Clinical and laboratory features of urinary tract infections in young infants.", "score": 0.013385262780911452, "content": "Urinary tract infection (UTI) is the most common serious bacterial infection in young infants. Signs and symptoms are often nonspecific. To describe clinical, demographic and laboratory features of UTI in infants ≤ 3 months old. Cross-sectional study of infants ≤ 3 months old with UTI diagnosed in a pediatric emergency department, for the period 2010-2012. UTI was defined as ≥ 50,000 colony-forming units per milliliter of a single uropathogen isolated from bladder catheterization. Paired urinalysis and urine culture from group culture-positive and group culture-negative were used to determine the sensitivity and specificity of pyuria and nitrite tests in detecting UTI. Of 519 urine cultures collected, UTI was diagnosed in 65 cases (prevalence: 12.5%); with male predominance (77%). The most common etiologies were Escherichia coli (56.9%), Klebsiella pneumoniae (18.5%) and Enterococcus faecalis (7.7%). Frequent clinical manifestations were fever (77.8%), irritability (41.4%) and vomiting (25.4%). The median temperature was 38.7°C. The sensitivity of the nitrite test was 30.8% (95%CI:19.9-43.4%), specificity of 100% (95%CI:99.2-100%). Pyuria ≥ 10,000/mL had a sensitivity of 87.7% (95%CI:77.2-94.5%), specificity of 74.9% (95%CI:70.6 -78.8%). The median peripheral white blood cell count was 13,150/mm3; C-reactive protein levels were normal in 30.5% of cases. The male: female ratio for urinary tract infection was 3.3:1. Non-Escherichia coli etiologies should be considered in empirical treatment. Fever was the main symptom. Positive nitrite is highly suggestive of UTI but has low sensitivity; whereas pyuria ≥ 10,000/mL revealed good sensitivity, but low specificity. Peripheral white blood cell count and C-reactive protein concentration have limited usefulness to suggest UTI."}, {"id": "wiki20220301en001_192883", "title": "Urinary tract infection", "score": 0.012920489296636086, "content": "Diagnosis In straightforward cases, a diagnosis may be made and treatment given based on symptoms alone without further laboratory confirmation. In complicated or questionable cases, it may be useful to confirm the diagnosis via urinalysis, looking for the presence of urinary nitrites, white blood cells (leukocytes), or leukocyte esterase. Another test, urine microscopy, looks for the presence of red blood cells, white blood cells, or bacteria. Urine culture is deemed positive if it shows a bacterial colony count of greater than or equal to 103 colony-forming units per mL of a typical urinary tract organism. Antibiotic sensitivity can also be tested with these cultures, making them useful in the selection of antibiotic treatment. However, women with negative cultures may still improve with antibiotic treatment. As symptoms can be vague and without reliable tests for urinary tract infections, diagnosis can be difficult in the elderly."}, {"id": "pubmed23n0679_18816", "title": "Escherichia coli septic arthritis of a lumbar facet joint following urinary tract infection.", "score": 0.012839506172839507, "content": "Septic arthritis of a lumbar facet joint is a rare condition. We report the case of a 77-year-old diabetic woman who developed fever and back pain 15 days after she had been diagnosed with a genitourinary infection for which she had received ciprofloxacin. Physical examination showed fever (38°C) and pain on pressure over the lower lumbar spinous vertebral apophyses and over the lower left paraspinal musculature. Investigations showed a white cell count of 8.4×10⁹/l, neutrophils 85.3%, erythrocyte sedimentation rate of 125 mm/h, and C-reactive protein of ≥9 mg/dl. Two blood cultures were both positive for Escherichia coli resistant to ciprofloxacin. There was no growth of pathogens from the urine cultures. Scintigraphy with gallium citrate Ga⁶⁷ showed vertical lower lumbar (L4-L5) radionuclide uptake lateralized to the left. Magnetic resonance imaging of the lumbar spine demonstrated signal changes and alteration of the structure at the left interapophyseal L4-L5 joint, an adjacent small collection of 1cm in diameter, and infiltration of the surrounding soft tissues, which extended to the epidural area, left conjunction hole, and paraspinal muscles. The patient was treated with intravenous cefotaxime and gentamicin and bed rest for 21 days, and recovered. This is the first report of interapophyseal arthritis caused by E. coli."}, {"id": "wiki20220301en098_56853", "title": "Nitrite test", "score": 0.01182412358882947, "content": "Urinary nitrite test A nitrite test is a standard component of a urinary test strip. A positive test for nitrites in the urine is called nitrituria. This test is commonly used in diagnosing urinary tract infections (UTIs). A positive nitrite test indicates that the cause of the UTI is a gram negative organism, most commonly Escherichia coli. The reason for nitrites' existence in the presence of a UTI is due to a bacterial conversion of endogenous nitrates to nitrites. This may be a sign of infection. However, other parameters, such as leukocyte esterase, urine white blood cell count, and symptoms such as dysuria, urinary urgency, fevers, and chills must be correlated to diagnose an infection."}, {"id": "article-30856_8", "title": "Urinary Tract Infection in Pregnancy -- Pathophysiology", "score": 0.01145320197044335, "content": "Organisms causing UTI in pregnancy are the same uropathogens which commonly cause UTI in non-pregnant patients. Escherichia coli is the most common organism isolated. An 18-year retrospective analysis found E. coli to be the causative agent in 82.5% of cases of pyelonephritis in pregnant patients. [3] Other bacteria which may be seen include Klebsiella pneumoniae, Staphylococcus, Streptococcus, Proteus, and Enterococcus species."}, {"id": "wiki20220301en254_18243", "title": "Urine test strip", "score": 0.01132948434039861, "content": "It is normal to find up to 3 (occasionally 5) leukocytes per high power field (40X) in a urine sample, with women having slightly higher results owing to vaginal contamination. Higher numbers indicate urinary infection. The urine test strip test for white blood cells detects leukocyte esterase, which is present in azurophilic granules of monocytes and granulocytes (neutrophilic, eosinophilic and basophilic). Bacteria, lymphocytes and epithelial cells from the genitourinary tract do not contain esterases. Neutrophil granulocytes are the leukocytes most commonly associated with urinary infections. A positive test for leukocyte esterase normally indicates the presence of bacteria and a positive nitrite test (although it is not always the case). Infections caused by Trichomonas, Chlamydia and yeasts produce leukocyturia without bacteriuria. The inflammation of the renal tissues (interstitial nephritis) can produce leukocyturia, in particular toxic interstitial nephritis with predominant"}, {"id": "wiki20220301en001_192882", "title": "Urinary tract infection", "score": 0.01091644204851752, "content": "Pathogenesis The bacteria that cause urinary tract infections typically enter the bladder via the urethra. However, infection may also occur via the blood or lymph. It is believed that the bacteria are usually transmitted to the urethra from the bowel, with females at greater risk due to their anatomy. After gaining entry to the bladder, E. Coli are able to attach to the bladder wall and form a biofilm that resists the body's immune response. Escherichia coli is the single most common microorganism, followed by Klebsiella and Proteus spp., to cause urinary tract infection. Klebsiella and Proteus spp., are frequently associated with stone disease. The presence of Gram positive bacteria such as Enterococcus and Staphylococcus increased. The increased resistance of urinary pathogens to quinolone antibiotics has been reported worldwide and might be the consequence of overuse and misuse of quinolones. Diagnosis"}, {"id": "pubmed23n0386_10564", "title": "Comparison of test characteristics of urine dipstick and urinalysis at various test cutoff points.", "score": 0.010905741114941163, "content": "We compare the test characteristics of urine dipstick and urinalysis at various test cutoff points in women presenting to emergency departments and an intermediate care center with symptoms of urinary tract infection. This was a prospective, observational study of adult women presenting to 1 of 2 community hospital EDs or an intermediate care center with dysuria, urgency, or urinary frequency on history, or suprapubic or costovertebral angle tenderness on examination. Patients who had taken antibiotics in the past 72 hours, had indwelling Foley catheters, symptomatic vaginal discharge, diabetes mellitus, immunodeficiency disorders, or were unable to provide a reliable history were excluded. The patient's clean-catch or catheterized urine specimen was tested immediately by a nurse using a Multistix 9 SG reagent strip. A second aliquot was sent within 1 hour of collection to the hospital laboratory, where a semiautomated microscopic urinalysis and a urine culture were performed. A positive urine culture was defined as more than 100,000 colonies of 1 or 2 uropathogenic bacteria per mL of urine at 48 hours. Dipstick and urinalysis data were compared with urine culture results. Sensitivity, specificity, and predictive values were calculated at various definitions of a positive test, or \"test cutoff points,\" for combinations of leukocyte esterase, nitrite, and blood on dipstick and for RBCs and WBCs on urinalyses. The probability of an erroneous decision to withhold treatment on the basis of a negative test result was defined as \"undertreatment,\" or 1 minus the negative predictive value. \"Overtreatment\" was defined as 1 minus the positive predictive value. Three hundred forty-three patients were enrolled in this study. Twelve patients were withdrawn because of missing laboratory results. Forty-six percent (152/331) of patients had positive urine cultures. If urine dipstick results are defined as positive when leukocyte esterase or nitrite is positive or blood is more than trace, the overtreatment rate is 47% (156/331) and the undertreatment rate is 13% (43/331). If urinalysis results are defined as positive when WBCs are more than 3 per high-power field or RBCs are more than 5 per high-power field, the overtreatment rate is 44% (146/331) and the undertreatment rate is 11% (36/331). Matched pairs of test characteristics were identified when the analysis was repeated using more than 10,000 colonies per mL as a positive culture. In this patient population, similar overtreatment and undertreatment rates were identified for various test cutoff points for urine dipstick tests and urinalysis. Although a urine dipstick may be equivalent to a urinalysis for the diagnosis of urinary tract infection, the limitations in the diagnostic accuracy of both tests should be incorporated into medical decisionmaking."}, {"id": "pubmed23n1048_11618", "title": "Antimicrobial susceptibility patterns of uropathogens isolated from pregnant women in KwaZulu-Natal Province: 2011 - 2016.", "score": 0.010443583118001722, "content": "Urinary tract infection (UTI) is one of the most common infections during pregnancy, which can lead to significant maternal and perinatal morbidity and mortality if left untreated. Challenges when treating UTIs in pregnancy include fetal protection and resistance development of uropathogens. Currently, the Essential Medicines List recommends nitrofurantoin to treat cystitis and ceftriaxone to treat pyelonephritis in pregnant women. To determine common pathogens causing UTI in pregnancy and their antibiotic susceptibility patterns. A retrospective analysis was performed of laboratory data for positive urine specimens from obstetric departments of 6 KwaZulu- Natal Province hospitals during 2011 - 2016. Identification and susceptibility testing were performed using the VITEK 2 system. Results were interpreted according to the breakpoints of the Clinical and Laboratory Standards Institute, USA. From 5 971 positive urine specimens, the most common isolate was Escherichia coli (n=3 236; 54.2%), followed by Klebsiella pneumoniae (n=770; 12.9%). Group B streptococcus (GBS) (n=239; 4.0%) and Enterococcus faecalis (n=251; 4.2%) were the most common Gram-positive pathogens. E. coli displayed significant resistance to trimethoprim-sulfamethoxazole (65.1%), cephalothin (38.3%), cefuroxime (27.3%), ciprofloxacin (16.9%) and amoxicillin-clavulanic acid (17.1%). Resistance to ceftriaxone and nitrofurantoin remained low ‒ 9.1% and 7.7%, respectively. Among Gram-positive pathogens, GBS displayed 100% penicillin susceptibility and E. faecalis showed 92.9% susceptibility to ampicillin. E. coli is unsurprisingly the most common cause of UTI in pregnancy in KwaZulu-Natal. Susceptibility to ceftriaxone and nitrofurantoin remains good. Among Gram positives, GBS is prevalent and susceptible to penicillin, while E. faecalis is susceptible to ampicillin. As antimicrobial resistance evolves, routine surveillance is necessary to modify recommended empirical antibiotic use."}, {"id": "pubmed23n1007_14583", "title": "A successful management after preterm delivery in a patient with severe sepsis during third-trimester pregnancy.", "score": 0.009900990099009901, "content": "A 33-year-old woman visited the emergency department presenting with fever and dyspnea. She was pregnant with gestational age of 31 weeks and 6 days. She had dysuria for 7 days, and fever and dyspnea for 1 day. The vital signs were as follows: blood pressure 110/70 mmHg, heart rate 118 beats/minute, respiratory rate 28/minute, body temperature 38.7℃, and oxygen saturation by pulse oximetry 84% during inhalation of 5 liters of oxygen by nasal prongs. Crackles were heard over both lung fields. There were no signs of uterine contractions. Chest X-ray and chest computed tomography scan showed multiple consolidations and air bronchograms in both lungs. According to urinalysis, there was pyuria and microscopic hematuria. She was diagnosed with community-acquired pneumonia and urinary tract infection (UTI) that progressed to severe sepsis and acute respiratory failure. We found extended-spectrum beta-lactamase producing <iEscherichia coli</i in the blood culture and methicillin-resistant Staphylococcus aureus in the sputum culture. The patient was transferred to the intensive care unit with administration of antibiotics and supplementation of high-flow oxygen. On hospital day 2, hypoxemia was aggravated. She underwent endotracheal intubation and mechanical ventilation. After 3 hours, fetal distress was suspected. Under 100% fraction of inspired oxygen, her oxygen partial pressure was 87 mmHg in the arterial blood. She developed acute kidney injury and thrombocytopenia. We diagnosed her with multi-organ failure due to severe sepsis. After an emergent cesarean section, pneumonia, UTI, and other organ failures gradually recovered. The patient and baby were discharged soon thereafter."}, {"id": "pubmed23n0130_617", "title": "Asymptomatic bacteriuria during pregnancy with special reference to group B streptococci.", "score": 0.009900990099009901, "content": "The relationship between significant bacteriuria (SB), i.e. 2 subsequent voided urine specimens with greater than or equal to 10(5) colony forming units (CFU)/ml, and the occurrence of bacteria in the urinary bladder detected by bladder punction, was investigated in asymptomatic pregnant women. From 30 (70%) of the 43 women with SB studied, bacteria were isolated from the urinary bladder. The same bacteria were found in the bladders of all 21 women with Escherichia coli, the one with Klebsiella pneumoniae, and the one with Staphylococcus saprophyticus in midstream urine. Six of 10 patients with group B streptococci (GBS), 1 of 4 patients with Streptococcus faecalis, and none of 5 patients with Staphylococcus epidermidis in voided specimens had bacteria in the aspirated urine. Serotype III was isolated from 8/10 patients with SB caused by GBS. One child born to a woman with GBS SB but no bacteria in the urinary bladder, got early onset septicaemia. The poor predictive value of SB with GBS, S. faecalis and S. epidermidis necessitates the increased use of bladder puncture for diagnosis of true asymptomatic bacteriuria (AB), i.e. AB with bacteria in the urinary bladder. SB with GBS even without bacteria in the urinary bladder, may constitute a threat to the baby's health."}, {"id": "pubmed23n0416_4501", "title": "Laboratory aspects of asymptomatic bacteriuria in pregnancy.", "score": 0.00980392156862745, "content": "A total of 1,661 pregnant women aged between 13 and 45 years were screened for bacteriuria by urine culture. Of the 1,661 culture results, 615 (37%) yielded no growth; 728 (43.8%) yielded no significant growth (presence of &lt;10(5) organisms/ml urine of one or more types of bacteria); 286 (17.2%) yielded mixed growth (presence of &gt;10(5) organisms/ml urine of more than one type of bacteria) and only 32 (1.9%) showed significant growth (presence of &gt;10(5) organisms/ml urine of a single bacterium). Urine microscopy was also conducted. Two hundred and twenty-four (13.5%) specimens had &gt;10 white blood cells/ml urine, of which 66 had &gt;100 white blood cells; 13 were from the significant growth group. Three hundred and seventy-four (22.5%) specimens showed the presence of bacteria, 42 (2.5%) had red blood cells, 370 (22.3%) had epithelial cells, 58 (3.5%) had crystals, and 14 (0.8%) had yeasts. The most common bacterium isolated was Escherichia coli (12; 40%); the others included group B Streptococcus (5; 15%), Klebsiella spp (5; 15%), Diphtheroids (2), and Candida albicans (2). Fifty-two percent of tested strains were sensitive to ampicillin; 24 of 28 strains (85.7%) were sensitive to ciprofloxacin; all 7 strains tested were sensitive to nitrofurantoin and all 20 strains tested were sensitive to cotrimoxazole; 14/20 (70%) and 16/17 (94.1%) were sensitive to cephalexin and cefuroxime respectively. This study shows that asymptomatic bacteriuria does occur in pregnant women, albeit at a very low rate in an urban setting like Cheras. Urine microscopy is not specific and only serves as a guide to bacteriuria. The commonest causative organisms are those from the gastrointestinal tract and vagina. The antibiogram showed that cefuroxime and cephalexin are likely to be effective in treating bacteriuria: ampicillin must be reserved for Gram-negative organisms. For Gram-positive organisms, of which Group B Streptococcus is important, ampicillin is still effective in vitro. Nitrofurantion and cotrimoxazole have excellent activity in vitro and should be considered for therapy. 17.2% of the urine culture yielded mixed growth: likely to indicate that contamination of urine specimens still happens despite the strict instructions given to patients about the collection of a midstream urine specimen. Proper collection, appropriate transport, and the early processing of urine specimens remain essential."}, {"id": "pubmed23n0760_2493", "title": "Anger management: bacteria soothe the savage host.", "score": 0.009708737864077669, "content": "A 5-year-old girl has come to you a week after completing a course of antibiotics for a febrile urinary tract infection (UTI). She now seems well and energetic. A urinalysis is now clear without traces of inflammation, including an absence of protein, blood, leukocyte esterase, and nitrites. Her urine is submitted for a test of cure and comes back positive, with over 100,000 colonies per milliliter of E. coli, the same kind of bacteria that was cultured from her urine when she was symptomatic with the UTI. Perplexed, her mother asks how her child can have bacteria once again in her bladder but not be symptomatic and asks if antibiotics are again necessary."}, {"id": "pubmed23n0808_16216", "title": "Effectiveness of an association of a cranberry dry extract, D-mannose, and the two microorganisms Lactobacillus plantarum LP01 and Lactobacillus paracasei LPC09 in women affected by cystitis: a pilot study.", "score": 0.009615384615384616, "content": "Urinary tract infections (UTIs) are the most common bacterial infection in women. Most UTIs are acute uncomplicated cystitis caused by Escherichia coli (86%). This study was undertaken to assess the effectiveness of an association of a cranberry dry extract, D-mannose, a gelling complex composed of the exopolysaccharides produced by Streptococcus thermophilus ST10 (DSM 25246) and tara gum, as well as the 2 microorganisms Lactobacillus plantarum LP01 (LMG P-21021) and Lactobacillus paracasei LPC09 (DSM 24243) in women affected by acute uncomplicated cystitis. Thirty-three premenopausal, nonpregnant women diagnosed with acute uncomplicated cystitis were enrolled in a pilot prospective study and completed the treatment protocol. Subjects were instructed to take 2 doses per day during the first month, and then to continue with 1 sachet per day until the sixtieth day. Nitrites and leukocyte esterase on urine dipstick testing were used as indicators of cystitis, with analysis performed at enrollment, after 30 and 60 days, and after 1 month of follow-up. Typical UTI symptoms, namely dysuria, frequent voiding of small volumes, urinary urgency, suprapubic pain, and gross hematuria were scored 0 to 3 and evaluated at each visit. Positive results for the presence of nitrites and leukocyte esterase were found in 14 and 20 subjects after 30 days and in 9 and 14 women after 60 days, respectively (P&lt;0.001). At the end of the follow-up period, positive results for nitrites and leukocyte esterase were recorded in only 4 and 3 of 24 and 19 subjects (16.7%, P=0.103; 15.8%, P=0.325, respectively), with negative results after 60 days. Typical symptoms of cystitis, specifically dysuria, frequent voiding, urgency, and suprapubic pain were significantly improved as well. No significant differences were recorded in the incidence and severity of hematuria at any visit. The long-term ability of an association of cranberry, D-mannose, an innovative gelling complex, and the 2 microorganisms tested to significantly improve the uncomfortable symptoms reported by women with acute cystitis has been suggested."}, {"id": "pubmed23n0109_9735", "title": "Significance of group B streptococci in urine cultures from males and non-pregnant females.", "score": 0.009615384615384616, "content": "Over a 2-year period, 1% of 24,000 urine cultures with possible relevant bacteria from males and non-pregnant females greater than or equal to 15 years of age were found to harbour group B streptococci (GBS) in quantities greater than or equal to 10(5) colony forming units (cfu)/ml; a further 0.9% harboured GBS in quantities greater than or equal to 10(4) but less than 10(5) cfu/ml. Patients with GBS in urine were evenly distributed by age. Those with greater than or equal to 10(5) cfu GBS/ml in voided urine more frequently had true bacteriuria (i.e. bacteria in the urine bladder) than did patients with less amounts (p = 0.01) as determined by suprapubic aspiration of 23 patients. One third (3/9) of the aspirated patients with greater than or equal to 10(5) cfu GBS/ml in simultaneously voided urine, had contaminated urine only and no true bacteriuria. The acute symptoms and clinical conditions of 128 patients with greater than or equal to 10(5) cfu GBS/ml urine were studied by matching 128 patients with negative urine cultures (less than 10(2) cfu/ml) and 128 patients with comparable quantity of Escherichia coli. The incidence of acute lower urinary tract symptoms in patients with GBS was greater than that in patients with negative urine cultures (p less than 0.01), and the same as that in patients with E. coli. The incidence of fever was lower in patients with GBS than in those with E. coli (p less than 0.01). The incidence of urinary tract abnormalities was greatest in patients with GBS in urine. No GBS serotype seems to have particular affinity to the urinary tract."}, {"id": "pubmed23n0712_21136", "title": "[Clinical predictors of asymptomatic bacteriuria during pregnancy].", "score": 0.009523809523809525, "content": "To estimate the prevalence of asymptomatic bacteriuria among pregnant women attended at our university prenatal care clinic and to identify probable clinical predictors. Across-sectional study was carried out from August 2008 to October 2009 at the Bahiana School of Medicine involving 260 pregnant women without symptoms of urinary tract infection. The following exclusion criteria were considered: presence of clinical signs such as fever, dysuria, vesical tenesmus, lumbar pain, history of active genital bleeding or loss of amniotic fluid, use of antimicrobial agents in the 30 days prior to sample collection, and refusal to participate in the project. The presence of single pathogen bacterial colonization ≥10(5) CFU/mL in the urine sample obtained from the middle jet was considered to be a dependent variable. The predictive factors evaluated were as follows: age, race, marital status, schooling, gestational age, hypertension, anemia, vaginal infection, sickle cell trait and previous history of urinary tract infection, urinary symptoms related to the lower urinary tract (frequency, urgency and nocturia) and data obtained from the urine summary (leukocyturia, increased bacterial flora, hematuria, proteinuria, and presence of nitrite). Statistical analysis was performed with the Statistical Package for the Social Sciences (SPSS) software version 13.0 and the level of significance was set at p&lt;0.05. Prevalences were expressed as percentage, and the confidence interval considered was 95% (95%CI). The prevalence of asymptomatic bacteriuria was 12.3% (95%CI=8.3-16.3). E. coli was the most frequent etiologic agent (59.4%). Logistic regression indicated that urgency to void (OR=5.99; 95%CI=2.20-16.31; p&lt;0.001); leukocyturia (OR=2.85; 95%CI=1.04-7.83; p=0.042) and increased bacterial flora (OR=10.62; 95%CI=3.95-28.56; p&lt;0.001) were independent predictors of asymptomatic bacteriuria. The prevalence of asymptomatic bacteriuria in the studied population was high. The prediction score created for the final logistic regression model has an accuracy of 91.9% for bacteriuria."}, {"id": "wiki20220301en092_8402", "title": "Leukocyte esterase", "score": 0.0094875184695544, "content": "Leukocyte esterase (LE) is an esterase (a type of enzyme) produced by leukocytes (white blood cells). A leukocyte esterase test (LE test) is a urine test for the presence of white blood cells and other abnormalities associated with infection. White blood cells in the urine can indicate a urinary tract infection (UTI). Positive test results may be clinically significant in the right context. The LE test is also used to screen for gonorrhea and for amniotic fluid infections. The combination of the LE test with the urinary nitrite test provides an excellent screen for establishing the presence of a UTI. Urine test strips (dipsticks) can screen for both. A urine sample that tests positive for both nitrite and leukocyte esterase should be cultured for pathogenic bacteria."}, {"id": "pubmed23n1015_1941", "title": "Antimicrobial susceptibility of microorganisms causing Urinary Tract Infections in Saudi Arabia.", "score": 0.009433962264150943, "content": "Urinary Tract Infections (UTIs) is one of the most common infections worldwide. UTIs remain a challenge to the healthcare system because of the emergence of antimicrobial resistance. The aim of this study is to report the most common UTI-causative organisms associated with the emergence of antimicrobial resistance in Saudi Arabia. a retrospective cross sectional study of 1918 positive urine culture samples of both gender collected over 9 months (May 2015 to February 2016) from a major tertiary hospital in Riyadh, Saudi Arabia. the median age of individuals involved in the study was 43 years, with males constituting 27.7% only of the population. Among cases deemed complicated (81.1%), common causes were diabetes, pregnancy, and immunocompromization, comprising 24.7%, 11.9%, and 10.8%, respectively. Escherichia coli (52%) was the most common uropathogen, followed by Klebsiella pneumoniae (15%), Pseudomonas aeruginosa (8%) Streptococcus agalactiae (Group B streptococcus) (7%), and Enterococcus faecalis (5%). Overall sensitivity studies showed the most highly resistant uropathogen was Escherichia coli (60%) followed by Klebsiella pneumoniae (16%), Pseudomonas aeruginosa (4%) Enterococcus faecalis (3%), and Enterobacter cloacae (2%). Concerning the first defense antibiotics prescribed for UTI, E. coli was most frequently resistant to Sulfamethoxazole/Trimethoprim (47%) followed by ciprofloxacin (34%). K. pneumoniae was most frequently resistant to Sulfamethoxazole/trimethoprim (35%) followed by cefuroxime (30%), while P. aeruginosa to ciprofloxacin (13%). Because of a high level of antimicrobial resistance amongst uropathogens in Saudi Arabia, the development of regional and national UTI guidelines is recommended."}, {"id": "pubmed23n0634_6116", "title": "Prevalence of asymptomatic bacteriuria among pregnant women in Shiraz, Iran.", "score": 0.009433962264150943, "content": "To determine the frequency of asymptomatic bacteriuria in pregnant women referred to a University College Hospital in Shiraz, Iran for perinatal care, and also to determine the relation between asymptomatic bacteriuria and pyuria. This cross-sectional case series study included 389 healthy pregnant women who were referred to Hafez Hospital, Shiraz, Iran, antenatal care unit for regular perinatal care between May and August 2007. A specimen from each candidate was collected and processed following the standard microbiological technique. All the subjects were evaluated for bacteriuria. The mean age of the patients was 26.3 +/= 4.2 years. The prevalence of asymptomatic bacteriuria was 5.1%. From 75 (19.2%) patients who had &gt; or = 5 pus cells in high power field, only 12 (16%) had positive urine culture. The most common isolated microorganism was Escherichia coli (70%) followed by Staphylococcus aureus (20%) and Group B Streptococcus (5%). We found a rate of bacteriuria in our cohort of asymptomatic pregnant subject that is well within the reported range from the literature. A negative test for pyuria is not a reliable indicator of the absence of asymptomatic bacteriuria in pregnant women. To prevent asymptomatic bacteriuria complications, all pregnant women should be screened at the first antenatal visit."}, {"id": "pubmed23n0237_10590", "title": "[The frequency of bacteriuria in pregnancy].", "score": 0.009345794392523364, "content": "The authors present the results of a bacteriological urine analysis of 1774 pregnant women controlled in the Gynecological Dispensary Pesćenica in Zagreb. In 2824 examined urine specimens sterile urinocultures were found in 67.6% and bacteriuria in 32.4%. Significant findings of bacteriuria were recorded in 10.5% and insignificant ones in 21.9% of the specimens. The most common bacteria were E. coli (45.24%), then Staphylococcus albus epidermidis (17.4%), Staphylococcus pyogenes aureus (14.64%), Proteus mirabilis (9.39%), and Enterococcus (5.24%). Urine specimens were taken by the dip slide method. A rather high percentage of significant asymptomatic bacteriurias (10.5%) suggests the early detection and therapy in urinary infections as the best prevention of pyelonephritis gravidarum and gestosis. A routine bacteriological urine analysis in pregnant women should be one of the factors included in prenatal care."}, {"id": "pubmed23n0391_16967", "title": "[Management of pregnancy and delivery after augmentation cystoplasty].", "score": 0.009259259259259259, "content": "We report 2 cases of women who became pregnant and experienced vaginal delivery after augmentation cystoplasty. CASE 1: A 23-year-old woman with spina bifida became pregnant 3 years after augmentation sigmoidocystoplasty which had been performed to treat intractable urinary tract infection and urinary incontinence. During pregnancy, she developed febrile urinary tract infection twice which required antibiotics together with tight adherence to clean intermittent catheterization. At 36 weeks of gestation, she was safely delivered of a healthy baby. No deterioration of urinary continence level and renal function was observed after the delivery. CASE 2: A 32-year-old woman became pregnant 23 years after augmentation ileocecocystoplasty which had been performed to reconstruct diverted urinary tract due to a congenital hour-glass bladder. At 19 weeks of gestation, she developed acute pyelonephritis and hydronephrosis at right kidney which required antibiotics and indwelling urethral catheter. At 21 weeks of gestation, a drip infusion of ritodrine hydrochloride was started and maintained until 34 weeks of gestation to inhibit premature uterine contraction. At 29 weeks of gestation, she developed acute pyelonephritis and progressive hydronephrosis at left kidney, for which percutaneous nephrostomy drainage was deemed to be mandatory. She was delivered of a healthy baby at 36 weeks of gestation. Ten days after the delivery, both nephrostomy tube and indwelling urethral catheter were removed and clean intermittent catheterization was resumed. Total renal function was maintained during and after the pregnancy, and no deterioration of urinary continence was observed after the delivery. Since urinary tract infection is extremely common during pregnancy after augmentation cystoplasty, prevention and prompt intervention for urinary tract infection should be mandatory. Significant upper tract obstruction, if developed, should be treated by an effective urinary drainage. Thus, urological as well as obstetrical appropriate management is mandatory for the safe accomplishment of pregnancy and delivery after augmentation cystoplasty."}, {"id": "pubmed23n0074_16942", "title": "[Diagnosis of urinary tract infections in puerperium].", "score": 0.009259259259259259, "content": "In 225 puerperant urine proofs were taken both by suprapubic puncture and by mid stream technique. The corresponding proofs were examined culturally and microscopically. 42% of the mid stream proofs containing more than 10(5) germs/ml were evaluated to be false-positive. The corresponding urine taken by suprapubic puncture was uninfected. A pathologic leucocyte-count is just a little representative for urinary tract infection as a normal leucocyte count excludes an infection. Therefore, the counting of leucocytes is worthless and should be given up to the favour of bacteriologic examinations. Every ward for puerperant should presuppose the technique to take urine proofs by suprapubic puncture."}, {"id": "pubmed23n0292_22093", "title": "Symptomatic urinary tract infection in women in primary health care. Bacteriological, clinical and diagnostic aspects in relation to host response to infection.", "score": 0.009174311926605505, "content": "To evaluate rapid diagnostic tests for bacteriuria in women with symptoms of urinary tract infection (UTI), and to analyse bacteriological and clinical findings in relation to host response to infection. Prospective study of symptomatic UTI in women. Primary health care centres. 819 women with signs and symptoms suggestive of UTI. History of UTI and clinical findings were recorded. After randomization but before antibiotic treatment, urine specimens were analysed for pyuria by sediment microscopy and for nitrite using a test strip, and cultures were performed. The systemic inflammatory response was assessed by C-reactive protein (CRP), erythrocyte sedimentation rate, and total white blood cell count. The combined use of tests for pyuria and nitrite resulted in a high sensitivity (0.93) and efficacy (0.85) when the prevalence of bacteriuria was 0.89. Escherichia coli and Staphylococcus saprophyticus accounted for 93% of the urinary isolates. Significantly more patients infected with S. saprophyticus than E. coli complained of dysuria (p &lt; 0.05), frequency (p &lt; 0.05) or flank pain (p &lt; 0.01). CRP agreed best with the clinical diagnosis of acute pyelonephritis. In women with a high probability of bacteriuria, i.e. those with symptoms of lower UTI, examination for pyuria and urinary nitrite offers high diagnostic efficacy. If either or both tests are positive urine cultures can be omitted."}, {"id": "First_Aid_Step2_492", "title": "First_Aid_Step2", "score": 0.009104199893673578, "content": "Present with dysuria, urgency, frequency, suprapubic pain, and possibly hematuria. Children may present with bed-wetting, poor feeding, recurrent fevers, and foul-smelling urine. The differential includes vaginitis, STDs, urethritis or acute urethral syndrome, and prostatitis. Diagnosed by clinical symptoms. In the absence of symptoms, treatment is warranted only for children, those with anatomical GU tract anomalies, pregnant women, those with instrumented urinary tracts, patients scheduled for GU surgery, and renal transplant patients. Urine dipstick/UA: ↑ leukocyte esterase (a marker of WBCs) is 75% sensitive and up to 95% specif c. ↑ nitrites (a marker of bacteria), ↑ urine pH (Proteus infections), and hematuria (seen with cystitis) are also commonly seen. Microscopic analysis: Pyuria (> 5 WBCs/hpf) and bacteriuria (1 organism/hpf = 106 organisms/mL) are suggestive. Urine culture: The gold standard is > 105 CFU/mL."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": true, "char_ranges": [[60, 268]], "word_ranges": [[9, 41]], "text": "Regarding weight, metformin to a lesser extent, SGLT-2 inhibitors and, above all, GLP-1 agonists have been associated with a significant decrease in weight in patients with DM-2 (answers 1,3 and 4 incorrect)."}, "2": {"exist": true, "char_ranges": [[269, 450]], "word_ranges": [[41, 70]], "text": "On the other hand, pioglitazone, as reflected in its data sheet, can produce dose-dependent weight gain, mainly due to fat accumulation and added, in some cases, to water retention."}, "3": {"exist": true, "char_ranges": [[60, 268]], "word_ranges": [[9, 41]], "text": "Regarding weight, metformin to a lesser extent, SGLT-2 inhibitors and, above all, GLP-1 agonists have been associated with a significant decrease in weight in patients with DM-2 (answers 1,3 and 4 incorrect)."}, "4": {"exist": true, "char_ranges": [[60, 268]], "word_ranges": [[9, 41]], "text": "Regarding weight, metformin to a lesser extent, SGLT-2 inhibitors and, above all, GLP-1 agonists have been associated with a significant decrease in weight in patients with DM-2 (answers 1,3 and 4 incorrect)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "A somewhat more complicated question than the previous one. Regarding weight, metformin to a lesser extent, SGLT-2 inhibitors and, above all, GLP-1 agonists have been associated with a significant decrease in weight in patients with DM-2 (answers 1,3 and 4 incorrect). On the other hand, pioglitazone, as reflected in its data sheet, can produce dose-dependent weight gain, mainly due to fat accumulation and added, in some cases, to water retention.", "full_answer_no_ref": "A somewhat more complicated question than the previous one. Regarding weight, metformin to a lesser extent, SGLT-2 inhibitors and, above all, GLP-1 agonists have been associated with a significant decrease in weight in patients with DM-2 ([HIDDEN]). On the other hand, pioglitazone, as reflected in its data sheet, can produce dose-dependent weight gain, mainly due to fat accumulation and added, in some cases, to water retention.", "full_question": "A 66-year-old woman diagnosed with type 2 diabetes mellitus since three months ago. She has a BMI of 31 kg/m2 and presents poor glycemic control despite a program of non-pharmacological measures (healthy diet, exercise). Which of the following hypoglycemic drugs is associated with weight gain and should we avoid in this patient?:", "id": 522, "lang": "en", "options": {"1": "Metformin (biguanide).", "2": "Pioglitazone (thiazolidinedione).", "3": "Canagliflozin (sodium-glucose cotransporter 2 inhibitor- iSGLT2).", "4": "Liraglutide (GLP-1 receptor agonist).", "5": NaN}, "question_id_specific": 165, "type": "ENDOCRINOLOGY", "year": 2021, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1011_13119", "title": "Role of Metformin, Sodium-Glucose Cotransporter-2 (SGLT2) Inhibitors, Glucagon-Like Peptide-1 (GLP-1) Receptor Agonists, and Orlistat based Multidrug Therapy in Glycemic Control, Weight Loss, and Euglycemia in Diabesity: A Real-World Experience.", "score": 0.015575757575757576, "content": "This study evaluated the real-world weight loss and glycemic outcomes of multidrug therapy (MDT) according to various combinations of metformin, sodium-glucose cotransporter -2 inhibitor (SGLT2i), glucagon-like peptide-1 receptor analogs (GLP1a), and orlistat in diabesity. Data retrospectively captured from medical records of 2 different centers in New Delhi for patients &gt;35 years-age having prediabetes/diabetes and on at least any one of the 4 above medications with &gt;6-months follow-up was analyzed. In total, 5,336 patient records were screened; 2,442 with prediabetes/diabetes were considered; 1,509 patients who fulfilled all criteria were analyzed. Use of metformin, SGLT2i, sulfonylureas, DPP4i, pioglitazone, orlistat, and GLP1a was 85.35%, 74.95%, 68.32%, 60%, 39.16%, 9.08%, and 4.17%, respectively. However, 365, 970, and 104 patients were on one of 4 concerned medications (Group-1; 24.18%), dual MDT (Group-2; 64.28%), and triple/quadruple MDT (Group-3; 6.89%). Metformin with SGLT2i was most commonly used dual MDT (94.12%). Analysis according to weight-loss quartiles from 558 patients showed 6.9 kg weight-loss in the highest quartile. People losing maximum weight were significantly younger; had higher use of metformin, SGLT2i, GLP1, orlistat, and lower pioglitazone use; greatest HbA1c reduction (-1.3 vs. -0.3; quartile-1 vs. quartile -4; <iP</i &lt; 0.001); and significantly higher occurrence of HbA1c&lt;5.7% (16.8% vs. 6.29%; quartile-1 vs. 4; <iP</i &lt; 0.001). Patients in Group-3 had the highest baseline BMI and maximum weight loss with highest number of patients with HbA1c&lt;5.7% (19.44% vs. 10.34%; Group-3 vs. Group-1; <iP</i &lt; 0.001). Greater weight loss with HbA1c reduction along with a greater number of patients attaining HbA1c &lt;5.7% highlights that MDT is the way forward to tackle diabesity in India."}, {"id": "pubmed23n0851_1776", "title": "Body Weight Gain and Hyperphagia After Administration of SGLT-2 Inhibitor: A Case Report.", "score": 0.015517241379310345, "content": "A detailed description is given of a case we encountered in which unexpectedly marked weight gain occurred following a treatment switch from a GLP-1 receptor agonist to an SGLT-2 inhibitor The patient, a 44-year-old man with type 2 diabetes mellitus, had gained about 10 kg in weight in the previous year. Therefore, metformin was replaced with liraglutide to obtain reduction of body weight. Although the patient lost about 8 kg (7%), during the 18-month period on the medication, the weight loss stabilized; therefore, the treatment was again switched to tofogliflozin to obtain further reduction of body weight. However, the patient reported increasing hunger and an exaggerated appetite from week 3 onward after the start of tofogliflozin, and gained about 9 kg in weight within 2 weeks, associated with a tendency towards increased HbA1c; therefore, tofogliflozin was discontinued. Immediate reinstitution of liraglutide resulted in reduction of the increased appetite, weight, and HbA1c level. Caution should be exercised against hyperphagia and weight gain due to hunger that may occur following discontinuation of a GLP-1 receptor agonist and/or initiation of an SGLT-2 inhibitor."}, {"id": "pubmed23n0997_19526", "title": "[Management of hyperglycaemia with non-insulin drugs in adult patients with type 2 diabetes].", "score": 0.014689089587875011, "content": "Treatment of diabetes mellitus type2 (DM2) includes healthy eating and exercise (150minutes/week) as basic pillars. For pharmacological treatment, metformin is the initial drug except contraindication or intolerance; in case of poor control, 8 therapeutic families are available (6 oral and 2 injectable) as possible combinations. An algorithm and some recommendations for the treatment of DM2 are presented. In secondary cardiovascular prevention, it is recommended to associate an inhibitor of the sodium-glucose cotransporter type 2 (iSGLT2) or a glucagon-like peptide-1 receptor agonist (arGLP1) in patients with obesity. In primary prevention if the patient is obese or overweight metformin should be combined with iSGLT2, arGLP1, or inhibitors of type4 dipeptidylpeptidase (iDPP4). If the patient does not present obesity, iDPP4, iSGLT2 or gliclazide, sulfonylurea, recommended due to its lower tendency to hypoglycaemia, may be used."}, {"id": "pubmed23n0909_334", "title": "Is insulin the preferred treatment for HbA1c &gt;9%?", "score": 0.014581396486764202, "content": "The algorithms and guidelines of the American Association of Clinical Endocrinologists and the American Diabetes Association recommend that insulin administration be strongly considered for people with type 2 diabetes (T2D) with HbA1c levels exceeding 9.0% and 10%, respectively. Although the caveat is given in both sets of recommendations that this is particularly appropriate when patients are \"symptomatic,\" referring to urinary frequency with increased thirst and appetite, weight loss, and ketosis, the clinical definition of such presentations may be ill-defined, and it is noteworthy that both documents consider insulin to offer particular benefit under such circumstances. However, with multiple options for glycemic treatment, it is of interest to reconsider this argument for insulin use. It should be recalled that in the UK Prospective Diabetes Study, diet alone was associated with a reduction in HbA1c from 9% to 7%. Drug-naïve people with T2D do often show surprisingly strong reductions in HbA1c with metformin-based dual-agent oral treatment approaches; a recent report showed that even with baseline HbA1c &gt;11%, the combination of metformin with a sulfonylurea, pioglitazone, or sitagliptin was associated with reduction in HbA1c from 11.6% to 6.0%. A 32-week study of the combination of rosiglitazone with metformin in patients with mean baseline HbA1c 8.9% showed a mean HbA1c reduction of 2.3%, and an open-label cohort with baseline HbA1c 11.8% had a reduction in HbA1c to 7.8%. With metformin plus sitagliptin, a mean placebo-adjusted HbA1c reduction of 2.1% from a baseline of 8.8% was reported, with those patients with baseline HbA1c &gt;9% having a 2.6% reduction in HbA1c, and an open-label cohort with baseline HbA1c 11.2% having a 2.9% reduction in HbA1c. Similar 2% HbA1c reductions from baseline levels of 9.1% were seen with metformin in initial combination with the sodium-glucose cotransporter 2 (SGLT2) inhibitor dapagliflozin. Although such dual oral agent approaches are more effective than monotherapy, with a combination regimen the HbA1c reduction will not be directly additive, because the expected reduction decreases at lower baseline HbA1c levels. As an example of this, administration of canagliflozin 300 mg daily to patients with baseline HbA1c &gt;9% reduced levels from 9.6% by 1.8%, whereas at a baseline HbA1c of 10% either canagliflozin 300 mg or metformin 2 g/day reduced HbA1c by 2%; the addition of both agents led to an HbA1c reduction by somewhat less than 3%, which appears concordant with a reduction by the second agent from approximately 8% (10% to 2%). Similar less-than-additive effects of the addition of exenatide QW to dapagliflozin have been reported, with HbA1c reduction from a baseline of 10.0%-10.1% of 1.9% and 1.6% with the individual agents, respectively, and a reduction of 2.2% with their combination. However, one may consider these approaches inferior to the expected HbA1c reduction with insulin, suggesting that insulin should, indeed, be the preferred treatment for people with T2D and HbA1c &gt;9%. Rather, studies comparing basal insulin directly with glucagon-like peptide-1 (GLP-1) receptor agonists (RA) suggest that the latter agents may offer superior benefit. The Diabetes Therapy Utilization: Researching Changes in HBA1C, Weight, and Other Factors Through Intervention with Exenatide Once Weekly (DURATION)-3 and Liraglutide Effect and Action in Diabetes (LEAD)-5 studies compared exenatide QW and liraglutide, respectively, with insulin glargine. Those study participants in the highest quartile of baseline HbA1c had levels ≥9.0% and ≥8.9%, with the GLP-1RA leading to 0.3% and 0.2% greater reductions in HbA1c, respectively, than insulin glargine. Another study comparing T2D patients receiving oral agents given liraglutide with those given insulin glargine showed that those in the highest baseline HbA1c quartile (mean 10.6%) had an HbA1c reduction of 3.1% with either agent. In the exenatide QW study, the reduction in HbA1c with this agent exceeded that with insulin glargine for those groups of study participants with HbA1c 9.0%-9.4%, 9.5%-9.9%, 10.0%-10.4%, 10.5%-10.9%, and even ≥11.0%. Similar superiority of the HbA1c-lowering effect of exenatide QW compared with that of insulin glargine was reported in a study with baseline HbA1c 8.5%. An individual-patient meta-analysis of six studies of another weekly GLP-1RA, namely dulaglutide, showed that at a baseline HbA1c of 10% the expected HbA1c reduction would be nearly 2.5%, and a study directly comparing dulaglutide with insulin glargine also showed a superior HbA1c-lowering effect of the former. Another advantage of the GLP-1RAs is their association with weight loss, rather than the weight gain associated with insulin treatment. An interesting potential combination is that of a GLP-1RA with a thiazolidinedione. In a study comparing the addition of exenatide QW and pioglitazone with the addition of basal-bolus insulin in 101 people receiving sulfonylureas and metformin with baseline HbA1c &gt;10%, HbA1c fell from &gt;11% by &gt;4% compared with &lt;4%, respectively, and the GLP-1RA plus thiazolidinedione treatment was associated with less weight gain and hypoglycemia. What can we conclude? Should HbA1c 11% be the new \"use insulin\" point? Insulin is an important part of our armamentarium for T2D, and is certainly needed for many patients, but with current therapeutic approaches including metformin, incretin-based treatments, SGLT2 inhibitors, and, possibly, thiazolidinediones, we can reconsider its use in many instances. Although there is no doubt that insulin is necessary for truly uncontrolled diabetes, we may wish to better define its correct indications."}, {"id": "pubmed23n0997_15301", "title": "Efficacy of SGLT2 Inhibitors as the Fifth Drug in the Management of Type 2 Diabetes Mellitus in Asian Indians not Controlled with at least 4 Oral Antidiabetic Drugs.", "score": 0.014568764568764568, "content": "To evaluate the efficacy of SGLT2 inhibitors as an add-on therapy along with stricter lifestyle modification in Asian Indian type 2 diabetes mellitus (T2DM) patients with inadequate glycemic control despite receiving an optimum dose of at least 4 oral antidiabetic drugs (OADs). A retrospective analysis of data of 808 T2DM patients being treated with an SGLT2 inhibitor (Dapagliflozin, Empagliflozin or Canagliflozin) as an add-on drug in patients with inadequate glycemic control despite receiving optimum doses of at least any four OADs(metformin, sulphonylureas, pioglitazone, DPP4 Inhibitors, alpha-Glucosidase Inhibitors) and who preferred not to initiate insulin. The average age of the patients included was 51.63 years (SD ± 9.88). 57.7% were males. Average weight was 81.95±16.08 kg. Mean duration of diabetes was 34.08±39.04 months. The mean baseline fasting plasma glucose was 198.21 ± 38.21 mg/dl and mean post prandial plasma glucose was 264.22 ± 45.22 mg/ dl. The baseline HbA1c was 8.92 ± 1.47 %. Total 87.4 % of the cases responded to addition of SGLT2 inhibitors during a mean follow-up period of 6 months. The fasting plasma glucose (FBS) was reduced by -63.65 ± 19.93 mg/dl to a mean FBS of 134.57 ± 33.65 mg/dl (P=0.001). The post prandial plasma glucose (PPBS) was reduced by -79.28 ± 23.57 mg/dl to a mean PPBS of 184.94 ± 38.34 mg/dl (P=0.001). The mean HbA1c reduced significantly by -1.63 ± 0.99 % (P= 0.001). The mean weight reduction at 6 months of therapy was -3.03± 01.84 kg that is 3.8 % decrease from baseline (p=0.001).The response in age group &lt; 55 years was 90.9 %, whereas in ≥55 years, it was 82.2% (p=0.001). The males responded more (91.0%) compared to females (82.5%) (p=0.001). Those with BMI &lt; 23.5 kg/ m2 had marginally higher but insignificant response of 93.0% as compared to 87.1% in patients with high a BMI (≥23.5 kg/m2) (p=0.253). Patients with &lt; 5years duration of diabetes responded better (91.8%) as compared to patients with a ≥ 5 years of diabetes (85.4%). SGLT2 inhibitors are effective in achieving desired glycemic goals even when used as a fifth add-on drug along with strict lifestyle modification in patients with inadequate glycemic control despite receiving an optimum dose of at least 4 oral antidiabetic drugs (OADs). SGLT2 inhibitors can be effectively used at any stage of diabetes."}, {"id": "pubmed23n1061_4543", "title": "[Short-term Glucose Lowering Effects of Sodium-glucose Cotransporter 2 Inhibitors Confirmed by Flash Glucose Monitoring in Two Outpatients with Type 1 Diabetes].", "score": 0.014545646884586903, "content": "Case 1 was a 41-year-old man with type 1 diabetes. He presented with poor glycemic control [hemoglobin A1c (HbA1c) of 8-9%] despite treatment with more than 20 units/day of insulin and 150 mg of miglitol. Before administration of sodium glucose cotransporter 2 (SGLT2) inhibitor, hyperglycemia was noted mainly at night by Flash Glucose Monitoring (FGM). Administration of ipragliflozin at 50 mg improved the hyperglycemia mainly at night (mean blood glucose, before administration: 205 mg/dl, day 6 of treatment: 119 mg/dl). Two months later, the HbA1c improved to 7.2% without hypoglycemia or ketosis. Case 2 was a 46-year-old woman with type 1 diabetes. She was morbidly obese and presented with poor glycemic control (HbA1c: 9-11%) although she was being treated with more than 50 units/day of insulin and 2,250 mg of metformin. Before administration of SGLT2 inhibitor, hyperglycemia was noted to be mainly nocturnal by FGM. Administration of dapagliflozin at 5 mg improved the hyperglycemia mainly at night on day 2 with improvement in the mean blood glucose level from 188 mg/dl before administration to 128 mg/dl on day 5. Four months later, the HbA1c improved to 8.0% without hypoglycemia and ketosis, and her body weight decreased from 92.1 to 89.8 kg. The hypoglycemic effect of SGLT2 inhibitors is independent of insulin. These agents also have various other effects, including weight loss, improvement of blood pressure and lipid metabolism. Here we report the short-term glucose lowering effects of two SGLT2 inhibitors, as confirmed by FGM, in two outpatients with type 1 diabetes."}, {"id": "pubmed23n1025_11433", "title": "Type 2 Diabetes Remission and Substantial Body Weight Reduction Achieved with Metformin and a Sodium-Glucose Cotransporter 2 Inhibitor.", "score": 0.014509605662285137, "content": "The overall goal in the treatment of type 2 diabetes mellitus (T2DM) is remission. However, the effects of a sodium-glucose cotransporter 2 inhibitor (SGLT2i) on remission of T2DM are unknown. We herein report a case involving an overweight 43-year-old man who completely recovered from T2DM after SGLT2i therapy (dapagliflozin at 5 mg/day). In the pretreatment period, he had a body mass index (BMI) of 26.0 kg/m<sup2</sup, hemoglobin A1c (HbA1c) concentration of 10.3%, advanced insulin resistance, pancreatic β-cell dysfunction, and fatty liver. Eighteen months after comprehensive therapy, including the administration of an SGLT2i and metformin, his BMI had decreased to 21.3 kg/m<sup2</sup and his glycemic control was almost normal (HbA1c of 5.3%) despite discontinuation of all hypoglycemic medications. This report is the first to propose the usefulness of the combination therapy of SGLT2i and metformin for achieving normal body weight and remission of newly diagnosed T2DM in a real-world clinical situation."}, {"id": "pubmed23n0790_2074", "title": "Effects of replacing metformin with pioglitazone on glycemic control in japanese patients with poorly controlled type 2 diabetes mellitus: A 12-week, open-label, prospective study.", "score": 0.014052540081128067, "content": "Insulin resistance is a critical aspect of the pathophysiology of type 2 diabetes mellitus and is also associated with other risk factors for cardiovascular disease (eg, dyslipidemia and hypertension). Accordingly, insulin resistance is a possible target for lowering plasma glucose concentration and preventing diabetic macroangiopathy. Biguanides, such as metformin, and thiazolidinediones (TZDs), such as pioglitazone, improve insulin resistance. The aims of this study were to assess the effects of replacing a biguanide with a TZD on glycemic control in patients with poorly controlled type 2 diabetes mellitus, and also to identify the factors affecting interpatient variation in the effects of treatment change. This was a 12-week, open-label, prospective study in which previously prescribed metformin (500 or 750 mg/d) was replaced with pioglitazone (15 or 30 mg/d) in patients with poorly controlled type 2 diabetes mellitus. Patients with a glycosylated hemoglobin (HbA1c) concentration &gt;7% despite treatment with diet, exercise, and hypoglycemic agents other than TZDs were eligible for the study. Patients who never received TZDs were also eligible for inclusion. Vital signs, metabolic parameters, and arterial stiffness were assessed at baseline and after 12 weeks of treatment with pioglitazone. The primary end point was change in HbA1c concentration after replacing metformin with pioglitazone. Tolerability was assessed by medical history, physical examination, and laboratory tests (aspartate aminotransferase, alanine aminotransferase, and γ-glutamyl transpeptidase). Twenty-one Japanese patients (15 women, 6 men; mean [SD] age, 61.8 [8.4] years; body mass index, 25.5 [3.0] kg/m(2)) were included in the study. HbA1c concentration was not significantly changed from baseline after 12 weeks of pioglitazone treatment (8.0% [0.7%] vs 8.2% [0.7%]). Fasting plasma glucose (FPG) concentration also was not significantly changed after the replacement of treatment (156 [27] vs 144 [30] mg/dL). In addition, the resistin concentration did not change significantly from baseline after 12 weeks of pioglitazone treatment (6.6 [3.8] vs 6.4 [3.6] ng/mL). In contrast, significant improvement from baseline was observed in triglyceride (TG) concentrations (157 [109] vs 117 [68] mg/dL; P = 0.003), high-density lipoprotein cholesterol (HDL-C) (55 [12] vs 61 [16] mg/dL; P = 0.016), remnant-like particle cholesterol (6.6 [6.0] vs 5.3 [3.5] mg/dL; P = 0.048), and serum adiponectin (8.8 [4.3] vs 23.3 [11.7] μg/mL; P &lt; 0.001). Pulse wave velocity was also significantly improved (1730 [361] vs 1622 [339] m/sec; P = 0.009). Changes in HbA1c were significantly correlated with serum fasting insulin concentration at baseline in the patients not receiving insulin preparations (r = -0.635, P = 0.013). The percentage change in serum adiponectin concentration was correlated with the percentage changes in HbA1c and FPG concentrations (HbA1c, r = -0.518, P = 0.019; FPG, r = -0.594, P = 0.006). Body weight was significantly increased after treatment (62.6 [11.9] vs 65.5 [12.2] kg; P &lt; 0.001). Mild edema was reported in 5 patients. One patient discontinued treatment due to an increase in serum creatine kinase activity to ~6.6 times the upper limit of normal. Replacement of metformin with pioglitazone did not produce significant differences in HbA1c and FPG concentrations from baseline after 12 weeks of treatment in these patients with poorly controlled type 2 diabetes mellitus. However, the replacement was effective in a subset of patients whose serum insulin concentrations were high or whose serum adiponectin concentrations were sensitive to TZDs. In addition, the replacement was associated with significant improvements in TG, HDL-C, serum adiponectin concentration, pulse wave velocity, and body weight increase from baseline."}, {"id": "InternalMed_Harrison_27815", "title": "InternalMed_Harrison", "score": 0.01370873786407767, "content": "lost 5–7% of their body weight during the 3 years of the study. Studies in Finnish and Chinese populations noted similar efficacy of diet and exercise in preventing or delaying type 2 DM. A number of agents, including α-glucosidase inhibitors, metformin, thiazolidinediones, GLP-1 receptor pathway modifiers, and orlistat, prevent or delay type 2 DM but are not approved for this purpose. Individuals with a strong family history of type 2 DM and individuals with IFG or IGT should be strongly encouraged to maintain a normal BMI and engage in regular physical activity. Pharmacologic therapy for individuals with prediabetes is currently controversial because its cost-effectiveness and safety profile are not known. The ADA has suggested that metformin be considered in individuals with both IFG and IGT who are at very high risk for progression to diabetes (age <60 years, BMI ≥35 kg/m2, family history of diabetes in first-degree relative, and women with a history of GDM). Individuals with IFG,"}, {"id": "pubmed23n0624_7324", "title": "Efficacy and safety of the human glucagon-like peptide-1 analog liraglutide in combination with metformin and thiazolidinedione in patients with type 2 diabetes (LEAD-4 Met+TZD).", "score": 0.013000717572073783, "content": "To determine the efficacy and safety of liraglutide (a glucagon-like peptide-1 receptor agonist) when added to metformin and rosiglitazone in type 2 diabetes. This 26-week, double-blind, placebo-controlled, parallel-group trial randomized 533 subjects (1:1:1) to once-daily liraglutide (1.2 or 1.8 mg) or liraglutide placebo in combination with metformin (1 g twice daily) and rosiglitazone (4 mg twice daily). Subjects had type 2 diabetes, A1C 7-11% (previous oral antidiabetes drug [OAD] monotherapy &gt;or=3 months) or 7-10% (previous OAD combination therapy &gt;or=3 months), and BMI &lt;or=45 kg/m(2). Mean A1C values decreased significantly more in the liraglutide groups versus placebo (mean +/- SE -1.5 +/- 0.1% for both 1.2 and 1.8 mg liraglutide and -0.5 +/- 0.1% for placebo). Fasting plasma glucose decreased by 40, 44, and 8 mg/dl for 1.2 and 1.8 mg and placebo, respectively, and 90-min postprandial glucose decreased by 47, 49, and 14 mg/dl, respectively (P &lt; 0.001 for all liraglutide groups vs. placebo). Dose-dependent weight loss occurred with 1.2 and 1.8 mg liraglutide (1.0 +/- 0.3 and 2.0 +/- 0.3 kg, respectively) (P &lt; 0.0001) compared with weight gain with placebo (0.6 +/- 0.3 kg). Systolic blood pressure decreased by 6.7, 5.6, and 1.1 mmHg with 1.2 and 1.8 mg liraglutide and placebo, respectively. Significant increases in C-peptide and homeostasis model assessment of beta-cell function and significant decreases in the proinsulin-to-insulin ratio occurred with liraglutide versus placebo. Minor hypoglycemia occurred more frequently with liraglutide, but there was no major hypoglycemia. Gastrointestinal adverse events were more common with liraglutide, but most occurred early and were transient. Liraglutide combined with metformin and a thiazolidinedione is a well-tolerated combination therapy for type 2 diabetes, providing significant improvements in glycemic control."}, {"id": "wiki20220301en595_19313", "title": "Dapagliflozin/saxagliptin/metformin", "score": 0.01299715909090909, "content": "Medical uses In the United States dapagliflozin/saxagliptin/metformin is indicated as an adjunct to diet and exercise to improve glycemic control in adults with type 2 diabetes. In the European Union it is indicated in adults aged 18 years and older with type 2 diabetes mellitus: to improve glycemic control when metformin with or without sulphonylurea (SU) and either saxagliptin or dapagliflozin does not provide adequate glycemic control. when already being treated with metformin and saxagliptin and dapagliflozin. References External links Anti-diabetic drugs AstraZeneca brands Biguanides Chloroarenes Combination drugs Glucosides Guanidines Phenol ethers SGLT2 inhibitors"}, {"id": "wiki20220301en596_20006", "title": "Canagliflozin/metformin", "score": 0.012755762755762755, "content": "Medical uses Canagliflozin/metformin is indicated in adults aged 18 years of age and older with type 2 diabetes as an adjunct to diet and exercise to improve glycemic control. Adverse effects To lessen the risk of developing ketoacidosis (a serious condition in which the body produces high levels of blood acids called ketones) after surgery, the FDA approved changes to the prescribing information for SGLT2 inhibitor diabetes medicines to recommend they be stopped temporarily before scheduled surgery. Canagliflozin, dapagliflozin, and empagliflozin should each be stopped at least three days before, and ertugliflozin should be stopped at least four days before scheduled surgery. Symptoms of ketoacidosis include nausea, vomiting, abdominal pain, tiredness, and trouble breathing. References Further reading External links Anti-diabetic drugs Biguanides Combination drugs Fluoroarenes Glucosides Guanidines SGLT2 inhibitors Thiophenes"}, {"id": "wiki20220301en607_26383", "title": "Pioglitazone/glimepiride", "score": 0.012702472293265132, "content": "Medical uses In the United States pioglitazone/glimepiride is indicated as an adjunct to diet and exercise to improve glycemic control in adults with type 2 diabetes mellitus who are already treated with a thiazolidinedione and sulfonylurea or who have inadequate glycemic control on a thiazolidinedione alone or a sulfonylurea alone. In the European Union pioglitazone/glimepiride is indicated for the treatment of people with type 2 diabetes mellitus who show intolerance to metformin or for whom metformin is contraindicated and who are already treated with a combination of pioglitazone and glimepiride. References External links Anti-diabetic drugs"}, {"id": "pubmed23n0995_2491", "title": "Therapeutic strategies for type 2 diabetes mellitus in women after menopause.", "score": 0.012675844429398932, "content": "As type 2 diabetes mellitus (T2DM) is affected by both chronological and ovarian ageing, it is common in postmenopausal women. This review analyses and critically appraises the literature regarding the optimal therapeutic strategies for T2DM in women after menopause. Lifestyle interventions, including changes in dietary habits and physical exercise in everyday life targeting a modest weight loss (5%), represent the cornerstone of management. Limited intake of alcohol and sodium, as well as smoking cessation, are additional lifestyle changes for both endothelial and bone health. Regarding medications, postmenopausal women should be initially treated with metformin, concurrently with lifestyle intervention. If glycosylated haemoglobin (HbA<sub1c</sub) remains over the target level (usually ≥7%), dipeptidyl peptidase-4 inhibitors (DPP-4i) or glucagon-like peptide-1 receptor agonists (GLP-1RA) should be preferred. Thiazolidinediones (TZDs) and canagliflozin should be avoided in postmenopausal women with increased fracture risk. Insulin should be used with caution to avoid hypoglycaemia. Bariatric surgery is a well established and effective therapeutic option for both weight loss and glycaemic control in very obese patients with T2DM; however, metabolic benefits should be balanced against nutritional deficiencies that often present after surgery. Proper control of hypertension, with avoidance of hypotension, is of great importance as a measure against falls. Annual tests for retinopathy and neuropathy are crucial for the same reason. Menopausal hormone therapy (MHT) has a beneficial effect on glucose homeostasis, reduces the risk of new-onset T2DM and improves glucose control in women with T2DM. T2DM has been considered a cardiovascular disease equivalent, which meant that postmenopausal women with the disease could not take MHT but current evidence supports an individualised approach to this issue. Therapeutic strategies for women with T2DM after menopause should aim to maximise benefits for metabolic, cardiovascular and bone health with the minimum of adverse effects, bearing in mind that most women will spend more than one-third of their life being of postmenopausal status."}, {"id": "wiki20220301en204_12500", "title": "Liraglutide", "score": 0.012455990611330417, "content": "Liraglutide was approved for medical use in the European Union in 2009, and in the United States in 2010. In 2019, it was the 142nd most commonly prescribed medication in the United States, with more than 4million prescriptions. Medical uses Liraglutide is a medication used for the treatment of type 2 diabetes or obesity. Type 2 diabetes Liraglutide improves control of blood glucose. As of 2017 it is unclear if incretin mimetics like liraglutide affect a person's risk of death. In diabetes it is a less preferred agent. It may be used in those in who metformin and another antidiabetic medication such as a sulfonylurea are not sufficient. Obesity Liraglutide may also be used together with diet and exercise for chronic weight management in adult patients. The body mass index (BMI) needs to be greater than 30 kg/m2, or greater than 27 kg/m2 together with high blood pressure, type 2 diabetes mellitus, or dyslipidemia."}, {"id": "pubmed23n0634_7166", "title": "A cardiologic approach to non-insulin antidiabetic pharmacotherapy in patients with heart disease.", "score": 0.012136752136752138, "content": "Classical non-insulin antihyperglycemic drugs currently approved for the treatment of type 2 diabetes mellitus (T2DM) comprise five groups: biguanides, sulfonylureas, meglitinides, glitazones and alpha-glucosidase inhibitors. Novel compounds are represented by the incretin mimetic drugs like glucagon like peptide-1 (GLP-1), the dipeptidyl peptidase 4 (DPP-4) inhibitors, dual peroxisome proliferator-activated receptors (PPAR) agonists (glitazars) and amylin mimetic drugs. We review the cardiovascular effects of these drugs in an attempt to improve knowledge regarding their potential risks when treating T2DM in cardiac patients. Metformin may lead to lethal lactic acidosis, especially in patients with clinical conditions that predispose to this complication, such as recent myocardial infarction, heart or renal failure. Sulfonylureas exert their effect by closing the ATP-dependent potassium channels. This prevents the opening of these channels during myocardial ischemia, impeding the necessary hyperpolarization that protects the cell. The combined sulfonylurea/metformin therapy reveals additive effects on mortality in patients with coronary artery disease (CAD). Meglitinides effects are similar to those of sulfonylureas, due to their almost analogous mechanism of action. Glitazones lower leptin levels, leading to weight gain and are unsafe in NYHA class III or IV. The long-term effects of alpha-glucosidase inhibitors on morbidity and mortality rates is yet unknown. The incretin GLP-1 is associated with reductions in body weight and appears to present positive inotropic effects. DPP-4 inhibitors influences on the cardiovascular system seem to be neutral and patients do not gain weight. The future of glitazars is presently uncertain following concerns about their safety. The amylin mimetic drug paramlintide, while a satisfactory adjuvant medication in insulin-dependent diabetes, is unlikely to play a major role in the management of T2DM. Summarizing the present information it can be stated that 1. Four out the five classical oral antidiabetic drug groups present proven or potential cardiac hazards; 2. These hazards are not mere 'side effects', but biochemical phenomena which are deeply rooted in the drugs' mechanism of action; 3. Current data indicate that the combined glibenclamide/metformin therapy seems to present special risk and should be avoided in the long-term management of T2DM with proven CAD; 4. Glitazones should be avoided in patients with overt heart failure; 5, The novel incretin mimetic drugs and DPP-4 inhibitors--while usually inadequate as monotherapy--appear to be satisfactory adjuvant drugs due to the lack of known undesirable cardiovascular effects; 6. Customized antihyperglycemic pharmacological approaches should be implemented for the achievement of optimal treatment of T2DM patients with heart disease. In this context, it should be carefully taken into consideration whether the leading clinical status is CAD or heart failure."}, {"id": "wiki20220301en343_31357", "title": "Sitagliptin/metformin", "score": 0.011230509903076275, "content": "Medical uses In the United States, sitagliptin/metformin is indicated as an adjunct to diet and exercise to improve glycemic control in adults with type 2 diabetes. In the European Union, sitagliptin/metformin is indicated as an adjunct to diet and exercise to improve glycemic control in people with type 2 diabetes; in combination with a sulfonylurea as an adjunct to diet and exercise \"in people inadequately controlled on their maximal tolerated dose of metformin and a sulfonylurea; as triple combination therapy with a peroxisome proliferator-activated receptor (PPAR) agonist (i.e., a thiazolidinedione) as an adjunct to diet and exercise in people inadequately controlled on their maximal tolerated dose of metformin and a PPAR agonist; and as add on to insulin as an adjunct to diet and exercise to improve glycemic control in people when stable dosage of insulin and metformin alone do not provide adequate glycemic control.\""}, {"id": "wiki20220301en497_24032", "title": "Semaglutide", "score": 0.010884281504685551, "content": "Medical uses Semaglutide is indicated as an adjunct to diet and exercise to improve glycemic control in adults with type 2 diabetes. In the United States, semaglutide is also indicated as an adjunct to diet and exercise for long-term weight management in adults with obesity (initial body mass index (BMI) ≥ 30 kg/m2) or overweight (initial BMI ≥ 27 kg/m2) with at least one weight-related comorbidity. Semaglutide was found to be inferior to tirzepatide, in a study of tirzepatide (LY3298176) vs semaglutide once weekly as add-on therapy to metformin in participants with type 2 diabetes (SURPASS-2), in both endpoints of reduction in A1C and body weight, with a roughly similar safety profile."}, {"id": "wiki20220301en247_9039", "title": "Albiglutide", "score": 0.010478679637965248, "content": "Albiglutide (trade names Eperzan in Europe and Tanzeum in the US) is a glucagon-like peptide-1 agonist (GLP-1 agonist) drug marketed by GlaxoSmithKline (GSK) for treatment of type 2 diabetes. As of 2017 it is unclear if it affects a person's risk of death. GSK has announced that it intends to withdraw the drug from the worldwide market by July 2018 for economic reasons. Medical uses Albiglutide is used for the treatment of type 2 diabetes in adults. It can be used alone (if metformin therapy is ineffective or not tolerated) or in combination with other antidiabetic drugs, including insulins. According to a 2015 analysis, albiglutide is less effective than other GLP-1 agonists for lowering glycated hemoglobin (HbA1c, an indicator for long-term blood glucose control) and weight loss. It also seems to have fewer side effects than most other drugs of this class, except for reactions at the injection site which are more common under albiglutide than, for example, under liraglutide."}, {"id": "wiki20220301en596_20005", "title": "Canagliflozin/metformin", "score": 0.0102538942720471, "content": "Canagliflozin/metformin, sold under the brand name Vokanamet among others, is a fixed-dose combination anti-diabetic medication used for the treatment of type 2 diabetes. It is used in combination with diet and exercise. It is taken by mouth. The most common side effects include hypoglycemia (low blood glucose levels) when used in combination with insulin or a sulphonylurea and vulvovaginal candidiasis (thrush, a fungal infection of the female genital area caused by Candida). Canagliflozin/metformin was approved for medical use in the European Union in April 2014, and for use in the United States in August 2014. Medical uses Canagliflozin/metformin is indicated in adults aged 18 years of age and older with type 2 diabetes as an adjunct to diet and exercise to improve glycemic control. Adverse effects"}, {"id": "article-36052_30", "title": "Type 2 Diabetes -- Treatment / Management", "score": 0.010205125581871196, "content": "If adequate glycemia cannot be achieved, metformin is the first-line therapy. Following metformin, many other therapies such as oral sulfonylureas, dipeptidyl peptidase-4 (DPP-4) inhibitors. Glucagon-like peptide-1 (GLP-I) receptor agonists, Sodium-glucose co-transporter-2 (SGLT2) inhibitors, pioglitazone, especially if the patient has fatty liver disease, alpha-glucosidase inhibitors, and insulin, are available. Recent studies have shown that the SGLT2 inhibitor, empagliflozin (EMPA), and the GLP-1 receptor agonist, liraglutide, reduce significant cardiovascular (CV) events and mortality. Hence, in patients with CV disease, these drugs should be considered next. For patients with T1DM, a regime of basal-bolus insulin is the mainstay of therapy. Also, insulin pump therapy is a reasonable choice. Since hypoglycemia portends increased mortality, preference should be given to therapies that do not induce hypoglycemia, for example, DPP-4 Inhibitors, SGLT-2 inhibitors, GLP-I receptor agonists, and pioglitazone with metformin. The other advantages of SGLT-2 inhibitors and GLP-I receptor agonists are a reduction in body weight, blood pressure (BP), and albuminuria."}, {"id": "wiki20220301en008_126432", "title": "Type 2 diabetes", "score": 0.010034228067014952, "content": ", there is insufficient data to recommend nonnutritive sweeteners, which may help reduce caloric intake. Medications Blood sugar control There are several classes of anti-diabetic medications available. Metformin is generally recommended as a first line treatment as there is some evidence that it decreases mortality; however, this conclusion is questioned. Metformin should not be used in those with severe kidney or liver problems. A second oral agent of another class or insulin may be added if metformin is not sufficient after three months. Other classes of medications include: sulfonylureas, thiazolidinediones, dipeptidyl peptidase-4 inhibitors, SGLT2 inhibitors, and glucagon-like peptide-1 analogs. As of 2015 there was no significant difference between these agents. A 2018 review found that SGLT2 inhibitors and GLP-1 agonists, but not DPP-4 inhibitors, were associated with lower mortality than placebo or no treatment."}, {"id": "wiki20220301en140_46083", "title": "Pioglitazone/metformin", "score": 0.009900990099009901, "content": "Pioglitazone/metformin, sold under the brand name Actoplus Met among others, is a fixed-dose combination anti-diabetic medication used to improve glycemic control in adults with type 2 diabetes. It contains pioglitazone, a thiazolidinedione, and metformin, a biguanide. Mechanisms Pioglitazone is a member of the thiazolidinedione class, it decreases insulin resistance in the periphery and in the liver resulting in increased insulin dependent glucose disposal and decreased hepatic glucose output. Metformin is a member of the biguanide class, improves glucose tolerance in patients with type 2 diabetes, lowering both basal and postprandial plasma glucose. Metformin decreases hepatic glucose production, decreases intestinal absorption of glucose and improves insulin sensitivity by increasing peripheral glucose uptake and utilization."}, {"id": "pubmed23n0650_13044", "title": "[Therapeutic behavior to follow in the following clinical case: treatment of type 2 diabetes].", "score": 0.009900990099009901, "content": "A 62-year old woman with obesity, high blood pressure and type 2 diabetes mellitus (DM2) was referred to a Vascular Risk Unit of the Internal Medicine Department due to elevated HbA1C (8.1%) in spite of having taken metformin (850 mg/12h) and glipizide (10 mg/12 h) regularly. She tries to exercise daily (walking 30 min) and has lost weight (from 5 to 12 kg) several times, but always regains what she has lost. Furthermore, she monitors her glucose levels in fasting every two weeks and generally has between 120 and 160 mg/dL. Her high blood pressure is being treated with enalapril/HCTZ and she also takes aspirin 100mg/day and simvastatin 20 mg/day. It is seen in her family background that one brother died suddenly at 50 years of age. Her physical examination shows a BMI of 32.4 Kg/m(2), and she has no edemas in the lower limbs. Her BP is 154/82 mmHg and creatinine 0.9 mg/dL. She has no microalbuminuria and her liver function is normal. What treatment do you think would be the more appropriate? 1 - Add glitazones. 2 - Add incretin mimetics (GLP 1/ DPP-4). 3 - Slow acting insulin."}, {"id": "pubmed23n1015_2842", "title": "[Short-term intensive combined therapy with metformin, sagliptin and dapagliflozin for newly diagnosed type 2 diabetes: efficacy, weight control and safety].", "score": 0.00980392156862745, "content": "To assess the efficacy and safety of short- term intensive hypoglycemic therapy with a triple regimen consisting of metformin, sagliptin and dapagliflozin in patients with newly diagnosed type 2 diabetes mellitus with hemoglobin Alc (HbA1c) of 9%-12%. We prospectively enrolled 58 patients with newly diagnosed type 2 diabetes, who were treated with metformin combined with sagliptin and dapagliflozin for 12 weeks on the basis of diabetic diet and regular exercise. Blood glucose was monitored during the treatment and the changes in HbA1c, fasting blood glucose (FBG), 2-hour postprandial blood glucose (2 hPBG), fasting insulin (FINS), 2-hour postprandial insulin (2 hPINS), fasting C-peptide (F-CP), 2-hour postprandial C-peptide (2 hP-CP), and body weight after treatment as well as the incidence of hypoglycemia and adverse events associated with the treatment were recorded. Two patients withdrew from the study for intolerance of gastrointestinal reactions, and another 2 withdrew for inconvenience of access to the medicines. Fifty-four of the patients finally completed the study, including 34 male and 20 female patients. After 12 weeks of therapy, all the patients showed significant improvements in FBG, 2 hPBG, HbA1c, HOMA-beta and HOMA-IR (<iP</i &lt; 0.001) with a mean reduction of HbA1c level by (4.19 ± 1.07)%, and the goal of HbA1c control to below 7.0% was achieved in 83.33% of the patients. The reduction of HbA1c was correlated with FBG (<ir</i=0.487, <iP</i=0.000), 2 hPBG (<ir</i=0.310, <iP</i=0.023), and HOMA-β (<ir</i=-0.398, <iP</i=0.003). The patients had a mean body weight loss by 2.47±3.38 kg (<iP</i &lt; 0.001) and a mean decrease of body mass index (BMI) by 0.90± 1.18 kg/m2 (<iP</i &lt; 0.001) after the therapy. The body weight-reducing effect was associated with the patients' baseline body weight (<ir</i=0.678, <iP</i=0.000), BMI (<ir</i=0.818, <iP</i=0.000), F-CP (<ir</i=0.282, <iP</i=0.039) and HOMA-IR (<ir</i=0.297, <iP</i=0.029). During the therapy 8 patients experienced hypoglycemic symptoms (10 times, 14.81%); 3 patients were diagnosed with hypoglycemia (blood glucose ≤3.9 mmol/L, 3 times), and the overall incidence of hypoglycemia was 5.56%. No serious hypoglycemia or infections of the urinary and reproductive systems occurred in these patients. Short-term intensive oral hypoglycemic therapy with metformin combined with sagliptin and dapagliflozin is effective for treatment of patients with newly diagnosed type 2 diabetes with HbA1c of 9%-12% and shows a good weight-reducing effect with a low risk of hypoglycemia. The combined therapy can effectively improve β-cell insulin secretion function, and is suitable for treatment of newly diagnosed type 2 diabetic patients with high blood glucose."}, {"id": "wiki20220301en012_87405", "title": "Diabetes medication", "score": 0.009615384615384616, "content": "Multiple retrospective studies have resulted in a concern about rosiglitazone's safety, although it is established that the group, as a whole, has beneficial effects on diabetes. The greatest concern is an increase in the number of severe cardiac events in patients taking it. The ADOPT study showed that initial therapy with drugs of this type may prevent the progression of disease, as did the DREAM trial. The American Association of Clinical Endocrinologists (AACE), which provides clinical practice guidelines for management of diabetes, retains thiazolidinediones as recommended first, second, or third line agents for type 2 diabetes mellitus, as of their 2019 executive summary, over sulfonylureas and α-glucosidase inhibitors. However, they are less preferred than GLP-1 agonists or SGLT2 inhibitors, especially in patients with cardiovascular disease (which liraglutide, empagliflozin, and canagliflozin are all FDA approved to treat)."}, {"id": "pubmed23n0550_4155", "title": "Glycaemic control without weight gain in insulin requiring type 2 diabetes: 1-year results of the GAME regimen.", "score": 0.009615384615384616, "content": "Weight gain appears to be unavoidable in patients with type 2 diabetes who are switched from oral agents to insulin therapy. Peripheral hyperinsulinism induced by the use of long-acting insulin may be the key to explain this adverse effect. The aim of this study was to investigate whether a regimen free of long-acting insulin can provide long-term glycaemic control without causing weight gain. This is an uncontrolled, 1-year study comprising 58 patients with type 2 diabetes and secondary failure, age 30-75 years, BMI 25-35 kg/m(2), HbA1c &gt; 7.5% and fasting C-peptide level &gt; 0.3 mmol/l. All patients were treated with the GAME regimen, a combination of glimepiride administered at 20:00 hours for nocturnal glycaemic control, insulin aspart three times daily for meal-related glucose control and metformin. Seventy-one per cent of the patients were considered evaluable. HbA1c decreased from 10.0 +/- 0.3 to 7.4 +/- 0.1% (p &lt; 0.001). Fifty-nine per cent reached HbA1c levels &lt;or= 7.5%. Symptomatic nocturnal hypoglycaemia was not reported. Body weight tended to decrease during the first 3 months (-1.0 +/- 0.5 kg, p = 0.06), but then gradually rose to a value 0.8 +/- 0.5 kg higher than at baseline (p = 0.12). This is 4.4 +/- 0.6 kg less than predicted for conventional regimens employing long-acting insulin (p &lt; 0.001). The GAME regimen provides long-term glycaemic control as well as stabilization of body weight in about 60% of type 2 patients presenting with secondary failure."}, {"id": "wiki20220301en012_87419", "title": "Diabetes medication", "score": 0.00958488171848092, "content": "SGLT-2 inhibitors block the re-uptake of glucose in the renal tubules, promoting loss of glucose in the urine. This causes both mild weight loss, and a mild reduction in blood sugar levels with little risk of hypoglycemia. Oral preparations may be available alone or in combination with other agents. Along with GLP-1 agonists, they are considered preferred second or third agents for type 2 diabetics sub-optimally controlled with metformin alone, according to most recent clinical practice guidelines. Because they are taken by mouth, rather than injected (like GLP-1 agonists), patients who are injection-averse may prefer these agents over the former. They may be considered first line in diabetic patients with cardiovascular disease, especially heart failure, as these medications have been shown to reduce the risk of hospitalization in patients with such comorbidities. Because they are not available as generic medications, however, cost may limit their feasibility for many patients."}, {"id": "pubmed23n0834_5369", "title": "Efficacy and safety of antihyperglycaemic drug regimens added to metformin and sulphonylurea therapy in Type 2 diabetes: a network meta-analysis.", "score": 0.009523809523809525, "content": "To assess the efficacy and safety of third-line adjuvant antihyperglycaemic agents in people with Type 2 diabetes mellitus failing metformin and sulphonylurea combination therapy. We searched MEDLINE, CENTRAL, clinicaltrials.gov and regulatory websites, and conducted a manual search of references in the identified studies. Randomized trials evaluating antihyperglycaemic agents in adults with Type 2 diabetes experiencing poor glycaemic control despite optimized metformin and sulphonylurea therapy (≥ 1500 mg metformin or maximum tolerated dose; ≥ 50% of maximum sulphonylurea dose for ≥ 3 weeks) were included. Data extraction included: study characteristics; change in HbA1c concentration; weight; systolic blood pressure; and relative risk of hypoglycaemia, urinary tract infections; and genital tract infections. A network meta-analysis was performed. A total of 20 trials evaluating 13 antihyperglycaemic agents were included. Compared with placebo/control, all antihyperglycaemic agents reduced HbA1c levels, albeit by differing magnitudes [range 7 mmol/mol (0.6%) for acarbose to 13 mmol/mol (1.20%) for liraglutide]. Sodium glucose cotransporter-2 inhibitors reduced weight (1.43-2.07 kg) whereas thiazolidinediones, glargine and sitagliptin caused weight gain (1.48-3.62 kg) compared with placebo/control. Sodium glucose cotransporter-2 inhibitors, rosiglitazone and liraglutide decreased systolic blood pressure compared with placebo/control, pioglitazone, glargine and sitagliptin (2.41-8.88 mm Hg). Glargine, thiazolidinediones, liraglutide, sitagliptin and canagliflozin increased hypoglycaemia risk compared with placebo/control (relative risk 1.92-7.47), while glargine and rosiglitazone increased hypoglycaemia compared with most antihyperglycaemic agents (relative risk 2.81-7.47). No antihyperglycaemic agent increased the risk of urinary tract infection, but canagliflozin increased the risk of genital tract infection by 3.9-fold compared with placebo/control. When added to metformin and a sulphonylurea, antihyperglycaemic agents had varying effects on efficacy and safety endpoints. These conclusions should be considered when clinicians choose between possible adjunctive agents."}, {"id": "pubmed23n0827_20698", "title": "Case Study: Weight loss in a patient with type 2 diabetes: Challenges of diabetes management.", "score": 0.009523809523809525, "content": "This patient with BMI 36 kg/m² and T2DM on insulin glargine and glyburide as well as atenolol for HTN was able to lose 10% of his initial body weight with a low-carbohydrate diet and exercise and adjustment of medications in approximately a 36-week time frame. Insulin glargine and glyburide were reduced gradually with blood glucose monitoring and replaced by an increase in metformin, start of liraglutide, and eventually phentermine/topiramate and canagliflozin (Figure). Therefore, medications that can exacerbate weight gain were discontinued in place of medications which promote weight loss."}, {"id": "wiki20220301en455_20871", "title": "Discovery and development of gliflozins", "score": 0.009433962264150943, "content": "Activity of SGLT-2 inhibitors in glycemic control Michael Nauck recounts that meta-analyses of studies about the activity of SGLT-2 inhibitors in glycemic control in type 2 diabetes mellitus patients shows improvement in the control of glucose, when compared with placebos, metformin, sulfonylurea, thiazolidinediones, insulin and more. The HbA1c was examined after SGLT-2 inhibitors were given alone (as monotherapy) and as an add-on therapy to the other diabetes medicines. The SGLT-2 inhibitors that were used were dapagliflozin and canagliflozin and others in the same drug class. The meta-analysis was taken together from studies ranging from period of few weeks up to more than 100 weeks."}, {"id": "pubmed23n0854_10174", "title": "Liraglutide and obesity in elderly: efficacy in fat loss and safety in order to prevent sarcopenia. A perspective case series study.", "score": 0.009433962264150943, "content": "For the growing numbers of obese elderly with diabetes, the glucagon-like peptide-1 (GLP-1) receptor analogue (liraglutide) appears a safe way to promote and maintain substantial weight loss. Given this background, the aim of this study was to assess the effect of the liraglutide treatment, at doses up to 3.0 mg per day, on the body composition, focusing on sarcopenia, in overweight and obese elderly with type 2 diabetes mellitus (T2DM). A perspective study was carried out in overweight and obese T2DM patients with HbA1c equal to 7.0 % (53 mmol/mol) ~10.0 % (86), under 3-month treatment (at least) of maximal dose of metformin at stable regime, and additional liraglutide at doses up to 3.0 mg per day. Body composition markers such as skeletal muscle index (SMI), android and gynoid fat mass, and arms and legs fat free mass, was measured by dual-energy X-ray densitometry (DXA) at baseline and after 24 weeks of liraglutide treatment. Glucose control was also carried out by glucose and HbA1c. Nine subjects (male/female 6/3, mean age 68.22 ± 3.86 years, BMI 32.34 ± 4.89 kg/m<sup2</sup) were evaluated. We noted a median decrease in BMI (-0.78 kg/m<sup2</sup), weight (-2000 g), fat mass (-1498 g) and android fat (-0.9 %), and a increase in SMI (+0.03 kg/m<sup2</sup) from baseline. Glycemic control also improved, with a median change HbA1c of -0.80 %. Twenty-four weeks of liraglutide treatment was associated with reductions in fat mass and android fat. In addition, in order to prevent sarcopenia, it preserved the muscular tropism."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 247]], "word_ranges": [[0, 38]], "text": "This is a typical case of cervical incompetence (and this patient has conization as a risk factor). This pathology consists of dilatation of the cervix in the absence of contractions, requiring cerclage to prevent miscarriage or immature delivery."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "This is a typical case of cervical incompetence (and this patient has conization as a risk factor). This pathology consists of dilatation of the cervix in the absence of contractions, requiring cerclage to prevent miscarriage or immature delivery. The cerclage is ideally performed electively, programmed, and not when the patient arrives at the ER \"in extemis\" (which are also done, but with a lower success rate... the ideal is to do them cold).", "full_answer_no_ref": "This is a typical case of cervical incompetence (and this patient has conization as a risk factor). This pathology consists of dilatation of the cervix in the absence of contractions, requiring cerclage to prevent miscarriage or immature delivery. The cerclage is ideally performed electively, programmed, and not when the patient arrives at the ER \"in extemis\" (which are also done, but with a lower success rate... the ideal is to do them cold).", "full_question": "A 32-year-old woman requests preconception counseling. The patient reports that she underwent cervical conization for a high-grade intraepithelial lesion (H-SIL) and subsequently had three miscarriages between 20 and 22 weeks gestation. She has no living children. On all three occasions she came to the emergency department with a feeling of weight in the hypogastrium, where she was found to be 8 cm dilated and with prominent amniotic membranes. She had never felt contractions before, what advice would you give her for the next pregnancy?", "id": 209, "lang": "en", "options": {"1": "I would prescribe oral atosiban prophylaxis throughout the pregnancy.", "2": "I would offer lung maturation with corticosteroids from 19-20 weeks of gestation.", "3": "I would recommend a cervical cerclage at 14 weeks gestation.", "4": "I would advise her not to attempt any more pregnancies because of the high risk of recurrence.", "5": "I would recommend resorting to assisted reproductive techniques."}, "question_id_specific": 185, "type": "GYNECOLOGY AND OBSTETRICS", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0892_20023", "title": "Prevention of spontaneous preterm birth: Guidelines for clinical practice from the French College of Gynaecologists and Obstetricians (CNGOF).", "score": 0.017170228445099484, "content": "In France, 60,000 neonates are born preterm every year (7.4%), half of them after the spontaneous onset of labor. Among preventable risk factors of spontaneous prematurity, only cessation of smoking is associated with decreased prematurity (level of evidence [LE]1). It is therefore recommended (Grade A). Routine screening and treatment of vaginal bacteriosis is not recommended in the general population (Grade A). The only population for which vaginal progesterone is recommended is that comprising asymptomatic women with singleton pregnancies, no history of preterm delivery, and a short cervix at 16-24 weeks of gestation (Grade B). A history-indicated cerclage is not recommended for women with only a history of conization (Grade C), uterine malformation (professional consensus), isolated history of preterm delivery (Grade B), or twin pregnancies for primary (Grade B) or secondary (Grade C) prevention of preterm birth. A history-indicated cerclage is recommended for a singleton pregnancy with a history of at least 3 late miscarriages or preterm deliveries (Grade A). Ultrasound cervical length screening is recommended between 16 and 22 weeks for women with a singleton previously delivered before 34 weeks gestation, so that cerclage can be offered if cervical length &lt;25mm before 24 weeks (Grade C). A cervical pessary is not recommended for the prevention of preterm birth in a general population of asymptomatic women with twin pregnancies (Grade A) or in populations of asymptomatic women with a short cervix (professional consensus). Although the implementation of universal screening by transvaginal ultrasound for cervical length at 18-24 weeks of gestation in women with a singleton gestation and no history of preterm birth can be considered by individual practitioners, this screening cannot be universally recommended. In cases of preterm labor, (i) it is not possible to recommend any one of the several methods (ultrasound of the cervical length, vaginal examination, or fetal fibronectin assay) over any other to predict preterm birth (Grade B); (ii) routine antibiotic therapy is not recommended (Grade A); (iii) prolonged hospitalization (Grade B) and bed rest (Grade C) are not recommended. Compared with placebo, tocolytics are not associated with a reduction in neonatal mortality or morbidity (LE2) and maternal severe adverse effects may occur with all tocolytics (LE4). Atosiban and nifedipine (Grade B), unlike beta-agonists (Grade C), can be used for tocolysis in spontaneous preterm labor without preterm premature rupture of membranes. Maintenance tocolysis is not recommended (Grade B). Antenatal corticosteroid administration is recommended for all women at risk of preterm delivery before 34 weeks of gestation (Grade A). After 34 weeks, the evidence is insufficiently consistent to justify recommending systematic antenatal corticosteroid treatment (Grade B), but a course of this treatment might be indicated in clinical situations associated with high risk of severe respiratory distress syndrome, mainly in case of planned cesarean delivery (Grade C). Repeated courses of antenatal corticosteroids are not recommended (Grade A). Rescue courses are not recommended (Professional consensus). Magnesium sulfate administration is recommended for women at high risk of imminent preterm birth before 32 weeks (Grade A). Cesareans are not recommended for fetuses in vertex presentation (professional consensus). Both planned vaginal and elective cesarean delivery are possible for breech presentations (professional consensus). Delayed cord clamping may be considered if the neonatal or maternal state allows (professional consensus)."}, {"id": "pubmed23n0887_3084", "title": "[Prevention of spontaneous preterm birth (excluding preterm premature rupture of membranes): Guidelines for clinical practice - Text of the Guidelines (short text)].", "score": 0.014140562813129184, "content": "To determine the measures to prevent spontaneous preterm birth (excluding preterm premature rupture of membranes)and its consequences. The PubMed database, the Cochrane Library and the recommendations from the French and foreign obstetrical societies or colleges have been consulted. In France, premature birth concerns 60,000 neonates every year (7.4 %), half of them are delivered after spontaneous onset of labor. Among preventable risk factors of spontaneous prematurity, only cessation of smoking is associated to a decrease of prematurity (level of evidence [LE] 1). This is therefore recommended (grade A). Routine screening and treatment of vaginal bacteriosis in general population is not recommended (grade A). Asymptomatic women with single pregnancy without history of preterm delivery and a short cervix between 16 and 24 weeks is the only population in which vaginal progesterone is recommended (grade B). A history-indicated cerclage is not recommended in case of only past history of conisation (grade C), uterine malformation (Professional consensus), isolated history of pretem delivery (grade B) or twin pregnancies in primary (grade B) or secondary (grade C) prevention of preterm birth. A history-indicated cerclage is recommended for single pregnancy with a history of at least 3 late miscarriages or preterm deliveries (grade A).). In case of past history of a single pregnancy delivery before 34 weeks gestation (WG), ultrasound cervical length screening is recommended between 16 and 22 WG in order to propose a cerclage in case of length&lt;25mm before 24 WG (grade C). Cervical pessary is not recommended for the prevention of preterm birth in a general population of asymptomatic women with a twin pregnancy (grade A) and in populations of asymptomatic women with a short cervix (Professional consensus). Although the implementation of a universal transvaginal cervical length screening at 18-24 weeks of gestation in women with a singleton gestation and no history of preterm birth can be considered by individual practitioners, this screening cannot be universally recommended. In case of preterm labor, (i) it is not possible to recommend one of the methods over another (ultrasound of the cervical length, vaginal examination, fetal fibronectin) to predict preterm birth (grade B); (ii) routine antibiotic therapy is not recommended (grade A); (iii) prolonged hospitalization (grade B) and bed rest (grade C) is not recommended. Compared with placebo, tocolytics are not associated with a reduction in neonatal mortality or morbidity (LE2) and maternal severe adverse effects may occur with all tocolytics (LE4). Atosiban and nifedipine (grade B), contrary to betamimetics (grade C), can be used for tocolysis in spontaneous preterm labour without preterm premature rupture of membranes. Maintenance tocolysis is not recomended (grade B). Antenatal corticosteroid administration is recommended to every woman at risk of preterm delivery before 34 weeks of gestation (grade A). After 34 weeks, evidences are not consistent enough to recommend systematic antenatal corticosteroid treatment (grade B), however, a course might be indicated in the clinical situations associated with the higher risk of severe respiratory distress syndrome, mainly in case of planned cesarean delivery (grade C). Repeated courses of antenatal corticosteroids are not recommended (grade A). Rescue courses are not recommended (Professional consensus). Magnesium sulfate administration is recommended to women at high risk of imminent preterm birth before 32WG (grade A). Cesarean is not recommended in case of vertex presentation (Professional consensus). Both planned vaginal or elective cesarean delivery is possible in case of breech presentation (Professional consensus). A delayed cord clamping may be considered if the neonatal or maternal state so permits (Professional consensus). Except for antenatal corticosteroid and magnesium sulfate administration, diagnostic tools or prenatal pharmacological treatments implemented since 30 years to prevent preterm birth and its consequences have not matched expectations of caregivers and families."}, {"id": "Obstentrics_Williams_2361", "title": "Obstentrics_Williams", "score": 0.012411440566780372, "content": "Afecting approximately 1 percent of fertile couples, recurrent pregnancy loss (RPL) is classically deined as three or more consecutive pregnancy losses <20 weeks' gestation or with a fetal weight < 500 g. Mindful of this threshold, data from two large studies showed the risk for a subsequent miscarriage to be similar whether following two or three prior pregnancy losses (Bhattacharya, 2010; Brigham, 1999). And, the American Society for Reproductive Medicine (2013) now deines RPL as two or more failed pregnancies conirmed by sonographic or histopathological examination. Primary PL refers to multiple losses in a woman who has never delivered a liveborn, and secondary PL refers to multiple pregnancy losses in a patient with a prior live birth. Remarkably, the chances for a successful pregnancy arei> 50 percent even after five losses (Table 18-4). TABLE 18-4. Predicted Success Rate of Subsequent Pregnancy According to Age and Number of Previous Miscarriages"}, {"id": "pubmed23n0960_18312", "title": "Early Accreta and Uterine Rupture in the Second Trimester.", "score": 0.010648417269988903, "content": "The differential diagnosis of third trimester bleeding can range from placenta abruptia to placenta previa to uterine rupture and the placenta accreta spectrum (PAS). However, patients with risk factors such as multiple cesarean sections (c-sections), advanced maternal age (AMA), grand multiparity, and single-layer uterine closure are at greater risk of developing these complications earlier than we would traditionally expect. This case recounts a 38-year-old gravida 6 preterm 3 term 1 abortus 1 live 4 (G6P3114) at 23 weeks and five days gestational age (GA) with a past medical history of preterm pregnancy, pre-eclampsia, chronic abruptia, three previous c-sections, and low-lying placenta who presented to the emergency department (ED) with vaginal bleeding. Initial workup revealed placenta accreta and possible percreta. The patient was placed on intramuscular (IM) corticosteroids in anticipation of preterm delivery. As soon as the patient was stable, she was discharged home. She presented to a different hospital the next day with the same complaints. Imaging was consistent with accreta and her presentation with abruption. During the hospital stay, the patient went into threatened preterm labor (PTL). At first, we suspected preterm premature rupture of membranes (PPROM) due to apparent pooling of amniotic fluid in the vaginal canal. Upon further work up, the diagnosis was consistent with chronic abruption oligohydramnios sequence (CAOS). Before this could be investigated, her hospital course was complicated by acute abruption and Category III/nonreassuring fetal heart rate (FHR) tracing. The patient underwent an emergency c-section at 26 weeks GA as well as a planned supracervical hysterectomy for desired permanent sterilization. During the operation, the patient suffered a postpartum hemorrhage (PPH) of 4500 mL. She was later discharged home on postoperative day (POD) eight."}, {"id": "pubmed23n0511_555", "title": "Standard term of pregnancy.", "score": 0.009900990099009901, "content": "What is it? This question arose early this year during a discussion with my doula partner, who lived in Great Britain for 20 years, and one of the midwives I work with, who attends only homebirths, which is very rare in France. The mom whose case we were discussing was late going into labor but not postdated according to the official pregnancy term here in France (37-42 WA--\"weeks of amenorrhea\"). The midwife expressed her discomfort with waiting. My doula partner and I felt differently, but we knew we were influenced by American and British midwives' practices. I had been shocked the previous December by the position of the chief of the maternity department in a private hospital (a small unit with no residents, in which nurse-midwives attend \"normal\" births and obstetricians are called in case of complications only). This OB explained to my client and me that if she didn't go into labor naturally, she would be called at 41 + 1 for a vaginal exam to check her cervix and would be induced at 41 + 2. Waiting until 42 weeks requires daily checks, for which he has neither the room nor personnel. He clearly stated that it was a matter of management of time and finances. A month later one of our clients reported the story of her brother and sister-in-law's planned homebirth in London. Their doctors had put a lot of pressure on the mother during her pregnancy with gestational diabetes regarding her length of term. They started to talk about induction. The parents didn't feel comfortable with this, and at that point our client had asked me to refer them to someone who could help them there. We referred them to the National Childbirth Trust and to the sweetest doula we know there. This doula (Liliana Lammers) and her famous doctor partner (Dr. Michel Odent) were a good match. The doctor advised waiting, on the condition that the health of the baby and the amount of fluid be checked daily at the local hospital. The mother had already been waiting several weeks past what was supposed to be her term. Finally, she went naturally into labor at home. The doctor and the doula came and, after some hours of observation, decided it would be wiser for the mother to deliver in the hospital. The doctor and doula were not comfortable with the prolonged prelabor, when, at nearly 44 weeks, the health of the baby and the amount of fluid had not been checked for five days. The mother finally had a vaginal birth without drugs at the hospital. After hearing this story, I suggested it would be interesting to collect the official lengths of term and the different routines in other countries as a learning tool and in order to give us something other than French protocol on which to base our practice. So I sent the question to every midwife for whom I had an e-mail address from the last Midwifery Today conference in Paris. Beyond this motivation was my own curiosity regarding the relationship between the official term in each country and its uses and routines. The most significant (because the most unique) answer, in my opinion, is from The Netherlands, where physiology is a priority."}, {"id": "pubmed23n0536_12712", "title": "[What should you tell a patient with a history of cervical incompetence in the first trimester?].", "score": 0.009900990099009901, "content": "Data is now more than ever available to inform couples at risk of second trimester miscarriage or preterm delivery. We are able to give customized information according to the obstetrical history and to the evolution of the cervix during the second trimester although the level of scientific evidence is limited or poor. Elective cerclage can be proposed to patients with a history of at least 3 second trimester miscarriages or preterm deliveries. There is no clear consensus on which patients could benefit from therapeutic cerclage. Indications would have to be motivated by a short cervix on ultrasound measurements and the cerclage performed before 24 weeks of gestation."}, {"id": "pubmed23n0689_15606", "title": "First case of vaginal radical trachelectomy in a pregnant Japanese woman.", "score": 0.00980392156862745, "content": "A diagnosis of cervical cancer during pregnancy poses difficult management and ethical problems. Survival of the patient is the foremost concern, but fetal viability and well-being must also be addressed. Radical trachelectomy (RT) has recently begun to be performed as a possible treatment modality for early stage invasive uterine cervical cancer in pregnant patients who would like to continue their pregnancy. A 32-year-old Japanese woman visited a local hospital for prenatal care, and was diagnosed with a FIGO I B1 adenocarcinoma of the uterine cervix. She had a strong desire to avoid pregnancy termination, so she was admitted to our hospital for fertility-preserving surgery. After extensive counseling, vaginal radical trachelectomy with abdominal pelvic lymphadenectomy was performed in the 16th gestational week. The excised uterine cervix and lymph nodes were pathologically negative for cancer. To maintain her pregnancy, daily vaginal disinfection with povidone iodine, bed rest, and administration of ritodrine and an ulinastatin vaginal suppository were continued until the delivery. At 34 weeks' gestation, an emergency cesarean section was performed because of sudden premature rupture of the membranes. A baby girl was born weighing 2112 g, with Apgar score of 8/9. The mother remains without evidence of recurrence at the time of this report. This is the first case of successful pregnancy and delivery in Japan after vaginal RT."}, {"id": "Obstentrics_Williams_5684", "title": "Obstentrics_Williams", "score": 0.00980392156862745, "content": "Prevention of preterm birth remains an elusive goal. Still, may be achievable. Of options, cerclage placement may be used to prevent pre term birth in at least three circumstances. First, the procedure may beneit women who have a history of recurrent midtrimester losses and who are diagnosed with cervical insuiciency. A second instance is the woman identiied during sonographic examination to have a short cervix. The third indication is a \"rescue\" cerclage, done emergently when cervical incompetence is recognized in women with threatened preterm labor."}, {"id": "pubmed23n1097_18310", "title": "Successful cervical cerclage in a dichorionic diamniotic twin pregnancy: A case report.", "score": 0.009708737864077669, "content": "A 26-year-old primigravid woman presented with a dichorionic diamniotic twin pregnancy after 7 years of infertility. No formal ultrasound was performed until a morphology check at 19 weeks and 4 days of gestation, at which time a shortened cervix was identified. The patient was already on vaginal progesterone pessaries from conception, as per her infertility specialist, and was advised to change to a rectal route of administration. At 20 weeks and 5 days, progesterone pessaries were increased to twice daily. A repeat scan at 21 weeks and 4 days showed a funnelled cervix 29 mm in length, a closed portion of 4-6 mm and bulging membranes. A speculum examination at this time showed a shortened cervix, 5 mm open, with visible membranes. A cervical cerclage was placed at 21 weeks and 5 days. The patient was given oral antibiotics for 1 week and was continued on progesterone pessaries. The patient was managed through the twins clinic and had serial ultrasound scans throughout the pregnancy. She went on to develop gestational diabetes and pre-eclampsia. She had a caesarean section at 33 weeks and 4 days due to pre-eclampsia, with abnormal doppler scans. Cervical cerclage was removed at the time of the caesarean section. Both twins were admitted to the nursery for prematurity and progressed well. This case report illustrates how a cervical cerclage can be utilised successfully in a primigravid dichorionic diamniotic twin pregnancy."}, {"id": "pubmed23n0800_14851", "title": "Breathlessness with pulmonary metastases: a multimodal approach.", "score": 0.009615384615384616, "content": "Case Study  Sarah is a 58-year-old breast cancer survivor, social worker, and health-care administrator at a long-term care facility. She lives with her husband and enjoys gardening and reading. She has two grown children and three grandchildren who live approximately 180 miles away. SECOND CANCER DIAGNOSIS  One morning while showering, Sarah detected a painless quarter-sized lump on her inner thigh. While she thought it was unusual, she felt it would probably go away. One month later, she felt the lump again; she thought that it had grown, so she scheduled a visit with her primary care physician. A CT scan revealed a 6.2-cm soft-tissue mass in the left groin. She was referred to an oncologic surgeon and underwent an excision of the groin mass. Pathology revealed a grade 3 malignant melanoma. She was later tested and found to have BRAF-negative status. Following her recovery from surgery, Sarah was further evaluated with an MRI scan of the brain, which was negative, and a PET scan, which revealed two nodules in the left lung. As Sarah had attended a cancer support group during her breast cancer treatment in the past, she decided to go back to the group when she learned of her melanoma diagnosis. While the treatment options for her lung lesions included interleukin-2, ipilimumab (Yervoy), temozolomide, dacarbazine, a clinical trial, or radiosurgery, Sarah's oncologist felt that ipilimumab or radiosurgery would be the best course of action. She shared with her support group that she was ambivalent about this decision, as she had experienced profound fatigue and nausea with chemotherapy during her past treatment for breast cancer. She eventually opted to undergo stereotactic radiosurgery. DISEASE RECURRENCE  After the radiosurgery, Sarah was followed every 2 months. She complained of shortness of breath about 2 weeks prior to each follow-up visit. Each time her chest x-ray was normal, and she eventually believed that her breathlessness was anxiety-related. Unfortunately, Sarah's 1-year follow-up exam revealed a 2 cm × 3 cm mass in her left lung, for which she had a surgical wedge resection. Her complaints of shortness of breath increased following the surgery and occurred most often with anxiety, heat, and gardening activities, especially when she needed to bend over. Sarah also complained of a burning \"pins and needles\" sensation at the surgical chest wall site that was bothersome and would wake her up at night. Sarah met with the nurse practitioner in the symptom management clinic to discuss her concerns. Upon physical examination, observable signs of breathlessness were lacking, and oxygen saturation remained stable at 94%, but Sarah rated her breathlessness as 7 on the 0 to 10 Borg scale. The nurse practitioner prescribed duloxetine to help manage the surgical site neuropathic pain and to assist with anxiety, which in turn could possibly improve Sarah's breathlessness. Several nonpharmacologic modalities for breathlessness were also recommended: using a fan directed toward her face, working in the garden in the early morning when the weather is cooler, gardening in containers that are at eye level to avoid the need to bend down, and performing relaxation exercises with pursed lip breathing to relieve anxiety-provoked breathlessness. One month later, Sarah reported relief of her anxiety; she stated that the fan directed toward her face helped most when she started to feel \"air hungry.\" She rated her breathlessness at 4/10 on the Borg scale. SECOND RECURRENCE: MULTIPLE PULMONARY NODULES  Sarah's chest x-rays remained clear for 6 months, but she developed a chronic cough shortly before the 9-month exam. An x-ray revealed several bilateral lung lesions and growth in the area of the previously resected lung nodule. Systemic therapy was recommended, and she underwent two cycles of ipilimumab. Sarah's cough and breathlessness worsened, she developed colitis, and she decided to stop therapy after the third cycle. In addition, her coughing spells triggered bronchospasms that resulted in severe anxiety, panic attacks, and air hunger. She rated her breathlessness at 10/10 on the Borg scale during these episodes. She found communication difficult due to the cough and began to isolate herself. She continued to attend the support group weekly but had difficulty participating in conversation due to her cough. Sarah was seen in the symptom management clinic every 2 weeks or more often as needed. No acute distress was present at the beginning of each visit, but when Sarah began to talk about her symptoms and fear of dying, her shortness of breath and anxiety increased. The symptom management nurse practitioner treated the suspected underlying cause of the breathlessness and prescribed oral lorazepam (0.5 to 1 mg every 6 hours) for anxiety and codeine cough syrup for the cough. Opioids were initiated for chest wall pain and to control the breathlessness. Controlled-release oxycodone was started at 10 mg every 12 hours with a breakthrough pain (BTP) dose of 5 mg every 2 hours as needed for breathlessness or pain. Sarah noted improvement in her symptoms and reported a Borg scale rating of 5/10. Oxygen therapy was attempted, but subjective improvement in Sarah's breathlessness was lacking. END OF LIFE  Sarah's disease progressed to the liver, and she began experiencing more notable signs of breathlessness: nasal flaring, tachycardia, and restlessness. Opioid doses were titrated over the course of 3 months to oxycodone (40 mg every 12 hours) with a BTP dose of 10 to 15 mg every 2 hours as needed, but her breathlessness caused significant distress, which she rated 8/10. The oxycodone was rotated to IV morphine continuous infusion with patient-controlled analgesia (PCA) that was delivered through her implantable port. This combination allowed Sarah to depress the PCA as needed and achieve immediate control of her dyspneic episodes. Oral lorazepam was also continued as needed. Sarah's daughter moved home to take care of her mother, and hospice became involved for end-of-life care. As Sarah became less responsive, nurses maintained doses of morphine for control of pain and breathlessness and used a respiratory distress observation scale to assess for breathlessness since Sarah could no longer self-report. A bolus PCA dose of morphine was administered by Sarah's daughter if her mother appeared to be in distress. Sarah died peacefully in her home without signs of distress. "}, {"id": "pubmed23n0568_8656", "title": "[Cervical cerclage and evidence-based medicine: if, how and when].", "score": 0.009615384615384616, "content": "Cervical cerclage has always been the main treatment option in cases of so-called cervical insufficiency, a condition that is notoriously associated with a high risk of second trimester abortion and/or preterm delivery. We can distinguish between a prophylactic cerclage, to be performed electively, usually at 13-16 weeks gestation, only when the woman has a history extremely suggestive for cervical incompetence (3 or more mid-trimester abortions or preterm deliveries) and a therapeutic cerclage. This last cerclage is recommended either for women who have ultrasonographic changes consistent with a short cervix or the presence of funneling after the 16-20 weeks gestation (urgent cerclage) and for women who present the asymptomatic dilation of the uterine cervix of at least 2 cm and/or a prolapse of the amniochorial membranes (emergent cerclage). So far there is still a lack of controlled and randomized trials that can unquestionably demonstrate the advantages of the cervical cerclage in comparison with a ''wait and see'' aptitude. The cerclage can be performed either transvaginally, usually according to the McDonald technique, or transabdominally. This last approach is recommended when a transvaginal cerclage has to be avoided because of technical difficulties depending on the conditions of the cervix or when the pregnant woman has a history of one or more failed transvaginal cerclages. Interesting perspectives are currently offered by the laparoscopic cerclage, a method that has been effective and unexpectedly safe till now."}, {"id": "pubmed23n1095_2800", "title": "Chorioamnionitis caused by <i>Serratia marcescens</i> in a healthcare worker: A case report.", "score": 0.009523809523809525, "content": "Healthcare workers (HCWs) are at an increased risk for exposure to infections. <iSerratia marcescens</i (<iS</i. <imarcescens</i) is a gram-negative, opportunistic and nosocomial pathogen belonging to the Enterobacterieae family. A few case reports have been published of chorioamnionitis caused by <iS. marcescens</i infection. Immunological changes during pregnancy can also affect the risk of infection. However, few studies have examined hospital-acquired bacterial infection in pregnant HCWs. A 33-year-old woman, a resident in anesthesiology, was admitted at 14 wk gestation for fever with chills. She had no medical history other than contact dermatitis of both hands that started from the beginning of the trainee. There was no obvious infection focus and no bacterial growth in blood cultures. She was discharged after 1 wk of empirical antibiotic treatment. At three weeks before the fever started, she had a blister on the site of contact dermatitis on both hands, she applied antibiotic ointment for three days and the blisters had healed. At 19 wk gestation, she had a high fever and was readmitted. Physical examination and image studies were nonspecific and the patient had no other symptoms. <iS. marcescens</i grew in blood cultures at 19 wk gestation. Treatment with intravenous antibiotics was started. However, she suffered a miscarriage at 22<sup4/7</sup wk gestation. Pathologically, the amniotic membrane showed chorioamnionitis with a focal infarct. Subsequently, a placenta tissue culture grew <iS. marcescens</i. HCWs can be exposed to pathogens that can cause opportunistic infections such as <iS. marcescens</i. Pregnancy affects the immune system, making it susceptible to opportunistic infections. Therefore, pregnant HCWs may require more preventive measures, including hand hygiene and avoid risk factors (ex. wrapping the skin)."}, {"id": "pubmed23n0935_16390", "title": "Elective abortion: Clinical practice guidelines from the French College of Gynecologists and Obstetricians (CNGOF).", "score": 0.009523809523809525, "content": "The number of elective abortions has been stable for several decades. Many factors explain women's choice of abortion in cases of unplanned pregnancies. Early initiation of contraceptive use and a choice of contraceptive choices appropriate to the woman's life are associated with lower rates of unplanned pregnancies. Reversible long-acting contraceptives should be favored as first-line methods for adolescents because of their effectiveness (grade C). Ultrasound scan before an elective abortion must be encouraged but should not be obligatory (professional consensus). As soon as the embryo appears on the ultrasound scan, the date of pregnancy is estimated by measuring the crown-rump length (CRL) or, from 11 weeks on, by measuring the biparietal diameter (BPD) (grade A). Because reliability of these parameters is ±5 days, the abortion may be done if measurements are respectively less than 90 mm for CRL and less than 30 mm for BPD (professional consensus). A medically induced abortion, performed with a dose of 200 mg mifepristone combined with misoprostol, is effective at any gestational age (Level of Evidence (LE) 1). Before 7 weeks, mifepristone should be followed 24-48 h later by misoprostol, administered orally, buccally, sublingually, or even vaginally followed if needed by a further dose of 400 μg after 3 h, to be renewed if needed after 3 h (LE 1, grade A). After 7 weeks, administration of misoprostol by the vaginal, sublingual, or buccal routes is more effective and better tolerated than by the oral route (LE 1). Cervical preparation is recommended for systematic use in surgical abortions (professional consensus). Misoprostol is a first-line agent for cervical preparation at a dose of 400 μg (grade A). Vacuum aspiration is preferable to curettage (grade B). A uterus perforated during surgical aspiration should not routinely be considered to be scarred (professional consensus). An elective abortion is not associated with a higher risk of subsequent infertility or ectopic pregnancy (LE 2). The medical consultation before an elective abortion generally does not affect the decision to end or continue the pregnancy, and most women are sufficiently certain about their choice at this time. Women appear to find the method used most acceptable and to be most satisfied when they were able to choose the method (grade B). Elective abortions are not associated with an increased rate of psychiatric disorders (LE 2). However, women with psychiatric histories are at a higher risk of psychological disorders after the occurrence of an unplanned pregnancy than women with such a history (LE 2). For surgical abortions, combined hormonal contraceptives - oral or transdermal - should be started on the day of the abortion, while the vaginal ring should be inserted 5 days afterwards (grade B). For medical abortions, the vaginal ring should be inserted in the week after mifepristone administration, while the combined contraceptives should begin the same day as the misoprostol or the day after (grade C). Contraceptive implants should be inserted on the same day as a surgical abortion, and may be inserted the day the mifepristone is administered for medical abortions (grade B and C respectively). In case of medical abortion, the implant can be inserted the same day the mifepristone is administered (grade C). Both the copper IUDs and levonorgestrel intrauterine system should be inserted on the day of the surgical abortion (grade A). After medical abortions, an IUD can be inserted in 10 days after mifepristone administration, after ultrasound scan verification of the absence of an intrauterine pregnancy (grade C)."}, {"id": "pubmed23n1061_5028", "title": "Mode of Delivery in the Setting of Repeated Vitreous Hemorrhages in Proliferative Diabetic Retinopathy: A Case Report and Review of the Literature.", "score": 0.009433962264150943, "content": "The state of pregnancy affects all organ systems including the eyes. Progression of diabetic retinopathy (DR) is a known association. In proliferative DR, there is an increased risk of vitreous hemorrhage (VH) during spontaneous vaginal delivery (SVD) due to the Valsalva maneuver. A 30-year-old female with poorly controlled type I diabetes and hypothyroidism on treatment was following up with the antenatal services at our hospital. This was her second pregnancy having had a previous miscarriage. Three months into her pregnancy, our Ophthalmology service was consulted to assess her and give our advice regarding the safest mode of delivery for her. Questioning revealed that she was following regularly elsewhere for proliferate DR with previous interventions and history of multiple and repeated VHs. When she was seen in our Ophthalmology clinic, she was anxious about the mode of delivery that was best suited for her with regard to her ocular condition. On examination, her visual acuity (VA) without correction was 20/40 in both eyes, improving to 20/20 in the right eye and 20/30 in the left eye after refraction. Her intra-ocular pressure was normal. A dilated fundus examination (DFE) showed changes of high-risk proliferative DR in both eyes and a VH in the right eye. Subsequent follow-up did not reveal any new complaints or concerns. She required one session of pan-retinal photocoagulation (PRP) in her first-trimester visit. DFE showed improvement in VH when compared to her initial examination. After discussing her condition with her obstetrician, it was decided to offer the patient a cesarean section (C/S) delivery, as her risk of developing VH during SVD was greater than normal. At 38 weeks of gestation, she delivered a healthy boy following an uneventful elective C/S. There were no visual complaints throughout her admission for the procedure or thereafter. During the reproductive age, DR is a leading cause of decreased vision. Pregnancy is an independent risk factor for progression of DR, with the stage of DR prior to conception being another. If not managed well, proliferative DR can result in VH, with the risk also existing in relation to SVD due to recurrent Valsalva maneuvers during labor. Our patient who initially presented with proliferative DR in both eyes and a VH in the right eye received one session of PRP to both eyes in the first trimester and was closely followed up throughout her pregnancy thereafter. When her due date neared, it was decided that the safest and most suitable mode of delivery was an elective C/S due to her increased risk of VH related to Valsalva maneuvers during SVD, especially since this was to be her first delivery."}, {"id": "pubmed23n0891_20224", "title": "[Prevention of preterm birth by uterine cervical cerclage].", "score": 0.009433962264150943, "content": "To review the scientific literature on cervical insufficiency and indications of cervical cerclage cervix. The PubMed database, the Cochrane Library and the recommendations from the French and international obstetrical societies between 1972 and June 2016 have been consulted. Cervical insufficiency is a pathophysiological concept and to date no consensual definition is available: the diagnosis is clinical and discussed retrospectively in case of patients with a history of late miscarriages and/or spontaneous preterm delivery, with asymptomatic dilatation of the cervix (professional consensus). The risk of preterm birth is higher in case of surgical cold-knife conisation as compared to loop electrosurgical excision (LE3) and laser vaporization has a negligible impact (LE3). In patients with a history of late pregnancy loss or preterm birth, investigations for the diagnosis of uterine malformation are recommended (grade C). No investigation is recommended for the diagnosis of a cervical insufficiency (professional consensus). A history-indicated cerclage is not recommended in case of only past history of conisation (grade C), uterine malformation (professional consensus), isolated history of preterm delivery (grade B) or twin pregnancies in primary (grade B) or secondary (grade C) prevention of preterm birth. A history-indicated cerclage is recommended for single pregnancy with a history of at least three late miscarriages or preterm deliveries (grade A). In case of history of one or two late miscarriages or preterm deliveries, there are not sufficient arguments to recommend a history-indicated cerclage (professional consensus). Further studies are needed. The ultrasound-indicated cerclage is not recommended in case of short cervical length during the 2nd trimester of single pregnancy without past history of gynecologic or obstetrical event (grade B). In case of past history of a single pregnancy delivery before 34 weeks gestation (WG), ultrasound cervical length screening is recommended between 16 and 22 WG in order to propose a cerclage in case of length&lt;25mm before 24 WG (grade C). Ultrasound-indicated cerclage is not recommended for multiple pregnancy with a short cervix (grade B). Emergency cerclage using the MacDonald technique is recommended during the second trimester of pregnancy in case of major changes of the cervix, with or without protrusion of the fetal membranes, but without premature rupture of membranes or chorioamnionitis (grade C). Tocolysis and antibiotics during cerclage should be considered individually (professional consensus). There is no reason to recommend a period of expectative before considering an emergency cerclage (professional consensus). A maximum gestational age to perform a cerclage cannot be recommended (professional consensus). A cervico-isthmic cerclage can be discussed in case of failure of MacDonald cerclage (professional consensus). Scientific data are insufficient to recommend or not a vaginal bacteriological analysis before performing a cerclage (professional consensus). The use of double cerclage does not improve perinatal outcome (NP3) and is not recommended (grade C). There is insufficient scientific argument to recommend a type of stitch over another (grade C). The available data are not in favor of a superiority of the Shirodkar cerclage in case of history- or ultrasound-indicated cerclage and the MacDonald cerclage is firstly recommended because technically easier and less risky (grade C). Overall, complications of cerclage are rare but potentially serious. The occurrence of complications is no different between the history-indicated and echo-indicated cerclage (LE4). There is no scientific evidence on the benefit of bed rest and adjuvant treatments (antibiotics or indomethacin) during history or ultrasound-indicated cerclage (professional consensus). Available data in the literature about cervical cerclage are generally of low level of evidence."}, {"id": "Obstentrics_Williams_1059", "title": "Obstentrics_Williams", "score": 0.009418207239397236, "content": "3. Nullpara-a woman who has never completed a pregnancy beyond 20 weeks' gestation. She may not have been pregnant or may have had a spontaneous or elective abortion(s) or an ectopic pregnancy. 4. Primipara-a woman who has been delivered only once of a fetus or fetuses born alive or dead with an estimated length of gestation of 20 or more weeks. In the past, a 500-g birthweight threshold was used to define parity. his threshold is now controversial because many states still use this weight to diferentiate a stillborn fetus from an abortus (Chap. 1, p. 3). However, the survival of neonates with birthweights < 500 g is no longer uncommon."}, {"id": "pubmed23n0763_24776", "title": "Preconception counseling for preventable risks.", "score": 0.009345794392523364, "content": "A healthy woman of reproductive age complained that when she saw me before pregnancy I did not advise her that she could check her varicella immunity and get vaccinated. She contracted chickenpox and endured unnecessary anxiety. This led me to think that it would be useful to have a summary of all the preconception counseling advice we should give to our patients to ensure the best pregnancy outcomes possible. Could Motherisk provide such a summary? Although favourable pregnancy outcomes cannot be guaranteed, when a pregnancy is planned, many risk factors can be reduced and modified to enhance pregnancy outcomes. In the summary provided we will discuss optimization of diet, weight, and exercise; discontinuation of smoking and drinking; controlling chronic medical conditions; starting supplementation with multivitamins and folic acid; and ensuring proper immunization."}, {"id": "Obstentrics_Williams_2502", "title": "Obstentrics_Williams", "score": 0.009345794392523364, "content": "Stoddard A, Eisenberg DL: Controversies in family planning: timing of ovulation after abortion and the conundrum of postabortion intrauterine device insertion. Contraception 84(2):119, 2011 Stoval N, Sibai B, Habli M: Is there a role for cerclage in twin gestation with short cervical length (CL)? Single center experience. Abstract No. 143, Am ] Obstet Gynecole208(1 Suppl):S73, 2013 Stubbleield PG, Altman M, Goldstein SP: Randomized trial of one versus two days of laminaria treatment prior to late midtrimester abortion by uterine evacuation: a pilot study. Am] Obstet GynecoI143(4):481, 1982 Sugibayashi S, Aeby T, Kim D, et al: Amniotic luid arborization in the diagnosis of previable preterm premature rupture of membranes. ] Reprod Med 57(3-4):136,e2012 Sullivan E, Silver M, LaCoursiere DY, et al: Recurrent fetal aneuploidy and recurrent miscarriage. Obstet Gynecol 104:784,e2004"}, {"id": "pubmed23n1125_9350", "title": "Endoscopic transabdominal cervical cerclage replacement after recurrent late miscarriage.", "score": 0.009259259259259259, "content": "Transabdominal cerclage (TAC) is a recognised treatment for recurrent spontaneous late miscarriage or preterm birth due to cervical weakness. This can be performed via an open procedure before and during pregnancy, or a laparoscopic technique preconception. Complications include cerclage failure and suture migration. We present a case highlighting these complications where laparoscopic removal of an open TAC and replacement led to two successful term deliveries. A woman in her thirties with a fibroid uterus, adenomyosis and a history of three spontaneous mid-trimester losses, had an open TAC at 13 weeks of gestation. Preterm premature rupture of the membranes occurred shortly after and at 18 weeks of gestation she underwent surgical evacuation of the uterus. Subsequent hysteroscopy confirmed migration of the cerclage through the cervical canal. We demonstrate the application of endoscopic gynaecological surgery to remove and replace the TAC with two successful term births by Caesarean section in the ensuing pregnancies."}, {"id": "pubmed23n1103_334", "title": "[Analysis of the effect of modified cervical cerclage in the treatment of cervical insufficiency].", "score": 0.009259259259259259, "content": "<bObjective:</b To discuss the surgical effect of modified cervical cerclage for the treatment of pregnant women with cervical insufficiency. <bMethods:</b The clinical data of 225 pregnant women who underwent modified cervical cerclage in Qilu Hospital (Qingdao) were selected for retrospective analysis from April 2014 to June 2020. Surgical success rate, full-term birth rate, preterm birth rate, prolonged pregnancy weeks and newborn birth weight were compared between singleton and twin pregnancies, preventive cerclage and emergency cerclage, surgery before and after 18 weeks, naturally and in vitro fertilization and embryo transfer (IVF-ET) conceived pregnant women respectively. <bResults:</b Among the 225 pregnant women, the gestational weeks of surgery were 14-24<sup+5</sup weeks, mean gestational weeks of delivery were 38<sup+2</sup weeks (35<sup+5</sup-39<sup+3</sup weeks), the number of prolonged gestation were (20.3±5.2) weeks, and the newborn birth weight was (3 065±735) g; the overall surgical success rate was 92.9% (209/225), and the miscarriage rate was 7.1% (16/225); among the surviving newborns, the full-term birth rate was 73.7% (154/209), and the preterm birth rate was 26.3% (55/209). All cases had no intraoperative complications. Among the 225 pregnant women, 202 (89.8%, 202/225) cases were singleton pregnancies, and 23 (10.2%, 23/225) cases were twin pregnancies; 201 (89.3%, 201/225) cases underwent preventive cervical cerclage, and 24 (10.7%, 24/225) cases underwent emergency cervical cerclage; 190 (84.4%, 190/225) cases underwent the surgery before 18 weeks, and 35 (15.6%, 35/225) cases underwent the surgery after 18 weeks; 49 (21.8%, 49/225) cases were conceived by IVF-ET. There was no statistically significant difference in the overall surgical success rate of single and twin group (<iP</i&gt;0.05). The full-term birth rate, newborn birth weight and prolonged pregnancy weeks of single group were higher than those of twin group (<iP</i&lt;0.05). There were no statistical differences between preventive and emergency cerclage in overall surgical success rate, full-term birth rate, preterm birth rate, and newborn birth weight (all <iP</i&gt;0.05). The pregnancy prolonged weeks of preventive cerclage was higher than that of emergency cerclage (<iP</i&lt;0.05). There were no statistically significant differences in the overall surgical success rate, full-term birth rate, preterm birth rate and birth weight of newborns at different surgical timings (all <iP</i&gt;0.05). The pregnancy prolonged week for those who underwent surgery before 18 weeks was higher than that of surgery after 18 weeks (<iP</i&lt;0.05). The premature birth rate of IVF-ET was higher than that of naturally conceived pregnant women (<iP</i&lt;0.05). <bConclusion:</b The modified cervical cerclage could effectively prolong the gestational weeks of delivery, reduce the rate of preterm birth, and the operation is simple and easy to promote. It could be used as a surgical option for patients with cervical insufficiency."}, {"id": "pubmed23n1150_14697", "title": "Persistent vs Recurrent Cushing's Disease Diagnosed Four Weeks Postpartum.", "score": 0.009174311926605505, "content": "Cushing's disease (CD) recurrence in pregnancy is thought to be associated with estradiol fluctuations during gestation. CD recurrence in the immediate postpartum period in a patient with a documented dormant disease during pregnancy has never been reported. <iCase Report</i. A 30-year-old woman with CD had improvement of her symptoms after transsphenoidal resection (TSA) of her pituitary lesion. She conceived unexpectedly 3 months postsurgery and had no symptoms or biochemical evidence of recurrence during pregnancy. After delivering a healthy boy, she developed CD 4 weeks postpartum and underwent a repeat TSA. Despite repeat TSA, she continued to have elevated cortisol levels that were not well controlled with medical management. She eventually had a bilateral adrenalectomy. <iDiscussion</i. CD recurrence may be higher in the peripartum period, but the link between pregnancy and CD recurrence and/or persistence is not well studied. Potential mechanisms of CD recurrence in the postpartum period are discussed below. We describe the first report of recurrent CD that was quiescent during pregnancy and diagnosed in the immediate postpartum period. Understanding the risk and mechanisms of CD recurrence in pregnancy allows us to counsel these otherwise healthy, reproductive-age women in the context of additional family planning."}, {"id": "pubmed23n0223_13874", "title": "[Report on 8 years' experience in the prenatal diagnosis of genetic defects. III. Outcome of pregnancy].", "score": 0.009174311926605505, "content": "The courses of 997 pregnancies out of 1113 prenatal diagnoses were analysed. The total rate of all fetal and neonatal losses is 11.6 per cent inclusively the therapeutically induced abortions. Peculiarities of our material are the shift to higher ages and the inclusion of pregnancies following amniofetography and fetoscopy. Without the last ones the perinatal mortality is 18.9 per thousand, the abortion rate is 2.6 per cent, the rate of premature deliveries (till the 37th gestational week) is 10.2 per cent and the rate of low birth weight babies below 2500 g is 6.1 per cent in connection with a frequency of caesarean sections of 9 per cent. 91 per cent of the newborns had Apgarscore of 8 to 10. The most favourable courses with an abortion rate of 2.2 per cent, a perinatal mortality of 11.8 per cent and a rate of low birth weight babies of 5.7 per cent are to be found following uncomplicated amniocentesis. Perinatal mortality is increased about the factor 10 combined with an threefold raise of prematurity in cases of brownish amniotic fluid. If the amniotic fluid was bloody and the repeated insertions during amniocentesis abortion rate is 10 per cent and perinatal mortality 44.4 per thousand. --3.2 per cent of pregnancies after fetoscopy are terminated by abortions. Perinatal mortality is 33.3 per thousand, the rate of low birth weight babies 26.7 per cent. Pregnancies following amniofetography have a most unfavourable course. The abortion rate is elevated to 11.2 per cent, perinatal mortality to 139.2 per thousand with a rate of low birth weight babies of 41.8 per cent. Following amniofetography every fourth newborn has a birth weight below 1500 grams.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0845_18810", "title": "[Management of Cervical Cancer Stage I B during Pregnancy].", "score": 0.00909090909090909, "content": "Management and treatment of stage I B1 cervical cancer during pregnancy depends on the estimated gestational age and personal desires. We report 4 cases of stage I B1 cervical cancer during pregnancy that were treated differently. Case 1: A 29- year-old woman, primipara, visited our hospital at 7 weeks' gestation. She was diagnosed with a stage I B1 cervical cancer by using conization at 12 weeks' gestation. She strongly desired childbirth and therefore was treated at 29 weeks' gestation with a simultaneous cesarean section and radical surgery. Case 2: A 26-year-old woman, para 1, was diagnosed with stage I B1 cervical cancer at 23 weeks' gestation. She was treated at 28 weeks' gestation with a simultaneous cesarean section and radical surgery. Case 3: A 36-year-old woman, para 7, at 18 weeks' gestation, visited our hospital because of a stage I A cervical cancer. She chose to undergo abortion and radical surgery, which were performed simultaneously at 21 weeks' gestation. After the surgery, she was diagnosed with a stage I B1 cervical cancer pathologically. Case 4: A 33-year-old woman, para 2, was diagnosed with a stage I B2 cervical cancer at 30 weeks' gestation and was treated with a simultaneous cesarean section and radical surgery at 31 weeks' gestation. "}, {"id": "pubmed23n0886_14953", "title": "[Induced abortion: Guidelines for clinical practice - Text of the Guidelines (short text)].", "score": 0.00909090909090909, "content": "Develop recommendations for the practice of induced abortion. The Pubmed database, the Cochrane Library and the recommendations from the French and foreign Gyn-Obs societies or colleges have been consulted. The number of induced abortions (IA) has been stable for several decades. There are a lot of factors explaining the choice of abortion when there is an unplanned pregnancy (UPP). Early initiation and choice of contraception in connection to the woman's life are associated with lower NSP. Reversible contraceptives of long duration of action should be positioned fist in line for the teenager because of its efficiency (grade C). Ultrasound before induced abortion must be encouraged but should not be obligatory before performing IA (Professional consensus). As soon as the sonographic apparition of the embryo, the estimated date of pregnancy is done by measuring the crown-rump length (CRL) or by measuring the biparietal diameter (BIP) from 11 weeks on (grade B). Reliability of these parameters being±5 days, IA could be done if measurements are respectively less than 90mm for CRL and less than 30mm for BIP (Professional consensus). A medical IA performed with a dose of 200mg mifepristone combined with misoprostol is effective at any gestational age (EL1). Before 7 weeks, mifepristone followed between 24 and 48hours by taking misoprostol orally, buccally sublingually or eventually vaginally at a dose of 400 ug possibly renewed after 3hours (EL1, grade A). Beyond 7 weeks, misoprostol given vaginally, sublingually or buccally are better tolerated with fewer side effects than oral route (EL1). It is recommended to always use a cervical preparation during an instrumental abortion (Professional consensus). Misoprostol is a first-line agent for cervical preparation at a dose of 400 mcg (grade A). Aspiration evacuation is preferable to curettage (grade B). A perforated uterus during an instrumental suction should not be considered as a scarred uterus (Professional consensus). IA is not associated with increased subsequent risk of infertility or ectopic pregnancy (EL2). The pre-abortion medical consultations does not affect, most of the time, the decision to request an IA. Indeed, a majority of women is quite sure of her choice during these consultations. Acceptability of the method of IA and satisfaction appears to be larger when they are able to choose the abortion method (grade B). There is no relationship between an increase in psychiatric disorders and IA (EL2). Women with psychiatric histories are at increased risk of mental disorders after the occurrence of an UPP (EL2). In case of instrumental abortion, oral estrogen-progestogen contraceptives and the patch should be started from the day of the abortion, the vaginal ring inserted within 5 days of IA (grade B). In case of medical abortion, the vaginal ring should be inserted within a week of taking mifepristone, oral estrogen-progestogen contraceptives and the patch should be initiated on the same day or the day after taking prostaglandins (grade C). In case of instrumental abortion, the contraceptive implant may be inserted on the day of the abortion (grade B). In case of medical abortion, the implant can be inserted on the day of mifepristone (grade C). The copper Intrauterine Device (IUD) and levonorgestrel should be inserted preferably on the day of instrumental abortion (grade A). In case of medical abortion, an IUD can be inserted within 10 days following mifepristone after ensuring by ultrasound of the absence of intrauterine pregnancy (grade C). The implementation of these guidelines may promote a better and more homogenous care for women requesting IA in our country."}, {"id": "pubmed23n0977_4656", "title": "Twin pregnancies treated with emergency or ultrasound-indicated cerclage to prevent preterm births.", "score": 0.009009009009009009, "content": "<bIntroduction:</b Multiple gestations are high-risk pregnancies associated with an increased risk of neonatal morbidity and mortality, mainly due to preterm births. Numerous interventions have been attempted in order to delay the time of delivery and subsequently, prevent preterm births in twin gestations. To date, no really effective intervention has been found. Use of cerclage in twin pregnancies has been controversial. Recently, however, small retrospective cohort studies have shown a potentially positive effect with the use of cerclage in twin pregnancies. The aim of this study was to evaluate pregnancies and neonatal outcomes in twin gestations with a short cervix treated with cervical cerclage at a single University Hospital.<bMethods:</b This retrospective cohort study included all women - with twin gestation and a short cervix - who had an ultrasound indicated or emergency cervical cerclage at the Department of Obstetrics and Gynecology, Aarhus University Hospital, Skejby, Denmark between January 1999 and May 2017. Cervical cerclage was offered to women before 26 weeks of gestation if: (1) the cervix at ultrasound was ≤20 mm without cervical dilatation (ultrasound-indicated cerclage), or (2) the cervix at ultrasound was ≤20 mm with cervical dilatation (emergency cerclage). Women with history-indicated cerclage placement or multifetal embryo reduction were excluded. A total of 65 women participated in the study.<bResults:</b The median gestational age at cerclage placement was 22.6 weeks with a median cervical length of 10 mm (range 0-20 mm). The frequency of preterm delivery before 32 weeks of gestation was 41.5% and 27.7% before 28 weeks. Median pregnancy latency was 77 days (range 4-148) and the median gestational age at delivery was 33.0 weeks. Gestational age at delivery was significantly lower among women with cervical dilatation and visible membranes than among women with a short cervix only (27.7 versus 33.6 weeks, <ip</i &lt; .01) and so was the median pregnancy latency (48 versus 81 days, <ip</i &lt; .05). Overall, neonatal survival was 91.5%.<bConclusion:</b Cervical cerclage in twin pregnancies may prolong pregnancy even when placed on a very short or dilated cervix. In our study, the procedure was safe and without any serious complications. The overall neonatal survival rate was high."}, {"id": "pubmed23n0066_1471", "title": "Recurrent miscarriage.", "score": 0.009009009009009009, "content": "On epidemiological evidence, the definition of recurrent miscarriage should be three or more consecutive pregnancy losses. Data should be collected to 28 weeks' gestation but analysis up to 20-22 weeks' or 500 g fetal weight should also be possible. General practitioners and gynaecologists should do what they feel is suitable for couples whose history does not meet these criteria but a diagnosis of recurrent miscarriage should not be made. Women meeting the definition can be subdivided into primary and secondary groups, respectively consisting of those who have lost all previous pregnancies and those who have had one successful pregnancy followed by consecutive losses."}, {"id": "pubmed23n0404_9766", "title": "Where no consent = death.", "score": 0.008928571428571428, "content": "Men must be made to understand the value of family planning - particularly in societies where men hold the power of decision in the family.  Dr. Kotha Pannikar, chairman of the Kedah Family Planning Association (FPA) in Malaysia, illustrated this point in discussion which followed the Consultation of Medical and Communication Fieldworkers conference in Kuala Lumpur in August, with a story about 1 of her own patients.  When the girl, who had a rheumatic heart, was 16, Dr. Pannikar advised the parents that she needed cardiac surgery if she were to be a healthy wife and mother.  But the parents lived some distance from Dr. Pannikar's surgery and did not heed the advice.  The girl was married to a carpenter from a traditional Chinese family, in which \"the man is lord and master.\" Her new home had no piped water, and in additional to normal domestic tasks she had to carry water from a source 1 1/2 miles agay.  In the 7th month of her 1st pregnancy, she went into cardiac failure.  After the 3rd pregnancy and a 3rd cardiac failure, Dr. Pannikar tried to arrange a sterilization \"but we could not get consent - her husband refused to turn up at the hospital.\" When the girl was admitted to hospital 6 months into her 4th pregnancy, Dr. Pannikar got hold of her patient's mother-in-law.  \"I told her if she wanted a servant in the house, it was easy to get one.  But no servant would look after her grandchildren the way their mother would.  I told her if she wanted to save the girl's life she had better speak to her son.\" During the 4th delivery, the girl went into cardiac arrest and spent 2 weeks in intensive care.  The mother-in-law prevailed upon her son to at least consent, and the girl was sterilized before she left hospital.  But \"it was a very near thing,\" Dr. Pannikar recalls \"and it wouldn't have happened if the husband had felt he was responsible in parenthood.\" The Kedah FPA makes special efforts to reach men.  Dr. Pannikar herself talks to men's organizations like the Lions and Rotary Clubs, and arranges education programs for trade unions and workers on the rubber estates.  She thinks women need to be told repeatedly that they have a basic human right to choose whether they want to have a baby, and when.  \"Women feel,\" she says, \"that their only function is to cook, wash clothes and feed the baby.  We need to tell them they have a part to play in the society of today because their children will be the citizens of tomorrow.\"o"}, {"id": "pubmed23n1073_316", "title": "[Clinical outcomes and influence factors of 435 singleton pregnancies with short cervix].", "score": 0.008928571428571428, "content": "<bObjective:</b To investigate the clinical outcomes of different treatment options on singleton short cervix and its influence factors. <bMethods:</b Totally 435 cases of singleton pregnancies who were diagnosed with short cervix (≤25 mm) between 12 to 33<sup+6</sup gestational weeks in Peking University First Hospital from January 2018 to December 2018 were enrolled, including 21 cases with cervical length &lt;10 mm, 414 cases with cervical length between 10 to 25 mm. The onset time was &lt;24 gestational weeks in 106 cases, while 104 cases were at 24-29<sup+6</sup gestational weeks and 225 cases of ≥30 gestational weeks. Gestational outcomes including delivery before 37 weeks, delivery before 34 weeks, neonatal birth weight (NBW) and adverse neonatal outcome (ANO) were compared among three treatment groups: rest group, progesterone group and cerclage group. Influence factors were also investigated. <bResults:</b (1) The incidence of short cervix in pregnancy was 7.07% (435/6 155), while 106 cases were at &lt;24 gestational weeks (1.72%, 106/6 155), 104 cases (1.69%, 104/6 155) at 24-29<sup+6</sup gestational weeks and 225 cases (3.66%, 225/6 155) at ≥30 gestational weeks. (2) In the group of cervical length &lt;10 mm, rate of delivery before 37 and 34 weeks were 62% (13/21) and 57% (12/21) respectively. One case of progesterone treatment underwent miscarriage. Compared with rest group (<in</i=8), delivery weeks [28.5 (25.0-40.0) vs 37.0 (28.0-40.0), <iP</i=0.020] and NBW [1 245 g (630-3 830 g) vs 2 648 g (1 560-3 830 g), <iP</i=0.028] were higher in cerclage group (<in</i=9), while ANO was not statistically different (<iP</i&gt;0.05). (3) In the group of cervical length ≥10 mm before 24 gestational weeks, the delivery weeks, incidence of delivery before 34 weeks, adjusted incidence of delivery before 37 weeks, NBW and ANO were not statistically different (<iP</i&gt;0.05) among rest group (<in</i=36), progesterone group (<in</i=26) and cerclage group (<in</i=34). In vitro fertilization (<iOR</i=11.97, 95%<iCI</i: 1.88-76.44, <iP</i=0.009), infection (<iOR</i=46.03, 95%<iCI</i: 5.12-413.58, <iP</i=0.001), sludge on sonography (<iOR</i=9.87, 95%<iCI</i: 1.69-57.60, <iP</i=0.011) and history of short cervix (<iOR</i=7.24, 95%<iCI</i: 1.04-50.24, <iP</i=0.045) were independent risk factors of preterm birth. (4) In the group of cervical length ≥10 mm and gestational weeks between 24-29<sup+6</sup, the delivery weeks, incidence of delivery before 37 weeks, incidence of delivery before 34 weeks, NBW and ANO were not statistically different (<iP</i&gt;0.05) among rest group (<in</i=52), progesterone group (<in</i=34) and cerclage group (<in</i=9). Infection was an independent risk factor of preterm birth (<iOR</i=56.40, 95%<iCI</i: 4.67-680.61, <iP</i=0.002). (5) Outcomes of 223 cases were relatively good in the group of cervical length ≥10 mm beyond 30 gestational weeks. The incidence of delivery before 34 weeks was 6.3% (14/223). The delivery weeks, incidence of delivery before 37 and 34 weeks, NBW and ANO were not statistically different (<iP</i&gt;0.05) among 3 groups. Infection (<iOR</i=10.91, 95%<iCI</i: 2.21-53.96, <iP</i=0.003) and history of preterm birth (<iOR</i=8.63, 95%<iCI</i: 1.25-59.65, <iP</i=0.029) were independent risk factors of preterm birth. <bConclusions:</b Short cervix is a common complication of pregnancy. Cervical cerclage is related with better outcome for patients with cervical length &lt;10 mm. Neither progesterone nor cervical cerclage improves pregnancy outcome for &gt;10 mm cervical length patients comparing with rest. Infection, sludge, in vitro fertilization, history of short cervix and history of preterm birth are independent risk factors of preterm birth in short cervix pregnancies."}, {"id": "pubmed23n1133_19127", "title": "<i>Peptoniphilus indolicus</i> infection in a pregnant woman: a case report.", "score": 0.008849557522123894, "content": "<iPeptoniphilus indolicus</i belongs is a gram-positive anaerobic coccus (GPAC), which can cause bacterial vaginitis. However, only a few studies have reported severe infection of <iP. indolicus</i. This study presented the first case of severe infection of <iP. indolicus</i during pregnancy. It aimed to help to fill the gap in the literature, find out the factors that accelerate infection and discuss the significance of the GPAC test. A 35-year-old woman was admitted due to unbearable abdominal pain with dilation of the cervical opening at 22+ weeks of gestation. A blood test revealed electrolyte disturbance and hypoproteinemia. A day before admission, the patient developed pain in the lower abdomen accompanied by yellow-green vaginal discharge. Two hours after admission, the patient suddenly presented with hyperpyrexia and chills. Timely and adequate antibiotic and cooling treatments were administered. After 14 h, the patient again developed chills that lasted for approximately 20 min, accompanied by uterine contractions and membrane rupture. After 3 h, she had a miscarriage and rapidly developed septic shock. She was transferred to the intensive care unit for further infection control, shock correction, and circulatory stabilization. The cultures of blood, secretion specimen, and amniotic fluid indicated <iP. indolicus</i infection using a matrix-assisted laser desorption/ionization time-of-flight mass spectrometry, an advanced tool for bacterial species identification. <iP. indolicus</i is an opportunistic pathogen in pregnant women. Poor physical conditions and pregnancy may accelerate disease progression and lead to severe inflammation."}, {"id": "pubmed23n1072_1265", "title": "Maternal and fetal effects of COVID-19 virus on a complicated triplet pregnancy: a case report.", "score": 0.008771929824561403, "content": "Coronavirus disease 2019 (COVID-19), the global pandemic that has spread throughout the world, is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Given the limited scientific evidence on the manifestations and potential impact of this virus on pregnancy, we decided to report this case. The patient was a 38 year-old Iranian woman with a triplet pregnancy and a history of primary infertility, as well as hypothyroidism and gestational diabetes. She was hospitalized at 29 weeks and 2 days gestational age due to elevated liver enzymes, and finally, based on a probable diagnosis of gestational cholestasis, she was treated with ursodeoxycholic acid. On the first day of hospitalization, sonography was performed, which showed that biophysical scores and amniotic fluid were normal in all three fetuses, with normal Doppler findings in two fetuses and increased umbilical artery resistance (pulsatility index [PI] &gt; 95%) in one fetus. On day 4 of hospitalization, she developed fever, cough and myalgia, and her COVID-19 test was positive. Despite mild maternal symptoms, exacerbated placental insufficiency occurred in two of the fetuses leading to the rapid development of absent umbilical artery end-diastolic flow. Finally, 6 days later, the patient underwent cesarean section due to rapid exacerbation of placental insufficiency and declining biophysical score in two of the fetuses. Nasopharyngeal swab COVID-19 tests were negative for the first and third babies and positive for the second baby. The first and third babies died 3 and 13 days after birth, respectively, due to collapsed white lung and sepsis. The second baby was discharged in good general condition. The mother was discharged 3 days after cesarean section. She had no fever at the time of discharge and was also in good general condition. This was a complicated triplet pregnancy, in which, after maternal infection with COVID-19, despite mild maternal symptoms, exacerbated placental insufficiency occurred in two of the fetuses, and the third fetus had a positive COVID-19 test after birth. Therefore, in cases of pregnancy with COVID-19 infection, in addition to managing the mother, it seems that physicians would be wise to also give special attention to the possibility of acute placental insufficiency and subsequent fetal hypoxia, and also the probability of vertical transmission."}, {"id": "pubmed23n0751_888", "title": "Prolonged and post-term pregnancies: guidelines for clinical practice from the French College of Gynecologists and Obstetricians (CNGOF).", "score": 0.008771929824561403, "content": "The duration of pregnancy varies between 40(+0) and 41(+3) weeks. Conventionally, and essentially arbitrarily, a pregnancy is considered to be \"prolonged\" after 41(+0) weeks, but the infant is not considered \"post-term\" until 42(+0) weeks (Professional consensus). A term birth thus occurs during the period from 37(+0) to 41(+6) weeks. In France, prolonged pregnancies (≥41(+0)weeks) involve 15-20% of pregnant women, and post-term pregnancies (≥42(+0) weeks) approximately 1%. The frequency of post-term pregnancies is very heterogeneous: in Europe and the United States, it ranges from 0.5% to 10% according to country. In prolonged pregnancies, the cesarean section rate-especially the emergency cesarean rate-is multiplied by approximately 1.5 (grade B). From 37(0-6) to 43(0-6) weeks, the risk of perinatal mortality increases regularly, from 0.7‰ to 5.8‰. Meconium aspiration syndrome is responsible for substantial morbidity and mortality, and its incidence increases regularly between 38(+0) and 42(+6) weeks, from 0.24‰ to 1.42‰ (grade B). Similarly, the risks of neonatal acidosis (grade B), 5-min Apgar scores less than 7 (grade B) and admissions to neonatal intensive care (grade B) increase progressively between 38(+0) and 42(+6) weeks. These risks appear to double for post-term growth-restricted newborns (grade C). Ultrasound dating of the pregnancy makes it possible to reduce the risk that it will be incorrectly considered prolonged and that labor will therefore be induced unnecessarily. To harmonize practices, if the crown-rump length (CRL) is correctly measured (this measurement should be taken between 11(+0) and 13(+6) weeks, when CRL should measure from 45 to 84mm), ultrasound dating based on it should be used to determine the official date pregnancy began, regardless of its difference from the date assumed by the patient or estimated based on the date of the last menstrual period. This rule does not apply to pregnancies by IVF, for which the date pregnancy began is defined by the date of oocyte retrieval (Professional consensus). From 37(0-6) to 43(0-6) weeks, the risk of perinatal mortality increases regularly and there is no threshold at which a clear increase in perinatal mortality becomes visible. Fetal monitoring by cardiotocography (CTG) that begins at 41(+0) weeks would cover approximately 20% of women and reduce perinatal morbidity compared with monitoring that begins at 42(+0) weeks (grade C). The frequency recommended for this monitoring ranges between two and three times a week (Professional consensus). For ultrasonography assessment, measurement of the largest fluid pocket is recommended, because measurement of the amniotic fluid index (that is, the sum of the four quadrants) is accompanied by more diagnoses of oligohydramnios, inductions of labor, and cesareans for fetal distress without any improvement in neonatal prognosis (grade A). The practice of assessing the Manning biophysical score increases the number of diagnoses of oligohydramnios and fetal heart rage (FHR) abnormalities and generates an increase in the rates of inductions and cesareans without improving neonatal prognosis. The use of this biophysical score in monitoring prolonged pregnancies is therefore not recommended (grade B). In the absence of a specific disorder, induction of labor can be proposed in patients between 41(+0) and 42(+6) weeks (grade B). Nonetheless, the choice of prolongation beyond above 42(+0) weeks appears to involve an increase in fetal risk, which must be explained to the patient and balanced against the potential disadvantages of induction (Professional consensus). Stripping the membranes can reduce the duration of pregnancy by increasing the number of patients going into labor spontaneously during the week afterward (grade B). Compared to an expectant approach, it does not increase the cesarean section rate (grade A). It reduces recourse to induction by 41% at 41(+0) weeks and by 72% at 42(+0) weeks (grade B), without increasing the risk of either membrane rupture or maternal or neonatal infection (grade B). Used as a tampon or vaginal gel, prostaglandins E2 (PGE2) are an effective method of inducing labor (grade A). They can be used to induce labor successfully, regardless of cervical ripeness (grade A). If misoprostol is chosen, the lowest dose is to be preferred, starting with a vaginal dose of 25μg every 3-6h (grade A). For misoprostol, more powerful studies remain necessary for better defining the doses, routes of administration, tolerance and indications. Misoprostol at any dose is contraindicated in women with uterine scars (grade B). Placement of an intracervical Foley catheter is an effective mechanical means of inducing labor, with less uterine hyperstimulation than prostaglandins and no increase in the cesarean section rate (grade A). Nonetheless, as the risk of infection might be increased, this technique requires more robust evaluation before entering general practice (grade B). In cases of meconium-stained amniotic fluid, pharyngeal aspiration before delivery of the shoulders is not recommended (grade A). The team managing a post-term newborn with meconium-stained amniotic fluid at birth must know how to perform intubation and, if the intubation is not helpful, endotracheal aspiration (grade C) and ventilation with a mask. Routine endotracheal intubation of a vigorous newborn is not recommended (grade A)."}, {"id": "Obstentrics_Williams_5759", "title": "Obstentrics_Williams", "score": 0.008695652173913044, "content": "pregnancy continuation. For women facing a poor pregnancy prognosis due to cervical dilation at midgestation, it seems reasonable to ofer emergency or rescue cerclage with appropriate counseling. However, it is unclear if such interventions truly confer a beneit or merely increase the risk of membrane rupture and infection (Hawkins, 2017)."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 63]], "word_ranges": [[0, 12]], "text": "In the case of free wall rupture, there is no palpable frémito."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "In the case of free wall rupture, there is no palpable frémito.", "full_answer_no_ref": "In the case of free wall rupture, there is no palpable frémito.", "full_question": "A 70-year-old female patient is admitted to the ICU after suffering anterior AMI treated by coronary angioplasty and stent placement in the anterior descending artery. Four days later she suddenly presented hypotension that required vigorous volume support, initiation of vasoactive drugs, orotracheal intubation and connection to mechanical ventilation. Physical examination revealed a murmur not previously present. Suspicion of a mechanical complication of the infarction led to transthoracic echocardiography showing pericardial effusion. Mark the CORRECT answer:", "id": 282, "lang": "en", "options": {"1": "Mortality with medical treatment is 20%.", "2": "In case of free wall rupture there is an oximetric jump in the right ventricle in the Swan-Ganz catheterization.", "3": "In case of free wall rupture, there is no palpable frémito.", "4": "Mechanical complications usually appear on the first post-infarction day.", "5": NaN}, "question_id_specific": 55, "type": "CARDIOLOGY AND VASCULAR SURGERY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0114_7402", "title": "Myocardial rupture following acute myocardial infarction.", "score": 0.01906318082788671, "content": "Ten patients, eight males and two females, suffered myocardial rupture following acute myocardial infarction and required surgery. There were five ventricular septal ruptures, four papillary muscle ruptures and one free wall rupture. Ventricular septal rupture was suspected clinically by the appearance of a new systolic murmur, usually associated with a thrill at the left sternal border. A left to right shunt was confirmed by bedside oximetry using a Swan-Ganz catheter. The mean pulmonary to systemic flow ratio was 3.04:1. Following cardiac catheterization all patients underwent corrective surgery with or without aortocoronary bypass grafting. Three patients with inferior wall myocardial infarction died. Papillary muscle rupture was suspected clinically following the abrupt onset of hypotension with severe acute pulmonary edema accompanied by a new systolic murmur. The diagnosis was confirmed by cardiac catheterization. All underwent surgery for mitral valve replacement with or without aortocoronary bypass grafting. One patient died postoperatively of multiorgan failure. Free wall rupture was suspected clinically by the sudden onset of loss of consciousness, apnea, junctional bradycardia and severe hypotension leading to electromechanical dissociation. The diagnosis was confirmed by demonstrating a significant pericardial effusion by two dimensional echocardiography. Immediate surgery was performed. This patient is totally asymptomatic on no drug treatment six months following discharge. Ten patients underwent emergency surgery for myocardial rupture. Operative mortality was 40%. Patients with ventricular septal rupture associated with an inferior myocardial infarction had a poor prognosis."}, {"id": "pubmed23n0974_20927", "title": "Right ventricular rupture induced by cardiopulmonary resuscitation.", "score": 0.017905459175553078, "content": "Right ventricular rupture is a rare complication of cardiopulmonary resuscitation and could be fatal. We report a survival case of right ventricular rupture induced by cardiopulmonary resuscitation in a patient with acute myocardial infarction. A 57-year-old man was admitted to our hospital with ventricular fibrillation. Although chest compression and defibrillation were performed, ventricular fibrillation continued. We inserted a percutaneous cardiopulmonary system and performed coronary angiography, which revealed occlusion of the left anterior descending artery. After coronary stenting and intra-aortic balloon pumping, we succeeded in defibrillation and vital signs became stable. Twenty hours after the intervention, systolic blood pressure dropped to 60 mmHg. Ultrasonic cardiogram at that time revealed massive pericardial effusion. We diagnosed cardiac tamponade, and 8Fr drainage tube was placed in the pericardial space. We determined that emergent operation was necessary because we suspected left ventricular rupture due to acute myocardial infarction or coronary rupture induced by percutaneous coronary intervention. However, operative findings revealed right ventricular free wall rupture, which could have been induced by chest compression. In these cases, we should consider the possibility of not only the rupture of left ventricle and coronary artery but also the rupture of right ventricle induced by cardiopulmonary resuscitation."}, {"id": "pubmed23n0074_12913", "title": "[Myocardial infarction complicating left ventricular free wall blowout rupture: a survival case after surgical repair].", "score": 0.01709901351845086, "content": "A 58-year-old man who suffered from acute myocardial infarction complicated with left ventricular rupture and subacute pericardial tamponade was reported. On admission, echocardiography strongly suspected presence of intrapericardial fluid. And immediate pericardiocentesis proved left ventricular free wall rupture (LVFWR). Coronary angiography with the support of IABP revealed occlusion of LAD (# 8). Percutaneous transluminal coronary angioplasty was performed with partial success. After pericardiotomy, the hemodynamic state was improved, however, 2 hours later, his blood pressure fell down to 40 mmHg suddenly. Emergent operation (re-mediastinumotomy+ ) was performed under the suspicion of left ventricular blowout rupture with the direct closure of the perforated site with 4 woven Dacron pledgets at bedside in ICU. The patient ran an uneventful postoperative course and is now doing well. Clinical and therapeutic features of LVFWR were discussed."}, {"id": "pubmed23n0958_22306", "title": "Bedside Echocardiography in Acute Myocardial Infarction Patients with Hemodynamic Deterioration.", "score": 0.014723294723294724, "content": "Ventricular septal (VS) rupture after acute myocardial infarction (AMI) is an uncommon complication in the reperfusion era. Bedside echocardiography (BECH) continues to be a strong diagnostic tool for emergency physicians treating dyspneic patients, especially for decision-making on the management strategies to use with these unstable patients. In the case we present here, a patient is diagnosed with a delayed mechanical complication after AMI, and a swift management plan is made with the aid of point-of-care BECH. The patient is a 72-year-old man with dyspnea who was admitted to the ED 5 days after receiving a primary percutaneous coronary intervention with stent implantation for AMI; in the ED, the patient was diagnosed, via BECH, with a VS rupture. On arrival, his vital signs and the results of his physical examination depicted shock and low perfusion with wet lung. A cardiac examination revealed a new 2/6 harsh holosystolic murmur along the left sternal border without pretibial oedema. Emergency physicians performed BECH, and subcostal views of the heart revealed a wide interventricular septal rupture and left-to-right shunting with minimal pericardial effusion. The patient underwent surgery immediately to repair the defect. The post-operative course was uneventful, and he was discharged in stable condition on the seventh day after the surgery. The use of BECH to recognize a VS rupture is critical because such a defect may be the most important determinant of mortality in AMI patients who are in shock. BECH thus can influence clinicians' acute management and disposition decisions."}, {"id": "pubmed23n0267_10368", "title": "[Subacute rupture of the free wall of the heart. Clinical echocardiographic and pathological aspects apropos of 10 cases].", "score": 0.014433962264150942, "content": "Three distinct forms of rupture of the heart may be identified after myocardial infarction: sudden rupture with massive intrapericardial haemorrhage, and sudden death with clinical signs of electromechanical dissociation; rupture into the pericardium resulting in a false aneurysm, the treatment of which is surgical; subacute rupture which accounts for 30% of cases in which bleeding into the pericardium is slow and/or repeated. Over an 8 year period and in a series of 2,400 consecutive infarcts admitted to the intensive care unit, 10 cases of subacute rupture of the heart were diagnosed. They were 6 men and 4 women, with a mean age of 73.6 years. The clinical presentation was isolated chest pain in 5 cases, syncope alone in 2 cases and the association of pain and syncope in 3 cases. Six patients were in shock on admission. In two cases, shock developed after admission. The infarction was confirmed biologically by a significant elevation of creatinine kinase in 9 out of 10 cases. Transmural infarction was observed in 9 cases: the infarct was electrocardiographically non-transmural in 1 case. Emergency echocardiography showed pericardial effusion in all cases, usually moderate, but sometimes compressive with an intrapericardial echogenic mass suggesting a thrombus. Haemodynamic improvement was obtained by medication allowing cardiac catheterisation which showed adiastole in 3 cases. Coronary angiography was performed in 7 cases. In 5 of the 7 cases, apart from occlusion of the artery presumed to be responsible for the infarct, the coronary vessels were diffusely infiltrated without significant stenosis. Left ventriculography was performed in 7 cases. In 6 of the 7 cases regional akinesis was demonstrated: the 7th case showed dyskinesia of the anterior wall. In two cases, contrast medium was observed to fill the pericardium during ventriculography, indicating myocardial rupture. The diagnosis of subacute rupture, suggested by clinical and paraclinical (particularly echocardiography), was confirmed in 9 cases at surgery and in the 10th case at autopsy. Surgery consisted of repairing the rupture. In the last two cases, biological glue was used to reinforce the surgical repair. The clinical outcome was good after surgery in 6 cases with a follow-up of 5 months to 8 years. The diagnosis of subacute rupture should therefore be made on clinical and echocardiographic criteria, as these results suggest that surgery is often possible, with a good prognosis."}, {"id": "pubmed23n0516_13635", "title": "[Infarct exclusion for postinfarction left ventricular free wall rupture with severe congestive heart failure].", "score": 0.014377470355731226, "content": "A 70-year-old man was transferred to our hospital with severe congestive heart failure and ventricular arrhythmia due to acute myocardial infarction. He had experienced chest pain 3 weeks previously and was admitted to another hospital for dyspnea, where he required assist ventilation, 1 week prior to the transfer. An echocardiogram revealed a broad anteroseptal infarction and very poor left ventricular function with an ejection fraction (EF) of 22%. He remained in a severe congestive heart failure condition despite a full administration of catecholamines. Coronary angiogram findings revealed an occlusion of the proximal left anterior descending coronary artery and 1 week later severe hypotension was suddenly presented. An echocardiogram showed pericardial effusion with signs of cardiac tamponade. A pericardiocentesis was performed and hemodynamic improvement was obtained for a short time, after which the patient underwent urgent open heart surgery. During the operation, exclusion of the anteroseptal akinetic area using an oval patch was performed under a cardiopulmonary bypass and ventricular fibrillation. Severe cardiac failure remained postoperatively and the patient could not be weaned from cardiopulmonary bypass, therefore, we implanted a percutaneous cardiopulmonary support (PCPS) and started intraaortic balloon pumping (IABP). The patient was weaned from PCPS at 26 days after surgery and from IABP at 30 days. Following hospital release, he has continued to do well without heart failure for 39 months after the operation."}, {"id": "pubmed23n1033_9637", "title": "Surviving Case of a Blowout-Type Left Ventricular Free Wall Rupture During Percutaneous Coronary Intervention for a Lateral Acute Myocardial Infarction.", "score": 0.013737734165923284, "content": "A 76-year-old man suffering from chest pain was admitted to our hospital with a suspected acute myocardial infarction (AMI). Emergent coronary angiography revealed a totally occluded proximal left circumflex artery (LCX). During primary percutaneous coronary intervention, his blood pressure suddenly fell within seconds, and he developed pulseless electrical activity (PEA). Surprisingly, the 12-lead electrocardiogram (ECG) findings including the heart rate remained unchanged before and after the PEA, but a heart rate reduction and asystole occurred a few minutes after developing PEA. After tracheal intubation and mechanical assistance by venoarterial extracorporeal membrane oxygenation (VA-ECMO), the sudden onset of PEA appeared to be caused by cardiac tamponade due to a blowout-type left ventricular free wall rupture (BO-LVFWR) diagnosed by transthoracic echocardiography. While pericardiocentesis was performed and the drained blood was directly continuously perfused intravenously to keep the VA-ECMO flow, the patient was moved to the operation room. The surgical findings revealed a solitary BO-LVFWR due to a lateral AMI, and a direct closure was performed. Successful perioperative management, oral medication administration, and rehabilitation lead to the patient being transferred to a rehabilitation hospital without any serious cerebral damage. This case report suggested the detailed onset pattern of a BO-LVFWR followed by a rapid diagnosis by echocardiography and lifesaving treatment."}, {"id": "pubmed23n0046_13457", "title": "[Doppler echocardiography in the diagnosis of mechanic complications of acute myocardial infarction].", "score": 0.013475499092558983, "content": "To evaluate the ability of bedside emergency Doppler/Echocardiographic (ECOCG/DP) studies in the diagnosis of mechanic complications during acute myocardial infarction (AMI). Retrospective analysis of 44 fatal AMI cases, studied by ECOCG/DP and with diagnostic confirmation by surgery and/or necropsy. Patients (pts) with AMI admitted to an Intensive Care Unit of a tertiary Hospital (UCIM), Hospital de Santa Maria. 44 fatal AMI cases were analysed (24 men and 20 women; mean age +/- SD: 72 +/- 9 years) and were divided in two groups according to Killip classification in Group 1 (III/IV): 35 pts and Group 2 (I/II): 9 pts. ECOCG/DP was performed in a routine basis at admission, using all standard views and by subcostal view when in an emergency scenario. In 20 pts with bad left ventricular function (LVF) (Group 1) at admission, ECOCG/DP monitoring showed that death was due to worsening of LVF, which was confirmed by necropsy. In the other 15 pts of this group, ECOCG/DP documented the clinical diagnosis of cardiac rupture (free wall: 4 pts; papillary muscle: 4 pts; interventricular septum: 7 pts) which was confirmed by surgery and/or necropsy. In the 9 pts of Group 2, ECOCG/DP disclosed, at admission, good LVF in all. In 5 pts there was a sudden worsening clinical status, and ECOCG/DP showed a severe pericardial effusion with right chambers collapse, highly suggestive of free wall rupture also confirmed at necropsy. In the other 4 pts, ECOCG/DP showed aggravation of wall motion abnormalities and of LVF without rupture, once again in agreement with necropsy. Five clinical cases are presented for illustration of this issue. In the 44 fatal AMI cases of our study there was complete agreement between the ECOCG/DP and necropsy studies. In AMI patients, ECOCG/DP monitoring can in a routine basis, evaluate wall motion abnormalities and LVF. In an emergency setting ECOCG/DP can diagnose all the mechanic complications with a great certainty."}, {"id": "wiki20220301en133_43048", "title": "Myocardial rupture", "score": 0.012375404530744335, "content": "Risk factors for rupture after an acute myocardial infarction include female gender, advanced age of the individual, first ischemic event, and a low body mass index. Other presenting signs associated with myocardial rupture include a pericardial friction rub, sluggish flow in the coronary artery after it is opened i.e. revascularized with an angioplasty, the left anterior descending artery being often the cause of the acute MI, and delay of revascularization greater than 2 hours. Diagnosis Due to the acute hemodynamic deterioration associated with myocardial rupture, the diagnosis is generally made based on physical examination, changes in the vital signs, and clinical suspicion. The diagnosis can be confirmed with echocardiography. The diagnosis is ultimately made at autopsy."}, {"id": "pubmed23n0390_21577", "title": "Review of ventricular rupture: key concepts and diagnostic tools for success.", "score": 0.011021374787466603, "content": "Although a rare complication of acute myocardial infarction (AMI), ventricular rupture is a serious event associated with significant mortality and morbidity. Patients normally present with hemodynamic instability, often in cardiogenic shock. Despite improvements in surgical techniques and diagnostic tools, post-myocardial infarction ventricular rupture remains a difficult therapeutic challenge. There are three categories of ventricular rupture: free wall rupture (FWR), ventricular septal rupture (VSR), and papillary muscle rupture (PWR). The incidence of FWR occurs following up to 10% of myocardial infarctions. VSR and PWR have a lower incidence of 1-2% and 0.5-5%, respectively. Patients often present with single-vessel coronary artery disease and usually do not have a positive history for a previous myocardial infarction. The incidence of post infarction angina in these patients is significantly greater than in patients without ventricular rupture. Delay in treatment and continued physical activity post infarction increases the risk of ventricular rupture. Diagnostic tools such as two-dimensional echocardiography and cardiac catheterization confirm the diagnosis of ventricular rupture in only 45-88% of cases. Knowledge of the disease progression is necessary to insure accurate and timely diagnosis. Due to the rapid deterioration of these patients, there is a 50-80% mortality rate within the first week if untreated. With surgical correction, patients can extend their 5-year survival rates to 65%. A good example of the complex course of ventricular rupture is the case of a 71-year-old patient at our institution. The patient presented in cardiogenic shock following an AMI. Preoperative diagnosis was unsuccessful in determining the extent of the ventricular rupture. The correct diagnosis was determined in the operating room, and both a mitral valve replacement and closure of a ventricular septal defect were completed. The patient was successfully treated with this difficult pathology."}, {"id": "pubmed23n0648_1127", "title": "Repair of ventricle free wall rupture after acute myocardial infarction: a case report.", "score": 0.009900990099009901, "content": "Acute myocardial infarction (AMI) may culminate in sudden death by ventricular fibrillation, cardiogenic shock, and cardiac rupture. We present a case of postinfarction rupture treated by direct closure and coronary artery bypass grafting after thrombolytic therapy. A 67-year-old woman with cardiac risk factors of hypertension, diabetes mellitus, and being post-menopausal was admitted complaining of chest pain and sweating. Thrombolytic therapy with streptokinase was started due to acute myocardial infarction. But, reperfusion criteria were not achieved. Echocardiography revealed a moderate pericardial effusion with mild right chamber collapse and pericardial thrombus. Cardiac catheterization revealed totally occluded left anterior descending (LAD) and circumflex coronary arteries. She was taken to the operating-room immediately. The pericardium was opened and a large amount of blood with thrombus was removed. Her hemodynamic indices improved immediately. There was active bleeding from multiple sites with a 4 mm rupture. Cardiopulmonary bypass was established. Direct closure of rupture was carried out. Reversed autogenous saphenous vein bypass grafts were placed to the LAD and second obtuse margin coronary arteries. Postoperative recovery was uneventful and she was discharged from hospital in good condition. She remained asymptomatic during first year following the surgery. This case demonstrates that left ventricular free wall rupture is not always fatal and that early diagnosis and emergency surgical therapy may be successful. The combination of surgical repair with revascularization should be considered, because 80% of patients who experience LVFWR have multivessel coronary artery disease."}, {"id": "pubmed23n0046_23193", "title": "[A case of acute right ventricular infarction and life-saving right ventricular assistance following emergency coronary revascularization and resection of a left ventricular aneurysm--discussion of indication and proper assist flow volume].", "score": 0.009900990099009901, "content": "Right ventricular assistance (RVA) using centrifugal pump in combination with IABP was used to treat a patient who was difficult to wean from a cardiopulmonary bypass following emergency coronary revascularization and resection of a ventricular aneurysm performed to treat acute right ventricular infarction due to a PTCA complication. After 131 hours of RVA at 3.2 to 4.8 l/min, it was possible to remove the pump. No heparin was administered during this time, changing the pump head twice, was used for 64 and 50 hour period, no thrombi were detected either time. After being weaned from RVA, the patient developed severe respiratory dysfunction, but on the 10th postoperative day (POD) IABP was weaned, and on the 13th POD the artificial respirator was withdrawn. The results of the postoperative cardiac catheterization were favorable, the patient was discharged on the 57th POD, and has returned to society at the present time. The indications for RVA include a central venous pressure &gt; 20 mmHg and a cardiac index &lt; 1.8 l/min/m2, and tissue perfusion pressure and general preoperative condition should severe as guides. The higher the assisted flow volume the more efficacious in relieving ventricular load, but, since there is a limit to how much the left ventricle and lungs can withstand, it should not exceed levels which ensure the maintainance of cardiac output and tissue perfusion pressure."}, {"id": "pubmed23n0870_9152", "title": "Intraprocedural left ventricular free wall rupture diagnosed by left ventriculogram in a patient with infero-posterior myocardial infarction and severe aortic stenosis.", "score": 0.00980392156862745, "content": "Left ventricular wall rupture remains a major lethal complication of acute myocardial infarction and hypertension is a well-known predisposing factor of cardiac rupture after myocardial infarction. An 87-year-old man was admitted to our hospital, diagnosed as acute myocardial infarction (AMI). The echocardiogram showed 0.67-cm(2) aortic valve, consistent with severe aortic stenosis (AS). A coronary angiography showed a chronic occlusion of the proximal left circumflex artery and a 99 % stenosis and thrombus in the mid right coronary artery. During percutaneous angioplasty of the latter, transient hypotension and bradycardia developed at the time of balloon inflation, and low doses of noradrenaline and etilefrine were intravenously administered as needed. The patient suddenly lost consciousness and developed electro-mechanical dissociation. Cardio-pulmonary resuscitation followed by insertion of an intra-aortic balloon pump (IABP) and percutaneous cardiopulmonary support were initiated. The echocardiogram revealed moderate pericardial effusion, though the site of free wall rupture was not distinctly visible. A left ventriculogram clearly showed an infero-posterior apical wall rupture. Surgical treatment was withheld because of the interim development of brain death. In this patient, who presented with severe AS, the administration of catecholamine to stabilize the blood pressure probably increased the intraventricular pressures considerably despite apparently normal measurements of the central aortic pressure. IABP, temporary pacemaker, or both are recommended instead of intravenous catecholamines for patients with AMI complicated with significant AS to stabilize hemodynamic function during angioplasty."}, {"id": "pubmed23n0530_16006", "title": "Delayed ventricular septal rupture after percutaneous coronary intervention in acute myocardial infarction.", "score": 0.009615384615384616, "content": "In the era before reperfusion therapy, ventricular septal rupture complicated 1approximate3% of acute myocardial infarctions (AMI) usually 3-5 days after onset. Studies have reported a positive correlation between the incidence of septal perforation and total occlusion of the coronary arteries. A 70-year old female patient was referred to the emergency room with the diagnosis of acute anterior myocardial infarction (MI) and recent cerebral infarction. The coronary angiogram showed a 90% stenosis at the mid-portion of the left anterior descending artery (LAD), and the lesion was successfully treated by percutaneous coronary intervention (PCI) with stent implantation. After PCI, the anterior wall motion improved on the follow-up echocardiogram. However, on the 20th hospital day, the patient condition deteriorated suddenly with pulmonary congestion. The echocardiography revealed a 1.3 cm ventricular septal defect at the apical septum with a left-to-right shunt. We report this rare case of delayed septal rupture in a patient with patent LAD after PCI and recovery of wall motion."}, {"id": "pubmed23n0075_14406", "title": "[A case report of right ventricular infarction clearly detected by transesophageal echocardiography].", "score": 0.009615384615384616, "content": "We reported a case of a 70 year-old woman who suffered from right ventricular infarction with cardiogenic shock, detected clearly by transesophageal echocardiography. On admission, her pulse rate was 31 bpm and her blood pressure was unobtainable. Conscious level was III-1-2 and she was cold and clammy. The ECG showed complete AV block with junctional escape rhythm at a rate of 31 bpm which required temporary pacing and ST elevation in leads II, III, a VF, V4R, V3R, V1. An echocardiogram showed akinesis of RV free wall and paradoxical septal motion. Transesophageal echocardiography was performed safely on the 5th hospital day and detected RV wall motion abnormality clearly. A Swan-Ganz catheter was inserted. Mean PCW was 12 mmHg. PA pressure was 19/11 mmHg. Mean RA pressure was 13 mmHg. Cardiac index was 1.33 l/min/m2. SvO2 was 54%. Volume loading, administration of dopamine, dobutamine and nitroprusside were started. Cardiac index increased to 1.88 l/min/m2, and SvO2 increased to 59%. On the 4th hospital day, mean RA pressure increased to 29 mmHg and PA pressure increased to 47/31 mmHg acutely. Endotracheal intubation was done and PEEP 6 cmH2O was used and mean RA pressure and PA pressure decreased. On the 6th hospital day, cardiac index increased 4.08 l/min/m2. Cardiac catheterization done two months after acute myocardial infarction showed 75% stenosis of the proximal right coronary artery."}, {"id": "pubmed23n0963_22274", "title": "Left ventricle pseudoaneurysm: Diagnosis by a new murmur.", "score": 0.009523809523809525, "content": "Incomplete rupture of the ventricle free wall can occur after myocardial infarction. This occurs when an organized thrombus and the pericardium seal the ventricular perforation. This can progress to the formation of a left ventricle pseudoaneurysm (LVPA). A 70-year-old male with an antero-septal ST-elevation myocardial infarction (STEMI) underwent an emergent left heart catheterization which revealed severe three-vessel disease with occluded grafts, non-amenable to re-vascularization, and an apical thrombus. As he was high-risk for repeat coronary artery bypass graft, he was medically managed. Transthoracic echocardiogram (TTE) showed a normal left ventricle ejection fraction (LVEF), apical anterior and inferior wall akinesis, moderate sized apical thrombus, and pericardial thickening. On hospital day 7, examination revealed a new 3/6 to-and-fro murmur that was loudest at the apex. The patient was asymptomatic with normal vital signs. A repeat TTE revealed an apical wall rupture with flow into the pericardial cavity and absence of the apical thrombus. A LVPA was diagnosed and the patient was immediately referred for surgical repair. This case illustrates the potential for developing LVPA in STEMI patients and the importance of physical examination. If identified early a potential emergent situation in a previously asymptomatic patient can be averted, thereby preventing fatal consequences. &lt;<bLearning objective:</b With the growing use of diagnostic testing the importance of physical examination is being lost. However, with an astute cardiac examination, potential complications such as a left ventricular pseudoaneurysm can be identified and promptly managed. In addition, a ventricular pseudoaneurysm must be considered in the differential as a rare complication in post ST-elevation myocardial infarction patients with a new murmur.&gt;."}, {"id": "pubmed23n0646_4190", "title": "No fate but what we make: a case of full recovery after out-of-hospital cardiac arrest.", "score": 0.009523809523809525, "content": "An 80 years old man suffered a cardiac arrest shortly after arrival to his local health department. Basic Life Support was started promptly and nine minutes later, on evaluation by an Advanced Life Support team, the victim was defibrillated with a 200J shock. When orotracheal intubation was attempted, masseter muscle contraction was noticed: on reevaluation, the victim had pulse and spontaneous breathing.Thirty minutes later, the patient had been transferred to an emergency department. As he complained of chest pain, the ECG showed a ST segment depression in leads V4 to V6 and laboratory tests showed cardiac troponine I slightly elevated. A coronary angiography was performed urgently: significant left main plus three vessel coronary artery disease was disclosed.Eighteen hours after the cardiac arrest, a quadruple coronary artery bypass grafting operation was undertaken. During surgery, a fresh thrombus was removed from the middle left anterior descendent artery. Post-operative course was uneventful and the patient was discharged seven days after the procedure. Twenty four months later, he remains asymptomatic.In this case, the immediate call for the Advanced Life Support team, prompt basic life support and the successful defibrillation, altogether, contributed for the full recovery. Furthermore, the swiftness in the detection and treatment of the acute reversible cause (myocardial ischemia in this case) was crucial for long-term prognosis."}, {"id": "pubmed23n0497_21555", "title": "Ventricular septal rupture after early successful thrombolytic therapy in acute myocardial infarction: a case report.", "score": 0.009433962264150943, "content": "Ventricular septal defect (VSD) is a severe complication of acute myocardial infarction and has a high mortality rate. This complication appears to have declined in the reperfusion era. It has mostly been reported in elderly or female patients who suffer from anterior wall infarction, patients with multivessel coronary artery disease (CAD) or occluded infarct-related artery (IRA) without collateral circulation, or patients who have had delayed reperfusion therapy. Here, we report the case of a 60-year-old male patient who presented with persistent chest pain and Killip I ST-segment-elevation myocardial infarction. Thrombolytic therapy was started 3 hours after the onset of chest pain. Based on the subsidence of chest pain, resolution of the elevated ST segment, and early peak of cardiac enzymes, reperfusion was thought to be successful. However, on the third day of admission, the patient complained of dyspnea after defecation and was found to have new-onset grade 3 pansystolic murmur over the left sternal border. Cardiac echography showed an apical VSD. A Swan-Ganz catheter was inserted into the right side of the heart; analysis of blood oxygen saturation revealed a 6% step-up of oxygen in the right ventricle. Coronary angiography showed only one-vessel CAD and TIMI 3 flow in the IRA. The patient received intensive medical management and underwent VSD repair and internal mammary artery bypass grafting to the left anterior descending artery. His recovery was uneventful. This case illustrates that VSD can be found in patients receiving early successful reperfusion therapy, with one-vessel CAD, and TIMI 3 flow in the IRA."}, {"id": "pubmed23n0754_4961", "title": "Delayed ventricular septal rupture complicating acute inferior wall myocardial infarction.", "score": 0.009345794392523364, "content": "Ventricular septal rupture is a potentially fatal complication of acute myocardial infarction. Its incidence has declined with modern reperfusion therapy. In the era of percutaneous coronary interventions, it occurs a median of 18-24 hours after myocardial infarction and is most commonly associated with anterior myocardial infarction. We present a case of delayed ventricular septal rupture complicating acute inferior wall myocardial infarction. A 53-year-old Caucasian male presented with epigastric pain for three days and electrocardiographic evidence for an acute inferior wall myocardial infarction. Coronary angiography revealed a total occlusion of the proximal right coronary artery. Reperfusion was achieved by balloon angioplasty followed by placement of a bare metal stent. On hospital day six, the patient developed acute respiratory distress, a new loud pansystolic murmur, and hemodynamic instability. Echocardiography revealed the presence of a large defect in the inferobasal interventricular septum with significant left-to-right shunt consistent with ventricular septal rupture. The patient underwent emergent surgical repair with a bovine pericardial patch. Ventricular septal rupture after myocardial infarction should be suspected in the presence of new physical findings and hemodynamic compromise regardless of revascularization therapy."}, {"id": "pubmed23n0799_5052", "title": "[Surgical therapy of myocardial infarction].", "score": 0.009345794392523364, "content": "Coronary artery bypass grafting (CABG) has been replaced by percutaneous coronary interventions in the treatment of myocardial infarction (MI) nowadays. The surgical repair is the only option for mechanical complications of MI. The aim of our study was to assess the results of surgical treatment of MI. From January 2008 to December 2012 one thousand nine hundred fifty nine patients were operated on at Centre of cardiovascular surgery and transplantations in Brno for coronary artery disease, 103 (5.3 %) of them suffered from acute MI. The interval between MI and operations was longer than 24 hours in more than half of the patients. Nineteen patients underwent PCI before operation, 32 were in cardiogenic shock with intraaortic balloon pump in 12, twelve patients were after cardiopulmonary resuscitation and 18 were on ventilation. CABG alone was performed in 78 patients, in 25 patients mechanical complication of MI occurred; rupture of papillary muscle with mitral regurgitation in 8, rupture of interventricular septum in 11, rupture of free wall of left ventricle in 1 and evolving aneurysm of left ventricle in 5 patients. Several serious complications occurred in the postoperative period; disturbances of heart rhythm, syndrome of low cardiac output and pulmonary complications with the necessity of prolonged ventilation being the most frequent. Fourteen patients died during hospital stay (mortality 13.4 %). Patients after acute MI create the highest-risk group for surgical treatment. The reasons comprise serious preoperative status, delayed re-perfusion of ischemic area and serious hemodynamic effect of mechanical complications of MI. A lot of complications may occur during postoperative course and mortality is high. In the survivals the long term follow-up is promising."}, {"id": "wiki20220301en133_43052", "title": "Myocardial rupture", "score": 0.009324059986959357, "content": "Prognosis The prognosis of myocardial rupture is dependent on a number of factors, including which portion of the myocardium is involved in the rupture. In one case series, if myocardial rupture involved the free wall of the left ventricle, the mortality rate was 100.0%. The chances of survival rise dramatically if the patient: 1. has a witnessed initial event; 2. seeks early medical attention; 3. has an accurate diagnosis by the emergentologist; and 4. happens to be at a facility that has a cardiac surgery service (by whom a quick repair of the rupture can be attempted). Even if the individual survives the initial hemodynamic sequelae of the rupture, the 30‑day mortality is still significantly higher than if rupture did not occur."}, {"id": "pubmed23n0891_21095", "title": "Respiratory and Cardiac Characteristics of ICU Patients Aged 90 Years and Older: A Report of 12 Cases.", "score": 0.009259259259259259, "content": "Objective To investigate the respiratory and cardiac characteristics of elderly Intensive Care Unit (ICU) patients.Methods Twelve senior ICU patients aged 90 years and older were enrolled in this study. We retrospectively collected all patients' clinical data through medical record review. The basic demographics, primary cause for admission, the condition of respiratory and circulatory support, as well as prognosis were recorded. Shock patients and pneumonia patients were specifically analyzed in terms of clinical manifestations, laboratory variables, echocardiography, and lung ultrasound Results.Results The mean age of the included patients was 95 years with a male predominance (8 to 4, 66.7%). Regarding the reasons for admission, 6 (50.0%) patients had respiratory failure, 1 (8.3%) patient had shock, while 5 (41.7%) patients had both respiratory failure and shock. Of the 6 patients who suffered from shock, only 1 was diagnosed with distributive shock, 5 with cardiogenic shock. Of the 5 cardiogenic shock patients, 1 was diagnosed with acute coronary syndrome. The rest 4 cardiogenic shock patients were diagnosed with Takotsubo cardiomyopathy. The patient with ST-segment elevation myocardial infarction died within 24 hours. Of the 4 Takotsubo patients, 1 died on day-6 and the other 3 patients were transferred to ward after heart function recovered in 1 to 2 weeks. Of the 10 pneumonia patients, 3 were diagnosed as community acquired pneumonia, and 7 as hospital acquired pneumonia. Only 3 patients were successfully weaned from ventilator. The others required long-term ventilation complicated with heart failure, mostly with diastolic heart failure. Lung ultrasound of 6 patients with diastolic dysfunction showed bilateral B-lines during spontaneous breathing trial.Conclusions Elderly patients in shock tend to develop Takotsubo cardiomyopathy. Diastolic heart dysfunction might be a major contributor to difficult weaning from ventilator in elderly patients. Bedside lung ultrasonography and echocardiography could help decide the actual cause of respiratory failure and shock more accurately and effectively."}, {"id": "pubmed23n0814_12759", "title": "Subacute myocardial rupture following tirofiban treatment.", "score": 0.009174311926605505, "content": "A 74-year-old male patient was admitted to our emergency department with post-MI angina. On account of the anginal complaint that continued for three days, a coronary artery angiography was undertaken. A percutaneous transluminal coronary angioplasty was performed, followed by the implantation of a coronary stent, and coronary perfusion (TIMI-3) was achieved in the left anterior descending artery. Medical treatment (with acetylsalicylic acid, clopidogrel, metoprolol, atorvastatin and enoxaparine) and tirofiban infusion were duly administered in the coronary care unit. After twenty-four hours, however, acute dyspne, hypotension and tachycardia developed, making it necessary to perform an echocardiography. Since the echocardiography revealed a frank pericardial effusion, the patient was immediately taken to the operation room. The ventricular free wall rupture was repaired with Surgicel, which was prepared in three layers and fixed to the myocardium by tissue glue; cardiopulmonary bypass was not used. To our knowledge, our study constitutes the first case report of a tirofiban-induced free wall rupture. "}, {"id": "pubmed23n0369_495", "title": "[Isolated coronary bypass operation in the 9th decade of life].", "score": 0.009174311926605505, "content": "The average age of patients undergoing cardiac surgery has increased continuously during the last three decades due to a progressively increasing number of older people in the population and the advances in operative and perioperative treatment in open heart surgery. Consequently we have investigated the short- and long-term results of isolated myocardial revascularization in patients who are in their ninth decade of life. Between 1 January 1995 and 31 December 1998, 121 patients (51 women, 70 men, age 80 to 88 years, median: 82 years) underwent isolated coronary artery bypass grafting. As part of the revascularization, a unilateral internal mammary artery graft (IMA) was used in 87% of cases. The in-hospital mortality was 6.6%. Analysis of predictors of mortality unveiled the following factors: ejection fraction less than 50%; history of recent left ventricular failure; extent of coronary artery disease; perioperative use of an intraaortic balloon pump (IABP) and symptomatic pericardial effusion. Use of the IMA revealed no influence on in-hospital mortality. The median follow-up time was 20 months (range: 2-48 months). Survival rates after 1, 2, and 3 years were 93.1%, 87.3% and 73.7% for women and 86.9%, 82.5% and 65.1% for men. These survival rates were comparable with those of the entire 82 year old population. Predictors for late death were male gender, history of stroke, history of arterial embolism, and postoperative pulmonary failure resulting in mechanical ventilation. During the follow-up period myocardial infarcts were subsequently not observed. Freedom from angina after 1, 2 and 3 years was 90.1%, 82.6% and 78.1%, respectively. At an interval of 1 year after the operation 87.6% of patients had not been hospitalized as a result of cardiac disorders (2 years: 80.1%, 3 years: 73.2%). Permanent nursing care was not required 1 year after the operation by 94.3% of patients (2 years: 91.5%, 3 years: 91.5%). Four percent of the survivors suffered from permanent delirium, 3% from depression, 5% from lack of concentration, and 6% from vertigo. In summary this study has revealed that, in patients over eighty years of age suffering from ischemic heart disease, coronary artery bypass grafting has acceptable short- and long-term results. Yearly mortality rates during the first 3 years after the operation are comparable with the expected mortality rate in an age-matched population."}, {"id": "pubmed23n0083_2933", "title": "[Acute cardiac rupture in myocardial infarction. A case report].", "score": 0.00909090909090909, "content": "A 60 year old woman with a large anterior wall myocardial infarction developed severe hypotension 12 hr after admission to the coronary care unit. X rays showed an enlarged cardiac shadow and echocardiography signs of pericardial effusion. Swan Ganz catheterization revealed severe venous hypertension and no suggestion of ventricular septal rupture. Emergency surgery, initiated with partial cardiopulmonary bypass, showed a 1 cm tear of the anterior wall of the left ventricle, close to the left anterior descending artery. A successful repair was obtained by suture on teflon pledgets. After a difficult postoperative course, the patient was doing well 8 months after surgery."}, {"id": "pubmed23n0528_14276", "title": "[The results of the treatment of right ventricle myocardial infarction].", "score": 0.00909090909090909, "content": "To present the results and experience in diagnosing and treating of patients with acute right ventricle infarction, during the period of hospitalization of one month, with adjuvant analyses of the obtained results in the period of fifteen years. Acute right ventricle infarction porved clinicaly, enzymologicaly, by ECG, echochardiographically or scintigraphically we treated with thrombolitic therapy within first six hours after admittion, with salvaged PTA in case of the cardiogenic shock or AV block II degrees-III degrees despite of thrommbolitic therapy, or with postponed PTA within first month of intrahospital treatment. In the period from 1990 to 2004, 3 225 patients of both sexes were treated for acute myocardial infarction at the different localization in patients' at the mean age of 53.7 +/- 5.8. One-hundred-thirty-nine (43.9%) patients were treated with thrombolitic therapy according to the speed up protocole. Heparin was administered to 160 (50.7%) patients with water load, and 17 (5.4%) patients had the primary percutaneous transluminal coronary angioplasty (PPTCA), so that the mechanical blood flow could be established, by the implantation of a stent when necessary. In 316 patients with right ventricle infarction, 58 (18.3%) had postponed and salvaged percutaneous transluminal coronary angioplasty (PTCA). Twenty-two (15.8%) patients had thrombolitic therapy, whereas 36 (22.5%) patients were treated with heparin. We had a successful balloon dilatation in 21 (36.2%), whereas 32 (55.2%) patients had 1-3 intracoronary stents inplanted, depending upon the necessity, and 5 (8.6%) patients from this group were sent to surgical intervention. In the group of 214 (67.7%) patients treated with heparin or thrombolitic therapy combined therapy, with PTCA, 12 (5.7%) patients died, whereas in the group of 124 (39.3%) patients treated only with heparin 26 (16.2%) patients died, statistically significant difference (p &lt; 0.001, chi2 = 18.423). Was noticed n the group of 1 204 patients with inferoposterior infarction, 122 (10.1%) patients died. In the group of 316 patients with right ventricle infarction, 38 (12%) died. In the group of 888 control patients with inferoposterior infarction, but without right ventricle infarction, 84 (9.4%) patients died. In the group of 2 021 patients (62.2%) with anterior infarction, 248 (12.3%) died. CONCLUSION. The obtained results showed that the patients with right ventricle infarction, due to the great expansion of necrosis and the involvement of the inferoposterior wall of the left ventricle, as well as the ischemia of sinus and AV nodes, were the patients of a high risk. That was why it was essential to do urgent widening of the artery to reestablish blood flow either by using drugs or by means of mechanical methods."}, {"id": "wiki20220301en129_21256", "title": "Takotsubo cardiomyopathy", "score": 0.009015594541910331, "content": "Furthermore, mechanical circulatory support (MCS) with an intra-aortic balloon pump (IABP) is well-established as supportive treatment. Prognosis Despite the grave initial presentation in some of the patients, most of the patients survive the initial acute event, with a very low rate of in-hospital mortality or complications. Once a patient has recovered from the acute stage of the syndrome, they can expect a favorable outcome and the long-term prognosis is excellent for most. Even when ventricular systolic function is heavily compromised at presentation, it typically improves within the first few days and normalises within the first few months. Although infrequent, recurrence of the syndrome has been reported and seems to be associated with the nature of the trigger. Stress cardiomyopathy is now a well-recognized cause of acute congestive heart failure, lethal abnormal heart rhythms, and rupture of the heart wall."}, {"id": "pubmed23n0228_16173", "title": "Subacute left ventricular free wall rupture following acute myocardial infarction: bedside hemodynamics, differential diagnosis, and treatment.", "score": 0.009009009009009009, "content": "Six patients with subacute left ventricular free wall rupture (anatomically proved) following acute myocardial infarction are presented. Diagnosis of cardiac rupture in every case was suspected several hours before death or surgical intervention, when clinical and hemodynamic data of cardiac tamponade were found. In three patients right atrial pressure decreased with inspiration and in the other three cases it did not show any modification. These latter three patients had associated right ventricular infarction; the abnormal respiratory behavior could be explained by restriction produced by a noncompliant right ventricle. All six patients improved initially with medical treatment (inotropics and fluid infusion) and three of them were operated upon. One of the latter patients died on the eighteenth postoperative day of extracardiac causes and two are long-term survivors."}, {"id": "pubmed23n0739_21064", "title": "Timely diagnosis of left ventricular posterior wall rupture by echocardiography: a case report.", "score": 0.008928571428571428, "content": "Left ventricular free wall rupture is responsible for up to 10% of in-hospital deaths following myocardial infarction. It is mainly associated with posterolateral myocardial infarction, and its antemortem diagnosis is rarely made.One of the medical complications of myocardial infarction is the rupture of the free wall, which occurs more frequently in the anterolateral wall in hypertensives, women, and those with relatively large transmural myocardial infarction usually 1-4 days after myocardial infarction.We herein present the case of a 66-year-old man suffering inferior wall myocardial infarction with abrupt hemodynamic decompensation 9 days after myocardial infarction. Emergent transthoracic echocardiography revealed massive pericardial effusion with tamponade, containing a large elongated mass measuring 1 × 8cm suggestive of hematoma secondary to cardiac rupture. In urgent cardiac surgery, the posterior wall between the left coronary artery branches was ruptured."}, {"id": "pubmed23n0220_14320", "title": "[Prognosis in complications of acute myocardial infarction requiring artificial respiration].", "score": 0.008928571428571428, "content": "From 1978 to 1981, 818 consecutive patients with acute myocardial infarction were admitted, 112 (13.7%) of whom required artificial ventilation because of complications. Their mean age (62) corresponded to the mean age of all acute myocardial infarction patients (63). 28 (25%) survived the hospitalization and were followed after discharge. 2 were lost to follow-up. After a mean follow-up period of 26 months, 8 patients had died and 18 were still alive, none of them free of symptoms. There was no difference of age, duration of respirator therapy and maximal creatine kinase activity between survivors (group A) and nonsurvivors (group B). In 50% of patients cardiac failure leading to endotracheal intubation was triggered or made worse by arrhythmias. The remaining 50% of patients showed pure pump failure. Again in these two subsets, cardiac failure was significantly less marked in group A than in group B according to the hemodynamic findings. In conclusion, inpatient mortality in patients with acute myocardial infarction requiring artificial ventilation was high (75%) and hemodynamic findings were significantly worse in those not surviving. Patients discharged from the hospital also had a reduced life expectancy (less than 50% after 3 years)."}, {"id": "pubmed23n0086_15177", "title": "[Surgical treatment of ventricular septal rupture following myocardial infarction in an aged patient with bronchial asthma].", "score": 0.008849557522123894, "content": "Urgent surgery for ventricular septal rupture following myocardial infarction in a 75-year-old female with bronchial asthma was successfully performed. On Feb 28, 1988, she had chest pain, and was admitted 5 days later because of the appearance of heart murmur. Pansytolic murmur (Levine 4/VI) on 3 LSB and piping sound on both lung fields was heard, ECG showed acute anteroseptal infarction. Right heart Swan-Ganz catheterization revealed left to right shunt, and the diagnosis was ventricular septal rupture following acute anteroseptal infarction with bronchial asthma. The initial hemodynamic condition was not serious, but soon after the diagnosis was confirmed, IABP was inserted and operation was indicated because of the advanced age, high shunt ratio (70%) and complication of bronchial asthma. The operation was performed a day after septal rupture. The perforation in the ventricular septum of the apex was sutured with a xenopericardium patch by mattres sutures through a left ventricle approach, and the ventricular wall was closed with this patch together. The postoperative course was uneventful, and the patient was discharged on the 43rd day after the operation."}, {"id": "pubmed23n0050_9131", "title": "[A successful repair of concomitant rupture of the interventricular septum and left ventricular free wall after acute myocardial infarction].", "score": 0.008849557522123894, "content": "A 74-year-old woman had acute anteroseptal myocardial infarction. Tissue plasminogen activator (t-PA) was infused intravenously about five hours later from the onset of myocardial infarction. Six hours after the infusion of t-PA her blood pressure fell suddenly with the appearance of a grade 3/6 holosystolic murmur. There was a prominent step up of oxygen saturation in the right ventricle which indicated the presence of a left-to-right shunt. Intraaortic balloon pumping for the support of cardiac function was ineffective. At the emergent operation concomitant rupture of the left ventricular free wall and interventricular septum was seen and successfully repaired. She could be weaned from cardiopulmonary bypass easily with the aid of the intraaortic balloon pumping. Her postoperative course was uneventful. The postoperative angiography showed good left ventricular wall motion without any residual shunt."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[326, 394]], "word_ranges": [[46, 58]], "text": "Sodium heparin is reserved for cases in which the patient is stable."}, "2": {"exist": true, "char_ranges": [[395, 647]], "word_ranges": [[58, 95]], "text": "Thromboendarterectomy could be performed urgently in selected centers (not available in all) in patients in whom systemic fibrinolysis is contraindicated, or in centers where the experience with this technique is proven and it can be performed quickly."}, "3": {"exist": true, "char_ranges": [[0, 325]], "word_ranges": [[0, 46]], "text": "The patient presented pulmonary thromboembolism which, in addition to being bilateral, produced severe hemodynamic involvement, shock and required mechanical ventilation. We are not told that he has any contraindication to fibrinolysis, so this would be the most appropriate option due to its rapid administration and action."}, "4": {"exist": true, "char_ranges": [[648, 842]], "word_ranges": [[95, 125]], "text": "The inferior vena cava filter is a treatment indicated in the acute phase in stable patients, when anticoagulation is contraindicated; or a posteriori, as prophylaxis, in this group of patients."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The patient presented pulmonary thromboembolism which, in addition to being bilateral, produced severe hemodynamic involvement, shock and required mechanical ventilation. We are not told that he has any contraindication to fibrinolysis, so this would be the most appropriate option due to its rapid administration and action. Sodium heparin is reserved for cases in which the patient is stable. Thromboendarterectomy could be performed urgently in selected centers (not available in all) in patients in whom systemic fibrinolysis is contraindicated, or in centers where the experience with this technique is proven and it can be performed quickly. The inferior vena cava filter is a treatment indicated in the acute phase in stable patients, when anticoagulation is contraindicated; or a posteriori, as prophylaxis, in this group of patients. The indications for fibrinolysis in the last SEPAR consensus document included patients at intermediate risk (they could benefit from it) and high risk. They classify intermediate risk as PESIs ≥ 1 or PESI III-IV. In this subgroup, patients with right ventricular dysfunction, troponin or BNP above the cutoff and deep vein thrombosis appear to benefit most from fibrinolysis. In the subgroup of high-risk patients (defined by hypotension or cardiogenic shock criteria), the use of systemic fibrinolysis is much clearer, as in the case presented in the question.", "full_answer_no_ref": "The patient presented pulmonary thromboembolism which, in addition to being bilateral, produced severe hemodynamic involvement, shock and required mechanical ventilation. We are not told that he has any contraindication to fibrinolysis, so [HIDDEN] due to its rapid administration and action. Sodium heparin is reserved for cases in which the patient is stable. Thromboendarterectomy could be performed urgently in selected centers (not available in all) in patients in whom systemic fibrinolysis is contraindicated, or in centers where the experience with this technique is proven and it can be performed quickly. The inferior vena cava filter is a treatment indicated in the acute phase in stable patients, when anticoagulation is contraindicated; or a posteriori, as prophylaxis, in this group of patients. The indications for fibrinolysis in the last SEPAR consensus document included patients at intermediate risk (they could benefit from it) and high risk. They classify intermediate risk as PESIs ≥ 1 or PESI III-IV. In this subgroup, patients with right ventricular dysfunction, troponin or BNP above the cutoff and deep vein thrombosis appear to benefit most from fibrinolysis. In the subgroup of high-risk patients (defined by hypotension or cardiogenic shock criteria), the use of systemic fibrinolysis is much clearer, [HIDDEN].", "full_question": "A 58-year-old man, three weeks after a severe ankle sprain presents, rapidly progressive, with dyspnea at rest, dizziness and syncope. On arrival at the hospital he has hypotension (systolic BP 80 mmHg, diastolic 40 mmHg) and poor perfusion. He is intubated and connected to mechanical ventilation and noradrenaline is started. Echocardiogram shows signs of pulmonary hypertension. Angio-CT shows multiple repletion defects occupying both main pulmonary arteries. Which of the following treatments would be associated with the most rapid hemodynamic improvement in this case?", "id": 464, "lang": "en", "options": {"1": "Intravenous perfused sodium heparin.", "2": "Thromboendartectomy.", "3": "Systemic fibrinolysis with rt-PA (alteplase) 100 mg intravenous.", "4": "Inferior vena cava filter.", "5": NaN}, "question_id_specific": 155, "type": "CRITICAL CARE AND EMERGENCIES", "year": 2019, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0391_7921", "title": "Mechanical and enzymatic thrombolysis for massive pulmonary embolism.", "score": 0.018162393162393164, "content": "To assess the efficacy and safety of mechanical fragmentation combined with intrapulmonary thrombolysis in massive pulmonary thromboembolism (PTE) with hemodynamic impairment. Fifty-nine patients diagnosed with massive PTE with hemodynamic impact were treated. The initial clinical symptoms were shock in 23 patients (38.9%), syncope in eight (13.5%), and dyspnea at rest in 28 (47.4%). Mean O2 saturation was 67.8%. Mean pulmonary artery pressure (PAP) was 42.1 mm Hg. During fragmentation, thrombolysis was administered in the form of a urokinase bolus of 200,000-500,000 U in 57 patients and 20 mg of recombinant tissue plasminogen activator (rt-PA) in two patients. The mean urokinase dose used was 2,500,000 IU, whereas the total dose of rt-PA was 100 mg. Heparin sodium infusion was performed to reach activated partial thromboplastin time ratios of 2. The follow-up consisted of clinical assessment, pulmonary scintigraphy, and echocardiography. The patients received treatment with dicoumarin for 6 months after the procedure. Clinical improvement was seen in 56 patients (94%). Three patients died. The mean PAP after the treatment was 21.8 mm Hg. The mean posttreatment Miller index was 0.35. Technical success was achieved in all cases and clinical symptoms improved in all cases except those in which the patients died. Pulmonary scintigraphy showed improved perfusion in all cases. Echocardiography was performed after 3-6 months, showing a mean pressure of 22.8 mm Hg (corrected values). There were no signs of recurrent PTE or arterial hypertension in the follow-up. The data provided confirm the efficacy and safety of mechanical fragmentation and pharmacologic thrombolysis in the treatment of massive PTE with hemodynamic impairment, showing improvement of symptoms and a decrease in PAP."}, {"id": "pubmed23n0260_210", "title": "[Acute pulmonary thromboembolism with severe hemodynamic compromise. The efficacy of systemic thrombolytic treatment in the coronary unit].", "score": 0.017185900248647065, "content": "A 79[correction of seventy]-year-old patient, who has been in bed a long time, suffered an episode of sudden dyspnea, tachycardia and tachypnea. An electrocardiogram registered at her admission in the coronary care unit showed a normal rhythm with right axis deviation and S1Q3T3 pattern and ST segment alterations. A ventilation-perfusion lung scanning demonstrated segmental perfusion defects with high probability of pulmonary embolism. She developed a low cardiac output syndrome, which neither responded to the volume expansion nor to the inotropic drugs. The bed-side hemodynamic measurements were a systolic pulmonary pressure of 60 mmHg, with a diastolic pressure of 18 mmHg and capillary wedge pressure of 13 mmHg with high pulmonary resistance. With this evidences, the diagnoses of massive pulmonary embolism was done, thrombolytic treatment was decided on. Two hundred and fifty thousands international units of streptokinase was administered, and then 100.000 UI/hour in 24 hours; after that with intravenous heparin. The low cardiac output syndrome disappeared. The patients recovered her systemic arterial pressure and her diuresis. The electrocardiographic signs vanished and both the pulmonary pressure and resistance decreased. We discussed the importance of intravenous thrombolytic treatment in massive pulmonary embolism. We concluded than this treatment is an useful strategy that not always needs a pulmonary arteriography, and could be used in low complexity centres, successfully in the massive pulmonary thromboembolism with severe hemodynamic damage."}, {"id": "pubmed23n0278_4599", "title": "[High doses and the rapid infusion of streptokinase for the treatment of massive pulmonary thromboembolism].", "score": 0.01562595373252762, "content": "We report the case of a 65 year old woman with no prior cardiac or pulmonary disease, who suffered pulmonary embolism (PE); diagnosis was made on the basis of the existence of risk factors, clinical, radiographic and electrocardiographic features, and a lung scan with perfusion defects and normal ventilation. PE was considered massive because the patient developed acute respiratory failure that required tracheal intubation and mechanical ventilation as well as obstructive shock, electrocardiographic and echocardiographic data of right ventricle overload, and pulmonary hypertension, with pulmonary artery pressure of 38 mmHg. She received an initial treatment with high doses (1,500,000 UI) and rapid infusion (1 hr) of intravenous streptokinase (SK) followed by heparin anticoagulation. Thereafter the hemodynamic disturbances improved and pulmonary artery pressure post-thrombolysis was 23 mmHg. In this report SK at high doses and rapid infusion showed effectiveness and security. We emphasize the usefulness of echocardiography as a diagnostic aid in patients with a previously healthy cardiopulmonary system, as well as the possible role of electrocardiogram as an early indicator of pulmonary reperfusion. This could be the first report of successful thrombolysis with high doses and rapid infusion of SK in massive PE."}, {"id": "pubmed23n1069_20364", "title": "Rapid dynamic bedside assessment of pulmonary perfusion defect by electrical impedance tomography in a patient with acute massive pulmonary embolism.", "score": 0.015443098123069612, "content": "Several animal studies have shown that regional lung perfusion could be effectively estimated by the hypertonic saline contrast electrical impedance tomography method. Here, we reported an application of this method to dynamically assess regional pulmonary perfusion defect in a patient with acute massive pulmonary embolism. A 68-year-old man experienced sudden dyspnea and cardiac arrest during out-of-bed physical activity on the first day after partial mediastinal tumor resection. Acute pulmonary embolism was suspected due to acute enlargement of right heart and fixed inferior venous cava measured with bedside ultrasound. The computed tomography pulmonary angiography further confirmed large embolism in both left and right main pulmonary arteries and branches. The regional time impedance curves, which were obtained by a bolus of 10 ml 10% NaCl through the central venous catheter, were then analyzed to quantitatively assess regional perfusion. Normal ventilation distribution with massive defects in regional perfusion in both lungs was observed, leading to a ventilation-perfusion mismatch and low oxygenation index (PaO<sub2</sub/FiO2 = 86 mmHg) at the first day of pulmonary embolism. The anticoagulation was performed with heparin, and the patient's condition (such as shock, dyspnea, hypoxemia, etc.), regional lung perfusion defect, and ventilation-perfusion mismatch continuously improved in the following days. In conclusion, this case implies that electrical impedance tomography might have the potential to assess and monitor regional perfusion for rapid diagnosis of fatal pulmonary embolism in clinical practice."}, {"id": "pubmed23n0945_14122", "title": "High-risk pulmonary embolism assessed by transthoracic echocardiography: A case report.", "score": 0.014493460862775719, "content": "Acute pulmonary embolism (APE) as a life-threatening illness may present with a wide range of manifestations. APE was diagnosed using computed tomographic pulmonary angiography (CTPA); however, transthoracic echocardiography (TTE) can reveal hemodynamic status. Early thrombolysis is the most effective therapy for the treatment of massive pulmonary embolism. Herein, we report a case of high-risk APE with a wide range of manifestations, including chest pain, dyspnea, low-blood pressure, and syncope. A 55-year-old, previously healthy woman, complained of dyspnea and pleuritic chest pain for 40 days, along with transitory (10 minutes) episodes of syncope that had occurred 2 days previously. Because of the high-risk APE, the patient received intravenous thrombolytic therapy with low-dose recombinant tissue plasminogen activator (rt-PA, 50 mg over 30 minutes) and an anticoagulant (subcutaneous low-molecular-weight heparin, once every 12 hours for 5 days). Five days after thrombolysis, bedside TTE revealed RV diastolic dimension decreased to 22 mm. Color ultrasonography revealed a significant decrease in systolic and mean pulmonary artery pressure. TTE may provide initial suspicion of APE and may help identify patients with unstable hemodynamic status before the onset of shock. Moreover, concomitant TTE signs of decreased RV load may predict better prognosis for high-risk APE patients."}, {"id": "pubmed23n0290_5299", "title": "[Pulmonary thromboembolism. A clinical case with unusual presentation].", "score": 0.009900990099009901, "content": "The authors describe a rare case of pulmonary thromboembolism with unusual clinical findings and emphasized the large difficulty encountered in formuling a correct diagnosis in a reasonable time. A man, 60 years old, was admitted to a Medical Division of our hospital for the appearance of chest pain and epigastric pain during effort in the last year. He smoked 20 cigarettes a day and drank wine (1 or 2 litres a day). He was affected by hypercholesterolemia and in the past reported relapsed thrombophlebitis in the left leg. Four years before admission to our hospital he underwent large and small left saphenectomy. He had no cardiac events in the past. After a non significant exercise stress test the patient was treated with nitrates and asa and was discharged from the hospital. At home the symptoms increased and after 8 months the patient was admitted again to the Cardiologic Division of the hospital. At admission he reported dyspnea and chest pain at rest, not only during effort and the ECG showed negative T waves in anterior and inferior leads. Intravenous heparine, nitrates and calcium antagonists stabilized the clinical picture. The following examinations revealed: reduction of the T wave negativity at the ECG registered during chest pain; mild enlargement of the heart at the chest roentgenogram; normal value of the left ventricle and apical and midseptal by ipokinesia at the transthoracic echocardiogram; normal coronary artery at the coronary arteriography. \"Vasospastic angina\" was diagnosed and the patient was discharged after 20 days, asymptomatic. After 15 days he returned to the hospital again for chest pain, dyspnea, hypotension and syncope despite therapy. At physical examination he showed a painful left tibio-tarsal tumefaction, an increased and splitting second heart sound in the pulmonary area and a systolic murmur in the third and fourth left interspace. The ECG showed a severe anterior ischemia, while a new transthoracic echocardiogram revealed a considerable dilatation of the right atrium, right ventricle and the main pulmonary artery with severe tricuspid regurgitation and pulmonary hypertension (mean PAP about 50 mmHg). The following pulmonary perfusion scintigraphy confirmed the diagnosis of pulmonary embolism and the selective right and left pulmonary arteriography exhibited multiple thrombi and large intravascular filling defects. The right heart catheterization confirmed a chronic precapillary pulmonary hypertension (mean PAP = 55 mmHg). About 24 hours after these examinations the patient died because of a cardiac arrest with electromechanical dissociation. Pulmonary thromboembolism is a potentially fatal disease characterized by a largely variable clinical presentation. Frequently pulmonary embolism diagnosis is difficult especially when clinical findings are unusual. In the case observed the \"typical\" chest and epigastric pains associated with the electrocardiographic findings directed diagnosis towards myocardial ischemia. Also after the coronary arteriography that showed normal coronary artery, the erroneous diagnosis persisted. Pulmonary embolism was correctly diagnosed too late to begin an effective therapy. These unusual clinical findings and diagnostic mistakes are stressed and critically reviewed in the article."}, {"id": "pubmed23n0920_6891", "title": "[A case of large pulmonary embolism in trunk and branches with main manifestation of syncope, vomiting and shock].", "score": 0.009900990099009901, "content": "Pulmonary embolism (PE) refers to the endogenous or exogenous emboli blocking pulmonary trunk or branches, causing clinical and pathophysiological syndrome of pulmonary circulation disorder, the incidence rate is high. Sometimes PE patients were lack of specific symptoms and signs, or without any symptoms, which often result in misdiagnosis, un-timely diagnosis, and the delay of treatment. A PE case with syncope, vomiting and shock, which was proved to be pulmonary artery trunk and branch wide embolism later, was presented so as to improve the understanding of the disease."}, {"id": "wiki20220301en170_30310", "title": "Anterior cerebral artery syndrome", "score": 0.00980392156862745, "content": "Management Pulse oximetry can guide the use of supplemental oxygen to maintain oxygen saturation greater than 94%. Hyperoxia should be avoided as may be detrimental in stroke. Hypertension is common in an acute ischemic stroke. A low BP is uncommon and may indicate symptoms exacerbation of a previous stroke due to poor perfusion. Blood pressure of 220/120 mmHg should receive treatment. There is a consensus approach of allowing permissive hypertension up to 220/120 mmHg for patients that are not candidates for thrombolysis.[21] However, for a patient that is a potential candidate for alteplase, attempt to control BP should be made immediately as goal BP for initiation of IV alteplase is 185/110 mmHg. Usually, titratable short-acting intravenous hypotensive agents are recommended to avoid dropping the BP too much once the patient is at goal. Hypotensive agents that can be options include labetalol, nicardipine, clevidipine, hydralazine, enalaprilat.[21]"}, {"id": "pubmed23n0678_4569", "title": "[Massive pulmonary thromboembolism treated successfully with streptokinase. Report of one case].", "score": 0.00980392156862745, "content": "Massive pulmonary thromboembolism has a high mortality. Early thrombolysis is the treatment of choice. We report a 79-year-old man admitted in shock. A chest angio-CAT scan showed a massive pulmonary thromboembolism. A transthoracic echocardiography showed a right cardiac dysfunction. Although the patient was in hemodynamic instability, he was subjected to thrombolysis with streptokinase, assisted with noradrenaline support and invasive mechanical ventilation. Parenteral anticoagulation was started thereafter. A second echocardiography, performed 72 hours later showed an improvement in right ventricular function. The patient had a nosocomial pneumonia that was treated. Noradrenalin and mechanical ventilation were discontinued nine and 15 days after thrombolysis. A new angio-CAT scan, 23 days after the procedure, was normal. The patient was discharged in good conditions 27 days after admission."}, {"id": "pubmed23n0366_18234", "title": "[A case of primary pulmonary artery myxosarcoma associated with severe pulmonary hypertension].", "score": 0.009708737864077669, "content": "A 50-year-old man presented with progressive dyspnea on exertion, but with no history of chest pain or syncope. Chronic pulmonary thromboembolism was suspected and he was referred to our hospital. On ausculation, a grade 3 systolic murmur was heard, that was loudest in the fifth intercostal space lateral to the right sternal border. Chest radiography showed mild cardiomegaly and ventilation-perfusion scan revealed absence of perfusion in the left lung and the upper field of the right lung. Contrast-enhanced helical CT showed large mural defects in both main pulmonary arteries, clearly delineated by contrast medium. The left pulmonary artery was nearly completely occluded, and eccentric defects were observed projecting into the lumen of the pulmonary trunk. A tumor originating in the pulmonary artery was suspected, but a definitive diagnosis of the mass could not be made with pulmonary angiography and magnetic resonance imaging. The mean pulmonary arterial pressure was 50 mmHg. Further radiologic examinations failed to reveal the source of the embolus or tumor. It was decided to attempt surgical excision under total cardiopulmonary bypass. At operation, a gelatinous, lustrous, yellowish mass was found partially occluding the right main pulmonary artery and completely occluding the left. The tumor adhered tightly to the intima of the vessel and was inoperable. The patient could not be weaned from percutaneous cardiopulmonary support and died 3 days after surgery. Histologic examination of the excised specimen revealed myxosarcoma."}, {"id": "pubmed23n0413_10604", "title": "[A case of successful pulmonary embolectomy for massive acute pulmonary thromboembolism].", "score": 0.009708737864077669, "content": "A 54-year-old man was admitted to our hospital complaining of sudden-onset dyspnea in shock. Chest computed tomography(CT) showed thrombi in the right main and left intermediate pulmonary arteries. The case was diagnosed as a massive acute pulmonary thromboembolism. Although his hemodynamic status was stable after catecholamine infusion, his dyspnea was still in progress. Emergency pulmonary embolectomy was performed and the life of patient was saved. It is thought that progressive dyspnea is an important sign of a deteriorating hemodynamic status and the predictive symptom indicating a surgical procedure in patients with massive acute pulmonary thromboembolism."}, {"id": "pubmed23n0476_8790", "title": "[A case of pulmonary thromboembolism due to idiopathic thrombosis of inferior vena cava, which was initially misdiagnosed as pneumonia].", "score": 0.009615384615384616, "content": "We report a case of a 73-year-old man with pulmonary embolism due to idiopathic thrombosis of the inferior vena cava. He was referred to our hospital because of a fever and cough of 2 weeks' duration despite treatment with an oral antibiotic. Chest radiography on the first visit showed an infiltrate in the right middle lung field. He was diagnosed as having pneumonia and admitted to our hospital for treatment. Following administration of intravenous antibiotics, his symptoms disappeared and the chest radiography findings improved. The abdominal CT obtained in an attempt to visualize the cause of liver dysfunction serendipitously revealed thrombosis of the inferior vena cava, which was suspected to have caused the pulmonary embolism. A subsequent lung perfusion scan revealed marked perfusion defects in the right middle and lower lobes. Chest CT revealed an embolus located in the right pulmonary artery. Since thrombolytic therapy was not effective, the placement of a filter in the inferior vena cava was performed to prevent the recurrence of pulmonary embolism. The patient has been asymptomatic without recurrence of the disease since the filter insertion."}, {"id": "pubmed23n0112_18030", "title": "[Differences in patients with chronic pulmonary embolism and primary pulmonary hypertension].", "score": 0.009523809523809525, "content": "Chronic pulmonary embolism is a rare disease which can occur at first with pulmonary hypertension. In these cases it may be difficult to distinguish between primary pulmonary hypertension. We examined nine patients with Chronic Pulmonary Embolism (CPE) (three females and six males, mean age 45 +/- 13 years, range 21-67 years) and ten patients with Primary Pulmonary Hypertension (PPH) (seven females and three males, mean age 35 +/- 13 years, range 10-56 years) who came to our attention during the years 1973-1986 (mean follow up 3 years). All patients had an electrocardiogram, chest x-ray, echocardiogram, cardiac catheterization with pulmonary angiography; seven patients with CPE and eight with PPH had perfusion lung scans. Progressive dyspnoea was the main feature in all the patients; four out of nine with CPE and none of the ones with PPH had a previous history of thrombophlebitis. In all the patients the electrocardiogram, chest x-ray and echocardiogram showed signs of pulmonary hypertension, so that a clear distinction between the two groups was not possible. Cardiac catheterization showed pulmonary pressure values higher in patients with PPH as compared to the ones with CPE (systolic pressure 96 mmHg vs 70 mmHg, diastolic pressure 49 mmHg vs 31 mmHg, mean pressure 65 mmHg vs 45 mmHg). Pulmonary angiography in more than half of the patients with CPE showed a \"cut off\" of two or more lobar branches of the pulmonary arteries. In the patients with PPH pulmonary angiography showed a dilatation of the main pulmonary artery and a diffuse bilateral hypoperfusion. Perfusion lung scan in all the cases of CPE showed zonal perfusion defects, while in all cases of PPH, with the exception of one, it was largely normal. Venograms in the districts of the inferior vena cava demonstrated thrombosis in two out of six patients with CPE. Negative venograms were found in the five patients with PPH who had this investigation performed. One patient with CPE had a surgical embolectomy, the other eight had anticoagulant oral treatment. During the follow-up period three patients with CPE and five with PPH died within five years and within fifteen months respectively, of the diagnosis.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0361_20929", "title": "[Management of serious pulmonary embolism].", "score": 0.009523809523809525, "content": "In-hospital mortality is high when pulmonary embolism is complicated by hemodynamic instability and/or pulmonary hypertension. Death occurs frequently within the first hours after admission. This implies specific diagnostic and therapeutic management. Spiral CT seems to be an excellent diagnostic procedure in this setting. However, pulmonary angiography and perfusion lung scan can also be employed. Cardiac echography can help in the diagnosis and therapeutic decision making. Supportive therapy mainly includes correction of hypovolemia if present, a limited volume loading in other cases, and the use of dobutamine. Norepinephrine is the drug of choice when hypotension is present. Thrombolytic agents are indicated in case of hemodynamic instability. Modalities of administration and contra indications are currently well established. Surgical embolectomy should be performed in cases of uncontrolled shock, when thrombolysis is contra-indicated or uneffective."}, {"id": "pubmed23n0356_4275", "title": "[Major pulmonary embolism].", "score": 0.009433962264150943, "content": "The diagnosis of major pulmonary embolism should be considered in case of acute respiratory distress, particularly when there is high thromboembolic risk. Although clinical symptoms are not specific, some are suggestive: syncope or dizziness with cyanosis and polypnoea, and especially arterial hypotension and cardiogenic shock. Diagnostic workup should be rapid and straight forward. Transthoracic echography is particularly useful to detect right heart thrombi and right ventricular overload. More information could be provided by helical computed tomography or perfusion lung scan or less commonly now by pulmonary angiography, depending on the patient's clinical condition and the available equipment. The mortality rate can reach 20 to 30%, and up to 65% after resuscitated cardiac arrest. Rapid desobstruction is justified through surgical embolectomy or intravenous thrombolysis favouring short duration protocols (alteplase over 2 h), in spite of the bleeding risk."}, {"id": "pubmed23n0256_16223", "title": "[A case of renal vein thrombosis and pulmonary embolism associated with diffuse membranous glomerulonephritis: the usefulness of low-molecular-weight heparin and urokinase therapy].", "score": 0.009345794392523364, "content": "We report a case of renal vein thrombosis (RVT) and pulmonary embolism associated with diffuse membranous glomerulonephritis. A 44-year-old Japanese male was referred to the Nephrology Department with heavy proteinuria. Renal biopsy revealed diffuse membranous glomerulonephritis and we administered PSL 30mg/day and dipyridamole 300mg/day. Three weeks later, he was admitted with severe chest pain, dyspnea and massive proteinuria. RVT and pulmonary embolism were detected on CT scan and perfusion lung scan. After a few days of continuous intravenous unfractionated heparin (UFH) therapy, we used 72 U (anti-FXa)/kg of intravenous low-molecular-weight heparin (LMWH) every 12 hours for 10 days. He also received urokinase at the dose of 120,000 U/day for 4 weeks and long-term therapy with warfarin potassium at the dose of 3 mg/day. One month later, the thrombi in the pulmonary arteries and inferior vena cava disappeared on CT scan and perfusion lung scan. LMWHs have a longer biological half-life and a lower bleeding tendency than UFH for an equivalent antithrombotic effect. This case indicates that intermittent intravenous LMWH administration combined with urokinase is effective against RVT and pulmonary embolism without any side effect."}, {"id": "pubmed23n0335_1047", "title": "[Thrombolysis in pulmonary embolism - initial experience].", "score": 0.009345794392523364, "content": "Pulmonary embolism (PE) is a clinical situation difficult to diagnose, at times of great clinical instability, above all when it is massive, which leads to difficulties in the approach and treatment of patients. The treatment has not had any major innovations in recent years, being conventional the use of heparin and more rarely embolectomy. Recently, some clinical trials have defended the use of thrombolytics. The objéctive of this paper is to present our experience, although the series is still small. From April 1996 to November 1997, 11 patients were admitted to our Cardiac Intensive Care Unit with the clinical suspicion of PE, 5 of which with great hemodynamic instability and suspicion of massive PE. The clinical presentation was sudden dyspnea and loss of consciousness in 2 patients, dyspnea and hypotension in 2 patients and shock and respiratory arrest in one case. Gasimetry revealed acute hypoxemia and hypocapnia in all cases, average partial blood pressure in O2 (pO2) of 59 mm Hg and CO2 (pCO2) of 19 mm Hg. ECC and thorax x-ray contributed to the diagnosis in 3 patients, transthoracic echocardiography was decisive for the diagnosis in 5 cases, with visualisation of the thrombus by transesophageal echocardiography in 3 patients. All patients were monitored by Swan-Ganz catheter, the average systolic pulmonary artery pressure (PAP) was 74 mm Hg. Thrombolysis with rTPA (10 mg bolus followed by 90 mg in perfusion in 2 hrs) was administered in 6 episodes in 5 patients. Only in the case of the patient in shock were other complications related to the use of thrombolytics namely high digestive hemorrhage. There was a clear clinical improvement in all cases with great relief of dyspnea reduction of cyanosis and jugular engurgitation. The patient in shock recovered systemic pressures and improved the hemodynamic state. A significant reduction in PAP was observed (average of 32.5 mm Hg). PE recurred in two cases: with one death and therapeutic thrombolytic was repeated in the other patient with good results. After discharge, all patients remained asymptomatic under oral anticoagulation. Despite this small series, the results favour the use of thrombolytics in PE with a clear clinical and hemodynamic improvement."}, {"id": "pubmed23n0627_23552", "title": "Pulmonary hypertension in patient with elevated homocystein level and blast injuries.", "score": 0.009259259259259259, "content": "38-year-old man had chronic deep venous thrombosis (DVT) as a result of multiple injuries caused by an explosion of grenade 12 years ago, with recurrent pulmonary thromboembolisms and pulmonary hypertension which was unrecognized for a decade. Patient was admitted with a progressive dyspnea and exercise intolerance (NYHA II). The diagnosis was established according to clinical symptoms, transthoracic echocardiography, phlebography, lung scintigraphy and pulmonary angiography. Oral anticoagulant therapy was introduced and cava filter indicated to implant. During phlebography a floating thrombus was found in the inferior cava vein underneath renal vein. Implantation was delayed and patient received systemic fibrinolytic therapy with streptokinase (7500 000 UI within 4 days), followed by heparin infusion and warfarin. Post-fibrinolytic phlebography showed clear lumen of inferior vena cava. Fibrinolysis had also affected pulmonary hypertension-systolic pressure in the right ventricle measured by Doppler echocardiography decreased from 90 to 65 mmHg. Permanent intravenous cava filter was implanted."}, {"id": "pubmed23n0305_10217", "title": "[Low doses of rtPA administered as a bolus in treatment of clinically acute massive pulmonary embolism].", "score": 0.009259259259259259, "content": "12 patients (7 male and 5 female) with confirmed pulmonary embolism (PE) with: angiography-5 cases, conventional contrast-enhanced CT-2 cases, echocardiography-2 cases, autopsy-3 cases were diagnosed as clinically acute PE. Criteria of clinically acute PE were: cardiac arrest-1 case-2 cases, shock-1 case, acute cor pulmonale-9 cases and acute cor pulmonale with shock. All patients were treated with heparin, administered with therapeutic prolongation of aPTT. Clinically acute PE (if possible confirmed with angiography, TC and/or echocardiography) was treated with rtPA administered in 10 minutes lasting bolus in doses 0.6-0.8 mg per kg of body weight (50 mg of rtPA during 10 minutes administered into peripheral veins). In 9 patients with pulmonary hypertension, significant decrease of tricuspidal gradient (measured echocardiographically during several hours after administration of rtPA) was documented. Improvement in PaO2, SaO2 and decrease of heart rate and respiratory rate were also achieved. No serious bleeding complications were observed after mentioned treatment. Control investigations (conventional contrast-enhanced CT and spiral CT) performed several days after rtPA administration revealed thrombus in pulmonary artery. We conclude: I rtPA administered in bolus simultaneously with heparin significantly decreased pulmonaryhypertension; rtPA administered simultaneously with heparin is safe method of treatment of PE; hemodynamic improvement after administration of rtPA is not univocal with full fibrynolitic effect."}, {"id": "pubmed23n0982_16661", "title": "Preoperative balloon pulmonary angioplasty enabled noncardiac surgery of a patient with chronic thromboembolic pulmonary hypertension (CTEPH): A case report.", "score": 0.009174311926605505, "content": "Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease with a poor prognosis, characterized by chronic thromboembolic obstruction of the pulmonary arteries and pulmonary hypertension. Balloon pulmonary angioplasty (BPA) is a newly emergent treatment for CTEPH, which may substitute pulmonary endarterectomy, the standard but more invasive treatment for CTEPH. Here, we report the case of a CTEPH patient who underwent 2 noncardiac surgeries without complications after preoperative intervention of BPA. A 79-year-old man presented with severe osteoarthritis of bilateral knees, with adaptation of total knee arthroplasty (TKA). Transthoracic echocardiogram revealed severe pulmonary hypertension with estimated right ventricular systolic pressure of 140 mm Hg. Pulmonary arteriography revealed total occlusion of the upper branch of the right pulmonary artery, and ventilation/perfusion scan showed multiple mismatched perfusion defects. His pulmonary artery pressure (PAP) was as high as 89/25 (46) mm Hg with normal range of pulmonary capillary wedge pressure. He was diagnosed with CTEPH. Four BPA sessions for 8 branches of the bilateral pulmonary arteries were done, until the mean PAP (mPAP) went under 30 mm Hg. For the TKA, we selected spinal anesthesia in order to minimize intraoperative hemodynamic changes. Cardiac surgeons were standby in case extracorporeal membrane oxygenation (ECMO) initiation was required. With appropriate pain management and use of intravenous vasopressors, intraoperative vital signs were stable. No symptoms of hemodynamic collapse were observed postoperatively. The patient was discharged on the 46th postoperative day following rehabilitation. Two years later, left-side unicompartment knee arthroplasty (UKA) was scheduled. Right heart catheterization study revealed the mPAP was 30 mm Hg, nearly the same value as the last study. The operation was performed under spinal anesthesia with continuous arterial pressure monitoring without need for intraoperative vasopressor. He was discharged without complications on the 24th postoperative day. BPA can be an effective preoperative intervention for CTEPH patients undergoing noncardiac surgery."}, {"id": "pubmed23n0265_12861", "title": "[A case of pulmonary thromboembolism with pulmonary hypertension with marked improvement by oral PGI2 analogue].", "score": 0.00909090909090909, "content": "The patient was a 71-year-old man with dyspnea and bilateral leg edema. He was admitted to our hospital with worsening of dyspnea and had received therapy for chronic heart failure in another hospital. On admission, chest X-ray film revealed dilatation of the cardiac silhouette (CTR: 58%). Electrocardiogram showed atrial fibrillation and negative T wave in II, III, and aVF. Cardiac arteriogram showed no organic lesions, but pulmonary hypertension, pulmonary artery pressure of 60/21/34 mmHg, right ventricular pressure of 40/9/20 mmHg were recognized in pulmonary hemodynamics. The diagnosis of chronic pulmonary thromboembolism was made on the basis of pulmonary arteriogram findings and multiple defects of lung perfusion scintigram. After administration of oral PGI2 analogue for one month, lung perfusion scintigram and right cardiac pressure were markedly improved."}, {"id": "pubmed23n0563_4336", "title": "[Characteristics of acute, hemodynamically stable pulmonary embolism presenting as a syncope].", "score": 0.00909090909090909, "content": "Syncope (S) occurs in approximately 10% patients with acute pulmonary embolism (APE) and is commonly ascribed to the massive, hemodynamically instable APE. The aim of the study was to assess the occurrence and significance of S revealing hemodynamically stable APE. We found syncope in 6 of 21 (29%) consecutive patients (16 females, 5 males; age from 46-87 years, mean age of 71 years) who were diagnosed with APE and in whom other reasons for S were excluded. All patients were treated with anticoagulation. They all survived hospitalization and no APE recurrences were found during in-hospital period. Patients with APE-S compared to patients with APE and without S had smaller baseline RVED (21.2 +/- 2 vs. 27.3 +/- 5.6, p = 0.01), however both groups did not differ statistically in baseline vital signs, angiographic, hemodynamic, other echocardiographic parameters as well as in the results of laboratory findings. It is concluded, that S signals hemodynamically stable APE more frequently than is quoted. APE-S patients could not be clearly discriminate from APE patients without S on the basis of the parameters studied and S did not impact the course of APE during in- hospital period."}, {"id": "pubmed23n0938_19450", "title": "Development of a postoperative occlusive thrombus at the site of an implanted inferior vena cava filter: A case report.", "score": 0.009009009009009009, "content": "Although an inferior vena cave (IVC) filter is placed to prevent fatal pulmonary embolism (PE), several complications associated with an IVC filter have been reported. We describe a case with symptomatic PE, of which the origin was an occlusive IVC thrombus that developed from the placement of an IVC filer after a laparoscopy-assisted total gastrectomy (LATG). A 71-year-old man underwent LATG under general anesthesia alone. He had an IVC filter implanted 13 years ago. An intravenous infusion of unfractionated heparin was substituted for the discontinuation of oral warfarin four days before the surgery. The proposed operation was performed and took a total of 404 minutes including the total duration of pneumoperitoneum that took 374 minutes. After the surgery, he experienced severe shivering reactions that required frequent bolus infusions of antihypertensive drugs. On the third postoperative day, he complained of dyspnea after taking a short walk, and subsequently lost consciousness. While he spontaneously recovered without requiring any resuscitation efforts, we performed computed tomography (CT) examination for suspected PE. The CT showed that a massive thrombus was occupying the intravenous space from the IVC filter to the left common iliac vein with several embolic defects in the peripheral pulmonary arteries present. An anticoagulant therapy was established with 10 mg of oral apixaban given twice a day for the first four days, followed by a reduction to 5 mg. On the 17th postoperative day, an ultrasound vascular examination confirmed the complete disappearance of deep venous thrombus (DVT). As an IVC filter itself may be a potential source of DVT, we should carefully manage patients with a previously implanted IVC filter throughout the perioperative period."}, {"id": "pubmed23n0260_12014", "title": "Acute pulmonary embolism. Aggressive therapy with anticoagulants and thrombolytics.", "score": 0.009009009009009009, "content": "Patients with acute pulmonary embolism are at risk for early death or chronic morbidity. Appropriate therapy can dramatically reduce the incidence of both. Oxygen and heparin therapy should be started as soon as the diagnosis is suspected. The condition of a hypotensive patient with right ventricular overload from acute pulmonary embolism usually is made worse by a fluid challenge; hypotension may be relieved by preload reduction or even by gentle diuresis. Norepinephrine (Levophed), isoproterenol hydrochloride (Isuprel), and epinephrine are the pressor agents of choice. Immediate thrombolysis is the standard of care for any patient with significant hypoxemia or hypotension due to proven pulmonary embolism. Beyond this, the potential benefit of using thrombolytic agents should be considered routinely for every patient with proven pulmonary embolism. Surgical embolectomy is useful for unstable pulmonary embolism when there are absolute contraindications to thrombolysis or when thrombolytic therapy fails. Empirical use of thrombolysis may be considered as a last-ditch effort for a critically ill patient when there is a high clinical suspicion of pulmonary embolism. Standard closed-chest cardiopulmonary resuscitation is ineffective when the pulmonary circulation is obstructed by thrombus. Emergency thoracotomy or femorofemoral cardiopulmonary bypass is appropriately used in patients with full cardiac arrest from pulmonary embolism."}, {"id": "pubmed23n0514_19976", "title": "Management of mobile right heart thrombi: a prospective series.", "score": 0.008928571428571428, "content": "Mobile right heart thrombi (MRHT) are uncommon but their true prevalence is unknown. The aim of our study was to assess the prevalence of MRHT by a systemic use of transthoracic echocardiography in a prospective series of consecutive patients admitted for acute severe pulmonary embolism (PE) and to adopt intravenous thrombolysis with recombinant tissue plasminogen activator (rt-PA) as the first line intention to treat patients with proven MRHT. We performed a systematic transthoracic echocardiogram from November 1997 to June 1999 in 335 consecutive patients admitted for suspected acute massive PE in whom the diagnosis was subsequently confirmed by perfusion lung scan or angiography. MRHT was identified in 12 of the 335 patients (4%). Nine patients presented a coil form and three patients a ball form. The thrombolytic employed in all cases was rt-PA according to the following protocol: 10 mg in a bolus and 40 mg over 2 h, followed by 50 mg over 5 h, up in a total dose of 100 mg, associated with a bolus of 5000 units of heparin. Control echocardiograms were performed 12 h after the initiation of treatment and at 12-month follow-up. Three patients died before the onset of thrombolytic infusion. The nine remaining patients were submitted to thrombolytic therapy using rt-PA. In seven of the nine remaining patients, MRHT was no longer observed after 12 h and the echocardiographic signs of RV overload had disappeared. The two last patients required adjunctive surgery because of evidence of persistent thrombus in a pulmonary artery. After 24 h, both scintigraphy and angiography demonstrated improved pulmonary perfusion. At 1-year follow-up, all patients were alive and the pulmonary artery pressure estimated by Doppler echocardiography was &lt;30 mm Hg. The incidence of right heart thrombus is low in patients admitted for acute PE. Thrombolytic therapy with rt-PA appears to be rapidly effective in most patients with MRHT. The thrombus usually resolves and pulmonary perfusion is rapidly improved. Systematic echocardiogram appears to be useful for rapidly detecting MRHT in patients with suspected massive PE."}, {"id": "pubmed23n0971_18306", "title": "Pulmonary embolectomy in a case of subacute pulmonary embolism, with previous unsuccessful fibrinolysis", "score": 0.008928571428571428, "content": "Pulmonary embolism is a potentially fatal heart condition that requires prompt restoration of blood flow in the pulmonary vascular bed and prevention of recurrent events. Mortality is associated to the degree of hemodynamic repercussion, complications and opportunity in the treatment. Male 33 years of age who began with sudden dyspnea, chest pain of moderate intensity, sweating and syncope. His admission vitals signs: blood pressure 100/70 mm Hg, heart rate 125 beats per minute, respiratory rate 24; peripheral saturation 85 %. Physical examination: grade I jugular engorgement at 45 degrees, rhythmic heart sounds, with auscultation of systolic murmur I/IV in tricuspid focus and second reinforced heart sound. Rest of exploration without relevant data. The echocardiogram showed data of right ventricular failure and systolic pulmonary artery pressure of 60 mm Hg; the angiotomography showed thrombosis of both branches of the pulmonary artery. The patient received fibrinolytic therapy with tecneteplase 50 mg single bolus and antithrombotic therapy. Due to persistence of residual thrombus, the patient underwent surgical bilateral embolectomy. Surgical pulmonary embolectomy rescue is an alternative management with highly satisfactory results."}, {"id": "pubmed23n0951_2305", "title": "Dasatinib-Induced Pulmonary Arterial Hypertension Treated with Upfront Combination Therapy.", "score": 0.008849557522123894, "content": "Pulmonary arterial hypertension (PAH) is a rare complication of dasatinib that was approved as a first-line therapy for chronic myelocytic leukemia (CML). A 24-year-old man presenting dyspnea at rest and leg edema was admitted to our hospital. He had been diagnosed with CML and prescribed dasatinib for 4 years. Chest X-ray showed significant bilateral pleural effusion and heart enlargement. Echocardiography revealed interventricular septal compression and elevated peak tricuspid regurgitation pressure gradient of 66.7 mmHg indicating severe pulmonary hypertension. After the other specific diseases to provoke PAH were excluded, he was diagnosed with dasatinib-induced PAH. Despite discontinuation of dasatinib and intravenous administration of diuretic for two weeks, World Health Organization (WHO) functional class was still II and mean pulmonary arterial pressure (PAP) was high at 37 mmHg. Therefore, we administered sildenafil and bosentan together as an upfront combination therapy three weeks after dasatinib discontinuation. Six months later, his symptoms improved to WHO functional class I and mean PAP was decreased to 31 mmHg. Although PAH is a rare complication of dasatinib, symptomatic patients prescribed with dasatinib should have an echocardiogram for PAH screening. Moreover, the upfront combination therapy would be a useful option for symptomatic patients after discontinuation of dasatinib."}, {"id": "pubmed23n0515_13859", "title": "[Therapy of acute pulmonary thromboembolism from the physician's standpoint].", "score": 0.008849557522123894, "content": "The therapy of acute pulmonary thromboembolism (APTE) is based on the clinical grade and ranges from ambulant therapy with anticoagulation, to thrombolysis, inferior vena cava (IVC) filtration, and catheter thrombectomy. In the absence of contraindications, initial treatment of APTE should consist of parenteral anticoagulation with unfractionated heparin. Long-term anticoagulation therapy, usually with warfarin, should be administered according to the individual risk profile of the patient. Thrombolytic therapy may be appropriate for patients with massive APTE with cardiac shock, syncope, etc. Similarly, thrombolysis has been reported to be effective in submassive APTE with right ventricular overload on echocardiography. IVC filters should be reserved for APTE with deep vein thrombosis (DVT) in which there are absolute contraindications to anticoagulation, recurrent thromboemboli despite therapeutic anticoagulation, and status after surgical thrombectomy. Relative indications for IVC filters that require individualized decision making include proximal DVT, especially with free-floating thrombi or in patients with limited cardiopulmonary reserve. For patients with massive APTE with contraindications to anticoagulation or in whom anticoagulation is uneffective, transcatheter aspiration with catheterization or fragmentation using a guidewire and rotating pig-tail catheter can be used. In addition, cardiopulmonary management such as supplemental oxygen, catecholamine administration, percutaneous cardiopulmonary support, etc. may be necessary for individual patients."}, {"id": "wiki20220301en025_8015", "title": "Pulmonary hypertension", "score": 0.008771929824561403, "content": "Exclude other diseases If the echocardiogram is compatible with a diagnosis of pulmonary hypertension, common causes of pulmonary hypertension (left heart disease and lung disease) are considered and further tests are performed accordingly. These tests generally include electrocardiography (ECG), pulmonary function tests including lung diffusion capacity for carbon monoxide and arterial blood gas measurements, X-rays of the chest and high-resolution computed tomography (CT) scanning. Ventilation/perfusion scintigraphy If heart disease and lung disease have been excluded, a ventilation/perfusion scan is performed to rule out CTEPH. If unmatched perfusion defects are found, further evaluation by CT pulmonary angiography, right heart catheterization, and selective pulmonary angiography is performed. CT scan"}, {"id": "pubmed23n0028_9906", "title": "[Results of embolectomy in massive pulmonary embolism (author's transl)].", "score": 0.008771929824561403, "content": "In ten patients successful embolectomy after acute massive pulmonary embolism was performed. Clinical symptoms included circulatory arrest and shock as well as collaps, syncope and dyspnoe. Pulmonary angiography regularly showed massive, bilateral emboli. In 9 patients more than one half of the pulmonary artery system was involved (perfusion defect more than 50%). Right heart catheterization demonstrated pulmonary hypertension in all cases. In 8 patients the pulmonary artery mean pressure (PAm) exceeded 30 mm Hg. In 9 patients there were signs of right heart failure (RVEDP more than 11 mm Hg). At recatheterization 6 to 30 (mean 19) days after operation using cardiopulmonary bypass there was a marked improvement of pulmonary angiograms, which were normal in 3 cases. PAm decreased from 34.3 mm Hg to 14.6 mm Hg postoperatively and RVEDP from 14.4 to 5.1 mm Hg (p less than 0.001). These results confirm, that pulmonary embolectomy leads to a good functional results."}, {"id": "pubmed23n0681_3608", "title": "[Successful operative case of chronic thromboembolic pulmonary hypertension clinically diagnosed as bronchial asthma].", "score": 0.008695652173913044, "content": "We report a case of a 70-year-old man with chronic thromboembolic pulmonary hypertension (CTEPH) in whom bronchial asthma had been clinically diagnosed and treated, and who showed remarkable improvement by pulmonary endarterectomy. He had dyspnea on exertion and had been clinically treated for bronchial asthma for 15 years. However, his symptoms did not improve after oral and inhaled corticosteroid therapy, and he had dyspnea at rest. CTEPH was suspected by echocardiography and computed tomography (CT) and he was admitted to our hospital. Perfusion scans showed multiple segmental perfusion defects with normal ventilation study, and contrast-enhanced CT showed intramural thrombi in both pulmonary arteries. Right cardiac catheterization revealed a mean pulmonary arterial pressure of 70 mm Hg and pulmonary vascular resistance of 1699 dyn.s.cm(-5) with chronic thromboembolic findings on pulmonary angiography. After surgery his pulmonary hemodynamics and symptoms significantly improved. CTEPH is rarely diagnosed at the initial visit because the only symptom is dyspnea on exertion, and it is often misdiagnosed as other respiratory diseases. But it is important to suspect and diagnose CTEPH in patients with unexplained dyspnea because this disease can be cured by surgery."}, {"id": "pubmed23n0682_24498", "title": "[Repeated prolonged thrombolytic therapy after unsuccessful thrombolysis in massive pulmonary embolism: a case report].", "score": 0.008695652173913044, "content": "We report on a 43-year-old woman who presented with shortness of breath and syncope due to massive pulmonary embolism. Transthoracic echocardiography showed signs of right ventricular overload, and contrast-enhanced chest computed tomography demonstrated filling defects in both main pulmonary arteries consistent with obstructing thrombi. Initially, thrombolytic therapy with recombinant tissue plasminogen activator was given, but shock was not resolved. Thrombolytic therapy was repeated with streptokinase and infusion was extended to 48 hours, which yielded a successful result without any hemorrhagic complication. Repeated prolonged thrombolytic therapy after initial unsuccessful thrombolysis can be considered an alternative option in massive pulmonary embolism."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[0, 142]], "word_ranges": [[0, 21]], "text": "The patient probably presents with bronchiolitis. At this stage, no additional tests should be performed unless there is a clinical worsening."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The patient probably presents with bronchiolitis. At this stage, no additional tests should be performed unless there is a clinical worsening.", "full_answer_no_ref": "The patient probably presents with bronchiolitis. At this stage, no additional tests should be performed unless there is a clinical worsening.", "full_question": "6-month-old infant presenting to the emergency department for respiratory distress. Examination: axillary temperature 37.2°C, respiratory rate 40 rpm, heart rate 160 bpm, blood pressure 90/45 mmHg, SatO2 95% on room air. He shows moderate respiratory distress with intercostal and subcostal retraction. Pulmonary auscultation: scattered expiratory rhonchi, elongated expiration and slight decrease in air entry in both lung fields. Cardiac auscultation: no murmurs. It is decided to keep the patient under observation in the hospital for a few hours. What do you consider the most appropriate attitude at this time with regard to the complementary tests?", "id": 502, "lang": "en", "options": {"1": "Request venous blood gas, leukocyte count and acute phase reactants.", "2": "Request chest X-ray.", "3": "Request arterial blood gases and acute phase reactants.", "4": "Do not request complementary tests.", "5": NaN}, "question_id_specific": 77, "type": "PEDIATRICS", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en013_148122", "title": "Acute bronchitis", "score": 0.01879964695498676, "content": "A variety of tests may be performed in people presenting with cough and shortness of breath: A chest X-ray is useful to exclude pneumonia which is more common in those with a fever, fast heart rate, fast respiratory rate, or who are old. A sputum sample showing neutrophil granulocytes (inflammatory white blood cells) and culture showing that has pathogenic microorganisms such as Streptococcus species. A blood test would indicate inflammation (as indicated by a raised white blood cell count and elevated C-reactive protein). Decreased breath sounds, crackles, wheezing, and rhonchi that clears with coughs may be heard in the chest. Dullness to percussion and pleural rub suggest disease extension beyond the bronchi such as seen with pneumonia. Paroxysms of cough followed by inspiratory whoop and vomiting suggests pertussis."}, {"id": "pubmed23n0824_2161", "title": "Clinical and Laboratory Findings in Patients With Acute Respiratory Symptoms That Suggest the Necessity of Chest X-ray for Community-Acquired Pneumonia.", "score": 0.016700473292765382, "content": "Pneumonia is a common illness in all parts of the world and is considered as a major cause of death among all age groups. Nevertheless, only about 5% of patients referring to their primary care physicians with acute respiratory symptoms will develop pneumonia. This study was performed to derive practical criteria for performing chest radiographs for the evaluation of community-acquired pneumonia (CAP). A total of 420 patients with acute respiratory symptoms and positive findings on chest radiograph were evaluated from December 2008 to December 2009. The subjects were referred to outpatient clinics or emergency departments of Birjand's medical university hospitals, Iran, and were enrolled as positive cases. A checklist was completed for each patient including their demographic information, clinical signs and symptoms (cough, sputum production, dyspnea, chest pain, fever, tachycardia, and tachypnea), abnormal findings in pulmonary auscultation and laboratory findings (erythrocyte sedimentation rate, C-reactive protein levels, and white blood cell count). An equal number of age-matched individuals with acute respiratory symptoms, but insignificant findings on chest radiography, were included as the control group. Finally, the diagnostic values of different findings were compared. The data showed that vital signs and physical examination findings are useful screening parameters for predicting chest radiograph findings in outpatient settings. Therefore, by implementing a prediction rule, we would be able to determine which patients would benefit from a chest X-Ray (sensitivity, 94% and specificity, 57%). This study's findings suggest that requesting chest radiographs might not be necessary in patients with acute respiratory symptoms unless the vital signs and/or physical examination findings are abnormal. Considering the 94% sensitivity of this rule for predicting CAP, a chest radiograph is required for patients with unreliable follow-ups or moderate to high likelihood of morbidity if CAP is not initially detected."}, {"id": "wiki20220301en571_22565", "title": "Pulmonary examination", "score": 0.01588572132257866, "content": "The pulmonary examination or respiratory examination is the portion of the physical examination where the physician examines the respiratory system for signs of disease. It is performed as a part of a complete physical examination, or the physician may choose to perform a focused respiratory exam. Classically, it is performed after the HEENT examination, and consists of four stages: inspection, palpation, percussion, and auscultation. If there are signs of respiratory disease, the physician may order additional tests including medical imaging, such as a chest X-ray or CT scan, or laboratory tests, such as a complete blood count. The information gathered from the physical examination, along with the medical history, is synthesized in order to produce a differential diagnosis and treatment plan. References Medical treatments"}, {"id": "wiki20220301en568_14379", "title": "List of medical tests", "score": 0.014638792800922444, "content": "medical test is a medical procedure performed to detect, diagnose, or monitor diseases, disease processes, susceptibility, or to determine a course of treatment. The tests are classified by speciality field allowing to know in which ward of hospital or by which specialist doctor are usually these tests performed. This list is not exhaustive but might be useful as a guide. Where available, ICD-10 codes are listed. Consulting Room Tests These tests are usually performed in a consulting room by any doctor and require no advanced equipment. general Temperature measurement, with a thermometer Patient's Respiratory rate measurement Blood oxygen concentration measurement taking the patient's pulse weighing, and measuring height and girth measuring blood pressure specific: abdominal palpation cardiac ausculation HEENT examination digital rectal examination neurological examination psychiatric assessment pulmonary auscultation vaginal examination"}, {"id": "wiki20220301en069_40749", "title": "Community-acquired pneumonia", "score": 0.01329185520361991, "content": "Hospitalization Some CAP patients require intensive care, with clinical prediction rules such as the pneumonia severity index and CURB-65 guiding the decision whether or not to hospitalize. Factors increasing the need for hospitalization include: Age greater than 65 Underlying chronic illnesses Respiratory rate greater than 30 per minute Systolic blood pressure less than 90 mmHg Heart rate greater than 125 per minute Temperature below 35 or over 40 °C Confusion Evidence of infection outside the lung Laboratory results indicating hospitalization include: Arterial oxygen tension less than 60 mm Hg Carbon dioxide over 50 mmHg or pH under 7.35 while breathing room air Hematocrit under 30 percent Creatinine over 1.2 mg/dl or blood urea nitrogen over 20 mg/dl White-blood-cell count under 4 × 10^9/L or over 30 × 10^9/L Neutrophil count under 1 x 10^9/L"}, {"id": "wiki20220301en200_20677", "title": "Acute chest syndrome", "score": 0.011660811865729898, "content": "Diagnosis The diagnosis of acute chest syndrome is made difficult by its similarity in presentation with pneumonia. Both may present with a new opacification of the lung on chest x-ray. The presence of fevers, low oxygen levels in the blood, increased respiratory rate, chest pain, and cough are also common in acute chest syndrome. Diagnostic workup includes chest x-ray, complete cell count, reticulocyte count, ECG, and blood and sputum cultures. Patients may also require additional blood tests or imaging (e.g. a CT scan) to exclude a heart attack or other pulmonary pathology. Prevention Hydroxyurea is a medication that can help to prevent acute chest syndrome. It may cause a low white blood cell count, which can predispose the person to some types of infection. Treatment Broad spectrum antibiotics to cover common infections such as Streptococcus pneumoniae and mycoplasma, pain control, and blood transfusion. Acute chest syndrome is an indication for exchange transfusion."}, {"id": "pubmed23n0075_10919", "title": "[A case of miliary tuberculosis associated with acute respiratory failure during pregnancy].", "score": 0.011524910270341467, "content": "A case of miliary tuberculosis associated with acute respiratory failure during pregnancy was reported. A 39-year-old, 29-week pregnant woman was admitted to our hospital with complaints of nonproductive cough and fever on June 12. On admission, her temperature was 38.2 degrees C; pulse rate was 90/min., and blood pressure was 120/76 mmHg. Physical examination revealed moist rales at right lung basis. Chest X-ray showed small nodular infiltrates in right lower lung field. Laboratory data revealed positive CRP, accelerated ESR and increased level of alpha 2-globulin. The number of T-cells was markedly decreased (14/mm3). The PPD skin test was negative, and the sputum smears for acid-fast bacilli were negative. Suspected of bacterial or viral pneumonia, the patient was treated with antibiotics (CPM, EM and CAZ), which had no effects for her. On June 16, the Chest X-ray showed infiltrates throughout bilateral lung fields, and the patient became increasingly dyspneic. On June 18, the results of arterial blood gas, analysis under room air were: PaO2 26.7 Torr, PaCO2 29.0 Torr, pH 7.505. Because of severe hypoxemia, she was intubated and placed on a volume-cycled respirator. Hydrocortisone (1000 mg, daily) was added to treatment because ARDS was suspected. Since the smears of tracheobronchial secretions showed acid-fast bacilli on June 24, she was diagnosed to have miliary tuberculosis. Then the intensive therapy with antituberculosis drugs (isoniazid 400 mg, rifampicin 450 mg, and streptomycin 1g, daily) was started. The non specific antibiotics were discontinued; hydrocortisone was tapered and stopped. The next week, she became afebrile and hypoxemia steadily improved.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en002_144456", "title": "Pneumonia", "score": 0.010555926916221033, "content": "Physical exam Physical examination may sometimes reveal low blood pressure, high heart rate, or low oxygen saturation. The respiratory rate may be faster than normal, and this may occur a day or two before other signs. Examination of the chest may be normal, but it may show decreased expansion on the affected side. Harsh breath sounds from the larger airways that are transmitted through the inflamed lung are termed bronchial breathing and are heard on auscultation with a stethoscope. Crackles (rales) may be heard over the affected area during inspiration. Percussion may be dulled over the affected lung, and increased, rather than decreased, vocal resonance distinguishes pneumonia from a pleural effusion. Imaging"}, {"id": "pubmed23n1130_20911", "title": "A 24-Year-Old Man With Dyspnea and a Broken Left Femur.", "score": 0.009900990099009901, "content": "A 24-year-old White man presented with 1-day complaints of progressive shortness of breath and fever. He recently underwent an open reduction and internal fixation of a left midshaft femur fracture from a skiing accident 4 days ago. He denied chest pain, skin rashes, hemoptysis, hematemesis, melena, or surgical site bleeding. On arrival, the patient appeared in mild respiratory distress with a respiratory rate of 23 breaths/min, temperature of 37.8°C, heart rate of 97 beats/min, BP of 95/54 mm Hg, and peripheral saturation of 97% on 6-L/min nasal canula. His initial peripheral saturation on room air was 67%. Physical examination was unremarkable, except for diffuse rhonchi on chest auscultation. Chest radiograph on admission showed alveolar opacities predominantly in bilateral lower lobes. A chest CT angiography revealed no evidence for pulmonary embolism. However, there were findings of diffuse bilateral ground-glass opacities with areas of patchy consolidation and innumerous micronodules in both lungs (Fig 1). Laboratory examination was significant for a drop of hemoglobin by 3 g/dL and hematocrit level by 7% since his hospital discharge 4 days earlier. His renal function and urine analysis were normal. Venous blood gas on admission showed pH of 7.39 and Pco<sub2</sub of 43 mm Hg. Because of unexplained acute anemia, nonspecific CT chest findings and progressive dyspnea, a bronchoscopy with BAL was performed. Four aliquots of 60 mL saline solution were injected for lavage with fluid return (Fig 2). BAL fluid showed WBC count of 0.411 × 10<sup3</sup/mm<sup3</sup, RBC count of 318 × 10<sup3</sup/mm<sup3</sup, 100% fresh RBCs, 73% neutrophil, 24% lymphocytes, 1% monocytes, and 2% eosinophils. BAL fluid cytologic condition is shown in Figure 3. A full vasculitis workup by rheumatology was unremarkable. Ophthalmologic and skin examination were unrevealing."}, {"id": "pubmed23n0531_12149", "title": "Diagnostic value of lung auscultation in an emergency room setting.", "score": 0.009900990099009901, "content": "In daily routine, physicians use history, physical examination and technology-based information such as laboratory tests and imaging studies to diagnose the patients' disease. We determined the diagnostic value of lung auscultation in patients admitted to the Medical emergency room with chest symptoms. Two-hundred-and forty-three consecutive patients (137 males), mean age 59.2 years were included. Internal Medicine registrars had to make a presumptive diagnosis, 1) after having taken the history and 2) after having auscultated the lungs. Thereafter, routine diagnostic procedures were performed. The estimated diagnosis was compared with the final diagnosis based on the written report to the Family Practitioner. Two-hundred-eighty-seven diagnoses were made. Eighteen percent of patients suffered from left heart failure, 13% from unexplained chest pain, 10.5% from chest wall pain, and 10.5% from pneumonia. Forty-one percent of the diagnoses were already correct when based only on the patient's history. Lung auscultation improved the diagnostic yield only in 1% and worsened it in another 3%. By multiple logistic regression, normal lung auscultation (OR 0.12 [95CI% 0.053-0.29]) was the independent predictor for not having a lung or heart disease. However, elevation of B-type natiuretic peptide (BNP) (OR 1.16 per 100 pg/ml (95CI% 1.004-1.35), wheezing (OR 0.023 [0.002-0.33]) and pCO2 (OR 0.25 (0.10-0.621) were independent predictors for having a heart disease, whereas wheezing (OR 7.41 [3.26-16.83]) and CRP (OR 1.008 per 10 units [1.003-1.014]) were risk factors for having a lung disease. In contrast to history taking, abnormal lung auscultation does not appear to contribute considerably to the final diagnosis in patients presenting with chest symptoms in an emergency room setting. However, normal lung auscultation is a valuable predictor for not having a lung or heart disease, whereas wheezing is a predictor for having a lung disease and not having a heart disease."}, {"id": "wiki20220301en324_30932", "title": "Myocardial infarction diagnosis", "score": 0.00980392156862745, "content": "Physical examination The general appearance of patients may vary according to the experienced symptoms; the patient may be comfortable, or restless and in severe distress with an increased respiratory rate. A cool and pale skin is common and points to vasoconstriction. Some patients have low-grade fever (38–39 °C). Blood pressure may be elevated or decreased, and the pulse can become irregular. If heart failure ensues, elevated jugular venous pressure and hepatojugular reflux, or swelling of the legs due to peripheral edema may be found on inspection. Rarely, a cardiac bulge with a pace different from the pulse rhythm can be felt on precordial examination. Various abnormalities can be found on auscultation, such as a third and fourth heart sound, systolic murmurs, paradoxical splitting of the second heart sound, a pericardial friction rub and rales over the lung. Electrocardiogram"}, {"id": "pubmed23n0643_17160", "title": "[Fulminant, life-threatening influenza A/H1N1 virus infection].", "score": 0.009708737864077669, "content": "A 52-year-old man presented with unproductive cough, fever and chill in our emergency department. Self-medication with amoxicillin over 3 days failed to improve his condition. The patient was in poor general condition. His body temperature was 38.4 C, with a heart rate of 124/min, a blood pressure of 120/70 mmHg and a positive shock index. At auscultation of the chest fine rales were heard over both lungs with diminished percussion sounds basal. The respiratory rate was 30/min and the oxygen saturation of 84% at room air. Laboratory: signs of inflammation; blood gas analysis: pronounced hypoxemia. A chest radiogram revealed signs of extensive pulmonary infiltrates on both sides. The patient was admitted to our Intensive Care Unit. He received piperacillin, sulbactam and levoflaxacin, ample fluid and non-invasive ventilation as well as intermittent catecholamine treatment. As there was no clinical improvement the patient was intubated on day 3. On bronchoscopy viral etiology was suspected. At this time the respiratory situation deteriorated. Acute respiratory distress syndrome (ARDS) was diagnosed. An antifungal and antiviral treatment (voriconazol, oseltamivir) was started and a cortisone pulse was attempted. The patient was transferred to another clinic where extracorporeal membranoxygenation (ECMO) was performed on the same day. The following day influenza A/H1N1-test was confirmed. Ten days after transfer, the patient regained spontaneous respiration, and he most likely survives the infection. The incidence of influenza (A/H1N1) has increased in Germany and severe and lethal courses have occurred. Therefore, the diagnostic and treatment algorithms need to be reconsidered in order to rapidly diagnose and treat infections."}, {"id": "pubmed23n0271_11816", "title": "The spectrum of patients strongly influences the usefulness of diagnostic tests for pneumonia.", "score": 0.009615384615384616, "content": "To study the influence of the spectrum of patients on the usefulness of five clinical cues, \"very annoying dyspnoea\", \"strong lateral chest pain\", crackles, C-reactive protein analysis, and erythrocyte sedimentation rate in the diagnosis of pneumonia. Evaluating the diagnostic properties of the cues against radiographic pneumonia at four steps in the diagnostic process, associated with increasing prevalence of pneumonia: 1. in all the 581 patients included, 2. in 402 of these patients who underwent physical chest examination, 3. in 188 patients classified by the doctors as having a lower respiratory tract infection, and 4. in 79 patients referred for radiography by the doctors. The municipal emergency clinic in Tromsø, Norway. 581 adult patients with respiratory tract infection. Sensitivity, specificity, Likelihood Ratio, and Positive predictive value. A tendency of decreasing specificity and Likelihood Ratio with increasing prevalence of pneumonia was demonstrated for all test, except for C-reactive protein analysis. This tendency may be explained either by the emphasis laid on the tests by the doctors when selecting patients for the diagnostic steps, or by an association between the evaluated tests and those emphasized by the doctors. As the diagnostic value of symptoms and signs are strongly influenced by selection, caution should be shown when transferring diagnostic values from one clinical setting to another."}, {"id": "wiki20220301en019_79996", "title": "Acute respiratory distress syndrome", "score": 0.009523809523809525, "content": "According to the 2012 Berlin definition, adult ARDS is characterized by the following: lung injury of acute onset, within 1 week of an apparent clinical insult and with the progression of respiratory symptoms bilateral opacities on chest imaging (chest radiograph or CT) not explained by other lung pathology (e.g. effusion, lobar/lung collapse, or nodules) respiratory failure not explained by heart failure or volume overload decreased Pa/Fi ratio (a decreased Pa/Fi ratio indicates reduced arterial oxygenation from the available inhaled gas): mild ARDS: 201 – 300 mmHg (≤ 39.9 kPa) moderate ARDS: 101 – 200 mmHg (≤ 26.6 kPa) severe ARDS: ≤ 100 mmHg (≤ 13.3 kPa) Note that the Berlin definition requires a minimum positive end expiratory pressure (PEEP) of 5 cm for consideration of the Pa/Fi ratio. This degree of PEEP may be delivered noninvasively with CPAP to diagnose mild ARDS."}, {"id": "pubmed23n0649_9101", "title": "[How much pulmonary diagnostics does the family physician need?].", "score": 0.009523809523809525, "content": "Spirometry plays the major role in pulmonary diagnostics in the family practice, but is still used much too rarely on patients with respiratory symptoms. Every patient with shortness of breath or a chronic, persistent cough should have spirometry performed. Needless to say, taking a selective medical history and auscultation of the lungs and heart are additional, important pillars for making a diagnosis. Measurement of the peak expiratory flow (PEF) can be especially helpful in emergencies. Pulse oxymetry also aids in the assessment of acute situations and additionally, can provide valuable information in the follow-up treatment of chronic respiratory diseases."}, {"id": "pubmed23n0799_21446", "title": "Acute respiratory distress in a silversmith.", "score": 0.009433962264150943, "content": "A 25-year-old young male patient presented in casualty department with severe respiratory distress on the fourth day from onset of symptoms. The patient was nonsmoker and had no antecedent medical or drug history. Prior to admission, patient had dry cough and bilateral pleuritic chest pain for the last three days. He was in severe respiratory distress with use of accessory muscles of respiration. On examination, he had heart rate of 120 beats/min, blood pressure (BP) of 150/80, respiratory rate of 48-52/min and central cyanosis present. On systemic examination, reduced intensity of breath sounds with extensive rhonchi and crepitation was found in both lung fields, with other examination being within normal limits. On pulse oximetry, oxygen saturation was 28% on room air, which increased up to 36% with the help of 4 L oxygen via nasal prongs. PaO2/FiO2 ratio was 100. Chest X-ray analysis was suggestive of non-cardiac pulmonary edema in view of bilateral fluffy opacity without cardiomegaly. In view of 2/3 positive criteria, his provisional diagnosis was Acute Respiratory Distress Syndrome (ARDS). He required mechanical ventilatory support and was gradually weaned over a period of 10 days. The patient was treated with broad spectrum antibiotics and other supportive measures. On re-evaluation of history, we found that he was a goldsmith by occupation, smelting silver and gold for the past 8-10 years. On the day of onset of symptoms, while smelting silver he was exposed to golden yellow fumes for around 15 minutes, with the quantum of exposure more than any other day earlier. From previous experience and analysis of similar silver metals, he was able to tell us that the silver was adulterated with large amount of cadmium on that day than before. Serum level of cadmium was 2.9 μg/L 6 days after initial exposure. At the time of discharge, he had residual opacities in the chest radiograph and resting oxygen saturation was 94% on room air. "}, {"id": "pubmed23n0836_15145", "title": "[Lung auscultation--an overview].", "score": 0.009433962264150943, "content": "The auscultation of the lungs is - among anamnesis - the most important part in the assessment of patients presenting with pulmonary symptoms. The lung auscultation is reproducible, cost efficient and very helpful to distinguish between differential diagnoses, in particular in emergency situations. Detection and description of lung sounds requires experience and should be performed by strict adherence to the internationally accepted terminology. "}, {"id": "wiki20220301en018_73205", "title": "Air embolism", "score": 0.009345794392523364, "content": "Venous or pulmonary air embolism occurs when air enters the systemic veins and is transported to the right side of the heart and from there into the pulmonary arteries, where it may lodge, blocking or reducing blood flow. Gas in the venous circulation can cause cardiac problems by obstructing the pulmonary circulation or forming an air-lock which raises central venous pressure and reduces pulmonary and systemic arterial pressures. Experiments on animals show that the amount of gas necessary for this to happen is quite variable. Human case reports suggest that injecting more than 100 mL of air into the venous system at rates greater than 100 mL/s can be fatal. Very large and symptomatic amounts of venous air emboli may also occur in rapid decompression in severe diving or decompression accidents, where they may interfere with circulation in the lungs and result in respiratory distress and hypoxia."}, {"id": "pubmed23n0419_14639", "title": "Counting respiratory rate in infants under 2 months: comparison between observation and auscultation.", "score": 0.009345794392523364, "content": "The World Health Organization's global programme for the control of acute respiratory infections relies on counting respiratory rate (RR) by observing abdominal and chest movements in order to diagnose pneumonia. However, few studies on the reliability of the observation method have been published. We counted RR simultaneously by observation and auscultation in 100 healthy infants at 1, 2, 4, 6 and 8 weeks of age for 15, 30 and 60 sec, and compared RRs obtained by the two methods. In all the age groups studied, the co-efficients of variation for the RRs recorded by observation or auscultation were similar. The mean RR by observation was higher by 1-3 breaths/min than mean RR by auscultation (p &lt; 0.001). The 95% confidence interval (+/-2 SD) for the difference between RR by the two methods ranged from +5 to -8 breaths/min for RR counted for 1 full minute. Our data support the assumption that observation is as reliable as auscultation for counting RR."}, {"id": "pubmed23n0976_16075", "title": "Lung injury from inhaling butane hash oil mimics pneumonia.", "score": 0.009259259259259259, "content": "\"Dabbing\", a relatively new form of THC use which utilizes Butane Hash Oil (BHO), an extraction of dried cannabis containing high levels of butane and terpene byproducts. The extraction process yields a waxy substance that is heated, vaporized and inhaled. We describe a lung injury as a result of BHO use. A previously healthy 18-year-old female presented to the ED with shortness of breath for 3-4 days. Initial oxygen saturation was 79% on room air. She was refractory to bronchodilators, steroids and supplemental O<sub2</sub. She has a 1-pack year smoking history and daily BHO abuse. Chest x-ray was positive for bilateral patchy infiltrates with mild hyperinflation. CT was negative for Pulmonary Embolus or other acute pathologic process. Sputum gram stain and blood cultures were negative. Arterial blood gases confirmed a pO2 of 73 mmHg. On physical exam she was tachycardic and tachypneic. Respiratory auscultation showed decreased air entry bilaterally with diffuse expiratory wheezing, bilateral rhonchi and a prolonged expiratory phase. We concluded her severe pneumonitis was secondary to daily BHO inhalation. Heating BHO to high temperatures, releases up to 75% of THC, compared to 5-20% THC in traditional smoked cannabis. At 978°F terpenes degrade into methacrolein and benzene. Methacrolein is structurally similar to acrolein, a pulmonary irritant, which causes acute lung injury and pulmonary edema in laboratory animals. We hypothesize a mechanism of lung injury and acute respiratory failure secondary to inhalation of high levels of methacrolein and benzene related to relatively novel phenomena of BHO use."}, {"id": "pubmed23n1065_12088", "title": "[Lung auscultation in the 21th century].", "score": 0.009259259259259259, "content": "Lung auscultation is an essential part of the physical examination for diagnosing respiratory diseases. The terminology standardization for lung sounds, in addition to advances in their analysis through new technologies, have improved the use of this technique. However, traditional auscultation has been questioned due to the limited concordance among health professionals. Despite the revolu tionary use of new diagnostic tools of imaging and lung function tests allowing diagnostic accuracy in respiratory diseases, no technology can replace lung auscultation to guide the diagnostic process. Lung auscultation allows identifying those patients who may benefit from a specific test. Moreover, this technique can be performed many times to make clinical decisions, and often with no need for- complicated and sometimes unavailable tests. This review describes the current state-of-the-art of lung auscultation and its efficacy based on the current respiratory sound terminology. In addition, it describes the main evidence on respiratory sound concordance studies among health professionals and its objective analysis through new technology."}, {"id": "wiki20220301en078_59515", "title": "Respiratory sounds", "score": 0.00909090909090909, "content": "Other tests of auscultation"}, {"id": "wiki20220301en009_14193", "title": "Sepsis", "score": 0.009009009009009009, "content": "The Surviving Sepsis Campaign has recommended 30 ml/kg of fluid to be given in adults in the first three hours followed by fluid titration according to blood pressure, urine output, respiratory rate, and oxygen saturation with a target mean arterial pressure (MAP) of 65 mmHg. In children an initial amount of 20 ml/kg is reasonable in shock. In cases of severe sepsis and septic shock where a central venous catheter is used to measure blood pressures dynamically, fluids should be administered until the central venous pressure reaches 8–12 mmHg. Once these goals are met, the central venous oxygen saturation (ScvO2), i.e., the oxygen saturation of venous blood as it returns to the heart as measured at the vena cava, is optimized. If the ScvO2 is less than 70%, blood may be given to reach a hemoglobin of 10 g/dL and then inotropes are added until the ScvO2 is optimized. In those with acute respiratory distress syndrome (ARDS) and sufficient tissue blood fluid, more fluids should be given"}, {"id": "pubmed23n0373_2003", "title": "[Auscultation of the lungs--still a useful examination?].", "score": 0.009009009009009009, "content": "Auscultation of the lungs has been a central element in clinical examination since the early part of the nineteenth century. However, the role of the stethoscope in our diagnostic work-up has more and more been challenged by newer diagnostic equipment. Research carried out over the last 30 years has given us new knowledge about the physical basis of lung sounds and the meaning of the sounds. Electronic stethoscopes and computer-based analysis of digital lung sounds are now available. Lungs auscultation findings should be interpreted with caution and be related to the case history and other clinical findings."}, {"id": "pubmed23n0113_1803", "title": "The diagnosis of adult pneumonia in general practice. The diagnostic value of history, physical examination and some blood tests.", "score": 0.008928571428571428, "content": "Because of lower respiratory infection that was treated with antibiotics on the suspicion of pneumonia, 71 patients aged 15 years or more were referred to the study by general practitioners. Using a positive chest X-ray as a \"gold standard\", 15% had pneumonia. The diagnostic value of variables from history, physical examination and blood tests was evaluated by calculating the likelihood ratio (LR). A duration of illness less than 24 hours before consulting the general practitioner was the variable from the history with the highest LR, 13.5. The white blood cell count and particularly the C-reactive protein analysis had a high diagnostic value, CRP greater than 50 mg/l had an LR of 37. In this selected material pulmonary symptoms and lung findings were of minor value in differentiating patients with and without pneumonia, with no LR exceeding 2.3. This can be explained to some extent by selection bias."}, {"id": "pubmed23n0941_15930", "title": "Report of a lung carcinoma extended to the left atrium through pulmonary vein.", "score": 0.008849557522123894, "content": "Lung cancers may extend along or grow through the pulmonary veins to invade or lie within the left atrium (LA). A 62-year-old man, previously healthy, presented with 1-month ventilatory-independent right hemithorax back pain, dry cough and large effort dyspnea. He also referred weight loss of 12 kg in 10 months and denied hemoptysis. As antecedents, he smoked for 40 years and moderate daily alcoholism. On physical examination, the patient was in good general condition, hydrated and regular respiration at rest [blood pressure (BP) =120/80 mmHg; heart rate (HR) =90 bpm; respiratory rate (RR) =16 rpm]. Cardiac auscultation revealed two standard rhythmic sounds without murmurs. Pulmonary auscultation revealed a slightly diminished vesicular murmur in the lower 1/3 of the right hemithorax without adventitious noises. Chest radiography showed a mass over the right lower lung. A CT scan confirmed the radiography image with the mass extending along the right inferior pulmonary vein and a tumor in the LA. Transthoracic and transesophageal echocardiography revealed large mass within the LA (occupying almost the entire cavity), measuring about 10 cm × 3 cm at its largest diameter, prolapsing into the left ventricle. Bronchoscopy, head CT scan, and whole-body bone scintigraphy investigation did not show any distant metastasis. The patient was successfully operated removing the intracardiac and inferior pulmonary vein tumor with the aid of cardiopulmonary bypass, followed by a right inferior lobectomy carried out after 25 days. After 30 days from surgery presented seizures associated a brain metastasis evidenced by CT when adjuvant radio and chemotherapy was started. During the next 90 days, the clinical conditions worsened, and the patient died 4 months after the surgical treatment. The case report has two primary justifications, even considering the poor outcome: (I) rarity and (II) the possibility of the surgical treatment."}, {"id": "pubmed23n0120_16415", "title": "The lung exam.", "score": 0.008849557522123894, "content": "Accurate diagnosis is essential for effective treatment. After history-taking, the physical examination is second in importance in assessing a pulmonary patient. The time-honored sequence of inspection, palpation, percussion, and auscultation is appropriate. Diagnostic tests are becoming more complex, more expensive, and more inclined to separate the patient and physician. The stethoscope is still the more commonly used diagnostic medical instrument, but it is not always used to best advantage. It is familiar, harmless, portable, and inexpensive. Its appropriate use improves medical practice and reduces costs. Improvements in sound recording and analysis techniques have spurred a renewed interest in lung sounds and their meaning. This is likely to lead to better understanding of what we hear, and perhaps to the development of new noninvasive diagnostic and monitoring techniques."}, {"id": "wiki20220301en019_80018", "title": "Acute respiratory distress syndrome", "score": 0.008771929824561403, "content": "If Pa:Fi < 300 mmHg (40 kPa), then the definitions recommended a classification as \"acute lung injury\" (ALI). Note that according to these criteria, arterial blood gas analysis and chest X-ray were required for formal diagnosis. Limitations of these definitions include lack of precise definition of acuity, nonspecific imaging criteria, lack of precise definition of hypoxemia with regards to PEEP (affects arterial oxygen partial pressure), arbitrary Pa thresholds without systematic data."}, {"id": "pubmed23n0092_1391", "title": "[Requirements in radiologic diagnosis from the internist-cardiology viewpoint].", "score": 0.008771929824561403, "content": "The article discusses the demands to be made on the various radiological methods in thoracic diagnostics in respect of their informative value for therapeutic consequences, as seen from the viewpoint of internal-cardiological intensive-care medicine. The importance of x-ray thoracic film in one or two planes is emphasised, with special reference to routine imaging. In consideration of the demonstration of essential pathological findings, routine chest x-ray film would be mandatory only the 40th year of age onwards and lateral projection from the 50th year. At any rate, about 50% of routine thorax x-rays in intensive-care wards show changes, the percentage being higher if there is a clinical suspicion of changes. Mention must be made of a so-called time phase lag between the x-ray thoracic findings and changed pulmonary arterial pressure. In diagnosis of pulmonary artery embolism the nuclear medicine methods are compared with pulmonary arterial angiography, taking sensitivity and specificity into account. 90% of pulmonary embolism show only non-specific changes in the chest x-ray. Together with these and perfusion scintigraphy a sensitivity of 98% can be attained, albeit with lower specificity, since perfusion angiography and angiography agree in only about 87% of the cases. The demands to be made on radiological diagnostics must be determined from case to case by the diagnostic effectivity in relation to the technical setup and cost. The cost aspect of the individual methods is of major importance when installing equipment in hospitals."}, {"id": "wiki20220301en016_6881", "title": "Pulmonary heart disease", "score": 0.008695652173913044, "content": "Signs and symptoms The symptoms/signs of pulmonary heart disease (cor pulmonale) can be non-specific and depend on the stage of the disorder, and can include blood backing up into the systemic venous system, including the hepatic vein. As pulmonary heart disease progresses, most individuals will develop symptoms like: Shortness of breath Wheezing Cyanosis Ascites Jaundice Enlargement of the liver Raised jugular venous pressure (JVP) Third heart sound Intercostal recession Presence of abnormal heart sounds Causes The causes of pulmonary heart disease (cor pulmonale) are the following: Acute respiratory distress syndrome (ARDS) COPD Primary pulmonary hypertension Blood clots in lungs Kyphoscoliosis Interstitial lung disease Cystic fibrosis Sarcoidosis Obstructive sleep apnea (untreated) Sickle cell anemia Bronchopulmonary dysplasia (in infants)"}, {"id": "pubmed23n1017_1195", "title": "[Pulmonary sonography-a valuable supplement to basic diagnostics for timely outpatient clarification of cough and dyspnea].", "score": 0.008695652173913044, "content": "Cough and dyspnea are among the most common symptoms in primary medical care and potentially threatening diseases must be excluded in a timely manner, especially acute heart failure and its causes, pneumonia, pleural effusion, pulmonary embolism and pneumothorax. Anamnesis, inspection, physical examination and technical basic diagnostics are usually sufficient for an initial risk stratification. A reliable suspected diagnosis can often be made in this way; however, it is not uncommon for the findings to be ambiguous. Chest X‑ray diagnostics and laboratory diagnostics are established as the standard approach for these situations; however, a major limitation of these diagnostic techniques is the lack of immediate availability in the general practitioner's office and laboratory results are not available until the next day or the day after. Furthermore, the sensitivity and specificity of these diagnostic procedures are limited but often overestimated, especially in the case of mild to moderately pronounced alterations and in early stages of a disease. Thoracic sonography can be used in these situations as a direct extension of the physical examination. Its diagnostic value is undisputed. The most important pathological findings, such as pleural effusion and subpleural consolidations can be immediately visualized with sufficient certainty using miniaturized handheld ultrasound devices. The concept of the ultrasound stethoscope, which has been under discussion for more than 15 years, can also be implemented as point-of-care ultrasound (POCUS). The POCUS will become established as routine diagnostics in the future, for example in emergency outpatient diagnostics. It is time for pulmonary ultrasound to be added to the repertoire of primary care diagnostics."}, {"id": "wiki20220301en009_161903", "title": "Emergency department", "score": 0.008660605492548505, "content": "Asthma and COPD Acute exacerbations of chronic respiratory diseases, mainly asthma and chronic obstructive pulmonary disease (COPD), are assessed as emergencies and treated with oxygen therapy, bronchodilators, steroids or theophylline, have an urgent chest X-ray and arterial blood gases and are referred for intensive care if necessary. Noninvasive ventilation in the ED has reduced the requirement for tracheal intubation in many cases of severe exacerbations of COPD. Special facilities, training, and equipment An ED requires different equipment and different approaches than most other hospital divisions. Patients frequently arrive with unstable conditions, and so must be treated quickly. They may be unconscious, and information such as their medical history, allergies, and blood type may be unavailable. ED staff are trained to work quickly and effectively even with minimal information."}]}}}}
{"correct_option": 5, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": true, "char_ranges": [[0, 274]], "word_ranges": [[0, 40]], "text": "Low BP with high jugular pressure should always raise the suspicion of cardiac tamponade. Fever and chest pain with dyspnea and tachypnea should raise suspicion of pericardial effusion with hemodynamic compromise. Paradoxical pulse is a typical finding of cardiac tamponade."}}, "full_answer": "Low BP with high jugular pressure should always raise the suspicion of cardiac tamponade. Fever and chest pain with dyspnea and tachypnea should raise suspicion of pericardial effusion with hemodynamic compromise. Paradoxical pulse is a typical finding of cardiac tamponade. Therefore, the correct answer is 5.", "full_answer_no_ref": "Low BP with high jugular pressure should always raise the suspicion of cardiac tamponade. Fever and chest pain with dyspnea and tachypnea should raise suspicion of pericardial effusion with hemodynamic compromise. Paradoxical pulse is a typical finding of cardiac tamponade. Therefore, the [HIDDEN].", "full_question": "A patient with a history of fever and chest pain comes to the hospital with dyspnea and tachypnea. On physical examination, the blood pressure cyphrads are low, jugular venous pressure is elevated with a deep descending sinus X, and he has a pulsus paradoxus. What pathology should be suspected?", "id": 71, "lang": "en", "options": {"1": "Ischemic heart disease.", "2": "Dilated cardiomyopathy.", "3": "Severe aortic valve stenosis.", "4": "Constrictive pericarditis.", "5": "Pericardial effusion with cardiac tamponade."}, "question_id_specific": 50, "type": "ANESTHESIOLOGY, CRITICAL CARE AND EMERGENCIES", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0552_7649", "title": "[Recurrent autoreactive pericardial effusion. Impact of an aetiological classification of pericarditis].", "score": 0.01692155196828094, "content": "A 36 year-old man suffered from fever, fatigue, pleurodynia and precordial discomfort. His family physician suspected febrile tracheobronchitis and treated it with ampicillin for 5 days. Because symptoms persisted an ECG was done which suggested acute myocardial infarction. The patient underwent an emergency coronary angiography which excluded coronary artery disease and aortic dissection. Pericarditis was suspected and the patient put on aspirin, 500 mg/d. Because of persisting cardiac symptoms an echocardiography was performed which revealed systolic separation between epi- and pericardium, characteristic of a small pericardial effusion after acute pericarditis. The symptoms improved after one week of treatment with diclofenac and the ECG had become normal. Two months later the patient was seen at our cardiac outpatient clinic. He had night sweats, sporadic precordial pain and severe dyspnoe. Further investigations revealed tachycardia (120/min), hypotension (95/70 mm Hg), pulsus paradoxus and jugular vein sustension. Echocardiography revealed a large pericardial effusion (\"swinging heart\"), which explained the low voltage and the electrical alternans in the ECG. Pericardiocentesis was carried out the same day to relieve the tamponade. It was followed by pericardioscopy and epi- as well as pericardial biopsy. 485 ml of a serous effusion were drained. Cytology and histology demonstrated a lymphocytic fibrinous pericarditis. Polymerase chain reaction (PCR) on viral and bacterial RNA and DNA of potentially cardiotropic agents remained negative. The pigtail catheter was left in place and 80 mg of gentamycin were given intrapericardially on day 1 and 2, followed by 500 mg of crystalloid triamcinolone acetate after the PCR was found to be negative. Oral treatment with 0.5 mg colchicine three times a day (off-label use) was started and maintained for 6 months. After 9 months no effusion was detected and the patient was free of symptoms. After exclusion of bacterial and viral pericardial infection, a high single dose of intrapericardial triamcinolone combined with long-term oral colchicine has proven to be a highly efficacious treatment of autoreactive pericarditis which will avoid relapses in most cases."}, {"id": "pubmed23n0495_3220", "title": "Cases from the Osler Medical Service at Johns Hopkins University.", "score": 0.015966921119592876, "content": "PRESENTING FEATURES: A 70-year-old African American man was admitted with a history of fever, chills, and malaise of several days' duration. His past medical history was notable for end-stage renal disease requiring hemodialysis, coronary artery disease, and aortic stenosis requiring a bioprosthetic aortic valve replacement. On the day of admission, the patient was noted to have a shaking chill while undergoing dialysis through his catheter and was admitted to the hospital. He complained of pain at the catheter insertion site, shortness of breath, and dyspnea on exertion, but denied chest pain. On physical examination, the patient had a temperature of 100.4 degrees F, with a heart rate of 64 beats per minute, blood pressure of 127/72 mm Hg, and an oxygen saturation of 97% on room air. He was a mildly obese man in no apparent distress. He had shotty cervical lymphadenopathy and a right subclavian dialysis catheter in place, with erythema and pus at the entry site. His jugular venous pressure was 10 cm H(2)O. Lung examination showed bibasilar rales. Heart sounds were normal, with no rub or gallop. He had a 2/6 systolic ejection murmur best heart at the left sternal border as well as a 3/6 holosystolic murmur at the apex that radiated to his left axilla. Examination of the abdomen and extremities was unremarkable. The patient's neurological examination was unremarkable, and he was alert and oriented to person, place, and time. Laboratory studies showed an elevated white blood cell count of 16,700 cells/microL. His blood urea nitrogen level was 43 mg/dL and his serum creatinine level was 4.9 mg/dL. Multiple blood cultures grew methicillin-resistant Staphylococcus aureus. An admission, chest radiograph showed no infiltrate. An admission electrocardiogram showed normal sinus rhythm with first degree atrioventricular block, left anterior fascicular block, and left ventricular hypertrophy. shows rhythm strips from lead II electrocardiograms 5 months before admission (top), on admission (middle) and 5 days after admission (bottom). What is the diagnosis?"}, {"id": "article-28076_10", "title": "Pulsus Paradoxus -- History and Physical", "score": 0.012740384615384615, "content": "Intracardiac shunts or moderate to severe valvular insufficiency Co-existing disease that significantly increases left or right ventricular diastolic pressure such severe systemic hypertension, aortic stenosis, or cor pulmonale Aortic dissection resulting in pericardial effusion/tamponade Cardiac tamponade in hypovolemia"}, {"id": "article-18905_7", "title": "Cardiac Tamponade -- History and Physical", "score": 0.012698412698412698, "content": "Patients with cardiac tamponade present similar to patients with other forms of cardiogenic or obstructive shock. They may endorse vague symptoms of chest pain, palpitations, shortness of breath, or in more severe cases, dizziness, syncope, and altered mental status.  They may also present in a pulseless electrical activity cardiac arrest. The classic physical findings in cardiac tamponade included in Beck’s triad are hypotension, jugular venous distension, and muffled heart sounds. Pulsus paradoxus, which is a decrease in systolic blood pressure by more than 10 mm Hg with inspiration is an important physical exam finding that suggests a pericardial effusion is causing cardiac tamponade. Pulsus paradoxus may be absent in patients with ASD, elevated diastolic pressures, pulmonary hypertension and aortic regurgitation. The Kussmaul sign - a paradoxical elevated in JVP and pressure during inspiration is sometimes seen in cardiac tamponade."}, {"id": "article-17749_11", "title": "Aortic Valve Disease -- History and Physical", "score": 0.012611017199280903, "content": "Aortic regurgitation when acute and/or severe can be suspected when the patient has a wide pulse pressure, and a low pitched early diastolic murmur is auscultated again over the right sternal border at the second intercostal space. Accentuated P2 may also be noticed due to elevated pressures in the pulmonary vasculature. Chronic aortic regurgitation may illicit a blowing diastolic decrescendo murmur with a positive correlation between the duration of murmur and the severity of the disease. Often auscultation of a laterally and inferiorly displaced apical impulse is present and sustained. Often there is the phenomenon of Corrigan pulse (water hammer), a bounding and forceful pulse that rapidly increases and collapses. Other less common physical exam findings include the de Musset sign, which is subtle head bobbing with a pulse, as well as the Quincke sign and Muller sign (pulsations on the fingernails and uvula, respectively). Similar physical exam findings to aortic stenosis can also be seen late in disease progression. Acute aortic regurgitation will present differently depending on the etiology. If a patient presents with tearing severe chest pain along with physical exam findings such as variation in blood pressure between the right and left extremities, consider aortic dissection as a cause. If the patient presented with a history of streptococcal infection along with fevers, swollen and tender joints, skin nodules, new onset of rash, then rheumatic heart disease would be high on the differential."}, {"id": "wiki20220301en028_9481", "title": "Kussmaul's sign", "score": 0.012299071633782084, "content": "Causes The differential diagnoses of Kussmaul's sign includes constrictive pericarditis, restrictive cardiomyopathy, pericardial effusion, and severe right-sided heart failure. With cardiac tamponade, jugular veins are distended and typically show a prominent x descent and an absent y descent as opposed to patients with constrictive pericarditis (prominent x and y descent), see Beck's triad. Other possible causes of Kussmaul's sign include: Right ventricular infarction - low ventricular compliance Right heart failure Cardiac tumours Tricuspid stenosis Restrictive cardiomyopathy Pulmonary embolism Constrictive pericarditis History Kussmaul's sign is named after the German doctor who first described it, Adolph Kussmaul (1822-1902). He is also credited with describing Kussmaul breathing. See also Pulsus paradoxus References Symptoms and signs: Vascular"}, {"id": "wiki20220301en025_33861", "title": "Pericarditis", "score": 0.012227258353536418, "content": "Physical examinations The classic sign of pericarditis is a friction rub heard with a stethoscope on the cardiovascular examination, usually on the lower left sternal border. Other physical signs include a person in distress, positional chest pain, diaphoresis (excessive sweating); possibility of heart failure in form of pericardial tamponade causing pulsus paradoxus, and the Beck's triad of low blood pressure (due to decreased cardiac output), distant (muffled) heart sounds, and distension of the jugular vein (JVD)."}, {"id": "wiki20220301en001_252441", "title": "Heart", "score": 0.011771874238362174, "content": "Pericardial disease The sac which surrounds the heart, called the pericardium, can become inflamed in a condition known as pericarditis. This condition typically causes chest pain that may spread to the back, and is often caused by a viral infection (glandular fever, cytomegalovirus, or coxsackievirus). Fluid can build up within the pericardial sac, referred to as a pericardial effusion. Pericardial effusions often occur secondary to pericarditis, kidney failure, or tumours, and frequently do not cause any symptoms. However, large effusions or effusions which accumulate rapidly can compress the heart in a condition known as cardiac tamponade, causing breathlessness and potentially fatal low blood pressure. Fluid can be removed from the pericardial space for diagnosis or to relieve tamponade using a syringe in a procedure called pericardiocentesis. Congenital heart disease"}, {"id": "article-38171_16", "title": "Cardiac Syncope -- History and Physical", "score": 0.011771822073329611, "content": "A careful physical exam will assess heart rate and rhythm. Any abnormalities will increase suspicion of cardiac arrhythmia. In addition, an elevated respiratory rate or hypoxia will increase suspicion of a pulmonary embolism. Jugular venous distention and hypotension are suggestive signs of an obstructive mechanical cardiac etiology. Pathological cardiac murmurs, specifically new ones, will clue one into a valvular etiology, hypertrophic cardiomyopathy, or an obstructive intracardiac lesion. Muffled sounds can be heard with pericardial tamponade. Pedal edema or other evidence of deep venous thrombosis increases a patient's risk of pulmonary embolism. [10]"}, {"id": "InternalMed_Harrison_20985", "title": "InternalMed_Harrison", "score": 0.011697181052019761, "content": "Diagnosis Due to the unstable condition of these patients, supportive therapy must be initiated simultaneously with diagnostic evaluation (Fig. 326-2). A focused history and physical examination should be performed, blood specimens sent to the laboratory, and an electrocardiogram (ECG) and chest x-ray obtained. Etiologies of Cardiogenic Shock or Pulmonary Edema Acute myocardial infarction/ischemia LV failure Ventricular septal rupture Papillary muscle/chordal rupture–severe MR Ventricular free wall rupture with subacute tamponade Other conditions complicating large MIs Post-cardiac arrest Post-cardiotomy Refractory sustained tachyarrhythmias Acute fulminant myocarditis End-stage cardiomyopathy LV apical ballooning Takotsubo’s cardiomyopathy Hypertrophic cardiomyopathy with severe outflow obstruction Aortic dissection with aortic insufficiency or tamponade Severe valvular heart disease Other Etiologies of Cardiogenic Shockb RV failure due to:"}, {"id": "pubmed23n1162_1248", "title": "A Rare Case of MRSA Pericarditis with Expanding, Purulent Pericardial Effusion Leading to Uremic Kidney Failure from a Right, Necrotic Toe.", "score": 0.011557935477029928, "content": "Purulent pericarditis is an extremely rare entity with only a few reported cases so far. This condition deserves prompt diagnosis because of its significant mortality rate if left untreated. A 76-year-old man with a past medical history of coronary artery disease (CAD) with percutaneous coronary intervention (PCI) to the left anterior descending artery (LAD) and right circumflex artery (RCA), ischemic cardiomyopathy with moderately reduced ejection fraction (EF 45-50%), peripheral artery disease (PAD), COVID-19 pneumonia complicated by fibrotic lung disease (on 3 liters of home oxygen), type-2 diabetes mellitus (T2DM), hypertension (HTN), hyperlipidemia (HLD), and chronic kidney disease (CKD) stage III presented with complaints of pleuritic chest pain and shortness of breath. On hospital day 1, he was afebrile and hemodynamically stable with physical exam remarkable for bibasilar crackles and dry gangrene of his right first toe. He developed progressive altered mental status, hypotension, oliguric renal failure, and respiratory distress on hospital day 6. On exam at this time, he had an elevated jugular venous distension (JVD) of 12-14 cm water, pericardial friction rub with decreased heart sounds, and orthopnea; all were consistent with cardiac tamponade clinically. An electrocardiogram (EKG) showed new ST elevations in leads I, II, and aVL with ST depression in aVR and V1 with only mild elevation in troponin I to 0.07 ng/mL. A transthoracic echocardiogram (TTE) was done on hospital day 7 and showed a moderate sized pericardial effusion with inferior vena cava (IVC) enlargement but no atrial collapse, ventricular collapse, IVC collapse, or respiratory variation in the mitral and tricuspid inflow velocities. Blood cultures grew methicillin-resistant <iStaphylococcus aureus</i (MRSA) on hospital day 6, and he was started on intravenous (IV) vancomycin. The differential diagnosis for his enlarging pericardial effusion included purulent pericarditis, uremic pericarditis, or hemorrhagic effusion. He had urgent diagnostic and therapeutic pericardiocentesis with removal of 350 milliliters of fluid. The pericardial fluid was cloudy, tan-brown with a gram stain showing gram-positive cocci in clusters and cultures growing MRSA, which confirmed the diagnosis of purulent pericarditis secondary to MRSA infection. After the pericardiocentesis, his blood pressure, respiratory distress, and renal failure improved. The source of the bacteremia was from osteomyelitis of his gangrenous, right toe with bone biopsy growing both MRSA and <iStreptococcus anginosus</i. He underwent toe amputation for definitive source control. He was discharged on hospital day 24 with a plan to complete 6 weeks of IV vancomycin."}, {"id": "InternalMed_Harrison_2955", "title": "InternalMed_Harrison", "score": 0.011429678419217357, "content": "Additional clues to the etiology and importance of a heart murmur can be gleaned from the history and other physical examination findings. Symptoms suggestive of cardiovascular, neurologic, or pulmonary disease help focus the differential diagnosis, as do findings relevant to the jugular venous pressure and waveforms, the arterial pulses, other heart sounds, the lungs, the abdomen, the skin, and the extremities. In many instances, laboratory studies, an ECG, and/or a chest x-ray may have been obtained earlier and may contain valuable information. A patient with suspected infective endocarditis, for example, may have a murmur in the setting of fever, chills, anorexia, fatigue, dyspnea, splenomegaly, petechiae, and positive blood cultures. A new systolic murmur in a patient with a marked fall in blood pressure after a recent MI suggests myocardial rupture. By contrast, an isolated grade 1 or 2 mid-systolic murmur at the left sternal border in a healthy, active, and asymptomatic young"}, {"id": "InternalMed_Harrison_17406", "title": "InternalMed_Harrison", "score": 0.011300537914280756, "content": "or absence of a third heart sound (S3). Accurate characterization of cardiac murmurs provides important insight into the natural history of many valvular and congenital heart lesions. Finally, the important role played by the physical examination in enhancing the clinician-patient relationship cannot be overestimated. THE GENERAL PHYSICAL EXAMINATION Any examination begins with an assessment of the general appear-ance of the patient, with notation of age, posture, demeanor, and 267 SEC Tion 2 DiAgnoSiS oF CARDiovASCulAR DiSoRDERS overall health status. Is the patient in pain or resting quietly, dyspneic or diaphoretic? Does the patient choose to avoid certain body positions to reduce or eliminate pain, as might be the case with suspected acute pericarditis? Are there clues indicating that dyspnea may have a pulmonary cause, such as a barrel chest deformity with an increased anterior-posterior diameter, tachypnea, and pursed-lip breathing? Skin pallor, cyanosis, and jaundice can be"}, {"id": "article-17744_7", "title": "Aortic Stenosis -- History and Physical", "score": 0.011199371911513416, "content": "The acquired aortic stenosis manifests with exertional dyspnea, syncope, angina, and, ultimately, heart failure. [17] [18] Typically, symptoms begin at the age of 50 to 70 years in patients with the bicuspid aortic valve and in greater than 70 years in patients with tri-leaflet valve calcific stenosis. Patients progressively experience a gradual decrease in exercise tolerance, dyspnea on exertion, and fatigue. Severe exertional dyspnea, paroxysmal nocturnal dyspnea, orthopnea, and pulmonary edema show various degrees of pulmonary venous hypertension. Angina results from the combination of the need for increased oxygen in hypertrophied myocardium and reduction of oxygen delivery secondary to the excessive compression of coronary vessels. Syncope is caused by the decrease in cerebral perfusion occurring during exertion when the arterial pressure declines due to systemic vasodilation and an inadequate increase in cardiac output related to stenosis. It is also due to the malfunction of the baroreceptor mechanism in severe aortic stenosis. Non-cardiac symptoms include gastrointestinal (GI) bleeding and cerebral emboli. GI bleeding is observed in patients with severe aortic stenosis and is often associated with angiodysplasia or other vascular malformations. It manifests from shear stress-induced platelet aggregation and reduction in the von Willebrand factor. [19] Cerebral emboli occur due to microthrombi formation on thickened bicuspid valves. There is also an observed increase in the risk of infective endocarditis in patients with aortic valve disease, especially with a bicuspid valve. On examination, carotid upstroke can be observed on palpation. A slow-rising, late-peaking, and a low-amplitude carotid impulse, pulsus parvus et tardus, is an expected finding in severe aortic stenosis and, when present, is specific to aortic stenosis. On auscultation, the second heart sound may lack a split and can be heard as a single sound during inspiration. It can also become paradoxical when the closure of the aortic valve gets delayed than the pulmonic valve. A mid-systolic ejection murmur, heard best over the right second intercostal space, with radiation into the right neck. However, high-frequency components may radiate to the apex in calcified aortic valves, and this phenomenon is called the Gallavardin phenomenon. The murmur becomes softer in LV failure and when there is a fall of stroke volume."}, {"id": "article-17105_12", "title": "Acquired Immune Deficiency Syndrome -- History and Physical -- Cardiac System [8]", "score": 0.011110261702724052, "content": "HIV infection and ART likely contribute to increased cardiovascular disease in patients. Common presenting symptoms may include chest pain, shortness of breath, or fatigue. The examination should proceed as one would when assessing for acute coronary syndrome or valvular disease, palpating for chest wall pain, observing for jugular venous distension and peripheral edema, and auscultating for abnormal heart sounds, murmurs, or evidence of pulmonary edema. Cardiovascular AIDS-related illnesses could include purulent pericarditis or cardiac tamponade caused by Mycobacterium tuberculosis . If these conditions are suspected, observing for Beck’s triad of low blood pressure, jugular venous distension, and muffled heart sounds may confirm a compressive pericardial effusion."}, {"id": "pubmed23n0944_25265", "title": "Diagnostic Challenges in Chronic Constrictive Pericarditis.", "score": 0.010705855906485331, "content": "Chronic constrictive pericarditis (CCP) is a disease that has multiple possible causes and is associated with variable clinical findings, depending on its severity. It develops insidiously, and in many cases, particularly in developed countries, no antecedent diagnosis can be found. These cases are termed idiopathic. Tuberculosis is the leading cause of constrictive pericarditis in developing nations but represents only a small minority in developed countries. Here the authors describe two different case reports where tuberculosis was the probable cause of CCP. A 21-year-old man born in Cape Verde living in Europe for 4 years and a 24-year-old man born in Guiné Bissau were both admitted due to intense precordial pain and syncope after exertion. Interestingly both had fatigability, dyspnea, chest discomfort and palpitations on exertion, as well as progressive involuntary weight loss and decubitus cough. On physical examination they had tachycardia, jaundice, cachexia, elevated jugular venous pressure, hepatomegaly and ascites. Both electrocardiograms showed prominent P waves and chest X-ray showed bilateral pulmonary interstitial infiltrates and enlargement of the right cavities. Analytically, elevated bilirubin, leukopenia and thrombocytopenia was also found in both. Echocardiography revealed findings, in both cases, compatible with CCP including less common signs as annulus reversus and annulus paradoxus. Thoraco-abdomino-pelvic CT from both patients revealed chronic liver disease with congestion, pleural effusion, pericardial calcifications, ascites and massive mediastinal and abdominal adenopathies. Blood cultures and IGRA test were negative. However, given the presumptive diagnosis of tuberculosis (TB), anti-TB therapy was started. Despite the diagnosis of \"end-stage\" CCP with very high operative risk multidisciplinary team decided after informed consent, to perform total anterior pericardiectomy, that occurred without complications. Pericardial and mediastinal biopsies, pericardial/pleural fluid cultures/ immune-phenotyping were inconclusive. Anti- tuberculosis therapy was maintained. After surgery, the patients had a remarkable clinical improvement (NYHA I) that persisted in 6- month follow-up. These two case reports illustrate that despite the markedly elevated operative risk of pericardiectomy in \"end-stage\" forms of disease after patients informed consent must be a considered option. The other point to consider is that, despite rare, tuberculosis still is a possible diagnosis to consider in CCP in Portugal."}, {"id": "wiki20220301en093_40523", "title": "Acute pericarditis", "score": 0.010416666666666666, "content": "Corticosteroids are usually used in those cases that are clearly refractory to NSAIDs and colchicine and a specific cause has not been found. Systemic corticosteroids are usually reserved for those with autoimmune disease. Prognosis One of the most feared complications of acute pericarditis is cardiac tamponade. Cardiac tamponade is accumulation of enough fluid in the pericardial space --- pericardial effusion --- to cause serious obstruction to the inflow of blood to the heart. Signs of cardiac tamponade include distended neck veins, muffled heart sounds when listening with a stethoscope, and low blood pressure (together known as Beck's triad). This condition can be fatal if not immediately treated."}, {"id": "pubmed23n0603_1094", "title": "Pericarditis as a presenting sign of infective endocarditis: two case reports and review of the literature.", "score": 0.010331727205337288, "content": "Pericarditis as a presenting sign of infective endocarditis is rare. Here we describe 2 cases and an additional 19 cases of pericarditis as a presenting sign of infective endocarditis reported during the last 40 y. 71% of patients were young males (mean age 43.2 y). The most commonly reported underlying conditions were diabetes mellitus type 2 (5 patients, 24%), and substance or alcohol abuse (4 patients, 19%). The native aortic valve was the most frequently involved valve. The most common symptoms were fever, cough or dyspnoea, and chest pain. Overt tamponade was diagnosed in 47% of the patients. However, pulsus paradoxus and pericardial friction rub were rare. A heart murmur was heard in 12 patients (57%). Staphylococcus aureus was the most commonly isolated pathogen concomitantly from blood and pericardial fluid. 16 patients (76%) were operated. Six underwent a pericardial procedure, 5 underwent valve replacement, 4 both, and 1 patient was operated for pseudoaneurysm. Mortality rates were 60% and 31% of patients treated with antibiotics alone versus antibiotics and surgical intervention, respectively. In patients presenting with pericarditis with or without cardiac tamponade, the possibility of infective endocarditis should be considered. Optimal therapy should consist of antibiotics and surgical intervention."}, {"id": "article-28076_13", "title": "Pulsus Paradoxus -- History and Physical", "score": 0.010227958937198068, "content": "An important thing to keep in mind when considering pulsus paradoxus while discerning between pericardial and non-pericardial disease is the following: pulsus paradoxus in non-pericardial disease will usually manifest with a drop in systolic and diastolic pressures. This is in contrast to pericardial disease in which the drop is mainly in systolic pressure, diastolic pressure is usually minimally affected, and thus pulse pressure will be narrower."}, {"id": "article-21729_13", "title": "Fibrinous Pericarditis -- History and Physical", "score": 0.010079240682688957, "content": "Other critical clinical signs to be aware of are signs of tamponade such as raised jugular venous pressure (JVP), muffled heart sounds, and decreased blood pressure. If pericardial tamponade is concerned, checking for a pulsus paradoxus is recommended. It is defined as a drop of systolic blood pressure by more than 10 mm hg during inspiration."}, {"id": "InternalMed_Harrison_17791", "title": "InternalMed_Harrison", "score": 0.010075408462505236, "content": "Suspected severe valve disease in symptomatic patients—dyspnea, angina, heart failure, syncope Infective endocarditis with need for cardiac surgery Asymptomatic patients with aortic regurgitation and cardiac enlargement or Prior to cardiac surgery in patients with suspected coronary artery disease New onset with angina or suspected undiagnosed coronary artery disease New-onset cardiomyopathy of uncertain cause or suspected to be due to coronary artery disease Prior to surgical correction, when symptoms or noninvasive testing suggests coronary disease Symptomatic patients with suspected cardiac tamponade or constrictive pericarditis Hypertrophic cardiomyopathy with angina Diseases of the aorta when knowledge of coronary artery involvement is necessary for management"}, {"id": "wiki20220301en329_16514", "title": "Postpericardiotomy syndrome", "score": 0.00980392156862745, "content": "Complications Complications include pericarditis, pericardial effusion, pleuritis, pulmonary infiltration, and very rarely pericardial tamponade. Of these cardiac tamponade is the most life-threatening complication. The pericardial fluid increases intra-pericardial pressure therefore preventing complete expansion of the atria and the ventricles upon the diastole. This causes equilibration of the pressure in all four heart chambers, and results in the common findings of the tamponade which are pulsus paradoxus, Beck's triad of hypotension, muffled heart sounds, and raised jugular venous pressure, as well as EKG or Holter monitor findings such as electrical alternans. Physically the patients who progress to severe pericardial tamponade obtundate, become mentally altered, and lethargic. If left untreated, severe decrease in cardiac output, vascular collapse, and hypoperfusion of body including the brain results in death."}, {"id": "pubmed23n0133_17859", "title": "Combined multiple-valve procedures. Factors influencing the early and late results.", "score": 0.00980392156862745, "content": "The early and late results were retrospectively evaluated in 57 cases of double or triple valve replacement or repair performed in 1970-1983. The causes of the valvular lesions were rheumatic fever (43 cases), bacterial endocarditis (6), syphilis (1) and unknown (7 cases). The preoperative NYHA classification was III in 29 patients and IV in 28, due mainly to dyspnea of effort. Cardiomegaly (mean radiologic volume 880 cm3/m2) and atrial fibrillation were the dominant clinical findings. Surgery was on emergency indications in five cases. Cold cardioplegia combined with external cardiac cooling has been used for myocardial protection since 1977. The valve replacements were 56 aortic, 50 mitral and 2 tricuspid. In addition there were three closed and two open mitral commissurotomies, two mitral plastic repairs, three tricuspid valve anuloplasties (DeVega) and one aortic anuloplasty. Follow-up (0.3-13, mean 3.5 years) was supplemented with a check-up including two-dimensional echophonocardiography and hematologic tests. The operative mortality (10/57 patients) fell from 26% in 1970-1976 to 12% in 1977-1983. The causes of death were low cardiac output in preoperatively ill patients (5), myocardial infarction (2), technical failure (2) and sepsis (1 case). There were 11 late deaths (6.7/100 patient-years of observation), the commonest cause (5 patients) being congestive heart failure. The respective incidences of thromboembolism, paravalvular leak and postoperative endocarditis were 2.1, 4.2 and 2.1 episodes/100 patient-years.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en025_33862", "title": "Pericarditis", "score": 0.009708737864077669, "content": "Complications Pericarditis can progress to pericardial effusion and eventually cardiac tamponade. This can be seen in people who are experiencing the classic signs of pericarditis but then show signs of relief, and progress to show signs of cardiac tamponade which include decreased alertness and lethargy, pulsus paradoxus (decrease of at least 10 mmHg of the systolic blood pressure upon inspiration), low blood pressure (due to decreased cardiac index), (jugular vein distention from right sided heart failure and fluid overload), distant heart sounds on auscultation, and equilibration of all the diastolic blood pressures on cardiac catheterization due to the constriction of the pericardium by the fluid."}, {"id": "article-25485_9", "title": "Myopericarditis -- History and Physical", "score": 0.009708737864077669, "content": "Early symptoms included precordial chest pain, fatigue, dyspnea, palpitations, and fever. Patients may give symptoms suggestive of a viral prodrome (a runny nose, arthralgia, low-grade fever) 1 to 2 weeks preceding the presentation. In predominant pericardial involvement, they can describe the pain as sharp, worse with a cough or inspiration and relieved by sitting forward. If there is significant myocardial involvement, there may be a continuous pain, and sometimes, it is hard to differentiate from myocardial ischemia pain, especially in people with cardiovascular risk factors. They may also have predominant heart failure symptoms such as shortness of breath, orthopnea, pedal edema, and fatigue. Rare symptoms include arrhythmias, syncope, and sudden cardiac arrest. Physical examination findings can be variable, but common findings may include fever, pericardial friction rub and features of heart failure. Look for other signs of systemic illness which may contribute to the etiology."}, {"id": "wiki20220301en074_29977", "title": "Pericardial effusion", "score": 0.009615384615384616, "content": "Patients with concern for cardiac tamponade may present with abnormal vitals and what's classically known as the Beck's triad, which consists of hypotension (low blood pressure), jugular venous distension and distant heart sounds. Though these are the classical findings; all three occur simultaneously in only a minority of patients. Patients presenting with cardiac tamponade may also be evaluated for pulsus paradoxus. Pulsus paradoxus is a phenomenon in which systolic blood pressure drops by 10 mmHg or more during inspiration. In cardiac tamponade, the pressure within the pericardium is significantly higher, hence decreasing the compliance of the chambers (the capacity to expand/ conform to volume changes). During inspiration, right ventricle filling in increased, which causes the Interventricular septum to bulge into the left ventricle, hence leading to reduced left ventricular filling and consequently reduced stroke volume and low systolic blood pressure. Exams"}, {"id": "wiki20220301en028_11540", "title": "Jugular venous pressure", "score": 0.009523809523809525, "content": "Prominent 'x' descent Cardiac tamponade Slow 'y' descent Tricuspid stenosis Cardiac tamponade Prominent & deep 'y' descent Constrictive pericarditis Parodoxical JVP (Kussmaul's sign: JVP rises with inspiration, drops with expiration) Pericardial effusion Constrictive pericarditis Pericardial tamponade"}, {"id": "article-28514_9", "title": "Rheumatic Heart Disease -- History and Physical", "score": 0.009523809523809525, "content": "Carditis is the most serious presentation of rheumatic fever. The symptoms and signs of carditis are dependent on the areas of the heart involved, which include the pericardium, myocardium, or heart valves. The presentation of a pericardial friction rub on auscultation leans toward the diagnosis of pericarditis. The presence of signs of congestive heart failure points toward a diagnosis of myocarditis, which includes but is not limited to lower extremity edema, shortness of breath with exertion or rest, abdominal distension, or inability to lay flat due to shortness of breath (orthopnea). Myocarditis in the absence of valvular disease is unlikely to be rheumatic in origin. Therefore, an apical systolic or basal diastolic murmur should be auscultated on physical exam. Mitral regurgitation is the most common valvular lesion, which is an apical pan-systolic murmur on auscultation. [8] Aortic regurgitation is less common. If patients have a known history of rheumatic heart disease, a change in the character of the murmur or the presence of a new murmur on auscultation leads to the diagnosis of acute rheumatic heart fever. Rheumatic heart disease predominantly affects the left-sided cardiac valves. [8] The tricuspid valve and rarely pulmonary valve can be affected, but very unlikely without mitral valve involvement."}, {"id": "InternalMed_Harrison_1782", "title": "InternalMed_Harrison", "score": 0.009499527856468367, "content": "Echocardiography should be performed in patients with a history of cardiac disease or if abnormalities are found on physical examination or the ECG. Echocardiographic diagnoses that may be responsible for syncope include aortic stenosis, hyper-trophic cardiomyopathy, cardiac tumors, aortic dissection, and pericardial tamponade. Echocardiography also has a role in risk stratification based on the left ventricular ejection fraction. Treadmill exercise testing with ECG and blood pressure monitoring should be performed in patients who have experienced syncope during or shortly after exercise. Treadmill testing may help identify exercise-induced arrhythmias (e.g., tachycardia-related AV block) and exercise-induced exaggerated vasodilation."}, {"id": "article-41931_6", "title": "Tricuspid Valve Endocarditis -- History and Physical", "score": 0.009433962264150943, "content": "History and physical exam are essential towards making an adequate diagnosis in cases of TVIE. Establishing diagnosis can be more difficult in these cases because presentation can often be subacute or present with absence of typical IE features. Peripheral phenomenon such as splinter hemorrhages is less common, as is a cardiac murmur. However, clinicians must always be aware of potential complications of this disease process that are manifested by the various embolic phenomenon. Characteristics, which should lead clinicians to consider TVIE, include: Fever Chills Anorexia Weight loss Fatigue Malaise Arthralgia Dyspnea on exertion A cough Pleuritic pain Abdominal Pain"}, {"id": "InternalMed_Harrison_1359", "title": "InternalMed_Harrison", "score": 0.009404533230887437, "content": "PART 2 Cardinal Manifestations and Presentation of Diseases Vital Signs Significant tachycardia and hypotension are indicative of important hemodynamic consequences of the underlying cause of chest discomfort and should prompt a rapid survey for the most severe conditions, such as acute MI with cardiogenic shock, massive pulmonary embolism, pericarditis with tamponade, or tension pneumothorax. Acute aortic emergencies usually present with severe hypertension but may be associated with profound hypotension when there is coronary arterial compromise or dissection into the pericardium. Sinus tachycardia is an important manifestation of submassive pulmonary embolism. Tachypnea and hypoxemia point toward a pulmonary cause. The presence of low-grade fever is nonspecific because it may occur with MI and with thromboembolism in addition to infection."}, {"id": "wiki20220301en068_46755", "title": "Pulsus paradoxus", "score": 0.009345794392523364, "content": "Causes Pulsus paradoxus can be caused by several physiologic mechanisms. Anatomically, these can be grouped into: cardiac causes, pulmonary causes and non-pulmonary and non-cardiac causes. Considered physiologically, PP is caused by: decreased right heart functional reserve, e.g. myocardial infarction and tamponade, right ventricular inflow or outflow obstruction, e.g. superior vena cava obstruction and pulmonary embolism, and decreased blood to the left heart due to lung hyperinflation (e.g. asthma, COPD) and anaphylactic shock. List of causes Cardiac: constrictive pericarditis. One study found that pulsus paradoxus occurs in less than 20% of patients with constrictive pericarditis. pericardial effusion, including cardiac tamponade cardiogenic shock Pulmonary: pulmonary embolism tension pneumothorax asthma (especially with severe asthma exacerbations) chronic obstructive pulmonary disease"}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": true, "char_ranges": [[791, 974]], "word_ranges": [[135, 161]], "text": "both array and NGS are usually reserved for patients with non-obvious clinical pictures, intellectual disability, or suspicion of a genetic syndrome that requires these specific tests"}, "2": {"exist": true, "char_ranges": [[633, 763]], "word_ranges": [[104, 130]], "text": "A Turner can also be diagnosed with a FISH (there would only be one signal for the X instead of the two that females usually have)"}, "3": {"exist": true, "char_ranges": [[791, 974]], "word_ranges": [[135, 161]], "text": "both array and NGS are usually reserved for patients with non-obvious clinical pictures, intellectual disability, or suspicion of a genetic syndrome that requires these specific tests"}, "4": {"exist": true, "char_ranges": [[339, 632]], "word_ranges": [[58, 104]], "text": "an adolescent with pubertal delay and short stature should be a Turner until proven otherwise (there were two questions in 2017). Therefore, if that is our first diagnostic suspicion, the HABITUAL test (as the statement says) for diagnosis should be a conventional karyotype (formula: 45, X0)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "This question may be a little more difficult, as the clinical case presented to us is very vague. An adolescent with pubertal delay and short stature; nothing more. With this information, she could have several genetic pathologies, but this is the MIR and we are told that the patient does not have an intellectual disability. In the MIR, an adolescent with pubertal delay and short stature should be a Turner until proven otherwise (there were two questions in 2017). Therefore, if that is our first diagnostic suspicion, the HABITUAL test (as the statement says) for diagnosis should be a conventional karyotype (formula: 45, X0). A Turner can also be diagnosed with a FISH (there would only be one signal for the X instead of the two that females usually have) or with an array. However, both array and NGS are usually reserved for patients with non-obvious clinical pictures, intellectual disability, or suspicion of a genetic syndrome that requires these specific tests (for example, an array to diagnose a 22q11 deletion syndrome or an NGS panel to diagnose a Noonan syndrome).", "full_answer_no_ref": "This question may be a little more difficult, as the clinical case presented to us is very vague. An adolescent with pubertal delay and short stature; nothing more. With this information, she could have several genetic pathologies, but this is the MIR and we are told that the patient does not have an intellectual disability. In the MIR, an adolescent with pubertal delay and short stature should be a Turner until proven otherwise (there were two questions in 2017). Therefore, if that is our first diagnostic suspicion, the HABITUAL test (as the statement says) for diagnosis should be a conventional karyotype (formula: 45, X0). A Turner can also be diagnosed with a FISH (there would only be one signal for the X instead of the two that females usually have) or with an array. However, both array and NGS are usually reserved for patients with non-obvious clinical pictures, intellectual disability, or suspicion of a genetic syndrome that requires these specific tests (for example, an array to diagnose a 22q11 deletion syndrome or an NGS panel to diagnose a Noonan syndrome).", "full_question": "15-year-old female presenting with delayed menarche and short stature. She does not have intellectual disability. Which of the following genetic tests would be routinely used for the diagnosis of this patient:", "id": 486, "lang": "en", "options": {"1": "Massive sequencing (NGS).", "2": "FISH.", "3": "DNA and/or RNA microarrays.", "4": "Karyotype.", "5": NaN}, "question_id_specific": 45, "type": "GENETICS", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1102_9340", "title": "Genetic testing in pediatric endocrine pathology.", "score": 0.017645245800585605, "content": "In genetic endocrine diseases, genetic testing is necessary for a precise diagnosis, which will provide a better knowledge of the evolution and prognosis and also indicate the adequate therapy, targeting the precise etiopathogenesis of the disease. Genetic testing in endocrinology is often based on classical cytogenetic techniques, molecular cytogenetic analysis or molecular biology techniques. Genetic testing in disorders of sex development includes the karyotype and SRY gene analysis and depending on the presence of associated clinical signs and on the observations at paraclinical examination, these tests will be followed by chromosomal array techniques and NGS sequencing. In short stature, the decision to perform a genetic test is taken depending on clinical, paraclinical and imaging signs. In case of a short stature associated with a low weight/length for gestational age, genetic testing is proposed to evaluate a Russell-Silver syndrome or if the short stature is associated with other clinical signs (e.g. intellectual disability), chromosomal analysis by microarray is proposed. If the short stature is disproportionate, it is indicated to perform a next generation sequencing (NGS) of a panel of genes involved in skeletal dysplasia. If an endocrine cause for short stature is observed at the hormonal evaluation, it is indicated to test a panel of genes involved in these pathways. In genetic obesity, depending on clinical signs associated to obesity, it will be a more targeted genetic testing. If obesity is associated with intellectual disability or other nonspecific neurological changes, a chromosomal analysis by microarray will be indicated. If monogenic obesity is suspected, NGS testing will be indicated (as genes panel or whole exome or genome analysis). Genetic testing in endocrine diseases brings an etiological diagnosis, but a favorable cost-benefit ratio derives from an adequate indication of these tests, generally proposed in expert centers for rare endocrine diseases."}, {"id": "wiki20220301en386_8081", "title": "XXXY syndrome", "score": 0.01688515246508977, "content": "Diagnosis Diagnosis of 48,XXXY is usually done by a standard karyotype. A karyotype is a chromosomal analysis in which a full set of chromosomes can be seen for an individual. The presence of the additional 2 X chromosomes on the karyotype are indicative of XXXY syndrome. Another way to diagnosis 48,XXXY is by chromosomal microarray showing the presence of extra X chromosomes. Chromosomal microarray (CMA) is used to detect extra or missing chromosomal segments or whole chromosomes. CMA uses microchip-based testing to analyze many pieces of DNA. Males with 48,XXXY are diagnosed anywhere from before birth to adulthood as a result of the range in the severity of symptoms. The age range at diagnosis is likely due to the fact that XXXY is a rare syndrome, and does not cause as extreme phenotypes as other variants of Klinefelter syndrome (such as XXXXY)."}, {"id": "pubmed23n1093_19060", "title": "Genotype-Phenotype Analysis of 8q24.3 Duplication and 21q22.3 Deletion in a Chinese Patient and Literature Review.", "score": 0.016338801385530358, "content": "Copy number variants (CNVs) are responsible for many patients with short stature of unknown etiology. This study aims to analyze clinical phenotypes and identify pathogenic CNVs in a patient with short stature, intellectual disability, craniofacial deformities, and anal imperforation. G-banded karyotyping and chromosomal microarray analysis (CMA) was used on the patient to identify pathogenic causes. Fluorescence in situ hybridization (FISH) was applied to explore the abnormal genetic origin. Literatures were searched using identified CNVs as keywords in the PubMed database to perform genotype-phenotype analysis. Cytogenetic analysis revealed a normal karyotype 46,XY. CMA detected a 6.1 Mb duplication at 8q24.3 and a 3.6 Mb deletion at 21q22.3. FISH confirmed that the abnormal chromosomes were inherited from paternal balanced translocation. We compared phenotypes of our patient with 6 patients with 8q24.3 duplication and 7 cases with 21q22.3 deletion respectively. A novel 8q24.3 duplication and 21q22.3 deletion was identified in a Chinese patient. Genotype-phenotype analysis demonstrated that patients with 8q24.3 duplication and 21q22.3 deletion had specific facial features, intellectual disability, short stature, and multiple malformations."}, {"id": "pubmed23n0879_17989", "title": "[Genetic and prenatal diagnosis of a pregnant women with mental retardation].", "score": 0.01588177581555065, "content": "To conduct genetic testing and prenatal diagnosis for a pregnant women with growth retardation, severe mental retardation, and a history of adverse pregnancies. G-banded chromosome analysis, fluorescence in situ hybridization (FISH), and whole genome DNA microarray were used to analyze the patient and her fetus. The women was found to be a chimera containing two cell lines with 47 and 46 chromosomes, respectively. Both have involved deletion of 18q21.2q23. FISH analysis suggested that the cell line containing 47 chromosomes has harbored a chromosome marker derived from chromosome 15. The marker has contained chromosome 15p involving the SNRPN locus and part of 15q, which gave rise to a karyotype of 47,XX,del18q21.3,+ish mar D15Z1+ SNRPN+[82]/46,XX,del18q21.3[18]. Whole genome DNA microarray confirmed that a 3.044 Mb fragment from 15q11.2q12 was duplicated, which involved NIPA1, SNRPN and other 17 OMIM genes. Duplication of this region has been characterized by low mental retardation, autism, developmental delay. Meanwhile, there was a 17.992 Mb deletion at 18q21.33q23, which contained 39 OMIM genes including TNFRSF11A and PHLPP1. This fragment was characterized by mental retardation, developmental delay, short stature, and cleft palate. Whole genome microarray analysis confirmed that there was a 17.9 Mb deletion at 18q21.33q23, which has been implemented with mental retardation, general growth retardation, short stature, and cleft palate. After genetic counseling, the family decided to terminate the pregnancy at 21st week. Combined chromosome karyotyping, FISH, and whole genome DNA microarray can determine the origin of marker chromosomes and facilitate delineation of its correlation with the clinical phenotype."}, {"id": "pubmed23n1075_771", "title": "Inconsistency of Karyotyping and Array Comparative Genomic Hybridization (aCGH) in a Mosaic Turner Syndrome Case.", "score": 0.015067079463364292, "content": "<bPurpose</b  Turner syndrome is a sex chromosomal aberration where majority of the patients have 45,X karyotype, while several patients are mosaic involving 45,X/46,XX; 46,X,i(Xq); and other variants. Cytogenetic analysis, karyotyping, is considered to be the \"gold standard\" to detect numerical and structural chromosomal abnormalities. In the recent years, alternative approaches, such as array comparative genomic hybridization (aCGH), have been widely used in genetic analysis to detect numerical abnormalities as well as unbalanced structural rearrangements. In this study, we report the use of karyotyping as well as aCGH in detecting a possible Turner syndrome variant. <bMethods</b  An apparent 16-year-old female was clinically diagnosed as Turner syndrome with premature ovarian failure and short stature. The genetic diagnosis was performed for the patient and the parents by karyotyping analysis. aCGH was also performed for the patient. <bMain Findings</b  Cytogenetic analysis of the patient was performed showing variant Turner syndrome (46,X,i(X)(q10)[26]/46,X,del(X)(q11.2)[11]/45,X[8]/46,XX[5]). The patient's aCGH result revealed that she has a deletion of 57,252kb of Xp22.33-p11.21 region; arr[GRCh37] Xp22.33-p11.21 (310,932-57,563-078)X1. Both aCGH and fluorescence in situ hybridization (FISH) results suggested that <ishort stature Homeobox-containing</i ( <iSHOX</i ) gene, which is located on Xp22.33, was deleted, though FISH result indicated that this was in a mosaic pattern. <bConclusion</b  In the recent years, aCGH has become the preferred method in detecting numerical abnormalities and unbalanced chromosomal rearrangements. However, its use is hindered by its failure of detecting mosaicism, especially low-level partial mosaicism. Therefore, although the resolution of the aCGH is higher, the cytogenetic investigation is still the first in line to detect mosaicism."}, {"id": "wiki20220301en112_15190", "title": "22q13 deletion syndrome", "score": 0.01485148514851485, "content": "Diagnosis and management Clinical genetics and genetic testing Genetic testing is necessary to confirm the diagnosis of PMS. A prototypical terminal deletion of 22q13 can be uncovered by karyotype analysis, but many terminal and interstitial deletions are too small to detect with this method. Chromosomal microarray should be ordered in children with suspected developmental delays or ASD. Most cases will be identified by microarray; however, small variations in genes might be missed. The falling cost for whole exome sequencing may replace DNA microarray technology for candidate gene evaluation. Biological parents should be tested with fluorescence in situ hybridization (FISH) to rule out balanced translocations or inversions. Balanced translocation in a parent increases the risk for recurrence and heritability within families (figure 3)."}, {"id": "wiki20220301en524_27065", "title": "Elective genetic and genomic testing", "score": 0.014270407169296707, "content": "Genetic testing for a variety of disorders has seen many advances starting with cytogenetics to evaluate human chromosomes for aneuploidy and other chromosome abnormalities. The development of molecular cytogenetics involving techniques such as fluorescence in situ hybridization (FISH) followed, permitting the detection of more subtle changes in the karyotype. Techniques to determine the precise sequence of nucleotides in DNA by DNA sequencing, notably Sanger sequencing was developed in the 1970s. In the 1980s the DNA microarray appeared, permitting laboratories to find copy number variants associated with disease that are below the level of detection of cytogenetics but too large to be detected by DNA sequencing. In recent years the development of high-throughput or next-generation sequencing has dramatically lowered the cost of DNA sequencing permitting laboratories to evaluate all 20,000 genes of the human genome at once through exome sequencing and whole genome sequencing. A"}, {"id": "wiki20220301en112_15183", "title": "22q13 deletion syndrome", "score": 0.012698288661405016, "content": "Prototypical terminal deletion of 22q13 can be uncovered by karyotype analysis, but many terminal and interstitial deletions are too small. The availability of DNA microarray technology for revealing multiple genetic problems simultaneously has been the diagnostic tool of choice. The falling cost for the whole exome sequencing and, eventually, whole genome sequencing, may replace DNA microarray technology for candidate evaluation. However, fluorescence in situ hybridization (FISH) tests remain valuable for diagnosing cases of mosaicism (mosaic genetics) and chromosomal rearrangements (e.g., ring chromosome, unbalanced chromosomal translocation). Although early researchers sought a monogenic (single gene genetic disorder) explanation, recent studies have not supported that hypothesis (see Etiology). Signs and symptoms"}, {"id": "pubmed23n0733_10360", "title": "Chromosome abnormalities in Indonesian patients with short stature.", "score": 0.012091503267973857, "content": "Short stature is associated with several disorders including wide variations of chromosomal disorders and single gene disorders. The objective of this report is to present the cytogenetic findings in Indonesian patients with short stature. G-banding and interphase/metaphase FISH were performed on short stature patients with and without other clinical features who were referred by clinicians all over Indonesia to our laboratory during the year 2003-2009. The results of chromosomal analysis of ninety seven patients (mean age: 10.7 years old) were collected. The group of patients with other clinical features showed sex chromosome abnormalities in 45% (18/40) and autosomal abnormalities in 10% (4/40), whereas those with short stature only, 42.1% (24/57) had sex chromosome abnormalities and 1.75% (1/57) had autosomal abnormalities. The autosomal chromosomal abnormalities involved mostly subtelomeric regions. Results discrepancies between karyotype and FISH were found in 10 patients, including detection of low-level monosomy X mosaicism in 6 patients with normal karyotype, and detection of mosaic aneuploidy chromosome 18 in 1 patient with 45,XX,rob(13;14)(q10;q10).Statistical analysis showed no significant association between the groups and the type of chromosomal abnormalities. Chromosome abnormalities account for about 50% of the short stature patients. Wide variations of both sex and autosomal chromosomes abnormalities were detected in the study. Since three out of five patients had autosomal structural abnormalities involving the subtelomeric regions, thus in the future, subtelomeric FISH or even a more sensitive method such as genomic/SNP microarray is needed to confirm deletions of subtelomeric regions of chromosome 9, 11 and 18. Low-level mosaicism in normal karyotype patients indicates interphase FISH need to be routinely carried out in short stature patients as an adjunct to karyotyping."}, {"id": "wiki20220301en230_14682", "title": "18p-", "score": 0.012028138935996482, "content": "Genetics 18p- describes a deletion of the short arm of chromosome 18. About half of the people with deletions have a breakpoint at the centromere. Those with it are said to have centromeric 18p-, and those without are said to have non-centromeric 18p-. Diagnosis Suspicion of a chromosome abnormality is typically raised due to the presence of developmental delays or birth defects. Diagnosis of 18p- is usually made via a blood sample. A routine chromosome analysis, or karyotype, is usually used to make the initial diagnosis, although it may also be made by microarray analysis. Increasingly, microarray analysis is also being used to clarify breakpoints. Prenatal diagnosis is possible via amniocentesis of chorionic villus sampling. MRI In some children without \"classic\" holoprosencephaly, microforms of holoprosencephaly may be noted on MRI, including missing olfactory tracts and bulbs and absent or hypoplastic corpus callosum. Treatment"}, {"id": "wiki20220301en349_34756", "title": "Distal 18q-", "score": 0.010559006211180125, "content": "Diagnosis Suspicion of a chromosome abnormality is typically raised due to the presence of developmental delays or birth defects. Diagnosis of distal 18q- is usually made from a blood sample. A routine chromosome analysis, or karyotype, is usually used to make the initial diagnosis, although it may also be made by microarray analysis. Increasingly, microarray analysis is also being used to clarify breakpoints. Prenatal diagnosis is possible using amniocentesis or chorionic villus sampling. Treatment At present, treatment for distal 18q- is symptomatic, meaning the focus is on treating the signs and symptoms of the conditions as they arise. To ensure early diagnosis and treatment, people with distal 18q- are suggested to undergo routine screenings for thyroid, hearing, and vision problems. History"}, {"id": "pubmed23n1112_25413", "title": "Clinical Profiles and Genetic Spectra of 814 Chinese Children With Short Stature.", "score": 0.010094075769132977, "content": "Data and studies based on exome sequencing for the genetic evaluation of short stature are limited, and more large-scale studies are warranted. Some factors increase the likelihood of a monogenic cause of short stature, including skeletal dysplasia, severe short stature, and small for gestational age (SGA) without catch-up growth. However, whether these factors can serve as predictors of molecular diagnosis remains unknown. We aimed to explore the diagnostic efficiency of the associated risk factors and their exome sequences for screening. We defined and applied factors that increased the likelihood of monogenic causes of short stature in diagnostic genetic tests based on next-generation sequencing (NGS) in 814 patients with short stature and at least 1 other factor. Pathogenic/likely pathogenic (P/LP) variants in genes, copy number variations, and chromosomal abnormalities were identified in 361 patients. We found P/LP variants among 111 genes, and RASopathies comprised the most important etiology. Short stature combined with other phenotypes significantly increased the likelihood of a monogenic cause, including skeletal dysplasia, facial dysmorphism, and intellectual disability, compared with simple severe short stature (&lt;-3 SD scores). We report novel candidate pathogenic genes, KMT2C for unequivocal growth hormone insensitivity and GATA6 for SGA. Our study identified the diagnostic characteristics of NGS in short stature with different risk factors. Our study provides novel insights into the current understanding of the etiology of short stature in patients with different phenotypes."}, {"id": "pubmed23n0971_10923", "title": "[Diagnosis of a case with partial 9p trisomy by next generation sequencing].", "score": 0.009900990099009901, "content": "To explore the genetic cause for a child featuring growth and mental retardation. Following conventional karyotyping analysis of the trio family, next generation sequencing (NGS) was carried out to explore the origin of the supernumerary marker chromosome. Fluorescence in situ hybridization (FISH) was used to confirm the result. The karyotypes of both parents were normal, while the proband was found to be 47,XX,+mar. NGS showed that the supernumerary marker has originated from chromosome 9p13.1p24.3 with a size of 39.77 Mb. FISH has confirmed the above finding. The 9p13.1-p24.3 trisomy probably underlies the abnormal phenotypes of the child. Cytogenetic analysis combined with NGS and FISH can provide accurate diagnosis for such disorders."}, {"id": "wiki20220301en607_6121", "title": "SYNGAP1-related intellectual disability", "score": 0.00980392156862745, "content": "The majority of mutations are considered de novo, however cases of inheritance from both somatic mosaic and germ-line mosaic parents have been reported. Diagnosis Diagnosis is based on genetic testing, with the recommended testing approach being Chromosomal Microarray Analysis followed by an Intellectual Disability multigene panel or Whole Exome Sequencing. A diagnosis is established following the identification of a heterozygous pathogenic (or likely pathogenic) point mutation of the SYNGAP1 gene (present in approximately 89% of patients), a micro deletion of chromosome 6 incorporating SYNGAP1 (approximately 11% of patients), or a balanced translocation disrupting SYNGAP1. EEG monitoring frequently shows generalized epilepsy, predominantly in the occipital regions. Seizure onset usually occurs around 2 years of age. MRI is usually normal."}, {"id": "pubmed23n1126_3649", "title": "Case Report: Genetic Analysis of a Small Supernumerary Marker Chromosome in a Unique Case of Mosaic Turner Syndrome.", "score": 0.009708737864077669, "content": "The aim of this study was to explore the source and morphology of a small supernumerary marker chromosome (sSMC) from karyotype analysis of a patient with a unique case of mosaic Turner syndrome. The study findings will provide technical reference and genetic counseling for similar cases. A female patient with 46,X,+mar karyotype was diagnosed by genetic karyotype analysis. Genetic methods including fluorescence <iin situ</i hybridization (FISH) and copy number variation sequencing (CNV-seq) based on low-depth whole-genome sequencing were used to explore the source and morphology of sSMC. FISH technology showed that 56.5% of the cells were X and 43.5% of the cells were XY. CNV-seq detection found that the sSMC was chrY, implying that the patient's karyotype was mos 45,X[58.6%]/46,XY[41.4%]. Retrospective karyotype analysis indicated that the female patient's sSMC was inherited from her father's small chrY. Customized FISH probe of Yq12 microdeletion was positive, indicating that the sSMC was a del(Y)(q12). Based on the results of genetic diagnosis, the specialist doctor gave a comprehensive genetic consultation and ordered regular follow-up examinations. The findings of the current study showed that the chromosome description of the unique Turner case was mos 45,X[56.5%]/46,X,del(Y)(q12)[43.5%]. FISH technology played a key role in diagnosis of mosaicism. The terminal deletion of mosaic chrY provided a scientific and an accurate explanation for masculinity failure and abnormal sexual development of the current case."}, {"id": "pubmed23n0746_19106", "title": "A unique combination of 17pter trisomy and 21qter monosomy in a boy with developmental delay, severe intellectual disability, growth retardation and dysmorphisms.", "score": 0.009615384615384616, "content": "Microduplication at 17p13.3 and microdeletion at 21q22 are both rare chromosomal aberrations. The presence of both genomic imbalances in one patient has not been previously reported in literature. In this study, we performed a molecular diagnostic testing with a whole genome microarray on a 3-year-old boy with developmental delay, mental retardation and multiple malformations. A routine G-banding karyotype analysis was performed using peripheral lymphocytes. Chromosome microarray analysis (CMA) was done using Affymetrix CytoScan™ HD array. Genomic imbalances were further confirmed by multiple ligation-dependent probe amplification (MLPA). The result of karyotyping was normal but CMA detected a 9.8 Mb microduplication at 17p13.3-13.1 (chr17: 1-9,875,545) and a 2.8 Mb microdeletion involving 21q22.3-qter (chr21: 45,239,077-48,097,372). The imbalances were due to a balanced translocation present in patient's mother. The patient was characterized with short stature, profound developmental delay, non-verbal, intellectual disability as well as craniofacial dysmorphism, subtle brain structural anomaly and sparse scalp hair. This is the first patient reported with a combination of a microduplication at 17p13.3-13.1 and a microdeletion at 21q22.3-qter. Both genomic imbalances were undetected by conventional karyotyping but were delineated with CMA test. Synergistic effect from the two rare genomic imbalances is likely responsible for the severe clinical phenotypes observed in this patient."}, {"id": "pubmed23n1157_15749", "title": "The Case with Short Stature and Intellectual Disability Caused by a Novel 2q12 Duplication.", "score": 0.009615384615384616, "content": "Copy number variation (CNV) is a kind of malfunction of DNA polymerase to produce extra genetic material which leads to more number of repeats in genes. The CNVs have been associated with different clinical phenotypes such as learning disabilities, short stature, and intellectual disability. The chromosomal microarray analysis is an effective diagnostic method for identifying new CNVs and understanding their clinical effects. In this case report, a variation that has not been reported previously in the literature is presented. This case report will contribute to increasing the knowledge. The CNV (arr [hg19] 2q12.1q12.3 (103,368,824-107,946,062) x3) detected in the index case was also detected in her father and male sibling. Key Words: DNA, Copy number variation, Chromosomal duplication, Intellectual disabilities."}, {"id": "pubmed23n0808_4109", "title": "Partial and complete trisomy 14 mosaicism: clinical follow-up, cytogenetic and molecular analysis.", "score": 0.009523809523809525, "content": "Trisomy 14 mosaicism is a rare chromosomal abnormality. It is associated with multiple congenital anomalies. We report a 15 year-old female with an unusual karyotype with three cell lines: 47,XX,+mar/47,XX,+14/46,XX. At six months old she had short stature, cleft palate, hyperpigmented linear spots in arms and legs and developmental delay. At present, she has mild facial dysmorphism and moderate mental retardation. Cytogenetic analysis was performed in peripheral blood lymphocytes and in the light and dark skin following standard methods. DNAarray - Oligo 180 k was carried out using Agilent Technologies and FISH analysis was accomplished using DNA BACs probes to confirm the result obtained by DNAarray. Methylation-Specific PCR (MS-PCR) of the MEG3 promoter and microsatellite analysis were performed. Microarray analysis confirmed partial trisomy 14 mosaicism; the marker chromosome was found to be from chromosome 14, the result was confirmed with FISH. Methylation (14q32.3) and microsatellite (14q11-14q32.33) analysis were carried out and UPD was discarded. The global result was: mos 47,XX,+del(14)(q11.2)[45]/47,XX,+14[10]/46,XX[45]. This is a unique case because of the coexistence of two abnormal cell lines, including one with +14 and another with +del(14)(q11.2). To our knowledge, only three patients have been reported with trisomy 14 and another abnormal cell line. The array analysis identified the marker chromosome and characterized the breakpoint. The del(14)(q11.2) does not seem to be related to any particular phenotypic characteristic of the patient; the clinical features of our patient observed until now, can be attributed to trisomy 14 mosaicism. Nevertheless, we cannot discard the manifestation of new symptoms related to her karyotype in the future."}, {"id": "pubmed23n1015_12760", "title": "Karyotyping and prenatal diagnosis of 47,XX,+ 8[67]/46,XX [13] Mosaicism: case report and literature review.", "score": 0.009523809523809525, "content": "Trisomy 8 mosaicism has a wide phenotypic variability, ranging from mild dysmorphic features to severe malformations. This report concluded a female pregnant woman with trisomy 8 mosaicism, and carefully cytogenetic diagnoses were performed to give her prenatal diagnostic information. This report also provides more knowledge about trisomy 8 mosaicism and the prenatal diagnostic for clinicians. In this present study, we reported one case of pregnancy woman with trisomy 8 mosaicism. Noninvasive prenatal testing prompted an abnormal Z-score, but further three dimension color ultrasound result suggested a single live fetus with no abnormality. The phenotypic of the pregnant woman was normal. Based on our results, there were no abnormal initial myeloid cells (&lt; 10<sup- 4</sup), which suggested that the patient had no blood diseases. The peripheral blood karyotype of the patient was 47,XX,+ 8[67]/46,XX [13], and karyotype of amniotic fluid was 46, XX. The next generation sequencing (NGS) result suggested that the proportions of trisomy 8 in different tissues were obviously different; and 0% in amniotic fluid. Last, the chromosomes of the patient and her baby were confirmed using chromosome microarray analysis (CMA), and the results were arr[GRCh37](8) × 3,11p15.5p13(230750-33,455,733) × 2 hmz and normal. This pregnancy woman was trisomy 8 mosaicism, but the phenotypic was normal, and also the fetus was normal. Carefully cytogenetic diagnoses should be performed for prenatal diagnose."}, {"id": "wiki20220301en465_9703", "title": "Genotype-first approach", "score": 0.009433962264150943, "content": "Genotype-first assessment is becoming the standard approach for clinical diagnosis of complex heterogeneous diseases. Microduplication and microdeletion syndromes have a range of characteristics, including intellectual disability and developmental delay, which vary in severity making patients with these syndromes very difficult to diagnose. Since the development of next-generation sequencing technologies, clinicians have been able to use a genotype-first approach to group these patients based on their microdeletion or duplication and document the disease features present in these groups. Chromosomal microarray analysis, in particular, is being used clinically to assist in diagnosing patients with microdeletion and microdulplication syndromes. In diseases, such as Autism spectrum disorder (ASD), where differentiating patients into disease subtype groups based on phenotype is challenging, genotype-first studies allow the classification of patients into subtypes based on their genetics."}, {"id": "pubmed23n1006_22194", "title": "[Reflection of a case misdiagnosed as trisomy 21 syndrome by G-banded chromosomal karyotyping analysis].", "score": 0.009433962264150943, "content": "To emphasize the clinical significance of copy number variations (CNVs) detection by describing a case misdiagnosed as trisomy 21 syndrome by G-banded chromosomal karyotype analysis. A girl with obesity and short stature was diagnosed as trisomy 21 syndrome by G-banded chromosomal karyotype analysis. Considering the discrepancy of her karyotype with her phenotype, genomic CNVs was detected by next-generation sequencing and the result was verified by quantitative PCR (qPCR). A microduplication of 16p11.2: 29 642 339-29 775 631 (133.292 kb) was detected. qPCR assay for QPRT and SPN located in the duplicated region confirmed the finding of CNVs assay. Meanwhile, her parents did not present similar duplication in 16p11.2. The 16p11.2 microduplication was a novel genomic structural variation in the girl, though it may not be associated with her clinical manifestations. Chromosomal microarray or next-generation sequencing-based CNVs detection can accurately determine the origin of small supernumerary marker chromosome and reduce the chance of misdiagnosis."}, {"id": "pubmed23n0988_8785", "title": "[Genotypic and phenotypic analysis of a patient with de novo partial monosomy 18p and partial trisomy 18q].", "score": 0.009345794392523364, "content": "To explore the genetic cause for a patient with intellectual disability, short stature and multiple congenital anomalies, and to correlate the result with the clinical phenotype. Routine karyotyping analysis was carried out on GTG-banded metaphase chromosomes. Single nucleotide polymorphism (SNP) microarray was used to detect microdeletions or microduplications in the patient. Fluorescence in situ hybridization (FISH) was used to ascertain the origin of aberrant chromosomes. The karyotype of the patient was 46,XY,der(18), while both of his parents had a normal karyotype. SNP array identified a 1.23 Mb deletion at 18p11.32-pter (chr18: 136 227-1 370 501, hg19) and a 33.76 Mb duplication at 18q21.1-qter (chr18: 44 250 359-78 013 728, hg19) in the patient. Above finding was confirmed by dual-color FISH with one color for 18p and another for 18q. The patient presented with some common features of 18p deletion and 18q duplication including intellectual disability and growth retardation, in addition with some features of 18p deletion including pectus excavatum, short stature and growth hormone (GH) deficiency. The patient showed progressive improvement of stature with GH therapy. Comparison of patients with previously reported dup(18q)+del(18p) recombinations suggested that, even for patients with similar breakpoints, their phenotypes have ranged from normal to severe and there were no consistent findings. As aberrations involving double chromosomal segments often result in phenotypic variability, it has been difficult to correlate the genotype of our patient with his phenotype."}, {"id": "pubmed23n0902_22109", "title": "[Application of chromosomal microarray analysis for the diagnosis of children with intellectual disability/developmental delay and a normal karytype].", "score": 0.009259259259259259, "content": "To assess the value of chromosomal microarray analysis (CMA) for the diagnosis of children with intellectual disability/developmental delay (ID/DD) but a normal karytype. Peripheral blood samples from 92 ID/DD patients were analyzed with CMA using Affymetrix CytoScan 750K arrays. The results were analyzed by ChAS v3.0 software. Eighteen cases (19.57%) were detected with abnormalities by CMA, among which 10 cases were diagnosed with microdeletion/microduplication syndromes. These included 2 Williams-Beuren syndromes, 2 Angelman syndromes, 2 Russell-Silver syndromes, 1 Smith-Magenis syndromes, 1 Wolf-Hirschhorn syndromes, 1 15q26 overgrowth syndrome and 1 Xq28 (MECP2) duplication syndrome. In addition, 8 cases were diagnosed with pathogenic copy number variations (pCNV). CMA can significantly improve the diagnostic rate for patients with ID/DD, which is of great value for the treatment of such children and guidance of reproduction for their parents. Therefore, CMA should become the first-line diagnostic test for patients with ID/DD."}, {"id": "pubmed23n1057_13999", "title": "A novel 1p33p32.2 deletion involving SCP2, ORC1, and DAB1 genes in a patient with craniofacial dysplasia, short stature, developmental delay, and leukoencephalopathy: A case report.", "score": 0.009174311926605505, "content": "Microdeletion syndromes occur from deletion of 5Mb of a chromosome in approximately 5% of patients with unexplained intellectual disability. Interstitial microdeletions at bands 1p33 and 1p32.2 of the short arm of chromosome 1 are rare and have not been previously reported in relation to disease. We present a case of a 39-month boy with Pierre Robin sequence, development delay/intellectual disability, growth retardation, short stature, leukoencephalopathy, craniofacial dysplasia, and speech delay. The child was referred to the Child health care department in October 2014 for his delayed language development and aggravated aggression. Molecular diagnostic testing with G-band karyotyping was normal but clinical microarray analysis detected a 10 Mb microdeletion at 1p33p32.2. The patient received rehabilitation. Three candidate genes were pinpointed to the deleted area, including ORC1, SCP2, and DAB1. Phenotype-genotype analysis suggested that these three genes are likely to be responsible for the main phenotypes observed in the patient, such as microcephaly, growth retardation, short stature, leukoencephalopathy, and development delay/intellectual disability. The spectrum of phenotypes this case presented with are likely to be caused by 1p33p32.2 deletion which could represent a new microdeletion syndrome."}, {"id": "pubmed23n1046_12231", "title": "[Clinical and genetic analysis of a rare case with mosaic partial trisomy 5p syndrome].", "score": 0.009174311926605505, "content": "To determine the size and origin of a small supernumerary marker chromosome (sSMC) identified in a patient featuring developmental retardation. High-throughput sequencing for copy number variation (CNV-seq) was carried out to delineate the sSMC identified upon G-banded chromosomal karyotyping. The genotype-phenotype correlation was explored by database retrieval and literature analysis. The patient was found to have a karyotype of mos 47,XX,+mar[36]/46,XX[23]. CNV-seq has identified a 18 Mb duplication at 5p14.1-p12 (hg19: 27,399,261-46,083,784)x2.6 with a mosaicism rate of approximately 60%. Patients with mosaic partial trisomy 5p may have extensive clinical manifestations, and the ratio of trisomy 5p cells is correlated with clinical severity of this syndrome."}, {"id": "pubmed23n0793_10570", "title": "A Rare, Recurrent, De Novo 14q32.2q32.31 Microdeletion of 1.1 Mb in a 20-Year-Old Female Patient with a Maternal UPD(14)-Like Phenotype and Intellectual Disability.", "score": 0.00909090909090909, "content": "We present a 20-year-old female patient from Indonesia with intellectual disability (ID), proportionate short stature, motor delay, feeding problems, microcephaly, facial dysmorphism, and precocious puberty who was previously screened normal for conventional karyotyping, fragile X testing, and subtelomeric MLPA analysis. Subsequent genome wide array analysis was performed on DNA from blood and revealed a 1.1 Mb deletion in 14q32.2q32.31 (chr14:100,388,343-101,506,214; hg19). Subsequent carrier testing in the parents by array showed that the deletion had occurred de novo in the patient and that her paternal 14q32 allele was deleted. The deleted region encompasses the DLK1/GTL2 imprinted gene cluster which is consistent with the maternal UPD(14)-like phenotype of the patient. This rare, recurrent microdeletion was recently shown not to be mediated by low copy repeats, but by expanded TGG repeats, flanking the 14q32.2q32.21 deletion boundaries, a novel mechanism of recurrent genomic rearrangement. This is another example how the application of high resolution genome wide testing provides an accurate genetic diagnosis, thereby improving the care for patients and optimizing the counselling for family. "}, {"id": "pubmed23n0886_19605", "title": "A case of 46,XX dysgenesis and marked tall stature; the need for caution in interpreting array comparative genomic hybridization (CGH).", "score": 0.00909090909090909, "content": "Gonadal dysgenesis with an apparently normal 46,XX karyotype is a rare cause of hypergonadotrophic hypogonadism. Tall stature is not a widely recognized association. A 15-year-old girl presented with primary amenorrhoea. Examination showed a non-dysmorphic girl of normal intellect with no breast development (Tanner stage B1P4A1) who was tall compared with her parents: height standard deviation score (SDS) +1.56 vs. midparental height of +0.23 SDS, and slim build (weight -0.13 SDS). Investigations showed a 46,XX karyotype, elevated gonadotropins (FSH 119 and LH 33.7 IU/L), serum estradiol &lt;5 pmol/L, uterine length 3.75 cm with cylindrical shape, and absent ovaries on ultrasound. Initially, a 364055-bp deletion on Xp21.2 was reported on array CGH. However, repeat analysis using BlueGnome CytoChip ISCA 4x180k v2.0 array was normal. With oral ethinyl estradiol induction puberty progressed to B4P4A2 but aged 18.4 years, the patient was remarkably tall with height SDS +2.88, weight SDS +0.97. Caution is needed in interpreting small changes with array CGH, particularly with the older assays. We postulate that the genetic change causing 46,XX gonadal dysgenesis in our patient may have also resulted in unsuppressed somatic growth. More critical height assessment, including parental height measurement, of future patients with 46,XX gonadal dysgenesis is recommended in order to determine whether or not a true association with tall stature may be present in certain cases."}, {"id": "pubmed23n0958_18776", "title": "A 13-year-old girl with 18p deletion syndrome presenting Turner syndrome-like clinical features of short stature, short webbed neck, low posterior hair line, puffy eyelids and increased carrying angle of the elbows.", "score": 0.009009009009009009, "content": "We report a 13-year-old girl with 18p deletion syndrome presenting Turner syndrome-like clinical features. A 13-year-old girl was referred for genetic counseling of Turner syndrome-like clinical features of short stature, short webbed neck, low posterior hair line, puffy eyelids and increased carrying angle of the elbows. The girl also had mild intellectual disability, psychomotor developmental delay, speech disorder, high-arched palate, hypertelorism and mid-face hypoplasia. Cytogenetic analysis of the girl revealed a karyotype of 46,XX,del(18) (p11.2). The parental karyotypes were normal. Array comparative genomic hybridization analysis on the DNA extracted from the peripheral blood revealed a 13.93-Mb deletion of 18p11.32-p11.21 or arr 18p11.32p11.21 (148,993-14,081,858) × 1.0 [GRCh37 (hg19)] encompassing 52 Online Mendelian Inheritance in Man (OMIM) genes including USP14, TYMS, SMCHD1, TGIF1, LAMA1, TWSG1, GNAL and PTPN2. Polymorphic DNA marker analysis revealed a maternal origin of the deletion. Females with Turner syndrome-like clinical features in association with intellectual disability, facial dysmorphism and psychomotor developmental delay should be suspected of having chromosome deletion syndromes."}, {"id": "pubmed23n1020_7797", "title": "Tetrasomy 18p Case Report.", "score": 0.009009009009009009, "content": "Tetrasomy 18p is a rare disorder. It is known to affect about 250 families worldwide. Tetrasomy 18p is also the most common type of isochromosome. Here we report a de novo tetrasomy 18p. The copy number variation of the patient was detected by microarray. Whether the abnormal gene was inherited from the parents was detected by karyotype analysis. Then the source of the chromosome was located by fluorescence in situ hybridization. Finally, we used MLPA technology to validate the results of patient testing. Microarray detection found that patients with 18p11.32p11.21 had duplication, with a copy number of four, which was tetrasomy 18 syndrome. The karyotype results showed 48,XY,+2mar?. Chromosome 18 telomere probe FISH experimental results: 48,XY,+i(18)(p10),+mar.ish. MLPA results showed that the number of chromosome 18 short arm copies is increased. Karyotype analysis results of his mother were 47,XX,+mar. Microarray results showed normal. Karyotype results of his father were normal. This case is de novo case, the patient's marker chromosome may be inherited from his mother, which does not rule out the influence of his mother's marker chromosome on his isochromosome 18."}, {"id": "pubmed23n0936_994", "title": "Novel contiguous gene deletion in peruvian girl with Trichothiodystrophy type 4 and glutaric aciduria type 3.", "score": 0.008928571428571428, "content": "Trichothiodystrophy type 4 is a rare autosomal recessive and ectodermal disorder, characterized by dry, brittle, sparse and sulfur-deficient hair and other features like intellectual disability, ichthyotic skin and short stature, caused by a homozygous mutation in MPLKIP gene. Glutaric aciduria type 3 is caused by a homozygous mutation in SUGCT gene with no distinctive phenotype. Both genes are localized on chromosome 7 (7p14). We report an 8-year-old female with short stature, microcephaly, development delay, intellectual disability and hair characterized for dark, short, coarse, sparse and brittle associated to classical trichorrhexis microscopy pattern. Chromosome microarray analysis showed a 125 kb homozygous pathogenic deletion, which includes genes MPLKIP and SUGCT, not described before. This is the first case described in Peru of a novel contiguous gene deletion of Trichothiodystrophy type 4 and Glutaric aciduria type 3 performed by chromosome microarray analysis, highlighting the contribution and importance of molecular technologies on diagnosis of rare genetic conditions."}, {"id": "pubmed23n1109_14608", "title": "The phenotype and rhGH treatment response of ring Chromosome 15 Syndrome: Case report and literature review.", "score": 0.008928571428571428, "content": "Ring chromosome 15 [r (15)] is an uncommon finding with various clinical manifestations. A common phenotype for these patients has not been established and data on the efficacy of recombinant human growth hormone (rhGH) treatment in patients with r (15) syndrome are limited. One short stature patient in our hospital with r (15) syndrome by whole exome sequencing (WES) and karyotype examination was included. All published r (15) syndrome cases as of March 15, 2021, were searched, and their clinical information was recorded and summarized. One 11.5-year-old female with prenatal and postnatal growth retardation, ventricular septal defect, intellectual disability, downward corners, short fifth metacarpal bone, scattered milk coffee spots, and a right ovarian cyst was included. Her height was 126.9 cm (-3.45 SDS). Karyotype analysis showed 46, XX, r (15). WES revealed a 4.5 Mb heterozygous deletion in the chromosome 15q26.2-q26.3 region, encompassing genes from ARRDC4 to OR4F15. Gonadotrophin-releasing hormone analogue (triptorelin) and rhGH were administered for 6 months. The height has increased 3.8 cm (+0.2SDS) and the calculated growth rate has improved from 4.7 to 7.6 cm/y. The literature review indicated the main clinical manifestations of r (15) syndrome with prenatal and postnatal growth retardation, characteristic craniofacial features, and multisystem abnormalities, and rhGH treatment is beneficial for r (15) syndrome patients with short stature. We delineate the clinical spectrum of r (15) syndrome with the identification of an additional individual and rhGH treatment is beneficial for r (15) syndrome patients with short stature."}, {"id": "Obstentrics_Williams_1778", "title": "Obstentrics_Williams", "score": 0.008880606792936278, "content": "This technique may be used for rapid identiication of a speciic chromosome abnormality and for veriication of suspected microdeletion or duplication syndromes, such as the 22q 11.2 microdeletion described earlier (p. 260). Because of its 1-to 2-day turnaround time, FISH is often selected for cases in which indings may alter pregnancy management. To perform FISH, cells are ixed onto a glass slide, and fluorescent-labeled probes are hybridized to the ixed chromosomes (Figs. 13-11 and 13-12). Each probe is a DNA sequence that is complementary to a region of the chromosome or gene being investigated. If the DNA sequence is present, hybridization is detected as a bright signal visible by microscopy. The number of signals indicates the number of chromosomes or genes of that type in the cell being analyzed. Findings are probe-speciic. Namely, FISH does not provide information on the entire chromosomal complement but merely the chromosomal or gene region of interest."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": true, "char_ranges": [[301, 398]], "word_ranges": [[44, 64]], "text": "option 1 speaks to us of an IgA nephropathy (it is not the case because the deposits are of IgG);"}, "2": {"exist": true, "char_ranges": [[0, 158]], "word_ranges": [[0, 22]], "text": "The diagnostic suspicion for the data given (especially the anti-GBM antibodies and the presence of renopulmonary syndrome) is that of Goodpasture's syndrome."}, "3": {"exist": true, "char_ranges": [[399, 516]], "word_ranges": [[64, 84]], "text": "option 3 is also discarded, because it is not a primary GMN, like the membranous one, but a secondary glomerulopathy;"}, "4": {"exist": true, "char_ranges": [[517, 637]], "word_ranges": [[84, 100]], "text": "option 4 is also false: the initial treatment is with corticosteroids and cyclophosphamide associated to plasmapheresis;"}, "5": {"exist": true, "char_ranges": [[642, 790]], "word_ranges": [[101, 124]], "text": "option 5 is also false, since the damage is not due to circulating immunocomplexes, but to antibodies deposited in the glomerular basement membrane."}}, "full_answer": "The diagnostic suspicion for the data given (especially the anti-GBM antibodies and the presence of renopulmonary syndrome) is that of Goodpasture's syndrome. The rest of the data (seminules in the biopsy, linear IgG deposition) supports the diagnosis. Knowing this, the options are easily discarded: option 1 speaks to us of an IgA nephropathy (it is not the case because the deposits are of IgG); option 3 is also discarded, because it is not a primary GMN, like the membranous one, but a secondary glomerulopathy; option 4 is also false: the initial treatment is with corticosteroids and cyclophosphamide associated to plasmapheresis; and option 5 is also false, since the damage is not due to circulating immunocomplexes, but to antibodies deposited in the glomerular basement membrane. This leaves option 2 as true: as it has been previously said, the treatment would be performed combining corticosteroids, cyclophosphamide and plasmapheresis.", "full_answer_no_ref": "The diagnostic suspicion for the data given (especially the anti-GBM antibodies and the presence of renopulmonary syndrome) is that of Goodpasture's syndrome. The rest of the data (seminules in the biopsy, linear IgG deposition) supports the diagnosis. Knowing this, the options are easily discarded: option 1 speaks to us of an IgA nephropathy (it is not the case because the deposits are of IgG); option [HIDDEN], because it is not a primary GMN, like the membranous one, but a secondary glomerulopathy; option [HIDDEN] the initial treatment is with corticosteroids and cyclophosphamide associated to plasmapheresis; and option [HIDDEN], since the damage is not due to circulating immunocomplexes, but to antibodies deposited in the glomerular basement membrane. This leaves option [HIDDEN] as it has been previously said, the treatment would be performed combining corticosteroids, cyclophosphamide and plasmapheresis.", "full_question": "A 38-year-old man consults for dyspnea and hemoptysis. Blood tests show creatinine 7 mg/dL, urea 250 mg/dL and high titer positive anti-GBM (anti-glomerular basement membrane antibodies). Renal biopsy shows crescents in 75% of the glomeruli and immunofluorescence shows a linear Ig deposition pattern. Which of the following is the correct answer?", "id": 216, "lang": "en", "options": {"1": "It is an IgA nephropathy with acute renal failure.", "2": "Plasmapheresis would be indicated.", "3": "It is a membranous glomerulonephritis.", "4": "Mycophenolate mofetil is the initial treatment of choice.", "5": "Glomerular involvement is caused by the presence of circulating immunocomplexes."}, "question_id_specific": 121, "type": "NEPHROLOGY", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0977_267", "title": "Development of anti-glomerular basement membrane glomerulonephritis during the course of IgA nephropathy: a case report.", "score": 0.01960972796308757, "content": "Anti-glomerular basement membrane (GBM) glomerulonephritis does not usually coexist with another glomerulonephritis such as IgA nephropathy. We present a rare case having a combination of these two diseases, and furthermore, histological evaluation could be performed before and after the development of anti-GBM glomerulonephritis over a period of only10 months. A 66-year-old woman was admitted with complaints of microscopic hematuria and mild proteinuria for the past 3 years. Serum creatinine level was normal at that time. The first renal biopsy was performed. Light microscopy revealed mesangial proliferative glomerulonephritis with fibro-cellular crescents in one out of 18 glomeruli, excluding one global sclerotic glomerulus. Immunofluorescence (IF) showed IgA and C3 deposition in the mesangium. Therefore, the diagnosis was IgA nephropathy. Eight months later, the patient's serum creatinine suddenly rose to 4.53 mg/dL and urinalysis showed 100 red blood cells per high power field with nephrotic range proteinuria (12.3 g/g<subCr</sub). The serological tests revealed the presence of anti-GBM antibody at the titer of 116 IU/mL. Treatments were begun after admission, consisting of hemodialysis, plasma exchange, and intravenous methylprednisolone pulse therapy. At 4 weeks after admission, the second renal biopsy was performed. Light microscopy revealed crescents in 18 of 25 glomeruli, excluding six global sclerotic glomeruli. IF showed linear IgG deposition along the GBM in addition to granular IgA and C3 deposition. Based on these findings, the diagnosis of anti-GBM glomerulonephritis and IgA nephropathy was confirmed. Renal function was not restored despite treatment, but alveolar hemorrhage was prevented. We report a patient with a diagnosis of anti-GBM disease during the course of IgA nephropathy. This case strongly suggests that the presence of autoantibodies should be checked to rule out overlapping autoimmune conditions even in patient who have previously been diagnosed with chronic glomerulonephritis, such as IgA nephropathy, who present an unusually rapid clinical course."}, {"id": "pubmed23n0567_9893", "title": "Patient with antibody-negative relapse of Goodpasture syndrome.", "score": 0.018812930577636458, "content": "Smoking in young men may trigger anti-GBM disease manifesting with hemoptysis. We present a male adolescent in whom hemoptysis was mistaken to be a sign of airway infection for several months and who later on underwent an unusual antibody-negative relapse. The 16-year-old patient had a history of smoking and therapy-refractant hemoptysis and, later, acute macrohematuria with renal insufficiency necessitating hemodialysis (initial creatinine 4.2 mg/ dl). Chest X-ray showed diffuse lung infiltration. Renal biopsy revealed linear IgG deposits along the glomerular basement membrane (GBM) and cellular crescents in 13/16 glomeruli, simultaneously increased anti-GBM antibodies were detected. Thus, anti-GBM glomerulonephritis was diagnosed. After treatment with prednisone, oral cyclophosphamide and plasmapheresis, chest X-ray and hemoptysis improved, but renal failure persisted. Anti-GBM antibodies were negative. 4 weeks later, the patient presented again with a clinical relapse of severe hemoptysis and respiratory insufficiency after smoke exposition. Despite negative anti-GBM antibodies, he was treated similarly to a relapse and after the second course of plasmapheresis the patients' general condition improved and hemoptysis subsided. During the next 10 months the patient was stable with negative antibodies. He was under intermittent hemodialysis until laboratory measurements showed improved renal function. Now, 30 months after the acute episode, the patient is off dialysis for 17 months with stable creatinine values of 1.9 - 2.4 mg/dl, and is currently being treated with antihypertensive medicaments, calcitriol, calciumacetate, natriumhydrogencarbonate and allopurinol. The prognosis of anti-GBM glomerulonephritis depends on serum creatinine and the need of dialysis at initial presentation. In these patients, one-year survival rate is 67% and 5% for kidney function. Of note, despite the unfavorable prognosis in our patient, renal function recovered after 1 year of hemodialysis treatment. It is important to consider that in patients with anti-GBM disease antibody-negative relapses are possible."}, {"id": "pubmed23n1041_23469", "title": "Nephrotic syndrome due to minimal-change disease superimposed on anti-glomerular basement membrane antibody positive glomerulonephritis; a case report.", "score": 0.018672919923571306, "content": "The prognosis for renal function in anti-GBM glomerulonephritis (anti-GBM GN) is extremely poor, and when renal impairment progresses severely, it is difficult to expect improvement. In addition, it is also known that once the disease activity can be controlled by aggressive treatment, its recurrence is rare. We experienced an anti-GBM GN that improved from severe renal dysfunction and relapsed. A possible cause was the superimpose of nephrotic syndrome due to minimal change disease (MCD). A 30-year-old man was admitted to our hospital because of general malaise, fever, oliguria and renal dysfunction. The patient's laboratory data showed serum creatinine as high as 6.6 mg/dl, and severe inflammation (C-reactive protein 20.6 mg/dl). Anti-glomerular basement membrane antibody (anti-GBM Ab) was detected in his serum, which led to the diagnosis of anti-GBM GN. Treatment was initiated with high-dose glucocorticoid (GC) and plasma exchange therapy (PE), and the patient's renal function and oliguria improved rapidly and he was discharged 40 days after admission. Renal biopsy findings showed cellular crescents associated with linear IgG depositions along the glomerular tufts compatible with anti-GBM GN, but only about one-third of the glomeruli was involved, suggesting that it still remains an early stage of the disease. However, 2 months after discharge, he had a relapse and was readmitted due to severe proteinuria with positive anti-GBM Ab. On the second admission, after high-dose GC and PE combined with intravenous cyclophosphamide, and remission was achieved. Despite the relatively minor renal biopsy findings, the patient showed rapid renal dysfunction and relatively rapid improvement with our treatment. Electron microscopy of the renal biopsy tissue showed significant foot process effacement on podocytes in the apparently normal glomeruli, without electron dense deposits. On the basis of clinical course and renal pathology, it is suggested that the present case was a rare complication of an early stage of anti-GBM GN and minimal change nephrotic syndrome. Although the simultaneous development of anti-GBM GN and MCD with anti-GBM antibody is unclear, it might have been precipitated by influenza infection or some unknown factor."}, {"id": "pubmed23n0049_11777", "title": "[A case of anti-GBM nephritis (crescentic glomerulonephritis) associated with membranous nephropathy].", "score": 0.016611524676040806, "content": "We report a case of endstage renal disease due to simultaneous occurrence of membranous nephropathy and crescentic glomerulonephritis associated with anti-GBM antibodies. The patient was a 60-year-old male and was hospitalized for prolonged anorexia and general malaise. On admission, his body temperature was 38.5 degrees C. Urinalysis revealed 3+ proteinuria and the sediment contained abundant erythrocytes. The urea nitrogen was 142.4 mg/dl, the creatinine 19.5 mg/dl, the potassium 6.47 mEq/dl and CRP 10.1 mg/dl. Anti-GBM antibodies were 1000EU/ml. Immediately after initiating hemodialysis, pulse steroid therapy, plasma exchange and continuous heparinization were performed. However, renal function had been impaired and maintenance hemodialysis was required. Histological examination of the renal specimen revealed marked epithelial crescent formation, whereas thickening of basement membrane and mesangial proliferation were not observed. By immunofluorescent staining, both bright linear and fine granular fixation of IgG and fine granular fixations of C3 along the glomerular capillary walls were observed. Electron microscopy showed subepithelial electron lucent deposits and thickening of the glomerular basement membrane, diagnostic of the advanced membranous nephropathy (stage IV)."}, {"id": "pubmed23n0876_3132", "title": "Goodpasture's syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report.", "score": 0.016465494620834426, "content": "Goodpasture's syndrome, a rare disease, is an organ-specific autoimmune disease mediated by anti-glomerular basement membrane antibodies. Its pathology is characterized by crescentic glomerulonephritis with linear immunofluorescent staining for immunoglobulin G on the glomerular basement membrane. Although rare, a few cases with absence of circulating anti-glomerular membrane antibodies have been described. The objective of this clinical case report is to describe and discuss a case of a 27-year-old white man who was hospitalized with a 1-year history of weight loss and a 1-month history of hemoptysis, with aggravation  the day before, having developed dyspnea and cough in the previous 24 hours. An analytical study showed normocytic normochromic anemia with a hemoglobin level of 7.2 g/dL and leukocytosis with normal renal function and coagulation times. A blood transfusion was performed without complications. Chest computed tomography revealed a reticulonodular infiltrate of both lungs. Bronchoscopy showed no apparent lesions. Sputum cultures, rapid urine antigens for Legionella pneumophila and Streptococcus pneumoniae, studies for Influenza, virologic markers and serologic studies for autoimmunity were all negative. At the end of the tenth day his general state deteriorated with fatigue, hematuria, and in 3 days he developed aggravation of renal function with recurrent hemoptysis and anemia. Immunosuppression with daily prednisolone 1 g administered intravenously was initiated. An urgent bronchoscopy showed no lesions. A kidney biopsy showed fibrinoid necrosis and cellular crescents. Immunofluorescence revealed a linear immunoglobulin G deposition compatible with Goodpasture's syndrome. Immunosuppressive therapy with daily cyclophosphamide 120 mg orally was added. Subsequently he was transferred to a referral center at which 21 sessions of plasmapheresis and four sessions of hemodialysis were performed with good response; he currently has no need of hemodialysis. The absence of circulating anti-glomerular basement membrane antibodies in Goodpasture's syndrome adds complexity to the diagnosis creating an unusual setting in a rare disease. In our case a kidney biopsy was essential for diagnosis and clinical approach. Studies have shown that early aggressive therapy leads to an improved prognosis. Physicians should consider tissue diagnoses such as bronchoscopy and kidney biopsy in pulmonary renal syndrome."}, {"id": "pubmed23n0564_11850", "title": "Anti-glomerular basement membrane antibody disease with granulomatous lesions on renal biopsy.", "score": 0.016281512605042014, "content": "We present the case of a 56-year-old woman with anti-glomerular basement membrane (anti-GBM) antibody disease accompanied by granulomatous reaction in the kidney. Three months prior to admission to our kidney center, she had suffered from interstitial pneumonia and had a slightly elevated level of MPO-ANCA (13 EU). Her serum level of creatinine was normal (0.72 mg/dl) but proteinuria (1+) and hematuria (2+, 1-4/HF) were present. She was admitted to our hospital because of general fatigue, loss of appetite, high fever (over 38.5 degrees C) and a rapid decline in renal function (creatinine 8.50 mg/dl). Hemodialysis therapy was started immediately after admission. The serological study was negative for MPO-ANCA and PR3-ANCA but positive for anti-GBM antibody (139 EU). Renal biopsy demonstrated necrotizing glomeruli, cellular crescents and grauloma formation with multinucleated giant cells. Immunofluorescence microscopy revealed linear staining of IgG and C3. We diagnosed graulomatous, crescentic and necrotizing glomerulonephritis, patho-logically. She was diagnosed as having anti-GBM antibody disease because alveolar hemorrhage was absent. Steroid therapy including methylprednisolone pulse therapy (500 mg/day, 3 days) and 2 courses of plasma exchange were effective in reducing the fever, anti-GBM antibody titer and C-reactive protein level. Her renal function recovered and she was able to quit hemodialysis therapy 68 days after the start of hemodialysis and she has shown no signs of pulmonary alveolar hemorrhage to date. The present case suggests that intensive therapy may restore renal function in anti-GBM disease even though renal function was sufficiently damaged and required hemodialysis therapy and active pathological changes were observed in renal biopsy specimens."}, {"id": "pubmed23n1052_1829", "title": "Goodpasture syndrome manifesting as nephrotic-range proteinuria with anti-glomerular basement membrane antibody seronegativity: A case report.", "score": 0.016255534471853256, "content": "The Goodpasture syndrome is an extremely rare disease, with renal and pulmonary manifestations, and is mediated by anti-glomerular basement membrane (anti-GBM) antibodies. Renal pathological changes are mainly characterized by glomerular crescent formation and linear immunofluorescent staining for immunoglobulin G on the GBM. There are few reports on the atypical course of the syndrome involving serum-negative anti-GBM antibodies. Therefore, we present a case of Goodpasture syndrome that presented with nephrotic-range proteinuria and was seronegative for anti-GBM antibodies. A 38-year-old Chinese man presented with a lung lesion that was discovered by physical examination a month prior to presentation. The chief concern was occasional hemoptysis without fever, cough, chest pain, and edema. Laboratory testing revealed that the urinary protein level and urine erythrocyte count were 7.4 g/24 hours and 144/high-power field (HPF), respectively. Serological testing for anti-GBM antibodies was negative. Chest computed tomography revealed multiple exudative lesions in both lungs, indicating alveolar infiltration and hemorrhage. Electronic bronchoscopy and pathological examination of the alveolar lavage fluid indicated no abnormalities. However, kidney biopsy suggested cellular crescent formation and segmental necrosis of the globuli, with linear IgG and complement C3 deposition on the GBM. These findings were consistent with the diagnosis of anti-GBM antibody nephritis. The patient underwent 7 sessions of double filtration plasmapheresis. He was also administered with intravenous methylprednisolone and cyclophosphamide. After renal function stabilization, he was discharged under an immunosuppressive regimen comprising of glucocorticoids and cyclophosphamides. Three months later, follow-up examination revealed that the 24-hour urine protein had increased to 13 g. Furthermore, the urine erythrocyte count was 243/HPF. After a 6-month follow-up, the patient achieved partial remission, with a proteinuria level of 3.9 g/24 hours and a urine erythrocyte count of 187/HPF. This extremely rare case of Goodpasture syndrome manifested with seronegativity for anti-GBM antibodies and nephrotic-range proteinuria. Our findings emphasize the importance of renal biopsy for the clinical diagnosis of atypical cases. Furthermore, because renal involvement achieved only partial remission despite therapy, early detection and active treatment of the Goodpasture syndrome is necessary to improve the prognosis of patients."}, {"id": "pubmed23n0722_2188", "title": "An unusual case of IgA-mediated anti-glomerular basement membrane disease.", "score": 0.016103059581320453, "content": "Anti-glomerular basement membrane (GBM) disease is mediated by circulating autoantibodies, principally IgG, targeted at the type IV collagen of GBM. The IgA variant of anti-GBM disease has rarely been described. We report a 65-year-old man with uremia, undergoing hemodialysis, who was referred because of hemoptysis. A chest X-ray showed diffuse infiltration in the right lung field. Laboratory data were remarkable for renal failure, anemia, and thrombocytopenia. Furthermore, laboratory evidence of microangiopathic hemolytic anemia was present. A kidney biopsy revealed diffuse crescentic glomerulonephritis. Circulating IgA anti-GBM antibody was found, as well as the presence of significant IgA deposition in a linear pattern along the GBM, suggesting an anti-GBM antibody-mediated disease. The patient was treated with plasmapheresis and pulse steroid therapy, which resulted in an immediate improvement in the pulmonary hemorrhage and hematological abnormalities. However, the patient did not regain renal function and remained on hemodialysis. "}, {"id": "pubmed23n0633_19109", "title": "[Case of rapidly progressive glomerulonephritis with anti-glomerular basement membrane antibody in the course of MPO-ANCA-associated pachymeningitis].", "score": 0.015592767867977187, "content": "A 56-year-old female developed rapidly progressive glomerulonephritis in the course of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated pachymeningitis that had been found four years previously. On admission, her serum creatinine increased from 0.8 mg dL to 1.84 mg dL and to 3.66 mg dL every 3 to 4 weeks. Urinalysis revealed that urinary protein excretion was 1.25 g day and 3+ hematuria. MPO-ANCA titer was found to be 50 EU and anti-glomerular basement membrane (GBM) antibody was also elevated to as high as 174 EU. Renal pathology revealed cellular to fibrocellular crescents in 21 out of 23 glomeruli with interstitial inflammation and fibrosis. Immunohistochemistry with anti IgG antibody showed linear staining along the glomerular capillary walls. Following plasma exchange and methylprednisolone pulse therapy, oral prednisolone at a dose of 50 mg day was instituted, but without significant effect. Subsequent cyclophosphamide pulse therapy was effective, resulting in the stabilization of serum creatinine at 2 mg dL and disappearance of urine abnormalities. In addition, the MPO-ANCA titer and anti-GBM antibody titer of the patient decreased to within the normal range in one month and three months, respectively. Pulmonary lesions were not found throughout the course. Recently the emergence of anti-GBM antibody-associated crescentic glomemrulonephritis in the course of MPO-ANCA-associated vasculitis has increasingly been reported. Accumulation of such cases may unravel the pathogenesis of these diseases. one month and three months, respectively. Pulmonary lesions were not found throughout the course. Recently the emergence of anti-GBM antibody-associated crescentic glomemrulonephritis in the course of MPO-ANCA-associated vasculitis has increasingly been reported. Accumulation of such cases may unravel the pathogenesis of these diseases."}, {"id": "pubmed23n0348_15723", "title": "[A case of Goodpasture's syndrome with massive pulmonary hemorrhage ameliorated by cyclophosphamide pulse therapy].", "score": 0.015332099051145569, "content": "A 22-year-old woman was admitted to our hospital for evaluation of fever, renal dysfunction, and a 3-month-history of macrohematuria. Laboratory evaluation revealed proteinuria (1.8 g/day), hypoproteinemia, microcytic microchromic anemia, renal failure (blood urea nitrogen 30.3 mg/dl, serum creatinine 4.0 mg/dl), and positive serum antiglomerular basement membrane (anti-GBM) antibody. Renal biopsy revealed cellular crescents in all 8 glomeruli and partial rupture of the GBM. The interstitium showed severe inflammatory cell infiltration. Immunofluorescent examination revealed linear deposits of IgG and C3 along the GBM. Pulmonary biopsy revealed linear deposits of IgG along the alveolar basement membrane in the immunofluorescent examination. A diagnosis of Goodpasture's syndrome was made because all of the diagnostic criteria were fulfilled. After admission, the patient's renal function deteriorated rapidly. Hemodialysis was started, and the patient was treated with methylprednisolone pulse therapy and oral prednisolone with double filtration plasma pheresis (DFPP). However, her renal function did not improve. On the 30th hospital day, she showed hemoptysis, and a chest X-ray and CT revealed massive bilateral pulmonary hemorrhage. Despite treatment with pulsed methylprednisolone, oral prednisolone (80 mg/day), and DFPP, the pulmonary hemorrhage improved only transiently, worsening again 5 days later. Cyclophosphamide pulse therapy was administered. After this treatment, the patient's pulmonary manifestations and pulmonary hemorrhage improved. At the present time she is on maintenance dialysis therapy without pulmonary manifestations. These findings suggest that cyclophosphamide pulse therapy is effective against Goodpasture's syndrome with massive pulmonary hemorrhage showing resistance to other conventional therapy."}, {"id": "pubmed23n0720_8401", "title": "IgA variant of anti-glomerular basement membrane glomerulonephritis associated with pulmonary hemorrhage and microangiopathic hemolytic anemia.", "score": 0.015272938443670152, "content": "A 70-year-old man with uremia was referred because of hemoptysis. A chest X-ray showed diffuse infiltration in the right lung field. Laboratory data were remarkable for renal failure, anemia, and thrombocytopenia. Furthermore, laboratory evidence of microangiopathic hemolytic anemia was present. A kidney biopsy revealed diffuse crescentic glomerulonephritis with linear staining of IgA along the glomerular basement membrane (GBM). No thrombotic microangiopathy was noted on renal biopsy. Circulating IgG anti-GBM antibody was not detected, and IgA anti-GBM antibody was not tested. The patient was treated with plasmapheresis and pulse steroid therapy, which resulted in an immediate improvement in the pulmonary hemorrhage and hematological abnormalities. However, the patient did not regain renal function and remained on hemodialysis."}, {"id": "pubmed23n0252_6472", "title": "[Acute kidney failure in glomerulonephritis and in angiitis].", "score": 0.015008068854222699, "content": "Some cases of postinfectious glomerulonephritis initially are oligoanuric. Renal biopsy is essential to distinguish the purely endocapillary and exsudative form from that with endo- and extracapillary proliferation. The former characterises spontaneously regressive poststreptococcal glomerulonephritis, which is now rare, whilst the latter is caused by various strains of gram-positive and gram-negative strains and entails a much less favourable outcome in terms of renal and patient survival. Acute renal failure is a common complication of angiitis, mainly polyarteris nodosa (PAN) and Wegener granulomatosis. A third variety of crescentic glomerulonephritis is due to anti-glomerular basement membrane (GBM) antibodies, with or without pulmonary haemorrhage. Glomerular immunofluorescence discloses a typical pattern of linear IgG deposits along the GBMs. Treatment based on plasma exchanges, corticosteroids and alkylating agents can prevent end stage renal failure when undertaken early. Other glomerulopathies may be complicated with acute renal failure, including haematuric forms of IgA nephropathy."}, {"id": "pubmed23n0308_16337", "title": "[A case of anti-basement membrane (BM) mediated disease presenting renal and pulmonary symptoms by divergent timing].", "score": 0.01466181506849315, "content": "A case of 49-year-old man with anti-GBM antibody and who manifested pulmonary and renal symptoms at divergent times. Thirty-six years previously, renal disease with unneglectable degree of proteinuria was noticed. One month before admission, he was found by chance to have elevated serum creatine (Scr); 3.4 mg/dl. At admission, his Scr was 13.7 mg/dl and Hb 12.7 g/dl, TP 5.2 g/dl with 3+ proteinuria and no glucosuria. He was a heavy smoker and remained so while admitted. Renal biopsy presented fibrocellular crescents in 100% of glomeruli with striking tubulointerstitial involvement. Immunofluorescence showed linear IgG deposition along the glomerular capillary wall. Hemodialysis was instituted, and after 13 hospital days, anti-GBM antibody at admission was high at 128 U, with negative PANCA. Plasmapheresis was also performed, but on the next day pulmonary hemorrhage occurred with a concomitant rise of anti-GBM to 250 U. Thus, steroid pulse therapy was conducted in combination with plasmapheresis. Pulmonary hemorrhage subsided along with lowering of anti-GBM (48 U), but renal failure persisted. The patient died of septicemia. Based on the clinical course of the case, the term \"anti-BM mediated disease\" may more properly delineate the entity of the disease rather than the classical eponym \"Goodpasture's disease\" which requires coexistence of pulmo- and renal manifestations for definition."}, {"id": "pubmed23n0315_8265", "title": "[An autopsy case of Goodpasture syndrome preceded with membranous glomerulonephritis].", "score": 0.014644569522618304, "content": "Goodpasture syndrome (GS) is an autoimmune disorder characterized by the association of pulmonary hemorrhage and rapidly progressive glomerulonephritis. The pathogenesis of GS is still unknown, but was shown to be the result that antibodies directed against glomerular basement membrane (GBM) antigens could injure both glomerular and pulmonary alveolar basement membrane. And membranous glomerulonephritis (MGN) is a glomerular disease characterized by epimembranous immune deposits and basement membrane thickening. MGN typically presents with the onset of nephrotic syndrome, but it often presents with only asymptomatic proteinuria. We reported an autopsy case of GS preceded with MGN. A 70-year-old man was admitted to our hospital with acute renal failure in May 2, 1996. Percutaneous renal biopsy demonstrated a crescentic glomerulonephritis associated with MGN and linear immunofluorescent staining of the basement membrane with antibodies to IgG. Two weeks later on admission he began to develop slight hemoptysis and chest X-ray showed pulmonary hemorrhage, Furthermore, his serum anti-GBM antibodies titer was very high. He was diagnosed as GS associated with MGN and treated with plasma exchange, glucocorticoid, and cyclophosphamide. Though his symptom was improved for intensive support, he suddenly died on June 22. Autopsied lungs showed focal pulmonary hemorrhage, but were not considered to be life-threatening. The cause of the death remained unclear."}, {"id": "pubmed23n0328_12103", "title": "[An experience of treatment of double positive myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) and anti-glomerular basement membrane antibodies in Goodpasture's syndrome onset of crescentic glomerulonephritis].", "score": 0.014438502673796792, "content": "A 68-year-old woman was admitted to Kinki University Hospital because of progressive renal failure. She had been well until two months before admission. Laboratory data were as follows: serum creatinine 4.1 mg/dl, BUN 69 mg/dl, MPO-ANCA 33 EU, anti-glomerular basement membrane antibodies (AGBMA) 118 U. Histological findings showed cellular and fibrocellular crescents in many glomeruli. Therefore, we diagnosed rapidly progressive glomerulonephritis (RPGN) due to MPO-ANCA and anti-GBM associated renal disease. The patient was started on prednisolone and double filtration plasmapheresis (DFPP) therapy. Subsequently, the values of MPO-ANCA and AGBMA decreased. However, the patient's condition suddenly worsened and she died of interstitial pneumonia. Autopsy examination revealed crescentic glomerulonephritis and alveolar hemorrhage with linear deposition of IgG along the glomerular and alveolar capillary walls by immunofluorescence studies. We considered this to be a rare case of Goodpasture's syndrome associated with not only anti-GBM antibodies, but also MPO-ANCA."}, {"id": "pubmed23n1140_22741", "title": "A Case Report of Crescentic Glomerulonephritis With Positive Serum Anti-glomerular Basement Membrane Without Linear Glomerular Basement Membrane Immunofluorescent Staining.", "score": 0.014162194394752534, "content": "Anti-glomerular basement membrane (anti-GBM) disease is an autoimmune disorder characterized by the production of circulating immunoglobulin G (IgG) antibodies that affect the kidneys and lungs, mainly in the form of rapidly progressive crescentic glomerulonephritis and pulmonary hemorrhage. Typically diagnosed on tissue biopsy, findings mainly include glomerular crescent formation, bright linear staining of GBM for IgG on direct immunofluorescence (IF), and the serologic presence of circulating anti-GBM antibodies. Variation in the laboratory results, where histological findings of linear IgG IF staining were present in the absence of circulating anti-GBM antibodies, have recently led to the use of the term \"atypical anti-GBM disease,\" which usually has a distinct benign clinical outcome as compared to typical anti-GBM disease. We report a case of a middle-aged woman who presented with renal failure without lung involvement. Upon further investigation, the patient was found to have strongly positive serum anti-GBM antibodies, but the tissue biopsy did not show typical findings of the anti-GBM disease. The patient showed modest improvement after multiple sessions of plasmapheresis and steroids, with stabilization of her renal parameters after the initial response. In our case, we will address the possibilities of the discrepancies between the serological and histopathological findings."}, {"id": "pubmed23n0302_5553", "title": "[Goodpasture syndrome: treatment initiation with plasmapheresis before histologic diagnostic verification].", "score": 0.01410105757931845, "content": "A 28 years old male patient presented, after a history of previous recurrent hemoptysis, with diffuse bilateral air space consolidation at chest radiography (CXR). Within 48 hours, partial respiratory insufficiency developed and required intubation. On a clinical and roentgenographic basis, the diagnosis of a Goodpasture syndrome was suspected. Plasmapheresis and immunosuppressive therapy with prednisone and cyclophosphamide were started immediately. Three days after admission, macrohematuria developed and serum creatinine began to rise to a maximum of 3.9 mg/dl. Totally, 13 plasmaphereses were performed within 27 days. Clinical, laboratory and radiological findings improved markedly. 30 days after admission, the patient was discharged and followed on an outpatient basis. Serum creatinine eventually decreased to 1.1 mg/dl. Initially, circulating antibodies against glomerular basement membrane (GBM) were positive, controls remained negative. Renal biopsy was performed after the acute phase and showed glomerulonephritis and linear immunoglobulin deposition along the GBM. Radiologic findings at CXR and high resolution computed tomography are demonstrated."}, {"id": "pubmed23n0736_1648", "title": "Anti-glomerular basement membrane glomerulonephritis with subsequent pulmonary hemorrhage in the course of pulmonary tuberculosis.", "score": 0.013941513650053434, "content": "A 66-year-old man with uremia and on hemodialysis was referred to our hospital because of hemoptysis. A chest radiograph showed diffuse infiltration in the right lung field. Laboratory data were remarkable for renal failure accompanied by hematuria and proteinuria. A kidney biopsy revealed diffuse crescentic glomerulonephritis with linear staining of IgG along the glomerular basement membrane (GBM). Circulating IgG anti-GBM antibody was not detected. Because the findings of renal biopsy suggested anti-GBM disease, the patient was treated with plasmapheresis and pulse steroid therapy, which resulted in a rapid resolution of his pulmonary symptoms and chest radiograph abnormalities. However, sputum culture submitted on admission yielded Mycobacterium tuberculosis 3 weeks later. Therefore, immunosuppressive agents were discontinued and antituberculous agents were administrated. No relapse of pulmonary hemorrhage occurred during the next 1-year period of follow-up, but the patient did not regain renal function and remained on hemodialysis."}, {"id": "pubmed23n0719_7650", "title": "Mesangial IgA deposits indicate pathogenesis of anti-glomerular basement             membrane disease.", "score": 0.013865065751858205, "content": "Anti-glomerular basement membrane (anti-GBM) disease is characterized by             crescentic glomerulonephritis with immunoglobulin G (IgG) autoantibodies to the             non-collagenous (NC1) domain of α3(IV) collagen presenting along the GBM. The             patient clinically manifests with rapidly progressive glomerulonephritis (RPGN)             with pulmonary hemorrhage (Goodpasture syndrome). In rare cases, other immunocomplexes             of IgA or IgM are involved, but their specificities have not been determined.             We report a rare case of a 31-year-old female who was diagnosed as having anti-GBM             disease with extensive IgA deposits in the mesangium. This patient presented heavy             hematuria, proteinuria with increasing creatinine, but no lung hemorrhage. Renal             biopsy showed crescentic glomerulonephritis (type Ⅰ) with strong IgA (3+) as lump             and branch shape. Therapies with pulse methylprednisolone, plasmapheresis and             cyclophosphamide administration were less effective. This case is different from             the present type Ⅰ crescentic glomerulonephritis and the specificity of IgA deposits             may implicate the pathogenesis of anti-GBM disease."}, {"id": "pubmed23n1121_6649", "title": "Anti-glomerular Basement Membrane Disease: A Rare Case Report of Changing Clinical Phenotype and Atypicalities.", "score": 0.013586592178770951, "content": "A man in his late 20s, a smoker, presented with nephrotic-range proteinuria and mild renal failure. He had no macroscopic hematuria or decreased urine output. Kidney biopsy was done which revealed a surprising diagnosis of anti-glomerular basement membrane (anti-GBM) disease. He was started on intravenous methylprednisolone, plasma exchanges, and cyclophosphamide. His anti-GBM antibody was, however, weak positive. After five sessions of plasma exchange, he was discharged with a negative anti-GBM antibody. The patient defaulted drugs and presented with rapidly progressive renal failure and hemoptysis after 1½ months. The patient was started on intravenous methylprednisolone, hemodialysis, plasma exchanges, and cyclophosphamide. Repeat biopsy after stabilization was suggestive of anti-GBM disease with fibrocellular crescents. Anti-GBM antibody was negative. Although the patient presented with an estimated glomerular filtration rate of 10 mL/min/1.73 m<sup2</sup and fibrocellular crescents, the patient improved with treatment and was discharged with a serum creatinine of 2.2 mg/dL. This patient had two presentations: one with nephrotic-range proteinuria and mild renal failure, revealing anti-GBM disease on biopsy, and the second with rapidly progressing renal failure which improved with treatment. There were many atypical features in his presentation. Nonabstinence from smoking might be a triggering factor for the second episode. The pathological antibodies may be against a nonconventional epitope or poorly complement fixing, resulting in negative anti-GBM antibody and good recovery in spite of severe renal failure."}, {"id": "pubmed23n0749_13675", "title": "[A case of rapidly progressive glomerulonephritis with anti-glomerular basement membrane antibody in the course of MPO-ANCA positive interstitial pneumonia].", "score": 0.013245863279291326, "content": "A 78-year-old man developed rapidly progressive glomerulonephritis (RPGN) in the course of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-positive UIP that had been found four years previously. When UIP was diagnosed, the MPO-ANCA titer was low and urine was negative for proteinuria and hematuria. On admission, his serum creatinine increased to 16.89 mg/dL and hemoglobin decreased to 5.2 g/dL. Urinalysis revealed that urinary protein excretion was 0.423 g/day and hematuria (30-40/HPF). The MPO-ANCA titer increased to 95.6 U/mL and anti-glomerular basement membrane (GBM) antibody titer elevated to 140 EU. Renal pathology revealed cellular crescents in 10 out of 11 glomeruli excluding two global sclerotic glomeruli. Immunofluorescence showed heavy linear deposits of IgG and C3 along the GBM. Treatments were begun after admission with hemodialysis and intravenous methylprednisolone pulse therapy, oral prednisolone at the dose 30 mg/day. Both MPO-ANCA and anti-GBM antibody were within the normal range after four months. However, the renal function was not restored despite treatment and he died of pulmonary infectious disease after six months from the onset of RPGN. Recently, many cases of RPGN with both MPO-ANCA and anti-GBM antibody have been reported. In this case, persistent UIP-associated MPO-ANCA appeared to have triggered RPGN by anti-GBM antibody."}, {"id": "pubmed23n1021_14322", "title": "Crescentic glomerulonephritis in children.", "score": 0.012348883779574278, "content": "To date, there is insufficient knowledge about crescentic glomerulonephritis (cGN), the most frequent immunologic cause of acute kidney injury in children. Over a period of 16 years, we retrospectively analyzed kidney biopsy results, the clinical course, and laboratory data in 60 pediatric patients diagnosed with cGN. The underlying diseases were immune complex GN (n = 45/60, 75%), including IgA nephropathy (n = 19/45, 42%), lupus nephritis (n = 10/45, 22%), Henoch-Schoenlein purpura nephritis (n = 7/45, 16%) and post-infectious GN (n = 7/45, 16%), ANCA-associated pauci-immune GN (n = 10/60, 17%), and anti-glomerular basement-membrane GN (n = 1/60, 2%). Patient CKD stages at time of diagnosis and at a median of 362 days (range 237-425) were CKD I: n = 13/n = 29, CKD II: n = 15/n = 9, CKD III: n = 16/n = 7, CKD IV: n = 3/n = 3, CKD V: n = 13/n = 5. Course of cGN was different according to class of cGN, duration of disease from first clinical signs to diagnosis of cGN by biopsy, percentage of crescentic glomeruli, amount of tubular atrophy/interstitial fibrosis and necrosis on renal biopsy, gender, age, nephrotic syndrome, arterial hypertension, dialysis at presentation, and relapse. Forty-eight/60 children were treated with ≥ 5 (methyl-) prednisolone pulses and 53 patients received oral prednis(ol)one in combination with mycophenolate mofetil (n = 20), cyclosporine A (n = 20), and/or cyclophosphamide (n = 6), rituximab (n = 5), azathioprine (n = 2), tacrolimus (n = 1), and plasmapheresis/immunoadsorption (n = 5). The treatment success of cGN is dependent on early diagnosis and aggressive therapy, as well as on the percentage of crescentic glomeruli on renal biopsy and on the underlying type of cGN. CsA and MMF seem to be effective alternatives to cyclophosphamide."}, {"id": "pubmed23n1163_21737", "title": "A case report of atypical anti-glomerular basement membrane disease.", "score": 0.011628300037183518, "content": "Anti-glomerular basement membrane (anti-GBM) disease is characterized by crescentic necrotizing glomerulonephritis, with linear deposits of immunoglobulin G (IgG) in the GBM. Classic anti-GBM disease is clinically associated with rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. Some patients have a better renal prognosis and milder symptoms than those with classic anti-GBM disease, which is termed atypical anti-GBM disease. A 43-year-old Japanese woman was admitted to our hospital complaining of hematuria that had persisted for more than one month. Serological examination revealed negativity for anti-nuclear, anti-neutrophilic cytoplasmic, and anti-GBM antibodies. However, renal biopsy showed cellular crescents. Immunofluorescence revealed strong diffuse linear capillary loop staining for IgG. An indirect immunofluorescence antibody method was performed by applying the patient serum to normal kidney tissue to confirm the presence of autoantibodies binding to the GBM. Using this method, anti-GBM antibodies were detected. The patient was treated with high-dose steroids, cyclophosphamide, and plasma exchange. Aggressive treatment resolved proteinuria and hematuria and improved renal function. Renal biopsy is crucial in the diagnosis of anti-GBM disease, especially when serological tests are negative. Accurately identifying the presence of anti-GBM disease is important to initiate optimal treatment."}, {"id": "pubmed23n1004_12214", "title": "Concurrent Anti-glomerular Basement Membrane Nephritis and IgA Nephropathy.", "score": 0.011403508771929825, "content": "Anti-glomerular basement membrane (GBM) nephritis is characterized by circulating anti-GBM antibodies and crescentic glomerulonephritis (GN) with deposition of IgG along the GBM. In a limited number of cases, glomerular immune complexes have been identified in anti-GBM nephritis. A 38-year-old female presented azotemia, hematuria, and proteinuria without any pulmonary symptoms. A renal biopsy showed crescentic GN with linear IgG deposition along the GBM and mesangial IgA deposition. The patient was diagnosed as concurrent anti-GBM nephritis and IgA nephropathy. Therapies with pulse methylprednisolone and cyclophosphamide administration were effective. Concurrent cases of both anti-GBM nephritis and IgA nephropathy are rare among cases of anti-GBM diseases with deposition of immune complexes. This rare case of concurrent anti-GBM nephritis and IgA nephropathy with literature review is noteworthy."}, {"id": "pubmed23n0534_4424", "title": "[A case of anti-GBM-antibody positive rapidly progressive glomerulonephritis who was weaned from hemodialysis after combination therapy with steroid and plasmapheresis].", "score": 0.011295928500496523, "content": "We report an anti-GBM antibody-positive crescentic glomerulonephritis patient who benefitted from maintenance hemodialysis 4 months after the initial treatment, which included steroid pulse therapy and plasma exchange. A-29-year-old male was referred to our hospital because of high fever, abnormal urinary findings (leukocytes 3+, protein 2+, occult blood 3+) and a moderate degree of azotemia(S-Cr 2.9 mg/dl). C-reactive protein (CRP) was 18.9 mg/dl and antibiotics were administered intravenously for 7 days under the diagnosis of pyelonephritis. High fever persisted, however, and S-Cr increased to 9.2 mg/dl even though a sufficient volume of urine was maintained. Blood and urine cultures were negative for bacteria. A kidney biopsy was performed and cellular crescents were observed around the glomeruli. No abnormal finding was observed in the lung and the nasopharyngeal region. To treat the crescentic glomerulonephritis, steroid and cyclophosphamide were administered while hemodialysis was carried out simultaneously. Although P-ANCA and C-ANCA were negative, anti-GBM antibody was proven to be positive thereafter (169 U) and six sessions of plasmapheresis were additionally performed to remove the antibody. Two months after the last plasmapheresis, the reduced urine volume (300 ml/day) gradually returned to normal. Hemodialysis was terminated because the S-Cr concentration reached a plateau at 4 mg/dl. Repeated biopsy revealed marked glomerulosclerosis, hence hypertension treatment and a low protein diet were ordered. In conclusion, residual renal function might improve even after 4 months of hemodialysis in cases of intensively treated anti-GBM-positive crescentic glomerulonephritis, though consecutive renoprotective therapy is required."}, {"id": "wiki20220301en146_15456", "title": "Rapidly progressive glomerulonephritis", "score": 0.011266192300675059, "content": "Classification RPGN can be classified into three types, based upon the immunofluorescence patterns: Type I Accounting for approximately 20% of RPGN, type I RPGN, also called anti-GBM glomerulonephritis, is characterized by the presence of autoantibodies directed against type IV collagen (specifically, the noncollagenous region of its α3 chain) in the glomerular basement membrane (GBM). Some cases are associated with antibodies directed against the basement membrane of lung alveoli, producing Goodpasture syndrome. The majority of type I disease, however, features anti-GBM antibodies alone; these cases are considered idiopathic. Type II Characterized by deposition of immune complexes in glomerular tissues, type II RPGN accounts for 25% of cases. Any immune complex disease—including systemic lupus erythematosus, acute proliferative glomerulonephritis, Henoch–Schönlein purpura, and IgA nephropathy—that involves the glomerulus may progress to RPGN if severe enough."}, {"id": "pubmed23n1032_11935", "title": "Atypical Anti-Glomerular Basement Membrane Disease With Diffuse Crescentic Membranoproliferative Glomerulonephritis: Case Report and Review of Literature.", "score": 0.011258697027197976, "content": "Anti-glomerular basement membrane (anti-GBM) disease occurs in fewer than two cases per million population. Patients usually present with features of rapidly progressive glomerulonephritis (RPGN) with or without pulmonary involvement. Anti-GBM disease is classically diagnosed by both demonstrating GBM linear immunofluorescence staining on kidney biopsy and detecting anti-GBM antibodies in serum. More than 90% of patients with anti-GBM disease either become dialysis-dependent or die if left untreated. Here, we report a 37-year-old man who presented with bilateral lower limb edema, hypertension, acute kidney injury (creatinine of 212 μmol/L), microscopic hematuria, and nephrotic range proteinuria (15 g/day). His kidney biopsy showed diffuse crescentic membranoproliferative glomerulonephritis and bright linear staining of GBM by immunoglobulin G consistent with anti-GBM disease; however, serum anti-GBM antibodies were negative. The patient was diagnosed with atypical anti-GBM disease and treated aggressively with intravenous pulse steroids, plasmapheresis, oral cyclophosphamide, and oral prednisolone with significant improvement in kidney function and proteinuria. Atypical anti-GBM disease should be considered in patients presenting with RPGN, even in the absence of serum anti-GBM antibodies. Early diagnosis and aggressive treatment in such cases are warranted to prevent irreversible kidney damage as the course of the disease might not be as benign as previously thought."}, {"id": "pubmed23n0916_15442", "title": "Effectiveness of Plasmapheresis in a Patient with Anti-glomerular Basement Membrane Antibody Glomerulonephritis with Advanced Kidney Dysfunction.", "score": 0.011146299056135123, "content": "Patients with anti-glomerular basement membrane antibody glomerulonephritis (anti-GBM GN) have severe kidney dysfunction, leading to end-stage renal disease. The effect of plasmapheresis and immunosuppressive treatment in patients with severe glomerular changes is controversial. A 62-year-old man was admitted with rapidly progressive glomerulonephritis and diagnosed with anti-GBM GN. He required hemodialysis. All glomeruli in the kidney biopsy specimen had cellular crescents without fibrotic changes, suggesting reversible damage. He was treated with plasmapheresis until the anti-glomerular basement membrane antibodies disappeared. His kidney function recovered, and dialysis was able to be discontinued. Frequent plasmapheresis in patients with dialysis-dependent anti-GBM GN may improve the kidney prognosis."}, {"id": "wiki20220301en146_15455", "title": "Rapidly progressive glomerulonephritis", "score": 0.010868629476584022, "content": "Diagnosis Serum analysis often aids in the diagnosis of a specific underlying disease. The presence of anti-glomerular basement membrane (GBM) antibodies suggests type I RPGN; antinuclear antibodies (ANA) may support a diagnosis of systemic lupus erythematosus and type II RPGN; and type III and idiopathic RPGN are frequently associated with anti-neutrophil cytoplasmic antibodies (ANCA)-positive serum. Impaired kidney function in an individual who has had the condition for fewer than three months is characteristic of RPGN. An ultrasonographic examination of the abdomen should be obtained. Although the presence of sediment in the urine on examination can indicate proliferative glomerulonephritis, many cases of rapidly progressive glomerulonephritis need a renal biopsy to make a diagnosis. Classification RPGN can be classified into three types, based upon the immunofluorescence patterns:"}, {"id": "wiki20220301en146_15788", "title": "Membranoproliferative glomerulonephritis", "score": 0.009829059829059829, "content": "Membranoproliferative glomerulonephritis (MPGN) is a type of glomerulonephritis caused by deposits in the kidney glomerular mesangium and basement membrane (GBM) thickening, activating complement and damaging the glomeruli. MPGN accounts for approximately 4% of primary renal causes of nephrotic syndrome in children and 7% in adults. It should not be confused with membranous glomerulonephritis, a condition in which the basement membrane is thickened, but the mesangium is not. Type There are three types of MPGN, but this classification is becoming obsolete as the causes of this pattern are becoming understood. Type I Type I, the most common by far, is caused by immune complexes depositing in the kidney. It is characterised by subendothelial and mesangial immune deposits. It is believed to be associated with the classical complement pathway. Type II"}, {"id": "wiki20220301en635_24446", "title": "Monoclonal gammopathy of renal significance", "score": 0.009723777528928704, "content": "Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) involves monoclonal immunoglobulins (usually IgG) depositing in the glomeruli and activating compliment leading to glomerular inflammation. Light microscopy shows a membranoproliferative, endocapillary proliferative or membranous glomerulonephropathy with electron dense deposits in the glomeruli being present on electron microscopy. The lesions stain positive for the Ig (usually IgG) as well as compliment; leading to granular immunofluorescent deposits in the mesangium and glomerular basement membrane."}, {"id": "wiki20220301en037_3246", "title": "Glomerulonephritis", "score": 0.009623392245766632, "content": "Rapidly progressive glomerulonephritis, also known as crescentic GN, is characterised by a rapid, progressive deterioration in kidney function. People with rapidly progressive glomerulonephritis may present with a nephritic syndrome. In management, steroid therapy is sometimes used, although the prognosis remains poor. Three main subtypes are recognised: Type 1 is Goodpasture syndrome, an autoimmune disease also affecting the lung. In Goodpasture syndrome, IgG antibodies directed against the glomerular basement membrane trigger an inflammatory reaction, causing a nephritic syndrome and the coughing up of blood. High dose immunosuppression is required (intravenous methylprednisolone) and cyclophosphamide, plus plasmapheresis. Immunohistochemistry staining of tissue specimens shows linear IgG deposits."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[597, 965]], "word_ranges": [[105, 166]], "text": "If we follow the diagnostic scheme for a premature telarche or suspicion of precocious puberty, we request bone age and abdominal ultrasound (the EO is not advanced as in precocious puberty, and we assume that with a small uterus they mean a prepubertal uterus); according to the complementary examinations that we are given, it does not seem to be precocious puberty,"}, "2": {"exist": true, "char_ranges": [[1458, 1537]], "word_ranges": [[250, 263]], "text": "Regarding breast biopsy, it would only be indicated if there are warning signs."}, "3": {"exist": true, "char_ranges": [[1271, 1457]], "word_ranges": [[218, 250]], "text": "Regarding the option of mammography, breast ultrasound is used in pediatrics, and in this case it would be indicated if we were told that there is breast asymmetry (we discard option 3)."}, "4": {"exist": true, "char_ranges": [[597, 965]], "word_ranges": [[105, 166]], "text": "If we follow the diagnostic scheme for a premature telarche or suspicion of precocious puberty, we request bone age and abdominal ultrasound (the EO is not advanced as in precocious puberty, and we assume that with a small uterus they mean a prepubertal uterus); according to the complementary examinations that we are given, it does not seem to be precocious puberty,"}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "It seems that they want to present us with precocious puberty (or premature telarche) but they do not provide any analytical data and the ultrasound data are ambiguous (we should assume that by a small uterus they are referring to a prepubertal uterus, but they do not provide any data on ovarian size). We are presented with the case of a three-year-old girl with advanced mammary development, in principle without any associated cause (in principle she does not take drugs that can increase the level of estrogen in the blood, she does not seem to use body creams or eat a lot of chicken meat). If we follow the diagnostic scheme for a premature telarche or suspicion of precocious puberty, we request bone age and abdominal ultrasound (the EO is not advanced as in precocious puberty, and we assume that with a small uterus they mean a prepubertal uterus); according to the complementary examinations that we are given, it does not seem to be precocious puberty, except for the clinical (Tanner IV). Strictly speaking, without analytical hormonal data, it seems that we could mark option 1, being necessary to follow the girl closely. If we take all the above data for granted, we could rule out option 4, which would be the treatment of a central precocious puberty. Regarding the option of mammography, breast ultrasound is used in pediatrics, and in this case it would be indicated if we were told that there is breast asymmetry (we discard option 3). Regarding breast biopsy, it would only be indicated if there are warning signs.", "full_answer_no_ref": "It seems that they want to present us with precocious puberty (or premature telarche) but they do not provide any analytical data and the ultrasound data are ambiguous (we should assume that by a small uterus they are referring to a prepubertal uterus, but they do not provide any data on ovarian size). We are presented with the case of a three-year-old girl with advanced mammary development, in principle without any associated cause (in principle she does not take drugs that can increase the level of estrogen in the blood, she does not seem to use body creams or eat a lot of chicken meat). If we follow the diagnostic scheme for a premature telarche or suspicion of precocious puberty, we request bone age and abdominal ultrasound (the EO is not advanced as in precocious puberty, and we assume that with a small uterus they mean a prepubertal uterus); according to the complementary examinations that we are given, it does not seem to be precocious puberty, except for the clinical (Tanner IV). Strictly speaking, without analytical hormonal data, it seems that we could mark option 1, being necessary to follow the girl closely. If we take all the above data for granted, we could rule out option 4, which would be the treatment of a central precocious puberty. Regarding the option of mammography, breast ultrasound is used in pediatrics, and in this case it would be indicated if we were told that there is breast asymmetry ([HIDDEN]). Regarding breast biopsy, it would only be indicated if there are warning signs.", "full_question": "A woman comes to the office with her 3 year old daughter because she has detected a slight mammary development since 3 months without taking any medication or any relevant history. Indeed, the physical examination shows a Tanner stage IV, with no growth of pubic or axillary hair. The external genitalia are normal. Ultrasonography reveals a small uterus and radiology reveals a bone age of 3 years. What attitude should be adopted?", "id": 384, "lang": "en", "options": {"1": "Follow-up every 3-4 months, as this is a temporary condition that often resolves on its own.", "2": "Breast biopsy.", "3": "Mammography.", "4": "Administration of GnRh analogues.", "5": NaN}, "question_id_specific": 151, "type": "PEDIATRICS", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en026_109891", "title": "Thelarche", "score": 0.017042606516290727, "content": "Premature thelarche Premature thelarche is a benign condition in which a young female has breast development before the age of 8 without any accompanied pubertal changes. Individuals undergoing isolated premature thelarche do not experience menstruation, pubic hair growth (pubarche), or the bone growth characteristic of puberty. Initial breast development can be bilateral or unilateral and usually begins with a firm, disc-like area of tissue under the areola which can be mistaken for a mass but is almost always a normal, physiologic process. The breast is often tender and palpation is sometimes painful but breast discharge is absent. Usually, the breasts do not develop past stage 3 on the Tanner Scale, hence maintaining adolescent nipples. Moreover, in 90% of patients with isolated premature thelarche, breast enlargement will resolve 6 months to 6 years after diagnosis."}, {"id": "pubmed23n0316_12040", "title": "[True hermaphroditism with bilateral ovotestis].", "score": 0.013982242137581943, "content": "A nineteen years old woman with ambiguous external genitalia was studied. This condition had been previously identified as a newborn, but her parents refused medical attention and it was reared as a girl. At 12-years, she began spontaneous mammary development, appearing pubic and axillary hair, and clitoral enlargement. The menarche occurred at 15-years and it was followed by irregular periods. Physical examination, showed absence of hirsutism and acne, normal mammary development equivalent to grade V of Tanner. The external genitalia showed fused labio-scrotal folds with an small introitus. The urethral meatus was absent and was later located inside the introitus. There was a big phallus similar to an adult penis with a normal glans, flexed by a chordee. Hormonal determinations discarded congenital adrenal hyperplasia. The karyotype was 46,XX and testosterone levels were in adult male range. Pelvic ultrasonography disclosed a normal uterus and both gonads in confirmed by laparoscopy identifying bilateral ovotestis. Testicular tissue was removed and plastic reconstruction of female genitals was done."}, {"id": "wiki20220301en290_36777", "title": "Puberty", "score": 0.013281410125025619, "content": "Pubic hair Pubic hair is often the second noticeable change in puberty, usually within a few months of thelarche. It is referred to as pubarche. The pubic hairs are usually visible first along the labia. The first few hairs are described as Tanner stage 2. Stage 3 is usually reached within another 6–12 months, when the hairs are too numerous to count and appear on the pubic mound as well. By stage 4, the pubic hairs densely fill the \"pubic triangle.\" Stage 5 refers to spread of pubic hair to the thighs and sometimes as abdominal hair upward towards the navel. In about 15% of girls, the earliest pubic hair appears before breast development begins."}, {"id": "wiki20220301en028_19348", "title": "Underarm hair", "score": 0.013187702265372168, "content": "Underarm hair, also known as axillary hair, is the hair in the underarm area (axilla). Development and function Underarm hair, as human body hair, usually starts to appear at the beginning of puberty, with growth usually completed by the end of the teenage years. Axillary hair goes through four stages of development, driven by weak androgens produced by the adrenal in males and females during adrenarche, and testosterone from the testicle in males during puberty. Like Tanner Staging for pubic hair, axillary hair can be staged according to the Wolfsdorf Staging system, named for pediatric endocrinologist Joseph Wolfsdorf, as follows: Wolfsdorf Stage 1 – no axillary hair Wolfsdorf Stage 2 – scant axillary hair (usually coinciding with onset of adrenarche) Wolfsdorf Stage 3 – coarse axillary hair, less than full-adult Wolfsdorf Stage 4 – full adult axillary hair Staging a patient's axillary hair will allow the physician to track the child's development longitudinally."}, {"id": "article-27608_16", "title": "Precocious Puberty -- History and Physical", "score": 0.011488079949564526, "content": "Linear growth acceleration is one of the important features of early puberty. So the exact height, weight, growth velocity (cm/year) and BMI should be documented.  In females, accurate Tanner staging of the breast should take place, which is particularly challenging in obese or overweight girls to differentiate between adipose tissue and the glandular breast tissue. In males, an orchidometer should be used to determine the testicular volume. Volumes of more than 4 ml confirm pubertal development.  In males and females with pubic hair and body odor, the absence of increased testicular volume and breast development should prompt investigation of peripheral causes. Unilateral testicular enlargement is likely due to testicular tumors."}, {"id": "wiki20220301en410_15531", "title": "Well-woman examination", "score": 0.011274093829555097, "content": "Breast ultrasound is a complementary study of mammography. In many women the tissue that makes up the breast is very dense, representing fibrous tissue and glandular tissue, which produces milk during lactation. This limits the radiologist interpreting the study, so, in these cases, the ultrasound is helpful, since this is capable of distinguishing tumors in women with dense breast tissue, where identification is otherwise difficult. Additionally, it is advisable to follow up a mammogram that shows indications of tumors with an ultrasound, to confirm, before more invasive procedures are undertaken. Pelvic exam The pelvic exam is part of the physical examination of the internal pelvic organs (uterus, cervix, ovaries), vagina, and external genitalia. This exam often includes three parts: Inspection of the external genitalia Bimanual examination Inspection of the cervix and vagina using a speculum."}, {"id": "article-29871_4", "title": "Tanner Stages -- Function", "score": 0.010748077637709744, "content": "In females, the normal onset of puberty ranges from 8 to 13 years old, averaging age 10 years in White Americans and age 8.9 years in African-Americans. Puberty in females begins with the development of breast buds under the areola, also known as thelarche, and represents entry into Tanner Stage 2. As puberty progresses, the glandular tissue of the breast increases in size and changes in contour. In females, thelarche is followed in 1 to 1.5 years by the onset of sexual hair (pubic and axillary), known as pubarche. Menarche, the onset of menses, arrives on average at age 12.5 years, regardless of ethnicity, following thelarche on average by 2.5 years (range 0.5 to 3 years).  Between Tanner Stage 2 and 3 breast development, females experience peak height velocity. African-American females have closer to 3 years between their thelarche and menarche, accounting for greater height potential."}, {"id": "wiki20220301en290_36774", "title": "Puberty", "score": 0.010637289741767354, "content": "In the months and years following the appearance of pubic hair, other areas of skin that respond to androgens may develop androgenic hair. The usual sequence is: underarm (axillary) hair, perianal hair, upper lip hair, sideburn (preauricular) hair, periareolar hair, and the beard area. As with most human biological processes, this specific order may vary among some individuals. Arm, leg, chest, abdominal, and back hair become heavier more gradually. There is a large range in amount of body hair among adult men, and significant differences in timing and quantity of hair growth among different racial groups. Facial hair is often present in late adolescence, but may not appear until significantly later. Facial hair will continue to get coarser, darker and thicker for another 2–4 years after puberty. Some men do not develop full facial hair for up to 10 years after the completion of puberty. Chest hair may appear during puberty or years after, though not all men develop it."}, {"id": "article-21673_4", "title": "Female Development -- Definition/Introduction", "score": 0.010091034008931639, "content": "As females develop, a universal means to classify where they are in puberty is known as Tanner stages. Stage one is prepubertal females. They will have no breast tissue or pubic hair. Stage two is when the breast bud begins to protrude with enlargement of the areola and the sparse presence of pubic hair. [2] Female typically experience their ‘growth spurt’ during Tanner stage two. [1] Continued breast and areola enlargement without distinct separation of the contour and darkening pubic hair along the mons pubis is known as Tanner stage three. Stage four is classified when a secondary mound above the breast forms and the pubic hair thickens but is not on the thigh. Menarche occurs in stage three or four. Stage five is the adult female body. It is when the nipple projects out of the areola and the pubic hairs reach the medial thigh. [3]"}, {"id": "Surgery_Schwartz_3859", "title": "Surgery_Schwartz", "score": 0.01003734827264239, "content": "accounts for the greatest num-ber of malpractice claims for errors in diagnosis and for the largest number of paid claims. Litigation often involves younger women, whose physical examination and mammogram may be misleading. If a young woman (≤45 years) presents with a palpable breast mass and equivocal mammographic findings, ultrasound examination and biopsy are used to avoid a delay in diagnosis.ExaminationInspection. The clinician inspects the woman’s breast with her arms by her side (Fig. 17-18A), with her arms straight up in the air (Fig. 17-18B), and with her hands on her hips (with and without pectoral muscle contraction).135,136 Symmetry, size, and shape of the breast are recorded, as well as any evidence of edema (peau d’orange), nipple or skin retraction, or erythema. With the arms extended forward and in a sitting position, the woman leans forward to accentuate any skin retraction.Figure 17-18. Examination of the breast. A. Inspection of the breast with arms at sides. B."}, {"id": "pubmed23n0360_16325", "title": "47,Xxx in an adolescent with premature ovarian failure and autoimmune disease", "score": 0.009900990099009901, "content": "Background: Premature ovarian failure (POF) is often associated with autoimmune disorders. The 47,XXX karyotype has also been associated with POF and other genitourinary abnormalities. Following is a case of a 17 year old with immune thrombocytopenic purpura (ITP), POF, 47, XXX and a positive antinuclear antibody (ANA).Case Report: A 17 year old Caucasian female was referred to the Adolescent Health Clinic for evaluation of oligomenorrhea with secondary amenorrhea. Thelarche occurred at 12 years, and menarche at 13 years of age. Since then she had a total of five menstrual periods, spaced 1-15 months apart and lasting 3-5 days. Her last menstrual period was six months prior to presentation. Past medical history was significant for chronic ITP diagnosed seven months prior to presentation, when she developed easy bruising. She was treated with IV gamma globulin and had a moderate response, but relapsed several weeks later. She was started on oral prednisone and had a good response, but continued to relapse whenever steroids were tapered. She was therefore maintained on prednisone 10 mg QOD. There was no family history of irregular menses or autoimmune disease. Physical exam revealed a well-appearing, slightly Cushingoid 17 year old. Physical and cognitive development were age-appropriate. There were no stigmata of Turner Syndrome. The thyroid was normal. Breasts were Tanner 5; public hair was Tanner 3. The external genitalia were normal and appeared well-estrogenized. The remainder of the exam was unremarkable. Pelvic ultrasound demonstrated a normal uterus and ovaries. Laboratory evaluation was significant for elevated gonadotropins and nondetectable estradiol. ANA was positive at 1:320 with a speckled pattern. Blood counts, serologies, complement levels, and coagulation studies were otherwise normal. Cytogenetic studies revealed a 47,XXX karyotype. The patient was placed on an estrogen/norethindrone hormone replacement patch for premature ovarian failure. To date, she has developed no further symptoms, and does not meet criteria for a diagnosis of systemic lupus erythematosis.Conclusions: A 47,XXX karyotype was found in a 17 year old with POF and ITP with a positive ANA. The presence of known autoimmune disease in a woman with POF should not dissuade the physician from evaluating for a potential genetic cause."}, {"id": "pubmed23n0634_5345", "title": "[Not Available].", "score": 0.009900990099009901, "content": "Sexual ambiguities are due to varied congenital or hormonal causes. A retrospective study carried out from January 1995 to December 2000 and followed by a prospective study from January 2001 to September 2002 sought to describe the clinical aspects of sexual ambiguities in Internal Medicine in a tertiary hospital Mali. Among 12 patients out of 2223 consultants identified (0.54 percent), 10 of them were phenotypically feminine and 2 phenotypically masculine. The average age of the patients was 14.3+- 8.9 years. Clinically, 3 out of the 10 phenotypically feminine patients presented an anomaly of the external genital organs; 5 out of the 10 had low axillary and pubic hair growth; 4 out of the 10 had delayed puberty; 6 out of the 10 had primary amenorrhoea; 7 out of 10 had hypoplasia of the mammary glands; and3 out of 10 had an inguinal mass. For the the 2 phenotypically masculine patients, one had a bilateral gynecomastia, a macroskelia of 2m04, a low axillary and pubic hair growth and the other had an anomaly of the external genital organs."}, {"id": "pubmed23n0876_17161", "title": "Trichotillomania: Bizzare Patern of Hair Loss at 11-Year-old Girl.", "score": 0.00980392156862745, "content": "Trichotillomania (TTM) is defined by the Diagnostics and Statistic Manual of Mental Disorders, 4th edition (DMS-IV) as hair loss from a patient`s repetitive self-pulling of hair. The disorder is included under anxiety disorders because it shares some obsessive-compulsive features. Patients have the tendency towards feelings of unattractiveness, body dissatisfaction, and low self-esteem (1,2). It is a major psychiatric problem, but many patients with this disorder first present to a dermatologist. An 11-year-old girl came to our department with a 2-month history of diffuse hair loss on the frontoparietal and parietotemporal area (Figure 1). She had originally been examined by a pediatrician with the diagnosis of alopecia areata. The patient`s personal history included hay fever and shortsightedness, and she suffered from varicella and mononucleosis. Nobody in the family history suffered from alopecia areata, but her father has male androgenetic alopecia (Norwood/Hamilton MAGA C3F3). The mother noticed that the child had had changeable mood for about 2 months and did not want to communicate with other persons in the family. The family did not have any pet at home. At school, her favorite subjects were Math and Computer Studies. She did not like Physical Education and did not participate in any sport activities during her free time. This was very strange because she was obese (body-mass index (BMI) 24.69). She was sometimes angry with her 13-year-old sister who had better results at school. The girl had suddenly started to wear a blue scarf. The parents did not notice that she pulled out her hair at home. Dermatological examination of the capillitium found a zone of incomplete alopecia in the frontoparietal and parietotemporal area, without inflammation, desquamation, and scaring. Hairs were of variable length (Figure 1). There was a patch of incomplete alopecia above the forehead between two stripes of hair of variable length (Figure 2). The hair pull test was negative along the edges of the alopecia. Mycological examination from the skin capillitium was negative. The trichoscopy and skin biopsy of the parietotemporal region of the capillitium (Figure 3) confirmed trichotillomania. Laboratory tests (blood count, iron, ferritin, transferrin, selenium, zinc, vitamin B12, folic acid, serology and hormones of thyroid gland) were negative. We referred the girl for ophthalmologic and psychological examination. Ophthalmologic examination proved that there was no need to add any more diopters. The psychological examination provided us with a picture in which she drew her family (Figure 4). The strongest authority in the family was the mother because she looked after the girls for most of the day. She was in the first place in the picture. The father had longer working hours and spent more time outside the home. He worked as a long vehicle driver. He was in the second place in the picture. There was sibling rivalry between the girls, but the parents did not notice this problem and preferred the older daughter. She was successful at school and was prettier (slim, higher, curly brown hair, without spectacles). Our 11-years-old patient noticed all these differences between them, but at her level of mental development was not able to cope with this problem. She wanted to be her sister's equal. The sister is drawn in the picture in the third place next to father, while the patient's own figure was drawn larger and slim even though she was obese. Notably, all three female figures had very nice long brown hair. It seemed that the mother and our patient had better quality of hair and more intense color than the sister in the drawing. The only hairless person in the picture was the father. The girl did not want to talk about her problems and feelings at home. Then it was confirmed that our patient was very sensitive, anxious, willful, and withdrawn. She was interested in her body and very perceptive of her physical appearance. From the psychological point of view, the parents started to pay more interest to their younger daughter and tried to understand and help her. After consultation with the psychiatrist, we did not start psychopharmacologic therapy for trichotillomania; instead, we started treatment with cognitive behavioral therapy, mild shampoo, mild topical steroids (e.g. hydrocortisone butyrate 0.1%) in solution and methionine in capsules. With parents' cooperation, the treatment was successful. The name trichotillomania was first employed by the French dermatologist Francois Henri Hallopeau in 1889, who described a young man pulling his hair out in tufts (3-5). The word is derived from the Greek thrix (hair), tillein (to pull), and mania (madness) (5). The prevalence of TTM in the general adult population ranges from 0.6% to 4%, and 2-4% of the general psychiatric outpatient population meet the criteria for TTM (2-5). The prevalence among children and adolescents has been estimated at less than 1% (5). The disease can occur at any age and in any sex. The age of onset of hair pulling is significantly later for men than for women (3). There are three subsets of age: preschool children, preadolescents to young adults, and adults. The mean age of onset is pre-pubertal. It ranges from 8 to 13 years (on average 11.3 years) (2-5). The occurrence of hair-pulling in the first year of life is a rare event, probably comprising &lt;1% of cases (5). The etiology of TTM is complex and may be triggered by a psychosocial stressor within the family, such as separation from an attachment figure, hospitalization of the child or parent, birth of a younger sibling, sibling rivalry, moving to a new house, or problems with school performance. It has been hypothesized that the habit may begin with \"playing\" with the hair, with later chronic pulling resulting in obvious hair loss (2). Environment is a factor because children usually pull their hair when alone and in relaxed surroundings. The bedroom, bathroom, or family room are \"high-risk\" situations for hair-pulling (5). Men and women also differed in terms of the hair pulling site (men pull hair from the stomach/back and the moustache/beard areas, while women pull from the scalp) (3). Pulling hair from siblings, pets, dolls, and stuffed animals has also been documented, often occurring in the same pattern as in the patient (5). Genetic factors contributing to the development of TTM are mutations of the SLITRK1 gene, which plays a role in cortex development and neuronal growth. The protein SAPAP3 has been present in 4.2% of TTM cases and patients with obsessive-compulsive disorder (OCD). It may be involved in the development of the spectrum of OCD. A significantly different concordance rate for TTM was found in monozygotic (38.1%) compared with dizygotic (0%) twins in 34 pairs (3). The core diagnostic feature is the repetitive pulling of hairs from one`s own body, resulting in hair loss. The targeted hair is mostly on the scalp (75%), but may also be from the eyebrows (42%), eyelashes (53%), beard (10%), and pubic area (17%) (3,5). There are three subtypes of hair pulling - early onset, automatic, and focused. Diagnostic criteria for TTM according to DSM-IV criteria are (2,3,5): 1) recurrent pulling of one`s hair resulting in noticeable hair loss; 2) an increasing sense of tension immediately prior to pulling out the hair or when attempting to resist the behavior; 3) pleasure, gratification, or relief when pulling out the hair; 4) the disturbance is not better accounted for by another mental disorder and is not due to a general medical condition (e.g., a dermatologic condition); 5) the disturbance causes clinically significant distress or impairment in social, occupational, or other important areas of functioning. The differential diagnosis includes alopecia areata (Table 1) (6), tinea capitis, telogen effluvium, secondary syphilis, traction alopecia, loose anagen syndrome, lichen planopilaris, alopecia mucinosa, and scleroderma (2-5). Biopsy of an involved area (ideally from a recent site of hair loss) can help to confirm the diagnosis (5). On histologic examination, there are typically increased numbers of catagen and telogen hairs without evidence of inflammation. Chronic hair pulling induces a catagen phase, and more hairs will be telogen hairs. Pigment casts and empty anagen follicles are often seen. Perifollicular hemorrhage near the hair bulb is an indicator of TTM (2). Complications of TTM are rare, but they comprise secondary bacterial infections with regional lymphadenopathy as a result of picking and scratching at the scalp. Many patients play with and ingest the pulled hairs (e.g. touching the hair to lips, biting, and chewing). Trichophagia (ingestion of the hair) can lead to a rare complication named trichobezoar (a \"hair ball\" in stomach). This habit is present in approximately 5% to 30% of adult patients, but it is less frequent in children. Patient with trichophagia present with pallor, nausea, vomiting, anorexia, and weight loss. Radiologic examination and gastroscopy should not be delayed (2,4,5). The management of the disease is difficult and requires strong cooperation between the physician, patient, and parents. The dermatologist cannot take part in the therapy, strictly speaking, but without the psychological, psychopharmacologic, and topic dermatologic treatment a vicious circle will be perpetuated. "}, {"id": "wiki20220301en235_37700", "title": "Germaine Greer", "score": 0.00980392156862745, "content": "A Paladin paperback followed, with cover art by British artist John Holmes, influenced by René Magritte, showing a female torso as a suit hanging from a rail, a handle on each hip. Clive Hamilton regarded it as \"perhaps the most memorable and unnerving book cover ever created\". Likening the torso to \"some fibreglass cast on an industrial production line\", Christine Wallace wrote that Holmes's first version was a faceless, breastless, naked woman, \"unmistakably Germaine ... hair fashionably afro-frizzed, waist-deep in a pile of stylised breasts, presumably amputated in the creation of a 'female eunuch' based on an assumed equivalence of testicles and mammary glands\". The book was reissued in 2001 by Farrar, Straus & Giroux at the instigation of Jennifer Baumgardner, a leading third-wave feminist and editor of the publisher's Feminist Classics series. According to Justyna Wlodarczyk, Greer emerged as \"the third wave's favorite second-wave feminist\". Arguments"}, {"id": "Gynecology_Novak_6744", "title": "Gynecology_Novak", "score": 0.009783798576902024, "content": "History and physical examination remain the most effective methods of follow-up in patients treated for endometrial cancer (392–394). Patients should be examined every 3 to 4 months during the first 2 years and every 6 months thereafter. About one-half of patients discovered to have recurrent cancer have symptoms, and 75% to 80% of recurrences are detected initially on physical examination. Particular attention should be given to peripheral lymph nodes, the abdomen, and the pelvis. Very few asymptomatic recurrences are detected by vaginal cytology. Chest x-ray every 12 months is an important method of posttreatment surveillance. Almost one-half of all asymptomatic recurrences are detected by chest x-ray. Other radiologic studies, such as CT scans, are not indicated for routine follow-up of patients who do not have symptoms."}, {"id": "wiki20220301en440_32779", "title": "Gynecomastia", "score": 0.009708737864077669, "content": "Diagnosis To diagnose gynecomastia, a thorough history and physical examination are obtained by a physician. Important aspects of the physical examination include evaluation of the male breast tissue with palpation to evaluate for breast cancer and pseudogynecomastia (male breast tissue enlargement solely due to excess fatty tissue), evaluation of penile size and development, evaluation of testicular development and an assessment for masses that raise suspicion for testicular cancer, and proper development of secondary sex characteristics such as the amount and distribution of pubic and underarm hair. Gynecomastia usually presents with bilateral involvement of the breast tissue but may occur unilaterally as well."}, {"id": "pubmed23n0591_4953", "title": "The association of primary hyperparathyroidism and primary ovarian failure: a de novo t(X; 2) (q22p13) reciprocal translocation.", "score": 0.009615384615384616, "content": "A 40-year-old female presented with primary amenorrhoea at 17 years of age. She was tall at 98th centile for height with eunuchoidal body habitus. Her breast development was Tanner stage 3, pubic and axillary hair Tanner stage 4 with normal external genitalia. Her bone age was 13.4 years at a chronological age of 17.8 years. Gonadotrophins were elevated indicating primary ovarian failure. A diagnostic laparotomy revealed hypoplastic, infantile uterus with bilateral streak gonads. Chromosomal analysis showed a balanced reciprocal translocation 46X, t(X; 2) (q22 p13). She became pregnant by in vitro fertilization with egg donation at the age of 36 years. At 13 weeks of gestation, she presented with intractable vomiting. She had raised corrected serum calcium and parathyroid hormone concentrations consistent with the diagnosis of primary hyperparathyroidism (PHPT). She underwent parathyroidectomy at 24 weeks of gestation with removal of a large left inferior parathyroid adenoma which normalized her serum calcium. Multipoint linkage from a genome-wide screen has identified a region of suggestive linkage on chromosome 2p13.3-14 in some cases of familial isolated hyperparathyroidism (FIHP). To our knowledge, this is the first case of primary amenorrhoea due to reciprocal translocation involving chromosome 2 and the X chromosome associated with PHPT. PHPT in this case is most likely to be as a result of chromosome 2 involvement where a locus for FIHP has been identified. Identification of the gene involved on chromosome 2p13.3-14 will be of considerable interest."}, {"id": "article-18587_16", "title": "New Breast Mass -- History and Physical -- Physical Examination", "score": 0.009615384615384616, "content": "Clinical examination of a breast lump is the first stage in the triple-assessment approach. Both breasts and axillae should be examined meticulously by the clinician, as well as carrying out a physical examination of other body systems as indicated by the history. Although it can be tempting to bypass the physical examination in favor of other, more targeted investigation modalities such as mammography or sonography, the findings of the physical examination are crucial for the effective diagnosis and management of breast disease. [16] Repeated studies have indicated that only by combining all three assessments can optimal sensitivity and specificity be achieved. [4] [16]"}, {"id": "pubmed23n0290_21592", "title": "Clinical breast examination.", "score": 0.009523809523809525, "content": "An astute clinician has an appreciation of the wide variation possible in normal breasts, but anticipates that the palpation of the breasts of an individual woman will be determined by basic facts concerning typical relative distribution of gland tissue, breast symmetry, the influence of life history, and possibly previous surgery. If the findings of a CBE are not as anticipated, the clinician must find out why this is so. This is a different frame of reference from asking whether a given lump or area should be considered suspicious for cancer. The basic questions are whether the findings of a CBE are consistent with typical breast structure and anatomy and in the context of the woman's life history. If these questions can be answered in the affirmative, the examination is complete; if not, further evaluation is necessary."}, {"id": "pubmed23n1146_16888", "title": "Case 308.", "score": 0.009433962264150943, "content": "An 11-year-old girl presented to the pediatric gastroenterology outpatient department of our institution with gradually increasing painless abdominal distention. The distention started 2 years earlier and was not associated with any other constitutional symptoms, vomiting, diarrhea, jaundice, hematemesis, or melaena. She reported early satiety and heaviness in the lower abdomen. The abdominal swelling was predominantly in the infraumbilical region and was soft at palpation. She was the first child of nonconsanguineous parents and had an uneventful perinatal course after a normal vaginal delivery. Her developmental milestones were normal. She had an average scholastic performance at school. There was no history of visual problems, seizures, or inappropriate behaviors. She had an early menarche 2 years previously. Her menstrual cycles were regular, and there was no abnormal vaginal discharge. Her breast development was normal (Tanner stage III), while pubic and axillary hair were absent (Tanner stage I). She was short for her age (104 cm; normal range, 120-154 cm). There was no history of short stature among her siblings or parents. Laboratory investigations were performed to measure thyroid-stimulating hormone (1354.34 μIU/mL; normal range, 0.35-5.5 μIU/mL), triiodothyronine (&lt;2.5 ng/dL [0.0385 pmol/L]; normal range, 100-200 ng/dL [1.54-3.08 pmol/L]), thyroxine (1.35 μg/dL [17.37 nmol/L]; normal range, 5-12 μg/dL [64.35-154.44 nmol/L]), β-human chorionic gonadotropin (&lt;1.2 mIU/mL; normal, &lt;5 mIU/mL), luteinizing hormone (0.08 mIU/mL; normal range, 0.1-6.0 mIU/mL), and follicle-stimulating hormone (6.93 mIU/mL; normal range, 0.3-2.0 mIU/mL) levels. Complete blood count was normal. An abdominal mass was suspected, and abdominopelvic CT was performed and followed by US; these examinations revealed multiple large cysts in both ovaries (Figs 1, 2A, 2B). The uterus was pubertal in shape, and endometrial thickness was 9 mm, representing normal follicular phase measurement. Serum CA-125 and inhibin levels were normal. To evaluate short stature, radiographs of the hand (Fig 3) and pelvis (Fig 3B) were obtained as part of a limited skeletal survey, keeping in mind the possible skeletal changes associated with hypothyroidism. In view of the hypothyroidism, US of neck was also performed (Fig 4). Treatment was started based on the clinical and radiologic parameters, and the child's condition improved with medical treatment."}, {"id": "article-37489_12", "title": "Gynecomastia -- History and Physical", "score": 0.009433962264150943, "content": "A careful review of family history, genetic history, medications, and recreational drug use should also be taken into consideration. A complete and thorough physical exam should be done. The head and neck exam should evaluate for any abnormal masses or thyroid abnormalities. Assess breasts for the nature of the tissue, masses, skin changes, nipple discharge, asymmetries, and tenderness, along with an axillary examination. The testes should be examined to look for asymmetry, masses, enlargement, or atrophy. Those males with feminizing characteristics should have endocrine testing and genetic testing. Any other positive findings on physical examination should be treated in an appropriate manner."}, {"id": "pubmed23n0739_21576", "title": "Precocious puberty in Turner Syndrome: report of a case and review of the literature.", "score": 0.009345794392523364, "content": "Turner Syndrome (TS) is caused by monosomy or structural abnormalities of the X chromosome, with a prevalence of about 1/2000 females live birth. Most important clinical features of TS are short stature and gonadal failure. Approximately one third of girls with TS may undergo spontaneous puberty. Here we report on the case of a girl with a rare 45X0/47XXX mosaic TS exhibiting a precocious puberty. The patient was diagnosed with TS at the age of 4 years, upon a diagnostic work-up for dysmorphic features. Chromosome analysis revealed a mosaic karyotype (45X0/47XXX). She presented with normal height and normal growth velocity so that Growth Hormone (GH) therapy was not started. She was referred to our Department at the age of 7 years and 10 months, because of vaginal bleeding. A physical examination revealed a Tanner stage III for breast and Tanner stage III for pubic hair development. Height and weight were within the normal range for age. Psychological evaluation showed moderate global developmental delay, together with emotional and social immaturity and reading difficulties. The growth rate was accelerated. Her bone age was 10 years. Pelvic ultrasound demonstrated increased size for age of both the uterus and the ovaries, with bilateral ovarian follicles. GnRH stimulation test revealed pubertal response of gonadotropins (peak LH 22.5 mIU/ml). MRI of the brain was normal. These clinical, radiologic and laboratory findings were consistent with a diagnosis of idiopathic central precocious puberty; therefore, GnRH analog therapy was started, in order to slow pubertal progression and to preserve adult stature. Furthermore, GH treatment was added to further improve adult height. Our case highlights the possibility of precocious puberty as an atypical clinical feature of TS. Thus, precocious puberty may occur in TS girls when a dosage compensation by the cell line with more than two X chromosomes allows normal ovarian function. GnRH analog therapy in addition to GH treatment should be recommended in TS girls with precocious puberty in order to slow pubertal progression and to preserve adult stature."}, {"id": "InternalMed_Harrison_6915", "title": "InternalMed_Harrison", "score": 0.009345794392523364, "content": "Breast cancer is about 1/150th as frequent in men as in women; 1720 men developed breast cancer in 2006. It usually presents as a unilateral lump in the breast and is frequently not diagnosed promptly. Given the small amount of soft tissue and the unexpected nature of the problem, locally advanced presentations are somewhat more common. When male breast cancer is matched to female breast cancer by age and stage, its overall prognosis is identical. Although gynecomastia may initially be unilateral or asymmetric, any unilateral mass in a man older than age 40 years should receive a careful workup including biopsy. On the other hand, bilateral symmetric breast development rarely represents breast cancer and is almost invariably due to endocrine disease or a drug effect. It should be kept in mind, nevertheless, that the risk of cancer is much greater in men with gynecomastia; in such men, gross asymmetry of the breasts should arouse suspicion of cancer. Male breast cancer is best managed"}, {"id": "pubmed23n0840_22918", "title": "An Unusual Presentation of 46,XY Pure Gonadal Dysgenesis: Spontaneous Breast Development and Menstruation.", "score": 0.009259259259259259, "content": "46,XY pure gonadal dysgenesis (Swyer syndrome) is characterized by normal female genitalia at birth. It usually first becomes apparent in adolescence with delayed puberty and amenorrhea. Rarely, patients can present with spontaneous breast development and/or menstruation. A fifteen-year-old girl presented to our clinic with the complaint of primary amenorrhea. On physical examination, her external genitals were completely female. Breast development and pubic hair were compatible with Tanner stage V. Hormonal evaluation revealed a hypergonadotropic state despite a normal estrogen level. Chromosome analysis revealed a 46,XY karyotype. Pelvic ultrasonography showed small gonads and a normal sized uterus for age. SRY gene expression was confirmed by multiplex polymerase chain reaction. Direct sequencing on genomic DNA did not reveal a mutation in the SRY, SF1 and WT1 genes. After the diagnosis of Swyer syndrome was made, the patient started to have spontaneous menstrual cycles and therefore failed to attend her follow-up visits. After nine months, the patient underwent diagnostic laparoscopy. Frozen examination of multiple biopsies from gonad tissues revealed gonadoblastoma. With this report, we emphasize the importance of performing karyotype analysis, which is diagnostic for Swyer syndrome, in all cases with primary or secondary amenorrhea even in the presence of normal breast development. We also suggest that normal pubertal development in patients with Swyer syndrome may be associated with the presence of a hormonally active tumor. "}, {"id": "article-18587_18", "title": "New Breast Mass -- History and Physical -- Physical Examination", "score": 0.009259259259259259, "content": "The breasts can most easily be palpated by asking the patient to lie back at approximately 30 degrees and rest their palm up underneath their head. Palpation of the breast must proceed in a structured manner; generally, clinicians will use a four-quadrant approach (upper outer, upper inner, lower outer, and lower inner quadrants), followed by palpating the areola and then the axillary tail. Particular attention should focus on the inframammary fold and the axillary tail. The normal breast is examined first, and the tissue is assessed for its overall consistency. Masses are most often detected in the upper outer quadrant, as most breast tissue is located here."}, {"id": "pubmed23n1059_10224", "title": "Growing Up Fast: Managing Autism Spectrum Disorder and Precocious Puberty.", "score": 0.009174311926605505, "content": "John is a 4-year-old boy with autism spectrum disorder (ASD) and developmental delay who presented with concerns about increasing aggressive behavior at a follow-up visit with his developmental-behavioral pediatrician. Diagnosis of ASD was made via Diagnostic and Statistical Manual of Mental Disorders, 5th version criteria at initial evaluation at 34 months. Medical history at that time was pertinent for rapid linear growth since the age of 1 and recent pubic hair growth and penile enlargement. Family history was significant for early puberty in a maternal uncle and 4 distant maternal relatives. Standardized testing included administration of the Childhood Autism Rating Scale 2-Standard, which was consistent with severe symptoms of ASD, and the Mullen Scales of Early Learning, which indicated moderate delay in fine motor skills and expressive language and severe delay in receptive language and visual receptive skills.At initial assessment, John's parents also reported a pattern of aggressive behavior, which included frequent hitting of other children at childcare, consistently forceful play with peers and family members, and nightly tantrums with hitting and throwing at bedtime. Triggers of aggressive behavior included other children taking his toys, transition away from preferred activities, and being told \"no.\"John was concurrently evaluated by a pediatric endocrinologist at 34 months. At that assessment, his height Z-score was +2.5, and he had Tanner 2 pubic hair, Tanner 3 genitalia, and 6 cc testicular volumes. Radiograph of the hand revealed a bone age of 6 years (+7.8 S.D.). Laboratory studies revealed a markedly elevated testosterone level and low gonadotropin (luteinizing hormone [LH] and follicle-stimulating hormone) levels and a normal dehydroepiandrosterone sulfate, suggestive of peripheral precocious puberty. Targeted genetic testing with sequencing of the LHCGR gene revealed a heterozygous D578G mutation resulting in the rare condition Familial Male-Limited Precocious Puberty (FMPP), characterized by constitutive activation of the LH receptor. FMPP, also referred to as testotoxicosis, was attributed as the cause of John's peripheral precocious puberty.By the age of 4, John's height Z-score was +3.1, his genitalia larger, and his bone age 10 years (+10.3 S.D.). His parents elected to start off-label therapy with bicalutamide (a nonsteroidal antiandrogen) and anastrazole (an aromatase inhibitor), recommended by the endocrinologist. Unexpectedly, as John's hyperandrogenism was treated, John's family reported intensified aggression toward other children and adults, especially at school, in addition to multiple daily instances of biting when upset. What is your next step in John's treatment of his challenging behavior? 1. Shenker A, Laue L, Kosugi S, et al. A constitutively activating mutation of the luteinizing hormone receptor in familial male precocious puberty. Nature. 1993;365:652-654."}, {"id": "wiki20220301en048_50518", "title": "Papal armorial", "score": 0.009174311926605505, "content": "|} Late Middle Ages and Renaissance Note that some of the images of the coats of arms shown below anachronistically include the external adornments of the papal tiara and the keys of Peter. These ornaments were not in use before the 1450s. Popes of the Early Modern period Most popes of the 16th to 18th centuries came from Italian noble families, but there were some exceptions, such as Sixtus V (1585-1590), who was of low birth. Popes of the modern period The last person elected as pope who was not already an ordained priest or monk was Leo X (Giovanni di Lorenzo de' Medici) in 1513. Thus, throughout the Early Modern period, the elected pope already had a coat of arms: if he did not have a family coat of arms to begin with, he would have adopted one upon being made bishop. Upon his election as pope, he would continue using his pre-existing coat of arms, in some cases with heraldic augmentations. This tradition was continued into the modern period."}, {"id": "pubmed23n0295_24071", "title": "Hetero- and isosexual pseudoprecocity associated with testicular sex-cord tumors in an 8 year-old male.", "score": 0.00909090909090909, "content": "Enlargement of the right breast, axillary hair, and acceleration of linear growth rate were first noted at 8 years of age in an otherwise healthy male with no known exposure to exogenous hormones. At 9.5 years of age the right subareolar mass was excised; histologic examination revealed fibrous breast tissue. Subsequently pubic hair appeared. At 10.7 years of age, the patient complained of right inguinal pain after a minor injury. Examination revealed a tall (height age 12.7 years), mature, muscular boy with enlarged (R: 5 x 3 x 2 cm; L: 3 x 2 x 3 cm) firm, irregular testes, Tanner stage II pubic hair, and modest axillary hair. No perioral pigmentation was present. Testicular ultrasonography revealed multilobular echogenic foci with calcifications. Bone age was 13 years, the LH and FSH secretory responses to GnRH were minimal (LH: &lt; 0.038--&gt;0.28 mIU/ml; FSH: &lt; 0.063--&gt;0.11 mIU/ml), and basal serum testosterone (&lt; 10 ng/dl) and estradiol (&lt; 10 pg/ml) values were undetectable. Following administration of human chorionic gonadotropin (hCG), the serum testosterone concentration increased to 275 ng/dl, while estradiol remained unmeasurable. Spermatic vein concentrations of testosterone were undetectable in the basal state and increased after hCG administration. After bilateral orchiectomy, pathologic examination revealed multifocal tumors composed of brightly eosinophilic, large polygonal cells arranged in nests, cords, and clusters within dense connective tissue or mucinous stroma with lamellar calcifications of varying sizes. These pathologic findings were compatible with a large cell calcifying Sertoli cell (sex-cord)tumor of the testes. Testosterone, estradiol, immunoreactive and bioactive aromatase activity were not detectable in the tumor. Thus, both heterosexual (gynecomastia) and isosexual (increased musculature, pubic and axillary hair) precocious puberty may occur in boys with testicular sex-cord tumors."}, {"id": "wiki20220301en252_39333", "title": "Elling Woman", "score": 0.00909090909090909, "content": "Examination The Elling Woman is believed to have been hanged, like the Tollund Man. The estimated year of death was dated to approximately 280 BCE in the Nordic Iron Age, also around the time of the Tollund Man; however, it is not possible to confirm whether or not they were both killed at exactly the same time. It also initially might have been impossible to tell the sex of her body, if the hair had not been preserved, although X rays were taken of her pelvis, which proved she was female. In 1978, the body was reexamined with radiographs, from which the sex was determined to be female and the original age-at-death estimate of 25 years was found to be accurate. This body is often identified by the braid on her head, which was tied into an elaborate knot. Elling Woman is believed to have been a human sacrifice. Demineralization, which often occurs with bog bodies, was found to be the initial cause of what was first understood as apparent osteoporosis in the remains. References"}, {"id": "pubmed23n0629_5851", "title": "Partial hypogonadotropic hypogonadism associated with the Leu266Arg and Gln106Arg mutation of the gonadotropin-releasing hormone receptor.", "score": 0.009009009009009009, "content": "We describe a patient with partial hypogonadotropic hypogonadism caused by a compound heterozygous GnRH-R mutation. She is a 20-year-old tall, eunuchoid female referred for evaluation of primary amenorrhea. Spontaneous thelarche occurred at the age of 15 years. Breast and pubic hair were at Tanner stages 3 and 4, respectively. Evaluation revealed low plasma estradiol level and absence of withdrawal bleeding after progestin challenge. Pelvic ultrasonography showed a small uterus and ovaries. Bone age was delayed at 14.5 years. Bone mineral density showed osteopenia. Endogenous LH secretory pattern was abnormal with low amplitude and frequency, but responded to pulsatile GnRH administration. The coding exons of the GnRH-R gene were amplified and the PCR products were sequenced bidirectionally. Two different mutations were identified: one in exon 1 (Gln106Arg) and the other in exon 3 (Leu266Arg)."}, {"id": "article-24564_8", "title": "Breast Lymphatics -- History and Physical", "score": 0.009009009009009009, "content": "Physical examination of the breast lymphatics occurs during the breast exam. The examiner can best palpate the axillary nodes with the patient sitting up and the examiner supporting the arms to the side, or the hands resting on the hips. The examiner should palpate the supraclavicular and infraclavicular lymph nodes as well."}, {"id": "wiki20220301en133_11763", "title": "Estrogen insensitivity syndrome", "score": 0.008928571428571428, "content": "The patient had a small uterus, with an endometrial stripe that could not be clearly identified. At the age of 15 years, 5 months, her bone age was 11 or 12 years, and at the age of 17 years, 8 months, her bone age was 13.5 years. Her bone mass was lower than expected for her age, and levels of osteocalcin and C-terminal telopeptide were both elevated, suggesting an increased rate of bone turnover. She was 162.6 cm tall, and her growth velocity indicated a lack of estrogen-induced growth spurt at puberty. The patient had normal pubic hair development (Tanner stage IV) and severe facial acne, which could both be attributed to testosterone. Her ovarian pathology was attributed to the elevated levels of gonadotropins. In addition to her absence of breast development and areolar enlargement, the patient also appeared to show minimal widening of the hips and a lack of subcutaneous fat deposition, which is in accordance with the established role of estrogen and ERα in the development of"}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[729, 1183]], "word_ranges": [[115, 185]], "text": "Paroxysmal hemicrania predominates in women, with episodes of pain similar to cluster headache, but with a shorter duration (2-30 min), and a higher frequency (5-30 episodes per day). As for SUNCT, the crises are much shorter, lasting seconds (5-240 seconds) and are usually refractory to treatment. Therefore the diagnosis would be of a paroxysmal hemicrania and its treatment of choice is indomethacin (which also the answer is a diagnostic criterion)."}, "2": {"exist": true, "char_ranges": [[594, 728]], "word_ranges": [[92, 115]], "text": "Cluster headache predominates in males whose duration can vary between 15-180 minutes, between once every 2 days, up to 8 times a day."}, "3": {"exist": true, "char_ranges": [[594, 728]], "word_ranges": [[92, 115]], "text": "Cluster headache predominates in males whose duration can vary between 15-180 minutes, between once every 2 days, up to 8 times a day."}, "4": {"exist": true, "char_ranges": [[594, 728]], "word_ranges": [[92, 115]], "text": "Cluster headache predominates in males whose duration can vary between 15-180 minutes, between once every 2 days, up to 8 times a day."}, "5": {"exist": true, "char_ranges": [[594, 728]], "word_ranges": [[92, 115]], "text": "Cluster headache predominates in males whose duration can vary between 15-180 minutes, between once every 2 days, up to 8 times a day."}}, "full_answer": "In this question we are presented with a case to deduce a diagnosis and then indicate which would be the treatment of choice. Both the characteristics of the case and the answers suggest that it is a trigemino-autonomic headache (intense, periocular pain with lacrimation and rhinorrhea). The idea is to make a differential diagnosis mainly between a cluster headache (whose treatments include answers 2, 3, 4 and 5), a paroxysmal hemicrania (whose treatment of choice is the indomethacin of answer 1) and a SUNCT (unilateral neuralgiform headache with conjunctival injection and lacrimation). Cluster headache predominates in males whose duration can vary between 15-180 minutes, between once every 2 days, up to 8 times a day. Paroxysmal hemicrania predominates in women, with episodes of pain similar to cluster headache, but with a shorter duration (2-30 min), and a higher frequency (5-30 episodes per day). As for SUNCT, the crises are much shorter, lasting seconds (5-240 seconds) and are usually refractory to treatment. Therefore the diagnosis would be of a paroxysmal hemicrania and its treatment of choice is indomethacin (which also the answer is a diagnostic criterion). Therefore correct answer 1 (Indomethacin). All data provided are based on the Diagnostic Criteria of the International Headache Society.", "full_answer_no_ref": "In this question we are presented with a case to deduce a diagnosis and then indicate which would be the treatment of choice. Both the characteristics of the case and the answers suggest that it is a trigemino-autonomic headache (intense, periocular pain with lacrimation and rhinorrhea). The idea is to make a differential diagnosis mainly between a cluster headache (whose treatments include answers 2, 3, 4 and 5), a paroxysmal hemicrania (whose treatment of choice is the indomethacin of [HIDDEN]) and a SUNCT (unilateral neuralgiform headache with conjunctival injection and lacrimation). Cluster headache predominates in males whose duration can vary between 15-180 minutes, between once every 2 days, up to 8 times a day. Paroxysmal hemicrania predominates in women, with episodes of pain similar to cluster headache, but with a shorter duration (2-30 min), and a higher frequency (5-30 episodes per day). As for SUNCT, the crises are much shorter, lasting seconds (5-240 seconds) and are usually refractory to treatment. Therefore the diagnosis would be of a paroxysmal hemicrania and its treatment of choice is indomethacin (which also [HIDDEN]). Therefore [HIDDEN]. All data provided are based on the Diagnostic Criteria of the International Headache Society.", "full_question": "A 40-year-old woman consults for approximately 20 episodes per day of intense left periocular pain lasting 15 minutes, accompanied by intense tearing and rhinorrhea. Her examination and MRI are normal. His treatment of choice would be:", "id": 238, "lang": "en", "options": {"1": "Indomethacin.", "2": "Lamotrigine.", "3": "Verapamil.", "4": "Prednisone.", "5": "Lithium carbonate."}, "question_id_specific": 145, "type": "NEUROLOGY", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0771_10057", "title": "The usual treatment of trigeminal autonomic cephalalgias.", "score": 0.019801980198019802, "content": "Trigeminal autonomic cephalalgias include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, and rhinorrhea (SUNCT). Conventional pharmacological therapy can be successful in the majority of trigeminal autonomic cephalalgias patients. Most cluster headache attacks respond to 100% oxygen inhalation, or 6 mg subcutaneous sumatriptan. Nasal spray of sumatriptan (20 mg) or zolmitriptan (5 mg) are recommended as second choice. The bouts can be brought under control by a short course of corticosteroids (oral prednisone: 60-100 mg/day, or intravenous methylprednisolone: 250-500 mg/day, for 5 days, followed by tapering off the dosage), or by long-term prophylaxis with verapamil (at least 240 mg/day). Alternative long-term preventive medications include lithium carbonate (800-1600 mg/day), methylergonovine (0.4-1.2 mg/day), and topiramate (100-200 mg/day). As a rule, paroxysmal hemicrania responds to preventive treatment with indomethacin (75-150 mg/day). A short course of intravenous lidocaine (1-4 mg/kg/hour) can reduce the flow of attacks during exacerbations of SUNCT. Lamotrigine (100-300 mg/day) is the preventive drug of choice for SUNCT. Gabapentin (800-2700 mg/day), topiramate (50-300 mg/day), and carbamazepine (200-1600 mg/day) may be of help. "}, {"id": "pubmed23n0540_20027", "title": "[Three Japanese cases of hypnic headache].", "score": 0.019149715961150816, "content": "We described three cases of hypnic headache with successful treatment by lithium carbonate or caffeine. This is the first detail report of Japanese cases. An endocrinological test and rhythm analyses of ambulatory blood pressure (ABP) and heart rate variability in a case suggested possible association between hypnic headache and hypothalamic-pituitary dysfunction. Case 1: A 48-year-old female migraineur complained of new-onset nocturnal headaches. Her headache awakened her from sleep between 1 AM and 2 AM. The headache occurred 3-4 times per week and lasted from 1 hour to 2 hours. The headache were moderate intensity and bilateral dull throbbing pain that located in the forehead to temples. There was no accompanying symptoms such as nausea, phonophobia, photophobia, nor the other autonomic features including conjunctival injection or tearing during the headache attacks. Physical and neurological examinations showed normal results except slight weakness and mild dysesthesia of the left arm due to a vertebral disk herniation at C5/6 level. In the pituitary endocrinological test, the prolactin level remarkably increased in response to the TRH loading. The single cosinor analysis demonstrated significant circadian rhythm of ABP parameters. However, the analysis did not demonstrate any significant circadian rhythm of Holter ECG parameters of time domain analysis and frequency analysis. Receiving 200 mg lithium bicarbonate before sleep, her nocturnal headache completely disappeared. Case 2: A 68-year-old woman had been followed up by her chronic tension-type headache since her forties. At her 66-years, she suffered from a new nocturnal headache. She awoke from sleep by the headache about 3 AM and the headache lasted 30 min. Moderate, dull headache located on her left temple to parietal head, 3-4 times/week. She was able to go back asleep without any medication after spontaneous headache cessation. She first complained the nocturnal headache at the 10 months later of the new headache appearance. She received 200 mg caffeine just before sleep and her headache has been disappeared. Case 3: 70-year-old women had been regularly visited our clinics for her migraine and chronic tension-type headache. She received amitriptyline and her headaches was well controlled. At her 69 years, she complained nocturnal headache. It occured every other day. The headache was moderate pulsative dull pain on the occipital region and lasted 90 minutes without any autonomic symptoms. Headache began between midnight and 1 AM. She told us her new nocturnal headache one year later of the onset. Oral caffeine (200 mg) just before sleep did not improve her headache and caused insomnia. Receiving 100 mg lithium before sleep, her hypnic headache disappeared completely. These three cases are compatible with the diagnostic criteria proposed in ICHD-II. There were some patients with hypnic headache in Japan and neurologists should pay attentions to this form of benign headache, because some beneficial treatments are currently available."}, {"id": "pubmed23n0799_20385", "title": "[Therapy of trigeminal autonomic headaches].", "score": 0.01886960391633289, "content": "Trigeminal autonomic cephalgias (TAC) are characterized by severe and strictly unilateral headaches with a frontotemporal and periorbital preponderance in combination with ipsilateral cranial autonomic symptoms, such as lacrimation, conjunctival injection, rhinorrhea, nasal congestion, and restlessness or agitation. One main differentiating factor is the duration of painful attacks. While attacks typically last 5 s to 10 min in SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing), paroxysmal hemicrania lasts 2-30 min and cluster headaches 15-180 min. Hemicrania continua represents a continuous TAC variant. From a therapeutic view, TACs differ substantially. Lamotrigine is used as first-choice prevention in SUNCT syndrome and indometacin in paroxysmal hemicrania. For cluster headaches, acute therapy with inhaled pure oxygen and fast-acting triptans (sumatriptan s.c. and intranasal zolmitriptan) is equally important to short-term preventive therapy with methysergide and cortisone and long-term prophylactic treatment comprising verapamil as drug of first choice and lithium carbonate and topiramate as drugs of second choice. In refractory cases of chronic cluster headache, neuromodulatory approaches such as occipital nerve stimulation and sphenopalatine ganglion stimulation are increasingly applied. "}, {"id": "pubmed23n0894_16750", "title": "Chronic Cluster Headache with an Atypical Presentation and Treatment Response.", "score": 0.016809629219701163, "content": "The management of cluster headache (CH) may be challenging. We report a 50-year-old male with recurrent attacks of dull and severe unilateral periorbital pain, lasting 30-45 minutes, twice a day, exclusively during sleep, and accompanied by ipsilateral rhinorrhea and lacrimation. The pain switched sides within every attack. CH treatment was initiated but the patient maintained recurrence rates compatible with chronic CH, even after increasing verapamil to 460 mg/day. Afterwards we decided to add lithium (800 mg/day). With this treatment the severity and recurrence of CH substantially decreased, despite the patient's autonomous decision to take lithium only during the acute phase of the cluster. The exclusively alternating location and the excellent response to short cycles of lithium represent two unique features of CH."}, {"id": "wiki20220301en003_97397", "title": "Headache", "score": 0.016351457840819542, "content": "Primary Ninety percent of all headaches are primary headaches. Primary headaches usually first start when people are between 20 and 40 years old. The most common types of primary headaches are migraines and tension-type headaches. They have different characteristics. Migraines typically present with pulsing head pain, nausea, photophobia (sensitivity to light) and phonophobia (sensitivity to sound) Tension-type headaches usually present with non-pulsing \"bandlike\" pressure on both sides of the head, not accompanied by other symptoms. Such kind of headaches maybe further classified into-episodic and chronic tension type headaches Other very rare types of primary headaches include: cluster headaches: short episodes (15–180 minutes) of severe pain, usually around one eye, with autonomic symptoms (tearing, red eye, nasal congestion) which occur at the same time every day. Cluster headaches can be treated with triptans and prevented with prednisone, ergotamine or lithium."}, {"id": "pubmed23n1019_19444", "title": "Pharmacotherapy for Cluster Headache.", "score": 0.015105908584169454, "content": "Cluster headache is characterised by attacks of excruciating unilateral headache or facial pain lasting 15 min to 3 h and is seen as one of the most intense forms of pain. Cluster headache attacks are accompanied by ipsilateral autonomic symptoms such as ptosis, miosis, redness or flushing of the face, nasal congestion, rhinorrhoea, peri-orbital swelling and/or restlessness or agitation. Cluster headache treatment entails fast-acting abortive treatment, transitional treatment and preventive treatment. The primary goal of prophylactic and transitional treatment is to achieve attack freedom, although this is not always possible. Subcutaneous sumatriptan and high-flow oxygen are the most proven abortive treatments for cluster headache attacks, but other treatment options such as intranasal triptans may be effective. Verapamil and lithium are the preventive drugs of first choice and the most widely used in first-line preventive treatment. Given its possible cardiac side effects, electrocardiogram (ECG) is recommended before treating with verapamil. Liver and kidney functioning should be evaluated before and during treatment with lithium. If verapamil and lithium are ineffective, contraindicated or discontinued because of side effects, the second choice is topiramate. If all these drugs fail, other options with lower levels of evidence are available (e.g. melatonin, clomiphene, dihydroergotamine, pizotifen). However, since the evidence level is low, we also recommend considering one of several neuromodulatory options in patients with refractory chronic cluster headache. A new addition to the preventive treatment options in episodic cluster headache is galcanezumab, although the long-term effects remain unknown. Since effective preventive treatment can take several weeks to titrate, transitional treatment can be of great importance in the treatment of cluster headache. At present, greater occipital nerve injection is the most proven transitional treatment. Other options are high-dose prednisone or frovatriptan."}, {"id": "pubmed23n0727_15440", "title": "Management of cluster headache.", "score": 0.01505752312203925, "content": "The prevalence of cluster headache is 0.1% and cluster headache is often not diagnosed or misdiagnosed as migraine or sinusitis. In cluster headache there is often a considerable diagnostic delay - an average of 7 years in a population-based survey. Cluster headache is characterized by very severe or severe orbital or periorbital pain with a duration of 15-180 minutes. The cluster headache attacks are accompanied by characteristic associated unilateral symptoms such as tearing, nasal congestion and/or rhinorrhoea, eyelid oedema, miosis and/or ptosis. In addition, there is a sense of restlessness and agitation. Patients may have up to eight attacks per day. Episodic cluster headache (ECH) occurs in clusters of weeks to months duration, whereas chronic cluster headache (CCH) attacks occur for more than 1 year without remissions. Management of cluster headache is divided into acute attack treatment and prophylactic treatment. In ECH and CCH the attacks can be treated with oxygen (12 L/min) or subcutaneous sumatriptan 6 mg. For both oxygen and sumatriptan there are two randomized, placebo-controlled trials demonstrating efficacy. In both ECH and CCH, verapamil is the prophylactic drug of choice. Verapamil 360 mg/day was found to be superior to placebo in one clinical trial. In clinical practice, daily doses of 480-720 mg are mostly used. Thus, the dose of verapamil used in cluster headache treatment may be double the dose used in cardiology, and with the higher doses the PR interval should be checked with an ECG. At the start of a cluster, transitional preventive treatment such as corticosteroids or greater occipital nerve blockade can be given. In CCH and in long-standing clusters of ECH, lithium, methysergide, topiramate, valproic acid and ergotamine tartrate can be used as add-on prophylactic treatment. In drug-resistant CCH, neuromodulation with either occipital nerve stimulation or deep brain stimulation of the hypothalamus is an alternative treatment strategy. For most cluster headache patients there are fairly good treatment options both for acute attacks and for prophylaxis. The big problem is the diagnosis of cluster headache as demonstrated by the diagnostic delay of 7 years. However, the relatively short-lasting attack of pain in one eye with typical associated symptoms should lead the family doctor to suspect cluster headache resulting in a referral to a neurologist or a headache centre with experience in the treatment of cluster headache."}, {"id": "pubmed23n0299_4940", "title": "[Cluster-tic syndrome: two case reports].", "score": 0.014266435319066899, "content": "Two patients with cluster-tic syndrome are reported. The first, a 43-years-old man, complaining of trigeminal pain in the right side of the face, accompanied by homolateral autonomic signs, such as ocular injection, sweating and drooped eyelid. The cluster attack was triggered by chewing, shaving and washing the face. The periodicity of bouts was six months. The pain was relieved by carbamazepine (800 mg/day). The second patient, a 43-year-old man, with an excruciant, neuralgic pain in the left side of the face, accompanied by tearing, conjuntival injection, drooped eyelid, rhinorrhea, photophobia and phonophobia. The neurologic examination showed triggered points in the first and second division of the trigeminal nerve. The patient was treated with verapamil (160 mg/day) and prednisone (60 mg/day), with relief of his symptoms. The periodicity of bouts was once a year. The literature was reviewed and 37 cases previosly reported are considered. We conclude that there are two different groups of patients. In the first group, the patients had cluster and trigeminal bouts in different time. In the second group, with only nine cases, the patients presented both cluster and trigeminal type of pain at the same time, as in the two cases reported here."}, {"id": "pubmed23n0989_21248", "title": "Hypnic headache: A review of 348 cases published from 1988 to 2018.", "score": 0.01250486949746786, "content": "Hypnic headache (HH) is a rare benign disorder described initially by Raskin in 1988. It is characterized by recurrent nocturnal episodes of headache that periodically awaken the sleeping patient and usually occur in the elderly. This review aimed to describe the clinical features of the HH cases published in the literature from 1988 to 2018. Based on literature search in the major medical databases (LiLacs, SciELO, Bireme, Medline, Embase, Current Contents, Scopus, EBSCO and PubMed), we have analyzed the case reports on HH that have been published from 1988 to 2018. We described 343 adults (69.0% women and 31.0% men) and 5 children (3 girls and 2 boys) diagnosed with HH. Average age for adults and children was, respectively, 58.0 ± 13.1 years (ranging from 15 to 85 years) and 9 years (ranging from 7 to 11 years). The diagnosis was made 7.6 ± 14.2 years (range 0.1 to 39 years) after onset of headache. Pain occurred during nocturnal sleep (94.8%), with an average duration of 90 min, bilaterally located (55.5%), having a dull character (74.4%), and moderate intensity (61.5%). In 94.5% of the patients, headache occurred for 10 or more days per month (mean of 21 days). Autonomic manifestations occurred in 7.6% of the patients, predominantly lacrimation (61.1%) and rhinorrhea (16.7%). Caffeine presented the best therapeutic response in acute treatment. In prophylaxis, lithium, caffeine and indomethacin were effective drugs in 77.8% of the patients. In 56.7% of the patients there was remission with treatment and in 72.7% of them, without recurrence. HH is a rare disease that usually occurs for the first time in older women but may begin in childhood. Lithium and caffeine are effective drugs for pain prophylaxis, but randomized clinical trials are required."}, {"id": "wiki20220301en223_26255", "title": "Hypnic headache", "score": 0.012110616656071202, "content": "Treatments Lithium carbonate 200–600 mg at bedtime is an effective treatment for most patients but for those that can not tolerate Lithium, Verapamil, indomethacin, melatonin or methysergide may be tried. Two patients have also responded to flunarizine 5 mg. It has also been shown that 1–2 cups of coffee or 100–200 mg of caffeine before bed can prevent hypnic headaches. A recent review of 348 cases available in the literature has been recently published. References External links Headaches"}, {"id": "wiki20220301en053_14549", "title": "Chronic paroxysmal hemicrania", "score": 0.010919658320368685, "content": "Treatments A ten-patient study conducted by Pareja et al. found that all patients diagnosed with CPH were responsive to indomethacin and were able to completely control their symptoms. Doses of the drug ranged from 25 mg per day to 150 mg per day with a median dose of 75 mg per 24-hour period. Almost all cases of CPH respond positively and effectively to indometacin, but as much as 25 percent of patients discontinued use of the drug due to adverse side effects, namely complications in the gastrointestinal tract. According to a case study by Milanlioglu et al., 100mg of lamotrigine, an antiepileptic drug, administered twice daily alleviated all painful symptoms. No side effects were noted after two months of treatment. Dosage of lamotrigine was decreased to 50mg a day after the first two months, and no symptoms or side-effects were recorded after a three-month followup."}, {"id": "InternalMed_Harrison_30161", "title": "InternalMed_Harrison", "score": 0.010665857113944546, "content": "Many experts favor verapamil as the first-line preventive treatment for patients with chronic cluster headache or prolonged bouts. While verapamil compares favorably with lithium in practice, some patients require verapamil doses far in excess of those administered for cardiac disorders. The initial dose range is 40–80 mg twice daily; effective doses may be as high as 960 mg/d. Side effects such as constipation and leg swelling can be problematic. Of paramount concern, however, is the cardiovascular safety of verapamil, particularly Prednisone 1 mg/kg up to Verapamil 160–960 mg/d 60 mg qd, tapering over 21 days Gabapentinb 1200–3600 mg/d Melatoninb 9–12 mg/d aNot available worldwide. bUnproven but of potential benefit."}, {"id": "pubmed23n0516_19120", "title": "[Coexistance of cluster headache and hemicrania continua: a case report].", "score": 0.00980392156862745, "content": "We reported a 36-year-old man, who suffered from cluster headache (CH) associated with hemicrania continua (HC). The continuous, dull or pressure-type headache appeared on the same side of the CH during the third month of a prolonged cluster period, and fluctuated in the severity of pain. This headache was aggravated when the CH was ameliorated by the administration of lithium carbonate. This converse relationship between CH and HC persisted during an on-off trial of the lithium carbonate, and the HC was exacerbated again after the complete cessation of CH. Retrobulbar pain and nasal congestion were present as components of HC similarly to CH, but they subsided gradually and the pressure-type vascular headache over the temporal area predominated later. The continuous headache lasted more than 3 months, and responded significantly to the indomethacin at a dose of 75mg/d. The clinical course of this patient suggests that HC and CH have a common pathomechanism including hyperactivation of the trigemino-vascular reflex, and may be different in the involvement of other central pathway of pain generation. Indomethacin may deserve consideration for the treatment of continuous headache that appears during an atypical course of other primary headaches."}, {"id": "pubmed23n0091_13090", "title": "Shortlasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, sweating, and rhinorrhea.", "score": 0.009708737864077669, "content": "Three grown-up males with a long-lasting history of rather uniform, unilateral headache in the ocular-periocular area, in cluster fashion, are examined. Pain paroxysms of short duration (15-60 sec) appear up to 5-30 times per h. The headache is unilateral without side shift. Conjunctival injection appears at the very beginning of the attack and is partly massive, lasting the entire duration of the attack, and fading away at the end of it. Tearing (massive), forehead sweating (subclinical) and rhinorrhea, all on the symptomatic side, accompany the attack. In the youngest patient, the headache became chronic after clustering for six months initially, and after approximately 3 1/2 years it became bilateral. However, even in this patient, a clear unilateral pain preponderance prevails, and the autonomic disturbances are all on the original pain side. Attacks can partly be precipitated by chewing, eating (e.g. citrus fruits), moving the head, etc. The headache is completely refractory to drug therapy, including indomethacin."}, {"id": "pubmed23n0024_5886", "title": "[Lithium treatment of chronic Horton's headaches].", "score": 0.009708737864077669, "content": "The authors used lithium carbonate in treatment of 7 patients with Horton's headaches of primarily or secondarily chronic character. In all patients the blood level of lithium was determined and it was found to reach therapeutic levels. Disappearance of attacks was achieved in 3 cases, significant improvement in 2, and in 2 cases treatment was ineffective. The mechanism of lithium action in this disease is discussed. The authors recommend lithium as worthy of use since other drugs are ineffective in this disease or they cannot be used, eg. steroids or indomethacin, in view of frequent coexistence of paptic ulcer."}, {"id": "pubmed23n0511_15265", "title": "Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome treated with microvascular decompression of the trigeminal nerve: case report.", "score": 0.009615384615384616, "content": "Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome is a very rare disorder characterized by short-lasting neuralgiform unilateral pain affecting the orbital-periorbital area and associated with autonomic phenomena consisting mainly of conjunctival injection, tearing, and rhinorrhea. Treatment of this condition is difficult; many drugs and surgical procedures have been tried with variable results. In the literature, two cases have been described with short-term response to microvascular decompression of the trigeminal root. We present the case of a patient with short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome who remains asymptomatic 2 years after microvascular decompression. A 56-year-old woman was referred to our clinic because she had experienced pain in the distribution of the first left trigeminal branch during the previous 2 years. She experienced paroxysms lasting from a few seconds to 1 to 2 minutes superimposed over a dull sensation of pain involving the same territory. The paroxysms had no refractory period and were triggered by touching the eye or the left side of the face, chewing, yawning, washing her hair, and even by light. Although the paroxysms were triggered by light touch or chewing, she was able to talk or touch herself while having the paroxysm. During pain attacks, she experienced tearing and ipsilateral conjunctival injection, eyelid edema and rhinorrhea, as well as intense photophobia. A magnetic resonance imaging scan revealed a vascular structure distorting and compressing the trigeminal root. The patient underwent microvascular decompression of the trigeminal root. At surgery, there was clear compression of the trigeminal root by a superior cerebellar artery loop that was resolved by interposing a Teflon patch. The patient awoke from the operation without pain, and all the accompanying signs and symptoms, such as photophobia, disappeared. The postoperative course was uneventful, and 2 years after treatment, the patient remains asymptomatic. Microvascular decompression could be an alternative therapeutic approach to this rare syndrome."}, {"id": "pubmed23n0388_5817", "title": "Episodic paroxysmal hemicrania with seasonal variation: case report and the EPH-cluster headache continuum hypothesis.", "score": 0.009523809523809525, "content": "Episodic paroxysmal hemicrania (EPH) is a rare disorder characterized by frequent, daily attacks of short-lived, unilateral headache with accompanying ipsilateral autonomic features. EPH has attack periods which last weeks to months separated by remission intervals lasting months to years, however, a seasonal variation has never been reported in EPH. We report a new case of EPH with a clear seasonal pattern: a 32-year-old woman with a right-sided headache for 17 years. Pain occurred with a seasonal variation, with bouts lasting one month (usually in the first months of the year) and remission periods lasting around 11 months. During these periods she had headache from three to five times per day, lasting from 15 to 30 minutes, without any particular period preference. There were no precipitating or aggravating factors. Tearing and conjunctival injection accompanied ipsilaterally the pain. Previous treatments provided no pain relief. She completely responded to indomethacin 75 mg daily. After three years, the pain recurred with longer attack duration and was just relieved with prednisone. We also propose a new hypothesis: the EPH-cluster headache continuum."}, {"id": "pubmed23n0353_2129", "title": "[Carotidynia as a form of presentation of paraxysmal hemicrania].", "score": 0.009433962264150943, "content": "Paroxysmal hemicrania is a well-defined clinical condition about which many articles have been published. Attempts have been made to explain the response of this illness to indomethacin, suggesting its possible cervical origin. In some patients it is set off by stimulation of certain trigger zones situated in this region. The exceptional radiation of the pain seen in our patient clearly supports this theory. A 34 year old man with a past history of a similar but briefer episode 5 years previously presented to us. He complained of repeated episodes of stabbing pain with no obvious cause. The pain started at the base of the neck and radiated along the right carotid vessels to the cheek, base of the nose and ipsilateral eye. This was accompanied by injection of the conjunctivae, tears, nasal congestion and nasal discharge. Each episode lasted 15 to 30 minutes and was repeated 20 to 25 times a day without any particular relation to the time of day. The neurological examination, MR and angio-MR were normal. Before being seen by us he had been treated with prednisone and verpamil without effect. Indomethacin at a dose of 100 mg/day controlled the problem completely. We report a case of paroxysmal hemicrania with a spontaneous description of pain starting at the base of the neck and radiating along the carotid vessels. We consider this clinical description to be of interest since it supports the theories of a cervicogenic origin of this type of headache."}, {"id": "pubmed23n0083_9295", "title": "Lithium-induced headache.", "score": 0.009433962264150943, "content": "A 23-year old woman developed headache and papilledema due to benign intracranial hypertension (BIH) while taking lithium carbonate for only seven months because of manic-depressive disease. Having discarded other causes, drug ingestion was the most likely etiology of the syndrome since it was observed that symptoms improved upon lithium withdrawal and worsened when the treatment was restarted. This report shows that BIH may appear as a side-effect of relatively short-term therapy with lithium and, therefore, funduscopic exams should be performed in every patient receiving this drug."}, {"id": "pubmed23n0060_210", "title": "Treatment of the elderly patient with headache or trigeminal neuralgia.", "score": 0.009259259259259259, "content": "The elderly as a whole suffer fewer headaches than the young. For the majority headache will represent a minor annoyance to be endured or treated with any available drug in the medicine chest. For some, migraine headaches or tension-type headaches become entwined with every daily activity. With the advent of modern pharmacology, headache can often be treated successfully. Trigeminal neuralgia is a source of particularly high morbidity among the elderly, but may be treated very satisfactorily with carbamazepine or baclofen. Paroxysmal hemicrania is exquisitely sensitive to indomethacin, while cluster headache patients receive relief from oxygen inhalation, corticosteroids or lithium. Headache may be the signature of the disease which leads to serious morbidity and mortality. The 'sentinel' headache of subarachnoid haemorrhage is evaluated by a physician in 15% of patients who will eventually rupture an intracranial aneurysm. Morning headache with nausea and vomiting may represent increased intracranial pressure caused by a tumour, haematoma or abscess. The elderly patient with a new headache needs emergency evaluation for temporal arteritis and rapid corticosteroid treatment if the diagnosis is confirmed, to prevent blindness. The broad spectrum of headache, at times a benign aggravation, while at others the harbinger of death, makes the careful evaluation of each headache imperative. This article attempts to make the difficult evaluation of head pain a little easier."}, {"id": "pubmed23n0550_3400", "title": "SUNCT syndrome associated with pituitary tumor: case report.", "score": 0.009174311926605505, "content": "For twelve years, the subject of this report, a 38-year-old man, presented a clinical condition compatible with the SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) syndrome. He presented a stabbing and intense daily pain located in the left pre-auricular and temporal regions. Each of these intense pain attacks lasted around one minute and presented a frequency of two to eight times per day. The pain was associated with ipsilateral lacrimation, conjunctival injection and rhinorrhea. MRI revealed a pituitary tumor with little suprasellar extent. The subjects serial assays of prolactin, GH, TSH and ACTH were within normal levels. Following transsphenoidal hypophysectomy, with complete removal of the tumor, the subject no more presented pain. The pathological diagnosis was non-secreting adenoma. Fourteen months after the surgery, he remains symptom-free."}, {"id": "pubmed23n0310_15508", "title": "Double-blind placebo-controlled trial of lithium in episodic cluster headache.", "score": 0.009174311926605505, "content": "Lithium is widely used in the prophylaxis of episodic cluster headache without formal evidence of efficacy. Placebo-controlled clinical trials are not easy in conditions characterized by frequent severe pain. In this study, it was assumed that lithium would work quickly if at all, and placebo response would be zero. Strict diagnostic criteria excluded uncertain or atypical cases. Patients were male in so-far untreated episodes expected to last for at least 3 weeks more. In a double-blind, placebo-controlled comparison of matched parallel groups, treatment was either slow-release lithium carbonate, 800 mg/day, or placebo. After 7 days, compliance was estimated by tablet count, blood was taken for lithium assay, efficacy was assessed (attacks stopped or substantially improved) and adverse reactions were recorded. The study was stopped after planned sequential analysis of the 27th patient (13 on lithium, 14 on placebo). Estimated compliance was usually but not always good. Plasma lithium levels were mostly in the range 0.5-0.6 mmol/l on lithium, zero on placebo. Cessation of attacks within 1 week occurred in two patients in each group, substantial improvement in 6/14 (43%) on placebo, 8/13 (62%; NS) on lithium. Only minor adverse events were reported. Lithium treatment was therefore associated with a useful subjective improvement rate but the assumptions made at outset had proved wrong. The trial was stopped because superiority over placebo could not be demonstrated. There were lessons for future trials."}, {"id": "pubmed23n0395_16097", "title": "[Amnesic presentations of the compulsive obsessional confusions (about 3 patients appearing in a consultation of memory)].", "score": 0.00909090909090909, "content": "Disorders or complaints of memory are a frequent cause of consultation in depression, major anxiety and psychiatry disease with personality disorders. We report 3 patients with obsessive compulsive disorder (OCD), without diagnosis and treatment, examined in a specialized memory consultation. They always had OCD with cognitive checking. Diagnosis of transient global amnesia and temporal complex seizure were discussed in 2 cases. Psychometric impairment only was observed in first free recall of a verbal memory task and was no specific. Behavioural during testing seemed to be very important to analyse. First, a 49-year-old man consulted because he had stereotyped transient amnesia lasted one minute, 2 or 3 times a week, since 6 months. He was a teacher. Transient amnesia always occurred during lessons. Suddenly he didn't know where he was or what he was speaking about. Episodes lasted one minute. After them, he had no confusion and no difficulty in concentration but intense anxiety. In an another hand, when he was in his car, after lessons, he could forget where he was during some minutes. CT scan and EEG were normal. Neuropsychological tests only objectived impairment in first free recall of Grober and Buschke's words. Patient explained that he could not prevent to check responses. He told us checking obsessive compulsive disorder during since long time ago. We discussed clear differences which existed between seizure and ruminations or preoccupations. Secondly, a 55-year-old woman was afraid of her memory performances. She was medical secretary and had no problem in her work but she would like a memory consultation to reassure herself. She was neither depressed nor anxious. She presented curious production in fluency task. She had to produce as many animals's names as possible: she could say 35 names which was an excellent performance but only in alphabetic order! Neuropsychological tests objectived impairment in her first free recall of Grober and Buschke's words. She tried in her first free recall to remember words in alphabetic order. She explained how she was bound to range everything in alphabetic order! She had a lot of rituals. She thought that she had an obsessive compulsive disorder but never consulted about this. The observation illustrated suspiscions about memory operations which could be observed in patients group with obsessive compulsive disorders. Finally, a 62-year-old man told us that he had presented a transient global amnesia during 4 hours. He had an important appointment and was upset about that. He didn't go to it and wandered in his flat. He always asked the same questions and forgot everything. He had no neurological deficit. He was anxious, sad and cried several times. He perfectly remembered the episod and thought that he had a panic attack! Verbal memory tests only objectived difficulties in his first free recall of Grober and Buschke words as the two others patients. He had a story of obsessive compulsive disorder with checking and rituals. In this observation, we discussed clear differences which existed between panic attacks and global transient amnesia. We analyzed patterns of neuropsychological performances which illustrated clinical features of obsessive compulsive disorder. These three patients impaired in their first free recall of verbal memory task. It is not a specific result. We observed during psychometric evaluation, strategic processing which impaired episodic memory: patients tried to check their performances. Memory complaints only were observed in checking obsessive compulsive disorder. It is a difficulty or a doubt about memory capacities. Difficulties could be due to particular cognitive processes who pertubate normal memory capacities."}, {"id": "pubmed23n0386_2359", "title": "[Lithium treatment and hyperparathyroidism].", "score": 0.00909090909090909, "content": "Lithium treatment, which is extensively used in bipolar affective disorders, may give rise to hypercalcaemia and sometimes to irreversible hyperparathyroidism. We present a patient who developed hyperparathyroidism following long-term treatment with lithium. After 15 years on lithium the patient was diagnosed with hypercalcaemia; at the same time the patient stopped her lithium medication. Two years later she developed depression with psychotic symptoms and was given electroconvulsive treatment. Measurements of serum calcium and parathormon showed that she had developed hyperparathyroidism. Neck exploration was performed, and two parathyroid adenomas (weight 650 mg and 880 mg), which had been detected by scintigraphy, were removed. Lithium treatment was restarted. One year later she was normocalcaemic and her mood was normal. In lithium-induced hyperparamyroidism, lithium should be replaced with other mood stabilizers, preferably an antiepilepticum. If cessation of lithium therapy does not lead to normocalcaemia, parathyroidectomy is indicated."}, {"id": "pubmed23n0541_13197", "title": "Side-locked headache as the chief complaint of inflammatory orbital pseudotumor (myositic form): a case report.", "score": 0.009009009009009009, "content": "The case of a 38-year-old woman with continuous unilateral side-locked headache is reported. She had continuous right-sided periorbital pain of mild to moderate intensity for the past 5 months. She also reported a few episodes of pain exacerbations every day. She had no autonomic features. Based on a normal CT scan ordered by her general physician, we started indomethacin (150 mg/day) as well as celecoxib (400 mg/day) for 2 weeks, without relief. Oral prednisone for 6 days provided important relief, and she stayed on daily use of steroids, refusing other forms of therapy. After 5 months she developed orbital and eyelid edema, with painful restrictions to eye movement. Orbital MRI and pathological exam demonstrated inflammatory orbital pseudotumor (myositic form)."}, {"id": "pubmed23n0585_10748", "title": "[Cluster headache and other trigeminal-autonomic headaches].", "score": 0.009009009009009009, "content": "Cluster headache is a primary headache with a male predominance that presents in two forms: episodic and chronic, occurring at 45-to 60-day intervals with one to three headaches a day lasting 45 min to 2 h. An attack starts by a violent unilateral retro-ocular pain with sympathetic signs such as tearing and rhinorrhea. Diagnosis is made by questioning and therefore requires no complementary tests. Treatment for the attack consists of injectable sumatriptan or oxygen therapy, with long-term treatment with verapamil, lithium salts, or Topiramate; in certain cases in which the number of attacks is greater than two, injections of corticosteroids at the emergence of the Arnold nerve can be used, or in cases of attacks resistant to all treatments, hypothalamus stimulation surgery can be useful."}, {"id": "pubmed23n0366_2044", "title": "SUNCT syndrome responsive to gabapentin (Neurontin).", "score": 0.008928571428571428, "content": "A 48-year-old male suffering with SUNCT (severe unilateral neuralgiform headache with conjunctival injection and tearing, rhinorrhea and sub-clinical sweating) presented in 1996 after a 10-year history of multiple failed therapies. The symptoms included strictly left-sided ocular, as well as facial and temple pain. The pain attacks were burning, sharp, shooting and occurred 25 times daily, lasting 2 to 3 minutes with tearing and conjunctival injection. There was no associated nausea or vomiting, but there was photophobia. No other autonomic changes were reported and the pain was not triggerable. Initially Indocin (indomethacin) was tried without significant benefit. Gabapentin (Neurontin) was then started with improvement at 1800 mg per day. The patient was then lost to follow-up for 3 years, as he moved from the Los Angeles area. He returned in 1999 having stopped the gabapentin after his prescription ran out in 1996, reporting the pain returned immediately. Again gabapentin was prescribed and at 900 mg three times daily he has been pain free for 12 months."}, {"id": "pubmed23n0248_4039", "title": "[Lithium therapy in Horton's neuralgia: preliminary results].", "score": 0.008928571428571428, "content": "Prophylactic use of Lithium salts in patient suffering from cluster headaches has been evaluated looking at the mean number of headache attacks in one critical period and the mean weekly duration of the period itself. These two elements have been compared to those observed on other drugs treatments. Plasma Lithium monitoring has been performed weekly during the trial. Authors discuss the results reported and the hypothetic basis of them."}, {"id": "Neurology_Adams_1432", "title": "Neurology_Adams", "score": 0.008924003865173648, "content": "Raskin described a headache syndrome in older patients that shares with cluster headache a nocturnal occurrence (hypnic headache). It also may occur with daytime naps. However, it differs in being bilateral and unaccompanied by lacrimation and rhinorrhea. He has successfully treated a number of his patients with 300 mg of lithium carbonate or 75 mg of sustained-release indomethacin at bedtime. The nosologic position of this hypnic headache syndrome is undetermined. Despite these considerations, the most hazardous cause of headache in the elderly is temporal (cranial) arteritis with or without polymyalgia rheumatica, as discussed further on."}, {"id": "pubmed23n0525_8405", "title": "[Recurrent aseptic osteonecrosis in Crohn's disease - extraintestinal manifestation or steroid related complication?].", "score": 0.008849557522123894, "content": "A 57-year-old woman complained about increasing pain and weakness in her hips and legs. 7 months earlier active (Crohn's) ileocolitis had been diagnosed. She had received several bouts of steroids and had been in clinical remission for 12 weeks under a dosage of 40 mg/d prednisone. Clinical examination, laboratory work up, x-rays and MRI of the pelvis, bone scan, neurologic examination and muscle biopsy showed unspecific results. THERAPY AN COURSE: Steroides were tapered and replaced by weekly intramuscular methotrexate 20 mg which resulted in long lasting clinical remission. Pain and weakness persisted. 6 months later MRI revealed osteonecrosis of both femoral heads. 4 1/2 years after the initial diagnosis of Crohn's disease the patient complained about pain in her lower legs without evidence of osteonecrosis in MRI. Another 2 years later avascular osteonecrosis was diagnosed by tibial bone biopsy. Now MRI verified patchy osteonecrosis of the tibiae. Further osteonecrosis of the left foot were diagnosed by MRI ten years after initial diagnosis of Crohn's disease in the now 67-year-old patient. She is still in remission on weekly intramuscular 15 mg methotrexate. The long interval between steroid treatment and recurrent avascular bone necrosis as well as the unusual pattern of bone involvement indicate that osteonecrosis is an extraintestinal manifestation of Crohn's disease. More reports and comparative studies are necessary to give more evidence that avascular osteonecrosis is an extraintestinal manifestation of inflammatory bowel disease."}, {"id": "pubmed23n0130_15376", "title": "Pseudotumor cerebri secondary to lithium carbonate.", "score": 0.008849557522123894, "content": "Three patients were initially seen with headache, blurred vision, and papilledema while taking lithium carbonate for their respective bipolar affective disorder. A diagnosis of pseudotumor cerebri was made in each case when a thorough evaluation revealed only elevated intracranial pressure. Two of the patients had complete resolution of their symptoms and papilledema after discontinuing use of the drug. Increased intracranial pressure with papilledema persisted in the third patient when she failed to adjust psychiatrically, necessitating continuance of the lithium carbonate therapy. A history of lithium carbonate ingestion should be sought in patients with the syndrome of pseudotumor cerebri. All patients receiving this drug should have a regular funduscopic examination."}, {"id": "pubmed23n0743_9725", "title": "[Complete atrioventricular block during lithium therapy within  therapeutic range].", "score": 0.008771929824561403, "content": "A 69-year-old man came to the emergency unit because of vertigo and presyncope. A bipolar disorder - known since an age of 15 years - has been treated with 2 × 450 mg lithium and 100 mg perazine per day for several years (no other medications). With the exception of a low heart rate (36/min) clinical examination findings were unremarkable. Electrocardiography revealed a permanent complete atrioventricular block with a heart rate of 36/min. Echocardiography showed a normal left ejection fraction (EF 65 %). Laboratory tests were mainly unremarkable, particularly the lithium levels (0,7 mmol/l) were within the therapeutic range. Continuous treatment with orciprenaline stabilized the heart rate at an average of 52/min. After pacing with a provisional pacemaker a permanent pacemaker was implanted without complications, and the symptoms of vertigo and dizziness disappeared. Pacemaker checkup on the following day still showed a complete atrioventricular block with a heart rate of 28/min. Complete atrioventricular block secondary to chronic lithium therapy even in therapeutic levels is a rare complication with poor prognosis. Therefore it should be treated consequently."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[25, 138]], "word_ranges": [[5, 25]], "text": "If I am not mistaken, you are describing a Celso's kerion for which the treatment of choice is oral griseofulvin."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The correct answer is 3. If I am not mistaken, you are describing a Celso's kerion for which the treatment of choice is oral griseofulvin.", "full_answer_no_ref": "The [HIDDEN] If I am not mistaken, you are describing a Celso's kerion for which the treatment of choice is oral griseofulvin.", "full_question": "A 6-year-old boy comes to the clinic accompanied by the monitor of a day care center in our neighborhood because of a painful lump 3 cm in diameter on palpation in the right occipital area of the scalp. He suffers from alopecia in this area and 3 adenomegalies of quite hard consistency in the right posterior cervical region. What would be the most appropriate treatment?", "id": 47, "lang": "en", "options": {"1": "Incision and drainage.", "2": "Topical Mupirocin.", "3": "Griseofulvin orally.", "4": "Intravenous cefazolin.", "5": "Topical Ketoconazole."}, "question_id_specific": 157, "type": "PEDIATRICS", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0717_24038", "title": "Unusually severe case of dermatosis neglecta.", "score": 0.019149715961150816, "content": "An 18-year-old black woman with cerebral palsy was admitted for evaluation of an intrathecal baclofen pump site infection. The dermatology service was consulted for treatment suggestions of a presumed diagnosis of chronic tinea capitis. Three courses of oral griseofulvin during the past 2 years failed to resolve the patient's chronic scalp dermatosis. Scalp lesions first began about 2 years earlier after hospitalization for placement of an intrathecal baclofen pump. The patient was unable to care for her scalp due to her cerebral palsy, and her mother interpreted the scalp condition as infectious. No routine shampoo care, scalp care, or topical treatment was performed for more than 1 1/2 years. The mother felt that touching the patient's scalp might cause pain and noted that the majority of her time was spent concentrating on more critical medical issues. Physical examination revealed coalescing hyperkeratotic plaques extending dorsally from the anterior hairline to the occipital scalp with small flecks of keratinous debris throughout the remaining hair (Figure 1). The plate-like plaques were devoid of hair, except at a few fissures where a few tufts of hair emerged. No cervical lymph nodes were appreciated on palpation. Treatment was initiated with compresses consisting of large warm water-soaked towels 4 times daily. Three times a day, a nursing staff applied 5% salicylic acid in olive oil to the scalp under a shower cap for approximately 1 hour. Over the following 2 days, a significant reduction in keratinous debris was appreciated. Within 2 weeks, the bulk of the plaques had been removed (Figure 2). At 6-week follow-up, the underlying scalp showed areas of fibrosis and possible scarring with a few emerging tufts of hair. On the basis of history and response to treatment with salicylic acid and routine scalp care, the patient was diagnosed with an unusually severe case of dermatosis neglecta."}, {"id": "pubmed23n0344_3092", "title": "[Tinea capitis and corporis caused by Trichophyton soudanense in an immigrant family from Africa].", "score": 0.014863387978142076, "content": "Several weeks before coming to Germany the two daughters (aged 3 and 6 years) of a family from Togo had developed desquamating skin changes over the hairy scalp. These had then spread to the trunk and limbs. The 8-weeks-old son also had discrete lesions on the hairy scalp and neck. In all of them these lesions had then spread and begun to itch markedly. When first seen as out-patients the father was free of symptoms, but the other members of the family had multiple, sharply circumscribed, partly confluent, dry and desquamating lesions, about 2-4 cm in diameter, with areas of alopecia and hair breaking off at skin level. In addition there were dry, desquamating, sharply circumscribed, partly hyperpigmented, partly infiltrated plaques, 1-3 cm in diameter, disseminated over the entire body surface, but especially the neck and limbs. Typical micromorphological characteristics for T. soudanese were demonstrated in the outer zones of a primary culture and the organism was also demonstrated in culture on Sabouraud-glucose-agar. Typical colonies on Löwenstein-Jensen medium allowed differentiation from Microsporum ferrugineum. The patients were treated systemically with griseofulvin and locally with ciclopiroxolamine. Marked clinical improvement occurred within 2 months and cultures became negative. But as fungal elements were still demonstrated in native preparations from two of the patients, treatment was continued. Efficacious treatment of tinea needs reliable diagnosis of the pathogen. Human infection with T. soudanese usually results from contact with other humans. If this infection occurs in persons not from Africa there is usually the history of indirect or direct contact with Africans. Increased international migration and tourism is likely to result in more cases of this kind: this pathogen should be considered in the differential diagnosis of tinea of scalp and body."}, {"id": "pubmed23n0976_13284", "title": "Tinea versicolor of the neck as side effect of topical steroids for alopecia areata.", "score": 0.014531375703942076, "content": "<bBackground:</b Treatment of alopecia areata (AA) involves use of high potency topical corticosteroids under occlusion that, even very effective, can lead to several adverse effects. <bObjective:</b We report 10 cases of patients with AA that, after using high potency topical corticosteroids, have developed tinea versicolor of the neck area. <bMethods:</b Ten patients with AA, aged 18-38 years, were prescribed with clobetasone propionate 0.05% cream under occlusion every other day but, after 3-4 months of treatment, they returned to our facility complaining the appearance of multiple white or red-brown round or oval macules in the neck area. <bResults:</b Diagnosis of pityriasis versicolor was confirmed by direct microscopy examination of skin scrapings in 10% potassion hydroxide (KOH) solution. All patients received systemic antifungal therapy associated with the daily use of ketoconazole shampoo. <bConclusion:</b Tinea versicolor of the neck should be included among a rare but possible side effect of prolonged application of high potency topical steroids on the scalp. These cases reinforce the importance of careful dermatologic examination and recommend preventive measures in patients with alopecia areata that are using these drugs."}, {"id": "pubmed23n0317_18365", "title": "[Mycetomas caused by Microsporum canis. Report of one case].", "score": 0.014238667183262301, "content": "We report an eight years old boy presenting with a pyogenic granuloma of the scalp, generalized alopecia, descamative plates in the neck, trunk and limbs and nail involvement. Cultures for fungus of all these lesions disclosed Microspore canis. The patient was treated with oral griseofulvin, miconazole and topical tolnaftate. Five years later and after several incomplete treatments, the patient returns with a fistulous mass of 15 x 8 cm in the dorsal area whose culture revealed Microspore canis. The mass was excised and oral ketoconazole was indicated. After three months of follow up, the patient was lost from control."}, {"id": "pubmed23n0049_20054", "title": "[A case of Bezold's abscess associated with cholesteatoma].", "score": 0.013553113553113554, "content": "Since the advent of antibiotics, otogenic complications have decreased considerably. However, incomplete antibiotic therapy has altered the clinical course of middle ear disease so as to be more insidious. This paper reports a case of Bezold's abscess associated with cholesteatoma. A 48-year-old man visited our hospital presenting with a 4-day history of right otorrhea and a tender swelling in the right neck. Physical examination showed a febrile patient (38.8 degrees C) with right facial paresis and trismus. A hyperemic, hard and tender swelling was observed in his right neck from the lateral cervical to the mental region. The tympanic membrane was invisible because of granulation and swelling of the posterior wall of the external auditory canal. Intravenous clindamycin and ceftazidime therapy was started immediately. A CT-scan revealed a diffuse shadow with bony destruction in the right mastoid cortex. Extensive abscess formation was also found in the right sternocleidomastoid muscle, in the anterior neck and in the posterior neck. He was diagnosed as having Bezold's abscess associated with cholesteatoma. Radical mastoidectomy and drainage of the neck abscess was performed on the third day under general anesthesia. The mastoid cavity was found to be filled with pus and cholesteatoma debris. A small area of defective bone was found at the mastoid tip, through which there were communications between the mastoid cavity and the abscesses in the neck. Bony destruction was also found in the horizontal and vertical portion of the facial canal. Bacteroides and three kinds of gram-negative rods were cultured from the mastoid cavity.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n1016_23343", "title": "Tinea Capitis: An Updated Review.", "score": 0.01213235294117647, "content": "Tinea capitis is a common and, at times, difficult to treat, fungal infection of the scalp. This article aimed to provide an update on the evaluation, diagnosis, and treatment of tinea capitis. A PubMed search was performed in Clinical Queries using the key term \"tinea capitis\". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to English literature. The information retrieved from the above search was used in the compilation of the present article. Patents were searched using the key term \"tinea capitis\" at www.freepatentsonline.com. Tinea capitis is most often caused by Trichophyton tonsurans and Microsporum canis. The peak incidence is between 3 and 7 years of age. Non-inflammatory tinea capitis typically presents as fine scaling with single or multiple scaly patches of circular alopecia (grey patches); diffuse or patchy, fine, white, adherent scaling of the scalp resembling generalized dandruff with subtle hair loss; or single or multiple patches of well-demarcated area (s) of alopecia with fine-scale, studded with broken-off hairs at the scalp surface, resulting in the appearance of \"black dots\". Inflammatory variants of tinea capitis include kerion and favus. Dermoscopy is a highly sensitive tool for the diagnosis of tinea capitis. The diagnosis can be confirmed by direct microscopic examination with a potassium hydroxide wetmount preparation and fungal culture. It is desirable to have mycologic confirmation of tinea capitis before beginning a treatment regimen. Oral antifungal therapy (terbinafine, griseofulvin, itraconazole, and fluconazole) is considered the gold standard for tinea capitis. Recent patents related to the management of tinea capitis are also discussed. Tinea capitis requires systemic antifungal treatment. Although topical antifungal therapies have minimal adverse events, topical antifungal agents alone are not recommended for the treatment of tinea capitis because these agents do not penetrate the root of the hair follicles deep within the dermis. Topical antifungal therapy, however, can be used to reduce transmission of spores and can be used as adjuvant therapy to systemic antifungals. Combined therapy with topical and oral antifungals may increase the cure rate."}, {"id": "pubmed23n0354_18608", "title": "Generalized pruritus without primary lesions. Differential diagnosis and approach to treatment.", "score": 0.011714770797962648, "content": "A 65-year-old man presented with recurrent generalized pruritus and excoriations of many years' duration. He had been treated with antihistamines, topical corticosteroids, and antibiotics for secondary wound infections, but improvement was only temporary. He had also been hospitalized for leg ulcers complicated by cellulitis. Examination revealed multiple oval and linear red papules and nodules measuring 0.5 to 2 cm in diameter. Some of the lesions were eroded and had a central crater and yellowish crust. The patient also had hypopigmented linear scars localized to the posterior scalp, neck, upper back, chest, abdomen, arms, and legs with sparing of the middle and lower back (figures 1 and 2). An ulcer measuring 1.5 x 2 cm that was surrounded by indurated skin was present on the medial aspect of his right ankle. The ulcer was partially covered by yellow exudate. There was no evidence of cellulitis. Liver enzyme, serum creatinine, and thyrotropin levels, as well as a chest roentgenogram, were normal. Wound cultures for bacteria and fungi were nonsignificant. A punch biopsy from a representative lesion showed an abrupt epidermal defect with sparse superficial lymphocytic infiltrate in the dermis. The patient was admitted to the hospital to isolate him from his home environment. He received a 10-day course of systemic cephalexin, topical clobetasol propionate ointment for the affected skin areas, and oral hydroxyzine for pruritus. Ultraviolet light therapy was instituted once daily and was to continue for 2 months. His lesions had improved moderately by the time he was discharged from the hospital. On follow-up 2 weeks later, his lesions were flat and had resulted in hypopigmented scars. Three months later, however, he had persistent, intense pruritus, and new excoriations had developed on his forearms and back. He improved after receiving treatment with oral doxepin hydrochloride."}, {"id": "wiki20220301en263_33876", "title": "Fungal folliculitis", "score": 0.011621711621711623, "content": "Treatment Oral antifungal medications are the standard of care. Due to the location of the dermatophytes within the hair follicle, treatment with topical antifungals is often unsatisfactory. In patients with tinea pedis or onychomycosis, re-inoculation and recurrence is common. In individuals with recurrent outbreaks, inoculation sources should be identified and treated appropriately. Historical therapies include oral potassium iodide, mildly filtered local X-radiation, and topical applications of Asterol as a fungicide in both tincture and ointment forms. In modern medicine, systemic antifungals, such as griseofulvin, ketoconazole, and itraconazole, are the standard. Therapy extends over at least 4–8 weeks, and treatment continues until all lesions are cleared. Currently, no data about relapse rates or the complications of not treating Majocchi granuloma exist."}, {"id": "wiki20220301en036_60803", "title": "Dermatophyte", "score": 0.011101973684210526, "content": "Treatment Tinea corpora (body), tinea manus (hands), tinea cruris (groin), tinea pedis (foot) and tinea facie (face) can be treated topically. Tinea unguum (nails) usually will require oral treatment with terbinafine, itraconizole, or griseofulvin. Griseofulvin is usually not as effective as terbinafine or itraconizole. A lacquer (Penlac) can be used daily, but is ineffective unless combined with aggressive debridement of the affected nail. Tinea capitis (scalp) must be treated orally, as the medication must be present deep in the hair follicles to eradicate the fungus. Usually griseofulvin is given orally for 2 to 3 months. Clinically dosage up to twice the recommended dose might be used due to relative resistance of some strains of dermatophytes."}, {"id": "article-32724_21", "title": "Black Piedra -- Treatment Planning", "score": 0.011062632620461102, "content": "Shaving the head (if culturally appropriate and with patient's willful consent) 2% ketoconazole or 2% miconazole shampoo or 1 to 1.5% ciclopirox shampoo applied once-to-twice-a-week for 3 to 4 weeks Oral terbinafine 250 mg once daily for 6 weeks Oral itraconazole 100 mg twice-a-day after a meal, with a citrus drink for 1 to 2 weeks Counseling on the maintenance of good scalp hygiene, avoidance of sharing combs, etc."}, {"id": "wiki20220301en021_83934", "title": "Scalp", "score": 0.011005775307834805, "content": "Lymphatic drainage Lymphatic channels from the posterior half of the scalp drain to occipital and posterior auricular nodes. Lymphatic channels from the anterior half drain to the parotid nodes. The lymph eventually reaches the submandibular and deep cervical nodes. Clinical significance Infection The 'danger area of the scalp' is the area of loose connective tissue. This is because pus and blood spread easily within it, and can pass into the cranial cavity along the emissary veins. Therefore, infection can spread from the scalp to the meninges, which could lead to meningitis."}, {"id": "pubmed23n0507_12488", "title": "Pimecrolimus-induced tinea incognito.", "score": 0.009900990099009901, "content": "A 6-year-old boy was brought to his primary care provider by his mother, who complained of a pruritic rash near his right eye. The eruption was described as a small, erythematous, slightly scaly plaque at the lateral margin of the right eyelid. The child was in good health and took no medications. The diagnosis of eczema was made; the patient was treated with pimecrolimus cream b.i.d. to the affected area. After 2-3 days of treatment, the itching and erythema completely resolved; however, a rough and scaly plaque persisted. After 1-2 weeks of treatment, the itching gradually returned, and the lesion began to increase in size. Multiple, similar lesions appeared several centimeters from the initially affected area. Pimecrolimus was discontinued; topical nystatin/triamcinolone ointment was prescribed. The eruption continued to spread, and the patient was referred to dermatology for further evaluation. The patient presented to the dermatology clinic with multiple annular, scaly papules and plaques with central clearing. Excoriations and mild inflammation were noted around all affected areas (Figure). A potassium hydroxide examination of the lesions revealed numerous hyphae. The nystatin/triamcinolone ointment was discontinued; oral griseofulvin was prescribed. The eruption improved dramatically after 3 weeks and eventually cleared completely after 5 weeks of treatment. Topical 2% ketoconazole cream was applied b.i.d. for the final 2 weeks of treatment."}, {"id": "pubmed23n0686_2732", "title": "[Furuncles on the scalp after a trip to Brazil].", "score": 0.009900990099009901, "content": "A 68-year-old woman suffered for six weeks from four skin eruptions on her head after returning from Brazil. The skin manifestations resembled furuncles, grew continually in size until they were about 2 cm in diameter and [corrected] she finally developed intermittent sharp pain on her head. On presentation she had a mild lymphadenopathy on her neck but no other systemic complaints. Each skin eruption had a central porus with seropurulent discharge and on examination within the central opening a whitish, tender moving mass could be detected. TREATMENT, COURSE AND DIAGNOSIS: We cautiously infiltrated each skin eruption with lidocaine. Immediately after infiltration a whitish maggot appeared from each nodule and could be easily extracted with a forceps. The maggots were identified as Dermatobia hominis larvae. After extraction a local antiseptic dressing was applied and the wounds healed without complications. Dermatobia hominis is a common cause of myiasis in Central- and South-America and should be taken into account in furuncular skin eruptions of returning travelers. The typical appearance of the skin eruption with a central porus, seropurulent discharge and a whitish, tender moving mass within the nodule is quite characteristic for myiasis. The patients often have [corrected] intermittent sharp pain in the area of the affected skin and report continuing growth of the nodules and a sensation of slight movement within the skin eruption. Extraction is accomplished with a forceps after lidocaine infiltration, alternatively an occlusive dressing could be applied by means of which the larvae can be removed easily from the cavity."}, {"id": "pubmed23n0516_14277", "title": "Kerion: an unusual presentation in the otolaryngology department.", "score": 0.00980392156862745, "content": "A 19-year-old farmer was referred by his general practitioner as an emergency to our otolaryngology department complaining of marked breathlessness of a few hours duration. He gave a three-day history of painful swelling and hair loss in the beard area of the right side of the neck. His upper airway was compromised unless extension of the neck was maintained. Larynx and pharynx were normal. The acute symptoms settled with intravenous antibiotics and hydrocortisone. Culture of skin scrapings revealed a growth of Tricophyton verrucosum. The neck swelling subsided after a course of oral griseofulvin followed by terbinafine. Difficulty in breathing due to fungal infection of the neck has not been previously reported in the English literature."}, {"id": "pubmed23n0988_10840", "title": "Successful Treatment with Fusidic Acid in a Patient with Folliculitis Decalvans.", "score": 0.009708737864077669, "content": "Dear Editor, Folliculitis decalvans (FD) is a rare form of primary neutrophilic cicatricial alopecia. It is a highly distressing disease that affects young and middle-aged adults, with a slight male predominance (1). The most frequent clinical manifestations are follicular pustules and diffuse and perifollicular erythema that heal with centrifugal scarring. Follicular tufting, erosions, and hemorrhagic crusts can also be present, and this alopecia is most often located at the vertex and occipital area. Patients frequently complain about pain, itching, or burning sensations, and the involvement of other body areas is rare (2). The pathogenesis of this disease remains unclear. Staphylococcus aureus and other hair follicle bacteria can often be isolated from the pustules, suggesting the role of a bacterial infection in its etiology. A defect in the host's immune response can also be postulated by reports of familial cases and the appearance of FD in patients with immunity dysfunctions. Other mechanical factors have been suggested, such as structural abnormalities of the follicle or local inflammation (2). Management of this alopecia is difficult and its course is typically chronic and relapsing. The treatment aim is to stop inflammation and further irreversible destruction of hair follicles. Antibiotics remain the first-line therapy, due both to their anti-inflammatory and antimicrobial properties (1). Although topical fusidic acid is widely used as adjuvant treatment, there are few data regarding its oral use. We report a case of folliculitis decalvans successfully treated with oral fusidic acid. Our patient was a 41-year old Cape Verdean woman with a two month history of alopecia with painful, purulent discharge at the vertex of the scalp. The patient was diagnosed with human immunodeficiency virus type 1 (HIV-1) infection 5 years prior and was stable on her regimen of efavirenz, tenofovir, and emtricitabine, with undetectable viral load. She denied application of topical or capillary products. Dermatological examination revealed a patch of cicatricial alopecia with crusts and follicular pustules (Figure 1). Direct microscopic examination and mycological culture showed no fungal element. A diagnosis of folliculitis decalvans was established and the patient was started on oral fusidic acid at a dose of 500 mg three times a day. Betamethasone dipropionate 0.05% and salicylic acid 3% lotion as well as azelaic acid 5% lotion were also applied to the affected area once daily. After two months of treatment, the patient showed clinical improvement, with less erythema and suppuration of the affected scalp. A partial hair regrowth was noted, mainly at the periphery. Subsequently the patient maintained only topical therapy, and no recurrences were observed after 6-months of follow-up. Fusidic acid is useful in the treatment of skin and soft tissue infections, particularly those due to S. aureus, as shown by randomized controlled studies (3). The clinical efficacy of fusidic acid in the treatment of folliculitis decalvans has been reported previously. Bogg was the first to describe this useful effect (4). Sutter also reported good results with fusidic acid used both topically and orally (500 mg three times a day) (5). However, both failed to report the treatment duration or the outcome on discontinuation. Abeck described three patients that responded to a three week oral course of fusidic acid (500 mg three times a day) and to a maintenance treatment with zinc sulfate (4). During the following year, recurrence was observed in only one patient after ending zinc sulfate therapy. Oral antibiotics are frequently used to treat folliculitis decalvans. Tetracyclines and the combination of clindamycin with rifampicin are the most commonly used (2). However, the disease usually progresses when treatment is stopped. Fusidic acid is an anti-staphylococcal drug with few adverse effects. It is highly bioavailable orally, and has a long plasma half-life. Despite years of clinical use in numerous countries, resistance rates remain at low levels to date (6). Since clinical series or cases including ours have shown good results, this drug should not be forgotten when considering treatment options for folliculitis decalvans."}, {"id": "pubmed23n1089_1711", "title": "Kerion celsi due to <i>Microsporum audouinii</i>: a severe form in an immunocompetent girl.", "score": 0.009708737864077669, "content": "A 9-year-old girl presented a large inflammatory cup-shaped scalp lesion with alopecia surrounded by pustules, dander, and suppuration associated with an occipital inflammatory lymphadenopathy for 1 month. Wood's light exam was positive as well as KOH mount showing ectothrix type hair involvement. Hair and pus culture on Sabouraud dextrose agar (SDA) added with chloramphenicol and supplemented with cycloheximide isolated a dermatophyte species identified as <iMicrosporum audouinii</i according to the colonies features. Species identification was confirmed by matrix-assisted laser desorption-ionization-time of flight mass spectrometry (MALDI-TOF MS) and the patient was treated for kerion celsi with terbinafine tablets 125 mg per day associated with a ketoconazole-based shampoo. The evolution was favorable, with hair regrowth after 2 months."}, {"id": "pubmed23n1025_2382", "title": "Scalp eschar and neck lymphadenopathy after tick bite (SENLAT) caused by Bartonella henselae in Korea: a case report.", "score": 0.009615384615384616, "content": "Tick-borne lymphadenopathy (TIBOLA) is an infectious disease, mainly caused by species from the spotted fever group rickettsiae and is characterized by enlarged lymph nodes following a tick bite. Among cases of TIBOLA, a case of scalp eschar and neck lymphadenopathy after tick bite (SENLAT) is diagnosed when an eschar is present on the scalp, accompanied by peripheral lymphadenopathy (LAP). Only a few cases of SENLAT caused by Bartonella henselae have been reported. A 58-year-old male sought medical advice while suffering from high fever and diarrhea. Three weeks before the visit, he had been hunting a water deer, and upon bringing the deer home discovered a tick on his scalp area. Symptoms occurred one week after hunting, and a lump was palpated on the right neck area 6 days after the onset of symptoms. Physical examination upon presentation confirmed an eschar-like lesion on the right scalp area, and cervical palpation revealed that the lymph nodes on the right side were non-painful and enlarged at 2.5 × 1.5 cm. Fine needle aspiration of the enlarged lymph nodes was performed, and results of nested PCR for the Bartonella internal transcribed spacer (ITS) confirmed B. henselae as the causative agent. With an isolated case of SENLAT and a confirmation of B. henselae in Korea, it is pertinent to raise awareness to physicians in other Asian countries that B. henselae could be a causative agent for SENLAT."}, {"id": "pubmed23n0542_25001", "title": "It's on the tip of my tongue.", "score": 0.009615384615384616, "content": "A 48-year-old white woman was admitted to the hospital with low-grade fever, night sweats, fatigue, nonproductive cough with dyspnea, bilateral knee pain, and swelling that progressed slowly over 6 weeks. She was a 30-pack-year smoker, and had received outpatient antibiotic therapy with clarithromycin and then cephalexin without improvement. The admission chest radiograph showed bilateral interstitial infiltrates, and an effusion was seen on knee radiographs. She was treated with levofloxacin, cefepime, and methylprednisolone with some improvement, but fevers persisted up to 104 degrees F/40 degrees C. She also developed multiple painful skin nodules (Figure 1) and an enlarging painful tongue ulcer (Figure 2). Her bilateral knee swelling and pain also worsened, and a bone scan showed increased activity. Skin biopsy showed acute and chronic inflammation with an abscess that contained \"yeast\" (Figure 3). Fungal culture from the skin lesion and joint fluid aspirate grew Blastomyces dermatitidis. Urine antigen and blood antigen enzyme-linked immunoassays for B. dermatitidis were positive. The patient was started on a 6-month course of itraconazole oral solution with slow resolution of her joint inflammation and skin lesions over the next several weeks."}, {"id": "pubmed23n0766_1069", "title": "Microsporum canis infection in three familial cases with tinea capitis and tinea corporis.", "score": 0.009523809523809525, "content": "We report a familial infection caused by Microsporum canis. The first two patients were a 30-year-old female and her son, a 5-year-old boy, who came in contact with a pet dog at a farm house. The boy then suffered from hair loss for 3 months. There were circular and patchy alopecia with diffuse scaling on his scalp. Meanwhile, his mother also developed patchy erythema and scaling on her face. Several weeks later, the boy's sister, a 4-year-old girl, was noted to have inconspicuous scaly plaques in the center of her scalp. The development of tinea capitis in the two children and tinea corporis in their mother were diagnosed based on the positive KOH examination. Morphologic characteristics and sequencing of the internal transcribed spacers 1 and 2, amplified from primary culture isolates, confirmed that their infections were caused by the zoophilic M. canis. Repetitive sequence-based molecular typing using the DiversiLab system secreted enzymatic activity analysis, and antifungal susceptibility indicated that these isolates might share the same source. The boy and girl were cured by the treatment with oral itraconazole and topical naftifine-ketoconazole cream after washing the hair with 2 % ketoconazole shampoo, and their mother was successfully treated by terbinafine orally in combination with topical application of naftifine-ketoconazole cream. "}, {"id": "pubmed23n0592_12882", "title": "Comparison of hairbrush, toothbrush and cotton swab methods for diagnosing asymptomatic dermatophyte scalp carriage.", "score": 0.009523809523809525, "content": "Tinea capitis may also present as a minimal infection, termed carrier state. Anthropophilic dermatophytes (i.e. Trichophyton tonsurans and Trichophyton violaceum) have been generally associated with high rates of asymptomatic carriage. The aim of this study was to compare the efficacy of the hairbrush, toothbrush and cotton swab methods for diagnosing scalp carriage as well as to determine the prevalence and related dermatophyte species for both asymptomatic and symptomatic tinea capitis in Adana Province, Turkey. A screening study was carried out between February 2006 and May 2006, covering three schools and a total of 1560 children with 857 (54.9%) boys and 703 (45.1%) girls, aged between 7 and 17 years (10.6 +/- 2.3 years). The diagnosis was made by using three of the methods mentioned above with inoculation onto Sabouraud glucose agar. Symptomatic tinea capitis was not detected in the study; however, 21 (1.3%) asymptomatic carriers, with 9 (42.9%) boys and 12 (57.1%) girls, aged 7 to 13 years (9.7 +/- 1.9 years) were detected. The diagnosis was made via hairbrush in 13, via cotton swab in 4 and via toothbrush in 4. The mean age (P = 0.075) and gender differences were found to be statistically insignificant (P = 0.26). The most common isolated species was Trichophyton mentagrophytes var. mentagrophytes (90.4%) followed by Trichophyton audouinii (4.8%) and Microsporum gypseum (4.8%). Nine children had Arab origin (P = 0.005), and 12 had immigrated from the south-eastern region of Anatolia, Turkey. The screening of 32 households of 21 children with asymptomatic carriage enabled the researchers to detect the carrier state in three mothers and one sister, resulting in a total of four households (12.5%), with T. mentagrophytes var. mentagrophytes isolated, by hairbrush method in three cases and cotton swab in one case. If the methods were to be used alone, the prevalence of asymptomatic carriage would be found as 1.0% (16 of 1592) in the hairbrush, 0.3% (4 of 1592) in the toothbrush and 0.3% (5 of 1592) in the cotton swab methods; whereas the combined use of these three methods could reveal a total prevalence of 1.6% (25 of 1592). The hairbrush method was significantly found to be more effective in detecting dermatophyte fungi than the toothbrush (P &lt; 0.01) and the cotton swab methods (P &lt; 0.05). There was also a statistically significant difference between the use of a single method and the combination of all other three methods (P &lt; 0.005). In summary, it was found that the prevalence of asymptomatic carriage did not cover symptomatic tinea capitis prevalence (1.6% vs. 0%), and the dominant species was zoophilic T. mentagrophytes (92%, 23 of 25). Asymptomatic carriage was not found to be related to age, gender and the coexistence of other dermatophytoses; however, race (Arab origin) was found to be the only risk factor. For laboratory diagnosis, no method was found to be nominated as a gold standard; hence, a combined use of diagnosing methods was suggested."}, {"id": "pubmed23n0662_21111", "title": "Inflammatory tinea capitis: non-healing plaque on the occiput of a 4-year-old child.", "score": 0.009433962264150943, "content": "Inflammatory tinea capitis is an uncommon condition in Singapore. In this case report we present a patient whom we managed for this condition. A 4-year-old girl presented to us with multiple pustules over the occipital scalp for 6 weeks, associated with painful cervical lymphadenopathy. Her condition did not respond to topical and oral antibiotics. The patient was diagnosed with kerion (inflammatory tinea capitis) and fungal culture of plucked hairs from the kerion grew Microsporum species of dermatophyte. She was treated with a course of oral griseofulvin and topical selenium sulfide shampoo. She was advised to bring her pet cats to the veterinarian for screening, as well as not to share combs with her other siblings. Her condition improved with the antifungal therapy, and there was no residual alopecia. Physicians should consider tinea capitis when they encounter a patient with scalp folliculitis or scarring alopecia in the appropriate clinical context."}, {"id": "pubmed23n0318_5641", "title": "[Dermatophytes isolated in our clinics. 5-year-study in Zaragoza].", "score": 0.009433962264150943, "content": "This review summarizes the different species of dermatophytes isolates in our laboratory between 1991 and 1995. We describe the clinical forms and establish the distribution over this period of time. Retrospective survey of samples from outpatients of the Dermatology Service in Miguel Servet Hospital where mycologic cultures are required. The extraction of samples is made by scrapes with a carpet or scalpel and they are cultured on Saboureaud agar with chloramphenicol and dermatophytes agar for 3 weeks. All plates were incubated at 28 degrees C. The identification of isolated strains is made by means of morphologic and physiologic criteria; the doubtful strains were identified in national referral center of Majadahonda CNMVISS. 4004 samples were analyzed from 3934 patients and 543 strains of dermatophytes were isolated. The frequencies were as follow: Microsporum canis (44%), Trichophyton mentagrophytes (31.4%), Trichophyton rubrum (18.6%), Epidermophyton floccosum (2.6%), Microsporum gypseum (1.4%), Trichophyton tonsurans (0.7%), Trichophyton verrucosum (0.7%), Trichophyton violaceum (0.2%) y Microsporum audouinii (0.2%). The most frequently observed dermatophytoses were Tinea corporis (54.8%), followed by Tinea unguium (12.6%), Tinea capitis (12.5%), Tinea pedis (8.3%), Tinea manuum (6.3%), Tinea cruris (4.7%) and Tinea barbae (0.7%). The zoophylic species are the most prevalent in our area and we have observed a raise of Microsporum canis in recent years. It is important to perform mycologic survey in every suspected lesion in older to determinate the true incidence of human dermatophytoses."}, {"id": "InternalMed_Harrison_4055", "title": "InternalMed_Harrison", "score": 0.009345998471448848, "content": "Oral griseofulvin or terbinafine plus 2.5% selenium sulfide or ketoconazole shampoo; examine family members Discontinuation of offending hair style or chemical treatments; diagnosis of trichotillomania may require observation of shaved hairs (for growth) or biopsy, possibly followed by psychotherapy aTo date, Food and Drug Administration–approved for men. bScarring alopecia can occur at sites of kerions. cMay also be scarring, especially late-stage traction alopecia. CAuSES of figuRATE SKin LESionS I. Primary cutaneous disorders A. Tinea B. Urticaria (primary in ≥90% of patients) C. Granuloma annulare D. Erythema annulare centrifugum E. Psoriasis II. A. 1. Erythema migrans (CDC case definition is ≥5 cm in diameter) 2. Urticaria (≤10% of patients) 3. 4. 5. 6. B. Nonmigratory 1. 2. 3. 4. Cutaneous T cell lymphoma (especially mycosis fungoides) aMigratory erythema with erosions; favors lower extremities and girdle area."}, {"id": "pubmed23n0689_13491", "title": "Cutaneous and subcutaneous phaeohyphomycosis.", "score": 0.009345794392523364, "content": "Case 1: A 17-year-old male rural worker from Bolivia living in La Plata (Argentina) for the past year had a lesion on the flexor side of his right forearm (6 x 4 cm). The lesion was formed by several confluent nodular areas, wine-red in color, some fistulized, with hemopurulent drainage. The area was hot and painless (Figure 1). On physical examination, no regional adenomegalies were reported. The following analyses were requested and results reported. Soft tissue ultrasound: material of solid consistency with layered liquid areas, located in the subcutaneous cellular tissue, with fistulous tract, connecting through superficial planes. Evidence of peripheral edema. Bacteriologic analysis (puncture aspiration): methicillin-sensitive Staphylococcus aureus. Mycologic analysis (puncture aspiration): negative; laboratory results: eosinophilia; and human immunodeficiency virus: nonreactive. Histopathologic examination: lesions of necrosis with granulomatous inflammatory reaction. Fungi techniques (periodic acid-Schiff, Grocott stains): negative. Bacilos acid-alcohol resistentes (acid-alcohol resistant bacillus) (BAAR) techniques (Kinyoun, Ziehl-Neelsen): negative. Foreign body examination tested with polarized light: negative. Mycologic and bacteriologic examinations were repeated, including a search for mycobacterium species using material obtained from the biopsy performed on the cutaneous lesion. Macromorphology: the colony was initially black and of creamy consistency, to later become velvety. Micromorphology: dark blastoconidia, then cylindrical phialides with elliptical conidia (Figure 2). Exophiala dermatitidis infection. On the basis of these characteristics, the diagnosis is phaeohyphomycosis due to Edermatitidis. The patient is treated with antimycotic therapy, with oral itraconazole (400 mg/d), plus indication of surgical procedure to remove the lesion. The patient's condition evolves favorably with no recidivant episodes after the sixth month post-treatment (Figure 3). During the first year, controls were scheduled every 2 months. Case 2: A 72-year-old diabetic man had a painful chronic varicose ulcer on the side of his left foot, with black friable exudate, 2x3 cm in diameter after 1 year. Every time the black material was removed, it would quickly grow back again. No response was obtained with different therapies applied to seal the lesion (Figure 4). Routine laboratory results included the following. Glucemy: 1.82 g/dL. Histopathology: filamentous septate fungal elements with positive Grocott stain (Figure 5 and Figure 6). Mycologic examination and culture: direct: fungal elements in dematiaceous group. Culture: positive for Curvularia lunata (Figure 7). The treatment selected was oral itraconazole (400 mg/d) for 12 months, with periodic laboratory controls, plus application of wet pads on the ulcer containing sodium borate and ketoconazole cream. At the fourth month, the ulcer had completely closed (Figure 8)."}, {"id": "pubmed23n0222_8046", "title": "[Torsion of appendix of testis and epididymis: a report of 4 cases].", "score": 0.009259259259259259, "content": "We report our clinical and pathological observations on four patients with torsion of appendix testis or epididymis, and reviewed 72 cases of torsion of the appendages of intrascrotal organs collected from the Japanese literature; 35 cases of torsion of appendix testis, 36 cases of torsion of appendix epididymis and 1 case of torsion of paradidymis . Case 1 was a 10-year-old boy visiting us because of pain and swelling in his right scrotum continuing for the past ten days. His right scrotum was found to contain a hen-egg sized tender mass. The testis and epididymis could not be differentiated by palpation. The blood count disclosed 10,000 white blood cells. At operation, two appendix epididymis were found in his right scrotum. One of them was twisted 360 degrees clockwise and 10 x 8 x 5 mm in size. Case 2 was a 11-year-old boy with complaint of pain in his left lower abdomen and left scrotum for the past three days. Palpation of his left scrotal contents revealed a slightly hard testis and tender epididymis. At operation, we found his left spermatic cord to be twisted 90 degrees counterclockwise and appendix epididymis 180 degrees counterclockwise. The twisted appendix epididymis was 10 x 8 x 7 mm in size. Case 3 was a 13-year-old boy whose complaint was left scrotal pain. The upper pole of his left testis was found to be swollen and tender by palpation. The exposure of his left scrotum at operation revealed that his left epididymis was abnormally attached to his left testis and had two appendices. One of these appendix epididymis was twisted 180 degrees clockwise and measured 10 x 10 x 8 mm. Case 4 was a 19-year-old male who suffered from right testicular pain for the past seven days. He had a history of three intermittent episodes of similar right testicular pain during the past two years. His right testis was enlarged and palpated slightly hard and tender. We explored his right scrotum surgically and found the appendix testis twisted and enlarged to little-finger's head size. However, it was impossible to determine whether the rotation was clockwise or counterclockwise because the twisted appendix was too severely damaged. The preoperative diagnosis was correct in one case and three were erroneously diagnosed as having torsion of spermatic cord. All four cases were treated by surgery which relieved all patients of discomfort."}, {"id": "pubmed23n0482_23041", "title": "[Screening Examination and Management of Dermatophytosis by Trichophyton tonsurans in the Judo Club of a University].", "score": 0.009259259259259259, "content": "Thirty-one members of the Judo Club of a certain university (age: 18~23) underwent a screening examination for dermatophytosis by Trichophyton tonsurans. Test items were: age, sex, height, weight, living mode, exercise duration, number of judo contestants, presence of foreign contestants, occurrence, if any, of dermatophytosis past or present according to a subject's answers to a questionnaire, medical examinations and mycological examinations (KOH, cellophane tape culture, and hairbrush culture). Twenty-four subjects (77%) replied that they had suffered from dermatophytosis in the past, and 8 subjects (26%) had had head eruption in the past. Eleven subjects (35%) had suspicious dermatophytosis at the time of screening; 3 of them were found positive by direct microscopy, 2 of them were positive by cellophane tape culture. Eleven subjects (35%) were found positive by the hairbrush culture, but only 2 had eruption-like folliculitis. The remaining 9 subjects were free from clinical symptoms and were judged to be asymptomatic carriers. As countermeasures, we recommended cleaning and the use of shampoo containing miconazole nitrate. Subjects with suspicious tinea corporis were treated with antimycotic ointment. The 7 subjects who showed more than 5 colonies by the hairbrush culture were treated with 1-week pulse therapy of 400 mg itraconazole, and 3 of these who took a total dose of a pulse became negative through one pulse therapy."}, {"id": "pubmed23n0958_20665", "title": "An Excellent Response to Tofacitinib in a Pediatric Alopecia Patient: A Case Report and Review.", "score": 0.009174311926605505, "content": "KD is an 8 year-old male patient who presented to our clinic in December 2016 with a history of patchy hair loss for many months duration that was worsening. KD's past medical history was notable for atopic dermatitis, and a positive family history of autoimmune thyroid disease. Upon examination he had well circumscribed areas of hair loss throughout his scalp, with exclamation mark hairs seen on dermoscopy. Eyebrows and eyelashes were intact, no epidermal changes of scale or erythema were noted on the scalp and no palpable lymph nodes were present. He was diagnosed with alopecia areata at this time and was treated with Clobetasol 0.05% solution QHS as well as Kenalog 2.5 mg/ml injections to the areas of hair loss. Patient followed up two months later with worsening of his alopecia at a rapid pace, presenting now with hair loss of the entire scalp and loss of the eyebrows. He was diagnosed with progression to alopecia universalis at that time, with a corresponding SALT (Severity of Alopecia Tool) score of 100. Both KD and his mother stated the hair loss was causing much distress in the patient's life both at school and at home. After a thorough discussion of treatment alternatives to include continued topical high dose steroids, intralesional injections, high dose oral methylprednisolone, topical irritation with anthralin, topical immunotherapy with diphenylcyclopropenone (DPCP) or squaric acid dibutylester (SADBE) and systemic immunosuppressives, both the mother, patient and clinician agreed to try tofacitinib 5 mg twice daily with continued usage of topical steroids. Patient and his family was counseled about support groups, and local meetings to ease the mental distress associated with this condition. After baseline labs were obtained and reported within normal limits, to include CBC, CMP, thyroid studies, lipids and Quanitferon gold, KD was started on tofacitinib 5 mg BID. Labs were repeated one month later, and 3 months ongoing thereafter. At KD's 3 month follow up, after starting tofacitinib 5 mg twice daily, KD showed complete regrowth of the eyebrows with minimal hair growth of the posterior occiput (Figure 1 a-d). At KD's 6 month follow up he had 100% regrowth of eyebrows and complete scalp regrowth, resulting in a SALT score of 0 (Figure 2). KD reported no side effects until month 6, after full hair regrowth, when patient started to report mild headaches. Drug holiday was offered but the patients family chose to discontinue treatment at this time as they were concerned side effects were secondary to medication usage. Unfortunately, patient was lost to follow-up after the discontinuation of treatment. From previous case reports we can postulate that his alopecia returned to baseline after discontinuation of tofacitinib. KD had an incredible response to treatment, as has been reported previously in literature of adolescents using these novel therapies. This is the youngest patient ever reported to be successfully treated with oral tofacitinib 5 mg twice daily for alopecia and its variants. J Drugs Dermatol. 2018;17(8):914-917."}, {"id": "pubmed23n0211_15133", "title": "[Microsporic tinea of the scalp in adults].", "score": 0.009174311926605505, "content": "Five cases of \"tinea capitis\" by Microsporum canis in adult were studied. Cellular immunological tests were performed on four of them and the results were according to clinical forms. The hormonal studies in these patients were normal to their ages. Three of them were old women. Two cases were atypical difficult to diagnose. For this reason the authors suggest to perform mycological studies in every scalp affections were it is impossible to do a clinical and evolutive positive diagnosis. All the patients healed with local and systemie antimycotical treatment."}, {"id": "pubmed23n0673_18558", "title": "Hair transplantation for therapy-resistant alopecia areata of the eyebrows: is it the right choice?", "score": 0.00909090909090909, "content": "Alopecia areata is a common skin disorder of presumed autoimmune etiology and it usually shows an unpredictable course. Treatment of alopecia areata is challenging. There is very little information on the use of surgical therapies for the treatment of alopecia areata in the medical published work. A 24-year-old male patient was referred to a private hair transplantation clinic owned by one of the authors for the treatment of therapy-resistant alopecia areata affecting both eyebrows. He had quickly lost all body hair 4 years prior beginning from the scalp. He received psoralen and ultraviolet A (PUVA) therapy for alopecia universalis and all body hair re-grew except his eyebrows. Alopecia areata was stable for the 18 months following the last medical treatment he received. Because there was no response to various medical therapeutic agents, we decided to transplant occipital hairs to the eyebrow area. After the patient understood and accepted all risks, occipital hairs were transplanted to the eyebrows by using the follicular unit extraction technique. Postoperatively, the patient did not receive any topical or systemic therapies for alopecia areata. Although 40% hair re-growth was detected in his eyebrows at 1 year postoperation, this rate was 80% by 2 years postoperation. However, there was resistance to re-growth in the medial eyebrow regions. New eyebrows grew as occipital hairs and required trimming. His satisfaction from the surgical procedure was 90% at the end of the 24th postoperative month. Surgical treatment of diseases like alopecia areata is still controversial. Our case report offers an additional contribution to the published work on the surgical methods used in the treatment of stable alopecia areata."}, {"id": "pubmed23n0562_4156", "title": "Asymptomatic dermatophyte scalp carriage in school children in Adana, Turkey.", "score": 0.00909090909090909, "content": "The aim of this study was to determine the prevalence of asymptomatic dermatophyte scalp carriage and symptomatic tinea capitis in Adana Province, Cukurova region, Turkey. For this purpose, a screening study was performed in five schools, between January 2004 and May 2005, covering a total of 5143 children with 2740 (53.3%) boys and 2403 (46.7%) girls, aged 7-14 years (9.6 +/- 2.0). The diagnosis was made using the cotton swab method with inoculation onto Sabouraud glucose agar amended with cycloheximide, chloramphenicol and gentamicin. Among 10 (0.2%) cases, six asymptomatic carriers (mean age 10.7 +/- 2.3) and four symptomatic cases (mean age 8.3 +/- 0.5) were detected, all of whom were boys and had immigrated from the south-eastern and eastern region of Anatolia, Turkey. The mean age differences were found to be statistically significant (Mann-Whitney U=3.000, P=0.046). Boys were found to be more prone to asymptomatic carriage (P=0.033), but not tinea capitis (P&gt;0.05). Zoophilic dermatophytes, namely Microsporum canis (40%) and Trichophyton mentagrophytes var. mentagrophytes (40%) were the most commonly isolated species, followed by anthropophilic Trichophyton tonsurans (10%), while no causative agent was detected in a case (10%) with tinea capitis superficialis. Scalp cultures were found to be dermatophyte-negative after 3- to 8-month follow-up in cases with asymptomatic carriage. As a conclusion, the prevalence of asymptomatic carrier state was similar with the prevalence of symptomatic cases, and we found a predominance of zoophilic species."}, {"id": "pubmed23n0899_12817", "title": "The Diagnosis and Treatment of Multiple Factitious Oral Ulcers in a 6-Year-Old Boy.", "score": 0.009009009009009009, "content": "Factitious ulcers are characterized by self-inflicted lesions with multifactorial origin. These lesions are frequently found in head, neck, and hands. This report shows a 6-year-old boy diagnosed with factitious oral ulcers that occurred after the self-biting of buccal vestibule and nail-scratching of gingival tissue. Clinically, a significant swelling was observed, hard on palpation, located at the right lower third of the face, next to the posterior area of the mandible. In the intraoral examination, ulcers at different healing stages were noted on the swelling area. During the anamnesis, the father reported a change in his familial structure that triggers psychological stress, providing the clues to the presumptive diagnosis of factitious oral ulcers. We prescribed the topical use of Gingilone® three times a day to control the local pain and inflammation. At 7-day follow-up, we noticed the reduction of extraoral swelling and the initial healing of the ulcers. The presumptive diagnosis was confirmed at 30-day follow-up, with the lasting remission of oral lesions. The treatments of factitious oral ulcers should be individually tailored for each patient, focused on a multidisciplinary approach, including psychotherapy and periodic clinical control. To the best of our knowledge, gaps of evidence lead to the lack of standardized clinical protocols on this issue."}, {"id": "pubmed23n0596_14190", "title": "In general practice, 'always expect the unexpected'.", "score": 0.009009009009009009, "content": "Mr SF, aged 72 years, presented to a senior colleague complaining of a scalp sore which was failing to heal. The patient had injured his head while mustering cattle 4 years earlier. He consulted his local medical officer at that time and was reassured and sent on his way. Six weeks before presenting to our practice, Mr SF had split his head open again. Although he was not overly concerned about it at the time, it had been slow to heal and he had consulted a naturopath. The naturopath was packing the scalp sore with comfrey leaves and had advised Mr SF to eat curry to aid with its healing. He had been seeing this alternative practitioner each week for the preceding 6 weeks. Mr SF had become disillusioned with the poor results he was getting. At the behest of his wife he was seeking another opinion."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[218, 328]], "word_ranges": [[37, 59]], "text": "the first cause to think about is giant cell arteritis as the cause of NOIA, so the correct option would be 2."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[329, 470]], "word_ranges": [[59, 80]], "text": "Option 4 would be considered if we were told of an AINO but with non-arteritic characteristics (without all the accompanying symptomatology)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "We are presented with a case of monocular amaurosis with a vascular profile, also in an older woman with a history of weight loss and what seems to be symptoms of mandibular claudication and polymyalgia rheumatica, so the first cause to think about is giant cell arteritis as the cause of NOIA, so the correct option would be 2. Option 4 would be considered if we were told of an AINO but with non-arteritic characteristics (without all the accompanying symptomatology).", "full_answer_no_ref": "We are presented with a case of monocular amaurosis with a vascular profile, also in an older woman with a history of weight loss and what seems to be symptoms of mandibular claudication and polymyalgia rheumatica, [HIDDEN]. Option 4 would be considered if we were told of an AINO but with non-arteritic characteristics (without all the accompanying symptomatology).", "full_question": "A 70-year-old woman with a history of anorexia, weight loss, discomfort in the muscles and proximal joints and pain in the temporomandibular region who comes to the emergency department for unilateral loss of vision (hand movement), sudden and painless onset (afferent pupillary defect).what test would you request first for diagnostic purposes?", "id": 381, "lang": "en", "options": {"1": "Lumbar puncture.", "2": "C Reactive Protein.", "3": "Magnetic resonance angiography.", "4": "Carotid ultrasound.", "5": NaN}, "question_id_specific": 139, "type": "NEUROLOGY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "InternalMed_Harrison_1431", "title": "InternalMed_Harrison", "score": 0.011765941800609307, "content": "A careful neurologic examination is an essential first step in the evaluation. In most cases, patients with an abnormal examination or a history of recent-onset headache should be evaluated by a computed tomography (CT) or magnetic resonance imaging (MRI) study. As an initial screening procedure for intracranial pathology in this setting, CT and MRI methods appear to be equally sensitive. In some circumstances, a lumbar puncture (LP) is also required, unless a benign etiology can be otherwise established. A general evaluation of acute headache might include cranial arteries by palpation; cervical spine by Pain induced by bending, lifting, cough Pain associated with local tenderness, e.g., region of temporal artery the effect of passive movement of the head and by imaging; the investigation of cardiovascular and renal status by blood pressure monitoring and urine examination; and eyes by funduscopy, intraocular pressure measurement, and refraction."}, {"id": "wiki20220301en003_97415", "title": "Headache", "score": 0.010546789392943238, "content": "One recommended diagnostic approach is as follows. If any urgent red flags are present such as visual loss, new seizures, new weakness, new confusion, further workup with imaging and possibly a lumbar puncture should be done (see red flags section for more details). If the headache is sudden onset (thunderclap headache), a computed tomography test to look for a brain bleed (subarachnoid hemorrhage) should be done. If the CT scan does not show a bleed, a lumbar puncture should be done to look for blood in the CSF, as the CT scan can be falsely negative and subarachnoid hemorrhages can be fatal. If there are signs of infection such as fever, rash, or stiff neck, a lumbar puncture to look for meningitis should be considered. If there is jaw claudication and scalp tenderness in an older person, a temporal artery biopsy to look for temporal arteritis should be performed and immediate treatment should be started. Neuroimaging"}, {"id": "pubmed23n1105_22827", "title": "Sudden-onset unilateral painless vision loss.", "score": 0.010225642363375505, "content": "A 75-year-old Caucasian woman presented with sudden-onset multifocal scotomas in her right eye's central vision for 1 day. There were subtle white intraretinal foveal lesions that correlated with patchy inner retinal hyperreflectivity on optical coherence tomography, suggestive of paracentral acute middle maculopathy. Initial cerebrovascular work-up was negative. Review of systems was positive for lethargy and jaw claudication. The sedimentation rate and c-reactive protein were elevated, but platelet count was normal. The patient was started on 60 mg oral prednisone daily and underwent bilateral temporal artery that confirmed the diagnosis of giant cell arteritis."}, {"id": "pubmed23n0810_19941", "title": "Atypical retinal vaso-occlusion with structural and functional resolution.", "score": 0.009900990099009901, "content": "The purpose is to report a patient with primary open-angle glaucoma that developed sudden painless unilateral vision loss, a sequential ophthalmoscopic appearance with features of both central retinal artery and later central retinal vein occlusion, and objective visual system dysfunction in the form of a relative afferent pupil defect, who spontaneously recovered vision along with complete resolution of the pupillary defect over several weeks. A 50-year-old woman with a long-standing history of glaucoma presented with acute, painless vision loss in one eye, a pallid retina with a cherry red macula, diffuse retinal hemorrhages, and a relative afferent pupil defect. Spectral domain optical coherence tomography and fluorescein angiography were essentially normal with neither retinal edema nor retinal ischemia to account for the visual dysfunction. Over the course of 2 months, the patient regained vision and the relative afferent pupil defect, typically a permanent manifestation of retinal destruction, resolved. Not all retinal vaso-occlusive phenomena can be completely attributed to a central retinal vein or artery occlusion. In the patient presented, there was no objective diagnostic testing that revealed a cause for the patient's vision loss or relative afferent pupillary defect. This combined with the complete recovery of vision and resolution of the relative afferent pupillary defect underscores a lack of comprehensive understanding of retinal vaso-occlusive disease."}, {"id": "pubmed23n0574_18362", "title": "[Temporal arteritis presenting with headache and abducens nerve palsy. Report of a case].", "score": 0.009900990099009901, "content": "A 71-year-old man visited our clinic with a 3-day history of severe throbbing headache and 1-day history of horizontal diplopia. He had had jaw claudication and pain in the neck and shoulder several days previously. His right eye was slightly esotropic and did not move laterally. There was no blepharoptosis, proptosis, lid edema, or conjunctival injection. The pupils were unremarkable. The remainder of the cranial nerve functions was intact. There was no limb weakness or sensory impairment. Superficial temporal arteries were swollen and tender on both sides. Laboratory examination showed elevated CRP level and high erythrocyte sedimentation rate. Cranial MR images were unremarkable. The cerebrospinal fluid was acellular with 45 mg/dl of protein. A diagnosis of temporal arteritis was made. Treatment with 50 mg of prednisolone brought about prompt disappearance of the headache. Right ocular movement fully recovered in 10 days. Temporal artery biopsy findings and response to corticosteroid were consistent with temporal arteritis. The motility pattern of the right eye was consistent with complete abducens nerve palsy, which is a rare manifestation of temporal arteritis. Although temporal arteritis is a rare cause of ophthalmoplegia in the elderly patients, swift diagnosis and treatment is necessary to avoid blindness."}, {"id": "pubmed23n1121_13718", "title": "Atypical Leber hereditary optic neuropathy with a 34-year interval between vision loss in both eyes.", "score": 0.00980392156862745, "content": "Leber hereditary optic neuropathy (LHON) is an inherited mitochondrial disease characterized by painless vision loss affecting both eyes. The disease usually develops in both eyes within weeks to months of onset. We report a case of LHON who presented with unilateral vision loss in childhood with an interval of more than 30 years between vision loss in the two eyes. A 43-year-old man presented with a 1-month history of vision loss in his right eye. At 9 years of age, his visual acuity in the left eye declined, and he had been treated with glaucoma eyedrops bilaterally at his eye clinic. At his first visit to our hospital, his BCVA was 0.15 in the right eye and 0.1 in the left eye, and critical flicker frequency was 16 Hz in the right eye and 15 Hz in the left eye, and he was negative for a relative afferent pupillary defect. The Goldman visual field showed central scotoma in both eyes. Fundus examination revealed slight redness of the right optic disc with meandering retinal small vessels, and the left optic disc had a slight pallor. Fluorescein angiography could not be performed because of liver dysfunction. OCT showed prominent bilateral thinning of the RNFL and retinal ganglion cell layer. Enhancement of the optic nerve was not apparent on orbital gadolinium-enhanced magnetic resonance imaging. Hematologic analysis revealed macrocytic anemia and low levels of vitamin B12 and folate. His mother had a presumptive diagnosis of LHON but did not receive genetic testing. A male cousin also had severe vision loss. Based on the likely family history of LHON, we performed genetic testing, which revealed the 11778 mitochondrial point mutation associated with this condition. We report a case of LHON with 34 years interval in vision loss in the fellow eye. LHON may develop in the second eye decades after its onset in the first. Detailed medical interviews and scrutiny, such as examination of family history, are warranted in consideration of LHON."}, {"id": "First_Aid_Step2_614", "title": "First_Aid_Step2", "score": 0.00980392156862745, "content": "If a 20-year-old female develops headaches after drinking red wine, think migraine. Associated symptoms/signs: Significant findings include fever or rash (consider meningitis or other infectious causes), jaw claudication (specific for temporal arteritis), or constitutional symptoms such as weight loss (associated with neoplastic, inflammatory, or infectious conditions). Photophobia, nausea, and vomiting are associated with migraine, aneurysmal SAH, and meningitis, but neck stiffness is more likely to accompany the latter two. Neurologic sequelae: Look for diplopia, mental status changes or associated symptoms (numbness, weakness, dizziness, ataxia, visual disturbances), papilledema, or pupillary abnormalities (partial CN III palsy or Horner’s syndrome). Patient risk factors: High-risk patients are > 50 years of age, immunocompromised, or with preexisting malignancy. If SAH is suspected, obtain a head CT without contrast. If CT is , LP is mandatory. Obtain a CBC."}, {"id": "pubmed23n0995_14786", "title": "Ultrasound-Assisted Diagnosis of Optic Neuritis in the Emergency Department: A Case Report.", "score": 0.009708737864077669, "content": "Optic neuritis is a common cause of subacute unilateral vision loss, occurring in 1-5 per 100,000 persons per year. It is more common in Caucasians, women, and those from countries with northern latitudes. Those aged 20-49 years are at greatest risk. The condition arises due to inflammation of the optic nerve. Inflammation may occur due to systemic inflammatory disorders, most commonly multiple sclerosis. A 21-year-old African-American male presented to our emergency department with a complaint of painful unilateral vision loss. On examination he was found to have a relative afferent pupillary defect and red desaturation. A bedside ultrasound suggested pseudopapilledema suggestive of optic neuritis. He was admitted to Neurology for confirmation of and treatment for optic neuritis. Magnetic resonance imaging confirmed optic neuritis. The patient was treated with i.v. steroids and discharged after improvement in visual function. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Optic neuritis is a clinical diagnosis. The subtle historical components and examination findings make it a diagnostic challenge for the busy emergency physician. Early diagnosis may improve visual outcomes. Discovery of pseudopapilledema on bedside ultrasound may be seen in optic neuritis, and is another finding that emergency physicians may assess for in patient presenting with unilateral vision loss."}, {"id": "pubmed23n0321_12634", "title": "Masticatory muscle pain: an important indicator of giant cell arteritis.", "score": 0.009708737864077669, "content": "Giant cell arteritis (GCA) is a polysymptomatic disease which constitutes an ophthalmic emergency because early recognition and management can prevent blindness. There is conflicting information in the literature on the validity, sensitivity, and specificity of various systemic symptoms and signs of GCA. This paper presents a review of our prospective studies on the subject, and our findings are particularly relevant to dentists. We investigated 363 patients in a prospective study. Positive temporal artery biopsy was seen in 106 patients and negative in 257 referred for diagnosis of GCA. Systemic symptoms and signs of GCA and erythrocyte sedimentation rate (Westergren-ESR) and C-reactive protein (CRP) levels were compared in these two groups of patients. The odds of having a positive temporal artery biopsy (i.e., GCA) were 9.1 times greater with jaw claudication (pain in masticatory muscles on eating), 3.4 times with neck pain, 3.2 times with CRP &gt; 2.45 mg/dL, 2.0 times with ESR 47.107 mm/hr, 2.7 times with ESR &gt; 107 mm/hr, and 2.0 times when the patients were aged &gt; or = 75 years. Other signs and symptoms did not show a significant association with a positive biopsy. Our study showed that \"normal\" ESR values do not rule out GCA but that CRP is a more useful test than ESR. Since jaw claudication is one of the most important symptoms of GCA, dentists should keep this possibility in mind when older patients come complaining of jaw pain while eating."}, {"id": "pubmed23n1145_24843", "title": "Paracentral Acute Middle Maculopathy After COVID-19 Disease: Multimodal Evaluation.", "score": 0.009615384615384616, "content": "To report the case and multimodal imaging findings of a healthy young woman who developed paracentral acute middle maculopathy (PAMM) 9 weeks after COVID-19 disease. Case report. Ultra-widefield fundus photography, macular spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography (FA), and OCT-angiography (OCT-A) were performed. A 36-year-old woman who developed PAMM 9 weeks after SARS-CoV-2 infection. A 36-year-old woman went to the emergency department (ED) with sudden, painless, left eye (LE) vision loss. The only relevant past medical history was COVID-19 disease 9 weeks before. Best corrected visual acuity (BCVA) was 20/200, a LE relative afferent pupillary defect (RAPD) was present and superficial hemorrhages throughout the macular area and peripheral retina were found. Nearly four hours after admission, LE BCVA recovered to 20/20 without RAPD. Five days after presentation in the ED, the patient returned with recurrent LE vision loss, with spontaneous recovery within 12 hours. Macular SD-OCT revealed hyperreflectivity of the inner plexiform and inner nuclear layers and the diagnosis of PAMM was established. The patient started oral acetylsalicylic acid and oral prednisolone. The patient did not report any new episodes of vision loss and there was a progressive resolution of abnormal fundus findings. SARS-CoV-2 infection increases the risk of vascular thrombotic events with possible involvement of the retinal circulation, and PAMM may present as a possible complication. Ophthalmologists should be able to recognize it promptly through multimodal imaging findings."}, {"id": "pubmed23n0596_4516", "title": "Anterior ischemic optic neuropathy due to giant cell arteritis with normal inflammatory markers.", "score": 0.009615384615384616, "content": "In anterior ischemic optic neuropathy (AION), it is important not to miss the diagnosis of giant cell arteritis (GCA) because this requires immediate steroid treatment to prevent involvement of the second eye and possible blindness. A missed diagnosis also might lead to fatal systemic complications. Observational case report. A 79-year-old woman noticed decreased visual and visual field loss in the right eye. At presentation, right visual acuity was 10/20 (ETDRS chart 2000). There was a right relative afferent pupillary defect of 0.6 log units. Asked for symptoms of GCA she complained about temporal and occipital headache, jaw claudication combined with malaise, and myalgia of the upper limbs. Laboratory tests showed normal inflammatory markers. Repeated tests confirmed ESR and CRP to be within the normal range. GCA being suspected, ultrasound of the superficial temporal arteries and temporal artery biopsy were performed unilaterally on the right side. Histology showed a chronic inflammatory cell infiltrate consistent with active GCA. The patient was treated with high-dose corticosteroids (250 mg methylprednisolone, three times/day, initially) and symptoms rapidly resolved, but visual loss remained unchanged. The case presented here proves that GCA with typical related visual loss (AION) is possible even when both ESR and CRP are in the normal range. Therefore, in the presence of typical symptoms, the clinician must not rely solely on laboratory testing, but start steroid therapy immediately and order a temporal artery biopsy."}, {"id": "wiki20220301en015_138436", "title": "Sciatica", "score": 0.009523809523809525, "content": "Cancer should be suspected if there is previous history of it, unexplained weight loss, or unremitting pain. Spinal epidural abscess is more common among those with diabetes mellitus or immunocompromised or who had spinal surgery, injection or catheter; it typically causes fever, leukocytosis and increased erythrocyte sedimentation rate. If cancer or spinal epidural abscess are suspected, urgent magnetic resonance imaging is recommended for confirmation. Proximal diabetic neuropathy typically affects middle aged and older people with well-controlled type-2 diabetes mellitus; onset is sudden causing pain usually in multiple dermatomes quickly followed by weakness. Diagnosis typically involves electromyography and lumbar puncture. Shingles is more common among the elderly and immunocompromised; usually (but not always) pain is followed by appearance of a rash with small blisters along a single dermatome. Acute Lyme radiculopathy may follow a history of outdoor activities during warmer"}, {"id": "pubmed23n0525_4392", "title": "[Blindness in both eyes due to late diagnosis of giant cell arteritis].", "score": 0.009523809523809525, "content": "Giant cell arteritis (GCA) is often diagnosed very late, variable \"facets\" of the disease exist render the diagnosis more difficult. Follow-up observations of five very old patients are reported in whom diagnosis was made too late, resulting in blindness of both eyes. Five patients (age 76-84 years, 4 women, one man) with GCA became blind in both eyes because diagnosis had been delayed (two patients) or onset of therapy was too late (three patients). In two patients who also had arterial hypertension, the symptom \"headache\" had been misleading. Symptoms of accompanying general diseases masked the real diagnosis, particularly in the second patient who had renal insufficiency, coronary artery disease, and unilateral obstruction of the internal carotid artery. Symptoms that failed to lead to the correct diagnosis were: muscle or chewing pain (three patients), circumscribed numbness around the mouth (second patient), and persistent headache despite normalization of blood pressure. Normal findings from cranial CTAs (two patients) led to the wrong reassurance of the patient. Swelling of the optic disk (two patients) was misdiagnosed by ophthalmologists, as was a retinal branch arterial occlusion (first patient). Three patients, afraid of possible side effects caused by glucocorticoids, took ineffective alternative medications. Poor vigilance led to blindness of the fifth patient with long-standing polymyalgia rheumatica. Targeted examinations at the onset of symptoms are necessary. GCA-symptoms were mis-constructed by additional diseases that disguised the correct diagnosis. The danger of bilateral blindness is particularly great in patients of great age."}, {"id": "pubmed23n1037_7823", "title": "A tearfully painful darkness.", "score": 0.009433962264150943, "content": "A 70-year-old woman presented with new onset of left eye and facial pain. Ophthalmic and neurological examinations, magnetic resonance imaging brain, erythrocyte sedimentation rate, and C-reactive protein were unrevealing. A few days later, she developed vision loss in her left eye. Examination revealed decreased visual acuity with a relative afferent pupillary defect in the left eye and a diffuse mild swelling of the left optic nerve head. Repeat magnetic resonance imaging showed T2 hyperintensity and enhancement of the intraorbital optic nerve and surrounding tissues with no other intracranial abnormalities. Serum studies showed elevated myelin oligodendrocyte glycoprotein IgG titer. She was treated with IV methylprednisolone 1000 mg daily for 3 days and was discharged on prolonged prednisone taper with return of vision to baseline."}, {"id": "pubmed23n0314_1466", "title": "[A 62-year-old man with an acute onset of consciousness disturbances].", "score": 0.009433962264150943, "content": "We report a 62-year-old man who developed coma and died in a fulminant course. The patient was well until May 1, 1996 when he noted chillness, tenderness in his shoulders, and he went to bed without having his lunch and dinner. In the early morning of May 2, his families found him unresponsive and snoring; he was brought into the ER of our hospital. He had histories of hypertension, gout, and hyperlipidemia since 42 years of the age. On admission, his blood pressure was 120/70, heart rate 102 and regular, and body temperature 36.3 degrees C. His respiration was regular and he was not cyanotic. Low pitch rhonchi was heard in his right lower lung field. Otherwise general physical examination was unremarkable. Neurologic examination revealed that he was somnolent and he was only able to respond to simple questions such as opening eyes and grasping the examiner's hand, but he was unable to respond verbally. The optic discs were flat; the right pupil was slightly larger than the left, but both reacted to light. He showed ptosis on the left side, conjugate deviation of eyes to the left, and right facial paresis. The oculocephalic response and the corneal reflex were present. His right extremities were paralyzed and did not respond to pain Deep tendon reflexes were exaggerated on the right side and the plantar response was extensor on the right. No meningeal signs were present. Laboratory examination revealed the following abnormalities; WBC 18,400/ml, GOT 131 IU/l GPT 50 IU/l, CK616 IU/l, BUN 30 mg/dl, Cr 2.1 mg/ dl, glucose 339 mg/dl, and CRP 27.4 mg/dl. ECG showed sinus tachycardia and ST elevation in II, III and a VF leads and abnormal q waves in I, V5, and V6 leads. Chest X-ray revealed cardiac enlargement but the lung fields were clear. Cranial CT scan revealed low density areas in the left middle cerebral and left posterior cerebral artery territories. The patient was treated with intravenous glycerol infusion and other supportive measures. At 2: 10 AM on May 3, he developed sudden hypotension and cardiopulmonary arrest. He was pronounced dead at 3:45 AM. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that the patient had acute myocardial infarction involving the inferior and the true posterior walls and left internal carotid embolism from a mural thrombus. Post mortem examination revealed occlusion of the circumflex branch of the left coronary artery due to atherom plaque rupture and myocardial infarction involving the posterior and the lateral wall with a rupture in the postero-lateral wall. Marked atheromatous changes were seen in the left internal carotid, the middle cerebral and the basilar arteries; the left internal carotid and the middle cerebral arteries were almost occluded by thrombi and blood coagulate. The territories of the left middle cerebral and the occipital arteries were infarcted; but the left thalamic area was spared. The neuropathologist concluded that the infarction was thrombotic origin not an embolic one as the atherosclerotic changes were severe. Cardiac rupture appeared to be the cause of terminal sudden hypotension and cardiopulmonary arrest. It appears likely that a vegetation which had been attached to the aortic valve induced thromboembolic occlusion of the left internal carotid artery which had already been markedly sclerotic by atherosclerosis. It is also possible that the vegetations in the aortic valve came from mural thrombi at the site of acute myocardial infarction, as no bacteria were found in those vegetations."}, {"id": "pubmed23n1076_12839", "title": "Childhood-Onset Leber Hereditary Optic Neuropathy: Particular Features.", "score": 0.009345794392523364, "content": "Leber hereditary optic neuropathy (LHON) is an optic neuropathy of mitochondrial inheritance. Childhood-onset disease is relatively rare and there are limited data on this important patient subgroup. We present 3 particular presentations of LHON. Patient 1 was an 8-year-old boy admitted to the emergency department reporting a progressive bilateral visual loss and intermittent headaches. Neuro-ophthalmological examination revealed a bilateral pseudopapilledema. Lumbar puncture identified intracranial hypertension and the brain and orbits magnetic resonance imaging showed T2 hyperintensity in the posterior region of the left optic nerve and the optic chiasm. Patient 2 was a 12-year-old boy admitted to the emergency department reporting painless, progressive central vision loss in the right eye. Fundus examination revealed a hyperemic disc and vascular network papillary and peripapillary vascular microdilations. Three months later, the left eye presented visual loss. Patient 3 was a 6-year-old female child referred to the neuro-ophthalmology specialist due to painless central visual loss in both eyes. Her BCVA was 1/10 and counting fingers in right and left eye, respectively, and fundus examination revealed a pallor optic disc in the temporal sector. The phenotype of childhood-onset disease may present itself distinct from classical adult-onset LHON. The absence of classical clinical features could lead to initial misdiagnosis. There should exist a high index of suspicion in children presenting unexplained subnormal vision in order to avoid potential diagnostic delays."}, {"id": "pubmed23n0244_2773", "title": "[The Tolosa-Hunt syndrome: report of a case with recurrent (9 times) painful ophthalmoplegia (author's transl)].", "score": 0.009345794392523364, "content": "A 48-year-old woman was referred to the First Dept. of Int. Med., Nagasaki Univ. Sch. Med., in August, 1979, with a six-month history of recurrent episodes of right-sided painful ophthalmoplegia and diplopia. An epidode affected the right eye, lasted one to two weeks, and relapsed every month. On examination she had a complete ptosis on the right side and pain on the right eye. All extraocular muscle supplied by the 3rd nerve were paralysed. The pupils were equal in size both sides, reacting to light completely. Visual acuity was normal except myopia. All the other cranial nerves and the remainder of central nervous system was normal. Results of thyroid function tests and of lumbar puncture were normal. The glucose tolerance test showed a mild diabetic pattern. Blood and CSF cultures for bacteria, fungi, and acid-fast bacillus were negative. The skull, brain CT scan, and carotid angiogram were within normal limits. A tentative diagnosis of Tolosa-Hunt syndrome was made after an unproductive search for a cause for this woman's painful ophthalmoplegia and unsuccessful treatment of ophthalmoplegia with antibiotics or diet therapy for mild hyperglycemia. The patient was given prednisolone 30 mg daily orally when she had the 9th attack of painful ophthalmoplegia Pain, ptosis, and diplopia disappeared in 5 days and she did not show any recurrence of symptoms over the next 7 months."}, {"id": "wiki20220301en053_41040", "title": "Posterior ischemic optic neuropathy", "score": 0.009259259259259259, "content": "Defective light perception in one eye causes an asymmetrical pupillary constriction reflex called the afferent pupillary defect (APD). Arteritic PION A-PION most commonly affects Caucasian women, with an average age of 73. At onset vision loss is unilateral, but without treatment it rapidly progresses to involve both eyes. Vision loss is usually severe, ranging from counting fingers to no light perception. Associated symptoms are jaw pain exacerbated by chewing, scalp tenderness, shoulder and hip pain, headache and fatigue. Perioperative PION Vision loss is usually apparent upon waking from general anesthesia. Signs observable to a bystander include long surgery duration and facial swelling. Vision loss is usually bilateral and severe, ranging from counting fingers to no light perception. Cause"}, {"id": "pubmed23n0334_10172", "title": "Lessons to be learned: a case study approach--a case of temporal arteritis.", "score": 0.009259259259259259, "content": "A 71-year-old male presented with a history of sudden partial visual loss in the right eye with an inferior visual field defect over the past 3-4 days. He had no history of headache or of facial pain. Clinical examination confirmed that vision on the right side was reduced to 6/18 and on the left to 6/12. The right eye showed a relative afferent pupillary defect. There was no other abnormality of the anterior segment of either eye. The right retina showed a pale swollen optic disc and a provisional diagnosis of anterior ischaemic optic neuropathy (AION) was made. An urgent erythrocyte sedimentation rate (ESR) was ordered and the patient was asked to return to the eye clinic in one month. However, 16 days later--when it was first recognised that his ESR was elevated to 75 mm in the first hour--the patient was recalled immediately in order to commence systemic steroid treatment; but regrettably, by this time, his right eye had become totally blind. In this case, although the attending doctor made a correct clinical diagnosis on presentation, he failed to act upon the result of the blood test."}, {"id": "wiki20220301en019_114604", "title": "Ankylosing spondylitis", "score": 0.009174311926605505, "content": "These diagnostic criteria include: Inflammatory back pain:Chronic, inflammatory back pain is defined when at least four out of five of the following parameters are present: (1) Age of onset below 40 years old, (2) insidious onset, (3) improvement with exercise, (4) no improvement with rest, and (5) pain at night (with improvement upon getting up) Past history of inflammation in the joints, heels, or tendon-bone attachments Family history for axial spondyloarthritis or other associated rheumatic/autoimmune conditions Positive for the biomarker HLA-B27 Good response to treatment with nonsteroidal anti-inflammatory drugs (NSAIDs) Signs of elevated inflammation (C-reactive protein and erythrocyte sedimentation rate) Manifestation of psoriasis, inflammatory bowel disease, or inflammation of the eye (uveitis) If these criteria still do not give a compelling diagnosis magnetic resonance imaging (MRI) may be useful. MRI can show inflammation of the sacroiliac joint. Imaging"}, {"id": "pubmed23n0479_17655", "title": "Progression of preexisting trigeminalgia to Tolose-Hunt-like syndrome. The importance of neuroimaging for early differential diagnosis.", "score": 0.009174311926605505, "content": "Recurrent unbearable, paroxysmal, unilateral facial pain in the distribution of one or more branches of the trigeminal nerve often provoked by sensory stimuli is typical for idiopathic trigeminal neuralgia. The less frequent localization in the area of ophthalmic branch (5%) is particularly controversial and should be distinguished from pathological lesions in the brainstem and middle and posterior cranial fossa and from diseases of the orbit and eye. This case study presents a 79-year-old woman with typical clinical features of 1st division trigeminalgia without any neurological loss and with normal results of laryngological, ophthalmological, and stomatological examinations as well as neuroimaging CT, and MR /MRA evaluation. Only the evoked potential blink and masseter reflexes demonstrated the pathological values in the early phase of illness. After 1 year of pharmacological treatment no improvement was achieved and the pain became neuropathic and paresis of 3rd, 4th and 6th nerves developed, as observed in Tolose-Hunt syndrome. MRI of the orbit revealed a pathological mass in its apex with a connection to the superior orbital fissure. However, treatment with steroids was completely ineffective. Surgical resection of the tumor (leiomyosarcoma) only partially reversed oculomotor palsy and diminished aching. In differential diagnosis of idiopathic and symptomatic trigeminalgia, early MR and MRA imaging is the most essential and sometimes may be the best single test to evaluate lesions even in distant areas of the nervous system branches."}, {"id": "pubmed23n0945_12479", "title": "Man with a Swollen Eye: Nonspecific Orbital Inflammation in an Adult in the Emergency Department.", "score": 0.00909090909090909, "content": "Nonspecific orbital inflammation (NSOI) is a rare idiopathic ocular pathology characterized by unilateral, painful orbital swelling without identifiable infectious or systemic disorders, which can be complicated by optic nerve compromise. A 50-year-old man presented to the Emergency Department with recurring, progressive painless left eye swelling, decreased visual acuity, and binocular diplopia in the absence of trauma, infection, or known malignancy. His physical examination was notable for left-sided decreased visual acuity, an afferent pupillary defect, severe left eye proptosis and chemosis, and restricted extraocular movements; his dilatated funduscopic examination was notable for ipsilateral retinal folds within the macula, concerning for a disruption between the sclera and the retina. Ocular examination of the right eye was unremarkable. Laboratory data were unrevealing. Gadolinium-enhanced magnetic resonance imaging showed marked thickening of the left extraocular muscles associated with proptosis, dense inflammatory infiltration of the orbital fat, and characteristics consistent with perineuritis. The patient was diagnosed with NSOI with optic neuritis and admitted for systemic steroid therapy; he was discharged on hospital day 2 after receiving high-dose intravenous (i.v.) methylprednisolone with significant improvement. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: NSOI is a rare and idiopathic ocular emergency, with clinical mimicry resembling a broad spectrum of systemic diseases such as malignancy, autoimmune diseases, endocrine disorders, and infection. Initial work-up for new-onset ocular proptosis should include comprehensive laboratory testing and gadolinium-enhanced magnetic resonance imaging. Timely evaluation by an ophthalmologist is crucial to assess for optic nerve involvement. Signs of optic nerve compromise include decreased visual acuity, afferent pupillary defect, or decreased color saturation. Patients with optic nerve compromise require admission for aggressive anti-inflammatory therapy with i.v. steroids in an attempt to reduce risk of long-term visual sequelae. Our case demonstrates a severe presentation of this disorder and exhibits remarkable visual recovery after 48 h of systemic i.v. steroid treatment."}, {"id": "First_Aid_Step2_571", "title": "First_Aid_Step2", "score": 0.00909090909090909, "content": "Also called giant cell arteritis; due to subacute granulomatous infl ammation of the large vessels, including the aorta, external carotid (especially the temporal branch), and vertebral arteries. The most feared manifestation is blindness 2° to occlusion of the central retinal artery (a branch of the internal carotid artery). Risk factors include polymyalgia rheumatica (affects almost half of TA patients), age > 50, and female gender. Presents with new headache (unilateral or bilateral); scalp pain and temporal tenderness; and jaw claudication. Fever, permanent monocular blindness, weight loss, and myalgias/arthralgias (especially of the shoulders and hips) are also seen. ESR > 50 (usually > 100). Ophthalmologic evaluation. Temporal artery biopsy: Look for thrombosis; necrosis of the media; and lymphocytes, plasma cells, and giant cells."}, {"id": "pubmed23n1033_13514", "title": "Leber's hereditary optic neuropathy following unilateral painful optic neuritis: a case report.", "score": 0.009009009009009009, "content": "Leber's hereditary optic neuropathy (LHON) is a maternally inherited mitochondrial disease, characterized by acute or subacute, painless, bilateral visual loss. LHON is often misdiagnosed as optic neuritis at an early stage because of the similarity of their clinical presentation. To date, there has been no reported case of actual optic neuritis and LHON in one patient. A 40-year-old, healthy man was referred to our clinic with acute painful visual loss in the right eye for 2 weeks. In the right eye, visual acuity decreased to 20/40, and the Ishihara colour test score was 8/14 with a relative afferent pupillary defect. Optic disc swelling was found only in the right eye, and magnetic resonance imaging revealed enhancement of the the right optic nerve, consistent with optic neuritis. After receiving 1 g of intravenous methylprednisolone daily for three days, his ocular pain resolved, and visual acuity improved to 20/20 within 2 weeks. Seven months later, the patient developed acute painless visual loss in the right eye. Visual acuity decreased to 20/200 in the right eye. There was no response to the intravenous methylprednisolone therapy at that time. Eight months later, he developed subacute painless visual loss in the left eye. Genetic testing for LHON was performed and revealed the pathologic mtDNA 11778 point mutation. We report a case with painful unilateral optic neuritis preceding the onset of LHON. Even if a typical optic neuritis patient has completely recovered from steroid treatment once in the past, it is advisable to keep in mind the possibility of LHON if acute or subacute loss of vision subsequently or simultaneously occurs in both eyes and does not respond to steroids."}, {"id": "pubmed23n0019_2125", "title": "[Arteriitis temporalis--a major disease developing in advance age (author's transl)].", "score": 0.009009009009009009, "content": "Arteriitis temporalis, a disease that was largely unknown in the early fifties, has gained importance in recent years. In the past ten years 32 patients suffering from arteriitis temporalis were diagnosed and treated both in the eye hospital and in the rheumatic-cardiological hospital of the Berlin-Buch Municipal Hospital. The following clinical findings were essential for the diagnosis: advanced or old age of the patient, massive headache in the temporal and/or occipital regions, myalgia primarily in the shoulders and the neck that responded relatively poorly to treatment, reduced eyesight, loss of weight and decrease in vitality. Extremely high BSR, an increased amount of alpha-2 and an increased level of alkaline phosphatase were most important among the laboratory findings. Corticosteroids have proved to be the medicine of choice for treating this condition."}, {"id": "pubmed23n0536_7105", "title": "Three presentations of monocular vision loss.", "score": 0.008928571428571428, "content": "Carotid artery disease is estimated to affect 30% of persons older than 50. Risk factors include hypertension, cigarette smoking, hyperlipidemia and diabetes mellitus. Symptoms ascribed to carotid artery lesions with stenosis of the artery or plaque formation include monocular vision loss and transient ischemic attacks. Patients can present with transient monocular vision loss as their initial symptom. Three patients from a geriatric clinic in Wilmington, Delaware presented with different complaints of vision loss with similar overall outcomes. Patient A was an 87-year-old woman who presented with dimming of vision on extreme left head turn. Dilated fundus examination found a retinal arterial emboli in the left eye (O.S.). Carotid duplex examination found 50% to 79% left internal carotid stenosis with no hemodynamic stenosis of the right internal carotid. Patient B was a 78-year-old woman who presented with a right superior altitudinal defect and transient vision loss in the right eye. Dilated fundus examination found retinal arterial emboli in the right eye (O.D.). Carotid duplex examination found 50% to 79% carotid stenosis in both the left and right internal carotids. Patient C was an 84-year-old man who complained of a superior altitudinal visual field defect O.D. Dilated fundus examination found a retinal arterial emboli O.D. Carotid duplex results showed calcified atherosclerotic plaques present at the level of the common carotid artery bifurcations bilaterally, with 50% to 70% narrowing of the right internal carotid artery with no significant narrowing of the left internal carotid artery. These 3 presentations show that in patients older than 50 who present with chief complaints of monocular vision loss, a differential diagnosis of carotid artery disease must be considered. Patients who exhibit retinal arterial emboli are at increased risk for stroke and vascular death. Appropriate measures for confirming a diagnosis include duplex ultrasound imaging, magnetic resonance angiography (MRA), and carotid angiography. Surgical techniques such as carotid angioplasty and carotid endarterectomy may be recommended."}, {"id": "pubmed23n0491_3108", "title": "Sometimes (what seems to be) a heart attack is (really) a pain in the neck.", "score": 0.008928571428571428, "content": "A 31-year-old patient complained of severe crushing chest pain that radiated to his left arm and jaw. After admission to the hospital, tests revealed a normal electrocardiogram, normal treadmill, normal coronary arteriogram, and normal cardiac enzymes. However, the patient continued to have pain, which was relieved by sublingual and intravenous nitroglycerine. He was discharged from the hospital with a diagnosis of \"musculoskeletal\" chest pain, taking nonsteroidal anti-inflammatory drugs, muscle relaxants, and narcotics. Two weeks later, the patient returned with worsening symptoms. Cardiac work-up was again negative. Thoracic and cervical spine radiographs were ordered for possible discogenic pain. After abnormalities were found on cervical radiographs, magnetic resonance imaging (MRI) was ordered, and the patient was referred to an orthopedic surgeon. Further work-up revealed a herniated disk at C6-C7, with radicular pain. Surgery on the suspect disk totally relieved the patient's pain."}, {"id": "pubmed23n1030_6059", "title": "Central retinal artery occlusion as initial presentation of Moyamoya disease in a middle-aged woman.", "score": 0.008849557522123894, "content": "To present a case of central retinal artery occlusion as the first symptomatic manifestation of Moyamoya disease in a middle-aged patient. Case report of a 48-year-old female Chinese-American patient who presented with sudden onset painless unilateral vision loss. Fundus photos, optical coherence tomography, fluorescein angiography, magnetic resonance angiography, computed tomography angiography, and catheter cerebral angiogram were performed. The patient's dilated fundus examination showed classic findings of a central retinal artery occlusion. Diagnostic brain imaging demonstrated extensive stenosis of the cerebrovascular network, with almost complete unilateral occlusion of the internal carotid artery along with compensatory collateral vessels. This led to a new diagnosis of Moyamoya disease. The patient was treated with extracranial-intracranial bypass surgery. Arterial abnormalities in patients with Moyamoya disease are uncommon and have previously only been reported in younger patients in their teens and 20s. Young and middle-aged patients presenting with central retinal artery occlusions should undergo complete neurologic workup including stroke evaluation; in this case, revealing Moyamoya disease, a rare yet life-threatening condition, as the underlying etiology."}, {"id": "pubmed23n1025_22054", "title": "Lessons of the month 4: Giant cell arteritis with normal inflammatory markers and isolated oculomotor nerve palsy.", "score": 0.008849557522123894, "content": "Giant cell arteritis (GCA) is an important condition to suspect and treat early, as failure to do so can result in anterior ischaemic optic neuropathy and subsequent permanent visual loss.A 71-year-old woman presented to her local emergency department with a 1-week history of constant, moderate-severe global headache associated with intermittent periorbital pain. Two weeks later she developed sudden horizontal diplopia. Examination demonstrated right oculomotor nerve palsy. Her erythrocyte sedimentation rate (ESR) was 9 mm/hr. Repeat blood tests 1 month later showed an ESR of 67 mm/hr. Temporal artery biopsy was positive.A review from a cohort of 764 patients with suspected GCA who underwent biopsy found the sensitivity of an elevated ESR and c-reactive protein was 84% and 86%, respectively, but the specificity was only 30%. Therefore, inflammatory markers should only act as a guide, and caution should be taken in their interpretation especially with respect to the time of sampling in the disease evolution.Isolated oculomotor nerve palsy in association with GCA is rare. The first case series was described by miller fisher in 1959 who observed two patients presenting with diplopia, ptosis and ocular palsies. In anyone over the age of 50 who develops a new, refractory headache and cranial neuropathy, GCA should be the first consideration."}, {"id": "pubmed23n1143_24145", "title": "Isolated Infiltrative Optic Neuropathy in an Acute Lymphoblastic Leukemia Relapse.", "score": 0.008771929824561403, "content": "Optic nerve infiltration as the first sign of isolated central nervous system relapse of acute lymphoblastic leukemia (ALL) is rare. A seven-year-old girl with standard-risk B-cell ALL who was in remission presented with sudden onset of left eye pain and loss of vision. Examination revealed no perception to light in the left eye with positive relative afferent pupillary defect. The optic disc was hyperemic and swollen with total obscuration of the disc margin associated with central retinal artery and vein occlusion. Magnetic resonance imaging of the brain and optic nerve showed left intraorbital optic nerve thickening associated with perineural enhancement and intraconal fat involvement. Lumbar puncture revealed leukemic infiltration with blast cells after a week of eye symptoms, while bone marrow aspiration was negative for malignant cells. A diagnosis of left leukemic optic nerve infiltration with central retinal artery and vein occlusion was made. A high index of suspicion with repeat cerebrospinal fluid sampling is crucial to confirm the diagnosis as vitreous biopsy may fail to reveal infiltrative cells."}, {"id": "wiki20220301en053_41039", "title": "Posterior ischemic optic neuropathy", "score": 0.008695652173913044, "content": "Signs and symptoms PION is characterized by moderate to severe painless vision loss of abrupt onset. One or both eyes may be affected and color vision is typically impaired. Ophthalmoscopic exam Looking inside the person's eyes at the time of onset, ophthalmoscope exam reveals no visible changes to the optic nerve head. Weeks after ischemic insult, nerve atrophy originating from the damaged posterior optic nerve progresses to involve the anterior optic nerve head. Four to eight weeks after onset, atrophy of the optic nerve head is observable upon ophthalmoscope exam. Pupils If both eyes are affected by PION, the pupils may look symmetrical. However, if the eyes are asymmetrically affected, i.e. one eye's optic nerve is more damaged than the other, it will produce an important sign called an afferent pupillary defect. Defective light perception in one eye causes an asymmetrical pupillary constriction reflex called the afferent pupillary defect (APD)."}, {"id": "pubmed23n0524_20048", "title": "[Transient trochlear nerve palsy as the presenting neurological sign of panarteritis nodosa].", "score": 0.008695652173913044, "content": "Panarteritis nodosa (PAN) is a systemic vasculitis affecting small and medium-sized arteries. Neuro-ophthalmological complications of PAN are rare but numerous, and may affect the eye, the visual and the oculomotor pathways. Such complications occur mainly in patients previously diagnosed with PAN. A 51-year-old woman presented with an isolated right trochlear (IV) palsy, in the setting of headaches and fluctuating fever of unknown etiology. Erythrocyte sedimentation rate was 13 mm and full blood cell count was normal. Previous chest X-ray and blood studies were negative for an infection or inflammation. Orbital and cerebral CT scan was normal. Spontaneous recovery of diplopia ensued over four days. Two days later, paresthesia and sensory paresis of the dorsal portion of the left foot were present. Lumbar puncture revealed 14 leucocytes (76 percent lymphocytes) with elevated proteins, but blood studies and serologies were negative. A diagnosis of undetermined meningo-myelo-radiculoneuritis was made. Because of a possible tick bite six weeks previously the patient was empirically treated with 2 g intravenous ceftriaxone for 3 weeks. Fever rapidly dropped. Six weeks after the onset of diplopia, acute onset of blindness in her right eye, diffuse arthralgias and fever motivated a new hospitalization. There was a central retinal artery occlusion of the right eye. Blood studies now revealed signs of systemic inflammation (ESR 30 mm, CRP 12 mg/L, ANA 1/80, pANCA 1/40, leucocytosis 12.4 G/L, Hb 111 g/L, Ht 33 percent). Biopsy of the left sural nerve revealed arterial fibrinoid necrosis. A diagnosis of PAN was made. Transient diplopia can be the heralding symptom of a systemic vasculitis such as PAN, giant cell arteritis and Wegener granulomatosis. In this patient the presence of accompanying systemic symptoms raised a suspicion of systemic inflammation, but the absence of serologic and imaging abnormalities precluded a specific diagnosis initially. A few weeks later, the presence of a second ischemic event (retinal) and positive blood studies led to a further diagnostic procedure. Oculomotor and abducens palsies have rarely been reported in association with PAN. We report the first case of trochlear nerve paresis as the inaugural neurological sign of PAN. This case highlights the importance of considering inflammatory systemic disorders in patients with acute diplopia particularly when they are young, lack vascular risk factors or cause, and complain of associated systemic symptoms."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[18, 175]], "word_ranges": [[3, 24]], "text": "The picture described is very suggestive of pseudocrisis with asynchronous limb movements, pelvic movements, crying and poor response to antiepileptic drugs."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Correct answer 1: The picture described is very suggestive of pseudocrisis with asynchronous limb movements, pelvic movements, crying and poor response to antiepileptic drugs.", "full_answer_no_ref": "Correct answer 1: The picture described is very suggestive of pseudocrisis with asynchronous limb movements, pelvic movements, crying and poor response to antiepileptic drugs.", "full_question": "In a patient diagnosed with epilepsy who presents with episodes of unresponsiveness to external stimuli, irregular movements of all four limbs, closed eyes, crying and pelvic movements, lasting five to twenty seconds and unresponsive to treatment with antiepileptic drugs, which complementary study is most likely to clarify the diagnosis?", "id": 32, "lang": "en", "options": {"1": "Video-EEG monitoring for diagnosis of pseudocrisis (psychogenic seizures).", "2": "Holter ECG for diagnosis of arrhythmic heart disease.", "3": "Routine EEG to diagnose the type of epilepsy (generalized or foc).", "4": "Brain MRI to detect epileptogenic lesions (cortical dysplasia, tumor, medial temporal sclerosis).", "5": "Determine capillary blood glucose for diagnosis of hypoglycemia."}, "question_id_specific": 64, "type": "NEUROLOGY AND NEUROSURGERY", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en323_32903", "title": "Vertiginous epilepsy", "score": 0.018162393162393164, "content": "Other means used in diagnosis of vertiginous epilepsy include: Electroencephalography (EEG) Magnetic resonance imaging (MRI) Positron emission tomography (PET) Neuropsychological testing The EEG measures electrical activity in the brain, allowing a physician to identify any unusual patterns. While EEGs are good for identifying abnormal brain activity is it not helpful in localizing where the seizure originates because they spread so quickly across the brain. MRIs are used to look for masses or lesions in the temporal lobe of the brain, indicating possible tumors or cancer as the cause of the seizures. When using a PET scan, a physician is looking to detect abnormal blood flow and glucose metabolism in the brain, which is visible between seizures, to indicate the region of origin. Management"}, {"id": "wiki20220301en063_48102", "title": "Psychogenic non-epileptic seizure", "score": 0.012961578178969483, "content": "The most definitive test to distinguish epilepsy from PNES is long term video-EEG monitoring, with the aim of capturing one or two episodes on both video recording and EEG simultaneously (some clinicians may use suggestion to attempt to trigger an episode). Additional clinical criteria are usually considered in addition to video-EEG monitoring when diagnosing PNES. By recording the event in question on video and EEG simultaneously, a clear diagnosis can usually be obtained. Laboratory testing can detect rising blood levels of serum prolactin if samples are taken in the right time window after most tonic-clonic or complex partial epileptic seizures. However, due to false positives and variability in results, this test is relied upon less frequently."}, {"id": "wiki20220301en228_24736", "title": "Paroxysmal dyskinesia", "score": 0.012637008381689232, "content": "The pathogenesis of PED has also been linked to mutations in the GLUT1 glucose transporter which can result in transient energy deficits in the basal ganglia. Diagnosis Diagnosis is similar, but slightly different for each type of PD. Some types are more understood than others, and therefore have more criteria for diagnosis. PKD The guidelines for diagnosing PKD were reviewed and confirmed by Unterberger and Trinka. PKD consists of unexpected forms of involuntary movements of the body. The patient is usually diagnosed sometime before their 20s, and is more likely diagnosed during childhood than early adulthood. Almost all PKD's are idiopathic, but there have been examples of autosomal dominant inheritance as well. Physical examination and brain imaging examinations show normal results, and an EEG shows no specific abnormalities as well. However, the negative synchronous EEG results can be used to prove that PKD is not a sort of reflex epilepsy, but a different disease."}, {"id": "wiki20220301en056_68359", "title": "Lennox–Gastaut syndrome", "score": 0.011817387505460901, "content": "To confirm diagnosis, awake and asleep EEG and magnetic resonance imaging (MRI) are performed. MRI is used to detect focal brain lesions. Ruling out other diagnoses Certain diagnoses must be ruled out before diagnosing LGS. These diagnoses are: Doose syndrome Dravet syndrome pseudo-Lennox Gastaut syndrome (atypical benign partial epilepsy) LGS is more easily distinguished from Doose syndrome by seizure type after the syndrome has progressed. Doose syndrome has more myoclonic seizures and LGS has more tonic seizures. The Doose syndromes is less likely to have cognitive disabilities. Pseudo-Lennox–Gastaut syndrome can be distinguished from LGS because pseudo-LGS has different spike-and-wave patterns on EEG. Treatment There are several treatment options, including medications, surgery, and diet. Medications In most patients with LGS, the treatment does not end seizure recurrence."}, {"id": "pubmed23n0371_17131", "title": "[Neuroimaging and electrophysiological study in epilepsy].", "score": 0.011666666666666665, "content": "There exist various morphological and biochemical changes closely associated with electrophysiological phenomena which cause epileptic seizures in the brains of epilepsy patients. Recent developments in investigation methods, not only electrophysiological(EEG and MEG), but also neuroimaging involving morphological imaging(CT and conventional MRI) and functional imaging(SPECT, PET, functional MRI and MRS) is able to demonstrate these changes. SPECT and PET can particularly clarify the changes of cerebral blood flow and glucose metabolism between interictal and ictal periods. In our experience of 423 patients who underwent epilepsy surgery for intractable seizures, these interventions provide important information to identify the epileptogenic foci. However, in practice, discordance in the results of these presurgical evaluations is recognized, and invasive intracranial recordings are needed in such cases. These problems in diagnosis were shown especially in patients with mesial temporal sclerosis and focal cortical dysplasia. To detect an epileptogenic focus more clearly, a combination of morphological and functional findings, new functional imaging such as neurotransmitter receptor imaging, EEG-triggered or neuropharmacological functional MRI, as well as, statistical parametric analysis may be needed."}, {"id": "wiki20220301en172_19823", "title": "Progressive myoclonus epilepsy", "score": 0.011447039199332779, "content": "Diagnosis Diagnosis of PME is based on the individual’s signs and symptoms as well as failure to respond to antiepileptic drugs and therapy. Further diagnosis support includes EEG results, genetic testing, enzyme testing, and skin and muscle biopsies. Gaucher’s disease can be diagnosed through enzyme testing as it is a metabolic disease. Lafora’s disease can be diagnosed using skin biopsies. While Action myoclonus renal failure (AMRF) syndrome can only be diagnosed using genetic test. Using EEG’s as a form of diagnosis can prove difficult as patients differ in their neurophysiology. In Lafora’s disease EEGs can show slowing background activity or focal discharges as well as epileptiform discharges. In ULD EEGs show generalized epileptiform discharges and in MERRF patients show background slowing. Therefore, diagnosis is best made using a combination of different tools like signs and symptoms, age of onset, EEG, gene testing, enzyme measurements, and biopsy of skin and muscle."}, {"id": "wiki20220301en000_158053", "title": "Epilepsy", "score": 0.011042253521126762, "content": "For adults, the testing of electrolyte, blood glucose and calcium levels is important to rule out problems with these as causes. An electrocardiogram can rule out problems with the rhythm of the heart. A lumbar puncture may be useful to diagnose a central nervous system infection but is not routinely needed. In children additional tests may be required such as urine biochemistry and blood testing looking for metabolic disorders. Together with EEG and neuroimaging, genetic testing is becoming one of the most important diagnostic technique for epilepsy, as a diagnosis might be achieved in a relevant proportion of cases with severe epilepsies, both in children and adults. For those with negative genetic testing, in some it might be important to repeat or re-analyze previous genetic studies after 2–3 years."}, {"id": "wiki20220301en000_158038", "title": "Epilepsy", "score": 0.01063034188034188, "content": "Diagnosis The diagnosis of epilepsy is typically made based on observation of the seizure onset and the underlying cause. An electroencephalogram (EEG) to look for abnormal patterns of brain waves and neuroimaging (CT scan or MRI) to look at the structure of the brain are also usually part of the initial investigations. While figuring out a specific epileptic syndrome is often attempted, it is not always possible. Video and EEG monitoring may be useful in difficult cases. Definition Epilepsy is a disorder of the brain defined by any of the following conditions: {| cellpadding=5 style=\"border:1px solid #ccc\" |- bgcolor=\"#fafafa\" | At least two unprovoked (or reflex) seizures occurring more than 24 hours apart One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years Diagnosis of an epilepsy syndrome |}"}, {"id": "pubmed23n0543_2570", "title": "Psychogenic pseudosyncope: an underestimated and provable diagnosis.", "score": 0.009900990099009901, "content": "The goal of this study was to estimate the frequency of psychogenic pseudosyncope in patients with \"syncope of unknown origin.\" Twenty to thirty percent of patients referred to epilepsy centers for refractory seizures have psychogenic seizures. With syncope, about 20-30% of the cases remain unexplained after a complete evaluation, but, unlike in seizures, a psychogenic etiology is not usually investigated. We prospectively evaluated patients referred to our epilepsy center for evaluation of recurrent syncope-like episodes, that is, limp, motionless fainting. All patients had a negative syncope workup. We performed EEG-video monitoring with activation by suggestion (\"induction\"), similar to what is used for diagnosis of psychogenic seizures. Activation was performed with patients standing or sitting up. The diagnosis of psychogenic pseudosyncope required: (1) an activation procedure that triggered the habitual event; (2) a clinical event of loss of postural tone and limp, motionless unresponsiveness with eyes closed; (3) normal EEG before, during, and after the clinical event, that is, no epileptiform abnormalities, a normal alpha rhythm during unresponsiveness, and no suppression of background or slowing as is typically seen in syncope. Ten patients were recruited over an 18-month period. Habitual syncope-like episodes were triggered in 9 of 10 (90%) patients, and all 9 were shown to have psychogenic pseudosyncope (eyes closed, motionless, unresponsive with normal EEG including normal alpha rhythm). In one patient, no episode was triggered, so a diagnosis could not be made. Among the 9 patients for whom episodes were recorded, age ranged from 21 to 60 (mean=36). Five were women. Duration of symptoms ranged from 6 months to 15 years (mean=4.2 years). Event frequency ranged from four per day to two per month. Prior evaluations for syncope included ECG in all patients, two-dimensional echocardiogram in three, Holter monitoring in two, and tilt-table test in five. Four patients had undergone cardiac catheterization, and one had received a pacemaker. Neurologic tests included CT of the head in seven and MRI of the brain in eight. Many patients with \"syncope of unknown origin\" may have psychogenic pseudosyncope, but most such patients do not undergo EEG-video monitoring, which is the only way to demonstrate a psychogenic etiology. Psychogenic pseudosyncope is not simply a diagnosis of exclusion, and can be firmly diagnosed. As is usually recommended for seizure-like events, patients with syncope-like events and a negative evaluation should undergo EEG-video monitoring with induction, specifically looking for a possible psychogenic etiology."}, {"id": "pubmed23n0206_20604", "title": "Hypoglycemic activation of focal abnormalities in the EEG of patients considered for temporal lobectomy.", "score": 0.009900990099009901, "content": "EEGs were recorded in 22 patients with medically refractory complex partial epilepsy undergoing presurgical evaluation and 11 age-matched controls while subjected to moderate levels of hypoglycemia to determine if changes activated were predictive of underlying pathology. Five patients had fasting EEGs showing focal abnormalities not seen in the non-fasting state. With hypoglycemia, EEG tracings in normal individuals showed diffuse background slowing, whereas 7 of 22 patients developed focal temporal changes, including focal spike and focal slow wave activation. The development of focal changes correlated well with clinical data concerning underlying focal pathology; focal abnormalities were not evoked in patients with multifocal disease. Hypoglycemic activation of the EEG may be a useful technique for predicting the presence of pathology in patients considered for anterior temporal lobectomy."}, {"id": "wiki20220301en583_4018", "title": "Diagnosis (American TV series)", "score": 0.009890205646566747, "content": "Kamiyah Morgan is a 6 year old little girl who suffers from a very unusual set of fainting episodes that will leave her unresponsive and immobile, and they can happen up to 300 times a day. When she experiences a fainting episode she will become completely paralyzed affecting everything in her body including her lungs, her mother states that every day that passes her ability to breathe diminishes. Kamiyah’s mother said that these fainting episodes started when she was about 8 months old as she would be crawling and suddenly tip over and go limp. At first their pediatric physician said the episodes looked like she was having a seizure, but after running an EEG there was no seizure being detected during the episodes. They then tried testing with MRI for any brain tumors or malignancies but again there was nothing. They were then referred over to the NIH or the National Institute of Health where their entire purpose is to be able to research and hopefully diagnose very strange cases."}, {"id": "wiki20220301en203_16065", "title": "Post-traumatic epilepsy", "score": 0.00980392156862745, "content": "Diagnosis To be diagnosed with PTE, a person must have a history of head trauma and no history of seizures prior to the injury. Witnessing a seizure is the most effective way to diagnose PTE. Electroencephalography (EEG) is a tool used to diagnose a seizure disorder, but a large portion of people with PTE may not have the abnormal \"epileptiform\" EEG findings indicative of epilepsy. In one study, about a fifth of people who had normal EEGs three months after an injury later developed PTE. However, while EEG is not useful for predicting who will develop PTE, it can be useful to localize the epileptic focus, to determine severity, and to predict whether a person will suffer more seizures if they stop taking antiepileptic medications. Magnetic resonance imaging (MRI) is performed in people with PTE, and CT scanning can be used to detect brain lesions if MRI is unavailable. However, it is frequently not possible to detect the epileptic focus using neuroimaging."}, {"id": "pubmed23n0094_19906", "title": "[Recently experienced ten cases of insulinoma--preoperative diagnosis of localization and intraoperative simultaneous monitoring of glucose and insulin].", "score": 0.00980392156862745, "content": "We have experienced 10 cases of insulinoma during the last 10 years from 1977 to 1986. All cases had strong hypoglycemic symptoms such as disturbance of consciousness, and insulinoma still tended to be misdiagnosed as epilepsy. The diagnosis of insulinoma was easily available from serum IRI (immunoreactive insulin)/plasma glucose ratio in all of the ten cases. As preoperative procedures for the diagnosis of localization, arteriography, computed tomography and portal blood sampling were positive in 6 of 8, 4 of 6 and 2 of 2 patients, respectively. At operation, all insulinomas could be identified by digital palpation. We performed simple excision of the tumor in 6 patients and distal pancreatectomy in 4 patients. The tumors were solitary and benign in all patients, ranging in size from 1.0 cm to 4.5 cm. Three cases were presented as case reports. In these cases, portal blood sampling and/or intraoperative monitoring of plasma glucose and serum IRI were performed. Portal blood sampling was effective even for a case which was negative in image diagnostic procedures. Furthermore, simultaneous monitoring of plasma glucose and serum IRI by quick radioimmunoassay seemed to be a good guide to the completeness of resection of insulin producing tumors."}, {"id": "wiki20220301en253_30183", "title": "Electroencephalography", "score": 0.009708737864077669, "content": "Epilepsy monitoring is typically done to distinguish epileptic seizures from other types of spells, such as psychogenic non-epileptic seizures, syncope (fainting), sub-cortical movement disorders and migraine variants, to characterize seizures for the purposes of treatment, and to localize the region of brain from which a seizure originates for work-up of possible seizure surgery. Hospitals use an EEG monitor to help diagnose a seizure. They use that information to help with the treatment process as well as discovering risks. \"Many professionals have stated the importance of EEG’s when it comes to suspected seizures, for diagnosis and evaluation\". Doctors will be able to use the EEG monitoring system to help look at some treatment options as well as some risk factors. As technology advances, researchers are finding new monitors that are more accurate in regards to seizures. \"Advanced techniques with continuous EEG and simplified technique with aEEG allows clinicians to detect more"}, {"id": "pubmed23n0046_18662", "title": "Regional brain glucose metabolism in patients with complex partial seizures investigated by intracranial EEG.", "score": 0.009708737864077669, "content": "We performed interictal 18F-2-fluoro-2-deoxy-D-glucose positron emission tomography (18FDG-PET) studies in 57 patients with complex partial epilepsy (CPE), not controlled by medical treatment and considered for surgical resection of their epileptic focus. A precise localization of the epileptic focus was obtained in 37 of these patients with a combination of subdural and depth electrodes. We visually inspected the metabolic images; we also measured glucose consumption in a number of brain regions and compared the values with those obtained in 17 normal controls. Eighty-two percent of the 57 patients had an area of glucose hypometabolism on the 18FDG-PET images. Six patients had a frontal epileptic focus, 3 of them had a frontal lobe hypometabolism. Twenty-six patients had a unilateral temporal lobe focus and all of them displayed a temporal lobe hypometabolism. The asymmetry was more pronounced in the lateral temporal cortex (-20%) than in the mesial part of the temporal lobe (-9.6%). In each cortical brain region on the side of the epileptic focus (except the sensorimotor cortex), glucose consumption rate was lower than in the contralateral region or than in controls. No differences could be found between patients with a seizure onset restricted to the hippocampus and patients with a seizure onset involving the hippocampus and the adjacent neocortex. Divergent metabolic patterns were obtained in 5 patients with bilateral temporal seizure foci. Combined with other non invasive techniques (EEG, neuroradiology), PET contributes increasingly to the selection of patients with CPE who could benefit from surgical treatment.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0603_20146", "title": "[Insulinoma misdiagnosed and treated as epilepsy].", "score": 0.009615384615384616, "content": "Although insulinoma constitutes almost 90% of neuroendocrine tumors localized in the pancreas, it is a rare disease. Quite commonly prior to the diagnosis there is a history of several years and misdiagnosis as neurological or cardiological disease is not infrequent. Patient, 22 years old, since 7 years experiencing multiple incidents of neuroglycopenia with concurrent hyperadrenergic reaction. Consciousness disturbances and muscle tremor together with feeling of hunger and tachycardia occurred mainly in the morning hours, or after physical exercise, and subsided after glucose intake. Increase in body weight, typical for insulinoma, was also observed. The patient was hospitalized twice in Pediatric Department and although hypoglycemia was observed, no additional testing was performed to exclude insulinoma; reported symptoms and abnormalities in EEG recording after provocation resulted in diagnosis and treatment of epilepsy. During hospitalization in the Department of Endocrinology fasting test was performed, which revealed inadequately high insulin level with glucose level of 41 mg% and signs of neuroglycopenia. The image of pancreas was normal in the acquired abdominal ultrasound and in CT a tumor was found in the tail of pancreas. The patient underwent laparoscopic operation and the clinical diagnosis was confirmed by histopathology. Antiepileptic drugs were discontinued. Total remission of symptoms was achieved. The presented case demonstrates the difficulties in correct interpretation of reported symptoms, while the results of biochemical tests and imaging studies point precisely to the diagnosis. Focal neurological signs resulting from multiple episodes of hypoglycemia may lead to misdiagnosis and treatment of epilepsia."}, {"id": "pubmed23n0213_13491", "title": "Hypometabolic cortical lesions in tuberous sclerosis with epilepsy: demonstration by positron emission tomography.", "score": 0.009523809523809525, "content": "Four patients with a well-established diagnosis of tuberous sclerosis and grand mal type epileptic seizures as their principal clinical symptom were examined by conventional surface electroencephalography (EEG), X-ray computed tomography, and positron emission tomography (PET) using the [18F]-2-fluoro-2-deoxyglucose method. The interictal EEG showed various abnormalities of poor localizing value, but no focal epileptic discharges. X-ray computed tomography demonstrated subependymal calcifications in all cases, although cortical lesions were found only twice. However, in the PET images of each patient one or two localized cortical foci with a metabolic rate for glucose more than 40% lower than in the respective contralateral region were clearly delineated. It may be assumed that those hypometabolic areas represent the epileptogenic cortical tubers, which are characteristic of the disease but usually cannot be detected in vivo by other methods."}, {"id": "wiki20220301en001_111077", "title": "Seizure", "score": 0.00950943056087613, "content": "In adults, testing electrolytes, blood glucose and calcium levels is important to rule these out as causes, as is an electrocardiogram. A lumbar puncture may be useful to diagnose a central nervous system infection but is not routinely needed. Routine antiseizure medical levels in the blood are not required in adults or children. In children additional tests may be required. A high blood prolactin level within the first 20 minutes following a seizure may be useful to confirm an epileptic seizure as opposed to psychogenic non-epileptic seizure. Serum prolactin level is less useful for detecting partial seizures. If it is normal an epileptic seizure is still possible and a serum prolactin does not separate epileptic seizures from syncope. It is not recommended as a routine part of diagnosis epilepsy."}, {"id": "wiki20220301en010_49780", "title": "Macropsia", "score": 0.009433962264150943, "content": "Epilepsy Macropsia may present itself as a symptom of both frontal lobe epilepsy and temporal lobe epilepsy, which may actually help in the diagnosis of those diseases. Children who experience nocturnal hallucinations accompanied by macropsia may seek medical care for panic attack disorders and instead are diagnosed with forms of epilepsy. Epilepsy patients may have no memory of the seizure, but can remember the hallucinations and aura which proceed the attack. Electroencephalography, or EEG imaging, can then be utilized while the patient experiences the episode. It may be subsequently concluded that the EEG is congruent with temporal or frontal lobe seizure. Anxiety and headaches accompany the episodes of visual distortion associated with epilepsy. While Valproic acid has been used to treat this type of seizure, anti-seizure medications appropriate for focal-onset seizures, like oxcarbazapine, have also been used successfully in the treatment of epilepsy-related macropsia."}, {"id": "pubmed23n0130_13895", "title": "Local cerebral metabolic rate for glucose during petit mal absences.", "score": 0.009433962264150943, "content": "Four patients with primary generalized or true petit mal epilepsy were studied with positron emission tomography using [18F]fluorodeoxyglucose (FDG). FDG studies were carried out during 10 minutes of hyperventilation before and again after medical control of spontaneous absences. Before seizures were controlled all 4 patients demonstrated frequent bilaterally synchronous three-per-second spike-and-wave discharges associated with altered consciousness. After spontaneous seizures were controlled, hyperventilation produced only electroencephalographic slowing without clinical symptoms in 3; the fourth patient had absences less frequently. Patterns of local cerebral metabolic rate for glucose (CMRGlc) were normal and identical for ictal and interictal scans; there was, however, a 2.5- to 3.5-fold diffuse ictal increase in global CMRGlc evident when ictal studies were compared with hyperventilation control studies in which no seizures occurred. The CMRGlc was similar in the two scans obtained from the patient who had absences during both studies. No anatomical substrate of petit mal epilepsy was identified. The CMRGlc in these patients during petit mal absences was higher than that recorded in other patients during partial or generalized convulsive seizures. This difference may reflect the fact that petit mal absences are not associated with postictal depression."}, {"id": "wiki20220301en025_34869", "title": "Ictal headache", "score": 0.009345794392523364, "content": "For the diagnosis it is necessary to perform an EEG during the headache that shows epilepsy-compatible discharges coinciding with the onset and cessation of the headache. The so-called hemicrania epileptica is a variant of EH characterized by the fact that head pain and EEG paroxysms are located on the same side. MRI is necessary to establish the cause, which, as in all focal epilepsies, can be varied: malformations/dysplasia, neoplasms, encephalopathies, traumatic brain injury, vasculopathies. Therapy. It depends on the etiology. During the headache, like most seizures, i.v. benzodiazepines are usually effective. Antiepileptic drugs can be used as preventive. 2. Ictal non-epileptic headache. Rare cases are reported. It is a condition that can be differentiated with certainty from the previous one if the headache episode is also present outside the seizure, that is, before and/or after, without specific EEG abnormalities. References External links"}, {"id": "pubmed23n0227_4297", "title": "Interictal cerebral glucose metabolism in partial epilepsy and its relation to EEG changes.", "score": 0.009345794392523364, "content": "Interictal positron computed tomography (PCT) with 18F-fluorodeoxyglucose was performed on 50 patients with partial seizures disorders. Electroencephalographic (EEG) monitoring was carried out during the metabolic studies using scalp and sphenoidal electrodes in 33 patients and stereotaxically implanted depth electrodes in 17. Four patients in this series had focal abnormalities on x-ray computed tomographic scans, but these were at the site of the presumed epileptogenic lesion in only 2. One or more discrete zones of hypometabolism were identified in 35 patients, and only 1 patient appeared to show focal interictal hypermetabolism. No quantitative relationship could be demonstrated between the degree of focal hypometabolism and either the frequency of interictal EEG spikes of the presence of focal nonepileptiform EEG changes. It was concluded that metabolic and electrophysiological techniques measure different aspects of cerebral dysfunction in seizure disorders. Although interictal PCT in patients with partial epilepsy usually demonstrates zones of hypometabolism this finding, per se, does not reveal the epileptic nature of the abnormality."}, {"id": "wiki20220301en128_19212", "title": "Unverricht–Lundborg disease", "score": 0.009259259259259259, "content": "Other methods to diagnose Unverricht–Lundborg disease are currently being explored. While electroencephalogram (EEG) is useful in identifying or diagnosing other forms of epilepsy, the location of seizures in ULD is currently known to be generalized across the entire brain. Without a specific region to pinpoint, it is difficult to accurately distinguish an EEG reading from an individual with ULD from an individual with another type of epilepsy characterized by generalized brain seizures. However, with recent research linking ULD brain damage to the hippocampus, the usefulness of EEG as a diagnostic tool may increase. Magnetic Resonance Imaging (MRI) is also often used during diagnosis of patients with epilepsy. While MRIs taken during the onset of the disease are generally similar to those of individuals without ULD, MRIs taken once the disease has progressed show characteristic damage"}, {"id": "pubmed23n0498_8099", "title": "Symptomatic occipital lobe epilepsy following neonatal hypoglycemia.", "score": 0.009259259259259259, "content": "This study reports on the clinical, electrophysiologic, and neuroradiologic aspects of patients with epilepsy secondary to neonatal hypoglycemia. Fifteen patients with epilepsy and/or posterior cerebral lesions, and neonatal hypoglycemia were studied in the epilepsy clinic between February 1990 and March 2003. The mean age was 12 years. The different types of neonatal hypoglycemia were as follows: four patients had transitional-adaptive, seven classic transient, two secondary-associated, and two severe recurrent hypoglycemia. As to epilepsy, we recognized a larger group of 12 patients characterized by focal seizures and posterior abnormalities on the electroencephalogram, the majority of whom had a good outcome, and a second group of two patients presenting electroclinical features of encephalopathy with refractory seizures. All patients except two manifested parieto-occipital lesions on neuroradiologic images. Neurologic examination was normal in one patient. Six patients had microcephaly; eight manifested visual disturbances. Fourteen patients were mentally retarded. One had a pervasive developmental disorder. This study indicates neonatal hypoglycemia may cause posterior cerebral lesions, abnormal findings at neurologic examination, and symptomatic epilepsy, most frequently occipital lobe epilepsy, usually with a good prognosis, and occasionally epileptic encephalopathy with refractory seizures. MRI studies are essential to define the characteristics of cerebral lesions after neonatal hypoglycemia."}, {"id": "pubmed23n0071_4019", "title": "Positron emission tomography findings relevant to neurosurgery for epilepsy.", "score": 0.009174311926605505, "content": "Using the 2-[F-18]fluorodeoxyglucose method, 213 positron emission tomographic (PET) studies of local brain glucose metabolism (CMRglu) were performed in 124 patients with various forms of epilepsy. Interictal PET scans of primary epileptics typically showed some global metabolic depression and decreased functional activity of insular, basal and anterior temporal cortex. Epilepsia partialis continua Kozevnikov was characterized by hypo- or hyper-metabolism of perirolandic cortex. Tuberous sclerosis was distinguished by neocortical foci of significantly decreased glucose consumption. Even in the interictal resting state, with regard to sensitivity (greater than 90%) and accuracy of focus localization. PET was superior to other diagnostic methods in typical temporal lobe epilepsy. Averaging 23% below normal CMRglu, the majority of hypometabolic foci were found in mesial temporal structures. Improved distinction between the epileptogenic area and the surrounding tissue showing comparatively normal functional responsiveness, was achieved by psychophysical activation using emotional speech or continuous visual recognition during PET scanning. In patients who had undergone total cerebral hemispherectomy because of uncontrolled epilepsy, remarkable recruitment of association areas was observed on both motor and speech activation."}, {"id": "pubmed23n0262_1928", "title": "[Continuous partial epilepsy disclosing diabetes mellitus].", "score": 0.00909090909090909, "content": "Continuous partial epilepsy (CPE) is characterized by isolated, subintrant clonus focalized to a limited territory with critical focal electroencephalography in a concordant territory. CPE is observed in various cortical lesions but also in disorders of metabolism and notably decompensated diabetes mellitus. We report a case of CPE without focal lesion at MRI which revealed hyperglycaemia without ketosis. The 54-year old female patient was hospitalised for C.P.E.. Early CT and later MRI gave normal results. Biochemistry showed hyperglycaemia without kenoturia, acidosis or hyperosmolality. Insulin therapy rapidly brought glycaemia down to its normal level and the clonsism disappeared. Five months later, the patient had no other seizure and the EEG was normal. Epileptic seizures are frequent in hyperglycaemia without ketosis (25% of the cases) where they are mainly partial and motor (75 to 86% of the cases), rarely associated with a focal lesion (15% of the cases with CT scan). They are rare in patients with ketoacidosis. This apparent protective effect of ketoacidosis may be attributed to an increase of GABA bioavailability consecutive to acidosis. CPE is resistant to antiepileptic treatments. In CPE induced by hyperglycaemia without ketosis normalization of blood glucose level with insulin therapy is concomitant with a rapid cure of epilepsy. Thus glycaemia should be measured in all patients presenting with CPE, the aim being to diagnose hyperglycaemia without ketosis rapidly to avoid hyperosmolality and to prescribe an adequate treatment based exclusively on insulin and rehydration."}, {"id": "pubmed23n0408_11448", "title": "[Acute repetitive giratory seizures as a manifestation of nonketotic hyperglycemia].", "score": 0.009009009009009009, "content": "This 71 years old women without any history of epilepsy had diabetes mellitus. She was admitted for repetitive giratory seizures in relation with non-ketotic hyperglycaemia. The EEG showed right centro-parietal paroxysmal slow activity. Symptomatology disappeared within 48 hours after insulin therapy. One month later, she presented with a left hemiplegia in relation with a right sylvian infraction. The role of focal transitory ischaemia in connection with hyperglycaemia is discussed."}, {"id": "wiki20220301en253_30173", "title": "Electroencephalography", "score": 0.008928571428571428, "content": "EEG is most often used to diagnose epilepsy, which causes abnormalities in EEG readings. It is also used to diagnose sleep disorders, depth of anesthesia, coma, encephalopathies, and brain death. EEG used to be a first-line method of diagnosis for tumors, stroke and other focal brain disorders, but this use has decreased with the advent of high-resolution anatomical imaging techniques such as magnetic resonance imaging (MRI) and computed tomography (CT). Despite limited spatial resolution, EEG continues to be a valuable tool for research and diagnosis. It is one of the few mobile techniques available and offers millisecond-range temporal resolution which is not possible with CT, PET or MRI."}, {"id": "pubmed23n0398_10811", "title": "Focal and global cortical hypometabolism in patients with newly diagnosed infantile spasms.", "score": 0.008928571428571428, "content": "To evaluate the occurrence and prognostic importance of focal defects in cerebral cortical glucose metabolism in infants with newly diagnosed symptomatic and cryptogenic infantile spasms. Ten children with symptomatic and seven with cryptogenic infantile spasms underwent MRI, video-EEG, and PET using fluorodeoxyglucose as a tracer within 2 weeks of diagnosis. PET was repeated at 1 year of age in 12 patients. Cortical hypometabolic foci were found in 13 children (77%) with newly diagnosed spasms (six cryptogenic and seven symptomatic). The hypometabolic foci disappeared in seven of nine reexamined at age 1. The occipital foci disappeared in all (n = 6). Focal findings on PET correlated well with focal findings on video-EEG. There was no difference in quantitative cortical or subcortical glucose metabolic rate at the onset of infantile spasms between children with cryptogenic and symptomatic etiology of spasms. The glucose metabolic rate at the onset of spasms or focal lesions in glucose metabolism did not have prognostic value for seizure outcome. Infantile spasms are often associated with transient cortical, especially occipital, hypometabolic foci that are not necessarily associated with structural lesions and do not indicate a poor prognosis."}, {"id": "wiki20220301en518_5803", "title": "Occipital epilepsy", "score": 0.008866461398106968, "content": "Diagnosis Procedures for diagnosis of occipital epilepsy include hematology, biochemistry, screenings for metabolic disorders, DNA analysis, and most commonly, MRI. Electroencephalogram (EEG) is also used to detect abnormal brain waves and activity that is reflected as slow waves, or spikes on the recordings. For occipital epilepsy, commonly identified abnormalities on the EEG when a seizure is not occurring (inter-ictal) includes posterior lateralized slow waves, asymmetrical alpha and photic following, and unilateral occipital spikes. Idiopathic cases may appear mostly normal, with occipital spikes or paroxysms. Ictal EEG’s show occipital paroxysmal fast activity, spiking, or both, as well as brief occipital flattening. About one-third of occipital seizures do not show any obvious changes."}, {"id": "wiki20220301en075_60927", "title": "Reflex seizure", "score": 0.008849557522123894, "content": "The activation of the hyper-excitable areas of the brain are additionally regulated by facilitating factors that may increase the likelihood of eliciting a seizure. Most commonly these include fatigue, sleep deprivation, or stress. Facilitating factors are different for each individual. Due to the large variance between the different kinds of reflex epilepsies, the specific mechanism causing reflex seizures may vary. Diagnosis The diagnosis of reflex epilepsy usually includes a comprehensive medical and family history as well as a variety of tests. These tests may include a electroencephalography (EEG), magnetic resonance imaging (MRI), as well as genetic testing. The procedure for diagnosing epilepsy generally follows three steps: Determining if the seizure or seizure like event is truly an epileptic seizure. Determining what kind of seizure that someone has suffered from. Determining if this seizure or seizures are a part of a specific epilepsy syndrome or disease."}, {"id": "pubmed23n0136_4722", "title": "[Hypoglycemic states in the clinical picture of emergency neuropathology].", "score": 0.008849557522123894, "content": "The authors made a clinico-electrophysiological analysis of hypoglycemic conditions in 20 patients admitted to the clinic with acute cerebral symptomatology. They describe the following neurological symptoms developing in hypoglycemia: paroxysmal disturbances of consciousness, including epileptic seizures; pseudostrokes; pseudotumours; comatose states. The paper presents criteria of the differentiation between primary cerebral disorders and neuroglycopenic symptoms of hypoglycemic conditions. These criteria include the development of consciousness disturbances in morning and after long intervals between meals, excessive weakness for sweets, fluctuations of the degree of focal and general cerebral symptoms from mild lipothymic states to the development of pronounced focal neurological symptomatology, the presence of high amplitude slow wave activity at all EEG leads and the efficacy of the intravenous administration of glucose. It has been shown that hypoglycemia may manifest itself by various cerebral disorders and that the development of recurrent hypoglycemic states may be responsible for secondary metabolic encephalopathy with various focal neurological symptomatology."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": true, "char_ranges": [[596, 858]], "word_ranges": [[94, 129]], "text": "Among the X-linked immunodeficiencies is Wiskott-Aldrich syndrome, an entity described with an initial triad of symptoms consisting of bleeding (typical BUT absent in the case: heavy bleeding after circumcision, bloody diarrhea), recurrent infections and eczema."}, "2": {"exist": true, "char_ranges": [[968, 1187]], "word_ranges": [[144, 177]], "text": "The Hyper-IgE option lacks very characteristic clinical data such as bone alterations and skin lesions, which are not atopic dermatitis, since they follow a different pattern (papulopustular rash on the face and scalp)."}, "3": {"exist": true, "char_ranges": [[1188, 1432]], "word_ranges": [[177, 212]], "text": "The option of transient hypogammaglobulinemia of infancy and the common severe and variable combined immunodeficiency fails, among other features, the determination of immunoglobulins G and M, which are at the lower limit, but within normality."}, "4": {"exist": true, "char_ranges": [[367, 595]], "word_ranges": [[58, 94]], "text": "are describing an immunodeficiency that by the family-maternal history, seems to be X-linked, as several males have died of a similar clinical condition (the father of the patient contributed the Y chromosome, the mother the X)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "To correctly answer this question it should be emphasized that among the symptomatology that is exposed we find: - 2-year-old child. - ENT infections. - Pulmonary infections. - Hospital admissions. - PTI. - Family history of infections with deaths due to infections in males (maternal family). - Atopic Dermatitis. All these data, in addition to the analytical data, are describing an immunodeficiency that by the family-maternal history, seems to be X-linked, as several males have died of a similar clinical condition (the father of the patient contributed the Y chromosome, the mother the X). Among the X-linked immunodeficiencies is Wiskott-Aldrich syndrome, an entity described with an initial triad of symptoms consisting of bleeding (typical BUT absent in the case: heavy bleeding after circumcision, bloody diarrhea), recurrent infections and eczema. In addition to thrombocytopenia, they are at increased risk for autoimmune phenomena and lymphoid neoplasms. The Hyper-IgE option lacks very characteristic clinical data such as bone alterations and skin lesions, which are not atopic dermatitis, since they follow a different pattern (papulopustular rash on the face and scalp). The option of transient hypogammaglobulinemia of infancy and the common severe and variable combined immunodeficiency fails, among other features, the determination of immunoglobulins G and M, which are at the lower limit, but within normality.", "full_answer_no_ref": "To correctly answer this question it should be emphasized that among the symptomatology that is exposed we find: - 2-year-old child. - ENT infections. - Pulmonary infections. - Hospital admissions. - PTI. - Family history of infections with deaths due to infections in males (maternal family). - Atopic Dermatitis. All these data, in addition to the analytical data, are describing an immunodeficiency that by the family-maternal history, seems to be X-linked, as several males have died of a similar clinical condition (the father of the patient contributed the Y chromosome, the mother the X). Among the X-linked immunodeficiencies is Wiskott-Aldrich syndrome, an entity described with an initial triad of symptoms consisting of bleeding (typical BUT absent in the case: heavy bleeding after circumcision, bloody diarrhea), recurrent infections and eczema. In addition to thrombocytopenia, they are at increased risk for autoimmune phenomena and lymphoid neoplasms. The Hyper-IgE option lacks very characteristic clinical data such as bone alterations and skin lesions, which are not atopic dermatitis, since they follow a different pattern (papulopustular rash on the face and scalp). [HIDDEN] [HIDDEN]", "full_question": "2-year-old boy. His personal history includes 3 episodes of acute otitis media, 1 meningococcal meningitis and 2 pneumonias (one middle lobe and one left upper lobe). She has been admitted on 3 occasions for thrombopenic purpura (on three occasions antiplatelet antibodies were negative and bone marrow showed normal megakaryocytes). Several males of the maternal family had died in childhood due to infectious processes. Physical examination showed lesions typical of atopic dermatitis. The immunological study showed a slight decrease in T-lymphocyte subpopulations; elevated IgA and IgE; decreased IgM and IgG at the lower limit of normal. What is the most likely diagnosis?", "id": 106, "lang": "en", "options": {"1": "Wiskott-Aldrich syndrome.", "2": "Hyper IgE syndrome.", "3": "Transient hypogammaglobulinemia of childhood.", "4": "X-linked severe combined immunodeficiency.", "5": "Common variable immunodeficiency."}, "question_id_specific": 135, "type": "GENETICS AND IMMUNOLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0703_8952", "title": "[Wiskott-Aldrich syndrome].", "score": 0.01846494213750851, "content": "The Wiskott-Aldrich syndrome is a primary immunodeficiency characterized by congenital microthrombocytopenia, eczema and recurrent infections. This paper reports the case of a 3-year-6-month male patient, whose maternal uncle died at the age of 3 months due to fulminant sepsis from a pulmonary infection. The patient was a product of the first pregnancy, he was born at 27 weeks' gestation and weighed 1,400 g. As a neonate he was hospitalized during the first 2 months of life because of a low gastrointestinal bleeding, thrombocytopenia and severe infections. In the next 4 months and before coming to our hospital the infant was hospitalized 54 times. On admission he presented disseminated dermatosis, enlarged neck lymph nodes and psychomotor retardation. Laboratory studies revealed hemoglobin 8.1 g/dL, platelets 31,000/uL, mean platelet volume 5.6 fL, IgM 39.3 mg/dL, IgA 67 mg/dL, IgG 1,380 mg/dL. On several occasions he received globular packages and platelet concentrates. The infusion of immunoglobulin G was started every 21 days. Bone marrow transplantation was delayed due to the complications that merited 13 hospitalizations and severe thrombocytopenia, low gastrointestinal bleeding, septic arthritis, infectious gastroenteritis, chronic suppurative otitis media and severe folliculitis. At the age of 4 years BMT of cord was performed, and 26 days after transplantation he presented septic shock and died. The prognosis of bone marrow transplantation in Wiskott-Aldrich syndrome and in other primary immunodeficiencies depends on the promptness of its performance at early stages in life. It is important that the first contact physicians be aware of the primary immunodeficiency signs and symptoms."}, {"id": "pubmed23n0423_22855", "title": "A case of IgG subclass deficiency with the initial presentation of transient hypogammaimmuno-globulinemia of infancy and a review of IgG subclass deficiencies.", "score": 0.018255746411086217, "content": "Primary immunodeficiency diseases are not common in children. The possibility of an immunological defect should be considered in any individual with repeated infections. A definite diagnosis for immodeficiency is sometimes difficult to achieve because of overlapping clinical manifestations. Immunoglobulin subclass deficiency is an immunological deficiency disease with which, one or more IgG subclasses are deficient. T cell immunity is normal. Patients may develop recurrent bacterial and respiratory infections or could remain asymptomatic. The authors report a case of immunoglobulin G subclass deficiency presenting initially as transient hypogammaglobulinemia of infancy. A 2 month-old boy presented to Siriraj Hospital with a history of chronic protracted diarrhea, disseminated scabies and sepsis. On presentation, he had generalized scaly and maculopapular rash with no palpable lymph nodes. CBC revealed WBC 22,100 cells/cm3 with PMN 42 per cent, lymphocytes 38 per cent, Eosinophils 4 per cent, Basophil 2 per cent and platelets 254,000/cm3. The immunoglobulin levels were as follows: IgG 181 mg/dl, IgA &lt; 6.6 mg/dl, IgM 26.3 mg/dl. Lymphocyte enumerations revealed CD4 of 2,433 cells/cm3 (N 1,460-5,160); CD8 4,682 cells/cm3 (N 650-2,450); CD19 1,588 cell/cm3 (N 500-1,500); CD16 230 cell/cm3 (N 573 +/- 264). The initial diagnosis was X-linked agammaglobulinemia vs common variable immunodeficiency disease. His diarrhea and five courses of sepsis responded well to antibiotics administration and courses of intravenous immunoglobulin (IVIG) replacement. His through IgG became normal at 2 years of age (after 12 months of IVIG). IVIG was stopped and the diagnosis was changed to transient hypogammaglobulinemia of infancy (THI). Nevertheless, during his 4 month follow-up he developed recurrent sinopulmonary infections (i.e, otitis media and pneumonia). Repeated immunoglobulin profile showed IgG 1,200 mg/dl, IgA 135 mg/dl, IgM 26 mg/dl, IgG subclass were IgG, 1,030 mg/dl (N 280-830), IgG2 30 mg/dl (N 40-2,400), IgG3 22 mg/dl (N 6-130), IgG4 3 mg/dl (N 3-120). A diagnosis of IgG2 subclass deficiency presenting early as transient hypogammaglobulinemia of infancy was then made. Treatment with monthly IVIG was reinitiated and the patient is currently doing well. The authors present a case of IgG subclass deficiency presenting as transient hypogammaglbulinemia of infancy. Follow-up of the immune profile and clinical manifestation is necessary for a definite diagnosis."}, {"id": "pubmed23n0521_19925", "title": "De novo mutation causing X-linked hyper-IgM syndrome: a family study in Taiwan.", "score": 0.017767295597484276, "content": "X-linked hyper-IgM syndrome (XHIM) is a rare primary immunodeficiency disorder caused by mutations of the gene encoding the CD40 ligand (CD40L). It is characterized by recurrent infections with markedly decreased serum IgG, IgA and IgE levels but normal or elevated IgM levels. We report the clinical manifestations and complete immune studies in the first family with molecularly proven XHIM in Taiwan. A 5-month-old boy presented with rapidly progressive pneumonia which responded poorly to antibiotics. High levels of IgM and very low levels of IgG, IgA, and IgE were noted in his plasma specimen: IgM, 128 mg/dl; IgG, 18 mg/dl; IgA, 4 mg/dl); IgE, 1 IU/ml. Whole blood flow cytometry when he was 21 months old showed that only a small percentage (0.48%) of his in vitro-activated CD4+ T cells expressed CD40L. When he was 3 years old, repeated flow cytometry showed essentially the same result (0.4%), compared with his father's CD40L expression of over 85%. The patient's mother had moderately decreased CD40L expression (74.4%). Hyper-IgM syndrome was confirmed by CD40L mutation analysis in the boy, which revealed a Lys 96 stop (nucleotide A307T) in exon 2 of CD40L, with a truncated protein resulting in the loss of the entire TNF domain. His mother was a carrier and apparently the individual in whom the mutation originated. Eleven other family members, including the patient's father, sister, and grandmother, and the mother's sisters and their children, all had normal results on CD40L mutation analysis. The patient has remained without significant bacterial infection on a regimen of monthly IVIG infusion and oral trimethoprim-sulfamethoxazole for Pneumocystis carinii pneumonia (PCP) prophylaxis, although he has had recurrent oral ulcers and neutropenia. Bone marrow transplantation is planned."}, {"id": "pubmed23n0525_4354", "title": "Wiskott-Aldrich syndrome complicated by an atypical lymphoproliferative disorder: a case report.", "score": 0.01755601755601756, "content": "Wiskott-Aldrich syndrome (WAS) is an X-linked syndrome consisting of eczema, recurrent pyogenic infection, and thrombocytopenia with decreased platelet volume. Immunologic studies reveal normal immunoglobulin G (IgG), decreased IgM, elevated IgA and IgE levels, and decreased T-cell function. Patients with WAS often have increased susceptibility to lymphoproliferative disorders (LPDs). We report a 3-year-old boy who had persistent thrombocytopenia with bleeding, recurrent infections, and chronic eczema with frequent skin infections since birth. A blood smear revealed small platelets (50% of normal size). Immunologic studies showed normal IgG (1880 mg/dL), decreased IgM (76 mg/dL) and increased IgA (228 mg/dL) and IgE (14,282 IU/mL) levels. The relative proportions of immune cells were CD2 52.2%, CD3 41.1%, CD4 23.4%, CD8 16.8%, CD19 8.0%, CD57 7.7% and active T cells 14.6%. T-cell dysfunction was detected on the multitest for cell-mediated immunity. The WAS diagnosis was confirmed by mutation analysis which demonstrated a 4-base pair deletion in WAS protein gene exon 1. His thrombocytopenia was uncontrolled despite intravenous immunoglobulin infusions, so splenectomy was performed. The platelet count then rose to about 60,000 to 80,000/microL. However, about 2 weeks after splenectomy, he developed generalized lymphadenopathy and lymphoma was misdiagnosed based on lymph node biopsy at another hospital where he was admitted for urgent care. However, our analysis of his lymph node pathology led to the diagnosis of atypical LPD (ALPD). The lymphadenopathy regressed spontaneously 1 month later without chemotherapy. Early and correct diagnosis of WAS complicated with ALPD is important to avoid unnecessary chemotherapy."}, {"id": "pubmed23n0703_8951", "title": "[Satisfactory evolution of a patient diagnosed in childhood with Bruton's disease].", "score": 0.017043847241867045, "content": "Bruton's agammaglobulinemia is a primary immunodeficiency with a disease onset during the first months of age, when the maternal serum immunoglobulin levels decrease. It is characterized by recurrent infections and agammaglobulinemia. We report the case of a 6-year-old male patient with third-degree consanguinity, product of a third pregnancy and complete immunization scheme. He had a history of oral candidiasis at the age of 3 months, chicken pox at the age of 7 months, and two episodes of complicated bronchopneumonia at the age of 1 year and 6 years. He was admitted to the hospital because of fever and cough. Examination of the chest showed rales and right basilar hypoventilation, and a blood cell count revealed leukocytosis and neutrophilia. The diagnosis of pneumonia was made. He was treated with IV antibiotics. Serum immunoglobulins were reported to be low (IgM 55 mg/dL, IgA 0.9 mg/dL, and IgG 199 mg/dL). With these findings the clinical diagnosis of X-linked agammaglobulinemia (ALX) was concluded. A molecular test was performed fining a BTK gene confirming the diagnosis of Bruton's disease. Therapy with intravenous IgG was started every 21 days. During his evolution, he presented three episodes of rhinosinusitis, one of suppurative otitis media, and four events of pneumonia that required 37 days of hospitalization. After hospital discharge, the patient was free of infections and he returned to his daily activities. In cases of recurrent and severe respiratory infections in children, we must consider primary immunodeficiency disease in the differential diagnosis, mainly antibiotic deficiency. Early diagnosis and treatment improves the survival and quality of life in these patients."}, {"id": "pubmed23n0393_18251", "title": "Recurrent pneumonia as warning manifestation for suspecting primary immunodeficiencies in children.", "score": 0.017009719839908517, "content": "Two hundred and eight children with recurrent pneumonia were studied over a 5-year period. Among these patients we found 10 cases with primary immunodeficiency disease: 6 cases of IgA deficiency, 1 case of X-linked agammaglobulinemia, 1 case of common variable immunodeficiency, 1 case of hyper IgM syndrome, and 1 case of Wiskott-Aldrich syndrome. This study describes the clinical features of these cases and assesses the usefulness of our immunodeficiency screening protocol. In this group 6 were males; the mean age at first episode of pneumonia was 3 years (range 3 months to 18 years), and the age of diagnosis ranged between 10 months and 19 years. The average number of episodes of pneumonia in each patient was 5 (range 2 to 12), and the number of hospitalizations ranged up to 13. The etiologic agents isolated from this recurrent pneumonia were S. pneumoniae, Moraxella, adenovirus, respiratory syncytial virus, and influenza B virus. Intravenous immunoglobulin was used in four cases. Two patients had chronic pulmonary damage with bronchiectasis and interstitial pneumonia. Only one patient died (Wiskott-Aldrich syndrome) during the follow-up from an intracranial hemorrhage. We found that the screening protocol applied to patients with recurrent pneumonia is a useful tool for ruling out the primary immunodeficiency disorders."}, {"id": "pubmed23n0027_424", "title": "Serum IgD and IgE concentrations in immunodeficiency diseases.", "score": 0.015323891339679591, "content": "Concentrations of IgD and IgE were measured in sera from 165 patients with well-defined immunodeficiency in an effort to find information possibly relevant to the roles of antibodies of these classes in host defense. Values for both immunoglobulins were generally quite low in patients who had marked deficiencies of all three major immunoglobulins, although occasional normal or high normal values for IgD were seen in hypogammaglobulinemic patients. Group mean IgD concentrations were also depressed in patients with Wiskott-Aldrich syndrome and in those with selective IgA deficiency; IgE concentrations were depressed in patients with X-linked immunodeficiency with hyper-IgM and in those with ataxia telangiectasia. IgD and IgE were both significantly elevated in patients with extreme hyperimmunoglobulinemia E and undue susceptibility to infection and in a patient with the Nezelof syndrome; none of these patients had histories suggestive of atopy. In addition, the mean IgE concentration was significantly elevated in patients with selective IgA deficiency, many of whom were atopic, and in those with the Wiskott-Aldrich syndrome. The highest IgD concentration (163 mg/100 ml) was found in serum from a boy with variable immunodeficiency who had a lifelong history of severe recurrent pharyngeal infections, primarily streptococcal in etiology. Recurrent staphylococcal infection was a feature common to many but not all patients with elevated serum IgE concentration. These data may prove useful in the future delineation of biologic roles for antibodies in these two immunoglobulin classes."}, {"id": "pubmed23n0079_3796", "title": "[Hypogammaglobulinemia G and A with hypergammaglobulinemia M. Apropos of 12 cases].", "score": 0.015151515151515152, "content": "Hyper IgM with low IgG and IgA is a rare humoral immunodeficiency. We presently report 12 new observations which have been clinically and immunologically studied. On one occasion the syndrome was found to be associated with congenital rubella. Since 10/12 children were male, X-linked inheritance is suggested which has been confirmed in 2 cases. In most cases (9/12), the first infections occurred within the first year of life. The syndrome is causing upper and lower respiratory tract infections due to bacteria, as well as gut infections. Lymphoid organ hyperplasia has been noted in 11/12 patients. Polyclonal hyper IgM serum contrasts with low or absent IgG, IgA and IgE. In some instances, some IgM antibody response was detected. A dysfunction of cellular immunity was not detected. Autoimmunity was detected in 3 patients. Finally, transient neutropenia occurred in 50% of the patients. Intravenous immunoglobulin G substitution treatment resulted in a significant reduction in the occurrence of infections as well as in normalization of growth rate. Immunoglobulin infusion also frequently induced correction of hyper IgM and neutropenia."}, {"id": "pubmed23n0517_20443", "title": "Hyper-IgM syndrome: report of one case.", "score": 0.01498652896273013, "content": "The hyper-IgM syndrome (HIM) is a rare primary immunodeficiency disorder caused by defects in the CD40 ligand (CD40L)/CD40-signaling pathway. It is characterized by recurrent infections with markedly decreased IgG, IgA and IgE levels but normal or elevated serum IgM levels. A 5-month-old boy presented with rapidly progressive pneumonia which responded poorly to antibiotics. High levels of IgM and very low levels of IgG, IgE and IgA were noted in his plasma specimen (IgM, 128 mg/dl; IgG, 18 mg/dl; IgE, 1 IU/ml; IgA, 4 mg/dl). The relative proportions of immune cells were CD3 24.6%, CD4 10.3%, CD8 2.2%, CD19 30.2%, CD57 1.0% and active T cells 1.1%. After IVIG treatment, the pneumonia improved. Repeat assessment at the age of 15 months showed IgM decreased to the normal range (32 mg/dl). Whole blood flow cytometry assay for CD40L expression confirmed the diagnosis of hyper-lgM syndrome when he was 21 months old. Only a small percentage (0.48%) of the patient's in vitro activated CD4+ T cells expressed CD40L, compared with 33.54% from a healthy control. The patient's father, mother and sister all had a normal CD40L expression activation patterns (43.52%, 40.78%, 34.11%, respectively). On a regimen of monthly IVIG infusion and oral trimethoprim-sulfamethoxazole for Pneumocystis carinii pneumonia (PCP) prophylaxis, the patient has had no recurrent infections."}, {"id": "pubmed23n0501_10269", "title": "[Clinical features of X-linked agammaglobulinemia: analysis of 8 cases].", "score": 0.014412216499503028, "content": "X-linked agammaglobulinemia (XLA), caused by mutations in Bruton's tyrosine kinase (BTK), is a common form of inherited antibody deficiency. There were very few case reports of this disease that were diagnosed only based on clinical findings in China. The purpose of this study was to evaluate the clinical features of 8 Chinese cases with XLA with BTK defect which were confirmed by flow cytometry and/or gene analysis. Based on clinical findings, 8 suspected XLA patients were confirmed by detecting the expression of BTK by flow cytometry and/or gene analysis of BTK. The history and thorough physical examination and routine immunological evaluation of 8 cases were collected and reviewed. The age of onset of all the 8 male patients were from 3 months to 3 years. The mean age at diagnosis was 6 years. Recurrent upper respiratory infection and pneumonia with fever were seen in all the patients. Nasopharynx infection was mainly contributed to upper respiratory infection. Very few or no otitis (1/8) and sinusitis (0/8) were involved. Polyarthritis without evidence of infection was common (3/8). Chronic diarrhea was documented during the first 2 years after the onset of the disease in 2 cases. Two of the patients suffered from meningitis one time each. Skin infection was not serious in two patients. Osteomyelitis occurred in one case, which occurred secondary to a trauma. One case had poliomyelitis-like disease that was considered to be related to polio vaccine. Only two cases had unconfirmed maternal family history of XLA. The prominent signs at diagnosis were dystrophia, growth and developmental retardation and markedly decreased or absent tonsils and lymph nodes. Concentration of all classes of serum immunoglobulins (Igs) and the number of B cells in the peripheral circulation were dramatically decreased. The ratio of CD4/CD8 in most of the patients (6/8) was markedly inverse. The age at diagnosis of this reported group was older. Clinical symptoms displayed recurrent upper respiratory infection (nasopharynx infection but rare or no otitis or sinusitis) and pneumonia; polyarthritis was common. There were no confirmed family history of XLA. Most of the patients showed inverse ratios of CD4/CD8, the reason and potential significance are unclear."}, {"id": "wiki20220301en037_31604", "title": "Wiskott–Aldrich syndrome", "score": 0.014056026829749458, "content": "Signs and symptoms WAS occurs most often in males due to its X-linked recessive pattern of inheritance, affecting between 1 to 10 males per million. The first signs are usually petechiae and bruising, resulting from a low platelet count (i.e. thrombocytopenia). Spontaneous nose bleeds and bloody diarrhea are also common and eczema typically develops within the first month of life. Recurrent bacterial infections typically develop by three months of age. The majority of children with WAS develop at least one autoimmune disorder, and cancers (mainly lymphoma and leukemia) develop in up to a third of patients. Immunoglobulin M (IgM) levels are reduced, IgA and IgE are elevated, and IgG levels can be normal, reduced, or elevated. In addition to thrombocytopenia, WAS patients have abnormally small platelets (i.e. microthrombocytes) and ~30% also have elevated eosinophil counts (i.e. eosinophilia)."}, {"id": "wiki20220301en037_31610", "title": "Wiskott–Aldrich syndrome", "score": 0.01361875637104995, "content": "Diagnosis The diagnosis can be made on the basis of clinical findings, the peripheral blood smear, and low immunoglobulin levels. Typically, IgM levels are low, IgA levels are elevated, and IgE levels may be elevated; paraproteins are occasionally observed. Skin immunologic testing (allergy testing) may reveal hyposensitivity. Individuals with Wiskott–Aldrich syndrome however are at higher risk for severe food allergies. Not all patients have a positive family history of the disorder; new mutations do occur. Often, leukemia may be suspected on the basis of low platelets and infections, and bone marrow biopsy may be performed. Decreased levels of WASp are typically observed. The current gold standard for diagnosis is DNA sequence analysis, which can detect WAS and the related disorders XLT and XLN in 95% of patients and carriers."}, {"id": "wiki20220301en559_4909", "title": "List of primary immunodeficiencies", "score": 0.013536953242835596, "content": "Normal numbers of B cells with decreased IgG and IgA and increased IgM: Hyper-IgM syndromes Normal numbers of B cells with isotype or light chain deficiencies: heavy chain deletions, kappa chain deficiency, isolated IgG subclass deficiency, IgA with IgG subclass deficiency, selective immunoglobulin A deficiency Specific antibody deficiency to specific antigens with normal B cell and normal Ig concentrations Transient hypogammaglobulinemia of infancy (THI)"}, {"id": "pubmed23n0059_21268", "title": "Early bone marrow transplantation in an infant with Wiskott-Aldrich syndrome.", "score": 0.013450710519259987, "content": "The Wiskott-Aldrich Syndrome (WAS) is a rare X-linked immunohematological disorder characterized by eczema, profound thrombocytopenia, and progressive immunodeficiency. Severe hemorrhage, overwhelming sepsis, or lymphoreticular malignancy usually cause death in childhood. Recently, bone marrow transplantation (BMT) has been curative in some well-established cases, but there is no general agreement about the place of BMT in infants with WAS before the development of significant immunological abnormalities. We describe the successful use of early histocompatible BMT in a 10-month-old infant in whom WAS was diagnosed on the basis of eczema, thrombocytopenia, small platelets, and raised serum immunoglobulin A (Ig) and IgE, but before the development of immunodeficiency as evidenced clinically by recurrent infections, or immunologically by low serum IgM or consistently abnormal lymphocyte responses to mitogens. After an unstable period for several weeks posttransplantation when he developed marked hepatomegaly and severe interstitial pneumonitis, he made a good recovery. His eczema and thrombocytopenia resolved and he has shown no clinical or laboratory evidence of immunodeficiency. It is now over 2 years since his BMT. Because of the poor prognosis of WAS, where a histocompatible donor is available, BMT at the earliest opportunity, despite the inherent risks of such a procedure, may be the best option for an infant with WAS."}, {"id": "pubmed23n0858_16600", "title": "[Clinical features and genotype analysis of 132 patients with Wiskott-Aldrich syndrome].", "score": 0.012908061292471684, "content": "To investigate the clinical and immunological laboratory features, gene mutations, treatment and prognosis in children with Wiskott-Aldrich syndrome (WAS). The clinical, laboratory characteristics, treatment and prognosis of 132 children with WAS, who visited Children's Hospital of Chongqing Medical University from April 2000 to June 2015, were analyzed retrospectively. All patients were male. The median age of disease onset was 15 days and the median age at diagnosis was 10 months. Of the 132 cases, 112 had classic WAS, 20 had X-linked thrombocytopenia (XLT). The median platelet count was 23×10(9)/L. All cases had the clinical characteristics of WAS including bleeding, eczema, and being susceptible to infection. The initial symptoms include hemorrhage (75.0%) and eczema (16.7%). Twenty-one cases had autoimmune diseases and one patient had leukemia. WAS protein (WASP) expression in 115 cases were measured by flow cytometry, 88 cases were negative, in 12 cases WASP decreased, in 5 cases it was normal, 10 cases had bimodal distribution. Eighty-one kinds of mutations were found in 122 families, including eight kinds of hot-spot mutations, which were 290 C&gt; N / 291G&gt; N (R86C / H / L), 665 C&gt; T (R211X), 155 C&gt; T (R41X), 168 C&gt; T (T45 M), IVS1+ 1 g&gt; t/ a, IVS6 + 5 g&gt; a, IVS8 + 1 g&gt; a and IVS8 + 1to + 6del gtga. Meantime, 29 kinds of novel mutations were found, which were 321T&gt;C, 415C&gt;A, 471C&gt;T, 102-105delC, 521 del C, 1330 del A, IVS2-2 a&gt;c, 168 C&gt;A/1412 C&gt; T, exon1-2 del/1412 C&gt;T, and so on. The proportion of CD3(+) T cells (31.3%), helper T cells (37.3%) and cytotoxic T cells (38.6%) in the peripheral blood declined. The serum levels of IgG (51.1%), IgA (43.3%) and IgE (40.0%) increased, IgM (25.6%) decreased. Of the 132 cases, 72 remain survived, of whom 36 cases received hematopoietic stem cell transplantation (HSCT), 14 patients with classic WAS received intravenous immunoglobulin (IVIG) therapy. With regular IVIG therapy, the frequency of infections was reduced and the patients' symptoms were improved. The clinical characteristics of Wiskott-Aldrich syndrome were early age of onset, microthrombocytopenia, eczema and recurrent infections. The proportion of T lymphocyte declined, the serum levels of IgG, IgA, and IgE increased, and level of IgM decreased in a part of patients. The detection of WAS gene mutation and WAS protein detection was the key diagnostic methods. Regular IVIG can gain more time for children who will receive HSCT and improve their quality of life."}, {"id": "article-17681_26", "title": "Antibody Deficiency Disorder -- Differential Diagnosis", "score": 0.012896825396825396, "content": "The most important differential diagnosis includes the following: X-linked agammaglobulinemia characterizes by recurrent bacterial infections in boys, and genetic studies may reveal the presence of Bruton tyrosine kinase (BTK) mutations. Transient hypogammaglobulinemia of newborns presents in newborns above the age of 4 months and characterizes by recurrent pneumonia, meningitis, otitis media, and other problems that resemble Bruton disease. It is a physiological defect in the immune system caused by maternal IgG disappearance and corrected soon but requires treatment. In super-IgM syndrome, recurrent bacterial infections occur, but the cause of this illness is a mutation in the gene encoding for CD40 on T lymphocytes that causes a failure in T and B lymphocyte cooperation. Common variable immunodeficiency presents with recurrent bacterial infections, including sinopulmonary problems but later in life (second-fourth decade), and the diagnosis is made once all causes of immunodeficiency have been ruled out. [6] [7] [21] [33]"}, {"id": "wiki20220301en067_45376", "title": "Hypogammaglobulinemia", "score": 0.012797933544675354, "content": "of primary immunodeficiency (PID). These different forms can affect different parts of the immune system, including immunoglobulin production. Primary immunodeficiencies usually have a delay of several years between initial clinical presentation and diagnosis. Some primary immune deficiencies include ataxia-telangiectasia (A-T), autosomal recessive agammaglobulinemia (ARA), common variable immunodeficiency (CVID), hyper-IgM syndromes, IgG subclass deficiency, isolated non-IgG immunoglobulin deficiencies, severe combined immunodeficiency (SCID), specific antibody deficiency (SAD), Wiskott-Aldrich syndrome, or X-linked agammaglobulinemia. CVID is the most common form of primary immunodeficiency. SCID is considered a medical emergency and suspected cases require immediate specialist center referral for diagnosis and treatment. It is more often that hypogammaglobulinemia develops as a result of another condition, which are called secondary or acquired immune deficiencies. These"}, {"id": "wiki20220301en062_59354", "title": "X-linked agammaglobulinemia", "score": 0.012641165755919854, "content": "Signs and symptoms Affects males 50% of the time if mother is a carrier for the gene. Children are generally asymptomatic until 6–9 months of age when maternal IgG decreases. Present with recurrent infections with Streptococcus pneumoniae, Haemophilus influenzae, Mycoplasma pneumoniae, hepatitis virus, and enterovirus CNS infections. Examination shows lymphoid hypoplasia (tonsils and adenoids, no splenomegaly or lymphadenopathy). There is significant decrease in all immunoglobulins. Genetics Most antibodies are gamma globulins. Antibodies are made mainly by plasma cells, which are daughter cells of the B cell line. The Btk enzyme plays an essential role in the maturation of B cells in the bone marrow, and when mutated, immature pro-B lymphocytes are unable to develop into pre-B lymphocytes, which normally develop into mature (naive) B cells that leave the bone marrow into the blood stream."}, {"id": "pubmed23n0626_19418", "title": "[Hyper-IgM syndrome in a boy with recurrent pneumonia and hepatosplenomegaly].", "score": 0.012531036623215395, "content": "We present a boy diagnosed at age 14 years with hyper-immunoglobulin (Ig) M syndrome, a congenital immunodeficiency characterized by reduced plasma concentrations of IgA, IgE and IgG, with normal or elevated concentrations of IgM. This syndrome is caused by a defect of CD40 ligand (CD40L) on T-helper lymphocytes, impeding the \"second signal\" during activation of B lymphocytes and interactions of T cells with dendritic cells and macrophages, resulting in the absence of secondary immune response (class switching, affinity maturation, immune memory), as well as responses to T-dependent antigens, with an impairment of cellular immunity. The history of the presented patient was dominated by frequent lower respiratory infections and failure to thrive. Physical examination demonstrated severe hepatosplenomegaly. The suspicion of hyper-IgM syndrome was raised by low plasma IgA (0.36 g/l) with high plasma IgM (35.5 g/l), while the concentration of IgG was within the normal range (12.1 g/l). The diagnosis was confirmed by flow cytometry, which demonstrated the absence of expression of CD40L on lymphocytes following stimulation by phorbolmyristylacetate and calcium ionophore. Since the time of diagnosis, intravenous immunoglobulin therapy has led to catch-up growth, recession of hepatosplenomegaly and reduction in the frequency of respiratory infections. Our report emphasizes the importance for the primary healthcare paediatrician to be well informed about the clinical presentation and pathogenesis of hyper-IgM syndrome, in order to provide early detection and increase the likelihood of success in treating this rare immunodeficiency. To the best of our knowledge, this is the first case of hyper-IgM syndrome reported in the Republic of Serbia."}, {"id": "wiki20220301en218_4190", "title": "List of skin conditions", "score": 0.012372812372812373, "content": "Bare lymphocyte syndrome Chronic granulomatous disease (Bridges–Good syndrome, chronic granulomatous disorder, Quie syndrome) Common variable immunodeficiency (acquired hypogammaglobulinemia) Complement deficiency DiGeorge syndrome (DiGeorge anomaly, thymic hypoplasia) Graft-versus-host disease Griscelli syndrome Hyper-IgE syndrome (Buckley syndrome, Job syndrome) Immunodeficiency with hyper-IgM Immunodeficiency–centromeric instability–facial anomalies syndrome (ICF syndrome) Isolated IgA deficiency Isolated primary IgM deficiency Janus kinase 3 deficiency Leukocyte adhesion molecule deficiency LIG4 syndrome Myeloperoxidase deficiency Neutrophil immunodeficiency syndrome Nezelof syndrome (thymic dysplasia with normal immunoglobulins) Omenn syndrome Purine nucleoside phosphorylase deficiency Severe combined immunodeficiency (alymphocytosis, Glanzmann–Riniker syndrome, severe mixed immunodeficiency syndrome, thymic alymphoplasia) Shwachman–Bodian–Diamond syndrome"}, {"id": "wiki20220301en559_4908", "title": "List of primary immunodeficiencies", "score": 0.012202503450112583, "content": "Predominantly antibody deficiencies In primary antibody deficiencies, one or more isotypes of immunoglobulin are decreased or don't function properly. These proteins, generated by plasma cells, normally bind to pathogens, targeting them for destruction. Absent B cells with a resultant severe reduction of all types of antibody: X-linked agammaglobulinemia (btk deficiency, or Bruton's agammaglobulinemia), μ-Heavy chain deficiency, l 5 deficiency, Igα deficiency, BLNK deficiency, thymoma with immunodeficiency B cells low but present or normal, but with reduction in 2 or more isotypes (usually IgG & IgA, sometimes IgM): common variable immunodeficiency (CVID), CD19 deficiency, TACI (TNFRSF13B) deficiency, BAFF receptor deficiency. Normal numbers of B cells with decreased IgG and IgA and increased IgM: Hyper-IgM syndromes"}, {"id": "wiki20220301en071_14447", "title": "Common variable immunodeficiency", "score": 0.012178655582325063, "content": "Diagnosis According to a European registry study, the mean age at onset of symptoms was 26.3 years old. As per the criteria laid out by ESID (European Society for Immunodeficiencies) and PAGID (Pan-American Group for Immunodeficiency), CVID is diagnosed if: the person presents with a marked decrease of serum IgG levels (<4.5 g/L) and a marked decrease below the lower limit of normal for age in at least one of the isotypes IgM or IgA; the person is four years of age or older; the person lacks antibody immune response to protein antigens or immunization. Diagnosis is chiefly by exclusion, i.e. alternative causes of hypogammaglobulinemia, such as X-linked agammaglobulinemia, must be excluded before a diagnosis of CVID can be made."}, {"id": "wiki20220301en225_1951", "title": "Humoral immune deficiency", "score": 0.012158326442125025, "content": "Absent B cells with a resultant severe reduction of all types of antibody: X-linked agammaglobulinemia (btk deficiency, or Bruton's agammaglobulinemia), μ-Heavy chain deficiency, l 5 deficiency, Igα deficiency, BLNK deficiency, thymoma with immunodeficiency B cells low but present, but with reduction in 2 or more isotypes (usually IgG & IgA, sometimes IgM): common variable immunodeficiency (CVID), ICOS deficiency, CD19 deficiency, TACI (TNFRSF13B) deficiency, BAFF receptor deficiency. Normal numbers of B cells with decreased IgG and IgA and increased IgM: Hyper-IgM syndromes Normal numbers of B cells with isotype or light chain deficiencies: heavy chain deletions, kappa chain deficiency, isolated IgG subclass deficiency, IgA with IgG subsclass deficiency, selective immunoglobulin A deficiency Transient hypogammaglobulinemia of infancy (THI)"}, {"id": "article-23400_15", "title": "Biochemistry, Immunoglobulin M -- Clinical Significance -- Selective IgM Deficiency", "score": 0.012094188960774029, "content": "Selective IgM deficiency (SIGMD) is a rare disorder with fewer than 300 cases reported. SIGMD is associated with an isolated deficiency in IgM in the presence of normal levels of other immunoglobulins such as IgG and IgA and normal levels of T cells and other leukocytes. [12] Individuals with SIGMD may be asymptomatic, or they may suffer from recurring infections from encapsulated bacteria (e.g., S. pneumoniae and H. influenzae ) in addition to viral infections. Additionally, SIGMD can be associated with malignancy, autoimmunity, or allergy. SIGMD may occur as a secondary effect of another disease, such as malignancy or bacteremia. Yet, primary causes of SIGMD have also been described, as some are associated with deletions on chromosome 22, for example. [12] The diagnosis of SIGMD is one of exclusion. Other diseases that result in low levels of multiple isotypes must be excluded, such as common variable immunodeficiency or X-linked agammaglobulinemia, which will likely result in reduced levels of several antibody isotypes. Conversely, Wiskott-Aldrich syndrome is often associated with low IgM yet elevated levels of IgG and IgA. Cold Agglutinin Disease"}, {"id": "wiki20220301en218_4191", "title": "List of skin conditions", "score": 0.011810415808974887, "content": "Severe combined immunodeficiency (alymphocytosis, Glanzmann–Riniker syndrome, severe mixed immunodeficiency syndrome, thymic alymphoplasia) Shwachman–Bodian–Diamond syndrome Thymoma with immunodeficiency (Good syndrome) Transient hypogammaglobulinemia of infancy Warts–hypogammaglobulinemia–infections–myelokathexis syndrome (WHIM syndrome) Wiskott–Aldrich syndrome X-linked agammaglobulinemia (Bruton syndrome, sex-linked agammaglobulinemia) X-linked hyper-IgM syndrome X-linked hypogammaglobulinemia X-linked lymphoproliferative disease (Duncan's disease) X-linked neutropenia"}, {"id": "pubmed23n0329_9114", "title": "Severe combined immunodeficiency with B-lymphocytes (T-B+SCID): report of two cases.", "score": 0.011628300037183518, "content": "Severe combined immunodeficiency (SCID) is a rare pediatric medical emergency in Taiwan. The early diagnosis of infants with SCID is very important because it can save the life of these critical infants. The essential clues important for early diagnosis of SCID patients include positive family history of early infant death, paucity of tonsil and lymphoid tissue, cutaneous fungal infection and lymphopenia. Severe combined immunodeficiency is a heterogeneous group of inherited disorders characterized by the failure of both cellular and humoral immunity. It can be categorized into SCID with B-lymphocytes predominant (T-B+SCID) and SCID with paucity of B-lymphocytes (T-B-SCID), according to the number of B-lymphocytes in the patient's peripheral circulation. We report two male infants with T-B+SCID who had been suffering from severe pulmonary distress with persistent O2 desaturation when they were transferred to our pediatric intensive care unit. Tracing back these infant's family histories, it was discovered that both of them had an elder brother who had died to overwhelming infection within the first year of life, and Pneumocystis carinii pneumonitis (PCP) was confirmed in the elder brother of case 2. After hospitalization, the immune condition of these two infants were evaluated which showed a decrease in T-cell and NK cell number, an increase in B-cell number, and decreased serum levels of all the Igs except IgM, which was elevated in case 1. These were the diagnostic immunological findings for T-B+SCID, which included X-linked SCID and Jak-3-deficient SCID. During hospitalization, severe mucocutaneous candidiasis and PCP were noted and confirmed in case 1 and PCP was highly suspected in case 2. Bone marrow transplantation, the only curable treatment for T-B+SCID at present, could not be performed in these two patients because of their grave clinical condition. Both of them expired due to their progressively downhill pulmonary conditions."}, {"id": "pubmed23n0886_14692", "title": "[Recurrent fever, hepatosplenomegaly and eosinophilia in a boy].", "score": 0.011404639175257733, "content": "A 2-year-old boy was admitted into the hospital because of cough and fever. Lymph node tuberculosis was noted when he was 2 months old and he was subsequently hospitalized several times because of cough and fever. After hospitalization the laboratory examination showed an increased eosinophia level in blood. The immune function tests shows decreased levels of IgG, IgA, and IgM. The patient had no response to anti-tuberculosis, anti-bacterial, and anti-fungal treatment, resulting in recurrent fever and progressive enlargement of the liver and spleen. Jam-like stools were noted 35 days after admission. B ultrasonography showed suspected intussusception. Laparotomy, reduction of intussusception and ileocecum angioplasty, biopsies of intestinal wall nodules and lymphoglandulae mesentericae, and hepatic biopsy were then performed under general anesthesia. The patient eventually died because of postoperative severe liver damage, disseminated intravascular coagulation and electrolyte disorder. Both the blood culture and hepatic biopsy tests showed Penicillium marneffei infecton. Immunodeficiency gene test was performed on the patient, his bother and their parents. T→G base substitution mutation (IVS1-3 T→G) in the CD40L gene was found in the patient. X-linked hyper-IgM syndrome was thus diagnosed in the patient. His mother was a carrier of the mutated CD40L gene, but his father was normal in the gene test. Hemizygous mutation in the CD40L gene was found in both the patient and his bother."}, {"id": "pubmed23n0533_4200", "title": "Bone and joint disease associated with primary immune deficiencies.", "score": 0.01138164063763055, "content": "Primary immune deficiencies (PIDs) are characterized by functional and/or quantitative abnormalities of one or more immune system components. Several bone and joint abnormalities can occur in patients with PID, with arthritis being the most common. Joint manifestations, of which arthritis is the most common, occur chiefly in humoral PIDs (agammaglobulinemia, common variable immunodeficiency, hyper-IgM syndromes, and IgA deficiency) and occasionally in other PIDs (chronic granulomatous disease and Wiskott-Aldrich syndrome). Monoarthritis or oligoarthritis is the usual pattern, although polyarthritis may occur, occasionally with nodules suggesting rheumatoid arthritis. Arthritis in patients with PID is usually infectious in nature, the most common causative organism being Mycoplasma, followed by Staphylococcus, Streptococcus, and Haemophilus. These bacteria can induce not only synovial infections, but also aseptic arthritogenic inflammatory responses. Arthritis having no demonstrable relation to chronic infection has been reported also and ascribed to dysimmunity-driven mechanisms that exhibit a number of specific features. Bone lesions are far less common and usually due to infections complicating humoral PID. Distinctive bone manifestations occur in a number of rare PIDs (e.g., hyper-IgE syndrome and Di George syndrome) and in syndromes characterized by spondyloepiphyseal dysplasia. Familiarity with PID syndromes both enhances the diagnostic capabilities of physicians and provides insight into the pathophysiology of bone and joint abnormalities associated with immune dysfunction. In children and occasionally in adults, a combination of bone and/or joint manifestations and hypogammaglobulinemia may indicate PID. When there is no evidence of lymphoproliferative disease, infection, or iatrogenic complications, investigations for PID should be obtained. PID-related arthritis is a unique model for studying the pathogenesis of presumably postinfectious arthritis and of inflammatory joint diseases including rheumatoid arthritis."}, {"id": "wiki20220301en186_26784", "title": "Immune disorder", "score": 0.011329652634000459, "content": "Primary immune deficiencies Severe combined immunodeficiency (SCID) DiGeorge syndrome Hyperimmunoglobulin E syndrome (also known as Job's Syndrome) Common variable immunodeficiency (CVID): B-cell levels are normal in circulation but with decreased production of IgG throughout the years, so it is the only primary immune disorder that presents onset in the late teens years. Chronic granulomatous disease (CGD): a deficiency in NADPH oxidase enzyme, which causes failure to generate oxygen radicals. Classical recurrent infection from catalase positive bacteria and fungi. Wiskott–Aldrich syndrome (WAS) Autoimmune lymphoproliferative syndrome (ALPS) Hyper IgM syndrome: X-linked disorder that causes a deficiency in the production of CD40 ligand on activated T-cells. This increases the production and release of IgM into circulation. The B-cell and T-cell numbers are within normal limits. Increased susceptibility to extracellular bacteria and opportunistic infections."}, {"id": "wiki20220301en121_8056", "title": "Hyper-IgM syndrome type 2", "score": 0.011214543861570475, "content": "Hyper IgM Syndrome Type 2 is a rare disease. Unlike other hyper-IgM syndromes, Type 2 patients identified thus far did not present with a history of opportunistic infections. One would expect opportunistic infections in any immunodeficiency syndrome. The responsible genetic lesion is in the AICDA gene found at 12p13. Hyper IgM syndromes Hyper IgM syndromes is a group of primary immune deficiency disorders characterized by defective CD40 signaling; via B cells affecting class switch recombination (CSR) and somatic hypermutation. Immunoglobulin (Ig) class switch recombination deficiencies are characterized by elevated serum IgM levels and a considerable deficiency in Immunoglobulins G (IgG), A (IgA) and E (IgE). As a consequence, people with HIGM have an increased susceptibility to infections."}, {"id": "First_Aid_Step2_928", "title": "First_Aid_Step2", "score": 0.010503705801822217, "content": "Combined (continued) Wiskott-Aldrich syndrome An X-linked disorder with less severe Band T-cell dysfunction. Patients have eczema, ↑ IgE/IgA, ↓ IgM, and thrombocytopenia. The classic presentation involves bleeding, eczema, and recurrent otitis media. ↑↑ risk of atopic disorders, lymphoma/leukemia, and infection from S. pneumoniae, S. aureus, and H. infl uenzae type b. Phagocytic Chronic granulomatous disease (CGD) Leukocyte adhesion def ciency Chédiak-Higashi syndrome An X-linked (2/3) or autosomal-recessive (1/3) disease with def cient superoxide production by PMNs and macrophages. Anemia, lymphadenopathy, and hypergamma-globulinemia may be present. A defect in the chemotaxis of leukocytes. An autosomal-recessive disorder that leads to Chronic skin, pulmonary, GI, and urinary tract infections; osteomyelitis and hepatitis. Infecting organisms are catalase . ↑ risk of infection with Aspergillus. May have granulomas of the skin and GI/GU tracts. Recurrent skin, mucosal, and pulmonary"}, {"id": "wiki20220301en477_12562", "title": "DOCK8 deficiency", "score": 0.010483870967741934, "content": "Diagnosis A diagnosis can only be definitively made after genetic testing to look for a mutation in the DOCK8 gene. However, it can be suspected with a high IgE level and eosinophilia. Other suggestive laboratory findings include decreased numbers of B cells, T cells, and NK cells; and hypergammaglobulinemia. It can be distinguished from autosomal dominant hyper-IgE (STAT3 deficiency) because people with DOCK8 deficiency have low levels of IgM and an impaired secondary immune response. IgG and IgA levels are usually normal to high. It can be distinguished from the similar X-linked Wiskott–Aldrich syndrome by the presence of thrombocytopenia and the consequent bloody diarrhea, as well as its pattern of inheritance. WHIM syndrome, caused by a mutation in CXCR4, is associated with similar chronic cutaneous viral infections."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": true, "char_ranges": [[0, 165]], "word_ranges": [[0, 28]], "text": "The confusional picture is determined by the dementia that the patient already suffers, so no matter how much we delay surgery, we are not going to achieve anything."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[166, 404]], "word_ranges": [[28, 68]], "text": "The elevation of blood pressure is due, in principle, to pain (so the first option is an analgesic) and then to the stressful situation that leads an already hypertensive patient to increase her blood pressure, so labetalol could help us."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": true, "char_ranges": [[405, 637]], "word_ranges": [[68, 104]], "text": "The last answer is not correct because it depends on the type of fracture, which is not specified in the statement: a pertrochanteric fracture would require closed osteosynthesis and a subcapital fracture would require a prosthesis."}}, "full_answer": "The confusional picture is determined by the dementia that the patient already suffers, so no matter how much we delay surgery, we are not going to achieve anything. The elevation of blood pressure is due, in principle, to pain (so the first option is an analgesic) and then to the stressful situation that leads an already hypertensive patient to increase her blood pressure, so labetalol could help us. The last answer is not correct because it depends on the type of fracture, which is not specified in the statement: a pertrochanteric fracture would require closed osteosynthesis and a subcapital fracture would require a prosthesis.", "full_answer_no_ref": "The confusional picture is determined by the dementia that the patient already suffers, so no matter how much we delay surgery, we are not going to achieve anything. The elevation of blood pressure is due, in principle, to pain (so the first option is an analgesic) and then to the stressful situation that leads an already hypertensive patient to increase her blood pressure, so labetalol could help us. [HIDDEN] because it depends on the type of fracture, which is not specified in the statement: a pertrochanteric fracture would require closed osteosynthesis and a subcapital fracture would require a prosthesis.", "full_question": "We are consulted to assess an 83-year-old woman admitted to the Trauma service for a hip fracture 6 hours ago. She has AP of hypertension, LBP, moderate dementia and lives in a nursing home. Her usual treatment is thiazide, atorvastatin, donepezil, Calcium and vitamin D. EF: Confused patient, pulse 90 bpm, respiratory rate 20 rpm, T art 170/88, jugular venous pressure normal. The CBC and chest X-ray are normal and the ECG shows sinus rhythm without ischemic alterations. Which of the following is the most correct therapeutic approach?", "id": 62, "lang": "en", "options": {"1": "Delay surgery until the confusional picture has disappeared.", "2": "Delay surgery and perform an echocardiogram.", "3": "Delay surgery until good blood pressure control.", "4": "Start a beta-blocker and initiate surgery.", "5": "Perform closed osteosynthesis, avoiding in any case the implantation of prosthesis."}, "question_id_specific": 119, "type": "ANESTHESIOLOGY AND CRITICAL CARE", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "Surgery_Schwartz_2833", "title": "Surgery_Schwartz", "score": 0.01129745417713629, "content": "an injection of 1% lidocaine solution around this structure should attenuate this reflexive response.The most common delayed complication following carotid endarterectomy remains myocardial infarction. The possibility of a postoperative myocardial infarction should be considered as a cause of labile blood pressure and arrhythmias in high-risk patients.Thyroid and Parathyroid Glands. Surgery of the thyroid and parathyroid glands can result in hypocalcemia in the immedi-ate postoperative period. Manifestations include ECG changes (shortened P-R interval), muscle spasm (tetany, Chvostek’s sign, and Trousseau’s sign), paresthesias, and laryngospasm. Treatment includes calcium gluconate infusion and, if tetany ensues, chemical paralysis with intubation. Maintenance treat-ment is thyroid hormone replacement (after thyroidectomy) in addition to calcium carbonate and vitamin D.Recurrent laryngeal nerve (RLN) injury occurs in less than 5% of patients. Of those with injury, approximately 10%"}, {"id": "pubmed23n0274_22316", "title": "[Anesthesia and intensive therapy for a patient with mitochondrial myopathy].", "score": 0.009900990099009901, "content": "Since 1983 we have been involved in the diagnostic work-up and emergency treatment of a female patient now 48 years old who has a mitochondrial myopathy resembling Luft's disease. The syndrome was first described in 1959, and in more detail in 1962, by Luft and et al., who reported a picture of hypermetabolism with high temperature, extreme sweating, tachycardia, dyspnoea at rest, polydipsia, polyphagia and irritability but normal thyroid function. In 1971 and 1976 Haydar and Di Mauro presented a second case and proposed treatment with chloramphenicol. Our patient has the third case of the syndrome reported so far: her case was initially published in 1987. CASE REPORT. Since her 17th year of life the patient had suffered from episodes of fever, tachycardia and sweating. At the age of 32 these attacks worsened, leading to unconsciousness and apnoea. The patient then had to be intubated, ventilated and sometimes resuscitated. The diagnosis of MH susceptibility and Luft's disease was made on biochemical grounds after the first muscle biopsy in 1983. Therapy with chloramphenicol failed. Therapy with beta blockers, vitamin C and K or E, coenzyme Q10 and a high-caloric diet was started in 1985. The patient was registered with an emergency service, which flew her to our ICU whenever she had a severe crisis. For milder episodes she was supplied with an oxygen breathing mask at home. Myalgia increased with the episodes starting in 1988, and the patient needed dantrolene infusions and analgesics at home. To facilitate venepuncture a Port-A-Cath system was implanted in 1987, which had to be removed four times due to infection and sepsis. A muscle biopsy was taken in Rotterdam, which revealed differences in mitochondrial function from the biochemical findings recorded in 1983 and not in keeping with Luft's disease. Unfortunately, the patient was not able to undergo further metabolic investigations or therapeutic trials. ANAESTHESIA. The patient received three local and six general anaesthetics in our clinic. The muscle biopsies, two in 1983 and one in 1985, were performed under local infiltration with procaine and were uneventful. The general anaesthetics were carried out without MH trigger substances following pretreatment with dantrolene for the following surgical procedures: the repair of an extensive arterio-venous fistula between the brachiocephalicus trunk and the right jugular and subclavian vein, revision of the sternum cerclage, implantations and explanations of infectious Port-A-Cath systems. We used etomidate, propofol and fentanyl or alfentanil with nitrous oxide and oxygen for induction and maintenance of anaesthesia. Muscle relaxation was induced with vecuronium or atracurium. All cardiovascular, respiratory, metabolic and temperature measurements stayed in normal ranges. After the extensive vascular repair (av fistula) the patient had to be mechanically ventilated for some hours until normal body temperature was restored. At the end of all other periods of anaesthesia she was extubated in the operating theatre. In five cases the postoperative period was uneventful. Only once she developed a crisis with hyperthermia, tachycardia, sweating and dyspnoea. INTENSIVE CARE. From 1985 to 1992 the patient was treated in our ICU 21 times. On 11 occasions she was already intubated and being ventilated by the emergency service on arrival. Extubation was usually possible within 2-20 h. During the crisis, heart rate was about 160-190 per minute and temperature above 40 degrees C. Serum values of CK, glucose, BUN, electrolytes, lactate and thyroid hormones were always in the normal ranges. Blood gas controls showed a constant respiratory alkalosis, arterial pCO2 values decreasing to 20 mm Hg or less. In addition to mechanical ventilation, treatment consisted in dantrolene infusions and droperidol injections, supplemented from 1989 onward with piritramide injections because of the increased severity of myalgia. In 1991 we gave propofol by"}, {"id": "pubmed23n0652_3528", "title": "[What's new in geriatric medicine].", "score": 0.009900990099009901, "content": "Several studies clarified the role of different interventions such as vitamine D replacement, denosumab treatment, and vertebroplasty in the prevention and management of falls and fractures. A trial tested the effectiveness of pharmaceutical assistance at the time of discharge, emphasizing the potential benefits for the patients and the health care system. Syncopal episodes frequently lead to hospital admission. A retrospective study evaluated the diagnostic yield of different tests and emphasized the importance to actively seek orthostatic hypotension in older patients. Finally, advances remain modest in the field of dementias."}, {"id": "pubmed23n0929_14420", "title": "Extracorporeal membrane oxygenation system as a bridge to reparative surgery in ventricular septal defect complicating acute inferoposterior myocardial infarction.", "score": 0.00980392156862745, "content": "Post-infarction ventricular septal defect (VSD) is a rare but potentially lethal complication of acute myocardial infarction. Medical management is usually futile, so definitive surgery remains the treatment of choice but the risk surgery is very high and the optimal timing for surgery is still under debate. A 55-year-old man with no previous medical history attended the emergency-room for 12 h evolution of oppressive chest pain and strong anginal pain 7 days ago. On physical examination, blood pressure was 96/70 mmHg, pansystolic murmur over left sternal border without pulmonary crackles. An electrocardiogram revealed sinus rhythm 110 bpm, elevation ST and Q in inferior-posterior leads. Transthoracic echocardiogram showed inferoposterior akinesia, posterior-basal septal rupture (2 cm × 2 cm) with left-right shunt. Suspecting VSD in inferior-posterior acute myocardial infarction evolved, we performed emergency coronarography with 3-vessels disease and complete subacute occlusion of the mid segment of the right coronary artery. Left ventriculography demonstrated shunting of contrast from the left ventricule to the right ventricule. He was rejected for heart transplantation because of his age. Considering the high surgical risk to early surgery and his hemodynamic and clinical stability, delayed surgical treatment is decided, and 4 days after admission the patient suffered hemodynamic instability so venoarterial extracorporeal membrane oxygenation system (ECMO) is implanted as a bridge to reparative surgery. The 9th day after admission double bypass, interventricular defect repair with pericardial two-patch exclusion technique, and ECMO decannulation were performed. The patient's postoperative course was free of complications and was discharged 10 days post VSD repair surgery. Follow-up 3-month later revealed the patient to be in good functional status and good image outcome with intact interventricular septal patch without shunt. ECMO as a bridge to reparative surgery in postinfarction VSD is an adequate option to stabilize patients until surgery."}, {"id": "pubmed23n0698_23113", "title": "[Patient whose surgery was postponed due to complete atrioventricular block on arrival at operating theater].", "score": 0.00980392156862745, "content": "An 86-year-old woman with low cardiac function was scheduled to undergo hip fracture surgery. Preoperative electrocardiogram showed complete left bundle brunch block, first degree atrioventricular block, left axis deviation and bigeminy. However, her electrocardiogram had changed to complete atrioventricular block on arrival at operating theater. ACC/AHA guideline on perioperative cardiovascular evaluation and care for non cardiac surgery indicates the assessment of both the urgency of the surgery and cardiac complications. Because complete atrioventricular block is classified to \"active cardiac conditions\", we decided to postpone the surgery for more detailed evaluation and treatment of cardiac conditions. In spite of the discontinuation of digoxin and carvegilol, complete atrioventricular block continued for a week, and the permanent pacemaker was inserted. The surgery was performed 2 weeks following the insertion of the pacemaker without any problems under combined general and lumbar epidural anesthesia."}, {"id": "wiki20220301en027_68001", "title": "Aneurysm of sinus of Valsalva", "score": 0.009708737864077669, "content": "Treatment Medical therapy of aneurysm of the aortic sinus includes blood pressure control through the use of drugs, such as beta blockers. Another approach is surgical repair. The determination to perform surgery is usually based upon the diameter of the aortic root (with 5 centimeters being a rule of thumb - a normal size is 2-3 centimeters) and the rate of increase in its size (as determined through repeated echocardiography). An alternative to surgical repair or a ruptured aneurysm is percutaneous closure. In this technique, a wire is introduced via a small incision in the groin and advanced through the vascular system to the aneurysm. A closure device is advanced along the wire before being expanded to straddle the site of rupture."}, {"id": "pubmed23n0945_6227", "title": "[Severe hypercalcemia of unusual cause, looking for the culprit: Case report and review of the literature].", "score": 0.009708737864077669, "content": "Hypercalcemia is not a rare event and can lead to severe consequences. Its main etiologies are primary hyperparathyroidism and neoplasic conditions. The iatrogenic etiology by vitamin D intoxication is more rarely found. A 76-year-old finish woman comes to the emergency room for chest pain. Her medical history is impossible to specify due to the language barrier and initial confusion. She has severe hypercalcaemia (4.14mmol/L), renal insufficiency, cardiac arrhythmia later complicated by an ischemic cardiac episode. Clinic and biologic examinations initially guided the research towards a hematological and neoplasic pathology. The iatrogenic etiology will be permitted by the contribution of details on its medical history and treatment learnt secondly. She was treated for post-surgical hypoparathyroidism by dihydrotachysterol, a vitamin D derivative. The cessation of substitution, treatment with hydration and biphosphonates allowed the rapid correction of hypercalcemia. Dihydrotachysterol intoxication is a rare etiology of hypercalcemia. Because of the longer half-life of this molecule, the risk of hypercalcemia seems to be greater than with other vitamin D derivatives. This molecule, withdrawn from the French market in 1982, is not detected by the dosage of 25 and 1.25 OH vitamin D. We report an original case of intoxication by dihydrotachysterol. The risk of hypercalcemia encountered with this molecule must be known. The close medical follow-up recommended in case of hypoparathyroidism seems to be particularly necessary in case of supplementation by this molecule."}, {"id": "pubmed23n0609_14977", "title": "Cardiotoxicity after massive amantadine overdose.", "score": 0.009615384615384616, "content": "Amantadine hydrochloride is an antiviral medication used as therapy for parkinsonism and as a cognitive enhancer. We report 2 cases of massive, acute ingestion of amantadine hydrochloride confirmed with serial serum levels. A 47-year-old woman presented to the emergency department (ED) 30 minutes after ingesting 10 g of amantadine (150 mg/kg) by her report. Initial ECG revealed a sinus rhythm with rate of 93 bpm, and a QRS of 84 msec. While in the ED, the patient sustained a pulseless cardiac arrest and the monitor revealed ventricular tachycardia. She was successfully defibrillated. Postdefibrillation ECG showed a sinus rhythm (rate = 82 bpm), QRS of 236 msec, and QTc of 567 msec. The serum potassium was 1.0 mEq/L (1.0 mmol/L). The patient was given 300 ml (300 cc) 3% sodium chloride IV over 10 minutes. Ten minutes after completion of the hypertonic saline infusion, the patient's ECG abnormalities resolved and the QRS was 88 msec. Her potassium was repleted over the next 11 hours postpresentation, and she also received an IV bolus of 4 g of magnesium sulfate immediately after the cardiac arrest. No further hypotension, dysrhythmia, conduction delay, or ectopy was noted during the patient's hospital stay. The second case involved a 33-year-old female patient who presented 1 hour after ingesting 100 tablets of amantadine hydrochloride (100 mg/tab). Initial ECG revealed sinus tachycardia with a QRS of 113 msec, an R wave in lead aVR of 4-5 mm and a QTc of 526 msec. Her serum potassium was 3.0 mEq/L (3.0 mmol/L), her serum calcium was 9.4 mg/dl (2.35 mmol/L), and serum magnesium was 2.1 mg/dl (0.86 mmol/L) on labs drawn at initial presentation. The patient was intubated for airway protection, and her potassium was repleted and corrected over the next 9 hours. Her ECG abnormalities improved 8 hours after initial presentation and normalized at approximately 14 hours postingestion. The patient was discharged home 11 days after her ingestion. Acute amantadine toxicity manifests with life-threatening cardiotoxicity. Concurrent, often profound, hypokalemia may complicate the administration of sodium bicarbonate in the management of cardiac dysrhythmias."}, {"id": "pubmed23n0597_23565", "title": "[Interaction of calcium drug and vitamin D3 with some medicines used in coronary heart disease therapy].", "score": 0.009615384615384616, "content": "130 young and middle age patients of both sexes with chronic form of coronary heart disease: functional class II-III stable exertional angina pectoris including functional class I-III chronic cardiac insufficiency were studied. In protocol 1 cured 70 patients (48 (68.6%) males and 22 (31.4%) females) 32-59 years of age (medium age was 48.4 +/- 3.25 years) with coronary heart disease. In protocol 2 (with prescription of calcium-D3) cured 60 patients (40 (66.7%) males and 20 (33.3%) females) 34-58 years of age (medium age was 47.8 +/- 3.12 years) with coronary heart disease. The groups were comparable on key parameters of disease. All patients had alimentary calcium deficit and (or) risk factors of osteoporosis, instrumental signs (X-ray filming and densitometry) of initial or evident osteoporosis. Correction of alimentary calcium deficit was realized by prescription of 1-3 tablets of calcium- Ds in different food intakes. Positive dynamics in decrease of functional class of angina pectoris and nitroglycerin requirement in both groups was noticed. Negative influence of calcium- D3 on studied indices of coronary heart disease severity was absent. The thirst and dry mouth in patients, who took furosemide, in group 1 were noticed against the background of body weight decrease (p &lt; 0.05) and increase of diuresis. Decrease of the therapy antiarrhythmic action (p &lt; 0.05) in patients, who took hydrochlorothiazide, was noticed too. It leaded to needs of furosemide and hydrochlorothiatide dose correction in protocol 1. In whole use of calcium- D3 together with anti-ischemic drugs in patients with chronic forms of coronary heart disease did not impair clinical course of angina pectoris and did not decrease efficiency of coronary heart disease therapy."}, {"id": "wiki20220301en047_45308", "title": "Hip fracture", "score": 0.009523809523809525, "content": "Most hip fractures are treated surgically by implanting a prosthesis. Surgical treatment outweighs the risks of nonsurgical treatment which requires extensive bedrest. Prolonged immobilization increases risk of thromboembolism, pneumonia, deconditioning, and decubitus ulcers. Regardless, the surgery is a major stress, particularly in the elderly. Pain is also significant, and can also result in immobilization, so patients are encouraged to become mobile as soon as possible, often with the assistance of physical therapy. Skeletal traction pending surgery is not supported by the evidence. Regional nerve blocks are useful for pain management in hip fractures. Peripheral nerve blocks may reduce pain on movement and acute confusional state, may improve time to first mobilisation, and may reduce the risk of postoperative lower respiratory tract infection. Surgery can be performed under general anaesthesia or with neuraxial techniques – choice is based on surgical and patient factors, as"}, {"id": "pubmed23n0037_938", "title": "[Hypo and hypercalcemia as an emergency].", "score": 0.009523809523809525, "content": "1. Hypo- and hypercalcemia can be explained as derangements of the calcium homeostasis. Hypocalcemic tetany usually alarming the patient tremendously is, at least in adults, rarely life-threatening. Hypercalcemia leads in 30% of the cases to clinical symptoms which may inadvertedly pass into a state of hypercalcemic crisis. This latter requires an often difficult emergency treatment. 2. Hypocalcemic tetany may be reversed by administering calcium i.v. or, in severe cases, by a calcium infusion. Only rarely are magnesium supplements necessary to let the tetany disappear. Vitamin D or dihydrotachysterol (DHT) do not correct hypocalcemia immediately, since their effects may be delayed up to 15-25 days. In order to normalize the serum calcium permanently, vitamin D or DHT treatment should be instituted as rarely as possible. 3. Initially, hypercalcemic crisis is best treated by forced intravenous fluid administration with normal saline (and furosemide) in combination with high doses of prednisone. Fluid-, sodium- and potassium balances ought to be checked during this type of treatment. A first evaluation of the effectiveness of these measures is recommended after 24 hours: treatment is continued in patients who respond favorably, while subjects who do not show a significant decrease of the serum calcium may either be given a phosphate infusion or mithramycine as a bolus. Calcitonin appears to be useful only to start treatment before institution of a phosphate infusion."}, {"id": "pubmed23n0651_7761", "title": "[Case of ischemic heart disease resulting from persistent diuresis after giant ovarian tumor resection].", "score": 0.009433962264150943, "content": "A patient with a giant ovarian tumor weighing about 7 kg was successfully removed by operation. However, her ECG demonstrated ischemic changes after the operation. We report a case of ischemic heart disease due to persistent diuresis after giant ovarian tumor resection. A 75-year-old, 56.5 kg, 143.5 cm woman was admitted to our hospital for ovarian tumor resection. The preoperative ECG showed normal sinus rhythm and no ischemic changes. Both general anesthesia and epidural anesthesia were planed. An epidural catheter was inserted at T12-L1. Anesthesia was induced with propofol 100 mg, fentanyl 100 microg and vecuronium 8 mg under 100% oxygen inhalation. General anesthesia was maintained with sevoflurane while epidural anesthesia was achieved using 0.375% ropivacaine 6 ml. During the operation, blood pressure was 90-110/70-80 mmHg, with SaO2, 100% and heart rate, 70-80 beats x min(-1). The content of tumor was suctioned for 30 minutes. Surgery was successfully finished without any other incidence. After extubation, her ECG changed to atrial fibrillation from normal sinus rhythm and showed ST-T depression. And then her systolic blood pressure became 80 mmHg or below, but we found continued diuresis at about 10 ml x kg(-1) x hr(-1) for over 2 hr. The total of 7 unit vasopressin was intermittently given for vasoconstriction and antidiuresis. Her hemodynamic was immediately restored, and ECG turned to normal ST-T. The patient had uneventful postoperative recovery."}, {"id": "pubmed23n0933_8774", "title": "[Geriatric medicine].", "score": 0.009433962264150943, "content": "2017 highlights benefits of prevention. Better control of cardiovascular risk reduces the incidence of dementia and monthly high-dose vitamin D the incidence of respiratory infections in nursing home. Pre-operative geriatric assessment lowers by 20% the rate of delirium after hip-fracture surgery and complications in vascular surgery. Deleterious effects are also reported. High-dose vitamin D triples the rate of falls in supplemented residents and doesn't improve gait speed in sedentary men. Widely used in cardiovascular prevention, antithrombotic therapy is associated with an astonishing risk of subdural bleeding that further increases with the number of drugs combined together. Finally, the non-pharmacological management of behavioral and psychotic symptoms in advanced dementia, although effective, doesn't reduce the associated burden for proxies."}, {"id": "pubmed23n1094_2769", "title": "The Young Heart Tears Easily Apart: A Case Report of Spontaneous Coronary Artery Dissection.", "score": 0.009345794392523364, "content": "Spontaneous coronary artery dissection (SCAD) is a rare cause of acute coronary syndrome (ACS), seen mostly in young females. The rarity and limited knowledge of the disease make its management challenging. Prompt diagnosis of the condition is extremely important to decrease both long- and short-term complications. Treatment options depend on hemodynamic stability and the location of the dissection- with more distal lesions treated more conservatively as opposed to proximal lesions which are treated with percutaneous coronary intervention (PCI) or coronary artery bypass graft (CABG). The following are the two cases with different presentation, management and outcomes. Our first patient was a 35-year-old woman with no medical history who presented with acute, anginal pain, diaphoresis and palpitations. She was hemodynamically stable on presentation, with work-up significant for electrocardiogram (ECG) with sinus bradycardia, ST elevation in leads V1-V6, and elevated troponin level of 4 ng/ml. There was no evidence of a pulmonary embolism on computed tomography (CT) of the chest. A coronary angiogram showed 100% dissection of the proximal to mid-left anterior descending (LAD) artery. Attempts to place a stent in the proximal to mid LAD were unsuccessful as the true lumen of the LAD was not accessible. The patient became hemodynamically unstable, and an emergent CABG was done, restoring blood flow. The patient recovered during her hospital stay and was discharged with dual antiplatelet therapy (DAPT), beta-blockers, and atorvastatin. The second patient was a 28-year-old woman, with a history of hypertension who presented with anginal chest pain. Workup showed ECG with minimal ST elevations in anteroseptal leads, with elevated troponin level to 0.71 ng/ml. Coronary angiogram showed 40-50% stenosis of the mid LAD with an aneurysmal segment. An echocardiogram showed no evidence of wall motion abnormalities, and she had a normal left ventricular ejection fraction (LVEF). She was discharged home the next day, on medical management. After two days, she returned to the hospital with similar complaints, with work-up significant for ECG with non-specific ST-T abnormality, and troponin level which peaked at 2.22 ng/ml. She was started on a heparin drip, and a repeat left heart catheterization revealed type 2 dissection of the mid to distal LAD, with intravascular ultrasound showing a fractional flow reserve of 0.76. She was discharged home on DAPT, beta-blocker, calcium channel blocker (CCB), and atorvastatin, with close cardiology follow up. These two cases highlight the importance of keeping in mind the possibility of SCAD, especially when relatively healthy young women present with anginal symptoms. Early diagnosis of the condition and prompt management are extremely important to ensure favourable outcomes. The two cases also describe the coronary angiogram findings in SCAD, and the different strategies employed in the management of this condition."}, {"id": "pubmed23n0070_2240", "title": "[The parathyroid risk in thyroid surgery. Argument against the early postoperative prescription of vitamin D. Experience with 729 thyroidectomies in 1988].", "score": 0.009345794392523364, "content": "729 consecutive patients underwent thyroidectomy in 1988 in the same institution, including 477 (68%) bilateral resections and 242 (33%) total thyroidectomies. An effort was made to see and save all 4 parathyroids and their blood supply. Early post-operative hypoparathyroidism was defined at day 5, by serum calcium less than 8 mg/dl. and serum phosphate less than 4 mg/dl or by serum calcium only if greater than 7.5 mg/dl. Patients afflicted with early hypoparathyroidism were given calcium tablets without any vit D for 1 year at most. Follow-up, checking serum Ca, P and i PIH was done on a 3 months basis during 1 year. Permanent hypoparathyroidism was defined by persistence of the above-mentioned criteria after 1 year, and eventually vit D was started. 27 patients (5.6% our of 477 bilateral thyroid resections) experienced early post-op hypoparathyroidism. Inciting factors were previous thyroid surgery (4), radioiodine treatment (2), modified neck dissection (2), sternal split with mediastinal node clearance (1), visualization of 1 parathyroid gland only (3 redo cases) and autotransplantation of more than 1 parathyroid (1 case). 1 patient was lost for follow-up. 25 others recovered a normal parathyroid function. 1 is permanently hypoparathyroid (1 redo case with other risk factors). Painstaking parathyroid dissection allows a 0% rate of permanent hypoparathyroidism after primary surgery, if vit D is not given in the early post-operative period. We suggest that avoidance of early vit D prescription in cases of early post-operative hypoparathyroidism, leading to mild sustained hypocalcemia, stimulates the spared parathyroid glands (including a possible 5th) and therefore allows full recovery of the parathyroid function."}, {"id": "pubmed23n0800_14851", "title": "Breathlessness with pulmonary metastases: a multimodal approach.", "score": 0.009259259259259259, "content": "Case Study  Sarah is a 58-year-old breast cancer survivor, social worker, and health-care administrator at a long-term care facility. She lives with her husband and enjoys gardening and reading. She has two grown children and three grandchildren who live approximately 180 miles away. SECOND CANCER DIAGNOSIS  One morning while showering, Sarah detected a painless quarter-sized lump on her inner thigh. While she thought it was unusual, she felt it would probably go away. One month later, she felt the lump again; she thought that it had grown, so she scheduled a visit with her primary care physician. A CT scan revealed a 6.2-cm soft-tissue mass in the left groin. She was referred to an oncologic surgeon and underwent an excision of the groin mass. Pathology revealed a grade 3 malignant melanoma. She was later tested and found to have BRAF-negative status. Following her recovery from surgery, Sarah was further evaluated with an MRI scan of the brain, which was negative, and a PET scan, which revealed two nodules in the left lung. As Sarah had attended a cancer support group during her breast cancer treatment in the past, she decided to go back to the group when she learned of her melanoma diagnosis. While the treatment options for her lung lesions included interleukin-2, ipilimumab (Yervoy), temozolomide, dacarbazine, a clinical trial, or radiosurgery, Sarah's oncologist felt that ipilimumab or radiosurgery would be the best course of action. She shared with her support group that she was ambivalent about this decision, as she had experienced profound fatigue and nausea with chemotherapy during her past treatment for breast cancer. She eventually opted to undergo stereotactic radiosurgery. DISEASE RECURRENCE  After the radiosurgery, Sarah was followed every 2 months. She complained of shortness of breath about 2 weeks prior to each follow-up visit. Each time her chest x-ray was normal, and she eventually believed that her breathlessness was anxiety-related. Unfortunately, Sarah's 1-year follow-up exam revealed a 2 cm × 3 cm mass in her left lung, for which she had a surgical wedge resection. Her complaints of shortness of breath increased following the surgery and occurred most often with anxiety, heat, and gardening activities, especially when she needed to bend over. Sarah also complained of a burning \"pins and needles\" sensation at the surgical chest wall site that was bothersome and would wake her up at night. Sarah met with the nurse practitioner in the symptom management clinic to discuss her concerns. Upon physical examination, observable signs of breathlessness were lacking, and oxygen saturation remained stable at 94%, but Sarah rated her breathlessness as 7 on the 0 to 10 Borg scale. The nurse practitioner prescribed duloxetine to help manage the surgical site neuropathic pain and to assist with anxiety, which in turn could possibly improve Sarah's breathlessness. Several nonpharmacologic modalities for breathlessness were also recommended: using a fan directed toward her face, working in the garden in the early morning when the weather is cooler, gardening in containers that are at eye level to avoid the need to bend down, and performing relaxation exercises with pursed lip breathing to relieve anxiety-provoked breathlessness. One month later, Sarah reported relief of her anxiety; she stated that the fan directed toward her face helped most when she started to feel \"air hungry.\" She rated her breathlessness at 4/10 on the Borg scale. SECOND RECURRENCE: MULTIPLE PULMONARY NODULES  Sarah's chest x-rays remained clear for 6 months, but she developed a chronic cough shortly before the 9-month exam. An x-ray revealed several bilateral lung lesions and growth in the area of the previously resected lung nodule. Systemic therapy was recommended, and she underwent two cycles of ipilimumab. Sarah's cough and breathlessness worsened, she developed colitis, and she decided to stop therapy after the third cycle. In addition, her coughing spells triggered bronchospasms that resulted in severe anxiety, panic attacks, and air hunger. She rated her breathlessness at 10/10 on the Borg scale during these episodes. She found communication difficult due to the cough and began to isolate herself. She continued to attend the support group weekly but had difficulty participating in conversation due to her cough. Sarah was seen in the symptom management clinic every 2 weeks or more often as needed. No acute distress was present at the beginning of each visit, but when Sarah began to talk about her symptoms and fear of dying, her shortness of breath and anxiety increased. The symptom management nurse practitioner treated the suspected underlying cause of the breathlessness and prescribed oral lorazepam (0.5 to 1 mg every 6 hours) for anxiety and codeine cough syrup for the cough. Opioids were initiated for chest wall pain and to control the breathlessness. Controlled-release oxycodone was started at 10 mg every 12 hours with a breakthrough pain (BTP) dose of 5 mg every 2 hours as needed for breathlessness or pain. Sarah noted improvement in her symptoms and reported a Borg scale rating of 5/10. Oxygen therapy was attempted, but subjective improvement in Sarah's breathlessness was lacking. END OF LIFE  Sarah's disease progressed to the liver, and she began experiencing more notable signs of breathlessness: nasal flaring, tachycardia, and restlessness. Opioid doses were titrated over the course of 3 months to oxycodone (40 mg every 12 hours) with a BTP dose of 10 to 15 mg every 2 hours as needed, but her breathlessness caused significant distress, which she rated 8/10. The oxycodone was rotated to IV morphine continuous infusion with patient-controlled analgesia (PCA) that was delivered through her implantable port. This combination allowed Sarah to depress the PCA as needed and achieve immediate control of her dyspneic episodes. Oral lorazepam was also continued as needed. Sarah's daughter moved home to take care of her mother, and hospice became involved for end-of-life care. As Sarah became less responsive, nurses maintained doses of morphine for control of pain and breathlessness and used a respiratory distress observation scale to assess for breathlessness since Sarah could no longer self-report. A bolus PCA dose of morphine was administered by Sarah's daughter if her mother appeared to be in distress. Sarah died peacefully in her home without signs of distress. "}, {"id": "InternalMed_Harrison_28527", "title": "InternalMed_Harrison", "score": 0.009259259259259259, "content": "use calcitriol (doses of 0.5–1 μg/d) because of the rapidity of onset of effect and prompt cessation of action when stopped, in comparison to other forms of vitamin D. A rise in blood calcium after several months of vitamin D replacement may indicate restoration of parathyroid function to normal. It is also appropriate to monitor serum PTH serially to estimate gland function in such patients."}, {"id": "pubmed23n1140_22727", "title": "Atypical Presentation of Interval Colorectal Cancer/Post-Colonoscopy Colorectal Cancer in a Nursing Home Patient.", "score": 0.009174311926605505, "content": "The Centers for Disease Control and Prevention estimates that there are around 1.7 million beds in certified nursing homes across the United States and approximately 1.3 million residents in long-term and end-of-life care. There could be several factors causing a delayed recovery in such patients, such as decreased ambulation, multiple comorbidities, and polypharmacy. An 83-year-old Caucasian woman sustained a fall resulting in compression fractures of the thoracic and lumbar spine. She had multiple comorbidities, including anemia of chronic disease, malnutrition, and a significant weight loss of 30 lbs over the four months prior to hospitalization. She was on antihypertensives, antidepressants, vitamin D, and calcium supplementation. Her medical history was significant for constipation with the passage of stools once in three days. Her family history was significant for colorectal cancer (CRC) and her screening colonoscopy three years ago was normal. Physical examination revealed no abdominal tenderness or distention. Subsequently, she developed edema in the left lower extremity. She underwent a venous Doppler/ultrasound study, which showed an occlusive thrombus from the common femoral vein to the popliteal vein. She was started on anticoagulants and supportive therapy. Four months later, while at the nursing home, she developed bloating and flatulence, in addition to pre-existing constipation. Examination revealed a 6 x 7 cm mass in the right lower quadrant without peritoneal signs. Bowel sounds were significantly decreased. CT imaging showed a 6-cm diameter cecal mass. The tumor was a low-grade 4 x 9 cm T4N0M0 cecal cancer, and she underwent placement of a Greenfield filter and subsequent hemicolectomy. She had methicillin-resistant <iStaphylococcus aureus</i infection and right upper extremity deep vein thrombosis (DVT), urinary tract infection, <iClostridium difficile</i colitis, and depression, all managed successfully and without sequelae in the post-operative period. Treatment on discharge comprised Coumadin maintenance for nine months with an international normalized ratio goal of 2-3, a back brace, antidepressants, and antihypertensive medications. She received follow-up care at home. Maintaining a high degree of suspicion for new and persistent symptoms in the elderly is essential to identify the underlying cause. One of the leading causes of post-colonoscopy CRC is a missed lesion. Careful attention to all cases of anemia as well as DVT in the elderly is also imperative to diagnose such missed cases. Future research should focus on the methods of CRC diagnosis in elderly patients with comorbidities apart from using colonoscopy alone."}, {"id": "pubmed23n1051_667", "title": "[Dual mobility total hip arthroplasty for the treatment of femoral neck fracture with hemiplegia].", "score": 0.00909090909090909, "content": "To investigate the clinical effects of dual mobility total hip prosthesis in treating femoral neck fracture patients with hemiplegia. A retrospective analysis was performed on 18 patients with femoral neck fracture combined with hemiplegia who underwent dual mobility total hip prosthesis replacement from March 2014 to December 2016. The follow up data of these patients was complete. There were 5 males and 13 females, aged 65 to 70 years old with an average of (66.50±1.38) years. The left side was involved in 12 cases, while the right side in 6 cases. There were 4 cases with Garden Ⅲ type and 14 cases with type Ⅳ. Limb muscle strength of hemiplegia were in grade Ⅳ. The posterior-lateral approach of hip joint was used in surgery for all patients. The implant position, dislocation and loosening of the prosthesis were evaluated by X-ray examination. Harris hip score and the Merle D'aubigne score were used to assess the hip function in the follow up. The operation duration was for 70-90 (81.56±7.48) min and the blood loss during the operation was for 160-200 (170.32± 12.56) ml. No blood was transfused during the operation. Postoperative incisions were healed at the first stage. The follow-up time was for 28-60(36.0±3.5) months. Harris hip score increased from 16.94±0.73 preoperatively to 96.19±1.27 at the final follow-up(<iP</i&lt;0.05). Merle D 'Aubigne score increased from 3.96±0.06 preoperatively to 16.81±0.63 at the final follow-up(<iP</i&lt; 0.05). No fracture or nerve or vascular injury were found during the operation. The postoperative X-ray showed that the prosthesis was in good position. No complications such as joint dislocation, dislocation of prosthesis, loosening of prosthesis, fracture around the prosthesis, pain in the front of thethigh, fracture of the self tapping screw in the ilium, and delayed infection occurred in the patients after operation. Dual mobility total hip prosthesis has the advantages of both good initial stability and low dislocation rate of the prosthesis, and the clinical application of total hip replacement in hemiplegic femoral neck fracture is satisfactory."}, {"id": "InternalMed_Harrison_874", "title": "InternalMed_Harrison", "score": 0.00909090909090909, "content": "FIgURE 11-17 Algorithm depicting assessment and management of falls in older patients. HR, heart rate. (From American Geriatrics Society and British Geriatrics Society: Clinical Practice Guideline for the Prevention of Falls in Older Persons. New York, American Geriatric Society, 2010.) sensory, nervous system, brain, cardiovascular, and musculoskeletal contributors. Interventions depend on the factors identified but often include medication adjustment, physical therapy, and home modifications. Meta-analyses of strategies to reduce the risk of falls have found that multifactorial risk assessment and management as well as individually targeted therapeutic exercise are effective. Supplementation with vitamin D at 800 IU daily may also help reduce falls, especially in older persons with low vitamin D levels."}, {"id": "pubmed23n0249_4188", "title": "Management of aneurysmal subarachnoid hemorrhage.", "score": 0.009009009009009009, "content": "Treatment of ischemic deficits caused by vasospasm relies on enhancing cardiac output, inducing arterial hypertension, and expanding the intravascular volume in an attempt to improve CBF. Different treatment protocols exist from institution to institution to achieve these goals. The role of calcium-channel blockers now is well established. The newest focus on prevention of vasospasm includes tPA and a variety of anti-inflammatory drugs and potential neuroprotective drugs under research. Endovascular therapy for vasospasm has an increasing role in treating patients who are unable to tolerate induced hypertension or aggressive volume augmentation. We will return to our index case of the 63-year-old woman with SAH caused by an ACoA aneurysm to review some major management issues. After placing a ventriculostomy and slowly lowering ICP, the patient became alert and was fully oriented. She had aneurysm surgery on hospital day 2, with an uncomplicated immediate postoperative course. A Swan-Ganz catheter, placed for intraoperative monitoring, was kept in place and she was hydrated with 125 mL/hour of normal saline, achieving a PAWP of 10 to 16 mm Hg. Her mean arterial blood pressure without pharmacologic intervention was 95 to 110 mm Hg. She had continued clinical improvement with resolution of her left hemiparesis. On hospital day 5, her ventriculostomy was clamped because cerebrospinal fluid drainage was minimal. The following morning, the patient was arousable only to deep pain and her left side was flaccid. An emergent CT scan demonstrated no new hemorrhage, no increase in ventricular size, and no infarct. Vasospasm was considered the most likely cause. Hypertensive therapy was about to be initiated with a phenylephrine drip, but within an hour she was fully alert and moving all extremities equally. A search for other potential causes of neurologic decline was undertaken and revealed a phenytoin level of 5.5. It was thought that the patient most likely had had a seizure and that her clinical deterioration represented a postictal state. She received a bolus infusion of phenytoin. On hospital day 7, the patient became confused, insisting that her nurse was her son and ordering him out of her \"apartment.\" Lower extremity weakness was detected. CT scan was unchanged. Phenylephrine was started but she developed precordial lead ST elevation and elevated cardiac enzymes. Topical nitrate therapy was initiated and phenylephrine was discontinued. The patient underwent emergent cerebral angiography, which demonstrated moderate to severe bilateral ACA spasm and moderate right MCA spasm.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "InternalMed_Harrison_28526", "title": "InternalMed_Harrison", "score": 0.009009009009009009, "content": "Signs of hypocalcemia include symptoms such as muscle twitching, a general sense of anxiety, and positive Chvostek’s and Trousseau’s signs coupled with serum calcium consistently <2 mmol/L (8 mg/dL). Parenteral calcium replacement at a low level should be instituted when hypocalcemia is symptomatic. The rate and duration of IV therapy are determined by the severity of the symptoms and the response of the serum calcium to treatment. An infusion of 0.5–2 mg/kg per hour or 30–100 mL/h of a 1-mg/mL solution usually suffices to relieve symptoms. Usually, parenteral therapy is required for only a few days. If symptoms worsen or if parenteral calcium is needed for >2–3 days, therapy with a vitamin D analogue and/or oral calcium (2–4 g/d) should be started (see below). It is cost-effective to use calcitriol (doses of 0.5–1 μg/d) because of the rapidity of onset of effect and prompt cessation of action when stopped, in comparison to other forms of vitamin D. A rise in blood calcium after"}, {"id": "pubmed23n0827_6236", "title": "Iatrogenic aortic insufficiency following mitral valve replacement: case report and review of the literature.", "score": 0.008928571428571428, "content": "We report a 28-year-old white female who suffered significant aortic insufficiency (AI) following mitral valve (MV) replacement for endocarditis. The patient had history of rheumatoid arthritis and presented to our emergency department with a 3-month history of dyspnea, orthopnea, fevers and weight loss, worsening over 2 weeks, for which she took intermittent acetaminophen. On admission, vital signs revealed blood pressure of 99/70 mm Hg, heart rate of 120 beats/minute, and temperature of 98.8 °F; her weight was 100 lbs. Physical exam revealed a thin and pale female. Cardiac auscultation revealed regular tachycardic rhythm with a third heart sound, and a short early systolic murmur at the left lower sternal border without radiation. Lungs revealed right lower lobe rhonchi. Initial pertinent laboratory evaluation revealed hemoglobin 9.6 g/dL and white blood cell count 17,500/μL. Renal function was normal, and hepatic enzymes were mildly elevated. Chest radiogram revealed right lower lobe infiltrate. Blood cultures revealed Enterococcus faecalis. Two-dimensional echocardiogram revealed large multilobed vegetation attached to the anterior MV leaflet with severe mitral regurgitation (MR), otherwise normal left ventricular systolic function. She was started on appropriate antibiotics and underwent MV replacement with 25-mm On-X prosthesis. She was noted post-operatively to have prominent systolic and diastolic murmurs. Repeat echocardiogram revealed normal mitral prosthesis function, with new moderately severe AI. Transesophageal echocardiogram revealed AI originating from a tethered non-coronary cusp, due to a suture preventing proper cusp mobility. The patient declined further surgery. She recovered slowly and was discharged to inpatient rehabilitation 4 weeks later. This case highlights the importance of vigilance to this potential serious complication of valve surgery with regard to diagnosis and treatment to prevent long-term adverse consequences. "}, {"id": "InternalMed_Harrison_17969", "title": "InternalMed_Harrison", "score": 0.008928571428571428, "content": "TREATMEnT ManageMent of aV conduction Block"}, {"id": "pubmed23n0618_7426", "title": "Delayed vascular injury and severe respiratory distress as a rare complication of a central venous catheter and total parenteral nutrition.", "score": 0.008849557522123894, "content": "Complications related to central venous catheters (CVCs) in the postoperative period can be fatal. We recently had a case of bilateral pleural effusion and respiratory distress caused by delayed vascular injury. A 79-y-old Japanese woman was admitted to our hospital because of advanced gastric carcinoma. A multiple-lumen CVC was placed through the left subclavian vein 1 d before surgery for postoperative nutritional management. The patient suddenly complained of dyspnea, and the chest X-ray film revealed right massive pleural effusion. Although the patient's symptoms soon disappeared after the thoracentesis, she again developed severe respiratory distress, and an endotracheal intubation was performed and her respiration was managed by mechanical ventilation. Computed tomographic scan of the chest revealed a displacement of the tip of the CVC out of the wall of the superior vena cava, mediastinitis, and leakage of intravenous fluid, which may have been caused by delayed vascular injury due to the CVC. The CVC was removed immediately after the diagnosis of delayed vascular injury at 10 d after surgery. The patient soon recovered with conservative treatment and was discharged from the hospital 43 d after surgery. This case highlights an extremely rare presenting complication of CVC placement and total parenteral nutrition."}, {"id": "pubmed23n0210_9490", "title": "Differential diagnosis of hypercalcaemia and indications for treatment.", "score": 0.008849557522123894, "content": "Primary HPT is a common medical problem of the middle-aged and elderly. The diagnosis is generally simple and seldom requires elaborate studies. The precise consequences of mild-to-moderate virtually asymptomatic disease are not clarified and provided there is regular follow-up attendance expectancy may be justified. In most patients, however, the experienced surgeon will have a more than 90% chance of success at the first exploration, which for the patient is a fairly safe procedure."}, {"id": "pubmed23n0721_1962", "title": "Indications and results for the Exogen™ ultrasound system in the management of non-union: a 59-case pilot study.", "score": 0.008771929824561403, "content": "This pilot series sought to assess the use of external ultrasound stimulation (Exogen™) in the treatment of femoral or tibial non-union. A continuous retrospective study was conducted from 2004 to 2009. It included patients with a non-united fracture or osteotomy at 6 months or more post-surgery, with less than 10mm inter-fragment gap. Daily 20-min ultrasound sessions were continued until bone healing was achieved or for a maximum 6-month duration. Radio-clinical control was performed at months 3 and 6; treatment compliance and transmitter positioning were checked at each follow-up visit. Sixty non-unions were included in the series. One patient was excluded for early material breakage. Mean fracture-to-surgery interval was 271 days. The 6-month consolidation rate was 88%. There was no loss to follow-up. Mean ultrasound treatment duration was 151 days (range, 90-240 days). Bone healing correlated significantly with stability of the internal fixation assembly (P=0.01). The seven cases of failure included four fixations,considered unstable at inclusion, one femoral non-union associated with BMI 45 and one inadequate subchondral roughening (at the time of arthrodesis). There was a significant difference in delay to non-union treatment start between the groups with (251 days) and without (420 days) bone healing. The present results are in line with the literature. The main prognostic factors were fracture fixation stability, short time to treatment, and inter-fragment gap less than 10mm. Bone healing rates in the literature are around 80% for non-union treated at around 6 months, versus 60% for more than 12 months' delay. Factors such as gender, bone site, smoking, numbers of previous operations or type of osteosynthesis do not impact consolidation. External treatment offers an alternative to traditional surgery (graft, or bone-marrow concentrate or bone morphogenetic protein injection), provided that the fracture fixation is stable. Bone healing rates are better, and the procedure is non-invasive. External treatment results using ultrasound are similar to those using electromagnetic fields; the main difference lies in treatment session duration, which is 20 min for ultrasound, versus 3 hours for electromagnetic fields. Active patient commitment is vital, as the treatment is delivered at home, although the machine is equipped with a monitor to count treatment cycles. The 88% bone healing rate supports advocating first-line implementation in non-union of less than 10mm with stable osteosynthesis. This rate is higher than in traditional surgery, with a unit cost at least 60% lower: €1772 for external therapy, versus €4480 for decortication with or without fracture fixation exchange (itemized 08c50 under the French healthcare treatment coding system). Level IV. Retrospective therapeutic study."}, {"id": "pubmed23n0746_21359", "title": "Reducing cardiovascular mortality in chronic kidney disease: something borrowed, something new.", "score": 0.008771929824561403, "content": "CLINICAL VIGNETTE: A 48-year-old man with chronic kidney disease stage five due to type II diabetes mellitus and hypertension was referred for hemodialysis initiation. His physical exam showed a blood pressure of 150/80, normal fundi, a positive fourth heart sound (S4), and trace pedal edema. Moderate aortic calcification was present on prior chest X-ray. The ECG showed left ventricle hypertrophy by voltage and slight prolongation of the QT interval. Medications included chlorthalidone, amlodipine, carvedilol, cholecalciferol, erythropoietin, and a phosphate binder. What additional therapy should be initiated to reduce vascular calcifications and cardiovascular mortality?"}, {"id": "wiki20220301en200_11479", "title": "Meniscus tear", "score": 0.008695652173913044, "content": "There are three phases that follow meniscal surgery. Each phase consists of rehabilitation goals, exercises, and criteria to move on to the next phase. Phase I starts immediately following surgery to 4–6 weeks or until the patient is able meet progression criteria. The goals are to restore normal knee extension, reduce and eliminate swelling, regain leg control, and protect the knee (Fowler, PJ and D. Pompan, 1993). During the first 5 days following the surgery, a passive continuous motion machine is used to prevent a prolonged period of immobilization which leads to muscular atrophy and delays functional recovery. During the 4–6 weeks post-surgical, active and passive non-weight bearing motions which flex the knee up to 90° are recommended. For patients with meniscal transplantation, further knee flexion can damage the allograft because of the increased shear forces and stresses. If any weight-bearing exercises are applied, a controlled brace should be worn on the knee to keep the"}, {"id": "InternalMed_Harrison_21076", "title": "InternalMed_Harrison", "score": 0.008695652173913044, "content": "500–800 mg, followed by continuous infusion at 2–5 mg/min) is now rarely used in this setting but may be tried for persisting, hemodynamically stable arrhythmias. Intravenous calcium gluconate is no longer considered safe or necessary for routine administration. It is used only in patients in whom acute hyperkalemia is known to be the triggering event for resistant VF, in the presence of known hypocalcemia, or in patients who have received toxic doses of calcium channel antagonists. Cardiac arrest due to bradyarrhythmias or asystole (B/A cardiac arrest) is managed differently (Fig. 327-3B). The patient is promptly intubated, CPR is continued, and an attempt is made to control hypoxemia and acidosis and identify other reversible causes. Epinephrine may be given intravenously or by an intraosseous route. Atropine is no longer considered effective for asystole or PEA, but can be used for bradyarrhythmias. External pacing devices are used to attempt to establish a regular rhythm when"}, {"id": "pubmed23n0277_19971", "title": "[A case of spontaneous rupture of the ascending aorta].", "score": 0.008620689655172414, "content": "We report a rare case of spontaneous rupture of the ascending aorta without any evidence of aneurysm formation or aortic dissection. A woman aged 64 was admitted to our cardiac care unit as an emergency patient with severe chest pain. Her face was pale and systolic blood pressure was 70 mmHg in spite of intravenous administration of dopamine (10 micrograms/kg/min). She had a history of hypertension for two years under good medical control. No trace of the chest trauma was noted before her admission. Physical examination revealed neck vein engorgement and distant heart sounds. Chest X-ray film showed enlargement of the cardiac silhouette. ECG showed no evidence of acute coronary syndrome. Pericardial effusion with a floating hematoma-like mass was detected by 2-dimensional echocardiogram. Pericardiocentesis revealed bloody pericardial fluid (Ht: 26%). Aortagraphy was performed resulting in a clinical diagnosis of acute aortic dissection, but there were no signs of a false lumen, aneurysm formation or extravasation of the contrast medium. Although continuous pericardial drainage was performed, she suddenly lost consciousness, collapsed and died. A longitudinal intimal laceration 5 cm long was observed in the ascending aorta. Pathological examination revealed cystic medial necrosis and irregularity of the elastic fibers in the media. No atheromatous plaque was noted in the intima. Spontaneous rupture of the aorta is a life-threatening condition that requires urgent surgery.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0755_2271", "title": "Vitamin D toxicity presenting as hypercalcemia and complete heart block: An interesting case report.", "score": 0.008620689655172414, "content": "Vitamin D deficiency is widely prevalent across the globe. This has lead to widespread use of vitamin D supplements in populations. We present our experience of vitamin D toxicity in a subject resulting in hypercalcemia and CHB (Complete Heart Block). A 70-year-old female, known hypertensive for thirty five years and diabetic for seven years underwent total knee replacement (TKR) for osteoarthritis left knee in December 2010. For perioperative glycemic control, multiple subcutaneous injections of insulin were advised. Patient later presented with poor glycemic control, decreased appetite and constipation for last 1 month with history of episodes of transient loss of consciousness for 15 days and recurrent vomiting. Biochemical work-up showed hypercalcemia (Serum calcium 12.4 mg/dL). Sr. albumin, ALP, Sr. phosphorus and PTH levels were normal, thus suggesting PTH independent hypercalcemia. Strong suspicion led us to check vitamin D levels in dilution which were 2016 ng/mL, thus confirming vitamin D toxicity. Retrospective analysis of treatment history revealed patient receiving 4 injections of Architol (6 Lac units im) prior to presentation. Work-up for malignancy was negative, brain imaging and EEG were normal. Holter was suggestive of intermittent CHB. Patient was given hydration, injection calcitonin 100 I.U. subcutaneously, injection pamidronate 60 mg infusion, with serum calcium levels normalizing, with relief in constipation, vomiting and behavioral improvement. However, persistence of rhythm disturbances led to permanent pacemaker placement. The present case highlights the dangers of indiscriminate vitamin D usage, exposing patients to potentially life threatening complications."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 83]], "word_ranges": [[0, 14]], "text": "BI-RADS Breast Imaging Reporting and Data System. BI-RADS 3 is defined by answer 3."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "BI-RADS Breast Imaging Reporting and Data System. BI-RADS 3 is defined by answer 3.", "full_answer_no_ref": "BI-RADS Breast Imaging Reporting and Data System. BI-RADS 3 is defined by answer [HIDDEN].", "full_question": "A 40-year-old woman consults because she has noticed a lump in the superoexternal quadrant of the right breast for the past month. She provides a mammography report describing a BIRADS 3 lesion. What is the best course of action?", "id": 592, "lang": "en", "options": {"1": "Reassure him, since an imaging test has already been done and malignancy has been ruled out.", "2": "This classification probably implies surgery since the probability of cancer is greater than 10%. He explains it to you and refers you preferentially to the Breast Unit.", "3": "This is a probably benign finding, since there is less than a 2% chance of cancer. He explains that it requires follow-up every 6-12 months until 24 months or a biopsy.", "4": "The findings are of low suspicion of cancer (between 2 and 10 %) but a biopsy is necessary.", "5": NaN}, "question_id_specific": 76, "type": "ONCOLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0424_10969", "title": "Evaluation of abnormal mammography results and palpable breast abnormalities.", "score": 0.01905453225660103, "content": "Because approximately 1 in 10 women with a breast lump or abnormal mammography result will have breast cancer, a series of decisions must be taken by a primary care practitioner to exclude or establish a diagnosis of breast cancer among these women. To determine the most accurate and least invasive means to evaluate an abnormal mammography result and a palpable breast abnormality. MEDLINE search (January 1966 to March 2003) for articles and reviews describing the accuracy of clinical examination, biopsy procedures, and radiographic examination for patients with abnormal mammography results or palpable breast abnormalities. The authors reviewed abstracts and selected articles that provided relevant primary data. Studies were included if 1) mammography, fine-needle aspiration biopsy, or core-needle biopsy was performed before a definitive diagnosis was obtained; 2) the study sample included 100 or more women; and 3) breast cancer status was determined from histopathology review of excisional biopsy specimens, from linkage with a state cancer registry or the Surveillance, Epidemiology, and End Results program, or from clinical follow-up of 95% or more of the study sample. One investigator abstracted results. Methods were evaluated for major potential biases, but methodologic scoring was not performed. Likelihood ratios for first screening mammography were 0.1 for the Breast Imaging Reporting and Data System (BI-RADS) assessment category \"negative or benign finding,\" 1.2 for \"probably benign finding,\" 7 for \"need additional imaging evaluation,\" 125 for \"suspicious abnormality,\" and 2200 for \"highly suggestive of malignancy.\" For fine-needle aspiration biopsy of a palpable lump performed by formally trained physicians, the likelihood ratio was infinity for an assessment of \"malignant,\" 2.6 for \"atypical/suspicious,\" and 0.02 for \"benign.\" When diagnostic mammography was used to evaluate a palpable lump or nonpalpable breast abnormality, the positive likelihood ratios were 5.6 and 9.4, and the negative likelihood ratios were 0.15 and 0.19, respectively. Women whose screening mammography results are interpreted as \"suspicious abnormality\" or \"highly suggestive of malignancy\" have a high risk for breast cancer and should undergo core-needle biopsy or needle localization with surgical biopsy. Women whose screening mammography results are interpreted as \"need additional imaging evaluation\" have a moderate risk for breast cancer and should undergo diagnostic mammography or ultrasonography to decide whether a nonpalpable breast lesion should be biopsied. Women whose screening mammography results are interpreted as \"probably benign finding\" have a low risk for breast cancer and can undergo follow-up mammography in 6 months. Either fine-needle aspiration biopsy or ultrasonography is recommended as the first diagnostic test of a palpable breast abnormality to distinguish simple cysts from solid masses. Fine-needle aspiration biopsy also allows characterization of a solid mass. Diagnostic mammography does not help determine whether a palpable breast mass should be biopsied and should not affect the decision to perform a biopsy."}, {"id": "wiki20220301en012_128008", "title": "Mammography", "score": 0.015033105394551178, "content": "The importance of these missed cancers is not clear, particularly if the woman is getting yearly mammograms. Research on a closely related situation has shown that small cancers that are not acted upon immediately, but are observed over periods of several years, will have good outcomes. A group of 3,184 women had mammograms that were formally classified as \"probably benign\". This classification is for patients who are not clearly normal but have some area of minor concern. This results not in the patient being biopsied, but rather in having early follow up mammography every six months for three years to determine whether there has been any change in status. Of these 3,184 women, 17 (0.5%) did have cancers. Most importantly, when the diagnosis was finally made, they were all still stage 0 or 1, the earliest stages. Five years after treatment, none of these 17 women had evidence of re-occurrence. Thus, small early cancers, even though not acted on immediately, were still reliably"}, {"id": "wiki20220301en185_5771", "title": "Breast cancer screening", "score": 0.012698412698412698, "content": "Mammography Mammography is a common screening method, since it is relatively fast and widely available in developed countries. Mammography is a type of radiography used on the breasts. It is typically used for two purposes: to aid in the diagnosis of a woman who is experiencing symptoms or has been called back for follow-up views (called diagnostic mammography), and for medical screening of apparently healthy women (called screening mammography). Mammography is not very useful in finding breast tumors in dense breast tissue characteristic of women under 40 years. In women over 50 without dense breasts, breast cancers detected by screening mammography are usually smaller and less aggressive than those detected by patients or doctors as a breast lump. This is because the most aggressive breast cancers are found in dense breast tissue, which mammograms perform poorly on."}, {"id": "pubmed23n0551_10377", "title": "Follow-up recommendations for benign breast biopsies.", "score": 0.011915706102821583, "content": "Histologically proven benign breast disease increases a woman's relative risk for subsequent cancer development. Yet follow-up guidelines for mammogram and clinical breast examination after a benign breast biopsy are lacking. Our objective was to determine if increased surveillance is indicated following a benign breast biopsy. Following institutional review board approval, a retrospective database review was conducted of prospectively gathered patients who had a benign breast biopsy (core or excisional) for an abnormality detected on mammogram, ultrasound, or clinical breast examination. Follow-up, for all subjects, was a clinical breast examination and mammogram or ultrasound at 6 months, 1 year, and 2 years after benign breast biopsy by a breast surgeon. End points were the need for additional biopsies or cancer detection. Statistical analysis was performed using chi-squared analysis. From January 2000 to July 2003, 156 patients age 18-86 years had a benign breast biopsy. During the 2 year follow-up, 20 patients (13%) required a subsequent biopsy. No significant difference was observed in mean age, race, menarche, menopause, parity, age at first live birth, use of oral contraceptives, history of prior biopsy, or the pathology of the initial lesion between those who needed a subsequent biopsy and those who did not. Seven excisional biopsies were performed (one at 6 months, four at 1 year, and two at 2 years follow-up) for growth of the benign breast biopsy lesion, and pathology remained concordant with the original diagnosis. Thirteen biopsies were done for new findings on mammogram or ultrasound. Three of these (1.9%) yielded a cancer diagnosis (one at 6 months, one at 1 year, and one at 2 years follow-up). No new lesions were identified on follow-up by clinical breast examination alone. Increased surveillance following a benign breast biopsy is necessary because of the increased need for subsequent biopsy or risk of cancer development. This should include imaging (mammography or ultrasound) and a clinical breast examination 6 months, 1 year, and 2 years after a benign breast biopsy."}, {"id": "article-18585_3", "title": "Breast Imaging Reporting and Data System -- Issues of Concern", "score": 0.011123136123136123, "content": "The final assessment includes the BI-RADS 0 to 6 categorization. A category assessment of BI-RADS 0 refers to an incomplete evaluation with further imaging required including additional mammographic views including spot compression or magnification and or ultrasound. BI-RADS 1 refers to a negative examination, meaning that there are no masses, suspicious calcifications or areas of architectural distortion. There can be no description of a finding in the report if it is categorized as a BI-RADS 1.  BI-RADS 2 is consistent with benign findings. Benign findings include secretory calcifications, simple cysts, fat-containing lesions, calcified fibroadenomas, implants and intramammary lymph nodes. BI-RADS 3 is probably benign and should have shortened interval follow-up to determine stability. The risk of malignancy is below 2%. There are very strict classifications to qualify a finding in the BI-RADS 3 category: a non-palpable, circumscribed mass on a baseline mammogram; a focal asymmetry, which becomes less dense on spot compression images, or a solitary group of punctate calcifications. Any findings other than this cannot be placed in the category 3. BI-RADS 4 is a suspicious abnormality, which can represent the chance of being malignant (in percent). The BI-RADS category 4 is subdivided into a, b, and c. The subcategory of (a) has a low probability of malignancy with a 2% to 10% chance of malignancy. The subcategory of (b) has an intermediate change of malignancy ranging from 10% to 50%. The subcategory of (c) has a high probability of malignancy ranging from 50% to 95%. BI-RADS 5 is highly suggestive of malignancy more than 95%. If something is placed in this classification and the pathology comes back as benign, the recommendation is still surgical consultation, because the pathology is discordant with the radiographic findings. The last category that was recently added is the BI-RADS 6, used for pathology proven malignancy."}, {"id": "wiki20220301en185_5777", "title": "Breast cancer screening", "score": 0.011083451872925556, "content": "The accidental harm from screening mammography has been underestimated. Women who have mammograms end up with increased surgeries, chemotherapy, radiotherapy and other potentially procedures resulting from the over-detection of harmless lumps. Many women will experience important psychological distress for many months because of false positive findings. Half of suspicious findings will not become dangerous or will disappear over time. Consequently, the value of routine mammography in women at low or average risk is controversial. With unnecessary treatment of ten women for every one woman whose life was prolonged, the authors concluded that routine mammography may do more harm than good. If 1,000 women in their 50s are screened every year for ten years, the following outcomes are considered typical in the developed world:"}, {"id": "pubmed23n0315_1784", "title": "Investigation of lesions detected by mammography. The Steering Committee on Clinical Practice Guidelines for the Care and Treatment of Breast Cancer. Canadian Association of Radiation Oncologists.", "score": 0.009900990099009901, "content": "To provide information and recommendations to facilitate decision-making when a mammographic abnormality is detected by screening. References identified by use of MEDLINE, AIDSLINE, CANCERLIT and reference lists of review articles to December 1996. Where experimental evidence is lacking, recommendations are based on expert opinion. The evidence is graded accordingly in \"levels\" (page S2). Exclusion or confirmation of the presence of cancer with minimum intervention and delay. When an abnormality is detected on screening mammography, clinical evaluation and thorough radiologic work-up are needed to determine its significance. Clinical evaluation should include a history and a thorough examination of the breast, axilla and supraclavicular areas. In the radiologic work-up, diagnostic mammograms should be obtained with additional views, spot compression and magnification views as appropriate. Current mammograms should be compared with previous mammograms whenever possible. The mammographic report should include a precise description of the abnormal features visualized and an estimate of the level of suspicion of cancer they imply. Whenever there is any doubt in the interpretation of mammograms, the interpretation of 2 experienced readers should be obtained. (The following radiologic classification into 4 categories is suggested: 1--benign, not due to cancer; 2--low risk, probability of cancer under 2%; 3--intermediate risk, probability of cancer 2% to 10%; 4--high risk, probability of cancer over 10%.) Ultrasonography can be used to clarify the nature of noncalcified nodular lesions. Management decisions require close communication between the woman and her physicians. Throughout, a clinician in charge should be identified who will coordinate and transmit all decisions. Management will depend on the estimated level of risk Category 1 abnormalities require no further investigation. Category 2 abnormalities may be followed up by periodic mammographic and clinical examinations. Follow-up examination of category 2 abnormalities should be carried out at approximately 6 and 12 months. If the abnormality is stable, examination should be repeated annually for 2 to 3 years thereafter. The rationale of follow-up should be explained, and women should be made aware that it is not possible to provide complete assurance that an abnormality is benign. Category 3 abnormalities usually require image-guided fine-needle or core biopsy. Every image-guided needle biopsy should be accompanied by a full report. Category 4 abnormalities should usually be excised. This may be preceded by image-guided needle biopsy. When surgical biopsy is carried out, the margins of the resected specimen must be free of tumour. The intact pathology specimen should be examined radiographically to confirm that all mammographic abnormalities have been removed. The patient should be kept fully informed as to the reason for each test and the meaning of its results. The process, from initial detection of the mammographic abnormality to the final management decision, should be completed as rapidly as possible. Guidelines were reviewed and revised by the Writing Committee, expert primary reviewers, secondary reviewers selected from all regions of Canada and by the Steering Committee. The final document reflects a consensus of all these contributors."}, {"id": "pubmed23n0247_4207", "title": "[The importance of mammography in relationship to the number of treated carcinomas of the breast. Partial results from 1976--1978 of a long term study (author's transl)].", "score": 0.009900990099009901, "content": "From 1976 to 1978 11, 197 women were examined clinically and mammographically. Biopsy material from 1,673 breasts were examined microscopically. In 536 cases, or almost every third case (32%), a carcinoma of the breast was detected. The cancer was bilateral in 19 cases and the total number of women was therefore 517. A clinically occult tumour was only found in 7.7% (40 of 517) of the cases. 5% of these patients were high risk patients and 2.7% preventive examinations. 5 women with occult carcinoma of the breast were under age 40 and 14 under age 50. Benign changes of the glandular tissue were found in 59.5% of the cases. Marked proliferative changes were found in 4.6% of the cases and carcinoma in situ was found in 3.8% of the patients. In the age group 45--54 benign and proliferative changes of the parenchyma occured almost twice as often as cancers. The ratio between benign and malignant findings was 1:1 in the age group 55--59 and was less than 1:2 in the age group over 70. A sophistication of the mammograhic technique must be obtained. A thorough microscopic examination of tissue from subcutaneous mastectomies and tissue obtained at the time of reduction mammoplasties showed occasionally unexpected malignant tissue in an unexpected location. Especially these cases are suitable for later comparison to the mammographies."}, {"id": "pubmed23n0357_10958", "title": "Atypical medullary carcinoma of the breast with cartilaginous metaplasia in a patient with a BRCA1 germline mutation.", "score": 0.00980392156862745, "content": "We examined a 34-year-old premenopausal woman who had noticed a left-breast lump a month previously. She had no past history of malignancies but had a family history of breast and ovarian cancers. Her mother had suffered from ovarian cancer when aged 47 years and had died of the disease at age 52. The younger two of the patient's four aunts had developed breast cancer when they were 37 and 48 years old. A physical examination showed an ill-defined mass, 1.5 cm in diameter, located in the upper outer quadrant of the patient's left breast. Mammography revealed diffuse microcalcification in both breasts but ultrasonography revealed an irregular tumorous lesion only in the left breast. Aspiration breast cytology revealed adenocarcinoma of the left breast. Modified radical mastectomy of the left breast and excision of a biopsy specimen from the right breast were carried out simultaneously. Histopathologically the left-breast tumor was an atypical medullary carcinoma with cartilaginous metaplasia, of histological grade 3, and the right-breast specimen showed fibrocystic changes with atypical ductal hyperplasia. Estrogen receptors were positive, but progesterone receptor was not detected on the tumor cells, which were immunopositive for nuclear p53 although c-erbB-2 overexpression was not observed. A nonsense germline mutation of the BRCA1 gene (exon5) was detected. The patient has been well since the operation (10 months). These findings may provide useful information about the carcinogenesis and biological behavior of BRCA1-associated breast cancers."}, {"id": "pubmed23n0900_19235", "title": "How Many of the Biopsy Decisions Taken at Inexperienced Breast Radiology Units Were Correct?", "score": 0.00980392156862745, "content": "In this study, we aimed to determine the need for biopsy in patients referred from other clinics for the performance of biopsy with the suspicion of breast cancer. 112 patients were included in the study. It was decided that their biopsies be performed following examinations in other clinics and they presented to the breast radiology unit of our hospital for a second opinion. The demographic characteristics, diagnostic studies completed in the other centers, properties of lesions, decision made as a result of examinations and BI-RADS (Breast Imaging Reporting and Data Systems) categorizations were recorded on the registration forms of the study patients. In addition, the quality of examinations, reasons of repeat tests, additional tests features and the last decision of our clinic were documented. The obtained data were analyzed in terms of re-examination, additional tests and change in the biopsy decision. Changes in the biopsy decisions for patients were specifically inquired. The biopsy decisions were cancelled in our breast radiology unit for 63 out of 112 patients (56.3%) whose biopsy decisions were made at an external institute. For 42 patients, examinations made by the other clinics were deemed adequate, yet there was no need for biopsy in 22 of them. The biopsy decisions were cancelled for 27 out of 47 patients (57.4%) with repeat examination and 18 out of 28 patients (64.3%) with additional tests because of the insufficient test quality. Incorrect, inadequate breast screening and false positivity were higher at inexperienced institutes."}, {"id": "pubmed23n0694_7473", "title": "A case of quadruple primary malignancies including breast, tongue, and thyroid cancers and osteosarcoma in a young female without karyotype abnormality.", "score": 0.009708737864077669, "content": "The patient was a 41-year-old, premenopausal woman with a chief complaint of well-circumscribed palpable, right breast mass without nipple discharge. Although she noticed the lump 3 months previously, the size of the tumor (1.1 × 0.9 cm(2)) had been stable. The patient's mother suffered from gastric cancer. Her previous history of the triple different malignancies was as follows: (1) left osteosarcoma [amputation of left lower leg at 15 years old (y/o)]. After the operation, she was treated with various kinds of anticancer drugs including a total of 45 g ifosphamide and 342 g methotrexate; (2) tongue cancer (right radical neck resection; 23 y/o); and (3) thyroid cancer (right lobectomy; 40 y/o). There was no evidence of recurrence of these malignancies at the present consultation. At the time of tongue cancer operation, chromosome abnormality was investigated, but the results were normal. Physical examination showed a well-delimited, elastic-firm, mobile tumor in the central outer right breast. Regional lymph nodes were not palpable. Mammography showed a focal asymmetry in the right upper breast on the mediolateral oblique view. Ultrasonography revealed a hypoechoic mass with irregular margins. Distant metastases could not be detected by whole-body computed tomography scan. The histology of the Mammotome(®) (vacuum-assisted core needle biopsy) specimen revealed that this tumor was low-grade ductal carcinoma in situ (DCIS). She underwent breast-conserving surgery with sentinel lymph node biopsy. On permanent histopathological examination, the diagnosis of the tumor was intracystic papilloma with low-grade DCIS. Surgical margin was negative, and sentinel lymph node metastases could not be observed. Estrogen and progesterone receptor (ER/PR) were strongly positive, but human epidermal growth factor receptor-2 (HER-2) overexpression was not tested because the lesion was DCIS. She has received no adjuvant therapy and is currently disease free 3 months after surgery."}, {"id": "wiki20220301en140_26841", "title": "BI-RADS", "score": 0.009615384615384616, "content": "BI-RADS Assessment Categories are: 0: Incomplete 1: Negative 2: Benign 3: Probably benign 4: Suspicious 5: Highly suggestive of malignancy 6: Known biopsy – proven malignancy An incomplete (BI-RADS 0) classification warrants either an effort to ascertain prior imaging for comparison or to call the patient back for additional views and/or higher quality films. A BI-RADS classification of 4 or 5 warrants biopsy to further evaluate the offending lesion. Some experts believe that the single BI-RADS 4 classification does not adequately communicate the risk of cancer to doctors and recommend a subclassification scheme: 4A: low suspicion for malignancy, about > 2% to ≤ 10% likelihood of malignancy 4B: intermediate suspicion of malignancy, about > 10% to ≤ 50% likelihood of malignancy 4C: moderate concern, but not classic for malignancy, about > 50% to < 95% likelihood of malignancy"}, {"id": "pubmed23n0920_10787", "title": "Breast screening: What can the interval cancer review teach us? Are we perhaps being a bit too hard on ourselves?", "score": 0.009615384615384616, "content": "The aim of this study was to determine the features that make interval cancers apparent on the preceding screening mammogram and determine whether changes in the ways of performing the interval cancer review will affect the true interval cancer rate. This study was approved by the clinical governance committee. Mammograms of women diagnosed with an interval cancer were included in the study if they had been allocated to either the \"suspicious signs\" group or \"subtle signs\" group, during the historic interval cancer review. Three radiologists, individually and blinded to the site of interval cancer, reviewed the mammograms and documented the presence, site, characteristics and classification of any abnormality. Findings were compared with the appearances of the abnormality at the site of subsequent cancer development by a different breast radiologist. The chi-squared test was used in the analysis of the results, seeking associations between recall concordance and cancer mammographic or histological characteristics. 111/590 interval cancers fulfilled the study inclusion criteria. In 17% of the cases none of the readers identified the relevant abnormality on the screening mammogram. 1/3 readers identified the relevant lesion in 22% of the cases, 2/3 readers in 28% of cases and all 3 readers in 33% of cases. The commonest unanimously recalled abnormality was microcalcification and the most challenging mammographic abnormality to detect was asymmetric density. We did not find any statistically significant association between recall concordance and time to interval cancer, position of lesion in the breast, breast density or cancer grade. Even the simple step of performing an independent blinded review of interval cancers reduces the rate of interval cancers classified as missed by up to 39%."}, {"id": "wiki20220301en393_20908", "title": "Triple test score", "score": 0.009523809523809525, "content": "The triple test score (TTS) is a diagnostic tool for examining potentially cancerous breasts. Diagnostic accuracy of the triple test score is nearly 100%. Scoring includes using the procedures of physical examination, mammography and needle biopsy. If the results of a TTS are greater than five, an excisional biopsy is indicated. Scoring To obtain the triple test score, a number from 1 through 3 is assigned to each one of the procedures. A score of 1 is assigned to a benign test result, 2 applies to a suspicious test result, and 3 applies to a malignant result. The sum of the scores of all three procedures is the triple test score. A score of 3 to 4 is most likely benign, whereas a score of greater than 6 is possibly malignant. References Breast cancer"}, {"id": "pubmed23n0509_8108", "title": "[Re-evaluating the role of breast ultrasound in current diagnostics of malignant breast lesions].", "score": 0.009523809523809525, "content": "New evaluation of breast ultrasound based upon review of new literature comparing ultrasound and mammography. Description and discussion of the published trials regarding breast imaging methods. Breast ultrasound is the preferable method in the case of a symptomatic patient (after clinical examination). In the case of a patient without symptoms (screening), breast ultrasound is ascribed a higher sensitivity for detecting breast cancer in women with dense breast tissue, women under the age of 50 and high-risk women. Mammographically occult cancers can be detected by sonography in 10 to 40 % of the cases depending on the patient's breast density and age. The mean size of cancers detected only by ultrasound is not significantly different to that only detected by mammography. The prevalence of breast cancers detected by ultrasound is approximately equal to the one detected by mammography, regarding the total number of examined patients. Breast ultrasound should be the preferred imaging procedure in the case of a palpable lump, leading to a definitive diagnosis itself or with an additional consecutive core needle biopsy. For women without symptoms, breast sonography should be mandatory and complementary to mammography in the case of breast density grade II (BI-RADS) or more. Application of breast ultrasound as a primary method or an alternative to mammography has not yet been evaluated sufficiently. It seems advisable in the case of women with dense breast tissue grade III and IV, women under the age of 50 and high-risk women. The implementation of breast ultrasound in this manner has to be checked by future trials."}, {"id": "Surgery_Schwartz_2203", "title": "Surgery_Schwartz", "score": 0.009458662143225901, "content": "The consequences of a false-positive screening test result also need to be considered. For example, when 1000 screening mammograms are taken, only 2 to 4 new cases of cancer will be identified; this number is slightly higher (6 to 10 prevalent cancers per 1000 mammograms) for initial screen-ing mammograms.106 However, as many as 10% of screening mammograms may be potentially suggestive of an abnormal-ity, which requires further imaging (i.e., a 10% recall rate). Of those women with abnormal mammogram findings, only 5% to 10% will be determined to have a breast cancer. Among women for whom biopsy specimen is recommended, 25% to 40% will have a breast cancer. A false-positive screening result is likely to induce significant emotional distress in patients, leads to unnecessary biopsy specimens, and has cost implications for the health care system.American Cancer Society guidelines for the early detec-tion of cancer are listed in Table 10-9.96 These guidelines are updated periodically to"}, {"id": "pubmed23n0535_1319", "title": "Proliferative high-risk lesions of the breast: contribution and limits of US-guided core biopsy.", "score": 0.009433962264150943, "content": "To retrospectively correlate high-risk proliferative breast lesions (radial scar, atypical lobular hyperplasia, lobular carcinoma in situ and papillary lesions) diagnosed on core biopsy with the definitive histopathological diagnosis obtained after surgical excision or with the follow-up, in order to assess the role of core biopsy in such lesions. To discuss the management of the patient after a core biopsy diagnosis of high-risk proliferative breast lesion. We evaluated 74 out of 1776 core biopsies consecutively performed on 67 patients. The histopathologic findings were as follows: 11 radial scars (RS), 3 atypical lobular hyperplasias (ALH), 3 lobular carcinomas in situ (LCIS), 57 benign papillary lesions. All patients underwent bilateral mammography, whole-breast ultrasound with a linear-array broadband transducer, and core biopsy with a 14 Gauge needle and a mean number of samples of 5 (range 4-7). Sixty-two of 67 patients, for a total of 69/74 lesions, underwent surgical biopsy despite benign histopathologic findings, mostly because of highly suspicious imaging for malignancy (BIRADS 4-5), whereas 5 patients refused surgery and have been followed up for a least 18 months and are still being followed up (2 with RS, 1 with ADH and 2 with papillary lesions). Among the core biopsied lesions with a diagnosis of RS (n = 11) pathology revealed one ductal carcinoma in situ (DCIS) (this case was characterized by granular microcalcifications on mammography and by a mass with irregular margins on ultrasound). Also in the group of ADH (n = 3) pathology revealed one DCIS (lesion not visible on mammography but depicted as a suspicious mass on US). In the group of LCIS (n = 3) pathologists found an invasive lobular carcinoma (ILC). Among the benign papillary lesions (n = 57) histopathologic analysis of the surgical specimen revealed 7 malignant lesions (4 papillary carcinomas and 3 DCIS), whose mammographic and ultrasound findings were indistinguishable from benign lesions. Altogether there were 10 false negative results (underestimation) out of 74 core biopsies with a diagnosis of high-risk proliferative breast lesions. The high rate of histological underestimation after core biopsy (10/74) (13.5%) demands a very careful management of patents with a core biopsy diagnosis of high-risk proliferative breast lesions, especially in the case of RS, lobular neoplasia and papillary lesions. However, the high imaging suspicion for malignancy prompts surgery. It is possible to assume that, when there is a low imaging suspicion for malignancy, when enough tissue has been sampled for pathology and no atypia is found within the lesions, surgery is not mandatory but a very careful follow-up is recommended. We must underline that there is no agreement regarding the quantity of tissue to sample. Vacuum-assisted biopsy may lead to better results, although there is as yet no proof that it can actually replace surgery in this group of lesions, since it seems only to reduce but not abolish the histological underestimation."}, {"id": "pubmed23n0338_3002", "title": "[History of mammography].", "score": 0.009433962264150943, "content": "The History of mammography began in 1913, when a Berliner surgeon, A. Salomon realized a roentgeno-histological study on 3,000 mastectomies. This work is the basis of mammography. Until 1938, few articles were published but were of little help to mammography. From 1947 to 1970, the second period brought the results of roentgenologic and clinical correlation. R. Leborgne was the first accountable for the wide development of this method. Since 1951, many American and European radiologists brought their contribution. Ch. Gros is the best known. He gave this technique an acknowledgment throughout the world for the diagnosis of breast diseases. Since 1970, the third period emphasizes the value of mammography as a technique for detection of breast cancer. Some \"Screening working groups\" are being set up. The problem is mainly economical."}, {"id": "wiki20220301en012_127997", "title": "Mammography", "score": 0.009345794392523364, "content": "Often women are quite distressed to be called back for a diagnostic mammogram. Most of these recalls will be false positive results. Of every 1,000 U.S. women who are screened, about 7% will be called back for a diagnostic session (although some studies estimate the number to be closer to 10% to 15%). About 10 of these individuals will be referred for a biopsy; the remaining 60 cases are found to be of benign cause. Of the 10 referred for biopsy, about 3.5 will have cancer and 6.5 will not. Of the 3.5 who have cancer, about 2 will have an early stage cancer that will be cured after treatment."}, {"id": "wiki20220301en185_5774", "title": "Breast cancer screening", "score": 0.009264223370719051, "content": "If suspicious signs are identified in the image, then the woman is usually recalled for a second mammogram, sometimes after waiting six months to see whether the spot is growing, or a biopsy of the breast. Most of these will prove to be false positives, resulting in sometimes debilitating anxiety over nothing. Most women recalled will undergo additional imaging only, without any further intervention. Recall rates are higher in the U.S. than in the UK. Effectiveness On balance, screening mammography in older women increases medical treatment and saves a small number of lives. Usually, it has no effect on the outcome of any breast cancer that it detects. Screening targeted towards women with above-average risk produces more benefit than screening of women at average or low risk for breast cancer."}, {"id": "pubmed23n1009_24025", "title": "Toddler Sleep Challenges: All in a Day's Work.", "score": 0.009259259259259259, "content": "Leo is a 26-month-old boy who you are seeing for an urgent care visit due to \"sleep difficulty,\" particularly sleep onset. Since age 1, he screams, hits, and kicks his mother every day, starting after she gets home from work at 5 PM (or before the family's dinnertime on her days off) and escalating over the course of the evening until he \"wears himself out\" and falls asleep in a crib in his own room around 9 to 10 PM Once asleep, he sleeps well through the night and wakes easily around 7 AM in a pleasant mood; his mother leaves for work soon after he awakens. He naps after lunch for 2 to 3 hours on weekdays at an in-home child care with 1 to 2 adult caregivers and 5 other children aged 0 to 5 years. He refuses to nap at home.Leo goes to bed easily when his father puts him to bed if his mother is not at home, but his mother feels that evenings are the only time she can spend with Leo, and so, she tries to put him to bed most nights. However, because of Leo's behaviors at bedtime with her, she feels inadequate, depressed, and guilty; when she tries to disengage or allow her husband to help, Leo screams, \"Mommy, mommy!\" and tries to gain access to her and resists his father putting him to bed until his mother returns. Both parents worry that \"he would not grow out of this,\" and his mother now avoids coming home from work for fear of Leo's behavior. Both parents feel that this situation is causing marital strain.Leo was born healthy at full-term and is an only child; pregnancy was complicated by hyperemesis gravidarum. Leo has been healthy and meeting developmental milestones. His parents describe his temperament as \"like his father at that age,\" \"easy, but never able to self-soothe,\" \"intense\" in his emotional reactions, persistent, \"strong-willed and serious,\" and \"shy and observant, withdrawn at first and then getting more pleasant after a while\" in novel situations. Behaviorally, he engaged in noninjurious head-banging at home when upset between 12 and 15 months; bit children a few times at child care between 20 and 24 months; and lately refuses to share or will push other children at child care every few weeks. His parents recently read a book about parenting \"spirited\" children but did not find it helpful. What would you do next?"}, {"id": "pubmed23n0052_18930", "title": "[Errors in mammography. II. False positives].", "score": 0.009259259259259259, "content": "The authors evaluate 261 consecutive mammographic false positives observed from 1985 to 1987. Histological evidence of benign lesion followed in all cases. The comparison with the actual number of cancers and of the whole of mammographic examinations performed in the study period allowed specificity and positive predictive value of mammography to be assessed as 99.5% and 83%, respectively. Specificity and predictivity are lower in younger women, but this is more likely to depend on a different age-related incidence of cancer and benign lesions than on an intrinsic limitation of the method. The reader's diagnostic aggressivity, more than his experience, seems to affect both specificity and predictivity. At review, false positives were mostly due to asymmetric densities (49) or to circumscribed opacities with clear-cut (44) or blurred (62) outlines, whereas irregular star-like opacities or distortions (19) were infrequent. Microcalcifications were, in most cases, apparently benign (39) or dubious (76); strong suspicion was rare (4). Overall, one-fourth to one-third (27.9%) of the cases were reported as strongly suspicious at review. Palpation and cytology were also falsely suspicious--that is, co-responsible for unnecessary biopsies in over 50% of cases. Our results suggest that further improvement in the specificity or positive predictive value of mammography seems unlikely. Moreover, the benign/malignant biopsy ratio (0.2:1) presently achieved in suspicious mammographic cases appears quite satisfactory."}, {"id": "pubmed23n1152_829", "title": "A Single-Center Audit of BI-RADS 3 Assessment Category Utilization in Mammography and Breast Ultrasound.", "score": 0.009174311926605505, "content": "<bPurpose:</b To evaluate outcomes of breast lesions assessed at our institution as probably benign (Breast Imaging Reporting and Data System [BI-RADS] category 3) with an expected malignancy rate of less than or equal to 2 %. <bMethods:</b Average-risk women with a BI-RADS 3 assessment following mammographic and/or ultrasound evaluation at our institution between January 1 and December 31, 2017 were included. Cancer yield was calculated within 90 days and at 6-month intervals up to 36 months. <bResults:</b Among 517 women (median age, 52 years; range, 13-89 years) with a BI-RADS 3 assessment, 349 (67.5 %) underwent biopsy or completed follow-up imaging up to 36 months. One hundred and 68 (32.5 %) were lost to follow-up. Thirty of 349 (8.6 %) had their imaging upgraded and underwent biopsy, yielding six cancers (cancer yield, 6 of 349 women [1.7 %]). Among 569 lesions assessed as BI-RADS 3, 92 (16.2 %) were characterized by morphologic features other than those validated as probably benign in prospective clinical studies. Fifty three of 517 women (10.3 %) had follow-up beyond 24 months, and 24 (4.6 %) had follow-up beyond 36 months. <bConclusion:</b Overall utilization of the BI-RADS 3 assessment category at our institution is appropriate with a 1.7 % cancer yield. However, the rate of loss to follow-up, percentage of non-validated findings assessed as probably benign, and redundancy in follow-up protocols are too high, and warrant intervention. A patient handout explaining the BI-RADS 3 assessment category and automatic scheduling of follow-up studies have been implemented at our center to address loss to follow-up."}, {"id": "pubmed23n0280_22066", "title": "Interpreting the mammogram report.", "score": 0.009174311926605505, "content": "The standardization of imaging techniques, interpretation and reporting has become an important issue as the use of mammographic screening has increased. Each mammogram report should indicate whether the breast is fatty or consists of dense glandular tissue. A brief description of abnormalities should be followed by the conclusion and recommendations. Well-circumscribed lesions have a 98 percent benign rate; these lesions generally do not require biopsy but can be followed at six-month intervals for a period of time. The overall rate of malignancy for biopsies prompted by mammography is 20 to 35 percent. Lesions interpreted as highly suspicious are malignant in 75 to 90 percent of cases. Mammographically detected tumors are generally smaller than palpable tumors at the time of diagnosis. In addition, patients who have mammographically detected lesions are more often node-negative and therefore have a better prognosis than patients with palpable lesions."}, {"id": "pubmed23n0800_14851", "title": "Breathlessness with pulmonary metastases: a multimodal approach.", "score": 0.00909090909090909, "content": "Case Study  Sarah is a 58-year-old breast cancer survivor, social worker, and health-care administrator at a long-term care facility. She lives with her husband and enjoys gardening and reading. She has two grown children and three grandchildren who live approximately 180 miles away. SECOND CANCER DIAGNOSIS  One morning while showering, Sarah detected a painless quarter-sized lump on her inner thigh. While she thought it was unusual, she felt it would probably go away. One month later, she felt the lump again; she thought that it had grown, so she scheduled a visit with her primary care physician. A CT scan revealed a 6.2-cm soft-tissue mass in the left groin. She was referred to an oncologic surgeon and underwent an excision of the groin mass. Pathology revealed a grade 3 malignant melanoma. She was later tested and found to have BRAF-negative status. Following her recovery from surgery, Sarah was further evaluated with an MRI scan of the brain, which was negative, and a PET scan, which revealed two nodules in the left lung. As Sarah had attended a cancer support group during her breast cancer treatment in the past, she decided to go back to the group when she learned of her melanoma diagnosis. While the treatment options for her lung lesions included interleukin-2, ipilimumab (Yervoy), temozolomide, dacarbazine, a clinical trial, or radiosurgery, Sarah's oncologist felt that ipilimumab or radiosurgery would be the best course of action. She shared with her support group that she was ambivalent about this decision, as she had experienced profound fatigue and nausea with chemotherapy during her past treatment for breast cancer. She eventually opted to undergo stereotactic radiosurgery. DISEASE RECURRENCE  After the radiosurgery, Sarah was followed every 2 months. She complained of shortness of breath about 2 weeks prior to each follow-up visit. Each time her chest x-ray was normal, and she eventually believed that her breathlessness was anxiety-related. Unfortunately, Sarah's 1-year follow-up exam revealed a 2 cm × 3 cm mass in her left lung, for which she had a surgical wedge resection. Her complaints of shortness of breath increased following the surgery and occurred most often with anxiety, heat, and gardening activities, especially when she needed to bend over. Sarah also complained of a burning \"pins and needles\" sensation at the surgical chest wall site that was bothersome and would wake her up at night. Sarah met with the nurse practitioner in the symptom management clinic to discuss her concerns. Upon physical examination, observable signs of breathlessness were lacking, and oxygen saturation remained stable at 94%, but Sarah rated her breathlessness as 7 on the 0 to 10 Borg scale. The nurse practitioner prescribed duloxetine to help manage the surgical site neuropathic pain and to assist with anxiety, which in turn could possibly improve Sarah's breathlessness. Several nonpharmacologic modalities for breathlessness were also recommended: using a fan directed toward her face, working in the garden in the early morning when the weather is cooler, gardening in containers that are at eye level to avoid the need to bend down, and performing relaxation exercises with pursed lip breathing to relieve anxiety-provoked breathlessness. One month later, Sarah reported relief of her anxiety; she stated that the fan directed toward her face helped most when she started to feel \"air hungry.\" She rated her breathlessness at 4/10 on the Borg scale. SECOND RECURRENCE: MULTIPLE PULMONARY NODULES  Sarah's chest x-rays remained clear for 6 months, but she developed a chronic cough shortly before the 9-month exam. An x-ray revealed several bilateral lung lesions and growth in the area of the previously resected lung nodule. Systemic therapy was recommended, and she underwent two cycles of ipilimumab. Sarah's cough and breathlessness worsened, she developed colitis, and she decided to stop therapy after the third cycle. In addition, her coughing spells triggered bronchospasms that resulted in severe anxiety, panic attacks, and air hunger. She rated her breathlessness at 10/10 on the Borg scale during these episodes. She found communication difficult due to the cough and began to isolate herself. She continued to attend the support group weekly but had difficulty participating in conversation due to her cough. Sarah was seen in the symptom management clinic every 2 weeks or more often as needed. No acute distress was present at the beginning of each visit, but when Sarah began to talk about her symptoms and fear of dying, her shortness of breath and anxiety increased. The symptom management nurse practitioner treated the suspected underlying cause of the breathlessness and prescribed oral lorazepam (0.5 to 1 mg every 6 hours) for anxiety and codeine cough syrup for the cough. Opioids were initiated for chest wall pain and to control the breathlessness. Controlled-release oxycodone was started at 10 mg every 12 hours with a breakthrough pain (BTP) dose of 5 mg every 2 hours as needed for breathlessness or pain. Sarah noted improvement in her symptoms and reported a Borg scale rating of 5/10. Oxygen therapy was attempted, but subjective improvement in Sarah's breathlessness was lacking. END OF LIFE  Sarah's disease progressed to the liver, and she began experiencing more notable signs of breathlessness: nasal flaring, tachycardia, and restlessness. Opioid doses were titrated over the course of 3 months to oxycodone (40 mg every 12 hours) with a BTP dose of 10 to 15 mg every 2 hours as needed, but her breathlessness caused significant distress, which she rated 8/10. The oxycodone was rotated to IV morphine continuous infusion with patient-controlled analgesia (PCA) that was delivered through her implantable port. This combination allowed Sarah to depress the PCA as needed and achieve immediate control of her dyspneic episodes. Oral lorazepam was also continued as needed. Sarah's daughter moved home to take care of her mother, and hospice became involved for end-of-life care. As Sarah became less responsive, nurses maintained doses of morphine for control of pain and breathlessness and used a respiratory distress observation scale to assess for breathlessness since Sarah could no longer self-report. A bolus PCA dose of morphine was administered by Sarah's daughter if her mother appeared to be in distress. Sarah died peacefully in her home without signs of distress. "}, {"id": "pubmed23n0024_7159", "title": "Results of breast biopsies for mammographic findings.", "score": 0.00909090909090909, "content": "The results of breast biopsies for mammographic findings have been presented, in which 314 biopsies were done on 274 patients. From this number of biopsies, the diagnosis of cancer was established in fifty-seven cases (18 per cent of the biopsies). More than 50 per cent of the lesions were infiltrating duct cell carcinomas. The number of breast biopsies required increased markedly after the national publicity in 1974. As more biopsies were done, the incidence of carcinoma increased, and a significant number of these were found in women less than fifty years old. We believe this justifies the continued judicious use of mammography, even in the younger patient, if clinically indicated."}, {"id": "wiki20220301en003_102966", "title": "Breast cancer", "score": 0.009036796536796537, "content": "Emphasis In 2009 the US science journalist Christie Aschwanden criticized that the emphasis on breast cancer screening may be harming women by subjecting them to unnecessary radiation, biopsies, and surgery. One-third of diagnosed breast cancers might recede on their own. Screening mammography efficiently finds non-life-threatening, asymptomatic breast cancers and precancers, even while overlooking serious cancers. According to the cancer researcher H. Gilbert Welch screening mammography has taken the \"brain-dead approach that says the best test is the one that finds the most cancers\" rather than the one that finds dangerous cancers."}, {"id": "pubmed23n1033_16798", "title": "Cancer Yield and Patterns of Follow-up for BI-RADS Category 3 after Screening Mammography Recall in the National Mammography Database.", "score": 0.009009009009009009, "content": "Background The literature supports the use of short-interval follow-up as an alternative to biopsy for lesions assessed as probably benign, Breast Imaging Reporting and Data System (BI-RADS) category 3, with an expected malignancy rate of less than 2%. Purpose To assess outcomes from 6-, 12-, and 24-month follow-up of probably benign findings first identified at recall from screening mammography in the National Mammography Database (NMD). Materials and Methods This retrospective study included women recalled from screening mammography with BI-RADS category 3 assessment at additional evaluation from January 2009 through March 2018 from 471 NMD facilities. Only the first BI-RADS category 3 occurrence for women aged 25 years or older with no personal history of breast cancer was analyzed, with biopsy or 2-year imaging follow-up. Cancer yield and positive predictive value of biopsies performed (PPV3) were determined at each follow-up. Results Among 45 202 women (median age, 55 years; range, 25-90 years) with a BI-RADS category 3 lesion, 1574 (3.5%) underwent biopsy at the time of lesion detection, yielding 72 cancers (cancer yield, 4.6%; 72 of 1574 women). For the remaining 43 628 women who accepted surveillance, 922 were seen within 90 days (with 78 lesions biopsied and 12 [15%] classified as malignant). The women still in surveillance (31 465 of 43 381 women [72.5%]) underwent follow-up mammography at 6 months. Of 3001 (9.5%) lesions biopsied, 456 (15.2%) were malignant (cancer yield, 1.5%; 456 of 31 465 women; 95% confidence interval [CI]: 1.3%, 1.6%). Among 18 748 of 25 997 women (72.1%) in surveillance who underwent follow-up at 12 months, 1219 (6.5%) underwent biopsy with 230 (18.9%) malignant lesions found (cancer yield, 1.2%; 230 of 18 748 women; 95% CI: 1.1%, 1.4%). Through 2-year follow-up, the biopsy rate was 11.2% (4894 of 43 628 women) with a cancer yield of 1.86% (810 malignancies found among 43 628 women; 95% CI: 1.73%, 1.98%) and a PPV3 of 16.6% (810 malignancies found among 4894 women). Conclusion In the National Mammography Database, Breast Imaging Reporting and Data System (BI-RADS) category 3 use is appropriate, with 1.86% cumulative cancer yield through 2-year follow-up. Of 810 malignancies, 468 (57.8%) were diagnosed at or before 6 months, validating necessity of short-interval follow-up of mammographic BI-RADS category 3 findings. © RSNA, 2020 <iOnline supplemental material is available for this article.</i See also the editorial by Moy in this issue."}, {"id": "pubmed23n0580_20005", "title": "Talk to your patients about breast disease.", "score": 0.008928571428571428, "content": "You have been caring for a 32-year-old woman for the past several years. She presented to your office 2 years ago because she noticed a new breast nodule. You examined her and noted marked breast density at her area of concern without an obvious mass. To be thorough, you referred her for mammography; the report stated that the breasts were asymmetrically dense without a distinct mass. You reassured her that her evaluation was negative. Two years later, the patient returned with an obvious mass at the same site. Biopsy revealed an infiltrating ductal carcinoma. Over the next 6 months, she is treated with lumpectomy, axillary node dissection, chemotherapy, and radiation. Shortly thereafter, you receive a letter from her attorney asking for your records. The patient claims that your care resulted in a delay in diagnosis of her breast cancer."}, {"id": "pubmed23n0235_12788", "title": "So-called interval cancers of the breast. Pathologic and radiologic analysis of sixty-four cases.", "score": 0.008928571428571428, "content": "Within a population-based breast cancer screening programs, 209 cancers were detected by regular mammographic screening. Additionally, 66 cancers were discovered between two consecutive screenings after one, two, or three negative screening examinations (interval cancers). The study group consisted of 25,920 women who have been participating since 1975 in a breast cancer screening program in Nijmegen, the Netherlands. In this program, single view mammography (lateromedial projection) was administered as the sole screening examination every two years. Physical examination was not part of the screening program. All previous histologic and radiologic material from 64 of those \"interval\" patients was available and was reviewed. In 19 of the 64 patients, direct or indirect signs of tumor were seen on the previous screening mammogram on review (observers error). In four cases the site of the tumor lay outside the imaging field (technical error). In 41 cases, no signs of tumor could be seen on the mammograms even on review. By calculated tumor doubling times, 20 of these 41 cases were probably too small to be detected at the last screening (\"real\" interval cancers). However, 21 cases were probably large enough but were somehow masked from radiologic detection. The mean reasons for this \"masking\" proved to be: 1) dense breast, 2) poorly outlined tumor mass of diffuse infiltrative type, mainly invasive lobular carcinomas, and 3) intraductal localization. The authors suggest that women with dense breasts be screened more frequently, using more views and modalities and with broader criteria for advising surgical biopsy. They also note that in general the two-year interval between screenings is probably longer than the optimal interval."}, {"id": "wiki20220301en349_11928", "title": "Breast biopsy", "score": 0.008909083693797372, "content": "A breast biopsy is usually done after a suspicious lesion is discovered on either mammography or ultrasound to get tissue for pathological diagnosis. Several methods for a breast biopsy now exist. The most appropriate method of biopsy for a patient depends upon a variety of factors, including the size, location, appearance and characteristics of the abnormality. The different types of breast biopsies include fine needle aspiration (FNA), vacuum assisted biopsy, core needle biopsy, and surgical excision biopsy. Breast biopsies can be done under ultrasound, MRI or a stereotactic biopsy technique. Vacuum assisted biopsies are typically done using stereotactic techniques when the suspicious lesion can only be seen on mammography. On average, 5-10 biopsies of a suspicious breast lesion will lead to the diagnosis of one case of breast cancer."}, {"id": "pubmed23n0254_403", "title": "Stereotactic breast biopsy.", "score": 0.008849557522123894, "content": "The substantial majority of questionable lesions detected by mammography are benign, and there is growing interest among health care professionals and patients in alternatives to surgical biopsy for diagnosing these lesions. Stereotactic breast biopsy is an x-ray guided method for localizing and sampling breast lesions discovered on mammography and considered to be suspicious for malignancy. Its use in sampling small, nonpalpable breast lesions has been investigated over the past 15 years, using fine-needle aspiration for cytology and, more recently, core-needle biopsy for histology. Multiple series comparing stereotactic biopsy with surgical biopsy have shown that stereotactic techniques accurately sample small lesions and have a sensitivity of 90 to 95 percent for breast cancer detection. State-of-the-art stereotactic breast biopsy is comparable in sensitivity to surgical biopsy, and the procedure is quicker, cheaper, and easier than the standard practice of preoperative, mammographically guided localization followed by surgical biopsy. In an age of miniaturization, stereotactic techniques provide miniature breast biopsies. The University of Chicago acquired the first prone stereotactic table in the United States in 1986, and we have found stereotactic breast biopsy to be a very good alternative for certain lesions that would otherwise require surgical biopsy for diagnosis. Most lesions (70 percent) sent to conventional biopsy at the University of Chicago between 1986 and 1989 were graded by observers as being in a low-suspicion category (less than 10 percent chance of malignancy based on mammographic findings), and the positive malignancy yield of this category of lesions was seven percent. These lesions were also examined with stereotactic fine-needle aspiration performed as a \"piggy-back\" procedure to the needle localization for surgery. The results of this study have led us to use stereotactic biopsy rather than surgical biopsy for low-suspicion lesions since then. We currently use stereotactic breast biopsy for about half the nonpalpable lesions considered for breast biopsy at our institution and find it to be reliable and readily accepted by informed patients. The introduction of automated core-biopsy guns has escalated interest in the technique, due to increased confidence in the histologic samples obtained and the ability to make specific benign diagnoses more frequently. Some centers have extended the potential use of stereotaxis to virtually all suspicious mammographic lesions, including those with a high probability of malignancy, to plan definitive surgery. Based on current estimates, there are now over 1,000 centers either investigating or using stereotactic biopsy for occult breast lesions."}]}}}}
{"correct_option": 5, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": true, "char_ranges": [[0, 100]], "word_ranges": [[0, 13]], "text": "The halo sign is characteristic of pulmonary aspergillosis, especially in an immuodepressed patient."}}, "full_answer": "The halo sign is characteristic of pulmonary aspergillosis, especially in an immuodepressed patient. But it is not pathognomonic; it has also been associated with TB, some neoplasms and Wegener's granulomatosis.", "full_answer_no_ref": "The halo sign is characteristic of pulmonary aspergillosis, especially in an immuodepressed patient. But it is not pathognomonic; it has also been associated with TB, some neoplasms and Wegener's granulomatosis.", "full_question": "An immunodeficient patient who presents a pneumonia with meniscus halo sign or crescentic contour on chest X-ray/CT suggests infection by a microorganism:", "id": 26, "lang": "en", "options": {"1": "Staphylococcus aureus.", "2": "Streptococcus pneumoniae.", "3": "Candida albicans.", "4": "Pseudomonas aeruginosa.", "5": "Aspergillus fumigatus."}, "question_id_specific": 117, "type": "INFECTIOUS", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en576_31556", "title": "Lung cavity", "score": 0.01354431489436311, "content": "Bacteria can cause lung cavities in one of two ways; they can either enter the lung through the trachea (windpipe), or they can enter through the bloodstream as septic pulmonary emboli (infected blood clots). Community-acquired pneumonia is an uncommon cause of lung cavities, but cavitary pneumonia is occasionally seen with Streptococcus pneumoniae or Haemophilus influenzae infection. However, since these two species of bacteria are such common causes of pneumonia, they may cause a significant fraction of all cavitary pneumonias. The most common bacterial causes of lung cavities are Streptococcus species and Klebsiella pneumoniae. Less commonly, the bacteria Staphylococcus aureus, Pseudomonas aeruginosa, Acinetobacter, Escherichia coli, and Legionella can cause cavitation. Nocardia is a bacterium that can cause pulmonary nocardiosis and lung cavities in people who are immunocompromised (have weak immune systems), including organ transplant recipients who are on immunosuppressants,"}, {"id": "wiki20220301en621_19621", "title": "Necrotizing pneumonia", "score": 0.013227513227513227, "content": "Causative organisms The most common pathogens responsible for NP are Streptococcus pneumonia, Staphylococcus aureus, Klebsiella pneumoniae. Other pathogens which are less likely to cause NP are bacteria like Haemophilus influenzae, Streptococcus anginosus group, Pseudomonas aeruginosa, Mycoplasma pneumoniae, Acinetobacter baumannii, Streptococcus pyogenes, Stenotrophomonas maltophilia, anaerobes like Fusobacterium nucleatum and Bacteroides fragilis; fungi like Aspergillus sp. and Histoplasma capsulatum; viruses like Influenza and Adenovirus."}, {"id": "wiki20220301en621_19618", "title": "Necrotizing pneumonia", "score": 0.012833737864077668, "content": "Necrotizing pneumonia (NP), also known as cavitary pneumonia or cavitatory necrosis, is a rare but severe complication of lung parenchymal infection. In necrotizing pneumonia, there is a substantial liquefaction following death of the lung tissue, which may lead to gangrene formation in the lung. In most cases patients with NP have fever, cough and bad breath, and those with more indolent infections have weight loss. Often patients clinically present with acute respiratory failure. The most common pathogens responsible for NP are Streptococcus pneumonia, Staphylococcus aureus, Klebsiella pneumoniae. Diagnosis is usually done by chest imaging, e.g. chest X-ray, CT scan. Among these CT scan is the most sensitive test which shows loss of lung architecture and multiple small thin walled cavities. Often cultures from bronchoalveolar lavage and blood may be done for identification of the causative organism(s)."}, {"id": "wiki20220301en116_5562", "title": "Hospital-acquired pneumonia", "score": 0.012564935064935064, "content": "Causes In some studies, the bacteria found in patients with HCAP were more similar to HAP than to CAP; compared to CAP, they could have higher rates of Staphylococcus aureus (S. aureus) and Pseudomonas aeruginosa, and less Streptococcus pneumoniae and Haemophilus influenzae. In European and Asian studies, the etiology of HCAP was similar to that of CAP, and rates of multi drug resistant pathogens such as Staphylococcus aureus and Pseudomonas aeruginosa were not as high as seen in North American studies. It is well known that nursing home residents have high rates of colonization with MRSA. However, not all studies have found high rates of S. aureus and gram-negative bacteria. One factor responsible for these differences is the reliance on sputum samples and the strictness of the criteria to discriminate"}, {"id": "wiki20220301en043_78392", "title": "Aspergillus", "score": 0.012266196801301164, "content": "A. fumigatus (the most common species) infections are primary pulmonary infections and can potentially become a rapidly necrotizing pneumonia with a potential to disseminate. The organism can be differentiated from other common mold infections based on the fact that it takes on a mold form both in the environment and in the host (unlike Candida albicans which is a dimorphic mold in the environment and a yeast in the body). Aspergillosis Aspergillosis is the group of diseases caused by Aspergillus. The most common species among paranasal sinus infections associated with aspergillosis is A. fumigatus. The symptoms include fever, cough, chest pain, or breathlessness, which also occur in many other illnesses, so diagnosis can be difficult. Usually, only patients with already weakened immune systems or who suffer other lung conditions are susceptible."}, {"id": "wiki20220301en343_15149", "title": "Classification of pneumonia", "score": 0.012122844827586207, "content": "Opportunistic pneumonia People with weakened immune defense, such as HIV/AIDS patients, are highly susceptible to opportunistic infections affecting the lungs. Most common pathogens are Pneumocystis jiroveci, Mycobacterium avium-intracellulare complex, Streptococcus pneumoniae, Haemophilus species. Less frequent pathogens are Cryptococcus neoformans, Histoplasma capsulatum, Coccidioides immitis, cytomegalovirus (CMV), and Toxoplasma gondii. Chemotherapy-induced immunodeficiency may lead to severe lung infections. Pathogens commonly associated with lung infectioins are bacteria (like Pseudomonas aeruginosa, Stenotrophomonas maltophilia, and Nocardia species), viruses (eg, respiratory syncytial virus, parainfluenza virus, influenza virus A and influenza B, and cytomegalovirus), and fungi (eg, Aspergillus, Fusarium, and Mucorales species, and Pneumocystis jirovecii)."}, {"id": "article-86102_7", "title": "Pneumonia in an Immunocompromised Patient -- Etiology", "score": 0.01200817577925396, "content": "Bacteria such as S. aureus , P. aeruginosa , Stenotrophomonas maltophilia , and Burkholderia cepacia complex, are the most common pathogens involved in pulmonary infections immediately after solid organ transplantation, especially heart and lung. ESKAPE pathogens ( Enterococcus faecium, Staphylococcus aureus, Klebsiella pneumoniae, Acinetobacter baumannii, Pseudomonas aeruginosa, and Enterobacter species) especially cause pulmonary infections after lung transplant. Due to the immunosuppression, infections from other opportunistic pathogens such as Cytomegalovirus (CMV) can also occur after solid organ transplantation. In the early phase following hematopoietic stem cell transplantation (HSCT), the majority of pulmonary infections are due to bacteria, for example, S. pneumoniae , Klebsiella , Gram-negative bacilli, and S. aureus . Up to three weeks following HSCT, which is the neutropenic phase, fungi, especially Aspergillus spp. are a common cause of infections, while CMV infections can occur up to three months following HSCT. Pneumocystis pneumonia (PCP) is uncommon after HSCT except in the setting of graft-vs-host disease. [2] [3] Following allogeneic cell transplantation, pulmonary infections due to Fusarium species can also occur, which are seen exclusively in the severely immunocompromised. [4]"}, {"id": "wiki20220301en116_5564", "title": "Hospital-acquired pneumonia", "score": 0.011919274007855885, "content": "Bacteria have been the most commonly isolated pathogens, although viral and fungal pathogens are potentially found in immunocompromised hosts (patients on chronic immunosuppressed medications, solid organ and bone marrow transplant recipients). In general, the distribution of microbial pathogens varies among institutions, partly because of differences in patient population and local patterns of anti microbial resistance in hospitals and critical care units' Common bacterial pathogens include aerobic GNB, such as Pseudomonas aeruginosa, Acinetobacter baumanii, Klebsiella pneumoniae, Escherichia coli as well as gram-positive organisms such as Staphylococcus aureus. In patients with an early onset pneumonia (within 5 days of hospitalization), they are usually due to anti microbial-sensitive bacteria such as Enterobacter spp, E. coli, Klebsiella spp, Proteus spp, Serratia mare scans, community pathogens such as Streptococcus pneumoniae, Haemophilus influenzae, and methicillin-sensitive S."}, {"id": "article-27349_19", "title": "Pneumocystis jirovecii Prophylaxis -- Differential Diagnosis", "score": 0.011535947712418301, "content": "Due to their poor immune response, patients with HIV and those with immunosuppression for other causes, such as malignancy, autoimmune disease, or iatrogenic due to underlying condition or organ transplant patients, require prophylaxis for numerous pathogens. These pathogens include: Aspergillus species Candida albicans Clostridium difficile Coccidioides immitis Cryptococcus neoformans Cytomegalovirus Histoplasma capsulatum Legionella pneumophila Microsporidium Mycobacterium avium complex Mycobacterium tuberculosis Pseudomonas aeruginosa Salmonella Staphylococcus aureus Streptococcus pneumoniae Streptococcus pyogenes Toxoplasma gondii [2] [3] [5]"}, {"id": "pubmed23n0062_8311", "title": "[Clinical features of 32 cases of fungal pneumonia].", "score": 0.011160714285714284, "content": "A total of 32 patients with mycoses other than cavity-formed aspergilloma were reviewed. The main pathogenic fungi were Aspergillus in 14, Candida in 8, Cryptococcus in 4, Trichosporon in 4 and Mucor in 2. Coinfection by two species was detected in 3 cases: Trichosporon and Aspergillus in 2 and Aspergillus and Candida in 1. The underlying diseases were hematologic malignancies in all cases except 1 case of lung cancer. The hematologic malignancies were mostly leukemias of various types. Cryptococcosis developed in patients given long-term corticosteroid treatment but not in leukemic patients. All cases of aspergillosis, candidiasis and mucormycosis were due to nosocomial infection. On the other hand, 3 of 4 cases of cryptococcosis or trichosporonosis were attributable to community-acquired infection. Two of 4 trichosporonosis cases were considered to have been acquired during 2-day home stays. The diagnosis of pulmonary mycosis was made pathologically in 18 and clinically in 14 cases. Of the latter, 6 cases had an air-crescent sign on chest X-ray films and 8 cases were culture-positive. Extrapulmonary involvement was seen in all 16 cases of candidiasis, cryptococcosis and trichosporonosis but not in 10 of 14 aspergillosis cases. Severe granulocytopenia was present in all cases except 4 cases of cryptococcosis and 3 cases of aspergillosis. Chest X-ray findings of aspergillosis were of two types: one was an air-crescent sign which was noted in the recovery phase from leukopenia and the other was gradually enlarging consolidation which was bound by the interlobar fissure and progressed to lobar penumonia. A diffuse granular shadow was not characteristic of any fungus species.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "article-86102_24", "title": "Pneumonia in an Immunocompromised Patient -- Evaluation", "score": 0.011071978868589037, "content": "Imaging modalities for evaluation of pneumonia include: Chest X-ray (CXR). This is the first-line imaging of choice in the evaluation of a suspected case of pneumonia. Posteroanterior (PA) and lateral radiographs should be obtained. However, CXR may be normal for up to 72 hours in immunocompromised patients despite having symptoms. Computed tomography (CT) of the chest should be obtained in patients with high suspicion of pneumonia and normal findings on CXR. In neutropenic patients, CXR will have minimal or no abnormalities and so performing a CT scan is highly recommended. Other investigations for specific etiologies include: Urine antigen testing for pneumococcus and Legionella ELISA and PCR for viruses such as Herpes Simplex Virus (HSV), Influenza A and B, and Cytomegalovirus (CMV) Serum antigen testing for Cryptococcus and Aspergillus Beta-D-glucan testing if a fungal pathogen is suspected. [2] [8] [17] [20]"}, {"id": "wiki20220301en116_5558", "title": "Hospital-acquired pneumonia", "score": 0.010401446296342157, "content": "Types Bacterial pneumonia: The majority of cases related to various rod shaped gram-negative organisms (52%) and Staphylococcus aureus (19%), usually of the MRSA type. Others are Haemophilus spp. (5%). In the ICU results were S. aureus (17.4%), Pseudomonas aeruginosa (17.4%), Klebsiella pneumoniae and Enterobacter spp. (18.1%), and Haemophilus influenzae (4.9%). Viral pneumonia: influenza and respiratory syncytial virus and, in the immunocompromised host, cytomegalovirus – cause 10–20% of infections. Ventilator-associated pneumonia"}, {"id": "article-23814_11", "title": "Job Syndrome -- History and Physical -- Immunological Features", "score": 0.01006170101221922, "content": "Recurrent pneumonia is typical, predominately due to S. aureus , and less frequently due to Streptococcus pneumoniae, and Haemophilus. It can get complicated with the development of recurrent lung abscesses, bronchiectasis, and pneumatoceles. These pneumatoceles can get colonized with Aspergillus and Pseudomonas . Superinfection with Pneumocystis carinii [5] was also reported."}, {"id": "wiki20220301en471_9068", "title": "Neutrophil-specific granule deficiency", "score": 0.009900990099009901, "content": "Neutrophil-specific granule deficiency ( previously known as lactoferrin deficiency) is a rare congenital immunodeficiency characterized by an increased risk for pyogenic infections due to defective production of specific granules and gelatinase granules in patient neutrophils. Symptoms and signs Atypical infections are the key clinical manifestation of SGD. Within the first few years of life, patients will experience repeated pyogenic infections by species such as Staphylococcus aureus, Pseudomonas aeruginosa or other Enterobacteriaceae, and Candida albicans. Cutaneous ulcers or abscesses and pneumonia and chronic lung disease are common. Patients may also develop sepsis, mastoiditis, otitis media, and lymphadenopathy. Infants may present with vomiting, diarrhea, and failure to thrive."}, {"id": "pubmed23n0619_10786", "title": "[A clinical study of 49 cases of invasive pulmonary aspergillosis].", "score": 0.009900990099009901, "content": "Studying the proven and probable invasive pulmonary aspergillosis (IPA) cases of some hospitals in Shanghai to provide evidence for the improvement of IPA clinical diagnosis and therapy. Forty-nine IPA cases were retrospectively analyzed for demography data, host factors, underlying conditions, chest CT, microorganism and histopathology examination, as well as therapy and clinical outcome. Of 49 subjects including 19 (38.8%) proven and 30 (61.2%) probable IPA, 3 patients (6.1%) had no host factors, 25 patients (51.0%) had IPA associated host factors and underlying conditions, while 21 patients (42.9%) had uncertain fundamental diseases. Chest CT evaluation demonstrated that radiological lesions include nodules in 29 patients, patching in 15, mass in 12, consolidation in 10, cavitation in 34, Halo sign in 19, air bronchogram in 18, crescentic sign in 6, bilateral in 33 and multifocal lesions in 38. The yielding rate of fungus culture in sputum was 26.5% (13/49), and in bronchoalveolar lavage fluid was 66.7% (10/15). Eleven of thirty-six patients (30.6%) had positive results of serum galactomannan antigen tests. Nineteen of twenty-one patients (90.5%) were proven as IPA by lung histologic examinations. Aspergillus fumigatus was the most common pathogen 81.0% (17/21). The responding rate to initial anti-fungus therapy was 50% (21/42). Our study suggests that in IPA patients, bilateral, multifocal and nodular lesion could be the most common radiological characteristic, while Halo and crescentic sign occur occasionally. Invasive technologies are more valuable to IPA diagnosis."}, {"id": "wiki20220301en194_21264", "title": "Peptidoglycan recognition protein 2", "score": 0.00980392156862745, "content": "Defense against infections PGLYRP2 plays a limited role in host defense against infections. PGLYRP2-deficient mice are more sensitive to Pseudomonas aeruginosa-induced keratitis and Streptococcus pneumoniae-induced pneumonia and sepsis. However, PGLYRP2-deficient mice did not show a changed susceptibility to systemic Escherichia coli, Staphylococcus aureus, and Candida albicans infections or intestinal Salmonella enterica infection, although the latter was accompanied by increased inflammation in the cecum. Although PGLYRP2 is not directly bacteriolytic, it has antibacterial activity against both Gram-positive and Gram-negative bacteria and Chlamydia trachomatis. Maintaining microbiome Mouse PGLYRP2 plays a role in maintaining healthy microbiome, as PGLYRP2-deficient mice have significant changes in the composition of their intestinal microbiome, which affect their sensitivity to colitis."}, {"id": "pubmed23n1129_19120", "title": "Study to assess aetiology, clinical and imaging characteristics of post Covid-19 pulmonary cavitation.", "score": 0.00980392156862745, "content": "The aim of this study is to determine the aetiology and characteristics of pulmonary cavities that developed in patients recovering from COVID-19 infection. Between 1<supst</sup May 2021 and 30<supst</sup June 2021, we found 9 post COVID-19 patients who developed lung cavities on chest radiograph or CT during the follow-up period. These patients underwent routine blood examination, sputum examination and bronchoscopy to identify the aetiologies for the lung cavities. The duration from the onset of COVID-19 symptoms to the detection of lung cavities ranged from 18 to 82 days. Out of 7 patients, 4 had recovered from severe COVID-19 disease, 2 from moderate and 1 from mild disease. After the diagnostic workup, 5 patients were found to have COVID-19 associated pulmonary aspergillosis (CAPA), 1 patient with mucormycosis and 1 patient with mycobacterium infection. Two patients with CAPA also had bacterial infection; sputum culture from both these patients grew Klebsiella pneumonia. Lung cavities can develop in patients recovering from COVID-19 pneumonia and fungal infection is the most common cause for such cavities."}, {"id": "pubmed23n0329_6548", "title": "[Pulmonary cavitation lesions in patients infected with the human immunodeficiency virus: an analysis of a series of 78 cases].", "score": 0.009791370317686107, "content": "To assess the clinical, radiologic and microbiological features of lung cavitation and HIV infection. Evaluation of the differences related to this disease in the last years. Retrospective review of all patients with lung cavitation and HIV infection admitted at our hospital from January 1989 until December 1994 and prospective study of all patients with the same characteristics during 1995 and 1996. Lung cavitation was defined as any parenchymal lesion, with air content, visible in a simple X-ray and greater than 1 cm of diameter. Criteria for confirmed, probable or possible diagnosis were defined. 78 cases of lung cavitation have been identified in 73 patients. The radiologic patterns included unilobar and multilobular involvement in 31 and 47 cases, respectively. Cavities were multiple and single in 40 and 38 cases respectively. Findings with fine needle aspiration biopsy (FNAB) were diagnostic in 11 out of 14 cases. A clinical diagnosis was performed in all 78 cases, with microbiological results in 69 cases (88.5%): Mycobacterium tuberculosis in 20, Pneumocystis carinii in nine, Pseudomonas aeruginosa in nine, Staphylococcus aureus in eight (5 endocarditis with cavitary septic emboli), Rhodococcus equi in six, P. aeruginosa and S. aureus in three, Salmonella enteritidis in three, Cryptococcus neoformans in two, Aspergillus fumigatus in two and others in 7 cases. Confirmed, probable and possible diagnosis was considered in 54, 15 and 9 cases, respectively. Thirteen episodes of spontaneous pneumothorax were found. The lung cavitation rate is low, compared with the number of admissions related to HIV infection; nevertheless, many of them are in close relationship with HIV infection, and most of them are caused by treatable infections. It is important to know the clinical and radiological characteristics, in order to establish an early diagnosis and an appropriate therapy. Pseudomonas aeruginosa is becoming an important cause of lung cavitation. In our series, spontaneous pneumo-thorax was not related to Pneumocystis carinii pneumonia in 61.5% of cases."}, {"id": "pubmed23n0989_9659", "title": "Differential diagnosis of pulmonary infections in immunocompromised patients using high-resolution computed tomography.", "score": 0.009708737864077669, "content": "The aims of this study were to compare the high-resolution computed tomography (HRCT) findings of pulmonary infections in immunocompromised patients and to assess the usefulness of HRCT in the differential diagnosis of these infections. A total of 345 immunocompromised patients with pulmonary infections were included in this study. The diagnoses of the patients consisted of bacterial pneumonia (123 cases), pneumocystis pneumonia (PCP) (105 cases), fungal pneumonia (80 cases), tuberculosis (15 cases), cytomegalovirus pneumonia (11 cases), and septic embolism (11 cases). Two chest radiologists retrospectively evaluated the computed tomography (CT) images, which consisted of 22 findings including ground-glass attenuation, consolidation, nodules, and thickening of the bronchial wall and interlobular septum. Associations between the CT criteria and infections were investigated using χ<sup2</sup test; multiple logistic regression analyses were conducted to identify the significant indicator for each infection. The area under the curve (AUC) of each model was calculated. Bronchial wall thickening was a significant indicator for bacterial pneumonia (p = 0.002; odds ratio [OR], 2.341; 95% confidence interval [CI], 1.378-3.978). The presence of a mosaic pattern and the absence of nodules were significant indicators for PCP (p &lt; 0.001; OR, 9.808; 95% CI, 4.883-13.699, and p &lt; 0.001; OR, 6.834; 95% CI, 3.438-13.587, respectively). The presence of nodules was a significant indicator for fungal infection (p = 0.005; OR, 2.531; 95% CI, 1.326-4.828). The AUC for PCP was the highest (0.904). HRCT findings are potentially useful for the differential diagnosis of some pulmonary infections in immunocompromised patients. • Differential diagnosis of pulmonary infections in immunocompromised patients could be established with the help of high-resolution computed tomography. • Bronchial wall thickening was a significant indicator for bacterial pneumonia. • The presence of a mosaic pattern and the absence of nodules were significant indicators for pneumocystis pneumonia."}, {"id": "article-86102_5", "title": "Pneumonia in an Immunocompromised Patient -- Etiology", "score": 0.009625690493857697, "content": "Patients with defective humoral immunity are at increased risk of infection from encapsulated bacteria such as Haemophilus influenzae and Streptococcus pneumoniae . Those with neutropenia are predisposed to infections from S. aureus , Gram-negative bacilli (including Pseudomonas aeruginosa ), as well as fungi such as Aspergillus spp. Impaired T-cell immunity can lead to a range of infections from: Viruses, such as Cytomegalovirus Intracellular bacteria, for example, Legionella Acid-fast bacteria such as Mycobacteria and Nocardia Fungi, such as Pneumocystis jirovecii , Aspergillus spp., Coccidioides immitis , Cryptococcus spp., Blastomyces dermatitidis , Histoplasma capsulatum , etc [3]"}, {"id": "wiki20220301en546_19535", "title": "Innate immune defect", "score": 0.009615384615384616, "content": "Interleukin-1 receptor-associated kinase deficiency is an inherited disorder of the immune system. This immunodeficiency leads to recurrent infections caused by the pyogenic bacteria, for example Streptococcus pneumoniae, Staphylococcus aureus and Pseudomonas aeruginosa, but not by other infectious agents. Most patients with IRAK-4 deficiency suffer from invasive bacterial infections, which can cause sepsis, meningitis or they affect the joints that can lead to inflammation and arthritis. These invasive infections can also cause areas of tissue breakdown and pus production (abscesses) on internal organs. In addition, patients are characterized by infections of the upper respiratory tract, eyes or skin. Although fever is a common reaction to bacterial infections, many people with IRAK-4 deficiency do not at first develop a high fever in response to these infections, even if the infection is severe. Most patients have their first bacterial infection before age 2, and the infections can"}, {"id": "pubmed23n0523_8345", "title": "Invasive pulmonary aspergillosis: frequency and meaning of the \"hypodense sign\" on unenhanced CT.", "score": 0.009615384615384616, "content": "The purpose of this study was to establish the diagnostic value of central hypointensity (\"hypodense sign\") in lung consolidations or nodules, in severely immunocompromised or neutropenic patients, suspected of having invasive pulmonary aspergillosis (IPA), and to assess its recognition on unenhanced CT scans. Serial CT scans of the lung were retrospectively reviewed in 43 consecutive immunosuppressed patients with IPA, and assessed for the presence of the hypodense sign using standard mediastinal and lung windowing settings, as well as a special, narrower window setting (width 110-140 HU; level 15-40 HU). The temporal relationship between the occurrence of the first CT-finding suspicious of IPA and the appearance of the hypodense sign, as well as between this and the occurrence of the crescent sign, cavitation or reduction in lesion size, was evaluated. Additionally, CT-scans from 89 immunocompromised patients with viral (n=45) or bacterial (n=44) pneumonia, investigated in the same time period at our institution were reviewed, with respect to the presence of the \"hypodense\" sign. Unenhanced CT scans revealed the hypodense sign in 11 neutropenic patients and 2 severely immunocompromised patients, out of a total of 43 patients with IPA evaluated in this study (30.2%). The mean time between the appearance of the first CT-findings of IPA (large nodule or consolidation +/- positive halo sign) and the hypodense sign was 7.8 days, while the time interval between the hypodense sign and the occurrence of crescent sign, cavitation, or decrease of the lesion's size was 8.3 days. The hypodense sign did not occur in any of the patients with viral or bacterial pneumonia, in the control series. We consider the hypodense sign to be a supplementary tool in the diagnosis of IPA. Its sensitivity was low in our series, but the high specificity makes it valuable in predicting IPA, anticipating the occurrence of cavitation or crescent sign, which are considered specific, but late findings of IPA. The hypodense sign is recognizable also on unenhanced CT, when a narrower lung window setting is used."}, {"id": "wiki20220301en029_32762", "title": "Hospital-acquired infection", "score": 0.009523809523809525, "content": "Types Hospital-acquired pneumonia Ventilator-associated pneumonia Urinary tract infection Gastroenteritis Puerperal fever Central line-associated blood stream infection Organisms Staphylococcus aureus Methicillin resistant Staphylococcus aureus Candida albicans Pseudomonas aeruginosa Acinetobacter baumannii' Stenotrophomonas maltophilia Clostridium difficile Escherichia coli Tuberculosis Vancomycin-resistant Enterococcus Legionnaires' disease Cause Transmission In-dwelling catheters have recently been identified with hospital acquired infections. To deal with this complication, procedures are used, called intravascular antimicrobial lock therapy that can reduce infections that are unexposed to blood-borne antibiotics. Introducing antibiotics, including ethanol, into the catheter (without flushing it into the bloodstream) reduces the formation of biofilms."}, {"id": "pubmed23n0101_2030", "title": "Overwhelming pneumonia.", "score": 0.009523809523809525, "content": "Overwhelming pneumonias remain an important cause of morbidity and mortality. These illnesses may be rapidly fatal; thus, many patients are treated empirically. Although the various etiologic agents cannot be differentiated on the basis of radiographic appearance, epidemiologic information may give a clue to the cause. Community-acquired overwhelming pneumonias are usually due to pyogenic bacteria (especially Streptococcus pneumoniae), mycoplasma, mycobacteria, and fungi. Hospital-acquired pneumonias are usually due to aerobic gram-negative bacilli. If the patient is immunocompromised, Pneumocystis carinii, Candida, and Aspergillus must be considered. Choice of optimal antimicrobial therapy requires that a specific etiology be identified. Gram's stain of sputum is often helpful in the diagnosis of community-acquired pneumonia. Invasive diagnostic techniques such as bronchoscopy and open lung biopsy are often required in nosocomial pneumonias and pneumonias in immunocompromised patients."}, {"id": "wiki20220301en019_39404", "title": "Keratitis", "score": 0.009433962264150943, "content": "Bacterial Bacterial keratitis. Bacterial infection of the cornea can follow from an injury or from wearing contact lenses. The bacteria involved are Staphylococcus aureus and for contact lens wearers, Pseudomonas aeruginosa. Pseudomonas aeruginosa contains enzymes that can digest the cornea. Fungal Fungal keratitis, caused by Aspergillus fumigatus and Candida albicans (cf. Fusarium, causing an outbreak of keratitis in 2005–2006 through the possible vector of Bausch & Lomb ReNu with MoistureLoc contact lens solution) Amoebic Acanthamoebic keratitis Amoebic infection of the cornea is a serious corneal infection, often affecting contact lens wearers. It is usually caused by Acanthamoeba. On May 25, 2007, the U.S. Center for Disease Control issued a health advisory due to increased risk of Acanthamoeba keratitis associated with use of Advanced Medical Optics Complete Moisture Plus Multi-Purpose eye solution."}, {"id": "pubmed23n1052_17572", "title": "Fatal Invasive Pulmonary Aspergillosis in COVID-19 Patient with Acute Myeloid Leukemia in Iran.", "score": 0.009433962264150943, "content": "Although patients with severe immunodeficiency and hematological malignancies has been considered at highest risk for invasive fungal infection, patients with severe pneumonia due to influenza, and severe acute respiratory syndrome coronavirus (SARS-CoV) are also at a higher risk of developing invasive pulmonary aspergillosis (IPA). Recently, reports of IPA have also emerged among SARS-CoV-2 infected patients admitted to intensive care units (ICUs). Here, we report a fatal case of probable IPA in an acute myeloid leukemia patient co-infected with SARS-CoV-2 and complicated by acute respiratory distress syndrome (ARDS). Probable IPA is supported by multiple pulmonary nodules with ground glass opacities which indicate halo sign and positive serum galactomannan results. Screening studies are needed to evaluate the prevalence of IPA in immunocompromised patients infected with SARS-CoV-2. Consequently, testing for the presence of Aspergillus in lower respiratory secretions and galactomannan in consecutive serum samples of COVID-19 patients with timely and targeted antifungal therapy based on early clinical suspicion of IPA are highly recommended."}, {"id": "wiki20220301en021_36121", "title": "Levofloxacin", "score": 0.009345794392523364, "content": "Its spectrum of activity includes most strains of bacterial pathogens responsible for respiratory, urinary tract, gastrointestinal, and abdominal infections, including Gram negative (Escherichia coli, Haemophilus influenzae, Klebsiella pneumoniae, Legionella pneumophila, Moraxella catarrhalis, Proteus mirabilis, and Pseudomonas aeruginosa), Gram positive (methicillin-sensitive but not methicillin-resistant Staphylococcus aureus, Streptococcus pneumoniae, Staphylococcus epidermidis, Enterococcus faecalis, and Streptococcus pyogenes), and atypical bacterial pathogens (Chlamydophila pneumoniae and Mycoplasma pneumoniae). Compared to earlier antibiotics of the fluoroquinoline class such as ciprofloxacin, levofloxacin exhibits greater activity towards Gram-positive bacteria but lesser activity toward Gram-negative bacteria, especially Pseudomonas aeruginosa."}, {"id": "pubmed23n0695_10285", "title": "Pulmonary aspergillosis in immunocompetent patients without air-meniscus sign and underlying lung disease: CT findings and histopathologic features.", "score": 0.009345794392523364, "content": "Pulmonary aspergillosis in immunocompetent patients has been described as a saprophytic infection with pre-existing lung lesions showing an air-meniscus sign on chest radiograph or CT scans. There have been rare articles dealing with pulmonary aspergillosis in immunocompetent patients without pre-existing lung lesions. To evaluate the CT findings of pulmonary aspergillosis in immunocompetent patients without air-meniscus and underlying lung disease and to correlate the CT findings and pathologic features of pulmonary aspergillosis in these patients. A total of seven surgically proven pulmonary aspergillosis found in immunocompetent patients without an air-meniscus and underlying lung disease (M:F = 1:6; mean age 63.4 years) were included. On CT, the lesion shape, margin, type, location, diameter, presence of satellite nodules, presence of CT halo sign or hypodense sign, and interval growth were evaluated. Histopathologic features of each lesion were classified as one of the following; primary aspergilloma, chronic necrotizing pulmonary aspergillosis, or invasive pulmonary aspergillosis. Correlation between CT findings and pathological features was performed. All lesions presented as a nodule or mass unable to differentiate from malignancy. Most lesions had well-defined margins (n = 4), appeared as solid lesions (n = 7), and were located in the upper lobe (n = 5). Mean diameter of lesions was 2.3 cm. Satellite nodules (n = 2), CT halo sign (n = 1), and hypodense sign (n = 4) were found. Only one lesion increased in size during follow-up. Lesions were pathologically classified as primary aspergilloma (n = 3) and chronic necrotizing pulmonary aspergillosis (n = 4). The hypodense sign on CT was pathologically proved as dense fungal hyphae filled in bronchus and CT halo sign as parenchymal hemorrhage. Pulmonary aspergillosis predominantly presented as a nodule or mass mimicking malignancy in the upper lobes on CT scan in elderly without underlying lung disease and immunosuppressive conditions except for age, and was histopathologically revealed to be either primary aspergilloma or chronic necrotizing pulmonary aspergillosis."}, {"id": "pubmed23n1136_23777", "title": "Fungal infection mimicking COVID-19 infection - A case report.", "score": 0.009259259259259259, "content": "For the last 2 years, one of the most frequent causes of respiratory failure is coronavirus disease 2019 (COVID-19). The symptoms are not specific. Imaging diagnostics, especially high-resolution computed tomography, is a diagnostic method widely used in the diagnosis of this disease. It is important to emphasize that not only SARS-CoV-2 infection may manifest as interstitial pneumonia. Other diseases such as other viral, fungal, atypical bacterial pneumonia, autoimmune process, and even cancer can also manifest as ground-glass opacities or consolidations in the imaging of the lungs. In this case report, we described a patient who manifested many symptoms that seemed to be COVID-19. However, all performed antigen and polymerase chain reaction tests were negative. The diagnostics must have been extended. Microbiological and mycological blood cultures and sputum cultures were performed. Blood cultures were negative but in sputum, <iCandida albicans</i and <iCandida glabrata</i were identified. Targeted therapy with fluconazole was implemented with a satisfactory result. The patient was discharged from the hospital in a good general condition with no complaints."}, {"id": "wiki20220301en056_70172", "title": "Pine oil", "score": 0.009174311926605505, "content": "Properties as a disinfectant Pine oil is a disinfectant that is mildly antiseptic. It is effective against Brevibacterium ammoniagenes, the fungi Candida albicans, Enterobacter aerogenes, Escherichia coli, Gram-negative enteric bacteria, household germs, Gram-negative household germs such as those causing salmonellosis, herpes simplex types 1 and 2, influenza type A, influenza virus type A/Brazil, influenza virus type A2/Japan, intestinal bacteria, Klebsiella pneumoniae, odor-causing bacteria, mold, mildew, Pseudomonas aeruginosa, Salmonella choleraesuis, Salmonella typhi, Salmonella typhosa, Serratia marcescens, Shigella sonnei, Staphylococcus aureus, Streptococcus faecalis, Streptococcus pyogenes, and Trichophyton mentagrophytes."}, {"id": "pubmed23n0052_7200", "title": "Invasive aspergillosis in immunocompromised patients: findings on plain film and (HR)CT.", "score": 0.009174311926605505, "content": "The CT and plain chest film abnormalities in eight patients with invasive pulmonary aspergillosis (IPA) are described and compared. The various radiologic findings of IPA were (sub)segmental and patchy consolidation, cavitation and an air crescent sign. CT had a higher sensitivity for multiplicity of lesions and cavitation compared with the plain chest film. Because these abnormalities are keypoints of the diagnosis, CT is recommended in patients suspected of IPA."}, {"id": "wiki20220301en208_36630", "title": "Polyhexanide", "score": 0.00909090909090909, "content": "Polyhexanide (polyhexamethylene biguanide, PHMB) is a polymer used as a disinfectant and antiseptic. In dermatological use, it is spelled polihexanide (INN) and sold under names such as Lavasept, Serasept, Prontosan and Omnicide. PHMB has been shown to be effective against Pseudomonas aeruginosa, Staphylococcus aureus (also the methicillin-resistant type, MRSA), Escherichia coli, Candida albicans (yeast), Aspergillus brasiliensis (mold), vancomycin-resistant enterococci, and Klebsiella pneumoniae (carbapenem-resistant enterobacteriaceae)."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[176, 317]], "word_ranges": [[31, 54]], "text": "It is most likely to be a pneumococcal infection that we cover with Ceftriaxone and with azithromycin we cover the so-called \"atypical\" ones."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[318, 535]], "word_ranges": [[54, 91]], "text": "Meropenem is an antibiotic with too broad a spectrum, which would be an option in in-hospital pneumonia caused by P. aeruginosa, a bacterial agent that can also cause pneumonia in HIV patients, but this is not common."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The first option seems to be the correct one, due to the low probability of P. jirovecii infection in a patient with more than 500 CD4 lymphocytes and undetectable viral load. It is most likely to be a pneumococcal infection that we cover with Ceftriaxone and with azithromycin we cover the so-called \"atypical\" ones. Meropenem is an antibiotic with too broad a spectrum, which would be an option in in-hospital pneumonia caused by P. aeruginosa, a bacterial agent that can also cause pneumonia in HIV patients, but this is not common.", "full_answer_no_ref": "The first option seems to be [HIDDEN], due to the low probability of P. jirovecii infection in a patient with more than 500 CD4 lymphocytes and undetectable viral load. It is most likely to be a pneumococcal infection that we cover with Ceftriaxone and with azithromycin we cover the so-called \"atypical\" ones. Meropenem is an antibiotic with too broad a spectrum, which would be [HIDDEN] in in-hospital pneumonia caused by P. aeruginosa, a bacterial agent that can also cause pneumonia in HIV patients, but this is not common.", "full_question": "A 45-year-old man consults for a productive cough, pleuritic pain in the right flank and fever of 48 h of evolution. He has a baseline O2 saturation of 88% and rales in the right base. Chest X-ray shows a right basal consolidation. She has a history of HIV infection well controlled with antiretroviral drugs (CD4 lymphocytes 550 ce/uL and undetectable HIV viral load). Which of the following empirical antimicrobial treatments do you consider most appropriate?", "id": 435, "lang": "en", "options": {"1": "Cefiriaxone 2 g and azithromycin 500 mg every 24 hours.", "2": "Cefiriaxone 2 g, azithrornicin 500 mg every 24 h and trimethoprim-sulfamethoxazole 5 mg/kg/8 h (based on trimethoprim doses).", "3": "Methyl-prednisolone 40 mg/day, cefiriaxone 2 g IV 124 h and trimethoprim-sulfamethoxazole 5 mg/kg/8 h (based on trimethoprim doses).", "4": "Meropenem I g/8 h and vancomycin I g/l2 h.", "5": NaN}, "question_id_specific": 121, "type": "INFECTIOUS DISEASES AND MICROBIOLOGY", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "InternalMed_Harrison_17062", "title": "InternalMed_Harrison", "score": 0.013252197430696416, "content": "Amoxicillin, 500 mg PO q8h × 60 d, likely to be effective if strain is penicillin sensitive Active disease: Ciprofloxacin, 400 mg IV q12h or doxycycline, 100 mg IV q12h plus Clindamycin, 900 mg IV q8h and/or rifampin, 300 mg IV q12h; switch to PO when stable × 60 d total plus Raxibacumab, 40 mg/kg IV over 2.25 h; diphenhydramine to reduce reaction Gentamicin, 2.0 mg/kg IV loading then 1.7 mg/kg q8h IV or Streptomycin, 1.0 g q12h IM or IV Alternatives include doxycycline, 100 mg bid PO or IV; chloramphenicol, 500 mg qid PO or IV Supportive measures; consideration for cidofovir, tecovirimat, antivaccinia immunoglobulin Streptomycin, 1 g IM bid or Gentamicin, 5 mg/kg per day div q8h IV for 14 days or Doxycycline, 100 mg IV bid or Chloramphenicol, 15 mg/kg up to 1 g IV qid or Ciprofloxacin, 400 mg IV bid Ribavirin 30 mg/kg up to 2 g × 1, followed by 16 mg/kg IV up to 1 g q6h for 4 days, followed by 8 mg/ kg IV up to 0.5 g q8h × 6 days"}, {"id": "InternalMed_Harrison_11649", "title": "InternalMed_Harrison", "score": 0.011775263787664527, "content": "For outpatient management, amoxicillin (1 g every 8 h) provides effective treatment for virtually all cases of pneumococcal pneumonia. Neither cephalosporins nor quinolones, which are far more expensive, offer any advantage over amoxicillin. Levofloxacin (500–750 mg/d as a single dose) and moxifloxacin (400 mg/d as a single dose) also are highly likely to be effective in the United States except in patients who come from closed populations where these drugs are used widely or who have themselves been treated recently with a quinolone. Clindamycin (600–1200 mg/d every 6 h) is effective in 90% of cases and azithromycin (500 mg on day 1 followed by 250–500 mg/d) or clarithromycin (500–750 mg/d as a single dose) in 80% of cases. Treatment failure resulting in bacteremic disease due to macrolide-resistant isolates has been amply documented in patients given azithromycin empirically. As noted above, rates of resistance to all these antibiotics are relatively low in some countries and much"}, {"id": "InternalMed_Harrison_3606", "title": "InternalMed_Harrison", "score": 0.011445726616005254, "content": "7. Volume overexpansion with IV fluid administration is not uncommon and contributes to the development of hyperchloremic acidosis during the later stages of treatment of DKA. Volume overexpansion should be avoided. A 25-year-old man with a 6-year history of HIV-AIDS complicated recently by Pneumocystis jiroveci pneumonia (PCP) was treated with intravenous trimethoprim-sulfamethoxazole (20 mg trimethoprim/kg per day). On day 4 of treatment, the following laboratory data were PART 2 Cardinal Manifestations and Presentation of Diseases H2O AQP-2AQP-3, 4 H2O"}, {"id": "InternalMed_Harrison_11645", "title": "InternalMed_Harrison", "score": 0.011355997311053492, "content": "As a result of the increased prevalence of resistant pneumococci, first-line therapy for persons ≥1 month of age is a combination of vancomycin (adults, 30–60 mg/kg per day; infants and children, 60 mg/kg per day) and cefotaxime (adults, 8–12 g/d in 4–6 divided doses; children, 225–300 mg/kg per day in 1 dose or 2 divided doses) or ceftriaxone (adults, 4 g/d in 1 dose or 2 divided doses; children, 100 mg/kg per day in 1 dose or 2 divided doses). If children are hypersensitive to β-lactam agents (penicillins and cephalosporins), rifampin (adults, 600 mg/d; children, 20 mg/d in 1 dose or 2 divided doses) can be substituted for cefotaxime or ceftriaxone. A repeat lumbar puncture should be considered after 48 h if the organism is not susceptible to penicillin and information on cephalosporin sensitivity is not yet available, if the patient’s clinical condition does not improve or deteriorates, or if dexamethasone has been administered and may be compromising clinical evaluation. When"}, {"id": "InternalMed_Harrison_9510", "title": "InternalMed_Harrison", "score": 0.010152479473916972, "content": "Cefoxitin, 2 g q6h; A combination of metronidazole (500 mg q8–12h) plus cefazolin (1–2 g q8h) or cefuroxime (1.5 g q8h) or ceftriaxone (1–2 g q12–24h) or cefotaxime (1–2 g q6–8h) A carbapenem (imipenem, 1 g q8h; meropenem, 1 g q8h; doripenem, 500 mg q8h); Piperacillin-tazobactam, 3.375 g q6hf; A combination of metronidazole (500 mg q8–12h) plus an antipseudomonal cephalosporin (cefepime, 2 g q8–12h; ceftazidime, 2 g q8h) or an antipseudomonal fluoroquinolone (ciprofloxacin, 400 mg q12h; levofloxacin, 750 mg q24h) — Dexamethasone (0.15 mg/ kg IV q6h for 2–4 d) should be added for patients with suspected or proven pneumococcal meningitis, with the first dose administered 10–20 min before the first dose of antibiotics. If MRSA is a consideration, add vancomycin (15 mg/kg q12hb) or linezolid (600 mg q12h); daptomycin should not be used in patients with pneumonia. If MRSA is a consideration, add vancomycin (15 mg/kg q12hb). 159, 201, and pathogen-specific chapters"}, {"id": "pubmed23n0363_22997", "title": "Toxic epidermal necrolysis following combination of methotrexate and trimethoprim-sulfamethoxazole.", "score": 0.009900990099009901, "content": "A 15-year-old boy with T-cell acute lymphoblastic leukemia (ALL) (FAB L1), diagnosed in 1995, received combination chemotherapy consisting of 6 weeks of induction (vincristine, epirubicin, L-asparaginase, prednisolone) and 2 weeks of consolidation (cytosine arabinosides, etoposide). After achieving remission, for further maintenance of remission, he was treated with 14 cycles of intensive chemotherapy consisting of 6-MP, 10 mg/kg orally on the first 4 days, and cyclophosphamide, 1200 mg/m2, vincristine, 1.5 mg/m2, epirubicin, 15 mg/m2, and cytosine arabinoside, 40 mg/m2, intravenously on days 4, 11, 39, and 40, respectively. On day 18 of each cycle, he received intravenous methotrexate (MTX) infusion in a total dose of 150 mg/m2 plus oral leucovorin (30 mg/m2 ) rescue 36 h after starting MTX therapy. In addition, oral trimethoprim-sulfamethoxazole was given regularly to prevent Pneumocystis carinii infection. The patient achieved remission during the first course of treatment, but 8 months later the disease relapsed. He then received four doses of MTX (800 mg intravenously) plus leucovorin rescue in the following 4 months. During the last MTX therapy, small hemorrhagic bullae were found on the lateral side of the right ankle, but subsided after a few days. Due to partial remission of the disease, he was admitted again in January 1999 for high-dose MTX therapy. An initial hemogram on admission revealed hemoglobin 7.2 g/dL, white cell count 15,200/mm3, platelet count 153/mm3, blood creatinine 0.5 mg/dL, and alanine leucine aminotransferase (ALT) 20 U/L. He received 8500 mg of MTX (5000 mg/m2 ) as a continuous intravenous infusion for 24 h. Thirty-six hours after the start of MTX infusion, leucovorin (30 mg, intravenous) rescue was initiated every 6 h for 3 days. Another preventive measure to cover MTX toxicity included aggressive intravenous fluid replacement (4 L/m2 /day) and the addition of 25 meq/L sodium bicarbonate to the intravenous fluid to alkalinize the urine. Concurrent medication included 6-MP (50 mg) once daily and trimethoprim-sulfamethoxazole (120 mg, 600 mg) twice daily every other day. Plasma MTX levels were 52.36 micromol/L 24 h after MTX infusion, 1.87 micromol/L after 48 h, 0.57 micromol/L after 72 h, and 0.41 micromol/L after 96 h. These indicated delayed MTX plasma clearance. The blood creatinine level was mildly elevated from 0.5 mg/dL to 0.7 mg/dL. Thirty-six hours after the administration of MTX, the patient developed an erythematous painful swelling on the right middle finger. The erythema, with subsequent large bulla formation, progressed to all the fingers, toes, palms, and the soles of the feet. Some erythematous to hemorrhagic papules also appeared on the bilateral elbows. Subsequently, diffuse tender erythema with extensive erosions and focal tiny pustules developed on the back, abdomen, proximal extremities, and face (Fig. 1a,b). A positive Nikolsky's sign was also present. A biopsy specimen of the right dorsal hand lesion revealed parakeratosis, detached acanthotic epidermis with scattered necrotic keratinocytes, dyskeratotic cells and nuclear atypia, neutrophilic exocytosis, and many neutrophils in the papillary dermis (Fig. 2). The skin condition deteriorated rapidly. Toxic epidermal necrolysis-like lesions involved 90% of the total body surface on the fifth day after MTX infusion. Mucositis, diarrhea, involuntary tremor, fever, and chills were noted. The patient was then sent to the burn unit for intensive skin care. Ten days after MTX therapy, profound agranulocytosis and thrombocytopenia (white cell count 100/mm3, platelets 14,000/mm3, and hemoglobin 5.6 g/dL) were found. The patient was then started on granulocyte colony stimulation factor (G-CSF, 5 microg/kg/day), but his general condition deteriorated rapidly and he died 6 days later due to septic shock and multiple organ failure."}, {"id": "InternalMed_Harrison_15144", "title": "InternalMed_Harrison", "score": 0.009900990099009901, "content": "12 Alternative treatments for mild to moderate PCP include dapsone/ trimethoprim, clindamycin/primaquine, and atovaquone. IV pentami dine is the treatment of choice for severe disease in the patient unable 8 to tolerate TMP/SMX. For patients with a Pao <70 mmHg or with an 6 a–a gradient >35 mmHg, adjunct glucocorticoid therapy should be used in addition to specific antimicrobials. Overall, treatment should be continued for 21 days and followed by secondary prophylaxis. 2 Prophylaxis for PCP is indicated for any HIV-infected individual who 0 has experienced a prior bout of PCP, any patient with a CD4+ T cell count of <200/μL or a CD4 percentage <15, any patient with unex-"}, {"id": "pubmed23n1069_15658", "title": "<i>In Vitro</i> Activity and <i>In Vivo</i> Efficacy of Cefiderocol against Stenotrophomonas maltophilia.", "score": 0.00980392156862745, "content": "Cefiderocol is a novel siderophore cephalosporin antibiotic with broad coverage against difficult-to-treat Gram-negative bacteria, including those resistant to carbapenems. Its activity against <iStenotrophomonas maltophilia</i was investigated <iin vitro</i against clinical isolates and in lung infection models using strains either resistant (SR202006) or susceptible (SR201934, SR200614) to trimethoprim-sulfamethoxazole. Cefiderocol demonstrated potent <iin vitro</i activity against all 217 <iS. maltophilia</i clinical isolates tested (MIC<sub50</sub, 0.063 μg/ml; MIC<sub90</sub, 0.25 μg/ml). Cefiderocol also demonstrated low MICs against the trimethoprim-sulfamethoxazole-resistant <iS. maltophilia</i strains (i.e., SR202006; MIC, 0.125 μg/ml). In a neutropenic mouse lung infection model, cefiderocol (30 mg/kg body weight and 100 mg/kg) demonstrated a significant, dose-dependent reduction in the lung viable bacteria cell count compared with untreated controls in <iS. maltophilia</i infection and was the only antibiotic tested to show a similar significant effect in a trimethoprim-sulfamethoxazole-resistant <iS. maltophilia</i infection. In immunocompetent rat lung infection models of <iS. maltophilia</i, humanized dosing of cefiderocol (2 g every 8 h) and meropenem (1 g every 8 h) revealed pharmacokinetic profiles similar to those in human subjects, and the humanized cefiderocol dosing significantly reduced the lung viable bacteria cell count compared with baseline controls, which received no intervention. Together, the results from these studies suggest that cefiderocol could provide an effective alternative treatment option for <iS. maltophilia</i infections in the lower respiratory tract, particularly strains resistant to empirical antibiotics, such as trimethoprim-sulfamethoxazole or minocycline."}, {"id": "pubmed23n0734_19045", "title": "Evaluation of the novel combination of high-dose daptomycin plus trimethoprim-sulfamethoxazole against daptomycin-nonsusceptible methicillin-resistant Staphylococcus aureus using an in vitro pharmacokinetic/pharmacodynamic model of simulated endocardial vegetations.", "score": 0.009708737864077669, "content": "Daptomycin-nonsusceptible (DNS) Staphylococcus aureus is found in difficult-to-treat infections, and the optimal therapy is unknown. We investigated the activity of high-dose (HD) daptomycin plus trimethoprim-sulfamethoxazole de-escalated to HD daptomycin or trimethoprim-sulfamethoxazole against 4 clinical DNS methicillin-resistant S. aureus (MRSA) isolates in an in vitro pharmacokinetic/pharmacodynamic model of simulated endocardial vegetations (10(9) CFU/g). Simulated regimens included HD daptomycin at 10 mg/kg/day for 14 days, trimethoprim-sulfamethoxazole at 160/800 mg every 12 h for 14 days, HD daptomycin plus trimethoprim-sulfamethoxazole for 14 days, and the combination for 7 days de-escalated to HD daptomycin for 7 days and de-escalated to trimethoprim-sulfamethoxazole for 7 days. Differences in CFU/g (at 168 and 336 h) were evaluated by analysis of variance (ANOVA) with a Tukey's post hoc test. Daptomycin MICs were 4 μg/ml (SA H9749-1, vancomycin-intermediate Staphylococcus aureus; R6212, heteroresistant vancomycin-intermediate Staphylococcus aureus) and 2 μg/ml (R5599 and R5563). Trimethoprim-sulfamethoxazole MICs were ≤0.06/1.19 μg/ml. HD daptomycin plus trimethoprim-sulfamethoxazole displayed rapid bactericidal activity against SA H9749-1 (at 7 h) and R6212 (at 6 h) and bactericidal activity against R5599 (at 72 h) and R5563 (at 36 h). A ≥8 log(10) CFU/g decrease was observed with HD daptomycin plus trimethoprim-sulfamethoxazole against all strains (at 48 to 144 h), which was maintained with de-escalation to HD daptomycin or trimethoprim-sulfamethoxazole at 336 h. The combination for 14 days and the combination for 7 days de-escalated to HD daptomycin or trimethoprim-sulfamethoxazole was significantly better than daptomycin monotherapy (P &lt; 0.05) and trimethoprim-sulfamethoxazole monotherapy (P &lt; 0.05) at 168 and 336 h. Combination therapy followed by de-escalation offers a novel bactericidal therapeutic alternative for high-inoculum, serious DNS MRSA infections."}, {"id": "InternalMed_Harrison_16192", "title": "InternalMed_Harrison", "score": 0.009708737864077669, "content": "No definitive trials have defined the best therapeutic algorithm for patients in whom TMP-SMX treatment for PCP is failing. If no other treatable infectious or noninfectious processes are detected and pulmonary dysfunction appears to be due to PCP alone, many authorities would switch from TMP-SMX to either IV pentamidine or IV clindamycin plus oral primaquine. Some authorities would add the second drug or drug combination to TMP-SMX rather than switching regimens. If patients are not already receiving them, glucocorticoids should be added to the regimen; the dosage and regimen, which are usually chosen empirically, depend on what glucocorticoid regimen (if any) the patient was receiving when PCP therapy was begun."}, {"id": "wiki20220301en017_53006", "title": "Haemophilus ducreyi", "score": 0.009615384615384616, "content": "Pathogenesis H. ducreyi is an opportunistic microorganism that infects its host by way of breaks in the skin or epidermis. Inflammation then takes place as the area of infection is inundated with lymphocytes, macrophages, and granulocytes. This pyogenic inflammation causes regional lymphadenitis in the sexually transmitted disease chancroid. Diagnosis Although antigen detection, serology, and genetic amplification methods are sometimes used to diagnose infections with H. ducreyi and the genetic tests have greater sensitivity, they are not widely available, so cultures are currently considered the \"gold standard\" test. Treatment The first line treatments are one of four options : azithromycin 1 g orally in a single dose, ceftriaxone 250 mg intramuscularly in a single dose, ciprofloxacin 500 mg orally 2 times a day for 3 days, or erythromycin base 500 mg orally 3 times a day for 7 days. See also Sexually transmitted disease References"}, {"id": "pubmed23n1117_12611", "title": "On the Treatment of <i>Pneumocystis jirovecii</i> Pneumonia: Current Practice Based on Outdated Evidence.", "score": 0.009615384615384616, "content": "<iPneumocystis jirovecii</i pneumonia (PCP) is a common opportunistic infection causing more than 400000 cases annually worldwide. Although antiretroviral therapy has reduced the burden of PCP in persons with human immunodeficiency virus (HIV), an increasing proportion of cases occur in other immunocompromised populations. In this review, we synthesize the available randomized controlled trial (RCT) evidence base for PCP treatment. We identified 14 RCTs that were conducted 25-35 years ago, principally in 40-year-old men with HIV. Trimethoprim-sulfamethoxazole, at a dose of 15-20 mg/kg per day, is the treatment of choice based on historical practice rather than on quality comparative, dose-finding studies. Treatment duration is similarly based on historical practice and is not evidence based. Corticosteroids have a demonstrated role in hypoxemic patients with HIV but have yet to be studied in RCTs as an adjunctive therapy in non-HIV populations. The echinocandins are potential synergistic treatments in need of further investigation."}, {"id": "pubmed23n1132_24461", "title": "Optimizing Antimicrobial Dosing for Critically Ill Patients with MRSA Infections: A New Paradigm for Improving Efficacy during Continuous Renal Replacement Therapy.", "score": 0.009523809523809525, "content": "The dosage regimen of vancomycin, teicoplanin and daptomycin remains controversial for critically ill patients undergoing continuous renal replacement therapy (CRRT). Monte Carlo simulation was applied to identify the optimal regimens of antimicrobial agents in patients with methicillin-resistant <iStaphylococcus aureus</i (MRSA) infections based on the mechanisms of different CRRT modalities on drug clearance. The optimal vancomycin dosage for patients received a CRRT doses ≤ 30 mL/kg/h was 20 mg/kg loading dose followed by 500 mg every 8 h, while 1 g every 12 h was appropriate when 35 mL/kg/h was prescribed. The optimal teicoplanin dosage under a CRRT dose ≤ 25 mL/kg/h was four loading doses of 10 mg/kg every 12 h followed by 10 mg/kg every 48 h, 8 mg/kg every 24 h and 6 mg/kg every 24 h for continuous veno-venous hemofiltration, continuous veno-venous hemodialysis and continuous veno-venous hemodiafiltration, respectively. When the CRRT dose increased to 30-35 mL/kg/h, the teicoplanin dosage should be increased by 30%. The recommended regimen for daptomycin was 6-8 mg/kg every 24 h under a CRRT dose ≤ 25 mL/kg/h, while 8-10 mg/kg every 24 h was optimal under 30-35 mg/kg/h. The CRRT dose has an impact on probability of target attainment and CRRT modality only influences teicoplanin."}, {"id": "InternalMed_Harrison_13594", "title": "InternalMed_Harrison", "score": 0.009523809523809525, "content": "Culture Results Intensive Phase Continuation Phase Extension of Total Treatment Culture positive HRZE for 2 months, daily or intermit-HR for 4 months, daily or 5 d/wk To 9 months, if 2 months of Z is not completed or culture tent (with dose adjustment) or conversion is prolonged and cavitation is evident on plain radiographa HR for 4 months, intermittent (with dose adjustment) Culture negative HRZE for 2 months 2 months To 6 months, if patient is infected with HIV Extrapulmonary HRZE for 2 months HR for 4–7 months, daily or 5 d/wkb To 9–12 months in TB meningitis. Some recommend 9 months for bone/joint TB. Resistant to H QRZEc or, less often, RZES for 6 months … Prolonged culture conversion, cavitation Resistant to R HZEQc (IAd) for 2 months HEQ(S) for 10–16 months Prolonged culture conversion, delayed response"}, {"id": "article-17474_16", "title": "Amoxicillin -- Administration -- Amoxicillin Dosages", "score": 0.009433962264150943, "content": "Adults: For adults, the recommended dosage of amoxicillin is 750 to 1750 mg/d, divided into doses and administered every 8 to 12 hours. Pediatric: For pediatric patients 3 months and older, the recommended amoxicillin dosage is 20 to 45 mg/kg/d, divided into doses and administered every 8 to 12 hours. H pylori infection (triple therapy): For the treatment of H pylori infection, the recommended dosing for triple therapy involves administering 1 g of amoxicillin, 500 mg of clarithromycin, and 30 mg of lansoprazole twice daily (every 12 hours) for 14 days. H pylori infection (dual therapy): For dual therapy against H pylori infection, the recommended dosing is 1 g of amoxicillin and 30 mg of lansoprazole, each administered 3 times daily."}, {"id": "InternalMed_Harrison_16713", "title": "InternalMed_Harrison", "score": 0.009433962264150943, "content": "Of the currently available agents, trimethoprim-sulfamethoxazole (TMP-SMX) appears to be an effective alternative for treatment of TE in resource-poor settings where the preferred combination of pyrimethamine plus sulfadiazine is not available. The daily dose of TMP-SMX (one double-strength tablet) that is recommended as the preferred regimen for prophylaxis of PcP is effective against TE. If patients cannot tolerate TMP-SMX, the recommended alternative is dapsone-pyrimethamine, which likewise is effective against PcP. Atovaquone with or without pyrimethamine also can be considered. Prophylactic monotherapy with dapsone, pyrimethamine, azithromycin, clarithromycin, or aerosolized pentamidine is probably insufficient. AIDS patients who are seronegative for Toxoplasma and are not receiving prophylaxis for PcP should be retested for IgG antibody to Toxoplasma if their CD4+ T cell count drops to <100/µL. If seroconversion has taken place, then the patient should be given prophylaxis as"}, {"id": "wiki20220301en089_23285", "title": "Vesicoureteral reflux", "score": 0.009345794392523364, "content": "Medical treatment Medical treatment entails low dose antibiotic prophylaxis until resolution of VUR occurs. Antibiotics are administered nightly at half the normal therapeutic dose. The specific antibiotics used differ with the age of the patient and include: Amoxicillin or ampicillin – infants younger than 6 weeks Trimethoprim-sulfamethoxazole (co-trimoxazole) – 6 weeks to 2 months After 2 months the following antibiotics are suitable: Nitrofurantoin {5–7 mg/kg/24hrs} Nalidixic acid Bactrim Trimethoprim Cephalosporins Urine cultures are performed 3 monthly to exclude breakthrough infection. Annual radiological investigations are likewise indicated. Good perineal hygiene, and timed and double voiding are also important aspects of medical treatment. Bladder dysfunction is treated with the administration of anticholinergics."}, {"id": "pubmed23n0491_17802", "title": "[Not Available].", "score": 0.009345794392523364, "content": "Rhodococcus equi is a facultative intracellular, obligate aerobe, partially acid fast, gram-positive pathogen that causes cavitary pneumonia in animals and immunocompromised humans. We describe 8 cases of R. equi pneumonia in patients with advanced HIV infection (CD4 counts less than 100/mm3), 7 males and 1 female (mean age 30.8 years), observed between 1991 and 1994. A history of exposure to farm animals was found in 4 patients. The most common presenting symptoms were fever, malaise, dyspnea, cough and hemoptysis, chest pain and weight loss. Chest x-rays showed tipical focal area of consolidation throughout the lung (3 upper, 3 lower and 2 middle fields) associated with cavitation in 4 cases. The definitive diagnosis in our hands was delayed only in the first case in which conflicting data resulted from blood culture (Bacillus sp. isolation) and sputum examen (acid-fast bacterium in the Ziehl-Neelsen stain). Final microbiological diagnosis depended on blood cultures (n=5), bronchoalveolar lavage (n=1), sputum (n=1), lung biopsy (n=1). All the patients were treated with prolonged courses of antibiotic therapy (259 days, range 120-340 in 6 dead patients; more than one year and two months respectively in two patients alive). According to microbial susceptibility TMP/SMX, vancomycin, imipenem, rifampin, aminoglycosides, macrolides and quinolons were more frequently used. Resistant R. equi mutants were selected during therapy with TMP/SMX (n=2), rifampin (n=1) and erythromycin (n=1). Five patient underwent pulmonary lobectomy after exclusion of metastatic bacterial lesions. Only 2 patients are alive, one after 365 days of antibiotic therapy and upper lung lobectomy, one after 60 days of antibiotic therapy. Optimal antimicrobial therapy and the role of surgery remain, in our experience, uncertain."}, {"id": "wiki20220301en014_132704", "title": "Creatine", "score": 0.009259259259259259, "content": "Maintenance phase After the 5–7 day loading phase, muscle creatine stores are fully saturated and supplementation only needs to cover the amount of creatine broken down per day. This maintenance dose was originally reported to be around 2–3 g/day (or 0.03 g/kg/day), however, recent studies have suggested 3–5 g/day maintenance dose to maintain saturated muscle creatine. Absorption Endogenous serum or plasma creatine concentrations in healthy adults are normally in a range of 2–12 mg/L. A single 5 gram (5000 mg) oral dose in healthy adults results in a peak plasma creatine level of approximately 120 mg/L at 1–2 hours post-ingestion. Creatine has a fairly short elimination half life, averaging just less than 3 hours, so to maintain an elevated plasma level it would be necessary to take small oral doses every 3–6 hours throughout the day. Clearance It has been shown that once supplementation of creatine stops, muscle creatine stores return to baseline in 4–6 weeks."}, {"id": "InternalMed_Harrison_2633", "title": "InternalMed_Harrison", "score": 0.009259259259259259, "content": "PART 2 Cardinal Manifestations and Presentation of Diseases Symptoms consistent with viral URI? Risk factors for HIV, gonorrhea? Group A Strep RADT or throat culture Penicillin allergy? No streptococcal testing Test accordingly Symptomatic management • Penicillin G 1.2 million units IM × 1, or • Penicillin VK 250 mg orally QID, or 500 mg orally BID, or • Amoxicillin 500 mg orally BID • Cephalexin 500 mg orally BID or TID (only if non-anaphylactic penicillin allergy), or • Azithromycin† 500 mg orally QD × 5 days, or • Clindamycin 300 mg orally TID Positive NoNegative* NOTE: All treatment durations are for 10 days with appropriate follow-up,unless otherwise specified.Yes Yes Yes No No No *Confirmation of a negative rapid antigen-detection test by a throat culture is not required in adults. †Macrolides do not treat F. necrophorum, a cause of pharyngitis in young adults (see text). Abbreviations: URI, upper respiratory infection; RADT, rapid antigen detection test"}, {"id": "pubmed23n0609_8033", "title": "Severe sepsis caused by Arcanobacterium haemolyticum: a case report and review of the literature.", "score": 0.009174311926605505, "content": "To describe a case of severe sepsis, cavitary pneumonia, and pyomyositis caused by Arcanobacterium haemolyticum. An 18-year-old male with a medical history significant for mild asthma presented to the emergency department complaining of a 7-day history of fever, diffuse myalgias, nausea, vomiting, diarrhea, and pain in his right upper quadrant, right shoulder, and left thigh. Cultures of blood, bronchoalveolar fluid, and surface and surgical swabs from the patient's left lower extremity grew A. haemolyticum. The patient was successfully treated with intravenous penicillin G 4 million units every 4 hours and azithromycin 500 mg once daily for 14 days. Within 36 hours after initiation of focused therapy, he became afebrile, pain decreased, and pulmonary symptoms abated. Oral azithromycin 500 mg/day for an additional 3 weeks was prescribed on discharge, and the patient showed no relapse at 2-month follow-up. A. haemolyticum is a weakly acid-fast, branching gram-positive bacillus most commonly implicated in pharyngitis in healthy adolescents and skin and soft-tissue infections in older, immunocompromised patients. Systemic infections are rarely reported in the literature. This organism remains susceptible to most classes of antimicrobials, including penicillins, cephalosporins, carbapenems, macrolides, tetracyclines, clindamycin, and vancomycin. Routine resistance has been reported only with trimethoprim/sulfamethoxazole. To our knowledge, there are no published case reports of severe sepsis caused by A. haemolyticum. While treatment options are numerous, we recommend the use of intravenous penicillin or a cephalosporin as first-line pharmacologic management of deep-seated infections caused by this rare organism."}, {"id": "InternalMed_Harrison_15145", "title": "InternalMed_Harrison", "score": 0.009174311926605505, "content": "FIGuRE 226-33 A. Decrease in the incidence of opportunistic infections and Kaposi’s sarcoma in HIV-infected individuals with CD4+ T cell counts <100/μL from 1992 through 1998. (Adapted and updated from FJ Palella et al: N Engl J Med 338:853, 1998, and JE Kaplan et al: Clin Infect Dis 30[S1]:S5, 2000, with permission.) B. Quarterly incidence rates of cytomegalovirus (CMV), Pneumocystis jiroveci pneumonia (PCP), and Mycobacterium avium complex (MAC) from 1995 to 2001. (From FJ Palella et al: AIDS 16:1617, 2002.) plained fever for >2 weeks, and any patient with a recent history of oropharyngeal candidiasis. The preferred regimen for prophylaxis is TMP/SMX, one double-strength tablet daily. This regimen also provides protection against toxoplasmosis and some bacterial respiratory pathogens. For patients who cannot tolerate TMP/SMX, alternatives for prophylaxis include dapsone plus pyrimethamine plus leucovorin, aerosolized pentamidine administered by the Respirgard II nebulizer, and"}, {"id": "pubmed23n0298_17347", "title": "The disposition of five therapeutically important antimicrobial agents in llamas.", "score": 0.00909090909090909, "content": "The disposition of five therapeutic antimicrobial agents was studied in llamas (Lama glama) following intravenous bolus administration. Six llamas were each given ampicillin, tobramycin, trimethoprim, sulfamethoxazole, enrofloxacin and ceftiofur at a dose of 12 mg/kg, 1 mg/kg, 3 mg/kg, 15 mg/kg, 5 mg/kg, and 2.2 mg/kg of body weight, respectively, with a wash out period of at least 3 days between treatments. Plasma concentrations of these antimicrobial agents over 12 h following i.v. bolus dosing were determined by reverse phase HPLC. Disposition of the five antimicrobial agents was described by a two compartment open model with elimination from the central compartment, and also by non-compartmental methods. From compartmental analysis, the elimination rate constant, half-life, and apparent volume of distribution in the central compartment were determined. Statistical moment theory was used to determine noncompartmental pharmacokinetic parameters of mean residence time, clearance, and volume of distribution at steady state. Based on the disposition parameters determined, and stated assumptions of likely effective minimum inhibitory concentrations (MIC) a dose and dosing interval for each of five antimicrobial agents were suggested as 6 mg/kg every 12 h for ampicillin; 4 mg/kg once a day or 0.75 mg/kg every 8 h for tobramycin; 3.0 mg/kg/15 mg/kg every 12 h for trimethoprim/sulfamethoxazole; 5 mg/kg every 12 h for enrofloxacin; and 2.2 mg/kg every 12 h for ceftiofur sodium for llamas. Steady-state peak and trough plasma concentrations were also predicted for the drugs in this study for llamas."}, {"id": "pubmed23n0643_15158", "title": "Good outcome with trimethoprim 10 mg/kg/day-sulfamethoxazole 50 mg/kg/day for Pneumocystis jirovecii pneumonia in HIV infected patients.", "score": 0.00909090909090909, "content": "Pneumocystis jirovecii pneumonia (PCP) in human immunodeficiency virus (HIV)-infected patients is usually treated with trimethoprim (TMP)-sulfamethoxazole (SMX) 1920 mg 3 times daily (approximately equivalent to TMP 15 mg/kg/day-SMX 75 mg/kg/day) for 21 days. Pharmacokinetic data suggest that lower doses would be equally efficacious and might be associated with a lower incidence of adverse effects. We conducted a retrospective review of case notes for the first episode of laboratory-confirmed PCP in HIV-infected patients treated at Auckland City Hospital, from January 1991 through December 2007. Seventy-three of 84 (87%) patients were treated with TMP-SMX 960 mg 4 times daily or 3 times daily (approximately TMP 10 mg/kg/day-SMX 50 mg/kg/day). The overall mortality was 5/73 (7%). The mortality in patients with severe disease (transcutaneous oxygen saturation on admission &lt; or =84%) was 3/16 (19%) and in patients admitted to the intensive care unit was 5/9 (56%). Fifteen of 73 (21%) patients required a change to an alternative treatment regimen because of adverse effects (rash in 10, rash plus fever in 3, neutropenia in 1, fever plus headache in 1). Treatment of PCP in adult HIV-infected patients with TMP-SMX 960 mg QID or TID appears to have comparable efficacy to treatment with higher doses and to be associated with a lower rate of treatment limiting adverse effects."}, {"id": "wiki20220301en481_31445", "title": "Histoplasma duboisii", "score": 0.009009009009009009, "content": "Isolated lesions may be cleared by surgical removed, although some have been known to heal spontaneously. In contrast, deep lesions and disseminated disease require antifungal drug therapy. To date, no antifungal drug studies have specifically investigated the agent of African histoplasmosis. Hence most treatment approaches are based on the therapeutic strategies used to treat classical histoplasmosis caused by H. capsulatum. Amphotericin B is a mainstay of antifungal treatment, with a recommended dose of 1 mg/kg/day, culminating in a minimum dose of 2 g. Clinical response is typically apparent after 2 weeks of intravenous administration. Ketoconazole is also effective, starting at 600–800 mg/day for 3 months followed by a reduced dose of 400 mg/day for a further 6 months. The organism is also thought to be susceptible to fluconazole in vivo. A multi-month course of Amphotericin B followed by itraconazole has been suggested for complicated infection in immunodeficient individuals."}, {"id": "wiki20220301en193_14898", "title": "Austin Aztex U23", "score": 0.009009009009009009, "content": "History"}, {"id": "InternalMed_Harrison_10928", "title": "InternalMed_Harrison", "score": 0.008942922608665914, "content": "Ampicillin 300 (mg/kg)/d, q6h 12 g/d, q4h Cefepime 150 (mg/kg)/d, q8h 6 g/d, q8h Cefotaxime 225-300 (mg/kg)/d, q6h 12 g/d, q4h Ceftriaxone 100 (mg/kg)/d, q12h 4 g/d, q12h Ceftazidime 150 (mg/kg)/d, q8h 6 g/d, q8h Gentamicin 7.5 (mg/kg)/d, q8hb 7.5 (mg/kg)/d, q8h Meropenem 120 (mg/kg)/d, q8h 6 g/d, q8h Metronidazole 30 (mg/kg)/d, q6h 1500–2000 mg/d, q6h Nafcillin 100–200 (mg/kg)/d, q6h 9–12 g/d, q4h Penicillin G 400,000 (U/kg)/d, q4h 20–24 million U/d, q4h Vancomycin 45-60 (mg/kg)/d, q6h 45-60 (mg/kg)d, q6–12hb aAll antibiotics are administered intravenously; doses indicated assume normal renal and hepatic function. bDoses should be adjusted based on serum peak and trough levels: gentamicin therapeutic level: peak: 5–8 μg/mL; trough: <2 μg/mL; vancomycin therapeutic level: peak: 25–40 μg/mL; trough: 5–15 μg/mL. Meningitis, Encephalitis, Brain Abscess, and Empyema White blood cells 10/μL to 10,000/μL; neutrophils predominate Glucose <2.2 mmol/L (<40 mg/dL) CSF/serum glucose <0.4"}, {"id": "wiki20220301en088_13113", "title": "Pralidoxime", "score": 0.008928571428571428, "content": "Pralidoxime has an important role in reversing paralysis of the respiratory muscles but due to its poor blood–brain barrier penetration, it has little effect on centrally-mediated respiratory depression. Atropine, which is choice of drug to antagonise the muscarinic effects of organophosphates, is administered even before pralidoxime during the treatment of organophosphate poisoning. While the efficacy of atropine has been well-established, clinical experience with pralidoxime has led to widespread doubt about its efficacy in treatment of organophosphorus poisoning. Dosage Adults: 30 mg/kg (typically 1–2 g), administered by intravenous therapy over 15–30 minutes, repeated 60 minutes later. It can also be given as a 500 mg/h continuous IV infusion. Children: 20–50 mg/kg followed by a maintenance infusion at 5–10 mg/kg/h. Intravenous infusions can lead to respiratory or cardiac arrest if given too quickly."}, {"id": "pubmed23n0076_7007", "title": "[The treatment of pulmonary tuberculosis in a special hospital. Its evolution from 1948 to 1986].", "score": 0.008928571428571428, "content": "Nine hundred and sixty six patients diagnosed of pulmonary and/or pleural tuberculosis and admitted to a specialized hospital from 1948 to 1986, have been retrospectively analyzed, investigating their treatment and evolution. Sixty two percent of patients did not fulfil pharmaceutical treatment as far as number and dose of drugs, evolving through the decades (50, 60, 70, and 80s) with a 100%, 82%, 37% and 3% respectively. A 13% of patients did not receive any chemotherapy, 16% underwent surgery, and 53% received a second treatment. Real or hidden monotherapy was given to 38% of patients. Isoniazide has been the most uniformly used drug. Streptomycin has been the most frequently underdosed used drug. Sputum culture turned negative in 42% and 51% of patients during the first 3 and 6 months respectively, with a 42% of positives persisting after one year and a 30% when discharged. A statistically significant difference is observed when comparing all the variables between admitted patients up to 1969 and from 69 to 86 in favor of the second period."}, {"id": "pubmed23n0066_10510", "title": "Therapy for women hospitalized with acute pyelonephritis: a randomized trial of ampicillin versus trimethoprim-sulfamethoxazole for 14 days.", "score": 0.008849557522123894, "content": "The efficacy of the traditionally recommended ampicillin (Amp) plus gentamicin (GM) regimen was compared with that of a trimethoprim-sulfamethoxazole (TMP/SMZ)-plus-GM regimen and the adequacy of 14 days total therapy for acute uncomplicated pyelonephritis (AUPN). Eighty-five women hospitalized for AUPN were randomly assigned to receive either Amp, 1 g intravenously (iv) every 6 h for 3 days, then 500 mg orally four times daily, or TMP/SMZ, 160/800 mg iv every 12 h for 3 days, then 160/800 mg orally twice daily. Initially, all patients also received GM every 8 h iv (mean, 606 doses). Antimicrobial resistance necessitated modifying therapy of 14 (32%) of the Amp recipients but of none of the TMP/SMZ recipients (P less than .001). Both regimens produced a satisfactory bacteriologic and clinical response in all cases. Reinfection occurred in 11% of Amp and in 8% of TMP/SMZ recipients. No patient experienced relapsing infection. The TMP/SMZ regimen was less costly and less likely to require modification due to antimicrobial resistance."}, {"id": "InternalMed_Harrison_11753", "title": "InternalMed_Harrison", "score": 0.008849557522123894, "content": "Resistant to methicillin Clindamycin (300–450 mg/kg tid), Same options as under “Drug of It is important to know the antibiotic susceptibility of TMP-SMX (1 or 2 ds tablets bid), mino-Choice” isolates in the specific geographic region. All draincycline or doxycycline (100 mg q12hb), age should be cultured. linezolid (600 mg bid) or tedizolid (200 mg once daily) aRecommended dosages are for adults with normal renal and hepatic function. bThe dosage must be adjusted for patients with reduced creatinine clearance. cFor the treatment of prosthetic-valve endocarditis, the addition of gentamicin (1 mg/kg q8h) and rifampin (300 mg PO q8h) is recommended, with adjustment of the gentamicin dosage if the creatinine clearance rate is reduced. dDaptomycin cannot be used for the treatment of pneumonia. eVancomycin-resistant S. aureus isolates from clinical infections have been reported."}, {"id": "InternalMed_Harrison_9509", "title": "InternalMed_Harrison", "score": 0.008831417624521073, "content": "Vancomycin, 15 mg/kg q12hb; Ceftriaxone, 2 g q12h; Metronidazole, 500 mg q8h Vancomycin, 15 mg/kg q12hb; Ceftriaxone, 2 g q12h Azithromycin, 500 mg PO × 1, then 250 mg PO qd × 4 days A respiratory fluoroquinolone (moxifloxacin, 400 mg IV/PO qd; gemifloxacin, 320 mg PO qd; or levofloxacin, 750 mg IV/PO qd); A β-lactam (cefotaxime, ceftriaxone, or ampicillinsulbactam) plus azithromycin Azithromycin or a respiratory fluoroquinolone An antipseudomonal β-lactam (cefepime, 1–2 g q8–12 h; ceftazidime, 2 g q8h; imipenem, 1 g q8h; meropenem, 1 g q8h; or piperacillin-tazobactam, 4.5 g q6h); An antipseudomonal fluoroquinolone (levofloxacin or ciprofloxacin, 400 mg q8h) or an aminoglycoside (amikacin, 20 mg/kg q24hc; gentamicin, 7 mg/kg q24he; or tobramycin, 7 mg/kg q24he) Cefoxitin, 2 g q6h; A combination of metronidazole (500 mg q8–12h) plus cefazolin (1–2 g q8h) or cefuroxime (1.5 g q8h) or ceftriaxone (1–2 g q12–24h) or cefotaxime (1–2 g q6–8h)"}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": true, "char_ranges": [[136, 264]], "word_ranges": [[22, 48]], "text": "Fixation in this case is better external to avoid all the material around the affected area, so 4 is correct and 1 and 2 is not."}, "2": {"exist": true, "char_ranges": [[136, 264]], "word_ranges": [[22, 48]], "text": "Fixation in this case is better external to avoid all the material around the affected area, so 4 is correct and 1 and 2 is not."}, "3": {"exist": true, "char_ranges": [[265, 334]], "word_ranges": [[48, 59]], "text": "The 3 would be considered after expiration of the infectious picture."}, "4": {"exist": true, "char_ranges": [[136, 264]], "word_ranges": [[22, 48]], "text": "Fixation in this case is better external to avoid all the material around the affected area, so 4 is correct and 1 and 2 is not."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The first thing is to treat the infection and for this we must remove all the osteosynthesis material, debride and give antibiotherapy. Fixation in this case is better external to avoid all the material around the affected area, so 4 is correct and 1 and 2 is not. The 3 would be considered after expiration of the infectious picture. TREATMENT - Suppressive antibiotic treatment: indicated in patients with Ciemy type C, it consists of prolonged oral antibiotic therapy for at least 6 months to \"cool\" the exacerbation of the clinical picture. - Curative treatment: includes a first surgery with aggressive tumor-like debridement of all affected tissues, profuse irrigation, stabilization with external fixator if stability is compromised, and eventual filling of the cavities with antibiotic-releasing substances. After prolonged periods of antibiotic therapy selected according to the antibiograms of the cultures, and once it is certain that the infection has been cured, special techniques for the reconstruction of the bone defect and to achieve adequate coverage of soft tissues should be considered. We are dealing with an infection associated with an implant (intramedullary nail) and pseudoarthrosis of the tibia fracture (absence of union after 11 months). The infection is understood by the clinical manifestations (suppuration, nonunion) and the history of diabetes and open fracture. Management overlaps with the management of chronic osteomyelitis. Isolated antibiotherapy (option 2) is indicated only in patients with severe comorbidity in whom surgical treatment would be more aggressive than continuing the disease. Nail dynamization associated with broad-spectrum antibiotherapy (option 1) is not indicated either, because (a) it has not demonstrated benefit in established pseudarthrosis and (b) we have the same issue as option 1, we do not eliminate biofilm. Option 3, discussed, would be considered only if there was no associated infection.", "full_answer_no_ref": "The first thing is to treat the infection and for this we must remove all the osteosynthesis material, debride and give antibiotherapy. Fixation in this case is better external to avoid all the material around the affected area, so [HIDDEN] and [HIDDEN]. The [HIDDEN] would be considered after expiration of the infectious picture. TREATMENT - Suppressive antibiotic treatment: indicated in patients with Ciemy type C, it consists of prolonged oral antibiotic therapy for at least 6 months to \"cool\" the exacerbation of the clinical picture. - Curative treatment: includes a first surgery with aggressive tumor-like debridement of all affected tissues, profuse irrigation, stabilization with external fixator if stability is compromised, and eventual filling of the cavities with antibiotic-releasing substances. After prolonged periods of antibiotic therapy selected according to the antibiograms of the cultures, and once it is certain that the infection has been cured, special techniques for the reconstruction of the bone defect and to achieve adequate coverage of soft tissues should be considered. We are dealing with an infection associated with an implant (intramedullary nail) and pseudoarthrosis of the tibia fracture (absence of union after 11 months). The infection is understood by the clinical manifestations (suppuration, nonunion) and the history of diabetes and open fracture. Management overlaps with the management of chronic osteomyelitis. Isolated antibiotherapy (option 2) is indicated only in patients with severe comorbidity in whom surgical treatment would be more aggressive than continuing the disease. Nail dynamization associated with broad-spectrum antibiotherapy (option 1) is not indicated either, because (a) it has not demonstrated benefit in established pseudarthrosis and (b) [HIDDEN]. [HIDDEN], discussed, would be considered only if there was no associated infection.", "full_question": "A 70-year-old woman, diabetic and hypertensive, who suffers a fall at home, presenting a 9 cm wound communicating with a fracture site of the right tibia. Radiographically, a short oblique fracture of the mid-distal third of the tibia was observed. An emergency operation was performed by cleaning (Friederich) and placement of an endomedullary steel-plated nail. At 11 months he presents with atrophic pseudarthrosis of the tibia with suppuration in the wound area. What will be his best immediate therapeutic option?", "id": 474, "lang": "en", "options": {"1": "Triple antibiotherapy (gram-positive, gram-negative and anaerobic) and cleaning of the surgical wound, removing the distal locks to promote bone consolidation.", "2": "Expectant attitude and antibiotic treatment with quinolones.", "3": "Autologous graft and growth factors (BMP 2 and 7) to stimulate the bone consolidation process, which is slowing.", "4": "Removal of the nail, debridement, placement of external fixator and antibiotherapy adjusted to culture results.", "5": NaN}, "question_id_specific": 142, "type": "ORTHOPEDIC SURGERY AND TRAUMATOLOGY", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0819_12704", "title": "[Advanced bone graft combined with locking compression plate for the treatment of middle and distal tibia nonunion].", "score": 0.017216117216117217, "content": "To explore methods of treating middle and distal tibia nonunion with the treatment of advanced bone graft combined with locking compression plate. From January 2011 to December 2012, 12 patients with middle and distal tibia nonunion were treated with advanced bone graft combined with locking compression plate. Among patients, there were 8 males and 4 females aged from 20 to 69 with an average of 47 years old. The time from first injuries to bone nonunion was from 9 months to 5 years, avergaed 19 months. Four cases were treated with external fixation, 6 cases were treated with plate fixation, 2 cases of 12 patients occurred broken of plate and nail. Eleven patients were non-infective bone nonunion and 1 patient was infective bone nonunion. Preoperative X-ray and CT showed all patients had sequestration and formation of ossified bone with different degrees. Operative time, blood loss, wound healing were observed, fracture healing time was evaluated by postoperative X-ray. Johner-Wruhs scoring standards was used to evaluate ankle joint function after operation at 10 months. Operative time ranged from 90 to 185 min with an average of (125.00±20.15) min; blood loss ranged from 225 to 750 ml with an average of (415.00±120.00) ml. All patients were followed up from 10 months to 2.5 years with an average of 1.5 years. Postoperative X-ray showed bone union was formed around fracture after operation at 4 months in all patients, 3 cases obtained bone healing within 6 months after operation, 9 cases obtained from 8 to 12 months. No infection, injury of nerve and vessles, and broken of plate and nail were ocurred. According to Johner-Wruhs scoring at 10 months after operation, 10 cases obtained excellent results, 1 good and 1 moderate. Advanced bone graft combined with locking compression plate, which can build fracture multi-point supporting based on full compression of bone nonunion to get effective fixation, is an effective method in treating middle and distal tibia nonunion."}, {"id": "pubmed23n0948_10377", "title": "[Effectiveness of limbs shortening and re-lengthening in treatment of tibial infectious bone defect and chronic osteomyelitis].", "score": 0.015199637023593466, "content": "To evaluate the limbs shortening and re-lengthening in the treatment of tibial infectious bone defect and chronic osteomyelitis. Between January 2011 and April 2016, 19 cases of tibial infectious bone defect and chronic osteomyelitis were treated with the limbs shortening and re-lengthening technique. There were 13 males and 6 females, aged from 22 to 62 years (mean, 44 years). The causes of injury included traffic accident injury in 16 cases, crush injury in 1 case, and falling from height in 2 cases. One patient was infected after plate internal fixation of closed tibial fracture and 18 patients after external fixation of open tibial fractures (Gustilo type IIIB). The mean previous operation times was 3 times (range, 2-5 times). The time from injury to bone transport operation was 3-11 months (mean, 6.5 months). The bone defect length was 2.0-5.5 cm (mean, 4.3 cm) after debridement. After tibial shortening, limb peripheral blood supply should be checked after release of the tourniquet. Seven wounds were closed directly, 5 were repaired with adjacent skin flap, 5 were repaired with sural neurovascular flap, 1 was repaired with medial head of gastrocnemius muscle flap, and 1 underwent skin grafting. Single arm external fixator or ring type external fixator were used, and completely sawed off between 2 sets of external fixation screws at proximal and distal metaphysis of the tibia. Limb lengthening was performed after 1 week with the speed of 1 mm/d. All patients were followed up 10-36 months with an average of 14 months. Two cases delayed healing of the wound after operation, and the other wounds healed primarily. Natural healing of the opposite end of the bone were found in 18 cases, and 1 case had nonunion in the opposite end of the bone because of incomplete removal of lesion bone. There were 5 cases of slow growth of the callus, and healed smoothly by \"accordion\" technology and injecting red bone marrow in 4 cases, and by bone grafting and internal fixation in 1 case. The time of bone lengthening was 1-3 months, the prolongation index was 1.6-2.7 cm/month (2.20 cm/month). The bone healing time was 7-13 months (mean, 11.1 months). According to tibial stem diagnostic criteria Johner-Wruhs score, 9 cases were excellent, 8 cases were good, 2 cases were fair, with an excellent and good rate of 89.5%. Limbs shortening and re-lengthening is an effective method for the treatment of tibial infectious bone defect and chronic osteomyelitis, with the advantages of improving the immediate alignment of the osteotomy ends, significantly shortening the bone healing time of opposite ends of bone."}, {"id": "pubmed23n1004_14096", "title": "Unreamed Intra-Medullary Nail Versus Half Pin External Fixator in Grade III [A &amp; B] Open tibia fractures.", "score": 0.01430976430976431, "content": "Tibia fracture is the most common long bone fracture. The fractures of tibia are commonly open fractures due to subcutaneous position of the tibia. The choice of technique for stabilization of open tibia fractures includes - External fixation, unreamed intra-medullary nails [URTN], Reamed intra-medullary nails, ORIF with Plating. To evaluate &amp; compare the results of Unreamed Intra-Medullary Nail Versus Half Pin External Fixator in Grade III [A &amp; B] Open tibia fractures. This prospective clinical study [Randomized chit box] was done on 50 patients presenting to our institute within 24 h of injury. Only those who were skeletally mature with open tibia fracture Grade IIIA &amp; IIIB [Gustilo-Anderson] were included in this study. After initial management, radiological assessment was done. Following this adequate wound debridement, skeletal stabilization with either primary URTN or external fixator was done. Inspection and debridement were repeated at 48-h intervals until the wound was considered clean. 50 cases [25 each group] were compared in terms of - Final Alignment of the Fracture, Presence of Infection/Non-union/Mal-union, Hardware failure, Time to Bone Union, Number of Operative Procedures after index admission. Mean time to full weight bearing was 20.96 weeks in URTN group versus 24.8 weeks in Ex-fix group. 5 in URTN group required further surgery for non-union versus 11 patients in Ex-fix group. There were 6 significant pin track infection. Removal of nail was required in 1 case of deep infection. This study supports the use of the URTN over External fixator in the treatment of severe open tibia fractures."}, {"id": "pubmed23n1037_14413", "title": "Bone Crushing in Infected Pseudarthrosis - An Extraordinary Way to Treat Osteomyelitis Caused by Resistant Bacteria.", "score": 0.012755102040816327, "content": "Osteomyelitis with multiresistant bacteria in non-union following fracture treated with osteosynthesis requires complete removal of infected sequestrum and dead bone. For consecutive bone defects, it is frequently necessary to bridge with a fixator external. The treatment is not only challenging due to reduced bone stock but also characterized by decreased bioavailability of antibiotics. We report a two-step-surgery approach to preserve the bone stock using autologous cancellous bone in a bacterial infected non-union for subsequently leg length reconstruction. The 24-year-old male patient from Belarus was admitted to our department with persistent wound secretion and subsequent osteomyelitis of the right femur 3 years after initial surgery, several revisions, and several different antibiotic therapies. Biopsy revealed methicillin-resistant and borderline oxacillin-resistant Staphylococcus aureus. Firstly, the Ilizarov ring fixator was removed and a vigorous debridement was performed by refreshing the pseudarthrosis, removing of sequestrum, and dead bone. Finally, an AO fixator external was applied for 10 weeks combined with appropriate antibiotic treatment followed by 5 weeks antibiotic-free window. The bone defect was stabilized by a long gamma trochanteric nail after removal of the AO fixateurexterne. A wide resection of the fragments was performed and the resected bone tissue was crushed and placed adjacent to the nail. Noteworthy, the biopsies of both re-section sides revealed same germs as detected in initial biopsies. Thus, antibiotics were administered for additional 3 months. Frequent radiographic and clinical controls showed a remodeling of the femur during a period of 3 years and no signs of infection. Subsequently, we restored leg length of 4 cm using a fully implantable motorized lengthening nail. In the end, the patient achieved full weight-bearing with unlimited range of motion in hip and knee. No further germ could be revealed in biopsies. In this case report, we used autologous bone from the infected side, crushed, and placed it adjacent to an intramedullary nail. Crushed bone tissue might improve bioavailability of antibiotics when dealing with multiresistant bacteria in non-union healed fracture side. Furthermore, this approach was able to provide new bone formation in a limb resulting in full weight-bearing."}, {"id": "article-30176_18", "title": "Tibia Nonunion -- Treatment / Management -- Operative Treatments", "score": 0.012443972635055436, "content": "Single-stage approach: This involves surgical debridement of all non-viable tissues and bone ends, multiple cultures to be taken from the nonunion site or canal reaming followed by revision open reduction and internal fixation or exchange nailing. Antibiotic administration is tailored to culture results and sensitivities [24] [25] . When open treatment is essential, autologous bone grafting has been recommended as an adjunct [16] . Staged approach: usually indicated for septic tibial nonunion. This involves steps to control infection followed by definitive surgery."}, {"id": "InternalMed_Harrison_10506", "title": "InternalMed_Harrison", "score": 0.010204770097493506, "content": "As mentioned above, correlation between cultures of bone and those of wound swabs or wound punctures is poor. Antibiotic treatment should be based on bone culture. If no bone biopsy is performed, empirical therapy chosen in light of the most common infecting agents and the type of clinical syndrome should be given. Wound debridement combined with a 4to 6-week course of antibiotics has been shown to render amputation unnecessary in about two-thirds of patients. According to the 2012 Infectious Diseases Society of America Clinical Practice Guideline for the Diagnosis and Treatment of Diabetic Foot Infections, the following management strategies should be considered. If a foot ulcer is clinically infected, prompt empirical antimicrobial therapy may prevent progression to osteomyelitis. When the risk of methicillin-resistant S. aureus is considered high, an agent active against these strains (e.g., vancomycin) should be chosen. If the patient has not recently received antibiotics, the"}, {"id": "pubmed23n0894_7468", "title": "Management of Large Bone Defects in Diaphyseal Fractures by Induced Membrane Formation by Masquelet's Technique.", "score": 0.009900990099009901, "content": "Management of the large gap in long bone fractures is a challenging problem after compound injuries. A novel technique called as Masquelet's technique of \"induced membrane formation\", is used to bridge a gap of more than 5 cm using bone cement as a spacer in first stage and autologous cancellous bone graft to fill the gap in second stage. We present two different and difficult cases with bone defects after open injuries associated with long bone fractures in this paper. First case is a 50-year-old lady with grade IIIa open fracture right distal femur with intra-articular extension and bone loss. She underwent wound debriment, stabilization of the fracture with locking compression plate along with antibiotic cement spacer, which is removed latter and underwent bone grafting. Another is a 15-year-old boy with open grade IIIb fracture tibia and fibula (mid-distal third junction) of right leg, wound debridement and ankle spanning triangular external fixation was applied on same day and after two months, external fixation was removed due to florid infection and plaster of Paris was applied. Instead of the tedious and demanding treatment options like Ilizarov, a new technique described by Masquelet is used here. It uses bone cement as a spacer to fill the cavity to form pseudo-membrane around it and in the second stage autologous cancellous bone graft fills the gap of even more than 5 cm, to achieve union. The membrane also secretes vascular and osteo-inductive factors to stimulate bone regeneration and also prevents resorption of the bone graft and achieves early fracture healing avoiding tedious options like bone transport in external fixator. By this two staged technique, union occurred clinically and radiologically in these two cases."}, {"id": "pubmed23n0039_12145", "title": "[Infected tibial shaft pseudarthroses, treatment and results (author's transl)].", "score": 0.009900990099009901, "content": "In a follow up study of 47 patients with infected pseudarthrosis of tibia therapy and results are declared. The most important matter in therapy is the osteosyntesis with compression of the pseudarthrosis. In the majority of cases externe stabilisation is used; seldom compression plate or medullary nailing is indicated. Such cases require much experience in the therapy of bone infection, although if the stabilisation with the fibula is tryed. Flush drainage, suction drainage, antibiotic therapy and cancellous bone grafting are necessary for localisation and decreasing of infection and for induction and acceleration of bone union. In some cases amutation can't be prevented. With correct judgement and consequent treatment in the majority of cases bone union will be obtained."}, {"id": "pubmed23n1163_5845", "title": "Masquelet Technique and Proximal Tibial Autograft Utilizing Avitus® Bone Harvester for Severely Comminuted Open Distal Radius Fracture with Extensive Bone Loss: A Case Report.", "score": 0.00980392156862745, "content": "Distal radius fractures are one of the most common fractures in the United States. Treatment usually involves internal fixation using a volar Henry approach with placement of a volar locking plate. Optimal treatment becomes less apparent when significant bone loss occurs. No case of an open distal radius fracture treated using a staged Masquelet technique involving proximal tibial autograft is available in the literature. Herein, we describe and discuss a case report of a novel technique to treat a large (5 cm) bone defect for an open distal radius fracture. A 59-year-old man suffered an open, comminuted, and intra-articular distal radius fracture with 5 cm of bone loss. He was treated using a staged Masquelet technique with incorporation of ipsilateral proximal tibial autograft with a bone harvester to obtain cancellous autograft and bone marrow graft. The patient initially underwent emergent I and D, acute carpal tunnel release, and internal and external fixation. A 5 cm bone void was filled with antibiotic cement. Four weeks later, the antibiotic cement was removed, cancellous bone graft and marrow were harvested from the proximal tibia, and the graft was placed within the prior bone void. Fracture site healing was confirmed radiographically and with computer-tomography imaging 3 months later. The patient has demonstrated excellent results 1 year post-operative with 60° of wrist flexion, 40° of wrist extension with mild pain, and full finger range of motion with radiographic union. Internal fixation with placement of a volar locking plate remains the mainstay of treatment for distal radial fractures. However, in more comminuted fractures with bone loss, treatment becomes more challenging. We have presented a unique case utilizing a staged Masquelet technique with incorporation of a proximal tibial autograft to educate readers on an alternative option and technique for autograft donor sites in these more complicated fractures."}, {"id": "pubmed23n0260_528", "title": "[Surgical treatment of diaphyseal tibial fractures. Choice of procedure and results of treatment of 187 fractures].", "score": 0.00980392156862745, "content": "From 1988 to 1990 a total of 187 fractures of the tibia (92 compound, 95 closed fractures) were treated with a primary osteosynthesis. In 102 fractures an external fixation was performed, 85 internal fixations were divided into 58 intramedullary nails and 27 plates. In a follow-up study the fracture healing was analysed, 90% of the patients were examined about 18 months after the accident. The mean healing time was between 12 weeks (internal fixation) and 16 weeks (external fixateur). After primary Fixateur externe 54 were treated by secondary internal fixation, intramedullary nailing was the method of choice. Contamination rate of the tibia, taken by an intraoperative wound swab before nailing was 30%. Infection occurred in 3.2%, non union and refractures in 1.5% and 1%. Over all 80% excellent and good results were found after consolidation. 10% fair and 10% bad results including three amputations after III degrees compound fractures and three death after polytrauma."}, {"id": "pubmed23n0916_11638", "title": "A Gustilo Type 3B Open Tibial Fracture Treated with a Proximal Flexor Hallucis Longus Flap: A Case Report.", "score": 0.009708737864077669, "content": "In the treatment of Gustilo Type 3B open tibial fractures, it is important to perform soft tissue reconstruction and bone reconstruction simultaneously. Gastrocnemius muscle and soleus muscle flaps are generally used as rotational flaps for the tibia. The distal third of the tibia can often not be covered with the gastrocnemius muscle and soleus muscle flaps. Treatment distal to the distal third of the tibia is difficult because fewer flap options are available. In the present report, we describe our experience with a Gustilo Type 3B open tibial fracture treated by gastrocnemius muscle and soleus muscle flaps, along with an additional proximally based flexor hallucis longus flap, which is a rare procedure. The participant was a 17-year-old male who injured his left tibia in a motorcycle traffic accident. Physical examination revealed a wound of 13 cm × 7 cm extending from the medial lower leg to the posterior aspect, with extensive skin loss. There was no nerve or vascular injury. The tibia was exposed, with detachment of the periosteum. The radiograph revealed a tibial shaft fracture. The AO/OTA classification was 42-A3.3, and it was classified as a Gustilo-Anderson Type 3B fracture. Gastrocnemius muscle and soleus muscle flaps were lifted in the area of the soft-tissue defect and then, placed over the tibia. Despite this, the distal portion of the tibia remained uncovered. Therefore, a flexor hallucis longus flap was lifted and placed over the distal portion of the tibia. On day 7 after the injury, the external fixation device was removed and the tibial shaft was fixated with two Ender nails (4.5 mm in diameter). The clinical course was satisfactory, and the skin graft and flap were successful. Bone union was achieved without infection, and the resulting range of motion was normal. For the treatment of Gustilo-Anderson Type 3B open tibial fractures, early treatment of the soft-tissue defect is vital. We surgically treated a Gustilo-Anderson Type 3B open tibial fracture with gastrocnemius muscle and soleus muscle flaps, along with an additional proximally based flexor hallucis longus flap, which is a rare procedure. In the event of a soft-tissue defect in the distal third of the tibia, the use of a proximally based flexor hallucis longus flap is an effective surgical approach."}, {"id": "pubmed23n1009_12107", "title": "Prevention of infection in open fractures: Where are the pendulums now?", "score": 0.009708737864077669, "content": "Soft tissue management and fracture fixation including initial external fixation in Gustilo-Anderson type II and type III open fractures are cornerstones in the treatment but details on timing and type of wound closure, irrigation and debridement, systemic and local antibiotics, antimicrobial-coated implants and the use of Bone Morphogenetic Protein-2 remain controversial. This article looks at current clinical evidence of these items for the management of open fractures. Timing of debridement and wound closure remains critical. Early debridement by an experienced team within 24 h seems adequate while gross contamination, a devascularized limb, a multi-injured patient and compartment syndrome require immediate surgical intervention. Wound closure during the first surgery was shown to result in reduced rates for infections and nonunion. If soft-tissue reconstruction is needed, it should be performed within the first 7 days. Regarding types of irrigation fluid, antiseptic and antibacterial solutions did not prove to be superior to saline. High pressure irrigation has not been demonstrated to be beneficial whereas antibiotic administration as soon as possible has been proven to be favorable. Administration of more than 72 h was not superior to shorter systemic antibiotic intervals. For Gustilo-Anderson type I and II, broad spectrum antibiotic therapy is reasonable. Additional aminoglycosides for broader coverage are recommended in Gustilo-Anderson type III fractures. There is newer literature on the beneficial effects of the use of local antibiotics, e.g. by antibiotic beads. Coating of internal fixation devices is a modern approach to improve infection prophylaxis and gentamicin-coated implants have been demonstrated to be safe in clinical application. Vacuum assisted closure (VAC) could not evidence negative pressure wound therapy to reduce infection risk, improve self-rated disability or quality of life in open fractures, however, enhance treatment costs. Recombinant human bone morphogenetic proteins (rhBMP)-2 showed promising data in Gustilo-Anderson type III open tibial shaft fractures with lower rates of invasive secondary procedures. In conclusion, there is evidence for thorough debridement and irrigation with saline, early soft tissue coverage and the use of systemic and local antibiotics. Except for a short-term soft tissue coverage VAC seems not to be beneficial and rhBMP-2 is an additional tool in Gustilo-Anderson type III open fractures."}, {"id": "pubmed23n0735_24439", "title": "Salvage procedures in lower-extremity trauma in a child with hereditary motor and sensory neuropathy type I: a case report.", "score": 0.009615384615384616, "content": "Fractures of the lower extremity are a common type of childhood injury and many can be treated without surgery. Dislocated and open fractures are an indication for fracture stabilization via either intramedullary nailing or, in the case of complicated fractures, external fixation. But if complications are likely because of diseases and disabilities (for example, a neuropathy) that can complicate the post-operative procedure and rehabilitation, what options does one have? We report a nine-year-old Caucasian girl who had hereditary motor and sensory neuropathy type I and who was admitted with a grade I open tibia fracture after a fall from a small height. Plain radiographs showed a dislocated tibia and fibula fracture. An open reduction with internal fixation with a compression plate osteosynthesis was performed, and soft tissue debridement combined with an external fixateur was undertaken. Three months later, she was re-admitted with localized swelling and signs of a local soft tissue infection in the middle of her tibia. Plain radiographs showed a non-union of the tibia fracture, and microbiological analysis confirmed a wound infection with cefuroxime-sensitive Staphylococcus aureus. Because of the non-union, the osteosynthesis was replaced with an Ilizarov external fixateur, and appropriate antibiotic therapy was initiated. Four months after the initial accident, the fracture was consolidated and we removed the external fixateur. If there is a pre-existing neuropathy and if disease makes it difficult for a child to follow all post-operative instructions, salvage procedures should be kept in mind in case of complications. There are multiple therapeutic options, including osteosynthesis, intramedullary nailing systems, cast therapy, or an external fixateur like the Ilizarov or Taylor spatial frame system. The initial use of an external fixateur such as an Ilizarov or Taylor spatial frame in patients with pre-existing neuropathies should be kept in mind as a possible treatment option in complicated fractures, especially in a child with pre-existing neurological or endocrine pathologies."}, {"id": "pubmed23n0728_18192", "title": "Recombinant human BMP-2 for the treatment of open tibial fractures.", "score": 0.009615384615384616, "content": "Recombinant human bone morphogenetic protein-2 (rhBMP-2) improves healing of open tibial fractures treated with intramedullary nail fixation. However, routine use has not occurred. The purposes of the current study were to provide a systematic review of the literature using rhBMP-2 in the treatment of acute open tibial fractures treated with intramedullary nail fixation and to provide a meta-analysis of the randomized, controlled trials. Multiple databases, reference lists of relative articles, and main orthopedic journals were searched. The basic information and major results were compared. Four studies with a total of 609 patients were included.The secondary intervention rate in the standard-of-care (SOC) group was significantly higher than in the rhBMP-2 combined with absorbable collagen sponge (rhBMP-2/ACS) group (27.1% vs 17.5%, respectively; P&lt;.01). The treatment failure rate in the SOC group was significantly higher (34.3% vs. 21.4%, respectively; P&lt;.01). No significant differences were found in infection rate, hardware failure rate, fracture healing rate at 20 weeks, and postoperative pain level. For patients treated with reamed intramedullary nail fixation, only the treatment failure rate in the SOC group was significantly higher (21.5% vs 14.2%, respectively; P=.02); no other significant difference was observed. Adding rhBMP-2 to the treatment of Gustilo-Anderson grade IIIA and B open tibial fractures led to net savings of approximately $6000 per case.Recombinant human bone morphogenetic protein-2 added to intramedullary nail fixation of open tibial fractures could reduce the frequency of secondary interventions and total health care costs. For reamed patients, adding rhBMP-2 reduced treatment failure. This analysis supports the clinical efficacy of rhBMP-2/ACS for the treatment of these severe fractures."}, {"id": "pubmed23n0929_24008", "title": "[Treatment of the postoperative infection of limbs fracture after internal fixation with vacuum sealing drainage (VSD) combined with continual irrigation].", "score": 0.009523809523809525, "content": "To explore the clinical effects of VSD combined with continual irrigation in treating the infection of limbs fracture after internal fixation. From March 2010 to June 2015, 10 patients with infection of limbs fracture after internal fixation were treated with VSD combined with continual irrigation. There were 7 males and 3 females, aged from 11 to 58 years with an average of 34.4 years. Course of disease was from 1 to 8 months with an average of 4.8 months. Postoperative infection occurred in fractures of ulna and radius of 4 cases, tibiofibular fractures of 3 cases, calcaneal fractures of 2 cases, femoral fractures of 1 case. Eight infections were open fracture and 2 infections were close fracture. In additon to above treatment, antibiotics, dressing changing or skingrafting were used in the patients. Informations of wound surface healing, change dressings, original infection focus were observed. All infections got control, the wound healing after change dressings or skingrafting, and no complications such as osteomyelitis were found. The mean treatment time was 38.4 days(ranged, 29 to 45 days) and replacement times was 2.2 times(ranged, 1 to 4 times). All patients were followed up, no recurrent infections were found at 1 year after fracture healing. VSD combined with continual irrigation can effectively decrease the incidence of complications and promote the wound growth, healing and considerably shorten the healing time. It is an effective method for the treatment of infection of limbs fracture after internal fixation."}, {"id": "pubmed23n0986_8054", "title": "Outcome of Locked Compressive Nailing in Aseptic Tibial Diaphyseal Nonunions without Bone Defect.", "score": 0.009433962264150943, "content": "Treatment of tibial diaphyseal nonunions are rather difficult. Plate-screw, intramedullary nailing and external fixation are the methods used for treatment. The aim of this study is to evaluate the treatment results of aseptic diaphyseal nonunions following tibia fractures by intramedullary compressive tibia nailing (IMCN) with or without bone graft. Twenty eight patients who had aseptic tibial nonunion without bone defects operated between 2005 and 2015 were included in the study. The mean age of our patients was 36.4 years (range 20-56 years). There were 22 males and 6 females. Fifteen of the patients exhibited hypertrophic nonunion and thirteen exhibited atrophic nonunion. The average time between fracture occurrence and presentation to our department was 1.6 years (range 1-20 years). All patients underwent fibular osteotomy by removal of a 2 cm bone block from the middle one-third of the fibulas. In all cases, IMCN was applied following the reaming procedure, then maximum bone contacts were achieved manually between proximal and distal bone fragments afterward, and dynamic compressive fixation with 1 mm of compression was performed by a single rotation of the compression screw at the top of the nail. Direct X-ray images were assessed according to the Rust criteria, and functional outcomes were assessed according to the Johner-Wrush criteria. Finite-element analysis was performed for 1 mm of compression. For statistical analysis, Fisher's exact test, Pearson's Chi-square test, and Mann-Whitney U-test were used. Union was achieved in all patients. Radiological union was obtained at an average of 15.5 ± 1.86 weeks. Functional results were found to be good or excellent in 25 (89.2%) patients and average or poor in 3 (10.8%) patients. One patient developed skin necrosis at the wound site, which was treated with rotational flap and skin graft. None of the patients developed implant failure, thromboembolism, deep-vein thrombosis, or infection. The use of compressive intramedullary nailing with or without bone graft is an effective method for the treatment of tibial nonunion."}, {"id": "pubmed23n0024_8786", "title": "[Plate fixation and open wound treatment in infected intramedullar nails].", "score": 0.009433962264150943, "content": "In treating patients who present with an extensive non-union due to infected intramedullary nail, bone healing can be achieved within a few weeks through a policy of active management. Following excision of the infected tissue and thorough irrigation, the fracture site is stabilized by a plate fixation and a packet with cancellous bone. The wound overlying the bone is left open to granulate. No complications were observed during the course of healing in 7 cases (5 tibia, 2 femur) managed this way. The method is based on classical principles of orthopedics and the treatment of infection. Such has been its impressive success, that it would seem to warrant not only close attention but also strong recommendation."}, {"id": "pubmed23n0779_20957", "title": "Treatment of open tibial fracture with bone defect caused by high velocity missiles: a case report.", "score": 0.009345794392523364, "content": "Tibia fracture caused by high velocity missiles is mostly comminuted and followed by bone defect which makes their healing process extremely difficult and prone to numerous complications. A 34-year-old male was wounded at close range by a semi-automatic gun missile. He was wounded in the distal area of the left tibia and suffered a massive defect of the bone and soft tissue. After the primary treatment of the wound, the fracture was stabilized with an external fixator type Mitkovic, with convergent orientation of the pins. The wound in the medial region of the tibia was closed with the secondary stitch, whereas the wound in the lateral area was closed with the skin transplant after Thiersch. Due to massive bone defect in the area of the rifle-missile wound six months after injury, a medical team placed a reconstructive external skeletal fixator type Mitkovic and performed corticotomy in the proximal metaphyseal area of the tibia. By the method of bone transport (distractive osteogenesis), the bone defect of the tibia was replaced. After the fracture healing seven months from the secondary surgery, the fixator was removed and the patient was referred to physical therapy. Surgical treatment of wounds, external fixation, performing necessary debridement, adequate antibiotic treatment and soft and bone tissue reconstruction are essential in achieving good results in patients with the open tibial fracture with bone defect caused by high velocity missiles. Reconstruction of bone defect can be successfully treated by reconstructive external fixator Mitkovic."}, {"id": "pubmed23n1062_15982", "title": "Treatment options for aseptic tibial diaphyseal nonunion: A review of selected studies.", "score": 0.009345794392523364, "content": "In aseptic tibial diaphyseal nonunions after failed conservative treatment, the recommended treatment is a reamed intramedullary (IM) nail.Typically, when an aseptic tibial nonunion previously treated with an IM nail is found, it is advisable to change the previous IM nail for a larger diameter reamed and locked IM nail (the rate of success of renailing is around 90%).A second change after an IM nail failure is also a good option, especially if bone healing has progressed after the first change.Fibular osteotomy is not routinely advised; it is only recommended when it interferes with the nonunion site.In delayed unions before 24 weeks, IM nail dynamization can be performed as a less invasive option before deciding on a nail change.If there is a bone defect, a bone graft must be recommended, with the gold standard being the autologous iliac crest bone graft (AICBG).A reamer-irrigator-aspirator (RIA) system might also obtain a bone autograft that is comparable to AICBG.Although the size of the bone defect suitable to perform bone transport techniques is a controversial issue, we believe that such techniques can be considered in bone defects &gt; 3 cm.Non-invasive therapies and biologic therapies could be applied in isolation for patients with high surgical risk, or could be used as adjuvants to the aforementioned surgical treatments. Cite this article: <iEFORT Open Rev</i 2020;5:835-844. DOI: 10.1302/2058-5241.5.190077."}, {"id": "pubmed23n1086_21287", "title": "Elastic Stable Intramedullary Nailing of Pediatric Tibial Fractures.", "score": 0.009174311926605505, "content": "Most pediatric tibial shaft fractures (75%)<sup1</sup can be treated nonoperatively; however, unstable and open fractures require surgical intervention. Titanium elastic nails have become a popular technique for fixation of pediatric tibial shaft fractures. They act as internal splints that impart relative stability to the fracture, promoting callus formation at the fracture site<sup2</sup. After the patient is placed in the supine position, the proximal tibial physis is marked using fluoroscopy. An anteromedial and anterolateral incision are made distal to the physis. Entry holes are created in the proximal part of the tibia, and appropriately sized titanium nails are introduced into the bone. Nail size should be 40% of the width of the canal, yielding 80% canal fill when 2 nails are used. The nails are prebent into a gentle C-shape to increase cortical contact at the apex so that 3-point fixation is achieved. The nails are passed to the fracture site, and the fracture is then reduced. The nails are then passed across the fracture site and stopped proximal to the distal tibial physis. The nails are then cut and tamped distally until there is just a short portion of nail left out of the proximal part of the tibia so that the nails can be removed once the fracture is healed. The wounds are then closed, and postoperative immobilization is applied. Many pediatric tibial shaft fractures can be treated with closed reduction and cast immobilization. Open fractures, or fractures that fail nonoperative management, can be treated with external fixation, open reduction and internal fixation (ORIF), or intramedullary stabilization<sup3</sup. Anatomic reduction and fracture compression can be achieved with ORIF; however, a drawback to this technique is the lack of soft-tissue coverage in the diaphyseal area of the tibia, which can lead to infection and wound-healing problems<sup4</sup. External fixation has traditionally been the technique of choice for open tibial fractures; however, with the ability to use flexible tibial nails in both open and closed tibial fractures, external fixation is now reserved for open fractures with large soft-tissue defects or in fractures with segmental bone loss. Intramedullary flexible nailing can be used in both open and closed tibial fractures, provides excellent fracture fixation, and utilizes incisions that are more cosmetically appealing to patients<sup5,6</sup. Outcomes following flexible nailing for pediatric tibial fractures are excellent. In a study of 19 patients undergoing flexible nailing for tibial shaft fractures, 18 had excellent or satisfactory results<sup7</sup. Compared with patients who had external fixation, those treated with flexible nails had less pain, shorter time to union, and better functional outcomes<sup2</sup. Compared with patients treated with ORIF, those who underwent flexible intramedullary nailing spent less time in the operating room and had lower rates of wound complications<sup4</sup. In the immediate postoperative period, clinicians should be aware of the risk of compartment syndrome, particularly in patients with high-energy injuries, older patients (&gt;14 years old), and heavier patients (&gt;50 kg)<sup8</sup. There is also an increased risk of soft-tissue irritation and fracture malunion in heavier patients treated with flexible nails<sup9,10</sup. Nail size should be 80% of the canal diameter (e.g., two 4.0-mm nails should be chosen for a canal that measures 10 mm).Nails should be properly contoured to avoid corticotomy of the far cortex during insertion; apex of the bend should be positioned at the level of the fracture.During insertion, leave room to advance nails further after they are cut proximally.Do not bury the proximal nail tips beneath the cortex as extraction will be difficult.Ensure that the ends of the nails are not lying up against the proximal tibial physis as this may cause premature growth arrest."}, {"id": "pubmed23n1107_21414", "title": "Uncoated vs. Antibiotic-Coated Tibia Nail in Open Diaphyseal Tibial Fracture (42 according to AO Classification): A Single Center Experience.", "score": 0.009174311926605505, "content": "Implant-associated infections remain one of the main problems in the treatment of open tibia fractures. The role of systemic antibiotic prophylaxis is now agreed and accepted; nevertheless, recent literature also seems to emphasize the importance of local antibiotic therapy at the fracture site. Several therapeutic strategies have been proposed to overcome this new need. Antibiotic-coated nails play crucial role in this, allowing both infection prevention and favoring the fracture stabilization. We describe the outcome of patients with open diaphyseal tibia fracture treated either with a standard uncoated nail or a gentamicin-coated nail from January 2016 to December 2018 at our second level emergency-urgency department. Primary outcomes were infection rate and bone union rate. Other outcomes reported are reoperation rate, time between injury and nailing, and safety of antibiotic nail. Numerical variables were tabulated using mean, standard deviation, minimum, maximum, and number of observations. Categorical variables were tabulated using number of observations. 23 patients treated with uncoated nail and 23 patients treated with antibiotic-coated tibia nail were included in the study and were evaluated for a minimum follow-up of 18 months. Among the 46 patients, 9 were Gustilo-Anderson type I, 21 type II, and 16 type III open fracture. Regarding the bone healing rate at 12 months, 16 fractures in the first group and 18 in the second were completely healed. 4 infections were found in the first group (3 superficial surgical site infection and 1 osteomyelitis) and 3 superficial infections in the second one. No adverse events have been recorded with antibiotic-coated nails. In this unicentric retrospective study observed no deep wound infections and good fracture healing in the use of antibiotic-coated nails. Antibiotic nails have been shown to play a role in the treatment of fractures in critically ill patients with severe soft tissue damage."}, {"id": "wiki20220301en142_30273", "title": "Open fracture", "score": 0.00911961444181312, "content": "An open fracture, also called a compound fracture, is a type of bone fracture in orthopedics that is frequently caused by high energy trauma. It is a bone fracture associated with a break in the skin continuity which can cause complications such as infection, malunion, and nonunion. Gustilo open fracture classification is the most commonly used method to classify open fractures, to guide treatment and to predict clinical outcomes. Advanced trauma life support is the first line of action in dealing with open fractures and to rule out other life-threatening condition in cases of trauma. Cephalosporins are generally the first line of antibiotics. The antibiotics are continued for 24 hours to minimize the risk of infections. Therapeutic irrigation, wound debridement, early wound closure and bone fixation are the main management of open fractures. All these actions aimed to reduce the risk of infections. Causes"}, {"id": "pubmed23n1103_8483", "title": "Extraction of Broken Tibial Interlock Nail with a Retrograde Hooked Guide Wire: A Novel Surgical Technique.", "score": 0.00909090909090909, "content": "Removal of a distal piece of a broken nail often possesses a technical challenge. Several methods have been described in the past to extract a distal piece by using specialized instruments like such as hooks, olive wires, and talwalkar radial square nail etc. It is difficult to extract a distal piece from a proximal incision site and often fracture or the nonunion site has to be opened. In this article, we describe a novel technique to extract a distal piece of broken intramedullary tibia nail by retrograde manner using a guide wire with a \"'U\"' shaped bend at its distal end to hook the tip of a distal piece of broken nail and help in extraction. A 43- year-s old male presented with complain of pain in left leg since 3 months. Patient had sustained left- sided compound Grade 2 tibia shaft fracture in a road traffic accident 4 years back. He was operated with tibia interlock nail followed by skin grafting for wound coverage in a different facility. On clinical examination: There was tenderness around distal tibia, no swelling, no coronal or sagittal plane fracture mobility, and no crepitus or loss of transmitted movements which suggested fracture union clinically. Radiographs confirmed complete union of tibia shaft fracture with hypertrophic nonunion of distal fibula with broken intramedullary nail IMN at the level of proximal most screw hole of distal locking holes with both distal locking screws broken. As fracture was united, we planned for removal of broken nail without opening fracture site. For extraction for distal tibial broken nail part, we used this new Retrograde Hooked Guide Wire technique. It is a simple, cost effective, minimally invasive procedure with minimal blood loss and decrease time of surgery that can be used before attempting more invasive extraction methods and hence should be included in standard procedures for extraction."}, {"id": "pubmed23n1126_75", "title": "Antibiotic artificial bone implantation and external fixation for the treatment of infection after intramedullary nail fixation: a retrospective study of 33 cases.", "score": 0.00909090909090909, "content": "To explore the clinical effect of antibiotic artificial bone implantation and external fixation in the treatment of infection after intramedullary nail fixation. We retrospectively reviewed the clinical data of patients with infection after intramedullary nail fixation treated from March 2010 to August 2020. There were 27 males and 6 female, aged from 12 to 67 years (average 42.27 years), 18 cases on the left side and 15 cases on the right side. Among them, 20 cases were open fractures with initial injury and 13 cases were closed fractures. All patients were treated with intramedullary nail removal, local debridement, antibiotic artificial bone implantation and external fixation. Because of bone defects, 19 patients underwent secondary autologous cancellous bone grafting after infection control. Postoperative wound healing, related inflammatory indicators, fixation time, and bone healing time were recorded and followed up. The 33 patients were followed up with period of 10 ~ 98 months (average 62.7 months). One patients failed to control the infection effectively after treatment, so received antibiotics artificial bone implantation again. Two patients also received antibiotic artificial bone implants again due to the recurrence of the infection. After treatment, infection was controlled and the fracture healed well. One patient received vacuum sealing drainage (VSD) due to persistent postoperative exudation, and five patients were also cured successfully after continuous dressing. Two patients had sinus tract after surgery, and the wound was cured by continuous dressing change. Nineteen patients received autogenous iliac bone grafts for healing due to bone defects ranging from 3 to 6.5 cm (average 4.15 cm) after infection control. The external fixation time of 33 patients ranged from 4 to 16 months (average 7.79 months), the bone healing time ranged from 4 to 13 months (average 6.67 months), and the related inflammatory indexes returned to normal within 2-8 weeks (average 4.48 weeks). Antibiotic artificial bone implantation and external fixation is an effective method for the treatment of infection after intramedullary nail fixation."}, {"id": "pubmed23n0750_21548", "title": "Distraction over nail using circular external fixation for septic pseudarthrosis of the tibia.", "score": 0.009009009009009009, "content": "We present a report of nine patients (eight women and one man; mean age 37 years) from 2010 to 2012 with septic pseudarthrosis of the tibia treated with bone transport over an intramedullary nail using a circular external fixator. The mean follow-up was 15 months (range: 10-21 months). A two stage approach was used. At the first stage, removal of the primary osteosynthesis and extensive bone debridement to healthy, bleeding bone margins was performed. The bone defect was packed with antibiotic loaded cement beads, and stabilization of the tibia was done with a unilateral external fixator or with a long leg posterior splint. The mean size of bone defect was 4 cm (range: 3.5-5.5 cm). At the second stage, two consecutive negative wound cultures and normal values of blood cell count, C-reactive protein (CRP), and estimated sedimentation rate (ESR) were obtained. Then we reamed and locked the intramedullary nailing of the tibia, applied a circular external fixator, and performed percutaneous corticotomy of the tibia opposite the site of the bone defect. Bone distraction over the nail was initiated at the eighth postoperative day at a rate of 1 mm/day. At the last follow-up, union was achieved in all cases without recurrence of bone infection. All patients experienced excellent (n=3) or good (n=6) knee and ankle function, as well as complete return to their daily activities. Two patients experienced pin-tract infection, and one patient experienced anterior knee pain at the entry point of the nail."}, {"id": "pubmed23n0204_6046", "title": "[Treatment of uninfected pseudarthroses of the lower extremity near the joint using the Küntscher medullary nail].", "score": 0.009009009009009009, "content": "The Küntscher nail has proved successful in the treatment of non-unions of the median third of the femur and lower leg after opening of the medullary cavity; in fact, this method is described in literature as the method of choice. It is based on the assumption that a sufficiently dimensioned intramedullary Küntscher nail together with the biological stimulative action of the bone meal, will result in a rapid osseous bridging of the non-union. This method has been successfully used in 128 cases even in non-unions which were close to the joint. Healing occurred in 92% of the cases. In about 11 cases it became necessary to perform a secondary operation. The high rate of infection, amounting to 12.4%, is relatively low compared with the very high rate of infective relapses following the first operation to achieve healing of the bone fractures."}, {"id": "article-30176_27", "title": "Tibia Nonunion -- Treatment / Management -- The following are details of operative treatment options and adjuncts reported in the literature:", "score": 0.009003746525399831, "content": "Locking compression plates: It is indicated for managing nonunion of the metaphyseal diaphyseal junction fractures, where the outcomes of exchange nailing would be doubtful. The procedure is performed along with bone grafting and without the removal of the underlying nail by gaining unicortical purchase fixation with locking head screws. It has been shown to have predictable good results [32] [33] [32] . External fixation : Considered in complex nonunions (e.g., when internal fixation is not possible or not recommended due to infection, substantial deformity, and/or bone loss [12] [34] [12] ."}, {"id": "pubmed23n1028_14062", "title": "Treatment of fracture-related infection of the lower extremity with antibiotic-eluting ceramic bone substitutes: case series of 35 patients and literature review.", "score": 0.008928571428571428, "content": "The current treatment concepts of fracture-related infection (FRI) [Consensus Conference (Anti-Infection Task Force (AITF)) on the definition of acute or chronic osteomyelitis (cOM)] are associated with unsolved challenges and problems, underlining the need for ongoing medical research. Literature review of treatments for FRI and description of own cases. We could include eight papers with 394 patients reporting treatments and outcome in FRI. The infection was resolved in 92.9% (mean) of all treatments. The mean follow-up was 25 months with a persistent non-union in 7% of the patients. We diagnosed 35 (19f/16m; 56.4 ± 18.6 years) patients with bone infections anatomically allocated to the proximal and distal femur (12×), the pelvis (2×), distal tibia (3×), tibial diaphysis (11×), the ankle joint (4×) and calcaneus (3×). These 35 patients were treated (1) with surgical debridement; (2) with antibiotic-eluting ceramic bone substitutes; (3) bone stabilization (including nail fixation, arthrodesis nails, plates, or external ring fixation), (4) optionally negative pressure wound therapy (NPWT) and (5) optionally soft tissue closure with local or free flaps. The mean follow-up time was 14.9 ± 10.6 months (min/max: 2/40 month). The overall recurrence rate is low (8.5%, 3/35). Prolonged wound secretion was observed in six cases (17.1%, 6/35). The overall number of surgeries was a median of 2.5. The results in the literature and in our case series are explicitly promising regarding the treatment of posttraumatic fracture-related infection."}, {"id": "pubmed23n0690_21465", "title": "Recombinant human bone morphogenetic protein-2: a randomized trial in open tibial fractures treated with reamed nail fixation.", "score": 0.008928571428571428, "content": "Recombinant human bone morphogenetic protein-2 (rhBMP-2) improves healing of open tibial fractures treated with unreamed intramedullary nail fixation. We evaluated the use of rhBMP-2 in the treatment of acute open tibial fractures treated with reamed intramedullary nail fixation. Patients were randomly assigned (1:1) to receive the standard of care consisting of intramedullary nail fixation and routine soft-tissue management (the SOC group) or the standard of care plus an absorbable collagen sponge implant containing 1.5 mg/mL of rhBMP-2 (total, 12.0 mg) (the rhBMP-2/ACS group). Randomization was stratified by fracture severity. The absorbable collagen sponge was placed over the fracture at wound closure. The primary efficacy end point was the proportion of subjects with a healed fracture as demonstrated by radiographic and clinical assessment thirteen and twenty weeks after definitive wound closure. Two hundred and seventy-seven patients were randomized and were the subjects of the intent-to-treat analysis. Thirteen percent of the fractures were Gustilo-Anderson Type IIIB. The proportions of patients with fracture-healing were 60% and 48% at week 13 (p = 0.0541) and 68% and 67% at week 20 in the rhBMP-2/ACS and SOC groups, respectively. Twelve percent of the subjects underwent secondary procedures in each group; more invasive procedures (e.g., exchange nailing) accounted for 30% of the procedures in the rhBMP-2/ACS group and 57% in the SOC group (p = 0.1271). Infection was seen in twenty-seven (19%) of the patients in the rhBMP-2/ACS group and fifteen (11%) in the SOC group (p = 0.0645; difference in infection risk = 0.09 [95% confidence interval, 0.0 to 0.17]). The adverse event incidence was otherwise similar between the treatment groups. The healing of open tibial fractures treated with reamed intramedullary nail fixation was not significantly accelerated by the addition of an absorbable collagen sponge containing rhBMP-2."}, {"id": "pubmed23n0948_3776", "title": "[Effectiveness analysis of induced membrane technique in the treatment of infectious bone defect].", "score": 0.008849557522123894, "content": "To evaluate the effectiveness of induced membrane technique in the treatment of infectious bone defect. Thirty-six patients (37 bone lesions) with infectious bone defects were treated with induced membrane technique between January 2011 and June 2014. There were 28 males and 8 females with an average age of 36 years (range, 20-68 years). All bone defects were post-traumatic infectious bone defect. The bone defect was located at the tibia and fibula in 24 cases (25 bone lesions), at femurs in 6 cases (6 bone lesions), at ulnas and radii in 2 cases (2 bone lesions), at calcanei in 3 cases (3 bone lesions), and at clavicle in 1 case (1 bone lesion). The average time between onset and the treatment of induced membrane technique was 6.2 months (range, 0.5-36.0 months); 15 patients were acute infections (disease duration was less than 3 months). At the first stage, after the removal of internal fixator (applicable for the patients who had internal fixation), complete debridement of infection necrotic bone tissue and surrounding soft tissue was performed and the bone defects were filled with antibiotic-impregnated cement spacers. If the bone was unstable after debridement, external fixator or plaster could be used for stabilization. Patients received sensitive antibiotics postoperatively. At the second stage (usually 6-8 weeks later), the cement spacer were removed, with preservation of the induced membrane formed by the spacer, and filled the bone defect with autologous iliac bone graft within the membrane. The hospitalization time after debridement was 17-30 days (mean, 22.2 days), and the hospitalization time after the second stage was 7-14 days (mean, 10 days). All the flaps healed uneventfully in 16 cases treated with local flap transposition or free flap grafting after debridement. One patient of femur fracture received Ilizarov treatment after recurrence of infection at 11 months after operation; 1 patient of distal femoral fracture received amputation after recurrence of infection at 1 month after operation; 1 patient of distal end of tibia and fibula fractures received ankle arthrodesis after repeated debridements due to the recurrence of infection; 1 patient of tibia and fibula fractures lost to follow-up. The other 32 patients (33 bone lesions) were followed up 1-5 years (mean, 2 years) without infection recurrence, and the infection control rate was 91.7% (33/36). All the patients had bony union, and the healing time was 4-12 months (mean, 7.5 months); no refracture occurred. One patient of femur bone defect had a lateral angulation of 15° and leg discrepancy of 1.5 cm. Superficial pin infection was observed in 7 cases and healed after intensive wound care and oral antibiotics. Adjacent joint function restriction were observed in 6 cases at last follow-up. Induced membrane technique is a simple and reliable technique for the treatment of infectious bone defect. The technique is not limited to the size of the bone defect and the effectiveness is satisfactory."}, {"id": "pubmed23n0399_10884", "title": "Ender nailing versus external fixation in the stabilization of type III open tibial shaft fractures.", "score": 0.008849557522123894, "content": "Management of severe open tibial shaft fractures presents a difficult challenge to the orthopaedic surgeon. They are frequently associated with loss of limb, infection and high levels of morbidity. All the authors considered, now, that there are five keys to successful treatment: antibiotic therapy, radical debridement and pulsed lavage irrigation, stabilization of fracture with minimal further devascularization, early soft tissue coverage and early bone-grafting. It rests, also, a number of controversies in the management of open tibial fractures, not least of which is the method of fracture stabilization: the choice between intramedullary nailing and external skeletal fixation, the use of reamed or unreamed nails. Fifty-seven patients with 62 open fractures of the tibial shaft type IIIA, B and C (Gustilo) were treated between 1.01.1994 and 31.12.1998 in the Department of Orthopaedic Surgery of the Emergency Hospital, Iasi, Romania. There were 29 cases type IIIA fractures, 26 cases type IIIB and 7 cases type IIIC (Mess score showed the viability of the limb). Our patients were 36 males and 21 females; their mean age was 36 years (range 17 to 70). Forty-six patients were injured in road traffic accidents and 11 in other traumatic incidents. In 33 cases the skeletal stabilization was achieved by Ender nailing under general or regional anesthesia. In 15 cases we used a bilateral uniplanar external fixator (Burghele) and 14 fractures were stabilized with an Ilizarov external fixator. We note a secondary amputation after the failure of the revascularization of the limb in a type IIIC fracture. All fractures united; the mean time to union was 30.2 weeks--in the external fixation group and 26.4 weeks--in the Ender nailing group. Malunions occurred slightly more frequently in the external fixation group that in the Ender nailing group (15.7% versus 5.8%). We noted--also--more secondary procedures required in the external fixation group. The infection rate was 3 of 33 (9.1%) in the Ender nailing group compared to 4 deep (13.8%) and 8 pin-tract infection (27.6%) in the external fixation group. Our study suggests that Ender nailing has several advantages over external fixation in the management of severe open tibial shafts fractures. Based on these results, over the last years, in our Department we use mainly the Ender nailing technique, as we consider it a better approach for these type of lesions."}, {"id": "pubmed23n0871_7041", "title": "Complex Compound Fracture of Tibia Managed with Distraction Osteogenesis.", "score": 0.008771929824561403, "content": "The treatment of tibia bone loss can be challenging. The surgical options for the treatment of bone loss include bone transport, vascularized fibula graft, and induced membrane. We present a case of complex compound fracture of tibia with bone loss. Interestingly patient sustained this injury in spite of having intramedullary nail in tibia which was inserted to stabilize previous fracture 9 months prior to trauma. The proximal half of the nail was protruding out of the wound at the time of presentation in emergency department. The nail was removed and stabilized with external fixator after wound closure. The bone gap and nonunion at fracture site was managed with Ilizarov fixator. At the end of treatment patient got satisfactory functional outcome. Ilizarov method is a biologic and comprehensive method for management of bone loss, non union and limb length discrepancy."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 315]], "word_ranges": [[0, 41]], "text": "Acute urticaria: characterized by erythematous-edematous, evanescent, pruritic, evanescent lesions, lasting less than 24 hours, without desquamation. The general condition is usually preserved. In children the annular pattern is more frequent. In both rubella and toxicoderma, the general condition is not preserved."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Acute urticaria: characterized by erythematous-edematous, evanescent, pruritic, evanescent lesions, lasting less than 24 hours, without desquamation. The general condition is usually preserved. In children the annular pattern is more frequent. In both rubella and toxicoderma, the general condition is not preserved. In staphylococcal shock, the general condition is affected and the lesions are blistering. Scabies lesions are preferably interdigital, in the form of papulocoses that can follow linear trajectories.", "full_answer_no_ref": "Acute urticaria: characterized by erythematous-edematous, evanescent, pruritic, evanescent lesions, lasting less than 24 hours, without desquamation. The general condition is usually preserved. In children the annular pattern is more frequent. In both rubella and toxicoderma, the general condition is not preserved. In staphylococcal shock, the general condition is affected and the lesions are blistering. Scabies lesions are preferably interdigital, in the form of papulocoses that can follow linear trajectories.", "full_question": "A 14-year-old female patient in good general condition presents since 4 days ago a very pruritic generalized cutaneous eruption formed by erythematous-edematous plaques between 2 and 15 cm in diameter without desquamation with a tendency to acquire an annular morphology that individually disappear in less than 24 hours. The mucous membranes are respected. Your first diagnostic impression would be:", "id": 17, "lang": "en", "options": {"1": "Urticaria.", "2": "Rubella.", "3": "Toxicoderma.", "4": "Staphylococcal toxic shock.", "5": "Scabies."}, "question_id_specific": 137, "type": "DERMATOLOGY", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en111_27496", "title": "Pemphigoid", "score": 0.01803107139982291, "content": "Presentation Primary lesions of small and large blisters, known as vesicles and bullae, are found on the skin and sometimes on the mucous membranes. Non-bullous pemphigoid In some patients, pemphigoid starts off with cutaneous manifestations of BP without bullae, as the only sign of the disease. Pruritic eczematous, papular, or urticaria-like skin lesions may also persist for weeks to months. Bullous phase The bullous stage of BP shows vesicles and bulla, appearing on apparently normal or erythematous skin, predominantly at the flexural aspects of the extremities and the lower trunk. Mucosal lesions, which typically are erosions of the oral mucosa, are present in 10 to 30 percent of patients. Occasionally, the blister fluid becomes blood-tinged. The blisters are tense, about 1–4 cm in diameter, leaving eroded and crusted areas, together with urticarial and infiltrated papules and plaques in an annular or figurate pattern."}, {"id": "wiki20220301en111_27518", "title": "Pemphigoid", "score": 0.0132641291810842, "content": "Other mucous membranes Less common sites that might get involved are nasopharynx, esophagus, and urethra. Nasopharyngeal involvement can lead to ulcerations of the septum and airway obstruction which might require tracheostomy. Esophageal disease may present with ulcerations, dysphagia, odynophagia, and stenosis. Stenosis at urethra, vaginal orifice and rectal have also resulted from chronic inflammation and scarring. Skin disease About 25% of patients have cutaneous lesions, with tense vesicles or bullae, mainly on the face, neck, and scalp. Healing of erosion is either with or without atrophic scars. Cutaneous lesions of mucous membrane pemphigoid presents in 2 subtypes: (1)presents as generalized eruption of tense bullae without scarring (2) presents as localised blisters on an erythematous base, resulting in atrophic scarring."}, {"id": "wiki20220301en111_27524", "title": "Pemphigoid", "score": 0.011586551819949987, "content": "Clinical assessment Presence of tense blisters and erosions that occur on skin without another identifiable cause. Desquamative gingivitis or mucositis involving oral, ocular, nasal, genital, anal, pharyngeal, laryngeal, and/or esophageal mucosae Presence of pruritic eczematous eruptions, or urticarial plaques without identifiable cause. Patient's age over 60 years old. Histopathology Lesional tissue, preferably of an intact vesicle or the edge of an intact bulla is obtained using punch biopsy for Haemotoxylin and Eosin (H&E) staining. The findings are sub-epidermal blister with dermal infiltrated with lymphocytes, neutrophils and eosinophils. Additional findings include sub-epidermal fibrosis which is consistent with the scarring nature of mucous membrane pemphigoid in older lesions and plasma cell infiltration."}, {"id": "article-135196_27", "title": "Toxic Epidermal Necrolysis -- Differential Diagnosis", "score": 0.010975740661227994, "content": "Other drug rashes Toxic shock syndrome(usually present with early multiorgan failures and unique cutaneous manifestations in the form of macular rashes affecting palm and soles that later on evolve to desquamation over the period of 14 days) Paraneoplastic pemphigus(as a mucocutaneous manifestation of malignancy) Exfoliative erythroderma(usually affects skin only and spares mucous membrane and is painless in most of the cases) Staphylococcal scalded skin syndrome in children: (evidence of staphylococcal infection, no history of drug intake, and it usually spares mucus membranes) Skin biopsy is also an important tool to differentiate TEN and SJS from these disorders."}, {"id": "wiki20220301en054_53528", "title": "Staphylococcal scalded skin syndrome", "score": 0.010498917299153525, "content": "Staphylococcal scalded skin syndrome (SSSS) is a dermatological condition caused by Staphylococcus aureus. Signs and symptoms The disease presents with the widespread formation of fluid-filled blisters that are thin walled and easily ruptured, and the patient can be positive for Nikolsky's sign. Ritter's disease of the newborn is the most severe form of SSSS, with similar signs and symptoms. SSSS often includes a widespread painful erythroderma, often involving the face, diaper, and other intertriginous areas. Extensive areas of desquamation might be present. Perioral crusting and fissuring are seen early in the course. Unlike toxic epidermal necrolysis, SSSS spares the mucous membranes. It is most common in children under 6 years, but can be seen in adults who are immunosuppressed or have kidney failure."}, {"id": "wiki20220301en255_30586", "title": "Erythema multiforme minor", "score": 0.009900990099009901, "content": "Erythema multiforme is usually a reaction of the skin and mucous membranes that occurs suddenly. It appears as a symmetrical rash and may include the mucous membrane lesions. This means that the body is sensitive to something that causes the skin and mucous membranes to react. The more common mild form is refer to as EM minor. It consists of a skin rash that involve no more than one mucosal surface. The sudden onset will progress rapidly as symmetrical lesions with circular color changes in some or all of the lesions. Rash will spread towards center or trunk of the body. Evenly distributed bumps on the skin become classic iris or target lesions. They have bright red borders and small white bumps in the center."}, {"id": "wiki20220301en027_33861", "title": "Skin condition", "score": 0.009834190966266438, "content": "The physical examination of the skin and its appendages, as well as the mucous membranes, forms the cornerstone of an accurate diagnosis of cutaneous conditions. Most of these conditions present with cutaneous surface changes termed \"lesions,\" which have more or less distinct characteristics. Often proper examination will lead the physician to obtain appropriate historical information and/or laboratory tests that are able to confirm the diagnosis. Upon examination, the important clinical observations are the (1) morphology, (2) configuration, and (3) distribution of the lesion(s). With regard to morphology, the initial lesion that characterizes a condition is known as the \"primary lesion\", and identification of such a lesions is the most important aspect of the cutaneous examination. Over time, these primary lesions may continue to develop or be modified by regression or trauma, producing \"secondary lesions\". However, with that being stated, the lack of standardization of basic"}, {"id": "pubmed23n0515_10499", "title": "[Mucous membrane involvement in the course of autoimmune blistering skin diseases and drug eruptions. Differential diagnosis].", "score": 0.00980392156862745, "content": "In the following paper the most common drug eruptions and auto-immune, blistering skin diseases in which mucous lesions precede skin lesions development are discussed. Non-characteristic clinical pictures of mucous lesions result in diagnostic difficulties of these diseases, and prognosis is dependent on prompt and precise diagnosis. The aim of the work was to describe clinical features of mucous membranes lesions and point to differential diagnosis."}, {"id": "pubmed23n0345_17473", "title": "[Cutaneous loxoscelism with edematous predominance].", "score": 0.009708737864077669, "content": "Loxoscelism is the clinical condition produced by the venom of spiders belonging to the genus Loxosceles. Human cases of loxoscelism have been observed in diverse countries of different continents in temperate and tropical regions. In Chile loxoscelism is caused by Loxosceles laeta, spider with domestic habits. Loxoscelism can be observed into two well definited clinical variants: cutaneous loxoscelism (CL) and systemic or viscerocutaneous loxoscelism (VCL) which occur in around 83.3 and 16.7% cases respectively. Within the universe of CL patients a clinical modality in which necrotic lesion is not present or is insignificant, but presenting a remarkable edema, particularly when the bite is on the face, which has received the name of CL with an edematous predominance (CLEP). In this paper the individual description and the assambled analysis of 10 cases, four males and six females, age ranging from 6 to 68 years, of CLEP are presented. Nine cases occurred in warm periods spring through fall and one in winter. In six cases the accident causing spider was seen and two of these were identified as L. laeta adult females. In all cases the patients went or were transported to emergency medical services 4-24 h after the bite. The predominant initial symptom was a burning stinging sensation at the site of the bite, followed by intensive pain which expanded the neighbour areas concomitantly with the emerging and progressive edema. In four of the nine patients in who the bite was on the face, the edema involved all of it, closed both eyelids and expanded to the neck and upper part of the thorax. In three cases the enormous edema was the only significant clinical manifestation, whereas in the remaining seven conjunctly with the edema, a small violaceous plaque or a blister of serous content gave place to a little livedoid plaque (diameter 0.3-0.8 cm) which evolved to desquamation without leaving any scarring. The edema was characterized by its brilliant rose color, painful and hard which is not accompanied by regional adenopathy. Treatment of the 10 patients depended on the moment in they were seen by us. It consisted on parenteral administration, according to age and weight, of 5-10 mg of chloroprofenpyridamine maleate every 8 hours for be continued every 12-24 hours until the patient was discharged. Parenteral route was preferred in order that it was going to be adequately absorbed. With the beginning of the antihistaminic treatment a clear diminution of pain and edema was obtained, being possible its total disappearance within 4-10 days. CLEP occurs in about 4% of loxoscelism cases, has a benign prognosis and an early response to adequate medical treatment. Without discarding the sensibility factor of the affected individual, there exist the impression that the edema may abort the necrotic process when it dilutes the enzymatic process produced by L.laeta venom. In Chile, the differential diagnosis must be planted with the following clinical entities: bites of hematophagous insects on the face, bee stings, Chagas' disease with facial port of entry and angioneurotic edema."}, {"id": "article-21300_13", "title": "Erythema Multiforme -- History and Physical", "score": 0.009708737864077669, "content": "Mucosal lesions are common, mostly in the mouth, but also in the genital and ocular mucous membranes. They are initially bullous, then quickly turn into painful erosions. Thick hemorrhagic crusts may cover the labial lesions, and a fibrin-whitish coating may line the mucosal erosions of the cheeks, palate, and genitalia. These mucosal lesions occur most often at the same time as the skin lesions but can be shifted a few days before or after the eruption of targets. While skin lesions are nonpainful, mucosal lesions are frequently painful. Pulmonary signs may also be present, such as a cough and dyspnea. They testify to a respiratory attack most often related to the inducing infection of the EM (mainly due to Mycoplasma pneumoniae). When extensive skin involvement occurs, some patients may show signs of dehydration. Others with mucosal involvement may lose weight because of difficulty eating."}, {"id": "pubmed23n0742_14680", "title": "Two contrasting post-zoster dermatomal phenomena.", "score": 0.009615384615384616, "content": "A 29-year-old, normotensive, nondiabetic man presented with a 9-day history of a scaly, pruritic eruption involving the right chest, axilla, and arm. He had a history of herpes zoster involving the same areas about 4 weeks ago. The present eruption started after the herpetic lesions had healed. Examination revealed scaly, erythematous plaques and papules involving the right side of the chest, axilla, and arm in a dermatomal pattern (figure 1). Removal of the scales revealed underlying bleeding points (positive Auspitz sign). The rest of the body, including scalp, palms, soles, and nails, were normal. There was no history suggestive of psoriasis in any family member. Systemic examination and routine investigations were noncontributory. A clinical diagnosis of psoriasis was made and confirmed by histopathologic examination of a skin biopsy sample. The patient was prescribed a topical clobetasol cream and oral levocetirizine. The eruption resolved completely after 3 weeks. A 43-year-old normotensive, nondiabetic woman presented with a 2-day history of fever, arthalgias, and generalized erythematous dermatitis. Five days ago, the patient had a toothache for which she was prescribed injectable ampicillin. After receiving ampicillin for 3 days, she developed fever, myalgias, and arthalgias, which was followed several hours later by an erythematous eruption. The dermatitis started on the trunk and, over a period of several hours, progressed to involve the face and limbs. The eruption was slightly pruritic. History revealed herpes zoster 7 months ago involving left thoracic dermatomes, for which the patient was treated with valacyclovir (1 g thrice a day x 7 days) and analgesics. There was no history of post-zoster neuralgia. On examination, the patient was febrile (oral temperature 102 degrees F), her heart rate was 118 beats per minute, and her blood pressure was 110/70 mm Hg. Cutaneous examination revealed an erythematous, maculopapular dermatitis involving the face and limbs in a bilaterally symmetrical pattern; the palms and soles were also bilaterally involved. The whole of the trunk was involved with erythematous and, in places, violaceous, maculopapular eruption except for a small area on the left side corresponding to T8 and T9 thoracic dermatomes (Figure 2). Complete blood cell counts revealed eosinophilia (9%) and liver function tests, kidney function tests, random blood sugar, routine urine examination, and blood and urine cultures were noncontributory. Histopathologic examination of lesional skin biopsy revealed an intense mononuclear cell infiltration with many eosinophils and an interface dermatitis with hydropic degeneration of basal keratinocytes, while in the spared area, only slight lymphocytic infiltration was present in a perivascular distribution. Based on the history and examination, a diagnosis of ampicillin-induced drug dermatitis was made. The ampicillin was stopped and the patient was put on a short course of oral prednisolone, antipyretics, and topical calamine. The patient was afebrile in 2 days and the eruption resolved completely in 8 days."}, {"id": "InternalMed_Harrison_4109", "title": "InternalMed_Harrison", "score": 0.009615384615384616, "content": "Staphylococcal scalded-skin syndrome (SSSS) and bullous impetigo are two blistering disorders associated with staphylococcal (phage group II) infection. In SSSS, the initial findings are redness and tenderness of the central face, neck, trunk, and intertriginous zones. This is followed by short-lived flaccid bullae and a slough or exfoliation of the superficial epidermis. Crusted areas then develop, characteristically around the mouth in a radial pattern. SSSS is distinguished from TEN by the following features: younger age group (primarily infants), more superficial site of blister formation, no oral lesions, shorter course, lower morbidity and mortality rates, and an association with staphylococcal exfoliative toxin (“exfoliatin”), not drugs. A rapid diagnosis of SSSS versus TEN can be made by a frozen section of the blister roof or exfoliative cytology of the blister contents. In SSSS, the site of staphylococcal infection is usually extracutaneous (conjunctivitis, rhinorrhea,"}, {"id": "wiki20220301en288_27334", "title": "Urticarial allergic eruption", "score": 0.009523809523809525, "content": "Urticarial allergic eruption is a cutaneous condition characterized by annular or gyrate urticarial plaques that persist for greater than 24 hours. See also Urticaria List of cutaneous conditions References External links Urticaria and angioedema"}, {"id": "article-21300_25", "title": "Erythema Multiforme -- Prognosis", "score": 0.009523809523809525, "content": "The prognosis is mainly related to the body surface area detached. The healing is obtained spontaneously in 2 to 3 weeks for the EMm and 4 to 6 weeks for the EMM. The mucosal lesions always take longer to heal. The healing of the mucocutaneous lesions is without scarring but with frequent dyschromia. Recurrences are seen in less than 5% of cases, mainly in forms due to herpes infection."}, {"id": "pubmed23n0947_16304", "title": "Clindamycin-induced Maculopapular Exanthema with Preferential Involvement of Striae Distensae: A Koebner phenomenon?", "score": 0.009433962264150943, "content": "Clindamycin is a lincomycin-derived antibiotic useful for the treatment of anaerobic and Gram-positive aerobic bacterial infections. Cutaneous adverse reactions are usually maculopapular exanthemas, although hypersensitivity syndrome, acute generalized exanthematous pustulosis, and Stevens-Johnson syndrome have also been reported (1). We report the case of a patient with a maculopapular rash triggered by clindamycin who developed cutaneous lesions on striae distensae (SD). A 47-year-old woman was referred to our clinic for pruritic cutaneous lesions which had started 6 days earlier. Her past clinical history included hypertension, hypothyroidism, hyperuricemia, cholecystectomy, caesarean section, and endometriosis-related abdominal surgery, and she was taking levothyroxine, allopurinol, imidapril, and omeprazole. The skin rash first developed on her neck and back on the 3rd day of clindamycin oral treatment (300 mg every 6 hours), which was prescribed as antibiotic prophylaxis for a tooth implant. General malaise (but not fever) was also reported. Physical examination revealed an erythematous maculopapular eruption symmetrically distributed on the neck, abdomen, and back (Figure 1, A), with isolated lesions involving the proximal upper and lower limbs (Figure 1, B). There was a striking vertical distribution of skin lesions along the SD on the lateral sides of the abdomen (Figure 1, C). No mucosal involvement was found, and laboratory studies showed no abnormalities. Clindamycin withdrawal was followed by prescription of a course of oral deflazacort, starting at 30 mg daily and tapering down during a 9-day period. On the 5th day of treatment, the rash had almost cleared with minimal desquamation (Figure 1, D). Eight weeks after clearance of the skin rash, informed consent was obtained in order to perform an allergological evaluation of clindamycin, including prick and intradermal (ID) tests on the forearm and patch tests on the upper back (2). For patch testing, powder of the commercial capsules (Dalacin®) was diluted in petrolatum (pet.) and water (aq.), resulting in a final 1% clindamycin dilution. Parenteral clindamycin preparations were used in therapeutic concentrations for prick tests (150 mg/mL) and dilutions in saline of 1/100 and 1/10 for the ID test. Other authors have reported that these concentrations do not seem to irritate the skin (3-6). Prick and ID tests were assessed after 20 min and 24 hours, respectively. Patch tests were removed after the 2nd day, and late reactions were evaluated on day 2 and day 4. Prick and ID test results after 20 min were negative. Late results of ID tests with clindamycin (1.5 and 15 mg/mL) were positive: erythematous infiltrated papules about 7×7 mm and 18×15 mm were observed at 24 hours and lasted until the 8th day. Patch tests with clindamycin 1% in pet. and 1% in aq. were also positive (+ on day 2 and day 4). Positive late skin tests suggested delayed-type non-IgE-mediated allergic clindamycin hypersensitivity. Oral challenge tests are considered to be the gold standard to establish or exclude drug hypersensitivity. Due to the positive result of late skin test to clindamycin, oral challenge was not performed in our patient (3,5). The Koebner isomorphic phenomenon has been described in cutaneous reactions induced by drugs, such as antibiotics and chemotherapy. Chronic pressure on the skin is probably involved in the onset of skin lesions in hand-foot eruptions induced by tyrosine kinase inhibitors (sorafenib and sutinib). Solar exposure and cutaneous trauma also seem to play a role in the location of papulopustular eruptions caused by endothelial growth factor receptor inhibitors (erlotinib) (7). More frequent involvement in traumatized skin and surgical scars has been reported in the context of linear IgA bullous dermatosis and leukocytoclastic vasculitis triggered by vancomycin and cefuroxime (8). SD are produced by non-penetrating physical trauma, similar to friction or pressure. Different dermatoses can develop along SD skin lesions (like plaque psoriasis, pustular psoriasis, lichen planus, vitiligo, discoid lupus erythematosus, lupus vasculitis, urticarial vasculitis, or chronic graft-versus-host disease) (9). Bevacizumab, etretinate, and corticosteroid-induced ulcers, hyperpigmentation caused by bleomycin, and urticariform lesions triggered by diclofenac are examples of different type of drug-induced abnormalities involving SD (10). In summary, we identified clindamycin as the cause of the cutaneous reactions that occurred in our patient on the basis of the results of the skin tests and clinical history. Our findings confirmed a delayed-type hypersensitivity reaction, possibly involving a T-cell-mediated immunologic mechanism. Intradermal and patch tests were found to be useful in order to confirm the diagnosis (4,5). We did not find reports in the literature of drug-induced cutaneous eruptions along the SD as a manifestation of a Koebner phenomenon. Clinical underreporting of this phenomenon could explain the scarce literature on this cutaneous adverse reaction."}, {"id": "pubmed23n0755_3767", "title": "[Lip synechiae after erythema multiforme].", "score": 0.009433962264150943, "content": "Mucosal erosions in bullous diseases and leading to mucosal sequelae are widely described in toxic epidermal necrolysis (TEN). These complications cause disfigurement and functional impairment. They are more rarely reported in erythema multiforme (EM). We report a case of lip adhesion following EM induced by Mycoplasma pneumoniae. A 12-year-old boy was hospitalized in a paediatric intensive care unit. He had a prominent target skin rash on the palms and soles. Mucosal injury was associated with conjunctivitis, balanitis without dysuria and hyperalgesic stomatitis. M. pneumoniae serology was positive with immunoglobulin M. We made a diagnosis of EM secondary to M. pneumoniae infection. Two months later, the skin lesions had completely disappeared but the patient's mouth opening was limited to 25 mm and he presented bilateral adhesions between the upper and lower lips of 5mm on the right and 8mm on the left resulting in aesthetic and functional damage. Mucosal damage and its sequelae have been widely described in TEN. Ophthalmic sequelae are more frequent. A case of labial synechiae secondary to TEN has been reported. In EM, mucosal lesions occur in 100% of cases with a further mucosal problem being present in 50% of patients. Mucosal damage has been reported during EM flares but there are no studies of side-effects after the acute episode. Oral mucosal adhesions can cause cosmetic sequelae, but above all they hinder functional prognosis. These complications must be prevented by making gutters of vestibular deepening and lip movements with maximum mouth opening several times a day, starting as soon as possible. Appropriate pain management should be undertaken to ensure patient comfort and avoid the need for analgesics and restriction of movement. Mucosal sequelae exist in EM. Whatever their cause, complications involving the mucosa must be prevented through early, tailored and multidisciplinary treatment. Adequate pain management must not be overlooked."}, {"id": "pubmed23n0961_20010", "title": "Annular Lesions: Diagnosis and Treatment.", "score": 0.009345794392523364, "content": "Annular lesions can present in a variety of diseases. Knowledge of the physical appearance and history of presentation of these skin findings can help in the diagnosis. A pruritic, annular, erythematous patch that grows centrifugally should prompt evaluation for tinea corporis. Tinea corporis may be diagnosed through potassium hydroxide examination of scrapings. Recognizing erythema migrans is important in making the diagnosis of Lyme disease so that antibiotics can be initiated promptly. Plaque psoriasis generally presents with sharply demarcated, erythematous silver plaques. Erythema multiforme, which is due to a hypersensitivity reaction, presents with annular, raised lesions with central clearing. Lichen planus characteristically appears as planar, purple, polygonal, pruritic papules and plaques. Nummular eczema presents as a rash composed of coin-shaped papulovesicular erythematous lesions. Treatment is aimed at reducing skin dryness. Pityriasis rosea presents with multiple erythematous lesions with raised, scaly borders, and is generally self-limited. Urticaria results from the release of histamines and appears as well-circumscribed, erythematous lesions with raised borders and blanched centers. Annular lesions occur less commonly in persons with fixed drug eruptions, leprosy, immunoglobulin A vasculitis, secondary syphilis, sarcoidosis, subacute cutaneous lupus erythematosus, and granuloma annulare."}, {"id": "pubmed23n0518_15836", "title": "Life-threatening disorders of mucous membranes.", "score": 0.009345794392523364, "content": "Oral mucosa is one of the first barriers to the outside world which encounters various antigens, microorganisms and physical agents. Numerous oral pathologies challenge the dermatologists. Some may be the first sign of an underlying immunosuppression, while others are the inevitable serious outcomes of long-lasting mucosal disease. The differential diagnosis is crucial in terms of prompt and effective treatment."}, {"id": "wiki20220301en247_14461", "title": "Tumid lupus erythematosus", "score": 0.009259259259259259, "content": "Tumid lupus erythematosus is a rare, but distinctive entity in which patients present with edematous erythematous plaques, usually on the trunk. Lupus erythematosus tumidus (LET) was reported by Henri Gougerot and Burnier R. in 1930. It is a photosensitive skin disorder, a different subtype of cutaneous lupus erythematosus (CLE) from discoid lupus erythematosus (DLE) or subacute CLE (SCLE). LET is usually found on sun-exposed areas of the body. Skin lesions are edematous, urticarialike annular papules and plaques. Topical corticosteroids are not effective as treatment for LET, but many will respond to chloroquine. LET resolves with normal skin, no residual scarring, no hyperpigmentation or hypopigmentation. Cigarette smokers who have LET may not respond very well to chloroquine. It has been suggested that it is equivalent to Jessner lymphocytic infiltrate of the skin. See also Lupus erythematosus List of cutaneous conditions References Cutaneous lupus erythematosus"}, {"id": "wiki20220301en070_18335", "title": "Oral mucosa", "score": 0.009259259259259259, "content": "Mucous Membrane Pemphigoid: Autoimmune disease which affects only mucous membranes with clinical presentation of hard and rigid blisters which then rupture eventually into deep ulcers. Cutaneous Lupus Erythematosus: These present as oral discoid lesions which may be present on the inner cheek and behind the lips. White papules may also be present."}, {"id": "pubmed23n0551_12392", "title": "Generalized linear porokeratosis.", "score": 0.009174311926605505, "content": "A 23-year-old woman was seen for widespread skin lesions present since the age of 2.5 years. Twenty years ago, she developed a brown macular lesion on her right buttock. The lesion became hyperkeratotic and subsequently spread through the posterior aspect of her right leg. It later spread to the right side of the trunk and to the right arm. When she was 9 years old, she developed similar lesions on her left arm and leg. After she was 13 years old, no new skin lesions appeared. There was no family history of similar lesions. On examination, there were numerous linear and whorled, reddish-brown, hyperkeratotic plaques, with central atrophy and raised borders, following Blaschko's lines on all of the extremities. These lesions on the extremities extended to the dorsum of the hands and feet (Fig. 1). She had hyperkeratotic lesions on the pressure points of both of the soles, but no palm involvement. The number of lesions on the right side was greater than that on the left. Reddish-brown annular plaques with central atrophy and raised borders, appearing in zosteriform configuration, and numerous individual 2-3-mm erythematous lichenoid papules were observed on the right side of the thorax and the right inguinal region (Fig. 2). No face, scalp, or mucous membrane involvement was seen. The nails of the second and fifth fingers of the right hand and the nail of the third finger of the left hand showed nail dystrophy with longitudinal ridges and pterygium. All the nails of the right foot and the nails of the first and fifth toes of the left foot showed dystrophic changes with subungual keratosis. The patient was otherwise in good health. Two biopsy specimens taken from a hyperkeratotic plaque and a lichenoid papule showed an epidermal invagination with angulated parakeratotic tier, denoting cornoid lamella. The epidermis just underneath the cornoid lamella displayed vacuolization and the granular layer was absent. The adjacent epidermis was atrophic, and hydropic degeneration within the basal cell layer was seen. In the dermis, a nonspecific, mild, chronic, inflammatory cell infiltrate, telangiectatic vessels, and pigment-laden macrophages were present. These findings were consistent with linear porokeratosis (Fig. 3). Microscopic examinations and mycologic cultures of the nails were negative. We decided to treat our case systemically with retinoids, but the patient refused this therapy. So, topical tretinoin 0.05% was started once a day. A marked improvement was observed in hyperkeratosis through the first 4 weeks of treatment and plateaued at 8 weeks. After 10 weeks, the lesions had almost disappeared. We planned to continue the applications every other day. One year later, she remains stable with application of topical tretinoin 0.05% twice a week and is satisfied with the final appearance. She is under regular follow-up."}, {"id": "pubmed23n0792_15532", "title": "[Pepmhigus of the mucous membrane of the mouth].", "score": 0.009174311926605505, "content": "Pemphigus is a rare autoimmune disease of the skin and mucous membranes. It is characterized by autoantibodies directed against the desmosomal adhesion proteins of epithelial cells (desmoglein type I and III) resulting in acantholysis of the epithelium. Painful blisters or erosions are typical clinical findings. Pemphigus is a severe disease that may be fatal. Early diagnosis of pemphigus is important in order to be able to start the treatment as soon as possible and to halt the progression of the disease and avoid serious secondary infections."}, {"id": "pubmed23n0559_8630", "title": "Juvenile mycosis fungoides treated with bexarotene and PUVA.", "score": 0.00909090909090909, "content": "A 14-year-old Caucasian boy presented with a 4-month history of a slightly pruritic eruption that began on the hips and later extended to the trunk and upper and lower limbs. The patient did not present fever, weight loss, or asthenia. Physical examination revealed multiple, red, desquamative, oval patches with areas of healthy skin between them, which covered nearly 50% of the body surface area. The palms, soles, face, and mucosa were not affected. In addition, he presented two violet-colored infiltrated plaques on the left thigh and right buttock (Fig. 1). There were multiple, &gt; 1 cm, freely mobile, axillary and inguinal nodes. In follow-up, the patient developed two red-colored, mobile, well-delimited cutaneous nodules of 2.5 cm in diameter in the right hemithorax and lumbar area. The lumbar nodule regressed spontaneously before treatment. The clinical diagnosis was mycosis fungoides. We obtained three skin biopsies, one from a patch lesion and the others from a nodule; the third was sent to a reference hospital to determine the rearrangement. Histologic examination was similar in the three biopsies and revealed an atypical lymphoid infiltrate in the superficial dermis with epidermotropism and a tumoral nodule of atypical, small-sized lymphocytes in the deep dermis and subcutaneous level (Fig. 2). The atypical infiltrate was CD3+, CD4+, CD8-, T-cell intracellular antigen (TIA)+/-, Epstein-Barr-encoded RNA (EBER)-, and CD56-. The biopsy of one left axillary adenopathy was compatible with mycosis fungoides (Fig. 3). Amongst the additional tests carried out was a blood analysis showing 5300 leukocytes (neutrophils, 35%; lymphocytes, 40.7%; monocytes, 16.8%; eosinophils, 6.40%) without Sézary cells, normal lactate dehydrogenase (LDH), immunoglobulin E (IgE) of 497 U/mL (normal, 3-100 U/mL), and beta2-microglobulin of 3.09 mg/L (normal, 1.64 +/- 0.58 mg/L). A bone marrow study and a thoraco-abdomino-pelvic scan were normal. The rearrangement in the skin was monoclonal, whereas in peripheral blood and lymph nodes it was polyclonal. With the diagnosis of mycosis fungoides stage IVA (according to the TNM classification), treatment was initiated with psoralen plus ultraviolet light A (PUVA), three times a week, plus oral bexarotene at a dose of 300 mg/m2/day. The parents were informed that this treatment was not approved for this age group and informed consent was obtained. The clinical tolerance to bexarotene was very good, although low doses of atorvastatin (10 mg/day) and 75-100 mg of thyroxine were needed to control the expected adverse reactions to oral retinoid. After 32 sessions of PUVA and 6 months of treatment with oral bexarotene, the skin patches regressed, except for the plaque on the left buttock and the nodule on the right hemithorax (Fig. 4). There was no evidence of lymphadenopathy clinically or via sonographic evaluation. Bexarotene was discontinued after patient clearance and resolution of adenopathies. Nevertheless, 5 months after discontinuation of oral treatment, the patient developed multiple, scaling, nonconfluent macules on the trunk and arms affecting almost 30% of the body surface area, which disappeared with the application of methylprednisolone aceponate. He did not present significant lymphadenopathies."}, {"id": "pubmed23n0277_12867", "title": "[Pemphigus vulgaris and benign cicatricial mucous membrane pemphigoid in the upper respiratory tract and esophagus].", "score": 0.00909090909090909, "content": "Pemphigus vulgaris and benign cicatricial membrane pemphigoid are both autoimmune, blistering, dermatologic diseases characterised clinically by tense bullae on skin or on mucous membranes. Both diseases are rare, but very serious, associated with a high death rate (pemphigus) or high morbidity with cicatricial mucosal lesions (pemphigoid) if untreated. These diseases are discussed and two case stories mentioned where the primary focus was in the upper aerodigestive tract, which is very seldom. The otolaryngologist can make an important contribution to the early recognition, diagnosis, and management of these diseases. The biopsy must undergo immunofluorescence examination."}, {"id": "pubmed23n0785_6991", "title": "An 8-month-old boy with purpuric skin lesions. Acute hemorrhagic edema of infancy.", "score": 0.009009009009009009, "content": "A previously healthy 8-month-old Hispanic boy presented with a 5-day history of an erythematous, non-pruritic papular eruption on both legs. The eruption was initially diagnosed as impetigo by his primary care practitioner but progressed despite trimethoprim / sulfamethoxazole therapy, with extension to the face, trunk, and all extremities. When the patient subsequently developed a fever of 100.8° F, emesis, diarrhea, and upper respiratory symptoms, he was referred to the pediatric dermatology clinic for evaluation. Further questioning revealed a 3-day febrile illness 6 weeks prior to presentation that was treated with ceftriaxone. Review of systems failed to identify any hematuria, blood in stool, or abdominal pain, but the parents did report swelling of the extremities and face, as well as decreased oral intake. On examination, the infant was in no apparent distress, afebrile, and had mild rhinorrhea. His mucous membranes were unaffected, and no lymphadenopathy or hepatosplenomegaly was noted. Cutaneous exam revealed numerous edematous erythematous to violaceous plaques on the cheeks, arms, buttocks, and legs with minimal involvement of the trunk. Several lesions on the arms had a distinct cockade (rosette or iris-like) pattern. There were no vesicles, bullae, or necrosis. Edema of the bilateral lower extremities was noted. Laboratory work up revealed a normal complete blood count (CBC), comprehensive metabolic panel, creatinine, and urinalysis. Platelets were borderline elevated at 439 TH/μL (140-440 TH/μL), and erythrocyte sedimentation rate and C-reactive protein (CRP) were minimally elevated at 22 mm (0-15 mm) and 3.1 mg/dL (0.0-0.99 mg/dL), respectively."}, {"id": "pubmed23n0529_23487", "title": "[Changes of laryngeal mucosa in the course of pemphigus vulgaris].", "score": 0.009009009009009009, "content": "Blister diseases are chronic autoimmune reactions connected with formation of intraepithelial blisters. Pemphigus vulgaris (PV) change is appear most often, almost 80% of all cases. Erosions on mucosa appear as first symptoms at 50-70% patients. Blisters occurring on the skin are typical for this illness and usually come into with weeks or months with delay in relation to the changes on mucous membranes. In this work we have described character and location of changes on mucous membranes at 5 patients with PV, diagnosed based on clinical symptoms and confirmed in immunofluorescent investigations."}, {"id": "pubmed23n0634_10768", "title": "Most common clinical presentations of cutaneous mastocytosis.", "score": 0.008928571428571428, "content": "The term mastocytosis is referred to as an array of uncommon, usually sporadic, heterogeneous clinical illnesses that result from the hyperplasia of tissue mast cells. It comprises many different clinical manifestations varying from indolent cutaneous forms to systemic and malignant conditions. The characteristic presentation of mastocytosis consists of cutaneous manifestations: either a solitary mastocytoma, urticaria pigmentosa, or less commonly, diffuse cutaneous mastocytosis. Urticaria pigmentosa is the most common manifestation of cutaneous mastocytosis that manifests as a generalized eruption of round or oval erythematous macules, papules and plaques with variable amounts of brown pigment, usually on the trunk, but may also occur in all regions of the body including face and mucous membranes. Pruritus, dermographism and Darier's sign are additional features of these eruptions. Mastocytosis may also be manifested as mastocytoma, a rare, benign, pediatric tumor that results from hyperplasia of mast cells in papillary dermis in the first few weeks of life. The clinical course of mastocytosis is variable. The prognosis for the majority of pediatric patients with urticaria pigmentosa is extremely good, and over half of cases clear completely by adolescence, while those with aggressive systemic mastocytosis or mast cell leukemia show a progressive course, usually with a fatal outcome."}, {"id": "article-42186_13", "title": "Mycoplasma pneumoniae–Induced Rash and Mucositis (MIRM) -- History and Physical", "score": 0.008928571428571428, "content": "In contrast, EM presents initially as a cutaneous acral rash with macules that evolve into papules, plaques, and subsequently, typical target lesions and/or atypical target lesions (those that are raised). These target lesions spread centripetally to the trunk and face. EM minor has little or no mucous membrane involvement, and EM major has a rash to one or more mucous membranes. SJS/TEN manifests as a rash of macules, purpura, diffuse erythema, atypical target lesions (those that are flat), flaccid blisters that are extensive (not sparse) in number and initially more centrally located and then coalesce and spread to the face and limbs, with extensive mucous membrane involvement to two or more mucosal sites. The amount of skin detachment differentiates the extent of SJS/TEN. SJS has less than 10% skin detachment. Ten to 30% skin detachment is overlap SJS/TEN.  Greater than 30% skin detachment is TEN."}, {"id": "pubmed23n0930_14387", "title": "Bullous Pemphigoid Masquerading as Erythema Annulare Centrifugum.", "score": 0.008849557522123894, "content": "Dear Editor, Bullous pemphigoid (BP), a relatively common autoimmune blistering disease in the elderly, is characterized by large, tense bullae on urticarial, erythematous, or normal skin. However, atypical BP with polymorphic clinical presentations is rarely encountered, leading to misdiagnosis and delayed treatments (1). BP with lesions resembling erythema gyratum repens or figurate erythema has been regarded as a paraneoplastic phenomenon (1). Herein we report a case with erythema annulare centrifugum-like presentation of BP without evidence of underlying malignancy. A 64-year-old woman first presented with multiple large, tense bullae on the trunk and four extremities. She was diagnosed with BP according to the typical clinical, histopathological, and direct immunofluorescence findings. There were no annular lesions at that time. After a treatment course of systemic corticosteroids and azathioprine, the cutaneous symptoms were controlled. One year after discontinuing her medications, a pruritic bullous eruption reappeared with several annular erythematous plaques (Figure 1, a). The patient reported no mucosal involvement and took no new medications before the onset of skin lesions. On physical examination, multiple circular and arcuate erythematous lesions with slightly raised borders were seen on the trunk and both legs. Some erosions and tiny vesicles were noted on the erythematous edges. There were no other systemic symptoms or abnormalities. Laboratory studies, including complete blood count, liver and renal function tests, electrolytes, antinuclear antibody, complement levels, anti-Ro and anti-La antibodies, urine routine, stool routine, and chest X-ray, were normal. The biopsy specimen obtained from the rim of the annular lesions revealed slight vacuolar change at the dermoepidermal junction and perivascular and interstitial lymphocytic infiltration with numerous eosinophils in the upper dermis (Figure 1, b). Direct immunofluorescence showed linear deposits of immunoglobulin G (IgG) and C3 along the basement membrane (Figure 1, c). Histopathological features and immunofluorescence examinations were consistent with BP. There was no evidence of hematological or solid malignancy from further imaging and laboratory testing. The patient was started on oral prednisolone 30 mg/day and azathioprine 150 mg/day, with significant improvement over the following month. Complete regression of all skin lesions was achieved two months later, so the prednisolone dose was gradually tapered and then ceased. Under maintenance monotherapy of azathioprine 100 mg/day, there were no signs of BP recurrence or malignant disease during the one-year follow-up period. The annular erythema variant of BP is extremely rare. Therefore, in this case, erythema multiforme, subacute cutaneous lupus erythematosus, erythema annulare centrifugum, and urticarial vasculitis should be considered in the clinical differential diagnoses. Pathological features and immunofluorescence results can clearly rule out these possibilities. Until now, only 13 cases of BP presenting as annular erythema had been documented in the English literature, described as figurate erythema-like, erythema gyratum repens-like, or erythema annulare centrifugum-like manifestations (1-3). An association with internal malignancy in patients with these types of lesions had been reported (1). Nevertheless, as in most previous case reports (3), malignant diseases were not found in our patient. The precise mechanism of the annular erythema form of BP is unknown. Some authors considered it a variant of pre-bullous phase lesions, usually presenting as itchy erythematous patches or urticarial plaques (4). Based on this case, however, this assumption is less likely because the annular, erythema annulare centrifugum-like skin lesions appeared one year after the initial onset of bullous eruption, and simultaneously with the exacerbation of the bullous phase of BP. The exact pathogenesis of annular BP may be similar to that in erythema annulare centrifugum. Further investigations are warranted to clarify this issue. It should be noted that an erythema annulare centrifugum-like or figurate erythema-like manifestation in the absence of underlying malignancy can occasionally be a feature of BP. Making the correct diagnosis may be difficult if there is no concurrent bullous presentation. Clinicians should be vigilant for the development of this type of BP. The histological and direct immunofluorescence findings and the detection of circulating autoantibodies by indirect immunofluorescence or enzyme-linked immunosorbent assay remain crucial tools for establishing a definitive diagnosis."}, {"id": "article-135196_26", "title": "Toxic Epidermal Necrolysis -- Differential Diagnosis", "score": 0.008849557522123894, "content": "Stevens-Johnson syndrome (SJS) and Toxic epidermal necrolysis (TEN) are in the same disease spectrum. The only difference is the severity of skin affection. If epidermal detachment is less than 10% of the total body surface area, it is SJS. While if the affected body surface area is more than 30%, this is considered TEN. Overlap between the two conditions occurs when the affected body surface area is between 10 to 29%. The main differential diagnosis is erythema multiform major (EMM). It mainly affects less than 10% of body surface area and is characterized by the symmetric acral distribution of target lesions with or without blister formation. In contrast, SJS and TEN consist mainly of skin blisters arising on erythematous macules in central distribution (face and trunk). Moreover, 2 or more mucous membranes are involved in 90% of SJS and TEN cases. Other differential diagnoses include: [46]"}, {"id": "InternalMed_Harrison_3934", "title": "InternalMed_Harrison", "score": 0.008844813722862504, "content": "In examining the skin it is usually advisable to assess the patient before taking an extensive history. This approach ensures that the entire cutaneous surface will be evaluated, and objective findings can be integrated with relevant historical data. Four basic features of a skin lesion must be noted and considered during a physical examination: the distribution of the eruption, the types of primary and secondary lesions, the shape of individual lesions, and the arrangement of the lesions. An ideal skin examination includes evaluation of the skin, hair, and nails as well as the mucous membranes of the mouth, eyes, nose, nasopharynx, and anogenital region. In the initial examination, it is important that the patient be disrobed as completely as possible to minimize chances of missing important individual skin lesions and permit accurate assessment of the distribution of the eruption. The patient should first be viewed from a distance of about 1.5–2 m (4–6 ft) so that the general"}, {"id": "wiki20220301en424_1508", "title": "Skin manifestations of sarcoidosis", "score": 0.008771929824561403, "content": "Sarcoidosis, an inflammatory disease, involves the skin in about 25% of patients. The most common lesions are erythema nodosum, plaques, maculopapular eruptions, subcutaneous nodules, and lupus pernio. Treatment is not required, since the lesions usually resolve spontaneously in two to four weeks. Although it may be disfiguring, cutaneous sarcoidosis rarely causes major problems. Classification Morphology Ulcerative sarcoidosis is a cutaneous condition affecting roughly 5% of people with sarcoidosis. Annular sarcoidosis is a cutaneous condition characterized by papular skin lesions arranged in annular patterns, usually with a red-brown hue. Pattern Morpheaform sarcoidosis is a very rare cutaneous condition characterized by specific cutaneous skin lesions of sarcoidosis accompanied by substantial fibrosis, simulating morphea. Erythrodermic sarcoidosis is a cutaneous condition and very rare form of sarcoidosis."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[121, 529]], "word_ranges": [[20, 81]], "text": "The analytical data suggest diabetes insipidus (high plasma osm with low urinary osm). Now we must differentiate between central diabetes insipidus (lack of ADH) or nephrogenic diabetes insipidus (ADH does not exert its action at the renal level). This is achieved by the vasopressin test (intravenous administration of ADH and remeasurement of urinary osmolarity). Therefore, the correct answer is option 3."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "We are faced with polyuria. Initially we rule out diabetes mellitus (our patient has a normal blood glucose of 96mg/dl). The analytical data suggest diabetes insipidus (high plasma osm with low urinary osm). Now we must differentiate between central diabetes insipidus (lack of ADH) or nephrogenic diabetes insipidus (ADH does not exert its action at the renal level). This is achieved by the vasopressin test (intravenous administration of ADH and remeasurement of urinary osmolarity). Therefore, the correct answer is option 3.", "full_answer_no_ref": "We are faced with polyuria. Initially we rule out diabetes mellitus (our patient has a normal blood glucose of 96mg/dl). The analytical data suggest diabetes insipidus (high plasma osm with low urinary osm). Now we must differentiate between central diabetes insipidus (lack of ADH) or nephrogenic diabetes insipidus (ADH does not exert its action at the renal level). This is achieved by the vasopressin test (intravenous administration of ADH and remeasurement of urinary osmolarity). Therefore, [HIDDEN].", "full_question": "A 34-year-old woman is admitted for polyuria and polydipsia. In the first 24 hours of admission a diuresis of 8.2 liters is found and a blood test shows a glycemia of 96 mg/dL, natremia of 148 mEq/L and plasma osmolality of 309 mOsm/kg with urinary osmolality of 89 mOsmlkg. What diagnostic test should be performed next?", "id": 425, "lang": "en", "options": {"1": "Hypertonic saline infusion test for serial determination of antidiuretic hormone.", "2": "Dehydration test (Miller test).", "3": "Administration of desmopressin with serial monitoring of urine osmolality.", "4": "Determination of antidiuretic hormone in plasma.", "5": NaN}, "question_id_specific": 92, "type": "ENDOCRINOLOGY", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0101_14281", "title": "Disorders of antidiuretic hormone.", "score": 0.018499573742540493, "content": "Disorders of thirst and vasopressin secretion present clinically in one of three ways: as hypotonic polyuria (DI), as hypodipsic hyponatremia, and as hyponatremia. In evaluating a patient with DI, the major challenge is to differentiate between primary polydipsia and neurogenic and nephrogenic DI. This is best accomplished through a series of steps that start with simple clinical observation, and progress, as necessary, to more complicated diagnostic procedures (Fig. 1). If the diagnosis is not clear from the clinical setting and the patient's history, the first step is to measure plasma osmolality and sodium under conditions of ad libitum fluid intake. If the results are clearly above the upper limit of normal range, primary polydipsia is excluded and the work-up can proceed directly to administration of vasopressin or DDAVP and/or a measurement of plasma vasopressin levels to differentiate between neurogenic and nephrogenic DI. If basal plasma osmolality and sodium fall within normal range, the standard dehydration test should be performed. If urine osmolality does not increase above that of plasma despite evident dehydration, primary polydipsia is excluded and the effect of vasopressin or DDAVP on urine osmolality should be examined to differentiate between neurogenic and nephrogenic DI. If administration of antidiuretic hormone increases urine osmolality by more than 50 per cent, the patient has severe neurogenic DI. If the increase in urine osmolality is less than 50 per cent, the patient has nephrogenic DI. In patients who do not concentrate urine above that of plasma in response to dehydration, the best approach is to measure plasma vasopressin, osmolality, and sodium after the latter have been increased above normal range by dehydration and/or infusion of hypertonic saline. When these results are plotted on a suitable nomogram (Fig. 2), neurogenic DI can be clearly diagnosed from the relative deficiency of vasopressin. In patients with normal vasopressin levels, primary polydipsia can be differentiated from nephrogenic DI by examining the relationship of urine osmolality to plasma vasopressin (Fig. 3), obtained during dehydration and/or graded vasopressin infusion. In evaluating a patient with sustained hypernatremia, it is only necessary to assess thirst, which can be done by a simple bedside observation. In a patient without obvious neurologic or cognitive impairment, absence of thirst in the face of plasma osmolality above 305 mosm/kg (plasma sodium above 150 mEq/L) is diagnostic for hypodipsic hypernatremia. In a patient who presents with hyponatremia, the main objective is to differentiate between hyper-, hypo-, and euvolemic (SIADH) types"}, {"id": "pubmed23n0245_791", "title": "[Partial defect in the secretion of antidiuretic hormone and disproportionate polydipsia (author's transl)].", "score": 0.017241379310344827, "content": "A 20-year-old patient was evaluated because of polydipsia and polyuria; by means of the dehydration test a partial defect in the secretion of antidiuretic hormone (ADH) was demonstrated, since the urinary osmolality after the administration of exogenous vasopressin was superior by 25 percent to the maximum spontaneous urinary osmolality reached after a period of fluid restriction. Nevertheless, there was also a component of psychogenic polydipsia because the daily basal fluid intake was superior to 15 liters, and in view of the fact that the urinary osmolality could reach 600 mOsm/kg, the endocrine defect cannot totally be responsible for the enormous volume of fluid intake. This is the first case in the world literature in which the association between potomania and deficiency in the secretion of ADH is reported. Since ADH is one of the factors which regulate the behaviour of various animal species it is possible that its deficiency may be directly responsible for the psychic disorder which led to the potomania. It is also possible that an anatomical hypothalamic lesion, too small to be demonstrated, might have a simultaneous effect on the centers regulating thirst and the neurons producing vasopressin."}, {"id": "pubmed23n0377_1434", "title": "Differential diagnosis of polyuric/polydipsic syndromes with the aid of urinary vasopressin measurement in adults.", "score": 0.016998166172943998, "content": "A water deprivation test or a hypertonic saline infusion test with the measurement of plasma osmolality and plasma vasopressin are the gold standard tests in the differential diagnosis of polyuric syndromes. Because commercially available vasopressin kits are too insensitive for this approach, and the concentration of vasopressin in urine is much higher than in plasma, urinary vasopressin measurements may be an alternative to the more difficult plasma vasopressin measurement. The diagnostic value of the measurement of urinary vasopressin with a rather insensitive commercially available vasopressin kit was compared with plasma vasopressin measurement by a highly sensitive radioimmunoassay (RIA). Thirteen normal subjects and 27 patients with polyuria/polydipsia were examined by an 8-h fluid deprivation test. In all blood samples (0800 h, 1200 h, 1400 h and 1600 h) and in all urine collections (2-hourly fractions), osmolality as well as vasopressin were measured. Using plasma vasopressin measurement with a highly sensitive RIA as gold standard test, nine patients were classified as having primary polydipsia, whereas 18 had partial or complete cranial diabetes insipidus. Whereas the substitution of plasma vasopressin measurement by urinary vasopressin measurement alone did not provide 100% separation between both groups, the product of urinary vasopressin and urinary osmolality related to plasma osmolality completely separated the patients with primary polydipsia from those with diabetes insipidus. Urinary measurement of vasopressin and osmolality alone, which was recommended as a noninvasive diagnostic procedure in children, was too insensitive for exact differential diagnosis in our adult patients. The simultaneous measurement of plasma vasopressin and plasma osmolality in a dehydration test is the most powerful diagnostic tool in the differential diagnosis of polyuria/polydipsia. However, if highly sensitive assays for plasma vasopressin measurements are not available, the measurement of urinary vasopressin with commercially available, less sensitive RIAs may be a diagnostic alternative, which showed nearly the same sensitivity as plasma vasopressin measurement in our study population."}, {"id": "pubmed23n0828_23978", "title": "X-Linked Recessive form of Nephrogenic Diabetes Insipidus in a 7-Year-Old Boy.", "score": 0.016167318919612497, "content": "Nephrogenic diabetes insipidus (NDI) is caused by the inability of renal collecting duct cells to respond to arginine vasopressin (AVP)/antidiuretic hormone (ADH). We present the case of a 7-year-old boy with a history of excretion of large amounts of dilute urine and polydipsia since infancy. The boy had several vomiting episodes with mild dehydration during the first 3 years of life. There was no evidence of headaches, dizziness or visual problems. He drinks between 2 and 3 L/day and has 24-hour diuresis of 2 liters, now. He has prepubertal appearance with appropriate weight [+0.85 standard deviation score (SDS)] and height (+0.15 SDS) for his age. His intelligence was also normal. The water deprivation test showed low urine osmolality after 8 hours of dehydration. After desmopressin administration, urine osmolality remained low. Serum osmolality was in the normal range for sex and age before and after desmopressin administration. This indicated a nephrogenic form of diabetes insipidus. Molecular analyses revealed a P286L [p.Pro(CCC)286Leu(CTC)] mutation in the AVPR2 gene, that was inherited from his mother. This patient is the first case with genetically confirmed X-linked inherited form of NDI in the Republic of Macedonia. Molecular analysis confirmed the clinical diagnosis and enabled genetic advice for this family. "}, {"id": "pubmed23n0623_24617", "title": "Diagnosis of the syndrome of inappropriate secretion of antidiuretic hormone.", "score": 0.015852130325814534, "content": "Hyponatremia is a frequent condition in elderly patients. In diagnostic workup, a 24-hour urine sample is used to measure urinary osmolality and urinary sodium concentration necessary to confirm the diagnosis of the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). This study was undertaken to test the hypothesis that a spot urine sample would be sufficient for urinalysis. In nine patients with SIADH, morning spot and 24-hour urine samples were examined for osmolality and sodium concentration. Levels of arginine vasopressin, atrial natriuretic and brain natriuretic peptides, renin, and aldosterone were measured in the supine and upright positions of patients and compared with nine healthy age-matched control patients. The patients had low plasma osmolality (median 266 mOsm/kg) and measurable levels of arginine vasopressin (median 1.8 pg/mL). Values of osmolality in the spot urine (median 298 mOsm/kg) and in the 24-hour urine (median 215 mOsm/kg) did not differ significantly; neither did sodium concentration (medians 80 mmol/L in the spot urine versus 45 mmol/L in the 24-hour urine). Patients had significantly elevated plasma levels of brain natriuretic peptide (P = 0.007), elevated mean arterial blood pressure (P = 0.03), and lower plasma levels of creatinine (P = 0.002) compared to the controls. A spot urine sample seems to be sufficient to confirm the diagnosis of SIADH."}, {"id": "article-20428_55", "title": "Arginine Vasopressin Disorder (Diabetes Insipidus) -- Evaluation -- 2. Diagnosis of the type of polyuria-polydipsia syndrome", "score": 0.015648116535791512, "content": "Urine osmolality reaches the normal reference range. Urine osmolality is stable on two to three consecutive hourly measurements, even with rising plasma osmolality. Plasma osmolality is higher than 295 to 300 mOsm/kg Plasma Na greater than 145 mEq If AVP-R is suspected in newborns and young infants, the diagnostic test of choice is DDAVP (1 mcg subcutaneously or intravenously over 20 minutes, maximum dose of 0.4 mcg/kg). In children, the water deprivation test should be closely monitored. If one of the following endpoints is reached, discontinue the trial: Urine osmolality reaches the normal reference range. Plasma osmolality greater than 295 mOsm/kg to 300 mOsm/kg Plasma sodium greater than 145 meq/L Loss of 5% of body weight or signs of volume depletion"}, {"id": "wiki20220301en130_7148", "title": "Fluid deprivation test", "score": 0.014803690195237074, "content": "A fluid or water deprivation test is a medical test which can be used to determine whether the patient has diabetes insipidus as opposed to other causes of polydipsia (a condition of excessive thirst that causes an excessive intake of water). The patient is required, for a prolonged period, to forgo intake of water completely, to determine the cause of the thirst. This test measures changes in body weight, urine output, and urine composition when fluids are withheld. Sometimes measuring blood levels of ADH (a synonym for vasopressin) during this test is also necessary. If there is no change in the water loss despite fluid deprivation, desmopressin may be administered to distinguish between the two types of diabetes insipidus which are central & nephrogenic diabetes insipidus. The time of deprivation may vary from 4 to 18 hours. The serum osmolality and urine osmolality are both measured in the test. Interpretation of WDT The conditions can be distinguished in the following way:"}, {"id": "pubmed23n0939_4940", "title": "Diagnostic value of the water deprivation test in the polyuria-polydipsia syndrome.", "score": 0.014403573544828843, "content": "Diabetes insipidus (DI) and primary polydipsia (PP) are characterised by polyuria and polydipsia. It is crucial to differentiate between these two disorders since the treatment is different. The aim of this study was to evaluate the diagnostic value of the short and an extended variant of the water deprivation test (WDT) and of measuring urinary vasopressin (AVP) in patients with polyuria and polydipsia. A retrospective, single-centre study based on WDTs performed between 2004 and 2014 including 104 consecutive patients with the polyuria-polydipsia syndrome. During a strict water deprivation, weight, urinary osmolality, urinary vasopressin and specific gravity were collected until one of the following was reached: i) &gt;3% weight reduction, ii) Urinary specific gravity &gt;1.020 or, urinary osmolality &gt;800 mOsm/L, iii) Intolerable adverse symptoms such as excessive thirst. Out of 104 patients (67 women, 37 men), 21 (20%) were diagnosed with DI and 83 (80%) with PP. The median (interquartile range; range) test duration was 14 hours (10-16; 3-36) in patients with DI and 18 hours (14-24; 7-48) in patients with PP (P=0.011). Of those diagnosed with PP, 22 (26%) did not reach urinary specific gravity &gt;1.020 nor urine osmolality &gt;800 mOsm/L. Urine AVP did not overlap between patients with PP and patients with central DI. The short WDT is of limited value in the diagnostic work-up of polydipsia and polyuria and a partial DI may have been missed in every fourth patient diagnosed with PP. Urinary AVP has excellent potential in discriminating PP from central DI."}, {"id": "pubmed23n0053_11997", "title": "A case of syndrome of inappropriate secretion of antidiuretic hormone (SIADH) with low plasma concentrations of antidiuretic hormone.", "score": 0.014130483387738674, "content": "A 66-year-old Japanese man presented with persistent hyponatremia without polydipsia and polyuria. Laboratory examination showed serum sodium of 117 mEq/l, plasma osmolality 239 mosm/kg, urine sodium 108 mEq/l, urine osmolality 577 mosm/kg, and normal levels (less than 2.0 pg/ml) of serum antidiuretic hormone (ADH). ADH release was regulated normally with changes in plasma osmolality. No obvious cause for the syndrome of inappropriate secretion of ADH (SIADH) could be detected. However, 20 months later, the patient had bouts of hematuria and was found to have cancer of the urinary bladder. Increased renal sensitivity to ADH was suspected as the underlying mechanism of SIADH."}, {"id": "pubmed23n0707_22077", "title": "Severe hyperosmolarity and hypernatremia in an adipsic young woman.", "score": 0.01404724106412662, "content": "Combined deficits in arginine vasopressin secretion (AVP) and thirst sensation can result in life threatening hyperosmolality and hypernatremia. Complications include seizures, profound volume contraction and renal failure. Fortunately, this is an uncommon clinical condition, with approximately 70 cases reported in the literature over the past 47 years [1]. Defects in AVP secretion and/or synthesis produce central diabetes insipidus (DI), polyuria with polydipsia, hypernatremia and hyperosmolality. Most awake and alert patients with an intact thirst stimulus will \"drink\" themselves back to a normal serum sodium and osmolality. However, if there is concomitant destruction of the osmoreceptors that regulate thirst, osmolal and volume homeostasis cannot be maintained. The relationships between urine osmolarity and serum osmolarity and plasma vasopressin levels are vital for distinguishing a reset osmostat from central DI. After obtaining approval from our institutional review board, we retrospectively reviewed the medical record of a 37-year-old patient who presented to our institution with a serum sodium of 176 mEq/l. Admission laboratory examination revealed: hemoglobin 12.8 g/dl; white blood cell count 4.7 × 103/µl, with a normal differential; random serum glucose 91 mg/dl ; sodium 176 mEq/l; plasma osmolality 366 mOsm/kg; BUN 33 mg/dl; serum creatinine 1 mg/dl; calcium 9.5 mg/dl; urine specific gravity 1.032; and urine osmolality 1,172 mOsm/kg. An MRI with contrast of the sella/ pituitary revealed an enhancing mass centered within the suprasellar cistern and anterior third ventricle, measuring 3.0 × 3.9 × 3.4 cm. The lesion appeared to involve the hypothalamus and displaced the optic chiasm inferiorly. Evaluation of pituitary function revealed normal serum levels of thyroid stimulating hormone, AM cortisol, luteinizing hormone, follicle stimulating hormone and prolactin. Figure 1 illustrates the relationship between measured serum AVP levels and serum osmolality. Figure 2 shows the relationship between measured urine and serum osmolality. If the serum AVP levels were not available, it would appear as though the patient had a reset osmostat. The kidneys appear to appropriately generate maximally concentrated urine at a serum osmolality above 348 but are unable to below this value. When compared with the normal curve, our patient's AVP levels were lower than expected for the corresponding osmolality. This pattern is consistent with a partial central DI. She does not have a reset osmostat. In the presence of significant volume contraction and a reduced GFR, her kidneys produced more concentrated urine despite markedly decreased central vasopressin production. As the volume contraction abated and the GFR improved, polyuria recurred, despite persistent hyperosmolarity and hypernatremia."}, {"id": "pubmed23n0119_3558", "title": "[Trials for simplified hypertonic saline test].", "score": 0.013811899482631191, "content": "Hypertonic saline test is indispensable for the evaluation of posterior pituitary function. However the test is not simple, including water loading, urine sampling and at least 45 min of hypertonic saline infusion, mostly because the test relies on urinary osmolality as an index of ADH secretion. The object of this study is try to simplify the test by directly measuring plasma ADH concentration before and after 10 min of hypertonic saline infusion. Intravenous infusion of hypertonic saline (5% NaCl, 0.24 ml/kg/min, for 10 min) was performed on normal subjects, patients with diabetes insipidus and patients with renal failure under chronic hemodialysis. Venous blood samples were obtained seriously including just before and after 10 min of the infusion. ADH was extracted from plasma using Sep-Pak C18 column and assayed by specific RIA. Minimum sensitivity of the assay was 0.25 pg/ml. The hypertonic saline infusion resulted in an increase of plasma osmolality by about 8 mOsm/kg H2O and plasma sodium concentration by 4 mEq/l. Plasma ADH increased from 0.77 +/- 0.09 to 3.42 +/- 0.73 pg/ml (m +/- SE, n = 8, p less than 0.01) in normal subjects of ad lib. water drinking and from 0.55 +/- 0.33 to 2.34 +/- 0.33 (m +/- SE, n = 4, p less than 0.05) in water loaded normal subjects (20 ml/kg of water, 60 min before hypertonic saline infusion).(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "article-27735_21", "title": "Primary Polydipsia -- Differential Diagnosis", "score": 0.013689281413087114, "content": "After polyuria (>40-50 ml/kg/24hrs) is confirmed, and urine osmolality is <800 mOsm/kg, serum sodium level has to be checked. If the serum sodium level is <135 meq, it is diagnostic of PP. If the serum sodium levels are >147, it is diagnostic of diabetes insipidus. If the serum sodium is between 135 and 147, the next step would be the water deprivation test. The traditional test that has been utilized by providers for a long time is the indirect water deprivation test that indirectly measures the activity of AVP. This test is started once hypotonic polyuria is confirmed, and serum sodium is between 135 and 147. Polyuria in primary polydipsia decreases with water deprivation (typically >8hrs), and urine osmolality increases (>800 mOsm/kg typically), this is diagnostic of PP. In diabetes insipidus, polyuria does not get better with water deprivation. If the urine osmolality remains <300 mOsm/kg after water deprivation, it is diagnostic of DI."}, {"id": "pubmed23n0253_8397", "title": "Altered renal handling of electrolytes in a child with central diabetes insipidus (CDI).", "score": 0.013652036156928525, "content": "A 12-year-old female child, with a history of polyuria and polydipsia of about three years duration, was admitted to Ethio-Swedish Paediatric Hospital, in Addis Abeba. Urine output in 24 hours averaged 5-6 litres, with a frequency of 15 times during the day and 7-8 times during the night. Random urine analysis showed an osmolality of 60 mOsm/kg, Na+ 27.1 mmol/L and K+ was 7.6 mmol/L. Basal plasma osmolality was 313 mOsm/kg with Na+ being 156 mmol/L and K+ 4.06 mmol/L. Water deprivation for nine hours failed to produce a concentrated urine, which was only 138 mOsm/kg at the end of the test, with a corresponding plasma osmolality of 336 mOsm/kg. After nine hours of water deprivation, urine Na+ increased from 27.1 to 37.3 mmol/L while K+ increased from 7.1 to 18.7 mmol/L. Lypressin, a vasopressin analogue, at a concentration of 0.3 IU/kg injected intramuscularly, resulted in a marked increase in urine osmolality to 586 mOsm/kg within two hours, associated with relief of symptoms. Urinary excretion of K+ was markedly increased during the vasopressin test while Na+ excretion was little affected. A case of central diabetes insipidus of undefined etiology is presented and the possibility of altered renal handling of electrolytes and an abnormal response to vasopressin in such cases is noted. The problem of management and the currently available treatment options are summarized."}, {"id": "article-25794_31", "title": "Neurohypophysis -- Evaluation -- Diabetic Insipidus", "score": 0.013447816200109779, "content": "Serum and urine sodium, serum and urine osmolality, hourly urine output, 24-hour urine volume, and specific gravity must be obtained. Central DI must be distinguished from nephrogenic DI. A water deprivation test can be used with or without desmopressin injection. In central DI, the vasopressin will correct urine osmolality while in nephrogenic DI, the correction will be suboptimal. [3] [25] [26] Criteria for the diagnosis of central DI: Polyuria in two consecutive hours of > 300 ml/hr or 4 to 5 ml/kg/hr in two consecutive hours Polyuria > 3L/24 hours or > 2ml/kg/hr in 24 hours Serum osmolality > 300 mOsm/kg Urine osmolality < 300 mOsm/kg Urine/plasma osmolality <  1 The specific gravity of less than 1.005"}, {"id": "pubmed23n1110_3988", "title": "Transient Antidiuretic Hormone Insufficiency Caused by Severe Hyperosmolar Hyperglycemic Syndrome Based on Nephrogenic Diabetes Insipidus.", "score": 0.013412109711919959, "content": "The hyperosmolar hyperglycemic state (HHS), an acute complication of diabetes mellitus with plasma hyperosmolarity, promotes the secretion of anti-diuretic hormone (ADH) and reduces the storage of ADH. Magnetic resonance T1-weighted imaging reflects ADH storage in the posterior pituitary lobe, which disappears when the storage is depleted. Whether the HHS induces ADH depletion leading to clinical manifestations has been unclear. A 55-year-old Japanese woman was admitted to our center because of mental disturbance and hypotension. She had received lithium carbonate for bipolar disorder and presented with polydipsia and polyuria from 15 years of age. On admission, she had mental disturbance (Glasgow Coma Scale, E4V1M1), hypotension (systolic blood pressure, 50 mmHg), and tachycardia (pulse rate, 123/min). Plasma glucose was 697 mg/dL osmolality was 476 mOsm/kg•H<sub2</subO, and bicarbonate was 23.7 mmol/L. The diagnoses of HHS and hypovolemic shock were made. During treatment with fluid replacement and insulin therapy, the urine volume continued to be approximately 3 to 4 L/day, and an endocrine examination revealed ADH insufficiency and nephrogenic diabetes insipidus. Desmopressin 10 μg/day and trichlormethiazide 2 mg/day were necessary and administered, and the endogenous ADH secretion improved gradually. The signal intensity of the pituitary posterior lobe, initially decreased on magnetic resonance T1 images, was also improved. This patient had ADH insufficiency associated with ADH depletion due to hyperosmolarity and nephrogenic diabetes insipidus. Clinicians should be aware of the risk of the development of critical HHS and relative ADH insufficiency in patients being treated with lithium carbonate."}, {"id": "wiki20220301en002_57945", "title": "Diabetes insipidus", "score": 0.013108038914490527, "content": "This test measures the changes in body weight, urine output, and urine composition when fluids are withheld to induce dehydration. The body's normal response to dehydration is to conserve water by concentrating the urine. Those with DI continue to urinate large amounts of dilute urine in spite of water deprivation. In primary polydipsia, the urine osmolality should increase and stabilize at above 280 mOsm/kg with fluid restriction, while a stabilization at a lower level indicates diabetes insipidus. Stabilization in this test means, more specifically, when the increase in urine osmolality is less than 30 Osm/kg per hour for at least three hours. Sometimes measuring blood levels of ADH toward the end of this test is also necessary, but is more time consuming to perform."}, {"id": "article-20428_54", "title": "Arginine Vasopressin Disorder (Diabetes Insipidus) -- Evaluation -- 2. Diagnosis of the type of polyuria-polydipsia syndrome", "score": 0.013106973347937203, "content": "To differentiate AVP-D and AVP-R and primary polydipsia, perform a water deprivation test and desmopressin (DDAVP) trial. Typically a 7-hour deprivation test is adequate to diagnose DI. Primary polydipsia may require more extended dehydration periods. The basic principle behind the water deprivation test is that in individuals with normal posterior pituitary and renal function (or those with primary polydipsia), an increase in plasma osmolality from dehydration stimulates AVP release from the posterior pituitary, which then leads to water reabsorption in the nephrons, thus resulting in concentration of urine and an increase in urine osmolality. In AVP-D or AVP-R, the urine fails to concentrate optimally with water deprivation, and there is persistent excretion of hypotonic urine. In adults, the water restriction test should be discontinued when one of the following is reached:"}, {"id": "wiki20220301en032_83058", "title": "Syndrome of inappropriate antidiuretic hormone secretion", "score": 0.01283532280836863, "content": "Diagnosis Diagnosis is based on clinical and laboratory findings of low serum osmolality and low serum sodium. Urinalysis reveals a highly concentrated urine with a high fractional excretion of sodium (high sodium urine content compared to the serum sodium). A suspected diagnosis is based on a serum sodium under 138. A confirmed diagnosis has seven elements: 1) a decreased effective serum osmolality - <275 mOsm/kg of water; 2) urinary sodium concentration high - over 40 mEq/L with adequate dietary salt intake; 3) no recent diuretic usage; 4) no signs of ECF volume depletion or excess; 5) no signs of decreased arterial blood volume - cirrhosis, nephrosis, or congestive heart failure; 6) normal adrenal and thyroid function; and 7) no evidence of hyperglycemia (diabetes mellitus), hypertriglyceridemia, or hyperproteinia (myeloma)."}, {"id": "pubmed23n0061_14703", "title": "The relationship between antidiuretic hormone and plasma or urine osmolalities during water restriction test and hypertonic saline loading test in normal children--a change in the apparent tubular response to AVP during these two tests.", "score": 0.012438574938574939, "content": "We present here the results of water restriction test (WRT) and hypertonic saline loading test (HSLT) in normal children. Maximal urine osmolality during WRT (W-Umax; 1040 +/- 154 mOsm/kg) may be age-dependent (W-Umax = 812 + 23*age, r = 0.52, p &lt; 0.05), although maximal arginine vasopressin (AVP) levels during WRT did not show any correlation with age. The relationship between plasma osmolality (Posm) and AVP during HSLT in children (AVP = 0.31* (Posm-277)) was similar to that in normal adults. A plateau urine osmolality during HSLT (H-Umax) was 713 +/- 109 mOsm/kg. It did not increase with age. AVP levels 3 h after the infusion did not correlate with age. Minimal AVP and Posm values (about 6 pg/ml, 295 mOsm/kg, respectively) for creating H-Umax apparently existed during HSLT. The minimal AVP value (about 6 pg/ml) for H-Umax (during HSLT) was higher than the AVP levels (2.41 +/- 1.37 pg/ml) at W-Umax (during WRT). W-Umax (1040 +/- 154 mOsm/kg) was significantly higher than H-Umax (713 +/- 109 mOsm/kg). Judging from the above comparison of AVP and Uosm (W, H-Umax) at the plateau state of WRT and HSLT in normal children, a change in the apparent tubular response to AVP may be one of the important factors to maintain circulatory volume (CV)."}, {"id": "InternalMed_Harrison_26611", "title": "InternalMed_Harrison", "score": 0.012271307452030342, "content": "Brain MRI Urinary frequency, nocturia, enuresis 24-h urine volume and osmolarity on unrestrictedfluid intake Volume >40 mL/kg Osmolarity <300 mosm/L Basal plasma AVP >1 pg/mL <1 pg/mL Pituitary bright spot Present Absent Anatomy Pathology? GU evaluation Volume <40 mL/kg Osmolarity >300 mosm/L If MRI and/or AVP assays with the requisite sensitivity and specificity are unavailable and a fluid deprivation test is impractical or undesirable, a third way to differentiate between pituitary DI, nephrogenic DI, and primary polydipsia is a trial of desmopressin therapy. Such a trial should be conducted with very close monitoring of serum sodium as well as urine output, preferably in hospital, because desmopressin will produce hyponatremia in 8–24 h if the patient has primary polydipsia."}, {"id": "wiki20220301en075_14909", "title": "Plasma osmolality", "score": 0.011976311560222793, "content": "Osmolality of blood increases with dehydration and decreases with overhydration. In normal people, increased osmolality in the blood will stimulate secretion of antidiuretic hormone (ADH). This will result in increased water reabsorption, more concentrated urine, and less concentrated blood plasma. A low serum osmolality will suppress the release of ADH, resulting in decreased water reabsorption and more concentrated plasma. Syndrome of inappropriate ADH secretion occurs when excessive release of antidiuretic hormone results in inappropriately elevated urine osmolality (>100 mOsmol/L) relative to the blood plasma, leading to hyponatraemia. This ADH secretion may occur in excessive amounts from the posterior pituitary gland, or from ectopic sources such as small-cell carcinoma of the lung. Elevation may be associated with stroke mortality. Calculated osmolarity (CO)"}, {"id": "pubmed23n0777_9374", "title": "Diabetes insipidus.", "score": 0.011754911754911754, "content": "Diabetes insipidus (DI) is characterized by hypotonic polyuria greater than 3 liters/24 hours in adults and persisting even during water deprivation. It is mostly due to a defect in arginin-vasopressin (AVP) synthesis (central DI); other causes are: AVP resistance (nephrogenic DI), abnormal thirst regulation (primary polydipsia) or early destruction of AVP by placental enzymes (gestational DI). A thorough medical history is warranted to investigate nocturnal persistence of polyuria (night waking being a good sign of its organic nature) to specify the onset and duration of the trouble, the medication use and the potential hereditary nature of the disorder. The next step is based on weight and blood pressure measurements and especially the quantification of beverages and diuresis over a 24-hour cycle. Assessment of signs of dehydration, bladder distention, pituitary hormone hyper- or hyposecretion, tumor chiasmatic syndrome, granulomatosis and cancer is required. The diagnosis is based on biological assessment, pituitary magnetic resonance imaging (MRI) and results of a desmopressin test. In severe forms of DI, urine osmolality remains below 250 mOsmol/kg and serum sodium greater than 145 mmol/L. In partial forms of DI (urine osmolality between 250 and 750), the water deprivation test demonstrating the incapacity to obtain a maximal urine concentration is valuable, together with vasopressin or copeptin measurement. The pituitary MRI is done to investigate the lack of spontaneous hyperintensity signal in the posterior pituitary, which marks the absence of AVP and supports the diagnosis of central DI rather than primary polydipsia (although not absolute); it can also recognize lesions of the pituitary gland or pituitary stalk. Acquired central DI of sudden onset should suggest a craniopharyngioma or germinoma if it occurs before the age of 30 years, and metastasis after the age of 50 years. Fifteen to 20% of head trauma lead to hypopituitarism, including DI in 2% of cases. Transient or permanent DI is present in 8-9% of endoscopic transphenoidal surgeries. Current advances in DI concern the etiological work-up, with in particular the identification of IgG4-related hypophysitis or many genetic abnormalities, opening the field of targeted therapies in the years to come."}, {"id": "pubmed23n1060_9822", "title": "ADIPSIC DIABETES INSIPIDUS AFTER SECOND RESECTION OF A HYPOTHAMIC ASTROCYTOMA.", "score": 0.011701839826839828, "content": "We report a case of adipsic diabetes insipidus (ADI) post-astrocytoma resection. Clinical and laboratory data are presented. A 16-year-old female with a history of incompletely resected hypothalamic astrocytoma was admitted with a headache. Head magnetic resonance imaging showed an interval increase in a suprasellar lesion with extension to the third ventricle. Following a second stage resection, she developed an increased urine output with diluted urine resulting in a negative fluid balance; however, she was unable to sense thirst. Blood tests showed a serum sodium of 155 mEq/dL (normal, 136 to 145 mEq/dL), serum osmolality at 321 mOs/kg (normal, 285 to 295 mOs/kg) and a urine osmolality of 128 mOsm/kg (normal, 300 to 1,600 mOsm/kg). Serum creatinine and potassium were normal. Pituitary hormone profiles were found to be normal: growth hormone 0.171 ng/mL (normal, 0.123 to 8.05 ng/mL), luteinizing hormone 3.44 mIU/mL (normal, 7.59 to 89.08 mIU/mL), follicle-stimulating hormone 5.60 mIU/mL (normal, 2.55 to 16.69 mIU/mL), thyroid-stimulating hormone 2.9 mIU/mL (normal, 0.35 to 4.94 mIU/mL), free thyroxine 0.92 ng/dL (normal, 0.7 to 1.48 ng/dL), adrenocorticotropic hormone 19.56 pg/mL (normal, 7.2 to 63.3 pg/mL), and prolactin 7.25 ng/mL (normal, 5.18 to 26.53 ng/mL). The patient was treated with desmopressin acetate 120 μg tablets twice daily with a fixed fluid intake of 1.5 to 2.0 L/day with close monitoring of fluid intake, output, and body weight. The response was good with a gradual reduction of serum sodium level of around 7 to 9 mEq/L/day. ADI is a rare entity of central diabetes insipidus, where the absence of polydipsia can be challenging in diagnosing and managing the condition. Cases of ADI are likely under reported and clinicians need to be aware of this condition."}, {"id": "InternalMed_Harrison_3621", "title": "InternalMed_Harrison", "score": 0.011563724678478778, "content": "Following the correction of hypernatremia and acute renal insufficiency with appropriate hydration (see below), the patient was subjected to a water deprivation test followed by administration of DDAVP. This test helps determine whether an inappropriate water diuresis is caused by CDI or NDI. The patient was water restricted beginning in the early morning, with careful monitoring of vital signs and urine output; overnight water deprivation of patients with diabetes insipidus is unsafe and clinically inappropriate, given the potential for severe hypernatremia. The plasma Na+ concentration, which is more accurate and more immediately available than plasma osmolality, was monitored hourly during water deprivation. A baseline AVP sample was drawn at the beginning of the test, with a second sample drawn once the plasma Na+ reached 148–150 meq/L. At this point, a single 2-μg dose of the V2 AVP receptor agonist DDAVP was administered. An alternative approach would have been to measure AVP"}, {"id": "wiki20220301en086_255", "title": "Hyperosmolar hyperglycemic state", "score": 0.011537163587277481, "content": "Diagnosis Criteria According to the American Diabetes Association, diagnostic features include: Plasma glucose level >30 mmol/L (>600 mg/dL) Serum osmolality >320 mOsm/kg Profound dehydration, up to an average of 9L (and therefore substantial thirst (polydipsia)) Serum pH >7.30 Bicarbonate >15 mEq/L Small ketonuria (~+ on dipstick) and absent-to-low ketonemia (<3 mmol/L) Some alteration in consciousness BUN > 30 mg/dL (increased) Creatinine > 1.5 mg/dL (increased) Imaging Cranial imaging is not used for diagnosis of this condition. However, if MRI is performed, it may show cortical restricted diffusion with unusual characteristics of reversible T2 hypointensity in the subcortical white matter."}, {"id": "article-27735_22", "title": "Primary Polydipsia -- Differential Diagnosis", "score": 0.010912698412698412, "content": "The administration of desmopressin differentiates between central and nephrogenic insipidus. If, after the administration of desmopressin, there is an increase of >50% in the urine osmolality, it is diagnostic of central DI. If there is an increase of <50% in the urine osmolality, it is diagnostic of nephrogenic DI. If the urine osmolality is between 300 mOsm/kg and 800 mOsm/kg after the water deprivation test, this could either be partial central DI or PP. To differentiate partial central DI from PP, desmopressin is administered. If the urine osmolality increases by > 9%, it is diagnostic of PP. If the Urine osmolality increases by <9%, it is diagnostic of partial central diabetes insipidus."}, {"id": "article-20428_60", "title": "Arginine Vasopressin Disorder (Diabetes Insipidus) -- Evaluation -- 2. Diagnosis of the type of polyuria-polydipsia syndrome", "score": 0.010906512116988237, "content": "Hypertonic saline (3% saline, 1027 mOsm/L) infusion coupled with plasma copeptin measurement is an alternative test that is now being recommended by many experts in the field of DI as the preferred test to be used in place of the water deprivation test."}, {"id": "pubmed23n0121_7561", "title": "An assessment of posterior pituitary function in patients with Sheehan's syndrome.", "score": 0.010905253283302064, "content": "Antidiuretic hormone (ADH) function was assessed in a group of 16 patients with Sheehan's syndrome and 17 controls. All patients were on adequate cortisone and thyroxine replacement therapy before testing. During the dehydration test the patients revealed an impairment of ADH function. The maximum urine osmolalities and the urine-plasma osmolality ratios were significantly lower in the patients with Sheehan's syndrome compared to controls (maximum urine osmolalities 633 +/- 38 (SEM) and 873 +/- 29 (SEM) mOsm/kg, respectively, P less than 0.001; urine-plasma osmolality ratios 2.15 +/- 0.14 (SEM) and 3.01 +/- 0.10 (SEM), respectively, P less than 0.001). Plasma osmolalities were significantly higher in the patients (296.1 +/- 1.2 (SEM) and 290 +/- 0.9 (SEM), respectively, P less than 0.001). The patients took a longer period to achieve these maximum urine osmolalities. Three of the patients with Sheehan's syndrome were diagnosed as having diabetes insipidus since their maximum urine osmolalities were below 600 mOsm/kg and following desmopressin all three had an increment in urine osmolality which exceeded 9%. In addition these three patients had a maximum urine-plasma osmolality ratio below 1.9. Thus, it appears the patients with Sheehan's syndrome have an impairment of ADH function which manifests in some as diabetes insipidus."}, {"id": "pubmed23n0059_5294", "title": "[Transient polyuria in pregnancy in diabetes insipidus and gestational diabetes].", "score": 0.010858050847457626, "content": "Two pregnant women developed overt polyuria (up to 11 l/day) and polydipsia during their second and third trimesters of pregnancy. In one patient hydronephrosis was present. Both patients suffered from mild gestational diabetes mellitus. Plasma sodium was 145 and 162 mmol/l. Polyuria and urinary hypo-osmolality responded well to desmopressin acetate. After delivery, polyuria and polydipsia disappeared in one patient and significantly improved in the other. Infusion of hypertonic saline one and two weeks respectively after delivery led to plasma hyper-osmolality (294 mosmol/kg and 305 mosmol/kg) without detectable stimulation of arginine vasopressin (AVP). Anterior pituitary function was normal. No stimulation of AVP occurred following insulin-induced hypoglycemia. AVP plasma disappearance after i.v. pulse injection of 1 microgram AVP as well as AVP plasma concentration after continuous infusion of 10 ng AVP/min was studied two weeks after delivery in one patient. The results suggested markedly elevated degradation of AVP compared to control subjects, probably due to an increased vasopressin activity. Eight months after delivery, hypertonic saline infusion in one patient led to a plasma-osmolality of 312 mosmol/kg without stimulation of AVP. In the second patient, AVP was not detectable (less than 0.2 pg/ml) six months after delivery when plasma osmolality was 290 mosmol/kg. Our studies demonstrate that a subclinical compensated diabetes insipidus was preexistent in both patients. Exacerbation occurred due to an increased AVP-clearance and presumably due to the hemodynamic and hormonal alterations during pregnancy, including a mild gestational diabetes mellitus."}, {"id": "article-20428_59", "title": "Arginine Vasopressin Disorder (Diabetes Insipidus) -- Evaluation -- 2. Diagnosis of the type of polyuria-polydipsia syndrome", "score": 0.010797897754419493, "content": "Copeptin (carboxy-terminal-Pro-vasopressin) is the C-terminal peptide of pro-vasopressin co-secreted with AVP from the posterior pituitary. [8] [53] Unlike plasma AVP measurement, copeptin measurement in the plasma is relatively less cumbersome. It has several advantages: copeptin can remain stable for days after blood sampling and can be measured relatively quickly. [53] Plasma levels of copeptin strongly correlate with plasma AVP levels over a wide range of osmolalities, both in healthy individuals and those with DI or primary polydipsia. [27] [54] Moreover, plasma copeptin demonstrates the same response to plasma osmolality and volume changes as plasma AVP. [9] [27] Several studies have been conducted to validate the utility of plasma copeptin in diagnosing hypotonic polyuric states and to distinguish one form from the other. [8] [9] [11] [38] [27] Hypertonic saline infusion test:"}, {"id": "wiki20220301en090_48172", "title": "Free water clearance", "score": 0.01073231205171345, "content": "For example, for an individual with a urine osmolality of 140 mOsm/L, plasma osmolality of 280 mOsm/L, and a urine production of 4 ml/min, the free water clearance is 2 ml/min, obtained from Interpretation Free water clearance can be used as an indicator of how the body is regulating water. A free water clearance of zero means the kidney is producing urine isosmotic with respect to the plasma. Values greater than zero imply that the kidney is producing dilute urine through the excretion of solute-free water. Values less than zero imply that the kidney is conserving water (likely under the influence of antidiuretic hormone, ADH), resulting in the production of concentrated urine. See also Renal clearance Renal physiology External links Overview at mcg.edu Overview at mmi.mcgill.ca Formula at mmi.mcgill.ca Renal physiology"}, {"id": "article-27735_26", "title": "Primary Polydipsia -- Differential Diagnosis", "score": 0.010549039681303274, "content": "If the level is less than 4.9 pmol/l, it is diagnostic of central DI. 96% of the patients with polyuria were accurately diagnosed with their respective diagnoses by measuring copeptin along with water deprivation/hypertonic saline infusion test. Given the cumbersomeness of the water deprivation test and the long duration involved with it, hypertonic saline infusion gained a reputation. Stimulation with hypertonic saline also requires frequent sodium checks and close monitoring. Keeping this in mind, a study was done with arginine infusion to measure the stimulated levels of copeptin instead of with hypertonic saline, which was promising. [29] More evidence is needed to substantiate this finding, though."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[88, 144]], "word_ranges": [[16, 26]], "text": "The diagnosis would be practically made with the AMA (1)."}, "2": {"exist": true, "char_ranges": [[146, 186]], "word_ranges": [[26, 33]], "text": "With 2 we would rule out hemochromatosis,"}, "3": {"exist": true, "char_ranges": [[188, 229]], "word_ranges": [[33, 41]], "text": "with 3 we would rule out Wilson's disease."}, "4": {"exist": true, "char_ranges": [[231, 325]], "word_ranges": [[41, 57]], "text": "With 4 we could rule out rare diseases such as biliary tract malformations or Caroli's disease"}, "5": {"exist": true, "char_ranges": [[330, 371]], "word_ranges": [[58, 66]], "text": "with 5 we could rule out viral hepatitis."}}, "full_answer": "Book picture of Primary Biliary Cirrhosis, one of those that do not occur in real life. The diagnosis would be practically made with the AMA (1). With 2 we would rule out hemochromatosis, with 3 we would rule out Wilson's disease. With 4 we could rule out rare diseases such as biliary tract malformations or Caroli's disease and with 5 we could rule out viral hepatitis.", "full_answer_no_ref": "Book picture of Primary Biliary Cirrhosis, one of those that do not occur in real life. The diagnosis would be practically made with the AMA (1). With 2 we would rule out hemochromatosis, with 3 we would rule out Wilson's disease. With 4 we could rule out rare diseases such as biliary tract malformations or Caroli's disease and with 5 we could rule out viral hepatitis.", "full_question": "A 52-year-old woman consulted because she had noticed during the previous week a yellowish discoloration of the conjunctivae. She does not refer to risky sexual behaviors or epidemiological history of risk of viral hepatitis. She does not consume alcohol or hepatotoxic drugs. She reports a one-year history of generalized pruritus, asthenia, dry mouth and absence of lacrimation with no known cause. Rest of the anamnesis without pathological data. Physical examination showed scratching lesions, conjunctival jaundice and non-painful hepatomegaly. The patient brings a blood test carried out in his company with the following pathological results: Bilirubin 3 mg/dl, FA 400 UI/ VSG 40mm 1 hour. Indicate which would be the best recommendation to establish the etiological diagnosis of the patient's condition:", "id": 8, "lang": "en", "options": {"1": "Anti-mitochondrial antibodies.", "2": "Study of Fe metabolism.", "3": "Study of copper metabolism.", "4": "Hepatic MRI.", "5": "Serology for B and C viruses."}, "question_id_specific": 232, "type": "DIGESTIVE", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1164_2588", "title": "Epstein-Barr Virus Hepatitis Masquerading as Painless Jaundice.", "score": 0.014586786644565475, "content": "Epstein-Barr virus (EBV) infection typically presents with pharyngeal symptoms and subclinical transaminitis. We present a case of a 27-year-old woman with no known past medical history who presented with painless jaundice and dark-colored urine for three days. Her review of systems was negative for fever, sore throat, nausea, vomiting, pruritus, or rash. Her last sexual contact was six months ago with a male partner, and she only drank alcohol socially. Family and surgical history were non-significant. Physical examination revealed 3+ bilateral conjunctival icterus without abdominal tenderness or organomegaly. She had elevated transaminases: alanine transaminase (ALT) of 1287U/L and aspartate aminotransferase of (AST) 1057U/L but her alkaline phosphatase (ALP) was only slightly above normal at 109U/L (normal range 35-104U/L), with a direct hyperbilirubinemia - total bilirubin 9.5mg/dl, direct bilirubin 6.8mg/dl; the abdominal ultrasound revealed non-dilated bile ducts. Hepatitis A, B, and C serology was negative, but her EBV serology showed an infection. She had incidental thalassemia minor without splenomegaly or asterixis. She was managed conservatively, and her liver enzymes trended down with supportive management. Although EBV is an uncommon cause of painless jaundice, this diagnosis should be considered, especially when other more common causes of jaundice have been ruled out. A high index of suspicion should be maintained to detect EBV hepatitis as it can easily be diagnosed through serological testing."}, {"id": "wiki20220301en023_9732", "title": "Anita Roddick", "score": 0.012958881203921867, "content": "Roddick explained that her hepatitis C was unexpectedly diagnosed in 2004, following a blood test that was part of a medical examination needed for a life insurance policy. The blood test indicated abnormal liver function and subsequent blood tests diagnosed hepatitis C. Roddick explained that she had a large blood transfusion in 1971, after the birth of her younger daughter, and that she was convinced that the transfusion had infected her with hepatitis C. This was about twenty years before blood donors in the United Kingdom were screened for hepatitis C. She reported that she had developed cirrhosis of the liver, and that her main symptoms were itching and poor concentration. She briefly mentioned that medical treatment with interferon did not suit her. Roddick explained that she kept fit and active, and that she attended biannual out-patient hospital appointments in Southampton, as well as being under review by the liver transplant team at the Addenbrooke's Hospital in Cambridge."}, {"id": "wiki20220301en330_13535", "title": "Social history of viruses", "score": 0.01154384328358209, "content": "Hepatitis is a disease of the liver that has been recognised since antiquity. Symptoms include jaundice, a yellowing of the skin, eyes and body fluids. There are numerous causes, including viruses – particularly hepatitis A virus, hepatitis B virus and hepatitis C virus. Throughout history epidemics of jaundice have been reported, mainly affecting soldiers at war. This \"campaign jaundice\" was common in the Middle Ages. It occurred among Napoleon's armies and during most of the major conflicts of the 19th and 20th centuries, including the American Civil War, where over 40,000 cases and around 150 deaths were reported. The viruses that cause epidemic jaundice were not discovered until the middle of the 20th century. The names for epidemic jaundice, hepatitis A, and for blood-borne infectious jaundice, hepatitis B, were first used in 1947, following a publication in 1946 giving evidence that the two diseases were distinct. In the 1960s, the first virus that could cause hepatitis was"}, {"id": "InternalMed_Harrison_23282", "title": "InternalMed_Harrison", "score": 0.010582010582010581, "content": "Approach to the Patient with Liver Disease Suspected Liver Disease Abnormal liver tests Acute < 6 months Chronic > 6 months Diagnostic evaluation 1. IgM Anti-HAV 2. HBsAg 3. IgM Anti-HBc 4. Anti-HCV 5. ANA, SMA 6. Monospot, heterophile 7. Ceruloplasmin 8. Alcohol history 9. Drug history Diagnostic evaluation 1. AMA 2. Drug history 3. Ultrasound/MRI 4. MRCP/ERCP Liver biopsy in acute liver disease: Reserved for patients in whom the diagnosis remains unclear despite medical evaluation Liver biopsy in chronic liver disease: Often valuable for diagnosis as well as staging and grading liver disease Diagnostic evaluation 1. HBsAg 2. Anti-HCV 3. Fe saturation, ferritin 4. Ceruloplasmin 5. ˜1AT 6. ANA, SMA 7. Ultrasound 8. Alcohol history Diagnostic evaluation 1. Drug history 2. AMA 3. P-ANCA 4. Ultrasound 5. MRCP/ERCP Hepatitic: °°ALT Mixed: ˛ALT, ˛AlkP Cholestatic: °°AlkP, °°gGT, ˛ALT Hepatitic: °°ALT Mixed: ˛ALT, ˛AlkP Cholestatic: °°AlkP, °°gGT, ˛ALT"}, {"id": "Pharmacology_Katzung_7019", "title": "Pharmacology_Katzung", "score": 0.010483184333759046, "content": "Her social history is significant for alcohol use (three to four glasses of wine/night). Her vital signs include the following: temperature 99.8°F, blood pressure 132/64 mm Hg, pulse 78 bpm, and respiratory rate 15/min. On physical examination, she had left upper abdominal tenderness with evidence of hepatomegaly and mild scleral icterus. Laboratory data revealed the following: alanine aminotransferase, 527 IU/L (normal 10–35 IU/L); aspartate aminotransferase, 425 IU/L (normal < 35 IU/L); and bilirubin, 2.9 mg/dL (normal 0.1–0.3 mg/dL). What medications do OTC cold and flu preparations typically contain? Which of the OTC medications might have contrib-uted to the patient’s current symptoms? KH, a 55-year-old woman, presents to the emergency department with nausea, vomiting, and complaints of new-onset flu symptoms over the past several days. Her past medical history is significant for allergic rhinitis and chronic lower back pain secondary to a work-related fall 2 years ago. Her"}, {"id": "article-23802_24", "title": "Jaundice -- History and Physical -- History", "score": 0.01034365574954335, "content": "Patients usually present with varying symptoms apart from yellowish discoloration of skin along with pruritus, thus providing clues to narrow down the etiology or can also be asymptomatic. A thorough questioning regarding the use of drugs, alcohol or other toxic substances, risk factors for hepatitis (travel, unsafe sexual practices), HIV status, personal or family history of any inherited disorders or hemolytic disorders is vital. Other important points include the duration of jaundice; and the presence of any coexisting signs and symptoms, like a joint ache, rash, myalgia, changes in urine and stool. [22] A history of arthralgias and myalgias before yellowing indicates hepatitis, either due to drugs or viral infections."}, {"id": "article-22784_30", "title": "Hepatitis -- History and Physical -- Viral Hepatitis", "score": 0.010161576538712991, "content": "Phase 2 (prodromal phase) - Patients in this phase usually present with anorexia, nausea, vomiting, malaise, pruritus, urticaria, arthralgias, and fatigue. Many times these patients are misdiagnosed as having gastroenteritis or viral infection. Phase 3 (icteric phase) - Patients in this phase present with dark-colored urine and pale-colored stool. Some patients develop jaundice and right upper quadrant pain with liver enlargement. Phase 4 (convalescent phase) - Patients typically start noticing the resolution of symptoms, and laboratory studies show liver enzymes returning to normal levels. [32]"}, {"id": "pubmed23n0735_17090", "title": "Fatal hepatitis E viral infection in pregnant women in Ghana: a case series.", "score": 0.009900990099009901, "content": "Viral infections during pregnancy can pose serious threats to mother and fetus from the time of conception to the time of delivery. These lead to congenital defects, spontaneous abortion and even death. The definitive diagnosis and management of pregnancy-related viral infections may be challenging especially in less resourced countries. We present clinical and laboratory responses to the diagnosis and management of three cases of fulminant hepatitis secondary to Hepatitis E viral infection in pregnancy.Case 1 was a 31-year-old Ghanaian woman who presented with a week's history of passing dark urine as well as yellowish discoloration of the eyes. She subsequently developed fulminant hepatitis secondary to Hepatitis E viral infection, spontaneously aborted at 24 weeks of gestation and later died.Case 2 was also a 31-year-old Ghanaian woman who was admitted with a four-day history of jaundice. She had low grade fever, but no history of abdominal pain, haematuria, pale stool or pruritus. She next developed fulminant hepatitis secondary to Hepatitis E viral infection. However, she did not miscarry but died at 28 weeks of gestation.Case 3 was a 17-year-old Ghanaian woman who was referred to the tertiary health facility on account of jaundice and anaemia. She had delivered a live male infant at maturity of 32 weeks but noticed she was jaundiced and had a presentation of active disease 3 days prior to delivery. The baby was icteric at birth and on evaluation, had elevated bilirubin (mixed type) with normal liver enzymes. Hepatitis E virus infection was confirmed in both mother and baby. However, the jaundice and the hepatomegaly resolved in mother and baby after 5 and 12 days respectively. To the best of our knowledge, these are the first documented cases of fatal fulminant hepatic failures resulting from HEV infection in Ghana."}, {"id": "pubmed23n0396_3498", "title": "[Dynamics of serum copper level in patients in the acute phase of hepatitis B and in early convalescence].", "score": 0.009900990099009901, "content": "Analysis of serum copper (Cu) level dynamics during the acute phase of hepatitis acute B and the early convalescence in compliance with gravity of the acute disease course. The study included 39 patients (12 men and 27 women), aged 18 to 76 years. They were hospitalised in the Department of Infectious Diseases of Medical Academy in Lublin because of the hepatitis acute B, without coexisting diseases. The diagnosis was based on the epidemiologic anamnesis, clinical symptoms, biochemical and serological examinations. The studied group was divided in respect to sex and the course of the disease as: light, medium-weighty and weighty. In all examined patients, the serum Cu level was determined according to the following scheme: at the first, tenth, twentieth and the last day of the hospitalisation and additionally one time at four weeks after discharging from the clinic. The serum Cu level was made by atomic absorption spectrometry (AAS) at the wave length of 324.8 nm. The received data were subjected to statistical analysis according to t-Student's test and in cases of significant differences according to the variants of c-Cochran and Cox's tests. According to the SI Unit Conversion Guide, the values 11.22 to 23.58 mumol/l were taken as the normal range. The values derived from the control group of 24 healthy persons (13 men and 11 women) aged 22 to 69 years. The significant increase of serum Cu level in comparison with the control values was found both in the acute phase of hepatitis B and the early convalescence. It could be observed a correlation between serum Cu level and the course of hepatitis viralis acuta B."}, {"id": "pubmed23n0972_7733", "title": "Spontaneous Cure of Acute Hepatitis C.", "score": 0.00980392156862745, "content": "The statistics proved that approximately 25% of the patients with acute HCV present with jaundice, and only 10-20% develop gastrointestinal symptoms. We present the case of a 58 year-old woman, with prior antecedents of arterial hypertension and diabetes mellitus since 25 years old, hypercholesterolemia and hypertriglyceridemia, psoriasis, epilepsy and depressive syndrome. She clinically presents asthenia, anorexia, itching, jaundice and choluria. The objective examination showed an orientated patient, without flapping, hemorrhagic dyscrasia or signs of chronic hepatic disease, with icteric mucosa and skin, abdominal pain, with hepatomegaly and splenomegaly. The laboratory tests have been compatible with acute hepatitis with colestatic pattern: AST/ALT 969/798 UI/ml, FA 796 UI/ml, GGT 2476 UI/ml, BT/BD 7.39/6.10, INR 0.9. The abdominal echography showed: hepatomegaly, regular borders, hepatic steatosis, splenomegaly without ascitic fluid. The viral serological tests revealed protection for hepatitis A ( IgM neg/IgG pos), negative for HVB infection (AgHBs neg, anti-HBc neg), negative for HVE and other viruses (CMV Herpes virus, Epstein Barr, HIV), positive antibodies for HCV and positive RNA VHC (164200 UI/ml), HCV genotype 3a, IL-28B CT, negative autoimmunity. The previous HCV tests were negative, sustaining the recent infection. We assumed an acute hepatitis C. The patient was symptomatically treated with hydroxyzine for the skin itch, with vitamin K for INR correction and she was closely monitored. She had good clinical and laboratorial evolution and she was discharged after one week, maintaining hepatology consultation. She spontaneously cleared HCV infection after 3 months, maintaining negative RNA VHC 6 months after infection. The patient has cured the HCV infection with no need for antiviral treatment."}, {"id": "pubmed23n0244_4563", "title": "[Finding and significance of copper protein complexes in liver cirrhoses (author's transl)].", "score": 0.00980392156862745, "content": "302 liver cirrhoses obtained from the post-mortem material 1970 to 1979 have been examined for the occurrence of the cuprous protein complexes by means of the orcein-staining according to Shikata. The part of the B-posthepatic cirrhoses (89%) could be simultaneously determined with the help of this method. Cuprous protein complexes have been found in bilious (100%), alcoholic (43.9%), etiologically uncertain (34.4%), and B-posthepatic (25,9%) liver cirrhoses. They prove an existing or experienced chronic cholestasis. The demonstration of copper has no principal significance for the etiologic classification of cirrhoses. In the bioptic diagnostics the demonstration of copper has a certain significance for the differentiation of the primary destructive cholangitis from chronically active hepatites of other genesis. The investigations incidentally revealed that HBsAg-containing hepatocytes could be identified by means of orcein in sections performed on archive material after destaining independent of the age of the sections and their primary staining."}, {"id": "wiki20220301en529_26162", "title": "Tenpō Tsūhō", "score": 0.009708737864077669, "content": "History The Tenpō Tsūhō came around a century after the introduction of the Hōei Tsūhō (Kyūjitai: 寳永通寳 ; Shinjitai: 宝永通宝) during the 5th year of the Hōei era (1708), which had a face value of 10 mon (while only containing 3 times as much copper as a 1 mon Kan'ei Tsūhō coin), but was discontinued shortly after it started circulating as it wasn't accepted for its nominal value."}, {"id": "pubmed23n0412_18897", "title": "Cases from the Osler Medical Service at Johns Hopkins University.", "score": 0.009615384615384616, "content": "A 37-year-old woman presented with increasing abdominal pain and jaundice. Six weeks before admission, she developed persistent diarrhea and jaundice of the skin. She also bruised easily, and her gums bled. In the subsequent weeks, her appetite decreased, she was fatigued, and she had nausea, vomiting, and abdominal distension. She had a history of drinking 1 quart of vodka every day for 20 years, with brief periods of abstinence; she stopped consuming alcohol 11 days before admission because it no longer provided symptomatic relief. Her past medical history was also notable for depression, including a suicide attempt 4 years earlier. She did not smoke, use illicit drugs, or have unprotected sexual intercourse. She had received no blood transfusions and had not traveled recently. She took no medications, except for occasional ibuprofen. On physical examination, she was thin and deeply jaundiced, and she trembled and responded slowly to questions. She was afebrile but tachypneic, and she had orthostatic hypotension. Her HEENT examination was notable for scleral and sublingual icterus, as well as crusted blood on her gums and teeth. The jugular veins were flat. The cardiac examination revealed tachycardia (heart rate, 103 beats per minute) without murmurs, rubs, or gallops. The abdomen was nontender and protuberant, with hypoactive bowel sounds; the spleen was not palpable, and there was no fluid wave or caput medusae. The liver percussed to 18 cm, with a smooth edge extending 10 cm below the costal margin. She had cutaneous telangiectases on her chest and bilateral palmar erythema. There was no peripheral edema. The neurologic examination was notable for asterixis. Her stool was guaiac positive. Laboratory studies revealed the following values: hematocrit, 21.2%; white blood cells, 17,310/mm(3); ammonia, 42 micromol/L; serum creatinine, 3.9 mg/dL; serum urea nitrogen, 70 mg/dL; albumin, 2.1 g/dL; total bilirubin, 26.8 mg/dL; alanine aminotransferase, 14 U/L; aspartate aminotransferase, 77 U/L; alkaline phosphatase, 138 U/L; prothrombin time, 103 seconds (international normalized ratio, 10.6); and urinary sodium, &lt;5 mg/dL. Urinalysis revealed an elevated specific gravity and numerous muddy granular casts. Hepatitis A, B, and C serologies were negative. On abdominal ultrasound examination, there was no ascites, and the liver was echogenic. The portal and hepatic veins were patent, and the hepatic arteries were normal. The spleen measured 14 cm. What is the diagnosis?"}, {"id": "article-22788_50", "title": "Hepatitis B -- Differential Diagnosis", "score": 0.009615384615384616, "content": "Wilson disease is a disease of excessive copper accumulation. It is associated with psychiatric disturbances due to copper accumulation in the basal ganglia. Kayser-Fleischer rings are pathognomonic for Wilson disease but are not completely sensitive (requires an expert ophthalmologist to confirm this finding). Laboratory evaluation that favors a diagnosis of Wilson disease includes low serum ceruloplasmin levels and elevated urinary copper, and if abnormal, requires further evaluation by a hepatologist. Alcoholic hepatitis Autoimmune hepatitis Cirrhosis Drug-induced liver injury Hemochromatosis Hepatitis A Hepatitis C Hepatitis D Hepatitis E Hepatocellular carcinoma Human immunodeficiency virus Wilson disease"}, {"id": "pubmed23n0840_2629", "title": "Acute Cholestatic Liver Injury From Hydralazine Intake.", "score": 0.009523809523809525, "content": "Hydralazine is a commonly used oral antihypertensive agent. We report a rare case of hydralazine-induced hepatotoxicity in the form of subacute hepatic necrosis. A 75-year-old African American woman presented with jaundice of 7-day duration. She was started on hydralazine 100 mg 3 times a day 10 weeks before presentation. On physical examination, scleral icterus was noted. Workup revealed elevated liver transaminases, alkaline phosphatase, and conjugated bilirubin. She had no history of liver disease, and liver function tests had been normal before starting hydralazine. Other etiologies, including viruses, common toxins, drugs, autoimmune, and copper-induced hepatitis, were excluded. Abdominal imaging studies did not show any evidence of intrahepatic or extrahepatic biliary ductal dilatation, and no pathologies were seen in the liver and pancreas. The patient's liver biopsy revealed extensive lobular hepatitis, significant necrosis, mixed inflammatory infiltrate, and no significant fibrosis, supporting a diagnosis of drug-induced liver injury. Hydralazine was immediately discontinued. She showed improvement of clinical and laboratory abnormalities within 5 days after discontinuation of hydralazine. To establish the diagnosis of hydralazine-induced liver injury, we used assessment tool outlined by the Council for International Organization of Medical Sciences (CIOMS) scale that led to \"high probable\" relationship. Although rare, clinically significant, and potentially life-threatening liver injury can result from use of hydralazine. Both clinical and histological presentations in our patient suggest acute liver injury. The hydralazine-induced hepatitis seems to be reversible as discontinuation of the drug improves clinical outcomes. We highly recommend monitoring of the liver function during hydralazine treatment. "}, {"id": "pubmed23n0930_24443", "title": "[Clinical and morphological correlations in occult hepatitis B].", "score": 0.009523809523809525, "content": "Occult hepatitis B (ОHB) characterized by the absence of blood HBsAg attracts the attention of specialists of different profiles; however, its clinical morphological aspects have not been practically studied. to estimate the proportion of OHB in the structure of fatal outcomes in chronic viral hepatitis (CVH) and to characterize its clinical course and structural changes on autopsy materials. A total of 455 autopsy cases of CVH were examined for its etiology in the S.P. Botkin Clinical Hospital of Infectious Diseases in 2014-2016. An in-depth prospective clinical analysis was made to investigate 28 cases of OHB in the stage of decompensated liver cirrhosis, which had subsequently culminated in death. The criteria of inclusion were history data and clinical symptoms of CVH in the detection of markers for hepatitis A, C, and D and HIV in serum HBcAb in the absence of HBsAg. HBsAbs were also determined. Along with the traditional morphological examination, immunohistochemistry (IHC) for HBsAg and HBcAg was carried out. There were 108 CVHB cases (23.7% of the total cases of CVH), including 77 OHB cases (71.3% of those of CVHB) while HBsAg was not determined. HBsAb-negative patients were more often observed to have clinical signs of jaundice (p&lt;0.05) and skin itching (p&lt;0.05). Dyspepsia and hemorrhagic manifestations prevailed in patients with HBsAb (more than 10 IU/l) (p&lt;0.05). All the cases were found to have characteristic morphological signs of CVH, including intranuclear inclusions and nuclear polymorphism in 10.7% of deaths. There was an IHC-positive reaction to VHB antigens in 28.6% of the patients and a doubtful reaction in 25.0%. Serum НВсAb may serve as a diagnostic marker for HBV infection. Clinical and morphological correlations enabled the authors to state that CVHB was present in all cases in the absence of serum HBsAg in the patients."}, {"id": "InternalMed_Harrison_23947", "title": "InternalMed_Harrison", "score": 0.009459924320605436, "content": "Diagnosing NAFLD requires demonstration of increased liver fat in the absence of hazardous levels of alcohol consumption. Thresholds for potentially dangerous alcohol ingestion have been set at more than one drink per day in women and two drinks per day in men based on epidemiologic evidence that the prevalence of serum aminotransferase elevations increases when alcohol consumption habitually exceeds these levels. In those studies, one drink was defined as having 10 g of ethanol and, thus, is equivalent to one can of beer, 4 ounces of wine, or 1.5 ounces (one shot) of distilled spirits. Other causes of liver fat accumulation (particularly exposure to certain drugs; Table 364-2) and liver injury (e.g., viral hepatitis, autoimmune liver disease, iron or copper overload, α1 antitrypsin deficiency) must also be excluded. Thus, establishing the diagnosis of NAFLD does not require invasive testing: it can be accomplished by history and physical examination, liver imaging (ultrasound is an"}, {"id": "pubmed23n0960_17714", "title": "Ginseng-Related Drug-Induced Liver Injury.", "score": 0.009433962264150943, "content": "Ginseng is commonly used as a medicinal herb for memory and concentration and general well-being. Drug-induced liver injury (DILI) is one of the most challenging disorders and trending events in the United States which are related to body building and weight loss supplements. Currently, herbal and dietary supplementation is the second most common cause of DILI. Here, we report on a 45-year-old healthy Chinese woman who presented with dull intermittent left upper quadrant abdomen pain for a month. Upon thorough history taking, she had been taking ginseng tea and supplementation for her menopausal symptoms for almost 3 months. Physical examination was unremarkable except mild tenderness in left upper quadrant of the abdomen. Liver function test showed aspartate transaminase (AST) 717 U/L, alanine transaminase (ALT) 343 U/L, total bilirubin 5 mg/dL, direct bilirubin 3.3 mg/dL, alkaline phosphatase 182 U/L, with international normalized ratio (INR) 1.2. Prior liver enzymes (6 months earlier) showed AST 21 U/L, ALT 18 U/L, total bilirubin 0.8 mg/dL, direct bilirubin 0.3 mg/dL, alkaline phosphatase 34 U/L, with INR 0.7. Viral serology for acute hepatitis B, C, E, cytomegalovirus, Epstein-Barr virus, and varicella zoster virus was negative. She was immune to hepatitis A. Her antinuclear antibody was positive. Her anti-Smith antibody, anti-smooth muscle antibody, HFE gene mutation, ceruloplasmin, alpha-1 antitrypsin serologies were within normal references. An abdomen sonogram showed fatty infiltration. Liver biopsy showed moderate to severe portal inflammation and marked lobular disarray. Portal and lobular inflammatory infiltrates consisted of a mixture of histiocytes, lymphocytes, plasma cells, eosinophils, and neutrophils with centrilobular necrosis and focal bridging necrosis, and necro-inflammation. After 6 weeks of follow-up, the patient improved physically, and the abdomen pain resolved. Ginseng has been widely used in the Chinese community as medicinal herb for a variety of conditions for decades. However, proper research has never been done regarding its pharmacokinetics, efficacy, and safety issues. In our case report, the idiosyncratic DILI resulted from ingestion of ginseng as herbal supplementation for premenopausal symptoms. Physicians should be aware of and suspect DILI in any patient with acute liver injury, and patients should be reminded that all medications and supplements have a potential to cause DILI."}, {"id": "article-23802_30", "title": "Jaundice -- Evaluation", "score": 0.009433962264150943, "content": "The results of the bilirubin, enzymes, and liver function tests will direct the diagnosis towards a hepatocellular or cholestatic cause and offer some idea of the duration and severity of the disease. Further evaluation can be conducted based on the initial assessment. Hepatocellular workup: viral serologies, autoimmune antibodies, serum ceruloplasmin, ferritin."}, {"id": "pubmed23n0975_8122", "title": "Cerebral Venous Sinus Thrombosis in Systemic Lupus Erythematosus.", "score": 0.009345794392523364, "content": "A 38-year-old woman presented with general weakness and vaginal bleeding. One month prior, she had been diagnosed with Evans syndrome (haemolytic anemia with positive Coombs test and thrombocytopenia) and was given oral steroid as maintenance therapy. Her serology examination was negative for hepatitis B, hepatitis C, and human immunodeficiency virus (HIV). Her obstetrical history was marked by miscarriage in second pregnancy and preeclampsia in third pregnancy. She used hormonal contraceptives until 5 months prior to admission. On physical examination, she had anemic conjunctiva and no organomegaly. Blood tests were significant for anemia (3.4 g/dl) and thrombocytopenia (28,000/µl). Her vaginal bleeding had ceased, however her platelet continued decreasing to 12,000/µl during first several days of hospitalization despite receiving platelet transfusion. On the tenth hospital day, she suddenly complained of severe headache and blurred vision. She had bilateral edema and erythema of palpebral, chemosis, decreased in visual acuity, and reduced ocular motility. Ear and nose examination were normal. Peripheral blood smear showed no blast. Prothrombine time (PT), INR, APTT tests were normal and D Dimer was slightly increased (3.3 mg/l; NV ≤0.5 mg/l). Urine examination revealed proteinuria with 24 hour urine protein was 1,863 mg (NV &lt;150 mg/day). We assessed her as cavernous sinus thrombosis and treated her empirically with intravenous broad-spectrum antibiotics, morphine drip. Either digital subtraction angiography or anticoagulant was deferred due to low platelet. Further examination revealed positive for ANA, anti-SSA, and diagnosis of SLE was established. Anticardiolipin antibodies of IgG and IgM and anti-beta2 glycoprotein antibodies of IgM and IgG tests were non reactive. Methylprednisolone pulse therapy (1g/day) was given for 3 consecutive days, and then tapered to oral methylprednisolone. She additionally received azathioprine 50 mg tab BID. Meanwhile her clinical symptoms alleviated and platelet count was increased, brain MRI and MR venography finally performed suggesting cerebral venous sinus thrombosis. She got additional oral anticoagulant rivaroxaban 15 mg tab BID and eventually discharged. Cerebral venous sinus thrombosis may be the presenting symptoms or occur concomitantly within the onset of SLE. Our patient had SLE, meeting 4 of the Systemic Lupus International Collaborating Clinic classification criteria (hemolytic anemia, thrombocytopenia, renal involvement, and positive for ANA test). Vasculitis due to endothelial cell injury mediated by immune-complex deposition is proposed to be the pathogenesis of CVST in SLE. Hypercoagulable state could be other etiology factor. Antiphospholipid antibodies were absent in our case as reported in some cases, emphasizing vasculitis as the underlying mechanism. Treatment of CVST in SLE consisting of anticoagulant, steroid, and immunosuppressant. This case elicits intriguing problem: CVST and thrombocytopenia. Anticoagulant treatment is proposed as the cornerstone treatment for CVST, however it was deferred due to risk of bleeding in thrombocytopenia. Steroid plays role in treatment of CVST in SLE, owing to its anti-inflammatory property. As shown in previous cases, the patient had remarkable response to high dose steroid treatment and eventually got anticoagulant after her platelet had increased. In summary, prompt diagnosis and treatment of CVST are important for a favorable prognosis."}, {"id": "pubmed23n0410_9415", "title": "[Rare, but important chronic liver diseases].", "score": 0.009345794392523364, "content": "The presence of steatosis and inflammatory infiltrate in liver biopsies is essential for the diagnosis of non-alcoholic steatohepatitis (NASH). These findings are similar to those with alcoholic liver disease. However, in the NASH-situation alcohol doesn't play an important role. Risk factors for the development of NASH are obesity and diabetes. Most of the patients are clinically asymptomatic. This means, that a diagnosis of NASH is a diagnosis of exclusion: Viral induced, autoimmune, metabolic and toxic liver disease have to be excluded. The disease has a benign clinical course. The risk of cirrhosis is low. So far, there is no established treatment. Preliminary reports suggest a positive effect of weight-loss and ursodeoxycholic acid. Wilson's disease, a copper storage disorder, in which biliary copper excretion is reduced, is inherited as an autosomal recessive trait. Most patients with Wilson disease become symptomatic between the ages of 6 and 15. In about 90% of patients serum ceruloplasmin levels and serum copper concentrations are reduced. Copper excreation is increased. Histologic examination of liver biopsy specimens reveals fatty infiltration, Mallory bodies and ballooned glycogen nuclei, abnormalities which are also found in alcoholic liver disease. The definitive diagnostic parameter is the quantitative determination of liver copper content (&gt; 250 micrograms/g dryweight). Untreated Wilson disease is always fatal. Lifelong treatment with anti-copper drugs are essential, D-penicillamine being the firstline therapy. Hereditary hemochromatosis (HH) is an iron overload disease inherited as an autosomal recessive trait. The frequency of the disease is high. The first symptoms usually can be found at the age of 20-50 years. Arthralgia develops in up to 50% of the patients. Many organs are involved, most often the liver. The organ is usually enlarged, transaminases are always moderately elevated. Laboratory findings disclose a marked elevation in serum ferritin and transferrin saturation. More than 80% of HH-patients are homozygous for the C282Y-mutation in the HFE-gene. The firstline treatment of HH is phlebotomy. Treatment is lifelong. When serum ferritin drops below 50 micrograms/l, the frequency of phlebotomy should be reduced (4-12 per year). If the patient already has cirrhosis, the risk of HCC is very high."}, {"id": "pubmed23n0973_23808", "title": "Paraneoplastic Encephalopathy in a Patient With Metastatic Lung Cancer: A Case Study.", "score": 0.009259259259259259, "content": "<bCASE STUDY</b RS, a 36-year-old female, presented to the emergency department (ED) of a large academic medical center upon the advice of her primary care provider because of 3 weeks of progressive mental status changes, weakness, and decreased oral intake. According to her husband, RS was diagnosed with stage IIIA large cell lung cancer 8 months earlier and was treated with concurrent chemotherapy (carboplatin, pemetrexed, and bevacizumab) and radiation therapy that was completed 4 months prior to admission. No other specific information about her treatment or outside health records was available. According to her husband, RS had been in her usual state of health until approximately 3 weeks prior, when she began having significant mental status changes. She first exhibited some difficulty finding words and later was noted to be putting food in a coffee maker. This spontaneously resolved after approximately 1 week; however, she rapidly developed slurred speech and began to make nonsensical statements. These manifestations also slowly improved but were followed by worsening speech deficit, difficulty walking, and impaired balance. During one of these episodes, she had an occurrence of incontinence. Her husband also noted an incident where her \"eyes were beating back and forth and the left side of her face was twitching.\" RS also had periods (according to her husband) where she \"did not seem to be interacting with her environment.\" These progressively worsened during the last week, and she completely stopped walking and talking 2 days prior to coming to the ED. According to her husband, RS had rheumatoid arthritis and no surgical history. Her family history was unknown except that RS's mother had \"seizures.\" RS had reportedly not used tobacco, alcohol, or drugs, and she was sexually active with her husband. Home medications included transdermal fentanyl 12 μg/hr patch changed every 72 hours; oxycodone-acetaminophen tablets 5-325 mg, two every 4 hours as needed for pain; prednisone 10 mg, one tablet daily; and megestrol 40 mg/mL suspension, 20 mL once daily for appetite stimulation. RS was admitted to an inpatient medical oncology service and evaluated by the oncology advanced practitioner (AP) on her second inpatient day. Upon exam, RS was nonverbal except for moaning in response to painful stimuli and to her sister's voice. Her vital signs were normal. She appeared ill but well-nourished, and she was mildly diaphoretic. Neurologic examination revealed that her pupils were slightly sluggish but equal, round, and reactive to light. Extraocular muscle movements were intact, but she did not move her eyes in response to commands. She tracked the AP and family members around the room with her eyes. Cranial nerve examination was intact with the exception of cranial nerves IX, X, and XI, which were difficult to examine given her inability to cooperate and open her mouth. Motor examination revealed increased tone throughout and intermittent, inconsistent resistance to passive movement. She was seen to move all four extremities spontaneously although not in response to commands. Deep tendon reflexes were intact and equal in all extremities. Examination of other body systems was as follows: there was dry, peeling skin on her lips, but her mucous membranes were moist and free of erythema or lesions. Her lungs were clear to auscultation bilaterally. Her heart rate and rhythm were regular, there were no murmurs, rubs, or gallops, and distal pulses were intact. Her abdomen was nondistended with normally active bowel sounds in all four quadrants. Her abdomen was soft, nontender to palpation, and without palpable masses. There was no peripheral discoloration, temperature changes, or edema, and examination of her skin was benign. <bWorkup</b On admission to the emergency department, serum laboratory studies were unrevealing for any potential causes of encephalopathy. Kidney and liver function were normal, making diagnoses of uremic and hepatic encephalopathies less likely. Cultures of the urine and blood were negative. Samples of cerebrospinal fluid (CSF) were obtained via lumbar puncture and were unrevealing for any abnormalities. Computed tomography (CT) of the head without contrast was negative for any acute intracranial process. Ultrasound of the right upper quadrant revealed a single, nonspecific, hypoechoic hepatic lesion. Computed tomography scans of the chest, abdomen, and pelvis demonstrated the primary malignancy in the upper lobe of the left lung, as well as possible metastatic disease within the left lung, right lung, and liver, and widespread osseous metastatic disease. Magnetic resonance imaging (MRI) of the brain performed 1 day after admission demonstrated numerous scattered punctate foci of enhancement throughout the supratentorial and infratentorial brain parenchyma, measuring at most 3 to 4 millimeters in diameter. There was no significant mass effect or midline shift. A paraneoplastic panel was sent to an outside laboratory and returned positive for antivoltage-gated potassium channel (VGKC) autoantibodies. <bDifferential Diagnosis</b Clinically, RS was exhibiting signs of encephalopathy, a broad term that indicates general brain dysfunction, the hallmark of which is altered mental status. Diagnosing encephalopathy is challenging, as many differential diagnoses must be considered. The clinician must consider metabolic derangements, toxic and infectious etiologies, psychiatric disorders, and less commonly, prion disorders and progressive dementia. Cultures of RS's blood and urine as well as other specialized endocrine tests were negative, decreasing the likelihood of a metabolic or infectious cause for her presentation. The abnormalities on her brain MRI were reviewed by a neuro-oncology team, who felt that the faint, nondescript nature of the visualized lesions was not suspicious for metastatic disease. Sequelae of seizures was also considered by neuro-oncology but dismissed given a grossly normal prolonged electroencephalogram. Some encephalopathies are caused by autoimmune or inflammatory mechanisms, which are confirmed by the presence of autoantibody markers and/or clear response to immunomodulatory treatment (Vernino, Geschwind, &amp; Boeve, 2007). These types of encephalopathies have been seen in patients with cancer and have thus been termed paraneoplastic. The presence of anti-VGKC antibodies on RS's paraneoplastic panel directed the inpatient medical oncology team toward a paraneoplastic neurologic disorder (PND) as the most likely diagnosis."}, {"id": "pubmed23n0325_8334", "title": "[Severe hemolytic anemia with tear drop red cells as initial manifestation of Wilson's disease].", "score": 0.009259259259259259, "content": "A 16-year-old girl was admitted for a detailed examination of hemolytic anemia in November 1995. Initial laboratory findings included a total bilirubin concentration of 1.46 mg/dl, hemoglobin of 9.1 g/dl, and a reticulocyte count of 89/1000 percent. The plasma haptoglobin concentration was below 10 mg/dl. A blood smear showed many dacryocytes and a few echinocytes and codocytes. GOT was 71 IU/l; GPT, 44 IU/l; and LDH, 812 IU/l; the results of a hepaplastin test were 45% of normal. On further investigation, the level of serum ceruloplasmin was found to be 4 mg/dl, and of serum copper, 43 micrograms/dl. Urinary copper excretion was markedly increased, at 345 micrograms per day. Slit-lamp examination of both corneas revealed obvious Kayser-Fleischer rings. A liver biopsy sample showed fibrosis histologically and an elevated copper concentration of 535 micrograms/g dry weight and 183 micrograms/g wet weight. In family studies, the patient's asymptomatic 5-year-old sister was observed to have metabolic abnormalities consistent with Wilson's disease. These findings suggested that the patient's hemolytic anemia with red cell deformities was due to abnormal copper metabolism associated with Wilson's disease."}, {"id": "pubmed23n0548_5253", "title": "Subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome successfully treated with cyclosporin A.", "score": 0.009174311926605505, "content": "A 17-year-old girl previously in good health presented with a 2-month history of recurrent, high-grade fever; general fatigue; anorexia; a 10-kg weight loss; and multiple, painful, reddish skin lesions on the lower abdomen. Some lesions were ulcerated, with an oily yellowish brown discharge. A systemic review was unremarkable other than bleeding from the nose. Her medical and family histories were unremarkable. On examination, the patient was pale, jaundiced, and febrile (temperature of 39 degrees C). She had enlarged lymph nodes in the axillary and inguinal areas. There was moderate hepatosplenomegaly. Local skin examination revealed multiple erythematous, tender, and firm subcutaneous nodules of variable size (1-2 cm) on the lower abdomen. Some nodules were ulcerated, with oily yellowish brown discharge and overlying ecchymosis (Figures 1 and 2). Mucous membranes were free of lesions. Laboratory investigations showed pancytopenia, an elevated erythrocyte sedimentation rate (&gt;80 mm/h), normal renal function tests, abnormal hepatic function tests (alanine aminotransferase 172 U/L, aspartate aminotransferase 229 U/L, alkaline phosphatase 725 U/L, and total bilirubin 100 mmol/L [normal range 0-18 mmol/L]), conjugated bilirubin 45 mmol/L (normal range 0-5 mmol/L), and high triglycerides 855 mg/dL (normal range 20-200 mg/dL). Prolonged prothrombin time, 26 seconds (normal range 13-16 seconds); prolonged activated partial thromboplastin time, 61 seconds (normal range 26-38 seconds); positive disseminated intravascular coagulation studies evidenced by low fibrinogen, 74 mg/dL (normal range 160-350 mg/dL); and positive fibrinogen degradation products were also noted. Throat, midstream urine, and blood culture results were negative. Serologic tests for syphilis, HIV, and hepatitis B and C viruses were negative. Epstein-Barr virus and cytomegalovirus serologic values revealed evidence of past infection. Tuberculin and Coombs tests were negative. The alpha1-antitrypsin level was normal. Antinuclear and anti-smith antibodies, rheumatoid factor, and cryoglobulins were negative. CT showed enlarged lymph nodes in the axillary and inguinal areas, bilateral small pleural effusion, moderate hepatosplenomegaly, severe fatty infiltration of the liver, and thickening of lower abdominal subcutaneous tissue. A liver biopsy showed steatohepatitis. Bone marrow aspirate and trephine were normal. A deep punch biopsy of a nodule from the right lower abdomen revealed lobular panniculitis with atypical lymphocytes and large macrophages with cytophagocytosis (\"beanbag\" cells) (Figures 3 and 4). Immunohistochemistry showed that these atypical cells were positive for CD3, CD8, granzyme B, and perforin, and negative for CD56. T-cell gene rearrangement studies on skin lesions revealed a monoclonal T-cell receptor (gamma-chain) gene rearrangement, supporting the diagnosis of subcutaneous panniculitis-like T-cell lymphoma. On presentation, the initial treatment included 6 U of fresh frozen plasma, 2 U of packed red blood cells, and 2 g IV fibrinogen for 3 consecutive days. The patient was started on prednisolone 60 mg orally once daily and cyclosporine A 5 mg/kg/d orally in two divided doses. The fever and other systemic symptoms and skin lesions resolved within 2 weeks after the treatment. The prednisolone dose was tapered gradually, and a maintenance dose of cyclosporine A was continued. The patient's condition remained in remission at 12-month follow-up; there was no evidence of clinical relapse."}, {"id": "pubmed23n1125_16823", "title": "Gastric Antral Vascular Ectasia as the First Presentation of Primary Biliary Cholangitis.", "score": 0.009174311926605505, "content": "Primary biliary cholangitis (PBC), a chronic, autoimmune, cholestatic disease, typically occurs in elderly women and commonly presents with pruritus, fatigue, and cholestasis and its complications. Gastric antral vascular ectasia (GAVE), an uncommon cause of upper gastrointestinal bleeding, leading to transfusion-dependent chronic iron deficiency anemia, as the first presentation of PBC is unusual. We present the case of an elderly female with recurrent melena and transfusion-dependent anemia for a year without any history of jaundice, ascites, or hepatic encephalopathy. Investigations revealed iron-deficiency anemia, elevated transaminases, alkaline phosphatase (ALP), coarse liver, splenomegaly, and portal vein dilatation on ultrasound. An endoscopic evaluation revealed erythematous linear stripes in the antrum suggestive of GAVE, without esophageal or gastric varices. FibroScan (Echosens, Paris, France) revealed advanced F3 fibrosis. Further etiological workup showed positive antinuclear and antimitochondrial antibodies, elevated IgM levels, and negative viral markers (hepatitis B, C, A, and E). Clinically significant portal hypertension was revealed by the hepatic venous pressure gradient (HVPG), while transjugular liver biopsy (TJLB) revealed lymphocytic infiltration of bile duct epithelium with the destruction of small and medium-sized bile ductules. Iron supplementation, low-dose ursodeoxycholic acid, and argon plasma coagulation were used to treat the patient. At the three-month follow-up, no melena was reported and her hemoglobin and liver function tests remained normal. Patients with PBC presenting with GAVE and recurrent melena as a presenting symptom are rarely reported. An awareness of this presentation is important for its early diagnosis and effective treatment."}, {"id": "pubmed23n0947_25111", "title": "Pharmacogenomics of drug-induced liver injury (DILI): Molecular biology to clinical applications.", "score": 0.00909090909090909, "content": "A 21-year old woman was admitted to hospital with a two-week history of painless jaundice, fatigue and anorexia having previously been fit and well. One month prior to presentation, the patient had taken a five-day course of amoxicillin-clavulanic acid for an infected skin cyst. Otherwise, she was only on the oral contraceptive pill and reported minimal alcohol intake. On examination, she was deeply jaundiced, but alert and oriented with no asterixis. She had no stigmata of chronic liver disease, but hepatomegaly extending 3 cm from below the right subcostal margin was evident. Investigations showed: white cell count 13.4 × 10<sup9</sup/L (normal 3.6-9.3), haemoglobin 11.8 g/dl (normal 11-15), platelet count 356 × 10<sup9</sup/L (normal 170-420), sodium 138 mmol/L (normal 134-144), potassium 3.5 mmol/L (normal 3.5-5.0), creatinine 32 µmol/L (normal 40-75), albumin 30 g/L (normal 35-48), alanine aminotransferase 707 IU/L (normal 15-54), alkaline phosphatase 151 IU/L (normal 30-130), bilirubin 384 µmol/L (normal 7-31) and prothrombin time 27.2 s (normal 11.7-14). Screening for hepatitis A, B, C, E, Epstein-Barr virus, cytomegalovirus and autoimmune hepatitis was negative. Tests for anti-smooth muscle, antinuclear, and anti-liver-kidney microsomal-1 antibodies were negative; immunoglobulin levels and ceruloplasmin levels were normal. Liver ultrasonography demonstrated a liver of normal contour with no biliary dilatation, a normal spleen size and patent vessels. Liver biopsy revealed severe portal interface hepatitis with lobular inflammation and scant plasma cells. Her clinical condition deteriorated in the following days with prothrombin time and bilirubin rising to 56.6 s and 470 µmol/L, respectively. At follow-up after 11 days, her alanine aminotransferase level was 1,931 IU/L. She developed grade 2 hepatic encephalopathy 14 days after presentation, and was listed for a super-urgent liver transplant. Human leucocyte antigen (HLA) typing was performed as a part of preparatory investigations and showed the patient carried the HLA haplotype HLA-DRB1∗15:02-DQB1∗06:01. Following orthotopic transplantation of a deceased donor graft her explant histology revealed severe ongoing hepatitis with multi-acinar necrosis (Fig. 1A and B). This case raised a number of important questions about the diagnosis of drug-induced liver injury and tools available for clinicians to make the best decisions for patient care: In this Grand Rounds article, we will explore these questions, describing the pathophysiology, diagnostic and prognostic biomarkers, and clinical management of drug-induced liver injury. We will also discuss ongoing areas of uncertainty."}, {"id": "InternalMed_Harrison_29041", "title": "InternalMed_Harrison", "score": 0.00909090909090909, "content": "TAblE 428-2 REPRESEnTATivE iRon vAluES in noRmAl SubJECTS, PATiEnTS wiTH HEmoCHRomAToSiS, AnD PATiEnTS wiTH AlCoHoliC livER DiSEASE Adult first-degree relative of patient with HH Subjects with unexplained liver disease Individual with suggestive symptoms (see text) Transferrin saturation and serum ferritin* TS <45% SF <300 TS ˜45% and/or SF >300 °gL Reassure, possibly retest later HFE Genotype PhlebotomyNormal Counsel and consider non-HFE hemochromatosis Serum ferritin – 300–1000 °g/L LFT normal Serum ferritin > 1000 °g/L and/or LFT abnormal Serum ferritin <300 °g/L LFT normal Observe retest in 1–2 years C282Y Homozygote C282Y/H63D (Compound Heterozygote) Confirmed iron overload *For convenience both genotype and phenotype (iron tests) can be performed together at a single visit in first-degree relatives. Liver biopsy No iron overload Investigate and treat as appropriate"}, {"id": "pubmed23n0777_87", "title": "Acute cytomegalovirus hepatitis in an immunocompetent host.", "score": 0.009009009009009009, "content": "A 52-year-old woman presented with a 1-week history of recurrent fevers and joint pains accompanied by abdominal and low back discomfort. She has a history of hypoparathyroidism and is on calcium supplements. Physical examination revealed fever and tachycardia. The rest of the examination was normal. Laboratory tests showed newly increased transaminase activity. Serum bilirubin and prothrombin time were normal. She was admitted for evaluation of acute hepatitis. Serology for hepatitis A, B, C and HIV were negative. Her serum acetaminophen and alcohol were undetected. Abdominal imaging was normal. Cultures were sterile. Additional tests for uncommon viral hepatitis included herpes simplex virus, cytomegalovirus and Epstein-Barr virus. Liver biopsy revealed non-specific inflammation. Subsequently, cytomegalovirus serology showed an IgM positive and negative IgG titre. Cytomegalovirus DNA qualitative PCR was also positive. No antiviral medication was given. She continued to have intermittent daily fever but reported no associated symptoms. She was discharged 9 days after admission in stable condition per her request with the advice to follow-up in the clinic in 1 week. Her serum hepatic profile returned to normal and she reported no more episodes of fever. Repeated titres of cytomegalovirus serology showed seroconversion. "}, {"id": "pubmed23n0074_3744", "title": "[Primary biliary cirrhosis--analysis of clinical material].", "score": 0.009009009009009009, "content": "The analysis is presented of 67 patients with primary biliary cirrhosis treated in the years 1971-1988. The group consisted of 60 women (89.5%) and seven men (10.5%). Presenting symptoms were mostly itching (66%) and jaundice (12%). The time between the onset of the first symptoms and the diagnosis was three years, on the average. Autoantibodies to mitochondria were present in 86% of patients. In 27% of cases markers of HB virus infection were found. Cholelithiasis was present in 19 patients (28%). Primary biliary cirrhosis was diagnosed with delay. Third degree of histological changes was observed in 38.8% of cases and in 18% of patients it was already fourth degree. Eighteen patients (27%) died after six years, on the average, from the onset of symptoms. The direct causes of death were, most frequently, liver failure and haemorrhage from oesophageal varices."}, {"id": "pubmed23n0327_11192", "title": "[Manganese superoxide dismutase-inhibiting autoantibodies in cholestatic Epstein-Barr viral hepatitis].", "score": 0.008928571428571428, "content": "A 21-year-old woman reported no serious previous illness. For 3 days before admission she had a fever, headache and joint pains. She had become progressively more jaundiced. Physical examination was normal except for enlarged liver and spleen, swollen lymph nodes and facial oedema. GOT (30 U/l), GPT (33 U/l) and alkaline phosphatase (172 U/l) were slightly elevated. Serum bilirubin was raised to 12.4 mg/dl. The total white blood cell count was normal, but there were 45% atypical lymphocytes (activated T lymphocytes). Abdominal sonography and endoscopic retrograde cholangiopancreatography were unremarkable. Serology for hepatitis A, B and C as well as for antimitochondrial antibodies was negative, but there were specific IgM (1:640) and IgG antibodies (1:80) against Epstein-Barr virus (EBV) capsid antigen in the immunofluorescence test. The EBV infection (infectious mononucleosis) was complicated by cholestatic hepatitis. High concentrations (1832 Göttingen units/ml) of enzyme-inhibiting autoantibodies against the antioxidative enzyme manganese-superoxide dismutase (MSD) were demonstrated. The autoantibodies reduced the antioxidative action of the enzyme by more than 70% and favoured the oxidative cell damage in vitro. After bed-rest for one week without further treatment the symptoms improved and the abnormal laboratory values, including the autoantibodies against MSD, regressed. Autoantibodies against MSD are formed during an acute infection with EBV. Their enzyme-inhibiting action promotes abnormalities of oxidative cell function and may thus be the cause of cholestatic hepatitis in this infection."}, {"id": "pubmed23n0338_15316", "title": "[The characteristics of the course of viral hepatitis C].", "score": 0.008928571428571428, "content": "Time-related course and results of examination were studied in patients with viral hepatitis C, having gotten infected through blood. Based on the comparison of similar indicators in patients with viral hepatitis B particular features were revealed of the clinical course of viral hepatitis C. These are as follows: short-in-duration prejaundice period, in other instances there is no prejaundice period at all, subfebrile states set in quite often; among other features are dyspepsia-like events together with signs of a damage to pancreas. There has been noted a slow dynamics of the icteric period reversibility, with biochemical indicators showing the same tendency."}, {"id": "pubmed23n0620_20686", "title": "Idiopathic autoimmune hemolytic anemia due to lecithin overdose: a case report.", "score": 0.008849557522123894, "content": "Idiopathic Autoimmune Hemolytic Anemia is a potentially fatal condition which requires prompt and potent treatment. Diagnosis of idiopathic autoimmune hemolytic anemia requires both serologic evidence of autoantibody presence and hemolysis. Although most of the times it is considered idiopathic, several underlying causes have been identified, like autoimmune and connective tissue diseases, viral infections, drugs or hyper function of the immune system. To our knowledge, this is the first case in the international literature describing lecithin-induced autoimmune hemolytic anemia. This case report is to highlight a rare but dangerous adverse reaction to overdose of lecithin. A 38 year old white female from Greece, presented to our emergency room with progressive fatigue over a period of ten days and icteric discoloration of her skin and conjunctiva. The patient had been taking lecithin supplements (1200 mg, 3 capsules a day) over a period of ten days for weight loss. She reports that the last 3 days, prior to the examination, she took 5 capsules/day, so that the supplement would take effect more rapidly. Her past medical, social and family history showed no disturbance. Relatives of the patient were requested to submit any blood-tests taken over a period of 20 days prior to the onset of symptoms caused by Lecithin. All tests proved that all functions were within normal scale. Her physical examination revealed pallor and jaundice without palpable hepatosplenomegaly. Blood biochemistry tests showed total bilirubin 7.5 mg/dl, with indirect bilirubin 6.4 mg/dl and complete blood count showed hemoglobin 7.6 g/dl with blood levels 21.4%. In every case of idiopathic autoimmune hemolytic anemia the administration of pharmaceutical substances should always be examined, except for the standard reasons that cause it. In this case the cause of hemolysis was attributed to the excessive intake of lecithin capsules for the loss of body weight. It is important that clinicians and immunologists are aware of this adverse effect."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": true, "char_ranges": [[645, 813]], "word_ranges": [[102, 126]], "text": "Temporomandibular ankylosis is not considered because, although relatively close to the orbital cavity, it is not part of the orbito-malar complex (option 1 discarded)."}, "2": {"exist": true, "char_ranges": [[814, 965]], "word_ranges": [[126, 149]], "text": "Involvement of the maxilla can lead to dental malocclusion, but usually occurs in fractures located lower than the orbital cavity (option 2 discarded)."}, "3": {"exist": true, "char_ranges": [[966, 1160]], "word_ranges": [[149, 179]], "text": "Naso-ethmoidal fractures are included in midface fractures, but the bones of the nose are located more anteriorly to the medial orbital rim, and therefore outside the orbit (option 3 discarded)."}, "4": {"exist": true, "char_ranges": [[130, 644]], "word_ranges": [[23, 102]], "text": "Assuming therefore that we are asked which is one of the most frequent complications of the floor of the orbit, two complications should always be highlighted that may indicate surgical treatment, even urgent: diplopia, due to dislocation of the inferior rectus muscle to the underlying maxillary sinus (and even its entrapment); and enophthalmos, which may cause other associated complications in the medium and long term, such as superior palpebral pseudo-ptosis due to loss of orbital volume (option 4 correct)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Fractures of the midface in the orbito-malar region may involve the floor and, to a lesser extent, the lateral wall of the orbit. Assuming therefore that we are asked which is one of the most frequent complications of the floor of the orbit, two complications should always be highlighted that may indicate surgical treatment, even urgent: diplopia, due to dislocation of the inferior rectus muscle to the underlying maxillary sinus (and even its entrapment); and enophthalmos, which may cause other associated complications in the medium and long term, such as superior palpebral pseudo-ptosis due to loss of orbital volume (option 4 correct). Temporomandibular ankylosis is not considered because, although relatively close to the orbital cavity, it is not part of the orbito-malar complex (option 1 discarded). Involvement of the maxilla can lead to dental malocclusion, but usually occurs in fractures located lower than the orbital cavity (option 2 discarded). Naso-ethmoidal fractures are included in midface fractures, but the bones of the nose are located more anteriorly to the medial orbital rim, and therefore outside the orbit (option 3 discarded).", "full_answer_no_ref": "Fractures of the midface in the orbito-malar region may involve the floor and, to a lesser extent, the lateral wall of the orbit. Assuming therefore that we are asked which is one of the most frequent complications of the floor of the orbit, two complications should always be highlighted that may indicate surgical treatment, even urgent: diplopia, due to dislocation of the inferior rectus muscle to the underlying maxillary sinus (and even its entrapment); and enophthalmos, which may cause other associated complications in the medium and long term, such as superior palpebral pseudo-ptosis due to loss of orbital volume ([HIDDEN]). Temporomandibular ankylosis is not considered because, although relatively close to the orbital cavity, it is not part of the orbito-malar complex ([HIDDEN]). Involvement of the maxilla can lead to dental malocclusion, but usually occurs in fractures located lower than the orbital cavity ([HIDDEN]). Naso-ethmoidal fractures are included in midface fractures, but the bones of the nose are located more anteriorly to the medial orbital rim, and therefore outside the orbit ([HIDDEN]).", "full_question": "20-year-old patient who comes to the emergency department after suffering a bicycle accident with facial trauma. A cranial CT scan was performed showing a fracture of the middle third of the face involving the orbito-malar region. One of the most frequent complications of this type of fracture is:", "id": 621, "lang": "en", "options": {"1": "Temporomandibular ankylosis.", "2": "Dental malocclusion.", "3": "Naso-ethmoidal pseudoarthrosis.", "4": "Enophthalmos.", "5": NaN}, "question_id_specific": 60, "type": "OPHTHALMOLOGY (ECTOPIC)", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0584_7976", "title": "Panfacial fractures: analysis of 33 cases treated late.", "score": 0.01951265943270512, "content": "The aim of this retrospective study was to analyze the characteristics of delayed panfacial fractures and evaluate treatment results. Thirty-three patients with delayed panfacial fractures were treated in the Maxillofacial Trauma Center of Peking University, School and Hospital of Stomatology between 1998 and 2004. Each patient was examined by computed tomography (CT) scans before operation. For those who had no severe opening restriction, dental impressions were taken to fabricate dental casts. For those with severely comminuted fractures, 3-dimensional (3D) models of the facial skeleton were used. Re-establishing the continuity of the mandible was the first step and then used as a platform to reconstruct the maxillary fractures via maxillomandibular fixation after Le Fort I osteotomy. The third step was to restore the mid- and upper-facial width and projection by coronal approach to expose the zygomatic complex and frontal bone/sinus and/or naso-orbito-ethmoid (NOE) fractures. There were 3 types of mandibular fractures that affected the treatment plan: 1) type I, mandibular body/symphysis fracture(s) (17/33, 51.52%); 2) type II, mandibular angle and/or condylar fracture(s) (6/33, 18.18%); and 3) type III, both mandibular body/symphysis and angle/condylar fractures (10/33, 30.30%). Fourteen cases were associated with NOE fractures (42.42%) and 3 cases had frontal sinus fractures (9.1%). Twelve cases had enophthalmos (36.36%) and 3 lost 1 eyeball. The order of treatment was dependent on the mandibular fracture type. For type I fractures, reconstructing the mandibular arch was the first step. For type II fractures, repairing the angle, ascending rami, and condylar areas was the first step. For type III fractures, when both mandibular height and arch were disrupted, freeing the malunited angle or condyle was the first step before restoring the mandibular arch form. Reconstruction of the mandibular height and projection was then carried out. For all 3 types, the second step was to restore the mid- and upper facial width and projection by reducing the zygomatic complex and frontal bone/sinus or NOE fractures. Maxillary fixation across the Le Fort I level was the last step. Le Fort I osteotomy was used for all 33 cases. Bone grafts and soft tissue suspension also were used. Twenty-one cases (63.64%) had good results, 7 (21.21%) cases were acceptable, and 5 (15.15%) were not good. There were 7 cases (21.21%) that still had soft tissue problems that needed secondary operations. Reconstruction of the mandible first with Le Fort I osteotomy is a good way to treat delayed panfacial fractures. Computed tomography and 3D CT, model surgery, and occasionally 3D models are necessary aids for diagnosis and treatment. Soft tissue problems, including lacerations and asymmetries, were often the factors that caused an unfavorable outcome."}, {"id": "pubmed23n0794_2527", "title": "Etiology, incidence and patterns of mid-face fractures and associated ocular injuries.", "score": 0.019419306184012067, "content": "A prospective study on mid-face fractures was carried out in the Department of Oral and Maxillofacial Surgery at College of Dentistry, Indore, from August 2007 to September 2009 to analyze etiology, incidence and patterns of midface fractures and associated ocular injuries. Two hundred patients were included in this study, amongst those who reported to the Department of OMFS, College of Dentistry, Indore. After confirmed diagnosis of mid face fracture all the patients were stratified according to age, sex, cause of the accident, influence of alcohol, location, type of fractures and associated ocular injuries. The study included 200 patients with a mean age of 29.6 years. The most frequently injured patients belonged to the 21-30 year-old age group. The male predilection was 76 %. Road traffic accident was the most common causative factor (64 %), followed by assault (21 %), cases of fall (9.5 %) and other causes (5.5 %). The most common fracture in this study was found to be zygomatic complex fractures (62.5 %) (more in the age group of 21-30 years). This was followed by Lefort II fractures (23 %), multiple fractures (10 %) and Lefort I fractures (6 %), Lefort III fractures (4.5 %) and Naso-ethmoidal fractures (4 %) in descending order. 84.5 % subjects were having ocular involvement. Subconjunctival hemorrhage was present mostly in 83.5 % followed by remaining as corneal injury 15 %, reduced acuity 11.5 %, diplopia 10.5 %, enophthalmos 8.5 %, telecanthus 5 %, hyphema 3.5 %, blindness 3 % and proptosis 0.5 %. Zygomatic complex fractures were the most frequent type of injury that was complicated by blindness or a serious eye injury (61 %). Collection of data regarding the epidemiology of maxillofacial fractures is important because it may assist healthcare providers to provide necessary information for the development and evaluation of preventive measures. Ocular injuries should have an early ophthalmological examination at the time of trauma to detect any kind of ocular dysfunction. "}, {"id": "pubmed23n1042_9268", "title": "Maxillofacial Trauma in Geriatric Population.", "score": 0.0186932215234102, "content": "The worldwide population is increasingly aging. Maxillofacial fractures of the geriatric population have been increased. Evaluation of the demographic variables, causes and the patterns of maxillofacial traumas in the elderly population is the main aim of this study. Seven hundred thirteen maxillofacial tomography images which were scanned between 2010 and 2019 were evaluated. Data from 50 patients aged 65 years old and/or older, who were treated for maxillofacial fracture at the Department of Otorhinolaryngology, Gaziantep University, were retrospectively analyzed. Two groups were created according to the facial fracture pattern. Facial fractures were reclassified into 2 groups; mandibula, orbital, zygomaticomaxillary complex group fractures and the other group of frontal, naso-orbito-ethmoid fractures and were used as a comparison. The mean age of the patients was 72.5 (min 65- max 93). The gender distribution was 17 females (34%) and 33 males (66%). The most common fractured bone was the nasal bone and the least one is the frontal bone. Approximately one-quarter of 50 fractures were seen in 70 to 79 years old. Falling is more common in females and men are more prone to work-related accidents than home-related accidents. Facial fractures in the elderly often seen in midface location. Falling is the common etiology of facial fracture in all genders at elderly. However, male dominance is seen in other etiological factors. Additional diseases in the elderly seem to increase the severity of facial fracture."}, {"id": "pubmed23n1152_16109", "title": "Ophthalmic Complications in Maxillofacial Trauma: A Prospective Study.", "score": 0.016693108919086708, "content": "To determine the incidence and types of ophthalmic complications associated with maxillofacial trauma over a period of 24 months. An institutional prospective study was conducted on 62 patients presenting with maxillofacial trauma to study the correlation between facial trauma and ophthalmic complications. Road traffic accidents were reported to be the primary etiologic factor for most trauma cases studied. Zygomaticomaxillary complex (ZMC) fracture was associated with more ophthalmic complications while fractures involving the orbital rims and walls were associated with severe complications. Maxillofacial trauma, particularly those associated with midface, including ZMC fracture, Le Fort II, Le Fort III, and naso-orbito-ethmoidal fractures, can commonly cause ophthalmic complications and blindness in rare cases. Hence, every patient with maxillofacial trauma should undergo an ophthalmic examination and should be placed under close observation for necessary treatment when required."}, {"id": "pubmed23n0369_18125", "title": "Late sequelae after high midface trauma.", "score": 0.016025641025641024, "content": "The upper midface area comprises mainly the naso-orbito-ethmoidal (NOE) region which plays a paramount role in facial expression. Fractures of this area often result in neglected bony defects in the fragile periorbital region with major secondary impairments such as traumatic telecanthus, orbital dystopia, and/or enophthalmos. Permanent cranial nerve deficits also can occur as the result of post-traumatic/post-operative sequelae. Seventy-one patients (age range 7-78 years) with severe high midface trauma, treated from January 1989 to December 1996, were reviewed with a minimum follow-up of 2 years. The patient population has been distributed according to the fracture type in three groups: Group 1 (n = 35): Isolated NOE with/without associated central midface injury; Group 2 (n = 22): NOE associated with craniofacial injury and Group 3 (n = 14): NOE associated with orbital displacement. The estimated post-surgical parameters included qualitative and quantitative data from the long-term clinical evaluation. Persistent headache and/or concentration difficulties were mainly noted in Group 1. Smell reduction or anosmia was reported mainly in Group 2. Deficits of the trigeminal and/or the facial nerve were found in Group 3. Enophthalmos and/or telecanthus were predominantly seen with injuries associated with orbital displacement."}, {"id": "pubmed23n1091_5003", "title": "Nasoorbitoethmoid fractures in a tertiary care hospital of eastern India: A prospective study.", "score": 0.015685328185328185, "content": "The purpose of this study was to report on the pattern of occurrence of nasoorbitoethmoid (NOE) fractures in Odisha and the various factors that influence their distribution. The study period was from January 1, 2016 to December 15, 2017. After approval from the Institutional Ethics Committee, all patients diagnosed with naso-orbito-ethmoid fractures reporting to the department of OMFS and Level-1 trauma centers were included in the study. Sociodemographic data along with the etiology and type of fracture were mentioned. Associated injuries to other body parts were noted. Open reduction was possible only in five cases of NOE fractures. The treatment plan including the operative approach and postoperative results was evaluated. A total of 1192 patients with facial fracture were seen, of which 52 (4.36%) patients had NOE fractures. Males far outnumbered females in a ratio of 9:1. Thirty-three patients (63.46%) had unilateral NOE fracture, while the rest 19 (36.54%) had bilateral NOE fracture. Sixteen (30.76%) cases were classified as Type I, 35 (67.30%) as Type II, and 1 (1.92%) as Type III. Road traffic accidents were the most common cause of NOE fractures (69%), followed by fall (17%) and assault (10%). The most common neurological injury to be associated with NOE fractures was pneumocephalus (29%), followed by diffuse axonal injury (8%). Telecanthus (100%) was found to be the primary clinical feature in patients of NOE fracture, followed by a depressed nasal bridge (92%). Fracture of the nasal bone was invariably associated with NOE fracture. Complications observed due to untreated NOE fractures included a shortened and retruded nose, shortened palpebral fissures, telecanthus, and enophthalmos. Contemporary management of NOE complex fractures demands precise diagnosis and immediate surgical management with anatomic reduction and rigid fixation of the involved bone segments. With an improvement in socioeconomic status and increased awareness among maxillofacial surgeons, hopefully, a greater number of NOE fracture patients will avail the benefits of open reduction in future."}, {"id": "pubmed23n0873_4203", "title": "Fractures in the Maxillofacial Region: A Four Year Retrospective Study.", "score": 0.014916269269158258, "content": "The incidence of maxillofacial injuries is on the rise due to motor vehicle accidents and increased incidence of violence in recent times. The aim of this retrospective study was to determine the incidence, aetiology, the pattern of fractures, their management with open reduction and internal fixation (ORIF) and complications, if any. A retrospective analysis of 621 fractures in 361 patients managed by ORIF over a four year period was carried out. The average age of patients was 24.3 years with a male to female ratio of 21.2:1. Panfacial fractures comprised 4.7%, frontal bone fractures 8.9%, orbital fractures 0.7%, naso-orbito-ethmoid complex (NOE) fractures 0.7%, zygomatic complex fractures 23.5%, fracture maxilla 11.5% and mandibular fractures 52.2% of all facial fractures. All the cases were successfully managed by ORIF under general anaesthesia (GA). Complications were noticed in 6.8% of cases in the form of reactive implants in 3.6%, deranged occlusion in 1% and infection at operated site in 1% cases which were managed satisfactorily. The findings of this study reveal sharp annual increase in the number of cases of maxillofacial trauma. Road traffic accidents (RTA) were the commonest cause and the age group most affected was between 20-25 years. ORIF of these fractures was chosen for its obvious advantages of direct anatomical reduction, early return to function and minimal complications."}, {"id": "pubmed23n0930_2873", "title": "Facial and Orbital Fractures: A Fifteen Years Retrospective Evaluation of North East Sicily Treated Patients.", "score": 0.013696831787152109, "content": "Orbital fractures are classified as diseases usually related to common midface trauma. It represents the most challenging treatment due to the complex anatomy, physiology, and aesthetic role. A midface trauma involves also the zygomatic complex and the nose, however the orbit fracture seems to be a more frequent disease due to its anatomical features. The purpose of this work is to retrospectively evaluate and record the frequency of the midfacial traumas and orbital fractures observed in the North Eastern Sicily. The results of the present data may be useful for the clinicians in order to recognize the kind of fracture just from the first general visit having a quick diagnosis and management. In the years between 2001 and 2016, about 1200 patients with midfacial trauma and about 100 patients involving the orbital floor have been evaluated. All those patients underwent the surgical fracture reduction and a CT scan follow up control at one month, three months, six months and one year. Data showed high percentage of orbital floor, nose and mandibular body and ramus fractures; moreover the most frequent causes of fractures seem to be related to motor vehicle accident, followed by assaults, work and fall. The results have highlighted the changing trends in the causes of facial injuries, particularly the increasing incidence of assaults and the falling incidence of motor vehicle accidents in developed countries. The quick diagnosis and management proved fundamental for the successful treatment. Clinicians should be able to recognize the first symptoms in order to avoid possible complications."}, {"id": "pubmed23n0830_3757", "title": "Analysis of symptoms according to areas of orbital floor in orbital inferior wall fractures.", "score": 0.009932958690722665, "content": "A considerable number of patients experiencing facial trauma are diagnosed with blowout fracture. Preoperative computed tomographic scan is often different from the actual surgical area. This study is restricted to orbital floor fracture. This study is expected to help speculating fracture site and making surgical plans according to symptoms of periorbital trauma. From March 2005 to September 2013, a total of 150 cases of orbital floor fracture surgeries have been analyzed. This study analyzed the preoperative symptoms at the certain fractured area of orbital floor, at the aspects of sagittal view of computed tomography, which is sectioned into anterior one-third, middle one-third, posterior one-third, and mixed types. Symptoms for analysis are diplopia, extraocular movement limitation, enophthalmos and other combined facial bone fractures, and the like. Fracture areas of orbital floor are 21 cases (14%) of anterior one-third, 47 cases (31%) of middle one-third, 7 cases (5%) of posterior one-third, and 75 cases (50%) of the mixed. Frequency of diplopia was 0 case, 24 cases (42.1%), 4 cases (7.0%), and 29 cases (50.9%), respectively. In the case of extraocular movement limitation, 0 case, 15 cases (39.5%), 2 cases (5.3%), and 21 cases (55.2%) were found, respectively. In the case of enophthalmos, 0 case, 5 cases (16.7%), 7 cases (23.3%), and 18 cases (60.0%) were found, respectively. The most commonly associated other facial bone fractures were nasal bone fractures. In the case of blowout fracture, diplopia, extraocular movement limitation, enophthalmos, and other symptoms are checked through physical examination. This study would help speculating fracture site and making surgical plans according to symptoms of periorbital trauma."}, {"id": "pubmed23n0614_19472", "title": "[Secondary reconstruction of posttraumatic orbital deformities with canthus dislocation].", "score": 0.009900990099009901, "content": "To explore the secondary surgical reconstruction for orbital bone deformities accompanied with canthus dislocation after trauma. From June 1998 to July 2007, 37 patients with secondary orbital bone fracture deformity accompanied with medial or lateral canthal ligament dislocation posttraumatically were treated, among whom there were 22 males and 15 females, aged 13-46 years old (21 on average). There were 29 cases of traffic accident, 6 of boxing injury and 2 of beating injury by sticks. The latest reconstruction was performed on these 37 cases during 3 months to 8 years after injuries. There were 11 cases of orbital maxillary zygoma (OMZ) fracture, 15 of naso-orbito-ethmoid (NOE) fracture, 8 of OMZ and NOE fracture and 3 of frontal fracture. There were 31 patients who were reconstructed for the first time and 6 for the second time. Typical bicoronal and subciliary incisions and intra-oral approach were employed to expose all the fractured sites. According to the fractured position and the degree of deformity and dislocation, the orbito-zygomatic fracture was repositioned after osteotomy and rigid fixation, or the healed fragments were trimmed with a burr and the depressed fragments were filled with autogenous bone such as ilium, cranial outer table or Medpor in order to reconstruct orbital wall framework; the orbital walls were repaired to correct the enophthalmos with autogenous bone or Medpor after the herniated orbital contents were released. The medial canthal ligament was anchored superior-posteriorly to the lacrimal fossa with transnasal wires fixation or fixed with titanium miniplates and nails. The 36 patients' incisions obtained healing by first intention after the operation, and 1 case failed because of wound infection from maxillary sinusitis. There were 24 patients who were cured successfully with facial appearance and function improved significantly. During the follow-up for 3-6 months, no complication was found such as dislocation of the implant, rejection and infection. Two patients still showed slight enophthalmos while 3 patients with canthus dislocation regained improved appearances but not satisfactory. At 6 months after operation, the CT scan conducted in 3 patients with autogenous bone and Medpor grafting showed all fractures were fixed rigidly. Surgical reduction combined with bone grafting is a satisfactory method for the correction of secondary orbital bone deformity, and the repair of canthus dislocation and correction of enophthalmos should be considered at the same time. An ideal result could be achieved only through all-round consideration and comprehensive treatment."}, {"id": "pubmed23n0573_8784", "title": "Occurrence of mandibulofacial injuries presenting to the otorhinolaryngology and head &amp; neck surgery department.", "score": 0.009900990099009901, "content": "Trauma is the fourth major cause of mortality in the Western countries, of which approximately one half involve maxillofacial injury. Statistics reported by emergency room officials show motor vehicles cause many of the injuries and deaths that occur in Iran. Having completed a retrospective descriptive study of 200 patients who experienced maxillofacial trauma, the authors report its occurrence with respect to age, sex, trauma type, and site of injury so as to evaluate the operational functionality of the Department of Otolaryngology and Head &amp; Neck Surgery of Hazrat-e Rasoul Akram Hospital from 2000 to 2004. Mandibular fractures (36.2%) occurred in the subsequently listed sites and at the specified frequencies: mandibular angle (9.7%), mandible body (6.9%), parasymphysis, ramus and subcondyle at 5.6% each, and symphysis at 2.8%. No condylar fractures were reported. Frontal bone fracture was observed in 9.7% of the patients with eye globe injury occurring simultaneously in 8.3% of corresponding cases. Orbital fracture (63.9%) also occurred in various cases as follows: orbital floor 39%, lateral rim 24%, inferior rim 22%, medial wall 11%, and the superior rim and orbital roof at 2% each. Motor vehicle accidents were the most common causes of trauma (42%). The most common fracture was in the zygoma (43%) with 8.3% of them being orbital injury. Fractures of mandibular bones (36.2%) and the maxilla (33%) were the most commonly seen in trauma occurring to the maxillomandibular region."}, {"id": "pubmed23n0083_4231", "title": "[Clinical study of mandibular condyle injury].", "score": 0.009708737864077669, "content": "Mandibular condyle fractures develop frequently and show the variable type of injury and complication. New opinions have emerged from recent investigation into condylar fractures. The author investigated 246 patients with condylar fractures who visited SNUDH from January 1980 to August, 1988, 8. with regard to clinical and treatment aspects, area and displacement of fractures, associated teeth injury and other body injury, complications. At last I have got the following results. 1. The incidence to condylar fractures in a series of 765 mandibular fractures may be as high as 32.2%. 2. The male patients are 3 times more than female patients. The highest frequency was recorded in the group 21-30 years of age. (34.1%). 3. Falls caused the greatest number of condylar fractures (45.2%) and next was in assult (25.6%), traffic accidents (22.4%). 4. Unilateral condylar fractures were present in 74.8%, giving a left: right ratio of 1.2:1. In cases of unilateral fracture, subcondylar fractures were by far the commonest (32.9%) but in cases of bilateral fracture, condylar neck fractures were by far the commonest. In children under 15 years of age, condylar neck fractures were more common but in patients over 16 years of age, subcondylar fractures were common. 5. Anteromedial fracture dislocations were by far the commonest (20.3%). In children under 15 years of age, fracture deviations were common but in patients over 16 years of age, fracture displacements were common. 6. 44.7% of patients with condylar fractures sustained the teeth injuries. Teeth fractures were by far the commonest. 7. Single condylar fractures showed a frequency of 30.5%. Of the concomitant fractures elsewhere in the mandible, symphysis fractures were by far the commonest (54.1%). 8. Associated other body injuries showed a frequency of 28.0%. Of them, head injuries were by far the commonest. 9. The mean interval from injury to treatment was 14.3 days. Of the treatment of condylar fractures, open reduction was by far the commonest (70.3%). Closed reduction comprised 19.9% and functional therapy comprised 8.5%. 10. In 67 patients with possible follow up period, the following complications were developed, two ankylosis, anterior open bite, mouth opening limitation, mouth opening deviation."}, {"id": "pubmed23n1003_17152", "title": "Patterns of facial fractures in children.", "score": 0.009523809523809525, "content": "Morbidity and mortality among children is usually the result of trauma. Because a child's face is retruded relative to the protecting skull, has a thicker layer of adipose tissue, more elastic bones, flexible sutures lines, the presence of tooth buds within the jaws, and the lack of pneumatisation of the sinuses, the facial bones fracture less commonly than in adults. Our aim was to assess the patterns of such fractures in children who presented to the department of Oral and Maxillofacial Surgery, King Edward Medical University/Mayo Hospital Lahore, Pakistan. All 535 eligible children between the ages of 1-16 years who presented during the two years December 2009 - December 2011 were included in the study. Facial fractures were diagnosed by clinical examination, plain radiographs, and computed tomography, and the pattern of fractures of the facial bones including the frontal bone, orbital bones, maxilla, zygoma, naso-orbito-ethmoidal complex, mandible, and dentoalveolar region was documented. The male:female ratio was 2:1 with 369 male (70%) and 166 female (31%) patients. Fall was the cause in 212 (39%), and in 167 (31%) it was road traffic accidents, while sports were the cause in 135 (25%). The naso-orboto-ethmoid complex was fractured in 37 cases (7%) while 104 children (19%) presented with isolated fractures of the zygomatic bone. The maxilla was fractured in 195 cases (36%), the mandible in 380 (71%), and dentoalveolar trauma was the cause in 256 (50%). The mandible was the bone that was most often fractured (mostly in boys and usually as a result of falls during summer vacations), with the peak occurring in those aged 8-12 years."}, {"id": "pubmed23n0748_7976", "title": "Treatment of complex facial fractures:  clinical experience of different timing and order.", "score": 0.009523809523809525, "content": "Given the variability of the timing and order of surgeries, it is difficult to choose the best treatment for patients with complex facial fractures. Based on the clinical experiences, the authors have reviewed their experience with the timing and order of operations depending on the sites of complex facial fractures and their concurrent injuries. The current study was based on a total of 105 patients with complex facial fractures from the year 2002 to 2011. After assessing the patients' clinical records, radiological data, and clinical photographs, the following data were analyzed: patients' age and sex, causes of injury, concurrent injuries, sites of fractures, the interval between trauma and the operations, the presence of additional surgeries, and the aesthetic and functional outcomes.For most of the patients, early operation was performed (within 2 weeks in 95.2%). Additional surgeries within 1 month after injuries were performed in 22 patients. Usually, a top-to-bottom direction repair was applied when head injuries were involved, and bottom-to-top direction repair was applied when occlusal problems were involved. Of 105 patients whom we were able to follow up, 49 patients showed complications or were dissatisfied with the outcomes. However, except them, most of the patients were satisfied with the outcomes of surgical treatments. There were 14 cases of cheek asymmetry, 9 enophthalmos, 30 paresthesia, 4 malocclusion, and a single case of persistent trismus.In the current study, satisfactory results could be achievable under the following principles: a repair should be done in the early stage after the onset of the injury; supportive surgeries should be done, if necessary, within 2 weeks (no later than 4 weeks); and the order of surgical treatment should be determined by the severity of bone fracture and the systemic status."}, {"id": "pubmed23n1072_12427", "title": "Prevalence of Ocular Complications in Patients with Zygomatic Bone Fractures in an Iranian Population.", "score": 0.009433962264150943, "content": "Damages to the middle third of the facial bone generally involve the orbital skeleton and can lead to eye impairment. In this study, it is attempted to determine the incidence of ophthalmic injuries in maxillofacial trauma with zygomatic bone fractures. One hundred and fifteen cases with ophthalmic (ocular) involvement after maxillofacial trauma were referred to the Shariati Hospital, Tehran, Iran, and were visited at the Ophthalmology Department between 2016 and 2018. Zygomatic fractures and resulting ocular complications were evaluated in 87 males and 28 females with the mean ages of 26 and 32 years, respectively. Subconjunctival ecchymosis was detected in 23.07% of men and 21.05% of women. Displacement of the palpebral fissure was detected in 26.5% of men and 27.6% of women. Furthermore, the unequal pupillary level was observed in 18.37% of men and 15.78% of women. Diplopia was detected in 8.9% of men and 10.5% of women. Additionally, enophthalmos was observed in 23.1% of men and 25% of women. The most common ocular presentations in midfacial trauma are diplopia and reduced visual acuity. Even after the operation, a significant number of patients experience poor vision and diplopia. Ophthalmology consultation is essential for these patients."}, {"id": "article-25562_29", "title": "Naso-Orbito-Ethmoid Fractures -- Complications", "score": 0.00937242975459536, "content": "Mental health issues, as patients with facial injuries, are at greater risk of developing post-traumatic stress disorder or anxiety-related disorders, particularly those who were victims of assault. [31]"}, {"id": "pubmed23n0913_4425", "title": "A study of sports-related orbital fractures in Singapore.", "score": 0.009345794392523364, "content": "With an increased popularity of sport and active living worldwide, our study aims to explore the incidence and features of sports-related orbital fractures in Singapore. 1421 computer tomography (CT) imaging scans of the face and orbits done at the National University Hospital over a 24-month period from January 2013 and December 2014 were reviewed retrospectively for orbital fractures. We identified 483 orbital fractures of which sports injury was the fourth most common etiology (n = 65; 13.5%) after road traffic accident (n = 131; 27.1%), geriatric fall (n = 81; 16.8%) and workplace injury (n = 67; 13.9%). The three most common sport in orbital fractures were soccer (n = 20; 30.8%), bicycling (n = 11; 16.9%) and jogging (n = 8; 12.3%). The three most common fracture patterns were zygomatico-maxillary complex fractures (n = 24; 36.9%), isolated one wall blowout fractures (n = 19; 29.2%) and naso-orbito-ethmoid fractures (n = 7; 10.8%). Sports-related orbital fractures were associated with a low mean age of patients (45.9 years, range, 14-79 years), a higher proportion of males (n = 58; 89.2%) than that from geriatric falls (n = 37, 45.6%) (P &lt; 0.01), a higher likelihood of unilaterality (n = 62; 95.4%) than that from traffic accidents (n = 99; 75.6%) (P &lt; 0.01) and a lower likelihood of pan-facial involvement (n = 4; 6.15%) than that from traffic accident (n = 60; 45.8%) (P &lt; 0.01). Sports-related orbital fractures are the fourth most common cause of orbital fractures. Though commonly seen in young male adults, in view of the aging population and people exercising more regularly, education of safety measures among sports users is paramount to preventing sports-related orbital fractures."}, {"id": "pubmed23n0401_12660", "title": "The causes and consequences of maxillofacial injuries in elderly people.", "score": 0.009345794392523364, "content": "The occurrence of trauma in older people is well-documented; however the incidence of maxillofacial trauma is scarcely reported. Therefore, the objective of this study is to determine the causes and consequences of maxillofacial trauma in older people. A five-year (March 95 - March 2000) retrospective study was carried out of all patients over the age of 65 years with facial trauma presenting to Accident and Emergency Department (A&amp;E). The information was collected using the medical notes and discharge summaries. The Departments of A&amp;E and Maxillofacial Surgery. A total of 42 patients' records were examined for study related data. A total of 42 patients were seen during the study period. Thirty-six gave a history of a fall, of which 15 had tripped, 5 had slipped, 3 resulted from a Transient Ischaemic Attack (TIA), 1 as a result of alcohol abuse, in 1 a prosthetic knee gave way and 11 gave no cause for the fall. Of the remaining 6 patients, 5 were assaulted and 1 had a wardrobe fall on top of him. The majority of the falls occurred during the winter months. Maxillofacial injuries were noted in 27 of the 42 patients. Sixteen patients had cheekbone fractures, 8 mandibular fractures, 2 midface and 1 orbital complex fracture. Twenty-five percent of cheekbone fractures and 50% of mandibular fractures were treated surgically. Medical history was noted in 27 patients. This study clearly demonstrates the majority of the facial trauma in the older people can be treated conservatively unless the patients complain of functional problems."}, {"id": "article-25562_23", "title": "Naso-Orbito-Ethmoid Fractures -- Differential Diagnosis", "score": 0.00925965527565117, "content": "Pan-facial fractures - these are fractures involving the upper, middle, and lower regions of the face."}, {"id": "wiki20220301en064_21413", "title": "Basilar skull fracture", "score": 0.009259259259259259, "content": "A basilar skull fracture is a break of a bone in the base of the skull. Symptoms may include bruising behind the ears, bruising around the eyes, or blood behind the ear drum. A cerebrospinal fluid (CSF) leak occurs in about 20% of cases and may result in fluid leaking from the nose or ear. Meningitis occurs in about 14% of cases. Other complications include injuries to the cranial nerves or blood vessels. A basilar skull fracture typically requires a significant degree of trauma to occur. It is defined as a fracture of one or more of the temporal, occipital, sphenoid, frontal or ethmoid bone. Basilar skull fractures are divided into anterior fossa, middle fossa and posterior fossa fractures. Facial fractures often also occur. Diagnosis is typically by CT scan."}, {"id": "wiki20220301en480_4380", "title": "Zygomaticomaxillary complex fracture", "score": 0.009217469795861685, "content": "There is an association of ZMC fractures with naso-orbito-ethmoidal fractures (NOE) on the same side as the injury. Concomitant NOE fractures predict a higher incidence of post operative deformity. Treatment Non-displaced or minimally displaced fractures may be treated conservatively. Open reduction and internal fixation is reserved for cases that are severely angulated or comminuted. The purpose of fixation is to restore the normal appearance of the face. Specific attention is given to the position of the malar eminence and reduction of orbital volume by realigning the zygoma and sphenoid. Failure to correct can result in rotational deformity and increase the volume of the orbit, causing the eye to sink inwards."}, {"id": "pubmed23n0879_2053", "title": "Do Radiologists and Surgeons Speak the Same Language? A Retrospective Review of Facial Trauma.", "score": 0.009174311926605505, "content": "The objective of the present study is to examine the concordance of facial fracture classifications in patients with trauma who underwent surgery and to assess the epidemiologic findings associated with facial trauma. Patients with trauma who underwent facial CT examination and inpatient operative intervention during a 1-year period were retrospectively analyzed. Patient demographic characteristics, the mechanism of injury, the radiology report, the surgical diagnosis, and clinical indications were reviewed. Fractures were documented according to bone type and were classified into the following subtypes: LeFort 1, LeFort 2, LeFort 3, naso-orbital-ethmoidal, zygomaticomaxillary complex (ZMC), orbital, and mandibular. Concordance between the radiology and surgery reports was assessed. A total of 115,000 visits to the emergency department resulted in 9000 trauma activations and 3326 facial CT examinations. One hundred fifty-six patients (4.7%) underwent facial surgical intervention, and 133 cases met criteria for inclusion in the study. The mean injury severity score was 10.2 (range, 1-75). The three most frequently noted injury mechanisms were as follows: assault (77 cases [57.9%]), a traffic accident (21 cases [15.8%]), and a fall (20 cases [15%]). The three most frequently noted facial bone fractures were as follows: mandible (100 cases [75.2%]), maxilla (53 cases [39.8%]), and orbit (53 cases [39.8%]). The five descriptors most frequently found in the radiology and surgery reports were the mandibular angle (25 cases), the orbital floor (25 cases), the mandibular parasymphysis (22 cases), the mandibular body (21 cases), and ZMC fractures (19 cases). A classification was not specified in 31 of the radiologic impressions (22.5%), with 28 of 31 radiologists expecting the surgeon to read the full report. The descriptors used in the radiology and surgery reports matched in 73 cases (54.9%) and differed in 51 cases (38.3%). No classifications were used by one or both specialties in nine cases (6.8%). For 38.3% of patients needing facial surgery, descriptors used in the radiologic and surgery reports differed. Speaking a common language can potentially improve communication between the radiology and surgery services and can help expedite management of cases requiring surgery."}, {"id": "pubmed23n0629_14904", "title": "Epistaxis as the only initial symptom in pediatric naso-orbital-ethmoid fracture complicated with meningitis.", "score": 0.00909090909090909, "content": "Epistaxis is a frequent finding in patients with facial trauma. Herein, we report an unusual presentation of pediatric naso-orbital-ethmoid (NOE) fracture with epistaxis as the only initial symptom. The course of the patient's condition was later complicated by meningitis, related in part to the delay in diagnosis. A 3-year-old girl with preexisting upper respiratory symptoms was involved in a traffic accident, sustaining blunt trauma to the right side of her face. During the initial examination, only right-sided epistaxis was noted. Five days later, she developed febrile convulsion and was admitted to the intensive care unit with other signs of meningitis such as mental status change and neck stiffness. Her craniofacial computed tomographic scan showed a right-sided NOE fracture with minimal displacement and without dura tear. The cerebrospinal fluid culture grew Streptococcus pneumoniae, which may be due to ascending infection as a result of cribriform plate fracture. Intravenous antibiotic therapy was initiated with good response, and she was discharged from the hospital after 2 weeks. The presence of epistaxis and periorbital bruise, together with other symptoms and signs, helps in the identification of NOE and cribriform plate fracture. A high index of suspicion with repetitive computed tomographic scans is necessary to achieve correct early diagnosis. Parental antibiotic therapy is indicated if ascending cerebrospinal fluid infection develops."}, {"id": "pubmed23n0071_4796", "title": "[Clinical studies on treatment of fractures of the zygomatic bone].", "score": 0.00909090909090909, "content": "The author has made clinical studies on treatment of fractures of the zygomatic bone in terms of frequency of fractures according to sex, age, fracture type, main manifestations and treatment methods from 106 patients with zygomatic bone fractures among 969 patients with maxillofacial bone fractures. The results obtained were as follow: 1. The frequency of malar bone fracture was 4 times more in male than that in female. 2. The most prevalent age of malar bone fracture was 21-30 years of age, and the nexts were followed 11-20, 31-40, 0-10, 41-50, 51-60, 61-70, and over 71 in the orders. 3. Among maxillofacial bone fractures, mandibular fracture was most prevalent as 76.3%, and the nexts were followed by the maxilla (10.8%), the molar bone (9.7%) and the nasal bone (3.3%). 4. Among 106 fractures of the malar bone, zygomatic bone fracture only was occupied 48.1%, but the rests were accomplished by another maxillofacial bone fractures. 5. In classification of molar bone fractures according to Knight and North's, group 3 fractures were most prevalent, and followed by group 2, 1, 4 and 6 in the orders. 6. Main manifestations were upper cheek flattening, lower eyelid ptosis, subconjunctival ecchymosis, epistaxis, difficulty of mouth opening, pain during mouth opening and others in the orders. 7. 93.4% of malar bone fracture has been treated surgically, but the rests treated conservatively, and [symbol: see text] shaped elastic stapler wire has been effectively used to get fixation at zygomatico-maxillary fractures."}, {"id": "pubmed23n0894_8066", "title": "Dental trauma and bicycle safety: a report in Italian children and adolescents.", "score": 0.009009009009009009, "content": "This retrospective study aims to analyze the pattern of oro-facial trauma from bicycle accidents in Italian children and adolescents, focusing on the safety devices used. The medical records of 1405 patients of the Dental Clinic of the University of Brescia, between the age of 0 to 18, who experienced a dento-facial trauma from the use of a bicycle, were analyzed. Data regarding age, gender, weight, height, dominant hand, type of bicycle, use of safety devices, location and type of dental trauma, teeth involved, bone fractures and soft tissue lesions were recorded. Statistical analysis was performed. The majority of the traumatic events occurred in children within the 8-10 years of age-range; 1085 teeth were injured, of which 975 permanent teeth (89.9%) and 110 primary teeth (10.1%). The most common dental lesions were the coronal fractures (complicated and not complicated) while the most frequently involved teeth were the upper central incisors; 11% of patients were also treated for maxillo-facial fractures. A protective helmet was worn only in 3% of the cases; not one patient wore a mouth-guard. The use of helmets was more frequent in children and adolescents riding racing-bikes competitively, compared to those who were mountain bikers (p &lt; 0.05). Bicycle accidents can have serious oro-facial consequences. Therefore, national and regional efforts should be made in Italy to promote head and mouth protection in cycling."}, {"id": "pubmed23n0069_5048", "title": "The incidence and management of middle third facial fractures at the University College Hospital, Ibadan.", "score": 0.009009009009009009, "content": "A study of 59 patients with middle third maxillofacial fractures over a 5 year period was undertaken. The incidence of, pattern of fracture, type of accompanying injuries, and assessment of the outcome of given treatment were studied. The young adult male featured prominently in the study giving a male/female ratio of 14:1. The zygomatico-maxillary complex was fractured in 42.4% of the cases, while the Le Fort 1 fracture was the most common of the Le Fort Fracture types. Road traffic accidents accounted for 81.4% of the aetiological factors, while armed robbery attacks was the next common source of trauma at 6.8%. Treatment by the Gillies temporal approach and immobilization within the tissues techniques produced very satisfactory results in 96% of the patients who received treatment. Loss of vision in one eye was the most common residual complication."}, {"id": "wiki20220301en162_12653", "title": "Le Fort fracture of skull", "score": 0.008989898989898989, "content": "Signs and symptoms Le Fort I — Slight swelling of the upper lip, ecchymosis is present in the buccal sulcus beneath each zygomatic arch, malocclusion, mobility of teeth. Impacted type of fractures may be almost immobile and it is only by grasping the maxillary teeth and applying a little firm pressure that a characteristic grate can be felt which is diagnostic of the fracture. Percussion of upper teeth results in cracked pot sound. Guérin's sign is present characterised by ecchymosis in the region of greater palatine vessels. Le Fort II and Le Fort III (common) — Gross edema of soft tissue over the middle third of the face, bilateral circumorbital ecchymosis, bilateral subconjunctival hemorrhage, epistaxis, CSF rhinorrhoea, dish face deformity, diplopia, enophthalmos, cracked pot sound. Le Fort II — Step deformity at infraorbital margin, mobile mid face, anesthesia or paresthesia of cheek."}, {"id": "pubmed23n0090_20814", "title": "[Facial bone fracture--statistical analysis and clinical aspects].", "score": 0.008928571428571428, "content": "We experienced 102 cases of facial bone fracture during 16 months of 1986 to 1987. These cases were analyzed statistically concerning causes, age and locations of the fracture. These fractures have increased rapidly in number. The causes were classified into three types; occurrence during sport, traffic accident and fighting, which were equal in number. There were 85% males and 15% females in the patient cohort, which were concentrated at the ages of 10-20 years. A large part of the fractures was mostly consisted of maxillo-facial components (95%). These trends were similar to the previous report of our clinic (1972-1979). On the other hand, not only severe dysfunctioning cases but also complicated cases increased in number, so that the several clinical aspects were reported. Case 1: 17-year-old male presented with retraction of left cheek caused by Rugby foot ball, whose malar bone was dislocated backward and anticlockwise, was treated with oroantral reduction and with the intermaxillary packing of silicon blocks. Case 2: 10-year-old boy with complaint of double vision occurred by head blow to right eye. Pure type blowout fracture of the orbital floor was presented, which was reconstructed by silicon plate from the incision of the lower eyelid. Case 3: 59-year-old male presented with 6 month history of diplopia and retraction of left eye ball, had been under the conservative care by an eye doctor. X-ray examination showed the intraorbital soft tissue was blown out into the ethmoidal sinus. However the transethmoidal reduction was performed, the result was not satisfactory.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n1055_4827", "title": "Craniofacial Trauma Due to Stone-pelting - Patterns of Injury and Management.", "score": 0.008849557522123894, "content": "The aims of the study were to elucidate the pattern of stone-pelting induced cranio-facial injuries and to document soft and hard tissue injuries, their management, and complications. A retrospective descriptive study was conducted using a sample of patients reporting to our department in the years 2015 to 2020. Cranio-facial injuries were assessed for soft and hard tissue injuries, including tissue loss, and corresponding management. Follow up ranged from 18 ± 6 months. &amp; A standardized surgical regime was followed for patient management, which included primary survey, debridement of wounds, and routine primary repair of soft tissue. Bony defect reconstruction was performed by open reduction and internal fixation. Cranial bone was used as split calvarial graft in postcraniectomy cranioplasty procedures, which were performed after 6 months. Local flaps were used for the reconstruction of soft tissue defects. Being a military hospital, majority of cases fell in the 20 to 30 age group with a male preponderance. The etiology in all cases was stone-pelting. Among cranio-facial injuries, cranial vault injuries and mid-face injuries (71%) were most prevalent, mandibular fractures (24%) and remaining were soft tissue injuries (5%). &amp; Frontal &amp; parietal bone injuries were seen in 23.6% cases (n = 9) and orbito-zygomatic complex injuries were seen in (36.8%) cases (n = 14). Isolated blow-out fractures were seen in 4 patients of our series. 52.6% of patients of our series suffered associated soft tissue injuries to the head, face, and neck region. The most common cause of injury was due to the direct impact of stone hitting the mid-face/cranial vault and the most common pattern of injury was gross comminution of the skeleton. &amp; 2 patients suffered ocular injuries that required management and 6 patients of our series who suffered head injuries to the cranium required a secondary cranioplasty procedure (n = 4) &amp;The most commonly used technique for treatment was open reduction internal fixation, which was used in 89% of patients. Soft-tissue injuries overall occurred most frequently on the forehead, nose, lips, and chin which was managed by primary suturing. Cranial vault injuries &amp; orbito-zygomatic complex fractures are most commonly seen in patients with stone-pelting injuries. Early management of such injuries improves outcomes in terms of function and restitution of preinjury skeleton structure. The most common patterns seen is gross comminution to the cranio-facial skeleton that can be treated with immediate primary wound repair after meticulous wound debridement and open reduction and internal fixation. Importance of stone-pelting as a cause of craniofacial injuries is highlighted as it leads to significant disruption of craniofacial skeleton."}, {"id": "pubmed23n0818_15083", "title": "Maxillofacial fracture experiences: a review of 152 cases.", "score": 0.008849557522123894, "content": "The fractures of facial structures lead to great morbidity. Cross-sectional studies are needed to evaluate the current state of maxillofacial traumas. Thus, this study aims to evaluate these experiences and to compare these results with the current literature. The medical records of the maxillofacial fracture cases hospitalized between January 2004 and November 2011 were examined. The age, sex, etiology, fracture localization and treatment method for each case were documented. The affected facial bones were grouped as mandible, maxilla, zygoma, naso-orbitoethmoid complex (NOEC) and blow-out. Nasal fractures were excluded. The cases were assigned to 3 groups with respect to age (below 16, above 65 and between 17 and 64). The chi Square test was used to assess the significance of the difference in mandibular fracture rates in the pediatric population compared to others. The total number of cases was 152. The total number of fractures was 185. Of the 152 cases, 117 were male and 35 were female. The average age was 31.4 (±18.3), ranging between 2 and 81. Thirty-one cases were 16 years old or less. Nine cases were 65 years old or more. Mandibular and zygomatic fractures were the most prevalent fractures in the adult group. Mandibular fractures were significantly more common in the pediatric age group compared to rest of the population (X(2), p&lt;0.05). Traffic accidents were the most common etiological factor, with a 55.3% ratio. Open reduction and internal fixation was the most frequently conducted treatment modality in all age groups. Retrospective studies are important for the projection of future prospects. In summary, our results indicate that pediatric fractures are mostly in the lower face and usually affect the condylar region, which is consistent with the literature."}, {"id": "pubmed23n0743_11302", "title": "Zygomaticomaxillary complex fractures and their association with naso-orbito-ethmoid fractures:  a 5-year review.", "score": 0.008771929824561403, "content": "Zygomaticomaxillary complex fractures associated with ipsilateral naso-orbito-ethmoidal fractures are more complex injuries than isolated zygomaticomaxillary complex fractures. This injury pattern can have significant long-term morbidity if not recognized and treated appropriately during the initial operation. The purpose of this study is to compare mechanisms of injury, treatment, and outcome between patients with zygomaticomaxillary complex fractures and those with zygomaticomaxillary complex and ipsilateral naso-orbito-ethmoidal fractures. A 5-year retrospective review of all patients treated with zygomaticomaxillary complex fractures at a level I trauma center was performed. Computed tomographic scans were reviewed to divide patients into those with zygomaticomaxillary complex fractures alone and those with zygomaticomaxillary complex and ipsilateral naso-orbito-ethmoidal fractures. Demographics, treatment protocols, outcomes, complications, reoperations, and length of follow-up were identified for both groups and compared to determine differences between these populations. A total of 245 patients were identified by the Current Procedural Terminology codes for zygomaticomaxillary complex fractures. One hundred eighty-five patients had zygomaticomaxillary complex fractures and 60 patients had zygomaticomaxillary complex/naso-orbito-ethmoidal injuries. The demographics for both populations were similar. There are differences between the groups with regard to mechanism of injury, operative findings, and techniques. The patients with zygomaticomaxillary complex/naso-orbito-ethmoidal fractures had higher rates of postoperative complications and deformities. Patients who sustain a zygomaticomaxillary complex fracture associated with an ipsilateral naso-orbito-ethmoidal fracture have a higher incidence of postoperative complications and deformities. It is important to recognize this fracture pattern early to help minimize postoperative morbidity. Risk, II."}, {"id": "pubmed23n0410_21117", "title": "An assessment of maxillofacial fractures: a 5-year study of 237 patients.", "score": 0.008771929824561403, "content": "This descriptive analytical study assesses the cause, type, incidence, demographic, and treatment data of maxillofacial fractures managed at our medical center during a 5-year period and compares them with the existing body of literature on the subject. A 5-year retrospective clinical and epidemiologic study evaluated 237 patients treated for maxillofacial fractures from 1996 to 2001 at one medical center. There were 211 male patients (89%) and 26 (11%) female patients. The patients ranged in age from 3 to 73 years, with 59.0% (140 patients) in the 20- to 29-year age group. A number of parameters, including age, gender, cause of injury, site of injury, type of injury, treatment modalities, and complications, were evaluated. All maxillofacial injuries were assessed and treated by a single oral and maxillofacial surgeon. Other concomitant bodily injuries were treated by appropriate consultant specialists. There were 173 (72.9%) mandibular, 33 (13.9%) maxillary, 32 (13.5%) zygomatic, 57 (24.0%) zygomatico-orbital, 5 (2.1%) cranial, 5 (2.1%) nasal, and 4 (1.6%) frontal injuries. Car accidents caused 73 (30.8%), motorcycle accidents caused 55 (23.2%), altercations 23 (9.7%), sports 15 (6.3%), and warfare caused 23 (9.7%) of the maxillofacial injuries. Regarding distribution of mandibular fractures, 32% were seen in the condylar region, 29.3% in the symphyseal-parasymphyseal region, 20% in the angle region, 12.5% in the body, 3.1% in the ramus, 1.9% in the dentoalveolar, and 1.2% in the coronoid region. The distribution of maxillary fractures was Le Fort II in 18 (54.6%), Le Fort I in 8 (24.2%), Le Fort III in 4 (12.1%), and alveolar in 3 (9.1%). Of the 173 mandibular fractures, 56.9% were treated by closed reduction, 39.8% by open reduction, and 3.5% by observation only. Of 33 maxillary fractures, 54.6% were treated using closed reduction, 40.9% using open reduction, and 4.5% with observation only. Approximately 52.1% of the patients were treated under general anesthesia, and 47.9% were treated under local anesthesia and sedation. Postsurgical complications were recorded in 5% of patients. These complications included infection, asymmetry, and malocclusion. Overall mortality in this series was 0.84% (2 patients); mortality was caused by pulmonary infection. The findings of this study, compared with similar studies reported in the literature, support the view that the causes and incidence of maxillofacial injuries vary from 1 country to another."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 236]], "word_ranges": [[0, 39]], "text": "We are being described an acute pain crisis in a patient with gonarthrosis. In this situation, the first thing to do is to resolve the pain crisis and to propose an appropriate conservative treatment for this osteoarthritis (1 correct)."}, "2": {"exist": true, "char_ranges": [[237, 304]], "word_ranges": [[39, 52]], "text": "A knee arthroplasty is not considered at the outset, so 2 is false."}, "3": {"exist": true, "char_ranges": [[305, 433]], "word_ranges": [[52, 75]], "text": "They are not telling us an infectious clinic to suspect an arthritis that would justify a debridement and washing so 3 is false."}, "4": {"exist": true, "char_ranges": [[540, 844]], "word_ranges": [[92, 144]], "text": "Baker's cyst would only interest us in a severe pain crisis in the differential diagnosis with a deep thrombosis and it is evaluated with an echo-Doppler, in the picture of gonarthrosis it has no value to detect a Baker's cyst. Tendinitis is diagnosed by examination, not with MRI. Therefore, 4 is false."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "We are being described an acute pain crisis in a patient with gonarthrosis. In this situation, the first thing to do is to resolve the pain crisis and to propose an appropriate conservative treatment for this osteoarthritis (1 correct). A knee arthroplasty is not considered at the outset, so 2 is false. They are not telling us an infectious clinic to suspect an arthritis that would justify a debridement and washing so 3 is false. In a patient with gonarthrosis there will always be meniscopathy, it is part of the degenerative changes. Baker's cyst would only interest us in a severe pain crisis in the differential diagnosis with a deep thrombosis and it is evaluated with an echo-Doppler, in the picture of gonarthrosis it has no value to detect a Baker's cyst. Tendinitis is diagnosed by examination, not with MRI. Therefore, 4 is false.", "full_answer_no_ref": "We are being described an acute pain crisis in a patient with gonarthrosis. In this situation, the first thing to do is to resolve the pain crisis and to propose an appropriate conservative treatment for this osteoarthritis ([HIDDEN]). A knee arthroplasty is not considered at the outset, so [HIDDEN]. They are not telling us an infectious clinic to suspect an arthritis that would justify a debridement and washing so [HIDDEN]. In a patient with gonarthrosis there will always be meniscopathy, it is part of the degenerative changes. Baker's cyst would only interest us in a severe pain crisis in the differential diagnosis with a deep thrombosis and it is evaluated with an echo-Doppler, in the picture of gonarthrosis it has no value to detect a Baker's cyst. Tendinitis is diagnosed by examination, not with MRI. Therefore, [HIDDEN].", "full_question": "A 73-year-old woman with a history of obesity, type 2 diabetes mellitus, hypertension and dyslipidemia. She consults for unbearable pain in the right knee of 5 days of evolution, without previous trauma. Examination: globular knee, moderate varus, extension and flexion limited by pain, diffuse medial pain. X-ray shows osteophytes and mild impingement of the medial interlining. What would be his initial management?", "id": 470, "lang": "en", "options": {"1": "Explanation of the diagnosis, relative rest, paracetamol 1g/8h plus metamizol 500 mg/ 8 h rescue naproxen.", "2": "Preferential referral to Traumatology outpatients for evaluation of total cemented prosthesis.", "3": "Preferential referral to Traumatology outpatient clinic for arthroscopic debridement.", "4": "Preferred MRI request for evaluation of meniscopathy, Baker's cyst and/or tendinitis.", "5": NaN}, "question_id_specific": 138, "type": "ORTHOPEDIC SURGERY AND TRAUMATOLOGY", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en012_37209", "title": "Tendinopathy", "score": 0.013476378693769999, "content": "Tendinopathy, a type of tendon disorder that results in pain, swelling, and impaired function. The pain is typically worse with movement. It most commonly occurs around the shoulder (rotator cuff tendinitis, biceps tendinitis), elbow (tennis elbow, golfer's elbow), wrist, hip, knee (jumper's knee, popliteus tendinopathy), or ankle (Achilles tendinitis). Causes may include an injury or repetitive activities. Groups at risk include people who do manual labor, musicians, and athletes. Less common causes include infection, arthritis, gout, thyroid disease, and diabetes. Diagnosis is typically based on symptoms, examination, and occasionally medical imaging. A few weeks following an injury little inflammation remains, with the underlying problem related to weak or disrupted tendon fibrils."}, {"id": "InternalMed_Harrison_26086", "title": "InternalMed_Harrison", "score": 0.012804713096275813, "content": "X-rays are indicated to evaluate chronic hand pain and hip pain thought to be due to OA, as the diagnosis is often unclear without confirming radiographs. For knee pain, x-rays should be obtained if symptoms or signs are not typical of OA or if knee pain persists after inauguration of effective treatment. In OA, radiographic findings (Fig. 394-7) correlate poorly with the presence and severity of pain. Further, radiographs may be normal in early disease as they are insensitive to cartilage loss and other early findings. Although MRI may reveal the extent of pathology in an osteoarthritic joint, it is not indicated as part of the diagnostic workup. Findings such as meniscal tears andcartilage and bone lesions occurin most patients with OA in the knee, but almost never warrant a change in therapy."}, {"id": "pubmed23n0965_8967", "title": "Knee Pain in Adults and Adolescents: The Initial Evaluation.", "score": 0.012089999269486448, "content": "Knee pain affects approximately 25% of adults, and its prevalence has increased almost 65% over the past 20 years, accounting for nearly 4 million primary care visits annually. Initial evaluation should emphasize excluding urgent causes while considering the need for referral. Key aspects of the patient history include age; location, onset, duration, and quality of pain; associated mechanical or systemic symptoms; history of swelling; description of precipitating trauma; and pertinent medical or surgical history. Patients requiring urgent referral generally have severe pain, swelling, and instability or inability to bear weight in association with acute trauma or have signs of joint infection such as fever, swelling, erythema, and limited range of motion. A systematic approach to examination of the knee includes inspection, palpation, evaluation of range of motion and strength, neurovascular testing, and special (provocative) tests. Radiographic imaging should be reserved for chronic knee pain (more than six weeks) or acute traumatic pain in patients who meet specific evidence-based criteria. Musculoskeletal ultrasonography allows for detailed evaluation of effusions, cysts (e.g., Baker cyst), and superficial structures. Magnetic resonance imaging is rarely used for patients with emergent cases and should generally be an option only when surgery is considered or when a patient experiences persistent pain despite adequate conservative treatment. When the initial history and physical examination suggest but do not confirm a specific diagnosis, laboratory tests can be used as a confirmatory or diagnostic tool."}, {"id": "pubmed23n0772_19934", "title": "An Intra-tendonous ganglion cyst causing impingement between the anterior cruciate ligament and anterior root of the medial meniscus: a case report.", "score": 0.009900990099009901, "content": "There are several reports of symptomatic ganglion cysts near the anterior cruciate ligament (ACL), posterior cruciate ligament (PCL), and lateral and medial meniscus, but symptomatic ganglia arising from the anterior horn of the medial meniscus to the ACL have not been reported. Here we report the arthroscopic resection of a ganglion cyst arising from the anterior horn of the medial meniscus with a meniscal tear to the ACL. A 43-year-old female presented with a 10-year history of continuous aching pain in the right knee, but without any history of trauma. Clinical examination revealed right-sided knee pain in the medial joint line, exacerbated by end range flexion and extension, a -10°-100° active range of movement, and a -5°-110° passive range of movement。McMurray's, patellar compression, and compression rotation tests were positive. Magnetic resonance imaging (MRI) and arthroscopic examination revealed a cyst related to the ACL and medial meniscus. Histological examination confirmed the cyst to be a ganglion cyst. We present a new type of ganglion cyst, this is the first reported case of an ganglion cyst impinged between the ACL and the medial meniscus. It is hoped that this study will provide a better understanding of the condition and lead to better diagnosis and treatment."}, {"id": "pubmed23n0773_16081", "title": "[Knee pain: choosing the right imaging].", "score": 0.009900990099009901, "content": "Gonalgia is a frequent reason for consultation of a primary care physician. The road leading to diagnosis is mainly clinical. A detailed medical history and physical examination are capital for establishing diagnostic hypotheses and choosing the most appropriate imaging test. Initially, a simple X-ray of the knee joint is the most common exam, even though it is not always needed, especially after a minor trauma. MRI and CT-scan allow a more detailed examination of the structures; however, they should only be ordered to answer a specific question. Most of the time, echography is reserved to extra-articular pathologies and for guiding an articular tap."}, {"id": "InternalMed_Harrison_26032", "title": "InternalMed_Harrison", "score": 0.009890563700185834, "content": "Additional imaging techniques may possess greater diagnostic sensitivity and facilitate early diagnosis in a limited number of articular disorders and in selected circumstances and are indicated when conventional radiography is inadequate or nondiagnostic (Table 393-5). Ultrasonography is useful in the detection of soft tissue abnormalities, such as tendinitis, tenosynovitis, enthesitis, bursitis, and entrapment neuropathies. Wider use, lower cost, better technology, and enhanced site-specific transducers now allow for routine use in outpatient care. Owing to low cost, portability, and wider use, ultrasound use has grown and is the preferred method for the evaluation of synovial (Baker’s) cysts, rotator cuff tears, tendinitis and tendon injury, and Approach to Articular and Musculoskeletal Disorders Strongly consider synovial fluid aspiration and analysis if there is Trauma with joint effusion Monarthritis in a patient with chronic polyarthritis"}, {"id": "article-26373_12", "title": "Osteoarthritis -- Evaluation", "score": 0.009870724251583926, "content": "A thorough history and physical exam (with a focused musculoskeletal exam) should be performed on all patients, with some findings summarized above. OA is a clinical diagnosis and can be diagnosed with confidence if the following are present: 1) pain worse with activity and better with rest, 2) age more than 45 years, 3) morning stiffness lasting less than 30 minutes, 4) bony joint enlargement, and 5) limitation in range of motion. A differential diagnosis should include rheumatoid arthritis, psoriatic arthritis, crystalline arthritis, hemochromatosis, bursitis, avascular necrosis, tendinitis, radiculopathy, among other soft tissue abnormalities. [9] [10]"}, {"id": "pubmed23n0728_10812", "title": "Arthroscopic management of calcific tendonitis of the medial collateral ligament.", "score": 0.00980392156862745, "content": "Calcific tendinitis most commonly occurs to the shoulder, but may also involve other structures of the locomotor system. It is a rare cause of knee pain. We report a 46-year-old woman with severe medial knee pain and limitation of movement in her right knee. There was a marked tenderness site at the proximal insertion of the medial collateral ligament (MCL). Flexion was able to provoke the painful symptoms in the medial knee. The involvement of differentiated diagnoses were excluded by history, laboratory and radiograph examinations, while X-ray, CT and MRI suggested calcific tendonitis of the MCL. Due to the failure of conservative treatments, we offered her arthroscopic excision of calcific deposit which was sent for biopsy. Histopathological evaluation confirmed the diagnosis of calcific tendinitis. This patient recovered shortly afterwards with immediate resolution of symptoms following excision. Thus far, calcifications involving the MCL have been documented thrice. Calcific tendonitis of the MCL diagnosed and treated by arthroscopy has not previously been reported, which can be challenging to diagnose and treat because of its rarity. Although conservative treatment appears to be frequently satisfactory, arthroscopic excision may be a better option for the refractory or severe cases."}, {"id": "wiki20220301en014_26817", "title": "Patella", "score": 0.00980392156862745, "content": "Etymology The word patella originated in the late 17th century from the diminutive form of Latin or or paten, meaning shallow dish. See also Patellar reflex Knee pain Osteoarthritis Lateral retinaculum Lateral release References External links Knee Sesamoid bones Bones of the lower limb"}, {"id": "pubmed23n0774_13662", "title": "[The \"Oxford\" unicondylar knee prostesis (UCP): 21 reviewed cases].", "score": 0.009708737864077669, "content": "The unicompartimental knee prosthesis known as \"Oxford\" is a non constraint prosthesis, entrusting the whole of its stability to an intact ligamentary apparatus. Where the support surfaces of most prostheses remain limited, even punctiform, the originality of the Goodfellow prosthesis lies in the fact that the prosthetic condyle, whatever the flexion angle is, leans against a mobile prosthetic meniscus with spheric superior concavity of the same radius as the condylian radius, which increases considerably the prosthetic leaning surfaces and therefore lessens the pressure constraints. The superior surface, concave, of this prosthetic meniscus takes charge of the rolling, where the inferior plane surface realizes the gliding on the metallic tibial plate. The total conformity of the components minimizes the forces of friction.Between July 1988 and March 1993, 24 patients underwent the placing of UCP. Three patients died and 2 were lost of sight. 19 patients could be seen again or checked, corresponding of 21 operated knees. Two knees benefited from the start from UCP (medial and lateral) and 2 knees had a UCP in the first instance and then a second UCP in the compartment left safe primarily. For the 21 UCP, there are 16 medial and 3 lateral. Our mean drawback is of 3 years and 3 months, all the drawbacks being superior to 1 year and 4 months. The mean age is of 64 years. There were 17 female and 2 male patients. The mean weight is of nearly 80 kg (79,8) and nearly 52% of the operated patients have an important overweight (Body Mass Index superior to 30). Preoperative clinical analysis. It is based on a retrospective study of files using the quotation described by AUBRIOT for the «GUEPAR» group. This one establishes a gradation of four levels for each of the three criteria retained (Pain, Mobility, Instability), thus determining a global result imposed by the lowest level retained.For walking, other factors than just the state of the operated knee may intervene, this being the reason why it doesn't show in this chart. The GUEPAR group quantifies it with letters A, B, C, D.Concerning pain, all 21 knees were quoted as \"Bad\" in preoperative. Pain constitutes the decisive argument for the operative indication. In our series, only one knee had an average amplitude, all the others had a mobility superior to 89°. In 5 cases there was a flessum between 11 and 20° (penalizing of a level). Concerning walking and stability, they were taken into account, thanks to a precise questionnaire about the daily life acts. Concerning the walking perimeter, it was found as unlimited (A) in 1 case, superior to 500 m (B) in 2 cases, inferior to 500 m (C) in 17 cases and limited to home (D) in 1 case. The early after effects. At the end of the intervention, the knee is placed into a splint with limited flexion. As soon as the second day the patient is sat on the border of his bed. The first partial support at the third of the body weight is authorized between the fourth and the fifth day, when at the same time flexion exercises on electrical splint are started, as soon as the Redon draining is removed. The average hospitalization length was of a fortnight. Among secondary late complications and retakes, let us stop on meniscal luxations which constitute a specific complication of the Oxford arthroplasty. They concern 3 times the medial compartment and 4 lateral compartment. They happened in 1 case early, at D 22, in 3 cases within the 6 first months and in 3 cases after 2 years. They were treated : 3 times by reduction under general anæsthetic, no more ; 3 times changing the meniscusus for a meniscusus of superior size and once by placing a total prosthesis at the place of the UCP. The deteriorations of the opposed compartment not prosthesized occured in three cases. They were treated by unicompartmental additional arthroplasty in two cases and by total prosthesis in the third case. The clinical results on pain are very satisfactory as from the early check up onwards we have 17 successes (no pain 11 cases and occasional pains 6 cases) and as after 3 years and 5 months in average, we have 19 successes (no pain : 10 cases - occasional pain : 9 cases). At the maximal drawback, the mobility is quoted very good in 7 cases and good in 13 cases, mean in 1 case. At the latest check up, we note an excellent stability in 17 cases and good in 3 cases, that is to say 20 successes and 1 case of stability quoted as mean. At the latest check up we note 17 successes (A and B) and 4 relative failures (C) concerning the quality of walking.At the question «are you pleased with the intervention and would you advise it to a friend?» and with the nuance «very pleased» and «simply satisfied», we get 10 cases «very pleased», 8 cases «pleased» and 3 cases «moderately satisfied»; only those 3 cases advise against the intervention. The radiological results are less satisfying as they show frequent imperfections : • for the 16 medial UCP : only 9 cases hypocorrected or normo axed, but 1 case strongly hypocorrected (residual varus of 7°) and 6 hypercorrected cases. • for the 5 lateral UCP : 3 normo-axed cases, 1 case strongly hypocorrected (residual valgus of 6°) and 1 case strongly hypercorrected (10° varus). • the failures due to rapid deterioration of the non prosthetized compartment occurred on hypercorrected knees. • on 21 knees, 14 borders of tibial plate were noticed, out of which 9 had no plate displacement and 5 had a slight displacement, at the origin of a small angular loss. • accumulations of cement on the tibial side, towards the back or in medial were noticed in 8 cases, which explains a slope of the tibial plate to the back inferior to 5° in 11 cases (should be of 7°). • 4 femoral components seem to be too posterior and one shows curved.In total, only 7 cases out of 21 were estimated with no peculiarities on the radiological point of view. It seems difficult to place a UCP well. The meniscal luxations are favored by an alignment rotational defect of the tibial plate, specially for the lateral UCP, the meniscus coming to hit the lip of the tibial plate during the lifting from a sitting position. For 5 of these luxations, we must recognize the existence of a ligamentary collateral laxity which should have altered the surgical indication either to an osteotomy, or to a total arthroplasty. Conclusions. Under the condition of respecting the absolute counter indications, of thoroughly evaluating the relative counter indications and of reducing at the best the defects linked to the surgical technique, the unicompartmental arthroplasty, including that of Oxford, gives good functional results after more than three years. In our series, the result on pain is constant if we exclude the cases with risk with ligamentary laxity and that of centered gonarthrosis at obese subject, that is to say 15 successes on 15 knees thus selected retrospectively. The gain on mobility is weak, of 5° in average. The result on stability is, as for pain, excellent, if we exclude the cases with risk, as we get then also 15 successes on 15 knees. Concerning the global result according to the quotation of Aubriot-Guepar, we note 14 successes and 1 relative failure. 4 knees were bad indications and should have benefited from a total arthroplasty or from an osteotomy. "}, {"id": "pubmed23n0852_20928", "title": "Acute leg pain with suspected beginning leg compartment syndrome and deep vein thrombosis as differential diagnoses in an unusual presentation of Brodie's abscess: a case report.", "score": 0.009615384615384616, "content": "Brodie's abscess is an uncommon form of subacute osteomyelitis where the main presenting symptom is mild to moderate pain of insidious onset for several months' duration. We report a case of a patient presenting with acute leg pain resembling that of a deep vein thrombosis, and a beginning leg compartment syndrome following a suspected ruptured Baker's cyst. Our case is unusual because of the acute presentation of the Brodie's abscess with acute leg pain and acute swelling without any preceding trauma; to the best of our knowledge, this presentation has not been reported before. A 17-year-old white boy presented to our out-patient clinic with a 6-month history of pain in his left knee joint of insidious onset. There was no history of trauma to the extremity. After performing physical and radiological (X-ray) examinations, we initially diagnosed medial meniscus damage. One week later he presented to our emergency department with acute sudden increase in the pain and swelling of his left knee, and pain and swelling of his left leg, without any trauma. Deep vein thrombosis and beginning leg compartment syndrome from ruptured Baker's cyst were initially diagnosed. Magnetic resonance imaging was performed and Brodie's abscess was the most probable diagnosis. We performed open surgical debridement and curettage with drainage of the abscess and administered postoperative antibiotics. He presented to our out-patient clinic 3 months postoperatively, where he was pain-free with no residual local tenderness. In cases of sudden acute increase in joint or extremity pain or swelling that has been insidiously present for months, Brodie's abscess should be considered as one of the differential diagnoses, as it may present acutely in cases with accompanying fasciitis and myositis and be clinically mistaken for deep vein thrombosis or limb compartment. Magnetic resonance imaging remains the gold standard imaging study, and surgical treatment followed by postoperative antibiotics remains the standard treatment."}, {"id": "pubmed23n1146_16199", "title": "Popliteus Tendon Injuries.", "score": 0.009615384615384616, "content": "Popliteus tendinopathies are rare injuries that can occur from overuse, trauma, or secondary causes, such as sesamoid bones or calcifications. They present with nonspecific symptoms and should be considered in any patient with posterolateral knee pain, instability, popliteus tenderness, and a positive Garrick test. Diagnosis can be made with magnetic resonance imaging, but arthroscopy remains the criterion standard. For minor popliteus tendinopathies, initial management involves conservative treatment, including rest, activity modification, physical therapy, and quadriceps strengthening. For more severe or refractory disease, corticosteroid injections and arthroscopy should be considered. [<iOrthopedics</i. 20XX;XX(X):xx-xx.]."}, {"id": "pubmed23n0553_3822", "title": "Deep vein thrombosis in an athletic military cadet.", "score": 0.009523809523809525, "content": "Resident's case problem. A 21-year-old healthy athletic male military cadet with complaint of worsening diffuse left knee pain was evaluated 4 days after onset. The knee pain began 2 hours after completing a long car trip, worsened over the subsequent 3 days, and became almost unbearable during the return trip. The patient reported constant pain, limited knee motion, and difficulty ambulating. In addition, he was unable to perform physical military training or attend academic classes due to the severe left knee pain. Past medical history revealed a mild left lateral calf strain 21/2 weeks prior, which completely resolved within 24 hours of onset. Our physical examination led us to either monoarticular arthritis, pseudothrombophlebitis (ruptured Baker's cyst), or a lower leg deep vein thrombosis (DVT) as the cause of knee pain. Diagnostic imaging of this patient revealed a left superficial femoral vein thrombosis and popliteal DVT, with bilateral pulmonary emboli (PE). A systematic differential diagnosis was undertaken to rule out a potentially fatal DVT diagnosis as the cause of knee pain, despite minimal DVT risk factors. The physical therapist in a direct-access setting must ensure timely evaluation and referral of a suspected DVT, even when patient demographics cause the practitioner to question the likelihood of this diagnosis. The physical examination findings, clinical suspicion, and established clinical prediction rules can accurately dictate the appropriate referral action necessary."}, {"id": "InternalMed_Harrison_26263", "title": "InternalMed_Harrison", "score": 0.00945945945945946, "content": "Periarticular Disorders of the Extremities 2248 by hip extension and flexion. Anserine bursitis is an inflammation of the sartorius bursa located over the medial side of the tibia just below the knee and under the conjoint tendon and is manifested by pain on climbing stairs. Tenderness is present over the insertion of the conjoint tendon of the sartorius, gracilis, and semitendinosus. Prepatellar bursitis occurs in the bursa situated between the patella and overlying skin and is caused by kneeling on hard surfaces. Gout or infection may also occur at this site. Bursitis is typically diagnosed by history and physical examination, but visualization by ultrasound may play a useful role in selected instances for diagnosis and directed guidance of glucocorticoid injection. Treatment of bursitis consists of prevention of the aggravating situation, rest of the involved part, administration of a nonsteroidal anti-inflammatory drug (NSAID) where appropriate for an individual patient, or local"}, {"id": "pubmed23n0927_17526", "title": "In-office arthroscopy for the evaluation of chronic knee pain: A case report.", "score": 0.009433962264150943, "content": "This is a case report detailing the use of in-office needle arthroscopy (mi-eye 2™) in a patient with chronic knee pain and inconclusive magnetic resonance imaging findings. The patient is a 40-year-old male who presented to our clinic after an extended history of right knee pain along the medial aspect with previous failed treatments. Magnetic resonance imaging without contrast had demonstrated full-thickness chondral fissuring of the lateral patellar facet, mild abnormal signals of the proximal patellar tendon and Hoffa's fat pad, and intact anterior cruciate ligament and posterior cruciate ligament. The patient was previously treated with an ultrasound-guided injection of 2 cm<sup3</sup of 1% lidocaine without epinephrine and 1 cm<sup3</sup of Kenalog-40 and scheduled for follow-up. At follow-up, clinical examination showed antalgic gait, minimal tenderness along medial joint line, medial pain in deep flexion, and no pain when in varus or valgus. Due to continued discomfort with a negative magnetic resonance imaging, in-office diagnostic arthroscopy was performed using mi-eye 2 revealing a tear of the mid-body of the medial meniscus. The patient subsequently underwent arthroscopic repair and is recovering well with complete resolution of medial joint pain. This report highlights the clinical utility of in-office diagnostic arthroscopy in the management of patients with persistent knee pain and negative or equivocal findings on magnetic resonance imaging."}, {"id": "pubmed23n0783_2067", "title": "[Diagnosis. History and physical examination].", "score": 0.009433962264150943, "content": "Family physicians play a key role in the diagnosis and management of patients with osteoarthritis. Diagnosis is mainly clinical and radiological. A complete history should be taken with meticulous physical examination of the joints. The history-taking should aim to detect risk factors and compatible clinical symptoms. Pain characteristics should be identified, distinguishing between mechanical and inflammatory pain, and an exhaustive examination of the joints should be performed, with evaluation of the presence of pain, deformity, mobility restrictions (both active and passive), crepitus, joint effusion, and inflammation. A differential diagnosis should be made with all diseases that affect the joints and/or produce joint stiffness. "}, {"id": "pubmed23n0862_5149", "title": "[Tarsal tunnel syndrome secondary to venous insufficiency. Case report].", "score": 0.009345794392523364, "content": "Tarsal tunnel syndrome is defined as an extrinsic and/or intrinsic compressive neuropathy of the posterior tibial nerve or one of its branches. Its causes include venous insufficiency. Clinical case: 51 year-old female patient from León, Guanajuato. Hypertensive, with Guillain-Barré syndrome for eight years, vascular insufficiency and obesity. Her condition started with left ankle and heel pain; she was treated with NSAIDs and rehabilitation and achieved partial improvement. X-rays and MRI of the left ankle showed posterior impingement. She underwent arthroscopy and improved but one month later she presented with severe pain in the left ankle and sole and dysesthesias. Electromyography showed a lesion of the posterior tibial nerve. We had the patient's case history, preoperative tests, and dorsoplantar and lateral X-ray views. The arthroscopic diagnosis was Flexor Hallucis Longus (FHL) tendinitis, synovitis and posterior ankle impingement. Synovectomy, decompression and smoothening of the FHL tendon were performed. The patient did poorly and underwent electromyography with axonotmesis of the medial plantar branch. After the nerve was released, Lazorthes venous plexus was found to be tortuous and compressing the entire nerve tract. The possible causes for this include intrinsic compression secondary to tumors, and anatomical changes of the tarsal tunnel. However, less often varices may confound the diagnosis and cause irreversible damage if not treated timely. The patient is currently pain free and can walk, has mild dysesthesias of the first toe and limited flexion."}, {"id": "pubmed23n0666_18896", "title": "Treating knee pain: history taking and accurate diagnoses.", "score": 0.009345794392523364, "content": "Prompt and effective diagnosis and treatment for common knee problems depend on practitioners' ability to distinguish between traumatic and inflammatory knee conditions. This article aims to enable practitioners to make accurate assessments, carry out knee examinations and undertake selected special tests as necessary before discharging or referring patients."}, {"id": "pubmed23n0916_3153", "title": "[Arthroscopic Finding of Knee Joint in Relation to Age and Its Comparison with Pre-Operative Clinical Finding - a Retrospective Study].", "score": 0.009259259259259259, "content": "PURPOSE OF THE STUDY In the retrospective study of two South Bohemian centres we present the comparison of pre-operative anamnestic clinical signs in relation to the arthroscopic intraoperative finding. The obtained data is used also to evaluate the arthroscopic finding in relation to age and sex. MATERIAL AND METHODS The arthroscopic findings of patients who underwent surgery in 2013-2014 period (1.1.2013-31.12.2014) at the Department of Trauma Surgery of České Budějovice Hospital, a.s. and in 2014 (1.1.-31.12.2014) at the Department of Orthopaedics and Traumatology of Písek Hospital, a.s. were evaluated. In total, 1 021 patients underwent surgery, with the mean age of 44 years. The patients were not selected. The group includes all the patients who underwent surgery, including those in whom repeat arthroscopy was performed, in the respective period of time, regardless of the mechanism of difficulties. A preoperative MRI scan was carried out in 470 patients. The referring physician was present during the examination. In all the patients undergoing surgery, the main clinical preoperative sign was examined based on the documentation, namely in the following order - hemarthros, locked knee, hydrops or merely a pain. In the arthroscopic finding, the medial meniscal lesion - anterior and posterior horn, and complete tear was assessed. The same was done for lateral meniscus. In anterior cruciate ligament - ACL - partial or complete tear was assessed. We identified the frequency of findings in relation to age and evaluated the correlations between the clinical signs and the arthroscopic finding. We calculated the sensitivity and specificity of hemarthros as a sign of ACL tear. The analysis was conducted based on the medical history in medical record documentation and the surgical protocol. The cartilage was not assessed. RESULTS Analysis of clinical and anamnestic signs in relation to arthroscopic findings 1. Negative arthroscopic findings (potential cartilage damage with no damage to other soft structures and normal arthroscopic findings) are in 83% accompanied by a mere knee pain. 2. High percentage of isolated locked joint (15%) in negative findings 3. Complete ACL tears are most frequently reported in the under-35 age category - 43% of 191 men who underwent surgery and 33% of 102 women. 4. Isolated injuries to ACL without the meniscus tear are frequent in younger patients - 30% - 40% of the total number of patients with injured ACL. 5. In patients older than 56 years of age the ACL damage is accompanied by concurrent meniscus tear (96% in men, 100% in women). 6. Sensitivity of hemarthros (68%) for complete ACL tear. Specificity of the presence of hemarthros in complete ACL lesions (91%) indicates that there are also complete ACL tears with no hemarthros whatsoever in the medical history. For partial tears the values of sensitivity and specificity are 27% and 67%, respectively. In partial tear, the presence of hemarthros is not a diagnostic lead. 7. In 15% of negative findings a \"locked knee\" was present. It was not a genuinely locked knee, but rather an antalgic position. Not every locked knee must necessarily mean a meniscus lesion or ACL tear. 8. Isolated meniscus tear is in 75% accompanied only by pain. 9. In our group of patients, isolated osteoarthrosis or malacic cartilage without any damage to ligaments or menisci was rare - only in 22 cases (2% of the entire group). DISCUSSION There are lots of studies which focus on comparing the clinical findings with perioperative pathology of knee joint and the importance of pre-operative clinical examination. Our extensive retrospective study proved that in 56-plus age category virtually each ACL injury is accompanied by a meniscal lesion, which can be explained by a possible ACL damage at a young age and subsequent instability resulting in meniscus tear or frequent presence of degenerative meniscal changes at an older age. A small number of isolated degenerative cartilage damage was established (2%). We fully agree with the authors who prove that the degenerative cartilage changes are ever since the very beginning accompanied by changes of the other soft structure of the knee. We revealed a high percentage of locked knee joint in negative arthroscopic findings. According to the clinical pre-operative examination, the locked knee does not automatically mean the meniscal lesion or ACL tear. In agreement with the others we prove a close association between hemarthros and ACL injury. CONCLUSIONS 1. A clinical examination, a detailed medical history is necessary 2. With hemarthros in medical history, there is a likelihood of complete ACL tear. Conversely, even a seemingly trivial knee sprain without hemarthros or locked knee can mean the ACL tear. 3. Where a mere pain is present, it mostly indicates an isolated meniscal damage or a negative finding. 4. Degenerative cartilage changes are accompanied by degeneration of menisci and ligaments. 5. Our group of patients did not include any case of hemarthros in the medical history with a negative arthroscopic finding. Hemarthros always indicated a more serious damage to knee soft structures. Key words: knee joint injuries, knee arthroscopy, sensitivity, specificity, hemarthros."}, {"id": "article-23827_11", "title": "Jumpers Knee -- History and Physical", "score": 0.009259259259259259, "content": "Patellar tendinopathy is mainly a clinical diagnosis made through a detailed history and meticulous physical examination. Appropriate questions which will cue in the diagnosis: Sport practiced, schedule of practice and competition, which position the athlete plays, and level of performance. The patient will usually complain of well-localized pain and tenderness on the inferior tip of the patella. [12] [2]"}, {"id": "InternalMed_Harrison_26013", "title": "InternalMed_Harrison", "score": 0.009245319436402239, "content": "Approach to Articular and Musculoskeletal Disorders 2222 application of manual pressure lateral to the patella may cause an observable shift in synovial fluid (bulge) to the medial aspect. The examiner should note that this maneuver is only effective in detecting small to moderate effusions (<100 mL). Inflammatory disorders such as RA, gout, pseudogout, and psoriatic arthritis may involve the knee joint and produce significant pain, stiffness, swelling, or warmth. A popliteal or Baker’s cyst may be palpated with the knee partially flexed and is best viewed posteriorly with the patient standing and knees fully extended to visualize isolated or unilateral popliteal swelling or fullness. Anserine bursitis is an often missed periarticular cause of knee pain in adults. The pes anserine bursa underlies the insertion of the conjoined tendons (sartorius, gracilis, semitendinosus) on the anteromedial proximal tibia and may be painful following trauma, overuse, or inflammation. It is often"}, {"id": "pubmed23n0739_20976", "title": "Arthroscopic lavage and debridement for osteoarthritis of the knee: an evidence-based analysis.", "score": 0.009174311926605505, "content": "The purpose of this review was to determine the effectiveness and adverse effects of arthroscopic lavage and debridement, with or without lavage, in the treatment of symptoms of osteoarthritis (OA) of the knee, and to conduct an economic analysis if evidence for effectiveness can be established.  QUESTIONS ASKED: Does arthroscopic lavage improve motor function and pain associated with OA of the knee?Does arthroscopic debridement improve motor function and pain associated with OA of the knee?If evidence for effectiveness can be established, what is the duration of effect?What are the adverse effects of these procedures?What are the economic considerations if evidence for effectiveness can be established? Osteoarthritis, the most common rheumatologic musculoskeletal disorder, affects about 10% of the Canadian adult population. Although the natural history of OA is not known, it is a degenerative condition that affects the bone cartilage in the joint. It can be diagnosed at earlier ages, particularly within the sports injuries population, though the prevalence of non-injury-related OA increases with increasing age and varies with gender, with women being twice as likely as men to be diagnosed with this condition. Thus, with an aging population, the impact of OA on the health care system is expected to be considerable. Treatments for OA of the knee include conservative or nonpharmacological therapy, like physiotherapy, weight management and exercise; and more generally, intra-articular injections, arthroscopic surgery and knee replacement surgery. Whereas knee replacement surgery is considered an end-of-line intervention, the less invasive surgical procedures of lavage or debridement may be recommended for earlier and more severe disease. Both arthroscopic lavage and debridement are generally indicated in patients with knee joint pain, with or without mechanical problems, that are refractory to medical therapy. The clinical utility of these procedures is unclear, hence, the assessment of their effectiveness in this review.  LAVAGE AND DEBRIDEMENT: Arthroscopic lavage involves the visually guided introduction of saline solution into the knee joint and removal of fluid, with the intent of extracting any excess fluids and loose bodies that may be in the knee joint. Debridement, in comparison, may include the introduction of saline into the joint, in addition to the smoothening of bone surface without any further intervention (less invasive forms of debridement), or the addition of more invasive procedures such as abrasion, partial or full meniscectomy, synovectomy, or osteotomy (referred to as debridement in combination with meniscectomy or other procedures). The focus of this health technology assessment is on the effectiveness of lavage, and debridement (with or without meniscal tear resection). THE MEDICAL ADVISORY SECRETARIAT FOLLOWED ITS STANDARD PROCEDURES AND SEARCHED THESE ELECTRONIC DATABASES: Ovid MEDLINE, EMBASE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews and The International Network of Agencies for Health Technology Assessment. THE KEYWORDS SEARCHED WERE: arthroscopy, debridement, lavage, wound irrigation, or curettage; arthritis, rheumatoid, osteoarthritis; osteoarthritis, knee; knee or knee joint. TIME FRAME: Only 2 previous health technology assessments were identified, one of which was an update of the other, and included 3 of 4 randomized controlled trials (RCTs) from the first report. Therefore, the search period for inclusion of studies in this assessment was January 1, 1995 to April 24, 2005. EXCLUDED WERE: case reports, comments, editorials, and letters. Identified were 335 references, including previously published health technology assessments, and 5 articles located through a manual search of references from published articles and health technology assessments. These were examined against the criteria, as described below, which resulted in the inclusion of 1 health technology assessment and its corresponding update, and 4 articles (2 RCTs and 2 level 4 studies) for arthroscopic lavage and 8 papers (2 RCTs and 6 level 4 studies) for arthroscopic debridement. English-language articles from PubMed, EMBASE, Cochrane Systematic Reviews, and health technology assessments from January 1, 1995 onwardStudies on OA of the knee with a focus on the outcomes of motor function and painStudies of arthroscopic procedures onlyStudies in which meniscal tear resection/meniscectomy (partial or full) has been conducted in conjunction with lavage or debridement. Studies that focus on inflammatory OA, joint tuberculosis, septic joints, psoriatic joints (e.g., psoriatic knee joint synovitis), synovitis, chondropathy of the knee and gonarthrosis (which includes varotic gonarthrosis)Studies that focus on rheumatoid arthritisStudies that focus on meniscal tears from an acute injury (e.g., sports injury)Studies that are based on lavage or debridement for microfracture of the kneeStudies in which other surgical procedures (e.g., high tibial osteotomy, synovectomy, have been conducted in addition to lavage/debridement)Studies based on malalignment of the knee (e.g., varus/valgus arthritic conditions).Studies that compare lavage to lavage plus drug therapyStudies on procedures that are not arthroscopic (i.e., visually guided) (e.g., nonarthroscopic lavage)Studies of OA in children. Arthroscopic lavage or debridement, with or without meniscectomy, for the treatment of motor function symptoms and pain associated with OA of the knee. Studies in which there was a comparison group of either diseased or healthy subjects or one in which subjects were their own control were included. Comparisons to other treatments included placebo (or sham) arthroscopy. Sham arthroscopy involved making small incisions and manipulating the knee, without the insertion of instruments. IN EARLY OA OF THE KNEE WITH PAIN REFRACTORY TO MEDICAL TREATMENT, THERE IS LEVEL 1B EVIDENCE THAT: Arthroscopic lavage gives rise to a statistically significant, but not clinically meaningful effect in improving pain (WOMAC pain and VAS pain) up to 12 months following surgery. The effect on joint function (WOMAC function) and the primary outcome (WOMAC aggregate) was neither statistically nor clinically significant. IN MODERATE OR SEVERE OA OF THE KNEE WITH PAIN REFRACTORY TO MEDICAL TREATMENT, THERE IS: Level 1b evidence that the effect on pain and function of arthroscopic lavage (10 L saline) and debridement (with 10 L saline lavage) is not statistically significant up to 24 months following surgery.Level 2 evidence that arthroscopic debridement (with 3 L saline lavage) is effective in the control of pain in severe OA of the medial femoral condyle for up to 5 years.For debridement in combination with meniscectomy, there is level 4 evidence that the procedure, as appropriate, might be effective in earlier stages, unicompartmental disease, shorter symptom duration, sudden onset of mechanical symptoms, and preoperative full range of motion. However, as these findings are derived from very poor quality evidence, the identification of subsets of patients that may benefit from this procedure requires further testing.In patients with pain due to a meniscal tear, of the medial compartment in particular, repair of the meniscus results in better pain control at 2 years following surgery than if the pain is attributable to other causes. There is insufficient evidence to comment on the effectiveness of lateral meniscus repair on pain control. Arthroscopic debridement of the knee has thus far only been found to be effective for medial compartmental OA. All other indications should be reviewed with a view to reducing arthroscopic debridement as an effective therapy. Arthroscopic lavage of the knee is not indicated for any stage of OA. There is very poor quality evidence on the effectiveness of debridement with partial meniscectomy in the case of meniscal tears in OA of the knee."}, {"id": "InternalMed_Harrison_26084", "title": "InternalMed_Harrison", "score": 0.009174311926605505, "content": "OA is the most common cause of chronic knee pain in persons over age 45, but the differential diagnosis is long. Inflammatory arthritis is likely if there is prolonged morning stiffness and many other joints are affected. Bursitis occurs commonly around knees and hips. A physical examination should focus on whether tenderness is over the joint line (at the junction of the two bones around which the joint is articulating) or is outside of it. Anserine bursitis, medial and distal to the knee, is an extremely common cause of chronic knee pain that may respond to a glucocorticoid injection. Prominent nocturnal pain in the absence of end-stage OA merits a distinct workup. For hip pain, OA can be detected by loss of internal rotation on passive movement, and pain isolated to an area lateral to the hip joint usually reflects the presence of trochanteric bursitis. No blood tests are routinely indicated for workup of patients with OA unless symptoms and signs suggest inflammatory arthritis."}, {"id": "pubmed23n0480_5021", "title": "Juxta-articular myxoma: a rare cause of painful restricted motion of the knee.", "score": 0.00909090909090909, "content": "A 68-year-old athletic woman presented to our institution in January 2002 with a several-month history of progressing complaints of pain, swelling, and loss of motion in the right knee. These manifestations had begun the previous July during a game of tennis. She experienced persisting pain and recurring effusions. Because the patient had been residing in another state between July and January, rheumatologic and orthopaedic evaluations of the knee, including a magnetic resonance imaging (MRI), had been performed at a geographically distant (but affiliated) institution. The resulting presumptive diagnosis was a \"wear and tear\" degenerative articular disorder of the knee. A program of anti-inflammatory medication and physical therapy was begun for several months but produced no therapeutic benefit by the time the patient presented at our institution. After examination confirmed marked losses of both flexion and extension of the knee, effusion, and exquisite medial joint tenderness, an MRI was repeated, using intra-articular gadolinium as a contrast agent. It revealed an intra-articular mass encircling the medial and posterior extents of the medial femoral condyle. An arthroscopic multiportal excisional biopsy was performed. It revealed the existence of a juxta-articular myxoma. The patient recovered most of the range of motion during the next several months, and the effusion and severe pain gradually dissipated. The patient was subsequently followed by sequential physical examinations and MRIs, performed at increasing intervals of time, without recurrence of a mass or of her flagrant symptoms in the first year post surgery. Though the patient's diagnosis was established and treatment outcome was satisfactory, many issues were brought up in this case regarding most appropriate selection of diagnostic tests and treatment approaches."}, {"id": "InternalMed_Harrison_25994", "title": "InternalMed_Harrison", "score": 0.00909090909090909, "content": "Approach to Articular and Musculoskeletal Disorders 2220 causes, can be assessed by inspection and palpation. Joint swelling or volume can be assessed by palpation. Distention of the articular capsule usually causes pain and evident enlargement or fluctuance. The patient will attempt to minimize the pain by maintaining the joint in the position of least intraarticular pressure and greatest volume, usually partial flexion. For this reason, inflammatory effusions may give rise to flexion contractures. Clinically, this may be detected as fluctuant or “squishy” swelling in larger joints and grape-like compressibility in smaller joints. Inflammation may result in fixed flexion deformities or diminished range of motion—especially on extension, when intraarticular pressure is increased. Active and passive range of motion should be assessed in all planes, with contralateral comparison. A goniometer may be used to quantify the arc of movement. Each joint should be passively manipulated through"}, {"id": "pubmed23n0667_4515", "title": "Short-term results of the Oxford phase 3 unicompartmental knee arthroplasty for medial arthritis.", "score": 0.009009009009009009, "content": "We evaluated short-term results of the Oxford phase 3 unicompartmental knee arthroplasty (UKA) in patients with medial compartment arthritis. The study included 38 patients (28 females, 10 males; mean age 67 years; range 56 to 75 years) who underwent UKA for isolated medial knee osteoarthritis. At the time of surgery, 28 patients were in the age group of 56-64 years, and 10 patients were in the age group of 65-75 years. All the patients had Ahlbäck grade 2 primary medial compartment arthritis that had been unresponsive to conservative treatment. None of the patients had symptoms of patellofemoral arthrosis. Patients underwent UKA with the Oxford phase 3 cemented meniscal-bearing unicondylar prosthesis using minimally invasive surgery. The results were assessed preoperatively and at final controls according to the Knee Society clinical and functional rating system. Postoperative radiographic evaluations were made according to the Oxford criteria. The mean follow-up period was 24 months (range 18 to 32 months). The mean preoperative active knee flexion increased from 121.8 degrees (range 110 degrees to 130 degrees ) to 130.9 degrees (range 120 degrees to 140 degrees) postoperatively (p&lt;0.05). There was no limitation in knee extension both pre- and postoperatively. The mean preoperative and postoperative knee scores were 64.6 (range 47 to 80) and 97.5 (range 89 to 100), and the mean functional scores were 59.6 (range 45 to 80) and 92.1 (range 70 to 100), respectively (p&lt;0.05). All the patients had an excellent knee score, while functional scores were excellent in 27 patients (71.1%) and good in 11 patients (28.9%). Postoperative radiographic measurements showed that the position of the femoral components was within acceptable ranges in all the patients with a mean of 3 degrees valgus (range 5 degrees valgus to 8 degrees varus) and 0.5 degrees extension (range 3 degrees extension to 2 degrees flexion). The positioning of the femoral components in relation to the mechanical axis was central in 30 patients and 2-mm lateral (range 2 mm medial to 4 mm lateral) in eight patients. The position of the tibial components was also within acceptable ranges in all the patients with a mean of 1.5 degrees varus (range 2 degrees varus to 2 degrees valgus) and a mean posterior inclination of 6.2 degrees (range 5 degrees to 7 degrees). All the tibial components showed full congruency with the medial, lateral, anterior, and posterior planes, except for one which had a 4-mm undersizing in the anterior plane. The polyethylene insert was central and parallel to the tibial component in all the patients. No osteophytes or cement debris that might lead to impingement were observed. All the components remained in position until the final controls. Complications such as insert dislocation, infection, pulmonary embolism, deep venous thrombosis, or neurovascular injury were not observed. None of the patients required revision surgery. Our findings show that, with proper patient selection and strict adherence to the surgical technique, short-term results of the Oxford phase 3 unicompartmental knee prosthesis are excellent or good in the treatment of medial compartment osteoarthritis."}, {"id": "wiki20220301en316_35267", "title": "Ottawa knee rules", "score": 0.009009009009009009, "content": "The Ottawa knee rules are a set of rules used to help physicians determine whether an x-ray of the knee is needed. They state that an X-ray is required only in patients who have an acute knee injury with one or more of the following: Age 55 years or older Tenderness at head of fibula Isolated tenderness of patella Inability to flex the knee greater than 90° Inability to bear weight both immediately and in the emergency department (4 steps)"}, {"id": "pubmed23n1023_13596", "title": "A potential risk factor of total knee arthroplasty: an infected Baker's cyst - a case report.", "score": 0.008928571428571428, "content": "In adults, Baker's cyst development is attributable principally to secondary alterations after degenerative changes. The latter changes often accompany osteoarthritis, and we frequently encounter patients with Baker's cysts seeking total knee arthroplasty (TKA). Baker's cysts are not usually subject to extensive preoperative evaluation because the cysts often disappear naturally after surgery, unaccompanied by any adverse symptoms. A 63-year-old woman presented with moderate pain in the left knee joint that had developed 1 year ago. Posterior knee pain was aggravated on maximum knee flexion. Three months previously, a popliteal mass had become palpable and the patient had undergone needle mass aspiration twice in a local orthopedic hospital, but the mass had recurred. We initially considered TKA for her severe degenerative osteoarthritis. However, we decided to perform only arthroscopic debridement and cyst excision because the patient was experienced severe pain only on maximal knee flexion, and did not want TKA. Pus gushed from the torn cyst during the operation. We diagnosed an infected Baker's cyst. The patient was treated with a first-generation cephalosporin postoperatively. A Baker's cyst that was aspirated and still causes symptoms with altered blood tests needs to be evaluated accurately before TKA."}, {"id": "wiki20220301en106_10744", "title": "OPQRST", "score": 0.008928571428571428, "content": "Severity The pain score (usually on a scale of 0 to 10). Zero is no pain and ten is the worst possible pain. This can be comparative (such as \"... compared to the worst pain you have ever experienced\") or imaginative (\"... compared to having your arm ripped off by an alien\"). If the pain is compared to a prior event, the nature of that event may be a follow-up question. The clinician must decide whether a score given is realistic within their experience – for instance, a pain score 10 for a stubbed toe is likely to be exaggerated. This may also be assessed for pain now, compared to pain at time of onset, or pain on movement. There are alternative assessment methods for pain, which can be used where a patient is unable to vocalise a score. One such method is the Wong-Baker faces pain scale. Time (history)"}, {"id": "pubmed23n0549_174", "title": "Diagnosis of medial knee pain: atypical stress fracture about the knee joint.", "score": 0.008849557522123894, "content": "Resident's case problem. A 19-year-old female, currently enrolled in a military training program, sought medical care for a twisting injury to her right knee. The patient reported her symptoms as similar to an injury she incurred 1 year previously while enrolled in the same military program. The patient's past medical history included a nondepressed fracture of the medial tibial plateau and complete tear of the deep fibers of the medial collateral ligament. Physical exam revealed nonlocalized anterior and medial knee pain without evidence of internal derangement. Initial knee and tibia radiographs were unremarkable. Referral for orthopedic physician evaluation resulted in concurrence with the therapist's diagnosis and plan of care, and the patient was allowed to continue with limited physical training demands. Despite periods of rest, the patient's symptoms progressively worsened upon attempts to resume running. The examining therapist referred the patient for magnetic resonance imaging (MRI) due to the patient's worsening symptoms, normal radiographs, and concern for a proximal tibia stress fracture. MRI revealed a severe proximal tibial metaphysis stress fracture. Stress fractures are commonly encountered injuries in individuals subjected to increased physical training demands. Early evaluation may not yield well-localized findings and may mimic other conditions. Nonmusculoskeletal conditions should be considered in the management of patients with stress fractures. This resident's case problem illustrates the importance of serial physical examinations and collaboration with other healthcare practitioners in the comprehensive assessment and management of a patient with a severe stress fracture."}, {"id": "pubmed23n0877_15238", "title": "[Analysis and comparison about musculoskeletal ultrasonoLranhv and x-rav of knee osteoarthritis].", "score": 0.008849557522123894, "content": "To analyze and compare the characteristics of musculoskeletal ultrasonography and X-ray of knee osteoarthritis, and to investigate the advantages of them. According to the inclusion and exclusion criteria, 57 cases (66 knees) were collected from February 2015 to May 2015. Among them, there were 48 females and 9 males with an average age of (58.9 +/- 9.8) years old (ranged, 41 to 78 years old). The main symptoms included unilateral or bilateral knee pain and locked joints explicit areas of tender points. The mean course of disease was (13.6 +/- 3.0) months. The results of musculoskeletal ultrasound and X-ray examinations were analyzed. According to Kellgren-Lawrence classification of knee joint on the X-ray: the musculoskeletal ultrasound results of patients with I degree synovial hyperplasia in 9 cases, joint effusion in 20 cases, meniscal disease in 13 cases, patellar pad inflammation in 5 cases, and patellar lesion in 8 cases. The musculoskeletal ultrasound results of patients with III degree: synovial hyperplasia in 20 cases,joint effusion in 31 cases, meniscal disease in 22 cases, patellar pad inflammation in 16 cases and patellar lesion in 17 cases. The musculoskeletal ultrasound results of patients with III degree: synovial hyperplasia in 6 cases,joint effusion in 6 cases, meniscal disease in 7 cases, patellar pad inflammation in 7 cases and patellar lesion in 5 cases. The musculoskeletal ultrasound can detect the pathological changes of knee soft tissue sensitively, provide an accurate location of lesions,and find lesions early. The musculoskeletal ultrasound should be applicated in the diagnosis of knee osteoarthritis."}, {"id": "pubmed23n1004_24979", "title": "Arthroscopic debridement for osteoarthritis of the elbow: Results and analysis of predictive factors.", "score": 0.008771929824561403, "content": "Osteoarthritis is the second most frequent cause of elbow stiffness, after trauma sequelae. Surgical treatment mainly consists of debridement. The main aim of the present study was to assess the efficacy of arthroscopic treatment of osteoarthritis of the elbow on Andrews-Carson score. Secondary objectives comprised assessment of the impact of associated procedures and of epidemiological factors on functional results. A prospective multicenter study involving 8 centers, in a symposium held by the French Society of Arthroscopy (SFA), included patients treated by arthroscopy for primary or secondary osteoarthritis of the elbow between January 2017 and March 2018, with a minimum 6 months' follow-up. Clinical assessment was based on change in Andrews-Carson functional score (AC), specific to osteoarthritis of the elbow, and on other functional scores: QuickDash (QD), Patient-Rated Elbow Evaluation (PREE), Mayo Elbow Performance Score (MEPS) and Self-Evaluation Elbow (SEE). Progression in pain on visual analog scale (VAS) and range of motion (RoM) was also assessed. Initial imaging work-up comprised standard X-ray and CT arthrography; paraclinical follow-up was based on X-ray. The impact of the following procedures associated to arthroscopic debridement was analyzed: radial head resection, ulnar nerve release, humeral fenestration, lateral ramp release, and medial collateral ligament posterior bundle release. The functional impact of epidemiological factors (age, handedness, manual occupation, smoking, body-mass index, and work accident/occupational disease status) and radiographic factors (foreign bodies, joint impingement, osteophytes, and fossa filling) was also assessed. The series comprised 87 patients: 75 male (86.2%); mean age, 49 years (range, 18-73 years). Arthroscopic debridement significantly improved all functional scores at a minimum 6 months, and notably the specific AC score: 113.6±25.4 (40-180) versus 178.7±20.2 (110-200) (P&lt;0.0001). Pain diminished significantly: 6.4±2.1 (0-10) versus 1.7±1.8 (0-8) (P&lt;0.0001). RoM increased significantly: flexion/extension, 93.44±20.5° (5-130°) versus 124.2±13.8° (90-160°) (P&lt;0.0001); pronation/supination, 147.6±25.6° (60-180°) versus 162.5±20.6° (100-180°) (P&lt;0.0001). Strength (kg) increased in flexion (8.8±4.0 (4 to 20) versus 15.3±5.1 (3 to 32) (P&lt;0.0008) and in grip [33.1±12.3 (10 to 58) versus 42.1±14.0 (2 to 68) (P&lt;0.0001)]. Epidemiologically, males showed better recovery than females for both pain and strength. There was a significant positive impact of manual work on functional recovery, pain and also strength. There was a significant negative impact of work-accident/occupational disease on pain and strength. Regarding associated procedures, lateral ramp debridement improved AC score, with a gain of 75.4±25.3 points (-5 to 110) vs. 49.6±23.5 (10 to 100) (P&lt;0.0001), and pain on VAS, with a fall of -5.6±2.1 points (-10 to -1) vs. -3.6±3.0 (-8.5 to 1) (P=0.0013). Ulnar nerve release, radial head resection and humeral fenestration had no positive impact. Preoperative foreign body was a factor for good prognosis. Cartilage wear, especially in the humeroulnar compartment, was associated with poorer functional results. Arthroscopic treatment of osteoarthritis of the elbow significantly improved clinical results at 6 months, with significant improvements in functional scores, pain, strength and range of motion. Gender, type of work and work-accident/occupational disease status influenced clinical results. Lateral ramp release is an often overlooked technical factor improving functional results. Radiologically, the best candidates are those presenting with a foreign body and no humeroulnar impingement. III, Prospective observational multicenter cohort study."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[0, 266]], "word_ranges": [[0, 42]], "text": "The history of contact with fresh water in an endemic area points to leptospirosis, and in the clinical picture we also find jaundice and conjunctival injection which also points to leptospirosis and is not so characteristically related to the other three pathogens."}, "2": {"exist": true, "char_ranges": [[0, 266]], "word_ranges": [[0, 42]], "text": "The history of contact with fresh water in an endemic area points to leptospirosis, and in the clinical picture we also find jaundice and conjunctival injection which also points to leptospirosis and is not so characteristically related to the other three pathogens."}, "3": {"exist": true, "char_ranges": [[0, 266]], "word_ranges": [[0, 42]], "text": "The history of contact with fresh water in an endemic area points to leptospirosis, and in the clinical picture we also find jaundice and conjunctival injection which also points to leptospirosis and is not so characteristically related to the other three pathogens."}, "4": {"exist": true, "char_ranges": [[0, 266]], "word_ranges": [[0, 42]], "text": "The history of contact with fresh water in an endemic area points to leptospirosis, and in the clinical picture we also find jaundice and conjunctival injection which also points to leptospirosis and is not so characteristically related to the other three pathogens."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The history of contact with fresh water in an endemic area points to leptospirosis, and in the clinical picture we also find jaundice and conjunctival injection which also points to leptospirosis and is not so characteristically related to the other three pathogens.", "full_answer_no_ref": "The history of contact with fresh water in an endemic area points to leptospirosis, and in the clinical picture we also find jaundice and conjunctival injection which also points to leptospirosis and is not so characteristically related to the other three pathogens.", "full_question": "A 25-year-old man with no past history of interest presents to the emergency department with fever, headache, myalgia, nausea, vomiting, abdominal pain, jaundice and conjunctival injection, 2 weeks after traveling to Thailand to participate in a freshwater regatta. What is the most likely diagnosis?", "id": 298, "lang": "en", "options": {"1": "Malaria.", "2": "Schistosomiasis.", "3": "Leptospirosis.", "4": "Rabies.", "5": NaN}, "question_id_specific": 103, "type": "INFECTIOUS DISEASES", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0497_13706", "title": "Etiologies of acute undifferentiated febrile illness in Thailand.", "score": 0.01656215921483097, "content": "Acute pyrexia of unknown origin (Acute PUO) was reported to affect approximately 200,000-400,000 patients each year reported by the national Annual Epidemiological Surveillance Report. The patients usually present with fever of less than two-week duration and non-specific symptoms such as malaise, myalgia, headache and loss of appetite. Its mortality rate is less than 0.02 percent. It would be interesting to find the etiologies and propose a management plan if the etiologies are discovered. This prospective epidemiologic study aimed to discover the etiologies of acute undifferentiated febrile illness in a tropical region like Thailand. Ten community-based hospitals were chosen as representatives in each part of Thailand to enroll patients into the study. Patients aged over two years old who presented with fever at the participating hospitals during year 1991-1993 were eligible for the study. Entry criteria of acute undifferentiated febrile illnesses (AUFI) included oral temperature over 38.3 degrees C within the last 24 hours, duration of fever ranging from 3-14 days, no specific single organ involvement by history taking and physical examination, normal or non-specific results of the following investigations: complete blood count, thick film for malaria, urinalysis and chest roentgenogram. The patients were hospitalized and a preset diagnostic protocol was performed. Other diagnostic procedures deemed necessary by attending physicians were perform. Patients were followed up within one month after hospital discharge. 1,240 patients were enrolled but only 1,137 case records and results of the serological tests were available for analysis. Etiologies could be found in 471 cases (38.7%). Primary bacteremia was detected in 36 cases (3.2%). E. coli, streptococci, salmonella, Enterobacter spp. and S. aureus were the five most common blood isolates. Serological studies revealed positive results for scrub typhus (7.5%), influenza (6.0%), dengue fever (5.7%), murine typhus (5.3%), enteric fever (1.9%), chikunkunya infection (1.1%), leptospirosis (1.1%) and melioidosis (0.9%). Thirteen cases succumbed (1.1%) in this study. The etiologies in the majority (61.3%) of AUFI remained unknown. Rickettsial infection, influenza and dengue fever are the most common identifiable diseases in a tropical country like Thailand especially during the rainy season. A management guideline for diagnosis and treatment of the AUFI with emphasis on primary bacteremia and antimicrobial-treatable AUFI was proposed."}, {"id": "pubmed23n0386_19198", "title": "Assessment of the clinical presentation and treatment of 353 cases of laboratory-confirmed leptospirosis in Hawaii, 1974-1998.", "score": 0.0164369378114282, "content": "Leptospirosis is frequently misdiagnosed as a result of its protean and nonspecific presentation. Leptospirosis, a zoonosis with global distribution, commonly occurs in tropical and subtropical regions; most reported cases in the United States occur in Hawaii. All laboratory-confirmed leptospirosis cases in the State of Hawaii from 1974 through 1998 (n=353) were clinically evaluated. The most common presentation involved nonspecific signs or symptoms, including fever, myalgia, and headache. Jaundice occurred in 39% of cases; conjunctival suffusion was described in 28% of these cases. Initiation of antibiotics before the seventh day of symptoms was associated with a significantly shortened duration of illness. Because early recognition and initiation of antibiotic therapy are important, clinicians should familiarize themselves with the clinical presentation of leptospirosis, and when evaluating a patient with a febrile illness, they should obtain exposure and travel histories and entertain the possibility of leptospirosis in the differential diagnosis."}, {"id": "pubmed23n0829_5822", "title": "Leptospirosis in the Tohoku region: re-emerging infectious disease.", "score": 0.014770002180074123, "content": "Leptospirosis is a zoonotic and disaster-related infectious disease. It is mainly endemic in subtropical or tropical countries and has not been reported since 2009 in the Tohoku region (northern Japan), including the Yamagata and Miyagi Prefectures. However, we experienced four patients with leptospirosis in the Tohoku region from 2012 to 2014; three patients (#1-3) live in the agricultural areas of the Yamagata Prefecture and one patient (#4) was a visitor to the Miyagi Prefecture. Patient 1 (81-year-old female) is a villager, with a rat bite, while Patient 2 (77-year-old male) and Patient 3 (84-year-old female) are farmers and were infected probably during agriculture work. Patient 4 (40-year-old male US citizen) was infected while traveling in Thailand. They had chief complaint of fever, headache, and myalgia and showed manifestations of hyperbilirubinemia (mean, 4.35 mg/dL), thrombocytopenia and acute kidney injury (AKI). All patients were diagnosed by polymerase chain reaction using blood and/or urine samples and a microscopic agglutination test for the anti-Leptospira antibody. All the patients were treated with infused antibiotics, including minocycline. The patients underwent hemodialysis due to severe AKI (mean serum creatinine, 4.44 mg/dL), except for Patient 2 with the normal serum creatinine level (1.12 mg/dL). All the patients recovered and were discharged. The presence of the three patients in the Yamagata Prefecture implies that leptospirosis does re-emerge in the Tohoku region. Therefore, careful survey of the pathogen is necessary for febrile patients with AKI who engage in agriculture or have a recent history of travelling in subtropical or tropical countries. "}, {"id": "pubmed23n0921_24626", "title": "A Tale of Black Eschar in a Returning Traveller.", "score": 0.014219443323920937, "content": "African tick-bite fever is an increasingly common cause for fever in the returning traveller. It needs to be considered in the febrile returning traveller with a characteristic rash: a black eschar. We describe a 51-year-old man returning from South Africa who presented to our emergency department with fever, headache, myalgia, and chills. On careful history and skin examination, a black eschar was found on the patient's left lateral shoulder, pointing toward a diagnosis of African tick-bite fever. The patient was treated with doxycycline and rapidly improved. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: In the emergency department, the diagnosis of African tick-bite fever is often overlooked in the pursuit of ruling out other travel-related illnesses, such as malaria. A thorough history, a complete physical examination, and a high level of suspicion are essential to the timely diagnosis and treatment of African tick-bite fever in the returning traveller."}, {"id": "pubmed23n0825_17041", "title": "Leptospirosis presenting as honeymoon fever.", "score": 0.013851054988688306, "content": "An increasing number of travelers from western countries visit tropical regions, questioning western physicians on the prophylaxis, the diagnosis and the therapeutic management of patients with travel-associated infection. In July 2014, a French couple stayed for an adventure-travel in Columbia without malaria prophylaxis. A week after their return the woman presented with fever, myalgia, and retro-orbital pain. Three days later, her husband presented similar symptoms. In both patients, testing for malaria, arboviruses and blood cultures remained negative. An empirical treatment with doxycycline and ceftriaxone was initiated for both patients. Serum collected from the female patient yielded positive IgM for leptospirosis but was negative for her husband. Positive Real-Time PCR were observed in blood and urine from both patients, confirming leptospirosis. Three lessons are noteworthy from this case report. First, after exclusion of malaria, as enteric fever, leptospirosis and rickettsial infection are the most prevalent travel-associated infections, empirical treatment with doxycycline and third generation cephalosporin should be considered. In addition, the diagnosis of leptospirosis requires both serology and PCR performed in both urine and blood samples. Finally, prophylaxis using doxycycline, also effective against leptospirosis, rickettsial infections or travellers' diarrhea should be recommended for adventure travelers in malaria endemic areas. "}, {"id": "pubmed23n0512_1449", "title": "Leptospiral pneumonia.", "score": 0.013453866395042865, "content": "Severe leptospirosis rarely presents with primary pulmonary manifestations, without any associated jaundice or renal dysfunction. The authors report a nine-year-old boy who presented with complaints of abrupt onset of high fever; with myalgia, headache, and pain in right chest region, productive cough with hemoptysis and vomiting developing over the past 72 hours. Chest radiograph showed consolidation in the right upper lobe with air bronchogram. A history of contact with sewage water and presence of conjunctival suffusion in a child with pneumonia made us suspect leptospirosis. Following prompt initiation of parenteral penicillin therapy the child's complaints resolved over the next five days. Dri-Dot test to detect anti-Leptospira antibodies was positive. The diagnosis of leptospirosis was confirmed by a positive microagglutination test to Leptospira interrogans serovar Australis by a fourfold rise in antibody titer in paired sera collected during convalescence. Leptospirosis presenting with pulmonary hemorrhage has been associated with significant mortality but it can be successfully treated with early clinical suspicion of alveolar hemorrhage and prompt therapy."}, {"id": "pubmed23n0685_5725", "title": "Malaria: an important emergency room diagnosis.", "score": 0.013116294967102453, "content": "Fever in a traveller returning from an area endemic with malaria is a medical emergency. Although malaria is rare in Canada, it is one of the commonest communicable diseases in the world. A history of prophylaxis is no guarantee that malaria has not been contracted. This case history details an example of failed prophylaxis, emergency room presentation, and emergency treatment for Plasmodium falciparum. Clinical manifestations of the disease can occur up to six months after return from a malarial region, especially if chemoprophylaxis delays initial presentation. Symptoms include the sudden onset of chills, rigor, fever, profuse sweating, prostration, malaise, myalgia, headache, anorexia, nausea, vomiting and diarrhea. A single negative blood film does not exclude the diagnosis."}, {"id": "InternalMed_Harrison_16947", "title": "InternalMed_Harrison", "score": 0.012624482917113527, "content": "Physicians in areas not endemic for schistosomiasis face considerable diagnostic challenges. In the most common clinical presentation, a traveler returns with symptoms and signs of acute syndromes of schistosomiasis—namely, cercarial dermatitis or Katayama syndrome. Central to a correct diagnosis is a thorough inquiry into the patient’s history of travel and exposure to freshwater bodies—whether slowor fast-running—in an endemic area. Differential diagnosis of fever in returned travelers includes a spectrum of infections whose etiologies are viral (e.g., dengue fever), bacterial (e.g., enteric fever, leptospirosis), rickettsial, or protozoal (e.g., malaria). In cases of Katayama syndrome, prompt diagnosis is essential and is based on clinical presentation, high-level peripheral-blood eosinophilia, and a positive serologic assay for schistosomal antibodies. Two tests are available at the CDC: the Falcon assay screening test/enzyme-linked immunosorbent assay (FAST-ELISA) and the"}, {"id": "article-24195_11", "title": "Leptospirosis -- History and Physical", "score": 0.012348450830713965, "content": "The differential diagnosis for Leptospirosis is extremely large and varies from benign processes like viral upper respiratory tract infections, other viral flu-like illnesses, to severe infections from rarer \"travel\" conditions including Dengue Fever, malaria, Hantavirus, hemorrhagic fevers, and typhoid fever. Also consider other more common conditions (which one would be likely to consider unless specific exposure history is known) like cholecystitis, mononucleosis, primary HIV, or if unvaccinated measles or rubella."}, {"id": "pubmed23n0350_14104", "title": "The spotted traveller.", "score": 0.012215099715099716, "content": "A 23 year old university student comes to see you with a febrile illness and a rash. She has just returned from a 6 week holiday in South East Asia having visited Thailand, Vietnam, Hong Kong and the Philippines. Prior to going away she went to a travel clinic, was appropriately immunised and given malaria prophylaxis which she has taken assiduously. Her symptoms have been present for about 3 days and consist of severe retro-orbital headache, diffuse myalgias, fevers and chills, and anorexia. The rash appeared the day before on her trunk and is now beginning to involve her arms with a slight papular element. She also has conjunctival haemorrhages and is febrile with a temperature of 38.9 degrees C. A full blood count done urgently does not show any malaria parasites but does reveal a low platelet count of 85,000 x 10(6)/L."}, {"id": "InternalMed_Harrison_13802", "title": "InternalMed_Harrison", "score": 0.012210012210012212, "content": "Mild Leptospirosis Most patients are asymptomatic or only mildly ill and do not seek medical attention. Serologic evidence of past inapparent infection is frequently found in persons who have been exposed but have not become ill. Mild symptomatic leptospirosis usually presents as a flu-like illness of sudden onset, with fever, chills, headache, nausea, vomiting, abdominal pain, conjunctival suffusion (redness without exudate), and myalgia. Muscle pain is intense and especially affects the calves, back, and abdomen. The headache is intense, localized to the frontal or retroorbital region (resembling that occurring in dengue), and sometimes accompanied by photophobia. Aseptic meningitis may be present and is more common among children than among adults. Although Leptospira can be cultured from the cerebrospinal fluid (CSF) in the early phase, the majority of cases follow a benign course with regard to the central nervous system; symptoms disappear within a few days but may persist for"}, {"id": "wiki20220301en580_18163", "title": "2017 Puerto Rico Leptospirosis outbreak", "score": 0.012075983717774762, "content": "The delayed onset of symptoms of leptospirosis can make it difficult to detect and distinguish. After an incubation period of typically 5 to 10 days, but potentially as early as 2 days and up to 30 days after infection, people infected with leptospira bacteria can develop flu-like symptoms including fever, chills, headache, myalgia (muscle pain), cough, vomiting, and diarrhea. The early signs are vague and often too general to be able to make a confident presumptive diagnosis of leptospirosis. In some cases, patients may present with only febrile illness with other differentials considered including meningitis, influenza, sepsis and many others. Other patients will present with the fulminant infection exhibiting signs of end stage liver failure, acute renal failure or severe pulmonary hemorrhage syndrome (SPHS). The test considered to be the gold standard for leptospirosis diagnosis by the WHO is one that is not very sensitive in the early stages of the disease, before the body"}, {"id": "InternalMed_Harrison_13816", "title": "InternalMed_Harrison", "score": 0.011745689655172413, "content": "The differential diagnosis of leptospirosis is broad, reflecting the diverse clinical presentations of the disease. Although leptospirosis transmission is more common in tropical and subtropical regions, the absence of a travel history does not exclude the diagnosis. When fever, headache, and myalgia predominate, influenza and other common and less common (e.g., dengue and chikungunya) viral infections should be considered. Malaria, typhoid fever, ehrlichiosis, viral hepatitis, and acute HIV infection may mimic the early stages of leptospirosis and are important to recognize. Rickettsial diseases, hantavirus infections (hemorrhagic fever with renal syndrome or hantavirus cardiopulmonary syndrome), and dengue share epidemiologic and clinical features with leptospirosis. Dual infections have been reported. In this light, it is advisable to conduct serologic testing for hantavirus, rickettsiae, and dengue virus when leptospirosis is suspected. When bleeding is detected, dengue"}, {"id": "InternalMed_Harrison_16415", "title": "InternalMed_Harrison", "score": 0.01143041133381631, "content": "Malaria is a very common cause of fever in tropical countries. The first symptoms of malaria are nonspecific; the lack of a sense of wellbeing, headache, fatigue, abdominal discomfort, and muscle aches followed by fever are all similar to the symptoms of a minor viral illness. In some instances, a prominence of headache, chest pain, abdominal pain, cough, arthralgia, myalgia, or diarrhea may suggest another diagnosis. Although headache may be severe in malaria, the neck stiffness and photophobia seen in meningitis do not occur. While myalgia may be prominent, it is not usually as severe as in dengue fever, and the muscles are not tender as in leptospirosis or typhus. Nausea, vomiting, and orthostatic hypotension are common. The classic malarial paroxysms, in which fever spikes, chills, and rigors occur at regular intervals, are relatively unusual and suggest infection with P. vivax or P. ovale. The fever is usually irregular at first (that of falciparum malaria may never become"}, {"id": "pubmed23n0986_21603", "title": "Serological evidence of human leptospirosis in patients with acute undifferentiated febrile illness from Uttarakhand, India: A pilot study.", "score": 0.010348360655737705, "content": "To the best of our knowledge, there are no reports of serological evidence of human leptospirosis from Uttarakhand state in India. The aim of this study was to screen for serological evidence of leptospirosis in patients with acute undifferentiated febrile illness at a tertiary care teaching hospital in Uttarakhand. A pilot study was conducted from March to November 2017. Fifty-three adult patients who presented in Medicine outpatient Department with a history of fever of ≥7 up to 14 days duration with or without other associated symptoms such as a headache, rashes, myalgia, arthralgia, and conjunctival suffusion were enrolled in the study using convenience sampling technique. Blood samples of these patients were collected and subjected to peripheral smear examination for malaria parasites, dengue immunoglobulin M (IgM) immunochromatographic card test, IgM Typhidot, Leptospira and Scrub typhus IgM ELISA, respectively. Aerobic blood culture was performed in 24 cases. Relevant clinico-epidemiological details were obtained as per the pro forma formulated in accordance with the modified Faine's criteria. Descriptive statistics. The study population consisted of 50.94% of males and 49.06% of females with a mean age ± standard deviation of 34.2 ± 15.2 years. Fifty febrile patients had additional symptoms of which myalgia was the most common (81.1%) followed by arthralgia (22.6%). Peripheral smears of all patients were negative for malaria parasites. Dengue and Typhidot IgM positivity was observed in two and eight patients, respectively. Six and five patients were tested positive by leptospira and scrub typhus IgM ELISA, respectively. <iSalmonella</i Typhi was isolated from blood sample of only one patient. Serum samples of two patients showed dual positivity. All six leptospira seropositive patients satisfied modified Faine's criteria. Leptospirosis is a seemingly unexplored infection in Uttarakhand and should be considered as a differential diagnosis in patients with acute undifferentiated febrile illness."}, {"id": "pubmed23n0903_10740", "title": "Weil's Disease from a Local New Orleans Bar.", "score": 0.009900990099009901, "content": "Leptospirosis is a zoonotic infection that typically presents with fever, myalgias, nausea, and vomiting after contact with contaminated waters or infected animals (typically rodents); and their excrements. Conditions favorable to the transmission of leptospirosis are common in LA and, without treatment, leptospirosis can lead to both liver and renal failure, meningitis, pulmonary hemorrhage and ultimately death. A 56 year old woman with no past medical history presented to the Emergency Department with weakness, myalgias, jaundice and decreased urine output for one week. On arrival, she appeared septic with a heart rate of 130 and fever. Her exam was significant for significant jaundice and diffuse abdominal pain. Laboratory studies were notable for WBC 14, hemoglobin of 12 and platelet count of 63. Creatinine was 8.5mg/dL with a blood-urea nitrogen of 96mg/dl. Total bilirubin was 19.4mg/dL and direct bilirubin was 13.7mg/dL. AST/ALT were 69/38 U/L, respectively and the alkaline phosphate was 160U/L. The patient was admitted to the hospital medicine wards for sepsis and multi-organ failure. She was started on broad spectrum antibiotics but her clinical condition continued to worsen with progressive decline in her hemoglobin and thrombocytopenia and worsening liver failure. She quickly became anuric necessitating dialysis and developed respiratory distress with bilateral pulmonary infiltrates and hemoptysis. Additional history was obtained from her employer that she works at a local New Orleans bar and had been cleaning out rats from the kitchen. Leptospirosis antibody was sent, which returned as positive. Her antibiotics were de-escalated to IV Ceftriaxone. She made a slow recovery over the next two-week period. Since 1987, there has been an average of 3 cases of Leptospirosis diagnosed per year, most of which have been from southeast LA. This case illustrates the importance of considering the diagnosis of Leptospirosis and Weil's Disease in patients in the southeast region of LA who present with multi-organ failure. In addition, our patient's occupational exposure was key to her diagnosis which emphasizes the importance of a detailed history in clinical decision making and patient outcomes."}, {"id": "wiki20220301en169_35386", "title": "Orientia tsutsugamushi", "score": 0.00980392156862745, "content": "The main symptom of O. tsutsugamushi infection is high (febrile) fever; however, the symptom is similar to other vector-borne tropical diseases such as malaria, leptospirosis, typhoid, murine typhus, chikungunya, and dengue fever. This makes precise clinical diagnosis difficult, which often leads to misdiagnosis. The initial indications are fever with chills, associated with headache, muscle pain (myalgia), sweating and vomiting. The appearance of symptoms (the incubation period) takes between 6 and 21 days. A simple visual diagnosis is the presence of an inflamed scar-like scab called eschar, which is regarded as \"the most useful diagnostic clue in patients with acute febrile illness\". Eschar is formed on the skin where an infected mite bit, usually seen in the armpit, groin or any abdominal area (Figure 7). In rare cases, it can be seen on the cheek, ear lobe and dorsum of the feet. But, the problem is that eschar is not always present; at the highest record, only 55% of scrub"}, {"id": "InternalMed_Harrison_13944", "title": "InternalMed_Harrison", "score": 0.009803528468323977, "content": "FIGuRE 211-2 Eschar at the site of the mite bite in a patient with rickettsialpox. (Reprinted from A Krusell et al: Emerg Infect Dis 8:727, 2002. Photo obtained by Dr. Kenneth Kaye.) FIGuRE 211-3 Top: Papulovesicular lesions on the trunk of the patient with rickettsialpox shown in Fig. 211-2. Bottom: Close-up of lesions from the same patient. (Reprinted from A Krusell et al: Emerg Infect Dis 8:727, 2002. Photos obtained by Dr. Kenneth Kaye.) 10–17 days, during which the eschar and regional lymphadenopathy frequently go unnoticed, disease onset is marked by malaise, chills, fever, headache, and myalgia. A macular rash appears 2–6 days after onset and usually evolves sequentially into papules, vesicles, and crusts that heal without scarring (Fig. 211-3); in some cases, the rash remains macular or maculopapular. Some patients develop nausea, vomiting, abdominal pain, cough, conjunctivitis, or photophobia. Without treatment, fever lasts 6–10 days."}, {"id": "pubmed23n0788_4616", "title": "Plasmodium knowlesi in travellers, update 2014.", "score": 0.009708737864077669, "content": "Since the initial discovery of Plasmodium knowlesi in Malaysia, cases have been reported from several neighbouring countries. Tourism has also resulted in an increasing number of cases diagnosed in Europe, America, and Oceania. In this review we focus on the risk of the travel-associated acquisition of P. knowlesi malaria. A search of the literature in PubMed was carried out to identify articles and literature on the distribution of P. knowlesi infections in Southeast Asia and details of its acquisition and importation by travellers to other continents. The cut-off date for the search was December 1, 2013. Search words used were: \"Plasmodium knowlesi\", \"Plasmodium knowlesi infections\", \"Plasmodium knowlesi travellers\", \"Plasmodium knowlesi prevalence\", \"Plasmodium knowlesi host\", \"Plasmodium knowlesi vector\" \"Plasmodium knowlesi RDT\", and \"Plasmodium knowlesi Malaysia\". Traveller numbers to Malaysia were obtained from the Tourism Malaysia website. A total of 103 articles were found. Using a selection of these and others identified from the reference lists of the papers, we based our review on a total of 66 articles. P. knowlesi malaria appears to be the most common malaria species in Malaysian Borneo and is also widely distributed on the Malaysian mainland. Furthermore, locally transmitted cases of P. knowlesi malaria have been reported in Thailand, the Philippines, Vietnam, Singapore, Myanmar, Indonesian Borneo, and Cambodia. Two cases have been reported from non-endemic countries in Asia (Japan and Taiwan) in people with a history of travel to Malaysia and the Philippines. Twelve cases were imported to their home countries by travellers from other continents: two from the USA, two from the Netherlands, two from Germany, and one each from Spain, France, Sweden, Finland, Australia, and New Zealand. In most cases, the infection was associated with a trip to or near forested areas. The symptoms were fever (n=12), headache (n=6), chills (n=6), nausea (n=4), myalgia (n=3), back pain (n=3), abdominal problems (n=1), anorexia (n=2), fatigue (n=2), malaise (n=1), arthralgia (n=1), sore throat (n=1) vomiting (n=2), and jaundice (n=1). All patients were treated successfully with currently available antimalaria treatments. The identification of the pathogen by microscopy can be problematic due to the morphological similarity of P. knowlesi to Plasmodium malariae. P. knowlesi appears to be a threat not only to the local population in Malaysia, but also to the estimated 25 million annual tourists and occupational travellers to Malaysia, especially those who visit rural, forested areas of the country. The P. knowlesi risk is not limited to Malaysia, and travellers from Southeast Asia presenting with possible malaria should be considered for a diagnostic work-up that includes P. knowlesi."}, {"id": "pubmed23n0753_24072", "title": "Acute schistosomiasis in travelers: 14 years' experience at the Hospital for Tropical Diseases, London.", "score": 0.009708737864077669, "content": "We report 79 cases of acute schistosomiasis. Most of these cases were young, male travelers who acquired their infection in Lake Malawi. Twelve had a normal eosinophil count at presentation and 11 had negative serology, although two had neither eosinophilia nor positive serology when first seen. Acute schistosomiasis should be considered in any febrile traveler with a history of fresh water exposure in an endemic area once malaria has been excluded."}, {"id": "pubmed23n0657_7936", "title": "[Leptospirosis (Weil's disease) in Augsburg].", "score": 0.009615384615384616, "content": "Three unrelated patients presented within three months at the Central Hospital of Augsburg, Southern Germany, with jaundice of initially unknown etiology. Patient (Pt.) 1, a 51-year old man was admitted with a history of nausea, vomiting, diarrhea, jaundice and anuria. Pt. 2 was a 58-year-old man who had fever and shivering, and had developed jaundice after a fishing-trip to Canada. Pt. 3 was a 66-year-old woman who presented at the Emergency Unit with recently developed jaundice and pain in the right lateral epigastric area. Laboratory results showed elevated levels for bilirubin, CK, BUN, creatinine and low thrombocytes in patients 1 and 2. An elevated lipase level was found in Pt 1, while Pt 3 had an elevated bilirubin and thrombocytopenia. In Pt 1 and 2 active leptospirosis was diagnosed by serological tests. The third patient showed a subsided leptospirosis, the jaundice having been due to a histologically confirmed drug-associated hepatitis. Patients 1 and 2, who had active disease, showed the full-blown clinical picture of Weil's disease with jaundice, renal failure and thrombocytopenia. After administration of penicillin G and a third generation cephalosporin (ceftriaxone), respectively, all symptoms disappeared. The 66-year-old woman (Pt 3) developed pneumonia and died of multiple organ failure. Leptospirosis is an important differential diagnosis in patients with recent onset of jaundice and acute renal failure. A detailed history may offer the crucial hint and serological tests provide proof. The clinical outcome mainly depends on starting antimicrobial therapy with penicillin G or a third generation cephalosporin as soon as practicable."}, {"id": "wiki20220301en354_4166", "title": "Albert Brown (American veteran)", "score": 0.009615384615384616, "content": "Following the Bataan Death March, Brown endured a three-year imprisonment in a Japanese POW camp from 1942 until he was liberated in the middle of September 1945. He ate nothing but rice while in the camp. Brown became afflicted with more than twelve diseases while in the camp, including dengue fever, malaria and dysentery. He also suffered a broken neck and back. He was released from the camp when he was 40 years old. He was nearly blind from maltreatment and had lost more than eighty pounds, then weighing less than one hundred pounds. A doctor told Brown that he would not live to be 50 years old due to the extent of his injuries. However, he lived to be 105 years old."}, {"id": "pubmed23n0393_5053", "title": "[Leptospirosis in children of Libreville: difficult diagnosis, apropos of 1 case].", "score": 0.009523809523809525, "content": "Leptospirosis is a widespread zoonosis, which is diagnosed less frequently in children than might be expected from the level of exposure to hazards, especially in tropical areas. A 15 1/2-year-old Gabonese boy was admitted following five days of fever, headache, myalgia, abdominal pain, diarrhea, intestinal bleeding, jaundice and conjunctival suffusion. Laboratory data showed abnormal liver and renal function tests, and diagnosis of Plasmodium falciparum malaria was confirmed by thin blood smear. The patient did not clinically improve despite antimalarial treatment and then leptospirosis was suspected. Serologic tests were performed and leptospirosis was later confirmed. Antibiotic treatment (cefuroxim) was given. The outcome was good, liver and renal tests returned to normal in a few days. In tropical area, leptospirosis should be considered in children who are diagnosed with either an unexplained fever, a pseudo-influenza syndrome, or jaundice with hepatorenal involvement and gastrointestinal bleeding."}, {"id": "pubmed23n0320_18341", "title": "Fever in the returned traveler.", "score": 0.009523809523809525, "content": "The most important cause of fever in the returned traveler is malaria. All febrile patients in which malaria is epidemiologically possible require urgent evaluation for P. falciparum malaria, which can be rapidly fatal in the nonimmune patient. Early diagnosis and therapy can prevent severe morbidity and mortality. Other less common causes of undifferentiated fever include acute schistosomiasis, the enteric fevers, rickettsial diseases, leptospirosis, and dengue fever. Early empiric therapy for suspected leptospirosis and the rickettsial infections is encouraged to decrease morbidity and mortality. About a quarter of febrile patients do not have an etiologic agent determined for their illness but recover without sequelae. Patients with fever and hemorrhagic manifestations within 3 weeks of their return need to be isolated for the remote possibility of a highly transmissible agent. Although the febrile traveler is always a challenge, the real world differential diagnosis is limited and a systematic approach via the history, physical examination, and selected laboratory tests is usually sufficient to confirm the diagnosis or eliminate potentially serious infections."}, {"id": "InternalMed_Harrison_13804", "title": "InternalMed_Harrison", "score": 0.009437994768068118, "content": "Physical examination may include any of the following findings, none of which is pathognomonic for leptospirosis: fever, conjunctival suffusion, pharyngeal injection, muscle tenderness, lymphadenopathy, rash, meningismus, hepatomegaly, and splenomegaly. If present, the rash is often transient; may be macular, maculopapular, erythematous, or hemorrhagic (petechial or ecchymotic); and may be misdiagnosed as due to scrub typhus or viral infection. Lung auscultation may reveal crackles, and mild jaundice may be present. The natural course of mild leptospirosis usually involves spontaneous resolution within 7–10 days, but persistent symptoms have been documented. In the absence of a clinical diagnosis and antimicrobial therapy, the mortality rate in mild leptospirosis is low."}, {"id": "pubmed23n0736_25200", "title": "Severe leptospirosis: treatment with intravenous corticosteroids and supportive care.", "score": 0.009433962264150943, "content": "Leptospirosis is a common zoonotic infection worldwide and is recognized as an emerging public health problem. Although commonly thought of as a tropical disease, incidence in temperate climates is increasing, with recent outbreaks in the United States and Germany, among other countries. The disease presents with symptoms ranging from fever, headache, nausea, and vomiting to life-threatening multiorgan failure characterized by acute liver failure, nephritis, pulmonary hemorrhage, meningitis, and cardiac arrhythmia. We describe a case of an otherwise healthy 28-year-old man who had just returned from a 2-month trip to Southeast Asia. He presented to our emergency department twice after his return with the complaint of fever and malaise. Initially, he was treated with symptomatic measures and discharged home with malaria smears and blood cultures pending. On his final presentation before admission, he presented with severe fatigue, myalgia, acute renal failure, and marked thrombocytopenia. After several days, inpatient testing revealed the patient's leptospira antibody titer was markedly positive. Given the nonspecificity of patient symptoms, early diagnosis of leptospirosis can be challenging. Diagnostic uncertainty may lead to delay in recommended intravenous antibiotic treatment. We present a case of severe leptospirosis treated exclusively with supportive measures and intravenous corticosteroids."}, {"id": "pubmed23n0796_4752", "title": "The importance of \"His\" story.", "score": 0.009433962264150943, "content": "A 73-year-old previously healthy man presented with a 3-day history of rigours, abdominal pain, diarrhoea, haemoptysis and myalgia. He had not been abroad recently, but reported being a farmer and having had a recent rat infestation. Laboratory investigations revealed acute kidney failure, deranged liver function tests, raised C reactive protein and a chest CT revealed bilateral ground-glass opacities. This presentation was consistent with icteric leptospirosis which was confirmed by serological testing. Following haemofiltration and the administration of antibiotics the patient made an excellent recovery from his leptospirosis. "}, {"id": "wiki20220301en076_42888", "title": "Childhood immunizations in the United States", "score": 0.009345794392523364, "content": "Vaccine There are two types of vaccines, Cervarix and Gardasil. They are both a three-dose series of injections that are recommended at 11 to 12 years of age, but is commonly given to persons older than 12. Cervarix is used around the world and is considered very safe. Some side effects that may arise are pain at injection site (9 in 10) redness or swelling at injection site (1 in 2) fever of 99.5 degrees Fahrenheit or higher (1 in 8) headache or fatigue (1 in 2) nausea, vomiting, diarrhea, or abdominal pain (1 in 4) brief fainting Gardasil is used more commonly in the United States but it also used around the world and is also considered very safe. Some side effects that may arise are pain at injection site (8 in 10) redness or swelling at injection site (1 in 4) fever mild (100 degrees Fahrenheit) (1 in 10) moderate (102 degrees Fahrenheit) (1 in 65) headache (1 in 3) brief fainting"}, {"id": "pubmed23n1060_3655", "title": "[Febrile episodes, headache and limb pain as well as generalized myalgia in a 27-year-old returning male traveller].", "score": 0.009259259259259259, "content": "Unspecific flu-like symptoms, such as fever, headache and limb pain are encountered very often by general practitioners and in emergency departments. In patients with sepsis and a history of travelling to warmer climates, the differential diagnosis needs to be broader than just commonly encountered viral infections. A 27-year-old Swiss man presented with the symptoms mentioned above after a holiday in the south of France. The pulmonary, hepatic and renal status rapidly deteriorated and the patient required intensive care. The initially suspected diagnosis of leptospirosis could be confirmed serologically during the course of the disease."}, {"id": "pubmed23n0249_16708", "title": "A twelve-year study of leptospirosis on Barbados.", "score": 0.009259259259259259, "content": "Between November 1979 and December 1991, 398 cases of severe leptospirosis were confirmed on Barbados (range for 1980-1991 23-56; mean 32.7; incidence 13.3/100,000/year). For the six-year periods 1980-1985 and 1986-1991 there was no significant change in incidence with time. Incidence is unlikely to change significantly in the next decade. Monthly average case numbers ranged from 1.4 (July) to 4.3 (November). The average (2.8) for June to December (the 7 wetter months) was not significantly higher than that (2.5) for January to May (the 5 drier months). The age range was 7-86. There were three times as many male cases (302) as female (96), and nearly 10 times as many in those &lt; 35. Although the highest number of cases (69) was in males aged 15-24, the highest incidence was in the older age groups, particularly the male 65-74 year-olds, and the female 55-64 year-olds. Leptospirosis was the proven cause of death in 55 (13.8%) hospital patients (annual range 0-13, mean 4.5). Some of a further 39 fatalities might have been cases. Death from leptospirosis was nearly twice as common among the women as among the men. Only one patient under 20 years of age died. Leptospira were isolated and identified from 117 (29.4%) of the 398 sick patients. The infecting organisms were bim (serogroup Autumnalis--75), copenhageni (Icterohaemorrhagiae-26), arborea (Ballum-14) and bajan (Australis-2). These infecting serovars could not be distinguished clinically, but infection was milder in children than in adults. Despite its predominance in surveyed children, serogroup Panama was virtually absent in this study. Rainfall is the major factor affecting the distribution of cases; not surprisingly, sanitation workers and agricultural workers appear to be the groups at highest risk. The general lack of clear-cut risk factors reflects the ubiquity of leptospires in the environment and the fact that the disease is not entirely occupational."}, {"id": "pubmed23n0732_25207", "title": "Abscess of urachal remnants presenting with acute abdomen: a case series.", "score": 0.009174311926605505, "content": "Urachal diseases are rare and may develop from a congenital anomaly in which a persistent or partial reopening of the fetal communication between the bladder and the umbilicus persists. The most frequently reported urachal anomalies in adults are infected urachal cyst and urachal carcinoma. The diagnosis of this entity is not always easy because of the rarity of these diseases and the atypical symptoms at presentation. Imaging techniques, such as ultrasonography and computed tomography have a significant role in recognizing the presence of urachus-derived lesions. Case presentation 1: A 25-year-old Arab-Berber man presented with a 10-day history of progressive lower abdominal pain accompanied by fever, vomiting, and low urinary tract symptoms to our emergency department. Laboratory data revealed leucocytosis. The diagnosis of an acute peritonitis was made initially. Abdominal ultrasonography revealed a hypoechoic tract from the umbilicus to the abdominal wall, and the diagnosis was rectified (infected urachal remnants). The patient was initially treated with intravenous antibiotics in combination with a percutaneous drainage. Afterwards an extraperitoneal excision of the urachal remnant including a cuff of bladder was performed. The histological analysis did not reveal a tumor of the urachal remnant. Follow-up examinations a few months later showed no abnormality.Case presentation 2: A 35-year-old Arab-Berber man, without prior medical history with one week of abdominal pain, nausea and vomiting, associated with fever but without lower urinary tract symptoms visited our emergency department. Laboratory data revealed leucocytosis. Abdominal ultrasonography was not conclusive. Computed tomography of the abdomen was the key to the investigation and the diagnosis of an abscess of urachal remnants was made. The patient underwent the same choice of medical-surgical treatment as previously described for case one, with a good follow-up result.Case presentation 3: A 22-year-old Arab-Berber man, with no relevant past medical history, presented to our emergency department because of suspected acute surgical abdomen. Physical examination revealed umbilical discharge with erythema and a tender umbilical mass. Abdominal ultrasonography and computed tomography scan confirmed the diagnosis of infected urachal sinus. Initial management was intravenous antibiotics associated with a percutaneous drainage with a good post-operative result, but a few days later, he was readmitted with the same complaint and the decision was made for surgical treatment consisting of excision of the infected urachal sinus. The clinical course was uneventful. Histological examination did not reveal any signs of malignancy. We describe our clinical observations and an analysis of the existing literature to present the various clinical, radiological, pathological and therapeutic aspects of an abscess of urachal remnants. To the best of our knowledge, this manuscript is an original case report because this atypical presentation is rarely reported in the literature and only a few cases have been described."}, {"id": "pubmed23n0859_13047", "title": "Early Indicators of Fatal Leptospirosis during the 2010 Epidemic in Puerto Rico.", "score": 0.009174311926605505, "content": "Leptospirosis is a potentially fatal bacterial zoonosis that is endemic throughout the tropics and may be misdiagnosed as dengue. Delayed hospital admission of leptospirosis patients is associated with increased mortality. During a concurrent dengue/leptospirosis epidemic in Puerto Rico in 2010, suspected dengue patients that tested dengue-negative were tested for leptospirosis. Fatal and non-fatal hospitalized leptospirosis patients were matched 1:1-3 by age. Records from all medical visits were evaluated for factors associated with fatal outcome. Among 175 leptospirosis patients identified (4.7 per 100,000 residents), 26 (15%) were fatal. Most patients were older males and had illness onset during the rainy season. Fatal case patients first sought medical care earlier than non-fatal control patients (2.5 vs. 5 days post-illness onset [DPO], p &lt; 0.01), but less frequently first sought care at a hospital (52.4% vs. 92.2%, p &lt; 0.01). Although fatal cases were more often diagnosed with leptospirosis at first medical visit (43.9% vs. 9.6%, p = 0.01), they were admitted to the hospital no earlier than non-fatal controls (4.5 vs. 6 DPO, p = 0.31). Cases less often developed fever (p = 0.03), but more often developed jaundice, edema, leg pain, hemoptysis, and had a seizure (p ≤ 0.03). Multivariable analysis of laboratory values from first medical visit associated with fatal outcome included increased white blood cell (WBC) count with increased creatinine (p = 0.001), and decreased bicarbonate with either increased WBC count, increased creatinine, or decreased platelet count (p &lt; 0.001). Patients with fatal leptospirosis sought care earlier, but were not admitted for care any earlier than non-fatal patients. Combinations of routine laboratory values predictive of fatal outcome should be considered in admission decision-making for patients with suspected leptospirosis."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[226, 293]], "word_ranges": [[29, 39]], "text": "Tuberculosis can affect lung and brain but not cutaneous abscesses."}, "3": {"exist": true, "char_ranges": [[0, 225]], "word_ranges": [[0, 29]], "text": "Nocardia can typically affect immunosuppressed patients, especially those with impaired cell-mediated immunity such as that produced by steroids, and can present with pulmonary involvement, brain abscesses and skin abscesses."}, "4": {"exist": true, "char_ranges": [[294, 336]], "word_ranges": [[39, 46]], "text": "Aspergillus also does not affect the skin."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Nocardia can typically affect immunosuppressed patients, especially those with impaired cell-mediated immunity such as that produced by steroids, and can present with pulmonary involvement, brain abscesses and skin abscesses. Tuberculosis can affect lung and brain but not cutaneous abscesses. Aspergillus also does not affect the skin.", "full_answer_no_ref": "Nocardia can typically affect immunosuppressed patients, especially those with impaired cell-mediated immunity such as that produced by steroids, and can present with pulmonary involvement, brain abscesses and skin abscesses. Tuberculosis can affect lung and brain but not cutaneous abscesses. Aspergillus also does not affect the skin.", "full_question": "A 64-year-old patient, farmer, former smoker (5 years), COPD and afflicted with rheumatoid arthritis on corticosteroid therapy. He consults the emergency department for presenting intense headache of 2 days of evolution with deviation of the oral commissure. As background, he reports that after a month of influenza, he persists with cough, purulent and occasionally hemoptotic expectoration, febrile fever, anorexia, asthenia and weight loss. On arrival, the patient had a fever of 38.2ºC, multiple skin abscesses on the hands, back and buttocks (some with fistulous tracts) and right central facial paralysis, apical infiltrates with small associated pleural effusion on chest X-ray and leukocytosis with neutrophilia. Among the following suspected diagnoses I would consider MOST likely:", "id": 365, "lang": "en", "options": {"1": "Lung neoplasm with brain metastases.", "2": "Disseminated tuberculosis.", "3": "Nocardiosis.", "4": "Aspergillosis.", "5": NaN}, "question_id_specific": 100, "type": "PNEUMOLOGY AND THORACIC SURGERY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0261_10430", "title": "[Two cases of invasive pulmonary aspergillosis in non-immunocompromised hosts].", "score": 0.015674908316573997, "content": "Invasive pulmonary aspergillosis generally occurs in immunocompromised hosts such as patients with leukemia, and other malignancies, who are receiving anti-cancer chemotherapy. In this report, two non-immunocompromised patients who developed invasive pulmonary aspergillosis are presented. Case 1: A 63-year-old man complained of productive cough and fever. He received antibiotic therapy from his personal physician. This symptoms did not respond, however, and dyspnea developed. He was then transferred to our hospital, about one month after the onset. The chest X-ray showed a meniscus shadow suggesting an aspergilloma in the right upper lung field and an infiltrative shadow in the remaining right lung field. Case 2: A 78-year-old man was admitted because of dyspnea, productive cough and appetite loss over the previous three months. The chest X-ray showed a meniscus shadow in the left upper field, an infiltrative shadow in the left lower field and a right pleural effusion sign was also observed. Both cases were diagnosed as having aspergillosis, early in their illness, by the detection of aspergillus antigen in their sera and histopathological and cultural studies of specimens obtained by TBLB. Both improved with intravenous amphotericin B (30 mg/day) and intravenous ulinastatin (200000 IU/day) administration. On the examinations conducted during hospitalization, there was no evidence of any immunosuppressive diseases or immunoincompetent conditions such as leukemia, and other malignancies human immunodeficiency virus infection, diabetes or alcoholism."}, {"id": "pubmed23n0619_10717", "title": "Body aches, tender bones and rapid loss of weight: a case report.", "score": 0.014681977872161921, "content": "Bone metastases presenting with pain and body-ache may be the first presentation of carcinoma in about a fourth of patients with cancer. Radiologically majority of the metastases are osteolytic and multiple. Sometimes these may be confused with infective or inflammatory conditions, particularly in young individuals, and degenerative conditions of the spine and hip in elderly, which may delay the diagnosis and treatment leading to poor outcomes. A 30 year old non-smoking male teetotaller presented with intermittent, high-grade nocturnal fever with night sweats of one year. He also had low back ache over his right hip. We found him febrile, pale and his long bones, ribs and pelvis were tender. He had a 3 x 4 cm tender and hard swelling over the upper part of his sternum. Another firm, non-tender swelling about 4 x 5 cm was seen in the right iliac region. Radiographs of the skull, spine and pelvis revealed multiple variable sized lytic lesions. A metastatic malignancy or disseminated tuberculosis was considered. His anti-tubercular therapy was intensified Fine needle aspiration from sternal lesion showed inflammatory cells. A bone marrow biopsy showed infiltration by tumor cells suggestive of metastatic adenocarcinoma. Patient's condition continued to deteriorate and he died within a fortnight of his hospitalization. Although masquerading as tuberculosis lytic lesions might be an evidence of malignant metastatic. Although, treatment is ineffective in this stage palliative efforts to improve quality of life should be made."}, {"id": "pubmed23n1154_22435", "title": "Coccidioidomycosis where you least expect it.", "score": 0.014345369688037337, "content": "An elderly man without history of travel presented with complaints of intermittent fever for 2 months, cough with scanty expectoration for 15 days and history of weight loss of 5 kg in 1 year. The chest X-ray and CT scan of the thorax showed dispersed centrilobular nodules and patchy subpleural consolidation in both lungs with mediastinal lymphadenopathy. He underwent bronchoscopy and bronchoalveolar lavage culture grew <iPseudomonas aeruginosa</i He was prescribed antibiotics based on culture sensitivity; however, patient continued to have symptoms. All relevant blood investigations were within normal limits. He underwent CT-guided biopsy of the right lung lesion during which clearing of the radio-opacities present in the initial CT scan and appearance of fresh lesions in different locations were observed. Migratory shadows were suspected. Fine-needle aspiration cytology showed features suggestive of coccidioidomycosis for which antifungals were started. After 1 month, he improved symptomatically and chest X-ray showed clearance of shadows."}, {"id": "InternalMed_Harrison_13076", "title": "InternalMed_Harrison", "score": 0.013016745159602303, "content": "CLINICAL MANIFESTATIONS Respiratory Tract Disease Pneumonia, the most common form of nocardial disease in the respiratory tract, is typically subacute; symptoms have usually been present for days or weeks at presentation. The onset is occasionally more acute in immunosuppressed patients. Cough is prominent and produces small amounts of thick, purulent sputum that is not malodorous. Fever, anorexia, weight loss, and malaise are common; dyspnea, pleuritic pain, and hemoptysis are less common. Remissions and exacerbations over several weeks are frequent. Roentgenographic patterns vary, but some are highly suggestive of nocardial pneumonia. Infiltrates vary in size and are typically dense. Single or multiple nodules are common (Figs. 199-1 and 199-2), sometimes suggesting tumors or metastases. Infiltrates and nodules tend to cavitate (Fig. 199-2). Empyema is present in one-quarter of cases. Co-infection with Nocardia and Mycobacterium tuberculosis has been reported from regions where"}, {"id": "InternalMed_Harrison_15974", "title": "InternalMed_Harrison", "score": 0.01217181665833652, "content": "Acute pulmonary infection is often diagnosed in association with point-source outbreaks. Typical symptoms include the abrupt onset of fever, chills, pleuritic chest pain, arthralgias, and myalgias. Cough is initially nonproductive but frequently becomes purulent as disease progresses. Chest radiographs usually reveal alveolar infiltrates with consolidation. Pleural effusions and hilar adenopathy are uncommon. Most patients diagnosed with pulmonary blastomycosis have chronic indolent pneumonia with signs and symptoms of fever, weight loss, productive cough, and hemoptysis. The most common radiologic findings are alveolar infiltrates with or without cavitation, mass lesions that mimic bronchogenic carcinoma, and fibronodular infiltrates. Hematogenous dissemination to the skin, bones, and genitourinary tract occurs most often in association with chronic pulmonary disease. Although blastomycosis is not considered an opportunistic infection, immunosuppression has been recognized as a risk"}, {"id": "article-19667_13", "title": "Coccidioidomycosis -- History and Physical", "score": 0.010648148148148148, "content": "Symptoms appear seven to 21 days post-exposure. Fever, cough, shortness of breath (SOB), and chest pain are most frequent. The clinical presentation may be acute or sub-acute based on the inoculum size. A headache, weight loss, and rash are often seen. The rash is faint, maculopapular, transient, occurs early during disease, and is, therefore, often missed. Erythema nodosum or erythema multiforme occurs more frequently in women. Migratory arthralgias are also common. The triad of fever, erythema nodosum, and arthralgias (especially of the knees and ankles) has been termed desert rheumatism. Laboratory findings include elevated erythrocyte sedimentation rate (ESR) and eosinophilia. Chest x-ray (CXR) shows unilateral infiltrates. Hilar and peritracheal adenopathy suggests the extrathoracic spread of the disease. Lung cavities are present in only 8% of adults but are more frequent in children."}, {"id": "wiki20220301en029_52548", "title": "Blastomycosis", "score": 0.01026072772238154, "content": "a flu-like illness with fever, chills, arthralgia (joint pain), myalgia (muscle pain), headache, and a nonproductive cough which resolves within days. an acute illness resembling bacterial pneumonia, with symptoms of high fever, chills, a productive cough, and pleuritic chest pain. a chronic illness that mimics tuberculosis or lung cancer, with symptoms of low-grade fever, a productive cough, night sweats, and weight loss. a fast, progressive, and severe disease that manifests as ARDS, with fever, shortness of breath, tachypnea, hypoxemia, and diffuse pulmonary infiltrates. skin lesions, usually asymptomatic, can be verrucous (wart-like) or ulcerated with small pustules at the margins. bone lytic lesions can cause bone or joint pain. prostatitis may be asymptomatic or may cause pain on urinating. laryngeal involvement causes hoarseness. 40% immunocompromised individuals have CNS involvement and present as brain abscess, epidural abscess or meningitis."}, {"id": "pubmed23n0838_23717", "title": "[Clinical analysis of the first patient with imported Middle East respiratory syndrome in China].", "score": 0.009900990099009901, "content": "To report the treatment of the first imported Middle East respiratory syndrome ( MERS ) in China, and to investigate the clinical features and treatment of the patient. On May 28th, 2015, the first patient of imported MERS to China was admitted to Department of Critical Care Medicine of Huizhou Municipal Central Hospital. The clinical features and treatments of this patient were analyzed. (1) A 43 years old male of South Korean nationality was admitted with the complaint of back ache for 7 days and fever 2 days with the following characteristics: back ache 7 days ago, without fever or cough or expectoration. He had been suspected to suffer from infection of Middle East respiratory syndrome coronavirus ( MERS-CoV ) by the Disease Control Department of South Korea, but no specific treatment was given. He had fever for 2 days with maximum body temperature of 39.7 centigrade. He had no chills, cough, expectoration, short of breath, abdominal pain, diarrhea, frequent micturition, or urgency or pain of urination, and no sore throat. The patient had a history of exposure to MERS-CoV patient. He was considered to be a patient of the second batch of South Korean epidemic. (2) Auxiliary examination: 3 copies of throat swab specimens for virus nucleic acid detection were performed by the Disease Prevention Control Center of China ( China CDC ), and they were positive on May 29th, 2015, and also for serum, sputum and stool. Based on the results of whole genome sequence analysis, the virus strains were implicated to be derived from Riyahh and Jeddah regions of Saudi Arabia. On admission, the patient's blood test showed that the white blood cell count was low ( 3.22×10(9)/L ), the proportion of the neutrophils was high ( 0.73 ), and that of the platelet was low ( 81×10(9)/L ). On admission, the patient's chest X-ray showed that a small amount of infiltration in the lung. (3) TREATMENT: a high-flow nasal cannula ( HFNC ) with oxygen concentration of 0.50-0.80 was given, with a flow rate was set at 60 L/min if tolerated. It was changed to a low flow oxygen inhalation nasal cannula on the 20th day, and oxygen treatment was stopped on the 24th day. Ribavirin 2.0 g was given as the first dose, and was switched to 600 mg every 8 h ( q8h ), and it was reduced to 600 mg q12h after 10 days, and extenuated since the 13th day. Ceftriaxone was added on the 4th day with 2.0 g a day , and it was changed to meropenem 2.0 g, q8h on the 7th day for 2 weeks. Gamma globulin was given for 7 days ( 20 g, qd ). Thymosin-α1 was given on the 8th day for 2 weeks. Interferon was given once a week, but only one dose was used. At the same time symptomatic treatment such as methimazole and liver protection therapy were given. (4) Patient began to cough at admission, and it disappeared on the 18th day. There was no sputum at first, then a small amount of sputum with a little blood appeared after the admission. Then there was cough without sputum. Mild shortness of breath and diarrhea after exertion were noticed. He had no chest pain, difficulty in breathing or other symptoms. There was dullness on percussion in both sides of chest, and it disappeared gradually. Fine moist rales were detectable in scapular area and interscapular area on the 5th day, and they disappeared after 3 days. Breath sounds on both sides was weak, and it became more obvious in the right lung after 5 days, and returned to normal after 18 days. He had a sustaining fever for 1 week with the maximum temperature of 39.5 centigrade, then the body temperature returned to normal. The viral nucleic acid test as performed by the Center for Disease Control of Guangdong ( CDC, Guangdong ) showed that the pharyngeal swab cultured turned negative on the 3rd day, that of serum specimens turned negative on the 8th day, that of stool specimen after 2 weeks, and it was persistently positive for sputum culture until 5 days before discharge. The oxygenation index gradually increased, and it was over 300 mmHg ( 1 mmHg = 0.133 kPa ) after 15 days. Pleural effusion was rapidly increased during the first week as shown by chest X-ray films, and it began to be absorbed gradually in the second week, but it was not completely absorbed until discharge. The disease course of the reported patient was short, with an acute onset, with fever as the chief complaint, but there were no respiratory symptoms, though there were high fever, cough, shortness of breath, diarrhea and other clinical symptoms after admission. Virus in sputum disappeared after treatment, but pleural effusion was not completely absorbed. Negative test for virus in sputum was late, indicating that clearance of virus was slow from the lungs. It is the first case of MERS in China, therefore, the clinical manifestations and the treatment strategy need to be further explored."}, {"id": "pubmed23n0542_23111", "title": "[Pulmonary nocardiasis with abscesses spreading to cerebrum, cerebellum and orbits].", "score": 0.009900990099009901, "content": "A 71-year-old woman presented with suspected tuberculosis. She reported having productive coughs, unwanted weight loss and subfebrile temperature in the preceding 3 months. She was known to have chronic obstructive pulmonary disease treated with corticoids given systemically and by inhalation. She was a heavy smoker. Computed tomography revealed a left apical lung abscess. In the further course of the disease magnetic resonance imaging of the head demonstrated multiple abscesses in both cerebral hemispheres and an abscess, 3.4 cm in diameter, in the right side of the cerebellum, as well as a intra-orbital tumor on the right. Needle aspirate of the eyeball grew Nocardia farcinica. Over 3 weeks antimicrobial treatment was given with imipenem and amikacin, followed by oral cotrimoxazole for 12 months. The abscesses completely regressed and after 12 months no recurrence was demonstrated either radiologically or clinically. Although nocardiasis is rare in Germany it must be included in the differential diagnosis of pneumonia with abscesses. This is especially so if acid-fast bacilli are found. As the resistance pattern of N. farcinica to antibiotics varies, early treatment is essential with antibiotics to which it is sensitive."}, {"id": "pubmed23n0640_17484", "title": "[Two cases of invasive pulmonary aspergillosis mimicking pneumonia in lung cancer patients].", "score": 0.00980392156862745, "content": "Invasive pulmonary aspergillosis (IPA) occurs predominantly in immunocompromised hosts, however increasing numbers of cases of IPA have been reported among basically immunocompetent patients who have some pulmonary abnormalities such as lung cancer and chronic obstructive pulmonary disease (COPD). Case 1. A 67-year-old man was admitted because of hemoptysis and purpura. He had COPD and small cell lung cancer and had finished chemotherapy 5 years previously. Chest X-ray showed pneumonia-like infiltration in his right lower lung field, and marked thrombocytopenia was pointed out. We started antibiotics and corticosteroids for community-acquired pneumonia (CAP) and idiopathic thrombocytopenic purpura. During treatment, we found Aspergillus fumigatus in his sputum culture and therefore added antifungal agents to his treatment. Despite intensive care, he died due to multi-organ dysfunction. Case 2. An 80-year-old man was admitted with fever and productive cough. He had COPD and non-small cell lung cancer and finished chemotherapy 2 months previously. Chest X-ray showed pneumonia-like infiltration in his right upper lung field. We started antibiotics and corticosteroids for acute exacerbation of COPD because of CAP. Several weeks later, after we observed initial improvement of his condition, pneumonia-like infiltration re-developed and Aspergillus fumigatus was detected in his sputum. We started antifungal agents and the treatment of IPA was successful, but he died because of idiopathic perforation of the sigmoid colon. In patients without myelosuppression, IPA could develop pneumonia-like pulmonary infiltrations."}, {"id": "pubmed23n0379_17065", "title": "Pulmonary nocardiosis: clinical experience in ten cases.", "score": 0.00980392156862745, "content": "Pulmonary nocardiosis is an infrequent infection whose incidence seems to be increasing due to a higher degree of clinical suspicion and the increasing number of immunosuppressive factors. To study the predisposing factors, clinical characteristics, diagnostic procedures, treatment and progress of pulmonary nocardiosis (PN). Review of 10 patients (9 male, 1 female, mean age 61) with PN in a 600-bed teaching hospital, diagnosed from 1992 to 1999. Associated diseases observed were chronic obstructive pulmonary disease (COPD) in 6 patients, human immunodeficiency virus (HIV) infection in 3 and polymyalgia rheumatica in 1. Four patients had received oral corticotherapy for COPD for over a year (mean dose 13 mg/day of prednisone or equivalent). The main reason for consultation was an increase in dyspnea in the patients with COPD (6/6) and fever in those with HIV (3/3). Mean time between onset of symptoms and diagnosis was 5 weeks. In 8 patients, the infection occurred outside the hospital setting. The infection was restricted to the lung in 9/10; in the remaining case, the central nervous system (CNS) and subcutaneous tissue were affected. Lobar or multilobar consolidation was the most frequent radiographic pattern found (6/10). Sputum culture was positive when performed (8 cases). Diagnosis was made or confirmed by bronchoscopy (bronchoaspirate or protected specimen brush) in 5 patients. Germs isolated were: Nocardia asteroides (8/10), Nocardia farcinica (1/10), Nocardia otitidiscaviarum (1/10). Cotrimoxazole was the most used empirical treatment (6/10). Resolution was achieved in 5 cases. Four subjects died: 1 HIV patient with disseminated nocardiosis, and 3 COPD patients, 2 of whom had received long-term corticotherapy. Illness recurred in only 1 case, due to failure to comply with treatment. (1) In our geographical setting Nocardia presents as a subacute or chronic pulmonary infection, mainly outside the hospital. (2) It tends to affect only the lung. (3) Diagnosis requires a high clinical suspicion, and can be made on the basis of a sputum culture. (4) Nocardia tends to attack patients with underlying COPD, or immunodepressed patients treated with glucocorticoids, or patients with HIV infection. (5) Mortality is high in both COPD and HIV patients. (6) In our area, cotrimoxazole seems to be the most commonly prescribed treatment."}, {"id": "pubmed23n1056_8409", "title": "Use of steroids to treat anti-tumor necrosis factor α induced tuberculosis-associated immune reconstitution inflammatory syndrome: Case report and literature review.", "score": 0.009708737864077669, "content": "Individuals with tuberculosis (TB) who are being treated with anti-tumor necrosis factor α (anti-TNFα) for coexisting conditions may experience unexpected exacerbations of TB after the initiation of antituberculous therapy, so-called anti-TNFα-induced TB-immune reconstitution inflammatory syndrome (anti-TNFα-induced TB-IRIS). Anti-TNFα-induced TB-IRIS is often treated empirically with corticosteroids; however, the evidence of the effectiveness of corticosteroids is lacking and the management can be a challenge. A 32-year-old man on long-term infliximab therapy for Crohn disease visited a clinic complaining of persistent fever and cough that had started 1 week previously. His most recent infliximab injection had been administered 14 days before the visit. A chest X-ray revealed a left pleural effusion, and he was admitted to a local hospital. A chest computed tomography (CT) scan revealed miliary pulmonary nodules; acid-fast bacilli were found in a sputum smear and a urine sediment sample; and polymerase chain reaction confirmed the presence of Mycobacterium tuberculosis in both his sputum and the pleural effusion. He was diagnosed with miliary TB. Antituberculous therapy was started and he was transferred to our hospital for further management. His symptoms initially improved after the initiation of antituberculous therapy, but 2 weeks later, his symptoms recurred and shadows on chest X-ray worsened. A repeat chest CT scan revealed enlarged miliary pulmonary nodules, extensive ground-glass opacities, and an increased volume of his pleural effusion. This paradoxical exacerbation was diagnosed as TB-IRIS associated with infliximab. A moderate-dose of systemic corticosteroid was initiated [prednisolone 25 mg/day (0.5 mg/kg/day)]. After starting corticosteroid treatment, his radiological findings improved immediately, and his fever and cough disappeared within a few days. After discharge, prednisolone was tapered off over the course of 10 weeks, and he completed a 9-month course of antituberculous therapy uneventfully. He had not restarted infliximab at his most recent follow-up 14 months later. We successfully managed a patient with anti-TNFα-induced TB-IRIS using moderate-dose corticosteroids. Due to the limited evidence currently available, physicians should consider the necessity, dosage, and duration of corticosteroids for each case of anti-TNFα-induced TB-IRIS on an individual patient-by-patient basis."}, {"id": "pubmed23n0064_19304", "title": "Clinical spectrum of pulmonary tuberculosis in older patients: comparison with younger patients.", "score": 0.009708737864077669, "content": "We compared the clinical-radiographic presentations of bacteriologically proven tuberculosis in 72 elderly (mean age: 71 yr) and 73 younger patients (mean age: 39 yrs). The tuberculin test (2 TU PPD) was positive in 55% and 92%, respectively. The prevalence of cough, dyspnea, anorexia, and weight loss was higher in the elderly (p less than .05), and night sweats were more prevalent in the younger patients (p less than .01). The radiographic pattern was not different between both groups (p greater than .10): \"usual\" apicoposterior lesions (with or without other abnormalities) were found in more than 70% of both groups; isolated \"unusual\" lesions consisted in both groups mainly of anterobasal infiltrations and sometimes of pleural effusions, rounded nodules, or miliary patterns. Yet, initially a wrong diagnosis was made more often in the elderly (p = .05). Malignancy, chronic pulmonary disease, and immunosuppression were more frequently encountered in the elderly (p less than .05), whereas alcoholism and smoking were more frequent in the younger patients (p less than .001). Tuberculosis-related mortality occurred in 6 elderly and 1 younger patient."}, {"id": "pubmed23n1058_5445", "title": "Recurrent Empyema Thoracic Secondary to Pulmonary Nocardiosis in Immunocompetent Patients.", "score": 0.009615384615384616, "content": "Pulmonary nocardiosis is a rare disorder that mainly affects immune-compromised patients. We report a 37-year-old male who presented with persistent fever associated with productive cough. During this course of therapy, he had recurrent admissions for empyema thoracic. Clinically, his vital signs were normal. Blood investigations show leukocytosis with a significantly raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Sputum acid-fast bacilli (AFB) was scanty 1+ and sputum mycobacterium culture was negative. Chest X-ray (CXR) showed consolidative changes with mild to moderate pleural effusion on the right side. Skin biopsy was taken and showed Paecilomyces species. A computed tomography scan (CT thorax) was performed and revealed a multiloculated collection within the right hemithorax with a split pleura sign. Decortications were performed and tissue culture and sensitivity (C+S) growth of <iNocardia</i species. And it is sensitive to sulfamethoxazole-trimethoprim and completed treatment for 4 months. This case highlights that pulmonary nocardiosis should be kept in mind in also immune-competent patients, especially in suspected cases of tuberculosis not responding to antitubercular therapy."}, {"id": "pubmed23n1097_6803", "title": "Case report: pulmonary nocardiosis caused by Nocardia exalbida in an immunocompetent patient.", "score": 0.009523809523809525, "content": "Nocardiosis is known as an opportunistic infection in immunocompromised hosts, but it occasionally has been reported in immunocompetent patient. The Nocardia exalbida is first-reported in 2006 from Japan, and a few cases of have been reported in only immunocompromised host, and the characteristic is still unclear. We herein describe the first case of pulmonary nocardiosis caused by N. exalbida in an immunocompetent patient. A77 -year-old Japanese man was admitted to our hospital on November 2, 2018. He was a lifelong non-smoker with no childhood history of respiratory disease. He had a medical history of dyslipidemia. One month before this admission fevers, sputum, mild cough were developed and he was evaluated in a clinic near our hospital. His diagnosis was community acquired pneumonia within his right middle lobe. He was treated with ceftriaxone 1 g/day intravenously for a week, however his symptoms relapsed a few days later. So, the physician retried ceftriaxone for another 3 days, but his symptoms did not improve. He was referred to our hospital. He was treated with sitafloxacin as an outpatient for a week, however his symptoms got worse. The chest CT showed consolidation and atelectasis in his right middle lobe. Low density area was scattered in consolidation, and right pleural effusion was observed. The patient was diagnosed with pulmonary abscess and he was admitted. Administration of piperacillin/tazobactam improved his condition. We switched antibiotics to amoxicillin/clavulanate, and he was discharged. After 2 weeks, he relapsed and was admitted again. After administration of piperacillin/tazobactam for 3 weeks, we perform bronchoscopy and Nocardia species were cultured from samples of the bronchial wash. The isolates were identified as N. exalbida using 16S rRNA gene sequencing. We prescribed Trimethoprim / Sulfamethoxazole (TMP/SMX) for 4 months. Then we switched to minocycline for renal dysfunction caused from TMP-SMX for 1 more month. After 5 months therapy, Consolidation on CT disappeared, and Nocardiosis was cured. we reported the first case of pulmonary nocardiosis caused by N. exalbida in an immunocompetent patient. N. exalbida infection might be associated with a good response to treatment."}, {"id": "pubmed23n1066_5176", "title": "A 49-Year-Old Man Presents With Fever of Unknown Origin and Cough.", "score": 0.009523809523809525, "content": "A 49-year-old man presented with 3 months of persistent fever, cough, shortness of breath, and chest tightness. He had no response to treatment with antibiotics. He had been treated with an empiric 2-week course of steroids approximately 2 months before presentation, with mild and transient improvement. He did not use tobacco and had not experienced any weight loss, hemoptysis, arthralgia, or myalgia, and was otherwise in good health. He denied contact with anyone with pulmonary TB or other respiratory illnesses."}, {"id": "InternalMed_Harrison_16057", "title": "InternalMed_Harrison", "score": 0.009436911414169044, "content": "Chronic Pulmonary Aspergillosis The hallmark of chronic cavitary pulmonary aspergillosis (also called semi-invasive aspergillosis, chronic necrotizing aspergillosis, or complex aspergilloma) (Fig. 241-1) is one or more pulmonary cavities expanding over a period of months or years in association with pulmonary symptoms and systemic manifestations such as fatigue and weight loss. (Pulmonary aspergillosis developing over <3 months is better classified as subacute invasive aspergillosis.) Often mistaken initially for tuberculosis, almost all cases occur in patients with prior pulmonary disease (e.g., tuberculosis, atypical mycobacterial infection, sarcoidosis, rheumatoid lung disease, pneumothorax, bullae) or lung surgery. The onset is insidious, and systemic features may be more prominent than pulmonary symptoms. Cavities may have a fluid level or a well-formed fungal ball, but pericavitary infiltrates and multiple cavities—with or without pleural thickening—are typical. An irregular"}, {"id": "pubmed23n1055_18239", "title": "A Clinical Challenge in the Emergency Department: A Case of Klebsiella Infective Endocarditis Presenting With Splenic Abscess.", "score": 0.009433962264150943, "content": "Infective endocarditis (IE) is a serious bacterial infection of the endocardium and/or heart valves that carries considerable morbidity and mortality. Often presenting with very non-specific symptoms, this disease presents many challenges to the emergency medicine practitioner. A 47-year-old male with no pertinent medical history presented to the emergency department complaining of shortness of breath. He stated that his symptoms had been persistent for the last three weeks and were associated with malaise and fatigue. CT of the abdomen/pelvis with IV contrast revealed a 7-cm hypodensity of the spleen concerning for abscess versus infarct. He denied any trauma or IV drug use. Follow-up ultrasound was ordered, which characterized the hypodensity as a splenic abscess. An echocardiogram was recommended for possible IE, and cardiology was consulted. The transthoracic echocardiogram was performed on hospital day 2, which showed minimal mitral valve thickening with mild mitral regurgitation. The interventional radiology (IR) service was consulted for the splenic abscess in order to perform CT-guided drainage. An IR drain was successfully placed on hospital day 3. On the same day, blood cultures grew Klebsiella pneumoniae. On hospital day 5, that patient was transferred to the ICU for possible empyema formation with signs of respiratory distress. The patient underwent CT of the chest that showed the development of a left-sided effusion. The patient had also been persistently tachycardic and febrile, with high leukocytosis since admission and worsening respiratory status. Transesophageal echocardiogram (TEE) was scheduled but put on hold due to worsening respiratory status. Repeat TEE was scheduled five days later, which showed mitral regurgitation and increased size of the vegetation despite antibiotic therapy. Two days later, he was scheduled for mitral valve repair. When reviewing our case, the patient had both common and uncommon aspects of splenic abscess or IE. First, despite having respiratory symptoms for two weeks, the primary reason he came to the hospital was due to the new onset of fevers. He was febrile, tachycardic, and with significant leukocytosis. He continued to have fevers despite antibiotic therapy and IR drainage of the abscess. With no history of IV drug use history, negative transthoracic echocardiography, lack of immunocompromising condition, and blood cultures with gram-negative rods, IE became less likely of a diagnosis.  Establishing the diagnosis of IE proved to be exceptionally complicated, especially in the setting of a COVID-19 pandemic. The most notable challenge was having a high index of suspicion despite any risk factors. The patient was a previously healthy 47-year-old male with no medical problems. IE continues to be a clinical challenge for physicians, especially in the emergency department, due to the lack of diagnostic criteria such as positive blood cultures or vegetations visualized on echocardiographic studies. IE has a wide gamut of presentations with different levels of acuity. Diagnosis is more straightforward when patients present with obvious risk factors, but, in many cases, such as this one, those risk factors may be absent. A high index of suspicion is required, especially in patients with additional findings such as splenic abscess, embolic phenomenon, focal neurologic deficit, mycotic aneurysm, decompensated heart failure, new murmurs, or pleural effusions."}, {"id": "pubmed23n1078_9677", "title": "Pulmonary actinomycosis and marijuana vaping.", "score": 0.009433962264150943, "content": "A 33-year-old man without significant medical history presented to the emergency department with a 6-month history of fatigue and 30 pounds of unintentional weight loss, with a recent cough and fever over the past week. He recalled two similar illnesses during college that did not require medical care. He denied tobacco use but reported inhaling marijuana 1-2 times daily over the past year with a vaping device. Physical exam was notable for a temperature of 100.0°F and an elevated blood pressure at 161/77 mm Hg. He was diaphoretic with clear breath sounds bilaterally. Chest imaging revealed diffuse ground glass opacities with subpleural sparing and mildly enlarged hilar lymph nodes. Bronchoscopy with transbronchial lung biopsies and needle aspirate of lymph nodes revealed organising pneumonia, and subsequent cultures grew <iActinomyces odontolyticus</i He was treated with amoxicillin and corticosteroids with subsequent resolution on repeat chest imaging."}, {"id": "pubmed23n1085_18777", "title": "A 44-Year-Old Woman With a 10-Year History of Dyspnea and Pulmonary Nodules.", "score": 0.009345794392523364, "content": "A 44-year-old woman was referred for evaluation of dyspnea on exertion and multiple nodular opacities on a chest CT scan. She had a medical history of autoimmune encephalitis, diabetes mellitus, hypertension, migraines, and allergic rhinitis. Ten years earlier, the patient was admitted to an outside institution with symptoms of shortness of breath. She was found to have multiple pulmonary nodules and was diagnosed empirically with and treated for sarcoidosis. She was told that her pulmonary nodules had improved on follow up. However, she continued to have symptoms of dyspnea. Due to progressive symptoms of shortness of breath, she was referred to pulmonology. She reported a weight gain of 80 pounds over the last year. She denied fever, chills, hemoptysis, night sweats, joint swelling, or skin rash. She is a former cigarette smoker with a 15 pack-year smoking history, quit smoking in 2005. She denied alcohol or drug use. She resided in Arkansas and Texas over the past decade. She previously worked as a teacher and is currently unemployed. She had no other relevant exposures. She denied a family history of autoimmune diseases or malignancies."}, {"id": "pubmed23n0853_20853", "title": "Unusual cause of chest pain: empyema necessitans and tubercular osteomyelitis of the rib in an immunocompetent man.", "score": 0.009259259259259259, "content": "A 33-year-old man, born in India but resident in the UK for 5 years, presented to the emergency department with a 4-week history of a dry cough and right-sided pleuritic chest pain. He reported systemic features, including fever and unintentional weight loss. His medical history included vitamin D deficiency. He had travelled to India 10 months previously and denied any exposure to tuberculosis (TB). He was an ex-smoker with a 20 pack history. Respiratory examination confirmed decreased air entry of the right lower lobe and stony dullness on percussion. His C reactive protein was 178 mg/L. A chest radiograph identified a moderate-sized right-sided pleural effusion and destruction of the lateral aspect of the right fifth rib, strongly suggestive of underlying malignancy. Further investigation with a CT of the thorax identified a focal lytic lesion in the right fifth rib, at its lateral aspect, with expansion of the rib observed. Ultrasound-guided pleural aspiration confirmed an exudative pleural effusion. Gram stain revealed no organisms or polymorphs. Four days post admission, the patient was transferred to the regional thoracic surgery unit and underwent video-assisted thoracic surgery, bronchoscopy and drainage of his empyema. His Mantoux tuberculin skin test and his TB Elispot were negative, suggesting that TB infection was unlikely. Culture confirmed no growth after 48 h incubation. Histology of his pleural biopsy identified multiple non-confluent necrotising granulomatous inflammation with very occasional acid-alcohol-fast bacilli-like organisms, highly suspicious for mycobacterial infection. The isolate, Mycobacterium tuberculosis, was identified by Accuprobe and HAIN tests, respectively. MPT64 erythrocyte sedimentation rate (ESR) results from the fifth rib were positive for M. tuberculosis. This case report discusses the aetiology, clinical presentation and pathophysiology of both empyema necessitans and tubercular osteomyelitis of the rib. "}, {"id": "pubmed23n0851_21128", "title": "A 35-year old woman with productive cough and breathlessness.", "score": 0.009259259259259259, "content": "A 35-year-old lady was seen in the outpatient clinic owing to fever, cough with mucopurulent expectoration, and breathlessness for the duration of 1 month. She had history of similar episodes treated with antibiotics four times during last 2 years. There was no history of recurrent sinusitis, diarrhea, and skin or soft tissue infection. She had no history of diabetes mellitus or steroid intake. She denied any history of facial trauma or dental infection in the past. There was no history of tuberculosis in her or in the family. Radiograph and CT scan of the chest revealed right upper lobe consolidation. Flexible fibreoptic bronchoscopy revealed multiple nodules at opening of right upper lobe bronchus. This clinicopathological conference describes the details of differential diagnoses, difficulties in achieving the final diagnosis and management of such patient. "}, {"id": "pubmed23n0685_5539", "title": "[Disseminated nocardiosis presenting as retroperitoneal abscess: a case report].", "score": 0.009174311926605505, "content": "A 64-year-old man presented to our emergency room with right back pain on July 10, 2009. At the emergency room, abdominal enhanced computed tomography revealed a cystic lesion in the retroperitoneum. Then he was referred to our department. We performed percutaneous drainage of the retroperitoneal lesion and aspirated white pus. The retroperitoneal cystic lesion proved to be an abscess. Microscopic examination of a Gram stained specimen of the abscess revealed gram-positive bacillary fragments ; therefore, we suspected the pathogen to be Nocardia. He had a history of chronic glomerulonephritis and had received treatment consisting of 20 mg prednisolone, and 75 mg cyclosporine per day. He was regularly visiting the department of cardiovascular for follow-up of chronic heart failure. On the day before his visit to our emergency room, his chest X-ray medicine had revealed a nodular shadow. Then he was referred to the department of respiratory medicine and was scheduled to receive a bronchoscopy later. We suspected the nodule of the lung also to be an abscess of Nocardia. Later, head computed tomography (CT) revealed a brain abscess the pathogen of which was Nocardia. Nocardia is a filamentous, gram-positive, branched bacterium and classified as an aerobic actinobacteria. Nocardia species are difficult to diagnose due to non-specific clinical and histological manifestation. We report this case of disseminated nocardiosis presenting as retroperitoneal abscess. The disseminated nocardiosis was diagnosed without delay by percutaneous drainage and appropriate treatment was provided."}, {"id": "pubmed23n0658_5556", "title": "[First case report of respiratory infection with Rothia aeria].", "score": 0.009174311926605505, "content": "A 53-year-old woman was admitted to our hospital with dysbasia and forgetfulness. Her past history included uveitis at age 39. Medical examinations led to a diagnosis of neurosarcoidosis. Although she was treated with prednisolone, her symptoms remained, so she received steroid pulse therapy twice, and administration of azathioprine. In early January 2007, a chest X-ray film showed nodules in the right upper lung that rapidly increased in size and number. A CT scan revealed multiple nodules including cavitary lesions in both lung fields. Examination of bronchial lavage fluid and a transbronchial lung biopsy showed a mycelium-like gram-negative filament. After the treatment with benzylpenicillin for 1 month, her laboratory data and radiological abnormalities markedly ima proved. However, switching to oral administration of amoxicillin caused the regrowth of the nodules. She was retreated with intravenous benzylpenicillin for 8 weeks, followed by oral administration of amoxicillin for 5 months, and her condition completely resolved. The causative organism was identified as Rothia aeria (described in 2004) by 16S rRNA gene sequencing. This is the first report of a case of pulmonary infection with this species."}, {"id": "pubmed23n1106_10051", "title": "Tuberculous pleural effusion in a patient with sympathetic ophthalmia on immunosuppression: a case report.", "score": 0.00909090909090909, "content": "Tuberculous pleural effusion (TPE) is paucibacillary, making its diagnosis difficult based on laboratory investigations alone. We present a case of a patient with a TPE who was initially misdiagnosed to have azathioprine-induced lung injury. The diagnosis of TPE was arrived at with the help of clinical assessment, laboratory and radiological investigations. A 25-year-old chronic smoker with sympathetic ophthalmia on long-term immunosuppression, latent tuberculosis infection and a significant family history of tuberculosis presented with a three-week history of productive cough, low-grade fever, night sweats and weight loss. Examination of the lungs showed reduced breath sounds at the right lower zone. Chest x-ray showed minimal right pleural effusion with a small area of right upper lobe consolidation. The pleural fluid was exudative with predominant mononuclear leukocytes. Direct smears of sputum and pleural fluid; polymerase chain reaction of pleural fluid; and sputum, pleural fluid and blood cultures were negative for M. tuberculosis (MTB) and other organisms. As he did not respond to a course of broad-spectrum antibiotics, he was then treated as a case of azathioprine-induced lung injury. However, his condition did not improve despite the cessation of azathioprine. A contrast-enhanced computed tomography of the thorax showed right upper lobe consolidation with tree-in-bud changes, bilateral lung atelectasis, subpleural nodule, mild right pleural effusion and mediastinal lymphadenopathy. Bronchoalveolar lavage was negative for malignant cells and microorganisms including, MTB. However, no pleural biopsy was done. He was empirically treated with anti-tubercular therapy for 9 months duration and showed complete recovery. A high index of suspicion for TPE is required in individuals with immunosuppression living in regions endemic to tuberculosis. Targeted investigations and sound clinical judgement allow early diagnosis and prompt treatment initiation to prevent morbidity and mortality."}, {"id": "pubmed23n0866_19442", "title": "Pulmonary nocardiosis in Chronic Obstructive Pulmonary Disease: A new clinical challenge.", "score": 0.00909090909090909, "content": "Pulmonary nocardiosis (PN) is a rare but severe disease caused by Nocardia spp. Despite the traditional description as opportunistic infection, case reports and case series of pulmonary nocardiosis have recently been reported in immunocompetent patients too, in particular among people with chronic pulmonary diseases such as advanced Chronic Obstructive Pulmonary Disease (COPD). PN is characterized by non-specific symptoms and radiological findings; bacteriological culture can be difficult. For the reasons above, diagnosis of PN is challenging, sometimes resulting in a misdiagnosis of tuberculosis. We report an interesting case of PN in a 75-year-old male with COPD. He complained a 3-months history of fatigue, evening rise in body temperature, night sweats, unexplained weight loss of 5 kg, worsening dyspnea, cough and mucopurulent sputum. The chest X-ray showed multiple nodules with cavitations bilaterally in the apical and subclavian regions. Nocardia cyriacigeorgica with 100% identity was identified in three sputum samples. Since the patient has never undergone a systemic and/or inhaled steroid therapy, and has no respiratory failure and comorbidities entailing immunodepression, it is conceivable that, in this immunocompetent patient, the COPD could represent an isolated risk factor for PN. Risk factors, clinical presentations, radiographic findings, differential diagnosis and review of the literature of PN cases in COPD, pointing out the similarities and differences, are also described. "}, {"id": "pubmed23n1086_3872", "title": "Empyema associated with a cough-induced rib fracture.", "score": 0.009009009009009009, "content": "A 44-year-old man presented to the emergency department with fever and right anterior chest pain. He reported a persistent cough and the development of sudden-onset right anterior chest pain after coughing. The inspiratory pain in the right lung was severe, and therefore deep breathing was impossible. Chest CT revealed a fracture in the right seventh rib with consolidation and pleural effusion. A pleural fluid culture test result was positive for methicillin-susceptible <iStaphylococcus aureus</i He was diagnosed with empyema associated with a cough-induced rib fracture. Thoracic drainage tube placement and intravenous antibiotic therapy successfully ameliorated his condition. He was discharged on day 13 and switched to an 8-week course of oral antibiotic therapy. There was no clinical relapse at the 6-month follow-up."}, {"id": "pubmed23n0422_1325", "title": "[Wegener's granulomatosis--a disease of many faces].", "score": 0.009009009009009009, "content": "We describe a case of Wegener's granulomatosis. The first symptoms included severe headache subsiding only after administration of dexamethasone. Despite a great number of diagnostic tests involving CT and MR of the head, the cause of the headache remained unknown. Because a chest x-ray revealed a nodule in the right lung, the patient was sent to our Clinic. Reevaluation of CT and MR pointed to a massive ethmoid sinusitis. 7-days' course of antibiotics and corticosteroids induced remission of the lung nodule. Several diagnoses were made: neoplasm, bacterial ethmoid sinusitis, trigeminal neuritis, thrombotic cavernous sinusitis and tuberculosis. Results of the ethmoid sinus biopsy together with a high c-ANCA concentration gave the correct diagnosis."}, {"id": "pubmed23n0422_20674", "title": "[Two cases of rheumatoid arthritis developed after polymyositis].", "score": 0.008928571428571428, "content": "We report two cases of rheumatoid arthritis (RA) who later had developed after polymyositis (PM). The first patient was 64-year old male who experienced muscular weakness of the four limbs in proximity 10 years ago. He was diagnosed as PM because of the elevated serum CK and the myogenic pattern of EMG, and his symptoms were improved by treatment with corticosteroid. He started to complain polyarthralgia 2 years ago, followed by interstitial pneumonia, pleuritis and skin ulcer. He was admitted because of exacerbated polyarthralgia, multiple subcutaneous nodules, skin eruption and fever. The level of serum CK was within normal range but CRP was elevated and CH 50 was decreased. The laboratory examination showed positive cryoglobulin and high titer of rheumatoid factor, but anti-Jo 1 antibody was negative. The hand X-ray showed bone erosions in bilateral wrist joints. Skin biopsy revealed leukocytoclastic vasculitis. Based on these findings, he was diagnosed as malignant RA. He was successfully treated with methylprednisolone pulse therapy, cyclophosphamide and prostaglandin E 1. The second patient was 77-year old male with pneumoconiosis who experienced muscular weakness of the four limbs in proximity 4 years ago. He was diagnosed as PM based on his clinical and laboratory findings and was treated with temporary corticosteroid. He started to have polyarthralgia last year, and he was admitted because of increasing arthralgia after the treatment of pulmonary tuberculosis. The level of serum CK was slightly elevated due to hypothyroidism, and CRP was highly elevated. Rheumatoid factor and cryoglobulin were positive, but anti-Jo 1 antibody was negative. The hand X-ray showed bone erosions in bilateral wrist joints. Crystals of pyrophosphate calcium was observed in knee joints. He was diagnosed as RA associate with pseudogout. His symptoms were relieved with corticosteroid, salazosulfapyridine and anti-tuberculous therapy. These two cases had altered their clinical features from PM to definite RA, and both had pulmonary complications. Previous reports described the cases of RA followed by PM, most of which were induced by such drugs as D-penicillamine, but the cases of PM who later had developed RA are extremely unusual. The overlapped cases of RA and PM tend to highly associate with pulmonary lesions."}, {"id": "pubmed23n0826_1017", "title": "A 66-year-old woman with fever, cough, and a tongue lesion.", "score": 0.008928571428571428, "content": "A 66-year-old woman presented with acute onset of fever, chills, and productive cough associated with right-sided chest pain. During a recent hospitalization for dyspnea, she had been diagnosed with Coombs-positive autoimmune hemolytic anemia and had been taking a tapering dose of prednisone starting approximately 6 weeks prior to admission. In the interim, her dyspnea had resolved on treatment with steroids. At the time of presentation, her prednisone dose was 40 mg. Additional medical history included VTE, for which the patient was receiving anticoagulation therapy, and steroid-induced diabetes mellitus. Many years earlier, she had been treated for TB in her home country. The patient had immigrated to Queens, New York, from a Nepalese village 8 years prior. While still in Nepal, she had worked on a farm and had been in close proximity to cows. In Queens, she lived with her family in a house with a small garden but had no pets. Recent travel included a visit to Nepal 9 months ago and a trip to Syracuse, New York, one month prior to presentation. She was a never smoker and did not consume alcohol. "}, {"id": "pubmed23n0851_7339", "title": "[Disseminated Nocardiosis Complicated by Multiple Brain Abscesses: A Case Report].", "score": 0.008849557522123894, "content": "We report a relatively rare case of a disseminated type of nocardiosis without lung involvement. A 75-year-old man developed moderate fever and disturbed consciousness and was admitted to our hospital. Laboratory examinations revealed signs of inflammation. Chest X-rays indicated no abnormalities, but brain MRI showed ring-like enhancement lesions in the right temporal and left frontal lobes. Similar lesions were identified in the left kidney and right leg. The brain lesions were purulent and were surgically irrigated. Gram- and Kinyoun-positive bacteria were identified, and the patient was diagnosed as suffering from a disseminated type of nocardiosis without lung involvement. He was treated with trimethoprim-sulfamethoxazole for over 10 months. The postoperative course was uneventful, and he was discharged without any neurological sequelae two months after surgery. Kinyoun staining was important in early diagnosis and hence providing appropriate therapy for life-threatening nocardiosis. "}, {"id": "InternalMed_Harrison_15160", "title": "InternalMed_Harrison", "score": 0.008777793414016324, "content": "infiltrates on chest x-ray. One may also see nodules, cavities, pleural effusions, and hilar adenopathy. While serologic testing is of value in the immunocompetent host, serologies are negative in 25% of HIV-infected patients with coccidioidal infection. Invasive aspergillosis is not an AIDS-defining illness and is generally not seen in patients with AIDS in the absence of neutropenia or administration of glucocorticoids. When it does occur, Aspergillus infection may have an unusual presentation in the respiratory tract of patients with AIDS, where it gives the appearance of a pseudomembranous tracheobronchitis. Primary pulmonary infection of the lung may be seen with histoplasmosis. The most common pulmonary manifestation of histoplasmosis, however, is in the setting of disseminated disease, presumably due to reactivation. In this setting respiratory symptoms are usually minimal, with cough and dyspnea occurring in 10–30% of patients. The chest x-ray is abnormal in ~50% of patients,"}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[155, 270]], "word_ranges": [[24, 45]], "text": "if we look at the patient's ability to maintain sleep, there is a clear fact that \"rules out\" the hypo/manic shift."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "In this question, one might suspect the possibility of an affective shift after the introduction of the antidepressant drug, according to the patient, but if we look at the patient's ability to maintain sleep, there is a clear fact that \"rules out\" the hypo/manic shift. Two weeks is not enough time for the drug to have taken full effect on mood, but an initial activation may appear, which patients sometimes do not cope with very well (something that does not seem to be the case). In this patient it is indicated to maintain the treatment and reevaluate in no more than one month.", "full_answer_no_ref": "In this question, one might suspect the possibility of an affective shift after the introduction of the antidepressant drug, according to the patient, but if we look at the patient's ability to maintain sleep, there is a clear fact that \"rules out\" the hypo/manic shift. [HIDDEN] is not enough time for the drug to have taken full effect on mood, but an initial activation may appear, which patients sometimes do not cope with very well (something that does not seem to be the case). In this patient it is indicated to maintain the treatment and reevaluate in no more than one month.", "full_question": "68-year-old woman, with a history of 2 major depressive episodes in her lifetime, who consults for symptoms of sadness, depressed mood, anhedonia, asthenia and anorexia compatible with a new depressive episode. She was prescribed 10 mg of escitalopram and was evaluated 2 weeks later. In this review the patient reports feeling very well, she wakes up early very hyperactive and with 'a lot of desire to do things', she says she has a lot of energy and is more talkative than usual. She does not report being irritable and is able to sleep for 6 hours continuously. Given this situation, what would you think the patient has?", "id": 392, "lang": "en", "options": {"1": "Bipolar disorder type I.", "2": "Drug-induced hypomania.", "3": "Normal response to escitalopram.", "4": "Frontal dementia.", "5": NaN}, "question_id_specific": 223, "type": "PSYCHIATRY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0104_5087", "title": "Cyclic 48-hour unipolar depression.", "score": 0.01378331349024636, "content": "This single case study reports on a 74-year-old woman with 48-hour cycles of mood disturbance for 2 years. Every other day she awakened feeling sad with low energy, decreased appetite, fatigue, diminished enjoyment of normal activities, increased irritability, occasional self-deprecatory thoughts, and difficulty concentrating. On alternative days she was active, outgoing, energetic, and cheerful. Her past history was marked by mild postpartum depressions, not requiring treatment, with each of her six pregnancies, and a more severe depression at age 57, which seemed to respond to Premarin. During the recent 2-year period of mood swings, the patient was given trials of several heterocyclic antidepressant medications, but side effects precluded the use of therapeutic doses or durations of treatment. Reluctantly, the patient agreed to a trial of lithium carbonate. After the second week of lithium treatment, at a level of 0.4 mEq/ml, she reported marked improvement, feeling fine every day without mood fluctuations. After almost 1 year at this blood level, she remains asymptomatic. Thus, the patient's cyclic 48-hour unipolar depression responded dramatically and completely to low doses of lithium carbonate."}, {"id": "wiki20220301en622_7422", "title": "Crazy About Her (film)", "score": 0.01229783338419286, "content": "Saul asks Adri to pretend they are both doctors when his young daughter visits, as her stepfather disapproves of his disorder. While posing as a doctor, Adri bails an uncomfortable Carla out of a visit from her parents. However, she still treats him coldly. Adri finally convinces Martha to seduce Victor with a pickup line, which fails until Carla steps in and tells her to admit her feelings bluntly, which goes well. Carla reveals to Adri that she is bipolar. She has episodes of extreme euphoria where she does things for fun without thinking of the consequences, like the night they met. She also has episodes of severe depression and frequent mood swings."}, {"id": "pubmed23n0647_23136", "title": "[A case of depression whose symptoms cured by setting her psychological base on the transcendent level].", "score": 0.010989010989010988, "content": "We report the case of a female in her 40s diagnosed with depression. She was raised by an eccentric father, suspected of having pervasive developmental disorder, and a dominant mother. After graduating from high school, she worked as a clerk in a company for twenty years or so; however, a change in her work environment made her fall into a depressive state. Her worsening depression caused her impulsive resignation and disappearance for about four months. She spent the duration of her disappearance traveling the country, with no dissociative episodes. After returning, she received treatment for depression as an inpatient for about four months. During the first month of hospitalization, she mainly complained of a depressive mood and anxiety over the prognosis of her disorder, while she made scarcely any progress in introspection. In the second month, she gradually advanced with introspective work, but, as her introspection progressed, her depressive mood became aggravated. The therapist avoided intervention to modify her cognition, and told her the following: \"it is better not to persist in managing your depressive mood itself because curing depression does not mean resolving the superficial depressive mood, but to achieve a condition not directly influenced by mood.\" Then, at the beginning of the third month, she became aware of \"the presence of God\" and, at the same time, her depressive mood greatly improved. She extended her sympathy to her mother with her unfortunate life history, and expected her mother to change as she herself had experienced, but, disappointed by her mother, she experienced anxiety attacks and came to realize her own internal rage against significant persons in her life including her mother. After \"the Great being\" experience, she, who had formerly attended Christian church for a short time, started to read the Bible, but she still hesitated about committing herself to \"religious following.\" One day during the last month of hospitalization, as she prayed to God for healing when she read a part in the Bible about a woman suffering from a hemorrhage for twelve years who touched the hem of Jesus' garment and was healed immediately (Matthew 9:20-22 and Luke 8:43-48), the patient suddenly experienced \"the salvation of God\" and realized what trust really meant. Through the experience, her clinical problems became totally cured, and the therapy concluded with her discharge from hospital. Several months later, she sent the therapist a letter including the following message: \"I am grateful to the Lord for salvation from anxiety and irritation, but to the therapist for helping me realize it.\" This clinical course can be understood based on the patient's clinical problems (e.g., despair, anxiety, and depression), arising from the breakdown of her efforts to maintain stability by founding her psychological base on her feelings of omnipotence, avoiding facing her internal negative psychological factors (e.g., rage), and these were automatically resolved when her psychological base was switched to the transcendent level through \"the Great being\" experience and \"the salvation of God.\" Such a sudden, marked improvement resembles what Miller and C'de Baca reported as \"quantum change,\" of which the characteristics are vividness, surprise, benevolence, and permanence. The therapist paid attention to maintain a constant psychological distance from the patient, not persisting in modifying her cognition, with the transcendent level being the basis for the entire therapy. This stance of the therapist itself was considered to prompt her transcendence and bring about her eventual cure. This clinical course seemed to be highly suggestive of a psychotherapeutic mechanism, indicating the close relationship between the transcendent level and basic trust."}, {"id": "wiki20220301en047_76", "title": "Mixed affective state", "score": 0.01061923583662714, "content": "psychic acceleration, however, (as seen in mania or hypomania) the thoughts move in a rapid progression, with many themes, rather than a singular one, being touched upon. Even when such experiences are accounted for on the basis of depression, the possibility does still exist, however, that the depressive episode may be complicated by other manic or hypomanic symptoms, in which case it is often prudent to attend to the patient's personal and family history (e.g., family history of bipolar disorder, early age of onset) to determine whether or not the patient has bipolar disorder."}, {"id": "InternalMed_Harrison_31685", "title": "InternalMed_Harrison", "score": 0.010566139105907636, "content": "DEPRESSIVE DISORDERS Clinical Manifestations Major depression is defined as depressed mood on a daily basis for a minimum duration of 2 weeks (Table 466-7). An episode may be characterized by sadness, indifference, apathy, or irritability and is usually associated with changes in sleep patterns, appetite, and weight; motor agitation or retardation; fatigue; impaired concentration and decision making; feelings of shame or guilt; and thoughts of death or dying. Patients with depression have a profound loss of pleasure in all enjoyable activities, exhibit early morning awakening, feel that the dysphoric mood state is qualitatively different from sadness, and often notice a diurnal variation in mood (worse in morning hours). Patients experiencing bereavement or grief may exhibit many of the same signs and symptoms of major depression, although the emphasis is usually on feelings of emptiness and loss, rather than"}, {"id": "wiki20220301en189_14654", "title": "Bipolar II disorder", "score": 0.009954190912467499, "content": "Depressive episodes in BP-II can present similarly to those experienced in unipolar depressive disorders. Patients characteristically experience a depressed mood and may describe themselves as feeling sad, gloomy, down in the dumps, hopeless, or for most of the day, nearly every day. In children, this can present with an irritable mood. Most patients report significant fatigue, loss of energy, or tiredness. Patients or their family members may note diminished interest in usual activities such as sex, hobbies, or daily routines. Many patients report a change in appetite along with associated weight change. Sleep disturbances may be present, and can manifest as problems falling or staying asleep, frequent awakenings, excessive sleep, or difficulties getting up in the morning. Around half of depressed patients develop changes in psychomotor activity, described as slowness in thinking, speaking, or movement. Conversely, they may also present with agitation, with inability to sit still or"}, {"id": "pubmed23n1106_17409", "title": "Acute and Maintenance Treatment of Bipolar Depression.", "score": 0.009937137330754353, "content": "The World Health Organization reported a lifetime prevalence of 2.4% for BD-I, BD-II and sub-threshold types of bipolar disorder (BD). Depressive episodes are more common than manic episodes for many BD patients. Studies show that depressive mood persists in 2/3 of life, even if they are under treatment. It may be difficult to diagnose BD in the event of depression in the first episode. The correct diagnosis and the treatment can be delayed for 6-8 years, and even longer if disorder starts in adolescence. It is reported that 40% of the patients who were initially diagnosed as unipolar were later diagnosed as BD. The features that enable us to diagnose BD depressive episode: 1) family history of BD or psychosis 2) early onset with depression 3) cyclothymic temperament characteristics 4) four or more depressive episodes in 10 years 5) agitation, anger, insomnia, irritability, excessive talkativeness or other 'mixed' or hypomanic features or psychotic symptoms during depressive episode, 6) clinical 'worsening' caused by the appearance of mixed symptoms after AD treatment 7) suicidal thoughts and attempts 8) substance abuse 9) hypersomnia in the depressive episode or sleeping too much during the day, overeating, psychomotor agitation. The number of studies conducted on BD depressive treatment is limited, the information was obtained by excluding this group from the studies or by compiling the information obtained from the treatment of unipolar depression. In this review, acute and maintenance treatment of the depressive episodes of BD will be discussed according to the treatment algorithms."}, {"id": "pubmed23n0853_1036", "title": "\"Is It Her Hormones?\": Psychiatric Diagnoses and Polycystic Ovarian Syndrome.", "score": 0.009900990099009901, "content": "Beth, whom you have cared for in your primary care practice since she was born, is a 15-year-old adolescent girl with no prior psychiatric history who developed significant symptoms of clinical depression, associated with self-injurious behavior (cutting on wrists, arms, and thighs). She denied any known precipitant for her depression.She is a ninth grade honors student in the gifted program at a local high school and is described as a talented musician, playing multiple musical instruments as well as soccer and basketball. She has good family support, was sociable, and had several close friends. She denied any history of trauma and denied ever using recreational drugs or other mood-altering substances.At this visit, she reported feeling \"sad and anxious.\" Family history was significant for maternal depression, which persisted through her teens and twenties. Her older sister had been diagnosed with Social Anxiety Disorder. Beth reported anhedonia, fatigue and irritable mood, lack of motivation, impaired concentration, and anxiety related to failing grades.You decide to begin medication because of the severity of her symptoms, and 1 week after starting fluoxetine 10 mg, she reportedly overdosed on an unknown quantity of acetaminophen. Within a few days of switching to escitalopram (due to persistent gastrointestinal complaints while taking fluoxetine), she developed homicidal ideation. She reported feeling grandiose, empowered, invincible, elated, and \"crazy,\" although she never demonstrated or endorsed psychotic symptoms. She became fixated upon the idea that she could kill someone and \"get away with it.\" At the time she tried to suffocate a peer with her hands, she was described as having \"a glazed over look in her eyes.\" Moods were now described as alternating between depressed and elated, with mood shifts occurring every few days. These symptoms did not improve after the antidepressant medication was discontinued.Subsequently, patient was admitted for acute psychiatric care, at which time she was described as depressed, but with an \"expansive and irritable\" mood, and with obsessive suicidal ideation. She had developed a plan to hang herself in the home. She started to believe that her mother was trying to give her \"poison.\" She reported panic attacks and said that she wanted to be in the hospital where she could feel \"safe.\" She claimed to have an \"entity inside of her body who was a bully\" and who was \"taking over her body\" and stated that he put her hand over her peer's mouth, as she watched.Psychological testing included the Minnesota Multiphasic Personality Inventory-Adolescent, showed significant paranoia, bizarre mentation, and poor reality testing. Along with interview and observation, it was determined that patient met criteria for the DSM-IV-TR clinical diagnosis of Other Specified Bipolar Disorder, with psychotic features.Aripiprazole was initiated at a dosage of 2 mg; however, moods were still described as fluctuating between extremes every few hours. It was discontinued after reaching 5 mg due to affective blunting. Risperidone 0.5 mg twice daily helped patient to feel and act \"more like herself\"; however, she continued to report significant depression. The addition of lamotrigine 25 mg daily, in addition to individual Dialectical Behavior Therapy, finally led to improvement of mood and a gradual return of her normal baseline, with reportedly stable emotional, social, and academic functioning.The patient's mother remained convinced that this adolescent's mood instability was caused by underlying hormonal problems so you refer her to endocrinology. Beth developed puberty at age 8, with menses occurring on average of twice yearly. She was found to have elevated free testosterone level of 8.6 (reference range, 1.2-7.5). She was of normal weight (body mass index = 21.85 kg/m) and did not manifest acne, male pattern hair thinning, or hirsutism. Thyroid functions, 17-OH progesterone, follicle-stimulating hormone/luteinizing hormone, and estradiol were within normal limits. Prolactin elevation (46.3) was assumed to be due to Risperidone. Patient refused ovarian ultrasound.After starting oral contraceptives to establish monthly menses, patient's emotional and behavioral symptoms continue to remain stable. After Beth decided on her own to discontinue psychotropic medications, she continued for 17 months following her initial visit to remain free of neuropsychiatric symptoms.Now that her symptoms seem resolved; you wonder what the medical diagnosis for Beth was? You wonder if \"hormones\" may have caused or contributed to her psychiatric presentation."}, {"id": "wiki20220301en167_27986", "title": "Tell Her About It", "score": 0.009900990099009901, "content": "\"Tell Her About It\" is a 1983 hit song written and performed by Billy Joel, from the album An Innocent Man. The song was number 1 on the Billboard Hot 100 charts for one week on September 24, 1983, replacing \"Maniac\" by Michael Sembello. The single was certified Gold by the RIAA for US sales of over 500,000 copies. Single A 'special version' mixed by John \"Jellybean\" Benitez was also released as a 12-inch maxi single. The cover art varied depending on the country of release. The remixed version was longer, approximately five-and-a-half minutes. On the B-side featured Billy Joel's song \"Easy Money\" from the same album, and a live recording of the song \"You Got Me Hummin'\" written by Isaac Hayes and David Porter. In the lyrics of the song, the singer exhorts a young man to tell the woman he loves how he feels about her before he misses his chance."}, {"id": "wiki20220301en009_48312", "title": "Diane Arbus", "score": 0.009808612440191388, "content": "Arbus experienced \"depressive episodes\" during her life, similar to those experienced by her mother; the episodes may have been made worse by symptoms of hepatitis. In 1968, Arbus wrote a letter to a personal friend, Carlotta Marshall, that says: \"I go up and down a lot. Maybe I’ve always been like that. Partly what happens though is I get filled with energy and joy and I begin lots of things or think about what I want to do and get all breathless with excitement and then quite suddenly either through tiredness or a disappointment or something more mysterious the energy vanishes, leaving me harassed, swamped, distraught, frightened by the very things I thought I was so eager for! I’m sure this is quite classic.\" Her ex-husband once noted that she had \"violent changes of mood\". On July 26, 1971, while living at Westbeth Artists Community in New York City, Arbus died by suicide by ingesting barbiturates and cutting her wrists with a razor. She wrote the words \"Last Supper\" in her diary"}, {"id": "pubmed23n1105_12868", "title": "Dienogest-induced major depressive disorder with suicidal ideation: A case report.", "score": 0.00980392156862745, "content": "Dienogest is a type of progestin used for the treatment of endometriosis (EM). However, a significant adverse effect of dienogest is depression; therefore, assessing for a history of mood disorders is recommended before prescribing the drug. Herein, we present the case of a patient with no history of psychiatric disorders who was diagnosed with dienogest-induced major depressive disorder. This case emphasizes the importance of close monitoring for negative mood changes in patients taking dienogest. A 41-year-old woman underwent surgery for EM. Postoperatively, her gynecologist prescribed dienogest (2 mg/d) to control EM symptoms. Two months after the initiation of dienogest, she manifested insomnia almost daily, gradually became depressed, lost interest in all activities, had incessant cries, and repeatedly thought of death. She had no history of major physical or psychiatric disorders. Major depressive disorder, single episode, severe. A psychiatric consultation was recommended, an antidepressant was prescribed, and dienogest was discontinued. Two weeks later, there was significant improvement in the symptoms, and after 4 weeks, she remained in a stable mood with no suicidal thoughts. She was followed up for 13 months with a maintenance dose of escitalopram (5 -10mg/d), until the psychiatrist recommended treatment discontinuation, with a confirmed state of remission. This was a case of dienogest-induced depression in a patient with no history of mood disorders. Clinicians should be aware of the possibility of the occurrence of severe depression in progestin users regardless of their previous history."}, {"id": "wiki20220301en195_25862", "title": "An Unquiet Mind", "score": 0.00980392156862745, "content": "Part 2: A Not So Fine Madness Jamison describes her episodes of mania and how they related to her personal and professional life. Her heightened energy and emotions make her a social at work and very efficient with her responsibilities, but irritable and restless in her marriage, which leads her to separating from her husband. She describes periods of reckless spending as characteristic of her mania, and how her brother helped her fix her financial situation. Jamison describes how, in her mania, her brain couldn't focus to read a single paragraph or listen to a song. Shortly after this she seeks treatment for the first time, and a colleague confronts her with her need to take lithium for her disease. Around this time Jamison starts seeing a psychiatrist with whom she starts psychotherapy sessions that would become a part of her routine for the rest of her life."}, {"id": "pubmed23n0591_1651", "title": "Restless legs syndrome induced by escitalopram: case report and review of the literature.", "score": 0.009708737864077669, "content": "Restless legs syndrome (RLS) is a sensorimotor disorder characterized by distressing sensations deep inside the limbs, typically occurring at bedtime or rest. These paresthesias involve an irresistible urge to move the limb, which provides temporary relief but at the expense of sleep and quality of life. The pathophysiology of RLS has been related to dopaminergic pathway dysfunction, thereby aligning it closely with depression from both pathophysiologic and treatment perspectives. Certain antidepressant drugs, including the selective serotonin reuptake inhibitors (SSRIs) and serotonin-norepinephrine reuptake inhibitors (SNRIs), may induce or exacerbate RLS. We describe the case of a 34-year-old woman with no history of RLS who came to the emergency department with acute decompensated heart failure. After 7 days of hospitalization, she was waitlisted to receive a heart transplant. Her mood became depressed, and she requested a psychiatric consultation; escitalopram 10 mg at bedtime was started. Within 2 days of starting therapy, she developed very severe (determined by a score based on an RLS symptom rating scale) RLS symptoms, warranting the discontinuation of escitalopram. Within 2 days of stopping therapy, her RLS symptoms improved considerably (rated as mild). One week later, the patient was rechallenged with a lower dose of escitalopram, and her very severe RLS symptoms reappeared. Within 2 days of stopping escitalopram, her RLS symptoms again improved, with complete resolution 1 week later. Using the Naranjo adverse drug reaction probability scale, which assesses the probability of a drug causing an adverse event, the patient's score was 9, indicating a definite adverse drug reaction. Although published case reports have linked fluoxetine, sertraline, citalopram, paroxetine, and mirtazapine to RLS, this is the first report, to our knowledge, of escitalopram as a cause of RLS. Based on this case and additional data published with other SSRIs and SNRIs, we believe that escitalopram should be added to the list of agents that can induce RLS."}, {"id": "wiki20220301en116_8986", "title": "If She Knew What She Wants", "score": 0.009708737864077669, "content": "Single release and impact Overview Similar in style to the Different Light lead single \"Manic Monday\", \"If She Knew What She Wants\" was issued as a follow-up single in April 1986 while \"Manic Monday\" was still cresting (its Hot 100 peak was no. 2). \"If She Knew What She Wants\" debuted at no. 80 on the Hot 100 dated 10 May 1986, which ranked \"Manic Monday\" at no. 18. \"If She Knew What She Wants\" would begin to lose momentum after reaching no. 50 in its third charting week, stalling at no. 29 on the Hot 100 dated 12 July 1986 in the final week of a five-week Top 40 tenure. \"If She Knew What She Wants\" would spend a total of ten weeks on the Hot 100. \"If She Knew What She Wants\" peaked at no. 31 on the UK Singles, which had afforded \"Manic Monday\" a no. 2 peak. \"If She Knew What She Wants\" also became a moderate chart success in Australia at no. 31 on the Kent Music Report, and in Canada where it peaked in its 10th week on the RPM 100 Singles chart at no. 29 on 26 July 1986."}, {"id": "pubmed23n1141_20355", "title": "Letter to the Editor: Depression As The First Symptom Of Frontal Lobe Grade 2 Malignant Glioma.", "score": 0.009615384615384616, "content": "Dear Editor, Next to focal neurological symptoms, epileptic seizures and head aches, brain tumors can less frequently bring about cognitive changes, slowed speech, difficulty sustaining mental functioning and psychiatric symptoms of personality changes and. loss of interest in daily activities, these symptoms may be evaluated as anxiety or depression. Depression is known to be a complication of brain tumours and may sometimes be seen after the presentation of neurological symptoms linked to brain tumours, and sometimes after tumor treatment (Oğuz et al. 2005, Litofsky et al. 2004, Moise and Madhusoodanan 2006, Oreskovic M et al. 2007, Rooney A et al. 2010). The dorsolateral prefrontal, orbitofrontal and medial frontal circuits constitute the three subcortical neuronal circuits in the frontal cortex. The dorsolateral prefrontal circuit is associated with planning and operational functions and lesions on it may give rise to apathy, abulia, perseveration, personality changes and planning disorder. Lesions involving the orbitofrontal circuit, which is associated with response suppression and disinhibition, may involve emotional lability and memory problems. Whereas lesions affecting the right orbitofrontal circuit give rise to elevated mood, lesions on the left orbitofrontal circuit lead to depressed mood. In cases with medial frontal circuit involvement, akinetic mutism may result from lesions in the superior medial region and anteroretrograde amnesia and confabulation are observed with lesions in the inferior medial region (Tosun et al. 2016, Chirchiglia 2018). A diagnosis of psychiatric disorder may be given during the first examination of patieants with primary brain tumours, especially if localized in the frontal lobe. Thorough history taking and physical examination are necessary for early diagnosis. The case reported here concerns a 29-year-old university graduate female patient, living with her partner and children, who consulted the clinic with complaints of tendency to frequent crying, anhedonia, having difficulty with speech fluency, forgetfulness and distractedness that had presented suddenly, 2 months previously, without any causative stressor. In her mental status examination, she appeared having normal self-care with appearance at her actual age. She was fully conscious and oriented, not willing to cooperate with the interview, had distinct difficulty in maintaining attention and with fluency of speech. Her mood was depressive. She described loss of appetite, fatigue and energy loss. Her difficulty in paying attention was pronounced. She did not have a history of psychotropic medication use or family history of psychiatric disease. She did not smoke or use alcohol or substance. After evaluating the clinical interview, a preliminary diagnosis of major depressive disorder was considered on the basis of the DSM-5 criteria. Routine blood tests were requested. Given the continuation of her complaints, the difficulty with fluent speech and the increase in tendency to sleep at the first week follow up, cranial MRI was planned. The MRI results showed on the right, in the frontal lobe a multilocular mass with precallosal extension, undiscernable margins with the right lateral aspect of the corpus callosum genu and dispersed cystic-necrotic areas with T2 signal series. The dimensions of the mass were nearly 5 x 3 cm causing a 1-cm right-to-left shift of the midline (Figure 1) DEPRESSION AS THE FIRST SYMPTOM OF FRONTAL LOBE GRADE 2 MALIGNANT GLIOMA 2 Türk Psikiyatri Dergisi 2 Turkish Journal of Psychiatry Letter to the Editor 143 144 The patient was referred for surgery with the preliminary diagnosis of high-grade glial tumour. Pathology results identified a grade 2 glioma. It was learned that radiotherapy sessions were begun after surgery. The patient did not have any symptoms of psychopathology during the 2 monthly psychiatric interviews made after surgery. Brain tumours generally indicate their presence with headache, seizures and other neurological symptoms and very rarely with depression as seen in the case of our patient. It should be kept in mind that atypical psychiatric symptoms may have an underlying organic lesion and subtle neurological symptoms should be investigated in detail. A recent meta-analysis on 37 observational studies determined a 21.7% prevalence of depression in a total of 4518 patients with intracranial tumours. Comorbidity of depression with brain tumor was demonstrated to worsen the quality of life, increase suicidal risk and lower the chance of survival (Huang et al. 2017). The possibility of psychiatric symptoms being the clinical clues for brain cancer was noted and the necessity of neuroimaging tests in cases of recent-onset psychosis or mood disorder symptoms, atypical personality changes and anorexia without body dysmorphic disorder was emphasized (Madhusoodanan et al. 2015). Loss of interest, tendency to frequent weeping, introversion and anhedonia were the sole complaints in the case discussed here. The increase in psychomotor retardation and slowing down of movements at the very first weekly control follow up necessitated neuroimaging. Despite the reports in the literature on the frequent association of unpreventable excessive behavior, disinhibition and irritability with right frontal injury and lesions (Okumuş and Hocaoğlu 2018), depression was the dominant symptom in the case presented here. There are differences between primary major depression and depression presenting with underlying somatic diseases which is known to occur at later ages (Rouchell et al. 2002). However, our patient was aged 29 years. Also, cases of depression due to somatic disease are less associated with family history of depression and suicidal ideation and attempts, while cognitive symptoms come to the foreground during mental status examination. (Sertöz and Mete 2004, Rouchell et al. 2002). Our patient did not have suicidal ideation or attempts, or a family history of depression. In apathy, which may be explained as emotional blunting, indifference or detachment from the external world, targeted behavior is also reduced next to the lack of emotional expression. The individual discussed here was learned not to sit at the table or change the television channel unless reminded to do so. When the reason was asked, she could not think of one. The reduction in emotional expression accompanies reduced insight, abulia and lack of empathy (Sözeri Varma et al. 2019). In depression, apathy is defined as 'sorrowless depression'. Our patient cried but had very blunted mimics and gestures. She explained that she could not help weeping even at times when she did not feel internally distressed. The seriousness of apathy, as a symptom difficult to differentiate from depression, is still not understood. Neuroimaging Figure 1- Cranial MRI of the patient 145 Received: 16.08.2020, Accepted: 04.12.2020, Available Online Date: 05.10.2021 1MD., Antalya Kepez State Hospital, Department of Psychiatry, Antalya, 2MD., Ordu University Training and Research Hospital, Department of Psychiatry, Ordu, Turkey e-mail: bosbora@yahoo.com https://doi.org/10.5080/u25957 studies indicate apathy to be a reflect of impaired frontal-subcortical circuits and the functional disorder of the connections between the ventromedial prefrontal cortex and the basal ganglia (Chase 2011). Comparison of 45 individuals with depression due to aging and 43 healthy individuals showed apathy to be associated with fronto-limbic gray and white matter abnormalities which continued after antidepressant treatment. The structural anomalies of the posterior subgenual cingulate gyrus and the uncinate fasciculus were discussed (Yuen 2014). The case discussed here is presented to emphasize the importance of brain imaging methods and detailed investigation of atypical symptoms for diagnostic approaches to psychiatric disorders. Especially, complaints at young age of depression with psychomotor retardation, reduced fluency of speech and sudden onset withdrawal without stressors should be a warning of secondary depression. Yours sincerely... Şerif Bora Nazlı1 , Muhammet Sevindik2 REFERENCES Chase TN (2011) Apathy in Neuropsychiatric Disease: Diagnosis, Pathophysiology, and Treatment. Neurotox Res 19:266-78. Chirchiglia D (2018) Pseudodepression as an Anticipatory Symptom of Frontal Lobe Brain Tumors. Int J Depress Anxiety 1:007. Huang J, Zeng C, Xiao J et al (2017) Association between depression and brain tumor: a systematic review and meta-analysis. Oncotarget 8:94932-43. Litofsky NS, Farace E, Anderson F et al (2004) Depression in patients with high-grade glioma: Results of the glioma outcomes project. Neurosurgery 54:358-67. Madhusoodanan S, Ting MB, Farah T et al (2015) Pyschiatric aspects of brain tumors: A review. World J Psychiatry 5:273-85. Moise D, Madhusoodanan S (2006) Psychiatric symptoms associated with brain tumors: a clinical enigma. CNS Spectr 2006;11:28-31. Oğuz N, Ilnem C, Yener F (2005) Psychiatric symptoms in brain tumors: Case reports. Bulletin of Clinical Psychopharmacology 15:18-21. Hocaoğlu Ç, Okumuş B (2018) Psychiatric manifestations and brain tumor: A case report and brief review. The Medical Journal of Mustafa Kemal University 9:42-9. Oreskovic NM, Strother CG, Zibners LM (2007) An unusual case of a central nervous system tumor presenting as a chief complaint of depression. Pediatric Emergency Care 23:486-8. Rooney A, Carson A, Grant R (2011) Depression in cerebral glioma patients: a systematic review of observational studies. J Natl Cancer Inst103:61-76. Rouchell AM, Pounds R, Tierney JG (2002) Depression Textbook of Consultation-Liaison Psychiatry, 2nd Edition, Volume 1. MG Wise, JR Rundell (Ed), Washington DC American Psychiatric Publishing, Inc, p.307-38. Özen SÖ, Hayriye ME (2004) Bedensel Hastalıklarda Depresyon. Klinik Psikiyatri Ek 2:63-9. Sözeri Varma G , Bingöl C , Topak O et al (2019) Relationship of apathy with depressive symptom severity and cognitive functions in geriatric depression. Arch Neuropsychiatry 56:133-8. Yuen GS, Gunning FM, Woods E et al (2014) Neuroanatomical correlates of apathy in late-life depression and antidepressant treatment response. J Affect Disord 166:179-86."}, {"id": "wiki20220301en069_420", "title": "Stacey Slater", "score": 0.009615384615384616, "content": "Although Stacey and Bradley split up at Christmas 2007 when her affair with Max is revealed, they reunite in December 2009 following Stacey's diagnosis with bipolar disorder (see below). Executive producer Diederick Santer commented on the storyline, saying \"Bradley and Stacey are together – that's what I want, that's what the audience wants and that's what the characters want. Together, they're very strong and they can take on the world. She's good with him, she takes her medication and on the face of it, she's the Stacey Slater we've always known. As ever with Stacey, though, there's a lot hidden. There are a lot of vulnerabilities there but she and Bradley want to make a go of things.\" The 2007 Christmas Day episode in which the affair was revealed is one of the most iconic episodes in EastEnders history and was watched by 14.34 million viewers, becoming the shows' biggest rating in three years and the highest watched TV programme that year altogether."}, {"id": "pubmed23n0507_12674", "title": "[Valpromide, Valproic acid and removal of small intestine in the treatment of a chronic depression: a case report].", "score": 0.009523809523809525, "content": "Valpromide (VPD) is an antiepileptic drug, derivative of Valproic acid (VPA), used as a mood-stabilizer in bipolar disorder for 25 years in several European countries. VPD is also used as an augmentation strategy in refractory depression. Despite chemical similarity between VPA and VPD, the pharmacokinetics of the 2 drugs in humans are quite distinct. We report a case of a patient, suffering from a bipolar treatment resistant depression, who dramatically improved after substituting VPD to VPA in association with fluoxetine. Mme X, 68 years old, has been hospitalized in March 2001 for the treatment of a resistant depression (TRD). She was suffering from removal of small intestine with chronic diarrhoea after a suicidal attempt two years ago. She had a bipolar disorder treated with VPD (1,200 mg/d) since 1 year. She presented a major depressive episode according to DSM IV with various symptoms like depressed mood, hypersomnia and difficulty initiating sleep, diminished ability to concentrate and to think, markedly diminished pleasure in all activities and major anxiety. Mme X fulfilled TRD diagnosis after resistance to two adequate antidepressants trials from different classes (clomipramine 175 mg/day and venlafaxine 300 mg/day). The antidepressant treatment (venlafaxine) was interrupted and she has been receiving a SSRI (fluoxetine 20 mg/day) for 4 weeks. After four weeks, she had a partial remission with persistent sleep problems, mood lability and anxiety. The VPA blood concentration was very low: 27 mg/L (normal range: 50 to 100 mg/L) in spite of a high dosage: 1,200 mg/day. Pharmacokinetic analysis of VPD shown that VPD transformation to VPA usually done in the intestine, was reduced because of the removal of hail intestine. We substituted VPD by VPA. Valproate blood concentration returned to normal range, induced dramatic improvement of depression within three days. VPD is an amide derivative of valproic acid (valproate), biotransformed by hydrolysis to its corresponding valproic acid. VPD is a prodrug of VPA. VPD is absorbed after transformation in gastro-intestinal mucous membrane. The adequate dosage of VPD (Depamide, 300 mg) is 4 to 6 tablets in acute manic phases, 2 to 4 tablets in long term treatment, 1 to 3 tablets in depressive episode. The biodisponibility of VPD is around 100% 75 and 90% of VPD is linked with protein albumin. The daily dosage determined the blood concentration of the active form (VPA), but this relation isn't linear. The optimal blood concentration of VPA (Depakine) ranges between 50 and 100 mg/L. the free form of VPA is influenced by protein disorders such as of hypoalbuminemia and by presence of fat acids in food. This case report demonstrates at a clinical level that VPD and VPA are not equivalent for treating bipolar depression. This case also suggests that a deep investigation of the pharmacokinetic of psychotropic drugs can help clinicians to resolve clinical problems of treatment of depression."}, {"id": "pubmed23n1011_4825", "title": "[If a patient has never experienced depression, should we tell him he has bipolar disorder? An updated systematic review on recurrent mania].", "score": 0.009523809523809525, "content": "Bipolar disorder (BD) is a severe and recurrent mood disorder. It is characterized by episodic changes in mood and energy/activity levels that are increased during mania/hypomania or decreased during depression. Recurrent mania (RM) is a mood disorder, which would be defined by at least two manic/hypomanic without depressive episodes. Despite a rich body of clinical descriptions, RM is still not integrated into the latest editions of disease classifications and continues to be subsumed under BD in clinical practice. We conducted a systematic review of the literature to pool data about RM prevalence within BD groups, identify differences between RM and BD and develop reliable knowledge about specificities of RM. Furthermore, we sought to identify the methodological bias inherent to RM studies. Relevant publications were identified by a systematic search of PubMed, Embase, ScienceDirect and PsychInfo databases according to PRISMA criteria, with no limitation of date. The following MESH terms were used: (mania OR manic) AND (unipolar) NOT (depress*) OR (\"unipolar mania\" OR \"unipolar manic\" NOT \"depress*\"). Twenty-three (23) of 186identified studies met eligibility criteria for our systematic review. The total sample included 1118RM subjects among 4796BD subjects. The weighted mean of RM prevalence was 23.2%. Compared to BD, RM was characterized by a predominance of men, an earlier age at illness onset, less rapid cycles and seasonal variations, longer manic episodes, less specific clinical features (suicide attempts, anxious disorders, catatonic symptoms, irritability, hyperactivity, racing thoughts), less family history of depression, more addictive comorbidities and worse response to lithium prophylaxis (P&lt;0.05). However, many studies failed to replicate these significant differences. RM studies were mainly retrospective. The major bias of RM studies were the lack of consensus on the defining criteria for RM and the risk of unreported depressive episodes, both in charts that were reviewed in retrospective studies and in prospective studies with insufficient follow-up duration. Although the literature on RM remains sparse, many authors agree that RM should be distinguished from BD. RM would concern almost 1 in 4 BD patients. Furthermore, several clinical variables could differentiate this mood disease from BD and may orient the specific therapeutic choice. However, clinical criteria are still not reliable enough to make a diagnosis of RM. Further studies are required to replicate the results of existing studies and to adjust for the effect of methodological biases."}, {"id": "pubmed23n0491_13877", "title": "[Psychiatric manifestations of vitamin B12 deficiency: a case report].", "score": 0.009433962264150943, "content": "Psychiatric manifestations are frequently associated with pernicious anemia including depression, mania, psychosis, dementia. We report a case of a patient with vitamin B12 deficiency, who has presented severe depression with delusion and Capgras' syndrome, delusion with lability of mood and hypomania successively, during a period of two Months. Case report - Mme V., a 64-Year-old woman, was admitted to the hospital because of confusion. She had no history of psychiatric problems. She had history of diabetes, hypertension and femoral prosthesis. The red blood count revealed a normocytosis with anemia (hemoglobin=11,4 g/dl). At admission she was uncooperative, disoriented in time and presented memory and attention impairment and sleep disorders. She seemed sad and older than her real age. Facial expression and spontaneous movements were reduced, her speech and movements were very slow. She had depressed mood, guilt complex, incurability and devaluation impressions. She had a Capgras' syndrome and delusion of persecution. Her neurologic examination, cerebral scanner and EEG were postponed because of uncooperation. Further investigations confirmed anemia (hemoglobin=11,4 g/dl) and revealed vitamin B12 deficiency (52 pmol/l) and normal folate level. Antibodies to parietal cells were positive in the serum and antibodies to intrinsic factor were negative. An iron deficiency was associated (serum iron=7 micromol/l; serum ferritin concentration=24 mg/l; serum transferrin concentration=3,16 g/l). This association explained normocytocis anemia. Thyroid function, hepatic and renal tests, glycemia, TP, TCA, VS, VDRL-TPHA were normal. Vitamin B12 replacement therapy was started with hydroxycobalamin 1 000 ng/day im for 10 days and iron replacement therapy. Her mental state improved dramatically within a few days. After one week of treatment the only remaining symptoms were lability of mood, delusion of persecution, Capgras' syndrome but disappeared totally 9 days after the beginning of the treatment. A neurologic examination was possible because of cooperation. All the tendon reflexes of inferior members were absent. The plantars were in flexion and there was a left inferior member hypoesthesia. The cerebral scan and EEG were normal. Fundic biopsy, realized by fibroscopy, revealed fundic atrophia and intestinal metaplasia compatible with Biermers' disease. The iron deficiency exploration concluded diet deficiency. Mme V. appeared euphoric, her speech was very rapid with play on words and overactivity. This hypomania state totally disappeared 3 days after. Six Months after her hospitalisation, she presented an hypothyroidism (TSH=3,780; T3=1,35; T4=1,08). A thyroid hormones replacement was started and she continued to receive Monthly B12 replacement. Discussion - This case report illustrates psychiatric manifestations of Biermers' disease. The clinical arguments in favour are: white woman, more than 60 Years old, no history of psychiatric problems, atypical symptoms (confusional state with psychiatric symptoms), fluctuation of symptoms (severe depression with confusional state, delusion of persecution and Capgras' syndrome; delusion with lability of mood and hypomania), dramatic improvement after 9 days of vitamin B12 replacement therapy. The biological arguments are: anemia, vitamin B12 deficiency, normal folate level, atrophia and fundic metaplasia, positive antibodies to parietal cells in the serum, association between Biermers' disease and autoimmune disease (Haschimoto thyroidite). Psychiatric manifestations can occur in the presence of low serum B12 levels but in the absence of the other well recognized neurological and haematological abnormalities of pernicious anemia. Mental or psychological changes may precede haematological signs by Months or Years. They can be the initial symptoms or the only ones. Verbank et al. described the case of a patient with vitamin B12 deficiency in whom hypomania, paranoia and depression had been successively presented during a period of 5 Years before anemia have been developed. The case of Mme V. is similar in the succession of severe depression with delusion of persecution and Capgras' syndrome, delusion with lability of mood and hypomania, during a period of two Months. This report seems to be the first one of a sequence of several psychiatric states with pernicious anemia during a period of two Months with normocytosis anemia. To illustrate this illness we reviewed the literature regarding psychopathology associated with B12 deficiency. The most common psychiatric symptoms were depression, mania, psychotic symptoms, cognitive impairment and obsessive compulsive disorder. The neuropsychiatric severity by vitamin B12 deficiency and the therapeutic efficacy depends on the duration of signs and symptoms. Conclusion - We recommend consideration of B12 deficiency and serum B12 determinations in all the patients with organic mental disorders, atypical psychiatric symptoms and fluctuation of symptomatology. B12 levels should be evaluated with treatment resistant depressive disorders, dementia, psychosis or risk factors for malnutrition such as alcoholism or advancing age associated with neurological symptoms, anemia, malabsorption, gastrointestinal surgery, parasite infestation or strict vegetarian diet. In first intention, B12 deficiency should be researched by serum B12 determination (normal 200-950 pg/ml). Studies of methylmalonic acid and homocysteine showed that they are very sensitive functional indicators of cobalamin status especially when other evidence of cobalamin (B12) deficiency was equivocal. Measurement of methylmalonic acid (normal 73-271 nmol/l) and homocysteine (normal 5,4-13,9 micromol/l) should not replace the measurement of serum cobalamin."}, {"id": "wiki20220301en023_41908", "title": "Matthew Good", "score": 0.009433962264150943, "content": "The following summer, Good planned to spend several months in Europe to write a book. However, just a few days into the trip, Good found himself overwhelmed emotionally, experiencing what he described as the \"absolute worst manic episode\" while visiting friends in Bristol. He returned to Vancouver, moving into his parents' home. While there, Good began to have an increasing dependence on Ativan. One night, while at his parents' house, Good took upwards of 45 Ativan pills and collapsed to the floor. The collapse was heard by his parents and he was rushed to the hospital. During a brief stay in the hospital's psychiatric ward, to which he willfully committed himself, Good was diagnosed with bipolar disorder. The genetic illness was traced back to his mother's side. Recalling past events and stages throughout his life, he has described the diagnosis as a relief, adding \"it was like finding the final pieces of the puzzle.\" Good wrote much of the material for his 2007 release, Hospital"}, {"id": "pubmed23n0395_16097", "title": "[Amnesic presentations of the compulsive obsessional confusions (about 3 patients appearing in a consultation of memory)].", "score": 0.009345794392523364, "content": "Disorders or complaints of memory are a frequent cause of consultation in depression, major anxiety and psychiatry disease with personality disorders. We report 3 patients with obsessive compulsive disorder (OCD), without diagnosis and treatment, examined in a specialized memory consultation. They always had OCD with cognitive checking. Diagnosis of transient global amnesia and temporal complex seizure were discussed in 2 cases. Psychometric impairment only was observed in first free recall of a verbal memory task and was no specific. Behavioural during testing seemed to be very important to analyse. First, a 49-year-old man consulted because he had stereotyped transient amnesia lasted one minute, 2 or 3 times a week, since 6 months. He was a teacher. Transient amnesia always occurred during lessons. Suddenly he didn't know where he was or what he was speaking about. Episodes lasted one minute. After them, he had no confusion and no difficulty in concentration but intense anxiety. In an another hand, when he was in his car, after lessons, he could forget where he was during some minutes. CT scan and EEG were normal. Neuropsychological tests only objectived impairment in first free recall of Grober and Buschke's words. Patient explained that he could not prevent to check responses. He told us checking obsessive compulsive disorder during since long time ago. We discussed clear differences which existed between seizure and ruminations or preoccupations. Secondly, a 55-year-old woman was afraid of her memory performances. She was medical secretary and had no problem in her work but she would like a memory consultation to reassure herself. She was neither depressed nor anxious. She presented curious production in fluency task. She had to produce as many animals's names as possible: she could say 35 names which was an excellent performance but only in alphabetic order! Neuropsychological tests objectived impairment in her first free recall of Grober and Buschke's words. She tried in her first free recall to remember words in alphabetic order. She explained how she was bound to range everything in alphabetic order! She had a lot of rituals. She thought that she had an obsessive compulsive disorder but never consulted about this. The observation illustrated suspiscions about memory operations which could be observed in patients group with obsessive compulsive disorders. Finally, a 62-year-old man told us that he had presented a transient global amnesia during 4 hours. He had an important appointment and was upset about that. He didn't go to it and wandered in his flat. He always asked the same questions and forgot everything. He had no neurological deficit. He was anxious, sad and cried several times. He perfectly remembered the episod and thought that he had a panic attack! Verbal memory tests only objectived difficulties in his first free recall of Grober and Buschke words as the two others patients. He had a story of obsessive compulsive disorder with checking and rituals. In this observation, we discussed clear differences which existed between panic attacks and global transient amnesia. We analyzed patterns of neuropsychological performances which illustrated clinical features of obsessive compulsive disorder. These three patients impaired in their first free recall of verbal memory task. It is not a specific result. We observed during psychometric evaluation, strategic processing which impaired episodic memory: patients tried to check their performances. Memory complaints only were observed in checking obsessive compulsive disorder. It is a difficulty or a doubt about memory capacities. Difficulties could be due to particular cognitive processes who pertubate normal memory capacities."}, {"id": "wiki20220301en075_55574", "title": "Synchestra", "score": 0.009345794392523364, "content": "At the end of a whirlwind year, Townsend began working on Strapping Young Lad's fourth album, Alien, in March 2004. Feeling that Strapping Young Lad did not live up to expectations, Townsend decided to take the next album to a new extreme. To prepare for Alien, Townsend stopped taking the medication prescribed to treat his bipolar disorder. \"I think that as an artist, in order for me to get to the next plateau, I kind of feel the need to explore things and sometimes that exploration leads you to places that are a little crazy,\" he explained. \"And Alien was no exception with that.\" Shortly after the release of Alien in March 2005, Townsend began putting together the next Devin Townsend Band record with the working title Human, intended as the more \"pleasant\" counterpart to Alien. The album was ultimately entitled Synchestra, and was \"basically a record about coming back down to earth after being in space with Alien for a while,\" according to Townsend."}, {"id": "pubmed23n0078_13878", "title": "Depression: when is psychotherapy not enough?", "score": 0.009311408016443989, "content": "In doing intensive psychotherapy or analysis with patients who suffer both personality and affective disorders, one must simultaneously maintain psychologic and biologic perspectives. Cooper, when talking of patients suffering from panic disorders, states that analysts must distinguish between psychologic efforts to cope with miscarried brain function and the psychologic efforts to cope with disturbances of the intrapsychic world. This is also true of patients who have affective disorders. For instance, patients who suffer from untreated affective disorder often speak of their experience of themselves as being out of control. They complain that they can never predict the stability of their emotional states. This aspect of their illness must be conceptualized not as reflecting faltering defensive operations and inadequate compromise formation but as the reaction of an otherwise healthy personality to the experience of being intermittently overwhelmed by biologically generated mood states. Cooper also states that biologic illnesses must be regarded as having influences that are both developmental and ongoing. The psychoendocrine work of Puig-Antic demonstrates the existence of endogenous depression in latency age children and Carlson and Kashani's recent clinical observations in preschool children support the notion that this illness can arise during periods of development. For such patients, the normal developmental tasks of childhood and adolescence may be severely compromised. For instance, extreme mood fluctuations of inexplicable origin may serve as a major disruption in the consolidation of a healthy sense of object constancy. Before closing, I would like to briefly mention two examples of the difficulties encountered when attempting to medicate and analyze the same patient. Ostow mentions patients who may attempt to use what he refers to as the \"drug cure\" to reinforce their resistance to psychotherapy. The analyst must be ever alert for this. An example occurred during my analysis of a 35-year-old novelist who had entered treatment for writer's block. She had been on medication for a number of years, had a strong family history of depression, and had relapses each time the medication had been discontinued or decreased. During the eighth month of analysis she reported that she had an interesting experience. She had forgotten to take her evening medication, something extremely unusual for her. She knew that antidepressants suppressed rapid-eye-movement sleep and that stopping tricyclics was often associated with vivid dreams and nightmares. Thus, she thought that the nightmare she experienced that night, something to do with being beaten up, could be explained pharmacologically.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0895_1203", "title": "A 9-Year-Old Girl With Persistent Obsessive and Compulsive Behaviors in a Primary Care Pediatric Practice.", "score": 0.009259259259259259, "content": "Chloe is a 9-year-old gal whose mother made an initial visit to a new pediatrician for concerns about her behavior. Chloe is apprehensive about the visit and frequently hides behind her mother.Her parents first noticed Chloe becoming angry and more emotional 3 years ago, which her parents did not initially understand. However, over the past year, she has started to have more worries and unusual behavior.Chloe and her mother report that when she walks through doorways, she will almost always go back and walks through again. At home, she will walk through doorways multiple times and at school, she will pretend she forgot something so her friends do not notice. She often will not walk downstairs and occasionally her mother has to carry her. Clothes are problematic for Chloe. If her father touches something of a specific color and then touches Chloe, she will have to change her clothes or take a shower. Sometimes, she will never be able to wear those clothes again. She had a recent episode where she could not stop tapping a red paper, because if she stopped, she said it would burst into flame. During the 2 weeks before the pediatric visit, symptoms increased to the point that she is now refusing to go to school. When she stays home, she lays in 1 place all day.Chloe is a fourth grade student. The family does not report academic concerns. She has friends. She denies any appetite or sleep problems. She endorses periods of sadness, lack of energy, and decreased interest in social activities, mostly because she worries and is embarrassed. She kept her behaviors hidden from her 5 siblings for the past year, and she talked only to her mother about them. She is worried her friends might discover her behaviors.The family history is notable for multiple paternal family members with anxiety and bipolar disorder and depression on mother's side. A few months ago, Chloe's family adopted a 7-year-old child with special needs from China.Her growth, vital signs, and physical examination are unremarkable. Her mother filled out the Short Mood and Feelings Questionnaire and the Screen for Child Anxiety-Related Emotional Disorders, which both had elevated scores."}, {"id": "wiki20220301en017_6326", "title": "Good Bye, Lenin!", "score": 0.009259259259259259, "content": "Development For director Wolfgang Becker, work on Good Bye, Lenin! began in the summer of 1999, but for screenwriter Bernd Lichtenberg, the work had already begun almost a decade earlier. Lichtenberg’s experience of the reunification period as a New West Berliner at a similar age to his protagonist Alex was formed into a story which already included many aspects of the later film, but first ended up \"in the drawer\" for a few years. He stated: “I had the feeling that it simply wasn’t the right time yet.” This only changed when he saw Becker's Life is All You Get (German: Das Leben ist eine Baustelle). Especially interested in the mix of sadness and comedy, which he himself also envisaged for his film, he believed he had found the right person to bring his idea to life. He was not mistaken. \"All of a sudden there was this energy\", recalls producer Stefan Arndt during the recording of the 5-page synopsis with Becker. “We knew we could tell the story in just the way we would like to.”"}, {"id": "pubmed23n0824_6126", "title": "A 9-year-old girl with persistent obsessive and compulsive behaviors in a primary care pediatric practice.", "score": 0.009174311926605505, "content": "Chloe is a 9-year-old gal whose mother made an initial visit to a new pediatrician for concerns about her behavior. Chloe is apprehensive about the visit and frequently hides behind her mother.Her parents first noticed Chloe becoming angry and more emotional 3 years ago, which her parents did not initially understand. However, over the past year, she has started to have more worries and unusual behavior.Chloe and her mother report that when she walks through doorways, she will almost always go back and walks through again. At home, she will walk through doorways multiple times and at school, she will pretend she forgot something so her friends do not notice. She often will not walk downstairs and occasionally her mother has to carry her. Clothes are problematic for Chloe. If her father touches something of a specific color and then touches Chloe, she will have to change her clothes or take a shower. Sometimes, she will never be able to wear those clothes again. She had a recent episode where she could not stop tapping a red paper, because if she stopped, she said it would burst into flame. During the 2 weeks before the pediatric visit, symptoms increased to the point that she is now refusing to go to school. When she stays home, she lays in 1 place all day.Chloe is a fourth grade student. The family does not report academic concerns. She has friends. She denies any appetite or sleep problems. She endorses periods of sadness, lack of energy, and decreased interest in social activities, mostly because she worries and is embarrassed. She kept her behaviors hidden from her 5 siblings for the past year, and she talked only to her mother about them. She is worried her friends might discover her behaviors.The family history is notable for multiple paternal family members with anxiety and bipolar disorder and depression on mother's side. A few months ago, Chloe's family adopted a 7-year-old child with special needs from China.Her growth, vital signs, and physical examination are unremarkable. Her mother filled out the Short Mood and Feelings Questionnaire and the Screen for Child Anxiety-Related Emotional Disorders, which both had elevated scores."}, {"id": "wiki20220301en000_158009", "title": "Elvis Costello", "score": 0.009174311926605505, "content": "1994 as himself on The Larry Sanders Show in the episode \"People's Choice\" 1996 as himself on The Larry Sanders Show in the episode \"Everybody Loves Larry\" 1997 as a barman in Spice World 1999 as himself in Austin Powers: The Spy Who Shagged Me, performing Burt Bacharach's \"I'll Never Fall In Love Again\" (with Bacharach), which also appears on its soundtrack album. 1999 as a younger version of himself in 200 Cigarettes 2001 as himself performing \"Fly Me to the Moon\" on the series finale of 3rd Rock from the Sun 2002 as himself on the episode \"How I Spent My Strummer Vacation\" of The Simpsons 2003 as Ben on Frasier, in the season 10 episode \"Farewell Nervosa\" 2003 as himself in I Love Your Work 2004 as himself in the UK TV Dead Ringers New Year Special, apparently and reportedly having serendipitously entered a filming venue. 2004 as himself in Two and a Half Men – Season 2, Episode 1 2004 as himself in De-Lovely 2006 as himself in Delirious"}, {"id": "pubmed23n0895_1202", "title": "Bullying and ADHD: Which Came First and Does it Matter?", "score": 0.00909090909090909, "content": "Aiden, a 13-year-old boy in the sixth grade who is relatively new to your practice, is seen for follow-up after his routine physical last month when you noted concerns for possible attention-deficit hyperactivity disorder (ADHD) and gave the family Vanderbilt Scales to complete. Aiden has a family history of ADHD, specific learning disabilities, and mood disorder.His mother reports that she is concerned about how Aiden is doing at school; his teachers are complaining that he is not doing his work, and she is worried that he may be kept back in school. Aiden first began having trouble in the third grade. He was retained in the fourth grade for academic and behavioral reasons. Now his mother has been receiving calls about him not paying attention, distracting others, and staring at his paper. At home, he does not want to do homework and gets very frustrated. In fifth grade, he had a psychoeducational evaluation and was found not eligible for services. His achievement testing showed average scores in reading, math, and writing. Cognitive testing demonstrated average scores for verbal and nonverbal abilities and memory but was significantly below average for processing speed. Aiden continues to have problems now in into the sixth grade.You speak with Aiden in the office and ask him about school. He says, \"It's bad. I'm failing.\" He believes his major problems at school are that he is not doing his homework, he easily becomes frustrated, and he argues with the teachers. He has supportive relationships with his family and friends at school. He gets along well with some of his teachers, noting that he loves his science teacher even though she is tough and \"gives hard homework.\" He describes his history teacher as \"annoying.\" When you ask what he means he states this teacher \"Can be not nice and says mean things. She picks on me a lot.\" His description is consistent with the use of shaming as a behavior he experiences at school.You review the completed parent and teacher Vanderbilt forms; both are consistent and concerning for combined type ADHD. You discuss the diagnosis of ADHD with his mother and both agree to revisit pharmacotherapy in September when the school year resumes. You give her resources on ADHD and classroom accommodations and discuss requesting a 504 plan at school. You also discuss behavioral therapy to better address his self-regulation skills.A week later, you receive a telephone call from Aiden's mother. \"Aiden got home today and he is more upset than I have ever seen him! His teacher told him in front of the class that he would probably stay back a year and now he is saying there is no point in going to school.\" She is not aware if retention has been recommended for Aiden.What would you say to Aiden's mother? What would you do next?"}, {"id": "wiki20220301en045_6056", "title": "Suzy Favor Hamilton", "score": 0.00909090909090909, "content": "In December 2012, after being confronted by a reporter, Favor Hamilton admitted that she had worked as an escort prostitute. Favor said her decision to become an escort was made under the influence of her antidepressant medication, a misdiagnosis of her bipolar disorder, and an unfamiliarity with the mental illness, and with the encouragement and prodding of her husband. She cited the effects of the suicide of her brother, Dan, in 1999, on her condition. She had learned from her therapist that the antidepressant she was taking had put her in a manic state, saying \"It wasn't Suzy. I keep trying to emphasize that wasn't me. It was the disease.\" After her prostitution became public, the Big Ten renamed its award for Female Athlete of the Year which had previously carried her name. Favor Hamilton also lost several sponsorships and athletic business relationships, including with Nike. She is now a speaker at mental health conferences, and wrote her memoir Fast Girl about healing from"}, {"id": "pubmed23n0772_17523", "title": "Bullying and ADHD: which came first and does it matter?", "score": 0.009009009009009009, "content": "Aiden, a 13-year-old boy in the sixth grade who is relatively new to your practice, is seen for follow-up after his routine physical last month when you noted concerns for possible attention-deficit hyperactivity disorder (ADHD) and gave the family Vanderbilt Scales to complete. Aiden has a family history of ADHD, specific learning disabilities, and mood disorder.His mother reports that she is concerned about how Aiden is doing at school; his teachers are complaining that he is not doing his work, and she is worried that he may be kept back in school. Aiden first began having trouble in the third grade. He was retained in the fourth grade for academic and behavioral reasons. Now his mother has been receiving calls about him not paying attention, distracting others, and staring at his paper. At home, he does not want to do homework and gets very frustrated. In fifth grade, he had a psychoeducational evaluation and was found not eligible for services. His achievement testing showed average scores in reading, math, and writing. Cognitive testing demonstrated average scores for verbal and nonverbal abilities and memory but was significantly below average for processing speed. Aiden continues to have problems now in into the sixth grade.You speak with Aiden in the office and ask him about school. He says, \"It's bad. I'm failing.\" He believes his major problems at school are that he is not doing his homework, he easily becomes frustrated, and he argues with the teachers. He has supportive relationships with his family and friends at school. He gets along well with some of his teachers, noting that he loves his science teacher even though she is tough and \"gives hard homework.\" He describes his history teacher as \"annoying.\" When you ask what he means he states this teacher \"Can be not nice and says mean things. She picks on me a lot.\" His description is consistent with the use of shaming as a behavior he experiences at school.You review the completed parent and teacher Vanderbilt forms; both are consistent and concerning for combined type ADHD. You discuss the diagnosis of ADHD with his mother and both agree to revisit pharmacotherapy in September when the school year resumes. You give her resources on ADHD and classroom accommodations and discuss requesting a 504 plan at school. You also discuss behavioral therapy to better address his self-regulation skills.A week later, you receive a telephone call from Aiden's mother. \"Aiden got home today and he is more upset than I have ever seen him! His teacher told him in front of the class that he would probably stay back a year and now he is saying there is no point in going to school.\" She is not aware if retention has been recommended for Aiden.What would you say to Aiden's mother? What would you do next?"}, {"id": "wiki20220301en282_24664", "title": "Folie à deux", "score": 0.009009009009009009, "content": "Mood-congruent delusions These correspond to a person's emotions within a given timeframe, especially during an episode of mania or depression. For example, someone with this type of delusion may believe with certainty that they will win $1 million at the casino on a specific night despite lacking any way to see the future or influence the probability of such an event. Similarly, someone in a depressive state may feel certain that their mother will get hit by lightning the next day, again in spite of having no means of predicting or controlling future events. Mood-neutral delusions These are unaffected by mood, and can be bizarre or non-bizarre; the formal definition provided by Mental Health Daily is \"a false belief that isn't directly related to the person's emotional state.\" An example would be a person who is convinced that somebody has switched bodies with their neighbor, the belief persisting irrespective of changes in emotional status."}, {"id": "pubmed23n0882_3374", "title": "Chronic Headaches After a Concussion in an Obese 16-Year-Old Girl.", "score": 0.008928571428571428, "content": "Jennifer is a 16-year-old Latina girl who is new to your practice. During her first well visit, she mentions that she has had daily headaches for 2 years. They began after sustaining a concussion in a car accident. Typically, her headaches are bilateral and \"squeezing\"; they occur in the afternoons and last for a few hours. Her concussion also resulted in depressed mood, which has improved over time.When you ask if her headaches have changed recently, she says that they have been worse for the last few days. The quality and severity are unchanged; however, they now occur first thing in the morning, are worse when supine, and no longer remit. In the last 2 days, she has developed new-onset blurry vision, nausea, dizziness, photophobia, and sonophobia. Although she previously experienced sadness with her concussion, she now feels irritable. She has never used tobacco, alcohol, or drugs, and she takes no medications.On examination, her body mass index is above the 99th percentile. You note mild papilledema bilaterally. She has no focal neurological deficits. The remainder of her examination is normal.You send her to an emergency department. Her head computed tomography is normal. A lumbar puncture demonstrates an opening pressure of 32 cm H2O; she feels relief after the procedure. She is admitted with a diagnosis of benign intracranial hypertension and is started on acetazolamide. What is the differential diagnosis of chronic headaches in an obese adolescent? How should a busy community pediatrician manage Jennifer acutely? What follow-up care should Jennifer receive?"}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 85]], "word_ranges": [[0, 10]], "text": "Increase enalapril dose according to tolerance and administer intravenous turosemide."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Increase enalapril dose according to tolerance and administer intravenous turosemide.", "full_answer_no_ref": "Increase enalapril dose according to tolerance and administer intravenous turosemide.", "full_question": "A 73-year-old woman is admitted with progressive dyspnea until she becomes at rest, orthopnea and weight gain of 4 kg. Physical examination showed blood pressure of 150/84 mm Hg, heart rate 100 beats/minute, increased jugular venous pressure, crepitant in both bases and malleolar edema. Usual treatment: enalapril 5 mg every 12 hours, furosemide 80 mg per day. What is the most appropriate treatment at this time?", "id": 285, "lang": "en", "options": {"1": "Administer fiirosemide intravenously.", "2": "Increase enalapril dose according to tolerance and administer intravenous furosemide.", "3": "Start a beta-blocker.", "4": "Add treatment with amlodipine.", "5": NaN}, "question_id_specific": 59, "type": "CARDIOLOGY AND VASCULAR SURGERY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0415_3568", "title": "Effects of high-dose furosemide and small-volume hypertonic saline solution infusion in comparison with a high dose of furosemide as bolus in refractory congestive heart failure: long-term effects.", "score": 0.014355659076864574, "content": "Diuretics have been accepted as first-line treatment in refractory congestive heart failure (CHF), but a lack of response to them is a frequent event. A randomized, single-blind study was performed to evaluate the effects of the combination of high-dose furosemide and small-volume hypertonic saline solution (HSS) infusion in the treatment of refractory New York Heart Association (NYHA) class IV CHF and a normosodic diet during follow-up. Materials and Methods One hundred seven patients (39 women and 68 men, age range 65-90 years) with refractory CHF (NYHA class IV) of different etiologies, who were unresponsive to high oral doses of furosemide, angiotensin-converting enzyme inhibitors, digitalis, and nitrates, were enrolled. Inclusion criteria included an ejection fraction (EF) &lt;35%, serum creatinine level &lt;2 mg/dL, blood urea nitrogen level &lt; or =60 mg/dL, reduced urinary volume, and low natriuresis. The patients were randomized in 2 groups (single-blind). Patients in group 1 (20 women and 33 men) received an intravenous (IV) infusion of furosemide (500-1000 mg) plus HSS (150 mL of 1.4%-4.6% NACl) twice a day in 30 minutes. Patients in group 2 (19 women and 35 men) received an IV bolus of furosemide (500-1000 mg) twice a day, without HSS, during a period lasting 6 to 12 days. Both groups received IV KCl (20-40 mEq) to prevent hypokalemia. At study entry, all patients underwent a physical examination and measurement of body weight (BW), blood pressure (BP), and heart rate (HR), an evaluation of signs of CHF, and measurement of control levels of serum Na, K, Cl, bicarbonate, albumin, uric acid, creatinine, urea, and glycemia daily during hospitalization, and measurements of the daily output of urine for Na, K, and Cl. A chest radiograph, electrocardiogram, and echocardiogram were obtained at study entry, during hospitalization, and at the time of discharge from the hospital. During the treatment and after discharge, the daily dietary Na intake was 120 mmol in group 1 versus 80 mmol in group 2, with a fluid intake of 1000 mL daily in both groups. An assessment of BW and 24-hour urinary volume, serum, and urinary laboratory parameters were performed daily until patients reached a compensated state, when IV furosemide was replaced with oral administration (250-500 mg/d). After discharge from the hospital, patients were observed as outpatients weekly for the first 3 months and, subsequently, once a month. The groups were similar in age, sex, EF, risk factors, treatment, and etiology of CHF. All patients showed a clinical improvement. Ten patients in both groups had hyponatremia at entry. A significant increase in daily diuresis and natriuresis was observed in both groups, but it was more significant in the group receiving HSS (P &lt;.05). The serum Na level increased in group 1 and decreased in group 2 (P &lt;.05). The serum K level was decreased in both groups (P &lt;.05). BW was reduced in both groups (P &lt;.05). Group 2 had an increase in serum creatinine level. Serum uric acid levels increased in both groups. BP values decreased and HR was corrected to normal values in both groups. In the follow-up period (31 +/- 14 months), 25 patients from group 1 were readmitted to the hospital for heart failure. In group 2, 43 patients were readmitted to the hospital at a higher class than at discharge. Twenty-four patients in group 1 died during follow-up, versus 47 patients in group 2 (P &lt;.001). This treatment is effective and well tolerated, improves the quality of life through the relief of signs and symptoms of congestion, and may delay more aggressive treatments. The effects were also beneficial in a long period for mortality reduction (55% vs 13% survival rate) and for clinical improvement."}, {"id": "wiki20220301en031_69247", "title": "Loop diuretic", "score": 0.014130483387738674, "content": "Pulmonary edema - Slow intravenous bolus dose of 40 to 80 mg furosemide at 4 mg per minute is indicated for patients with fluid overload and pulmonary odema. Such dose can be repeated after 20 minutes. After the bolus, a continuous intravenous infusion can be given at 5 to 10 mg per hour. For those with underlying renal impairment or severe heart failure, up to 160 to 200 mg bolus dose can be given."}, {"id": "pubmed23n0085_20484", "title": "Treatment of severe hypertension with atenolol and betaxolol with once-daily regimens. Hemodynamic aspects.", "score": 0.01409470277635837, "content": "The effectiveness and safety of once-daily administration of drugs in the treatment of moderate to severe hypertension was studied. Forty men taking diuretics were randomized to atenolol (A, n = 18), 50 mg/day, or betaxolol (B, n = 22), a new B1-blocker, 20 mg/day, if their SDAP was 105 to 125 mm Hg at baseline (weeks 2 to 4). At week 6, if SDAP was greater than 95 mm Hg, minoxidil (M), 5.5 mg/day, was added. The patients were seen every two weeks to week 16 (end of drug titration) and then every four weeks to week 32. The dosages were increased to 200 mg/day for A, 80 mg/day for B, and 20 mg/day for M as needed. Physical examinations, chest x-ray films, ECGs, echocardiograms, spirometric studies, 24-h ambulatory arterial pressures (AAP), and blood chemistry analyses were done at baseline and during treatment. A and B combined with a diuretic (furosemide, F) and M decreased the arterial pressures and heart rates equally well by both clinical and AAP measurements (p less than .001). The IVS was decreased (p less than .05), whereas LVIDd, RVIDd, and cardiothoracic ratios were increased by both A and B (p less than .05, p less than .01). No changes were noted in LVPW, LVM, EF, FS, spirometric values, or blood chemistry analyses. Common side effects were weight gain, edema, and hypertrichosis. Once-daily administration of A or B in combination with F and M were effective in the treatment of moderate to severe hypertension. Although effective, prolonged use of M may lead to volume overload and cardiomegaly. The significance of these latter findings is not yet known."}, {"id": "pubmed23n0315_430", "title": "Randomised trial of high-dose isosorbide dinitrate plus low-dose furosemide versus high-dose furosemide plus low-dose isosorbide dinitrate in severe pulmonary oedema.", "score": 0.013471971066907775, "content": "Nitrates and furosemide, commonly administered in the treatment of pulmonary oedema, have not been compared in a prospective clinical trial. We compared the efficacy and safety of these drugs in a randomised trial of patients with severe pulmonary oedema and oxygen saturation below 90%. Patients presenting to mobile emergency units with signs of congestive heart failure were treated with oxygen 10 L/min, intravenous furosemide 40 mg, and morphine 3 mg bolus. 110 patients were randomly assigned either to group A, who received isosorbide dinitrate (3 mg bolus administered intravenously every 5 min; n=56) or to group B, who received furosemide (80 mg bolus administered intravenously every 15 min, as well as isosorbide dinitrate 1 mg/h, increased every 10 min by 1 mg/h; n=54). Six patients were withdrawn on the basis of chest radiography results. Treatment was continued until oxygen saturation was above 96% or mean arterial blood pressure had decreased by 30% or to below 90 mm Hg. The main endpoints were death, need for mechanical ventilation, and myocardial infarction. The analyses were by intention to treat. Mechanical ventilation was required in seven (13%) of 52 group-A patients and 21 (40%) of 52 group-B patients (p=0.0041). Myocardial infarction occurred in nine (17%) and 19 (37%) patients, respectively (p=0.047). One patient in group A and three in group B died (p=0.61). One or more of these endpoints occurred in 13 (25%) and 24 (46%) patients, respectively (p=0.041). High-dose isosorbide dinitrate, given as repeated intravenous boluses after low-dose intravenous furosemide, is safe and effective in controlling severe pulmonary oedema. This treatment regimen is more effective than high-dose furosemide with low-dose isosorbide nitrate in terms of need for mechanical ventilation and frequency of myocardial infarction."}, {"id": "pubmed23n0094_13483", "title": "Dose-ranging study of isosorbide-5-mononitrate in chronic congestive heart failure treated with diuretics and angiotensin-converting enzyme inhibitor.", "score": 0.01318975955717004, "content": "The hemodynamic response of isosorbide-5-mononitrate (IS-5-MN) to the addition of the widely used therapy of diuretic drugs and the maximally tolerated dose of enalapril for heart failure was assessed in 8 patients with congestive heart failure (CHF) (New York Heart Association class II and III). The diuretic therapy was furosemide, 40 to 80 mg/day, with or without amiloride, 5 to 10 mg/day. The dose of enalapril was 5 to 20 mg/day. Four hours after the administration of the morning dose of enalapril, a Swan-Ganz catheter was positioned in the pulmonary artery. Patients received increasing doses of IS-5-MN to produce a satisfactory decrease in pulmonary capillary wedge pressure. Two of the first 3 patients studied had a large reduction in blood pressure when given 10 mg of IS-5-MN. Subsequent patients were therefore given an initial dose of 5 mg, the total dose being 5 to 20 mg over 2 hours. Results at baseline and 1 hour after the final dose of IS-5-MN are expressed as mean +/- standard deviation. Both pulmonary artery systolic and diastolic pressures decreased significantly (p less than 0.05) by 12.2 +/- 8.9/4.2 +/- 5.2 mm Hg, from 47.2 +/- 16.0/21.6 +/- 6.0 mm Hg to 35.0 +/- 15.2/17.4 +/- 9.3 mm Hg. Pulmonary capillary wedge pressure decreased by 8.6 +/- 4.4 mm Hg, from 22.1 +/- 5.4 to 13.6 +/- 7.5 mm Hg (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0320_22194", "title": "Long-term efficacy, tolerability, and safety of the combination of enalapril and felodipine ER in the treatment of hypertension. Enalapril-Felodipine ER Factorial Study Group.", "score": 0.011322565168719014, "content": "A recent 8-week, double-masked, placebo-controlled, 3 x 4 factorial-design study demonstrated that enalapril-felodipine extended-release (ER) combinations had statistically significant additive effects for reducing both sitting systolic blood pressure (SiSBP) and sitting diastolic blood pressure (SiDBP) and were generally well tolerated in hypertensive patients with SiDBPs ranging from 95 to 115 mm Hg. The present open-label study was undertaken to assess the long-term efficacy, tolerability, and safety of such combinations. Patients from the factorial study were eligible for the 1-year, open-label extension. Initially, all patients received enalapril 5 mg-felodipine ER 2.5 mg once daily; if SiDBP was not controlled (&lt; 90 mm Hg) after 4 weeks of treatment, the dose was titrated upward at 2- to 4-week intervals to a maximum of enalapril 10 mg-felodipine ER 10 mg. Hydrochlorothiazide (HCTZ) 12.5 mg was added to the regimen of patients whose hypertension was not controlled at the highest enalapril-felodipine ER dose. A total of 507 patients were enrolled, of whom 502 were assessable. At their last study visit, 391 (78%) of the assessable patients were receiving only an enalapril-felodipine ER combination. The enalapril-felodipine ER combinations resulted in mean trough SiDBPs of 85 to 89 mm Hg (decreases of 13 to 16 mm Hg from baseline) and SiSBPs of 137 to 140 mm Hg (decreases of 13 to 21 mm Hg). Overall, 407 (81%) of the 502 assessable patients achieved an SiDBP &lt; 90 mm Hg or a reduction from baseline &gt; or = 10 mm Hg (responders); such a response was recorded in 331 patients (66%) taking a combination of enalapril-felodipine ER alone and 76 patients (15%) taking the combination with the addition of HCTZ 12.5 mg. Blood pressure reductions were maintained throughout the treatment period. Drug-related adverse events were relatively infrequent, often transient, usually mild, and apparently not dose related. The most frequently reported drug-related adverse events were edema/swelling, asthenia/fatigue, dizziness, cough, and headache. These results suggest that combination therapy with enalapril-felodipine ER is effective for long-term blood pressure reduction, has an excellent safety profile, and is generally well tolerated. Addition of low-dose HCTZ to the enalapril-felodipine ER combination appears to provide further blood pressure control without increasing drug-related adverse events."}, {"id": "wiki20220301en010_106213", "title": "Ascites", "score": 0.010112516273014693, "content": "Monitoring diuresis: Diuresis can be monitored by weighing the person daily. The goal is weight loss of no more than 1.0 kg/day for people with both ascites and peripheral edema and no more than 0.5 kg/day for people with ascites alone. If daily weights cannot be obtained, diuretics can also be guided by the urinary sodium concentration. Dosage is increased until a negative sodium balance occurs. A random urine sodium-to-potassium ratio of > 1 is 90% sensitivity in predicting negative balance (> 78-mmol/day sodium excretion). Diuretic resistance: Diuretic resistance can be predicted by giving 80 mg intravenous furosemide after 3 days without diuretics and on an 80 mEq sodium/day diet. The urinary sodium excretion over 8 hours < 50 mEq/8 hours predicts resistance."}, {"id": "wiki20220301en031_69246", "title": "Loop diuretic", "score": 0.009900990099009901, "content": "Clinical use Loop diuretics are principally used in the following indications: Heart failure - Giving 2.5 times of previous oral dose twice daily for those with acute decompensated heart failure is a reasonable strategy. However, daily assessment of clinical response is needed to adjust the subsequent doses. Edema associated with liver cirrhosis, and nephrotic syndrome Cerebral edema - intravenous furosemide can be combined with mannitol to initiate rapid diuresis. However, the optimum duration of such treatment remains unknown. Frequent fluid status monitoring is required to prevent intravascular volume depletion which leads to reduced cerebral perfusion. A bolus intravenous dose of 10 or 20 mg of furosemide can be administered and then followed by intravenous bolus of 2 or 3% hypertonic saline to increase the serum sodium level."}, {"id": "pubmed23n0107_12338", "title": "Effect of felodipine in refractory hypertension.", "score": 0.009900990099009901, "content": "Felodipine (Plendil), a new drug, has been used in the treatment of five patients with refractory essential hypertension (WHO II-III). Their mean blood pressure at the last outpatient visit before the study was opened was 195 +/- 25/129 +/- 21 mmHg (mean +/- s.d.) (range 175-235/110-165 mmHg), despite treatment with combinations of diuretics, beta-blockers and vasodilators, including minoxidil and captopril. Felodipin is a dihydropyridine derivative, a calcium antagonist that exerts a relaxant effect on resistance vessels. The first period of the study consisted of a 5-day stay in hospital followed by 3 months during which observations were carried out at the Outpatients' Department. After the first days in hospital felodipine therapy was introduced at a dose of 25 mg three times daily, given together with diuretics, beta-blockers and, in one case, captopril. At 8.00 immediately before the first dose was given, the blood pressure was 178 +/- 19/118 +/- 19 mmHg (mean +/- s.d.); 2 h later it was 144 +/- 18/85 +/- 4 mmHg, at which level it remained throughout the rest of the study. At the 3-month follow-up the mean pressure (recorded at the Outpatients' Department) was 138 +/- 20/89 +/- 14 mmHg. Side-effects included headache, flushing, palpitations and ankle oedema (in two patients during the second part of the study); they were of a mild to moderate degree and did not interfere with the treatment. There was no evidence of general fluid retention, and the body weight remained constant.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0596_19971", "title": "The effects of amlodipine and enalapril on renal function in adults with hypertension and nondiabetic nephropathies: a 3-year, randomized, multicenter, double-blind, placebo-controlled study.", "score": 0.00980392156862745, "content": "Placebo-controlled trials have found that angiotensin-converting enzyme inhibitors (ACEIs) decrease proteinuria and slow the progression of nondiabetic nephropathies. However, head-to-head comparisons of ACEIs and calcium channel blockers (CCBs) have shown conflicting results. Indeed, a recent metaanalysis concluded that there is still uncertainty about the greater renoprotection seen with ACEIs or angiotensin II receptor blockers in nondiabetic patients with renal disease, particularly when using true glomerular filtration rate (GFR) as the primary outcome. The objective of this 3-year, randomized, multicenter, double-blind, placebo-controlled study was to compare true GFR decline (measured by yearly 51Cr-EDTA blood clearance) in nondiabetic, nonnephrotic adult hypertensive patients with estimated creatinine clearance of 20 to 60 mL/min.1.73 m(2), when randomized to a CCB (amlodipine, 5-10 mg/d) or an ACEI (enalapril, 5-20 mg/d). Patients (aged 18-80 years) entered a 4-week placebo run-in washout period and previous antihypertensive drugs were tapered off over 2 weeks. Add-on treatments were atenolol (50-100 mg/d), loop diuretics (furosemide, 20-500 mg/d or torsemide, 5-200 mg/d), alpha-blockers (prazosin, 2.5-5 mg/d or doxazosin, 1-16 mg/d), and centrally acting drugs (rilmenidine, 1-2 mg/d or methyldopa, 250-500 mg/d). The primary end point was true GFR measured by yearly (51)Cr-EDTA blood clearance. Secondary end points included a clinical composite of renal events and tolerability collected by a full clinical and laboratory evaluation at each study visit. Post hoc analyses for the change in GFR, proteinuria, and time to clinical events were also planned on baseline proteinuria subgroups (&lt;1 and &gt;or=1 g/d) before unblinding the database. Three hundred eighteen patients entered the run-in period and 263 patients (156 men/107 women; mean age, 58 years) were randomized to receive either amlodipine (5 mg/d, n=132) or enalapril (5 mg/d, n=131). Blood pressure declined from 165/102 mm Hg to 138/84 mm Hg and 138/85 mm Hg with amlodipine and enalapril, respectively (no between-group significance). Only 20.8% of the patients randomized to ACEI treatment received diuretics at the last observation. No statistically significant difference was found between amlodipine and enalapril in GFR decline (-4.92 and -3.98 mL/min.1.73 m(2), respectively, at last observation) and composite secondary end point after a median follow-up of 2.9 years, including in the subgroup of patients with proteinuria &gt;1 g/d at baseline. Protein excretion rate decreased significantly from baseline in patients taking enalapril plus diuretics (median -270 mg/d; P&lt;0.001) but not in patients taking amlodipine plus diuretics (-25 mg/d at last observation). In this cohort of nondiabetic, nonnephrotic hypertensive patients, no statistically significant difference in true GFR decline was found over 3 years between amlodipine-treated patients and enalapril-treated patients with main add-on treatment with ss-blockers, including in the subgroup of patients with proteinuria &gt;1 g/d."}, {"id": "pubmed23n0323_18776", "title": "[The Hypertension Optimal Treatment Study: efficacy and tolerability on the 36th month].", "score": 0.00980392156862745, "content": "The international, prospective, randomized HOT study was aimed at determining the influence of a targeted BP reduction on cardiovascular morbidity and mortality. Patients were randomly allocated to 3 DBP targets (&lt; 80, &lt; 85, &lt; 90 mmHg). In addition, the impact of a coprescription of aspirin was studied. The BP target had to be reached within 3 months, according to a well-defined strategy : felodipine 5 mg o.d. as a 1st intention drug, 1, 2 or 3 additional drugs, if necessary, on the following steps. BP measurements were made, using an oscillometric automatic device (Hestia). From April 1992 to October 1994, 18,790 patients with an age range 50-80 years, coming from 26 countries, entered the study. The data collected on the 36th month were in agreement with those obtained on the 12th and the 24th months. Baseline DBP was reduced by 21, 23 and 25 mmHg in the 90, 85 and 80 mmHg target groups, respectively. The rate of patients whose DBP reached the target, obviously increased from the 3rd to the 12th month: from 43 to 56%, 60 to 70%, 74 to 83% in the 90, 85 and 80 mmHg, target groups, respectively. From the 2nd to the 3rd year, BP control was further improved, with a slightly higher rate of controlled patients in the elderly (age &gt; 60 y), especially in the 80 mmHg target group. From inclusion to the 3rd month, one-drug treated patients decreased, whereas 2- or 3-drug treated patients increased. Felodipine-treated patients decreased on the 36th month, but remained over 80%. From the 6th to the 36th month, additional prescription of a betablocker or an ACE-inhibitor increased from 36 to 39%, and from 23 to 28%, respectively; moreover, the side-effects rate decreased from 10.5 to 3.6%, with a special decline in ankle edema from 4 to 1%. In conclusion, the BP reduction observed on the 36th month was of the same extent as that observed in the first months. It seems obviously possible to reach a targeted DBP and to maintain it over time, along with a good acceptability of the treatment. Targeted DBP could be more easily achieved in elderly patients, possibly due to a better drug compliance."}, {"id": "pubmed23n0347_9959", "title": "Hypersensitivity myocarditis associated with ephedra use.", "score": 0.009708737864077669, "content": "Ephedrine has previously been described as a causative factor of vasculitis but myocarditis has not yet been associated with either ephedrine or its plant derivative ephedra. A 39-year-old African American male with hypertension presented to Rush Presbyterian St. Luke's Medical Center with a 1-month history of progressive dyspnea on exertion, orthopnea, and dependent edema. He was taking Ma Huang (Herbalife) 1-3 tablets twice daily for 3 months along with other vitamin supplements, pravastatin, and furosemide. Physical examination revealed a male in mild respiratory distress. The lung fields had rales at both bases without audible wheezes. Internal jugular venous pulsations were 5 cm above the sternal notch. Medical therapy with intravenous furosemide and oral enalapril was initiated upon admission. Cardiac catheterization with coronary angiography revealed normal coronary arteries, a dilated left ventricle, moderate pulmonary hypertension, and a pulmonary capillary wedge pressure of 34 mm Hg. The patient had right ventricular biopsy performed demonstrating mild myocyte hypertrophy and an infiltrate consisting predominantly of lymphocytes with eosinophils present in significantly increased numbers. Treatment for myocarditis was initiated with azothioprine 200 mg daily and prednisone 60 mg per day with a tapering course over 6 months. Anticoagulation with warfarin and diuretics was initiated and angiotensin-converting enzyme inhibition was continued. Hydralazine was added later. One month into therapy, an echocardiogram demonstrated improved left ventricular function with only mild global hypokinesis. A repeat right ventricular biopsy 2 months after the first admission showed no evidence of myocarditis. At 6 months, left ventricular ejection fraction was normal (EFN 50%) and the patient asymptomatic. Ephedra (Ma Huang) is the suspected cause of hypersensitivity myocarditis in this patient due to the temporal course of disease and its propensity to induce vasculitis."}, {"id": "pubmed23n0334_11641", "title": "Sildenafil citrate and blood-pressure-lowering drugs: results of drug interaction studies with an organic nitrate and a calcium antagonist.", "score": 0.009615384615384616, "content": "Sildenafil, a selective inhibitor of cyclic guanosine monophosphate (cGMP)-specific phosphodiesterase type 5 (PDE5), is a well-tolerated and highly effective treatment for erectile dysfunction. The mechanism of action of sildenafil depends on activation of the nitric oxide (NO)-cGMP pathway during sexual stimulation, which results in corpus cavernosal smooth muscle relaxation and penile erection. Endogenously derived NO is also involved in blood pressure regulation through its effect on basal vascular tone, which is mediated by cGMP levels. Organic nitrates and NO donors exert their therapeutic effects on blood pressure and vascular smooth muscle by the same mechanism as endogenous NO. Since both sildenafil and organic nitrates exert their pharmacologic effects via increases in cGMP concentrations, a double-blind, placebo-controlled, crossover study was undertaken to investigate the effects of sildenafil coadministered with glyceryl trinitrate on blood pressure and heart rate in healthy male subjects. The hemodynamic effects of sildenafil were also evaluated in a second placebo-controlled crossover study in men with hypertension who were taking the calcium antagonist amlodipine, which has a mechanism of action that does not involve the cGMP pathway. In the first crossover study, subjects were treated with oral sildenafil (25 mg, 3 times a day for 4 days) or placebo and then challenged on day 4 with a 40-minute, stepwise, intravenous infusion of glyceryl trinitrate (0.5 mg/mL in 5% dextrose at an initial infusion rate of 2.5 microg/min and doubling every 5 minutes to a maximum rate of 40 microg/min) 1 hour after taking sildenafil or placebo. On day 5, subjects received a sublingual glyceryl trinitrate tablet (500 microg) 1 hour after taking 25 mg of sildenafil or placebo. During sildenafil treatment, the subjects were significantly less tolerant of intravenously administered glyceryl trinitrate than during placebo treatment, based on the occurrence of a &gt;25 mm Hg decrease in blood pressure or the incidence of symptomatic hypotension (p &lt;0.01). When a sublingual glyceryl trinitrate tablet was administered on day 5, a 4-fold greater decrease in systolic blood pressure was observed for the subjects during the sildenafil treatment period than during the placebo treatment period. The changes in heart rate were negligible during both glyceryl trinitrate challenges. In conclusion, sildenafil potentiated the hypotensive effects of glyceryl trinitrate, an organic nitrate. Thus, sildenafil administration to patients who are using organic nitrates, either regularly and/or intermittently, in any form is contraindicated. In the second crossover study, men with hypertension, who were taking 5 or 10 mg/day of amlodipine, received a single oral dose of 100 mg sildenafil or placebo. Coadministration of sildenafil did not significantly affect the pharmacokinetics of amlodipine. In the 4 hours after dosing, differences in the mean maximum change from baseline in supine systolic and diastolic blood pressures between the sildenafil plus amlodipine and the placebo plus amlodipine treatment periods were -8 mm Hg and -7 mm Hg, respectively (p &lt; or =0.002). The mean maximum supine heart rate increased 2.1 beats/min during sildenafil plus amlodipine treatment and decreased 1.5 beats/min during placebo plus amlodipine treatment (p &lt;0.02). The adverse events in this study were predominantly mild or moderate and did not cause discontinuation of treatment. Adverse events considered to be related to sildenafil treatment included headache, nausea, and dyspepsia. In patients with hypertension who were taking amlodipine therapy, sildenafil produced additive, but not synergistic, reductions in blood pressure. The difference in the mean maximum change from baseline in blood pressure between sildenafil plus amlodipine and placebo plus amlodipine was comparable to the decrease in blood pressure reported for healthy men taking sildenafil alone. (ABSTRACT TRUNCATED)"}, {"id": "pubmed23n0133_18346", "title": "[Comparative study of 2 diuretic-containing combination preparations in patients with edematous heart failure].", "score": 0.009615384615384616, "content": "The efficacy and tolerability of two combinations, namely 50 mg spironolactone + 20 mg furosemide (SF) or 50 mg spironolactone + 5 mg butizide (SB), were compared in a randomised intraindividual trial in 22 patients with congestive heart failure. The parameters used were: weight, ankle- and calf-circumference, blood pressure, resting pulse, resting ECG, spirometry and blood chemistry. The physicians' judgement of the success of treatment was also recorded. Clinical symptoms improved clearly in both groups and in most cases there was significant improvement of the various parameters. The trend towards improvement was more apparent with SF. The physicians considered SF to be more effective in 12 cases compared to one case with SB. In all other cases both treatments were considered equally effective. The blood chemistry data showed relevant differences: serum-potassium levels were less scattered with SF and showed a - desirable - shift into the upper normal range. The number of patients with elevated serum-creatinin-levels increased during SB-treatment whereas the opposite was noted with SF. This could be due to furosemide's positive effects on renal functions."}, {"id": "wiki20220301en238_21745", "title": "Paracetamol poisoning", "score": 0.009523809523809525, "content": "Intravenous acetylcysteine is given as a continuous infusion over 20 hours for a total dose 300 mg/kg. Recommended administration involves infusion of a 150 mg/kg loading dose over 15 to 60 minutes, followed by a 50 mg/kg infusion over four hours; the last 100 mg/kg are infused over the remaining 16 hours of the protocol. Intravenous acetylcysteine has the advantage of shortening hospital stay, increasing both doctor and patient convenience, and allowing administration of activated charcoal to reduce absorption of both the paracetamol and any co-ingested drugs without concerns about interference with oral acetylcysteine. Intravenous dosing varies with weight, specifically in children. For patients less than 20 kg, the loading dose is 150 mg/kg in 3 mL/kg diluent, administered over 60 minutes; the second dose is 50 mg/kg in 7 mL/kg diluent over 4 hours; and the third and final dose is 100 mg/kg in 14 mL/kg diluent over 16 hours."}, {"id": "pubmed23n0112_10375", "title": "A long-term open study of a frusemide/amiloride combination ('Frumil') in elderly patients with congestive cardiac failure.", "score": 0.009523809523809525, "content": "Twenty-seven patients (mean age 72 years) with symptoms of congestive cardiac failure who had been controlled by treatment with 1 to 2 tablets per day of a combination preparation of frusemide (40 mg) and amiloride (5 mg) for at least 12 weeks were studied. The study was designed to assess the efficacy and tolerability of continuing treatment with the combination for a further 9 months, i.e. a total period of 12 months. Assessments of disease status and laboratory data were made every 3 months. Reasonable control of symptoms was achieved over the 12-month period although the majority of patients did not show a clinically important change. Some overall trends towards increasing oedema, dyspnoea and orthopnoea were observed as the study progressed, but these changes were not regarded as unusual considering the patients' age group. Significant increases in pulse rate and decreases in blood pressure were demonstrated, suggesting that diuresis was maintained. The combination preparation was well tolerated without any reports of side-effects."}, {"id": "pubmed23n0742_14212", "title": "Severe hypertension and pulmonary edema associated with systemic absorption of topical phenylephrine in a child during retinal surgery.", "score": 0.009433962264150943, "content": "Topical phenylephrine solutions are widely used in eye procedures to promote pupil dilation without cycloplegia. We report a case of intraoperative severe hypertension and acute pulmonary edema occurring in a child during retinal surgery after possible systemic absorption of topical phenylephrine eyedrops. Our objective is to discuss the proper treatment and preventive strategies for such a complication. A 4-year-old, male patient, 18.4 kg in weight, physical status ASA I was admitted for right retinal detachment surgery. Anesthesia was induced with sevoflurane in oxygen, followed by glycopyrrolate (5.0 μg/kg), propofol 25 mg, fentanyl 50 μg and cisatracurium 0.15 mg/kg given intravenously. Anesthesia was maintained with sevoflurane 2-2.5% in a mixture of nitrous oxide and oxygen (60%:40%). After incision, two drops of 10% aqueous phenylephrine were administered topically by the surgeon to the right eye for further pupil dilation. Few minutes later, the noninvasive blood pressure rose to 220/120 mmHg and the heart rate increased to 140 beats/min. Oxygen saturation (SpO(2)) dropped from 99% (with an inspired oxygen concentration (FiO(2)) of 0.4) to 82%. Auscultation revealed crepitations throughout the chest and a blood-stained frothy fluid was aspirated from the trachea with possible development of acute pulmonary edema. Hydralazine (5 mg) and furosemide (10 mg) were administered intravenously. Seven minutes later, the blood pressure returned to normal and the SpO(2) increased to 92% on FiO(2) of 1.0, with decreased intratracheal secretions. After approximately 20 minutes, the SpO(2) had improved to 99%, with a FiO(2) of 1.0 and the blood pressure was 109/63 mmHg and heart rate was 121 beats/min. The FiO(2) gradually reduced back to 0.4 over 30 min with no further desaturation. The patient was discharged from the post anesthesia care unit 5 h after surgery with adequate spontaneous breathing, SpO(2) 99% on room air, normal blood pressure and pulmonary auscultation. Anesthesiologists and ophthalmologists should be aware of the possible cardiovascular side-effects of topical phenylephrine, and it should be used cautiously with appropriate intraoperative monitoring of hemodynamic variables. Moreover, preventive strategies to minimize systemic absorption of the drug should be taken."}, {"id": "pubmed23n0584_10591", "title": "Treatment of heart failure with normal left ventricular ejection fraction.", "score": 0.009433962264150943, "content": "Underlying causes and precipitating causes of heart failure (HF) should be treated when possible. Persons with HF and normal left ventricular ejection fraction (LVEF) should have maintenance of sinus rhythm, treatment of hypertension, myocardial ischemia, dyslipidemia, and anemia, slowing of the ventricular rate below 90 bpm, and reduction of salt overload. First-line drug treatment in the management of these persons is the use of loop diuretics combined with beta blockers and angiotensin-converting enzyme (ACE) inhibitors. If persons are unable to tolerate ACE inhibitors because of cough, angioneurotic edema, rash, or altered taste sensation, angiotensin II type I receptor antagonists (ARBs) should be given. If HF persists despite diuretics, beta blockers, and ACE inhibitors or ARBs, isosorbide dinitrate plus hydralazine should be administered. Beta blockers, verapamil, diltiazem, and digoxin may be used to slow a rapid ventricular rate in persons with supraventricular tachyarrhythmias. Digoxin should not be used in persons with HF in sinus rhythm with normal LVEF. Exercise training should be encouraged in persons with mild to moderate HF to improve functional status and to decrease symptoms."}, {"id": "pubmed23n0799_2817", "title": "Greater efficacy of aldosterone blockade and diuretic reinforcement vs. dual renin-angiotensin blockade for left ventricular mass regression in patients with resistant hypertension.", "score": 0.009345794392523364, "content": "We report the results of an echocardiographic substudy carried out in a trial comparing the effects of two different treatment strategies - mineralocorticoid receptor blockade (MRB) and dual renin-angiotensin system blockade (RASB) - in patients with resistant hypertension. Both strategies reduce left ventricular mass index (LVMI), but they have not been compared in patients with resistant hypertension. After 4-week treatment with 300 mg irbesartan + 12.5 mg hydrochorothiazide + 5 mg amlodipine, 86 patients with resistant hypertension were randomized to the add-on 25 mg spironolactone (MRB group, n = 46) or 5 mg ramipril (RASB group, n = 40) groups for 12 weeks. Treatment intensity was increased at week 4, 8 or 10 if home blood pressure (BP) was equal to or above 135/85 mmHg, by sequentially adding 20-40 mg furosemide and 5 mg amiloride (MRB group), or 10 mg ramipril and 5-10 mg bisoprolol (RASB group). Transthoracic echography was performed at baseline and week 12. Daytime ambulatory BP decreased by 19 ± 12/11 ± 8 mmHg in the MRB group and by 8 ± 13/7 ± 7 mmHg in the RASB group (P = 0.0003/0.03). LVMI decreased by 8.2 ± 18.9 g/m in the MRB group, whereas it increased by 1.8 ± 19.1 g/m in the RASB group (P = 0.03). The decreases in posterior wall thickness, left ventricular (LV) end-systolic diameter, E/e' ratio and left atrial area were significantly greater with MRB than with RASB. The difference between groups remained significant after adjustment for the decrease in ambulatory BP. In patients with resistant hypertension, MRB-based treatment decreased both BP and LVMI more efficiently than a strategy based on dual RASB."}, {"id": "pubmed23n0527_12063", "title": "[Double blind study of the efficacy and safety of the fixed dose combination of enalapril 10 mg/nitrendipine 20 mg versus the increase of amlopidine dose in essential hypertensive patients not controlled with amlodipine 5 mg].", "score": 0.009280444777076874, "content": "Combined therapy or dose-tiration are acceptable second-line therapeutic options after a first treatment failure. This double blind clinical trial compared the fixed dose combination of enalapril 10 mg/nitrendipine 20 mg (E/N) with amlopidine 10 mg (A) in 323 hypertensive patients not previously controlled with amlodipine 5 mg. After 6 weeks of treatment, the E/N and A groups had similar percentages of blood pressure normalization (55% versus 60.2%; p = 0.4588). The adverse events related with the treatment were significantly less frequent with E/N than with a (19.8% versus 37%; p = 0.0029), especially due to a lower incidence of malleolar edema in E/N (11.1% versus 33.6%; p &lt; 0.0001). Combining the efficacy and tolerability data, treatment with E/N permitted control of blood pressure of 2.8 patients per every patient with adverse events, while this rate for A was 1.6 to 1."}, {"id": "pubmed23n0086_21054", "title": "Does furosemide alter the hemodynamic response to rapid intravascular transfusion of the anemic fetal lamb?", "score": 0.009259259259259259, "content": "The purpose of this study was to define the hemodynamic response to rapid intravascular transfusion of the anemic fetal lamb and to determine whether furosemide alters that response. Sixteen experiments were performed in nine chronically instrumented gravid ewes between 0.8 and 0.9 of timed gestation. On day 1 of each experiment, each fetus was subjected to hemorrhage (40 ml/kg of estimated fetal weight) over 1 hour. On day 2, plasma was withdrawn from the stored fetal blood until the hematocrit was approximately 70%, and the packed red blood cells were returned to the fetus intravenously over 10 minutes. Each fetus received either furosemide (2 mg/kg) or control saline solution intravenously at time zero and again at 5 minutes. The order of experiments was randomly determined for each animal. Hemorrhage resulted in a similar decrease in fetal hematocrit in the two groups. The mean +/- SEM fetal hematocrit before hemorrhage was 38 +/- 3% in the furosemide group (n = 8) and 36 +/- 2% in the control group (n = 8). On day 2, the mean +/- SEM fetal hematocrit before transfusion was 28 +/- 2% in the furosemide group and 25 +/- 1% in the control group. There was no significant difference between groups in the fetal hemodynamic response to transfusion. At the end of the transfusion, the fetal central venous pressure had increased from 4.9 +/- 0.5 to 6.2 +/- 0.5 mm Hg in the furosemide group (p = 0.01) and from 3.9 +/- 0.2 to 5.8 +/- 0.3 mm Hg in the control group (p = 0.0001). Fetal mean arterial pressure increased from 42 +/- 1 to 50 +/- 1 mm Hg in the furosemide group (p = 0.0001) and from 40 +/- 1 to 46 +/- 1 mm Hg in the control group (p = 0.0007). Fetal heart rate decreased from 187 +/- 2 to 169 +/- 5 beats/min in the furosemide group (p = 0.004) and from 188 +/- 4 to 170 +/- 5 beats/min in the control group (p = 0.0008). Transfusion did not significantly change fetal pH in either group. At 120 minutes, the fetal PO2 had increased from 17 +/- 1 to 19 +/- 1 mm Hg in the furosemide group (p = 0.03) and from 19 +/- 1 to 21 +/- 2 mm Hg in the control group (p = 0.05). We conclude that rapid transfusion of the anemic fetal lamb resulted in modest increases in fetal central venous pressure and mean arterial pressure.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0213_4419", "title": "Frusemide/amiloride combination ('Frumil') in heart failure: an open, multi-centre study in general practice.", "score": 0.009259259259259259, "content": "A total of 95 patients seen in general practice with oedema associated with heart failure took part in an open study of the efficacy and tolerability of a combination tablet containing 40 mg frusemide and 5 mg amiloride. The study was of 3-months' duration and patients received a dosage of 1 to 2 tablets once daily. Efficacy was assessed by physicians' scores for ankle, leg and lumbosacral oedema and pulmonary crepitations, body weight and physicians' global clinical impression. In addition, patients recorded their symptom severity in study diaries during the first 7 days of therapy, and the day before each trial visit. On the basis of the physicians' overall impression of response to therapy, 89 (93.7%) of the patients were graded as 'excellent', 'good' or 'adequate' responders. Improvement in severity scores for oedema, crepitations and body weight also followed this pattern, although certain symptoms were mild or absent in some patients at recruitment. Nine patients were withdrawn from the study, 4 due to drug-related adverse effects. There was no evidence of any consistent change in serum potassium levels or other clinical chemistry, liver function tests or haematology during the study."}, {"id": "wiki20220301en216_28404", "title": "Clevidipine", "score": 0.009174311926605505, "content": "Cleviprex is administered intravenously and should be titrated to achieve the desired blood pressure reduction. Blood pressure and heart rate should be monitored continually during infusion. Cleviprex is a single use product that should not be diluted and should not be administered in the same line as other medications. Once the stopper is punctured, Cleviprex should be used within 12 hours and any unused portion remaining in the vial should be discarded. Change IV lines in accordance with hospital protocol. An IV infusion at 1–2 mg/hour is recommended for initiation and should be titrated by doubling the dose every 90 seconds. As the blood pressure approaches goal, the infusion rate should be increased in smaller increments and titrated less frequently. The maximum infusion rate for Cleviprex is 32 mg/hour. Most patients in clinical trials were treated with doses of 16 mg/hour or less."}, {"id": "Neurology_Adams_6746", "title": "Neurology_Adams", "score": 0.009152175721773858, "content": "In the past, when effective treatment was not available, the outcome was often fatal. Lowering of the blood pressure with antihypertensive drugs may reverse the picture in a day or two. The same can be accomplished by administering magnesium sulfate in the eclamptic woman. However, antihypertensive drugs must be used cautiously; a safe target is a pressure of 150/100 mm Hg or a 20 percent reduction in mean pressure. One may use intravenous sodium nitroprusside, 0.5 to 0.8 mg/kg/min; a calcium channel blocker such as nifedipine, 10 to 20 mg sublingually; or intravenous beta-adrenergic blockers (labetalol, 20 to 40 mg intravenously followed by an infusion at 2 mg/min, or esmolol are favored). Longer-acting antihypertensive agents, such as ACE inhibitors and calcium channel blockers, must follow these. If there is already evidence of brain edema and increased intracranial pressure, dexamethasone, 4 to 6 mg every 6 h, is sometimes added, but its effect, and the use of hyperosmolar"}, {"id": "pubmed23n0505_14631", "title": "Randomized, controlled, parallel-group comparison of ambulatory and clinic blood pressure responses to amlodipine or enalapril during and after treatment in adult chinese patients with hypertension.", "score": 0.00909090909090909, "content": "Few studies have examined the relative efficacy and tolerability of antihypertensive drug classes in Chinese populations. This study compared the efficacy, tolerability, and duration of antihypertensive effect of amlodipine besylate and enalapril in Chinese patients with hypertension, including elderly patients with isolated systolic hypertension. This randomized, double-blind, double-dummy, parallel-group dose-titration study was conducted at the Department of Medicine and Therapeutics, Chinese University of Hong Kong. Chinese patients aged 18 to 80 years with primary hypertension were enrolled. After a 4-week placebo run-in period, patients were randomly assigned to receive active oral, once-daily treatment with amlodipine (5 mg) or with enalapril (5 mg) for 14 weeks. Treatment doses were titrated at weeks 4 and 8 if necessary according to blood pressure (BP) response and if the dose had been tolerated. Patients also underwent 24-hour ambulatory BP monitoring (ABPM) at the end of the placebo run-in, after the first and last doses of active treatment, and 48 hours after discontinuation of treatment to determine the duration of drug action and to mimic the effect of 2 missed doses. Eighty patients were recruited for the study (26 men, 54 women; mean [SD] age, 60.5 [11.6] years) (40 patients per group). Thirty-seven patients in each group completed the active treatment phase. Baseline trough BPs were similar: 167.7 (15.0)/94.6 (9.7) mm Hg in the amlodipine group and 168.6 (11.9)/93.4 (9.5) mm Hg in the enalapril group. After 14 weeks of treatment, amlodipine (mean [SD] final dose, 6.3 [2.3] mg) produced greater reductions than enalapril (mean [SD] final dose, 13.3 [6.6] mg) in trough BP (-20.8 [13.2]/-9.2 [9.0] vs -5.5 [14.9]/-3.2 [10.6] mm Hg, respectively; P &lt; or = 0.01). Most of the effect of amlodipine persisted for 72 hours after the last dose (-18.9 [14.6]/-11.1 [11.7] mm Hg), but enalapril had no significant antihypertensive effect at 72 hours (-1.3 [12.3]/-1.8 [9.1] mm Hg). Similar observations were found with ABPM recordings. Cough was reported in 5 patients (12.5%) and 13 patients (32.5%) in the amlodipine and enalapril groups, respectively, but was thought to be treatment related in only 6 patients (15.0%), all in the enalapril group. One of the patients in the enalapril group withdrew from the study because of cough, and 1 patient in the amlodipine group withdrew because of ankle edema."}, {"id": "pubmed23n0392_19644", "title": "[Effect of long-term treatment with enalapril, losartan and their combination on the quality of life of patients with congestive heart failure].", "score": 0.00909090909090909, "content": "To study the effect a combination of enalapril and losartan on life quality in patients with congestive heart failure (HF). One hundred and eighty six patients with NYHA functional classes II to IV HF were examined. The study inclusion criteria were as follows: a left ventricular (LV) end-diastolic volume of &gt; 160 ml, a LV ejection fraction of &lt; 35%, sinus rhythm, a cardiothoracic index of &gt; 0.55, no history data on prior treatment with an angiotensin-converting enzyme inhibitor (ACEI) and/or an AT1-antagonist, a patient's written free-will consent to participate in the study. The exclusion criteria were as follows: pacemaker migration, an artificial pacemaker, high-degree block, atrial fibrillation, cerebral circulatory disorders. All the patients were divided into 4 groups and received basic therapy with cardicet, 60-120 mg/day, aspirin, 250 mg/day, furosemide, 80-440 mg/week, and digoxin, 0.25-0.5 mg/day. Group 1 comprised 60 patients who refused therapy with ACEI and/or AT1-antagonist despite that they had indications for their use and they had been convinced many times. In Group 2 (n = 82) enalapril, an ACEI, was added to the basic therapy. Its initial dose of 2.5 mg/day was given once and slowly incremented to the therapeutical one (10-20 mg/day). Group 3 patients (n = 56) on the basic therapy were additionally treated with the AT1-antagonist losartan in a daily dose of 25-50 mg. They were started on 12.5 mg a day. In group 4 the basic therapy was added by a combination of enalapril and losartan in the same doses. The follow-up was 48 weeks. The efficiency of the treatments was controlled by the personal questionnaires SF-36, Life with Heart Failure, by evaluating the magnitude of clinical HF manifestations and by estimating the total life quality inxed. The data were analyzed by assuming that all the patients received the treatments. As compared with the conventional therapy and the use of each drug alone, a combination of the ACEI enalapril and the AT1-antagonist losartan promotes a more significant increase in the satisfaction of the patients with their vital activity, in the critical rate of their self-assessment of the \"internal picture\" of disease, and leads to a greater improvement of the quality of their life as a whole. The ICAE-AT1-antagonist combination exerts a positive impact on life quality in patients with heart failure."}, {"id": "wiki20220301en301_12567", "title": "Gantacurium chloride", "score": 0.009009009009009009, "content": "Adverse effects Histamine release—hypotension, reflex tachycardia and cutaneous flushing Gantacurium chloride is not associated with histamine release when administered as a rapid bolus (<5 seconds administration time) at doses up to and including 0.45 mg/kg (≤2.5xED95) according to one small study in healthy human volunteers. At 0.54 mg/kg (just under 3xED95 dose), one of four volunteers experienced histamine release with associated hypotension (30% maximum decrease in blood pressure and 13% maximum increase in heart rate) but no cutaneous flushing. At the highest administered dose of 0.72 mg/kg, three of four volunteers experienced histamine release with associated hypotension (17% to 34% maximum decrease in blood pressure and 16% to 25% increase in heart rate) and cutaneous flushing. These effects were transient and lasted no more than two minutes and did not require any adjunctive treatment to address the changes in blood pressure or heart rate."}, {"id": "pubmed23n0082_11556", "title": "Antihypertensive monotherapy with nitrendipine in general practice.", "score": 0.009009009009009009, "content": "Efficacy and feasibility of antihypertensive monotherapy with the calcium antagonist nitrendipine were investigated in a 6-week open trial in 768 patients with mild to moderate essential hypertension from 191 practicing internists and general practitioners. Previous antihypertensive therapy (n = 501) was withheld for 1 week and therapy then started with nitrendipine 20 mg q.d. If diastolic blood pressure before tablet intake in the morning stayed above 90 mm Hg or fell less than 10 mm Hg, the dose could be doubled to the maximum dose of 20 mg b.i.d. Alternatively, if blood pressure control was good, the dose could be halved to 10 mg q.d. One hundred thirty-four patients discontinued therapy prematurely because of unwanted effects mostly characteristic with dihydropyridines (headaches, flushes, and ankle edema) and mostly within the first 3 weeks. In 72% of the remaining 634 patients, the goal blood pressure was achieved by nitrendipine monotherapy (10 mg q.d. in 8%, 20 mg q.d. in 87%, and 20 mg b.i.d. in 5%) and diastolic blood pressure was between 90 and 95 mm Hg in another 3%. Reductions of blood pressure did not result in changes of heart rate or weight. Nitrendipine was effective in patients of all age groups but patients older than 65 years showed a significantly greater fall of systolic and mean arterial pressure than middle aged or young patients. Nitrendipine's efficacy under conditions of general practice and the high proportion of patients responding to once daily administration appear well suited for first-line therapy of uncomplicated hypertension. The incidence of side effects might have been smaller if therapy had started with a smaller dose."}, {"id": "wiki20220301en230_27348", "title": "Elotuzumab", "score": 0.008928571428571428, "content": "Dosage and administration In combination with lenalidomide and dexamethasone The package insert advises that intravenous administration with 10 mg/kg every week for the first 2 cycles (each cycle is 28 days) and every 2 weeks thereafter, with the appropriate doses of lenalidomide and low dose dexamethasone is acceptable for treatment. For additional information on dosing dexamethasone and/or lenalidomide, refer to the package inserts. In combination with pomalidomide and dexamethasone Elotuzumab is recommended through intravenous administration at 10 mg/kg each week for the first 2 cycles (each cycle is 28 days). At the start of cycle 3, administer 20 mg/kg every 4 weeks, while administering the recommended dose of pomalidomide and low dose dexamethasone. For additional information on dosing dexamethasone and/or dexamethasone, refer to the package inserts."}, {"id": "pubmed23n0271_15347", "title": "Continuous infusion of furosemide in the treatment of patients with congestive heart failure and diuretic resistance.", "score": 0.008928571428571428, "content": "To assess the value of treatment with continuous intravenous infusion of furosemide (F) in patients with refractory congestive heart failure. Open uncontrolled dose-response study. Patients with congestive heart failure (those with New York Heart Association (NYHA) classes III and IV with an assessed amount of oedema of more than 5 kg and diuretic resistance were included [n = 10]). Diuretic resistance was defined as: failure to lose weight and/or inappropriate urinary sodium excretion (50 mmol 24 h-1) despite bed rest for a period of 2-3 days, salt and water restriction, orally and intravenously administered furosemide in a dose of 250 mg day-1, digoxin, and when possible an ACE inhibitor. Included patients were treated with continuous F infusion at a delivery rate of 20 mg-1 over 24 h. The infusion rate was gradually heightened up to a maximum dose of 160 mg h-1. Daily physical examination, history of side-effects, determination of serum electrolytes and 24-h electrolyte excretion during treatment with furosemide. Weight loss (mean +/- SD; 12.5 +/- 5 kg) and relief of symptoms was achieved in all patients. Mean (+/- SD) 24-h sodium output rose from 19 +/- 16 mmol 24 h-1 (n = 10) on oral therapy with 250 mg F to 137 +/- 85 mmol 24 h-1 (n = 8) during 80 mg h-1 and to 268 +/- 124 mmol 24 h-1 (n = 3) on the maximal dose of 160 mg h-1. Continuous infusion of F under careful monitoring of the patient is a safe, controllable and efficient treatment in patients with severe congestive heart failure and diuretic resistance."}, {"id": "pubmed23n0417_2417", "title": "Adjunctive sympathoplegic therapy to ACE inhibition in Blacks with congestive heart failure: a comparison of alpha-1 with beta-1 blockade on exercise tolerance and cardiac sympathovagal reflex activity.", "score": 0.008849557522123894, "content": "Congestive heart failure (CHF) is characterized by an initial compensatory, but subsequently deleterious, activation of both the renin-angiotensin (RAS) and the sympathetic nervous system (SNS). Incomplete suppression of the SNS may contribute to the residual mortality during optimal ACE inhibitor therapy in CHF. Carvedilol, a mixed alpha and beta-blocker with antioxidant properties, and other pure beta-adrenoceptor blockers reduce morbidity and mortality in Caucasians with CHF. However, beta-blocker monotherapy is of poor efficacy in Blacks with essential hypertension or in the treatment of glaucoma. The efficacy of beta-blockers in the treatment of African Americans with congestive heart failure is a controversial issue with conflicting findings. The aims of the present study were to examine and compare the cardiovascular, autonomic, and clinical effects of additional alpha-1, or beta-1 blockade in ACE-inhibitor treated Black patients with moderate to severe CHF. Twenty-eight Nigerian patients with chronic CHF stabilized on digoxin and diuretics, were randomized to 3 groups of similar demographics according to a single blind, parallel group design. The patients were aged 53 +/- 6 years, and comprised 14 men and 14 women, with a mean cardiothoracic ratio of 0.66 +/- 0.03, and ejection fraction of 0.38 +/- 0.10, 60% hypertensive etiology. Group 1 patients received 5 mg enalapril alone, group 2 received 5 mg enalapril + 1 mg prazosin, and group 3 received 5 mg enalapril + 50 mg atenolol. All medication was taken daily for 4 weeks. Blood pressure, heart rate, pressure rate product, 6-minute walk test, NYHA class, and cardiac autonomic reflexes were measured at baseline and again at 2 and 4 weeks of treatment. Two-way repeated measures ANOVA, and a one-way ANOVA were used in data analysis. The 3 treatments caused significant (P&lt;.001 ANOVA) and similar improvements for the NYHA class (-1.0 to -1.6), and increased the 6-minute distance covered (+130 m to +205 m). Although no treatment differences were observed, a trend suggesting a greater improvement with enalapril + atenolol became apparent. By the fourth week, the sympathoplegic treatments, enalapril + atenolol, and enalapril + prazosin, caused significant reductions in the pressure rate product (-3726 +/- 1885 mm Hg x beats/min; -3498 +/- 396 mm Hg beats/min, respectively), (compared to enalapril alone (-1349 +/- 894 mm Hg x beats/min) (P&lt;.001 ANOVA). During the Valsalva maneuver, the phase IV bradycardia were significantly greater after treatment with enalapril + atenolol (944 +/- 66 msec) or with enalapril + prazosin (825 +/- 48 msec), compared to enalapril alone (760 +/- 45 msec) (P&lt;.001 ANOVA). The phase II Valsalva tachycardia were similar between treatments. The respiratory sinus arrhythmia ratio increased significantly (P&lt;.005 ANOVA) and equally on all treatments. However, the pressor and chronotropic responses to forearm isometric handgrip increased significantly on the enalapril + prazosin combination (P&lt;.02), compared to the other treatments. Our findings demonstrated not only the safety of providing additional therapy with alpha-1 or beta-1 receptor blockade concurrent with ACE inhibition in Blacks with CHF, but also the resultant improvement in exercise tolerance and NYHA class. Compared to using ACE inhibition alone, the combined therapies caused a marked reduction in the pressure rate product, an index of myocardial oxygen consumption, and a greater enhancement of cardiac parasympathetic activity. Selective beta-1 blockade caused a greater enhancement of central baroreceptor vagal activity compared to alpha-1 blockade. Conversely, the pressor and chronotropic abnormalities during forearm isometric handgrip in CHF, were normalized by alpha-1, but not beta-1, blockade. Thus, the combined reflex cardiac vagal augmentation following selective beta-1 blockade, and the hemodynamic effects of alpha-1 antagonism with concurrent ACE inhibition, may be of major therapeutic and prognostic benefit in Blacks with non-ischemic (hypertensive) CHF stabilized on digoxin and diuretics."}, {"id": "pubmed23n0074_6371", "title": "Hypertension in the elderly: a study of a combination of atenolol, hydrochlorothiazide and amiloride hydrochloride.", "score": 0.008849557522123894, "content": "The anti-hypertensive effects of atenolol (Tenormin) 50 mg, a potassium-sparing diuretic (half-strength Moduretic) comprising hydrochlorothiazide 25 mg plus 2.5 mg amiloride hydrochloride, and the 'free' combination of atenolol and diuretic were compared in elderly hypertensive patients aged 60-79 years. After a four-week run-in period on placebo, patients were randomly assigned, in a double-blind manner, to atenolol or diuretic treatment, each for four weeks. Thereafter patients were given the 'free' combination for a further four weeks and this treatment was continued for six months. Blood pressure and heart rate were measured after the patient had rested for five minutes supine and after two minutes standing. These blood pressure measurements were made at least 24 hours after the preceding dose using a Random Zero sphygmomanometer. Results from 26 of the 27 patients entered into the study showed an advantage for combination therapy combined with either atenolol or diuretic treatment alone. No significant difference was found between treatments in the frequency of supraventricular and ventricular ectopic beats occurring in six patients who underwent 24-hour ambulatory ECG monitoring. However, ectopic activity was reduced in some patients during beta-blocker treatment. Few adverse effects occurred with any treatment. Three patients withdrew during the placebo period and three withdrew while taking active treatment. This study has shown that the combination of atenolol, hydrochlorothiazide and amiloride hydrochloride is an effective, safe, well-tolerated antihypertensive drug regimen when used once daily in elderly hypertensive patients."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 173]], "word_ranges": [[0, 29]], "text": "In healthy adults there may be palpable inguinal nodes up to 2 centimeters that can be considered normal. A complementary study of these normal lymph nodes is not warranted."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "In healthy adults there may be palpable inguinal nodes up to 2 centimeters that can be considered normal. A complementary study of these normal lymph nodes is not warranted.", "full_answer_no_ref": "In healthy adults there may be palpable inguinal nodes up to 2 centimeters that can be considered normal. A complementary study of these normal lymph nodes is not warranted.", "full_question": "A 24-year-old woman consults after noticing inguinal lymphadenopathy. The interrogation does not reveal the presence of any local discomfort or data suggestive of sexually transmitted infection. The examination revealed two lymphadenopathies, one in each groin, 1 cm in diameter, soft, mobile, non-painful. There are no skin lesions on the lower limbs, anus or perineum. Which test do you consider essential?", "id": 301, "lang": "en", "options": {"1": "A lues serology since it is most likely a Treponema pallidum infection.", "2": "A gynecological examination to rule out ovarian cancer.", "3": "By the clinical characteristics it seems to be normal lymph nodes and complementary explorations should not be done.", "4": "A Paul-Bunnell test should be performed in order to rule out infectious mononucleosis.", "5": NaN}, "question_id_specific": 232, "type": "INFECTIOUS DISEASES", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en393_31405", "title": "Inguinal lymphadenopathy", "score": 0.018304351443547716, "content": "Inguinal lymphadenopathy causes swollen lymph nodes in the groin area. It can be a symptom of infective or neoplastic processes. Infective aetiologies include Tuberculosis, HIV, non-specific or reactive lymphadenopathy to recent lower limb infection or groin infections. Another notable infectious cause is Lymphogranuloma venereum, which is a sexually transmitted infection of the lymphatic system. Neoplastic aetiologies include lymphoma, leukaemia and metastatic disease from primary tumours in the lower limb, external genitalia or perianal region and melanoma. References Further reading Inflammations Diseases of veins, lymphatic vessels and lymph nodes"}, {"id": "wiki20220301en154_45003", "title": "Genital ulcer", "score": 0.017740429505135387, "content": "Overview A genital ulcer may be located on the vulva, penis, perianal region, or anus. Globally, the incidence of genital ulcers is estimated to be approximately 20 million cases annually. The most likely cause of a genital ulcer varies depending on the characteristics of a population and location. The most common cause of genital ulcers in the United States is herpes simplex infections, with syphilis the second most common cause, and chancroid the third. These common causes of genital ulcer disease (HSV-1, HSV-2 and treponema pallidum) can all be efficiently transmitted through oral sex. Important signs associated with genital ulcers that may assist in the diagnosis of the cause of the genital ulcer may include the presence of tender or non-tender enlarged lymph nodes in the groin area, a painful or non-painful genital ulcer, or the presence of vesicular lesions, which are small, painful, elevated blisters."}, {"id": "pubmed23n0999_2351", "title": "[A chancre instead of cancer].", "score": 0.016584429824561403, "content": "Syphilis (lues) is a sexually transmitted infection caused by the spirochete Treponema pallidum. In adolescents, the diagnosis of primary syphilis can be made promptly by taking a sexual medical history and inspecting the glans penis. A 17-year-old male was referred to the paediatric oncology centre for additional diagnostics due to inguinal lymphadenopathy, with a strong suspicion of a malignant lymphoma. None of the physicians took a sexual medical history or investigated the glans penis, as a result of which essential information was lacking. The combination of inguinal lymphadenopathy and the ultrasound findings for the inguinal region made the physicians only consider a malignancy. However, it actually concerned a reactive lymphadenopathy associated with primary syphilis. This case demonstrates that a full medical history and thorough physical examination can prevent the need for costly and invasive diagnostics."}, {"id": "wiki20220301en042_58337", "title": "Inguinal lymph nodes", "score": 0.016453503863575806, "content": "Clinical significance The presence of swollen inguinal lymph nodes is an important clinical sign because lymphadenopathy (swelling) may indicate an infection, or spread as a metastasis from cancers, such as anal cancer and vulvar cancer. Inguinal lymph nodes may normally be up to 2 cm. The cut-off value for normal sized inguinal nodes is up to 10 mm. Additional images References Lymphatic organ anatomy"}, {"id": "article-23529_8", "title": "Anatomy, Abdomen and Pelvis: Inguinal Lymph Node -- Clinical Significance -- Lymphadenopathy", "score": 0.01250683433570257, "content": "Swollen lymph nodes usually indicate infection from bacteria or viruses. Swollen inguinal lymph nodes could indicate an infection of areas of the lower body. One of the more concerning causes of inguinal lymphadenopathy is sexually transmitted infections. Sexually transmitted infections that commonly presents with inguinal lymphadenopathy are lymphogranuloma venereum, secondary syphilis, and chancroid caused by Chlamydia trachomatis (L1-L3), Treponema pallidum , and Haemophilus ducreyi , respectively. Lymphogranuloma venereum"}, {"id": "InternalMed_Harrison_10857", "title": "InternalMed_Harrison", "score": 0.012456441462526655, "content": "Diagnosis Although most genital ulcerations cannot be diagnosed confidently on clinical grounds alone, clinical findings (Table 163-7) FIGURE 163-5 Chancroid: multiple, painful, punched-out ulcers with undermined borders on the labia occurring after autoinoculation. Sexually Transmitted Infections: Overview and Clinical Approach FIGURE 163-6 Genital herpes. A relatively mild, superficial ulcer is typically seen in episodic outbreaks. (Courtesy of Michael Remington, University of Washington Virology Research Clinic.) FIGURE 163-7 Lymphogranuloma venereum (LGV): striking ten-der lymphadenopathy occurring at the femoral and inguinal lymph nodes, separated by a groove made by Poupart’s ligament. This “sign-of-the-groove” is not considered specific for LGV; for example, lym-phomas may present with this sign. InITIAL MAnAgEMEnT of gEnITAL oR PERIAnAL uLCER"}, {"id": "wiki20220301en012_95894", "title": "Lymphogranuloma venereum", "score": 0.01211414504583293, "content": "The secondary stage most often occurs 10–30 days later, but can present up to six months later. The infection spreads to the lymph nodes through lymphatic drainage pathways. The most frequent presenting clinical manifestation of LGV among males whose primary exposure was genital is unilateral (in two-thirds of cases) lymphadenitis and lymphangitis, often with tender inguinal and/or femoral lymphadenopathy because of the drainage pathway for their likely infected areas. Lymphangitis of the dorsal penis may also occur and resembles a string or cord. If the route was anal sex, the infected person may experience lymphadenitis and lymphangitis noted above. They may instead develop proctitis, inflammation limited to the rectum (the distal 10–12 cm) that may be associated with anorectal pain, tenesmus, and rectal discharge, or proctocolitis, inflammation of the colonic mucosa extending to 12 cm above the anus and associated with symptoms of proctitis plus diarrhea or abdominal cramps."}, {"id": "Gynecology_Novak_2917", "title": "Gynecology_Novak", "score": 0.01201963416680651, "content": "Several clinical presentations are highly suggestive of specific diagnoses: 1. A painless and minimally tender ulcer, not accompanied by inguinal lymphadenopathy, is likely to be syphilis, especially if the ulcer is indurated. A nontreponemal rapid plasma reagin (RPR) test, or venereal disease research laboratory (VDRL) test, and a confirmatory treponemal test—fluorescent treponemal antibody absorption (FTA ABS) or microhemagglutinin–T. pallidum (MHA TP)—should be used to diagnose syphilis presumptively. Some laboratories screen samples with treponemal enzyme immunoassay (EIA) tests, the results of which should be confirmed with nontreponemal tests. The results of nontreponemal tests usually correlate with disease activity and should be reported quantitatively. 2."}, {"id": "article-32329_33", "title": "Anatomy, Abdomen and Pelvis: Female External Genitalia -- Clinical Significance -- Sexually Transmitted Infections", "score": 0.011890853389412467, "content": "Treponema pallidum : Syphilis infections result from Treponema pallidum . This infection usually manifests as a painless chancre in the primary stage. If the disease is left untreated, it will progress to the secondary stage. In the secondary stage, it manifests as fever, widespread maculopapular skin rashes involving the palms and soles,  widespread lymphadenopathy (epitrochlear node is pathognomic), and genital lesions similar to genital warts (condylomata lata- has a rounder surface when compared with condylomata acuminata). If there is still no treatment during the secondary stage, the infection will progress into the tertiary stage. The tertiary stage causes necrotic lesions called Gummas, neurological symptoms such as tabes dorsalis, Argyll Robertson pupils, and general paresis, cardiac symptoms such as aortitis. The treatment of syphilis is with the use of penicillin."}, {"id": "InternalMed_Harrison_10861", "title": "InternalMed_Harrison", "score": 0.011462641446056306, "content": "Source: From RM Ballard, in KK Holmes et al (eds): Sexually Transmitted Diseases, 4th ed. New York, McGraw-Hill, 2008. HSV-1 from HSV-2 has prognostic implications, because the latter causes more frequent genital recurrences. Painless, nontender, indurated ulcers with firm, nontender inguinal adenopathy suggest primary syphilis. If results of dark-field examination and a rapid serologic test for syphilis are initially negative, presumptive therapy should be provided on the basis of the individual’s risk. For example, with increasing rates of syphilis among MSM in the United States, most experts would not withhold therapy for this infection pending watchful waiting and/or subsequent detection of seroconversion. Repeated serologic testing for syphilis 1 or 2 weeks after treatment of seronegative primary syphilis usually demonstrates seroconversion."}, {"id": "wiki20220301en012_95897", "title": "Lymphogranuloma venereum", "score": 0.010557717250324256, "content": "Diagnosis The diagnosis usually is made serologically (through complement fixation) and by exclusion of other causes of inguinal lymphadenopathy or genital ulcers. Serologic testing has a sensitivity of 80% after two weeks. Serologic testing may not be specific for serotype (has some cross reactivity with other chlamydia species) and can suggest LGV from other forms because of their difference in dilution, 1:64 more likely to be LGV and lower than 1:16 is likely to be other chlamydia forms (emedicine). For identification of serotypes, culture is often used. Culture is difficult. Requiring a special medium, cycloheximide-treated McCoy or HeLa cells, and yields are still only 30-50%. DFA, or direct fluorescent antibody test, PCR of likely infected areas and pus, are also sometimes used. DFA test for the L-type serovar of C. trachomatis is the most sensitive and specific test, but is not readily available."}, {"id": "InternalMed_Harrison_10864", "title": "InternalMed_Harrison", "score": 0.010355944179473591, "content": "Demonstration of H. ducreyi by culture (or by PCR, where available) is most useful when ulcers are painful and purulent, especially if inguinal lymphadenopathy with fluctuance or overlying erythema is noted; if chancroid is prevalent in the community; or if the patient has recently had a sexual exposure elsewhere in a chancroid-endemic area (e.g., a developing country). Enlarged, fluctuant lymph nodes should be aspirated for culture or PCR to detect H. ducreyi as well as for Gram’s staining and culture to rule out the presence of other pyogenic bacteria."}, {"id": "article-23529_10", "title": "Anatomy, Abdomen and Pelvis: Inguinal Lymph Node -- Clinical Significance -- Lymphadenopathy", "score": 0.01031386147417089, "content": "Syphilis is a bacterial infection caused by the spirochete Treponema pallidum. Clinical presentation of secondary syphilis can present with diffuse lymphadenopathy along with fever, skin rashes, and condylomata lata. [7] Chancroid"}, {"id": "InternalMed_Harrison_4562", "title": "InternalMed_Harrison", "score": 0.010108189212666825, "content": "Lymphadenopathy may be an incidental finding in patients being examined for various reasons, or it may be a presenting sign or symptom of the patient’s illness. The physician must eventually decide whether the lymphadenopathy is a normal finding or one that requires further study, up to and including biopsy. Soft, flat, submandibular nodes (<1 cm) are often palpable in healthy children and young adults; healthy adults may have palpable inguinal nodes of up to 2 cm, which are considered normal. Further evaluation of these normal nodes is not warranted. In contrast, if the physician believes the node(s) to be abnormal, then pursuit of a more precise diagnosis is needed. APPROACH TO THE PATIENT:"}, {"id": "InternalMed_Harrison_9486", "title": "InternalMed_Harrison", "score": 0.010054301713897845, "content": "Approach to the Patient with an Infectious Disease regions (e.g., popliteal, inguinal, epitrochlear, axillary, multiple cervical regions), with notation of the location, size (normal, <1 cm), presence or absence of tenderness, and consistency (soft, firm, or shotty) and of whether the nodes are matted (i.e., connected and moving together). Of note, palpable epitrochlear nodes are always pathologic. Of patients presenting with lymphadenopathy, 75% have localized findings, and the remaining 25% have generalized lymphadenopathy (i.e., that involving more than one anatomic region). Localized lymphadenopathy in the head and neck region is found in 55% of patients, inguinal lymphadenopathy in 14%, and axillary lymphadenopathy in 5%. Determining whether the patient has generalized versus localized lymphadenopathy can help narrow the differential diagnosis, as various infections present differently."}, {"id": "article-28956_50", "title": "Sexually Transmitted Infections -- History and Physical -- Granuloma Inguinale", "score": 0.009761300970092179, "content": "Females and males: Signs and symptoms: Patients will present with highly vascularized lesions over the genitals and perineum that tend to be painless. [37] [38] It can cause severe scarring. Physical Exam: Typical findings include ulcer-like lesions that are beefy red, consistent with high vascularization that bleeds easily with manipulation. Subcutaneous granulomas may be present, but lymphadenopathy is uncommon. The lesions tend to be relatively large and irregular. It is often found to be associated with secondary infections. Four main lesions can be seen on examination: 1. Ulcerovegetative: large painless ulcer on the patient's physical exam. 2. Nodular: soft and erythematous that tend to ulcerate throughout the infectious process. 3. Cicatricial: dry ulcerations that tend to transition into plaques. 4. Hypertrophic: lesions are thick and painless. [37] [39]"}, {"id": "InternalMed_Harrison_4570", "title": "InternalMed_Harrison", "score": 0.009737297467358817, "content": "node is an enlarged left supraclavicular node infiltrated with metastatic cancer from a gastrointestinal primary. Metastases to supraclavicular nodes also occur from lung, breast, testis, or ovarian cancers. Tuberculosis, sarcoidosis, and toxoplasmosis are nonneoplastic causes of supraclavicular adenopathy. Axillary adenopathy is usually due to injuries or localized infections of the ipsilateral upper extremity. Malignant causes include melanoma or lymphoma and, in women, breast cancer. Inguinal lymphadenopathy is usually secondary to infections or trauma of the lower extremities and may accompany sexually transmitted diseases such as lymphogranuloma venereum, primary syphilis, genital herpes, or chancroid. These nodes may also be involved by lymphomas and metastatic cancer from primary lesions of the rectum, genitalia, or lower extremities (melanoma)."}, {"id": "wiki20220301en013_140385", "title": "Yaws", "score": 0.009708737864077669, "content": "Serological tests cannot distinguish yaws from the closely related syphilis; no test distinguishing yaws from syphilis is widely available. The two genomes differ by about 0.2%. PCR and DNA sequencing can distinguish the two. There are also no common blood tests which distinguish among the four treponematoses: syphilis (Treponema pallidum pallidum), yaws (Treponema pallidum pertenue), bejel (Treponema pallidum endemicum), and pinta (Treponema carateum). Haemophilus ducreyi infections can cause skin conditions that mimic primary yaws. People infected with Haemophilus ducreyi lesions may or may not also have latent yaws, and thus may or may not test positive on serological tests. This was discovered in the mid 2010s. It seems that a recently diverged strain of Haemophilus ducreyi has evolved from being a sexually transmitted infection to being a skin ulcer pathogen that looks like yaws. Yaws has been reported in nonendemic countries."}, {"id": "article-28956_53", "title": "Sexually Transmitted Infections -- History and Physical -- Lymphogranuloma venereum (LGV)", "score": 0.009708737864077669, "content": "Females and males: Signs and symptoms: Patients will present with painful lymphadenopathy localized to the inguinal area. Patients may note the initial presentation of a pustule that gradually progresses to large painful ulceration. [41] Men tend to present with early or acute stages, while women typically present much later. [19] Physical Exam: Lymphogranuloma venereum presents with two stages: Primary phase is a small painless papule/pustule that will ulcerate and can be visualized throughout the affected genital area. During the secondary phase, patients present with unilateral lymphadenopathy that is fluctuant with palpation or may be suppurative in a presentation known as buboes. [38] Buboes tend to rupture in the acute phase and progress to a thickened mass. [42]"}, {"id": "pubmed23n0620_7089", "title": "Glandular fever and pulmonary artery thrombosis in a paraplegic patient, who had undergone splenectomy for splenic trauma sustained along with spinal cord injury: misdiagnosed initially as urine infection and later as lymphoma when CT scan revealed enlarged lymph nodes: a case report.", "score": 0.009615384615384616, "content": "A 36-year-old male sustained fracture of first lumbar vertebra, splenic tear and paraplegia in a motorcycle accident in 2001; splenectomy was performed. In 2008, he presented with temperature and feeling rough. With a diagnosis of urine infection, he was prescribed ciprofloxacin, followed by trimethoprim, amoxicillin, and gentamicin, as temperature did not subside. White cell count was 21.2 x 109/L; lymphocytes were 13.05 x 109/L (1.00 - 4.00). Therefore, computerised tomography (CT) of chest and abdomen was performed. Thrombus was present in pulmonary arteries bilaterally involving the lobar and segmental branches. Enlarged lymph nodes were seen in axillae, chest, abdomen and inguinal regions. Radiological diagnosis was lymphoma. Cell marker showed an excess of large granular lymphocytes and activated lymphocytes. The Glandular Fever Slide Test was positive. Subsequently, Paul Bunnell test was also positive. Epstein Barr virus serology was consistent with recent Epstein Barr virus infection. Antibiotic was omitted; enoxaparin was prescribed for pulmonary artery thrombosis. Learning points from this case: (1) Although routine administration of antibiotic to a spinal cord injury patient with pyrexia may be acceptable in outpatient setting, other possibilities such as infection by multi-drug resistant organism, viral infection, venous or, arterial thrombosis should be considered if a patient does not respond promptly to antibacterial therapy. (2) When full blood count showed lymphocytosis (comprising &gt; 50% of white blood cells) with atypical morphology, lymphocyte surface markers, Paul Bunnell test, and Epstein Barr virus serology should be performed. These tests would have led to a diagnosis of infectious mononucleosis, and abdominal imaging studies could have been avoided. (3) Lymphoid hyperplasia is the hallmark of infectious mononucleosis; therefore, we should have suspected glandular fever rather than lymphoma when CT scan revealed enlarged lymph nodes in abdomen, mediastinum, axillae and inguinal regions in this patient, who had lymphocytosis with atypical morphology. (4) A soft tissue mass, situated inferior to left hemidiaphragm in this asplenic patient, was misinterpreted as lymph nodes; review of CT led to the correct diagnosis of splenunculus. (5) Acute infection with Epstein Barr virus may lead to transient induction of anti-phospholipid antibodies, which can cause vascular thrombosis. (6) This case illustrates the value of reviewing test results and discussion with senior doctors, as these measures help to recognize medical errors and improve patient care."}, {"id": "wiki20220301en067_61541", "title": "Sexual health clinic", "score": 0.009615384615384616, "content": "In a private room or space, the patient will partially undress. The clinician may inspect the patient's: Throat and lymph nodes of the neck for inflammation Pubic hair for lice Lymph nodes of the groin for swelling Genitals, anus, and surrounding areas for sores and warts The clinician may swab the patient's: Throat to test for gonorrhea and possibly chlamydia Cheek, inside, to diagnose HIV Sores of the genitals, anus, and surrounding areas to test for herpes Urethra to test for gonorrhea and possibly chlamydia Vagina to test for chlamydia and possibly gonorrhea Cervix to test for cervical intraepithelial neoplasia (a Pap test) Rectum to test for gonorrhea and possibly chlamydia The clinician may take small blood samples by pricking a finger or from a vein to test for HIV, syphilis, and possibly herpes and hepatitis C."}, {"id": "pubmed23n0954_11063", "title": "Unusual Manifestations of Secondary Syphilis: Case Presentations.", "score": 0.009523809523809525, "content": "Dear Editor, Syphilis is an infection caused by Treponema pallidum. Without treatment, it goes through the following stages: primary, secondary, latent, and tertiary (1). The clinical picture of secondary syphilis is very variable (2,3). We present two rare cases of secondary syphilis, one with nodular lesions initially considered to be lymphoma and second with periostitis, which was initially interpreted as an osteoma. To date, only 15 cases with nodular lesions and 10 cases with periostitis in secondary syphilis have been reported in the literature. The first patient was a 59 year old man who presented in a private practice with nodular lesions on the face and axillary and inguinal folds (Figure 1, a, b). The initial diagnostic consideration was lymphoma. A biopsy specimen was taken, and the histopathological features revealed epidermal hyperplasia with papillomatosis, minimal spongiosis with many neutrophils and with a marked inflammatory infiltrate in dermis, consisting of lymphocytes, plasma cells, and neutrophils; the diagnosis of interfaced dermatitis was established (Figure 1, d, e). After one month, the patient presented to our clinic with numerous nodular lesions, some of them painful, located on the trunk and intertriginous folds, including the intergluteal cleft - the lesions in this area being suggestive of condylomata lata (Figure 1, c). The diagnosis of secondary syphilis was taken into consideration, and screening serum tests were performed and found reactive: a Venereal Diseases Research Laboratory (VDRL) titer of 1:64 and Treponema pallidum Hemaglutination Assay (TPHA) titer of 1:80. Hepatitis and anti-human immunodeficiency virus (HIV) antibodies serology was negative. The biopsy was repeated and showed the same histopathological changes. In addition, Warthin-Starry staining was performed, revealing the presence of some spiral micro-organisms in the dermis corresponding to Treponema pallidum (Figure 1, f). A diagnosis of secondary syphilis was established, and the patient was treated with benzathine penicillin G 2.4 million units by intramuscular injection once a week for 2 consecutive weeks. The skin lesions regressed within 1 month, and serological tests showed a VDRL titer of 1:8 3 months after treatment. The second patient was a homosexual male, 35 years old, diagnosed with HIV infection, stage B2. He presented with bone pain in the calves and forearms, with insidious onset. He also presented with an associated erythematous maculo-papular rash on the trunk and limbs and generalized lymphadenopathy (Figure 2, a, b). The tibial crest and radius were sensitive to palpation. A right leg radiography was performed, raising suspicion of osteoid osteoma. The CT scan excluded the diagnosis of osteoma; taking into account the epidemiological context, the diagnosis of syphilis was suspected. The diagnosis was confirmed by leg ultrasound examination (2D US) which showed thickening of the compact tibial bone associated with subperiosteal destructive and proliferative changes (Figure 2, c, d) and by serology for syphilis: the VDRL titer was 1:32 and the TPHA titer was 1:80. The patient was treated with benzathine penicillin 2.4 million units, once a week, for 2 consecutive weeks, with clinical improvement. Syphilis continues to be a serious public health problem worldwide, even if it is a controllable disease due to diagnostic tests and effective and accessible treatment. According to the World Health Organization in 2008, the estimated number of new cases of sexually transmitted diseases in adults with syphilis is 10.6 million cases (4). The cases presented in this paper were characterized by unusual manifestations, requiring good collaboration between the dermatologist and other specialties. In the first case, the diagnosis of secondary syphilis was confirmed by positive serological, clinical, and histopathological findings. The main differential diagnosis of nodular syphilis includes lymphoma, sarcoidosis, Kaposi's sarcoma, atypical mycobacteriosis, deep fungal infections, leprosy, tuberculosis, leishmaniasis, and lymphomatoid papulosis (5). Another important differential diagnosis is between secondary and tertiary syphilis, especially when ulcerating nodules are present. Tertiary syphilis is characterized by unilateral, deep ulcerating nodules with necrotizing granulomas (6). Bone involvement during syphilis is mainly represented by polyarthritis, synovitis, osteitis, and periostitis (7,8). Syphilitic periostitis is characterized by localized or diffuse pain, particularly during the night, which is relieved by movement. The skull, the shoulder girdle, and the long bones are the most common sites of involvement (9). In conclusion, we presented two different cases of secondary syphilis that contribute to the clinical experience of rare cases presented in the literature, raising the awareness of dermatologists and other specialists about less specific clinical aspects of syphilis."}, {"id": "InternalMed_Harrison_10874", "title": "InternalMed_Harrison", "score": 0.009523809523809525, "content": "adenopathy that is sometimes mistaken for malignancy; syphilis, LGV, HSV infection, and chancroid involving the anus can produce inguinal adenopathy because anal lymphatics drain to inguinal lymph nodes."}, {"id": "wiki20220301en302_4978", "title": "Cervical lymphadenopathy", "score": 0.009433962264150943, "content": "Cervical lymphadenopathy is a sign or a symptom, not a diagnosis. The causes are varied, and may be inflammatory, degenerative, or neoplastic. In adults, healthy lymph nodes can be palpable (able to be felt), in the axilla, neck and groin. In children up to the age of 12 cervical nodes up to 1 cm in size may be palpable and this may not signify any disease. If nodes heal by resolution or scarring after being inflamed, they may remain palpable thereafter. In children, most palpable cervical lymphadenopathy is reactive or infective. In individuals over the age of 50, metastatic enlargement from cancers (most commonly squamous cell carcinomas) of the aerodigestive tract should be considered. Classification Cervical lymphadenopathy can be thought of as local where only the cervical lymph nodes are affected, or general where all the lymph nodes of the body are affected. Causes"}, {"id": "article-24577_17", "title": "Lymphogranuloma Venereum -- Differential Diagnosis", "score": 0.009433962264150943, "content": "Sexually transmitted infections are the first to consider in the differential diagnosis of LGV. The exclusion of diseases which cause genital ulceration and inguinal adenopathy including herpes simplex, syphilis, chancroid, herpes, and granuloma inguinale will help narrow the diagnosis. HIV and lymphoma can also cause generalized lymphadenopathy. Dermatological conditions and trauma which can cause genital ulcerations should also be in the differential diagnosis."}, {"id": "wiki20220301en032_60070", "title": "Lymphadenopathy", "score": 0.009345794392523364, "content": "Lymph node enlargement is recognized as a common sign of infectious, autoimmune, or malignant disease. Examples may include: Reactive: acute infection (e.g., bacterial, or viral), or chronic infections (tuberculous lymphadenitis, cat-scratch disease). The most distinctive sign of bubonic plague is extreme swelling of one or more lymph nodes that bulge out of the skin as \"buboes.\" The buboes often become necrotic and may even rupture. Infectious mononucleosis is an acute viral infection usually caused by Epstein-Barr virus and may be characterized by a marked enlargement of the cervical lymph nodes. It is also a sign of cutaneous anthrax and Human African trypanosomiasis Toxoplasmosis, a parasitic disease, gives a generalized lymphadenopathy (Piringer-Kuchinka lymphadenopathy). Plasma cell variant of Castleman's disease - associated with HHV-8 infection and HIV infection"}, {"id": "pubmed23n0510_12917", "title": "[Inguinal lymphogranuloma venereum in a man having sex with men: perhaps an example of the missing link to explain the transmission of the recently identified anorectal epidemic].", "score": 0.009345794392523364, "content": "A 38-year-old man who had sex with men, presented at the outpatient department for Sexually Transmitted Diseases in Amsterdam with a painful, red, fluctuating swelling in the left groin and general discomfort. He had been sexually active in the population of men who have sex with men, in which an anorectal lymphogranuloma venereum (LGV) epidemic has recently been discovered. Unlike other cases where there was anorectal involvement, this patient was the first case of LGV with the classical inguinal presentation although he had not visited the tropics where the inguinal form of LGV occurs as an STD. Routine investigation using PCR on material from urethra and rectum and from the urine, repeatedly failed to detect LGV. However, PCR on pus aspirated from the enlarged lymph node demonstrated Chlamydia trachomatis serovar type L2. Treatment with doxycycline 100 mg twice daily was started. This case illustrates that routine analysis from urethra and rectum and of urine may fail to detect LGV. Furthermore, this case of a patient who probably had LGV initially in the urethra may be the missing link in explaining the route of transmission of the anorectal LGV epidemic."}, {"id": "InternalMed_Harrison_12283", "title": "InternalMed_Harrison", "score": 0.009259259259259259, "content": "The presentation of chancroid does not usually include all of the typical clinical features and is sometimes atypical. Multiple ulcers can coalesce to form giant ulcers. Ulcers can appear and then resolve, with inguinal adenitis (Fig. 182-2) and suppuration following 1–3 weeks later; this clinical picture can be confused with that of lymphogranuloma venereum (Chap. 213). Multiple small ulcers can resemble folliculitis. Other differential diagnostic considerations include the various infections causing genital ulceration, such as primary syphilis, secondary syphilis (condyloma latum), genital herpes, and donovanosis. In rare cases, chancroid lesions become secondarily infected with bacteria; the result is extensive inflammation. FIGURE 182-2 Chancroid with characteristic penile ulcers and associated left inguinal adenitis (bubo)."}, {"id": "pubmed23n0805_14300", "title": "Primary syphilis of the oropharynx: an unusual location of a chancre.", "score": 0.009174311926605505, "content": "A 33-year-old man presented with a two-week history of an asymptomatic ulcer of the oropharynx and submandibular lymph nodes swelling. Laboratory examinations were normal, but serological tests revealed positivity for rapid plasma reagin, Treponema pallidum haemagglutination assay and anti-T. pallidum IgM antibodies. Since the patient denied any homosexual relationship, a biopsy of the lesion was performed, which confirmed primary syphilis. The patient received an intramuscular injection of Benzathine Penicillin G (2.4 MU) with complete resolution of the lesion. Extragenital chancres occur in at least 5% of patients with primary syphilis, and the oral mucosa is the most frequent location as a consequence of orogenital/oroanal contact with an infectious lesion. Because of their transient nature, these oral ulcerations are often underestimated by the patient or by any unsuspecting clinician. Health professionals should consider the recent sexual history of their patients and should be prepared to recognise oral and systemic manifestations of sexually transmitted infections. "}, {"id": "pubmed23n0668_207", "title": "Oropharyngeal lesions and cervical lymphadenopathy: syphilis is a differential diagnosis that is still relevant.", "score": 0.00909090909090909, "content": "Syphilis (lues), a chronic infectious disease caused by Treponema pallidum, has been increasing in incidence during the last few years. Therefore, while clinically it is often not suspected, syphilis is increasingly becoming a differential diagnosis in routine pathology. To report our experience with five cases of cervical lymphadenopathy and/or oropharyngeal lesions, clinically thought to be lymphomas, lymph node metastases or carcinoma, in which we made the mostly clinically unsuspected diagnosis of syphilis. Fine needle aspiration of enlarged cervical lymph nodes was evaluated by cytology and flow cytometry (fluorescence-activated cell sorting analysis), and biopsies were examined by using histology. In addition, all materials were also subjected to immunostaining, silver staining and molecular (PCR) testing. Fine needle aspiration cytology revealed follicular hyperplasia in two cases and granulomatous lymphadenitis in one case. In three patients, concomitant biopsy of co-existing oropharyngeal lesions revealed histological findings compatible with syphilis. T pallidum was detected in all cytological and histological samples by immunohistochemistry/immunocytochemistry and PCR. Subsequently, a diagnosis of syphilis was confirmed clinically and by serology. Syphilitic lymphadenitis is still a relevant differential diagnosis of cervical lymphadenopathy, and it is clinically often not suspected. Co-existing oropharyngeal lesions should alert the physician to this differential diagnosis; and lesions with compatible morphology should be tested with immunohistochemistry and immunocytochemistry and/or molecular analysis to confirm the diagnosis of syphilis."}, {"id": "wiki20220301en017_7708", "title": "Chancroid", "score": 0.00909090909090909, "content": "About half of infected men have only a single ulcer. Women frequently have four or more ulcers, with fewer symptoms. The ulcers are typically confined to the genital region most of the time. The initial ulcer may be mistaken as a \"hard\" chancre, the typical sore of primary syphilis, as opposed to the \"soft chancre\" of chancroid. Approximately one-third of the infected individuals will develop enlargements of the inguinal lymph nodes, the nodes located in the fold between the leg and the lower abdomen. Half of those who develop swelling of the inguinal lymph nodes will progress to a point where the nodes rupture through the skin, producing draining abscesses. The swollen lymph nodes and abscesses are often referred to as buboes."}, {"id": "wiki20220301en071_45148", "title": "Sézary disease", "score": 0.009009009009009009, "content": "Generalized erythroderma– redness of the skin Lymphadenopathy – swollen, enlarged lymph nodes Atypical T-cells – malignant lymphocytes known as \"Sézary cells\" seen in the peripheral blood with typical cerebriform nuclei (brain-shaped, convoluted nuclei) Hepatosplenomegaly– enlarged liver and spleen Palmoplantar keratoderma – thickening of the palms of the hands, and soles of the feet Diagnosis Those who have Sézary disease often present with skin lesions that do not heal with normal medication. A blood test generally reveals any change in the levels of lymphocytes in the blood, which is often associated with a cutaneous T-cell lymphoma. Finally, a biopsy of a skin lesion can be performed to rule out any other causes."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[135, 310]], "word_ranges": [[24, 50]], "text": "in an immunocompromised patient and also with data of ocular involvement, admission for intravenous treatment would be indicated due to the high risk of possible complications."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "I think this question is not clearly in the Infectious Diseases syllabus, and may overlap with OFT and DERMA, but as I understand it, in an immunocompromised patient and also with data of ocular involvement, admission for intravenous treatment would be indicated due to the high risk of possible complications.", "full_answer_no_ref": "I think this question is not clearly in the Infectious Diseases syllabus, and may overlap with OFT and DERMA, but as I understand it, in an immunocompromised patient and also with data of ocular involvement, admission for intravenous treatment would be indicated due to the high risk of possible complications.", "full_question": "A 71-year-old woman with a history of rheumatoid arthritis on sulfasalazine, prednisone and etanercept. She goes to the emergency room for 72 hours of clinical manifestations compatible with facial herpes zoster affecting the right hemiface, auricular pavilion, respecting the forehead and conjunctival chemosis. What would be the appropriate treatment?", "id": 23, "lang": "en", "options": {"1": "Symptomatic treatment of pain only.", "2": "Topical treatment with acyclovir.", "3": "Outpatient treatment with acyclovir, valacyclovir or oral famciclovir.", "4": "Hospital admission and treatment with acyclovir or famciclovir iv.", "5": "Parenteral Ig and vaccination."}, "question_id_specific": 113, "type": "INFECTIOUS", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0942_16337", "title": "Treatment of herpes zoster ophthalmicus: a systematic review and Canadian cost-comparison.", "score": 0.017998385794995964, "content": "A systematic review and cost comparison were conducted to determine the optimal treatment of active herpes zoster ophthalmicus (HZO) in immunocompetent adults. A literature search of MEDLINE, EMBASE, CINAHL, Cochrane Library, BIOSIS Previews and Web of Science, ClinicalTrials.gov, International Clinical Trials Registry Platform, Networked Digital Library of Theses and Dissertations, and Canadian Health Research Collection was performed. The search period was from January 1990 to March 2017. Collectively, 516 immunocompetent patients with active HZO treated with oral antivirals were included. Randomized controlled trials (RCTs) investigating treatment of active HZO in immunocompetent adults, with one oral acyclovir monotherapy arm, were included. Studies fulfilling inclusion criteria were subjected to quality assessment and data extraction. Provincial drug formularies were consulted to extrapolate cost comparison for investigated treatment regimens. A total of 1515 titles and abstracts and 9 full-text articles were assessed. Three RCTs met the inclusion criteria. Treatment with oral acyclovir (800 mg 5 times daily for 10 days) was superior to placebo in the prevention of ocular manifestations. Oral famciclovir (500 mg 3 times daily for 7 days) and valacyclovir (1000 mg 3 times daily for 7 days) resulted in comparable rates of ocular manifestations relative to oral acyclovir (800 mg 5 times daily for 7 days). According to provincial drug formulary data, famciclovir and valacyclovir are more affordable across Canada with the recommended dosing schedules. Oral famciclovir and valacyclovir are reasonable alternatives to oral acyclovir for treatment of active HZO in immunocompetent individuals. Their simpler dosing schedules are associated with a cost benefit that is consistent across Canada."}, {"id": "wiki20220301en018_58885", "title": "Shingles", "score": 0.01666782415445517, "content": "Zoster ophthalmicus Treatment for zoster ophthalmicus is similar to standard treatment for shingles at other sites. A trial comparing acyclovir with its prodrug, valacyclovir, demonstrated similar efficacies in treating this form of the disease. The significant advantage of valacyclovir over acyclovir is its dosing of only three times/day (compared with acyclovir's five times/day dosing), which could make it more convenient for people and improve adherence with therapy."}, {"id": "wiki20220301en463_16802", "title": "Herpes zoster ophthalmicus", "score": 0.015685328185328185, "content": "Treatment Treatment is usually with antivirals such as acyclovir, valacyclovir, or famcyclovir by mouth. There is uncertainty as to the difference in effect between these three antivirals. Antiviral eye drops have not been found to be useful. These medications work best if started within 3 days of the start of the rash. Cycloplegics prevent synechiae from forming. References External links Varicella zoster virus-associated diseases Ophthalmology Wikipedia medicine articles ready to translate"}, {"id": "wiki20220301en033_16811", "title": "Postherpetic neuralgia", "score": 0.015615275813295616, "content": "Secondary prevention A 2013 Cochrane meta-analysis of 6 randomized controlled trials (RCTs) investigating oral antiviral medications given within 72 hours after the onset of herpes zoster rash in immunocompetent people for preventing postherpetic neuralgia (PHN) found no significant difference between placebo and acyclovir. Additionally, there was no significant difference in preventing the incidence of PHN found in the one RCT included in the meta-analysis that compared placebo to oral famciclovir treatment within 72 hours of HZ rash onset. Studies using valacyclovir treatment were not included in the meta-analysis. PHN was defined as pain at the site of the dermatomic rash at 120 days after the onset of rash, and incidence was evaluated at 1, 4, and 6 months after rash onset. Patients who are prescribed oral antiviral agents after the onset of rash should be informed that their chances of developing PHN are no different than those not taking oral antiviral agents."}, {"id": "pubmed23n0591_14859", "title": "Herpes zoster antivirals and pain management.", "score": 0.014938684503901897, "content": "Evaluation of evidence-based strategies for managing herpes zoster (HZ) and the pain of postherpetic neuralgia (PHN). Approximately 20% of the world's population suffers from herpes zoster at least once in a lifetime, with 10% to 20% having ophthalmic involvement. Treatment of the acute disease with oral antivirals may reduce the incidence and severity of complications but does not reliably prevent PHN or postherpetic itch (PHI). The acute pain abates as the acute phase resolves; the long-term pain of PHN or PHI may be severe and difficult to manage. Although many therapeutic agents have efficacy in the management of these complications, relief is frequently partial for months to the remainder of the lifetime. Literature review was performed using the resources of the Harvard Medical School/Massachusetts Eye and Ear Infirmary Ophthalmic library as well as the National Library of Medicine and the National Institutes of Health PubMed service searching by pertinent topics, authors, and journals. If started within 72 hours of the onset of the acute HZ rash, the oral antiviral agents acyclovir, valacyclovir, and famciclovir significantly shorten the periods of acute pain, virus shedding, rash, acute and late-onset anterior segment complications, and, in the case of valacyclovir and famciclovir, the incidence and severity of PHN. However, these medications do not prevent PHN, which remains a common and debilitating complication of HZ in older patients, requiring assiduous pain management. Tricyclic antidepressants, antiseizure drugs, opioids, and topical analgesics all offer some pain relief, and may be combined. Options are available to manage HZ and reduce the pain of PHN. However, prevention, now possible with the HZ vaccine, is preferable to treatment."}, {"id": "wiki20220301en013_136999", "title": "Varicella zoster virus", "score": 0.014664664664664664, "content": "The mutation rate for synonymous and nonsynonymous mutation rates among the herpesviruses have been estimated at 1 × 10−7 and 2.7 × 10−8 mutations/site/year, respectively, based on the highly conserved gB gene. Treatment Within the human body it can be treated by a number of drugs and therapeutic agents including acyclovir for the chicken pox, famciclovir, valaciclovir for the shingles, zoster-immune globulin (ZIG), and vidarabine. Acyclovir is frequently used as the drug of choice in primary VZV infections, and beginning its administration early can significantly shorten the duration of any symptoms. However, reaching an effective serum concentration of acyclovir typically requires intravenous administration, making its use more difficult outside of a hospital. Vaccination"}, {"id": "pubmed23n0638_22081", "title": "[Management of herpes zoster infection].", "score": 0.014133600725779499, "content": "Approximately 10 to 30% of the population will suffer from herpes zoster (HZ) during their lifetime. Prompt treatment of acute HZ with acyclovir, valacyclovir or famciclovir is recommend, if patients are over 50 years old or have severe or moderate pain or severe or moderate rash or they are immonocompromised or suffer from herpes zoster ophtalmicus. Zoster lesions contain high concentrations of Varicella zoster virus that can spread, and cause chicken pox. There is no universal recommendations for varicella vaccination. It has been shown that zoster vaccine markedly reduced morbidity from herpes zoster and postherpetic neuralgia among older adults."}, {"id": "article-35540_14", "title": "Herpes Zoster Ophthalmicus -- Treatment / Management", "score": 0.013868962219033956, "content": "Antiviral agents: Ideally, treatment with systemic antiviral agents should begin within 72 hours of disease onset. Initiation should not be delayed while awaiting definitive diagnosis or ophthalmology follow-up. Topical antiviral agents may be considered, but there is limited evidence regarding their utility in managing HZO. Immunocompetent adult dosing (choose single agent): Acyclovir 800 mg orally five times per day for at least 7 days Valacyclovir 1000 mg orally every eight hours for at least 7 days (may require renal dosing) Famciclovir 500 mg orally three times per day for at least 7 days (may require renal dosing) Immunocompromised adult dosing (choose a single agent): Acyclovir 10 mg/kg of ideal body weight (IBW) intravenously (IV) every eight hours for at least 7 days Foscarnet 90 mg/kg IV every 12 hours (typically reserved for severe or acyclovir-resistant disease)"}, {"id": "pubmed23n1149_17012", "title": "Antiviral treatment in outpatients with herps zoster in six major areas of China, 2010-2019.", "score": 0.013811899482631191, "content": "The objective of this study was to assess the status and trends of antiviral treatment in outpatients with herpes zoster in China. Prescription data on antiviral drugs were extracted from the database of the Hospital Prescription Analysis Program of China according to the inclusion criteria. Yearly prescriptions and costs were calculated, and trends were analyzed. The trends were further stratified by age, sex, and specific drug use. The distribution of defined daily costs (DDCs) of valaciclovir and famciclovir were analyzed, and trends in the median DDCs were identified. A total of 132,911 prescriptions from 49 hospitals located in six major areas of China were included in the analysis. The yearly prescriptions containing antivirals increased from 8,819 in 2010 to 16,361 in 2019. The percentage of prescriptions for patients aged 65 years and above also increased (27.7% in 2010 to 31.0% in 2019), and the number of prescriptions for females was higher than those for males (<iP</i &lt; 0.001). The average cost of antivirals per prescription decreased; thus, the yearly cost showed no increasing trend. The main prescribed antivirals were valaciclovir and famciclovir, which progressively increased in prescriptions. The use of acyclovir decreased during the study period. Prescriptions containing topical formulations, acyclovir and penciclovir, both increased. The DDCs of valaciclovir and famciclovir decreased dramatically. The use of antivirals has increased over the decade, while the cost has not. Antiviral treatments adhere well to recent recommendations, except for the use of topical antivirals. The findings of this study may benefit the healthcare source allocation and management of herpes zoster in China."}, {"id": "article-19888_61", "title": "Conjunctivitis -- Treatment / Management", "score": 0.013645745738093417, "content": "Treatment of herpes zoster conjunctivitis includes a combination of oral antivirals and topical steroids; however, steroids should only be part of therapy in consultation with ophthalmology. Antiviral doses differ from those used for herpes simplex and consist of acyclovir 800 mg PO 5 times a day, famciclovir 500 mg PO 3 times a day, or valacyclovir 1 g PO 3 times a day, each for 7 to 10 days."}, {"id": "wiki20220301en165_11893", "title": "Herpes gladiatorum", "score": 0.0134446198962328, "content": "Treatment Herpes outbreaks should be treated with antiviral medications like Acyclovir, Valacyclovir, or Famcyclovir, each of which is available in tablet form. Oral antiviral medication is often used as a prophylactic to suppress or prevent outbreaks from occurring. The recommended dosage for suppression therapy for recurrent outbreaks is 1,000 mg of valacyclovir once a day or 400 mg Acyclovir taken twice a day. In addition to preventing outbreaks, these medications greatly reduce the chance of infecting someone while the patient is not having an outbreak."}, {"id": "wiki20220301en063_70120", "title": "Herpetic whitlow", "score": 0.013390487854664217, "content": "In adults, it is more common for the primary source to be the genital region, with a corresponding preponderance of HSV-2. It is also seen in adult health care workers such as dentists because of increased exposure to the herpes virus. Contact sports are also a potential source of infection with herpetic whitlows. Treatment Although it is a self-limited illness, oral or intravenous antiviral treatments, particularly acyclovir, have been used in the management of immunocompromised or severely infected patients. It is usually given when the condition fails to improve on its own. Topical acyclovir has not been shown to be effective in management of herpetic whitlow. Famciclovir has been demonstrated to effectively treat and prevent recurrent episodes. Lancing or surgically debriding the lesion may make it worse by causing a superinfection or encephalitis."}, {"id": "pubmed23n0785_9215", "title": "A comparative study to evaluate the efficacy and safety of acyclovir and famciclovir in the management of herpes zoster.", "score": 0.012913820774082783, "content": "Over the years, acyclovir has been the oral antiviral agent approved for the treatment of patients with acute herpes zoster,Its effectiveness in lessening the acute signs and symptoms of herpes zoster has been established but the effects on post herpetic neuralgia are less clear cut. Famciclovir is a new member of guanine nucleoside family of drugs. It is a well absorbed oral form of penciclovir with longer half life. This was a open comparative randomized study carried out to compare the safety and efficacy of famciclovir administered at 250mg thrice daily with acyclovir 800mg five times daily for the treatment of acute uncomplicated herpes zoster in immunocompetent individuals aged above 40 years. To assess the clinical profile of Herpes zoster, compare the efficacy and safety of acyclovir and famciclovir in the treatment of herpes zoster and to describe the effectiveness of acyclovir and famciclovir preventing post herpetic neuralgia. A total of 100 newly zoster were randomized in 1:1 ratio into acyclovir and famciclovir groups after inclusion criteria were satisfied.Treatment was initiated within 72 hrs of onset of symptoms and was continued for 7 days and evaluated at the end of each week up to six weeks period for full crusting of the lesions, complete healing of the lesion and loss of acute pain. It was observed that famciclovir was as effective as acyclovir with no significant difference in time taken for full crusting, complete healing of lesions or loss of acute pain. Famciclovir was well tolerated with a better safety profile comparable to that of acyclovir. Constipation, headache, nausea and vomiting were the most commonly reported adverse effects, but constipation was considered to have a possible relationship to treatment. In conclusion, oral famciclovir administered three times daily for 7 days during acute zoster infection is as effective as acyclovir, administered 800mg five times daily.In addition it offers significant benefit by providing a well tolerated, cost effective, convenient dosage regime and accelerated rate of lesion resolution and a reduced duration of PHN."}, {"id": "wiki20220301en202_12109", "title": "Acute retinal necrosis", "score": 0.012868291129160695, "content": "Treatment Medication Currently treatment of ARN consists of antiviral therapy administered orally. Typical antiviral agents used include famciclovir, valganciclovir, and valacyclovir. While on these medications, a patient's kidney function should be watched. Some physician's also may administer the antiviral agents via intravitreal delivery. Though controversial, some physicians administer steroids (prednisone) and antithrombotic therapy (aspirin). Some commonly administered antiviral agents are as follows: Acyclovir Famciclovir Valacyclovir Gancicilovir Valganciclovir Research In a study done published by the British Journal of Ophthalmology, the cases of ARN/BARN reported in 2001-2002 in the UK, Varicella Zoster Virus was the most common culprit for the disease and presented mostly in men than in women."}, {"id": "wiki20220301en301_40256", "title": "Herpes esophagitis", "score": 0.012863311996569782, "content": "Differential diagnosis CMV, VZV as well as HIV infections of the esophagus can have a similar presentation. Tissue culture is the most accurate means of distinguishing between the different viral causes. Caustic esophagitis, pill-induced esophagitis as well as yeast esophagitis can have a similar clinical presentation. Prevention Herpes simplex virus is commonly found in humans, yet uncommonly results in systemic manifestations. Suppression of HIV with antiretroviral medications, careful monitoring of immunosuppressive medications are important means of prevention. Antiviral prophylaxis such as daily acyclovir in immunocompromised individuals may be considered. Treatment Antivirals such as acyclovir, famciclovir, or valacyclovir may be used. Intravenous acyclovir is reserved for individuals who cannot swallow due to the pain, individuals with other systemic manifestations of herpes or severely immunocompromised individuals. References"}, {"id": "InternalMed_Harrison_14451", "title": "InternalMed_Harrison", "score": 0.012810878511361604, "content": "lymphoproliferative malignancies), both chickenpox and herpes zoster (including disseminated disease) should be treated, at least at the outset, with IV acyclovir, which reduces the occurrence of visceral complications but has no effect on healing of skin lesions or pain. The dose is 10 mg/kg every 8 h for 7 days. For low-risk immunocompromised hosts, oral therapy with valacyclovir or famciclovir appears beneficial. If medically feasible, it is desirable to decrease immunosuppressive treatment concomitant with the administration of IV acyclovir. Patients with varicella pneumonia often require ventilatory support. Persons with zoster ophthalmicus should be referred immediately to an ophthalmologist. Therapy for this condition consists of the administration of analgesics for severe pain and the use of atropine. Acyclovir, valacyclovir, and famciclovir all accelerate healing. Decisions about the use of glucocorticoids should be made by the ophthalmologist. The management of acute"}, {"id": "pubmed23n0887_6966", "title": "Valacyclovir versus acyclovir for the treatment of herpes zoster ophthalmicus in immunocompetent patients.", "score": 0.01262847581865987, "content": "Herpes zoster ophthalmicus affects the eye and vision, and is caused by the reactivation of the varicella zoster virus in the distribution of the first division of the trigeminal nerve. An aggressive management of acute herpes zoster ophthalmicus with systemic antiviral medication is generally recommended as the standard first-line treatment for herpes zoster ophthalmicus infections. Both acyclovir and its prodrug valacyclovir are medications that are approved for the systemic treatment of herpes zoster. Although it is known that valacyclovir has an improved bioavailability and steadier plasma concentration, it is currently unclear as to whether this leads to better treatment results and less ocular complications. To assess the effects of valacyclovir versus acyclovir for the systemic antiviral treatment of herpes zoster ophthalmicus in immunocompetent patients. We searched CENTRAL (which contains the Cochrane Eyes and Vision Trials Register; 2016, Issue 5), Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid MEDLINE Daily, Ovid OLDMEDLINE (January 1946 to June 2016), Embase (January 1980 to June 2016), Web of Science Conference Proceedings Citation Index-Science (CPCI-S; January 1990 to June 2016), BIOSIS Previews (January 1969 to June 2016), the ISRCTN registry (www.isrctn.com/editAdvancedSearch), ClinicalTrials.gov (www.clinicaltrials.gov), and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP; www.who.int/ictrp/search/en). We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 13 June 2016. We considered all randomised controlled trials (RCTs) in which systemic valacyclovir was compared to systemic acyclovir medication for treatment of herpes zoster ophthalmicus. There were no language restrictions. Two review authors independently selected trials, evaluated the risk of bias in included trials, and extracted and analysed data. We did not conduct a meta-analysis, as only one study was included. We assessed the certainty of the evidence for the selected outcomes using the GRADE approach. One study fulfilled the inclusion criteria. In this multicentre, randomised double-masked study carried out in France, 110 immunocompetent people with herpes zoster ophthalmicus, diagnosed within 72 hours of skin eruption, were treated, with 56 participants allocated to the valacyclovir group and 54 to the acyclovir group. The study was poorly reported and we judged it to be unclear risk of bias for most domains.Persistent ocular lesions after 6 months were observed in 2/56 people in the valacyclovir group compared with 1/54 people in the acyclovir group (risk ratio (RR) 1.93 (95% CI 0.18 to 20.65); very low certainty evidence. Dendritic ulcer appeared in 3/56 patients treated with valacyclovir, while 1/54 suffered in the acyclovir group (RR 2.89; 95% confidence interval (CI) 0.31 to 26.96); very low certainty evidence), uveitis in 7/56 people in the valacyclovir group compared with 9/54 in the acyclovir group (RR 0.96; 95% CI 0.36 to 2.57); very low certainty evidence). Similarly, there was uncertainty as to the comparative effects of these two treatments on post-herpetic pain, and side effects (vomiting, eyelid or facial edema, disseminated zoster). Due to concerns about imprecision (small number of events and large confidence intervals) and study limitations, the certainty of evidence using the GRADE approach was rated as low to very low for the use of valacyclovir compared to acyclovir. This review included data from only one study, which had methodological limitations. As such, our results indicated uncertainty of the relative benefits and harms of valacyclovir over acyclovir in herpes zoster ophthalmicus, despite its widespread use for this condition. Further well-designed and adequately powered trials are needed. These trials should include outcomes important to patients, including compliance."}, {"id": "wiki20220301en099_37695", "title": "Genital herpes", "score": 0.012578616352201259, "content": "In people experiencing their first episode of genital herpes oral acyclovir may reduce the duration of symptoms and lesions but the risk of adverse effects is not certain. There may also be little or no difference between topical acyclovir and placebo in terms of duration of symptoms and lesions and the risk of adverse effects. Valacyclovir is a prodrug that is converted to acyclovir once in the body. It helps relieve the pain and discomfort and speeds healing of sores. It only comes in caplets and its advantage is that it has a longer duration of action than acyclovir. An example usage is by mouth twice per day for ten days for primary lesion, and twice per day for three days for a recurrent episode. Famciclovir is another antiviral drug that belongs to the same class. Famciclovir is a prodrug that is converted to penciclovir in the body. The latter is the one active against the viruses. It has a longer duration of action than acyclovir and it only comes in tablets."}, {"id": "InternalMed_Harrison_14231", "title": "InternalMed_Harrison", "score": 0.012472527472527473, "content": "Application at initial symptoms reduces healing time by 1 d. Therapy should be undertaken in consultation with an ophthalmologist. Valacyclovir may be more effective than acyclovir for pain relief; otherwise, it has a similar effect on cutaneous lesions and should be given within 72 h of rash onset. The duration of postherpetic neuralgia is shorter than with placebo. Famciclovir showed overall efficacy similar to that of acyclovir in a comparative trial. It should be given ≤72 h after rash onset. Acyclovir causes faster resolution of skin lesions than placebo and provides some relief of acute symptoms if given within 72 h of rash onset. Combined with tapering doses of prednisone, acyclovir improves quality-of-life outcomes. Antiviral Chemotherapy, Excluding Antiretroviral Drugs"}, {"id": "wiki20220301en001_99060", "title": "Ramsay Hunt syndrome type 2", "score": 0.012153455681374464, "content": "Treatments for Ramsay Hunt Syndrome Type 2 are used to reduce further damage causes by the viral infection. These medications will not reverse any damage that has already occurred at the time that they are prescribed. Initial treatment with a corticosteroid such as prednisone and the antiviral drug such as acyclovir (500 milligrams five times a day), valacyclovir (1000 mg three times a day) or famciclovir (500 mg three times a day) for 5 to 7 days is standard, however some studies have shown later damage to the facial nerve and recommend 21 days of antivirals. Studies indicate that treatment started within 72 hours of the onset of facial paralysis improves the chances of the patient experiencing significant recovery. Chances of recovery appear to decrease when treatment is delayed. Delay of treatment may result in permanent facial nerve paralysis. However, some studies demonstrate that even when steroids are started promptly, only 22% of all patient achieve full recovery of facial"}, {"id": "InternalMed_Harrison_14257", "title": "InternalMed_Harrison", "score": 0.012141670977050609, "content": "Because VZV is generally less sensitive to acyclovir than is HSV, higher doses of acyclovir must be used to treat VZV infections. In immunocompromised patients with herpes zoster, IV acyclovir reduces the frequency of cutaneous dissemination and visceral complications and—in one comparative trial—was more effective than vidarabine. Acyclovir, administered at oral doses of 800 mg five times a day, had a modest beneficial effect on localized herpes zoster lesions in both immunocompromised and immunocompetent patients. Combination of acyclovir with a tapering regimen of prednisone appeared to be more effective than acyclovir alone in terms of quality-of-life outcomes in immunocompetent patients over age 50 with herpes zoster. A comparative study of acyclovir (800 mg PO five times daily) and valacyclovir (1 g PO three times daily) in immunocompetent patients with herpes zoster indicated that the latter drug may be more effective in eliciting the resolution of zoster-associated pain."}, {"id": "pubmed23n0294_11796", "title": "Efficacy of famciclovir in the treatment of herpes zoster.", "score": 0.012068739341466614, "content": "Although vidarabine was the first systemic antiviral drug for the treatment of acute herpes zoster, the agent now used most frequently is acyclovir, a far safer drug that became available a decade ago. However, even with widespread use of acyclovir, postherpetic neuralgia (PHN) remains a principal cause of postinfectious morbidity. Newer antiviral agents, such as famciclovir and valacyclovir, have recently been introduced for the treatment of uncomplicated herpes zoster. In a double-blind, randomized study, 500 mg of famciclovir three times daily for 7 days was compared with placebo; in a second study, 500 mg of famciclovir three times daily for 7 days was compared with 800 mg of acyclovir five times daily for 7 days. Famciclovir significantly reduced duration of viral shedding (P = 0.0001) and accelerated lesion resolution compared with placebo. Famciclovir was comparable to acyclovir for these acute parameters. Most importantly, famciclovir recipients lost PHN two times faster than those receiving placebo (P = 0.02 all patients; P = 0.004 patients &gt; or = 50 years) resulting in a reduction in the median duration of PHN (56 days all patients; 100 days patients &gt; or = 50 years). This reduction translated to a 3.5-month reduction in the median duration of PHN for patients 50 years or older, those at greatest risk for developing the most common complication of herpes zoster. Famciclovir 500 mg administered three times a day for 7 days is an effective and well-tolerated treatment for acute herpes zoster, and is the only oral antiviral agent proven to reduce the duration of PHN when administered during acute zoster infection."}, {"id": "wiki20220301en489_1287", "title": "Neonatal infection", "score": 0.011878610365307948, "content": "Women with a history of genital herpes, can be treated with antiviral drugs to prevent symptomatic lesions and viral shedding that could infect the infant at birth. The antiviral medications used include acyclovir, penciclovir, valacyclovir, and famciclovir. Only very small amounts of the drug can be detected in the fetus. There are no increases in drug-related abnormalities in the infant that could be attributed to acyclovir. Long-term effects of antiviral medications have not been evaluated for their effects after growth and development of the child occurs. Neutropenia can be a complication of acyclovir treatment of neonatal HSV infection, but is usually transient. Treatment with immunoglobulin therapy has not been proven to be effective and is not recommended. Epidemiology"}, {"id": "wiki20220301en023_63168", "title": "Aciclovir", "score": 0.011768219832735962, "content": "A related prodrug form, valaciclovir came into medical use in 1995. It is converted to aciclovir in the body after absorption. In 2009, acyclovir in combination with hydrocortisone cream, marketed as Xerese, was approved in the United States for the early treatment of recurrent herpes labialis (cold sores) to reduce the likelihood of ulcerative cold sores and to shorten the lesion healing time in adults and children (six years of age and older). Society and culture Names Aciclovir is the International Nonproprietary Name (INN) and British Approved Name (BAN) while acyclovir is the United States Adopted Name (USAN) and former British Approved Name. It was originally marketed as Zovirax; patents expired in the 1990s and since then it is generic and is marketed under many brand names worldwide. Notes References"}, {"id": "article-17176_3", "title": "Acyclovir -- Indications", "score": 0.011711866592169166, "content": "Despite the long-term use of acyclovir to treat HSV encephalitis, there has not been a systematic review regarding the efficacy of this disease/treatment combination. Current systematic reviews addressing its safety and efficacy are ongoing, with the primary outcome being the mortality rate. A secondary outcome measure is the quality of life. [4] HSV keratitis has been shown to respond to oral acyclovir and topical steroids in pediatric patients. [5]"}, {"id": "pubmed23n1074_3379", "title": "Efficacy of valacyclovir and famciclovir in herpes zoster: A comparative study.", "score": 0.011680769868923179, "content": "The objective was to evaluate the efficacy of antiviral agent valacyclovir compared with famciclovir in the treatment of herpes zoster. A comparative study was conducted over a period of 1 year. Data relevant to the study were collected from 60 patients, with active herpes zoster presenting to the outpatient department within 72 hr of the first occurrence of zoster rash. They were divided in to two groups of 30 patients each. The first group of patients received valacyclovir tablet 1000 mg thrice daily, whereas those in the second group were given famciclovir tablet 500 mg thrice daily. Both the drugs were given for 7 days. Periodic follow-up till 29<supth</sup day was done for assessment of the effects of given drugs. Significant decrease was observed on comparison of pain scores between the two groups using the visual analog scale, with the drug valacyclovir, than in the famciclovir group at day 29. Furthermore, valacyclovir treatment accelerated the resolution of zoster associated pain in more number of patients compared with famciclovir. Oral valacyclovir administered during acute zoster infection for a period of 7 days offers significant benefit compared to famciclovir by providing a well tolerated and greater resolution of pain while maintaining the favorable safety profile, making valacyclovir more efficacious and a better drug in management of Herpes Zoster in comparison to famciclovir."}, {"id": "pubmed23n0680_13874", "title": "Nongenital herpes simplex virus.", "score": 0.011511072090527753, "content": "Nongenital herpes simplex virus type 1 is a common infection usually transmitted during childhood via nonsexual contact. Most of these infections involve the oral mucosa or lips (herpes labialis). The diagnosis of an infection with herpes simplex virus type 1 is usually made by the appearance of the lesions (grouped vesicles or ulcers on an erythematous base) and patient history. However, if uncertain, the diagnosis of herpes labialis can be made by viral culture, polymerase chain reaction, serology, direct fluorescent antibody testing, or Tzanck test. Other nonoral herpes simplex virus type 1 infections include herpetic keratitis, herpetic whitlow, herpes gladiatorum, and herpetic sycosis of the beard area. The differential diagnosis of nongenital herpes simplex virus infection includes aphthous ulcers, acute paronychia, varicella-zoster virus infection, herpangina, herpes gestationis (pemphigoid gestationis), pemphigus vulgaris, and Behçet syndrome. Oral acyclovir suspension is an effective treatment for children with primary herpetic gingivostomatitis. Oral acyclovir, valacyclovir, and famciclovir are effective in treating acute recurrence of herpes labialis (cold sores). Recurrences of herpes labialis may be diminished with daily oral acyclovir or valacyclovir. Topical acyclovir, penciclovir, and docosanol are optional treatments for recurrent herpes labialis, but they are less effective than oral treatment."}, {"id": "wiki20220301en138_13991", "title": "Varicella vaccine", "score": 0.011453823953823954, "content": "weeks, because live vaccines that are administered too soon within one another may not be as effective. It may be usable in people with HIV infections who have a good blood count and are receiving appropriate treatment. Specific antiviral medication, such as acyclovir, famciclovir, or valacyclovir, are not recommended 24 hours before and 14 days after vaccination."}, {"id": "wiki20220301en169_39507", "title": "Lawrence Corey", "score": 0.011438923395445135, "content": "In the early 1980s, Corey worked with Nobel Prize-winning biochemist and pharmacologist Dr. Gertrude Elion to demonstrate that an antiviral that was selective and specific for a viral-specified enzyme could be safely and effectively administered to control a chronic viral infection (herpes simplex virus type 2 or HSV-2). Corey first conceived of and demonstrated the core concepts and direct line association between quantitative viral load reduction and clinical benefit using topical, intravenous and oral formulations of acyclovir in classic studies performed between 1980 and 1984. Acyclovir was the first antiviral drug to get rapid approval from the FDA, and it was Corey's studies that defined its use in genital herpes. These studies led to the licensure for acyclovir in a wide variety of infections such as HSV-1, HSV-2 and varicella chickenpox virus, including the first use of an antiviral for daily long term use. Acyclovir and its derivatives valacyclovir and famciclovir are the"}, {"id": "InternalMed_Harrison_14258", "title": "InternalMed_Harrison", "score": 0.011334590404357846, "content": "and valacyclovir (1 g PO three times daily) in immunocompetent patients with herpes zoster indicated that the latter drug may be more effective in eliciting the resolution of zoster-associated pain. Orally administered acyclovir (600 mg five times a day) reduced complications of herpes zoster ophthalmicus in a placebo-controlled trial."}, {"id": "InternalMed_Harrison_14408", "title": "InternalMed_Harrison", "score": 0.0112151964610981, "content": "c. Suppression of recurrent genital herpes: Oral acyclovir (400–800 mg bid) or valacyclovir (500 mg daily) is given. Patients with >9 episodes per year should take oral valacyclovir (1 g daily or 500 mg bid) or famciclovir (250 mg bid or 500 mg bid). 2. Oral-labial HSV infections a. First episode: Oral acyclovir is given (200 mg 5 times per day or 400 mg tid); an oral acyclovir suspension can be used (600 mg/m2 qid). Oral famciclovir (250 mg bid) or valacyclovir (1 g bid) has been used clinically. The duration of therapy is 5–10 days. b."}, {"id": "wiki20220301en386_27951", "title": "Herpes simplex keratitis", "score": 0.011176628249798981, "content": "Epithelial keratitis Epithelial keratitis is treated with topical antivirals, which are very effective with low incidence of resistance. Treatment of the disease with topical antivirals generally should be continued for 10–14 days. Aciclovir ophthalmic ointment and Trifluridine eye drops have similar effectiveness but are more effective than Idoxuridine and Vidarabine eye drops. Oral acyclovir is as effective as topical antivirals for treating epithelial keratitis, and it has the advantage of no eye surface toxicity. For this reason, oral therapy is preferred by some ophthalmologists. Ganciclovir and brivudine treatments were found to be equally as effective as acyclovir in a systematic review. Valacyclovir, a pro-drug of acyclovir likely to be just as effective for ocular disease, can cause thrombotic thrombocytopenic purpura/Hemolytic-uremic syndrome in severely immunocompromised patients such as those with AIDS; thus, it must be used with caution if the immune status is unknown."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[343, 444]], "word_ranges": [[53, 70]], "text": "Therefore, it is necessary to wait for the imaging test to confirm the etiology (option 1 incorrect)."}, "2": {"exist": true, "char_ranges": [[445, 523]], "word_ranges": [[70, 80]], "text": "The most frequent cause is aldosterone-producing adenoma (option 2 incorrect)."}, "3": {"exist": true, "char_ranges": [[0, 342]], "word_ranges": [[0, 53]], "text": "We are presented with a patient with resistant arterial hypertension, assuming that he has primary hyperaldosteronism due to hypokalemic metabolic alkalosis. When the diagnosis is biochemically confirmed, the next test to be performed is a CT scan to determine the subtype and rule out the presence of an adrenal carcinoma (option 3 correct)."}, "4": {"exist": true, "char_ranges": [[524, 595]], "word_ranges": [[80, 90]], "text": "Spironolactone is the medical treatment of choice (option 4 incorrect)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "We are presented with a patient with resistant arterial hypertension, assuming that he has primary hyperaldosteronism due to hypokalemic metabolic alkalosis. When the diagnosis is biochemically confirmed, the next test to be performed is a CT scan to determine the subtype and rule out the presence of an adrenal carcinoma (option 3 correct). Therefore, it is necessary to wait for the imaging test to confirm the etiology (option 1 incorrect). The most frequent cause is aldosterone-producing adenoma (option 2 incorrect). Spironolactone is the medical treatment of choice (option 4 incorrect).", "full_answer_no_ref": "We are presented with a patient with resistant arterial hypertension, assuming that he has primary hyperaldosteronism due to hypokalemic metabolic alkalosis. When the diagnosis is biochemically confirmed, the next test to be performed is a CT scan to determine the subtype and rule out the presence of an adrenal carcinoma ([HIDDEN]). Therefore, it is necessary to wait for the imaging test to confirm the etiology ([HIDDEN]). The most frequent cause is aldosterone-producing adenoma ([HIDDEN]). Spironolactone is the medical treatment of choice ([HIDDEN]).", "full_question": "A 58-year-old man with a 6-year history of hypertension consults for poor blood pressure control despite treatment with an angiotensin-converting enzyme inhibitor, a diuretic and a calcium antagonist. On consultation she presented with blood pressure of 149/100 mmHg. Laboratory tests: creatinine 1.2 mg/dl, potassium 2.2 mEq/l and compensated metabolic alkalosis; the rest of the biochemical study, blood count, coagulation and urinary sediment were normal. Point out the correct statement:", "id": 564, "lang": "en", "options": {"1": "The origin of hypertension in this case is excessive secretion of aldosterone caused by autonomic hyperfunction of the adrenal medulla.", "2": "In most cases the anatomical substrate is a bilateral hyperplasia of the adrenal cortex.", "3": "CT scan is part of the diagnostic study in case of biochemical confirmation.", "4": "Spironolactone is contraindicated in the management of this pathology.", "5": NaN}, "question_id_specific": 126, "type": "NEPHROLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0640_19248", "title": "Hypokalemia induced myopathy as first manifestation of primary hyperaldosteronism - an elderly patient with unilateral adrenal hyperplasia: a case report.", "score": 0.01879964695498676, "content": "Primary hyperaldosteronism is only rarely caused by unilateral adrenal hyperplasia. A 73-year-old hypertensive Greek man (on 10 mg amlodipine for the last ten years) presented in the emergency department with severe muscle weakness of all limbs. The initial physical and laboratory examination revealed normal blood pressure, muscle weakness, severe hypokalemia, sinus rhythm and U wave, rhabdomyolysis and metabolic alkalosis. The patient was immediately treated with intravenous administration of potassium-rich solutions, 25 mg spironolactone with progressive dose titration up to 100 mg. Because of high arterial blood pressure, irbesartan was added. On day 6, muscle weakness was completely restored with decrease of arterial blood pressure and further improvement of laboratory tests. The combination of hypokalemia with arterial hypertension raised the suspicion of primary hyperaldosteronism; therefore, we performed abdomen computed tomography scan, which revealed a nodular mass (15 mm in diameter) in the left adrenal gland. Plasma renin activity was in the lower normal range with a three-fold increase of plasma aldosterone concentration. We performed total resection of the left adrenal gland and the histopathological examination revealed hyperplasia of the left adrenal gland. This report presents a rare case of an elderly patient under antihypertensive treatment the last ten years for essential hypertension, who admitted to our emergency department with hypokalemia - induced myopathy as first manifestation of primary hyperaldosteronism due to unilateral adrenal hyperplasia."}, {"id": "pubmed23n0818_17244", "title": "[High doses of aldosterone antagonist is a condition of sufficient blood pressure control in bilateral adrenal hyperplasia].", "score": 0.017078071182548796, "content": "Primary aldosteronism occurs in 1-10% of hypertensive patients and is classified in adenomas or bilateral adrenal hyperplasia. Computed tomography (CT) or magnetic resonance imaging can be used to discriminate these subtypes and in guiding treatment selection. This case report describes a 65-year-old man with hypertension and hypokalaemia during 25 years. Bilateral adrenal hyperplasia was diagnosed based on a CT, and an oral sodium-loading test with measurement of renin and aldosterone confirmed the diagnosis. Blood pressure and potassium in plasma was normalized during treatment with the mineralocorticoid receptor antagonist eplerenon. "}, {"id": "pubmed23n0912_14559", "title": "Diagnosis and management of primary aldosteronism.", "score": 0.016680339075745145, "content": "Primary aldosteronism (PA) is the most common form of secondary hypertension (HTN), with an estimated prevalence of 4% of hypertensive patients in primary care and around 10% of referred patients. Patients with PA have higher cardiovascular morbidity and mortality than age- and sex-matched patients with essential HTN and the same degree of blood pressure elevation. PA is characterized by an autonomous aldosterone production causing sodium retention, plasma renin supression, HTN, cardiovascular damage, and increased potassium excretion, leading to variable degrees of hypokalemia. Aldosterone-producing adenomas (APAs) account for around 40% and idiopathic hyperaldosteronism for around 60% of PA cases. The aldosterone-to-renin ratio is the most sensitive screening test for PA. There are several confirmatory tests and the current literature does not identify a \"gold standard\" confirmatory test for PA. In our institution, we recommend starting case confirmation with the furosemide test. After case confirmation, all patients with PA should undergo adrenal CT as the initial study in subtype testing to exclude adrenocortical carcinoma. Bilateral adrenal vein sampling (AVS) is the gold standard method to define the PA subtype, but it is not indicated in all cases. An experienced radiologist must perform AVS. Unilateral laparoscopic adrenalectomy is the preferential treatment for patients with APAs, and bilateral hyperplasia should be treated with mineralocorticoid antagonist (spironolactone or eplerenone). Cardiovascular morbidity caused by aldosterone excess can be decreased by either unilateral adrenalectomy or mineralocorticoid antagonist. In this review, we address the most relevant issues regarding PA screening, case confirmation, subtype classification, and treatment."}, {"id": "pubmed23n0413_13366", "title": "[Primary aldosteronism and pregnancy: report of 2 cases].", "score": 0.015772478887232988, "content": "Based on two patients, we discuss the difficulties in diagnosing and managing primary aldosteronism in pregnancy, which derive from changes of the renin-angiotensin-aldosterone axis, from the uncertainty regarding blood pressure control along gestation and postpartum, and from the contraindication to the use of spironolactone. The first case is a 27 years old woman with a long standing refractory hypertension, a hemorrhagic stroke with left brachial hemiplegia and crural hemiparesia, two miscarriages, one stillbirth and one offspring with intrauterine growth retardation. Due to hypokalemia, a plasma aldosterone/renin activity ratio of 91, and a negative genetic screening for glucocorticoid remediable aldosteronism (GRA), a primary hyperaldosteronism with normal adrenals in CT scan was diagnosed, and good blood pressure control was attained with spironolactone. After two and a half years of normotension, a fifth pregnancy, managed with methyldopa evolved with satisfactory blood pressures, plasma potassium, fetal growth, uterine and umbilical arterial resistance indexes, and maternal endothelial function. At 37 1/2 weeks of pregnancy the patient delivered a healthy newborn weighing 2,960 g. Blood pressure rose during the 48 hours of postpartum in the absence of proteinuria and required i.v. hydralazine. The second patient is a 37 years old woman, with known refractory hypertension for 7 years, hypokalemia, plasma aldosterone/renin activity ratio greater than 40, normal adrenals in the CAT scan, and a negative genetic screening for GRA. She had normotensive pregnancies 5 and 3 years prior to the detection of hypertension, with hypertensive crisis in both postpartum periods, retrospectively considered as expressions of primary hyperaldosteronism."}, {"id": "pubmed23n0547_4857", "title": "[Modern pharmacological aspects of hyperaldosteronism therapy].", "score": 0.015191995390015192, "content": "The prevalence of primary hyperaldosteronism is 5-10% of all hypertensive patients, and clearly above the estimated prevalence in the past. In nearly 30% of patients with therapy resistant hypertension, primary hyperaldosteronism is detected if they are investigated thoroughly. This will result in 1.5 to 2.5 million people in Germany suffering from primary hyperaldosteronism. Besides efficient diagnostic procedures, an effective treatment is of increasing importance. The aldosterone-producing adenoma (Conn's syndrome) is primarily cured by operation, in most cases performed endoscopically. Bilateral hyperplasia, which is found in two-thirds of primary hyperaldosteronism, is treated primarily by mineralocorticoid receptor antagonist: 12.5-50 mg/day spironolactone (in case of anti-androgenic side-effects alternatively by 50-100 mg/day eplerenone). If the blood pressure can not be lowered by this first-line treatment, an additional treatment with potassium-sparing diuretics, calcium-antagonists, ACE-inhibitors or angiotensin-2-antagonists is necessary. The start of medication should be closely monitored by serum electrolyte and creatinine controls."}, {"id": "pubmed23n0422_20006", "title": "[Reninoma: a rare but curable cause of high blood pressure, a case report].", "score": 0.014588610563842763, "content": "We report a case of a renin secreting tumor, which is a very rare cause of secondary high blood pressure. A 22-year-old woman was hospitalised for exploration of high blood pressure (160/110 mmHg) with severe hypokaliemia (2,7 mmol/l) and secondary hyperaldosteronism. Physical examination was normal except the high blood pressure. Bioassays show increased kaliuresis (66 mmol/24h), plasma renin (89 pg/ml in clinostastism--108 pg/ml in orthostatism), pro-renin (1207 pg/ml in clinostastism--1412 pg/ml in orthostatism) and aldosterone (210 pg/ml in clinostastism--566 pg/ml in orthostatism). The rest of the endocrine tests were normal (cortisol and ACTH at 8:00 am, urinary free cortisol, overnight 1 mg dexamethasone suppression test). Doppler ultrasound method, performed by an experienced radiologist, did not show renal artery stenosis. Abdominal computerized tomography showed a nodular formation at the upper pole of the right kidney, isodense to renal medullary. The size tumor was 15 mm. The renal vein sampling shows high values of renin on both sides whereas, for the pro-renin, the values were higher on the tumor side. In spite of treatment with CEI (Converting Enzyme Inhibitors) and calcium antagonists, the blood pressure was not controlled. Hypokaliemia persisted (3 mmol/l) in spite of high daily potassium intake (64 mmol/l of potassium chloride). After tumor resection, reninoma was diagnosed by the pathology examination and blood pressure, plasma rennin, plasma aldosterone level returned to normal."}, {"id": "wiki20220301en083_35405", "title": "Apparent mineralocorticoid excess syndrome", "score": 0.014110965487112277, "content": "Diagnosis Other conditions such as Liddle's Syndrome can mimic the clinical features of AME, so diagnosis can be made by calculating the ratio of free urinary cortisol to free urinary cortisone. Since AME patients create less cortisone, the ratio will much be higher than non-affected patients. Alternatively, one could differentiate between the two syndromes by administering a potassium-sparing diuretic. Patients with Liddle's syndrome will only respond to a diuretic that binds the ENaC channel, whereas those with AME will respond to a diuretic that binds to ENaC or the mineralcorticoid receptor. Treatment The treatment for AME is based on the blood pressure control with Aldosterone antagonist like Spironolactone which also reverses the hypokalemic metabolic alkalosis and other anti-hypertensives. Renal transplant is found curative in almost all clinical cases.AME is exceedingly rare, with fewer than 100 cases recorded worldwide."}, {"id": "pubmed23n0316_19475", "title": "[Primary hyperaldosteronism. Apropos of 2 cases].", "score": 0.013699204021784667, "content": "Primary hyperaldosteronism (PHA) represents less than 1 to 2% of all causes of hypertension (HT). We report 2 cases of primary hyperaldosteronism which emphasize the difficulty of distinguishing neoplastic PHA from idiopathic PHA, observed in a 60-year-old woman and a 42-year old woman, respectively. In both cases, the diagnosis of PHA was suggested by marked hypokalaemia with inappropriate potassium excretion and was confirmed by hyperaldosteronaemia and low and poorly stimulated renin activity. In the first case, computed tomography showed nodular hyperplasia of the 2 adrenal glands. The patient was treated with spironolactone and calcium channel blockers which controlled blood pressure and serum potassium. In the second case, computed tomography and magnetic resonance imaging revealed an adrenocortical adenoma confirmed by pathological examination after the operation. The diagnosis of primary hyperaldosteronism is based on three steps: detection, positive diagnosis and aetiological diagnosis. Detection is essentially based on demonstration of hypokalaemia. Positive diagnosis is based on demonstration of elevated aldosterone secretion with inhibited renin secretion. The aetiological diagnosis is dominated by the differentiation between Conn's adenoma and bilateral adrenal hyperplasia, which has therapeutic implications."}, {"id": "pubmed23n0562_10424", "title": "[Clinical characteristics and surgery outcomes of unilateral nodular adrenal hyperplasia in primary aldosteronism: study of 145 cases].", "score": 0.013550747549573151, "content": "To investigate the clinical characteristics, differential diagnosis, and surgery outcome of unilateral nodular adrenal hyperplasia (UNAH). The clinical data of 145 patients with primary aldosteronism, 67 males and 78 females, aged 37.9 (19-60), including 78 cases of aldosterone-producing adenoma (APA), 14 cases of UNAH, and 55 cases of idiopathic bilateral adrenal hyperplasia (BAH), were collected. Radioimmunoassay was used to examine the blood and urine aldosterone and plasma rennin activity. Automatic biochemical apparatus was used to examine the blood and urine electrolytes, renal functions, and urine microalbumin. Twelve-lead electrocardiography, echocardiography, and plain scanning of enhanced CT scanning of the bilateral adrenals were conducted. Adrenal venous sampling (AVS) was conducted in 62 patients to collect blood samples from vena cava and bilateral suprarenal veins to detect the levels of aldosterone and cortisol. All UNAH patients and 3 BAH patients underwent unilateral adrenalectomy and three APA patients underwent unilateral adrenalectomy or adenoma resection. Then the patients were followed up for 39.2 months. The incidence of UNAH is 9.7% in the primary aldosteronism patients. There were no significant differences in age, gender, duration of hypertension, blood pressure (SBP, DBP), and indexes indicating damages in target organs of hypertension (left ventricular hypertrophy rate, blood creatinine, urine microalbumin, etc) among these three groups. The level of serum potassium of the APA group was significantly lower than that of the BAH group (P &lt; 0.01), and the levels of plasma and urine aldosterone of the APA group were significantly higher than those of the BAH group (P &lt; 0.05 and P &lt; 0.01). The serum potassium of the UNAH group was higher than that of the APA group and lower than that of the BAH group, and the levels of plasma and urine aldosterone of the UNAH group were both higher than those of the APA group and lower than those of the BAH group, however all not significantly (all P &gt; 0.05). The coincidence rate of CT was 50% (7/14) in the UNAH group. The accuracy of AVS for diagnosis of UNAH was 85.7% (12/14). After operation, the serum potassium and plasma aldosterone concentrations returned normal in all the UNAH patients. Blood pressure returned to normal in 50% (7/14) of the UNAH patients, and was improved in the other 50% (7/14) patients. UNAH can be cured by adrenal surgery. The diagnostic values of clinical examination and adrenal CT are limited. AVS is essential in diagnosing UNAH patients."}, {"id": "wiki20220301en104_37759", "title": "Pseudohyperaldosteronism", "score": 0.013536953242835596, "content": "Pseudohyperaldosteronism (also pseudoaldosteronism) is a medical condition which mimics the effects of elevated aldosterone (hyperaldosteronism) by presenting with high blood pressure (hypertension), low blood potassium levels (hypokalemia), metabolic alkalosis, and low levels of plasma renin activity (PRA). However, unlike hyperaldosteronism, this conditions exhibits low or normal levels of aldosterone in the blood. Causes include genetic disorders (e.g. Apparent mineralocorticoid excess syndrome, Liddle's syndrome, and types of Congenital adrenal hyperplasia), acquired conditions (e.g. Cushing's syndrome and mineralocorticoid-producing adrenal tumors), metabolic disorders, and dietary imbalances including excessive consumption of licorice. Confirmatory diagnosis depends on the specific root cause and may involve blood tests, urine tests, or genetic testing; however, all forms of this condition exhibit abnormally low concentrations of both plasma renin activity (PRA) and plasma"}, {"id": "pubmed23n0742_19388", "title": "[Primay hyperaldosteronism--diagnostic and treatment].", "score": 0.013437950937950938, "content": "Primary hyperaldosteronism (PHA) is characterized by an increased Aldosterone synthesis which is independent of the Renin-Angiotensin-Aldosterone-System (RAAS). The prevalence of PHA in patients who present in specialized hypertension centers is approx. 10 %. Besides patients with the classical symptoms known as \"Conn-Trias\" (hypertension, hypokalemia, metabolic alkalosis), the more frequent normokalemic patients with PHA also show a worse outcome compared to patients with essential hypertension. Identifying these patients is an important task in the evaluation of hypertension since targeted treatment options are available. Screening for PHA using the Aldosterone-Renin-Ratio (ARR) should be performed in patients with hypokalemic, severe or resistant hypertension. In addition, young patients with early onset of severe hypertension and/or positive family history should be screened. A positive screening result should be followed by a confirmatory test. The saline infusion test is the preferred clinical test for confirming a suspected PHA since it is accessible and time efficient. Other confirmatory tests are not used on a regular basis. After any confirmatory test, CT- or MRI-imaging and adrenal vein sampling (AVS) is used in order to differentiate between a unilateral adenoma, a bilateral hyperplasia or another cause of PHA. CT or MRI usually cannot discriminate smaller tumors form hyperplasia. Therefore AVS is used to detect lateralization of autonomous aldosterone production. Lateralization of aldosterone production indicates a unilateral adenoma. In these cases, laparoscopic adrenalectomy is the therapeutic option of choice with a hypertension cure rate of up to 60 %. If no lateralization is detectable, bilateral hyperplasia as the underlying cause of PHA is likely. Pharmacological inhibition of the mineralocorticoid receptor is the preferred treatment option in these cases. If Spironolactone is not well tolerated, Eplerenone and potassium-sparing diuretics should be prescribed. Often, however, in order to fully control hypertension, additional antihypertensive therapy is necessary."}, {"id": "pubmed23n0567_22837", "title": "Primary aldosteronism: renaissance of a syndrome.", "score": 0.01330085450257124, "content": "Great strides have been made in our understanding of the pathophysiology of primary aldosteronism syndrome since Conn's description of the clinical presentation of a patient with an aldosterone-producing adenoma (APA) more than 50 years ago. It is now recognized that the APA is just one of the seven subtypes of primary aldosteronism. APA and bilateral idiopathic hyperaldosteronism (IHA) are the most common subtypes of primary aldosteronism. Although most clinicians had thought primary aldosteronism to be a rare form of hypertension for more than three decades, it is now recognized to be the most common form of secondary hypertension. Using the plasma aldosterone to plasma renin activity ratio as a case-finding test, followed by aldosterone suppression confirmatory testing, has resulted in much higher prevalence estimates of 5-13% of all patients with hypertension. In addition, there has been a new recognition of the aldosterone-specific cardiovascular morbidity and mortality associated with aldosterone excess. Although thought to be daunting and complex in the past, the diagnostic approach to primary aldosteronism is straightforward and can be considered in three phases: case-finding tests, confirmatory tests and subtype evaluation tests. Patients with hypertension and hypokalaemia (regardless of presumed cause), treatment-resistant hypertension (three antihypertensive drugs and poor control), severe hypertension (&gt;or= 160 mmHg systolic or &gt;or= 100 mmHg diastolic), hypertension and an incidental adrenal mass, onset of hypertension at a young age or patients being evaluated for other forms of secondary hypertension should undergo screening for primary aldosteronism. In patients with suspected primary aldosteronism, screening can be accomplished by measuring a morning (preferably between 0800 and 1000 h) ambulatory paired random plasma aldosterone concentration (PAC) and plasma renin activity (PRA). An increased PAC:PRA ratio is not diagnostic by itself, and primary aldosteronism must be confirmed by demonstrating inappropriate aldosterone secretion. Aldosterone suppression testing can be performed with orally administered sodium chloride and measurement of urinary aldosterone or with intravenous sodium chloride loading and measurement of PAC. Unilateral adrenalectomy in patients with APA or unilateral adrenal hyperplasia results in normalization of hypokalaemia in all these patients; hypertension is improved in all and is cured in approximately 30-60% of them. In bilateral adrenal forms of primary aldosteronism, unilateral or bilateral adrenalectomy seldom corrects the hypertension and they should be treated medically with a mineralocorticoid receptor antagonist."}, {"id": "wiki20220301en073_38761", "title": "Hyperaldosteronism", "score": 0.012862025544162619, "content": "Primary Primary aldosteronism (hyporeninemic hyperaldosteronism) was previously thought to be most commonly caused by an adrenal adenoma, termed Conn's syndrome. However, recent studies have shown that bilateral idiopathic adrenal hyperplasia is the cause in up to 70% of cases. Differentiating between the two is important, as this determines treatment. Also, see congenital adrenal hyperplasia. Adrenal carcinoma is an extremely rare cause of primary hyperaldosteronism. Two familial forms have been identified: type I (dexamethasone suppressible), and type II, which has been linked to the 7p22 gene. Features Hypertension Hypokalemia (e.g., may cause muscle weakness) Alkalosis Investigations High serum aldosterone Low serum renin High-resolution CT abdomen Management Adrenal adenoma: surgery Bilateral adrenocortical hyperplasia: aldosterone antagonist, e.g., spironolactone"}, {"id": "pubmed23n0349_17935", "title": "[Primary hyperaldosteronism--12 clinical cases].", "score": 0.012185665226542595, "content": "To show clinical, biochemical, and morphological data of 12 patients with primary hyperaldosteronism: eight with an aldosterone-producing adenoma and four with adrenal hyperplasia. To compare clinical and biochemical parameters of the patients with adenoma and hyperplasia. For those with adenoma, to verify clinical and biochemical modifications after adrenalectomy. In the 12 patients with hyperaldosteronism, retrospective analysis of clinical (age, sex, blood pressure), biochemical (plasmatic and urinary potassium, plasmatic aldosterone, plasma renin activity, and plasmatic aldosterone/renin activity ratio), and morphological (computed tomography, magnetic resonance, and norcholesterol scintigraphy) data was performed. 1--In the 12 patients with hyperaldosteronism (seven female), the age was 51.0 +/- 10.2 years (mean +/- standard deviation), the systolic pressure 200.9 +/- 34.5 mm Hg and the diastolic pressure 120.0 +/- 12.3 mm Hg. Hypertension was diagnosed 12.0 +/- 10.1 years before. As biochemical evidence, we found kalaemia of 3.06 +/- 0.28 and urinary potassium of 63.4 +/- 16.5 mEq/l, renin activity 0.98 +/- 1.02 ng/ml/h, plasmatic aldosterone of 49.4 +/- 36.0 ng/dl, aldosterone/renin activity &gt; 30 in 83% of the cases. As morphological evidence, computed tomography allowed diagnosis in nine patients, suggested it in two, being doubtful in one. Performed on four patients, resonance confirmed the tomography in three and was not contributive in one. The scintigraphy performed in four patients visualized two adenomas, was negative in one adenoma and in one hyperplasia. 2--In the eight patients with adenoma (six female), the youngest age and the highest diastolic pressure compared with patients with hyperplasia were statistically significant (p &lt; 0.01 and 0.05). In the adenomas, the biochemical changes were more pronounced, but not statistically significant. The plasmatic aldosterone/renin activity ratio was also higher in the adenoma cases. 3--After the adrenalectomy, blood pressure became normal in five patients and was more easily therapeutically controlled in three. The average systolic and diastolic pressures decreased and the biochemical parameters became normal in all patients. The pre/post surgical modification of these parameters had statistical significance (systolic pressure decrease, p &lt; 0.01; diastolic pressure decrease, p &lt; 0.01; kalaemia increase, p &lt; 0.001; renin activity increase, p &lt; 0.01; aldosterone decrease, p &lt; 0.02). The plasmatic aldosterone/renine activity ratio normalized in all patients. In diagnosing primary hyperaldosteronism, biochemical (kalaemia, urinary potassium, plasmatic aldosterone, renin activity, aldosterone plasmatic/renin activity) and tomography studies were important. On comparing the patients with hyperplasia with those with adenoma, we found that the latter are younger and exhibit higher diastolic pressure, both findings with statistical significance. After adenoma surgery, blood pressure became normal in five patients and improved in three, these findings, and the improvement of the kalaemia, plasmatic aldosterone, and renin activity parameters were statistically significant."}, {"id": "InternalMed_Harrison_27001", "title": "InternalMed_Harrison", "score": 0.012175324675324676, "content": "In patients with normal adrenal morphology and family history of early-onset, severe hypertension, a diagnosis of GRA should be Disorders of the Adrenal Cortex Clinical suspicion of mineralocorticoid excess Patients with hypertension and Severe hypertension (>3 BP drugs, drug-resistant) or Family history of early-onset hypertension or cerebrovascular events at < 40 years of age Measurement of aldosterone-renin ratio (ARR) on current blood pressure medication (stop spironolactone for 4 wks) and with hypokalemia corrected (ARR screen positive if ARR >750 pmol/L: ng/ml/h and aldosterone >450 pmol/L) (consider repeat off ˜-blockers for 2 wks if results are equivocal) Negative E.g., saline infusion test (2 liters physiologic saline over 4 h IV), oral sodium loading, fludrocortisone suppression Confirmation of diagnosis Rare: Both renin and Aldo suppressed"}, {"id": "pubmed23n0996_15138", "title": "[Primary hyperaldosteronism in a population of hypertensive patients].", "score": 0.011787551527952848, "content": "The diagnosis of primary hyperaldosteronism (PHPA) has progressively increased over the last years and some authors consider it as the main cause of secondary hypertension. We studied the prevalence of PHPA in hypertensive patients followed at the Hypertension Unit from July 1999 to July 2017. A total of 2500 patients were included and diagnosis of PHPA was done in 79 of them (3.2%). It was more frequent in women (55.7%) with an increased incidence in the elderly, as compared to previous studies (27.8%). Initial diagnosis was suspected upon the presence of inappropriate kaliuria and metabolic alkalosis, associated to an aldosterone/plasma renin activity ratio &gt; 30 (ng/dl)/(ng/ml/h). After confirmation of the presence of PA, imaging techniques to determine the etiology were performed. In this way, 29 cases (36.8%) of aldosterone-producing adenoma and 5 cases of bilateral adrenal hyperplasia with nodules were identified. Computed tomography identified the adenomas and hyperplasias with bilateral cortical nodules in all patients. Adrenalectomy and/or antialdosteronics were efficient in controlling blood pressure in 69.9% of cases. Of note in this series was the remission of stage 3 chronic renal failure in two cases, the high prevalence of hypercalciuric urinary lithiasis and a case of breast carcinoma after prolonged treatment with spironolactone."}, {"id": "wiki20220301en019_21526", "title": "Primary aldosteronism", "score": 0.011534861249509315, "content": "Primary hyperaldosteronism has a number of causes. About 33% of cases are due to an adrenal adenoma that produces aldosterone, and 66% of cases are due to an enlargement of both adrenal glands. Other uncommon causes include adrenal cancer and an inherited disorder called familial hyperaldosteronism. Some recommend screening people with high blood pressure who are at increased risk, while others recommend screening all people with high blood pressure for the disease. Screening is usually done by measuring the aldosterone-to-renin ratio in the blood (ARR) whilst off interfering medications and a serum potassium over 4, with further testing used to confirm positive results. While low blood potassium is classically described in primary hyperaldosteronism, this is only present in about a quarter of people. To determine the underlying cause, medical imaging is carried out."}, {"id": "pubmed23n1115_3220", "title": "[Chronic kidney disease after adrenalectomy in a patient with primary aldosteronism].", "score": 0.011482562908041075, "content": "We report one case of estimated glomerular filtration rate (eGFR) decline after taking unilateral adrenalectomy due to aldosterone adenoma. A 60-year-old male with 23-year history of hypertension was reported to the endocrinologist due to hypokalemia (serum potassium 3.01 mmol/L). Urine microalbumin/creatinine (ALB/CR) was 70.15 mg/g, serum creatinine was 82 μmol/L and eGFR was 89.79 mL/(min·1.73 m<sup2</sup). Random serum aldosterone was 172.2-203.5 ng/L, and random plasma rennin activity was 0-0.17 μg/(L·h). His captopril challenge test suggested that his aldosterone le-vels were suppressed by 8% (&lt; 30%) and the adrenal enhanced computed tomography scan revealed a left adrenal tumor. The patient was diagnosed with primary hyperaldosteronism (PA), aldosterone adenoma and underwent left laparoscopic adrenalectomy. Histological examination confirmed adrenal cortical adenoma. One week after the operation, his serum creatinine was increased to 127 μmol/L compared with preoperative level; eGFR was 32.34 mL/(min·1.73 m<sup2</sup). His systolic blood pressure (SBP) was 110 mmHg and diastolic blood pressure (DBP) was 60 mmHg (hypotensive drugs discontinued), and serum potassium level was 5.22 mmol/L. At the end of the 2-year follow up, the serum creatinine of this patient remained at 109-158 μmol/L and eGFR fluctuated from 63.28-40.12 mL/(min·1.73 m<sup2</sup). PA is one of the most common causes of secondary hypertension. Several studies have reported renal function deterioration of PA patients after unilateral adrenalectomy, like the patient in this article. Age, preoperative plasma aldosterone concentration, albuminuria and preoperative potassium level might be significant predictors of a decrease in the eGFR. Growing evidence suggests that aldosterone could contribute to structural kidney damage, arterial injury and hemodynamic disorder. At the same time, patients with PA exhibit glomerular hyperfiltration and glomerular vascular hypertension, leading to the misinterpretation of renal function in PA patients as subtle kidney damage may be masked by the glomerular hyperfiltration before treatment. After a unilateral adrenalectomy, glomerular hyperfiltration by aldosterone excess is resolved and renal damage can be unmasked. In conclusion, kidney function deterioration after adrenalectomy can be detected in some patients with PA. Thus, accurate evaluation of kidney function in patients with PA may be essential, especially for those with preoperative risk factors for postoperative renal impairment. After unilateral adrenalectomy, close monitoring of renal function and adequate management are required for PA patients."}, {"id": "wiki20220301en035_40479", "title": "Amiloride", "score": 0.011329652634000459, "content": "minimal efficacy. For people with resistant hypertension, already taking a thiazide diuretic, an angiotensin converting enzyme inhibitor (ACE-i) or an angiotensin II receptor blocker (ARB), and a calcium channel blocker, the addition of amiloride (or spironolactone) was better at reducing blood pressure than adding a beta-blocker (bisoprolol) or an alpha-1 blocker (doxazosin). When combined with hydrochlorothiazide, the addition of amiloride had positive effects on blood pressure and blood sugar tolerance. Amiloride may therefore be useful for preventing the metabolic side effects of thiazide diuretics, allowing for the use of higher thiazide doses (in line with how they were originally studied)."}, {"id": "wiki20220301en024_713", "title": "Antihypertensive drug", "score": 0.011320915926179084, "content": "Sodium nitroprusside, a very potent, short-acting vasodilator, is most commonly used for the quick, temporary reduction of blood pressure in emergencies (such as malignant hypertension or aortic dissection). Hydralazine and its derivatives are also used in the treatment of severe hypertension, although they should be avoided in emergencies. They are no longer indicated as first-line therapy for high blood pressure due to side effects and safety concerns, but hydralazine remains a drug of choice in gestational hypertension. Renin inhibitors Renin comes one level higher than angiotensin converting enzyme (ACE) in the renin–angiotensin system. Renin inhibitors can therefore effectively reduce hypertension. Aliskiren (developed by Novartis) is a renin inhibitor which has been approved by the U.S. FDA for the treatment of hypertension. Aldosterone receptor antagonist Aldosterone receptor antagonists: eplerenone spironolactone"}, {"id": "wiki20220301en104_37769", "title": "Pseudohyperaldosteronism", "score": 0.011298037275276266, "content": "Treatment Specific treatment of pseudohyperaldosteronism depends on the inciting cause. General management focuses on countering the effects of excess mineralocorticoid activity to achieve adequate blood pressure control and avoid end-organ damage and cardiovascular mortality. In some cases, specific antihypertensive medications may be recommended. In Liddle's syndrome, ENaC-binding potassium-sparing diuretics (e.g. amiloride or triamterene) are used to counter the excess ENaC activity. In AME, the mineralocorticoid receptor-binding potassium-sparing diuretics (e.g. spironolactone or eplerenone) are used to limit aldosterone receptor activity. Other medications such as glucocorticoids are added in AME and CAH to inhibit ACTH and further cortisol production. Lifestyle changes such as a low sodium diet are also used for managing hypertension, and cessation of licorice intake is recommended in cases of licorice overconsumption."}, {"id": "wiki20220301en073_38758", "title": "Hyperaldosteronism", "score": 0.010936431989063567, "content": "Hyperaldosteronism is a medical condition wherein too much aldosterone is produced by the adrenal glands, which can lead to lowered levels of potassium in the blood (hypokalemia) and increased hydrogen ion excretion (alkalosis). This cause of mineralocorticoid excess is primary hyperaldosteronism reflecting excess production of aldosterone by adrenal zona glomerulosa. Bilateral micronodular hyperplasia is more common than unilateral adrenal adenoma. Signs and symptoms It can be asymptomatic, but these symptoms may be present: Fatigue Headache High blood pressure Hypokalemia Hypernatraemia Hypomagnesemia Intermittent or temporary paralysis Muscle spasms Muscle weakness Numbness Polyuria Polydipsia Tingling Metabolic alkalosis Nocturia Blurry Vision Dizziness/Vertigo"}, {"id": "wiki20220301en070_64324", "title": "Secondary hypertension", "score": 0.010758228395952269, "content": "11β-hydroxylase deficiency, aka apparent mineralocorticoid excess syndrome, involves a defect in the gene for 11β-hydroxysteroid dehydrogenase, an enzyme that normally inactivates circulating cortisol to the less-active metabolite cortisone. At high concentrations cortisol can cross-react and activate the mineralocorticoid receptor, leading to aldosterone-like effects in the kidney, causing hypertension. This effect can also be produced by prolonged ingestion of liquorice (which can be of potent strength in liquorice candy), by causing inhibition of the 11β-hydroxysteroid dehydrogenase enzyme and likewise leading to secondary apparent mineralocorticoid excess syndrome. Frequently, if liquorice is the cause of the high blood pressure, a low blood level of potassium will also be present. Cortisol induced hypertension cannot be completely explained by the activity of Cortisol on Aldosterone receptors. Experiments show that treatment with Spironolactone (an inhibitor of the aldosterone"}, {"id": "wiki20220301en026_51136", "title": "Spironolactone", "score": 0.010541566746602718, "content": "The clinical benefits of spironolactone as a diuretic are typically not seen until 2–3 days after dosing begins. Likewise, the maximal antihypertensive effect may not be seen for 2–3 weeks. Unlike with some other diuretics, potassium supplementation should not be administered while taking spironolactone, as this may cause dangerous elevations in serum potassium levels resulting in hyperkalemia and potentially deadly abnormal heart rhythms. High blood pressure About 1 in 100 people with hypertension have elevated levels of aldosterone; in these people, the antihypertensive effect of spironolactone may exceed that of complex combined regimens of other antihypertensives since it targets the primary cause of the elevated blood pressure. However, a Cochrane review found adverse effects at high doses and little effect on blood pressure at low doses in the majority of people with high blood pressure. There is no evidence of person-oriented outcome at any dose in this group."}, {"id": "pubmed23n0571_10703", "title": "The spironolactone, amiloride, losartan, and thiazide (SALT) double-blind crossover trial in patients with low-renin hypertension and elevated aldosterone-renin ratio.", "score": 0.010072679381493586, "content": "There is continuing variation in diagnosis and estimated prevalence of primary hyperaldosteronism. The higher estimates encourage search for adrenal adenomas in patients with elevated ratios of plasma aldosterone to renin. However, it is more likely that patients with normal plasma K+ and aldosterone belong to the polygenic spectrum of low-renin hypertension rather than have the same monogenic syndrome as classic Conn's. Our primary hypothesis was that in low-renin patients with normal plasma K+ and aldosterone, a thiazide diuretic, bendroflumethiazide, would be as effective as spironolactone in overcoming the Na+ retention and lowering blood pressure. Secondary objectives were to compare the dose response for each diuretic and to evaluate amiloride as an alternative to spironolactone. Fifty-seven patients entered and 51 patients completed a placebo-controlled, double-blind, randomized crossover trial. Entry criteria included low plasma renin, normal K+, elevated aldosterone-renin ratio, and a previous systolic blood pressure response to spironolactone of &gt; or = 20 mm Hg. Two doses each of spironolactone and bendroflumethiazide were compared. The crossover also included amiloride and losartan. Outcome measures were blood pressure, plasma renin, and other biochemical markers of diuretic action. Spironolactone 100 mg and bendroflumethiazide 5 mg caused similar falls in systolic blood pressure, whereas bendroflumethiazide 2.5 mg was 5/2 mm Hg less effective in reducing blood pressure than either bendroflumethiazide 5 mg or spironolactone 50 mg (P&lt;0.005). Amiloride 40 mg was as effective as the other diuretics. Biochemical indices of natriuresis showed bendroflumethiazide to be less effective than either spironolactone or amiloride; plasma renin rose 4-fold on spironolactone but only 2-fold on bendroflumethiazide (P=0.003). In hypertensive patients with a low plasma renin but normal K+, bendroflumethiazide 5 mg was as effective as spironolactone 100 mg in lowering blood pressure, despite patients being selected for a previous large fall in blood pressure on spironolactone. Because this result differs from that expected in primary hyperaldosteronism, our finding argues against low-renin hypertension including a large, undiagnosed pool of primary hyperaldosteronism. However, spironolactone was the more effective natriuretic agent, suggesting that inappropriate aldosterone release or response may still contribute to the Na+ retention of low-renin hypertension."}, {"id": "pubmed23n0712_10216", "title": "Mineralocorticoid hypertension.", "score": 0.009900990099009901, "content": "Hypertension affects about 10 - 25% of the population and is an important risk factor for cardiovascular and renal disease. The renin-angiotensin system is frequently implicated in the pathophysiology of hypertension, be it primary or secondary. The prevalence of primary aldosteronism increases with the severity of hypertension, from 2% in patients with grade 1 hypertension to 20% among resistant hypertensives. Mineralcorticoid hypertension includes a spectrum of disorders ranging from renin-producing pathologies (renin-secreting tumors, malignant hypertension, coarctation of aorta), aldosterone-producing pathologies (primary aldosteronism - Conns syndrome, familial hyperaldosteronism 1, 2, and 3), non-aldosterone mineralocorticoid producing pathologies (apparent mineralocorticoid excess syndrome, Liddle syndrome, deoxycorticosterone-secreting tumors, ectopic adrenocorticotropic hormones (ACTH) syndrome, congenitalvadrenal hyperplasia), and drugs with mineraocorticoid activity (locorice, carbenoxole therapy) to glucocorticoid receptor resistance syndromes. Clinical presentation includes hypertension with varying severity, hypokalemia, and alkalosis. Ratio of plasma aldosterone concentraion to plasma renin activity remains the best screening tool. Bilateral adrenal venous sampling is the best diagnostic test coupled with a CT scan. Treatment is either surgical (adrenelectomy) for unilateral adrenal disease versus medical therapy for idiopathic, ambiguous, or bilateral disease. Medical therapy focuses on blood pressure control and correction of hypokalemia using a combination of anti-hypertensives (calcium channel blockers, angiotensin converting enzyme inhibitors, or angiotensin receptor blockers) and potassium-raising therapies (mineralcorticoid receptor antagonist or potassium sparing diuretics). Direct aldosterone synthetase antagonists represent a promising future therapy."}, {"id": "wiki20220301en026_2976", "title": "Congenital adrenal hyperplasia due to 17α-hydroxylase deficiency", "score": 0.009708737864077669, "content": "The adrenal cortex is hyperplastic and overstimulated, with no impairment of the mineralocorticoid pathway. Consequently, levels of DOC, corticosterone, and 18-hydroxycorticosterone are elevated. Although these precursors of aldosterone are weaker mineralocorticoids, the extreme elevations usually provide enough volume expansion, blood pressure elevation, and potassium depletion to suppress renin and aldosterone production. Some persons with 17α-hydroxylase deficiency develop hypertension in infancy, and nearly 90% do so by late childhood. The low-renin hypertension is often accompanied by hypokalemia due to urinary potassium wasting and metabolic alkalosis. These features of mineralocorticoid excess are the major clinical clue distinguishing the more complete 17α-hydroxylase deficiency from the 17,20-lyase deficiency, which only affects the sex steroids. Treatment with glucocorticoid suppresses ACTH, returns mineralocorticoid production toward normal, and lowers blood pressure."}, {"id": "pubmed23n0021_11375", "title": "[Primary hyperaldosteronism. Diagnostic procedure useful in hospital routine].", "score": 0.009523809523809525, "content": "Personal experience in the management of three cases of primary hyperaldosteronism, in which a cure was obtained by surgical removal of an adrenocortical adenoma, is was used in the elaboration of a diagnostic procedure requiring hospitalisation for 12 days. During 6 days, the patient is kept on a diet containing 100 mEq Na and K, and blood potassium values are repeatedly determined. Other causes of hypertension are ruled out. On the 6th day, baselines for blood renin and urinary aldosterone are calculated. Next, a hyposodic diet is given for 4 days, and a diuretic is administered on the last of these days, after which renin is determined \"in response to stimulation\". Lastly, two days of i.v. NaCl loading are followed by the determination of urinary aldosterone \"during inhibition\". If the picture is positive for hyperaldosteronism, the patient is discharged and followed during treatment with spironolactone, and eventually subjected to renal and adrenal arteriography to determine the site of the adenoma. Division of the procedure into increasingly complex steps enables the examination to be halted at any point when evidence in support of the suspected diagnosis fails to appear. This feature, coupled with the simplicity of the procedures adopted, enables all young subjects admitted for unexplained hypertension to be screened for hyperaldosteronism, with the assurance of obtaining certain diagnosis without an excessively long stay in hospital."}, {"id": "wiki20220301en002_196117", "title": "Blood pressure", "score": 0.009512748188706377, "content": "Currently, the RAS is targeted pharmacologically by ACE inhibitors and angiotensin II receptor antagonists, also known as angiotensin receptor blockers (ARBs). The aldosterone system is directly targeted by spironolactone, an aldosterone antagonist. The fluid retention may be targeted by diuretics; the antihypertensive effect of diuretics is due to its effect on blood volume. Generally, the baroreceptor reflex is not targeted in hypertension because if blocked, individuals may suffer from orthostatic hypotension and fainting. Measurement"}, {"id": "pubmed23n0989_678", "title": "Hypertension due to a deoxycorticosterone-secreting adrenal tumour diagnosed during pregnancy.", "score": 0.009433962264150943, "content": "Mineralocorticoid hypertension is most often caused by autonomous overproduction of aldosterone, but excess of other mineralocorticoid precursors can lead to a similar presentation. 11-Deoxycorticosterone (DOC) excess, which can occur in 11-β hydroxylase or 17-α hydroxylase deficiencies, in DOC-producing adrenocortical tumours or in patients taking 11-β hydroxylase inhibitors, may cause mineralocorticoid hypertension. We report a 35-year-old woman who in the third trimester of pregnancy was found to have a large adrenal mass on routine obstetric ultrasound. On referral to our unit, persistent hypertension and long-standing hypokalaemia was noted, despite good compliance with multiple antihypertensives. Ten years earlier, she had hypertension noted in pregnancy which had persisted after delivery. A MRI scan confirmed the presence of a 12 cm adrenal mass and biochemistry revealed high levels of DOC and low/normal renin, aldosterone and dehydroepiandrosterone, with normal catecholamine levels. The patient was treated with antihypertensives until obstetric delivery, following which she underwent an adrenalectomy. Histology confirmed a large adrenal cortical neoplasm of uncertain malignant potential. Postoperatively, blood pressure and serum potassium normalised, and the antihypertensive medication was stopped. Over 10 years of follow-up, she remains asymptomatic with normal DOC measurements. This case should alert clinicians to the possibility of a diagnosis of a DOC-producing adrenal tumours in patients with adrenal nodules and apparent mineralocorticoid hypertension in the presence of low or normal levels of aldosterone. The associated diagnostic and management challenges are discussed. Learning points: Hypermineralocorticoidism is characterised by hypertension, volume expansion and hypokalaemic alkalosis and is most commonly due to overproduction of aldosterone. However, excess of other mineralocorticoid products, such as DOC, lead to the same syndrome but with normal or low aldosterone levels. The differential diagnosis of resistant hypertension with low renin and low/normal aldosterone includes congenital adrenal hyperplasia, syndrome of apparent mineralocorticoid excess, Cushing's syndrome, Liddle's syndrome and 11-deoxycorticosterone-producing tumours. DOC is one intermediate product in the mineralocorticoid synthesis with weaker activity than aldosterone. However, marked DOC excess seen in 11-β hydroxylase or 17-α hydroxylase deficiencies in DOC-producing adrenocortical tumours or in patients taking 11-β hydroxylase inhibitors, may cause mineralocorticoid hypertension. Excessive production of DOC in adrenocortical tumours has been attributed to reduced activity of the enzymes 11-β hydroxylase and 17-α hydroxylase and increased activity of 21-α hydroxylase. The diagnosis of DOC-producing adrenal tumours is challenging because of its rarity and poor availability of DOC laboratory assays."}, {"id": "wiki20220301en086_36538", "title": "Bartter syndrome", "score": 0.009349931823413787, "content": "Bartter syndrome consists of low levels of potassium in the blood, alkalosis, normal to low blood pressures, and elevated plasma renin and aldosterone. Numerous causes of this syndrome probably exist. Diagnostic pointers include high urinary potassium and chloride despite low serum values, increased plasma renin, hyperplasia of the juxtaglomerular apparatus on kidney biopsy, and careful exclusion of diuretic abuse. Excess production of prostaglandins by the kidneys is often found. Magnesium wasting may also occur. Homozygous patients suffer from severe hypercalciuria and nephrocalcinosis."}, {"id": "pubmed23n0708_2505", "title": "Surgical management of primary aldosteronism. not everything that shines is gold.", "score": 0.009345794392523364, "content": "Primary aldosteronism (PA) is a syndrome which includes a group of clinical entities in which aldosterone production is inappropriately high and nonsupressible by sodium loading. The most frequent causes of PA are adrenal adenoma and unilateral or bilateral primary hyperplasia. We report a case of a 55-year-old man with a 10-year history of hypertension in whom functional hormonal studies were indicative of PA. Because adrenal venus sampling was not available at our hospital, the investigation was conducted with a computed tomography (CT) scan and a scan with 131-iodocholesterol (NP-59) which both revealed a left adrenal adenoma. The tumor was excised laparoscopically without any complications and the histological findings confirmed the diagnosis of an aldosterone-producing adenoma. Blood pressure remained normal despite the discontinuation of antihypertensive drugs, further supporting that the adrenal tumor was indeed the cause of high blood pressure. Unfortunately, blood pressure began to rise again 2 months later, and laboratory findings indicated the presence of PA once again. Spironolactone was instituted and blood pressure significantly improved and was finally controlled by the addition of amlodipine. We report this case to underline the difficulties in the discrimination between adenoma and hyperplasia in everyday clinical practice. Although the CT and scintigraphic findings strongly pointed toward an adenoma, the fact that PA re-appeared shortly after the operation, indicated that the underlying cause of the PA was hyperplasia and not adenoma after all."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 219]], "word_ranges": [[0, 27]], "text": "He is describing a textbook Reiter's syndrome: palmo-plantar keratoderma, arthritis and ocular manifestations, together with probably a chlamydial urethritis, perhaps asymptomatic (they do not explain urethral exudate)."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "He is describing a textbook Reiter's syndrome: palmo-plantar keratoderma, arthritis and ocular manifestations, together with probably a chlamydial urethritis, perhaps asymptomatic (they do not explain urethral exudate). Of course, the patient may also have HIV infection or even secondary syphilis (in addition to Reiter's).", "full_answer_no_ref": "He is describing a textbook Reiter's syndrome: palmo-plantar keratoderma, arthritis and ocular manifestations, together with probably a chlamydial urethritis, perhaps asymptomatic (they do not explain urethral exudate). Of course, the patient may also have HIV infection or even secondary syphilis (in addition to Reiter's).", "full_question": "Gustavo comes to the emergency room with skin lesions and general malaise of several days of evolution. He has psoriasiform lesions on the trunk with involvement of palms and soles. He also presents asymmetric non-suppurative joint inflammation and bilateral ocular redness as well as erosions on the glans penis. In the subsequent anamnesis Gustavo recognizes a risky sexual contact 20 days before. What is his diagnosis?", "id": 292, "lang": "en", "options": {"1": "HIV infection.", "2": "Secondary syphilis.", "3": "Reiter's syndrome.", "4": "Erythema multiforme.", "5": NaN}, "question_id_specific": 213, "type": "DERMATOLOGY, VENEREOLOGY AND PLASTIC SURGERY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0992_1246", "title": "Psoriasiform lesions of glans and palms: A rare presentation in secondary syphilis.", "score": 0.018442971273159952, "content": "Erythematous scaly papules on the palms and soles are a common manifestation of secondary syphilis. We report a case of 19-year-old male who presented with erythematous, scaly, psoriasiform lesions over the palms and glans penis. The papules over the palms showed tenderness on blunt vertical pressure. There was a history of sexual contact and ulcer over the glans around 2 months back, which resolved on its own. Venereal Disease Research Laboratory test was positive in 1:32 dilution. <iTreponema pallidum</i hemagglutination test was also positive. This case highlights the atypical presentation of secondary syphilis."}, {"id": "pubmed23n1087_16390", "title": "Unusual clinical manifestation and challenging serological interpretation of syphilis: insights from a case report.", "score": 0.018424611223799865, "content": "The clinical manifestations of recent syphilis can be variable, with typical and atypical patterns. Several conditions may cause atypical clinical aspects, including human immunodeficiency virus (HIV) co-infection. Besides the clinical features, co-infections may completely alter syphilis serological tests, causing interpretative difficulties and diagnostic delays. Aim of the work is to describe the difficulties encountered during the diagnostic evaluation of atypical skin manifestations and of the serology for syphilis of an HIV-infected patient who had contracted it several times. In 2020, a 52-year old HIV-positive bisexual male patient was admitted to our department with a 4-month history of moderately itchy cutaneous lesions localized at his neck, trunk and arms. In 2013, the patient presented with a classic syphilitic roseola of the trunk and a secondary syphilis was diagnosed, with increased levels of rapid plasma reagin (RPR), Treponema pallidum hemagglutination assay (TPHA), anti-Treponema pallidum IgM and IgG Index. A second episode occurred in 2018, as a primary syphilis with multiple ulcerative lesions of the penis, and increased levels of RPR, IgG and IgM. In 2019, a further episode of secondary syphilis was treated with Doxycycline. In 2020, erythematous and papular lesions with vesicular components and urticarial erythema multiforme (EM)-like lesions were present at the neck, trunk and arms. Serological tests and Nucleic Acid Amplification Test (NAAT) for Treponema Pallidum were performed, as well as a cutaneous biopsy with histological and immunohistochemical evaluation of one lesion. NAAT was negative for T. pallidum. Serological test results were discordant with a new syphilis infection, showing only increased levels of RPR and anti-Treponema IgG. The cutaneous biopsy revealed a non specific histological pattern, while the immunohistochemical evaluation with anti-spirochetal antibodies was mandatory for the diagnosis of recent syphilis, showing clusters of rod-shaped elements, some of which with spiral form, focally present at the epidermis and adnexal structures. Nowadays, syphilis may present with atypical clinical and serological features. Physicians should be aware of these possible alterations and consider syphilis even in case of uncommon clinical aspect and unclear serological tests. Cutaneous biopsy and immunohistochemical exam may be mandatory for the diagnosis."}, {"id": "pubmed23n0637_6431", "title": "Resurgence of syphilis: a diagnosis based on unusual oral mucosa lesions.", "score": 0.017543859649122806, "content": "Known as \"the great imitator,\" secondary syphilis may clinically manifest itself in myriad of ways, involving different organs (including the oral cavity), and mimicking, both clinically and histologically, several diseases, thereby making diagnosis a challenge for clinicians. We highlight an interesting case of a 45-year-old man on whose diagnosis of secondary syphilis was based on the presence of unusual oral lesions, consisting of a well delimited, raised, nonhomogeneous, and corrugated white plaque on the right buccal mucosa which mimicked, clinically and histologically, a \"leukoplakia-like\" plaque and several whitish oral mucous patches localized on the lower labial mucosa and the right lateral margin of the tongue. After the oral lesions, the patient developed a symmetric maculopapular cutaneous rash on the palms, soles, and the trunk of the body. Furthermore, during the anamnesis the patient stated an asymptomatic ulcerative lesion on the glans penis, which had appeared 7 months before the onset of the oral lesions and spontaneously disappeared after 2 weeks. The history of these genital and cutaneous lesions suggested performing serologic tests for syphilis, revealing strongly positive titers and leading us to making a diagnosis of secondary syphilis. This case is remarkable because it displays an unusual oral sign, associated with secondary syphilis; in fact, only occasionally does syphilis manifest itself with a \"leukoplakia-like\" plaque. Dentists should consider secondary syphilis in the differential diagnosis of white and/or ulcerative oral lesions, above all in at-risk patients, given the continuing rise of syphilis in western Europe."}, {"id": "pubmed23n0665_6370", "title": "[Syphilis in the context of HIV-infection--a complex disease].", "score": 0.01709773237701949, "content": "A 39-year-old man complained about a slightly reddish non-itching rash evolving on his body during the last few weeks without any general symptoms. Physical examination revealed trunk-dominated roseola, papules, a few nodules with haemorrhagic crust on top, and round hyperkeratotic clavus-like lesion on the left plantar foot. In his mouth, he had a few up to 1.5 cm large erosions, and on his capillitium a diffuse alopecia. Serologic testing for syphilis was positive with Treponema-pallidum-particle-agglutination test at 1:163840, VDRL 1:64, positive IgG-FTA-ABS and 19S-IgM-FTA-ABS tests, and a pleocytosis of the liquor. In addition, there were a co-infection with mycoplasma hominis and first diagnosis of HIV infection (CDC-stadium A1). The patient was diagnosed as having secondary syphilis with suspicion of neurologic involvement and was therefore treated with 6x5 Mio. I.E. Penicillin G i.v. per day for 14 days after the initial application of 40 mg methylprednisolone. Under this regimen complete resolution of the skin lesions was noted over a 4 week period as well as slow re-growth of the hair. HIV infection at stadium A1 did not require antiretroviral treatment. A non-pruritic rash should always point to the differential diagnosis of syphilis. If syphilis is diagnosed, any other sexually transmitted disease including HIV should be excluded as possible co-infection. In case of HIV, neurosyphilis can develop at an earlier stage of common syphilis."}, {"id": "pubmed23n0903_10743", "title": "A Case of Early Neurosyphilis.", "score": 0.016465494620834426, "content": "Neurosyphilis is an infection of the central nervous system by Treponema pallidum, which can occur after the initial syphilis infection. Although commonly associated with late stage disease, patients with early neurosyphilis may present with acute syphilitic meningitis, meningovascular syphilis, or uveitis. A 28 year old man with a past medical history of HIV (CD4 364);, and recent diagnosis of uveitis presented to the Emergency Department with a positive RPR result. His visual acuity had been gradually declining over the past few months. He denied painless or painful ulcerating lesions on his penis, or scrotum, difficulty concentrating, dermatitis on the soles/palms, or difficulty with proprioception. Physical exam was notable for atrophic hyperpigmented polycyclic, annular plaques and patches along the hairline as well as several areas of confluent hyperpigmented polycyclic plaques and nodules on the patient's face, back, left arm, and right posterior leg. Fundoscopic exam revealed bilateral posterior uveitis and chorioretinitis. Evaluation of cerebrospinal fluid revealed a lymphocytic pleocytosis with a positive VDRL and FTA-ABS. Aqueous crystalline penicillin G was initiated for treatment of early neurosyphilis. Within six hours of beginning the infusion, the patient had a documented temperature of 101.8°F, heart rate of 128 beats per minute, blood pressure 142/84, with generalized malaise and headache. Fever and tachycardia resolved over the next 12 hours, with weakness and headache resolving within 1-2 days. His symptom complex was consistent with the Jarisch-Herxheimer reaction. Histopathology of skin biopsy of the back showed perivascular inflammation and rare spirochetes, consistent with secondary syphilis. The patient completed 14 days of aqueous crystalline penicillin G and was discharged after receiving the first of three benzathine penicillin injections. The initial manifestations of syphilis in this patient were posterior uveitis and pruritic skin plaques. His diagnosis should be appropriately classified as secondary syphilis with concomitant symptomatic early neurosyphilis, requiring 14 days of aqueous crystalline penicillin G. This type of presentation is not specific to immunocompromised populations and must be considered even in the general population. Making the diagnosis of early neurosyphilis, regardless of stage, is critical, as it necessitates a longer duration of treatment. Furthermore, clinicians should be reminded of the profound immunologic reaction, Jarisch-Herxheimer, which may occur when treating any treponemal disease."}, {"id": "wiki20220301en001_138382", "title": "Syphilis", "score": 0.016061980347694634, "content": "Primary syphilis is typically acquired by direct sexual contact with the infectious lesions of another person. Approximately 2–6 weeks after contact (with a range of 10–90 days) a skin lesion, called a chancre, appears at the site and this contains infectious spirochetes. This is classically (40% of the time) a single, firm, painless, non-itchy skin ulceration with a clean base and sharp borders approximately 0.3–3.0 cm in size. The lesion may take on almost any form. In the classic form, it evolves from a macule to a papule and finally to an erosion or ulcer. Occasionally, multiple lesions may be present (~40%), with multiple lesions being more common when coinfected with HIV. Lesions may be painful or tender (30%), and they may occur in places other than the genitals (2–7%). The most common location in women is the cervix (44%), the penis in heterosexual men (99%), and anally and rectally in men who have sex with men (34%). Lymph node enlargement frequently (80%) occurs around the"}, {"id": "pubmed23n0482_11642", "title": "[Syphilis. Clinical aspects of Treponema pallidum infection].", "score": 0.01577384634417914, "content": "Syphilis is a sexually transmitted infection by Treponema pallidum. Without antibiotic treatment syphilis lasts for several decades and may develop up to 4 different clinical stages. Usually, the disease begins with a distinct painless and indurated ulcer at the contact site: the primary chancre. An indolent regional lymph node swelling is usually associated with the syphilitic chancre. After spontaneous healing of the primary lesion and several weeks of latency, the clinical symptoms of secondary syphilis occur. Treponema pallidum bacteremia leads to common symptoms like fever and malaise, but also to a generalized lymphadenopathy, and a broad variety of lesions of the skin and mucosal membranes. Non-pruritic transient exanthems often involving palms and soles, condylomata lata, and a specific angina with mucous patches of the oral cavity are prominent signs. After several relapses, which are characterized by a decreasing intensity of clinical symptoms, secondary syphilis then resolves spontaneously. A second period of latency follows, lasting 3-12 years. Then the outcome of untreated syphilis becomes apparent: spontaneous healing by elimination/inactivation of the spirochetes (75%) or transition to tertiary syphilis (25%). Two kinds of granulomatous skin reactions are typical for tertiary syphilis: superficial nodular syphilids and gummas. The bones, as well as the cardiovascular and central nervous system, may also be involved. Finally, metasyphilis with severe and sometimes lethal neurological symptoms (tabes dorsalis, progressive paralysis) occurs 10 to 30 years after primary infection. Except for irreversible tissue destruction which occurs prior to therapy, all stages of syphilis can be cured completely."}, {"id": "pubmed23n0886_4049", "title": "Syphilis: an atypical case of sepsis and multiple anogenital lesions in secondary syphilis.", "score": 0.01499974315508296, "content": "The incidence of syphilis has historically been cyclical in nature, often in relation to the rise and fall of public health initiatives directed toward eradication along with social attitudes toward sexual practices. The incidence of syphilis has increased by 15% in the last 6 years in the United States, with similar increases worldwide. Herein, we present an atypical case of syphilis presenting with severe septic shock and multiple anogenital lesions in an immunocompetent host. A 22-year-old male with no significant past medical history presented with fevers, chills, sore throat, diaphoresis, and diarrhea. He was febrile, tachycardic, hypotensive, and unresponsive to fluid resuscitation requiring short-term vasopressor support. Physical exam revealed diffuse lymphadenopathy; lower extremity macular rash involving the soles of the feet; papular non-pustular lesions on the scrotum; and a 0.5 cm non-tender irregular, healing lesion on the shaft of the penis. Laboratory analysis was significant for leukocytosis and elevated creatinine. Serum screening rapid plasma reagin was positive, and further testing revealed a titer of 1:32, with confirmation via fluorescent treponemal antibody absorption test. The patient was diagnosed with secondary syphilis, which was determined to be the underlying etiology of the sepsis as all other serological evaluations were negative. He was treated with penicillin G benzathine 2.4 million units intramuscular and supportive management, with improvement of symptoms. The patient engaged in high-risk sexual behaviors, including prior unprotected sexual contact with males. New research indicates that up to one-third of patients may present with atypical cutaneous manifestations, as demonstrated by this patient. It is important for physicians to familiarize themselves with the varied clinical presentations of syphilis, which include multiple anogenital lesions and tender primary lesions in primary or secondary syphilis."}, {"id": "pubmed23n0518_11638", "title": "With this eruption, there is not a second to lues.", "score": 0.01436335403726708, "content": "A 28-year-old white man presented to the Emergency Department with a 24-hour history of an eruption on his extremities, trunk, and face. The patient was known to be HIV positive with a CD4 count of 527 and a viral load of 20,300. He denied fever, chills, malaise, and headache. His social history was significant for the fact that he was in a monogamous homosexual relationship. He had no recent travel, pet exposures, or sick contacts. Physical examination revealed stable vital signs and no documented fever. A maculopapular eruption was present on his face, trunk, and extremities (Figures 1 and 2). There was no palmar or plantar involvement. He was treated with diphenhydramine and topical 2.5% hydrocortisone and advised to return if his condition did not improve. Twelve days after the initial evaluation, the patient consulted us again due to progression of his dermatitis. He had no additional complaints other than an eruption on both palms but neither sole. (Figure 3). The eruption now demonstrated erythematous pink-red oval macules and papules 1-2 cm in size distributed on his scalp, face, trunk, and arms. A few papules contained fine collarettes of scale. Further questioning revealed that the patient had experienced a tender rectal ulcer 2 months previously. A punch biopsy and rapid plasma reagin were performed. The histopathologic examination revealed interface dermatitis with lymphocytes, plasma cells, occasional neutrophils, and a prominent lymphoplasmacytic perivascular dermatitis with infiltration of the vessel walls. Warthrin-Starry and Steiner methods demonstrated spirochetes at the dermal-epidermal junction and in vessel walls, consistent with Treponema pallidum (Figure 4). Rapid plasma reagin and fluorescent Treponema antibody were both reactive with a Venereal Disease Research Laboratory (VDRL) of 1:16. The patient was diagnosed as having secondary syphilis and treated with 2.4 million units of IM benzathine penicillin for 3 weeks. His eruption resolved after the initial treatment and he did not experience a Jarisch-Herxheimer reaction."}, {"id": "wiki20220301en001_138385", "title": "Syphilis", "score": 0.014248366013071896, "content": "Secondary syphilis occurs approximately four to ten weeks after the primary infection. While secondary disease is known for the many different ways it can manifest, symptoms most commonly involve the skin, mucous membranes, and lymph nodes. There may be a symmetrical, reddish-pink, non-itchy rash on the trunk and extremities, including the palms and soles. The rash may become maculopapular or pustular. It may form flat, broad, whitish, wart-like lesions on mucous membranes, known as condyloma latum. All of these lesions harbor bacteria and are infectious. Other symptoms may include fever, sore throat, malaise, weight loss, hair loss, and headache. Rare manifestations include liver inflammation, kidney disease, joint inflammation, periostitis, inflammation of the optic nerve, uveitis, and interstitial keratitis. The acute symptoms usually resolve after three to six weeks; about 25% of people may present with a recurrence of secondary symptoms. Many people who present with secondary"}, {"id": "pubmed23n0819_11284", "title": "[Clinical and paraclinical features of syphilitic uveitis].", "score": 0.014200163324562198, "content": "Syphilis, caused by Treponema pallidum agent, results in polymorphic and non-specific ocular manifestations. Early diagnosis and institution of individualized treatment play a large role in the prognosis. The increase in syphilis over the past several years requires the ophthalmologist to consider this diagnosis in the setting of any intraocular inflammatory involvement. To describe epidemiological, clinical and paraclinical features and natural history of syphilitic uveitis. Retrospective, descriptive and non-comparative study of a series of patients hospitalized between 2007 and 2013 in our department of ophthalmology for management of ocular inflammation associated with a positive syphilitic serology. Thirteen patients of mean age 52.5 years ± 12.9 (33-82 years) were included. All were male and were followed for six months. Co-infection with human immunodeficiency virus (HIV) was present in four of them. Other risk factors discovered on history were unprotected sexual relations, multiple partners, homosexual relations, co-infection with another sexually transmitted disease (STD) or an occupational risk. Decreased visual acuity (VA) was present in all patients, with an average initial VA of 0.71 ± 0.81 LogMAR, i.e. 2/10. Involvement was bilateral in 38% (n=5) of cases. Papilledema was present in 10 patients. Seven patients exhibited vasculitis, 6 patients a necrotizing retinitis, 2 patients with placoid lesions, 7 patients with panuveitis and 2 patients with macular edema. We did not find any patients with isolated anterior uveitis. Three patients exhibited concomitant extraocular involvement with cutaneous palmoplantar lesions. Spectral domain optical coherence tomography (SD-OCT) found a fragmentation of the external limiting membrane and a disorganization of the ellipsoid line in two patients. Cerebrospinal fluid was studied for all patients. Eight of them exhibited lymphocytic meningitis, and we found the presence of anti-Treponema pallidum hemagglutination assay antibody (TPHA) in 9 patients and anti-veneral disease research laboratory antibody (VDRL) in 1 patient. Syphilis polymerase chain reaction (PCR) in the aqueous humor was positive in 50% (n=6) of studied cases and the PCR for Epstein Barr virus came back positive in four specimens out of eight. False positive reactions were observed for Lyme disease in eight patients. The four HIV-positive patients showed bilateral lesions more frequently, but less severe and with a favorable outcome. Antibiotic treatment with ceftriaxone (2 grams per day intramuscularly for 15 to 21 days) and local treatment (corticoids and mydriatics) in the case of inflammation of the anterior segment, allowed a regression of the inflammation in all of our patients as well as an improvement in VA (average final VA 0.09 ± 0.17 LogMAR, i.e. approximately 8/10). One Jarisch Herxheimer reaction occurred and was resolved with systemic corticosteroid therapy. A change in the retinal pigment epithelium was the main sequela in 44% of cases (n=8 eyes). Every structure of the eye may be involved with syphilis; therefore, syphilis must be systematically sought during the etiologic assessment of ocular inflammation even in the absence of historical risk factors. HIV-positive patients must be handled in the same way as immunocompetent patients. Collaboration with the internist is essential for the diagnosis, monitoring, and staging, especially in search of neurosyphilis. The clinical course is favorable with early treatment."}, {"id": "pubmed23n0879_10194", "title": "Immune reconstitution inflammatory syndrome associated with secondary syphilis.", "score": 0.013315405968077725, "content": "Immune reconstitution inflammatory syndrome (IRIS) is a condition associated with paradoxical worsening and/or new onset of an opportunistic infection in HIV patients following the initiation of anti-retroviral therapy or switching to more potent antiretroviral therapy (ART) regimen. Although IRIS associated with many opportunistic infections (OIs) has been well reported, syphilis has very rarely been mentioned in this regard. A 52-year-old male, diagnosed with AIDS six weeks ago, presented with the disseminated non-pruritic painless skin rash. He denied any fever, cough, shortness of breath, and joint pain or swelling. The patient had no similar symptoms, genital ulcers, or any medical illness in the past. CD4 cell count and viral load were 40 cells/mm<sup3</sup and 280,000 copies/ml, respectively, while screening tests for OIs including rapid plasma reagin test, quantiferon, cryptococcal antigen, and toxoplasma tests were negative at the time of HIV diagnosis. After three days of initiation of anti-retroviral therapy, he developed the above-mentioned skin rash. Repeat rapid plasma regain (RPR) test at this time was also negative. Punch biopsy of the skin lesion demonstrated findings suggestive of secondary syphilitic lesions, which was confirmed by immunostain. The repeat RPR, CD4 cell count, and viral load showed a titer of 1:256, 257 cells/mm<sup3</sup, and 5000 copies/ml, respectively. His skin rashes faded away, and RPR titer trended down on treatment with benzathine penicillin without discontinuation of ART. The presence of an IRIS response does not predict overall HIV or OI treatment responses, and discontinuation of ART is not generally recommended as the benefits of treating HIV infection outweighs the risk associated with IRIS."}, {"id": "pubmed23n0823_24556", "title": "Lues maligna praecox: an important consideration in HIV-positive patients with ulceronodular skin lesions.", "score": 0.011408441600413677, "content": "Syphilis is commonly known as \"the great imitator\" owing to its varied clinical manifestations. Secondary syphilis has a variety of presentations, with the most common manifesting as a diffuse papulosquamous eruption on the palms and soles. Lues maligna praecox is a rare form of secondary syphilis, with severe constitutional symptoms, seen primarily in HIV-positive individuals. We report an atypical case of suspected lues maligna in a 45-year-old male. The patient was HIV-positive with a CD4 count of 441. He presented to our clinic with large painful gummatous ulcers in the groin and lower back. He also reported daily fevers, night sweats, and weight loss consistent with secondary syphilis. Prior to this episode the patient had a history of acute active syphilis (RPR 1:128) in 2012 treated at that time with a single dose of 2.4 million units intramuscular benzathine penicillin; he had no reported exposures since that time. The patient was treated with three weekly doses of benzathine penicillin, 2.4 million units, given intramuscularly. This case demonstrates the importance of recognizing the varied clinical presentation of secondary syphilis and keeping lues maligna in consideration for ulceronodular skin lesions in patients who are HIV-positive."}, {"id": "pubmed23n0767_17819", "title": "Vesicular syphilid in a seropositive patient.", "score": 0.011261685174728652, "content": "Syphilis is a sexually transmitted infection with various stages of evolution and a myriad of presentations. To avoid a delay in diagnosis, it is important to recognize secondary syphilis presenting with vesicular lesions. A patient presented with maculopapular rash of recent onset with several vesicles and related the eruption to paracetamol taken one day before. The differential diagnoses considered were drug eruption, pityriasis lichenoides et varioliformis acuta, pityriasis rosea and secondary syphilis. HIV, VDRL (1:256) and TPHA tests were positive and histopathology revealed lymphohistiocytic infiltrate and plasma cells. Thus, a diagnosis of secondary syphilis coexisting with HIV was confirmed. The patient was administered benzathine penicillin and anti-retroviral therapy was started. He responded very well to treatment. We report this case because of the rarity of vesicular eruption in secondary syphilis. "}, {"id": "pubmed23n0573_20778", "title": "[A case of secondary syphilis with hepatitis].", "score": 0.01120879120879121, "content": "Hepatitis is a rare clinical manifestation of syphilis. In this report a 50 years old male patient who was diagnosed as secondary syphilis presenting with hepatitis has been discussed. The patient was admitted to the hospital with high fever and skin rash, and his history revealed a suspected sexual contact. He indicated that he had been admitted to a health center eight months ago because of the presence of a penile wound, however VDRL (Venereal Disease Research Laboratory) test was negative at that time. Fever (39.5 degrees C), jaundice in skin and sclera, generalized macular and maculopapular skin rash including palms and soles, lymphadenopathy and hepatosplenomegaly were detected in physical examination. Laboratory tests yielded elevated erythrocyte sedimantation rate, high CRP levels and elevated liver enzyme levels, however viral hepatitis markers together with VDRL and TPHA (Treponema pallidum hemagglutination) tests were found negative. Ceftriaxone therapy was initiated because of the presence of high fever (40 degrees C) and 30 leukocyte/mm3 in urine, and the absence of bacteria in Gram staining of urine sample. However, the antibiotic therapy was discontinued since fever persisted. As the clinical signs and symptoms strongly indicated syphilis, the serological tests were repeated and VDRL positivity at 1/8 and TPHA positivity at 1/1280 titers were detected. Ceftriaxone therapy was restarted and continued for 14 days with complete cure. Since the spouse of the patient was also found VDRL and TPHA positive, she was treated with penicilin. The presentation of this case emphasized the importance of repeating the serological tests for syphilis since they might be negative in the early stages of infection. The case also indicates that syphilis should be considered in the differential diagnosis of hepatitis."}, {"id": "pubmed23n1114_24067", "title": "A case of primary and secondary syphilis presenting together as immune reconstitution inflammatory syndrome.", "score": 0.011048056025114706, "content": "Immune reconstitution inflammatory syndrome (IRIS) is a condition during the clinical course of HIV infection in which there is paradoxical worsening and/or new onset of opportunistic infections in a HIV-positive patient who has recently been started on anti-retroviral therapy (ART). We present a case of AIDS with CD4 count of 20 cells/μl who presented within 6 weeks of starting ART with a CD4 count of 160 cells/μl and a painless solitary genital ulcer along with annular dark-colored plaques over soles. His screening test for syphilis was negative both during baseline evaluation, prior to initiation of ART, and during his clinical presentation. His disease was confirmed based on a positive treponema pallidum hemagglutination test report and a suggestive skin biopsy. He responded well to three doses of Benzathine Penicillin and continuation of ART. There are very few case reports of syphilis presenting as IRIS and this case is all the more unique as he had features of both primary and secondary syphilis occurring together within 6 weeks of starting ART. This report would reiterate the fact that syphilis and HIV co-infection can alter the natural course of both the diseases and a high index of suspicion is required for treating them."}, {"id": "InternalMed_Harrison_13694", "title": "InternalMed_Harrison", "score": 0.010983813327727559, "content": "Secondary Syphilis The protean manifestations of the secondary stage usually include mucocutaneous lesions and generalized nontender lymphadenopathy. The healing primary chancre may still be present in ∼15% of cases, and the stages may overlap more frequently in persons with concurrent HIV infection. The skin rash consists of macular, papular, papulosquamous, and occasionally pustular syphilides; often more than one form is present simultaneously. The eruption may be very subtle, and 25% of patients with a discernible rash may be unaware that they have dermatologic manifestations. Initial lesions are pale red or pink, nonpruritic, discrete macules distributed on the trunk and proximal extremities; these macules progress to papular lesions that are distributed widely and that frequently involve the palms and soles (Fig. 206-3; see also Figs. 25e-18 and 25e-19). Rarely, severe necrotic lesions (lues maligna) may appear; they are more commonly reported in HIV-infected individuals."}, {"id": "pubmed23n0960_4444", "title": "Secondary syphilis masquerading as lupus vulgaris in an HIV-infected patient: A diagnosis suggested by histology.", "score": 0.01055167829843802, "content": "We report a case of secondary syphilis mimicking lupus vulgaris in an HIV-infected patient. A 21-year-old Brazilian man presented with a two-month history of asymptomatic cutaneous lesions accompanied by fever and fatigue. Dermatological evaluation revealed an erythematous, crusted, large plaque on the neck with the 'apple jelly' sign on diascopy and two smaller scaly elements on the trunk and left palm. Bacteriological examinations for bacteria and mycobacteria gave negative results. Histology revealed psoriasiform epidermal hyperplasia and dermal lymphoplasmacytic infiltrate. Serology for syphilis was positive, and immunohistochemistry confirmed the presence of Treponema pallidum in lesional skin. A diagnosis of secondary syphilis was made, and the patient was successfully treated with benzathine penicillin G. Cutaneous manifestations of secondary syphilis are protean and skin tuberculosis may be considered in the differential diagnosis, especially in HIV-infected patients. In the current case, clinical examination, and particularly, 'apple jelly' sign positivity, was suggestive of lupus vulgaris, but only typical histopathology and immunohistochemistry led to the correct diagnosis of secondary syphilis."}, {"id": "pubmed23n1155_24722", "title": "Annular and Psoriasiform Secondary Syphilis in a Nine-Year-Old Girl Child: A Case Report.", "score": 0.009900990099009901, "content": "Syphilis, a sexually transmitted infection, may pose a challenge to diagnosis if it presents in an unusual form and in rare areas of the body. Non-typical lesions such as annular, maligna, nodular, nodular-ulcerative, corymbiform, leukoderma, pustular, berry-like, and chancriform presentations comprise about 29.6% of the skin manifestations of secondary syphilis. Although typical secondary syphilis is usually not associated with pruritus, 42% of secondary syphilis patients experience itching. A less frequently seen subtype of secondary syphilis is annular secondary syphilis. Its prevalence is approximately 5.7-13.6%. It occurs more commonly in children and people with dark skin. The location is mainly on the cheeks, frequently near the angle of the mouth. In rare cases, it can occur over the penis, feet, and legs. Syphilis in children is a very rare condition as children are seldom sexually active. Infection can occur through either close contact such as kissing, breastfeeding, vertical transmission, or secondary to abuse. We report a rare case of secondary syphilis having psoriasiform as well as annular lesions manifesting mainly on the palms and soles along with generalized lymphadenopathy in a nine-year-old girl. No evidence of hepatosplenomegaly, icterus, or anemia was seen clinically as well as on sonography. <iTreponema pallidum</i hemagglutination test was strongly positive. Venereal disease research laboratory test showed a titer of 1:128. Hepatitis B and HIV surface antigen tests were negative. Based on clinical and serological findings, the patient was diagnosed with secondary syphilis, having annular as well as psoriasiform lesions. The patient received tablets of azithromycin 250 mg on the first day. Because of gastritis, the patient was shifted to doxycycline 50 mg twice a day for 14 days. The skin lesions subsided completely after 10 days."}, {"id": "pubmed23n0660_16545", "title": "[Clinical presentation of syphilis].", "score": 0.009900990099009901, "content": "Since the turn of the millennium, the incidence of syphilis is on the rise in France and in other developed countries. The majority of syphilis cases currently occur in men having sex with men and half of the cases occur in HIV-positive patients. Multiple partners and non protected intercourses are frequently reported. About 80% of syphilis recently diagnosed in France are symptomatic and correspond to primary or secondary syphilis. The other potential circumstances of diagnosis of syphilis include the presence of risk factors, an intercourse with an infected partner, the serological follow-up of a previous syphilis and a systematic screening during pregnancy. Clinical features are mainly represented by skin and mucosal lesions. However, extra-cutaneous involvement and biological abnormalities are quite frequent during secondary syphilis especially ophthalmic complications which are source of sequelae due to the delay for diagnosis. In the post-HAART era, it seems that clinical presentation and serological response after treatment is similar in HIV infected patients in comparison to HIV uninfected patients and that patients with early syphilis shoud be treated with one dose of benzathine penicillin G while the unique treatment for neurosyphilis is intravenous penicilline G."}, {"id": "InternalMed_Harrison_4040", "title": "InternalMed_Harrison", "score": 0.009880287677213299, "content": "In secondary syphilis, there are scattered red-brown papules with thin scale. The eruption often involves the palms and soles and can resemble pityriasis rosea. Associated findings are helpful in making the diagnosis and include annular plaques on the face, nonscarring alopecia, condyloma lata (broad-based and moist), and mucous patches as well as lymphadenopathy, malaise, fever, headache, and myalgias. The interval between the primary chancre and the secondary stage is usually 4–8 weeks, and spontaneous resolution without appropriate therapy occurs. SELECTED CAuSES of PAPuLoSquAMouS SKin LESionS 1. Primary cutaneous disorders a. b. c. d. e. Parapsoriasis, small plaque and large plaque f. 2. 3. a. Lupus erythematosus, primarily subacute or chronic (discoid) lesionsc b. Cutaneous T cell lymphoma, in particular, mycosis fungoidesd c. d. Reactive arthritis (formerly known as Reiter's syndrome) e."}, {"id": "pubmed23n0697_18550", "title": "An unusual cutaneous manifestation of Crohn's disease.", "score": 0.009615384615384616, "content": "A 61-year-old man with a 12-year history of quiescent Crohn's disease on mesalamine presented to his gastroenterologist in April 2009, complaining of abdominal cramping, diarrhea, and a 25-lb weight loss over 6 weeks. He did not respond to prednisone 50 mg and 6-mercaptopurine 100 mg daily. Abdominal computed tomography findings revealed diffuse submucosal edema consistent with extensive colitis. Colonoscopy demonstrated diffuse inflammation with erythema, friability, and shallow ulcerations in the rectum and colon. Biopsies were consistent with Crohn's colitis. He was admitted for infliximab infusion for his unremitting diarrhea. Five days before admission, the patient noted mild swelling and redness of the left lower eyelid, which progressed to involve the right lower eyelid with frank pus draining from both eyes. He had no visual impairment or eye pain. Two days before admission, an ophthalmologist prescribed a steroid eyedrop with no relief. He also complained of seropurulent painful skin lesions on his face and scalp, which spread to involve his upper trunk and proximal arms. On admission to the hospital, dermatology, ophthalmology, and infectious disease consultations were obtained to rule out disseminated infection before initiation of infliximab therapy. The patient was afebrile and hemodynamically stable. His oral mucosa was normal. He had prominent bilateral lower eyelid edema, erythema, and superficial erosions with hemorrhagic crusting and frank green purulent drainage from both eyes, with crusting along the lower lash line and bilateral sclera injection (Figure 1). On his scalp, face, trunk, and proximal extremities, he had 25 to 30 erythematous, 4- to 8-mm papulopustules with narrow red halos, some with central necrosis and crusting (Figure 2). Cultures from the purulent ocular drainage and pustules on the trunk and arms were all negative for bacteria, virus, and fungi. Gram stain from the eye drainage showed polymorphonuclear leukocytes without organisms. Tissue cultures were negative for bacterial, fungal, and mycobacterial infection. Skin biopsy taken from the central upper back demonstrated subcorneal pustules with areas of eroded epidermis and collections of neutrophils in the superficial dermis (Figure 3). Special stains were negative for organisms. He received infliximab infusion 5 mg/kg for a total dose of 420 mg over 2 hours. Within 48 hours of infusion, there was notable decrease in size of lesions, in addition to reduction of purulent drainage from both eyes. The patient was discharged home following infliximab infusion. His skin lesions resolved during a period of 2 weeks, leaving small pink atrophic scars. He received his second infusion of infliximab 2 weeks after discharge with continued improvement in his gastrointestinal symptoms."}, {"id": "pubmed23n1059_12612", "title": "Secondary Syphilis.", "score": 0.009615384615384616, "content": "A 40-year-old male presented the the emergency department (ED) due to a diffuse body rash after a sexual encounter. Examination revealed a maculopapular rash that included the palms and soles of the feet bilaterally. A rapid plasma reagin was positive, and the patient was treated with 2.4 million units of benzathine benzylpenicillin intramuscularly. Secondary syphilis can mimic many disease processes but classically presents as a painless macular rash on the palms of the hands and soles of the feet. Diagnosis is based upon clinical examination coupled with serological testing. Emergency department management should include 2.4 million units of benzathine benzylpenicillin intramuscularly and mitigation strategies."}, {"id": "pubmed23n0935_18190", "title": "A secondary syphilis rash with scaly target lesions.", "score": 0.009523809523809525, "content": "A 40-year-old man reported a 5-day history of fever and malaise, followed by a pruritic generalized rash. He had well-demarcated erythematous papules and plaques with scaling. The patient was diagnosed with secondary syphilis. The skin biopsy showed a psoriasiform lichenoid dermatitis with plasma cells. The anti-T. pallidum antibody confirmed the presence of spirochetes. He was also found to be hepatitis C virus and human immunodeficiency virus positive. The characteristic rash of secondary syphilis may appear as maculopapular, evolving initially from macules to small reddish-brown papules with minor scaling later. When the scaling is prominent, lesions can be difficult to differentiate from guttate psoriasis. Typical target lesions are most often associated with erythema multiforme, but they can rarely occur in secondary and congenital syphilis. Syphilis should be suspected in high-risk patients presenting a variety of atypical syndromes such as neurologic symptoms, uveitis or cholestatic hepatitis, especially if palmoplantar lesions are present."}, {"id": "pubmed23n0546_17380", "title": "[Fever and exanthema as manifestations of the second stage of syphilis].", "score": 0.009523809523809525, "content": "A man aged 30 had been suffering from episodes of fever for several weeks. He had diarrhoea and had developed generalized maculopapular exanthema that also affected the palms of his hands and soles of his feet. After viral causes were excluded the symptoms proved to be caused by syphilis. His condition was complicated by uveitis. The patient recovered after a single dose of benzyl penicillin and local mydratics and corticosteroid eye drops. The incidence of syphilis is rising and its clinical spectrum is broad. Therefore in patients with fever and exanthema of unknown origin this disease should be considered. One should be aware of the wide variety of complications that can result from syphilis."}, {"id": "pubmed23n0039_12324", "title": "Secondary syphilis: a clinico-pathological review.", "score": 0.009433962264150943, "content": "The histological appearances found in biopsies from fifty-seven patients with secondary syphilis have been correlated with the clinical morphology of the eruptions. Considerable variation of histological pattern was encountered, and the frequency with which some of the classically described changes were found to be absent or inconspicuous is stressed. Of particular interest were the findings that, in nearly one-quarter of the biopsies, plasma cell infiltration was either absent or very sparse, and that vascular damage was seen in less than half. Where present, the vessel changes were almost entirely confined to swelling of the endothelial cells. Proliferation of the endothelial cells was most uncommon. The epidermis was very frequently involved in the inflammatory process. Exocytosis, spongiosis, parakeratosis, and acanthosis were the most frequent changes. No consistent histological difference between papular and papulo-squamous lesions could be found but macular lesions demonstrated more superficial and less intense dermal infiltration as well as less severe epidermal involvement. In late secondary lesions, the infiltrate became granulomatous, but in other respects the duration of the exanthem could not be correlated with the pathology. The differential diagnosis from pityriasis lichenoides and other inflammatory dermatoses is discussed and the value of histopathology in the diagnosis of secondary syphilis is emphasized."}, {"id": "pubmed23n0343_17329", "title": "Two recent cases of tertiary syphilis.", "score": 0.009259259259259259, "content": "Tertiary syphilis is now a rare disease in Europe, mainly as a result of occasional antibiotherapy for concomitant infections. However early syphilis is rising in USA and Germany, and it is necessary to maintain an high level of knowledge and suspicion to achieve a diagnosis in the tertiary stage of the disease. In this report two patients with benign tertiary syphilis are described. The first one is a 55-year-old female with erythemato-violaceous annular scaling plaques on the right buttock and scapula and on both thighs, which had a negative and then a low VDRL titer. The second case is a 33-year-old mentally handicapped female with erythematous plaques, with psoriasiform scaling in the trunk and well defined crusted ulcers on the face, which also had negative VDRL. Biopsy of the skin lesions revealed plasmocytic infiltrate with endothelial swelling without granulomas and with negative silver stains in both patients. The investigation for cardiovascular and neurological involvement was negative in both patients. Diagnosis of tertiary syphilis can be difficult as clinical pictures can be misleading, similar to other granulomatous diseases, and serological titers can be low or negative. We recall the necessity of ruling out neurological and cardiac involvement in this stage of syphilis. These cases are reported as a reminder of the possibility of syphilis, so that new cases are not misdiagnosed and mistreated as other diseases."}, {"id": "pubmed23n1158_18232", "title": "Case of Secondary Syphilis with Mucocutaneous, Articular, and Pulmonary Involvement in a 74-Year-Old Moroccan Man.", "score": 0.009174311926605505, "content": "BACKGROUND Syphilis is a sexually transmitted infection (STI) caused by Treponema pallidum. If untreated, primary syphilis can progress to secondary syphilis, which has a characteristic rash and diverse systemic features. This report is of a case of secondary syphilis with mucocutaneous, articular, and pulmonary involvement. CASE REPORT A 74-year-old Moroccan man presented with an 8-week history of bilateral knee pain and swelling. On examination, he had bilateral knee effusions. Articular puncture brought an inflammatory fluid with a significant presence of white blood cells. Inflammatory markers were elevated. X-rays of both knees showed bilateral osteoarthritis with intra-articular calcification in the left knee. Nonsteroidal anti-inflammatory drugs and colchicine were prescribed, but were ineffective. A closer clinical examination of the patient revealed pigmented papules on the palms, soles, oral mucosa, trunk, and genitals. Treponema pallidum hemagglutination assay and Venereal Disease Research Laboratory results were positive in the blood (titers 1: 32) and joint fluid. A computed tomography scan of the chest revealed a focal opacity in the lateral basal segment of the right lung. The diagnosis of secondary syphilis with mucocutaneous, articular, and pulmonary involvement was made. The evolution was favorable after a single intramuscular injection of benzathine-penicillin. CONCLUSIONS Arthritis, mucocutaneous involvement, and lung lesions can be manifestations of secondary syphilis. A detailed anamnesis, clinical examination, serology, and imaging techniques are the pillars of diagnosing this condition."}, {"id": "pubmed23n0345_20190", "title": "Two recent cases of tertiary syphilis.", "score": 0.009174311926605505, "content": "Tertiary syphilis is now a rare disease in Europe, mainly as a result of occasional antibiotherapy for concomitant infections. However early syphilis is rising in USA and Germany, and it is necessary to maintain an high level of knowledge and suspicion to achieve a diagnosis in the tertiary stage of the disease. In this report two patients with benign tertiary syphilis are described. The first one is a 55-year-old female with erythemato-violaceous annular scaling plaques on the right buttock and scapula and on both thighs, which had a negative and then a low VDRL titer. The second case is a 33-year-old mentally handicapped female with erythematous plaques, with psoriasiform scaling in the trunk and well defined crusted ulcers on the face, which also had negative VDRL. Biopsy of the skin lesions revealed plasmocytic infiltrate with endothelial swelling without granulomas and with negative silver stains in both patients. The investigation for cardiovascular and neurological involvement was negative in both patients. Diagnosis of tertiary syphilis can be difficult as clinical pictures can be misleading, similar to other granulomatous diseases, and serological titers can be low or negative. We recall the necessity of ruling out neurological and cardiac involvement in this stage of syphilis. These cases are reported as a reminder of the possibility of syphilis, so that new cases are not misdiagnosed and mistreated as other diseases."}, {"id": "InternalMed_Harrison_13704", "title": "InternalMed_Harrison", "score": 0.009154942071926618, "content": "FIGuRE 206-3 Secondary syphilis. Left: Maculopapular truncal eruption. Middle: Papules on the are uncertain, it may be appropriate palms. Right: Papules on the soles. (Courtesy of Jill McKenzie and Christina Marra.) to conclude that even patients with 1136 early syphilis who have such findings do indeed have asymptomatic neurosyphilis and should be treated for neurosyphilis; such treatment is particularly important in patients with concurrent HIV infection. Before the advent of penicillin, the risk of development of clinical neurosyphilis in untreated asymptomatic persons was roughly proportional to the intensity of CSF changes, with the overall cumulative probability of progression to clinical neurosyphilis ∼20% in the first 10 years but increasing with time. Most experts agree that neurosyphilis is more common in HIV-infected persons, while immunocompetent patients with untreated latent syphilis and normal CSF probably run a very low risk of subsequent neurosyphilis. In several"}, {"id": "pubmed23n1146_8911", "title": "Concurrent coronavirus disease 2019 and primary syphilis in a young man: A rare case report.", "score": 0.00909090909090909, "content": "The global rise of syphilis infections and the ongoing coronavirus disease 2019 (COVID-19) pandemic are causes for concern. We herein report a rare case of concurrent primary syphilis and COVID-19. A 29-year-old man was admitted with a diagnosis of COVID-19. Although COVID-19 pneumonia appeared during ciclesonide and favipiravir treatment, his symptoms improved without developing severe hypoxemia. A small, red ulcer on the left-side of his glans penis was noted and left inguinal lymph node swellings were detected on computed tomography (CT). He reported that his last engagement in sexual intercourse had been 3 months previously, and that his partner had subsequently been diagnosed with syphilis. Although both serum Treponema pallidum (TP) antibody and rapid plasma reagin (RPR) quantitative tests were negative on the day of admission, we clinically diagnosed a suspected case of primary syphilis and started treatment with amoxicillin (1500 mg/day). We subsequently learned that the TP antibody and RPR quantitative tests had been positive 4 days before starting syphilis treatment. Amoxicillin treatment was continued for 61 days, and the ulcer gradually improved. One year later, the RPR quantitative test was negative, and CT revealed a reduction in size of the inguinal lymph nodes and no residual signs of COVID-19 pneumonia. The prevalence of syphilis has been increasing even during the COVID-19 pandemic, and the incidence of concurrent syphilis and COVID-19 might be higher than is recognized. Asking patients with COVID-19 about high-risk sexual behavior and genital lesions could help with early diagnosis of syphilis."}, {"id": "pubmed23n0945_15042", "title": "A 30-Year-Old Man with HIV, Fever, and a Rash.", "score": 0.00909090909090909, "content": "Patients who present with papular rashes have a wide differential diagnosis particularly in the setting of immune compromise. A 30-year-old male diagnosed with HIV since 2009, never on antiretroviral therapy, with a nadir CD4 count of 333 cells/mm<sup3</sup and a current viral load of 44,300 copies/mL, presented with a diffuse monomorphic papular eruption that began on his trunk and extremities and subsequently spread to the penis and scrotum, sparing the distal acral sites. A thorough infectious workup revealed a positive rapid plasma reagin (RPR) and varicella IgM and IgG antibodies. Interestingly, the patient had been diagnosed and treated for syphilis in the past with a recent downtrending RPR drawn prior to hospitalization. Repeat RPR was elevated and a preliminary histopathology report demonstrated folliculocentric inflammation with lymphocytes, plasma cells, and polymorphonuclear leukocyte predominance supported the diagnosis of syphilis. After receiving intramuscular penicillin G benzathine, he developed intermittent fevers and new papules. Intravenous (IV) acyclovir was initiated for presumed disseminated varicella given his positive varicella-zoster virus IgM and IgG. However, final pathology results revealed a large spirochete burden. The fevers and rash progression were attributed to the development of a Jarisch-Herxheimer reaction. IV acyclovir was discontinued and he completed a course of intramuscular penicillin G benzathine. He was also given a course of doxycycline for rectal chlamydia which was diagnosed during hospitalization."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[193, 503]], "word_ranges": [[33, 78]], "text": "an unstable thorax due to multiple rib fractures (costal volet) would cause progressive hypoventilation with atelectasis of the pulmonary parenchyma, which would progress on the one hand to hypercapnia and respiratory acidosis, and on the other, to later hypoxemia due to infection associated with atelectasis."}, "2": {"exist": true, "char_ranges": [[504, 757]], "word_ranges": [[78, 116]], "text": "Respiratory infection due to aspiration would also occur later, and does not appear in all cases of severe chest trauma, only if there has been a decrease in the level of consciousness (associated TBI, uncontrolled intubation with bronchoaspiration...)."}, "3": {"exist": true, "char_ranges": [[0, 161]], "word_ranges": [[0, 27]], "text": "Pulmonary contusion is the most serious lesion and the one with the worst prognosis in thoracic trauma. It is also the injury that causes hypoxemia the earliest."}, "4": {"exist": true, "char_ranges": [[758, 960]], "word_ranges": [[116, 147]], "text": "Post-traumatic hypovolemia (in this case, as there are multiple rib fractures, it could be due to hemothorax) would occur earlier and would be associated in the first place with hemodynamic instability."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Pulmonary contusion is the most serious lesion and the one with the worst prognosis in thoracic trauma. It is also the injury that causes hypoxemia the earliest. Contrary to what we may think, an unstable thorax due to multiple rib fractures (costal volet) would cause progressive hypoventilation with atelectasis of the pulmonary parenchyma, which would progress on the one hand to hypercapnia and respiratory acidosis, and on the other, to later hypoxemia due to infection associated with atelectasis. Respiratory infection due to aspiration would also occur later, and does not appear in all cases of severe chest trauma, only if there has been a decrease in the level of consciousness (associated TBI, uncontrolled intubation with bronchoaspiration...). Post-traumatic hypovolemia (in this case, as there are multiple rib fractures, it could be due to hemothorax) would occur earlier and would be associated in the first place with hemodynamic instability.", "full_answer_no_ref": "Pulmonary contusion is the most serious lesion and the one with the worst prognosis in thoracic trauma. It is also the injury that causes hypoxemia the earliest. Contrary to what we may think, an unstable thorax due to multiple rib fractures (costal volet) would cause progressive hypoventilation with atelectasis of the pulmonary parenchyma, which would progress on the one hand to hypercapnia and respiratory acidosis, and on the other, to [HIDDEN] due to infection associated with atelectasis. Respiratory infection due to aspiration would also occur later, and does not appear in all cases of severe chest trauma, only if there has been a decrease in the level of consciousness (associated TBI, uncontrolled intubation with bronchoaspiration...). [HIDDEN] (in this case, as there are multiple rib fractures, it could be due to hemothorax) would occur earlier and would be associated in the first place with hemodynamic instability.", "full_question": "35-year-old man admitted for severe chest trauma with multiple rib fractures. After responding favorably to treatment with analgesics and oxygen, he begins to present severe hypoxemia. Indicate the most probable cause of this deterioration:", "id": 477, "lang": "en", "options": {"1": "Chest wall instability due to multiple fractures.", "2": "Aspiration respiratory infection.", "3": "Alteration of gas exchange due to pulmonary contusion.", "4": "Post-traumatic hypovolemia.", "5": NaN}, "question_id_specific": 128, "type": "CRITICAL CARE", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0649_11484", "title": "Long-term pulmonary function after recovery from pulmonary contusion due to blunt chest trauma.", "score": 0.017067550050410486, "content": "Blunt chest trauma can cause severe acute pulmonary dysfunction due to hemo/pneumothorax, rib fractures and lung contusion. To study the long-term effects on lung function tests after patients' recovery from severe chest trauma. We investigated the outcome and lung function tests in 13 patients with severe blunt chest trauma and lung contusion. The study group comprised 9 men and 4 women with an average age of 44.6 +/- 13 years (median 45 years). Ten had been injured in motor vehicle accidents and 3 had fallen from a height. In addition to lung contusion most of them had fractures of more than three ribs and hemo/pneumothorax. Ten patients were treated with chest drains. Mean intensive care unit stay was 11 days (range 0-90) and mechanical ventilation 19 (0-60) days. Ten patients had other concomitant injuries. Mean forced expiratory volume in the first second was 81.2 +/- 15.3%, mean forced vital capacity was 85 +/- 13%, residual volume was 143 +/- 33.4%, total lung capacity was 101 +/- 14% and carbon monoxide diffusion capacity 87 +/- 24. Post-exercise oxygen saturation was normal in all patients (97 +/- 1.5%), and mean oxygen consumption max/kg was 18 +/- 4.3 ml/kg/min (60.2 +/- 15%). FEV1 was significantly lower among smokers (71.1 +/- 12.2 vs. 89.2 +/- 13.6%, P = 0.017). There was a non-significant tendency towards lower FEV1 among patients who underwent mechanical ventilation. Late after severe trauma involving lung contusion, substantial recovery was demonstrated with improved pulmonary function tests. These results encourage maximal intensive care in these patients. Further larger studies are required to investigate different factors affecting prognosis."}, {"id": "pubmed23n0047_8540", "title": "[Surfactant administration in acute respiratory failure].", "score": 0.01626123744050767, "content": "We report the case of a 21-year-old man who developed adult respiratory distress syndrome (ARDS) after severe lung contusion due to a car accident. At the scene of the accident the patient was awake and oriented, but there were signs of hypoxaemia (SaO2 by pulse oximetry: 86%). The trachea was intubated in the emergency room and, after diagnosis of multiple rib fractures on the right side (the patient nos. 4-11) and emergency treatment, was extubated 16 h later. During spontaneous breathing there was no improvement of pulmonary function, and the patient was transferred to the intensive care unit 5 days later and reintubated because of acute respiratory failure. He then developed the signs of severe ARDS. No improvement occurred during conventional ventilatory treatment including inversed-ratio ventilation, high-frequency ventilation, and ventilation via a double-lumen tube. On day 15 a bovine surfactant preparation (38 mg/kg body wt.) was instilled into both lungs. Initially there was deterioration of the pulmonary function, probably due to crusts in the bronchial mucous membrane. After aspiration of the crusts at bronchoscopy, there was progressive respiratory improvement. The inspired oxygen concentration and PEEP level could be reduced, and the ventilatory ratio normalised within 14 days. This therapeutic intervention improved pulmonary function and probably led to the successful outcome after 36 days of ventilatory support."}, {"id": "pubmed23n1143_15037", "title": "Hemostatic Achievement After Introduction of Venovenous Extracorporeal Membrane Oxygenation for Severe Multiple Trauma: A Case Study.", "score": 0.016133162612035852, "content": "Venovenous extracorporeal membrane oxygenation (VV-ECMO) is indicated for patients with severe respiratory failure who cannot be managed with a ventilator. We report a case of severe chest trauma with an injury severity score of 66, in which hemostasis was achieved after VV-ECMO. A 29-year-old male patient sustained a fall injury from a 4-m cliff. The fall resulted in significant traumatic cerebral hemorrhage, bilateral pulmonary contusion, hemothorax, pelvic fracture, and limb fracture. During transcatheter arterial embolization, the patient continued to bleed from the left lung and showed progressive hypoxemia. In addition, the patient was unable to maintain tidal volume and experienced hypercapnia, and thus, VV-ECMO was introduced, followed by a thoracotomy to stop the bleeding. On the third day of hospitalization, the patient was weaned off VV-ECMO, and on day 35, he was transferred to a rehabilitation hospital for recovery. VV-ECMO may serve as a \"bridge\" until hemostatic maneuvers for severe chest trauma are completed and may contribute to help ensure adequate respiration."}, {"id": "pubmed23n1053_4397", "title": "Massive pulmonary haemorrhage due to severe trauma treated with repeated alveolar lavage combined with extracorporeal membrane oxygenation: A case report.", "score": 0.015422077922077922, "content": "Massive pulmonary haemorrhage can spoil the entire lung and block the airway in a short period of time due to severe bleeding, which quickly leads to death. Alveolar lavage is an effective method for haemostasis and airway maintenance. However, patients often cannot tolerate alveolar lavage due to severe hypoxia. We used extracorporeal membrane oxygenation (ECMO) to overcome this limitation in a patient with massive pulmonary haemorrhage due to severe trauma and succeeded in saving the life by repeated alveolar lavage. A 22-year-old man sustained multiple injuries in a motor vehicle accident and was transferred to our emergency department. On admission, he had a slight cough and a small amount of bloody sputum; computed tomography revealed multiple fractures and mild pulmonary contusion. At 37 h after admission, he developed severe chest tightness, chest pain, dizziness and haemoptysis. His oxygen saturation was 68%. Emergency endotracheal intubation was performed, and a large amount of bloody sputum was suctioned. After transfer to the intensive care unit, he developed refractory hypoxemia and heparin-free venovenous ECMO was initiated. Fibreoptic bronchoscopy revealed diffuse and profuse blood in all bronchopulmonary segment. Bleeding was observed in the trachea and right bronchus, and repeated alveolar lavage was performed. On day 3, the patient's haemoptysis ceased, and ECMO support was terminated 10 d later. Tracheostomy was performed on day 15, and the patient was weaned from the ventilator on day 21. Alveolar lavage combined with ECMO can control bleeding in trauma-induced massive pulmonary haemorrhage, is safe and can be performed bedside."}, {"id": "pubmed23n0479_23010", "title": "Pulmonary contusion in severe head trauma patients: impact on gas exchange and outcome.", "score": 0.01510676965015902, "content": "To evaluate the impact on morbidity and mortality of pulmonary contusion in multiple-trauma patients with severe head trauma. Matched-paired, case-control study ICU at a tertiary university hospital. During a 3-year period, 313 consecutive multiple-trauma patients with severe head trauma (Glasgow coma scale [GCS], &lt;/= 8) who were admitted to the ICU. Case-control matching criteria were as follows: (1) age difference within 5 years; (2) sex; (3) GCS in two categories; (4) similar pattern of injury; and (5) simplified acute physiology score II in five categories. A pulmonary contusion, defined by the clinical context and the result of a chest CT scan, was diagnosed in 90 patients. Analysis was performed on 90 pairs who were matched with 100% success. Ninety patients (29%) presented a diagnosis of pulmonary contusion. The presence of pulmonary contusion had an impact on the PaO(2)/fraction of inspired oxygen (FIO(2)) ratio, which was significantly reduced in patients with a pulmonary contusion. The ICU stay, the occurrence of complications such as nosocomial pneumonia or ARDS, the Glasgow outcome scale, and the mortality rate did not significantly differ between case patients and control subjects. Mortality also was not affected when patients were stratified according to the severity of the PaO(2)/FIO(2) ratio. In the study patients, pulmonary contusion alters gas exchange but does not appear to increase the morbidity and mortality of multiple-trauma patients with head trauma. A sample-size effect may limit the interpretation of the study."}, {"id": "pubmed23n0764_1410", "title": "Acute refractory hypoxemia after chest trauma reversed by high-frequency oscillatory ventilation: a case report.", "score": 0.012968540829986613, "content": "Polytrauma often results in significant hypoxemia secondary to direct lung contusion or indirectly through atelectasis, systemic inflammatory response, large volume fluid resuscitation and blood product transfusion. In addition to causing hypoxemia, atelectasis and acute lung injury can lead to right ventricular failure through an acute increase in pulmonary vascular resistance. Mechanical ventilation is often applied, accompanied with recruitment maneuvers and positive end-expiratory pressure in order to recruit alveoli and reverse atelectasis, while preventing excessive alveolar damage. This strategy should lead to the reversal of the hypoxemic condition and the detrimental heart-lung interaction that may occur. However, as described in this case report, hemodynamic instability and intractable alveolar atelectasis sometimes do not respond to conventional ventilation strategies. We describe the case of a 21-year-old Caucasian man with severe chest trauma requiring surgical interventions, who developed refractory hypoxemia and overt right ventricular failure. After multiple failed attempts of recruitment using conventional ventilation, the patient was ventilated with high-frequency oscillatory ventilation. This mode of ventilation allowed the reversal of the hemodynamic effects of severe hypoxemia and of the acute cor pulmonale. We use this case report to describe the physiological advantages of high-frequency oscillatory ventilation in patients with chest trauma, and formulate the arguments to explain the positive effect observed in our patient. High-frequency oscillatory ventilation can be used in the context of a blunt chest trauma accompanied by severe hypoxemia due to atelectasis. The positive effect is due to its capacity to recruit the collapsed alveoli and, as a result, the relief of increased pulmonary vascular resistance and subsequently the reversal of acute cor pulmonale. This approach may represent an alternative in case of failure of the conventional ventilation strategy."}, {"id": "pubmed23n0076_9831", "title": "[The effect of severe closed chest trauma on gas exchange].", "score": 0.012422360248447204, "content": "Ventilation and gas exchange lung functions were studied in 110 patients with severe closed chest trauma. In chest trauma that was not accompanied by intrapulmonary traumatic changes the main pathogenetic mechanism of gas exchange damage was marked pain syndrome. Such patients did not suffer from severe arterial hypoxemia and their intrapulmonary shunting did not exceed 15%. Analgesia and, if necessary, lung decompression improved considerably respiratory parameters and prevented the onset of severe pulmonary failure. Patients with intrapulmonary traumatic changes (lung contusion, intrapulmonary hematomas) were characterized by progressing arterial hypoxemia due to a considerable increase in intrapulmonary shunting. These patients are managed mainly by preventive therapy of pulmonary hyperhydration, thorough tracheobronchial cleansing, cough stimulation, prevention of pneumonia."}, {"id": "wiki20220301en213_37736", "title": "Pulmonary contusion", "score": 0.012001830435144354, "content": "A large amount of force is required to cause pulmonary contusion; a person injured with such force is likely to have other types of injuries as well. In fact, pulmonary contusion can be used to gauge the severity of trauma. Up to three quarters of cases are accompanied by other chest injuries, the most common of these being hemothorax and pneumothorax. Flail chest is usually associated with significant pulmonary contusion, and the contusion, rather than the chest wall injury, is often the main cause of respiratory failure in people with these injuries. Other indications of thoracic trauma may be associated, including fracture of the sternum and bruising of the chest wall. Over half of fractures of the scapula are associated with pulmonary contusion. The contusion is frequently found underlying fracture sites. When accompanied by a fracture, it is usually concentrated into a specific location—the contusion is more diffuse when there is no fracture. Pulmonary lacerations may"}, {"id": "wiki20220301en213_37739", "title": "Pulmonary contusion", "score": 0.011551499348109517, "content": "Pulmonary contusion is found in 30–75% of severe cases of chest injury, making it the most common serious injury to occur in association with thoracic trauma. Of people who have multiple injuries with an injury severity score of over 15, pulmonary contusion occurs in about 17%. It is difficult to determine the death rate (mortality) because pulmonary contusion rarely occurs by itself. Usually, deaths of people with pulmonary contusion result from other injuries, commonly traumatic brain injury. It is controversial whether pulmonary contusion with flail chest is a major factor in mortality on its own or whether it merely contributes to mortality in people with multiple injuries. The estimated mortality rate of pulmonary contusion ranges from 14–40%, depending on the severity of the contusion itself and on associated injuries. When the contusions are small, they do not normally increase the chance of death or poor outcome for people with blunt chest trauma; however, these chances"}, {"id": "wiki20220301en213_37694", "title": "Pulmonary contusion", "score": 0.01154205758132637, "content": "Signs and symptoms take time to develop, and as many as half of cases are asymptomatic at the initial presentation. The more severe the injury, the more quickly symptoms become apparent. In severe cases, symptoms may occur as quickly as three or four hours after the trauma. Hypoxemia (low oxygen concentration in the arterial blood) typically becomes progressively worse over 24–48 hours after injury. In general, pulmonary contusion tends to worsen slowly over a few days, but it may also cause rapid deterioration or death if untreated. Causes"}, {"id": "pubmed23n0235_14184", "title": "Epidural analgesia or mechanical ventilation for multiple Rib fractures?", "score": 0.010897603106976204, "content": "A protocol for treating thoracic trauma is proposed. Severe pulmonary lesion with increased venous admixture (e.g. contusio, atelectasis, aspiration) is treated by mechanical ventilation. Rib fractures with minor pulmonary lesion and therefore with only moderately abnormal gas exchange but with remarkably reduced vital capacity (even with flail chest) are controlled by thoracic epidural analgesia following vital capacity, tidal volume and respiratory rate. If both a severe pulmonary lesion and serial rib fractures are present, the patient is ventilated for 2-3 days and then extubated to breath spontaneously with epidural analgesia. The indication for a mechanical ventilation or for spontaneous breathing with thoracic epidural analgesia is therefore deducted more from functional variables than from morphological facts. The course of a consecutive series of 283 patients is presented. 155 patients were treated with primary ventilation and 112 patients with primary epidural analgesia, while 16 patients could be managed with general analgesia. The duration of treatment morbidity and mortality show this protocol to be very useful."}, {"id": "pubmed23n1116_11141", "title": "[Successful treatment of an adult with severe chest trauma under extracorporeal membrane oxygenation: a case report].", "score": 0.010669362084456423, "content": "Veno-venous extracorporeal membrane oxygenation (VV-ECMO) is a valuable treatment option for chest trauma individuals and some patients required surgery. A 35-year-old female patient with severe chest trauma was admitted to Affiliated Hospital of Zunyi Medical University on February 27, 2020. The patient was hospitalized with chest pain and dyspnea due to fall from a height. Emergency chest CT revealed a right fluid pneumothorax (60% of right lung compression), left hemothorax, little pericardial effusion, and multiple emphysemas in the lower neck, chest, back and mediastinum. Invasive ventilator was difficult to maintain oxygen saturation. After evaluation, the VV-ECMO was established, then, she received a thoracotomy. There was a 1.2 cm trachea rupture observed during operation, and the trachea was repaired. The operation lasted 4 hours with the continuous support of VV-ECMO. When the patient's haemodynamics and oxygenation was stable, ECMO was removed. Sixteen days later, the patient's chest CT showed that, the chest wall subcutaneous emphysema was reduced, and the exudative lesions of both lungs were absorbed, indicating that the patient was treated effectively and reached the discharge standard with no complication. During the treatment of this patient, VV-ECMO was applied rapidly and lasted for a short period, which provided the patient with the opportunity of emergency operation and finally the patient was fully recovered. VV-ECMO can provide support for patients with severe trauma and refractory hypoxemia."}, {"id": "wiki20220301en213_37688", "title": "Pulmonary contusion", "score": 0.010254968128983877, "content": "The severity ranges from mild to severe: small contusions may have little or no impact on health, yet pulmonary contusion is the most common type of potentially lethal chest trauma. It occurs in 30–75% of severe chest injuries. The risk of death following a pulmonary contusion is between 14–40%. Pulmonary contusion is usually accompanied by other injuries. Although associated injuries are often the cause of death, pulmonary contusion is thought to cause death directly in a quarter to half of cases. Children are at especially high risk for the injury because the relative flexibility of their bones prevents the chest wall from absorbing force from an impact, causing it to be transmitted instead to the lung. Pulmonary contusion is associated with complications including pneumonia and acute respiratory distress syndrome, and it can cause long-term respiratory disability. Classification"}, {"id": "wiki20220301en213_37695", "title": "Pulmonary contusion", "score": 0.01001788908765653, "content": "Causes Pulmonary contusion is the most common injury found in blunt chest trauma, occurring in 25–35% of cases. It is usually caused by the rapid deceleration that results when the moving chest strikes a fixed object. About 70% of cases result from motor vehicle collisions, most often when the chest strikes the inside of the car. Falls, assaults, and sports injuries are other causes. Pulmonary contusion can also be caused by explosions; the organs most vulnerable to blast injuries are those that contain gas, such as the lungs. Blast lung is severe pulmonary contusion, bleeding, or edema with damage to alveoli and blood vessels, or a combination of these. This is the primary cause of death among people who initially survive an explosion. Unlike other mechanisms of injury in which pulmonary contusion is often found alongside other injuries, explosions can cause pulmonary contusion without damage to the chest wall."}, {"id": "pubmed23n0259_11063", "title": "[Disorders of respiratory function in rib fractures].", "score": 0.009900990099009901, "content": "In the one-year prospective study 71 injured patients were observed (75% male and 25% female). Traffic traumatism was the dominant case (45%). The wounded are divided in the groups with one side fracture of ribs (left/right) and on both sides fracture of ribs considering the side of fracture, and there is consideration about the kind of fracture--there are single fracture of ribs and serial fracture of ribs. The samples of artery blood were followed in PaCO2, %SaO2 and level pH in three points of time: when the patients came, after 24 and after 48 hours. In the group with the both side fracture of the ribs, the fall of worth pH was observed after 48 hours, PaCO2 is increasing to the 6.98 kPa. PaO2 is falling after 48 hours. In %SaO2 there is no considerable difference at any time, but%SaO2 is the highest in the second group. With the serial fracture of ribs wounded are considerate the fall of worth pH which is progressively increasing and is the highest after 48 hours. PaCO2 is increasing in the both groups, but with the serial fracture the worth are considerably higher. PaO2 and %SaO2 are much lower after 48 hours. The authors conclude that the wounded on both sides and wounded with serial fracture along one or several lines of with fracture of all ribs suffer the highest respiratory insufficiency (ARI), so they need artificial ventilation as respiratory support."}, {"id": "pubmed23n0767_826", "title": "Hemodynamic and respiratory support using venoarterial extracorporeal membrane oxygenation (ECMO) in a polytrauma patient.", "score": 0.009860831127195005, "content": "There are few reports in the literature regarding the use of venoarterial extracorporeal membrane oxygenation (ECMO) for double-dysfunction from both heart and lung contusions in polytrauma patients. This article reports a 48-year-old patient admitted after a traffic accident. He rapidly progressed to shock with low cardiac output due to myocardial contusion and refractory hypoxemia due to pulmonary contusion, an unstable chest wall and bilateral pneumothorax. ECMO was an effective rescue procedure in this dramatic situation and was successfully discontinued on the fourth day after the trauma. The patient also developed an extensive brain infarction and eventually died on the seventh day after admission. "}, {"id": "pubmed23n0955_12454", "title": "Blunt rupture of the thoracic duct after severe thoracic trauma.", "score": 0.00980392156862745, "content": "A 53-year-old man was admitted to our trauma center after sustaining thoracoabdominal injuries, secondary to a rear-end motor vehicle collision. As he stepped out of his vehicle, he was struck by a tractor trailer at 55 mph. The following were the initial vital signs on his arrival: heart rate 140 beats/min, blood pressure 142/80 mm Hg, respiratory rate 28 breaths/min, temperature 36.8°C, and oxygen saturation 93%. The Glasgow Coma Scale score was 15 and the Injury Severity Score was 59. He was evaluated and resuscitated per the advanced trauma life support protocols. The focused assessment with sonography for trauma examination was negative. Initial findings included bilateral chest wall and thoracic spine tenderness, subcutaneous emphysema in the chest and neck, and an unstable pelvis. He required bilateral chest tubes and a pelvic binder. CT imaging revealed a left temporal epidural hematoma, multiple facial fractures, a sternal fracture, a left scapula fracture, acromioclavicular fractures, bilateral hemopneumothoraces, pulmonary contusions, extensive pneumomediastinum compressing the right atrium, multiple rib fractures (2-10 on the left with a flail segment and 2-8 on the right) (figure 1), an unstable open-book pelvic fracture which included bilateral superior and inferior pubic rami fractures, sacral and left iliac wing fractures, and symphysis pubis diastasis.Figure 1Three-dimensional CT scan reconstruction demonstrating left-sided flail chest.The patient developed hypotension and severe respiratory distress, and was intubated. ECG revealed no dysrhythmias. Echocardiogram revealed significant left ventricular wall dysfunction consistent with myocardial contusion and right atrial compression. His troponins were also significantly elevated. He required significant resuscitation with crystalloids, blood products and vasopressors. He underwent bronchoscopy, esophagram and upper endoscopy to exclude tracheoesophageal injury, and these were negative. On hospital day 2, the patient was hemodynamically stable, and pressors were discontinued. His pelvic fractures were repaired using external fixation and sacral screws. Given his extensive left flail chest, he underwent reconstruction of his left chest wall on hospital day 5. Open reduction and internal fixation of his left ribs, 3 to 6 anteriorly and 4 to 7 posteriorly, with titanium plates was performed (figure 2). He had an epidural catheter inserted for analgesia. On postoperative day 2 after chest wall reconstruction, the patient was extubated and resumed enteral feeds. Overnight, the output from the left-sided chest tube changed from serosanguinous to milky. A sample was sent for triglycerides and lymphocyte counts confirming the diagnosis of chylothorax. His chest tube output increased to approximately 2000 mL/day. A lymphangiogram was performed with Lipiodol to diagnose the location of the chylous leak. It revealed contrast extravasation at the level of T3 to T4. An MRI was also performed to better define the anatomic course of the thoracic duct.Figure 2Postoperative chest X-ray demonstrating left chest wall reconstruction. Conservative management: placing the patient nulla per os (NPO), and starting total parenteral nutrition (TPN), octreotide and midodrine.Thoracic duct embolization by interventional radiology.CT-guided thoracic duct disruption.Thoracotomy with thoracic duct ligation."}, {"id": "pubmed23n0087_6804", "title": "Late sequelae of lung contusion.", "score": 0.00980392156862745, "content": "Twenty-four patients with severe lung contusion and multiple rib fractures were studied at a mean 4.9 years (range 2-9 years) after injury. All patients had been in good health before the accident. After the accident 15 (63 per cent) patients had respiratory symptoms such as dyspnoea at rest or moderate exercise (4), pain (8), cough or increased expectoration (11) and frequent bronchopulmonary infections (5). Three patients had changed their job because of respiratory disturbance. The average vital capacity, forced expiratory volume in 1 s, maximal voluntary ventilation and CO transfer factor were reduced respectively to 87, 88, 82 and 83 per cent of predicted values (P less than 0.01), while total lung capacity, residual volume and helium mixing time showed no definite changes (P greater than 0.05). Arterial blood gases at rest and at maximum exercise showed slight changes only. Maximal working capacity and ECG, as well as the ventilatory cost of moderate exercise were normal, where as the CO2 recovery time after moderate exercise was slightly increased (P less than 0.05). Overall there was a tendency towards poorer function in patients treated with artificial ventilation. Chest radiographs were normal in 10 patients (42 per cent), and moderate changes were seen in 14 patients. Diaphragmatic movements were essentially normal in all patients. Severe injury to the chest causes frequent respiratory symptoms. However, objective tests were only moderately reduced when compared with normal values. There was no unequivocal association between the subjective symptoms and the pulmonary function."}, {"id": "pubmed23n0847_7082", "title": "[Surgical Stabilisation of Flail Chest Injury: Indications, Technique and Results].", "score": 0.009708737864077669, "content": "Multiple rib fractures with segmental chest wall instability are caused by high-energy chest trauma and are associated with significant morbidity and mortality. Flail chest injuries are mostly combined with lung injury (contusion, rupture, laceration) and subsequent pneumothorax or haemothorax. Early mechanical ventilation with internal pneumatic splinting is a conservative treatment for flail chest in patients with respiratory insufficiency. The surgical stabilisation of a flail chest is an effective method of treatment and is beneficial for selected patients. It shortens the duration of mechanical ventilation and thus reduces morbidity associated with prolonged ventilatory support. In addition, it decreases long-term pain and the inability of a flail chest to heal due to malunion, non-union or progressive collapse of the flail segment. Surgical stabilisation of a flail chest is indicated when the clinical examination shows progressive respiratory dysfunction confirmed by the results of multiple detector computer tomography (MDCT) of the thorax. Thirty-three consecutive patients who underwent surgical stabilisation of a flail chest at the Trauma Centre between 2010 and 2014 were retrospectively evaluated. This included patient demographics, chest injury extent, results of pre-operative chest imaging (MDCT), surgical stabilisation technique and post-operative outcome. In addition to providing a radiographic finding of respiratory failure, the result of MDCT chest examination was considered an important criterion for surgical intervention. Surgical stabilisation of the chest wall was performed at an interval ranging from 2 hours to 11 days after injury. Intra-thoracic procedures were indicated in patients with lung injury (pulmonary laceration). The surgical procedure was completed by chest tube placement. Surgical stabilisation was carried out using 3 to 8 plates for flail segment fixation involving 3 to 4 ribs. The duration of post- operative mechanical ventilation was 5 days on the average. It was longer in patients with associated injuries such as craniocerebral trauma or severe pulmonary contusion. Tracheostomy was performed in seven patients requiring prolonged mechanical ventilation. Two patients had superficial surgical site infection. No death was recorded in the follow-up period. Surgical stabilisation of the flail chest segment is considered an effective procedure in selected patients, leading to improvement of respiratory function. By allowing for a shorter period of time on mechanical ventilation, it reduces the occurrence of complications due to ventilatory support. The result of MDCT chest examination in patients with fail chest is an important indication criterion for surgical fixation."}, {"id": "pubmed23n0213_9937", "title": "Partial blood oxygen pressure and pulmonary ventilation changes in patients with fractures with a view to traumatic fat embolism development.", "score": 0.009708737864077669, "content": "The body's response to the effects of mechanical injury, taking the form of shock during the first hours and the onset of fat embolism in the subsequent period, is substantially higher in patients with multiple or associated injuries, both as regards the severity of manifestations and prognostic risk. Also the death rate due to this sort of complication is seen rising. Two groups of injured persons with isolated (n = 33) and multiple fractures (n = 33) were used to show that dynamic follow-up of PaO2 in the blood could serve as a criterion of the risk of fat embolism development in the body and, in particular, as a prognostic criterion for the progress of fat embolism. Special point was made of findings demonstrating a time relationship between PaO2 deterioration in the early post-injury period (up to 24-36 hours) as compared with the period of 48-72 hours after the injury. PaO2 is seen dropping rapidly in injured persons showing signs of fat embolism syndrome development. The decrease can be recorded as early as the free interval phase, i.e. prior to the manifestation of the clinical signs of fat embolism. The findings of low PaO2 levels in the blood are in accordance with respiratory ventilation disturbances and impaired diffusion documented in our investigation."}, {"id": "pubmed23n0493_4037", "title": "The stove-in chest: a complex flail chest injury.", "score": 0.009615384615384616, "content": "The stove-in chest is a rare form of flail chest in which there is collapse of a segment of the chest wall, associated with a high immediate mortality. A 65-year-old male pedestrian was admitted with severe chest pain and dyspnoea, after being struck by a car. The initial chest radiograph demonstrated multiple right-sided rib fractures and pulmonary contusion. His gas exchange was good, and after pain relief via an epidural catheter was achieved, an intercostal drain was inserted into the right hemi-thorax. Clinically apparent deformation of the chest then occurred. A further chest radiograph confirmed the stove-in chest. The patient remained well initially, but on day 5 he deteriorated precipitously with respiratory failure, and signs of systemic sepsis. He died despite maximal ventilatory and inotropic support on the Intensive Care Unit (ICU). Post-mortem examination demonstrated congested, oedematous lungs with a right-sided empyema. The management of complex flail chest injuries requires treatment to be tailored to the individual patient. Early ventilatory support, despite good gas exchange, may have closed down the pleural space prevented the empyema. Prophylactic ventilation and possibly surgical stabilisation of the chest wall should be considered early in the course of admission, even when the conventional parameters to indicate ventilation are not met."}, {"id": "pubmed23n0224_14110", "title": "Crystalloid resuscitation of patients with pulmonary contusion.", "score": 0.009615384615384616, "content": "One hundred nine patients with the diagnosis of pulmonary contusion were studied retrospectively. Thirteen deaths were respiratory related (12 percent of patients). All of the patients were quickly resuscitated with crystalloid solutions as necessary to restore perfusion to normal. Twenty-eight of the most severely injured patients, all of whom were intubated and ventilated and in whom serial PaO2 and total protein determinations were available, were examined for the relationship between crystalloid induced hemodilution as measured by the plasma colloid oncotic pressure and oxygenation as measured by the PaO2/FiO2 ratio. When survivors and nonsurvivors were analyzed by group, both individually and collectively, no correlation was found between oxygenation and oncotic pressure. Survivors and nonsurvivors exhibited similar post-traumatic courses in the PaO2/FiO2 ratios with differences not becoming significant until the eleventh day after injury. We conclude that contusion is not a progressive lesion unless pneumonia supervenes and that pulmonary dysfunction after contusion is unrelated to hemodilution."}, {"id": "pubmed23n1160_20094", "title": "BLUNT TRAUMA INTERCOSTAL LUNG HERNIATION AND DELAYED EXTRA PLEURAL HEMATOMA.", "score": 0.009523809523809525, "content": "Blunt chest trauma is an important cause of morbidity and mortality in traumatized emergency patients. We report the case of a 74-year-old man who suffered a glenohumeral joint dislocation, trans trochanteric femur fracture, multiple rib fractures, diaphragmatic rupture with chest herniation of the spleen and stomach associated with herniation of the lung through an anterior chest wall defect after blunt trauma. Although immediate surgical repair was performed, he developed a delayed complication of multiple rib fracture in the form of large extrapleural hematoma that had to be surgically removed. Due to massive pulmonary contusion and prolonged pulmonary collapse, we used surfactant to facilitate alveolar opening after evacuation of the hematoma."}, {"id": "pubmed23n0236_5345", "title": "Clinical predictors of the adult respiratory distress syndrome.", "score": 0.009523809523809525, "content": "One hundred thirty-six patients meeting our criteria for one or more of eight clinical conditions were prospectively observed for the development of the adult respiratory distress syndrome. A high risk population was identified, including those with sepsis syndrome (38 percent), documented aspiration of gastric contents (30 percent), multiple emergency transfusions (24 percent), and pulmonary contusion (17 percent). The risk from multiple major fractures appeared low but contributed to the risk from other factors. The risk associated with just one factor (25 percent) was compounded by the presence of two (42 percent) and three (85 percent) simultaneous factors, and this finding was more predictive of ARDS than the injury severity score or initial arterial oxygenation. Of the ARDS cases, 76 percent occurred in the initial 24 hours after meeting the criteria. ARDS did not occur after 72 hours unless there was late development of sepsis (3 of 136 patients)."}, {"id": "pubmed23n0819_13143", "title": "Nuss procedure for severe flail chest after blunt trauma.", "score": 0.009433962264150943, "content": "Flail chest is a life-threatening condition that occurs when 3 or more consecutive ribs are segmentally fractured due to severe trauma and become detached from the rest of the thoracic cage. Flail chest is usually associated with other intrathoracic injuries, including pulmonary contusion, which can result in respiratory failure. We present a case of a 44-year-old man who was hit by a truck and a forklift resulting in multiple rib fractures bilaterally, and bilateral hemopneumothorax along with left chest wall depression and severe flail chest. The Nuss procedure was performed for both stabilization of severe flail chest and elevation of the depressed chest wall. The patient was weaned from mechanical ventilation on the first postoperative day and was ultimately discharged without any complications. "}, {"id": "wiki20220301en213_37719", "title": "Pulmonary contusion", "score": 0.009377367375770357, "content": "Treatment No treatment is known to speed the healing of a pulmonary contusion; the main care is supportive. Attempts are made to discover injuries accompanying the contusion, to prevent additional injury, and to provide supportive care while waiting for the contusion to heal. Monitoring, including keeping track of fluid balance, respiratory function, and oxygen saturation using pulse oximetry is also required as the patient's condition may progressively worsen. Monitoring for complications such as pneumonia and acute respiratory distress syndrome is of critical importance. Treatment aims to prevent respiratory failure and to ensure adequate blood oxygenation. Supplemental oxygen can be given and it may be warmed and humidified. When the contusion does not respond to other treatments, extracorporeal membranous oxygenation may be used, pumping blood from the body into a machine that oxygenates it and removes carbon dioxide prior to pumping it back in. Ventilation"}, {"id": "pubmed23n0993_21685", "title": "A Comprehensive Analysis of Traumatic Rib Fractures in an Acute General Hospital in Singapore.", "score": 0.009345794392523364, "content": "Rib fractures are common sequelae after blunt chest wall trauma. They can occur in isolation or association with life-threatening injuries to the head, thorax, and abdomen and may be complicated by hemothorax, pneumothorax, or lung contusions. Contiguous rib fractures can result in flail chest, which is associated with increased morbidity and mortality. This study aims to compare the risk factors, treatment modalities, and outcomes between patients with flail chest and nonflail chest postblunt trauma. Data were retrospectively collected from all patients admitted with rib fractures from January 2016 to December 2016 to the Department of General Surgery, Khoo Teck Puat Hospital, Singapore. The outcomes identified were mortality, pain scores on injury day 1, 3, 5, and 7, injury severity score, duration of mechanical ventilation, worst partial pressure arterial oxygen/fraction of inspired oxygen (PaO<sub2</sub/FiO<sub2</sub) ratio, length of intensive care unit (ICU) stay, and pulmonary complications. Motor vehicle accident was the most common cause of rib fractures (63.1%, <in</i = 123). Patients with flail chest had more associated pneumothorax (53.8% vs. 35.2%) and lung contusions (53.8% vs. 30.2%) compared to those without flail chest and underwent more investigations such as inpatient-computed tomography scans (76.9% vs. 59.3%), interventions such as chest tube insertion (61.5% vs. 19.8%), and ICU admission (46.1 vs. 13.7%). Patients also had higher pain scores, used more analgesic modalities, and had increased inpatient mortality (30.8% vs. 4.4%). Flail chest is associated with higher morbidity and mortality. Proactive management from a multidisciplinary team such as identification of high-risk patients in particular patients with flail chest, early admission to critical care, and protocols including multimodal pain management, respiratory support, and rehabilitation should be instituted."}, {"id": "pubmed23n0124_13019", "title": "Determinants of outcome after pulmonary contusion.", "score": 0.009345794392523364, "content": "During the past 5 1/2 years, 86 patients were treated for pulmonary contusion resulting from blunt trauma. Injury mechanism was motor vehicle in 65 patients (76%), farming in nine (10%), fall in eight (9%), and miscellaneous in four (5%). There were 68 males (79%) and 18 females. Ages ranged from 4 to 75 years (mean, 32 years). Twenty-two patients (26%) presented in hypovolemic shock. Injury Severity Score (ISS) averaged 26 (range, 9-57). Intubation was performed in the Emergency Department in 21 patients (24%), 19 of whom were severely hypoxic with pO2/FIO2 ratio less than 300. Thirty-four patients were ultimately treated with mechanical ventilation for 1 to 103 days (mean, 9.1 days). The average hospital stay was 22 days. Eleven patients (13%) died. Mortality was significantly greater (p less than 0.05) in patients with ISS greater than or equal to 25, initial Glasgow Coma Scale less than or equal to 7, transfusion of greater than three units of blood, and pO2/FIO2 less than 300. Mortality was not correlated with either presence of shock or amount of intravenous fluid administration. Eighteen patients with concomitant flail chest demonstrated no increase in mortality but were likely to require mechanical ventilation (p less than 0.05). The extent of contusion assessed on admission chest roentgenogram was not predictive of mortality or need for intubation. We recommend aggressive treatment of associated injuries, craniocerebral trauma, and selective mechanical ventilation based upon degree of intrapulmonary shunt."}, {"id": "pubmed23n0322_8556", "title": "Operative chest wall stabilization in flail chest--outcomes of patients with or without pulmonary contusion.", "score": 0.009259259259259259, "content": "The aim of operative chest wall stabilization in patients with flail chest and respiratory insufficiency is to reduce ventilator time and avoid ventilator associated complications. The purpose of this retrospective study was to analyze the indications and outcomes of operative chest wall stabilization in defined groups of patients sustaining flail chest with and without pulmonary contusion. The hospital records of 405 patients with multiple trauma (Injury Severity Score &gt; 17) between 1988 and 1994 were reviewed. Forty-two patients sustained flail chest. Twenty of these underwent operative chest wall stabilization for the following indications: 1) flail chest with indication for thoracotomy due to intrathoracic injury (n = 6); 2) flail chest without pulmonary contusion (n = 9); 3) paradoxical movement of a chest wall segment in the weaning period from the respirator (n = 3); and 4) severe deformity of the chest wall (n = 2). For the purpose of analysis the patients were separated into groups: group 1: operative chest wall stabilization in flail chest without pulmonary contusion (n = 10); group 2: operative chest wall stabilization in flail chest with pulmonary contusion (n = 10); group 3: flail chest without pulmonary contusion and without chest wall stabilization (n = 18); group 4: flail chest with pulmonary contusion and without chest wall stabilization (n = 4). Data were coded for time of operation, duration of ventilatory support, and complications. There were no significant differences in age, severity of injury, and extent of injury between groups 1, 2, and 3 (p &lt; 0.42). Group 4 was excluded for statistical analysis because of the small number of patients. Patients in group 1 required a shorter ventilatory support time compared to patients in group 3 (6.5+/-7.0 versus 26.7+/-29.0 days) and group 2 (p &lt; 0.02). In group 2 (ventilator time 30.8+/-33.7 days) early extubation was only possible in patients being operated on for chest wall instability during weaning from the ventilator. One patient in group 1, three patients in group 2 and five patients in group 3 developed pneumonia with further disturbance of gas exchange. All patients in group 1 survived; deaths in group 2 were attributed to massive hemorrhage in two and septic multiorgan failure in one patient. Four patients in group 3 died of head injury, one of acute respiratory distress syndrome, one of severe hemorrhage, and one of multiple organ failure. In patients with flail chest and respiratory insufficiency without pulmonary contusion, operative chest wall stabilization permits early extubation. Patients with pulmonary contusion do not benefit from chest wall stabilization. Secondary operative chest wall stabilization in these patients is indicated when progressive collapse of the chest wall is evident during weaning from the ventilator."}, {"id": "pubmed23n0408_11402", "title": "[Disorders of external respiration in severe combined trauma in an acute period of traumatic disease].", "score": 0.009259259259259259, "content": "Disorders of external respiration, clinical significance and prognostical information of the gas exchange system indexes in an acute period of traumatic disease (TD) in severe combined trauma (SCT) were studied. Pronounced stable changes of the external respiration indexes in injured persons for all forms of SCT, constituting high prognostic information in dynamics of course of TD, were revealed."}, {"id": "pubmed23n0312_905", "title": "Traumatic ventricular septal defect.", "score": 0.009174311926605505, "content": "A 26 year old man was admitted to hospital following a traffic accident. He had been sitting in the back of a car without wearing a seat belt. He suffered crush injuries on the anterior chest wall, trunk, and legs. On admission he was awake and cooperative, but restless, and obviously in severe pain. Radiography of the skull, facial bones, chest, spine, pelvis, and legs revealed a shaft fracture of the left femur and tibia and fracture of the 7th and 8th right ribs. The patient was transferred to the University Hospital of Zurich for further assessment and surgical repair of the lower limb fractures three days later. Because of worsening clinical condition with onset of partial respiratory insufficiency and new loud systolic murmur at the left sternal edge, a transthoracic echocardiography was performed, which showed an apical ventricular septal defect. Surgery was performed immediately. The ventricular septal defect was successfully repaired using a Teflon felt patch and interrupted sutures with pledgets, and sealed with glue. At six months' follow up the patient was doing well. Ventricular septal defects after blunt chest trauma occur either because of heart compression between sternum and the spine or because of myocardial infarction. In the present case the ventricular septal defect appeared three days after the accident, probably secondary to a post-traumatic myocardial infarction. Patients with blunt chest trauma and suspicion of cardiac contusion should be monitored carefully."}, {"id": "pubmed23n0107_14631", "title": "[Significance of lung contusion in mortality following polytrauma. Possibilities for therapeutic influence].", "score": 0.009174311926605505, "content": "Multiple trauma is often associated with blunt thoracic injuries. Especially lung contusion can result in respiratory insufficiency and therefore a higher mortality rate. In our prospective study comparing 8 multiple trauma patients with and without associated lung contusion, we found that respiratory function was already significantly disturbed (decrease of paO2/FiO2 and increase of AaDO2, a rise in extravascular lung water (EVLW) both early after trauma and also with a second peak following the 4th day. This group (LK) developed significantly more cases of respiratory distress (ARDS). The disturbance of respiratory function seen initially was interpreted as a consequence of the direct mechanical impact, leading to the formation of interstitial fluid and hematoma. The frequent development of ARDS in the LK-group probably results from a pronounced activation of cellular and humoral mechanisms and therefore an enforced injury of the pulmonary capillary bed. A significant increase of pulmonary infections or the development of sepsis was not seen in the LK-group and is probably not responsible for the higher ARDS-rate in this group."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[15, 170]], "word_ranges": [[2, 27]], "text": "Stage G3a corresponds to a filtration rate between 45-59 ml/min. Stage A1 corresponds to albuminuria less than 30 mg/ml. Therefore the correct option is 1."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Easy question. Stage G3a corresponds to a filtration rate between 45-59 ml/min. Stage A1 corresponds to albuminuria less than 30 mg/ml. Therefore the correct option is 1.", "full_answer_no_ref": "Easy question. Stage G3a corresponds to a filtration rate between 45-59 ml/min. Stage A1 corresponds to albuminuria less than 30 mg/ml. Therefore [HIDDEN].", "full_question": "A 66-year-old woman with type 2 diabetes mellitus. When assessing her renal function, she presents a G3a/A1 stage. To which values does this stage correspond, the most frequent in patients with diabetic nephropathy?", "id": 572, "lang": "en", "options": {"1": "Glomerular filtration rate 45-59 ml/min/1.73 m² and albuminuria <30 mg/ml.", "2": "Glomerular filtration rate 30-44 ml/min/1.73 m² and albuminuria <30 mg/ml.", "3": "Glomerular filtration rate 45-59 ml/min/1.73 m² and albuminuria 30-300 mg/ml.", "4": "Glomerular filtration rate 15-29 ml/min/1.73 m² and albuminuria <30 mg/ml.", "5": NaN}, "question_id_specific": 195, "type": "NEPHROLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0703_7302", "title": "Albuminuria and estimated glomerular filtration rate as predictors of diabetic end-stage renal disease and death.", "score": 0.017908538535337384, "content": "We investigated predictive value of albuminuria and estimated GFR (eGFR) for ESRD in Pima Indians with type 2 diabetes. Beginning in 1982, 2420 diabetic Pima Indians ≥18 years old were followed until they developed ESRD or died or until December 31, 2005. Individuals were classified at baseline by urinary albumin-to-creatinine ratio (ACR) and by eGFR, calculated by the Chronic Kidney Disease Epidemiology Collaboration equation. Predictors of ESRD and mortality were examined by proportional hazards regression. During a mean follow-up of 10.2 years, 287 individuals developed ESRD. Incidence of ESRD among individuals with macroalbuminuria (ACR ≥ 300 mg/g) was 9.3 times that of those with normoalbuminuria (ACR &lt; 30 mg/g), controlled for age, gender, and duration of diabetes. Incidence among individuals with eGFR 15 to 29 ml/min per 1.73 m(2) was 81.9 times that of those with eGFR 90 to 119 ml/min per 1.73 m(2). Models that combined albuminuria and eGFR added significant predictive information about risk of ESRD or death compared with models containing eGFR or albuminuria alone. The hazard ratio for ESRD associated with a 10-ml/min per 1.73 m(2) lower eGFR was 1.36, whereas that associated with an increase in albuminuria category was 2.69; corresponding hazard ratios for death were 1.15 and 1.37. These results suggest that incorporation of quantitative information about albuminuria into staging systems based on eGFR adds significant prognostic information about risk for diabetic ESRD and death."}, {"id": "article-28357_50", "title": "Chronic Kidney Disease -- Staging", "score": 0.017905459175553078, "content": "The 6 categories include: G1: GFR 90 ml/min per 1.73 m 2 and above G2: GFR 60 to 89 ml/min per 1.73 m 2 G3a: GFR 45 to 59 ml/min per 1.73 m 2 G3b: GFR 30 to 44 ml/min per 1.73 m 2 G4: GFR 15 to 29 ml/min per 1.73 m 2 G5: GFR less than 15 ml/min per 1.73 m 2 or treatment by dialysis The 3 levels of albuminuria include albumin-creatinine ratio (ACR): A1: ACR less than 30 mg/gm (less than 3.4 mg/mmol) A2: ACR 30 to 299 mg/gm (3.4 to 34 mg/mmol) A3: ACR greater than 300 mg/gm (greater than 34 mg/mmol)"}, {"id": "article-28357_3", "title": "Chronic Kidney Disease -- Introduction", "score": 0.01610062893081761, "content": "The 6 categories include: G1: GFR 90 ml/min per 1.73 m 2 and above G2: GFR 60 to 89 ml/min per 1.73 m 2 G3a: GFR 45 to 59 ml/min per 1.73 m 2 G3b: GFR 30 to 44 ml/min per 1.73 m 2 G4: GFR 15 to 29 ml/min per 1.73 m 2 G5: GFR less than 15 ml/min per 1.73 m 2 or treatment by dialysis The three levels of albuminuria include an albumin-creatinine ratio (ACR) A1: ACR less than 30 mg/gm (less than 3.4 mg/mmol) A2: ACR 30 to 299 mg/gm (3.4 to 34 mg/mmol) A3: ACR greater than 300 mg/gm (greater than 34 mg/mmol)."}, {"id": "pubmed23n0628_7909", "title": "Increased risk of renal deterioration associated with low e-GFR in type 2 diabetes mellitus only in albuminuric subjects.", "score": 0.014999442399910783, "content": "The significance of estimated glomerular filtration rate (e-GFR) in diabetic nephropathy has yet to be clearly determined. We therefore compared albuminuria and e-GFR for usefulness in predicting progressive decline in renal function. A total of 1,303 subjects with type 2 diabetes mellitus whose e-GFR was more than 30 mL/min/1.73 m(2) were followed for three years. Associations of clinical staging based on AER and that based on e-GFR with progression of renal insufficiency (e-GFR &lt;30 mL/min/1.73 m(2)) were evaluated. On univariate analysis, both clinical stages based on e-GFR and AER were significant variables (p&lt;0.05). On multiple logistic regression analysis, the odds ratio for macroalbuminuria was 132.3, and that for microalbuminuria was 10.3 while that for e-GFR less than 60 mL/min/1.73 m(2) was 9.0 for further deterioration of renal function. On the other hand, subjects without albuminuria exhibited a rate of disease progression of less than 1% irrespective of e-GFR level. Both albuminuria and reduced e-GFR are significant and independent risk factors for further deterioration of diabetic nephropathy, though albuminuria had a greater odds ratio than reduced e-GFR for deterioration of renal function over a three-year period. e-GFR exhibited additive risk for deterioration of diabetic nephropathy within three years only when albuminuria was present."}, {"id": "pubmed23n0582_11889", "title": "Sequence of progression of albuminuria and decreased GFR in persons with type 1 diabetes: a cohort study.", "score": 0.014652014652014652, "content": "The sensitivity of albuminuria in predicting loss of kidney function has been questioned. We determined the sequence of kidney disease stages (microalbuminuria, macroalbuminuria, low estimated glomerular filtration rate [eGFR], and end-stage renal disease [ESRD]) and characterized those without albuminuria before a low eGFR. The Pittsburgh Epidemiology of Diabetes Complications Study is a prospective cohort investigation of childhood-onset type 1 diabetes. 480 study participants with eGFR greater than 60 mL/min/1.73 m(2) (mean age, 27 years; diabetes duration, 19 years at study entry) were prospectively followed up for 16 years. Low eGFR was defined as creatinine clearance less than 60 mL/min/1.73 m(2) from timed urine collections; microalbuminuria, as albumin excretion rate between 20 to 200 microg/min (30 to 300 mg/24 h); macroalbuminuria, as albumin excretion rate greater than 200 microg/min (&gt;300 mg/24 h); and ESRD, as dialysis or renal transplantation. The 33 of 480 individuals (7%) who developed ESRD had prior albuminuria. 71 of 480 (15%) individuals developed low eGFR. 66 of 71 (93%) had prior/concurrent albuminuria, and 5 of 71 (7%) did not. Incident low eGFR values in the 5 patients were: (1) 54, (2) 58, (3) 59, (4) 59.7, and (5) 59.8 mL/min/1.73 m(2). 3 of 5 (60%; patients 1, 4, and 5) subsequently developed albuminuria. Final eGFRs in the 5 patients were: (1) 94, (2) 86, (3) 60, (4) 65, and (5) 54 mL/min/1.73 m(2), respectively. GFR and insulin sensitivity were not measured, but estimated. Incident decreased eGFR in patients without preceding/concurrent albuminuria may be caused by misclassification or a temporary eGFR decrease. Moderately decreased eGFR may occur rarely in patients with type 1 diabetes without preceding albuminuria."}, {"id": "pubmed23n0663_6510", "title": "Prognostic implications of the urinary albumin to creatinine ratio in veterans of different ages with diabetes.", "score": 0.014648033126293996, "content": "Albuminuria is associated with an increased risk of death independent of level of renal function. Whether this association is similar for adults of all ages is not known. We examined the association between the albumin to creatinine ratio (ACR) and all-cause mortality after stratification by estimated glomerular filtration rate (eGFR) and age group in 94 934 veterans with diabetes mellitus. Cohort members had at least 1 ACR recorded in the Veterans Affairs Health Care System between October 1, 2002, and September 30, 2003, and were followed up for death through October 15, 2009. From the youngest to the oldest age group, the prevalence of an eGFR less than 60 mL/min/1.73 m(2) ranged from 11% to 41%; microalbuminuria (ACR 30-299 mg/g) ranged from 19% to 28%; and macroalbuminuria (ACR &gt; or =300 mg/g) ranged from 3.2% to 3.7%. Of patients with an eGFR less than 60 mL/min/1.73 m(2), 72% of those younger than 65 years, 74% of those 65 to 74 years old, and 59% of those 75 years and older had an eGFR of 45 to 59 mL/min/1.73 m(2). In all age groups, less than 35% of these patients had albuminuria (ie, ACR &gt; or =30 mg/g). In patients 75 years and older, the ACR was independently associated with an increased risk of death at all levels of eGFR after adjusting for potential confounders. In younger age groups, this association was present at higher levels of eGFR but seemed to be attenuated at lower levels [corrected]. The ACR is independently associated with mortality at all levels of eGFR in older adults with diabetes and may be particularly helpful for risk stratification in the large group with moderate reductions in eGFR."}, {"id": "pubmed23n0823_19472", "title": "Albuminuria and reduced glomerular filtration rate for predicting the renal outcomes in type 2 diabetic patients.", "score": 0.014453748006379585, "content": "The first clinical manifestation of diabetic kidney disease is usually the development of microalbuminuria. However, recent studies have focused on diabetic patients with reduced glomerular filtration rate (GFR) without albuminuria. To evaluate the association of albuminuria and GFR with renal outcomes, we performed an observational study. A total of 3231 type 2 diabetic patients were included in this study between 2003 and 2005. There were 1249 women and the mean age was 59 ± 12 years. The renal endpoints were defined as the initiation of renal replacement therapy (RRT) or 50% reduction from the baseline of estimated GFR (eGFR). At baseline, 669 (20.7%) patients had eGFR &lt;60 mL/min per 1.73 m(2) and 1134 (35.1%) had albuminuria. During the mean follow-up period of 5.9 ± 1.6 years, 107 patients initiated RRT. A 50% reduction of eGFR from the baseline value was found in 279 patients. None of the normoalbuminuric subjects with or without reduced eGFR required RRT during the observational period (P &lt; 0.01). Compared to normoalbuminuria patients with eGFR ≥60 mL/min per 1.73 m(2) at baseline, the group of normoalbuminuria patients with reduced eGFR had a 2.5-fold risk of developing the renal endpoints, (95% confidence interval (CI): 1.0-6.3, P = 0.053). Patients with microalbuminuria with eGFR ≥60 mL/min per 1.73 m(2) at baseline had a 5.0-fold risk of developing the evaluated renal endpoints (95% CI: 2.8-8.8, P &lt; 0.001). Albuminuria was a significant predictor for the evaluated renal endpoints, but the impact of eGFR is likely to be less than that of albuminuria."}, {"id": "wiki20220301en019_111576", "title": "Glomerular filtration rate", "score": 0.014345434543454345, "content": "The severity of chronic kidney disease (CKD) is described by six stages; the most severe three are defined by the MDRD-eGFR value, and first three also depend on whether there is other evidence of kidney disease (e.g., proteinuria): 0) Normal kidney function – GFR above 90 mL/min/1.73 m2 and no proteinuria 1) CKD1 – GFR above 90 mL/min/1.73 m2 with evidence of kidney damage 2) CKD2 (mild) – GFR of 60 to 89 mL/min/1.73 m2 with evidence of kidney damage 3) CKD3 (moderate) – GFR of 30 to 59 mL/min/1.73 m2 4) CKD4 (severe) – GFR of 15 to 29 mL/min/1.73 m2 5) CKD5 kidney failure – GFR less than 15 mL/min/1.73 m2 Some people add CKD5D for those stage 5 patients requiring dialysis; many patients in CKD5 are not yet on dialysis. Note: others add a \"T\" to patients who have had a transplant regardless of stage."}, {"id": "wiki20220301en019_111573", "title": "Glomerular filtration rate", "score": 0.01377057315358531, "content": "Normal ranges The normal range of GFR, adjusted for body surface area, is 100–130 average 125 mL/min/1.73m2 in men and 90–120 ml/min/1.73m2 in women younger than the age of 40. In children, GFR measured by inulin clearance is 110 mL/min/1.73 m2 until 2 years of age in both sexes, and then it progressively decreases. After age 40, GFR decreases progressively with age, by 0.4–1.2 mL/min per year. Decreased GFR A decreased renal function can be caused by many types of kidney disease. Upon presentation of decreased renal function, it is recommended to perform a history and physical examination, as well as performing a renal ultrasound and a urinalysis. The most relevant items in the history are medications, edema, nocturia, gross hematuria, family history of kidney disease, diabetes and polyuria. The most important items in a physical examination are signs of vasculitis, lupus erythematosus, diabetes, endocarditis and hypertension."}, {"id": "pubmed23n1163_18969", "title": "Association of Estimated Glomerular Filtration Rate With Progression of Albuminuria in Individuals With Type 2 Diabetes.", "score": 0.013680331644403501, "content": "To elucidate the association of glomerular filtration rate (GFR) at baseline with subsequent progression of albuminuria in individuals with type 2 diabetes. This was a single-center retrospective cohort study of 6,618 Japanese adults with type 2 diabetes and urinary albumin-to-creatinine ratio of &lt;300 mg/g, comprising 2,459 women and 4,159 men with a mean (± SD) age of 60 ± 12 years. The exposure was baseline estimated GFR (eGFR) (mL/min/1.73 m2), treated as a categorical variable and classified into five categories: ≥90, 75-90, 60-75, 45-60, and &lt;45, as well as a continuous variable. The outcome was progression of albuminuria category (i.e., from normoalbuminuria to micro- or macroalbuminuria or from micro- to macroalbuminuria). Hazard ratios (HRs) for the outcome were estimated using the multivariable Cox proportional hazards model. In the analysis treating baseline eGFR as a continuous variable, the multivariable-adjusted restricted cubic spline model was used. During the median follow-up period of 6.3 years, 1,190 individuals reached the outcome. When those with a baseline eGFR of 75-90 mL/min/1.73 m2 were considered the reference group, HRs (95% CIs) for the outcome in those with a baseline eGFR of ≥90, 60-75, 45-60, or &lt;45 mL/min/1.73 m2 were 1.38 (1.14-1.66), 1.34 (1.14-1.58), 1.81 (1.50-2.20), or 2.37 (1.84-3.05), respectively. Furthermore, the inverse J-shaped curve was more clearly shown by the spline model. This study of Japanese adults with type 2 diabetes suggests that both high and low GFRs are implicated in the pathogenesis of albuminuria progression. Glomerular hyperfiltration may induce kidney damage. We asked whether glomerular filtration rate (GFR) is associated with subsequent development and progression of albuminuria in people with diabetes. This retrospective cohort study of 6,618 adults with type 2 diabetes has shown that both an estimated GFR (eGFR) ≥90 mL/min/1.73m2 and an eGFR &lt;75 mL/min/1.73m2 at baseline were risk factors for the subsequent category progression of albuminuria. This study suggests that both a high and low GFR are implicated in the pathogenesis of albuminuria development and progression in people with diabetes."}, {"id": "wiki20220301en085_59103", "title": "Contrast-induced nephropathy", "score": 0.013549239920687376, "content": "Roxana Mehran score The Roxana Mehran score is a clinical prediction rule to estimate probability of nephropathy (increase ≥25% and/or ≥0.5 mg/dl in serum creatinine at 48 h): Risk Factors: Systolic blood pressure <80 mm Hg - 5 points (if systolic BP less than 80 mmHg for at least one hour requiring inotropic support) Intra-arterial balloon pump - 5 points Congestive heart failure, counting as NYHA class III (marked limitation in activity due to symptoms, even during less-than-ordinary activity) or worse, or history of pulmonary edema - 5 points Age >75 y - 4 points Hematocrit level <39% for men and <35% for women - 3 points Diabetes mellitus- 3 points Contrast media volume - 1 point for each 100 mL Decreased kidney function: Serum creatinine level >1.5 g/dL - 4 points or Estimated Glomerular filtration rate (online calculator) 2 for 40–60 mL/min/1.73 m2 4 for 20–40 mL/min/1.73 m2 6 for < 20 mL/min/1.73 m2"}, {"id": "pubmed23n0625_6569", "title": "Is a reduced estimated glomerular filtration rate a risk factor for stroke in patients with type 2 diabetes?", "score": 0.013535486706218415, "content": "Although chronic kidney disease is a risk factor for cardiovascular disease it is unclear whether diabetic patients with a reduced glomerular filtration rate (GFR), independent of (micro)albuminuria, carry an increased risk of stroke. We therefore investigated the independent effect of estimated GFR (eGFR) on stroke events in patients with type 2 diabetes mellitus (T2DM). We studied T2DM patients with an eGFR &gt;or=15 ml min(-1) per 1.73 m(2), who had no history of stroke. Patients were divided into four categories by the eGFR at baseline for comparison: &gt;or=90, 60-89, 30-59 and 15-29 ml min(-1) per 1.73 m(2). The end point was an incident stroke event. The Cox proportional hazard model was used to calculate the hazard ratio (HR) and 95% confidence interval (CI). The study included a total of 1300 T2DM patients (546 women and 754 men) with a mean (+/-s.d.) age of 63+/-13 years. During a mean follow-up period of 3.7+/-1.4 years, 91 patients experienced an incident stroke event. Although a lower eGFR was associated with an increased stroke risk using a univariate model, statistical significance disappeared after adjusting for other risk factors including albuminuria. The HR (95% CI) was 0.75 (0.40-1.41, P=0.373), 0.99 (0.50-1.95, P=0.964) and 0.91 (0.36-2.28, P=0.844) for patients with eGFRs of 60-89, 30-59 and 15-29 ml min(-1) per 1.73 m(2), respectively, compared with patients with an eGFR &gt;or=90. Clinical albuminuria remained a significant risk factor for stroke, and the adjusted HR compared with normoalbuminuria was 2.40 (1.46-3.95, P=0.001). In conclusion, the association between reduced GFR and stroke events in patients with T2DM is likely to be mediated by albuminuria."}, {"id": "article-28359_11", "title": "Renal Function Tests -- Procedures -- Glomerular Filtration Rate", "score": 0.01322841829170943, "content": "Serum creatinine is also utilized in GFR estimating equations such as the Modified Diet in Renal Disease (MDRD) and the CKD-EPI (Chronic Kidney Disease Epidemiology Collaboration) equation. These eGFR equations are superior to serum creatinine alone since they include race, age, and gender variables. GFR is classified into the following stages based on kidney disease. Kidney Disease Improving Global Outcomes (KDIGO) stages of chronic kidney disease (CKD): Stage 1 GFR greater than 90 ml/min/1.73 m² Stage 2 GFR-between 60 to 89 ml/min/1.73 m² Stage 3a  GFR 45 to 59 ml/min/1.73 m² Stage 3b GFR 30 to 44 ml/min/1.73 m² Stage 4 GFR of 15 to 29 ml/min/1.73 m² Stage 5-GFR less than 15 ml/min/1.73 m² (end-stage renal disease)"}, {"id": "pubmed23n0534_4219", "title": "Assessment of glomerular filtration rate in addition to albuminuria is important in managing type II diabetes.", "score": 0.013000717572073783, "content": "Although much emphasis has been placed on screening for albuminuria in type II diabetic patients, less attention has been focused on the role of glomerular filtration rate (GFR) in the assessment of risk. Herein, we examined the association between GFR and vascular complications in a consecutive cohort of 5174 type II diabetic patients between 1995 and 2000. Renal function was assessed by GFR (estimated by Modification of Diet in Renal Disease equation). The frequency of chronic kidney disease (CKD) as defined by GFR &lt;60 ml/min/1.73 m(2), micro- and macrovascular complications, and their associations were analyzed. In this study cohort, 6% had serum creatinine &gt; or =150 micromol/l and 15.8% had CKD. After adjustment for potential confounders, including urinary albumin excretion, odds ratios [95% confidence interval (CI)] across different stages of estimated GFR (&gt; or =90, 60-89, 30-59, 15-29, &lt;15 ml/min/1.73 m(2)) for macrovascular disease were 1.00, 1.42 [1.12-1.80], 1.80 [1.32-2.45], 2.74 [1.64-4.56], and 4.05 [1.77-9.26], respectively (P for trend &lt;0.001); for retinopathy were 1.00, 1.23 [1.04-1.46], 1.80 [1.40-2.30], 2.05 [1.25-3.37], and 4.12 [1.56-10.90], respectively (P for trend &lt;0.001); for sensory neuropathy were 1.00, 1.53[1.27-1.85], 2.09 [1.58-2.76], 4.32 [2.41-7.77], and 3.16 [1.25-8.02], respectively (P for trend &lt;0.001); and for microalbumuria (with GFR &lt;15 ml/min/1.73 m(2) excluded from the analysis) were 1.00, 1.51 [1.30-1.75], 5.80 [4.52-7.44], and 52.5 [16.4-168.2] respectively (P for trend &lt;0.001). Measurement of serum creatinine alone without GFR may underestimate renal impairment in type II diabetic patients. Decreasing GFR was significantly associated with increasing frequency of micro- and macrovascular complications."}, {"id": "wiki20220301en023_76150", "title": "Assessment of kidney function", "score": 0.012745740498034076, "content": "There are several different techniques used to calculate or estimate the glomerular filtration rate (GFR or eGFR). The above formula only applies for GFR calculation when it is equal to the Clearance Rate. The normal range of GFR, adjusted for body surface area, is 100–130 average 125 (mL/min)/(1.73 m2) in men and 90–120 (mL/min)/(1.73 m2) in women younger than the age of 40. In children, GFR measured by inulin clearance is 110 (mL/min)/(1.73 m2) until 2 years of age in both sexes, and then it progressively decreases. After age 40, GFR decreases progressively with age, by 0.4–1.2 mL/min per year. Estimated GFR (eGFR) is now recommended by clinical practice guidelines and regulatory agencies for routine evaluation of GFR whereas measured GFR (mGFR) is recommended as a confirmatory test when more accurate assessment is required."}, {"id": "wiki20220301en025_100458", "title": "Chronic kidney disease", "score": 0.011991499696417728, "content": "Protein in the urine is regarded as an independent marker for worsening of kidney function and cardiovascular disease. Hence, British guidelines append the letter \"P\" to the stage of chronic kidney disease if protein loss is significant. Stage 1: Slightly diminished function; kidney damage with normal or relatively high GFR (≥90 ml/min/1.73 m2) and persistent albuminuria. Kidney damage is defined as pathological abnormalities or markers of damage, including abnormalities in blood or urine tests or imaging studies. Stage 2: Mild reduction in GFR (60–89 ml/min/1.73 m2) with kidney damage. Kidney damage is defined as pathological abnormalities or markers of damage, including abnormalities in blood or urine tests or imaging studies. Stage 3: Moderate reduction in GFR (30–59 ml/min/1.73 m2):. British guidelines distinguish between stage 3A (GFR 45–59) and stage 3B (GFR 30–44) for purposes of screening and referral."}, {"id": "wiki20220301en278_14588", "title": "Canagliflozin", "score": 0.011707434824589637, "content": "Evidence shows that apart from positive effects on glycemic levels, canagliflozin also reduces the risk of heart attacks and heart failures. SGLT2 inhibitors, including canagliflozin, reduce the likelihood of hospitalization for congestive heart failure or progression of renal disease in persons with diabetes mellitus type 2 and reduce the likelihood of stroke and heart attack in persons with diabetes mellitus type 2 who have known atherosclerotic vascular disease. Contraindications Canaglifozin is contraindicated in: Type 1 diabetes Diabetic ketoacidosis Severe renal impairment (estimated glomerular filtration rate <30 mL/min/1.73 m2), end-stage renal disease Patients on dialysis"}, {"id": "wiki20220301en019_111542", "title": "Glomerular filtration rate", "score": 0.0116999747027574, "content": "The normal range of GFR, adjusted for body surface area, is 100–130 average 125 mL/min/1.73m2 in men and 90–120 mL/min/1.73m2 in women younger than the age of 40. In children, GFR measured by inulin clearance is 110 mL/min/1.73 m2 until 2 years of age in both sexes, and then it progressively decreases. After age 40, GFR decreases progressively with age, by 0.4–1.2 mL/min per year. Estimated GFR (eGFR) is now recommended by clinical practice guidelines and regulatory agencies for routine evaluation of GFR whereas measured GFR (mGFR) is recommended as a confirmatory test when more accurate assessment is required."}, {"id": "pubmed23n1043_17505", "title": "The prognosis of subjects showing a reduced estimated glomerular filtration rate without albuminuria in Japanese patients with type 2 diabetes: a cohort study for diabetic kidney disease.", "score": 0.011580209516597249, "content": "To determine the renal and cardiovascular prognosis and all-cause mortality of Japanese patients with type 2 diabetes showing a reduced estimated glomerular filtration rate (eGFR) without albuminuria. A population of 675 patients with type 2 diabetes was prospectively observed for 4 years to determine the renal and cardiovascular outcomes and mortality. The subjects were divided into the four groups: those with a preserved eGFR and no albuminuria (n = 306), a preserved eGFR and albuminuria (n = 151), a reduced eGFR and no albuminuria (n = 96), and a reduced eGFR and albuminuria (n = 122). The Cox proportional hazard model and Fine and Gray method were used to assess between-group differences in the risk of mortality and cardiovascular events. In the group with a reduced eGFR, the eGFR value did not significantly change in the subjects without albuminuria (0 ± 8 mL/min/1.73 m<sup2</sup), whereas it decreased continuously in those with albuminuria (-6 ± 12 mL/min/1.73 m<sup2</sup). The incidence of cardiovascular events was significantly (P = 0.03) higher in the subjects with albuminuria (17%) than those without albuminuria (7%) in the group with a reduced eGFR. Cardiovascular events were significantly (P &lt; 0.01) more frequent in the group with a reduced eGFR than in those with a preserved eGFR in both subjects with and without albuminuria. The risk of end-stage kidney disease in non-albuminuric subjects with a reduced eGFR is considered to be low. We should focus on cardiovascular prognosis, because these patients are still at high risk of cardiovascular events, even though the prognosis is better in comparison to albuminuric patients."}, {"id": "wiki20220301en023_76154", "title": "Assessment of kidney function", "score": 0.01153701941103516, "content": "The severity of chronic kidney disease (CKD) is described by six stages; the most severe three are defined by the MDRD-eGFR value, and first three also depend on whether there is other evidence of kidney disease (e.g., proteinuria): 0) Normal kidney function – GFR above 90 (mL/min)/(1.73 m2) and no proteinuria 1) CKD1 – GFR above 90 (mL/min)/(1.73 m2) with evidence of kidney damage 2) CKD2 (mild) – GFR of 60 to 89 (mL/min)/(1.73 m2) with evidence of kidney damage 3) CKD3 (moderate) – GFR of 30 to 59 (mL/min)/(1.73 m2) 4) CKD4 (severe) – GFR of 15 to 29 (mL/min)/(1.73 m2) 5) CKD5 kidney failure – GFR less than 15 (mL/min)/(1.73 m2) Some people add CKD5D for those stage 5 patients requiring dialysis; many patients in CKD5 are not yet on dialysis. Note: others add a \"T\" to patients who have had a transplant regardless of stage."}, {"id": "wiki20220301en019_111568", "title": "Glomerular filtration rate", "score": 0.011317091649501622, "content": "The CKD-EPI equation performed better than the MDRD (Modification of Diet in Renal Disease Study) equation, especially at higher GFR, with less bias and greater accuracy. When looking at NHANES (National Health and Nutrition Examination Survey) data, the median estimated GFR was 94.5 mL/min per 1.73 m2 vs. 85.0 mL/min per 1.73 m2, and the prevalence of chronic kidney disease was 11.5% versus 13.1%. Despite its overall superiority to the MDRD equation, the CKD-EPI equations performed poorly in certain populations, including black women, the elderly and the obese, and was less popular among clinicians than the MDRD estimate. The CKD-EPI equation is: where SCr is serum creatinine (mg/dL), k is 0.7 for females and 0.9 for males, a is −0.329 for females and −0.411 for males, min indicates the minimum of SCr/k or 1, and max indicates the maximum of SCr/k or 1."}, {"id": "wiki20220301en019_111561", "title": "Glomerular filtration rate", "score": 0.011312611709316012, "content": "Example: A person has a plasma creatinine concentration of 0.01 mg/mL and in 1 hour produces 60 mL of urine with a creatinine concentration of 1.25 mg/mL. The common procedure involves undertaking a 24-hour urine collection, from empty-bladder one morning to the contents of the bladder the following morning, with a comparative blood test then taken. The urinary flow rate is still calculated per minute, hence: To allow comparison of results between people of different sizes, the CCr is often corrected for the body surface area (BSA) and expressed compared to the average sized man as mL/min/1.73 m2. While most adults have a BSA that approaches 1.7 m2 (1.6 m2 to 1.9 m2), extremely obese or slim patients should have their CCr corrected for their actual BSA. BSA can be calculated on the basis of weight and height."}, {"id": "nurse-article-28357_2", "title": "Chronic Kidney Disease (Nursing) -- Introduction", "score": 0.010793812680605135, "content": "The 6 categories include: G1: GFR 90 ml/min per 1.73 m2 and above G2: GFR 60 to 89 ml/min per 1.73 m2 G3a: GFR 45 to 59 ml/min per 1.73 m2 G3b: GFR 30 to 44 ml/min per 1.73 m2 G4: GFR 15 to 29 ml/min per 1.73 m2 G5: GFR less than 15 ml/min per 1.73 m2 or treatment by dialysis The three levels of albuminuria include albumin-creatinine ratio (ACR) A1: ACR less than 30 mg/gm (less than 3.4 mg/mmol) A2: ACR 30 to 299 mg/gm (3.4 to 34 mg/mmol) A3: ACR greater than 300 mg/gm (greater than 34 mg/mmol)."}, {"id": "wiki20220301en025_100457", "title": "Chronic kidney disease", "score": 0.010339151606597853, "content": "Additional imaging Additional tests may include nuclear medicine MAG3 scan to confirm blood flow and establish the differential function between the two kidneys. Dimercaptosuccinic acid (DMSA) scans are also used in kidney imaging; with both MAG3 and DMSA being used chelated with the radioactive element technetium-99. Stages A glomerular filtration rate (GFR) ≥ 60 ml/min/1.73 m2 is considered normal without chronic kidney disease if there is no kidney damage present. Kidney damage is defined signs of damage seen in blood, urine, or imaging studies which includes lab albumin/creatinine ratio (ACR) ≥ 30. All people with a GFR <60 ml/min/1.73 m2 for 3 months are defined as having chronic kidney disease."}, {"id": "pubmed23n0360_8751", "title": "Course of glomerular filtration rate in albuminuric type 2 diabetic patients with or without diabetic glomerulopathy.", "score": 0.009900990099009901, "content": "To evaluate and compare the clinical course and prognosis in type 2 diabetic patients with persistent albuminuria, with biopsy-proven diabetic glomerulosclerosis (DG), or with nondiabetic glomerulopathies (NDG). A kidney biopsy was performed in 34 consecutive type 2 diabetic patients with persistent albuminuria (&gt; or = 300 mg/24 h). Glomerular filtration rate (GFR) (51Cr-EDTA) was determined at least once a year, and albuminuria, arterial blood pressure, and HbA1c were determined every 3-6 months. The biopsy revealed DG in 26 patients (25 men/1 woman) (DG group), age 52 +/- 2 (mean +/- SEM) years, and NDG in 8 patients (7 men/1 woman) (NDG group), age 54 +/- 3 years. The patients were followed for a median of 7.7 years (range 1.0-14.2). In the DG group, GFR decreased from 82 (24-146) to 38 (2-116) ml.min-1.1.73 m-2 (P &lt; 0.001), with a median rate of decline in GFR of 5.6 (0.3-21.6) ml.min-1.year-1, and in the NDG group, GFR decreased from 107 (89-135) to 90 (17-119) ml.min-1.1.73 m-2 (P &lt; 0.05), with a median rate of decline in GFR of 1.3 (0.3-7.6) ml.min-1.year-1 (P &lt; 0.05 between groups). In the DG group, albuminuria increased from 1.4 (0.3-7.2) to 2.6 (0.1-21.6) g/24 h (P &lt; 0.05) and in the NDG group, decreased from 2.2 (0.8-8.7) to 0.8 (0.2-2.5) g/24 h (P = 0.05). Mean arterial blood pressure (MABP) decreased from 118 +/- 3 to 104 +/- 3 mmHg (P &lt; 0.05) in the DG group, whereas it remained unchanged in the NDG group (106 +/- 3 vs. 105 +/- 3 mmHg). In the DG group, the rate of decline in GFR correlated with systolic blood pressure (r = 0.62, P &lt; 0.001), MABP (r = 0.52, P &lt; 0.01), albuminuria (r = 0.55, P &lt; 0.005), and GFR at entry (r = -0.45, P &lt; 0.05). Our study demonstrated a more rapid decline in GFR and a progressive rise in albuminuria in type 2 diabetic patients with DG compared with type 2 diabetic patients with NDG."}, {"id": "pubmed23n0360_15445", "title": "Course of renal function in type 2 diabetic patients with abnormalities of albumin excretion rate.", "score": 0.00980392156862745, "content": "Heterogeneity in renal structure has been described in type 2 diabetic patients with both microalbuminuria and proteinuria; in fact, only a subset of type 2 diabetic patients have the typical diabetic glomerulopathy. However, it is currently unknown whether abnormalities in albumin excretion rate (AER) have a different renal prognostic value depending on the underlying renal structure. Aims of this study were: 1) to study the course of renal function in type 2 diabetic patients with altered AER; 2) to evaluate the relationship between the course of glomerular filtration rate (GFR) and renal structure; and 3) to evaluate the relationship between the course of GFR and baseline AER levels, metabolic control, and blood pressure levels during a follow-up period of 4 years. A total of 108 type 2 diabetic patients, 74 with microalbuminuria (MA) and 34 with proteinuria (P), were recruited into a prospective study that encompassed: 1) a baseline kidney biopsy with morphometric measurements of glomerular parameters; 2) intensified antihypertensive treatment for an average 4-year period (blood pressure target &lt;140/90 mmHg); and 3) determinations of GFR at baseline and every 6 months. Mean (+/- SD) GFR significantly decreased from baseline in both MA (-1.3+/-9.4 [95% CI -3.51 to +0.86], P &lt; 0.05) and P (-3.0+/-13.0 ml x min(-1) x 1.73 m(-2) per year [-7.71 to +1.61], P &lt; 0.01). However, the changes in GFR were quite heterogeneous. Thus, on the basis of percent GFR change per year from baseline (delta%GFR), both MA and P patients were defined as progressors or nonprogressors when they were below or above the median, respectively. Baseline parameters of glomerular structure had a strong influence on the course of GFR. Indeed, the odds ratios of being progressors significantly increased across the quartiles of baseline glomerular basement membrane (GBM) width and mesangial fractional volume [Vv(mes/glom)], being 2.71 and 2.85 higher, respectively, in the fourth quartile than in the first quartile (P &lt; 0.01 for both). Conversely, nonprogressors outnumbered progressors in the first quartile of GBM width (odds ratio: 2.14, P &lt; 0.05) and in the first quartile of Vv(mes/glom) (odds ratio: 2.28, P &lt; 0.01). Baseline albumin excretion rate (AER) did not influence delta%GFR; in fact, the number of progressors did not increase across quartiles of baseline AER among either MA or P. Similarly, mean blood pressure levels during follow-up (and intensified antihypertensive therapy) did not affect the course of GFR: the number of progressors and nonprogressors did not change across quartiles of mean blood pressure. In contrast, HbA1c during follow-up had an impact on delta%GFR: the odds ratio for being a progressor increased across quartiles of HbA1c, particularly for the highest quartile (HbA1c &gt;9.0%). In conclusion, the course of renal function is heterogeneous in type 2 diabetic patients with microalbuminuria or proteinuria. In fact, a subset of patients has a rapid decline in GFR over a 4-year follow-up period; these patients have more advanced diabetic glomerulopathy and worse metabolic control than the remaining patients, whose GFR remains stable. These two cohorts are otherwise undistinguishable as regards the degree of AER at baseline and tight blood pressure control. Kidney biopsy has an important prognostic role in these patients. Thus, tight blood pressure control, when not associated with satisfactory glycemic control, is unable to prevent rapid GFR decline in type 2 diabetic patients with typical diabetic glomerulopathy."}, {"id": "pubmed23n0389_19530", "title": "Evidence of a threshold value of glycated hemoglobin to improve the course of renal function in type 2 diabetes with typical diabetic glomerulopathy.", "score": 0.009708737864077669, "content": "We recently observed that the course of glomerular filtration rate (GFR) rapidly declines in a subgroup of Type 2 diabetic patients (D) with abnormalities of albumin excretion rate (AER) and typical diabetic nephropathy, despite tight blood pressure control. The aim of this study was to evaluate whether amelioration of blood glucose control, using insulin, improves the course of GFR. GFR decay was measured by spline modeling analysis of the plasma clearance rate of 51CR-EDTA, assessed every 6 months. We identified two groups of D using morphometric analysis of renal biopsy, who had values of glomerular basement membrane (GBM) and fractional mesangial volume (Vv mes/glom) respectively below (Group A: 38) or above (Group B: 50) the mean+2SD of values found in 27 kidney donors (GBM: 389 nm; Vv mes/glom: 0.25), as previously described in detail. Median AER was similar at base line in the 2 groups (109 microg/min, 29-1950, in Group A, 113 microg/min, 37-1845, in Group B; n.s.). Conventional metabolic therapy (sulphonylureas and/or biguanides) was used both in Group A and B during a 3 year follow-up period (Period 1). Group B was further divided in two subgroups with body mass index below (Group B, a) and above (Group B, b) the value of 30 kg/m2. Mean +/- SD HbA1c was 8.2 +/- 1.6% in Group A, 8.3 +/- 1.7% in Group B (a) (n.s.) and 9.1 +/- 1.7% in Group B (b) (n.s.). Tight blood pressure control was achieved and maintained using angiotensin converting enzyme inhibitors and/or beta blockers and/or calcium antagonists and/or thiazides. The mean arterial blood pressure (MAP) was 92 +/- 3 mmHg in Group A and 91 +/- 4 mmHg in Group B (n.s.). GFR decay was significantly greater in Group B than in Group A (Group A vs B: +1.21 +/- 0.71 vs -5.86 +/- 1.61 ml/min/1.73 m2/year). Median AER significantly rose in Group B (177 microg/min, p&lt;0.05 vs base line) but not in Group A (134 microg/min, n.s.) during the third year of follow-up. Groups A and B were then followed over 4.1 years (range 3.1-4.4) (Period 2) maintaining the above described antihypertensive regimen, resulting in MAP values similar to those described during Period 1. Group A patients were treated with the same conventional glycemic control during Period 2. Group B (a) was conversely treated with intensive insulin therapy to achieve a HbA1c value below 7.5% (3 daily injections of regular and 1 or 2 daily injections of intermediate acting insulin associated with metformin 500 mg twice daily in 64% of the patients). Group B (b) patients were only treated by metformin (850 mg thrice daily) to achieve a HbA1c value below 7.5%. HbA1c decreased below the 7.5% target value in Group B (a) (7.0 +/- 1.6%, p&lt;0.01 vs Period 1), but not in Group B (b) (8.0 +/- 1.6%, p&lt;0.05 vs Period 1) and in Group A (8.3 +/- 1.7%, n.s. vs Period 1). The GFR decay of Group B, a during Period 2 was lower than that during Period 1 (Period 1 vs Period 2: -5.9 +/- 1.8 vs -1.8 +/- 0.7 ml/min/1.73 m2/year, p&lt;0.01). GFR decay during Period 2 was similar to that observed during Period 1 in Group A (Period 1 vs Period 2: +1.21 +/- 0.71 vs +0.7 +/- 0.6 ml/min/1.73 ml/year, n.s.) and in Group B (b) (Period 1 vs Period 2: -4.4 +/- 0.71 vs -4.2 +/- 0.6 ml/min/1.73 m2/year, n.s.). Median AER did not significantly change in the fourth year of Period 2 , either in Group A or B (Group A vs B: 141 vs 152 microg/min, n.s.). In conclusion, our findings seem to suggest that amelioration of blood glucose control is attained both by insulin and metformin intensive treatment, but only insulin decreases and maintains HbA1c levels below 7.5%. These pattens of HbA1c appear to be a threshold value in order to significantly blunt GFR decay in a subgroup of Type 2 diabetic patients with typical diabetic glomerular lesions, who are less responsive to tight blood pressure control alone. Conversely, the cohort of patients with less severe diabetic glomerulopathy steadily show constant GFR patterns, despite similar abnormalities of albumin excretion rate, and HbA1c average values above 7.5%."}, {"id": "pubmed23n0297_3729", "title": "Glomerular hyperfiltration in the prediction of nephropathy in IDDM: a 10-year follow-up study.", "score": 0.009615384615384616, "content": "Glomerular hyperfiltration has been proposed as an independent risk factor for the development of diabetic nephropathy in patients with IDDM. In a case-controlled prospective study of IDDM patients without albuminuria, serial glomerular filtration rate (GFR) measurements were performed over an observation period of 10 years. A group of 25 IDDM patients (20 men, 5 women; initial age, 29 [17-49] years) with glomerular hyperfiltration (GFR &gt;135 ml x min(-1) x 1.73 m(-2)) were matched for age, sex, and duration of diabetes with 25 IDDM patients (20 men, 5 women; initial age, 30 [17-48] years) with glomerular normofiltration (GFR 83-135 ml x min(-1) x 1.73 m(-2)). GFR, urinary albumin excretion rate (AER), blood pressure, and glycated hemoglobin were measured at baseline and at 5, 8, and 10 years. The two groups had similar entry levels of blood pressure, AER, and glycated hemoglobin. Metabolic control was similar in the two groups during follow-up. The final GFR remained higher in the group with hyperfiltration (122 [109-135] vs. 103 [95-111] ml x min(-1) x 1.73 m(-2); P = 0.02) despite a nonsignificantly faster rate of fall of GFR compared with that of the control group (2.54 [1.20-3.88] vs. 1.50 [1.01-1.99] ml x min(-1) x year(-1); P = 0.14). A similar number of patients in each group progressed to either microalbuminuria or macroalbuminuria (n = 4 vs. n = 3) or developed hypertension (blood pressure, &gt;160/95 mmHg; n = 3 vs. n = 4). End-of-study AER was, however, higher in the group with hyperfiltration (geometric mean [95% CI]: 18.9 [11.3-31.6] vs. 11.0 [8.1-15.0]; P = 0.05), and baseline glomerular hyperfiltration was an independent determinant of end-of-study blood pressure (P = 0.04). The strongest predictors of end-of-study AER and blood pressure were their baseline values (P &lt; 0.04 and P &lt; 0.01, respectively). In conclusion, levels of AER and blood pressure are the main risk factors for renal outcome, while glomerular hyperfiltration appears to play a lesser role."}, {"id": "pubmed23n0264_4450", "title": "Glomerular structure in nonproteinuric IDDM patients with various levels of albuminuria.", "score": 0.009523809523809525, "content": "Although microalbuminuria is known to foretell the later development of overt proteinuria in patients with insulin-dependent diabetes mellitus (IDDM), different investigators have reported different levels of albuminuria as being predictive. However, whether different levels of albuminuria reflect differences in glomerular structure is not well known. In this study, we divided a cohort of 66 nonproteinuric long-standing (duration 20 +/- 7 years) IDDM patients, who had both renal functional and structural studies performed, into four groups according to their urinary albumin excretion rate (AER). The several different levels of microalbuminuria previously reported to be predictive served to demarcate these groups: group I, AER &lt; or = 22 mg/24 h (upper limit for normal in our laboratory) (33 patients); group II, AER 23-45 mg/24 h (11 patients); group III, AER 46-100 mg/24 h (13 patients); and group IV, AER 101-220 mg/24 h (9 patients). Creatinine clearance was similar in groups I, II, and III but was lower in group IV. Systemic hypertension was present in five patients in group I, one in group II, seven in group III, and five in group IV. Mean values for glomerular basement membrane (GBM) width and volume fraction of the mesangium [Vv(mes/glom)] were greater in all groups than in a group of 52 age-matched normal kidney donors (P &lt; 0.0001). Also, filtration surface density [Sv(PGBM)], inversely related to Vv(mes/glom) (r = 0.61, P &lt; 0.0001), was reduced in all diabetic groups compared with the normal group (P &lt; 0.0001). Structural measures were identical in group I and II.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0415_5769", "title": "Low glomerular filtration rate in normoalbuminuric type 1 diabetic patients: an indicator of more advanced glomerular lesions.", "score": 0.009345794392523364, "content": "Increased urinary albumin excretion rate is widely accepted as the first clinical sign of diabetic nephropathy. However, it is possible that some diabetic patients could first manifest reduced glomerular filtration rate (GFR) or hypertension. Relatively advanced diabetic renal lesions can be present in some diabetic patients with long-standing normoalbuminuria, and this might indicate increased risk of progression to microalbuminuria and then to overt diabetic nephropathy. The aim of this study was to identify a group of normoalbuminuric type 1 diabetic patients with low GFR and compare them with normoalbuminuric patients with normal GFR. Altogether, 105 normoalbuminuric type 1 diabetic patients with at least 10 years of diabetes duration that had a renal biopsy performed for research purposes were studied. Patients were divided according to GFR into groups with normal (&gt;/=90 ml x min(-1) x 1.73 m(-2)) or reduced (&lt;90 ml x min(-1) x 1.73 m(-2)) GFR. Clinical and renal structural parameters were compared between these two groups. Glomerular structural parameters were estimated by electron microscopic morphometry. The 23 patients with reduced GFR had more advanced diabetic glomerular lesions. The finding of reduced GFR was much more common among female patients, particularly if retinopathy and/or hypertension were also present. This report confirms that reduced GFR occurs among long-standing normoalbuminuric type 1 diabetic patients and is associated with more advanced diabetic glomerular lesions and, probably, with increased risk of progression. For these reasons, we suggest that regular measurements of GFR be performed in long-standing normoalbuminuric type 1 diabetic female diabetic patients, especially in those with retinopathy or hypertension."}, {"id": "pubmed23n0325_18925", "title": "[How I evaluate...diabetic nephropathy. First part: micro- and macroalbuminuria].", "score": 0.009259259259259259, "content": "Diabetic nephropathy (DN) appears in about 30% of patients with type 1 diabetes (D1) and 15 to 60% of patients with type 2 diabetes (D2). It is preceded by microalbuminuria. Microalbuminuria is defined as an albumin excretion rate between 30 and 300 mg/24 h (on a 24-hour urine collection) or between 20 and 200 micrograms/min (on an overnight collection) in at least two out of three consecutive collections made within a 6-month period. Alternative screening techniques use either dipstick (Micral-Test II) or the albumin to creatinine ratio on an early morning urine sample (30-300 mg/g creatinine). Once persistent microalbuminuria is confirmed, 80% of type 1 diabetic patients and 20 to 50% of type 2 diabetic patients will progress to DN. In D2, microalbuminuria also represents a powerful predictor of early mortality from cardiovascular disease. Macroalbuminuria (AER &gt; 300 mg/24 h, corresponding to a total protein excretion &gt; 500 mg/24 h) will eventually lead to a end-stage renal insufficiency within 10 to 20 years. In D2, numerous patients will die from cardiovascular disease before reaching end-stage renal failure. Angiotensin-converting enzyme inhibitors can slow down the evolution toward DN when prescribed when microalbuminuria appears. Screening for microalbuminuria should therefore be a part of the annual clinical assessment in every diabetic patient."}, {"id": "wiki20220301en043_69809", "title": "Diabetic nephropathy", "score": 0.009208373536938373, "content": "Diabetic nephropathy, also known as diabetic kidney disease, is the chronic loss of kidney function occurring in those with diabetes mellitus. Diabetic nephropathy is one of the leading causes of chronic kidney disease (CKD) and end-stage renal disease (ESRD) globally. Protein loss in the urine due to damage to the glomeruli may become massive, and cause a low serum albumin with resulting generalized body swelling (edema) and result in the nephrotic syndrome. Likewise, the estimated glomerular filtration rate (eGFR) may progressively fall from a normal of over 90 ml/min/1.73m2 to less than 15, at which point the patient is said to have end-stage renal disease. It usually is slowly progressive over years."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[0, 113]], "word_ranges": [[0, 18]], "text": "Transfusing this patient and stuffing him with antibiotics is giving him bread for today and hunger for tomorrow."}, "2": {"exist": true, "char_ranges": [[146, 211]], "word_ranges": [[23, 32]], "text": "Androgens and platelet transfusions don't fix the problem either."}, "3": {"exist": true, "char_ranges": [[414, 697]], "word_ranges": [[67, 112]], "text": "we are inclined towards allogeneic bone marrow transplantation if he has an HLA-identical sibling, since this is the treatment of choice according to the protocol of the Spanish Society of Hematology and Hemotherapy for patients under 40 years of age with severe bone marrow aplasia."}, "4": {"exist": true, "char_ranges": [[254, 402]], "word_ranges": [[40, 66]], "text": "An autologous transplant is not reasonable, since the bone marrow of a patient with bone marrow aplasia is less than 25%, so little can be obtained."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Transfusing this patient and stuffing him with antibiotics is giving him bread for today and hunger for tomorrow. We want something more lasting. Androgens and platelet transfusions don't fix the problem either. We are left with the other three options. An autologous transplant is not reasonable, since the bone marrow of a patient with bone marrow aplasia is less than 25%, so little can be obtained. Therefore, we are inclined towards allogeneic bone marrow transplantation if he has an HLA-identical sibling, since this is the treatment of choice according to the protocol of the Spanish Society of Hematology and Hemotherapy for patients under 40 years of age with severe bone marrow aplasia. Correct answer, 3.", "full_answer_no_ref": "Transfusing this patient and stuffing him with antibiotics is giving him bread for today and hunger for tomorrow. We want something more lasting. Androgens and platelet transfusions don't fix the problem either. We are left with the other three options. An autologous transplant is not reasonable, since the bone marrow of a patient with bone marrow aplasia is less than 25%, so little can be obtained. Therefore, we are inclined towards allogeneic bone marrow transplantation if he has an HLA-identical sibling, since this is the treatment of choice according to the protocol of the Spanish Society of Hematology and Hemotherapy for patients under 40 years of age with severe bone marrow aplasia. [HIDDEN]", "full_question": "A 29-year-old patient comes to your office with a diagnosis of severe bone marrow aplasia. What is the treatment of choice?", "id": 180, "lang": "en", "options": {"1": "Periodic transfusions and antibiotics.", "2": "Androgens and platelet transfusions.", "3": "Allogeneic bone marrow transplantation if HLA identical sibling.", "4": "Autologous bone marrow transplantation to avoid rejection.", "5": "Cyclosporin A and antithymocyte globulin."}, "question_id_specific": 232, "type": "HEMATOLOGY", "year": 2013, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0088_5710", "title": "Immunosuppressive therapy versus bone marrow transplantation for children with aplastic anemia.", "score": 0.018362533692722373, "content": "A total of 15 patients 1 to 16 years of age were treated for aplastic anemia (13 of a severe degree) and followed-up for a mean of 24 months (range 2 to 64 months). Six patients had an HLA-matched sibling and underwent allogeneic bone marrow transplantation. Nine patients who lacked a suitable donor were given immunosuppressive therapy. Antithymocyte globulin was the initial treatment for eight of these nine patients. Two patients who failed to respond to antithymocyte globulin were then treated with cyclosporine A. Pretreatment age, hematologic measurements, duration of follow-up, and interval prior to therapy were similar between the two groups. All of the patients receiving bone marrow transplants had a complete response and now have normal blood cell counts. Six of nine patients (67%) responded to antithymocyte globulin and are now transfusion free, although three have mild thrombocytopenia. Both patients given cyclosporine A had a good response and are also transfusion free. Patients who underwent marrow transplantation had a significantly shorter period of transfusion dependence for RBCs (9 v 4 weeks, P less than .005) and platelets (5 v 21 weeks, P less than .05). The patients given immunosuppressive therapy have significantly lesser platelet counts in follow-up but have similar values for both hemoglobin and absolute granulocyte counts. Although HLA-matched bone marrow transplantation leads to a faster and more complete recovery for children with aplastic anemia, immunosuppressive therapy can provide a good outcome for children with this disorder."}, {"id": "pubmed23n0371_15932", "title": "[Second allogenic bone marrow transplantation after late graft rejection in a patient with severe aplastic anemia].", "score": 0.017662620603797072, "content": "Allogeneic bone marrow transplantation (BMT) is the treatment of choice in young patients (pts) with severe aplastic anemia (SAA) who have an HLA identical sibling donor. Late graft rejection to following allogeneic BMT for SAA is a significant clinical problem and is associated with a high risk of mortality. The optimal treatment strategy for patients with late graft rejection after first BMT is still an open question. We report 12-year-old patient with acquired SAA who underwent BMT from his HLA identical sister. BMT was first-line treatment within 3 months of diagnosis. Preparative therapy was Cyclophosphamide (Cy) 200 mg/kg body mass (BM) during 4 days. Graft versus host disease (GVHD) was prevented with Methotrexate (MTX), Methylprednisolone (MPDN) and Cyclosporin A (CsA). After 17 months, during which patient was with normal blood counts and full donor chimaerism, graft rejection occurred. The patient was re-engrafted from the same donor after conditioning with Cy 200 mg/kg BM plus horse antithymocyte globulin (ATG)--2 vials (á 25 mg)/10 kg BM over 4 days. Before the collection, donor's bone marrow was activated with low dose rhGM-CSF (3 micrograms/kg one day). Following a secondary BMT engraftment has sustained. The patient is alive with full donor chimaerism 26 months from secondary transplantation, without acute or chronic GVHD."}, {"id": "pubmed23n0418_8724", "title": "Bone marrow transplantation for patients with acquired severe aplastic anemia using cyclophosphamide and antithymocyte globulin: the experience from a single center.", "score": 0.017273471818926363, "content": "Between 1998 and 2001, 31 (24 male, 7 female) patients with severe aplastic anemia (SAA) and a median age of 19 years (range, 4-39 years) received an allogeneic bone marrow transplantation. Marrow donors were genotypically HLA-identical siblings in 30 cases and a monozygous twin in one case. The median time from diagnosis to bone marrow transplantation was 1 month (range, 0.5-5 months). Conditioning regimen consisted of cyclophosphamide (CY) combined with antithymocyte globulin (ATG), in all patients. For graft-versus-host disease (GvHD) prophylaxis, all patients received methotrexate and cyclosporin. A total of 84% of patients had sustained grafts, whereas 16% rejected grafts between 3 and 20 months after transplantation. Of the five rejecting patients, three are alive with successful second engraftments and two died from infections. Acute grade II-IV GvHD was seen in only 11% of patients. A limited chronic GvHD was seen in one patient. With a median follow-up of 18 months (range, 5-42 months), survival rate was 86% and Karnofsky score was at least 90%. This study confirms the high success rate of the CY/ATG regimen in SAA allografted from an HLA-identical sibling. Early and late graft failure remains a problem and may require modification of this regimen."}, {"id": "pubmed23n0537_19197", "title": "[Hematopoietic stem-cell transplantation in aplastic anemia].", "score": 0.016986496090973704, "content": "Severe aplastic anemia is a rare syndrome characterized by bone marrow failure with cytopenias and hypocellular bone marrow biopsy (usually 10-15%), without blasts or myelodysplasia. The first choice treatment for these patients is allogeneic bone marrow transplantation from a sibling matched for HLA-A, HLA-B and HLA-DR. Unfortunately only 30% of patients have an HLA-matched sibling (a 25% chance per sibling). The alternative treatment for severe aplastic anemia for the rest of the patients (70%) is immunosuppression with antithymocyte globuline and cyclosporine. The evolution of bone marrow transplantation since 1970's has been positive in terms of survival and transplant success (initial overall survival 43% vs. 90% lately, and graft rejection of 29% vs. 4%). The favorable outcome of bone marrow transplantation for severe or very severe aplastic anemia is due to: the use of conditioning with antithymocyte globuline and cyclophosphamide, the use of graft-vs.-host disease prophylaxis with short curse methotrexate and cyclosporine and the use of filtrated and irradiated blood products. For those patients without an HLA-matched related donor the first treatment to use is the immunosuppression with antithymocyte globuline and cyclosporine. Another option emerged in the late 80's is the unrelated bone marrow transplantation, with survival hardly half of the HLA-identical related bone marrow transplants. In our country, the first allogeneic bone marrow transplant was done in the Instituto Nacional de Ciencias Médicas y Nutrición, Salvador Zubirán, in a patient with aplastic anemia, making possible to perform this procedure safely in our country."}, {"id": "pubmed23n0268_2258", "title": "Cyclophosphamide combined with antithymocyte globulin in preparation for allogeneic marrow transplants in patients with aplastic anemia.", "score": 0.015941756103684177, "content": "Graft rejection has been a problem after marrow grafts for patients with aplastic anemia who were conditioned with cyclophosphamide (CY). Rejection lessened when patients were given the marrow donor's peripheral blood buffy-coat cells in addition to the marrow, but this result was achieved at the price of more chronic graft-versus-host disease (GVHD). Results with second transplants suggested that CY alternating with antithymocyte globulin (ATG) was more immunosuppressive than CY alone. Therefore, the current study explored CY and ATG without buffy-coat cell transfusions in 39 patients with aplastic anemia given marrow transplants from HLA-identical family members (siblings in 38 cases, father in 1 case). We hoped both to minimize the risks of graft rejection and of chronic GVHD and to improve survival. Patients were 2 to 52 years of age (median, 24.5); 87% had received previous transfusions, and 41% had therapy with immunosuppressive agents before transplant. They were administered four daily doses of CY (total, 200 mg/kg) alternating with three doses of ATG (total, 90 mg/kg) followed by an HLA-identical marrow graft. Methotrexate and cyclosporine were administered to prevent GVHD. Two patients rejected their grafts (5%), and both were successfully retransplanted. Acute (grade 2 or 3) GVHD occurred in 15% and chronic GVHD in 34% of patients. The actuarial survival rate at 3 years was 92%, which compares favorably to the 72% survival rate in 39 historical patients who were matched with current patients for age and risk factors for rejection and GVHD. CY/ATG is a well-tolerated and effective conditioning program for marrow grafting in aplastic anemia that, when combined with GVHD prevention by methotrexate/cyclosporine, results in excellent survival."}, {"id": "pubmed23n0874_12263", "title": "Management of Aplasia.", "score": 0.015235778009500637, "content": "Prior to the availability of specific treatment for aplastic anaemia (AA) with antithymocyte globulin (ATG) and cyclosporin or bone marrow transplantation (BMT), most patients died within 6 months of presentation, but the survival of patients with severe AA has improved dramatically over the last two decades and many patients now survive long term. Nevertheless, AA still remains a potentially life threatening disease. Because response to treatment with ATG and cyclosporin is delayed for two to three months and sometimes longer, patients usually need intensive transfusional support and careful attention to infection prophylaxis and treatment, particularly fungal infection. It is important to adopt a careful transfusional policy at diagnosis, in particular to help prevent HLA alloimmunisation which results in refractoriness to platelet transfusions, and sensitisation to minor histocompatibility antigens which is thought to mediate graft rejection between HLA identical siblings. The indications for immunosuppressive therapy or HLA identical sibling BMT are fairly clear, but transplantation from volunteer unrelated donors remains a highly controversial and high risk procedure in patients with AA. The effects of haemopoietic growth factors in AA patients have been perhaps not surprisingly disappointing, and not without significant toxicity, with the exception of granulocyte colony stimulating factor (G-CSF) which is well tolerated clinically. G-CSF may be useful in the treatment of severe resistant infections and it may be of benefit when used after treatment with ATG and cyclosporin. "}, {"id": "pubmed23n0725_12052", "title": "Audit of peripheral stem cell transplantation for aplastic anemia in multitransfused infected patients.", "score": 0.015211104684788895, "content": "Allogeneic hematopoietic stem cell transplantation is a curative modality for aplastic anemia; the preferred stem cell source is bone marrow. However, allogeneic peripheral blood stem cell transplantation (PBSCT) used in high-risk patients is associated with higher risk of chronic graft-versus-host disease (GVHD). Our center receives multitransfused, alloimmunized, infected, late referrals for transplant. Forty-one patients of median age 22 years (range 8-37) received allogeneic-PBSCT from human leukocyte antigen (HLA)-matched sibling donors. The median time since diagnosis was 12 months (range 4-65) and median pretransplant transfusions were 37 (range 6-160). Six patients were platelet refractory and one alloimmunized for pan-red blood cell (RBC) antigens. Several patients had pretransplant icterus or renal dysfunction and 26 (63.4%) had unresponsive bacterial/fungal infections. Our conditioning regimen included fludarabine 30 mg/m(2) for 6 days (days -10 to -5), cyclophosphamide 60 mg/kg/d for 2 days (days -6 to -5), and antithymocyte globulin (ATGAM) 30 mg/kg/d for 4 days (day -4 to -1), which was reduced to 2 days in 2 patients. We used standard GVHD prophylaxis with cyclosporine and methotrexate on days 1, 3, 6, 11. The median follow-up period was 29 months (range 6-78) and median engraftment time 10 days (range 8-17). Thirty-one patients (75.6%) were treated for infections, with 20 of these on antifungals for preexisting infections. There were two graft rejections and 10 (24.4%) deaths, with three intracranial hemorrhages, two rejections with infection, three cases of refractory GVHD (acute/overlap syndrome) with cytomegalovirus reactivation, and two invasive fungal infections. Overall incidence of acute GVHD was 39% with 2 grade IV cases. Ten (25%) cases developed chronic GVHD, with extensive GVHD in four. With more experience using shortened course of ATGAM, HLA-matched donor transfusions, and availability of newer antifungals, we have been able to decrease PBSCT-related mortality. Further improvement will be possible with early referrals."}, {"id": "pubmed23n0100_10122", "title": "[Bone marrow transplantation in severe aplastic anemia].", "score": 0.014685756395410043, "content": "Bone marrow transplantations were performed on 15 patients (aged 5-39 years) with severe aplastic anaemia. Twelve patients are alive 76-1930 days (median 668 d) after transplantation, with complete haematopoetic recovery. Total-body radiation with 3.6 Gy in four patients, cyclosporin A administration to ten patients and buffy-coat transfusion to nine patients entirely prevented early rejection. Two patients died of pneumonia (aspergillus; varicella-zoster virus), one patient died of bleeding from a splenic-artery aneurysm. In patients under the age of 40 years with severe aplastic anaemia bone marrow transplantation as early as possible after diagnosis is the treatment of choice if HLA-identical siblings are available as donors. In patients over 40 years treatment should at first be tried with antithymocyte globulin."}, {"id": "pubmed23n0263_16382", "title": "Haemopoietic growth factors in aplastic anaemia: a cautionary note. European Bone Marrow Transplant Working Party for Severe Aplastic Anaemia.", "score": 0.014569256756756757, "content": "We are concerned about the inappropriate use of haemopoietic growth factors in patients with severe aplastic anaemia (SAA). The treatment of choice for this disorder is bone-marrow transplantation from an HLA-identical sibling donor if the patient is younger than 45 years, but it must be done soon after onset before the patient becomes sensitised by multiple red-cell and platelet transfusions. Other patients should receive immunosuppressive therapy with antithymocyte globulin alone or with cyclosporin or oxymetholone. Haemopoietic growth factors may have a role in stimulation of granulopoiesis after immunosuppressive therapy, but there is no evidence that they can correct the underlying stem-cell defect in SAA, and therefore no justification for their use alone in newly diagnosed SAA. Such treatment is harmful because it delays bone-marrow transplantation, or immunosuppressive therapy in older patients and those without suitable donors, thus reducing the chances of a successful outcome."}, {"id": "pubmed23n0284_2966", "title": "[Treatment of severe acquired aplastic anemia].", "score": 0.01375534188034188, "content": "Severe acquired aplastic anaemia is associated with high morbidity and mortality despite improvement in the results obtained both with bone marrow transplantation and with immunosuppressive treatment. Early bone marrow transplantation is the treatment of choice for patients under 45 years of age, if the neutrophil granulocyte count is less than 0.2-0.5 x 10(9)/l and an HLA-identical sibling donor is available. Other patients should receive primary immunosuppression with antithymocyte globulin, cyclosporin and glucocorticoid in combination with granulocyte colony-stimulating factor. If they fail to respond after three months, and a donor is available, such patients may be treated with bone marrow transplantation. However, transplantation with marrow from donors other than HLA-identical siblings is still at an experimental stage."}, {"id": "pubmed23n0076_7463", "title": "Bone marrow transplantation for severe aplastic anemia in children.", "score": 0.013378052457039493, "content": "For young adults and children who have a bone marrow donor who is a genotypic or phenotypic sibling match, bone marrow transplantation is now the preferred treatment for severe aplastic anemia. For those who lack such a matched donor, use of matched unrelated donors and family member donors who are mismatched for a single HLA antigen have been successful and appear to be the treatment of choice. Patients lacking either of these alternatives should receive antilymphocyte globulin, either alone or combined with cyclosporine as a first step. Although the success rate of marrow transplants in our series using mismatched family donors is similar to that following treatment with antilymphocyte globulin, several caveats must be kept in mind. First, the results reported with use of alternative donors must be confirmed with study of larger numbers of patients and longer follow-up. Second, the preparative regimen given prior to bone marrow transplantation destroys the patient's residual bone marrow, whereas antilymphocyte globulin cyclosporine A and androgens do not. The sequence of immunosuppression followed by transplantation with alternative donor marrow should produce greater long-term hematopoietic improvement. Unfortunately, when marrow transplant follows one or more courses of immunosuppressive therapy, nonengraftment is then a problem because of sensitization to blood cell antigens. It should also be kept in mind that studies done in children, especially in those younger than 6 years old, show that these patients respond better to transplantation than to treatment regimens not including marrow transplantation. Therefore, for the child with severe aplastic anemia, every effort should be made to identify a suitable bone marrow donor. Finally, we need to determine the specific components of the conditioning regimen and the constitution of the donor marrow necessary for engraftment and to minimize potential long-term complications, and there should be only a tolerable degree of graft-versus-host disease. Many of the transplant-related problems that plagued us in the 1970s have still not been fully resolved, but many have shown improvement. As we enter the 1990s, increasing the pool of marrow donors for patients with severe aplastic anemia who lack an HLA-matched sibling will continue to be a top priority for research."}, {"id": "wiki20220301en010_97423", "title": "Aplastic anemia", "score": 0.013197383609080415, "content": "Treatment Treating immune-mediated aplastic anemia involves suppression of the immune system, an effect achieved by daily medicine or, in more severe cases, a bone marrow transplant, a potential cure. The transplanted bone marrow replaces the failing bone marrow cells with new ones from a matching donor. The multipotent stem cells in the bone marrow reconstitute all three blood cell lines, giving the patient a new immune system, red blood cells, and platelets. However, besides the risk of graft failure, there is also a risk that the newly created white blood cells may attack the rest of the body (\"graft-versus-host disease\"). In young patients with an HLA-matched sibling donor, bone marrow transplant can be considered as a first-line treatment. Patients lacking a matched sibling donor typically pursue immunosuppression as a first-line treatment, and matched, unrelated donor transplants are considered second-line therapy."}, {"id": "pubmed23n0403_2783", "title": "[Favorable current prognosis after HLA-identical bone marrow transplantation for children with required severe aplastic anemia; evaluation of 30 years of bone marrow transplantation at the Leiden University Medical Center].", "score": 0.012740133429788601, "content": "To evaluate the results of 30 years of allogeneic HLA-identical bone marrow transplantation (BMT) as the treatment for children with acquired severe aplastic anaemia. Retrospective, descriptive. Of all patients who underwent an HLA-identical sibling-donor BMT for severe aplastic anaemia at the Department of Paediatrics, Leiden University Medical Center, in the period 1971-2000, and had a follow-up period of at least 1 year, the medical data were reviewed. The patients were split into 2 groups: patients transplanted before 1989 (n = 24), and patients who had their BMT from 1989 onwards (n = 20). This was due to a change in the treatment policy, namely a reduction in the period between diagnosis and BMT, resulting in fewer blood transfusions as well as changes in the prophylaxis against graft-versus-host disease (GvHD) from 1989 onwards (combination therapy using methotrexate and cyclosporin). There was an increase in the 1-year actuarial survival rate from 67% in the period before 1989 to 90% thereafter. The incidence of GvHD has significantly decreased since the introduction, in 1989, of the combination therapy using methotrexate and cyclosporin, with only 1/20 patients suffering from acute GvHD versus 13/24 prior to 1989 (p = 0.002). No patients acquired chronic GvHD after 1989, whereas before 1989, 10 patients had acquired this (p = 0.001). The prognosis of allogeneic HLA-identical sibling transplantation for paediatric patients with severe aplastic anaemia has considerably improved over the last 30 years due to improved supportive care, a significant decrease in GvHD and a shorter period between diagnosis and BMT, with the result that less blood transfusions have been required and less sensitisation has occurred. The long-term survival chance has increased to 90%."}, {"id": "pubmed23n0972_1075", "title": "[Pure red cell aplasia following the rapid reduction and discontinuation of cyclosporine for mixed chimerism after allogeneic bone marrow transplantation].", "score": 0.012588543270711426, "content": "A 19-year-old male with therapy-related myelodysplastic syndrome underwent allogeneic bone marrow transplantation with reduced-intensity conditioning from his HLA-identical sibling whose ABO blood type exhibited major incompatibility with the patient. After post-transplantation 1 month, chimerism analysis of the bone marrow revealed mixed chimerism with 30% of recipient cells, and after post-transplantation 3 months, complete remission was maintained; however, recipient granulocytes were elevated up to 50% per the chimerism analysis. Next, pancytopenia developed following the rapid discontinuation of the immunosuppressive agent. Although neutrophils and platelets spontaneously recovered, anemia progressed. Based on severe erythroid hypoplasia in the bone marrow and the elevation of anti-ABO isohemagglutinin against donor-derived red blood cells, the patient was diagnosed with pure red cell aplasia (PRCA) following hematopoietic cell transplantation. Because complete chimerism was attained at the PRCA onset even for B cells, we decided to conservatively manage PRCA with only red blood cell transfusion. Notably, after 2 months of the PRCA onset, anemia improved. This case suggests that the therapeutic strategy for PRCA following hematopoietic cell transplantation should be determined by considering the status of each patient, including chimerism."}, {"id": "pubmed23n0640_17350", "title": "[Twenty years of severe aplastic anemia treatment at Department of Hematology, University Department of Medicine, Zagreb University Hospital Center, Zagreb, Croatia].", "score": 0.012542266257765196, "content": "Aplastic anemia is a bone marrow disease characterized by marrow aplasia and pancytopenia. Because hematopoietic stem cell transplantation (HSCT) cures severe aplastic anemia (SAA), it is the treatment of choice for younger patients. For many years, antithymocyte globulin (ATG) has been standard immunosuppressive therapy for those aplastic anemia patients that have no HLA matched related donor. ATG significantly improves aplastic anemia outcome, especially when combined with cyclosporine (CSP). The response rate varies from 40% to 70% and long-term survival is comparable with patients receiving marrow transplant. From 1983 until 2006, 46 SAA patients received HLA identical sibling marrow graft. In the same period, 50 patients received standard immunosuppressive therapy combined from horse or rabbit ATG, 6 methyl prednisolone and cyclosporine. Out of 46 transplant patients, 27 received a combination of cyclophosphamide and thoraco-abdominal irradiation. The overall probability of survival for SAA patients that underwent marrow grafting is 51%, and for patients receiving immunosuppressive treatment 20%. We analyzed a cohort of patients receiving treatment after 1990 and found the probability of survival to be 64% for bone marrow transplanted patients and 36% for patients receiving immunosuppression. Infection is the main cause of death in both groups. In conclusion, we documented improving results using ATG in patients with SAA."}, {"id": "pubmed23n0053_15533", "title": "Treatment of adults with severe aplastic anemia: primary therapy with antithymocyte globulin (ATG) and rescue of ATG failures with bone marrow transplantation.", "score": 0.01226696495152871, "content": "To evaluate a policy of immunosuppression with antithymocyte globulin (ATG) as primary therapy for adults with severe aplastic anemia (SAA) regardless of the availability of an HLA-identical bone marrow donor. Thirty-one consecutive adults with SAA who satisfied the age criteria for allogeneic bone marrow transplantation (BMT) (age less than 51 years) were treated with ATG 20 mg/kg/day for 10 days along with high-dose corticosteroids. Patients with an HLA-identical donor received a transplant if they did not respond to ATG or if they developed life-threatening complications during or soon after ATG administration. Eight patients with no response to ATG were also treated with oral cyclosporine 12.5 mg/kg/day. Eleven patients had a complete and five a partial response to ATG; two patients improved with cyclosporine treatment, resulting in an overall response rate of 58% to immunosuppression. Nine of 14 patients with donors received a BMT: seven because they did not respond to ATG and two because of serious infections. Seven grafts were obtained from related and two from unrelated donors. There was no significant difference in survival between those with and without a related HLA-identical donor (log-rank p value = 0.969). At a median follow-up of 58 months, 26 of 31 are alive with an actuarial survival of 80% at 5 years. Two patients died of infection, two died from complications of BMT, and one remains transfusion-dependent. One patient died of refractory leukemia at 30 months; one patient relapsed with hypoplasia 95 months after initial therapy with ATG. He showed a complete response to treatment with cyclosporine. No other late hematologic events have occurred. This treatment approach resulted in the restoration of hematopoiesis and independence from transfusion in 80% of patients with SAA entered into the study. The efficacy of allogeneic BMT in salvaging cases in which ATG failed does not appear to be compromised. Follow-up for the development of clonal hematologic disorders remains an important part of this treatment policy."}, {"id": "pubmed23n0479_16799", "title": "Long-term outcome after bone marrow transplantation for severe aplastic anemia.", "score": 0.011538222305156436, "content": "From January 1978 to December 2001, 133 patients with severe aplastic anemia (SAA) underwent non-T cell-depleted allogeneic bone marrow transplantation from an HLA-identical sibling donor, at the Hospital Saint Louis using either the combination of cyclophosphamide (Cy) and thoracoabdominal irradiation (TAI; n=100) or Cy and antithymocyte globulin (ATG; n=33), as a conditioning regimen. With 13.6 years of follow-up, the 10-year survival estimate was 64%. Four factors were associated with lower survival: older age, use of Cy-TAI, any form of treatment prior to transplantation (either androgens or immunosuppressive therapy, [IST]), and grade II to IV acute graft-versus-host disease (GvHD). TAI was the sole factor associated with the occurrence of acute GvHD. The risk of cancers (15-year cumulative incidence, 10.9%) was associated with older age and with the use of cyclosporine as IST before transplantation. Cumulative incidences and risk factors of nonmalignant late effect including avascular osteonecrosis and late bacterial, viral, and fungal infection were also analyzed. Improved results using Cy-ATG as conditioning can lead to more than 90% chance of cure in patients with SAA. Even if, in our experience, the role of Cy-ATG versus that of Cy-TAI remained inextricably related to the year of transplantation, the major detrimental role of the GvHD disease in the long-term outcome and its relation to TAI supports avoidance of irradiation in the conditioning regimen. Furthermore, avoidance of any IST before transplantation in patients with a sibling donor is a prerequisite for attaining such excellent results."}, {"id": "pubmed23n0305_18713", "title": "Conditioning with cyclophosphamide/antithymocyte globulin for allogeneic bone marrow transplantation from HLA-matched siblings in children with severe aplastic anemia.", "score": 0.011535947712418301, "content": "Graft rejection has been a problem after bone marrow transplantation for patients with severe aplastic anemia (SAA). Ten children with SAA were conditioned for bone marrow transplantation from HLA-identical siblings, using cyclophosphamide (CY, 50 mg/kg) plus antithymocyte globulin (ATG, 15 mg/kg) for 4 successive days. Marrow was infused 36 h after the last dose of CY. Cyclosporin A and methotrexate were administered as graft-versus-host disease (GVHD) prophylaxis. All patients achieved durable engraftment at follow-up of 7-41+ months (mean, 25) without significant GVHD. Since investigators have used different sources, doses, and time schedules of ATG, we compared our results with other published reports. We conclude that CY/ATG conditioning is well tolerated and effective in children with SAA."}, {"id": "wiki20220301en010_35425", "title": "Myelodysplastic syndrome", "score": 0.01106751913203526, "content": "Treatments may include supportive care, drug therapy, and hematopoietic stem cell transplantation. Supportive care may include blood transfusions, medications to increase the making of red blood cells, and antibiotics. Drug therapy may include the medications lenalidomide, antithymocyte globulin, and azacitidine. Certain people can be cured with chemotherapy followed by a stem-cell transplant from a donor. About seven per 100,000 people are affected, with about four per 100,000 people newly acquiring the condition each year. The typical age of onset is 70 years. The outlook depends on the type of cells affected, the number of blasts in the bone marrow or blood, and the changes present in the chromosomes of the affected cells. The typical survival time following diagnosis is 2.5 years. The conditions were first recognized in the early 1900s. The current name came into use in 1976. Signs and symptoms"}, {"id": "pubmed23n0111_77", "title": "A comparison between ALG and bone marrow transplantation in treatment of severe aplastic anemia.", "score": 0.0110551229195297, "content": "One hundred patients with severe aplastic anemia were treated and evaluated in a prospective study at our hospital between January 1976 and October 1983. 28 patients had a HLA-identical sibling donor and were treated with bone marrow transplantation. 72 patients without a HLA-identical sibling donor were given antilymphocyte globulin followed by oral low dose androgen therapy. One and a half years to nine years after treatment 13 patients (46%) survive in the transplant group and 53 patients (74%) survive in the second group. All except one in the second group have self-sustaining hematopoiesis without need for transfusions. There is one major difference between the two therapies. Marrow transplantation restores bone marrow function completely and no late hematological complications have been seen in this group. The majority of patients treated with antilymphocyte globulin in contrast have residual abnormalities of hemopoiesis: macrocytosis, mild granulocytopenia and mild thrombocytopenia. Relapse (11 of 72 patients) and clonal hematological disorders, such as paroxysmal nocturnal hemoglobinuria (4 patients) and leukemia (one patient) can occur years after complete bone marrow reconstitution with antilymphocyte globulin. These late disorders are of concern. In spite of this we conclude that antilymphocyte globulin treatment is an effective therapy with low early mortality and morbidity and a high chance for a long sustained remission. Results are better or at least equivalent to bone marrow transplantation and patients with donors should be given the option of transplantation or antilymphocyte globulin."}, {"id": "pubmed23n0289_8812", "title": "[Urgent allogeneic bone marrow transplantation using a preparative regimen of cyclophosphamide anti-human thymocytes rabbit globulin in a patient with severe aplastic anemia with pneumonia].", "score": 0.010768669169795578, "content": "A 16-year-old girl was diagnosed as having severe aplastic anemia (SAA) had received emergency complicated by sever pneumonia. She had an HLA-identical younger brother and been urgently transplantation with her brother's marrow following a preparative regimen of CY+rabbit antithymocyte globulin (ATG). Granulocyte transfusions carried out before and after the transplant prevented exacerbation of the pneumonia. The pneumonia was cured in association with the hematopoietic recovery after BMT. No signs or symptoms of acute or chronic graft-versus-host disease were recognized and her hematological data are normal. The rabbit ATG was thought to be effective in preventing rejection and could be used in the preparative regimen instead of total body irradiation."}, {"id": "pubmed23n0073_6499", "title": "Treatment of aplastic anemia.", "score": 0.01025801244499987, "content": "Survival of patients with aplastic anemia after immunosuppressive therapy with ATG/ALG ranges from 35% to 60%. However, long-term follow-up on these patients has indicated a high frequency of hematologic complications, including PNH, myelodysplasia, ANL, and recurrent aplasia. In contrast to immunosuppressive therapy, allogeneic marrow transplantation results in cure of aplasia. Problems initially limiting the success of HLA-matched allogeneic marrow transplants included graft rejection and complications associated with acute and chronic GVHD. Infusion of donor buffy coat cells along with marrow or alternatively more intensive immunosuppressive regimens containing irradiation have substantially decreased the risk of rejection. However, buffy coat infusion increases the incidence of chronic GVHD and irradiation treatment adds to toxicity of the conditioning regimen as well as producing long-term complications. The incidence and severity of acute GVHD have been significantly decreased by the use of MTX/CSP as GVHD prophylaxis; however, this regimen has had no impact on the incidence of chronic GVHD. Long-term survival in multiply transfused patients after HLA-identical marrow transplantation is on the order of 60% to 70%; survival in untransfused patients approximates 80%. Patients less than age 18 transplanted on protocols currently active in Seattle have greater than 90% survival. Further increases in survival must come from improvement in preventing and treating chronic GVHD. Patients diagnosed with aplastic anemia should have rapid HLA typing performed to identify possible marrow donors. Transfusions from prospective marrow donors should be avoided and the patient referred to a major treatment center. We continue to recommend allogeneic marrow transplantation for patients with severe aplastic anemia who are less than 40 years old and who have HLA-identical related donors. Immunosuppressive therapy should be tried first in patients without HLA-matched donors and for patients over the age of 40. HLA-mismatched marrow transplantation and use of unrelated marrow donors for severe aplastic anemia remain areas of active research."}, {"id": "wiki20220301en022_39010", "title": "Hematopoietic stem cell transplantation", "score": 0.01017156862745098, "content": "Bone-marrow transplantation usually requires that the recipient's own bone marrow be destroyed (myeloablation). Prior to the administration of new cells (engraftment), patients may go for several weeks without appreciable numbers of white blood cells to help fight infection. This puts a patient at high risk of infections, sepsis, and septic shock, despite prophylactic antibiotics. However, antiviral medications, such as acyclovir and valacyclovir, are quite effective in prevention of HSCT-related outbreak of herpetic infection in seropositive patients. The immunosuppressive agents employed in allogeneic transplants for the prevention or treatment of graft-versus-host disease further increase the risk of opportunistic infection. Immunosuppressive drugs are given for a minimum of six months after a transplantation, or much longer if required for the treatment of graft-versus-host disease. Transplant patients lose their acquired immunity, for example immunity to childhood diseases such"}, {"id": "wiki20220301en399_15277", "title": "High-dose chemotherapy and bone marrow transplant", "score": 0.009900990099009901, "content": "High-dose chemotherapy and bone marrow transplant for other cancers While the high-dose chemotherapy and bone marrow transplant treatment is known for its impact on breast cancer, the treatment is presently used to treat other types of cancer, including testicular cancer, neuroblastoma, multiple myeloma, and various types of leukemias and lymphomas, like Hodgkin and non-Hodgkin Lymphoma. There are two types of stem cell (bone marrow) transplants: autologous stem cell transplant, where the person's own stem cells are collected, frozen, and stored before the chemotherapy regimen and transfused back into their body by IV after chemotherapy, and allogeneic stem cell transplant, where the stem cells come from a donor that matches the person's HLA type to prevent the risk of graft-versus-host disease."}, {"id": "pubmed23n0347_22693", "title": "[Experience in the use of allogeneic bone marrow transplantation in severe forms of aplastic anemia at the Byelorussian hematological center].", "score": 0.009900990099009901, "content": "Investigation of the response to antilymphocytic globulin (ALG) and transplantation of allogenic bone marrow (TABM) in patients with a severe form of aplastic anemia (AA). 15 patients were treated for severe AA in 1995-1997. 8 patients of group 1 were given ALG/cyclophosphamide with subsequent TABM from HLA-identical sib donor. To prevent graft versus host reaction the patients took cyclosporin A (CSA) + prednisolone. Those who had no sib donor (7 patients) were treated with ALG/CSA (group 2). A complete remission at 7-30-month follow-up was observed in 6 patients of group 1. One patient died of infectious complications in rejection of the transplant and one patient died of acute graft versus host reaction. None of group 2 patients achieved the remission. One patient died of infectious complications. The others need continuous hemotransfusion therapy. In spite of high probability of early complications after TABM, TABM-subjected patients are more likely to achieve a complete remission and recurrence-free survival than those given immunosuppressive therapy alone."}, {"id": "wiki20220301en093_6577", "title": "X-linked severe combined immunodeficiency", "score": 0.00980392156862745, "content": "Bone marrow transplantation (BMT) is a standard curative procedure and results in a full immune reconstitution, if the treatment is successful. Firstly, a bone marrow transplant requires a human leukocyte antigen (HLA) match between the donor and the recipient. The HLA is distinct from person to person, which means the immune system utilizes the HLA to distinguish self from foreign cells. Furthermore, a BMT can be allogenic or autologous, which means the donor and recipient of bone marrow can be two different people or the same person, respectively. The autologous BMT involves a full HLA match, whereas, the allogenic BMT involves a full or half (haploidentical) HLA match. Particularly, in the allogenic BMT the chances of graft-versus-host-disease occurring is high if the match of the donor and recipient is not close enough. In this case, the T-cells in the donor bone marrow attack the patient's body because the body is foreign to this graft. The depletion of T-cells in the"}, {"id": "pubmed23n0672_20342", "title": "[The use of allogeneic bone marrow transplantation and immunosuppressive therapy in the treatment of patients with acquired aplastic anemia].", "score": 0.00980392156862745, "content": "To evaluate the efficiency of related and unrelated allogeneic bone marrow transplantation (alloBMT) versus immunosuppressive therapy (IST) in patients with aplastic anemia (AA) having no HLA-compatible bone marrow donor. The study covered 61 patients (34 men and 27 women) diagnosed as having acquired AA. Of them, 51 patients were diagnosed as having severe AA, 5 had supersevere AA, and 5 had non-severe AA. Combined IST (antithymocyte globulin (ATG) + cyclosporin A (CsA)) was used in 43 patients; allo-BMT was performed in 18. The basic types of ATG (ATGAM (Pfizer), thymoglobulin (Genzim), ATG (Fresenius), and goat antilymphocyte globulin (ALG) (Research Institute of Gerontology, Ministry of Health of the Russian Federation) were administered. CsA was given in a dose of 5 mg/kg/day. The standard conditioning regimen (ATGAM + cyclophosphanum) and fludarabine-containing (fludarabine + cyclophosphanum + ATG; busulfan + fludarabine + ATG) programs were used in the allo-BMT group. A combination of CsA and metothrexate was given to prevent a graft-versus-host reaction. Among the IST-receiving patients, overall survival (OS) was 71%. After the first course of IST by follow-up month 6, the response rate was 74%. The second course of IST was performed in 7 patients unresponsive after the first-line IST and in 8 patients with recurrent AA. After the second course of IST, the response rate was 46.7%. Four patients who failed to achieve remission after 2 courses of IST received its third course. A complete response was obtained in 3 patients. In 18 patients following allo-BMT (related and unrelated), OS was 86%; event-free survival was 65. In 12 patients after related allo-BMT, OS was 91.7%. Related allo-BMT is the method of choice if there is a HLA-compatible sibling. If there are contraindications to it or no related donor, IST with ATG + CsA is indicated. Ineffective IST is an indication for unrelated allo-BMT that may be recommended as life-saving therapy for young patients under 40 years of age."}, {"id": "wiki20220301en102_21502", "title": "Beta thalassemia", "score": 0.009708737864077669, "content": "Treatment Beta thalassemia major Affected children require regular lifelong blood transfusions. Bone marrow transplants can be curative for some children. Patients receive frequent blood transfusions that lead to or potentiate iron overload. Iron chelation treatment is necessary to prevent damage to internal organs in cases of iron overload. Advances in iron chelation treatments allow patients with thalassemia major to live long lives with access to proper treatment. Popular chelators include deferoxamine and deferiprone. The oral chelator deferasirox was approved for use in 2005 in some countries,. Bone marrow transplantation is the only cure and is indicated for patients with severe thalassemia major. Transplantation can eliminate a patient's dependence on transfusions. Absent a matching donor, a savior sibling can be conceived by preimplantation genetic diagnosis (PGD) to be free of the disease as well as to match the recipient's human leukocyte antigen (HLA) type."}, {"id": "pubmed23n0339_6187", "title": "Late graft failure 8 years after first bone marrow transplantation for severe acquired aplastic anemia.", "score": 0.009708737864077669, "content": "A 14-year-old patient with acquired very severe aplastic anemia (VSAA) underwent bone marrow transplantation (BMT) from his HLA-identical brother. Preparative therapy was cyclophosphamide (CY) 200 mg/kg over 4 days. GVHD prophylaxis was with cyclosporin A (CsA) for a year. After an 8 year follow-up during which the patient was well with normal blood counts, graft failure occurred. At this time marrow chimerism studies demonstrated that 85% of hemopoiesis was of recipient origin. The patient was re-engrafted from the same donor after conditioning with CY 200 mg/kg over 4 days plus rabbit antithymocyte globulin (ATG) 3.5 mg/kg/day for 3 days. After 140 days follow-up he has a normal blood count. The possible causes of the graft failure are discussed. This case demonstrates that, although rarely, very late graft failure may occur after BMT for AA and highlights the need for long-term monitoring even in apparently successfully transplanted patients."}, {"id": "wiki20220301en000_105158", "title": "Chemotherapy", "score": 0.009615384615384616, "content": "Immunosuppression and myelosuppression Virtually all chemotherapeutic regimens can cause depression of the immune system, often by paralysing the bone marrow and leading to a decrease of white blood cells, red blood cells, and platelets. Anemia and thrombocytopenia may require blood transfusion. Neutropenia (a decrease of the neutrophil granulocyte count below 0.5 x 109/litre) can be improved with synthetic G-CSF (granulocyte-colony-stimulating factor, e.g., filgrastim, lenograstim). In very severe myelosuppression, which occurs in some regimens, almost all the bone marrow stem cells (cells that produce white and red blood cells) are destroyed, meaning allogenic or autologous bone marrow cell transplants are necessary. (In autologous BMTs, cells are removed from the person before the treatment, multiplied and then re-injected afterward; in allogenic BMTs, the source is a donor.) However, some people still develop diseases because of this interference with bone marrow."}, {"id": "pubmed23n0631_17651", "title": "[Results of immunosupressive therapy in children with severe aplastic anaemia. Report of the Polish Paediatric Haematology Group].", "score": 0.009615384615384616, "content": "Bone marrow transplantation from HLA identical family donors is the treatment of choice for children with severe aplastic anaemia (SAA). When there is no donor available, combined immunosuppressive therapy is given. evaluation of results of immunosupressive therapy in children with severe aplastic anaemia. SAA was diagnosed in 105 children (42 girls, 73 boys), aged 2-18 years, in the eleven haematological centres in Poland, between 1993-2007. All patients received the Severe Aplastic Anaemia Working Party of the EBMT protocol which included: antilymphocyte globulin or antithymocyte globulin, cyclosporin A, prednisolone. Granulocyto- or granulocytomacrophagic-cell stimulation factor was additionally administered during deep neutropenia. Haematological response was evaluated on day 84 or 112 and 180 of the therapy. complete remission occurred in 53 patients (51.5%), partial remission in 27 (24.7%), no response was obtained in 25 children (23.8%) on day 180, of the therapy. Period of observation was from 12 months to 12.5 years. During this time relapse occurred in 10 patients (9.5%). We observed 22 deaths: 8 early, during the first 3 months of IS and 14 after the first 3 months of immunosuppresive therapy (IS). At present 70 children (66.6%) are in first remission with lasts from 12 months to 12.5 years. The survival at 12.5-years is 78.6%. During the 12.5 years of follow-up we had two cases with a late clonal complication (PNH and MDS). Transformation to acute nonlymphoblastic leukaemia was observed in two of our patients. 1. Immunosuppresive therapy (IS) in children with SAA, without bone marrow family donors, is more effective after introduction of combined IS (12.5 years survival in this study was 80% for children with very severe aplastic anaemia (v SAA). 2. In our studies among the children followed up after IS therapy, there were: 1 case of periodic nocturnal haemoglobinuria (PNH), 1 case of myelodysplastic syndrome (MDS) and 2 cases of myeloid leukaemia (probability of incidence was 3.8%)."}, {"id": "wiki20220301en399_15255", "title": "High-dose chemotherapy and bone marrow transplant", "score": 0.009523809523809525, "content": "Most women could not afford to pay for the treatment themselves, due to high cost of US $50,000 to $400,000 per patient. As long as HMOs and other insurers regarded the regime as experimental or investigational, there was no contractual obligation to cover it. While, in the mid-1980s, fewer than 100 bone marrow transplants a year were performed on breast cancer patients, the uptake of HDC/BMT increased six-fold between January 1, 1989 and June 30, 1995. Between those dates 19,291 autotransplants were reported to the Autologous Blood and Marrow Transplant Registry; 5,886 were for breast cancer. After 1992, breast cancer was the most common indication for autotransplant. Only 11% of women with stage 2/3 disease, and less than one percent of those with stage 4 disease, participated in randomized trials. The International Bone Marrow Transplant Registry estimated that at least 4,000 women were treated with HDC/BMT from 1989 through 1993, with fewer than 10% doing so within trials. Based"}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[190, 536]], "word_ranges": [[36, 96]], "text": "there is a warning sign: there is no arterial pulse and the burn is circumferential to the limb. This is an emergency that, if allowed to evolve, will compromise the viability of the affected limb and end in amputation: an emergency escharotomy must be performed in order to relieve the pressure of the third space on the arterial vascular trunk."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The question is tricky: it speaks of a burn of 10% of the body surface and the treatment of choice for second degree burns less than 20% of the BSA should be topical and monitored. However, there is a warning sign: there is no arterial pulse and the burn is circumferential to the limb. This is an emergency that, if allowed to evolve, will compromise the viability of the affected limb and end in amputation: an emergency escharotomy must be performed in order to relieve the pressure of the third space on the arterial vascular trunk.", "full_answer_no_ref": "The question is tricky: it speaks of a burn of 10% of the body surface and the treatment of choice for second degree burns less than 20% of the BSA should be topical and monitored. However, there is a warning sign: there is no arterial pulse and the burn is circumferential to the limb. This is an emergency that, if allowed to evolve, will compromise the viability of the affected limb and end in amputation: an emergency escharotomy must be performed in order to relieve the pressure of the third space on the arterial vascular trunk.", "full_question": "A young man comes to the emergency room with a second-degree flame burn of 10% of the body surface, affecting the right arm in an extensive and circular manner. There is no arterial pulse in the hand measured by Doppler. What is the treatment of choice?", "id": 270, "lang": "en", "options": {"1": "Occlusive sulfadiazine-arginine cures and depth assessment at one week.", "2": "Lymphatic drainage and assess a vascular by-pass.", "3": "Escharotomy or emergency decompression incisions.", "4": "Expectant management.", "5": "Amputation of the extremity."}, "question_id_specific": 135, "type": "DERMATOLOGY AND PLASTIC SURGERY", "year": 2015, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en173_27476", "title": "Escharotomy", "score": 0.01238082627118644, "content": "Neurovascular integrity should similarly be monitored frequently and in a scheduled manner. Capillary refilling time, Doppler signals, pulse oximetry, and sensation distal to the burned area should be checked every hour. Limb deep compartment pressures should be checked initially to establish a baseline. Subsequently, any increase in capillary refill time, decrease in Doppler signal, or change in sensation should lead to rechecking the compartment pressures. Compartment pressures greater than 30 mm Hg should be treated by immediate decompression via escharotomy and fasciotomy, if needed."}, {"id": "Surgery_Schwartz_12757", "title": "Surgery_Schwartz", "score": 0.011869747899159663, "content": "explored to evaluate for bleeding points. One must be very cautious if attempting to ligate these to ensure that adjacent structures such as nerves are not included in the ligature.The hand must be evaluated for adequacy of perfusion to the hand as a whole as well as the individual digits. Capillary refill, turgor, Doppler signal, and bleeding to pinprick all pro-vide useful information regarding vascular status. The finger or hand with vascular compromise requires urgent operative explo-ration. Unlike the complete amputation, in which the amputated part can be cold preserved (see later section, “Amputation and Replantation”), devascularization without amputation produces warm ischemia, which is tolerated only for a matter of hours.For the noncritical vascular injury, two treatment options exist. Simple ligation will control hemorrhage. At least one of the palmar arterial arches is intact in 97% of patients, so this will usually not compromise hand perfusion.5 Each digit also has two"}, {"id": "wiki20220301en062_34434", "title": "Ankle–brachial pressure index", "score": 0.011416490486257928, "content": "A Doppler ultrasound blood flow detector, commonly called Doppler wand or Doppler probe, and a sphygmomanometer (blood pressure cuff) are usually needed. The blood pressure cuff is inflated proximal to the artery in question. Measured by the Doppler wand, the inflation continues until the pulse in the artery ceases. The blood pressure cuff is then slowly deflated. When the artery's pulse is re-detected through the Doppler probe the pressure in the cuff at that moment indicates the systolic pressure of that artery. The higher systolic reading of the left and right arm brachial artery is generally used in the assessment. The pressures in each foot's posterior tibial artery and dorsalis pedis artery are measured with the higher of the two values used as the ABI for that leg. Where PLeg is the systolic blood pressure of dorsalis pedis or posterior tibial arteries and PArm is the highest of the left and right arm brachial systolic blood pressure"}, {"id": "pubmed23n0949_5645", "title": "Pressure guided surgery of compartment syndrome of the limbs in burn patients.", "score": 0.009900990099009901, "content": "Compartment syndrome is a serious complication of high voltage electrical burns, limb carbonization and deep circular burns with delayed escharotomy. Without treatment, ischemic tissue damage leads to irreversible necrosis. Treatment is emergency surgical decompression. The burned patient is usually not searchable and cannot always be readily examined because of bulky dressings; diagnosis of compartment syndrome is always hard to make. The pressure transducer used in central arterial catheters is easy available. We used it to measure pressure in muscular compartments. We measured compartment pressure three times at different depths in all cases of electrical burn, carbonization and deep circumferential burns with delayed escharotomy. We also took the pressure in the uninjured limb. The pressure assessment device was composed of a blood pressure transducer commonly used in arterial catheters for arterial pressure monitoring with three connecting branches. The first branch was connected to the 'arterial pressure exit' in the monitoring device. The second, an IV tube, was connected to one litre of physiological serum in a pressure bag inflated to 200 mmHg. The third, also an IV tube with a sterile extension cable, was directly connected to an 18G standard straight needle to be inserted in the tissues for which interstitial pressure had to be measured. In patients with thermal burns, we measured pressure before and after escharotomy. Threshold intracompartmental pressure was 35 mmHg. We carried out pressure assessment of all muscular compartments during and at the end of surgery. The pressure transducer provides a pressure value in all muscular compartments with a time of installation and measuring of less than 5 minutes. Sensitivity is measured at +/- 1 mmHg. Operation is simple, non-operator dependent, and accessible to medical and paramedic teams. The pressure transducer allows accurate diagnosis of early or established compartment syndrome. It requires no additional equipment and its application does not delay therapeutic management. Its use helps with fasciotomy decision, especially after escharotomy, guides the surgeon in the exploration of different compartments and verifies the effectiveness of surgery."}, {"id": "pubmed23n0536_5544", "title": "Revascularization in distal lesions of upper extremity arteries.", "score": 0.009900990099009901, "content": "A study was made of the course of ischemia and hemodynamic disorders in 53 patients with occlusion of the distal arterial system of the upper extremities. Circulation was examined by ultrasound Doppler, tetrapolar rheology, transcutaneous oxymetry and angiography. Critical ischemia was discovered in 58.5% of patients. In occlusion of one of the forearm arteries, extremity circulation was made for. Circulatory decompensation was recorded in occlusion of both forearm arteries with intact distal bed and non-functioning palmar arches of the hand as well as in lesions of hand and finger arteries. All 53 patients were operated on, 66 operations were accomplished using magnifying optic appliances. Provided the distal bed was well preserved, use was made of direct revascularization techniques (36) whereas non-standard methods were employed in the event of its obliteration: arterialization of the outlets of the subcutaneous veins of the hand and autotransplantation of the greater omentum (30). Beneficial results were obtained in 91.5% and unsatisfactory in 8.5% of patients. The lethality accounted for 1.2% while the incidence of amputations was quoted as 5.7%. Good results offer the period as long as 5 years were well preserved in 87.6% of patients. Our experience indicates the efficacy of the use of revascularization techniques for the treatment of different versions of occlusion of the distal segments of upper extremity arteries."}, {"id": "wiki20220301en003_138800", "title": "Paramedic", "score": 0.00980392156862745, "content": "Thorocostomy and pericardiocentesis to relieve pneumothorax and pericardial tamponade Intravenous (IV) and intraosseous (IO) cannulation Oxygen administration and positive pressure ventilation via bag-valve-mask, CPAP device, or ventilator Fluid resuscitation Administration of emergency drugs/medications (see section below) Bleeding control and management of shock Spinal injury management, including immobilization and safe transport Fracture management, including assessment, splinting, and dislocation reduction Obstetrics, including assessment, childbirth, and recognition of and procedures for obstetrical emergencies such as breech presentation, cord presentation, and placental abruption Management of burns, including classification, estimate of surface area, recognition of more serious burns, and treatment Triage of patients in a mass casualty incident Surgical procedures such as field amputation, escharotomy, or thorocotomy (if trained and credentialed)"}, {"id": "pubmed23n0349_20012", "title": "[Prevention of extremities amputation in patients with diabetic foot complications].", "score": 0.00980392156862745, "content": "In order to decrease the number of amputations for \"diabetic foot\", these patients should undergo elective or delayed operations. It is obligatory before the operation to correct carbohydrate metabolism and hemodynamics. Sodium succinate in combination with conventional angioprotective treatment is used for this purpose. Microcirculation is evaluated using oxymonitor ISM-2 and tetrapolar rheography. Blood flow disturbances are evaluated according to ultrasound dopplerography data. Operations are performed with the use of a primary or delayed suture at the definite level of the extremity where oxygen tension of the skin is not lower than 33 mm Hg, and the index of minute blood flow--not lower than 1.8 ml/min per 100 cm3 of the tissue. When operating on the foot it is obligatory to leave loose excessive brims of tissues to facilitate the placement of broad-grip sutures without tension. It is advisable to use through flowing--aspiration drainage and to perform surgical treatment of the deep phlegmon of the foot through club-shaped approach."}, {"id": "pubmed23n0114_15358", "title": "Hand burns in children under 5 years of age.", "score": 0.009708737864077669, "content": "In order to evaluate the epidemiology and functional results of hand burns in young children, 92 consecutive patients (126 hand burns) under age 5 years admitted to a Burn Center were reviewed. Scald burns (49 per cent) were most common, followed by flame (34 per cent), contact (14 per cent) and electrical burns (3 per cent). The child was left unattended by an adult in 53 per cent of cases and documented abuse was present in 6 per cent. The mean total body surface area (TBSA) burned was 17 per cent, and 77 patients (85 per cent) had additional burns in other areas (arms 34 per cent, legs 31 per cent, chest 29 per cent and face 27 per cent). Palmar burns occurred in 24 hands (19 per cent), dorsal in 41 (33 per cent), while both surfaces were burned in 61 (48 per cent). Joints involved included the MP in 96 (76 per cent), PIP in 87 (69 per cent) and DIP in 80 (63 per cent). The depth was superficial partial thickness in 53 (47 per cent), deep partial in 55 (44 per cent), and full thickness in 18 hands (14 per cent); a total of 29 hands were grafted (15 deep partial and 14 full thickness). Escharotomies were required in 12 hands (9 per cent) (9 flame and 3 scald) and partial amputation of digits was required in 3 (2 per cent). Follow-up was available in 46 hands from 7 to 120 months (mean 39 months). Partial thickness burns (34) healed with normal (32) or near-normal (2) hand function and developmental delay occurred in one patient.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0839_11850", "title": "[Estimation of regional blood flow in reimplanted segments of upper extremity].", "score": 0.009708737864077669, "content": "To examine the state of regional circulation in replanted segment of upper limb and hand, to determine diagnostic and prognostic value of radionuclide scintigraphy. The study included 26 patients who underwent replantation of upper extremity segments. The control group included 12 patients who underwent autologous transplantation of toes on hand. All patients underwent isotope scintigraphy, ultrasound Doppler and pulse oximetry. Groups were comparable by gender, age, severity of injury. Depending on postoperative course two groups were determined: with favorable (27 patients) and complicated postoperative period (11 patients). Two types of regional circulation were identified according to dynamic scintigraphy: prevalence of blood flow in operated limb (type 1); prevalence of blood flow in healthy limb, or equal volumetric blood flow in operated and healthy limbs (type 2). Favorable early postoperative period was associated with prevalence of blood flow in operated limb. Only in 2 (7.7%) patients patients in this group acute arterial thrombosis was diagnosed. At the same time thrombosis of microanastomosis occurred in 45.4% of cases in the second group what is 5.5 times higher than in the first group. Significant prevalence of blood flow in operated limb is favorable prognostic sign of the early postoperative period. On the other hand the identity of blood flow or prevalence of such in healthy limb predispose to acute vascular complications in replantate or autoplantate. Radionuclide scintigraphy provides detailed assessment of regional blood flow in operated limb. However clinical monitoring has leading role in diagnosis of acute circulatory disorders in replantate or autoplantate."}, {"id": "pubmed23n0701_1056", "title": "[Early treatment of high-voltage electric burn wound in the limbs].", "score": 0.009615384615384616, "content": "To summarize the experience of early treatment of high-voltage electric burn wounds in the limbs. Fifty-four patients (50 males and 4 females, aged from 10 to 56 years) with high-voltage electric burn wounds in 97 limbs (67 upper limbs and 30 lower limbs) were hospitalized in our burn wards from January 2003 to December 2010. A total of 119 burn wounds in wrist-forearm, forearm-elbow-upper arm, shoulder-axillary region, ankle-foot, lower leg, around the knee, thigh-inguinal region were treated with incision for decompression within 10 days after burn. Under the premise of relatively stable systemic condition of the patients, certain surgical operations were performed as follows. (1) Sixteen limbs with 16 wounds were amputated, among them forearm amputation was performed for 5 upper limbs with necrosis, with preservation of elbow joints, and the residual wounds of the elbow and upper arm were repaired with pedicled latissimus dorsi musculo-cutaneous flaps; 1 upper limb with upper arm amputated, with preservation of shoulder joint, was repaired with pedicled latissimus dorsi musculo-cutaneous flap. (2) Ninety-five wounds were covered with various tissue flaps with abundant blood supply after early debridement, in which 3 brachial arteries, 1 vein, 1 brachial artery and vein were reconstructed in 5 wrist wounds, artery reconstruction was performed in elbow wound of 1 case with injured brachial artery. (3) Eight wounds were treated with free skin grafting. Wound healing conditions were observed and followed up. Wounds in 16 limbs healed after amputation and repair. Blood supply and (or) venous return of hands were restored in 5 wrist wounds after vessel reconstruction. After artery reconstruction, abundant blood supply was observed in 1 case with injured brachial artery and amputation was avoided. Necrosis occurred in distal parts of tissue flaps in 5 wounds after grafting, in which 2 wounds healed after removal of necrotic tissue followed by closure with suture, and 3 wounds healed after debridement and free skin grafting. Tissue flap infection occurred in wrist (5 wounds), elbow (1 wound), ankle-foot (2 wounds), and healed after debridement and suture. The other tissue flaps survived after grafting. Six wounds healed after skin grafting. Partial necrosis occurred in 2 wounds after skin grafting, and they were healed after second skin grafting. Thirty-seven patients were followed up for 6 to 12 months, the skin flaps survived with satisfactory appearance and texture. Early extensive compartment release through fasciectomies and escharectomies, early debridement, early vascular grafting, early wound coverage with contemporary reparative and reconstructive surgical techniques are rational options for the treatment of high-voltage electric burns in the limbs."}, {"id": "pubmed23n0699_15990", "title": "[Preoperative Doppler evaluation of vascular perforators in the anterolateral thigh flap harvest].", "score": 0.009615384615384616, "content": "To evaluate the accuracy of Hadeco ES-1000spm hand-held doppler during the anterolateral thigh (ALT) flap harvest. Twenty-five patients (26 sides) with ALT flaps for head and neck reconstruction between May 2005 and May 2010 received preoperative Doppler examination for the location of the cutaneous perforators of ALT flaps. The Doppler signals and body mass index (BMI) were recorded preoperatively according to ABC system. The locations of Doppler signals and of the actual cutaneous perforators at surgery were plotted and compared. The diameter of perforators was measured. One to three cutaneous perforators of the ALT flap were consistently found at specific locations. They were named perforators A, B, C from proximal to distal. Perforators A, B and C were present in 15 (58%), 24 (92%) and 20 (77%) cases and the diameter (&gt; 0.5 mm) of A, B and C were 11/15, 22 (92%) and 8 (40%) respectively. The Doppler signal was within 0.5 cm of the actual perforator location in 85% flaps. The accuracy of Doppler decreased with increase of BMI. Preoperative assessment by hand-held Doppler is useful in predicting the perforator vessels' locations and diameter although it's accuracy is limited."}, {"id": "pubmed23n0083_18095", "title": "[Early treatment of third degree burns of the entire hand].", "score": 0.009523809523809525, "content": "Treatment and result of burned total hand was reported here in 4 hands (4 cases). According to the treatment and anatomical characteristics, burned hand was divided into 4 areas: dorsum of hand, dorsum of finger, thenar-wrist area and volar-digit area. The treatment of total hand in third degree burns included emergency escharotomy of hand and digits, early escharotomy and immediate skin grafting on the dorsum of hand, thenar-wrist and volar-digit area. But on the dorsum of digit area this sort of hand was often deepen to the phalangeal bones and joints. After healing of early graft, the exposed bones and joints were decorticated with immediate grafting on the fresh bone marrow surface and with the finger tip repair so as to preserve the length of digits. When the bone marrow surface grafting healed metallic finger splints were bent sequentially to functional position for 8 weeks in order to ankylosis of interphalangeal joints. Followed up 4 hands with good results. And only 5 distal phalanges were loss in 20 digits. But in previous cases this kinds of digits were often amputated with poor results."}, {"id": "pubmed23n0068_318", "title": "[Economic amputations of the lower limbs due to ischemia. I].", "score": 0.009523809523809525, "content": "The problem relating to leg amputation following ischemia are analysed in the first part of this study, bearing in mind that amputation must be as conservative as possible in order to ensure the best quality of life. Following a short review of the topic and the introduction of recent trends, the case for amputation, which must be early in order to be conservative, is studied. The first part of this article concludes with a discussion of Doppler and clinical evaluations as techniques used to determine the level of amputation."}, {"id": "pubmed23n0233_10783", "title": "Burn wound management.", "score": 0.009433962264150943, "content": "In this chapter the local therapy for burns is discussed. Between 400 and 500 children with burns are treated every year at the Red Cross War Memorial Children's Hospital in Cape Town, but in only 10% of them do the burns affect over 20% of the body surface. These latter patients are treated in special rooms equipped for intensive therapy. Open and closed methods of treatment for burns used in addition to early excision are compared. The first aim is early skin cover for areas with skin loss preserving as much function as possible and achieving the best possible cosmetic result. Local therapy must be atraumatic to prevent extension of the skin lesion. Bacterial contamination must be prevented as far as possible by keeping the wound clean. Emergency treatment and the course of wound healing up to the third week after the injury using the appropriate dressings are described. Early excision until the fifth day after the accident should be used mainly for burns of the hand, deep second degree burns of up to 10% of the body surface, deep second degree burns over the joints and deep second degree burns of the neck. It must be admitted that the depth of the burn can only be definitely estimated between the seventh and tenth day after the accident. If no autografts are available homografts or grafts from animals are used. The age of the patient, associated injuries, associated diseases and the extent of the burn all play a role in determining the prognosis. Furthermore endogenous bacterial infections, absorption of local therapeutic agents and the state of the surrounding skin do also influence the healing process. Finally the various local therapeutic agents like sulphamylon, silver sulphadiazine and betadine are discussed. A 0.05% solution of silver nitrate is also active against gram-negative infections. Skin transplants are disinfected with a solution containing one third 0.25% acetic acid, one third 3% cent hydrogen peroxide and one third saline. Hydrogen peroxide must not be applied to burns that are healing spontaneously. A classification of burns to help to choose the appropriate local therapy is proposed."}, {"id": "pubmed23n0129_9021", "title": "The prediction of healing in ischemic lesions of the foot. A comparison of Doppler ultrasound and elevation reactive hyperemia.", "score": 0.009433962264150943, "content": "The results of the Elevation Reactive Hyperemia Test (ERHT) and the transcutaneous Doppler ultrasound ankle blood pressure (DAP) have been compared in a series of 115 cases with ischemic lesions of the foot. The ERHT was carried out by simple elevation of the foot or elevation with the circulation temporarily obstructed by a blood pressure cuff following which the foot was gradually lowered until the reactive hyperemia was seen in the skin and the height above the right atrium was estimated. The appearance of the hyperemia at 35 cm above the right atrium will allow healing of local ischemic lesions and at the height of 45 cm or above indicates a zone at which amputation may be carried out successfully. In the same series the DAP using 60 mmHg as the cut off point showed 40% healing with a pressure less than this level and 40% failed with a pressure above this level. The DAP gave no consistent prediction of the healing of ischemic lesions or of amputations."}, {"id": "pubmed23n1009_13642", "title": "Iatrogenic Compartment Syndrome Secondary to Burn Dressing in a 2-Year-Old Child.", "score": 0.009345794392523364, "content": "We report a severe case of compartment syndrome due to a compressive burn dressing. An otherwise healthy 2-year-old girl presented at her local health center with a superficial partial-thickness thermal burn on the dorsum of the mid phalanx of the second finger of her right hand. A compressive dressing was applied solely to the affected finger. Forty-eight hours afterward, the patient presented in the emergency room with severe pain of the finger. After removal of the dressing, a circular constrictive eschar was observed at the base of the finger, secondary to ischemia due to the compressive dressing. Emergent lateral escharotomies were performed, with immediate recovery of distal perfusion. One week afterward, the patient underwent surgical debridement of the burn on the dorsum of her finger and escharectomy of the ischemic eschar at the base. The lesions were covered with partial-thickness skin grafts. This case shows that acute compartment syndrome can lead to severe sequelae, such as the loss of an extremity or body segment. We must take utmost care in all our actions to avoid any (negligent) act that could lead to severe or permanent damage to our patients."}, {"id": "pubmed23n0371_15869", "title": "[Surgical therapy of acute and chronic arterial occlusions below the inguinal ligament].", "score": 0.009345794392523364, "content": "The history and physical examination are extremely important in the management of vascular surgical patients because a correct diagnosis can usually be made on the basis of information obtained from these two modalities. The severity of the chronic occlusive process leads to characteristic symptoms in the extremity: claudication, rest pain, skin ulcerations and gangrene. Chronic progressive lesions permit enlargement of collateral blood supply which, for a time, minimizes the severity of symptoms. Milde degrees of arterial insufficiency (claudication) can be treated conservatively. Unreconstructed chronic critical ischemia predicts a poor outcome in terms of survival and limb salvage. The outlook with arterial reconstructive surgery is by far better. Arteriography ist the most reliable diagnostic test for occlusive lesions. It ist essential for the operative planning. Vein-bypass procedures are, if feasible, very effective in most cases. Acute embolic occlusion: Sudden occlusion of a previously patent artery is usually a dramatic event producing severe ischemia of the distal tissue. The characteristic symptoms and signs are the 5 P's: pallor, pain, paresthesia, paralysis, pulselessness. Emergent restoration of blood flow by operation may be essential to prevent limb loss. Milder forms of ischemia (acute thrombosis--acute or chronic disease) can be treated initially with intravenous heparin if the extremity is not threatened (minimal sensory loss, no muscle weakness). Elective surgery at a later date is highly successful."}, {"id": "pubmed23n0248_8858", "title": "Intramuscular pressure in the burned arm: measurement and response to escharotomy.", "score": 0.009259259259259259, "content": "Using a wick catheter technique, sequential measurements of intramuscular pressure were obtained in 31 burned arms in 18 patients. Abnormally high pressures were recorded in 90 percent of extremities and exceeded the potentially harmful level of 30 mm Hg in 42 percent. Correlation of intramuscular pressure with signs and symptoms of extremity compression, including Doppler pulses, was poor. Intramuscular pressure elevation appeared to parallel edema formation beneath the burn wound. A high incidence of pressure measurements in excess of 30 mm Hg was found in patients who had 30 percent or greater total body surface area injury (67 percent), 10 percent or greater full-thickness burns (75 percent), and extremities with circumferential involvement (57 percent). In every case echarotomy produced a dramatic decrease in intramuscular pressure, while a randomized group of extremities that were not decompressed developed sustained pressures as high as 64 mm Hg despite the presence of intact Doppler pulses. Extremities treated in this manner appeared slower in resolving edema and regaining motion and strength. Measurement of intramuscular pressure beneath the burn eschar is recommended in evaluating all patients at risk from extremity burns."}, {"id": "article-18197_28", "title": "Below-Knee Amputation -- Contraindications", "score": 0.009259259259259259, "content": "Doppler may assess for gross blood flow, and ankle-brachial indices can evaluate an individual and lower versus upper extremities. Oxygen pressures in the toes and transcutaneous oxygen pressure are useful for determining oxygenation on a microvascular level. In cases of profound vascular insufficiency, bypass grafting or the placement of stents may be necessary before performing a BKA. Some researchers used indocyanine green near-infrared (ICG NIR) fluorescence imaging to predict postoperative skin flap necrosis. [18] [19]"}, {"id": "pubmed23n0887_17540", "title": "Civilian blast-related burn injuries.", "score": 0.009174311926605505, "content": "There is limited English literature describing the experience of a civilian hospital managing blast-related burn injuries. As the largest regional burn unit, we reviewed our cases with the aim of identifying means to improve current management. A 6-year retrospective analysis of all patients coded as sustaining blast-related burns was conducted through the unit's burns database. Medical case notes were reviewed for information on burn demographics, management and outcomes. 42 patients were identified. Male to female ratio was 37:5. Age range was 12-84 years, (mean=33 years). Total body surface area (%TBSA) burn ranged from 0.25% to 60%, (median=1%). The most common burn injury was flame (31/42, 73.8%). Gas explosions were the most common mechanism of injury (19 cases; 45.2%). 7/42 cases (16.7%) had full ATLS management pre-transfer to the burns unit. The Injury Severity Score (ISS) ranged from 0-43 (median=2). 17/42 (40.4%) patients required admission. 37/36 (88.1%) patients were managed conservatively of which 1 patient later required surgery due to deeper burns. 5/42 (11.9%) patients required surgical management at presentation and these were noted to be burns with &gt;15% TBSA requiring resuscitation. One case required emergency escharotomies and finger amputations. All patients survived their burn injuries. Blast-related burn injuries are generally uncommon in the civilian setting. Following proper assessment, most of these cases can be deemed as minor injuries and managed conservatively. Improvement in burns management education and training at local emergency departments would provide efficient patient care and avoid unnecessary referrals to a burns unit."}, {"id": "pubmed23n0875_548", "title": "Determining End Points for Critical Limb Ischemia Interventions.", "score": 0.009174311926605505, "content": "Critical limb ischemia is a condition that has increased in prevalence and carries a high degree of morbidity. Although endovascular therapy for treatment of patients with critical limb ischemia has undergone significant advances with improved outcomes over the past decade, these patients often have multilevel disease, and it may take weeks or months for ulceration healing. For this reason, the acceptable therapeutic end points during and immediately following revascularization remain somewhat obscure. There are multiple tools available to guide the treating vascular specialist in this regard. Establishment of in-line flow to the foot and the angiosome containing the ulceration, appearance of a \"wound blush,\" restoration of pulses, and bleeding at the ulcer site are basic tenets intraprocedurally. Postprocedural noninvasive testing including the ankle-brachial and toe-brachial indices, segmental pressure measurements, pulse volume recordings, transcutaneous oxygen tension, skin perfusion pressures (SPPs), and toe pressures all play a role in determining the likelihood of clinical improvement. Newer technologies such as two-dimensional (2D) perfusion angiography, fluorescence angiography, and tissue oxygen saturation mapping may allow better real-time assessment of flow restoration. In combination with close clinical follow-up and wound care, these tools provide treating physicians with a better grasp of the necessary end points to optimize patients for clinical improvement. "}, {"id": "InternalMed_Harrison_19794", "title": "InternalMed_Harrison", "score": 0.00909090909090909, "content": "usually are used in conjunction with pharmacologic thrombolysis. Surgical revascularization is preferred when restoration of blood flow must occur within 24 h to prevent limb loss or when symptoms of occlusion have been present for more than 2 weeks. Amputation is performed when the limb is not viable, as characterized by loss of sensation, paralysis, and the absence of Doppler-detected blood flow in both arteries and veins."}, {"id": "pubmed23n0980_20462", "title": "[Analysis of causes and treatment methods of complication of early acute kidney injury in four severely burned patients].", "score": 0.009009009009009009, "content": "<bObjective:</b To analyze the causes of complication of early acute kidney injury (AKI) in four severely burned patients, and to explore the related treatment methods. <bMethods:</b The clinical data of 4 patients with severe burn complicated with early AKI admitted to Guangzhou Red Cross Hospital Affiliated to Medical College of Jinan University (hereinafter referred to as our hospital) from June 2014 to December 2017 were retrospectively analyzed. All the patients were male, aged 23-33 (30±5) years old, with depth of burns ranged from deep partial-thickness to full-thickness, complicated with myofascial compartment syndrome of extremities and varying degrees of striated muscle injury, and treated in other hospitals before transfer to our hospital. The patients were numbered from small to large according to the total burn area. The total burn area of patients No. 1, 2, 3, and 4 was 10%, 80%, 90%, and 95% total body surface area respectively, their occurrence time of early AKI was 48, 11, 29, and 48 hours after injury respectively, and their time of arriving our hospital was 60, 11, 29, and 144 hours after injury respectively. Hypovolemic shock occurred in patients No. 2 and 3 at admission to our hospital. All the patients received continuous renal replacement therapy (CRRT) after admission to our hospital. Under the support of hemodynamic monitoring and organ function monitoring, the limbs complicated with myofascial compartment syndrome were incised, thorough decompression exploration was performed, and necrotic muscle tissue was removed or amputation was performed. After escharectomy and decompression of limbs, fresh granulation wounds were formed by temporarily covering wounds with Jieya dressing skin or pig skin, multiple debridements, and vacuum sealing drainage. Fresh granulation wounds and other wounds underwent staged eschar excision and shaving were covered with autologous Meek skin graft, particulate skin graft, reticular skin graft and small skin graft respectively. The treatment outcome, CRRT time, operation times, time of recovery of serum creatinine and myoglobin, length of hospital stay, and follow-up were recorded. <bResults:</b All the 4 patients were cured after transfer to our hospital. Among them, totally 5 limbs of patients No. 1 and No. 4 underwent amputation because of complication of myofascial compartment syndrome and a large amount of necrotic muscle which could not be preserved. Patients No. 1, 2, 3, and 4 were treated with CRRT for 19, 35, 14, and 25 days respectively and performed with operation for 5, 6, 10, 8 times respectively. Serum creatinine of patients No. 1, 2, 3, and 4 returned to normal on 22, 35, 37, and 48 days after transfer respectively, and their serum myoglobin returned to normal on 18, 28, 25, and 30 days after transfer respectively. Patients No. 1, 2, 3, and 4 were hospitalized for 52, 105, 148, and 156 days and discharged after basic wound healing. Follow-up for 1 to 36 months showed no abnormal renal function in 4 patients. <bConclusions:</b The early AKI in patients No. 1 and 4 was caused by rhabdomyolysis after severe burn complicated with myofascial compartment syndrome, while that of the other 2 cases were also related to hypovolemic shock and poor renal perfusion. The success rate of early AKI treatment in severely burned patients can be effectively improved by removing the causes of diseases at the same time of CRRT and actively treating burn wounds under the support of organ function and hemodynamic monitoring."}, {"id": "pubmed23n0994_16338", "title": "Does the new vascular management of acute limb ischemia have effective results with lower treatment costs.", "score": 0.009009009009009009, "content": "Aim To compare hospital costs of acute limb ischemia treatment in two periods of time and to show evidence of long-term repercussions on reducing costs during successful treatment. Methods Retrospective analysis of data obtained from 100 patients' medical history in the period 2000-2016 at the Clinic of Vascular Surgery Sarajevo: group A - 60 patients with acute limb ischemia in the period 2005-2016 and group B - 40 patients with acute limb ischemia (ALI) in the period 2000-2005. From 2000 to 2005 conservative treatment method was used, invasive diagnostic and surgical procedures were often delayed for a shorter or longer period of time. During the period from 2005 to 2016, the management model and safe practice included emergency diagnostic procedures, colour-Doppler, arteriography, emergency surgery (embolectomy by Fogharty and if necessary, vascular by-pass). Results Better health service for the patients with acute limb ischemia was offered in the period 2005-2016, which relied on proven medical treatment trends. The largest share of the total costs of each patient included costs of hospital bed with significant difference between the period 2005-2016 and 2000-2005, mean of 1398.71 KM and 2480.45KM, respectively (p&lt;0.0001), indicating rationalization of time that patients spend at the Vascular Clinic. Conclusion This trend of money/fund savings is an example of good practice, effectiveness and efficiency in the treatment of ALI and as such was used in patients with other vascular diseases."}, {"id": "pubmed23n0589_16229", "title": "Pediatric upper extremity burns: outcomes of emergency department triage and outpatient management.", "score": 0.008928571428571428, "content": "Pediatric upper extremity burns are common. Though current American Burn Association guidelines recommend burn unit referral for burns involving the hands or major joints, many minor injuries are treated in the emergency department (ED) or outpatient setting. Despite the large number of burn patients managed by primary care providers, no large studies have been performed to assess effectiveness. A retrospective 5-year review of the epidemiology and outcomes associated with pediatric upper extremity burns treated at an urban ED was performed. Two hundred sixty-nine patients were identified. The mechanism of burn, percentage of total body surface area (%TBSA) affected, plastic surgery consultations (for wound management recommendations and additional treatment), complications, and surgical interventions were examined. Mechanisms of burn included direct contact (47%), scald (29%), flame (12%), electrical (10%), and friction or chemical (1.5%). Fifty percent of patients suffered from burns over less than 1% TBSA; close to 95% had burns on less than 5% TBSA. Seventy-five percent of patients had second-degree burns, 21% had first-degree burns, and 2% had third-degree burns. Forty patients (15%) had a plastic surgery consult. Seven patients (3%) required skin grafting. Complications occurred in five (2%) patients and included two cases of hypertrophic scarring; two patients with flexor contractures, one case of compartment syndrome requiring fasciotomy, and one late infection. These results suggest that although significant burns are usually cared for in specialized burn centers, the majority of childhood burns to the upper extremity are relatively minor and often treated in the primary care setting. Most patients had small areas of injury and healed without complications. Contact burns are an ever-increasing proportion of childhood burns and should be seemingly preventable. Education to parents and primary care physicians should be reemphasized. It appears that minor upper extremity burns treated by our urban ED staff are handled appropriately and result in favorable outcomes."}, {"id": "wiki20220301en059_66337", "title": "Dorsalis pedis artery", "score": 0.008849557522123894, "content": "The dorsalis pedis communicates with the plantar blood supply of the foot through the deep plantar artery. Along its course, it is accompanied by a deep vein, the dorsalis pedis vein. Function The dorsalis pedis artery supplies oxygenated blood to the dorsal side of the foot. Clinical significance Pulse The dorsalis pedis artery pulse can be palpated readily lateral to the extensor hallucis longus tendon (or medially to the extensor digitorum longus tendon) on the dorsal surface of the foot, distal to the dorsal most prominence of the navicular bone which serves as a reliable landmark for palpation. It is often examined, by physicians, when assessing whether a given patient has peripheral vascular disease. It is absent, unilaterally or bilaterally, in 2–3% of young healthy individuals. Ultrasound The dorsalis pedis artery may be studied using ultrasound. Doppler ultrasound can be used to investigate blood flow."}, {"id": "pubmed23n0348_10945", "title": "Management of acute nontraumatic upper limb ischemia.", "score": 0.008849557522123894, "content": "A retrospective review of all patients presenting to a tertiary referral center with acute nontraumatic upper limb ischemia between January 1992 and June 1997 was undertaken to examine the role of intraarterial thrombolysis in the management of such cases. Twenty-one patients were identified in the radiology and vascular surgery departments' registers. Twenty (95%) underwent angiography, demonstrating subclavian artery occlusion in four, axillary in two, brachial in 13, and one at the digital level. Intraarterial thrombolysis was attempted in 12 patients. There were three technical failures, all requiring embolectomy. Six had complete lysis and resolution of their symptoms. One patient had partial lysis but experienced no further rest pain. Thrombolysis was unsuccessful in two cases with one subsequently requiring embolectomy and the other surgical bypass. Three patients had surgical intervention as their primary procedure with two favorable outcomes and one ending in above-elbow amputation. Five patients were treated conservatively with heparin, resulting in three partial and two full recoveries. One patient experienced complete resolution of symptoms with an intravenous prostacyclin infusion. Both electrocardiograms (ECG) and echocardiograms (ECHO) were of limited diagnostic aid, and long-term warfarin anticoagulation was prescribed to all patients. There was no recurrence of upper limb ischemia at a median follow up of 18 months. Intraarterial thrombolysis is an effective first line treatment for acute nontraumatic upper limb ischemia in selected cases."}, {"id": "wiki20220301en033_5564", "title": "Eschar", "score": 0.008771929824561403, "content": "Eschar may be allowed to slough off naturally, or it may require surgical removal (debridement) to prevent infection, especially in immunocompromised patients (e.g. if a skin graft is to be conducted). If eschar is on a limb, it is important to assess peripheral pulses of the affected limb to make sure blood and lymphatic circulation is not compromised. If circulation is compromised, an escharotomy, or surgical incision through the eschar, may be indicated."}, {"id": "pubmed23n0367_16458", "title": "[Arterial embolisms of the lower extremities].", "score": 0.008771929824561403, "content": "Embolism is one of the most frequent causes of lower limbs acute arterial occlusion [1]. Of the total number of peripheral embolism 56% of cases involve lower limbs arteries [2]. Inadequate and late treatment of the lower limbs embolism is associated with high morbidity and mortality rate. The aim of this paper was to study the aetiology of lower limbs embolism and to detect factors influencing early and late results after the operative treatment. The study included 204 patients with 224 lower limbs embolism, treated surgically at the Institute of Cardiovascular Diseases of the Clinical Centre of Serbia in Belgrade in the period between 1993 and 1997. There were 107 (52.2%) female and 97 (47.8%) male patients. Thirty two (14.3%) patients were younger than 50 years, 64 (28.6%) were between 51 and 65, 101 (45.1%) between 66-75, while 27 patients (12.1%), were older than 75. Twenty (8.9%) patients were admitted less than 6 hours before the operation, 79 (33.3%) between 6 and 24 hours, and 125 (55.8%) more than 24 hours before the operation (Table 1). One hundred (53.6%) patients had motor and 133 (59.4%) sensor paralysis on admission. Table 2 shows arterial localization of the lower limbs embolism. The popliteal artery was involved in most cases. During the operation transfemoral arterial approach was used in 132 (58.9%) cases, while transpopliteal in 92 (41.1%) cases. Fourteen cases required bypass surgery, 43 fasciotomy, 2 intraoperative streptokinase and 4 intraoperative angiography. All patients were controlled using physical and CW Doppler ultrasonographic examinations immediately after the operation, and then one, six and 12 months, as well as every year. In 173 (84.4%) patients cardiac causes of embolism were found, in 8 (3.9%) noncardiac, while in 8 (3.9%) the cause could not be established. Of all cardiac causes absolute arrhythmia was most frequent. Table 3 and Table 4 show the aetiology of the lower limb embolism. The early amputation rate was 23 (10.3%) cases, while limb salvage was recorded in 174 (77.7%) patients. Of all saved limbs complete recovery was noted in 162 (72.4%) cases and peroneal nerve paresis in 12 (5.3%) cases. The early postoperative mortality rate was 27 (12.0%). Table 5 shows early results of embolectomy. The early results (limb salvage, complete recovery, rethrombosis, early reoperations, amputations rate, morbidity and mortality rate) of embolectomy were statistically significant: worse in cases when the embolus was located in the abdominal aorta and popliteal artery; in cases with a long time interval before the operation as well as in patients with sensor-motoric paralysis on admission (Tables 6-8). Of the total number of patients in 87 (56.5%) cases a late control examination was carried out. Forty nine (31.8%) patients died before the late control, while 18 (11.7%) did not come to control examination. Late recidivation of embolism was found in 3 cases. In these patients the cause could not be found, and they were treated by anticoagulant drugs."}, {"id": "pubmed23n0861_12074", "title": "Amputation Following Hand Escharotomy in Patients with Burn Injury.", "score": 0.008695652173913044, "content": "Hand burns are commonly seen in patients with burn injury. In the past, focus was on lifesaving measures, but with advances in burn care during the last century, the paradigm shifted to digital salvage and eventually to functional digital salvage. Good outcomes are heavily dependent on the care that is rendered during the initial management of the burn. A retrospective medical record review was conducted through the Central Illinois Regional Burn Center Patient Registry. Patients with burn injury treated with upper extremity and hand escharotomy between January 1, 2000, and December 31, 2005, were included in the study. We identified a total of 34 patients with 57 burned hands. Six hands required delayed amputation of digits despite recognition of neurovascular compromise and escharotomy, yielding a 10% amputation rate. No correlation could be drawn with regard to total body surface area, age, or sex. Important principles in the acute phase include early splinting, recognition of the need for escharotomy and complete escharotomy when necessary, early excision and grafting, and involvement of occupational therapy for splinting and to guide both active and passive exercises. Although uncommon, some extremity burns may require subsequent amputation despite prompt attention and optimal treatment. In our case series, the need for amputation after successful escharotomies of salvageable digits was associated with full-thickness and electrical burns."}, {"id": "pubmed23n0944_25283", "title": "Acute Iatrogenic Limb Ischaemia, a Report of 2 Late Presentation Cases.", "score": 0.008695652173913044, "content": "With increasing use of percutaneous vascular procedures, access complications that present to a vascular surgeon increase. The most limb-threatening condition is acute limb ischaemia. Acute limb ischaemia is the most common vascular surgical emergency. In spite of recent advances in vascular surgery, it continues to carry a poor prognosis, if not early diagnosed and managed. This is a case-report of 2 patients referenced to a vascular surgery emergency department of a tertiary hospital with late acute limb ischaemia. Patient 1: Male, 42 years, alcoholic, autonomous, presented with pain with elbow active movements in a secondary hospital. Excluded acute orthopaedic injury, doctor recorded signs of acute limb ischaemia and referenced patient to a tertiary hospital, where vascular surgeon diagnosed an acute advanced upper limb ischaemia. Bed-side Eco-Doppler showed an echogenic linear material on a thrombosed umeral artery, surgically confirmed to be a guidewire (Fig.1. Surgical extraction of intra-umeral guidewire). Reviewing patient history, this guidewire should have been missed over 6 months, by the time the patient was hospitalized on an ICU for alcoholic coma. Patient underwent umeral, radial and ulnar thromboembolectomy and had a no-reflow status. However, poor persistent global status, with limited mobilization, pressure forces and prolonged vasotropic support, promoted progression of a cyanotic leg plaque to a necrotic evolving leg ulcer with septic response, despite persistent good perfusion of the foot (Fig.2. Necrotic evolving leg ulcer). Unfortunately, the two reported patients underwent urgent major limb amputation, patient 1 above the elbow, and patient 2 above the knee. Acute limb ischaemia continues to carry a poor limb and life prognosis if not early diagnosed. We should be alert for the increasingly prevalence of iatrogenic acute limb ischaemia, and regularly evaluate perfusion status of limbs after any percutaneous procedure."}, {"id": "pubmed23n1038_2910", "title": "Validation of a low-cost simulation strategy for burn escharotomy training.", "score": 0.008620689655172414, "content": "Escharotomy is the primary effective intervention to relieve constriction and impending vascular compromise in deep, circumferential or near-circumferential burns of the extremities and trunk. Training on escharotomy indications, technique and pitfalls is essential, as escharotomy is both an infrequent and high-risk procedure in civilian and military medical environments, including low-resource settings. Therefore, we aimed to validate an educational strategy that combines video-based instruction with a low-cost, low-fidelity simulation model for teaching burn escharotomy. Pre-hospital and hospital-based medical personnel, with varying degrees of burn care-related experience, participated in a one-hour training session. The first part of the training consisted of video-based instruction that described the indications, preparation, steps, pitfalls and complications associated with escharotomy. The second part of the training consisted of a supervised, hands-on simulation with a previously described low-cost, low-fidelity escharotomy model. Participants were then offered two psychometrically validated instruments to assess their learning experience. 40 participants were grouped according to prior burn care and surgical experience: attending surgeons (6), surgery and emergency medicine residents and fellows (26), medical students (5), and pre-hospital personnel (3). On two psychometrically validated questionnaires, participants at both the attending and trainee levels overwhelmingly confirmed that our educational strategy met best educational practices on the criteria of active learning, collaboration, diverse ways of learning, and high expectations; they also highly rated their satisfaction with and self-confidence under this learning strategy. An educational strategy that combines video-based instruction and a low-cost, low-fidelity escharotomy simulation model was successfully demonstrated with participants across a broad range of prior burn care experience levels. This strategy is easily reproducible and broadly applicable to increase the knowledge and confidence of medical personnel before they are called to perform escharotomy. Important applications include resource-limited environments and deployed military settings."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 339]], "word_ranges": [[0, 53]], "text": "FiO2 of 1 and hyperventilation with mechanical ventilation, demonstrated by the low PCO2, fail to sufficiently raise PO2, due to a V/Q disturbance, with probable shunt in the area of pulmonary atelectasis, probably acute, since the lung has not been able to create compensatory mechanisms to limit perfusion in the poorly ventilated areas."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "FiO2 of 1 and hyperventilation with mechanical ventilation, demonstrated by the low PCO2, fail to sufficiently raise PO2, due to a V/Q disturbance, with probable shunt in the area of pulmonary atelectasis, probably acute, since the lung has not been able to create compensatory mechanisms to limit perfusion in the poorly ventilated areas.", "full_answer_no_ref": "FiO2 of 1 and hyperventilation with mechanical ventilation, demonstrated by the low PCO2, [HIDDEN], due to a V/Q disturbance, with probable shunt in the area of pulmonary atelectasis, probably acute, since the lung has not been able to create compensatory mechanisms to limit perfusion in the poorly ventilated areas.", "full_question": "While you are on call in the Emergency Department of your hospital, you have to attend a 64-year-old patient with acute respiratory failure. His clinical condition is critical, with low oxygen saturation and hemodynamic instability. An urgent chest X-ray was performed showing atelectasis of 2/3 of the right lung. Orotracheal intubation and assisted ventilation were performed, with Fi02 of 1.0. Subsequent arterial blood gas analysis showed pH 7.23, Pa02 60 mmHg and PaC02 30 mmHg. What was the cause of the hypoxemia?", "id": 368, "lang": "en", "options": {"1": "Short circuit.", "2": "Hypoventilation.", "3": "Low inspired 02 pressure.", "4": "Neuromuscular disease.", "5": NaN}, "question_id_specific": 121, "type": "PNEUMOLOGY AND THORACIC SURGERY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en010_32319", "title": "Respiratory failure", "score": 0.015714943587381992, "content": "This type of respiratory failure is caused by conditions that affect oxygenation, such as: Low ambient oxygen (e.g. at high altitude) Ventilation-perfusion mismatch (parts of the lung receive oxygen but not enough blood to absorb it, e.g. pulmonary embolism) Alveolar hypoventilation (decreased minute volume due to reduced respiratory muscle activity, e.g. in acute neuromuscular disease); this form can also cause type 2 respiratory failure if severe. Diffusion problem (oxygen cannot enter the capillaries due to parenchymal disease, e.g. in pneumonia or ARDS) Shunt (oxygenated blood mixes with non-oxygenated blood from the venous system, e.g. right to left shunt) Type 2 Hypoxemia (PaO2 <8kPa or normal) with hypercapnia (PaCO2 >6.0kPa). The basic defect in type 2 respiratory failure is characterized by: {| class=\"wikitable\" |PaO2 || decreased (< )or normal |- | PaCO2 || increased (> ) |- | PA-aO2 || normal |- |pH || <7.35 |}"}, {"id": "wiki20220301en008_58623", "title": "Tracheal intubation", "score": 0.01442571899615314, "content": "Intubation may be necessary for a patient with decreased oxygen content and oxygen saturation of the blood caused when their breathing is inadequate (hypoventilation), suspended (apnea), or when the lungs are unable to sufficiently transfer gasses to the blood. Such patients, who may be awake and alert, are typically critically ill with a multisystem disease or multiple severe injuries. Examples of such conditions include cervical spine injury, multiple rib fractures, severe pneumonia, acute respiratory distress syndrome (ARDS), or near-drowning. Specifically, intubation is considered if the arterial partial pressure of oxygen (PaO2) is less than 60 millimeters of mercury (mm Hg) while breathing an inspired O2 concentration (FIO2) of 50% or greater. In patients with elevated arterial carbon dioxide, an arterial partial pressure of CO2 (PaCO2) greater than 45 mm Hg in the setting of acidemia would prompt intubation, especially if a series of measurements demonstrate a worsening"}, {"id": "wiki20220301en010_32316", "title": "Respiratory failure", "score": 0.011963748782450346, "content": "Respiratory failure results from inadequate gas exchange by the respiratory system, meaning that the arterial oxygen, carbon dioxide, or both cannot be kept at normal levels. A drop in the oxygen carried in the blood is known as hypoxemia; a rise in arterial carbon dioxide levels is called hypercapnia. Respiratory failure is classified as either Type 1 or Type 2, based on whether there is a high carbon dioxide level, and can be acute or chronic. In clinical trials, the definition of respiratory failure usually includes increased respiratory rate, abnormal blood gases (hypoxemia, hypercapnia, or both), and evidence of increased work of breathing. Respiratory failure causes an altered mental status due to ischemia in the brain. The typical partial pressure reference values are oxygen Pa O2 more than 80 mmHg (11 kPa) and carbon dioxide Pa CO2 less than 45 mmHg (6.0 kPa). Cause"}, {"id": "wiki20220301en028_45077", "title": "Generalized hypoxia", "score": 0.011622146071590768, "content": "Causes Restricted oxygen flow to the body's tissue that leads to hypoxia can be caused by a variety of situations or other underlying conditions. High altitude (above 3048 metres/10,000 feet) Hypoventilation – failure of respiratory pump due to any cause (fatigue, barbiturate poisoning, pneumothorax, etc.) Ventilation perfusion mismatch Obstructed airway Drowning Abnormal pulmonary function Chronic obstructive pulmonary diseases (COPD) Neuromuscular diseases or interstitial lung disease Constrained blood flow to a tissue (such as atherosclerosis or vasoconstriction) Blockage in blood flow like a sickle cell crisis Low or no blood flow caused by bleeding or heart attack Malformed vascular system such as an anomalous coronary artery Limited oxygen transportation due to anemia While respiring in the open air at high altitudes, the human body experiences altitude sickness and hypoxemia due to a low partial pressure of oxygen, decreasing the carriage of oxygen by hemoglobin."}, {"id": "wiki20220301en159_7352", "title": "Alveolar–arterial gradient", "score": 0.011560946771474241, "content": "Because A–a gradient is approximated as: (150 − 5/4(P)) – at sea level and on room air (0.21x(760-47) = 149.7 mmHg for the alveolar oxygen partial pressure, after accounting for the water vapor), the direct mathematical cause of a large value is that the blood has a low , a low Pa, or both. is very easily exchanged in the lungs and low Pa directly correlates with high minute ventilation; therefore a low arterial Pa indicates that extra respiratory effort is being used to oxygenate the blood. A low indicates that the patient's current minute ventilation (whether high or normal) is not enough to allow adequate oxygen diffusion into the blood. Therefore, the A–a gradient essentially demonstrates a high respiratory effort (low arterial Pa) relative to the achieved level of oxygenation (arterial ). A high A–a gradient could indicate a patient breathing hard to achieve normal oxygenation, a patient breathing normally and attaining low oxygenation, or a patient breathing hard and still"}, {"id": "wiki20220301en009_161903", "title": "Emergency department", "score": 0.011421911421911422, "content": "Asthma and COPD Acute exacerbations of chronic respiratory diseases, mainly asthma and chronic obstructive pulmonary disease (COPD), are assessed as emergencies and treated with oxygen therapy, bronchodilators, steroids or theophylline, have an urgent chest X-ray and arterial blood gases and are referred for intensive care if necessary. Noninvasive ventilation in the ED has reduced the requirement for tracheal intubation in many cases of severe exacerbations of COPD. Special facilities, training, and equipment An ED requires different equipment and different approaches than most other hospital divisions. Patients frequently arrive with unstable conditions, and so must be treated quickly. They may be unconscious, and information such as their medical history, allergies, and blood type may be unavailable. ED staff are trained to work quickly and effectively even with minimal information."}, {"id": "wiki20220301en013_49192", "title": "Mechanical ventilation", "score": 0.010503025617785834, "content": "When 100% oxygen (1.00 Fi) is used initially for an adult, it is easy to calculate the next Fi to be used, and easy to estimate the shunt fraction. The estimated shunt fraction refers to the amount of oxygen not being absorbed into the circulation. In normal physiology, gas exchange of oxygen and carbon dioxide occurs at the level of the alveoli in the lungs. The existence of a shunt refers to any process that hinders this gas exchange, leading to wasted oxygen inspired and the flow of un-oxygenated blood back to the left heart, which ultimately supplies the rest of the body with de-oxygenated blood. When using 100% oxygen, the degree of shunting is estimated as 700 mmHg - measured Pa. For each difference of 100 mmHg, the shunt is 5%. A shunt of more than 25% should prompt a search for the cause of this hypoxemia, such as mainstem intubation or pneumothorax, and should be treated accordingly. If such complications are not present, other causes must be sought after, and positive"}, {"id": "wiki20220301en246_9470", "title": "Diffuse alveolar damage", "score": 0.010327883498615208, "content": "Berlin Criteria: as stated on UpToDate (2020) Timing: onset of respiratory symptoms within one week of a injury/insult. Chest Imaging: either chest x-ray or CT scan, must show bilateral opacities that cannot be fully explained by other conditions such as effusion, lung/lobar collapse, or lung nodules. Origin of Edema: respiratory failure that cannot be fully explained by cardiac failure or fluid overload, this needs objective assessment such as an echocardiogram. Impaired Oxygenation: this can be determined by looking at the ratio of arterial oxygen tension to fraction of inspired oxygen (PaO2/FiO2) that can be obtained based on an arterial blood gas test. Note: all PaO2/FiO2 ratios used in the determination of the severity of ARDS require that the patient be on a ventilator at a setting that includes 5 cm H2O or more of positive end-expiratory pressure (PEEP) or continuous positive airway pressure (CPAP)."}, {"id": "wiki20220301en010_32317", "title": "Respiratory failure", "score": 0.010301984469370147, "content": "The typical partial pressure reference values are oxygen Pa O2 more than 80 mmHg (11 kPa) and carbon dioxide Pa CO2 less than 45 mmHg (6.0 kPa). Cause Several types of conditions can potentially result in respiratory failure: Conditions that reduce the flow of air into and out of the lungs, including physical obstruction by foreign bodies or masses and reduced breathing due to drugs or changes to the chest. Conditions that impair the lungs' blood supply. These include thromboembolic conditions and conditions that reduce the output of the right heart, such as right heart failure and some myocardial infarctions. Conditions that limit the ability of the lung tissue to exchange oxygen and carbon dioxide between the blood and the air within the lungs. Any disease which can damage the lung tissue can fit into this category. The most common causes are (in no particular order) infections, interstitial lung disease, and pulmonary oedema. Diagnosis"}, {"id": "wiki20220301en362_16701", "title": "Intermittent positive pressure breathing", "score": 0.009994090450863836, "content": "Intermittent positive pressure breathing (IPPB) is a respiratory therapy treatment for people who are hypoventilating. While not a preferred method due to cost, IPPB is used to expand the lungs, deliver aerosol medications, and in some circumstances ventilate the patient. Indications IPPB may be indicated for patients who are at risk for developing atelectasis and who are unable or unwilling to breathe deeply without assistance. In patients with severe lung hyperinflation, IPPB may decrease dyspnea and discomfort during nebulized therapy. Contraindications Most contraindications are relative, such as nausea, hemodynamic instability, tracheal fistula, singulation and hemoptysis. Untreated tension pneumothorax is an absolute contraindication. IMPLEMENTATION When treating atelectasis - Therapy should be volume-oriented 2. Tidal volumes(VT) must be measured 3. VT goals must be set 4. VT goal of 10-15mL/kg ofbody weight"}, {"id": "pubmed23n0284_2385", "title": "[A case of emergency admission for CO2 narcosis in a patient with amyotrophic lateral sclerosis].", "score": 0.009900990099009901, "content": "A 68-year-old man with severe dyspnea was admitted as an emergency case. He had no past history of any respiratory or neuromuscular diseases. Immediately after insufflation of oxygen, respiratory arrest occurred. The blood gas analysis showed hypoxemia and severe hypercapnia (PaO2; 32 mmHg, PaCO2; 127 mmHg). We diagnosed as CO2 narcosis, and he was treated with a respirator in the ICU. He showed nonflaccid bilateral diaphragmatic paralysis and muscle atrophy of the upper extremities. As the EMG showed giant spikes of neurogenic pattern, he was diagnosed as ALS. Weaning from the respirator failed because of his respiratory muscle fatigue. He was given rehabilitation during the day time and ventilatory support with the respirator during the night. We conclude that if we meet with an emergency patient with CO2 narcosis without any pulmonary disorder, we have to suspect neuromuscular diseases, e.q. ALS. In some of such cases, mechanical ventilation supports social rehabilitation."}, {"id": "pubmed23n0718_21084", "title": "[A case of postural hypoxemia with a final diagnosis of myasthenia gravis].", "score": 0.00980392156862745, "content": "We present a case of postural hypoxemia with a final diagnosis of myasthenia gravis (MG). A 62-year-old man experienced double vision in his left eye from December 2008 and received a diagnosis of diabetic neuropathy. From mid-December he began to experience breathing difficulties at night when in a supine position and was admitted to our hospital. Bilateral diaphragmatic elevation was observed on a chest X-ray film, and lower lung atelectasis and an anterior mediastinal tumor were observed on chest CT. However, his breathing difficulties only occurred when he was in a supine position. Therefore, we performed blood gas analysis in supine and sitting positions. Hypoxemia, hypercapnia and an increase in A-aDO2 were observed in the supine position, leading to a diagnosis of postural hypoxemia. Due to the exacerbation of his double vision, the patient was referred to the ophthalmology and neurology departments where he tested positive for anti-acetylcholine receptor antibodies and also on a tensilon test, resulting in a final diagnosis of MG. During the tensilon test, the patient's breathing difficulties in the supine position improved, and therefore his postural hypoxemia was thought to have resulted from diaphragmatic muscle weakness as a result of MG. MG respiratory failure is typically of the acute fulminating type and is considered to be a critical condition. However, it should be noted that there are cases, such as the present one, in which MG presents as postural hypoxemia."}, {"id": "pubmed23n0302_5864", "title": "[Ipsilateral pneumothorax in one-lung respiration. A rare, recently diagnosed and atypical complication of a double lumen tube].", "score": 0.009708737864077669, "content": "The authors report a rare, recently diagnosed and atypical mishap during one-lung ventilation (OLV) via a double lumen tube (DLT) and left-sided thoracotomy: an ipsilateral pneumothorax during ventilation of the right lung. This occurred in a 63-year-old patient with chronic obstructive airway disease who was scheduled for urgent repair of a descending thoracic aortic aneurysm. Anaesthesia and surgery were uneventful until aortic cross-clamping release. The common presentation of increased intrathoracic extrapleural pressure owing to a pneumothorax in patients with mechanically ventilated lungs is a rapid decrease in oxygen saturation, followed or paralleled by haemodynamic deterioration. Although the above presentation could be seen in this case, the diagnosis of a tension pneumothorax was delayed twice. First, symptoms were initially obscured by haemodynamic changes resulting from a head-down tilt and aortic declamping. Second, since the lack of consolidation after aortic declamping focused attention on the airway problems, complications resulting from the use of a DLT were primarily considered. In particular, since breathing sounds were detectable initially, malposition or torsion of the DLT had to be excluded by fibre-optic bronchoscopy, which involved a further delay. Finally, two observations led to the diagnosis of a right-sided tension pneumothorax: (1) bullae of the contralateral lung, detected during thoracotomy; (2) the finding that ventilation of both lungs and the left lung subsequently increased arterial (SaO2) and mixed venous oxygen saturation (SvO2) and the circulatory status, but ventilation of the right lung caused a deterioration. Chest radiography and insertion of a chest tube with drainage of air, thereafter, validated our hypothesis. The time course of oxygen desaturation during OLV and tension pneumothorax was as severe as expected; the time course of haemodynamic deterioration, however, appeared quicker and had more impact than expected. Assuming that mediastinal deviation was not hindered by contralateral intrathoracic pressure during thoracotomy, we believed that circulation should be depressed later or to a lesser extent in patients with an intraoperative pneumothorax. Yet, during thoracotomy, decrease in cardiac filling and output during tension pneumothorax in OLV obviously results primarily from the immovability of the mediastinum owing to mediastinal fixation and is at least as decisive as the contralateral intrathoracic pressure in closed-chest patients. In summary, a tension pneumothorax during one-lung ventilation and thoracotomy is a rare, but disastrous complication during the use of a DLT, which has not, to our knowledge, been reported previously. We recommend that tension pneumothorax be added to the list of complications and problems during OLV by the use of a DLT, especially in patients with structural lung diseases."}, {"id": "wiki20220301en548_4827", "title": "Respiratory compromise", "score": 0.009637188208616781, "content": "Respiratory compromise describes a deterioration in respiratory function with a high likelihood of rapid progression to respiratory failure and death. Respiratory failure occurs when inadequate gas exchange by the respiratory system occurs, with a low oxygen level or a high carbon dioxide level. Causes Patients in acute care hospitals, particularly those with respiratory conditions, are at risk for developing respiratory compromise. Respiratory failure requiring emergency mechanical ventilation occurs in over 40,000 patients per year in the United States. In postoperative patients in the United States, the National Surgical Quality Improvement Program reports that 1.03% of all surgical patients require an unplanned intubation postoperatively."}, {"id": "InternalMed_Harrison_21216", "title": "InternalMed_Harrison", "score": 0.00963027240399503, "content": "General principles of respiratory evaluation in patients with PNS involvement, regardless of cause, include assessment of pulmonary mechanics, such as maximal inspiratory force (MIF) and vital capacity (VC), and evaluation of strength of bulbar muscles. Regardless of the cause of weakness, endotracheal intubation should be considered when the MIF falls to <–25 cmH2O or the VC is <1 L. Also, patients with severe palatal weakness may require endotracheal intubation in order to prevent acute upper airway obstruction or recurrent aspiration. Arterial blood gases and oxygen saturation from pulse oximetry are used to follow patients with potential respiratory compromise from PNS dysfunction. However, intubation and mechanical ventilation should be undertaken based on clinical assessment rather than waiting until oxygen saturation drops or CO2 retention develops from hypoventilation. Noninvasive mechanical ventilation may be considered initially in lieu of endotracheal intubation but is"}, {"id": "wiki20220301en019_80018", "title": "Acute respiratory distress syndrome", "score": 0.009615384615384616, "content": "If Pa:Fi < 300 mmHg (40 kPa), then the definitions recommended a classification as \"acute lung injury\" (ALI). Note that according to these criteria, arterial blood gas analysis and chest X-ray were required for formal diagnosis. Limitations of these definitions include lack of precise definition of acuity, nonspecific imaging criteria, lack of precise definition of hypoxemia with regards to PEEP (affects arterial oxygen partial pressure), arbitrary Pa thresholds without systematic data."}, {"id": "Neurology_Adams_10449", "title": "Neurology_Adams", "score": 0.009615384615384616, "content": "are usually necessary (see further on). However, a fairly severe impairment of ventilation may occur before the first sign of dyspnea appears and before there is elevation of arterial carbon dioxide content. Incipient respiratory failure may be evident by tachypnea and a decrease in arterial oxygen tension (Po2 less than 85 mm Hg) reflecting pulmonary atelectasis. When respiratory failure arises gradually as the patient weakens over days, there is slight tachycardia, diaphoresis, restlessness, and tachypnea. Attempts to forestall intubation and positive-pressure ventilation by using negative-pressure cuirass-type devices have been unsatisfactory in our experience. Patients with oropharyngeal weakness require intubation even earlier so as to prevent aspiration, but full mechanical ventilation is not always necessary at the same time. Patients in these circumstances should obviously be admitted to an intensive care unit staffed by personnel skilled in maintaining ventilation and airway"}, {"id": "pubmed23n1007_14583", "title": "A successful management after preterm delivery in a patient with severe sepsis during third-trimester pregnancy.", "score": 0.009523809523809525, "content": "A 33-year-old woman visited the emergency department presenting with fever and dyspnea. She was pregnant with gestational age of 31 weeks and 6 days. She had dysuria for 7 days, and fever and dyspnea for 1 day. The vital signs were as follows: blood pressure 110/70 mmHg, heart rate 118 beats/minute, respiratory rate 28/minute, body temperature 38.7℃, and oxygen saturation by pulse oximetry 84% during inhalation of 5 liters of oxygen by nasal prongs. Crackles were heard over both lung fields. There were no signs of uterine contractions. Chest X-ray and chest computed tomography scan showed multiple consolidations and air bronchograms in both lungs. According to urinalysis, there was pyuria and microscopic hematuria. She was diagnosed with community-acquired pneumonia and urinary tract infection (UTI) that progressed to severe sepsis and acute respiratory failure. We found extended-spectrum beta-lactamase producing <iEscherichia coli</i in the blood culture and methicillin-resistant Staphylococcus aureus in the sputum culture. The patient was transferred to the intensive care unit with administration of antibiotics and supplementation of high-flow oxygen. On hospital day 2, hypoxemia was aggravated. She underwent endotracheal intubation and mechanical ventilation. After 3 hours, fetal distress was suspected. Under 100% fraction of inspired oxygen, her oxygen partial pressure was 87 mmHg in the arterial blood. She developed acute kidney injury and thrombocytopenia. We diagnosed her with multi-organ failure due to severe sepsis. After an emergent cesarean section, pneumonia, UTI, and other organ failures gradually recovered. The patient and baby were discharged soon thereafter."}, {"id": "pubmed23n0361_22099", "title": "[Mechanical ventilation in neuromuscular diseases: do not start too early, but certainly not too late].", "score": 0.009523809523809525, "content": "Three patients had chronic respiratory disorders: a 42-year-old man with glycogenosis type II was tired, had headaches, poor pulmonary function values and, according to the arterial blood gas values, hypercapnia; a man aged 24 with Duchenne's muscular dystrophy had variable moderate dyspnoea with hypoxia and hypercapnia, and a man aged 64 years with an mitochondrial myopathy complained of dyspnoea and headache but had good blood gas values. The symptoms and abnormalities of the first patient were suppressed by nocturnal ventilatory support through a nasal mask system, the second preferred to refrain from ventilatory support and died a few weeks later and the symptoms of the third patient decreased without ventilatory support. Assessing a ventilatory disorder in patients with a neuromuscular disease is not always simple. Symptoms suggestive of nocturnal hypoventilation may occur in patients without respiratory insufficiency. It is also possible for patients with chronic respiratory insufficiency to be free of symptoms. Determinations of the arterial blood gas values are the most reliable method. Since normal daytime values do not exclude a nocturnal respiratory insufficiency, it is advisable in case of suspicion of nocturnal hypoventilation to measure the arterial blood gas values at night, as well. Nocturnal pulse oximetry does not always adequately reflect the degree of hypoventilation. In view of the positive effects of assisted respiration, adequate diagnostic examinations and early referral to a centre for home mechanical ventilation are advisable."}, {"id": "InternalMed_Harrison_20815", "title": "InternalMed_Harrison", "score": 0.009453882330358, "content": "Whichever mode of MV is used in acute respiratory failure, the evidence from several important controlled trials indicates that a protective ventilation approach guided by the following principles (and summarized in Fig. 323-1) is safe and offers the best chance of a good outcome: (1) Set a target tidal volume close to 6 mL/kg of ideal body weight. (2) Prevent plateau pressure (static pressure in the airway at the end of inspiration) exceeding 30 cm H2O. (3) Use the lowest possible fraction of inspired oxygen (Fio2) to keep the Sao2 at ≥90%. (4) Adjust the PEEP to maintain alveolar patency while preventing overdistention and closure/reopening. With the application of these techniques, the mortality rate among patients with acute hypoxemic respiratory failure has decreased to ~30% from close to 50% a decade ago."}, {"id": "pubmed23n1100_21125", "title": "[Non-invasive ventilation of the lungs in neuromuscular diseases].", "score": 0.009433962264150943, "content": "To describe a clinical case and to analyze our own practice of using NIVL in a myasthenia (MG) gravis patient. Since 2018 in the Republican Research and Clinical Center of Neurology and Neurosurgery NIVL has been performed in 29 patients (21 amyotrophic lateral sclerosis patients and 8 MG patients). The research was carried out using the portable polysomnograph Polymate YH-1000C (BMC, China) and in the Sleep Laboratory of the Republican Clinical Medical Center of the Presidential Administration of the Republic of Belarus using SOMNOlab V 2.19 (Weinmann, Germany). Respiratory support was provided by the Ventimotion 2 device (Weinmann, Germany). The article presents our own experience of using NIVL in MG patient and profound description of the diagnostic and therapeutic complex. The development of chronic respiratory failure in NMD is based on a violation of the ventilation-perfusion ratio in the alveoli as a result of the development of hypoventilation due to restrictive disorders (namely, due to weakness of the respiratory muscles). Compensatory mechanisms eventually lead to an increase in the work on the affected respiratory muscles that leads to the formation of a vicious circle. The use of NIVL provides adequate ventilation of the lungs providing rest for the respiratory muscles. Like any other medical intervention NIVL has indications and contraindications, advantages and disadvantages that are described in this article. The use of NIVL helps to reduce the risk and frequency of respiratory complications, the number and duration of hospitalizations that significantly affects the prognosis and course of NMD as well as improves the quality of life and the level of adaptation of patients."}, {"id": "pubmed23n1060_15322", "title": "Possible silent hypoxemia in a COVID-19 patient: A case report.", "score": 0.009345794392523364, "content": "It has been hypothesized that silent hypoxemia is the cause of rapid progressive respiratory failure with severe hypoxia that occurs in some COVID-19 patients without warning. A 60-year-old male presented cough without any breathing difficulty. Vital signs showed blood pressure 130/75 mmHg, pulse 84x/minute, respiratory rate (RR) 21x/minute, body temperature 36.5C, and oxygen saturation (SpO2) 75% on room air. RT-PCR for COVID-19 were positive. On third day, he complained of worsening of breath shortness, but his RR was still normal (22x/minute) with SpO2 of 98% on 3 L/minute oxygen via nasal cannula. On fifth day, he experienced severe shortness of breath with RR 38x/minute. He was then intubated using a synchronized intermittent mandatory ventilation. Blood gas analysis showed pH 7.54, PaO2 58.9 mmHg, PaCO2 31.1 mmHg, HCO3 26.9mEq/L, SaO2 94.7%, FiO2 30%, and P/F ratio 196 mmHg. On eighth day, his condition deteriorated with blood pressure 80/40 mmHg with norepinephrine support, pulse 109x/minute, and SpO2 72% with ventilator. He experienced cardiac arrest and underwent basic life support, then resumed strained breathing with return of spontaneous circulation. Blood gas analysis showed pH 7.07, PaO2 58.1 mmHg, PaCO2 108.9 mmHg, HCO3 32.1mEq/L, SaO2 78.7%, FiO2 90%, and P/F ratio 65 mmHg. Three hours later, he suffered cardiac arrest again and eventually died. Possible mechanisms of silent hypoxemia are V/Q mismatch, intrapulmonary shunting, and intravascular microthrombi. Silent hypoxemia might be considered as an early sign of deterioration of COVID-19 patients, thus, physician may be able to intervene early and decrease its morbidity and mortality."}, {"id": "pubmed23n0990_9534", "title": "Acute non-invasive ventilation - getting it right on the acute medical take.", "score": 0.009345794392523364, "content": "Non-invasive ventilation (NIV) given to the right patient, in the right setting, in the right way and at the right time improves outcomes. However, national audits reveal poor practice in patient selection, clinical judgement, treatment initiation and availability of trained staff. NIV is indicated for persistent acute hypercapnic respiratory failure (AHRF) with acidosis after usual medical management in chronic obstructive pulmonary disease (COPD) exacerbation and even without acidosis in neuromuscular disorders or other restrictive conditions eg obesity hypoventilation or kyphoscoliosis. Having trained staff in a suitable environment with adequate equipment are keys to its success, along with close monitoring. A plan should be put in place at the time of initiating NIV about the ceiling of care, eg escalation to intubation or palliation, if the patient is not improving with NIV. Early NIV failure is most likely due to technical issues, such as inadequate pressures or mask leak, while late failure is usually the consequence of advanced disease. Any presentation with AHRF is a poor prognostic indicator and outpatient respiratory follow-up is indicated following discharge. For selected patients with COPD who remain hypercapnic 2 weeks after an exacerbation, domiciliary NIV can reduce admissions and improve survival. For patients with neuromuscular disorders or kyphoscoliosis a presentation with AHRF almost always indicates the need for domiciliary NIV."}, {"id": "article-31075_45", "title": "Ventilator Management -- Issues of Concern -- Airway Pressure Release Ventilation (APRV)", "score": 0.009315283902505725, "content": "Minute ventilation, then, will depend on T low and the patient's tidal volumes during T high. Indications for the use of APRV: ARDS that is difficult to oxygenate with AC Acute lung injury Postoperative atelectasis. Advantages of APRV: APRV is a good mode for lung protection ventilation. The ability to set the P high means that the operator has control over the plateau pressure which can significantly lower the incidence of barotrauma Because the patient initiates his respiratory efforts, there is better gas distribution secondary to improved V/Q matching. Constant high pressure means more recruitment (open lung strategy) APRV may improve oxygenation in ARDS patients who are difficult to oxygenate on AC APRV may reduce the need for sedation and neuromuscular blocking agents as the patient may be more comfortable than with other modes. Disadvantages and contraindications:"}, {"id": "pubmed23n0978_13933", "title": "[Successful extracorporeal membrane oxygenation (ECMO) treatment in Legionella pneumonia].", "score": 0.009259259259259259, "content": "The mortality of severe ARDS is almost 60%. Ventilation-associated lung-injury can be avoided by low-pressure, low-volume ventilation. Potential use of ECMO in case of refractory hypoxemia beside modern ventilatory therapy can be considered. Increasing numbers of respiratory ECMO runs are seen worldwide, though the efficacy remains controversial. The authors present the first successful venovenous-ECMO treatment in severe ARDS in our Institute. We report the case of a 67-year-old male who was admitted with community-acquired pneumonia caused by Legionella. Despite empirical and later targeted antibiotic therapy, severe ARDS with sepsis evolved. Neither ventilation nor prone position resulted in permanent improvement in oxygenation. The patient was referred to our Institute for extracorporeal life support (ECLS) therapy. On admission, blood gas showed severe hypoxemia with mild hypercapnia (PaO<sub2</sub/FiO<sub2</sub: 60, pCO<sub2</sub: 53 mmHg at PEEP: 14 mmHg, PIP: 45 mmHg). X-ray showed bilateral patchy infiltrates while cardiac impairment (EF: 45%) and dilated right ventricle were seen on echocardiography. Elevated pulmonary artery pressure (mPAP: 41 mmHg) was measured. After implantation of femoral-jugular VV ECMO, oxygen saturation was appropriate with lung protective ventilation (FiO<sub2</sub: 0.5, TV: 3-4 ml/kg). Improving lung function enabled us to stop ECMO after 8 days and further 5 days later the patient was weaned off ventilation. After 21 days of intensive care we discharged him to the referral hospital. By reporting this case we emphasise the potential role of respiratory ECMO. Consideration should be given to increase the contingent of this modality in the Hungarian intensive care in accordance with international practice. Orv Hetil. 2019; 160(6): 235-240."}, {"id": "pubmed23n1062_16798", "title": "An 84-Year-Old Woman with Shortness of Breath and Low Oxygen Saturation: \"Think Outside the Box\".", "score": 0.009259259259259259, "content": "An 84-year-old woman, who had been admitted to the emergency department (ED) several times because of dyspnoea, was treated for acute exacerbation of chronic respiratory failure without satisfactory clinical improvement. According to her medical history, 8 years earlier, she underwent a complicated cardiosurgical procedure that required tracheostomy and mechanical ventilation in the post-operative period for 45 days. Traditional X-Ray did not show any abnormal findings; however, high resolution thorax computed tomography (HRCT) scan revealed a severe tracheal stenosis, which was confirmed with bronchoscopy, and required immediate tracheostomy. Tracheal stenosis is a rare but severe complication that should be suspected when a patient with previous tracheostomy presents to the ED with dyspnoea even if tracheostomy had been closed many years before, because adaptive mechanism results in asymptomatic life for a long period."}, {"id": "InternalMed_Harrison_2827", "title": "InternalMed_Harrison", "score": 0.00923916068347526, "content": "The most common cause of respiratory hypoxia is ventilation-perfusion mismatch resulting from perfusion of poorly ventilated alveoli. Respiratory hypoxemia may also be caused by hypoventilation, in which case it is associated with an elevation of Paco2 (Chap. 306e). These two forms of respiratory hypoxia are usually correctable by 248 inspiring 100% O2 for several minutes. A third cause of respiratory hypoxia is shunting of blood across the lung from the pulmonary arterial to the venous bed (intrapulmonary right-to-left shunting) by perfusion of nonventilated portions of the lung, as in pulmonary atelectasis or through pulmonary arteriovenous connections. The low Pao2 in this situation is only partially corrected by an Fio2 of 100%."}, {"id": "InternalMed_Harrison_19946", "title": "InternalMed_Harrison", "score": 0.0092381213283414, "content": "Arterial blood gas testing is often helpful in assessing respiratory 1663 disease. Hypoxemia, while usually apparent with pulse oximetry, can be further evaluated with the measurement of arterial PO2 and the calculation of an alveolar gas and arterial blood oxygen tension difference ([A–a]DO2). Patients with diseases that cause ventilation-perfusion mismatch or shunt physiology have an increased (A–a) DO2 at rest. Arterial blood gas testing also allows the measurement of arterial PCO2. Hypercarbia can accompany severe airway obstruction (e.g., COPD) or progressive restrictive physiology, as in patients with neuromuscular weakness."}, {"id": "wiki20220301en159_7349", "title": "Alveolar–arterial gradient", "score": 0.009181707365026935, "content": "For example, consider hypoventilation. Patients can exhibit hypoventilation for a variety of reasons; some include CNS depression, neuromuscular diseases such as myasthenia gravis, poor chest elasticity as seen in kyphoscoliosis or patients with vertebral fractures, and many others. Patients with poor ventilation lack oxygen tension throughout their arterial system in addition to the respiratory system. Thus, the river will have decreased flow throughout both parts. Since both the \"A\" and the \"a\" decrease in concert, the gradient between the two will remain in normal limits (even though both values will decrease). Thus patients with hypoxemia due to hypoventilation will have an A-a gradient within normal limits."}, {"id": "pubmed23n0612_3049", "title": "[Clinical value of noninvasive positive-pressure ventilation in chronic obstruction pulmonary disease combined with type II respiratory failure: a 4-year retrospective study].", "score": 0.009174311926605505, "content": "To evaluate the value of noninvasive positive-pressure ventilation (NIPPV) in treatment of patients with chronic obstruction pulmonary disease (COPD) combined with type II respiratory failure (RF). From June 15th, 2002 to June 15th, 2006, there were 351 inpatients with COPD combined with type II RF. Those treated with NIPPV were categorized as treatment group; those who were not treated by NIPPV served as control group. All patients were divided into four subgroups according to results of blood gas analysis as follows. Mild RF group: 50 mm Hg &lt; or = arterial partial pressure of carbon dioxide (PaCO2) &lt; or = 65 mm Hg, 1 mm Hg=0.133 kPa; medium RF group: 66 mm Hg &lt; or = PaCO2 &lt; or = 80 mmHg; severe RF group: 81 mm Hg &lt; or = PaCO2 &lt; or = 95 mm Hg; extremely severe RF group: &gt; or = 96 mm Hg. NIPPV was used in treatment group on top of conventional treatment. Values of blood gas analysis, length of stay, cost of hospitalization, rate of cannulation and fatality rate were observed in all groups before treatment and after treatment. After being treated with NIPPV, all patients with COPD combined with type II RF in different degrees, arterial partial pressure of oxygen (PaO2) were raised in different degrees, and PaCO2 were all lowered in different degrees. Blood pH, PaO2 and PaCO2 showed statistically significant difference between treatment group and control group in severe and extremely severe RF patients (all P &lt; 0.05). The length of stay of patients with RF in different degrees, was shortened obviously, also the cost of hospitalization, rate of cannulation and fatality rate were all significantly reduced in treatment group. In contrast to mild, medium RF patients, rate of cannulation and fatality rate were increased in extremely severe RF group (all P &lt; 0.05). NIPPV is beneficial to COPD combined with type II RF in different degrees."}, {"id": "pubmed23n0497_23686", "title": "Acute respiratory failure induced by mechanical pulmonary ventilation at a peak inspiratory pressure of 40 cmH2O.", "score": 0.00909090909090909, "content": "The effects of high pressure mechanical pulmonary ventilation at a peak inspiratory pressure of 40 cmH(2)O were studied on the lungs of healthy newborn pigs (14-21 days after birth). Forty percent oxygen in nitrogen was used for ventilation to prevent oxygen intoxication. The control group (6 pigs) was ventilated for 48 hours at a peak inspiratory pressure less than 18 cmH(2)O and a PEEP of 3-5 cmH(2)O with a normal tidal volume, and a respiratory rate of 20 times/min. The control group showed few deleterious changes in the lungs for 48 hours. Eleven newborn pigs were ventilated at a peak inspiratory pressure of 40 cmH(2)O with a PEEP of 3-5 cmH(2)O and a respiratory rate of 20 times/min. To avoid respiratory alkalosis, a dead space was placed in the respiratory circuit, and normocarbia was maintained by adjusting dead space volume. In all cases in the latter group, severe pulmonary impairments, such as abnormal chest roentgenograms, hypoxemia, decreased total static lung compliance, high incidence of pneumothorax, congestive atelectasis, and increased lung weight were found within 48 hours of ventilation. When the pulmonary impairments became manifest, 6 of the 11 newborn pigs were switched to the conventional medical and ventilatory therapies for 3-6 days. However, all of them became ventilator dependent, and severe lung pathology was found at autopsy. These pulmonary insults by high pressure mechanical pulmonary ventilation could be occurring not infrequently in the respiratory management of patients with respiratory failure."}, {"id": "pubmed23n0720_20917", "title": "The addition of a membrane oxygenator to a ventricular assist device in a patient with acute respiratory distress syndrome.", "score": 0.009009009009009009, "content": "A 12-year-old boy with Marfan's syndrome required a biventricular assist device (VAD) after an aortic root replacement. The patient developed acute respiratory distress syndrome and required escalating ventilator support. We hypothesized that the addition of a membrane oxygenator in series with the assist device would improve gas exchange and allow for a more lung-protective ventilator approach. A membrane oxygenator was placed in series with the right VAD resulting in a blood path of right atrium to VAD to oxygenator to pulmonary artery. Circuit function was gauged by monitoring flow and oxygenator pressures and periodic circuit inspections and oxygenator blood gases. Heparin was titrated to maintain unfractionated antifactor Xa levels of .3-.7 IU/mL and partial thromboplastin time of 60-80 seconds. The initial sweep gas supplying the oxygenator was 5 L/min at an F1O2 of 1.0, which achieved a pH &gt; 7.40 and a PF ratio &gt; 250. The pre- and post-oxygenator pressures were 55-60 mmHg and 45-50 mmHg, respectively, and the measured flow at the oxygenator outlet was 2.0-2.2 L/min. The patient was changed from high-frequency oscillatory ventilation to pressure-controlled synchronized intermittent ventilation with pH maintained at 7.35-7.40 and PF ratio &gt; 250. Paralytics were discontinued and the patient's neurologic condition was deemed intact. The patient hemorrhaged after a sternal closure and required transfusions and antifibrinolytics that led to thrombus in the membrane and membrane circuitry, which were replaced without incident. The patient's respiratory status remained stable; however, his overall condition worsened as a result of additional organ dysfunction and septicemia, and he did not survive. The addition of a membrane oxygenator to a VAD is feasible and supplements gas exchange permitting the use of more lung protective ventilation."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[173, 428]], "word_ranges": [[31, 82]], "text": "if we see repercussion in the jugular pulse, it must be in the right cavities. Apart from the fact that the v wave appears during systole, while the atria are filling: if the RV flow rises to the RA, what will happen is that the v wave will be very large?"}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Basic semiology. However, emergency reasoning: systolic murmur, something that in systole had to be closed and is not, or had to open, and does not: options 1, 3 and 5. And if we see repercussion in the jugular pulse, it must be in the right cavities. Apart from the fact that the v wave appears during systole, while the atria are filling: if the RV flow rises to the RA, what will happen is that the v wave will be very large?", "full_answer_no_ref": "Basic semiology. However, emergency reasoning: systolic murmur, something that in systole had to be closed and is not, or had to open, and does not: options 1, 3 and 5. And if we see repercussion in the jugular pulse, it must be in the right cavities. Apart from the fact that the v wave appears during systole, while the atria are filling: if the RV flow rises to the RA, what will happen is that the v wave will be very large?", "full_question": "If in a patient with chronic heart failure we detect prominent v waves in the jugular venous pulse and on cardiac auscultation a holosystolic murmur is auscultated in the area of the xiphoid appendage that is accentuated with deep inspiration. What is the valvulopathy responsible for this physical examination?", "id": 167, "lang": "en", "options": {"1": "Mitral insufficiency.", "2": "Pulmonary insufficiency.", "3": "Tricuspid insufficiency.", "4": "Aortic insufficiency.", "5": "Aortic stenosis."}, "question_id_specific": 85, "type": "CARDIOLOGY AND CARDIOVASCULAR SURGERY", "year": 2013, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en063_19524", "title": "Valvular heart disease", "score": 0.01761025720244072, "content": "On auscultation of a patient with mitral stenosis, there may be a holosystolic murmur at the apex, radiating to the back or clavicular area, a third heart sound, and a loud, palpable P2, heard best when lying on the left side. Patients also commonly have atrial fibrillation. Patients may have a laterally displaced apex beat, often with heave In acute cases, the murmur and tachycardia may be only distinctive signs. Tricuspid regurgitation Patients with tricuspid regurgitation may experience symptoms of right-sided heart failure, such as ascites, hepatomegaly, edema and jugular venous distension."}, {"id": "wiki20220301en063_19525", "title": "Valvular heart disease", "score": 0.01715542521994135, "content": "Tricuspid regurgitation Patients with tricuspid regurgitation may experience symptoms of right-sided heart failure, such as ascites, hepatomegaly, edema and jugular venous distension. Signs of tricuspid regurgitation include pulsatile liver, prominent V waves and rapid y descents in jugular venous pressure. Auscultatory findings include inspiratory third heart sound at left lower sternal border (LLSB) and a blowing holosystolic murmur at LLSB, intensifying with inspiration, and decreasing with expiration and Valsalva maneuver. Patients may have a parasternal heave along LLSB. Atrial fibrillation is usually present in patients with tricuspid regurgitation Causes"}, {"id": "wiki20220301en024_55490", "title": "Aortic regurgitation", "score": 0.01695402298850575, "content": "If there is increased stroke volume of the left ventricle due to volume overload, an ejection systolic 'flow' murmur may also be present when auscultating the same aortic area. Unless there is concomitant aortic valve stenosis, the murmur should not start with an ejection click. There may also be an Austin Flint murmur, a soft mid-diastolic rumble heard at the apical area; it appears when a regurgitant jet of blood from severe aortic regurgitation partially closes the anterior mitral leaflet. Peripheral physical signs of aortic regurgitation are related to the high pulse pressure and the rapid decrease in blood pressure during diastole due to blood returning to the heart from the aorta through the incompetent aortic valve, although the usefulness of some of the eponymous signs has been questioned: Phonocardiograms detect AI by having electric voltage mimic the sounds the heart makes. Characteristics- indicative of aortic regurgitation are as follow:"}, {"id": "pubmed23n0217_12967", "title": "[M-mode echocardiographic standardization of interventricular septal motion and its clinical significance].", "score": 0.015333818344619173, "content": "Systolic and diastolic motions of the interventricular septum (IVS), especially of its lower portion at the level of the chordae tendineae, were evaluated by M-mode echocardiography in normal subjects and in patients with various cardiac disorders. The following conclusions were derived from this study. In normal subjects, downward motion of the IVS exhibited three patterns; namely, P1, between the onset of electrical depolarization and the onset of the second heart sound; P2, between the onset of the second heart sound and the E point of the anterior mitral leaflet; and P3, between the E point of the anterior mitral leaflet and the end of the left ventricular rapid filling phase, during each cardiac cycle. The systolic IVS pattern (P1) of atrial septal defect was classified as follows: Normal type: nearly normal posterior motion during ventricular systole, Flat type: flat motion during ventricular systole, Paradoxical (early systolic) type: anterior motion during the first half of ventricular systole, followed by normal posterior motion, Paradoxical (pansystolic) type: anterior motion during ventricular systole. In atrial septal defect, the right ventricular dimension was markedly increased in the flat and paradoxical (pansystolic) types compared with those of the normal and paradoxical (early systolic) types. Marked downward IVS motion (P2) was observed in cor pulmonale with paradoxical pulse, pulmonary hypertension, Ebstein's anomaly, pulmonic insufficiency, atrial septal defect, funnel chest, tricuspid insufficiency and constrictive pericarditis. In cor pulmonale with paradoxical pulse, the deep downward motion (P2) was observed more distinctly during inspiration compared to expiration, and right ventricular inflow velocity pattern was characterized by an apparent increase in peak flow in velocity of the diastolic rapid filling wave during inspiration. Two interesting findings were a deep \"y\" trough of the jugular pulse tracing and prominent P2 in funnel chest. Therefore, it was likely that exaggerated P2 seemed to be direct evidence of a marked increase in right ventricular rapid filling in the presence of normal or decreased left ventricular rapid filling. The augmented septal dip of P3 was observed in cases with the third heart sound as in normal subjects, and those with mitral insufficiency, and ventricular septal defect, constrictive pericarditis and mitral stenosis. We theorized that exaggerated P3 results from the \"sucking action\" secondary to increased left ventricular rapid filling velocity in cases with the third heart sound or constrictive pericarditis.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "wiki20220301en024_39601", "title": "Mitral stenosis", "score": 0.015211104684788895, "content": "Associated lesions With severe pulmonary hypertension, a pansystolic murmur produced by functional tricuspid regurgitation may be audible along the left sternal border. This murmur is usually louder during inspiration and diminishes during forced expiration (Carvallo’s sign). When the cardiac output is markedly reduced in MS, the typical auscultatory findings, including the diastolic rumbling murmur, may not be detectable (silent MS), but they may reappear as compensation is restored. The Graham Steell murmur of pulmonary regurgitation, a high-pitched, diastolic, decrescendo blowing murmur along the left sternal border, results from dilation of the pulmonary valve ring and occurs in patients with mitral valve disease and severe pulmonary hypertension. This murmur may be indistinguishable from the more common murmur produced by aortic regurgitation (AR), although it may increase in intensity with inspiration and is accompanied by a loud and often palpable P2. Echocardiography"}, {"id": "pubmed23n0270_11588", "title": "[From cardiac auscultation to echo-Doppler. Limitations of both methods].", "score": 0.014149102263856362, "content": "Physiological tricuspid and pulmonary regurgitations are very often found by Echo-Doppler. They are generally slight, inaudible and devoid of significance. Tricuspid insufficiency nevertheless has the great advantage of enabling the calculation of pulmonary pressures. Auscultation is a good method for the diagnosis of rheumatic mitral insufficiency or related to prolapse, but is not reliable in other situations. Doppler is an excellent method for the qualitative and etiological diagnosis of mitral insufficiency but enables only semi-quantification. It also has the disadvantage of discovering minimal mitral insufficiency, the significance of which is uncertain. In contrast to auscultation, Doppler enables precise quantification in mitral stenosis. Auscultation is a good method for the diagnosis of aortic valve disease with the exception of slight insufficiency and stenosis in the elderly. Doppler enables the quantification of stenosis and semi-quantification of insufficiency. The existence of physiological aortic regurgitation is by no means certain. In conclusion, auscultation remains an important tool in cardiological diagnosis but has notable limitations. Echo-Doppler is a major advance but it is important to be aware of its limitations."}, {"id": "wiki20220301en340_257", "title": "Cardiovascular examination", "score": 0.01375534188034188, "content": "For the best cardiac examination, it is important to have the patient both sit up and lay down at a 30-45˚ angle. Tapping with the fingertips (also known as percussion) can be used to estimate the size of the heart, though palpation is more accurate. From the left side of the chest, the doctor can tap the spaces between the ribs with the tips of their middle finger to listen for the dullness that will be present over the heart. Listening with a stethoscope (also known as auscultation) to all four areas of the heart: aortic, pulmonic, tricuspid and mitral. Any murmurs, rubs or gallops should be noted. Gallops are also known as a third (S3) or fourth (S4) heart sound. The absence of abnormalities (normal) may be recorded as \"no m/r/g\". The ACC and the AHA have called cardiac auscultation \"the most widely used method of screening for valvular heart disease.\" Because of its importance to the cardiac examination, cardiac auscultation has been covered in-depth elsewhere."}, {"id": "wiki20220301en068_46138", "title": "Cardiac examination", "score": 0.013636363636363636, "content": "Finally the sacrum and ankles are checked for pitting edema which is caused by right ventricular failure in isolation or as part of congestive cardiac failure. Auscultation One should comment on S1 and S2 – if the splitting is abnormal or louder than usual. S3 – the emphasis and timing of the syllables in the word Kentucky is similar to the pattern of sounds in a precordial S3. S4 – the emphasis and timing of the syllables in the word Tennessee is similar to the pattern of sounds in a precordial S4. If S4 S1 S2 S3 Also known as a gallop rhythm. diastolic murmurs (e.g. aortic regurgitation, mitral stenosis) systolic murmurs (e.g. aortic stenosis, mitral regurgitation) pericardial rub (suggestive of pericarditis) The base of the lungs should be auscultated for signs of pulmonary oedema due to a cardiac cause such as bilateral basal crepitations."}, {"id": "pubmed23n0699_13171", "title": "Ventricular septal defect with aortic valve insufficiency in a New Zealand White rabbit.", "score": 0.013453866395042865, "content": "A heart murmur was detected in a 10 mo old, female New Zealand White rabbit. Auscultation revealed cardiac murmurs both at the left and right hemithorax. Phonocardiography confirmed the systolic-diastolic nature of the left-sided and the systolic character of the right-sided murmur. Electrocardiography showed normal sinus rhythm; tall R waves and large T waves in lead II; and deep S waves in leads II, III, and aVF. Thoracic radiography demonstrated generalized cardiomegaly with prominent pulmonary vasculature. Echocardiography revealed a perimembraneous ventricular septal defect with aortic insufficiency. Signs of biventricular volume overload, relative pulmonic stenosis, and pulmonary valve insufficiency were also seen as consequences of the defect. Necropsy demonstrated a ventricular septal defect just below the aortic valve, a dilated pulmonary trunk, dilated and hypertrophied ventricles, dilated atria, and rightward displacement of the aortic root. Cardiac histopathology showed ventricular cardiomyocyte degeneration (swelling and hypereosinophilia of the cytoplasm with a loss of cross striation, and nuclear hyperchromasia), cartilaginous metaplasia of the aorta, and subendocardial fibrosis of the right ventricular flow tract."}, {"id": "wiki20220301en063_19523", "title": "Valvular heart disease", "score": 0.01325594457966673, "content": "On auscultation of a patient with mitral stenosis, typically the most prominent sign is a loud S1. Another finding is an opening snap followed by a low-pitched diastolic rumble with presystolic accentuation. The opening snap follows closer to the S2 heart tone with worsening stenosis. The murmur is heard best with the bell of the stethoscope lying on the left side and its duration increases with worsening disease. Advanced disease may present with signs of right-sided heart failure such as parasternal heave, jugular venous distension, hepatomegaly, ascites and/or pulmonary hypertension (presenting with a loud P2). Signs increase with exercise and pregnancy. Mitral regurgitation Patients with mitral regurgitation may present with heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea, palpitations, or pulmonary edema."}, {"id": "pubmed23n0859_17506", "title": "The maverick heart sound.", "score": 0.01324644347900162, "content": "An asymptomatic 29-year-old woman presented for prenatal counselling. She had a history of a heart murmur since childhood and a previous echocardiogram suggesting 'enlargement of the heart'. Physical exam revealed normal jugular venous pressure and contour. Precordial palpation was unremarkable. Auscultation, however, was abnormal; findings on inspiration and expiration are presented in Figure 1, sound clip. Based on the phonocardiogram and online supplementary audio clip, which of the following is correct? An early diastolic filling sound (S3) is heard, indicating increased right ventricular filling pressures.An ejection click without respiratory variation and a systolic ejection murmur are heard, consistent with bicuspid aortic valve stenosis.An ejection click with respiratory variation and a systolic ejection murmur are heard, consistent with pulmonic valve stenosis.A holosystolic murmur with inspiratory augmentation is heard, indicating tricuspid regurgitation."}, {"id": "wiki20220301en011_153782", "title": "Heart murmur", "score": 0.013000000000000001, "content": "Mitral regurgitation typically is a holosystolic (pansystolic) murmur heard best at the apex, and may radiate to the axilla or precordium. A systolic click may be heard if there is associated mitral valve prolapse. Valsalva maneuver in mitral regurgitation associated with mitral valve prolapse will decrease left ventricular preload and move the murmur onset closer to S1, and isometric handgrip, which increases left ventricular afterload, will increase murmur intensity. In acute severe mitral regurgitation, a holosystolic (pansystolic) murmur may not be heard. Pulmonary valve stenosis typically is a crescendo-decrescendo systolic murmur heard best at the left upper sternal border, associated with a systolic ejection click that increases with inspiration (due to increased venous return to the right side of the heart) and sometimes radiates to the left clavicle."}, {"id": "wiki20220301en063_19522", "title": "Valvular heart disease", "score": 0.012240632930288103, "content": "Medical signs of aortic regurgitation include increased pulse pressure by increased systolic and decreased diastolic blood pressure, but these findings may not be significant if acute. The patient may have a diastolic decrescendo murmur best heard at left sternal border, water hammer pulse, Austin Flint murmur, and a displaced apex beat down and to the left. A third heart sound may be present Mitral stenosis Patients with mitral stenosis may present with heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea, palpitations, chest pain, hemoptysis, thromboembolism, or ascites and edema (if right-sided heart failure develops). Symptoms of mitral stenosis increase with exercise and pregnancy"}, {"id": "InternalMed_Harrison_2904", "title": "InternalMed_Harrison", "score": 0.012044445701037632, "content": "Tricuspid regurgitation (TR) with normal pulmonary artery pressures, as may occur with infective endocarditis, may produce an early systolic murmur. The murmur is soft (grade 1 or 2), is best heard at the lower left sternal border, and may increase in intensity with inspiration (Carvallo’s sign). Regurgitant “c-v” waves may be visible in the jugular venous pulse. TR in this setting is not associated with signs of right heart failure. Mid-Systolic Murmurs Mid-systolic murmurs begin at a short interval after , end before S (Fig. 51e-1C), and are usually crescendo-decrescendo in configuration. Aortic stenosis is the most common cause of a mid-systolic murmur in an adult. The murmur of AS is usually loudest to the right of the sternum in the second intercostal space (aortic area, Fig. 51e-2) and radiates into the carotids. Transmission of the mid-systolic murmur to the apex, where it becomes higher-pitched, is common (Gallavardin effect; see above)."}, {"id": "article-25204_7", "title": "Double Orifice Mitral Valve -- History and Physical", "score": 0.012024546660769572, "content": "A double orifice mitral valve (DOMV) is usually an asymptomatic entity unless accompanied by mitral valve stenosis, regurgitation, or a concomitant congenital cardiac anomaly. In patients with an associated congenital heart defect, it is usual for that particular disorder to manifest on clinical examination as there are no signs specific to DOMV. In isolated DOMV, the extent of symptoms depends on the grade of left atrial pressure resulting in pulmonary congestion. Symptoms are primarily due to diminished cardiac output and can range from tachypnea, dyspnea, and wheezing to poor feeding and failure to thrive in the pediatric population. In asymptomatic patients, the physical examination may be completely unremarkable as isolated DOMV is benign, even to cardiac auscultation. However, if complicated by mitral valve stenosis (MS), one may appreciate the murmur of MS, a low-pitched mid-diastolic murmur best heard at the apex. The same can be said for DOMV with mitral regurgitation (MR), a blowing pansystolic murmur is appreciated at the apical region. As patients develop symptoms, one may find clinical signs of heart failure such as increased work of breathing, peripheral cyanosis, increased jugular venous pressure, palpation of a parasternal heave, pulmonary crackles on auscultation as well as evidence of hypervolemia such as peripheral edema."}, {"id": "wiki20220301en105_44809", "title": "Right ventricular hypertrophy", "score": 0.012010694740360112, "content": "People may rarely present with the symptoms of Ortner's syndrome, which include cough, haemoptysis and hoarseness. Signs On physical examination, the most prominent features are due to the development of right-sided heart failure. These can include a raised jugular venous pressure, ascites, left parasternal heave and a tender, enlarged liver on palpation. On inspection, patients may be chronically ill, cyanotic, cachectic and occasionally jaundiced. On auscultation, an accentuated second pulmonary sound (S2), a third heart sound termed a ‘right ventricular gallop’, as well as a systolic murmur over the tricuspid area accentuated by inspiration may be present. On occasion, the systolic murmur can be transmitted and auscultated over the liver. Less typically, diastolic murmur may also be heard as a result of pulmonary insufficiency."}, {"id": "InternalMed_Harrison_2927", "title": "InternalMed_Harrison", "score": 0.011913626209977662, "content": "DIASTOLIC HeART MuRMuRS early Diastolic Murmurs (Fig. 51e-1E) Chronic AR results in a high-pitched, blowing, decrescendo, early to mid-diastolic murmur that begins after the aortic component of S2 (A2) and is best heard at the second right interspace (Fig. 51e-6). The murmur may be soft and difficult to hear unless auscultation is performed with the patient leaning forward at end expiration. This maneuver brings the aortic root closer to the anterior chest wall. Radiation of the murmur may provide a clue to the cause of the AR. With primary valve disease, such as that due to congenital bicuspid disease, prolapse, or endocarditis, the diastolic murmur tends to radiate along the left sternal border, where it is often louder than appreciated in the second right interspace. When AR is caused by aortic root disease, the diastolic murmur may radiate along the right sternal border. Diseases of the aortic root cause dilation or distortion of the aortic annulus and failure of leaflet"}, {"id": "InternalMed_Harrison_17405", "title": "InternalMed_Harrison", "score": 0.011729549520247195, "content": "imaging. The evidence base that links the findings from the history and physi-cal examination to the presence, severity, and prognosis of cardiovas-cular disease has been established most rigorously for coronary artery disease, heart failure, and valvular heart disease. For example, obser-vations regarding heart rate, blood pressure, signs of pulmonary congestion, and the presence of mitral regurgitation (MR) contribute importantly to bedside risk assessment in patients with acute coronary syndromes. Observations from the physical examination in this set-ting can inform clinical decision making before the results of cardiac biomarkers testing are known. The prognosis of patients with systolic heart failure can be predicted on the basis of the jugular venous pressure (JVP) and the presence or absence of a third heart sound (S3). Accurate characterization of cardiac murmurs provides important insight into the natural history of many valvular and congenital heart lesions. Finally, the"}, {"id": "wiki20220301en058_11178", "title": "Transthoracic echocardiogram", "score": 0.011686214516403195, "content": "Structures Examples of TTE views of various structures of the heart. Aortic valve Mitral valve Tricuspid valve Pulmonary valve Measurements Routine TTE exams can provide a significant wealth of information about the heart's structure and function: Left ventricular size, thickness, systolic function, and diastolic function Right ventricular size and systolic function Aortic valve Aortic valve sclerosis & stenosis Aortic valve insufficiency Mitral valve Mitral stenosis Mitral regurgitation Tricuspid valve Tricuspid regurgitation (stenosis is possible, but rare) Pulmonary valve Pulmonary regurgitation (stenosis is possible, but rare) Inferior vena cava size as estimate of central venous pressure Aortic root size for thoracic ascending aortic aneurysm Pericardial effusion size"}, {"id": "pubmed23n0549_15147", "title": "[Heart murmur--auscultation or echocardiography in the diagnostic assessment of congenital or valvular heart disease?].", "score": 0.011601301923882569, "content": "The incidence of patients with degenerative valvular but also of patients with congenital heart disease surviving until adulthood or even old age will increase in the next decades. Auscultation with the stethoscope remains an important diagnostic means in the detection and treatment of heart disease. Heart murmurs (especially systolic heart murmurs) are extremely common. There are helpful clues to differentiate heart murmurs. It can occasionally be relatively simple to differentiate a systolic murmur due to valvular heart disease from an innocent, ejection murmur; however, there are important limitations of auscultation. Overall, auscultation and clinical examination alone do not suffice to correctly diagnose and treat patients with heart failure or a murmur Clinically significant aortic stenosis, aortic regurgitation and mitral regurgitation as well as hypertrophic cardiomyopathy are not uncommonly missed or misinterpreted. An echocardiographic exam is mandatory in all patients with more than a soft systolic murmur, any diastolic murmur, cardiac symptoms and/or ECG changes."}, {"id": "InternalMed_Harrison_18629", "title": "InternalMed_Harrison", "score": 0.011458083113139563, "content": "The neck veins in patients with severe TR are distended with prominent c-v waves and rapid y descents (in the absence of TS). TR is more often diagnosed by examination of the neck veins than by auscultation of the heart sounds. Other findings may include marked hepatomegaly with systolic pulsations, ascites, pleural effusions, edema, and a positive hepatojugular reflex. A prominent RV pulsation along the left parasternal region and a blowing holosystolic murmur along the lower left sternal margin, which may be intensified during inspiration (Carvallo’s sign) and reduced during expiration or the strain phase of the Valsalva maneuver, are characteristic findings. The murmur of TR may sometimes be confused with that of MR unless attention is paid to its variation during the respiratory cycle and the extent of RV enlargement is appreciated. Atrial fibrillation (AF) is usually present in the chronic phase of the disease."}, {"id": "wiki20220301en063_19521", "title": "Valvular heart disease", "score": 0.01134391272005951, "content": "Medical signs of aortic stenosis include pulsus parvus et tardus, that is, diminished and delayed carotid pulse, fourth heart sound, decreased A2 sound, sustained apex beat, precordial thrill. Auscultation may reveal a systolic murmur of a harsh crescendo-decrescendo type, heard in 2nd right intercostal space and radiating to the carotid arteries. Aortic regurgitation Patients with aortic regurgitation may experience heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea, palpitations, and angina pectoris. In acute cases patients may experience cyanosis and circulatory shock."}, {"id": "wiki20220301en024_39597", "title": "Mitral stenosis", "score": 0.01104280510018215, "content": "Diagnosis Physical examination Upon auscultation of an individual with mitral stenosis, the first heart sound is usually loud and may be palpable (tapping apex beat) because of increased force in closing the mitral valve. The first heart sound is made by the mitral and tricuspid heart valves closing. These are normally synchronous, and the sounds are termed M1 and T1, respectively. M1 becomes louder in mitral stenosis. It may be the most prominent sign. If pulmonary hypertension secondary to mitral stenosis is severe, the P2 (pulmonic) component of the second heart sound (S2) will become loud."}, {"id": "wiki20220301en011_153755", "title": "Aortic valve", "score": 0.011033300506984717, "content": "Evaluation Evaluation of the aortic valve can be done with several modalities. Auscultation with a stethoscope is quick and easy. It contributes the A2 component to the second heart sound and changes with inspiration (\"splitting\") Transthoracic echocardiography (TTE) is used as the first test because it is non-invasive. Using TTE, the degree of stenosis and insufficiency can be quantified to grade the valve dysfunction. Transesophageal echocardiography is less often used for aortic stenosis & insufficiency because the angle between the probe and the aortic valve is not optimal (the best window is a transgastric view). MRI and CT can be used to evaluate the valve, but much less commonly than TTE. Quantification of the maximum velocity through the valve, the area of the opening of the valve, calcification, morphology (tricuspid, bicuspid, unicuspid), and size of the valve (annulus, sinuses, sinotubular junction) are common parameters when evaluating the aortic valve."}, {"id": "wiki20220301en028_11539", "title": "Jugular venous pressure", "score": 0.010928534167970788, "content": "An exaggerated \"y\" wave or diastolic collapse of the neck veins from constrictive pericarditis is referred to as Friedreich's sign. Raised JVP, normal waveform Bradycardia Fluid overload Heart failure Raised JVP, absent pulsation Superior vena cava syndrome Large 'a' wave (increased atrial contraction pressure) Tricuspid stenosis Right heart failure Pulmonary hypertension Cannon 'a' wave (atria contracting against closed tricuspid valve) Atrial flutter Premature atrial rhythm (or tachycardia) Third degree heart block Ventricular ectopics Ventricular tachycardia Absent 'a' wave (no unifocal atrial depolarisation) Atrial fibrillation Large 'v' wave (c–v wave) Tricuspid regurgitation Absent 'x' descent Tricuspid regurgitation (sometimes 'x' wave is replaced by a positive wave) Prominent 'x' descent Cardiac tamponade Slow 'y' descent Tricuspid stenosis Cardiac tamponade Prominent & deep 'y' descent Constrictive pericarditis"}, {"id": "wiki20220301en011_153783", "title": "Heart murmur", "score": 0.010742771684945165, "content": "Tricuspid valve regurgitation presents as a holosystolic (pansystolic) murmur at the left lower sternal border with radiation to the left upper sternal border. Prominent v and c waves may be seen in the JVP (jugular venous pressure). The murmur will increase with inspiration. Hypertrophic obstructive cardiomyopathy (or hypertrophic subaortic stenosis) will be a systolic crescendo-decrescendo murmur best heard at the left lower sternal border. Valsalva maneuver will increase the intensity of the murmur, as will changing positions from squatting to standing. Atrial septal defect will present with a systolic crescendo-decrescendo murmur best heard at the left upper sternal border due to increased volume going through the pulmonary valve, and is associated with a fixed, split S2 and a right ventricular heave."}, {"id": "wiki20220301en011_153785", "title": "Heart murmur", "score": 0.010713345617267701, "content": "Mitral stenosis typically presents as a diastolic low-pitched decrescendo murmur best heard at the cardiac apex in the left lateral decubitus position. It may be associated with an opening snap. Increasing severity will shorten the time between S2(A2) and the opening snap. (i.e. In severe MS the opening snap will occur earlier after A2) Tricuspid valve stenosis presents as a diastolic decrescendo murmur at the left lower sternal border, and signs of right heart failure may be seen on exam. Pulmonary valve regurgitation presents as a diastolic decrescendo murmur at the left lower sternal border. A palpable S2 in the second left intercostal space correlates with pulmonary hypertension due to mitral stenosis. Continuous and Combined Systolic/Diastolic Patent ductus arteriosus may present as a continuous murmur radiating to the back."}, {"id": "wiki20220301en063_19520", "title": "Valvular heart disease", "score": 0.010549265067866938, "content": "Minor tricuspid insufficiency is common in healthy individuals. In more severe cases it is a consequence of dilation of the right ventricle, leading to displacement of the papillary muscles which control the valve's ability to close. Dilation of the right ventricle occurs secondary to ventricular septal defects, right to left shunting of blood, eisenmenger syndrome, hyperthyroidism, and pulmonary stenosis. Tricuspid insufficiency may also be the result of congenital defects of the tricuspid valve, such as Ebstein's anomaly. Signs and symptoms Aortic stenosis Symptoms of aortic stenosis may include heart failure symptoms, such as dyspnea on exertion (most frequent symptom), orthopnea and paroxysmal nocturnal dyspnea, angina pectoris, and syncope, usually exertional."}, {"id": "InternalMed_Harrison_18229", "title": "InternalMed_Harrison", "score": 0.010532407407407407, "content": "Signs Many of the signs encountered in cor pulmonale are also present in HF patients with a depressed EF, including tachypnea, elevated jugular venous pressures, hepatomegaly, and lower-extremity edema. Patients may have prominent v waves in the jugular venous pulse as a result of tricuspid regurgitation. Other cardiovascular signs include an RV heave palpable along the left sternal border or in the epigastrium. The increase in intensity of the holosystolic murmur of tricuspid regurgitation with inspiration (“Carvallo’s sign”) may be lost eventually as RV failure worsens. Cyanosis is a late finding in cor pulmonale and is secondary to a low cardiac output with systemic vasoconstriction and ventilation-perfusion mismatches in the lung."}, {"id": "wiki20220301en257_19885", "title": "Heart click", "score": 0.010504549214226635, "content": "With newer, non-invasive imaging techniques, the origin of other, so-called adventitial sounds or heart clicks has been appreciated. These are short, high-pitched sounds. The mitral valve in cases of mitral stenosis may open with an opening snap on the beginning of diastole. Patients with mitral valve prolapse may have a mid-systolic click along with a murmur, referred to as apical late systolic murmur. Early systolic clicks may also be present in some patients. Aortic and pulmonary stenosis may cause an ejection click immediately after S1. References Symptoms and signs: Cardiac Audible medical signs"}, {"id": "article-28048_17", "title": "Pulmonary Regurgitation -- History and Physical", "score": 0.010473873247595876, "content": "Cardiac examination findings are typically within normal limits in individuals with physiological pulmonary regurgitation. Some individuals with a lean physique may detect a faint early diastolic murmur in cases of mild pulmonary regurgitation. As pulmonary regurgitation increases significance, a systolic ejection murmur may be audible at the left upper sternal border due to increased right ventricle stroke volume. A third heart sound may be present, while a fourth one is uncommon. Mildly accentuated right ventricle impulse is generally observed in patients with severe pulmonary regurgitation; however, the jugular venous pressure is usually normal. A prominent jugular venous \"a\" wave may indicate pulmonary artery hypertension, while a prominent \"v\" wave is in patients with severe tricuspid valve regurgitation. [27]"}, {"id": "article-28020_14", "title": "Cardiogenic Pulmonary Edema -- History and Physical -- Physical Examination", "score": 0.01019555817610063, "content": "Cardiovascular Findings Tachycardia and hypotension may be present along with jugular venous distention. Auscultation of the heart helps to differentiate between the various causes of valvular lesions causing pulmonary edema. Auscultation typically reveals an S3 gallop in volume overload states, which may be associated with accentuation of the pulmonic component of S2. Several different types of murmurs can be heard depending on the cause of the valvular lesion. Mitral stenosis produces a low-pitched, rumbling diastolic murmur associated with an opening snap at the apex, which becomes accentuated on expiration and produces loud S1. Mitral regurgitation produces a blowing, high-pitched pan-systolic murmur best heard at the apex, radiating to the left axilla and accentuating on expiration, producing soft S1. Aortic stenosis produces a harsh crescendo-decrescendo ejection systolic murmur at the aortic area, increasing on expiration, usually radiating towards the right side of the neck. Aortic regurgitation produces a high-pitched blowing early diastolic murmur best heard in the aortic area, greatest during expiration."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[60, 256]], "word_ranges": [[10, 42]], "text": "as in these six months she has shown a clear clinical and biological improvement (decrease in acute phase reactants), I would maintain the therapeutic approach taken and wait for a new evaluation."}, "3": {"exist": true, "char_ranges": [[392, 530]], "word_ranges": [[64, 89]], "text": "If what you are looking for is a remission of the disease as soon as possible, you could choose to consider associating an anti-TNF alpha?"}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "This is a patient with rheumatoid arthritis. In my opinion, as in these six months she has shown a clear clinical and biological improvement (decrease in acute phase reactants), I would maintain the therapeutic approach taken and wait for a new evaluation. However, I consider that this question could have another valid answer, 3. It depends a little on the attitude of each rheumatologist. If what you are looking for is a remission of the disease as soon as possible, you could choose to consider associating an anti-TNF alpha?", "full_answer_no_ref": "This is a patient with rheumatoid arthritis. In my opinion, as in these six months she has shown a clear clinical and biological improvement (decrease in acute phase reactants), I would maintain the therapeutic approach taken and wait for a new evaluation. However, I consider that this question could have another valid answer, [HIDDEN]. It depends a little on the attitude of each rheumatologist. If what you are looking for is a remission of the disease as soon as possible, you could choose to consider associating an anti-TNF alpha?", "full_question": "A 42-year-old female patient reports pain with inflammatory features and swelling in both wrists, 2nd and 3rd metacarpophalangeal and proximal interphalangeal joints bilaterally and left ankle of 4 months of evolution accompanied by morning stiffness of more than one hour duration. Hand X-ray shows an erosion in the styloid process of the ulna in the right carpus. Laboratory tests showed Hb: 10 g/dL with ESR of 45 mm in the first hour, CRP 16 mg/L, rheumatoid factor 160 IU/ML. After 6 months of treatment with indomethacin and methotrexate, the patient persists with pain and swelling of both carpals, morning stiffness lasting 30 minutes and a CBC showing an ESR 30 mm in the first hour and a CRP 9 mg/dL. Regarding the attitude to take, which of the following is true:", "id": 154, "lang": "en", "options": {"1": "Suspend the prescribed treatment due to lack of response and initiate prednisone at high doses for symptom control only.", "2": "Maintain the therapeutic attitude taken since we have only been on it for 6 months and it would be necessary to wait a minimum of 9 months to evaluate therapeutic response.", "3": "If there is no medical contraindication, consider adding an anti-TNF alpha to the treatment.", "4": "Start a second disease-modifying drug as soon as possible, since it would not be possible to start treatment with biologic therapy alone after methotrexate.", "5": "Consider starting treatment with anti-CD20 therapy associated with methotrexate."}, "question_id_specific": 75, "type": "RHEUMATOLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0494_16537", "title": "A good response to early DMARD treatment of patients with rheumatoid arthritis in the first year predicts remission during follow up.", "score": 0.01951637471439452, "content": "To describe the frequency and duration of remission in the Utrecht rheumatoid arthritis cohort of patients followed since diagnosis, and the clinical and treatment characteristics of patients with remission v those without. In 1990 the Utrecht rheumatoid arthritis cohort study group started a clinical trial in which patients with recent onset of rheumatoid arthritis (&lt;1 year) were randomised into four treatment groups: hydroxychloroquine (n = 169); intramuscular gold (n = 163); methotrexate (n = 166); and pyramid (n = 64). After two years, rheumatologists were allowed to prescribe any disease modifying antirheumatic drug. Remission was defined as: duration of morning stiffness &lt; or =15 min, mean VAS pain &lt; or =10 mm, Thompson joint score &lt; or =10, and ESR &lt; or =30 mm/h during at least six months. Cox regression analysis was used to determine baseline clinical, demographic, and treatment predictors of remission. Mean follow up duration was 62 months. Thirty six per cent achieved at least one period of remission. Median duration between diagnosis and the first remission period was 15 months for the intramuscular gold group, 18 months for the methotrexate and hydroxychloroquine groups, and 24 months for the pyramid group (NS). Predictors of remission were early response to initial treatment, less pain, rheumatoid factor negativity, and lower joint score at baseline. After a mean follow up duration of 62 months, only 36% of the patients had fulfilled the remission criteria at least once. A good response to treatment during the first year seems to be independently associated with remission rather than initial treatment alone."}, {"id": "pubmed23n0579_12858", "title": "[Leflunomide as a second choice treatment in patients with rheumatoid arthritis].", "score": 0.018706293706293706, "content": "Leflunomide is a relatively new disease modifying antirheumatic drug (DMARD) and a number of studies evaluating its effectiveness and safety in daily medical practice is limited. Evaluation of effectiveness and safety of leflunomide treatment in patients with active rheumatoid arthritis in whom methotrexate was ineffective or contraindicated. Eighty one patients (66 women and 15 men) with RA diagnosed according to ARA (The American Rheumatism Association) criteria were included in the study. The mean age was 57.6+/-11.7 years and the mean disease duration was 7.7+/-7.1 years. The inclusion criteria were: disease activity according to DAS28 (Disease Activity Score)&gt;3.2 and contraindications to methotrexate or ineffective methotrexate treatment for at least 3 months. At the beginning of the study 49 of patients were treated with methotrexate in weekly dose of 17+4.2mg and 32 were not treated with DMARDs. Oral glicocorticosteroids in stable doses of 5-15mg of prednisone were given to 66 (77.7%) of them. There was no statistically significant difference in radiological progression of the disease according to Steinbrocker's scale between groups (treated and not treated with methotrexate). Monotherapy with leflunomide was started with loading dose of 100mg for 3 days, and then 20mg daily. Combination therapy was introduced without loading dose. Evaluation was performed monthly and included: duration of morning stiffness, pain and disease activity according to VAS (visual-analogue) scale, the number of tender and swollen joints, blood count, ESR, CRP, aminotransferases activity, and the presence and intensity of adverse reactions. The results of treatment were evaluated after 5 months in 37 of patients and adverse reactions which happened until the end of 5th month were evaluated in all included patients. The mean DAS28 values improved exponentially during consecutive months and the difference between them was statistically significant. Adverse reactions during 5 months of treatment were observed in 36(44,4%) of patients and in 6(7,4%) of cases the treatment had to be stopped because of side effects. The frequency of adverse reactions was similar in monotherapy and combination therapy group. Leflunomide therapy can be effective in patients with active rheumatoid arthritis in whom methotrexate is contraindicated or insufficient. Combination of leflunomide with methotrexate is safe and does not increase the frequency of adverse reactions."}, {"id": "pubmed23n0807_23953", "title": "[Evaluation of methotrexate effect on the acute-phase response in rheumatoid arthritis after 12-week treatment].", "score": 0.018605053651782624, "content": "DAS28 index calculated with regard for ESR, the number of swollen/painful joints and evaluation of the patient's condition by VAS is universally used to estimate activity of rheumatoid arthritis (RA). There is a variant of calculation using C-reactive protein (CRP) instead of ESR. Our experience indicates that ESR decreases more slowly than CRP during treatment and better reflects dynamics of patients' condition. From the practical standpoint it is important to estimate activity of RA because therapeutic modalities are chosen based on the DAS28 value. To study the influence of pharmaceutical form of methotrexate on the acute-phase response in rheumatoid arthritis. The study included 32 patients (24 women, 8 men) aged 19-76 (mean 47.5 +/- 28.5) yr with active RA (DAS28 &gt; 3.2) 4-30 months (11.5 +/- 7.4, median 8) in duration. Diagnosis was made using AXR criteria (1987), none of the patients previously received methotrexate injections. Inclusion criteria: initially high ESR (Westegren, mm/hr) and/or CRP (mg/l measured by a highly sensitive method). All patients were given methotrexate subcutaneously for 12 weeks as monotherapy (initial dose 10 mg, maximum one 25 mg/week). The cumulative dose was 211.36 +/- 17.2 mg. Side effects did not require withdrawal of methotrexate. CRP level decreased faster than ERS: a 70% decrease of CRP by week 12 was recorded more frequently than that of ESR. Slow dynamics of the number of swollen joints compared with CRP may be due to the low cumulative dose of methotrexate. Duration of the disease had no effect on dynamics of acute phase characteristics. Methotrexate injections resulted in markedly delayed development of clinical signs of improvement compared with laboratory values. CFP levels fell down much faster than ESR, Remission or low activity of RA (estimated from DAS28) occurred only in 38% of the cases after 3 month monotherapy by methotrexate injections. It is concluded that efficacy of this drug should be estimated no sooner than 4 months after the onset of the treatment."}, {"id": "pubmed23n0123_2125", "title": "Pulse methotrexate therapy in rheumatoid arthritis. A controlled prospective roentgenographic study.", "score": 0.016504329004329004, "content": "To assess whether weekly pulse methotrexate therapy alters radiographic progression of joint disease in patients with rheumatoid arthritis. Prospective, controlled study. Hand, wrist and foot roentgenograms obtained before, at the onset of, and during methotrexate treatment were scored for degree of joint-space narrowing and erosions by three rheumatologists using a standard method. Sequential sample of 24 patients with active definite or classical rheumatoid arthritis and previous unsuccessful treatment; of these, 3 were excluded due to drug ineffectiveness; 2, due to side effects; and 1, due to refusal to take methotrexate. Treatment with nonsteroidal anti-inflammatory drugs and prednisone was continued. Methotrexate was given weekly to control clinical evidence of disease in patients. After having had an average of 30 months of therapy, the 18 patients who continued to receive methotrexate therapy showed significant (p less than 0.05) clinical improvement, as evidenced by their decreased joint counts and joint scores, duration of morning stiffness, pain scales, and sedimentation rates. Despite patients' prolonged clinical improvement, the mean rate of development of erosions and joint-space narrowing during methotrexate therapy was not significantly different from the rate of radiographic progression before methotrexate therapy (0.043 compared with 0.041; p greater than 0.05). Weekly pulse methotrexate is effective for the long-term management of clinical disease activity in patients with refractory rheumatoid arthritis but may not be a disease-modifying agent by roentgenographic criteria."}, {"id": "pubmed23n0803_17794", "title": "Digital vasculitis in a patient with rheumatoid arthritis responded well to adalimumab.", "score": 0.01595677050222505, "content": "42-year-old old female patient, followed up with diagnosis of rheumatoid arthritis for 15 years, was admitted with necrotising ulcer of left hand 1st and 2nd fingertips and pain, swelling, limitation of movement, and morning stiffness at bilateral wrist, and metacarpophalangeal and proximal interphalangeal joints. Laboratory tests revealed elevated acute phase reactants. Radial and ulnar arteries were clear in upper extremity Doppler ultrasound. The patient was diagnosed as RA activation and digital ulcer and administered iloprost infusion for five days and 1 mg/kg corticosteroid and 20 mg/week methotrexate (MTX). After one month, a partial regression of clinical and laboratory findings was observed. However, 6 months later, due to relapsed and increased complaints and findings, adalimumab 40 mg was administered. Two months later, clinical and laboratory findings apparently decreased. "}, {"id": "wiki20220301en046_42765", "title": "Polymyalgia rheumatica", "score": 0.012615714608263716, "content": "Another test that checks the level of C-reactive protein (CRP) in the blood may also be conducted. CRP is produced by the liver in response to an injury or infection, and people with polymyalgia rheumatica usually have high levels. However, like the ESR, this test is also not very specific. Polymyalgia rheumatica is sometimes associated with temporal arteritis, a condition requiring more aggressive therapy. To test for this additional disorder, a biopsy sample may be taken from the temporal artery. Treatment Prednisone is the drug of choice for PMR, and treatment duration is frequently greater than one year. If the patient does not experience dramatic improvement after three days of 10–20 mg oral prednisone per day, the diagnosis should be reconsidered. Sometimes relief of symptoms occurs in only several hours."}, {"id": "pubmed23n0692_5225", "title": "Remission in early rheumatoid arthritis treated with conventional DMARDs. Results of a two-year follow-up study of El Ayachi Moroccan cohort.", "score": 0.012160633484162896, "content": "This study aimed to evaluate remission in patients with early RA treated by conventional DMARDs and to identify its possible predictor factors. Patients with early RA (&lt;12 months) were enrolled in a 2-year follow-up study. Standard evaluation completed at baseline and at 24 months included clinical, laboratory, functional and structural assessment. Clinical remission after 2 years of follow-up was defined when DAS28 was less than 2.6. Possible predictor factors for remission were analyzed. Fifty-one patients (88.2% women, mean age of 46.9 [24-72] years, mean disease duration of 24 [6-48] weeks) were enrolled in this study. The delay in referral for specialist care was 140 [7-420] days. Rheumatoid factor, anti-CCP, HLA-DRB1*01 and DRB1*04 alleles were present respectively in 62.5, 56.6, 11.8, and 45.1% of patients. At 24 months, 77.2% received a median dose of 5 (0-8) mg/day of prednisone and 65.2% was taking methotrexate (MTX). 13.6% of patients had stopped their DMARD because of socioeconomic difficulties. At 24 months, we noted a significant improvement of morning stiffness, pain score, swollen joint count, ESR, CRP, DAS28 and HAQ scores. Remission at 2 years was noted in 34.8% of patients and was significantly associated in univariate but not in multivariate analysis to male sex (P=0.02) and to short delay in referral for specialist (P=0.03). In this cohort of early RA patients treated with conventional DMARDs, especially with methotrexate in monotherapy, remission at 2-year of follow-up was obtained in one third of patients. No predictor factors of remission were found out. These results should be verified by further studies."}, {"id": "wiki20220301en013_67365", "title": "Methotrexate", "score": 0.011622146071590768, "content": "Not everyone with rheumatoid arthritis responds favorably to treatment with methotrexate, but multiple studies and reviews showed that the majority of people receiving methotrexate for up to one year had less pain, functioned better, had fewer swollen and tender joints, and had less disease activity overall as reported by themselves and their doctors. X-rays also showed that the progress of the disease slowed or stopped in many people receiving methotrexate, with the progression being completely halted in about 30% of those receiving the drug. Those individuals with rheumatoid arthritis treated with methotrexate have been found to have a lower risk of cardiovascular events such as myocardial infarctions (heart attacks) and strokes. Results of a systematic review exploring the comparative effectiveness of treatments of early rheumatoid arthritis can be improved with combination therapy of tumor necrosis factor (TNF) or non-TNF biologics with methotrexate alone."}, {"id": "InternalMed_Harrison_25258", "title": "InternalMed_Harrison", "score": 0.01076555023923445, "content": "In 2012 a joint task force of the ACR and EULAR updated the treatment guidelines for RA. They do make a distinction between patients with early RA (<6 months of disease duration) and patients with established RA. These guidelines highlight the need to switch or add DMARD therapy after 3 months of worsening or persistent moderate/high disease activity. If disease still persists after 3 months of intense DMARD therapy, addition of a biologic agent is warranted. Treatment with a biologic agent or aggressive combination DMARD therapy was also recommended as initial therapy in certain patients with high disease activity and poor prognosis. However, it has not been clearly established that this more intensive initial approach is superior to starting with methotrexate alone and, in the absence of an inadequate therapeutic response, moving rapidly to combination therapy."}, {"id": "wiki20220301en011_6033", "title": "Rheumatology", "score": 0.010296574770258981, "content": "Treatment Most rheumatic diseases are treated with analgesics, NSAIDs (nonsteroidal anti-inflammatory drug), steroids (in serious cases), DMARDs (disease-modifying antirheumatic drugs), monoclonal antibodies, such as infliximab and adalimumab, the TNF inhibitor etanercept, and methotrexate for moderate to severe rheumatoid arthritis. The biologic agent rituximab (anti-B cell therapy) is now licensed for use in refractory rheumatoid arthritis. Physiotherapy is vital in the treatment of many rheumatological disorders. Occupational therapy can help patients find alternative ways for common movements that would otherwise be restricted by their disease. Patients with rheumatoid arthritis often need a long term, coordinated and a multidisciplinary team approach towards management of individual patients. Treatment is often tailored according to the individual needs of each patient which is also dependent on the response and the tolerability of medications."}, {"id": "pubmed23n0863_13824", "title": "Correlations between immunogenicity, drug levels, and disease activity in an Italian cohort of rheumatoid arthritis patients treated with tocilizumab.", "score": 0.009900990099009901, "content": "The aim of this study was to evaluate the real-life immunogenicity of anti-drug antibodies, drug levels, and disease activity in an Italian cohort of rheumatoid arthritis patients treated with tocilizumab (TCZ). We evaluated 126 TCZ-treated patients with rheumatoid arthritis (16 males and 110 females; mean age 59±12 years, range 26-83; mean disease duration 11±5 years) with inadequate 12-week response to any synthetic and biological disease-modifying anti-rheumatic drugs, in a retrospective analysis. One-hundred and seven patients were treated with methotrexate mean dose 12.6±1.3 mg/week in combination with TCZ, 13 received TCZ monotherapy, and six received leflunomide 20 mg/day plus TCZ; all patients were treated with prednisone mean dose 6.4±1.2 mg/day. They had a 28-joint Disease Activity Score (DAS28) of &gt;3.2, an erythrocyte sedimentation rate (ESR) of &gt;30 mm/hour, and CRP levels of &gt;1.0 mg/dL. We evaluated at baseline and after 6 months of treatment: DAS28; rheumatoid factor (RF) IgM, IgA, and IgG; anti-citrullinated peptide antibody; ESR; CRP; TNF-α; and IL-6. TCZ and anti-TCZ antibodies were detected using LISA-TRACKER Duo TCZ. TCZ levels of &lt;10 µg/mL were considered low and &gt;10 µg/mL high. After 6 months of treatment only one patient was positive for anti-TCZ antibodies. There were correlations between DAS28, ESR, and CRP and IL-6 levels in all patients. Comparison of the 84 patients with TCZ levels of &lt;10 µg/mL and the 42 with TCZ levels of &gt;10 µg/mL showed the following differences: DAS28: 3.09±1.32 vs 2.78±1.32, P=0.0005; ESR: 27±14.8 vs 14±12 mm/hour, P=0.0001; CRP: 1.47±1.05 vs 0.65±0.80 mg/dL, P=0.0086; TNF-α: 10.2±1.2 vs 9.9±1.1 pg/mL, P=0.999; IL-6: 3.65±4.75 vs 3.62±4.41 pg/mL, P=0.97; anti-citrullinated peptide antibody: 85.2±93.7 vs 86.7±90.3 IU/mL, P=0.94; RF IgM: 72.4±62.7 vs 68.3±61.6 IU/mL, P=0.754; RF IgA: 41.7±36.4 vs 47.8±42.1 U/mL, P=0.449; and RF IgG: 46.4±46.1 vs 59.3±58.2 U/mL, P=0.212. These findings show that the occurrence of anti-drug antibodies against TCZ is very rare and that there are statistically significant correlations between TCZ levels of &gt;10 µg/mL and ESR, CRP levels, and DAS28. "}, {"id": "pubmed23n0352_3941", "title": "Long-term efficacy and toxicity of cyclosporin A + fluocortolone + methotrexate in the treatment of rheumatoid arthritis.", "score": 0.00980392156862745, "content": "The therapeutic efficacy and tolerability of the combination of cyclosporin A, methotrexate and fluocortolone was evaluated after 96 months of treatment in 140 patients with rheumatoid arthritis. The initial dose of CyA was 5 mg/kg per day and was subsequently modified on the basis of the individual clinical response. Fluocortolone was initially administered at a dose that was sufficient to control disease activity (80-130 mg/week) and then was gradually tapered down to a maintenance dose of 15-20 mg/week. MTX was given intravenously at a dose of 15 mg once weekly for 4 consecutive weeks and then, after a 2-week interval, every 2 weeks or every month depending on the evolution of the disease. At the end of the study a statistically significant improvement was observed in both clinical (VAS, grip-strength, duration of morning stiffness, number of swollen joints, number of painful joints, Ritchie's index and Lee's functional index) and laboratory parameters: ESR (p = 0.000); alpha 2 globulins (p = 0.000); hemoglobin (p = 0.000); CRP (p &lt; 0.001); and rheumatoid factor (p = 0.000). Radiological evaluation revealed little progression in anatomic lesions (Larsen score p = 0.699; number of erosions p = 0.344), thus suggesting that our protocol may be capable of showing down both bone resorption and cartilage loss. Renal toxicity, defined as an increase in plasma creatinine concentrations of more than 50% of the baseline value, was observed in 12 patients (8.5%), but the drug was discontinued in only one, who simultaneously presented high blood pressure. The positive results so far achieved in our study must be interpreted as being due to the combined action of the individual drugs, which made it possible for them to be used at relatively low dosages that minimised the onset of their side effects while maintaining the efficacy of their suppressive action."}, {"id": "pubmed23n0420_22704", "title": "Five-year followup of rheumatoid arthritis patients after early treatment with disease-modifying antirheumatic drugs versus treatment according to the pyramid approach in the first year.", "score": 0.00980392156862745, "content": "To evaluate whether the clinical advantages observed after 1 year in a randomized controlled clinical trial, in which 2 treatment strategies were compared (the early disease-modifying antirheumatic drug [DMARD] approach versus the pyramid approach), persist after 5 years. In this study, 238 patients with recently diagnosed rheumatoid arthritis (RA) were randomized to either the pyramid group (n = 56) or the early DMARD group (n = 182). Patients assigned to the pyramid group received nonsteroidal antiinflammatory drugs for at least 1 year after inclusion (the mean +/- SD lag time until first prescription of a DMARD was 14 +/- 9 months). Patients in the early DMARD group were treated with a DMARD immediately after inclusion. After 5 years, data were available for 44 patients in the pyramid group (79%) and 145 patients in the early DMARD group (80%). No prolongation of the clinical advantages in favor of the early DMARD group, as observed after the first year, was demonstrated. Nevertheless, a significantly shorter delay time until complete response and a higher number of patients with overall clinically relevant improvement at several assessment points were observed in the early DMARD group compared with the pyramid group. The clinical results in favor of the early DMARD group, as observed after the first year, were not as evident after 5 years. This indicates that a more aggressive treatment approach in early RA is required, and that treatment should be continued for a prolonged period of time, in order to maintain the advantages obtained in the first year."}, {"id": "pubmed23n0559_10214", "title": "Prognosis of 5-year radiographic erosions of the wrist according to early, late, and persistent wrist swelling or tenderness in patients with early rheumatoid arthritis.", "score": 0.009708737864077669, "content": "To determine whether early inflammatory activity in the first year of disease compared to persistent or later occurrence of swelling or tenderness in the wrist joints is associated with 5-year erosions in the same joint in patients with early rheumatoid arthritis (RA). A cohort of 195 patients with early active RA was enrolled in the Finnish RA Combination Trial. Swelling and tenderness of wrists were assessed at baseline and at 3, 6, 12, 24, 36, and 48 months. Radiographs of the wrists were taken at the baseline and at 5 years. The 237 wrist joints of 125 patients without erosions at baseline were classified according to wrist swelling, i.e., I: never swollen; II: swollen during first year only; III: swollen during the second to fourth year only; and IV: swollen during the first year and followup, and similarly according to tenderness. Thirty percent of the wrists were never swollen in all clinical examinations; 43% were swollen only during the first year; 11% were not swollen in the first year, but were swollen at some time during 24-48 months; and 16% of wrists were swollen during the first year and at some time during 24-48 months. At 5 years, 64% of 237 wrists remained free of erosions. Erosions developed in 82% of wrists that were swollen during both the first year and 24-48 months, versus 56% of wrists that were not swollen at first year but were swollen during 24-48 months, 31% of wrists that were swollen during the first year only, and 11% of wrists that were never swollen. Similar results were seen for joint tenderness. Wrist swelling during the first year only is associated with less future wrist radiographic damage than persistent swelling or swelling only during the followup. Our results emphasize the value of early and continuous suppression of inflammatory activity in early RA."}, {"id": "pubmed23n0285_18656", "title": "Comparison of cyclosporin A and methotrexate in the treatment of psoriatic arthritis: a one-year prospective study.", "score": 0.009523809523809525, "content": "To compare the effectiveness and toxicity of cyclosporin A (CsA) vs low-dose methotrexate (MTX) over a period of one year in the treatment of psoriatic arthritis (PsA) with peripheral involvement. Thirty-five patients with PsA were enrolled in a prospective, controlled, randomized trial. CsA was initially given in doses of 3 mg/kg/day to a maximum permitted dose of 5 mg/kg/day; MTX was given in oral doses of 2.5 mg every 12 hours for 3 consecutive doses each week up to a maximum dose of 15 mg/weekly. Clinical and laboratory evaluations were performed at entry and monthly thereafter. After 6 and 12 months the number of painful joints, the number of swollen joints, the Ritchie index, the duration of morning stiffness, grip strength, CRP, the patient's and the physician's assessment of PsA activity, as well as the PASI, were significantly improved in both treatment groups. ESR values were significantly reduced only in the MTX group (p &lt; 0.01), which also showed a significantly increase of liver enzymes. The changes in the main clinical and laboratory parameters during the course of CsA or MTX treatment were not significantly different except for the AST and ALT levels (p &lt; 0.05). After one year of therapy CsA and MTX were withdrawn in 41.2% and 27.8% of the patients respectively, but these differences were not statistically significant. Our one-year prospective trial shows that low-dose CsA and MTX are both effective in the treatment of PsA, but the differences in the tolerability of these drugs must be considered at the start of therapy."}, {"id": "pubmed23n0287_17303", "title": "The effectiveness of early treatment with \"second-line\" antirheumatic drugs. A randomized, controlled trial.", "score": 0.009523809523809525, "content": "To compare two therapeutic strategies for patients with recent-onset rheumatoid arthritis. Open, randomized clinical trial. Outpatient clinics of six clinical centers. 238 consecutive patients with recently diagnosed rheumatoid arthritis. Delayed or immediate introduction of treatment with slow-acting antirheumatic drugs (SAARDs). Primary end points were functional disability, pain, joint score, and erythrocyte sedimentation rate at 6 and 12 months and progression of radiologic abnormalities at 12 months. Statistically significant advantages at 12 months for patients receiving the SAARD strategy (immediate treatment with SAARDs) with regard to all primary end points that may be clinically important are indicated by the differences in improvements from baseline and their 95% CIs. These differences were 0.3 (95% CI, 0.2 to 0.6) for disability (range, 0 to 3), 10 mm (CI, 1 to 19 mm) for pain (range, 0 to 100 mm), 39 (CI, 4 to 74) for joint score (range, 0 to 534), and 11 mm/h (CI, 3 to 19 mm/h) for erythrocyte sedimentation rate (range, 1 to 140 mm/h), all in favor of SAARD treatment. The SAARD strategy also appears to be advantageous at 6 months. Radiologic abnormalities progressed at an equal rate in the SAARD and the non-SAARD groups; the difference in progression (range, 0 to 448) was 1 (CI, -3 to 5). Analyses were based on the intention-to-treat principle and thus included 29% of patients in the non-SAARD group who discontinued the non-SAARD treatment strategy; treatment was usually discontinued because of insufficient effectiveness. The SAARD strategy including two alternative SAARDs could not be continued by 8% of patients, usually because of adverse reactions. Early introduction of SAARDs may be more beneficial than delayed introduction for patients with recently diagnosed rheumatoid arthritis."}, {"id": "pubmed23n0622_24421", "title": "[Early arthritis: action desired - treatment required].", "score": 0.009433962264150943, "content": "Rheumatoid Arthritis (RA) is the most prevalent inflammatory joint disease in adults and shows a destructive course in most cases. The outcome of the disease - functional decline and invalidity - necessitates an early therapy. Recent studies demonstrate that the initiation of the treatment with a disease modifying antirheumatic drug (DMARD) treatment within the first three months after the onset of symptoms is crucial for sustained improvement of prognosis as well as therapeutic success and outcome. In the early stage of the disease, the criteria for the classification of Rheumatoid Arthritis (RA) are frequently not met. Up to over 50% of the patients show an arthritis, which cannot be classified and therefore is seen as undifferentiated arthritis (UA). Early therapeutic intervention appears to prevent the chronification of the disease; thus an early and appropriate disease modifying therapy is mandatory. Age, gender, involvement of the hands, positive rheumatoid factor, as well as the detection of anti cyclic-citrullinated peptide antibodies (anti-CCP Ab) are predictors of the development of RA. Beside conventional X-rays, there are other imaging methods such as magnetic resonance tomography imaging, Power-Doppler or contrast medium enhanced sonography, which may enable the detection not only of synovitis but also of erosive lesions at very early stages. Those patients suffering from UA carry a high risk for the development of a destructive arthritis as seen in RA, and therefore should be treated with an adequate DMARD. In these cases methotrexate is still the drug of first choice."}, {"id": "pubmed23n0751_10102", "title": "[Case of successful pregnancy and childbirth in a rheumatoid arthritis patient treated with etanercept].", "score": 0.009345794392523364, "content": "The patient was a 34-year-old woman who, at age 23, was diagnosed with rheumatoid arthritis (RA) presenting with morning stiffness, swelling and tenderness of bilateral knee joints and metacarpophalangeal (MP) joints of the right second and third fingers, increased C-reactive protein (CRP) levels, and a high level of rheumatoid factor (RF). The patient was maintaining remission with oral dose of bucillamine (BUC; 300 mg/day); however, due to the deterioration of arthralgia at age 26, she was additionally administered 8 mg/week of methotrexate (MTX), which improved the symptoms. Thereafter, the prescription of BUC was discontinued. At age 31, she experienced onsets of swelling and tenderness in both the knee joints and wrists and in MP joints of the right second and third fingers; further, CRP levels increased to 5.44 mg/dL, resulting in increased RA activity. The concomitant administration of infliximab was started at a dose of 3 mg/kg, which helped achieve favorable RA control. At age 32, approximately 2 years before childbirth, the prescription of infliximab was changed to 25 mg/dose of etanercept administered twice a week because the patient wished to conceive. Remission was maintained even after the drug change; therefore, MTX was discontinued and the patient was treated with etanercept alone. After she was confirmed to be pregnant in March of the following year, administration of etanercept was continued for treating of RA even during pregnancy. During that time, RA was favorably controlled, and the patient gave birth to a baby boy weighing 3192 g in October of the same year. The Apgar score of the baby was favorable. This case is considered important because, to the best of our knowledge, this may be the first report of a planned pregnancy and childbirth in a patient under administration of a biological preparation."}, {"id": "pubmed23n1015_8061", "title": "Sixth-month remission as a predictor for twelve-month remission in polymyalgia rheumatica.", "score": 0.009259259259259259, "content": "To investigate clinical and laboratory prognostic factors of remission after one year of follow-up in patients with polymyalgia rheumatica (PMR) treated with low-dose prednisone. In this observational study, in a monocentric Italian Rheumatology Unit, we enrolled eighty-one consecutive PMR patients. Clinical and laboratory tests were performed every 3 months. Clinical remission was defined as the lack of symptoms, while laboratory remission was defined as erythrocyte sedimentation rate ≤40 mm/h and C-reactive protein (CRP) ≤0.5 mg/dl. Thirty-eight patients reached complete (clinical and laboratory) remission after 12 months of follow-up. A significant lower percentage of complete remission was seen in female gender compared to male (33.9 % vs. 78.2%, p=0.0001) at univariate analysis. No significant differences were found at baseline according to response to therapy during follow-up, while CRP values at the sixth month were significantly lower in patients who reached complete remission after one year (median: 0.4 mg/dl vs. 1 mg/dl, p=0.017). CRP&lt;0.5 mg/dl at 6 months was independently associated with complete remission at 12 months in the multivariate analysis. The sixth month of therapy is a target for the management of PMR because it can help to identify patients at greater risk of exacerbations, who may benefit from a tighter follow-up and more aggressive therapeutic strategy. Higher CRP values at 6 months appear to be associated with a higher risk of longer steroid therapy."}, {"id": "pubmed23n0674_17896", "title": "Can Cyclosporine-A associated to methotrexate maintain remission induced by anti-TNF agents in rheumatoid arthritis patients? (Cynar pilot study).", "score": 0.009174311926605505, "content": "Biological therapies, such as etanercept, adalimumab and infliximab, have demonstrated good efficacy in inducing rheumatoid arthritis to low disease activity levels. Nevertheless, their cost, as well as the related risk of side effects, especially in long-term therapies, are still high. Furthermore, there is a good deal of evidence proving loss of efficacy of such therapies in the long term, often necessitating the shift from one specific anti-TNF biological treatment to another. There are also other open debates on the amount of time a patient should undergo an anti-TNF therapy, on the possibility of inducing a complete remission in early arthritis and, once remission or low disease activity is obtained, on the possibility of interrupting the anti-TNF-based therapy. In this study we investigated whether A-Cyclosporin and Methotrexate association may be effective in maintaining low disease activity obtained by anti-TNF therapies. Twenty-three rheumatoid arthritis-affected patients, whose diagnosis was made according to ACR criteria, with a disease duration of less than 3 years, and DAS28&lt;3.2 that reached a level of low disease activity within 6-8 months from beginning anti-TNF and Methotrexate therapy, were enrolled in the study. After the suspension of anti-TNF therapy, patients were started on A-Cyclosporine (2-3 mg/kg/day) and Methotrexate (15mg/week) therapy. DAS28, Pain VAS, Erythrosedimentation Rate (ESR), and C Reactive Protein (CRP) were all tested at time 0 and at 6 months, as well as liver and kidney profiles, after the interruption of the anti-TNF therapy and the beginning of A-Cyclosporine and Methotrexate therapy. Side effects were also recorded. Of 23 patients undergoing the A-Cyclosporin and Methotrexate therapy for maintaining low disease activity in rheumatoid arthritis obtained by 6-8 months of anti-TNF therapy, 21 completed the study with a 6 month follow-up. Thirteen patients maintained clinical parameters within low disease activity values, while 8 patients showed an increase in DAS28 and other parameters. Only two patients showed an increase in blood pressure that was diagnosed after two months from the beginning of the A-Cyclosporin and Methotrexate therapy. The reduction in the dosage of A-Cyclosporin from 3mg/kg/day to 2mg/kg/day caused a slow normalization of blood pressure values. Our data seem to suggest that more than half of the patients undergoing A-Cyclosporin and Methotrexate therapy seemed to maintain low disease activity parameters of rheumatoid arthritis, obtained after 6-8 months of anti-TNF therapy. Further studies on larger populations are necessary in order to confirm such results and identify predictor factors for different responses."}, {"id": "pubmed23n0369_18149", "title": "The anti-rheumatic effect of multiple synovectomy in patients with refractory rheumatoid arthritis.", "score": 0.009174311926605505, "content": "We assessed the anti-rheumatic effects of radical multiple synovectomy (RaMS) in patients with rheumatoid arthritis (RA) who did not respond to intensive medical treatment. The selection of patients into three groups, A, B or C, was randomised. Patients assigned to group A (n = 28) continued the prescribed pre-operative medication and had RaMS. Patients assigned to group B (n = 20) were started on a combination therapy with disease-modifying anti-rheumatic drugs (DMARDs) after radical multiple synovectomy. Nineteen RA patients who were started on the same combination therapy as group B but who did not undergo surgery served as controls (group C). The clinical and radiographic findings were assessed for at least 3 years after surgery. Patients in the surgically treated groups (groups A and B) showed a significant reduction in the number of swollen and painful joints and in their ESR and serum CRP levels, and this effect was maintained for at least 3 years. More than 40% of the patients remained in clinical remission during the observation period. The surgical outcome seemed to be superior to that of the controls and did not differ between group A and group B. Articular destruction (assessed by the carpal height ratio) did not progress in the patients who were in clinical remission."}, {"id": "pubmed23n0287_749", "title": "A randomized double-blind controlled trial of sulphasalazine combined with pulses of methylprednisolone or placebo in the treatment of rheumatoid arthritis.", "score": 0.00909090909090909, "content": "Thirty-eight patients with rheumatoid arthritis meeting American College of Rheumatism (ACR) criteria were entered in a randomized controlled trial (RCT) of 6 months to assess whether monthly treatment with i.v. methylprednisolone (MP) enhances or accelerates the efficacy of sulphasalazine (SSZ). All patients had failed at least one second-line agent and were randomized to receive SSZ (2g/day) and pulses of MP (5 mg/kg), or SSZ+ (2 g/day) and pulses of saline (SA). A single infusion of 2 h was carried out in both groups for a total of three times (0, 1 and 2 months). The two groups were comparable at baseline regarding their demographic and clinical characteristics. Disease activity was evaluated every 2 months by means of: (1) joint count; (2) morning stiffness; (3) grip strength; (4) visual analogue pain score; (5) health assessment questionnaire; and (6) erythrocyte sedimentation rate. All outcome measures improved significantly in both groups (P &lt; 0.001). Evaluation at each follow-up visit showed no significant differences between the groups in any of the adverse effects attributable to SSZ therapy (one SA vs two MP). Adverse effects attributable to SA/MP therapy were rare and mild. We concluded that repeated pulses of MP during the first 3 months of treatment did not improve the efficacy of SSZ. Therefore, there is no justification for using MP in this way during the induction phase of SSZ therapy."}, {"id": "pubmed23n0623_10864", "title": "Micafungin plus fluconazole in an infected knee with retained hardware due to Candida albicans.", "score": 0.009009009009009009, "content": "To describe the use of micafungin and fluconazole in the management of a fungal prosthetic joint infection caused by Candida albicans. A 55-year-old female who had undergone total left knee arthroplasty due to rheumatoid arthritis presented with symptoms of a left knee infection. Intravenous vancomycin 1 g every 12 hours and intravenous ampicillin/sulbactam 1.5 g every 6 hours were initiated. Arthrocentesis produced cloudy synovial fluid with a white blood cell (WBC) count of 5.995 x 10(3)/microL. C-reactive protein (CRP) was 19.8 mg/dL and erythrocyte sediment rate (ESR) was greater than 120 mm/h. Gram stain was negative, but intraoperative cultures grew C. albicans. Four days later the patient's condition worsened and repeat arthrocentesis showed WBC count of 16.8 x 10(3)/microL with budding yeast in the synovial fluid. Antibiotics were stopped and liposomal amphotericin B 5 mg/kg once daily was started but was stopped after a few doses due to renal failure. Intravenous micafungin 100 mg daily was initiated; intravenous fluconazole 400 mg daily was added 2 days later and subsequently changed to oral fluconazole after 2 days of therapy. The patient received combination micafungin/fluconazole therapy for 8 weeks. After approximately 8 weeks of therapy, the CRP level and ESR had decreased from 19.8 to 7.1 mg/dL and greater than 120 to 81 mm/h, respectively. The patient's pain and range of motion in her knee had returned to baseline levels at last follow-up after the total knee arthroplasty. After 8 weeks of combination therapy, micafungin was discontinued but oral fluconazole was continued; approximately 8 weeks later the patient relapsed, requiring removal of the prosthetic knee hardware. Fungal prosthetic joint infections are rare, but definitive data regarding appropriate treatment are lacking. Echinocandins are an attractive treatment option due to their enhanced biofilm penetration. In our patient, treatment with micafungin plus fluconazole for 8 weeks followed by fluconazole monotherapy was associated with an initial good outcome in the treatment of a C. albicans prosthetic knee infection with retained hardware. This was, to our knowledge, the first case using micafungin in a prosthetic joint infection. Although micafungin plus fluconazole showed positive results in our patient, more data are needed regarding combination therapy for fungal prosthetic joint infections."}, {"id": "pubmed23n0778_7198", "title": "What is the best treatment strategy for early RA?", "score": 0.009009009009009009, "content": "Treatment of early rheumatoid arthritis has to be started very early, when the diagnosis is made, preferentially before 6 months of symptoms. Combination therapy with conventional disease-modifying anti-rheumatic drugs (DMARDs) with low-dose, oral glucocorticoids in the induction phase from the start gives the best results. The patient should be monitored systematically, at start between 1 and 3 months, and the patient should have access to additional visits if a flare or arthritis or adverse event occurs. The treatment should aim to remission (no tender and swollen joints, no signs of inflammatory activity), which can be reached by 60-80% of the patients. Intra-articular glucocorticoid injections as part of the treatment strategy increase the suppression of arthritis and retard joint destruction. Biological drugs are reserved for patients who have consistent active disease and who do not respond to conventional combinations. "}, {"id": "pubmed23n0348_14495", "title": "Autologous stem cell transplantation: a possible treatment for refractory juvenile chronic arthritis?", "score": 0.008928571428571428, "content": "In adults, autologous stem cell transplantation (ASCT) has been described recently as a possible treatment for severe autoimmune disease refractory to conventional treatment. We report here the four first children with severe forms of juvenile chronic arthritis (JCA) treated with ASCT. We studied three children with systemic JCA and one child with polyarticular JCA. Unprimed bone marrow was harvested 1 month prior to ASCT. T-cell depletion of the graft was performed with CD2 and CD3 antibodies. We used a preparative regimen of antithymocyte globulin (ATG; 20 mg/kg), cyclophosphamide (Cy; 200 mg/kg) and low-dose total body irradiation (TBI; 4 Gy). Methotrexate (MTX) and cyclosporin A (CsA) were stopped before ASCT; prednisone was tapered after 2 months. After ASCT, our patients showed an anti-inflammatory-drug-free follow-up of 6-18 months with a marked decrease in joint swelling, pain and morning stiffness. The erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and haemoglobin (Hb) returned to near-normal values within 6 weeks. Despite T-cell depletion, there was a very rapid immune reconstitution. Two patients developed a limited varicella zoster virus (VZV) eruption which was treated by acyclovir."}, {"id": "InternalMed_Harrison_25234", "title": "InternalMed_Harrison", "score": 0.008916094537942766, "content": "Several developments during the past two decades have changed the therapeutic landscape in RA. They include (1) the emergence of methotrexate as the disease-modifying antirheumatic drug (DMARD) of first choice for the treatment of early RA; (2) the development of novel highly efficacious biologicals that can be used alone or in combination with methotrexate; and (3) the proven superiority of combination DMARD regimens over methotrexate alone. The medications used for the treatment of RA may be divided into broad categories: nonsteroidal anti-inflammatory drugs (NSAIDs); glucocorticoids, such as prednisone and methylprednisolone; conventional DMARDs; and biologic DMARDs (Table 380-2). Although disease for some patients with RA is managed adequately with a single DMARD, such as methotrexate, the situation in most cases demands the use of a combination DMARD regimen that may vary in its components over the treatment course depending on fluctuations in disease activity and emergence of"}, {"id": "InternalMed_Harrison_25256", "title": "InternalMed_Harrison", "score": 0.008905346820809248, "content": "As mentioned earlier, methotrexate is the DMARD of first choice for initial treatment of moderate to severe RA. Failure to achieve adequate improvement with methotrexate therapy calls for a change in DMARD therapy, usually transition to an effective combination regimen. Effective combinations include: methotrexate, sulfasalazine, and hydroxychloroquine (oral triple therapy); methotrexate and leflunomide; and methotrexate plus a biological. The combination of methotrexate and an anti-TNF agent, for example, has been shown in randomized, controlled trials to be superior to methotrexate alone not only for reducing signs and symptoms of disease, but also for retarding the progression of structural joint damage. Predicting which patients will ultimately show radiologic joint damage is imprecise at best, although some factors such as an elevated serum level of acute-phase reactants, high burden of joint inflammation, and the presence of erosive disease are associated with increased"}, {"id": "pubmed23n0644_20107", "title": "[Effects of wenhua juanbi recipe on TNF-alpha and IL-1beta in peripheral blood of rheumatoid arthritis patients].", "score": 0.008849557522123894, "content": "To observe the clinical effect of Wenhua Juanbi Recipe (WJR) in treating rheumatoid arthritis (RA), its effects in reducing the dosage of Western medicine used and stabilizing condition of disease, as well as its influences on peripheral blood levels of tumor necrosis factor alpha (TNF-alpha), interleukin 1beta (IL-1beta) and anti-cyclic citrullinated peptide antibody (anti-CCP), for the sake of exploring its preliminary acting mechanism. One hundred patients with RA were randomly assigned to 2 groups, the control group and the treated group, 50 in each group. All were treated with oral administration of methotrexate (MTX,7.5 mg per week), sulfasalazine (0.5 g, tid) and meloxicam (Mobic, 7.5 mg, bid), but to the treated group WJR was given additionally. The therapeutic course for both groups was 3 months. Clinical effect, changes of symptoms and physical signs, dosages of western medicines used, and laboratory indices in 2 groups after treatment were observed, and cases of relapse 3 months after treatment were figured out. The total effective rate in the treated group was higher than that in the control group (88.0% vs 76.0%, P&lt;0.05). The improvements in scores of symptoms and signs [joint pain (0.61 +/- 0.59), swelling (1.49 +/- 1.20), tenderness (0.90 +/- 0.69), movement (0.68 +/- 0.62), griping strength (68.56 +/- 6.50) mm Hg, morning stiff time (23.26 +/- 9.26) min], and in levels of laboratory indices (TNF-alpha, IL-1beta, anti-CCP, RF, ESR, CRP, PLT and Ig) in the treated group after treatment were significantly superior to those in the control group (P&lt;0.05 or P&lt;0.01). The dosages of MTX [(82.11 +/- 11.35) mg vs (94.75 +/- 10.23) mg] and meloxicam [(108.85 +/- 16.13) mg vs (189.63 +/- 18.44) mg] used, and the relapse rate in the treated group were lower significantly (P&lt;0.05, P&lt;0.01) than those in the control group respectively. Effect of combined therapy of WJR and Western medicines is superior to that of using Western medicines alone in treating RA; WJR can reduce the dosages of Western medicines used and the relapse rate, as well as stabilize the condition of illness. It has the effects of immune regulating and anti-inflammatory reaction. Its mechanism for treating RA is possibly the inhibition on cytokines of TNF-alpha and IL-1beta."}, {"id": "pubmed23n0653_215", "title": "The Stop Arthritis Very Early (SAVE) trial, an international multicentre, randomised, double-blind, placebo-controlled trial on glucocorticoids in very early arthritis.", "score": 0.008849557522123894, "content": "Glucocorticoids (GCs) are often used as early arthritis treatment and it has been suggested that they induce remission or at least delay the development of rheumatoid arthritis (RA) and the need to start disease-modifying antirheumatic drugs (DMARDs). To test the effect of GCs on patients with very early arthritis (symptom duration of &lt;16 weeks) in a randomised controlled trial. Patients received a single intramuscular injection of 120 mg methylprednisolone or placebo (PL) and were followed up for 52 weeks. Primary end point was drug-free clinical remission, both at weeks 12 and 52. Among secondary outcomes were fulfillment of remission criteria at weeks 2, 12 or 52, time course of 'core set variables' and proportion of patients starting DMARDs. 17.0% of all analysed subjects (65/383) achieved persistent remission: 17.8% (33/185) of the PL group, 16.2% (32/198) of the patients receiving methylprednisolone (OR=1.13, 95% CI 0.66 to 1.92, p=0.6847). Analyses of secondary end points showed significant clinical benefits of the GC only at week 2. These differences subsequently disappeared. DMARDs were started in 162 patients: 50.3% methylprednisolone and 56.7% PL patients had to start DMARD treatment (OR=0.78, 95% CI 0.49 to 1.22, p=0.30). Significantly more patients with polyarthritis than with oligoarthritis received DMARDs (OR=2.84, 95% CI 1.75 to 4.60, p&lt;0.0001). Neither remission nor development of RA is delayed by GC treatment. Remission is rare in the first year of very early arthritis, occurring in &lt;20% of the patients. Also, the need to start DMARDs was not influenced by GC treatment."}, {"id": "wiki20220301en001_89043", "title": "Rheumatoid arthritis", "score": 0.008793290043290044, "content": "Monitoring progression Many tools can be used to monitor remission in rheumatoid arthritis. DAS28: Disease Activity Score of 28 joints () is widely used as an indicator of RA disease activity and response to treatment. Joints included are (bilaterally): proximal interphalangeal joints (10 joints), metacarpophalangeal joints (10), wrists (2), elbows (2), shoulders (2) and knees (2). When looking at these joints, both the number of joints with tenderness upon touching (TEN28) and swelling (SW28) are counted. The erythrocyte sedimentation rate (ESR) is measured and the affected person makes a subjective assessment (SA) of disease activity during the preceding 7 days on a scale between 0 and 100, where 0 is \"no activity\" and 100 is \"highest activity possible\". With these parameters, DAS28 is calculated as: From this, the disease activity of the affected person can be classified as follows:"}, {"id": "pubmed23n0363_22997", "title": "Toxic epidermal necrolysis following combination of methotrexate and trimethoprim-sulfamethoxazole.", "score": 0.008771929824561403, "content": "A 15-year-old boy with T-cell acute lymphoblastic leukemia (ALL) (FAB L1), diagnosed in 1995, received combination chemotherapy consisting of 6 weeks of induction (vincristine, epirubicin, L-asparaginase, prednisolone) and 2 weeks of consolidation (cytosine arabinosides, etoposide). After achieving remission, for further maintenance of remission, he was treated with 14 cycles of intensive chemotherapy consisting of 6-MP, 10 mg/kg orally on the first 4 days, and cyclophosphamide, 1200 mg/m2, vincristine, 1.5 mg/m2, epirubicin, 15 mg/m2, and cytosine arabinoside, 40 mg/m2, intravenously on days 4, 11, 39, and 40, respectively. On day 18 of each cycle, he received intravenous methotrexate (MTX) infusion in a total dose of 150 mg/m2 plus oral leucovorin (30 mg/m2 ) rescue 36 h after starting MTX therapy. In addition, oral trimethoprim-sulfamethoxazole was given regularly to prevent Pneumocystis carinii infection. The patient achieved remission during the first course of treatment, but 8 months later the disease relapsed. He then received four doses of MTX (800 mg intravenously) plus leucovorin rescue in the following 4 months. During the last MTX therapy, small hemorrhagic bullae were found on the lateral side of the right ankle, but subsided after a few days. Due to partial remission of the disease, he was admitted again in January 1999 for high-dose MTX therapy. An initial hemogram on admission revealed hemoglobin 7.2 g/dL, white cell count 15,200/mm3, platelet count 153/mm3, blood creatinine 0.5 mg/dL, and alanine leucine aminotransferase (ALT) 20 U/L. He received 8500 mg of MTX (5000 mg/m2 ) as a continuous intravenous infusion for 24 h. Thirty-six hours after the start of MTX infusion, leucovorin (30 mg, intravenous) rescue was initiated every 6 h for 3 days. Another preventive measure to cover MTX toxicity included aggressive intravenous fluid replacement (4 L/m2 /day) and the addition of 25 meq/L sodium bicarbonate to the intravenous fluid to alkalinize the urine. Concurrent medication included 6-MP (50 mg) once daily and trimethoprim-sulfamethoxazole (120 mg, 600 mg) twice daily every other day. Plasma MTX levels were 52.36 micromol/L 24 h after MTX infusion, 1.87 micromol/L after 48 h, 0.57 micromol/L after 72 h, and 0.41 micromol/L after 96 h. These indicated delayed MTX plasma clearance. The blood creatinine level was mildly elevated from 0.5 mg/dL to 0.7 mg/dL. Thirty-six hours after the administration of MTX, the patient developed an erythematous painful swelling on the right middle finger. The erythema, with subsequent large bulla formation, progressed to all the fingers, toes, palms, and the soles of the feet. Some erythematous to hemorrhagic papules also appeared on the bilateral elbows. Subsequently, diffuse tender erythema with extensive erosions and focal tiny pustules developed on the back, abdomen, proximal extremities, and face (Fig. 1a,b). A positive Nikolsky's sign was also present. A biopsy specimen of the right dorsal hand lesion revealed parakeratosis, detached acanthotic epidermis with scattered necrotic keratinocytes, dyskeratotic cells and nuclear atypia, neutrophilic exocytosis, and many neutrophils in the papillary dermis (Fig. 2). The skin condition deteriorated rapidly. Toxic epidermal necrolysis-like lesions involved 90% of the total body surface on the fifth day after MTX infusion. Mucositis, diarrhea, involuntary tremor, fever, and chills were noted. The patient was then sent to the burn unit for intensive skin care. Ten days after MTX therapy, profound agranulocytosis and thrombocytopenia (white cell count 100/mm3, platelets 14,000/mm3, and hemoglobin 5.6 g/dL) were found. The patient was then started on granulocyte colony stimulation factor (G-CSF, 5 microg/kg/day), but his general condition deteriorated rapidly and he died 6 days later due to septic shock and multiple organ failure."}, {"id": "pubmed23n0490_11780", "title": "Patient retention and hand-wrist radiograph progression of rheumatoid arthritis during a 3-year prospective study that prohibited disease modifying antirheumatic drugs.", "score": 0.008771929824561403, "content": "To quantitate patient retention and radiographic progression rates in serial hand/wrist radiographs of patients with rheumatoid arthritis (RA) who were not being treated with disease modifying antirheumatic drugs (DMARD). A total of 1433 RA patients with 1-7 years' disease duration entered a 3-year prospective randomized double-blind clinical trial comparing the nonsteroidal antiinflammatory drugs (NSAID) etodolac (300 or 1000 mg daily) and ibuprofen (2400 mg daily). Standardized hand/wrist radiographs were obtained yearly and at dropout if &gt; 6 months after entry. DMARD were not permitted. Joint erosion, joint space narrowing (JSN), and total scores of 3 readers were averaged. At entry, mean duration of RA was 3.5 years (range 1-7); ages were 21-78 years; patients were 71% female, 84% Caucasian, 67% rheumatoid factor (RF) positive; tender joint count was 29, swollen joint count 22, Westergren erythrocyte sedimentation rate (ESR) 49, and C-reactive protein (CRP) 2.44. There were 824 (57.5%) patients who completed &gt;or= 6 months and had paired radiographs; 46% completed 48 weeks; 31%, 98 weeks; and 19%, 147 weeks. Months between paired radiographs (time in study) averaged 23.1 (range 6-36). Mean progression rates for total, erosion, and JSN scores (5.08, 2.53, and 2.54 units per year, respectively) were significantly associated with time in study, baseline RF, ESR, CRP, swollen joint count, presence of erosions at entry, and with 20% and 50% composite clinical responses. Painful joint count and RA duration were weakly associated only with progression of erosions. Progression rates were not associated with age, sex, corticosteroid use, or prior DMARD use. Patients who completed the 3-year trial had less severe disease activity and radiographic progression than those who dropped out. In this 3-year prospective double-blind clinical trial that prohibited DMARD, retention rates (57.5%, 46%, 31%, and 19% at 0.5, 1, 2, and 3 years) were similar to those in the non-DMARD-treated placebo groups of recent published studies. Radiographic progression rates are reported for 824 non-DMARD-treated patients during RA of 1-10 years' duration. This information may be useful as background information in the interpretation of longterm clinical trials that evaluate joint radiographic outcomes."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[173, 319]], "word_ranges": [[29, 48]], "text": "In principle, antibiotic treatment is correct and before considering changes in treatment, the possibility of empyematization should be evaluated."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "As long as fever persists in an evolving pneumonia, there is a risk of empyematization, especially if a small effusion has already occurred at the beginning of the picture. In principle, antibiotic treatment is correct and before considering changes in treatment, the possibility of empyematization should be evaluated.", "full_answer_no_ref": "As long as fever persists in an evolving pneumonia, there is a risk of empyematization, especially if a small effusion has already occurred at the beginning of the picture. In principle, antibiotic treatment is [HIDDEN] and before considering changes in treatment, the possibility of empyematization should be evaluated.", "full_question": "A 56-year-old woman with a history of well-controlled schizophrenia and no toxic habits. Admitted for middle lobe pneumonia with a small associated metaneumonic pleural effusion and on treatment with levofloxacin 500 mg/24h . She presented good clinical evolution except for persistent febrile fever and leukocytosis on the sixth day of treatment. Microbiological studies are not available. The most appropriate course of action is:", "id": 176, "lang": "en", "options": {"1": "The evolution is normal, treatment should be maintained until completing 10 days.", "2": "It is considered a therapeutic failure and antibiotic treatment should be modified.", "3": "Perform thoracentesis to rule out empyema.", "4": "Add corticosteroids at a dose of 0.5 mg/Kg/day to antibiotic treatment.", "5": "Bronchoscopy with biopsy, aspiration and bronchoalveolar lavage."}, "question_id_specific": 57, "type": "PNEUMOLOGY", "year": 2013, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0518_17855", "title": "[Failure of levofloxacin therapy in two cases of community-acquired pneumonia caused by fluoroquinolone-resistant Streptococcus pneumoniae and complicated with empyema].", "score": 0.01951265943270512, "content": "Community acquired pneumonia (CAP) due to Streptococcus pneumoniae is a frequent cause of morbidity and mortality. We communicate two cases of CAP with complications. In both cases levofloxacin-resistant S. pneumoniae was isolated in pleural effusion. Patient 1: A 51-year-old man who had not received previous treatment with quinolones was admitted to the hospital for CAP and initially treated with levofloxacin (500 mg/24h iv). Four days later pleural effusion developed and fluid culture isolated levofloxacin-resistant S. pneumoniae (MIC &gt; 32 .g/ml). The outcome was favorable following chest tube placement and treatment with beta-lactam antibiotics. Patient 2: A 73-year-old man with a history of chronic obstructive pulmonary disease was admitted due to CAP and was initially treated with levofloxacin (500 mg/24 h iv). He was transferred to our hospital after 10 days of treatment with this antibiotic, following the development of pleural effusion with isolation of levofloxacin-resistant S. pneumoniae (MIC = 12 .g/ml). The patient was treated with chest tube placement and beta-lactam antibiotics with a favorable outcome. Patients with CAP treated empirically must be closely followed, both clinically and radiologically, to facilitate early detection of complications due to bacterial resistance to the prescribed antibiotic. Patients with CAP who have received quinolones in the weeks before the development of pneumonia should not been treated empirically with these antibiotics because of the risk of resistance development."}, {"id": "pubmed23n1023_1806", "title": "Severe Pulmonary Infection in a 20-Month-Old Female.", "score": 0.012444444444444445, "content": "Community-Acquired Pneumonia (CAP) is a common reason for hospitalization of a pediatric patient. We report a 20-month-old female admitted for suspected CAP. History included a week-long cough, fever, dyspnea, single occurrence of seizure-like activity, and a sick contact. Initial chest X-ray (CXR) showed left lower lobe pneumonia and parapneumonic effusion with a complex left pleural effusion. Ultrasound findings prompted the need for contrast-enhanced computed tomography (CT) of the chest. Contrast-enhanced CT of the chest confirmed a large pleural effusion with major atelectasis and mediastinal shift. The patient was treated with empiric antibiotics, video-assisted thoracoscopic surgical (VATS) decortication of empyema, and chest tube placement. Due to intraoperative complications, the VATS decortication was aborted and patient was transferred to the pediatric intensive care unit (PICU). A thoracentesis with culture failed to isolate a bacterial organism. Dexamethasone was started after repeat CXR showed persistent infiltrate. Subsequent contrast-enhanced CT of the chest showed a large collection of air and persistent consolidation. The patient received repeat VATS decortication and reinsertion of a chest tube. Repeat pleural fluid cultures failed to isolate a bacterial organism. Infectious disease (ID) consult recommended linezolid 140 mg Q8H for 4 weeks. Seven days after second VATS, a respiratory pathogen panel was positive for rhinovirus/enterovirus. With resolution of leukocytosis and clinical improvement, the patient was discharged with the chest tube in place and pediatric surgery outpatient follow-up. After three months, sequalae from both the infection and interventions presented ."}, {"id": "wiki20220301en116_5576", "title": "Hospital-acquired pneumonia", "score": 0.012153455681374464, "content": "Although gram-negative bacilli are a common cause they are rarely found in the respiratory tract of people without pneumonia, which has led to speculation of the mouth and throat as origin of the infection. Diagnosis In hospitalised patients who develop respiratory symptoms and fever, one should consider the diagnosis. The likelihood increases when upon investigation symptoms are found of respiratory insufficiency, purulent secretions, newly developed infiltrate on the chest X-Ray, and increasing leucocyte count. If pneumonia is suspected material from sputum or tracheal aspirates are sent to the microbiology department for cultures. In case of pleural effusion, thoracentesis is performed for examination of pleural fluid. In suspected ventilator-associated pneumonia it has been suggested that bronchoscopy or bronchoalveolar lavage is necessary because of the risks of incorrect clinical diagnoses."}, {"id": "wiki20220301en045_78654", "title": "Aspiration pneumonia", "score": 0.011677814938684503, "content": "Treatment is typically with antibiotics such as clindamycin, meropenem, ampicillin/sulbactam, or moxifloxacin. For those with only chemical pneumonitis, antibiotics are not typically required. Among people hospitalized with pneumonia, about 10% are due to aspiration. It occurs more often in older people, especially those in nursing homes. Both sexes are equally affected. Signs and symptoms The person may have an insidious course with increased respiratory rate, foul-smelling sputum, hemoptysis, and fever. Complications may occur, such as exudative pleural effusion, empyema, and lung abscesses. If left untreated, aspiration pneumonia can progress to form a lung abscess. Another possible complication is an empyema, in which pus collects inside the lungs. If continual aspiration occurs, the chronic inflammation can cause compensatory thickening of the insides of the lungs, resulting in bronchiectasis."}, {"id": "wiki20220301en025_92733", "title": "Pleural empyema", "score": 0.010561716556880402, "content": "and oral therapy. Switching to oral antibiotics can be considered upon clinical and objective improvement (adequate drainage and removal of chest tube, declining CRP, temperature normalization). Oral antibiotic treatment should then be continued for another 1–4 weeks, again based on clinical, biochemical and radiological response."}, {"id": "wiki20220301en424_25378", "title": "Borrelia turicatae", "score": 0.009900990099009901, "content": "Treatment for relapsing fever can include various antibiotics. TBRF spirochaetes are susceptible to penicillin and other β-Lactam antibiotics, as well as tetracyclines, macrolides, and possibly fluoroquinolones. Although the CDC has not yet developed specific treatment guidelines for TBRF, experts generally recommend tetracycline 500 mg every 6 hours for 10 days as the preferred oral regimen for adults. If tetracyclines are contraindicated, erythromycin, 500 mg (or 12.5 mg/kg) every 6 hours for 10 days is an effective alternative. For patients with central nervous system involvement, parenteral therapy with ceftriaxone 2 g/day for 10–14 days is preferred. All patients treated with antibiotics should be observed during the first 4 hours of treatment for a Jarisch-Herxheimer reaction, which is a worsening of symptoms characterized by rigors, hypotension, and high fever. The reaction occurs in over 50% of cases and may be difficult to distinguish from a febrile crisis. Given appropriate"}, {"id": "pubmed23n0287_22187", "title": "[Etiologic aspects and therapeutic problems of purulent pleurisy in Abidjan (Côte d'Ivoire)].", "score": 0.009900990099009901, "content": "We present the results of a retrospective study of 127 cases of empyema admitted to the pneumophtisiology department of the Centre hospitalier universitaire de Treichville (Abidjan), between January 1985 and December 1989. We present the pathogens identified in the pleural fluid and the course of the disease during treatment by repeat thoraco-centesis and systemic antibiotics. During the study period, pleural empyema represented 2.7% of all admissions to the pneumophtisiology department, and 20.5% of those presenting with pleural effusions. Bacteriological examination was recovered in 88 of the 127 patients, and was positive in 57 cases (64.7% of those examined). Of those with positive bacteriology, 50 (56.8%) had non-tuberculous bacterial infections, and 7 (7.9%) had tuberculous infection. Among the non-tuberculous bacterial infections, Gram-negative bacilli were most common (72%), and Pseudomonas was the species most frequently identified (48%). The mean stay in hospital was 47 days (range 10-143) and in 82 patients (64.6%), the outcome was favourable. The presentation was complicated by encystment in 36 cases (28.4%) and 9 patients (7%) died in hospital."}, {"id": "pubmed23n0261_5835", "title": "[Treatment of community-acquired pneumonia by pristinamycin (Pyostacine 500). Results of a non comparative open study].", "score": 0.00980392156862745, "content": "Activity of natural streptogramin (NSG) appears well adapted to pathogens responsible for CAP. The goal of this multicenter pilot study was to bring first data about efficacy of NSG in treatment of CAP. PATIENTS METHOD: Ten days of a NSG (1 gr b.i.d. or t.i.d.) regimen was administered to 46 hospitalized adult patients for CAP defined with fever &gt; 38 degrees C, respiratory symptoms and X-ray opacity. Severely ill patients were excluded. A broncho-pulmonar sample (expectoration or trantracheal aspiration or protected distal sample) was performed in all patients. two patients were excluded because of pulmonary embolism (n = 1) or tuberculosis (n = 1) and 44 patients were analyzed. 50% of them had associated disease, 20% had failure of prior antibiotherapy. At inclusion, mean fever was 39.2 +/- 0.7 degrees C, respiratory rate was 22 +/- 5/mn, PaO2 was 74 +/- 10 mmHg, chest X-ray showed bilateral opacity in 16%, unilateral in 84% and pleural fluid level in 6 cases. Etiological diagnosis was determined in 70% of cases. Streptococcus pneumoniae (n = 14), Haemophilus influenzae (n = 5), Legionella pneumophila (n = 2), Mycoplasma pneumoniae (n = 2) and Chlamydia psittaci (n = 1) were the most frequent isolated pathogens. 40 patients (91%) were cured with NSG and delay to obtain apyrexia was 4.4 +/- 3.9 days. NSG was stopped in 4 patients: 1 clinical and bacteriological failure (Klebsiella pneumoniae), 2 clinical failures (1 pneumococcus with purulent pleurisy, 1 pneumococcus with worsening of respiratory status), 1 patient with resistant H. influenzae strain in spite of favourable clinical evolution. NSG was well tolerated in 86% of patients. these data invite to carry on evaluation of first line therapy of CAP with NSG."}, {"id": "pubmed23n0710_18081", "title": "[Pneumonia caused by Fusobacterium necrophorum: is Lemierre syndrome still current?].", "score": 0.009708737864077669, "content": "Fusobacterium necrophorum is a non-spore-forming gram-negative anaerobic bacillus that may be the causative agent of localized or severe systemic infections. Systemic infections due to F.necrophorum are known as Lemierre's syndrome, postanginal sepsis or necrobacillosis. The most common clinical course of severe infections in humans is a progressive illness from tonsillitis to septicemia in previously healthy young adults. A septic thrombophlebitis arising from the tonsillar veins and extending into the internal jugular vein leads to septicemia and septic emboli contributing to the development of necrotic abscesses especially in lungs and other tissues such as liver, bone and joints. In this case report, a previously healthy man with pneumonia and empyema due to F.necrophorum has been presented. A 22 year-old man suffering from sore throat for seven days was admitted to emergency department with ongoing fever and dysphagia for three days. On admission he was already taking amoxicillin-clavulanic acid and his complaints were relieved with continuation of therapy to a total of 10 days. However, five days after the cessation of treatment he developed productive cough, fever and generalized myalgia. On physical examination, there were crackles on right lower lung, and chest X-ray revealed pulmonary consolidation on the right middle lobe. Levofloxacin therapy was started based on the diagnosis of pneumonia. While polymorphonuclear leucocytes and intracellular gram-negative bacilli were seen in Gram stained sputum smear, sputum culture was reported as normal flora. Although the patient's status had started to improve with treatment, his condition deteriorated with development of fever and dyspnea. Chest X-ray revealed consolidation, pulmonary infiltrates, pleural effusion and air-fluid level on the right. Meropenem, clarithromycin and linezolid were initiated and a chest tube was inserted with the preliminary diagnosis of necrotizing pneumonia, empyema and type-1 respiratory failure. While there was no growth on bronchoalveolar lavage fluid culture, thoracentesis material inoculated into thioglycolate broth revealed turbidity. Further inoculation onto Schaedler agar which was incubated under anaerobic conditions, yielded growth of catalase negative, indol positive, gram-negative anaerobic bacilli identified as F.necrophorum by BBL Crystal system (Becton Dickinson, USA). The detailed history of the patient revealed that fish bone had stuck in his throat a week ago. Clarithromycin and linezolid were discontinued and he was recovered within six weeks of meropenem treatment. F.necrophorum infection should be considered in the differential diagnosis of persistent head and neck infections with rapidly progressive metastatic necrotic lesions especially in healthy young adults and clindamycin or metranidazol should be added to the treatment protocols."}, {"id": "pubmed23n0126_7531", "title": "[Retrospective study of 77 cases of purulent pleurisy].", "score": 0.009615384615384616, "content": "77 cases of non-tuberculous empyema were studied retrospectively. The data of past history, laboratory, radiological, bacteriological and outcome were gathered. The mean delay between initial symptoms and clinical diagnosis was 2.6 weeks in this study. It was longer for those anaerobic features (p less than 0.02) whose details were unravelled. \"Blind\" antibiotic therapy before the first pleural aspirate is still common (46.7% of cases) and did not alter the frequency with which the causal bacteria was found, nor the distribution of the bacterial population. Pleural aspirate enabled the germ or germs responsible to be identified in 63% of cases. Streptococcus pneumoniae (n = 15) and anaerobic bacterias (n = 26) infections were predominant. Early pleural drainage in a trained unit care is the essential element in the treatment in association with appropriate antibiotics and prolonged physiotherapy. This triple therapy approach leads to a medical cure in practically all the patients and avoids a recourse to surgery."}, {"id": "wiki20220301en587_22067", "title": "Selective norepinephrine reuptake inhibitor", "score": 0.009523809523809525, "content": "In the use of atomoxetine in children (6 years or older up to 70 kg) with attention-deficit hyperactivity disorder, acute treatment should be started with approximately 0.5 mg/kg orally daily. The dose should be increased after a minimum of 3 days up to approximately 1.2 mg/kg daily (target dose) as a single or two divided doses (in the morning and late afternoon). For children older than 6 years old, over 70 kg, acute treatment should be started with 40 mg/day orally and increased up to 80 mg/day after a minimum of 3 days. The dose can be taken as a single dose in the morning or in two divided doses (in the morning and late afternoon). After 2–4 weeks the dose can be increased to 100 mg/ daily."}, {"id": "pubmed23n0291_23567", "title": "[Clinical course and treatment of pleural empyema in children].", "score": 0.009523809523809525, "content": "Purulent pleurisy has become rare. It is often masked by previous antibiotic treatment so that functional prognosis may be poor. Twenty children with purulent pleurisy of the large cavity admitted from 1987 to 1993 were included in the study: there were nine infants (age 5 to 18 months) with pleuro-pulmonary staphylococcal infection (group I) and 11 children (4-13 years) (group II). Clinical, biological, bacteriological and radiologic findings were analysed retrospectively as was the outcome. Patients of group I were admitted in poor general condition. X-ray showed moderate effusion and characteristic signs of staphylococcal infection. The bacteria identified in seven patients (77%) was S aureus. Recovery was rapid with antibiotics and simple local treatment. X-rays were normal two months after hospital discharge in seven patients (77%). One infant presented cicatricial bullous emphysema which required segmental resection. Patients of group II were admitted for moderate respiratory signs after a relatively long delay (14 days) since the onset of symptoms. X-rays showed considerable effusion in all and mediastinal shift in five patients (45%). Streptococcus pneumoniae was identified in one patient only. Local treatment of empyema was difficult; the effusion, already fibrinous, required repeated use of chest tubes in eight cases and surgical decortication in three. X-rays, performed 2 months after hospital discharge, were normal in only three patients. Long-term course was nevertheless favorable since chest X-rays at 5 months were normal in all children of both groups. Early recognition of purulent pleurisy is important in children aged over 3 years to ensure effective drainage before the effusion becomes fibrinous. All patients in whom the first tube was inserted after more than 10 days had a difficult follow-up requiring repeated chest drainages or surgery. Ultrasonography was a useful aid for diagnosis and local treatment. Computed tomography was useful for adapting treatment after several days of course."}, {"id": "wiki20220301en089_23285", "title": "Vesicoureteral reflux", "score": 0.009433962264150943, "content": "Medical treatment Medical treatment entails low dose antibiotic prophylaxis until resolution of VUR occurs. Antibiotics are administered nightly at half the normal therapeutic dose. The specific antibiotics used differ with the age of the patient and include: Amoxicillin or ampicillin – infants younger than 6 weeks Trimethoprim-sulfamethoxazole (co-trimoxazole) – 6 weeks to 2 months After 2 months the following antibiotics are suitable: Nitrofurantoin {5–7 mg/kg/24hrs} Nalidixic acid Bactrim Trimethoprim Cephalosporins Urine cultures are performed 3 monthly to exclude breakthrough infection. Annual radiological investigations are likewise indicated. Good perineal hygiene, and timed and double voiding are also important aspects of medical treatment. Bladder dysfunction is treated with the administration of anticholinergics."}, {"id": "pubmed23n0018_7413", "title": "[Current persistance of pleural empyema. Study of 31 observations (author's transl)].", "score": 0.009433962264150943, "content": "Antibiotic treatment of pleuro-pulmonary infectious diseases with ordinary germs brought a notable decrease in the frequency of pleural empyema without preventing them completely. The authors report 31 cases observed over the last 10 years. The initial signs corresponded to the classical picture but were rapidly masked by antibiotic treatment; it resulted in delay in hospital admission of over one month for more than half of the cases, for diagnosis was overlooked for a long time. In 19 patients, the pleural liquid was aseptic probably because of the antibiotics and of the lack of identification of anaerobic germs. The authors insist on the practical modalities of a medical treatment by puncture-lavage which, when associated with general antibiotic treatment, cured patients in 22 cases. On the other hand, the existence of bronchopleural fistula entailed surgery."}, {"id": "wiki20220301en068_66243", "title": "Cladribine", "score": 0.009345794392523364, "content": "Two main approaches to multiple sclerosis treatment maintenance therapy are used – immunomodulation and immunosuppression and alternatively, immune reconstitution therapy. Classified as the latter, cladribine tablets are administered intermittently as a short treatment course without continuous immunosuppression. In contrast to maintenance therapies, clinical efficacy extends beyond the dosing period. Cladribine tablets are administered as 2 courses separated by 1 year (a maximum of 20 days of treatment). The recommended cumulative dose is 3.5 mg/kg weight over 2 years, administered as 1 treatment course of 1.75 mg/kg per year. Each treatment course consists of 2 treatment weeks, one at the beginning of the first month and one at the beginning of the second month of the respective treatment year. Each treatment week consists of 4 or 5 days on which a patient receives 10 mg or 20 mg (1 or 2 tablets) as a single daily dose based on body weight."}, {"id": "pubmed23n0473_189", "title": "[Pleuropulmonary staphylococcal infections in the infant].", "score": 0.009345794392523364, "content": "In a study of 44 infants and young children with staphylococcal pneumonia and pleurisy, cases were found to be divisible into three clinical groups: those with predominantly digestive tract symptomatology (seven); those in acute respiratory distress (28); and those with signs of central nervous system disorder (nine). Characteristic radiographic findings often permitted an early etiologic diagnosis, confirmed later by bacteriologic culture or by the course of the disease. The length of hospitalization was found to be directly proportional to the length of time between the onset of symptoms and the start of adequate therapy. Nineteen patients required thoracotomy, two were drained by a Küss trocar, 13 required repeated pleural aspiration, five underwent spontaneous resorption of the empyema and five had no pleural effusion. In several instances antibiotics were used in very high dosage (three to four times the suggested maximal dose). The main agents used were erythromycin, chloramphenicol and ristocetin; the dosage of the last named never exceeded the maximal recommended dose. Twenty-eight patients were less than one year old; of these seven died. Sixteen were over one year of age, the oldest being six years; all of these were cured."}, {"id": "pubmed23n0977_7478", "title": "A severe case of Streptococcal pyogenes empyema following influenza A infection.", "score": 0.009259259259259259, "content": "Any immunological mechanisms induced by influenza virus could cause severe secondary bacterial superinfection such as those by Streptococcus pyogenes [group A streptococcus (GAS)], Streptococcus pneumoniae or Staphylococcus aureus. Over recent years, the frequency of pleural empyema has increased in children with influenza infection. We present a severe case of acute empyema caused by S.pyogenes after influenza A infection. A previously healthy 39-year old woman was diagnosed as influenza A and received oral Oseltamivir 75 mg twice daily for 5 days. She had no vaccination of influenza A. Although her influenza A infection improved, she complained of fever and cough to our institute. Chest radiography showed encapsulated pleural effusion of the left lung and pleural effusion which was consistent with acute empyema. Then, she was diagnosed as having acute empyema and was admitted to our institute. Streptococcus pyogenes was identified by pleural fluid culture on day 4. thus, MNZ was changed to clindamycin (CLDM) 600 mg three times a day. While thoracic drainage with intrapleural urokinase and combination antibiotic therapy of ceftriaxone and CLDM were performed, her general condition and chest radiographic findings were not improved. She received video-assisted thoracic debridement on day 10. After the operation, the antibiotic therapy was changed to ABPC 6 g daily iv. Due to good clinical course, the antibiotic therapy was switched to oral amoxicillin 500 mg three times daily on day 28. Then, she was discharged. Influenza A virus infection could lead to severe GAS infection, while the latter can occur in otherwise healthy individual as well. Physician must consider the possibility of severe GAS infection after influenza A infection."}, {"id": "pubmed23n0627_18339", "title": "Thoracic empyema - a review based on three cases reports.", "score": 0.009259259259259259, "content": "Complicated parapneumonic effusion is one in which an invasive procedure is necessary for its resolution and empyema means pus in the pleural space. An early diagnosis and therapy of these conditions results in less morbidity and mortality. CT of the chest is important to study complex pleural effusions. Loculated effusions, those occupying more than 50% of the thorax, or which show positive Gram stain or bacterial culture, or a purulent effusion with a pH below 7.20, with a glucose level below 60 mg/dl or a LDH level more than three times the upper limit of normal for serum, are indications for an invasive procedure. These characteristics result from the evolution of a not well treated parapneumonic effusion, through the three stages: (1) exsudative; (2) fibrinopurulent; (3) fibrotic. Depending on the stage therapeutic methods vary from therapeutic thoracentesis, insertion of a chest tube with or without instillation of fibrinolytics, video-assisted thoracoscopic surgery, and lung decortication. A review of all these aspects are done based on a series of three cases reports with very different clinical presentation: one patient with empyema by Streptococcus pyogenes and that died rapidly due to massive hemoptysis; a patient with empyema due to acute pneumonia developing during an airflight; a patient with empyema and bacteraemia by Streptococcus pneumonia leading to the diagnosis of an unknown HIV infection."}, {"id": "wiki20220301en017_7716", "title": "Chancroid", "score": 0.009174311926605505, "content": "Antibiotics Macrolides are often used to treat chancroid. The CDC recommendation is either a single oral dose (1 gram) of azithromycin, a single IM dose (250 mg) of ceftriaxone, oral (500 mg) of erythromycin three times a day for seven days, or oral (500 mg) of ciprofloxacin twice a day for three days. Due to a paucity of reliable empirical evidence it is not clear whether macrolides are actually more effective and/or better tolerated than other antibiotics when treating chancroid. Data is limited, but there have been reports of ciprofloxacin and erythromycin resistance. Aminoglycosides such as gentamicin, streptomycin, and kanamycin has been used to successfully treat chancroid; however aminoglycoside-resistant strain of H. ducreyi have been observed in both laboratory and clinical settings.[7] Treatment with aminoglycosides should be considered as only a supplement to a primary treatment."}, {"id": "pubmed23n0360_4843", "title": "[Pneumonia caused by Haemophilus influenzae. Study in a series of 58 patients].", "score": 0.009174311926605505, "content": "Haemophilus influenzae tends to form part of the usual respiratory flora in adults, especially if they have a chronic underlying disease or are smokers. Pneumonia due to H. influenzae is frequently involved in respiratory infections and its level of resistance to ampicillin has remained stable over the last five years. Most of the literature on the subject was published more than 10 years ago. In this study, we describe the clinical features and evolution of 58 adult patients admitted to hospital for pneumonia due to H. influenzae over a 2-year period, with this group accounting for 6.5% of all the patients admitted with pneumonia during this time period. The etiological diagnosis was made using a good quality sputum sample. Forty patients (69%) were male. The mean age (+/- SD) of the group was 67 (+/-16.8) years and all the patients had at least one underlying disease. The mean duration of the symptoms was 6.7 days. All patients presented an increase in the quantity or purulence of the sputum. On admittance, respiratory failure was present in 52 patients (90%). Gram-negative coccus-bacilli were observed in the direct sputum test and H. influenzae grew in the culture. In two cases, H. influenzae was recovered from the blood culture and in one from bronchial aspiration obtained through bronchoscopy. Another pathogen was identified in 28 patients (48%). In 21 it was another pyogenic bacteria (15 S. pneumoniae, 4 M. catharralis, 1 K. pneumoniae, 1 E. coli), an atypical microorganism in 5 (3 C. pneumoniae, 2 C. burnetii) and a respiratory virus in 2 (syncytial and influenza A). Atypical bacteria and respiratory virus were detected using serological techniques. The radiographic infiltrate was unilobar in 54 of the 58 patients and all showed an alveolar pattern. The empirical treatment included the administration of a third generation cephalosporin (or a fluoroquinolone in patients allergic to penicillin). The evolution was favorable in all the cases in which H. influenzae was the only pathogen or was accompanied by an atypical microorganism or a respiratory virus. Four patients with mixed bacterial pneumonia died (2 S. pneumoniae, 1 E. coli and 1 M. catharralis). The study indicates that pneumoniae due to H. influenzae affects a population with an underlying disease, preferably pulmonary, that it has a longer clinical period than that for pneumococcal pneumonia, that it is slightly bacteremic and, that, usually, it evolves benignly with a low mortality."}, {"id": "wiki20220301en045_78672", "title": "Aspiration pneumonia", "score": 0.009126984126984126, "content": "Treatment The main treatment of aspiration pneumonia revolves around the use of antibiotics to remove the bacteria causing the infection. Broad antibiotic coverage is required to account for the diverse types of bacteria possibly causing the infection. Currently recommended antibiotics include clindamycin, meropenem, ertapenem, ampicillin/sulbactam, and moxifloxacin. Treatment with piperacillin/tazobactam, cefepime, levofloxacin, imipenem, or meropenem is recommended in cases of potential antibiotic resistance. The typical duration of antibiotic therapy is about 5 to 7 days. If there is a large accumulation of fluid within the lungs, drainage of the fluid may also aid in the healing process."}, {"id": "pubmed23n1030_17973", "title": "Pleural effusion in an immunocompetent host with cryptococcal pneumonia: A case report.", "score": 0.00909090909090909, "content": "Pulmonary cryptococcosis is an opportunistic infection that mainly occurs among immunocompromised patients although it can sometimes occur in immunocompetent individuals. However, the imaging findings of pulmonary cryptococcosis in immunocompetent hosts differ from those in immunosuppressed patients. In addition, the most common imaging findings of isolated pulmonary cryptococcosis are single or multiple nodules. Cavities and the halo sign are, however, prevalent in immunosuppressed patients. In immunocompetent patients, lung consolidation, pleural effusion or cavities are scarce. A 29-year-old Asian male was admitted to our hospital with complaints of cough and fever that had persisted for a month. As a chest computed tomography scan showed consolidation in his left lower lobe, he was initially diagnosed with pneumonia and received antibiotic treatment. A second review of the chest computed tomography image revealed multiple cavities and pleural effusion. Flexible fiberoptic bronchoscopy was subsequently performed, bronchoalveolar lavage fluid and serum cryptococcal antigen tests were positive. Cryptococcus capsules were observed in bronchoalveolar lavage fluid ink stain. Histopathological examination of a percutaneous lung biopsy from the left lower lobe further revealed granulomatous inflammation, and periodic acid-Schiff staining showed red-colored yeast walls, signifying pulmonary cryptococcosis. The patient was then treated with a daily dose of fluconazole (0.4 g), but the cough and fever still persisted. We therefore changed treatment to voriconazole (0.2 g, twice a day), and the patient's clinical outcome was satisfactory. Although rare, clinicians should not disregard the possibility of cavities and pleural effusion occurring in immunocompetent hosts without underlying diseases."}, {"id": "pubmed23n0659_19555", "title": "Are there any differences in the community acquired pneumonias admitted to hospital over the past decade?", "score": 0.00909090909090909, "content": "The past few years have seen a decline in community acquired pneumonia (CAP) in children in the western world, although this has gone hand-in-hand with more serious cases needing hospital admission. Our study characterises cases of CAP admitted to hospital and compares this data with a 2001 study. We collected data on 63 admissions over a six-month period. The majority were aged 0-2 years old. Chest X-ray showed consolidation/atelectasy in 58 (92.1%) and pleural effusion (PE) in 17 (27.0%), of which 11 were empyema (17.4% of all admissions). The bacterial agent was isolated in five cases: Streptococcus pyogenes (two, pleural fluid), Streptococcus pneumoniae (two, blood culture) and Haemophilus influenzae (one, blood culture). Sixty-one children (96.8%) were prescribed antibiotherapy. The median length of hospital stay was five days. Patients with PE were older, had a longer course of fever, higher inflammatory parameters, longer hospital stay and longer course of iv antibiotics. Compared to the prior study we found greater severity of CAP, with higher prevalence of PE and empyema. Nevertheless there was a shorter course of fever during hospital stay and shorter hospital stay. We also noticed less antibiotic prescription prior to admission and greater prescription of ampicillin during hospital stay. In the literature, the higher severity of CAP has been partially attributed to the emergence of more aggressive serotypes of Stretococcus pneumoniae not included in the heptavalent vaccine. There is therefore a greater interest in new vaccines containing them. Complicated CAP should be referred to centres specialising in its diagnosis and management."}, {"id": "pubmed23n0324_18178", "title": "Invasive diagnostic techniques for pneumonia: protected specimen brush, bronchoalveolar lavage, and lung biopsy methods.", "score": 0.009009009009009009, "content": "We suggest the following strategy for managing patients with pneumonia. For nonventilated patients with either CAP or HAP, empiric antibiotic treatment should be started according to approved guidelines, and if the clinical evolution of the patient is not adequate, fiberoptic bronchoscopy including PSB and BAL could be considered, with modification of the antibiotic treatment accordingly. In ventilated patients with either CAP or HAP, respiratory secretion sampling using noninvasive techniques should be conducted upon clinical suspicion of VAP and before starting a new antibiotic treatment. Antibiotic therapy according to approved guidelines should be started as soon as possible and maintained during the first 48 hours if the patient's evolution is satisfactory and condition has stabilized. Then, initial antibiotic treatment should be adjusted according to cultures. If there is a clear diagnostic alternative to VAP and cultures are negative, this is the only case in which antibiotic treatment could be withdrawn. If the patient's clinical evolution is inadequate (persistence of fever, leukocytosis, increasing infiltrates, and respiratory failure), fiberoptic bronchoscopy with PSB and BAL and modification of the initial antibiotic regimen should be sought. Open lung biopsy may be indicated in patients with diffuse pulmonary infiltrates in whom a diagnosis has not been achieved by other methods, including bronchoscopy. Transbronchial lung biopsy should not be viewed as a diagnostic technique for pneumonia except in immunosuppressed patients with diffuse alveolar infiltrates."}, {"id": "pubmed23n0360_22946", "title": "[Pneumonia due to Haemophilus influenzae.Study in a series of 58 patients]", "score": 0.009009009009009009, "content": "Haemophilus influenzae tends to form part of the usual respiratory flora in adults, especially if they have a chronic underlying disease or are smokers. Pneumonia due to H. influenzae is frequently involved in respiratory infections and its level of resistance to ampicillin has remained stable over the last five years. Most of the literature on the subject was published more than 10 years ago. In this study, we describe the clinical features and evolution of 58 adult patients admitted to hospital for pneumonia due to H. influenzae over a 2-year period, with this group accounting for 6.5&amp;#37; of all the patients admitted with pneumonia during this time period. The etiological diagnosis was made using a good quality sputum sample. Forty patients (69&amp;#37;) were male. The mean age (+/- SD) of the group was 67 (+/-16.8) years and all the patients had at least one underlying disease. The mean duration of the symptoms was 6.7 days. All patients presented an increase in the quantity or purulence of the sputum. On admittance, respiratory failure was present in 52 patients (90&amp;#37;). Gram-negative coccus-bacilli were observed in the direct sputum test and H. influenzae grew in the culture. In two cases, H. influenzae was recovered from the blood culture and in one from bronchial aspiration obtained through bronchoscopy. Another pathogen was identified in 28 patients (48&amp;#37;). In 21 it was another pyogenic bacteria (15 S. pneumoniae, 4 M. catharralis, 1 K. pneumoniae, 1 E. coli), an atypical microorganism in 5 (3 C. pneumoniae, 2 C. burnetii) and a respiratory virus in 2 (syncytial and influenza A). Atypical bacteria and respiratory virus were detected using serological techniques. The radiographic infiltrate was unilobar in 54 of the 58 patients and all showed an alveolar pattern. The empirical treatment included the administration of a third generation cephalosporin (or a fluoroquinolone in patients allergic to penicillin). The evolution was favorable in all the cases in which H. influenzae was the only pathogen or was accompanied by an atypical microorganism or a respiratory virus. Four patients with mixed bacterial pneumonia died (2 S. pneumoniae, 1 E. coli and 1 M. catharralis). The study indicates that pneumoniae due to H. influenzae affects a population with an underlying disease, preferably pulmonary, that it has a longer clinical period than that for pneumococcal pneumonia, that it is slightly bacteremic and, that, usually, it evolves benignly with a low mortality."}, {"id": "wiki20220301en568_3770", "title": "Pharmacology of cyproterone acetate", "score": 0.008928571428571428, "content": "The effective dosage of CPA for inhibition of ovulation in women, this being an antigonadotropic effect, is 1 mg/day. CPA alone has been found to suppress ovulation in 3 of 5 women at a dose of 0.5 mg/day and in 5 of 5 women at a dose of 1 mg/day in studies. Ovulation inhibition with 1–2 mg/day CPA in combination with 1–2 mg/day estradiol valerate as a birth control pill (brand name Femilar) occurred in 94.4% of 108 women during the third treatment cycle in one study and in almost 100% of 26 women over 12 treatment cycles in another study (except for one woman who ovulated during her first treatment cycle)."}, {"id": "pubmed23n0217_9614", "title": "[Comparative course of pneumonia with suppuration].", "score": 0.008928571428571428, "content": "Certain clinical, biochemical, bacteriologic and radiographic parameters are compared, as well as the evolution and treatment of two groups of purulent pneumonias followed during the two trienniums 1968-1970 and 1980-1982. In the more recent triennium stands on the decrease in the incidence of this type of pneumoniae as well as a shorter hospital admittance and a lesser need for surgical therapy."}, {"id": "wiki20220301en103_34584", "title": "Cavernous sinus thrombosis", "score": 0.008849557522123894, "content": "Differential diagnosis Orbital cellulitis Internal carotid artery aneurysm Stroke Migraine headache Allergic blepharitis Thyroid exophthalmos Brain tumor Meningitis Mucormycosis Trauma Treatment Recognizing the primary source of infection (i.e., facial cellulitis, middle ear, and sinus infections) and treating the primary source expeditiously is the best way to prevent cavernous sinus thrombosis. Antibiotics Broad-spectrum intravenous antibiotics are used until a definite pathogen is found. Nafcillin 1.5 g IV q4h Cefotaxime 1.5 to 2 g IV q4h Metronidazole 15 mg/kg load followed by 7.5 mg/kg IV q6h Vancomycin may be substituted for nafcillin if significant concern exists for infection by methicillin-resistant Staphylococcus aureus or resistant Streptococcus pneumoniae. Appropriate therapy should take into account the primary source of infection as well as possible associated complications such as brain abscess, meningitis, or subdural empyema."}, {"id": "pubmed23n0416_6104", "title": "[The influence of the initial antibacterial treatment effectiveness, clinical and etiological factors on non-resolvent severe pneumonia and outcome].", "score": 0.008849557522123894, "content": "To evaluate the influence of the initial pneumonia treatment effectiveness, clinical and etiological factors, local innate immune response intensity on pneumonia non-resolution and mortality. One hundred one bronchoscopy and bronchoalveolar lavage performed to 68 severe community (CAP) or hospital-acquired pneumonia patients survived during initial 5-7-day treatment period. Initial treatment effectiveness, age, pneumonia type, presence of mechanical ventilation, comorbidities, coma, antibacterial treatment, pathogen isolation and treatment changes were assessed. Logistic regression analysis was performed to detect factors associated with non-resolving pneumonia and mortality. Etiotropical treatment was administered to 30.9% of patients. Initial antibacterial treatment was corrected in 64.7% of all cases. After 21-30 days from the pneumonia onset survived 66.2% of patients (n=43). Pneumonia course could be evaluated in 52 cases. Delayed resolution of the pneumonia was stated in 32.7% of cases (n=17). Pneumonia was cured or condition improved in 64.3% of the CAP patients (n=9) and 68.4% of the hospital-acquired pneumonia patients (n=26). Only ineffective initial pneumonia treatment significantly increased probability of non-resolving pneumonia on multivariate analysis (OR 16.92, 95% CI 2.02-141.72, p&lt;0.05). Influence of etiotropic treatment was not significant. Isolation of two or more pathogens from bronchoalveolar lavage fluid after the initial treatment was significantly associated with mortality on multivariate analysis (OR 6.25, CI 1.06-36.74, p&lt;0.05). The other analyzed variables had no significant influence on pneumonia resolution and outcome. In conclusion, the initial pneumonia treatment failure increases probability of the non-resolving pneumonia. Etiotropical treatment has no impact on pneumonia outcome when adequate empirical antibacterial treatment is administered, however, it allows to reduce unnecessary use of the broad-spectrum antibacterials. Mortality is associated with the presence of the polymicrobial infection after the initial pneumonia treatment."}, {"id": "wiki20220301en292_619", "title": "Aggressive periodontitis", "score": 0.008771929824561403, "content": "Scale and Polish Root Surface Debridement (RSD) Antibiotics: There is evidence that the additional use of systemic antibiotics in conjunction with non-surgical periodontal treatment results in a more favourable clinical response, as compared to just periodontal treatment alone, as it helps to suppress pathogenic bacteria and create a health-associated biofilm. There have been many antibiotic regimes proposed for the treatment of AgP. However, the combination of choice according to current research is a combination of amoxicillin (500 mg, thrice/day) and metronidazole (200 mg, thrice/day), for 7 days, starting on the day of the final debridement. Doxycycline (100 mg, once/day, with a starting first dose of 200 mg) is the choice of antibiotics for patients allergic to penicillin. Light Amplification by Stimulated Emission of Radiation (LASER) Therapy"}, {"id": "pubmed23n0402_13469", "title": "[Jacopo's chest (bad history of a pleural empyema). A case report].", "score": 0.008771929824561403, "content": "We present a case of pleural empyema, occurred in a healty 7 years boy. He was admitted to our hospital because of a lobare pneumonitis. The patient was administered with a 2 degrees generation Cefalosporine given intramuscularly and with Corticosteroid (1 mg/kg/die). After an initial improvement of his clinical conditions, he got worse so that he underwent a TC scan which showed the presence of a left pleural empyema requiring the insertion of an intercostal tube drainage followed by an intervention of decortication. The boy had some evidence of a staphylococcal etiology such as the evolution in empyema itself, the augmentation of antistafilolisinic title found during the illness, and the typical finding of blebs on chest radiograph. As cultures from both blood and drainage liquid samples remained sterile, we were unable to demonstrate a clear bacterial etiology of the empyema. It remains doubtful if corticosteroid administration could contribute to the severity of the pneumonia evolution."}, {"id": "Surgery_Schwartz_4945", "title": "Surgery_Schwartz", "score": 0.008761195353374125, "content": "can be avoided in patients with small effusions associated with resolving pneumonia. These patients typically present with cough, fever, leukocytosis, and uni-lateral infiltrate, and the effusion is usually a result of a reactive, parapneumonic process. If the effusion is small and the patient responds to antibiotics, a diagnostic thoracentesis may be unneces-sary. If the effusion is large and compromising respiratory efforts, or if the patient has a persistent white blood cell count despite improving signs of pneumonia, an empyema of the pleural space must be considered. In these patients, early and aggressive drainage with chest tubes is required, possibly with surgical intervention.Once the decision is made to access a pleural effusion, the next step is to determine if a sample of the fluid or complete drainage of the pleural space is desired. This step is influenced by the clinical history, the type and amount of fluid present, the Brunicardi_Ch19_p0661-p0750.indd 73601/03/19"}]}}}}
{"correct_option": 5, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[425, 491]], "word_ranges": [[65, 75]], "text": "Lennox Gastaut develops at an early age with other symptomatology,"}, "3": {"exist": true, "char_ranges": [[523, 571]], "word_ranges": [[82, 89]], "text": "3 usually presents with complex partial seizures"}, "4": {"exist": true, "char_ranges": [[492, 518]], "word_ranges": [[75, 81]], "text": "4 is the same as absences,"}, "5": {"exist": true, "char_ranges": [[0, 156]], "word_ranges": [[0, 24]], "text": "A patient of adolescent age with myoclonus (\"violent jumps\") at breakfast time will almost always lead us to juvenile myoclonic epilepsy (answer 5 correct),"}}, "full_answer": "A patient of adolescent age with myoclonus (\"violent jumps\") at breakfast time will almost always lead us to juvenile myoclonic epilepsy (answer 5 correct), a very well characterized entity. Among other features is the previous history of seizures or absences, the worsening with nighttime departures, and the EEG with generalized polyp spike discharges at a higher frequency than that of absences. Of the remaining answers, Lennox Gastaut develops at an early age with other symptomatology, 4 is the same as absences, and 3 usually presents with complex partial seizures and either way, the EEG is clearly distinct from a temporal focus.", "full_answer_no_ref": "A patient of adolescent age with myoclonus (\"violent jumps\") at breakfast time will almost always lead us to juvenile myoclonic epilepsy ([HIDDEN]), a very well characterized entity. Among other features is the previous history of seizures or absences, the worsening with nighttime departures, and the EEG with generalized polyp spike discharges at a higher frequency than that of absences. Of the remaining answers, Lennox Gastaut develops at an early age with other symptomatology, 4 is the same as absences, and 3 usually presents with complex partial seizures and either way, the EEG is clearly distinct from a temporal focus.", "full_question": "An 18-year-old female patient with a history of absences between 6 and 9 years of age, generalized tonic-clonic seizures of recent onset and violent jumping of the upper limbs at breakfast. The clinical manifestations worsen with nighttime weekend outings. An EEG shows acute polyp spike discharges at 6 cycles/second. The most likely diagnosis is:", "id": 130, "lang": "en", "options": {"1": "Great epileptic disease.", "2": "Lennox-Gastaut syndrome.", "3": "Symptomatic epilepsy due to mesial temporal sclerosis.", "4": "Small atypical disease.", "5": "Juvenile myoclonic epilepsy."}, "question_id_specific": 82, "type": "NEUROLOGY AND NEUROSURGERY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0648_13197", "title": "[Clinical and electroencephalographic characteristics of epilepsy with myoclonic absences].", "score": 0.017475875693336373, "content": "Epilepsy with myoclonic absences (EMA) is a type of childhood epilepsy characterized by a specific seizure type, i.e. myoclonic absences (MA). This study aimed to investigate the clinical and electrophysiological characteristics of EMA. Video-EEG monitoring was carried out in 6 patients with EMA, and 2 of them were examined with simultaneous deltoid muscle surface electromyogram (EMG). The clinical and EEG characteristics, treatment and prognoses of EMA were analyzed. Of the 6 patients, 3 were female, and 3 were male. The age of onset was from 2 years and 3 months to 11 years (average 5 years and 2 months). MA was the sole seizure type in 5 patients. One patient presented generalized tonic clonic seizures (GTCS) at the onset and then switched to MA. The manifestations of MA included an impairment of consciousness of variable intensity, rhythmic myoclonic jerks with evident tonic contraction mainly involving the upper extremities, a deviation of head and body to one side or asymmetrical jerks observed in some cases, a duration ranging from 2 to 30 s, an abrupt onset and termination, a high frequency of attacks, at least several times to over 30 times per day, and easily provoked by hyperventilation. The ictal EEG consisted of rhythmic 3 Hz spike and wave discharges that were bilateral, synchronous and symmetrical in all patients. The deltoid muscle EMG recording in 2 patients showed rhythmic myoclonus at the same frequency as the spike and waves. The interictal EEG showed generalized spike and wave discharges in all patients, and focal discharges in some patients. Valproate was the drug of choice, which was often combined with other antiepileptic drugs. The ages at follow up ranged from 6 years and 4 months to 19 years. Seizures were controlled from 8 months to 3 years in 4 cases. The treatment at the onset was late in one case and was irregular in another who had GTCS during the course of the disease. These two cases were followed up for 2 years and 6 months and 5 years, respectively. Seizures could not be controlled in the 2 patients with intellectual impairment. EMA was a rare type of childhood epilepsy characterized by MA. Clinical observation and ictal video-EEG and EMG were essential to diagnose EMA. Valproate alone or combined with other antiepileptic drugs given early could have a favorable effect to EMA. Delayed therapy and the presence of GTCS might suggest poor prognosis."}, {"id": "pubmed23n0327_12491", "title": "[Juvenile myoclonic epilepsy].", "score": 0.016569667921944538, "content": "We conclude that despite inevitable variability the clinical picture of JME is characteristic. It is easy to diagnose JME if one thinks of it while the history should be thoroughly analyzed. An EEG recording during sleep confirms the diagnosis. An early diagnosis of JME permits adequate prognosis of the subsequent course of epilepsy, and adequate therapy brings remission in most of the patients. If treatment starts following the large number of severe GTC seizures, the response to therapy is incomplete. The persistency of the illness throughout the life, the need for continuous medication and therapeutic unresponsiveness in cases with late diagnosis, do not justify the increasing misconception that JME is of benign nature. Diagnosis of JME is rare because of insufficient familiarily of physicians with the illness. Juvenile myoclonic epilepsy (JME) is an idiopathic generalized epileptic syndrome characterized with the combination of myoclonic, generalized tonic-clonic (GTC) and absence seizures that are readily provoked by sleep deprivation. Forty-three patients, aged from 14 to 51 years, participated in a 5-year follow-up study. Diagnosis was made according to the criteria (Table 1) for diagnosis of JME set by Panayiotopoulos et al. (1994). Nineteen patients made their first contact with a neurologist at the Institute of Neurology and were diagnosed as JME, while the remaining 24 were referred to from other medical institutions with a diagnosis of therapy resistant to focal epilepsy. All patients underwent a somatic and neurological examination, \"mini mental test,\" EEG in waking and CT scan of the brain. Some patients had EEG performed during sleep and some had MRI of the head. JME began between 9 and 26 (average 17) years. All patients had myoclonic seizures, 98% had GTC and 23% absence seizures. The first myoclonic seizure occurred between 9 and 24 years while the frst GTC seizure occurred between 10 and 32 years. Myoclonic seizures (83% of patients) and GTC seizures (70% of patients) occurred most often immediately after awaking. The most frequent provocative factors were insufficient sleep, alcohol abuse and tiredness. Epilepsy in the family was present in 39%, focal neurological deficiency in 9% and pathological findings on of CT and MRI in 7% of patients. Waking EEG was pathological in 77% of patients; it included generalized spike-wave discharges in 73%, multiple spike-wave complexes in 33% and focal discharges in 12% of patients, respectively. In all 26 patients tested, sleep EEG was pathological most often with multiple spike-wave complexes in 85% and 3-4 Hz spike-wave complexes in 57% of patients. The correct diagnosis of JME following a comprehensive examination was made in 24 (56%) patients after a delay of 1 to 35 years. In 24 patients with delayed diagnosis of JME the replacement of earlier medication with valproic acid (VPA) induced remission in 18 patients (75%) while 1 patient (4%) experienced a reduction in the number of seizures. Five patients (21%) did not respond to VPA medication: 2 due to a weak compliance, another 2 due to inefficient medication and 1 because of the preexistent malabsorption syndrome. In 19 patients (44%) with initial diagnosis of JME, VPA was introduced immediately upon diagnosis. Of them, 15 (79%) had excellent response to VPA, 1 refused therapy and for 3 patients there is no information. In 2 patients VPA was substituted due to side effects (hepatotoxicity and alopetia) with lamotrigine (low doses), which brought about decrease in frequency and mitigation in myoclonic seizures."}, {"id": "pubmed23n0311_15596", "title": "[Differential diagnosis in idiopathic generalized epilepsies with tonic-clonic seizures: assessment of the use of ambulatory EEG and video/EEG monitoring].", "score": 0.016328978938949613, "content": "The value of long-term cassette EEG (24-EEG) and Video/EEG for differential diagnosis and classification of idiopathic epilepsies with generalised tonic-clonic seizures (GTCS) was evaluated in twenty-eight patients. The analysis of clinical and EEG features allowed proper classification of epileptic syndrome in twenty-two (79%) patients. In twelve cases absences or myoclonic seizures appeared beside GTCS after 1-9 years from epilepsy onset (mean 3.3 yrs). EEG and clinical data allowed to classify epilepsy in nine (75%) of those patients: in six patients as juvenile absence epilepsy and in three as juvenile myoclonic epilepsy. Sixteen patients suffered from GTCS only (mean duration of epilepsy 10.6 years); in thirteen of them (81%) the diagnosis of epilepsy with GTCS on awakening (AGM) could be established. 24-EEG and Video/EEG helped to demonstrate (a) interictal generalized spike/polyspike-wave discharges (SW/PSW) 3-6 Hz not present in routine EEG in 25% of patients, (b) typical circadian distribution of discharges in AGM patients and (c) absences and myoclonic seizures in 32% of patients. Slow spike-wave variants and focal changes in EEG which could suggest secondarily generalized GTCS were the main diagnostic problem."}, {"id": "pubmed23n0317_4256", "title": "Clinical observations of juvenile myoclonic epilepsy in 131 patients: a study in South India.", "score": 0.01594022415940224, "content": "We studied clinical features of 131 patients with juvenile myoclonic epilepsy (JME). The prevalence was 7.7% among the epileptic patients registered. The mean age at onset was 13.37+/-4.93 years and the diagnosis was established at a mean age of 19.53+/-7.85 years. Absence seizures were reported by 27 (20.6%) patients, myoclonic jerks by 131 (100%) and generalized tonic-clonic seizures (GTCS) by 111 (84.7%). The triad of absence seizures, myoclonic jerks and GTCS was noted in 23 (17.5%) patients, 88 (67.2%) had myoclonic jerks and GTCS, 4 (3%) had absence seizures and myoclonic jerks and 16 (12.2%) had only myoclonic jerks. Early onset absences were seen in 21 (16%) patients and the onset was late in 6 (4.6%). Absences antedated other types of seizures in all the patients. Myoclonic jerks were predominantly unilateral or had unilateral onset in 22 (16.8%). In 17 (13%) patients GTCS antedated myoclonic jerks. Myoclonic jerks had characteristic circadian distribution in 112 (85.5%) patients. On awakening GTCS occurred in 87 (78.4%) patients and in 4 (3.6%) patients they were purely nocturnal. Sleep deprivation was the most important precipitating factor (54.2%). Initial electroencephalogram (EEG) showed classical generalized spike or multiple-spike slow-wave paroxysms in 81% of records. Focal EEG abnormalities were noted in 20.6% of records. The most common focal abnormality was voltage asymmetry. A family history of epilepsy was noted in 31 (23.6%) probands. Diagnosis of JME was made in all the cases in the clinic. The factors responsible for delay in diagnosis of the 36 patients seen by neurologists included failure to ask or interpret the history which was otherwise suggestive of myoclonic jerks in all 36 (100%) cases, the type of seizure for which the patients sought medical attention, and misinterpretation of EEGs in 28 patients. Diagnosis of partial epilepsy was made in seven patients. The factors responsible for such diagnoses were, unilateral jerks in one patient, unilateral jerks and absence seizures in three patients and focal EEG abnormalities in three patients."}, {"id": "pubmed23n0047_10451", "title": "Benign myoclonic epilepsy of infancy: electroclinical symptomatology and differential diagnosis from the other types of generalized epilepsy of infancy.", "score": 0.015716374269005847, "content": "Benign myoclonic epilepsy in infancy (BME) is characterized by the occurrence of brief myoclonic attacks in normal infants aged 4 months to 3 years. There is no prior personal history, although in some patients 1 or 2 isolated febrile convulsions may occur prior to the onset of myoclonias. A family history of epilepsy or febrile convulsions is present in 30% of cases. Myoclonic attacks are short and mild, they involve mainly the head and upper limbs. The psychomotor development continues normally after the onset of seizures. The EEG shows a normal background activity and generalized spike-wave or polyspike-wave discharges associated with the myoclonias. These abnormalities are activated by drowsiness and during the first stages of sleep. A clinical and EEG photosensitivity is present in one-third of the patients. Myoclonias can be easily controlled by valproate monotherapy. Rare grand mal seizures can occur during adolescence, after withdrawal of drug treatment. The psychomotor evolution is good if treatment is started early. When myoclonias begin during the first year of life, the diagnoses of cryptogenic infantile spasms and of non-epileptic benign infantile myoclonus must be eliminated. In cases with a later onset, the following diagnoses can usually be easily discarded: cryptogenic Lennox-Gastaut syndrome, myoclonic-astatic epilepsy and unclassified epilepsies with the association of myoclonias and other types of seizures."}, {"id": "pubmed23n0482_20456", "title": "[Characteristics of clinical manifestations and EEG of Lennox-Gastaut syndrome].", "score": 0.014797008547008548, "content": "Lennox-Gastaut syndrome (LGS) is one of the most severe and refractory form of childhood epilepsy. The purpose of this study was to investigate the clinical and EEG characteristics of patients with LGS. Sixty-two patients with LGS, including 37 males and 25 females, were followed-up regularly per three months or per six months, therapy was adjusted according to the changes in seizures and EEG, and the clinical data were analyzed in detail. The onset occurred between the age of 8 months and 12 years, with the peak at 1-4 years of age, accounting for 61%; a late onset which occurred after 8 years of age, was unusual. Furthermore, one patient who developed LGS at the age of 13 years and remained to have all the features of seizures and EEG at 35 years of age was identified as adult's LGS. Forty-three patients were classified as symptomatic, perinatal events were the predominant factors in this group. The others were cryptogenic. It was noted that 11 cases had a history of West syndrome. A transformation process from West syndrome to LGS was observed in another 7 cases. Every patient had two or more seizure types during the course of the disease; tonic seizure, atypical absence seizure, head drop or sudden falls were the characteristic types. The degree of mental deficit was variable from slight to profound deterioration, but mental and behavioral disturbances existed in every case as a rule. In all cases electroencephalogram (EEG) background was abnormal and consisted of diffuse slow spike-and-waves (1-1.5CPS), predominant in frontal and temporal regions. Twenty-four cases had the polyspike-wave. Bursts of fast rhythms (10-14CPS) were observed in 29 patients during sleep. The choice of antiepileptic drugs (AEDs) was based on the seizure types; routinely, 2 or more kinds of AEDs were used in combination, the classic drugs, valproate and clonazepam were firstly recommended; the other drugs, such as lamotrigine and topiramate that are used as add-on therapy were further consideration. Although the total effect was not satisfactory, the severity and frequency of seizures in almost all cases had lessened to some extent. LGS shows diverse manifestations; comprehensive diagnosis is crucial, active and efficacious treatment can improve the mental and behavioral development and prognosis as a whole."}, {"id": "pubmed23n0027_12197", "title": "Lennox-Gastaut's syndrome--prognosis of the secondary generalized epilepsies.", "score": 0.014697120158887786, "content": "On 62 cases with Lennox-Gastaut's syndrome aged four to 31, the clinical-electroencephalographic findings were summarized as follows; (1) Age of onset was over 10 years in 10 cases (16.1%). (2) Mental deficiencies were more severe in those with onset earlier than age three. (3) Behavioral problems were observed in 34 cases (54.8%); 21 with hyperactive and 13 with hypoactive ones -- 18 hyperactive cases (85.7%) with the onset taking place before age six, and 12 hypoactive cases (92.3%), all whose age is now over 10. (4) The number of clinical seizures showed a tendency in which monoictal manifestation decreased from 25 to three whereas polyictal one increased from 13 to 59 cases during the course of a decade. (5) Interictal EEG findings were pseudorhythm of slow spike-wave with or without focal spikes, and so-called runs of rapid spikes during sleep recording. Focal spikes were observed in 25 cases (40.3%); mainly in the frontal area among those under 10 years old, and in the anterior temporal area among those over 20. The rapid spikes were demonstrated in 19 cases (30.6%) in the over-15 age group and appeared to be correlated with epileptic drop seizures and atypical complex absences."}, {"id": "pubmed23n0759_10090", "title": "West syndrome followed by juvenile myoclonic epilepsy: a coincidental occurrence?", "score": 0.014506172839506172, "content": "West syndrome is an age-dependent epilepsy with onset peak in the first year of life whose aetiology may be symptomatic or cryptogenic. Long-term cognitive and neurological prognosis is usually poor and seizure outcome is also variable. Over the past two decades a few patients with favourable cognitive outcome and with total recovery from seizures were identified among the cryptogenic group suggesting an idiopathic aetiology. Recent research has described two children with idiopathic WS who later developed a childhood absence epilepsy. We reviewed the medical records of patients with West syndrome admitted to the our Child Neuropsychiatry Unit in the last 15 years in order to know the clinical evolution of infantile spasms.We report a child with West syndrome with onset at 8 months of age followed by some clusters of bilateral, arrhythmic myoclonic jerks of the upper limbs, mainly on awakening, synchronous with the generalized discharges of 4 Hz spike-wave occurring at 12 years of age and by co-occurrence of a later generalized tonic-clonic seizure at 14 years and four months, both sensitive to Levetiracetam suggesting a juvenile myoclonic epilepsy. This unusual evolution, never previously reported, suggests that both electroclinical features mentioned above may share some pathophysiological processes genetically determined which produce a susceptibility to seizure and emphasizes that the transition between different age-related epileptic phenotypes may involve also the West syndrome."}, {"id": "wiki20220301en429_18535", "title": "Epilepsy syndromes", "score": 0.014476818764889134, "content": "Juvenile myoclonic epilepsy Juvenile myoclonic epilepsy (JME) is a genetic generalised epilepsy that occurs in patients aged 8 to 20 years. Patients have normal cognition and are otherwise neurologically intact. The most common seizure is myoclonic jerks, although generalized tonic-clonic seizures and absence seizures may occur as well. Myoclonic jerks usually cluster in the early morning after awakening. The EEG reveals generalized 4–6 Hz spike wave discharges or multiple spike discharges. These patients are often first diagnosed when they have their first generalized tonic-clonic seizure later in life, when they experience sleep deprivation (e.g., freshman year in college after staying up late to study for exams). Alcohol withdrawal can also be a major contributing factor in breakthrough seizures, as well. The risk of the tendency to have seizures is lifelong; however, the majority have well-controlled seizures with anticonvulsant medication and avoidance of seizure precipitants."}, {"id": "wiki20220301en429_18536", "title": "Epilepsy syndromes", "score": 0.014229994428014231, "content": "Lennox-Gastaut syndrome Lennox-Gastaut syndrome (LGS) is a generalized epilepsy that consists of a triad of developmental delay or childhood dementia, mixed generalized seizures, and EEG demonstrating a pattern of approximately 2 Hz \"slow\" spike-waves. Onset occurs between two and 18 years. Epilepsy is consider a chronic (meaning it lasts for a long time) condition that is defined by seizures. Lennox-Gastaut syndrome (LGS) is a rare and severe form of epilepsy. As in West syndrome, LGS result from idiopathic, symptomatic, or cryptogenic causes, and many patients first have West syndrome. Authorities emphasize different seizure types as important in LGS, but most have astatic seizures (drop attacks), tonic seizures, tonic-clonic seizures, atypical absence seizures, and sometimes, focal seizures. Anticonvulsants are usually only partially successful in treatment."}, {"id": "pubmed23n0070_15833", "title": "[\"Benign\" form of myoclonic epilepsy in children].", "score": 0.014129353233830846, "content": "Among 62 children with myoclonic epilepsy who had first seizures between 1 and 10 years, without clinical or radiological evidence of brain lesion, we selected the 16 patients who had exhibited several types of fits and had stopped having seizures for over two years. First seizures occurred between 18 months and 4 years, and they were generalized clonic, tonic-clonic or tonic. After a mean 3 months' period, patients started also to have absence and myoclonic fits. During the period with various types of seizures, that lasted a mean 10 months, patients were ataxic and hyperkinetic, and 11 of them suffered myoclonic absence status for several hours or days. The EEG showed a high voltage rhythmic slow-wave activity with spikes, differing from the slow spike wave tracing of the Lennox-Gastaut syndrome, and there was no photosensitivity. The mean duration of the epilepsy was 1 year and 4 months and the last seizures were convulsive, occurring mainly during sleep. The clinical and EEG pattern, the high familial incidence are shared by the Doose syndrome, of which the present series seems to be a subgroup, as are other well-defined syndromes: benign and severe myoclonic epilepsies of infancy."}, {"id": "pubmed23n0351_3259", "title": "Eye closure related spike and wave discharges: clinical and syndromic associations.", "score": 0.014102564102564103, "content": "Precipitation of spike and wave (SW) discharges in some epileptic patients by eye closure (EC) has rarely been reported. To disclose the clinical characteristics and classification of syndromes of epileptic patients with SW discharges induced by EC, we investigated 10 patients (1 M, 9 F) showing this peculiar EEG feature. The patients aged between 9-39 years (mean 20.6 +/- 9.058), underwent short-term (1-3.5 hr) video-EEG investigations in order to document the appearance of the SW discharges within 3 seconds of the act of EC, in at least two occasions. Clinical analysis showed that 5 female patients who had the syndrome of juvenile myoclonic epilepsy (JME) had a later onset of epilepsy (13-15 years) than the 3 patients (3 girls) with eyelid myoclonia with absences (EMA) (3-8 years of age at onset). The remaining 2 patients who were diagnosed as childhood absence epilepsy (CAE) and juvenile absence epilepsy (JAE) according to the international classification, did not show photosensitivity on the video-EEG. All but one of the 5 JME patients had experienced myoclonic seizures in intermittent photic stimulation (IPS) at the time of EC, associated with multiple spike and wave discharges. Two of the 3 EMA patients exhibited typical absences with eyelid myoclonia during the act of EC. The high rate of family history of epilepsy in first degree relatives of our patients was an outstanding feature, which could have future implications in research of the genetic basis of epilepsy patients with ECS."}, {"id": "pubmed23n0271_19232", "title": "Juvenile myoclonic epilepsy: a 5-year prospective study.", "score": 0.01397907647907648, "content": "We made a long term prospective study of 66 patients with juvenile myoclonic epilepsy (JME). Prevalence was 10.2% among 672 patients with epilepsies. Sex distribution was equal. Sixty-three were not diagnosed on referral; JME was not initially recognized in the epilepsy clinic in 22. Clinical typical absence seizures were reported in 33.3%, myoclonic jerks in 97% and generalized tonic-clonic seizures (GTC) in 78.8% of the patients. Mean age (+/- SD) at onset was 10.5 +/- 3.4 years (range 5-16 years) for absence seizures, 15 +/- 3.5 years (range 8-26 years) for myoclonic jerks, and 16 +/- 3.5 years (9-28) years (range 1-9 years) and GTC by 4.4 +/- 2.7 years (range 1-8 years) in 14 (21.2%) patients who manifested all three types of seizure. Absence were never antedated by myoclonic jerks or GTC. Myoclonic jerks occurred on awakening in 87.5% of the patients. GTC occurred mainly on awakening, but other patients had nocturnal or diurnal GTC with no circadian distribution. Neurologic examination was normal for all patients except for tremor of the hands similar to essential tremor, noted in 35% of patients. Computed tomography (CT) brain scans were normal: 93% of patients had precipitating factors: sleep deprivation (89.5%), fatigue (73.7%), photosensitivity (36.8%; television and video games 8.8%), menstruation (24.1% of women), mental concentration (22.8%), and stress (12.3%). Incidence of JME among siblings (13 of 41 examined families) implies an autosomal recessive mode of inheritance for this Arab population. EEGs were frequently normal in treated patients. At least one abnormal EEG was recorded in 56 (84.9%) patients. Abnormalities consisted mainly of generalized discharges of spike/double spike and/or polyspike and slow wave. Frequent multiple spikes and discharge fragmentations varied from 0.5- to 20-s duration (mean 6.8 s). Twenty (30.3%) had focal abnormalities, and 18 (27.3%) had photoconvulsive discharges. Eighty-eight percent of patients remained seizure-free for &gt; or = 3 years of follow-up. Effective treatment was achieved with valproate (VPA); control of myoclonic jerks was improved with clonazepam (CZP). CZP monotherapy did not consistently prevent GTC. Adding small doses of CZP with simultaneous reduction of VPA was the most effective and better tolerated form of medication, particularly in patients demonstrating an adverse reaction or requiring a large VPA dosage. VPA dosage was successfully reduced in 15 patients who were seizure-free for &gt; 2 years and had infrequent seizures before treatment, but 9 of 11 patients relapsed after VPA discontinuation.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0710_20674", "title": "[Electroclinical features of myoclonic-atonic epilepsy].", "score": 0.013721583189928449, "content": "To summarize the electroclinical characteristics of myoclonic atonic epilepsy (MAE) in children. The clinical data, video electroencephalogram (EEG) and simultaneous electromyography (EMG) of MAE patients were analyzed. The treatment and its effects were followed up. In 47 MAE patients, 25 had a history of febrile seizures (FS), 20 had a family history of FS or epilepsy. All patients had a normal development before the illness. The age of afebrile seizure onset was between 1.4 years to 5.8 years. The first seizure was generalized tonic-clonic seizure (GTCS) in 41 patients (87.2%). All patients had multiple seizure types, including 47 GTCS (97.9%), 34 myoclonic atonic seizures (72.3%), 47 myoclonic seizures (100%), 32 atonic seizures (68.1%), 36 atypical absences (76.6%) and 3 tonic seizures (6.4%). EEG backgrounds were slow or parietal θ rhythm, interictal EEG showed 1-4 Hz (predominant 2-3 Hz) generalized spike and wave or poly spike and wave discharges in all cases. Seizures were controlled by antiepileptic drugs (AEDs) in 41 patients (87.2%). Valproate was used in 37. Lamotrigine was used in 26. Mild mental retardation was observed in 10 children after the onset of the illness. The clinical features of MAE included the following: the development was normal before the onset of the illness; the onset of seizure type was often GTCS. All patients had multiple generalized seizure types. Myoclonic atonic seizure was its characteristic seizure type. EEG showed generalized discharges. Early diagnosis and rational choice of AEDs are important for getting a better prognosis."}, {"id": "pubmed23n0387_5450", "title": "Myoclonic-astatic epilepsy of early childhood--clinical and EEG analysis of myoclonic-astatic seizures, and discussions on the nosology of the syndrome.", "score": 0.013515870247645622, "content": "The aim of this study is to elucidate the clinical and neurophysiological characteristics of the myoclonic, myoclonic-astatic, or astatic seizures in patients with myoclonic-astatic epilepsy (MAE) of early childhood, and to discuss on the nosology of this unique epileptic syndrome. The subjects included 30 patients, who fulfilled the following modified International League Against Epilepsy (ILAE) criteria for MAE, and whose main seizures were captured by video-electroencephalographs (EEG) or polygraphs. The modified ILAE criteria includes: (1) normal development before onset of epilepsy and absence of organic cerebral abnormalities; (2) onset of myoclonic, myoclonic-astatic or astatic seizures between 7 months and 6 years of age; (3) presence of generalized spike- or polyspike-wave EEG discharges at 2-3 Hz, without focal spike discharges; and (4) exclusion of severe and benign myoclonic epilepsy (SME, BME) in infants and cryptogenic Lennox-Gastaut syndrome based on the ILAE definitions. The seizures were investigated precisely by video-EEG (n=5), polygraph (n=2), and video-polygraph (n=23), which identified myoclonic seizures in 16 cases (myoclonic group), atonic seizures, with or without preceding minor myoclonus, in 11 cases (atonic group), and myoclonic-atonic seizures in three cases. All patients had a history of drop attacks, apart from ten patients with myoclonic seizures. Myoclonic seizures, involving mainly the axial muscles were classified into those with mild intensity not sufficient to cause the patients to fall (n=10) and those that are stronger and sufficient to cause astatic falling due to flexion of the waist or extension of the trunk (n=6). Patients in the atonic group fell straight downward, landed on their buttocks, and recovered immediately. Analysis of the ictal EEGs showed that all attacks corresponded to the generalized spike or polyspikes-and-wave complexes. In the atonic form, the spike-and-wave morphology was characterized by a positive-negative-deep-positive wave followed by a large negative slow wave. In two patients, the intensity of the atonia appeared to correspond to the depth of the positive component of the spike-and-wave complexes. We did not detect any significant differences in the clinical and EEG features and prognosis, between the atonic and myoclonic groups. Although the determination of exact seizure type is a prerequisite for diagnosing an epileptic syndrome, the strict differentiation of seizure type into either a myoclonic or atonic form, does not appear to have a significant impact on the outcome or in delineating this unique epileptic syndrome. At present, we consider it better to follow the current International Classification of Epileptic Syndromes and Epilepsies until a more appropriate system than the clinico-electrical approach for classifying patients with MAE is available."}, {"id": "pubmed23n0788_11072", "title": "Clinical and EEG characteristics of Juvenile Myoclonic Epilepsy.", "score": 0.013432499436556233, "content": "Objective : To determine the clinical and electroencephalographic characteristics of patients with Juvenile Myoclonic Epilepsy (JME). In this descriptive case series study, 60 patients of Juvenile myoclonic epilepsy (JME) were included. After detailed history clinical examination, Electroencephalography (EEG) with standard protocol was performed in all patients and was analyzed by a neurologist. Out of 60 patients, 26 (43.3%) were males and 34 (56.6%) were females. Mean age at the onset of myoclonic jerks (MJ) and generalized tonic clonic seizures (GTCS) was 13.7 ± 2.12 years and 14.15 ± 1.79 years respectively. Average delay in the diagnosis was 5.2 years. Myoclonic jerks (MJ) were present in all patients, GTCS in 52 (86.6%), and absence seizures in 8 (13.33%) patients. 6 (10%) had only Myoclonic Jerks. First seizure type was MJ in 52 (86.6%) and absence in 8 (13.3%). Most common precipitating factors were sleep deprivation in 80% and fatigue in 66.6%. Family history for epilepsy was positive in 20%. Diagnosis by referring physicians was JME in only 6 (10%) patients. EEG was abnormal in 42 patients (70%) showing generalized , 4- to 6-Hz polyspike and wave in 27 (45%), generalized single spike/ sharp waves in 7 patients (11.6%), 8 (13.3%) patients had 3-Hz spike-and-wave (SW) activity in addition to the polyspike-and-wave (PSW) pattern. Independent focal EEG abnormalities were noted in 12 patients (20%). Many of our patients were misdiagnosed by the referring physicians and were prescribed inappropriate antiepileptic drugs. Factors causing misdiagnosis were failure to elicit history of myoclonic jerks, misinterpreting myoclonic jerks as partial seizures and misinterpretation of EEG abnormalities."}, {"id": "wiki20220301en429_18528", "title": "Epilepsy syndromes", "score": 0.013009217960154622, "content": "Childhood absence epilepsy Childhood absence epilepsy (CAE) is a genetic generalized epilepsy that affects children between the ages of 4 and 12 years of age, although peak onset is around five to six years old. These patients have recurrent absence seizures, brief episodes of unresponsive staring, sometimes with minor motor features such as eye blinking or subtle chewing. The EEG finding in CAE is generalized 3 Hz spike and wave discharges. Some go on to develop generalized tonic-clonic seizures. This condition carries a good prognosis because children do not usually show cognitive decline or neurological deficits, and the seizures in the majority cease spontaneously with ongoing maturation."}, {"id": "wiki20220301en233_6198", "title": "Panayiotopoulos syndrome", "score": 0.01293297345928925, "content": "In Panayiotopoulos’ original study, ictal vomiting occurred in only 24 children out of 900 patients of all ages with epileptic seizures. Twenty-one were otherwise normal children (idiopathic cases constituting what is now considered Panayiotopoulos syndrome), and 3 had symptomatic epilepsies. Half of the seizures were lengthy, lasting for hours (autonomic status epilepticus). The EEG of the 21 idiopathic cases showed great variations: 12 had occipital paroxysms or spikes alone or with extraoccipital spikes; 2 had central spikes and giantsomatosensory evoked spikes; 2 had midline spikes; 1 had frontal spikes; 1 had brief generalized discharges; and 3 had consistently normal EEG. Subsequent attention was focused on the predominant group with occipital spikes, which was established as \"early onset benign childhood epilepsy with occipital paroxysms\". The other group of 9 children with extraoccipital spikes or normal EEGs was reevaluated much later; their clinical manifestations and"}, {"id": "pubmed23n0554_469", "title": "Epilepsy with myoclonic absences.", "score": 0.012537103577566006, "content": "Among the epileptic syndromes that are defined mainly on the basis of a characteristic seizure type, epilepsy with myoclonic absences (EMA) stands out as a somewhat controversial entity. This is because the sound and evident clinical characteristics on which it was identified some 30 years ago have evolved, mostly as a consequence of changes in the practical management of epilepsies and to the description of myoclonic components in a variety of other generalised epilepsies with absences. Myoclonic absences (MA) are described as typical absences with sudden onset and offset that are associated with generalised spike and wave (SW) discharges on the ECG, with distinctive traits. Clinically, absences are associated with axial hypertonia (the subject usually bends forward and slightly raises their shoulders and arms), and jerks synchronous with the SW discharges. Neurophysiologically, axial hypertonia and rhythmic jerks may be recorded on polygraphic surface electromyogram leads in association with the typical SW discharges; as such, despite an ECG, the diagnosis may be missed in the absence of video documentation of the seizure and/or adequate polygraphy. MA need to be distinguished from absences with other types of prominent myoclonic accompaniment (perioral, eyelid, limbs).The prognosis of EMA remains variable. Modern therapeutic combinations, such as valproic acid and ethosuximide, or valproic acid and lamotrigine, are usually effective; however, in a proportion of patients, seizures are resistant to drug treatment. These patients may experience cognitive deterioration and, in some cases, evolution towards a more severe form of epilepsy, including the Lennox-Gastaut syndrome. The more benign cases usually present with MA as the only seizure type, while patients who experience other seizures, especially generalised tonic-clonic seizures, in association with MA may have a less favourable outcome."}, {"id": "pubmed23n0546_22772", "title": "Diagnostic issues and treatment of cryptogenic or symptomatic generalized epilepsies.", "score": 0.012535548570573266, "content": "To clarify the diagnostic issues and treatment of patients with cryptogenic or symptomatic generalized epilepsies, not including West syndrome (WS), we investigated electroclinical change during the clinical course, and treatment effects in these patients. The selection criteria were minor generalized seizures as their main seizure type and diffuse epileptic discharges as their main EEG findings. Regarding EEG, we included EEGs that predominantly displayed multifocal spike-waves because of the inclusion of severe epilepsy with multiple independent spike foci (SE-MISF). We divided the subjects into two groups according to their main seizure types: Group A (54 patients) with brief tonic seizures and Group B (24 patients) with myoclonic seizures and/or atypical absences. The main epileptic syndromes were considered to be Lennox-Gastaut syndrome and SE-MISF in Group A, and epilepsy with myoclonic-astatic seizures in Group B. A history of WS was often seen in Group A, but it was exceptional in Group B. During the clinical course, seizure types did not basically change in Group A. EEG patterns were changeable in both groups. Although there was some overlap in electroclinical manifestations among epileptic syndromes, a transition between the two groups was not seen. High-dose valproate and ethosuximide were the most effective in Groups A and B, respectively. Long-term prognosis was significantly more favorable in Group B than in Group A."}, {"id": "pubmed23n0408_10053", "title": "Idiopathic generalised epilepsies with 3 Hz and faster spike wave discharges: a population-based study with evaluation and long-term follow-up in 71 patients.", "score": 0.012284097677188802, "content": "For several years we have been following patients with intractable, childhood-onset idiopathic generalised epilepsies with &gt; or = 3 Hz spike-wave discharges. Our need to find explanations for their intractability was the starting point for this study. We were interested in identifying characteristics, which would predict intractability; evaluating how these patients were treated and whether polytherapy was useful. We identified patients with &gt; or = 3 Hz spike-wave discharges by reviewing EEG reports recorded between 1983 and 1992. Data were collected from medical records and through personal interviews. We identified 82 patients with tentative idiopathic generalised epilepsy. Eleven were excluded. Thirty-eight patients had childhood absence epilepsy, 18 had juvenile absence epilepsy, 13 had juvenile myoclonic epilepsy and two had eyelid myoclonia with absences: 89.5, 78, 38 and 0% of the patients in each group, respectively, had been seizure free for more than 2 years. Twenty percent of the patients had intractable seizures. All intractable patients with juvenile absence epilepsy had rhythmic, random eyelid blinking and generalised tonic-clonic seizures. A history of more than ten generalised tonic-clonic seizures was associated with intractability in juvenile myoclonic patients. Monotherapy with ethosuximide or valproate resulted in seizure control in 65% of patients. Seventeen patients (24%) were treated with polytherapy, six achieved remission. These six patients had childhood absence epilepsy and juvenile absence epilepsy. Positive outcome was found in childhood absence epilepsy and juvenile absence epilepsy. Intractable seizures were more frequent among patients with juvenile myoclonic epilepsy. None of them benefited from polytherapy with conventional anti-epileptic drugs."}, {"id": "wiki20220301en024_53182", "title": "Myoclonus", "score": 0.012245254963701565, "content": "Juvenile myoclonic epilepsy (JME) usually consists of jerking and muscle twitches of the upper extremities. This may include the arms, shoulders, elbows, and very rarely, the legs. JME is among the most common types of epilepsy and can affect one of every 14 people with the disease. These seizures typically occur shortly after waking up. Onset for JME can be seen around puberty for most patients. Administration of medications that also treat multiple seizure types is usually the most effective form of treatment. Lennox-Gastaut syndrome (LGS), or childhood epileptic encephalopathy, is a rare epileptic disorder accounting for 1–4% of childhood epilepsies. The syndrome has much more severe symptoms ranging from multiple seizures daily, learning disabilities, abnormal findings in electroencephalogram (EEG). Earlier age of seizure onset is correlated with higher risk of cognitive impairment."}, {"id": "article-36251_9", "title": "Juvenile Myoclonic Epilepsy -- Evaluation", "score": 0.01208580347681645, "content": "History provides most of the clues necessary for a diagnosis of JME. Physical examination is generally unremarkable. An electroencephalogram (EEG) provides supporting evidence for diagnosing JME. An interictal EEG is abnormal in the majority of patients with JME. [16] If the routine EEG is normal, an overnight and sleep-deprived EEG is abnormal in almost every patient. The abnormalities are seen mostly during the transition from sleep to awakening. [17] The typical EEG in JME shows diffuse, symmetric, bilateral 4 to 6 hertz (Hz) polyspike and wave discharges with a fronto-central predominance. The background has normal alpha rhythm, and approximately half of the patient population will have focal or asymmetric abnormalities. [18] Ictal or continuous EEG recording shows 10-16 Hz polyspike discharges with myoclonic jerks. The spikes correlate with myoclonic jerks. [19] The EEG findings in GTC (low voltage fast activity with spike and wave discharges) and absence seizures (generalized 3 Hz spike and wave discharges) associated with JME are similar to those seen with other epilepsies. Since it is known to be photosensitive epilepsy, photic stimulation provides enhanced yield on EEG in JME."}, {"id": "wiki20220301en429_18534", "title": "Epilepsy syndromes", "score": 0.011411094705758278, "content": "Febrile infection-related epilepsy syndrome Febrile infection-related epilepsy syndrome (FIRES) Frontal lobe epilepsy Frontal lobe epilepsy, usually a symptomatic or cryptogenic localization-related epilepsy, arises from lesions causing seizures that occur in the frontal lobes of the brain. These epilepsies can be difficult to diagnose because the symptoms of seizures can easily be confused with nonepileptic spells and, because of limitations of the EEG, be difficult to \"see\" with standard scalp EEG. Juvenile absence epilepsy is an idiopathic generalized epilepsy with later onset than CAE, typically in prepubertal adolescence, with the most frequent seizure type being absence seizures. Generalized tonic-clonic seizures can occur. Often, 3 Hz spike-wave or multiple spike discharges can be seen on EEG. The prognosis is mixed, with some patients going on to a syndrome that is poorly distinguishable from JME."}, {"id": "wiki20220301en334_34775", "title": "Myoclonic astatic epilepsy", "score": 0.01085551822176273, "content": "The outcome is unfavorable if generalized tonic-clonic, tonic, or clonic seizures appear at the onset or occur frequently during the course. Generalized tonic-clonic seizures usually occur during the daytime in this disorder, at least in the early stages. Nocturnal generalized tonic-clonic seizures, which may develop later, are another unfavorable sign. If tonic seizures appear, prognosis is poor. Status epilepticus with myoclonic, astatic, myoclonic-astatic, or absence seizures is another ominous sign, especially when prolonged or appearing early. Failure to suppress the EEG abnormalities (4- to 7-Hz rhythms and spike-wave discharges) during therapy and absence of occipital alpha-rhythm with therapy also suggest a poor prognosis (Doose 1992a)."}, {"id": "wiki20220301en123_27515", "title": "Idiopathic generalized epilepsy", "score": 0.01081548321808062, "content": "Juvenile absence epilepsy Juvenile absence epilepsy is similar to CAE but has an onset between ages 9 and 13. Other differences are that patients with this disorder have less frequent but longer absence seizures than those with CAE. There are a number of possible genetic loci for this disorder, though no causative genes have been demonstrated. Juvenile myoclonic epilepsy Also known as Janz syndrome, juvenile myoclonic epilepsy (JME) is a common form of epilepsy, accounting for ~10% of all cases and ~25% of cases of idiopathic generalized epilepsies. Many children with CAE go on to develop JME. JME first presents between the ages of 12 and 18 with prominent myoclonic seizures. These seizures tend to occur early in the morning. Patients with JME may also have generalized tonic-clonic seizures and absence seizures. Linkage of this disorder has been shown to mutations in the genes GABRA1, CACNB4, CLCN2, GABRD2, EFHC1, and EFHC2."}, {"id": "pubmed23n0214_31", "title": "Some clinical and EEG aspects of benign juvenile myoclonic epilepsy.", "score": 0.010367502784112002, "content": "Twelve patients with benign juvenile myoclonic epilepsy (BJME) representing 4% of our population of epileptics (n = 275) are presented. Only two patients (17%) had myoclonic jerks as the only seizure type. Seven (58%) had generalized tonic-clonic seizures (GTCS) and myoclonus. Three patients (25%) had absence seizures (AS), GTCS, and myoclonic jerks. Electroencephalographic evidence of photosensitivity was found in four (33%). Auditory precipitation of seizures was found in one patient. As is the case with other primary generalized epilepsies, the onset of BJME seems to be age specific. In our series the mean age of onset in years was 4.3 for AS, 14.75 for myoclonic jerks, and 16.4 for GTCS. It took an average of 8.5 years from the onset of BJME (range, 2-20 years) and 6.5 years from the onset of GTCS (range, 2 months-6 years) until the condition was properly recognized. Five patients experienced at least one episode of myoclonic status epilepticus. Generalized, paroxysmal, symmetric polyspike and slow wave discharges are the typical EEG finding. These complexes, however, showed considerable interpatient variability. Sleep deprivation proved to be the most valuable activating procedure. Valproic acid monotherapy effectively controlled myoclonic jerks as well as associated GTCS in most patients."}, {"id": "pubmed23n0260_5359", "title": "[Janz's juvenile myoclonic epilepsy: a little-known frequent syndrome. A study of 85 patients].", "score": 0.009900990099009901, "content": "Juvenile myoclonic epilepsy (JME) constitutes 10% of all epilepsies. Despite this syndrome being well defined, its diagnosis is usually delayed. The aim of this study was to analyze the clinical and electroencephalographic characteristics to facilitate guidelines to contribute to its recognition. From January 1986 to July 1993 the clinical and EEG data of 85 patients with JME were prospectively studied. In 68 cases (80%) the polygraphic study of sleep was also analyzed during a nap period. The series included 44 males and 41 females of a mean age of 28 years (range: 13-63). Fifty-six percent of the cases showed family history of epilepsy and/or febrile convulsions. All the patients had myoclonic crisis with the age of 15 being the mean age of initiation (range: 8-27). Eighty-seven percent also had generalized tonic-clonic crisis and 18% typical absences. Myoclonias were presented daily up the administration of adequate treatment in 60% of the cases with 21% having myoclonic status. The mean interval from the initiation of the myoclonic crisis to diagnosis of JME was of 10.6 years. On monotherapy with valproic acid and following a mean follow up period of 23.8 months, 86% of the patients remained free of crisis. Nonetheless, the rate of recurrence was 100% in the 19 patients who discontinued the treatment. Surveillance EEG was normal on some occasion in 88% of the cases. The most characteristic paroxysms were the following: wave-point at 4-5 Hz and generalized rapid wave-polypoint. Light stimulation provoked a paroxysmal response in one third of the cases. Sleep EEG was abnormal in all the patients. An activation of the paroxysms during non-REM sleep in 78% of the cases and on waking up in 25%. Juvenile myoclonic epilepsy is a well defined syndrome. Its diagnosis is based on directed anamnesis allowing myoclonic jerks to be collected which often remain unperceived, and EEG exploration with sleep tracing in which the characteristic outbreaks of wave-point or generalized rapid wave-polypoints may be discovered."}, {"id": "pubmed23n0286_2555", "title": "[Clinical-electrophysiological pattern of juvenile myoclonic epilepsy].", "score": 0.00980392156862745, "content": "18 patients (9 females, 9 males) with juvenile myoclonic epilepsy (JME) were studied. Despite a fairly long duration of the disease (from 1 to 34 years--mean 9.5 years) the patients had not been properly diagnosed. In all cases routine serial EEG examinations, 24-hour EEG with Medilog System 9000, and polygraphic Video/EEG/EMG recording were done (Videometry Processor, Glonner). Typical attack triads occurred in 7 cases (39%), grand mal seizures and myoclonic attacks in 10 cases (56%), one patient had only myoclonic seizures. EEG demonstrated already in routine recording occurrence of seizure activity with spike/polyspike-slow wave 3-6 Hz complexes. 24-hour EEG made possible demonstration of these complexes in the remaining 4 cases. Seizure activity during clinical myoclonic seizures were recorded in 8 cases, and during absences in 2 cases. A valuable method was also Video/EEG/EMG recording which showed occurrence of both myoclonic seizures (in 5 cases) and absences (9 cases). Three patients with absences were not aware of their seizures. In 8 patients EEG findings demonstrated focal abnormalities which contributed to previous diagnostic errors. The described clinical and electrophysiological features met fully the diagnostic criteria of JME. In the Polish literature this is the first report on such a large groups of JME cases. Attention is called to diagnostic difficulties due to poor knowledge of this disease and its manifestations which leads to inaccurate history taking. Another cause is non-availability of full neurophysiological diagnostic facilities in many centers."}, {"id": "pubmed23n0325_19180", "title": "Severe idiopathic generalized epilepsy of infancy with generalized tonic-clonic seizures.", "score": 0.009788044772564897, "content": "While the literature on infantile epilepsies with minor and major seizures is extensive, little consideration has been given to infantile epilepsy with generalized tonic-clonic seizures (GTCS) alone. The aim of the present study was to analyze the data of a large group of patients and their families to obtain further insight into the clinical picture and pathogenesis of this type of epilepsy. The 101 children (58 boys, 43 girls) met the following inclusion criteria: onset of the epilepsy with febrile or afebrile GTCS in the first 5 years of life, absence of primary organic brain lesion or progressive brain disease, severe course with frequent febrile and/or afebrile GTCS, failure of conventional anticonvulsive therapy. The epilepsy predominantly afflicts normally developed infants, boys and girls being about equally affected. The epilepsy begins with frequent febrile or afebrile GTCS, characteristically of long duration and often with alternating lateralization. In half of the cases additional myoclonic or myoclonic astatic seizures and/or absences occur. The initial GTCS phase is the same in epilepsies with and without minor seizures. Erratic myoclonias are especially characteristic. With advancing age, the symptomatology becomes increasingly polymorphic due to the occurrence of additional simple and complex focal and tonic seizures. Severe impairment of mental development soon after onset is a leading symptom. The overall death rate was 9%. Only 11% of the patients had been seizure-free for at least two years at final examination. The EEG was initially normal and subsequently exhibited diffuse 4-7/s rhythms, and only later spikes and waves of irregular shape (87%). Focal sharp waves occurred transiently in 26%. The family history and EEG of probands and relatives showed the pathogenesis to be decisively determined by genetic factors. Early infantile GTCS epilepsy represents a genetically determined (idiopathic) epileptic encephalopathy. It overlaps with other forms of early childhood epilepsy such as severe myoclonic epilepsy, severe type of myoclonic astatic epilepsy, as well as early childhood absence epilepsy with GTCS."}, {"id": "wiki20220301en207_16744", "title": "Spike-and-wave", "score": 0.009708737864077669, "content": "Lennox-Gastaut syndrome Epileptic encephalopathies are a group of conditions that result in deterioration of sensory, cognitive, and motor functions due to consistent epileptic activity. Lennox-Gastaut syndrome (LGS) is a childhood epileptic encephalopathy characterized with generalized seizures and slow spike-wave activity while awake. LGS is a combination of atonic absences, tonic seizures, cognitive deterioration, and slow spike-wave activity in the EEG. This syndrome usually results from focal, multifocal, or diffuse brain damage and can be divided into symptomatic and cryptogenic types. Cognitive deterioration with high-frequency spike-wave activity affects most patients 2–9 years old with generalized seizures. The age of onset for LGS is between 1 and 10 years, between 2 and 6 years for symptomatic cases and 5 and 8 years for cryptogenic cases. Episodes can be triggered by modifications of treatment, which usually involves benzodiazepines, or changes in the conditions of life."}, {"id": "pubmed23n0579_4799", "title": "Usefulness of a morning routine EEG recording in patients with juvenile myoclonic epilepsy.", "score": 0.009708737864077669, "content": "To evaluate if a standard awake EEG recording in the morning is superior to afternoon awake EEG session in detecting generalized epileptiform discharges (GEDs) in patients with juvenile myoclonic epilepsy (JME). The study group included 29 consecutive patients (23 women; mean age 22.3+/-6.3 years; age at onset of JME 15.4+/-3.4 years) with JME. Out of 29 patients 5 were untreated, 9 patients were treated with valproate, 8 with lamotrigine, 6 with levetiracetam and 1 patient with valproate plus phenobarbital. Two routine consecutive interictal EEG recordings were performed at 9a.m. and at 3p.m., respectively, while the subject was awake, on the same day after a a regular nocturnal sleep at own home. The morning EEG recording showed GEDs (i.e., generalized polispike and waves, photoparoxysmal response, or both). in 20/29 patients. In 15 of these 20 patients, the afternoon recording was normal and this difference was statistically significant (p &lt; or = 0.001). Moreover, there was a striking reduction of GEDs in three of the remaining five patients. Nine/29 patients had both morning and afternoon EEG recording normal. The results of this study have illustrated a significant greater rate of detection of generalized epileptiform abnormalities by performing standard awake EEG in the morning in comparison with an afternoon session."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[0, 301]], "word_ranges": [[0, 48]], "text": "Having antibodies against the core implies natural contact, and that it is IgG, which is not acute. The absence of e antigen rules out active replication. And the persistence of surface antigen (HBsAg) and virus DNA indicates that it is still present. Together, these indicate an asymptomatic carrier."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Having antibodies against the core implies natural contact, and that it is IgG, which is not acute. The absence of e antigen rules out active replication. And the persistence of surface antigen (HBsAg) and virus DNA indicates that it is still present. Together, these indicate an asymptomatic carrier. Carriers of the pre-core mutant usually have recurrent symptomatic periods and increased liver inflammation. There would be insufficient data to rule it out completely, but it is not among the answers and indicates a completely normal physical examination.", "full_answer_no_ref": "Having antibodies against the core implies natural contact, and that it is IgG, which is not acute. The absence of e antigen rules out active replication. And the persistence of surface antigen (HBsAg) and virus DNA indicates that it is still present. Together, these indicate an asymptomatic carrier. Carriers of the pre-core mutant usually have recurrent symptomatic periods and increased liver inflammation. There would be insufficient data to rule it out completely, but [HIDDEN] and indicates a completely normal physical examination.", "full_question": "Indicate the clinical situation in relation to hepatitis B virus infection in a 5-year-old patient from Nigeria, with normal physical examination and the following serology for hepatitis B: HBsAg + / ANTI-HBs - / HbeAg - / ANTI-HBe + / ANTI-HBc IgM - / ANTI-HBc IgG + / DNA HBV +:", "id": 276, "lang": "en", "options": {"1": "Acute infection.", "2": "Chronic infection.", "3": "Vaccinated patient.", "4": "Asymptomatic carrier.", "5": NaN}, "question_id_specific": 75, "type": "DIGESTIVE SYSTEM", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0220_8430", "title": "Frequency and persistence of serologic markers following three different manifestations of hepatitis B virus infection.", "score": 0.017027417027417027, "content": "30 asymptomatic chronic carriers of hepatitis B surface antigen (HBsAg), 6 asymptomatic blood donors transiently infected with hepatitis B virus, and 38 patients with acute hepatitis B were tested for HBsAg, anti-HBs, HBeAg, anti-HBe and anti-HBc. Comparison of these results revealed significant variation in the frequency of HBeAg which was present in 1 (3.3%) carrier, 2 (33.3%) of the transiently positive donors, and in 24 (63.2%) of the patients with acute hepatitis. Anti-HBe was found in 28 (93.3%) of the carriers, 4 (66.6%) of the transiently positive donors, and in 8 (21%) of the patients. Variation was also seen in the strength of anti-HBc, with only the chronic carriers having titres which were consistently high (above 1,000). Retesting the two groups of donors after a period of approximately 2 years showed no change in the serologic status of the chronic carriers, while amongst the transient HBsAg positives the 2 HBeAg reactives had seroconverted, 1 of the anti-HBe positives had become non-reactive, and 2 of the 6 had developed anti-HBs. 6 of the patients with acute hepatitis B were serologically reexamined during convalescence and showed results similar to those seen in the transiently HBsAg-positive donors, with clearance of HBsAg in all, seroconversion from HBeAg to anti-HBe in 4, and the production of anti-HBs in 4."}, {"id": "pubmed23n0894_14737", "title": "[Atypical serological profiles in hepatitis B infections: investigation of S gene mutations in cases with concurrently positive for HBsAg and anti-HBs].", "score": 0.01562592482983131, "content": "Hepatitis B virus (HBV) causes different clinical manifestations, ranging from asymptomatic carriage to fulminant or chronic hepatitis. Serological tests are widely used for the diagnosis of HBV infections to detect viral markers. However, facing with atypical serological profiles in some patients leads to problems in interpreting of the results and management of the patients. The aims of this study were to investigate the atypical serologic profiles seen in patients screened for HBV infection and the S gene mutations in patients with concurrent positivity of HBsAg and anti-HBs. A total of 592 sera from patients (332 male, 260 female; age range: 13-84 years, mean age: 43.9 years) prediagnosed as HBV infection between January to September 2013, and screened for HBV markers (HBsAg, anti-HBs, HBeAg, anti-HBe, anti-HBc-IgM, anti-HBc-total and HBV-DNA) were included in the study. Of those samples 364 were screened only for HBsAg and anti-HBs markers. S gene mutations were investigated by direct sequencing method in sera which were concurrent positive for HBsAg and anti-HBs. In our study, 5.2% (31/592) of the sera yielded atypical serologic profiles. Of these 13 cases were concurrently positive for HBsAg and anti-HBs; nine were HBeAg positive, anti-HBe and HBV-DNA negative; eight were HBeAg, anti-HBe and HBV-DNA positive; and one was HBsAg and anti-HBs negative, anti-HBe and HBV-DNA positive. The rate of concurrent positivity of HBsAg and anti-HBs was 3.6% (13/364), while 76.9% (10/13) of those cases were also positive for HBV-DNA. DNA sequencing was performed for seven out of 10 samples which were positive for HBsAg, anti-HBs and HBV-DNA, however three samples were not used because of the low amounts. Sequence analysis of seven samples showed S gene mutations in two samples, one was sS143L with sS193L, a HBV vaccine escape mutation, and the other was sP120R, a HBV immune escape mutation. Of the patients 2.7% (10/364) was negative for both HBsAg and anti-HBs; in which nine were HBV-DNA negative and anti-HBe positive, while one was positive for both HBV-DNA and anti-HBe. The rate of concurrent positivity of HBeAg and anti-HBe was found as 1.4% (8/592), and all of these samples were HBV-DNA positive. No single positivity for HBsAg, anti-HBc, anti-HBs or HBV-DNA was not detected in any of the patients. In conclusion, HBsAg and anti-HBs concurrent positivity was the most frequently detected atypical profile in our study (3.6%), and in some (2/7) of these patients S gene mutations were determined."}, {"id": "wiki20220301en023_14705", "title": "Seroconversion", "score": 0.013717783992139672, "content": "On a serological assay, the presence of hepatitis B surface antigen (HBsAg) indicates an individual with a currently active hepatitis B infection, whether acute or chronic. The presence of core antibody (anti-HBc) indicates an individual with an infection in general, whether current or previously resolved. The presence of surface antibody (anti-HBs) indicates an individual with immunity to hepatitis B, whether due to previously resolved infection or due to hepatitis B vaccination. For example, an individual who has never had any exposure to HBV, either by vaccine or by infection, would test negative for the entire serology panel. An individual who has been vaccinated and never had an infection will test seropositive for anti-HBs due to vaccination and negative for markers of infection. An individual with an acute HBV infection would test positive for HBsAg and anti-HBc (total and IgM) while negative for anti-HBs. An individual with a chronic infection would test positive for HBsAg and"}, {"id": "pubmed23n0083_6842", "title": "The etiology of acute hepatitis superimposed upon previously unrecognized asymptomatic HBsAg carriers.", "score": 0.013634246517339205, "content": "To study the etiology of acute hepatitis superimposed upon previously unrecognized asymptomatic HBsAg carriers, paired sera were collected in acute and convalescence phases for measurement of HBeAg, anti-HBe, hepatitis B virus DNA and anti-delta from 76 adult patients with acute hepatitis who were HBsAg positive but IgM anti-HBc negative or positive only at low titer. None of them were IgM anti-hepatitis A virus positive on admission. Of the 34 patients who were HBeAg positive initially, two (5.9%) were diagnosed as having delta superinfection, and another two (5.9%) were suspected to have non-A, non-B virus superinfection because of a transient decrease of serum hepatitis B virus DNA. The remaining 30 (88.2%) cases were hepatitis B virus DNA negative with or without anti-HBe seroconversion on follow-up. The episodes of acute hepatitis in these cases may represent \"immune clearance of HBeAg\" or \"immune clearance of hepatitis B virus with delayed anti-HBe seroconversion,\" respectively, in the natural course of chronic hepatitis B virus infection. Of the patients who were anti-HBe positive initially, 23 (54.8%) were diagnosed as having delta superinfection, including eight with de novo seroconversion of anti-delta and 15 with a rising titer of anti-delta; 10 (23.8%) were positive for hepatitis B virus DNA and were considered as reactivation of hepatitis B virus, and the other nine (21.4%) were suspected as having non-A, non-B virus superinfection.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "article-22788_41", "title": "Hepatitis B -- Evaluation -- Interpretation of Serologic Markers", "score": 0.013259259259259259, "content": "Following serologic markers are often tested: Hepatitis B surface antigen (HBsAg), antibody to Hepatitis B surface antigen (anti-HBs), Hepatitis B core Ab (Anti-HBc) IgM, Hepatitis B core Ab (Anti-HBc) IgG, Hepatitis B e antigen (HBeAg), and Hepatitis B e antibody (anti-HBe). [10] HBsAg: Acute infection (less than 6 months) or chronic infection (more than 6 months). Anti-HBs: Recovery from acute infection or immunity from vaccination. HBeAg: Mostly associated with high viral load. Anti-HBe: Low replicative phase. Anti-HBc IgM: Acute infection, an only marker present in the window period, can be present during exacerbation of chronic infection. Anti-HBc IgG: Exposure to infection, chronic infection (if present along with HBsAg), recovery from acute infection (if present with anti-HBs), if isolated presence, may represent occult infection."}, {"id": "wiki20220301en206_1632", "title": "Hepatitis B", "score": 0.01305321946942884, "content": "Shortly after the appearance of the HBsAg, another antigen called e antigen (HBeAg) will appear. Traditionally, the presence of HBeAg in a host's serum is associated with much higher rates of viral replication and enhanced infectivity; however, variants of the virus do not produce the 'e' antigen, so this rule does not always hold true. During the natural course of an infection, the HBeAg may be cleared, and antibodies to the 'e' antigen (anti-HBe) will arise immediately afterwards. This conversion is usually associated with a dramatic decline in viral replication. If the host is able to clear the infection, eventually the HBsAg will become undetectable and will be followed by IgG antibodies to the surface antigen and core antigen (anti-HBs and anti HBc IgG). The time between the removal of the HBsAg and the appearance of anti-HBs is called the window period. A person negative for HBsAg but positive for anti-HBs either has cleared an infection or has been vaccinated previously."}, {"id": "wiki20220301en094_18175", "title": "Window period", "score": 0.01294871794871795, "content": "Hepatitis B Two periods may be referred to as window period in hepatitis B infection: (1) the period that elapses during HBsAg to HBsAb seroconversion, i.e. between the disappearance of surface antigen (HBsAg) from serum and the appearance of HBsAb (anti-HBs), and (2) the period between infection and appearance of HBsAg. During the window of HBsAg to HBsAb seroconversion, IgM anti-core (HBc-IgM) is the only detectable antibody. HBV DNA may be positive as well. This window period does not occur in persons who develop chronic hepatitis B, i.e. who continue to have detectable HBV DNA for greater than 6 months (HbsAg remains positive), or in people who develop isolated HBcAb positivity, i.e. who lose HBsAg, but do not develop HBsAb (HBV DNA may or may not remain positive). See also Incubation period, the time between infection and the appearance of symptoms References HIV/AIDS Serology"}, {"id": "wiki20220301en023_14706", "title": "Seroconversion", "score": 0.012770451926769939, "content": "individual with an acute HBV infection would test positive for HBsAg and anti-HBc (total and IgM) while negative for anti-HBs. An individual with a chronic infection would test positive for HBsAg and total anti-HBc (IgM and IgG), but negative for IgM anti-HBc and anti-HBs. An individual who has successfully resolved their HBV infection will test negative for HBsAg, positive for anti-HBc, and may test negative or positive for anti-HBs, although most will test positive."}, {"id": "wiki20220301en023_14702", "title": "Seroconversion", "score": 0.012348450830713965, "content": "In the typical disease course for hepatitis B, the individual will first seroconvert for hepatitis B surface antigen (HBsAg). While some can convert within one week, most individuals take about four weeks after initial infection to convert. Anti-core antibodies (anti-HBc) are the first antibodies produced by the body, first in short-term IgM (anti-HBc IgM), and subsequently in long-term IgG; while levels of IgM anti-HBc will peak around sixteen weeks after exposure and fall within about seven to eight months, IgG anti-HBc will remain detectable in the serum as a sign of chronic infection for years. IgM anti-HBc concentration will fall regardless of whether or not the individual clears the infection. The window period for HBsAg/anti-HBs testing occurs as concentration of HBsAg falls and before the body develops anti-HBs antibodies, lasting approximately six to eight weeks in most individuals. During this time, serology assays can test for total anti-HBc. Levels of anti-surface antibody"}, {"id": "wiki20220301en349_29113", "title": "Vaccine-induced seropositivity", "score": 0.011390079608083283, "content": "Hepatitis B When a person gets a hepatitis B vaccine then the most common test for hepatitis B will show them to be positive. The usual course of action, in this case, is to give the person a panel of tests for HBsAg, anti-HBc, and anti-HBs (hepatitis B surface antigen, anti-hepatitis B core, and anti-hepatitis B surface). A person who has never been exposed to hepatitis B but has gotten the vaccine will be positive for anti-HBs but negative for the other two tests in the panel. Other combinations of positive and negative in this test can mean other things, such as acute, chronic, or past infection."}, {"id": "wiki20220301en001_273611", "title": "Hepatitis", "score": 0.01109211543941059, "content": "Viral hepatitis Viral hepatitis is primarily diagnosed through blood tests for levels of viral antigens (such as the hepatitis B surface or core antigen), anti-viral antibodies (such as the anti-hepatitis B surface antibody or anti-hepatitis A antibody), or viral DNA/RNA. In early infection (i.e. within 1 week), IgM antibodies are found in the blood. In late infection and after recovery, IgG antibodies are present and remain in the body for up to years. Therefore, when a patient is positive for IgG antibody but negative for IgM antibody, he is considered immune from the virus via either prior infection and recovery or prior vaccination. In the case of hepatitis B, blood tests exist for multiple virus antigens (which are different components of the virion particle) and antibodies. The combination of antigen and antibody positivity can provide information about the stage of infection (acute or chronic), the degree of viral replication, and the infectivity of the virus."}, {"id": "pubmed23n0806_9756", "title": "Occult hepatitis B virus infection with positive hepatitis B e antigen.", "score": 0.010836457357075915, "content": "Hepatitis B e antigen (HBeAg) is a marker to indicate active replication of hepatitis B virus (HBV). Occult HBV infection (OBI), referred to persistence of HBV DNA in serum and/or liver without detectable serum hepatitis B surface (HBsAg), usually has low HBV DNA levels. The presence of HBeAg in OBI is unusual. We report 2 patients who presented negative for HBsAg but positive for HBeAg and HBV DNA. HBV markers were quantified in the longitudinal sera in a period of 1-2years. The HBV DNA sequences were analyzed in 2 patients' sera and 1 patient's liver. Both patients were also positive for total anti-HBs and anti-HBc but negative for anti-HBe and anti-HBc IgM. HBV DNA levels were 234-567IU/ml in case 1 and 42-1130IU/ml in case 2. The alignment analysis of the S gene showed that HBV in both patients was genotype C, serotype adr. Cloning analysis of the a determinant of HBsAg showed that the immune escape mutants were predominant in both patients over the follow-up period. The HBV had double mutations (A1762T and G1764A) in the basal core promoter but had no mutation in the pre C/C gene in both patients. The patients with negative HBsAg but positive HBeAg may represent a unique type of OBI. Test for HBeAg would be critical to identifying such type of OBI."}, {"id": "Obstentrics_Williams_7579", "title": "Obstentrics_Williams", "score": 0.010120255357054487, "content": "Figure 55-2 details the sequence of the various HBV antigens and antibodies in acute infection. The first serological marker to be detected is the hepatitis B surface antigen (HBsAg) , often preceding the increase in transaminase levels. As HBsAg disappears, antibodies to the surface antigen develop (anti-HBs), marking complete resolution of disease. Hepatitis B core antigen is an intracellular antigen and not detectable in serum. However, anti -HBc is detectable wi thin weeks of HBsAg appearance. The hepatitis Be antigen (HBeAg) is present during times of high viral replication and often correlates with detectable HBV DNA. After acute hepatitis, approximately 90 percent of adults recover completely. The 10 percent who remain chronically infected are considered to have chronic hepatitis B. Chronic HBV infection is oten asymptomatic but may be clinically suggested by persistent anorexia, weight loss, fatigue, and"}, {"id": "wiki20220301en206_1631", "title": "Hepatitis B", "score": 0.010024665257223397, "content": "The surface antigen (HBsAg) is most frequently used to screen for the presence of this infection. It is the first detectable viral antigen to appear during infection. However, early in an infection, this antigen may not be present and it may be undetectable later in the infection as it is being cleared by the host. The infectious virion contains an inner \"core particle\" enclosing viral genome. The icosahedral core particle is made of 180 or 240 copies of the core protein, alternatively known as core antigen, or HBcAg. During this 'window' in which the host remains infected but is successfully clearing the virus, IgM antibodies specific to the core antigen (anti-HBc IgM) may be the only serological evidence of disease. Therefore, most diagnostic panels contain HBsAg and total anti-HBc (both IgM and IgG)."}, {"id": "pubmed23n0022_10512", "title": "The significance of the Australia antigen (HBsAg) persistent healthy carrier \"status\": a long-term follow-up study of 34 cases.", "score": 0.009900990099009901, "content": "Thirty-four persistent healthy carriers of HBsAg (serum HBsAg detectable for longer than 3 months with normal liver function tests and normal liver histology or slight aspecific abnormalities) were discovered by routine testing of household relatives of B virus hepatitis patients. The carriers were followed-up for 11 to 37 months by clinical control, liver function tests and liver needle biopsy. None carrier had previous jaundice. During the follow-up period, in 17 of the 34 subjects, was there no evidence of deterioration in either clinical state, liver function of pathological findings. In 5 of the 34 carriers, HBsAg disappeared from serum after a period ranging from 6 to 12 months. The remaining 12 cases developed clinical and histological picture of acute viral hepatitis after 6 to 29 months (mean 12 months). Of these 12 patients, 6 recovered and become HBsAg; 2 remained HBsAg healthy carriers despite normalization of biochemical and histological abnormalities; 3 progressed from the acute stage to antigen positive CAH. The remaining one case could not be followed-up after the acute hepatitis. Our data indicate that the outcome of the HBsAg carrier state is unpredictable and stress the need of long-term follow-up surveillance."}, {"id": "pubmed23n0283_20693", "title": "[Asymptomatic carriers of HBsAg: is a follow-up necessary?].", "score": 0.00980392156862745, "content": "We evaluated the clinical and epidemiological data of 142 HBsAg carriers. This prospective trial is part of a program of study and follow-up in HVB patients. The median age was 34.58 years old, males 56.3%. The average follow-up was 32.4 months. Complete clinical history, routine analysis, liver function tests, alfa-fetoprotein, serology for HVB, HCV and HDV and abdominal ecography were done in all patients. DNA-HVB was done only in special cases. Patients with less than 6 months of follow-up were excluded. The 118 remaining carriers were classified into two groups, depending on ALT values. Group 1 (normal ALT): included 98 carriers, 3 of them developed an active chronic hepatitis that was treated with interferon. A small CHC was diagnosed in another patient and it was resected. Group 2 (elevated ALT): included 20 carriers, only 9 of them agreed to biopsy and we found severe hepatic lesions in 4 of them. No group presented coinfection with HCV or HDV. No patient died. We conclude that the study and follow-up of asymptomatic HBsAg carriers permits an early diagnosis and treatment of the complications of this pathology (chronic hepatitis, CHC, etc); in our study, three patients developed chronic hepatitis, successfully treated with interferon, and one small size CHC was diagnosed in another patient. The study of relatives permits also detect subclinic liver disease and facilitates vaccination to prevention transmission of this infection."}, {"id": "pubmed23n0040_1913", "title": "The significance of HB antigenemy in apparently healthy persons in the clinic for liver diseases.", "score": 0.009708737864077669, "content": "The study tries to clarify the affliction of liver as a consequence of the permanent HB antigenemy in apparently healthy persons. The study proves beyond doubt that in the majority of the HB antigen carriers such histological changes of the liver can be found that can be attributed only to an infection by the hepatitis virus. The majority of the HB antigen carriers are suffering either from the chronic focal (58%) or from the diffuse (21%) persistent hepatitis. A smaller percentage is suffering from more dangerous hepatopathies (acute viral hepatitis 4,2%, hepatitis chron. aggressiva 4.2%, cirrhosis 1,4%). The kind of the illness can be determined with the histological examination only because of the absence of the clinical symptomatology and because of the liver function tests are in such cases frequently normal. Our investigations indicate that the diffuse and focal forms of persistent hepatitis can remain unchanged overlong periods and the same histological findings over a number of years. The chronic persistent hepatitis, however, may develop through clinically imperceptible changes into a chronic aggressive hepatitis, and the inapparent acute hepatitis can even pass over directly into cirrhosis. The identification of various forms of hepatitis, from light instances to the most severe cases, among the HB antigen carriers proves that the acute viral hepatitis of the type B may have in all the phases of its development a clinically asymptomatic course; it may even asymptomatically pass over into hepatopathies of the most severe kinds. The state of health of persons with HB antigenemy must be systematically followed up. For these reasons the histological examinations of the bioptic liver material that are made from time to time during the follow up of the illness have a decisive role."}, {"id": "article-22784_48", "title": "Hepatitis -- Evaluation -- Chronic Infection", "score": 0.009616273693943597, "content": "Evaluation of hepatitis B virus infection can be complicated, and some uncommon but possible scenarios should be kept in mind while investigating. Patients can test negative for HBsAg and anti-HBs but can have the presence of anti-HBc. This situation is possible when the result is false positive but can also happen in patients who are in the time window where they have cleared HBsAg from the blood, but anti-HBs has not yet appeared. Some patients who have cleared hepatitis B virus infection but have lost the anti-HBs over the years can test negative both for HBsAg and anti-HBs but positive for anti-HBc.  Also, patients infected with the hepatitis B virus many years ago can sometimes develop a core mutant variant of the hepatitis B virus where they can test negative for HBeAg and positive for anti-HBe even though the virus may still be active and is replicating. When lab findings like these are detected, the hepatitis B virus DNA PCR assay to check for viral replication is recommended."}, {"id": "pubmed23n0293_11196", "title": "[Basic and clinical aspects of hepatitis virus carriers].", "score": 0.009615384615384616, "content": "Among the six species of hepatitis viruses, HBV (hepatitis B virus) and HCV (hepatitis C virus) can induce persistent infection. HBV and HCV are transmitted parenterally, of which maternal transmission and transfusion-associated infection is a major route respectively. We opened the special clinic for carriers detected through blood donation, and followed them at regular intervals for their health care. The prevalence rate of HBV carriers decreased from 3.0% to 1.2% in these 10 years, and that of HCV decreased from 0.9 to 0.4% in these 4 years. Prevalence rate of HBV peaks at 50s and that of HCV peaks at 60s. Due to nearly complete screening of donated blood, post-transfusion hepatitis almost disappeared. HBV vaccine for neonates born from infected mothers reduced the new incidence of HBV carriers. In HBV carriers seroconversion of HBeAg to HBeAb occurs at teens with transient hepatitis and appearance of mutant virus. Ninety percent of the carriers remains healthy for the lifetime but some of them aggravate into chronic hepatitis leading to HCC (hepatocellular carcinoma). In HCV acute infection at adult age succeeds to chronic infection and eventually to liver cirrhosis with sporadic appearance of HCC. On the other hand, less than 50% of HCV carriers seem to be asymptomatic and do not lead to grave disease. In HBV carriers tendency to reject the virus occurs and eventually HBV is cleared in some percentage of the population. In contrast HCV does not tend to be cleared. HBsAb is a defensive antibody. In contrast HCVAb is not a defensive antibody but an infective antibody like HBcAb. DNA polymerase is a good marker of disease state in HBV, and HCV RNA is a good marker of HCV proliferation. Treatment with IFN is sometimes effective for seroconversion in HBV, and for eradication of virus in HCV."}, {"id": "pubmed23n0589_8432", "title": "[Polyclonal activation due to Epstein-Barr virus superinfection in a case with chronic hepatitis B].", "score": 0.009523809523809525, "content": "Primary infection with Epstein-Barr virus (EBV) often occurs subclinically during childhood, resulting in a latent infection of B lymphocytes. In this report, a chronic hepatitis B case who presented with a serologic profile mimicking acute hepatitis B virus (HBV) infection and exhibiting transient autoantibody positivities because of the polyclonal activation of B cells due to EBV reactivation has been presented. The test results of 56 years old male patient who suffered from fatigue and pain on the right upper quadrant, revealed high levels of liver enzymes (AST: 187 U/L, ALT: 569 U/L), positivity of HBsAg, anti-HBc IgG and anti-HBe, and negativity of anti-HBc IgM, HBeAg and anti-HBs. Since HBV-DNA level was found 405,974 copies/mL by quantitative real time polymerase chain reaction (PCR), the patient was taken into follow-up. At the 6th month AST and ALT levels further elevated (352 U/L and 609 U/L, respectively), and anti-HBc IgM and anti-HBs became positive in addition to the previous positive markers of HBV. With the suspicion of superinfection, further laboratory investigations yielded negative results in CMV-IgM and Paul Bunnel test, while positive results in EBV anti-VCA IgM and IgG, anti-EBNA IgM and IgG, anti-p22 IgM and IgG and anti-EA IgM. In the follow-up period high levels of autoantibody positivities [rheumatoid factor (42.200 U/ml), anti-nuclear antibody (1/100) and anti-Ro-52] together with increased levels of total IgG, IgM and IgA were detected. In the following months, the levels of transaminases, total immunoglobulins and HBV-DNA have distinctively decreased, and in the 20th month the previous HBV profile regained (HBsAg, anti-HBc IgG and anti-HBe positive, anti-HBc IgM and anti-HBs negative, HBV-DNA: 6984 copies/ml) and the other pathological test results returned to normal. As a result, ALT increases seen during the course of chronic hepatitis B should not always be considered as HBV manifestations and the unusual serologic patterns should be evaluated as a consequence of superinfection with various viral agents."}, {"id": "pubmed23n0228_15078", "title": "[Hepatitis B with a fatal outcome in a 3-month-old infant of a healthy chronic carrier mother].", "score": 0.009523809523809525, "content": "A 81 day old male infant developed an acute hepatitic failure and died shortly thereafter. Determinations of HBs antigen and antibody (AB) and HBeAg and AB were performed in the parents and sibling of the infected child. The mother and a sister were an asymptomatic carrier of HBsAg, the first HBeAg positive and the second HBeAB positive. An elder sibling was HBsAg and HBeAg positive in this serum. The father was anti-HBs positive. In the family of the sister, the man and two childs were HBsAg and anti-HBsAB negative. A new baby in the family of the propositus born and a combination of HB vaccine and HBIg (hepatite B immuno-globulin) was started at birth. Unfortunately the child died of S.D.I.S. (Sudden Death Infant Syndrome). The HB vaccine was immuno-genetic in this infant and the anti-HBs in the immuno-globulin M (IgM) was positive as in several adults controls. This case allow us to discuss vertical transmission of hepatitis B, the clinical aspect of neonatal hepatitis and the preventing HB infection by combinaison of HB vaccine and HBIg."}, {"id": "pubmed23n0061_3726", "title": "Hepatitis B virus infection in Ethiopian immigrants to Israel.", "score": 0.009433962264150943, "content": "Two groups of immigrants from Ethiopia, one of 86 and the other of 165 individuals, aged 0-40, were examined for hepatitis B virus (HBV) infection in 1987-88, 3-7 years after their arrival in Israel. The results were compared with those obtained in the same age-group among Ethiopians who immigrated to Israel in 1980-82. The immigrants were found to be in good physical condition, their liver function tests were normal and no clinical evidence of chronic liver disease was found. Of the 22 children aged 0-4, 16 had anti-HBs as a result of vaccination at birth against HBV and they were excluded from the comparative study. In the age-groups 5-40 there was no significant change in the percentage of individuals positive for HBsAg, anti-HBs or anti-HBc only, compared with the group examined in 1980-82. There were two significant findings in this study: a) In 1987-88 [corrected], 8-9% of HBsAg-positive individuals had HBeAg and 64-81% had anti-HBe, while in 1980-82, 36% of those positive for HBsAg had HBeAg and only 25% had anti-HBe. b) At the time of arrival recent infection by HBV was indicated by the presence of IgM anti-HBc in 57% of those positive for HBsAg and 21% in whom anti-HBc was the sole serological HBV marker. In 1987-88 no IgM anti-HBc was found in HBsAg-positive persons or in those with anti-HBc only. These results indicate that most HBV infections in this population had occurred before their arrival in Israel. There is a profound change in the epidemiology of HBV infection in this Ethiopian population following immigration, which is probably due to environmental changes as well as to vaccination against HBV of all young children aged less than or equal to 3 years."}, {"id": "pubmed23n0127_13849", "title": "[Natural history of the state of asymptomatic carrier of HBsAg: 7-year follow-up].", "score": 0.009433962264150943, "content": "Ninety-six chronic asymptomatic HBsAg carriers underwent liver biopsy. Liver histology was normal in 5 cases, showed nonspecific changes in 67, chronic persistent hepatitis in 18, and chronic-active hepatitis in 6. Seventy-four patients were followed for up to 105 months (mean 80 months) in order to evaluate the occurrence of clinical, biochemical, serological or histological changes. Only two patients cleared the HBsAg, respectively 10 and 96 months after undergoing liver biopsy; the latter patient became anti-HBs positive 6 months after he cleared HBsAg. All 10 patients who initially were negative for both HBeAg and anti-HBe became anti-HBe positive during follow-up. All 4 patients who were HBeAg positive at the time of liver biopsy cleared HBeAg 6 to 39 months thereafter. Two of them became anti-HBe positive. None of the patients initially HBeAg negative became positive for this antigen during follow-up. Significant increases of serum transaminases were observed in 5 patients; in one superinfection by delta agent was documented, the other 4 being constantly anti-delta negative. Three of the latter patients underwent repeat liver biopsy, which showed progression from minimal changes to chronic persistent hepatitis in one, and from minimal changes to chronic active hepatitis in another. In the third patient, repeat biopsy showed persistence of chronic persistent hepatitis. chronic hepatitis occurs in about 25% of chronic asymptomatic HBsAg carriers; clearance of HBsAg is a rare event among these patients; the HBe system has little diagnostic or prognostic value; delta superinfection is rare; however, deterioration of liver histology may occur even in the absence of delta superinfection."}, {"id": "InternalMed_Harrison_23525", "title": "InternalMed_Harrison", "score": 0.009347631423766573, "content": "Another important serologic marker in patients with hepatitis B is HBeAg. Its principal clinical usefulness is as an indicator of relative infectivity. Because HBeAg is invariably present during early acute hepatitis B, HBeAg testing is indicated primarily in chronic infection. In patients with hepatitis B surface antigenemia of unknown duration (e.g., blood donors found to be HBsAg-positive) testing for IgM anti-HBc may be useful to distinguish between acute or recent infection (IgM anti-HBc-positive) and chronic HBV infection (IgM antiHBc-negative, IgG anti-HBc-positive). A false-positive test for IgM anti-HBc may be encountered in patients with high-titer rheumatoid factor. Also, IgM anti-HBc may be reexpressed during acute reactivation of chronic hepatitis B."}, {"id": "pubmed23n0932_25379", "title": "Occult Hepatitis B Virus Infection and Associated Genotypes among HBsAg-negative Subjects in Burkina Faso.", "score": 0.009345794392523364, "content": "The presence of HBV DNA in the liver (with detectable or undetectable HBV DNA in the serum) of individuals tested HBsAg negative by currently available assays is defined occult B Infection (OBI). It remains a potential transmission threat and risk to HBV chronic infection. The purpose of this study was to determine the OBI prevalence among HBsAg negative subjects and to characterize associated genotypes. Blood samples of 219 HBsAg-negative subjects tested by ELISA were collected. HBV DNA was investigated in all samples. Viral loads were determined using quantitative real-time PCR. All samples were screened for HBV markers (anti-HBc, anti-HBe, HBsAg). The Pre-S/S region of the HBV genome was sequenced. The database was analyzed using the SPSS and Epi info software. Phylogenetic analysis was performed using the BioEdit and MEGA software. Of the 219 samples, 20.1% were anti-HBc positive, 1.8% HBeAg and 22.8% were anti-HBe positive. Fifty-six (56) (25.6%) of the samples had a detectable HBV DNA and viral loads ranging from 4 IU/mL to 13.6 10<sup6</sup IU/mL. Sixteen of them (16/56) had a viral load &lt; 200 IU/mL, resulting in an OBI prevalence of 7.3% (16/219) in our study. The remaining 40 subjects had viral loads &gt; 200 IU/mL, resulting in a \"false OBI\" prevalence of 18.3% (40/219). HBV genotype E was predominant followed by the quasi-sub-genotype A3. A single \"false OBI\" strain had the characteristic mutation G145R. Other mutations were observed and all located in the major hydrophilic region (MHR) of the S gene. The study reported a prevalence of 7.3% of occult hepatitis B infection. It confirms the predominance of genotype E and the existence of a subgroup of quasi-sub-genotype A3 of HBV in Burkina Faso. It further provides information on the presence of \"false OBI.\" This study has found mutations in the major hydrophilic region (MHR) of the pre-S/S gene of HBV."}, {"id": "pubmed23n0581_20382", "title": "[The follow-up of serum aminotransferase levels and investigation of hepatitis B virus load in inactive HBsAg carriers].", "score": 0.009259259259259259, "content": "The aim of this study was to follow-up the serum alanine aminotransferase (ALT) levels in inactive HBsAg carriers during one year period and investigate the association between hepatitis B virus (HBV) DNA levels detected at the end of the year. At May 2005, 61 patients with HBeAg negative/anti-HBe positive chronic HBV infection, followed in our viral hepatitis clinic were included to the study. The patients' ultrasonographic examination of the liver were normal, they had no history of taking alcohol or routine medication, were anti-HCV seronegative and had normal ALT levels during the last 6 months and at the beginning of the study. Serum ALT levels of patients were followed in the 3rd, 6th and 12th months of the study, and blood HBV-DNA levels were analysed quantitatively in 12th month. During the one year period 89% (54/61) of the patients yielded continously normal ALT levels, while 11% (7/61) showed at least one ALT value above the normal levels (ALT &gt; 1.2x). Total HBV-DNA positivity rate was found as 59% (36/61). In inactive HBsAg carrier group,--namely HBeAg negative and serum ALT levels constantly normal--57.4% (31/54) of patients were HBV-DNA positive and 23 (42.6%) were negative. Amongst the HBV-DNA positive patients the viral load were detected as 10(4)-10(5) copies/ml in six (19.4%), and &lt;10(4) copies/ml in 25 (80.6%) patients. In patients who had at least one ALT value above normal limits, 71.4% (5/7) of them were found HBV-DNA positive; two with HBV-DNA values of &gt;10(5) copies/ml and three with values between 10(4)-10(5) copies/ml. In conclusion, although ALT levels may be normal, it should always be taken into consideration that more than half of inactive HBsAg carriers exhibited low level viral replication, thus HBV-DNA and liver enzyme levels should be monitored routinely in order not to miss the acute manifestations."}, {"id": "InternalMed_Harrison_23421", "title": "InternalMed_Harrison", "score": 0.009240290226205719, "content": "well as those with chronic HBV infection, anti-HBc is predominantly of the IgG class. Infrequently, in ≤1–5% of patients with acute HBV infection, levels of HBsAg are too low to be detected; in such cases, the presence of IgM anti-HBc establishes the diagnosis of acute hepatitis B. When isolated anti-HBc occurs in the rare patient with chronic hepatitis B whose HBsAg level is below the sensitivity threshold of contemporary immunoassays (a low-level carrier), anti-HBc is of the IgG class. Generally, in persons who have recovered from hepatitis B, anti-HBs and anti-HBc persist indefinitely."}, {"id": "article-22784_47", "title": "Hepatitis -- Evaluation -- Chronic Infection", "score": 0.009174504976537677, "content": "Patients who have chronic hepatitis B infection can have positive HBsAg for life.  These patients can be inactive carriers of the hepatitis B virus or may have active chronic hepatitis. All patients with chronic hepatitis B virus infection have the presence of anti-HBc. If HBeAg may or may not be present, but if it present in patients with active chronic hepatitis, it can indicate viral replication. Similarly, hepatitis B virus DNA may or may not be present, but high levels indicate active chronic hepatitis. Patients with chronic infection of hepatitis B usually have an absence of anti-HBs, but the presence of anti-HBs with positive HBsAg in patients with chronic infection with the hepatitis B virus means that the antibody was unsuccessful in inducing the viral clearance."}, {"id": "pubmed23n0049_12776", "title": "[Asymptomatic HBs antigenemia in pregnant women].", "score": 0.009174311926605505, "content": "The authors have dispensarized for more than ten years HBsAg carriers at the Second Medical Clinic of the Olomouc Faculty Hospital when antigenaemia was detected during blood donorship. Since 1990 the authors dispensarize also women where HBs antigenaemia was revealed during pregnancy. In the presented paper the authors analyze this group of women. In 1990-1991 the authors examined blood samples for HBsAg from 12,042 symptom-free pregnant women. HBsAg positivity was proved in 34 women (0.28%). Eleven women (32.3%) are regularly followed up since delivery. The authors know nothing about the fate of 10 women (29.4%), as they did not respond to repeated invitations to the surgery. It must be however mentioned that 6 of them are Vietnamese and probably left Czechoslovakia. Four women (11.7%) did not attend the clinic for examination but the authors know that they were delivered of their babies elsewhere. In the remainder, i.e. 8 women the delivery was normal, usually in term. The neonates were passively as well as actively immunized at the appropriate dates. Their umbilical blood (if collected) was HBsAg positive, while the venous blood was in all instances negative. All 20 women who attended examination were throughly examined (ultrasound, HBeAg, HBeAb etc.) and the clinical picture is that of a \"healthy\" HBsAg carrier (not verified by histological examination)."}, {"id": "Pathology_Robbins_3888", "title": "Pathology_Robbins", "score": 0.0091354677234331, "content": "Anti-HBs antibody appears after the acute disease is over and usually is not detected until a few weeks to several months after HBsAg disappears. Anti-HBs may persist for life and confers protection, which is the rationale for current vaccines containing HBsAg. HBeAg and HBV DNA appear in serum soon after HBsAg and signify ongoing viral replication. Persistence of HBeAg is an indicator of progression to chronic hepatitis. The appearance of anti-HBe antibodies implies that an acute infection has peaked and is on the wane. IgM anti-HBc becomes detectable in serum shortly before the onset of symptoms, concurrent with the onset of elevated serum aminotransferase levels (indicative of hepatocyte destruction). Over a period of months, the IgM anti-HBc antibody is replaced by IgG anti-HBc. As in the case of anti-HAV, there is no direct assay for IgG anti-HBc; its presence is inferred from decline of IgM anti-HBc in the face of rising total anti-HBc."}, {"id": "wiki20220301en089_27823", "title": "Jade Ribbon Campaign", "score": 0.009133367929423975, "content": "Reasons for lack of diagnosis The danger of hepatitis B lies in its silent transmission and progression. Many chronic hepatitis B carriers are asymptomatic (have no symptoms) and feel perfectly healthy. Chronically infected individuals may exhibit normal blood tests for liver function and be granted a clean bill of health. The diagnosis cannot be made without a specific blood test for the presence of the hepatitis B surface antigen (HBsAg), a marker for chronic infection. Since the detection of hepatitis B is so easily missed, even by doctors, it is also up to the patient to specifically request the HBsAg test. Early detection not only benefits the person tested, but prevents infection from being passed silently from one child to another, and from one generation to another."}, {"id": "pubmed23n0238_1631", "title": "[Seven-year follow-up studies on asymptomatic HBs Ag carriers].", "score": 0.00909090909090909, "content": "This report covers 7-year follow-up studies on 35 HBs Ag carriers whose anti-HBc titer levels were 10 (log 2) or more and who had normal liver functions at the start of the studies, when 9 of them (25.7%) were HBe Ag positive, 24 (68.6%) anti-HBe positive and 2 (5.7%) negative to both. 1. There was no significant difference in the incidence of abnormal SGPT levels as a whole between HBe Ag positives and anti-HBe positives. But abnormally high SGPT levels of 100 KU or more were observed at a higher percentage among HBe Ag positives (4/9. 44.4%) than among anti-HBe positives (1/24. 4.2%. p less than 0.02). 2. Based on the results of the 7-year studies, all cases were classified into six clinical stages. HBe Ag positives were divided into three groups by the stage with different SGPT levels: 5 cases (15.2%) whose SGPT leves never rose above 50 KU were classified as Stage 1; 3 cases with chronic active hepatitis (9.6%) whose highest SGPT levels were over 200 KU (2 new cases and 1 case with a relapse of chronic inactive hepatitis) as Stage 2 and 1 case (3.0%) seroconverted to anti-HBe positive following an acute relapse of chronic inactive hepatitis as Stage 3. Anti-HBe positives were divided into another three groups similarly according to their mean HBs Ag titer levels: 6 cases (18.2%) whose mean HBs Ag titer levels ranged from 10 to 13 (log 2) were classified as Stage 4; 13 cases (39.4%) whose mean HBs Ag titer levels ranged from 6 to 9 as Stage 5 and 5 cases (15.2%), including 2 cases turned negative to HBs Ag, whose mean HBs Ag titer levels were below 5 as Stage 6. The average age of each group increased wth its clinical stage, namely, 32.6 in Stage 1, 34.3 in Stage 2, 33.0 in Stage 3, 34.5 in Stage 4 and 37.0 in Stage 5, but the average age in Stage 6 was 29.0. 3. All HBe Ag positives showed fluctuations in HBs Ag titer levels. The fluctuations were particularly noteworthy among cases with chronic active hepatitis in Stage 2 during an acute relapse. The HBs Ag titers rose just before the acute relapses in a case of chronic active hepatitis when SGPT levels went over 200 KU. This suggested a proliferation of the virus. On the other hand, in anti-HBe positives, a decrement of the virus was suggested by the fact that an increasing proportion of cases showed their HBs Ag titer levels to fluctuate or to become lower with the progress of stages (p less than 0.05) and two cases turned negative to HBs Ag as plotted in Stage 6. And the proportion of cases with abnormal SGPT levels decreased with the progress of stages (p less than 0.05). One case whose SGPT level was 125 KU, highest among anti-HBe positives, followed the clinical course of chronic inactive hepatitis and lowered in HBs Ag titer. 4. Between HBe Ag and anti-HBe cases, there were considerable differences in the occurrence of liver disturbances and their clinical courses..."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[0, 182]], "word_ranges": [[0, 25]], "text": "Refeeding syndrome occurs in patients with previous malnutrition exposed to either oral, enteral or parenteral nutritional therapy. All responses are causes of malnutrition except 3."}, "2": {"exist": true, "char_ranges": [[0, 182]], "word_ranges": [[0, 25]], "text": "Refeeding syndrome occurs in patients with previous malnutrition exposed to either oral, enteral or parenteral nutritional therapy. All responses are causes of malnutrition except 3."}, "3": {"exist": true, "char_ranges": [[0, 182]], "word_ranges": [[0, 25]], "text": "Refeeding syndrome occurs in patients with previous malnutrition exposed to either oral, enteral or parenteral nutritional therapy. All responses are causes of malnutrition except 3."}, "4": {"exist": true, "char_ranges": [[0, 182]], "word_ranges": [[0, 25]], "text": "Refeeding syndrome occurs in patients with previous malnutrition exposed to either oral, enteral or parenteral nutritional therapy. All responses are causes of malnutrition except 3."}, "5": {"exist": true, "char_ranges": [[0, 182]], "word_ranges": [[0, 25]], "text": "Refeeding syndrome occurs in patients with previous malnutrition exposed to either oral, enteral or parenteral nutritional therapy. All responses are causes of malnutrition except 3."}}, "full_answer": "Refeeding syndrome occurs in patients with previous malnutrition exposed to either oral, enteral or parenteral nutritional therapy. All responses are causes of malnutrition except 3.", "full_answer_no_ref": "Refeeding syndrome occurs in patients with previous malnutrition exposed to either oral, enteral or parenteral nutritional therapy. All responses are causes of malnutrition except [HIDDEN].", "full_question": "A patient on enteral nutritional support presents 72 hours after starting enteral nutrition with a CBC showing hypophosphoremia and hypokalemia, with clinical signs of heart failure. The patient is diagnosed with refeeding syndrome. Indicate which of the following is NOT considered a risk factor for a patient presenting with this condition:", "id": 183, "lang": "en", "options": {"1": "Previous caloric malnutrition.", "2": "Anorexia nervosa.", "3": "Non-morbid obesity.", "4": "Elderly.", "5": "Prolonged vomiting and diarrhea."}, "question_id_specific": 64, "type": "ENDOCRINOLOGY", "year": 2013, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0492_627", "title": "Refeeding syndrome in patients with gastrointestinal fistula.", "score": 0.019327731092436976, "content": "Although refeeding syndrome has been well documented in starved patients, obese patients, those with anorexia nervosa, malnourished elderly individuals, and certain postoperative patients, little is known about the presence and the importance of refeeding syndrome in patients with gastrointestinal fistula and insufficient nutrition support over the long term. The objective of this study was to estimate the morbidity of this syndrome in these patients, to assess the safety and efficacy of our graduated refeeding regimen, and to emphasize the importance of this syndrome. One hundred fifty-eight patients with gastrointestinal fistula during the past 2 y were reviewed. Fifteen of these patients were diagnosed as having refeeding syndrome. They were started on the refeeding procedure according to our regimen, and changes in their serum levels of electrolytes were recorded. The symptoms and signs they presented were noted. All patients were successfully advanced to full nutrition support. During the refeeding procedure, patients presented with weakness, paralysis of limbs, slight dyspnea, paresthesia, tachycardia, edema, and diarrhea. Serum phosphorus concentration decreased in all patients within 24 h of refeeding, reaching a mean nadir after 3.3 +/- 1.5 d and another 6.1 +/- 2.1 d to return to above 0.70 mM/L upon phosphorus supplementation. Three patients treated with growth hormone presented more severe hypophosphatemia (&lt;0.20 mM/L) than the others. 1) Refeeding syndrome occurs commonly in patients with malnutrition secondary to gastrointestinal fistula. 2) Alterations in phosphate metabolism are central to the refeeding syndrome. 3) Supplementation with electrolytes (including especially phosphate) and vitamins is the focal point of the treatment of this syndrome. 4) Growth hormone treatment may aggravate hypophosphatemia."}, {"id": "pubmed23n0318_5663", "title": "[Malnutrition and total parenteral nutrition: a cohort study to determine the incidence of refeeding syndrome].", "score": 0.01896117900790798, "content": "The Refeeding Syndrome is conformed by a series of clinical manifestations related to electrolytic alterations associated with the restarting of the nutritive contribution both enteral and parenteral. To detect the Refeeding Syndrome incidence in malnourished patients who required nutritional, enteral or endovenous support and its relationship with mortality. A cohort study was performed in the service of Nutritional Support of the IMSS (Social Security Mexican Institute) Specialties Hospital CMN León, from June 1995 to May 1996. All patients with mild and severe malnutrition were included, they received endovenous or enteral nutritious support for more than 7 days, without presenting previous electrolytic unbalance. Serum potassium, phosphorous, and magnesium levels were determined before starting the nutritious support and also on the 3rd, 7th, and 10th days. Descriptive statistics, Student's t and Z test were used, with a 5% significance level. 148 patients with total nutritional support, 23 (16%) of them with restrained malnutrition and 65 (44%) with severe deficit. 54 men and 34 women with an average age of 51.6 +/- 19.4 years. Nineteen patients were eliminated due to a nutrition period of less than 7 days, and other 19 were also eliminated for presenting electrolytic alterations before the nutritive support started. An incidence of 48% of electrolytic alterations compatible with the refeeding syndrome was the result in the remaining 50 patients. The alterations were: hypomagnesemia 13/24, hypokalemia 12/24 and hypophosphatemia 4/24; in 55% of the cases the syndrome appeared at the third day of administration. Hospital sojourn of patients with the syndrome was 26.7 +/- 18 days vs 15.3 +/- 7 (p &lt; 0.05) of those who did not present it. 15 patients died, 5 of them had electrolytic alterations before nutrition, 7 (29%) with refeeding syndrome and 3 (12%) did not presented it (p = 0.059). Refeeding Syndrome is a frequent entity in malnourished patients submitted to enteral or parenteral nutrition; at least in this study it was of 48%; its presence was followed by a longer hospital stay and a higher mortality rate."}, {"id": "pubmed23n0747_7325", "title": "Occurrence of refeeding syndrome in adults started on artificial nutrition support: prospective cohort study.", "score": 0.01838731443994602, "content": "Refeeding syndrome is a potentially life-threatening condition characterised by severe intracellular electrolyte shifts, acute circulatory fluid overload and organ failure. The initial symptoms are non-specific but early clinical features are severely low-serum electrolyte concentrations of potassium, phosphate or magnesium. Risk factors for the syndrome include starvation, chronic alcoholism, anorexia nervosa and surgical interventions that require lengthy periods of fasting. The causes of the refeeding syndrome are excess or unbalanced enteral, parenteral or oral nutritional intake. Prevention of the syndrome includes identification of individuals at risk, controlled hypocaloric nutritional intake and supplementary electrolyte replacement. To determine the occurrence of refeeding syndrome in adults commenced on artificial nutrition support. Prospective cohort study. Large, single site university teaching hospital. Recruitment period 2007-2009. 243 adults started on artificial nutrition support for the first time during that admission recruited from wards and intensive care.  occurrence of the refeeding syndrome. Secondary outcome: analysis of the risk factors which predict the refeeding syndrome. Tertiary outcome: mortality due to refeeding syndrome and all-cause mortality. 133 participants had one or more of the following risk factors: body mass index &lt;16-18.5≥(kg/m(2)), unintentional weight loss &gt;15% in the preceding 3-6 months, very little or no nutritional intake &gt;10 days, history of alcohol or drug abuse and low baseline levels of serum potassium, phosphate or magnesium prior to recruitment. Poor nutritional intake for more than 10 days, weight loss &gt;15% prior to recruitment and low-serum magnesium level at baseline predicted the refeeding syndrome with a sensitivity of 66.7%: specificity was &gt;80% apart from weight loss of &gt;15% which was 59.1%. Baseline low-serum magnesium was an independent predictor of the refeeding syndrome (p=0.021). Three participants (2% 3/243) developed severe electrolyte shifts, acute circulatory fluid overload and disturbance to organ function following artificial nutrition support and were diagnosed with refeeding syndrome. There were no deaths attributable to the refeeding syndrome, but (5.3% 13/243) participants died during the feeding period and (28% 68/243) died during hospital admission. Death of these participants was due to cerebrovascular accident, traumatic injury, respiratory failure, organ failure or end-of-life causes. Refeeding syndrome was a rare, survivable phenomenon that occurred during hypocaloric nutrition support in participants identified at risk. Independent predictors for refeeding syndrome were starvation and baseline low-serum magnesium concentration. Intravenous carbohydrate infusion prior to artificial nutrition support may have precipitated the onset of the syndrome."}, {"id": "pubmed23n1149_2546", "title": "Delayed appearance of refeeding syndrome in a patient with anorexia nervosa: A case report.", "score": 0.017577673271143318, "content": "Refeeding syndrome (RFS) can be a severe and life-threatening complication of anorexia nervosa (AN) associated with electrolyte abnormalities and organ damage, and occurs with the transition from a prolonged catabolic to anabolic state, particularly with an overzealous nutrient supply. There is no unequivocal definition of RFS, although hypophosphatemia is recognized as a crucial factor in its pathogenesis. RFS can be responsible for cardiovascular complications, such as heart failure, left ventricular damage, and arrhythmias, because of different potential mechanisms: electrolyte imbalances, increased retention of sodium and liquids secondary to insulin secretion, and excessive fat emulsion supplementation. We report on the case of a 13-y-old male patient with severe AN in whom a delayed and reversible myocardial dysfunction was documented during cautious nutritional replenishment, even in the absence of serum electrolyte imbalances. Seven days after the inception of integrative enteral nutrition, heart failure was unexpectedly documented as follows: reduction in fraction ejection, presence of mild bilateral perimalleolar edema, and increased n-terminal prohormone of brain natriuretic peptide. A more pronounced water restriction protocol and delayed achievement of goal feeding rate, resulting also in lower sodium intake, were implemented to reduce cardiac overload with a full resolution of the complication in approximately 2 mo. Refeeding patients with AN could be complicated by heart failure despite cautious nutritional replenishment and regardless of electrolyte imbalance, even in a later phase of recovery. Therefore, strict adherence to recommendations for nutritional replenishment and close monitoring of cardiac function should always be considered when refeeding patients with AN."}, {"id": "wiki20220301en110_39302", "title": "Refeeding syndrome", "score": 0.017261904761904763, "content": "Clinical situations The syndrome can occur at the beginning of treatment for anorexia nervosa when patients have an increase in calorie intake and can be fatal. It can also occur after the onset of a severe illness or major surgery. The shifting of electrolytes and fluid balance increases cardiac workload and heart rate. This can lead to acute heart failure. Oxygen consumption is increased which strains the respiratory system and can make weaning from ventilation more difficult. Diagnosis Refeeding syndrome can be fatal if not recognized and treated properly. An awareness of the condition and a high index of suspicion are required in order to make the diagnosis. The electrolyte disturbances of the refeeding syndrome can occur within the first few days of refeeding. Close monitoring of blood biochemistry is therefore necessary in the early refeeding period."}, {"id": "pubmed23n0757_16129", "title": "Metabolic and nutritional needs to normalize body mass index by doubling the admission body weight in severe anorexia nervosa.", "score": 0.016964924838940586, "content": "Anorexia nervosa exhibits one of the highest death rates among psychiatric patients and a relevant fraction of it is derived from undernutrition. Nutritional and medical treatment of extreme undernutrition present two very complex and conflicting tasks: (1) to avoid \"refeeding syndrome\" caused by a too fast correction of malnutrition; and (2) to avoid \"underfeeding\" caused by a too cautious refeeding. To obtain optimal treatment results, the caloric intake should be planned starting with indirect calorimetry measurements and electrolyte abnormalities accurately controlled and treated. This article reports the case of an anorexia nervosa young female affected by extreme undernutrition (BMI 9.6 kg/m(2)) who doubled her admission body weight (from 22.5 kg to 44 kg) in a reasonable time with the use of enteral tube feeding for gradual correction of undernutrition. Refeeding syndrome was avoided through a specialized and flexible program according to clinical, laboratory, and physiological findings."}, {"id": "pubmed23n0596_8104", "title": "Death resulting from overzealous total parenteral nutrition: the refeeding syndrome revisited.", "score": 0.01675099138520443, "content": "Commentary is provided on the pivotal paper by Weinsier and Krumdieck from 1981 describing 2 patients who developed profound and fatal refeeding syndrome following initiation of aggressive total parenteral nutrition. This classic description was among the first to describe the overwhelming cardiovascular and pulmonary manifestations that can accompany parenteral refeeding with carbohydrate in chronically malnourished patients. The syndrome has also been described with oral and enteral nutrition. One of the hallmarks of the syndrome is hypophosphatemia. Since 1981, dosing schemes for addressing hypophosphatemia have been refined. Other manifestations of the syndrome include other electrolyte abnormalities such as hypokalemia and hypomagnesemia, hyperglycemia, fluid and sodium retention, and neurologic and hematologic complications. Case reports of refeeding syndrome continue to be published, particularly in the anorexia nervosa population. Stressed, critically ill patients may be at risk of refeeding following short periods of fasting; hypophosphatemia is commonly encountered in this situation. It behooves the current nutrition support practitioner to keep in mind the types of patients at risk of refeeding syndrome and to approach refeeding of such patients with caution and careful monitoring."}, {"id": "pubmed23n0740_25169", "title": "Enteral nutrition for feeding severely underfed patients with anorexia nervosa.", "score": 0.016242346116661296, "content": "Severe undernutrition nearly always leads to marked changes in body spaces (e.g., alterations of intra-extracellular water) and in body masses and composition (e.g., overall and compartmental stores of phosphate, potassium, and magnesium). In patients with severe undernutrition it is almost always necessary to use oral nutrition support and/or artificial nutrition, besides ordinary food; enteral nutrition should be a preferred route of feeding if there is a functional accessible gastrointestinal tract. Refeeding of severely malnourished patients represents two very complex and conflicting tasks: (1) to avoid \"refeeding syndrome\" caused by a too fast correction of malnutrition; (2) to avoid \"underfeeding\" caused by a too cautious rate of refeeding. The aim of this paper is to discuss the modality of refeeding severely underfed patients and to present our experience with the use of enteral tube feeding for gradual correction of very severe undernutrition whilst avoiding refeeding syndrome, in 10 patients aged 22 ± 11.4 years and with mean initial body mass index (BMI) of 11.2 ± 0.7 kg/m(2). The mean BMI increased from 11.2 ± 0.7 kg/m(2) to 17.3 ± 1.6 kg/m(2) and the mean body weight from 27.9 ± 3.3 to 43.0 ± 5.7 kg after 90 days of intensive in-patient treatment (p &lt; 0.0001). Caloric intake levels were established after measuring resting energy expenditure by indirect calorimetry, and nutritional support was performed with enteral feeding. Vitamins, phosphate, and potassium supplements were administered during refeeding. All patients achieved a significant modification of BMI; none developed refeeding syndrome. In conclusion, our findings show that, even in cases of extreme undernutrition, enteral feeding may be a well-tolerated way of feeding."}, {"id": "pubmed23n1053_3502", "title": "Extremely severe anorexia nervosa: Hospital course of 354 adult patients in a clinical nutrition-eating disorders-unit.", "score": 0.01602809706257982, "content": "The clinical nutrition-eating disorders-unit in Raymond Poincaré Hospital is a reference center for the management of severe malnutrition and its complications in patients with anorexia nervosa (AN). The purpose of this study is to specify socio-demographic, anamnesic and clinical characteristics of AN patients hospitalized for extreme malnutrition, to identify types and prevalence of medical complications presented during their hospitalization for refeeding and the evolution of patients nutritional status. Demographic, clinical and paraclinical data of 354 severely malnourished AN patients were collected, during their first hospitalization in the unit, between November 1997 and January 2014, through medical records. The prevalence of medical complications was compared between the 2 AN subtypes (restricting and binging-purging). 339 patients were female and mean age was 28.7 ± 10.7 years old. Duration of AN was 9.5 ± 9 years, 173 (48.9%) patients had a restricting AN subtype. BMI at admission was 12.2 ± 1.6 kg/m<sup2</sup, 280 (79.3%) patients had already been hospitalized for AN in other hospitals before. Psychiatric comorbidities were present in 168 (47.5%) patients. Associated somatic comorbidities concerned 70 (19.8%) patients. Outcomes during hospitalization were marked by 4.1 ± 3.9 kg weight gain on 36.9 ± 30.5 days. Enteral nutrition was provided in 304 (85.9%) patients. Main medical complications during hospitalization were: anemia (79%), neutropenia (53.9%), hypertransaminasemia (53.7%), osteoporosis (46.3%), hypokalemia (39.5%), hypophosphatemia (26%), hypoglycemia (13.8%), infectious complications (24.3%), cardiac dysfunction (7.1%), and proven gelatinous bone marrow transformation (6.5%). Hypokalemia was more frequent in binging-purging subtype. Lympho-neutropenia and hypertransaminasemia were more frequent in restricting subtype. During their hospitalization, 35 (10%) patients were referred to medical intensive care unit and 5 patients died. AN patients hospitalized for severe malnutrition in a specialized clinical nutrition unit have severe and frequent medical complications. Psychiatric comorbidities are also frequent and could complicate medical care. A specialized and multidisciplinary management of these patients is therefore essential."}, {"id": "pubmed23n0790_6269", "title": "Safe refeeding management of anorexia nervosa inpatients: an evidence-based protocol.", "score": 0.015705838876570587, "content": "Anorexia nervosa is associated with several serious medical complications related to malnutrition, severe weight loss, and low levels of micronutrients. The refeeding phase of these high-risk patients bears a further threat to health and potentially fatal complications. The objective of this study was to examine complications due to refeeding of patients with anorexia nervosa, as well as their mortality rate after the implementation of guidelines from the European Society of Clinical Nutrition and Metabolism. We analyzed retrospective, observational data of a consecutive, unselected anorexia nervosa cohort during a 5-y period. The sample consisted of 65 inpatients, 14 were admitted more than once within the study period, resulting in 86 analyzed cases. Minor complications associated with refeeding during the first 10 d (replenishing phase) were recorded in nine cases (10.5%), four with transient pretibial edemas and three with organ dysfunction. In two cases, a severe hypokalemia occurred. During the observational phase of 30 d, 16 minor complications occurred in 14 cases (16.3%). Six infectious and 10 non-infectious complications occurred. None of the patients with anorexia nervosa died within a follow-up period of 3 mo. Our data demonstrate that the seriousness and rate of complications during the replenishment phase in this high-risk population can be kept to a minimum. The findings indicate that evidence-based refeeding regimens, such as our guidelines are able to reduce complications and prevent mortality. Despite anorexia nervosa, our sample were affected by serious comorbidities, no case met the full diagnostic criteria for refeeding syndrome."}, {"id": "wiki20220301en110_39303", "title": "Refeeding syndrome", "score": 0.015406162464985995, "content": "Treatment In critically ill patients admitted to an intensive care unit, if phosphate drops to below 0.65 mmol/L (2.0 mg/dL) from a previously normal level within three days of starting enteral or parenteral nutrition, caloric intake should be reduced to 480 kcals per day for at least two days while electrolytes are replaced. Daily doses of thiamine, vitamin B complex (strong) and a multivitamin and mineral preparation are strongly recommended. Blood biochemistry should be monitored regularly until it is stable. Although clinical trials are lacking in patients other than those admitted to intensive care, it is commonly recommended that energy intake should remain lower than that normally required for the first 3–5 days of treatment of refeeding syndrome for all patients."}, {"id": "pubmed23n0776_22651", "title": "Hypokalemia during the early phase of refeeding in patients with cancer.", "score": 0.015199637023593466, "content": "Refeeding syndrome occurs in patients with severe malnutrition when refeeding begins after a long period of starvation. This syndrome increases the risk of clinical complications and mortality. Hypophosphatemia is considered the primary characteristic of the syndrome. The aim of our study was to investigate the presence of other electrolyte alterations in patients with cancer during the early stage of refeeding. In this observational study, we enrolled 34 patients with cancer of the upper aerodigestive tract receiving upfront radiotherapy who were also enrolled in a nutrition program. A caloric intake assessment, anthropometric measurements and biochemical laboratory tests were performed. Significant weight loss (∼20%) was found in these patients. In the patients receiving artificial nutrition, we found lower levels of potassium and total protein compared with those who were fed orally (p = 0.03 for potassium and 0.02 for protein, respectively). Patients on enteral tube feeding had a higher caloric intake compared with those who were fed orally (25±5 kcal/kg/day vs. 10±2 kcal/kg/day). Hypokalemia, like hypophosphatemia, could be a complication associated with refeeding in patients with cancer. Hypokalemia was present in the early stages of high-calorie refeeding."}, {"id": "pubmed23n0595_8556", "title": "Refeeding syndrome: a potentially fatal condition but remains underdiagnosed and undertreated.", "score": 0.015189158667419538, "content": "To describe two cases of successfully prevented refeeding syndrome in a high-risk group of patients. Case 1 was a 70-y-old woman who presented with a 4-mo history of poor dietary intake and ill health due to a connective tissue disease leading to myositis and dysphagia and complicated by respiratory failure needing mechanical ventilation. Twelve hours after starting nasogastric tube feeding, she developed a cardiac arrest from which she was successfully resuscitated. Repeated attempts to wean her from the ventilator failed. Case 2 was a 15-y-old girl who was readmitted after a total colectomy for severe ulcerative colitis with diarrhea and vomiting leading to significant weight loss. Her body mass index was 11.4 kg/m(2). In case 1, after consultation by the clinical nutrition team, the diagnosis of refeeding syndrome was made and the patient was duly started on a high-protein, high-fat, low-carbohydrate diet, multivitamin and trace-element supplements, and electrolyte infusion. Subsequently she was successfully weaned from the ventilator. In case 2, further investigation by the clinical nutrition team revealed low baseline electrolyte concentrations including potassium, magnesium, calcium, and phosphate and low serum albumin. Her low body mass index and baseline electrolyte concentrations put her at high risk of developing refeeding syndrome. She was initially started on low-calorie feeding, multivitamin and minerals, and her electrolytes were carefully monitored. She made a good recovery. Refeeding syndrome is a life-threatening, underdiagnosed, treatable condition but there is a need for a wider awareness of the condition among health professionals."}, {"id": "pubmed23n0725_16590", "title": "Refeeding syndrome: clinical and nutritional relevance.", "score": 0.015141242937853107, "content": "Feedback syndrome is characterized clinically by neurological alterations, respiratory symptoms, arrhythmias and heart failure few days after refeeding. It happens due to severe electrolyte changes, such as hypophosphatemia, hypomagnesemia and hypokalemia associated with metabolic abnormalities that may occur as a result of nutritional support (oral, enteral or parenteral) in severely malnourished patients. To evaluate its causes and the preventive dietary measures aiming to reduce the morbimortality. Was conducted literature review in SciELO, LILACS, Medline / PUBMED, Cochrane Library and government websites in Portuguese, English and Spanish. The survey was about the last 15 years, selecting the headings: refeeding syndrome, malnutrition, hypophosphatemia, hypokalemia, hypomagnesemia. The monitoring of metabolic parameters and electrolyte levels before starting nutritional support and periodically during feeding should be based on protocols and the duration of therapy. Patients at high risk and other metabolic complications should be followed closely, and depletion of minerals and electrolytes should be replaced before starting the diet. A multidisciplinary team of nutrition therapy can guide and educate other health professionals in prevention, diagnosis and treatment of the syndrome."}, {"id": "pubmed23n0289_16947", "title": "[The incidence of the refeeding syndrome in cancer patients who receive artificial nutritional treatment].", "score": 0.0150650789255054, "content": "We determine the incidence of the malnutrition syndrome and its relation with probable risk factors, in 106 patients, with a mean age of 53.3 +/- 15.4 years, with a diagnosis of cancer confirmed histologically, and who received artificial nutrition either enterally or endovenously, during an average of 16.8 +/- 2 days, which included a supply of 60 to 100 mmol/day of phosphorus. We considered there to be a renutrition syndrome when there as hypophosphatemia; &lt; 2.5 mmol/l, which took place during the nutritional treatment phase, and previous to which, the patients had normal serum levels of phosphorus. The serum electrolyte concentrations were measured prior to the start of the treatment, and daily during the first week, and later every 3 days until the end. The study variables were: age, sex, type of cancer, degree of malnutrition, degree of hypophosphatemia, day on which in occurred, and clinical manifestations associated to this. The relative risk was calculated for the variables of age, sex, malnutrition and cancer. The incidence of the renutrition syndrome was 24.5%; it was more frequent in the enteral group than in the endovenous group (37.5% vs. 18.9%, p &lt; 0.005); and it took place 72 hours after starting the nutritional support, in 61.5% of the cases, with a mean phosphorus concentrations of 1.9 mmol/l; the most frequent clinical manifestations were the neuromuscular ones (30%), and the most frequent type of cancer was lymphoma (15.4%). The risk factors were age greater than 60 years (RR = 1.7), and moderate or severe malnutrition 8RR = 2.0). We conclude that the prevalence of the renutrition syndrome is high in the cancer patients, despite an intense preventive treatment with phosphorus."}, {"id": "pubmed23n0378_16809", "title": "Life-threatening refeeding syndrome in a severely malnourished anorexia nervosa patient.", "score": 0.014772727272727272, "content": "Overzealous refeeding in chronically malnourished anorexia nervosa patients may cause life-threatening complications. We describe a 14-year-old girl with anorexia nervosa who had a decrease in body weight from 45 kg to 25.5 kg over an 18-month period. She received 40 kcal.kg-1.d-1 carbohydrate-rich nutrition via enteral and parenteral routes. Her serum phosphate concentration dropped from a baseline of 1.39 mmol/L (4.3 mg/dL) to 0.19 mmol/L (0.6 mg/dL) on Day 4 of refeeding. Concurrent with the development of hypophosphatemia, she became drowsy and developed generalized muscle weakness, impaired myocardial contractility, thrombocytopenia, and gastrointestinal bleeding. Fluid overload with pulmonary edema complicated her recovery from these adverse events. After intravenous phosphate supplementation and fluid restriction, the symptoms of refeeding syndrome gradually resolved within 2 weeks. In chronically malnourished anorexia nervosa patients, nutritional support should be instituted gradually to avoid rapid electrolyte shifts and fluid overload. Serum phosphate concentrations, fluid status, and blood cell counts should be closely monitored."}, {"id": "wiki20220301en002_33526", "title": "Anorexia (symptom)", "score": 0.014425876976837955, "content": "Sudden cardiac death Anorexia is a relatively common condition that can lead patients to have dangerous electrolyte imbalances, leading to acquired long QT syndrome which can result in sudden cardiac death. This can develop over a prolonged period of time, and the risk is further heightened when feeding resumes after a period of abstaining from consumption. Refeeding syndrome Care must be taken when a patient begins to eat after prolonged starvation to avoid the potentially fatal complications of refeeding syndrome. The initial signs of refeeding syndrome are minimal, but can rapidly progress to death. Thus, the reinitiation of food or oral intake is usually started slowly and requires close observation under supervision by trained healthcare professionals. This is usually done in a hospital or nutritional rehabilitation center. Management Anorexia can be treated with the help of orexigenic drugs."}, {"id": "pubmed23n1114_11705", "title": "A standard enteral formula versus an iso-caloric lower carbohydrate/high fat enteral formula in the hospital management of adolescent and young adults admitted with anorexia nervosa: a randomised controlled trial.", "score": 0.014185110663983903, "content": "The nutritional rehabilitation of malnourished patients hospitalised with anorexia nervosa is essential. The provision of adequate nutrition must occur, while simultaneously, minimising the risk of refeeding complications, such as electrolyte, metabolic, and organ dysfunction. The aim of this study was to compare the efficacy and safety of an iso-caloric lower carbohydrate/high fat enteral formula (28% carbohydrate, 56% fat) against a standard enteral formula (54% carbohydrate, 29% fat). Patients (aged 15-25 years) hospitalised with anorexia nervosa were recruited into this double blinded randomised controlled trial. An interim analysis was completed at midpoint, when 24 participants, mean age 17.5 years (± 1.1), had been randomly allocated to lower carbohydrate/high fat (n = 14) or standard (n = 10) feeds. At baseline, there was no significant difference in degree of malnutrition, medical instability, history of purging or serum phosphate levels between the two treatment arms. A significantly lower rate of hypophosphatemia developed in patients who received the lower carbohydrate/high fat formula compared to standard formula (5/14 vs 9/10, p = 0.013). The serum phosphate level decreased in both feeds, however it decreased to a larger extent in the standard feed compared to the lower carbohydrate/high fat feed (standard feed 1.11 ± 0.13 mmol/L at baseline vs 0.88 ± 0.12 mmol/L at week 1; lower carbohydrate/high fat feed 1.18 ± 0.19 mmol/L at baseline vs 1.06 ± 0.15 mmol/L at week 1). Overall, serum phosphate levels were significantly higher in the lower carbohydrate/high fat feed compared with standard feed treatment arm at Week 1 (1.06 ± 0.15 mmol/L vs 0.88 ± 0.12 mmol/L, p &lt; 0.001). There was no significant difference in weight gain, number of days to reach medical stability, incidence of hypoglycaemia, or hospital length of stay. The results of this study indicate that enteral nutrition provided to hospitalised malnourished young people with anorexia nervosa using a lower carbohydrate/high fat formula (28% carbohydrate, 56% fat) seems to provide protection from hypophosphatemia in the first week compared to when using a standard enteral formula. Further research may be required to confirm this finding in other malnourished populations. ANZCTR, ACTRN12617000342314. Registered 3 March 2017, http://anzctr.org.au/Trial/Registration/TrialReview.aspx?ACTRN=12617000342314."}, {"id": "pubmed23n0659_3698", "title": "Specialized refeeding treatment for anorexia nervosa patients suffering from extreme undernutrition.", "score": 0.014085205574567276, "content": "Refeeding severely malnourished patients with Anorexia nervosa requires specialized in-patient treatment to reduce medical risks, to avoid refeeding syndrome and other life-threatening situations. The authors present a retrospective cohort nutritional rehabilitation study of 33 very severe Anorexia nervosa in-patients, aged 22.8 ± 7.6 years (mean ± SD) and with an initial body mass index ≤ 12 kg/m(2), treated in a specialized Eating Disorders Unit. Thirty-three female patients were included and treated. Mean BMI increased from 11.3 ± 0.7 Kg/m(2), to 13.5 ± 1 Kg/m(2), and mean body weight from 29.1 ± 3.2 Kg to 34.5 ± 3.3 Kg, after 60 days of intensive in-patient treatments (p &lt; 0.0001). Feeding was carefully instituted; caloric intake levels were established after measuring REE by indirect calorimetry. Nutritional support was initiated with temporary nasogastric feeding in 30 patients, and with oral supplementation in 3 patients. Vitamins, potassium and phosphate supplements were administered during refeeding. All patients achieved a significant increase in body weight, none developed refeeding syndrome as far as laboratory and clinical investigations were concerned. Our findings show that, even in cases of extreme undernutrition, if feeding is performed cautiously and in a specialized unit, it is possible to avoid the refeeding syndrome."}, {"id": "pubmed23n0359_9304", "title": "Heart risk associated with weight loss in anorexia nervosa and eating disorders: electrocardiographic changes during the early phase of refeeding.", "score": 0.013652036156928525, "content": "Refeeding syndromes with electrolyte aberrations, heart failure and arrhythmias may complicate the nutritional rehabilitation of emaciated patients with eating disorders. Therefore, electrocardiographic (ECG) changes and changes in serum electrolyte concentrations following refeeding were studied in 37 admissions of 32 teenage girls with eating disorders. On admission they were all on a weight-losing course and weighed 37.0+/-8.0 kg (mean +/- SD) following a weight loss of 14.2+/-7.2 kg. On ECG recordings there was a prolongation of the QT interval and an increased QT dispersion. Serum concentrations of sodium, potassium and magnesium were with few exceptions normal. Serum concentrations of creatinine were high in relation to the low body weight, indicating protein catabolism. The first 2 wk of refeeding resulted in a weight gain of 1.7+/-0.2 kg without signs of refeeding syndromes or electrolyte aberrations. QT prolongation and dispersion normalized within the 3 d of refeeding. It is concluded that oral refeeding of patients with eating disorders and weight loss can be performed efficiently and without causing refeeding syndromes. QT pathology, a consequence of acute starvation and a risk factor for cardiac arrhythmias, normalizes within days. In view of the need to balance adequate refeeding and reduction of QT pathology against the risks of refeeding syndromes the start of refeeding of severely emaciated patients is best performed in a hospital setting where monitoring of ECG and serum electrolytes is possible."}, {"id": "wiki20220301en302_17929", "title": "Anorexia nervosa", "score": 0.013176638176638177, "content": "Refeeding syndrome The rate of refeeding can be difficult to establish, because the fear of refeeding syndrome (RFS) can lead to underfeeding. It is thought that RFS, with falling phosphate and potassium levels, is more likely to occur when BMI is very low, and when medical comorbidities such as infection or cardiac failure, are present. In those circumstances, it is recommended to start refeeding slowly but to build up rapidly as long as RFS does not occur. Recommendations on energy requirements vary, from 5–10 kcal/kg/day in the most medically compromised patients, who appear to have the highest risk of RFS, to 1900 kcal/day. Prognosis"}, {"id": "pubmed23n1039_6531", "title": "Nutritional management of celiac crisis in an elderly adult: A case report of the rare presentation of celiac disease in a 75-y-old woman.", "score": 0.012940705128205127, "content": "In adults, a very uncommon presentation of celiac disease (CD) is a celiac crisis, a life-threatening and severe form of the disease having a dramatic onset with diarrhea and metabolic acidosis with electrolyte and fluid imbalance. Treatment of celiac crisis requires a gluten-free diet; however, the risk for refeeding syndrome (RFS) should be considered in patients showing marked malabsorption symptoms and important unintentional weight loss. Therefore, to avoid metabolic and potentially fatal complications of re-nutrition, nutritional management is crucial for a safe recovery after a celiac crisis. This review reports the rare onset of celiac crisis in a 75-y-old woman presenting with severe malnutrition resulting in &gt;40% weight loss in 3 mo, after a period of severe diarrhea and vomiting. She arrived at the hospital showing electrolyte imbalance, hypoalbuminemia, lower limb edema, multiple bowel movements (&gt;10/d) with steatorrhea, sarcopenia with profound asthenia, hyporexia due to intolerance to any food, and vomiting after meals. After being diagnosed with CD, the first approach was a gluten-free diet, which demonstrated only small and slow improvements of gastrointestinal symptoms. Therefore, a second approach was parenteral nutrition (PN) support that dramatically helped the patient's recovery. Here we describe the nutritional management during the inpatient stay for clinical stabilization and the following outpatient visits during and after the support with PN, until the patient's complete recovery to a regular follow-up."}, {"id": "wiki20220301en049_15552", "title": "Failure to thrive", "score": 0.01263134361725911, "content": "failure to thrive then treatment is directed towards the underlying condition. Special care should be taken to avoid refeeding syndrome when initiating feeds in a malnourished patient. Refeeding syndrome is caused by a shift in fluid and electrolytes in a malnourished person as they receive artificial refeeding. It is potentially fatal, and can occur whether receiving enteral or parenteral nutrition. The most serious and common electrolyte abnormality is hypophosphatemia, although sodium abnormalities are common as well. It can also cause changes in glucose, protein, and fat metabolism. Incidence of refeeding syndrome is high, with one prospective cohort study showing 34% of ICU experienced hypophosphatemia soon after feeding was restarted."}, {"id": "pubmed23n0477_14816", "title": "[Hypophosphatemia and refeeding syndrome: a severe and underdiagnosed adverse effect].", "score": 0.011958306561005211, "content": "A 68-year-old woman was hospitalised because of generalised weakness and development of confusional state, related to severe hyponatremia, probably due to an eating disorder with malnutrition. During the first days of hospitalisation the patient eats surprising large amounts of food. The worsening of the confusional state in spite of normalisation of natremia, and the progressive development of anemia and thrombocytopenia, leads to the discovery of a new onset severe hypophosphatemia. The rapid fall in plasma levels of phosphorus, magnesium and potassium are the main futures of the refeeding syndrome. Its clinical manifestations are neurological, muscular, haematological and renal. The development of this syndrome is associated with a high mortality. The refeeding syndrome is seen when carbohydrates are introduced after a period of malnutrition. Identification of patients at risk for the refeeding syndrome (anorexia nervosa, chronic alcoholism, chronic malnutrition, elderly patients, oncology patients), the introduction of cautious progressive nutrition and the careful monitoring of vital signs, electrolytes levels and fluid balance, allows to prevent morbidity and mortality of this syndrome."}, {"id": "pubmed23n0516_6757", "title": "Severe hypophosphatemia in a patient with anorexia nervosa during enteral refeeding.", "score": 0.01175378905041757, "content": "Hypophosphatemia is a seldom but potentially fatal complication of the nutritional recovery or refeeding syndrome in patients with protein-calorie malnutrition or starvation. We report here the case of a 35-year-old anorexic patient who presented a severe but uncomplicated hypophosphatemia during enteral refeeding, despite phosphorus supplementation. Serum phosphorus monitoring is recommended in severely malnourished anorexic patients, particularly during the first week of refeeding, be it parenteral or enteral."}, {"id": "pubmed23n0951_13722", "title": "Refeeding syndrome: relevance for the critically ill patient.", "score": 0.011717171717171718, "content": "To provide an overview of recent findings concerning refeeding syndrome (RFS) among critically ill patients and recommendations for daily practice. Recent literature shows that RFS is common among critically ill ventilated patients. Usual risk factors for non-ICU patients addressed on ICU admission do not identify patients developing RFS. A marked drop of phosphate levels (&gt;0.16 mmol/l) from normal levels within 72 h of commencement of feeding, selects patients that benefit from hypocaloric or restricted caloric intake for at least 48 h resulting in lower long-term mortality. RFS is a potentially life-threatening condition induced by initiation of feeding after a period of starvation. Although a uniform definition is lacking, most definitions comprise a complex constellation of laboratory markers (i.e. hypophosphatemia, hypokalemia, hypomagnesemia) or clinical symptoms, including cardiac and pulmonary failure. Recent studies show that low caloric intake results in lower mortality rates in critically ill RFS patients compared with RFS patients on full nutritional support. Therefore, standard monitoring of RFS-markers (especially serum phosphate) and caloric restriction when RFS is diagnosed should be considered. Furthermore, standard therapy with thiamin and electrolyte supplementation is essential."}, {"id": "wiki20220301en072_35433", "title": "VIPoma", "score": 0.011478952039202068, "content": "Symptoms and signs The major clinical features are prolonged watery diarrhea (fasting stool volume > 750 to 1000 mL/day) and symptoms of hypokalemia and dehydration. Half of the patients have relatively constant diarrhea while the rest have alternating periods of severe and moderate diarrhea. One third have diarrhea < 1yr before diagnosis, but in 25%, diarrhea is present for 5 yr or more before diagnosis. Lethargy, muscle weakness, nausea, vomiting and crampy abdominal pain are frequent symptoms. Hypokalemia and impaired glucose tolerance occur in < 50% of patients. Achlorhydria is also a feature. During attacks of diarrhea, flushing similar to the carcinoid syndrome occur rarely. Diagnosis Besides the clinical picture, fasting VIP plasma level may confirm the diagnosis, and CT scan and somatostatin receptor scintigraphy are used to localise the tumor, which is usually metastatic at presentation."}, {"id": "pubmed23n0315_15485", "title": "Cardiac arrest and delirium: presentations of the refeeding syndrome in severely malnourished adolescents with anorexia nervosa.", "score": 0.01138888888888889, "content": "To describe the clinical presentation of the refeeding syndrome and highlight the dangers of performing nutritional rehabilitation too rapidly in a severely malnourished patient. Retrospective case review of adolescents admitted with anorexia nervosa who developed the refeeding syndrome. Between July 1993 and July 1994, 3 of 48 adolescent females developed the refeeding syndrome. While the cardiac complications occurred in the first week of refeeding, the delirium characteristic of this syndrome occurred later and was more variably related to hypophosphatemia. Refeeding malnourished patients with anorexia nervosa can be associated with hypophosphatemia, cardiac arrhythmia and delirium. Refeeding patients with anorexia nervosa who are &lt; 70% of ideal body weight should proceed with caution, and the caloric prescription should be increased gradually. Supplemental phosphorus should be commenced early and serum levels maintained above 3.0 mg/dL. Cardiac and neurologic events associated with refeeding are most likely to occur within the first weeks, justifying close monitoring of electrolyte and cardiac status."}, {"id": "pubmed23n1114_5923", "title": "Rapid renutrition improves health status in severely malnourished inpatients with AN - score-based evaluation of a high caloric refeeding protocol in severely malnourished inpatients with anorexia nervosa in an intermediate care unit.", "score": 0.011300877893056664, "content": "Refeeding syndrome is a feared complication of refeeding patients with anorexia nervosa. There are now a number of controlled studies showing that refeeding with an initial high calorie count is more beneficial than cautious refeeding and is safe under continuous monitoring. However, there have yet not been studies in severe anorexia nervosa. We present an observational study in two different samples. The first sample consists of those 1075 out of a total of 3230 patients with anorexia nervosa treated in our hospital within 4 years for whom a complete admission laboratory was available and who had an age of at least 18 years at admission. A risk score was calculated from the number of pathological laboratory values out of 12 parameters indicating either refeeding syndrome or health hazards related to malnutrition. The second sample was obtained from a special ward for patients with eating disorders medically at-risk. During the period in question, 410 patients with anorexia nervosa were treated there. 142 patients had a BMI of 13 or less and at the same time a complete data set with the mentioned 12 laboratory parameters at admission and weekly in the following 4 weeks after admission. The risk represented by the laboratory parameters is significantly and negatively correlated to BMI and much higher for the group of patients with a BMI below 13 than for those with a higher BMI (χ<sup2</sup sig &lt; 0.000). The 142 patients in the special care unit gain an average of more than 4.1 kg within 4 weeks on the high-calorie diet. With this rapid weight gain, the risk score decreases highly significantly. Neither hypophosphatemia nor rhabdomyolysis is found under phosphate substitution. Hyperhydration occurred often, which manifests itself in the drop in haematocrit by the second week. Under thorough medical surveillance, supplementation of phosphate and thiamine, and substitution of electrolytes whenever necessary rapid renutrition appeared to be save even in extremely malnourished inpatients with anorexia nervosa. As measured by the laboratory values, the health status of the severely malnourished patients improves significantly on a high-calorie diet. Except for hyperhydration, there was no evidence of a refeeding syndrome."}, {"id": "pubmed23n1018_11823", "title": "Refeeding syndrome as treatment complication of anorexia nervosa.", "score": 0.01101920057964014, "content": "Refeeding syndrome (RS) is one of the serious complications during treatment of anorexia nervosa. It includes hormonal and metabolic changes that occur during the process of refeeding in chronically malnourished patient when nutrition is introduced in an excessive and improper amount. RS manifests in water-electrolyte imbalances, including hypophosphatemia (the mostimportant diagnosticmarker), hypokalemia, hyponatremia, hypomagnesaemia, fluid retention, vitamin deficiency and metabolic acidosis. It applies to either oral and parenteral supplementation. In the treatment of malnourished patients with anorexia nervosa, it is essential to establish an initial caloric amount that will stimulate weight gain from the beginning of treatment, increase its effectiveness while minimizing the risk of RS. Recent research suggests that the current recommendations may be too stringent in this respect and require further updating. Awareness of the risks associated with RS, including significant mortality, appears to be currently insufficient also among physicians. There is a need for far more specialized multidisciplinary centers for patients with anorexia nervosa and also appropriate algorithms and standards of care for that population. The aim of this paper is to systematize the current knowledge about RS and RS prevention, to increase awareness of its occurrence and present the results of the latest research on safe resupplementation of patients suffering from anorexia nervosa."}, {"id": "pubmed23n0556_20710", "title": "Enteral refeeding syndrome after long-term total parenteral nutrition.", "score": 0.010835035171318357, "content": "Early enteral feeding (EF) may result in fever, elevated white blood cell count, increased serum levels of liver enzymes, and diarrhea. We name the complications \"enteral refeeding syndrome\", as a subtype of refeeding syndrome, because they are likely to result from long-term lack of lumen nutrition. The aim of this study was to investigate the characteristics of enteral refeeding syndrome after long-term total parenteral nutrition (TPN), and the solution for the disease. We collected the clinical data of 100 patients with gastrointestinal fistula, who were cured from Apirl 2001 to July 2002. Their fasting time, daily stool frequency, body temperature, heart rate, respiratory rate, levels of transaminases, alkaline phosphatase (AKP), and gamma-glutamylcyclotransferase (gamma-GT), white blood cell count, and systemic inflammatory reaction syndrome (SIRS) score were recorded before and 1, 3, 5, 10, and 15 days after EF. Student's t test and analysis of variance were used to analyze the data. Of the 100 patients, 56 were cured after selective resection of intestinal fistula, 15 were cured by emergency operation, and 29 recovered spontaneously. The levels of AKP and gamma-GT increased significantly on the 3rd day after EF [On the 3rd day after EF, (243.0 +/- 121.6) U/L and (177.2 +/- 109.9) U/L vs. before EF (181.5 +/- 127.5) U/L and (118.4 +/- 94.2) U/L, P &lt; 0.05], and decreased gradually afterwards. The SIRS scores on the 1st day (1.05 +/- 1.08) and 3rd day (0.96 +/- 1.11) after EF were significantly higher than that before EF (0.72 +/- 0.84), then decreased to 0.83 +/- 0.91, 0.49 +/- 0.73 and 0.32 +/- 0.60 on the 5th, 10th and 15th days after EF. The number of patients with diarrhea at 1, 3, 5, 10 and 15 days post-EF were 31, 26, 12, 13, and 7, respectively. The longer the TPN lasts, the more severe the enteral refeeding syndrome becomes. Continuous EF is effective for the syndrome. Early enteral nutrition is useful in preventing it."}, {"id": "pubmed23n0372_13667", "title": "Refeeding procedures after 43 days of total fasting.", "score": 0.010414163262036369, "content": "Refeeding syndrome encompasses fluid and electrolyte imbalances and metabolic, intestinal, and cardiorespiratory derangements associated with appreciable morbidity and mortality. Although refeeding syndrome has been well documented in concentration-camp subjects, and more recently during parenteral therapy of critically ill patients, little is known about the importance of refeeding syndrome during recovery from a hunger strike. Thus, we studied the response to a four-step dietary replenishment routine in eight hunger strikers who refused food for 43 d. In this retrospective, observational study, we assessed the safety and efficacy of the refeeding procedure and analyzed the clinical and nutritional course of the cohort during both starvation and refeeding, mainly on the basis of clinical as well as a few biochemical determinations. During starvation, average weight loss was about 18% and, with the exception of occasional oral vitamins and electrolytes, the subjects consumed only water. Available body-composition and biochemical profiles showed no clinically significant changes during starvation, but one-half of the group displayed spontaneous diarrhea at some time before refeeding. Stepwise nutritional replenishment lasted for 9 d, after which all patients tolerated a full, unrestricted diet. Only one episode of diarrhea occurred during this phase, and both clinical and biochemical indexes confirmed a favorable clinical course, without any manifestation of refeeding syndrome. In conclusion, we observed the following: 1) Hypophosphatemia and other micronutrient imbalances did not occur, nor was macronutrient intolerance detected. 2) Despite some episodes of diarrhea, nutritional replenishment was not associated with significant enteral dysfunction. 3) There was some fluid retention, but this was mild. 4) Acute-phase markers were abnormally elevated during the refeeding phase, without associated sepsis or inflammation."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[311, 490]], "word_ranges": [[54, 85]], "text": "Answer 1 cannot be because a marrow aplasia does not explain the choluria, the elevation of LDH, nor in the study of irregular antibodies is positive in the form of panagglutinin;"}, "2": {"exist": true, "char_ranges": [[703, 805]], "word_ranges": [[117, 133]], "text": "Answer 2 is not possible either, since a spherocytosis does not justify the presence of panagglutinin."}, "3": {"exist": true, "char_ranges": [[806, 1112]], "word_ranges": [[133, 179]], "text": "Answer 3 is the one I consider correct: an autoimmune hemolytic anemia would justify the data given: elevated LDH and bilirubinemia due to red cell destruction, polychromatophilia, spherocytosis and anisocytosis because the marrow is working hard to try to compensate for the anemia, which is regenerative."}, "4": {"exist": true, "char_ranges": [[1494, 1876]], "word_ranges": [[236, 296]], "text": "Answer 4 gives rise to considerable doubt; it is not possible because a pernicious anemia does not justify the presence of panagglutinin although it would justify the elevation of LDH and bilirubin; moreover, it is an intramedullary, arregenerative hemolysis, there is no reticulocytosis or release into the blood of immature forms in an attempt to compensate and fix the situation."}, "5": {"exist": true, "char_ranges": [[1877, 1988]], "word_ranges": [[296, 317]], "text": "Answer 5 is not true because no blasts are seen in the blood nor does it explain the presence of panagglutinin."}}, "full_answer": "A long statement full of rather confusing data. We can attack that question in two ways: by looking at the key data and going all the way to the diagnosis we are considering or by discarding one by one. The choluria, LDH, presence of panagglutinin and the peripheral blood smear \"smells\" like hemolytic anemia. Answer 1 cannot be because a marrow aplasia does not explain the choluria, the elevation of LDH, nor in the study of irregular antibodies is positive in the form of panagglutinin; an aplasia is a marrow failure characterized by a total or partial disappearance of hemopoietic progenitors. In addition, pancytopenia is not observed, which is what would incline us more towards this pathology. Answer 2 is not possible either, since a spherocytosis does not justify the presence of panagglutinin. Answer 3 is the one I consider correct: an autoimmune hemolytic anemia would justify the data given: elevated LDH and bilirubinemia due to red cell destruction, polychromatophilia, spherocytosis and anisocytosis because the marrow is working hard to try to compensate for the anemia, which is regenerative. The study of irregular antibodies and the presence of panagglutinin also supports this response, since the binding of an antibody to the hematocyte promotes its lysis and destruction. The girl presents cough and fever, consistent with a respiratory infection. And the initial treatment is corticosteroids. This is confirmed by Sans Sabrafens in his book \"Clinical Hematology\" [1]. Answer 4 gives rise to considerable doubt; it is not possible because a pernicious anemia does not justify the presence of panagglutinin although it would justify the elevation of LDH and bilirubin; moreover, it is an intramedullary, arregenerative hemolysis, there is no reticulocytosis or release into the blood of immature forms in an attempt to compensate and fix the situation. Answer 5 is not true because no blasts are seen in the blood nor does it explain the presence of panagglutinin.", "full_answer_no_ref": "A long statement full of rather confusing data. We can attack that question in two ways: by looking at the key data and going all the way to the diagnosis we are considering or by discarding one by one. The choluria, LDH, presence of panagglutinin and the peripheral blood smear \"smells\" like hemolytic anemia. Answer 1 cannot be because a marrow aplasia does not explain the choluria, the elevation of LDH, nor in the study of irregular antibodies is positive in the form of panagglutinin; an aplasia is a marrow failure characterized by a total or partial disappearance of hemopoietic progenitors. In addition, pancytopenia is not observed, which is what would incline us more towards this pathology. [HIDDEN], since a spherocytosis does not justify the presence of panagglutinin. [HIDDEN]: an autoimmune hemolytic anemia would justify the data given: elevated LDH and bilirubinemia due to red cell destruction, polychromatophilia, spherocytosis and anisocytosis because the marrow is working hard to try to compensate for the anemia, which is regenerative. The study of irregular antibodies and the presence of panagglutinin also supports this response, since the binding of an antibody to the hematocyte promotes its lysis and destruction. The girl presents cough and fever, consistent with a respiratory infection. And the initial treatment is corticosteroids. This is confirmed by Sans Sabrafens in his book \"Clinical Hematology\" [1]. [HIDDEN]; it is not possible because a pernicious anemia does not justify the presence of panagglutinin although it would justify the elevation of LDH and bilirubin; moreover, it is an intramedullary, arregenerative hemolysis, there is no reticulocytosis or release into the blood of immature forms in an attempt to compensate and fix the situation. [HIDDEN].", "full_question": "A 32-year-old woman with cerebral palsy from childbirth comes to the emergency department for a few days of dark urine associated with an episode of high fever and dry cough. On admission, the CBC showed 16900 leukocytes/mm3 (85% S, 11% L, 4% M), hemoglobin 6.3 g/dL; MCV 109 fl, 360000 platelets/mm3. In the biochemistry LDH 2408; bilirubin 6.8 mg/dl, (unconjugated bilirubin 6.1 mg/dl), normal GOT and GPT. The morphological study of blood showed macrocytic anisocytosis with frequent spherocytic forms and polychromatophilia without blasts. The irregular antibody study is positive for panagglutinin, making crossmatching difficult. What would be your suspicion and the most appropriate treatment?", "id": 115, "lang": "en", "options": {"1": "Medullary aplasia and immunotherapy with thymoglobulin and cyclosporine.", "2": "Hereditary spherocytosis and splenectomy.", "3": "Autoimmune hemolytic anemia associated with respiratory infection and corticosteroids.", "4": "Pernicious anemia and periodic injections of vitamin B12.", "5": "Acute leukemia and chemotherapy."}, "question_id_specific": 98, "type": "HEMATOLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1015_11034", "title": "IgA-mediated autoimmune hemolytic anemia in an infant.", "score": 0.018183320935614512, "content": "Autoimmune Hemolytic anemia (AIHA) a relatively uncommon form of hemolytic anemia in children, occurs due to the premature destruction of red blood cells caused by presence of autoantibodies directed against antigens on RBCs. Warm reactive AIHA is the most common form due to IgG isotype of immunoglobulin class binding to autologous RBCs at 37<sup0</supC and confirmed with a positive DAT screening. We present a case of DAT-negative primary warm AIHA in an infant due to IgA antibody. A 10 month old male infant presented with dark colored urine and irritability for past two months, with associated history of fever, diarrhea and vomiting. He had received one red cell transfusion 10 days prior. On physical examination he had pallor with tachycardia without splenomegaly. On investigation his hemoglobin was 5.8 g/dl, WBC 25.9 × 10<sup3</sup/mm<sup3</sup and normal platelets counts. Peripheral blood smear had spherocytes and biochemical values showed high bilirubin and LDH. Immunohematological work up revelaed polyspecific DAT was negative but monospecific DAT screening showed strong (4+) positivity for IgA and a weak IgG positivity. The patient was diagnosed as IgA-mediated Warm AIHA and was started on prednisolone at 2 mg/kg/day following which hemoglobin improved over the next 2 months. After 2 weeks, prednisolone was tapered and stopped by the end of 3 months. Patients with clinical and laboratory evidence of acute hemolysis, an additional screening for IgA antibody may be done even in cases where poly-specific DAT is negative. Early detection helps in avoiding further investigations and provide efficient management."}, {"id": "pubmed23n1155_14862", "title": "Severe hemolysis with negative direct antiglobulin test: A case report.", "score": 0.017838819164890676, "content": "A 49-year-old woman with type 2 diabetes mellitus (T2DM) presented to the emergency department. Her examination showed marked pallor, exhaustion, lethargy, yellowish eyes, anorexia, nausea and vomiting. Hematuria; negative standard direct antiglobulin test (DAT); normal glucose 6 phosphate dehydrogenase (G6PD); hemoglobin (Hb), 4.8 g/dl; Mean cell volume (MCV), 91fl; platelet count, 233 × 10<sup6</sup/L; Total bilirubin, 7.0 mg/dl; Glucose, 316 mg/dl; lactate dehydrogenase (LDH), 1750U/L. Undoubtedly, therapeutic panel should have been used for hemolytic anemia. Intravenous (IV) fluids and 2 units of packed cell were transfused. Methylprednisolone with rituximab were started for the patient. After 3 weeks of the patient admission, she was discharged home with stable vital signs and Hb, 10 g/dl. We concluded in the cases that presented along with a severe drop in Hb and evidence of hemolysis which non immune hemolytic anemia is excluded in spite of negative standard DAT limited transfusion besides corticosteroids combined with rituximab, could be helpful in saving the patient."}, {"id": "pubmed23n0870_7365", "title": "Paroxysmal Nocturnal Haemoglobinuria in the Differential Diagnosis of Unresponsive Iron Deficiency Anemia: A Case Report.", "score": 0.017615384615384616, "content": "A 16-year-old male patient who was on oral iron treatment for iron deficiency anemia for the last one year was seen at the Haematology clinic with complaints of weakness, pallor, and jaundice. A complete blood count revealed Hb of 4.2 mmol/L, Hct of 0.14, and MCV of 76 fl. A blood smear showed 50% neutrophils, 40% lymphocytes, and 10% monocytes with anisocytosis, poikilocytosis, polichromasia in erythrocytes and normoblasts. Reticulocyte count was under 1%. There was a slight erythroid hyperplasia in the bone marrow aspiration. Biochemical examinations showed total bilirubin of 3.9 mg/dL, indirect bilirubin of 3.4 mg/dL, and lactate dehydrogenase (LDH) of 6085 U/L (220-450). In re-evaluating the history of the patient, he was seen to be complaining of dark discoloration of morning urine. Perl's reaction was found to be positive for hemosiderin in the urine sediment. Because Ham's test was positive, the levels of CD55, 58, and 59 proteins on erythrocyte membranes were found to be lower. The patient was started 32 mg of methylprednisolone and his anaemia was improved by the 14th day of treatment. When evaluating iron deficiency anemia resistant to iron supplementation, PNH should be kept in mind. "}, {"id": "pubmed23n0351_21300", "title": "Macrocytic anemia and thrombocytosis associated with thymoma: a case report.", "score": 0.015764936817568395, "content": "Thymomas are often associated with autoimmune disorders. We report on a 45-year-old female patient with thymoma and hypogammaglobulinemia (Good's syndrome) who developed symptomatic macrocytic anemia (Hb 4.4 g/dl, MCV 112 fl) and thrombocytosis (Plt 442 G/l). Besides hypogammaglobulinemia (IgG 589 mg/dl), an inverted ratio of CD4(+)/CD8(+) cells was seen. The bone marrow biopsy showed a slightly hypercellular bone marrow with normal granulopoiesis, normal megakaryopoiesis and a mild dyserythropoiesis without any ring-sideroblasts. The in-vitro stem cell culture from the bone marrow revealed an atypical growth of macroclusters, reduced BFU-E and CFU-GEMM colony growth, whereas the CFU-GM colony growth was within the normal range. The chromosomal analysis showed a normal karyotype. The plasma vitamin B(12) and folate levels were within normal ranges, and we could not detect any autoantibodies. These findings excluded the differential diagnoses pure red cell aplasia (PRCA) and pernicious anemia. After resection of the thymoma of mixed cell type, the macrocytic anemia and thrombocytosis disappeared. The clinical course was complicated by a cerebral palsy and a life-threatening fungal septicemia after surgery. In the third year after thymectomy, hyporegenerative macrocytic anemia and thrombocytosis reappeared and an immunosuppressive treatment with prednisolone (1 mg/kg BW) was started. After initiation of the prednisolone therapy, reticulocyte counts increased and macrocytic anemia as well as thrombocytosis disappeared. The normalization of these laboratory parameters during glucocorticoid therapy suggests that in rare cases the constellation of macrocytic anemia, thrombocytosis and hypogammaglobulinemia may be due to an underlying immunologic mechanism."}, {"id": "pubmed23n0818_22421", "title": "[A case of autoimmune lymphoproliferactive syndrome and literature review].", "score": 0.015385505234542195, "content": "To summarize the clinical characteristics, diagnosis and treatment of a case with autoimmune lymphoproliferative syndrome (ALPS) . The patient was diagnosed as autoimmune lymphoproliferactive syndrome (ALPS) after being admitted to the Department of Rheumatism and Immunology of Tianjin Children's Hospital in February 20, 2014. The clinical characteristics, physical examination, laboratory tests, gene tests, and treatment process were analyzed and related literature was reviewed. The patient was a 16-month- old boy.Since the first month of life, he started to have repeatedly fever, diarrhea, shortness of breath, lymphadenopathy, hepatosplenomegaly, anemia (HGBmin 50 g/L) and thrombocytopenia (min 35 × 10⁹/L) . But multiple exams showed a normal peripheral blood leukocyte count, hypergammaglobulinemia (IgG 19 800 mg/L, IgA 1 710 mg/L, IgM 2 590 mg/L) and significantly increased serum vitamin B12. Flow cytometric measures showed that CD3⁺ CD4⁻ CD8⁻ T lymphocytes significantly increased ( &gt; 10%) at four times. The count of CD3⁺ TCRαβ⁺ CD4⁻ CD8⁻T lymphocytes (double negative T cells; DNTs) &gt;3% twice. The genetic test showed that 309th FAS gene area showed heterozygous mutations, the boy was diagnosed as ALPS. Added examinations of lymphocytes apoptosis induced by FAS was positive. He was treated with prednisone 15 mg once daily and immunomodulator 150 mg three times a day, while in maintaining period with normal levels of hemoglobin and platelet, the dose of prednisone was reduced gradually. Till now, the patient has been treated and observed for 8 months. We retrieved the reports of ALPS in the databases at home and abroad published in recent 10 years, more than 400 cases reported from foreign countries, but there were only 5 domestic cases. Among those, 4 had onset in infancy and 1 at 6-years of age. All the cases presented servere lymphadenopathy and hepatosplenomegaly with anemia (4 of them with hemolytic anemia) and thrombocytopenia. Three cases had a history of frequent infection, one of them had glomerulonephritis. All patient with significant high level of serum immunoglobulin ( &gt; 1.5 times upper limit of normal range), in 3 of them serum vitamin B12 was &gt; 1.5 pg/L (the other 2 cases missed the exam). In 5 cases CD3⁺ CD4⁻ CD8⁻T cells &gt; 10%, and in 2 case DNTs were 8.9% and 15.7% respectively (the other 3 cases missed the exam). Three cases were clearly detected with FAS mutations. All patients were treated with corticosteroid, 2 of them were added with mycophenolate mofetil. The therapy presented effective result in early 1-3 months, but no long-term follow-up reports were available. ALPS is a disorder of disrupted lymphocyte homeostasis caused by defective Fas-mediated apoptosis, and it is one of the primary immunodeficiency diseases. The onset of the disease occurs during infancy mainly. Clinical lymphoid hyperplasia and autoimmune phenomena are outstanding signs, which can be associated with frequent infections and allergies. The level of serum vitamin B12 &gt; 1.5 pg/L and the count of CD3⁺ CD4⁻ CD8⁻ T cell show important significance. Exact diagnosis should depend on detecting DNTs and FAS gene."}, {"id": "pubmed23n0297_3204", "title": "[Autoimmune hemolytic anemia with eosinophilia in elderly patient].", "score": 0.014601652011723955, "content": "A 70-year-old woman was admitted to our hospital in November 1992 for evaluation of anemia. Physical examination revealed anemia, jaundice, swelling of axial and inguinal lymph nodes, and splenomegaly. Abnormal hematological findings were as follows: Hb of 3.9 g/dl, reticulocyte count of 58.2% (61.7 x 10(4)/microliters), hyperplasia of normal erythroblasts in bone marrow, and eosinophilia (21.0%, 2352/microliters) in peripheral blood. Routine laboratory examinations revealed polycolonal hypergammaglobulinemia 3.0 g/dl, a high level of serum LDH (797 IU/I) and a total bilirubin of 2.4 mg/dl (indirect, 1.6 mg/dl). The serum haptoglobin level was very low (&lt; 5 mg/dl). Results of serological examinations were as follows: IgG of 3366 mg/dl, CH50 of 16.0 U/ml, positive Coombs test 2+, and positive tests for antinuclear antibody, rheumatoid factor, and cold agglutinin. CRP was negative. PHA-stimulated lymphocyte blast formation, NK activity, and ADCC activity were found to be suppressed, and the percentage of CD4-positive lymphocytes in peripheral blood was also low. An axillary lymph node biopsy revealed reactive lymphadenitis. No signs or history suggested allergy, collagen disease, or parasitic infection. Autoimmune hemolytic anemia (AIHA) complicated by immunologic abnormalities and eosinophilia was diagnosed. Oral prednisolone markedly reduced the hemolytic anemia, eosinophilia, lymph node swelling, and splenomegaly, but NK activity remained low."}, {"id": "wiki20220301en011_95937", "title": "Spherocytosis", "score": 0.01419294990723562, "content": "Spherocytosis is the presence of spherocytes in the blood, i.e erythrocytes (red blood cells) that are sphere-shaped rather than bi-concave disk shaped as normal. Spherocytes are found in all hemolytic anemias to some degree. Hereditary spherocytosis and autoimmune hemolytic anemia are characterized by having only spherocytes. Causes Spherocytes are found in immunologically-mediated hemolytic anemias and in hereditary spherocytosis, but the former would have a positive direct Coombs test and the latter would not. The misshapen but otherwise healthy red blood cells are mistaken by the spleen for old or damaged red blood cells and it thus constantly breaks them down, causing a cycle whereby the body destroys its own blood supply (auto-hemolysis). A complete blood count (CBC) may show increased reticulocytes, a sign of increased red blood cell production, and decreased hemoglobin and hematocrit. The term \"non-hereditary spherocytosis\" is occasionally used, albeit rarely."}, {"id": "pubmed23n0282_16640", "title": "[An elderly case of thrombotic thrombocytopenic purpura].", "score": 0.013415067519545132, "content": "A 78-year-old woman was admitted to our hospital because of disorientation and fever on January 21, 1992. Two days before admission she experienced vomiting, anorexia and general malaise. Laboratory examinations on admission disclosed a hemoglobin level of 11.1 g/dl and a platelet count of 8,000/microliters. The peripheral blood smear revealed anisocytosis with numerous schistocytes and poikilocytes. Polychromatophilic and nucleated red blood cells were also seen, and the reticulocyte count was 38/1000. Her serum lactate dehydrogenase (LDH) value was 2,977 WU and the total serum bilirubin level was 3.5 mg/dl with 2.7 mg/dl indirect reacting fraction. Serum creatinine was 4.7 mg/dl. Her consciousness became semicomatose after a systemic seizure which lasted approximately 15 seconds and her hemoglobin level decreased to 8.5 g/dl on hospital day 2. Therefore, we diagnosed her as having thrombotic thrombocytopenic purpura (TTP) because of the presence of all 5 features, that is, thrombocytopenia, microangiopathic hemolytic anemia, fluctuating neurologic abnormalities, renal dysfunction and fever. A plasmapheresis with fresh frozen plasma (FFP) replacement was begun on that day. She was also treated with anti-platelet agents, 80 mg/day aspirin, and 300 mg/day dipyridamole. Moreover, packed red blood cells (PRC) were infused. While also receiving diphenylhydantoin and phenobarbital to prevent convulsions, status epilepticus developed on day 3. Because of inhibited spontaneous respiration which was an adverse effect derived from diazepam and sodium thiamylal administered intravenously to treat the status epilepticus, an artificial respiration was initiated.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en051_5938", "title": "Pancytopenia", "score": 0.01239427456083279, "content": "Diagnosis Pancytopenia usually requires a bone marrow biopsy in order to distinguish among different causes. anemia: hemoglobin < 13.5 g/dL (male) or 12 g/dL (female). leukopenia: total white cell count < 4.0 x 109/L. Decrease in all types of white blood cells (revealed by doing a differential count). thrombocytopenia: platelet count < 150×109/L. Treatment Treatment is done to address the underlying cause. To tide over immediate crisis Blood transfusion with packed red blood cells (PRBC) or platelet transfusion may be done. Sometimes there are obvious clinical clues to suggest underlying B12 deficiency for a cause of pancytopenia. In this selected cases even with severe anemia blood product transfusions can be avoided and vitamin B12 treatment itself suffice. In other situations like acute leukemia, Myelodysplastic syndrome, aplastic anemia etc. disease specific therapy is needed. References External links EID Journal (Volume 6, Number 6), CDC, December 2000."}, {"id": "wiki20220301en218_13340", "title": "Hematologic disease", "score": 0.012044445701037632, "content": "Myeloid Hemoglobinopathies (congenital abnormality of the hemoglobin molecule or of the rate of hemoglobin synthesis) Sickle cell disease Thalassemia Methemoglobinemia Anemias (lack of red blood cells or hemoglobin) Iron-deficiency anemia Megaloblastic anemia Vitamin B12 deficiency Pernicious anemia Folate deficiency Hemolytic anemias (destruction of red blood cells) Genetic disorders of RBC membrane Hereditary spherocytosis Hereditary elliptocytosis Congenital dyserythropoietic anemia Genetic disorders of RBC metabolism Glucose-6-phosphate dehydrogenase deficiency (G6PD) Pyruvate kinase deficiency Immune mediated hemolytic anemia (direct Coombs test is positive) Autoimmune hemolytic anemia Warm antibody autoimmune hemolytic anemia Idiopathic Systemic lupus erythematosus (SLE) Evans syndrome (antiplatelet antibodies and hemolytic antibodies) Cold autoimmune hemolytic anemia Cold agglutinin disease Paroxysmal cold hemoglobinuria (rare)"}, {"id": "pubmed23n1028_22226", "title": "Diagnosis and management of autoimmune hemolytic anemia in children.", "score": 0.011765095777821966, "content": "The aim of this study is to evaluate the clinical, biological and hematological profiles of autoimmune hemolytic anemia (AIHA) in children and to specify its etiologies, therapeutic modalities, and treatment responses. This is a 14-year retrospective study of AIHA cases collected at the department of pediatric emergency and reanimation of Hedi Chaker University Hospital in Sfax. We included patients under 14 years old with clinical and biological features of hemolysis and a positive direct antiglobulin test (DAT). The selected patients' demographic characteristics, physical signs, laboratory findings, and treatment responses were recorded. Thirteen cases of AIHA were collected, including 8 girls and 5 boys. The median age at diagnosis was 4 years and 6 months (range: 8 months to 13 years). Consanguinity was reported in 6 cases and 4 patients had a previous infection history. The onset of AIHA was progressive in 9 cases, marked by an anemic syndrome and hemolysis symptoms in 6 and 8 cases, respectively. The clinical triad (pallor, jaundice and splenomegaly) was found in only 4 cases. At the time of diagnosis, the median hemoglobin (Hb) level was 6g/dL (range: 4.2 to 9.2g/dL), anemia was non-regenerative in 2 patients. Thrombocytopenia and neutropenia were noted in 5 and 1 patient, respectively. Peripheral smear examination showed spherocytosis in 2 cases. All the patients had a positive DAT. Of these, 10 were positive with IgG and 3 with both IgG and C3d. AIHA was secondary to other conditions in 9 patients: infection (3 cases), autoimmune disease (4 cases), and immunodeficiency (2 cases). All the patients received first-line corticosteroid therapy but only 8 of them required blood transfusions due to severe anemia. Complete remission was obtained in 7 cases. Corticosteroid resistance and dependence were noted in 1 and 2 cases, respectively. During evolution, additional therapy was indicated in 4 patients and it included cyclosporine A, azathioprine, and mycophenolate mofetil (MMF). After a median follow-up of 4.5 years, the cure rate was 80% and only 1 patient (a boy) died due to his underlying pathology. Our study highlights the rarity, severity, and heterogeneity of etiological contexts of AIHA in children. The therapeutic difficulties justify specific expertise in pediatric hematology."}, {"id": "wiki20220301en068_25915", "title": "Warm antibody autoimmune hemolytic anemia", "score": 0.011241953210865128, "content": "Diagnosis Diagnosis is made by a positive direct Coombs test, other lab tests, and clinical examination and history. The direct Coombs test looks for antibodies attached to the surface of red blood cells. Clinical findings Laboratory findings include severe anemia, normal MCV (mean corpuscular volume) , and hyperbilirubinemia (from increased red cell destruction) that can be of the conjugated or unconjugated type. Treatment Corticosteroids and immunoglobulins are two commonly used treatments for warm antibody AIHA. Initial medical treatment consists of prednisone. If ineffective, splenectomy should be considered. If refractory to both these therapies, other options include rituximab, danazol, cyclosphosphamide, azathioprine, or ciclosporin. High-dose intravenous immune globulin may be effective in controlling hemolysis, but the benefit is short lived (1–4 weeks), and the therapy is very expensive. See also List of circulatory system conditions References"}, {"id": "pubmed23n1043_7936", "title": "[A Case of Simultaneous Acute Lymphoblastic Leukemia Diagnosis with Crimean-Congo Hemorrhagic Fever].", "score": 0.01095658271489511, "content": "Crimean-Congo hemorrhagic fever (CCHF) is a zoonotic disease that can be presented with fever, fatigue, generalized joint/body pain, diarrhea and bleeding in various parts of the body. The risk of developing a severe fatal disease in humans, the possibility of being infected with aerosols and the risk of being used as a biological weapon make the disease still an important health problem all over the world as there is no a specific treatment and vaccine that has proven effective againt the virus today. The pathogenesis of the disease is not known, but vascular endothelial damage is prominent. Therefore, it progresses with thrombocytopenia, anemia, leukopenia and this hematological findings can be confused with hematological malignancies. Acute lymphoblastic leukemia (ALL) is a malignancy included in differential diagnoses and occurs as a result of mutations occuring at a stage of differentiation in the lymphoid precursor cells in the bone marrow. In this study, we present a case of ALL who was diagnosed with CCHF simultaneously. A 43-year old female patient who works in the library and does not have a chronic disease other than asthma and thyroid disorder, has admitted to our hospital with the complaints of intermittent fever, weakness, generalized joint and body pain for about 3 weeks. She had fever and the physical examination revealed bilateral cervical and right postauricular lymphadenopathies. Her aspartate aminotransferase: 77 U/L, alanine aminotransferase: 117 U/L, lactate dehydrogenase: 616 U/L, hemoglobin: 8.27 g/dl, leukocyte count: 15.690/mm3 , neutrophil count: 550/mm3 (%3.5), lymphocyte count: 6690/mm3 (%42.6), platelet count: 102.100/mm3 , C-reactive protein: 163.6 mg/L was detected and the patient was hospitalized on 5 August 2019 for further examination and treatment. Considering that the patient may have viral infection in the foreground the requested test results were detected as; anti-CMV IgM negative, anti-CMV IgG positive, anti-toxoplasma IgM negative, anti-toxoplasma IgG positive, anti-rubella IgM negative, anti-rubella IgG positive, HBsAg negative, anti-HBc IgM negative, antiHBs positive, anti-HAV IgM negative, anti-HAV IgG positive, anti-HCV negative, anti-HIV negative, EpsteinBarr virus (EBV) VCA IgM negative, EBV VCA IgG positive, EBV EBNA IgG positive. Brucella Rose Bengal and Coombs tube agglutination was found be negative. As the cytopenia of the patient deepened, the patient was accepted to have neutropenic fever and it was planned to start piperacillin-tazobactam 4 x 4.5 g/day and two units of erythrocyte replacement therapy. When the patient's history was questioned again, it was learned that she had a tick on her neck about three weeks ago and she had removed the tick herself; 4-5 days later she had the complaints of fever and flu like symptoms and also diarrhea complaints lasting for 3-4 days. Considering the current anamnesis and laboratory findings, the patient was thought to have CCHF and the patient was isolated. The serum sample taken from patient with an initial diagnosis of CCHF and sent to Department of Microbiology Reference Laboratory Public Health Agency of Turkey. The patient was referred to the Antalya Training and Research Hospital. The patient's CCHF serum result was positive. Ribavirin treatment was not initiated in the patient who was accepted to be in the convalescence period, piperacillin-tazobactam 4 x 4.5 g/day treatment was continued and supportive treatment was given. In the follow-up, as the patient's neutropenia, thrombocytopenia and lymphocytopenia still continuing, she was transferred to hematology clinic for malignancy examination and bone marrow biopsy performed by hematology and B cell ALL was diagnosed. She was accepted to be convalescent in terms of CCHF and chemotherapy was started for ALL treatment by hematology. The patient is still being followed up by the hematology clinic and allogenic hematopoietic stem cell tranplantation is planned for the patient. As a result, CCHF is a disease that can be confused with many differential diagnosis. With this case, it is aimed to draw attention to the diagnostic difficulties of CCHF and ALL and to be the first case in the literature."}, {"id": "Pediatrics_Nelson_3246", "title": "Pediatrics_Nelson", "score": 0.010762017278298753, "content": "Laboratory Diagnosis. The peripheral blood smear in autoimmune hemolytic anemia usually reveals spherocytes and occasionally nucleated RBCs. The reticulocyte count varies because some patients have relatively low reticulocyte counts as a result of autoantibodies that cross-react with RBC precursors. Treatment and Prognosis. Transfusion for the treatment of autoimmune hemolysis is challenging because crossmatching is difficult, as the autoantibodies react with virtually all RBCs. In addition to transfusion, which may be lifesaving, management of autoimmune hemolytic anemia depends on antibody type. Management may involve administration of corticosteroids and, at times, intravenous immunoglobulin. Corticosteroids reduce the clearance of sensitized RBCs in the spleen. In drug-induced hemolysis, withdrawal of the drug usually leads to resolution of the hemolytic process. More than 80% of children with autoimmune hemolytic anemia recover spontaneously."}, {"id": "wiki20220301en352_31595", "title": "Fostamatinib", "score": 0.009900990099009901, "content": "Approval for treatment of autoimmune hemolytic anemia (AIHA) is in Stage 1 of Phase II trials. This study is a Phase 2, multi-center, open label, Simon two-stage study to evaluate the safety and efficacy of fostamatinib disodium in the treatment of warm antibody autoimmune hemolytic anemia. Primary outcome measures examined include a hemoglobin response measured by levels higher than 10 g/dL and 2 g/dL higher than the baseline hemoglobin. Responses were studied for a period of 12 weeks and for a dose of 150 mg in the morning and evening. The study began in April 2016 and is estimated to conclude in September 2017. The study is currently recruiting participants from U.S. states including Arizona, California, D.C., Massachusetts, New York, North Carolina, and Texas. Subjects must have had a diagnosis of primary or secondary warm antibody AIHA, and must have failed at least 1 prior treatment regimen for AIHA. Subjects cannot have a platelet count less than 30,000/μL, have AIHA secondary"}, {"id": "pubmed23n0085_1633", "title": "[Hereditary spherocytosis first diagnosed upon the development of aplastic crisis; a case report].", "score": 0.009900990099009901, "content": "We report a Childhood case of hereditary spherocytosis (HS) first diagnosed upon the development of aplastic crisis. A 6-year-old boy presented with fever and anemia. Although there was neither icterus nor splenomegaly at first, mild icterus and splenomegaly gradually developed with improvement of anemia. The diagnosis of HS was made on the basis of the presence of numerous spherocytes on the peripheral smear, increased osmotic fragility and the auto-hemolysis test result. The severe anemia in the early course with a marked decrease in the bone marrow erythroid cells and the absence of icterus and splenomegaly indicate that it was due to aplastic crisis. In the virological study, anti-human parvovirus (HPV) antibody titers were increased: the values of anti-HPV IgM were high and those of anti-HPV IgG were suddenly elevated. We thus considered that this HS case developed aplastic crisis by HPV infection."}, {"id": "Surgery_Schwartz_10036", "title": "Surgery_Schwartz", "score": 0.009895833333333333, "content": "cell assumes a more spherical, less deformable shape, and the spherocytic eryth-rocytes are sequestered and destroyed in the spleen and hemolytic anemia ensues. HS is inherited primarily (70–80% of the time) in an autosomal dominant fashion; the estimated prevalence in Western populations is roughly 1 in 2000.26Patients with typical HS forms may have mild jaundice. Splenomegaly usually is palpable on physical examination. Laboratory examination reveals varying degrees of anemia: patients with mild forms of the disease may not have anemia; patients with moderate to severe forms may have hemoglobin levels as low as 4 to 6 g/dL. The mean corpuscular volume is typically low to normal or slightly decreased. For screening, a combined elevated mean corpuscular hemoglobin concentra-tion and an elevated erythrocyte distribution width are an excel-lent predictor. Other laboratory indicators of HS include those providing evidence of rapid red blood cell destruction, includ-ing elevated"}, {"id": "wiki20220301en092_32685", "title": "Paroxysmal cold hemoglobinuria", "score": 0.009806042252505587, "content": "For intravascular hemolysis, the laboratory parameters include increased serum free hemoglobin, lactate dehydrogenase, unconjugated bilirubin, and reduced haptoglobin. Urine tests may show elevated hemoglobinuria and hemosiderinuria in chronic cases. Reticulocytosis may not be apparent in the acute phase or when there is viral-induced myelosuppression. Once the clinical suspicion of autoimmune hemolytic anemia is made, direct antiglobulin test (DAT) or direct Coombs' test is the first line of investigation to confirm the presence of warm autoantibodies. Testing with polyspecific and IgG-specific antiglobulin agents is usually negative, and that with C3-specific agent may be positive. On excluding warm autoimmune hemolytic anemia (WAIHA), the cold agglutinin titer should be examined for cold agglutinin disease (CAD). The diagnosis of PCH is suspected when both WAIHA and CAD are excluded. The complement level is usually low."}, {"id": "pubmed23n0574_16560", "title": "Hepatocellular carcinoma with chronic B-type hepatitis complicated by autoimmune hemolytic anemia: a case report.", "score": 0.00980392156862745, "content": "A 57-year-old man consulted a local hospital because of a persistent slight fever. At the age of 37 years he was diagnosed having B-type hepatitis, but left the liver dysfunction untreated. Twenty years later, he was diagnosed having chronic hepatitis B, hepatocellular carcinoma (HCC) and macrocytic anemia, and referred to our hospital for further investigation. A HCC with a maximum diameter of 5.2 cm was detected in segment 8. Results of blood tests included 1.8 mg/dL serum total bilirubin, 0.9 mg/dL bilirubin, less than 10 mg/dL haptoglobin, 7.9 g/dL hemoglobin, 130 fL MCV, and 14.5% reticulocytes. A bone marrow sample showed erythroid hyperplasia. The direct Coombs test gave a positive result. We diagnosed the anemia as autoimmmune hemolytic anemia (AIHA), for which prednisolone could not be administered due to positivity for HBsAg and HBeAg. After preparation of washed blood cells for later transfusion, the patient underwent systematic resection of segment 8. The cut surface of the resected specimen demonstrated an encapsulated yellow-brownish tumor measuring 52 mm multiply 40 mm which was diagnosed pathologicaly as moderately differentiated HCC. On the 9th postoperative day, the patient's temperature rose to 38 centigrade, and exacerbated hemolysis was observed. The maximum total bilirubin value was 5.8 mg/dL and minimum hemoglobin level was 4.6 g/dL. He tolerated this period without blood transfusion. Currently he is being followed up as an outpatient, and shows no signs of HCC recurrence or symptoms of anemia. AIHA associated with HBV infection has been described in only three previous cases, and the present case is the first in which surgery was performed for accompanying HCC."}, {"id": "pubmed23n0257_4573", "title": "[Hereditary spherocytosis: clinical characteristics and treatment with splenectomy].", "score": 0.00980392156862745, "content": "To analyse the clinico-biological characteristics at diagnosis, the clinical course, and the response to splenectomy of a series of patients with hereditary spherocytosis (HS). The clinical records of 61 patients diagnosed of HS along 30 years were reviewed. The diagnosis was based upon the existence of family history, physical findings, blood cell examination, reticulocyte count, peripheral blood spherocytes, red-cell osmotic fragility, auto-haemolysis, serum haptoglobin, LDH, non-conjugated bilirubin and direct anti-human globulin test. Data regarding the time of diagnosis and clinical course were taken into account in every case. Within the sub-group of 29 patients undergoing splenectomy, the changes in haemoglobin rates and reticulocyte and platelet counts after surgery were evaluated. Of the 61 patients, 35 were men and 26 women; the median age at diagnosis was 13 years (range: 0-64 years). Family history was positive in only 40% of the cases. The mean haemoglobin rate was 112 g/L (range: 46-151 g/L), over 60% of the patients having anaemia. The mean reticulocyte count was 282 x 10(9)/L (range: 31-583 x 10(9)/L), this being above 100 x 10(9)/L in 91% of the cases. Red-cell osmotic fragility with fresh blood was increased in 86% of the cases, and in 97% after blood incubation. Serum haptoglobins were decreased, whereas LDH was increased in 58% of the patients and non-conjugated bilirubin in 72%. Splenomegaly appeared in the clinical course in 87% of the patients; cholelithiasis was present in 31.5% of them. Haemoiytic crises were seen in 45% of patients, aplastic phases in 7%, and transfusion was needed by 16% of the patients to variable extents. Splenectomy was performed in 50% of the instances before 14 years of age (range: 4-64 years), and it increased haemoglobin rates in 40 g/L, anaemia being corrected in all cases; the mean reticulocyte count returned to normal, but thrombocytosis developed after surgery, it being present in 82% of the cases 2-3 months later. (1) Wide clinical variability is seen in HS, from severe forms requiring frequent transfusion to asymptomatic cases. (2) Highly frequent findings in HS are reticulocytosis and splenomegaly; relatively frequent were anaemia, haemolytic crisis and cholelithiasis, in this order. (3) Anaemia was always corrected after splenectomy, which also rose the haemoglobin rate even in the cases without anaemia, and returned the reticulocyte count to normal values."}, {"id": "wiki20220301en178_38961", "title": "Ranson criteria", "score": 0.009708737864077669, "content": "Acute pancreatitis not secondary to gallstones At admission: Blood glucose > 11.11 mmol/L (> 200 mg/dL) Age > 55 years Serum LDH > 350 IU/L Serum AST > 250 IU/L WBC count > 16000 cells/mm3 Within 48 hours: Serum calcium < 2.0 mmol/L (< 8.0 mg/dL) Hematocrit decreased by > 10% Oxygen (hypoxemia with PaO2 < 60 mmHg) BUN increased by 1.8 or more mmol/L (5 or more mg/dL) after IV fluid hydration Base deficit (negative base excess) > 4 mEq/L Sequestration of fluids > 6 L Acute pancreatitis secondary to gallstones At admission: Glucose > 220 mg/dl Age > 70 years LDH > 400 IU/L AST > 250 IU/ 100 ml WBC count > 18000 cells/mm3 Within 48 hours: Serum calcium < 8 mg/dL Hematocrit decreased by > 10% Base deficit > 4 mEq/L BUN increased by > 2 mg/dL Sequestered fluid > 6L"}, {"id": "pubmed23n0800_9003", "title": "Autoimmune hemolytic anemia induced by levofloxacin.", "score": 0.009615384615384616, "content": "Drug-induced autoimmune hemolytic anemia is a rare condition. We report the case of a 32-year-old white female who presented to the emergency department with generalized fatigue, fever, and jaundice. The patient reported using levofloxacin few days prior to presentation for urinary tract infection. The patient had evidence of hemolytic anemia with a hemoglobin of 6.7 g/dL which dropped to 5 g/dL on day 2, the direct Coombs test was positive, indirect bilirubin was 5.5 mg/dL, and LDH was 1283 IU/L. Further testing ruled out autoimmune disease, lymphoma, and leukemia as etiologies for the patient's hemolytic anemia. Levofloxacin was immediately stopped with a gradual hematologic recovery within few days. "}, {"id": "wiki20220301en020_33127", "title": "Hereditary spherocytosis", "score": 0.009523809523809525, "content": "Symptoms include anemia, jaundice, splenomegaly, and fatigue. Furthermore, the detritus of the broken-down blood cells – unconjugated or indirect bilirubin – accumulates in the gallbladder, and can cause pigmented gallstones to develop. In chronic patients, an infection or other illness can cause an increase in the destruction of red blood cells, resulting in the appearance of acute symptoms, a hemolytic crisis. On a blood smear, Howell-Jolly bodies may be seen within red blood cells. Primary treatment for patients with symptomatic HS has been total splenectomy, which eliminates the hemolytic process, allowing normal hemoglobin, reticulocyte and bilirubin levels. Spherocytosis patients who are heterozygous for a hemochromatosis gene may suffer from iron overload, despite the hemochromatosis genes being recessive."}, {"id": "pubmed23n0030_11754", "title": "[Prednisone therapy in patients with pernicious anemia (report of 2 cases)].", "score": 0.009523809523809525, "content": "The effects of Prednisone therapy at two patients with pernicious anemia were studied on haematologic response, gastric secretion and gastric mucosal hystology. The treatment resulted in each case in correctin of the megaloblastic to normoblastic erythropoesis, and increse in reticulocytes, hemoglobin level and red cell count. Schilling-s test was normalised in one case that means that Prednison enchanced B12 absorption. Gastric secretion of acid and gastric hystology were not respond during the Prednisone therapy. It was discused about immunological problems in pernicious anemia which are very important and required continued investigations."}, {"id": "pubmed23n0414_11794", "title": "Severe autoimmune cytopenias in treatment-naive hepatitis C virus infection: clinical description of 35 cases.", "score": 0.009433962264150943, "content": "To determine the clinical characteristics and outcome of patients with chronic hepatitis C virus (HCV) infection presenting severe autoimmune cytopenia unrelated to interferon alpha therapy, we analyzed characteristics and outcomes of 35 patients with HCV (16 from our departments and 19 from the literature). We considered active autoimmune hemolytic anemia (AHA) as a decrease of at least 2 g/dL in hemoglobin levels, an increase of at least 0.6 mg/dL in the serum unconjugated bilirubin level, a reticulocyte count &gt;5%, and a positive direct Coombs test. Severe neutropenia was defined as a neutrophil count &lt;0.5 x 10(9)/L, and severe thrombocytopenia as a platelet count &lt;30 x 10(9)/L. We identified the following cytopenias: AHA (17 cases), severe thrombocytopenia (16 cases), aplastic anemia (2 cases), severe neutropenia (1 case), refractory sideroblastic anemia (1 case), and pure red cell aplasia (1 case). Three patients simultaneously presented 2 types of severe cytopenias. Twenty-seven patients (77%) were female and 8 (23%) male, with a mean age at diagnosis of cytopenia of 51.7 years (range, 18-84 yr). Immunologic markers were detected in 19 (68%) of 28 patients, the most frequent being hypocomplementemia in 16 (57%), cryoglobulins in 15 (54%), antinuclear antibodies in 12 (43%), and rheumatoid factor in 5 (18%). Other associated processes were autoimmune diseases in 14 (50%) of 28 and human immunodeficiency virus (HIV) coinfection in 3 (9%) of 32. We found clinical and immunologic differences between HCV patients with AHA and those with severe thrombocytopenia. Patients with HCV-related AHA showed a higher prevalence of associated autoimmune diseases (71%), cryoglobulins (67%), and cirrhosis (59%). All had a good response to corticosteroids, but a poor prognosis (47% mortality). In contrast, patients with HCV-related severe thrombocytopenia had a lower prevalence of associated autoimmune diseases (11%), a poorer response to corticosteroids (55%), and lower mortality (6%), with HIV/HBV coinfections in some patients. The 35 cases presented demonstrate that different types of immune-mediated cytopenias may be severe and clinically significant in patients with HCV infection. Hemolytic anemia and severe thrombocytopenia were the most frequent cytopenias observed. Most patients responded well to corticosteroids, although a higher rate of mortality was observed in those with liver cirrhosis."}, {"id": "pubmed23n0589_8548", "title": "[Treatment and results of therapy in autoimmune hemolytic anemia].", "score": 0.009433962264150943, "content": "Basic principles in the therapy of idiopathic autoimmune hemolytic anemia induced by warm antibody were glucocorticoides and splenectomy. Immunosupresive drugs, plasmaferesis and intravenous high doses gamma globulin therapy are also useful. In secundary autoimmune hemolytic anemia induced by warm antibody we treated basic illness. During the period of 1990-1992 we treated 21 patients with primary autoimmune hemolytic anemia and 6 patients with secondary /4 CLL and 2 Non-Hodgkin's lymphoma/. Complete remission we found as a normalisation of reticulocites and hemoglobin level respectively. Complete remission by corticoides we got in 14/21 patients, partial response in 2/21 respectively. Complete response by splenectomy we got in 2/3 splenoctomized patients (idiopathic type). For successful treatment secondary hemolytic anemias we treated primary diseases (CLL and malignant lymphoma) and we got in 4/6 patients complete remission. Our results were standard in both type of autoimmune hemolytic anaemias induced by warm antibody."}, {"id": "pubmed23n0325_8334", "title": "[Severe hemolytic anemia with tear drop red cells as initial manifestation of Wilson's disease].", "score": 0.009345794392523364, "content": "A 16-year-old girl was admitted for a detailed examination of hemolytic anemia in November 1995. Initial laboratory findings included a total bilirubin concentration of 1.46 mg/dl, hemoglobin of 9.1 g/dl, and a reticulocyte count of 89/1000 percent. The plasma haptoglobin concentration was below 10 mg/dl. A blood smear showed many dacryocytes and a few echinocytes and codocytes. GOT was 71 IU/l; GPT, 44 IU/l; and LDH, 812 IU/l; the results of a hepaplastin test were 45% of normal. On further investigation, the level of serum ceruloplasmin was found to be 4 mg/dl, and of serum copper, 43 micrograms/dl. Urinary copper excretion was markedly increased, at 345 micrograms per day. Slit-lamp examination of both corneas revealed obvious Kayser-Fleischer rings. A liver biopsy sample showed fibrosis histologically and an elevated copper concentration of 535 micrograms/g dry weight and 183 micrograms/g wet weight. In family studies, the patient's asymptomatic 5-year-old sister was observed to have metabolic abnormalities consistent with Wilson's disease. These findings suggested that the patient's hemolytic anemia with red cell deformities was due to abnormal copper metabolism associated with Wilson's disease."}, {"id": "pubmed23n1027_21278", "title": "A rare case report of autoimmune haemolytic anemia in a female child due to a Donath-Landsteiner antibody.", "score": 0.009345794392523364, "content": "Paroxysmal cold hemoglobinuria is a rare form of autoimmune hemolytic anemia caused by the Donath-Landsteiner autoantibody. The condition is characterized by the presence of an IgG biphasic hemolysin with specificity to the P blood group antigen. The antibody biphasic action may be demonstrated in the Donath-Landsteiner test. While paroxysmal cold hemoglobinuria can be manifested at any age, it typically appears in children following a viral upper respiratory syndrome or immunization, though rarely. This report describes a 23-months old girl presented with 5 days history of fever, erythrocytopenia, leukocytosis and occurrence of dark urine. On admission, the physical examination showed pallor, no scleral icterus, a mild hyperemic throat and no hepatosplenomegaly. The investigations revealed severe anemia with hemoglobin of 44g/L, increased reticulocyte count (10.67%), elevated lactate dehydrogenase (2603IU/L), decreased serum haptoglobin (0.159g/L), normal G6PD. Direct antiglobulin test was positive with C3d and C3c complement components only. Direct and indirect Donath-Landsteiner tests were positive. The girl was treated with a intravenous immunoglobulin infusion and Cefotaxime. She received transfusion of red blood cells, crossmatched, although P antigen untyped. Despite this in vitro serological incompatibility she had a hemoglobin increase. The patient was discharged in stable condition on the seventh day following admission. Paroxysmal cold hemoglobinuria is a hemolytic anemia for which a specific diagnostic test is available. Timely recognition of the disease by pediatricians is crucial as well as the highly skilled hospital blood bank staff performing Donath-Landsteiner testing."}, {"id": "Pediatrics_Nelson_1643", "title": "Pediatrics_Nelson", "score": 0.009314301513790006, "content": "Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by hypogammaglobulinemia developing after an initial period of normal immune function, most commonly in the second and third decades of life (see Table 73-1). Serum IgG levels are less than 500 mg/dL (usually <300 mg/dL) with IgA levels less than 10 mg/dL and/or low IgM levels. Antibody titers to protein antigens, suchas tetanus and diphtheria, and to polysaccharide antigens,such as pneumococcus, are low or absent. T-cell numbersand function are highly variable, and B-cell numbers can be normal or low. Patients exhibit normal-sized or enlargedtonsils and lymph nodes and may have splenomegaly. Theyare susceptible to frequent respiratory tract infections due to Streptococcus pneumoniae, Haemophilus influenzae type b, and Mycoplasma. Gastrointestinal infections with Giardia, Campylobacter, Salmonella, Helicobacter, and enteroviruses are common. Autoimmune hemolytic anemia and thrombocytopenia occur"}, {"id": "pubmed23n0492_16489", "title": "Delayed hemolytic transfusion reaction due to anti-S antibody in patient with anti-Jk(a) autoantibody and multiple alloantibodies.", "score": 0.009259259259259259, "content": "We describe the case of a 60-year-old woman with a delayed hemolytic transfusion reaction (DHTR). She had a history of an ulcerative colitis, blood transfusion because of rectal bleeding, and surgical removal of descendent and sigmoid colon. At admission, laboratory data showed Hb 6.3 g/dL, reticulocytes 120 x 10(9)/L, serum total bilirubin 1.2 mg/dL (direct bilirubin: 0.2 mg/dL). Pretransfusion antibody screening procedures were positive. A monospecific autoanti-Jk(a) and three alloantibodies (anti-c, -E, -K) were identified by immunohematologic studies. The patient received two units of crossmatch compatible concentrated red blood cells. Six days later biochemical serum values showed Hb 6.2 g/dL, LDH 975 I.U./L and total bilirubin 2.95 mg/dL (direct 0.35 mg/dL). Crossmatches with red cell suspension of transfused blood units and a post-transfusion serum were repeatedly positive. Laboratory tests showed the presence of anti-S alloantobody in the serum and eluate. Moreover, pre-transfusion serum of the patient was retrospectively retested: anti-S was not detected. These data suggested a DHTR. The present case is unusual and interesting because of the association of a rare autoanti-Jk(a), non responsible for anemia, and four alloantibodies of which anti-S involved in a DHTR."}, {"id": "pubmed23n0942_1936", "title": "[Eperythrozoonosis complicated with hemophagocytic syndrome: report of four cases and review of literature].", "score": 0.009259259259259259, "content": "<bObjective:</b To analyze the clinical characteristics of eperythrozoonosis complicated with hemophagocytic syndrome (HPS) in 4 children. <bMethods:</b Four patients diagnosed with eperythrozoonosis complicated with HPS in the Children's Hospital Affiliated Capital Institute of Pediatrics during the period from June 2014 to July 2016 were enrolled. The clinical manifestations, laboratory examination data and therapeutic strategies were analyzed. A literature search (search terms included 'eperythrozoonosis' and 'hemophagocytic syndrome') was conducted using CNKI, Wanfang database, Chinese biomedical literature database and PubMed to include recently published studies (searched from the database establishment to January 2017). <bResults:</b Four patients were included in the study. One was boy and the other three were girls. The age range of the 4 patients was between 9 months and 17 years (9 months, 2 years and 17 years, 11 months respectively). All the patients presented with recurrent high fever. During the course of fever, 3 patients presented with rash, and 2 patients presented with joint pain and swelling, which mimicked systemic juvenile idiopathic arthritis. Only 1 patient had the contact history of infectious disease. All patients had normal or decreased white blood cell count ((0.80-13.12)×10<sup9</sup/L), suffered from varied degrees of anemia and showed the increased C reactive protein (13.0-84.7 mg/L) anderythrocyte sedimentation rate (13-72 mm/1 h). Examination of peripheral blood smears confirmed eperythrozoonosis. After fever continued about 1 month, all the 4 patients rapidly progressed. Among the 4 patients, 1 patient died for giving up further therapy, and the other 3 patients completely recovered after treatment, including azithromycin for the treatment of eperythrozoonosis, and high-dose intravenous methylprednisolone pulse therapy and human immunoglobulin for the treatment of HPS. For the disease not satisfactory, the hemophagocytic lymphohistiocytosis-2004 (HLH-2004) protocol is given. After the hospitalization of 1 to 2 months, the conditions improved and the children were discharged from hospital. Three patients were followed up for 8 months to 2 years, and their conditions were stable. In the PubMed database, no report was found. Nine cases of children with eperythrozoonosis were found in CNKI, Wanfang database and Chinese biomedical literature database, and 1 case was complicated with HPS. These findings, taken together our report, provided the data of 5 children with eperythrozoonosis complicated with HPS (4 cases were younger than 2 years old). A patient had contact history of infectious disease. Five patientss showed fever of unknown origin. All the patients had severe eperythrozoonosis, and 2 cases at younger age died. <bConclusions:</b Children with eperythrozoonosis often present with the protracted fever of unknown origin, and clinical manifestations mimic those of juvenile idiopathic arthritis (systemic type). The patients with eperythrozoonosis of mild-to-moderate disease severity may have a good prognosis. Children with severe eperythrozoonosis, especially those HPS cases with early onset before 2 years old, may have high risk of mortality. Once the patient's condition aggravates in the course of fever, HPS should be highly suspected. For the patients with eperythrozoonosis complicated with HPS, early diagnosis and the combination of anti-infection with the treatment of HPS are crucial for a good prognosis. For the treatment of HPS, HLH-2004 protocol is recommended."}, {"id": "wiki20220301en558_27134", "title": "Anemia in pregnancy", "score": 0.009174311926605505, "content": "MCV 80 - 100 fL - Iron deficiency - Infection - Hypothyroidism - Liver disease or alcohol use - Drug-induced - Hemolysis - Vitamin B12 or folate deficiency MCV > 100 fL - Vitamin B12 or folate deficiency - Drug induced - Liver disease or alcohol use - Hypothyroidism - Myelodysplastic Syndromes Pregnancy Pregnant women need almost twice as much iron as women who are not pregnant do. Not getting enough iron during pregnancy raises risk of premature birth or a low-birth-weight baby. Hormonal changes in the pregnant woman result in an increase in circulating blood volume to 100 mL/kg with a total blood volume of approximately 6000–7000 mL. While red cell mass increases by 15–20% during pregnancy, plasma volume increases by 40%. Hemoglobin levels less than 11 g/dL during the first trimester, less than 10.5 g/dL during the second and third trimesters and less than 10 mg/dL in the postpartum period are considered anemic."}]}}}}
{"correct_option": 5, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": true, "char_ranges": [[137, 364]], "word_ranges": [[20, 54]], "text": "The statement lists the criteria for ARDS (acute respiratory distress syndrome): PaO2/fiO2 < 200 + bilateral pulmonary infiltrates + PCP<18. So the diagnosis is respiratory distress and therefore the correct answer is option 5."}}, "full_answer": "Direct response question. The question tells the story of a patient with acute pancreatitis who is developing acute respiratory failure. The statement lists the criteria for ARDS (acute respiratory distress syndrome): PaO2/fiO2 < 200 + bilateral pulmonary infiltrates + PCP<18. So the diagnosis is respiratory distress and therefore the correct answer is option 5.", "full_answer_no_ref": "Direct response question. The question tells the story of a patient with acute pancreatitis who is developing acute respiratory failure. The statement lists the criteria for ARDS (acute respiratory distress syndrome): PaO2/fiO2 < 200 + bilateral pulmonary infiltrates + PCP<18. So the diagnosis is respiratory distress and therefore [HIDDEN].", "full_question": "A patient admitted for acute pancreatitis starts with tachypnea, tachycardia, sweating and progressive cyanosis. PaO2 is 55 mm Hg (PaO2/FiO2 ratio<200). CXR shows bilateral alveolar pulmonary infiltrates. Pulmonary capillary wedge pressure is normal. Oxygen therapy does not improve the situation. What is the most probable diagnosis:", "id": 75, "lang": "en", "options": {"1": "Nosocomial pneumonia.", "2": "Cardiac failure.", "3": "Carcinomatous lymphangitis.", "4": "Pulmonary thromboembolism.", "5": "Respiratory distress."}, "question_id_specific": 65, "type": "ANESTHESIOLOGY, CRITICAL CARE AND EMERGENCIES", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0069_19432", "title": "[Acute respiratory distress caused by distal neoplastic pulmonary emboli].", "score": 0.019324122479462285, "content": "In contrast to pulmonary parenchyma metastases or lymphangitic carcinomatosis, neoplastic emboli of small pulmonary arteries and capillaries frequently go unrecognized and are only discovered at autopsy. Five patients (48 +/- 12 years old) were admitted to 3 intensive care units for severe acute respiratory failure and died between the first and the tenth day following hospitalization. Each patient had a history of rapidly progressive dyspnea, and physical examination showed clinical evidence of right ventricular failure. The lungs were clear on chest X-rays and the ECG revealed sinus tachycardia with a right QRS axis. The mean partial pressures of oxygen (PaO2) and carbon dioxide (PaCO2) were, respectively, 50.8 +/- 9.1 mm Hg and 22.2 +/- 2.4 mm Hg. A swan-Ganz catheter, inserted into 4 patients, revealed pulmonary arterial hypertension (55, 43, 37, 28) with capillary wedge pressure within the normal limits and cardiac output normal or low (3.0, 3.8, 4.4, 5.0 l/min). Pulmonary angiograms from each patient showed decreased distal lung perfusion without any proximal defects suggestive of pulmonary embolism. The inferior vena cava always appeared clear. Malignant cells were found upon autopsy (4 cases) in the lumina of the pulmonary arterioles and the primary site of the cancer was determined in 3 patients (2 hepatomas and 1 pancreatic carcinoma). The last patient had a known breast cancer with bone marrow metastases and clinical, hemodynamic and angiographic evidence of neoplastic emboli. The clinical course of neoplastic emboli can suggest acute pulmonary embolism, but the diagnosis can only be advanced after pulmonary angiography, especially if the patient is to have a cancer.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0648_13195", "title": "[Extracorporeal membrane oxygenation for severe acute respiratory and heart failure in a child with severe pneumonia].", "score": 0.017345794392523366, "content": "To report clinical application of Extracorporeal membrane oxygenation for severe acute respiratory and heart failure in a child with severe pneumonia. A seven-year old male patient with severe pneumonia complicated with heart and lung function failure was admitted to PICU in 28th of December, 2008.Veno-artery access was set up via euthyphoria cannulation in operative incision. Blood was drained from the right atrium through a cannula introduced via femoral veins, and returned via femoral artery. The inter-surface of the ECMO equipment system was completely coated with heparin-coating technique. Anticoagulation was maintained with heparin to keep the activated clotting time (ACT) between 150 and 200 seconds and heparin usage dose was 10 U/(kg.h), mean blood flow was 1/2-2/3 of 80-120 ml/(kg.min) during ECMO assistant period. During ECMO, ventilator settings were gradually reduced to allow lung rest, i.e. peak inspiratory pressure less than 25 cm H2O (1 cm H2O=0.098 kPa), end expiratory pressure 8-10 cm H2O, rate 10-15 breaths per minute and FiO2 30%-40%. In management of ECMO, the incipient blood flow was set at 0.8 L/min, the radio of oxygen and blood flow was 1:1, FiO2 60%. After ten minutes of ECMO working, the blood oxygen saturation of radial artery increased from 40 mm Hg (1 mm Hg=0.133 kPa) to 177 mm Hg, Lac decreased from 3.5 mmol/L to 2.8 mmol/L. Four hours later, blood gas analysis of radial artery showed PaO2 202 mm Hg, PCO2 44 mm Hg, Lac 1.5 mmol/L, blood flow was set at 0.6 L/min, FiO2 60%, PaO2 kept above 150 mm Hg. 96 hours after ECMO supporting, the blood flow was set at 0.4 L/min [20 ml/(kg.min)], the results of blood gas analysis of radial artery was PaO2 190 mm Hg, PaCO2 36 mm Hg, SaO2 100%, Lac 0.9 mmol/L, then the child weaned off successfully from ECMO. Two days later, the child was successfully extubated. After two weeks treatment, the patient was discharged. The main complication associated with extracorporeal membrane oxygenation were bleeding. ECMO is an effective mechanical assistant therapy method for severe pulmonary and cardiac failure in a child."}, {"id": "pubmed23n0818_4672", "title": "Acute Respiratory Distress Syndrome diagnosis after coronary artery bypass: comparison between diagnostic criteria and clinical picture.", "score": 0.01686176142697882, "content": "Acute Respiratory Distress Syndrome (ARDS) is a potential complication of cardiac surgery, given that patients undergoing CABG frequently have hypoxemia and pulmonary dysfunction during initial hours after surgery. Thus, ARDS criteria in these patients are more likely to be positive while these criteria may not match the patient`s clinical picture. We aimed to investigate frequency of rapid onset hypoxemia in Pressure of Arterial Oxygen to Fractional Inspired Oxygen Concentration (PaO2/FiO2) less than 200 and diffuse pulmonary infiltrates as two diagnostic criteria forwards and compared these criteria with the clinical picture of the patients after Coronary Artery Bypass Graft (CABG) in this study. The study was prospective case series which carried out in about six months. All patients admitted to intensive care unit of Tehran Heart Center, who had undergone CABG on cardiopulmonary pump (CPB) recruited in the study. After considering inclusion criteria, age, sex, duration of intubation, arterial blood gas and chest radiography, on 24 hours and 48 hours after admission to the ICU were recorded. Then, patients with rapid onset of hypoxemia (PaO2/FiO2≤200mmHg) and diffuse pulmonary infiltrates and without sign or symptoms of obvious heart failure (probable positive ARDS cases) criteria were recorded and comparison between these probable positive cases with clinician`s clinical diagnosis (blinded to the study) was performed. In this study, a total of 300 patients after on-pump coronary artery bypass surgery were included. Postoperatively, 2 (0.66 %) in the 24 hours and 4 (1.33%) patients in 48 hours after surgery were positive for the two ARDS criteria according to the checklists, but; nobody had saved persistently ARDS criteria persistently during 48 hours after surgery. At the same time, clinician did not report any case of ARDS among 300 patients. In this study patients with ARDS criteria had no significant differences in age (P.value=0.937) and sex (P.value=0.533). Duration of intubation in patients with ARDS (14.26 ± 4.25 hours) in the first 48 hours was higher but not statistically different from the group without ARDS (11.60 ± 5.45 hours) (P.value=0.236). ARDS diagnosis based on rapid onset of hypoxemia (PaO2/FiO2≤200 mmHg) and diffuse pulmonary infiltrates and without signs or symptoms of obvious heart failure criteria in patients undergoing CABG could lead to overdiagnosis or misdiagnosis in less than 24 hours follow up. We recommend following patients for more than 24 hours and revise the current ARDS criteria for CABG patients. "}, {"id": "pubmed23n0980_2401", "title": "Acute Respiratory Failure in Renal Transplant Recipients: A Single Center Experience.", "score": 0.015243206211463804, "content": "We evaluated the frequency and cause of acute respiratory failure in renal transplant recipients. Our single-center retrospective observational study included consecutive renal transplant recipients who were admitted to an intensive care unit for acute respiratory failure between 2011 and 2017. Acute respiratory failure was defined as oxygen saturation &lt; 92% or partial pressure of oxygen in arterial blood &lt; 60 mm Hg on room air and/or requirement of noninvasive or invasive mechanical ventilation. Of 187 renal transplant recipients, 35 (18.71%) required intensive care unit admission; 11 of these patients (31.4%) were admitted to the intensive care unit with acute respiratory failure. Six of these patients (54.5%) had pneumonia and had shown infiltrates on chest radiography, which were shown in a minimum of 3 zones of the lung (2 with Klebsiella pneumonia, 1 with Acinetobacter species, 1 with Proteus mirabilis, 2 with no microorganisms). The other reasons for acute respiratory failure were cardiogenic pulmonary edema (2 patients), acute respiratory distress syndrome (2 patients, due to acute pancreatitis and acute cerebrovascular thromboembolism), and exacerbation of chronic obstructive pulmonary disease (1 patient). Six patients (54.5%) needed invasive mechanical ventilation because of pneumonia (3 patients), cardiogenic pulmonary edema (2 patients), and cerebrovascular thromboembolism (1 patient). Hemodialysis was administered in 5 patients (45%). Six of 11 patients died due to pneumonia (3 p atients), cardiogenic pulmonary edema (2 patients), and cerebrovascular thromboembolism (1 patient). Among the 5 survivors, 3 (60%) had recovered previous graft function. Acute respiratory failure is associated with high mortality and morbidity in renal transplant recipients. Main causes of acute respiratory failure were bacterial pneumonia and cardiogenic pulmonary edema in our study population. Extended chemoprophylaxis for bacterial and fungal infection and early intensive care unit admission of patients with acute respiratory failure may improve outcomes."}, {"id": "pubmed23n0931_4919", "title": "Early Identification of Acute Respiratory Distress Syndrome in the Absence of Positive Pressure Ventilation: Implications for Revision of the Berlin Criteria for Acute Respiratory Distress Syndrome.", "score": 0.014588601439408676, "content": "To assess whether patients breathing spontaneously under standard oxygen could be recognized early as acute respiratory distress syndrome patients according to the current Berlin definition. A post hoc analysis from two prospective studies. Twenty-three French ICUs. All patients admitted for acute hypoxemic respiratory failure and treated with noninvasive ventilation were analyzed. Patients with cardiogenic pulmonary edema, acute exacerbation of chronic obstructive pulmonary disease, or hypercapnia were excluded. None. The PaO2/FIO2 ratio was estimated at admission under standard oxygen and then under noninvasive ventilation 1 hour after initiation and within the first 24 hours. Among the 219 patients treated with noninvasive ventilation for acute hypoxemic respiratory failure, 180 (82%) had bilateral infiltrates including 161 patients with PaO2/FIO2 less than or equal to 300 mm Hg under standard oxygen. Among them, 127 were treated with positive end-expiratory pressure of at least 5 cm H2O, and 120 (94%) fulfilled criteria for acute respiratory distress syndrome within the first 24 hours. The mortality rate of patients with bilateral infiltrates and PaO2/FIO2 less than or equal to 300 mm Hg under standard oxygen was 29%, a rate very close to that of intubated patients with acute respiratory distress syndrome in the Berlin definition. Almost all patients with pulmonary bilateral infiltrates and a PaO2/FIO2 less than or equal to 300 mm Hg under standard oxygen fulfilled the acute respiratory distress syndrome criteria under noninvasive ventilation within the first 24 hours. Their mortality rate was similar to that reported in the Berlin definition of acute respiratory distress syndrome. Therefore, spontaneous breathing patients with the acute respiratory distress syndrome criteria could be identified early without positive pressure ventilation."}, {"id": "wiki20220301en246_9470", "title": "Diffuse alveolar damage", "score": 0.014040630073274538, "content": "Berlin Criteria: as stated on UpToDate (2020) Timing: onset of respiratory symptoms within one week of a injury/insult. Chest Imaging: either chest x-ray or CT scan, must show bilateral opacities that cannot be fully explained by other conditions such as effusion, lung/lobar collapse, or lung nodules. Origin of Edema: respiratory failure that cannot be fully explained by cardiac failure or fluid overload, this needs objective assessment such as an echocardiogram. Impaired Oxygenation: this can be determined by looking at the ratio of arterial oxygen tension to fraction of inspired oxygen (PaO2/FiO2) that can be obtained based on an arterial blood gas test. Note: all PaO2/FiO2 ratios used in the determination of the severity of ARDS require that the patient be on a ventilator at a setting that includes 5 cm H2O or more of positive end-expiratory pressure (PEEP) or continuous positive airway pressure (CPAP)."}, {"id": "wiki20220301en019_79988", "title": "Acute respiratory distress syndrome", "score": 0.013646308451069827, "content": "Causes may include sepsis, pancreatitis, trauma, pneumonia, and aspiration. The underlying mechanism involves diffuse injury to cells which form the barrier of the microscopic air sacs of the lungs, surfactant dysfunction, activation of the immune system, and dysfunction of the body's regulation of blood clotting. In effect, ARDS impairs the lungs' ability to exchange oxygen and carbon dioxide. Adult diagnosis is based on a PaO2/FiO2 ratio (ratio of partial pressure arterial oxygen and fraction of inspired oxygen) of less than 300 mm Hg despite a positive end-expiratory pressure (PEEP) of more than 5 cm H2O. Cardiogenic pulmonary edema, as the cause, must be excluded."}, {"id": "pubmed23n0304_8300", "title": "Increased mortality of older patients with acute respiratory distress syndrome.", "score": 0.013618326118326118, "content": "To examine the relationship between age and mortality in ARDS patients and evaluate the importance of factors that increase the mortality of older ARDS patients. Prospective inception cohort study. Community-based referral hospital. Two hundred fifty-six ARDS patients identified from May 1987 to December 1990. ARDS was defined by the following: (1) PaO2/PAO2 &lt; or = 0.2; (2) pulmonary capillary wedge pressure &lt; or = 15 mm Hg; (3) total static thoracic compliance &lt; or = 50 mL/cm H2O; (4) bilateral infiltrates on chest radiograph; and (5) an appropriate clinical setting for ARDS. Comparison of organ failure, incidence of sepsis, patient demographics, arterial oxygenation, and level of support in those 55 years and younger and those older than 55 years of age. Withdrawal of support in patients who died. Seventy-two of 112 patients older than 55 years (64%) died vs 65 of 144 patients 55 years and younger (45%) (p = 0.002). Examination of patient groups using age identified older than 55 years as a \"cutpoint\" above which mortality was greater (p = 0.002). Older nonsurvivors did not differ from nonsurvivors 55 years or younger with respect to gender, smoking history, ARDS risk factors, ARDS identifying characteristics, APACHE II (acute physiology and chronic health evaluation), number of organ failures, or the incidence of sepsis. In the 48 h prior to death, nonsurvivors 55 years and younger had more organ failure (3.4 +/- 0.2 vs 2.8 +/- 0.2; p = 0.03), higher fraction of inspired oxygen (0.82 +/- 0.03 vs 0.68 +/- 0.03; p = 0.008), and higher positive end-expiratory pressure levels (13 +/- 1 vs 8 +/- 1; p = 0.001) than older nonsurvivors. Despite more severe expression of disease, only 32 (50%) nonsurvivors 55 years and younger had support withdrawn. Significantly more nonsurvivors older than 55 years (73%) had support withdrawn (p = 0.009). Even in the absence of chronic disease states, withdrawal was more likely for patients older than 55 years (21/51) than in those 55 years and younger (3/32; p &lt; 0.001). Mortality is significantly higher for patients with ARDS older than 55 years. Decisions to withdraw support are made more often in ARDS patients older than 55 years. These data suggest that age bias may influence decisions to withdraw support."}, {"id": "First_Aid_Step2_1083", "title": "First_Aid_Step2", "score": 0.013599090687667452, "content": "Phase 1 (acute injury): Normal physical exam; possible respiratory alkalosis. Phase 2 (6–48 hours): Hyperventilation, hypocapnia, widening A-a oxygen gradient. Phase 3: Acute respiratory failure, tachypnea, dyspnea, ↓ lung compliance, scattered rales, diffuse chest infltrates on CXR (see Figure 2.15-5). F IGU R E 2.1 5-5. AP CXR showing a diffuse alveolar filling pattern 2° to ARDS. (Reproduced, with permission, from Kasper DL et al. Harrison’s Principles of Internal Medicine, 16th ed. New York: McGraw-Hill, 2005: 1497.) ■ Phase 4: Severe hypoxemia unresponsive to therapy; ↑ intrapulmonary shunting; metabolic and respiratory acidosis. The criteria for ARDS diagnosis (according to the American-European Consensus Conference defnition) are as follows: Acute onset of respiratory distress. PaO2/FiO2 ratio ≤ 200 mmHg. Bilateral pulmonary infltrates on CXR. No evidence of cardiac origin (capillary wedge pressure < 18 mmHg or no clinical evidence of elevated left atrial pressure)."}, {"id": "pubmed23n0351_3696", "title": "Acute respiratory distress syndrome: frequency, clinical course, and costs of care.", "score": 0.013006756756756757, "content": "To define the occurrence rate of acute respiratory distress syndrome (ARDS) using established criteria in a well-defined general patient population, to study the clinical course of ARDS when patients were ventilated using a \"lung-protective\" strategy, and to define the total costs of care. A 3-yr (1993 through 1995) retrospective descriptive analysis of all patients with ARDS treated in Kuopio University Hospital. Intensive care unit in the university hospital. Fifty-nine patients fulfilled the definition of ARDS: Pao2/Fio2 &lt;200 mm Hg (33.3 kPa) during mechanical ventilation and bilateral infiltrates on chest radiograph. None. With a patient data management system, the day-by-day data of hemodynamics, ventilation, respiratory mechanics, gas exchange, and organ failures were collected during the period that Pao2/Fio2 ratio was &lt;200 mm Hg (33.3 kPa). The frequency of ARDS was 4.9 cases/100,000 inhabitants/yr. Pneumonia and sepsis were the most common causes of ARDS. Mean age was 43+/-2 yrs. At the time of lowest Pao2/Fio2, the nonsurvivors had lower arterial and venous oxygen saturations and higher arterial lactate than survivors, whereas there were no differences between the groups in other parameters. Multiple organ dysfunction preceded the worst oxygenation in both the survivors and nonsurvivors. The intensive care mortality was 37%; hospital mortality and mortality after a minimum 8 months of follow-up was 42%. The most frequent cause of death was multiple organ failure. The effective costs of intensive care per survivor were US $73,000. The outcome of ARDS is unpredictable at the time of onset and also at the time of the worst oxygenation. Keeping the inspiratory pressures low (30-35 cm H2O [2.94 to 3.43 kPa]) reduces the frequency of pneumothorax, and might lower the mortality. Most patients are young, and therefore the costs per saved year of life are low."}, {"id": "InternalMed_Harrison_20935", "title": "InternalMed_Harrison", "score": 0.012878787878787878, "content": "MAJOR COMPLICATIONS Cardiopulmonary Complications Ventilation-perfusion mismatching produces a fall in arterial Po2 early in the course. Increasing alveolar epithelial injury and capillary permeability result in increased pulmonary water content, which decreases pulmonary compliance and interferes with oxygen exchange. In the absence of pneumonia or heart failure, progressive diffuse pulmonary infiltrates and arterial hypoxemia occurring within 1 week of a known insult indicate the development of mild acute respiratory distress syndrome (ARDS) (200 mmHg < Pao2/Fio2 ≤ 300 mmHg), moderate ARDS (100 mmHg < Pao2/Fio2 ≤ 200 mmHg), or severe ARDS (Pao2/Fio2 ≤100 mmHg). Acute lung injury or ARDS develops in ~50% of patients with severe sepsis or septic shock. Respiratory muscle fatigue can exacerbate hypoxemia and hypercapnia. An elevated pulmonary capillary wedge pressure (>18 mmHg) suggests fluid volume overload or cardiac failure rather than ARDS. Pneumonia caused by viruses or by"}, {"id": "wiki20220301en010_32319", "title": "Respiratory failure", "score": 0.012597217658012926, "content": "This type of respiratory failure is caused by conditions that affect oxygenation, such as: Low ambient oxygen (e.g. at high altitude) Ventilation-perfusion mismatch (parts of the lung receive oxygen but not enough blood to absorb it, e.g. pulmonary embolism) Alveolar hypoventilation (decreased minute volume due to reduced respiratory muscle activity, e.g. in acute neuromuscular disease); this form can also cause type 2 respiratory failure if severe. Diffusion problem (oxygen cannot enter the capillaries due to parenchymal disease, e.g. in pneumonia or ARDS) Shunt (oxygenated blood mixes with non-oxygenated blood from the venous system, e.g. right to left shunt) Type 2 Hypoxemia (PaO2 <8kPa or normal) with hypercapnia (PaCO2 >6.0kPa). The basic defect in type 2 respiratory failure is characterized by: {| class=\"wikitable\" |PaO2 || decreased (< )or normal |- | PaCO2 || increased (> ) |- | PA-aO2 || normal |- |pH || <7.35 |}"}, {"id": "InternalMed_Harrison_20709", "title": "InternalMed_Harrison", "score": 0.012389139036703507, "content": "This type of respiratory failure occurs with alveolar flooding and subsequent intrapulmonary shunt physiology. Alveolar flooding may be a consequence of pulmonary edema, pneumonia, or alveolar hemorrhage. Pulmonary edema can be further categorized as occurring due to elevated pulmonary microvascular pressures, as seen in heart failure and intravascular volume overload or ARDS (“low-pressure pulmonary edema,” Chap. 322). This syndrome is defined by acute onset (≤1 week) of bilateral opacities on chest imaging that are not fully explained by cardiac failure or fluid overload and of shunt physiology requiring positive end-expiratory pressure (PEEP). Type I respiratory failure occurs in clinical settings such as sepsis, gastric aspiration, pneumonia, near-drowning, multiple blood transfusions, and pancreatitis. The mortality rate among patients with ARDS was traditionally very high (50–70%), although changes in patient care have led to mortality rates closer to 30% (see below)."}, {"id": "wiki20220301en358_2761", "title": "Fraction of inspired oxygen", "score": 0.012022050199390101, "content": "Equations Abbreviated alveolar air equation PAO2, PEO2, and PIO2 are the partial pressures of oxygen in alveolar, expired, and inspired gas, respectively, and VD/Vt is the ratio of physiologic dead space over tidal volume. Medicine In medicine, the FIO2 is the assumed percentage of oxygen concentration participating in gas exchange in the alveoli. Uses The FIO2 is used in the APACHE II (Acute Physiology and Chronic Health Evaluation II) severity of disease classification system for intensive care unit patients. For FIO2 values equal to or greater than 0.5, the alveolar–arterial gradient value should be used in the APACHE II score calculation. Otherwise, the PaO2 will suffice. The ratio between partial pressure of oxygen in arterial blood (PaO2) and FIO2 is used as an indicator of hypoxemia per the American-European Consensus Conference on lung injury. A high FIO2 has been shown to alter the ratio of PaO2/FIO2."}, {"id": "wiki20220301en250_13735", "title": "Maternal near miss", "score": 0.011674718196457327, "content": "Cardiovascular dysfunction a) Shock b) Cardiac Arrest c) Severe hypoperfusion (lactate >5 mmol/L or >45 mg/dL) d) Severe acidosis (pH<7.1) e) Use of continuous vasoactive drugs f) Cardio-pulmonary resuscitation Respiratory dysfunction g) Acute cyanosis h) Gasping i) Severe tachypnea (respiratory rate>40 breaths per minute) j) Severe bradypnea (respiratory rate<6 breaths per minute) k) Severe hypoxemia (O2 saturation <90% for ≥60min or PAO2/FiO2<200) l) Intubation and ventilation not related to anaesthesia Renal dysfunction m) Oliguria non responsive to fluids or diuretics n) Severe acute azotemia (creatinine >300 μmol/ml or >3.5 mg/dL) o) Dialysis for acute renal failure Coagulation dysfunction p) Failure to form clots q) Severe acute thrombocytopenia (<50,000 platelets/ml) r) Massive transfusion of blood or red cells (≥ 5 units) Hepatic dysfunction s) Jaundice in the presence of pre-eclampsia"}, {"id": "wiki20220301en101_49425", "title": "Pulmonary wedge pressure", "score": 0.010827848335959952, "content": "Noninvasive estimation techniques have been proposed. Clinical significance Because of the large compliance of pulmonary circulation, it provides an indirect measure of the left atrial pressure. For example, it is considered the gold standard for determining the cause of acute pulmonary edema; this is likely to be present at a PWP of >20mmHg. It has also been used to diagnose severity of left ventricular failure and mitral stenosis, given that elevated pulmonary capillary wedge pressure strongly suggests failure of left ventricular output. Traditionally, it was believed that pulmonary edema with normal PWP suggested a diagnosis of acute respiratory distress syndrome (ARDS) or non cardiogenic pulmonary edema (as in opiate poisoning). However, since capillary hydrostatic pressure exceeds wedge pressure once the balloon is deflated (to promote a gradient for forward flow), a normal wedge pressure cannot conclusively differentiate between hydrostatic pulmonary edema and ARDS."}, {"id": "First_Aid_Step2_1080", "title": "First_Aid_Step2", "score": 0.010537654552266768, "content": "Removal of the extrinsic cause or treatment of underlying infection if identifed. Corticosteroid treatment may be used if no cause is identif ed. Causes include ventilation-perfusion (V/Q) mismatch, right-to-left shunt, hypoventilation, low inspired O2 content (important at altitudes), and diffusion impairment. Findings depend on the etiology. ↓HbO2 saturation, cyanosis, tachypnea, shortness of breath, pleuritic chest pain, and altered mental status may be seen. Pulse oximetry: Demonstrates ↓ HbO2 saturation. CXR: To rule out ARDS, atelectasis, or an infltrative process (e.g., pneumonia) and to look for signs of pulmonary embolism. ABGs: To evaluate PaO2 and to calculate the alveolar-arterial (A-a) oxygen gradient ([(Patm − 47) × FiO2 − (PaCO2/0.8)] − PaO2). An ↑ A-a gradient suggests a V/Q mismatch or a diffusion impairment. Figure 2.15-4 summarizes the approach toward hypoxemic patients. Is PaCO2 increased? Hypoventilation Yes Yes No No Is PAO2 − PaO2 increased?"}, {"id": "pubmed23n0033_6956", "title": "Studies on pulmonary function in patients during respiratory treatment. Diagnostic and prognostic evaluations.", "score": 0.009900990099009901, "content": "Twenty-nine patients, divided into three groups: 1) chronic obstructive pulmonary disease; 2) acute or chronic pulmonary disease with left heart failure; 3) respiratory insufficiency after peritonitis, pancreatitis, and/or sepsis, were studied during respirator treatment with regard to gas exchange, breathing mechanics and central circulation. The dead space ventilation was somewhat greater in group 1 than in the other groups. The alveolar-arterial oxygen tension difference was least in group 1, greater in group 2 and extremely high in group 3. Neither dynamic compliance of the thorax nor inspiratory resistance showed any significant differences between the groups. The cardiac output had the highest values in group 3. The venous admixture was generally small in group 1 and extremely large in group 3. The pulmonary artery pressures were highest in group 2. Three variables proved to be valuable when assessing the prognosis of a patient: a large venous admixture; a large alveolar-arterial oxygen tension difference, and a high pulmonary artery pressure indicated a less favourable prognosis."}, {"id": "pubmed23n0641_23073", "title": "THE TREATMENT OF ANOXEMIA IN PNEUMONIA IN AN OXYGEN CHAMBER.", "score": 0.00980392156862745, "content": "1. The use of an oxygen chamber in the treatment of pneumonia patients makes it possible to administer this gas for long periods of time under exactly known conditions. The medical and nursing care of the patient is greatly facilitated. 2. Prolonged inhalation of oxygen varying from 40 to 60 per cent appears to be without harm. 3. Oxygen administered to intensely anoxemic patients almost immediately clears up this anoxemia. Cyanosis disappears with the anoxemia. 4. The removal of patients from the high oxygen while they are still sick and while examination shows that there are still extensive edema and infiltration of the lung results in a return of the intense anoxemia. 5. It is sometimes impossible to clear up the anoxemia, even when as high as 60 per cent of oxygen is given, especially when there are considerable edema and infiltration of the lungs. 6. Five cases in which the prognosis was grave recovered. Three cases, one of tuberculosis, one with a Pneumococcus Type III infection, and a third with a pneumonia superimposed on a chronic pulmonary condition, died. 7. In all cases there appeared to be an improvement in the patient's condition. In one case, particularly, with an intense degree of anoxemia, the patient became moribund and pulseless. Following the administration of 60 per cent of oxygen there was a lowering of the heart rate from 160 to 120, the return of the pulse to the radial artery, the color became bright pink, and there was a remarkable change in the clinical condition. 8. The anoxemia of pneumonia is due, in large measure, to an impairment of the respiratory surface of the lungs. The greater the lung involvement the greater the anoxemia. Especially is this so when the pneumonic process extends throughout the lungs so that there are many patches of bronchopneumonia, with accompanying bronchitis and edema, as evidenced by the presence of râles throughout the lungs. 9. Rapid and shallow breathing of the degree observed in pneumonia is, as far as present evidence shows, of less importance in the production of anoxemia."}, {"id": "wiki20220301en018_60928", "title": "Extracorporeal membrane oxygenation", "score": 0.009708737864077669, "content": "Guidelines that describe the indications and practice of ECMO are published by the Extracorporeal Life Support Organization (ELSO). Criteria for the initiation of ECMO vary by institution, but generally include acute severe cardiac or pulmonary failure that is potentially reversible and unresponsive to conventional management. Examples of clinical situations that may prompt the initiation of ECMO include the following: Hypoxemic respiratory failure with a ratio of arterial oxygen tension to fraction of inspired oxygen (PaO2/FiO2) of <100 mmHg despite optimization of the ventilator settings, including the fraction of inspired oxygen (FiO2), positive end-expiratory pressure (PEEP), and inspiratory to expiratory (I:E) ratio Hypercapnic respiratory failure with an arterial pH <7.20 Refractory cardiogenic shock Cardiac arrest Failure to wean from cardiopulmonary bypass after cardiac surgery As a bridge to either heart transplantation or placement of a ventricular assist device"}, {"id": "pubmed23n0312_22146", "title": "Inhaled nitric oxide in acute respiratory distress syndrome.", "score": 0.009523809523809525, "content": "Inhaled nitric oxide has been shown to improve oxygenation in select patients with acute respiratory distress syndrome (ARDS). The purpose of this study was to evaluate the clinical response to four concentrations of inhaled nitric oxide (NO) in 20 patients with ARDS. All patients with ARDS were eligible for the study. ARDS was defined as (1) the presence of a predisposing factor; (2) a PaO2/FiO2 ratio &lt; 200; (3) bilateral infiltrates on chest radiograph; and (4) absence of evidence of congestive heart failure and pulmonary artery wedge pressure &lt; 18 mm Hg. Patients received each of four doses (1, 15, 30, and 60 ppm) in random order, each for a 3-hour period. Cardiovascular variables were continuously monitored, and arterial and mixed venous blood gas measurements were obtained at 30 minutes and 3 hours. Thirteen of the 20 patients demonstrated a significant increase in their PaO2/FiO2 (&gt; 20% increase) when treated with inhaled NO. The administration of inhaled NO was associated with an increase in oxygenation at doses of 1, 15, and 30 ppm, but not 60 ppm. Increasing NO dose to more than 1 ppm did not significantly improve response. Mean pulmonary artery pressure decreased with increasing NO concentration, but this did not reach statistical significance. Nine of the 13 responding patients and 2 of the 7 nonresponding patients survived. Inhaled NO was successful in increasing PaO2/FiO2 by &gt; 20% in 65% of the surgical patients in this trial. Response to NO could not be predicted by initial PaO2/FiO2 or pulmonary artery pressures. A trial of inhaled NO at a dose of &lt; 10 ppm may be helpful in ARDS patients requiring increasing FiO2 and positive end-expiratory pressure."}, {"id": "pubmed23n0918_14369", "title": "High peak PaO2 values associated with adverse outcome in patients treated with noninvasive ventilation for acute cardiogenic pulmonary edema and pneumonia.", "score": 0.009433962264150943, "content": "Noninvasive ventilation (NIV) has a sigificant impact on mortality in acute respiratory failure (ARF). Predictive parameters for mortality are of high interest. We retrospectively analyzed 3759 blood gas analysis and clinical parameters of 475 patients presenting with ARF based on acute cardiogenic pulmonary edema and/or pneumonia. The influence of peak arterial oxygen partial pressure levels (PaO2) with respect to its predictive value for in-hopital and long-term mortality was investigated. Overall intra-hospital mortality was 24%. Peak PaO2 levels in kPa were significantly higher in non-survivors (20.01±10.11) compared to survivors (15.65±6.79, P&lt;0.001). A univariate Cox proportional-hazards analysis for long-term mortality revealed associations with maximum PaO2 levels (overall cohort: HR= 1.02; 95% CI: 1.007-1.03; P=0.003; CPE: HR= 1.02; 95% CI: 0.99-1.04, P=0.05, pneumonia: HR= 1.02; 95% CI: 1-1.4, P=0.02). A PaO2 cut-off value of 13 kiloPascal (kPa) was calculated by means of Youden Index and remained true even after correction for APACHE 2 Score (HR= 1.50; 95% CI: 1.00-2.25; P=0.05) and for PaCO2 (HR= 1.63; 95% CI: 1.14-2.33; P=0.01). Peak PaO2 levels were associated with worse in-hopital and long-term mortality in patients treated with NIV due to ARF. These findings may indicate that application of high oxygen may be detrimental in such patients."}, {"id": "pubmed23n0083_16662", "title": "[Prognosis of chronic obstructive pulmonary disease after acute respiratory failure].", "score": 0.009259259259259259, "content": "We followed the course of 53 patients (male 38, female 15 and average age 66 +/- 10) with chronic obstructive pulmonary disease hospitalized in our intensive care unit due to acute episode of respiratory failure. Etiologies of acute respiratory failure, patient's clinical data and arterial blood gases analysis were recorded. Most of the results showed no significant difference between the initial survival and the non-survival group. Low hematocrit. hypoalbulinemia and severe hypoxemia were found in the dead group but relative hypercapnea in the survival group was noted. Infection was the most common cause of acute respiratory failure and following by congestive heart failure, pneumothorax and asphyxia. Intubation and mechanical ventilator were needed in all patients except 3 cases to whom only conservative treatment was given. Hospital mortality was 50.9 percent (27/53) and most of the cases (22/53) died in the intensive care unit. Twenty six cases (49.1%) were discharged from the hospital and follow-up was continued for at least two years. Fifteen patients died during two year follow-up and except for one suicide case all died of repeated respiratory failure. The two year survival was only 20.8 percent (11/53). The prognosis of patients with chronic obstructive pulmonary disease following an episode of acute respiratory failure is poor."}, {"id": "pubmed23n0212_4594", "title": "[Acute respiratory failure in a medical intensive care unit. Incidence and prognosis].", "score": 0.009174311926605505, "content": "Acute respiratory failure had occurred in 89 of 1594 patients in a medical intensive care unit (5.6%), 26.8% of all patients (332) on long-term mechanical ventilation. Compared with the other chronically ventilated patients those with acute respiratory failure averaged a lower age, the proportion of women was higher and the duration of ventilation longer. The death rate was significantly higher (78.7% compared with 58.3%). The important prognostic factors included the underlying disease, additional abnormal organ function, severity of pulmonary gas exchange abnormality, and advanced age. If there was septicaemia, peritonitis, liver cirrhosis with bleeding oesophageal varices or polytrauma with acute renal failure the death rate was over 80%; after hypovolaemic shock, pancreatitis or postoperative pulmonary failure it was less than 65%. Patients who had abnormal function of at most one other organ in addition and an inspiratory arterial pO2 difference below 250 mm Hg, measured 12 hours after onset of mechanical ventilation, had a relatively favourable prognosis with a death rate of 33%, while in the other groups of patients it was 86-100%."}, {"id": "First_Aid_Step2_1084", "title": "First_Aid_Step2", "score": 0.009122267475687807, "content": "PaO2/FiO2 ratio ≤ 200 mmHg. Bilateral pulmonary infltrates on CXR. No evidence of cardiac origin (capillary wedge pressure < 18 mmHg or no clinical evidence of elevated left atrial pressure). There is no standard treatment. Treat the underlying disease and maintain adequate perfusion to prevent end organ damage. Minimize injury induced by mechanical ventilation by ventilating with low tidal volumes. Use PEEP to recruit collapsed alveoli and titrate PEEP and FiO2 to achieve adequate oxygenation. Goal oxygenation is PaO2 > 60 mmHg or SaO2 > 90% on FiO2 ≤ 0.6. Slowly wean patients from ventilation, and follow with extubation trials (see Table 2.15-6). Pulmonary hypertension is defned as a mean pulmonary arterial pressure of > 25 mmHg (normal = 15 mmHg). It is classifed as either 1° (if the etiology"}, {"id": "pubmed23n0070_21246", "title": "Factors affecting perioperative pulmonary function in acute respiratory failure.", "score": 0.00909090909090909, "content": "To determine the magnitude, duration, and associated factors of perioperative changes in pulmonary function, we retrospectively reviewed the medical records of 145 patients who required preoperative mechanical ventilation for acute respiratory failure before undergoing 200 surgical procedures. Patients were grouped into five pulmonary diagnostic categories: (1) adult respiratory distress syndrome (ARDS) (n = 49); (2) pneumonia (n = 20); (3) atelectasis (n = 65); (4) congestive heart failure (n = 11); and (5) acute ventilatory failure (n = 55). Sixty patients underwent intra-abdominal surgery, 135 patients required surgery on the periphery, and five patients had a thoracotomy. For all patients, PaO2/FIO2 declined significantly from 321 mm Hg (mean) preoperatively to 258 mm Hg intraoperatively, and shunt fraction (Qs/QT) increased from 0.16 to 0.23 without a significant change in PaCO2. The magnitude of the increase in Qs/QT did not differ among pulmonary diagnostic groups. Preoperatively, patients undergoing laparotomy had lower PaO2/FIO2 (278 vs 340) and higher Qs/QT (0.19 vs 0.14) than patients requiring surgery on the periphery. Intraoperatively, Qs/QT increased more during abdominal procedures than during peripheral procedures. Intraoperative hypoxemia (PaO2/FIO2 less than 80 mm Hg) occurred during 13 procedures. Hypoxemic patients had a mean increase in Qs/QT of 0.20 (0.25 preoperatively to 0.45 intraoperatively), and a significant increase in PaCO2 from 38 mm Hg to 45 mm Hg intraoperatively). In general, these patients had ARDS (n = 10), sepsis (n = 10), a laparotomy (n = 9), and intraoperative mechanical ventilation via the Ohio Anesthesia ventilator (n = 8), a commonly used operating room ventilator. Their preoperative peak airway pressure (54 cm H2O) and minute ventilation (20 L/min) requirements exceeded the capabilities of the Ohio Anesthesia ventilator and likely contributed to impaired gas exchange intraoperatively. Within the first several hours postoperatively, PaO2/FIO2 recovered to preoperative levels in all patients, even in those who had severe intraoperative hypoxemia develop and who underwent laparotomy. We conclude that most patients with acute respiratory failure receiving preoperative mechanical ventilation experienced mild-to-moderate deterioration in intraoperative pulmonary oxygen exchange that rapidly returned to preoperative levels after surgery. We recommend that necessary surgery not be postponed by concern that pulmonary function will be worsened by surgery and anesthesia."}, {"id": "pubmed23n0806_6557", "title": "Effects of high-volume hemofiltration on alveolar-arterial oxygen exchange in patients with refractory septic shock.", "score": 0.00909090909090909, "content": "High-volume hemofiltration (HVHF) is technically possible in severe acute pancreatitis (SAP) patients complicated with multiple organ dysfunction syndrome (MODS). Continuous HVHF is expected to become a beneficial adjunct therapy for SAP complicated with MODS. In this study, we aimed to explore the effects of fluid resuscitation and HVHF on alveolar-arterial oxygen exchange, the Acute Physiology and Chronic Health Evaluation II (APACHE II) score in patients with refractory septic shock. A total of 89 refractory septic shock patients, who were admitted to ICU, the Provincial Hospital affiliated to Shandong University from August 2006 to December 2009, were enrolled in this retrospective study. The patients were randomly divided into two groups: fluid resuscitation (group A, n=41), and fluid resuscitation plus high-volume hemofiltration (group B, n=48). The levels of O2 content of central venous blood (CcvO2), arterial oxygen content (CaO2), alveolar-arterial oxygen pressure difference P(A-a)DO2, ratio of arterial oxygen pressure/alveolar oxygen pressure (PaO2/PaO2), respiratory index (RI) and oxygenation index (OI) were determined. The oxygen exchange levels of the two groups were examined based on the arterial blood gas analysis at different times (0, 24, 72 hours and 7 days of treatment) in the two groups. The APACHE II score was calculated before and after 7-day treatment in the two groups. The levels of CcvO2, CaO2 on day 7 in group A were significantly lower than those in group B (CcvO2: 0.60±0.24 vs. 0.72±0.28, P&lt;0.05; CaO2: 0.84±0.43 vs. 0.94±0.46, P&lt;0.05). The level of oxygen extraction rate (O2ER) in group A on the 7th day was significantly higher than that in group B (28.7±2.4 vs. 21.7±3.4, P&lt;0.01). The levels of P(A-a)DO2 and RI in group B on the 7th day were significantly lower than those in group A. The levels of PaO2/PaO2 and OI in group B on 7th day were significantly higher than those in group A (P&lt;0.05 or P&lt;0.01). The APACHE II score in the two groups reduced gradually after 7-day treatment, and the APACHE II score on the 7th day in group B was significantly lower than that in group A (8.2±3.8 vs. 17.2±6.8, P&lt;0.01). HVHF combined with fluid resuscitation can improve alveolar-arterial-oxygen exchange, decrease the APACHE II score in patients with refractory septic shock, and thus it increases the survival rate of patients."}, {"id": "pubmed23n1105_25021", "title": "The Influence of Hypercapnia and Atmospheric Pressure on the Pao2/Fio2 Ratio-Pathophysiologic Considerations, a Case Series, and Introduction of a Clinical Tool.", "score": 0.009009009009009009, "content": "The ratio between Pao2 and Fio2 is used as a marker for impaired oxygenation and acute respiratory distress syndrome classification. However, any discrepancy between Fio2 and o2 fraction in the alveolus affects the Pao2/Fio2 ratio. Correcting the Pao2/Fio2 ratios using the alveolar gas equation may result in an improved reflection of the pulmonary situation. This study investigates the difference between standard and corrected Pao2/Fio2 in magnitude, its correlation with the mortality of acute respiratory distress syndrome classification, and trends over time. A register and a retrospective study combined with the development of a mathematical model to determine the difference between standard and corrected Pao2/Fio2 ratio for various levels of Paco2 and atmospheric pressure. ICU in a secondary hospital in The Netherlands. Patients admitted to the ICU for pneumonia or acute respiratory distress syndrome. Register cohort: January 1, 2010, till March 1, 2020 (n = 1008). Retrospective cohort: March 1, 2020, till June 1, 2020 (n = 34). The register was used to determine the 7-day ICU mortality per acute respiratory distress syndrome classification based on the standard and corrected Pao2/Fio2 ratio. The retrospective dataset correlated the Paco2 with Pao2/Fio2 ratio over time in patients with assumed stable oxygenation. The model demonstrated an increased difference between the standard and corrected Pao2/Fio2 ratios by a lower Fio2 and atmospheric pressure and higher Pao2 and Paco2. Reclassification of severe acute respiratory distress syndrome resulted in an increase in mortality from 28.1% for standard Pao2/Fio2 to 30.6% for corrected Pao2/Fio2 ratios. Acute Physiology and Chronic Health Evaluation scores correlated better with 7-day ICU-mortality when corrected Pao2/Fio2 ratio was used for classification. For patients with Fio2 less than 50% (n = 55), change in Paco2 correlated with change in Pao2/Fio2 ratio (r = -0.388; p = 0.003). A corrected Pao2/Fio2 ratio was calculated. Correcting the Pao2/Fio2 ratio for the alveolar gas equation predominantly affects patients with high ratios between Pao2 and Fio2 and Paco2 and at low atmospheric pressure. Using the corrected Pao2/Fio2 ratio for acute respiratory distress syndrome classification results in improved correlation with the 7-day ICU mortality and increases generalization among acute respiratory distress syndrome studies. The authors provide a free, web-based tool."}, {"id": "article-27355_19", "title": "Atypical Bacterial Pneumonia -- Treatment / Management -- Criteria for Admission", "score": 0.009009009009009009, "content": "Respiration rate more than 30 bpm Oxygen saturation less than 90% on room air Hypotension Severe lung disease, COPD, emphysema, Heart failure, diabetes Altered mental status Delirium"}, {"id": "pubmed23n0571_17690", "title": "Acute respiratory distress following liposuction.", "score": 0.008928571428571428, "content": "An active duty male presented to the emergency room with dyspnea for 2 days after undergoing liposuction surgery. Upon presentation, the patient was afebrile, tachycardic, tachypneic, and hypoxemic. The initial chest radiograph demonstrated bilateral patchy opacities and the PaO2/FiO2 ratio was &lt;200. The patient was admitted to the medical intensive care unit for supportive care. He was treated empirically for pneumonia. Blood and sputum cultures were negative. A computed tomography angiogram of the chest was negative for pulmonary embolism but did reveal a bilateral, perihilar air space process. The patient's oxygen requirement improved and the abnormal chest radiographic findings resolved over the next 48 hours. Given his clinical presentation, negative workup, and rapid recovery, the patient was given a presumptive diagnosis of pulmonary fat embolism. Fat embolism occurs when adipocytes and small blood vessels are damaged during the liposuction procedure. Patients may present with low-grade fever, tachycardia, tachypnea, hypoxemia, and hypocapnia. The differential diagnosis includes venous thromboembolism, aspiration pneumonitis, and pneumonia. The mainstay of treatment for pulmonary fat embolism is supportive care. The risk of mortality is 5 to 15%."}, {"id": "pubmed23n0572_3861", "title": "Characteristics of community-acquired pneumonia in patients with chronic obstructive pulmonary disease.", "score": 0.008928571428571428, "content": "Community-acquired pneumonia is a frequent event in the course of chronic obstructive pulmonary disease (COPD). The aim of the present study was to provide information on clinical and microbiological characteristics and outcome of community-acquired pneumonia in these patients, in a comparative study with the non-COPD population. Prospective study of cases. A university hospital in Lleida, Spain. During a 6 year-period, we prospectively studied the clinical and radiological manifestations, microbiological data and outcome of all patients with community-acquired pneumonia. A comparative analysis of characteristics of pneumonia between 132 patients with a definitive diagnosis of COPD and 575 patients who did not have this underlying disease was performed. COPD was associated with an older and predominantly male population. These patients frequently had concomitant comorbidities such as diabetes mellitus or chronic heart failure. Clinical presentation was more severe, manifested by septic shock, tachypnea, lower values of pH, pO(2) and oxygen saturation, and greater values of pCO(2). Purulent expectoration was also more frequent in this subset of patients. Admission was usually required for patients with COPD, and length of hospitalization was significantly increased; however, difference in the mortality rate was not observed. Although the spectrum of responsible microorganisms was very similar, the incidence of Pseudomonas aeruginosa and other Gram-negative bacilli was increased in COPD, particularly among patients with advanced situation and/or oral corticosteroid treatment. Community-acquired pneumonia in patients with COPD was associated with epidemiological and clinical particularities mainly related to the underlying disease but showed only minor differences in outcome parameters. Gram-negative bacilli and P. aeruginosa are potential pathogens that need to be considered."}, {"id": "pubmed23n0327_15171", "title": "Determinants of myocardial performance after blunt chest trauma.", "score": 0.008849557522123894, "content": "This study investigates whether factors that determine myocardial performance (preload, afterload, heart rate, and contractility) are altered after isolated unilateral pulmonary contusion. Catheters were placed in the carotid arteries, left ventricles, and pulmonary arteries of anesthetized, ventilated (FiO2=0.5) pigs (31.2+/-0.6 kg; n=26). A unilateral, blunt injury to the right chest was delivered with a captive bolt gun (n=17) followed by tube thoracostomy. To control for anesthesia and instrumentation at FiO2 of 0.5, one group received tube thoracostomy only (sham injury; n=6). To control for effects of hypoxia without chest injury, an additional sham-injury group (n=3) was ventilated with FiO2 of 0.12. To generate cardiac function (i.e., Starling) curves, lactated Ringer's solution was administered in three bolus infusions at serial time points; the slope of stroke index versus ventricular filling pressure defines cardiac contractility. By 4 hours after pulmonary contusion, pulmonary vascular resistance, airway resistance, and dead space ventilation were increased, whereas PaO2 (72+/-6 mm Hg at FiO2=0.5) and dynamic compliance were decreased (all p &lt; 0.05). Despite profound lung injury, arterial blood pressure, heart rate, cardiac filling pressures, and output remained within the normal range, which is inconsistent with direct myocardial contusion. The slope of pulmonary capillary wedge pressure versus left ventricular end-diastolic pressure (LVEDP) regression was reduced by more than 50% from baseline (p &lt; 0.05), but there was no significant change in the slope of the central venous pressure versus LVEDP regression. By 4 hours after contusion, the slope of the stroke index versus LVEDP curve was reduced by more than 80% from baseline (p &lt; 0.05). By the same time after sham injury with FiO2 of 0.12 (PaO2 &lt; 50 mm Hg), the regression had decayed a similar amount, but there was no change in the slope after sham injury with FiO2 of 0.5 (PaO2 &gt; 200 mm Hg). After right-side pulmonary contusion, the most often used estimate of cardiac preload (pulmonary capillary wedge pressure) does not accurately estimate LVEDP, probably because of changes in the pulmonary circulation or mechanics. Central venous pressure is a better estimate of filling pressure, at least in these conditions, probably because it is not directly influenced by the pulmonary dysfunction. Also, ventricular performance can be impaired by depressed myocardial contractility and increased right ventricular afterload even with normal left ventricular afterload and preload. It is thus conceivable that occult myocardial dysfunction after pulmonary contusion could have a role in the progression to cardiorespiratory failure even without direct cardiac contusion."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[115, 313]], "word_ranges": [[19, 57]], "text": "The presence of leukocytes + nitrites in the urine is very suggestive of UTI. And if the urine Gram detects G- bacteria, it is probably a UTI due to E Coli. Many E Coli are resistant to amoxicillin."}, "2": {"exist": true, "char_ranges": [[0, 90]], "word_ranges": [[0, 15]], "text": "Other drugs are a good choice in urinary tract infections caused by large negative bacilli"}, "3": {"exist": true, "char_ranges": [[0, 90]], "word_ranges": [[0, 15]], "text": "Other drugs are a good choice in urinary tract infections caused by large negative bacilli"}, "4": {"exist": true, "char_ranges": [[0, 90]], "word_ranges": [[0, 15]], "text": "Other drugs are a good choice in urinary tract infections caused by large negative bacilli"}, "5": {"exist": true, "char_ranges": [[0, 90]], "word_ranges": [[0, 15]], "text": "Other drugs are a good choice in urinary tract infections caused by large negative bacilli"}}, "full_answer": "Other drugs are a good choice in urinary tract infections caused by large negative bacilli (most likely: E. coli). The presence of leukocytes + nitrites in the urine is very suggestive of UTI. And if the urine Gram detects G- bacteria, it is probably a UTI due to E Coli. Many E Coli are resistant to amoxicillin.", "full_answer_no_ref": "Other drugs are a good choice in urinary tract infections caused by large negative bacilli (most likely: E. coli). The presence of leukocytes + nitrites in the urine is very suggestive of UTI. And if the urine Gram detects G- bacteria, it is probably a UTI due to E Coli. Many E Coli are resistant to amoxicillin.", "full_question": "A 13-month-old infant comes to the emergency department with fever up to 39ºC of 48h of evolution with no other associated symptoms. Examination by organs and devices with no significant findings, highlighting good general condition. You were going to discharge him home but the Pediatrics attending on duty asks for a systematic urine and urine culture by catheterization. The urine shows leukocyturia ++, hematuria + and nitrites ++ and the urine Gram-negative bacilli are observed. In the blood analysis there is no leukocytosis and the C-reactive protein is 50 mg/l. The attending now tells you that the child does not need to be admitted and to prescribe an oral antibiotic. State the least appropriate empirical antibiotic treatment in this case:", "id": 191, "lang": "en", "options": {"1": "Amoxicillin.", "2": "Amoxicillin-clavulanic acid.", "3": "Cefuroxime-axetil.", "4": "Cotrimoxazole.", "5": "Cefixime."}, "question_id_specific": 125, "type": "PEDIATRICS", "year": 2013, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0826_24419", "title": "[The etiology of urinary tract infections and antimicrobial susceptibility: study based on children hospitalized in 2012].", "score": 0.01578785075547333, "content": "Assessment of the etiology of urinary tract infections and pathogen drug sensitivity in hospitalized children. We analyzed 156 medical records of patients admitted to the Clinical Department of Pediatrics, Bielański Hospital in Warsaw in 2012, with a suspected UTI. Positive urine culture results were found in 113 (72.4%) children (68; 60.2% of girls and 45; 39.8% of boys), aged from 2 months to 17.9 years (the average age was 2 years and 3 months). E. coli was the most frequent isolated pathogen - 92.0% of patients (104/113). The greatest sensitivity of pathogens showed to cephalosporins of the second and third generation (80.5-90.3%). The sensitivity to amoxicillin with clavulanic acid was 71.7% and 41.6% for ampicillin. The length of hospital stay and treatment ranged from 2 to 16 days (average 8.6 days). In 60.2% (68/113) of patients were treated with second cephalosporin, in 17.7% (20/113) with third generation cephalosporins. Only 11.5% of them (13/113) received amoxicillin with clavulanic acid. Before the treatment, 69.9% (79/113) of children had a fever from 38 up to 41,7ºC, and the fever persisted for the average of 2.5 days (1-8 days). We found significantly higher levels of CRP in children aged between 2-4 in comparison to other age groups (p= 0.0290). In 44.2% (50/113) of children the cystourethrography was performed and in 22% (11/50) cases we recognized a unilateral or bilateral vesicoureteral-ureter of a I to IV degree, on one or both sides. The most common etiological agent of UTIs in children remains E. coli. The sensitivity of urinary pathogens to the commonly used antibiotics is still high, however, finds a large percentage of strains resistant to ampicillin and to amoxicillin with clavulanic acid. The antibiotic recommended for empiric therapy of UTIs in children should be cephalosporins, if there is such a possibility, the treatment should be based on drug sensitivity tests of the organisms grown. Because of the relatively long hospitalization of children with UTIs and the possibility of hospital complications, sequential treatment should also be considered sequential."}, {"id": "pubmed23n0646_23992", "title": "[The diagnosis and therapy of first community acquired urinary tract infection in children].", "score": 0.015696649029982364, "content": "A first urinary tract infection (UTI) in childhood is more prevalent in females &lt; 5-years-old. Circumcision generally protects males from UTI, however, during the month following the procedure, the prevalence of infection increases up to 12 times in circumcised boys when compared with those not circumcised. Almost all the infections are caused by aerobic Gram-negative bacteria of which E. coli are responsible for 70-90% of the cases. Signs and symptoms of UTI vary in different age groups. Factors associated with the likelihood of UTI are: non-circumcised male, fever &gt; 40 degrees C, and a fever &gt; 39 degrees C for more than 48 hours with no other focus of infection on physical examination. Urinalysis and urine microscopy are screening tests for UTI. In children with clinical symptoms and signs suggesting UTI, the results of these tests have a positive predictive value (if both are positive), or negative predictive value (if both are negative) approximating 100%. The definitive diagnosis of UTI is based on the urine culture. Bag urine culture is associated with a very high rate of contamination. Therefore, in non-toilet trained children, urine culture should be obtained directly from the urinary bladder either by supra pubic aspiration or in and out transurethral catheterization. Mid stream clean voided urine specimens obtained from circumcised males in the first months of life are also acceptable. Depending on the clinical presentation, oral therapy can begin from as early as two months of age, and the recommended empiric drugs for first febrile UTI are cefuroxime axetil, or amoxicillin clavulanate. Cephlexin is recommended for cystitis."}, {"id": "wiki20220301en041_56072", "title": "Pyelonephritis", "score": 0.012909511630739251, "content": "During the course of antibiotic treatment, serial white blood cell count and temperature are closely monitored. Typically, the intravenous antibiotics are continued until the person has no fever for at least 24 to 48hours, then equivalent antibiotics by mouth can be given for a total of twoweek duration of treatment. Intravenous fluids may be administered to compensate for the reduced oral intake, insensible losses (due to the raised temperature) and vasodilation and to optimize urine output. Percutaneous nephrostomy or ureteral stent placement may be indicated to relieve obstruction caused by a stone. Children with acute pyelonephritis can be treated effectively with oral antibiotics (cefixime, ceftibuten and amoxicillin/clavulanic acid) or with short courses (2 to 4days) of intravenous therapy followed by oral therapy. If intravenous therapy is chosen, single daily dosing with aminoglycosides is safe and effective."}, {"id": "pubmed23n0816_11292", "title": "[Etiology and clinical course of urinary tract infections in infants less than 3 months-old].", "score": 0.012467292596583038, "content": "Infants less than 3 months of age with urinary tract infection are usually hospitalized. Recent studies show that a less aggressive management for those patients aged ≥ 29 days may be feasible. To determine the complication rate in infants&lt;3 months of age with urinary tract infection, and to identify the causative agents and their antibiotic susceptibility. A retrospective study was conducted on infants&lt;3 months of age with positive urinalysis results, together with a positive urine culture from a catheterized specimen and seen in the Emergency Department from 2007 to 2012. Demographics, clinical and microbiology (microorganism isolated and antibiotic susceptibility) data were collected. The complications rate (bacteremia, bacterial meningitis, renal abscess, surgical intervention, Intensive Care Unit admission, or death) were calculated for the overall sample and for different age groups (&lt;29, 29-60, and 61-90 days). A total of 460 patients are included; 137 (29.8%)&lt;29, 166 (36.1%) 29-60, and 157 (34.1%) 61-90 days of age. Twenty four (5.4%; 95% CI: 3.6-7.8) had bacteremia; 15 (10.9%; 95% CI: 6.7-17.3) were&lt;29 days; 8 (4.9%; 95% CI: 2.5-9.4) were 29-60 days, and one (0.7%; 95% CI: 0.1-3.7) was 61-90 days of age (P&lt;.001). One neonate (0.8%; 95% CI: 0.1-4.1) had bacterial meningitis, and 2, renal abscess. Escherichia coli was the common pathogen identified (87.2%) in the urine culture, with a susceptibility to amoxicillin-clavulanate, gentamicin, and cefixime of 89.2, 97.0, and 96.0%, respectively. Complications are low in infants&lt;3 months of age with UTI, especially in those ≥ 29 days of age. The identification of patients at very low risk for complications would allow a less aggressive management. Escherichia coli antibiotic susceptibility remains stable, but continuing careful surveillance is essential to optimize empirical antibiotic treatment."}, {"id": "pubmed23n0740_2206", "title": "[Prescribing of antibiotics in patients admitted from Emergency Departments: a multicenter study].", "score": 0.011744505494505494, "content": "The infectious disease is the main source of care demand in Pediatric Emergency Departments (PED) and is a frequent cause of hospital admission with antibiotics. Our objectives are: 1) to determine the diseases that are seen in PED that required admission with antibiotics; 2) to determine the microbiological methods used and, 3) to analyze the characteristics of the indicated treatment. A prospective multicenter study was conducted in 22 Spanish hospitals. We included patients younger than 18 years seen in PED on day 14 of each month between June 2009 and May 2010 who required hospitalization with systemic antibiotics. Patients admitted to Intensive Care Unit were excluded. There were 30,632 consultations in the PED during the study period. A total of 1,446 (4.7%) patients were hospitalized, 395 (27.3%) of them with antibiotics. Ninety-five patients (24.1%) had received antibiotics before admission. Three hundred twenty (81%) children underwent at least one microbiological test, with blood culture (69.9%) and urine culture (30.9%) being the most requested ones. The main diagnoses at admission were pneumonia (29.4%), urinary tract infection (15.4%), and fever without source (12.1%). Twenty five different antibiotics were prescribed, with cefotaxime (27.8%) and amoxicillin-clavulanate (23.4%) being the most prescribed ones. A single antibiotic was prescribed to 80.8% of patients, and parenteral administration was the most indicated (93.7%). Antibiotic therapy was prescribed in one in every 4 patients who required admission to hospital. Pneumonia was the most common source. Blood culture was the most frequent microbiological test requested in the PED. A limited number of beta-lactam antibiotics represented the majority of antibiotic prescriptions."}, {"id": "pubmed23n1133_20014", "title": "Predictors of Empiric Antibiotic Use in the Emergency Department in Children Without Urinary Tract Infections.", "score": 0.01163751777035739, "content": "Urinary tract infections (UTIs) are a common diagnosis within the pediatric emergency department (ED). Because of the necessary delay in obtaining urine culture results, clinicians must decide whether to prescribe antibiotics for a suspected UTI before urine culture results. The primary objective of this study was to identify the proportion of children given empiric antibiotics who subsequently did not meet consensus definition of an UTI. The secondary objective was to identify factors associated with return visits to the ED after an index visit for UTI. We also attempted to identify predictors of prescription of empiric antibiotics for children who did not have a UTI. This was a retrospective chart review of all patients between the ages of 2 months and 18 years diagnosed with a UTI between July 2016 and June 2017 in the ED of a single urban quaternary care center. Patients were excluded for the following reasons: use of bag for urine collection, subsequent admission to the hospital, receipt of antibiotics within the previous 3 days, use of antibiotics for an indication other than a UTI, and urine culture obtained at an outside facility. Of 404 included patients, 389 (96.2%) were discharged on antibiotics and 243 (62.4%) did not have a UTI. On the multivariate analysis, age ≧ 36 months was associated with increased odds of receiving antibiotics and not having a UTI while both ≥1+ leukocyte esterase and ≥1+ nitrites on urinalysis were associated with decreased odds of receiving antibiotics and not meeting UTI criteria. Sixty-two patients revisited the ED within 30 days of the initial visit, 24 (38.7%) of which met criteria for UTI during the index visit. Prescription of antibiotics at the time of the index visit was associated with decreased odds of reutilization, whereas an extended-spectrum β-lactamase producing organism cultured from urine at the index visit was associated with increased odds of reutilization. A high number of patients discharged on empiric antibiotics did not meet criteria for a UTI. We did not identify clinically useful factors that predicted prescription of empiric antibiotics for children who do not have a UTI. We believe that unnecessary antibiotic prescriptions could be substantially decreased by decreasing empiric use of antibiotics coupled with reliable follow-up for positive urine cultures."}, {"id": "wiki20220301en041_56061", "title": "Pyelonephritis", "score": 0.01119047619047619, "content": "Diagnosis Laboratory examination Analysis of the urine may show signs of urinary tract infection. Specifically, the presence of nitrite and white blood cells on a urine test strip in patients with typical symptoms are sufficient for the diagnosis of pyelonephritis, and are an indication for empirical treatment. Blood tests such as a complete blood count may show neutrophilia. Microbiological culture of the urine, with or without blood cultures and antibiotic sensitivity testing are useful for establishing a formal diagnosis, and are considered mandatory."}, {"id": "wiki20220301en001_192883", "title": "Urinary tract infection", "score": 0.010817307692307692, "content": "Diagnosis In straightforward cases, a diagnosis may be made and treatment given based on symptoms alone without further laboratory confirmation. In complicated or questionable cases, it may be useful to confirm the diagnosis via urinalysis, looking for the presence of urinary nitrites, white blood cells (leukocytes), or leukocyte esterase. Another test, urine microscopy, looks for the presence of red blood cells, white blood cells, or bacteria. Urine culture is deemed positive if it shows a bacterial colony count of greater than or equal to 103 colony-forming units per mL of a typical urinary tract organism. Antibiotic sensitivity can also be tested with these cultures, making them useful in the selection of antibiotic treatment. However, women with negative cultures may still improve with antibiotic treatment. As symptoms can be vague and without reliable tests for urinary tract infections, diagnosis can be difficult in the elderly."}, {"id": "Pediatrics_Nelson_2117", "title": "Pediatrics_Nelson", "score": 0.009945478473397256, "content": "Assess H and P for “low risk” status Age? <1 mo >1 mo Low risk Not low risk CBC with differential, blood culture, urine culture All parameters normal? No Yes All parameters normal? NoYes outpatients without empirical antibiotic treatment or, alternatively, treated with intramuscular ceftriaxone. Regardless of antibiotic treatment, close follow-up for at least 72 hours, including re-evaluation in 24 hours or immediately with any clinical change, is essential. Children with a positive blood culture require immediate re-evaluation, repeat blood culture, consideration for lumbar puncture, and empirical antibiotic treatment."}, {"id": "pubmed23n0828_21301", "title": "[Management of urinary tract infections in children. Recommendations of the Pediatric Infectious Diseases Group of the French Pediatrics Society and the French-Language Infectious Diseases Society].", "score": 0.009900990099009901, "content": "Urine dipsticks have to be used more frequently for the screening of urinary tract infections (UTI) in febrile infants and children (grade A). Confirmation of the UTI by urine culture should prefer other methods of sampling than the urine bag: sampling jet, urethral catheterization, or pubic puncture (grade A). The percentage of Escherichia coli producing extended-spectrum beta-lactamases (ESBL) in children accounts for less than 10 % in France and does not justify revising the 2007 recommendations (grade B). An increase in the use of carbapenems in first-line treatment is a major environmental hazard and exposes the patient to the risk of untreatable infections. For febrile UTI, the expert group recommended: (1) recover the results of susceptibility testing as soon as possible to quickly adapt treatment for possible resistant strains; (2) favor initial treatment with aminoglycosides (particularly amikacin) which remain active in the majority of ESBL strains for patients seen in the pediatric emergency department and/or hospital; (3) ceftriaxone (IV or IM) remains an appropriate treatment for patients seen in the emergency department or outpatient clinic because the percentage of ESBL-producing enterobacteria strains remains low; (4) use oral cefixime (grade B) in nonsevere cases and low-risk patients defined as age&gt;3 months, general condition preserved, disease duration of fever&lt;4 days, no associated comorbidity, and no history of urinary tract infection, uropathy, or prior antibiotic therapy in the last 3 months; (5) oral relay for parenteral treatment is guided by in vitro susceptibility testing, in an attempt to reduce the use of oral cephalosporins to limit the selection of resistant bacterial strains. The total duration of treatment recommended is usually 10 days. Except for special circumstances, there is no need to prescribe retrograde cystography or antibiotic prophylaxis after a first febrile urinary tract infection. For cystitis, the panel recommends systematic urinalysis and initial prescription before the results of the urine culture of one of the three following oral antibiotics: amoxicillin-clavulanate, cotrimoxazole, cefixime. The total duration of antibiotic treatment is 5days to tailor treatment based on clinical progression and antibiotic susceptibility."}, {"id": "pubmed23n0415_511", "title": "Is a negative dipstick urinalysis good enough to exclude urinary tract infection in paediatric emergency department patients?", "score": 0.009900990099009901, "content": "Urinary tract infection is a common cause of serious bacterial infection in young children. The non-specific presentation has implications for misdiagnosis and the potential for long-term complications. To determine if a negative dipstick urinalysis is adequate to exclude urinary tract infection in children aged 0-10 years. Data was subdivided into two age groups: 0-2 years and 2-10 years. Retrospective case note review over an 8-month period. Cases included required a printed urinalysis recorded from the Clinitek 50 (Bayer) machine and a printed microscopy and culture result. We defined a negative urinalysis as being negative for all of blood, protein, leucocytes and nitrites. A total of 375 cases were included for statistical calculation. Three hundred and seventy-five cases gave a prevalence of 10.7% with a sensitivity of 92.5%, specificity of 39.4% and a negative predictive value of 97.8%. In the 0-2-year-old group, we demonstrated a prevalence of 15%, a sensitivity of 87.5%, specificity of 39.7% and a negative predictive value of 94.7%. This compares to the older group (2-10 years) with a prevalence of 7.0%, a sensitivity of 100%, specificity of 39.7% and a negative predictive value of 100%. Prevalence of urinary tract infection varied with age with a higher prevalence in the 0-2 years age group. The lower negative predictive value and the higher clinical importance in this age group means that dipstick urinalysis is inadequate to exclude urinary tract infection. Conversely, we believe that children in the 2-10 years age group can adequately have urinary tract infection excluded with a negative dipstick urinalysis."}, {"id": "pubmed23n1088_13074", "title": "[Febrile syndrome in children younger than 29 days].", "score": 0.00980392156862745, "content": "Acute fever of unknown origin (FUO) in children under 29 days is a worrying situation because of the risk of serious bacterial infection (SBI). to study the main clinical and laboratory characteristics of a group of hospitalized children under 29 days with diagnosis of FUO. Retrospective study of children under 29 days hospitalized due to FUO. The clinical records of the patients were reviewed, recording age, sex, history of fever before consultation, temperature at admission, estimated severity at admission and discharge, discharge diagnoses, laboratory tests, and indicated treatments. Patients were classified according to the severity of the discharge diagnosis, as severe (S) and non-severe (NS). The inclusion criteria were term newborn, age less than 29 days, fe ver &gt; 38°C registered at home or admission, and history of &lt; 4 days. 468 children with FUO were admitted. Concordance between severity at admission and discharge was low (Kappa = 0.125; p = 0.0007). 26.1% of children were S and 73.9% NS. In the S group, urinary tract infection domínate (70.5%) and in the NS, FUO (67.6%). The cut-off levels for leukocytes/mm3, C-reactive protein, and neutrophils/mm3 showed negative predictive values to rule out severe bacterial infection. Conclu sions: Most of the newborns presented mild severity at admission, but 24% of them had SBI, thus hospitalization and close clinical observation are always necessary. Laboratory tests, such as CRP, white blood cell and neutrophils count are not good predictors of SBI. Early treatment with antibio tics for patients who meet the low-risk criteria is debatable."}, {"id": "Pediatrics_Nelson_2115", "title": "Pediatrics_Nelson", "score": 0.00971605031257061, "content": "Most episodes of fever in children younger than 3 years of age have a demonstrable source of infection elicited by history, physical examination, or a simple laboratory test. In this age group, the most commonly identified serious bacterial infection is a UTI. A blood culture to evaluate for occult bacteremia, and urinalysis and urine culture to evaluate for a UTI, should be considered for all children younger than 3 years of age with fever without localizing signs. Stool culture should be obtained in those with diarrhea marked by blood or mucous. Ill-appearing children should be admitted to the hospital and treated with empirical antibiotics. Approximately 0.2% of well-appearing febrile children 3 to 36 months of age vaccinated against S. pneumoniae and"}, {"id": "pubmed23n0879_3976", "title": "Resistance to oral antibiotics in 4569 Gram-negative rods isolated from urinary tract infection in children.", "score": 0.009708737864077669, "content": "To investigate antibiotic resistance among pathogens isolated from urines in a tertiary care children's hospital in Italy. Retrospective analysis of prospectively collected data on antibiotic susceptibility of Gram-negatives isolated from urines at the Istituto Giannina Gaslini, Genoa - Italy from 2007 to 2014. Antibiotic susceptibility was evaluated. By means of CLSI criteria from 2007 to 2010, while from 2011 EUCAST criteria were adopted. Data on susceptibility to amoxicillin-clavulanate, co-trimoxazole, cefuroxime, nitrofurantoin, fosfomycin and ciprofloxacin were evaluated for Escherichia coli, while for other Enterobacteriaceae data were collected for amoxicillin-clavulanate, co-trimoxazole and ciprofloxacin and for ciprofloxacin against Pseudomonas aeruginosa. Univariate and multivariable analyses were performed for risk factors associated with resistance. A total of 4596 Gram-negative strains were observed in 3364 patients. A significant increase in the proportion of resistant strains was observed for E.coli against amoxicillin-clavulanate, cefuroxime and ciprofloxacin and for others Enterobacteriaceae against co-trimoxazole and ciprofloxacin. Resistance to nitrofurantoin and fosfomycin was very infrequent in E.coli. Logistic regression analysis showed that repeated episode of urinary tract infections was a risk factor for E.coli resistance to amoxicillin-clavulanate, co-trimoxazole and cefuroxime, while admission in one of the Units usually managing children with urinary tract malformations was significantly associated to resistance to amoxicillin-clavulanate and cefuroxime. In conclusion the present study shows an increase in antibiotic resistance in pediatric bacteria isolated from urines in children, especially in presence of repeated episodes and/or urinary tract malformations. This resistance is worrisome for beta-lactams and cotrimoxazole, and start to increase also for fluoroquinolones while nitrofurantoin and fosfomycin still could represent useful drugs for oral treatment of these infections. • Infections are frequent in patients with urinary tract malformations • Antibiotic prophylaxis can select for resistant pathogens What is New: • The increase in the resistance to β-lactams, co-trimoxazole or fluoroquinolones in pathogens causing urinary tract infections cause a reduction of drugs with oral formulations available for therapy • Old drugs like nitrofurantoin and fosfomycin can represent attractive compounds for oral treatment of urinary tract infections in children presence of resistance to other drug classes."}, {"id": "pubmed23n0845_7858", "title": "[Children less than 3 months hospitalised due to acute febrile syndrome. 5 years clinical experience].", "score": 0.009708737864077669, "content": "Acute fever of unknown origin (AFUO) is established when the anamnesis and physical examination cannot identify the cause. In infants less than 3 months-old this is situation for concern, due to the risk of a serious bacterial infection. To describe the clinical and laboratory variable of patients with AFUO, in order to look for clues in order to base studies on the decisions arising drom this problem. A report is presented on a retrospective study conducted on a cohort of children less than three months-old admitted to the Hospital Roberto del Río (2007-2011) due to an AFUO. Clinical histories were reviewed and the patients were grouped, according to the severity of the admission diagnosis, into severe and non-severe. They were compared in strata determined by the variables of clinical interest. A total of 550 children were admitted with AFUO during the study period. There was low agreement between the severity on admission and at discharge (kappa=0.079; P=.26). There were 23.8% of children in the severe group and 76.2% in the non-severe group. Urinary tract infection predominated in the severe group (68.7%) and 40.7% with acute febrile syndrome in the non-severe group. The cut-off levels for C-reactive protein, white cells, and neutrophils per mm(3), to calculate the fixed and variable indices, only showed negative predictive values of some use for ruling out serious bacterial infection. The ROC curves with white cell and neutrophil counts and C-reactive protein, did not provide andy fixed indices of clinical use. More than one-third (34.6%) of lumbar punctures were traumatic or failures. According to the results of this study, there is an obvious excess of hospital admissions, little usefulness in the examinations to identify serious bacterial infection, a high percentage lumbar punctures traumatic and lumbar punctures failures, and an excess of antibiotic treatments. A review of clinical criteria and procedures is needed."}, {"id": "pubmed23n0989_8293", "title": "What is the role of Ewingella americana in humans? A case report in a healthy 4-year-old girl.", "score": 0.009615384615384616, "content": "Ewingella americana (Ea) is a Gram-negative, lactose-fermenting, oxidase-negative and catalase-positive bacterium that was first described in 1983 as a new genus and species in the family Enterobacteriaceae. It is not known whether Ea is a true pathogen or simply an opportunistic infectious agent, as most of the cases have been described in patients at risk. A 4-year-old girl described here was hospitalized due to a productive cough over the previous 3 weeks and a fever &gt; 38 °C associated with tachypnea over the previous 2 days. Her familial and personal medical histories were negative for relevant diseases, including respiratory infections. At admission, she was febrile (axillary temperature 39.2 °C) and had dyspnea with retractions, grunting and nasal flaring. A chest examination revealed fine crackling rales in the left upper field associated with bilateral wheezing. A chest X-ray revealed segmental consolidation of the lingula of the left lung. Laboratory tests revealed leukocytosis (15.,800 white blood cells/mm<sup3</sup with 50.3% neutrophils), a slight increase in serum C-reactive protein (11.9 mg/L) and normal procalcitonin values (&lt; 0.12 ng/mL). A nasopharyngeal swab culture did not reveal viral or bacterial respiratory pathogens, including atypical bacteria. A blood culture revealed the presence of a Gram-negative, lactose-fermenting rod that was oxidase negative and catalase positive. The isolate was identified by means of the VITEK®2 identification system (bioMérieux, Firenze, Italy) as Ea. This identification was confirmed by sequencing the 16 s ribosomal deoxyribonucleic acid (rDNA). The pathogen was sensitive to aminoglycoside, fluoroquinolones, carbapenems, cefotaxime, and ceftazidime but was intermediate against sulfametoxazole/trimethoprim and resistant to amoxicillin-clavulanic acid, fosfomycin, and oxacillin. The child was immediately treated orally with amoxicillin-clavulanic acid and erythromycin. Based on the results of a blood culture and sensitivity tests, the amoxicillin-clavulanic acid medication was stopped after 3 days. Erythromycin was continued for a total of 10 days, and the child was discharged after 3 days in the hospital. Follow-up visit 1 month later did not reveal any respiratory problems. This case shows that Ea infections in healthy subjects are mild even in pediatric age, and the need for antibiotic therapy is debated. Cases occurring in subjects with underlying chronic disease can be significantly more complicated and require appropriate antibiotic therapy."}, {"id": "pubmed23n0130_9799", "title": "[Urinary tract infections in childhood: importance of immediate urine culture. Personal experience with 4176 tests].", "score": 0.009615384615384616, "content": "Since infections of the urinary tract in children often present diagnostic difficulties, it is essential to be able to identify these patients with simple, reliable methods. After a clinical-laboratory study on 4176 urine cultures, the Authors affirm that the reliability of this test is much greater when the parents have been instructed in the correct way of collection the urine specimen, and when in the laboratory the \"slide\" is carried out within three hours of taking the sample."}, {"id": "pubmed23n0414_2549", "title": "[Diagnostic test in emergency departments for bacterial infections in infants younger than 12 months].", "score": 0.009523809523809525, "content": "To evaluate clinical and analytic numeric data that may help the emergency departments to identify bacterial infections in infants. A retrospective study of 430 infants with bacterial growth in cultures (culture from blood, 30; urine, 207; stools, 193, and/or cerebrospinal fluid, n 25) was performed. These patients were compared with a control group (n 430), randomly selected from patients aged less than 12 months with negative cultures who were hospitalized with suspected infection. Neonates and surgical patients were excluded from both groups. Statistical analysis was performed using Student's t-test for independent samples, Levene's test for the study of equality of variances, bivariate correlation and one-factor ANOVA, and receiver-operating characteristic (ROC) curves and odds ratios were calculated when statistically significant (p &lt; 0.05) results were obtained. These analyses were performed using the SPSS 10.0 statistical software package. Of the infants admitted to the pediatric unit, 11.7 % had at least one positive bacterial culture. Temperature (p 0.005), leucocyte count (p 0.003), percentage of neutrophils (p &lt; 0.0001) and C-reactive protein (p &lt; 0.0001) were significantly higher in infants with positive cultures. In invasive infections significant differences were found in sex (more frequent in males) (p 0.03), heart rate (p &lt; 0.0001) and respiratory rate (p 0.003). In the ROC curves, the best diagnostic yield was obtained for C-reactive protein (0.93 for a cutoff value of 29 mg/l, 86 % specificity and 91 % sensitivity). C-reactive protein is essential for diagnosis of bacterial infection in infants in the emergency department."}, {"id": "pubmed23n0505_6061", "title": "Treatment of urinary tract infections among febrile young children with daily intravenous antibiotic therapy at a day treatment center.", "score": 0.009433962264150943, "content": "Urinary tract infections (UTIs) are common among infants and toddlers. Children can be treated effectively with short courses (2-4 days) of intravenous (IV) therapy followed by oral therapy. If IV therapy is chosen, use of once-daily dosing may allow outpatient management instead of hospital admission. However, no description of ambulatory treatment with IV antibiotics of UTI among febrile children has been reported to date. We aimed to describe the feasibility and complications of outpatient management with IV antibiotics of UTI among febrile children, at the day treatment center (DTC) of a tertiary-care pediatric hospital. Between April 1, 2002, and March 31, 2003, a prospective cohort of patients 3 months to 5 years of age who were examined in the emergency department (ED) and diagnosed as having presumed febrile UTI were treated according to a clinical protocol. Patients were treated at the DTC unless they met exclusion criteria, in which case they were hospitalized. The DTC was open 7 days per week, including holidays, from 8:30 am to 4:30 pm. At the DTC, patients were initially treated with a daily dose of IV gentamicin, until the child had been afebrile for at least 24 hours, and with oral amoxicillin, until preliminary urine culture results were available. Children allergic to penicillin received gentamicin only. IV antibiotics were administered through peripheral IV access; the IV catheter's patency was maintained with injection of 50 U of heparin once daily throughout the treatment period. Parental satisfaction with the DTC experience was assessed with an anonymous, self-administered questionnaire. Two hundred ninety-one episodes of presumed febrile UTI were diagnosed in the ED, of which 212 (72.9%) were sent to the DTC. There were 71 hospital admissions (24.4%); in 9 of these instances, the child was admitted because parents refused or were unable to comply with DTC treatment. Adherence to the treatment protocol in the ED was excellent; in 92.1% of presumed febrile UTI episodes (268 of 291 episodes), the patient was referred to the appropriate setting for treatment. In 8 instances, patients who met an exclusion criterion were sent to the DTC. They should have been hospitalized, according to the protocol. At the DTC, a final diagnosis of UTI was made in 178 of the 212 episodes (84%). Patients treated at the DTC, with a final diagnosis of UTI, had a median age of 12.0 months (range: 3-68 months), and their mean initial temperature was 39.2 degrees C (SD: 1.1 degrees C). Patients were afebrile by 24 hours in 52% of UTI episodes and by 48 hours in 82%. Minor problems with IV access occurred in 9.0% of cases. The duration of IV antibiotic therapy at the DTC was 1.9 days (SD: 0.9 day). The mean number of visits to the DTC, including appointments for renal ultrasound and voiding cystourethrography evaluations, was 3.5 (SD: 0.9). Parents were present at all scheduled visits in 98.9% of cases. Four patients needed to be hospitalized from the DTC, but in only 1 case was hospital admission related to UTI treatment. Four patients with UTI treated in the DTC had positive blood cultures, 2 with Escherichia coli (both successfully treated at the DTC) and 2 with contaminants. For 4 children treated at the DTC, UTI was caused by gentamicin-resistant E coli. One patient became afebrile within 24 hours after treatment initiation with IV gentamicin; he was then treated with oral cefixime. A second patient was treated with IV ceftriaxone, administered at the DTC once culture results were available, and remained febrile for &lt;72 hours. The last 2 patients were hospitalized; one, who was also allergic to cephalosporins, had been febrile for 72 hours at the time of hospitalization (once hospitalized, he was treated with IV amikacin), and the other was admitted to the hospital for an unrelated problem, namely, scalp cellulitis. None of these 4 patients was initially bacteremic or became bacteremic during the treatment period. Repeat urine culture was performed within 14 days after treatment initiation in 146 instances, and results were negative in all cases. At telephone follow-up assessments 14 days after discharge, no patient had been rehospitalized because of UTI. Successful treatment at the DTC (defined as attendance at all visits, normalization of temperature within 96 hours, negative control urine cultures, if performed, and absence of hospitalization from the DTC) was observed in 96.6% of the 178 UTI episodes. Overall adherence of physicians to the protocol at the DTC was 87.1% (95% confidence interval: 82.2-92.0%). One hundred seventy-two satisfaction questionnaires were returned and revealed good, very good, or excellent parental satisfaction in 98.8% of cases. Our data show that ambulatory treatment with IV antibiotics, at a DTC, may be used for at least three-fourths of UTIs among febrile children 3 months to 5 years of age. It is safe and feasible and appears very satisfactory to parents. Although ambulatory treatment with IV antibiotics is more invasive than oral therapy during the initiation of UTI treatment, it ensures almost full compliance, allows close medical supervision, and facilitates investigations related to the UTI. It is an interesting alternative to hospitalization."}, {"id": "pubmed23n0599_9102", "title": "[Evaluation of impact of CRP rapid test in management of febrile children in ambulatory pediatric practice].", "score": 0.009433962264150943, "content": "Fever without source (FWS) is a common cause of children visits to pediatric practices. Clinical evaluation does not always rule out efficiently an invasive bacterial infection. Among blood markers, several publications have suggested the value of C-reactive protein (CRP). This study was performed to assess, in private practices, the impact of rapid CRP test compared to usual technique at the laboratory for the management of children with FWO. The study was undertaken in 2006-2007, in 14 pediatric practices. Pediatricians had to enroll all children, older than three months, consulting for FWS, for whom CRP was prescribed. The pediatric practices were distributed in two groups: in the first one, pediatricians had rapid CRP tests (NycoCard) CRP test, Progen Biotechnique) and in the second one, they sent children to laboratory for the dosage of CRP as usually. Between October 2006 and June 2007, 227 children were enrolled by 17 pediatricians: 159 in the group with rapid CRP test (group 1), 68 in the group without (group 2). The cost of routine biological tests (micro or macro CRP, blood cell count and urine cultures) was on average lower for group 1 compared to the group 2: respectively 7.7 versus 39.3 euro (P&lt;0.0001), a reduction from approximately 80% of cost. In group 1, more dipstick urine tests (22.6 versus 4.4, P=0.0009), less urine cultures (19.5% versus 67.6% P&lt;0.0001) and blood prescriptions were prescribed (3.8% versus 100%, P&lt;0.0001), pulmonary X-rays were not different (23.9% versus 19.1%, P=0.4). It was not observed difference in antibiotic prescription between the two groups (15.7% group 1 versus 19.1% group 2, p=0.5). The average time to obtain the results examinations in laboratory was approximately 11h (median 4.5h, extremes 45 min to two days), for five min in group 1. In group 1, children management was different according to the CRP levels. During the follow-up, no difference was observed between the two groups except less hospitalizations in group 1 (2.9% versus 15.3%, P=0.0015). This study suggests the interest of rapid CRP test for febrile children in ambulatory pediatric practice by reducing number and cost of laboratory examinations and timesaving for patients and practitioners."}, {"id": "pubmed23n0614_13881", "title": "[Evolution of Escherichia coli antibiotic resistances in urine samples from the community].", "score": 0.009345794392523364, "content": "The objectives of this work are two: first, to evaluate the resistance of Escherichia coli to several antibiotics and their trends over a six-year period in strands isolated in urine samples from patients receiving health-care in general practitioner offices in our environment; and second, to evaluate if empirical treatment regimens commonly accepted in our country would be applicable in our environment depending on the results of this study. We analyzed the urine cultures positive for Escherichia coli obtained from samples collected at the 10 primary health care centers of the health-care area of El Bierzo and Laciana (Leon, Spain) between the years 2002 and 2007. In vitro resistances of these germs to several common use antibiotics were determined: fosfomycin, nitrofurantoin, tobramycin, cefuroxime, cefixime, amoxicillin-clavulanic acid, cotrimoxazole, ciprofloxacin, norfloxacin, and ampicillin. The existence of statistically significant (p &lt; 0.05) differences in sensitivity comparing the years 2002 and 2007, including all antimicrobials except cefixime, was analyzed by the chi-square test. For cefixime we compared the results between 2002 and 2005. An increase of the resistance of Escherichia coli isolated in urine to all antimicrobials under study has occurred, except for nitrofurantoin, being the differences statistically significant in most cases. Nevertheless, resistances to fosfomycin and nitrofurantoin have remained below 6% throughout the study period. Resistances to tobramycin and cefuroxime were slightly over 10% and cefixime below 3.4%, although in the last one we only have data until 2005. Resistances to amoxicillin-clavulanic acid, initially low, have progressively increase reaching 20.6% in 2007. The same has happened for cotrimoxazole, ciprofloxacin, norfloxacin and ampicillin, passing 32% in 2007 in the first three cases and 62% in the last one. Variations in bacterial resistance patterns for Escherichia coli obliges to have an updated knowledge of them to adapt general empirical treatment uses to each specific health-care area."}, {"id": "pubmed23n0043_16875", "title": "[Clinical and laboratory correspondence to outpatients with the extreme value of C-reactive protein].", "score": 0.009345794392523364, "content": "It is the policy of Tenri Hospital to notify the patient promptly whenever an extreme laboratory data value is detected. We investigated the utility of forwarding clinical and laboratory correspondence to outpatients with extreme value of C-reactive protein (CRP). Sixty-eight outpatients with CRP levels more than 20 mg/dl detected during 1986 were studied. CRP was measured by turbidometric method, and a sample with CRP level more than 15 mg/dl was diluted with CRP negative serum (CRP level less than 0.2 mg/dl) and was reanalyzed. Fifty-two of 68 patients (76%) had infectious diseases as the causal disease of high CRP, and eight (12%) had other diseases. In the remaining (12%) the causes were unknown. In most patients the causal diseases were diagnosed within one or two days, but diagnosis required more than 4 days in those with acute pyelonephritis, meningitis, liver abscess or renal abscess, as these diseases were diagnosed after the examination of urine or cerebrospinal fluid, or after ultrasonography. Thirty-seven of 58 patients (64%) who had appointments with their physician on the day of the laboratory examination were admitted the same day, and two of 10 patients (20%) who had appointments on the following day were admitted on that day. Seventeen of 25 patients (68%) with urea-N levels more than 30 mg/dl, cholinesterase levels less than 0.7 delta pH and albumin levels less than 3.5 g/dl required more than 15 days to recover, while 29 of 32 patients (91%) with only 2 or fewer of these laboratory values required less than 14 days. The prompt notification of extreme CRP value is an important aspect of medical care. The examination of urine and cerebrospinal fluid and ultrasonography are necessary screening techniques accompanying examination of blood and plain chest X-ray. Urea-N, cholinesterase and albumin values should be determined at the same time as CRP value to assess prognosis."}, {"id": "pubmed23n0493_22095", "title": "[Bacterial pathogens, resistance patterns and treatment options in community acquired pediatric urinary tract infection].", "score": 0.009259259259259259, "content": "Epidemiology and resistance patterns of bacterial pathogens in pediatric UTI show large interregional variability and rates of bacterial resistances are changing due to different antibiotic treatment. We intended to evaluate data from northern Germany. In 100 children (53 female, 47 male, mean age 4.4 +/- 4.2 years) with community acquired UTI, who presented in the emergency department of our medical school from 2000 - 2002, urine cultures were performed. Inclusion criteria were: acute voiding symptoms, significant bacteriuria with growth of at least 10 (5) colony-forming units/ml urine, leukocyturia &gt; 50/ micro l. Exclusion criteria were underlying renal diseases, anatomic abnormalities of the urinary tract, age &lt; 2 months and recurrent UTI. Patients presented with a mean rectal temperature of 38.6 +/- 1.3 degrees C, mean CRP of 66 +/- 68 mg/dl, mean WBC 13 500 +/- 5 600/ micro l and mean urinary leukocytes of 425 +/- 363/ micro l. In urine cultures E. coli was found in 47 % of the cases, Enterococcus faecalis 23 %, Proteus mirabilis 8 %, Klebsiella oxytoca 4 %, Pseudomonas aeruginosa 5 % and others 13 %. In 76 % one and in 24 % two different bacterial species (60 % Enterococcus faecalis) were cultured. Mean resistance rates were in all bacteria (in E. coli): Ampicillin 53 % (69 %), Ampicillin and Sulbactam 51 % (61 %), Cefalosporin 1 (st) generation (Cefaclor) 48 % (24 %), Cefalosporin 2 (nd) generation (Cefuroxim) 40 % (3 %), Cefalosporin 3 (rd) generation (Cefuroxim) 33 % (0 %), Tobramycin 30 % (2 %), Ciprofloxacine 0 %, Cotrimoxazole 40 % (42 %), Nitrofurantoin 12 % (0 %). The resistance rates to Ampicillin (+/- Sulbactam) did not increase as compared to previous analyses (1990 - 1995), however, resistance rates to Cotrimoxazole and 1 (st) generation Cefalosporines increased about 20 %. We conclude that the policies for treatment of UTI in children should be re-evaluated every 5 years according to local resistance rates."}, {"id": "pubmed23n0375_13959", "title": "[Evaluation of dipstick for diagnosis of urinary tract infection in children and adults].", "score": 0.009259259259259259, "content": "Dipstick is used as a first test for screening urinary tract infection. 1087 urine samples from paediatric and adult patients were processed with dipstick and direct microscopic observation of pellet as well as of a Gram stain. A culture was also performed in all of them and was considered as the reference method in order to evaluate all other tests. Sensitivity of dipstick and of Gram stain was higher in urines from adults than in those from children, but direct examination of pellet was better in paediatrics. Specificity of the three screening tests previous to culture presented few variations in both groups. Predictive positive value of dipstick and direct pellet was slightly better in children's urines; on the contrary, Gram stain was better in adults. Negative predictive value was similar for the three parameters. The dipstick here evaluated is a good method for screening of urinary tract infection, though its positivity obliges to a bacteriologic follow up in order to get a certainty diagnosis. Nevertheless, in children less than 2 year old we recommend universal urine culture."}, {"id": "pubmed23n0760_602", "title": "Antimicrobial susceptibility of organisms causing community-acquired urinary tract infections in Gauteng Province, South Africa.", "score": 0.009174311926605505, "content": "Patients with community-acquired urinary tract infections (UTIs) frequently present to healthcare facilities in South Africa (SA). To provide information on UTI aetiology and antimicrobial susceptibility of pathogens. We recruited women with UTI-related symptoms, who tested positive for ≥2 urine dipstick criteria (proteinuria, blood, leucocytes or nitrites) at 1 public and 5 private primary healthcare facilities in 2011. Demographic and clinical data were recorded and mid-stream urine (MSU) specimens were cultured. UTI pathogens were Gram-stained and identified to species level. Etest-based antimicrobial susceptibility testing was performed for amoxicillin/clavulanic acid, cefixime, cefuroxime, ciprofloxacin, fosfomycin, levofloxacin, nitrofurantoin, norfloxacin and trimethoprim/sulphamethoxazole. Of the 460 women recruited, 425 MSU samples were processed and 204 UTI pathogens were identified in 201 samples. Most pathogens were Gram-negative bacilli (GNB) (182; 89.2%) and 22 (10.8%) were Gram-positive cocci (GPC). Escherichia coli was the most frequent GNB (160; 79.6%), while Enterococcus faecalis was the predominant GPC (8; 4.0%). The UTI pathogens had similar susceptibility profiles for fosfomycin (95.5%; 95% confidence interval (CI) 92.6 - 98.4), the 3 fluoroquinolones (94.1%; 95% CI 90.8 - 97.4), nitrofurantoin (91.7%; 95% CI 87.8 - 95.6), cefuroxime (90.1%; 95% CI 86.0 - 94.3) and cefixime (88.2%; 95% CI 83.7 - 92.6). UTI pathogens were less susceptible to amoxicillin/clavulanic acid (82.8%; 95% CI 77.5 - 88.0) when compared with fluoroquinolones and fosfomycin. Trimethoprim/ sulphamethoxazole was the least efficacious antimicrobial agent (44.3% susceptible; 95% CI 37.4 - 51.2). This study provides relevant data for the empirical treatment of community-acquired UTIs in SA."}, {"id": "pubmed23n0051_3565", "title": "[Urinary tract infections in children].", "score": 0.009174311926605505, "content": "The diagnosis of urinary tract infections were established on fifty children in our hospital in the last year. Most of the patients were between the age of 1-12 month (28 cases, 56%). The ratio of female/male was 3.5. Fever was the most common symptom (17 cases, 34%). In the routine urinalysis, proteinuria and pyuria were revealed in 11 cases (22%), and 44 cases (88%), respectively. E. coli was the most common microorganism isolated from urine cultures (70%). Twenty-two patients were regularly followed up and recurrence was observed in 7 patients (32%)."}, {"id": "wiki20220301en089_23285", "title": "Vesicoureteral reflux", "score": 0.00909090909090909, "content": "Medical treatment Medical treatment entails low dose antibiotic prophylaxis until resolution of VUR occurs. Antibiotics are administered nightly at half the normal therapeutic dose. The specific antibiotics used differ with the age of the patient and include: Amoxicillin or ampicillin – infants younger than 6 weeks Trimethoprim-sulfamethoxazole (co-trimoxazole) – 6 weeks to 2 months After 2 months the following antibiotics are suitable: Nitrofurantoin {5–7 mg/kg/24hrs} Nalidixic acid Bactrim Trimethoprim Cephalosporins Urine cultures are performed 3 monthly to exclude breakthrough infection. Annual radiological investigations are likewise indicated. Good perineal hygiene, and timed and double voiding are also important aspects of medical treatment. Bladder dysfunction is treated with the administration of anticholinergics."}, {"id": "pubmed23n0815_7708", "title": "Impact of the lab-score on antibiotic prescription rate in children with fever without source: a randomized controlled trial.", "score": 0.00909090909090909, "content": "The Lab-score, based on the combined determination of procalcitonin, C-reactive protein and urinary dipstick results, has been shown accurate in detecting serious bacterial infections (SBI) in children with fever without source (FWS) on retrospective cohorts. We aimed to prospectively assess the utility of the Lab-score in safely decreasing antibiotic prescriptions in children with FWS and to determine its diagnostic characteristics compared to common SBI biomarkers. Randomized controlled trial in children 7 days to 36 months old with FWS, allocated either to the Lab-score group (Lab-score reported, blinded WBC count) or to the control group (WBC, bands and C-reactive protein determined, blinded procalcitonin and Lab-score), followed up until recovery. Demographic data, antibiotic prescription rate, admission rate and diagnostic properties of the Lab-score were analyzed. 271 children were analyzed. No statistically significant difference concerning antibiotic prescription rate was observed: 41.2% (54 of 131) in the Lab-score group and 42.1% (59 of 140) in the control group (p = 1.000). If recommendations based on the Lab-score had been strictly applied, a hypothetical 30.6% treatment rate would have been encountered, compared to the overall 41.7% observed rate (p = 0.0095). A Lab-score ≥3 showed the following characteristics: sensitivity 85.1% (95% CI: 76.5-93.6%), specificity 87.3% (95% CI: 82.7-91.8%), positive predictive value 68.7% (95% CI: 58.7-78.7%), negative predictive value 94.1% (95% CI: 91.5-97.9%), positive and negative likelihood ratios: 6.68 and 0.17 respectively. Area under the receiver operating characteristic curve was best for the Lab-score (0.911, 95% CI: 0.871-0.950). No difference regarding antibiotic treatment rate was observed when using the Lab-score, due to lack of adherence to the related recommendations. However, if strictly followed, a significant 26.5% reduction of antibiotic prescriptions would have been encountered. Medical education needs to be reinforced in order to observe rather than treat low-risk well-appearing children with FWS. ClinicalTrials.gov NCT02179398."}, {"id": "pubmed23n0871_11216", "title": "[Microbiología, sensibilidad antibiótica y factores asociados a bacteriemia en la prostatitis aguda].", "score": 0.009009009009009009, "content": "The aim of the study was to analyze the characteristics of patients with acute prostatitis presenting to the Emergency Department, the microbiological findings, antibiotic susceptibility, and bacteraemia associated factors. Observational and cohort study with prospective follow-up including patients with acute prostatitis presenting to the Emergency Department from January-December 2012. Data were collected for demographic variables, comorbidities, microbiological findings, antibiotic treatment and outcome. Two hundred and forty one episodes of acute prostatitis were included. Mean age was 62.9 ± 16 years, a history of prostate adenoma was reported in 54 cases (22.5%) and prior manipulation of the lower urinary tract in 40 (17%). Mean symptoms duration was 3.38 ± 4.04 days, voiding symptoms were present in 176 cases (73%) and fever in 154 (64%). Seventy patients (29%) were admitted to the hospital and 3 died. From 216 urine cultures, 128 were positive (59%) and 24 (17.6%) out of 136 blood cultures. Escherichia coli was the main pathogen (58.6% of urine cultures and 64% of blood cultures) with resistant strains to fluoroquinolones, cotrimoxazole and amoxicillin/clavulanic in 27.7%, 22.9% and 27.7% of cases respectively. In the univariate analysis, only chills were associated to bacteraemia (p=0.013). At 30-day follow-up, patients with bacteraemia returned more frequently to the Emergency Department (p=0.037) and were more often admitted to the hospital (p=0.003). Patients with acute prostatitis discharged from the Emergency Department need clinical follow-up and monitoring of microbiological findings in order to assure an adequate antibiotic treatment. Return to Emergency Department and admission to the hospital were significantly more frequent among patients with bacteraemia."}, {"id": "pubmed23n1006_4444", "title": "Urine Specific Gravity and the Accuracy of Urinalysis.", "score": 0.009009009009009009, "content": "A recent study in young infants found that different cutoffs maximized the accuracy of the urine white blood cell count in dilute versus concentrated urine samples. We aimed to confirm this finding and to determine its impact on clinical care. We conducted a retrospective analysis of data gathered on consecutive children &lt;24 months of age with visits to the emergency department during a 5-year period. We evaluated the accuracy of screening tests for urinary tract infection (UTI) in dilute and concentrated urine samples. We also calculated the number of children who would have been treated differently in a hypothetical cohort of 1000 children presenting with fever had urine specific gravity (SG) been taken into consideration. We included 10 078 children. The ability to rule in UTI (as measured by the positive likelihood ratio [LR]) was similar in dilute and concentrated urine for the leukocyte esterase test (11.76 vs 10.71, respectively). The positive LR for urine white blood cell count per high-powered field was higher in dilute urine (9.83 vs 6.12). In contrast, the positive LR for the nitrite test was lower in dilute urine (20.54 vs 47.44). Despite these differences, we found little change in the number of children treated with antibiotics in predictive models that took urine SG into consideration. Although we found that urine SG influences the accuracy of some components of the urinalysis, its inclusion in the decision-making process had negligible effect on the clinical care of children with UTI."}, {"id": "pubmed23n1140_24590", "title": "The Effect of the COVID-19 Pandemic on Urine Culture Results and Resistance to Antibiotics in the Emergency Department.", "score": 0.008928571428571428, "content": "This study aimed to investigate the effect of the COVID-19 pandemic on urine culture results and antibiotic sensitivities in patients with suspected urinary tract infections (UTI) admitted to the emergency department (ED) and determine more accurate treatment modalities for patients. The primary endpoint of our study was to determine the change in antibiotic resistance of UTI agents in the pre-and post-COVID period. In the study, urine samples were sent from ED to the microbiology laboratory with a preliminary diagnosis of UTI between June 1, 2019, and July 1, 2021. Urine samples with the growth of 105 cfu/mL and above in urine cultures or with the growth of 103 cfu/mL and above in urine sample cultures taken from catheters were examined. At the end of the exclusions, the results of a total of 1,090 patients were evaluated. Urine cultures and an-tibiotic susceptibility tests of the patients included in the study were examined in two periods (pre-pandemic and post-pandemic). A total of 1,090 aerobic urine cultures sent from the ED between June 2019 and June 2021 were finalized in the microbiology laboratory. Of the 1,090 urine cultures sent from the ED within the 24 months included in the study, 497 (45.59%) were sent eight months before the COVID-19 pandemic. Growth was detected in 33 (6.63%) cultures. In the 16 months after the pandemic, 593 (54.41%) urine cultures were sent. Growth was seen in 69 (11.6%) cultures. The positivity rate obtained from urine cultures sent after the COVID-19 pandemic was significantly higher than those sent before the COVID-19 pandemic (p = 0.005). According to cultures and antibiogram results, resistance to ampicillin, cefuroxime, cefuroxime axetil, cefoxitin, cefixime, ceftazidime, ceftriaxone, and amoxicillin-clavulanic acid decreased significantly compared with pre-COVID-19 (p &lt; 0.05). In addition, Extended Spectrum Beta-Lactamase (ESBL) resistance decreased significantly compared with the prepandemic period (p = 0.012). In this study, we found that the susceptible to antibiotics increased significantly in the post-COVID-19 period compared to the pre-COVID-19 period."}, {"id": "pubmed23n0752_13728", "title": "[Comparative study of C-reactive protein and procalcitonin in the severity diagnosis of pyelonephritis in children].", "score": 0.008928571428571428, "content": "The aim of this study is to compare two biologic parameters; C-reactive protein (CRP) and procalcitonin (PCT) in the detection of acute renal lesions assessed by DMSA scintigraphy in the urinary tract infection in child. In a prospective study, serum PCT, CRP and leukocyte counts were measured for children admitted, between January and December 2010, with a first episode of febrile urinary tract infection. Seventy-five children were enrolled in the study. Thirty-three patients had renal lesions (group A) and 42 had a normal DMSA scintigraphy (group B). The mean PCT level was significantly higher in group A than in group B (8.81 ng/mL versus 1.7 ng/mL, P=0.01). In this study, using receiver operating characteristic (ROC) curve, we identified that the optimal cut-off value with ideal sensitivity and specificity for PCT in detection of renal lesions was 0.76 ng/mL and for CRP, it was 70 mg/L. The sensitivity, the negative predictive value and the indice of Youden of the cut-off value of PCT were significantly higher than CRP (82% versus 70%; 84% versus 70% and 0.58 versus 0.25). This study confirmed that the serum PCT level was more sensitive and specific than the CRP in the detection of renal lesions in the first urinary tract infection in child."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[0, 164]], "word_ranges": [[0, 28]], "text": "This is cryptorchidism. It has to be treated before the age of 2 years, and hormonal treatment is currently in disuse. The treatment of first choice is orchidopexy."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "This is cryptorchidism. It has to be treated before the age of 2 years, and hormonal treatment is currently in disuse. The treatment of first choice is orchidopexy.", "full_answer_no_ref": "This is cryptorchidism. It has to be treated before the age of 2 years, and hormonal treatment is currently in disuse. The treatment of first choice is [HIDDEN].", "full_question": "12-month-old boy, who in the health examinations practiced since birth presents right testicle in inguinal canal that is not possible to descend to the scrotum. Mark the CORRECT answer:", "id": 323, "lang": "en", "options": {"1": "The most likely diagnosis is retractile testis.", "2": "Wait until two years of age for spontaneous decrease of the synovial fluid.", "3": "Human chorionic gonadotropin is the treatment of first choice.", "4": "The indication for orchidopexy should not be deferred.", "5": NaN}, "question_id_specific": 150, "type": "UROLOGY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0513_9119", "title": "The efficacy of human chorionic gonadotropin in retractile testis.", "score": 0.017812105926860028, "content": "To evaluate the efficacy of hCG therapy on retractile testis in various testicular locations and age groups. This study comprised 123 boys whose diagnosis were retractile testis with scrotal base, high scrotal and superficial inguinal localizations with an average age of 4,2 (1-8) years. 123 boys with retractile testes were given a total dose of 9000 hCG and its effect on scrotal base, high scrotal and superficial inguinal located testes were evaluated after 2 weeks and 6 months of completing hCG course. The patients were evaluated in four age groups such as less than 2 years of age, between 2-4, 4-6 years and more than 6 years of age and the response rates were also noted in these age groups. In unilateral cases, the response in base, high scrotal and superficial inguinal levels were found as 100%, 83,3%, 72,7% respectively where as these rates were found as 100%, 92,6%, 76,3% respectively in bilateral cases after 2 weeks of hCG therapy. The response rates in less than 2 year old group, 2-4, 4-6 and more than 6 years group were found as 0%, 80%, 78,7%, 100% respectively in unilateral cases and 33,3%, 90%, 86,9% and 83,3% respectively in bilateral cases. After 6 months, reascend was observed in 12,4% in unilateral group and 6,7% in bilateral group. 72,7-100% of retractile testes respond to hCG administration with the highest response in the age of more than six year group. High response rates observed in retractile testes after hCG therapy in more than 2 years old age group indicate that hCG must be given as a primary treatment in retractile testes and orchiopexy for the failures. Also patients must be followed up closely for reascend cases."}, {"id": "pubmed23n0096_18018", "title": "Acquired undescended (ascended) testis: effects of human chorionic gonadotropin.", "score": 0.016846175089754212, "content": "A total of 7 boys 4 to 10 years old was evaluated for undescended testes. All patients had been seen previously by a pediatric urologist and diagnosed as having a retractile testis. In fact 4 boys had undergone surgical correction of a contralateral undescended testis at an earlier date at which time the testis in question could be manipulated into the scrotum. Another boy was examined under anesthesia and the operation was canceled because the testis could be brought into the scrotum. Subsequently, on followup evaluation 2 to 8 years later the ipsilateral testis could not be manipulated into the scrotum. Of the boys 6 then were treated with a short course of human chorionic gonadotropin. Four patients had a positive response but in 3 the testis was undescended again at examination 6 months later. Of the boys 6 ultimately underwent orchiopexy. Boys with highly retractile testes require periodic examination until puberty to ensure that those testes do not ascend secondarily."}, {"id": "pubmed23n0539_14754", "title": "[HCG in the treatment of cryptorchidism. The effect of age and position of the testis].", "score": 0.016808480959687542, "content": "In a recent study, the effect of human chorionic gonadotropin (HCG) in the treatment of children with cryptorchidism aged less than four years was questioned. The purpose of the present study was to determine, on the basis of a retrospective examination of patients' records, the effect of HCG treatment, whether outcomes are age-dependent and whether the effects of treatment are related to the position of the testis. Patients diagnosed with undescended testis who had been treated at the Department of Urology at Gentofte Hospital (Copenhagen) in the period from 1 November 1998 to 31 May 2003 were identified. Patients who had been treated with HCG were included in the sample. Boys who at an earlier stage had been operated on in the inguinal or scrotal region, or who had suffered from inguinal hernia, were excluded, and clinical retractile testes were separated from true undescended testes. The criterion of success for efficient treatment was complete descent to the bottom of the scrotum. In the 306 records examined, 121 patients met the inclusion criteria. In total, there were 170 undescended testes. The patients' median age at time of treatment was 3.6 years. The overall success rate was 34.7%. The success rates in the individual age categories were: 1-2 years, 36.7%; 3-4 years, 33.4%; and 5-13 years, 35%. The position of the testis at the beginning of treatment showed that the lower the pretreatment position, the better the success rate. The overall success of HCG treatment of cryptorchidism was 34.7%. No age dependency of HCG effects was found, but the position of the testis before treatment influenced the success rate."}, {"id": "pubmed23n0842_13138", "title": "Ascending testis following inguinal hernia repair in children.", "score": 0.015842564515130886, "content": "Failure to replace the testes in the scrotum during hernia repair leads to iatrogenic undescended testes. At other times, the testes may spontaneously move back to the inguinal area after being placed in the scrotum, thus resulting in ascending testes. The cases in this study were assessed. Records of 910 boys operated due to inguinal hernia were assessed retrospectively. Following hernia repair, the testes were placed in the scrotum. After the operation, all the testes were checked for being in the scrotum. They were called for follow-up after the operation. Their testes were checked for remaining in the scrotum. Ascending testes were detected in 4 (0.43%) of the patients. These patients had scrotal hypoplasia and/or retractile testes. Their age ranged between 1-3 years. Ascending testes were bilateral in 2 patients, and on the right side in 2. Human chorionic gonodotropin (hCG) was initiated in 3 patients. Two of them improved. Two underwent scrotal orchiopexy. These patients may benefit from hCG in the early postoperative period. Later, scrotal orchiopexy may be needed. Patients who have retractile testes or scrotal hypoplasia in addition to inguinal hernia need orchiopexy together with herniorrhaphy."}, {"id": "pubmed23n0394_16949", "title": "[Non-scrotal testes; first line of management].", "score": 0.01579601990049751, "content": "For non-scrotal testes a distinction can be made between retractile testes (completely descended and normally developed but sometimes situated subcutaneously in the groin area), retained testes (testes cannot be brought into the scrotum or this can only be achieved using light manual pressure) and ectopic testes (lying outside of the descent trajectory). It is estimated that 0.7-0.8% of all boys have as yet undescended testes. The first few days after the birth are the most suitable for testing and registration, as then the cremaster reflex is absent. Registration should take place in both the youth healthcare file and in the 'growth book' for the parents. Retractile testes do not require treatment. There is no consensus concerning the treatment of (possible) acquired nonscrotal testes. For undescended testes the management depends on previous testes localisations. For ectopic testes and testes that have never been scrotal, a referral for surgical treatment should be made prior to the second birthday. Orchidopexy (a better description is orchidofuniculolysis followed by orchidopexy) is only justified in the case of testes which have never descended. In the case of a clear indication, the general practitioner should make a prompt referral (before the second birthday) and in other cases assurance should be provided and an expectant policy adopted until puberty."}, {"id": "pubmed23n0418_14757", "title": "Prospective evaluation of human chorionic gonadotropin in the differentiation of undescended testes from retractile testes.", "score": 0.015603408567480425, "content": "We prospectively evaluated the efficacy of human chorionic gonadotropin (HCG) in the treatment of undescended testis and sought to determine whether HCG assists in the differentiation of undescended testis from retractile testis. Patients with undescended testes were offered HCG. Testis position, laterality and the presence or absence of a hypoplastic scrotum were noted. The same physician (G. W. K.) recorded physical findings prospectively and stated clinical impression of descent. A total of 67 patients with 90 undescended or retractile testes were treated and evaluated with HCG. Of the 64 undescended testes 13 (20%) descended with HCG therapy, with none requiring subsequent surgery. Of the 26 retractile testes 15 (58%) descended with HCG (p &lt;0.001). Based on physical examination, 100% of retractile testes descended if the testis was in the high scrotal position but only 40% descended if the testis was in the superficial pouch or inguinal area. In the undescended testes group no ectopic or nonpalpable testis descended with HCG. Evaluation of HCG with age demonstrated minimal response (15%) to HCG at less than 24 months, and a peak response between ages 2 and 6 years (75%) with response decreasing thereafter. HCG may have a limited role in the evaluation of undescended testis in patients younger than 2 years. HCG can serve as an adjunct in the clinical diagnosis of retractile testis in older children."}, {"id": "pubmed23n0318_8095", "title": "Effect of a combined GnRH/hCG therapy in boys with undescended testicles: evaluated in relation to testicular localization within the first week after birth.", "score": 0.015199637023593466, "content": "Among 509 boys referred with undescended testicles, 112 had true undescended testicles unilaterally and 62 bilaterally. Patients with true undescended testicles were offered hormonal treatment unless the condition was associated with hernia or previous operations. Boys less than 5 years old were primarily treated with gonadotrophin releasing hormone (GnRH), while boys more than 5 years old were primarily treated with human chorionic gonadotrophin (hCG). If the effect of the primary treatment was insufficient the other hormone was given. Testicular descent was obtained for 64% (23/36) of the intraabdominally located testicles in boys with bilaterally undescended testicles versus only 14% (3/21) in boys with unilaterally undescended testicles (p &lt; .001). Treating 1 to 4 years old boys with GnRH resulted in descent in 16 of 95 testicles and secondary treatment with hCG yielded an additional 34, whereas secondary treatment of 5 to 13 years old boys with GnRH added only 10 descended testicles to 51 of 101 testicles. Reading the maternity records of 272 of the boys support other studies showing that testicles may reascend. Boys with endocrinological or \"surgical\" causes of incomplete testicular descent were relatively more likely to have had one or two undescended testicles during the first postnatal week compared with boys found to have only retractile testicles (p &lt; .001). Treatment with hormones resulted in descent in 56% of boys whose testicles were both descended within one week after birth. Conversely, only 1 of 20 boys with unilateral testicular undescent postnatally was sufficiently treated with hormones (p &lt; .001). The hormonal effect in boys with bilaterally undescended testicles at delivery did not differ significantly from boys with either one or none undescended testicle postnatally. In 35 of 51 boys (69%) in whom the hormonal effect was insufficient, operation revealed a \"surgical cause\" of the incomplete testicular descent."}, {"id": "pubmed23n0594_12978", "title": "[The undescended testis: arguments in favour of early treatment, provided retractile testis and acquired non-scrotal testis have been excluded].", "score": 0.015173285821024196, "content": "--Guidelines for the treatment ofundescended testis (UDT) are sparse. Often an operation in the second year of life is advised. --Recent data indicate that the normal maturation process, which will ultimately lead to a normal quantity and quality of germ cells, is impaired as early as in the first half year of a newborn's life. None of the guidelines take this into account. Spontaneous descent after the fourth month following birth, of testes that have previously not descended, hardly ever occurs. --No differences have been shown in complication numbers between surgery before and after the first birthday. Orchidopexy prior to the 13th birthday reduces the risk of testicular cancer. --Therefore, based on these data, it is advised to perform orchidopexy in the second half of the first year of a newborn's life. In older boys a UDT must be treated before the 13th birthday. --In the Netherlands a lot more orchidopexies are done despite what may be expected based on prevalence numbers of UDT: testes retaining a normal volume that would most probably have descended spontaneously come puberty. --It remains important to carry out a careful physical examination and document the position of the testes soon after birth, and later on if UDT is suspected, to avoid unnecessary operations on retractile testes and acquired UDT."}, {"id": "wiki20220301en017_70969", "title": "Cryptorchidism", "score": 0.014617971231085373, "content": "Diagnosis The most common diagnostic dilemma in otherwise normal boys is distinguishing a retractile testis from a testis that will not descend spontaneously into the scrotum. Retractile testes are more common than truly undescended testes and do not need to be operated on. In normal males, as the cremaster muscle relaxes or contracts, the testis moves lower or higher (\"retracts\") in the scrotum. This cremasteric reflex is much more active in infant boys than older men. A retractile testis high in the scrotum can be difficult to distinguish from a position in the lower inguinal canal. Though various maneuvers are used to do so, such as using a cross-legged position, soaping the examiner's fingers, or examining in a warm bath, the benefit of surgery in these cases can be a matter of clinical judgment."}, {"id": "pubmed23n0289_8759", "title": "Reappraisal of the role of human chorionic gonadotropin in the diagnosis and treatment of the nonpalpable testis: a 10-year experience.", "score": 0.01460685284214696, "content": "We retrospectively evaluated the ability of human chorionic gonadotropin (HCG) to make the nonpalpable cryptorchid testis become palpable and promote testicular descent. Through surgical bookings we identified 94 patients younger than 11 years who received HCG between 1984 and 1994 for the diagnosis or treatment of a nonpalpable undescended testis. The dose of HCG was 1,500 IU/m.2 intramuscularly 2 times weekly for 4 weeks. Testis location was determined by physical examination before and after hormone administration, and confirmed at surgical exploration. Of the 99 nonpalpable testes identified in 94 patients 39 (39%) became palpable following HCG administration and only 2 (2%) completely descended. A total of 60 testes remained nonpalpable with the most common reason being an absent or severely atrophic testis (40, 67%). Of the testes remaining nonpalpable after hormonal stimulation 73% were surgically located at or distal to the internal ring. HCG is preoperatively efficacious in causing the nonpalpable undescended testis to become palpable. For patients failing to respond to hormonal stimulation we recommend preliminary inguinal exploration, since most testes or testicular remnants are located within the inguinal canal or immediately below the internal ring."}, {"id": "Pediatrics_Nelson_3509", "title": "Pediatrics_Nelson", "score": 0.014588601439408676, "content": "The undescended testis is usually histologically normal at birth. Atrophy and dysplasia are found after the first year of life. Some boys have congenital dysplasia in the contralateral descended testis. Surgical correction at an early age results in greater chance of adult fertility. Administration of human chorionic gonadotropin causes testosterone release from functioning testes and may result in descent of retractile testes. Orchidopexy is usually done in the second year of life. Most extra-abdominal testes can be brought into the scrotum with correction of the associated hernia. If the testis is not palpable, ultrasound or magnetic resonance imaging may determine its location. The closer the testis is to the internal inguinal ring, the better the chance of successful orchidopexy."}, {"id": "pubmed23n1028_17531", "title": "[German guideline on undescended testis-what is relevant in daily routine?]", "score": 0.014533870887462047, "content": "With an incidence of 0.7-3% in male infants, undescended testicles is one of the most common congenital anomalies. In the first 6 months of life, the testicles may spontaneously descend in up to 70% of individuals. If the testicle is not in a scrotal position afterwards, fertility can gradually be reduced and the risk of a testicular tumor increases. Therefore, the current German guideline for undescended testis recommends that therapy should be take place between 6 and 12 months of life. After extensive information on the advantages and disadvantages, hormone therapy with the aim of a descensus or in those with bilateral anomaly with the aim of improving the germ cell pool can be offered. After the first year of life, hormone therapy is obsolete. Otherwise, surgical intervention is the treatment of choice. In the case of gliding or deep inguinal testis via scrotal or inguinal access, in the case of nonpalpable and sonographically undetectable testis, laparoscopy is carried out for diagnosis and simultaneous therapy. In the first postoperative year, adequate follow-up should be done to detect a re-ascensus and/or insufficient growth. Regular self-examinations from the age of 15 serve for the early detection of a testicular tumor that occurs only very rarely (approximately 0.003%)."}, {"id": "pubmed23n1027_23747", "title": "Comparison of diagnostic and treatment guidelines for undescended testis.", "score": 0.01452020202020202, "content": "Cryptorchidism or undescended testis is the single most common genitourinary disease in male neonates. In most cases, the testes will descend spontaneously by 3 months of age. If the testes do not descend by 6 months of age, the probability of spontaneous descent thereafter is low. About 1%-2% of boys older than 6 months have undescended testes after their early postnatal descent. In some cases, a testis vanishes in the abdomen or reascends after birth which was present in the scrotum at birth. An inguinal undescended testis is sometimes mistaken for an inguinal hernia. A surgical specialist referral is recommended if descent does not occur by 6 months, undescended testis is newly diagnosed after 6 months of age, or testicular torsion is suspected. International guidelines do not recommend ultrasonography or other diagnostic imaging because they cannot add diagnostic accuracy or change treatment. Routine hormonal therapy is not recommended for undescended testis due to a lack of evidence. Orchiopexy is recommended between 6 and 18 months at the latest to protect the fertility potential and decrease the risk of malignant changes. Patients with unilateral undescended testis have an infertility rate of up to 10%. This rate is even higher in patients with bilateral undescended testes, with intra-abdominal undescended testis, or who underwent delayed orchiopexy. Patients with undescended testis have a threefold increased risk of testicular cancer later in life compared to the general population. Self-examination after puberty is recommended to facilitate early cancer detection. A timely referral to a surgical specialist and timely surgical correction are the most important factors for decreasing infertility and testicular cancer rates."}, {"id": "pubmed23n0056_419", "title": "The undescended testicle.", "score": 0.01428930817610063, "content": "Cryptorchidism results from a complex hereditary series of incompletely understood events involving the HPG axis. The incidence is indirectly related to birth weight and dramatically decreases during the first 3 months after birth. Many nonscrotal testes are retractile and require no therapy whatsoever. True cryptorchid testes develop identifiable histologic alterations within 2 years of parturition, and endocrine abnormalities are often detectable during infancy. Hormonal therapy with hCG is effective in causing descent in only a small percent of children with cryptorchidism. GnRH nasal spray is no different from placebo in double-blind studies in which retractile testes have been excluded. The results are best in low-lying testes and in older children, but a recognized late-failure rate requires continued surveillance. HCG therapy appears to be of little use in nonpalpable cryptorchid testes. The risk of testicular cancer is increased in men with a history of cryptorchidism and even includes the contralateral descended testes. This risk may be reduced by early orchidopexy. Fertility is impaired in men with cryptorchidism and is reported to be no better than 75% and 50%, respectively, in men who have undergone successful unilateral or bilateral orchidopexy. There is unconfirmed evidence that orchidopexy carried out before the age of 2 years may improve these fertility rates. It is recommended that all children with cryptorchid testes undergo treatment by the age of 1 or 2 years. The parents of children with a nonpalpable testis should be informed of the high rate of testicular absence. If hormonal therapy is to be used, it must be initiated at 10 months of age. Treatment failures must be identified quickly to allow prompt referral of these children to a pediatric urologist or surgeon for orchidopexy."}, {"id": "pubmed23n0362_16775", "title": "Management of undescended testis.", "score": 0.013716404077849862, "content": "The term cryptorchidism indicates a testis, which has failed to descend to the scrotum and is located at any point along the normal path of descent or at an ectopic site. Hormones play a pivotal role in testicular descent except during the migration to the level of internal inguinal ring. Cryptorchidism is present in about 4.5% of newborns with a higher incidence in preterms. The incidence decreases to 1.2% by the first year. It is classified as palpable and impalpable. The most common site of an ectopic testis is superficial inguinal pouch. Retractile testis is often bilateral and most common in boys between 5 and 6 years of age. Hypospadias and inguinal hernias are the most common associated anomalies seen with undescended testis. A thorough clinical examination helps in arriving at the etiology. A short hCG stimulation test helps to exclude anorchia. Different imaging techniques are of little help in diagnosis and require the help of an experienced radiologist. Laparoscopy has an important role in the diagnosis and management of undescended testis. The common complications include torsion and atrophy of testis. Infertility is seen in about 40% of unilateral and 70% of bilateral cryptorchidism. Undescended testis is 20 to 40 times more likely to undergo malignant transformation than normal testis. Both hCG and GnRH have been used with limited success in these children. All boys with cryptorchidism should be referred to a pediatric surgeon before 2 years of age. These children should be followed up every year after surgery to identify testicular tumors."}, {"id": "pubmed23n0909_21405", "title": "Practical approach to evaluating testicular status in infants and children.", "score": 0.013429571303587053, "content": "To review the differences between normal, retractile, ectopic, ascended, and undescended testes and to describe the optimal way to perform a testicular examination to distinguish one from the other, as well as to demonstrate that ultrasound imaging is not necessary and to clarify when to consider specialist referral. This paper is based on selected findings from a MEDLINE search on undescended testes and orchiopexy referrals, and on our experience at the Urology Clinic at the Children's Hospital of Eastern Ontario in Ottawa, including review of referrals to our clinic for undescended testes and the resultant findings of normal variants versus surgical cases. The MeSH headings used in our MEDLINE search included <iundescended testicle, retractile testicle, ectopic testicle, ascended testicle, referral and consultation,</i and <iorchiopexy</i. An <iundescended testis</i is defined as the true absence of one testis (or both testes) from normal scrotal position. Ectopic and ascended testes will likewise be absent from the scrotum, the latter having been present at one point in development. Differentiating among testicular examination findings is important, as descended and retractile testes are managed conservatively, while prompt surgical intervention should be offered for ascended, ectopic, and undescended testes. Uncertainty surrounding the diagnosis of an undescended testis causes anxiety, might lead to unwarranted imaging, and might increase the wait list for specialty assessment. For this reason, avoidance of ultrasound in the evaluation of undescended testes was included in the recent Choosing Wisely Canada campaign. We seek to clarify the physical examination findings in the evaluation of possible undescended testes, the suggested referral parameters, and the subsequent management. Undescended testes and their variants are common. As decision for referral is based on the primary care physician's physical examination findings, we clarify distinguishing between normal and abnormal findings on testicular examination to aid in appropriate referral for subspecialist evaluation. Consultation, if needed, should be sought at 6 months' corrected gestational age, or at detection if later than 6 months, without delay for ultrasound imaging, as surgical management is recommended for those patients with undescended, ectopic, or ascended testes."}, {"id": "pubmed23n0085_20490", "title": "[Treatment of an undescended testis with human chorionic gonadotropin (HCG)].", "score": 0.01335978835978836, "content": "Seventy one patients with ectopic testis of age between 2 and 11.5 years were treated with human chorionic gonadotropin (HCG) at doses recommended by the International Health Foundation. The descent of testis to the scrotum was achieved in almost half of the treated boys (49.3%). The descent was successful mainly in cases of lower inguinal position of the undescended testicle, and only rarely when the testicle was situated higher. The descent was never successful in cases when the scrotum was small and underdeveloped."}, {"id": "pubmed23n0263_12527", "title": "Effect of human chorionic gonadotrophin (hCG)/follicle-stimulating hormone treatment versus hCG treatment alone on testicular descent: a double-blind placebo-controlled study.", "score": 0.012940151797934552, "content": "The medical treatment of retentio testis remains controversial because of ineffectiveness and/or adverse events. Follicle-stimulating hormone (FSH) seems to influence the spontaneous descent of the testis; furthermore, it induces luteinizing hormone (LH) receptors. Therefore, we performed a double-blind placebo-controlled study to investigate the effect of FSH with human chorionic gonadotrophin (hCG) versus hCG alone in retentio testis patients. Twenty-two boys with retentio testis were investigated, excluding retractile testis. Group A (N = 14: four with bilateral and 10 with unilateral retentio testis; mean age 3.15 years) was treated with 150 IU of FSH twice a week for 2 weeks followed by 150 IU of FSH and 250 IU of hCG (half the recommended World Health Organization dose) twice a week for another 4 weeks. Group B (N = 8: two with bilateral and six with unilateral retentito testis; mean age 3.3 years) was treated with 250 IU of hCG twice a week for 6 weeks. Testicular position, volume and consistency as well as the appearance of the scrotum and the penile length were determined at the start of the treatment as well as at weeks 2, 4, 6 and 12 by two independent investigators. Blood investigation consisted of measurements of LH, FSH, testosterone and sex hormone-binding globulin. Successful descent was considered when the testis reached a mid- or low scrotal position. In group A, 6/18 testes descended successfully. In group B, 6/10 testes descended. Of the unsuccessfully treated patients, six of group A and three of group B underwent surgery.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0950_12835", "title": "The volume of unilaterally undescended testis after hCG therapy compared to orchidopexy and combined methods.", "score": 0.012673436167738934, "content": "The aim of the study was to compare the effects of human chorionic gonadotropin (hCG) therapy with those of surgical or combined therapy on testicular volume (TV) in boys at different ages with unilateral canalicular undescended testis (UDT). In total, 155 boys aged 1 to 12 years were treated: either surgically (ST), or by 50 IU/kg body weight hCG administration every three days for five weeks (HT), or by a combination of the two. The patients underwent ultrasound examination of TV before the treatment, 9-12 (median 10) and 24-39 months (median 32) after therapy. The testicular atrophy index (TAI) of the affected testicle was calculated. The success rate was 94.7% for ST, 39.2% for HT and 98% for HST patients. The atrophy rate was 5.3% for ST, 0% for HT and 2% for HST. Neither treatment type nor patient age significantly influenced gonadal atrophy. No significant differences in TV of the affected testis were observed after treatment between the groups. The TAI values were significantly the lowest in HT group (p = 0.0006). Both TV and TAI changes from the baseline values did not differ between the treatment groups. At the 24- to 39-month follow-up, no significant differences were observed in the change in baseline TV and baseline TAI between age groups. TV of the affected testis increased significantly (p = 0.0000), and TAI decreased significantly over time (p = 0.01), with no significant differences depending on the age group, treatment type or the interaction of the two factors. The hCG therapy did not impair the development of affected and healthy testes, neither as single nor as neoadjuvant therapy, both during early assessment and after 2-3 years. Patients' age at the initiation of treatment seems irrelevant."}, {"id": "wiki20220301en003_74506", "title": "Testicle", "score": 0.012265512265512264, "content": "Testes follow the \"path of descent\" from high in the posterior fetal abdomen to the inguinal ring and beyond to the inguinal canal and into the scrotum. In most cases (97% full-term, 70% preterm), both testes have descended by birth. In most other cases, only one testis fails to descend (cryptorchidism) and that will probably express itself within a year. Pubertal The testes grow in response to the start of spermatogenesis. Size depends on lytic function, sperm production (amount of spermatogenesis present in testis), interstitial fluid, and Sertoli cell fluid production. After puberty, the volume of the testes can be increased by over 500% as compared to the pre-pubertal size. Testicles are fully descended before one reaches puberty. Clinical significance Protection and injury"}, {"id": "pubmed23n0045_8652", "title": "Cryptorchidism: incidence and sperm quality in infertile men.", "score": 0.012251779692768828, "content": "In a population of 8500 men attending the andrology outpatient clinic, 200 men (2.35%) were recorded as having some disturbances with the descent of the testes into the scrotum. Medical history of the patients revealed that 51 underwent unilateral orchidopexy; 40 bilateral orchidopexy; and 24 were treated with human chorionic gonadotropin in order to induce descent of their testes. In addition, 6 patients reported spontaneous descent of the testes, and 13 others were found to be unilaterally cryptorchid upon physical examination. Results of semen analysis, hormonal profile, testes position, and testicular volume were compared to those of 105 proven fertile men. The major finding of this study shows that post-partum undescended testes suffer from primary Sertoli cell malfunction as reflected by elevated serum follicle stimulating hormone levels. Serum luteinizing hormone and testosterone levels were within the normal range. Surgical descent of the testes did not improve sperm production, proved by low sperm quality of all the study groups, compared to the cryptorchid group. Among the patients who were operated on, no correlation was found between age at operation and semen variables. All groups showed poor sperm quality which can be defined as oligoteratoasthenozoospermia. The degree of spermatogenic damage was in the following order of diagnosis or treatment: bilateral orchidopexy greater than cryptorchid testes greater than hormonal treatment greater than unilateral orchidopexy greater than late spontaneous descent of the testes. Thus, it is advisable to postpone surgical treatment of cryptorchidism and apply this only after a waiting period, and if the hormonal approach has failed to descend the testis."}, {"id": "pubmed23n0378_7552", "title": "Pitfalls of conventional human chorionic gonadotropin stimulation test to detect hormonally functional cryptorchid testes in midchildhood.", "score": 0.012142703632065335, "content": "To report two cases misdiagnosed as bilateral anorchism in midchildhood on the basis of multiple conventional human chorionic gonadotropin (HCG) stimulation tests and sonograms of the abdomen and pelvis. In two young male patients with cryptorchidism who were considered to have anorchism, we describe the findings on clinical examination, the testosterone levels before and after standard HCG stimulation testing, and sonographic findings during the midchildhood period. In both cases, as the children approached puberty the diagnosis was found to be incorrect. Two boys, 8 and 91/2 years old, were seen in consultation in our Pediatric Endocrine Clinic with a presumed diagnosis of anorchism. In the first case, multiple conventional HCG stimulation tests were done. In the second case, a single stimulation test was performed during routine follow-up assessments. In both cases, testosterone levels before and after HCG stimulation were consistent with the diagnosis of absent functional testicular tissue. Sonograms of the abdomen and pelvis also failed to detect the testicles. Both patients were ultimately noted to be pubertal (at 14 1/2 and &gt;13 1/2 years, respectively) and to have early pubertal testosterone levels. A testicle was detected in one patient by abdominal computed tomographic scan and in the other by palpation of the inguinal canal. Conventional dosing and duration of the HCG stimulation test, as widely recommended in standard textbooks and in articles in the medical literature, may not elicit positive HCG-induced testosterone responses during midchildhood for detection of functional testicular tissue. During the midchildhood period, which is characterized by low gonadotropin, low sex steroid production, and a highly sensitive hypothalamic-pituitary-gonadal axis to feedback inhibition, a prolonged HCG stimulation test-perhaps of 4 to 6 weeks' duration-may be necessary. In addition, other investigational modalities may need to be used to detect the presence of functional testicular tissue during this developmental period."}, {"id": "Surgery_Schwartz_11520", "title": "Surgery_Schwartz", "score": 0.011256863941427699, "content": "the incidence of infertility is approx-imately two times higher in men with unilateral orchidopexy compared to men with normal testicular descent.The use of chorionic gonadotropin occasionally may be effective in patients with bilateral undescended testes, suggest-ing that these patients are more apt to have a hormone insuf-ficiency than children with unilateral undescended testicle. The combination of micro-penis and bilateral undescended testes is an indication for hormonal evaluation and testoster-one replacement if indicated. If there is no testicular descent after a month of endocrine therapy, operative correction should be undertaken. A child with unilateral cryptorchidism should have surgical correction of the problem. The operation is typi-cally performed through a combined groin and scrotal incision. The cord vessels are fully mobilized, and the testicle is placed in a dartos pouch within the scrotum. An inguinal hernia often accompanies a cryptorchid testis. This should be"}, {"id": "pubmed23n0378_10483", "title": "13 Years' experience with the combined hormonal therapy of cryptorchidism.", "score": 0.011236308699483822, "content": "We analyze the results of the combined treatment with luteinizing hormone releasing hormone (LH-RH) and human chorionic gonadotropin (HCG) of a large series of patients with cryptorchidism. Between 1987 and 1999 and after strict differentiation between cryptorchid, retractile and gliding testes, 2,467 boys with 2,962 cryptorchid-gliding testes were treated with the combined hormonal therapy. LH-RH was administrated as a nasal spray at a dosage of 1.2 microg daily for a period of 4 weeks. HCG was injected intramuscularly, 5 times at 2-day intervals at a dosage adjusted according to the age. In the prospective study 2,476 boys with 2,962 cryptorchid testes were hormonally treated. Of the 2,962 evaluated cases 1,200 (40.52%) have been treated surgically after the hormone therapy. In 1,762 cases, 59.48% of cryptorchid testes were in the scrotum after combined hormone treatment. Treatment with LH-RH and HCG induced the descent of the testes to a normal scrotal position of boys with cryptorchidism in 59.48% of the evaluated cases. The combined treatment was effective for inducing descent of cryptorchid and gliding testes. According to the evaluated intraoperative findings, the failure of the combined therapy in 40.52% of the cases is due to the fact that the free descent is limited by local factors such as anatomical alterations of the inguinal canal, epididymal abnormalities or ectopic distal attachment of the lig. testis."}, {"id": "wiki20220301en017_70954", "title": "Cryptorchidism", "score": 0.010725117176730081, "content": "Undescended testes are associated with reduced fertility, increased risk of testicular germ-cell tumors, and psychological problems when fully-grown. Undescended testes are also more susceptible to testicular torsion (and subsequent infarction) and inguinal hernias. Without intervention, an undescended testicle will usually descend during the first year of life, but to reduce these risks, undescended testes can be brought into the scrotum in infancy by a surgical procedure called an orchiopexy. Although cryptorchidism nearly always refers to congenital absence or maldescent, a testis observed in the scrotum in early infancy can occasionally \"reascend\" (move back up) into the inguinal canal. A testis that can readily move or be moved between the scrotum and canal is referred to as retractile. Cryptorchidism, hypospadias, testicular cancer, and poor semen quality make up the syndrome known as testicular dysgenesis syndrome. Signs and symptoms"}, {"id": "Pediatrics_Nelson_3507", "title": "Pediatrics_Nelson", "score": 0.010219239831984427, "content": "Retractile testes are normal testes that retract into the inguinal canal from an exaggerated cremasteric reflex. The diagnosis of retractile testes is likely if testes are palpable inthe newborn period but not at later examination. Frequentlyparents describe seeing their son’s testes in his scrotum whenhe is in the bath and seeing one or both “disappear” when hegets cold. Available @ StudentConsult.com Torsion of the testis is an emergency requiring prompt diagnosis and treatment to save the affected testis. Torsion accounts for 40% of cases of acute scrotal pain and swelling and is the major cause of the acute scrotum in boys less than 6 years of age. It is thought to arise from abnormal fixation of the testis to the scrotum. On examination the testicle is swollen and tender, and the cremasteric reflex is absent. The absence of blood flow on nuclear scan or Doppler ultrasound is consistent with torsion."}, {"id": "pubmed23n0347_9355", "title": "[Cryptorchism. Outcome of treatment and referral patterns in an unselected group of patients in a 3-year period].", "score": 0.009900990099009901, "content": "The age of diagnosis and referral together with the efficacy of HCG treatment were studied retrospectively in 196 unselected cryptorchid patients seen over a period of three years. The median age of diagnosis was 2 years and 7 months, whereas the median age of referral was three years later. During the period of study, guidelines for referral and therapy were published in a nationwide journal and in the local region, and a slight but significant fall in age of referral was seen thereafter. The median age of treatment with HCG, 92 patients, was 6 years and 11 months, and median age of surgery, was 7 years and 7 months. The rate of success with HCG was for bilateral testes 41% and for unilateral testes 21%, giving an overall success rate of 30%. This result is lower than previously reported, which is most likely explained by a higher suprascrotal position of testes before treatment. Guidelines and recommendations for referral and therapy seem to influence the time of referral, which is, however, in this study not in accordance with the consensus of definitive treatment before the age of two years. Early diagnosis is recommended and should be followed by referral to a paediatric department with particular interest and knowledge about cryptorchidism."}, {"id": "pubmed23n0689_4877", "title": "Orchiopexy-laparoscopy or traditional surgical technique in patients with an undescended palpable testicle.", "score": 0.009852216748768473, "content": "To compare orchiopexy by laparoscopy versus traditional surgical technique in patients with an undescended palpable testicle in the inguinal canal. A prospective, comparative, observational, longitudinal, and double-blind research was done between August 2006 and March 2009 in the Centro de Especialidades Médicas del Estado de Veracruz, \"Dr. Rafael Lucio\"; 63 patients underwent surgery, age 1-10 years, all with the diagnosis of palpable undescended testicle in the inguinal canal; in 33 patients, the traditional surgical technique and in 30 patients laparoscopy were done. A visual analogue scale (VAS) was used to evaluate post-surgery pain. A testicle ultrasound was practiced before surgery and at 6 months after it. The majority of patients were 1-4 years old with a median age of 2.3 years; 51 cases were unilateral and 12 cases were bilateral; 37 testicles were descended with the open traditional surgical technique and 38 through laparoscopy (75 testicles); 44 on the right side and 31 on the left side; there was a hernia associated with 37 undescended testicles, 23 with open surgical technique, and 14 by laparoscopy, without relapsing in any patient. The median surgery time with the open surgical technique was 38 minutes and by laparoscopy, it was 45 minutes. The gobernaculum testis was sectioned by laparoscopy in 23 descended testicles to facilitate the procedure, in the remaining 11 it was not necessary; whereas in the open technique, all the gobernaculum testis were sectioned. In 80% of cases, the laparoscopy caused less pain when compared with the other technique. All patients regardless of the technique used left hospital during the first 24 hours. All have had follow-up for more than 6 months with a median of 18 months, with satisfactory results in relation to size and location of the testicle, with a good ultrasound correlation, and not finding any statistical differences between surgical techniques. There were no accidents with any of the techniques, and 1 patient with the open technique had an important hematoma; hemophilia was later diagnosed in the patient. The esthetical aspect was better with laparoscopy, but the cost was 15% more expensive with the open technique. Both techniques had satisfactory results without any significant differences to make us choose one over the other. It is the surgeons' decision based on experience and training on laparoscopy to choose any of the techniques."}, {"id": "pubmed23n0204_6465", "title": "[Surgical indications in testicular ectopias].", "score": 0.00980392156862745, "content": "The ideal age for treatment is still controversial. However, all recent histological studies show that the number of spermatogonia and the diameter of the tubules remain virtually normal until the beginning of the third year of life. Treatment therefore should take place in the second year, after all chances of spontaneous migration have vanished and before lesions due to cryptorchidism develop. Chorionic gonadotropic hormones should be used systematically; they avoid surgery in only one out of five cases, but they make it possible to test the possibilities of testicular descent and testosterone secretion. Surgical treatment should follow precise rules. The spermatic vessels might, if necessary, be severed, but care must be taken to preserve the gubernaculum. The alternative is, microsurgical autotransplantation. Only time will show whether the poor fertility of these patients is bettered by early treatment."}, {"id": "wiki20220301en017_70973", "title": "Cryptorchidism", "score": 0.009732417712526639, "content": "When the undescended testis is in the inguinal canal, hormonal therapy is sometimes attempted and very occasionally successful. The most commonly used hormone therapy is human chorionic gonadotropin (hCG). A series of hCG injections (10 injections over five weeks is common) is given and the status of the testis/testes is reassessed at the end. Although many trials have been published, the reported success rates range widely, from roughly 5% to 50%, probably reflecting the varying criteria for distinguishing retractile testes from low inguinal testes. Hormone treatment does have the occasional incidental benefits of allowing confirmation of Leydig cell responsiveness (proven by a rise of the testosterone by the end of the injections) or inducing additional growth of a small penis (via the testosterone rise). Some surgeons have reported facilitation of surgery, perhaps by enhancing the size, vascularity, or healing of the tissue. A newer hormonal intervention used in Europe is the use"}, {"id": "pubmed23n0307_8893", "title": "The fate of undescended testes in patients with gastroschisis.", "score": 0.009708737864077669, "content": "Cryptorchidism is frequently associated with gastroschisis, yet little is published on its management in such circumstances. In a review of 10 consecutive boys with gastroschisis since 1980, 4 had undescended testes. Gestational age and birth weight did not differ from the 6 boys with normally descended testes. The first two patients had associated arthrogryposis multiplex congenita. The first underwent bilateral orchidopexy at 9 years of age for inguinal testes. In the second patient, the left testis was intraabdominal at the level of the sigmoid colon at birth; at 3 months of age, when a left inguinal hernia repair was required, left groin exploration revealed the testis at the internal ring and orchidopexy was performed successfully. In the third patient the left spermatic vessels were divided at the time of gastroschisis repair and the testis anchored in the prebubic area. The second-stage orchidopexy was performed at 16 months. In the last patient the intraabdominal testis could be placed in a scrotal pouch without mobilisation or division of the vessels. From our experience and a review of the literature we conclude that: 1) undescended testes are frequently associated with gastroschisis; 2) mechanical factors rather than prematurity are likely responsible for this association; 3) if the testis easily reaches the scrotum, orchidopexy can be done safely at the time of gastroschisis repair; 4) if the testis does not reach easily and appears to have a gubernaculum, it may be preferable to leave it in place since spontaneous descent can occur."}, {"id": "pubmed23n0263_19027", "title": "[Results of treating cryptorchism with HCG in boys previously qualified for surgical treatment].", "score": 0.009708737864077669, "content": "Hundred one boys, aged between 1.5 and 13 years, previously classified for surgery for uni- or bilateral cryptorchidism were examined. In 78 patients (77.2%) cryptorchidism was diagnosed, including 51 cases (50.5%) of unilateral undescended testicle. In the remaining 23 boys (22.9%) migrating testes were found: unilateral in 6 cases (5.9%) and bilateral in 17 cases (16.8%). An improvement was achieved after HCG therapy in 21 cases of migrating testes (91.3%), partial improvement in 1 case (4.35%), and no effect in 1 case (4.35%). The treatment with HCG produced recovery in 20 cases (25.6%) of cryptorchidism, partial improvement in cases (23.2%), and no effect in 40 cases (51.2%). A complete recovery was achieved in 41 out of 101 patients, i.e. in 40.6%, partial improvement in 19 cases (18.6%), and no effect in 41 cases (40.6%). The treatment with HCG enabled an avoidance of unnecessary surgery in 40.6% of all examined boys."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": true, "char_ranges": [[334, 417]], "word_ranges": [[53, 67]], "text": "none of these 3 options have any association with drusen in the contralateral eye)."}, "2": {"exist": true, "char_ranges": [[418, 665]], "word_ranges": [[67, 105]], "text": "AMD in its exudative form presents with both this clinical presentation (loss of vision and metamorphopsia) and the characteristic fundus described in the question, in addition to the fact that drusen are usually observed in the contralateral eye."}, "3": {"exist": true, "char_ranges": [[334, 417]], "word_ranges": [[53, 67]], "text": "none of these 3 options have any association with drusen in the contralateral eye)."}, "4": {"exist": true, "char_ranges": [[334, 417]], "word_ranges": [[53, 67]], "text": "none of these 3 options have any association with drusen in the contralateral eye)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "None of the other three options give that clinical picture (DPV is usually asymptomatic, CRVO produces abrupt and total or near total loss of vision with the typical \"cherry red spot\" in the fundus, and both an arteritic and non-arteritic NOIA would produce papillary edema usually with peripapillary but not macular hemorrhages, and none of these 3 options have any association with drusen in the contralateral eye). AMD in its exudative form presents with both this clinical presentation (loss of vision and metamorphopsia) and the characteristic fundus described in the question, in addition to the fact that drusen are usually observed in the contralateral eye.", "full_answer_no_ref": "None of the other three options give that clinical picture (DPV is usually asymptomatic, CRVO produces abrupt and total or near total loss of vision with the typical \"cherry red spot\" in the fundus, and both an arteritic and non-arteritic NOIA would produce papillary edema usually with peripapillary but not macular hemorrhages, and none of these 3 options have any association with drusen in the contralateral eye). AMD in its exudative form presents with both this clinical presentation (loss of vision and metamorphopsia) and the characteristic fundus described in the question, in addition to the fact that drusen are usually observed in the contralateral eye.", "full_question": "An 84-year-old woman presents with loss of vision in the left eye of 4 days of evolution accompanied by metamorphopsia. The macula shows abundant hard exudates, two small deep hemorrhages and a localized neurosensory retinal detachment. In the contralateral eye there are abundant soft drusen. Which of the following diagnoses do you think is the most likely?", "id": 311, "lang": "en", "options": {"1": "Acute posterior vitreous detachment.", "2": "Exudative age-related macular degeneration (AMD).", "3": "Central retinal artery obstruction.", "4": "Non-arteritic anterior ischemic optic neuropathy.", "5": NaN}, "question_id_specific": 217, "type": "OPHTHALMOLOGY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0059_11482", "title": "[Preventive treatment using laser of age-related macular degeneration of the contralateral eye after age-related macular degeneration of the first eye].", "score": 0.01633125442036906, "content": "Since 1982, and with informed patient consent, we have photocoagulated confluent drusen and limited serous pigment epithelium detachment (SPED) in the fellow eye of ten patients suffering from advanced, disciform type, age-related macula degeneration (ARMD). This treatment was only carried out on appearance of metamorphopsia. Photocoagulation was performed with either the green ray of the argon laser, or the yellow ray of a dye laser. Spots of about 200 microns were placed in a grid-like fashion among the drusen. No complications were observed due to the treatment. The follow-up period on these ten patients, eight women and two men, mean age 77 years, was two to eight years, and the three patients have died. The drusen disappeared completely in three patients and partially in one. The functional results seemed favorable in three cases. In one case of confluent drusen associated with SPED and serous retinal detachment, vision improved remarkably from 0.3 to 0.5 with a Parinaud 2, with a follow-up of five years. In another case, the improvement was from 0.4 to 0.7 but the patient died after only a few months. In another case, vision has been stable for five years. The vision of the seven remaining patients deteriorated; three cases showed central areolar sclerosis, and one case a localised new vessel with vision less than 0.1. In three cases vision dropped to 0.2 and Parinaud 6, but they have been stable for at least four years (eight years for one patient).(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0828_15704", "title": "The causes of hyperreflective dots in optical coherence tomography excluding diabetic macular edema and retinal venous occlusion§.", "score": 0.015640273704789834, "content": "To investigate the causes of hyperreflective dots (HRDs) in spectral domain optical coherence tomography (OCT) excluding diabetic macular edema (DME) and RVO (retinal vein occlusion). The medical records of 56 patients with HRDs documented by OCT were reviewed retrospectively. The patients with DME and RVO were excluded from the study in order to prevent misdiagnosing hard exudates or HRDs. The causes, unilaterality or bilaterality of HRD and demographic properties of the patients with HRD were evaluated. Sixty four eyes of 56 patients having HRDs were included in this study. Of the patients with HRD, 17 (30.36%) were women and 39 (69.64%) were men. The ages of patients were between 13 to 84 years (median 60.18 years). The causes of HRD were as follows: papilledema in 4 eyes (6.25%), active neovascular age related macular degeneration (AMD) in 33 eyes (51.56%), familial dominant drusen in 2 eyes (3.13%), central serous chorioretinopathy in 19 eyes (29.69%) and commotio retina in 2 eyes (3.13%), choroidal folds in one eye (1.56%), branch retinal artery occlusion in one eye (1.56%), central retinal artery occlusion in one patient (1.56%) and Best vitelliform macular dystrophy in one eye (1.56%). The most common cause of HRD was AMD. The causes of HRDs in both eyes were AMD and papilledema. The most common causes of HRDs excluding DME and RVO seem as active exudative AMD. The presence of HRDs in retinal diseases might affect the decisions and the results of the treatment and the prognosis of diseases."}, {"id": "pubmed23n0789_403", "title": "Small retinal haemorrhages accompanied by macular soft drusen: prevalence, and funduscopic and angiographic characteristics.", "score": 0.01546268656716418, "content": "To investigate the prevalence and clinical significance of small retinal haemorrhages accompanied by macular soft drusen in exudative age-related macular degeneration (AMD). This observational case series included patients who had first been diagnosed with exudative AMD. Small retinal haemorrhages were defined as preretinal or intraretinal haemorrhages, no larger than half the disc diameter in size and located within 3000 μm of the fovea centre. If there was more than one haemorrhage, the entire affected area was less than two-thirds of the disc diameter. Macular soft drusen was defined as the presence of soft drusen (≥125 μm in diameter) within the macular area. The presence of retinal angiomatous proliferation (RAP) was estimated based on the results of indocyanine green angiography (ICGA). The prevalence of reticular pseudodrusen was also estimated. Among the 1921 eyes from 1604 patients who were newly diagnosed with exudative AMD during the 40 months prior to the study, 101 eyes (5.3%) from 79 patients presented with the fundus characteristics described above. ICGA images were available for 69 eyes. Among these eyes, 28 eyes (43.1%) and 25 eyes (38.5%) were found to have type 1 and 2 RAP, respectively. A chorioretinal anastomosis (type 3 RAP) was identified in 12 (18.5%) eyes. Reticular pseudodrusen were noted in 78 eyes (77.2%). The presence of small retinal haemorrhages accompanied by macular soft drusen was highly predictive of RAP. The high prevalence of both soft drusen and reticular pseudodrusen in these eyes may suggest a profound decrease in choroidal perfusion in these eyes."}, {"id": "wiki20220301en090_51094", "title": "Drusen", "score": 0.01360146862483311, "content": "Drusen, from the German word for node or geode (singular, \"Druse\"), are tiny yellow or white accumulations of extracellular material that build up between Bruch's membrane and the retinal pigment epithelium of the eye. The presence of a few small (\"hard\") drusen is normal with advancing age, and most people over 40 have some hard drusen. However, the presence of larger and more numerous drusen in the macula is a common early sign of age-related macular degeneration (AMD). Classification Drusen are associated with aging and macular degeneration are distinct from another clinical entity, optic disc drusen, which is present on the optic nerve head. Both age-related drusen and optic disc drusen can be observed by ophthalmoscopy. Optical coherence tomography scans of the orbits or head, calcification at the head of the optic nerve without change in size of globe strongly suggests drusen in a middle-age or elderly patient."}, {"id": "article-133116_10", "title": "Wet Age-Related Macular Degeneration (Wet AMD) -- History and Physical", "score": 0.01280096563115431, "content": "On examination, patients frequently have decreased best-corrected visual acuity (BCVA), and Amsler grid evaluation may reveal areas of central or paracentral scotoma or visual distortion. Ophthalmic examination of the anterior segment of the eye is usually normal. ARMD-related CNV has several different appearances on the dilated funduscopic exam. [17] These include: A gray-green membrane deep into the retina is usually associated with an overlying neurosensory retinal detachment. There may be the presence of blood, lipid, or subretinal fluid. RPE detachments appear clinically as dome-shaped, sharply demarcated elevations of the RPE; these may also be serous, fibrovascular, drusenoid, or hemorrhagic. [18] There may be massive subretinal hemorrhage with central vision loss or, less commonly, breakthrough vitreous hemorrhage with peripheral vision loss. RPE tears. Disciform scars may be present, which may appear as white or yellow subretinal membranes with or without RPE hyperplasia."}, {"id": "pubmed23n0561_1363", "title": "Fifteen-year cumulative incidence of age-related macular degeneration: the Beaver Dam Eye Study.", "score": 0.01245498199279712, "content": "To describe the 15-year cumulative incidence of signs of early and late age-related macular degeneration (AMD). Population-based cohort study. We included 3917 persons, 43 to 86 years of age at the time of a baseline examination in 1988 through 1990 and with information collected in follow-up in 1993 through 1995, and/or 1998 through 2000, and/or 2003 through 2005. Grading of stereoscopic fundus photographs using the Wisconsin Age-Related Maculopathy Grading System. Cumulative incidence of drusen type and size, pigmentary abnormalities, geographic atrophy, and exudative AMD accounting for competing risk of death. The 15-year cumulative incidence was 14.3% for early AMD (the presence of either soft indistinct drusen or the presence of pigmentary abnormalities together with any type of drusen) and 3.1% for late AMD (presence of exudative AMD or geographic atrophy). There was an increased incidence of AMD lesions with age (P&lt;0.05). Individuals &gt; or = 75 years of age at baseline had significantly (P&lt;0.01) higher 15-year incidences of the following characteristics than people 43 to 54 years of age: larger drusen (125 mum in diameter, 24.1% vs 10.6%), soft indistinct drusen (18.7% vs 6.5%), retinal pigmentary abnormalities (20.2% vs 3.7%), exudative macular degeneration (4.4% vs 0.4%), and pure geographic atrophy (3.2% vs 0%). Controlling for age, compared with those with small numbers of only small hard drusen (1-2), those with large numbers of only hard drusen (&gt; or =8) had an increased 15-year age-adjusted incidence of both soft indistinct drusen (16.3% vs 4.7%) and pigmentary abnormalities (10.6% vs 2.7%). Eyes with soft indistinct drusen or pigmentary abnormalities at baseline were more likely to develop late AMD at follow-up than eyes without these lesions (17.8% vs 1.2% and 12.9% vs 1.7%, respectively). We document the long-term incidence of signs of AMD and a continuum from small hard drusen to late AMD in older persons in the population. The 15-year cumulative incidence of late AMD in people &gt; or = 75 years of age (8%) indicates a public health problem of significant proportions because the United States population this age is expected to increase by 54% between 2005 and 2025."}, {"id": "wiki20220301en029_81277", "title": "Macular degeneration", "score": 0.012357938872338077, "content": "AMD-like pathology begins with small yellow deposits (drusen) in the macula, between the retinal pigment epithelium and the underlying choroid. Most people with these early changes (referred to as age-related maculopathy) still have good vision. People with drusen may or may not develop AMD. In fact, the majority of people over age 60 have drusen with no adverse effects. The risk of developing symptoms is higher when the drusen are large and numerous, and associated with the disturbance in the pigmented cell layer under the macula. Large and soft drusen are thought to be related to elevated cholesterol deposits. Early AMD Early AMD is diagnosed based on the presence of medium-sized drusen, about the width of an average human hair. Early AMD is usually asymptomatic. Intermediate AMD Intermediate AMD is diagnosed by large drusen and/or any retinal pigment abnormalities. Intermediate AMD may cause some vision loss, but, like early AMD, it is usually asymptomatic."}, {"id": "wiki20220301en210_15682", "title": "Maculopathy", "score": 0.012037742235762039, "content": "A maculopathy is any pathological condition of the macula, an area at the centre of the retina that is associated with highly sensitive, accurate vision. Forms of maculopathies Age-Related Macular Degeneration is a degenerative maculopathy associated with progressive sight loss. It is characterised by changes in pigmentation in the Retinal Pigment Epithelium, the appearance of drusen on the retina of the eye and choroidal neovascularization. AMD has two forms; 'dry' or atrophic/non-exudative AMD, and 'wet' or exudative/neovascular AMD. Malattia Leventinese (or Doyne’s honeycomb retinal dystrophy) is another maculopathy with a similar pathology to wet AMD. Cellophane Maculopathy A fine glistening membrane forms over the macula, obscuring the vision."}, {"id": "pubmed23n0520_6757", "title": "The time pattern of bilateral exudative age-related macular degeneration.", "score": 0.012020905923344948, "content": "To study time patterns in bilateral exudative age-related macular degeneration (AMD) and the pattern of drusen before and after the onset of exudative AMD. Out of 2220 individuals in the Icelandic genetic study of AMD, 151 had bilateral exudative AMD. We searched for previous records in the Icelandic University Retina Unit. For the 65 patients with a fluorescein angiography record of both eyes, we established the time between the onset of disease in each eye. For the 53 patients with colour fundus photographs of the latter eye taken prior to the occurrence of exudative disease, we graded the drusen before and after the onset of exudative AMD in the second eye. The time interval between the onset of exudative AMD in the first and second eyes was 2.5 years (95% CI: 1.8-3.2; n = 65) and the median was 1.8 years. In 82% of cases the second eye was affected within 4 years. Soft drusen in the macula were found in 95% of eyes that later developed exudative disease (n = 53). Soft and hard drusen decreased in number in the central macula following the development of exudative disease. Bilateral exudative AMD develops within a few years in both eyes. Drusen are less visible following the onset of exudative AMD in the second eye."}, {"id": "article-133116_42", "title": "Wet Age-Related Macular Degeneration (Wet AMD) -- Differential Diagnosis", "score": 0.01183003380009657, "content": "Breakthrough vitreous hemorrhage can also occur in wet ARMD, and diagnosis may be challenging if there is a poor view on dilated funduscopic examination. Diagnosis may be established by evaluating the patient’s fellow eye or obtaining a thorough history. Other potential causes of vitreous hemorrhage include: Proliferative diabetic retinopathy Retinal tear or retinal detachment Hemorrhagic posterior vitreous detachment Neovascularization from other causes, including vein occlusions, radiation retinopathy, or sickle cell retinopathy"}, {"id": "pubmed23n0082_3185", "title": "Age-related macular degeneration.", "score": 0.011821305841924399, "content": "Age-related macular degeneration (AMD) is the leading cause of irreversible severe visual loss in the United States in people over 50 years of age. The nonexudative stage includes hard drusen (associated with localized dysfunction of the retinal pigment epithelium [RPE]), soft drusen (associated with diffuse dysfunction of the RPE), and geographic (areolar) atrophy. These fundus changes may predispose the eye to develop the neovascular/exudative stages of AMD. Most patients who develop severe visual loss from AMD have this exudative stage. Treatment for AMD has been shown to be effective for only a small proportion of patients who have a well-defined choroidal neovascular membrane (CNVM) more than 200 microns from the foveal center. Even in successfully treated cases, severe visual loss is postponed only for about 18 months because of the high rate of recurrent CNVMs that extend into the fovea. Thus, despite recent breakthroughs in laser treatment for AMD, most patients who develop the exudative form of AMD will develop central visual impairment. At the present time, the only available treatments for the majority of patients who develop the exudative form of AMD are low vision aids. Investigators are currently evaluating whether treatment is effective for membranes within 200 microns of the foveal center. Future studies need to be directed toward further understanding of the pathogenesis, treatment and prevention of the blinding complications of AMD."}, {"id": "pubmed23n0371_1601", "title": "[Macular diseases--application of automated static perimetry and optical coherence tomography].", "score": 0.011765112979687877, "content": "The usefulness of automated static perimetry and optical coherence tomography in the management of macular diseases has been described. Scotomata in eyes with central serous chorioretinopathy could be evaluated with central 10-degree automated static perimetry. The degree of visual field defects in eyes with the disease varied greatly with mean deviation of -10 dB or less in as many as 10% of the subjects. Although retinitis pigmentosa is a diffuse retinal dystrophy, eyes with a moderately advanced stage of retinitis pigmentosa should be managed as a macular disease, because the functioning retina is confined within the vascular arcade. The progressive nature in this stage of the disease could be demonstrated with a central 10-degree automated static perimetry measured once or twice a year and the use of univariate linear regression of mean deviation, in half of the patients with a mean follow-up period of 5 years. Functional recovery in eyes with exudative age-related macular degeneration after laser surgery or submacular surgery could be evaluated with central 10-degree automated static perimetry. Eyes with increased mean deviation in spite of reduced visual acuity after therapeutic intervention should also be evaluated. Macular function could also be evaluated using a color test. A newly developed color saturation discrimination test was applied to patients with age-related macular degeneration, retinitis pigmentosa, and cone dystrophy. The degree of dyschromatopsia was less in eyes with age-related macular degeneration than in those with retinitis pigmentosa or cone dystrophy with the same level of acuity loss. The highly protrusive nature of the orange-red nodule in eyes with idiopathic polypoidal choroidal vasculopathy was demonstrated with dimensional measurement with OCT. The degree of protrusion was greater than in eyes with serous pigment epithelial detachment, which suggests that the polypoidal lesion is covered with rigid tissues including Bruch's membrane. Parafoveal retinal sensitivity obtained with automated static perimetry was studied in correlation with retinal thickness measured using OCT in eyes with branch retinal vein occlusion showing macular edema without macular non-perfusion or massive retinal hemorrhages. The increased retinal thickness due to macular edema is closely correlated with retinal sensitivity both at the fovea and in the parafoveal area. Eighty-nine phakic eyes of 46 patients with retinitis pigmentosa patients were studied to detect cystoid macular edema using OCT. Cystoid lesions were observed in the macula in 12 eyes in 6 (13%) of 46 patients. Some eyes with OCT-proven cystoid macular edema did not show dye pooling in the fluorescein angiogram. The width of the total area of cystoid lesions was positively correlated with best-corrected visual acuity but the thickness of the neurosensory retina at the center of the fovea was not. OCT findings of successfully repaired macular holes were categorized into 3 groups. Eyes with U-type showed a normal foveal contour and a dark layer corresponding to the outer segment of photoreceptors. Eyes with V-type showed a notch in the surface of repaired neurosensory retina without a dark layer on the retinal pigment epithelium. Those with W-type showed a defect of the neurosensory retina, where the retinal pigment epithelium was exposed. The visual results were excellent in eyes with U-type, but poor in those with W-type."}, {"id": "wiki20220301en564_9633", "title": "Acute visual loss", "score": 0.011753269706610111, "content": "Acute visual loss is a rapid loss of the ability to see. It is caused by many ocular conditions like retinal detachment, glaucoma, macular degeneration, and giant cell arteritis, etc. Main causes Retinal detachment Retinal detachment should be considered if there were preceding flashes or floaters, or if there is a new visual field defect in one eye. If treated early enough, retinal tear and detachment can have a good outcome. Glaucoma Angle-closure glaucoma should be considered if there is painful loss of vision with a red eye, nausea or vomiting. The eye pressure will be very high typically greater than 40 mmHg. Emergent laser treatment to the iris may prevent blindness. Macular degeneration Wet macular degeneration should be considered in older people with new distortion of their vision with bleeding in the macula. Vision can often be regained with prompt eye injections with anti-VEGF agents. Giant cell arteritis"}, {"id": "pubmed23n0867_10836", "title": "[Age-related Macular Degeneration in the Japanese].", "score": 0.010682307605122975, "content": "Age-related macular degeneration (AMD) in the Japanese often shows different clinical features from those described in Caucasians. For example, we often observe choroidal neovascularization (CNV) in elderly patients without drusen in the fundus. The high incidence of polypoidal choroidal vasculopathy (PCV) in AMD among Japanese is well-known. The reason why such differences occur in clinical manifestations of AMD has been one of my main interests. In this review article, I will discuss the characteristics of AMD in the Japanese population, as found in our recent study. I. Prevalence and clinical characteristics of AMD in the Japanese population. Cohort studies are important to determine the prevalence and incidence of diseases. In Japan, cohort studies began to be carried out rather late compared with Western countries. Although good cohort studies from Japan are reported in the literature, the size of the cohorts was not sufficiently large to determine the prevalence of AMD. However, a recent meta-analysis of Asian cohorts has shown that the prevalence of late AMD in Asians is not different from that reported in Caucasians. On the other hand, the prevalence of early AMD appears lower in the Japanese than in Caucasians. Recently, we have published the results of the Nagahama Cohort study. In this cohort study, we found a high prevalence of drusen. It seems that the incidence of dry AMD is likely to increase among Japanese. In Japan, most retina specialists classify AMD into three categories : typical AMD, PCV, and retinal angiomatous proliferation (RAP). However, there are no definite diagnostic criteria to distinguish between the three conditions. To compare the clinical features of Japanese and Western cases of AMD, and to determine the incidence of the three types of AMD, we exchanged data about 100 consecutive cases between Kyoto University and Centre d'Ophtalmologie de Paris, France. Interestingly, the diagnoses made by the two institutes were not always in agreement. We also found more cases of PCV among the Japanese than among the French. II. PCV. About 50% of exudative AMD cases in the Japanese population are PCV. Because of its peculiar angiographic findings, PCV has long been considered to be a distinct clinical entity different from the usual exudative AMD. Also, there have been serious discussions on the nature of PCV. In our analyses, about 20% of PCV cases show rather large lesion sizes that exceed the vascular arcade. Scar formation in the macula and compromised vision are frequent findings in such cases. The occurrence of PCV in the inferior staphyloma or in angioid streaks shows heterogeneity in PCV. These findings suggest that PCV may be a finding on indocyanine green angiography rather than a distinct clinical entity. Spectral domain OCT examination shows that the branching vascular network of PCV is located between the retinal pigment epithelium and Bruch's membrane. In cases with retinal pigment epithelial detachment, CNV from the branching vascular network was found to extend along the roof of the detached retinal pigment epithelium. Such findings show that the branching vascular network of PCV is type 1 CNV. Complement factor H (CFH) and age-related maculopathy 2 (ARMS2)/High temperature requirement 1 (HTRA1) located on chromosome 10 (10q26) are well-established disease susceptible genes of AMD. In the Japanese, the prevalence of CFH Y402H gene polymorphism is low and ARMS2/HITRA1 plays a more important role in the development of AMD. In ARMS2 A69S polymorphism, a large deletion/insertion (443de1/54ins) that is reported in Caucasians was also found in Japanese. Thus, the genetic background of Caucasian and Japanese AMD is quite similar, as is also the case with exudative AMD and PCV. Our findings show that PCV is not a distinct clinical entity but is a subtype of exudative AMD. III. Exudative AMD with choroidal vascular hyperpermeability. Choroidal vascular hyperpermeability observed in central serous chorioretinopathy can be found in about 20% to 30% of exudative AMD cases in Japanese. Such cases often show a thick choroid, lack of drusen, and rather good visual prognosis with slow progression of the disease. Recently, \"pachychoroid neovasculopathy\" has been described by a group from New York. Such cases of AMD with choroidal vascular hyperpermeability, a thick choroid, and lack of drusen appears to belong to pachychoroid neovasculopathy. We studied the risk allele frequencies of CFH I62V and ARMS2 A69S gene polymorphisms in three groups : usual exudative AMD, pachychoroid neovasculopathy, and normal controls. Interestingly, cases of pachychoroid neovasculopathy show different gene polymorphisms of CFH I62V and ARMS2 A69S from the usual cases of exudative AMD and a more similar pattern to normal controls. Therefore, the possible mechanisms of the CNV development in such cases may differ from the classic well-documented drusen-dependent pathways. IV. Atrophic AMD in Japanese. Data from the Nagahama Cohort study show an increasing prevalence of drusen in Japanese. Recently, more extensive information on drusen has become available and the redefinition of drusen is currently in progress. In particular, the importance of reticular pseudodrusen (RPD) is more widely appreciated. This type of drusen is often found in Japanese AMD. Although the nature and location of RPD are still debatable, many investigators believe that this type of drusen is located under the sensory retina rather than under Bruch's membrane. In our analyses, RPD was found in 18.4% of late AMD cases in Japanese. It was more common in eyes with RAP or atrophic AMD and was seldom found in PCV. ARMS2 A69S gene polymorphism was found more frequently in cases of exudative AMD with RPD, than in cases of exudative AMD without RPD. Eyes with RPD show a thin choroid and diminished vascular densities of choroidal vessels."}, {"id": "pubmed23n0867_10837", "title": "[Humphrey Perimetry and Retinal Diseases].", "score": 0.010557691236280572, "content": "Since, in most eyes with retinal diseases quality of vision is greatly affected by visual field defects including paracentral scotoma and inferior field defects, visual function should be assessed by central 30- or 10-degrree automated static perimetry as well as visual acuity testing. The reduction of light sensitivity, demonstrated in the results of Humphrey central 10-2 perimetry, is more apparent than visual acuity loss in eyes with central serous chorioretinopathy (CSC), in which patients complain of dimness in the visual field of the affected eye. While reduced light sensitivity in eyes with acute CSC is well correlated with the height of subretinal fluid, marked and irreversible light sensitivity loss is demonstrated in the absence of subretinal fluid in eyes with chronic CSC due to structural damage in the photoreceptors. Various degrees of light sensitivity loss are seen in eyes wih age-related macular degeneration corresponding to intraretinal or subretinal pathology including intra- or subretinal fluid, fibrous scarring containing choroidal neovascularization and atrophic changes. The mean deviation (MD) of Humphrey central 10-2 perimetry is useful in predicting the visual outcome in eyes with exudative AMD after photodynamic therapy or intravitreal injection of anti-vascular endothelial growth factor. The progression of retinitis pigmentosa is well assssed with MD of Humphrey central 10-2 perimetry, which decreases linearly in the stage of residual visual field of 10 degrees or less. The age of patients with visual loss below 0.5 is delayed in eyes showing pencil-like configuration of \"Traquair's island of visual field\", in which a small area of normal light sensitivity around the fixation point is surrounded by absolute scotoma. With less visual acuity loss compared with that seen in eyes with central retinal artery occlusion; eyes with branch retinal artery occlusion show marked visual field defects, which are permanent and profound simulating the nasal-step pattern seen in eyes with glaucoma. Non-perfusion areas in fluorescein angiograms of eyes with branch retinal vein occlusion generally demonstrate reduced light sensitivity in the results of Humphrey central 30-2 perimetry, the degree of which tends to correlate with severity of non-perfusion. The light sensitivity in the area of detached retina in eyes with rhegmatogenous retinal detachment is generally reduced to show absolute scotoma, yet these eyes recover greatly after successful surgical repair."}, {"id": "InternalMed_Harrison_2407", "title": "InternalMed_Harrison", "score": 0.009909602592529421, "content": "• Next, examine the macula. The macula is the area between the superior and inferior temporal vascular arcades, and its center is the fovea. You can examine the macula by pointing your ophthalmoscope about 15° temporal to the optic disc. Alternatively, ask the patient to look into the center of the light. Note the foveal reflex and the presence of any hemorrhage, exudate, abnormal blood vessels, scars, deposits, or other abnormalities. • Examine the retinal blood vessels by re-identifying the optic disc and following each of the four main branches away from the disc. The veins are dark red and relatively large. The arteries are narrower and bright red. • Ask the patient to look in the eight cardinal directions to allow you to view the peripheral fundus. In a patient with a well-dilated pupil, it is possible to visualize as far as the equator."}, {"id": "pubmed23n1098_15161", "title": "A Case of Idiopathic Dense Vitreous Hemorrhage: Suspected Rupture of a Large Retinal Arterial Macroaneurysm on the Optic Disc.", "score": 0.009900990099009901, "content": "We report a novel case of vitreous hemorrhage associated with suspected rupture of 2-disc-diameter retinal arterial macroaneurysm on the optic disc. A 90-year-old woman presented with blurred vision (sudden onset) in her left eye. Examination of the fundus revealed acute onset vitreous hemorrhage of unknown origin without retinal detachment. She underwent vitrectomy, but after excision of the dense vitreous hemorrhage, a 2-disc-diameter hematoma appeared on the optic disc and was removed promptly. Because the bleeding at the base of the hematoma was of arterial origin and pulsating, the first vitrectomy could not achieve hemostasis. Five days after the first surgery, we performed a second vitrectomy. This revealed a subretinal hemorrhage along the superior and inferior arcade vessels and a macular hole, which was almost completely closed with an inverted internal limiting membrane flap. Unfortunately, the macular hole reopened 41 days after the second surgery. In patients presenting with only a large hematoma on the optic disc, it might be prudent to leave the hematoma. However, this large retinal arterial macroaneurysm was on a rare location on the optic disc, making it doubly difficult for the surgeons to diagnose and choose the best option intraoperatively. The differential diagnosis for dense vitreous hemorrhage of unknown origin should include a large retinal arterial macroaneurysm on the optic disc."}, {"id": "InternalMed_Harrison_2322", "title": "InternalMed_Harrison", "score": 0.009879597558964209, "content": "Optic Disc Drusen These are refractile deposits within the substance of the optic nerve head (Fig. 39-13). They are unrelated to drusen of the retina, which occur in age-related macular degeneration. Optic disc drusen are most common in people of northern European descent. Their diagnosis is obvious when they are visible as glittering particles on the surface of the optic disc. However, in many patients they are hidden beneath the surface, producing pseudopapilledema. It is important to recognize optic disc drusen to avoid an unnecessary evaluation for papilledema. Ultrasound or computed tomography (CT) scanning is sensitive for detection of buried optic disc drusen because they contain calcium. In most patients, optic disc drusen are an incidental, innocuous finding, but they can produce visual obscurations. On perimetry they give rise to enlarged blind spots and arcuate CHAPTER 39 Disorders of the Eye"}, {"id": "pubmed23n1133_15372", "title": "Long-term follow-up of a case of Coats disease in a 10-year-old boy with spontaneous peeling of preretinal macular fibrosis: a case report.", "score": 0.00980392156862745, "content": "Coats disease is a retinal vascular disorder characterized by aneurysms and telangiectasias. Macular fibrosis is a complication of Coats disease that results in vision loss. Macular fibrosis rarely develops in the natural course and often occurs after treatment with intravitreal bevacizumab, photocoagulation, or cryotherapy. Here, we have described an unusual case of spontaneous peeling of preretinal macular fibrosis in a patient with untreated Coats disease. A 10-year-old Japanese boy presented with vision loss in his left eye. The patient's left visual acuity was 20/28. Fundus examination of his left eye revealed thick preretinal macular fibrosis around the optic disc and macula. In addition, retinal telangiectasis, microaneurysms, hard exudates, and retinal hemorrhages were observed in the left peripheral temporal retina. We diagnosed his condition as Coats disease with preretinal macular fibrosis. Two months later, optical coherence tomography revealed preretinal macular fibrosis detachment at the foveal lesion without any treatment. During follow-up, preretinal macular fibrosis at the macular lesion was completely detached. Further, posterior vitreous detachment was observed and the shape of the macula and the patient's left visual acuity had improved. In our case, both formation and spontaneous peeling of preretinal macular fibrosis occurred without any treatment for Coats disease, which is an unusual finding. Vitreous changes might have occurred during the natural clinical course, causing subsequent posterior vitreous detachment and resulting in spontaneous peeling of fibrosis."}, {"id": "pubmed23n1011_12904", "title": "Acute Unilateral Anterior Ischemic Optic Neuropathy Secondary to Optic Nerve Head Drusen: Report of a Rare Coexistence.", "score": 0.00980392156862745, "content": "A 45-year-old white male noticed on awakening the painless loss of inferior vision in the left eye 2 days ago. He was otherwise well and his medical history was unremarkable. Visual acuity was 20/20 in OD and 20/32 in OS with a left inferior altitudinal defect and right blind spot enlargement demonstrable on visual field test. On fundus examination, both disc margins were blurred and the left disc was diffusely oedematous, with linear haemorrhages in the adjacent nerve fibre layer. Radiologic imaging and laboratory tests were unremarkable. Bilateral optic nerve head drusen (ONHD) was demonstrated by optical coherence tomography and fundus autofluorescence imaging. Unilateral acute non-arteritic anterior ischemic optic neuropathy (NAION) and concomitant bilateral ONHD were diagnosed. NAION may develop secondary to ONHD. Therefore, clinicians should be aware of this rare association and inform the patients about this risk. Patients with ONHD should be followed-up periodically in terms of possible ischemic complications."}, {"id": "pubmed23n0892_12025", "title": "Clinical Features and Course of Patients with Peripheral Exudative Hemorrhagic Chorioretinopathy.", "score": 0.009708737864077669, "content": "To evaluate the clinical characteristics of patients who were followed in our clinic with the diagnosis of peripheral exudative hemorrhagic chorioretinopathy (PEHC). Medical records of 12 patients who were diagnosed with PEHC in İstanbul University İstanbul Faculty of Medicine, Department of Ophthalmology between July 2006 and June 2014 were reviewed retrospectively. This study included 21 eyes of 12 patients. Four (33.3%) of the patients were male and 8 (66.7%) were female and ages ranged between 73 and 89 years. Eight (66.7%) of the patients were referred to us with the diagnosis of choroidal mass. Unilateral involvement was found in 3 and bilateral involvement in 9 patients. Temporal quadrants were involved in all eyes. Fifteen eyes (71.4%) had subretinal hemorrhage and hemorrhagic/serous retinal pigment epithelial detachment, 11 (52.4%) had lipid exudation, 5 (23.8%) had chronic retinal pigment epithelium alterations, 2 (9.5%) had subretinal fibrosis and 1 (4.8%) had vitreous hemorrhage. PEHC lesions were accompanied by drusen in 11 eyes (52.4%), geographic atrophy in 2 eyes (9.5%), and choroidal neovascularization scar in 2 eyes (9.5%). Treatment was done in both eyes of a patient for lesions which threatened the macula, in a patient with bilateral macular edema and in a patient with vitreous hemorrhage. The remaining eyes were followed-up without any treatment because the lesions did not threaten the macula and they showed no progression during follow-up. PEHC is a degenerative disease of peripheral retina that is seen in older patients, and signs of age-related macular degeneration (AMD) may accompany this pathology. Especially in patients with AMD findings, the peripheral retina must be evaluated carefully for existing PEHC lesions."}, {"id": "wiki20220301en067_41614", "title": "Metamorphopsia", "score": 0.009615384615384616, "content": "Pathology The mechanisms that result in the development of metamorphopsia involve structural changes in the retina of the eye (retinal mechanism) as well as processing changes in the cerebral cortex of the brain (cortical mechanism). The retinal mechanism involves the displacement of retinal layers which results in the mislocation of light on the retina. The cortical mechanism, which was discovered after the retinal mechanism, is affected by perceptual “filling-in” and visual crowding effects. The cortical mechanism was found to work in combination with the retinal mechanism to contribute to metamorphopsia in long-standing maculopathy or after the treatment of macular disorders. Causes of Metamorphopsia Metamorphopsia can be a symptom of a number of eye disorders involving the retina or macula. Some of these conditions include the following: Age-related macular degeneration Epiretinal membrane and vitreomacular traction Posterior vitreous detachment Macular hole"}, {"id": "pubmed23n0601_21762", "title": "Peripheral field loss: something old, something new.", "score": 0.009615384615384616, "content": "A 37-year-old patient presented with acute visual field loss diagnosed as non-arteritic anterior ischemic optic neuropathy in the setting of optic nerve drusen. Her visual field loss had progressed when compared to the visual field done 2 years previously. Fundus examination showed bilateral optic nerve head drusen and left retinal nerve fiber layer edema consistent with non-arteritic anterior ischemic optic neuropathy."}, {"id": "pubmed23n1028_22933", "title": "Severe vision loss secondary to retinal arteriolar occlusions after multiple intravitreal brolucizumab administrations.", "score": 0.009523809523809525, "content": "To describe a case of unilateral retinal arteriolar occlusion following multiple intravitreal brolucizumab injections for neovascular age-related macular degeneration (nAMD). A 92-year-old Caucasian woman presented with blurry vision in her left eye (OS) after receiving the third dose of intravitreal brolucizumab. At the time of presentation, visual acuity (VA) was 20/40 in her right eye (OD) and had decreased from 20/150 to count finger (CF) at 1-foot OS. On examination, there was no evidence of active inflammation in the anterior chamber OU. Dilated fundus examination showed no vitritis in OD and 1+ vitreous cells OS, flame-shaped hemorrhage at the superior optic disc margin, and retinal whitening surrounding the proximal portion of the supero-temporal branch of the central retinal artery. There were drusen in OS and retinal pigment epithelial (RPE) changes in the maculae of OU. Intra-arteriolar greyish deposits were seen OS. Fluorescein angiography (FA) showed hyper-fluorescence in the maculae corresponding to fibrovascular pigment epithelial detachments (PED) OU. No peri-vascular leakage was noted OU. Delayed filling of multiple arterioles in early and late phases OS was observed on FA. The patient was diagnosed with retinal arteriolar occlusion associated with repeated intravitreal brolucizumab administrations. Retinal arteriolar occlusion with severe vision loss, possibly secondary to inflammatory responses, can occur after subsequent intravitreal brolucizumab injections, even if no inflammation occurred after initial administrations. Vaso-occlusive disease should be considered as a potential ocular complication, with acute as well as delayed onset, following intravitreal brolucizumab therapy."}, {"id": "pubmed23n0966_18478", "title": "Acute visual loss and optic disc edema followed by optic atrophy in two cases with deeply buried optic disc drusen: a mimicker of atypical optic neuritis.", "score": 0.009523809523809525, "content": "Sudden visual loss and optic disc edema caused by optic neuritis (ON) is usually followed by significant visual recovery. However, little or no recovery occurs when the loss is caused by atypical ON, especially in patients with neuromyelitis optica (NMO). Optic disc drusen (ODD) is a cause of pseudo optic disc edema and may be a predisposing factor for non-arteritic anterior ischemic optic neuropathy (NAION), thereby mimicking atypical ON. In such cases, if globular concretions are seen protruding from the disc substance, ODD may be suspected. The purpose of this paper is to describe two patients with acute visual loss followed by optic disc atrophy initially labeled as atypical ON. Though not suspected on clinical examination, optical coherence tomography (OCT) revealed deeply buried ODD as a predisposing factor for NAION. Case 1: A 48-year-old woman had bilateral sequential visual loss associated with optic disc edema. Despite treatment, vision did not improve and severe disc pallor ensued. Atypical ON was suspected. Eventually, she was started on immunosuppressant therapy based on a tentative diagnosis of NMO-spectrum disorder. On examination 5 years later, only severe optic disc pallor was observed, but OCT radial B-scans showed ovoid hyporeflective areas in the retrolaminar region of both eyes, compatible with ODD; this led to a diagnosis of NAION and deeply buried ODD. Case 2. A 35-year-old woman with suspicion of ON in the left eye and a history of previous atypical ON in the right eye was referred for neuro-ophthalmic examination which revealed diffuse optic disc pallor and a dense arcuate visual field defect in the right eye. OCT B-scans passing through the disc showed large ovoid areas of reduced reflectivity in the retrolaminar region of the optic disc in the right eye. These findings helped confirm the diagnosis of NAION in one eye, with deeply buried ODD as predisposing factor. Deeply buried ODD may be associated with NAION causing irreversible visual loss and optic disc pallor, a condition easily mistaken for atypical ON. Awareness of such occurrence is important to avoid unnecessary testing and minimize the risk of mismanagement."}, {"id": "pubmed23n0407_7043", "title": "[Characteristics of optical coherence tomography for exudative age-related macular degeneration].", "score": 0.009345794392523364, "content": "To observe the characteristics of optical coherence tomography (OCT) for exudative age-related macular degeneration (AMD). Thirty-eight patients (42 eyes) diagnosed as exudative AMD by color fundus photography, fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) were examined by OCT. The results of OCT were analyzed and compared with that of FFA and ICGA. The characteristics of OCT for exudative AMD: fibrotic choroidal neovascularization (34 eyes), serous detachment of neurosensory epithelium (38 eyes), detachment of the nerve fiber layer (8 eyes), and hemorrhagic and serous detachment of retinal pigment epithelium (11 eyes and 9 eyes, respectively). The major characteristic of OCT of exudative AMD is fibrotic choroidal neovascularization combined with serous and hemorrhagic detachment of neurosensory epithelium or retinal pigment epithelium, which would be helpful for diagnosing and evaluating exudative AMD."}, {"id": "pubmed23n0739_5604", "title": "Visual prognosis of eyes with submacular hemorrhage associated with exudative age-related macular degeneration.", "score": 0.009345794392523364, "content": "To study the retinal structural changes associated with submacular hemorrhage due to exudative age-related macular degeneration (AMD) and their relationships with visual prognosis. We retrospectively reviewed the medical records of 31 consecutive patients (31 eyes) with visual impairment due to an acute submacular hemorrhage associated with typical AMD (10 eyes) or polypoidal choroidal vasculopathy (21 eyes). Optical coherence tomography (OCT) revealed that submacular hemorrhage exhibited intense hyperreflectivity beneath the neurosensory retina and often seemed to infiltrate it. In the OCT sections, mild to moderate amorphous hyperreflectivity and/or hyperreflective dots were observed within the neurosensory retina, resulting in the loss of the junctions between the inner (IS) and outer (OS) segments of the photoreceptors. Of the 31 eyes, the foveal IS/OS line could be seen incompletely in 12 eyes and was totally absent in 16 eyes. The initial integrity of the foveal photoreceptor layer was correlated with the final visual acuity; the initial detection of the IS/OS just beneath the fovea was correlated with good final visual acuity (r = 0.375, p = 0.038). As a hallmark of integrity of the foveal photoreceptor layer, the initial detection of the IS/OS just beneath the fovea may predict good visual outcomes."}, {"id": "pubmed23n0580_7032", "title": "Posterior vitreomacular adhesion: a potential risk factor for exudative age-related macular degeneration?", "score": 0.009259259259259259, "content": "To compare the state of the posterior vitreous in exudative age-related macular degeneration (AMD) with eyes with nonexudative AMD and controls. Prospective, observational case series. B-scan ultrasonography and optical coherence tomography (OCT) were performed in 163 eyes from 82 subjects older than 55 years, 50 eyes with exudative AMD, 57 with nonexudative AMD, and 56 control eyes. Main outcome measures were the number of eyes with complete posterior vitreous detachment (PVD) by ultrasound and the number of eyes with central vitreomacular adhesion by OCT. By ultrasonography, 17 (34.0%) of 50 eyes with exudative AMD had PVD as compared with 41 (71.9%) of 57 eyes with nonexudative AMD (P = .00002) and 34 (60.7%) of 56 controls (P = .017). OCT detected persistent central vitreoretinal adhesion surrounded by a detached posterior vitreous cortex in 18 (36%) of 50 eyes with exudative AMD, significantly higher than in nonexudative AMD (4/57 [7%]; P &lt; .0001) and in controls (6/56 [10%]; P = .002). Persistent attachment of the posterior vitreous cortex to the macula may be another risk factor for the development of exudative AMD via vitreoretinal traction inducing chronic low-grade inflammation, by maintaining macular exposure to cytokines or free radicals in the vitreous gel, or by interfering in transvitreous oxygenation and nutrition of the macula. Inducing PVD may provide prophylactic benefit against exudative AMD."}, {"id": "pubmed23n0532_15940", "title": "[Optic disc drusen and acute vision loss].", "score": 0.009259259259259259, "content": "Optic disc drusen (ODD) are calcified nodules within the optic nerve head. These are products of degenerated retinal ganglion cells axoplasmic transport, also known as hyaline or colloid bodies. They are mainly encountered as an incidental benign finding, or as a benign cause of swollen discs (in the differential diagnosis of papilledema). The majority of the patients are asymptomatic, and a slowly progressive sub-clinical visual field loss is found in about 80% of the patients. However, acute painless symptomatic visual field loss, occasionally massive, is rare. This is a case history of 4 patients who developed acute painless visual field loss which was attributed to optic disc drusen. The clinical features and proposed ischemic pathophysiology of acute vision loss and ODD are discussed."}, {"id": "pubmed23n0816_15660", "title": "[Clinical and pathological observation of seven cases of spontaneous sub-retinal hemorrhage].", "score": 0.009174311926605505, "content": "To improve pathological understanding of massive sub-retinal hemorrhage. Retrospective case series study. The clinical and pathological data of 7 cases of massive sub-retinal hemorrhage which were examined in the Pathological Department of Tianjin Eye Hospital from May 1988 to April 2012 were collected. The serial section of eyeball specimens were made with HE and PAS staining. The pathological section were reviewed under the light microscope. Analysis were made again combining with patients' clinical history, imaging findings and the pathological features. In 7 patients, 6 patients were male, 1 patient was female. The age range was from 60 to 82 years old and the average age was 71.7 years old. Four cases were on the right eye and 3 cases were on the left eye. The main clinical feature was sudden loss of vision and 2 cases had acute glaucoma symptoms. Ultrasound examination showed choroidal tumor in 6 cases and retinal detachment with vitreous hemorrhage in one case. The color Doppler ultrasound examination demonstrated choroidal substantive occupying lesion in 3 cases and two of them were detected with blood flow signal. The MRI were examined in 3 cases which showed iso-high signal in T1W1 and iso-low signal in T2W1 suggesting the choroidal melanoma in 2 cases and sub-retinal hemorrhage in 1 case. Six cases were diagnosed clinically as choroidal tumor or melanoma and 1 case was diagnosed as acute angle-closure glaucoma. The enucleation were performed in 7 cases. In these cases, 6 cases were diagnosed pathologically as vitreous hemorrhage, hemorrhagic RPE detachment and massive subretinal hemorrhage. The related choroidal lesions included soft drusen of Bruch membrane in 3 cases, choriocapillaris wall degeneration in 2 cases, fibrovascular membrane formation under RPE in 2 cases, choroidal chronic non-granulomatous inflammation in 4 cases, choroidal vessels wall thickening and sclerosis in 2 cases and choroidal vessels anomaly in one case. The secondary angle-closure glaucoma or angle-open glaucoma were accompanied respectively in 2 cases. Another case of 7 cases was diagnosed pathologically as the rupture of retinal macroaneurysm with massive sub-retinal hemorrhage. Massive sub-retinal hemorrhage liked to occur in the elderly male patients. The main manifestations were sudden visual loss. A small number of patients had symptoms of acute glaucoma. Ophthalmic ultrasound and imaging characteristics were usually similar to the choroidal substantive neoplasm and were easily misdiagnosed as choroidal melanoma. Pathological examination revealed that the main reasons of massive hemorrhage were from the hemorrhagic RPE detachment and the rupture of retinal macroaneurysm."}, {"id": "pubmed23n0682_8384", "title": "Central serous papillopathy by optic nerve head drusen.", "score": 0.009174311926605505, "content": "We report a 38-year-old man with a complaint of blurred vision in his right eye for the previous 5 days. He had bilateral optic disc drusen. Fluorescein angiography revealed multiple hyperfluorescent foci within temporal optic discs and temporal inferior arcade in late phase. Optical coherence tomography showed bilateral peripapillary serous detachment as well as right macular detachment. This is the first reported case of a concurrent peripapillary and macular detachment in a patient with central serous papillopathy by optic disc drusen. Central serous papillopathy is an atypical form of central serous chorioretinopathy that should be considered as a potential cause of acute loss of vision in patients with optic nerve head drusen."}, {"id": "pubmed23n0057_11201", "title": "Macular lesions predisposing to senile disciform macular degeneration.", "score": 0.00909090909090909, "content": "Senile disciform macular degeneration (SMD) is a neovascular/exudative form of age-related macular degeneration (AMD). In this study 340 eyes were followed up to assess the progression of SMD. The 340 eyes consisted of two groups. Group 1 was composed of 157 eyes with age-related macular changes other than choroidal neovascular membrane. Group 2 was made up of the contralateral eyes of 183 unilateral SMD eyes. Average ages were 61 and 64 in groups 1 and 2, respectively, and respective follow-up periods were 44 and 52 months. Choroidal neovascular membrane developed in 12 eyes in group 1 (7.6%) and in 19 eyes in group 2 (10.4%), a total of 31 eyes (9.1%). Retinal pigment epithelium (RPE) detachment was found as a predisposing lesion in 25 of these 31 eyes. Choroidal neovascular membrane developed in 12 of the 24 eyes with large RPE detachments. In 3 eyes neovascular membrane developed from an RPE detachment which had evolved from soft drusen. There were 3 eyes among the 62 eyes with soft drusen in which neovascular membrane developed directly from soft drusen. Based on these results, we classified AMD into 3 types; 1) atrophic, 2) predisciform, which includes RPE detachment and soft drusen, and 3) SMD."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": true, "char_ranges": [[94, 154]], "word_ranges": [[16, 26]], "text": "Lefort type I fracture does not affect the orbital contents."}, "2": {"exist": true, "char_ranges": [[0, 93]], "word_ranges": [[0, 16]], "text": "Diplopia on vertical gaze is highly suggestive that the bony walls of the orbit are affected."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Diplopia on vertical gaze is highly suggestive that the bony walls of the orbit are affected. Lefort type I fracture does not affect the orbital contents. Knowing this information, the only correct answer is 2. If we were in doubt about whether or not Lefort's type I fracture affects the orbit, just look at the answers. Whoever posted the question wanted to make sure that the only valid answer was number 2; that is why they added the tag line \"but just in case we check it with a CT scan\".", "full_answer_no_ref": "Diplopia on vertical gaze is highly suggestive that the bony walls of the orbit are affected. Lefort type I fracture does not affect the orbital contents. Knowing this information, [HIDDEN]. If we were in doubt about whether or not Lefort's type I fracture affects the orbit, just look at the answers. Whoever posted the question wanted to make sure that [HIDDEN]; that is why they added the tag line \"but just in case we check it with a CT scan\".", "full_question": "Given a direct trauma to the right side of the face after which the patient presents unilateral palpebral hematoma, diplopia in the vertical gaze and difficulty in opening the mouth, which of the following statements is true?", "id": 137, "lang": "en", "options": {"1": "We are with great probability in front of a Lefort I type fracture of the middle third of the face.", "2": "This is probably a unilateral orbitomalar fracture. The diagnosis would ideally be verified by CT (computed axial tomography).", "3": "It is a fracture of the base of the skull at the level of the carotid foramen.", "4": "The probable diagnosis is fracture with dislocation of the mandibular condyle.", "5": "A mandibular fracture is probably associated with a Lefort I type midface fracture."}, "question_id_specific": 164, "type": "OTORHINOLARYNGOLOGY AND MAXILLOFACIAL SURGERY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en240_439", "title": "Mandibular fracture", "score": 0.017323775388291517, "content": "that becomes progressively worse to the unaffected side). When the mouth is opened, there may be deviation of the mandible towards the fractured side. Bilateral condylar fractures may cause the above signs and symptoms, but on both sides. Malocclusion and restricted jaw movement are usually more severe. Bilateral body or parasymphysis fractures are sometimes termed \"flail mandible\", and can cause involuntary posterior movement of the tongue with subsequent obstruction of the upper airway. Displacement of the condyle through the roof of the glenoid fossa and into the middle cranial fossa is rare. Other rare complications of mandibular trauma include internal carotid artery injury, and obliteration of the ear canal due to posterior condylar dislocation. Bilateral condylar fractures combined with a symphyseal fracture is sometimes termed a guardsman's fracture. The name comes from this injury occurring in soldiers who faint on parade grounds and strike the floor with their chin."}, {"id": "pubmed23n0770_23956", "title": "Which craniofacial fractures are associated with external auditory canal bleeding?", "score": 0.016865079365079364, "content": "External auditory canal bleeding (EACB) after facial trauma has been strongly associated with skull base fractures; however, EACB also can occur with other craniofacial fractures. The aim of this study was to analyze the frequency and causes of EACB in different craniofacial fracture types. The investigators used a retrospective cohort study design and enrolled a sample composed of patients with craniofacial fractures evaluated and treated from April 2006 through December 2011. The predictor variable was fracture type, which was categorized into 4 types: skull base fracture, midface fracture, and mandibular fracture with and without involvement of the condyle. The frequency of EACB among fracture types was compared with SPSS 13.0 (SPSS, Inc, Chicago, IL) and χ(2) test. Computed tomographic (CT) scans were analyzed to determine the cause of EACB. EACB was found in 43 of 573 craniofacial fracture cases, with a frequency of 7.5%. There were 19 EACB sides in 123 skull base fracture cases (15.4%), 26 EACB sides in 150 mandibular fracture cases involving the 196 condyles (13.3%; of these 196 condyles, 92.3% were intracapsular condylar fractures [ICFs]), 2 EACB sides in 150 mandibular fracture cases not involving the condyle (1.3%), and 1 EACB case in 150 midface fracture cases (0.7%). Statistical analysis of EACB frequency for each fracture type showed a significant difference between skull base or mandibular fractures with condylar involvement and midface or mandibular fractures without condylar involvement (P &lt; .05). However, there was no significant difference between skull base and mandibular fractures involving the condyle and midface fracture and mandibular fractures not involving the condyle (P &gt; .05). EACB is uncommon in craniofacial fractures. The frequency varies significantly based on fracture type. Skull base fracture and mandibular ICF are the 2 main causes of EACB."}, {"id": "pubmed23n0411_9046", "title": "[Clinical study of three-dimensional reconstruction of computed tomography in diagnosis of condylar fractures].", "score": 0.01675320179993077, "content": "To explore a method of getting good three-dimensional (3D) reconstruction images of temporomandibular joint (TMJ) by computed tomography (CT), and evaluate diagnosis value of 3DCT in condylar fractures. Of the 14 patients studied, 12 were male and 2 were female with age ranging from 4 to 37 years old. 4 were old fractures and the other 10 were fresh fractures. All patients' orthopanotomograms were taken first, and then were scanned with CT. The scanning conditions were 300-345 mAS and 120kV, and the scanning methods included cross-sectional (11/14), coronal-sectional (2/14) and spiral (1/14) (pitch: 1, thickness of layers: 2.5 mm) scanning. Scanning scopes: The cross-sectional and spiral scanning were from 1 cm above the Frankfort horizontal plane to the middle of the mandibular ramus or Menton; The coronal-sectional scanning was from the mastoid process to the anterior point of the mandibular ramus or Pogonion. The thickness of the scanning layers was 2.5 mm (12/14) or 5 mm (2/14). Finally, 3D reconstructed images were obtained by shaded surface display (SSD). The cross-sectional images were obtained by being removed the cervical vertebra, the styloid process, the occipital bone and part of the mastoid process with Subtract Manual Irroi before reconstruction to avoid interference with the observation of TMJ and skull basis. 1. The best 3D CT reconstructed images were obtained by 2.5 mm-thin-layer cross-sectional scanning by being removed parts of the adjacent structures, and the bone lines were clear and smooth without adjacent bone structures shading TMJ. The quality of images taken spirally was similar to those taken cross-sectionally. While the coronal scanning neglected some important anatomic symbols which might be valuable to diagnose condylar displacement. 2. Of the 14 patients, 7 were unilateral condylar fractures and 7 were bilateral (21 sides altogether), among which 18 sides were high fractures of condyles and 3 were fractures of condylar neck. High oblique line fractures and comminuted fractures had major condylar rotation displacement which moved forward, downward and inside, whereas, vertical fractures only had minor displacement. Inward rotation displacement occurred in medial bent fractures. 10 of 14 patients (15 sides) had been conducted operation, and the same situations as seen from 3DCT reconstructed images were found. 3DCT images can display condylar fractures accurately and directly, and are very useful for surgeons to select treating methods of condylar fractures."}, {"id": "wiki20220301en240_438", "title": "Mandibular fracture", "score": 0.015998551074619657, "content": "This type of fractured mandible can involve one condyle (unilateral) or both (bilateral). Unilateral condylar fracture may cause restricted and painful jaw movement. There may be swelling over the temporomandibular joint region and bleeding from the ear because of lacerations to the external auditory meatus. The hematoma may spread downwards and backwards behind the ear, which may be confused with Battle's sign (a sign of a base of skull fracture), although this is an uncommon finding so if present, intra-cranial injury must be ruled out. If the bones fracture and overlie each other there may be shortening of the height of the ramus. This results in gagging of the teeth on the fractured side (the teeth meet too soon on the fractured side, and not on the non fractured side, i.e. \"open bite\" that becomes progressively worse to the unaffected side). When the mouth is opened, there may be deviation of the mandible towards the fractured side. Bilateral condylar fractures may cause the"}, {"id": "pubmed23n0628_20360", "title": "Mandibular condylar fractures and acute atlanto-axial subluxation. Part 1 - A new clinical and nosographic evidence.", "score": 0.015085081950099175, "content": "The aim of this study was to analyze the acute repercussions of condylar mandibular fractures on occipital-atlanto-axial joint. Twenty-five non-consecutive cases (16 males and 9 females, mean age: 22.96/range 14-36 years), observed and treated in the Maxillofacial Surgery Department of the University of L'Aquila have been considered. Types of fractures examined included: unilateral: 19 cases (solitary: 12; associated with other mandibular fractures: 7; homolateral: 2); bilateral: 6 cases (equivalent: 2; not equivalent: 4). A control group was constituted of 10 patients, 5 males and 5 females, aged from 19 to 24 years (mean-range: 21.6) suffering from acute isolated cervical distorsion (whiplash). The study has been performed by means of the analysis of X-ray and computed tomography (CT)-CT/3D of the mandibular condylar regions, the occipital-atlanto-axial structures and the cervical region. In all the cases of fractures of the mandibular condyle an acute alteration of the junctional atlanto-axial structures was present. In case of unilateral solitary condylar fractures the authors have observed an atlas rotation, homolateral to the side of the condylar fracture, independent from the level of the fracture (intra- or extracapsular). The rotation seems to be proportional to the entity of the condylar fragments dislocation on the horizontal plane and it causes a modification of the articular relations between atlas and axis (atlanto-axial subluxation) and between the atlas and the occipital bone. The authors have observed a constant derangement of the cranio-axial joint on the three planes of the space. In particular, on the vertical plane the CT reconstructions show on the right and left side a different height between the atlanto-axial articular spaces. The largest one is homolateral to the side of the condylar fracture. In case of unilateral condylar fractures associated with other mandibular fracture (homolateral or not) the authors have observed the same alterations of the occipital-atlo-epistropheal joint, but while on the horizontal plane the rotation of the atlas is always homolateral to the condylar fracture, on the vertical plane the largest atlanto-axial articular space is homolateral to the mandibular fracture with more dislocation of the fragments of fracture (usually the associated not homolateral mandibular fracture). In case of bilateral condylar fractures the authors have observed no alteration of the cranio-cervical joint. In the non-equivalent fractures, they have observed the atlas rotation on the horizontal plane and the junctional derangement on the vertical plane, homolateral to the condylar fracture with greatest dislocation. In the control group the loss of the physiological cervical lordosis has been observed. Alterations on the horizontal and vertical planes, as the rotation of the atlas, atlanto-axial subluxation or the joint derangement, instead, has never revealed. The authors state that these results represent a new nosographic entity associated with the condylar mandibular fractures with important clinical, insurance and legal repercussions."}, {"id": "pubmed23n0794_2527", "title": "Etiology, incidence and patterns of mid-face fractures and associated ocular injuries.", "score": 0.014810275266840305, "content": "A prospective study on mid-face fractures was carried out in the Department of Oral and Maxillofacial Surgery at College of Dentistry, Indore, from August 2007 to September 2009 to analyze etiology, incidence and patterns of midface fractures and associated ocular injuries. Two hundred patients were included in this study, amongst those who reported to the Department of OMFS, College of Dentistry, Indore. After confirmed diagnosis of mid face fracture all the patients were stratified according to age, sex, cause of the accident, influence of alcohol, location, type of fractures and associated ocular injuries. The study included 200 patients with a mean age of 29.6 years. The most frequently injured patients belonged to the 21-30 year-old age group. The male predilection was 76 %. Road traffic accident was the most common causative factor (64 %), followed by assault (21 %), cases of fall (9.5 %) and other causes (5.5 %). The most common fracture in this study was found to be zygomatic complex fractures (62.5 %) (more in the age group of 21-30 years). This was followed by Lefort II fractures (23 %), multiple fractures (10 %) and Lefort I fractures (6 %), Lefort III fractures (4.5 %) and Naso-ethmoidal fractures (4 %) in descending order. 84.5 % subjects were having ocular involvement. Subconjunctival hemorrhage was present mostly in 83.5 % followed by remaining as corneal injury 15 %, reduced acuity 11.5 %, diplopia 10.5 %, enophthalmos 8.5 %, telecanthus 5 %, hyphema 3.5 %, blindness 3 % and proptosis 0.5 %. Zygomatic complex fractures were the most frequent type of injury that was complicated by blindness or a serious eye injury (61 %). Collection of data regarding the epidemiology of maxillofacial fractures is important because it may assist healthcare providers to provide necessary information for the development and evaluation of preventive measures. Ocular injuries should have an early ophthalmological examination at the time of trauma to detect any kind of ocular dysfunction. "}, {"id": "pubmed23n0083_4231", "title": "[Clinical study of mandibular condyle injury].", "score": 0.014784506273867976, "content": "Mandibular condyle fractures develop frequently and show the variable type of injury and complication. New opinions have emerged from recent investigation into condylar fractures. The author investigated 246 patients with condylar fractures who visited SNUDH from January 1980 to August, 1988, 8. with regard to clinical and treatment aspects, area and displacement of fractures, associated teeth injury and other body injury, complications. At last I have got the following results. 1. The incidence to condylar fractures in a series of 765 mandibular fractures may be as high as 32.2%. 2. The male patients are 3 times more than female patients. The highest frequency was recorded in the group 21-30 years of age. (34.1%). 3. Falls caused the greatest number of condylar fractures (45.2%) and next was in assult (25.6%), traffic accidents (22.4%). 4. Unilateral condylar fractures were present in 74.8%, giving a left: right ratio of 1.2:1. In cases of unilateral fracture, subcondylar fractures were by far the commonest (32.9%) but in cases of bilateral fracture, condylar neck fractures were by far the commonest. In children under 15 years of age, condylar neck fractures were more common but in patients over 16 years of age, subcondylar fractures were common. 5. Anteromedial fracture dislocations were by far the commonest (20.3%). In children under 15 years of age, fracture deviations were common but in patients over 16 years of age, fracture displacements were common. 6. 44.7% of patients with condylar fractures sustained the teeth injuries. Teeth fractures were by far the commonest. 7. Single condylar fractures showed a frequency of 30.5%. Of the concomitant fractures elsewhere in the mandible, symphysis fractures were by far the commonest (54.1%). 8. Associated other body injuries showed a frequency of 28.0%. Of them, head injuries were by far the commonest. 9. The mean interval from injury to treatment was 14.3 days. Of the treatment of condylar fractures, open reduction was by far the commonest (70.3%). Closed reduction comprised 19.9% and functional therapy comprised 8.5%. 10. In 67 patients with possible follow up period, the following complications were developed, two ankylosis, anterior open bite, mouth opening limitation, mouth opening deviation."}, {"id": "wiki20220301en240_443", "title": "Mandibular fracture", "score": 0.01446975354742345, "content": "Research has shown that panoramic radiography is similar to computed tomography in its diagnostic accuracy for mandible fractures and both are more accurate than plain film radiograph. The indications to use CT for mandible fracture vary by region, but it does not seem to add to diagnosis or treatment planning except for comminuted or avulsive type fractures, although, there is better clinician agreement on the location and absence of fractures with CT compared to panoramic radiography. Classification There are various classification systems of mandibular fractures in use. Location This is the most useful classification, because both the signs and symptoms, and also the treatment are dependent upon the location of the fracture. The mandible is usually divided into the following zones for the purpose of describing the location of a fracture (see diagram): condylar, coronoid process, ramus, angle of mandible, body (molar and premolar areas), parasymphysis and symphysis."}, {"id": "pubmed23n0365_4821", "title": "Fractures of the mandibular condyle. Part 1: patterns of distribution of types and causes of fractures in 348 patients.", "score": 0.014181594661046716, "content": "This prospective study was designed to record relevant characteristics of mandibular condyle fractures and to evaluate the relationship between these. Data were recorded on sex, age, cause of trauma, level of fracture, dislocation of the mandibular head, dental state and associated fractures of all patients diagnosed in our hospital during the period 1984-1996 with mandibular condyle fractures. Data were analysed in our Computer Department. The sample comprised 348 patients with 444 fractures, and a male:female ratio of 2:1. Traffic accidents were the most common cause: 103 (41%) of the unilateral and 54 (56%) of the bilateral fractures, followed by alleged assault and falls. Low fractures were the most common -n = 314 of 444 (71%). The causes that involved considerable force (traffic accidents and falls) resulted in more dislocations of the mandibular head, more bilateral fractures, a tendency to fractures higher on the condyle and significantly more intracapsular fractures. Absence of molar occlusion also gave more high and fewer low fractures, but played no part in dislocation of the mandibular head from the glenoid fossa."}, {"id": "pubmed23n1088_10444", "title": "Diagnostic accuracy of physical examination findings for midfacial and mandibular fractures.", "score": 0.013917159763313609, "content": "To assess the diagnostic accuracy of physical examination findings used to identify patients at risk for midfacial or mandibular fractures. A five-year retrospective cohort was constructed from all emergency department patients with a midfacial or mandibular trauma. The sensitivity, specificity, pre-test probability, positive predictive value, negative predictive value, positive likelihood ratio and negative likelihood ratio data was calculated for 19 and 14 physical examination findings for midfacial and mandibular fractures respectively. Computed Tomography and panoramic radiography were used as index tests. A total of 1484 patients were identified among whom 40.4% midfacial and 33.4% mandibular fractures were diagnosed. Overall, specificity was found to be higher than sensitivity. Regarding midfacial fractures, high specificity was found for raccoon eyes, malar eminence flattening and all the findings that are related to palpation, the nasal, ocular and intra-oral assessment. Malar eminence flattening, external nasal deformity, nasal septum hematoma, change of globe position and palpable step-off had ad high positive predictive value and positive likelihood ratio. Regarding mandibular fractures high specificity was found for mouth opening restriction, auditory canal bleeding, intra-oral assessment related findings, palpable step-off, inferior alveolar nerve paresthesia, the angular compression test and chin axial pressure test. The diagnostic accuracy of relevant physical examination findings were identified for the prediction of midfacial and mandibular fractures."}, {"id": "wiki20220301en240_433", "title": "Mandibular fracture", "score": 0.01367318711717052, "content": "Mandibular fracture, also known as fracture of the jaw, is a break through the mandibular bone. In about 60% of cases the break occurs in two places. It may result in a decreased ability to fully open the mouth. Often the teeth will not feel properly aligned or there may be bleeding of the gums. Mandibular fractures occur most commonly among males in their 30s. Mandibular fractures are typically the result of trauma. This can include a fall onto the chin or a hit from the side. Rarely they may be due to osteonecrosis or tumors in the bone. The most common area of fracture is at the condyle (36%), body (21%), angle (20%) and symphysis (14%). While a diagnosis can occasionally be made with plain X-ray, modern CT scans are more accurate."}, {"id": "pubmed23n0403_3548", "title": "A comprehensive classification of craniofacial fractures: postmortem and clinical studies with two- and three-dimensional computed tomography.", "score": 0.013522138013562026, "content": "A comprehensive classification of midfacial/craniofacial fractures, based on two- and three-dimensional computed tomography (2D and 3D-CT) is presented. We performed a postmortem analysis of 24 patients who had died from trauma with signs of craniofacial fractures, based on 2D and 3D-CT studies with pathoanatomical findings. In addition, CT findings for 100 patients with craniofacial injuries requiring an emergency CT were correlated with surgical findings and follow-up results. On the basis of the analysis of a total of 377 fractures a classification system is proposed. The system is based on the use of the AO/ASIF (Arbeitsgemeinschaft für Osteosynthesefragen/Association for the Study of Internal Fixation) scheme, defining three types (A, B, C), three groups within each type (e.g. A1, A2, A3) and three subgroups within each group (e.g. A1.1, A1.2, A1.3) with increasing severity from A1.1 (lowest) to C3.3 (highest). The craniofacial region is divided into three units: the lower midface (I), the upper midface (II) and the craniobasal-facial unit (III). Lateral and central fractures are also distinguished. Type A fractures are non-displaced fractures, type B are displaced fractures and type C are complex/defect fractures. Groups A1, B1 and C1 comprise fractures of an isolated unit; groups A2, B2 and C2, combined fractures without involvement of the skull base; and groups A3, B3 and C3 are those combined fractures with involvement of the skull base. A correlation between the severity of the fracture and (i). the number of posttraumatic functional limitations (Spearman rank test, correlation coefficient r=0.42), (ii). the need for bone grafting or dural plastic (r=0.39) and (iii). facial asymmetry (r=0.37), was observed. The proposed classification system allows standardised documentation of midfacial and craniofacial fractures, including those not precisely defined by the Le Fort classification scheme."}, {"id": "wiki20220301en162_12652", "title": "Le Fort fracture of skull", "score": 0.013484656754191712, "content": "A Le Fort fracture of the skull is a classic transfacial fracture of the midface, involving the maxillary bone and surrounding structures in either a horizontal, pyramidal or transverse direction. The hallmark of Lefort fractures is traumatic pterygomaxillary separation, which signifies fractures between the pterygoid plates, horseshoe shaped bony protuberances which extend from the inferior margin of the maxilla, and the maxillary sinuses. Continuity of this structure is a keystone for stability of the midface, involvement of which impacts surgical management of trauma victims, as it requires fixation to a horizontal bar of the frontal bone. The pterygoid plates lie posterior to the upper dental row, or alveolar ridge, when viewing the face from an anterior view. The fractures are named after French surgeon René Le Fort (1869–1951), who discovered the fracture patterns by examining crush injuries in cadavers. Signs and symptoms"}, {"id": "wiki20220301en308_18722", "title": "Dislocation of jaw", "score": 0.013051513051513051, "content": "may be fractured. Superior dislocations occur after being punched below the mandibular ramus as the mouth remains half-open. Since great force occurs in a punch, the angle of the jaw will be forced upward moving towards the condylar head. This can result in a fracture of the glenoid fossa and displacement of the condyle into the middle cranial fossa, potentially injuring the facial and vestibulocochlear nerves and the temporal lobe. Lateral dislocations move the mandibular condyle away from the skull and are likely to happen together with jaw fractures."}, {"id": "wiki20220301en308_18721", "title": "Dislocation of jaw", "score": 0.012981959674566678, "content": "There are four different positions of jaw dislocation: posterior, anterior, superior and lateral. The most common position is anterior, while the other types are rare. Anterior dislocation shifts the lower jaw forward if the mouth excessively opens. This type of dislocation may happen bilaterally or unilaterally after yawning. The muscles that are affected during anterior jaw dislocation are the masseter and temporalis which pull up on the mandible and the lateral pterygoid which relaxes the mandibular condyle. The condyle can get locked in front of the articular eminence. Posterior dislocation is possible for people who get injured by being punched in the chin. This dislocation will push the jaw back affecting the alignment of the mandibular condyle and mastoid. The external auditory canal may be fractured. Superior dislocations occur after being punched below the mandibular ramus as the mouth remains half-open. Since great force occurs in a punch, the angle of the jaw will be forced"}, {"id": "wiki20220301en035_14844", "title": "Battle's sign", "score": 0.012554860061874956, "content": "Battle's sign, also known as mastoid ecchymosis, is an indication of fracture of middle cranial fossa of the skull. These fractures may be associated with underlying brain trauma. Battle's sign consists of bruising over the mastoid process as a result of extravasation of blood along the path of the posterior auricular artery. The sign is named after William Henry Battle. Battle's sign takes at least one day to appear after the initial traumatic basilar skull fracture, similar to raccoon eyes. It is usually seen after head injuries resulting in injury to mastoid process leading to bruising. Battle's sign may be confused with a spreading hematoma from a fracture of the mandibular condyle, which is a less serious injury. See also Basilar skull fracture Raccoon eyes Black eye References Traumatology Injuries"}, {"id": "wiki20220301en225_19282", "title": "Facial trauma", "score": 0.012231106034646867, "content": "Signs and symptoms Fractures of facial bones, like other fractures, may be associated with pain, bruising, and swelling of the surrounding tissues (such symptoms can occur in the absence of fractures as well). Fractures of the nose, base of the skull, or maxilla may be associated with profuse nosebleeds. Nasal fractures may be associated with deformity of the nose, as well as swelling and bruising. Deformity in the face, for example a sunken cheekbone or teeth which do not align properly, suggests the presence of fractures. Asymmetry can suggest facial fractures or damage to nerves. People with mandibular fractures often have pain and difficulty opening their mouths and may have numbness in the lip and chin. With Le Fort fractures, the midface may move relative to the rest of the face or skull."}, {"id": "pubmed23n1044_26161", "title": "Comparison of accuracy of computed tomography scan and ultrasonography in the diagnosis of mandibular fractures.", "score": 0.01161337018028688, "content": "Ultrasonography (USG) allows to the examination of soft tissue and osseous tissues in the head-and-neck region. This study compared the accuracy of USG and computed tomography (CT) scan in the diagnosis of mandibular fractures. In this prospective observational study, spiral CT scan was prescribed for the lower face and, if necessary, midface and upper face in 42 trauma patients suspected of mandibular fractures, referring to Imam Reza Hospital in Tabriz. Two radiologists evaluated the CT scans. Then, another radiologist examined all the patients with USG with a frequency of 7-12 MHz. Ultrasonographic diagnostic results were recorded and compared with the results of the CT scan examinations. The results were reported using descriptive statistical methods. The specificity and sensitivity of USG were 100% and 91.1%, respectively. The USG sensitivities in the angle, condyle, condylar neck, and symphysis fractures were 100%, 91.6%, 85.7%, and 80%, respectively, and the specificity was 100% in all that anatomical regions. Among the confounding factors, the sensitivity of the USG (84.6%) was the lowest in the presence of hematoma; however, its specificity remained 100%. One case of symphysis fracture was not detected in the absence of any confounding factors in USG examination. Although the sensitivity, specificity, and diagnostic accuracy of the USG were at high levels, there were some limitations, making it difficult to definitively replace USG with CT scans, especially in the case of condylar fractures and in the presence of confounding factors such as hematoma and swelling."}, {"id": "pubmed23n0278_13488", "title": "Radiology of maxillofacial trauma.", "score": 0.011434702636419374, "content": "There has been a rising incidence of maxillofacial injuries during the past decade as a result of an increasing number of assaults and motor vehicle accidents. The maxillofacial region is one of the most complex areas of the human body, and the radiographic imaging of this region becomes even more difficult in traumatized patients because of their clinical condition and their inability to cooperate. Imaging modalities used in the evaluation of the traumatized maxillofacial region include conventional (plain) films, tomography, panoramic radiography, computed tomography, three-dimensional computed tomography, DentaScan, and magnetic resonance imaging. Each modality is discussed with regard to technique, advantages, and disadvantages. Plain films and computed tomography, the modalities that are used most in evaluating maxillofacial structures, are discussed in more detail. The normal anatomy and radiologic features are presented for both of these modalities. Radiographic evaluation of maxillofacial injury begins with a knowledge of the direct and indirect radiographic signs of injury seen on most imaging modalities. Computed tomography also has allowed a method of classifying facial fractures that is based on the involvement of the facial buttresses or struts. Three horizontal, two coronal, and five sagittal oriented struts are described. Limited fractures are differentiated from transfacial fractures by the lack of involvement of the pterygoid plates in the limited fractures. Limited fractures also can be subclassified as solitary (fracture of a single strut) or complex (fractures of multiple struts). A portion of the orbit is involved in almost every form of facial fracture; therefore, evaluation of facial injuries should always include the orbital structures. Although both can occur simultaneously, orbital injuries can be divided into soft tissue and bony vault injuries. Similar to midface fractures, orbital fractures also can be classified as solitary (fracture involves a single wall) or complex (fracture involves more than one wall or a part of a midface fracture). Computed tomography is of great value in evaluating both forms of injury. Magnetic resonance imaging is becoming increasingly important in the evaluation of orbital soft tissue injuries. Classification of midface injuries includes the solitary strut fractures and the complex strut fractures. Solitary strut fractures include fractures of the nasal arch, zygomatic arch, and isolated sinus wall fractures. Complex strut fractures include the nasal complex fractures, zygomatic (tripod) and zygomaticomaxillary fractures, transfacial fractures (LeFort fractures), and facial smash fractures. Each fracture type and its radiographic appearance are discussed.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "wiki20220301en225_19286", "title": "Facial trauma", "score": 0.010281385281385282, "content": "At the beginning of the 20th century, René Le Fort mapped typical locations for facial fractures; these are now known as Le Fort I, II, and III fractures (right). Le Fort I fractures, also called Guérin or horizontal maxillary fractures, involve the maxilla, separating it from the palate. Le Fort II fractures, also called pyramidal fractures of the maxilla, cross the nasal bones and the orbital rim. Le Fort III fractures, also called craniofacial disjunction and transverse facial fractures, cross the front of the maxilla and involve the lacrimal bone, the lamina papyracea, and the orbital floor, and often involve the ethmoid bone, are the most serious. Le Fort fractures, which account for 10–20% of facial fractures, are often associated with other serious injuries. Le Fort made his classifications based on work with cadaver skulls, and the classification system has been criticized as imprecise and simplistic since most midface fractures involve a combination of Le Fort fractures."}, {"id": "pubmed23n0678_14622", "title": "Surgical treatment on displaced and dislocated sagittal fractures of the mandibular condyle.", "score": 0.010002494387627837, "content": "The purpose of this study was to evaluate the effect of surgical treatment on displaced and dislocated sagittal fractures of the mandibular condyle (SFMC). Twenty-four patients with 28 displaced and dislocated SFMCs were distinguished into type M, type C, and type L fractures according the location of the fracture line. The fractured fragment was reduced and fixated with two 0.6-mm 4-hole micro-plates via a preauricular temporal incision. The fragment was extirpated when it was too small to be fixated. The postoperative position and profile of the fragment was examined by orthopantomogram radiograph or computed tomography (CT). The function of the temporal and zygomatic branches of the facial nerve was inspected. The occluding relation was surveyed, the interincisal distance at maximum mouth opening was measured, and the deviation from the midline during mouth opening was recorded. Twenty-three condyles (82%) suffered dislocated fractures with the condylar fragment out of the glenoid fossa. Five condyles (18%) were displaced, but not dislocated. There were 2 (7%) type M, 19 (68%) type C (3 comminuted), and 7 (25%) type L fractures (1 comminuted), respectively. Twenty-one (75%) fractured fragments received free-graft procedures with 2 micro-plates. Four (14%) fragments were reduced and fixated without being dissected free of their attachments. Three (11%) fragments were extirpated. There were no permanent facial never branch injuries. Micro-plate removal was necessary because of postoperative infection and necrosis of the fractured fragment in 1 condylar process. No other patients could be found with obvious postoperative bone resorption. The average postoperative maximum mouth opening and deviation at 6 months were improved significantly. The postoperative occlusion was good in 22 cases. Access with the preauricular incision, and the dislocated and displaced fragment can be reduced and fixated to its normal position easily. Free-graft procedure is a suitable surgical treatment if the fractured fragment cannot be reduced without dissection free of the pterygoid muscle attachment. Although most fractured fragments in SFMCs have to be dissected free, there are no obvious complications in dislocated and displaced SFMCs after surgical treatment."}, {"id": "wiki20220301en336_10994", "title": "Mandible", "score": 0.009948785807052366, "content": "Clinical significance Fracture One fifth of facial injuries involve a mandibular fracture. Mandibular fractures are often accompanied by a 'twin fracture' on the opposite side. There is no universally accepted treatment protocol, as there is no consensus on the choice of techniques in a particular anatomical shape of mandibular fracture clinic. A common treatment involves attachment of metal plates to the fracture to assist in healing. The mandible may be dislocated anteriorly (to the front) and inferiorly (downwards) but very rarely posteriorly (backwards). The articular disk of the temporomandibular joint prevents the mandible from moving posteriorly, making the condylar neck particularly vulnerable to fractures."}, {"id": "pubmed23n1009_15212", "title": "[Sagittal fracture of mandibular condyle: a review of 151 cases].", "score": 0.009900990099009901, "content": "<bObjective:</b A retrospective research was made to summarize the clinical characteristics, treatment methods, results of the adult cases with sagittal fracture of mandibular condyle (SFMC). <bMethods:</b One hundred and fifty-one cases of hospitalized patients were enrolled. The age, sex, etiology, level of fracture, degree of displacement, associated facial fractures, treatment methods and results were retrospectively analyzed. <bResults:</b The patient's age ranged from 16 to 81 years old, with a median age of 38.5 years. The male to female ratio was 2.97∶1. The most involved age group was 20-29 years old [35.1% (53/151)]. Falls [53.6% (81/151)] were the most common cause. According to the classification of He (2009) and Duan (2011), the most common type of SFMC was type A [60.5% (130/215)] and the displacement type [80.9% (174/215)]. Eighty-six point zero percent (185/215) of SFMC were treated by surgery. The surgical rates of type A, B and M fractures were 91.5% (119/130), 79.6% (43/54) and 88.5% (23/26), with significant differences between the groups (<iP</i&lt;0.05). The surgical rates of the displacement and dislocation type were 89.7% and 100%, with significant differences. The differences between the fixations of type A, type B and type M fractures were statistically significant. The follow-up results showed that, 78.7% (59/75) of patients treated with surgery had normal occlusion, no joint symptoms and no limited mandibular movement. Patients treated with conservative therapy had good occlusion and an average maximum mouth opening of 36.25 mm with malunion occurred in 5/6 of the condyles. <bConclusions:</b Under appropriate surgical indications, surgical treatment of SFMCs could achieve significantly better outcomes than conservative treatment."}, {"id": "pubmed23n1069_6317", "title": "Operator Experience and Fracture Location Affects the Rate of Facial Nerve Injury in Condylar Fractures: An Analysis of 89 Cases.", "score": 0.00980392156862745, "content": "The purpose of this study was to measure the frequency and identify risk factors for facial nerve injury (FNI) in the open treatment of condylar neck and subcondylar fractures. A prospective cohort study was conducted over 5 years on patients who were treated surgically for mandibular condylar fractures using the retomandibular transparotid approach (RMTA). The primary result was FNI occurrence (yes/no). The predictor variables were demographic, fracture location, and pattern (dislocation, present or not), as well as surgeon experience. Post-treatment functional facial nerve changes were initially assessed in the operating room as the patient regained consciousness and documented thereafter within, the 1st and 3rd weeks, and 3rd and 6th months. Appropriate statistics were computed and, SPSS version 16 was used to analyze the data. χ<sup2</sup test and Fisher exact test were used to assess significance (P ≤ 0.05). Eighty-nine patients with 102 condylar fractures (63 subcondylar and 26 condylar neck), with a mean age of 28.5±7.5 years and 91% men were evaluated. There were 15 subjects (16.8%) with FNI and among them 6 subjects had persistent facial weakness for 6-8 weeks that completely resolved within 3 months, with no permanent facial nerve paralysis. The marginal mandibular (n = 7), buccal (n = 6), and zygomatic (n = 2) were the facial nerve branches involved. Risk factors for FNI were operator' inexperience, fracture-dislocation, and condylar neck fracture to the site and location of the fracture. Multivariate logistic regression showed that the location of the fracture at neck level (0.030∗), fracture dislocation (&lt;0.001∗), and operator's inexperience (0.003∗) were significant risk factors for postoperative facial nerve injury (P ≤ 0.05). If conducted properly, the RMTA is a safe method for treating condylar fractures with rare major complications; however, fracture dislocation, fractured condylar neck, and operator' in-experience were significantly associated with increased risk of developing transient postoperative FNI."}, {"id": "wiki20220301en240_464", "title": "Mandibular fracture", "score": 0.009723609723609724, "content": "Epidemiology Mandible fracture causes vary by the time period and the region studied. In North America, blunt force trauma (a punch) is the leading cause of mandible fracture whereas in India, motor vehicle collisions are now a leading cause. On battle grounds, it is more likely to be high velocity injuries (bullets and shrapnel). Prior to the routine use of seat belts, airbags and modern safety measures, motor vehicle collisions were a leading cause of facial trauma. The relationship to blunt force trauma explains why 80% of all mandible fractures occur in males. Mandibular fracture is a rare complication of third molar removal, and may occur during the procedure or afterwards. With respect to trauma patients, roughly 10% have some sort of facial fracture, the majority of which come from motor vehicle collisions. When the person is unrestrained in a car, the risk of fracture rises 50% and when an unhelmeted motorcyclist the risk rises 4-fold."}, {"id": "pubmed23n0387_17881", "title": "[X-ray and clinical characteristics in sagittal fracture of the mandibular condyle].", "score": 0.009708737864077669, "content": "To study the X-ray and clinical characteristics in sagittal fracture of the mandibular condyle (SFMC). 14 cases (15 sides) of SFMC in 48 cases who had condylar fractures were studied in the research, every patient was examined by the conventional x-ray, 2D-CT and 3D-CT, and followed up for 3-39 months. The fracture line mainly passed through lateral 1/3 and middle 1/3 of anterior condyle and the middle 1/3 of posterior condyle. Following-up (3-39 months) showed that the patients' occlusions were normal in all cases. The fracture line mainly located in the lateral pterygoid muscle fossa. Good results can be gotten by effective treatment. The 2D-CT and 3D-CT are valuable to the diagnosis."}, {"id": "wiki20220301en240_435", "title": "Mandibular fracture", "score": 0.009562670014647416, "content": "Other symptoms may include loose teeth (teeth on either side of the fracture will feel loose because the fracture is mobile), numbness (because the inferior alveolar nerve runs along the jaw and can be compressed by a fracture) and trismus (difficulty opening the mouth). Outside the mouth, signs of swelling, bruising and deformity can all be seen. Condylar fractures are deep, so it is rare to see significant swelling although, the trauma can cause fracture of the bone on the anterior aspect of the external auditory meatus so bruising or bleeding can sometimes be seen in the ear canal. Mouth opening can be diminished (less than 3 cm). There can be numbness or altered sensation (anesthesia/paraesthesia in the chin and lower lip (the distribution of the mental nerve)."}, {"id": "pubmed23n0219_9385", "title": "[Statistical notes on sinusal fractures of the face].", "score": 0.009523809523809525, "content": "Factors of age, sex, localization and associated lesions were analyzed for 9 319 cases of fractures of the face, using data collected by the Army Computer Centre for Medical Data Processing."}, {"id": "wiki20220301en308_18723", "title": "Dislocation of jaw", "score": 0.009482515510434292, "content": "Posterior, superior and lateral dislocations are uncommon injuries and usually result from high-energy trauma to the chin. By contrast, anterior dislocations are more often the result of low-energy trauma (e.g. tooth extraction) or secondary to a medical condition that affects the stability of the joint (e.g. seizures, ligamentous laxity, degeneration of joint capsule). Diagnosis As with other joint dislocations, clinical history and examination are crucial for diagnosis of a jaw dislocation. Commonly, plain and panoramic X-ray radiographies are used to determine the relative position of the mandibular condyle. If a complex or unusual injury is suspected, three-dimensional computed tomography is most reliable in diagnosing dislocation and possibly associated fractures or soft tissue injuries."}, {"id": "pubmed23n0308_9270", "title": "Unilateral medial dislocation of the temporomandibular joint.", "score": 0.009433962264150943, "content": "We present our experience of the rare condition of unilateral medial dislocation of the temporomandibular joint (TMJ) in 11 patients with head trauma who had received a direct lateral blow on the chin. The diagnosis was made by direct coronal CT of the TMJ performed from 6 h to 7 days following the injury. In 6 patients, subcondylar fracture of the ipsilateral mandibular ramus was also demonstrated. A second CT performed 11-16 months following the first one demonstrated pseudoarthrosis of the fractured ramus in these 6 patients. The second CT was identical to the first in the remaining 5 patients with pure dislocation of the condyle. All patients suffered from severe disability of the TMJ. The maximal vertical distance between the upper and lower incisors in patients with uncomplicated dislocation ranged between 8 and 12 mm. In cases with complicated medial condylar dislocation with fracture and pseudoarthrosis of the mandibular ramus, this distance ranged between 16 and 25 mm, probably because of additional movement in the area of the pseudoarthrosis. The maximal vertical distance between the incisors was compared with a control group of 20 normal adults who had values from 40 to 52 mm. Medial unilateral dislocation of the TMJ can appear in two forms: uncomplicated or complicated, with pseudoarthrosis of the ipsilateral mandibular ramus."}, {"id": "pubmed23n0035_3652", "title": "[The diagnosis of condylar process fractures].", "score": 0.009433962264150943, "content": "The detection of condylar process fractures by clinical examination and the interpretation of radiographs belong to the stomatologist's diagnostic tasks. On the contrary, the exact location of the fracture lines and the recognition of the types of proximal fragment displacement often present problems. Diagnostic errors may also occur, mainly resulting from radiological illusions. The comprehensive evaluation of the data from 3027 patients with injuries of the facial bones, involving 1050 condylar process fractures, and the verification of the relevant radiological findings permitted to determine the location of the fracture lines and the types of fragment displacement, to recognize misinterpretations and to give suggestions for establishing a definite diagnosis."}, {"id": "pubmed23n0043_15955", "title": "[Sagittal fracture of the mandibular condyle (SFMC): its clinical image diagnosis].", "score": 0.009259259259259259, "content": "Each of 12 SFMC patients (13 TMJs) underwent 8 X-ray examinations. It was found that SFMCs were easily missed from the conventional X-ray views, while CPMOT (Condyle pterygoid-maxillo oblique tomography) and CT were effective to demonstrate them. The reasons for SFMCs' being missed from the conventional views are discussed. It is concluded that the best procedure to show a possible SFMC is: the reversed Town's view--&gt;TMJ coronal tomography or CPMOT--&gt;CT (using coronal or CPMOT sections as the first choice). The authors declare that SFMC could not be explained by the acknowledged theory that the neck of condyle is a safety mechanism to protect the brain."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": true, "char_ranges": [[38, 131]], "word_ranges": [[5, 19]], "text": "Option 1 is strongly discarded because endophthalmitis typically presents with a lot of pain."}, "2": {"exist": true, "char_ranges": [[882, 1068]], "word_ranges": [[141, 175]], "text": "Technically, both option 2 and 4 (an acute hemorrhagic DPV) could cause these symptoms, but the most correct option is option 2 (and I am sure it is the one the examiner wants answered)."}, "3": {"exist": true, "char_ranges": [[132, 252]], "word_ranges": [[19, 39]], "text": "Option 3 of wet form of age-related macular degeneration is also ruled out, because it usually occurs in older patients,"}, "4": {"exist": true, "char_ranges": [[882, 1068]], "word_ranges": [[141, 175]], "text": "Technically, both option 2 and 4 (an acute hemorrhagic DPV) could cause these symptoms, but the most correct option is option 2 (and I am sure it is the one the examiner wants answered)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "This question could be controversial. Option 1 is strongly discarded because endophthalmitis typically presents with a lot of pain. Option 3 of wet form of age-related macular degeneration is also ruled out, because it usually occurs in older patients, although what a patient with myopia magna may present with is myopic macular degeneration (with a similar clinical picture to AMD, but this is a different pathology). A posterior vitreous detachment (PVD) is usually not very symptomatic, producing myodesopsias but no loss of vision or pain; however, some PVDs are of the hemorrhagic type, by traction of the vessels, producing a vitreous hemorrhage that would cause loss of vision. In any case, due to the history of myopia magna and previous intraocular surgery, the first diagnosis to rule out would be retinal detachment, since these are two risk factors for this pathology. Technically, both option 2 and 4 (an acute hemorrhagic DPV) could cause these symptoms, but the most correct option is option 2 (and I am sure it is the one the examiner wants answered).", "full_answer_no_ref": "This question could be controversial. Option 1 is strongly discarded because endophthalmitis typically presents with a lot of pain. Option 3 of wet form of age-related macular degeneration is also ruled out, because it usually occurs in older patients, although what a patient with myopia magna may present with is myopic macular degeneration (with a similar clinical picture to AMD, but this is a different pathology). A posterior vitreous detachment (PVD) is usually not very symptomatic, producing myodesopsias but no loss of vision or pain; however, some PVDs are of the hemorrhagic type, by traction of the vessels, producing a vitreous hemorrhage that would cause loss of vision. In any case, due to the history of myopia magna and previous intraocular surgery, the first diagnosis to rule out would be retinal detachment, since these are two risk factors for this pathology. Technically, [HIDDEN].", "full_question": "A 47-year-old man with myopia magna, who underwent cataract surgery 2 years ago, comes to the emergency room reporting a profound and painless loss of vision in his right eye. Which of the following diagnoses can cause this symptomatology?", "id": 309, "lang": "en", "options": {"1": "Post-surgical endophthalmitis.", "2": "Retinal detachment.", "3": "Age-related macular degeneration, wet form.", "4": "Posterior vitreous detachment.", "5": NaN}, "question_id_specific": 215, "type": "OPHTHALMOLOGY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0725_4623", "title": "[Posterior vitreous detachment and cystoid macular oedema post-cataract surgery: a case report].", "score": 0.015582808280828083, "content": "The posterior vitreous detachment consists in the separation between the posterior vitreal cortex and internal limiting membrane of the retina. This is the peak of the vitreal para-physiological age-related modifications. This problem occurs in 6% of normal people of age between 45 and 65 years and in 65% of individuals between 65 and 85 years. Several elements can be responsible for vitreous modifications, such as senility, myopia, aphakia, pseudophakia, diabetes, degeneration vitreous retinal hereditary, traumatisms, inflammation. A 75 year old male patient has come to our attention for a left eye cataract. He has undergone to a series of OCT: the first before surgery showed a perifoveal vitreous detachmen; the following ones until six months after surgery put in evidence the DPV progression accompanied by EMC, relating it with visual symptomatology. Therefore, OCT is a useful tool for a clinical analysis but also for the contribution to research concerning the pathogenesis of diseases due to vitreo-retinal modification."}, {"id": "wiki20220301en028_4306", "title": "Retinal detachment", "score": 0.01475924291458272, "content": "Retinal detachment is more common in those with severe myopia (above 5–6 diopters), as their eyes are longer, their retina is thinner, and they more frequently have lattice degeneration. The lifetime risk increases to 1 in 20. Myopia is associated with 67% of retinal detachment cases. Patients suffering from a detachment related to myopia tend to be younger than non-myopic detachment patients. Retinal detachment can occur more frequently after surgery for cataracts. The estimated of risk of retinal detachment after cataract surgery is 5 to 16 per 1000 cataract operations. The risk may be much higher in those who are highly myopic, with a frequency of 7% reported in one study. Young age at cataract removal further increased risk in this study. Long term risk of retinal detachment after extracapsular and phacoemulsification cataract surgery at 2, 5, and 10 years was estimated in one study to be 0.36%, 0.77%, and 1.29%, respectively."}, {"id": "pubmed23n1107_19022", "title": "Bilateral Retinal Detachments in a Healthy 22-year-old Woman After Moderna SARS-COV-2 Vaccination.", "score": 0.014381914381914381, "content": "Although uncommon, retinal detachments are medically urgent and can result in permanent vision loss if untreated. Bilateral retinal detachments in healthy individuals are even more rare. In addition, there are no cases to date of retinal detachment associated with either coronavirus disease 2019 (COVID-19) or after receiving the Moderna (mRNA-1273) severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) vaccine. A 22-year-old woman with myopia but no ocular trauma or other major medical history presented to the emergency department with 5 days of progressive, painless vision loss in her right eye. On examination, her visual acuity with corrective lenses was 20/70 in the right eye, 20/20 in the left eye, and 20/25 with both eyes open. Point-of-care ultrasound of the eye showed a retinal detachment in the right eye. She was subsequently seen by ophthalmology and diagnosed with bilateral retinal detachments (macula off in the right, macula on in the left), despite being asymptomatic in her left eye. She underwent bilateral vitrectomies for simultaneous rhegmatogenous retinal detachments. Although the patient denied any preceding trauma, she did note having received her second dose of the COVID-19 vaccine 10 days before the onset of symptoms.Why Should an Emergency Physician Be Aware of This? We present a rare and unusual case of simultaneous bilateral retinal detachments in a healthy, young woman with no major medical history or medications. She received the COVID-19 vaccine a few days prior. Our case outlines a possible association with the vaccine and emphasizes the importance of ultrasonography in diagnosing time-sensitive medical conditions."}, {"id": "pubmed23n0688_8193", "title": "[Pseudophakic retinal detachment].", "score": 0.014043245756800743, "content": "Modern phacoemulsification has established itself as a safe and very rewarding surgical procedure. Patients and surgeons may not associate late complications with the initial surgery. However, recent studies have demonstrated that such a causal relationship may persist for many years after the cataract procedure and that there is a significant increase in the risk for developing a retinal detachment during the postoperative years. The mean time period between cataract surgery and pseudophakic retinal detachment is between 3 and 4 years. Even uncomplicated cataract surgery alters the physiological processes within the eye and can lead to progressive destruction of the vitreous for many years after the surgery. Therefore, the risk for a retinal detachment is increased for at least 10 years after the initial procedure. In recent epidemiological studies, the most important risk factors for pseudophakic retinal detachment were myopia, younger age and male gender. If all factors are combined, the cumulative risk for developing a retinal detachment after cataract surgery may rise to 20 %. Additional factors that may increase this risk are additional intraoperative complications, for example, rupture of the posterior capsule, vitreous loss or dropped nucleus. Compared to phakic retinal detachments, pseudophakic patients on average present with a shorter history of visual symptoms, are older, more commonly male and display fewer, smaller and more anteriorly located retinal breaks that frequently are only detected during surgery. The anatomic success rates have improved significantly over the past years, in particular through the advances and increasing popularity of primary vitrectomy. However, functional results are still disappointing. Only about half of the patients will achieve reading ability without low vision aids. The increased and long-term risk for pseudophakic retinal detachment should be part of the preoperative consent process of any cataract surgery, in particular, in young myopic males."}, {"id": "wiki20220301en059_60326", "title": "Posterior vitreous detachment", "score": 0.013674798721527694, "content": "Age and refractive error play a role in determining the onset of PVD in a healthy person. PVD is rare in emmetropic people under the age of 40 years, and increases with age to 86% in the 90s. Several studies have found a broad range of incidence of PVD, from 20% of autopsy cases to 57% in a more elderly population of patients (average age was 83.4 years). People with myopia (nearsightedness) greater than 6 diopters are at higher risk of PVD at all ages. Posterior vitreous detachment does not directly threaten vision. Even so, it is of increasing interest because the interaction between the vitreous body and the retina might play a decisive role in the development of major pathologic vitreoretinal conditions, such as epiretinal membrane. PVD may also occur in cases of cataract surgery, within weeks or months of the surgery."}, {"id": "pubmed23n0060_696", "title": "[Stability of posterior chamber lenses 3-5.5 years after implantation in capsular rupture].", "score": 0.013654943179266047, "content": "In cases of PC-IOL implantation and ruptured capsule there will be a higher risk of the lens loosening into the vitreous, although many successful cases have been reported. Three years ago we investigated 42 eyes with PC-IOL that had experienced previous posterior capsular or zonular rents and partial vitreous loss. Three to 5.5 years after PC-IOL implantation we have now performed a second investigation on 38 of these patients to examine fixation of the lenses, visual acuity, intraocular pressure and the fundus of the eye. The 38 lenses all remained stable. The rate of patients achieving 20/40 or better visual acuity decreased from 68% at the first investigation to 55% (21/38). Excluding all patients with vision-limiting preoperative conditions such as age-related macular degeneration, we found a relation-ship of 83% to 75% (21/28). In addition to the three retinal detachments and two cases of endophthalmitis found during the first investigation, we also found another retinal detachment at the follow-up examination. Only two of these six eyes with severe complications had useful vision. We conclude from our investigation that PC-IOL implanted following a posterior capsule-zonular break during ECCE can remain stable, but still lead to serious complications."}, {"id": "pubmed23n0566_13361", "title": "Two-year results of surgical removal of choroidal neovascular membranes related to non-age-related macular degeneration.", "score": 0.01266025641025641, "content": "To present the 2-year outcomes of surgical removal of non-age-related macular degeneration (AMD)-related choroidal neovascular membranes and to evaluate any association between visual outcome and baseline clinical factors. Retrospective consecutive case series. All patients who had surgery for non-AMD-related choroidal neovascularisation (CNV) between November 1997 and March 2003 under the care of a single surgeon (WA) were included in the study. Baseline data including patient age, duration of subfoveal CNV, preoperative visual acuity (VA), lesion size, lesion components and aetiology were collected. The primary outcome was VA change with secondary outcomes retinal detachment, operative peripheral retinal break formation, CNV recurrence and cataract. A total of 52 eyes were included in the study. The aetiology of CNV was: punctate inner choridopathy 21 (40%); idiopathic 8 (15%); pathologic myopia 6 (12%); ocular histoplasmosis syndrome 1 (2%); and other 16 (31%). The mean age of patients was 41(range 14-72) years. 24-month follow-up was available for 41 (80%) eyes. The mean logMAR equivalent baseline acuity was 1.1 and mean lesion size 1.2 disc areas. An improvement in VA &gt;1 Snellen line was noted in 26 (63%) eyes, whereas 10 (24%) eyes remained the same (within 1 line) and 5 (12%) lost &gt;1 line of acuity. Improvement in VA was associated with worse baseline VA (84% for eyes with VA &lt;or=6/36 vs 31% for those with VA&gt;6/36, p=0.001). No evidence of association between 2-year visual outcome and any other baseline factor under study was observed. Peripheral retinal breaks were noted in 5 (10%) eyes at the time of surgery, and 3 (5.8%) eyes developed postoperative retinal detachments. Persistent/recurrent CNV was noted in 17 (33%) eyes. The median time to presentation of CNV in these eyes was 27 (range 2-172) weeks. Five eyes underwent cataract surgery during the follow-up period. The mean age of these patients was significantly higher than the mean age of those who did not require cataract surgery (57 vs 37 years, p=0.014). Surgical excision of non-AMD-related CNV resulted in improvement of VA in the majority of eyes. Worse presenting acuity was associated with better visual improvements."}, {"id": "pubmed23n0502_2778", "title": "[Macular translocation--first experience].", "score": 0.012546269238262225, "content": "The age related macular degeneration (ARMD) is the most common cause of the central visual acuity loss in persons of age more than 60 years in the well developed countries. Rotation of the macula is nowadays a progressive method of choice of treatment of the exsudative form of ARMD. The aim of this surgical technique is to relocate the neuroretinal epithelium of the central region of the retina to a position situated outside the border of the subfoveolar lesion. Three eyes of three patients (2 woman and one man) were operated on during the period between March and May 2001 at the Department of Ophthalmology of the School of Medicine, Charles University in Pilsen. The method used was the scleral imbrication, which belongs to methods designated as \"limited translocation\". The mean age of the patients was 62 years and the follow up period was 2 years. After the surgery the relocation of the fovea was observed in all three eyes. In two eyes, the postoperative period was complicated by tractional retinal detachment that occurred three weeks after the surgery as a consequence of progressive proliferative vitreoretinopathy (PVR). Both eyes with the retinal detachment were re-operated. In both of them, the repeated pars plana vitrectomy, epiretinal membranes removal with relaxing retinectomy was performed and after maximal mobilization of the retina, the silicone oil implantation followed. In the second patient, the postoperative period was complicated by elevation of the intraocular pressure and a radial retinal fold running from the encircling buckle indentation up to the macula. Slightly improved function was noticed only in the first patient after following cataract surgery with intraocular lens implantation. In the two other eyes, as noticed at the last follow up check, the postoperative complications caused severe decrease of the central visual acuity although the retina remained attached. Macular translocation procedure is in stage of development and its surgical techniques are being further modified. The risk of postoperative complications with profound loss of central visual acuity corresponds to the level of the technical difficulty and extent of surgical intervention."}, {"id": "wiki20220301en536_30480", "title": "Atul Kumar (ophthalmologist)", "score": 0.012146743900298403, "content": "Achievements and positions Kumar is a specialist in diseases of the retina, vitreous and uvea and their management. His academic disciplines include Vitreoretinal surgery, Ophthalmic Lasers, Uveal diseases, Macular Hole surgery, anti-VEGF injections, Age Related Macular Degeneration, Retinal Detachment surgery, Myopic Traction Maculopathy, Pathological Myopia and Macular Hole Retinal Detachment."}, {"id": "pubmed23n0597_21915", "title": "Fellow eye findings of highly myopic subjects operated for retinal detachment associated with a macular hole.", "score": 0.011972693856117626, "content": "To identity anatomic risk factors involved in the onset of retinal complications causing decrease of visual acuity (VA) in the fellow eyes of highly myopic patients operated for retinal detachment with macular hole (RDMH). Cohort study. Ninety-eight patients (mean age, 51.5+/-8.0 years) with bilateral high myopia (mean myopia of the fellow eye, 20.4+/-5.5 diopters) affected by RDMH in the other eye at baseline. Evaluation of the anatomic features at baseline and during 84+/-2.7 months of follow-up by biomicroscopic examination, indirect binocular ophthalmoscopy, B-scan ultrasonography, and optical coherence tomography. Detection of anatomic features associated with onset of retinal complications causing decrease of VA during the follow-up period. The fellow eyes were divided into 2 groups according to the clinical features of the RDMH eyes: Group 1, presence of posterior vitreous detachment (PVD); and Group 2, presence of posterior vitreous schisis (PVS). At baseline, the incidence of PVD in group 1 was 31 of 47 eyes (65.9%) and the incidence of PVS in Group 2 was 42 of 51 eyes (82.3%). At the end of follow-up, group 1 eyes had a lower incidence of retinal complications causing visual decrease than group 2 eyes (group 1, 2/47 eyes; group 2, 9/51 eyes). Fellow eyes of RDMH cases with higher degree of myopia and peculiar vitreoretinal features including PVS, posterior epiretinal membrane, severe posterior staphyloma, and chorioretinal atrophy are more likely to develop retinal complications causing decrease of VA."}, {"id": "wiki20220301en564_9633", "title": "Acute visual loss", "score": 0.011518046709129513, "content": "Acute visual loss is a rapid loss of the ability to see. It is caused by many ocular conditions like retinal detachment, glaucoma, macular degeneration, and giant cell arteritis, etc. Main causes Retinal detachment Retinal detachment should be considered if there were preceding flashes or floaters, or if there is a new visual field defect in one eye. If treated early enough, retinal tear and detachment can have a good outcome. Glaucoma Angle-closure glaucoma should be considered if there is painful loss of vision with a red eye, nausea or vomiting. The eye pressure will be very high typically greater than 40 mmHg. Emergent laser treatment to the iris may prevent blindness. Macular degeneration Wet macular degeneration should be considered in older people with new distortion of their vision with bleeding in the macula. Vision can often be regained with prompt eye injections with anti-VEGF agents. Giant cell arteritis"}, {"id": "pubmed23n0988_22962", "title": "Retinal Detachment in 31 Eyes with Retinitis Pigmentosa.", "score": 0.011185474375658425, "content": "To describe clinical features and treatment outcomes of retinal detachment (RD) in eyes with retinitis pigmentosa (RP). Single-center, retrospective, interventional case series. All RP patients with RD examined between April 2003 and December 2013 and minimum 2 months of follow-up. Medical records of RP patients were screened and 31 eyes with RD were included. Family history of RP, duration of symptoms, age at presentation, associated ocular and systemic findings, and detailed ophthalmic evaluation including presenting visual acuity, type and amount of refractive error, fundus findings, electroretinogram details, surgical details, and postoperative complications and outcomes were evaluated. Univariate analysis was done to determine risk factors associated with RD in eyes with RP and risk factors associated with poor visual outcomes after treatment. Subset analysis was also done for comparing the functional and anatomical outcomes between patients undergoing scleral buckling or vitrectomy. Final surgical reattachment rate, best-corrected visual acuity. Mean age at presentation was 22 years (median, 17; range, 4-63). Mean duration of symptoms was 12 months (median, 3 months: range, 3 days-60 months). Associated ocular findings included nyctalopia (n = 23), myopia (n = 21), and hyperopia (n = 10). Systemic associations included hearing loss (n = 5), deaf-mutism (n = 1), and Bardet-Biedel syndrome (n = 1). No association between degree of myopia and RD was noted (P = 0.63). Observed retinal breaks included horse-shoe-shaped tear (n = 15), lattice with hole (n = 7), atrophic retinal hole (n = 3), retinal dialysis (n = 3), and macular hole (n = 3). The most common location of breaks was superotemporal quadrant (n = 15). Younger age, male gender, and presence of posterior vitreous detachment were strongly associated with RD with odds ratio of 1.3 (P = 0.001), 8.3 (P = 0.010), and 6 (P = 0.003), respectively. Retinal reattachment was achieved in 13 of 13 eyes (100%) with scleral buckle and 9 of 10 eyes (90%) with vitrectomy. Vision improved from 1.63±0.52 to 0.87±0.25 logarithm of the minimum angle of resolution (P &lt; 0.001) at a mean follow-up of 33 months (median, 24, range; 1-145). Rhegmatogenous RD in eyes with RP is rare. Precocious vitreous degeneration and sparse pigmentation in younger male patients has a role in etiopathogenesis. Visual prognosis remains poor despite satisfactory surgical outcomes."}, {"id": "pubmed23n1072_5904", "title": "Incidence of Retinal Detachment after Macular Surgery.", "score": 0.009900990099009901, "content": "Macular surgery has become an increasingly atraumatic procedure for the eye with the surgical methods that have been further developed in recent years. The most common complications include cystoid macular oedema and retinal detachment, more rarely endophthalmitis. The aim of this retrospective study is to record the number of retinal detachments following elective macular surgery. In this study we included all patients who underwent pars plana vitrectomy (ppV, 20 or 25 gauge) in the years 2009 - 2016. We then identified the patients who were hospitalised again because of retinal detachment. For the affected patients, the rate of retinal detachment, functional outcomes and possible risk factors were recorded. A total of 904 eyes were identified, of which 667 had surgery for epiretinal membrane, 188 for macular hole, and 49 for vitreomacular traction with a 20 or 25 gauge ppV. Of these 904, retinal detachment occurred in 17 (1.88%) cases. The mean time between first ppV and second ppV with retinal detachment was 248 days (3 - 1837 days). Two of the 17 patients had at least one retinal break before or during surgery. The retinal break was located inferior in six cases, superior in four; in four cases PVR retinal detachment and in three cases the foramina were distributed. Mean visual acuity was 0.27 (decimal) before macular surgery and 0.28 at the time of last presentation. Modern vitrectomy techniques reduce the complications in elective macular surgery, but do not replace the surgeon's experience."}, {"id": "pubmed23n1073_5019", "title": "Posterior pole retinal breaks causing posterior pole retinal detachment in a middle-aged man with retinal vasculitis and moderate myopia.", "score": 0.00980392156862745, "content": "A 38-year-old man presented with sudden decreased vision in the right eye 3 years ago due to vitreous haemorrhage. During follow-up, right eye fundus showed evidence of vasculitis, non-perfusion areas and neovascularisation elsewhere. Systemic evaluation findings of positive Mantoux test, QuantiFERON Gold test and right apical pleuroparenchymal fibrosis observed on high-resolution CT of the chest were suggestive of postinfection probable tubercular aetiology. He was treated with oral steroids, antitubercular therapy, intravitreal bevacizumab and anterior retinal cryopexy, leading to resolution of vasculitis and vitreous haemorrhage. Later he developed peripheral retinal flap and posterior retinal breaks at 8-month and 11-month follow-up, respectively, which were managed by barrage laser. He maintained a stable visual acuity of 20/20, N6 for the next 2 years. He then presented with sudden decreased vision in the right eye (20/50, N10). Right eye fundus showed posterior pole retinal detachment with lifting of previously barraged posterior retinal breaks. He underwent vitreoretinal surgery with gas tamponade. Recent 1-month postoperative visit showed successful retinal reattachment and visual recovery of 20/20, N6."}, {"id": "pubmed23n0786_4332", "title": "Rhegmatogenous retinal detachment--an ophthalmologic emergency.", "score": 0.00980392156862745, "content": "Rhegmatogenous retinal detachment is the most common retinological emergency threatening vision, with an incidence of 1 in 10 000 persons per year, corresponding to about 8000 new cases in Germany annually. Without treatment, blindness in the affected eye may result. Selective review of the literature. Rhegmatogenous retinal detachment typically presents with the perception of light flashes, floaters, or a \"dark curtain.\" In most cases, the retinal tear is a consequence of degeneration of the vitreous body. Epidemiologic studies have identified myopia and prior cataract surgery as the main risk factors. Persons in the sixth and seventh decades of life are most commonly affected. Rhegmatogenous retinal detachment is an emergency, and all patients should be seen by an ophthalmologist on the same day that symptoms arise. The treatment consists of scleral buckle, removal of the vitreous body (vitrectomy), or a combination of the two. Anatomical success rates are in the range of 85% to 90%. Vitrectomy is followed by lens opacification in more than 70% of cases. The earlier the patient is seen by an ophthalmologist, the greater the chance that the macula is still attached, so that visual acuity can be preserved. Rhegmatogenous retinal detachment is among the main emergency indications in ophthalmology. In all such cases, an ophthalmologist must be consulted at once."}, {"id": "pubmed23n0360_23255", "title": "[Analysis of 100 cases of retinal detachment treated with conventional surgery in the years 1997-1998].", "score": 0.009708737864077669, "content": "The observed increasing number of severe cases of retinal detachment was the ground of the attempt to state the causes of this phenomenon. Retrospective evaluation of 100 consecutive cases of retinal detachment operated on with conventional methods between September 1997 and October 1998 was performed. The following data were analysed: sex and age of patients, period between the first symptoms and time of diagnosis and surgery. The clinical characteristics of retinal surgery comprise: extent of detachment, macular involving, type and number of breaks, degenerations and PVR. In the majority of cases very severe retinal detachments were observed, including 3-4 quadrants, with large tears or multiple breaks, with PVR B or C. Duration of the detachment ranged from several days in very few cases to several months, on average lasting several weeks. Attachment of the retina was achieved in 80 cases, improvement of the visual acuity in 47. Our data indicate the necessity to improve the early diagnosis of retinal detachment and changes predisposing to its development."}, {"id": "pubmed23n0852_3539", "title": "MYCOBACTERIUM MANITOBENSE MASQUERADING AS CORYNEBACTERIUM PSEUDODIPHTHERITICUM IN A CASE OF POSTCATARACT SURGERY ENDOPHTHALMITIS.", "score": 0.009615384615384616, "content": "To describe a case of postoperative Mycobacterium manitobense endophthalmitis with good visual outcome that is the first report of endophthalmitis by this organism. Clinical and microbiological description of a patient with postoperative endophthalmitis. A 50-year-old chronic alcoholic man was referred to us with decreased vision in his right eye for 4 days. He had undergone cataract surgery with intraocular lens implantation in his right eye a month ago. The presenting vision in his right eye was counting fingers close to face. He was diagnosed as a case of postoperative endophthalmitis. Subsequently, the patient underwent pars plana vitrectomy and vitreous biopsy. The microbiologic investigation of the vitreous biopsy showed poorly stained gram-positive beaded bacilli that were acid fast, and growth in culture was identified as Corynebacterium pseudodiphtheriticum by Vitek 2 bacterial identification system. However, DNA analysis confirmed the organism to be M. manitobense. The patient responded well to repeat interventions with intravitreal antibiotics with a final reported visual acuity of 20/30 at 4 months after first intervention. Although known to be an organism causing soft-tissue infections, M. manitobense can also cause postsurgical endophthalmitis. The diagnosis can be confused with Corynebacterium sp. on smear and culture. Subsequent DNA sequencing of the culture provides definite identification of the organism."}, {"id": "pubmed23n0678_14026", "title": "[Clinical observations of macular hole with and without retinal detachment in high myopic eyes].", "score": 0.009615384615384616, "content": "To study the clinical features and the pathogenesis of macular hole with and without retina detachment (RD) in high myopic eyes. It was a retrospective series case study. The charts of high myopic patients with macular hole at our hospital from June 2006 to February 2007 were retrospectively reviewed and analyzed. Patients were divided into two groups (the RD group and non-RD group) depending on the presence of RD or not. Their clinical data and optic coherence tomography (OCT) results were further analyzed. SPSS 13.0 was used for the statistic analysis. When comparing the quantitative aspects like age, axial length and refraction, t-test was used. Categorical data, such as sex ratio, occurrence of vitreous traction, posterior staphyloma and retinoschisis were compared by using χ(2) test. Fisher's test was used in comparing eye laterality, incidence of white hole, visual acuity and posterior vitreous detachment (PVD). During this period, there were 43 patients fitting the including criteria. Among them, 36 patents (37 eyes) were in the RD group and 7 patients (7 eyes) in the no-RD group. In the RD group, the average age was 56.1, 24.3% of them (9/37) were male; percentage of left and right eyes was (11/37) and 70.3% (26/37), respectively; average refraction was (-8.9 ± 2.2) D; average axial length was (28.7 ± 2.0) mm. Visual acuity was ≤ 0.05 (72.2%) in 26 eyes and 0.05 - 0.2 (27.8%) in 10 patients. The incidence of complete and non-complete PVD was 89.2% (33/37) and 10.8% (4/37), respectively. White hole presented in 35.1% (3/37) patients. Vitreous traction and retinoschisis presented in 27.0% (10/37) and 35.1% (13/37) patients, respectively. In the non-RD group, the average age was 47.6; 16.7% of them (1/7) were male; left and right eyes were involved in 42.9% (3/7) and 57.1% (4/7), respectively. Average refraction was (-9.0 ± 1.9) D; average axial length was (28.9 ± 1.5) mm. Vision acuity was ≤ 0.05 in 3 patients (42.9%); between 0.05 - 0.2 in 3 eyes (42.9%) and ≥ 0.2 in 1 eye (14.3%). Incidence of complete and non-complete PVD was 85.7% (6/7) and 14.3% (1/7), respectively. White hole was observed in 14.3% (1/7) patients; 42.9% (3/7) patients were accompanied with vitreous traction and 71.4% (5/7) with retinoschisis. B-scan ultrasonography showed posterior staphyloma in all 44 eyes. The results of statistical analysis showed that the gender (χ(2) = 0.008) and eye laterality (χ(2) = 0.449) as well as refraction (t = 0.193), axial length (t = -0.25) and visual acuity (χ(2) = 4.509) of these two groups were similar (P &gt; 0.05). The incidences of vitreous traction (χ(2) = 0.709), white hole (χ(2) = 1.179), PVD (χ(2) = 0.071) and retinoschisis (χ(2) = 3.207) were also similar (P &gt; 0.05). But the age of the non-RD group is significantly younger than the RD group (t = 1.66, P &lt; 0.05). Various pathogenesis may involved in the occurrence of retinal detachment in highly myopic eyes with macular hole. Further study is required to improve our understanding of this entity."}, {"id": "pubmed23n0318_15287", "title": "[Early posterior capsule fibrosis after combined cataract and vitreoretinal surgery with intraocular air/SF6 gas tamponade].", "score": 0.009523809523809525, "content": "The surgical approach in treating coexisting vitreoretinal disease and cataract is controversial. We report on patients who developed early posterior capsular fibrosis after combined cataract and vitreoretinal surgery with air/SF6-gas tamponade. The medical records of 15 consecutive eyes (13 patients) who underwent combined phacoemulsification with intraocular lens implantation and vitreoretinal surgery with intraocular air/SF6-gas tamponade were retrospectively analyzed. The indications for vitreous surgery included: subfoveal neovascular membrane in age-related macular degeneration (5 eyes), macular hole (4 eyes), macular pucker (2 eyes), rhegmatogenous retinal detachment (2 eyes), persistent vitreous haemorrhage after branch retinal vein occlusion (1 eye), persistent vitreous haemorrhage and/or tractional retinal detachment in proliferative diabetic retinopathy (1 eye). The mean follow-up period was 7 months (1-13 months). A control group consisted of 15 eyes (15 patients) who underwent the equal combined operation without intraocular tamponade. The indications for vitreous surgery were persistent vitreous haemorrhage in proliferative diabetic retinopathy (5 eyes), persistent vitreous haemorrhage after branch retinal vein occlusion (5 eyes), asteroid hyalosis (2 eyes), macular pucker (1 eye), posttraumatic vitreous haemorrhage (1 eye), acute retinal necrosis (1 eye). The mean follow-up was 8 months (2-13 months). The posterior capsule was examined at the slit lamp microscopy with maximal dilated pupils. We defined posterior capsular opacification (PCO) as severe if posterior capsule was fibrotic, diffusely thickened and opaque. Modest PCO was characterized by focal fibrotic opacifications at otherwise clear posterior capsule. Severe posterior capsular fibrosis developed in 9 eyes (60%) after 2-14 weeks postoperatively (mean 8 weeks) including 3 of 6 eyes with air tamponade (50%) and 6 of 9 eyes with 20% SF6-gas tamponade (66.7%). In 6 eyes (40%) Nd:YAG-laser capsulotomy was performed 4-14 weeks postoperatively (mean 8.5 weeks). In the control group modest PCO developed in 8 eyes (53.3%) 1-13 months postoperatively (mean 6.5 months) none requiring Nd:YAG-laser capsulotomy during follow-up period. Combined cataract and vitreoretinal surgery with intraocular air/SF6-gas tamponade induces severe posterior capsular fibrosis in the early postoperative period. The capsular fibrosis is presumably caused by accumulation of fibrin and proliferation stimulating factors in the narrow space between intraocular lens and air/SF6-gas bubble."}, {"id": "pubmed23n0819_20135", "title": "[Bilateral retinal detachment and high myopia: report of nine cases].", "score": 0.009523809523809525, "content": "Bilateral retinal detachments are rare, but their occurrence increases in cases of high myopia. The objective of our research is to study their incidence, management and postoperative results. This is a study of the medical records of nine patients with high myopia and simultaneous or consecutive bilateral rhegmatogenous retinal detachment. This is a retrospective study of the medical records of nine patients (18 eyes), aged 11-38 years old, with high myopia and simultaneous or consecutive bilateral retinal detachment. All had surgery on our medical service between September 1, 2010 and September 1, 2011. Bilateral retinal detachments represented 4.11% of the total cases operated during this period (219 patients) and 9.17% of the retinal detachments with high myopia (98 patients). The sex ratio is 1 male to 8 females, with an average age of 31 years old. The detachments were simultaneously bilateral for 3 patients. The initial corrected visual acuity varied between 1/40 and 4/10, macular retinoschisis was found in one case, and the breaks found were atrophic holes and horseshoe breaks. Scleral buckling with cryotherapy was performed in all patients, with a primary reattachment rate of 88.8%; and no vitreoretinal surgery was performed. The final visual acuity varied between 1/20 and 6/10. The incidence of bilateral retinal detachment increases in cases of associated high myopia; it is observed essentially among young patients. Management is difficult because of the risk of associated vitreoretinal proliferation, and the final visual recovery depends on the type of detachment and the degree of myopia. Classical surgery performed correctly and early allows for satisfactory results in most cases."}, {"id": "wiki20220301en056_16268", "title": "Visual impairment", "score": 0.009433962264150943, "content": "The most common causes of blindness worldwide in 2010 were: Cataracts (51%) Glaucoma (8%) Age-related macular degeneration (5%) Corneal opacification (4%) Childhood blindness (4%) Refractive errors (3%) Trachoma (3%) Diabetic retinopathy (1%) Undetermined (21%) About 90% of people who are visually impaired live in the developing world. Age-related macular degeneration, glaucoma, and diabetic retinopathy are the leading causes of blindness in the developed world. Among working-age adults who are newly blind in England and Wales the most common causes in 2010 were: Hereditary retinal disorders (20.2%) Diabetic retinopathy (14.4%) Optic atrophy (14.1%) Glaucoma (5.9%) Congenital abnormalities (5.1%) Disorders of the visual cortex (4.1%) Cerebrovascular disease (3.2%) Degeneration of the macula and posterior pole (3.0%) Myopia (2.8%) Corneal disorders (2.6%) Malignant neoplasms of the brain and nervous system (1.5%) Retinal detachment (1.4%) Cataracts"}, {"id": "pubmed23n0965_21245", "title": "[Outpatient vitreoretinal surgery without next-day examination: Feasibility and acceptability].", "score": 0.009433962264150943, "content": "To assess the feasibility and acceptability of outpatient care without next-day examination for patients undergoing retinal surgery. Patients undergoing ambulatory vitreoretinal surgery between November 2013 and February 2016 at the Vienna medical center were included in this retrospective study. The age, comorbidities, indication, surgical technique and type of anesthesia used, symptoms, intraocular pressure and biomicroscopic examination data at the D0, D7 and M1 visits were recorded. Patient satisfaction with the outpatient treatment was collected by phone call in April 2016. Fifty-three surgeries on 49 patients (24 women, 25 men) with a mean age of 70 years (range, 39-91 years) were analyzed. The surgery was pars plana vitrectomy in all cases, with 26 cases of epiretinal membrane surgery, 7 vitreomacular traction syndrome, 4 vitreous hemorrhage, 6 macular hole, 4 dislocation of lens material, 5 retinal detachment and 1 macular retinoschisis in high myopia. The type of anesthesia was general in 64.1 % of cases (34) and local in 36.9 % of cases (19). Of the D0 examination data, 100 % were compatible with the patient being discharged to home. One patient consulted before the D7 exam for the occurrence of a subconjunctival hemorrhage. There were seven cases (13.2 %) of intraocular pressure elevation and two cases of vitreous hemorrhage (3.8 %) on the D7 examination data. Twenty-seven patients (55.1 %) were reached by phone and all of them were satisfied with their outpatient management. Outpatient treatment of patients without next-day examination for vitreoretinal surgery is possible and well accepted."}, {"id": "wiki20220301en059_60325", "title": "Posterior vitreous detachment", "score": 0.009347631423766573, "content": "Complications The risk of retinal detachment is the greatest in the first 6 weeks following a vitreous detachment, but can occur over 3 months after the event. The risk of retinal tears and detachment associated with vitreous detachment is higher in patients with myopic retinal degeneration, lattice degeneration, and a familial or personal history of previous retinal tears/detachment. Causes The vitreous (Latin for \"glassy\") humor is a gel which fills the eye behind the lens. Between it and the retina is the vitreous membrane. With age the vitreous humor changes, shrinking and developing pockets of liquefaction, similar to the way a gelatin dessert shrinks and detaches from the edge of a pan. At some stage the vitreous membrane may peel away from the retina. This is usually a sudden event, but it may also occur slowly over months."}, {"id": "pubmed23n0203_8283", "title": "Treatment of Candida endophthalmitis.", "score": 0.009345794392523364, "content": "A 51-year-old man who was being treated with corticosteroids for chronic extrinsic asthma developed biliary tract sepsis, candidemia, and Candida endophthalmitis with vitreous fluff-ball lesions in both eyes. Extensive vitreous fibrosis and retinal detachment with loss of useful vision occurred in his left eye, which had a vitreous biopsy. Useful vision was maintained in his right eye with two full courses of systemic amphotericin B, 5-flucytosine, and a cataract extraction. Encapsulated Candida organisms remained in the vitreous of his right eye at the time of death. Useful vision can be preserved without aggressive vitreous surgery and intravitreal anti-fungal agents in eyes with intravitreal Candida albicans."}, {"id": "pubmed23n0988_22919", "title": "Long-Term Safety and Efficacy of Limited Vitrectomy for Vision Degrading Vitreopathy Resulting from Vitreous Floaters.", "score": 0.009259259259259259, "content": "Vitreous floaters can lower visual acuity (VA) and degrade contrast sensitivity function (CSF). Limited vitrectomy improves VA and normalizes CSF, but long-term results in a large series with objective quantitative outcome measures are lacking. Case series. One hundred ninety-five eyes of 145 patients (87 men, age = 57.6 ± 4.3 years; 58 women, age = 61.5 ± 12.0 years) reporting bothersome vitreous floaters were compared to 70 age-matched controls. Posterior vitreous detachment (PVD) alone was the cause in 96/195 (49.2%), myopic vitreopathy alone was the cause in 30/195 (15.4%), PVD with myopic vitreopathy was the cause in 56/195 (28.7%), and asteroid hyalosis was the cause in 13/195 eyes (6.7%). Limited vitrectomy with 25-gauge instruments was performed without surgical PVD induction, preserving 3 to 4 mm of retrolental vitreous in phakic eyes. Follow-up averaged 32.6 ± 23.5 months (range, 3-115 months), with 2 years or more in 144 eyes, 3 years or more in 69 eyes, 4 years or more in 51 eyes, and 5 years or more in 24 eyes. Visual acuity, 39-item National Eye Institute Visual Function Questionnaire (VFQ) results, CSF (Weber index), and quantitative ultrasonography results. After surgery, vitreous echodensity decreased by 94.1% (P &lt; 0.0001) and VFQ results improved by 19.3% (P &lt; 0.0001). Preoperative VA was 0.68 ± 0.21, improving to 0.77 ± 0.19 after surgery (P &lt; 0.0001). Preoperative CSF was degraded by 91.3% compared with controls (P &lt; 0.0001), normalizing at 1, 3, 6, 12, 24, 36, and 48 months after surgery (P &lt; 0.00005 for each). There were no cases of endophthalmitis. There were 3 retinal tears and 3 retinal detachments that underwent successful repair. Clinically significant vitreous hemorrhage developed in 2 patients, clearing spontaneously. Two macular puckers and 4 recurrent floaters from new PVD were cured by re-operation. Cataract surgery occurred in 21 of 124 patients (16.9%; mean age, 64 ± 7 years; none younger than 53 years), an average of 13.1 ± 6.8 months after vitrectomy. Limited vitrectomy for Vision Degrading Vitreopathy decreases vitreous echodensity, improves patient well-being, improves VA, and normalizes CSF. The long-term efficacy and safety profiles suggest this may be a safe and effective treatment for clinically significant vitreous floaters, warranting a prospective randomized trial."}, {"id": "pubmed23n0547_24031", "title": "[The incidence of retinal tears in patients with posterior vitreous detachment].", "score": 0.009259259259259259, "content": "Posterior vitreous detachment (PVD) is a common finding in older patients, characterized by detachment of the posterior hyaloid membrane (PHM) from the retinal surface. The detachment of PHM normally occurs without complications, however, one has to be aware that retinal tear is its most common complication. The aim of the study was to determine the incidence of retinal tears in eyes with PVD. A series of 40 patients (70 eyes) with PVD were included in this retrospective study. Eyes with a history of ocular trauma, surgery or intraocular inflammation were excluded. Patient charts were reviewed to collect the following information: age, sex, profession, type and duration of symptoms, best corrected visual acuity, refractive status, prior ocular disease, coincidental retinal pathology-lattice degeneration, number, type and location of retinal tears and treatment. Statistical analysis was done with the SPSS 11.0.3 software (SPSS Inc., USA). Besides descriptive statistics, Student's t-test and chi2-test were used. Among all study eyes with PVD, 34 (48.6%) were myopic, 24 (34.3%) hypermetropic and 12 (17.1%) emetropic; statistical analysis showed a significant difference (chi2 = 10.40, df=2, p &lt; 0.01). In 6 (8.6%) eyes with PVD lattice malignant degeneration of peripheral retinal was diagnosed. Thorough examination of the fundus periphery revealed 16 (22.8%) eyes with PVD were found to have retinal tears, 11 (15.7%) had only one retinal tear and 5 (7.1%) two retinal tears. All retinal tears were treated with argon laser photocoagulation. Superotemporal eye quadrant was the most common localization of retinal tears (56.25%). These results indicate that thorough fundus periphery examination should be done in all patients with PVD because it can cause rather rarely though retinal tears that represent a potentially sight threatening condition."}, {"id": "pubmed23n1049_24556", "title": "Long-term effects of phacoemulsification and intraocular lens implantation in a patient with pathologic myopia and extremely long axial length: A case report.", "score": 0.009174311926605505, "content": "To report a rare case of phacoemulsification cataract surgery and intraocular lens implantation that improved visual acuity and capsular stability in a patient with pathologic myopia and axial length &gt;38 mm. A 51-year-old Chinese man with high myopia since childhood who had lost sight in his left eye at the age of 25 due to retinal detachment. He was referred for ophthalmological assessment due to decreased vision in the right eye, in which the best-corrected visual acuity at distance was hand motion. The patient was diagnosed with cataract, high myopia, subluxated lens, and loose zonules in the right eye. The left eyeball showed atrophy. The patient underwent uneventful phacoemulsification. An intraocular lens (Sensar AR40M) and capsular tension ring were implanted within the capsular bag. After surgery, the patient was given eye drops containing tobramycin and dexamethasone eye drops for 1 month and eye drops containing 0.1% sodium diclofenac for 2 months. There were no postoperative complications. During 1-year follow-up, uncorrected visual acuity was 20/80 and the manifest refraction was -2.50DS/-1.00DC*80, with corrected distance visual acuity of 20/60. Cataract surgery maintained adequate vision for daily living. Implantation of specific lens and capsular tension ring as well as prolonged use of non-steroidal anti-inflammatory drugs may help prevent capsular contraction and posterior capsule opacification in patients with pathologic myopia and extremely long axial length."}, {"id": "pubmed23n0135_16897", "title": "Bilateral rhegmatogenous retinal detachment.", "score": 0.009174311926605505, "content": "During a 4-year period, 1978-1981, 34 patients with bilateral rhegmatogenous retinal detachment were operated on at the University Eye Hospital in Helsinki. The incidence of bilaterality in the entire detachment population was 10%, in the aphakic group it was 16%. In 24 cases (71%) the interval from the first to the second eye detachment was less than 5 years, 6 patients (18%) had bilateral detachment simultaneously. The mean age of the patients when the first eye was affected (46 years, range 6-73) was significantly younger than the mean age of those with unilateral detachment (58 years, range 6-83) (P less than 0.001). Previous eye diseases were significantly (P less than 0.01) more common in patients with bilateral than in those with unilateral detachment, but the incidences of myopia, aphakia and lattice degeneration of the retina did not differ significantly between these groups. At least one of these predisposing factors was found in 85% and two or more of them in 53% of bilateral detachments. The retina was re-attached in 80% of the 44 eyes operated on during the study period. Of the 24 eyes operated on earlier, 71% were blind (visual acuity CF 1 m or worse). The latest visual acuities in both eyes or in the better eye for all patients were: greater than or equal to 0.5 in 38%, 0.4-0.2 in 35%, 0.1-CF2 m in 12%, and less than or equal to CF1 m in 15%."}, {"id": "wiki20220301en028_4308", "title": "Retinal detachment", "score": 0.00909767595732363, "content": "Although retinal detachment usually occurs in one eye, there is a 15% chance of developing it in the other eye, and this risk increases to 25–30% in patients who have had cataracts extracted from both eyes. Symptoms of Rhegmatogenous Retinal Detachment A retinal detachment is commonly but not always preceded by a posterior vitreous detachment which gives rise to these symptoms: flashes of light (photopsia) – very brief in the extreme peripheral (outside of center) part of vision a sudden dramatic increase in the number of floaters Sometimes a detachment may be due to atrophic retinal holes in which case it may not be preceded by photopsia or floaters. Although most posterior vitreous detachments do not progress to retinal detachments, those that do produce the following symptoms:"}, {"id": "pubmed23n0912_6575", "title": "A Case of Rhegmatogenous Retinal Detachment at Late Stage following Endogenous Bacterial Endophthalmitis.", "score": 0.00909090909090909, "content": "To report a case of rhegmatogenous retinal detachment in the late stage, despite the fact that it had previously been halted after intravitreal injection of an antimicrobial agent against endogenous bacterial endophthalmitis (EBE). This study involved a 62-year-old male who had previously been diagnosed with septicemia due to liver abscess and the detection of <iKlebsiella pneumoniae</i in a culture of his liver abscess, and who underwent ophthalmic examination after his conjunctival hyperemia had failed to improve. Visual acuity could not be measured due to his general condition being poor and his declining level of consciousness. Slit lamp examination revealed bilateral iritis and cataracts, and the fundus was invisible due to vitreous opacity. Ultrasonic B-mode examination showed subretinal abscess and exudative retinal detachment, leading to the diagnosis of EBE. Vitreous injections of antibiotics were administered to both of his eyes. His right eye became affected by phthisis bulbi, but the condition in his left eye subsided, leaving a scarred lesion near the macula. However, complete retinal detachment occurred in his left eye approximately 10 months after the vitreous injection. During vitreous surgery, proliferative membrane formation was observed in the posterior pole area, and an irregular retinal break was detected in the scar margin caused by the traction of the proliferative membrane. After vitreous surgery, the retina was reattached under silicone oil. In cases of EBE, even if the inflammation has previously subsided, strict follow-up examinations are necessary, since complications such as rhegmatogenous retinal detachment may occur at a late stage."}, {"id": "First_Aid_Step1_605", "title": "First_Aid_Step1", "score": 0.009062149198897846, "content": "Breaks more common in patients with high myopia and/or history of head trauma. Often preceded by posterior vitreous detachment (“flashes” and “floaters”) and eventual monocular loss of vision like a “curtain drawn down.” Surgical emergency. Central retinal artery Acute, painless monocular vision loss. Retina cloudy with attenuated vessels and “cherry-red” spot occlusion at fovea (center of macula) A . Evaluate for embolic source (eg, carotid artery atherosclerosis, cardiac vegetations, patent foramen ovale). Retinitis pigmentosa Inherited progressive retinal degeneration. Nyctalopia (night blindness) Ž peripheral vision loss. Bone spicule-shaped deposits A . Papilledema Optic disc swelling (usually bilateral) due to  ICP (eg, 2° to mass effect). Enlarged blind spot and elevated optic disc with blurred margins A . Leukocoria Loss (whitening) of the red reflex. Important causes in children include retinoblastoma A , congenital cataract, toxocariasis."}, {"id": "pubmed23n0672_22709", "title": "Combined vitreous and cataract surgeries in highly hyperopic eye.", "score": 0.009009009009009009, "content": "We report a case of a patient with a highly hyperopic eye who underwent cataract surgery combined with vitreous surgery to create a posterior vitreous detachment (PVD) to prevent choroidal neovascularization (CNV). A 78-year-old man noticed a decrease in his vision due to a cataract in his right eye. The patient had a severe visual loss in his left eye because of a CNV 2 years after a cataract surgery. His visual acuities were 20/30 OD and 20/600 OS, and funduscopic examination showed an orange-colored lesion OD and degenerative subretinal fibrosis OS. The posterior vitreous was attached to the retina in both eyes. The axial length was 18.9 mm OD and 19.0 mm OS. Cataract surgery combined with vitreous surgery to create PVD was performed on the right eye, and the vision improved to 20/20 with no signs of developing CNV after 5 years. We conclude that cataract surgery combined with vitreous surgery to create a PVD may prevent the development of CNV in highly hyperopic eyes."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[14, 223]], "word_ranges": [[3, 31]], "text": "the presence of thickening of the subepithelial collagen layer, more evident with Masson's trichrome (special stain that allows differentiation of the collagen fibers), is pathognomonic of collagenous colitis."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "In this case, the presence of thickening of the subepithelial collagen layer, more evident with Masson's trichrome (special stain that allows differentiation of the collagen fibers), is pathognomonic of collagenous colitis. Atrophy together with epithelial denudation are characteristic of this disease, which does not usually cause macroscopic alterations of the mucosa. An increased density of intraepithelial lymphocytes and the absence of mention of architectural alterations of the crypts (characteristic of ulcerative colitis and Crohn's disease) support this diagnosis.", "full_answer_no_ref": "In this case, the presence of thickening of the subepithelial collagen layer, more evident with Masson's trichrome (special stain that allows differentiation of the collagen fibers), is pathognomonic of collagenous colitis. Atrophy together with epithelial denudation are characteristic of this disease, which does not usually cause macroscopic alterations of the mucosa. An increased density of intraepithelial lymphocytes and the absence of mention of architectural alterations of the crypts (characteristic of ulcerative colitis and Crohn's disease) support this diagnosis.", "full_question": "A 59-year-old woman presenting with chronic watery diarrhea of 4 months' evolution. In the endoscopy, the mucosa did not show relevant aspects. In particular, no ulcers or friable areas were observed. A biopsy of the transverse colon was performed. Histopathology revealed a thickened area below the superficial lining epithelium, which was more evident by Masson's trichrome technique and involved epithelial atrophy and denudation. There was also a clear increase in intraepithelial lymphocyte density. The diagnosis of the intestinal lesion is?", "id": 279, "lang": "en", "options": {"1": "Chronic ulcerative colitis.", "2": "Pseudomembranous colitis.", "3": "Collagenous colitis.", "4": "Fibrosing Crohn's disease.", "5": NaN}, "question_id_specific": 32, "type": "PATHOLOGICAL ANATOMY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0833_17920", "title": "[Evolution of ideas on microscopic colitis].", "score": 0.017419787091491105, "content": "The literature review gives the present-day views of the definition, etiology, pathogenesis, diagnosis, and treatment of microscopic colitis (MC). In the present view, MC is an inflammatory bowel disease of unknown etiology, which is characterized by chronic watery diarrhea, no macroscopic signs of large bowel involvement in the presence of specific pathomorphological changes. There are two major forms of MC, which are similar in its clinical picture, yet, heterogeneous in histological criteria: collagenous colitis (CC) and lymphocytic colitis (LC). As of now, the prevalence of MC is about 100 cases per 100,000 population, which is similar with that in other inflammatory bowel diseases, such as ulcerative colitis and Crohn's disease. MC generally prevails in women aged over 50 years. The etiology and pathogenesis of MC have not fully investigated. Watery diarrhea is as a predominant pathognomonic symptom in all the patients with MC. The major histological criterion for the diagnosis of CC is subepithelial collagen lining thickening (more than 10 pm) and that for LC is higher intraepithelial lymphocyte counts (more than 20 intraepithelial lymphocytes/100 epitheliocytes). The topical glucocorticosteroid budesonide is currently the only agent, the efficacy of which has been proven in both inducing and maintaining remission in patients with MC in many clinical trials."}, {"id": "pubmed23n0598_13529", "title": "[Microscopic colitis - review].", "score": 0.016998166172943998, "content": "Microscopic colitis (MC) is an encompassing term for two diseases; collagenous colitis and lymphocytic colitis. The colon appears normal by colonoscopy and a diagnosis is only obtained with a biopsy. The histopathology of collagenous colitis is mainly characterized by a thickening of the subepithelial basement membrane of the colonic mucosa with a band of collagen. Lymphocytic colitis is mainly characterized by an intraepithelial lymphocytosis without the collagen thickening. Even though the two diseases have a distinctive pathology their clinical symptoms are characterized by chronic watery diarrhea without bleeding. Microscopic colitis is thought to cause about 4-13% of all chronic diarrhea but their relative frequency is much higher among older people. The mean annual incidence for collagenous and lymphocytic colitis has been increasing. Steroids are the most effective treatment for microscopic colitis and budesonide is the most studied and effective therapy for MC. The aim of this paper is to give a review of two relatively new diseases which are among the most common cause of chronic diarrhea, especially among older people."}, {"id": "pubmed23n0302_11357", "title": "[Are lymphocytic and collagenous colitis two forms of a single disease? Arguments taken from a biopsy quantitative study].", "score": 0.01662087912087912, "content": "Collagenous colitis and lymphocytic colitis are defined by a clinicopathologic syndrome with chronic watery diarrhea, microscopic lesions of colonic biopsies, and normal barium enema and colonoscopy. A histopathological study was performed on multiple colorectal biopsies to compare 12 cases of collagenous colitis (defined by a subepithelial collagen thicker than 10 microns) and 7 cases of lymphocytic colitis (defined by a number of intraepithelial lymphocytes more than 20 per 100 epithelial cells at least in one biopsied site). The study included a semiquantitative analysis of inflammatory infiltrate in the lamina propria, crypts distortion and epithelial detachment. The number of intraepithelial lymphocytes per 100 epithelial cells was determined in surface epithelium and crypts. The subepithelial collagen thickening was studied by computerised morphometry. The intraepithelial lymphocytes, villous atrophy and thickness of the subepithelial collagen were also determined in gastric and duodenal biopsies. In collagenous colitis, the subepithelial collagenous thickness ranged from 10 to 40 microns in the colon (median 20.99 microns). In 4 cases of collagenous colitis, no thickening of the collagen plate was seen in the rectum. We found constant epithelial detachment and mucosal distortion. In lymphocytic colitis, the thickness of the subepithelial collagen ranged from 6 to 10 microns in 4 cases and was less than 6 microns in 3 cases (median 6.24 microns). The median number of intraepithelial lymphocytes in surface epithelium was 22.35 (range 18.2 to 40) in lymphocytic colitis versus 12.22 (range 4.6 to 24.4) in collagenous colitis. In conclusion, we observed an overlap of both the collagenous plate thickness and the number of intraepithelial lymphocytes in collagenous colitis and lymphocytic colitis. This result favours a unified histogenesis for these two entities."}, {"id": "pubmed23n0574_19509", "title": "[Microscopic colitis: pathogenesis].", "score": 0.01630827908200171, "content": "Microscopic colitis (MC) is a chronic inflammatory process observed in colon biopsies of patients with chronic aqueous diarrhea. It is called microscopic because diagnosis is determined by histological studies since the microscopic characteristics of the colon endoscopy are normal. Two patterns exist: Lymphocytic Microscopic Colitis and Collagenous Microscopic Colitis. Etiology is unknown, and the proposed pathogenic mechanisms indicate an immunological phenomenon. Based on this, the authors of this study hypothesize that lymphocytic infiltration of the lamina propria could be related to cytotoxic lymphocytes CD8 as causative agents of colon tissue damage. Prove hypothesis of immunological pathogenesis of MC. APPARATUS AND METHODS: Thirty eight (38) patients with diagnosed MC were recruited for the last four years in the Pathology Laboratory at Ricardo Palma University. Twenty two (22) colon biopsies with the most severe histological lesions were selected. These biopsies were obtained from 17 patients: 5 patients had 2 biopsies in 2 colonoscopy sessions. Biopsies were fixed in neutral formaldehyde, processed through the paraffin inclusion method, and stained with hematoxiline-eosine and Masson trichromic to distinguish collagenous tissue. Immunohistochemistry was conducted in 4- or 5-micron-thick histological sections processed through the immunoperoxidase method. Nineteen (19) biopsies corresponded to Lymphocytic MC and 3 to Collagenous MC. Lymphocytic MC showed intraepithelial lymphocytosis, dystrophic epithelial damage in the areas of lymphocytic infiltration, lamina propria inflammation with lymphocytes and plasma cells, and normal basement membrane. Collagenous MC showed thickened basement membrane due to the presence of a collagenous band, mild to moderate intraepithelial lymphocytosis, vacuolization,and frequent detachment of protective epithelium. Twenty two (22) biopsies were positive in the immunohistochemical studies."}, {"id": "pubmed23n0600_20905", "title": "Lymphocytic, collagenous and other microscopic colitides: pathology and the relationship with idiopathic inflammatory bowel diseases.", "score": 0.016195109461016516, "content": "Collagenous colitis and lymphocytic colitis are the two major conditions characterized by chronic watery diarrhoea, without endoscopic or radiological lesions, but with histological abnormalities and therefore considered as \"microscopic colitis\". The histology of colonic biopsies shows inflammation of the mucosa, and either thickening of the subepithelial collagen band or an increase of lymphocytes in the surface epithelium. Different variant forms have been reported under separate names. These are probably not specific entities. The incidence of microscopic colitis is slightly less than the incidence of chronic idiopathic inflammatory bowel diseases (IBD). Microscopic colitis and IBD are clearly different entities. The relation between both entities is weak but double. Biopsy samples from patients with IBD may mimic the features of lymphocytic or collagenous colitis, both in the initial onset and during follow-up. In the large majority of these cases, endoscopy shows or has shown mucosal lesions. In rare cases, however, a double diagnosis was made. Certain patients, usually of older age, presented first with a microscopic, usually collagenous colitis and developed subsequently genuine ulcerative colitis."}, {"id": "pubmed23n0543_8057", "title": "Histopathological diagnosis of microscopic colitis.", "score": 0.016151332327802918, "content": "A typical symptom of microscopic colitis (MC) is chronic watery diarrhea with normal endoscopic findings and characteristic inflammatory changes in histopathology. Treatment of the disease is mainly empiric. MC has two main subtypes: lymphocytic colitis and collagenous colitis. There are also untypical histopathological forms of MC: MC with giant cells, MC not otherwise specified (NOS) and cryptal lymphocytic coloproctitis. Some other histopathological changes in MC have been observed, especially Paneth cell hyperplasia or epithelial degeneration. Eosinophilic colitis, acute colitis, amyloidosis, ulcerative colitis and Crohn's disease should be taken into consideration in differential diagnosis. The most reliable biopsy material for histopathological examination are samples obtained from transverse colon. Some studies proved that treatment of MC makes it possible to reduce not only clinical, but also histopathological, manifestations."}, {"id": "wiki20220301en070_39278", "title": "Microscopic colitis", "score": 0.015941756103684177, "content": "Diagnosis Colonoscopic appearances are normal or near normal. As the changes are often patchy, an examination limited to the rectum may miss cases of microscopic colitis, and so a full colonoscopy is necessary. Multiple colonic biopsies are taken in order to make the diagnosis. Histological features of colonic biopsies indicating microscopic colitis are: greater than 20 intraepithelial lymphocytes per 100 epithelial cells and, additionally, 10-20 μm of a thickened subepithelial collagen band in collagenous colitis. Inflammation of the lamina propria, with mainly mononuclear cells, may be observed in collagenous colitis."}, {"id": "wiki20220301en003_40015", "title": "Ulcerative colitis", "score": 0.015584415584415586, "content": "The simple clinical colitis activity index was created in 1998 and is used to assess the severity of symptoms. Endoscopic The best test for diagnosis of ulcerative colitis remains endoscopy, which is examination of the internal surface of the bowel using a flexible camera. Initially, a flexible sigmoidoscopy may be completed to establish the diagnosis. The physician may elect to limit the extent of the initial exam if severe colitis is encountered to minimize the risk of perforation of the colon. However, a complete colonoscopy with entry into the terminal ileum should be performed to rule out Crohn's disease, and assess extent and severity of disease. Endoscopic findings in ulcerative colitis include: erythema (redness of the mucosa), friability of the mucosa, superficial ulceration, and loss of the vascular appearance of the colon. When present, ulcerations may be confluent. Pseudopolyps may be observed."}, {"id": "pubmed23n0313_13912", "title": "The role of mucosal biopsy in the diagnosis of chronic diarrhea: value of multiple biopsies when colonoscopic finding is normal or nonspecific.", "score": 0.014495028780743067, "content": "There are controversies about taking routine mucosal biopsy when the gross colonoscopic finding is normal. This study was conducted to determine the frequency of clinically important histological abnormalities, prospectively, in chronic diarrhea patients with grossly normal or nonspecific colonoscopic findings. One hundred and eighteen patients suffering from nonbloody diarrhea with average frequency of more than two times a day for more than 4 weeks were included. Multiple biopsies (cecum, ascending colon, mid-transverse colon, descending colon, sigmoid colon and rectum) were taken during colonoscopic examinations and each biopsy specimen was reviewed by one pathologist after H&amp;E and Masson-trichrome staining. Clinically significant abnormalities (2 collagenous colitis, 1 lymphocytic colitis, 1 eosinophilic enterocolitis, 1 ulcerative colitis and 4 melanosis coli) were observed in 9 patients (7.6%). Sixteen cases (13.6%) of borderline histological abnormalities were observed (8 cases of possible collagenous colitis and 8 cases showing some features of lymphocytic colitis). Ninety two cases (78.8%) showed nonspecific inflammation only. Clinically important histological lesions can exist in significant percentage in spite of normal or nonspecific colonoscopic findings, which can justify routine mucosal biopsy in the evaluation of chronic diarrhea patients. The clinical significance of borderline histological abnormalities needs to be determined by careful follow-up studies."}, {"id": "pubmed23n0917_8957", "title": "Endoscopic findings and colonic perforation in microscopic colitis: A systematic review.", "score": 0.014475642934140958, "content": "Microscopic colitis (MC) is a clinical syndrome of severe watery diarrhea with few or no endoscopic abnormalities. The incidence of MC is reported similar to that of other inflammatory bowel diseases. The need for histological confirmation of MC frequently guides reimbursement health policies. With the advent of high-definition (HD) coloscopes, the incidence of reporting distinct endoscopic findings in MC has risen. This has the potential to improve timely diagnosis and cost-effective MC management and diminish the workload and costs of busy modern endoscopy units. Publications on distinct endoscopic findings in MC available until March 31st, 2017 were searched systematically (electronic and manual) in PubMed database. The following search terms/descriptors were used: collagenous colitis (CC) OR lymphocytic colitis (LC) AND endoscopy, colonoscopy, findings, macroscopic, erythema, mucosa, vasculature, scars, lacerations, fractures. An additional search for MC AND perforation was made. Eighty (n=80) articles, predominantly single case reports (n=49), were found. Overall, 1582 (1159F; 61.6±14.1 years) patients (pts) with MC and endoscopic findings were reported. The majority of articles (n=62) were on CC (pts 756; 77.5% females). We identified 16 papers comprising 779 pts (69.2% females) with LC and 7 articles describing 47 pts (72.3% females) diagnosed as MC. The youngest patient was 10 and the oldest a 97-year-old. Aside diarrhea, symptoms included abdominal pain, weight loss, bloating, flatulence, edema and others. In the study group we found 615 (38.8%) persons with macroscopic lesions in gut. Isolated linear ulcerations were identified in 7 pts (1.1%) while non-ulcerous lesions i.e. pseudomembranes, a variable degree of vasculature pruning &amp; dwindling, mucosal lacerations and abnormalities such as erythema/edema/nodularity, or surface textural alteration in 608 pts (98.1%). The location of endoscopic findings was not reported in 27 articles. The distinct endoscopic findings were described in the left (descending, sigmoid, rectum - 10/21/11 studies), right (cecum, ascending - 7/7 studies), transverse colon (n=12), as well as duodenum (n=4), and terminal ileum (n=2). In 17 (1.1%) pts colonic perforation occurred. Endoscopic findings are recognized with increased frequency in pts with MC. This could improve MC diagnosis by prompting a more extensive biopsy protocol in such cases and an earlier initiation of treatment. Procedure-related perforation has been reported in this group; therefore, cautious air insufflation is advisable when endoscopic findings are recognised."}, {"id": "wiki20220301en070_39279", "title": "Microscopic colitis", "score": 0.014150943396226415, "content": "Pathology Microscopic colitis is characterized by an increase in inflammatory cells, particularly lymphocytes, in colonic biopsies with an otherwise normal appearance and architecture of the colon. Inflammatory cells are increased both in the surface epithelium (\"intraepithelial lymphocytes\") and in the lamina propria. The key feature is more than 20 intra-epithelial lymphocytes per 100 epithelial cells. These are the principal features of lymphocytic colitis. An additional distinguishing feature of collagenous colitis is a thickened subepithelial collagen layer, which may be up to 30 micrometres thick, that occurs in addition to the features found in lymphocytic colitis. The fact that the two types of microscopic colitis share many features including epidemiology, risk factors and, response to therapy has led to the suggestion that they are actually subtypes of the same disease."}, {"id": "wiki20220301en126_13995", "title": "Lymphocytic colitis", "score": 0.014133516625139616, "content": "Lymphocytic colitis is a subtype of microscopic colitis, a condition characterized by chronic non-bloody watery diarrhea. Causes No definite cause has been determined. The peak incidence of lymphocytic colitis is in persons over age 50; the disease affects women and men equally. Some reports have implicated long-term usage of NSAIDs, proton pump inhibitors, and selective serotonin reuptake inhibitors, and other drugs. Associations with other autoimmune disorders suggests that overactive immune responses occur. Diagnosis The colonoscopy is normal but histology of the mucosal biopsy reveals an accumulation of lymphocytes in the colonic epithelium and connective tissue (lamina propria). Collagenous colitis shares this feature but additionally shows a distinctive thickening of the subepithelial collagen table."}, {"id": "wiki20220301en003_40016", "title": "Ulcerative colitis", "score": 0.013936152973586129, "content": "Ulcerative colitis is usually continuous from the rectum, with the rectum almost universally being involved. Perianal disease is rare. The degree of involvement endoscopically ranges from proctitis (rectal inflammation) to left sided colitis (extending to descending colon), to extensive colitis (extending proximal to descending colon). Histologic Biopsies of the mucosa are taken during endoscopy to confirm the diagnosis of UC and differentiate it from Crohn's disease, which is managed differently clinically. Histologic findings in ulcerative colitis includes: distortion of crypt architecture, crypt abscesses, and inflammatory cells in the mucosa (lymphocytes, plasma cells, and granulocytes). Unlike the transmural inflammation seen in Crohn's disease, the inflammation of ulcerative colitis is limited to the mucosa."}, {"id": "wiki20220301en034_9032", "title": "Colitis", "score": 0.013792040682667489, "content": "An important investigation in the assessment of colitis is biopsy. A very small piece of tissue (usually about 2mm) is removed from the bowel mucosa during endoscopy and examined under the microscope by a histopathologist. It can provide important information regarding the cause of the disease and the extent of bowel damage. Types There are many types of colitis. They are usually classified by the cause. Types of colitis include: Autoimmune Inflammatory bowel disease (IBD) – a group of chronic colitides. Ulcerative colitis (UC) – a chronic colitis that affects the large intestine. Crohn's disease (CD) – another type of IBD that often leads to colitis. Unknown Microscopic colitis – a colitis diagnosed by microscopic examination of colonic tissue; macroscopically (\"to the eye\") it appears normal. Lymphocytic colitis Collagenous colitis Treatment-caused Diversion colitis Chemical colitis Chemotherapy-induced colitis Radiation colitis Checkpoint inhibitor induced colitis"}, {"id": "wiki20220301en605_24721", "title": "Segmental colitis associated with diverticulosis", "score": 0.013730610952833176, "content": "Types There are four types of SCAD, based on endoscopic appearance. Pattern A is characterized by involvement of crescentic folds and is the most common type of SCAD (52%). Pattern B has an appearance similar to mild-to moderate ulcerative colitis (30.40%), whereas pattern C appears similar to Crohn's disease (10.90%). Pattern D is the least common, and appears similar to severe ulcerative colitis (6.50%). Diagnosis SCAD is diagnosed via colonoscopy, often incidentally during examination for unrelated concerns. Colonoscopy shows erythema of the colonic mucosa, which may be characterized by friability and exudate. The descending and sigmoid colon are typically involved. Biopsies of the affected area and the unaffected rectum confirm the diagnosis. Biopsies of SCAD show evidence of chronic inflammation. Rectal biopsies show normal mucosa."}, {"id": "wiki20220301en070_39276", "title": "Microscopic colitis", "score": 0.013390487854664217, "content": "Microscopic colitis refers to two related medical conditions which cause diarrhea: collagenous colitis and lymphocytic colitis. Both conditions are characterized by the presence of chronic non-bloody watery diarrhea, normal appearances on colonoscopy and characteristic histopathology findings of inflammatory cells. Signs and symptoms The main symptom is persistent non-bloody watery diarrhea, which may be profuse. People may also experience abdominal pain, fecal incontinence, and unintentional weight loss. Microscopic colitis is the diagnosis in around 10% of cases investigated for chronic non-bloody diarrhea."}, {"id": "pubmed23n0811_11290", "title": "Histology of microscopic colitis-review with a practical approach for pathologists.", "score": 0.013345101500441305, "content": "Microscopic colitis has emerged as a major cause of chronic watery non-bloody diarrhoea, particularly in elderly females. The term is used as an umbrella term to categorize a subgroup of colitides with distinct clinicopathological phenotypes and no significant endoscopic abnormalities. Lymphocytic colitis is defined by an increased number of surface intraepithelial lymphocytes, and collagenous colitis by a thickened collagen band underneath the surface epithelium. There is increased inflammation in the lamina propria, but only little or no crypt architectural distortion. Incomplete and variant forms showing less characteristic features have been reported under different names. The differential diagnosis mainly includes resolving infectious colitis and changes related to the intake of drugs such as non-steroidal anti-inflammatory drugs. Substantial clinical and histological overlap between lymphocytic and collagenous colitis has been described, raising the suspicion that the conditions are two histological manifestations of the same entity, possibly representing different manifestations during the disease course or different stages of disease development. In this review, we provide a practical approach for pathologists, with a focus on diagnostic criteria and differential diagnosis, and discuss recent insights into the pathogenesis of disease and the relationship with classic chronic inflammatory bowel disease, i.e. Crohn's disease and ulcerative colitis. "}, {"id": "wiki20220301en003_39991", "title": "Ulcerative colitis", "score": 0.01307605315238903, "content": "The clinical presentation of ulcerative colitis depends on the extent of the disease process. Up to 15% of individuals may have severe disease upon initial onset of symptoms. A substantial proportion (up to 45%) of people with a history of UC without any ongoing symptoms (clinical remission) have objective evidence of ongoing inflammation. Ulcerative colitis is associated with a generalized inflammatory process that can affect many parts of the body. Sometimes, these associated extra-intestinal symptoms are the initial signs of the disease. Extent of involvement In contrast to Crohn's disease, which can affect areas of the gastrointestinal tract outside of the colon, ulcerative colitis is usually confined to the colon. Inflammation in ulcerative colitis is usually continuous, typically involving the rectum, with involvement extending proximally (to sigmoid colon, ascending colon, etc). In contrast, inflammation with Crohn's disease is often patchy, with so-called \"skip lesions.\""}, {"id": "wiki20220301en023_54770", "title": "Inflammatory bowel disease", "score": 0.012995429429019715, "content": "No disease specific markers are currently known in the blood, enabling the reliable separation of Crohn's disease and ulcerative colitis patients. The way doctors can tell the difference between Crohn's disease and UC is the location and nature of the inflammatory changes. Crohn's can affect any part of the gastrointestinal tract, from mouth to anus (skip lesions), although a majority of the cases start in the terminal ileum. Ulcerative colitis, in contrast, is restricted to the colon and the rectum. Microscopically, ulcerative colitis is restricted to the mucosa (epithelial lining of the gut), while Crohn's disease affects the full thickness of the bowel wall (\"transmural lesions\"). Lastly, Crohn's disease and ulcerative colitis present with extra-intestinal manifestations (such as liver problems, arthritis, skin manifestations and eye problems) in different proportions."}, {"id": "pubmed23n0697_7483", "title": "[Microscopic colitis: histopathological review with a clinicopathological correlation].", "score": 0.012812812812812813, "content": "Microscopic colitis is a clinicopathological entity which, in addition to typical symptoms such as watery diarrhea, is characterized by its specific histopathology. Since colonoscopy yields normal findings, microscopic colitis belongs in a histological domain. The term encompasses two forms: lymphocytic and collagenous colitis. Histologically, lymphocytic colitis shows an increase in intraepithelial lymphocytes of more than 20 lymphocytes per 100 surface colonocytes, while collagenous colitis is characterized by a thickened subepithelial collagen layer of more than 10 µm. Specific stains help in the quantification of both. Since microscopic colitis does not always affect the entire colon and the number of intraepithelial lymphocytes varies physiologically, obtaining stepwise biopsies of the colon (with information on location where possible) is recommended. A thickened collagen layer is relatively specific for collagenous colitis, whereas intraepithelial lymphocytosis is also found in other diseases. Therefore, to make a correct diagnosis, it is important to correlate histological findings with clinical symptoms, including the main symptom of watery diarrhea."}, {"id": "wiki20220301en084_292", "title": "Collagenous colitis", "score": 0.012595591542959965, "content": "Collagenous colitis is an inflammatory bowel disease affecting the colon specifically with peak incidence in the 5th decade of life, affecting women more than men. Its clinical presentation involves watery diarrhea in the absence of rectal bleeding. It is often classified under the umbrella entity microscopic colitis, that it shares with a related condition, lymphocytic colitis. Signs and symptoms Microscopic colitis causes chronic watery diarrhea with greater than 10 bowel movements per day. Some patients report nocturnal diarrhea, abdominal pain, urgency, fecal incontinence, fatigue, dehydration and weight loss. Patients report a significantly diminished quality of life. Causes The cause of collagenous colitis is unknown."}, {"id": "pubmed23n0080_374", "title": "Pitfalls in the diagnosis of collagenous colitis: experience with 75 cases from a registry of collagenous colitis at the Johns Hopkins Hospital.", "score": 0.012513842746400886, "content": "Collagenous colitis is a relatively rare disorder presenting mainly in middle-aged women as watery diarrhea. Endoscopic and radiographic studies of the colon are usually normal, and diagnosis must be made by biopsy. The characteristic biopsy findings are a combination of increased mucosal inflammation (collagenous colitis) as well as subepithelial collagenous thickening. The mucosal inflammatory changes include increased lamina propria plasma cells, prominent intraepithelial lymphocytes, and in some cases, numerous eosinophils. The collagenous thickening has qualitative as well as quantitative differences from normal, and may be highlighted by Masson trichrome stains. Simply quantitating the thickness of a subepithelial collagen layer is neither adequate nor necessary for the diagnosis of collagenous colitis. Major problems in diagnosing collagenous colitis arise from focusing solely on the subepithelial region without attention to inflammatory changes. For example, tangential sectioning of normal colon results in an artifactually thickened basement membrane, and such cases have been wrongly interpreted as collagenous colitis. If biopsies lack the characteristic inflammatory pattern, a tangentially cut thick basement membrane should be ignored. The key to correct diagnosis of collagenous colitis is analyzing the summation of various inflammatory changes plus subepithelial collagenization, rather than focusing on any single feature in isolation."}, {"id": "pubmed23n0568_8742", "title": "Evolution of collagenous colitis into severe and extensive ulcerative colitis.", "score": 0.012434009447068631, "content": "Collagenous colitis is an inflammatory mucosal disorder of the colon with distinctive histopathological features, including a thickened subepithelial collagen layer. The clinical course is usually benign, but serious complications, including death, may occur. In the present report, a 69-year-old woman with watery diarrhea and collagenous colitis developed bloody diarrhea that was refractory to treatment medications, including corticosteroids and azathioprine. Endoscopic and histopathological studies showed a focal neutrophilic inflammatory process that progressed to a diffuse and extensive form of colitis, eventually requiring total proctocolectomy. Careful histological review of the resected colon showed no evidence of persistent collagenous colitis. These findings suggest an important need for continued long-term follow-up of patients with collagenous colitis because superimposed and serious colonic complications may occur, including a severe and extensive pancolitis refractory to medications and necessitating total proctocolectomy."}, {"id": "pubmed23n0511_11519", "title": "[Clinical significance of erosive and/or small ulcerative lesions in the colon and terminal ileum--short-term follow-up study].", "score": 0.012298794509093443, "content": "Various etiologies and diseases may be related to erosions and/or small ulcers without gross inflammatory changes in the surrounding mucosa found in the colon and terminal ileum during colonoscopy. However, studies on follow-up of these lesions are rare. Thus, we investigated the clinical significance of these lesions and their characteristics helpful for differential diagnosis. We reviewed the data of 183 patients with colonoscopically observed erosive or small ulcerative lesions (&lt;2 cm), and analyzed them according to the location, number, and size of lesions, histopathologic findings, chief complaints, laboratory findings, changes of symptoms, and changes in lesions during 4-12 week follow-up period. Histopathologic findings of these lesions included acute nonspecific inflammation, chronic nonspecific inflammation, Crohn's disease, tuberculous colitis, ischemic colitis, Behcet's disease, cytomegalovirus infection, eosinophilic colitis, ulcerative colitis or pseudomembranous colitis, but most of them were nonspecific (84%). In patients with nonspecific inflammation, histopathologic findings, symptoms, location and multiplicity of the lesions were not prognostic factors for the persistency of symptoms and lesions during follow-up period. Two patients with acute inflammation, who showed no improvement in symptoms and lesions, were later diagnosed as Crohn's disease. Erosive or small ulcerative lesions without macroscopic inflammatory changes in the surrounding mucosa during colonoscopy, are mainly nonspecific. However, careful follow-up is required when the symptoms and/or lesions are not improved."}, {"id": "pubmed23n0648_22118", "title": "Microscopic colitis in patients presenting with chronic diarrhea.", "score": 0.011787280701754384, "content": "To investigate the prevalence of microscopic colitis among patients presenting with chronic watery diarrhea. Colonic biopsies from 400 patients presenting with chronic watery diarrhea and other symptoms pertaining to lower gastrointestinal tract were studied. After a detailed clinical history and thorough physical examination full length colonoscopy was done using flexible colonoscope. Colonic biopsies were taken from abnormal and normal areas. Three to five micron thick sections were cut and stained with hematoxylin and eosin and Masson's trichrome stain to highlight sub epithelial collagen. Fifteen out of 400 (3.7%) colonic biopsies from patients presenting with chronic diarrhea had evidence of microscopic colitis. Five out of fifteen biopsies (33%) were diagnosed as collagenous colitis, 10 biopsies (67%) had evidence of lymphocytic colitis; 14/400(3.5%) histologically normal biopsies were taken as controls to compare various demographic and risk factors. Ten out of 15 patients (67%) were clinically diagnosed as irritable bowel syndrome. In the remaining five an infective etiology was suspected. On colonoscopy12/15 (80%) had no abnormality and 3/15 (20%) had mild hyperemia. A possibility of microscopic colitis should be considered while examining colonoscopic biopsy of a patient with chronic watery diarrhea and normal colonoscopy to avoid the misdiagnosis that may affect the treatment of patients."}, {"id": "article-19710_3", "title": "Collagenous and Lymphocytic Colitis -- Introduction", "score": 0.011695906432748537, "content": "Histologic changes define the two types of microscopic colitis. Often this presentation of symptoms will lead to evaluation for other types of inflammatory bowel disease including Crohn disease and ulcerative colitis. The incidence has been increasing throughout northern Europe and northern North America, which is more common in females. [2]"}, {"id": "wiki20220301en070_39282", "title": "Microscopic colitis", "score": 0.011665904189268675, "content": "Epidemiology Incidence and prevalence of microscopic colitis nears those of ulcerative colitis and Crohn's disease. Studies in North America found incidence rates of 7.1 per 100,000 person-years and 12.6 per 100,000 person-years for collagenous colitis for lymphocytic colitis, respectively. Prevalence has been estimated as 103 cases per 100,000 persons. People who develop microscopic colitis are characteristically, though not exclusively, middle-aged females. The average age of diagnosis is 65 but 25% of cases are diagnosed below the age of 45. History The condition of microscopic colitis was first described as such in 1982. Lymphocytic colitis was described in 1989. Collagenous colitis was recognised earlier, in 1976. References External links MayoClinic.com Colitis Steroid-responsive inflammatory conditions"}, {"id": "pubmed23n0796_5144", "title": "Microscopic colitis: Common cause of unexplained nonbloody diarrhea.", "score": 0.011566877277437902, "content": "Microscopic colitis (MC) is characterized by chronic, watery, secretory diarrhea, with a normal or near normal gross appearance of the colonic mucosa. Biopsy is diagnostic and usually reveals either lymphocytic colitis or collagenous colitis. The symptoms of collagenous colitis appear most commonly in the sixth decade. Patients report watery, nonbloody diarrhea of a chronic, intermittent or chronic recurrent course. With collagenous colitis, the major microscopic characteristic is a thickened collagen layer beneath the colonic mucosa, and with lymphocytic colitis, an increased number of intraepithelial lymphocytes. Histological workup can confirm a diagnosis of MC and distinguish the two distinct histological forms, namely, collagenous and lymphocytic colitis. Presently, both forms are diagnosed and treated in the same way; thus, the description of the two forms is not of clinical value although this may change in the future. Since microscopic colitis was first described in 1976 and only recently recognized as a common cause of diarrhea, many practicing physicians may not be aware of this entity. In this review, we outline the epidemiology, risk factors associated with MC, its etiopathogenesis, the approach to diagnosis and the management of these individuals. "}, {"id": "wiki20220301en183_34351", "title": "Pancolitis", "score": 0.011554738835059584, "content": "Pancolitis, in its most general sense, refers to inflammation of the entire colon. This can be caused by a variety of things. Pancolitis or universal colitis is frequently used in a more specific fashion to denote a very severe form of ulcerative colitis. This form of ulcerative colitis is spread throughout the entire large intestine including the right colon, the left colon, the transverse colon, descending colon, and the rectum. A diagnosis can be made using a number of techniques but the most accurate method is direct visualization via a colonoscopy. Symptoms are similar to those of ulcerative colitis but more severe and affect the entire large intestine. Patients with ulcerative colitis generally exhibit symptoms including rectal bleeding as a result of ulcers, pain in the abdominal region, inflammation in varying degrees, and diarrhea (often containing blood). Pancolitis patients exhibit these symptoms and may also experience fatigue, fever, and night sweats. Due to the loss of"}, {"id": "pubmed23n0412_7259", "title": "[Ulcerative colitis with segmental involvement].", "score": 0.011437908496732027, "content": "Ulcerative colitis is a chronic inflammatory disease affecting areas of the colon or the full length. From the endoscopic point of view, ulcerative colitis presents lesions that stretch continuously from the rectum to variable colon segments, a characteristic that is of great value when distinguishing it from Crohn's disease. Continuous involvement, without healthy patches, justifies ending endoscopic exploration once the distal end of the lesion has been reached. To retrospectively study the frequency of segmental lesions in the colonoscopies performed in patients with ulcerative colitis. Diagnosis of ulcerative colitis and proctitis was established by clinical, endoscopic, histologic, analytical, and radiological criteria. The indication and number of endoscopies was made on the basis of the clinical criteria of diagnosis, acute episodes, refractoriness or dysplasia screening. The extent of the examination also depended on clinical criteria: the severity of the episode, tolerance to colonoscopy or the degree of cleansing. A total of 155 coloscopies were performed. In 113 colonoscopies (73%) the distal end of the lesion was reached and in 70 (45%) the cecum was reached. Of the 80 patients, 27 (33%) presented ulcerative proctitis at diagnosis. Nine of the 80 patients (11.3%) biopsies were performed in healthy colonic patches, which confirmed histological normality. Six of the 9 patients were receiving no treatment. In all patients except two, the cecum was reached in one or more of the colonoscopies. The distribution of the segmental lesions varied but these were mainly found in the periappendicular region and in the cecum in 6 of the 7 patients in whom the cecum was reached. Of the 80 patients, endoscopic evidence of rectal sparing was found in 5 (6.3%); of these, 4 were receiving systemic or topical treatment. Histological analysis confirmed the absence of inflammatory lesions in these patients. The only patient who was not receiving treatment presented microscopic lesions compatible with ulcerative colitis. Endoscopic segmental lesions in ulcerative colitis were present in 11.3% of patients. Segmental lesions were most frequently found in the cecum and periappendicular region. Endoscopic and histologic evidence of rectal sparing may be the result of systemic or topical treatment."}, {"id": "InternalMed_Harrison_22298", "title": "InternalMed_Harrison", "score": 0.011355887752425912, "content": "FIGURE 345-5 Colonic polyps. A. Pedunculated colon polyp on a thick stalk covered with normal mucosa (arrow). B. Sessile rectal polyp. FIGURE 345-4 Causes of colitis. A. Chronic ulcerative colitis with diffuse ulcerations and exudates. B. Severe Crohn’s colitis with deep ulcers. C. Pseudomembranous colitis with yellow, adherent pseudo-membranes. D. Ischemic colitis with patchy mucosal edema, subepithelial hemorrhage, and cyanosis. FIGURE 345-6 Colon adenocarcinoma growing into the lumen. FIGURE 345-7 Flat serrated polyp in the cecum. A. Appearance of the lesion under conventional white-light imaging. B. Mucosal patterns and boundary of the lesion enhanced with narrow band imaging. C. Submucosal lifting of the lesion with dye (methylene blue) injection prior to resection."}, {"id": "pubmed23n0516_4491", "title": "[Microscopic colitis].", "score": 0.01121111145713307, "content": "A 38-year old woman is admitted in the gastroenterology unit for a disabling episode of watery diarrhoea, not bloody and refractory to anti-diarrheic drugs. Different diagnostic exams remain negatives, with the exception of colic biopsies who disclose a lymphocytic colitis, one of the forms of the microscopic colitis entity. Microscopic colitis is an anatomo-clinic syndrome characterized by the presence of histological abnormalities on colic biopsies amongst patients suffering from chronic watery diarrhoea without endoscopic anomalies. Two clinical entities are today well known: collagenous colitis is characterized by a thickening of the sub-epithelial collagen band of the colon; lymphocytic colitis is defined as an increased level of lymphocytic cells, more than 20%, in the epithelial surface of colorectal mucosa. Inflammatory lesions of the chorion and alterations of the epithelial surface are seen in the two types of colitis. Their etiology remains unknown. It could be due to inflammatory lesions from autoimmune origin, activated by various exogenous agents such as bacteria and drugs. Clinical (feminine prevalence, frequent association with auto-immune diseases) and morphological similarities lead to argue and to hypothesis that both entities could represent different stages of the same disease, the lymphocytic colitis being the early stage. Various treatments have been proposed, for instance 5-aminosalicylates, but today mainly synthetic corticoids are used, especially budesonide. Others entities are recently been described: the chronic pericrypt eosinophilic enterocolitis and the colonic epithelial lymphocytosis (\"epidemic\")."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 169]], "word_ranges": [[0, 26]], "text": "Since a splenectomized patient is considered a \"special\" patient, he should receive antibiotic treatment as soon as possible, even if the wound does not appear infected."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Since a splenectomized patient is considered a \"special\" patient, he should receive antibiotic treatment as soon as possible, even if the wound does not appear infected. It is necessary to make sure that the dog is yours (you should ask if the animal is correctly vaccinated) and if the patient is correctly vaccinated (5 doses of tetanus, it would not require tetanus); make a notification of Animal Aggression, for observation of this and take measures in case this is necessary.", "full_answer_no_ref": "Since a splenectomized patient is considered a \"special\" patient, he should receive antibiotic treatment as soon as possible, even if the wound does not appear infected. It is necessary to make sure that the dog is yours (you should ask if the animal is correctly vaccinated) and if the patient is correctly vaccinated (5 doses of tetanus, [HIDDEN]); make a notification of Animal Aggression, for observation of this and take measures in case this is necessary.", "full_question": "Luis is a 25-year-old young man who underwent splenectomy after a bicycle accident 1 year ago. He has a dog that bit him 24 hours ago and has caused a small wound on his right hand. He went to his health center (located 3 hours from the nearest hospital) for fever of 39ºC, pain in the wound and general malaise. On examination, BP 100/60 mm Hg, HR 110 beats per minute, slight swelling in the wound without pus. Which of the following actions is most indicated at this time?", "id": 437, "lang": "en", "options": {"1": "Send to hospital for rabies and tetanus vaccination and keep under observation.", "2": "Clean the wound and administer intramuscular nonspecific ganunaglobulin.", "3": "Give 400 mg of oral moxifloxacin and send to the hospital.", "4": "Give clindamycin 600 mg oral every 8 hours and observation.", "5": NaN}, "question_id_specific": 118, "type": "EPIDEMIOLOGY AND PREVENTIVE MEDICINE", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0917_25587", "title": "Bite Wounds Caused by a Wild Boar: A Case Report.", "score": 0.014876114477119354, "content": "A 74-year-old man was attacked by a wild boar while on his way home from his farm in the daytime in winter 2017 on the rural Izu peninsula. He did not provoke the boar; however, hunters were hunting animals in the mountains near the farm around the same time. The boar bit his left leg, and the man fell to the ground. The boar continued biting the man's left leg, and the man delivered a few kicks to the boar's face with his right leg. The boar then bit his right foot and ran away. The man was taken to a hospital, and a physical examination revealed 3 bite wounds on his left leg and right foot. The wounds were irrigated with sterilized saline and closed with sutures under local anesthesia. He received antibiotics and a tetanus toxoid booster. The next day, his wounds were found to be infected, and pus was drained from them. After these treatments, his wounds healed successfully. Animal bite wounds are frequently contaminated. Accordingly, in addition to early proper wound treatment, close observation of the wound is required for both the early detection of any signs of infection and early medical intervention, including appropriate drainage of pus and irrigation as necessary."}, {"id": "pubmed23n0647_3755", "title": "[Two severe tetanus cases infected in an urban area of Kawasaki City].", "score": 0.013377926421404684, "content": "We report two cases of severe tetanus infection. Case 1: A 73-year-old non-vaccinated man who fell in a local park developed a wound on the left little finger. The wound was debrided and a tetanus toxin shot given on day 4 following the injury. He developed trismus on day 6 requiring deep sedation and mechanical ventilation in the intensive care unit (ICU), with human anti-tetanus immune globulin (TIG) and antibiotics administered. Despite a very severe autonomic dysfunction, he recovered and was discharged mobile after 2 months of rehabilitation. Case 2: A 37-year-old woman fully vaccinated against tetanus in her childhood had apparently had booster vaccine for at least 20 years and was being treated for hyperthyroidism with thiamazole. She sustained two lacerations on the fingers of her right hand in her backyard. She noticed difficulty in opening her mouth on day 3 following the injury and was seen on day 7, for high fever and difficulty in speaking. She was diagnosed clinically as having tetanus and underwent wound debridement, and a shot of tetanus toxin, TIG, and antibiotics. On hospital admission day 2, she developed spasms and her blood pressure dropped drastically. She died the next day due to endotoxin shock caused by other bacteria. C. tetani is widely distributed in Japan, and these cases underscore the importance of maintaining adequate tetanus antibody levels through booster administration every 10 years in immune adults and appropriate post-exposure treatment with tetanus toxin and/or prophylactic TIG administration."}, {"id": "pubmed23n0751_2217", "title": "[The hospital-borne tetanus in the reference service of the Donka National Hospital in Conakry (2001-2011)].", "score": 0.009900990099009901, "content": "Become almost non-existent in the developed countries, the hospital-borne tetanus always stays of current events in our country in spite of the forensic problem which it puts. The objectives of this study were to determine prevalence of this affection, to describe its clinical picture and to determine its lethality. It is about a retrospective study of a duration of 11 years realized in the service of the infectious diseases of Conakry. Among 8649 hospitalizations from 2001 till 2012 we brought together 239 cases of tetanus (2.7%) among which 60 hospital-borne tetanus (0.7%). Men represented 73% of these cases, with a sex-ratio M/F of 2.7. The age bracket of 20-40 years was the most affected with 32 cases (53.3%). A single patient had begun his vaccinal calendar which had remained incomplete. Both national hospitals of the CHU of Conakry and private hospitals were the biggest suppliers of this hospital-borne tetanus with respectively 22 and 27 cases (36.6 and 45%). Tetanus related to IM of quinine represented 26 cases (43.3%) whereas the hernial cure was found in 16 cases (26.6%). The average duration of invasion and incubation was respectively 1.5 days and 6 days for the dead (n = 45.7%) and 2 days and 10.5 days for the survivors. Three-quarters of 60 patients died. The fight against this type of tetanus passes inevitably by an improvement of the working conditions, a strict application of the rules of asepsis and the in-service training of the medical and paramedical staff."}, {"id": "pubmed23n1059_1819", "title": "A case report: Community-acquired <i>Pseudomonas aeruginosa</i> necrotizing fasciitis in a morbidly obese diabetic young man can be fatal.", "score": 0.00980392156862745, "content": "We present a case study of a 26-year-old morbidly obese man with a three-day history of right leg pain and swelling. The swelling was associated with low grade fever. He was alert and conscious upon presentation to the hospital. His physical examination showed gross swelling of the entire right lower limb with no systemic manifestations. There was no discharge and bullae from the swelling area of the leg. He had high blood sugar and was newly diagnosed with type 2 diabetes mellitus. He was diagnosed with necrotizing fasciitis. An intravenous imipenem-cilastatin 500 mg every 6 h together with clindamycin 900 mg every 8 h was started empirically. Extensive wound debridement was performed. The swab culture obtained intraoperatively grew <iPseudomonas aeruginosa.</i He required an above knee amputation due to worsening infection despite wound debridement. Post-operatively, he developed acute kidney injury with severe metabolic acidosis, which required daily hemodialysis. However, the patient deteriorated due to septic shock with multi-organ failure, resulting in his death."}, {"id": "pubmed23n0638_18214", "title": "[Did we forget tetanus?].", "score": 0.00980392156862745, "content": "Currently, in our country (Republic of Serbia) tetanus is a rarely occurring disease, mainly affecting people older than 65 years of age. A small number of reported cases is mainly due to appropriate immunization. Therefore, each case of tetanus may be considered as failure of health care system to provide adequate immunization. A 71-year-old woman was injured in her garden. She sustained laceration in the left coccygeal region. The next day the wound was treated by a surgeon, but tetanus postexposure prophylaxis was not administrated. On the fifth day following the incident, the symptoms and signs of tetanus became apparent, and the patient died two days later. Postmortem examination revealed the wound that was not adequately treated, since there was a foreign body and a dressing inserted in the wound. Signs of acute (aerobic) infection were also present. Tetanus is a severe, potentially lethal disease that is absolutely preventable. Mistakes in immunization and surgical treatment of the wound can be considered as medical malpractice."}, {"id": "pubmed23n0807_543", "title": "The Bolognese surgeon Giuseppe Ruggi: how and why the aseptic surgery was introduced in Bologna in the middle half of the XIX century.", "score": 0.009708737864077669, "content": "The first reliable statistic data about perioperatory mortality were published in 1841 by the French Joseph-Francois Malgaigne (1806-1863): he referred to a mean mortality of 60% for amputations and this bad result was to be attributed mainly to hospital acquired diseases. The idea of \"hospital acquired disease\" although vague, included five infective nosologic entities, which at that time were diagnosed more frequently: erysipelas, tetan, pyemia, septicemia, and gangrene. Nonetheless, the suppuration with pus production was considered from most of the surgeons and doctors of that time as a necessary and unavoidable step in the process of wound healing. During the end of the eighteenth century, hospitals of the main European cities were transforming into aggregations of several wards, where the high concentration of patients created poor sanitary conditions and a consistent increase of perioperatory mortality. In 1865, Lister applied his first antiseptic dressing on the surface of an exposed fracture. These experimental attempts lead to an effective reduction of wound infections respect to the dressing with strings used previously. Lister's innovations in the field of wound treatment were based on two brand new concepts: germs causing rot were ubiquitarious and the wound infection was not a normal step in the process of wound healing. The concept of antisepsis was hardly accepted in the European surgical world: \"Of all countries, Italy is the most indifferent and uninterested in experimenting this method, which has been so favorably judged from the greatest surgical societies in Germany\". This quotation from the young surgeon Giuseppe Ruggi (1844-1925) from Bologna comes from his article where he presented his first experiences on aseptic medications started the previous year in the Surgical Department of Maggiore Hospital in Bologna. In his report, Ruggi described the adopted technique and suggested that the medication should be extended to all the surgical patients of the hospital:\"… this is needed to totally remove from the hospital all those elements of infection which grow in the wounds dressed with the old method\". The experimentation of this new dressing for the few treated cases was rigorous and concerned both the sterilization of surgical tools with the fenic acid (5%) and the shaving of the skin. Ruggi also observed that there was no correlation between the seriousness of the wound and its extension or way of healing: when \"simple\" cases that \"should heal without complication\" showed fever he often realized that \"it was often due to a medication performed without following the rules for an accurate disinfection and dressing\". Ruggi thought that the fever was connected to \"reabsorption of pyrogenic substances, which can be removed cleaning and disinfecting the wound\" in cases of wounds not accurately dressed and rarely medicated. Frequent postoperative medications of the wound were able to eliminate the fever within 2 h. Ruggi's attitude toward the fine reasoning lead him to introduce the concept of immunodeficiency related to physical deterioration: \"… patients treated for surgical disease may sometimes suffer from complications of medical conditions, which initially escape the most accurate investigations… The surgical operation could, in some cases, hold the balance of power\". The obtained results, published in 1879, appear extremely interesting. As he wrote in 1898, for the presentation of his case record of more than 1000 laparotomies, he had started \"… operating as a young surgeon without any tutor, helped only by his mind and what he could deduce from publications existing at the moment …\"."}, {"id": "pubmed23n0231_5685", "title": "Results of treatment of patients with severe tetanus. A study o 18 cases.", "score": 0.009708737864077669, "content": "From 1964 to 1980 a total of 18 patients with tetanus were treated at Tampere Central Hospital, 5 (28%) of whom died. No assurance regarding earlier immunization could be obtained in 15 cases. In 13 cases the site of infection was a small scratch on the finger, the legs or the face. The mean incubation period was 8 days. Ten patients suffered from severe tetanus and 8 from moderate tetanus. Eight patients, of whom 2 died, were treated in an ordinary ward. After the surgical intensive care unit was set up, 10 patients received treatment there. Three died. The mean duration of hospitalization was 27 days and the mean treatment period in the intensive care unit 24 days. Seven patients were asymptomatic when discharged and 6 had minor after-effects. Cardiovascular complications can be prevented by intensive care, but pulmonary embolism remains the commonest cause of death. Special attention should be paid to its prevention."}, {"id": "pubmed23n0985_15530", "title": "Influenza A with hemorrhagic shock and encephalopathy syndrome in an adult: A case report.", "score": 0.009615384615384616, "content": "Hemorrhagic shock and encephalopathy syndrome (HSES) is a type of acute encephalopathy mainly seen in infants. It is a syndrome encompassing an onset of high fever, disturbance of consciousness, convulsion, and shock that rapidly progresses to watery diarrhea and liver and renal dysfunctions. It is extremely rare in adults, and the number of reports is limited worldwide. We report the case of an adult patient with HSES, which occurred after influenza A infection. A 52-year-old man visited his family doctor 2 days after he noticed fever and was diagnosed with influenza A using an influenza rapid diagnosis kit; he underwent treatment on an outpatient basis. He was immediately hospitalized after developing fever, abdominal pain, malaise, and shock 16 hours after the commencement of the treatment. Abrupt acute brain swelling was noted 24 hours after hospitalization. The antibody titer to influenza A (H3N2) was 1:40. Computed tomography obtained 24 hours after treatment initiation confirmed acute cerebral edema and cerebral herniation. Electroencephalogram at that time showed a flat line. For the treatment of influenza A, laninamivir 150 mg was started immediately after the diagnosis by the family doctor, and 600 mg dose was given daily after hospitalization (or since 24 hours after the treatment initiation). For the management of shock, dobutamine 3 μg/kg/min and noradrenaline up to 0.2 μg/kg/min were used together with bolus infusion. The patient was declared brain dead on his 6th hospital day and he died on his 27th hospital day. Drastic courses such as that in our case with HSES can follow influenza infections even in adults."}, {"id": "pubmed23n0264_4519", "title": "[Surveillance of the course of 6030 surgical wounds].", "score": 0.009615384615384616, "content": "This report describes a 4-year prospective study of post-operative wound infections, utilizing a program of wound surveillance. Surgical wounds after 6030 operations of a general surgery service were surveyed by the authors and a specialist nurse, daily, and in the follow-up clinic for 30 days. The rates of infections showed a decline over the years of surveillance. On the first year there was a 6.37 per cent incidence of infections, and the fourth year, of 4.7 per cent. This represented an improvement in hospitalization days and expenses."}, {"id": "pubmed23n0802_9071", "title": "Delayed Presentation of DPD Deficiency in Colorectal Cancer.", "score": 0.009523809523809525, "content": "Case Study  Mr. D., a 55-year-old male, presented to the medical oncology service with a diagnosis of stage III adenocarcinoma of the sigmoid colon. He presented 7 weeks post sigmoid colectomy with lymph node resection and was initiated on adjuvant chemotherapy with CAPOX (capecitabine [Xeloda] and oxaliplatin [Eloxatin]). Standard dosing was used: oxaliplatin at 130 mg/m(2) on day 1 and capecitabine at approximately 2,000 mg/m(2)/day (rounded to the nearest 500-mg tablet size) for 14 days on and 7 days off (1 cycle = 21 days). A capped body surface area of 2.4 m2 was used, due to the patient's body habitus. Adverse Effects  Mr. D. did not report any complications of therapy during cycle 1, days 1-7, other than grade 1 diarrhea, which was amenable to diphenoxylate/atropine when taken. The next week, he reported significant malaise and fatigue associated with persistent diarrhea occurring every 30 minutes for 5 days. Mr. D. was instructed to go to the emergency room for an immediate evaluation, but he refused. Mr. D. presented to the clinic in poor condition on day 14 of cycle 1. His diarrhea had increased to grade 3 and was not controlled with either loperamide or diphenoxylate/atropine, though he was not taking his medications as directed. He had been instructed to take two 2-mg loperamide tablets after the first loose stool, followed by 1 tablet of diphenoxylate/atropine 2 hours later. He could then alternate this with loperamide every 2 hours as needed, not to exceed 8 tablets of loperamide per day. Instead, he had taken 2 tablets of loperamide after the first loose stool, but either waited 6 hours to take 1 tablet of diphenoxylate/atropine or otherwise chose not to alternate the medications at all despite continued diarrhea, depending on the day. Mr. D.'s timing in taking his supportive medications was inconsistent, and his explanations of this timing were not exact. He also reported persistent grade 3 nausea with vomiting for 5 days, which did not improve with ondansetron and prochlorperazine, though he again did not take these consistently. He was advised to alternate ondansetron and prochlorperazine every 4 hours as needed, but only took one or the other medication approximately 3 times per day. According to Mr. D., his adverse effects initially began on day 9 of cycle 1. He had lost approximately 14 kg (31 lb) during cycle 1. Clinically, he was found to have grade 2 mucositis and grade 1 hand-foot syndrome. At the time of this visit, his absolute neutrophil count was 3,000/ìL, his hemoglobin was 14.4 g/dL, his hematocrit 42.2%, and his platelet count was 139,000/ìL. His kidney function was within the normal range. Mr. D. refused hospitalization despite the primary team's recommendation. He also refused to undergo stool sampling for Clostridium difficile. He was given IV fluids along with adjustments in supportive medications, including a prescription for 10% tincture of opium. He was instructed to use 0.6 mL every 6 hours in addition to alternating loperamide with diphenoxylate/atropine as noted previously. He was advised to rinse his mouth with a baking soda solution for relief of his grade 1 mucositis, and alternation of antiemetics every 4 hours was reiterated. He was to return prior to initiation of cycle 2 for further evaluation. Worsening Symptoms  The next day, Mr. D.'s wife called the clinic to report that her husband's diarrhea continued despite the use of tincture of opium and that it was associated with hematochezia. He was also experiencing a worsening of his mucositis, with an associated swelling of the tongue. He was instructed to present to the emergency center, which he did on day 16 of cycle 1. By then, he was found to be febrile at 39.5°C. He was tachycardic, with a heart rate of 126, and he was experiencing significant abdominal pain associated with the diarrhea. The mucositis was worsening, with new odynophagia. At this time, Mr. D.'s absolute neutrophil count had dropped dramatically to 160/ìL, his hemoglobin was 13.1 g/dL, his hematocrit was 39.2%, and his platelet count was 68,000/ìL. He was admitted to the inpatient service and started on empiric antibiotics. His blood cultures remained negative during hospitalization, but stool cultures were positive for C. difficile. His antimicrobial regimen was deescalated to oral vancomycin once his stool volume decreased. He was treated with an institutional compounded mouthwash of diphenhydramine, aluminum/magnesium hydroxide, and viscous lidocaine for the mucositis, which also slowly improved. He was given a dose of growth factor. Neutropenia eventually resolved, with an absolute neutrophil count of 4,820/ìL on the day of discharge. He was discharged 26 days after initiating cycle 1, at which time his myelosuppression and mucositis were also resolved. Throughout his course, he did not report any neurotoxicity. DPD Testing  Due to his severe symptoms of neutropenia, mucositis, and diarrhea, Mr. D. was tested for dihydropyrimidine dehydrogenase (DPD) deficiency. Testing confirmed a heterozygous IVS14+IG&gt;A mutation. For this reason, all further adjuvant therapy was withheld, and he was followed on clinical surveillance only. "}, {"id": "pubmed23n0005_8109", "title": "[Comparative evaluation of risk factors in acute generalized tetanus].", "score": 0.009523809523809525, "content": "The investigations included a group of 26 patients with acute generalized tetanus, cared for in the Clinic according to a unitary method. A number of 17 prognostic, clinical and laboratory criteria were analyzed from the viewpoint of the pathogenic and clinicoevolutive incidence and significance. According to the severity, lethal course of the disease the following sequence was established in order of gravity: serum sickness, kypokaliemia, hyperpyruvicemia greater than an incubation of less 6 days, invasion within less than 24 hours, hyperlactacidemia greater than age over 60 years, persistent hypertension and tachycardia, hyperazoltemia, hyperglycemia, frequent paroxysmal contractions (before sedation) greater than late admission to hospital, associated cardiopulmonary pathology, hyponatriemia insufficient dressing of the wound and rural environment. The importance of the biological indices is emphasized, both as elements of prognosis and as orientative criteria for the treatment of the case."}, {"id": "pubmed23n0988_25316", "title": "Appropriate Prophylactic Antibiotic Use in Clean Wound Surgery Under Local Anesthesia.", "score": 0.009433962264150943, "content": "Although guidelines to prevent surgical site infections (SSIs) were published more than a decade ago, prophylactic antibiotics are still used subjectively in clinical practice. In this study, we evaluated the safety of single-dose preoperative intravenous antibiotics without postoperative antibiotics in the field of clean wound surgery performed under local anesthesia. We also surveyed the present clinical conditions for prophylactic antibiotic use in the plastic surgery departments of training hospitals in Korea. A total of 360 consecutive patients who underwent clean wound surgery under local anesthesia in an outpatient clinic from March 2018 to October 2018 were reviewed. In the study group, a single surgeon administered first-generation cephalosporins intravenously within 1 hour of skin incision and did not prescribe additional antibiotics. In the control group, 2 other surgeons prescribed oral first-generation cephalosporins postoperatively for 2 to 3 days without preoperative antibiotics. A telephone survey about perioperative antibiotic regimens was conducted at the departments of plastic surgery in training hospitals. There were 128 patients in the study group and 232 patients in the control group. There were no significant differences between the 2 groups regarding SSIs and other surgical complications. A total of 41 training hospitals answered the survey and every hospital had protocols of prescribing postoperative oral antibiotics routinely at the time of discharge with a mean duration of 3.9 days. Only 11 hospitals (26.8%) prescribed parenteral antibiotics before surgery as well as postoperative oral antibiotics. Intravenous injection of single-dose first-generation cephalosporins 1 hour before surgery without postoperative antibiotics did not increase the incidence of SSIs compared with the usual practice of giving only postoperative antibiotics prescription for 2 to 3 days in cases of clean wound surgery performed under local anesthesia. Proper antibiotic prophylaxis should be performed by surgeons in training hospitals without hesitation."}, {"id": "pubmed23n0283_14888", "title": "[Tetanus in the Murcia region: the clinico-epidemiological characteristics of 150 cases].", "score": 0.009433962264150943, "content": "150 tetanus cases registered on the region of Murcia have been retrospectively analyzed, they have been collected from the patients admitted at a Intensive Care Unit during a period of 18 years; the clinical together with the epidemiological features, as well as their variations, have been studied through out the years. The impact of a vaccination program in adults which was performed in our region during 1981 has been also evaluated in relationship with the incidence of disease and the economical cost of it. Incidence remained homogeneous until 1982, from that date on a sudden decrease on the number of cases was observed, related with the vaccination program [Period previous to the vaccination program: mean 10 cases/year, versus 5 cases/year since it was started (p &lt; 0.001)]. Regarding the epidemiological characteristics, it is remarkable the shift of the disease toward a more advanced age of onset together with a predominance on females beginning in 1978, but without reaching statically significance. More frequent route of infection is nowadays the intramuscular suppurative injection. Besides this fact the severity of the cases have been increasing (from 59% to 71%, p &lt; 0.005), which has determined that the global mortality of the disease remains almost the same (38%). Mortality has no relationship with age, but is related with being a female (p &lt; 0.05), with intramuscular injection as route of infection (p &lt; 0.025), with the clinical stage (p &lt; 0.001) and with a short incubation period (p &lt; 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0777_21657", "title": "[Snakes as pets - consequences of an exotic hobby].", "score": 0.009345794392523364, "content": "We report on a young man who presented at our emergency unit with pain and swelling of his left hand, after he had been bitten into his left middle finger by a sidewinder rattlesnake one hour ago. Local findings were a swollen left middle finger, a red-livid discoloration along his nail rim with paleness of the surrounding skin. Vital signs were stable, ECG showed sinus rhythm, laboratory parameters were normal, without signs of liver or kidney damage and without coagulopathy. Diagnosis was local tissue reaction due to a snake bite of a sidewinder rattlesnake without evidence of systemic toxic effect. Due to the absence of systemic toxic effects the patient received monitoring of his vital signs and we controlled local tissue reaction constantly and laboratory parameters every 6 hours, as recommended by the \"Giftnotrufzentrale\" (poison emergency advisory service). The patient left hospital on his own will against medical advice in the night after first laboratory control, which showed no signs of organ damage and we recommended reasessment the following morning. At that time the swelling had extended to the whole arm, furthermore large hematoma reaching up to the axilla had developed over night. Again we contacted the \"Giftnotrufzentrale\" and decided to begin the administration of an antivenom, after allergic testing. The administration was without complications, the swelling decreased constantly and since laboratory controll still showed no signs of systemic toxin effect, we could discharge the patient on day 3. Follow-up visit 6 months later showed complete and natural healing. Snake bites are altogether rare among our patients, nevertheless since possible toxin effects and its dynamics are unpredictable and can vary highly, they demand monitoring at close intervals of vitals signs, local swelling and laboratory parameters. As early as possible an advisory service, such as \"Giftnotrufzentrale\" should be contacted to acquire information on possible toxin effects and availability of antivenoms. Contact to other medical disciplines (e.g. dermatology, intensive care unit, surgery, neurology, dialysis…) should be sought, depending on the further course of toxin effects. Possible comorbidities as well as allergisation due to previous bites strongly influence the course of the disease and should be evaluated. We recommend to keep precise records on the ocurrence of systemic toxin effects, as well as on local findings (e.g. fotodocumentation, marking of erythema, measurement of swelling). Manipulation of the wound is usually ineffective and therefore not recommended, also in respect of self-endangerment. After stabilization of the patient a vaccination against tetanus, if necessary, should not be forgotten."}, {"id": "pubmed23n0261_15477", "title": "[Surveillance of the clinical course of 6,030 surgical wounds].", "score": 0.009345794392523364, "content": "This report describes a 4 year prospective study of postoperative wound infections, utilizing a program of wound surveillance. Surgical wounds after 6030 operations of a general surgery service were surveyed by the authors and a specialist nurse, daily and in the follow up clinic for 30 days. Results were reported to all the surgeons monthly. The rates of infections demonstrated a decline over the years of surveillance. In the first year there was a 6.37 percent incidence of infection and the fourth year 4.7 percent. This represents an improvement in hospitalization days and expenses."}, {"id": "pubmed23n0594_22938", "title": "Case of skin injuries due to stings by crown-of-thorns starfish (Acanthaster planci).", "score": 0.009259259259259259, "content": "A case of skin injuries due to stings by crown-of-thorns starfish, Acanthaster planci, in a 53-year-old Okinawan woman is reported. She went to a beach to gather shellfish on 8 April 2001 and fell to the ground with her left palm on a crown-of-thorns starfish that happened to be close to her. She hurried to the emergency section of our hospital. An emergency doctor sterilized the wound and administered an antibiotic, an analgesic agent and an injection of a tetanus antitoxin. He tried to remove the remaining spines from the palm with great difficulty. Because swelling and subcutaneous indurations of the left palm had persisted thereafter, oral and topical administration of corticosteroid started on 13 April. Physical examination at the dermatology section revealed approximately 10 stab wounds of the left palm with pus, subcutaneous bleeding and many abrasions around them. X-rays of the left hand showed foreign bodies, 2-10 mm in size, located on the lesions. The patient was treated with a topical injection of 2 mg triamcinolone acetonide (Kenacort-A), diluted fivefold with 1% Xylocaine, once a week. Some of the foreign body granulomatous lesions improved but pain and subcutaneous indurations persisted in most of the lesions. Because the X-ray photographs showed many remaining spines, surgical excision to remove them was performed under local anesthesia 3 months after the injury. All the symptoms improved after the operation. Scanning electron microscopic examination of the spines revealed that their tips had fragile lattice-like structures."}, {"id": "pubmed23n0899_11670", "title": "[Tetanus associated with medical treatments: about a case].", "score": 0.009259259259259259, "content": "Tetanus prophylaxis in routine wound management is a major strategy for tetanus prevention in health care settings. Failed wound management interventions leave patient dangerously exposed to this disease. We report the case of a patient with tetanus occurred after medical treatment for head injury performed in a healthcare facility without appropriate tetanus prophylaxis. This study aims to remind clinicians of the importance of prophylaxis in previously unvaccinated wounded patients or with a doubtful immune status. A 52-year-old patient who had not previously been vaccinated against tetanus was admitted to Yalgado Ouédraogo University Hospital CHU/YO with cervical pain, dysphagia, difficulty walking and opening the mouth. The patient had a personal history of deep wound on his forehead sutured without previous anti-tetanus prophylaxis approximately three weeks before. Physical examination on admission showed loose lockjaw, abdominal spasm, dysphagia, a body temperature of 36,5 °C and a scar on his forehead measuring about 7 cm. The diagnosis of generalized tetanus (stage II) infection occurring after forehead wound was retained. Treatment outcome was favorable and the patient was dismissed on September 18, 2015. The prevention of tetanus associated with an appropriate treatment requires rigorous application of aseptic techniques, systematization of antitetanus serum therapy in the management of previously unvaccinated patients or with a doubtful immune status presenting with a deep wound."}, {"id": "pubmed23n0530_1070", "title": "The gift that keeps on giving.", "score": 0.009174311926605505, "content": "Case 1: A 39-year-old man with chronic lower extremity lymphedema was admitted to the hospital with acute fever, chills, and left lower extremity pain, swelling, and erythema for the third time in as many months. Examination revealed a temperature of 39 degrees C (102.2 degrees F), and erythmatous induration on the left leg (Figure). The patient was treated with IV clindamycin and cefazolin, with clinical improvement. He was discharged with azithromycin, 500 mg daily for 3 days, done twice monthly. Case 2: A 52-year-old morbidly obese man with stasis dermatitis presented with acute lower extremity pain, swelling, and associated fever. He had been taking prophylactic antibiotics for his recurrent cellulitis for more than a decade and had significantly decreased his number of reoccurrences while on this therapy. He was admitted to the hospital, treated with IV cefazolin, and had a rapid improvement over 48 hours. He was subsequently discharged with continued suppressive antibiotic therapy."}, {"id": "pubmed23n1058_23503", "title": "[Clinical study on the application of covered vacuum sealing drainage technology to the bite of venomous snakes of Trimeresurus stejnegeri in Guangxi].", "score": 0.009174311926605505, "content": "To established the diagnostic criteria for venomous snakebite of Trimeresurus stejnegeri in Guangxi by ourselves, and explore the clinical effect and mechanism of covered vacuum sealing drainage (VSD) in the treatment for venomous snakebite of Trimeresurus stejnegeri in Guangxi. According to the Chinese emergency medicine for snakebite and the Chinese snake, the diagnostic criteria for venomous snakebite of Trimeresurus stejnegeri in Guangxi were formulated: (1) the responsible venomous snake was identified as Trimeresurus stejnegeri in Guangxi; (2) the appearance and morphology of the venomous snake described by the patient basically conformed to the characteristics of Trimeresurus stejnegeri in Guangxi; (3) clinical manifestations of hematotoxin included local swelling, severe wound pain, and subcutaneous ecchymosis in some patients; having (1) or both (2) and (3) could be diagnosed. The patients with venomous snakebite of Trimeresurus stejnegeri in Guangxi admitted to Snake Injury Treatment Base in Central and Northern Guangxi/Liuzhou Integrated Chinese and Western Medicine Snake Injury Treatment Center from January 2016 to January 2020 were enrolled. The patients were divided into the general treatment group and the covered VSD technology group, with 60 patients in each group. The general treatment group was treated with antivenom, anti-tetanus, closed injection around the wound, anti-inflammatory, magnesium sulfate gauze applied on the affected limb, symptomatic support treatment. The covered VSD technique was used in the covered VSD technology group based on the treatment options of the general treatment group. Treatment cycle of both groups were calculated from the next day of admission and lasted for 7 days. In the treatment cycle, blood was collected at 08:00 every day. The red blood cell count (RBC) and hemoglobin (Hb) were detected by automatic blood cell analyzer. The prothrombin time (PT), activated partial thromboplastin time (APTT) and fibrinogen (Fib) were detected by automatic blood coagulation analyzer, and the affected limb swelling degree and the appearance of subcutaneous ecchymosis were recorded. At different time points in the treatment cycle, the dynamic change trends of PT, APTT and Fib in the covered VSD technology group and the general treatment group were significantly different. Fib in both groups decreased on the 1-4 days, and gradually rose on the 5th day, and the lowest Fib value in the covered VSD technology group on the 4th day was higher than that in the general treatment group (g/L: 0.70±0.03 vs. 0.41±0.01, P &lt; 0.05). In the treatment cycle, PT of both groups increased in the early and middle stage, but decreased in the later stage. The peak value of PT of the covered VSD technology group on the 5th day was significantly lower than that of the general treatment group (s: 25.2±0.1 vs. 35.4±0.2, P &lt; 0.05), and the PT of the covered VSD technology group returned to the normal range on the 7th day, while the PT of the general treatment group was still abnormal. APTT in both groups increased at the beginning of the treatment cycle and gradually decreased. The peak value of APTT of the covered VSD technology group on the 3th day was lower than that in the general treatment group (s: 47.3±0.1 vs. 55.7±0.2, P &lt; 0.05), and the rate of increase and decline was also more gradual than that in the general treatment group. There was no significant difference in RBC or Hb between the two groups. With the passage of time, the degree of affected limb swelling was relieved to different degrees in both groups, and the remission degree in the covered VSD technology group was more obvious than that in the general treatment group, and there was significant difference between the two groups (χ <sup2</sup = 86.060, P = 0.000). The occurrence rate of subcutaneous ecchymosis in the covered VSD technology group was significantly lower than that in the general treatment group (13.3% vs. 40.0%, χ <sup2</sup = 10.909, P = 0.002). The application of covered VSD technology to the venomous snakebite of Trimeresurus stejnegeri in Guangxi does not aggravate the bleeding. It is beneficial to the reduction of the swelling of the affected limb, and also promotes the recovery of coagulation function, which can better control the occurrence of adverse events caused by coagulation dysfunction."}, {"id": "pubmed23n0712_25142", "title": "Traumatic hand injuries: the emergency clinician's evidence-based approach.", "score": 0.00909090909090909, "content": "At the start of your Saturday afternoon shift, you are not surprised to see that several patients are waiting to be seen for physical injuries. The first patient is a 34-year-old woman who sustained injury to her hand while skiing, 2 hours prior to her arrival. She reports falling with her hand still tethered to the pole's grip, landing on her outstretched right hand. She felt a painful snap in her right thumb, which still hurts, but otherwise she did not sustain any other trauma. Her only complaint currently is pain at the base of the right thumb. The patient is otherwise completely healthy, has no past medical or surgical history, and takes no medications. Upon examination, the affected hand appears to be surprisingly normal except for mild tenderness and swelling over the ulnar aspect of her first metacarpophalangeal joint and mildly decreased strength in her pincher grasp. X-ray reveals no fracture. You wonder if there is additional testing that should be done to evaluate this injury. You move on to a second patient, a 24-year-old man who cut his ring finger knuckle when he punched a wall 2 days ago. Physical examination reveals a small puncture wound over the IV metacarpophalangeal joint with mild swelling, erythema, warmth, and decreased range of motion secondary to pain. X-ray reveals no fracture, but there's something suspicious about this case. A third patient is a 37-year-old industrial worker whose finger contacted the stream of a high-powered grease injector. Physical examination reveals a small puncture wound over the volar proximal interphalangeal joint of his left long finger, mild tenderness to palpation over the area, and slight decreased range of motion secondary to pain. You wonder if the injury is as benign as it looks."}, {"id": "pubmed23n0787_7054", "title": "[Experience of two cases of tetanus without a clear history of trauma].", "score": 0.00909090909090909, "content": "Tetanus develops following inoculation of damaged human tissue with Clostridium tetani which transforms into a vegetative rod-shaped bacterium and produces the tetanospasmin. Usually we make a diagnosis of tetanus based on typical symptoms and history of trauma. But, when patients have no noticeable history of trauma, we have to diagnose tetanus on the basis of the clinical course and symptoms. We report herein on two cases of tetanus without a clear history of trauma. The first patient visited us with the chief complaints of pain in the neck and shoulder, and difficulty in opening the mouth. Based on these symptoms, we diagnosed tetani in the first stage and we started treatment, consisting mainly of human anti-tetanus immunoglobulin on the first day. The second patient also had typical symptoms of tetanus. However, he rejected the use of human anti-tetanus immunoglobulin on the first day. Because his symptoms worsened on the 2nd day, we insisted that he used it. On the 5th hospital day he developed partial opisthotonus of neck. However he recovered without tracheotomy or intratracheal intubation."}, {"id": "pubmed23n0641_22631", "title": "CICATRIZATION OF WOUNDS : IX. INFLUENCE ON THE HEALING OF WOUNDS OF VARIATIONS IN THE OSMOTIC TENSION OF THE DRESSING.", "score": 0.009009009009009009, "content": "In the study of the action of non-antiseptic substances on the rate of cicatrization, the chief obstacle encountered is the facility with which wounds become reinfected under an aseptic dressing. At the beginning of Experiment 1 the wound was sterile. It was subjected to flushing with distilled water for 2 hours, then to flushing with 30 per cent sodium chloride solution for another 2 hours. During that time no special precaution was taken to sterilize the wound and the dressing was left intact until the following morning. It was then found that the wound contained from 30 to 50 bacteria per field. The following day, after the wound had been subjected to the same treatment, the number of bacteria had increased to 50 and 100 per field, and as an immediate consequence the surface of the wound increased from 12 to 12.6 sq. cm. in 2 days. The wound was then dressed antiseptically and was found to be sterile 3 days later. Reinfection again took place the following day in spite of antiseptic dressing with chloramine paste 4 parts per 1,000, which was applied for 20 hours. In Experiment 2 similar results were observed. After 2 days of flushing with distilled water, the number of bacteria had increased to 50 per field. The wound was thereupon sterilized, but new reinfection ensued a few days later. Another wound on the same patient became reinfected under the same conditions after 1 day of sterile dressing. In none of the patients could the wounds be kept in a sterile condition throughout the whole experiment. It was impossible to maintain the sterility of a wound under aseptic dressing. Dakin's solution was therefore injected every 4 hours, or less often, according to the degree of infection, or chloramine paste was applied during the night. If there were 3 or 4 bacteria per field, the experiment was discontinued in order that the wound might be sterilized again. The cicatrization and bacteriological curves of Experiment 4 show that by the application of chloramine paste a wound may be maintained in an appropriately bacteriological condition for carrying out an experiment. Nevertheless, in spite of the antiseptic precautions taken, it was necessary to interrupt this experiment on two occasions, on December 13 to 15 and on December 18 to 22, in order that a complete sterilization of the wound might be effected. When the sterilization was performed as soon as the bacteria were discovered, little retardation occurred in the process of cicatrization. Moreover, the reinfection from the skin was often due to fine bacilli which have but mild retarding action on the rate of healing. The use of at least six flushings in 2 hours with Dakin's solution or of 12 hours' dressing with chloramine paste 10 parts per 1,000, was necessary to keep the wound in a condition of surgical asepsis. The action of distilled water was studied in Experiments 1, 2, and 3. In Experiment 1 the wound was subjected to flushing with distilled water first for 2 hours, then 4 hours, and later for 8 hours per day. The wound was maintained in a condition of mild infection. No marked modification, either acceleration or retardation, was noted in the rate of repair during the period that the treatment was applied. From November 21 to 25 the wound was almost clean and the observed curve remained parallel to the calculated curve, showing that distilled water did not retard the rate of healing. In Experiment 2 the wound was subjected to uninterrupted flushing with distilled water, first for 2 and 8 hours, then for 24 hours. It was continued from November 24 to 30; viz., for 112 hours out of 120, without the occurrence of any marked modification of the course of healing. The bacteriological curve showed that from November 22 to 27 inclusive the wound was kept aseptic. The slight retardation which occurred afterwards was probably brought about by the infection. In Experiment 3 the wound was subjected to flushing with distilled water, first for 2, then for 4, 6, and 8 hours, a total of 20 hours in 4 days. From November 21 to 24 the wound remained surgically aseptic. No modification in the rate of healing occurred. The action of the hypertonic sodium chloride solution was studied in a similar way. In Experiment 4 the wound was flushed at first with 40 per cent sodium chloride solution, from December 4 to 9 for 12 hours a day, and from December 10 to 13 for 24 hours a day, making a total of 144 hours out of 240 hours. At the end of this time the surface area of the wound coincided exactly with the calculated area. Owing to reinfection the experiment was suspended. From December 24 to 29 the wound was kept in contact with 50 per cent sodium chloride solution for 54 hours, and after December 30 flushing with 80 per cent solution for 24 hours a day was resorted to. The total amount of time involved in the above treatments was 174 hours with 40 per cent solution, 72 hours with 50 per cent solution, and 120 hours with 80 per cent solution. On January 1, the surface measured 11 sq. cm. and the calculated surface was 11.3 sq. cm. On January 5 the. surface observed was 10 sq. cm. and the calculated surface was 9 sq. cm. It should be noticed that on January 5 the bacteria numbered 4 per field, which might account for the difference. In Experiment 5 the wound was flushed for 24 hours every day with 50 per cent sodium chloride solution from December 11 to 18, a total of 192 hours. From December 18 to 24 the wound was dressed with agar-agar cakes containing 40 per cent sodium chloride. The concentration was raised to 50 per cent from December 24 to 27. The cicatrization curve indicates only a slight retardation of the repair which can be attributed to infection when both cicatrization and infection curves are compared. The temporary acceleration on the 13th may have been due to the influence of the dressing, but as it did not occur again an experimental error is probably the cause of the change observed in the curve. In Experiment 6 two practically identical wounds at a distance of but a few centimeters from each other were located on the right thigh of Patient 721. The areas of the wounds were respectively 40 and 33 sq. cm. One of the wounds was flushed with distilled water only. The other was subjected to the action of 40 per cent sodium chloride solution. From December 20 to 25 both wounds were in a condition of surgical asepsis. However, the cicatrization curves show that in spite of the difference of treatment the rate of healing was not modified. The rate of healing of the wounds did not therefore apparently undergo any measurable modification under the influence of distilled water or hypertonic salt solution. It is well known that the osmotic changes of the medium have a marked influence on tissues deprived of circulation. But it seems that a tissue with normal circulation is protected by it against the changes of the osmotic pressure occurring at its surface. The above experiments show that apparently the conditions of the tissues of a wound are not modified by the changes of the osmotic pressure of the dressing. The beneficial effects of hypertonic sodium chloride solution on the sterilization of wounds and on the rate of healing recently described by various surgeons are possibly an illusion due to lack of precise technique."}, {"id": "pubmed23n0334_16712", "title": "[Remarks on injuries which were the source of tetanus and were based on observations from the Clinic of Infectious Diseases in Cracow].", "score": 0.009009009009009009, "content": "From 1992 to 1996, 95 patients with tetanus were treated in the Chair and Department of Infectious Diseases in Cracow. Most of them came from rural area, and at old age (median 68 years). Small, trivial skin injuries were the most often identified portal of entry. Only few patients applied to doctor after injury for prophylaxis against tetanus. The authors emphasise that small skin injuries, which may be portal of entry for tetanus, should not be left abandoned."}, {"id": "pubmed23n1130_20911", "title": "A 24-Year-Old Man With Dyspnea and a Broken Left Femur.", "score": 0.008928571428571428, "content": "A 24-year-old White man presented with 1-day complaints of progressive shortness of breath and fever. He recently underwent an open reduction and internal fixation of a left midshaft femur fracture from a skiing accident 4 days ago. He denied chest pain, skin rashes, hemoptysis, hematemesis, melena, or surgical site bleeding. On arrival, the patient appeared in mild respiratory distress with a respiratory rate of 23 breaths/min, temperature of 37.8°C, heart rate of 97 beats/min, BP of 95/54 mm Hg, and peripheral saturation of 97% on 6-L/min nasal canula. His initial peripheral saturation on room air was 67%. Physical examination was unremarkable, except for diffuse rhonchi on chest auscultation. Chest radiograph on admission showed alveolar opacities predominantly in bilateral lower lobes. A chest CT angiography revealed no evidence for pulmonary embolism. However, there were findings of diffuse bilateral ground-glass opacities with areas of patchy consolidation and innumerous micronodules in both lungs (Fig 1). Laboratory examination was significant for a drop of hemoglobin by 3 g/dL and hematocrit level by 7% since his hospital discharge 4 days earlier. His renal function and urine analysis were normal. Venous blood gas on admission showed pH of 7.39 and Pco<sub2</sub of 43 mm Hg. Because of unexplained acute anemia, nonspecific CT chest findings and progressive dyspnea, a bronchoscopy with BAL was performed. Four aliquots of 60 mL saline solution were injected for lavage with fluid return (Fig 2). BAL fluid showed WBC count of 0.411 × 10<sup3</sup/mm<sup3</sup, RBC count of 318 × 10<sup3</sup/mm<sup3</sup, 100% fresh RBCs, 73% neutrophil, 24% lymphocytes, 1% monocytes, and 2% eosinophils. BAL fluid cytologic condition is shown in Figure 3. A full vasculitis workup by rheumatology was unremarkable. Ophthalmologic and skin examination were unrevealing."}, {"id": "pubmed23n0740_15442", "title": "[Tetanus in Poland in 2010].", "score": 0.008928571428571428, "content": "Epidemiological assessment of the incidence of tetanus in Poland in 2010 was based on the analysis of aggregate data provided by the State Sanitary Inspection and published an annual newsletter: Infectious diseases and intoxications in Poland in 2010, compiled by MP Czarkowski, E Cielebak, B Kondej, E Staszewska, Warsaw in 2011 and based on an annual newsletter: Vaccinations in Poland in 2010, compiled by MP Czarkowski, E Cielebak, B Kondej, E Staszewska, Warsaw 2011. A more accurate characterization of disease was based on the individual reports sent to the Department of Epidemiology, NIPH-NIH. In the last decade the incidence of tetanus has remained below 0.08/100 000 inhabitants. The average annual incidence was lower than in the previous decade, but within the last ten years to the annual variation is difficult to assign a distinct incidence tendency. Rather, they correspond to random fluctuations. In 2010, 16 cases were reported, including which occurred in 2009. All cases was reported as probable--diagnosis based on clinical signs and information about injuries. Overall incidence was 0.042/100 000, which was small and not significanty different from incidence in 2009 (0.05/100 000). All cases in 2010, were over 59 years of age, which is a strong expression of the trend observed for years that the disease is present in older age groups where the level of vaccination against tetanus is particularly low. Among people infected, there were three men and 13 women. Four cases ended with deaths. Three of these were women, among whom mortality was 23.1%, and one death occurred in a man, mortality 33.3%. Three deaths occurred among the 6 cases in people over 79 years of age, mortality 50%. The incubation period of the disease in 3 cases was less than 7 days, in 4 cases 8-14 days, in 4 cases 15-21 days, in 2 cases 22 days and more. Portals of entry of infection in 7 cases accounted for abrasion or laceration, in 6 cases the puncture wound in one it was bitten wound, and in 2 cases, the gates of infection remained unknown. In 3 cases involved people getting vaccinated, and 13 were not given information about the vaccination. No case has not occurred in a person with a documented vaccination. High degree of vaccination of children and adolescents against tetanus makes the tetanus, in these age groups almost completely eliminated, and the few cases occured in people in older age groups. But this is not a disease that can be eliminated from the environment and the risk of non-vaccinated people will always be in the case of injury with rupture of skin and contamination of the wound. Therefore it is important to maintain the current level of children and adolescents vaccinated against tetanus and pay attention to vaccination after injuries and adequate supply of injuries. This is particularly true of the elderly population."}, {"id": "wiki20220301en219_14536", "title": "Ludwig Rehn", "score": 0.008881799204379849, "content": "Ludwig Rehn's most famous surgery occurred on 7 September 1896. This surgery opened the field of cardiac surgery. Before this successful surgery, wounds of the heart were considered fatal. The patient, Willhelm Justus, was a 22-year-old gardener who had been discharged from the military because of an irregular heartbeat. On 7 September he was wounded by a knife and a passerby found him. He arrived at the State Hospital at 3:30 am. At the hospital, he was described as deathly pale with labored breathing and a barely palpable pulse. He had a non-bleeding 1.5cm wound in the left side of his heart. On 8 September the patient had developed a hemothorax, a collection of blood in the space between the chest wall and the lung. Orders thus far had been to apply ice bags to the wound and to apply a camphor, a pain relieving, topical cream. Willhelm had a fever of 100.76 °F and a respiratory rate of 68 breaths/min, a normal one is 12-20 breaths/min. On 9 September his pulse was weak and"}, {"id": "pubmed23n0289_132", "title": "[A 78-year-old man with young onset parkinsonism and sudden death].", "score": 0.008849557522123894, "content": "We report a right-handed 78-year-old man with early onset parkinsonism. The patient had an onset of micrographia at 23 years of the age in 1939. Seven years later he started to drag his right foot, and at 38 years of age, he walked with small steps with festination. Tremor was also present in his right hand. His daily life was independent as a otorhinolaryngologist. He visited our clinic on March 24, 1977 when he was mentally sound and showed mild parkinsonism consisting of masked face, stooped posture, small step gait, bradykinesia, and right side dominant rigidity and tremor. He showed good response to trihexyphenidyl and amantadine HCl. Two month later, he developed dyskinesia and some worsening of parkinsonism, and was admitted to our hospital for the first time. He was treated with 400 to 600 mg/day of levodopa/ carbidopa. He showed marked improvement, however, dyskinesia remained in his mouth. He was doing well until 77 years of age (June of 1993) when he developed hallucination and motor fluctuations. He was admitted again to our hospital on June 22, 1993. On admission, he was alert and appeared mentally sound. However, Hasegawa dementia scale was 18/30. Upward gaze was slightly restricted (3/5). Voice was somewhat small but no masking was noted. His posture was stooped and the gait was of small step. Dyskinesia was noted during walk. No rigidity nor tremor was noted. Deep tendon reflexes were lost but no sensory loss or motor weakness was noted. Routine laboratory studies were unremarkable. A cranial CT scan revealed only mild to moderate cortical atrophy. Motor and sensory conduction velocities were within normal limits, however, motor action potentials could not be obtained with stimulation to the right common peroneal nerve. He was treated with 600 mg/day of levodopa with carbidopa, 100 mg of amantadine HCl, 300 mg of Dops, and 25 mg of tiapride. He continued to show motor fluctuations, and was discharged on July 23, 1993. Since then his motor functions had become progressively worse with frequent falls, but he was still able to walk without support. On October 3 of 1994, he went to bed as usual. On the next morning, he was found dead in his bed at 9: 30. The patient was discussed in neurological CPC, and the chief discussant arrived at the conclusion that the patient had young-onset Parkinson's disease with Lewy bodies in the substantia nigra. Opinions were divided between Parkinson's disease and Lewy body negative young onset parkinsonism. Postmortem examination revealed obstruction of the trachea by aspirated foods, and the cause of death appeared to have been suffocation by the foods. Macroscopically, the external appearance of the brain was unremarkable except for slight frontal atrophy. The substantia nigra showed depigmentation in the lateral part, but the pigmentation of the medial part was well preserved. Upon histologic examination, the number of pigmented neurons in the dorsomedial part was well preserved. In the lateral part, pigmented neurons were well preserved in the dorsal area, however, in the ventral area, only non-pigmented neurons were seen; they appeared to be neurons in the pars reticulata. No gliosis was seen in any of the nigral areas. No Lewy bodies were seen in the remaining neurons. So-called immature neurons with rounded shape without neuromelanin could not be detected. The locus coeruleus neurons were well preserved. The putamen and the other basal ganglia structures were also intact. Slight myelin pallor was noted in the subcortical white matter, however, otherwise cerebral cortices were normal. The histology of this patient is unique in that only the ventrolateral part of the substantia nigra showed abnormal finding consisting of lack of pigmented neurons without gliosis. It is not clear whether the nigral change represents degeneration or a congenital \"hypoplasia'. To our knowledge, such a unique pathology of the substantia nigra has not been reported in the literature. Our patient ma"}, {"id": "Surgery_Schwartz_1255", "title": "Surgery_Schwartz", "score": 0.008849557522123894, "content": "among woolsorters in England in the late 1800s. The largest recent epidemic of inhalational anthrax occurred in 1979 in Sverdlovsk, Russia, after accidental release of anthrax spores from a military facility. Inhalational anthrax develops after a 1to 6-day incubation period, with nonspe-cific symptoms, including malaise, myalgia, and fever. Over a short period of time these symptoms worsen, with development of respiratory distress, chest pain, and diaphoresis. Character-istic chest roentgenographic findings include a widened medi-astinum and pleural effusions. Rapid antigen tests are under development for identification of this gram-positive rod, so a key element of establishing the diagnosis is eliciting an expo-sure history. Postexposure prophylaxis consists of administra-tion of either ciprofloxacin or doxycycline.100 If an isolate is demonstrated to be penicillin-sensitive, the patient should be switched to amoxicillin. Inhalational exposure followed by the development of symptoms"}, {"id": "pubmed23n0697_18550", "title": "An unusual cutaneous manifestation of Crohn's disease.", "score": 0.008771929824561403, "content": "A 61-year-old man with a 12-year history of quiescent Crohn's disease on mesalamine presented to his gastroenterologist in April 2009, complaining of abdominal cramping, diarrhea, and a 25-lb weight loss over 6 weeks. He did not respond to prednisone 50 mg and 6-mercaptopurine 100 mg daily. Abdominal computed tomography findings revealed diffuse submucosal edema consistent with extensive colitis. Colonoscopy demonstrated diffuse inflammation with erythema, friability, and shallow ulcerations in the rectum and colon. Biopsies were consistent with Crohn's colitis. He was admitted for infliximab infusion for his unremitting diarrhea. Five days before admission, the patient noted mild swelling and redness of the left lower eyelid, which progressed to involve the right lower eyelid with frank pus draining from both eyes. He had no visual impairment or eye pain. Two days before admission, an ophthalmologist prescribed a steroid eyedrop with no relief. He also complained of seropurulent painful skin lesions on his face and scalp, which spread to involve his upper trunk and proximal arms. On admission to the hospital, dermatology, ophthalmology, and infectious disease consultations were obtained to rule out disseminated infection before initiation of infliximab therapy. The patient was afebrile and hemodynamically stable. His oral mucosa was normal. He had prominent bilateral lower eyelid edema, erythema, and superficial erosions with hemorrhagic crusting and frank green purulent drainage from both eyes, with crusting along the lower lash line and bilateral sclera injection (Figure 1). On his scalp, face, trunk, and proximal extremities, he had 25 to 30 erythematous, 4- to 8-mm papulopustules with narrow red halos, some with central necrosis and crusting (Figure 2). Cultures from the purulent ocular drainage and pustules on the trunk and arms were all negative for bacteria, virus, and fungi. Gram stain from the eye drainage showed polymorphonuclear leukocytes without organisms. Tissue cultures were negative for bacterial, fungal, and mycobacterial infection. Skin biopsy taken from the central upper back demonstrated subcorneal pustules with areas of eroded epidermis and collections of neutrophils in the superficial dermis (Figure 3). Special stains were negative for organisms. He received infliximab infusion 5 mg/kg for a total dose of 420 mg over 2 hours. Within 48 hours of infusion, there was notable decrease in size of lesions, in addition to reduction of purulent drainage from both eyes. The patient was discharged home following infliximab infusion. His skin lesions resolved during a period of 2 weeks, leaving small pink atrophic scars. He received his second infusion of infliximab 2 weeks after discharge with continued improvement in his gastrointestinal symptoms."}, {"id": "pubmed23n0732_1705", "title": "[Not Available].", "score": 0.008771929824561403, "content": "D Dosseh Ékoué, A Doleaglenou, Y-K Fortey, A-E Ayite Objective: to see whether there was a difference in therisk of local infection for surgical wounds in a tropical settingdepending on whether a wound was dressed or left open beyond 48hours post-operatively. Over a four month period, 102 patients undergoingintra-abdominal surgery classified as clean or clean-contaminatedwere randomized into two equal groups. The \"with dressing\" groupunderwent a wound dressing change and re-application every two days.In the \"without dressing\" group, the wound wasleft open to the air after a first dressing change at 48 hours. There was no difference in post-operative temperaturecurve; post-operative wound infection rate was 2% in eachgroup. Suture removal was performed two days earlier in the \"withoutdressing\" group and hospital stay was decreased by twodays. The expense of repeated dressing changes was also lessened. There is no benefit to leaving a wound dressingin place longer than 48 hours after surgery; costs related to prolongedhospitalization and expenses of dressing changes are decreased bya policy of leaving incisions undressed after 48 hours."}, {"id": "pubmed23n0973_5759", "title": "Cervicofacial necrotising fasciitis by clindamycin-resistant and methicillin-resistant <i>Staphylococcus aureus</i> (MRSA) in a young healthy man.", "score": 0.008695652173913044, "content": "An otherwise healthy 24-year-old man presented with 1 week of fever, facial pain and swelling. He initially sought care at an outside hospital, where he was diagnosed with folliculitis and sent home with oral antibiotics. On arrival at our institution, CT neck was ordered, which demonstrated diffuse submental phlegmon, prompting incision and drainage. After initial improvement, the patient experienced high fevers and increased swelling just 12 hours later. The decision was made to take the patient for operative exploration, and wide debridement was performed due to suspicion for necrotising fasciitis intraoperatively that was ultimately confirmed on final pathology. Final speciation of intraoperative culture demonstrated a clindamycin-resistant and methicillin-resistant strain of <iStaphylococcus aureus</i The patient was managed with intravenous antibiotics, additional debridement and careful wound care. Delayed partial closure of wound was eventually performed once patient showed marked and persistent clinical improvement. The patient was discharged on hospital day 12 with close follow-up."}, {"id": "pubmed23n1130_8439", "title": "[A study about the epidemiological characteristics of rabies of the cases of medical treatment from a certain hospital in Beijing from 2011 to 2020].", "score": 0.008695652173913044, "content": "From 2011 to 2020, there were 111 213 cases of rabies exposed people recruited from the rabies immunization clinic of a hospital in Beijing. The monthly distribution of patients in each year was not statistically significant (<iP</i&gt;0.05). The distribution of patients showed remarkable seasonality, with the exposure peak from May to October. The ratio of male to female was 1∶1.3. The majority of patients were aged 20-29 years old (39.1%) and in-service personnel (56.5%). Level-Ⅱ wounds (84.2%) were more common than level-Ⅲ wounds (14.9%). The number of visits to level-Ⅲwounds increased rapidly since 2017. The most common injured body part was hand (60.7%). Dogs were the most common animal for injuries (60.6%), followed by cats (32.3%), of which most were host animals (75.5%). The vaccination rate from 2016 to 2020 [49.8% (24 276/48 703)] was significantly higher than that from 2011 to 2015[18.6% (6 559/35 272)](<iχ²=</i8597.18, <iP</i&lt;0.001)."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[169, 253]], "word_ranges": [[26, 39]], "text": "Endometrial cytology is performed blindly and has a large number of false negatives."}, "3": {"exist": true, "char_ranges": [[25, 168]], "word_ranges": [[5, 26]], "text": "The study that gives us more information about endometrial pathology is hysteroscopy, which allows us to perform a directed endometrial biopsy."}, "4": {"exist": true, "char_ranges": [[254, 299]], "word_ranges": [[39, 47]], "text": "MRI will help us to stage endometrial cancer."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The correct answer is 3. The study that gives us more information about endometrial pathology is hysteroscopy, which allows us to perform a directed endometrial biopsy. Endometrial cytology is performed blindly and has a large number of false negatives. MRI will help us to stage endometrial cancer.", "full_answer_no_ref": "The [HIDDEN] The study that gives us more information about endometrial pathology is hysteroscopy, which allows us to perform a directed endometrial biopsy. Endometrial cytology is performed blindly and has a large number of false negatives. MRI will help us to stage endometrial cancer.", "full_question": "A 67-year-old patient with a history of menopause at 55 years of age, 3 pregnancies with 3 euthyroid deliveries, type 2 diabetes of 6 years of evolution, treatment with nifedipone for hypertension. She consulted for intermittent scanty metrorrhagia of 2 months of evolution. The gynecological examination showed external genitalia without lesions, an atrophic cervix, a normal uterus and appendages on palpation and a normal cytological study of the cervix. The transvaginal ultrasound study shows a 7 mm hyperechogenic endometrium. Which of the following tests is the most appropriate and most sensitive to establish a diagnosis?", "id": 112, "lang": "en", "options": {"1": "Conization of the cervix.", "2": "Endometrial cytology.", "3": "Hysteroscopy and endometrial biopsy.", "4": "Magnetic resonance imaging of the pelvis.", "5": "Examination under anesthesia of the genital tract and biopsy of the cervix and endometrium."}, "question_id_specific": 154, "type": "GYNECOLOGY AND OBSTETRICS", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0363_22476", "title": "[Guideline for the diagnosis of postmenopausal bleeding. PMPB Working Group of the SGGG].", "score": 0.015874015748031496, "content": "2.1. History and clinical-gynecological investigation including a Pap smear are the first step in the clarification. The history should make sure if there is in fact bleeding from the genital and not from the urological or the intestinal region. Drug intake should be recorded, and risk factors for the development of endometrial carcinoma should be considered. This will not affect further investigation. The clinical-gynecological investigation should prove the source of postmenopausal bleeding according to the anatomical site--uterine, infra-, or suprauterine. The causes of infrauterine bleeding may easily be diagnosed by means of inspection of the external genitalia and further by using a speculum. The causes of uterine bleeding are of major importance. Cytology and colposcopy, supported by bimanual investigation, exclude cervical carcinoma as a cause of bleeding. Atypical endometrial cells on the cytological smear arouse suspicion of endometrial carcinoma. 2.2. Transvaginal sonography (TVS) is the next step if the above-mentioned investigations are negative. Both adnexa should always be investigated and the findings sonographically documented, so that solid cystic masses in the adnexal area can be better identified as suprauterine causes of postmenopausal bleeding. Then the uterus should be investigated. Further procedures are decided from the results of measurement of the longitudinal section of the endometrium at the level of maximum endometrial thickness. If the endometrial thickness is _&lt;4 mm, an observant attitude can be assumed. After 3 months the patient should be controlled against using TVS. If bleeding recurs or the endometrial thickness is &gt;4 mm on TVS, the procedure given in subparagraph 2.3 should be followed. In case the endometrial thickness is &gt;4mm or not measurable, a histomorphological investigation according to subparagraph 2.3 should be performed. In such cases, saline infusion sonohysterography(SIS) is useful as a simple method to supplement TVS. It can aid in the decision making as to which further, more invasive measures should be taken (endometrial biopsy/hysteroscopic resection). Computerized tomography or magnetic resonance imaging are, as a rule, not indicated in patients with postmenopausal bleeding. 2.3. A definite diagnosis is possible only on the basis of a histological investigation. If TVS or SS show evidence of a polypoid state, removal under hysteroscopic control is the diagnostic method of choice. In cases of symmetrical or asymmetrical thickening of the endometrium on SIS, a less invasive biopsy may be sufficient. If the biopsy specimen does not yield representative diagnostic material, one should proceed as described above. A fractionated curettage should as a rule not be performed solely, but in combination with hysteroscopy."}, {"id": "pubmed23n0363_9360", "title": "[Transvaginal sonography of the postmenopausal endometrium].", "score": 0.014516430171769977, "content": "The most frequent symptom suggesting endometrial pathology is uterine bleeding. Each postmenopausal uterine bleeding requires fraction explorative curettage and histopathologic examination of the material obtained from the cervical canal and uterine cavity. The aim of this study was to estimate the efficacy of ultrasonography as a non-invasive method in detection of endometrial pathology in postmenopausal women, and to find out whether its more frequent use could safely decrease the number of curettages in detection of these conditions. A prospective investigation has been performed in postmenopausal women not menstruating for more than a year, and who reported to the Department of Obstetrics and Gynaecology in Novi Sad for uterine bleeding during 1996 and 1997. Each woman underwent ultrasonographic examination by vaginal probe of 5 MHz and fraction curettage, whereas the samples from cervical canal (if obtained) and from uterine cavity were sent to histopathologic examination to the Institute of Pathology in Novi Sad. Standard statistical methods have been used for the analysis of the results. Validity of the applied ultrasonographic method in detecting endometrial pathology has been estimated by calculation of its sensitivity and specificity. A satisfactory visualisation of the endometrium has been obtained in all 35 examined cases. The thinnest endometrium was 1 mm wide and the thickest one was--25 mm. The fraction curettage has been used to obtain material from the cervix in 2 cases and from the uterine cavity in 35 cases. In our patients with uterine bleeding, who were in the postmenopause for 13 years on average, endometrial atrophy was recorded in 17.14%, endometrial polyp in 11.43%, endometrial hyperplasia in 22.86%, endometrial adenocarcinoma in 42.86% and uterine sarcoma in 5.71%. There were 2 false negative ultrasonographic findings (2 cases of endometrial hyperplasia) and sonographic thickness less than standard versus 2 false positive cases (endometrium thicker than the limit value, 7 and 14 mm, with no real pathology) which showed that the sensitivity of the investigated method was 93.10% and the specificity was 66.66%, if the limit value for the thickness of endometrium was 3 mm. Ultrasonographic thickness of endometrium means maximum double thickness in longitudinal plane, i.e. the distance of the opposite bordering surfaces of endometrium and myometrium. The procedure of measurements of endometrial thickness is simple in most cases. Most authors agree that there is a positive correlation between the thickness of endometrium and its pathological conditions. The most often used limit values are 3 and 4 mm. Higher limit values of endometrial thickness increase the sensitivity of the method even to 100%, but negatively affect its specificity making this method inadequate as a screening method for endometrial carcinoma. Many authors insist on introducing other criteria for evaluation of the endometrium i.e. for taking its thickness as the only criterion. Ultrasonography does not provide a completely safe differentiation between benign hyperplasia and endometrial carcinoma. The transvaginal sonography is an efficient and acceptable, noninvasive method for early detection of endometrial pathology in postmenopausal women. The thickened endometrium during menopause is the most significant ultrasonographic criterion implicating its pathology. The vaginosonographically measured thickness of 3 mm and less, gives a relatively safe prediction of endometrial atrophy, whereas the thickness above 3 mm requires explorative curettage and histopathologic examination of the endometrium, no matter if the woman has or has not uterine bleeding. (ABSTRACT TRUNCATED)"}, {"id": "pubmed23n0039_4419", "title": "[Review of cervical smears in a gynaecologic department after a period of ten years under the consideration of colposcopic findings(author's transl)].", "score": 0.013284101519395637, "content": "The study comprises 96042 cytologic cases within a period of 10 years. After screening women were admitted for biopsies. The histologic findings are reported. By 690 \"right positive smears\" 71 squamous cell cervical cancers stage Ib or more 87 microcarcinomata-1, 377 carcinomata-in-situ of the cervix, 100 dysplasias, 47 adenocarcinomata and 8 different malign tumors were found. 61 cases having histologic diagnosis outside are just mentioned. 629 cases which had been diagnosed in our hospital are described extensively. The first biopsy was nearly always taken by selective scraping of the ecto- and endocervix. Technical improvements of this method are explained. The efficiency of cytodiagnosis is especially pointed out by separating cases which could have been recognized or suspected by means of inspection or colposcopy only. 140 out of 282 carcinomata-in-situ and 7 invasive, mainly endocervical cancers (Ib-III), could only be diagnosed by a smear. In 17 woemn who turned out to have invasive cancer (Ib -III) a positive smear was the only reason for admission. Careful inspection of the cervix in the hospital, however, was sufficent to reveal the correct diagnosis. In our material the cervical smear could be of little help in cancer diagnosis of the upper genital tract such as adenocarcinoma of the corpus uteri, sarcoma and ovarian cancer. 39 of the 45 women with endometrial cancer cells in the specimen had bleeding anomalies, especially postmenopausal. The number of \"false negative smears\" mainly yields from histologic examination of 2415 uteri after hysterectomy and 4497 specimen after curettage of cervix and corpus uteri. In the first group 1 microcarcinoma-2 and 5 carcinomata-in-situ, in the second group which obviously is less representative 4 carcinomata-in-situ were found unexpectedly. It is also searched for cases which had a negative smear first and a positive or suspect smear later. This happened in 41 patients. The underlying lesion were 5 advanced cancers, 2 microcarcinomata-3 and 34 carcinomata-in-situ. Up to 30 months elapsed between the last negative cytologic finding and histologic diagnosis. 121 out of 811 suspect or positive smears were \"false positive\". Cytologic grouping III, IV and V inconsistently matched with the corresponding histologic results. The type and extension of squamous cell atypias are anticipated with little certainty. The great number of suspect specimen (group III) both in microcarcinoma-4 (6 in 78) and carcinoma-in-situ (33 in 344) was striking. Therefore we consider a histologic diagnosis to be necessary in this group as well as in group IV and V. The method of fractioned cervical scraping makes the decision of hospital admission easier. Low risk for the patient does not imply any loss in diagnostic security."}, {"id": "wiki20220301en002_155454", "title": "Obstetrics and gynaecology", "score": 0.013051090243514245, "content": "Colposcopy: If the results of a cervical cancer screening test, such as Pap smear or HPV test, are abnormal this more thorough examination of the cervix and vaginal tissues may be needed. Loop electrical excision procedure (LEEP): a procedure to quickly remove abnormal vaginal tissue within the cervix. A local anesthetic and a solution to enhance the points of removal visually is administered during the process. There is a chance of experiencing watery, pinkish discharge, brownish discharge, and mild cramping. Endometrial biopsy: a procedure that collects a tissue sample from the endometrium lining of the uterus. The sample is tested and checked under a microscope for abnormals cells or indicators of cancer. IUD insertion: an intrauterine device that is T-shaped and is placed in the uterus through the cervix. It is a reversible contraceptive that can be done in a doctor's office."}, {"id": "wiki20220301en024_103785", "title": "Heavy menstrual bleeding", "score": 0.012398373983739837, "content": "Diagnosis The NICE guidelines states that: \"Many women presenting to primary care with symptoms of HMB can be offered treatment without the need for further examination or investigation. However, investigation via a diagnostic technique might be warranted for women for whom history or examination suggests a structural or endometrial pathology or for whom the initial treatment has failed.\" Diagnosis is largely achieved by obtaining a complete medical history followed by physical exam and vaginal ultrasonography. If need be, laboratory tests or hysteroscopy may be used. The following are a list of diagnostic procedures that medical professionals may use to identify the cause of the abnormal uterine bleeding. Pelvic and rectal examination to ensure that bleeding is not from lower reproductive tract (i.e. vagina, cervix) or rectum Pap smear to rule out cervical neoplasia Pelvic ultrasound scan is the first line diagnostic tool for identifying structural abnormalities."}, {"id": "wiki20220301en017_58093", "title": "Endometrial cancer", "score": 0.011984982672314208, "content": "Transvaginal ultrasound to examine the endometrial thickness in women with postmenopausal bleeding is increasingly being used to aid in the diagnosis of endometrial cancer in the United States. In the United Kingdom, both an endometrial biopsy and a transvaginal ultrasound used in conjunction are the standard of care for diagnosing endometrial cancer. The homogeneity of the tissue visible on transvaginal ultrasound can help to indicate whether the thickness is cancerous. Ultrasound findings alone are not conclusive in cases of endometrial cancer, so another screening method (for example endometrial biopsy) must be used in conjunction. Other imaging studies are of limited use. CT scans are used for preoperative imaging of tumors that appear advanced on physical exam or have a high-risk subtype (at high risk of metastasis). They can also be used to investigate extrapelvic disease. An MRI can be of some use in determining if the cancer has spread to the cervix or if it is an endocervical"}, {"id": "wiki20220301en168_15949", "title": "Endometrial biopsy", "score": 0.01188295831152974, "content": "Procedure Generally, an endometrial biopsy follows this process: The patient is asked to lie on the table with her feet in the stirrups for a pelvic examination. She may or may not be given localized anesthesia. A speculum will be inserted into the vagina to spread the walls of the vagina apart to expose the cervix. The cervix will then be cleansed with an antiseptic solution. A tenaculum, a type of forceps, will hold the cervix steady for the biopsy. The biopsy curette will be inserted into the uterine fundus and with a scraping and rotating motion some tissue will be removed. The removed tissue will be placed in formalin or equivalent for preservation. The tissue will be sent to a laboratory, where it will be processed and tested. It will then be read microscopically by a pathologist who will provide a histologic diagnosis."}, {"id": "pubmed23n0410_14083", "title": "[Use of hysteroscopy, ultrasonography and selected hormonal tests for diagnosis of hyperplastic endometrial changes].", "score": 0.011554071746530505, "content": "1. Diagnostic value of ultrasonic endometrium thickness measurement and estimation of ultrasonic endometrium qualitative features in detecting of pathological changes at women in perimenopausal period. 2. Estimation of comparative estrogens and testosterone level in diagnostic of endometrial changes at women in postmenopausal period. 3. Qualification usefulness of analysis of macroscopic hysteroscopy uterine cavity image in detecting of pathological changes at women in perimenopausal period. 4. Comparative analysis of fractional curettage and hysteroscopy as a method of receiving material from uterus to histological estimation at women in perimenopausal period. Material to investigations consist of 242 patients in age 40-86 years, which were divided into groups in dependence from obtained diagnosis on the ground histological estimation sampling received from uterus (BZ--\"without changes\", M--submucosus myomas, P--endometrial polyps, H--endometrial hyperplasia, CA--endometrial carcinoma). In all of women transvaginal ultrasonic estimation of endometrium (TVS) and fractional curettage were executed. In 160 from them also hysteroscopy with endometrial biopsy was performed. In 53 women in postmenopausal period not using of hormonal replacement therapy one marked concentration estron (E1), estradiol (E2) and testosterone (T) in serum of blood. TVS were performed using a transducer with an emission frequency of 7.0 MHz (B &amp; K Medical 3535). Serum concentration of testosterone and estradiol were executed with EIA assay, however concentration of estron with RIA assays. To endoscopic investigations diagnostic and operative hysteroscops and resectoscope were used. Average thickness of endometrium in group CA, H and P (properly 8.96 and 6.09 and 5.02 mm) showed essential differences in comparison with group BZ (3.38 mm, Tab. 1). In group CA and H greatest cumulation of abnormal features of TVS image (endometrial thickness &gt; 2.5 mm, hyperechoic, heterogeneous and containing cystic spaces endometrium, deformation of middle echo, indistinctness of endo-myometrial border, presence of focal changes in structure of endometrium and presence of fluid in uterine cavity &gt; 1 cm3) was ascertained (Tab. 2, 3, 4). Hysteroscopic macroscopic image showed conformity with histological estimation in groups BZ, M, P, H and CA properly in 78.35, 93.75, 88.33, 22.22 and 88.33% (Tab. 7). On base of histological estimation 159 (65.7% of chances) of pathological changes (P-68, CA-37, M-32, H-22 chances) were detected (Tab. 8). Among them were 41 of asymptomatic chances (P-24, M-7, H-6, CA-4 chances). In hysteroscopy, all but one chance of pathological changes were detected, however curettage skipped most of focal changes. 1. Ultrasonic measurement of endometrial thickness is sensitive index in detecting cancer and pathological endometrial hyperplasia, instead possesses smaller value in detecting of endometrial polyps and endometrial myomas. 2. Connection of measurement of endometrial thickness with estimation of qualitative features of endometrial and uterine cavity TVS image improves results of detecting all of types intrauterine pathology. 3. Dependence between estrogens level and with thickness of endometrium was confirmed. Practical value of hormonal estimations in diagnostics of endometrial pathological changes is however not large. 4. Analysis of hysteroscopic picture is characterised high agreement with histological investigation with reference to endometrial cancer, endometrial polyps and submucosus myomas. 5. Hysteroskopy with direct biopsy possesses superiority over curettage in detecting all of types of intrauterine pathology, in particular focal changes. 6. Hysteroscopy should determine method of choice at women with recurrent bleedings from uterus, which with curettage pathological changes did not detect."}, {"id": "wiki20220301en017_58091", "title": "Endometrial cancer", "score": 0.010163176759102055, "content": "Examination Routine screening of asymptomatic people is not indicated since the disease is highly curable in its early, symptomatic stages. Instead, women, particularly menopausal women, should be aware of the symptoms and risk factors of endometrial cancer. A cervical screening test, such as a Pap smear, is not a useful diagnostic tool for endometrial cancer because the smear will be normal 50% of the time. A Pap smear can detect disease that has spread to the cervix. Results from a pelvic examination are frequently normal, especially in the early stages of disease. Changes in the size, shape or consistency of the uterus or its surrounding, supporting structures may exist when the disease is more advanced. Cervical stenosis, the narrowing of the cervical opening, is a sign of endometrial cancer when pus or blood is found collected in the uterus (pyometra or hematometra)."}, {"id": "wiki20220301en166_28865", "title": "Pelvic examination", "score": 0.010057333970377448, "content": "The pelvic exam during pregnancy is similar to the exam for non-pregnant women. One difference is that more attention is give to the uterus and cervix. The growth of the uterus is measured each visit, although this does not require a pelvic exam. As the due date approaches, the assessment of the cervix will indicate whether labor has begun or is progressing. Much time is spent determining the health of the fetus. A normal finding during the exam on the pregnant patient is that the cervix has a bluish tinge in early pregnancy. If a bluish tinge is observed in non-pregnant women, this is a sign of hypoxia. See also Well-woman examination Pap test Trauma-Informed Care References Female genital procedures"}, {"id": "pubmed23n1137_3792", "title": "Superficial spreading cervical squamous cell carcinoma in situ involving the endometrium: a case report and review of the literature.", "score": 0.009900990099009901, "content": "The spread of cervical squamous cell carcinoma to the inner surface of the uterus with replacement of the endometrium is rare. Continuity of the lesion must be demonstrated to confirm superficial spread and rule out concomitant endometrial cancer. We present the case of a 66-year-old white woman with superficial spreading squamous cell carcinoma of the cervix that involved the endometrium. Her relevant past history included conization of the cervix to treat cervical intraepithelial neoplasia III with positive margins. She subsequently had three negative cervical vaginal cytology results, each with a positive high-risk human papillomavirus test. Transvaginal ultrasound showed occupation of the entire uterine cavity by dense material consistent with pyometra in addition to myometrial thinning due to tension and cervical dilation. The patient presented with greenish vaginal discharge of 3 months' duration. The cervix was not visible during speculum examination. Access for endometrial sampling was not possible, raising suspicion of post-conization cervical stenosis. The patient was treated with laparoscopic hysterectomy with double adnexectomy. Histologic examination showed superficial squamous cell carcinoma invading the cervix to a depth of 2.8 mm; superficial spreading squamous cell carcinoma in situ was also observed in the lower uterine segment and endometrium. The patient was free of symptoms 12 months after surgery. Squamous cell carcinoma of the cervix with superficial spread to the endometrium is not included in the 2020 (fifth edition) World Health Organization Classification of Female Genital Tract Tumors or the 2018 International Federation of Gynecology and Obstetrics cervical cancer staging system. More clinical cases are needed to identify other prognostic factors and inform clinical practice guidelines on the management of this disease."}, {"id": "pubmed23n0517_8369", "title": "[Results of diagnostic hysteroscopy in a 7-year period in the gynecological clinic of \"UMBAL-Pleven\"].", "score": 0.009900990099009901, "content": "The aim of the authors is to show the data for the reception diagnosis, age, histological results and the conduct after the performed diagnostic hysteroscopies in Gynecological clinic of UMBAL-Pleven. For the fulfillment of this aim was made a prospective study for 7 years' period: from 01/01/1997 to 31/01/2003. The objects of observation were 74 women of age from 16 to 65 years, with performed hysteroscopies for gynecologic complaints. There were performed 74 diagnostic hysteroscopies for the studied period. The hysteroscopic findings were 20 cases with endometrial polyposis, 14--submucosal myoamatic nodes, deforming the uterine cavity, 4--cervical polyp, 19--increased endometrium, 9--Asherman syndrome, 1--bicomous uterus, 1--a suspected section for endometrial carcinoma and 6 cases without pathologic findings. There were performed 59 trial abrasions and the removed materials were sent for histological examination The performed comparative analysis between the hysteroscopic presentation and histological findings showed a coincidence of the diagnosis. It was made the conclusion, that the hysteroscopy is an easy, accessible and inexpensive diagnostic method, which must take its place as one of the basic contemporary diagnostic methods in gynecology."}, {"id": "pubmed23n1086_19676", "title": "Identification and treatment of a cervical sinus tract in a patient with 10 years of infertility.", "score": 0.00980392156862745, "content": "To introduce a special case of endometrial cavity fluid (ECF), highlighting the application of hysteroscopy and laparoscopic surgical techniques in the treatment of cervical sinus tract. Narrated video featuring the diagnosis and surgical management of a case of recurrent ECF. Informed consent was obtained from the patient, and approval was granted by the ethics committee of the First Affiliated Hospital of the Wenzhou Medical University. Academic tertiary hospital. A 36-year-old woman, gravida 0, had menstrual spotting for 13 years after abdominal myomectomy of a 104 × 86 × 111-mm myoma on the posterior uterine wall near the cervix. She failed to conceive after her marriage for 10 years, and 5 operations, including hysteroscopy and laparoscopy, were performed to increase pregnancy opportunities. She also underwent in vitro fertilization and embryo transfer procedures many times, but failed. Transvaginal sonography preoperatively suggested that ECF sometimes appeared and sometimes disappeared. The local echo of the posterior wall of the cervix was enhanced. A 40-mm cystic dark area was found beside the right ovary, which seemed to connect with the cervical hyperechoic part. Additionally, a solid mass of the right adnexa with abundant blood supply was detected. First, hysteroscopy was performed to explore the ECF. A deep and narrow cervical sinus with a steady stream of accumulated blood overflowed in the lower part of the cervix, and a normal uterine cavity was found. Laparoscopic adhesiolysis and enucleation of the cystic structure that connected to the sinus tract then were performed. Hysteroscopy was repeated to determine the thinnest cervical region by the light transmission test. A horizontal incision was made on the thinnest layer. Scar tissues were removed. The incision was sutured in full layer intermittently and continuously under laparoscopy. The postoperative thickness of the muscular layer in the sinus was confirmed by light transmission test of hysteroscopy. The patient was discharged on the third day after operation, uneventfully. Histopathologic examination showed that the cystic structure and scar tissue contained smooth muscle tissue and were covered by both mucinous columnar epithelium of the cervical canal and endometrial glandular epithelium. Restoration of normal anatomy, removal of uterine effusion, and symptomatic relief. At the 6-month follow-up, the patient's menstrual cycles returned to normal without the recurrence of menstrual spotting. The ultrasound scan also showed a symmetrical uterus without ECF. Patients with ECF who underwent assisted reproductive surgeries were related to the poor prognosis. However, the treatment should be different according to the causes, appearance time, and accumulation amount, including expectant treatment, postponement of embryo transfer, transvaginal aspiration, laparoscopic salpingectomy, or proximal tubal occlusion. For patients with recurrent ECF and/or special appearance on ultrasound, endoscopic examination is necessary. In addition, patients with large myomas at difficult locations required a uniform strategy to reduce the intraoperative and postoperative complications, especially for the nulligravida women."}, {"id": "pubmed23n0482_22277", "title": "Comparison of hydrosonography and transvaginal ultrasonography in the detection of intracavitary pathologies in women with abnormal uterine bleeding.", "score": 0.00980392156862745, "content": "The aim of the study was to compare the accuracy of hydrosonography with that of transvaginal ultrasonography in detection of intracavitary pathologies in patients with history of abnormal uterine bleeding. Prospective, randomized, and unblinded study. A total of 197 women (n = 130 premenopausal and n = 67 postmenopausal) aged between 23 and 71 years (mean age 45.7 +/- 8.9) presenting with a history of abnormal uterine bleeding were included into the study. Hydrosonography was carried out by experienced gynecologists, on the same setting in an outpatient clinic immediately after the performance of transvaginal sonography. The finally obtained surgical-pathologic findings were compared with the results obtained from transvaginal sonography and hydrosonography. Sensitivity, specificity, positive, and negative predictive values were calculated for each procedure. The surgical-pathologic examination confirmed normal physiologic endometrium in 50 (48%) of 104 women who were said to have normal endometrium on transvaginal sonography. Seventy (75%) of 93 women diagnosed of intracavitary pathologies on transvaginal sonography were confirmed by surgical-pathologic findings. The sensitivity, specificity, positive predictive value, and negative predictive value of transvaginal sonography in the detection of intracavitary pathology were 56, 68, 75, and 48%, respectively. Surgical-pathologic results revealed intracavitary pathologies in 23 (30%) of 76 women who were said to have normal endometrium on hydrosonography. Among 121 women diagnosed of intracavitary pathologies on hydrosonography, 101 (81%) women were confirmed after histological evaluation of the surgical specimens. The sensitivity, specificity, positive predictive value, and negative predictive value of hydrosonography in the detection of intracavitary pathology were 81, 73, 83, and 70%, respectively. Sensitivity and negative predictive value were significantly higher with hydrosonography. There were five cases of endometrial malignancy in which one of the case of malignancy was on polyp and two cases of endometrial hyperplasia with atypia which were not stated on sonographic results. Hydrosonography is more accurate than transvaginal ultrasography in the detection of intracavitary pathologies in women with abnormal uterine bleeding."}, {"id": "pubmed23n0843_6206", "title": "An Exceptional Case of Complete Septate Uterus With Unilateral Cervical Aplasia (Class U2bC3V0/ESHRE/ESGE Classification) and Isolated Mullerian Remnants: Combined Hysteroscopic and Laparoscopic Treatment.", "score": 0.009708737864077669, "content": "To report the combined hysteroscopic and laparoscopic treatment of a complete septate uterus with unilateral cervical aplasia (class U2bC3V0/ESHRE/ESGE classification) and isolated mullerian remnants. Step-by-step presentation of the surgical treatment (Canadian Task Force classification 4). Complete septate uterus with unilateral cervical aplasia (formally Robert's uterus) is characterized by the presence of a uterine septum completely dividing the endometrial cavity into an obstructed hemicavity and a contralateral nonobstructing hemicavity connected normally to the existing cervix. It has always been described as isolated without any associated anomaly. A 30-year-old woman was referred to our department for dysmenorrhea and primary infertility. Hysterosalpingography showed the presence of a right (RT) hemiuterus with a patent fallopian tube; further evaluation with 2- and 3-dimensional ultrasound and magnetic resonance imaging showed an externally normal-appearing uterus, a right normal hemicavity connected normally with the existed cervix and, a left hemicavity fully divided from the right one by a complete septum and not connected with the cervix. Interestingly, a peculiar complex mass with cystic areas, attached posterolaterally from the left side to the uterine wall at the level of the isthmus and the upper cervix, was also diagnosed. The study protocol was approved by our local institutional review board. During outpatient hysteroscopy, a right uterine hemicavity with a single ostium was identified without any communication with the left hemicavity. The patient was then scheduled for combined laparoscopic and hysteroscopic treatment. During laparoscopy, a normal uterine body with multiple myomas and a pseudocystic lesion attached posteriorly and left laterally to the uterus at the level of the isthmus and the upper cervix were shown; no communication between the cystic part of that lesion and the isthmus or the cervicovaginal canal was observed. During hysteroscopy, a longitudinal incision of the septum with a 5F bipolar electrode was performed; the left hemicavity was opened, and the corresponding tubal ostium was identified. The pseudocystic lesion was then excised after opening and sent for pathological analysis; the defect was closed with interrupted intracorporeal knots. A single normal endometrial cavity with both tubal ostia was obtained, thus restoring obstruction by unification of the uterine cavity. A histologic report of the removed pseudocystic lesion was compatible with the diagnosis of mullerian remnants. A follow-up hysteroscopy 3 months after showed a normal uterine cavity without postsurgical adhesions. The use of 3-dimensional ultrasound and magnetic resonance imaging in combination with the new ESHRE/ESGE classification system gives the opportunity to obtain a precise representation of the female genital anatomy even in the presence of complex anomalies. Although a septate uterus with unilateral cervical aplasia has been already described, the presence of mullerian remnants is a rare entity associated with cyclic pelvic pain, thus needing adequate recognition and treatment. The combined hysteroscopic and laparoscopic approach offers a unique opportunity for the treatment of complex anomalies."}, {"id": "pubmed23n0731_24083", "title": "[Comparison of the measurement line cervical and endometrial ultrasound techniques using two].", "score": 0.009615384615384616, "content": "Cervical and endometrial measurement in the gynecological and obstetric patients is of vital importance. There is no consensus for the correct way in which should be made the measurement, more than anything is for an opinion of some experts. To determine whether there are differences in measurement of endometrial or sagittal cross section of the uterus and cervix in the measurement of fractional linear fashion or along the cervical canal. Using a transvaginal ultrasound, was measured in a transverse and longitudinal endometrial lining. And by fractional measuring along the cervical canal and a linear manner from the internal to the external. We studied a total of 63 patients. The mean endometrial transversal measurement in a cross was 7.1. mm (SD +/- 3.3) The mean endometrial longitudinally measured were: 7.9 mm (SD +/- 3.4). The mean cervical measurement was fractionally 3.3 cm (SD +/- 0.4) Mean cervical linear measurement was 3.9 cm (SD +/- 0.4). Using student's t test where the value of p in the endometrial measurement was 0.0871 and p value in cervical measurement was 0.009, the latter being statistically significant. With respect to the measurement of the endometrial lining, there is no significant difference do any of the two different techniques. However, measurement of the cervix, another significant difference (p = 0.009), so it should be further investigated which of these two techniques is the right way to establish more accurate diagnoses."}, {"id": "wiki20220301en043_17456", "title": "Vaginal bleeding", "score": 0.00958640914147032, "content": "Diagnostic evaluation The cause of the bleeding can often be discerned on the basis of the bleeding history, physical examination, and other medical tests as appropriate. The physical examination for evaluating vaginal bleeding typically includes visualization of the cervix with a speculum, a bimanual exam, and a rectovaginal exam. These are focused on finding the source of the bleeding and looking for any abnormalities that could cause bleeding. In addition, the abdomen is examined and palpated to ascertain if the bleeding is abdominal in origin. Typically a pregnancy test is performed as well. If bleeding was excessive or prolonged, a CBC may be useful to check for anemia. Abnormal endometrium may have to be investigated by a hysteroscopy with a biopsy or a dilation and curettage."}, {"id": "pubmed23n0910_22439", "title": "A Rare Uterine Malformation: Asymmetric Septate Uterus.", "score": 0.009523809523809525, "content": "To demonstrate a step by step surgical hysteroscopy technique in a patient with asymmetric uterine septum and transverse uterine septum that was not previously described in the literature. Resection of an asymmetric uterine septum by laparoscopy and ultrasound-guided hysteroscopy (Canadian Task Force classification III). The video was assumed exempt from official review by our institutional review board. A septate uterus is defined as the uterus in which the uterine cavity is longitudinally divided by the septum [1]. The most common uterine anomaly, septate uterus has a spectrum of configurations ranging from complete septate to incomplete septate uterus. Asymmetric uterine septum was reported only as case reports in the literature and is described as Robert's uterus [2]. This unique malformation is described as a septate uterus with a noncommunicating hemicavity, composed of a blind uterine horn usually with unilateral hematometra, and a contralateral unicornuate uterine cavity. The external uterine shape is normal. The asymmetric septum with transverse uterine septum in the present case has not yet been reported in the literature. A 29-year-old woman presented to our clinic with primary amenorrhea, cyclic pelvic pain, and the desire to have pregnancy. She previously had failed 2 laparoscopy and hysteroscopy procedures for fertility treatments. Hysterosalpingography previously had been failed. The patient previously underwent magnetic resonance imaging. The magnetic resonance imaging report states there was no connection between the uterus and cervix. On external genital organs assessment, there was no abnormal sign. Ultrasonography revealed 2 uterine cavities and hematometra. Both ovaries were in normal view. In view of her examination findings, the patient was scheduled for laparoscopy and hysteroscopy. Laparoscopy revealed extensive adhesions on both the pelvis and upper abdomen. Initially, the uterus and ovaries were not visualized. Adhesiolysis was performed, and normal anatomy was restored. After this step, the operation was continued by laparoscopy and ultrasound-guided hysteroscopy. Under ultrasound and laparoscopy guidance, the transverse uterine septum at the level of uterine isthmus was incised and the left endometrial cavity was observed with hysteroscopy. The asymmetric uterine septum was then incised, and the right-sided endometrial cavity was then accessed. Finally, the uterine septum was completely incised and both sides of the endometrial cavities were merged. The patient had an uncomplicated postoperative course and was discharged 24 hours after surgery. She returned for follow-up examination in the second month after surgery. She had regular menstrual cycles, and her pain was cured. Hysteroscopy and laparoscopy combined with ultrasound is a useful method for the diagnosis and treatment of asymmetric uterine septum. The skill and experience of the laparoscopic surgeon is another important factor to identify and manage unusual uterine malformations."}, {"id": "pubmed23n0378_21611", "title": "[The role of transvaginal ultrasound and sonohysterography in the diagnosis and staging of endometrial adenocarcinoma].", "score": 0.009523809523809525, "content": "The aim of this study is to evaluate the accuracy of sonohysterography in early diagnosis of endometrial tumor lesions and in the detection of myometrial infiltration for staging. We performed sonohysterography as a preoperative test in 24 patients with an hystologic diagnosis of endometrial adenocarcinoma obtained by hysteroscopy and biopsy. The mean age of the patient was between 50 and 82 years. The sonohysterographic examination was performed by using 5.0 and 6.0 MHz transvaginal probes and a 5 or 7 French hysteroinjectors with inflating balloon. 19 of the 24 patients were enrolled in the study: in 2 cases the examination was not technically performable, 2 patients refused surgical treatment and 1 patient had a cervical adenocarcinoma with extension to the myometrium. In each patient we evaluated the number and the size of the lesions and the degree and the depth of myometrial infiltration. Each parameter was compared with the final histopathologic examination. Sonohysterography showed a single lesion in 15 patients, whereas in 4 patients it showed multiple lesions; in 1 of these patients it showed 3 lesions which were, in reality, a single lesion that infiltrated the first half of the myometrium. Myometrial infiltration was correctly evaluated by the examination in 17 of the 19 women (89.4%): 16 positive and 1 negative case. The sensitivity was 88%, the specificity 100%, the positive predictive value 100% and the negative predictive value 33%. The sonohysterography allowed to evaluate exactly the depth of myometrial invasion in 15 of the 16 cases (93.7%), in which a myometrial infiltration was suspected. With regard to this parameter the sensitivity was 85.7%, the specificity was 100%, the positive predictive value 100% and the negative predictive value 90.9%. Although the introduction of transvaginal ultrasonography in clinical practice allows to obtain an early diagnosis of endometrial adenocarcinoma, about half patients seems to present already at the diagnosis myometrial invasion. Moreover 50% of these patients seems to have pelvic lymphonodes and about 29% positive paraaortic lymphonodes. Currently myometrial invasion is evaluated by the extemporary frozen test and confirmed by the definitive hystologic examination. It would be helpful to have a technique able to detect and evaluate infiltration before surgery. The results of this study suggest that sonohysterography could have a role in preoperative staging. However these data need to be confirmed by further studies."}, {"id": "pubmed23n0733_1968", "title": "[Comparative study of transvaginal sonography and outpatient hysteroscopy for the detection of intrauterine diseases].", "score": 0.009433962264150943, "content": "Intrauterine diseases are common morbid disorders. Endometrial and endocervical polyps, myomas, synechiae, uterine malformations, endometrial hyperplasia and endometrial cancer are cited among intrauterine pathology. The investigations using transvaginal sonography and outpatient hysteroscopy had been a gold standard. Transvaginal sonography shows endometrial thickness and heterogeneous variations within the echogenecity of the endometrium uterine pathology. Transvaginal sonography is easy to apply for evaluation of intrauterine pathology and it has high sensitivity to diagnostic for intrauterine disorders. Hysteroscopy was used the gold standard control. It permitted the better identification of intrauterine pathology but the histologic examination has been used for definitive diagnostic. Difficulty apprenticeship this technique had very decrease your access. To evaluate the efficiency of transvaginal ultrasonography and outpatient hysteroscopy in the diagnosis of intrauterine pathology. The study conducted was a retrospective diagnostic-type test. They involved a total of 469 women underwent diagnostic hysteroscopy in 2006 in Campinas University. Seventy-nine women were excluded due to lack of ultrasound results in their medical charts. One-hundred and forty-seven premenopausal women and two-hundred and forty-three postmenopausal women. For statistical analysis, the sensitivity, specificity, positive predictive value, negative predictive value and accuracy. The gold standard of the ultrasonography was the hysteroscopy and the gold standard of the hysteroscopy was the endometrium biopsy. The mean age of postmenopausal women was 61 ± 9.4 years. We observed 6.6% of endometrial hyperplasia and cancer and 54% of endometrial polyps. Ultrasonography had a sensitivity of 95.6%, a specificity of 7.4% and an accuracy of 53.7%, while hysteroscopy had a sensitivity of 95.7%, a specificity of 83% and an accuracy of 88.7%. The mean age of premenopausal women was 40 ± 8.2 years. Endometrial cancer was not observed and two cases of endometrial hyperplasia were found. We observed 34% of endometrial polyps. Sensibility was 52.9%, specificity was 68.4% and the accuracy was 61.2% for polyps on ultrasonography while in hysteroscopy was 78.8%, 67.6% and 73.1% respectively. For myoma, sensitivity was 70.6% and 64.3%, specificity was 44.3% and 98.1% and accuracy was 63.3% and 91.2% in ultrasonography and hysteroscopy respectively. Hysteroscopy had better diagnostic accuracy than ultrasonography for the detection of intrauterine pathology."}, {"id": "pubmed23n0558_3040", "title": "Synchronous occurrence of primary neoplasms in the uterus with squamous cell carcinoma of the cervix and adenocarcinoma of the endometrium.", "score": 0.009345794392523364, "content": "Synchronous primary malignant neoplasms of the uterus are uncommon. Patients with synchronous cervical and endometrial cancers are even rarer. We describe a case of cervical squamous cell carcinoma and endometrial endometrioid adenocarcinoma occurring simultaneously in a 47-year-old woman presenting with massive menstrual bleeding. The concept of synchronous primary malignancies of the genital tract is also reviewed in this report. A 47-year-old overtly obese female presented with menometrorrhagia of over 6 months' duration. Pelvic examination detected a large cervix but apparently normal externals. Magnetic resonance imaging revealed a mass over the cervical region and endometrial lesions in the uterine cavity. Surgical exploration disclosed a cervical tumor and erosion of the endometrium. The pathologic findings were compatible with synchronous occurrence of primary neoplasms in the uterus with squamous cell carcinoma of the cervix and adenocarcinoma of the endometrium. Synchronous genital tract neoplasms are rare but cause more clinical problems than a single neoplasm. It is practical to pay more attention to the differential diagnosis of primary and metastatic tumors. The second primary cancer that occurs in an individual with endometrial cancer may offer an opportunity for early detection. The prognosis for a patient with synchronous gynecologic malignancies does not seem to be worse."}, {"id": "pubmed23n0101_15140", "title": "[Comparison of the progesterone test and uterus sonography as screening procedures in the detection of patients at risk of endometrial cancer].", "score": 0.009345794392523364, "content": "The increasing incidence of endometrial cancer requires more attention to early detection. Postmenopausal women without symptoms were examined by progesterone challenge test (96 diabetics, 111 without diabetes) and by sonography (44 diabetics, 74 without diabetes) in order to recognize proliferation of the endometrium. The application of progesterone induced a bleeding in 4% of the women. The diagnostic curettage performed 4-6 weeks after this test revealed almost always atrophic endometrium. We found a good correspondence between abnormalities of the uterine cavity detected by sonography and the results of the pathological examination after notice pathological changes of the endometrium."}, {"id": "pubmed23n0342_10010", "title": "Clinical evaluation of follow-up methods and results of atypical glandular cells of undetermined significance (AGUS) detected on cervicovaginal Pap smears.", "score": 0.009259259259259259, "content": "The aim of this study was to evaluate the efficacy of the follow-up methods and results of atypical glandular cells of undetermined significance (AGUS) detected on cervicovaginal Pap smears. From May 1991 to December 1996, we have performed 407, 451 cervicovaginal Pap smears, of which 326 patients were identified as AGUS. Of the 326 patients, 268 patients were followed by repeat Pap smears, colposcopy, cone biopsy, or endometrial curettage. The incidence of AGUS on Pap smears is approximately 0.08%. The mean age of the patients was 43 years (range 22-79 years). The most common complaint was abnormal vaginal bleeding. The gross findings of the cervix were normal to mild erosion. The following past histories of patients could affect the AGUS results on Pap smear: 30 had cone biopsy, 21 had Pap smears on pregnancy and within 8 weeks after delivery or evacuation, 3 were on hormonal replacement therapy, 2 had intrauterine devices for contraception, and 5 were undergoing follow-up after treatment of cervical cancer. The benign lesions detected during follow-up periods were 6 microglandular hyperplasia of the cervix, 5 atypical squamous metaplasia of the cervix, 2 cervical endometriosis, 2 tubal metaplasia, 10 cervical myoma, 11 cervical polyps, 9 endometrial polyps, 3 uterine myoma, 1 pelvic endometriosis, 1 ovarian endometriosis, and 4 uterine adenomyosis. The premalignant or malignant lesions of the cervix were 4 low-grade squamous intraepithelial lesions, 24 high-grade squamous intraepithelial lesions, 8 glandular atypia/dysplasia, 5 adenocarcinoma in situ, 3 microinvasive adenocarcinoma, and 4 invasive adenocarcinoma. The neoplastic lesions of the uterus were 6 endometrial hyperplasia, 11 endometrial adenocarcinoma, 1 malignant mixed Müllerian tumor, and 1 metastatic endometrial adenocarcinoma. Sixty-seven (25%) of 268 patients followed up were identified as having clinically significant lesions of the cervix or uterus. The detection rates of abnormal lesions were 3.1% with repeated Pap smears (3/98), 28.4% with colposcopic-directed biopsy (31/109), 63.6% with cone biopsy (35/55), and 29.7% with endometrial curettage (19/64). AGUS on Pap smears showed various benign and malignant lesions of the cervix or uterus. The clinicians must communicate with the pathologists regarding the patient's clinical information as well as the origin of the atypical glandular cells in Pap smears. We recommend that patients with AGUS on Pap smear should undergo immediate intensive diagnostic studies, including colposcopic-directed biopsy with endocervical curettage or cone biopsy, to detect cervical lesions and endometrial curettage to detect endometrial lesions."}, {"id": "pubmed23n0282_6157", "title": "[Evaluation of the endometrium by vaginal ultrasonography].", "score": 0.009259259259259259, "content": "A group of 131 women aged between 35 and 70 years old were examined using endo-vaginal ultrasonography. Of the patients examined, 45 had been in menopause for at least 2 years and 86 were pre-menopause. 48.9% of the post-menopause patients reported loss of blood and 69.8% of pre-menopause patients reported alterations in the menstrual cycle. Ultrasonography was performed using an endo-vaginal 5 MHz (Ansaldo 600) probe with the patient in a dorsosacral position. During the course of ultrasonography the appearance of the endometrium, in particular its thickness, dyshomogeneity and, in pre-menopause women, its correspondence with the stage of the ovarian cycle, were examined. Taking these parameters into account, the type of endometrial echo patterns were classified, separating the cases into pre- and post-menopause groups. Of the women in menopause, 22 underwent curettage due to symptoms, independently of endometrial thickness which was over 4 mm in 50% of cases, 3 women underwent curettage following ultrasonographic indications, whereas the remaining 20 underwent hysterectomy due to other pathologies. Of the pre-menopausal group, 10 underwent curettage following ultrasonographic indications and 42 due to symptoms; the remaining patients underwent hysterectomy due to pathologies not related to the endometrium. A clearly defined role of endovaginal ultrasonography in the diagnosis of endometrial pathologies emerges if ultrasonographic findings are correlated with histological ones. In the group of post-menopausal women (45), all those cases which were histologically classified as endometrial carcinoma (4) were associated with an endometrial thickness over or equal to 4 mm.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0314_20681", "title": "Colposcopy, cervicography, speculoscopy and endoscopy. International Academy of Cytology Task Force summary. Diagnostic Cytology Towards the 21st Century: An International Expert Conference and Tutorial.", "score": 0.009174311926605505, "content": "The colposcope was developed in 1925 and is well established in clinical gynecologic practice for defining and delineating cytologically detected lesions mainly of the cervix but also the vagina and vulva. Additionally, various endoscopic procedures in gastroenterology, pulmonary and urologic lesions enhance the cytologic detection and histologic verification of precancerous and cancerous lesions. The cost-effectiveness of all these devices and their applicability, particularly in countries with a limited health budget, is a major issue. This task force considered aspects of the present state of the art and the challenges in the 21st century. Automated cytology can interface with colposcopic examination in a number of significant ways. Automated cytologic analysis of conventional cervical smears can potentially direct colposcopic examination by predicting the nature of a lesion, assist in determining which patients should receive colposcopy and, in some settings, thereby reduce the number of colposcopies. Potentially, various combinations of automated cytology and colposcopy may be used to generate screening protocols that might result in more effective and inexpensive screening. The role of cervicography, or high-resolution cervical photography, as a screening device remains to be defined. Sensitivity for high grade lesions is generally no greater than that in cytology, and specificity appears lower. The interpretation of cervical photographs in triage of mildly abnormal cytology may prove to be useful in countries with established cytology programs. In areas of the world where cytology screening programs are not in place, the interpretation of cervical photographs may have its most dramatic effect. Cost-effectiveness analyses are needed. There are, at present, insufficient data for the evaluation of speculoscopy, a procedure using chemiluminescent illumination of the cervix for visualization of acetowhite areas. Basic training in colposcopy should be integrated into the residency programs of obstetrics and gynecology. Criteria for the adequate training of colposcopists should be developed. Continuing education programs in colposcopy should be developed when they are not already in existence. The cost-effectiveness of integrating colposcopy as a primary screening technique should be evaluated. Following a high-grade squamous intraepithelial lesion (HSIL) cytology result, colposcopically directed punch biopsy should be taken with or without endocervical curettage. This generally should precede the loop electrosurgical excision procedure (LEEP); however, in certain circumstances direct LEEP may be indicated. LEEP under colposcopic vision is an efficient way to treat an HSIL lesion of the cervix because the histologic extent and margins can be determined, unlike with laser surgery or cryosurgery. It is also more cost-effective than cold knife conization because general anesthesia and an operating room are unnecessary. Following LEEP, the endocervical canal should be examined colposcopically for any evidence of involvement. Lesions in the endocervix can then be removed with a different-shaped loop. Further research into Raman spectroscopy as a diagnostic aid in cervical pathology is needed, as is the use of micrococolpohysteroscopy for in vivo cytologic analyses, especially of the endocervical canal and transformation zone. Hysteroscopy is the most direct method for the diagnosis and treatment of intrauterine diseases. Hysteroscopic endometrial biopsy is more accurate than conventional biopsy methods. Cervical invasion of endometrial cancer can be detected by hysteroscopy. The depth of invasion, however, is more accurately determined by magnetic resonance imaging or computed tomography. Many topics for ongoing research and/or implementation are mentioned under \"Consensus Position,\" above. (ABSTRACT TRUNCATED)"}, {"id": "pubmed23n0393_113", "title": "[Diagnostic procedures of pathological bleeding in women population: comparison of hysteroscopy, ultrasonography and microscopic examination endometrium].", "score": 0.009174311926605505, "content": "The goal of this study was to asses the diagnostic value of hysteroscopy, cytology, ultrasonography and histopathology in various pathological states in endometrium. 250 patients with abnormal uterine bleeding were examined. The wide range of several diagnoses were achieved with 8 cases of cancer. Used methods were found to be complementary because 7 cases of cancer were recognised by histopathologic method, 6 by hysteroscopy and 5 by a cytological test (3 results were suspicious). To the risk group were qualified by ultrasonography all patients in postmenopausal age."}, {"id": "pubmed23n0601_3853", "title": "[A falsely reassuring cervical smear in adenocarcinoma of the external os].", "score": 0.00909090909090909, "content": "3 women with only mild changes in cervical smears were later found to be suffering from cervical adenocarcinoma. The first patient was 53 years old. Her smears repeatedly showed Pap 3 with moderately atypical glandular cells. After 3 colposcopic examinations with biopsies and 2 loop electrosurgical excision procedures of the cervix which showed no histological signs of malignancy, diagnostic conization revealed an adenocarcinoma of the endocervix. She underwent a radical hysterectomy and chemoradiation because of positive pelvic nodes. The second patient was 30 years old and had persistent vaginal discharge and an enlarged cervix, but no cytological abnormalities. Colposcopy was unsatisfactory and the tissue obtained by loop electrosurgical excision was normal. Adenocarcinoma was diagnosed after conization. She was treated with radical hysterectomy and radiotherapy but died after one year. The third patient, aged 26, had a long history of slightly abnormal Pap smears and vulvar condylomata, and was referred with vaginal discharge. A severe abnormal smear with glandular atypia was followed by colposcopical biopsies and conization, which revealed an endocervical adenocarcinoma. She underwent radical hysterectomy. Adenocarcinoma is a rare type of cervical cancer: III cases out of 584 patients with cervical cancer in 2003 in the Netherlands. This neoplasm is more difficult to detect than cervical squamous cell carcinoma. Cervical cytology is not an effective tool for screening and diagnosis. Due to the localization, multifocality and diversity in its presentation, the assessment of cytology has a high false-negative percentage. Screening may be enhanced by combining cytology with testing for high-risk HPV types, notably type 18. If cervical cytology shows persistent atypical glandular cells with no conclusive histological result, then due to the endocervical localisation of the lesions adenocarcinoma can only be excluded by conization."}, {"id": "pubmed23n0642_22472", "title": "Can routine gynecologic examination contribute to the diagnosis of cervical involvement by primary endometrial cancer?", "score": 0.00909090909090909, "content": "There are few data in the literature as to whether findings at routine preoperative gynecologic examination of patients with primary endometrial cancer including cervical cytology, colposcopy and rectovaginal bimanual pelvic exam could predict cervical extension of the disease. The present retrospective study was undertaken to preoperatively identify potential clinical parameters associated with the histological diagnosis of cervical involvement by primary endometrial cancer in the hysterectomy specimen. We reviewed the records of 104 patients with Stage II endometrial cancer treated at our institution between 1985 and 2005 by simple or radical abdominal hysterectomy with special emphasis on cervical Pap smear, colposcopy, cervical palpation as well as rectal parametrial assessment. Patients with Stage I disease operated on before and after each study patient were selected as controls (n = 208). Patients with more advanced disease were excluded. Overall, 312 records of patients with primary endometrial cancer were reviewed. Patients with Stage II disease had a significantly lower prevalence (p &lt; 0.0001) of endometrioid carcinomas and a significantly higher (p &lt; 0.01) prevalence of G3 tumors compared to the control patients. Pap smears and colposcopic findings were abnormal in 39% of patients with Stage II and in 9% and 10% of patients with Stage I disease (p &lt; 0.0001). Of patients with Stage II disease, 42% had a suspicious cervical palpation compared to only 4% of patients with Stage I disease (p &lt; 0.0005). Parametrial assessment was suspicious in 16% of patients with Stage II disease and in no patient with Stage I disease (p &lt; 0.001). The four routine clinical parameters Pap smear, colposcopy, cervical palpation and rectal parametrial examination are significantly more often pathologic in patients with Stage II than in Stage I disease. The majority of patients with Stage II disease had at least one of these tests positive. Thus they may be useful to preoperatively detect cervical involvement by primary endometrial cancer."}, {"id": "wiki20220301en248_38962", "title": "Gynaecologic cytology", "score": 0.009009009009009009, "content": "Gynecologic cytology, also Gynecologic cytology, is a field of pathology concerned with the investigation of disorders of the female genital tract. The most common investigation in this field is the Pap test, which is used to screen for potentially precancerous lesions of the cervix. Cytology can also be used to investigate disorders of the ovaries, uterus, vagina and vulva. Gynaecology Cytopathology Pathology"}, {"id": "pubmed23n0971_14387", "title": "[COMPARISON OF ULTRASOUND INVESTIGATION METHODS IN POSTMENOPAUSE].", "score": 0.009009009009009009, "content": "The aim of the study is to improve the diagnostic approach in assessing the state of the endometrium with the help of ultrasonic endometrial diagnostics in accordance with the IETА criteria in combination with three-dimensional Doppler indices, calculating the volume of the endometrium, and the 3D reconstruction in postmenopausal women. 167 postmenopausal women underwent a 2D pelvic ultrasound examination and a combined three-dimensional complex pelvic ultrasound examination that included calculation of the volume of the endometrium, three-dimensional dopplerometric indices (vascularization index, blood flow index and vascularization ratio), and 3D-reconstruction mode. In the second stage, the patients underwent hysteroscopy/endometrial bipyroscopy with morphological evaluation of tissue samples, which retrospectively performed analysis of echographic and dopplerometric criteria in patients with benign changes in the endometrium and their comparison with atrophic endometrium. In addition, a comparative analysis of the 2D ultrasound method and its combination with a complex 3D study was carried out. Most of the echographic criteria between the groups of hyperproliferative pathology and endometrial atrophy had statistical differences, but there were no significant differences in comparison with the group of synechia of the uterine cavity and cystic atrophy. While the analysis of Dopplerometric criteria both in the two-dimensional mode and with its combination with trimer techniques demonstrated a statistical difference between the indices in the groups of the endometrial hyperpliphyral pathology and the endometrium atrophy, including its cystic form, and also the synechia of the uterine cavity. Based on the results of the comparative analysis of the ultrasound modes, the combination of 2D study with a complex 3D study increases the sensitivity of the method by 12%, and the specificity by 13%. Three-dimensional echography with the determination of dopplerometric indices and volume of endometrium and 3D reconstruction is highly accurate in the diagnosis of endometrial pathology, its wide practical application at the preoperative stage will allow to improve the quality of diagnostics and to formulate clear criteria for hysteroscopy in postmenopausal women. The results of the study show that the thickness of the endometrium is not an absolute criterion in determining the pathology of the endometrium. The main criterion in the diagnosis of hyperproliferative changes in the endometrium in postmenopause is the presence in it of vascularization."}, {"id": "wiki20220301en043_2443", "title": "Adenomyosis", "score": 0.008928571428571428, "content": "Histopathology The diagnosis of adenomyosis is through a pathologist microscopically examining small tissue samples of the uterus. These tissue samples can come from a uterine biopsy or directly following a hysterectomy. Uterine biopsies can be obtained by either a laparoscopic procedure through the abdomen or hysteroscopy through the vagina and cervix. The diagnosis is established when the pathologist finds invading clusters of endometrial tissue within the myometrium. Several diagnostic criterion can be used, but typically they require either the endometrial tissue to have invaded greater than 2% of the myometrium, or a minimum invasion depth between 2.5 and 8mm."}, {"id": "pubmed23n0475_12850", "title": "[Comparison between hysterosonography and hysterosalpinography in the study of endometrial abnormalities in infertility patients].", "score": 0.008928571428571428, "content": "We compare effectiveness between HSS and HSG for detection of endometrial abnormalities among patient with infertility. Prospective, cross section, comparative. 33 patients were studied. HSS and HSG were carried out. The images of both studies were compared, settled down a presumptive diagnosis of normality or abnormality. The analysis included percentage of agreement, coefficient of agreement Kappa, square Ji, reason of verisimilitude. Of 33 studied patients was diagnostics correlation in 17 (52%). The pathologies with more correlation were congenital malformations and submucous miomas, with less correlation were adherences. Other opposing pathologies were polyps and corioplacentary remains. In 9 patients (27%) we find normality in both studies. When comparing the positive studies in each group we don't find differs statistically significant (P &gt; 0.05). The association among both techniques was high (K = 0.70). The reason of verisimilitude of the tests was 98% for HSS and 90% for HSG. Both studies are useful to evaluate the uterine cavity. However histerosonography has bigger sensibility and specificity, besides is a quick, easy, and economic study, with less discomfort and complications than histerosalpingography."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": true, "char_ranges": [[360, 450]], "word_ranges": [[53, 69]], "text": "The first one because it is not a bacterial meningitis that would present with more fever,"}, "2": {"exist": true, "char_ranges": [[0, 325]], "word_ranges": [[0, 47]], "text": "The picture described is typical of a viral meningoencephalitis, more specifically VHZ, with significant tropism by the temporal lobe, causing febrile fever and in many cases aphasia, so the correct option would be 2, since the cerebrospinal fluid normally shows a moderate lymphocytic pleocytosis, with normal glycorrhachia."}, "3": {"exist": true, "char_ranges": [[451, 542]], "word_ranges": [[69, 81]], "text": "the third one because limbic encephalitis usually presents afebrile with behavioral changes"}, "4": {"exist": true, "char_ranges": [[547, 626]], "word_ranges": [[82, 96]], "text": "the last one because the image they describe is not an abscess, well delimited."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The picture described is typical of a viral meningoencephalitis, more specifically VHZ, with significant tropism by the temporal lobe, causing febrile fever and in many cases aphasia, so the correct option would be 2, since the cerebrospinal fluid normally shows a moderate lymphocytic pleocytosis, with normal glycorrhachia. The rest are not correct because: The first one because it is not a bacterial meningitis that would present with more fever, the third one because limbic encephalitis usually presents afebrile with behavioral changes and the last one because the image they describe is not an abscess, well delimited.", "full_answer_no_ref": "The picture described is typical of a viral meningoencephalitis, more specifically VHZ, with significant tropism by the temporal lobe, causing febrile fever and in many cases aphasia, so [HIDDEN], since the cerebrospinal fluid normally shows a moderate lymphocytic pleocytosis, with normal glycorrhachia. The rest are [HIDDEN]: The first one because it is not a bacterial meningitis that would present with more fever, [HIDDEN] limbic encephalitis usually presents afebrile with behavioral changes and [HIDDEN] the image they describe is not an abscess, well delimited.", "full_question": "A 52-year-old man comes to the Emergency Department with headache and fever (37.8°C) of 2 days' evolution. In the last few hours, he also had difficulty in nomination and comprehension. The examination did not show nuchal rigidity, the most striking finding being the presence of a mixed aphasia. The cranial CT shows a faint hypodensity in the left temporal lobe without mass effect and without contrast uptake. Which of the following statements is correct?", "id": 374, "lang": "en", "options": {"1": "Bacterial meningitis is the first diagnostic impression and treatment with 3rd generation cephalosporin should be initiated as soon as possible.", "2": "Most likely this patient's CSF shows a lymphocyte-predominant pleocytosis with normal glycorrhachia.", "3": "We would suspect limbic encephalitis.", "4": "This is an early stage brain abscess.", "5": NaN}, "question_id_specific": 101, "type": "NEUROLOGY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0357_15322", "title": "[An unusual presentation of herpetic encephalitis].", "score": 0.013710178365427279, "content": "Herpetic encephalitis (HE) is the commonest cause of acute sporadic encephalitis in the United States and Europe. In 20% of cases, the clinical course is atypical and this may lead to delay in diagnosis and treatment. A 24 year old patient presented with aseptic meningitis, which had been present for the previous 10 days and which then became complicated by fever and aphasia. The cerebrospinal fluid (CSF) showed marked pleocytosis and increased protein. Cerebrospinal puncture was found to be positive for DNA of the herpes simplex virus. Magnetic resonance (MR) imaging showed a temporal lesion with intense uptake of contrast. Treatment was started with acyclovir and the patient improved over the days following this. At present the diagnosis of herpetic encephalitis is based on polymerase chain reaction (PCR) studies of the CSF and MR imaging. The correlation between patients with HE with cerebrospinal puncture showing positive for herpes and alterations on MR is 89%. In view of the 20% of atypical forms, a high degree of clinical suspicion is necessary to try to obtain early diagnosis and treatment."}, {"id": "pubmed23n0763_15924", "title": "Three cases of neoplastic meningitis initially diagnosed with infectious meningitis in emergency department.", "score": 0.012178072111846946, "content": "Neoplastic meningitis (NM) is diagnosed by the presence of malignant cells in the cerebrospinal fluid (CSF). We report 3 patients with NM, who were misdiagnosed with infectious meningitis in emergency department (ED). Case 1. A 68-year-old man visited our ED with a 3-month history of headache. With MRI and CSF study, he was diagnosed with tuberculous meningitis. After 20 days, repeated CSF cytology showed malignant cells. His diagnosis was lung cancer with NM. Case 2. A 57-year-old man visited regional hospital ED with a 3-week history of headache and diplopia. Brain MRI was not contributory. With CSF examination, his diagnosis was aseptic meningitis. With worsening headache, he was referred to our ED. Repeated CSF showed malignant cells. His diagnosis was stomach cancer with NM. Case 3. A 75-year-old man visited a regional hospital with headache lasting for 4 months. His diagnosis was sinusitis. Persistent symptom brought him back, and he developed recurrent generalized seizures. Brain MRI showed diffuse leptomeningeal enhancement suggesting meningitis, and he was transferred to our ED. CSF exam showed malignant cells. His diagnosis was NM with unknown primary focus. When evaluating the patients with headache in ED, NM should be kept in mind as a differential diagnosis of meningitis. "}, {"id": "InternalMed_Harrison_11094", "title": "InternalMed_Harrison", "score": 0.010569433537788908, "content": "A brain abscess typically presents as an expanding intracranial mass lesion rather than as an infectious process. Although the evolution of signs and symptoms is extremely variable, ranging from hours to weeks or even months, most patients present to the hospital 11–12 days following onset of symptoms. The classic clinical triad of headache, fever, and a focal neurologic deficit is present in <50% of cases. The most common symptom in patients with a brain abscess is headache, occurring in >75% of patients. The headache is often characterized as a constant, dull, aching sensation, either hemicranial or generalized, and it becomes progressively more severe and refractory to therapy. Fever is present in only 50% of patients at the time of diagnosis, and its absence should not exclude the diagnosis. The new onset of focal or generalized seizure activity is a presenting sign in 15–35% of patients. Focal neurologic deficits including hemiparesis, aphasia, or visual field defects are part of"}, {"id": "pubmed23n0291_6940", "title": "[Early diagnosis of herpes simplex virus encephalitis by single photon emission computed tomography (SPECT) in patients with normal MRI].", "score": 0.009900990099009901, "content": "Since treatment of herpes simplex virus encephalitis (HSVE) is most effective when started early, a sensitive and specific method for early diagnosis would be of great benefit. MRI and CT are commonly used for this purpose. In this study, we presented two patients who had serologically confirmed HSVE and had normal CT and MRI, but were diagnosed as having HSVE by means of SPECT in the early stage. Case 1 was a 56-year-old man who suddenly developed alexia. On admission, physical and neurological examination were unremarkable except for alexia, agraphia, acalculia, and left-right disorientation. Brain CT, MRI, and cerebral angiography were all normal. However, SPECT showed hyperaccumulation of 99m Tc-HM-PAO in the right temporal-occipital area. On the 5th hospital day, he became comatose. CSF study revealed marked pleocytosis. Even then, MRI including Gd-enhanced study was normal while SPECT continued to show hyperaccumulation. Detection of herpes simplex virus DNA in CSF by polymerase chain reaction was negative. Anti-HSV antibody titer in CSF and serum confirmed intrathecal production of the antibody on the 14th hospital day. Abnormal accumulation of tracer in SPECT returned to normal on the 31st day when he was alert but still had a mild Gerstman syndrome. Case 2 was a 61-year-old man with disturbance of consciousness, mental dysfunction, and generalized convulsion. He was diagnosed as having HSVE by means of CSF pleocytosis, detection of HSV DNA in CSF by polymerase chain reaction, and presence of anti-HSV antibody in the CSF. CT and MRI again revealed no abnormality while SPECT clearly showed hyperaccumulation in the left temporal lobe in an early stage. Hyperaccumulation of lipophilic tracer on SPECT study, especially in the temporal lobes, has been reported in the early stage of HSVE by previous investigators. Unlike MRI or enhanced CT, the increased tracer accumulation in SPECT does not reflect disruption of the blood-brain-barrier or inflammatory edema, but reflects hyperperfusion or some other HSVE related abnormality which is currently unknown. From these observations, we suggest that local hyperperfusion occurs before local inflammation, and that SPECT is the most useful scanning method for early diagnosis of HSVE when this disease is clinically suspected."}, {"id": "pubmed23n0211_11561", "title": "[Current therapeutic orientation in intracranial suppuration].", "score": 0.009900990099009901, "content": "Changes in diagnosis and management of intracranial infections have been studied on a continuous series of 102 cases 1968 through 1980. Use of CT scan has not increased the number of patients diagnosed in acute phase (before the fifteenth day) but has increased the rate of case observed before the second day (37 p. cent with and 27 p. cent without the CT scan). However the neurological status and specially the state of consciousness has not changed. Four specific data of abscess diagnosis have been defined on 56 cases suspected to have an intracranial infection; 16 out of these cases have proven by surgery not to be infectious lesion. Evolution under treatment is best followed by CT. Final aspect on CT are not different after puncture or after excision. Average delay of disappearance of edema is 20 days and of abscess is 44 days. Importance of bacteriological study in the choice of antibiotics is underlined by comparison of bacteriological and clinical results. Since the introduction of a laboratory of bacteriology in the hospital in 1976, we observed a decrease of sterile cultures (11.5 p. cent and 56 p. cent before 1976), an increase of the number of germs identified in each case and specially anaerobic germs (40 p. cent after and 10 p. cent before 1976) and a decrease in mortality and sequelae (respectively 8,3 p. cent and 19.4 p. cent before 1976 and 11,5 p. cent and 33 p. cent before 1976). Use of CT scan and progress in bacteriological study have led us to simplify our surgical attitude in case of intracranial abscess: puncture as soon as the diagnosis is done on CT; antibiotics according to the bacteriological study and survey by CT in neurosurgical unit."}, {"id": "pubmed23n0780_6287", "title": "Paraneoplastic limbic encephalitis resembling acute herpetic encephalitis.", "score": 0.00980392156862745, "content": "Introduction. Paraneoplastic limbic encephalitis (PLE) is a rare disorder that typically follows a chronic or subacute course of personality changes, memory loss, seizures, and hallucinations. Early diagnosis is difficult and characteristic symptoms can be mimicked by a variety of conditions. We present a case of PLE, initially presenting as acute herpetic encephalitis. Case Presentation. A 56-year-old male was admitted for evaluation of acute onset headache, fever, and confusion. On neurological examination he was confused with MMSE score of 15/30. CSF analysis revealed marked lymphocytic pleocytosis. A possible diagnosis of acute herpetic encephalitis was rendered and patient was treated with acyclovir. CSF PCR was negative. Cranial MRI revealed bilateral hyperintense lesions in medial temporal lobes with contrast enhancement. Despite treatment with acyclovir patient was deteriorated; thus, a paraneoplastic syndrome was suspected. Chest CT showed a right paratracheal lymph node mass, while a biopsy revealed neuroendocrine lung cancer. Auto antibodies to Hu were also detected. The patient was treated with steroids and chemotherapy. Six months later, he had complete tumour remission and marked neurological improvement. Discussion. PLE can rarely invade acutely, being indistinguishable from herpetic encephalitis. Inclusion of PLE in the differential diagnosis of acute encephalitis is of great clinical significance. "}, {"id": "pubmed23n0627_15825", "title": "[Indication of neuro-imaging for the initial management and the follow-up of acute community-acquired bacterial meningitis].", "score": 0.00980392156862745, "content": "Lumbar puncture is the best way to prove bacterial meningitis. It should be performed without any delay if the diagnosis is suspected. Herniation is a rare complication of LP. CT is normal in most cases of purulent meningitis, including those complicated by a subsequent herniation; normal CT results does not mean that performing a LP is safe. Three main clinical features can help determine which patient is at risk of herniation and should have a CT before LP. This risk has to be determined rapidly in the emergency ward while assessing anamnestic data, localization signs or symptoms, and level of consciousness. Cranial imaging (mainly MRI) is useful in the course of bacterial meningitis. Patients who do not respond well to treatment or with atypical presentation, persistence of fever, or new neurological signs should undergo brain imaging; MRI and CT may identify subdural effusions, brain abscesses, empyemas, hydrocephaly, or brain parenchymal changes (cerebritis, infarction, hemorrhage). CT and MRI are useful to screen for an ENT cause of bacterial meningitis, and mandatory in case of pneumococcal meningitis. Numerous MRI sequences are useful to identify bacterial meningitis complications: SE T1 without and with gadolinium injection, SE T2, FLAIR, gradient-echo T2, diffusion weighted imaging, MR angiography."}, {"id": "pubmed23n0511_18996", "title": "[Multiple brain abscesses: a case report].", "score": 0.009747557195921169, "content": "10 days before admission a 45-year old female experienced general weakness, and T 38 degrees C. During that period she had no cardio-respiratory nor neurological complaints, and the temperature varied between 37.5 degrees C and 38 degrees C. Her medical history was unremarkable, without immunodeficiency. The day before admission she presented with left arm paresis and during the next day it progressed to paralysis. She had no headache. On admission the following diagnostic procedures were performed: the cranial CT scan showed two lesions (possibly meta lesions). Chest X-ray was normal. WBC=15x10(9)/L, ESR=90/120. On the second day following admission brain MRI showed multiple abscesses in both hemispheres, mostly in the gray/white junction. High doses of IV metronidasol, cephtriaxon and cipfloxacin were administered without obtaining specimens for micro-biological diagnosis. In next two days she developed coma, respiratory insufficiency and septic temperature. Brain surgery was not performed due to severe involvement of the brain with multiple abscesses. Repeated chest X-ray revealed bilateral pneumonia. A lethal outcome occurred on the third day, regardless of all efforts. Autopsy showed multiple brain abscesses as well as on the lungs and liver. A beta-hemolytic streptococcal infection was established. Prevention includes treatment of the infection source. The classic triad of headache, fever and focal deficit occur in less than 50% of patients. Even in such cases brain abscess must be reconsidered CT appearance of brain abscess is similar to that of neoplastic and other infectious and non infectious diseases--especially in the stage of early cerebritis. If the CT findings are not clear, MRI should be performed."}, {"id": "pubmed23n0304_5258", "title": "[Fatal brain stem infarction due to rupture of a brain abscess: a case report].", "score": 0.009708737864077669, "content": "We report a case of a brain abscess which initially presented with subcortical hematoma and ultimately resulted in fatal brain stem infarction due to its rupture into the subarachnoid space. A 50-year-old male was admitted to a nearby hospital with complaints of headache, fever, and sensory aphasia. He had ventricular septal defect found 15 years previously, sinusitis, and liver cirrhosis. Computerized tomographic (CT) scan revealed a left temporal subcortical hematoma. Gadolinium-DTPA enhanced magnetic resonance imaging (MRI) showed faint ring-like enhancement at the margin of the lesion. The left internal carotid angiogram demonstrated the vascular blush and early venous filling of the vein of Labbé. Administration of antibiotics and predonine resulted in resolution of fever within five days. MRI obtained 17 days after the onset showed typical ring-like enhancement. The mass was just adjacent to the lateral ventricle. The patient was transferred to our hospital for further examination and treatment 21 days after the onset. Fever had recurred 2 days before admission to our hospital. One day after admission, the patient began to vomit. About 15 hours following this symptom, he suddenly became comatose and tetraplegic. CT scan demonstrated a rupture of the abscess. Emergent drainage from the lateral ventricle and the abscess cavity was undertaken. Follow-up CT scan revealed multiple infarctions involving the upper brain stem and the bilateral thalamus. He died on the 29th day after the onset. The mechanisms of hemorrhage with a brain abscess and cerebral infarction after rupture of brain abscess are discussed. Hemorrhage with brain abscess is extremely rare. However, brain abscess should be considered as a possible etiology of an atypical hematoma. To avoid fatal rupture of the brain abscess, immediate treatment is essential. Once the rupture of the brain abscess occurs, its contents might cause vasospasm severe enough to cause cerebral infarction."}, {"id": "pubmed23n1162_7671", "title": "When time is short, and we are late!: A story of chronic meningitis.", "score": 0.009708737864077669, "content": "We often face situations when the exact etiological diagnosis of meningitis is difficult. The reason behind this is that many pathogens have similar clinical, radiological, and laboratory pictures. The low yield of the pathogen in cerebrospinal fluid (CSF), non-availability of detail tests in all corners of the world, delay in availability of reliable results (like cultures), and difficulty in performing confirmatory tests like brain biopsy (in inconclusive cases) make the job of a clinician challenging. We report here a case where a late diagnosis of a disease owing to inconclusive results leads to dissemination. The complications following the introduction of the treatment based on presumption lead to further difficulty. We remained inclined to our diagnosis based on clinical judgement, acknowledged and managed the inflammatory changes secondary to the infection, and finally won the long battle. So, sometimes we need to make decisions based on clinical grounds. We need to depend on the fact that uncommon presentations of common diseases are commoner than a common presentation of uncommon diseases."}, {"id": "pubmed23n1130_10328", "title": "[COVID-19 and herpesvirus encephalitis].", "score": 0.009615384615384616, "content": "The SARS-CoV-2 virus, which causes COVID-19, could give rise to damage the nervous system. Many studies have been conducted on this topic, but few have focused specifically on encephalitis. The effect of SARS-CoV-2 on the clinical expression of other neurotropic viruses, such as Herpesviridae, is unknown. We describe the cases of two young men (39 and 18 years old) in whom SARS-CoV-2 had been detected -reverse transcription polymerase chain reaction (RT-PCR)-, and with a clinical diagnosis and cerebrospinal fluid (CSF) analysis consistent with encephalitis. The first patient had a positive PCR for varicella zoster virus in CSF, while the second had a positive PCR for herpes simplex virus types 1 and 2. The first patient, who was recently diagnosed with human immunodeficiency virus, presented with fever, headache, vomiting, cough, inappropriate behaviour and epileptic seizures; the second was seen to have fever, headache, myalgia and exanthema. Both offered the same laboratory findings (lymphopenia and high interleukin 6). CSF showed pleocytosis with a predominance of monomorphonuclear cells, hyperproteinorrachia and normal glycorrhachia. A cranial CT scan showed only mild diffuse cerebral oedema in the first case. Both cases were treated with corticosteroids, antibiotics and acyclovir. The second progressed favourably, while the first did not. Little is known about co-infection of SARS-CoV-2 with neurotropic viruses, such as Herpesviridae, and we have only limited evidence of direct neurological involvement of SARS-CoV-2, due to the technical difficulty of detecting it in the nervous system, thus making it important to take co-infection into account in order to be able to establish an early diagnosis and treatment to improve prognosis."}, {"id": "pubmed23n0761_17939", "title": "[Initial patient assessment of infectious diseases and diagnostic steps with fever].", "score": 0.009615384615384616, "content": "The initial assessment of patients with infectious diseases is challenging because of the extremely broad differential diagnosis as well as different host pathogen interactions influenced by a different immune status. The formal initial assessment, including the present and past medical history, thorough physical examination, clinical first impressions as well as routine laboratory analyses, is the basis of every preliminary diagnosis. Specific chief complaints have to be recognized in order to narrow down the differential diagnosis. In cases of life-threatening illnesses, such as septicemia, endocarditis, bacterial meningitis and severe pneumonia, the first diagnostic and therapeutic steps should be performed in a rapid sequence: bacterial blood samples, sputum and/or liquor samples are required and the initial antibiotic therapy has to be chosen empirically as the relevant bacterial spectrum related to the suspected illness must be covered. In less urgent cases it is recommended that a multi-step diagnostic approach be carried out which takes the differential diagnosis into account and prioritizes the probabilities. In the latter situation antibiotic treatment should be delayed to diagnose the infection correctly. Importantly, atypical courses must necessitate careful and critical reassessment of the diagnosis. "}, {"id": "pubmed23n0745_24678", "title": "Tuberculosis meningo-encephalitis with positive csf for KB and slowly favourable evolution. Case report.", "score": 0.009523809523809525, "content": "Meningoencephalitis produce by Koch bacillus is a disease including various symptoms, with often atypical onset and evolution, and with an etiological diagnosis being rarely established. It therefore remains a disease with severe prognosis, despite adequate treatment. a 47 years old male from Neamt County, with chronic otitis in his recent medical history, comes to the emergency room for fever, severe headache, confusion, equilibrium disorders, influenced health status, and meningeal irritation signs. Given his otitis and antibiotic therapy received in recent medical history, the patient was initially diagnosed and treated for beheaded bacterial meningitis (348 elements/mm3, 89% lymphocytes, 5% granulocytes, proteinorrhachia 1.54 g/l). Clinical and paraclinical evolution was poor with persistent fever and headache, impaired mental state, and the spinal puncture showed increased pleocytosis (725 elements/mm3), lymphocytosis (78%), increased proteinorrhachia (2.12 g/l), and low levels of glycorrhachia and chlorurorrhachia. Tuberculosis meningoencephalytis was suspected (confirmed after 3 weeks through cultures on Lowenstein-Jensen medium: positive for Mycobacterium tuberculosis), and a tuberculostatic treatment associating four drugs and intravenous Ciprofloxacin was started. Although the treatment included substances against cerebral edema and neurotrophic agent, his evolution showed persistent headache syndrome. We noticed very slow decrease in CSF pleocytosis (after a month) of treatment the patient still had 240 elements/mm3, 85% lymphocytesand 15% granulocytes. Moreover, due to hepatic toxicity a reduction of the tuberculostatic doses was needed. The beginning of the tuberculosis infection symptoms is often surprising, with clinical signs regarding secondary determinations (meningoencephalitis) association with another infection (otitis--wrong diagnosis). as well as persistent yet slowly favorably evolving symptoms, both clinically and paraclinically (CSF parameters). The etiological agent is extremely rarely isolated, due to a very slow growth of the Koch bacillus on special culture mediums and also because such diagnosis is not often considered before starting the treatment."}, {"id": "pubmed23n0235_11555", "title": "A contribution to the rapid diagnosis of subdural empyema.", "score": 0.009523809523809525, "content": "The authors report their experience with CT scanning ina series of 4 cases of subdural empyema, over a period of 10 months; all the patients were male, aged 16 to 25 years. A review of the literature shows that this disease, in spite of the large variety and efficacy of the available antibiotics, is marked by a significant mortality and morbidity rate if the diagnosis is delayed. Study of the cases reported here, as well as those in the literature, attests that CT scanning of the brain is nowadays the method of choice for early diagnosis of these lesions, and for timing of appropriate intervention according to variations in the development stage of the suppuration. From the study of this series of cases, it is clear that in the future, CT scanning of the brain will certainly allow the hope of a greater survival rate and an appreciable decrease in morbidity in cases of subdural empyema."}, {"id": "Neurology_Adams_5554", "title": "Neurology_Adams", "score": 0.009487837495933783, "content": "The infection may take the form of a brainstem encephalitis, or “rhombencephalitis,” specifically with several days of headache, fever, nausea, and vomiting followed by asymmetrical cranial-nerve palsies, signs of cerebellar dysfunction, hemiparesis, quadriparesis, or sensory loss. Respiratory failure has been reported. Of 62 cases of Listeria brainstem encephalitis reported 25 years ago by Armstrong and Fung 8 percent were in immunosuppressed patients, however, in the current era 20 percent or fewer of affected patients are immunocompetent. In the elderly, no additional cause of immune disorder appears to be necessary. Meningeal signs were present in the only half the patients in the above mentioned series, and the spinal fluid often showed misleadingly mild abnormalities. CSF cultures yielded Listeria in only 40 percent of cases (blood cultures were even more often normal). Consistent with our experience, the early CT scan was often normal; MRI, however, has revealed abnormal"}, {"id": "Neurology_Adams_5888", "title": "Neurology_Adams", "score": 0.009484691549100146, "content": "Diagnosis The CSF findings are much the same as in aseptic meningitis (lymphocytic pleocytosis, mild protein elevation, normal glucose values). Occasionally, early sampling of CSF may show few or no cells and subsequent tests may be more typical of an inflammatory disorder. Recovery of virus from blood or CSF is usually not possible and PCR testing is routinely only applied during local epidemics and for the detection of herpes viruses. Recently, it has been possible to use next generation sequencing to identify obscure causes of encephalitis. However, antiviral immunoglobulin (Ig) M antibody is present in the serum and CSF within the first days of symptomatic disease and can be detected and quantified by means of ELISA, making it preferable to other testing serologic for specific diagnosis. Some patients have not developed antibodies by the time of admission to the hospital and the test may have to be repeated in several days. The MRI may be normal or show signal changes and edema in"}, {"id": "pubmed23n0782_20812", "title": "Neurocysticercosis in a 23-year-old Chinese man.", "score": 0.009433962264150943, "content": "Male, 23 FINAL DIAGNOSIS: Neurocysticerosis Symptoms: Diplopia • fever • headache • insomnia • neck stiffness • vomiting Albendazole Clinical Procedure: - Specialty: Neurology. Challenging differential diagnosis. Neurocysticercosis is a brain infection caused by the larval stage of the tapeworm Taenia (T.) solium. It is the most important parasitic disease of the human central nervous system and represents the most common cause of acquired epilepsy in developing countries. Here, we report the case of a 23-year-old Chinese man who presented to the emergency department with a 7-day history of helmet headache radiating to the nuchal region and associated with vomiting, confusion, and fever. Cerebrospinal fluid (CSF) was clear, with increased pressure, lymphocytic pleocytosis, decreased glucose, and increased protein levels. Bacterial antigen detection test on CSF was negative, as were CSF bacterial and fungal cultures. Despite broad-spectrum antibiotic and antiviral therapy, the patient still complained of insomnia, diplopia, headache, neck stiffness, and pain in the sacral region. A second LP was performed and CSF had the same characteristics as the first LP. A brain and spinal cord MRI revealed widespread arachnoiditis and small septated cysts with CSF-like signal in the cisterna magna, within the fourth ventricle, and at the level of L3-L4. Cysticercus-specific immunoglobin G antibodies were detected by ELISA in the CSF. The patient received albendazole (15 mg/kg/day) and dexamethasone (5 mg/day) for 4 weeks, with progressive resolution of neurological symptoms. This case shows that, even if rare, neurocysticercosis may be responsible for meningeal symptoms and should be included in the differential diagnosis, especially in patients from endemic countries."}, {"id": "pubmed23n0233_9522", "title": "Fifteen-year review of the mortality of brain abscess.", "score": 0.009433962264150943, "content": "Ninety consecutive cases of brain abscess admitted to this center between 1964 and 1978 have been reviewed. The overall mortality has fallen in three consecutive 5-year periods from 42 to 21 to 9.7%. A number of factors seem to be responsible for this. Early surgical intervention was associated with the reduction in mortality between the first and second 5-year periods. Recognition of the significance and extent of cerebral edema, confirmed since computed tomographic (CT) scans have been available, led to a greater use of steroids during the last 5-year period, but the number of patients thus treated was too small to permit an assessment of any effect on mortality. There is no evidence to suggest a change in the natural history of the disease, and surgical management has not altered significantly. Experience with CT scanning in this center in the diagnosis of brain abscess is limited. It is therefore not possible no assess whether any improvement in mortality may have arisen from the early and accurate diagnosis obtainable with this technique. Improvement in culture technique has been of major importance, leading to a better understanding of the bacteriology of brain abscesses. This has allowed a more rational antibiotic program to be instituted, in particular the use of agents active against obligate anaerobes."}, {"id": "pubmed23n0585_15464", "title": "Traumatic pseudoaneurysm of the middle meningeal artery: possible indicators for early diagnosis in the computed tomography era.", "score": 0.009345794392523364, "content": "Traumatic pseudoaneurysms of the middle meningeal artery, which are associated with high mortality, are difficult to detect early by CT. We provide serial CT scans to show the steps of their formation and suggest characteristics that could be useful in the detection. A 25-year-old man was initially in deep coma had an anisocoric pupil after a traffic accident. Brain CT showed basal skull fracture and traumatic subarachnoid hemorrhage with severe brain swelling. Emergent decompressive craniectomy was performed, and 2 days later, an EDH appeared at the left temporal fossa. Careful examination of the image revealed a hypodense nodule inside the acute hematoma. He underwent craniotomy to remove the hematoma. Serial CT of the residual hematoma showed the gradual development of an organized hematoma around the hypodense nodule. The nodule had low density, which was strongly enhanced on CT after injection of contrast medium. The nodule was highly suspected to be a vascular lesion. A middle meningeal artery pseudoaneurysm was discovered through a 3-dimensional computed tomographic angiography. He underwent another craniotomy to remove the pseudoaneurysm. The diagnostic approach was CT, 3-dimensional CT, and craniotomies. Four CT findings may be useful for early diagnosis: (1) basal skull fracture in the temporal region; (2) hypodense nodule within an acute hematoma; (3) hypodense nodule within an organized and encapsulated hematoma; and (4) strong and homogenous enhancement of the hypodense nodule within an organized and encapsulated hematoma. Three-dimensional computed tomographic angiography is an effective and noninvasive tool to confirm this diagnosis."}, {"id": "pubmed23n0263_7382", "title": "[The characteristics of the present-day clinical course of tuberculous meningitis].", "score": 0.009345794392523364, "content": "Clinical patterns of tuberculous meningitis have been analyzed for 32 admissions to the bacterial meningitis department of the 2nd Moscow Infection Hospital in 1983-1991. Early diagnosis of tuberculous meningitis caused great difficulties because of rare cases of tuberculous history, atypical symptoms (an acute onset, in particular), an obscure meningeal syndrome, rare neurological symptoms, atypical liquor characteristics (frequent neutrophil pleocytosis, a small protein rise, normal glucose). Secondary bacterial meningitides presented most serious difficulties for differential diagnosis. So did cerebral abscesses and viral meningitis. Antituberculous therapy should be started at first sings of tuberculous nature of meningitis as the disease outcomes are determined to a large extent by early administration of proper treatment."}, {"id": "pubmed23n0718_9589", "title": "Two cases of dengue meningitis: a rare first presentation.", "score": 0.009259259259259259, "content": "Dengue, a mosquito-borne disease caused by a flavivirus, is recognized in over 120 countries with 3.6 billion people living in areas at risk. Neurological manifestations are infrequently reported as clinical consequences of dengue infection. Though severe dengue may be associated with meningoencephalitis, meningitis is a rare initial presentation of otherwise uncomplicated dengue fever. We report two adult patients who presented with fever, headache, and nuchal rigidity without the typical symptoms of dengue infection. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis in one and slight neutrophilic pleocytosis in the other with a normal glucose value and negative bacterial cultures. Dengue was suspected because thrombocytopenia was symptomatic in one patient and documented during the hospital course, and was confirmed by demonstration of IgM antibody in the cerebrospinal fluid samples specific for dengue in both cases. Our report demonstrates that meningitis with or without encephalitis can be the first manifestation of dengue infection. In endemic areas, dengue infection should be considered as a probable etiological agent of meningitis. Regular monitoring of platelet count can be an invaluable diagnostic screening tool. In appropriate clinical settings detection of anti-dengue IgM both in serum and in CSF may lead to correct diagnosis."}, {"id": "pubmed23n0351_16459", "title": "[Meningitis (II)--acute bacterial meningitis].", "score": 0.009259259259259259, "content": "Acute meningitis is a medical emergency, particularly in patients with rapidly progressing disease, mental status changes or neurological deficits. The majority of cases of bacterial meningitis are caused by a limited number of species, i.e. Streptococcus pneumoniae, Neisseria meningitis, Listeria monocytogenes, group B Streptococci (Streptococcus agalactiae), Haemophilus influenzae and Enterobacteriaceae. Many other pathogens can occasionally cause bacterial meningitis, often under special clinical circumstances. Treatment of meningitis includes two main goals: Eradication of the infecting organism, and management of CNS and systemic complications. Empiric therapy should be initiated without delay, as the prognosis of the disease depends on the time when therapy is started. One or two blood cultures should be obtained before administering the first antibiotic. Empiric therapy is primarily based on the age of the patient, with modifications if there are positive findings on CSF gram stain or if the patient presents with special risk factors. It is safer to choose regimens with broad coverage, as they can usually be modified within 24-48 hours, when antibiotic sensitivities of the infecting organism become available. Adjunctive therapy with dexamethasone is also administered in severely ill patients concomitantly with the first antibiotic dose. In patients who are clinically stable and are unlikely to be adversely affected if antibiotics are not administered immediately, including those with suspected viral or chronic meningitis, a lumbar puncture represents the first step, unless there is clinical suspicion of an intracerebral mass lesion. Findings in the CSF and on CT scan, if performed, will guide the further diagnostic work-up and therapy in all patients."}, {"id": "First_Aid_Step2_461", "title": "First_Aid_Step2", "score": 0.009223972210043506, "content": "MRI may demonstrate a contrast-enhancing lesion in the temporal lobe (in HSV). HSV encephalitis requires immediate IV acyclovir. CMV encephalitis is treated with IV ganciclovir +/– foscarnet. Give doxycycline for suspected Rocky Mountain spotted fever, Lyme disease, or ehrlichiosis. A focal, suppurative infection of the brain parenchyma, usually with a “ringenhancing” appearance due to fbrous capsule. The most common infective organisms are streptococci, staphylococci, and anaerobes; multiple organisms are often implicated (80–90% of cases are polymicrobial). Nonbacterial Although other medications may be used, rifampin is the frequently tested prophylaxis of choice for close contacts of patients with meningococcal meningitis. The presence of RBCs in CSF without a history of trauma indicates HSV encephalitis. HSV encephalitis is associated with high morbidity. PCR is highly sensitive and specific. A full course of IV acyclovir is mandatory."}, {"id": "pubmed23n0818_22025", "title": "Parafalcine empyema, a tricky infectious cause of headache: a case report.", "score": 0.009174311926605505, "content": "Headache caused by subdural empyema is usually associated with fever and symptoms and/or clinical signs of meningeal irritation and increased intracranial pressure. We describe a patient with headache with absence of these signs or symptoms of meningeal irritation or intracranial pressure, who turned out to have a parafalcine subduralempyema. A 28-year-old man had headache for 2 weeks, which had started with visual symptoms with duration of 5 minutes. Two days later, he developed fever. During these 2 weeks, he had recurrence of visual symptoms for 4 times, with duration of several minutes.Neurologic examination at presentation on the emergency department showed no meningeal irritation or papilledema. However, on closer examination, a limited homonymous hemianopsia on the left side and a drift of the left leg were found. Magnetic resonance imaging showed parafalcine subdural empyema on the right side of the falx and a small brain abscess right occipitally. Neuronavigated craniotomy was performed, which confirmed the presence of empyema and allowed culture of the specimens. Streptococcus milleri group was cultured,which allowed narrowing of the antibiotic therapy to Benzylpenicillin12 million entities per 24 hours. Headache and subdural empyema diminished during treatment, and at follow-up 12 weeks after start of treatment, patient had no remaining complaints. Parafalcine-located subdural empyema can present without presence of clear localizing symptoms or signs like meningeal irritation and increased intracranial pressure. When headache is accompanied with fever, one should extensively question neurologic symptoms, and a thorough neurologic examination should be done."}, {"id": "pubmed23n0240_11765", "title": "[Brain abscesses. Value of computed tomography. A review of seven cases (author's transl)].", "score": 0.009174311926605505, "content": "Based on a series of seven cases of subtentorial abscess, the authors analyze the results of different methods of exploration. Though in certain clinical conditions (intracranial hypertension and meningeal infections) the etiology is of no consequence, in most cases of definite diagnosis can be made of a space-occupying lesion by the use of EEG, arteriography, and scintigraphy examinations, without establishing the precise nature of the affection. As expected, computed tomography appears to be the most reliable examination. Diagnosis was confirmed by this method in 6 of the 7 cases, and it also enabled the number, size, and location of the lesions to be determined. Typical appearances after injection of an iodized contrast medium revealed the development of an abscess following the intracerebral infection, and determined the time for surgical intervention. In spite of intensive care and antibiotic therapy, an abscess remains a \"delayed-action bomb\", with poor prognosis, requiring drainage or surgical excision as soon as conditions are appropriate. The mortality and morbidity of this rare, and therefore poorly recognized, affection should improve with systematic use of the scanner during meningeal infections."}, {"id": "pubmed23n0855_9691", "title": "Paraneoplastic limbic encephalitis with associated hypothalamitis mimicking a hyperdense hypothalamic tumor: a case report.", "score": 0.00909090909090909, "content": "Paraneoplastic limbic encephalitis is an uncommon association of common malignancies such as small cell lung carcinoma, testicular teratoma, and breast carcinoma. The nonspecific nature of the clinical presentation, lack of freely available diagnostic markers, and requirement for advanced imaging techniques pose a great challenge in the diagnosis of this disease in resource-poor settings. A 64-year-old previously healthy Sri Lankan man was admitted to the general medical unit with subacute memory impairment regarding recent events that had occurred during the previous 3 weeks. Initial noncontrast computed tomography of the brain revealed a hyperdensity in the hypothalamic region surrounded by hypodensities extending toward the bilateral temporal lobes; these findings were consistent with a possible hypothalamic tumor with perilesional edema. The patient later developed cranial diabetes insipidus, which was further suggestive of hypothalamic disease. Interestingly, gadolinium-enhanced magnetic resonance imaging of the brain showed no such lesions; instead, it showed prominent T2-weighted signals in the inner mesial region, characteristic of encephalitis. The possibility of tuberculosis and viral encephalitis was excluded based on cerebrospinal fluid analysis results. Limbic encephalitis with predominant hypothalamitis was suspected based on the radiological pattern. Subsequent screening for underlying malignancy revealed a mass lesion in the right hilum on chest radiographs. Histological examination of the lesion showed small cell lung cancer of the \"oat cell\" variety. We suggest that the initial appearance of a hyperdensity in the hypothalamus region on noncontrast computed tomography is probably due to hyperemia caused by hypothalamitis. If hypothalamitis is predominant in a patient with paraneoplastic limbic encephalitis, magnetic resonance imaging will help to differentiate it from a hypothalamic secondary deposit. Limbic encephalitis should be considered in a patient with computed tomographic evidence of a central hyperdensity surrounded by bitemporal hypodensities. This pattern of identification will be useful for early diagnosis in resource-poor settings."}, {"id": "pubmed23n0871_23765", "title": "[Not Available].", "score": 0.00909090909090909, "content": "In spite of modern antibiotic treatment, bacterial meningitis still has a dubious prognosis. Secondary complications are responsible for death or permanent neurologic deficits. The indication for imaging is 2-fold. Beside the search for a source of infection, an early detection of secondary complications is attempted.We report about a patient with hematogenous pneumococcal meningitis, who developed a subdural empyema and vascular stenoses of the basal cerebral arteries. These changes as well as the resulting infarctions were detected with MRI in an early state. Thus, an adequate therapy could be initiated. Especially FLAIR images, diffusion-weighted sequences and a time of flight MRA proved to be valuable in this setting. "}, {"id": "pubmed23n0748_617", "title": "Pearls and oy-sters: tuberculous meningitis: not a diagnosis of exclusion.", "score": 0.009009009009009009, "content": "A 21-year-old man presented to his local emergency department with 5 days of headache, which was dull, occipital, bilateral, nonthrobbing, and progressively worsening. It was associated with mild fever, photophobia, and neck pain and stiffness. He had no history of headache, chronic illness, recent vaccinations, cutaneous rash, cough, diarrhea, arthralgia, or myalgia. He was from Ecuador and had been living in the United States for less than 1 year. He had been incarcerated while in Ecuador. Sublingual temperature on admission was 102.6°F. Other vital signs were within normal limits. On physical examination, he appeared thin but not cachectic. He had meningismus and photophobia, but no papilledema and his mental status was alert and attentive. There were no focal neurologic deficits. CSF contained red blood cells: 24 × 10(3)/μL; white blood cells: 85/μL (lymphocytic predominant); protein: 128 mg/dL; and glucose: 48 mg/dL (CSF/serum glucose ratio = 0.53). CSF Gram stain and cultures, PPD test, and blood and urine cultures were all negative. CT scan of the head on day of admission was entirely normal. MRI without gadolinium contrast showed a single punctate T2 hyperintensity in the left frontal periventricular white matter. Chest radiograph was clear. He received empiric vancomycin, ceftriaxone, and acyclovir. Corticosteroids were not given. The patient did not improve with antibiotics and continued to be intermittently febrile. On day 5, he became abruptly more somnolent, then comatose, opening eyes only to pain, his pupils were 5 mm and reactive, he had intact brainstem reflexes, withdrawing both arms and legs. Emergent head CT showed development of hydrocephalus and a ventriculoperitoneal shunt was emergently placed. The neurologic examination did not improve after shunt placement, and repeat head CT showed increased hydrocephalus with bilateral cerebral infarcts. On day 11, he was transferred to Columbia University Medical Center for intensive care. He was febrile and comatose. He did not open his eyes to pain, pupils were 7 mm minimally reactive, brainstem reflexes were intact, and he exhibited extensor posturing to pain. Mannitol was given, corticosteroid therapy was started, and an extraventricular drain was placed. The next day, his right pupil was 8 mm and nonreactive. MRI showed diffuse contrast enhancement of the arachnoid, extensive infarction of basal ganglia, midbrain, and pons, and small ring-enhancing lesions in the cerebellum (figure 1, A-D). Repeat lumbar puncture showed red blood cells: 550 × 10(3)/μL; white blood cells: 250/μL (14% neutrophils, 80% lymphocytes, 6% monocytes); protein: 65 mg/dL; and glucose: &lt;10 mg/dL (CSF/serum glucose ratio = 0.08). CSF testing for Cryptococcus and toxoplasmosis was negative. CSF acid fast bacilli (AFB) smear was negative ×2, and CSF nucleic acid amplification test was also negative for tuberculosis. Serum HIV test was negative. Not until 14 days after initial presentation and 3 days after transfer to the intensive care unit was antituberculosis therapy finally started, because the pattern of infarcts on the MRI suggested basilar meningitis and he had not improved on broad-spectrum antibiotics. That same day, the first sputum AFB smear was positive, as were all succeeding daily sputum AFB smears. Tuberculosis nucleic acid amplification was positive from the sputum, but persistently negative from the CSF. Daily portable chest radiographs had been normal (read as likely atelectasis), but chest CT showed dense consolidations in the left lung and diffuse micronodular opacities throughout both lungs. Two days later, only 21 days after the onset of his headache, the patient died of cardiopulmonary arrest secondary to transtentorial cerebral herniation. Thirteen days later, the CSF culture became positive for Mycobacterium tuberculosis sensitive to streptomycin, isoniazid, ethambutol, rifampin, and pyrazinamide."}, {"id": "First_Aid_Step2_456", "title": "First_Aid_Step2", "score": 0.009009009009009009, "content": "CT or MRI to rule out other diagnoses. CBC may reveal leukocytosis; CSF fndings vary (see Table 2.8-4). TAB LE 2.8-3. Causes of Meningitisa,b GBS E. coli/GNRs Listeria S. pneumoniae Neisseria meningitidis H. infl uenzae type b Enteroviruses N. meningitidis Enteroviruses S. pneumoniae HSV S. pneumoniae GNRs Listeria N. meningitidis a Causes in HIV include Cryptococcus, CMV, HSV, VZV, TB, toxoplasmosis (brain abscess), and JC virus (PML). b Note: The incidence of H. infl uenzae meningitis has ↓ greatly with the introduction of the H. infl uenzae vaccine in the last 10–15 years. TABLE 2.8-4. CSF Prof les"}, {"id": "pubmed23n0325_2840", "title": "[A 64-year-old woman with progressive gait disturbance and dementia for one year].", "score": 0.008928571428571428, "content": "We report a 64-year-old Japanese woman who died one year after the onset of progressive gait disturbance and dementia. She noted a difficulty in holding a glass and hand tremor in June of 1996 when she was 63 years old. In July of 1996, she tended to lean toward left when she walked. She also noted truncal titubation. In November of 1996, she started to have visual hallucination and delusion in which she said \"I see something is flying on the wall.\", \"Somebody has come into my room\", and things like that. She was admitted to our service on November 22, 1996. On admission, she was alert and general physical examination was unremarkable. Neurologic examination revealed disturbance in recent memory. Hasegawa's dementia rating scale was 22/30. She showed vivid visual hallucination with colors in which she saw faces of dwarfs and angels, a space ship, and others. Higher cerebral functions were normal. She showed left oculomotor palsy which was a sequel of an aneurysm and subarachnoid hemorrhage nine years before. Otherwise cranial nerves were unremarkable. She showed ataxic gait, limb ataxia, truncal titubation, and postural hand tremor. She had no weakness and no muscle atrophy. Deep tendon reflexes were within normal limits. Plantar response was flexor. Sensation was intact. Laboratory examination was also unremarkable. Complete survey for occult malignancy was negative. CSF was under a normal pressure and cell count was 1/microliter, total protein 27 mg/dl, and sugar 68 mg/dl. Cranial CT scan was unremarkable. MRI was not obtained because of the presence of an aneurysm clip in the left internal carotid-posterior communication artery junction. She showed progressive deterioration in her mental function. By January 1997, she became unable to stand or walk with marked dementia. Repeated CSF exams and cranial CT scans were unremarkable. She suffered from several episodes of aspiration pneumonia. A trial of three days methylprednisolone pulse therapy was given starting on March 7, 1997, which was of no effect on her neurologic status. On March 28, 1997, she was intubated because of acute respiratory distress syndrome. In April 2, her body temperature rose to 38 degrees C. On April 9, 1997, her blood pressure dropped and resuscitation was unsuccessful. She was pronounced dead on the same day. The patient was discussed in a neurologic CPC and the chief discussant arrived at the conclusion that the patient had primary leptomeningeal lymphoma. Other possibilities entertained among the audience included brain stem encephalitis of unknown type, carcinomatous cerebellar degeneration plus limbic encephalitis, Creutzfeldt-Jakob disease, thalamic degeneration, and progressive multifocal leukoencephalopathy. Post-mortem examination revealed thickening and clouding of the leptomeninges; Gram-positive diplococci were found in the leptomeninges. This meningitis appeared to have been an complication in the terminal stage of her illness. Microscopic examination revealed astrocytosis in the midbrain tegmentum. Cerebral cortices showed only mild astrtocytosis. No cerebellar atrophy was seen and Purkinje cells were retained which excluded paraneoplastic cerebellar degeneration. Neuropathologic diagnosis was bacterial meningitis, however, the presence of brain stem encephalitis prior to the onset of bacterial meningitis could not be excluded. It is interesting to note that the diagnosis of the primary neurologic disease of this patient was not easy even after autopsy. As autopsy permission was obtained only for the brain, it was not clear whether or not this patient had an occult malignancy somewhere in her body, however, there was no evidence to indicate paraneoplastic degeneration of the central nervous system. As the patient did not have meningeal signs until one month before her death, it is difficult to ascribe her entire neurologic problems to her meningitis. Finally, her visual hallucination was vivid and colorful; we thought this might have been"}, {"id": "pubmed23n0597_15824", "title": "[Fever without a source. Diagnostic difficulties in invasive bacterial diseases in children observed on bacterial meningitis model].", "score": 0.008928571428571428, "content": "Acute infection of the central nervous system is the most common cause of fever associated with signs and symptoms of CNS disease in children. Unfortunately most of these symptoms are non-specific. Specific signs appear very often late in the course of illness. 761 cases of bacterial meningitis in children above 1 month of life were investigated. Only 46.1% of cases were correctly diagnosed during the first doctor's exam. We found that correct diagnosis depends on time between the first symptoms of illness and the first physician visit. If this period of time is below 16 hours the risk of wrong diagnosis is very high due to lack of specific signs of CNS infection. In many cases only fever is present then."}, {"id": "wiki20220301en003_97415", "title": "Headache", "score": 0.008853353464989791, "content": "One recommended diagnostic approach is as follows. If any urgent red flags are present such as visual loss, new seizures, new weakness, new confusion, further workup with imaging and possibly a lumbar puncture should be done (see red flags section for more details). If the headache is sudden onset (thunderclap headache), a computed tomography test to look for a brain bleed (subarachnoid hemorrhage) should be done. If the CT scan does not show a bleed, a lumbar puncture should be done to look for blood in the CSF, as the CT scan can be falsely negative and subarachnoid hemorrhages can be fatal. If there are signs of infection such as fever, rash, or stiff neck, a lumbar puncture to look for meningitis should be considered. If there is jaw claudication and scalp tenderness in an older person, a temporal artery biopsy to look for temporal arteritis should be performed and immediate treatment should be started. Neuroimaging"}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[414, 545]], "word_ranges": [[71, 93]], "text": "Advise a woman not to become pregnant when there are treatments that ensure a very low risk of thrombosis and/or fetal involvement?"}, "2": {"exist": true, "char_ranges": [[270, 390]], "word_ranges": [[48, 67]], "text": "if treatment is given, it should be during the entire pregnancy, prothrombotic status is not only during the puerperium,"}, "3": {"exist": true, "char_ranges": [[1011, 1246]], "word_ranges": [[172, 210]], "text": "we see that aspirin is used in case of phospholipid syndrome and that in the case of this woman, without previous thrombosis, without previous abortions (at least two are needed) and also being heterozygous, treatment is not necessary."}, "4": {"exist": true, "char_ranges": [[1011, 1246]], "word_ranges": [[172, 210]], "text": "we see that aspirin is used in case of phospholipid syndrome and that in the case of this woman, without previous thrombosis, without previous abortions (at least two are needed) and also being heterozygous, treatment is not necessary."}, "5": {"exist": true, "char_ranges": [[65, 157]], "word_ranges": [[13, 28]], "text": "acenocoumarol should NOT be given to a pregnant woman because of the risk of teratogenicity."}}, "full_answer": "Reading the answers, the first thing that is clear to us is that acenocoumarol should NOT be given to a pregnant woman because of the risk of teratogenicity. Anyone who does so during residency should be punished with 100 slaps on the back. We discard answer 5. Second: if treatment is given, it should be during the entire pregnancy, prothrombotic status is not only during the puerperium, so answer 2 discarded. Advise a woman not to become pregnant when there are treatments that ensure a very low risk of thrombosis and/or fetal involvement? Answer 1 discarded. We are left with the possibility of not giving any treatment because it is low risk or administering low-dose aspirin. It is true that factor V Leiden in heterozygosis is classified as low risk, who has not presented a previous thrombotic episode nor is there a combination with another thrombophilia; if we look at the CHEST guide, VIII edition and review what was said at the last congress of the Spanish Society of Gynecology and Obstetrics, we see that aspirin is used in case of phospholipid syndrome and that in the case of this woman, without previous thrombosis, without previous abortions (at least two are needed) and also being heterozygous, treatment is not necessary. Correct answer, 3.", "full_answer_no_ref": "Reading the answers, the first thing that is clear to us is that acenocoumarol should NOT be given to a pregnant woman because of the risk of teratogenicity. Anyone who does so during residency should be punished with 100 slaps on the back. We discard answer 5. Second: if treatment is given, it should be during the entire pregnancy, prothrombotic status is not only during the puerperium, so answer 2 discarded. Advise a woman not to become pregnant when there are treatments that ensure a very low risk of thrombosis and/or fetal involvement? Answer 1 discarded. We are left with the possibility of not giving any treatment because it is low risk or administering low-dose aspirin. It is true that factor V Leiden in heterozygosis is classified as low risk, who has not presented a previous thrombotic episode nor is there a combination with another thrombophilia; if we look at the CHEST guide, VIII edition and review what was said at the last congress of the Spanish Society of Gynecology and Obstetrics, we see that aspirin is used in case of phospholipid syndrome and that in the case of this woman, without previous thrombosis, without previous abortions (at least two are needed) and also being heterozygous, treatment is not necessary. [HIDDEN].", "full_question": "A 25-year-old woman who wishes to become pregnant and wants to know what treatment she should take during the eventual pregnancy, as she is a heterozygous factor V Leiden carrier. She has never had any thrombotic phenomena. The determination of this factor was performed as a family study after an episode of pulmonary embolism in a sibling. What treatment should be advised?", "id": 116, "lang": "en", "options": {"1": "Since pregnancy is a prothrombotic state, there would be a high risk of venous thromboembolism, so pregnancy should be discouraged.", "2": "Treatment with low molecular weight heparin at prophylactic doses should be carried out in the immediate puerperium, with optional follow-up during pregnancy.", "3": "Factor V Leiden in heterozygosis is a low-risk thrombophilia and there is no need for any treatment in pregnancy and puerperium.", "4": "Low-dose aspirin should be advised during pregnancy and puerperium.", "5": "Treatment with antivitamin K drugs (acenocoumarol) during pregnancy."}, "question_id_specific": 101, "type": "HEMATOLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0564_22420", "title": "[Thrombophilia and pregnancy].", "score": 0.019237883832778392, "content": "The risk of venous thromboembolism (VTE) increases up to 5-10-fold during pregnancy and VTE represents the first cause of maternal mortality. The annual incidence of VTE is 0.97 per 1000 women during pregnancy and 7.19 per 1000 in the puerperium. The risk is higher in carriers of inherited thrombophilia. Prophylaxis of VTE during pregnancy in thrombophilic women, is still controversial, whereas there is agreement on the used of LMWH or oral anticoagulants during puerperium. LMWH is suggested during pregnancy in antithrombin deficient women, compound heterozygotes for prothrombin G20210A and factor V Leiden, and homozygotes for these conditions, with no prior VTE. In heterozygotes for F V Leiden or prothrombin G20210A with no prior VTE surveillance is preferred during pregnancy and LMWH or OA during puerperium. For patients with APLAs and no prior VTE or fetal loss, one of the following approaches is suggested: prophylactic LMWH and/or low-dose aspirin, mini dose heparin, surveillance (7 degrees ACCP). Patients with APLAs and a history of thrombosis should receive therapeutic-dose LMWH or UH plus low-dose aspirin during pregnancy and long term OA postpartum. In women with prior VTE and inherited thrombophilia, prophylactic or intermediate-dose LMWH is recommended during pregnancy plus post-partum OA. Intermediate-dose LMWH during pregnancy is suggested in antithrombin-deficient women, compound heterozygotes for prothrombin G20210A and factor V Leiden, and homozygotes for these conditions."}, {"id": "pubmed23n0383_13526", "title": "Anticoagulation in pregnancy and the puerperium.", "score": 0.018518518518518517, "content": "For the management of acute thrombotic events in pregnancy therapeutic doses of low molecular weight heparins (LMWH) may be used, unless the shorter half-life of intravenous unfractionated heparin (UH) and predictable reversibility by protamine are important. Treatment should be continued up until delivery and into the puerperium. Pregnant women who have had an acute thrombotic event should be delivered by a specialist team. In the case of recent thrombosis, delivery should be planned and the time during which anticoagulation therapy is ceased around the time of delivery should be minimised. Therapeutic doses of LMWH contraindicate the use of regional anaesthesia, and a switch to intravenous UH before delivery may allow greater flexibility in this regard. Prophylactic doses of LMWH can be used to reduce the risk of recurrent thromboembolic events in pregnancy. The regimen used will depend on the previous history, the family history and the presence of risk factors, including the genetic and acquired causes of thrombophilia. Women with mechanical heart valves are at high risk during pregnancy and require therapeutic anticoagulation throughout pregnancy under the direction of experienced specialists. Low-dose aspirin can reduce the risk of recurrent pre-eclampsia by about 15%, but the role of UH and LMWH in the prevention of recurrent miscarriage or obstetric complications associated with uteroplacental insufficiency is still uncertain."}, {"id": "pubmed23n0811_15808", "title": "Diagnosis, treatment, and prevention of venous thromboembolism in pregnancy.", "score": 0.018375566370196342, "content": "Pregnancy and the puerperium put women at increased risk of venous thromboembolism (VTE) due to both baseline maternal risk factors and the development of pregnancy-related prothrombotic anatomic and physiologic changes. Pregnant women are at an approximately 5-fold increased risk of VTE compared with nonpregnant women, and the risk of VTE increases further (to ≥ 20-fold) in puerperium; risk remains increased until approximately 12 weeks postpartum. Pregnancy-related VTE accounts for about 10% of maternal deaths in the developed world. Clinicians should promptly evaluate any signs or symptoms suspicious for VTE, generally starting with ultrasound of the lower extremities. For treatment of women with established VTE, low molecular weight heparins (LMWHs) are preferred due to a favorable safety and efficacy profile. Unfractionated heparin (UFH) and potentially fondaparinux are alternatives. Warfarin should be avoided in the antepartum period due to teratogenicity, and the non-vitamin K oral anticoagulants are currently not recommended due to the lack of data. Low molecular weight heparin, UFH, and warfarin are all acceptable in the postpartum period and for breast-feeding women, but the non-vitamin K oral anticoagulants should be avoided. Prophylaxis (generally with LMWH or in some cases UFH) is recommended for women at highest risk of pregnancy-related VTE, such as those with inherited thrombophilias and a strong family or personal history of VTE. Prophylaxis with LMWH and aspirin is recommended for women with antiphospholipid syndrome. Clinicians should engage in multidisciplinary discussion, particularly around the time of delivery, to manage the details of anticoagulation in their pregnant patients. "}, {"id": "pubmed23n0481_13310", "title": "Anticoagulation during pregnancy.", "score": 0.018329427519250083, "content": "Venous thromboembolism (VTE) occurs infrequently but is a leading cause of illness and death during pregnancy and the puerperium. In the general population the incidence of pregnancy-associated VTE is approximately 1 in 1500 deliveries. The risk of VTE is five times higher in a pregnant than in a nonpregnant woman. Postpartum the VTE risk is even higher. Women with congenital abnormalities or persistent presence of antiphospholipid antibodies have an increased risk of VTE during pregnancy and the puerperium. Women with previous VTE have an approximately 3.5-fold increased risk of recurrent VTE during pregnancy. Heparin does not cross the placenta and is therefore the anticoagulant of choice. In case of acute thrombosis during pregnancy, treatment is performed in the same manner as for nonpregnant patients. There is an ongoing debate whether pregnant women with previous venous thrombosis should routinely receive prophylactic anticoagulation. Patients who have hereditary antithrombin deficiency, antiphospholipid antibodies, a combined abnormality, or a history of a severe thrombotic event (pulmonary embolism or extended deep vein thrombosis) should be advised to use prophylactic heparin during pregnancy, starting during the first trimester. Postpartum prophylaxis should be given to all women with an increased risk for VTE. A large body of evidence has been presented that hypercoagulability may cause recurrent abortions, stillbirth, and preeclampsia. There is no doubt that the antiphospholipid syndrome is strongly associated with fetal loss. The combination of heparin and aspirin significantly decreases the fetal loss rate during pregnancy and thus this is the treatment of choice in this patient group. Several studies indicate that women with recurrent miscarriage may benefit from heparin administration during pregnancy, however, data from controlled trials have not yet been published. In women with artificial heart valves, maternal and fetal complications are frequent despite anticoagulation, but oral anticoagulants can reduce the risk for maternal complications."}, {"id": "pubmed23n0552_2687", "title": "The risk of recurrent venous thromboembolism in pregnancy and puerperium without antithrombotic prophylaxis.", "score": 0.018162393162393164, "content": "Whether or not pregnant women with a previous episode of venous thromboembolism (VTE) should receive antithrombotic prophylaxis is a matter of debate. In order to estimate the rate of recurrent deep venous thrombosis (DVT) or pulmonary embolism (PE) during pregnancy and puerperium we retrospectively investigated a cohort of 1104 women with previous VTE; after a single DVT or isolated PE, 88 of them became pregnant at least once without receiving antithrombotic prophylaxis. Overall, 155 pregnancies and 120 puerperium periods without prophylaxis were recorded. There were nine recurrences during pregnancy and 10 during puerperium, with a rate of 5.8% [95% confidence interval (CI) 3.0-10.6] and 8.3% (95%CI 4.5-14.6) respectively. In pregnancy, the rate of recurrence was 7.5% (95%CI 4.0-13.7) if the first VTE was unprovoked, related to pregnancy or to oral contraceptive use, whereas no recurrence occurred if the first VTE was related to other transient risk factors. In puerperium, the rate of recurrence was 15.5% (95%CI 7.7-28.7) in women with a pregnancy-related first VTE, with a risk 3.9-times higher than in the remaining women. Inherited thrombophilia was not associated with a statistically significant increase in risk of recurrence in pregnancy or in puerperium, yet the rate of recurrence in puerperium was 14.2% (95%CI 5.7-31.4) in overall carriers of factor V Leiden and 30% (95%CI 10.7-60.3) in carriers with a pregnancy-related first VTE, with a risk 6.8 times higher than in women without thrombophilia and with a non pregnancy-related first VTE."}, {"id": "pubmed23n0752_15864", "title": "Favorable outcome under anticoagulant therapy in a high risk pregnancy case report and short review of the (recent) literature.", "score": 0.01721981014055486, "content": " The incidence of venous thromboembolism is significantly increased during pregnancy, recurrent venous thromboembolism being a serious complication because it is potentially life-threatening. According to recent ACCP guidelines, women with \"high-risk\" thrombophilias (e.g., homozygosity for factor V Leiden) who had a single prior episode of VTE treated with oral anticoagulants, should receive LMWH or UFH during pregnancy and puerperium, followed by resumption of long-term anticoagulants postpartum.We present the case of a young woman with a history of severe deep vein thrombosis of the inferior vena cava, occurring during oral contraceptive use. Subsequent investigation revealed homozygosity for Leiden mutation. She was treated with enoxaparin throughout gestation and 6 weeks postpartum and no complications appeared."}, {"id": "pubmed23n0647_3759", "title": "[Thrombophilia, preeclampsia and other pregnancy complications].", "score": 0.016950369891546364, "content": "The aim of this paper is to present the latest developments in therapy and prophylaxis of deep vein thrombosis and other pregnancy complications in women with inherited or acquired thrombophilia and in women with mechanical heart valves. The data presented in the paper have been extracted from the Current Contents database. It is well known that the hypercoagulable state in pregnant women, caused either by the physiological changes of pregnancy or by inherited thrombophilia, increases the risk of venous thromboembolism (VTE), pulmonary embolism (PE), preeclampsia, recurrent early and late fetal loss, intrauterine growth retardation (IUGR), placental abruption, and other less probable complications of pregnancy and its outcome. In women with mechanical heart valves, the risk of systemic embolism is also seen to increase during pregnancy. According to data analyzed, positive antiphospholipid antibodies (APLA) as well as anticardiolipin antibody and lupus anticoagulant (nonspecific inhibitor) positivity, homozygosity and heterozygosity for factor V Leiden mutation and heterozygosity for the prothrombin G20210A variant, MTHFR C677T variant homozygosity and hyperhomocysteinemia are in strong association with pregnancy complications and severe pregnancy outcome. The strongest association for late fetal loss was seen in women with protein S deficiency. In order to reduce such risks, anticoagulation therapy is administered throughout pregnancy. The antithrombotic agents available for the prevention and treatment of VTE during pregnancy and pregnancy complications include unfractionated heparin (UFH), low-molecular-weight heparin (LMWH) and aspirin. Vitamin K antagonists are contraindicated in pregnancy. Low-dose aspirin may have a role in the prevention of some pregnancy complications, although its safety in early pregnancy is uncertain. LMWH and UFH are quite safe and efficacious when properly selected, dosed and monitored. The efficacy and safety of LMWH have been demonstrated in the prevention and treatment of VTE in pregnancy. LMWH in association with aspirin administered throughout pregnancy have been shown to be associated with a lower risk of complications in women with APLA syndrome. Women at a high risk of preeclampsia are recommended to use low-dose aspirin throughout pregnancy. When there is a history of preeclampsia, the administration of anticoagulation therapy is not recommended as a prophylaxis in subsequent pregnancies, as the risk appears to be already decreased as compared with previous pregnancy. LMWH has probable advantages over UFH for the incidence of side effects. In pregnant women with mechanical heart valves, anticoagulant therapy during pregnancy should include assessment of additional risk factors for thromboembolism including valve type, position, and history of thromboembolism, and decision should also be strongly influenced by the patient's preferences. If the risk of thromboembolism in patients with mechanical heart valves is considered very high, and efficacy or safety of prophylaxis with UFH or LMWH is not satisfactory (older-generation prosthesis in the mitral position or history of thromboembolism), administration of vitamin K antagonists throughout pregnancy is recommended with replacement by UFH or LMWH close to delivery. It should be considered that limited effectiveness of UFH or LMWH in patients with mechanical heart valves might be due to inadequate dosing. The necessity of anticoagulation therapy in women with inherited or acquired thrombophilia is biologically plausible; nevertheless, optimum management in such cases remains unknown."}, {"id": "pubmed23n0448_2051", "title": "Thrombosis during pregnancy: risk factors, diagnosis and treatment.", "score": 0.01589323627540188, "content": "Venous thromboembolism occurs infrequently but is a leading cause of illness and death during pregnancy and the puerperium and remains a diagnostic and therapeutic challenge. In the general population the incidence of pregnancy associated VTE has been estimated to vary from 1 in 1000 to 1 in 2000 deliveries. The risk of VTE is five times higher in a pregnant woman than in a nonpregnant woman of similar age. Postpartum VTE is more common than antepartum VTE. Women with congenital abnormalities or persistent presence of antiphospholipid antibodies have an increased risk of VTE during pregnancy and the puerperium. In individuals with well defined hereditary thrombosis risk factors, such as the factor V:R506Q mutation, the factor II:G20210A variation, antithrombin-deficiency or protein C-deficiency, a relative risk of pregnancy associated VTE between 3.4 and 15.2 has been found. Women with previous VTE have an approximately 3.5 fold increased risk of recurrent VTE during pregnancy compared to non-pregnant periods. Our ability to diagnose deep-vein thrombosis clinically is generally poor and is further hampered during pregnancy since dyspnea, tachypnea, swelling and discomfort in the legs are common. Objective diagnosis is essential for treatment decisions. Exposure to radiation of less than 50,000 microGy (5 rad) has not been associated with a significant risk of fetal injury. Therefore, besides sonography, routine diagnostic procedures should be performed, if clinically necessary. Heparin does not cross the placenta and is therefore the anticoagulant treatment of choice during pregnancy. In case of acute new onset of thrombosis during pregnancy, treatment is performed like in non-pregnant patients with acute deep vein thrombosis or pulmonary embolism. There is ongoing debate, whether or not pregnant women with previous venous thrombosis should routinely receive prophylactic anticoagulation. In patients who have hereditary antithrombin deficiency, antiphospholipid antibodies, a combined abnormality or a history of a severe thrombotic event (pulmonary embolism, extended deep vein thrombosis) should be advised to use prophylactic heparin during pregnancy, starting during the first trimester. Post partum prophylaxis should be given in all women with an increased risk for VTE."}, {"id": "pubmed23n0601_23605", "title": "Venous thromboembolism, thrombophilia, antithrombotic therapy, and pregnancy: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition).", "score": 0.01575757575757576, "content": "This article discusses the management of venous thromboembolism (VTE) and thrombophilia, as well as the use of antithrombotic agents, during pregnancy and is part of the American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Grade 1 recommendations are strong and indicate that benefits do, or do not, outweigh risks, burden, and costs. Grade 2 recommendations are weaker and imply that the magnitude of the benefits and risks, burden, and costs are less certain. Support for recommendations may come from high-quality, moderate-quality or low-quality studies; labeled, respectively, A, B, and C. Among the key recommendations in this chapter are the following: for pregnant women, in general, we recommend that vitamin K antagonists should be substituted with unfractionated heparin (UFH) or low-molecular-weight heparin (LMWH) [Grade 1A], except perhaps in women with mechanical heart valves. For pregnant patients, we suggest LMWH over UFH for the prevention and treatment of VTE (Grade 2C). For pregnant women with acute VTE, we recommend that subcutaneous LMWH or UFH should be continued throughout pregnancy (Grade 1B) and suggest that anticoagulants should be continued for at least 6 weeks postpartum (for a total minimum duration of therapy of 6 months) [Grade 2C]. For pregnant patients with a single prior episode of VTE associated with a transient risk factor that is no longer present and no thrombophilia, we recommend clinical surveillance antepartum and anticoagulant prophylaxis postpartum (Grade 1C). For other pregnant women with a history of a single prior episode of VTE who are not receiving long-term anticoagulant therapy, we recommend one of the following, rather than routine care or full-dose anticoagulation: antepartum prophylactic LMWH/UFH or intermediate-dose LMWH/UFH or clinical surveillance throughout pregnancy plus postpartum anticoagulants (Grade 1C). For such patients with a higher risk thrombophilia, in addition to postpartum prophylaxis, we suggest antepartum prophylactic or intermediate-dose LMWH or prophylactic or intermediate-dose UFH, rather than clinical surveillance (Grade 2C). We suggest that pregnant women with multiple episodes of VTE who are not receiving long-term anticoagulants receive antepartum prophylactic, intermediate-dose, or adjusted-dose LMWH or intermediate or adjusted-dose UFH, followed by postpartum anticoagulants (Grade 2C). For those pregnant women with prior VTE who are receiving long-term anticoagulants, we recommend LMWH or UFH throughout pregnancy (either adjusted-dose LMWH or UFH, 75% of adjusted-dose LMWH, or intermediate-dose LMWH) followed by resumption of long-term anticoagulants postpartum (Grade 1C). We suggest both antepartum and postpartum prophylaxis for pregnant women with no prior history of VTE but antithrombin deficiency (Grade 2C). For all other pregnant women with thrombophilia but no prior VTE, we suggest antepartum clinical surveillance or prophylactic LMWH or UFH, plus postpartum anticoagulants, rather than routine care (Grade 2C). For women with recurrent early pregnancy loss or unexplained late pregnancy loss, we recommend screening for antiphospholipid antibodies (APLAs) [Grade 1A]. For women with these pregnancy complications who test positive for APLAs and have no history of venous or arterial thrombosis, we recommend antepartum administration of prophylactic or intermediate-dose UFH or prophylactic LMWH combined with aspirin (Grade 1B). We recommend that the decision about anticoagulant management during pregnancy for pregnant women with mechanical heart valves include an assessment of additional risk factors for thromboembolism including valve type, position, and history of thromboembolism (Grade 1C). While patient values and preferences are important for all decisions regarding antithrombotic therapy in pregnancy, this is particularly so for women with mechanical heart valves. For these women, we recommend either adjusted-dose bid LMWH throughout pregnancy (Grade 1C), adjusted-dose UFH throughout pregnancy (Grade 1C), or one of these two regimens until the thirteenth week with warfarin substitution until close to delivery before restarting LMWH or UFH) [Grade 1C]. However, if a pregnant woman with a mechanical heart valve is judged to be at very high risk of thromboembolism and there are concerns about the efficacy and safety of LMWH or UFH as dosed above, we suggest vitamin K antagonists throughout pregnancy with replacement by UFH or LMWH close to delivery, after a thorough discussion of the potential risks and benefits of this approach (Grade 2C)."}, {"id": "pubmed23n0357_22237", "title": "[Current management of thromboembolism in pregnancy and puerperium].", "score": 0.01497939636930616, "content": "Venous thromboembolism (VTE) remains the leading cause of maternal death. Today, various risk factors and conditions are known to increase the risk for VTE associated with pregnancy. Having identified the individual risk of a pregnant women, appropriate preventive measures can be taken. If VTE occurs during pregnancy, an appropriate immediate diagnostic work-up is essential in order to avoid further complications. For deep vein thrombosis (DVT) the diagnostic tool of choice is color-coded duplex-sonography, for pulmonary embolism (PE) perfusion/ventilation lung scan can be used. Integrating a detailed individual and family history, the presence of thrombophilia or other risk factors, a risk stratification can be undertaken. These risk categories are defined in the present paper and the appropriate treatment measures are described. As oral anticoagulants cross the placenta and may cause embryopathy in any trimester, oral anticoagulants should be avoided throughout pregnancy. Therefore, heparin is the anti-coagulant of choice for pregnant women, with low molecular weight heparins (LMWH) having distinctive pharmacological advantages over unfractionated heparins. Besides a potential for bleeding, the main side effects of heparin include heparin-induced thrombocytopenia which prompts for platelet monitoring, especially in the first weeks of heparin treatment, and, secondly, heparin-induced osteoporosis, which is a potential sequel of long-term heparin administration. Even though there are abundant reports in the literature on the use of LMWH in pregnant women, that show that they are safe and effective, LMWH are not specifically licensed for the use in pregnancy."}, {"id": "pubmed23n0448_175", "title": "Pregnancy-associated thrombosis.", "score": 0.014708886618998978, "content": "Venous thromboembolism (VTE) occurs infrequently but is a leading cause of illness and death during pregnancy and the puerperium. In the general population the incidence of pregnancy associated VTE is approximately 1 in 1500 deliveries The risk of VTE is five times higher in a pregnant than in a non-pregnant woman. Postpartum the VTE-risk is even higher. Women with congenital abnormalities or persistent presence of antiphospholipid antibodies have an increased risk of VTE during pregnancy and the puerperium. In individuals with well defined hereditary thrombosis risk factors, such as the factor V:R506Q mutation, the factor II:G20210A variation, antithrombin-deficiency or protein C-deficiency, a relative risk of pregnancy associated VTE between 3.4 and 15.2 has been found. Women with previous VTE have an approximately 3.5 fold increased risk of recurrent VTE during pregnancy compared to non-pregnant periods. Our ability to diagnose pregnancy-associated VTE clinically is generally poor, since dyspnea, tachypnea, swelling and discomfort in the legs are common. Objective diagnosis is essential for treatment decisions. Exposure to radiation of less than 50,000 microGy (5 rad) has not been associated with a significant risk of fetal injury Therefore, besides sonography, routine diagnostic procedures should be performed, if clinically necessary. Heparin does not cross the placenta and is therefore the anticoagulant of choice. In case of acute thrombosis during pregnancy, treatment is performed like in nonpregnant patients. There is ongoing debate, whether or not pregnant women with previous venous thrombosis should routinely receive prophylactic anticoagulation. In patients who have hereditary antithrombin deficiency, antiphospholipid antibodies, a combined abnormality or a history of a severe thrombotic event (pulmonary embolism, extended deep vein thrombosis) should be advised to use prophylactic heparin during pregnancy, starting during the first trimester. Post partum prophylaxis should be given in all women with an increased risk for VTE."}, {"id": "pubmed23n0615_16432", "title": "Thrombophilia and anticoagulation in pregnancy: indications, risks and management.", "score": 0.01453833066969119, "content": "Venous thromboembolism (VTE) is a leading cause of maternal morbidity and mortality in pregnancy and the puerperium. To reduce the incidence of VTE, it is helpful to understand the haemostatic changes during pregnancy and to recognise thrombophilic states. According to the individual risk profile a prophylactic or therapeutic anticoagulation needs to be considered. A narrative, non-systematic overview of articles published in English, German or French over the past three decades with an emphasis on manuscripts from 2003 to 2008. Heparins are the main agents used for anticoagulation during pregnancy. Low-molecular-weight heparins have more advantages than unfractionated heparin and should be preferred. Vitamin-K antagonists are not recommended in this condition as first-line treatment because of the risk for embryopathy and fetal bleeding, but they can be given under certain conditions. Subgroups of patients, such as women with prosthetic heart valves, require special attention. Adverse pregnancy outcomes due to hereditary thrombophilia are new indications for use of anticoagulants during pregnancy. National and international guidelines on prevention and treatment of thromboembolism are helpful in applying the proper regimen in pregnant women."}, {"id": "pubmed23n0976_9535", "title": "[VENOUS THROMBOEMBOLISM DURING PREGNANCY AND THE PUERPERIUM - WHO? WHEN? AND HOW TO TREAT?]", "score": 0.01404724106412662, "content": "Venous thromboembolism (VTE) is a potentially life-threatening medical condition during pregnancy and the puerperium. During pregnancy, the risk of VTE is increased four to tenfold compared to non-pregnant women of comparable age. The risk is even higher in the puerperium. Physician awareness followed by adequate treatment may reduce the number of events. The most important risk factors are previous VTE or thrombophilia, although other acquired risk factors may result in similar impacts. Treatment is based on personalized risk assessment at the first patient visit during pregnancy, followed by repeated assessment of complications or at admission and final assessment at delivery. Hydration and mobilization are advised for all women. Pharmacological prevention by low-molecular-weight heparin (LMWH) is advised based on risk stratification. International guidelines differ by indications and range of management options. The purpose of this review is to summarize our knowledge on risk factors for VTE during pregnancy and puerperium and guide management options."}, {"id": "pubmed23n0519_8168", "title": "[The frequency of venous thromboembolism in women with Leiden mutation in association with pregnancy and puerperium].", "score": 0.013951001361073304, "content": "The assessment of the frequency of venous thromboembolism (VTE) in women with F V Leiden in association with pregnancy and puerperium and according these results and available data to formulate the principles of thromboprophylaxis. Retrospective case control study. The assessment of frequency of VTE in the group of 224 women with F V Leiden in heterozygous form in association with 460 pregnancies and in the group of 40 women with F V Leiden in homozygous form in association with 70 pregnancies. This frequency of VTE in those groups was compared with the frequency of VTE in the control group of 201 women without F V Leiden in association with 422 pregnancies. F V Leiden evaluation was done in the period of 1996-2003. In the group of women with F V Leiden in heterozygous form VTE occurred 44-fold during pregnancy and puerperium. In 17 cases VTE was manifested in pregnancy (once in Ist trimester, twice in IInd trimester, 14 times in IIIrd trimester), in 27 women VTE occurred in puerperium and always within the first 10 days after delivery. Proximal venous thrombosis was diagnosed in 34 cases, in 5 cases being complicated by pulmonary embolism. In 10 women thrombosis was distal. The frequency of VTE is 9.6%. In the group of women with homozyous form VTE occurred in 14 cases (20%). In 5 cases VTE occurred during pregnancy, in 9 cases after delivery and in all cases within first 2 weeks after delivery. The frequency of VTE in the control group is 0.24%. The results were statistically assessed by Fishers exact test in programme NCSS 2004. Frequency of VTE in both cohorts of women with F V Leiden reached statistical significance in comparison with the control group. Pregnancy and puerperium are significant risk factors for VTE in the group of women with F V Leiden in heterozygous form and mainly in homozygous form."}, {"id": "pubmed23n0564_21950", "title": "The management of thrombosis in pregnancy: role of low-molecular-weight heparin.", "score": 0.01343498866680404, "content": "Fatal pulmonary embolism remains the most common cause of mortality among pregnant women in many Western countries. The physiological changes of pregnancy produce a hypercoagulable state that increases the risk of venous thromboembolism (VTE). Women with inherited or acquired thrombophilias are at particularly high risk of VTE during pregnancy, and thromboprophylaxis may be advisable in some cases. Thrombophilia is also associated with complications of pregnancy, including fetal loss, pre-eclampsia, intra-uterine growth restriction, and placental abruption. The antithrombotic agents available for the prevention and treatment of VTE during pregnancy, and pregnancy complications, include unfractionated heparin (UFH), low-molecular-weight heparin (LMWH) and aspirin. Vitamin K antagonists are contra-indicated in pregnancy. Low-dose aspirin may have a role in the prevention of some pregnancy complications, although its safety in early and late pregnancy is uncertain. The efficacy and safety of LMWHs have been demonstrated for the prevention and treatment of VTE in pregnancy. These agents are increasingly being used in place of UFH, which is associated with a higher incidence of side effects compared with LMWH, in addition to the need for regular laboratory monitoring. Evidence is also emerging to support the use of LMWH in the prevention of recurrent fetal loss, although further trials are needed to explore the role of LMWHs in this indication and in the prevention of other complications of pregnancy."}, {"id": "pubmed23n0653_4816", "title": "Efficacy and safety of nadroparin and unfractionated heparin for the treatment of venous thromboembolism during pregnancy and puerperium.", "score": 0.013338530163267075, "content": "The optimal treatment of pregnancy associated VTE (venous thromboembolism) has not been established yet. The assessment of the efficacy and safety of low molecular weight heparin (LMWH) nadroparin and unfractionated heparin (UFH) used for the treatment of pregnancy and puerperium related VTE. Primary study goals were to analyze the incidence of recurrent VTE (proximal extension or pulmonary thromboembolism), thrombocytopenia, major and minor hemorrhages and skin allergic reactions. The study also included the incidence of miscarriages, stillbirth and neonatal abnormalities. We also studied the relationship between the presence of thrombophilia and the occurrence of complications during VTE treatment. Seventy-two women with antepartal VTE treated with s.c. LMWH during entire pregnancy and 88 women with postpartal VTE initially treated with either s.c. LMWH or i.v.UFH were under follow-up during the entire treatment. Thrombophilia testing included antithrombin, protein C and protein S activity levels, Activated protein C (APC) resistance, LA, ACL, FV Leiden, FII G20210A and MTHFR C677T mutations. Twice a day weight based therapeutic regimen was applied for LMWH and activated partial thromboplastin time (aPTT) adjusted UFH dosages. After 2-6 weeks of antepartal deep vein thrombosis (DVT) treatment the dose of nadroparin was reduced to intermediate level. The duration of LMWH therapy during pregnancy was 1-35 weeks, on average 16 weeks. One case (0.62%) of DVT propagation into the vena cava occurred in a woman with antithrombin deficiency treated with LMWH. Two women (1.25%) had minor bleeding and 5 (3.125%) had minimal bleeding, while 3 (1.9%) had skin allergic reactions. The rate of successful pregnancy outcome was 97.2%. There were no cases of stillbirth or neonatal congenital abnormalities. Thrombophilia was found in 86 women (53.7%). No statistically significant correlation between the presence of thrombophilia and treatment complications were found. Nadroparin is both safe and effective for the treatment of DVT during pregnancy and puerperium."}, {"id": "pubmed23n0791_17021", "title": "Pregnancy-Related Venous Thromboembolism.", "score": 0.013317384370015948, "content": "Pregnancy is associated with an increased risk of venous thromboembolism (VTE), with a reported incidence ranging from 0.49 to 2 events per 1000 deliveries. Risk factors include advanced maternal age, obesity, smoking, and cesarian section. Women with a history of previous VTE are at a 4-fold higher risk of recurrent thromboembolic events during subsequent pregnancies. Additionally, the presence of concomitant thrombophilia, particularly factor V Leiden (homozygosity), prothrombin gene mutation (homozygosity), or antiphospholipid syndrome (APS), increases the risk of pregnancy-related VTE. Low-molecular-weight heparin (LMWH) and unfractionated heparin (UFH) are the drugs of choice for anticoagulation during pregnancy. LMWH is preferred due to ease of use and lower rates of adverse events. Women with high thromboembolic risk particularly those with a family history of VTE should receive antepartum thromboprophylaxis. Women with low thromboembolic risk or previous VTE caused by a transient risk factor (ie, provoked), who have no family history of VTE, may undergo antepartum surveillance. Postpartum anticoagulation can be considered in women with both high and low thromboembolic risk. "}, {"id": "pubmed23n0535_7006", "title": "[Pregnancy-associated venous thromboembolic disease: prediction, prevention, and therapy].", "score": 0.01322547508988187, "content": "Thromboembolic disease remains a leading cause of maternal mortality during pregnancy and the puerperium. Rational and risk-adapted administration of heparin prophylaxis depends on 1. the identification of those women who have an increased risk of thrombosis and 2. the accurate quantification of this risk. In women without prior thrombosis, the presence of a heterozygous factor V Leiden or heterozygous G20210A mutation in the prothrombin gene is associated with a pregnancy-associated thrombotic risk of approximately 1 in 400. Thus, in pregnant carriers of either one of these mutations the risk of venous thromboembolism is low. Therefore, no heparin prophylaxis is recommended. A combination of the two genetic risk factors can increase the risk to a modest level of 1 in 25. In women with a single episode of prior thrombosis associated with a transient risk factor, e.g. surgery or trauma, and no additional genetic risk factor, the probability of a pregnancy-associated thrombosis appears also to be low. However, data are sparse and conflicting. In contrast, in women with a prior idiopathic venous thrombosis who carry an additional hereditary risk factor or who have a positive family history of thrombosis, a high risk (&gt;10%) can be expected supporting the indication for active antepartum and postpartum heparin prophylaxis. Despite the remarkable progress in risk stratification, the absolute magnitude of risk and the optimal management in many cases is an issue of ongoing debate."}, {"id": "wiki20220301en165_29294", "title": "Hypercoagulability in pregnancy", "score": 0.013100934153565731, "content": "Strategies A consensus on the correct anticoagulation regimen during pregnancy is lacking. Treatment is tailored to the particular individual based on her risk of complications. Warfarin and other vitamin K-inhibiting agents are contraindicated during the first trimester of pregnancy because of the teratogenic effects, and should not be administered when the pregnancy is confirmed. Rather, women who are on chronic anticoagulation may be given the option of conversion to either unfractionated heparin or low molecular weight heparin (LMWH), such as tinzaparin, prior to a planned conception. LMWH is as safe and efficacious as unfractionated heparin. A blood test including platelets and a clotting screen should be performed prior to administration of anticoagulant regimens in pregnancy."}, {"id": "pubmed23n1018_6720", "title": "Prophylaxis and Therapy of Venous Thrombotic Events (VTE) in Pregnancy and the Postpartum Period.", "score": 0.013009358344873892, "content": "Venous thromboembolisms and pulmonary embolisms are one of the main causes of morbidity and mortality in pregnancy. The increased risk of thrombotic events caused by the physiological changes during pregnancy alone does not justify any medical antithrombotic prophylaxis. However, if there are also other risk factors such as a history of thromboses, hormonal stimulation as part of fertility treatment, thrombophilia, increased age of the pregnant woman, severe obesity or predisposing concomitant illnesses, the risk of thrombosis should be re-evaluated - if possible by a coagulation specialist - and drug prophylaxis should be initiated, where applicable. Low-molecular-weight heparins (LMWH) are the standard medication for the prophylaxis and treatment of thrombotic events in pregnancy and the postpartum period. Medical thrombosis prophylaxis started during pregnancy is generally continued for about six weeks following delivery due to the risk of thrombosis which peaks during the postpartum period. The same applies to therapeutic anticoagulation after the occurrence of a thrombotic event in pregnancy; here, a minimum duration of the therapy of three months should also be adhered to. During breastfeeding, LMWH or the oral anticoagulant warfarin can be considered; neither active substance passes into breast milk."}, {"id": "pubmed23n0600_4753", "title": "Evidence-based indications for thrombophilia screening.", "score": 0.012981088254810882, "content": "Thrombophilic defects have been shown to be associated with an increased risk of venous thrombosis, fetal loss, and gestational complications. The knowledge about the clinical relevance of thrombophilic defects is increasing, and evidence-based indications for thrombophilia screening are therefore discussed in this review. Selective thrombophilia screening based on previous personal and/or family history of venous thromboembolism is more cost-effective than universal screening in all patient groups evaluated. In the majority of patients with acute venous thrombosis, the results of thrombophilia screening do not influence the duration of oral anticoagulation. The only patient population who clearly profits from thrombophilia screening in this situation are patients with a newly diagnosed antiphospholipid syndrome, because prolonged anticoagulation can avoid the high incidence of recurrence in this patient population. Because of the increased risk of venous thrombosis during pregnancy and the puerperium, thrombophilia screening is indicated in selected patients with a previous history of venous thrombosis or a positive family history. Significant associations with early and late pregnancy loss are observed for carriers of the heterozygous factor V Leiden mutation, the heterozygous prothrombin-mutation G20210A and anticardiolipin antibodies, while protein S deficiency is significantly associated with late pregnancy loss. Antithrombotic drugs like UFH, LMWH or low-dose aspirin may have a potential therapeutic benefit in patients with recurrent pregnancy loss and thrombophilia, but placebo-controlled, multicenter trials are urgently needed to clarify this issue. Although a supra-additive effect for the risk of venous thrombosis is observed between oral contraceptives and some thrombophilias, the absolute incidence of venous thromboembolism is low in premenopausal women and mass screening strategies are therefore unlikely to be effective. While antiphospholipid antibodies are known to be associated with arterial thrombosis, screening for heritable thrombophilias is not useful in arterial thrombosis, although subgroup analysis indicates that they may play a role particularly in young patients and children."}, {"id": "pubmed23n0623_22949", "title": "Venous thromboembolism in pregnancy: diagnosis, management and prevention.", "score": 0.012781497261107728, "content": "A pregnant woman has a two- to five-fold higher risk of venous thromboembolism (VTE) than a non-pregnant woman of the same age and, in developed countries, she is more likely to die from fatal pulmonary embolism (PE) than from obstetric haemorrhage. The increased VTE risk is mediated through normal physiological changes of pregnancy including alterations in haemostasis that favour coagulation, reduced fibrinolysis and pooling and stasis of blood in the lower limbs. Thrombophilia, smoking, obesity, immobility and postpartum factors such as infection, bleeding and emergency surgery (including emergency caesarian section) also increase the risk of pregnancy-related VTE. The diagnosis of VTE can be safely established with acceptable radiation exposure to the fetus using readily available imaging modalities such as ultrasound, ventilation perfusion lung scanning and computed tomographic pulmonary angiography. However, the optimal diagnostic strategies still remain to be determined. If there is no contraindication to anticoagulation, commencing treatment prior to objective confirmation should be strongly considered. For the mother and fetus, effective and safe treatment is readily available with low-molecular-weight heparin (LMWH), but optimal dosing of these agents in pregnancy remains controversial. Emerging data support antepartum LMWH prophylaxis for women with previous VTE if the event was unprovoked or in the presence of thrombophilia. On the other hand, women with prior provoked VTE and no thrombophilia or women with asymptomatic thrombophilia (but a family history of VTE) can safely be managed with antepartum surveillance. Postpartum prophylaxis is recommended for women with prior VTE or thrombophilia (and a family history of VTE)."}, {"id": "pubmed23n0398_13343", "title": "Management of thromboembolic disease in pregnancy.", "score": 0.01246854266758179, "content": "Venous thromboembolism (VTE) is the leading cause of maternal mortality and morbidity in developed countries including Singapore. The physiological changes of pregnancy and other factors, such as maternal age, parity, obesity, operative delivery, general anaesthesia and congenital and acquired thrombophilia, further increase the risk of VTE throughout all three trimesters of pregnancy, including the puerperium. VTE has a wide spectrum of clinical presentations and a high index of clinical suspicion is vital. Clinicians should not withhold the use of chest X-rays and ventilation-perfusion (V/Q) lung scans in pregnancy as the radiation emitted is well within the safety limits to the fetus. Most treatment guidelines are based on studies in non-pregnant populations. Heparin is the preferred anticoagulant as it does not cross the placenta and therefore carries no teratogenic risk to the fetus. There is increasing experience and confidence in the use of fixed dose subcutaneous low molecular weight heparin (LMWH) which removes the need for cumbersome monitoring, thereby allowing outpatient treatment. LMWH may also have a lower risk of osteopaenic complications compared to unfractionated heparin. With the exception of acute phase treatment of pulmonary embolism, LMWH is used in all other aspects of the treatment of VTE in pregnancy, including thromboprophylaxis. Risk stratification of women into high and low risk allows judicious use of anticoagulants for thromboprophylaxis. Antenatal thromboprophylaxis with LMWH is reserved for high-risk women, while low-risk women will only require such cover in the postpartum period."}, {"id": "pubmed23n0578_4210", "title": "Management of thromboembolism in pregnancy.", "score": 0.012196908651380851, "content": "The incidence of venous thromboembolism is increased during pregnancy and the postpartum period. This risk is high for women with documented hereditary or acquired risk factors who have experienced a prior thrombotic event. These individuals require a minimum of prophylactic dose anticoagulation with unfractionated or low molecular weight heparin during pregnancy, with anticoagulation continuing for 4 to 6 weeks postpartum. Women receiving therapeutic dose anticoagulation with warfarin before pregnancy for a hereditary or acquired condition should be transitioned to therapeutic doses of unfractionated heparin or low molecular weight heparin before or within 6 weeks of becoming pregnant, and can then resume warfarin postpartum. Women experiencing a thromboembolic event during pregnancy should receive therapeutic treatment with unfractionated heparin or low molecular weight heparin during pregnancy, with anticoagulation continuing for 4 to 6 weeks postpartum, and for a total of at least 6 months."}, {"id": "pubmed23n0292_2595", "title": "[Prevention and treatment of thrombosis in pregnancy].", "score": 0.011997226074895978, "content": "Pregnancy and especially delivery and the puerperium are associated with an increased risk of thromboembolic disease. Intravenous high dose heparin is the therapy of choice for manifest thromboembolic disease in pregnancy. However, high-dose heparin fails to prevent postthrombotic chronic venous insufficiency in more than one-third of the cases. Low-dose heparin may be used for antithrombotic prophylaxis during pregnancy. However, low-dose heparin may induce a substantial loss of bone density in up to 30% of cases and may be complicated by heparin-associated thrombopenia in up to 2%. This review discusses strategies to reduce these considerable risks. Prospective studies suggest that the risk of recurrence after prior deep vein thrombosis may be somewhat overestimated. These data suggest new therapeutic options in women with no risk factors other than a personal history of thrombosis. Improved diagnostic techniques may contribute to a better evaluation of the individual risk by assessing possible underlying problems such as resistance to activated protein C or deficiencies of coagulation inhibitors. Also, duration of prophylactic anticoagulation may be reduced by targeting treatment to periods of increased risk such as immobilisation, dehydration, surgery, delivery and the puerperium. Recently, evidence has been provided indicating that the use of low molecular weight heparins may be associated with reduced loss of bone density and a significantly attenuated risk of heparin-associated thrombopenia."}, {"id": "pubmed23n0683_5340", "title": "[Thromboprophylaxis during pregnancy and the puerperium: highlights from current guidelines].", "score": 0.011680988184747582, "content": "Venous thromboembolism (VTE) is one of the leading causes of maternal deaths worldwide. Mortality and morbidity of VTE are potentially preventable, since two-thirds of these women have identifiable risk factors and may benefit from appropriate thromboprophylaxis. Individual and careful assessment of the personal and family history as well as the assessment of pre-existing and new-onset/transient risk factors during pregnancy and after delivery are mandatory for an effective prevention of VTE. Current guidelines (American College of Chest Physicians 2008, AWMF-Guideline 003/001 2009 and the Royal College Guideline No. 37 2009) provide practical recommendations for risk stratification regarding low, intermediate and high risk conditions. At high risk are women with previous VTE or thrombophilia. Corresponding to risk stratification grade C recommendations have been made for VTE prophylaxis during pregnancy and the puerperium. Prophylaxis with low molecular weight heparin (LMWH) should begin as early in pregnancy as practical. In women with lower risk mobilisation, avoidance of dehydration and mechanical methods (e. g., graduated compressive stockings) are sufficient. After delivery women with intermediate risk should be given LMWH for 7 days, women at high risk for 6 weeks or as long as additional risk factors are present. All women who have additional risk factors and who have had an elective Caesarean section should receive prophylactic LMWH for 7 days as should also all women who have had a Caesarean section in labour or an emergency Caesarean section. At the onset of labour, in case of any vaginal bleeding, prior to induction of labour or 12 h before an elective Caesarean section, antenatal LMWH prophylaxis should be discontinued, LMWH prophylaxis can be continued for 4-6 h after vaginal and for 6-12 h after Caesarean delivery when the women do not have an increased risk of haemorrhage. Current guidelines recommend than LMWH are the agents of choice for antenatal thromboprophylaxis; in comparison to unfractionated heparin, LMWH are associated with a substantially lower risk of heparin-induced thrombocytopenia and osteoporosis. Both oral anticoagulants and heparin are safe when breast-feeding."}, {"id": "pubmed23n0491_12673", "title": "[Indications and monitoring of antithrombotic prophylaxis for venous thromboembolism during pregnancy and post-partum].", "score": 0.01162251655629139, "content": "Pregnancy and puerperium are well-known risk factors for venous thromboembolism, but the actual incidence of the disease is low (about 1/1,500 pregnancies). Pregnancy-associated venous thromboembolism is rare, though it is still the second cause of maternal death in France. Several types of prophylaxis are available, mainly clinical vigilance and aggressive investigation of women with symptoms of venous thromboembolism, or antithrombotic prophylaxis. Given the low incidence of the pathology, it seems desirable to select high-risk groups of women for such strategies. The most studied and identified risk factors are prior episodes of venous thromboembolism and biological thrombophilias. Prophylaxis through low molecular weight heparin during pregnancy and the puerperium should be considered mainly in these two groups. Noteworthy, no prospective and randomized study is available, and treatment recommendations are grade C."}, {"id": "pubmed23n0419_2511", "title": "Inherited thrombophilias and anticoagulation in pregnancy.", "score": 0.011562238930659982, "content": "Thromboprophylaxis, primary or secondary, should be considered in selected pregnant women with inherited thrombophilias; such women may be divided into high-, medium- and low-risk categories on the basis of the specific thrombophilic defect and any personal or family history of venous thromboembolism (VTE). Women at high risk of VTE should receive treatment doses of low-molecular-weight heparin (LMWH) throughout pregnancy and should remain on anticoagulation for 6 weeks postpartum, or, where appropriate, long-term. Women at moderate risk should be treated with prophylactic fixed-dose LMWH throughout pregnancy and for 6 weeks postpartum. Women at low risk should receive prophylactic fixed-dose LMWH for 6 weeks postpartum, and low-dose aspirin LDA should be considered during pregnancy. LWMH offers important advantages over unfractionated heparin (UFH); heparin-induced thrombocytopaenia (HIT) and osteopaenia are rarely seen. For treatment doses of LMWH, dosage adjustment based on anti-Xa levels is usually required as pregnancy progresses. Warfarin should be avoided throughout pregnancy. LMWH, UFH and warfarin are safe for breast-feeding mothers."}, {"id": "wiki20220301en012_2016", "title": "Factor V Leiden", "score": 0.011518679355693121, "content": "Women with factor V Leiden have a substantially increased risk of clotting in pregnancy (and on estrogen-containing birth control pills or hormone replacement) in the form of deep vein thrombosis and pulmonary embolism. They also may have a small increased risk of preeclampsia, may have a small increased risk of low birth weight babies, may have a small increased risk of miscarriage and stillbirth due to either clotting in the placenta, umbilical cord, or the fetus (fetal clotting may depend on whether the baby has inherited the gene) or influences the clotting system may have on placental development. Note that many of these women go through one or more pregnancies with no difficulties, while others may repeatedly have pregnancy complications, and still others may develop clots within weeks of becoming pregnant. See also Prothrombin G20210A References Further reading Factor V Leiden Thrombophilia Explained - Genome.gov External links"}, {"id": "pubmed23n0716_8995", "title": "Obstetric complications and pregnancy-related venous thromboembolism: the effect of low-molecular-weight heparin on their prevention in carriers of factor V Leiden or prothrombin G20210A mutation.", "score": 0.011491988442343053, "content": "Whether the administration of low-molecular-weight heparin (LMWH) during pregnancy is effective in preventing obstetric complications and pregnancy-related venous thromboembolism (VTE) in women who are carriers of factor V Leiden (FVL) and/or prothrombin variant G20210A (PTm) is controversial. This observational study investigated the possible efficacy of pharmacological treatment with LMWH ± aspirin (ASA) in pregnancy outcomes in 1,011 pregnancies of 416 women with thrombophilia (FVL and/or PTm). Most patients were chosen on the basis of previous obstetrical complications (36%), or because of familial or personal history of venous/arterial thromboembolism (28% and 18%, respectively); 74 patients (18%) were incidentally identified. The outcome was evaluated according to the type of treatment and of the period of pregnancy when the treatment was started. After adjustment for observation before and after diagnosis of thrombophilia, previous miscarriages and VTE, parity, age and centre, we observed that LMWH had a protective effect on miscarriages (odds ratio [OR] 0.52, 95% confidence interval [CI] 0.29-0.94) and VTE (OR 0.05, 95% CI 0.01-0.21). ASA appeared to have no effect on the prevention of obstetric complications and VTE. A nested analysis performed in 116 women with two or more obstetric complications confirmed that the highest number of live births was recorded in the group under LMWH prophylaxis (OR 0.19, 95% CI 0.05-0.75). These results suggest that LMWH prophylaxis reduces the risk of obstetric complications in carriers of FVL and/or PTm, particularly in those with previous obstetric events. Furthermore, LMWH prophylaxis reduces the risk of pregnancy-related VTE."}, {"id": "wiki20220301en165_29298", "title": "Hypercoagulability in pregnancy", "score": 0.01142874937717987, "content": "A risk score of four points or higher means prophylaxis in the ante partum period is needed, as well as at least six weeks post partum. A previous distal DVT indicates a minimum of 12 weeks (three months) of therapeutic anticoagulation therapy. A previous proximal DVT or pulmonary embolism requires a minimum of 26 weeks (6.5 months) of therapy If the therapy duration reaches delivery time, the remaining duration may be given after delivery, possibly extending the minimum of six weeks of post partum therapy. In a very high risk, high-dose ante partum prophylaxis should be continued at least 12 weeks after delivery. Women with antiphospholipid syndrome should have an additional low-dose prophylactic treatment of aspirin. Cautions All anticoagulants (including LMWH) should be used with caution in women with suspected coagulopathy, thrombocytopaenia, liver disease and nephropathy."}, {"id": "pubmed23n0535_7005", "title": "Thrombophilia and pregnancy complications.", "score": 0.011419931802097407, "content": "Venous thromboembolism is the leading cause of pregnancy-associated morbidity and mortality. Women with thrombophilia have an increased risk of VTE in pregnancy and puerperium. In individuals with hereditary thrombosis risk factors a relative risk of pregnancy associated VTE ranging from 3.4 to 15.2 has been found. Women with previous VTE have an approximately 3.5-fold increased risk of recurrent VTE during pregnancy compared to non-pregnant periods. Data on the association of thrombophilia and pregnancy loss and pre-eclampsia are conflicting. Besides an established association with antiphospholipid antibodies, available data suggest associations for antithrombin deficiency, hyper-homo-cysteinemia, factor V Leiden, prothrombin G20210A variation and protein S-deficiency. A contribution of thrombophilia to the risk of pre-eclampsia is less well established. A limited number of prospective studies did not reveal an increased risk of pregnancy complications in unselected women with thrombosis risk factors. Data of only one controlled trial on the prevention of pregnancy loss with low molecular weight heparin (LMWH) are available, which revealed a strikingly positive effect. Thrombophilia screening might be justified in women with pregnancy loss and treatment with LMWH might be considered in those with pregnancy loss and thrombophilia. Further prospective studies and controlled interventional trials are urgently needed."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[0, 259]], "word_ranges": [[0, 39]], "text": "We are being shown a picture of lumbar canal stenosis (Answer 4 correct). The patient shows the characteristic clinical picture: lumbar pain that is relieved by flexing the trunk forward, radicular pain with lower limb involvement and neurogenic claudication."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "We are being shown a picture of lumbar canal stenosis (Answer 4 correct). The patient shows the characteristic clinical picture: lumbar pain that is relieved by flexing the trunk forward, radicular pain with lower limb involvement and neurogenic claudication. Low back pain in a patient over 60 years of age accompanied by leg weakness when walking, which has to be interrupted at a certain distance, called neurogenic claudication, is the typical picture of stenosis. The patient is relieved by flexing the spine forward because it widens the lumbar canal and worsens with extension...", "full_answer_no_ref": "We are being shown a picture of lumbar canal stenosis ([HIDDEN]). The patient shows the characteristic clinical picture: lumbar pain that is relieved by flexing the trunk forward, radicular pain with lower limb involvement and neurogenic claudication. Low back pain in a patient over 60 years of age accompanied by leg weakness when walking, which has to be interrupted at a certain distance, called neurogenic claudication, is the typical picture of stenosis. The patient is relieved by flexing the spine forward because it widens the lumbar canal and worsens with extension...", "full_question": "The most likely diagnosis of a 74-year-old patient who since two months ago begins with lumbar pain radiating to lower limbs, neurogenic claudication and limitation to extension the trunk is:", "id": 473, "lang": "en", "options": {"1": "L4-L5 disc herniation.", "2": "Lumbar vertebral fracture.", "3": "L5-S1 vertebral instability.", "4": "Lumbar canal stenosis.", "5": NaN}, "question_id_specific": 141, "type": "ORTHOPEDIC SURGERY AND TRAUMATOLOGY", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0816_19930", "title": "Successful operative management of an upper lumbar spinal canal stenosis resulting in multilevel lower nerve root radiculopathy.", "score": 0.016846175089754212, "content": "Lumbar stenosis is a common disorder, usually characterized clinically by neurogenic claudication with or without lumbar/sacral radiculopathy corresponding to the level of stenosis. We present a case of lumbar stenosis manifesting as a multilevel radiculopathy inferior to the nerve roots at the level of the stenosis. A 55-year-old gentleman presented with bilateral lower extremity pain with neurogenic claudication in an L5/S1 distribution (posterior thigh, calf, into the foot) concomitant with dorsiflexion and plantarflexion weakness. Imaging revealed grade I spondylolisthesis of L3 on L4 with severe spinal canal stenosis at L3-L4, mild left L4-L5 disc herniation, no stenosis at L5-S1, and no instability. EMG revealed active and chronic L5 and S1 radiculopathy. The patient underwent bilateral L3-L4 hemilaminotomy with left L4-L5 microdiscectomy for treatment of his L3-L4 stenosis. Postoperatively, he exhibited significant improvement in dorsiflexion and plantarflexion. The L5-S1 level was not involved in the operative decompression. Patients with radiculopathy and normal imaging at the level corresponding to the radiculopathy should not be ruled out for operative intervention should they have imaging evidence of lumbar stenosis superior to the expected affected level. "}, {"id": "pubmed23n1163_16841", "title": "Nerve root compression due to lumbar spinal canal tophi: A case report and review of the literature.", "score": 0.015325895478567234, "content": "Gout in the spine and adnexa is rare in clinical practice and can also be easily misdiagnosed, we reported a patient with nerve root compression due to lumbar gout stones in the lumbar spinal canal. A 51-year-old male was admitted to the hospital with lumbar pain with numbness in the left lower limb for more than 6 months. The physical examination showed that tenderness and percussion pain were present at L4-S1 spinous process. Straight leg raise test: 50° on the left side were positive. Laboratory tests showed that the sUA was 669 μmol/L, MRI of the lumbar spine showed that cystic T1WI low signal and T2WI mixed high signal shadows were seen in the spinal canal at the level of L4-L5. Combining with lab examinations, imaging examinations, and histopathological results, the patient was diagnosed with lumbar spinal canal tophi. After active improvement of all examinations, the patient underwent surgical treatment with decompression and internal fixation of the L4-L5 segment. After surgery, the patient's symptoms improved and muscle strength returned to normal. Among the 95 previously reported patients with lumbar gout, the ratio of men to women was 2.96:1, and the peak age group of incidence was 56 to 65 years. The onset of the disease was mainly in a single segment of the lumbar spine, with 34.41% of all cases occurring at the L4-L5 level. 61.05% of the patients had a history of gout attacks or hyperuricemia, and the most frequently involved site was the foot and ankle, followed by the wrist. Sixty-seven patients underwent surgical treatment, and 22 chose conservative treatment, with overall satisfactory results. The incidence of lumbar gout is low and relatively rare in the clinic and pathological biopsy is still the gold standard. Vertebral plate incision and decompression are often selected for surgical treatment, and whether to perform fusion should be comprehensively considered for the destruction of vertebral bone by gout and the reasonable selection of the extent of surgical resection. Whether choosing surgical treatment or conservative therapy, the control of uric acid levels should be emphasized."}, {"id": "pubmed23n0977_4210", "title": "Limbus Vertebral Fracture Presenting as Cauda Equina Syndrome Masquerading An Acute Disc Prolapse - A Rare Case Report and Review of Literature.", "score": 0.013368549370174725, "content": "Limbus vertebral fracture is an uncommon injury described in adolescents. It refers to the separation of a bony fragment from the unfused ring apophysis of lumbar vertebral end plate. It usually presents with back pain with/without radiculopathy masquerading an acute disc prolapse. However, the presentation as acute cauda equina syndrome has seldom been reported. A 15-year-old male presented to the casualty with a history of acute-onset low back pain and bilateral lower limb radicular pain with weakness, after lifting of a heavy Indian musical instrument (Dholak). This was associated with urinary retention and numbness in perineal region. Examination revealed L5 and S1 weakness with absent ankle jerks bilaterally. Bulbocavernosus reflex was absent. Emergency magnetic resonance imaging imaging was done, which revealed a limbus fracture of cephalad part of L4 vertebral body with displaced fragment into the spinal canal causing compression of the cauda equina. Emergency surgery was done in the form of L3-L4 midline interlaminar microscopic decompression. The patient had complete neurological recovery including the bladder control within 1 month of surgery. The patient had no functional deficits during follow-up at 3 and6 months. Dynamic radiographs taken at the end of 6 months did not show any sign of instability. When an adolescent patient with no previous history of backpain presents with acute cauda equina syndrome, a possibility of limbus fracture should also be considered. Early diagnosis and surgical decompression in such cases can bring about complete neurological recovery and excellent clinical outcome."}, {"id": "pubmed23n1152_8492", "title": "Posterior lumbar interbody fusion for dysplastic lumbar spondylolisthesis with high-grade slippage in two adolescent siblings: two case reports.", "score": 0.01334060494189705, "content": "Lumbar spondylolisthesis is reported to present with a familiar pattern, with the dysplastic type of spondylolysis being minor but more hereditary than the isthmic type. Siblings presenting during adolescence with neurological symptoms owing to high-grade dysplastic-type spondylolisthesis are rare. The older brother suffered from left leg pain and numbness and dysesthesia of the right posterior thigh and calf and could not walk without a crutch at the age of 15 years. He had canal stenosis with disc bulging and dysplastic bilateral facet joint at L5/S1. The L5 vertebral body was slipped anterior downward to S1, with a round-shaped S1 cranial endplate. We diagnosed dysplastic-type spondylolisthesis and performed posterior lumbar interbody fusion at L5/S with mild reduction and sublaminar wiring at L4/5. The younger brother had no neurological symptoms at age 14 years but suffered from bilateral lower leg numbness at age 18 years. He had canal stenosis with disc bulging at L4/5 and L5/S1 and dysplastic bilateral facet joint at L5/S1 with right pars defect. The L5 vertebral body was vertically displaced anterior to the S1 vertebral body, with an S1 round-shaped cranial endplate. We diagnosed dysplastic-type spondylolisthesis, and posterior lumbar interbody fusion at L4/5 and L5/S with reduction was performed. Their neurological symptoms of the lower legs disappeared, and interbody bone fusion was obtained. The surgical technique for high-grade dysplastic spondylolisthesis remains controversial in terms of in situ fusion versus reduction. We recommend that surgery be performed promptly at the end of bone maturation because neurological symptoms often appear at the end of bone maturation. Because high-grade slips are rare but siblings may be present, the sibling should also be screened when dysplastic spondylolisthesis is detected."}, {"id": "article-24483_18", "title": "Anatomy, Back, Lumbosacral Trunk -- Surgical Considerations", "score": 0.012511740238830001, "content": "Over 95% of lumbar disc herniations affect either the L4/L5 or L5/S1 disc spaces, or both. Patients commonly experience sharp, stabbing low back pain that radiates from the lower lumbar region down to the foot. [6] [7] [8] Nonsurgical management usually suffices in cases of lower extremity radiculopathy [9] [10] [11] and acute lumbar disc herniation. [8] The most common indication for surgical intervention is persistent, intractable pain despite at least 6 weeks of optimal conservative treatment. [12]"}, {"id": "wiki20220301en024_74659", "title": "Lumbar spinal stenosis", "score": 0.012469802472644592, "content": "Society and culture United States Under rules promulgated by Titles II and XVI of the United States Social Security Act, spinal stenosis is recognized as a disabling condition under Listing 1.04 C. The listing states: \"Lumbar spinal stenosis resulting in neurogenic claudication, established by findings on appropriate medically acceptable imaging, manifested by chronic nonradicular pain and weakness, and resulting in inability to ambulate effectively, as defined in 1.00B2b.\" The regulation is written specifically for lumbar stenosis. References External links Geriatrics Spinal cord disorders Bones of the vertebral column"}, {"id": "wiki20220301en024_74641", "title": "Lumbar spinal stenosis", "score": 0.012427789601702644, "content": "Among people with lower-extremity pain in combination with back pain, lumbar stenosis as the cause is two times more likely in those older than 70 years of age while in those younger than 60 years it is less than half as likely. The character of the pain is also useful for diagnosis. When the discomfort does not occur while seated, the likelihood of lumbar spinal stenosis increases considerably, around 7.4 times. Other features increasing the likelihood of lumbar stenosis are improvement in symptoms on bending forward (6.4 times), pain that occurs in both buttocks or legs (6.3 times), and the presence of neurogenic claudication (3.7 times). On the other hand, the absence of neurogenic claudication makes lumbar stenosis much less likely as the explanation for the pain. Causes"}, {"id": "wiki20220301en119_13364", "title": "Spinal disc herniation", "score": 0.012408012408012408, "content": "Lumbar disc herniations occur in the back, most often between the fourth and fifth lumbar vertebral bodies or between the fifth and the sacrum. Here, symptoms can be felt in the lower back, buttocks, thigh, anal/genital region (via the perineal nerve), and may radiate into the foot and/or toe. The sciatic nerve is the most commonly affected nerve, causing symptoms of sciatica. The femoral nerve can also be affected and cause the patient to experience a numb, tingling feeling throughout one or both legs and even feet or a burning feeling in the hips and legs. A herniation in the lumbar region often compresses the nerve root exiting at the level below the disc. Thus, a herniation of the L4–5 disc compresses the L5 nerve root, only if the herniation is posterolateral. Cervical disc herniation"}, {"id": "wiki20220301en013_49860", "title": "Lumbar vertebrae", "score": 0.01225705329153605, "content": "Most individuals have five lumbar vertebrae, while some have four or six. Lumbar disorders that normally affect L5 will affect L4 or L6 in these latter individuals. Segmental movements The range of segmental movements in a single segment is difficult to measure clinically, not only because of variations between individuals, but also because it is age and sex dependent. Furthermore, flexion and extension in the lumbal spine is the product of a combination of rotation and translation in the sagittal plane between each vertebra. Ranges of segmental movements in the lumbar spine (White and Punjabi, 1990) are (in degrees): Congenital anomalies Congenital vertebral anomalies can cause compression of the spinal cord by deforming the vertebral canal or causing instability."}, {"id": "InternalMed_Harrison_1475", "title": "InternalMed_Harrison", "score": 0.01206228281208233, "content": "The straight leg–raising (SLR) maneuver is a simple bedside test for nerve root disease. With the patient supine, passive flexion of the extended leg at the hip stretches the L5 and S1 nerve roots and 113 CHAPTER 22 Back and Neck Pain 4th Lumbar vertebral body 5th Lumbar vertebral body 4th Lumbar pedicle L4 root Protruded L4-L5 disk L5 Root S1 Root S2 Root Protruded L5-S1 disk FIguRE 22-3 Compression of L5 and S1 roots by herniated disks. (From AH Ropper, MA Samuels: Adams and Victor’s Principles of Neurology, 9th ed. New York, McGraw-Hill, 2009; with permission.) the sciatic nerve. Passive dorsiflexion of the foot during the maneuver adds to the stretch. In healthy individuals, flexion to at least 80° is normally possible without causing pain, although a tight, stretching sensation in the hamstring muscles is common. The SLR test is positive if the maneuver reproduces the patient’s usual back or limb pain. Eliciting the SLR sign in both the supine and sitting positions can help"}, {"id": "article-24451_15", "title": "Lumbar Degenerative Disk Disease -- History and Physical", "score": 0.011952853951808375, "content": "The straight leg raise (SLR) test consists of a supine patient having his/her fully extended leg passively stretched from 0 to about 80 degrees. The onset of radiating back pain in either leg supports a diagnosis of a stenotic canal. A herniation compressing the L5 nerve root will present as a weakness of ankle dorsiflexion and an extension of the great toe. This deficit also may diminish the Achilles tendon reflex. L4 radiculopathy may present with weakness in the quadriceps and a decreased patellar tendon reflex. Documentation is paramount, as these initial findings likely will be used as a baseline for all future evaluations."}, {"id": "article-24464_27", "title": "Anatomy, Back, Lumbar Spine -- Clinical Significance", "score": 0.011338100102145046, "content": "Compression, injury, or irritation of the lumbar spinal nerve roots can occur from multiple potential sources. Most commonly, this occurs as a consequence of the degenerative cascade or an acute disc herniation. Lumbar radiculopathy (aka sciatica) describes the constellation of symptoms resulting from lumbar nerve compression. Individuals present with variable degrees of radiating pain, paresthesia (numbness/tingling), and weakness in the lower extremities. Lumbar stenosis is a condition in which the narrowing of the spinal canal occurs. This narrowing is usually secondary to degenerative spondylosis and spondylolisthesis. Classically individuals present with low back and/or lower extremity pain/paresthesias, which worsen with lumbar extension, prolonged standing, and ambulation. The pathophysiology appears to be due to mechanical compression of the lumbosacral spinal nerve roots resulting in ischemia. [16] [17]"}, {"id": "InternalMed_Harrison_1471", "title": "InternalMed_Harrison", "score": 0.011053864168618267, "content": "PART 2 Cardinal Manifestations and Presentation of Diseases FIguRE 22-1 Vertebral anatomy. (From A Gauthier Cornuelle, DH Gronefeld: Radiographic Anatomy Positioning. New York, McGraw-Hill, 1998; with permission.) Radicular pain is typically sharp and radiates from the low back to a leg within the territory of a nerve root (see “Lumbar Disk Disease,” below). Coughing, sneezing, or voluntary contraction of abdominal muscles (lifting heavy objects or straining at stool) may elicit the radiating pain. The pain may increase in postures that stretch the nerves and nerve roots. Sitting with the leg outstretched places traction on the sciatic nerve and L5 and S1 roots because the nerve passes posterior to the hip. The femoral nerve (L2, L3, and L4 roots) passes anterior to the hip and is not stretched by sitting. FIguRE 22-2 Spinal column. (From A Gauthier Cornuelle, DH Gronefeld: Radiographic Anatomy Positioning. New York, McGraw-Hill, 1998; with permission.)"}, {"id": "article-29356_9", "title": "Spinal Stenosis -- History and Physical", "score": 0.010967074132492113, "content": "Stenosis in the lumbar spine can lead to neurogenic claudication, myeloradiculopathy symptoms, sensory disturbances, motor weakness, and pathologic reflexes. Patients will present with complaints of cramping pain in the leg, calf, and or buttocks. They might report an increase in pain with walking or standing for prolonged periods and relief when sitting or leaning forward while using a shopping cart. [9] Disk herniation is most common at the L4-5 and L5-S1 levels. A herniated disk at L5-S1 can lead to plantarflexion weakness, decrease sensation in the lateral foot, and cause pain in the posterior leg. A disk herniation at L4-5 can lead to a foot drop and numbness in the large toe web and dorsal aspect of the foot. Lastly, an L3-4 disk herniation can lead to knee extension weakness, numbness in the medial foot, and pain in the anterior thigh."}, {"id": "article-24453_17", "title": "Lumbar Disc Herniation -- History and Physical", "score": 0.010802469135802469, "content": "L5 nerve root exits at the L5-S1 foramina. When compressed by a herniated disc, it causes back pain that radiates into the buttock, lateral thigh, lateral calf, the dorsum of the foot, and the great toe. Sensory loss is present on the web space between the big toe and second toe, the dorsum of the foot, and lateral calf. There is a weakness in hip abduction, knee flexion, foot dorsiflexion, big toe dorsiflexion, foot inversion, and eversion. Patients present with decreased semitendinosus/semimembranosus reflex. Weakness in foot dorsiflexion makes it challenging to walk on the heels. Chronic L5 radiculopathy may cause atrophy of the extensor digitorum brevis and the tibialis anterior of the anterior leg."}, {"id": "pubmed23n0915_17750", "title": "Tophaceous gout causing lumbar stenosis: A case report.", "score": 0.009900990099009901, "content": "Gout in the spine is very rare. The clinical symptoms of the spinal gout are various and lack of specificity. The authors report a case of spinal gout causing lumbar stenosis. We never find such wide-invasive spinal gouty lesion in the published studies. A 68-year-old male had low back pain radiating to bilateral lower limbs, accompanying with intermittent claudication that lasted for 3 months and aggravated 5 days ago. Spinal gout, lumbar stenosis. The patient underwent L2-L4 laminectomy, L2/3 L3/4 an d L4/5 discectomy and transforaminal lumbar interbody fusion with pedicle screw fixation. Dual-energy computed tomography detected extensive tophaceous deposits in L1/2 L2/3 L3/4 and L4/5 lumbar discs as well as the posterior column, especially L2-L3 and L4-L5 facet joints. During the surgery, we found a mass of chalky white material at the posterior column of L3 to L5 vertebral bodies, which also involved the intervertebral discs. Pathological examination confirmed the diagnosis of spinal gout. Although spinal gout is thought to be rare, the diagnosis should be considered if the patient had severe back pain and a history of gout. Dual-energy computed tomography is highly recommended for these patients."}, {"id": "Surgery_Schwartz_6305", "title": "Surgery_Schwartz", "score": 0.009900990099009901, "content": "to provide crucial collateral flow in maintaining lower limb viabil-ity. It must be emphasized, however, that patients should be subjected to angiography only if their symptoms warrant surgi-cal intervention.Differential DiagnosisDegenerative hip or spine disease, lumbar disk herniation, spinal stenosis, diabetic neuropathy, and other neuromuscular problems can produce symptoms that may be mistaken for vas-cular claudication. Such cases can be distinguished from true claudication by the fact that the discomfort from neuromuscular problems is often relieved by sitting or lying down, as opposed to cessation of ambulation. In addition, complaints that are Table 23-13Clinical outcome of renal artery stent placement in the treatment of renovascular hypertension and renal insufficiencyAUTHORYEARPATIENT NO.TECHNICAL SUCCESS (%)FOLLOW-UP (MONTHS)RENAL INSUFFICIENCY (%)RENOVASCULAR HYPERTENSION (%)COMPLICATION (%)RESTENOSIS"}, {"id": "pubmed23n0286_9578", "title": "Lumbar spinal stenosis.", "score": 0.00980392156862745, "content": "Symptoms for spinal stenosis apparently result from an incongruity between the capacity and contents of the spinal nerve passages. These symptoms are most frequently seen in men in their fifth or sixth decade of life. Spinal extension generally exacerbates the claudication-type symptoms (lower-extremity pain and paresthesia), whereas spinal flexion diminishes these symptoms. Differential diagnosis is needed to rule out vascular claudication due to atherosclerosis. Decisions regarding surgery should be made based not only on diagnostic imaging but also on a thorough history and clinical examination."}, {"id": "Neurology_Adams_1596", "title": "Neurology_Adams", "score": 0.009767170644341705, "content": "Herniation of Lumbar Intervertebral Discs (Table 10-1) This condition is a major cause of severe and chronic or recurrent low back and leg pain. It occurs mainly during the third and fourth decades of life when the nucleus pulposus is still gelatinous. The disc between the fifth lumbar or first sacral vertebrae (L5-S1) is most often involved, and, with decreasing frequency, that between the fourth and fifth (L4-L5), third and fourth (L3-L4), second and third (L2-L3), and—quite infrequently—the first and second (L1-L2) lumbar vertebrae. Disc disease is relatively rare but well described in the thoracic portion of the spine. It is frequent in the cervical spine, especially at the fifth and sixth and the sixth and seventh cervical vertebrae (see further on)."}, {"id": "pubmed23n0638_22379", "title": "Traumatic burst fracture in a patient with a lumbar artificial disc.", "score": 0.009708737864077669, "content": "Lumbar disc arthroplasty is now a common treatment for lumbar degenerative disc disease. Whereas the immediate and delayed complications in patients with artificial lumbar discs are well reported, the durability of artificial disc hardware after severe spine trauma is unknown. The authors describe the management of a rare case of a traumatic lumbar burst fracture in a patient who had undergone disc arthroplasty. This 31-year-old male contractor had undergone placement of an L4-5 Charité artificial disc (DePuy Spine) and L5-S1 anterior lumbar fusion 10 months before he fell from a roof and sustained a traumatic L-3 burst fracture with significant canal compromise and cauda equina injury. Despite the considerable compressive load on his spine, the artificial disc (L4-5) remained intact without any radiological signs of hardware failure, and the vertebrae above (L-4) and below (L-5) the artificial disc had no signs of injury. For the L-3 burst fracture the patient underwent an open decompressive laminectomy at L2-3 and posterior fusion with instrumentation from L-2 to L-4. At 24 months postinjury, he had returned to full work activities as a contractor with minimal back pain and mild right lower-extremity sensory changes and weakness left over from the trauma. The total disc arthroplasty at L4-5 is functional and has preserved motion, and there is a solid fusion at L2-4 and L5-S1. This case demonstrates that a lumbar artificial disc can tolerate a significant load from trauma and remain functional without hardware failure even after a traumatic burst fracture at the adjacent lumbar vertebral body and shows the successful treatment of this fracture, with posterior fusion preserving the motion of an artificial disc."}, {"id": "pubmed23n0005_3938", "title": "Clinical features of lumbar spinal stenosis.", "score": 0.009708737864077669, "content": "In contrast with patients with herniations of the nucleus pulposus, those with spinal stenosis experience onset of symptoms at a slightly older age; more males are affected than females. The symptoms tend to be somewhat more chronic, and therefore, the patients will have symptoms of back pain for a longer period of time before developing radiating root pains and will not come to surgical treatment until relatively late. Bilateral root symptomatology is more common although examination shows multiple nerve root involvement only slightly more frequently as well as involvement of the L1 to L4 nerve roots. The spinal movements tend to be somewhat better and straight leg raising tests are usually symmetrical and somewhat less restricted in those patients than in the acute disk syndrome. Postfusion and post-chemonucleolysis spinal stenosis will, of course, have the symptomatology of the initial problem, but their recurrent problems would tend to parallel those of spinal stenosis rather than disk herniation."}, {"id": "wiki20220301en257_4545", "title": "Spinal disease", "score": 0.009683724235963042, "content": "Lumbar spinal stenosis is classified as a narrowing of the spinal canal in the lumbar region of the vertebrae. This may lead to compression of the nerve root of the spinal cord and result in pain of the lower back and lower extremities. Other symptoms include impaired walking and a slightly stooped posture due to loss of disc height and bulging of the disc. Lumbar spinal stenosis is very prevalent with 9.3% of the general population producing symptoms and the number is continuing to rise in patients older than 60. It's generally an indication for spinal surgery in patients older than 65 years of age. However, there is a myth and fear among most patients that only surgery is the cure for such conditions and spine surgery is very risky. There are many non-surgical treatments available to prevent, halt and even reverse many spine diseases. Also, some surgery patients can be operated on in a daycare procedure or with minimum length of stay in hospital, with statistically good outcomes."}, {"id": "pubmed23n0868_18857", "title": "Firearm bullet settling into the lumbar spinal canal without causing neurological deficit: A report of two cases.", "score": 0.009615384615384616, "content": "Uncertainty still exists regarding the treatment of the patients presenting with gunshot wounds to the spine. Neurological insults, cerebrospinal fluid fistula, infection, lead or copper toxicity, migration of bullets, and spinal instability are included among the common challenging issues. An 18-year-old woman was admitted with low back pain following a gunshot injury five days ago. She was neurologically intact. Radiological examinations showed that a bullet was settled in L4-5 disc space. The bullet was removed with a unilateral L4-5 partial hemilaminectomy and discectomy from the left side. The second case was of a 29-year-old man admitted with radiating leg pain on the right side following a gunshot injury from his left side of lower back four months ago. He had only positive straight leg raising test. Radiological studies showed two bullets, one was in the psoas muscle on the left side and the other was in spinal canal that had caused a burst fracture of the L5 vertebra. Following L5 laminectomy and bilateral L5-S1 facetectomy, the bullet was removed from the spinal canal and L5-S1 transpedicular posterior stabilization was performed. The postoperative period of both patients was unremarkable. Bullet settling into the lumbar spinal canal without causing neurological deficit may require surgical intervention. Removal of bullets provided not only pain relief in both the cases but also prevented future complications such as migration of the bullets, plumbism, and neuropathic pain and instability."}, {"id": "pubmed23n1049_9587", "title": "Clinical Presentations of Lumbar Disc Degeneration and Lumbosacral Nerve Lesions.", "score": 0.009615384615384616, "content": "Lumbar disc degeneration is defined as the wear and tear of lumbar intervertebral disc, and it is mainly occurring at L3-L4 and L4-S1 vertebrae. Lumbar disc degeneration may lead to disc bulging, osteophytes, loss of disc space, and compression and irritation of the adjacent nerve root. Clinical presentations associated with lumbar disc degeneration and lumbosacral nerve lesion are discogenic pain, radical pain, muscular weakness, and cutaneous. Discogenic pain is usually felt in the lumbar region, or sometimes, it may feel in the buttocks, down to the upper thighs, and it is typically presented with sudden forced flexion and/or rotational moment. Radical pain, muscular weakness, and sensory defects associated with lumbosacral nerve lesions are distributed on lower extremities, the buttock, lower abdomen, and groin region. A lumbosacral plexus lesion presents different symptoms in the territories of the lumbar and sacral nerves. Patients with lumbar plexus lesion clinically present with weakness of hip flexion, knee extension, thigh adduction, and sensory loss in the lower abdomen, inguinal region, and over the entire medial, lateral, and anterior surfaces of the thigh and the medial lower leg, while sacral plexus lesion presents clinical symptoms at nerve fibers destined for the sciatic nerve, common peroneal nerve, and pudendal nerve. Weakness of ankle inversion, plantar flexion, and foot drop are the main clinical manifestations of the sacral plexus lesion area. Numbness and decreased sensation are also present along the anterolateral calf and dorsum of the foot. On examination, foot eversion is usually stronger than foot dorsiflexion. The patients may also present with pain and difficulty of bowel movements, sexual dysfunction assessments, and loss of cutaneous sensation in the areas of the anal canal, anus, labia major, labia minor, clitoris, penis, and scrotum."}, {"id": "pubmed23n0904_21891", "title": "Lumbar vertebral body and pars fractures following laminectomy.", "score": 0.009523809523809525, "content": "A 56-year-old alcoholic male incurred L5 vertebral body and bilateral L4 pars fractures with progressive L4 on L5 anterolisthesis following low-energy falls while intoxicated. Recently, he had a L3-S1 laminectomy for lumbar spinal stenosis with claudication. Preoperative imaging and radiographs were negative for pars defects and instability, so an isolated decompressive surgery was performed. Following low-energy falls, his outpatient work-up revealed fractures through the bilateral L4 pedicles and posterior third of L5 vertebral body, with recurrence of axial back pain and bilateral lower extremity radiculopathy. He underwent revision decompression from L4-S1 and posterior instrumented fusion with transforaminal lumbar interbody fusion performed at each revised level. His axial back pain and radiculopathy improved postoperatively. Instability of a lumbar spine fracture pattern can be due to the remote or prior iatrogenic disruption of the posterior ligamentous complex. Our patient benefitted from surgery and his low back pain was resolved."}, {"id": "article-24463_27", "title": "Lumbar Spinal Stenosis -- History and Physical", "score": 0.009523809523809525, "content": "Some patients with LSS may be asymptomatic and have a normal neurologic examination. Other individuals with asymptomatic LSS may have neurologic deficits due to superimposed lumbar radiculopathy. The Valsalva maneuver often does not exacerbate LSS-related radicular pain as it does in IV disk prolapse. Only 10% of patients present with a positive straight leg raise test. [24] Pedal pulses should also be checked during the physical exam to rule out vascular claudication. A patient who can perform a five-repetition sit-to-stand (5R-STS) test in 10.4 seconds may be considered to have no functional impairment. [25]"}, {"id": "pubmed23n1138_6027", "title": "Vertical split fracture of the vertebral body following oblique lumbar interbody fusion: A case report.", "score": 0.009433962264150943, "content": "Oblique lumbar interbody fusion (OLIF) is an effective and safe surgical technique widely used for treating spondylolisthesis; however, its use is controversial because of several associated complications, including endplate injury. We report a rare vertebral body fracture following OLIF in a patient with poor bone quality. A 72-year-old male patient visited our clinic for 2 years with lower back pain, leg radiating pain, and intermittent neurogenic claudication. Lumbar magnetic resonance imaging revealed L4-5 stenosis. We performed OLIF with percutaneous pedicle screw fixation and L4 subtotal decompressive laminectomy. We resected the anterior longitudinal ligament partially for anterior column release and inserted a huge cage to maximize segmental lordosis. No complications during and after the operation were observed. Further, the radiating pain and back pain improved, and the patient was discharged. Two weeks after the operation, the patient visited the outpatient department complaining of sudden recurred pain, which occurred while going to the bathroom. Radiography and computed tomography revealed a split fracture of the L5 body and an anterior cage displacement. In revision of OLIF, we removed the dislocated cage and filled the bone cement between the anterior longitudinal ligament and empty disc space. Further, we performed posterior lumbar interbody fusion L4-5, and the screw was extended to S1. After the second surgery, back pain and radiating pain in the left leg improved, and he was discharged without complications. In this case, owing to insufficient intervertebral space during L4-5 OLIF, a huge cage was used to achieve sufficient segmental lordosis after anterior column release, but a vertebral body coronal fracture occurred. In patients with poor bone quality and less flexibility, a huge cage and over-distraction could cause a vertebral fracture; hence, selecting an appropriate cage or considering a posterior approach is recommended."}, {"id": "InternalMed_Harrison_1467", "title": "InternalMed_Harrison", "score": 0.009363657839915811, "content": "Nerve root injury (radiculopathy) is a common cause of neck, arm, low back, buttock, and leg pain (see Figs. 31-2 and 31-3). The nerve roots exit at a level above their respective vertebral bodies in the cervical region (e.g., the C7 nerve root exits at the C6-C7 level) and below their respective vertebral bodies in the thoracic and lumbar regions (e.g., the T1 nerve root exits at the T1-T2 level). The cervical nerve roots follow a short intraspinal course before exiting. By contrast, because the spinal cord ends at the vertebral L1 or L2 level, the lumbar nerve roots follow a long intraspinal course and can be injured anywhere from the upper lumbar spine to their exit at the intervertebral foramen. For example, disk herniation at the L4-L5 level can produce not only L5 root compression, but also compression of the traversing S1 nerve root (Fig. 22-3). The lumbar nerve roots are mobile in the spinal canal, but eventually pass through the narrow lateral recess of the spinal canal and"}, {"id": "wiki20220301en032_84501", "title": "Lordosis", "score": 0.009345794392523364, "content": "Diagnosis Measurement and diagnosis of lumbar hyperlordosis can be difficult. Obliteration of vertebral end-plate landmarks by interbody fusion may make the traditional measurement of segmental lumbar lordosis more difficult. Because the L4–L5 and L5–S1 levels are most commonly involved in fusion procedures, or arthrodesis, and contribute to normal lumbar lordosis, it is helpful to identify a reproducible and accurate means of measuring segmental lordosis at these levels. A visible sign of hyperlordosis is an abnormally large arch of the lower back and the person appears to be puffing out his or her stomach and buttocks. Scanning"}, {"id": "pubmed23n0254_7472", "title": "[Lumbar canal stenosis. Retrospective study of 158 operated cases].", "score": 0.009345794392523364, "content": "The authors report the results of a retrospective study of 158 lumbar spinal stenosis (LSS), all operated (111 degenerative, 26 congenital, 21 mixed). Eighty seven percent of the patients had a low-back pain and 81.6% a radicular pain. Only 57.6% of them had a polyradicular claudication. A neurological deficit (motor, sensitive, or involving sphincters) was present in 36.6% of cases. A myelographic block was noted in 23.4% of cases, and in 20.3% a spondylolisthesis with an intact neural arch was found. Surgery consisted of a posterior lateral spinal canal calibration, sometimes associated with a ventral canal calibration (via the posterior route) (6.3%), and/or excision of a disc herniation at one (47.5%) or two levels (3.8%). Mean follow-up after surgery was 7.8 months. The global result was good or excellent in 75.2% of cases. Radicular pain was relieved in 89.1% of cases, and polyradicular claudication in 90.1% of cases. Neurological deficit improved in 50.6% of cases. In only 59.8% of cases relief of low-back pain was achieved. Statistically low-back pain (lasting for over 2 years) improved less, but a preoperative spondylolisthesis didn't influence the quality of the result regarding this symptom. Semiology, pathophysiology, and surgery particularly regarding spine stability are discussed."}, {"id": "wiki20220301en008_121492", "title": "Back pain", "score": 0.009259259259259259, "content": "Spinal disc disease Spinal disc disease occurs when the nucleus pulposus, a gel-like material in the inner core of the vertebral disc, ruptures. Rupturing of the nucleus pulposus can lead to compression of nerve roots. Symptoms may be unilateral or bilateral, and correlate to the region of the spine affected. The most common region for spinal disk disease is at L4–L5 or L5–S1. The risk for lumbar disc disease is increased in overweight individuals due to the increased compressive force on the nucleus pulposus, and is twice as likely to occur in men. A 2002 study found that lifestyle factors such as night shift work and lack of sporting activity can also increase the risk of lumbar disk disease."}, {"id": "pubmed23n0751_23527", "title": "Comparison of radicular symptoms caused by lumbar disc herniation and lumbar spinal stenosis in the elderly.", "score": 0.009259259259259259, "content": "Comparative study using combined data from 2 prospective cohort studies. To expose the differences between the clinical characteristics of neurogenic claudication from magnetic resonance image-documented lumbar spinal stenosis (LSS) and lumbosacral radicular syndrome from acute, magnetic resonance image-documented, lumbar disc herniation (LDH). LSS and LDH are the common lumbar disorders that produce lower extremity pain. Though known factors such as pain induced by walking for LSS and the rapid onset of symptoms for LDH are useful for differentiating these disorders, exploration of differences in other factors has received limited study. This study included participants aged 60 yr or older from 2 previous studies. One examined walking limitations caused by LSS and the second the natural history of LDH in elderly adults. The clinical features of both groups were compared by calculating means, medians, and standard deviations for continuous variables, and frequencies for categorical variables. χ test was used to explore differences between LSS and LDH for categorical variables, and Student t test or Mann-Whitney test for continuous variables. Participants with LSS had more medical comorbidity, less intense leg pain, and less disability than those with LDH. Leg pain was more common in the anterior thigh, anterior knee and shin in LDH, and in the posterior knee in LSS. Trunk flexion was more impaired in LDH. Positive straight leg raising and femoral stretch signs were common in LDH, and rare in LSS. Abnormal Achilles reflexes were noted more frequently in LSS. In addition to established factors, greater leg pain intensity, greater disability, and pain in the anterior leg are more common in the elderly with LDH than in the elderly with LSS. Normal trunk flexion, absence of nerve root tension signs and abnormal Achilles reflexes are more common in LSS. 3."}]}}}}
{"correct_option": 5, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": true, "char_ranges": [[1269, 1435]], "word_ranges": [[193, 214]], "text": "In unstable situations as in the case described, with significant visual disturbances, surgical intervention is usually of choice, with transsphenoidal decompression."}}, "full_answer": "Pituitary apoplexy is a syndrome characterized by the sudden onset of headache accompanied by ocular motility disturbances and a variable degree of pituitary insufficiency. The cause is usually a rapidly growing pituitary mass secondary to a vascular event (infarction or hemorrhage). General symptoms may range from vomiting or nausea to meningeal irritation. Compression on the chiasm and optic nerve can cause various campimetric deficits (usually greater bitemporal hemianopsia in the superior quadrants) and decreased visual acuity, even blindness. If the mass grows lateral to the sella turcica it can compress the oculomotor nerves, with the common motor ocular nerve being the most frequently affected, because of its more medial course in the cavernous sinus. The test of choice for the diagnosis of pituitary apoplexy is MRI, as CT may not distinguish the region clearly enough to distinguish degenerative or cystic changes from previous bleedings. The finding of erosion of the anterior clinoid processes in the skull CT may support a chronic lesion, which as a result of an acute vascular phenomenon has caused the symptoms. The management of these patients involves strict control of water and electrolyte disturbances and correction of hormonal deficits. In unstable situations as in the case described, with significant visual disturbances, surgical intervention is usually of choice, with transsphenoidal decompression.", "full_answer_no_ref": "Pituitary apoplexy is a syndrome characterized by the sudden onset of headache accompanied by ocular motility disturbances and a variable degree of pituitary insufficiency. The cause is usually a rapidly growing pituitary mass secondary to a vascular event (infarction or hemorrhage). General symptoms may range from vomiting or nausea to meningeal irritation. Compression on the chiasm and optic nerve can cause various campimetric deficits (usually greater bitemporal hemianopsia in the superior quadrants) and decreased visual acuity, even blindness. If the mass grows lateral to the sella turcica it can compress the oculomotor nerves, with the common motor ocular nerve being the most frequently affected, because of its more medial course in the cavernous sinus. The test of choice for the diagnosis of pituitary apoplexy is MRI, as CT may not distinguish the region clearly enough to distinguish degenerative or cystic changes from previous bleedings. The finding of erosion of the anterior clinoid processes in the skull CT may support a chronic lesion, which as a result of an acute vascular phenomenon has caused the symptoms. The management of these patients involves strict control of water and electrolyte disturbances and correction of hormonal deficits. In unstable situations as in the case described, with significant visual disturbances, surgical intervention is usually of choice, with transsphenoidal decompression.", "full_question": "A 51-year-old woman comes to the emergency department with a sudden decrease in visual acuity, severe headache, nausea and vomiting. Hypotensive and afebrile. She presented right ophthalmoparesis due to involvement of the third cranial nerve. A cranial CT scan shows a mass in the hyperdense selar region with erosion of the anterior clinoid processes. What is the best approach to follow?", "id": 34, "lang": "en", "options": {"1": "Suspect chemical meningitis derived from a ruptured epidermoid tumor and start immediate treatment with corticosteroids.", "2": "It would indicate the performance of a cerebral angiography to rule out an aneurysm, since it is most likely that we are facing a case of subarachnoid hemorrhage and the mass that is evident in the CT is a thrombosed parasellar aneurysm.", "3": "Admission to ICU and treatment of the shock suffered by the patient and once stabilized perform brain MRI for scheduled surgery.", "4": "Urgent biochemistry and hemogram, initiation of high-dose corticosteroid therapy and urgent transsphenoidal surgery.", "5": "Lumbar puncture to rule out bacterial meningitis after starting empirical antibiotherapy. Once the patient was stabilized, study of the selar mass."}, "question_id_specific": 66, "type": "NEUROLOGY AND NEUROSURGERY", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0707_2317", "title": "Pituitary apoplexy can mimic acute meningoencephalitis or subarachnoid haemorrhage.", "score": 0.012741916095684577, "content": " Pituitary apoplexy is an uncommon but life-threatening condition that is often overlooked and underdiagnosed. We report a 45-year-old man who presented to our emergency department with a sudden onset headache, acute confusion, signs of meningeal irritation and ophthalmoplegia. An initial diagnosis of acute meningoencephalitis was made, which was amended to pituitary apoplexy following thorough investigation within the emergency department.A 45-year-old man was brought to our emergency department by ambulance with a history of sudden onset of frontal headache and acute confusion. His wife provided the history. There was no significant past medical history of diabetes, hypertension, recent travel abroad, exposure to sick contacts, involvement in outdoor pursuits such as hiking/cave diving, or trauma. He worked in a bank and had been well until 24 h prior to the onset of sudden headache, which was gradually worsening in nature and associated with increasing confusion. The patient's wife reported that he had neither experienced any fevers, night sweats, or coryzal symptoms nor received any recent vaccinations. He was not on any regular medications. He was a non-smoker and occasionally consumed alcohol. There was no significant family history. On examination in the ED, his temperature was 37.6°C, his pulse was 110/min, and he was normotensive and normoglycaemic. A macular blanching rash was noted over the patient's trunk. The patient was disoriented to time and place. Neurological examination revealed reduced GCS (11/15-E3, M6, V2), marked neck stiffness, a positive Kernig's sign and a right sixth nerve palsy.A provisional diagnosis of acute meningoencephalitis was made and the patient was started on a course of intravenous antibiotics with benzyl penicillin 1.2 g, cefotaxime 2 g and acyclovir 750 mg. Baseline blood investigations revealed hyponatraemia (122 mmol/l), a white-cell count of 11 × 109/l and a C-reactive protein &gt; 250. Due to the sudden onset of the symptoms and lack of prodrome, an urgent CT head scan was performed to rule out a cerebrovascular event. The scan demonstrated an enlarged pituitary gland (3 cm in diameter) with impingement of the optic chiasm. The centre of the enlarged pituitary gland was noted to be hypodense in comparison to its periphery, which was consistent with a diagnosis of pituitary apoplexy. A subsequent MRI confirmed the diagnosis (Figure 1) of an enlarged sella containing abnormal soft tissue with increased signal intensity suggestive of haemorrhage (Figure 1A).Post-MRI a lumbar puncture was performed revealing glucose 3.4 mmol/l, protein 1.0 g/l, red cells of 53/mm3 and white cells of 174/mm3 with predominant neutrophilia. No organisms were seen, and CSF cultures and HSV DNA tests were found to be negative. Endocrinological investigations demonstrated low concentrations of thyroid hormones [TSH: 0.14 mIu/l (0.35-5.5 mlU/l), FT3: 1.1 nmol/l (1.2-3.0 nmol/l), FT4: 9.6 pmol/l (8-22 pmol/l)], gonadal hormones (LH: &lt; 1 u/l) and prolactin: 16 u/l (&lt;450 u/l). Serum FSH was 2.9 u/l (0.8-11.5 u/L) and cortisol 575 nmol/l (450-700 nmol/l). The patient was treated for hypopituitarism based on clinical and radiological findings with intravenous fluids, hydrocortisone (100 mg) and thyroxine (50 μg) as loading doses in the ED.Within 24 h of commencement of therapy the patient's GCS rose to 15, and within 48 h there was marked improvement in the right sixth cranial nerve palsy. Formal visual field assessment demonstrated temporal visual field loss in the left eye. The patient was discharged to his usual residence a week later and follow-up was organised with both the endocrinologists and ophthalmologists. Follow-up MRI demonstrated that there was no significant change in either size or signal characteristics of the pituitary fossa mass (Figure 1B)."}, {"id": "InternalMed_Harrison_10924", "title": "InternalMed_Harrison", "score": 0.011975432374784777, "content": "When bacterial meningitis is suspected, blood cultures should be immediately obtained and empirical antimicrobial and adjunctive dexamethasone therapy initiated without delay (Table 164-1). The diagnosis of bacterial meningitis is made by examination of the CSF (Table 164-2). The need to obtain neuroimaging studies (CT or MRI) prior to LP requires clinical judgment. In an immunocompetent patient with no known history of recent head trauma, a normal level of consciousness, and no evidence of papilledema or focal neurologic deficits, it is considered safe to perform LP without prior neuroimaging studies. If LP is delayed in order to obtain neuroimaging studies, empirical antibiotic therapy should be initiated after blood cultures are obtained. Antibiotic therapy initiated a few hours prior to LP will not significantly alter the CSF WBC count or glucose concentration, nor is it likely to prevent visualization of organisms by Gram’s stain or detection of bacterial nucleic acid by"}, {"id": "wiki20220301en026_4627", "title": "Subarachnoid hemorrhage", "score": 0.009900990099009901, "content": "Angiography After a subarachnoid hemorrhage is confirmed, its origin needs to be determined. If the bleeding is likely to have originated from an aneurysm (as determined by the CT scan appearance), the choice is between cerebral angiography (injecting radiocontrast through a catheter to the brain arteries) and CT angiography (visualizing blood vessels with radiocontrast on a CT scan) to identify aneurysms. Catheter angiography also offers the possibility of coiling an aneurysm (see below). In emergency department patients complaining of acute-onset headache without significant risk factors for SAH, evidence suggests that CT scanning of the head followed by CT angiography can reliably exclude SAH without the need for a lumbar puncture. The risk of missing an aneurysmal bleed as the cause of SAH with this approach is less than 1%. Lumbar puncture"}, {"id": "pubmed23n0372_4488", "title": "Pituitary abscess presenting with cranial nerve paresis. Case report and review of literature.", "score": 0.009900990099009901, "content": "Non-adenomatosus lesions of the pituitary represent a small part of the intrasellar processes and they have heterogeneous presentation. Making a precise diagnosis is of great importance, as it may lead to more efficient management. A 65-year-old man was admitted to the hospital because of headache and right cranial nerve III palsy. Basic laboratory work-up was normal whereas endocrinological assessment revealed hypopituitarism without diabetes insipidus. Plain radiography showed an enlarged sella and frontal and paranasal sinusitis. Computed tomography (CT) and magnetic resonance imaging (MRI) of the sella revealed an intrasellar lesion with extension to the sphenoid and cavernous sinuses as well as the suprasellar region, exerting pressure on the optic chiasm. On T1-weighted images the mass had a low-intensity signal with a smooth enhancing rim with bright signal. Given the presence of multiple sinusitis and imaging characteristics a pre-operative diagnosis of pituitary abscess was made. The patient was operated via transphenoidal route and purulent material was drained out. Cultures of the material were positive for Staphylococcus aureus. Antibiotics as well as cortisol replacement therapy were given. Three months later hypopituitarism persisted but there was significant improvement in the neurological findings. We report a case of an unusual presentation of a pituitary abscess. High index of suspicion, the presence of associated conditions such as pituitary tumors, meningitis or sinusitis, as well as diabetes insipidus and specific imaging features are the main diagnostic clues. Pre-operative diagnosis, which will lead to prompt antibiotic therapy and transphenoidal drainage, can decrease high mortality and morbidity associated with this disease."}, {"id": "pubmed23n0289_3646", "title": "[Bilateral chronic subdural hematomas presented with subarachnoid hemorrhage: report of two cases].", "score": 0.00980392156862745, "content": "Computed tomography (CT) findings of chronic subdural hematomas are usually diagnostic, unless hematomas are isodense and bilateral. The authors report two cases of bilateral chronic subdural hematomas, in which CT scans on admission were both misdiagnosed as delayed subarachnoid hemorrhage (SAH). The first case was a 43-year-old woman who suffered from a sudden onset of headache and nausea. She had no past history of head injury. CT scans on admission did not clearly reveal the Sylvian fissures and the mesencephalic cistern, without any mass effects. A lumbar puncture demonstrated xanthochromic cerebrospinal fluid (CSF), which was considered to be responsible for her headache. Cerebral angiography performed on day 4 failed to demonstrate any cerebral vascular disorders. Follow-up CT scans on day 7 demonstrated a high density lesion in the left subdural space. Magnetic resonance images (MRIs) confirmed a diagnosis of bilateral chronic subdural hematomas. Removal of the hematomas cleared all signs and symptoms smoothly. The second case was a 44-year-old man who was referred from another hospital because of xanthochromic CSF found by lumbar puncture. He began to suffer headache and be subject to vomiting 6 weeks earlier and these symptoms were still present on the day of admission. CT scans did not clearly show the cerebral cisterns without mass effects. Because the second lumbar puncture showed xanthochromic CSF again, SAH from aneurysm was suspected. However, emergency cerebral angiography failed to demonstrate cerebral aneurysms. MRI performed two days later demonstrated bilateral chronic subdural hematomas. Following surgery, the patient improved immediately and was discharged from hospital without any complications. In both cases, a retrospective study of the angiograms revealed the evidence of bilateral avascular areas over the convexities in the venous phase. The reason why these subdural hematomas were missed at the time of angiography was due to too much attention being paid to the arterial phase in an effort aimed only at identifying cerebral aneurysms. There are no reports of chronic subdural hematoma which demonstrated sudden onset of headache associated with xanthochromic CSF."}, {"id": "pubmed23n0253_21144", "title": "[Cerebral abscess. Clinical review of 26 cases].", "score": 0.00980392156862745, "content": "The introduction of new diagnostic and therapeutic techniques has changed the clinical attitude and consequences of brain abscesses (BA). To analyse clinical-radiological features, therapy, prognostic factors and evolution of BA in our institution. Retrospective study of all clinical records of patients diagnosed with BA from 1982 to 1992. Twenty-six patients with a mean age of 46.2 years were selected. The incidence was 2.6 patients/10,000 admission/year. Among 17 patients (65%) some extraprenchymatous infectious focus was found, which was located at the otorhynolaryngeal area in twelve patients. Mean duration of symptoms was 12.9 days, headache being the most common of them (69%). With CT 18 patients had a single mass, eight patients multiple masses, and 21 patients a ring enhancement when the contrast material was introduced. The causative organism was recovered from 15 patients. The organism recovered more frequently were Streptococcus spp, Enterobacteriaceae and Staphylococcus aureus. Twenty patients (77%) underwent surgical therapy, which consisted in ablation (12) or drainage (8). All patients received antibiotics for a mean of 37 days: the most frequent antibiotic combination used was penicillin+chloramphenicol. Six patients died (23%) and 7 remained with sequelae. Although statistically non-significant, the acute presentation was associated with a higher mortality rate, and the use of dexamethasone was associated with a lower mortality rate (p = 0.053 and 0.062, respectively). BA is associated with a high mortality rate and a high sequelae rate despite appropriate diagnostic and therapeutic measures. ORL infection is the most frequent predisposing factor. The use of dexamethasone is not associated with a higher mortality rate."}, {"id": "wiki20220301en003_97415", "title": "Headache", "score": 0.009708737864077669, "content": "One recommended diagnostic approach is as follows. If any urgent red flags are present such as visual loss, new seizures, new weakness, new confusion, further workup with imaging and possibly a lumbar puncture should be done (see red flags section for more details). If the headache is sudden onset (thunderclap headache), a computed tomography test to look for a brain bleed (subarachnoid hemorrhage) should be done. If the CT scan does not show a bleed, a lumbar puncture should be done to look for blood in the CSF, as the CT scan can be falsely negative and subarachnoid hemorrhages can be fatal. If there are signs of infection such as fever, rash, or stiff neck, a lumbar puncture to look for meningitis should be considered. If there is jaw claudication and scalp tenderness in an older person, a temporal artery biopsy to look for temporal arteritis should be performed and immediate treatment should be started. Neuroimaging"}, {"id": "pubmed23n0558_21137", "title": "[Operative treatment of anterior cranial base meningiomas].", "score": 0.009708737864077669, "content": "The aim of the study is to assess outcome of surgical treatment of anterior cranial base meningiomas with special emphasis on the evaluation of visual skills after surgery. A series of 52 consecutive patients operated on for anterior cranial base meningioma in the last ten years is reported. 18 patients had decreased visual acuity on admission, 6 experienced blindness in one eye, and 4 were totally blind; defects of visual fields were found in 17 patients. Primary optic atrophy and secondary optic atrophy were noted in fundcoscopy in 20 and 2 patients, respectively. Papilloedema was found in 3 patients. Meningiomas were resected radically (Simpson I surgery) in 13 patients and in 34 patients Simpson II surgery with coagulation of the dural attachment was made; meningiomas were partially removed in 5 patients (Simpson IV surgery). In one patient dense hemiparesis occurred after the surgery, and 3 others presented adynamic syndrome, one of whom made a good recovery in follow-up examination. Two patients died: the first one due to a large brain oedema and the second one due to myocardial infarction after uncomplicated postoperative course. The postoperative course in the other 46 patients was uneventful with good outcome. Visual acuity improved in 15 cases and did not change in 10 patients; visual acuity further decreased in 3 patients. Visual recovery is significantly related to preoperative visual acuity values of no less than 0.3 D and to the presence of normal optic discs on fundoscopic examination additionally tumour size less than 3.5 cm favourably affects visual prognosis in meningiomas of tuberculum sellae."}, {"id": "pubmed23n0765_23058", "title": "Sudden headache, third nerve palsy and visual deficit: thinking outside the subarachnoid haemorrhage box.", "score": 0.009615384615384616, "content": "A 75-year-old lady presented with sudden severe headache and vomiting. Examination was normal, and CT and lumbar puncture not convincing for subarachnoid haemorrhage. Shortly thereafter, she developed painless diplopia. Examination confirmed right third cranial nerve palsy plus homonymous left inferior quadrantanopia. Urgent cerebral MRI with angiography was requested to assess for a possible posterior communicating artery aneurysm, but revealed an unsuspected pituitary mass. Pituitary adenoma with pituitary apoplexy was diagnosed. Pituitary apopolexy is a syndrome comprising sudden headache, meningism, visual and/or oculomotor deficits, with an intrasellar mass. It is commonly due to haemorrhage or infarction within a pituitary adenoma. Treatment includes prompt steroid administration, and potentially surgical decompression. While subarachnoid haemorrhage is an important, well-recognised cause of sudden severe headache, other aetiologies, including pituitary apoplexy, should be considered and sought. "}, {"id": "pubmed23n0762_4733", "title": "Painful ophthalmoplegia: the role of imaging and steroid response in the acute and subacute setting.", "score": 0.009615384615384616, "content": "Although reports of single cases of painful ophthalmoplegia (PO) are common, studies considering larger case series are lacking. Here, we aimed to determine the relative frequencies of ocular neuropathies, the causes, the usefulness of diagnostic procedures and the role of steroid treatment in PO. Between January 2006 and September 2012, 149 patients' charts who presented with diplopia in our emergency department were studied retrospectively. 34 of them met the inclusion criteria that included recent (≤3 days) symptom onset and a minimum of diagnostic work. 32% of single or combined ocular motor nerve palsies were of diabetic microvascular etiology and most of them were IIIrd or VIth nerve neuropathies. The most useful, in terms of sensitivity and specificity of diagnostic test in the acute setting was ESR, whereas MR-angiography and focused cavernous sinus imaging led to diagnosis in the post-acute phase. Pain response to steroids was non-specific, in contrast to palsy improvement after steroid administration which was indicative of Tolosa-Hunt syndrome or temporal arteritis. Although acute and subacute PO might be intuitively associated with Tolosa-Hunt syndrome or sinister pathology such as aneurysmal hemorrhage, our data show that these causes are far less common than diabetic microvascular palsies. Brain CT, MR-imaging of brainstem, cerebellum or hemispheres, CSF analysis and pain response to steroids are nonspecific and hence less helpful in order to arrive at a diagnosis. Instead, improved ocular motility after steroid treatment, as well as MR-angiography and cavernous sinus imaging appear more useful for this purpose."}, {"id": "wiki20220301en228_5192", "title": "Mollaret's meningitis", "score": 0.009523809523809525, "content": "Investigations include blood tests (electrolytes, liver and kidney function, inflammatory markers and a complete blood count) and usually X-ray examination of the chest. The most important test in identifying or ruling out meningitis is analysis of the cerebrospinal fluid (fluid that envelops the brain and the spinal cord) through lumbar puncture (LP). However, if the patient is at risk for a cerebral mass lesion or elevated intracranial pressure (recent head injury, a known immune system problem, localizing neurological signs, or evidence on examination of a raised ICP), a lumbar puncture may be contraindicated because of the possibility of fatal brain herniation. In such cases, a CT or MRI scan is generally performed prior to the lumbar puncture to exclude this possibility. Otherwise, the CT or MRI should be performed after the LP, with MRI preferred over CT due to its superiority in demonstrating areas of cerebral edema, ischemia, and meningeal inflammation."}, {"id": "pubmed23n0215_13590", "title": "Review of 1,000 consecutive cases of severe head injury treated before the advent of CT scanning.", "score": 0.009523809523809525, "content": "This is a review of 1,000 consecutive cases of severe head injury admitted to our Neurosurgical Department between January 1973 and August 1976, before the advent of CT scanning. All patients were comatose following head injury (GCS less than or equal to 8) and were treated homogeneously by the same neurosurgical team by a protocol that included immediate resuscitation on arrival, diagnosis of intracranial lesions by angiography, early surgery when needed, mechanical ventilation, steroids, and mannitol. Extracranial lesions, even if preponderant, were treated by various specialists in the Neurosurgical Department, which for all practical purposes operated as an Emergency Department. Admission criteria were very broad with no preadmission selection. The overall mortality for this series was 45%. A little less than half the patients made good recoveries or remained moderately disabled (47%); 6% were severely disabled, and 2% survived in a persistent vegetative state. More than two-thirds of the patients were brought to our Neurosurgical Department after a short stay at a general hospital; 72% were admitted within 6 hours of injury; 71% were traffic accident victims; and 34% had significant associated extracranial injuries. Carotid angiography was performed in 78% of the patients and indicated the presence of an intracranial haematoma requiring surgery in 36% of the whole series. Mortality was significantly higher in operated than in unoperated patients (56% versus 39%); those treated surgically, however, were older, in worse clinical condition, and showed a higher incidence of acute subdural haematomas associated with brain contusion. Carotid angiography proved very effective in revealing the presence of an expansive lesion but failed to reflect the severity of brain damage, since the group with \"negative\" angiograms showed a high mortality (52%). Patients with a lucid interval had a higher percentage of surgical lesions than those with immediate coma (58% versus 26%); but fully 42% of them did not require surgery, and 25% had negative angiograms. From the prognostic point of view the clinical data elicited after initial resuscitation were highly predictive of the outcome: some individual neurological signs, such as mydriasis, posturing and eye movements, were not inferior to the GCS score in that respect. Age also proved a strong predictor, since elderly patients are more likely to have severe subdural and parenchymal lesions and their clinical severity is accordingly greater.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "wiki20220301en011_61500", "title": "Intracranial aneurysm", "score": 0.009433962264150943, "content": "Subarachnoid bleed If an aneurysm ruptures, blood leaks into the space around the brain. This is called a subarachnoid hemorrhage. Onset is usually sudden without prodrome, classically presenting as a \"thunderclap headache\" worse than previous headaches. Symptoms of a subarachnoid hemorrhage differ depending on the site and size of the aneurysm. Symptoms of a ruptured aneurysm can include: a sudden severe headache that can last from several hours to days nausea and vomiting drowsiness, confusion and/or loss of consciousness visual abnormalities meningism dizziness Almost all aneurysms rupture at their apex. This leads to hemorrhage in the subarachnoid space and sometimes in brain parenchyma. Minor leakage from aneurysm may precede rupture, causing warning headaches. About 60% of patients die immediately after rupture. Larger aneurysms have a greater tendency to rupture, though most ruptured aneurysms are less than 10 mm in diameter."}, {"id": "pubmed23n0627_15825", "title": "[Indication of neuro-imaging for the initial management and the follow-up of acute community-acquired bacterial meningitis].", "score": 0.009433962264150943, "content": "Lumbar puncture is the best way to prove bacterial meningitis. It should be performed without any delay if the diagnosis is suspected. Herniation is a rare complication of LP. CT is normal in most cases of purulent meningitis, including those complicated by a subsequent herniation; normal CT results does not mean that performing a LP is safe. Three main clinical features can help determine which patient is at risk of herniation and should have a CT before LP. This risk has to be determined rapidly in the emergency ward while assessing anamnestic data, localization signs or symptoms, and level of consciousness. Cranial imaging (mainly MRI) is useful in the course of bacterial meningitis. Patients who do not respond well to treatment or with atypical presentation, persistence of fever, or new neurological signs should undergo brain imaging; MRI and CT may identify subdural effusions, brain abscesses, empyemas, hydrocephaly, or brain parenchymal changes (cerebritis, infarction, hemorrhage). CT and MRI are useful to screen for an ENT cause of bacterial meningitis, and mandatory in case of pneumococcal meningitis. Numerous MRI sequences are useful to identify bacterial meningitis complications: SE T1 without and with gadolinium injection, SE T2, FLAIR, gradient-echo T2, diffusion weighted imaging, MR angiography."}, {"id": "wiki20220301en014_91322", "title": "Aneurysm", "score": 0.009345794392523364, "content": "Diagnosis Diagnosis of a ruptured cerebral aneurysm is commonly made by finding signs of subarachnoid hemorrhage on a computed tomography (CT) scan. If the CT scan is negative but a ruptured aneurysm is still suspected based on clinical findings, a lumbar puncture can be performed to detect blood in the cerebrospinal fluid. Computed tomography angiography (CTA) is an alternative to traditional angiography and can be performed without the need for arterial catheterization. This test combines a regular CT scan with a contrast dye injected into a vein. Once the dye is injected into a vein, it travels to the cerebral arteries, and images are created using a CT scan. These images show exactly how blood flows into the brain arteries."}, {"id": "pubmed23n1143_20721", "title": "A point-of-care evaluation after visual loss following paraclinoid aneurysm repair: the role of sonographic and pupillometer assessment.", "score": 0.009345794392523364, "content": "Visual complications represent common deficits following surgical or endovascular repair of paraclinoid aneurysms. Different etiologies should be investigated to prevent devastating consequences. Herein we present a point-of-care evaluation to investigate sudden visual loss after coiling of paraclinoid aneurysms. A 20-year-old male was admitted for a sudden headache. Head computed tomography showed a subarachnoid hemorrhage and subsequent angiography revealed a 9-mm left supraclinoid aneurysm of the internal carotid artery treated with endovascular coil embolization. Thirty minutes after intensive care unit admission, the patient reported a left amaurosis. To exclude secondary etiologies, an immediate evaluation with point-of-care devices (color-doppler and B-mode ultrasound and automated pupillometry) was performed. Sonographic evaluations were negative for ischemic/thrombotic events and neurologic pupil index within physiological ranges provide evidence of third cranial nerve responsiveness. The symptomatology resolved progressively over 120 minutes with low-dose steroid therapy, 30° head-of-bed elevation, and blood pressure management. Visual deficits can occur after endovascular procedure and should be investigated. Suspected visual loss is a neurological emergency that deserves a prompt evaluation. Ultrasound and automated pupillometry have proved to be an effective, rapid, reliable, and non-invasive combination for a clinical decision-making strategy in the management of post-procedural acute visual deficits."}, {"id": "pubmed23n0217_5796", "title": "[Two cases of cerebral aneurysms combined with polycystic kidneys].", "score": 0.009259259259259259, "content": "Two cases of cerebral aneurysm combined with polycystic kidneys (PCKs) were presented. Case 1, a 24-year-old hypertensive male, was referred to our clinic owing to sudden onset of severe headache at August 20, 1982. Neurological findings on admission were stuporous, right vitreous hemorrhage (so-called Terson's syndrome), and hypertension. CT scans showed subarachnoid hemorrhage, and right MCA bifurcation aneurysm with marked vasospasms by cerebral angiography was revealed. Intentional delayed operation with V-P shunt was performed. He discharged with mild left upper limb paresis, and visual impairment on the right. Bilateral PCKs were confirmed by postoperative DIP and CT scan. Case 2, a 51-year-old female, who suddenly complained of severe headache, was referred to our department 3 days after subarachnoid hemorrhage. One year previously, she had been pointed out PCKs. Neurological findings on admission at February 29, 1980, were drowsy, left third cranial nerve palsy, and hypertension. Cerebral angiography showed multiple aneurysms (bilateral IC-PC &amp; A-com). Neck clipping (1-IC-PC &amp; A-com) and coating (r-IC-PC) were performed at the next day of admission, and V-P shunt operation was followed about 8 weeks after first operation. About 2 weeks after discharge, she suddenly became loss of consciousness and expired. Autopsy revealed intracerebral hemorrhage in left basal ganglia and thalamus. Both kidneys were PCKs of Potter type 3 and cysts of the liver were also noted. In young hypertensive patients with cerebral aneurysms, it should be in mind whether PCKs may be combined or not, and cerebral angiography in PCKs were reasonable to find out harbored cerebral aneurysm.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0267_9372", "title": "[Pituitary abscess, treated by medication].", "score": 0.009259259259259259, "content": "Pituitary abscesses are rare. The case reported here concerns a 28-year old African. Gradual development of diplopia over 6 months was the first clinical manifestation. Three months later this development had reached a more severe and infectious context, with complete right ophthalmoplegia, meningitis and coma (GCS = 9). CT scan showed an image in favour of a pituitary abscess with suprasellar extension, associated with thrombophlebitis of the cavernous sinus. An antibiotic therapy consisting of cefotaxime and metronidazole administered for 1 month, and netilmicin for 15 days succeeded in controlling the infectious syndrome. This resulted in cure of visual disorders, reduction in size of the CT scan image and reconstruction of the pituitary sella which had been destroyed. The diagnosis of pituitary abscess should be made when confronted with an infectious syndrome (unexplained fever, repeated meningitis). CT does not recognize the nature of the hypophyseal mass it shows: necrosis of a pituitary adenoma, giant aneurysm or craniopharyngioma may mimic local infection. Surgery confirms the diagnosis and is regarded as the best treatment. The patient's life is threatened when meningitis is present, and the functional prognosis is poor when recovery from visual disorders is compromised due to late diagnosis. In this paper a comparative analysis of the clinical course of the disease and therapeutic data in our patient is presented and compared with other reported cases."}, {"id": "pubmed23n0135_1804", "title": "[Acute spontaneous subdural hematoma associated with multiple aneurysms--a case report].", "score": 0.009174311926605505, "content": "A case of acute spontaneous subdural hematoma associated with three aneurysms is reported. On March 12, 1984, a 47-year-old woman experienced the sudden onset of severe headache over the bilateral frontal region and vomiting. Three hours later, she was transferred to our hospital by ambulance car because of continuous headache and vomiting. She had no history of head trauma. She had been medicated hypertension for five years. On admission she suffered from headache and nausea. But there was no clinical sign in physical and neurological examinations. The meningeal irritation was not present, but lumbar puncture showed slightly pinky CSF with normal pressure. A plain computed tomographic scan showed a thin high density mass in the left temporal extra-axial region and the slight deviation of the midline structures to the right. Left carotid arteriogram showed an avascular region over the left cerebral convexity, an aneurysm of the left A2-A3 junction and a questionable aneurysm of the bifurcation of left middle cerebral artery. Right carotid arteriogram showed an aneurysm of the bifurcation of right middle cerebral artery. We diagnosed this case as an acute subdural hematoma by CT scan and arteriogram. We were perplexed preoperatively whether this bleeding was spontaneous or secondary to the rupture of aneurysm, and we could not deny the possibility of a ruptured aneurysm. On March 15, 1984, three days after onset, operation was performed. At operation, a small subdural hematoma was removed, and the underlying cortex was normal.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n1012_24691", "title": "Isolated Posterior Spinal Artery Aneurysm Presenting with Spontaneous Thrombosis After Subarachnoid Hemorrhage.", "score": 0.00909090909090909, "content": "The cause of subarachnoid hemorrhage is more likely to be intracranial than spinal. Bleeding, although common with spinal arteriovenous malformations and spinal cord tumors, rarely occurs with ruptured isolated spinal artery aneurysms. Here, we report a case of isolated thoracic posterior spinal artery aneurysm presenting with thrombosis after subarachnoid hemorrhage. A 67-year-old woman presented with sudden-onset nausea and low back and right thigh pain that worsened with movement. Computed tomography (CT) and magnetic resonance imaging (MRI) scans of the head suggested a small subarachnoid hemorrhage in the high-convexity sulcus, and lumbar puncture showed bloody cerebrospinal fluid. There was no apparent intracranial aneurysm on CT angiography; however, spinal MRI showed a lesion on the right side of the spinal cord at Th10. Contrast-enhanced CT showed an enhancing lesion at this site on day 7 that was not present on day 15. Selective right Th10 intercostal artery angiography on day 22 showed no evidence of aneurysm. The lesion was suspected to be a thrombotic spinal artery aneurysm. Given the unclear natural history of this entity, surgery was performed on day 36. After right Th10 hemilaminectomy and opening the dura, the arachnoid and adhesions were found to be thickened. A fusiform-shaped thrombosed aneurysm continuous with the radiculopial artery was removed. The patient was discharged without neurologic deterioration. Isolated spinal artery aneurysm is a rare cause of subarachnoid hemorrhage. It is expected that additional cases will clarify the natural history and indications for treatment."}, {"id": "pubmed23n0323_9623", "title": "[Brain abscess. Clinicomicrobiologic study and prognostic analysis of 59 cases].", "score": 0.00909090909090909, "content": "Clinical, microbiological, therapeutic and prognostic characteristics of brain abscesses were analyzed as well as the influence of CT in their evolution. Retrospective study of 59 patients with the diagnosis of brain abscess of bacterial source before (group A) and after (group B) the introduction of CT (25 and 34 patients, respectively). The most common symptom was headache (76.3%) and the most common abnormality in physical examination was a decrease in the level of consciousness (61%) and this abnormality was associated with a higher mortality rate (13% versus 41.6%; p &lt; 0.05) and also a higher proportion of neurologic sequelae (50% versus 85.7%; p &lt; 0.05). The diagnosis was obtained earlier in group B. The hematogenous source predominated (32.2%); an adjacent source was identified in 28.8% and an apparent source was not recognized in 27.2% (40% in group A versus 17.6% in group B). Anaerobic and microaerophilic streptococci were the bacteria recovered most frequently. Gram-negative aerobic bacteria were the most common in otogenic abscesses. The use of corticosteroids had no influence upon mortality, but it was associated with a lower percentage of neurological sequelae (40% versus 14%; p &lt; 0.05). The introduction of CT decreased mortality (40% in group A versus 23.5% in group B, although this difference was not significant) and also sequelae (86.6% in group A versus 57.6% in group B; p &lt; 0.05). Leaving apart cases of bacterial endocarditis, in which death was due to the underlying heart disease and a systemic sepsis picture, mortality attributed to brain abscess was 20.3%. The introduction of CT has meant a significant breakthrough for the diagnosis, treatment and follow-up of these patients and has contributed to improvement in survival. In our series, the diagnosis of brain abscess was obtained earlier and the number of brain abscesses with no apparent source has decreased since the introduction of CT. Moreover, CT sensitivity is really good for locating multiple abscesses. Overall, the prognosis of these patients has improved since the introduction of this technique. Nevertheless, brain abscess is still associated with a relevant morbi-mortality rate."}, {"id": "pubmed23n0041_5426", "title": "[Rupture of intracranial aneurysm due to cerebral angiography (author's transl)].", "score": 0.009009009009009009, "content": "The authors report a case of rupture of intracranial aneurysm by angiography which was done four hours after the subarachnoid hemorrhage. Case; A thirty-one year old male patient was brought to our outpatient's clinic by ambulance because of conscious loss and convulsive seizure on Feb. 5th, 1974. Lumbar puncture showed grossly hemorrhage in the CSF. Immediately he was hospitalized and administered anticonvulsants, hypotensive drugs, antibrinolytic agents and corticosteroid. His signs and symptoms on admission were mild headache, nausea, nuchal rigidity, anisocoria (right greater than left) and left hyper reflexia. This attach was his second. (He first noted the bleeding attack on January 30, 1974). Four hours after this attack cerebral angiography was done under local anesthesia with heavy premedication. Puncture of common carotid arteries were uneventful. Three injections of 60% Conray, at the dose of 8 ml each, were performed and three films were taken. Few minutes after injections, he suddenly became unconscious and ceased respiration for a few seconds. Blood pressure was 210 mmHg at systolic, although 120 mmHg two minutes before. Immediately resuscitation started. His respiration reappeared within 0.5 minute and his vital signs gradually improved. We stopped examination. When returned to his bed, right pupil dilated and optic fundi showed bleeding bilaterally. Arteriography showed a large dumbbell shaped aneurysm at the trification of the right middle cerebral artery but no finding of hematoma (Fig. 1). We decided emergency operation at once. When started the operation his both sides pupil dilated, B.P. was very low. OPERATION: Right side large frontolateral craniectomy was done. Large subdural hematoma (Fig. 2), severe diffuse subarachnoid hemorrhage (Fig. 3) and intracerebral hematoma were found. Aneurysmal neck clipping was successfully done. His level of consciousness was semicomatous. But gradually his state deteriorated and died one week after the operation. There was severe edema in both sides cerebrum. The brain stem, especially interbrain, and pons, had fallen into softening, so called respirator brain. This complication of angiography is very rare. This case is the 24th reported case of the ruptured aneurysm by angiography."}, {"id": "Neurology_Adams_5629", "title": "Neurology_Adams", "score": 0.009009009009009009, "content": "The initial elevation of intracranial pressure and threatening temporal lobe or cerebellar herniation can be managed by the use of intravenous mannitol (or hypertonic saline) and dexamethasone, 6 to 12 mg q6h. If improvement does not begin promptly, it becomes necessary to aspirate the abscess stereotactically or remove it by an open procedure that also allows precise etiologic diagnosis by Gram stain and culture. The decision regarding aspiration or open removal of the abscess is governed by its location and the course of clinical signs and by the degree of mass effect and surrounding edema as visualized by repeated scans."}, {"id": "pubmed23n0305_22028", "title": "[A 64-year-old woman with severe headache and progressive disturbance of consciousness].", "score": 0.008928571428571428, "content": "We report a 64-year-old woman who developed nausea, headache, and consciousness disturbance. She was well until four years before the onset of her neurologic illness when (April of 1990 at her 59 years of the age) she was found to have an early cancer in her anterior wall of the lower stomach. Subtotal gastrectomy was performed and the operative result was reported as curative. Four years after the surgery (December of 1994 at her 64 years of the age), she noted suboccipital headache and nausea which had become progressively worse and she was admitted to our service on May 24, 1995. On admission, she appeared chronically ill but general physical examination was unremarkable with normal vital signs. Neurologically she was alert and not demented, and the higher cerebral functions were intact. Cranial nerves were also unremarkable. She was able to walk in tandem and on heels. No motor weakness or ataxia was noted. Deep tendon reflexes were moderately increased, however, no Babinski sign was noted. Although she had headache, no meningeal signs were seen. Slight superficial and vibratory sensory loss was noted in both feet. Routine blood work was again unremarkable except for slight increase in CEA to 8.3 ng/dl (N &lt; 5 ng/dl). The opening pressure of lumbar CSF was 180 mm H2O and the CSF contained 39 cells/microliter, 79 mg of protein, and 10 mg/dl of glucose. Approximately half of the cells were atypical malignant cells. Plain CT was unremarkable, however, tentorial border showed enhancement after contrast infusion. FGS showed no malignant tumors in the stomach. She was treated with intravenous glycerol and whole brain radiation, however, she continued to complain of severe headache, and her sensorium started to be disturbed one month after the admission. Follow-up cranial CT scan revealed enlargement of the lateral and the third ventricles. Her consciousness progressively deteriorated and she became comatose three months after the admission. Repeated cranial CT scan showed enlargement of the ventricles, but no mass lesions were seen within the brain. She developed respiratory arrest on September 25 of the same year. She was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had a gastric cancer with meningeal seeding developing meningeal carcinomatosis. The cause of deep coma was ascribed to damage of cerebral cortical areas secondary to metastatic carcinoma cells and fibrinous materials in the surface of the brain. Postmortem examination revealed thickening and clouding of leptomeninges of the cerebral convexity. On histologic observation, patchy areas of fibrous thickening were seen in the cerebral leptomeninges; in such areas, adenocarcinomatous cells were seen scattered. The basal meninges were free of carcinoma cells, however, leptomeninges of the cerebellum and brain stem tegmentum contained scattered carcinoma cells. The lateral and the third ventricles were enlarged, however, insides of the brain were free of pathologies; the ependymal layer were intact. In the stomach no carcinoma cells were remaining. Pneumonic changes were seen in the right upper and the left lower lobes which appeared to be the direct cause of her death. No evidence of tentorial herniation was noted. The cause of her deep coma was not clearly determined, however, combination of hydrocephalus and cortical malfunction due to leptomeningeal carcinoma cell infiltration and fibrinous material accumulation appeared to have played a role."}, {"id": "pubmed23n0087_8714", "title": "[A case of favorable outcome of conservative treatment of acute otogenic cerebellar abscess].", "score": 0.008928571428571428, "content": "The authors report a case of conservatively cured abscess in the left cerebellar hemisphere demonstrated by CT. The patient was admitted in serious condition to a neurotraumatology centre. After pharmacological treatment a quick improvement of patient's health was achieved and control CT examination during his stay in hospital and after discharge from hospital confirmed the relation between clinical improvement and regression of changes in CT. The presented case points to the possibility of conservative treatment of brain abscesses if the patient meets certain clinical criteria, and CT provides the possibility of repeated checking of the dynamics of intracranial lesion."}, {"id": "pubmed23n0975_22594", "title": "Recurrent Chemical Meningitis Due to Parasellar Epidermoid Cyst.", "score": 0.008849557522123894, "content": "Intracranial epidermoid cysts are exceedingly rare lesions that result from a disorder of gastrulation. They are seen only in the pediatric patient population. We describe a 44-year-old Hispanic woman who presented with acute confusion. The family reported two months of progressive headaches and two weeks of fever, blurred central vision, and restricted visual fields. On examination, the patient appeared ill, with a low-grade fever and stiff neck. Neurological testing was limited but grossly non-focal. Computerized tomography (CT) of the head and magnetic resonance imaging (MRI) of the brain showed a large cystic mass arising in the sella, where it displaced the normal pituitary gland. Cerebrospinal fluid (CSF) showed mildly elevated opening pressure with high protein, low glucose, and neutrophilic pleocytosis. Extensive serum and CSF evaluation were negative for infectious agents. The patient was initially started on empiric treatment for presumed infectious meningoencephalitis. As tests for bacterial and viral pathogens were normal, she was switched to fluconazole. The mental status returned to normal and she was discharged home with close follow up. She returned one month later with a recurrent headache, nausea, and stiff neck. The examination showed meningismus but was otherwise non-focal. MRI of the brain showed no change in the parasellar mass. Repeat CSF showed an even higher white blood cell (WBC) count and protein with continued hypo-glycorrhachia. She underwent trans-nasal trans-sphenoidal hypophysectomy and pathology revealed a squamous epithelium-lined keratin-filled cyst suggestive of an epidermoid cyst. The patient responded well to surgery and was discharged on pituitary hormone supplements alone. To our knowledge, this is a first adult case of recurrent chemical meningitis secondary to a ruptured epidermoid cyst in the sella."}, {"id": "pubmed23n0713_22385", "title": "Subtentorial subdural empyema: report of two cases and review of the literatures.", "score": 0.008849557522123894, "content": "Subtentorial subdural empyema is a rare form of intracranial suppuration. We     present two cases treated at our department within the last 11 years. The common     source was an ear infection. Both patients presented with headache, fever,     vomiting and stiff neck. Only one patient had disturbed consciousness. Both     patients received aggressive antibiotic therapy. The first patient was treated     with suboccipital craniectomy and evacuation of pus collection, while the second     patient was treated conservatively with antibiotics and ventriculoperitoneal     shunt for his associated supratentorial hydrocephalus. Both blood cultures and     empyema collection were sterile. Neuroimaging with computed tomography and     magnetic resonance imaging permitted accurate diagnosis and localization of the     purulent collections. At follow up of 11 years for the first case and 10 months     for the second, both patients had complete neurological recovery except for     right mild sixth nerve palsy in the patient with conservative treatment."}, {"id": "pubmed23n0984_919", "title": "Rupture of Thrombosed Cerebral Aneurysm During Antithrombotic Therapy for Ischemic Stroke: Case Report and Literature Review.", "score": 0.008771929824561403, "content": "Thrombosed cerebral aneurysm (TCA) can cause cerebral infarction. However, treatment of cerebral infarction due to embolism from TCA is controversial because of the risk of rupture, and no consensus has been established for the treatment of patients with this condition. A 75-year-old woman suffered left hemiparesis. Computed tomography (CT) showed a high-density round mass in the right sylvian fissure, which was suspected to be a nonruptured TCA. Magnetic resonance (MR) angiography and CT angiography demonstrated an aneurysm in the distal part of the right middle cerebral artery with poor opacification of most of the aneurysm, suggesting partial thrombosis. Diffusion-weighted MR imaging revealed high intensity in the right frontal lobe, in a distribution distal to the aneurysm. The diagnosis was cerebral infarction due to embolism from a partially thrombosed aneurysm. She was treated with antithrombotic therapy. On day 4, she suddenly became comatose. CT and CT angiography revealed subarachnoid hemorrhage (SAH) and enlarged opacification in the aneurysm, respectively. She underwent neck clipping of the aneurysm, but her neurologic improvement was poor. TCA causing ischemic stroke followed by SAH is extremely rare, with only 4 previous reported cases. All patients had received antithrombotic therapies, and most aneurysms had ruptured within a few days after starting antithrombotic therapy. The outcomes were extremely poor. We suggest that antithrombotic therapy might be avoided for these patients. Early surgical treatment without antithrombotic therapy is recommended to prevent aneurysm rupture and recurrent distal embolism from the TCA."}, {"id": "pubmed23n0022_9427", "title": "[Clinical onset of meningitis in chromophobe adenoma of the pituitary: 2 cases].", "score": 0.008771929824561403, "content": "Two cases of purulent meningitis in subjects with misinterpreted chromophobe adenoma of the hypophysis are presented. Radiography in the acute stage revealed typical enlargement of the sella turcica and thus established the diagnosis, which was eventually confirmed histologically during surgery. The frequency with which this complication has occurred in a personal series is contrasted with its rarity in the literature, particularly neurosurgical works. It is felt, therefore, that the association of adenoma and meningitis is more common than is generally supposed. Routine cranial radiography is recommended in all cases of purulent meningitis, especially in young subjects and adults, from the outset. This will reveal hypophyseal involvement and enable replacement hormonal therapy with cortisone to be added to symptomatic and antibiotic management in cases of concomitant hypophyseal and adrenal insufficiency."}, {"id": "pubmed23n0366_21006", "title": "[A case of delayed subrachnoid hemorrhage from vertebrobasilar artery dissecting aneurysm].", "score": 0.008695652173913044, "content": "We report a case of delayed subarachnoid hemorrhage (SAH) from a vertebrobasilar artery dissecting aneurysm (VBA-DA). The patient was a healthy 32-year-old woman with a sudden onset of severe occipitalgia. Next day, her headache improved gradually, and she consulted with our department. Although we initially suspected that she was suffering from SAH, neurological findings, CT, and cerebrospinal fluid examination did not reveal any abnormal conditions, including SAH. Therefore, she was treated conservatively with analgesics. Twelve days after the initial onset of the headache, she was admitted because of severe re-attack of headache, rt. hemiparesis with rt. oculomotor nerve palsy and loss of consciousness. CT revealed moderate SAH and cerebral angiograms showed VBA-DA. After the cerebral angiography, bleeding reoccurred two times and she lost her life. We present the case, review the literature and discuss the relationship between presenting symptom of headache and non-hemorrhagic VBA-DA. A few cases of non-hemorrhagic VBA-DA have been reported in the literature in which the only presenting symptom was headache, followed by delayed SAH from non-hemorrhagic dissecting aneurysm. Consequently, we concluded that her initial symptom of headache was due to dissection of vertebrobasilar artery, and that SAH was due to delayed hemorrhage of non-hemorrhagic VBA-DA. Even when neurological findings, CT and cerebrospinal fluid examination reveal no abnormalities in the early stage after the sudden onset of headache, especially in the occiptal or nuchal regions, non-hemorrhagic VBA-DA, which has a risk of fatal hemorrhage, cannot be ruled out with certainty. Therefore, MRI, MRA, three-dimensional CT, or cerebral angiography should be performed in such cases."}, {"id": "pubmed23n0120_12084", "title": "[A case of multiple septic intracranial aneurysms--successful treatment with surgical and conservative therapy in the same case].", "score": 0.008695652173913044, "content": "Here reported is a case of multiple septic intracranial aneurysms which were successfully treated with surgical and conservative therapy. A 44-year-old man was admitted to our hospital because of headache, vomiting and visual disturbance. He had had a fever and had been under treatment for a respiratory tract infection during the preceding 3 months. Physical examination on admission revealed pansystolic heart murmur over the cardiac apex. Neurological examination revealed neck stiffness, papilledema and right homonymous hemianopsia. Laboratory data showed the presence of inflammatory process. A CT scan showed a high density area in the left occipital region, and vertebral angiography showed a saccular aneurysm on a distal branch of the left occipitotemporal artery. Fourteen days after admission, the operation of clipping the neck of the aneurysmal artery was performed and the hematoma evacuated to lower the increased intracranial pressure. He had been well after the operation until 3 weeks later when a follow-up angiography showed a new unruptured aneurysm on a distal branch of the right middle cerebral artery with a relapse of the infection. Then, he was treated with appropriate antibiotics and antifibrinolytic agents. A repeated angiography 1 month later showed resolution of the aneurysm. The mechanism of resolution of septic aneurysm and its treatment are discussed."}, {"id": "wiki20220301en623_12323", "title": "Recurrent painful ophthalmoplegic neuropathy", "score": 0.008620689655172414, "content": "Magnetic resonance angiography (MRA) or CT angiography (CTA) can be used to examine cerebral blood vessels and to rule out vascular abnormalities, such as an aneurysm. In cases where intracranial vascular lesions, for example, subarachnoid hemorrhage cannot be completely ruled out after performing MRA or CTA, physicians may consider using traditional digital subtraction angiography (DSA) for more detailed investigation. Laboratory tests Lumbar punctures and blood tests might be performed on those with RPON to identify other possible causes of cranial neuropathy, including diabetes, inflammatory diseases, infections, tumors, and other systemic diseases that involve either the central nervous system or the peripheral nervous system. The detection of abnormalities in these tests suggests that RPON is highly unlikely to be the culprit in cranial neuropathy, and more diagnostic tests should be done to find out the underlying condition."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[144, 571]], "word_ranges": [[21, 89]], "text": "the risk of malignization, i.e. of developing a gestational trophoblastic neoplasm, ranges from 5 to 20% depending on whether it is a partial mole or a complete mole, respectively. According to this protocol: \"Patients will be monitored weekly with hcg dosage until it becomes undetectable, for three consecutive times. After that the monitoring will be monthly, for six months and then every two months for another six months."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The answer is 2. According to the SEGO (Spanish Society of Gynecology and Obstetrics) in its 2005 protocol \"Gestational trophoblastic disease\", the risk of malignization, i.e. of developing a gestational trophoblastic neoplasm, ranges from 5 to 20% depending on whether it is a partial mole or a complete mole, respectively. According to this protocol: \"Patients will be monitored weekly with hcg dosage until it becomes undetectable, for three consecutive times. After that the monitoring will be monthly, for six months and then every two months for another six months. Based on the available data, a follow-up of three to six months is recommended for partial mole and 12 months for complete mole.\" Therefore, subsequent controls are strictly necessary.The risk of a new molar gestation, although increased compared to the normal population, is not 50%.", "full_answer_no_ref": "[HIDDEN] According to the SEGO (Spanish Society of Gynecology and Obstetrics) in its 2005 protocol \"Gestational trophoblastic disease\", the risk of malignization, i.e. of developing a gestational trophoblastic neoplasm, ranges from 5 to 20% depending on whether it is a partial mole or a complete mole, respectively. According to this protocol: \"Patients will be monitored weekly with hcg dosage until it becomes undetectable, for three consecutive times. After that the monitoring will be monthly, for six months and then every two months for another six months. Based on the available data, a follow-up of three to six months is recommended for partial mole and 12 months for complete mole.\" Therefore, subsequent controls are strictly necessary.The risk of a new molar gestation, although increased compared to the normal population, is not 50%.", "full_question": "A 24-year-old woman, primigestation, suffers a spontaneous abortion at 7 weeks gestation. The anatomopathological study of the abortive remains indicates molar disease. We should inform you that:", "id": 343, "lang": "en", "options": {"1": "The risk of a new molar gestation in a future pregnancy is 50%.", "2": "You should not become pregnant until periodic controls and have spent one year with negative BHCG levels.", "3": "Subsequent controls are not necessary if the evacuation of the trophoblastic tissue was complete.", "4": "It is necessary to perform periodic controls since 40% of the cases will develop a gestational trophoblastic neoplasia.", "5": NaN}, "question_id_specific": 158, "type": "GYNECOLOGY AND OBSTETRICS", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0403_4413", "title": "[Gestational trophoblastic tumors and recent clinical information].", "score": 0.018442971273159952, "content": "Recent clinical advances in the field of gestational trophoblastic diseases are described. WHO modified its risk factor scoring system. This change was proposed to combine both the basic FIGO anatomic staging with the modified WHO risk factor scoring system. Patients who score as low-risk are treated with single agent chemotherapy, such as methotrexate (MTX), and patients refractory to MTX are treated with a combination chemotherapy, EMA/CO. Patients who score as high-risk are treated with EMA/CO, and patients refractory to the first line chemotherapy may be successfully treated with EP/EMA. Recent epidemiological data showed that women with complete hydatidiform moles could anticipate normal reproduction in the future. Studies found that pregnancies after treatment of molar pregnancy resulted in 69% full-term, live births; 8% premature deliveries; 1% ectopic pregnancies, and 0.5% stillbirths. First-trimester spontaneous abortions occurred in 17% of pregnancies, and major and minor malformations were detected in 0.4% of infants. Patients with hydatidiform mole were at increased risk of developing molar pregnancy in subsequent conceptions. After having one molar pregnancy, the risk of having molar disease in a future gestation was about 1%. The risk of persistent gestational trophoblastic tumors was increased by long-term oral contraceptive use before conception. In a large, multicenter, case-control study, the risk was shown to be increased in women who had ever used oral contraceptives, but was highest for women taking oral contraceptives during the cycle in which they became pregnant. Partial hydatidiform moles were never previously proven to transform into choriocarcinoma; however, a recent study with molecular techniques clearly showed that partial moles could transform into choriocarcinoma. All patients with suspected partial moles should be reviewed centrally and require hCG follow-up."}, {"id": "wiki20220301en036_29141", "title": "Gestational trophoblastic disease", "score": 0.01803107139982291, "content": "In the past, it was seen as important not to get pregnant straight away after a GTD. Specialists recommended a waiting period of 6 months after the hCG levels become normal. Recently, this standpoint has been questioned. New medical data suggest that a significantly shorter waiting period after the hCG levels become normal is reasonable for approximately 97% of the patients with hydatidiform mole. Risk of a repeat GTD The risk of a repeat GTD is approximately 1 in 100, compared with approximately 1 in 1000 risk in the general population. Especially women whose hCG levels remain significantly elevated are at risk of developing a repeat GTD. Persistent trophoblastic disease The term «persistent trophoblastic disease» (PTD) is used when after treatment of a molar pregnancy, some molar tissue is left behind and again starts growing into a tumour. Although PTD can spread within the body like a malignant cancer, the overall cure rate is nearly 100%."}, {"id": "pubmed23n0482_23918", "title": "Placental site trophoblastic tumor arising from antecedent molar pregnancy.", "score": 0.017565485362095533, "content": "Placental site trophoblastic tumor (PSTT) is a rare form of gestational trophoblastic disease. Little is known about its pathogenesis and natural history. This report describes two cases that arose in patients with documented complete hydatidiform moles and summarizes the antecedent prenatal histories of PSTTs based on a detailed Medline literature analysis. A 28-year-old, G(2)P(2) female had a live, 12-week gestation fetus and a coexisting molar pregnancy. Her hCG levels dropped promptly from 1.5 million to 23,273 IU/ml after termination, but rose shortly thereafter together with the onset of recurrent vaginal bleeding. Curettage revealed persistent mole. Persistently elevated hCG led to hysterectomy disclosing a fundal PSTT. The second case was that of a 48-year-old, G(2) woman who presented with symptoms of preeclampsia, hyperthyroidism, and elevated hCG. Curettage yielded a complete hydatidiform mole. Although the hCG level decreased for a short period, it soon increased despite treatment with methotrexate. A second curettage revealed a PSTT. A Medline literature analysis of PSTT, which consists almost entirely of individual cases and several small series, disclosed that PSTT is preceded in 61% of cases by normal term pregnancy, 12% molar pregnancy, 9% spontaneous abortion, 8% therapeutic abortion, and 3% with ectopic pregnancy, stillbirths or preterm delivery. No information is known in 7%. This report describes two additional cases of PSTT preceded by complete molar pregnancy. PSTT is a well recognized, but uncommon form of gestational trophoblastic disease. Although little is known about its pathogenesis, it is preceded not uncommonly by an abnormal pregnancy, including a molar pregnancy."}, {"id": "pubmed23n0080_18466", "title": "Occurrence of molar pregnancy in patients undergoing elective abortion: comparison with other clinical presentations.", "score": 0.017246506095747842, "content": "Clinical data of molar pregnancies found in women undergoing elective abortion (group 1, n = 39) were compared to those of molar pregnancies in women who experienced spontaneous abortions (group 2, n = 157) and women in whom molar pregnancy was discovered before symptoms of spontaneous abortion were evident (group 3, n = 209). Group 1 women were younger and experienced uterine evacuation at an earlier stage of amenorrhea than groups 2 and 3. Group 3 had larger uteri at evacuation and longer intervals of positive tests for the beta-subunit of human chorionic gonadotropin during the postmolar phase as compared with groups 1 and 2. On the basis of available provincial data for the number of elective abortions, the estimated incidence of molar pregnancies in this population was 1:2,699. The presence of malignant gestational trophoblastic neoplasia was documented in a single patient in group 1. The incidence of malignant gestational trophoblastic neoplasia in this group was not significantly different from that in groups 2 and 3. Routine pathologic examination of the products of conception in women undergoing elective abortion coupled with routine assays of the beta-subunit of human chorionic gonadotropin when molar pregnancy is found can identify both noninvasive and invasive trophoblastic disease in these women."}, {"id": "pubmed23n0501_5170", "title": "Low risk of relapse after achieving undetectable HCG levels in women with complete molar pregnancy.", "score": 0.016817410966647822, "content": "Complete hydatidiform molar pregnancies occur in approximately 1 of 1,000 conceptions. After uterine evacuation of the trophoblastic tissue, women are followed up with serial serum human chorionic gonadotropin (hCG) measurements. Patients are considered to have attained remission when their hCG level spontaneously declines to an undetectable level and remains there during a 6-month follow-up period. This standard effectively detects all disease recurrence; however, it is resource intensive, delays child bearing, and is subject to significant noncompliance. Our objective was to determine the risk of disease recurrence after hCG spontaneously declines to undetectable levels. We used a database from the New England Trophoblastic Disease Center to analyze hCG levels in patients with complete molar pregnancies. Among 1,029 women with complete molar pregnancy and complete data, 15% developed persistent gestational trophoblastic neoplasia. The rate of persistent neoplasm among those whose hCG level fell spontaneously to undetectable levels was 0.2% (2/876, 95% confidence interval 0-0.8%). No women developed persistent gestational trophoblastic neoplasia after their hCG level fell to undetectable levels using an assay with a sensitivity of 5 mIU/mL (n = 82, 95% confidence interval 0-4.5%). Based on our experience with women with complete hydatidiform molar pregnancies whose hCG values spontaneously fell to undetectable levels after molar evacuation, we conclude that the risk of recurrent neoplasm after hCG levels fall to less than 5 mIU/mL approaches zero."}, {"id": "pubmed23n0208_14120", "title": "[Studies on the viability of trophoblast after termination of various kinds of pregnancies (author's transl)].", "score": 0.016612399494983054, "content": "Although normal value of hCG (LH level) does not necessarily indicate eradication of viable trophoblast, its confirmation has been demonstrated as a clinically useful guide for the probable prevention of choriocarcinoma after hydatidiform mole by Takeuchi et al. Choriocarcinoma preceded by other pregnancies than hydatidiform mole which has the highest risk for choriocarcinoma has drawn more attention than before in connection with the decrease of postmolar choriocarcinoma. So that I have studied the regression rate of urinary gonadotropin (hCG) after the termination of various kinds of pregnancies. In 2,433 cases of induced abortion, 695 cases of spontaneous abortion, 1,724 cases of term delivery and 43 cases of hydatidiform mole, their urinary hCG were determined to the level of physiological range of LH. The rate of hCG regression was in the order of term delivery, spontaneous abortion, induced abortion and hydatidiform mole. The younger was the gestational age of trophoblast, the slower was the regression of hCG. At one month after the termination of pregnancy, 80.1%, 11%, 0.3%, 8% and 4.1%, and at two month 55.8%, 1.6%, 0.5%, 4% and 0.5% for hydatidiform mole, induced abortion of less than 12 week of gestation, spontaneous abortion of less than 12 week of gestation, spontaneous abortion of between 13 and 20 week of gestation respectively still showed abnormal hCG value. One percent of induced abortion at 5 month, 4% of spontaneous abortion at 3 month, 0.3% of term delivery at 4 month still maintained abnormal titer. No malignant sequelae in patients under the investigation have ever been observed in the follow up period between 3 and 8 years."}, {"id": "pubmed23n0256_1552", "title": "Persistence of gestational trophoblastic disease for longer than 1 year following evacuation of hydatidiform mole.", "score": 0.01652616084571123, "content": "A spontaneous fall in the radioimmunoassay for the beta subunit of hCG to less than 2 mIU/mL documents regression of hydatidiform mole following evacuation of a molar pregnancy. Continued negative hCG levels for the year after evacuation indicates the absence of risk for persistent gestational trophoblastic disease. This report describes an unusual case of recurrent nonmetastatic gestational trophoblastic disease 16 months after initial evacuation. A 29-year-old woman presented at 19 weeks' gestation with severe preeclampsia and vaginal bleeding. Pelvic ultrasonography demonstrated a molar pregnancy. Pathology following uterine evacuation confirmed a hydatidiform mole. Serial hCG levels fell progressively to less than 2 mIU/mL over the following 25 weeks. She remained compliant with oral contraceptive pills despite having no sexual activity. Sixteen months after uterine evacuation, recurrence of gestational trophoblastic disease was documented by a rising beta-hCG, negative pelvic ultrasound, normal liver function tests, and normal computed tomography of the head. Endometrial curettage showed no chorionic villi or molar tissue. She was treated with five courses of actinomycin D and has remained disease-free for the following 5 years. This late recurrence of gestational trophoblastic disease suggests that those with a molar pregnancy may benefit from surveillance beyond 1 year after uterine evacuation."}, {"id": "article-22233_9", "title": "Gestational Trophoblastic Disease -- Epidemiology", "score": 0.015507603534291037, "content": "Risk factors for molar pregnancy include extremes of age, ethnicity, and a prior history of a HM, suggesting a genetic etiology. The risk of a complete mole is higher for women older than 35 years and younger than 21 years and 7.5 times higher for women older than 40 years. The risk of repeat molar pregnancy in women with a history of molar pregnancy is approximately 1% which is 10 to 20 times the risk in the general population. [1] Interestingly, a history of prior spontaneous abortion has been reported to confer a 2- to 3-fold increased risk of molar pregnancy compared to women without a history of spontaneous abortion. [3] Following 2 molar gestations, the risk of having a third mole is > 10%. [7] It is also important to note that malignant transformation such as choriocarcinoma or placental-site trophoblastic tumor can occur following any pregnancy."}, {"id": "pubmed23n0525_17277", "title": "Postevacuation hCG levels and risk of gestational trophoblastic neoplasia in women with complete molar pregnancy.", "score": 0.015491452991452992, "content": "Women diagnosed with complete hydatidiform molar pregnancy are at 15% to 28% risk of developing persistent gestational trophoblastic neoplasia (GTN) requiring further management with chemotherapy. Our objective was to develop human chorionic gonadotropin (hCG) criteria that establish a patient's risk of developing persistent GTN or achieving remission from their baseline risk within a few weeks of molar evacuation. We used a database from the New England Trophoblastic Disease Center to analyze hCG levels from 1,029 women with complete molar pregnancies. We conducted a retrospective cohort study using data from 1973 to 2001. Women whose hCG level declined below 50 mIU/mL during their follow-up were found to be at no more than 1.1% risk for developing persistent GTN, irrespective of when this level was reached. Women whose hCG levels was below 200 mIU/mL in the fourth week after evacuation (59.8% of all women), or below 100 mIU/mL in the sixth week after evacuation (65.8% of all women), had a risk of persistence below 9%. hCG levels above 2,000 mIU/mL in the fourth week after evacuation (13.3% of women) were associated with a 63.8% risk of developing persistent disease. These data may allow clinicians to evaluate the risk of persistence that their patients with complete molar pregnancy have based on early hCG results after molar evacuation. In the fourth week after molar evacuation, 59.8% of women may be counseled that their risk of developing persistent GTN is substantially reduced from their baseline, whereas 13.3% of women may be warned that their risk of developing persistent GTN is greater than 50%. II-2."}, {"id": "pubmed23n0105_13561", "title": "Increased frequency of complete hydatidiform mole in women with repeated abortion.", "score": 0.014958560743885182, "content": "The association between spontaneous abortion and gestational trophoblastic disease (GTD) has been investigated in a study based on 93 women with 2 consecutive (repeated) spontaneous abortions and 82 control subjects who delivered normal babies. Nine molar pregnancies were observed among 7 of the 93 cases of repeated abortion while no control reported previous GTD. This difference was statistically significant and was not explained by allowance for age and number of pregnancies between cases and controls (chi 2(1) = 4.20; P = 0.04). When the observed number (9) of hydatidiform mole in the 385 pregnancies of the women with repeated abortion was compared with the expected one (0.28) based on the regional frequency data, the estimated relative risk was 32.1 with a 95% confidence interval from 13.9 to 63.3. The present findings confirm the association between GTD and spontaneous abortion and indicate that the risk is larger in women with repeated abortions."}, {"id": "pubmed23n0798_17361", "title": "[Reservation of fertility for seventeen patients with placental site trophoblastic tumor].", "score": 0.014711409395973155, "content": "To approach the efficiency and feasibility of preserving the fertility for patients with placental site trophoblastic tumor (PSTT). Totally 2 086 cases of gestational trophoblastic neoplasm (GTN) patients registered in Peking Union Medical College Hospital between 1998 and 2013. Fifty-seven of them were PSTT patients, 40 cases of which suffered hysterectomy, the rest 17 PSTT patients who preserved their fertility were analyzed retrospectively. The computerized database of clinical and pathological reports was reviewed in this cohort. The clinical manifestation of PSTT was not specific compared to other types of GTN. The average age of the 17 patients was 29.5 years old (range 22-39 years). The most common antecedent pregnancy was term birth (8 cases), the others were spontaneous abortion in 4 case, artificial abortion in 3 cases and molar pregnancy in 2 cases. The baseline serum β-hCG was slightly elevated and 12 patients (12/15) were less than 1 000 U/L. In this cohort, nine of the patients were in stage I, while the other eight cases were in stage III . The patients suffered conservative surgery, including dilation and curettage of uterus in 7 cases, open abdomen uterine lesion excision in 4 cases, laparoscopic uterine lesion excision in 3 cases, hysteroscopic uterine lesion excision in 1 case, and pulmonary lobectomy in 2 cases. Two of the patients didn't received chemotherapy, while the other 15 cases suffered combination chemotherapy. Compared with 40 patients who suffered hysterectomy during the same interval, fertility preservation group did not result in poor outcomes or high risk of relapse rate. Six subsequent pregnancies happened after the therapy, two of them were during their second-trimester, while four patients had healthy babies by vaginal delivery in two and cesarean section in two. The scar of the uterus was fairly well during the cesarean sections. Reservation of fertility therapy could be considered in highly-selected patients for young women who strongly desired to preserve their fertility and with localized lesion. Exactitude follow-up after therapy should be recommended. Contraception should also be recommended for at least one year after the chemotherapy. Vaginal delivery could be an option for the future pregnancies."}, {"id": "wiki20220301en036_29137", "title": "Gestational trophoblastic disease", "score": 0.014400073610599928, "content": "The use of a reliable contraception method is very important during the entire follow up period, as patients are strongly advised against pregnancy at that time. If a reliable contraception method is not used during the follow-up, it could be initially unclear to clinicians as to whether a rising hCG level is caused by the patient becoming pregnant again, or by the continued presence of GTD. In women who have a malignant form of GTD, hCG concentrations stay the same (plateau) or they rise. Persistent elevation of serum hCG levels after a non molar pregnancy (i.e., normal pregnancy [term pregnancy], or preterm pregnancy, or ectopic pregnancy [pregnancy taking place in the wrong place, usually in the fallopian tube], or abortion) always indicate persistent GTD (very frequently due to choriocarcinoma or placental site trophoblastic tumour), but this is not common, because treatment mostly is successful."}, {"id": "pubmed23n0512_9094", "title": "Persistent gestational trophoblastic disease is rarely, if ever, derived from non-molar first-trimester miscarriage.", "score": 0.014345794392523363, "content": "Traditional epidemiologic data suggest that persistent gestational trophoblastic disease (pGTD), may follow, and be derived from, either molar pregnancy, non-molar term pregnancy or first-trimester non-molar miscarriage. We examined a database of cases of possible or probable hydatidiform moles and proven pGTD derived from the Regional Trophoblastic Disease Unit, Charing Cross Hospital, London. There were 424 cases (6%), in whom the initial registered diagnosis was that of PHM or CHM but central histopathological review diagnosed a definite non-molar hydropic abortion (HA). In eight of the 424 (2%), although the histology of the most recent index pregnancy was that of non-molar miscarriage, there was a previous history of pregnancy affected by hydatidiform mole; two of these developed subsequent pGTD. Of a further 86 cases referred for a histopathological opinion prior to registration which demonstrated definite non-molar HA, none developed pGTD (zero of 510 (0%, 95% CI 0-0.7%)). During the same period there were 352 cases with pGTD requiring chemotherapy. In 31 cases, the only known pregnancy was the preceding apparent non-molar HA. However, of these, there were only three cases in whom the preceding histological products of conception had been centrally reviewed and were suggestive of non-molar pregnancy. However, in all three of these cases, the specimens were inadequate for definite exclusion of molar pregnancy. In one case in whom no material was available for review, molecular genetic analysis using restriction fragment length polymorphisms was carried out, and the choriocarcinoma was genetically derived from a previous molar pregnancy rather than the preceding HA. There were therefore no cases identified on the database of the trophoblastic disease unit of pGTD requiring treatment in whom the trophoblastic tumour could be genetically proven to have arisen from the preceding first trimester non-molar HA. We suggest that the risk of pGTD developing from a histologically confirmed non-molar HA is less than 1 in 50,000 and that the majority of pGTD cases previously reported to have been caused by a non-molar miscarriage probably represent disease due to an unrecognised early molar pregnancy."}, {"id": "wiki20220301en036_29140", "title": "Gestational trophoblastic disease", "score": 0.01404869640163758, "content": "In this scoring system, women with a score of 7 or greater are considered at high risk. It is very important for malignant forms of GTD to be discovered in time. In Western countries, women with molar pregnancies are followed carefully; for instance, in the UK, all women who have had a molar pregnancy are registered at the National Trophoblastic Screening Centre. There are efforts in this direction in the developing countries too, and there have been improvements in these countries in the early detection of choriocarcinoma, thereby significantly reducing the mortality rate also in developing countries. Becoming pregnant again Most women with GTD can become pregnant again and can have children again. The risk of a further molar pregnancy is low. More than 98% of women who become pregnant following a molar pregnancy will not have a further hydatidiform mole or be at increased risk of complications."}, {"id": "wiki20220301en036_29144", "title": "Gestational trophoblastic disease", "score": 0.013891248937977909, "content": "However, the incidence of rare diseases (such as GTD) is difficult to measure, because epidemiologic data on rare diseases is limited. Not all cases will be reported, and some cases will not be recognised. In addition, in GTD, this is especially difficult, because one would need to know all gestational events in the total population. Yet, it seems very likely that the estimated number of births that occur at home or outside of a hospital has been inflated in some reports. Terminology Gestational trophoblastic disease (GTD) may also be called gestational trophoblastic tumour (GTT). Hydatidiform mole (one type of GTD) may also be called molar pregnancy. Persistent disease; persistent GTD: If there is any evidence of persistence of GTD, usually defined as persistent elevation of beta hCG (see «Diagnosis» below), the condition may also be referred to as gestational trophoblastic neoplasia (GTN). See also Trophoblastic neoplasms References External links"}, {"id": "pubmed23n0383_12290", "title": "Pregnancy outcomes of patients who conceived within 1 year after chemotherapy for gestational trophoblastic tumor: a clinical report of 22 patients.", "score": 0.013703703703703704, "content": "The aim of this study was to explore the risk of pregnancy of patients who conceived within 1 year after successful chemotherapy for gestational trophoblastic tumor (GTT). From 1966 to 1996, 22 patients who conceived within 1 year after chemotherapy were followed up and analyzed retrospectively. Among 22 patients, 9 had term deliveries and 1 had a premature birth, 6 had induced abortion at the patient's request, and 6 had therapeutic abortion because of various indications such as repeated hydatidiform mole (1 case), intrauterine death (1 case), inevitable abortion (1 case), and threatened abortion (3 cases). The fetal loss rate was 27.1% (6/22). The incidence rate of gestational trophoblastic disease (GTD) was 9.1% (2/22). The incidence rate of GTT was 4.5% (1/22). The average interval between completion of chemotherapy and pregnancy was 10.25 months in the group of term pregnancies and 5.86 months in that of fetal loss (P &lt; 0.05), indicating that the longer the interval, the lesser the risk of GTD. The results suggest that contraception for 1 year is necessary in patients with GTT after successful chemotherapy. However, in the case of a patient who conceives within 1 year, it is not necessary to terminate pregnancy, but the pregnancy must be carefully watched."}, {"id": "wiki20220301en036_29129", "title": "Gestational trophoblastic disease", "score": 0.013676991832331637, "content": "All five closely related tumours develop in the placenta. All five tumours arise from trophoblastic cells. The trophoblast is the membrane that forms the wall of the blastocyst in the early development of the fetus. In a normal pregnancy, trophoblastic cells aid the implantation of the fertilised egg into the uterine wall. But in GTD, they develop into tumour cells. Cause Two main risk factors increase the likelihood for the development of GTD: 1) The woman being under 20 years of age, or over 35 years of age, and 2) previous GTD. Although molar pregnancies affect women of all ages, women under 16 and over 45 years of age have an increased risk of developing a molar pregnancy.Being from Asia/of Asian ethnicity is an important risk factor. Hydatidiform moles are abnormal conceptions with excessive placental development. Conception takes place, but placental tissue grows very fast, rather than supporting the growth of a fetus."}, {"id": "wiki20220301en073_24425", "title": "Trophoblastic neoplasm", "score": 0.013478668541959681, "content": "Management of GTN requires pathology review, treatment options and monitoring of hCG. Therefore, it can be treated with curettage, hysterectomy and single agent or multi agent chemotherapy. Although this group of diseases are highly susceptible to chemotherapy, prognosis depends on the type of GTN and whether the tumor has spread to other areas of the body. Cause and Risk factors The exact cause of gestational trophoblastic neoplasia (GTN) is unknown. GTN often arises after molar pregnancies but can also occur after any gestation including miscarriages and term pregnancies. Although risk factors may impact on the development of the tumor, most do not directly cause of disease. According to the some studies, the risk of complete molar pregnancy is highest in women over age 35 and younger than 20. The risk is even higher for women over age 45. Signs and Symptoms The symptoms of GTN will vary from person to person. People with the same disease may not have all the symptoms listed."}, {"id": "pubmed23n0834_2417", "title": "Postmolar gestational trophoblastic neoplasia: beyond the traditional risk factors.", "score": 0.013439434129089302, "content": "To investigate the slope of linear regression of postevacuation serum hCG as an independent risk factor for postmolar gestational trophoblastic neoplasia (GTN). Multicenter retrospective cohort study. Academic referral health care centers. All subjects with confirmed hydatidiform mole and at least four measurements of β-hCG titer. None. Type and magnitude of the relationship between the slope of linear regression of β-hCG as a new risk factor and GTN using Bayesian logistic regression with penalized log-likelihood estimation. Among the high-risk and low-risk molar pregnancy cases, 11 (18.6%) and 19 cases (13.3%) had GTN, respectively. No significant relationship was found between the components of a high-risk pregnancy and GTN. The β-hCG return slope was higher in the spontaneous cure group. However, the initial level of this hormone in the first measurement was higher in the GTN group compared with in the spontaneous recovery group. The average time for diagnosing GTN in the high-risk molar pregnancy group was 2 weeks less than that of the low-risk molar pregnancy group. In addition to slope of linear regression of β-hCG (odds ratio [OR], 12.74, confidence interval [CI], 5.42-29.2), abortion history (OR, 2.53; 95% CI, 1.27-5.04) and large uterine height for gestational age (OR, 1.26; CI, 1.04-1.54) had the maximum effects on GTN outcome, respectively. The slope of linear regression of β-hCG was introduced as an independent risk factor, which could be used for clinical decision making based on records of β-hCG titer and subsequent prevention program."}, {"id": "pubmed23n1013_18140", "title": "Gestational Trophoblastic Neoplasia After Human Chorionic Gonadotropin Normalization Following Molar Pregnancy: A Systematic Review and Meta-analysis.", "score": 0.013157894736842105, "content": "To estimate the incidence of gestational trophoblastic neoplasia following complete and partial molar pregnancy after reaching normal human chorionic gonadotropin (hCG) levels to guide evidence-based follow-up recommendations. MEDLINE, EMBASE, Web of Science, POPLINE, Cochrane, and ClinicalTrials.gov were searched from inception to November 2018, using the intersection of \"gestational trophoblastic disease,\" \"molar pregnancy,\" and \"human chorionic gonadotropin\" themes. Search results were screened to identify cohort studies of molar pregnancy reporting gestational trophoblastic neoplasia development, with at least 6 months of intended normal hCG follow-up. Two reviewers independently identified articles for inclusion. Data were extracted using a standardized form. For meta-analysis, cumulative incidence of gestational trophoblastic neoplasia, with CIs by the Agresti-Coull method, and pooled risk ratios (RRs) comparing complete and partial mole were calculated. Among the 19 eligible studies that reported adequate data for inclusion in the primary meta-analysis, we found low incidence of gestational trophoblastic neoplasia after normal hCG level following both complete mole (64/18,357, 0.35%, 95% CI 0.27-0.45%), and partial mole (5/14,864, 0.03%, 95% CI 0.01-0.08%). There was a significantly higher risk of gestational trophoblastic neoplasia after complete compared with partial molar pregnancy (RR 4.72, 95% CI 1.81-12.3, P=.002). Among gestational trophoblastic neoplasia cases after normal hCG level following complete mole, 89.6% occurred when the time from evacuation to normalization was 56 days or longer, and 60.7% were diagnosed beyond the commonly recommended 6-month surveillance interval. Sensitivity analyses, including those limiting to studies at low risk of bias, did not significantly affect results. We found an overall incidence of gestational trophoblastic neoplasia of 15.7% for complete mole (1,354/8,611, 95% CI 15.0-16.5%) and 3.95% for partial mole (221/5,593, 95% CI 3.47-4.50%). Gestational trophoblastic neoplasia development after normal hCG level following molar pregnancy is rare. Recommendations for frequency and duration of hCG follow-up can be minimized to lessen burden on patients and informed by the type of molar pregnancy and time interval from uterine evacuation to hCG normalization. PROSPERO, CRD42019116414."}, {"id": "pubmed23n0789_5549", "title": "Gestational trophoblastic neoplasia: A 6 year retrospective study.", "score": 0.012517006802721088, "content": "To study the clinical presentations of gestational trophoblastic neoplasia and its response to chemotherapy. This is a retrospective study of 28 women of gestational trophoblastic neoplasia evaluated over a period of 6 years from January 2004 to December 2009. Patients were evaluated on the basis of their age, number of deliveries, history of abortion or molar pregnancy, and the treatment received. All patients were scored on the basis of WHO scoring system. Patients with low risk (score &lt;/=6) received single agent chemotherapy with methotrexate or actinomycin D. Patients with high risk (score &gt;/=7) received multiple agent chemotherapy with EMACO regimen. After completion of chemotherapy patients were followed for a minimum of 2 years. The response to treatment was evaluated during follow-up by clinical examination, beta hCG levels and imaging as and when required. Out of 28 women only 27 could be evaluated, because 1 patient was lost to follow-up. Out of 27 patients, 18 patients (66.67%) achieved complete remission with the first-line chemotherapy and additional 25.92% (7/27) achieved complete remission with second line chemotherapy resulting in complete remission of 92.5% (25/27). Gestational trophoblastic neoplasia is curable if patient is properly evaluated and scored. It shows good response to chemotherapy."}, {"id": "pubmed23n1086_23728", "title": "Surveillance for gestational trophoblastic neoplasia following molar pregnancy: a cost-effectiveness analysis.", "score": 0.0122992700729927, "content": "Historically, published guidelines for care after molar pregnancy recommended monitoring human chorionic gonadotropin levels for the development of gestational trophoblastic neoplasia until normal and then for 6 months after the first normal human chorionic gonadotropin. However, there are little data underlying such recommendations, and recent evidence has demonstrated that gestational trophoblastic neoplasia diagnosis after human chorionic gonadotropin normalization is rare. We sought to estimate the cost-effectiveness of alternative strategies for surveillance for gestational trophoblastic neoplasia after human chorionic gonadotropin normalization after complete and partial molar pregnancy. A Markov-based cost-effectiveness model, using monthly cycles and terminating after 36 months/cycles, was constructed to compare alternative strategies for asymptomatic human chorionic gonadotropin surveillance after the first normal (none; monthly testing for 1, 3, 6, and 12 months; or every 3-month testing for 3, 6, and 12 months) for both complete and partial molar pregnancy. The risk of reduced surveillance was modeled by increasing the probability of high-risk disease at diagnosis. Probabilities, costs, and utilities were estimated from peer-reviewed literature, with all cost data applicable to the United States and adjusted to 2020 US dollars. The primary outcome was cost per quality-adjusted life year ($/quality-adjusted life year) with a $100,000/quality-adjusted life year willingness-to-pay threshold. Under base-case assumptions, we found no further surveillance after the first normal human chorionic gonadotropin to be the dominant strategy from both the healthcare system and societal perspectives, for both complete and partial molar pregnancy. After complete mole, this strategy had the lowest average cost (healthcare system, $144 vs maximum $283; societal, $152 vs maximum $443) and highest effectiveness (2.711 vs minimum 2.682 quality-adjusted life years). This strategy led to a slightly higher rate of death from gestational trophoblastic neoplasia (0.013% vs minimum 0.009%), although with high costs per gestational trophoblastic neoplasia death avoided (range, $214,000 to &gt;$4 million). Societal perspective costs of lost wages had a greater impact on frequent surveillance costs than rare gestational trophoblastic neoplasia treatment costs, and no further surveillance was more favorable from this perspective in otherwise identical analyses. No further surveillance remained dominant or preferred with incremental cost-effectiveness ratio of &lt;$100,000 in all analyses for partial mole, and most sensitivity analyses for complete mole. Under the assumption of no disutility from surveillance, surveillance strategies were more effective (by quality-adjusted life year) than no further surveillance, and a single human chorionic gonadotropin test at 3 months was found to be cost-effective after complete mole with incremental cost-effectiveness ratio of $53,261 from the healthcare perspective, but not from the societal perspective (incremental cost-effectiveness ratio, $288,783). Largely owing to the rare incidence of gestational trophoblastic neoplasia after human chorionic gonadotropin normalization after molar pregnancy, prolonged surveillance is not cost-effective under most assumptions. It would be reasonable to reduce, and potentially eliminate, current recommendations for surveillance after human chorionic gonadotropin normalization after molar pregnancy, particularly among partial moles. With any reduction in surveillance, patients should be counseled on symptoms of gestational trophoblastic neoplasia and established in routine gynecologic care."}, {"id": "wiki20220301en303_13536", "title": "Placental site trophoblastic tumor", "score": 0.012066365007541479, "content": "Placental site trophoblastic tumor is a form of gestational trophoblastic disease, which is thought to arise from intermediate trophoblast. It may secrete human placental lactogen (human chorionic somatomammotropin), and result in a false-positive pregnancy test. Placental site trophoblastic tumor is a monophasic neoplasm of the implantation site intermediate trophoblast, and usually a benign lesion, which comprises less than 2% of all gestational trophoblastic proliferations. Preceding conditions include molar pregnancy (5%). Compared to choriocarcinoma or invasive mole, hemorrhage is less conspicuous and serum β-HCG level is low, making early diagnosis difficult. Immunohistochemistry: Often stains with hPL, keratin, Mel-CAM, EGFR. Treatment Because chemotherapy is ineffective; the patient should undergo hysterectomy. Prognosis 10–20% of cases metastasize leading to death. References External links Health issues in pregnancy Germ cell neoplasia"}, {"id": "wiki20220301en032_59230", "title": "Molar pregnancy", "score": 0.011581091322470632, "content": "Etymology The etymology is derived from hydatisia (Greek \"a drop of water\"), referring to the watery contents of the cysts, and mole (from Latin mola = millstone/false conception). The term, however, comes from the similar appearance of the cyst to a hydatid cyst in an Echinococcosis. See also Gestational trophoblastic disease References External links Humpath #3186 (Pathology images) Clinically reviewed molar pregnancy and choriocarcinoma information for patients from Cancer Research UK MyMolarPregnancy.com: Resource for those diagnosed with molar pregnancy. Links, personal stories, and support groups. Germ cell neoplasia Pregnancy with abortive outcome"}, {"id": "pubmed23n0541_9218", "title": "Guidelines following hydatidiform mole: a reappraisal.", "score": 0.011407919547454431, "content": "The aim of this study was to determine how often patients with complete hydatidiform mole (CHM) who spontaneously achieve normal human chorionic gonadotrophin (hCG) levels subsequently develop persistent or recurrent gestational trophoblast disease. Four hundred and fourteen cases of CHM registered at the Hydatidiform Mole Registry of Victoria were reviewed retrospectively after molar evacuation. Maternal age, gestational age, gravidity and parity were determined for each patient, as well as the need for chemotherapy. Among the 414 patients, 55 (13.3%) required chemotherapy for persistent trophoblastic disease. None of the patients whose hCG levels spontaneously fell to normal subsequently developed persistent molar disease. Weekly hCG measurements are recommended for all patients until normal levels are achieved. For patients who attain normal hCG levels within 2 months after evacuation, it seems safe to discontinue monitoring once normal levels are achieved. Patients who fail to achieve normal hCG levels by 2 months after evacuation should be monitored with monthly hCG measurements for 1 year after normalisation to assure sustained remission."}, {"id": "wiki20220301en000_4713", "title": "Abortion", "score": 0.011198945981554678, "content": "Complications after second-trimester abortion are similar to those after first-trimester abortion, and depend somewhat on the method chosen. The risk of death from abortion approaches roughly half the risk of death from childbirth the farther along a woman is in pregnancy; from one in a million before 9 weeks gestation to nearly one in ten thousand at 21 weeks or more (as measured from the last menstrual period). It appears that having had a prior surgical uterine evacuation (whether because of induced abortion or treatment of miscarriage) correlates with a small increase in the risk of preterm birth in future pregnancies. The studies supporting this did not control for factors not related to abortion or miscarriage, and hence the causes of this correlation have not been determined, although multiple possibilities have been suggested."}, {"id": "pubmed23n0557_20206", "title": "Postevacuation hCG levels and risk of gestational trophoblastic neoplasia among women with partial molar pregnancies.", "score": 0.0110551229195297, "content": "To develop human chorionic gonadotropin (hCG) criteria that determine a patient's risk of developing persistent gestational trophoblastic neoplasia (GTN) or achieving remission after partial mole evacuation. We used a database from the New England Trophoblastic Disease Center to analyze hCG levels from 284 women with partial molar pregnancies diagnosed between 1973 and 2003. An hCG level &gt;199 mIU/mL in the third through eighth week following molar evacuation was associated with at least a 35% risk of GTN. Women with partial mole who have elevated hCG levels within the first few weeks after molar evacuation are at increased risk for developing GTN."}, {"id": "pubmed23n0511_9522", "title": "Gestational trophoblastic disease: is intensive follow up essential in all women?", "score": 0.011027208438211674, "content": "To determine the timescale of the registration process for gestational trophoblastic disease and its impact on hCG level at registration and subsequent need for chemotherapy. A prospective observational study using a standardised protocol for registration, assessment and treatment for molar pregnancy. A supra-regional tertiary referral centre for gestational trophoblastic disease. A total of 2046 consecutive women registered between January 1994 and December 1998 with a diagnosis of molar pregnancy. Data at and after registration, collected prospectively on a computerised database, were statistically analysed (by multiple logistic regression and ANOVA). Relationship between length of time to and hCG value at registration; also the subsequent need for chemotherapy. A total of 2046 women with a diagnosis of molar pregnancy were registered in the study period. The mean time interval between first evacuation and registration at the referral centre was 47 days (median 37, range 0-594). One hundred and five out of 2046 (5.1%) women needed chemotherapy. Sixty-three precent of the women (1296 out of 2046) had a normal level of urinary hCG (less than 40 IU/24 hours) at the time of registration and only one (0.08%) needed chemotherapy. Binary logistic regression analysis showed a statistically significant relationship between time to registration, hCG value, histology, pretreatment risk score and decision to administer chemotherapy. Women with gestational trophoblastic disease who were registered late were significantly more likely to have normal levels of hCG and were less likely to need chemotherapy. A less intensive follow up may be justified in women with gestational trophoblastic disease who are registered with a normal hCG level."}, {"id": "pubmed23n0541_9219", "title": "Persistent trophoblast disease following partial molar pregnancy.", "score": 0.010764598954783003, "content": "Human chorionic gonadotrophin (hCG) follow-up data were analysed retrospectively in all patients registered in the Hydatidiform Mole Registry at the Royal Women's Hospital, Melbourne from January 1992 to January 2001 to determine the risk of persistent trophoblast disease following partial molar pregnancy and to review the present follow-up protocol of patients suffering from partial hydatidiform molar pregnancy (PHM). Demographic factors were determined for all 344 cases with a review diagnosis of PHM, included age, history of previous hydatidiform mole, gestation length, hCG levels and compliance with follow-up. Six of the 344 patients diagnosed with PHM required treatment with single-agent methotrexate and folinic acid rescue. All six patients achieved and maintained a complete biochemical remission after chemotherapy. hCG regression assays were analysed for 235 patients: 225 patients had at least one normal hCG measurement during follow-up, of whom 152 (64.7%) patients obtained normal values within 2 months after evacuation. All patients obtained normal levels within 32 weeks after evacuation of the partial hydatidiform mole. Only 63 (25.6%) patients completed the recommended follow-up program. No patient who achieved normal hCG levels required chemotherapy because of a recurrent gestational trophoblastic tumour. This study indicates that 1.7% of all partial mole pregnancy patients needed treatment for malignant sequelae. In contrast, no patient diagnosed with partial mole had a biochemical or clinical relapse after achieving normal levels of hCG, consistent with previous studies. Patients who have had a partial hydatidiform mole should be followed by hCG assays until normal levels are achieved and then follow-up can be safely discontinued."}, {"id": "InternalMed_Harrison_7903", "title": "InternalMed_Harrison", "score": 0.010715249662618085, "content": "(See Chap. 117) Gestational trophoblastic disease encompasses hydatidiform mole, choriocarcinoma, placental site trophoblastic tumor, and assorted miscellaneous and unclassifiable trophoblastic tumors. Moles are the most common, occurring in 1 in 1500 pregnancies in the United States. The incidence is higher in Asia. In general, if the serum level of β-human chorionic gonadotropin (β-hCG) returns to normal after surgical removal (evacuation) of the mole, the illness is considered gestational trophoblastic disease. By contrast, if the β-hCG level remains elevated after mole evacuation, the patient is considered to have gestational trophoblastic neoplasia. Choriocarcinoma occurs in 1 in 25,000 pregnancies. Maternal age >45 years and prior history of molar pregnancy are risk factors. A previous molar pregnancy makes choriocarcinoma about 1000 times more likely to occur (incidence 1–2%)."}, {"id": "Obstentrics_Williams_2689", "title": "Obstentrics_Williams", "score": 0.010627243529189496, "content": "Cormano ], Mackay G, Holschneider C: Gestational trophoblastic disease diagnosis delayed by the hook efect. Obstet GynecoIo126(4):811, 2015 Dantas PRS, Maesd. I, Filho ]R, et al: Does hormonal contraception during molar pregnancy follow-up influence the risk and clinical aggressiveness of gestational trophoblastic neoplasia after controlling for risk factors? Gynecol Oncol September 16, 2017 [Epub ahead of print] Davis MR, Howitt BE, Quade B], et al: Epithelioid trophoblastic tumor: a single institution case series at the New England Trophoblastic Disease Center. Gynecol Oncol 137(3):456,o20o15 Delmis ], Pfeifer 0, Ivanisecvic M, et al: Sudden death from trophoblastic embolism in pregnancy. Euro] Obstet Gynecol Reprod Bioi 92:225, 2000 de Medeiros SF, Norman RJ: Human chorionic gonadotrophin protein core and sugar branches heterogeneity: basic and clinical insights. Hum Reprod Update 15(1):69,o2009"}, {"id": "wiki20220301en036_29133", "title": "Gestational trophoblastic disease", "score": 0.009998443821973233, "content": "Differential diagnosis These are not GTD, and they are not tumours Exaggerated placental site Placental site nodule Both are composed of intermediate trophoblast, but their morphological features and clinical presentation can differ significantly. Exaggerated placental site is a benign, non cancerous lesion with an increased number of implantation site intermediate trophoblastic cells that infiltrate the endometrium and the underlying myometrium. An exaggerated placental site may occur with normal pregnancy, or after an abortion. No specific treatment or follow up is necessary. Placental site nodules are lesions of chorionic type intermediate trophoblast, usually small. 40 to 50% of placental site nodules are found in the cervix. They almost always are incidental findings after a surgical procedure. No specific treatment or follow up is necessary. Treatment Treatment is always necessary."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "#ERROR!", "full_answer_no_ref": "#ERROR!", "full_question": "A 27-year-old woman, 10 weeks pregnant, with persistent severe allergic asthma. She is currently adequately controlled with daily inhaled budesonide and rescue inhaled salbutamol on demand. She comes to your office concerned about the possible teratogenic effects of her anti-asthma medication. Which of the following would be the correct approach?", "id": 210, "lang": "en", "options": {"1": "Given that asthma improves during pregnancy in most patients, the best thing for the patient and the fetus is to suspend anti-asthma treatment.", "2": "Discontinue budesonide because it has been associated with an increased risk of fetal malformations and replace it with an oral anti-leukotriene (montelukast).", "3": "Withdraw current treatment and replace it with oral prednisone at the lowest possible dose.", "4": "Maintain the current treatment and reassure the patient about its side effects and the need for adequate asthma control during pregnancy.", "5": "Replace budesonide with an anti-IgE monoclonal antibody (omalizumab) because of its greater safety in pregnancy since it is not a drug."}, "question_id_specific": 187, "type": "GYNECOLOGY AND OBSTETRICS", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0534_4859", "title": "Managing asthma in expectant mothers.", "score": 0.018042071197411004, "content": "Pregnancy does not appear to have a consistent effect on the frequency or severity of asthma. The most common cause of worsening asthma in pregnancy is likely to be noncompliance with medication. Emphasizing to the patient in advance that fetal well-being is dependent on maternal well-being may help prevent this.In general, well controlled asthma is not associated with a higher risk of adverse pregnancy outcomes. Essential to successful asthma management is patient education that helps to ensure effective medication use, avoidance of triggers, and prompt treatment. This education should include measurement of peak expiratory flow rate and a written asthma action plan. Most of the medications that are used to control asthma in the general population can be safely used in pregnant women. Inhaled beta-adrenoceptor agonists (beta-agonists), cromolyn sodium (sodium cromoglycate), and inhaled and systemic corticosteroids all appear to be very well tolerated by the fetus. Budesonide and beclomethasone should be considered as the preferred inhaled corticosteroids for the treatment of asthma in pregnancy. Use of the leukotriene receptor antagonists zafirlukast and montelukast in pregnancy is probably safe but should be limited to special circumstances, where they are viewed essential for asthma control. Zileuton should not be used in pregnancy.Acute asthma exacerbations in pregnant women should be treated in a similar manner to that in non-pregnant patients. Maternal blood glucose levels should be monitored periodically in pregnant women receiving systemic corticosteroids because of the deleterious effects of hyperglycemia upon embryos and fetuses. During pregnancy, maternal arterial oxygen saturations should be kept above 95% if possible for fetal well-being. Ambulatory oxygenation should be checked prior to discharge to ensure that women do not desaturate with their daily activities.Acute exacerbations of asthma during labor and delivery are rare. Dinoprost, ergometrine, and other ergot derivatives can cause severe bronchospasm, especially when used in combination with general anesthesia, and should be avoided in asthmatic patients. Pregnant women who have been treated with corticosteroids in the past year may require stress-dose corticosteroids during labor and delivery. Most asthma medications, including oral prednisone, are considered compatible with breast-feeding."}, {"id": "pubmed23n0533_22662", "title": "Outcome of pregnancy in a randomized controlled study of patients with asthma exposed to budesonide.", "score": 0.01551083962151156, "content": "Budesonide is the only inhaled corticosteroid to be given a category B pregnancy rating by the US Food and Drug Administration, based on observational data from the Swedish Medical Birth Registry. However, data from large randomized controlled trials are lacking. To compare pregnancy outcomes among patients with recent-onset mild-to-moderate persistent asthma receiving low-dose budesonide vs placebo. In a randomized, double-blind, placebo-controlled trial, 7241 patients aged 5 to 66 years with mild-to-moderate persistent asthma for less than 2 years and no previous regular corticosteroid therapy received once-daily budesonide or placebo via dry powder inhaler in addition to their usual asthma medication for 3 years. This trial was followed by a 2-year open-label treatment period. The daily dose of budesonide was 400 microg for adults. The study included 2473 females aged 15 to 50 years at randomization. Pregnancy was not an exclusion criterion (except for U.S. patients). Of 319 pregnancies reported, 313 were analyzed. Healthy children were delivered in 81% and 77% of all pregnancies in the budesonide and placebo groups, respectively. Of the 196 pregnancies reported by participants taking budesonide, 38 (19%) had adverse outcomes: 23 (12%) had miscarriages, 3 (2%) had congenital malformations, and 12 (6%) had other outcomes. Of the 117 pregnancies reported in the placebo group, 27 (23%) had adverse outcomes: 11 (9%) had miscarriages, 4 (3%) had congenital malformations, and 12 (10%) had other outcomes. Treatment with low-dose inhaled budesonide in females with mild-to-moderate persistent asthma does not seem to affect the outcome of pregnancy."}, {"id": "pubmed23n0731_13630", "title": "Chapter 16: Asthma in pregnancy.", "score": 0.014391077531930203, "content": "The course of asthma during pregnancy may be affected by maternal physiological changes and triggers of asthma such as viral infections, exposure to allergens, and nonadherence with therapy. If asthma is uncontrolled, there are recognized harmful effects not only to the mother but also  to the fetus. However, with effective asthma control, most women have outcomes, at or near that of the general population. Many medications are considered appropriate for use in pregnancy including inhaled corticosteroids (ICSs) such as budesonide, beclomethasone dipropionate, and fluticasone  and the leukotriene receptor antagonists montelukast and zafirlukast. When ICSs or ICS/long-acting beta(2)-adrenergic agonist combinations are not effective during exacerbations of asthma, short courses of oral corticosteroids should be administered earlier rather than later. Spirometry  and flow volume loop tracings are useful measures of pulmonary function for gravidas. Results may be compared with nonpregnant reference values. Vocal cord dysfunction may be suspected when the inspiratory loop is truncated. The gravida does not reject the fetus because of lack of vascular  continuity, a trophoblast layer causing separation, and suppressive mechanisms at the placental interface. The secretion of IL-10 increases in pregnancy and is lower in women with recurrent spontaneous abortions. Only immunoglobulin G (IgG) subclasses are transported across the placenta, especially  IgG1, IgG3, and IgG4. Fetal B cells can produce endogenous IgE by 20 weeks of gestation."}, {"id": "pubmed23n0517_11728", "title": "The management of asthma and rhinitis during pregnancy.", "score": 0.014097560975609756, "content": "Asthma and rhinitis frequently complicate pregnancy. The course of asthma may be adversely altered by gestation, placing the mother and fetus at risk. Therefore, pregnant patients with persistent asthma require an aggressive asthma management plan that includes environmental control measures and the use of long-term controller medications. Inhaled corticosteroids (ICSs) are the preferred long-term controller medication for persistent asthma, based on efficacy. However, safety concerns regarding corticosteroids may cause physicians or patients to seek an alternate, less effective treatment during pregnancy. The Food and Drug Administration's pregnancy category ratings are based on animal and human safety data. Because ICSs were previously rated pregnancy category C (i.e., with human studies lacking and animal studies either lacking or positive for fetal risk), other asthma controllers, such as cromolyn and nedocromil, that carry a pregnancy category B rating (i.e., showing no evidence of fetal risk in humans or animal studies negative for fetal risk) appeared to be more desirable for use during pregnancy. One ICS, budesonide, was reclassified as pregnancy category B based on human data supporting its use during pregnancy. In moderate and severe persistent asthma, add-on therapy may be considered, including long-acting beta2-adrenergic agonists, leukotriene receptor antagonists, and theophylline. Because rhinitis may adversely affect quality of life and the course of asthma, recommendations for aggressive management also apply."}, {"id": "pubmed23n0510_2733", "title": "[Asthma and pregnancy -- efficacy and safety of medication during pregnancy].", "score": 0.013977762650329021, "content": "Bronchial asthma is one of the most common medical problems in pregnancy, with prevalance around 4-7 %. Asthma clinical course is variable, with worsening of symptoms in one third of the cases. The most critical period occurs between the 24th and 36th week of gestation. Symptoms usually regress completely after delivery during the following three months and they are expected to recur, with the same pattern, in subsequent pregnancies. Asthma control during pregnancy depends on pregnant patient education and safe medication, preferably inhaled, that prevents crises. In chronic asthma, usually prescribed drugs (inhaled corticosteroids: beclometasone and budesonide) are effective and safe to the fetus. There are several drugs to be added to inhaled steroids -- beta agonists bronchodilators: short and long acting -- according to the severity of bronchial constriction and frequency of crises. Treatment of acute asthma should follow recommended guidelines, since uncontrolled asthma increases more the risk of pregnancy complications (low birth weight and premature delivery) than the eventual medication risks to pregnancy."}, {"id": "InternalMed_Harrison_20181", "title": "InternalMed_Harrison", "score": 0.013106973347937203, "content": "Pregnancy Approximately one-third of asthmatic patients who are pregnant improve during the course of a pregnancy, one-third deteriorate, and one-third are unchanged. It is important to maintain good control of asthma because poor control may have adverse effects on fetal development. Compliance may be a problem because there is often concern about the effects of antiasthma medications on fetal development. The drugs that have been used for many years in asthma therapy have now been shown to be safe and without teratogenic potential. These drugs include SABA, ICS, and theophylline; there is less safety information about newer classes of drugs such as LABA, antileukotrienes, and anti-IgE. If an OCS is needed, it is better to use prednisone rather than prednisolone because it cannot be converted to the active prednisolone by the fetal liver, thus protecting the fetus from systemic effects of the corticosteroid. There is no contraindication to breast-feeding when patients are using these"}, {"id": "pubmed23n0886_834", "title": "Novel targets of omalizumab in asthma.", "score": 0.012788226961517274, "content": "Omalizumab is a recombinant humanized anti-IgE monoclonal antibody approved in the US for moderate to severe persistent allergic asthma (severe persistent asthma in the European Union), uncontrolled despite treatment with inhaled corticosteroids and long-acting beta2 agonists. It reduces asthma exacerbations, symptoms, oral corticosteroid doses, and improves quality of life. Omalizumab may have an antiviral effect when used as a preventive therapy for fall exacerbations in children and teenagers. Two proof-of-concept studies have evaluated omalizumab in nonatopic asthma and showed that it is safe and possibly efficacious in some patients. Omalizumab has been successfully studied as add-on to specific immunotherapy in moderate allergic asthma. Its safety in pregnancy has been assessed in the EXPECT registry. Case series also report positive effects in cases of allergic bronchopulmonary aspergillosis, and in nasal disorders frequently associated with asthma. Last, omalizumab may have corticosteroid-sparing effect in a subset of patients with eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). Recent studies argue in favor of positive effects of omalizumab beyond its current indications in asthma. Well-designed studies are needed in order to demonstrate the safety and efficacy of omalizumab in these possible novel indications."}, {"id": "wiki20220301en002_60424", "title": "Asthma", "score": 0.012516588249487271, "content": "Leukotriene receptor antagonists (anti-leukotriene agents such as montelukast and zafirlukast) may be used in addition to inhaled corticosteroids, typically also in conjunction with a LABA. For adults or adolescents who have persistent asthma that is not controlled very well, the addition of anti-leukotriene agents along with daily inhaled corticosteriods improves lung function and reduces the risk of moderate and severe asthma exacerbations. Anti-leukotriene agents may be effective alone for adolescents and adults, however there is no clear research suggesting which people with asthma would benefit from anti-leukotriene receptor alone. In those under five years of age, anti-leukotriene agents were the preferred add-on therapy after inhaled corticosteroids. A 2013 Cochrane systematic review concluded that anti-leukotriene agents appear to be of little benefit when added to inhaled steroids for treating children. A similar class of drugs, 5-LOX inhibitors, may be used as an"}, {"id": "wiki20220301en002_60426", "title": "Asthma", "score": 0.012321779744346116, "content": "Mast cell stabilizers (such as cromolyn sodium) are safe alternatives to corticosteroids but not preferred because they have to be administered frequently. Oral Theophyllines are sometimes used for controlling chronic asthma, but their used is minimized because of their side effects. Omalizumab, a monoclonal Antibody Against IgE, is a novel way to lessen exacerbations by lessening the levels of circulating IgE that play a significant role at allergic asthma. Anticholinergic medications such as ipratropium bromide have not been shown to be beneficial for treating chronic asthma in children over 2 years old, but is not suggested for routine treatment of chronic asthma in adults."}, {"id": "pubmed23n0423_3437", "title": "Treatment of allergic rhinitis during pregnancy.", "score": 0.011924982307147912, "content": "Allergic rhinitis is a frequent problem during pregnancy. In addition, physiological changes associated with pregnancy can affect the upper airways. Evidence-based guidelines on the management of allergic rhinitis have recently been published, the most recent being the Allergic Rhinitis and its Impact on Asthma (ARIA)--World Health Organization consensus. Many pregnant women experience allergic rhinitis and particular attention is required when prescribing drugs to these patients. Medication can be prescribed during pregnancy when the apparent benefit of the drug is greater than the apparent risk. Usually, there is at least one drug from each major class that can be safely utilised to control symptoms. All glucocorticosteroids are teratogenic in animals but, when the indication is clear (for diseases possibly associated, such as severe asthma exacerbation), the benefit of the drug is far greater than the risk. Inhaled glucocorticosteroids (e.g. beclomethasone or budesonide) have not been incriminated as teratogens in humans and are used by pregnant women who have asthma. A few histamine H(1)-receptor antagonists (H(1)-antihistamines) can safely be used as well. Most oral decongestants (except pseudoephedrine) are teratogenic in animals. There are no such data available for intra-nasal decongestants. Finally, pregnancy is not considered as a contraindication for the continuation of allergen specific immunotherapy."}, {"id": "wiki20220301en002_60422", "title": "Asthma", "score": 0.01192377028071079, "content": "Corticosteroids are generally considered the most effective treatment available for long-term control. Inhaled forms are usually used except in the case of severe persistent disease, in which oral corticosteroids may be needed. Dosage depends on the severity of symptoms. High dosage and long term use might lead to the appearance of common adverse effects which are growth delay, adrenal suppression, and osteoporosis. Continuous (daily) use of an inhaled corticosteroid, rather than its intermitted use, seems to provide better results in controlling asthma exacerbations. Commonly used corticosteroids are budesonide, fluticasone, mometasone and ciclesonide."}, {"id": "wiki20220301en002_60411", "title": "Asthma", "score": 0.011841685285668688, "content": "either because optimum doses of asthma medications do not work (called \"refractory\" asthma) or because individuals are either unable (e.g. inability to afford treatment, poor inhaler technique) or unwilling (e.g., wish to avoid side effects of corticosteroids) to take optimum doses of prescribed asthma medications (called \"difficult to treat\" asthma). In practice, it is not possible to distinguish \"refractory\" from \"difficult to treat\" categories for patients who have never taken optimum doses of asthma medications. A related issue is that the asthma efficacy trials upon which the pharmacological treatment guidelines are based have systematically excluded the majority of people with asthma. For example, asthma efficacy treatment trials always exclude otherwise eligible people who smoke, and smoking blunts the efficacy of inhaled corticosteroids, the mainstay of asthma control management."}, {"id": "wiki20220301en038_67958", "title": "Budesonide/formoterol", "score": 0.011595238095238094, "content": "Common (≥1/100 to <1/10) side effects include candidiasis, headache, tremor, palpitations, throat irritation, coughing, and dysphonia. Pneumonia is a common side effect in people with COPD, and other, less common side effects have been documented. There were concerns that the LABA component increases the risk of death in children with asthma, however these concerns were removed in 2017. Therefore, this combination is only recommended in those who are not controlled on an inhaled steroid alone. There is tentative evidence that typical doses of inhaled steroids and LABAs are safe in pregnancy. Both formoterol and budesonide are excreted in breast-milk."}, {"id": "wiki20220301en041_48902", "title": "Montelukast", "score": 0.011084284460052678, "content": "Montelukast, sold under the brand name Singulair among others, is a medication used in the maintenance treatment of asthma. It is generally less preferred for this use than inhaled corticosteroids. It is not useful for acute asthma attacks. Other uses include allergic rhinitis and hives of long duration. For allergic rhinitis it is a second-line treatment. Common side effects include abdominal pain, cough, and headache. Severe side effects may include allergic reactions, such as anaphylaxis and eosinophilia. Use in pregnancy appears to be safe. Montelukast is in the leukotriene receptor antagonist family of medications. It works by blocking the action of leukotriene D4 in the lungs resulting in decreased inflammation and relaxation of smooth muscle."}, {"id": "article-38192_15", "title": "Asthma in Pregnancy -- Treatment / Management", "score": 0.011026077097505669, "content": "Salbutamol is the preferred reliever due to its high safety profile. Inhaled corticosteroids (ICS) are the preferred controller medications. It is safe to use ICS, theophylline, and montelukast during pregnancy. Prolonged use of systemic steroids has been associated with pregnancy-related complications, especially in the first trimester. But systemic steroids if indicated they should be used the same as in non-pregnancy (Evidence C). Research has suggested that management of asthma in pregnancy based on the fraction of exhaled nitric oxide (FENO) and symptoms significantly reduces asthma exacerbations. [9] In moderate-persistent asthma, a long-acting beta 2 agonist combined with an inhaled anti-inflammatory agent or inhaled corticosteroid is recommended for treatment. In severe asthma, oral corticosteroids and long-acting beta agonists are recommended. Inhaled glucocorticoids are relatively safe although there is a potential risk for endocrine and metabolic disturbances in fetuses. Sustained use of systemic steroids may increase the risk of congenital malformation, prematurity, neonatal insufficiency, low birth weight, preeclampsia, and gestational diabetes. [10] If anesthesia is indicated during labor, regional anesthesia is preferred. [11] [7]"}, {"id": "wiki20220301en337_39171", "title": "Budesonide", "score": 0.009900990099009901, "content": "Medical uses Asthma Budesonide is given by metered-dose inhaler or nebulizer for maintenance and prophylactic treatment of asthma, including patients who require oral corticosteroids and those who may benefit from a systemic dose reduction. Inflammatory bowel disease Formulations of delayed-release budesonide are an effective treatment for mild-to-moderately active Crohn's disease involving the ileum and/or ascending colon. A Cochrane review found evidence for up to three months (but not longer) of maintenance of remission in Crohn's disease. Budesonide assists in the induction of remission in people with active ulcerative colitis. Budesonide is highly effective and recommended as the drug of choice in microscopic colitis, for induction and maintenance of remission, and for both the lymphocytic colitis and collagenous colitis forms. Allergic rhinitis Budesonide in the form of nasal sprays is a treatment for allergic rhinitis."}, {"id": "pubmed23n0802_14822", "title": "Omalizumab in pregnant women treated due to severe asthma: two case reports of good outcomes of pregnancies.", "score": 0.009900990099009901, "content": "The article presents case reports of 2 women who became pregnant during therapy with omalizumab. Both subjects suffered from very severe asthma and were treated chronically with all available medications including systemic steroids (first - 20 mg prednisolone/day, second - 40 mg prednisolone/day). Both were enrolled into treatment with omalizumab and started regular therapy in 2007. The course of asthma significantly improved in both cases (withdrawal of oral steroids or significant reduction of their dose, better asthma control). The first woman, 32-year-old, became pregnant in 2010 and gave birth in Oct 2010 - it was her 3(rd) pregnancy, and 3(rd) labor. The second woman, 31-year-old, also became pregnant in 2010 and gave birth in Jan 2011 - it was her 5(th) pregnancy and 2(nd) labor. Both had severe asthma exacerbations during previous pregnancies and labors, and decided to continue therapy with omalizumab. The first woman, besides omalizumab, was treated with high doses of inhaled corticosteroids (ICS) and long-acting β agonists (LABA) while the second one was treated with high doses of ICS, LABA and 2.5 mg to 5 mg prednisone/day. The pregnancies proceeded without asthma exacerbations. The first woman delivered a healthy girl (Apgar 9, weight 3200 g, length 56 cm) in the 40(th) week of pregnancy by caesarean section due to the narrow pelvis. The second one delivered a healthy boy (Apgar 9, weight 3800 g, length 56 cm) in week 40 by caesarean section due to the aggravating obstetrical history. In both cases, treatment with omalizumab did not affect pregnancies and newborns. "}, {"id": "wiki20220301en015_4085", "title": "Hypersensitivity", "score": 0.00980392156862745, "content": "Allergic bronchial asthma can be treated with any of the following: inhaled short- and long-acting bronchodilators (anticholinergics) along with inhaled corticosteroids, leukotriene antagonists, use of disodium cromoglycate, and environmental control. Experimentally, a low dose of methotrexate or cyclosporin and omalizumab (a monoclonal anti-IgE antibody) has been used. Treatment of autoimmune disorders (e.g., SLE) include one or a combination of NSAIDs and hydroxychloroquine, azathioprine, methotrexate, mycophenolate, cyclophosphamide, low dose IL-2, intravenous immunoglobulins, and belimumab. Omalizumab is a monoclonal antibody that interacts with the binding site of the high-affinity IgE receptor on mast cells. It is an engineered, humanized recombinant immunoglobulin. Moderate to severe allergic bronchial asthma can improve with omalizumab. Delayed hypersensitivity reactions Treatment of type 4 HR involves the treatment of the eliciting cause."}, {"id": "pubmed23n0747_17279", "title": "[A case of an asthma patient receiving omalizumab during pregnancy].", "score": 0.009708737864077669, "content": "We describe the first report in Japan of a woman who received omalizumab during pregnancy and delivery. Her asthma was so severe that she had been taking systemic corticosteroids since 22 years old, but asthma was poorly controlled. She had been pregnant seven times before, but almost every time asthma control had worsened and spontaneous abortion resulted, so she had only one child. She confirmed that she was not intending to become pregnant, and initiated use of omalizumab in August 2009. However, pregnancy was identified after she had taken the drug 3 times. We explained the risks in detail, but the patient wanted to keep taking omalizumab, as her asthma control was improved and she thought she could continue the pregnancy. We therefore decided to continue with omalizumab therapy. In October, she caught a cold and experienced asthma exacerbation. Despite the risk, she decided to suspend omalizumab therapy after taking the drug 7 times, as she was not feeling any benefit from therapy. Threat of abortion was identified in February 2010, so a 544-g female baby was delivered at 26 weeks gestation by Cesarean section. The baby had to be hospitalized in the neonatal intensive care unit because of low birth weight, but she has been developing and growing without handicap. We report this case as the first known case of pregnancy and delivery for a woman receiving omalizumab in Japan. Omalizumab may be safe to use in pregnant women with difficult-to-control asthma."}, {"id": "pubmed23n0581_14776", "title": "Omalizumab: new drug. Asthma: too many unknowns for an anti-IgE.", "score": 0.009662061239731142, "content": "(1) Long-term therapy for severe asthma (stage 4) currently consists of a high-dose inhaled steroid plus a long-acting beta-2 agonist. (2) Omalizumab is a recombinant anti-IgE antibody marketed for the treatment of severe allergic asthma associated with high circulating IgE levels, as an adjunct to inadequate ongoing treatment. Omalizumab is administered subcutaneously every 2 to 4 weeks. (3) Omalizumab has been evaluated in many clinical trials but only one, a double-blind randomised placebo-controlled trial lasting 28 weeks and involving 419 patients, corresponded to the approved indications. Omalizumab adjunction to ongoing treatment prevented one emergency department admission for asthma every 2.5 years on average, but this trial provided weak evidence as it suffered from several biases. In particular, the two groups were not identical at baseline in terms of prior frequency of asthma exacerbations. These were more frequent in the omalizumab group. In addition, many analyses were retrospective, few events occurred, and the protocol was modified 4 times. (4) A combined analysis of this and 4 other trials involving slightly different patients suggests that omalizumab prevents one asthma emergency approximately every 3 patient-years. (5) During comparative trials, the main adverse effects were anaphylaxis and local injection site reactions. Omalizumab may increase the risk of parasitic infections. Cases of laryngeal oedema and angioedema have been reported. (6) The possibility of an increased risk of cancer is to be examined in a post-marketing cohort study. (7) Treatment can cost more than 1700 euros a month. (8) In practice, in view of the potentially severe adverse effects of omalizumab, the evidence supporting its efficacy is too weak. It is better to carefully tailor existing treatments to the individual patient."}, {"id": "pubmed23n0557_3816", "title": "[A pregnant woman with severe asthma effectively treated by inhalational lidocaine therapy].", "score": 0.009615384615384616, "content": "We report a case of a pregnant woman with severe asthma that was not controlled with ordinary medications but was effectively treated by inhalational lidocaine treatment. The case was a 27-year-old woman who had been repeatedly hospitalized due to acute asthma since her infancy. The patient had an episode of asthma attack caused by the use of aspirin. The daily medication for controlling her asthma included 1.5mg betamethasone. In February, 2004, she was hospitalized because of asthma exacerbation during her 11th week of pregnancy. Despite intensive treatments including repetition of inhaled beta2-agonist and anti-cholinergic drugs, intravenous injection of betamethasone and theophylline, and a leukotriene receptor-antagonist, no obvious improvement in severe cough, wheeze, or hypoxemia was observed for more than 3 weeks. Then inhalational lidocaine was introduced according to the method described by Mayo Clinic, USA. Namely, following inhalation of beta2-agonist, 40 to 100mg lidocaine was given via an ultrasonic nebulizer 5 times a day. Interestingly, symptoms such as wheezing or cough and also her hypoxemia dramatically improved following this treatment and reduction of systemic corticosteroid became possible. Finally, she was delivered of a girl by caesarean section. Nebulized lidocaine treatment may be an useful option as supplementary treatment for refractory asthma especially in pregnant cases."}, {"id": "pubmed23n0512_20662", "title": "Management of asthma during pregnancy.", "score": 0.009615384615384616, "content": "Asthma is estimated to affect up to 4% of pregnancies. Management of asthma during pregnancy follows the same approach as in the general population. Aggressive treatment should be entertained because asthma under poor control during pregnancy can lead to poor outcomes for the mother and child. The foundations of management are environmental avoidance procedures, proper pharmacologic agents, and specific allergen immunotherapy. For pregnant women with persistent asthma, the use of inhaled cromolyn or inhaled budesonide should be considered as first-line agents. Short-acting beta-agonists can be used as needed in all asthma categories. Other agents such as salmeterol, leukotriene modifiers, newer inhaled corticosteroids, and omalizumab may be considered in women who showed a good response to these agents before pregnancy."}, {"id": "pubmed23n0946_888", "title": "Case study: A Combination of Mepolizumab and Omaluzimab injections for severe asthma.", "score": 0.009523809523809525, "content": "A Combination of Mepolizumab and Omaluzimab injections for severe asthma. Patients with severe persistent asthma account for a large proportion of asthma morbidity and health care expenditures. In this case report we describe the use of a combination of omalizumab and mepolizumab in severe asthma with elevated IgE levels and eosinophilic phenotype. We are treating a 55 year old woman with severe persistent eosinophilic asthma and elevated IgE levels. Her regimen included salmeterol/fluticasone propionate inhaler 500/50 one puff twice a day, budesonide nebulizer twice a day, tiotropium respimat inhaler two puffs daily, montelukast 10 mg daily, Albuterol as needed, Azithromycin 250 mg three times a week, and also omalizumab injections. She was having recurrent asthma exacerbations requiring the use of oral corticosteroids. Due to frequent exacerbations, we referred her for Bronchial Thermoplasty. This procedure was denied by insurance and therefore the patient was started on 20 mg PO prednisone daily. Mepolizumab was added approximately 4 months after starting chronic PO prednisone. We were able to taper down the steroids and she is currently on 4 mg daily. Since we added the mepolizumab injections along with the omalizumab injections, we have been able to decrease the prednisone steadily and currently the patient is on 4 mg daily. The plan is to taper off the corticosteroids slowly as the clinical status allows. Combination of omalizumab and mepolizumab could become the gold standard for severe asthma patients that have elevated IgE levels and an eosinophilic phenotype. A Combination of Mepolizumab and Omaluzimab injections for severe asthma."}, {"id": "pubmed23n1166_1646", "title": "Successful and safe treatment of severe steroid depended eosinophilic asthma with mepolizumab in a woman during pregnancy.", "score": 0.009523809523809525, "content": "A 26-year-old female with steroid dependent eosinophilic asthma and nasal polyps who had successfully been treated with mepolizumab for 17 consecutive months with complete steroid withdrawal and symptoms control, stopped biologic treatment due to pregnancy efforts. Mepolizumab discontinuation resulted in frequent exacerbations and daily symptoms despite high dose ICS/LABA and re-initiation of oral steroids. Mepolizumab was initiated again, followed by improvement of asthma control and gradual withdrawal of steroids within 2 months. The patient became pregnant during the fourth month of mepolizumab re-initiation. The patient presented two asthma exacerbations during pregnancy treated with short course (3 days) oral steroids and delivery was uneventful (female, Apgar 9, weight 2750 g, length 59 cm) in week 40 by caesarean section."}, {"id": "wiki20220301en038_67960", "title": "Budesonide/formoterol", "score": 0.009433962264150943, "content": "Use for both maintenance and as needed treatment is also known as single maintenance and reliever therapy ( SMART) and is a well-established treatment. It has been shown to reduce asthma exacerbations that require oral corticosteroids, hospital visits better than maintenance inhaled corticosteroids alone at a higher dose, or inhaled corticosteroid at the same or higher dose with a long acting bronchodilator (LABA)), with a short-acting bronchodilator (SABA) as a reliever. More studies using budesonide/formoterol SMART in children are needed. Side effects Common (up to 1 in 10 people) Mild throat irritation Coughing Hoarseness Oral candidiasis (thrush. significantly less likely if the patient rinses their mouth out with water after inhalations) Headache Often mild, and usually disappear as the medication continues to be used: Heart palpitations Trembling Uncommon (up to 1 in 100 people)"}, {"id": "pubmed23n0639_10271", "title": "[Treatment of asthma during pregnancy].", "score": 0.009433962264150943, "content": "Treatment of asthma during pregnancy leads to discussion concerning medication and complications of pregnancy.Insecurity about possible teratogenity leads in the first trimester in 40% of pregnant women to reduction or cessation of asthma medication.This results, in pregnant women with moderate to severe asthma, in an increased consumption of rescue medication and number of exacerbations, and reduced asthma control. Asthma-exacerbations are significantly correlated with low birthweight. Of the majority of asthma medications, consumption during pregnancy has not been shown to cause harmful effects on the foetus. Adequate therapy for maintenance and exacerbations are essential, noting that the treatment in essence should not differ between pregnant and nonpregnant asthmatic women. A preconceptional consultation by a pulmonary physician and gynaecologist could improve care for pregnant women with asthma."}, {"id": "pubmed23n0368_22858", "title": "Budesonide inhalation suspension: a review of its use in infants, children and adults with inflammatory respiratory disorders.", "score": 0.009345794392523364, "content": "Budesonide, a topically active corticosteroid, has a broad spectrum of clinically significant local anti-inflammatory effects in patients with inflammatory lung diseases including persistent asthma. In infants and young children with persistent asthma, day- and night-time symptom scores, and the number of days in which beta2-agonist bronchodilators were required, were significantly lower during randomised, double-blind treatment with budesonide inhalation suspension 0.5 to 2 mg/day than placebo in 3 multicentre trials. Significantly fewer children discontinued therapy with budesonide inhalation suspension than with placebo because of worsening asthma symptoms in a study that included children who were receiving inhaled corticosteroids at baseline. Recent evidence indicates that budesonide inhalation suspension is significantly more effective than nebulised sodium cromoglycate in improving control of asthma in young children with persistent asthma. At a dosage of 2 mg/day, budesonide inhalation suspension significantly reduced the number of asthma exacerbations and requirements for systemic corticosteroids in preschool children with severe persistent asthma. In children with acute asthma or wheezing, the preparation was as effective as, or more effective than oral prednisolone in improving symptoms. In children with croup, single 2 or 4mg dosages of budesonide inhalation suspension were significantly more effective than placebo and as effective as oral dexamethasone 0.6 mg/kg or nebulised L-epinephrine (adrenaline) 4mg in alleviating croup symptoms and preventing or reducing the duration of hospitalisation. Early initiation of therapy with budesonide inhalation suspension 1 mg/day appears to reduce the need for mechanical ventilation and decrease overall corticosteroid usage in preterm very low birthweight infants at risk for chronic lung disease. In adults with persistent asthma, budesonide inhalation suspension &lt; or =8 mg/day has been compared with inhaled budesonide 1.6 mg/day and fluticasone propionate 2 mg/day administered by metered dose inhaler. Greater improvements in asthma control occurred in patients during treatment with budesonide inhalation suspension than with budesonide via metered dose inhaler, whereas fluticasone propionate produced greater increases in morning peak expiratory flow rates than nebulised budesonide. Several small studies suggest that the preparation has an oral corticosteroid-sparing effect in adults with persistent asthma and that it may be as effective as oral corticosteroids during acute exacerbations of asthma or chronic obstructive pulmonary disease. The frequency of adverse events was similar in children receiving budesonide inhalation suspension 0.25 to 2 mg/day or placebo in 12-week studies. During treatment with budesonide inhalation suspension 0.5 to 1 mg/day in 3 nonblind 52-week studies, growth velocity in children was generally unaffected; however, a small but statistically significant decrease in growth velocity was detected in children who were not using inhaled corticosteroids prior to the introduction of budesonide inhalation suspension. Hypothalamic-pituitary-adrenal axis function was not affected by short (12 weeks) or long (52 weeks) term treatment with nebulised budesonide. In conclusion, budesonide inhalation suspension is the most widely available nebulised corticosteroid, and in the US is the only inhaled corticosteroid indicated in children aged &gt; or =1 year with persistent asthma. The preparation is suitable for use in infants, children and adults with persistent asthma and in infants and children with croup."}, {"id": "pubmed23n0881_19735", "title": "Allergy Medications During Pregnancy.", "score": 0.009345794392523364, "content": "Allergic diseases are common in women of childbearing age. Both asthma and atopic conditions may worsen, improve or remain the same during pregnancy. Primary care physicians commonly encounter women receiving multiple medications for pre-existing atopic conditions, who then become pregnant and require medication changes to avoid potential fetal injury or congenital malformations. Each medication should be evaluated; intranasal and inhaled steroids are relatively safe to continue during pregnancy (budesonide is the drug of choice), second-generation antihistamines of choice are cetirizine and loratadine, leukotriene receptor antagonists are safe, sparing use of oral decongestants during the first trimester and omalizumab may be used for both uncontrolled asthma and for antihistamine-resistant urticaria. Medications to avoid during pregnancy include intranasal antihistamines, first-generation antihistamines, mycophenolate mofetil, methotrexate, cyclosporine, azathioprine and zilueton. Common allergic diseases may develop de novo during pregnancy, such as anaphylaxis."}, {"id": "pubmed23n0530_22589", "title": "[The management of bronchial asthma during pregnancy--Hungarian experiences].", "score": 0.009259259259259259, "content": "The prevalence of bronchial asthma is 4-8% among pregnant women. The complications, which threaten the asthmatic pregnancies according to the literature, are the following: spontaneous abortion, diabetes mellitus, caesarean delivery, pre-eclampsia, low gestational weight, neonatal icterus. The aim of the study was a retrospective analysis of the data of asthmatic pregnant patients managed between 2000 and 2004, with a special consideration on the treatment and gynecologic complications. The data of 53 persistent asthmatic patients--who had already undergone delivery--were collected. All of them were treated according to the guidelines issued in 2000 by the American College of Allergy, Asthma and Immunology and the American College of Obstetricians and Gynecologists: the inhalative corticosteroid budesonide, the long-acting beta-agonist formoterol or salmeterol and the short-acting beta-agonist terbutaline were used. The mean peak expiratory flow of the asthmatic pregnant patients was 71 +/- 16% of predicted, and the mean partial arterial oxygen tension 96 +/- 9 mmHg (means +/- SE). 10 of the 53 patients had cesarean delivery, 3 developed pre-eclampsia, 1 diabetes mellitus. The mean gestational age was 38.84 +/- 2.17 weeks and the weight of newborns 3132 +/- 604 g. The hospitalization was prolonged due to the infants' hyperbilirubinaemia in 3 cases. No congenital malformations or spontaneous abortions were detected. Based on the results of this retrospective study it can be concluded, that bronchial asthma slightly decreases the weight of newborns. The appropriate treatment of asthma during pregnancy resulted that the prevalence of gynecologic complications did not exceed the prevalence observed in the normal population--without increasing the risk of congenital malformations."}, {"id": "wiki20220301en354_4156", "title": "Mometasone/formoterol", "score": 0.009231467262320256, "content": "It is not for the treatment of acute bronchospasm. To relieve acute symptoms, a rapid-onset short-duration inhaled bronchodilator (such as salbutamol) should be available. Warnings and precautions Long-acting β adrenoreceptor agonists (LABAs) are subject to a boxed warning against the possibility of an increased risk of asthma-related death. Formoterol belongs to the LABA class of drugs. As there does not exist at the time of the monograph's publication adequate research to determine whether the rate of asthma-related death is increased with formoterol, it is therefore recommended by the FDA that LABAs only be used for patients not adequately controlled on other asthma controlling medications or whose disease severity clearly warrants initiation of dual therapy."}, {"id": "pubmed23n1098_22761", "title": "Real-Life Benefit of Omalizumab in Improving Control of Bronchial Asthma During COVID-19 Pandemic.", "score": 0.009174311926605505, "content": "Biologic therapy is recommended by Global Initiative for Asthma (GINA) guidelines in asthma patients not controlled with maximal inhaled therapy corresponding to GINA step 4. Omalizumab is an anti- immunoglobulin E (IgE) monoclonal antibody and the first biological available for the add-on treatment of severe allergic asthma, approved by Food and Drug Administration (FDA) in 2003. Diagnosing and managing asthma patients during coronavirus disease 2019 (COVID-19) pandemic since early 2020 has been challenging, mainly due to the risk of contracting COVID-19 disease and to the limited access to hospital care and pulmonary function tests. We report a case of a 52-year-old female patient, diagnosed with adult-onset asthma in 2018, who was first referred to the Allergy Department of our hospital in January 2019 for dyspnea, wheezing, and worsening cough. Despite continuous inhaled therapy and good inhalation technique, she had frequent asthma symptoms, requiring short courses of oral corticosteroids (CS). Physical examination and pulmonary function tests on admission revealed broncho-obstructive syndrome and laboratory tests showed mild inflammation and high total serum IgE. She continued to have two moderate-severe exacerbations after stepping up to maximal inhaled therapy plus oral montelukast and theophylline, according to GINA step 4. By the end of 2019, we additionally started omalizumab, which resulted in prompt clinical benefits and resolution of asthma symptoms. Given the ongoing COVID-19 pandemic limiting in-person visits, virtual follow-ups indicated adequate control of his symptoms, as proved by asthma control test and no need for hospital presentation."}, {"id": "pubmed23n0974_13664", "title": "What is safe enough - asthma in pregnancy - a review of current literature and recommendations.", "score": 0.009174311926605505, "content": "Although asthma is one of the most serious diseases causing complications during pregnancy, half of the women discontinue therapy thus diminishing the control of the disease, mostly due to the inadequate education and fear of adverse events. Sadly, this is sometimes encouraged by insufficiently educated physicians. Since the incidence and the prevalence of asthma is increasing, it is important to arouse the importance of proper asthma therapy during pregnancy. Inadequate therapy, as well as interrupting or discontinuing therapy, may result in adverse perinatal outcomes for both mother and child. The main goal of asthma control during pregnancy is control of symptoms and prevention of exacerbations, same as in every asthmatic, but even more important. Maintaining optimal lung function, as well as regular daily activities, ensures maintenance of optimal fetal oxygenation. The therapy should be adapted depending on the frequency and severity of daily and nocturnal symptoms, demand for reliever therapy, by the limitations in everyday activities and the frequency of emergency asthma-related hospitalizations. Pre-conceptual education and therapy are very important and should be supported by an asthma action plan adjusted for the period of pregnancy. It is very important to note that most of the drugs used before pregnancy can be safely continued during pregnancy. Pharmacological and non-pharmacological therapy should be used in parallel. Pregnant women should be informed about the nature of the disease, therapy used during pregnancy, possible complications, avoidance of triggers, proper administration of therapy and, most important, why should the therapy be continued throughout the pregnancy on individual basis. Although drug treatment should be based on using drugs with less harm risk, if control of severe symptoms is needed to be achieved in order to protect both mother and child, any anti-asthmatic drug would have the beneficial benefit/harm ratio. There is no solid evidence that asthma treatment during pregnancy causes adverse outcomes for the mother and child but for many, especially new drugs, there is not enough data gathered. On the other hand, harmfulness of uncontrolled asthma during pregnancy is well documented so every effort should be put on preserving good control of asthma during pregnancy."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[0, 86]], "word_ranges": [[0, 16]], "text": "It refers to a thyroid nodule, and the indicated test is a FNA (fine needle puncture)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "It refers to a thyroid nodule, and the indicated test is a FNA (fine needle puncture). I have read the answer Emilio has written on the challenge; the question is in the endocrine block and it seems he is trying to make it clear that it is a nodule. Ultrasound facilitates FNA but is not mandatory, a 2 cm thyroid nodule can be punctured without echo. It is important to have an analysis of thyroid function prior to the puncture; but since he is talking about an asymptomatic patient, it seems clear that she is not hyperthyroid. I do not think it is contested and I think it is a question that most will have answered well.", "full_answer_no_ref": "It refers to a thyroid nodule, and the indicated test is a FNA (fine needle puncture). I have read the answer Emilio has written on the challenge; the question is in the endocrine block and it seems he is trying to make it clear that it is a nodule. Ultrasound facilitates FNA but is not mandatory, a 2 cm thyroid nodule can be punctured without echo. It is important to have an analysis of thyroid function prior to the puncture; but since he is talking about an asymptomatic patient, it seems clear that she is not hyperthyroid. I do not think it is contested and I think [HIDDEN].", "full_question": "A 52-year-old woman from a village on the Costa Brava notices an otherwise asymptomatic lump in the anterior region when applying cream to her neck; she goes to her general practitioner who confirms the presence of a firm, smooth mass, 2 cm in maximum diameter, which rises with swallowing. No palpable lymphadenopathy. What tests would you order at the outset?", "id": 187, "lang": "en", "options": {"1": "A determination of thyroglobulin in blood.", "2": "A cervical CT scan.", "3": "A determination of circulating antithyroid antibodies (antithyroglubulin and antiperoxidase).", "4": "A fine needle puncture.", "5": "A determination of free T3."}, "question_id_specific": 68, "type": "ENDOCRINOLOGY", "year": 2013, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0628_4027", "title": "Identification of a neck lump as a lymph node metastasis from an occult contralateral papillary microcarcinoma of the thyroid: key role of thyroglobulin assay in the fine-needle aspirate.", "score": 0.017195767195767195, "content": "Thyroglobulin (Tg) assay of material from fine-needle aspiration of neck masses can help distinguish neck masses of thyroid origin from other masses. We describe its utility in a patient with an unusual constellation of findings, a neck lump identified as a lymph node metastasis from a contralateral occult papillary thyroid carcinoma (PTC). A 56-year-old woman was referred to our center for evaluation of a 15-mm right lateral cervical neck mass which was strongly hypoechoic, not homogenous and contained several microcalcifications. There was no family history of thyroid disease, the patient was euthyroid and was not taking medications for thyroid disorders. On physical examination the thyroid was slightly enlarged and was normal on ultrasound except for a 1 x 3 mm hypoechoic nodule in the middle of the left lobe. Ultrasound-guided fine-needle aspiration biopsy (FNAB) of the right lateral cervical mass was performed with the Tg concentration of the FNAB washout liquid being &gt;300 ng/mL and the cytology showing lymphoid elements mixed with polymorphous epithelial cells with atypical nuclei, suggesting lymph node metastasis from a cancer of epithelial origin. A lymph node metastasis from a papillary thyroid microcarcinoma (micro-PTC) was the presumptive diagnosis with the preoperative staging being Tx N1b. The patient underwent total thyroidectomy and bilateral lymph node dissection. At pathology, the right cervical mass was confirmed as lymph node metastasis of a PTC, and a unifocal micro-PTC was found in the middle left lobe. The patient was readmitted for a therapeutic (131)I dose (4810 MBq). At the time of (131)I administration, the whole-body scan showed only minimal thyroid bed uptake and serum Tg was &lt;1 ng/mL. She was maintained on l-thyroxine treatment (150 microg/d). Five year later she did not have evidence of recurrent or residual PTC. We describe the first case of contralateral lymph node metastasis from a unifocal micro-PTC identified by the detection of high Tg levels in the wash-out liquid of FNAB."}, {"id": "pubmed23n0361_3831", "title": "Appearance of antithyroglobulin antibodies as the sole sign of metastatic lymph nodes in a patient operated on for papillary thyroid cancer: a case report.", "score": 0.013595814033281866, "content": "We report a case in which the appearance of a progressively rising titer of thyroglobulin (Tg) antibodies was the most sensitive marker of papillary thyroid cancer relapse at loco-regional lymph nodes. The patient, a 60-year-old man, had been treated with total thyroidectomy plus lymphadenectomy and radioiodine ablation for a 1-cm papillary thyroid cancer. Twelve years after surgery, while undergoing levothyroxine (LT4) therapy, Tg antibodies were first detected and progressively rose from 99 to 1,697 U/mL in 18 months, while serum Tg remained undetectable. Both neck ultrasonography and 131I whole-body scan (WBS) were unremarkable. Enlarged lymph nodes became palpable at the left laterocervical region only 2 years after the first appearance of Tg antibodies and were surgically removed after cancer relapse was confirmed by fine-needle aspiration cytology. This case emphasizes the possibility that in thyroid cancer patients who have undergone total thyroidectomy, the appearance of Tg antibodies may indicate metastatic lymph nodes even when serum Tg is undetectable and WBS negative."}, {"id": "wiki20220301en319_25480", "title": "Neck mass", "score": 0.012658632484096793, "content": "A neck mass or neck lump is an ambiguous mass found in the neck area. There are many different possible causes, including congenital conditions like branchial anomalies and thyroglossal duct cysts. Workup Workup of a neck mass includes a medical history and a physical examination, where important characteristics are location, size, shape, consistency, tenderness, mobility, and color. When this is not conclusive, further workup includes: Blood tests Medical imaging: Contrast CT is generally the initial study of choice for adults. Medical ultrasound of the neck is useful in children because it avoids the radiation dose of CT. In some cases, fine needle aspiration may assist in the diagnosis. See also Cervical lymphadenopathy References External links Human head and neck"}, {"id": "wiki20220301en184_6403", "title": "Thyroid nodule", "score": 0.010718599033816424, "content": "Malignancy Only a small percentage of lumps in the neck are malignant (around 4 – 6.5%), and most thyroid nodules are benign colloid nodules. There are many factors to consider when diagnosing a malignant lump. Trouble swallowing or speaking, swollen cervical lymph nodes or a firm, immobile nodule are more indicative of malignancy, whereas a family history of autoimmune disease or goiter, thyroid hormonal dysfunction or a soft, painful nodule are more indicative of benignancy. The prevalence of cancer is higher in males, patients under 20 years old or over 70 years old, and patients with a history of head and neck irradiation or a family history of thyroid cancer. Solitary thyroid nodule"}, {"id": "pubmed23n0396_21131", "title": "Erythema nodosum associated with reactivation tuberculous lymphadenitis (scrofula).", "score": 0.009900990099009901, "content": "A 73-year-old African American female presented to our clinic with painful lower extremity lesions of 2 weeks duration. She was in her usual state of health until 3 months prior to presentation when she reported symptoms of fatigue and weakness. She also noticed an enlarging mass on the left side of her neck. She denied fevers, chills, night sweats or cough. Her symptoms were unresponsive to a course of oral dicloxacillin. The neck mass enlarged over 8 weeks and she was referred to our institution for evaluation. CT scan of the neck showed an enlarged lymph node. Ten days prior to her presentation in dermatology, a fine needle aspirate of the enlarging lymph node revealed necrotizing granulomas. Tissue was sent for routine mycobacterial and fungal cultures. Routine blood work, chest radiograph, and a tuberculin skin test were also performed. At the time of her dermatology visit she described the development of multiple new painful, non-pruritic lesions, bilaterally on the lower extremities. She also reported a red crusted area that appeared at the site of her tuberculin test that was placed subsequent to the development of her lower extremity lesions. Her past medical history was significant for Parkinson's disease, hypothyroidism and hypertension. Her current medications included l-thyroxine, estrogen and diltiazem. Her travel history was only remarkable for a trip to Jamaica the previous spring. She was born and raised in Haiti. She reported a history of a positive tuberculin skin test 20 years ago, but received no therapy. Physical examination revealed a 2 x 3 centimeter firm, nontender left lateral neck mass (Fig. 1). Her right forearm revealed an erythematous, ulcerated, indurated plaque 1.5 cm in diameter (Fig. 2.). Her lower extremities revealed tender 0.5 to 1 cm erythematous nodules below the knees bilaterally (Fig. 3). A punch biopsy of a lower extremity nodule revealed a mild pervisacular dermal infiltrate. Within the subcutaneous tissue there was septal widening. There was also a lymphohistiocytic infiltrate with a slight admixture of neutrophils within the septa of the fat lobules. There was no evidence of necrotizing vasculitis or collagen necrosis. An acid-fast stain was not performed. The histologic findings were consistent with a diagnosis of erythema nodosum. Her laboratory evaluation including CBC, electrolytes, thyroid studies, angiotensin converting enzyme level and chest radiograph were normal. Approximately 1 week after her dermatological evaluation, the fine-needle aspirate culture grew Mycobacterium tuberculosis. A diagnosis of tuberculous lymphadenitis associated with erythema nodosum was confirmed. The patient was started on quadruple therapy of isoniazid, rifampin, ethambutol and pyrazinamide. Her lower limb skins lesions rapidly resolved over the subsequent month and her neck mass also diminished in size. She completed 6 months of antituberculous therapy with complete resolution of her lymphadenopathy."}, {"id": "pubmed23n0318_10348", "title": "[Value of immunohistocytochemistry in the diagnosis of laterocervical masses in patients with a previous history of thyroid carcinoma].", "score": 0.009900990099009901, "content": "The aim of this study is to evaluate the additional diagnostic significance of immunocytochemical staining of thyreoglobulin (TG) in Fine Needle Aspiration Biopsy (FNAB) of neck lymph-nodes, in patients with a previous history of thyroid carcinoma. Twenty-five smears performed by ultrasound-guided FNAB on laterocervical nodes with a 21-23 gauge needle were evaluates. All smears were stained according to Papanicolaou and microscopically examined. Of these 25 smears, 15 were diagnostic and 10 were non diagnostic. Of the 15 diagnostic cases, 10 were positive for metastatic lesions from thyroid neoplasm and the other 4 were classified as reactive lymphoadenitis. One smear for each case was selected for the immunohistochemical stain. All the 10 non-diagnostic cases showed no reaction to thyreoglobulin. Neoplastic cells, from 9 out of 11 cytologically positive smears, expressed thyreoglobulin in the cytoplasm. In one case no reaction was evident and the other one was discarded for technical reasons. In 3 of the 4 cases cytologically classified as lymphoadenitis, immunoreactive thyreoglobulin was not found. In the fourth case, blastic-like cells showed a scanty cytoplasmic rime which was immunoreactive for TG and thus was classified as a metastatic tumour. On this basis, it is suggested that FNAB should be performed routinelly in the diagnostic evaluation of neck masses of unknown origin in patients with a previous history of thyroid neoplasm. If the FNAB is inconclusive, a second aspiration should be performed while immunoperoxidase stain to evidentiate TG may be an adjuntive diagnostic tool in cytologically negative cases."}, {"id": "pubmed23n0086_7108", "title": "[Fluctuations in the titers of anti-thyroid hormone and anti-thyroglobulin antibodies in 4 cases of Graves' disease during long-term treatment period].", "score": 0.00980392156862745, "content": "We studied 4 cases of Graves' disease with anti-thyroid hormone antibodies. Changes in the serum levels of triiodothyronine(T3), thyroxine(T4), free T4, thyrotropin(TSH), and thyroglobulin(Tg), as well as titers of anti-Tg antibodies, anti-thyroid hormone antibodies, anti-TSH receptor antibodies(TRAb) and anti-microsomal antibodies(MCHA) during 2 10 years' treatment periods were examined in each case. Case 1; A woman, who was diagnosed as having Graves' disease when she was 10 years old, had been treated with methimazole(MMI) or propylthiouracil(PTU). Treatment with the antithyroid drug had been discontinued by herself when she was 19 years old until she was 24 years old, when she was pregnant and consulted our hospital. Since her serum levels of T3 were unusually high, examination of her serum for the presence of anti-T3 antibodies was done. The presence of anti-T3 antibodies in her serum was confirmed. Case 2; A woman, who was diagnosed as having Graves' disease at the age of 41, had been treated with MMI or PTU. Presence of serum anti-T3 antibodies was found in a screening test for the antibodies. Serial sera were obtained during the 5 year observation period when she was treated with MMI, PTU, and subtotal thyroidectomy. Titers of anti-Tg antibodies in her sera were in the normal range. Case 3; A woman, who was diagnosed as having Graves' disease at the age of 11, had been treated with MMI or PTU. Presence of anti-T3 and anti-T4 antibodies were found in her sera in a screening test. Serial sera obtained during the 4 year treatment period were tested. Case 4; A woman, who was diagnosed as having Graves' disease at the age of 14, had been treated with MMI. Presence of anti-T3 and anti-T4 antibodies was found in her sera in a screening test. Serial sera obtained during the 2 year treatment period were tested. Titers of anti-Tg were increased when the levels of TSH or titers of TRAb were increased. The results suggested that TSH and TRAb, which are thyroid stimulating substances, increased serum levels of Tg, which resulted in the increase of titers of anti-Tg. Because of the possibility that administration of PSL could modify B lymphocyte functions, periods during which PSL was administered were excluded from the examination of the correlation between Tg concentrations or titers of anti-Tg and titers of anti-thyroid hormone antibodies, as in Cases 2 and 4, respectively.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0649_2209", "title": "[Usefulness of the determination of thyroglobulin in lymph node aspirates of patients with papillary thyroid carcinoma and positive antithyroglobulin antibodies].", "score": 0.00980392156862745, "content": "We wanted to study the utility of thyroglobulin determination in the washout of fine needle aspiration (FNAB-Tg) of lymph metastatic nodes in patients with papillar thyroid carcinoma (PTC) and positive serum thyroglobulin antibodies (AbTg). We have studied 11 patients (49.9+/-11.8 years old, 70% females) with PTC and positive AbTg in which a whole-body scanning (WBS) after (131)I treatment showed pathological uptake in lymph cervical nodes. An ultrasound-guided fine-needle aspiration biopsy (US-FNAB) was performed for cytological research. Needle-washout with 1 ml ClNa 0.9% was employed to determine FNAB-Tg. In 16/17 suspicious nodes Tg-FNAB concentration was higher than 7 ng/dl (223.3+/-314.2 [7-1009]). AbTg were negative in the washout obtained. WBS was able to detect 94% lymphadenopathies, whereas 76.5% were detected with ultrasound and 70.6% using cytology. The FNAB-Tg was positive in 94% of nodules, which was higher than combining US and FNAB-cytology both together (88.2%). One hundred per cent of pathological nodules were detected using US plus FNAB-Tg. FNAB-Tg determination is an useful technique for diagnosis of metastatic lymph nodes of patients with PTC and is unaffected by the presence of serum AbTg."}, {"id": "pubmed23n0944_20253", "title": "The importance of endoscopic ultrasound fine-needle aspiration in the diagnosis of solid pseudopapillary tumor of the pancreas: two case reports.", "score": 0.009708737864077669, "content": "Solid pseudopapillary tumor of the pancreas, otherwise known as solid and cystic tumor or Frantz tumor, is an unusual form of pancreatic carcinoma, with unknown etiopathogenesis, that accounts for 0.2 to 2.7% of all pancreatic tumors. It is defined as an exocrine pancreatic neoplasia that mainly affects women between the second and third decade of life, and its management is not well defined. Endoscopic ultrasound offers a key anatomical advantage in accessing the pancreas and endoscopic ultrasound fine-needle aspiration has become the gold standard method for the diagnosis of pancreatic lesions. Case 1: A 31-year-old white Hispanic woman presented with epigastric pain for 5 months. An abdominal ultrasound revealed a single 2 cm nodule in the uncinate process of her pancreas. Endoscopic ultrasound showed a regular, well-defined solid lesion with alternating cystic areas at the uncinate process of her pancreas, measuring 1.7 × 1.4 cm; endoscopic ultrasound fine-needle aspiration was then performed with cytopathological analysis compatible with solid pseudopapillary tumor. Body computed tomography confirmed the absence of metastases and she underwent conventional duodenopancreatectomy. However, she died 4 days after surgery due to postoperative surgical complications. Case 2: A 35-year-old Hispanic woman presented with left upper quadrant abdominal pain for 3 months, associated with a palpable mass at this region. A computed tomography scan showed a solitary nodule in the pancreatic body. Endoscopic ultrasound showed a regular, well-defined, homogeneous lesion with small anechoic (cystic) areas, measuring 2 × 2 cm, in between the pancreatic body and neck. Endoscopic ultrasound fine-needle aspiration was performed and cytopathological analysis was suggestive of a pseudopapillary solid tumor. She underwent a body-tail laparoscopic pancreatectomy with splenectomy. Nine months after the diagnosis, she remains asymptomatic, continuing regular follow-up in the oncology out-patient clinic. Solid pseudopapillary tumor is a rare pancreatic malignancy. Endoscopic ultrasound fine-needle aspiration is the gold standard method to characterize and diagnose this type of pancreatic lesion, making this an invaluable tool to help guide clinical management and improve the preoperative diagnostic yield."}, {"id": "InternalMed_Harrison_26906", "title": "InternalMed_Harrison", "score": 0.009708737864077669, "content": "Follow-Up Whole-Body Thyroid Scanning and Thyroglobulin Determinations Serum thyroglobulin is a sensitive marker of residual/ recurrent thyroid cancer after ablation of the residual postsurgical thyroid tissue. However, newer Tg assays have functional sensitivities as low as 0.1 ng/mL, as opposed to older assays with functional sensitivities of 1 ng/mL, reducing the number of patients with truly undetectable serum Tg levels. Because the vast majority of papillary thyroid cancer recurrences are in cervical lymph nodes, a neck ultrasound should be performed about 6 months after thyroid ablation; ultrasound has been shown to be more sensitive than WBS in this scenario."}, {"id": "pubmed23n0702_3386", "title": "Toxoplasmosis presenting as a swelling in the axillary tail of the breast and a palpable axillary lymph node mimicking malignancy: a case report.", "score": 0.009615384615384616, "content": "Lymphadenopathy is a common finding in toxoplasmosis. A breast mass due to toxoplasmosis is very rare, and only a few cases have been reported. We present a case of toxoplasmosis that presented as a swelling in the axillary tail of the breast with a palpable axillary lymph node which mimicked breast cancer. A 45-year-old otherwise healthy Caucasian woman presented with a lump on the lateral aspect of her left breast. Her mother had breast cancer that was diagnosed at the age of 66 years. During an examination, we discovered that our patient had a discrete, firm lump in the axillary tail of her left breast and an enlarged, palpable lymph node in her left axilla. Her right breast and axilla were normal. The clinical diagnosis was malignancy in the left breast. Ultrasound and mammographic examinations of her breast suggested a pathological process but were not conclusive. She had targeted fine-needle aspiration cytology (FNAC) and core biopsy of the lesions. FNAC was indeterminate (C3) but suggested a possibility of toxoplasmosis. The core biopsy was not suggestive of malignancy but showed granulomatous inflammation. She had a wide local excision of the breast lump and an axillary lymph node biopsy. Histopathology and immunohistochemical studies excluded carcinoma or lymphoma but suggested the possibility of intramammary and axillary toxoplasmic lymphadenopathy. The results of Toxoplasma gondii IgM and IgG serology tests were positive, supporting a diagnosis of toxoplasmosis. Toxoplasmosis rarely presents as a pseudotumor of the breast. FNAC and histology are valuable tools for a diagnosis of toxoplasmosis, and serology is an important adjunct for confirmation."}, {"id": "pubmed23n0423_12464", "title": "[Results of fine needle aspiration biopsy, frozen section diagnosis and definite histological results in thyroid pathology. Report of 163 cases].", "score": 0.009615384615384616, "content": "In thyroid diseases, the place of fine needle aspiration biopsy still continues to be discussed: the sensibility and specificity vary greatly in the literature. Frozen section diagnosis is necessary to form a diagnostic strategy. The objective of this study was compare the results of fine needle aspiration biopsy, frozen section diagnosis, and definitive histologic results in a population of 163 patients and to draw conclusions about treatment. From 1994 to 1999, 163 patients (132 females and, 31 males) undergoing thyroid surgery were included in this retrospective study, after a standard preoperative work-up. Those with a single palpable nodule and hypofixation on scintigraphy underwent fine needle aspiration before surgery. These results were compared with the definitive histologic results. A loboisthmectomy was performed in 88 cases (54%), a subtotal thyroidectomy in 34 cases (21%), and a total thyrodectomy in 41 cases (25%). In the latter group, an associated neck dissection was performed in 18 cases (11%); a frozen section diagnosis was obtained in all cases of thyroid nodules. This study demonstrated a single nodule in 97 cases (60%), multiple nodules in 27 cases (17%), multinodular goitre in 34 cases (21%), and 5 Basedow diseases (3%). Sixty-two cases (38%) of thyroid nodules underwent fine needle aspiration before surgery. In 25 cases (15%), definitive pathology showed a malignant lesion. The frozen section diagnosis had a sensitivity of 73% and a specificity of 99%, and the fine needle aspiration biopsy had a sensitivity of 40% and a specificity of 100%. The authors propose fine needle aspiration biopsy in the following cases: a single palpable nodule and hypofixation on scintigraphy or a surgical contra indication; and direct surgery in symptomatic thyroid disease or if there are one or several full nodules &gt; 2 cm. In near future, these indications will be modified with the increasing reliability of fine needle aspiration biopsy."}, {"id": "pubmed23n0694_7473", "title": "A case of quadruple primary malignancies including breast, tongue, and thyroid cancers and osteosarcoma in a young female without karyotype abnormality.", "score": 0.009523809523809525, "content": "The patient was a 41-year-old, premenopausal woman with a chief complaint of well-circumscribed palpable, right breast mass without nipple discharge. Although she noticed the lump 3 months previously, the size of the tumor (1.1 × 0.9 cm(2)) had been stable. The patient's mother suffered from gastric cancer. Her previous history of the triple different malignancies was as follows: (1) left osteosarcoma [amputation of left lower leg at 15 years old (y/o)]. After the operation, she was treated with various kinds of anticancer drugs including a total of 45 g ifosphamide and 342 g methotrexate; (2) tongue cancer (right radical neck resection; 23 y/o); and (3) thyroid cancer (right lobectomy; 40 y/o). There was no evidence of recurrence of these malignancies at the present consultation. At the time of tongue cancer operation, chromosome abnormality was investigated, but the results were normal. Physical examination showed a well-delimited, elastic-firm, mobile tumor in the central outer right breast. Regional lymph nodes were not palpable. Mammography showed a focal asymmetry in the right upper breast on the mediolateral oblique view. Ultrasonography revealed a hypoechoic mass with irregular margins. Distant metastases could not be detected by whole-body computed tomography scan. The histology of the Mammotome(®) (vacuum-assisted core needle biopsy) specimen revealed that this tumor was low-grade ductal carcinoma in situ (DCIS). She underwent breast-conserving surgery with sentinel lymph node biopsy. On permanent histopathological examination, the diagnosis of the tumor was intracystic papilloma with low-grade DCIS. Surgical margin was negative, and sentinel lymph node metastases could not be observed. Estrogen and progesterone receptor (ER/PR) were strongly positive, but human epidermal growth factor receptor-2 (HER-2) overexpression was not tested because the lesion was DCIS. She has received no adjuvant therapy and is currently disease free 3 months after surgery."}, {"id": "pubmed23n0053_6910", "title": "Detection of thyroglobulin in fine needle aspirates of nonthyroidal neck masses: a clue to the diagnosis of metastatic differentiated thyroid cancer.", "score": 0.009523809523809525, "content": "We studied the feasibility of employing the measurement of thyroglobulin (Tg) in the washout of the needle used to perform the fine needle aspiration cytology (FNA-Tg) for the differential diagnosis of nonthyroidal neck masses of unknown etiology. We studied 35 patients presenting for 1 or more neck lumps outside the thyroid gland. A previous history of treated differentiated thyroid cancer (DTC) was given by 23 patients and of nonthyroidal malignancy by 3 patients. FNA-Tg was measured in the Tg-free serum used to wash out the needle employed for the cytology. Finally, all patients were treated by surgery. FNA-Tg was always detectable in 14 patients with thyroid cancer metastases demonstrated by histology, with a mean (+/- SD) of 27,087 +/- 37,622 ng/FNA (P less than 0.002) compared to patients without thyroid cancer metastases (mean +/- SD, 12.1 +/- 4.8 ng/FNA in 7 cases; undetectable in 14 cases). Assuming 21.7 ng/FNA (the mean +/- 2 SD of the negative patients) as the cut-off value, all patients with metastases from DTC were detected by FNA-Tg. FNA-Tg had better negative predictive value than cytology, since this last technique gave 10 inconclusive results, comprising 2 false negative results in patients with metastases from DTC. Our results indicate that elevated concentrations of FNA-Tg in nonthyroidal neck nodes strongly suggest the diagnosis of metastases from DTC."}, {"id": "pubmed23n0406_579", "title": "Hangman's fracture caused by suspected child abuse. A case report.", "score": 0.009433962264150943, "content": "This report highlights the difficulties associated with diagnosing cervical spine injuries in children especially as the history and mechanism of injury may often be unclear and the normal variations in roentgenographic appearance may be confusing. As far as we are aware this is only the second case of traumatic Hangman's fracture in a child under the age of 3 years and the only case where there is a strong probability of child abuse. A female child aged 23 months was admitted with a 5-day history of irritability and general malaise. Her father reported noticing that she was reluctant to move her neck. He denied any possibility of trauma. On admission she had neck stiffness with a temperature of 37 degrees C and supported her neck with her hands. There was evidence of otitis media of her right ear. Her physical examination was otherwise normal. A full blood count and lumbar puncture were within normal limits. Cervical spine x rays suggested a Hangman's fracture of C2 with slight anterior subluxation of C2 on C3 and a kyphus at that level. Computerized Tomography demonstrated no significant canal encroachment. An isotope bone scan was non-diagnostic. She was treated in a moulded cervical collar with neck held in slight extension. Her symptoms resolved and further radiographs showed improved alignment. Repeat CT scans seven weeks post admission showed callus formation. At follow-up at one year she remains asymptomatic. Hangman's fracture is very rare in children under 3 years and the considerable normal variations further complicate diagnosis. Swischuk described the posterior cervical line connecting the spinous process of C1-C3 vertebrae on the lateral projection to differentiate a true fracture dislocation from physiological anterior displacement. A detailed history, roentgenograms, bone scans, CT scans and MRI scans are often required for accurate diagnosis."}, {"id": "pubmed23n0571_12628", "title": "Thyroglobulin detection in fine-needle aspirates of cervical lymph nodes: a technique for the diagnosis of metastatic differentiated thyroid cancer.", "score": 0.009433962264150943, "content": "Fine-needle aspiration cytology is frequently used for differential diagnosis of neck masses of unknown origin. Inconclusive and even false-negative results are not uncommon. To evaluate the utility of thyroglobulin (Tg) measurement in fine-needle aspirates (FNA-Tg) for detecting cervical lymph node (CLNs) metastases from differentiated thyroid carcinomas. An ultrasound-guided fine-needle aspiration was done in 67 patients with 83 suspicious enlarged CLNs to obtain material for cytology and Tg measurement in the needle washout, using an immunometric chemiluminescent assay. Measurement of anti-Tg antibodies (FNA-TgAb) was also carried out in half of all the aspirates. Subjects were divided into two groups: one of 16 patients awaiting thyroidectomy and the other of 51 patients in follow-up after surgery. The first group of patients had positive FNA biopsy (FNAB-Tg) in 14 out of the 18 studied CLNs with a range of 3.2-43 352 ng/ml, while FNAB-cytology indicated metastasis in only 8 out of the 14 CLNs with positive histology. A total of 65 CLNs were studied in the follow-up group. Lymphadenectomy was performed in 23 patients and 28 aspirated CLNs were removed. Histology confirmed the diagnosis of metastasis suggested by FNAB-Tg in 20 CLNs and of reactive lymphadenitis in the remaining 8 CLNs. FNAB-cytology was positive in only 11 CLNs. Sensitivity of FNAB-Tg was not affected by the studied FNAB-TgAb. The FNAB-Tg achieved a sensitivity of 100% in both groups. FNAB-Tg is an easy and inexpensive technique which proved to increase the diagnostic of cytology in the early diagnosis of papillary carcinoma recurrence to CLN even in the presence of serum TgAb."}, {"id": "pubmed23n1104_15002", "title": "Redifferentiation of BRAF V600E-Mutated Radioiodine Refractory Metastatic Papillary Thyroid Cancer After Treatment With Dabrafenib and Trametinib.", "score": 0.009345794392523364, "content": "Radioactive iodine-refractory metastatic differentiated thyroid cancer (RAIR) is associated with a poor prognosis. Multikinase inhibitors have demonstrated improvement in progression-free but not overall survival in such patients, but usage is limited by significant adverse effects and the development of resistance. Clinical research has demonstrated improvement in progression-free survival with the combined use of the BRAF/MEK inhibitor in patients with metastatic melanoma and anaplastic thyroid cancer with the BRAF<supV600E </supmutation and has shown promise in redifferentiation of BRAF-positive RAIR differentiated thyroid cancer.  A 58-year-old woman went to her primary care physician for a growing mass on the left side of her neck. CT imaging noted a 6 x 8 x 6 cm mixed cystic and solid mass and lymphadenopathy. Core biopsy subsequently showed metastatic papillary thyroid cancer (Stage III, PT4a/PN1b), and she underwent a total thyroidectomy with left neck dissection. She then received 204mCi <sup131</supI post-total thyroidectomy. Unfortunately, her thyroglobulin continued to increase post-radioactive iodine (RAI) treatment, indicating persistent and/or recurrent thyroid cancer. An RAI-131 whole-body scan on the thyrogen protocol showed no significant RAI uptake. A fluorodeoxyglucose (FDG)-positron emission tomography (PET) CT scan was then performed, which showed recurrent metastatic disease with hypermetabolism noted in the left thyroid bed and FDG-avid bilateral cervical lymph nodes and pulmonary nodules. Given these findings, her cancer was classified as radioactive iodine refractory (RAIR). Molecular testing indicated the BRAF<supV600E</sup mutation. After a discussion with the patient, it was decided to initiate therapy with a BRAF inhibitor (dabrafenib 150 mg twice a day) and MEK inhibitor (trametinib 2 mg once a day) in an attempt to redifferentiate RAIR. Repeat RAI-131 thyrogen whole body scan one month after initiation of therapy demonstrated left level 2 cervical lymphadenopathy radioiodine uptake. The patient subsequently received 216 mCi <sup131</supI treatment given evidence of redifferentiation. Her post-treatment scan indicated additional uptake in a left lower lobe pulmonary nodule as well as a left paratracheal mass indicating successful RAI-131 uptake by metastases. Her thyroglobulin level, six months post-RAI, decreased to 4.0 indicating an encouraging response. Further surveillance, including imaging studies, is planned. This case illustrates the re-differential potential for dabrafenib and trametinib treatment in patients with BRAF<supV600E</sup-mutated RAIR differentiated thyroid cancer. This therapy has been shown to be successful in small series of patients and could potentially be offered to RAIR patients with the BRAF<supV600E</sup mutation as an alternative to multikinase treatment given its favorable side-effect profile."}, {"id": "pubmed23n0552_16987", "title": "The diagnostic value of thyroglobulin concentration in fine-needle aspiration of the cervical lymph nodes in patients with differentiated thyroid cancer.", "score": 0.009345794392523364, "content": "Recurrent differentiated thyroid cancer generally occurs first in the neck. Ultrasound is sensitive in detecting enlarged cervical lymph nodes but is not specific enough. Ultrasound-guided fine-needle biopsy increases the specificity but still may fail to detect a recurrence of the disease in the cystic metastatic lymph nodes. The aim of the study was to estimate the value of Tg concentration in the needle washout after fine-needle aspiration of suspicious lymph nodes. The 105 patients studied had presented one or more enlarged suspicious cervical lymph nodes. All had undergone total thyroidectomy and (131)I ablative therapy. Serum thyroglobulin (Tg) concentration was within the 0.15-711.5 ng/ml range (mean 22.24 ng/ml) and Tg recovery range 94-100%. The positive Tg washout concentration cut-off value was established as equal to the mean plus two standard deviations of the Tg washout concentration of patients with negative cytology. Lymph node involvement was diagnosed by cytology in 15 patients and in 28 lymph nodes. Positive Tg washout concentration was found in 22 patients and in 48 lymph nodes. All the lymph nodes which turned out to have positive cytology had a positive Tg washout concentration. All lymph nodes with positive cytology were positive in pathology. Seven patients and 20 lymph nodes with negative cytology were positive in the Tg washout concentration test. All but one patients and all but two lymph nodes with a positive Tg washout concentration had positive pathology. 1. Ultrasound-guided fine-needle biopsy is not sensitive enough to detect all metastatic lymph nodes. 2. The Tg washout concentration test is 100% sensitive in the detection of metastatic lymph nodes. 3. Cytology in ultrasound- guided fine-needle biopsy is 100% specific. 4. The Tg washout concentration test carries a risk of false-positive results. 5. Both methods should be used for early detection of metastatic lymph nodes in patients with differentiated thyroid cancer."}, {"id": "pubmed23n0951_12459", "title": "Current and Emerging Therapies for HER2-Positive Women With Metastatic Breast Cancer.", "score": 0.009259259259259259, "content": "<bCASE STUDY</b DE, a 31-year-old premenopausal woman with a nonsignificant medical history, noticed a right breast mass after playing basketball in September 2011. She initially attributed the mass to slight trauma, but after 2 weeks, she realized the mass was increasing in size. Her primary care physician ordered a bilateral screening mammogram and ultrasound. Mammography revealed no evidence of malignancy in the left breast. In the right breast, at the 7 o'clock position, a loose cluster of faint calcifications spanned a 2.2-cm area. Ultrasound confirmed an irregular hypoechoic mass in the right breast measuring 3.5 × 2.7 × 2.8 cm. Ultrasound of the right axilla identified two enlarged right axillary lymph nodes. Ultrasound core-needle biopsy of the suspicious right breast mass confirmed invasive ductal carcinoma, nuclear grade 2, Ki67 index of 55%, estrogen receptor-positive (H score of 180), progesterone receptor-positive (H score of 135), HER2-positive (3+ on immunohistochemistry). Utilizing the TNM (tumor, node, metastasis) staging system, she was clinically staged with a stage IIB (cT2, cN1, M0) invasive breast tumor. The computerized axial tomography (CT) scan of the chest, abdomen, and pelvis demonstrated the known right breast mass and two enlarged right axillary lymph nodes; however, no metastatic disease was noted. Nuclear bone scan revealed no bone metastases. Her medical oncologist recommended she receive neoadjuvant chemotherapy. The patient was treated with 6 cycles of neoadjuvant docetaxel at 75 mg/m², carboplatin at an AUC (area under the curve) of 6, and trastuzumab (Herceptin) at 6 mg/kg (TCH), which she tolerated well. She then underwent a right segmental mastectomy with axillary lymph node dissection and was found to have a residual 1.0-cm invasive ductal carcinoma, representing a 60% tumor volume reduction. None of 13 axillary lymph nodes were positive for disease. Pathologic staging confirmed a stage IA (ypT1, ypN0, M0) tumor. DE completed 33 fractions of radiation therapy to the right breast. She initiated endocrine therapy with tamoxifen at 20 mg daily and received 1 year of maintenance trastuzumab (6 mg/kg). Due to vaginal discharge and weight gain, endocrine therapy was switched from tamoxifen to toremifene (Fareston), which she tolerated relatively well. She continued routine follow-up, with no evidence of disease. In September 2014, DE presented to her primary care physician complaining of left hip pain. Magnetic resonance imaging (MRI) of the left hip revealed T2 hyperintense masses within the right anterior superior iliac crest, right sacrum, and left iliac body consistent with skeletal metastases. She was referred back to her medical oncologist, and per National Comprehensive Cancer Network (NCCN) guidelines, a biopsy of the suspicious lesion was obtained. The bone biopsy of the lytic lesion was consistent with metastatic breast cancer, which was estrogen receptor-positive, progesterone receptor-positive, and HER2-positive (3+ on immunohistochemistry). Restaging CT scan of the chest, abdomen, and pelvis revealed new 4- to 6-mm pulmonary nodules, hilar and mediastinal lymphadenopathy, new liver lesions, and bone lesions. Nuclear bone scan confirmed multiple bone metastases of the right and left iliac bones and sternum. Complete blood cell count with differential and complete metabolic panel were within normal ranges. The CA 27-29 tumor marker for breast cancer was elevated at 495 U/mL (normal range, &lt; 37 U/mL). DE was understandably devastated by the new diagnosis. She questioned how the treatment plan was to be established. Her medical oncologist struck a somewhat optimistic tone. He explained that metastatic breast cancer was not yet considered to be curable, but periods of disease stability and chronicity were possible. He explained that the HER2 positivity was perhaps the most important factor in delineating her treatment options. He told her that current treatment options were numerous and increasing in number."}, {"id": "pubmed23n0996_7183", "title": "Ultrasonography, Cytology, and Thyroglobulin Measurement Results of Cervical Nodal Metastasis in Patients With Unclear Papillary Thyroid Carcinoma.", "score": 0.009174311926605505, "content": "<bObjective:</b This study aimed to evaluate the ultrasonography (US), cytology, and thyroglobulin (Tg) measurement results of nodal metastasis in patients showing unclear US or cytology results of primary papillary thyroid carcinoma (PTC). <bMethods:</b From January 2016 to December 2018, 179 patients underwent US-guided fine-needle aspiration (FNA) to diagnose lymphadenopathy in the neck. Among them, 36 patients underwent subsequent total thyroidectomy and nodal dissection, and cervical lymph node (LN) metastasis from PTC was confirmed. However, two patients were excluded because of mismatch between the US and pathological findings of LNs. US images and cytological slides for metastatic LNs were retrospectively analyzed, and serum and FNA Tg levels for metastatic LNs were investigated using data from the electric medical records. Primary PTC patients with suspicious results on both US and cytology were classified as the clear group, and the remaining patients were classified as the unclear group. <bResults:</b Of the 34 patients, 24 had clear results of primary PTC on both US and cytology (clear group), whereas 10 had unclear results of primary PTC on US or cytology (unclear group). Of the 10 patients in the unclear group, seven had suspicious nodal metastasis from PTC on cytology after US-guided FNA of the cervical LN, and the remaining three had negative cytology but a positive Tg measurement. Metastatic LNs with cystic change tended to show a positive Tg measurement but negative cytology. <bConclusions:</b The combination of US, cytology, and Tg measurement is necessary for diagnosing nodal metastasis from PTC. In cases with unclear primary PTC on US or cytology, the detection of nodal metastasis may be helpful for assessing primary PTC."}, {"id": "pubmed23n0995_19384", "title": "THYROID METASTASIS FROM CLEAR CELL CARCINOMA OF THE KIDNEY 16 YEARS AFTER NEPHRECTOMY.", "score": 0.00909090909090909, "content": "The thyroid gland is one of the most vascularized organs in the body. However, metastatic disease to the thyroid gland is rare. When it does occur kidney is the most common primary tumor site, followed by melanoma, lung, breast, esophagus, uterus and colon carcinoma. We describe the case of an isolated thyroid metastasis from clear cell renal carcinoma occurring 16 years after nephrectomy. An 82 years-old woman presented for the recent growth of a right thyroid nodule, diagnosed 3 years before, when a fine needle aspiration biopsy found a benign cytology suggesting a well-differentiated follicular thyroid adenoma. Her medical history included type 2 diabetes mellitus, atrial fibrillation and a right nephrectomy for a clear cell renal carcinoma done 16 years before. The patient has lost weight but she was otherwise asymptomatic. The right lobe goiter was painless, firm, and mobile with deglutition, without signs of local compression or latero-cervical lymphadenopathy. Thyroid ultrasonography revealed an enlarged (9.9 cm) macronodular right lobe, with multiple cystic areas, with normal left lobe and a thrombus in the right internal jugular vein. Thyroid function tests were normal. The patient was suspected of thyroid carcinoma and underwent a near total thyroidectomy. Histopathological examination revealed a metastasis of clear cell renal carcinoma in the right thyroid gland lobe (8.5/5/5 cm). Further imaging showed no primary tumor or other metastases. Metastatic renal carcinoma to the thyroid should be considered in any patient presenting with a thyroid mass and a medical history of operated renal cell carcinoma, since it can occur up to 25 years after nephrectomy."}, {"id": "pubmed23n0510_16442", "title": "[Usefulness of radioiodine scanning in patients with moderate/high risk differentiated thyroid carcinoma in whom thyroglobulin after thyroxin withdrawal is undetectable after initial treatment].", "score": 0.00909090909090909, "content": "We selected 92 patients without antithyroglobulin antibodies (TgAb), in whom thyroglobulin (Tg) after L-thyroxin withdrawal was undetectable (&lt;1 ng/ml) 6-12 months after initial therapy and who were considered to be at moderate / high risk for recurrence by this criteria: age &gt;45 years; tumor size &gt;1.5 cm; and lymph nodes metastases in 43 (46.7%), local invasion in 26 (28.2%) or distant metastases in 23 (25%). Control whole-body scanning was negative in 78.2% of the cases and showed cervical uptake in the others. Cases presenting thyroid bed uptake in the absence of tumor recurrence did not receive radioiodine and Tg remained undetectable one year after the initial evaluation in all. Cervical uptake was not observed in 4/13 cases on repeated scan. In contrast, even in the absence of uptake and with undetectable Tg, 7 patients with recurrence confirmed by ultrasound (US) received surgical treatment. US showed 92.8% sensitivity for the detection of local-regional disease. The present study suggests that even moderate/high-risk patients without TgAb and with undetectable Tg levels (off T4) do not require radioiodine scanning after initial treatment and can be evaluated by cervical US."}, {"id": "pubmed23n0641_14730", "title": "[Patient with relapsed hyperparathyroidism and neck swelling].", "score": 0.009009009009009009, "content": "72-year-old woman with a history of primary hyperparathyroidism, for which she underwent surgery years previously, went to see her general practitioner because of a swelling in her neck that had been present for a few months and was growing in size. Other than this she had no symptoms. During the physical examination a solid elastic, non-fixed swelling with a diameter of about 3 cm was palpable on the right of the neck, medially to the sternocleidomastoid muscle. The swelling did not move when she swallowed. Laboratory tests and an MRI scan were suggestive of parathyroid carcinoma. An examination of the neck showed a large, irregular, lobed soft tumour and several small deposits with a yellowish brown appearance. Histology showed no characteristics of malignancy, but showed a picture consistent with the diagnosis of 'parathyromatosis', a rare disorder characterized by hormonally active ectopic parathyroid tissue. Treatment is primarily surgical, aimed at radical resection. Medicinal therapy using a calcimimetic agent may have a role as an adjuvant treatment."}, {"id": "pubmed23n0818_10946", "title": "Diagnostic value of thyroglobulin measurement with fine-needle aspiration biopsy for lymph node metastases in patients with a history of differentiated thyroid cancer.", "score": 0.009009009009009009, "content": "The aim of this study was to evaluate the diagnostic value of FNA-Tg for detecting lymph node metastases in patients with a history of differentiated thyroid cancer (DTC). A total of 58 patients with DTC diagnosis and evidence of single or multiple suspicious cervical lymph nodes were assessed. All underwent total or near-total thyroidectomy with (35 cases) or without (23 cases) radioiodine (RAI) ablation, followed by thyroid stimulating hormone (TSH) suppression therapy. A total of 68 lymph nodes were examined by ultrasound-guided fine needle aspiration (US-FNA) for both cytological examination and FNA-Tg measurement. Serum Tg and anti-thyroglobulin antibody (TgAb) levels were also measured. Diagnostic performance including sensitivity, specificity, accuracy, positive (PPV) and negative predictive value (NPV) of FNAC and FNA-Tg were calculated and compared. The Spearman's rank correlation coefficient was used to estimate the relationship between FNA-Tg and serum TgAb. The FNA-Tg levels were significantly higher with DTC metastatic lymph nodes (median 927.7 ng/mL, interquartile range 602.9 ng/mL) than non-metastatic lymph nodes (median 0.1 ng/mL, interquartile range 0.4 ng/mL) (p&lt;0.01). Considering 1.0 ng/mL as a threshold value for FNA-Tg, the sensitivity, specificity, accuracy, PPV and NPV of FNA-Tg were 95.7%, 95.5%, 95.6%, 97.8% and 91.3%, respectively. The sensitivity and accuracy of the combination of FNAC and FNA-Tg were significantly higher than that of FNAC alone (p&lt;0.05). The diagnostic performance of FNA-Tg was not significantly different between cases with or without RAI ablation, and the serum TgAb levels did not interfere with FNA-Tg measurements. Measurement of FNA-Tg is useful. The combination of FNAC and FNA-Tg is more sensitive and accurate for detecting lymph node metastases in patients with a history of DTC than FNAC alone. Serum TgAbs appear to be irrelevant for measurement of FNA-Tg."}, {"id": "pubmed23n0506_8296", "title": "Cases from the Osler Medical Service at Johns Hopkins University.", "score": 0.008928571428571428, "content": "PRESENTING FEATURES: An 85-year-old black woman presented to the Osler Medical Service complaining of a pruritic, erythematous scaly rash that was on her right thigh and abdomen and that had been worsening over the prior 3 months. She also complained of increasing fatigue, decreased exercise tolerance, and a 5-lbs weight loss. There was no orthopnea, paroxysmal nocturnal dyspnea, fevers, chills, or night sweats. She denied recent travel and exposures to or contact with ill people. Her past medical history was unremarkable. There was no history of eczema, atopy, or dermatologic conditions. Her only medication was a baby aspirin taken daily. On physical examination, she was afebrile, her blood pressure was 110/72 mm Hg, and her pulse was 82 beats per minute with a room air oxygen saturation 98%. She was mildly obese but in no apparent distress. She had 1-cm anterior cervical lymphadenopathy bilaterally and a 1-cm left axillary lymph node. Cardiovascular and chest examination was unremarkable. Her abdomen was soft and nontender, with a faint, erythematous rash that was mildly scaly but nontender in her abdominal skin fold. Her right thigh showed a large erythematous area, approximately 15 x 20 cm, which was scaly with multiple areas of discoloration (Figure 1). There was no palpable mass. She had slight edema in her right leg. Her left thigh was normal. Neurologic examination was nonfocal. Laboratory studies were notable for a white blood cell count of 17,000 cells/microL, with a differential of 46% lymphocytes. The absolute lymphocyte count was 8000 cells/microL. Hematocrit was 28%. Platelet count was normal. A comprehensive metabolic panel was normal. A peripheral blood smear (Figure 2) showed numerous atypical lymphocytes with cerebriform nuclei. Peripheral blood flow cytometry showed the presence of a clonal population of T cells that expressed CD4 and CD5 but showed a loss of CD7. What is the diagnosis?"}, {"id": "pubmed23n0259_12529", "title": "Role of neck ultrasonography in the follow-up of patients operated on for thyroid cancer.", "score": 0.008928571428571428, "content": "The aim of the study was to evaluate the role of neck ultrasonography in follow-up of patients with differentiated thyroid cancer. Sixty-three patients had total thyroidectomy and 131I ablation for differentiated thyroid cancer and had a negative whole body scan during follow-up. They were admitted for a high resolution neck ultrasound examination. Sixteen of 63 patients presented images suspicious for lymph node metastasis and/or for local recurrences (4 cases). Fine needle aspiration confirmed the suspicion of malignancy in 12 patients: only lymph node metastasis in 8 cases, local recurrence and lymph node metastasis in 3 cases, and in one case only local recurrence. Fine needle aspiration was suspicious for lymphadenitis in 4 cases. Thyroglobulin levels were very high in all patients with local recurrence and/or lymph node metastasis but undetectable in 2 cases presenting node metastasis and in 4 cases with lymphadenitis. All but one patient were admitted for surgery and the cytological diagnosis was confirmed. Early identification of a pathologic mass in the neck is a desirable goal; high resolution echography can play an important role in the follow-up of these patients and can detect local recurrences even when there is a negative whole body scan or undetectable thyroglobulin level."}, {"id": "pubmed23n1165_21079", "title": "[A Case of Oropharyngeal Tularemia Mimicking Lymphoma During Pregnancy].", "score": 0.008849557522123894, "content": "Tularemia is a zoonotic bacterial infectious disease caused by a gram-negative coccobacillus namely Francisella tularensis. In humans, disease leads to several different clinical forms (ulceroglandular, glandular, oculoglandular, respiratory, typhoidal and oropharyngeal). Since the main mode of transmission of the disease to humans in Türkiye is by drinking water contaminated with F.tularensis, the oropharyngeal form is the most common clinical manifestation. Since tularemia cases with pregnancy are rare, the literatüre about maternal and fetal complications of tularemia is sparse. In this report, a case of oropharyngeal tularemia mimicking lymphoma during pregnancy was presented. A 33-year-old 11-week pregnant patient living in a village in Sivas province admitted to the infectious diseases and clinical microbiology outpatient clinic with the complaint of swelling in the neck region that continued for six days. The patient, who was engaged in animal husbandry stated that she consumed raw milk and admitted to the otorhinolaryngology outpatient clinic of a hospital 10 days ago with the complaints of fever, chills, and sore throat. She stated that her complaints did not regress with the amoxicillin-clavulanate treatment recommended by her doctor and she noticed the swelling in her neck on the 4th day of the treatment. Upon further questioning, it was understood that the patient had a history of consumption of unchlorinated spring water. Her vital signs were normal and physical examination revealed non-fluctuant lymph nodes with the largest of 5 x 2 cm in the right posterior cervical region, and 3 x 2 cm in the left. Laboratory tests revealed a blood leukocyte count of 13.32 x 103/mm3 (75% granulocytes), a blood hemoglobin of 11.4 g/dL, an erythrocyte sedimentation rate of 45 mm/hour, and C-reactive protein of 90 mg/L. A non-contrast MRI examination revealed wall thickening of the nasopharynx and enlarged lymph nodes which were suspicious for lymphoma with significant diffusion restriction on diffusionweighted images. As the past medical history and clinical findings were suggestive for tularemia, the microagglutination test (MAT) was studied, but it was reported as negative with a titer at 1/80. Since the patient's complaints continued and tularemia cases were encountered in our region in the past years, the repeated MAT after two weeks was reported as positive with a titer at 1/320. An oropharyngeal form of tularemia was diagnosed and oral ciprofloxacin (2 x 750 mg) was given for three weeks by starting at the 14th gestational week. Lymphoma was excluded by histopathological examination of the fine needle aspiration biopsy performed on the patient's cervical lymph nodes, but the biopsy sample was compatible with granulomatous diseases. Histopathological findings of diagnostic biopsies of the larynx and nasopharynx were reactive. A healthy male baby, 2425 grams, 47 cm, was delivered by cesarean section from the patient who presented with labor contractions at the 37th week of pregnancy. There was no sign of congenital infection in the newborn. The patient and the baby were followed up to the end of one year and no abnormality was found. The evaluation of 17 cases reported in the literatüre including this case, suggest that tularemia may progress to involve serious obstetric complications during pregnancy, such as abortion, premature birth and intrauterine fetal death when appropriate and effective antibiotic treatment is not given."}, {"id": "pubmed23n0904_18228", "title": "Influence of presence/absence of thyroid gland on the cutoff value for thyroglobulin in lymph-node aspiration to detect metastatic papillary thyroid carcinoma.", "score": 0.008849557522123894, "content": "Thyroglobulin measurement with fine-needle aspiration (Tg-FNA) is a sensitive method for detecting metastatic papillary thyroid carcinoma (PTC). However, the diagnostic threshold is not well established and the influence of the thyroid gland on the cutoff value is also controversial. In this study, patients were classified into two groups according to the presence or absence of thyroid tissue, to determine an appropriate cutoff value for clinical practice. Patients with a history of thyroid nodules or surgery for PTC and with enlarged cervical lymph nodes on an FNA examination were enrolled for Tg-FNA detection. One hundred ninety-six lymph nodes (189 patients) were included: 100 from preoperative patients, 49 from patients treated with partial thyroid ablation, and 47 from patients with total thyroid ablation. In 149 lymph nodes from patient with thyroids, the cutoff value for Tg-FNA was 55.99 ng/mL (sensitivity, 95.1%; specificity, 100%), whereas in 47 lymph nodes from patients without a thyroid, it was 9.71 ng/mL (sensitivity, 96.7%; specificity, 100%). Thus, the cutoff value for Tg-FNA was higher in patients with thyroids than in patients without thyroids. The cutoff value for Tg-FNA is influenced by residual thyroid tissue, and a higher cutoff value is recommended for patients with thyroids than for patients without thyroids."}, {"id": "pubmed23n0555_21750", "title": "Spontaneous cervical lymphocele.", "score": 0.008771929824561403, "content": "Primary (spontaneous) cervical lymphoceles in adults are extremely rare. More frequently occurring acquired cervical lymphoceles have been described in the setting of a neck trauma or after a neck dissection. We report a case of a spontaneous left cervical lymphocele in a previously asymptomatic female. A 44-year-old woman presented with a 2-month history of a left neck mass initially noted by her physician during a routine physical examination. She denied prior head and neck surgery or neck trauma. CT scan of the neck revealed a left cystic mass. Fine-needle aspiration of the cyst yielded chylous material and lymphocytes. The surgical specimen grossly and microscopically was consistent with a lymphocele. The diagnosis was confirmed using D2-40 antibody targeting lymphatic endothelial cells lining the cyst. Primary cervical lymphocele should be included in the differential diagnosis of a solitary neck mass in an adult."}, {"id": "pubmed23n1150_14692", "title": "Impact of Thyroid Tissue Status on the Cut-Off Value of Lymph Node Fine-Needle Aspiration Thyroglobulin Measurements in Papillary Thyroid Cancer.", "score": 0.008771929824561403, "content": "<bObjective:</b To study the optimal cut-off value of thyroglobulin measurement in a fine-needle aspiration (FNA-Tg) in diagnosing malignant lymph nodes and benign lymph nodes (LNs) according to the thyroid tissue status. <bMethods:</b A total of 517 LNs were aspirated: 401 preoperative LNs, 42 LNs after subtotal thyroidectomy and 74 suspected LNs after total thyroidectomy. The cut-off value of FNA-Tg was obtained from receiver operating characteristic (ROC) analysis. The cut-off value with the best diagnostic performance was then obtained by comparing different cut-off values from other studies. <bResults:</b LN FNA-Tg levels differed between preoperative and total thyroid disease (<ip</i &lt; 0.001) and subtotal thyroidectomy and total thyroidectomy (<ip</i = 0.03), but not between preoperative and subtotal thyroidectomy (<ip</i = 1.00). Accordingly, those 443 LNs with preoperative and subtotal thyroidectomy were compared to those 74 without thyroid tissue. The optimal cut-off value in thyroid tissue group was 19.4 ng/ml and the area under the ROC curve (AUC) was 0.95 (95% CI 0.92-0.97). The optimal cut-off value in thyroid tissue absence group was 1.2 ng/ml and the AUC was 0.93 (0.85-0.98). After the analysis and comparison of multiple cut-off values, the optimal diagnostic performance was still found to be 19.4 ng/ml and 1.2 ng/ml. <bConclusion:</b The influential factors of FNA-Tg are still controversial, and the optimal cut-off value of FNA-Tg can be determined based on the presence or absence of thyroid tissue. FNA-Tg can be used as an important auxiliary method for diagnosing cervical metastatic LNs of thyroid cancer."}, {"id": "pubmed23n0594_7724", "title": "Erdheim-Chester disease with cutaneous features in an Indian patient.", "score": 0.008695652173913044, "content": "A 60-year-old Indian woman presented with multiple asymptomatic, firm swellings over the face that had been progressively increasing for the past 3.5 years. She complained of dry cough and dyspnea of 2 years' duration, which was diagnosed as interstitial lung disease (ILD) based on chest radiography and high-resolution computed tomography. Apart from occasional backaches, the patient had no other systemic complaints. The results of the general physical examination was normal, with no lymphadenopathy. Cutaneous examination revealed multiple (5) firm, yellowish to skin-colored well-defined nodules with irregular margins ranging in size from 1 x 1 cm to 4 x 8 cm present over the left periorbital region and right jawline, with overlying telangiectasias on the skin (Figure 1). On examination of the chest, she had generalized rhonchi and crepts; the remainder of the systemic examination results were normal. Fine needle aspiration cytology from the nodule in the periorbital area revealed a dispersed population of spindle cells, numerous foam cells, and giant cells suggestive of xanthogranuloma. Histopathology from the nodule on the jawline showed abundant foamy histiocytes in the dermis with few multinucleated giant cells and lymphocytes (Figure 2). The foamy histiocytes were periodic acid-Schiff-positive. On immunohistochemistry, histiocytes were CD68-positive. Findings of routine investigations including hemogram, peripheral blood smear, and blood chemistry were normal. Radiography of the lumbosacral spine showed mixed osteolytic and osteosclerotic lesions involving L5-S1, the left sacroiliac joint, and the left iliac blade. Findings from radiography of the long bones and skull were normal. Contrast-enhanced computed tomographic scan of the abdomen and pelvis showed areas of osteolysis, with marginal sclerosis present bilaterally that involved the iliac blades and the right half of the sacrum. A methylene diphosphate bone scan revealed increased tracer uptake seen at the fifth lumbar vertebrae, bilateral sacroiliac bone, and left iliac bone and areas of photopenia in sacrum. Bone marrow aspiration from the sternum showed normal cells with a normal erythroid-to-myeloid ratio. Ultrasonographic B scanning of the eyes showed no retro-orbital involvement. Erdheim-Chester disease was diagnosed in this patient on the basis of diagnostic histopathology, radiologic features involving bones, and ILD and treatment with 40 mg oral prednisolone daily was started. Surgical debulking of her skin lesions was planned, but the patient refused due to her worsening ILD."}, {"id": "pubmed23n0616_5479", "title": "[Thyroglobulin levels in needle lymph node cytology for the detection of papillary thyroid cancer recurrence].", "score": 0.008695652173913044, "content": "During the detection of neck recurrence in patients with Papillary Thyroid Carcinoma (PTC), sometimes it is difficult to distinguish metastatic from inflammatory neck lymph nodes. The measurement of serum thyroglobulin (sTg) under thyroid hormone suppression therapy the presence of serum thyroglobulin antibodies (sAbTg), the diagnostic whole body scan and cytology can give false negative results. Measurement of thyroglobulin in the washout fluid from fine-needle aspiration biopsy (FNAB) of suspicious neck lymph nodes could improve the diagnostic accuracy. To evaluate the usefulness of detecting Tg in lymph nodes (LTg) suspicious by ultrasonography (US) and compare it to cytology. Between the years 2004 and 2007 we prospectively studied 30 patients with PTC and cervical US findings of suspicious recurrence. LTg was assayed in US guided FNAB used for cytology. Sixteen out of 30 patients underwent surgery using as selective criteria an LTg higher than sTg or a positive cytology. Surgery confirmed the presence of metastasis in all 15 patients with positive LTg (8 with positive cytology) and in 1 patient with negative LTg and positive cytology (a case with undifferentiated thyroid cancer). The sensitivity was 93.7% for LTg and 56.2% for cytology. We identified by LTg 3 of 6 patients with undetectable sTg and positive sAbTg. The presence of LTg showed a higher sensitivity than cytology for the detection of cervical lymph node metastasis. This method is useful even in the presence of sAbTg."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 151]], "word_ranges": [[0, 23]], "text": "Currently the molecular classification of breast cancer is based on the study of hormone receptors, HER2 and the tumor cell proliferation index (Ki67)."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Currently the molecular classification of breast cancer is based on the study of hormone receptors, HER2 and the tumor cell proliferation index (Ki67). We have four fundamental types: Luminal (A: HR+, HER2- and low Ki67 and B: HR+, HER2+/ - and high Ki67), HER2 + (HR -, HER2 + and high Ki67) and Basal Like or triple negative (HR -, HER2 - and high Ki67).", "full_answer_no_ref": "Currently the molecular classification of breast cancer is based on the study of hormone receptors, HER2 and the tumor cell proliferation index (Ki67). We have four fundamental types: Luminal (A: HR+, HER2- and low Ki67 and B: HR+, HER2+/ - and high Ki67), HER2 + (HR -, HER2 + and high Ki67) and Basal Like or triple negative (HR -, HER2 - and high Ki67).", "full_question": "A 67-year-old woman diagnosed with an infiltrating ductal carcinoma of the breast with no family history of neoplasia. What additional studies should be performed on the tumor because of its clinical and therapeutic implications:", "id": 406, "lang": "en", "options": {"1": "Complete phenotypic study by flow cytometry.", "2": "Study of hormone receptors and HER2.", "3": "Study of hormone receptors, ecadherin and study of first degree relatives.", "4": "BRCA l-2 study and study of first-degree relatives.", "5": NaN}, "question_id_specific": 33, "type": "ONCOLOGY (ECTOPIC)", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1012_9657", "title": "Clinical features and first degree relative breast cancer, their correlation with histological tumor grade: a 5-year retrospective case study of breast cancer in Mizoram, India.", "score": 0.015277777777777777, "content": "The aim was to assess the association of histological tumor grade with other clinical features and epidemiological factors of women with invasive breast carcinoma. A retrospective study of 103 Mizo breast cancer patients visiting hospitals was made in Aizawl, Mizoram, Northeast India. With a prior consent, information on epidemiological factors and family history in relation to cancer was obtained. Clinical reports were obtained from their medical records. The frequency of distribution was calculated for age at diagnosis and tumor characteristics. Statistical analysis for different variables was done using a chi-square test. p &lt; 0.05 was considered significant. The histological tumor grades in our studies were found to be associated with lymph node invasion (p &lt; 0.021), different subtype of hormone receptor such as ER status (p &lt; 0.004), ER/PR status (p &lt; 0.007), HER2/neu status (p &lt; 0.014), and ER/PR/HER2 status (p &lt; 0.025). A patient with a family history of breast cancer in their 1st degree relative is also seen to have association in determining the tumor grade (p &lt; 0.003). Reproductive history, lifestyle and dietary habits, tobacco, and alcohol consumption were found to have no influence on breast cancer tumor grade. Our results showing significant correlation between status of lymph node, ER, PR, and HER2/neu oncoprotein and family history with 1st degree relative breast cancer are the first time report to target and focus on the possible role of biomarkers for diagnosis among the Mizo tribal breast cancer patients."}, {"id": "pubmed23n0267_6734", "title": "The significance of family history for patients with carcinoma of the breast.", "score": 0.015201976794155567, "content": "Since the risk of carcinoma of the breast is increased in women with a family history of the disease, new primary carcinomas of the breast may be increased after treatment. Women with several relatives with carcinoma of the breast are thought to be at higher risk of having a second primary carcinoma of the breast develop and mastectomy is more frequently recommended. The computerized registry of the Mount Sinai Medical Center Breast Service was used to identify 1,337 patients with complete information concerning family history. Three hundred fifty-nine patients with a family history of carcinoma of the breast were compared with women with no family history. Compared with patients with no family history of carcinoma of the breast, patients with a family history of carcinoma of the breast were significantly younger (54.0 versus 55.8 years of age, p &lt; 0.01), were significantly more likely to have used oral contraceptives (26 versus 13 percent, p &lt; 0.001), had significantly more ductal carcinoma in situ (10 versus 4 percent, p &lt; 0.01), and were significantly more often treated with breast conservation (42 versus 31 percent, p &lt; 0.001). Simultaneous contralateral carcinoma of the breast was diagnosed more frequently in patients with a family history (3 versus 1 percent, p &lt; 0.025), but metachronous contralateral carcinomas were not increased. In comparing the two groups, there were no significant differences in proportion premenopausal, parity, use of postmenopausal hormones, tumor size, tumor differentiation, nodal involvement, TNM stage, estrogen receptor status, or use of adjuvant radiation, chemotherapy, or tamoxifen. Complete five-year follow-up evaluation for 748 patients, 179 with a family history, found no differences in local, distant, or disease-free survival rates for mastectomy or breast conservation in relation to family history. Outcome for patients with first-degree affected relatives and those with more than one affected relative was the same as those with no family history. These results indicate that women with a family history of carcinoma of the breast should be treated no differently than women with no family history."}, {"id": "wiki20220301en304_20305", "title": "BRCA mutation", "score": 0.01424775517683709, "content": "Relative indications for testing for a mutation in BRCA1 or BRCA2 for newly diagnosed or family members include a family history among 1st (FDR), 2nd (SDR), or 3rd(TDR) degree relatives usually on the same side of the family but not limited: A known mutation (BRCA1 or BRCA2) in a cancer susceptibility gene within the family Women affected with any breast cancer diagnosed under the age of 30 Women affected with triple negative breast cancer (TNBC) (estrogen receptor negative, progesterone receptor negative, and HER2/neu negative) under the age of 50 Two relatives (FDR/SDR) diagnosed under the age of 45 Three relatives (FDR/SDR) diagnosed with average age of 50 or less Four relatives at any ages Ovarian cancer with either an additional diagnosed relative or a relative with male breast cancer A single family member with both breast and ovarian cancer Male breast cancer Pancreatic cancer with breast or ovarian cancer in the same individual or on the same side of the family"}, {"id": "pubmed23n0671_14807", "title": "Family history of breast cancer in first-degree relatives and triple-negative breast cancer risk.", "score": 0.013848071956541098, "content": "Triple-negative breast cancer accounts for less than 20% of breast cancers overall, but is the predominant subtype among carriers of mutations in BRCA1. However, few studies have assessed the association between breast cancer family history and risk of triple-negative breast cancer. We examined the relationship between having a family history of breast cancer in first-degree relatives and risk of triple-negative breast cancer, and risk of two other breast cancer subtypes defined by tumor marker expression. We evaluated data collected by the Breast Cancer Surveillance Consortium from 2,599,946 mammograms on 1,054,466 women, among whom 15% reported a first-degree family history of breast cancer. Using Cox regression in this cohort, we evaluated subtype-specific associations between family history and risk of triple-negative (N = 705), estrogen receptor-positive (ER+, N = 10,026), and hormone receptor-negative/HER2-expressing (ER-/PR-/HER2+, N = 308) breast cancer among women aged 40-84 years. First-degree family history was similarly and significantly associated with an increased risk of all the subtypes [hazard ratio (HR) = 1.73, 95% confidence interval (CI): 1.43-2.09, HR = 1.62, 95% CI: 1.54-1.70, and HR = 1.56, 95% CI: 1.15-2.13, for triple-negative, ER+, and ER-/PR-/HER2+, respectively]. Risk of all the subtypes was most pronounced among women with at least two affected first-degree relatives (versus women with no affected first-degree relatives, HR(triple-negative) = 2.66, 95% CI: 1.66-4.27, HR(ER+) = 2.05, 95% CI: 1.79-2.36, HR(ER)-(/PR)-(/HER2+) = 2.25, 95% CI: 0.99-5.08). Having a first-degree family history of breast cancer was associated with an increased risk of triple-negative breast cancer with a magnitude of association similar to that for the predominant ER+ subtype and ER-/PR-/HER2+ breast cancer."}, {"id": "pubmed23n0556_5828", "title": "Influence of young age at diagnosis and family history of breast or ovarian cancer on breast cancer outcomes in a population-based cohort study.", "score": 0.013550021105951878, "content": "The objective of this study was to examine the association of: (i) diagnosis at age &lt;/=35, (ii) first-degree family history of breast or ovarian cancer (BOC) and (iii) a research based definition of genetic risk, with tumor characteristics, treatment and survival in breast cancer (BC). Consenting female participants in the population-based Ontario Familial Breast Cancer Registry diagnosed with primary invasive BC between 1996 and 1998 were followed prospectively until 2005. Among 967 women, 105 were &lt;/=35 years old at diagnosis and 686 were classified as genetic risk cases, including 349 with a first-degree family history. Individuals diagnosed at age &lt;/=35 were more likely to self-detect tumors, to present with inflammatory BC, to have invasive ductal carcinoma of no special type, high T stage, and tumors with lymphovascular invasion (LVI), high grade and negative estrogen receptors. Younger women were more likely to receive chemotherapy and less likely to receive hormonal therapy. Diagnosis &lt;/=35 years old was associated with significantly reduced distant recurrence free survival, an effect that did not persist after adjustment for tumor and treatment related variables. Poor outcomes were restricted to younger women with hormone responsive BC. Family history was associated with increased rates of mammographic detection of BC, lower tumor stage and less frequent inflammatory BC, but had no association with BC outcomes. Women diagnosed with BC at age &lt;/=35 have more aggressive tumors; these adverse tumor characteristics, rather than age, lead to poor outcomes. Family history was not associated with survival."}, {"id": "pubmed23n0289_14463", "title": "Breast cancer and family history: a multivariate analysis of levels of tumor HER2 protein and family history of cancer in women who have breast cancer.", "score": 0.013532763532763533, "content": "The HER2 gene, located on the long arm of chromosome 17, codes for a protein with the characteristics of a growth factor receptor. In a preliminary study, we reported that high levels of tumor HER2 (erbB-2/neu) protein are associated with a family history of breast cancer (that is, one or more female blood relatives with breast cancer). We have now collected a larger number of subjects (94) and performed a multivariate analysis of the independent variables family history of breast cancer, tumor estrogen receptor, age, and tumor DNA index. Family history of breast cancer was assessed by questioning the patient, in many cases by telephone. HER2 levels were significantly higher in women with a family history of breast cancer (p = 0.015, two-tailed t-test). The 27 women with family history were predominantly postmenopausal, mean age 61 +/- 2.3 (mean +/- SEM), versus a mean age of 56 +/- 1.7 for the 67 women with no family history. Of the 27 women with a family history of breast cancer, 13 had a first-degree relative (mother or sister) with the disease. The remaining 14 women had other relatives (grandmothers, aunts, cousins, or a niece) with breast cancer. The results of multiple linear regression analysis, with HER2 as the dependent variable, showed that family history of breast cancer was significantly associated with elevated HER2 levels in the tumors (p = 0.0038), after controlling for the effects of age, tumor estrogen receptor, and DNA index. The association of family history of breast cancer and elevated tumor HER2 protein suggests that postmenopausal familial breast cancer may be associated with altered HER2 expression."}, {"id": "pubmed23n0657_19800", "title": "Four new cases of double heterozygosity for BRCA1 and BRCA2 gene mutations: clinical, pathological, and family characteristics.", "score": 0.013221846939616113, "content": "Double heterozygosity (DH) for BRCA1 and BRCA2 mutations is a very rare finding, particularly in non-Ashkenazi individuals, and only a few cases have been reported to date. In addition, little is known on the pathological features of the tumors that occur in DH cases and on their family history of cancer. Four carriers of pathogenic mutations in both BRCA1 and BRCA2 were identified among women who underwent genetic counseling for hereditary susceptibility to breast and ovarian carcinoma at three different Italian institutions. Clinical, pathological, and family history data were collected from medical records and during genetic counseling sessions. All identified DH cases developed breast carcinoma and three of them were also diagnosed with ovarian carcinoma. Mean ages of breast and ovarian cancer diagnosis were 42.7 and 48.6 years, respectively. The majority of breast cancers showed a BRCA1-related phenotype, being negative for hormone receptors and HER2. Two cases reported different gastrointestinal tumors among relatives. Although the individuals described in this study show more severe clinical features in comparison to previously reported BRCA1 and BRCA2 DH cases, our observations support the hypothesis of a non specific phenotype of DH cases in terms of age of disease onset. In addition, our observations indicate that in DH patients breast carcinogenesis appears to be driven mainly by the mutations in BRCA1. The possible association of DH for BRCA gene mutations with gastrointestinal tumors is in keeping with previous reports, but needs to be confirmed by further analyses."}, {"id": "pubmed23n0227_16419", "title": "The relationship between family history, exposure to exogenous hormones, and estrogen receptor protein in breast cancer.", "score": 0.011887465328226127, "content": "Eight-hundred and thirteen patients were prospectively studied to examine the influence of family history and the prior use of exogenous hormones as covariables in the subsequent expression of estrogen receptor protein (ERP) in the primary tumor of patients with breast cancer. Cases were divided by menstrual status; there were 385 pre- and perimenopausal and 428 postmenopausal patients. The influence of prior exposure to estrogen replacement therapy (ERT) in postmenopausal patients or oral contraceptives (OC) in pre- and perimenopausal patients on tumor ERP was analyzed controlling for family history: none, first degree (1 degree, mother or sister), second degree (2 degrees, grandmother or aunt), or both 1 degree and 2 degrees relatives. The results showed no influence of the prior use of ERT in postmenopausal women on subsequent tumor ERP. Among pre- and perimenopausal women, those with a family history of breast cancer in only a 1 degree relative, showed a borderline significant association between prior OC usage and subsequent tumor ERP. The use of OC was consistently associated with ERP negative tumors (9/9) whereas of 29 patients who had no prior OC exposure 17 had ERP negative tumors (P = 0.04, Fisher's Exact Test). Analysis of the prior exposure to OC, verified with the primary care physician or pharmacist, showed that these patients first used OC at the mean age of 32.2 years, had used OC for a mean duration of 41.9 months and stopped OC use a mean of 79.5 months before being diagnosed as having breast cancer. These results suggest that in a subset of patients with breast cancer, and a first degree relative only who had breast cancer, prior exposure to OC may influence the subsequent ERP status of the tumor. This is not due to exogenous estrogen saturation of receptors as there was a long latent period between exposure and diagnosis. Alternative hypotheses as to the mechanism of selection of subsequent tumor ERP may be either inhibition of ERP positive preneoplastic or tumor cell clones early in the evolution of the tumor or early selection of a tumor capable of endogenous estrogen synthesis with receptor saturation."}, {"id": "pubmed23n0976_164", "title": "BRCA1/BRCA2 mutations in Japanese women with ductal carcinoma in situ.", "score": 0.011616161616161616, "content": "Ductal carcinoma in situ (DCIS) is considered a component of the clinical spectrum of breast cancer even in those with BRCA1/2 mutation. The aim of this study was to report the feature of DCIS raised in Japanese women with BRCA1/2 mutations. A total of 325 Japanese women with breast cancer (BC) (with or without invasive cancer) were referred for genetic counseling and underwent genetic testing for mutations in the BRCA1 and BRCA2 genes in Showa University Hospital between December 2011 and August 2016. And 49 of them who were pathologically diagnosed as DCIS were included in this study. Logistic regression models were fit to determine the associations between potential predictive factors and BRCA status. A Cox proportional hazards model is used to predictive value of parameters for Ipsilateral breast tumor recurrence (IBTR) and contralateral breast tumor recurrence (CBTR). (a) Of 325 patients (with or without invasive cancer), 19.1% (62/325) tested positive for BRCA1/BRCA2 mutations. And 18.4% (9/49) was positive for BRCA1/BRCA2 mutations in DCIS, compared with 19.2% (53/276) in IDC (p = 1.000). Among BRCA mutations, 14.5% (9/62) had DCIS compared with nonmutations (15.2%, 40/263). Incidence of DCIS was 3.0% (1/33) of BRCA1 mutations and 27.5% (8/29) of BRCA2 mutation (p = 0.009). (b) Median age of diagnosis in BRCA mutation carriers was 39 years, compared with 46 years in noncarriers. Age, Family history (FH) of BC, FH of first or second BC and total number of relatives with BC diagnosis (DX) has significant difference between BRCA mutation carriers and noncarriers in univariate analysis. In a multivariate logistic model, total relatives with BC DX ≥ 2 (odds ratio [OR], 5.128; 95% confidence interval [CI], 1.266-20.763; p = 0.022), age at diagnosis ≤35 years (OR 0.149, 95% CI 0.023-0.954, p = 0.045) and ER+/HER2+ status (OR 5.034, 95% CI 1.092-23.210, p = 0.038) remained as independent significant predictors for BRCA mutation. Ki67 index (cut off by 14% or 30%) did not differ between BRCA mutation carriers and noncarriers (p = 0.459 and p = 0.651). (c) There was a significant difference in ER-positive tumors among BRCA2 carriers and noncarriers (p = 0.042). Subgroup analysis showed BRCA2 carriers tend to be of higher grade (Grade 2 and 3), more frequently ER+/PR+ (p = 0.041) and lower proliferation (Ki67 index) than noncarriers, whereas differences in nuclear grade and ki67 index were not found significantly in our study. (d) BRCA mutation was not associated with an increased risk of IBTR and CBTR. DCIS is equally as prevalent in patients who were BRCA mutation carriers as in high familial-risk women who were noncarriers, but occurs at earlier age. BRCA2 carriers have higher incidence in DCIS than that of BRCA1 carriers, and tend to be higher grade and more frequently ER positive and lower proliferation. Total relatives with BC DX ≥2, age at diagnosis ≤35 years and ER+/HER2+ might be independent predictors for BRCA mutation in Japanese women with DCIS and patients of these risk factors should be recommended to receive genetic counseling and BRCA testing."}, {"id": "wiki20220301en188_32824", "title": "Somatostatin receptor 2", "score": 0.009900990099009901, "content": "other components of the endocrine system and nervous system, so it can be drawn that the receptor family has great influence among these systems. The family was first discovered in a segment of a rat's pituitary gland known as the tumor cell line. A cell line is grown as a culture under controlled conditions, so the first discovery was found by culturing these cells in controlled conditions and in an environment outside of its norm. There, researchers found that the tumor cell line expresses a cell dividing inhibitor known as the transforming growth factor beta (TGF-beta) and also acts as an inhibitor to the milk producing hormone in female mammals, prolactin, and growth hormones. Researchers studied the activity of the receptors by conducting an assay with Ligand binding studies, which basically means they were conducting studies to see how prevalent the binding of the receptors occurred. Differences in how prevalently they receptors bonded revealed the existence of multiple"}, {"id": "pubmed23n0396_8364", "title": "Does family history influence survival in breast cancer cases?", "score": 0.009900990099009901, "content": "A few studies have suggested a relatively better prognosis for breast cancer (BC) cases reporting a positive family history (FH). We aimed at comparing the survival of patients according to FH in a large hospital-based series of 1,278 BC cases. Information on FH for BC was obtained at diagnosis by interview. All cases reporting a first- or second-degree FH for breast carcinoma were compared with cases without FH. Overall survival was estimated using a product-limit method. Hazard ratios (HRs) and the corresponding 95% confidence intervals (95% CIs), adjusted for confounding factors, were computed using proportional hazard models. Overall, 240 (18.8%) cases reporting, at diagnosis, a positive FH (156 with at least 1 first-degree relative and 84 with at least 1 second-degree relative) were compared with 1,038 patients without FH for BC. No significant differences were found in terms of distribution of age at diagnosis, tumor stage, nodal involvement, receptor status and histology. Cumulative survival rates at 5 years for cases without FH and with first-degree and second-degree FH for BC were 79.8 (95% CI 77.0-83.0), 78.6 (95% CI 70.0-88.0) and 80.2 (95% CI 68.0-92.0), respectively (log-rank test, chi(2) (2) = 0.02, p = 1.0). After adjustment for age, pathologic size and nodal involvement, the HR among cases of invasive cancer with a first-degree FH of BC was 0.91 (95% CI 0.55-1.48); however, the HR for cases with second-degree FH was 1.18 (95% CI 0.62-2.25) compared to cases without FH. Our study, based on a large series of consecutive invasive BC cases, did not find any significant survival differences associated with a positive FH for breast carcinoma, suggesting the existence of a large heterogeneity among BC cases with FH."}, {"id": "wiki20220301en301_10160", "title": "Combined small-cell lung carcinoma", "score": 0.00980392156862745, "content": "Pemetrexed has been shown to improve survival in non-squamous cell NSCLC, and is the first drug to reveal differential survival benefit in large cell lung carcinoma. C-SCLC appear to express female hormone (i.e. estrogen and/or progesterone) receptors in a high (50–67%) proportion of cases, similar to breast carcinomas. However, it is at present unknown whether blockade of these receptors affects the growth of c-SCLC. Prognosis Current consensus is that the long-term prognosis of c-SCLC patients is determined by the SCLC component of their tumor, given that \"pure\" SCLC seems to have the worst long-term prognosis of all forms of lung cancer. Although data on c-SCLC is very sparse, some studies suggest that survival rates in c-SCLC may be even worse than that of pure SCLC, likely due to the lower rate of complete response to chemoradiation in c-SCLC, although not all studies have shown a significant difference in survival."}, {"id": "pubmed23n0324_15364", "title": "Clinical characteristics of breast cancer patients with family history.", "score": 0.00980392156862745, "content": "This study was conducted to acquire information as to the clinicopathological characteristics of breast cancer patients with family history. Of 583 patients with breast cancer, 60 (10.3%) had family history in at least one relative within the second-degree. The affected family member was most frequently a sister (43%), followed by the mother (23%) and an aunt (20%). Comparison of the data for the patients between with and without family history revealed no significant differences for any of mean age, menopausal status, histological type, histological staging, and estrogen receptor status. Although the sample size was small, neither the survival rate nor the bilaterality of disease was influenced by the family history of breast cancer."}, {"id": "pubmed23n0806_4708", "title": "Risk of breast cancer and family history of other cancers in first-degree relatives in Chinese women: a case control study.", "score": 0.009769174845510722, "content": "Few studies have systematically reported the relationship between the risk of breast cancer and family history of other cancers. This study was designed to systematically determine the relationship between breast cancer risk and family history of other cancers in first-degree relatives. Between January 2006 and June 2011, 823 women diagnosed with breast cancer were included, and age-matched women diagnosed with benign breast disease were selected as controls. Family history of other cancers in first-degree relatives was recorded by trained reviewers. Multivariate logistic regression was applied to analyze the relationships. A family history of esophagus cancer (OR: 2.70, 95% CI: 1.11 - 6.57), lung cancer (OR: 2.49 95% CI: 1.10 - 5.65), digestive system cancer (OR: 1.79, 95% CI: 1.14 - 2.79) and any cancer (OR: 2.13, 95% CI: 1.49 - 3.04) in first-degree relatives was directly associated with increased breast cancer risk. In subgroup analysis, the risk of hormone receptor positive breast cancer was increased in subjects with a family history of lung cancer (OR: 3.37, 95% CI: 1.45 - 7.82), while the risk of hormone receptor negative breast cancer was increased in subjects with a family history of esophagus cancer (OR: 6.19, 95% CI: 2.30 - 16.71), uterus cancer (OR: 6.92, 95% CI: 1.12 - 42.89), digestive tract cancer (OR: 2.05, 95% CI: 1.03 - 4.10) and gynecology cancer (OR: 6.79, 95% CI: 1.46 - 31.65). Additionally, a significant increase in breast cancer was observed with a family history of digestive system cancer for subjects 50 y and younger (OR: 1.88, 95% CI: 1.03 - 3.43), not for subjects 50 y older (OR: 1.67, 95% CI: 0.86 - 3.25). Breast cancer aggregates in families with several types of cancer especially for digestive system cancer. The influence of a family history of other cancers seems more likely to be limited to hormone receptor negative breast cancer."}, {"id": "wiki20220301en034_42642", "title": "Selective estrogen receptor modulator", "score": 0.009708737864077669, "content": "Tamoxifen is a first-line hormonal treatment of ER-positive metastatic breast cancer. It is used for breast cancer risk reduction in women at high risk, and as adjuvant treatment of axillary node-negative and node-positive, ductal carcinoma in situ. Tamoxifen treatment is also useful in the treatment of bone density and blood lipids in postmenopausal women. Adverse effects include hot flushes and more serious is two to three times higher relative risk of developing endometrial cancer compared to women of an age-matched population. Toremifene, a chlorinated tamoxifen derivative, causes fewer DNA adducts in liver than seen with tamoxifen in preclinical studies and was developed to avoid hepatic carcinomas. It is used as endocrine therapy in women with ER/PR-positive stage 4 or recurrent metastatic breast cancer and has demonstrated similar efficacy compared to tamoxifen as adjuvant treatment of breast cancer and in the treatment of metastatic breast cancer."}, {"id": "pubmed23n0494_12555", "title": "Prognosis of breast cancer patients with familial history classified according to their menopausal status.", "score": 0.009708737864077669, "content": "Breast cancer patients were classified in the family history positive (FHP) group when they had at least one second-degree relative who was a breast cancer patient. The results of a comparative study with patients classified in the family history negative (FHN) group showed the prognosis of the FHP group was significantly better than that of the FHN group. However, when those patients were classified according to their menopausal status at onset, there were no significant differences in survival rates between the FHP and FHN groups with onset before menopause, whereas the survival rate of the FHP group was significantly higher than that of the FHN group with onset after menopause. The same results were found when the FHP group was subgrouped into the FHP group with first-degree relatives and the FHP group with second-degree relatives. Further investigations on background factors revealed that the patients with onset before menopause showed no significant differences between the FHP and FHN groups in age at surgery, diameter of the tumor, histologic grade, the number of metastatic lymph nodes, body weight, estrogen receptor (ER) status, and the values of CEA and CA15-3 before surgery. On the other hand, the FHP patients with onset after menopause showed significantly lower numbers of metastatic lymph nodes and trends showing higher ER values and lower CA15-3-values. Therefore the favorable prognosis in the FHP group seems to be attributable to the higher survival rate of the FHP patients with onset after menopause."}, {"id": "wiki20220301en191_9178", "title": "Metaplastic carcinoma", "score": 0.009615384615384616, "content": "Metaplastic carcinoma, otherwise known as metaplastic carcinoma of the breast (MCB), is a heterogeneous group of cancers that exhibit varied patterns of metaplasia and differentiation along multiple cell lines. This rare and aggressive form of breast cancer is characterized as being composed of a mixed group of neoplasms containing both glandular and non-glandular patterns with epithelial and/or mesenchymal components. It accounts for fewer than 1% of all breast cancer diagnoses. It is most closely associated with invasive ductal carcinoma of no special type. (IDC), and shares similar treatment approaches. Relative to IDC, MCB generally has higher histological grade and larger tumor size at time of diagnosis, with a lower incidence of axillary lymph node involvement. MCB tumors are typically estrogen receptor (ER), progesterone receptor (PR), and human epidermal growth factor-2 (HER-2) negative, meaning hormone therapy is generally not an effective treatment option, which correlates to"}, {"id": "wiki20220301en490_9660", "title": "Abemaciclib", "score": 0.009523809523809525, "content": "As of early 2016, abemaciclib was involved in 3 Phase III clinical trials: The JUNIPER Study is comparing abemaciclib against erlotinib in patients with stage IV non-small-cell lung carcinoma Due to collect data until September 2017. The MONARCH 2 study is investigating the effectiveness of abemaciclib in combination with fulvestrant for women with breast cancer. It is due to end in Feb 2017. In March 2017, Eli Lilly announced that it had met its primary endpoint of superior progression-free survival (PFS) over placebo plus fulvestrant in patients with estrogen receptor positive and HER2 negative advanced or metastatic breast cancer. This result led to the September 2017 FDA approval. The MONARCH 3 study is investigating the effectiveness of abemaciclib, plus either anastrozole or letrozole, as a first-line treatment for women with breast cancer. The trial is expected to end in June 2017."}, {"id": "pubmed23n0933_10246", "title": "Family History and Risk of Second Primary Breast Cancer after <i>In Situ</i> Breast Carcinoma.", "score": 0.009523809523809525, "content": "<bBackground:</b Incidence rates of <iin situ</i breast carcinomas have increased due to widespread adoption of mammography. Very little is known about why some women with <iin situ</i breast cancer later develop second primary breast cancers.<bMethods:</b In this population-based nested case-control study among <iin situ</i breast cancer survivors, including 539 cases with a second primary breast cancer and 994 matched controls, we evaluated the association between first-degree family history of breast cancer and risk of developing a second primary breast cancer.<bResults:</b First-degree family history of breast cancer was associated with an increased risk of developing a second primary breast cancer among women with a previous <iin situ</i breast cancer [odds ratio (OR) = 1.33, 95% confidence interval (CI), 1.05-1.69] and those with two or more affected first-degree relatives had an even higher risk (OR = 1.94; 95% CI, 1.15-3.28). Those whose relative was diagnosed at less than 50 years old were more likely to develop a second primary breast cancer (OR = 1.78; 95% CI, 1.24-2.57). No difference in risks associated with number or age of affected relatives was observed by menopausal status.<bConclusions:</b Results from this study suggest that first-degree family history of breast cancer may be an important risk factor for development of a second primary breast cancer among women with a previous <iin situ</i breast cancer.<bImpact:</b Given the growing population of <iin situ</i breast cancer survivors, a better understanding of risk factors associated with development of a second primary breast cancer is needed to further understand risk. <iCancer Epidemiol Biomarkers Prev; 27(3); 315-20. ©2018 AACR</i."}, {"id": "wiki20220301en406_12297", "title": "Dynamic angiothermography", "score": 0.009433962264150943, "content": "DATG is able to detect changes in blood flow that are indicative of breast cancer, may be used for younger patients, is completely non-invasive (no need for radiation or contrast agent, no need for compression of the breast) and is lower cost than alternatives requiring minimal facilities. This technology, performed quickly (5–6 minutes for visit) and very precise, is useful for screening and is also able to detect precancerous lesions. Studies have been conducted that have shown how it is possible, by means of this methodology, to diagnose invasive ductal carcinoma and infiltrating lobular carcinoma with the same accuracy. DATG can be strategic for young patients, or patients with dense breasts where the contrastive performance of mammography is challenged. Another application of DATG is the monitoring of at-risk patients with increased changes of breast cancer who take hormone replacement therapy (sometimes taken to reduce menopause symptoms) and participate in in-vitro"}, {"id": "pubmed23n0759_12700", "title": "Retrospective analysis of clinicopathological characteristics and family history data of early-onset breast cancer: a single-institutional study of Hungarian patients.", "score": 0.009433962264150943, "content": "Patients at young age (≤ 35 years) diagnosed with breast cancer (BC) are considered to have poor prognosis. The aim of the present study was to retrospectively analyse clinicopathological characteristics and prognosis in a group of young BC patients. We included women diagnosed with invasive breast carcinoma younger than/or at the age of 35 years. Between 1999 and 2009, 107 women with early-onset BC were selected from the database of the 2nd Department of Pathology at Semmelweis University. For clinicopathological comparison, 55 women (36-45 years), 214 women (46-65 years), 110 women (66-75 years) and 58 women (76 ≤ years) were also included in the analysis. Family history, clinicopathological and follow-up data were analysed. The tissue specimens were reviewed for histological type, nuclear grade, and estrogen receptor (ER), progesterone receptor (PgR), Ki67 and HER2 status (IHC4). The mean age in the study group was 31.6 years at the time of diagnosis. Histology showed a high incidence of grade III tumours in this group of patients (67.9 %), while only four cases (3.8 %) were considered grade I. According to the immunohistochemical results, 35.3 % of the study cases were considered as Luminal B (LumB: either being higly proliferative or co-expressing HER2) and 33.3 % as triple negative breast carcinomas (TNBC). The detailed questionnaire related to family history was completed and received in 49/107 cases (45.8 %). Analysis of these data revealed an affected family history of breast or ovarian carcinoma in first and second degree relatives in 51.0 %. A high proportion (52.0 %) of TNBC was observed among young women with a family history of the disease. Survival analysis of the 107 patients showed that 25 (23.3 %) women died until 31 December 2012. No significant difference in survival was detectable considering the regimen of systemic treatment (p = 0.188). Regarding clinicopathological parameters, the immunophenotypes, grade, pT and pN values differred substantially between the age groups (p = 0.001, for all), and the shortest relapse-free survival was seen among the youngest BC patients. This analysis illustrates that breast cancer arising in young women is characterized by the presence of less favorable subtypes such as LumB and TNBC. The increased proportion of TNBC was especially remarquable in the group of patients presenting with family history of the disease. The fact that a high rate of death occurred and no significant difference in OS were notable regarding the scheme of systemic therapies (neoadjuvant vs. adjuvant) highlight the necessity of the development of new treatment strategies."}, {"id": "pubmed23n0263_11157", "title": "A clinicopathological analysis of breast cancer in patients with a family history.", "score": 0.009345794392523364, "content": "A study was conducted to investigate the clinical and pathological characteristics of breast cancer in patients with a family history (FH). Among 4,481 primary breast cancer patients, 394 (8.8%) had families which included two or more breast cancer patients within three generations (FH(+)group). This group was compared with the remaining 3,969 patients (FH(-) group) with the following results: (1) The tumor diameter in the FH(+) group was slightly less than that in the FH(-) group [not significant (NS)], with fewer lymph node metastases (P &lt; 0.05); (2) the positive rates for the estrogen receptor were 52% (138/266) and 49% (1,216/2,481), respectively (NS); (3) expression of the c-erbB-2 protein was observed in 14 out of 40 (35%) and 32 out of 100 cases (32%), respectively (NS); (4) the relative risk of bilateral occurrence in the FH(+) group was 1.4, with a 95% confidence interval of 0.9-2.4; (5) the 15-year survival rate was 72% and 60%, respectively, suggesting a better prognosis for the FH(+) group (P &lt; 0.01); and (6) multivariate analysis showed that the contribution of FH to postoperative survival was marginal (P = 0.07). Factors related to the hormonal environment such as age at menarche (P = 0.08) and age at menopause (P = 0.08) made a greater but non-significant contribution to the prognosis of the FH(+) group than to that of the FH(-) group. However, further genetic and molecular biological analyses of familial breast cancer are needed in order to clarify the mechanisms of cancer accumulation within families."}, {"id": "wiki20220301en188_32364", "title": "Parathyroid hormone 1 receptor", "score": 0.009259259259259259, "content": "Interactions Parathyroid hormone 1 receptor has been shown to interact with Sodium-hydrogen exchange regulatory cofactor 2 and Sodium-hydrogen antiporter 3 regulator 1. Model organisms Model organisms have been used in the study of PTH1R function. A conditional knockout mouse line called Pth1rtm1a(EUCOMM)Hmgu was generated at the Wellcome Trust Sanger Institute. Male and female animals underwent a standardized phenotypic screen to determine the effects of deletion. Additional screens performed: - In-depth immunological phenotyping See also Parathyroid hormone receptor References Further reading External links G protein-coupled receptors"}, {"id": "wiki20220301en470_13910", "title": "Eftilagimod alpha", "score": 0.009174311926605505, "content": "Clinical Trials Ongoing Clinical Studies As of February 2020, three clinical studies are ongoing: Metastatic breast carcinoma (HER2− HR+) In the AIPAC study efti is administered in combination with paclitaxel to women with HER2− metastatic breast cancer whose disease progressed after endocrine therapy. This Phase IIb trial is a randomized, double-blind, placebo-controlled study aiming to enroll 241 patients. It had an open run-in phase with 15 patients being treated and the results were published at the 2018 ASCO annual meeting. The study is ongoing and is expected to show results in the first half of 2020. Solid Tumors The INSIGHT Phase I study is investigating the feasibility and safety of different routes of drug delivery (e.g. intra-tumoral, intra-peritoneal, and subcutaneous)."}, {"id": "pubmed23n0673_14736", "title": "Risk of second breast cancer according to estrogen receptor status and family history.", "score": 0.009174311926605505, "content": "A recent study reported an increased risk of contralateral estrogen-negative breast cancer after a first primary estrogen-negative breast cancer. Our study aims to confirm this result and to evaluate how the risk of second breast cancer occurrence is affected by family history of breast cancer and anti-estrogen treatment. We included all 4,152 women diagnosed with breast cancer between 1995 and 2007, using data from the population-based Geneva Cancer Registry. We compared the incidence of second breast cancer among patients according to estrogen receptor (ER) status with that expected in the general population by age-period Standardized Incidence Ratios (SIRs). Among the cohort, 63 women developed second breast cancer. Patients with ER-positive first tumors had a decreased risk of second breast cancer occurrence (SIR: 0.67, 95% CI: 0.48-0.90), whereas patients with ER-negative primary tumors had an increased risk (SIR: 1.98, 95% CI: 1.19-3.09) limited to ER-negative second tumors (SIR: 7.94, 95% CI: 3.81-14.60). Patients with positive family history had a tenfold (SIR: 9.74, 95% CI: 3.57-21.12) higher risk of ER-negative second tumor which increased to nearly 50-fold (SIR: 46.18, 95% CI: 12.58-118.22) when the first tumor was ER-negative. Treatment with anti-estrogen decreased the risk of second ER-positive tumors but not ER-negative tumors. The risk of second ER-negative breast cancer is very high after a first ER-negative tumor, in particular among women with strong family history. Surveillance and prevention of second cancer occurrence should consider both ER status of the first tumor and family history."}, {"id": "wiki20220301en185_5662", "title": "Risk factors for breast cancer", "score": 0.00909090909090909, "content": "Hormonal contraceptives may produce a slight increase in the risk of breast cancer diagnosis among current and recent users, but this appears to be a short-term effect. In 1996 the largest collaborative reanalysis of individual data on over 150,000 women in 54 studies of breast cancer found a relative risk (RR) of 1.24 of breast cancer diagnosis among current combined oral contraceptive pill users; 10 or more years after stopping, no difference was seen. Further, the cancers diagnosed in women who had ever used hormonal contraceptives were less advanced than those in nonusers, raising the possibility that the small excess among users was due to increased detection. The relative risk of breast cancer diagnosis associated with current and recent use of hormonal contraceptives did not appear to vary with family history of breast cancer. Some studies have suggested that women who began using hormonal contraceptives before the age of 20 or before their first full-term pregnancy are at"}, {"id": "pubmed23n0597_20324", "title": "Population-based estimates of the relation between breast cancer risk, tumor subtype, and family history.", "score": 0.00909090909090909, "content": "Many studies that have estimated the breast cancer risk attributable to family history have been based on data collected within family units. Use of this study design has likely overestimated risks for the general population. We provide population-based estimates of breast cancer risk and different tumor subtypes in relation to the degree, number, and age at diagnosis of affected relatives. Cox Proportional Hazards to calculate risks (hazard ratios; 95% confidence interval) of breast cancer and tumor subtypes for women with a family history of breast cancer relative to women without a family history among a cohort of 75,189 women age &gt;or=40 years of whom 1,087 were diagnosed with breast cancer from June 1, 2001-December 31, 2005 (median follow-up 3.16 years). Breast cancer risk was highest for women with a first-degree family history (1.54; 1.34-1.77); and did not differ substantially by the affected relative's age at diagnosis or by number of affected first-degree relatives. A second-degree family history only was not associated with a significantly increased breast cancer risk (1.15; 0.98-1.35). There was a suggestion that a positive family history was associated with risk of triple positive (Estrogen+/Progesterone+/HER2+) and HER2-overexpressing tumors. While a family history of breast cancer in first-degree relatives is an important risk factor for breast cancer, gathering information such as the age at diagnosis of affected relatives or information on second-degree relative history may be unnecessary in assessing personal breast cancer risk among women age &gt;or=40 years."}, {"id": "wiki20220301en514_11828", "title": "Tesevatinib", "score": 0.009009009009009009, "content": "Tesevatinib (KD019, XL647) is an experimental drug proposed for use in kidney cancer and polycystic kidney disease. The drug was first developed by Exelixis, Inc. and was later acquired by Kadmon Corporation. Tesevatinib binds to and inhibits several tyrosine receptor kinases that play major roles in tumor cell proliferation and tumor vascularization, including epidermal growth factor receptor (EGFR; ERBB1), epidermal growth factor receptor 2 (HER2; ERBB2), vascular endothelial growth factor receptor (VEGFR), and ephrin B4 (EphB4). The drug activity was initially studied in non-small cell lung cancer. In a 2007 pre-clinical study with xenograft tumors of an erlotinib-resistant cell line tesevatinib substantially inhibited the growth of these tumors. In polycystic kidney disease, a histological study of the drug effects and toxicity in rats and mice was published in July 2017."}, {"id": "pubmed23n0895_25005", "title": "Family history of cancer other than breast or ovarian cancer in first-degree relatives is associated with poor breast cancer prognosis.", "score": 0.009009009009009009, "content": "Whether a first-degree family history of others cancers (FHOC) than breast or ovarian cancer (BOC) is associated with breast cancer prognosis remains unknown. Thus, the aim of the present study was to clarify this issue. Women who were diagnosed with invasive breast cancer at the Renmin Hospital of Wuhan University from 2010 to 2013 were included in the study. The demographic and clinicopathological characteristics of these patients were extracted. FHOC was considered positive for any patient who had a relative who had been diagnosed with cancer other than BOC. Disease-free survival (DFS) was calculated based on the date of diagnosis. DFS was analyzed using the Cox proportional hazards model. A total of 434 breast cancer patients were included in this study. Among these patients, 61 (14.06%) had a positive FHOC in first-degree relatives. Patients with a positive FHOC tended to have HER2-positive breast cancer (p = 0.03). In the survival analysis, FHOC was associated with poor DFS in both univariate (HR = 2.21 (1.28-3.83), 95% CI: 1.28-3.83, p &lt; 0.01) and multivariate (HR = 2.50, 95% CI: 1.24-5.04, p = 0.01) analyses, especially in patients with luminal A subtypes. The results demonstrated an increased risk of recurrence in breast cancer patients with FHOC, especially in patients with luminal A subtype."}, {"id": "wiki20220301en260_31508", "title": "Atypical ductal hyperplasia", "score": 0.008928571428571428, "content": "Cancer risk based on follow-up The relative risk of breast cancer based on a median follow-up of 8 years, in a case control study of US registered nurses, is 3.7. See also Ductal carcinoma in situ Breast cancer Collagenous spherulosis References External links What is atypical ductal hyperplasia? (hopkinsmedicine.org) Benign neoplasms Breast neoplasia"}, {"id": "pubmed23n0570_12337", "title": "Familial breast cancer. Part II: Relationships with histology, staging, steroid receptors and serum tumor markers.", "score": 0.008928571428571428, "content": "To identify differences in clinical characteristics, histological features, hormone receptor status, and tumor marker expression between patients with sporadic and familial breast cancer. As in the previous Part I of this study, two groups of women with breast cancer were compared. The first group (group I) included 504 patients with a family history of breast cancer. The second (control) group (group II) consisted of 300 patients not reporting such a history in their relatives. The examined parameters in this report were stage and axillary lymph node involvement at the time of the initial diagnosis, treatment methods, hormone receptor status, and serum levels of the tumor markers CEA and CA 15.3. The data were processed and analysed using the SPSS statistical package. The statistical significance of differences between groups and subgroups was evaluated by x(2) Pearson's test and Student's paired t-test. Compared to sporadic cases, patients with familial breast cancer were more often diagnosed at an advanced III or IV stage; metastatic involvement of the regional lymph nodes was more frequent in group I patients. In the same group more radical surgical procedures combined with chemotherapy and local irradiation were performed. In group I the percentage of negative hormone receptors was higher (35.3% versus 22.6%; p &lt;0.0001) for estrogen receptors (ER), and 47.6% versus 32.6% (p &lt;0.0001) for progesterone receptors (PR). Also, in group I raised serum levels of CA 15.3 were significantly more frequent compared with group II (48% versus 35.5%, p &lt;0.0789), and this applied also for CEA values above 50 ng/ml (10.6% versus 1.5%, p &lt;0.0002). Familial breast cancer displays particular clinical characteristics, distinguishing it from the sporadic type of the disease. Patients with familial breast cancer are usually diagnosed at an advanced stage. Commonly, the hormone receptors are negative and the serum concentrations of tumor markers elevated. The steroid receptor status represents the most reliable predictor of response to hormonotherapy and an important prognostic factor of the patient's outcome. As a result of their particular characteristics, these patients require more radical surgical techniques combined with pre- or postoperative local radiotherapy and systemic chemotherapy."}, {"id": "pubmed23n0407_7924", "title": "HER-2/neu status and tumor morphology of invasive breast carcinomas in Ashkenazi women with known BRCA1 mutation status in the Ontario Familial Breast Cancer Registry.", "score": 0.008865841418534626, "content": "The prevalence of BRCA1 germline mutations is greater in the Ashkenazi Jewish population than in the general North American population. The Ontario Familial Breast Cancer Registry collects clinical and family history data in familial breast carcinoma cases, and unselected Ashkenazi breast carcinomas, and acts as a tumor tissue repository. Using this resource, we examined the tumor morphology, hormone receptor status, and HER-2/neu protein overexpression in Canadian Ashkenazi breast carcinoma patients whose germline BRCA1 mutation status is known. Thirty-eight tumors from 32 BRCA1 carriers and 354 tumors from 334 noncarriers were analyzed. The tumors in BRCA1 mutation carriers were more likely to be high grade (P &lt; 0.0001) and estrogen receptor negative (P &lt; 0.004). There was an increased frequency of typical medullary carcinomas in mutation carriers when all tumors were analyzed. However, this difference did not remain statistically significant when only the first tumor diagnosed in each patient was included in the analysis. There was no difference in HER-2/neu protein overexpression between the two groups overall (P = 0.07). However, when the analysis was restricted to Grade III tumors, there were significantly fewer HER-2/neu-positive tumors in the mutation carriers versus noncarriers (3.1% vs. 21.5%, P = 0.012). No significant differences were found in the incidence of lymph node status, progesterone receptor status, lymphatic vessel invasion, degree of lymphocytic infiltration, or in the presence of ductal carcinoma in situ associated with the invasive tumors. Increasing awareness of the morphologic and immunophenotypic features more commonly found in BRCA1-associated breast carcinomas may lead to a wider use of these characteristics in genetic screening programs and provide further clues to their pathogenesis."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 455]], "word_ranges": [[0, 80]], "text": "The NNT (number needed to treat), means how many patients we would need to treat with an intervention to obtain a benefit; in this question how many patients do we need to treat with warfarin to prevent a stroke. To calculate this we use the following formula: NNT= 1/RRA (RRA: absolute risk reduction). RRA= Io - Ie (Io: incidence in those not exposed to warfarin; Ie: incidence in those exposed to warfarin). RRA: 5,2%- 2,2%= 3= 0,03. NNT: 1/0.03= 33.3."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The NNT (number needed to treat), means how many patients we would need to treat with an intervention to obtain a benefit; in this question how many patients do we need to treat with warfarin to prevent a stroke. To calculate this we use the following formula: NNT= 1/RRA (RRA: absolute risk reduction). RRA= Io - Ie (Io: incidence in those not exposed to warfarin; Ie: incidence in those exposed to warfarin). RRA: 5,2%- 2,2%= 3= 0,03. NNT: 1/0.03= 33.3.", "full_answer_no_ref": "The NNT (number needed to treat), means how many patients we would need to treat with an intervention to obtain a benefit; in this question how many patients do we need to treat with warfarin to prevent a stroke. To calculate this we use the following formula: NNT= 1/RRA (RRA: absolute risk reduction). RRA= Io - Ie (Io: incidence in those not exposed to warfarin; Ie: incidence in those exposed to warfarin). RRA: 5,2%- 2,2%= [HIDDEN]. NNT: 1/0.03= [HIDDEN].", "full_question": "An 86-year-old woman in whom nonvalvular atrial fibrillation has been detected. She has a CHADS2 score of 3 points. In the literature, similar patients on warfarin therapy have a stroke risk of 2.2% versus 5.2% in patients without warfarin. What would be the number needed to treat (NNT) to prevent embolic stroke with anticoagulation therapy?", "id": 526, "lang": "en", "options": {"1": "3", "2": "19,2.", "3": "33,3.", "4": "49,5.", "5": NaN}, "question_id_specific": 54, "type": "PREVENTIVE MEDICINE", "year": 2021, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0803_19632", "title": "Medical Costs of Oral Anticoagulants vs Warfarin for Atrial Fibrillation Patients with Different Stroke Risks.", "score": 0.018978233495232956, "content": "The Apixaban for the Reduction in Stroke and Other Thromboembolic Events in Atrial Fibrillation (ARISTOTLE), Randomized Evaluation of Long-term Anticoagulation Therapy (RE-LY), and Rivaroxaban Once Daily Oral Direct Factor Xa Inhibition Compared with Vitamin K Antagonism for Prevention of Stroke and Embolism Trial in Atrial Fibrillation (ROCKET-AF) trials demonstrated that the oral anticoagulants (OACs), apixaban, dabigatran, and rivaroxaban, respectively, are efficacious for stroke prevention among nonvalvular atrial fibrillation (NVAF) patients. Based on clinical trial results this study evaluated medical costs of clinical events associated with use of individual OACs relative to those of warfarin in NVAF patients with moderate and high stroke risk. Rates for primary and secondary efficacy and safety outcomes (i.e., clinical events) among NVAF patients with CHADS2 = 2 and ≥3 were determined from the three OAC trials. One-year incremental costs among patients with clinical events from a US payer perspective were obtained from the literature and inflation adjusted to 2010 costs. Medical costs for clinical events associated with each OAC vs. warfarin were estimated and compared. For NVAF patients with moderate stroke risk (CHADS2 = 2) differences in clinical event medical costs vs. warfarin were -$298, -$143, and +$117 per patient year for apixaban, dabigatran (150 mg), and rivaroxaban, respectively (negative numbers indicate cost reduction). For NVAF patients with high stroke risk (CHADS2 ≥ 3) differences in clinical event medical costs vs. warfarin were -$697, +$2, and -$100 for apixaban, dabigatran (150 mg), and rivaroxaban, respectively. Medical cost differences associated with OACs vs. warfarin vary according to stroke risk. Of the three OACs, apixaban demonstrated consistent medical cost reductions vs. warfarin for NVAF patients with moderate and high stroke risks."}, {"id": "pubmed23n0814_6008", "title": "Oral anticoagulation in atrial fibrillation.", "score": 0.01672194582642344, "content": "Atrial fibrillation affects approximately 5 million patients in the United States. The rate of stroke in adults with atrial fibrillation depending on their risk factors varies between 1-20% annually. Anticoagulation with vitamin K antagonists such as warfarin has been the mainstay therapy but it is cumbersome and requires close follow-up. Since 2010, three new oral anticoagulants have received Food and Drug Administration approval for stroke prevention in atrial fibrillation. This review summarizes data from three landmark trials: RE-LY, ROCKET-AF, and ARISTOTLE. In addition, issues relating to cost, reversal, drug interactions, and perioperative discontinuation are discussed. Compared to Warfarin, Dabigatran 150 mg twice daily lowered the primary outcome of stroke/systemic embolism by 34% (number needed to treat/yr 169) and had similar incidence of major bleeding. Rivaroxaban demonstrated non inferiority compared to the warfarin group for the primary outcome of stroke and systemic embolism and major bleeding. Apixaban showed a relative risk reduction for the primary outcome of 21% (number needed to treat 300), and lowered major bleeding down by 31% (number needed to treat /yr 104). Apixaban also showed a mortality benefit compared to warfarin (3.52 vs. 3.94%/year, p 0.047). All 3 oral anticoagulants lowered rates of intracranial hemorrhage. The use of Rivaroxaban and Apixaban has been projected to reduce medical costs when compared to warfarin, and Dabigatran is projected to have similar costs. All the 3 oral anticoagulants have robust randomized controlled trials supporting their comparability to warfarin therapy for stroke prevention in non valvular atrial fibrillation, with Apixaban showing superiority in incidence of strokes, major bleeding and mortality."}, {"id": "pubmed23n0381_14811", "title": "Secondary prevention of stroke in patients with nonvalvular atrial fibrillation: optimal intensity of anticoagulation.", "score": 0.01453833066969119, "content": "Nonvalvular atrial fibrillation (NVAF) is frequently seen in elderly people and has become a main cause of cardioembolic stroke. The efficacy of anticoagulation for primary prevention of stroke or transient ischaemic attacks (TIAs) in patients with NVAF has been established by prospective, randomised and controlled trials. Warfarin decreased the frequency of all strokes by 68% and the rate of the combined outcome of stroke, systemic embolism or death by 48%. Anticoagulation with warfarin using international normalised ratios (INRs) ranging from 2.0 to 3.0 is recommended for patients with NVAF, who have any of the risk factors identified by the Atrial Fibrillation Investigators (AFI) [previous stroke or TIA, history of hypertension, diabetes mellitus, advanced age (&gt; or = 65 years old), congestive heart failure and coronary artery disease], the American College of Chest Physicians (ACCP) [increased age (&gt; 75 years old), prior stroke, hypertension and heart failure], or the Stroke Prevention in Atrial Fibrillation (SPAF) investigators [women &gt; 75 years old, prior stroke, systolic blood pressure &gt; 160mm Hg, recent heart failure, and fractional shortening &lt; 25% on echocardiography]. For the secondary prevention of stroke, the efficacy of adjusted-dose warfarin therapy has been demonstrated by 2 major randomised trials. SPAF III (INR 2.0 to 3.0) demonstrated a lower incidence of ischaemic stroke or systemic embolism (3.4 %/year) compared with low fixed-dose warfarin plus aspirin (acetylsalicylic acid) [11.9%]. The European Atrial Fibrillation Trial [EAFT] (INR 2.5 to 4.0) showed a lower incidence of all stroke (4.0 %/year) with adjusted-dose warfarin compared with placebo (12.0 %/year). The incidence of major bleeding in the adjusted-dose warfarin group in SPAF III and EAFT was 2.4 and 2.8 %/year, respectively. EAFT incidence rates for the occurrence of a first ischaemic or haemorrhagic complication analysed by INR range indicated that the rate was lowest at INRs of 2.0 to 2.9, and higher with INRs of 3.0 to 3.9. Therefore, the optimal intensity of anticoagulation for prevention of recurrent stroke seems to be an INR of between 2.0 and 3.0, as for primary prevention. Retrospective and prospective studies from Japan reported that in the elderly, haemorrhagic complications occur frequently with INRs above 2.6 and major ischaemic events cannot be prevented at INRs below 1.6. Therefore, an INR target between 1.6 and 2.6 may be an alternative for secondary prevention of stroke in elderly patients with NVAF who have a potential risk of bleeding, to avoid both major ischaemic and haemorrhagic events. Antiplatelets may be administered in patients who are unable to manage taking warfarin properly or who have a high risk of falling and subsequently sustaining a head injury, although the efficacy of antiplatelets for secondary prevention of stroke in NVAF has not yet been established."}, {"id": "wiki20220301en293_5623", "title": "Management of atrial fibrillation", "score": 0.014488146062312654, "content": "Chronic anticoagulation Among patients with nonvalvular AF, anticoagulation with warfarin can reduce stroke by 60% while antiplatelet agents can reduce stroke by 20%. The combination of aspirin and clopidogrel reduced the risk of stroke by 25%, but increased the risk of major bleeding by 57%, which means that this combination is inferior to warfarin, and is not an alternative for patients who are judged to be at high risk of bleeding on warfarin therapy."}, {"id": "wiki20220301en023_84337", "title": "Stroke", "score": 0.014288331196189778, "content": "Previous stroke or TIA Keeping blood pressure below 140/90 mmHg is recommended. Anticoagulation can prevent recurrent ischemic strokes. Among people with nonvalvular atrial fibrillation, anticoagulation can reduce stroke by 60% while antiplatelet agents can reduce stroke by 20%. However, a recent meta-analysis suggests harm from anticoagulation started early after an embolic stroke. Stroke prevention treatment for atrial fibrillation is determined according to the CHA2DS2–VASc score. The most widely used anticoagulant to prevent thromboembolic stroke in people with nonvalvular atrial fibrillation is the oral agent warfarin while a number of newer agents including dabigatran are alternatives which do not require prothrombin time monitoring. Anticoagulants, when used following stroke, should not be stopped for dental procedures."}, {"id": "wiki20220301en293_5620", "title": "Management of atrial fibrillation", "score": 0.013493958468996455, "content": "Latest ESC guidelines on atrial fibrillation recommend assessment of bleeding risk in AF using the HAS-BLED (Hypertension, Abnormal renal/liver function, Stroke, Bleeding history or predisposition, Labile International Normalized Ratio, Elderly, Drugs/alcohol concomitantly) bleeding risk schema as a simple, easy calculation, whereby a score of ≥3 indicates \"high risk\" and some caution and regular review of the patient is needed. The HAS-BLED score has also been validated in an anticoagulated trial cohort of 7329 patients with AF – in this study, the HAS-BLED score offered some improvement in predictive capability for bleeding risk over previously published bleeding risk assessment schemas and was simpler to apply. With the likely availability of new oral anticoagulants that avoid the limitations of warfarin (and may even be safer), more widespread use of oral anticoagulation therapy for stroke prevention in AF is likely."}, {"id": "pubmed23n0879_25284", "title": "[A study on the evaluation of anticoagulation status comparing of CHADS2 versus CHA2DS2-VASc scores in patients with non valvular atrial fibrillation in Xinjiang area].", "score": 0.013378076062639821, "content": "To evaluate the current status of anticoagulation therapy in patients with atrial fibrillation(AF)in Xinjiang, and compare the two scoring systems(CHADS2 and CHA2DS2-VASc scores) in determining the risk of strokes in AF patients in Xinjiang. Subjects with AF were collected by searching the electronic and paper medical records from 35 hospitals in Xinjiang area during October 2013 to October 2014, and followed up for the incident strokes after 10 to 12 months. Totally, 5 953 AF patients were enrolled in the study with the age of (67.9±12.0) years old, and men to women ratio of 1.44. Most patients were in age groups of 60-69 (23.92%) and 70-79 years (37.81%). Among patients with a CHADS2 score of 1 or less, the CHA2DS2-VASc scores of these subjects ranged from 0 to 3. After 10 to 12 months of follow-up, 22 patients developed new strokes. Only 30.79% patients ( n=1 460) received the anticoagulation treatment among those (n=4 742) who need to be treated with anticoagulation drugs. In patients receiving anticoagulant therapy, 1 162 patients were treated with warfarin, and 298 patients with new oral anticoagulant drugs.Totally 1 110 patients treated with warfarin were monitored with international normalized ratio (INR). The median INR was 1.14 with only 97 cases meeting the recommended INR ranging of 2.0-3.0 in the guidelines. The compliance rate was 8.74%. The current status of anticoagulation for AF in Xinjiang area is characterized by \"low anticoagulation rate\" and \"low compliance rate\". The CHA2DS2-VASc score is more suitable for predicting the risk of strokes in patients with non valvular atrial fibrillation in Xinjiang area."}, {"id": "pubmed23n1053_4083", "title": "Nine-year trend of oral anticoagulant use in patients with embolic stroke due to nonvalvular atrial fibrillation.", "score": 0.013354700854700854, "content": "Direct oral anticoagulants (DOACs) are increasingly used as an alternative to warfarin in patients with nonvalvular atrial fibrillation (NVAF). However, whether there is sufficient prescription of oral anticoagulants (OACs) to decrease the incidence of embolic stroke remains unclear. We conducted a retrospective observational study of patients hospitalized with ischemic stroke between January 1, 2010 and December 31, 2018. During the 8 years, the annual incidence ratio of embolic stroke to all ischemic strokes did not decrease over time (21-33%) except for that in 2018. The proportion of OAC users did not also change over time (from 23% to 45% [overall 31%], <iP</i = .78). Among the OAC users, 19% patients were warfarin users, and 12% patients were DOAC users. In 73% of warfarin users, prothrombin time was subtherapeutic, whereas in 60% of DOAC users, the dose was adequately prescribed. OACs were prescribed more often in patients with high CHADS2 score than in those with low score (<iP</i = .01). The number of patients who had no medical history of a doctor visit before admission increased significantly in the recent period of 2015-2018 (22% vs 8% in the previous period of 2010-2014) (<iP</i = .01). The incidence of embolic stroke patients without OACs did not decrease over time, and OACs in patients with NVAF have not been sufficient, even in DOAC era. In recent years, the incidence of undiagnosed AF has increased. To prevent embolic stroke, a correct AF diagnosis beforehand is important."}, {"id": "pubmed23n0947_26083", "title": "Periprocedural Outcomes of Direct Oral Anticoagulants Versus Warfarin in Nonvalvular Atrial Fibrillation.", "score": 0.013296227581941867, "content": "Direct oral anticoagulants (DOACs) are surpassing warfarin as the anticoagulant of choice for stroke prevention in nonvalvular atrial fibrillation. DOAC outcomes in elective periprocedural settings have not been well elucidated and remain a source of concern for clinicians. The aim of this meta-analysis was to evaluate the periprocedural safety and efficacy of DOACs versus warfarin in patients with nonvalvular atrial fibrillation. We reviewed the literature for data from phase III randomized controlled trials comparing DOACs with warfarin in the periprocedural period among patients with nonvalvular atrial fibrillation. Substudies from 4 trials (RE-LY [Randomized Evaluation of Long-Term Anticoagulation Therapy], ROCKET AF [Rivaroxaban Once Daily Oral Direct Factor Xa Inhibitor Compared with Vitamin K Antagonism for Prevention of Stroke and Embolism Trial in Atrial Fibrillation], ARISTOTLE [Apixaban for the Prevention of Stroke in Subjects With Atrial Fibrillation], and ENGAGE-AF [Effective Anticoagulation With Factor xA Next Generation in Atrial Fibrillation]) were included in the meta-analysis. DOACs as a group and warfarin were compared in terms of the 30-day pooled risk for stroke/systemic embolism, major bleeding, and death, according to whether the study drug was interrupted or not periprocedurally. The overall relative risk (RR) was estimated with a random-effects model. The I<sup2</sup test was used to assess heterogeneity in RR among the studies. In the uninterrupted anticoagulant strategy, there were no differences in the rates of stroke/systemic embolism (pooled risk, 0.6% [29 events/4519 procedures] versus 1.1% [31/2971]; RR, 0.70; 95% confidence interval [CI], 0.41-1.18) and death (1.4% versus 1.8%; RR, 0.77; 95% CI, 0.53-1.12) between DOACs and warfarin and significantly fewer major bleeding events (2.0% versus 3.3%; RR, 0.62; 95% CI, 0.47-0.82) with DOACs compared to warfarin. Under an interrupted strategy, there was no significant difference between DOACs versus warfarin for stroke/systemic embolism (0.4% [41/9260] versus 0.5% [31/7168]; RR, 0.95; 95% CI, 0.59-1.55), major bleeding (2.1% versus 2.0%; RR, 1.05; 95% CI, 0.85-1.30), and death (0.7% versus 0.6%; RR, 1.24; 95% CI, 0.76-2.04). The studies were homogeneous ( I<sup2</sup=0.0%) for all calculated pooled associations except for the RR of death in the interrupted strategy ( I<sup2</sup=26.3%). The short-term safety and efficacy of DOACs and warfarin are not different in patients with nonvalvular atrial fibrillation periprocedurally. Under an uninterrupted anticoagulation strategy, DOACs are associated with a 38% lower risk of major bleeding compared with warfarin."}, {"id": "wiki20220301en248_20372", "title": "Atrial fibrillation", "score": 0.01294463568559954, "content": "For those with non-valvular atrial fibrillation, DOACs are at least as effective as warfarin for preventing strokes and blood clots embolizing to the systemic circulation (if not more so) and are generally preferred over warfarin. DOACs carry a lower risk of bleeding in the brain compared to warfarin, although dabigatran is associated with a higher risk of intestinal bleeding. Dual antiplatelet therapy with aspirin and clopidogrel is inferior to warfarin for preventing strokes and has comparable bleeding risk in people with atrial fibrillation. In those who are also on aspirin, however, DOACs appear to be better than warfarin."}, {"id": "pubmed23n0799_5500", "title": "Comparing mortality in patients with atrial fibrillation who are receiving a direct-acting oral anticoagulant or warfarin: a meta-analysis of randomized trials.", "score": 0.012861869313482217, "content": "In patients with non-valvular atrial fibrillation (AF), direct-acting oral anticoagulants (DOACs) are at least non-inferior to warfarin for the prevention of stroke and systemic embolism. The main objective of this study was to obtain reliable and precise estimates for all-cause mortality, vascular mortality and bleeding mortality in patients with AF receiving a DOAC or warfarin for stroke prevention. A meta-analysis was performed on phase 3 randomized trials that compared a DOAC with warfarin for stroke prevention in AF. Published data were pooled by use of the DerSimonian random-effect model, with revman 5.2 and comprehensive meta analysis software version 2. The results were presented as risk ratios (RRs), absolute risk reduction (ARR), and number-needed-to-treat (NNT). A total of 71 683 patients were included in this meta-analysis from four randomized controlled trials (median patient follow-up: 1.8-2.8 years) that compared a DOAC with warfarin for stroke prevention in AF. As compared with warfarin, DOACs significantly reduced all-cause mortality (RR 0.89, 95% confidence interval [CI] 0.85-0.94; ARR 0.76%, 95% CI 0.39-1.13%; NNT = 132), vascular mortality (RR 0.88, 95% CI 0.82-0.94; ARR 0.53%, 95% CI 0.23-0.83%; NNT = 189), and bleeding mortality (RR 0.54, 95% CI 0.44-0.67; ARR 0.32%, 95% CI 0.21-0.43%; NNT = 313). As compared with warfarin therapy for stroke prevention in patients with AF, DOACs significantly reduce all-cause mortality, vascular mortality, and bleeding mortality. This mortality benefit appears to be driven by the reduction in vascular-related and bleeding-related mortality, which, in turn, may be related to the reduction in intracranial bleeding."}, {"id": "pubmed23n0721_22676", "title": "Current trial-associated outcomes with warfarin in prevention of stroke in patients with nonvalvular atrial fibrillation: a meta-analysis.", "score": 0.012768449824287388, "content": "Although several new antithrombotic agents have been developed for stroke prevention in patients with nonvalvular atrial fibrillation (AF), many patients will continue to be treated with warfarin worldwide. We performed a meta-analysis of safety and efficacy outcomes in patients with AF treated with warfarin for stroke prevention in large contemporary randomized controlled trials (RCTs). We searched the MEDLINE, EMBASE, and Cochrane databases for relevant studies; RCTs comparing warfarin with an alternative thromboprophylaxis strategy with at least 400 patients in the warfarin arm and reporting stroke as an efficacy outcome were included. Eight RCTs with 55,789 patient-years of warfarin therapy follow-up were included. Overall time spent in the therapeutic range was 55% to 68%. The annual incidence of stroke or systemic embolism in patients with AF taking warfarin was estimated to be 1.66% (95% CI, 1.41%-1.91%). Major bleeding rates varied from 1.40% to 3.40% per year across the studies. The risk of stroke per year was significantly higher in elderly patients (2.27%), female patients (2.12%), patients with a history of stroke (2.64%), and patients reporting no previous exposure to vitamin K antagonists (1.96%). There was a significant increase in the annual incidence of stroke with progressively increasing CHADS(2) (congestive heart failure, hypertension, age, diabetes, and prior stroke) scores. Current use of warfarin as a stroke prevention agent in patients with AF is associated with a low rate of residual stroke or systemic embolism estimated to be 1.66% per year. Compared with a previous meta-analysis, there has been significant improvement in the proportion of time spent in therapeutic anticoagulation, with a resultant decline in observed stroke rates."}, {"id": "wiki20220301en470_19617", "title": "HAS-BLED", "score": 0.012482389405466329, "content": "2020 ESC guidelines on atrial fibrillation recommend assessment of bleeding risk in AF using the HAS-BLED bleeding risk schema as a simple, easy calculation, whereby a score of ≥3 indicates \"high risk\" and some caution and regular review of the patient is needed. The HAS-BLED score has also been validated in an anticoagulated trial cohort of 7329 people with AF - in this study, the HAS-BLED score offered some improvement in predictive capability for bleeding risk over previously published bleeding risk assessment schemas and was simpler to apply. With the likely availability of new oral anticoagulants that avoid the limitations of warfarin (and may even be safer), more widespread use of oral anticoagulation therapy for stroke prevention in AF is likely."}, {"id": "wiki20220301en466_37386", "title": "SAMe-TT2R2 score", "score": 0.01208580347681645, "content": "The SAMe-TT2R2 score is a clinical prediction rule to predict the quality of vitamin K antagonist anticoagulation therapy as measured by time in therapeutic INR range (TTR) (VKA e.g. warfarin). It has been suggested that it can aid in the medical decision making between VKAs and new oral anticoagulant/non-VKA oral anticoagulant (NOAC e.g. dabigatran, rivaroxaban, apixaban or edoxaban) in patients with atrial fibrillation (AF). This score can be used with patients with ≥1 additional stroke risk factors using the CHA2DS2-VASc score, where oral anticoagulation is recommended or should be considered. This score reflects the need to offer an improved patient care pathway when using oral anticoagulants. While NOACs avoid the need for drug monitoring (e.g. INR monitoring), they have an unstable bioavailability and are not indicated for patients with chronic kidney failure or in patients with valvular replacement surgery."}, {"id": "wiki20220301en001_181798", "title": "Transient ischemic attack", "score": 0.011877828054298644, "content": "Anticoagulants may be started if the TIA is thought to be attributable to atrial fibrillation. Atrial fibrillation is an abnormal heart rhythm that may cause the formation of blood clots that can travel to the brain, resulting in TIAs or ischemic strokes. Atrial fibrillation increases stroke risk by five times, and is thought to cause 10-12% of all ischemic strokes in the US. Anticoagulant therapy can decrease the relative risk of ischemic stroke in those with atrial fibrillation by 67% Warfarin is a common anticoagulant used, but direct acting oral anticoagulants (DOACs), such as apixaban, have been shown to be equally effective while also conferring a lower risk of bleeding. Generally, anticoagulants and antiplatelets are not used in combination, as they result in increased bleeding risk without a decrease in stroke risk. However, combined antiplatelet and anticoagulant therapy may be warranted if the patient has symptomatic coronary artery disease in addition to atrial"}, {"id": "wiki20220301en194_5563", "title": "VKORC1", "score": 0.011633680925019508, "content": "Warfarin is a commonly prescribed oral anticoagulant, or blood thinner used to treat blood clots such as deep vein thrombosis and pulmonary embolism and to prevent stroke in people who have atrial fibrillation, valvular heart disease or artificial heart valves. Warfarin causes inhibition on VKORC1 activities and leads to a reduced amount of vitamin K available to serve as a cofactor for clotting proteins. Inappropriate dosing of warfarin has been associated with a substantial risk of both major and minor hemorrhage. As the pharmacological target of warfarin, VKORC1 is considered a candidate gene for the variability in warfarin response. Previous researches have shown that the CYP2C9 genotype of patients also played a role in warfarin metabolism and response. Gene The human gene is located on chromosome 16. Two pseudogenes have been identified on chromosome 1 and the X chromosome. Clinical relevance"}, {"id": "pubmed23n0479_8228", "title": "Anticoagulation therapy for stroke prevention in atrial fibrillation: how well do randomized trials translate into clinical practice?", "score": 0.011497631879797486, "content": "Warfarin has been shown to be highly efficacious for preventing thromboembolism in atrial fibrillation in randomized trials, but its effectiveness and safety in clinical practice is less clear. To evaluate the effect of warfarin on risk of thromboembolism, hemorrhage, and death in atrial fibrillation within a usual care setting. Cohort study assembled between July 1, 1996, and December 31, 1997, and followed up through August 31, 1999. Large integrated health care system in Northern California. Of 13,559 adults with nonvalvular atrial fibrillation, 11,526 were studied, 43% of whom were women, mean age 71 years, with no known contraindications to anticoagulation at baseline. Ischemic stroke, peripheral embolism, hemorrhage, and death according to warfarin use and comorbidity status, as determined by automated databases, review of medical records, and state mortality files. Among 11,526 patients, 397 incident thromboembolic events (372 ischemic strokes, 25 peripheral embolism) occurred during 25,341 person-years of follow-up, and warfarin therapy was associated with a 51% (95% confidence interval [CI], 39%-60%) lower risk of thromboembolism compared with no warfarin therapy (either no antithrombotic therapy or aspirin) after adjusting for potential confounders and likelihood of receiving warfarin. Warfarin was effective in reducing thromboembolic risk in the presence or absence of risk factors for stroke. A nested case-control analysis estimated a 64% reduction in odds of thromboembolism with warfarin compared with no antithrombotic therapy. Warfarin was also associated with a reduced risk of all-cause mortality (adjusted hazard ratio, 0.69; 95% CI, 0.61-0.77). Intracranial hemorrhage was uncommon, but the rate was moderately higher among those taking vs those not taking warfarin (0.46 vs 0.23 per 100 person-years, respectively; P =.003, adjusted hazard ratio, 1.97; 95% CI, 1.24-3.13). However, warfarin therapy was not associated with an increased adjusted risk of nonintracranial major hemorrhage. The effects of warfarin were similar when patients with contraindications at baseline were analyzed separately or combined with those without contraindications (total cohort of 13,559). Warfarin is very effective for preventing ischemic stroke in patients with atrial fibrillation in clinical practice while the absolute increase in the risk of intracranial hemorrhage is small. Results of randomized trials of anticoagulation translate well into clinical care for patients with atrial fibrillation."}, {"id": "wiki20220301en323_15747", "title": "Edoxaban", "score": 0.011473453966991448, "content": "Compared with warfarin it has fewer drug interactions. It was developed by Daiichi Sankyo and approved in July 2011, in Japan for prevention of venous thromboembolisms following lower-limb orthopedic surgery. It was also approved in the United States by the Food and Drug Administration (FDA) in January 2015, for the prevention of stroke and non–central-nervous-system systemic embolism. It was approved for use in the European Union in June 2015. It is on the World Health Organization's List of Essential Medicines. Medical uses In the United States, edoxaban is approved for treating deep vein thrombosis and pulmonary embolism following five to ten days of initial therapy with a parenteral anticoagulant. It is also approved for reducing the risk of blood clots in people with nonvalvular atrial fibrillation."}, {"id": "pubmed23n0853_18929", "title": "Non-vitamin K Oral Anticoagulants Versus Warfarin for Patients with Atrial Fibrillation: Absolute Benefit and Harm Assessments Yield Novel Insights.", "score": 0.011419513457556936, "content": "Benefits and/or harms (including costs) of non-vitamin K oral anticoagulants (NOACs) versus warfarin therapy need appreciation in relative and absolute terms. Accordingly, we derived clinically relevant relative and absolute benefit/harm parameters for NOACs (apixaban, dabigatran, rivaroxaban, edoxaban) compared to warfarin from four clinical trials involving atrial fibrillation (AF) patients. For each trial, we tabulated patient numbers enduring four important outcomes and calculated unadjusted relative risk reduction (RRR) and number needed to treat (NNT)/year values (and 95% confidence intervals) for the NAOC compared to warfarin. These outcomes were as follows: stroke/systemic embolism (primary endpoint), hemorrhagic stroke, major bleeds, and death. We also addressed drug acquisition costs. Each NOAC was noninferior to warfarin for primary-outcome prevention; RRRs were 12-33% and NNT/year values were 182-481, and all but one indicated statistically significant superiority. All the NOACs yielded statistically significant reductions in hemorrhagic stroke risk; RRRs were 42-74% and NNT/year values were 364-528. Major bleeding risk was comparable in both groups. Apixaban yielded a lower NNT/year for preventing death than for primary-outcome prevention. Compared to warfarin, NOAC acquisition costs were 70- to 140-fold greater. For the primary outcome, the absolute benefits of NOACs were modest (NNT/year values being large). Reduced hemorrhagic stroke rates with NOACs could be due to superior embolic infarct prevention and fewer consequential hemorrhagic transformations. Among apixaban recipients, the absolute mortality benefit exceeded that for the primary outcome, indicating prevention of additional unrelated deaths. The substantially greater NOAC acquisition costs need viewing against probable greater safety and the avoidance of monitoring bleeding risks."}, {"id": "pubmed23n0862_18137", "title": "Cost-Effectiveness of High-Dose Edoxaban Compared with Adjusted-Dose Warfarin for Stroke Prevention in Non-Valvular Atrial Fibrillation Patients.", "score": 0.011061077699537611, "content": "To estimate the quality-adjusted life-years (QALYs), costs, and cost-effectiveness of high-dose edoxaban compared with adjusted-dose warfarin in patients at risk for stroke who have nonvalvular atrial fibrillation (NVAF) and a creatinine clearance (Clcr ) of 15-95 ml/minute. A Markov model was created to compare the cost-effectiveness of high-dose edoxaban and adjusted-dose warfarin in patients with a Clcr of 15-95 ml/minute. The model was performed from a U.S. societal perspective and assumed patients initiated therapy at 70 years of age, had a mean CHADS2 (congestive heart failure, hypertension, age 75 or older, diabetes, stroke) score of 3, and no contraindications to anticoagulation. The model assumed a cycle length of 1 month and a lifetime horizon (maximum of 30 years/360 cycles). Data sources included renal subgroup analysis of the Effective Anticoagulation with Factor Xa Next Generation in Atrial Fibrillation (ENGAGE-AF) trial and other published studies. Outcomes included lifetime costs (2014 US$), QALYs, and incremental cost-effectiveness ratios. The robustness of the model's conclusions was tested using one-way and 10,000-iteration probabilistic sensitivity analysis (PSA). Patients treated with high-dose edoxaban lived an average of 10.50 QALYs at a lifetime treatment cost of $99,833 compared with 10.11 QALYs and $123,516 for those treated with adjusted-dose warfarin. The model's conclusions were found to be robust upon one-way sensitivity analyses. PSA suggested high-dose edoxaban was economically dominant compared with adjusted-dose warfarin in more than 99% of the 10,000 iterations run. High-dose edoxaban appears to be an economically dominant strategy when compared with adjusted-dose warfarin for the prevention of stroke in NVAF patients with a Clcr of 15-95 ml/minute and an appreciable risk of stroke."}, {"id": "wiki20220301en248_20386", "title": "Atrial fibrillation", "score": 0.010544910993341487, "content": "Prognosis Atrial fibrillation increases the risk of heart failure by 11 per 1000, kidney problems by 6 per 1000, death by 4 per 1000, stroke by 3 per 1000, and coronary heart disease by 1 per 1000. Women have a worse outcome overall than men. Evidence increasingly suggests that atrial fibrillation is independently associated with a higher risk of developing dementia. Blood clots Prediction of embolism Among Danish men aged 50, with no risk factors, the 5-year risk of stroke was 1.1% and with AF alone 2.5%. For women the risks were slightly less, 0.7% and 2.1%. For men aged 70, the 5-year risk of stroke was 4.8% and with AF alone 6.8%. For women aged 70 the risk was again lower than for men, 3.4% with no added risk factor and 8.2% with AF. Determining the risk of an embolism causing a stroke is important for guiding the use of anticoagulants. The most accurate clinical prediction rules are: CHADS2 CHA2DS2-VASc score"}, {"id": "wiki20220301en293_5614", "title": "Management of atrial fibrillation", "score": 0.01006993006993007, "content": "diabetes. For patients with LAF, the risk of stroke is very low and is independent of whether the LAF was an isolated episode, paroxysmal, persistent, or permanent. The risk of systemic embolization (atrial clots migrating to other organs) depends strongly on whether there is an underlying structural problem with the heart (e.g. mitral stenosis) and on the presence of other risk factors, such as diabetes and high blood pressure. Finally, patients under 65 are much less likely to develop embolization compared with patients over 75. In young patients with few risk factors and no structural heart defect, the benefits of anticoagulation may be outweighed by the risks of hemorrhage (bleeding). Those at a low risk may benefit from mild (and low-risk) anticoagulation with aspirin (or clopidogrel in those who are allergic to aspirin). In contrast, those with a high risk of stroke derive most benefit from anticoagulant treatment with warfarin or similar drugs. A new class of anticoagulant"}, {"id": "pubmed23n0407_19897", "title": "[Anticoagulation in permanent atrial fibrillation after 75 years of age].", "score": 0.009900990099009901, "content": "More than 10% of the population over 75 years old is concerned by non valvular permanent atrial fibrillation which is responsible for at least 30% of ischemic strokes. The indication of an anticoagulant therapy is discussed in two different situations: primary or secondary prevention of stroke and acute phase of stroke. Patients over 75 years old have a high risk of stroke (&gt; 8% year). All the studies have demonstrated the benefit of a primary or secondary prevention by antivitamin K with an INR between 2 and 3 (reduction of the relative risk of about 68%). Conversely, the efficacy of aspirin has not been proven in this population of elderly patients. Once stroke has occurred, it is not recommended to initiate an anticoagulation (unfractioned or low molecular weight heparin) within the first hours. Prevention of venous thrombosis remains necessary. Currently, less than 30% of the patients older than 75 years are given anticoagulation, the risk of the treatment being probably overestimated. The risk benefit ratio should be evaluated more properly for a given patient."}, {"id": "wiki20220301en023_84333", "title": "Stroke", "score": 0.00988349885408709, "content": "Those with atrial fibrillation have a 5% a year risk of stroke, and this risk is higher in those with valvular atrial fibrillation. Depending on the stroke risk, anticoagulation with medications such as warfarin or aspirin is useful for prevention. Except in people with atrial fibrillation, oral anticoagulants are not advised for stroke prevention—any benefit is offset by bleeding risk. In primary prevention, however, antiplatelet drugs did not reduce the risk of ischemic stroke but increased the risk of major bleeding. Further studies are needed to investigate a possible protective effect of aspirin against ischemic stroke in women."}, {"id": "wiki20220301en293_5613", "title": "Management of atrial fibrillation", "score": 0.00978349488178277, "content": "Most patients with AF are at increased risk of stroke. The possible exceptions are those with lone AF (LAF), characterized by absence of clinical or echocardiographic findings of other cardiovascular disease (including hypertension), related pulmonary disease, or cardiac abnormalities such as enlargement of the left atrium, and age under 60 years . The incidence of stroke associated with AF is 3 to 5 percent per year in the absence of anticoagulation, which is significantly higher compared to the general population without AF (relative risk 2.4 in men and 3.0 in women). A systematic review of risk factors for stroke in patients with nonvalvular AF concluded that a prior history of stroke or TIA is the most powerful risk factor for future stroke, followed by advancing age, hypertension, and diabetes. For patients with LAF, the risk of stroke is very low and is independent of whether the LAF was an isolated episode, paroxysmal, persistent, or permanent. The risk of systemic embolization"}, {"id": "pubmed23n0650_13479", "title": "Relationship between CHADS2 score and ischemic stroke during rhythm control therapy for paroxysmal atrial fibrillation.", "score": 0.009708737864077669, "content": "The CHADS2 score has been proposed for stratifying patients with nonvalvular atrial fibrillation (NVAF) according to the risk of thromboembolism in the AHA/ACC/ESC guidelines. However, there is little information about its usefulness for predicting the long-term risk of ischemic stroke in Japanese patients with paroxysmal AF. We retrospectively evaluated the incidence of ischemic stroke and the efficacy of anticoagulant therapy in paroxysmal AF patients on rhythm control therapy who were stratified by their CHADS2 score. The subjects were 334 NVAF atients (229 men and 105 women, mean age, 68 +/- 12 years, mean follow-up period, 60 +/- 35 months) who were categorized into low risk (score 0), moderate risk (1 or 2), and high risk (3 or more) groups for thromboembolism. The low, moderate, and high risk groups accounted for 34%, 50%, and 16% of the patients, respectively. Among 257 patients without warfarin therapy, the annual rate of symptomatic ischemic stroke was 0.6% in the score 0 group, 0.5% in the score 1 group, 3.1% in the score 2 group, and 9.6% in the score 3 or more group. Among 77 patients treated with warfarin (target PT-INR: 1.6-3.0), the stroke rate was 0% in the score 0 group, 0% in the score 1 group, 1.4% in the score 2 group, and 6.6% in the score 3 or more group. The annual rate of ischemic stroke was 0.88% in patients treated with warfarin versus 2.67% in those without warfarin, or a decrease in risk of 68% with warfarin (P &lt; 0.01). In Japanese patients with paroxysmal AF, the CHADS2 score is useful for predicting the risk of ischemic stroke. Anticoagulant therapy is needed to prevent ischemic stroke in patients with paroxysmal AF, especially those who have a CHADS2 score of 2 or more."}, {"id": "pubmed23n0250_0", "title": "Cost-effectiveness of warfarin and aspirin for prophylaxis of stroke in patients with nonvalvular atrial fibrillation.", "score": 0.009708737864077669, "content": "To examine the cost-effectiveness of prescribing warfarin sodium in patients who have nonvalvular atrial fibrillation (NVAF) with or without additional stroke risk factors (a prior stroke or transient ischemic attack, diabetes, hypertension, or heart disease). Decision and cost-effectiveness analyses. The probabilities for stroke, hemorrhage, and death were obtained from published randomized controlled trials. The quality-of-life estimates were obtained by interviewing 74 patients with atrial fibrillation. Costs were estimated from literature review, phone survey, and Medicare reimbursement. In the base case, the patients were 65 years of age and good candidates for warfarin therapy. Treatment with warfarin, aspirin, or no therapy in the decision analytic model. Quality-adjusted survival and marginal cost-effectiveness of warfarin as compared with aspirin or no therapy. For patients with NVAF and additional risk factors for stroke, warfarin therapy led to a greater quality-adjusted survival and to cost savings. For patients with NVAF and one additional risk factor, warfarin therapy cost $8000 per quality-adjusted life-year saved. For 65-year-old patients with NVAF alone, warfarin cost about $370,000 per quality-adjusted life-year saved, as compared with aspirin therapy. However, for 75-year-old patients with NVAF alone, prescribing warfarin cost $110,000 per quality-adjusted life-year saved. For patients who were not prescribed warfarin, aspirin was preferred to no therapy on the basis of both quality-adjusted survival and cost in all patients, regardless of the number of risk factors present. Treatment with warfarin is cost-effective in patients with NVAF and one or more additional risk factors for stroke. In 65-year-old patients with NVAF but no other risk factors for stroke, prescribing warfarin instead of aspirin would affect quality-adjusted survival minimally but increase costs significantly."}, {"id": "pubmed23n0678_3353", "title": "Cost-effectiveness of dabigatran compared with warfarin for stroke prevention in atrial fibrillation.", "score": 0.009615384615384616, "content": "Warfarin reduces the risk for ischemic stroke in patients with atrial fibrillation (AF) but increases the risk for hemorrhage. Dabigatran is a fixed-dose, oral direct thrombin inhibitor with similar or reduced rates of ischemic stroke and intracranial hemorrhage in patients with AF compared with those of warfarin. To estimate the quality-adjusted survival, costs, and cost-effectiveness of dabigatran compared with adjusted-dose warfarin for preventing ischemic stroke in patients 65 years or older with nonvalvular AF. Markov decision model. The RE-LY (Randomized Evaluation of Long-Term Anticoagulation Therapy) trial and other published studies of anticoagulation. The cost of dabigatran was estimated on the basis of pricing in the United Kingdom. Patients aged 65 years or older with nonvalvular AF and risk factors for stroke (CHADS₂ score ≥1 or equivalent) and no contraindications to anticoagulation. Lifetime. Societal. Warfarin anticoagulation (target international normalized ratio, 2.0 to 3.0); dabigatran, 110 mg twice daily (low dose); and dabigatran, 150 mg twice daily (high dose). Quality-adjusted life-years (QALYs), costs (in 2008 U.S. dollars), and incremental cost-effectiveness ratios. The quality-adjusted life expectancy was 10.28 QALYs with warfarin, 10.70 QALYs with low-dose dabigatran, and 10.84 QALYs with high-dose dabigatran. Total costs were $143 193 for warfarin, $164 576 for low-dose dabigatran, and $168 398 for high-dose dabigatran. The incremental cost-effectiveness ratios compared with warfarin were $51 229 per QALY for low-dose dabigatran and $45 372 per QALY for high-dose dabigatran. The model was sensitive to the cost of dabigatran but was relatively insensitive to other model inputs. The incremental cost-effectiveness ratio increased to $50 000 per QALY at a cost of $13.70 per day for high-dose dabigatran but remained less than $85 000 per QALY over the full range of model inputs evaluated. The cost-effectiveness of high-dose dabigatran improved with increasing risk for stroke and intracranial hemorrhage. Event rates were largely derived from a single randomized clinical trial and extrapolated to a 35-year time frame from clinical trials with approximately 2-year follow-up. In patients aged 65 years or older with nonvalvular AF at increased risk for stroke (CHADS₂ score ≥1 or equivalent), dabigatran may be a cost-effective alternative to warfarin depending on pricing in the United States. American Heart Association and Veterans Affairs Health Services Research &amp; Development Service."}, {"id": "wiki20220301en012_859", "title": "Warfarin", "score": 0.009537487111503904, "content": "Warfarin is best suited for anticoagulation (clot formation inhibition) in areas of slowly running blood (such as in veins and the pooled blood behind artificial and natural valves), and in blood pooled in dysfunctional cardiac atria. Thus, common clinical indications for warfarin use are atrial fibrillation, the presence of artificial heart valves, deep venous thrombosis, and pulmonary embolism (where the embolized clots first form in veins). Warfarin is also used in antiphospholipid syndrome. It has been used occasionally after heart attacks (myocardial infarctions), but is far less effective at preventing new thromboses in coronary arteries. Prevention of clotting in arteries is usually undertaken with antiplatelet drugs, which act by a different mechanism from warfarin (which normally has no effect on platelet function). It can be used to treat people following ischemic strokes due to atrial fibrillation, though direct oral anticoagulants (DOACs) may offer greater benefits."}, {"id": "pubmed23n0737_9781", "title": "Cost-effectiveness of apixaban vs warfarin for secondary stroke prevention in atrial fibrillation.", "score": 0.009523809523809525, "content": "To compare the cost-effectiveness of apixaban vs warfarin for secondary stroke prevention in patients with atrial fibrillation (AF). Using standard methods, we created a Markov decision model based on the estimated cost of apixaban and data from the Apixaban for Reduction in Stroke and Other Thromboembolic Events in Atrial Fibrillation (ARISTOTLE) trial and other trials of warfarin therapy for AF. We quantified the cost and quality-adjusted life expectancy resulting from apixaban 5 mg twice daily compared with those from warfarin therapy targeted to an international normalized ratio of 2-3. Our base case population was a cohort of 70-year-old patients with no contraindication to anticoagulation and a history of stroke or TIA from nonvalvular AF. Warfarin therapy resulted in a quality-adjusted life expectancy of 3.91 years at a cost of $378,500. In comparison, treatment with apixaban led to a quality-adjusted life expectancy of 4.19 years at a cost of $381,700. Therefore, apixaban provided a gain of 0.28 quality-adjusted life-years (QALYs) at an additional cost of $3,200, resulting in an incremental cost-effectiveness ratio of $11,400 per QALY. Our findings were robust in univariate sensitivity analyses varying model inputs across plausible ranges. In Monte Carlo analysis, apixaban was cost-effective in 62% of simulations using a threshold of $50,000 per QALY and 81% of simulations using a threshold of $100,000 per QALY. Apixaban appears to be cost-effective relative to warfarin for secondary stroke prevention in patients with AF, assuming that it is introduced at a price similar to that of dabigatran."}, {"id": "pubmed23n0859_17479", "title": "Comparison of Aspirin and Naoxintong Capsule () with Adjusted-Dose Warfarin in Elderly Patients with High-Risk of Non-Valvular Atrial Fibrillation and Genetic Variants of Vitamin K Epoxide Reductase.", "score": 0.009433962264150943, "content": "To compared the therapeutic effect of a Chinese patent medicine Naoxintong Capsule (, NXT) and aspirin with adjusted-dose warfarin in Chinese elderly patients (over 65 years) with nonvalvular atrial fibrillation (NVAF) and genetic variants of vitamin K epoxide reductase (VKORC1), who are at high-risk of thromboembolism. A total of 151 patients, with NVAF and AA genotype of VKORC1-1639 (a sensitive genotype to warfarin) and a CHA<sub2</subDS<sub2</sub-VASc clinical risk score of 2 or above, were chosen for this study. Patients were randomized into two groups and orally treated with a combination of aspirin (100 mg/day) and NXT (1.6 g thrice a day) or adjusted-dose warfarin [international normalized ratio 2.0-3.0). The primary end points including ischemic stroke and death as well as the secondary end points including hemorrhage events were followed up for at least 1 year. Baseline clinical data and the rates of primary end points were similar between groups. However, the rate of serious bleeding (secondary event) in the combination therapy group was lower than that in the adjusted-dose warfarin group (0% vs. 7.9%, odds ratio: 0.921, 95% confidence interval: 0.862-0.984, P=0.028). Aspirin combined with NXT and warfarin displayed comparable rates of primary end point including ischemic stroke and all-cause death during the 1-year follow-up. However, as compared with warfarin, the combination therapy reduced the rate of serious bleeding. Therefore, aspirin combined with NXT might provide an alternative pharmacotherapy in preventing ischemic stroke for elderly patients with NAVF who cannot tolerate warfarin. (No. ChiCTR-TRC-13003596)."}, {"id": "wiki20220301en293_5619", "title": "Management of atrial fibrillation", "score": 0.009426847662141781, "content": "To compensate for the increased risk of stroke, anticoagulants may be required. However, in the case of warfarin, if someone with AF has a yearly risk of stroke that is less than 2%, then the risks associated with taking warfarin outweigh the risk of getting a stroke from AF. However, since these older data, there is now greater recognition of the importance of good anticoagulation control (as reflected by time in therapeutic range) as well as greater awareness of bleeding risk factors as well as data from recent trials that aspirin carries a similar rate of major bleeding to warfarin, especially in the elderly."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": true, "char_ranges": [[0, 200]], "word_ranges": [[0, 32]], "text": "Patient with familial adenomatous polyposis. All statements are correct except 2. Treatment should be surgical when polyposis is observed. At 40 years of age is the usual point of incidence of cancer."}, "2": {"exist": true, "char_ranges": [[0, 200]], "word_ranges": [[0, 32]], "text": "Patient with familial adenomatous polyposis. All statements are correct except 2. Treatment should be surgical when polyposis is observed. At 40 years of age is the usual point of incidence of cancer."}, "3": {"exist": true, "char_ranges": [[0, 200]], "word_ranges": [[0, 32]], "text": "Patient with familial adenomatous polyposis. All statements are correct except 2. Treatment should be surgical when polyposis is observed. At 40 years of age is the usual point of incidence of cancer."}, "4": {"exist": true, "char_ranges": [[0, 200]], "word_ranges": [[0, 32]], "text": "Patient with familial adenomatous polyposis. All statements are correct except 2. Treatment should be surgical when polyposis is observed. At 40 years of age is the usual point of incidence of cancer."}, "5": {"exist": true, "char_ranges": [[0, 200]], "word_ranges": [[0, 32]], "text": "Patient with familial adenomatous polyposis. All statements are correct except 2. Treatment should be surgical when polyposis is observed. At 40 years of age is the usual point of incidence of cancer."}}, "full_answer": "Patient with familial adenomatous polyposis. All statements are correct except 2. Treatment should be surgical when polyposis is observed. At 40 years of age is the usual point of incidence of cancer.", "full_answer_no_ref": "Patient with familial adenomatous polyposis. All statements are correct except [HIDDEN]. Treatment should be surgical when polyposis is observed. At 40 years of age is the usual point of incidence of cancer.", "full_question": "A 30-year-old man with a family history of a father who died at 38 years of age from colon cancer. A colonoscopy is performed and shows hundreds of adenomas throughout the colon. Which of the following statements is false?", "id": 6, "lang": "en", "options": {"1": "The patient has familial adenomatous polyposis.", "2": "The most appropriate management is annual follow-up colonoscopy and colectomy at age 40.", "3": "If the patient does not undergo surgical treatment, he/she will almost certainly develop colorectal cancer.", "4": "First degree relatives should be studied.", "5": "The patient's children have a 50% risk of suffering the same disease."}, "question_id_specific": 45, "type": "DIGESTIVE", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0653_5733", "title": "Clinical inquiries. What's the most effective way to screen patients with a family history of colon cancer?", "score": 0.018874643874643875, "content": "The best approach for screening patients hinges on the number, degree, and age of relatives diagnosed with colorectal cancer (CRC) or adenomatous polyps (AP). Screening should begin at 40 years of age for patients with a family history of CRC or AP in at least 1 first-degree relative or CRC in at least 2 second-degree relatives. Patients at highest risk-who have 1 first-degree relative diagnosed with CRC or AP before 60 years of age or multiple first-degree relatives diagnosed at any age-should begin screening with colonoscopy at 40 years of age or 10 years younger than the earliest affected relative and undergo a repeat colonoscopy every 5 years. Patients who have a first-degree relative diagnosed with CRC or AP after 60 years of age or 2 or more second-degree relatives with CRC should start screening at 40 years of age, with routine options and follow-up intervals. (Routine options and follow-up intervals include any of the following 3 regimens: annual high-sensitivity fecal occult blood testing, sigmoidoscopy every 5 years combined with high-sensitivity fecal occult blood testing every 3 years, or screening colonoscopy every 10 years.)."}, {"id": "pubmed23n0315_4476", "title": "An unusually severe phenotype for familial adenomatous polyposis.", "score": 0.018350930115636, "content": "Familial adenomatous polyposis (FAP) is a dominantly inherited predisposition to the development of many hundreds to thousands of adenomatous polyps of the colon. The mean age of onset is around 15 years, symptoms may arise in the third decade, and the median age for the development of colonic cancer is 35-40 years. Prophylactic colectomy reduces the risk of death from colorectal cancer to such an extent that late sequelae such as upper gastrointestinal tumours have become the main cause of mortality in appropriately managed patients. The age at which colonic surveillance begins reflects the natural history of the disease. Onset of polyp formation and cancer in childhood is very unusual, but has recently been associated with a specific mutation at codon 1309 in exon 15 where a more severe phenotype is sometimes observed. The case histories of two families are reported in which there is childhood onset of polyps in the youngest generation and in one case a carcinoma, in whom mutations have been identified in exon 11 of the APC gene. Several other affected relatives were diagnosed at ages ranging from 5-48 years, some already with a cancer at the time of first screening. Since the aim of screening for colonic polyps is prevention of colonic cancer, family members at risk should be offered genetic assessment and direct mutation testing where this is possible, usually in the early teens. In the absence of a genetic test (the situation in about one third of families) or in a known gene carrier, annual colonoscopy examination is advised from the same age. Clinicians should take note of the family history and be prepared to consider much earlier intervention if symptoms occur in a child with a family history of FAP. Where childhood onset of polyps has occurred, other children at risk in the family must be offered earlier genetic testing and endoscopic surveillance."}, {"id": "pubmed23n0934_5997", "title": "Colorectal Cancer Screening and Surveillance in Individuals at Increased Risk.", "score": 0.017284495439835245, "content": "Individuals at increased risk of developing colorectal cancer include those with a personal or family history of advanced adenomas or colorectal cancer, a personal history of inflammatory bowel disease, or genetic polyposis syndromes. In general, these persons should undergo more frequent or earlier testing than individuals at average risk. Individuals who have a first-degree relative with colorectal cancer or advanced adenoma diagnosed before 60 years of age or two first-degree relatives diagnosed at any age should be advised to start screening colonoscopy at 40 years of age or 10 years younger than the earliest diagnosis in their family, whichever comes first. In individuals with ulcerative colitis or Crohn disease with colonic involvement, colonoscopy should begin eight to 10 years after the onset of symptoms and be repeated every one to three years. Individuals who have a first-degree relative with hereditary nonpolyposis colorectal cancer should begin colonoscopy at 25 years of age and repeat colonoscopy every one to two years. In persons with a family history of adenomatous polyposis syndromes, screening should begin at 10 years of age or in a person's mid-20s, depending on the syndrome; repeat colonoscopy is typically required every one to two years. Screening colonoscopy should begin at eight years of age in individuals with Peutz-Jeghers syndrome. If results are normal, colonoscopy can be repeated at 18 years of age and then every three years. Persons with sessile serrated adenomatous polyposis should begin annual colonoscopy as soon as the diagnosis is established."}, {"id": "pubmed23n0685_7334", "title": "Familial adenomatous polyposis.", "score": 0.016849557522123894, "content": "Familial adenomatous polyposis represents approximately 1% of all colorectal tumours and is caused by germline mutations in the adenomatous polyposis coli (APC) gene. A 38-year-old lady presented with abdominal pain, diarrhoea and iron deficiency anemia. There was no history of colorectal cancer in the family. Colonoscopy showed hundreds of polyps throughout the colon sparing the rectum, and an ulcerative tumour of the sigmoid colon. The diagnosis was familial adenomatous polyposis (FAP) and adenocarcinoma of the sigmoid colon. Colectomy with ileorectal anastomosis was performed and later on she was given chemotherapy and advice life long surveillance. The patient had one brother and one sister, without clinical symptoms. The brother had a single hyperplastic rectal polyp, while the sister refused colonoscopy. The patient has 2 sons, the elder son had normal colonoscopic findings, and the younger son was also diagnosed as a patient of FAP and referred for colectomy."}, {"id": "pubmed23n0594_1270", "title": "Colonoscopy for screening and follow up of patients with a family history of colorectal cancer.", "score": 0.015592767867977187, "content": "To determine the minimum family history of colorectal cancer (CRC), which justifies colonoscopy and to establish whether further colonic assessment is necessary after a negative screening colonoscopy. A retrospective review of every colonoscopy undertaken for family screening at the Royal Berkshire and Battle Hospitals, Reading between October 1996 and July 2004. Four hundred and thirty-two patients (261 women) with an average age of 48 years (range 14-84) were screened. Three cancers in patients over the age of 60 years and 49 adenomas were found in 37 patients. Twenty three of 281 (8%) patients with a 'low-risk' family history (one in 12 or less lifetime risk of developing CRC) had either a cancer or an adenoma. Eighteen of 151 (12%) patients with a 'high-risk' family history (one in 10 or greater) had a similar positive colonoscopy. Thirteen of 15 patients who had an adenoma aged under 45 years had a high-risk family history. Seventy-three patients subsequently underwent two or more follow-up colonoscopies. There were 22 adenomatous polyps found in 12 patients (16%) at the first screening, nine adenomas in seven patients in the second colonoscopy and four adenomas found in four patients in all subsequent colonoscopies. Patients with a low-risk family history have a similar adenoma pick-up to that of the general population. These patients need not be screened below the age of 50 unless symptomatic. Follow up of low-risk family history (FH) patients with a negative screening colonoscopy is unlikely to be beneficial."}, {"id": "pubmed23n0249_20615", "title": "[Effectiveness and costs of screening colonoscopy].", "score": 0.015228147333699834, "content": "Screening colonoscopy is always indicated when rectosigmoidoscopy reveals an adenoma, since this lesion roughly doubles the patient's risk of contracting colonic cancer. Follow-up should be performed at intervals of about three years after endoscopic removal of all colorectal polyps. Repeated screening examinations are recommended for the following genetic diseases that carry an increased risk of colorectal carcinoma: familial adenomatous polyposis (FAP) and its genetic variant, hereditary non-polyposis colorectal cancer syndrome (HNPCC) and hamartomatous polyposis syndromes (e.g. Peutz-Jeghers). Also in the case of familial \"sporadic\" carcinoma of the colon, regular screening colonoscopies for first degree relatives are recommended. Although the use of regular screening colonoscopies in patients with a long history of extensive ulcerative colitis is controversial, the recent results support such examinations. While the benefit of screening colonoscopy or sigmoidoscopy of the general population from the age of 50 onward must be affirmed, it should be weighed against the costs involved in such an undertaking. At the present time, the American Cancer Society recommends that from the age of 50 onward, the annual fecal test for occult blood should be supplemented by sigmoidoscopy performed every three to five years."}, {"id": "wiki20220301en186_40062", "title": "Colorectal polyp", "score": 0.014446444644464446, "content": "Several genes have been associated with polyposis, such as GREM1, MSH3, MLH3, NTHL1, RNF43 and RPS20. Familial adenomatous polyposis Familial adenomatous polyposis (FAP) is a form of hereditary cancer syndrome involving the APC gene located on chromosome q521. The syndrome was first described in 1863 by Virchow on a 15-year-old boy with multiple polyps in his colon. The syndrome involves development of multiple polyps at an early age and those left untreated will all eventually develop cancer. The gene is expressed 100% in those with the mutation and it is autosomal dominant. 10% to 20% of patients have negative family history and acquire the syndrome from spontaneous germline mutation. The average age of newly diagnosed patient is 29 and the average age of newly discovered colorectal cancer is 39. It is recommended that those affected undergo colorectal cancer screening at younger age with treatment and prevention are surgical with removal of affected tissues."}, {"id": "pubmed23n0044_2445", "title": "[Value of screening of familial adenomatous polyposis for the prevention of colorectal cancer].", "score": 0.01411764705882353, "content": "Familial adenomatous polyposis coli is a hereditary autosomal dominant disease which spontaneously and inevitably leads to degeneration of colorectal adenomas and requires preventive surgical treatment. The aim of this study was to evaluate the age of colorectal degeneration and the need for a screening technique in family members. Between 1983 and 1989, 141 patients were treated for familial adenomatous polyposis in our surgical center. Mean age at surgery was 32 years and 64 patients (45.4 percent) had a colorectal carcinoma. Thirty had an in situ tumor (mean age: 30 years) and 34 had an invasive adenocarcinoma (mean age: 45 years), 7 of whom died of their cancer. No colonic cancer was found in patients younger than 20. Thirty-eight percent of the patients under 40 years of age, 73 percent of the patients older than 40 years and 81 percent of those older than 50 had an adenocarcinoma. Fifty percent of the patients with carcinoma were younger than 40 years and 7 percent were less than 25 years old. Seventy-one patients were symptomatic at the time of operation (mean age: 40 years), 32 (45 percent) had a colonic cancer. In 70 patients, familial adenomatous polyposis was detected by screening (mean age: 24) and 2.8 percent had a colonic carcinoma. We conclude that the age-related risk of developing colonic carcinoma requires prophylactic surgery in asymptomatic patients before 20 years of age, and that routine familial screening would be of some benefit."}, {"id": "pubmed23n0339_597", "title": "Failure to diagnose hereditary colorectal cancer and its medicolegal implications: a hereditary nonpolyposis colorectal cancer case.", "score": 0.014043844196515953, "content": "We describe a patient who had precancerous colonic symptoms and a positive family history of multiple occurrences of early-onset colorectal cancer in her first-degree and second-degree relatives consistent with hereditary nonpolyposis colorectal cancer. Hereditary nonpolyposis colorectal cancer diagnosis had not been made before her diagnosis of carcinoma of the cecum with liver metastasis. She died at age 20, leading to litigation. Controversies about standards of care, their malpractice implications, and pertinent legal issues are discussed. Review of the medical and family history was made by the expert witness (HTL) with appropriate documentation of the chronology of symptoms, as derived from depositions. These documents revealed that the patient's mother had repeatedly discussed with the caregivers her concern about the family history of colon cancer and the need for appropriate surveillance. The patient's colonic symptoms progressed for a period of three years. Flexible sigmoidoscopy was performed by a nonphysician. The physician who ordered the procedure considered this appropriate because isolated polyps were reported in the patient's father and paternal uncle, which apparently led him to believe that the diagnosis was familial adenomatous polyposis. During litigation procedures, a pedigree was constructed and found to be consistent with hereditary nonpolyposis colorectal cancer. The case was settled in favor of the plaintiff before trial. It is essential to understand the natural history of hereditary nonpolyposis colorectal cancer, inclusive of the need for surveillance colonoscopy in patients at increased risk by virtue of their position in their family pedigree."}, {"id": "pubmed23n0262_21802", "title": "A screening clinic for relatives of patients with colorectal cancer in a district general hospital.", "score": 0.013937451437451436, "content": "A family cancer screening clinic was set up to screen and counsel subjects at above average risk of developing colorectal cancer. Criteria for referral were one first degree relative under 50 years or two of any age with colorectal cancer. Pedigree information was used to estimate lifetime risks of developing colorectal cancer and offer appropriate screening: colonoscopy for high risks (greater than 1 in 10), faecal occult blood testing for lower risks. One hundred and eleven subjects from 76 families were seen over four years. Forty two families gave a pedigree consistent with dominantly inherited non-polyposis colorectal cancer syndrome (HNPCC). Three subjects from one family were found to have familial adenomatous polyposis. Ninety two colonoscopies yielded 21 patients with polyps (12 had tubular adenomas, including one with early malignant invasion). Thirty three per cent (four of 12) of the tubular adenomas were beyond the reach of a flexible sigmoidoscope. Three hundred and forty two further high risk relatives were identified from the family history."}, {"id": "article-31161_20", "title": "Villous Adenoma -- Treatment / Management", "score": 0.013934621808637557, "content": "Average risk (no first-degree relative to colon cancer): Colonoscopy at age 50 No adenoma or carcinoma, repeat in 10 years One to two small (no more than 1 cm) tubular adenomas with low-grade dysplasia, repeat in 5 to 10 years Three to ten adenomas, or a large (at least 1 cm) adenoma, or any adenomas with high-grade dysplasia or villous features, repeat in 3 years. More than ten adenomas on a single exam, repeat within 3 years Increased risk (positive family history in the first-degree relative before age 60, or in two or more first-degree relatives at any age if not a hereditary syndrome) Colonoscopy at age 40, or 10 years before the youngest case in the immediate family (whichever is earlier). Repeat with above surveillance guidelines with the caveat that maximum time between screening should be 5 years. High-risk (hereditary colon cancer/polyposis syndromes): FAP: Annual flexible sigmoidoscopy; if genetically proven FAP, consider colectomy."}, {"id": "wiki20220301en010_160840", "title": "Colorectal cancer", "score": 0.013878406708595387, "content": "Those with a family history in two or more first-degree relatives (such as a parent or sibling) have a two to threefold greater risk of disease and this group accounts for about 20% of all cases. A number of genetic syndromes are also associated with higher rates of colorectal cancer. The most common of these is hereditary nonpolyposis colorectal cancer (HNPCC or Lynch syndrome) which is present in about 3% of people with colorectal cancer. Other syndromes that are strongly associated with colorectal cancer include Gardner syndrome and familial adenomatous polyposis (FAP). For people with these syndromes, cancer almost always occurs and makes up 1% of the cancer cases. A total proctocolectomy may be recommended for people with FAP as a preventive measure due to the high risk of malignancy. Colectomy, removal of the colon, may not suffice as a preventive measure because of the high risk of rectal cancer if the rectum remains. The most common polyposis syndrome affecting the colon is"}, {"id": "wiki20220301en605_1637", "title": "Serrated polyposis syndrome", "score": 0.013779128672745694, "content": "First degree relatives of people with SPS are at a higher risk of colorectal cancer and SPS. As such, these individuals should undergo screening with colonoscopy beginning at the earliest of the following: 40 years of age, the age of the youngest diagnosis of SPS in the family, or 10 years younger than the earliest CRC related to SPS. Repeat colonoscopy should be performed at 5 year intervals. Prognosis The overall risk of colorectal cancer is about 19.9%. However, the risk of cancer varies widely and depends on age, polyp burden, phenotype and the presence of dysplasia on histology. Endoscopic surveillance can decrease the risk of progression to cancer."}, {"id": "article-19735_8", "title": "Colonoscopy -- Indications", "score": 0.013550021105951878, "content": "Patients with a high risk of developing colorectal cancer receive the screening procedure before the age of 50 years, and it is repeated every 1, 2, or 5 years based upon the primary risk and findings during the procedure. Examples of high-risk populations include a history of inflammatory bowel disease, a family history of colorectal cancer at age <60 years, hereditary polyposis (Such as Peutz Jegher syndrome and Familial Adenomatous Polyposis, caused by an APC gene mutation) and non-polyposis syndromes (LYNCH I and II), and surveillance after resection of colorectal cancer. Individuals with first degree relatives diagnosed with colon cancer are encouraged to undergo their first colonoscopy at age 40, or 10 years prior to the age the relative was diagnosed, whichever comes first. Elective colonoscopy is performed for reasons such as known or occult gastrointestinal bleeding or stool positive for occult blood, unexplained changes in bowel habits, patterns, iron deficiency anemia or weight loss in elderly patients, persistent abdominal pain, suspected inflammatory or infectious colitis and barium enema showing radiographic structural abnormalities."}, {"id": "pubmed23n0588_2854", "title": "Familial adenomatous polyposis in children younger than age ten years: a multidisciplinary clinic experience.", "score": 0.012650427722198057, "content": "Children with familial adenomatous polyposis have a greater mortality and morbidity in the first decade of life compared with the general population. Some children with a more severe disease phenotype present early with colorectal adenomata and may require colectomy at an early age. We present our multidisciplinary clinic experience with familial adenomatous polyposis in children younger than age ten years at the time of presentation. A cross-sectional analysis was performed on all patients with suspected or confirmed familial adenomatous polyposis presenting in the first decade of life and followed by the multidisciplinary Pediatric Hereditary Polyposis Clinic at our institutions. Analysis included demographics, clinical presentation and course, gene mutation testing, endoscopic-histologic findings, and surgical outcome. Twenty-two children (11 males) presented with suspected or confirmed familial adenomatous polyposis. Two were discharged from follow-up after negative adenomatous polyposis coli gene mutation testing. The rest underwent annual hepatoblastoma surveillance through age ten years with negative findings. Twelve patients presented with symptoms: six had de novo familial adenomatous polyposis. Seven had gastrointestinal hemorrhage and went on to colonoscopy. Four patients with adenomatous polyposis coli gene mutation at codon 1309 were referred for colectomy before age ten years. Referral to colectomy was earlier in patients with 1309 mutation and with de novo familial adenomatous polyposis. Children with familial adenomatous polyposis younger than age ten years may present presymptomatically for disease surveillance. Familial adenomatous polyposis with adenomatous polyposis coli gene mutation at codon 1309 entails a risk of a more aggressive phenotype; early colectomy may be indicated in children harboring this gene mutation."}, {"id": "pubmed23n0500_23027", "title": "Management of Portuguese patients with hyperplastic polyposis and screening of at-risk first-degree relatives: a contribution for future guidelines based on a clinical study.", "score": 0.012353210309592803, "content": "Hyperplastic polyposis (HP) is a rare condition characterized by the presence of multiple hyperplastic polyps in the colon, which has been associated to an increased risk of colorectal cancer (CRC). Guidelines for management of this disease remain, so far, undefined. To evaluate, in symptomatic patients with HP, phenotypic characteristics as well as results of a screening program in their at-risk first-degree relatives. Pedigree information and clinical and endoscopic data of 14 patients with HP was studied. SEVENTEEN AND METHODS: at-risk first-degree relatives from six families were also invited to perform screening colonoscopy. Twelve of fourteen (86%) patients had fewer than 100 colorectal polyps. Polyps' sizes ranged from 2 to 25 mm and were uniformly distributed through the whole colon in 43% of the patients. Hyperplastic polyps predominated, but 11/14 (79%) patients also harbored serrated as well as classic adenomatous polyps. CRC was present in 6/14 (43%) of the patients at the time of diagnosis. Familial history of CRC/polyps was positive in 6/12 (50%) of cases. Colonoscopy in at-risk relatives disclosed polyps in 10/17 (59%) of cases with at least one additional patient having criteria for HP. Although small, this series demonstrates that a high level of suspicion is needed to diagnose the HP syndrome, in which serrated adenomas seem to be the hallmark. Although an elevated percentage of CRC was observed in this series of symptomatic patients with HP, prospective studies in asymptomatic individuals are needed to clearly quantify the risk of CRC in patients with HP. Because familial aggregation of HP was present in 3/12 (25%) of kindreds, screening colonoscopy should be offered to first-degree relatives."}, {"id": "pubmed23n0782_4878", "title": "The natural history of familial adenomatous polyposis syndrome: a 24 year review of a single center experience in screening, diagnosis, and outcomes.", "score": 0.012167982770997846, "content": "Understanding the natural history of Familial Adenomatous Polyposis (FAP) will guide screening and aid clinical management. Patients with FAP, age ≤20years presenting between 1987 and 2011, were reviewed for presentation, diagnosis, extraintestinal manifestations, polyp burden, family history, histology, gene mutation, surgical intervention, and outcome. One hundred sixty-three FAP patients were identified. Diagnosis was made by colonoscopy (69%) or genetic screening (25%) at mean age of 12.5years. Most children (58%) were asymptomatic and diagnosed via screening due to family history. Rectal bleeding was the most common (37%) symptom prompting evaluation. Colon polyps appeared by mean age of 13.4years with &gt;50 polyps at the time of diagnosis in 60%. Cancer was found in 1 colonoscopy biopsy and 5 colectomy specimens. Family history of FAP was known in 85%. 53% had genetic testing, which confirmed APC mutation in 88%. Extraintestinal manifestations included congenital hypertrophy of the retinal pigment epithelium (11.3%), desmoids (10.6%), osteomas (6.7%), epidermal cysts (5.5%), extranumerary teeth (3.7%), papillary thyroid cancer (3.1%), and hepatoblastoma (2.5%). Six patients died secondary to FAP. Clinical presentation and manifestations in pediatric FAP are variable. We suggest an individualized patient-oriented screening algorithm that allows for earlier screening and appropriate management."}, {"id": "Surgery_Schwartz_8538", "title": "Surgery_Schwartz", "score": 0.011921891058581706, "content": "of first-degree relatives of FAP patients beginning at age 10 to 15 years has been the traditional mainstay of screening. Today, following genetic counseling, APC gene testing may be used to screen family members, pro-viding an APC mutation has been identified. If APC testing is positive in a relative of a patient with a known APC mutation, annual flexible sigmoidoscopy beginning at age 10 to 15 years is done until polyps are identified. If APC testing is negative, the relative can be screened starting at age 50 years per average-risk guidelines. If APC testing is refused or unavailable, or if a mutation cannot be identified, annual flexible sigmoidoscopy beginning at age 10 to 15 years is performed until age 24 years. Screening flexible sigmoidoscopy is then done every 2 years until age 34 years, every 3 years until age 44 years, and then every 3 to 5 years.FAP patients are also at risk for the development of adeno-mas anywhere in the gastrointestinal tract, particularly in the"}, {"id": "pubmed23n0604_23206", "title": "Colonic adenoma risk in familial colorectal cancer--a study of six extended kindreds.", "score": 0.011858797573083286, "content": "Most colorectal cancers (CRCs) arise from adenomatous polyps, but the effects of CRC family history on adenoma risk are not well known. This issue is clinically relevant since several medical societies currently recommend earlier and more rigorous colorectal screening for individuals with a strong family history of CRC. Colonoscopies were performed in 236 first-, second-, and third-degree relatives of 40 index CRC cases from six large kindreds selected from a large population database. The kindreds were selected for significantly greater risk of CRCs compared with the overall population. Known hereditary colon cancer syndromes were clinically and genetically excluded. Thirty-seven percent of relatives were found to have adenomas on colonoscopy. The average age of diagnosis for colon cancer was 63 yr and advanced adenomas 56 yr. Independent predictors of adenomatous polyps in the relatives were advancing age (P &lt; 0.0001), male gender (P &lt; 0.001), and greater degree of relation to CRC cases (P &lt; 0.01). There was no significant predilection of colorectal tumors for the right or left colon. A higher degree of relationship to CRC cases was a significant predictor of having simple and advanced adenomas, but not hyperplastic polyps after adjustment for age and gender. These data support the current recommendations for colonoscopy starting before the age of 50 yr in individuals with a strong family history of CRC."}, {"id": "pubmed23n0320_511", "title": "Family history of colorectal adenomatous polyps and increased risk for colorectal cancer.", "score": 0.011599530947928881, "content": "The risk for colorectal cancer among family members of patients with colorectal cancer is well established, but the risk among family members of patients with colorectal adenomas is less well established. To examine the risk for colorectal cancer among first-degree relatives of patients with adenoma compared with that among first-degree relatives of controls without adenoma. Reconstructed cohort study. Three university-based colonoscopy practices in New York City. 1554 first-degree relatives of 244 patients with newly diagnosed adenomas and 2173 first-degree relatives of 362 endoscopically normal controls. Structured interviews were used to obtain family history. Adjusted relative risks (RR) were estimated from Cox proportional hazards regression models. The risk for colorectal cancer was elevated (RR, 1.74 [95% CI, 1.24 to 2.45]) among first-degree relatives of patients with newly diagnosed adenomas compared with the risk among first-degree relatives of controls. This increased risk was the same for parents (RR, 1.58 [CI, 1.07 to 2.34]) and siblings (RR, 1.58 [CI, 0.81 to 3.08]). First-degree relatives of patients with adenomas did not have elevated risk for other cancers. The risk for colorectal cancer among family members increased with decreasing age at diagnosis of adenoma in probands. Among first-degree relatives of patients who were 50 years of age or younger when the adenoma was diagnosed, the risk was more than four times greater (RR, 4.36 [CI, 2.24 to 8.51]) than that among first-degree relatives of patients who were older than 60 years of age when the adenoma was diagnosed. First-degree relatives of patients with newly diagnosed adenomas, particularly of patients who are 50 years of age or younger at diagnosis, are at increased risk for colorectal cancer and should undergo screening similar to that recommended for relatives of patients with colorectal cancer."}, {"id": "pubmed23n0547_6951", "title": "What is the appropriate screening protocol in Lynch syndrome?", "score": 0.01146384479717813, "content": "Lynch syndrome families have a substantial risk of developing colorectal cancer (CRC). The recommended surveillance protocol includes colonoscopy every 2 years from age 20-25 years. It is yet unknown whether annual screening of patients aged 40-60 years is more effective than bi-annual screening, whether patients who had an adenoma removed should be re-examined after a year and whether surveillance of second-degree relatives is indicated. The aim of this study was to address these issues. All carriers of a mismatch repair gene mutation who participated in the surveillance program were selected from the Dutch Lynch syndrome registry. The results of colonoscopy were prospectively collected. A total of 666 mutation carriers were identified in 110 families. Fourty-one CRCs were detected during endoscopic follow-up, of which 34 (83%) were diagnosed between age 40 and 60 years. In five of 34 patients, CRC was diagnosed within 1 year after colonoscopy, eight cancers were diagnosed between 1 and 2 years and the remaining tumors more than 2 years after colonoscopy. All eight CRCs detected between 1 and 2 years were at local stage. At least one adenoma was diagnosed at 141 examinations. The risk of developing CRC during follow-up in carriers with an adenoma was similar as in carriers without an adenoma at the previous colonoscopy. 280 parent-child couples with at least one Lynch syndrome-related carcinoma were identified in 110 families. In only 19 (6.8%) of these couples, CRC developed earlier in the child than an Lynch syndrome-associated cancer in the parent. The current surveillance protocol, i.e., bi-annual colonoscopy in first-degree relatives independent of age and endoscopic findings, appears to be appropriate."}, {"id": "wiki20220301en245_7341", "title": "Attenuated familial adenomatous polyposis", "score": 0.011441974884865128, "content": "Attenuated familial adenomatous polyposis is a form of familial adenomatous polyposis, a cancer syndrome. It is a pre-malignant disease that can develop into colorectal cancer. A patient will have fewer than a hundred polyps located typically in right side of the colon. Cancer might develop as early as the age of five, though typically presents later than classical FAP. See also Familial adenomatous polyposis Birt–Hogg–Dubé syndrome Cowden syndrome Cronkhite–Canada syndrome Juvenile polyposis MUTYH Peutz–Jeghers syndrome References External links Gastrointestinal cancer Hereditary cancers"}, {"id": "wiki20220301en034_81317", "title": "Familial adenomatous polyposis", "score": 0.011289429994465966, "content": "Epidemiology The incidence of the mutation is between 1 in 10,000 and 1 in 15,000 births. By age 35 years, 95% of individuals with FAP (>100 adenomas) have polyps. Without colectomy, colon cancer is virtually inevitable. The mean age of colon cancer in untreated individuals is 39 years (range 34–43 years). Attenuated FAP arises when APC is defective but still somewhat functional. As a result, it retains part of its ability to suppress polyps. Therefore, attenuated FAP manifests as colorectal cancer unusually late (age 40–70, average=55), and typically with few, or at least far fewer polyps (typically 30), than the more usual version of FAP, at an age when FAP is no longer considered much of a likelihood or risk according to usual FAP epidemiology. Comparison of FAP variants This table compares the different subtypes of FAP:"}, {"id": "pubmed23n0421_3681", "title": "Screening for familial colorectal cancer: the need for continuing education. A case report.", "score": 0.011152761857554448, "content": "A 37 year old female patient was diagnosed with sigmoid colon cancer in our clinic five years ago (January 1998). The family history revealed three deaths due to colorectal cancer (maternal grandmother, mother's sister and patient's sister), and the patient's mother had been diagnosed with adenomatous polyps (endoscopically removed). Histopathological diagnosis was moderate / poorly differentiated adenocarcinoma. Resection of sigmoid colon was performed and adjuvant chemotherapy was carried out, with uneventful evolution. The patient was annually followed-up (colonoscopy, abdominal ultrasound, laboratory tests). The last admission was in January 2003. No recurrence or metastases were found. The patient's mother, who was admitted at the same time, had been diagnosed with urinary bladder tumor. Subjects with a personal or family history of colorectal cancer should routinely have a colonoscopy beginning from age 40 or earlier. It is important for such patients to be followed-up closely not only for recurrence or metastases, but also for detection and treatment of a second primary cancer at an early stage"}, {"id": "pubmed23n0511_18072", "title": "Clinical identification and long-term surveillance of 22 hereditary non-polyposis colon cancer Italian families.", "score": 0.01105276013678554, "content": "To assess the efficacy of a hereditary non-polyposis colon cancer (HNPCC) identification and surveillance policy. Familial clustering of colorectal cancer (CRC) and extracolonic cancers (ECs) was investigated in 1520 consecutive CRC patients and relatives. HNPCC was identified by Amsterdam criteria, and individuals at risk were offered biennial colonoscopy and other examinations, starting from age 25 years. Twenty-two HNPCC families were identified. The CRC prevalence was 27.8% (121/435), decreasing from 59.4% in the first generation to 24.4% and 8% in the second and third generation, respectively. Twenty-nine patients had multiple CRC and 34 patients (in 12 families) had ECs.A total of 199/331 at-risk individuals accepted surveillance. The mean follow-up was 48+/-32 months. CRCs were detected at first surveillance in four out of 199 surveilled individuals (2%); in two surveilled individuals (1%), three CRCs developed during follow-up. The overall CRC incidence was 7/199 (3.5%) in surveilled individuals and 5/132 (3.7%) in unsurveilled individuals. CRCs were less advanced in surveilled than in unsurveilled patients. Eleven individuals had 22 adenomas (one with high-grade dysplasia). Three individuals had adenomas at first surveillance; two of them and eight more individuals during surveillance. Seven surveilled individuals and six unsurveilled individuals, all belonging to families with a history of EC, had EC during the study period. All patients with CRC detected by surveillance are alive. One of the unsurveilled patients who had CRC died 18 months after the diagnosis. Data confirm the importance of the family history collected in each patient with CRC for identification of HNPCC and support the efficacy of repeated colonoscopies for early diagnosis and prevention of CRC in at-risk members. Reasons for surveillance failure could be an accelerated progression of small adenomas and a lesion missing at colonoscopy. Longer follow-up is required to assess the efficacy of surveillance for EC."}, {"id": "pubmed23n0522_7906", "title": "Risk stratification for colorectal cancer and implications for screening.", "score": 0.010977506580521657, "content": "In addition to the well-recognized syndromes described (FAP, HNPCC) clusters of colorectal cancers occur in families much more often than would be expected by chance. This familial clustering in about 10-20% of colorectal cancers has implications for screening because the immediate family members of a patient with apparent sporadic colorectal cancer have a twofold to threefold increased risk of the disease. The magnitude of the risk depends on the age at diagnosis of the index case, the degree of kinship of the index case to the at-risk case, and the number of affected relatives. In addition to screening the easily identifiable high-risk groups such as FAP and HNPCC, care should be taken to recognize intermediate-risk patients and to provide them with appropriate screening recommendations. Because the molecular basis and the natural history of these intermediate-risk patients are largely unknown, screening recommendations are as yet more empirical. If a person has a first degree relative with colon cancer, average risk colon cancer screening is recommended, but starting at age 40 years. The decreased age is given because the risk at age 40 for those with an affected first-degree relative is similar to the risk at age 50 for the general population. An individual with two first-degree relatives affected with colon cancer or one first-degree relative diagnosed under the age of 60 y should have colonoscopy beginning at age 40, or 10 years younger than the earliest case in the family. Colonoscopy should be repeated every five years if negative. An even stronger family history of colon cancer syndromes of colon cancer should suggest the consideration of one of the inherited syndromes."}, {"id": "pubmed23n1010_23124", "title": "Colorectal cancer screening for patients with a family history of colorectal cancer or adenomas.", "score": 0.01093418576165865, "content": "To review and summarize the recently developed Canadian Association of Gastroenterology screening recommendations for patients with a family history of colorectal cancer (CRC) or adenoma from a family medicine perspective. A systematic review and meta-analysis was performed to synthesize knowledge regarding family history and CRC. The Cochrane Central Register of Controlled Trials, MEDLINE, and EMBASE were searched with the following MeSH terms: <icolorectal cancers or neoplasms, screen or screening or surveillance,</i and <ifamily or family history.</i Known hereditary syndromes were excluded. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to establish certainty in reviewed evidence. Most recommendations are conditional recommendations with very low-quality evidence. Individuals who have 1 first-degree relative (FDR) with CRC or an advanced adenoma diagnosed at any age are recommended to undergo colonoscopy every 5 to 10 years starting at age 40 to 50 years or 10 years younger than the age at diagnosis of the FDR, although fecal immunochemical testing at an interval of every 1 to 2 years can be used. Individuals with FDRs with non-advanced adenomas or a history of CRC in second-degree relatives should be screened according to average-risk guidelines. Lifestyle modification can statistically significantly decrease risk of CRC and should be considered in all patients. These guidelines acknowledge the many factors that can increase an individual's risk of developing CRC and allow for judgment to be employed depending on the clinical scenario. Lifestyle advice already given to patients for weight, blood pressure, and heart disease management will reduce the risk of CRC if implemented, and this combined with more targeted screening for higher-risk individuals will hopefully be successful in decreasing CRC mortality in Canada."}, {"id": "wiki20220301en014_100820", "title": "Colonoscopy", "score": 0.010890173410404625, "content": "Subsequent rescreenings are then scheduled based on the initial results found, with a five- or ten-year recall being common for colonoscopies that produce normal results. People with a family history of colon cancer are often first screened during their teenage years. Among people who have had an initial colonoscopy that found no polyps, the risk of developing colorectal cancer within five years is extremely low. Therefore, there is no need for those people to have another colonoscopy sooner than five years after the first screening. Some medical societies in the US recommend a screening colonoscopy every 10 years beginning at age 50 for adults without increased risk for colorectal cancer. Research shows that the risk of cancer is low for 10 years if a high-quality colonoscopy does not detect cancer, so tests for this purpose are indicated every ten years."}, {"id": "pubmed23n0479_144", "title": "Colonoscopy surveillance of individuals at risk of familial colorectal cancer.", "score": 0.010836897468500752, "content": "Individuals with first degree relatives affected with colorectal cancer (CRC) at a young age, or more than one relative affected but who do not fulfil the Amsterdam criteria for a diagnosis of hereditary non-polyposis colon cancer (HNPCC), are believed to be at an increased risk of CRC. However, there is a paucity of prospective data on the potential benefit of colonoscopic surveillance in such groups categorised by empiric family history criteria. We report a prospective study of 448 individuals seeking counselling about their perceived family history of CRC. Following pedigree tracing, verification, and risk assignment by genetic counsellors, colonoscopy was undertaken for those at a moderate or high risk (HNPCC). Those classified as low risk were reassured and discharged without surveillance. Here we report our findings at the prevalence screen in the 176 patients of the 448 assessed who underwent colonoscopy. Fifty three individuals had a family history that met Amsterdam criteria (median age 43 years) and 123 individuals were classed as moderate risk (median age 43 years). No cancers were detected at colonoscopy in any group. Four individuals (8% (95% confidence limits (CL) 0.4-15%)) in the high risk group had an adenoma detected at a median age of 46 years and all four were less than 50 years of age. Five (4% (95% CL 0.6- 8%)) of the moderate risk individuals had an adenoma at a median age of 54 years, two of whom were less than 50 years of age. These findings indicate that the prevalence of significant neoplasia in groups defined by family history is low, particularly in younger age groups. These prospective data call into question the value of colonoscopy before the age of 50 years in moderate risk individuals."}, {"id": "Surgery_Schwartz_8549", "title": "Surgery_Schwartz", "score": 0.010327570518653321, "content": "history of this disease (average-risk population) is approximately 6%, but rises to 12% if one first-degree relative is affected and to 35% if two first-degree relatives are affected. Age of onset also impacts risk, and a diagnosis before the age of 50 years is associated with a higher incidence in family members. Screening colonoscopy is recommended every 5 years beginning at age 40 years or begin-ning 10 years before the age of the earliest diagnosed patient in the pedigree. While there are no specific genetic abnormalities that are associated with familial colorectal cancer, any of the defects found in either the LOH pathway or MSI pathway may be present in these patients.Prevention: Screening and SurveillanceBecause the majority of colorectal cancers are thought to arise from adenomatous polyps, preventive measures focus on identi-fication and removal of these premalignant lesions. In addition, many cancers are asymptomatic, and screening may detect these tumors at an early and"}, {"id": "pubmed23n0515_6258", "title": "A scoring system for the strength of a family history of colorectal cancer.", "score": 0.010012265024655203, "content": "Family history of colorectal cancer is associated with an increased risk for the disease, although there are many combinations of family history that are hard to correlate with risk status. A scoring system for family history of colorectal cancer was designed to make risk more readily quantifiable. A colonoscopy database was used to test the following points system: each first-degree relative with colorectal cancer = 3 points; each second-degree relative with colorectal cancer = 1 point. Families with one or more first-degree relative affected under 50 years of age = an extra 3 points. Families with one or more second-degree relative affected under 50 years of age = an extra 1 point. Families with multiple relatives on the same side of the family = an extra 3 points (first-degree relatives), 1 point (second-degree relatives), or 2 points (first-degree and second-degree relatives). Points were added and categories defined as follows: low risk, 1 to 4 points; medium risk, 5 to 7 points; high risk, 8 to 10 points; very high risk, &gt;10 points. A control group of average-risk patients having screening colonoscopy was used. Categories were compared in number of adenomas, hyperplastic polyps, and cancers. The records of 992 patients were used to test the system. Mean adenomas per patient per group were 0.4 for controls, 1.0 for low risk, 1.0 for medium risk, 1.7 for high risk, and 1.7 for very high risk. Cancers per group were 2 of 196 for controls, 8 of 513 for low risk, 3 of 171 for medium risk, 3 of 84 for high risk, and 1 of 28 for very high risk. The score categories were combined to produce revised risk levels of low (score 1 to 7) and high (&gt;7). Average adenomas per patient in the revised categories were 0.4 (control), 1.0 (low risk), and 1.7 (high risk). The odds ratio of having one to two adenomas was 1.73 (1.19-2.50, 95% confidence limits) in the low-risk group and 2.39 (1.41-4.01) in the high-risk group. Odds ratios for having three or more adenomas were 5.70 (2.44-13.32) in the low-risk group and 10.35 (3.97-26.97) in the high-risk group. In the two-category system proposed here of quantifying familial risk of colorectal cancer, patients having less than 8 points were at low risk and those with 8 or more were at high risk. Surveillance and chemoprevention protocols can be designed through use of these risk categories. A scoring system for family history of colorectal cancer can make risk assessment easier and facilitate both collaborative studies and patient triage into appropriate screening programs."}, {"id": "pubmed23n0491_5611", "title": "First-degree relatives of patients with advanced colorectal adenomas have an increased prevalence of colorectal cancer.", "score": 0.009930437774992012, "content": "The risk of colorectal cancer in relatives of patients with adenomatous colonic polyps is not well defined. This study assessed whether finding colonic neoplasia during screening colonoscopy was related to the family history of colorectal cancer among the participants' parents and siblings. Self-reported family history of colorectal cancer was recorded for all participants in a screening colonoscopy study. The size and location of all polyps were recorded before their removal and histologic examination. Participants were grouped according to the most advanced lesion detected. Three thousand one hundred twenty-one patients underwent complete colonoscopic examination. Subjects with adenomas were more likely to have a family history of colorectal cancer than were subjects without polyps (odds ratio [OR], 1.36; 95% confidence interval [CI], 1.09-1.70). The finding of a small (&lt;1 cm) tubular adenoma as the most advanced lesion was associated with only a modest increase in the OR of colorectal cancer in family members (OR, 1.26; 95% CI, 0.99-1.61), but the presence of an advanced adenoma was associated with a higher OR (OR, 1.62;5% CI, 1.16-2.26). Younger age of adenoma diagnosis was not related to a higher prevalence of a family history of colorectal cancer. Relatives patients with advanced colorectal adenomas have an increased risk of colorectal cancer. Individuals with advanced colorectal adenomas should be counseled about the increased risk of colorectal cancer among their relatives."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[468, 616]], "word_ranges": [[79, 106]], "text": "Between 1 and 3, I choose 3 for the same reason and because of the emphasis on the decline of functional independence that occurs in these patients."}, "2": {"exist": true, "char_ranges": [[174, 467]], "word_ranges": [[28, 79]], "text": "There are behavioral variants, but in the MIR they usually want you to think of other types of dementias in the face of behavioral symptoms. In fact, the appearance of these symptoms in early stages should alert us and open the range of diagnostic possibilities. Therefore, we discard 2 and 4."}, "3": {"exist": true, "char_ranges": [[468, 616]], "word_ranges": [[79, 106]], "text": "Between 1 and 3, I choose 3 for the same reason and because of the emphasis on the decline of functional independence that occurs in these patients."}, "4": {"exist": true, "char_ranges": [[174, 467]], "word_ranges": [[28, 79]], "text": "There are behavioral variants, but in the MIR they usually want you to think of other types of dementias in the face of behavioral symptoms. In fact, the appearance of these symptoms in early stages should alert us and open the range of diagnostic possibilities. Therefore, we discard 2 and 4."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "I answer this question thinking more about what they want to ask than what they are really asking. There is heterogeneity in the development of Alzheimer's disease symptoms. There are behavioral variants, but in the MIR they usually want you to think of other types of dementias in the face of behavioral symptoms. In fact, the appearance of these symptoms in early stages should alert us and open the range of diagnostic possibilities. Therefore, we discard 2 and 4. Between 1 and 3, I choose 3 for the same reason and because of the emphasis on the decline of functional independence that occurs in these patients.", "full_answer_no_ref": "I answer this question thinking more about what they want to ask than what they are really asking. There is heterogeneity in the development of Alzheimer's disease symptoms. There are behavioral variants, but in the MIR they usually want you to think of other types of dementias in the face of behavioral symptoms. In fact, the appearance of these symptoms in early stages should alert us and open the range of diagnostic possibilities. Therefore, we [HIDDEN] Between 1 and 3, I [HIDDEN] for the same reason and because of the emphasis on the decline of functional independence that occurs in these patients.", "full_question": "A 75-year-old woman brought for consultation by her family because they have been finding her depressed and with memory lapses for months. They are concerned that she may have Alzheimer's disease. The patient refers that she does not think anything is wrong with her and that she is as usual. In what order do the following symptoms generally occur in the progression of Alzheimer's disease:", "id": 573, "lang": "en", "options": {"1": "Mood changes, behavioral symptoms, cognitive deficits.", "2": "Behavioral symptoms, motor symptoms, decline of functional independence.", "3": "Mood changes, cognitive deficit, decline of functional independence.", "4": "Behavioral symptoms, mood changes, motor symptoms.", "5": NaN}, "question_id_specific": 92, "type": "NEUROLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en023_16498", "title": "Behavioral neuroscience", "score": 0.01386924406726387, "content": "Parkinson's disease, a degenerative disorder of the central nervous system that often impairs the sufferer's motor skills and speech. Huntington's disease, a rare inherited neurological disorder whose most obvious symptoms are abnormal body movements and a lack of coordination. It also affects a number of mental abilities and some aspects of personality. Alzheimer's disease, a neurodegenerative disease that, in its most common form, is found in people over the age of 65 and is characterized by progressive cognitive deterioration, together with declining activities of daily living and by neuropsychiatric symptoms or behavioral changes. Clinical depression, a common psychiatric disorder, characterized by a persistent lowering of mood, loss of interest in usual activities and diminished ability to experience pleasure."}, {"id": "wiki20220301en231_13405", "title": "Alzheimer's disease", "score": 0.01382547471730318, "content": "Alzheimer's disease (AD) is a neurodegenerative disease that usually starts slowly and progressively worsens. It is the cause of 60–70% of cases of dementia. The most common early symptom is difficulty in remembering recent events. As the disease advances, symptoms can include problems with language, disorientation (including easily getting lost), mood swings, loss of motivation, self-neglect, and behavioral issues. As a person's condition declines, they often withdraw from family and society. Gradually, bodily functions are lost, ultimately leading to death. Although the speed of progression can vary, the typical life expectancy following diagnosis is three to nine years."}, {"id": "pubmed23n0311_1380", "title": "Noncognitive disturbances in Alzheimer's disease: frequency, longitudinal course, and relationship to cognitive symptoms.", "score": 0.013085870680807389, "content": "To investigate the frequency and longitudinal course of symptoms of depression, agitation, and psychosis in a longitudinally studied sample of patients with Alzheimer's disease (AD). Longitudinal study of AD patients with follow-up assessments at 6-month intervals for an average of more than 3 years. Alzheimer's Disease Research Center of the Mount Sinai Medical Center and the Bronx VA Medical Center, New York. A total of 153 AD patients. Blessed Test of Information, Memory and Concentration (BIMC) and the Alzheimer's Disease Assessment Scale cognitive (ADAS-Cog) and noncognitive (ADAS-NC) subscales. At entry into the study, more than 90% of patients had a behavioral disturbance that was rated as mild or worse on one of the 10 ADAS noncognitive items; and 40% had at least one rating that was moderate or severe. Correlational analyses indicated that, with the exception of the two mood-related items, noncognitive symptoms on the ADAS were not highly correlated with one another. Only one of the noncognitive items, concentration, was strongly correlated with the severity of cognitive impairment. On average, patients showed progressively worse cognitive functioning over time as measured both by the ADAS-Cog and the BIMC. The mean severity of noncognitive symptoms did not change during the course of a 5-year follow up. The severity of behavioral disturbance at any one evaluation was negatively correlated with change in behavior during the next 6 months and was not correlated with cognitive decline. Mild behavioral disturbances are common, whereas moderate to severe behavioral symptoms are less frequent in this population of AD patients. Disturbances in mood and manifestations of agitation and psychotic symptoms are not closely related to one another and show little progressive worsening over time. Rather, they tend to be episodic such that increasing severity at one time is usually followed by improvement later. Concentration problems are a manifestation of cognitive dysfunction rather than behavioral disturbance in AD. Implications of these results for treatment of noncognitive disturbances in AD are discussed."}, {"id": "wiki20220301en001_276310", "title": "Dementia", "score": 0.012762501450284255, "content": "Dementia manifests as a set of related symptoms, which usually surface when the brain is damaged by injury or disease. The symptoms involve progressive impairments in memory, thinking, and behavior, which negatively impact a person's ability to function and carry out everyday activities. Aside from memory impairment and a disruption in thought patterns, the most common symptoms include emotional problems, difficulties with language, and decreased motivation. The symptoms may be described as occurring in a continuum over several stages. Consciousness is not affected. Dementia ultimately has a significant effect on the individual, caregivers, and relationships in general. A diagnosis of dementia requires a change from a person's usual mental functioning and a greater cognitive decline than what is caused by normal aging. Several diseases and injuries to the brain, such as a stroke, can give rise to dementia. However, the most common cause is Alzheimer's disease, a neurodegenerative"}, {"id": "wiki20220301en001_10080", "title": "Neuron", "score": 0.01212797619047619, "content": "Alzheimer's disease (AD), also known simply as Alzheimer's, is a neurodegenerative disease characterized by progressive cognitive deterioration, together with declining activities of daily living and neuropsychiatric symptoms or behavioral changes. The most striking early symptom is loss of short-term memory (amnesia), which usually manifests as minor forgetfulness that becomes steadily more pronounced with illness progression, with relative preservation of older memories. As the disorder progresses, cognitive (intellectual) impairment extends to the domains of language (aphasia), skilled movements (apraxia), and recognition (agnosia), and functions such as decision-making and planning become impaired."}, {"id": "pubmed23n0314_8660", "title": "[A longitudinal study of the course of Alzheimer type dementia].", "score": 0.009900990099009901, "content": "We carried out a prospective longitudinal study of 41 patients diagnosed as probably having a mild form of Alzheimer's disease according to the data of an investigation of prevalence done in 1991 in the municipality of Habana Vieja. The research covered two phases, with an interval of one year between them. During both phases the patients were interviewed by the same neurology resident in a door-to-door survey using the Mini-Mental State, Hughes scale (CDR) and Blessed scale, to evaluate higher mental functions. We determined the progress of the disease over the course of two years (1991-1992 and 1992-1993) and the frequency and degree of deterioration of cognitive functions during a period of one year. There was evidence of progressive worsening of the illness in 46.4% of the patients. In 34.2% this was to a moderate form and in 12.2% to a severe form. There was no progression in 46.3%. In this group 17.0% continued with a diagnosis of doubtful dementia and 29.3% as having slight dementia. The other 7.3% of the total number of patients (n = 41) were reclassified as normal. Cognitive functions almost always showed a tendency to deteriorate over time, but in a small percentage of patients they did not deteriorate and some even improved. The cognitive functions which deteriorated most were those of orientation, language and copying, with an average deterioration of 28% and 24% respectively with regard to their initial values."}, {"id": "pubmed23n0644_19348", "title": "Report of ten years' activity in an Alzheimer's disease assessment unit.", "score": 0.00980392156862745, "content": "After ten years' treatment with cholinesterase inhibitors (AcheI) in Alzheimer's disease (AD), we report here the activity of the Alzheimer's Disease Assessment Unit of IRCCS C. Mondino, Pavia, Italy. From September 2000 to December 2007, 794 out-patients (of 2236 referred to our Assessment Unit for cognitive disturbances) with AD of mild to moderate degree were treated with cholinesterase inhibitors (M/F: 273/521, mean age 73.6+/- 8.4 yrs, range 52-85 yrs). Outcome measures were scores on Mini Mental State Examination (MMSE), ADL, IADL and Neuropsychiatric Inventory (NPI). Mean treatment duration was 36.9+/-16.1 months. After three months' treatment, MMSE scores remained stable (responders) in 60% of cases and improved (increase of 3 or more points - good responders) in 15%, with good preservation of autonomy. After 15 months, the percentage of \"good responders\" decreased to 7%, while after 15, 27 and 39 months the percentage of responders progressively decreased to 40%, 30% and 8%, respectively; greater impairment in instrumental with respect to basic everyday activities was noted. No variables capable of predicting the response to treatment were detected. The onset of behavioral disturbances caused significant (p&lt;0.02) worsening of both cognition and function and, in 12% of cases, suspension of treatment. Our results therefore confirm the efficacy of AcheI in AD of mild to moderate degree even in a nonselected population; efficacy also seems to persist in long-term treatment. This report, although brief and mainly descriptive, can make a contribution to better knowledge of the usefulness of these drugs in AD of mild to moderate extent in everyday clinical practice."}, {"id": "pubmed23n0750_19272", "title": "The influences of psychotic symptoms on the activities of daily living of individuals with Alzheimer disease: a longitudinal analysis.", "score": 0.009770787295810044, "content": "Psychotic symptoms associated with Alzheimer Disease (AD) contribute to excess functional dependence. Longitudinal studies have generally examined the association between rates of functional decline and the occurrence of psychotic symptoms from either a single evaluation or from multiple evaluations rather than through changes in frequency and severity of symptoms. Although the presence or absence of psychotic symptoms at initial or follow-up examinations may be associated with changes in functional status, the nature of the relationship between changes in these domains cannot be inferred. We examine the association between changes in the frequency of psychotic symptoms and changes in dependence in activities of daily living (ADL) over a period ranging from 1 to 74 months (median = 17.7). Data from a cohort of 234 individuals referred to a memory clinic were analyzed using multilevel linear regression. Information on ADL, behavioral and psychological symptoms, depression, and cognition was collected. An increase in the frequency of psychotic symptoms had a unique influence on the deterioration of basic ADL, after controlling for demographic variables, changes in cognition, depression, and other behavioral and psychological symptoms (B = -.017, p = .003). However, changes in psychotic symptoms did not significantly contribute to declines in the ability to perform instrumental ADL (B = -.008, p = .439). Changes in psychotic symptoms may influence basic but not instrumental ADL over time. These findings may have ramifications for studies and treatment plans for individuals with AD who demonstrate psychotic symptoms."}, {"id": "wiki20220301en318_25497", "title": "Variably protease-sensitive prionopathy", "score": 0.009708737864077669, "content": "Patients present with behavioral and psychiatric symptoms, speech deficits (aphasia and/or dysarthria) and progressive cognitive and motor decline (dementia, ataxia, parkinsonism, psychosis, aphasia and mood disorder). Average age at onset is 70 years, and duration of survival is 24 months. About 40% of patients have a family history of dementia. Like CJD, it can be mistaken for Alzheimer's dementia."}, {"id": "pubmed23n0090_6949", "title": "[Clinical aspects and course of Alzheimer's disease].", "score": 0.009708737864077669, "content": "The diagnosis of Alzheimer's disease must be considered in all subjects over 40 years of age whose mental functions have insidiously become altered and are progressively getting worse. The initial phase of the disease, which precedes dementia, is characterized by disorders of memory often associated with changes in personality and behaviour and with subtle disorders of speech, reasoning and abstraction ability and visuo-constructive capabilities. The results of neurological and paraclinical examinations are normal. This phase lasts from two to four years and may be diagnosed as \"possible Alzheimer's disease\". When the disease is established the patient has lost his autonomy and can only be kept at home with the help of his relatives. Dementia gradually becomes worse, with a combination of memory disturbance, aphasia-apraxia-agnosia syndrome, impaired intellectual faculties and disorders of behaviour. Computerized tomography shows evidence of cerebral atrophy. This phase lasts from three to six years and may be called \"probable Alzheimer's disease\". The terminal phase of the disease includes somatic lesions, and all mental functions are profoundly altered. Death occurs seven to ten years after the first symptoms. Histopathological data are necessary to make a diagnosis of \"confirmed Alzheimer's disease\", since there is no clinical, morphological, electrophysiological or radiological sign that is pathognomonic of that disease."}, {"id": "wiki20220301en002_93721", "title": "Huntington's disease", "score": 0.009615384615384616, "content": "Signs and symptoms Signs and symptoms of Huntington's disease most commonly become noticeable between the ages of 30 and 50 years, but they can begin at any age, and present as a triad of motor, cognitive, and psychiatric symptoms. In 50% of cases, the psychiatric symptoms appear first. Their progression is often described in early stages, middle stages, and late stages with an earlier prodromal phase. In the early stages, subtle personality changes, problems in cognition, and physical skills, irritability, and mood swings occur, all of which may go unnoticed, and these usually precede the motor symptoms. Almost everyone with HD eventually exhibits similar physical symptoms, but the onset, progression, and extent of cognitive and behavioral symptoms vary significantly between individuals."}, {"id": "pubmed23n0490_19507", "title": "Change in the mini-mental state exam in Alzheimer's disease over 2 years: the experience of a dementia clinic.", "score": 0.009615384615384616, "content": "The decline in the Mini-Mental State Exam (MMSE) over 2 years was assessed in males with Alzheimer's disease (AD; N = 136) seen in a dementia clinic. The average initial MMSE was 21.0 (SD+/-3.9; range 14--29) and declined 2.8 points (+/-4.7; range -17 to +6) over 2 years. The mode for change on the MMSE was 0 (N = 22) while the median fell between 2 and 3 points lost. Fifty-five of 136 patients (39.7%) had unchanged or better scores. There was no significant correlation between the initial MMSE and rate of change (r = -0.16; p = 0.06). While the progression of AD is quite variable from patient to patient, our data indicate that in most it is associated with little if any change in the MMSE even over 2 years. The MMSE is not an adequate tool to monitor change in the individual patient with AD."}, {"id": "wiki20220301en024_102284", "title": "Frontotemporal dementia", "score": 0.009526839859249831, "content": "The confusion between Alzheimer's and FTD is justifiable due to the similarities between their initial symptoms. Patients do not have difficulty with movement and other motor tasks. As FTD symptoms appear, it is difficult to differentiate between a diagnosis of Alzheimer's disease and FTD. There are distinct differences in the behavioral and emotional symptoms of the two dementias, notably, the blunting of emotions seen in FTD patients. In the early stages of FTD, anxiety and depression are common, which may result in an ambiguous diagnosis. However, over time, these ambiguities fade away as this dementia progresses and defining symptoms of apathy, unique to FTD, start to appear."}, {"id": "pubmed23n0500_5510", "title": "[Rates of progression in mild cognitive impairment].", "score": 0.009523809523809525, "content": "The aim of the study which was based on a five year prospective scheme was the evaluation of progressive changes in persons with a diagnosed mild cognitive impairment (MCI). A result of 3 in the Global Deterioration Scale (Reisberg's criteria), allowed for mild cognitive impairment diagnosis. The CGI scale result in the 5th year of observation was the bases to divide the studied population into a group with a stable MCI course and a group with progressive symptoms. 41 persons finished the five years of observation, out of a total 46 chosen for the study. After 5 years, 26 persons had no changes in the CGI result, whilst 15 persons showed a worsening in their clinical picture. At the initial qualification to the study the two groups of patients did not differ in their psychiatric state. However differences in the ADAS-kog could be seen in the second measurement done after the first year of the observation. Amongst those persons who had a worsening above 1.68 in the first year of the observation, Alzheimer's disease could be diagnosed definitely more frequently in the further stages of the observation. Evaluation of cognitive function impairment progression seems to be one of the most important diagnostic elements and should be included in the diagnostic criteria of MCI."}, {"id": "pubmed23n1059_22427", "title": "Late-onset attention-deficit/hyperactivity disorder as a differential diagnosis of dementia: a case report.", "score": 0.009433962264150943, "content": "Although adult attention-deficit/hyperactivity disorder has recently gained increased attention, few reports on attention-deficit/hyperactivity disorder in the pre-elderly or elderly have been published. Here, we present the case of a patient with attention-deficit/hyperactivity disorder who gradually developed dementia-like symptoms as she aged, which initially made her condition difficult to distinguish from early onset Alzheimer's disease. This report illustrates that some types of attention-deficit/hyperactivity disorder may be misdiagnosed as dementia. The patient was a 58-year-old woman. Although she presented with a tendency for inattentiveness and forgetfulness since childhood, she did not have a history of psychiatric disorders prior to consultation. Around the age of 52 years, her inattentiveness and forgetfulness gradually progressed, and at 57 years of age, she became inattentive and forgetful that it interfered with her work and daily life. For example, she forgot meetings with important clients and transferred money to the wrong bank account; these failures resulted in poor management of her company. At home, she experienced increasing difficulties with remembering prior commitments with her family and misplacing items, which her family members noticed. With the encouragement of her family and employees, who worried that she was suffering from dementia, she visited our memory clinic, whereby she was suspected of having early onset Alzheimer's disease. However, neuropsychological tests and brain imaging evaluations did not reveal any significant abnormalities. After dismissing various possible diagnoses, including dementia, other organic diseases, mood disorders, and delirium, we diagnosed her with attention-deficit/hyperactivity disorder. Treatment with 18 mg of methylphenidate was initiated, and significant improvements in her symptoms were observed within a few days; for example, she stopped losing her things, was able to concentrate for long durations, and could complete more tasks than she could before treatment. Since initiating treatment, she has returned to work and has been able to perform her daily activities without difficulty. This case supports that some patients with late-onset attention-deficit/hyperactivity disorder may gradually develop dementia-like symptoms during the pre-elderly and elderly stages of life. Therefore, clinicians should consider late-onset attention-deficit/hyperactivity disorder as a differential diagnosis of some types of dementias."}, {"id": "pubmed23n0267_18986", "title": "Age at onset of Alzheimer's disease: relation to pattern of cognitive dysfunction and rate of decline.", "score": 0.009433962264150943, "content": "We examined the pattern of cognitive impairment and rate of cognitive and functional decline as a function of age at symptom onset in 127 patients with probable Alzheimer's disease (AD). At baseline, early-onset (before age 65) and late-onset groups were mildly and comparably impaired on the modified Mini-Mental State Examination (mMMS) and the Blessed Dementia Rating Scale-Part 1 (BDRS). Repeated-measures analysis of variance revealed significantly more rapid decline in early-onset subjects over a 2-year follow-up period. Multivariate linear regression analyses indicated that age at symptom onset strongly predicted rate of decline on the mMMS and the BDRS, even after controlling for symptom duration, gender, family history of dementia, and baseline mMMS and BDRS scores. Early- and late-onset AD subjects also differed in terms of pattern of performance on the mMMS. Early-onset subjects scored significantly lower than late-onset subjects on attentional items of the mMMS at baseline and follow-up. Conversely, late-onset subjects scored significantly lower than early-onset subjects on memory and naming items at baseline, and the two groups were comparable on these tasks at follow-up. Results provide longitudinal evidence of more rapid cognitive and functional decline in subjects with early-onset AD and suggest that early-onset AD may be characterized by predominant impairment of attentional skills."}, {"id": "pubmed23n1141_20355", "title": "Letter to the Editor: Depression As The First Symptom Of Frontal Lobe Grade 2 Malignant Glioma.", "score": 0.009345794392523364, "content": "Dear Editor, Next to focal neurological symptoms, epileptic seizures and head aches, brain tumors can less frequently bring about cognitive changes, slowed speech, difficulty sustaining mental functioning and psychiatric symptoms of personality changes and. loss of interest in daily activities, these symptoms may be evaluated as anxiety or depression. Depression is known to be a complication of brain tumours and may sometimes be seen after the presentation of neurological symptoms linked to brain tumours, and sometimes after tumor treatment (Oğuz et al. 2005, Litofsky et al. 2004, Moise and Madhusoodanan 2006, Oreskovic M et al. 2007, Rooney A et al. 2010). The dorsolateral prefrontal, orbitofrontal and medial frontal circuits constitute the three subcortical neuronal circuits in the frontal cortex. The dorsolateral prefrontal circuit is associated with planning and operational functions and lesions on it may give rise to apathy, abulia, perseveration, personality changes and planning disorder. Lesions involving the orbitofrontal circuit, which is associated with response suppression and disinhibition, may involve emotional lability and memory problems. Whereas lesions affecting the right orbitofrontal circuit give rise to elevated mood, lesions on the left orbitofrontal circuit lead to depressed mood. In cases with medial frontal circuit involvement, akinetic mutism may result from lesions in the superior medial region and anteroretrograde amnesia and confabulation are observed with lesions in the inferior medial region (Tosun et al. 2016, Chirchiglia 2018). A diagnosis of psychiatric disorder may be given during the first examination of patieants with primary brain tumours, especially if localized in the frontal lobe. Thorough history taking and physical examination are necessary for early diagnosis. The case reported here concerns a 29-year-old university graduate female patient, living with her partner and children, who consulted the clinic with complaints of tendency to frequent crying, anhedonia, having difficulty with speech fluency, forgetfulness and distractedness that had presented suddenly, 2 months previously, without any causative stressor. In her mental status examination, she appeared having normal self-care with appearance at her actual age. She was fully conscious and oriented, not willing to cooperate with the interview, had distinct difficulty in maintaining attention and with fluency of speech. Her mood was depressive. She described loss of appetite, fatigue and energy loss. Her difficulty in paying attention was pronounced. She did not have a history of psychotropic medication use or family history of psychiatric disease. She did not smoke or use alcohol or substance. After evaluating the clinical interview, a preliminary diagnosis of major depressive disorder was considered on the basis of the DSM-5 criteria. Routine blood tests were requested. Given the continuation of her complaints, the difficulty with fluent speech and the increase in tendency to sleep at the first week follow up, cranial MRI was planned. The MRI results showed on the right, in the frontal lobe a multilocular mass with precallosal extension, undiscernable margins with the right lateral aspect of the corpus callosum genu and dispersed cystic-necrotic areas with T2 signal series. The dimensions of the mass were nearly 5 x 3 cm causing a 1-cm right-to-left shift of the midline (Figure 1) DEPRESSION AS THE FIRST SYMPTOM OF FRONTAL LOBE GRADE 2 MALIGNANT GLIOMA 2 Türk Psikiyatri Dergisi 2 Turkish Journal of Psychiatry Letter to the Editor 143 144 The patient was referred for surgery with the preliminary diagnosis of high-grade glial tumour. Pathology results identified a grade 2 glioma. It was learned that radiotherapy sessions were begun after surgery. The patient did not have any symptoms of psychopathology during the 2 monthly psychiatric interviews made after surgery. Brain tumours generally indicate their presence with headache, seizures and other neurological symptoms and very rarely with depression as seen in the case of our patient. It should be kept in mind that atypical psychiatric symptoms may have an underlying organic lesion and subtle neurological symptoms should be investigated in detail. A recent meta-analysis on 37 observational studies determined a 21.7% prevalence of depression in a total of 4518 patients with intracranial tumours. Comorbidity of depression with brain tumor was demonstrated to worsen the quality of life, increase suicidal risk and lower the chance of survival (Huang et al. 2017). The possibility of psychiatric symptoms being the clinical clues for brain cancer was noted and the necessity of neuroimaging tests in cases of recent-onset psychosis or mood disorder symptoms, atypical personality changes and anorexia without body dysmorphic disorder was emphasized (Madhusoodanan et al. 2015). Loss of interest, tendency to frequent weeping, introversion and anhedonia were the sole complaints in the case discussed here. The increase in psychomotor retardation and slowing down of movements at the very first weekly control follow up necessitated neuroimaging. Despite the reports in the literature on the frequent association of unpreventable excessive behavior, disinhibition and irritability with right frontal injury and lesions (Okumuş and Hocaoğlu 2018), depression was the dominant symptom in the case presented here. There are differences between primary major depression and depression presenting with underlying somatic diseases which is known to occur at later ages (Rouchell et al. 2002). However, our patient was aged 29 years. Also, cases of depression due to somatic disease are less associated with family history of depression and suicidal ideation and attempts, while cognitive symptoms come to the foreground during mental status examination. (Sertöz and Mete 2004, Rouchell et al. 2002). Our patient did not have suicidal ideation or attempts, or a family history of depression. In apathy, which may be explained as emotional blunting, indifference or detachment from the external world, targeted behavior is also reduced next to the lack of emotional expression. The individual discussed here was learned not to sit at the table or change the television channel unless reminded to do so. When the reason was asked, she could not think of one. The reduction in emotional expression accompanies reduced insight, abulia and lack of empathy (Sözeri Varma et al. 2019). In depression, apathy is defined as 'sorrowless depression'. Our patient cried but had very blunted mimics and gestures. She explained that she could not help weeping even at times when she did not feel internally distressed. The seriousness of apathy, as a symptom difficult to differentiate from depression, is still not understood. Neuroimaging Figure 1- Cranial MRI of the patient 145 Received: 16.08.2020, Accepted: 04.12.2020, Available Online Date: 05.10.2021 1MD., Antalya Kepez State Hospital, Department of Psychiatry, Antalya, 2MD., Ordu University Training and Research Hospital, Department of Psychiatry, Ordu, Turkey e-mail: bosbora@yahoo.com https://doi.org/10.5080/u25957 studies indicate apathy to be a reflect of impaired frontal-subcortical circuits and the functional disorder of the connections between the ventromedial prefrontal cortex and the basal ganglia (Chase 2011). Comparison of 45 individuals with depression due to aging and 43 healthy individuals showed apathy to be associated with fronto-limbic gray and white matter abnormalities which continued after antidepressant treatment. The structural anomalies of the posterior subgenual cingulate gyrus and the uncinate fasciculus were discussed (Yuen 2014). The case discussed here is presented to emphasize the importance of brain imaging methods and detailed investigation of atypical symptoms for diagnostic approaches to psychiatric disorders. Especially, complaints at young age of depression with psychomotor retardation, reduced fluency of speech and sudden onset withdrawal without stressors should be a warning of secondary depression. Yours sincerely... Şerif Bora Nazlı1 , Muhammet Sevindik2 REFERENCES Chase TN (2011) Apathy in Neuropsychiatric Disease: Diagnosis, Pathophysiology, and Treatment. Neurotox Res 19:266-78. Chirchiglia D (2018) Pseudodepression as an Anticipatory Symptom of Frontal Lobe Brain Tumors. Int J Depress Anxiety 1:007. Huang J, Zeng C, Xiao J et al (2017) Association between depression and brain tumor: a systematic review and meta-analysis. Oncotarget 8:94932-43. Litofsky NS, Farace E, Anderson F et al (2004) Depression in patients with high-grade glioma: Results of the glioma outcomes project. Neurosurgery 54:358-67. Madhusoodanan S, Ting MB, Farah T et al (2015) Pyschiatric aspects of brain tumors: A review. World J Psychiatry 5:273-85. Moise D, Madhusoodanan S (2006) Psychiatric symptoms associated with brain tumors: a clinical enigma. CNS Spectr 2006;11:28-31. Oğuz N, Ilnem C, Yener F (2005) Psychiatric symptoms in brain tumors: Case reports. Bulletin of Clinical Psychopharmacology 15:18-21. Hocaoğlu Ç, Okumuş B (2018) Psychiatric manifestations and brain tumor: A case report and brief review. The Medical Journal of Mustafa Kemal University 9:42-9. Oreskovic NM, Strother CG, Zibners LM (2007) An unusual case of a central nervous system tumor presenting as a chief complaint of depression. Pediatric Emergency Care 23:486-8. Rooney A, Carson A, Grant R (2011) Depression in cerebral glioma patients: a systematic review of observational studies. J Natl Cancer Inst103:61-76. Rouchell AM, Pounds R, Tierney JG (2002) Depression Textbook of Consultation-Liaison Psychiatry, 2nd Edition, Volume 1. MG Wise, JR Rundell (Ed), Washington DC American Psychiatric Publishing, Inc, p.307-38. Özen SÖ, Hayriye ME (2004) Bedensel Hastalıklarda Depresyon. Klinik Psikiyatri Ek 2:63-9. Sözeri Varma G , Bingöl C , Topak O et al (2019) Relationship of apathy with depressive symptom severity and cognitive functions in geriatric depression. Arch Neuropsychiatry 56:133-8. Yuen GS, Gunning FM, Woods E et al (2014) Neuroanatomical correlates of apathy in late-life depression and antidepressant treatment response. J Affect Disord 166:179-86."}, {"id": "pubmed23n0259_2108", "title": "Patients with dementia and their caregivers 3 years after diagnosis. A longitudinal study.", "score": 0.009345794392523364, "content": "To document caregivers' perceptions of the deterioration in functional ability of persons with dementia over time, to identify the most problematic behavior for caregivers at two stages of dementing illness, and to compare the perceived informational needs of caregivers at diagnosis and 3 years later. Single cohort. Surveys were mailed at time 1 and respondents were followed up after 3 years (time 2). Midwestern hospital dementia assessment clinic with a family physician director. Continuing care was by community physicians. Thirty elderly patients with dementia who were evaluated at the dementia clinic. Data were provided by their caregivers. Patients' scores on the Activities of Daily Living section of the questionnaire declined (bathe self, P = .03; transfer from bed or chair, P = .03; and groom self, P = .06). Significant deterioration in behaviors over time was found in incontinence (P = .04). Fewer patients were depressed at time 2 (P = .02). The patient behaviors found most troublesome at time 1 were worrying about memory loss, losing or hiding things, feeling blue, experiencing restlessness, having difficulty calculating, experiencing a lack of interest, and having false ideas. At time 2, the greatest problems were having a short attention span, failing to recognize persons or things, experiencing a lack of interest, experiencing restlessness, repeating himself or herself, forgetting where he or she is, speaking incoherently, and being incontinent. Questions caregivers most wanted answered at time 1 concerned possible treatment, the future course of illness, and the cause of the symptoms. At time 2, the concerns were the future course of illness, possible treatment, and disease inheritance. There was significantly more interest in family agreement about care (P = .004) and the need for legal guardianship (P = .001) at time 2. Caregivers' perceptions of the most frequent and troublesome behaviors of patients with dementia were documented at different stages of the disease. The importance caregivers attached to their requests for information reflected changing but continuing needs for reassurance about the patient's diagnosis and treatment and for help with the psychosocial consequences of dementia. Physicians must be aware of caregivers' needs at different stages of the disease process and be equipped to help them appropriately."}, {"id": "wiki20220301en395_27415", "title": "Music therapy for Alzheimer's disease", "score": 0.009259259259259259, "content": "The forms of music therapy are broad in nature, and can range from individual or group singing sessions, to active participation in music making, to listening to songs individually. Alzheimer’s disease (AD) is a fatal disease that continuously deteriorates brain chemistry over time. Accounting for more than 60% of the dementia in older people, AD gradually leads to detrimental effects on cognitive function, linguistic abilities, and memory. Within populations living with Alzheimer's, music therapy is sometimes used to assist in palliating the behavioral and psychological symptoms of this disease. Music therapy is based in scientific findings and can elicit change in individuals as well as groups through music. Personalized music therapy has been shown in some cases to be able to lessen certain symptoms, including behavioral symptoms, such as physical or verbal outbursts and hallucinations, and cognitive symptoms related to dementia."}, {"id": "pubmed23n0327_4877", "title": "Update on diagnostic methods, natural history and outcome variables in Alzheimer's disease.", "score": 0.009259259259259259, "content": "The diagnosis of Alzheimer's disease (AD) currently relies on history obtained from family or friends and on mental status assessment matched to National Institute of Neurological and Communicative Disorders and Stroke criteria. Progression over time may or may not be typical, suggesting alternate diagnoses such as Lewy body or frontotemporal dementias. Apolipoprotein E genotype does not appear to be useful as a diagnostic marker. The usefulness of brain imaging in AD must be reexamined. Critical events in the natural history of AD, such as institutionalization and loss of ability for self-care, could be used as end points. Loss of ability for instrumental tasks, such as driving, traveling alone, or managing finances, would be preferable for early-stage stabilization studies. Different symptomatic domains of AD (mood, cognition, functional autonomy, behavior, motoricity) can be quantified using specific outcome measures. Although cognitive loss has been considered a core symptom of AD from a regulatory perspective, loss of functional autonomy and behavioral disinhibition are considered more important by clinicians and families. Recently, the availability of new scales has led to an interest in all of these domains. Results from symptomatic drug studies suggest a differential effect of cholinesterase inhibitors on cognition versus muscarinic agonists on functional autonomy and behavior. Hence there is a need to measure these domains separately and, eventually, to attempt combination therapy. Quality of life is a difficult but important dimension of AD therapeutic research, and it requires further methodological research."}, {"id": "pubmed23n1122_17394", "title": "Most families tend to realize progress of Alzheimer's disease when behavioural and psychological symptoms are obvious.", "score": 0.009174311926605505, "content": "Alzheimer's disease (AD) is a common cognitive disease that can progress at an accelerating rate. Even with early diagnosis, the families might not recognize AD progressing unless behavioural and psychological symptoms of dementia (BPSD) develop. In many cases, discrepancies could exist between family-assessed AD stage and diagnosed AD stage. This study explored such discrepancies and potential clinical implications. Participants were 161 new outpatients with AD or mild cognitive impairment at four memory clinics whose AD stage was diagnosed using the Revised Hasegawa Dementia Scale (HDS-R) and Mini-Mental State Examination (MMSE). We classified patients into four groups according to AD severity. Family members completed the Functional Assessment Staging (FAST) scale during an interview. We then assigned patients to three groups according to discrepancies between family-assessed and diagnosed AD stage. Families also completed the Neuropsychiatric Inventory Questionnaire (NPI-Q), which assesses 12 neuropsychiatric domains, in order to examine the presence of BPSD in relation to AD stage. Most families (74%-80%) assessed patients as having milder AD than the diagnosed stage. NPI-Q scores and duration of education significantly affected discrepancies with HDS-R and MMSE scores. The NPI-Q domains of anxiety, apathy/indifference, aberrant motor behaviours, and appetite/eating disturbance significantly affected family-assessed FAST. Families of patients with more years of education assessed the AD stage as more advanced than the diagnosed stage. Surprisingly, living together did not significantly affect the discrepancy. Most families assessed AD as milder than the clinically diagnosed AD stage. In addition, high NPI-Q scores and more years of school education significantly affected the discrepancy. Family-assessed FAST was significantly affected by the NPI-Q domains of anxiety, apathy/indifference, aberrant motor behaviours, and appetite/eating disturbance. These results suggest that obvious BPSD are significant factors for Japanese families to recognize AD progress."}, {"id": "wiki20220301en273_15509", "title": "Signs and symptoms of Parkinson's disease", "score": 0.00909090909090909, "content": "Neuropsychiatric Parkinson's disease causes neuropsychiatric disturbances, which mainly include cognitive disorders, mood disorders, and behavior problems, and can be as disabling as motor symptoms. Since L-Dopa, the widely used drug in Parkinson's disease treatment, is decarboxylated by aromatic L-amino acid decarboxylase (AADC), which is found in both dopaminergic and serotonergic neurons, it is possible for serotonergic neurons to convert L-Dopa into dopamine and generate excessive neuronal death by creating reactive oxygen species and quinoproteins. The association of serotonin with mood and cognition may explain some of the side-effects observed in patients treated with L-Dopa due to serotonin deficit."}, {"id": "pubmed23n0413_20890", "title": "Psychiatric symptomatology and prodromal Alzheimer's disease.", "score": 0.00909090909090909, "content": "The aim of this study was to determine the prevalence of psychiatric symptoms among nondemented individuals with memory changes and whether such symptoms predict progression of functional decline or diagnosis of Alzheimer disease (AD). A semi-structured interview was administered at baseline to controls (n = 32) and to nondemented subjects with memory changes (n = 112) and to each subject's collateral source. The interview assessed the impact of cognition on functional abilities in daily life and a variety of psychiatric symptoms, including symptoms of psychosis, depression, and personality change. Participants were followed annually for 3 years to determine who had progressive functional decline and who progressed to meet clinical criteria for AD. Those diagnosed with AD on follow-up had more symptoms of personality change, such as agitation and passivity, at baseline than those who did not progress to meet clinical criteria for AD. Mild depressive symptoms were also more common among individuals at baseline who subsequently 'converted' to AD. Symptoms of personality change were associated with a more rapid increase in functional difficulty over time, whereas depressive symptoms were not. Changes in personality are more common among subjects with memory changes who go on to develop AD. Particular types of personality change, such as agitation and passivity, are related to progression of functional difficulty over time. Depressive symptoms, although common in prodromal AD, are not associated with a more rapid functional decline."}, {"id": "wiki20220301en080_45803", "title": "Post-concussion syndrome", "score": 0.009009009009009009, "content": "Psychological and behavioral Psychological conditions, which are present in about half of people with PCS, may include irritability, anxiety, depression, and a change in personality. Other emotional and behavioral symptoms include restlessness, aggression, and mood swings. Some common symptoms, such as apathy, insomnia, irritability, or lack of motivation, may result from other co-occurring conditions, such as depression. Higher mental functions Common symptoms associated with a diagnosis of PCS are related to cognition, attention, and memory, especially short-term memory, which can also worsen other problems such as forgetting appointments or difficulties at work. In one study, one in four people diagnosed with PCS continued to report memory problems a year after the injury, but most experts agree that cognitive symptoms clear within six months to a year after injury in the vast majority of individuals."}, {"id": "pubmed23n0066_9545", "title": "Progression of cognitive impairment in Alzheimer's disease.", "score": 0.009009009009009009, "content": "Change in cognitive function was assessed over 12 months in 110 patients over the age of 65 satisfying National Institute of Neurological and Communicative Disorders and Stroke and the Alzheimer's Disease and Related Disorders Association (NINCDS/ADRDA) criteria for \"probable\" Alzheimer's Disease. A highly significant deterioration in cognitive function was observed. Decline in cognitive scores was relatively normally distributed. Patients who died during the follow-up had more apraxia at entry to the study than survivors. A greater rate of decline was seen in patients whose parents suffered from dementia (but not in those where a sibling or other relative was affected), in subjects who had moderate dementia, and those who had been ill for less than 24 months. Age, age of onset, and the presence or absence of aphasia or apraxia had no influence on rate of progression. A cluster analysis revealed three patterns of decline."}, {"id": "wiki20220301en295_38806", "title": "Anti-NMDA receptor encephalitis", "score": 0.008928571428571428, "content": "Prior to the development of a symptom complex that is specific to anti-NMDA receptor encephalitis, people may experience prodromal symptoms, including headaches, flu-like illness, or symptoms similar to an upper respiratory infection. These symptoms may be present for weeks or months prior to disease onset. Beyond the prodromal symptoms, the disease progresses at varying rates, and patients may present with a variety of neurologic symptoms. During the initial stage of the disease, symptoms vary slightly between children and adults. However, behavior changes are a common first symptom within both groups. These changes often include agitation, paranoia, psychosis, and violent behaviors. Other common first manifestations include seizures and bizarre movements, mostly of the lips and mouth, but also including pedaling motions with the legs or hand movements resembling playing a piano. Some other symptoms typical during the disease onset include impaired cognition, memory deficits, and"}, {"id": "pubmed23n0413_16465", "title": "Long-term cognitive and functional decline in late onset Alzheimer's disease: therapeutic implications.", "score": 0.008928571428571428, "content": "National Institute of Clinical Excellence guidelines advocate the use of the Mini-Mental Test Examination and a functional assessment as a means of measuring treatment response. However, there is little knowledge of the change expected in those with Alzheimer's disease in clinical practice. to describe the long-term variability of the Mini-Mental Test Examination and Blessed Dementia Rating Scale. 374 Alzheimer's disease patients referred to psychiatric services in southeast London were followed annually over a 3-year period. the mean Mini-Mental Test Examination score for the total group at baseline was 9.9 points. Individual variability in the rate of cognitive and functional decline is large and around 40% of patients after 1 year, and up to one-quarter of patients after 3 years who survived, show no change or an improvement in scores compared with baseline measures. in the evaluation of individual treatment response the rate of change, as measured by the Mini-Mental Test Examination and Blessed Dementia Rating Scale, is of limited value."}, {"id": "wiki20220301en469_12650", "title": "Outline of the human brain", "score": 0.008849557522123894, "content": "Neurodegeneration and dementia Neurodegeneration – an umbrella term for the progressive loss of structure or function of neurons, including death of neurons. Multiple sclerosis – an inflammatory disease in which the myelin sheaths around the axons of the brain and spinal cord are damaged. Parkinson's disease – Early symptoms include shaking, rigidity, slowness of movement and difficulty with walking and gait. Later symptoms include cognitive and behavioral problems, with dementia commonly occurring in the advanced stages. The motor symptoms result from the death of dopamine-generating cells in a region of the mid-brain. Alzheimer's disease – The most common form of dementia. Beginning with an array of symptoms including memory loss, as the disease progresses the individual often withdraws from family and society and requires 24/7 supervision. It is predicted to affect 1 in 85 people globally by 2050."}, {"id": "pubmed23n0270_6214", "title": "A longitudinal study of Alzheimer's disease: measurement, rate, and predictors of cognitive deterioration.", "score": 0.008849557522123894, "content": "This study measured the annual rate of cognitive change in patients with Alzheimer's disease and determined the effects of clinical variables on that rate. It also compared the ability of two cognitive scales to measure change over the entire range of dementia severity. The cognitive subscale of the Alzheimer's Disease Assessment Scale and the Blessed test for information memory and concentration were given to 111 patients with Alzheimer's disease and 72 nondemented elderly comparison subjects at 6-month intervals for up to 90 months. Longitudinal changes in scores on the cognitive subscale were measured with several different methods of data analysis. For the patients with Alzheimer's disease, the annual rate of change in cognitive subscale scores showed a quadratic relationship with dementia severity in which deterioration was slower for mildly and severely demented patients than for patients with moderate dementia. Gender, age at onset, and family history of dementia had no effect on the rate of cognitive deterioration. The comparison group showed a slight improvement in cognitive performance over time. All data analytic methods gave similar results. The cognitive subscale of the Alzheimer's Disease Assessment Scale was more sensitive to change in both mild and severe dementia than was the Blessed test. These results suggest that cognitive deterioration is slow during the early and very late stages of Alzheimer's disease and more rapid during the middle stages. No clinical variables other than degree of cognitive impairment and previous rate of cognitive decline predicted rate of deterioration. These results have implications for treatment trials and attempts to identify subgroups."}, {"id": "wiki20220301en262_12851", "title": "Parkinson's disease", "score": 0.008771929824561403, "content": "Attempts to classify PD into different subtypes have been made, with focus put on age at onset, progression of symptoms and dominance of tremor, but none have been adopted. Signs and symptoms The most recognizable early symptoms are movement (\"motor\") related. Non-motor symptoms, including autonomic dysfunction (dysautonomia), neuropsychiatric problems (mood, cognition, behavior or thought alterations), and sensory (especially altered sense of smell) and sleep difficulties, are usually associated with later stages, but may present at the time of diagnosis. Motor Four motor symptoms are considered as cardinal signs in PD: tremor, slowness of movement (bradykinesia), rigidity, and postural instability."}, {"id": "pubmed23n0050_14188", "title": "Predictors of cognitive and functional progression in patients with probable Alzheimer's disease.", "score": 0.008771929824561403, "content": "We followed 65 patients with probable Alzheimer's disease, who were initially mildly to moderately impaired, with semiannual assessments of cognitive and functional performance for up to 4 years. Scores on the Mini Mental State Examination and a combination of instrumental and self-maintenance scale of activities of daily living were regressed on time of examination (measured in 6-month increments) to estimate cognitive and functional progression rates in individual patients. Lower scores on the verbal neuropsychological tests at the time of study entry, more aggressive behavior, and sleep disturbance during the first year of observation predicted faster cognitive progression. Faster functional progression was predicted by paranoid behavior, hallucinations and activity disturbances during the first year and the presence of extrapyramidal signs and lower scores on nonverbal neuropsychological tests at the time of entry into the study. Hallucinations occurred independently of cognitive severity and may identify a distinct subgroup of patients with rapid functional progression. Because of the greater significance of functional progression for caregivers' ability to manage patients, the presence of specific behavior problems early in the disease course may help to identify individuals who will experience greater functional decline and be at risk for earlier institutionalization."}, {"id": "wiki20220301en429_18438", "title": "Sex differences in memory", "score": 0.008695652173913044, "content": "Multiple studies have found that there is a significant difference in the symptoms of Alzheimer's disease that affect the sexes. Some of these behavioral and psychological symptoms of dementia (BPSD) include depression, anxiety, dysphoria, nighttime disturbances, and aggression. Several recent studies have found that women tend to exhibit symptoms such as depression and anxiety more often than men. One study has even gone as far as to suggest that having depression at any point during midlife increases chances of Alzheimer's Disease developing later by up to 70%. Men, on the other hand, exhibit symptoms such as aggression and other socially inappropriate behaviors more often. In addition, it has been found that men are more likely to have coronary artery disease which has been known to damage the blood brain barrier (BBB) by causing micro vascular lesions. Damage to the blood brain barrier seems to be connected to cognitive decline and several forms of dementia, including Alzheimer's"}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[201, 356]], "word_ranges": [[28, 48]], "text": "Hyperkalemia produces repolarization disturbances that result in the installation of a large, symmetrical, narrow-based T, visible in the precordial leads."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The correct answer is: 2. Hyperkalemia. Spironolactone, as a potassium-sparing diuretic, inhibits the action of aldosterone at the renal level, being responsible for a decrease in potassium excretion. Hyperkalemia produces repolarization disturbances that result in the installation of a large, symmetrical, narrow-based T, visible in the precordial leads. The QT space is shortened. These anomalies appear with a kalemia of around 5.5 to 6 mmol/l. Above 6.5 mmol/L, electrocardiographic changes are constant, and are dominated by conduction disturbances (making ventricular extrasystoles possible).", "full_answer_no_ref": "[HIDDEN] Hyperkalemia. Spironolactone, as a potassium-sparing diuretic, inhibits the action of aldosterone at the renal level, being responsible for a decrease in potassium excretion. Hyperkalemia produces repolarization disturbances that result in the installation of a large, symmetrical, narrow-based T, visible in the precordial leads. The QT space is shortened. These anomalies appear with a kalemia of around 5.5 to 6 mmol/l. Above 6.5 mmol/L, electrocardiographic changes are constant, and are dominated by conduction disturbances (making ventricular extrasystoles possible).", "full_question": "An 80-year-old patient with a history of hypertension and on treatment with enalapril and spironolactone comes to the hospital with asthenia and severe muscle weakness. Blood pressure is 110/70 mmHg. In the ECG, there are sharp and elevated T waves, ventricular extrasystoles and short QT. What is the most likely diagnosis?", "id": 164, "lang": "en", "options": {"1": "Hypercalcemia.", "2": "Hyperkalemia.", "3": "Hypomagnesemia.", "4": "Hypocalcemia.", "5": "Hypernatremia."}, "question_id_specific": 123, "type": "NEPHROLOGY", "year": 2013, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0593_14703", "title": "Effects of presentation and electrocardiogram on time to treatment of hyperkalemia.", "score": 0.019327731092436976, "content": "To assess the time to treatment for emergency department (ED) patients with critical hyperkalemia and to determine whether the timing of treatment was associated with clinical characteristics or electrocardiographic abnormalities. The authors performed a retrospective chart review of ED patients with the laboratory diagnosis of hyperkalemia (potassium level &gt; 6.0 mmol/L). Patients presenting in cardiac arrest or who were referred for hyperkalemia or dialysis were excluded. Patient charts were reviewed to find whether patients received specific treatment for hyperkalemia and, if so, what clinical attributes were associated with the time to initiation of treatment. Of 175 ED visits that occurred over a 1-year time period, 168 (96%) received specific treatment for hyperkalemia. The median time from triage to initiation of treatment was 117 minutes (interquartile range [IQR] = 59 to 196 minutes). The 7 cases in which hyperkalemia was not treated include 4 cases in which the patient was discharged home, with a missed diagnosis of hyperkalemia. Despite initiation of specific therapy for hyperkalemia in 168 cases, 2 patients died of cardiac arrhythmias. Among the patients who received treatment, 15% had a documented systolic blood pressure (sBP) &lt; 90 mmHg, and 30% of treated patients were admitted to intensive care units. The median potassium value was 6.5 mmol/L (IQR = 6.3 to 7.1 mmol/L). The predominant complaints were dyspnea (20%) and weakness (19%). Thirty-six percent of patients were taking angiotensin-converting enzyme (ACE) inhibitors. Initial electrocardiograms (ECGs) were abnormal in 83% of patient visits, including 24% of ECGs with nonspecific ST abnormalities. Findings of peaked T-wave morphology (34%), first-degree atrioventricular block (17%), and interventricular conduction delay (12%) did not lead to early treatment. Vital sign abnormalities, including hypotension (sBP &lt; 90 mmHg), were not associated with early treatment. The chief complaint of \"unresponsive\" was most likely to lead to early treatment; treatment delays occurred in patients not transported by ambulance, those with a chief complaint of syncope and those with a history of hypertension. Recognition of patients with severe hyperkalemia is challenging, and the initiation of appropriate therapy for this disorder is frequently delayed."}, {"id": "pubmed23n0697_7870", "title": "Electrolyte disorders and arrhythmogenesis.", "score": 0.017838819164890676, "content": "Electrolyte disorders can alter cardiac ionic currents kinetics and depending on the changes can promote proarrhythmic or antiarrhythmic effects. The present report reviews the mechanisms, electrophysiolgical (EP), electrocardiographic (ECG), and clinical consequences of electrolyte disorders. Potassium (K⁺) is the most abundent intracellular cation and hypokalemia is the most commont electrolyte abnormality encountered in clinical practice. The most significant ECG manifestation of hypokalemia is a prominent U wave. Several cardiac and non cardiac drugs are known to suppress the HERG K⁺ channel and hence the I(K), and especially in the presence of hypokalemia, can result in prolonged action potential duration and QT interval, QTU alternans, early afterdepolarizations, and torsade de pointes ventricular tachyarrythmia (TdP VT). Hyperkalemia affects up to 8% of hospitalized patients mainly in the setting of compromised renal function. The ECG manifestation of hyperkalemia depends on serum K⁺ level. At 5.5-7.0 mmol/L K⁺, tall peaked, narrow-based T waves are seen. At &gt; 10.0 mmol/L K⁺, sinus arrest, marked intraventricular conduction delay, ventricular techycardia, and ventricular fibrillation can develop. Isolated abnormalities of extracellular calcium (Ca⁺⁺) produce clinically significant EP effects only when they are extreme in either direction. Hypocalcemia, frequently seen in the setting of chronic renal insufficiency, results in prolonged ST segment and QT interval while hypercalcemia, usually seen with hyperparathyroidism, results in shortening of both intervals. Although magnesium is the second most abudent intracellular cation, the significance of magnesium disorders are controversial partly because of the frequent association of other electrolyte abnormalities. However, IV magnesium by blocking the L-type Ca(⁺⁺) current can successfully terminate TdP VT without affecting the prolonged QT interval. Finally, despite the frequency of sodium abnormalities, particularly hyponatremia, its EP effects are rarely clinically significant."}, {"id": "wiki20220301en026_74327", "title": "Hyperkalemia", "score": 0.01778093883357041, "content": "Hyperkalemia is an elevated level of potassium (K+) in the blood. Normal potassium levels are between 3.5 and 5.0mmol/L (3.5 and 5.0mEq/L) with levels above 5.5mmol/L defined as hyperkalemia. Typically hyperkalemia does not cause symptoms. Occasionally when severe it can cause palpitations, muscle pain, muscle weakness, or numbness. Hyperkalemia can cause an abnormal heart rhythm which can result in cardiac arrest and death. Common causes of hyperkalemia include kidney failure, hypoaldosteronism, and rhabdomyolysis. A number of medications can also cause high blood potassium including spironolactone, NSAIDs, and angiotensin converting enzyme inhibitors. The severity is divided into mild (5.5–5.9mmol/L), moderate (6.0–6.4mmol/L), and severe (>6.5mmol/L). High levels can be detected on an electrocardiogram (ECG). Pseudohyperkalemia, due to breakdown of cells during or after taking the blood sample, should be ruled out."}, {"id": "pubmed23n1014_9838", "title": "Licorice induced pseudohyperaldosteronism, severe hypertension, and long QT.", "score": 0.016849557522123894, "content": "Excessive intake of licorice may cause pseudohyperaldosteronism which, in turn, may lead to hypertension and hypokalemia. Severe hypokalemia may lead to electrocardiogram (ECG) changes including long QT interval potentially progressing into malignant arrhythmias. Here we present a 43-year-old woman admitted to the hospital with chest pain and a stinging sensation in the upper extremities. Her peak blood pressure was 177/98 mmHg and the blood test revealed low plasma potassium of 1.9 mmol/L. The ECG revealed flattened T-waves and long QT interval. Prior to admission, the patient had increased licorice ingestion to a total of some 70 g daily. The licorice intake was stopped and potassium was administrated orally and intravenously. Plasma potassium normalized and the ECG changes remitted. To our knowledge a few other cases of licorice-induced pseudohyperaldosteronism and long QT interval have previously been reported. This underlines the importance of quantifying licorice intake in younger people with unexplained high blood pressure and low potassium. Even small amounts of licorice daily may increase the risk of developing hypertension; therefore, licorice should be asked for specifically. Even though licorice intake is very easy to cover in the patient's history, it is often missed. Excessive licorice intake may course severe hypokalemia causing long QT interval in the ECG recording, potentially progressing into arrhythmias and even cardiac arrest/sudden death. Hypokalemia &lt;3 mmol/L and present ECG changes should be treated with potassium intravenously. Licorice-induced hypertension may be associated with syndrome of apparent mineralocorticoid excess (SAME). Plasma renin and aldosterone are both low at diagnosis and normalize when licorice is stopped."}, {"id": "pubmed23n0713_7316", "title": "A rare electrocardiographic manifestation of a rare form of multiple electrolyte disturbances: hyperparathyroid crisis.", "score": 0.01665774685576666, "content": "The surface electrocardiogram (ECG) has been used as a useful method for detection of metabolic disturbances for a long time. However, it may be difficult to distinguish the exact disturbance when more than one metabolic abnormality exists in a patient simultaneously. Although, \"classic\" ECG characterizations of common electrolyte disturbances are well described, multiple concurrent electrolyte disturbances may lead to ECG abnormalities that may not be easily detectable. This ECG concerns a 60-year-old male presented with general fatigue, weakness, epigastric pain, anorexia, nausea and extreme hypercalcemia (serum total and ionized calcium levels 20.5 mg/dL and 12.02 mg/dl, respectively), hypokalemia and hypomagnesemia associated with elevated parathyroid hormone (1160 pg/ml) and normal serum vitamin D level (97 ng/ml) . This rare manifestation of primary hyperparathyroidism has been named hyperparathyroid crisis in the literature. Hyperparathyroid crisis is an emergency form of multiple electrolyte abnormalities that manifest as a life-threatening hypercalcemia and simultaneous hypokalemia and hypomagnesemia; these two later are believed to be caused by diuretic effect of calcium on the renal tubules. The unique pattern of ECG in our patient first was misdiagnosed as prominent T waves with prolongation of the QT corrected (QTc) interval, which has been reported several times in patients with hyperparathyroidism crisis, compatible with our patient. But more investigation revealed that, the QTc interval not only is not prolonged, it is shortened as it is expected from the effect of hypercalcemia on electrocardiogram. The exact pattern of the patient`s ECG (figure 1) can be interpreted as it follows: (1) Flattening of the T wave, (2) a prominent U wave, (3) prolongation of the descending limb of the T wave such that it overlapped with the next U wave (4) virtual absence of ST segment and (5) shortening of the QT corrected interval. In conclusion, it should be emphasized when the T and U waves are separated by a very short segment they can mimic the appearance of a prolonged QT interval. However, more investigation can demonstrate the exact electrocardiographic pattern especially in multiple electrolyte disturbances, when \"classic\" ECG patterns are not expectable."}, {"id": "pubmed23n1025_14101", "title": "Child with acute weakness: don't forget the salts.", "score": 0.015548567435359888, "content": "Case summaryA 10-year-old boy presented with severe progressive generalised weakness on a background of 3 days of diarrhoea and vomiting. Vital signs were normal. Peripheral neurological examination revealed grade 1-2 power in all limbs, hypotonia and hyporeflexia. Sensation was fully intact. Cranial nerve examination and speech were normal. The ECG (figure 1) and initial venous blood gas (figure 2) are shown.edpract;107/1/21/F1F1F1Figure 1ECG.edpract;107/1/21/F2F2F2Figure 2Venous blood gas. QUESTION 1: What abnormalities are present on the ECG?Peaked T waves, prolonged PR segment and loss of P waves?Shortening of the QT interval and Osborn waves (J waves)?T wave flattening/inversion, prominent U waves and long QU interval?Prolonged QT interval with multiple atrial and ventricular ectopics? QUESTION 2: How would you manage this patient's hypokalaemia? QUESTION 3: What is the likely diagnosis?Conversion disorder.Myasthenia gravis.Periodic paralysis.Guillain-Barré syndrome.Botulism. QUESTION 4: What interventions can be considered for long-term treatment of this condition? <iAnswers can be found on page 2</i."}, {"id": "wiki20220301en327_30127", "title": "Thyrotoxic periodic paralysis", "score": 0.013506435199042204, "content": "Diagnosis Hypokalemia (low blood potassium levels) commonly occurs during attacks; levels below 3.0 mmol/l are typically encountered. Magnesium and phosphate levels are often found to be decreased. Creatine kinase levels are elevated in two thirds of cases, usually due to a degree of muscle injury; severe elevations suggestive of rhabdomyolysis (muscle tissue destruction) are rare. Electrocardiography (ECG/EKG) may show tachycardia (a fast heart rate) due to the thyroid disease, abnormalities due to cardiac arrhythmia (atrial fibrillation, ventricular tachycardia), and conduction changes associated with hypokalemia (U waves, QRS widening, QT prolongation, and T wave flattening). Electromyography shows changes similar to those encountered in myopathies (muscle diseases), with a reduced amplitude of the compound muscle action potentials (CMAPs); they resolve when treatment has commenced."}, {"id": "pubmed23n0070_2469", "title": "[Syncope caused by iatrogenic hypercalcemia].", "score": 0.013279421153436902, "content": "A course of the disease of a 68-year-old female who had been taking medigoxin, furosemide, verapamil and an unknown amount of spironolactone and potassium salt due to congestive heart failure is presented. She was admitted to emergency department of the University Hospital Rebro after an episode of syncopal attack because of arrhythmia due to hyperkalemia (8.9 nmol/L). She has had a fast idioventricular rhythm, followed by atrial tachycardia after that and with fast ventricular rhythm, S-T segment depression and a tall and peaked T-wave. In the following electrocardiograms left anterior hemiblock appeared, a tall R-wave of the anterolateral location, supraventricular and ventricular premature beats and atrioventricular block of the first degree. The patient had signs of non-oliguric form of acute renal failure at the admission which was a partly explanation for the development of hyperkalemia, together with the use of spironolactone and potassium salt. After the treatment she had normal serum creatinine values. She suffered from combined mitral valve disease: stenosis with a predominant regurgitation of the II/III degree. She was discharged from the hospital in a compensated state with normal serum potassium values."}, {"id": "pubmed23n1138_2324", "title": "Clinical and electrocardiogram presentations of patients with high serum potassium concentrations within emergency settings: a prospective study.", "score": 0.013152804642166345, "content": "Elevated potassium level is a common and reversible peri-arrest condition. Diagnosis and management of hyperkalemia in a short time is critical, where electrocardiogram (ECG) alterations might be helpful. We aimed to investigate the role of clinical features and ECGs in early diagnosing and treating hyperkalemia. Prospectively, adult patients who presented to the emergency department (ED) from July 2019 to March 2020 with hyperkalemia (serum potassium ≥5.5mmol/L) were included. History was obtained, and laboratory investigations and ECGs were performed at the presentation and before initiating hyperkalemia therapy. Hyperkalemia severity was divided into mild (5.5-5.9mmol/L), moderate (6.0-6.4mmol/L), and severe (≥6.5mmol/L). A cardiologist and emergency physician blinded to laboratory values, study design, and patients' diagnoses interpreted ECGs and presenting symptoms independently to predict hyperkalemia. Sixty-seven hyperkalemic patients with a mean (±SD) serum potassium level of 6.5±0.7mmol/L were included in this study. The mean age was 63.9±15.1, and 58.2% were females. Hyperkalemia was mild in 10.4%, moderate in 40.3%, and severe in 49.3%. Almost two thirds of patients (71.6%) had hypertension, 67.2% diabetes, and 64.2% chronic kidney disease. About one-quarter of patients (22.4%) were asymptomatic, while fatigue (46.3%), dyspnea (28.4%), and nausea/vomiting (20.9%) were the most common presenting symptoms. Normal ECGs were observed in 25.4% of patients, while alterations in 74.6%. Atrial fibrillation (13.4%), peaked T wave (11.9%), widened QRS (11.9%), prolonged PR interval (10.5%), and flattening P wave (10.5%) were the most common. Peaked T wave was significantly more common in severe hyperkalemia (87.5%) than in mild and moderate hyperkalemia (12.5%, 0.0%, respectively) (p=0.041). The physicians' sensitivities for predicting hyperkalemia were 35.8% and 28.4%, improved to 51.5% and 42.4%, respectively, when limiting the analyses to severe hyperkalemia. The mean (±SD) time to initial hyperkalemia treatment was 63.8±31.5 min. Potassium levels were positively correlated with PR interval (r=0.283, p=0.038), QRS duration (r=0.361, p=0.003), peaked T wave (r=0.242, p=0.041), and serum levels of creatinine (r=0.347, p=0.004), BUN (r=0.312, p=0.008), and CK (r=0.373, p=0.039). The physicians' abilities to predict hyperkalemia based on ECG and symptoms were poor. ECG could not be solely relied on, and serum potassium tests should be conducted for accurate diagnosis."}, {"id": "pubmed23n0972_14273", "title": "A case of massive pericardial effusion associated with hypocalcemic cardiomyopathy.", "score": 0.012924202397886608, "content": "A 60-year-old woman with a 6-year history of numbness in her hands was admitted to hospital with dyspnea. Laboratory findings showed the elevation of creatine kinase (creatine kinase MB isoenzyme was less than 4 IU/l). Chest X-ray revealed cardiomegaly and pulmonary edema. Electrocardiogram showed a T wave inversion in V<sub2-5</sub and a prolonged QT interval. Echocardiography demonstrated reduced left ventricular ejection fraction (LVEF) and massive pericardial effusion. The patient was diagnosed with heart failure. Further testing found hypocalcemia and idiopathic hypoparathyroidism. In addition to diuretics, calcium replacement therapy for hypocalcemia improved the LVEF and reduced pericardial effusion. Hypocalcemia rarely leads to heart failure and pericardial effusion. In our case, heart failure and the massive pericardial effusion were secondary to hypocalcemia due to idiopathic hypoparathyroidism. &lt;<bLearning objective:</b Hypocalcemia should be considered in patients with heart failure, reduced ejection fraction, and massive pericardial effusion. Supportive findings for diagnosis of heart failure caused by hypocalcemia are numbness, elevation of creatine kinase, T wave inversion, and prolonged QT interval.&gt;."}, {"id": "InternalMed_Harrison_17561", "title": "InternalMed_Harrison", "score": 0.012402167369054785, "content": "FIguRE 269e-24 Prominent U waves (II, III, and V4–V6) with ventricular repolarization prolongation in a patient with severe hypokalemia. CHAPTER 269e Atlas of Electrocardiography FIguRE 269e-25 Abbreviated ST segment such that the T wave looks like it takes off directly from QRS in some leads (I, V4, aVL, and V5) in a patient with severe hypercalcemia. Note also high takeoff of ST segment in V2/V3 simulating acute ischemia. PART 10 Disorders of the Cardiovascular System FIguRE 269e-26 SR with LVH, left atrial abnormality, and tall peaked T waves in the precordial leads with inferolateral ST depressions (II, III, aVF, and V6); left anterior fascicular block and borderline prolonged QT interval in a patient with renal failure, hypertension, and hyperkalemia; prolonged QT is secondary to associated hypocalcemia."}, {"id": "pubmed23n0765_8735", "title": "Global T-wave inversions with isolated hypomagnesemia.", "score": 0.012389847104014461, "content": "The physiological actions of magnesium within the cardiac conduction system and myocytes have yet to be fully elucidated. Because concurrent hypocalcemia or hypokalemia were also present in previous human reports, specific electrocardiographic effects of isolated hypomagnesemia have not been clearly delineated. We report a case in which dynamic electrocardiogram (ECG) changes were demonstrated in isolated hypomagnesemia. A 37-year-old man with history of heavy alcohol use was admitted for syncope. The ECG showed global T-wave inversions with prolonged corrected QT (QTc) duration on ECG. Extensive work-up including cardiac catheterization was unremarkable. His serum magnesium was noted to be low at 1.1 mg/dL, and his serum calcium and potassium were within normal limits. The patient received magnesium infusion with subsequent ECGs showing resolution of his global T-wave inversions and prolonged QTc. This case is unique because it reports dynamic ECG changes in a patient with isolated hypomagnesemia. Although isolated hypomagnesemia is commonly believed to result in dysrhythmia, we were unaware of any previous cases of ECG abnormalities in humans. Clinically, we advise checking serum magnesium and correcting hypomagnesemia when prolonged QTc duration and global T-wave inversions are seen on ECG."}, {"id": "wiki20220301en003_152371", "title": "Electrocardiography", "score": 0.012108621457189495, "content": "Electrolytes disturbances and intoxication: Digitalis intoxication Calcium: hypocalcemia and hypercalcemia Potassium: hypokalemia and hyperkalemia Serotonin Toxicity Ischemia and infarction: Wellens' syndrome (LAD occlusion) de Winter T waves (LAD occlusion) ST elevation and ST depression High Frequency QRS changes Myocardial infarction (heart attack) Non-Q wave myocardial infarction NSTEMI STEMI Sgarbossa's criteria for ischemia with a LBBB Structural: Acute pericarditis Right and left ventricular hypertrophy Right ventricular strain or S1Q3T3 (can be seen in pulmonary embolism) History"}, {"id": "article-29201_13", "title": "Sodium Polystyrene Sulfonate -- Monitoring", "score": 0.011627906976744186, "content": "Since SPS is not selective for potassium ions, other electrolyte imbalances may occur while using SPS in hyperkalemic patients. Patients should be monitored carefully for the signs and symptoms of other electrolyte abnormalities such as hypokalemia,  hypomagnesemia, and hypocalcemia. Hypokalemia may be associated with cardiac arrhythmias and severe muscle weakness. Hypokalemia may be evident as prolongation of the QT interval, T-wave inversions, prominent U waves (an extra upward wave after the T-wave) in Electrocardiogram (ECG). Some patients may present with significant symptoms of rebound hyperkalemia, which may be evident in ECG as a tall peaked T wave. Hypomagnesemia can present with muscle weakness and potentiate hypokalemia. Hypocalcemia presents with tremors, muscle weakness, tetany, and rarely, seizures."}, {"id": "pubmed23n0986_5808", "title": "Thyrotoxic periodic paralysis with ventricular tachycardia.", "score": 0.011241174284652544, "content": "A 47-year-old man presented to our emergency department (ED) with limbs weakness for 2 h. His heart rate was 127 beats per minute and blood pressure was 95/49 mm Hg. He found weakness of limbs after 4-h sleep. Physical examinations revealed that the muscle strength of upper limbs is 3/5, and lower limbs are 2/5. Electrocardiogram (ECG) revealed wide QRS complex, monomorphic ventricular tachycardia (VT) with ST-segment depression and long QT interval. Serum potassium level was extremely low as 1.0 mEq/L. This led to periodic hypokalemic paralysis. Due to severe hypokalemia with possible atrioventricular block, the patient was admitted to the intensive care unit. During hospitalization, his potassium level returned to 5.1 mEq/L on the first day. He had a low level of thyroid stimulating hormone (TSH) of &lt;0.03 micro-IU/mL (normal range: 0.25-4.00) and a high free thyroxine (T4) level of 2.43 ng/dL (normal range: 0.89-1.79 ng/dL). Therefore, hyperthyroidism was diagnosed, and 5 mg of methimazole was administered twice a day. The patient was discharged on the seventh day after admission. The final diagnosis is thyrotoxic periodic paralysis (TPP), also as known as nocturnal paralysis or night palsy."}, {"id": "pubmed23n0687_7613", "title": "Diagnosis and clinical approach in Gitelman's syndrome.", "score": 0.010947675724230856, "content": "Hypokalemia, defined as a plasma potassium concentration &lt;3.5 mmol/l, is the most common electrolyte abnormality encountered in our clinical practice. Unfortunately, in many cases, the etiologies were unclear and resulted in a wrong treatment. Indeed, the true etiology could be such a 'rare' one and could be found by doing a comprehensive work up. One of this is Gitelman's syndrome, a rare genetic disorder characterized by hypokalemic alkalosis, hypomagnesemia, hypocalciuria, and secondary aldosteronism without hypertension. Since this disorder is found in 1% Caucasian populations, this is one of the most frequently inherited renal tubular disorders. A 27 year old man came to emergency room with weakness and generalised muscle cramps. He was investigated three months before for a similar electrolyte disturbance which was found to be inconclusive. The routine laboratory data in emergency room revealed a potassium concentration of 2.3 mmol/l. He had never used diuretics or hormonal therapy nor had history of vomiting or diarrhea. He had normal blood pressure and the blood gas analysis revealed metabolic alkalosis. On his ECG (electrocardiography), we found the prominent U wave. Despite his low concentration of serum potassium and cloride, the concentration of these electrolytes in urine were extremely high. We also found hipomagnesemia. The calcium concentration in serum was normal with slightly hypocalciuria. Even with aggressive oral and intravenous potassium suplementation, the patient remained hypokalemic. In cases when the etiology of hypokalemia is unclear, we should perform some investigations to confirm the diagnosis and give the proper treatment. In Gitelman's syndrome, where the defect in the distal tubule cannot be corrected, the treatment must be a life-long. Most patients require oral potassium and magnesium supplementation, since drug therapy is usually incompletely effective."}, {"id": "InternalMed_Harrison_31544", "title": "InternalMed_Harrison", "score": 0.010913257447687742, "content": "Hyperkalemia is defined as a serum potassium level >5.5 mmol/L (>5.5 meq/L) and can neurologically present as muscle weakness with or without paresthesias. Hyperkalemia becomes life threatening when it produces electrocardiographic abnormalities such as peaked T waves or a widened QRS complex. In these cases, prompt treatment is essential and consists of strategies that protect the heart against arrhythmias (calcium gluconate administration); promote potassium redistribution into cells (with glucose, insulin, and β2-agonist medications); and increase potassium removal (through sodium polystyrene sulfonate, loop diuretics, or hemodialysis). Hypercalcemia usually occurs in the setting of either hyperparathyroidism or systemic malignancy. Neurologic manifestations include encephalopathy as well as muscle weakness due to reduced neuromuscular excitability. Seizures can occur but are more common in states of low calcium."}, {"id": "wiki20220301en003_152367", "title": "Electrocardiography", "score": 0.010533651931501393, "content": "Diagnosis Numerous diagnoses and findings can be made based upon electrocardiography, and many are discussed above. Overall, the diagnoses are made based on the patterns. For example, an \"irregularly irregular\" QRS complex without P waves is the hallmark of atrial fibrillation; however, other findings can be present as well, such as a bundle branch block that alters the shape of the QRS complexes. ECGs can be interpreted in isolation but should be applied – like all diagnostic tests – in the context of the patient. For example, an observation of peaked T waves is not sufficient to diagnose hyperkalemia; such a diagnosis should be verified by measuring the blood potassium level. Conversely, a discovery of hyperkalemia should be followed by an ECG for manifestations such as peaked T waves, widened QRS complexes, and loss of P waves. The following is an organized list of possible ECG-based diagnoses."}, {"id": "article-23269_10", "title": "Hypokalemia -- History and Physical", "score": 0.010522302706824247, "content": "Hypokalemia can result in a variety of cardiac dysrhythmias. Although cardiac dysrhythmias or ECG changes are more likely to be associated with moderate to severe hypokalemia, there is a high degree of individual variability and can occur with even mild decreases in serum levels. This variability is dependent on concomitant factors such as magnesium depletion, digitalis therapy, among others. Moreover, characteristic ECG changes do not manifest in all patients. The ECG changes that occur are T-wave flattening initially, followed by ST depression and the appearance of a U wave that can be difficult to distinguish from the T wave. The U wave is often seen in the lateral precordial leads of V4 to V6. Prolongation of the PR and QT interval can also occur. Risk of arrhythmias is highest in older patients, those with heart disease and those receiving digoxin or antiarrhythmic drugs. Administration of anesthesia in the setting of hypokalemia is also a risk for dysrhythmias and impaired cardiac contractility but more so with acute rather than chronic hypokalemia."}, {"id": "article-23182_14", "title": "Hyperkalemia -- Evaluation", "score": 0.010214280003198208, "content": "The first test that should be ordered in a patient with suspected hyperkalemia is an ECG since the most lethal complication of hyperkalemia is cardiac condition abnormalities which can lead to dysrhythmias and death. Elevated potassium causes ECG changes in a dose-dependent manner: K = 5.5 to 6.5 mEq/L ECG will show tall, peaked t-waves K = 6.5 to 7.5 mEq/L ECG will show loss of p-waves K = 7 to 8 ECG mEq/L will show widening of the QRS complex K = 8 to 10 mEq/L will produce cardiac arrhythmias, sine wave pattern, and asystole"}, {"id": "pubmed23n0634_595", "title": "Periodic paralysis: rare presenting symptom of thyrotoxicosis.", "score": 0.009900990099009901, "content": "Paralysis due to hypokalemia results from an acute shift of potassium into cells or excessive potassium deficit. In the absence of potassium deficit, it is observed in Familial Hypokalemic Periodic Paralysis and in Thyrotoxic Hypokalemic Periodic Paralysis (TPP). This report describes the initial presentation of hyperthyroidism as sudden quadriplegia associated with hypokalemia. A healthy 25-year-old Puerto Rican policeman came to the emergency room with sudden paralysis in the four extremities of five hours evolution. He woke up in the morning and could not get up. The day before admission his legs felt weak, and it was hard to get out of bed. He arrived home at 7:00 PM, ate pasta and vegetables, and went to sleep at 10:00 PM. He had no diarrhea or weight loss, no history of medications or illicit drugs. He has a cousin and an aunt with the diagnosis of hypo-thyroidism. The admission temperature was 36.0 degrees C, pulse 96 per minute, respiratory rate 18 per minute, blood pressure 160/70 mmHg. He was alert and oriented as to time, place and person. He could talk properly and was in no respiratory distress. He had no exophtalmos or lid lag. The thyroid was not enlarged or tender. No pseudoclubbing or pretibial edema was found. There was flaccid paralysis of all extremities, 0/5 legs and 1/5 arms. Deep tendon reflexes could not be elicited. The cranial nerves and sensory examination were normal. The hemogram was within normal limits as were the renal and liver functions. Serum sodium was 140 mEq/L, potassium 1.48 mEq/L, phosphorus 1.4 mEq/L. A random glucose was 155 mg/dl and the arterial Ph was 7.41. The urine potassium was 7.04 mEq/L, sodium 60.8 mg/dl. TSH levelwas &lt; 0.03 ug/d], TUP 50.69% (24-40%), T4 17.6 ug/dl (4.7-11.4 ug/dl) Free T4 Index 28.23. He was managed with intravenous potassium chloride, 80 mEq in a period of seven hours with cardiac monitor. The serum potassium level, after the infusion was completed, was 6.70 mEq/L. No cardiac arrhythmia was documented. Muscle strength recovery was gradual and it was complete 4 hours after the infusion was initiated. The next day the potassium level was within normal limits but a wide pulse pressure and tachycardia still persisted."}, {"id": "pubmed23n0496_7258", "title": "[Life threatening hyperkalemia: the value of the electrocardiogram].", "score": 0.00980392156862745, "content": "In two men, aged 19 and 64, with chronic renal insufficiency and subacute symptoms of malaise and weakness of the leg muscles, broad QRS complexes were seen in the ECG. The younger patient developed an asystole and resuscitation was unsuccessful. His blood potassium level was found to be 8.3 mmol/l. The older patient recovered after administration of calcium gluconate. His blood potassium level was found to be 8.5 mmol/l. An 80-year-old woman who was taking various drugs because of heart failure also complained of muscle weakness. Her blood potassium level was 7.2 mmol/l and her ECG showed narrow complexes. She recovered without calcium gluconate after a change in medication. Hyperkalemia is a potentially life-threatening electrolyte disorder that may require immediate treatment. The changes in the ECG, especially widening of the QRS complexes, are the most important clues to the severity of the hyperkalemia. A treatment protocol based on ECG changes may reduce the mortality in these patients."}, {"id": "wiki20220301en030_8778", "title": "Electrolyte imbalance", "score": 0.009708737864077669, "content": "Treatment Primary treatment of hypercalcemia consists of administering IV fluids. If the hypercalcemia is severe and/or associated with cancer, it may be treated with bisphosphonates. For very severe cases, hemodialysis may be considered for rapid removal of calcium from the blood. Hypocalcemia Hypocalcemia describes when calcium levels are too low in the blood, usually less than 8.5 mg/dL. Causes Hypoparathyroidism and vitamin D deficiency are common causes of hypocalcemia. It can also be caused by malnutrition, blood transfusion, ethylene glycol intoxication, and pancreatitis. Symptoms Neurological and cardiovascular symptoms are the most common manifestations of hypocalcemia. Patients may experience muscle cramping or twitching, and numbness around the mouth and fingers. They may also have shortness of breath, low blood pressure, and cardiac arrhythmias."}, {"id": "wiki20220301en025_37052", "title": "Torsades de pointes", "score": 0.009695443456362635, "content": "The ECG tracing in torsades demonstrates a polymorphic ventricular tachycardia with a characteristic illusion of a twisting of the QRS complex around the isoelectric baseline (peaks, which are at first pointing up, appear to be pointing down for subsequent \"beats\" when looking at ECG traces of the \"heartbeat\"). It is hemodynamically unstable and causes a sudden drop in arterial blood pressure, leading to dizziness and fainting. Depending on their cause, most individual episodes of torsades de pointes revert to normal sinus rhythm within a few seconds; however, episodes may also persist and possibly degenerate into ventricular fibrillation, leading to sudden death in the absence of prompt medical intervention. Torsades de pointes is associated with long QT syndrome, a condition whereby prolonged QT intervals are visible on an ECG. Long QT intervals predispose the patient to an , wherein the R-wave, representing ventricular depolarization, occurs during the relative refractory period at"}, {"id": "pubmed23n0475_2110", "title": "Electrocardiographic manifestations in patients with thyrotoxic periodic paralysis.", "score": 0.009615384615384616, "content": "Thyrotoxic periodic paralysis (TPP) commonly precedes the overt symptoms and signs of hyperthyroidism and may be misdiagnosed as other causes of paralysis (non-TPP). Because the cardiovascular system is very sensitive to elevation of thyroid hormone, we hypothesize that electrocardiographic manifestations may aid in early diagnosis of TPP. We retrospectively identified 54 patients who presented to the emergency department (ED) with hypokalemic paralysis during a 3.5-year period. Thirty-one patients had TPP and 23 patients had non-TPP, including sporadic periodic paralysis, distal renal tubular acidosis, diuretic use, licorice intoxication, primary hyperaldosteronism, and Bartter-like syndrome. Electrocardiograms during attacks were analyzed for rate, rhythm, conduction, PR interval, QRS voltage, ST segment, QT interval, U waves, and T waves. There were no significant differences in age, sex distribution, and plasma K+ concentration between the TPP and non-TPP groups. Plasma phosphate was significantly lower in TPP than non-TPP. Heart rate, PR interval, and QRS voltage were significantly higher in TPP than non-TPP. Forty-five percent of TPP patients had first-degree atrioventricular block compared with 13% in the non-TPP group. There were no significant differences in QT shortening, ST depression, U wave appearance, or T wave flattening between the 2 groups. Relatively rapid heart rate, high QRS voltage, and first-degree AV block are important clues suggesting TPP in patients who present with hypokalemia and paralysis."}, {"id": "pubmed23n0862_23184", "title": "Not your regular high: cardiac dysrhythmias caused by loperamide.", "score": 0.009523809523809525, "content": "Loperamide, a non-prescription anti-diarrheal agent, is a peripheral mu-opioid receptor agonist that is excluded from the blood-brain barrier by p-glycoprotein at therapeutic doses. Overdoses of loperamide penetrate the central nervous system (CNS), leading to abuse. We report cardiac conduction abnormalities and dysrhythmias after ingestion of a recreational supra-therapeutic dose of loperamide confirmed with an elevated blood loperamide concentration. A 48-year-old woman with a history of alcohol and benzodiazepine abuse presented to the emergency department (ED) with somnolence, weakness and slurred speech. She was taking 20 to 40 tablets of 2 mg loperamide 1-2 times/day for weeks along with clonazepam and whiskey. Vital signs were: blood pressure (BP), 124/90 mmHg; heart rate (HR), 88/min; respiratory rate(RR), 20/min; T, 36.9 °C; O2 saturation 100% on room air (RA). Glucose was 6.4 mmol/L. Electrocardiogram (ECG) had a ventricular rate of 58/min, QRS 164 ms, QT 582 ms with no discernable p-waves. Lactate was 3.5 mmol/L and potassium was 6.2 mEq/L. Labs were notable for an anion gap of 20 mEq/L, ethanol of 3.9 mmol/L, creatinine of 2.3 mg/dL and loperamide concentration of 210 ng/mL (average therapeutic plasma concentration 1.2 ng/mL). She became hypotensive, but responded to fluids. Following treatment for hyperkalemia with calcium, insulin, dextrose, and hypertonic sodium bicarbonate a repeat ECG had a ventricular rate of 66/min, QRS 156 ms, and QT 576 ms. Magnesium was given and pacer pads were placed. During the infusion of magnesium, her BP fell to 92/58 mmHg with a HR of 54/min, RR 14/min, O2 saturation of 97% on RA so the infusion was stopped. The ECG after the magnesium infusion had a ventricular rate of 51/min, QRS of 134 ms, and QT 614 ms. In the ICU she had multiple runs of non-sustained ventricular tachycardia that did not require therapy. Over the next 48 h she improved and was transferred to a floor bed. On day four of hospitalization the patient left against medical advice. At that time, her ECG showed sinus tachycardia with a heart rate 114/min, QRS 82 ms, QT 334 ms. Loperamide produces both QRS and QT prolongation at supra-therapeutic dosing. A blood loperamide concentration of 210 ng/mL is among the highest concentrations reported. Supra-therapeutic dosing of loperamide is promoted on multiple drug-use websites and online forums as a treatment for opioid withdrawal, as well as for euphoric effects. With the current epidemic of prescription opioid abuse, toxicity related to loperamide, an opioid agonist that is readily available without a prescription is occurring more frequently. It is important for clinicians to be aware of the potentially life-threatening toxicity related to loperamide abuse in order to provide proper diagnosis, management and patient education."}, {"id": "wiki20220301en031_37318", "title": "Hypovolemic shock", "score": 0.009433962264150943, "content": "Again, the above is outlined for a healthy 70 kg individual. Clinical factors must be taken into account when assessing patients. For example, elderly patients taking beta blockers can alter the patient's physiologic response to decreased blood volume by inhibiting mechanism to increase heart rate. As another, patients with baseline hypertension may be functionally hypotensive with a systolic blood pressure of 110 mmHg. Non-bleeding Various laboratory values can be abnormal in hypovolemic shock. Patients can have increased BUN and serum creatinine as a result of pre-renal kidney failure. Hypernatremia or hyponatremia can result, as can hyperkalemia or hypokalemia. Lactic acidosis can result from increased anaerobic metabolism. However, the effect of acid–base balance can be variable as patients with large GI losses can become alkalotic."}, {"id": "pubmed23n0634_3064", "title": "Ventricular fibrillation as the first manifestation of primary hyperaldosteronism.", "score": 0.009433962264150943, "content": "50 years old female patient, with history of diabetes mellitus and hypertension, receiving metformin (500 mg BID) and atenolol (50 mg BID), presented to the Emergency Room with asthenia and dizziness. The patient was also receiving alternative medication (Dragon Blanco) which contains no licorice. During the emergency workup she developed syncope and three episodes of ventricular fibrillation. She was electrically defibrillated and treated with amiodarone and potassium replacement. The patient was admitted to the Intensive Care Unit. Physical exam: BP: 160/90 mm Hg, RR: 15, Pulse: 83: Cardiovascular: grade II systolic murmur which irradiated to the neck. The rest of the examination was unremarkable. Labs: Na: 138 meg/dl, K: 1.6 meg/dl, Cl: 84 meg/dl, BUN: 17 mg/dl, Creat.: 1.1 mg/dl, Gluc.: 148 mg/dl, Renin: &lt; 0.15 mcgr/ml, Aldosterone: 20.1 mcg%. Aldosterone-Renin ratio: 133. Chest X-Ray: cardiomegaly. EKG: RBBB, long QT segment and prominent broad \"u\" waves compatible with severe hypokalemia. A CT SCAN of the Abdomen/Pelvis showed a 3.2 cm right adrenal mass, most likely adenomatous. The patient was discharged with the diagnosis of primary aldosteronism. Due to the diagnosis of diabetes mellitus, hypertension and the three episodes of ventricular fibrillation, surgical treatment was postponed until stress tests and eventual coronary angiographic studies were performed. We found in our review of the medical literature 9 reports of fibrillation associated with hyperaldosteronism. Of those, only two were associated with primary aldosteronism, one of them with a fatal outcome. This case is highly unusual and emphasizes the importance of an adequate diagnosis of secondary hypertension."}, {"id": "pubmed23n0918_6418", "title": "Severe Hyperkalemia: Can the Electrocardiogram Risk Stratify for Short-term Adverse Events?", "score": 0.009386486959565902, "content": "The electrocardiogram (ECG) is often used to identify which hyperkalemic patients are at risk for adverse events. However, there is a paucity of evidence to support this practice. This study analyzes the association between specific hyperkalemic ECG abnormalities and the development of short-term adverse events in patients with severe hyperkalemia. We collected records of all adult patients with potassium (K+) ≥6.5 mEq/L in the hospital laboratory database from August 15, 2010, through January 30, 2015. A chart review identified patient demographics, concurrent laboratory values, ECG within one hour of K+ measurement, treatments and occurrence of adverse events within six hours of ECG. We defined adverse events as symptomatic bradycardia, ventricular tachycardia, ventricular fibrillation, cardiopulmonary resuscitation (CPR) and/or death. Two emergency physicians blinded to study objective independently examined each ECG for rate, rhythm, peaked T wave, PR interval duration and QRS complex duration. Relative risk was calculated to determine the association between specific hyperkalemic ECG abnormalities and short-term adverse events. We included a total of 188 patients with severe hyperkalemia in the final study group. Adverse events occurred within six hours in 28 patients (15%): symptomatic bradycardia (n=22), death (n=4), ventricular tachycardia (n=2) and CPR (n=2). All adverse events occurred prior to treatment with calcium and all but one occurred prior to K<sup+</sup-lowering intervention. All patients who had a short-term adverse event had a preceding ECG that demonstrated at least one hyperkalemic abnormality (100%, 95% confidence interval [CI] [85.7-100%]). An increased likelihood of short-term adverse event was found for hyperkalemic patients whose ECG demonstrated QRS prolongation (relative risk [RR] 4.74, 95% CI [2.01-11.15]), bradycardia (HR&lt;50) (RR 12.29, 95%CI [6.69-22.57]), and/or junctional rhythm (RR 7.46, 95%CI 5.28-11.13). There was no statistically significant correlation between peaked T waves and short-term adverse events (RR 0.77, 95% CI [0.35-1.70]). Our findings support the use of the ECG to risk stratify patients with severe hyperkalemia for short-term adverse events."}, {"id": "wiki20220301en083_35402", "title": "Apparent mineralocorticoid excess syndrome", "score": 0.009345794392523364, "content": "Apparent mineralocorticoid excess is an autosomal recessive disorder causing hypertension (high blood pressure), hypernatremia (increased blood sodium concentration) and hypokalemia (decreased blood potassium concentration). It results from mutations in the HSD11B2 gene, which encodes the kidney isozyme of 11β-hydroxysteroid dehydrogenase type 2. In an unaffected individual, this isozyme inactivates circulating cortisol to the less active metabolite cortisone. The inactivating mutation leads to elevated local concentrations of cortisol in the aldosterone sensitive tissues like the kidney. Cortisol at high concentrations can cross-react and activate the mineralocorticoid receptor due to the non-selectivity of the receptor, leading to aldosterone-like effects in the kidney. This is what causes the hypokalemia, hypertension, and hypernatremia associated with the syndrome. Patients often present with severe hypertension and end-organ changes associated with it like left ventricular"}, {"id": "pubmed23n0768_3849", "title": "Severe hypokalemic paralysis as a manifestation of a mitochondrial disorder.", "score": 0.009345794392523364, "content": "Mitochondrial disorder (MtD) is usually a multisystem disease due to impaired mitochondrial energy production. Severe hypokalemia resulting in muscle weakness and rhabdomyolysis has not been reported as a phenotypic feature of MtD. Here we describe a 60-year-old male patient who developed myalgias followed by generalized muscle weakness a few days before admission. Symptoms were attributed to severe hypokalemia that occurred after the patient had discontinued spironolactone, a competitive antagonist of the aldosterone receptor, four months earlier on his own judgment. Spironolactone was given for 10 years to treat suspected primary hyperaldosteronism (Conn's syndrome). He presented with myopathic face, bilateral ptosis, hypertelorism, brachydactylia, weakness of the axial and limb muscles, and bilateral leg edema. Hypertelorism and brachydactylia are known as physical traits of MtD. Laboratory investigations revealed hypokalemia of 1.7 mmol/l and elevated serum levels of creatine kinase (2,772 U/l). Electrocardiogram showed sinus rhythm, left bundle-branch-block, repolarization abnormalities, and prolonged QTc (571 ms), which is associated with a propensity to ventricular arrhythmias. Diagnostic work-up revealed bilateral adenomas of the suprarenal glands. Conn's syndrome was regarded as a manifestation of MtD, since MtDs are frequently associated with endocrine abnormalities. The patient also presented with occasional double vision, ptosis, renal insufficiency, bilateral renal cysts, hypertriglyceridemia, arterial hypertension, and hypertrophic cardiomyopathy. Taken together, we have made the diagnosis of MtD. In conclusion, MtD may be associated with adrenal adenomas, which may cause severe symptomatic hypokalemia, manifesting as generalized weakness and myalgias due to rhabdomyolysis. Endocrine involvement may be a phenotypic feature of MtD. "}, {"id": "pubmed23n0991_24383", "title": "A somnolent woman in her fifties with acute circulatory failure.", "score": 0.009259259259259259, "content": "A woman in her fifties was admitted to hospital with decreased awareness and circulatory failure. She had been treated with left atrial cryoablation a few weeks before admission and had been cardioverted a few days after the procedure because of relapse of atrial fibrillation. On admission, the patient had systolic blood pressure of 80 mm Hg and an ECG with broad QRS-complexes at 380 ms. We suspected intoxication and she was intubated to administer activated charcoal after gastric lavage. She was cardiovascularly unstable and in need of intravenous infusion of noradrenaline and adrenaline. Further investigations at her home suggested that she had poisoned herself with 4-5 g flecainide, 0.3 g oxazepam and 0.5 g meclizine. After administration of 500 mmol sodium bicarbonate and 5 mmol calcium chloride, the QRS complexes narrowed temporarily. On day 2, due to sustained bradycardia and hypotension despite receiving adrenergic medications, a temporary pacemaker was implanted, leading to improved heart rate and blood pressure. She experienced several complications including hypertensive pulmonary oedema, atrial fibrillation, extensively prolonged QT interval because of polypharmacy and Takotsubo cardiomyopathy. She was discharged from the hospital in good health on day 17. At a follow-up visit at the outpatient clinic 12 weeks later, cardiac function had normalised. The QT interval was now normal; however, there were persistent T-wave inversions in leads I, aVL and V4-6. Flecainide blocks sodium channels in cardiomyocytes. Intoxication with flecainide is rare, with mortality rates of about 10 %. Sodium bicarbonate in larger doses has been reported to stabilise patients with flecainide intoxication due to modification of the binding of flecainide to sodium receptors in cardiomyocytes, and due to alkalisation which makes flecainide detach from sodium receptors. Our patient had a temporary effect with narrowing of QRS complexes after receiving sodium bicarbonate. She also showed a beneficial effect from implantation of a temporary pacemaker, although earlier case reports have described problems with high thresholds and capture failure."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[130, 204]], "word_ranges": [[22, 32]], "text": "pH between 7.20-7.25 Prepathological value. Repeat microtome in 15-20 min."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "- pH less than 7.20 Pathological value. Indication for fetal extraction by the quickest route, in this case a cesarean section. - pH between 7.20-7.25 Prepathological value. Repeat microtome in 15-20 min. - pH greater than 7.25. Value within the limits of normality. Observation.", "full_answer_no_ref": "- pH less than 7.20 Pathological value. [HIDDEN], in this case a cesarean section.\n- pH between 7.20-7.25 Prepathological value. Repeat microtome in 15-20 min.\n- pH greater than 7.25. Value within the limits of normality. Observation.", "full_question": "Woman 40 weeks gestation in labor with 6 cm dilation. She presents a decelerative fetal pattern in cardiotocographic recordings, so it is decided to perform a fetal blood smear to assess fetal well-being. Result 7.22. The correct conduct is:", "id": 208, "lang": "en", "options": {"1": "Severe acidosis. Urgent cesarean section.", "2": "Pre-pathological value repeat microtome 15-20 minutes.", "3": "Moderate acidosis. Repeat microtome in 1-2 hours.", "4": "Value in normal limits, leave natural evolution of labor.", "5": "Repeat at the time of possible error in obtaining the shot."}, "question_id_specific": 183, "type": "GYNECOLOGY AND OBSTETRICS", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0305_3664", "title": "[Pre-pathologic-pathologic cardiotocographic pattern in second stage of labor. Analysis of the incidence of acidosis and recommendations for fetal blood gas analysis].", "score": 0.01667129758266185, "content": "Fetal heart rate (FHR) patterns from 746 consecutive, documented vaginal deliveries within a 1 year period were reported on using the Hammacher Score. Characteristic FHR patterns were described and the frequency of acidosis calculated. FHR score, the single FHR parameters, baseline (BL), floatingline (FL) and oscillation type (OT) and the acid-base balance of the neonate were submitted to a correlation analysis according to Spearman. FHR patterns reported as ominous (FHR score &gt; or = 5) were observed in 25.9% and were associated with a frequency of acidosis (pHUA &lt; or = 7.20) of 38.1% Suspicious fetal heart rate patterns (FHR score 3-4) were seen in 60%, here the frequency of acidosis was 8.5%. With the inclusion of decelerations by the parameter FL an increased frequency of acidosis of 29% was registered only when 4 points were allocated. Total FHR score and the score parameter baseline (BL) correlated closest with the pH changes at the end of birth. Tachycardic FHR patterns showed the highest frequency of acidosis (55%) and ominous tracings (83%). The commonest FHR pattern, normocardia with decelerations (48%) exhibited only a low frequency of acidosis (8%) and ominous tracings (15%) with an average pH value of 7.27 +/- 0.08. To prevent an unnecessary operative delivery in the presence of an ominous FHR finding, whether in the late first stage or early second stage when birth is not imminent, a fetal blood analysis should be carried out. With a suspiciously assessed fetal heart rate pattern the fetal blood analysis will only rarely reveal a severe acidosis (pHUA &lt; or = 7.10)."}, {"id": "pubmed23n0517_8370", "title": "[Reference values range of the fetal oxygen saturation and its dispersal during labor without cardiotocographic evidence for fetal distress].", "score": 0.016023626995098655, "content": "The objective of this study is to establish the reference values range of the fetal oxygen saturation during the first and the second period of labor and their dispersal according to the extent of cervical dillatation in cases with normal FHR--absence of fetal hypoxia and asphyxia of the newborn. This is a prospective study which involves 94 women with normal FHR. All of the newborns are with umbilical artery pH values greater than 7.15 and 5 min Apgar score greater than 7; there was no necessity for any reanimation procedures, assisted ventilation or intensive care treatment. The fetal oxygen saturation (SpO2) is monitored by fetal pulseoxymeter Nellcor N 400, fetal sensors FS - 14. Cardiotocographic monitoring is carried out simultaneously. Blood is obtained from the fetal scalp during labor for blood gas and pH analysis, and umbilical artery pH as well as the Apgar score of the newborn are determined. The average monitoring time during the first period of labor is 107.19+/-29.49 min. with reliability of the recordings 86.54+/-6.10%. The average monitoring time for the second period of labor is 36.72+/-8.31 min. with reliability of the recordings 75.42 +/-9.61%. The mean SpO2 values are 48.71+/-5.52% during the first period and 47.30+/-4.62% during the second period of labor. The reference SpO2 values ranging between the 25-th and 75-th percentile in fetuses with normal FHR are 46-52 % for the first and 44-50 % for the second period. The results for fetal SpO2 during the different stages of cervical dillatation are as follows: for 4-5 cm - 49.49+/-5.12%, for 6-7 cm - 48.76+/-5.42%, for 8-9 cm - 48.39+/-5.49%. The fetal SpO2 dispersal during cervical dillatation of 4-5, 6-7 and 8-9cm accordingly demonstrates a nonsignificant decrease of SpO2 for the different groups (p&gt;0.05). The fetal SpO2 dispersal between the first and the second period of labor also demonstrates decrease of SpO2 values and shows a minor statistically significant difference (p &lt; 0.05 - Repeated measures ANOVA), which is considered to be within the normal range and does not reflect on the newborn's well-being."}, {"id": "pubmed23n0001_274", "title": "Recognition and significance of maternogenic fetal acidosis during intensive monitoring of labor.", "score": 0.015746109246261825, "content": "FHR monitoring and microanalysis of fetal blood are mutually complementary procedures, and optimal knowledge of the fetal state is achieved by making use of both, the former for the preliminary screening of all cases at risk and the latter for the purpose of deciding on obstetric management where pathological changes are evident in the FHR. The major difficulty in obtaining a precise value for the fetal acid-base balance lies in the occurence of \"falsely abnormal\" cases, i.e. cases in which the fetal pH falls during labor but the clinical condition at birth is good (APGAR greater than or equal to 7). In our own series the incidence of such cases among fetuses at risk was 11.2% (Tab. I). In the majority of these cases the fetal acidosis is thought to be a result of increased metabolic acidosis in the mother (maternogenic fetal metabolic acidosis). The importance of the maternogenic fetal acidosis during labor lies in the fact that unless it is recognised, rapid extraction of the fetus will appear necessary on clinical grounds, although it is in fact unnecessary, since this form of acidosis has no adverse effect on the fetus. Various parameters have been proposed for the differential diagnosis of the maternogenic fetal acidosis. These include the feto-maternal difference in base deficit (F/M deltaBD), the materno-fetal differences in pHqu 40 (M/F deltapHqu 40) the materno-fetal difference actual pH (M/F actual deltapH), and the materno-fetal difference in base deficit of the extra-cellular fluid (M/F deltaBDHb5). A critical analysis of these parameters has been carried out on the results of microtests performed during a 5 year period (1968-1972) at the First Clinic of Obstetrics and Gynecology of Milan University. The cases comprised 59 regarded as normal (normal course of pregnancy, spontaneous commencement of labor at term, clear amniotic fluid, regular FHR, spontaneous birth, APGAR at 90 sec between 8 and 10, weight at birth greater than 2500 g), and 335 considered to be at risk (maternal disease, presence of meconium stained amniotic fluid and/or abnormal changes in FHR). In all of these cases the FHR was recorded by cardiotokography, and the tracings were interpreted according to HON. Microsamples of blood were taken from both mother and fetus during labor and the following determinations were carried out: actual pH, pHqu 40, Hb concentration, hemoglobin oxygen saturation, base deficit Hb5 (BDHb5). The maternofetal differences were then calculated. The same determinations were carried out on samples of maternal blood and of arterial and venous cord blood taken immediately after delivery. The clinical condition of the infant was evaluated by the APGAR score at 90 seconds after birth."}, {"id": "pubmed23n0590_11317", "title": "[Fetal monitoring during the active second stage of labor].", "score": 0.015727149377081535, "content": "To determine the best way of fetal monitoring during the active second stage of labor. Articles were searched using PubMed and Cochrane library. Active phase of labor begins with the onset of maternal pushing. It is characterised by frequent and prolonged uterine contractions as well as maternal bearing down efforts. Altogether those mechanical forces lead to an intrauterine pressure increase up to 250 mm Hg and to a marked reduction in placental perfusion. A prolonged period of expulsion will lead to an impairment of fetal oxygenation as well as a rise in carbon dioxide level. Melchior's FHR classification is specific of the active second stage of labor and described five fetal heart rate patterns: from type 0 to type 4. A reduction in pH and a rise in lactate and P(CO2) values occurred as one progresses from type 0 to type 4 (NP4). During the active phase of labor every method has a significant rate of signal loss. Loss of sensor contact occurred in up to 64% of time with oximetry, 35 to 48% of recordings obtained via external Doppler sensors have more than 20% of signal loss. Even 8 to 11% of recordings obtained via scalp sensors have more than 20% of signal loss. Fetal scalp blood sampling does not allow a continuous recording of fetal well-being and is difficult to perform during this stage. No method has a 100% sensitivity to detect metabolic acidosis. Normal FHR of 1.3% are coupled with an acidosis. Even ST analysis exhibited a small but real false negative rate (NP4). Active second phase of labor is at high risk of fetal acidosis, and required a close follow-up of the FHR. The length of maternal bearing down efforts should be matched to the fetal heart rhythm Melchior's classification pattern. Optimal length of bearing down efforts could be 30 min for type 0, 20 min for type 1 and 10 min for type 2, 3 or 4 (NP4)."}, {"id": "pubmed23n0217_6989", "title": "pH measurement from the fetus during labor--a task for the midwife?", "score": 0.01542935137380571, "content": "After theoretical education on the development of fetal acidosis and the fetal blood sampling technique, 12 midwives in a training program carried out or assisted in 40 fetal blood sampling procedures. Sampling was possible at a cervical dilatation of 1.5 cm. 11/12 midwives were successful in their first sampling attempt. The instrument used for analysis was an automated microprocessor device with a minimum sampling volume of 15 microliter of blood. There was a statistically highly significant correlation between the results obtained with the instrument tested and the reference device (r = 0.9; n = 36). 82% of the measurements fell within +/- 0.04 pH units of the reference device. With the instrument placed in the labor room, and using the current technique with very small blood samples, it is possible to obtain a fetal pH value within 10 minutes after the development of a pathological fetal heart rate pattern."}, {"id": "pubmed23n0791_23278", "title": "Fetal heart rate pattern interpretation in the second stage of labor using the five-tier classification: impact of the degree and duration on severe fetal acidosis.", "score": 0.014393052302888367, "content": "The aim of this study was to clarify the association between fetal heart rate (FHR) tracing interpretation levels in the second stage of labor and poor fetal acid-base balance. The database at one tertiary hospital in Nagoya, Japan, was retrospectively reviewed for women with singleton fetuses in cephalic presentation and vaginal labor at ≥37 + 0 gestational weeks between 1 June 2011 and 30 April 2012. Continuous FHR tracings in the second stage of labor were subdivided into 15-min intervals, each of which we called a window, from the beginning of labor through delivery, and were assessed according to the five-tier classification proposed by the Japan Society of Obstetrics and Gynecology, in which level 1 is normal, level 2 is subnormal, and levels 3-5 are abnormal patterns. In total, 777 parturient women were eligible for the study protocol. The numbers of women with maximal levels of 1, 2, 3, 4, and 5 were 3, 77, 341, 349, and 7, respectively. No cases of severe fetal acidosis (pH &lt; 7.0 or base excess &lt;-12 mmol/L) were recorded when the maximal levels were below 3. Both the pH and base excess of the umbilical artery decreased with higher levels of FHR tracings interpretation (P &lt; 0.001). Both the summations of level-4 windows and level-3 and level-4 windows were significantly higher in women with severe fetal acidosis than in women without (P &lt; 0.001), indicating that the duration of abnormal levels is associated with severe fetal acidosis. Both the degree and duration of FHR tracing abnormalities correlate with severe fetal acidosis."}, {"id": "pubmed23n0269_6725", "title": "The effect of labor on the normal values of umbilical blood acid-base status.", "score": 0.013709677419354839, "content": "Although several investigators have attempted to define the normal values of umbilical cord blood pH and gases, there is considerable controversy about the optimal cutoff values to diagnosis intrauterine asphyxia. A possible reason for this might be that several studies have included data from fetuses born after different duration of labor. To determine the effect of labor and the duration of second stage on labor on umbilical arterial acid-base status at birth in healthy term infants. Umbilical artery acid-base status was determined in patients (n = 356) who met the following criteria: 1) singleton term pregnancy with no significant medical, obstetric, or neonatal complications; 2) vertex presentation if delivered vaginally; 3) neither regional nor general anesthesia applied if delivered vaginally; 4) no use of oxytocin; 5) normal fetal heart rate patterns; 6) clear amniotic fluid; 7) Apgar scores at 1 and 5 min &gt; or = 7; 8) appropriate fetal weight for gestational age. Patients were divided into three groups. A-patients delivered by cesarean section (CS) in the absence of labor (n = 135); B-patients delivered by CS during first stage of labor (n = 62); C-patients with vaginal birth (n = 159). A Kruskal-Wallis ANOVA with post-hoc procedures and stepwise multiple regression analysis were performed. 1) There were significant differences in cord arterial acid-base values between study groups (pH: no labor CS, 7.27 +/- 0.05 vs labor CS, 7.26 +/- 0.05 vs vaginal birth, 7.24 +/- 0.07, p &lt; 0.00001). 2) There was a significant fall in cord arterial pH and bicarbonate concentrations with increased duration of second stage of labor in newborns born vaginally (pH: duration of second stage, 1-30 min, 7.25 +/- 0.07 vs 31-60 min, 7.22 +/- 0.06 vs &gt; 61 min, 7.21 +/- 0.07, p &lt; 0.05). 3) Analysis of confounding variables which could influence the cord pH such as parity, use of vacuum, gestational age, maternal age, and birth weight by stepwise multiple regression analysis indicated that only the duration of second stage of labor pain had a significant relationship with cord blood pH. There is a significant fall in umbilical artery pH and bicarbonate with the presence of labor and increased duration of second stage of labor in healthy term neonates. This should be taken into consideration in evaluating neonatal well-being by cord blood pH and acid-base measurements."}, {"id": "pubmed23n0365_20287", "title": "Clinical usefulness of pulse oximetry in the fetus with non-reassuring heart rate pattern?", "score": 0.013189143341815097, "content": "The objective of this study was the evaluation of intrapartum pulse oximetry as an indicator of fetal distress and the condition of the newborn during clinical routine surveillance in an University Perinatal Center. Between 1998 and 1999 pulse oximetry (SpO2) was used additionally to routine fetal monitoring by electronic fetal heart rate tracing (CTG) and fetal blood sampling (FBA) in 128 cases with nonreassuring heart rate pattern. Cut off values were FIGO Score &lt; 8 for the heart rate pattern and for fetal blood sampling during labor results of &lt; 7.25 (preacidosis). The condition of the newborn was defined by the APGAR score with the cut off &lt; 7 at 1 minute, while the biochemical status was evaluated by means of arterial blood sampling of the umbilical artery directly after birth using a pH of &lt; 7.20 to verify acidosis. Predictive values of critically low SpO2 values (&lt; 30%) for at least 10 minutes as well as corresponding sensitivities and specificities were calculated together with 95% confidence intervals to identify fetal distress or a depressed condition of the newborns. Of 128 fetuses included in this study 66 (52%) were born spontaneously, 23 (18%) were born by operative vaginal delivery and 39 (31%) by means of cesarean section. The high rate of cesarean section was due to cephalopelvic disproportion in 29 cases. Fetal outcome was evaluated with a clinical score: mean APGAR score value 8.5 SD +/- 1. The mean value of the pH in the umbilical artery was 7.23 +/- 0.04. During a SpO2 monitoring period of 18,381 minutes we analyzed a contact time of 63%. Comparing SpO2 values of &lt; 30% with preacidosis in the fetal blood sampling, we found a positive predictive value of merely 0.17 (95% CI: 0.00-0.64). Of 9 preacidotic cases during delivery only 1 was indicated by a saturation value below 30% (sensitivity 0.11, 95% CI: 0.00-0.48). The specificity and negative predictive value were calculated as 0.83 (95% CI: 0.65-0.94) and 0.76 (95% CI: 0.58-0.89) respectively. Of eleven cases with acidosis in the blood of the umbilical cord artery, pH &lt; 7.20, only 2 were indicated by a SpO2 values below 30%. Which is equivalent to a sensitivity of 0.18 (95% CI: 0.03-0.52). Results of a receiver operator curve analysis showed no substantial deviation from the diagonal. The area under the curve was 0.62, the 95% CI (0.47-0.76) indicating no significant discrimination. Three of 49 fetuses with SpO2 recording during the last 10 minutes were born in clinical depressed status (APGAR &lt; 7). None was indicated by a SpO2 value below 30%. Fetal distress and impaired condition of the newborn are not identified or predicted during routine application of SpO2 monitoring in the fetus during labor with adequate safety."}, {"id": "pubmed23n1065_18374", "title": "Impaired validity of the new FIGO and Swedish CTG classification templates to identify fetal acidosis in the first stage of labor.", "score": 0.013181347150259069, "content": "Cardiotocography (CTG) is the main method of intrapartum fetal surveillance. In 2015 a new guideline was introduced by the International Federation of Gynecology and Obstetrics (FIGO), FIGO-15. In Sweden it was adjusted to SWE-17, replacing the previous national template, SWE-09. This study, conducted at one university hospital and one regional hospital in southern Sweden, evaluated the diagnostic validity of these three templates to detect fetal acidosis during the first stage of labor. A total of 73 neonates with pH &lt;7.1 in umbilical cord artery or vein at cesarean delivery during the first stage of labor were identified retrospectively. For each acidotic neonate, three non-acidemic neonates, with a pH ≥7.2 in cord artery and vein, and Apgar scores ≥9 at five and ten minutes, in all 219 neonates, were selected. The CTG tracings before birth in acidemic neonates, and tracings at the same cervical dilatation in the non-acidemic neonates, were independently assessed by three professionals from the obstetric staff, blinded to group and clinical data. Based on their categorizations of the included variables (baseline, variability, accelerations, decelerations and contraction rate), each CTG tracing was systematically classified according to the three templates. The sensitivity and specificity to identify acidemia by the classification pathological were determined for each template. Interobserver agreement in the assessments of tracings as pathological or not was analyzed, using free-marginal Kappa index. The sensitivity for patterns classified as pathological to identify acidemia was similar for FIGO-15 (71%) and SWE-17 (77%, <ip</i = .13), and the specificity was 97% for both. SWE-09 had a significantly higher sensitivity (95%, <ip</i &lt; .001) albeit with a lower specificity (90%, <ip</i &lt; .001) than the other two templates. Among acidemic neonates, the fraction of tracings classified as normal was higher with SWE-17 (9.6%) than with SWE-09 (0%; <ip</i = .01) and FIGO-15 (1.4%; <ip</i = .06). For tracings from neonates with acidemia, agreement for three independent assessors was strong (κ 0.85) with SWE-09, and weak for FIGO-15 (κ 0.47), and SWE-17 (κ 0.51). For tracings from neonates without acidemia, the agreement was almost perfect for FIGO-15 (κ 0.91), strong withSWE-17 (κ 0.90) and moderate with SWE-09 (κ 0.78). The ability of FIGO-15 and SWE-17 to identify fetal acidosis is considered insufficient. The combination of a high sensitivity and a high specificity makes SWE-09 the most discriminatory template during the first stage of labor."}, {"id": "pubmed23n0218_7701", "title": "[Lowering the cesarean section rate by exclusion of cardiotocographically suspected acidosis using fetal blood gas analysis].", "score": 0.012924606462303232, "content": "In 871 deliveries out of a total of 3980 effected during 1980-1982 at the Winterthur Gynaecological Hospital, we found it necessary to perform one or several microanalyses of blood (blood gas analyses) sub partu. Among these, we found retrospectively that in 22 cases the microanalysis of the blood gas had prevented us from performing caesarean section which would have seemed necessary if we had relied on the cardiotocographical findings alone. Micro-analysis of blood gas made it possible to exclude the presence of pre-acidosis or acidosis which would have made Caesarean section imperative; in all these cases, normal delivery via the vagina was achieved, and in no case did this result in severe acidosis. All newborn had a 5-minute Apgar score of 7 and higher. We can conclude from these results, therefore, that the indication for an immediate termination of delivery via Caesarean section should not depend solely on a pathological cardiotocogram; the final decision should be arrived at only after micro-analyses of blood gas have confirmed the presence of foetal pre-acidosis or acidosis."}, {"id": "pubmed23n1048_5423", "title": "Earlier and improved screening for impending fetal compromise.", "score": 0.012837625628140704, "content": "The use of pH and base excess (FSSPHBE) from fetal scalp sampling (FSS) was abandoned when cardiotocography (CTG) was believed to be sufficiently accurate to direct patient management. We sought to understand the fetus' tolerance to stress in the 1st stage of labor and to develop a better and earlier screening test for its risk for developing acidosis. To do so, we investigated sequential changes in fetal pH and BE obtained from FSS in the 1st stage of labor as part of a research protocol from the 1970s. We then examined the utility of multiple of the median (MoM's) conversion of BE and pH values, and the capacity of Fetal Reserve Index (FRI) scores to be a proxy for such changes. We then sought to examine the predictive capacity of 1st stage FRI and its change over the course of the first stage of labor for the subsequent development of acidosis risk in the 2nd stage of labor. Using a retrospective research database evaluation, we evaluated FSSPHBE data from 475 high-risk parturients monitored in labor and their neonates for 1 h postpartum.We categorized specimens according to cervical dilatation (CxD) at the time of FSSPHBE and developed non-parametric, multiples of the median (MOMs) assessments. FRI scores and their change over time were used as predictors of FSSPHBE. Our main outcome measures were the changes in BE and pH at different cervical dilatations (CxD) and acidosis risk in the early 2nd stage of labor. FSSPHBE worsens over the course of the 1st stage. The implications of any given BE are very different depending upon CxD. At 9 cm, -8 Mmol/L is 1.1 MOM; at 3 cm, it would be 2.0 MOM. The FRI level and its trajectory provide a 1st stage screening tool for acidosis risk in the second stage. Fetal acid-base balance (\"reserve\") deteriorates beginning early in the 1st stage of labor, irrespective of whether the fetus reaches a critical threshold of concern for actual acidosis. The use of MoM's logic improves appreciation of such information. The FRI and its trajectory reasonably approximate the trajectory of the FSSPHBE and appears to be a suitable screening test for early deterioration and for earlier interventions to keep the fetus out of trouble rather than wait until high risk status develops."}, {"id": "wiki20220301en022_65589", "title": "Cardiotocography", "score": 0.012459085629817339, "content": "Updated 2015 FIGO Intrapartum Fetal Monitoring Guidelines FIGO has recently modified the guidelines on intrapartum fetal monitoring, proposing the following interpretation: Normal: No hypoxia or acidosis; no intervention necessary to improve fetal oxygenation state. Baseline 110–160bpm Variability 5–25bpm No repetitive decelerations (decelerations are defined as repetitive when associated with >50% contractions) Suspicious: Low probability of hypoxia/acidosis, warrants action to correct reversible causes if identified, close monitoring or adjunctive methods. Lacking at least one characteristic of normality, but with no pathological features. Pathological: High probability of hypoxia/acidosis, requires immediate action to correct reversible causes, adjunctive methods, or if this is not possible expedite delivery. In acute situations, delivery should happen immediately. Baseline <100bpm Reduced or increased variability or sinusoidal pattern"}, {"id": "wiki20220301en022_65560", "title": "Fetal distress", "score": 0.011894132653061225, "content": "Signs and symptoms Generally it is preferable to describe specific signs in lieu of declaring fetal distress that include: Decreased movement felt by the mother Meconium in the amniotic fluid (\"meconium stained fluid\") Non-reassuring patterns seen on cardiotocography: increased or decreased fetal heart rate (tachycardia and bradycardia), especially during and after a contraction decreased variability in the fetal heart rate late decelerations Biochemical signs, assessed by collecting a small sample of baby's blood from a scalp prick through the open cervix in labor fetal metabolic acidosis elevated fetal blood lactate levels (from fetal scalp blood testing) indicating the baby has a lactic acidosis Some of these signs are more reliable predictors of fetal compromise than others. For example, cardiotocography can give high false positive rates, even when interpreted by highly experienced medical personnel. Metabolic acidosis is a more reliable predictor, but is not always available."}, {"id": "pubmed23n0480_1896", "title": "[Usefulness of the examination of fetal blood oxygen saturation (FSpO2) and fetal heart rate (FHR) as a prognostic factor of the newborn outcome].", "score": 0.011745770837613863, "content": "Cardiotocography has become the standard for fetal monitoring in labor. False-positive findings during electronic fetal heart rate monitoring may were not associated with neonatal acidemia. Because of the poor specificity of fetal heart rate monitoring in predicting fetal distress, new methods are being investigated as a way to improve the accuracy of assessing the infant's condition during labor. The aim of this study was to determinate the efficiency of fetal blood oxygen saturation (FSpO2) and computer analysis of the fetal heart rate (Co-CTG) in the late 1-st stage of labor as a prognostic factor of newborn acidemia. Total 62 subjects were studied. During labors and deliveries fetal oxygen saturation was continuously recorded, with use of Nellecor N-400 fetal pulse oximeter and continous CTG were performed by Hewlett Packard 50A. Transdermal fetal oxygen saturation measurements and CTG results obtained during the labors was analyzed using MONAKO system (ITAM Zabrze). The results were compared with the values of pH and base deficit in the umbilical artery measured just after delivery. The sensitivity, specificity, negative, positive predictive values and Youden factor based on FHR and FSpO2, for prognosis of neonatal acidosis were: 65%, 80%, 16%, 97.5% 60% and 0.135 respectively FHR; and 100%, 60%, 100%, 96.8% and 0.968 respectively FSpO2. 1. The examination of fetal blood oxygen saturation in the labor is a useful prognostic factor of the newborn outcome. 2. The best predictive value for intrapartum fetal asphyxia with metabolic acidosis was found when fetal pulse oximetry is added to cardiotocography."}, {"id": "wiki20220301en022_65581", "title": "Cardiotocography", "score": 0.011134150739271547, "content": "In a study by Tarvonen et al. (2019), it was demonstrated that the occurrence of saltatory pattern (already with the minimum duration of 2 minutes) in CTG tracings during labor was associated with fetal hypoxia indicated by high umbilical vein (UV) blood erythropoietin (EPO) levels and umbilical artery (UA) blood acidosis at birth in human fetuses. As saltatory patterns preceded late decelerations of fetal heart rate (FHR) in the majority of cases, saltatory pattern seems to be an early sign of fetal hypoxia. According to the authors, awareness on this gives obstetricians and midwives time to intensify electronic fetal monitoring and to plan possible interventions before fetal asphyxia occurs."}, {"id": "Obstentrics_Williams_2269", "title": "Obstentrics_Williams", "score": 0.010857528046607924, "content": "If repeat test >6, observe and repeat per protocol Repeat testing same day; if biophysical profile score :;6, deliver Deliver AFV = amnionic fluid volume; NST = nonstress test. Reproduced with permission from Manning FA, Morrison I, Harman CR, et al: Fetal assessment based on fetal biophysical profile scoring: experience in 19,221 referred high-risk pregnancies. II. An analysis of false-negative fetal deaths, Am J Obstet Gynecol. 1987 Oct;157(4 Pt 1 ):880-884. I. ):J0 downstream impedance (Chap. 10, p. 213). For growth 7.30 7.25 0E:J E:J= 7.20 7.10 7.05 FIGURE 17-9 Mean umbilical vein pH (±2 SD) obtained by cordocentesis in relation to fetal biophysical profile score category. (Data from Manning, 1993.) API determination for which �5 cm was considered abnormal (Chap. 11, p. 230). his abbreviated biophysical proile required approximately 10 minutes to perform, and they concluded that it was a superb antepartum surveillance method because there were no unexpected fetal deaths."}, {"id": "article-18300_4", "title": "Ultrasound Biophysical Profile -- Potential Diagnosis", "score": 0.010482795412109896, "content": "Diagnosis and management must take into consideration maternal and fetal risk factors. A biophysical profile can only diagnose oligohydramnios or polyhydramnios. Otherwise, it is an indicator of fetal wellbeing reflecting the blood pH. A score of 8 to 10 is normal and provides reassurance that the risk of fetal asphyxia within 1 week is extremely low. Rates of this study being a false negative range from fetal death rates of 0.55 per 1000 to 0.83 per 1000. [2] A score of 6 is considered either equivocal or abnormal. If oligohydramnios is diagnosed, the study should be repeated in 24 hours or the patient should possibly be induced if at term. If the score is 6 and all the points off relate to fetal movement, this is more reassuring, but the study should be repeated in a short interval. A score of 2 to 4 is not reassuring, and the recommendation is to either move toward labor induction or cesarean section depending on what is most prudent. A score of 0 indicates impending fetal asphyxia and delivery should be by urgent cesarean section at a hospital with NICU capability."}, {"id": "pubmed23n0507_23621", "title": "[Evolution of indications for cesarean section between 1991 and 2000 in materials from the Pathology Clinic in the Department of Pregnancy and Labor, Pomeranian Medical University in Szczecin].", "score": 0.009900990099009901, "content": "The present study was designed to analyze retrospectively the course of 2693 pregnancies and deliveries between 1999 and 2000 at the Department of Pathology of Pregnancy and Labor, Pomeranian Academy of Medicine in Szczecin. Attention was focused on the frequency of cesarean sections including preventive and urgent cases, obstetric and paraobstetric pathologies complicating pregnancy, perinatal and maternal mortality. The efficacy of diagnosing fetal distress in pregnancy and labor by continuous fetal monitoring with cardiotocography versus ultrasonography, in particular umbilical artery Doppler velocimetry, was studied. The material consisted of 2693 pregnant women (1476 (54.8%) primiparous and 1217 (45.2%) multiparous) aged 15-46 years, and their 2802 newborns. Gestational age varied between 23 and 43 weeks. Two groups were formed: I--1268 women delivering by cesarean section between 1991 and 1995; II--1425 women delivering by cesarean section between 1996 and 2000. In 1995/1996, complete ultrasonography supplemented with umbilical artery Doppler flow and intrauterine invasive intervention (cordocentesis, amnioinfusion, amnioreduction, intrauterine fetal blood transfusion) were introduced, as well as screening for diabetes in pregnancy and immunological tests for anticardiolipin antibodies. At that time, the Program of Improved Prenatal Care in Poland was fully implemented. Moreover, the Intensive Therapy of Newborns Uni was established at the Department of Pathology of Pregnancy and Labor. Depending on the moment the decision to perform cesarean section was taken, the prospective group was divided into two groups: antenatal (group P--1036 gravida) and intranatal (group S--1657 parturients). Depending on indications for cesarean section, urgent (N) were distinguished from preventive (Pr) cases. CTG interpretation was performe using qualitative and semi-quantitative Fischer's method. CTG recordings during labor were interpreted according to the qualitative method proposed by FIGO in 1987. An analysis of PI, S/D, RI, and flow curve (absence (AEDF) or reversion (REDF) of end-diastolic umbilical artery blood flow) in umbilical artery Doppler velocimetr was performed. A decrease of more than two standard deviations from average valu for several pregnancy time intervals was regarded as pathological. The absence (AEDF) or reversion (REDF) of end-diastolic umbilical artery blood flow was considered to be a high risk factor. The status of newborns was assessed using 5 min Apgar score and parameters o acid-base equilibrium in umbilical artery blood. The following conclusions were drawn: 1. Analysis of cases during the last decade of the 20th century revealed: --increased rate of cesarean sections with a shift in indications from urgent to preventive; --increase in obstetric risk associated with higher percentage of complication during pregnancy; --higher percentage of cesarean sections in premature labor; --correlation between premature labor and increased risk of poor newborn statu --decrease in perinatal mortality of newborns. 2. Analysis of methods for antepartum fetal monitoring revealed: --abnormal CTG patterns and abnormal blood flow in umbilical artery with ab sence or reversion of end-diastolic blood flow the coexistence of which were diagnostically effective for prediction of poor newborn status. 3. Analysis of fetal monitoring methods revealed: --normal intrapartum CTG patterns and normal antepartum blood flow in umbilical artery were markers of good newborn status; --abnormal CTG patterns and abnormal blood flow in umbilical artery had lim ited predictive value regarding poor newborn status. 4. Evolution of indications for cesarean section was multifactorial in character. New and more effective methods for fetal monitoring are needed."}, {"id": "pubmed23n0721_24144", "title": "[\"Quantitative cardiotocography\"--clinical practice guideline].", "score": 0.009900990099009901, "content": "The \"quantitative cardiotocography\" method provides important information about the condition of the fetus during labor, non-invasively and in real time. Since the forecast pH-results are being updated every 5 (five) minutes, significant differences in the readings for pH can be observed. In certain cases this can hamper the evaluation of fetal condition and lead to contradictions. Such inconveniences can be easily avoided by using the arithmetic average value of the last 6 forecast results for pH, generated by the quantitative cardiotocography software. Another inconvenience of the \"quantitative cardiotocography\" method lies in the fact that the components involved in the formation of CTG-score seem to be unequal in terms of potential for evaluating the fetal condition. Based on that, we believe that clinical practice guideline is needed. We developed such guideline based on two main criteria: the arithmetic average value over the last 6 (six) pH estimates; which components of the CTG-score are involved in the formation of the pH forecast readings. Our previous studies demonstrated that in 85% of all cases, the actual pH value of the newborn was in the range of -0037/+0046 from the average arithmetic value of the last six (6) pH estimates before the delivery. Based on this variability in the pH forecast results we defined three groups of findings--normal (pH 7.350 to 7.237), suspect (pH of 7.237 to 7.137) and abnormal (pH &lt; 7.137). In another study, we differentiated several varieties of CTG-score (with very high, high, satisfactory and low predictive value), depending on the observed deviations between forecast and actual pH results. pH forecast results should always be assessed together with the composition of the CTG-score. This can be achieved by using the presented clinical guideline, which also contains recommendations for adopting specific obstetric behavior, based on the \"quantitative cardiotocography\" readings. In future studies we will investigate if it is possible the presented clinical practice guideline to replace fetal blood sampling for intrapartal assessment of the fetal condition. Further clinical studies will clarify whether the use of this clinical guideline could lead to a reduction in the incidence of metabolic acidosis in newborns or change the percentage of operative deliveries compared with the cases where classical indirect cardiotocography was used."}, {"id": "pubmed23n0408_4808", "title": "The effect of labor on maternal and fetal vitamins C and E.", "score": 0.00980392156862745, "content": "This study was conducted to compare maternal and fetal plasma, amniotic fluid, and chorioamnion levels of vitamins C and E in term (&gt;38 weeks' gestation) subjects undergoing elective repeat cesarean section (CS) without labor with values of subjects of similar gestational age and dietary intake undergoing labor and vaginal delivery (VD). Healthy women undergoing elective repeat CS (n = 5) or uncomplicated VD (n = 5) at term (&gt;38 weeks' gestation) were studied. For CS patients, maternal and fetal (cord) blood, amniotic fluid, and chorioamnion samples were collected at time of surgery. For VD patients, maternal blood and amniotic fluid were obtained at 5 cm cervical dilation and fetal cord blood and chorioamnion were collected at delivery. Each patient completed a nutritional questionnaire. Plasma and membrane vitamin E concentrations were determined by reversed-phase high-performance liquid chromatography and standardized to cholesterol or membrane protein, respectively. Vitamin C was determined with the use of the 2,4-DNPH method. Dietary intakes for vitamins C and E as well as maternal and fetal vitamin E plasma concentrations were similar for CS and VD patients. In both groups, maternal levels were higher than fetal levels(P &lt;.05). Chorioamnion membrane vitamin E measurements in both groups were similar. Vitamin C concentrations in CS and VD patients were highest in amniotic fluid, lower in fetal plasma, and lowest in maternal plasma. However, mean vitamin C concentrations in maternal plasma, amniotic fluid, and fetal plasma of VD patients were significantly lower, being only 20% +/- 6%, 29% +/- 11%, and 22% +/- 2% of values obtained from CS patients. During labor in healthy women at term, uterine contractile activity may generate reactive oxygen species (ROS) through the process of repetitive ischemia and reperfusion. With the significant depletion of vitamin C during labor, we speculate that water-soluble vitamin C scavenges ROS in the aqueous phase and recycles lipid-soluble vitamin E to combat ROS-induced tissue damage."}, {"id": "pubmed23n0668_25611", "title": "[A comparative study of the prognostic pH values of the fetus in utero, generated by the \"quantitative cardiotocography\" computer method, and the actual pH values measured immediately after delivery].", "score": 0.00980392156862745, "content": "The presented methodology for quantitative evaluation of the cardiotocographic (CTG) findings unconditionally provides essential opportunities for improving the diagnostic potential in modern obstetric practice. Current literature data concerning the clinical application of this method are scarce. Credible clinical trials are needed to determine what is the correlation between estimated and actual values of the studied variables. A prospective study on 110 pregnant women was performed. All patients were monitored via indirect cardiotocography. The recordings were stored and analyzed by the computerized method for \"quantitative cardiotocography\". We compared the last prognostic fetal pH value, generated by the \"quantitative cardiotocography\" software during labor, with the actual pH measured from the umbilical artery (UA) of the newborn. For each of the stored CTG recordings we quantified the difference between the last forecast and the actual pH of the newborn. In 82% of these cases this difference was in range of -0081/+0074 from the projected results. However during the study we discovered that there is a significantly better correlation between the arithmetic average of the last 6 (six) predicted results and the actual pH of the newborn. In 85% of these cases the difference between forecast and actual pH values lies a in range of -0037/+0046. Using the arithmetic average of the last 6 (six) predicted results for pH leads to a significant increase in the clinical value of the \"quantitative cardiotocography\". More studied are needed if we are to find opportunities to further reduce the existing prediction variability."}, {"id": "Obstentrics_Williams_2268", "title": "Obstentrics_Williams", "score": 0.009788332472896231, "content": "Because the biophysical proile is labor intensive and requires a person trained in sonography, Clark and coworkers (1989) used an abbreviated biophysical proile as a irst-line screening test in 2628 singleton pregnancies. Speciically, a vibroacoustic nons tress test was performed twice weekly and combined with TABLE 17-3. Interpretation of Biophysical Profile Score 10 Normal, nonasphyxiated fetus 8/10 (Normal AFV) Normal, nonasphyxiated fetus 8/8 (NST not done) 8/10 (Decreased AFV) Chronic fetal asphyxia suspected 6 Possible fetal asphyxia o to 2 Almost certain fetal asphyxia If amnionic fluid volume abnormal, deliver If normal fluid at >36 weeks with favorable cervix, deliver If repeat test :;6, deliver If repeat test >6, observe and repeat per protocol Repeat testing same day; if biophysical profile score :;6, deliver Deliver AFV = amnionic fluid volume; NST = nonstress test."}, {"id": "wiki20220301en089_24946", "title": "Postterm pregnancy", "score": 0.009741831383622428, "content": "Baby Reduced placental perfusion – Once a pregnancy has surpassed the 40-week gestation period, doctors closely monitor the mother for signs of placental deterioration. Toward the end of pregnancy, calcium is deposited on the walls of blood vessels, and proteins are deposited on the surface of the placenta, which changes the placenta. This limits the blood flow through the placenta and ultimately leads to placental insufficiency, and the baby is no longer properly nourished. Induced labor is strongly encouraged if this happens. Oligohydramnios – Low volume of amniotic fluid surrounding the fetus. It is associated with complications such as cord compression, abnormal heart rate, fetal acidosis, and meconium amniotic fluid. Meconium aspiration syndrome – Respiratory compromise secondary to meconium present in infant's lungs. Macrosomia – Estimated fetal weight of ≥ 4.5 kg. It can further increase the risk of prolonged labor and shoulder dystocia."}, {"id": "pubmed23n0001_2782", "title": "Maternal and fetal plasma levels of free corticosteroids in pathological deliveries.", "score": 0.009708737864077669, "content": "Does intrauterine acidosis induce increased steroid secretion? The concentration of free steroids (CS) increases in both fetal and maternal plasma during labor and delivery. Fetal levels are higher after vaginal than after cesarean section. These differences may indicate an important role of the fetal adrenal gland in the induction of labor or they may reflect merely the fetal response to the stress of delivery. During incrased intrauterine stress steroid secretion is increased as shown here. We examined 41 mothers and their infants during pathological labor. Pathology was assessed from fetal acidosis and/or a clinically obstetric disease of the mother or fetus. The 41 cases included 9 cesarean sections, 8 forceps deliveries; 24 spontaneous deliveries of which 7 were premature. At the time of delivery the pH and CS level were determined in maternal and umbilical vessels in all cases. During spontaneous labor blood samples were also taken during the different stages of labour. A competetive protein binding assay with transcortin without fractionation of the steroids was used. Progesteron was determined by the same assay. The level of this hormone, however, remains unchanged and hence any changes reflect changes in CS. The levels of CS were correlated with the pH values and compared to previously obtained normal values. During pathological deliveries CS levels in both mother and fetus are normal as long as there is no acidosis (Fig. 1). If acidosis is present the CS level in the umbilical artery is usually higher than normal. In 13 out of 18 vaginal deliveries the CS level was above normal, in the other 5 at the upper limit of normal (Fig. 1 and 2). At the same time the a--v difference becomes smaller and sometimes even negative. No changes were noted in maternal and umbilical venous blood (Tab. I and II). Similar dependence on the pH was found for cesarean sections (Tab. III). In premature deliveries without acidosis in the umbilical artery the CS levels were lower in both mother and fetus (Tab. I). These results indicate that the fetal adrenal gland reacts to acidosis, i.e., intrauterine stress, with increased corticosteroid secretion. This rise depends on the pH of fetal blood and not on the type of delivery (Fig. 3)."}, {"id": "pubmed23n0509_16952", "title": "Severe variable decelerations and fetal pulse oximetry during the second stage of labor.", "score": 0.009708737864077669, "content": "The aim of the study was to investigate the usefulness of fetal pulse oximetry in cases of severe variable decelerations in the second stage of labor. It is a prospective study including 58 patients. Thirty-eight patients (group A) had a normal uncomplicated labor and 20 patients (group B) developed severe variable decelerations during the second stage of labor. All patients were primiparous with normal pregnancies and had electronic fetal monitoring of labor in conjunction with fetal pulse oximetry. An estimation of fetal pH and base deficit was performed at delivery in all patients. There was no statistically significant difference in relation to maternal age and gestational age between the two groups. Group A patients did not delivered neonates with metabolic acidosis. Six out of 20 (group B) patients delivered neonates with a pH &lt;7.10 despite a fetal pulse oximetry reading of &gt;30%. It appears that fetal pulse oximetry is not capable of detecting pre-acidotic or acidotic fetuses during the second stage of labor in patients with severe variable decelerations and the management of such patients should be supported by fetal scalp pH when indicated or otherwise the obstetrician should expedite delivery either with assisted operative delivery or cesarean section. Fetal heart rate monitoring was introduced into clinical practice over 30 years ago. It continues to be the predominant method of intrapartum fetal surveillance despite worries about its accuracy and efficacy."}, {"id": "pubmed23n0415_3383", "title": "Intrapartum surveillance of IUGR fetuses with cardiotocography and fetal pulse oximetry.", "score": 0.009615384615384616, "content": "To investigate the efficacy and safety of intrapartum fetal pulse oximetry, as a predictor of metabolic acidosis at birth of fetuses with intrauterine growth retardation (IUGR). We studied 18 IUGR fetuses (group I) and a control group of 30 appropriate for gestational age (AGA) fetuses (group II) during labor. Both groups had abnormal fetal heart rate tracings and were monitored simultaneously throughout labor with cardiotocography and fetal pulse oximetry. Apgar scores, pH and base excess of fetal blood obtained from the umbilical artery after delivery were compared in both groups. The Fetal Surveillance Unit of the 2nd Department of Obstetrics and Gynecology, Aretaieion Hospital, Medical School, Athens University. In IUGR fetuses, when their oxygen saturation value (FSPO2) was less than 34%, cord artery pH was 7.10 +/- 0.04, base excess -13 +/- -1 mmol/l and Apgar scores &lt; or =5 at the 5th min, and when FSPO2 was over 35%, artery pH was 7.29 +/- 0.08, base excess -8 +/- -2 mmol/l and Apgar scores &gt; or =7 at the 5th minute. In cases of drops of FSPO2)below 30% for more than 2 min, labor was completed operatively and cord pH was 7.00 +/- 0.04, base excess -15 +/- -2 mmol/l and Apgar scores &lt; or =5 at the 5th minute. In AGA fetuses, when FSPO2 was over 30%, artery pH was over 7.20, base excess &lt;-11 mmol/l and Apgar scores &gt; or =9 at the 5th minute; in contrast, when FSPO2 was &lt;30% for 2 min, a cesarean section was performed and cord pH was &lt; or =7.02, base excess &gt; or =-13 mmol/l and Apgar scores &lt; or =4 at the 5th minute. In IUGR fetuses, FSPO2 values less than 34% represent an acidotic status, while values of &gt; or =35% are well tolerated. Fetal pulse oximetry proved reliable, according to umbilical cord blood measurements and Apgar scores, reducing cesarean deliveries in cases of nonreassuring cardiotocographic patterns in IUGR fetuses."}, {"id": "pubmed23n0222_2390", "title": "[Determination of the mean pH of fetal blood during the pushing phase of labor].", "score": 0.009615384615384616, "content": "The pH of the fetus and mother in the phase of pressing in 100 normal deliveries, which served as the control group, was analysed. An average decrease of the pH by 0.07 in both the fetus and the mother was recorded, starting from the full opening of the cervix until the termination of labour. This result is evaluated by the determination of the pH in 121 cases of disturbed pregnancies, singling out 9.9% of fetus in preacidosis and 11.6% of fetus in acidosis, which is significantly higher than in the control group. It is pointed out that the detection of acute fetal suffering in labour should not be limited to the time of opening only but that it should be carried out also in the phase of pressing."}, {"id": "pubmed23n0946_8578", "title": "Management of Labor and Delivery After Fetoscopic Repair of an Open Neural Tube Defect.", "score": 0.009523809523809525, "content": "To report labor, delivery, and neonatal outcomes in a cohort of women delivering neonates who had undergone fetoscopic neural tube defect repair. We conducted a retrospective cohort study from April 2014 to January 2018. All patients met Management of Myelomeningocele Study eligibility criteria. We included patients with completed second-trimester fetoscopic neural tube defect repair (laparotomy, uterine exteriorization, and minimally invasive access through two or three uterine ports) followed by standardized management of labor and delivery at our institution. Outcomes included rates of vaginal delivery, term delivery, and intrapartum cesarean delivery as well as obstetric and neonatal outcomes after oxytocin. Complications of interest included preterm prelabor rupture of membranes, chorioamnionitis, uterine dehiscence or rupture, 5-minute Apgar score less than 7, and neonatal acidosis (umbilical artery pH less than 7.15). Thirty-four patients had fetoscopic repair, followed by 17 vaginal deliveries (50%, 95% CI 32-68%). Median gestational age was 38 1/7 weeks at vaginal delivery (range 26 0/7-40 2/7 weeks of gestation) and 37 1/7 weeks of gestation at cesarean delivery (range 25 5/7-40 5/7 weeks of gestation); 62% of deliveries occurred at term. Eight patients had prelabor cesarean delivery: three nonurgent and five urgent (for nonreassuring fetal heart tracings). Twenty-six patients labored; six were induced and 20 labored spontaneously. Of the latter, five were augmented. Of 26 laboring patients, 17 delivered vaginally and nine underwent urgent cesarean delivery (35%, 95% CI 17-56%; seven nonreassuring fetal heart tracings and two breech). There were no cases of uterine rupture or dehiscence. Most (94%, 95% CI 80-99%) had normal 5-minute Apgar scores; one neonate (3%, 95% CI 0-15%) had acidosis but normal Apgar scores. Our data regarding trial of labor, use of low-dose oxytocin, and vaginal delivery after prenatal fetoscopic neural tube defect repair are reassuring. Importantly, fetoscopic repair may permit delivery at advanced gestational ages."}, {"id": "pubmed23n0255_16893", "title": "Fetal blood sampling in labor.", "score": 0.009523809523809525, "content": "Cardiotocography is the most widely used method to monitor fetal well-being during labor. It has been shown that this technology is affected by a large intra and interobserver variability, that reduces its reliability. Moreover, it should be remembered that CTG is in condition to detect early fetal heart rate patterns that may indicate fetal hypoxaemia but it is not able to assess precisely the level of fetal compromise. This assessment can be done by examining fetal blood for pH that can be easily obtained in labor. If present, the level of acidemia, is established by measuring the pH which can be of practical use in clinical management. Values above 7.24 are considered normal, and pH values lower than 7.20 are expression of fetal acidemia requiring a quick delivery. The technique of FBS can be easily performed with very few complications. The main indication is one of the abnormal or suspicious CTG patterns. By using FBS is a complementary diagnostic tool to CTG it is possible to reduce the number of false positive thus reducing the number of unnecessary obstetrical interventions."}, {"id": "pubmed23n1119_15204", "title": "Manual fetal stimulation during intrapartum fetal surveillance: a randomized controlled trial.", "score": 0.009433962264150943, "content": "Manual fetal stimulation, either by mechanical manipulation or by stimulation of the fetal scalp, is known to evoke a fetal heart response in a normal fetus. This study aimed to assess the clinical effectiveness of manual fetal stimulation in the assessment of fetal well-being during labor vs no stimulation among women with a singleton pregnancy and to investigate the maternal and neonatal outcomes in the 2 groups. This was a randomized controlled trial conducted in the department of obstetrics and gynecology at a tertiary care teaching hospital between 2014 and 2016. The inclusion criteria included women with a singleton pregnancy at ≥37 weeks of gestation with cephalic presentation in labor having one of the following abnormalities on fetal heart tracing: fetal heart rate of &lt;110 bpm or &gt;160 bpm, variable decelerations, late decelerations, and minimal or absent beat-to-beat variability. The exclusion criteria included women requiring immediate cesarean delivery, conditions that would preclude a vaginal delivery, and intrauterine fetal demise or a major fetal congenital abnormality. The women were followed up in labor and randomized to either the manual stimulation group or the no stimulation group when one of the cardiotocography abnormalities were present. In the manual stimulation group, the fetus was stimulated abdominally by holding the head in the palm of 1 hand when the cervical dilatation was &lt;3 cm or vaginally by pinching the scalp of the fetus when the cervical dilatation was ≥3 cm. After delivery, a cord blood sample was collected and pH estimated. Mother and baby were followed up until discharge, and mode of delivery, cord blood pH at birth, Apgar scores at 1 minute and 5 minutes, neonatal intensive care unit admissions, and duration of stay were the outcomes studied. Data were entered and compiled as frequency and percentage for categorical variables. For continuous variables, data were calculated using mean and standard deviation. The chi-square test was used for assessing the association between the intervention and fetal and maternal outcomes. A total of 327 women were included in the trial, of whom 164 were in the manual fetal stimulation group (group 1) and 163 were in the \"no stimulation\" group (group 2). The cesarean delivery rates were 25.61% in group 1 and 30.67% in group 2 (P=.308). The mean cord blood pH levels at birth were 7.267±0.027 in group 1 and 7.265±0.024 in group 2 (P=.479), and the Apgar scores at 1 minute and 5 minutes (P=.169 and P=.423, respectively, between the 2 groups) were not found to be statistically different among the 2 groups. There was no considerable change in fetomaternal outcomes with manual fetal stimulation in women having nonreassuring cardiotocographic changes in labor."}, {"id": "pubmed23n0533_15191", "title": "Fetal monitoring in labor: are accelerations good enough?", "score": 0.009433962264150943, "content": "To investigate whether accelerations of the fetal heart rate in response to scalp stimulation (the scalp stimulation test) before fetal scalp blood sampling (FBS) are such a good predictor of fetal well-being as to render the FBS unnecessary. A retrospective observational study. Cardiotocograms (CTG) from 54 fetuses during labor in whom 70 FBS procedures were performed, were analysed by an investigator blinded to the outcome, to determine whether accelerations were present in response to fetal scalp stimulation during vaginal examination (VE) prior to the FBS. This was compared with the pH obtained at FBS in a 2 x 2 table. The primary outcome measure was the false negative rate of the scalp stimulation test. There were accelerations at 48 VEs before FBS (n = 70). In five cases there was fetal acidosis (pH&lt;or=7.20). Three of these five fetuses had accelerations at VE, giving a false negative rate of 6.25%. These data do not support previous reports that accelerations in response to fetal scalp stimulation before FBS exclude fetal acidosis when the CTG is suspicious and FBS is otherwise indicated. A larger sample may help answer this question."}, {"id": "article-131252_28", "title": "Root Cause Analysis and Medical Error Prevention -- Function -- Case Example 3", "score": 0.009413067552602437, "content": "A 26-year-old primigravida woman with labor pains was admitted to a busy hospital's labor and delivery suite at 39 weeks of gestation. There were no associated high-risk factors. The patient was admitted to the labor ward and managed according to routine protocol. She progressed in spontaneous labor, but the cardiotocograph showed prolonged fetal bradycardia lasting for 3 and a half minutes at 4 centimeters (cm) cervical dilatation. The fetal bradycardia did not resolve with initial conservative measures."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 96]], "word_ranges": [[0, 12]], "text": "Idiopathic thrombocytopenic purpura, but with very low platelets, so replenishment is necessary."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": true, "char_ranges": [[97, 134]], "word_ranges": [[12, 19]], "text": "The proposed steroid dose is too low."}}, "full_answer": "Idiopathic thrombocytopenic purpura, but with very low platelets, so replenishment is necessary. The proposed steroid dose is too low.", "full_answer_no_ref": "Idiopathic thrombocytopenic purpura, but with very low platelets, so replenishment is necessary. The proposed steroid dose is too low.", "full_question": "A 19-year-old girl with no medical history of interest, except for a self-limited case of influenza 3 weeks earlier, who comes to the emergency department with petechiae and ecchymosis of spontaneous onset. On physical examination the patient was in good general condition, afebrile, normotensive and oriented in time and space. There were petechiae scattered throughout the lower extremities and abdomen and small ecchymoses in decubitus areas. No lymphadenopathy or splenomegaly were palpated. Laboratory tests showed the following findings: Hb 12.6 g/dL, leukocytes 5,500/mm3, platelets 7000/mm3. The peripheral blood smear showed normal erythrocyte morphology, normal differential leukocyte count and the planetary count was concordant with the autoanalyzer figure without platelet aggregates. Biochemistry and proteinogram, beta 2 microglobulin and LDH are normal. Which of the following do you think is the most appropriate initial treatment?", "id": 232, "lang": "en", "options": {"1": "Platelet transfusion.", "2": "Weekly Rituximab.", "3": "Cyclophosphamide in 4-day pulses every 21 days.", "4": "Daily plasmapheresis.", "5": "Prednisone at 1 mg/day for 2-3 weeks."}, "question_id_specific": 103, "type": "HEMATOLOGY", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0059_11730", "title": "[Thrombotic thrombocytopenic pupura (TTP)--remission following treatment with high-dose immunoglobulin].", "score": 0.016986496090973704, "content": "A 60-year-old man was admitted to our hospital because of fever, hemorrhagic tendency, anemia and neurological abnormality. A blood count revealed that the hemoglobin was 6.8 g/dl, the reticulocyte was 17.3 percent with 2 erythroblasts per 100 white cells, the white cell count was 7,100/microliters and the platelet count was 0.8 x 10(4)/microliters. Peripheral blood smear demonstrated marked fragmentation of red cells. Bone marrow examination disclosed the marked erythroid hyperplasia. Although the bleeding time was prolonged (14 minutes 30 seconds), the other hemostatic data were within normal limits. The serum bilirubin level was 1.57 mg/dl; LDH level, 1,437 U/l; creatinine level, 0.92 mg/dl; BUN level 14.7 mg/dl. Haptoglobin was below 10 mg/dl. Results of immunological tests were all negative except the result of PAIgG (576.6 ng/10(7) cells). The urinalysis showed proteinuria, microhematuria and trace granular and hyaline casts. A diagnosis of thrombotic thrombocytopenic purpura was made. The patient was initially treated with prednisolone (60 mg), aspirin (1,000 mg), dipyridamole (150 mg), gabexate mesilate (1.5 g), sodium oxagrel (80 mg) daily with little response. The thirty days after admission, infusion of gamma globulin (20 g, daily) was given for 3 days. The clinical state and laboratory findings became dramatically improved shortly after the administration of gamma globulin and the laboratory data came to be normalized after 1 month. After ten months of this treatment, the patient is remained asymptomatic and the hematological data are within normal range without using any drug. A trial seems justified to confirm the value of this mode of therapy."}, {"id": "pubmed23n0771_21251", "title": "[Chronic lymphocytic leukemia].", "score": 0.013406593406593406, "content": "Chronic lymphocytic leukemia (CLL) is a lymphoproliferative disorder that accounts for approximately 30 % of adult leukemias and 25 % of Non-Hodgkin lymphomas (NHL). It is the most common form of leukemia in the western world (incidence 3-5/100 000). Elderly people are mainly affected, median age at diagnosis is around 70 years and there is a slight predominance in men. The etiology of the disease is unknown. The initial symptoms are nonspecific. Cervical lymphadenopathy and splenomegaly followed by general fatigue are seen most commonly. Other possible symptoms include night sweats, fever, loss of weight (so-called B symptoms) and frequent infections. Several patients develop autoimmune complications as autoimmune hemolytic anemia (AIHA) or immune thrombocytopenia (ITP). To confirm the diagnosis more than 5000 B-lymphocytes/µl need to be present. The expression of the typical surface markers CD5, CD19, and CD23 has to be confirmed by flow cytometry. Imaging studies as X-ray of the chest, ultrasound of the abdomen, or CT scan are used to assess the degree of lymphadenopathy or organomegaly. A bone marrow biopsy is not mandatory for the diagnosis. According to the European Binet staging system, CLL is divided into 3 stages (A, B and C). Patients in Binet stage A have 0 to 2 areas of node or organ enlargement with normal levels of hemoglobin and platelets. Binet stage B patients have 3 to 5 areas of node or organ enlargement and normal or slightly decreased levels of hemoglobin and platelets. Binet stage C patients have anemia (hemoglobin &lt; 10 g/dl) and/or thrombocytopenia (platelet counts &lt; 100 000/µl), with or without lymphadenopathy or organomegaly. As there is no survival benefit associated with early intervention, asymptomatic patients with early stage CLL (Binet stage A and B) are usually not treated but are followed on a \"watch and wait\" principle. Treatment indications include stage Binet C or signs of an active disease as rapidly progressive lymphadenopathy or organomegaly together with physical limitation, B symptoms that cannot be tolerated, rapidly deteriorating blood values, or rapidly increasing leukocyte counts. The patient's physical condition has major impact on the treatment decision. Currently immunochemotherapy with fludarabine, cyclophosphamide and the CD20-antibody rituximab (FCR) is the standard of care in previously untreated and physically fit CLL-patients. An alternative regimen is the combination of bendamustine and rituximab (BR). Physically compromised patients can be treated with the oral drug chlorambucil or with bendamustine with or without rituximab. Due to high morbidity and mortality, allogeneic stem cell transplantation is limited to a small group of patients and should be discussed in a high-risk situation, such as 17p deletion, lack of response to standard therapy or early relapse."}, {"id": "pubmed23n0497_4748", "title": "Cases from the Osler Medical Service at Johns Hopkins University.", "score": 0.013398877626484534, "content": "PRESENTING FEATURES: An 18-year-old white man was admitted to the Osler Medical Service with the chief complaint of back pain. Two weeks prior to admission, the patient developed diffuse and aching upper back pain. Over the next couple of days, he also developed severe anterior chest pain that was somewhat pleuritic in nature but diffuse and extending bilaterally into the shoulders. One week prior to admission, he developed intermittent fevers and night sweats. The patient denied any lymphadenopathy, pharyngitis, sick contacts, shortness of breath, rash, or bleeding. He was seen by a physician and told that he had thrombocytopenia. There was no history of recent or remote unusual bleeding episodes. His medical history was unremarkable except for a childhood diagnosis of attention deficit/hyperactivity disorder. He was not taking any medications and had no history of tobacco, alcohol, or illicit drug use. He had no risk factors for human immunodeficiency virus infection. Physical examination showed that he was afebrile and had normal vital signs. He was a well-appearing man who was lying still because of pain. HEENT examination was unremarkable. There was no pharyngeal erythema or exudates. His lungs were clear. His neck was supple and without lymphadenopathy. Examination of his back and chest revealed no focal tenderness. There was no hepatosplenomegaly, and his skin was without petechiae or rashes. Examination of the patient's joints showed pain on passive and active movement of his shoulders bilaterally, but no frank arthritis. There was no rash, petechiae, or echymoses. Chest radiograph and electrocardiogram were unremarkable. On admission, the laboratory examination was notable for a hematocrit level of 32.5%, with a mean corpuscular volume of 79 fL, and white blood cell count of 2.8 x 10(3)/microL. Platelet count was 75 x 10(3)/microL. A white blood cell differential revealed 7% bands, 53% polys, 34% lymphs, 5% atypical lymphocytes, 2% nucleated red cells, and a few young unidentified cells. His chemistry studies were unremarkable. What is the diagnosis?"}, {"id": "pubmed23n0818_22421", "title": "[A case of autoimmune lymphoproliferactive syndrome and literature review].", "score": 0.012991452991452993, "content": "To summarize the clinical characteristics, diagnosis and treatment of a case with autoimmune lymphoproliferative syndrome (ALPS) . The patient was diagnosed as autoimmune lymphoproliferactive syndrome (ALPS) after being admitted to the Department of Rheumatism and Immunology of Tianjin Children's Hospital in February 20, 2014. The clinical characteristics, physical examination, laboratory tests, gene tests, and treatment process were analyzed and related literature was reviewed. The patient was a 16-month- old boy.Since the first month of life, he started to have repeatedly fever, diarrhea, shortness of breath, lymphadenopathy, hepatosplenomegaly, anemia (HGBmin 50 g/L) and thrombocytopenia (min 35 × 10⁹/L) . But multiple exams showed a normal peripheral blood leukocyte count, hypergammaglobulinemia (IgG 19 800 mg/L, IgA 1 710 mg/L, IgM 2 590 mg/L) and significantly increased serum vitamin B12. Flow cytometric measures showed that CD3⁺ CD4⁻ CD8⁻ T lymphocytes significantly increased ( &gt; 10%) at four times. The count of CD3⁺ TCRαβ⁺ CD4⁻ CD8⁻T lymphocytes (double negative T cells; DNTs) &gt;3% twice. The genetic test showed that 309th FAS gene area showed heterozygous mutations, the boy was diagnosed as ALPS. Added examinations of lymphocytes apoptosis induced by FAS was positive. He was treated with prednisone 15 mg once daily and immunomodulator 150 mg three times a day, while in maintaining period with normal levels of hemoglobin and platelet, the dose of prednisone was reduced gradually. Till now, the patient has been treated and observed for 8 months. We retrieved the reports of ALPS in the databases at home and abroad published in recent 10 years, more than 400 cases reported from foreign countries, but there were only 5 domestic cases. Among those, 4 had onset in infancy and 1 at 6-years of age. All the cases presented servere lymphadenopathy and hepatosplenomegaly with anemia (4 of them with hemolytic anemia) and thrombocytopenia. Three cases had a history of frequent infection, one of them had glomerulonephritis. All patient with significant high level of serum immunoglobulin ( &gt; 1.5 times upper limit of normal range), in 3 of them serum vitamin B12 was &gt; 1.5 pg/L (the other 2 cases missed the exam). In 5 cases CD3⁺ CD4⁻ CD8⁻T cells &gt; 10%, and in 2 case DNTs were 8.9% and 15.7% respectively (the other 3 cases missed the exam). Three cases were clearly detected with FAS mutations. All patients were treated with corticosteroid, 2 of them were added with mycophenolate mofetil. The therapy presented effective result in early 1-3 months, but no long-term follow-up reports were available. ALPS is a disorder of disrupted lymphocyte homeostasis caused by defective Fas-mediated apoptosis, and it is one of the primary immunodeficiency diseases. The onset of the disease occurs during infancy mainly. Clinical lymphoid hyperplasia and autoimmune phenomena are outstanding signs, which can be associated with frequent infections and allergies. The level of serum vitamin B12 &gt; 1.5 pg/L and the count of CD3⁺ CD4⁻ CD8⁻ T cell show important significance. Exact diagnosis should depend on detecting DNTs and FAS gene."}, {"id": "pubmed23n0753_19757", "title": "Idiopathic thrombocytopenic purpura associated with splenic tuberculosis: case report.", "score": 0.012717433320448397, "content": "Tuberculosis is still one of the most prevalent and fatal infectious diseases in spite of considerable improvements in medical science. Splenic tuberculosis is a rare form of extrapulmonary tuberculosis. There are limited numbers of cases in which immune thrombocytopenia is associated with splenic tuberculosis. We report a case of immune thrombocytopenic purpura due to splenic tuberculosis. Our case was a 58-year-old female with headache, gum bleeding, redness in legs, and ecchymoses on the arms for 10 days. On admission to hospital, laboratory tests were as follows: platelet count 6.000/mmc (150 000-450 000), haemoglobin: 12 g/dl, WBC: 8000/mm3, erythrocyte sedimentation rate: 58 mm/h and C-reactive protein was in normal ranges. After standard laboratory tests, the patient was diagnosed with idiopathic thrombocytopenic purpura. The patient presented abdominal lymphadenopathies and spleen in normal size in radiological examinations. Diagnostic laparotomy and splenectomy and lymph node excision was performed and splenic tuberculosis was detected in pathologic and microbiologic examination. The patient was successfully treated with apheresis platelets suspension, intravenous immunoglobulin and antituberculous therapy. In conclusion, splenic tuberculosis should be suspected in patients who have fever, abdominal lymphadenopathies and immune thrombocytopenic purpura. Histopathological examination is still an ideal method to confirm the diagnosis, suitably aided by microbiological examination."}, {"id": "pubmed23n1041_19599", "title": "Immune Thrombocytopenia Purpura Secondary to COVID-19.", "score": 0.012708787218591141, "content": "A 73-year-old female with past medical history of essential hypertension, hyperlipidemia, seasonal allergies, and chronic back pain presented to the hospital with complaints of headaches, fevers, fatigue, generalized body aches, shortness of breath, and diarrhea. Initial complete blood count was remarkable for leukopenia with an absolute lymph count of 0.60 K/µL and severe thrombocytopenia (platelet count &lt; 3 K/µL). She was tested for COVID-19 via nasopharyngeal swab polymerase chain reaction (PCR) testing and found positive. Additional labs showed an elevated D-dimer, C-reactive protein, fibrinogen, and lactate dehydrogenase. Vitamin B12 and folate levels were obtained and found to be normal. Peripheral smear showed no schistocytes or additional hematologic abnormalities apart from thrombocytopenia. The patient was transfused one unit of platelets with no improvement in platelet count. Fibrinogen count was obtained and found in normal range at 458 mg/dL. Prothrombin time (PT), activated partial thromboplastin time (aPTT), and international normalized ratio (INR) were all found to be normal. Immune thrombocytopenia purpura (ITP) was suspected and intravenous immunoglobulin (IVIG) was administered at a dose of 1 g/kg/day for two doses. By day 4, the patient had marked response to treatment with platelet recovery to 105 K/µL and subsequently discharged by day 5 with complete resolution of symptoms and platelet count of 146 K/µL. Twenty-eight days after discharge, she presented to hematology clinic with platelet count of 8 K/µL. Repeat nasopharyngeal swab PCR COVID testing was negative and she was treated with IVIG and pulse dexamethasone with prompt response, confirming suspicion of underlying, undiagnosed ITP prior to COVID infection."}, {"id": "InternalMed_Harrison_1704", "title": "InternalMed_Harrison", "score": 0.011236222534339514, "content": "Fever ˜38.3° C (101° F) and illness lasting ˜3 weeks and no known immunocompromised state History and physical examination Stop antibiotic treatment and glucocorticoids Obligatory investigations: ESR and CRP, hemoglobin, platelet count, leukocyte count and differential, electrolytes, creatinine, total protein, protein electrophoresis, alkaline phosphatase, AST, ALT, LDH, creatine kinase, antinuclear antibodies, rheumatoid factor, urinalysis, blood cultures (n=3), urine culture, chest x-ray, abdominal ultrasonography, and tuberculin skin test"}, {"id": "wiki20220301en069_23972", "title": "Waldenström macroglobulinemia", "score": 0.010921912624699003, "content": "Chemistry tests include lactate dehydrogenase (LDH) levels, uric acid levels, erythrocyte sedimentation rate (ESR), kidney and liver function, total protein levels, and an albumin-to-globulin ratio. The ESR and uric acid level may be elevated. Creatinine is occasionally elevated and electrolytes are occasionally abnormal. A high blood calcium level is noted in approximately 4% of patients. The LDH level is frequently elevated, indicating the extent of Waldenström macroglobulinemia–related tissue involvement. Rheumatoid factor, cryoglobulins, direct antiglobulin test and cold agglutinin titre results can be positive. Beta-2 microglobulin and C-reactive protein test results are not specific for Waldenström macroglobulinemia. Beta-2 microglobulin is elevated in proportion to tumor mass. Coagulation abnormalities may be present. Prothrombin time, activated partial thromboplastin time, thrombin time, and fibrinogen tests should be performed. Platelet aggregation studies are optional. Serum"}, {"id": "InternalMed_Harrison_1673", "title": "InternalMed_Harrison", "score": 0.010710553814002089, "content": "Fever >38.3°C (101°F) on at least two occasions 2. Illness duration of ≥3 weeks 3. 4. Diagnosis that remains uncertain after a thorough history-taking, physical examination, and the following obligatory investigations: determination of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level; platelet count; leukocyte count and differential; measurement of levels of hemoglobin, electrolytes, creatinine, total protein, alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, creatine kinase, ferritin, antinuclear antibodies, and rheumatoid factor; protein electrophoresis; urinalysis; blood cultures (n = 3); urine culture; chest x-ray; abdominal ultrasonography; and tuberculin skin test (TST). The range of FUO etiologies has evolved over time as a result of changes in the spectrum of diseases causing FUO, the widespread Percentage of Cases Due to Indicated Cause"}, {"id": "pubmed23n1100_12252", "title": "Megadose Methylprednisolone for Immune Thrombocytopenia in an Infant Positive for SARS-CoV-2: A Case Report.", "score": 0.009900990099009901, "content": "BACKGROUND Immune thrombocytopenia (ITP) is rare in infants under 1 year old. Bleeding often occurs when the platelet count is &lt;20 000/uL. The disease can progress because of accompanying COVID-19 disease. CASE REPORT A 9-month-old boy, weighing 8.5 kg, came to the hospital with petechiae on the forehead, cheeks, mouth, and extremities. The patient had rhinorrhea for 3 days previously and was febrile, pale, weak, and could not drink. He had the measles-rubella vaccination 19 days prior. Physical examination showed no abnormalities of the eyes, ears, nose, throat, and mouth. Heart and lungs were within normal limits, with no organomegaly, lymphadenopathy, or congenital anomaly of the abdomen. Laboratory examination showed hemoglobin, 12.7 g/dL; leukocytes, 7420/uL; platelet count, 16 000/uL; and hematocrit, 37.9%. Erythrocyte sedimentation rate was 14 mm at 1 h and 21 mm at 2 h. Peripheral blood smear showed normal RBC morphology, normal leukocytes, and few platelets. IgG was reactive and IgM was nonreactive on rapid antibody test. RT-PCR was positive for SARS-COV-2. Chest-X-ray showed pneumonia. The diagnosis was newly diagnosed ITP with COVID-19. Patient was treated with 30 mg/kg body weight/day of IV methylprednisolone for 3 days (250 mg); then 20 mg/kg body weight/day (175 mg) orally for 4 days in 3 divided doses. Azithromycin 100 mg/day, zinc 20 mg/day, and vitamin C 50 mg/day orally were also given. CONCLUSIONS COVID-19 screening is highly recommended during this pandemic to identify it as a potential cause of childhood ITP. Megadose methylprednisolone had an excellent response in alleviating ITP with confirmed COVID-19 in an infant."}, {"id": "pubmed23n1009_24869", "title": "Teetering on a liver's edge: a case report highlighting clinical decision-making in thrombocytopenia.", "score": 0.009900990099009901, "content": "This report illustrates the importance of a detailed history and physical exam and careful analysis of hematologic parameters when diagnosing ITP. This case demonstrates that even with subtle deviations from typical ITP findings one must promptly reevaluate the diagnosis. This case also highlights the importance of peripheral smear review by an expert in pediatric hematopathology. A previously healthy 10 year-old Asian boy presented with 2 months of easy bruising. Review of systems was negative for any constitutional symptoms. On examination, he appeared well but had numerous large ecchymoses. He had no appreciable lymphadenopathy or splenomegaly. The liver was palpable 1.5 cm below the costal margin. A complete blood count (CBC) showed: platelets = 17 × 109/L, hemoglobin = 128 g/L, white blood cell count = 5.43 × 109/L, and neutrophils = 1.63 × 109/L. A blood smear was reported as normal. Urate was 370 umol/L and lactate dehydrogenase (LDH) was 803 U/L. The child was admitted with a presumptive diagnosis of immune thrombocytopenic purpura (ITP) and treated with intravenous immunoglobulin. The following day, the blood smear was reviewed by a hematopathologist who identified blasts. A bone marrow aspiration (BMA) confirmed the diagnosis of precursor B-cell acute lymphoblastic leukemia. In children presenting with suspected ITP, leukemia should always be considered. A BMA was historically performed on all patients with presumed ITP to rule out leukemia. In 2011, the American Society of Hematology (ASH) stopped recommending routine BMA in patients suspected of having ITP. ASH advises in cases with unusual findings on history, physical examination or CBC, it is reasonable to perform a BMA. Our patient had mild hepatomegaly, which may have qualified him for a BMA. He also had an elevated LDH and urate, which are not listed as criteria for BMA by ASH but were considered atypical for ITP by the clinical team. A literature search did not reveal any primary data assessing these markers. While corticosteroids are a first line treatment in ITP, they must be reserved for when clinicians are confident that the patient does not have leukemia. Steroid administration prior to diagnosing leukemia results in delayed diagnosis and may increase the risk of complications and decrease survival."}, {"id": "wiki20220301en020_74481", "title": "Thrombocytopenia", "score": 0.009871452825612418, "content": "Signs and symptoms Thrombocytopenia usually has no symptoms and is picked up on a routine complete blood count. Some individuals with thrombocytopenia may experience external bleeding such as nosebleeds, or bleeding gums. Some women may have heavier or longer periods or breakthrough bleeding. Bruising, particularly purpura in the forearms and petechiae in the feet, legs, and mucous membranes, may be caused by spontaneous bleeding under the skin. Eliciting a full medical history is vital to ensure the low platelet count is not secondary to another disorder. Ensuring that the other blood cell types, such as red blood cells and white blood cells are not also suppressed, is also important. Painless, round, and pinpoint (1 to 3 mm in diameter) petechiae usually appear and fade, and sometimes group to form ecchymoses. Larger than petechiae, ecchymoses are purple, blue, or yellow-green areas of skin that vary in size and shape. They can occur anywhere on the body."}, {"id": "pubmed23n0390_20630", "title": "Severe thrombocytopenia possibly associated with TMP/SMX therapy.", "score": 0.00980392156862745, "content": "To report a case of possible severe, life-threatening thrombocytopenia associated with trimethoprim/sulfamethoxazole (TMP/SMX) therapy. A 54-year-old white woman received a 10-day course of TMP/SMX for treatment of chronic sinusitis. One day after finishing the course of TMP/SMX therapy, the presented to the emergency department because of the development of scattered petechiae on both hands and blood blisters in her mouth. On admission, her complete blood cell count results revealed a severely low platelet count of 2 x 10(3)/mm3. Other laboratory test results were normal, except for elevated blood glucose (nonfasting blood glucose). TMP/SMX was believed to be the most likely cause of thrombocytopenia. She was treated successfully with a transfusion of 2 units of platelets and oral prednisone. Her platelet count increased to 110 x 10(3)/mm3 4 days after discontinuation of TMP/SMX. She was discharged on hospital day 5. On follow-up (2 wk after hospital discharge), her platelet count was normal (351 x 10(3)/mm3). TMP/SMX has been implicated as a cause of thrombocytopenia, which is defined as platelet count &lt; 150 x 10(3)/mm3. Although it is uncommon, spontaneous severe bleeding may occur when platelet count decreases to &lt; or = 10 x 10(3)/mm3. Thrombocytopenia associated with TMP/SMX appears to be an immune-mediated process resulting in platelet destruction by drug-dependent platelet antibodies. Treatment of thrombocytopenia associated with TMP/SMX therapy includes discontinuation of the offending drug and the use of corticosteroids. Platelet transfusion and intravenous immunoglobulin may be required in some patients. Thrombocytopenia associated with TMP/SMX therapy can be serious or life threatening because it may result in significant bleeding complications. This hematologic adverse effect of TMP/SMX may occur even with the usual recommended dosage and duration of therapy. Careful monitoring of complete blood cell count, including platelet count, before and during TMP/SMX therapy is suggested."}, {"id": "pubmed23n0528_991", "title": "[Castleman disease].", "score": 0.00980392156862745, "content": "A 66 years female, who was since last year under astenia, arthralgias, pimply lesions in spread plates and tests showing eritrosedimentation over 100 mm, anemi, leucocitosis with neutrofilia, policlonal hypergammaglobulinemia, slight proteinuria and IgE on 900. This patient was sporadically treated with corticoids. When made the medical consult had lost 34lb., was under anorexy, as well as dyspepsia. Hemoglobyn 6.9 gr/dl, leucocytes 20000/mm3, neutrofils at 90%, proteinogram the same as former, with hypoalbuminemia. She was taking prednisona, 16 mg/day. When examined showed depress of conscience, astenia, and dermic lesions already quoted. 4 cm nonpainful right axillary adenopaty adhered to deep planes. Medulogram with increased iron, hyperegenerative. Ganglionar biopsia: linfoid hyperplasic process linked to inmune response. Toracoabdominal tomography with adenomegalia in torax and retroperitoneo. Skin biopsia: neutrofilic vasculitis. The patient suspends the 16 mg of prednisona and fever as well as generalized adenopatias come up. After laying aside other ethiologies, and understanding as Castleman Multicentric disease, it is started to supply prednisona 1 mg/kg of weight with a clinical and biochemical fast and outstanding response. After 7 months it was progressively suspended the esteroids and 60 days later, the process fall back; for that, corticoids are restarted, with a good evolution. The illness of Castleman although it is not very frequent, it should be considered as differential diagnosis in those clinical cases that are accompanied with important general commitment, linphadenopaties and respons to steroid therapy."}, {"id": "wiki20220301en069_23982", "title": "Waldenström macroglobulinemia", "score": 0.009793123264055167, "content": "The International Prognostic Scoring System for Waldenström's Macroglobulinemia is a predictive model to characterise long-term outcomes. According to the model, factors predicting reduced survival are: Age > 65 years Hemoglobin ≤ 11.5 g/dL Platelet count ≤ 100×109/L B2-microglobulin > 3 mg/L Serum monoclonal protein concentration > 70 g/L The risk categories are: Low: ≤ 1 adverse variable except age Intermediate: 2 adverse characteristics or age > 65 years High: > 2 adverse characteristics Five-year survival rates for these categories are 87%, 68% and 36%, respectively. The corresponding median survival rates are 12, 8, and 3.5 years. The International Prognostic Scoring System for Waldenström's Macroglobulinemia has been shown to be reliable. It is also applicable to patients on a rituximab-based treatment regimen. An additional predictive factor is elevated serum lactate dehydrogenase (LDH)."}, {"id": "pubmed23n0401_18953", "title": "Extreme thrombocytosis as a sign of coeliac disease in the elderly: case report.", "score": 0.009708737864077669, "content": "Increase in the number of blood platelets to over 1,000,000/mm3 in elderly patients is generally considered secondary to a myeloproliferative or neoplastic disease. To report the case of an elderly woman hospitalized for extreme thrombocytosis associated with severe anaemia, who was found to be suffering from coeliac disease. The patient, aged 83 years, was hospitalized presenting with fatigue. Laboratory tests showed microcytic hypochromic anaemia (haemoglobin 4 g/dl) and extreme thrombocytosis (platelet count 1,400,000/mm3). Physical examination was normal, with the exception of marked thinness. There was no evidence of macroscopic bleeding from the gastrointestinal or genitourinary tracts. She had never suffered from gastrointestinal problems and had no family history of gastroenterological diseases. Oesophagogastroduodenoscopy and histology of the gastric and duodenal mucosa evidenced atrophic gastritis and an adenomatous polyp. The duodenal mucosa showed total villous atrophy, suggesting the diagnosis of coeliac disease. Antiendomysial IgA and anti-transglutaminase IgA antibodies were also positive. Colonoscopy was negative. An ultrasound examination of the abdomen was normal, and the spleen was within the normal range. A peripheral blood smear showed no alterations in erythrocyte morphology typical of hyposplenism due to coeliac disease. The platelet count decreased rapidly after blood transfusions, when both serum iron and ferritin levels were still below normal limits. Furthermore, we observed a significant inverse correlation between the platelet count and haemoglobin concentration (r = -0.94, P &lt; 0.003). Platelet count and red blood cell count normalized after 2 months of a gluten-free diet; the haemoglobin concentration was also normal at this time. After 1 year of following a gluten-free diet, the patient remained well and had no complaints. There were no gastrointestinal disturbances. All haematological parameters were within normal limits. Intestinal biopsies showed normal villi and crypts without inflammatory infiltration of the lamina propria. This case shows that the association of haematological signs--extreme thrombocytosis and severe anaemia--considered in an elderly patient to be typical of myeloproliferative disorders or neoplastic conditions can be due to coeliac disease; thus, coeliac disease must also be considered among the possible diagnoses."}, {"id": "pubmed23n0505_17973", "title": "Clinical features of Waldenstrom macroglobulinemia in Korea.", "score": 0.009708737864077669, "content": "Waldenstrom macroglobulinemia (WM) is a lymphoproliferative disorder characterized by monoclonal IgM. Its rarity makes it difficult to know the clinical manifestations and outcomes of patients with WM. The clinical records of 13 patients diagnosed with WM between 1983 and 2003 were reviewed, and 12 patients were eligible. The median age was 57 years (range, 40 to 85), and the male to female ratio was 2. B symptoms and hyperviscosity requiring plasmapheresis existed in 5 and 4 patients, respectively, at the time of diagnosis. Hepatomegaly and splenomegaly were detected in 5 and 3 patients, respectively. Sites of extranodal involvement were bone (3) and lung (1) in 3 patients. The peripheral neuropathy was complicated in 3 patients. (Ed note: check this sentence.) Cryoglobulin was checked in 6 patients and it was detected in 3 of them. The median concentration of serum IgM was 4.2 g/dL (0.7-6.2). The median albumin, hemoglobin, WBC, and platelet levels were 2.8 g/dL, 8 g/dL, 5,400/microL, and 138,000/microL, respectively. One patient had transitional cell carcinoma concomitantly, and one patient developed small cell lung cancer. Of the 11 patients receiving chemotherapy (7-chlorambucil, 2-melphalan, 1-cyclophosphamide, 1-CHOP), 4 patients showed the objective responses including 2 complete remissions, but they all ultimately relapsed. The response rate of second-line therapy was 14% (1/7). After a median follow-up of 20 months, 3 patients were still alive with disease. The median overall and progression-free survival were 24 months (95% confidence interval (CI): 5-43) and 24 months (95% CI: 8-40), respectively. The initial high levels of serum IgM and severe anemia reflect a lack of suspicion of WM at the early stage. Careful suspicion and proper diagnostic approaches will allow more patients to show an improved outcome."}, {"id": "pubmed23n0660_1372", "title": "[Malignant peritoneal mesothelioma].", "score": 0.009708286882199926, "content": "The peritoneal mesothelioma is a rare pathology with unspecific symptoms reason to be a difficult diagnosis. We report a case of a 58 year old man with diabetes mellitus type 2, arterial hypertension and smoking; without precedent of asbestos exposure. The patient presented a one month history characterized by progressive increase of the abdominal volume and sensation of fullness; three weeks later they added breathlessness and hyporexia. The patient was in regular general condition; he was not presenting hepatic stigmas, edema or adenomegalies. The examination of thorax and cardiovascular it was normal. The abdomen distended by ascites, not painful, liver and spleen not examined. Laboratory: Hemoglobin 11,9 gr/dl, WBC 6840/mm3 Bands 1 %, lymphocytes 10 %, platelets 620000/mm3, PT 12 seconds, PTT 34 seconds, glucose 158 mg/dl, BUN 20,5 mg/ dl, creatinine 1,2 mg/dl, proteins 6,1 gr/dl, albumin 2,6 gr/dl. LDH 316 U/l, beta2microglobulin 2,2 mg/l (0.83-1.15 mg/l). HBV and HCV negative. Ca 19.9, CEA, AFP and PSA negative. Hemocultive negative. Ascitic fluid: ADA 20,3 U/l, serum-ascitic albumin gradient (SAAG) 1,1. Leukocytes 2237 cells/mm3, PMN 6 %, lymphocytes 90 %, mesothelial cells 4 %, proteins 4,6 gr/dl, albumin 2,34 gr/dl, glucose 44 mg/dl, LDH 1918 U/l. Gram and cultive: negatives. BAAR and cultive: negative . Cytology: mesothelial cells with changes of type reagent, Block cell for tumour cells: negative. Abdominal US: increased peritoneum and abundant ascitic fluid. Thoracic-abdominal CT: left side pleural effusion, severe ascites with thick epyplon. Upper GI endoscopy: moderate gastritis. Colonoscopy: two small sessile polyps in sigmoid colon. The finds of the laparoscopy were interpreted like carcinomatosis or peritoneal tuberculosis. The report of the peritoneal biopsy was informed as suggestive of undifferentiated carcinoma; the reappraisal with inmunohystochemic (calretinin +,cytokeratin +, vimentin +) indicated malignant peritoneal mesothelioma, type epithelial. The evolution was torpid. The patient was transferred to the Service of Oncology where they initiated chemotherapy with Cysplatin (CDDP) and died 20 days later. The malignant mesothelioma peritoneal is a unfrequent entity, with limited therapeutic options; generally detected late, with a palliative treatment."}, {"id": "pubmed23n1085_24991", "title": "Case 294.", "score": 0.009615384615384616, "content": "History A 50-year-old woman presented to the emergency department of our hospital with a 2-day history of lower limb pain associated with unusual asthenia and diffuse arthralgia over the past 3 weeks. She was a native of Guinea and had lived in France for most of her life, working as a personal care assistant. Her only medical history of note was an occurrence of fetal death at 12 weeks gestation when she was 35 years old. She had bilateral lower limb swelling, without changes in skin temperature or color. All proximal and distal arterial pulses were felt. General physical examination findings were otherwise unremarkable. Her laboratory tests showed a decreased hemoglobin concentration of 8.9 g/dL (normal range, 12-16 g/dL), a decreased platelet count of 45 × 10<sup9</sup/L (normal range, 150-400 × 10<sup9</sup/L), a C-reactive protein level of 158 mg/L (normal range, &lt;5 mg/L) and a d-dimer level of 2000 mg/L (normal range, &lt;500 mg/L). Compression US of the lower limbs revealed bilateral calf vein thrombosis involving the fibular and posterior tibial veins. Curative anticoagulation using low-molecular-weight heparin (enoxaparin, subcutaneous injection of 100 units per kilogram of body weight twice a day) was started. The day after the start of anticoagulation therapy, the patient reported dyspnea and acute chest and abdominal pain. Her vital signs were assessed, and she had elevated blood pressure and increased heart rate and respiratory rate, but she remained afebrile. Her cardiac auscultation was unremarkable, besides tachycardia. Skin examination revealed small areas of necrosis on the fingertips of her right hand. Laboratory studies were repeated and showed an increase in serum creatinine level from a baseline value of 0.49 mg/dL to a new value of 1.01 mg/dL (normal range, 0.6-1.1 mg/dL), an apparition of low-grade proteinuria of 0.43 g per day (normal range, &lt;0.3 g/day), and a high serum troponin level of 1066 ng/L (normal range, &lt;14 ng/L), whereas electrocardiography showed no ST segment modification and echocardiography revealed a moderately altered left ventricular ejection fraction (45%). There was no coronary occlusion seen at emergency coronarography. Contrast-enhanced CT of the chest, abdomen, and pelvis was performed (Figs 1, 2) together with cardiac MRI (Figs 3, 4)."}, {"id": "InternalMed_Harrison_15799", "title": "InternalMed_Harrison", "score": 0.009615384615384616, "content": "During the prodrome, the differential diagnosis of HPS is difficult, but by the time of presentation or within 24 h thereafter, a number of diagnostically helpful clinical features become apparent. Cough usually is not present at the outset. Interstitial edema is evident on a chest x-ray. Later, bilateral alveolar edema with a central distribution develops in the setting of a normal-sized heart; occasionally, the edema is initially unilateral. Pleural effusions are often seen. Thrombocytopenia, circulating atypical lymphocytes, and a left shift (often with leukocytosis) are almost always evident; thrombocytopenia is a particularly important early clue. Hemoconcentration, hypoalbuminemia, and proteinuria should also be sought for diagnosis. Although thrombocytopenia virtually always develops and prolongation of the partial thromboplastin time is the rule, clinical evidence for coagulopathy or laboratory indications of disseminated intravascular coagulation (DIC) are found in only a"}, {"id": "pubmed23n1102_22675", "title": "Case 294: Catastrophic Antiphospholipid Syndrome.", "score": 0.009523809523809525, "content": "History A 50-year-old woman presented to the emergency department of our hospital with a 2-day history of lower limb pain associated with unusual asthenia and diffuse arthralgia over the past 3 weeks. She was a native of Guinea and had lived in France for most of her life, working as a personal care assistant. Her only medical history of note was an occurrence of fetal death at 12 weeks gestation when she was 35 years old. She had bilateral lower limb swelling, without changes in skin temperature or color. All proximal and distal arterial pulses were felt. General physical examination findings were otherwise unremarkable. Her laboratory tests showed a decreased hemoglobin concentration of 8.9 g/dL (normal range, 12-16 g/dL), a decreased platelet count of 45 × 10<sup9</sup/L (normal range, [150-400] × 10<sup9</sup/L), a C-reactive protein level of 158 mg/L (normal range, &lt;5 mg/L), and a d-dimer level of 2000 mg/L (normal range, &lt;500 mg/L]). Compression US of the lower limbs revealed bilateral calf vein thrombosis involving the fibular and posterior tibial veins. Curative anticoagulation using low-molecular-weight heparin (enoxaparin, subcutaneous injection of 100 units per kilogram of body weight twice a day) was started. The day after the start of anticoagulation therapy, the patient reported dyspnea and acute chest and abdominal pain. Her vital signs were assessed, and she had elevated blood pressure and increased heart rate and respiratory rate, but she remained afebrile. Her cardiac auscultation was unremarkable, besides tachycardia. Skin examination revealed small areas of necrosis on the fingertips of her right hand. Laboratory studies were repeated and showed an increase in serum creatinine level from a baseline value of 0.49 mg/dL to a new value of 1.01 mg/dL (normal range, 0.6-1.1 mg/dL), an apparition of low-grade proteinuria of 0.43 g per day (normal range, &lt;0.3 g/ day), and a high serum troponin level of 1066 ng/L (normal range, &lt;14 ng/L), whereas electrocardiography showed no ST segment modification and echocardiography revealed a moderately altered left ventricular ejection fraction (45%). There was no coronary occlusion seen at emergency coronarography. Contrast-enhanced CT of the chest, abdomen, and pelvis was performed (Figs 1, 2) together with cardiac MRI (Figs 3, 4)."}, {"id": "pubmed23n0697_18552", "title": "Cutaneous manifestations of Strongyloides stercoralis hyperinfection in an HIV-seropositive patient.", "score": 0.009433962264150943, "content": "A 41-year-old human immunodeficiency virus (HIV)-positive man was hospitalized with complaints of a 4-week history of nausea and vomiting, associated with decreased oral intake, and a 4-day history of frontal headache and fever. His medical history was significant for a gunshot wound to the head 3 years prior, with a residual seizure disorder. He also had two previous hospitalizations, both for culture-negative bacterial meningitis; the first episode occurred 12 months before admission and the second episode occurred 5 months later. At that time, he was found to be positive for serum antibodies against HIV and a CD4+ T-lymphocyte count of 126/mm3. He had no known drug allergies and was not receiving any medication. On admission, the patient was febrile (104.0 degrees F) and hypotensive (blood pressure, 92/40 mm Hg). Pertinent physical examination findings included cachexia with bitemporal wasting, dry mucus membranes, adherent white patches on the oral mucosa, and negative Kernig's and Brudzinski's signs. His laboratory results revealed macrocytic anemia, a decreased serum sodium of 125 mEq/L, and a normal total leukocyte count with a CD4+ T-lymphocyte count &lt; 50/mm3. Lumbar puncture opening pressure was elevated at 160 mm Hg, and cerebrospinal fluid analysis showed an increased white cell count of 97/microL (84% lymphocytes), a decreased glucose level of 26 mg/dL, and a decreased protein level of 42 mg/dL. The patient was started on empiric therapy that included intravenous ampicillin and cefotaxime, oral Bactrim, and clotrimazole lozenges for thrush. Cerebrospinal fluid culture was positive for Escherichia coli, sensitive to cefotaxime. Two days later, the patient developed fine, erythematous, nonblanchable macules primarily on his abdomen, with minimal involvement of his thorax and back. His skin lesions remained unchanged for the next 2 weeks. Repeat lumbar puncture was performed after 14 days of cefotaxime. The cerebrospinal fluid analysis showed an elevated white cell count of 7/microL (100% lymphocytes), a decreased glucose level of 53 mg/dL, and a decreased protein level of 33 mg/dL. The cerebrospinal fluid culture was now positive for Pseudomonas aeruginosa resistant to cefotaxime. The patient was started on imipenem. On day 34 of his admission, the patient became tachypneic with complaints of dyspnea. A chest roentgenogram revealed bilateral patchy infiltrates. He was transferred to the intensive care unit and intubated for hypoxemic respiratory failure (arterial blood gas values on 6 L of oxygen: pH, 7.46; bicarbonate, 23; and oxygen saturation, 37). That evening, the patient was also noted to have diffuse petechiae and purpura in a reticulated pattern over his abdomen (Figure 1A and 1B), most heavily concentrated in the periumbilical region, extending to the axillae and upper thighs. A 3x3-mm punch biopsy from abdominal skin demonstrated Strongyloides stercoralis larvae in the dermis (Figure 2A and 2B). His sputum specimen was teeming with adult S stercoralis worms (Figure 3) and, subsequently, numerous S stercoralis larvae were observed not only from the bronchoalveolar lavage but also from the nasogastric fluid specimen. These findings confirmed the diagnosis of disseminated strongyloidiasis. On hospital day 35, the patient was doing poorly and was started on thiabendazole (1250 mg twice daily for 28 days). Nine days later, ivermectin (4.5 mg once daily for 3 days for 2 courses) was also added. He continued to clinically deteriorate. The patient died 31 days after systemic antihelminthic treatment was initiated."}, {"id": "pubmed23n1155_14862", "title": "Severe hemolysis with negative direct antiglobulin test: A case report.", "score": 0.009433962264150943, "content": "A 49-year-old woman with type 2 diabetes mellitus (T2DM) presented to the emergency department. Her examination showed marked pallor, exhaustion, lethargy, yellowish eyes, anorexia, nausea and vomiting. Hematuria; negative standard direct antiglobulin test (DAT); normal glucose 6 phosphate dehydrogenase (G6PD); hemoglobin (Hb), 4.8 g/dl; Mean cell volume (MCV), 91fl; platelet count, 233 × 10<sup6</sup/L; Total bilirubin, 7.0 mg/dl; Glucose, 316 mg/dl; lactate dehydrogenase (LDH), 1750U/L. Undoubtedly, therapeutic panel should have been used for hemolytic anemia. Intravenous (IV) fluids and 2 units of packed cell were transfused. Methylprednisolone with rituximab were started for the patient. After 3 weeks of the patient admission, she was discharged home with stable vital signs and Hb, 10 g/dl. We concluded in the cases that presented along with a severe drop in Hb and evidence of hemolysis which non immune hemolytic anemia is excluded in spite of negative standard DAT limited transfusion besides corticosteroids combined with rituximab, could be helpful in saving the patient."}, {"id": "wiki20220301en034_55852", "title": "Hairy cell leukemia", "score": 0.009363657839915811, "content": "Diagnosis The diagnosis of HCL may be suggested by abnormal results on a complete blood count (CBC), but additional testing is necessary to confirm the diagnosis. A CBC normally shows low counts for white blood cells, red blood cells, and platelets in HCL patients. However, if large numbers of hairy cells are in the blood stream, then normal or even high lymphocyte counts may be found. On physical examination, 80–90% of patients have an enlarged spleen, which can be massive. This is less likely among patients who are diagnosed at an early stage. Peripheral lymphadenopathy (enlarged lymph nodes) is uncommon (less than 5% of patients), but abdominal lymphadenopathy is a relatively common finding on computed tomography scans."}, {"id": "pubmed23n0653_10897", "title": "Possible green tea-induced thrombotic thrombocytopenic purpura.", "score": 0.009345794392523364, "content": "A case of a patient who developed thrombotic thrombocytopenic purpura (TTP) after consuming a weight-loss product containing green tea is reported. A 38-year-old, 68-kg Caucasian woman arrived at the emergency department with a one-week history of malaise, fatigue, and petechiae of the skin. She had no symptoms of infection and denied illegal drug use. Her medical history included hypothyroidism, for which she was treated with levothyroxine 150 microg daily for the past four years. She reported that she had been using a green tea preparation for the two months before admission to lose body weight. The daily preparation contained 200 mg of green tea extract 5:1, equivalent to 1 g of natural green tea. On clinical examination, the patient appeared acutely ill and was afebrile, with pallor, petechiae, and purpura of the extremities. Laboratory test results at the time of admission revealed that the patient had anemia and marked thrombocytopenia. A peripheral blood smear demonstrated a feature of microangiopathic hemolytic anemia. Immunoglobulin G autoantibodies against ADAM metallopeptidase with thrombospondin type 1 motif, 13 were detected. On hospital day 3, the patient appeared confused and exhibited aphasia that was initially transient but then recurrent. Brain computerized tomography did not exhibit focal pathology. Over the next few days, her neurologic symptoms subsided and her platelet count and hematocrit value gradually increased. Plasmapheresis was performed (12 procedures). Corticosteroid treatment was also initiated. After 20 days of hospitalization, the patient was discharged. A 38-year-old woman developed TTP after consuming a weight-loss product containing green tea extract for two months."}, {"id": "pubmed23n0519_1503", "title": "[Laboratory findings in patients with hemorrhagic fever with renal syndrome].", "score": 0.009345794392523364, "content": "To examine the frequency and distribution of hematologic and biochemical laboratory findings in 94 patients with hemorrhagic fever with renal syndrome (HFRS) in the epidemic year 2002. The following laboratory findings were retrospectively analyzed: erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), hemoglobin, hematocrit, leukocyte count and differential percentage (segmented neutrophils, band neutrophils, atypical lymphocytes), platelet count, coagulation tests, blood urea nitrogen (BUN), creatinine, urine, potassium, bilirubin (BIL), aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyltransferase (GT), alkaline phosphatase (ALP), and serum protein electrophoresis. The study included 94 HFRS patients treated at the Dr Fran Mihaljević University Hospital for Infectious Diseases in Zagreb during 2002. ESR increase, mostly mild to moderate, was found in 86.2% of study patients. Increased CRP was recorded in 98.9% of study patients, however, one-fourth had CRP higher than 100 mg/L. Leukocytosis was recorded in 38.3% (10.1 +/- 4.2 x 10(9)/L), thrombocytopenia in 89.4% patients (68.2 +/- 48.3 x 10(9)/L), and severe thrombocytopenia (x 10(9)/L) in six patients. Three patients had abnormal coagulation tests. Increased values of BUN and creatinine were recorded in more than a half of patients, while only four patients had mild hyperkalemia. Only three patinets required hemodialysis. Mildly to moderately increased values of aminotransferases (AST, ALT, GT) were observed in more than 2/3; hypoalbuminaemia in nearly 1/3, and elevated alpha-2 fraction in more than 2/3 of patients. The majority of patients had pathologic urine findings. First laboratory abnormalities were usually found between day 5 and 7 of the disease (increased CRP level, thrombocytopenia, leukocytosis, and elevation of hemoglobin and hematocrit). Biochemical abnormalities(elevation of cratinine and urea, increased levels of aminotransferases) usually occurred at the beginning of the second week, and ESR increase in the second week of disease. The majority of our patients had laboratory findings characteristic of HFRS. Thrombocytopenia and increased level of CRP were the most common laboratory findings during the first week of the disease. Renal and liver impairment occurred at the beginning of the second week of the disease."}, {"id": "pubmed23n0482_11099", "title": "Cases from the Osler Medical Service at Johns Hopkins University.", "score": 0.009259259259259259, "content": "A 47-year-old white woman with a history of stage III squamous cell carcinoma of the anus was transferred to Johns Hopkins Hospital for further evaluation of renal failure, hemolytic anemia, and thrombocytopenia. The patient was first diagnosed with squamous cell carcinoma of the anus 1 year before admission. She was treated with external beam radiation of the pelvis and two cycles of mitomycin C-based chemotherapy (a cumulative dose, 34 mg/m(2)). Her clinical course was complicated by Clostridium difficile colitis and myositis successfully treated with prednisone. Three months before admission, the patient developed dysuria. Her creatinine increased from normal to 1.7 mg/dL, and microscopic hematuria was present. A renal ultrasound and an abdominal computed tomographic scan showed no abnormalities or obstruction. One month before admission, she underwent a cystoscopy, which showed only radiation-induced changes in the bladder. Two weeks before admission, the patient became delirious and was taken to a hospital, where she was found to be anemic, with a hematocrit level of 23.7%, and thrombocytopenic with a platelet count of 110,000/mm(3). Her creatinine level was 5.9 mg/dL. Previous values of hematocrit, platelet count, and serum creatinine were normal. On admission at Johns Hopkins Hospital the patient had no complaints. She was afebrile on physical examination and had normal vital signs. Head, neck, chest, cardiovascular, and abdominal examinations were normal. There was skin pallor, but no echymoses or petechiae. She was alert and oriented with normal mental status. Her neurologic examination was normal. Laboratory data showed a white blood cell count of 6390/mm(3), a hematocrit level of 26.5%, and a platelet count of 26,000/mm(3). Her blood urea nitrogen level was 57 mg/dL, creatinine level was 4.0 mg/dL, and lactate dehydrogenase was 550 U/L (reference, 115 to 275 U/L). Urinalysis showed innumerable red blood cells and large protein. A peripheral blood smear showed fragmented red blood cells, schistocytes, no abnormal white blood cells, and few platelets. There was no radiographic or clinical evidence of relapse of her squamous cell carcinoma. What is the diagnosis?"}, {"id": "pubmed23n0880_16409", "title": "An improbable and unusual case of thrombotic thrombocytopenia purpura.", "score": 0.009259259259259259, "content": "Thrombotic thrombocytopenic purpura (TTP) is a life-threatening medical emergency which may be difficult to recognize given the wide spectrum in which it presents. A delay in treatment may be catastrophic as untreated cases of TTP have a mortality rate exceeding 90%. Given the high fatality rate of untreated TTP and its range of presenting symptoms, we present our unusual case of TTP in a post-splenectomy patient with early treatment and positive outcome. This case describes a 54-year-old female who presented with hematuria and gingival bleeding, followed by the development of a bilateral lower extremity petechial rash. Her past medical history was significant for multiple episodes of TTP, the last of which resulted in a splenectomy and a 20-year history of remission thereafter. On exam, she was alert, well appearing, and neurologically intact. Her only significant finding was a bilateral lower extremity petechial rash. Laboratory studies revealed mild anemia and thrombocytopenia, an elevated lactate dehydrogenase, and a decreased haptoglobin. Peripheral smear showed poikilocytosis, helmet cells, and schistocytes. Corticosteroid therapy was promptly initiated, her platelets were monitored closely, and she underwent urgent therapeutic plasma exchange. Due to the risk of significant morbidity and mortality that may result from delayed treatment of TTP as well as the significant variations of presentation, TTP requires a consistently high index of suspicion. Our patient suffered multiple relapses of TTP within a 30-year span, underwent splenectomy in early adulthood, and presented with atypical symptoms during her most recent relapse illustrating how persistent TTP can be as well as how unusually it may present. Providers should be aware of the vast spectrum of presentation and remember that TTP may recur following splenectomy despite prolonged remission. "}, {"id": "pubmed23n1107_20179", "title": "A Case of Evans Syndrome and Unstable Angina.", "score": 0.009174311926605505, "content": "Evans syndrome (ES) is characterized by autoimmune hemolytic anemia (AIHA) and immune-mediated thrombocytopenia. It is more common in the pediatric population than in adults. ES has been reported to be associated with thrombotic events and rarely can lead to acute coronary syndrome (ACS). There have been only a few reported cases of ACS secondary to ES. We present an interesting case of ES with unstable angina (UA) which had a limited response to oral and intravenous (IV) steroids requiring rituximab. A 64-year-old male with past medical history significant for hypertension, hyperlipidemia, diabetes mellitus and coronary artery disease, presented to the emergency room complaining of a 2-week history of chest pain, shortness of breath and hematuria. Physical examination indicated splenomegaly but was otherwise unremarkable with no petechiae or rash. Labs showed hemoglobin of 9.6 g/dL, platelet count 58 × 10<sup3</sup/µL, troponin &lt; 0.03 ng/mL, lactic acid 2.5 mmol/L and with parameters indicative of hemolysis, evidenced by elevated lactate dehydrogenase, low haptoglobin and elevated bilirubin levels. Electrocardiography (EKG) demonstrated ST depression in leads I, aVL, V5 - V6 and T wave inversions in lead III and aVL, which were new compared to previous EKG. Peripheral blood smear indicated spherocytes. Direct antiglobulin test was positive for immunoglobulin G (IgG). Patient was admitted for ES and initially treated with oral prednisone 80 mg daily. He was also diagnosed with UA thought to be possibly secondary to ES. He then underwent cardiac stress test which showed mild reversible inferior apical ischemia. Cardiac catheterization revealed 95% stenosis of proximal left circumflex artery requiring single drug eluding stent placement and dual antiplatelet therapy. Patient continued to have anemia despite blood transfusions, although platelet count improved. Prednisone was transitioned to high-dose IV dexamethasone, and patient was also started on rituximab which resulted in stabilization of anemia. The presentation of ES with ACS is a rare occurrence. ACS can be challenging to manage as stent placement may be required followed by dual antiplatelet therapy. Treatment of ES involves steroids followed by rituximab, IV immunoglobulin (IVIG) or splenectomy for non-responsive cases. Early intervention and management can prevent mortality and morbidity."}, {"id": "pubmed23n0286_11225", "title": "Efficient diagnosis of thrombocytopenia.", "score": 0.009174311926605505, "content": "Thrombocytopenia may be a benign, incidental finding in an asymptomatic patient or the sign of a potentially life-threatening disorder. The history, physical examination and peripheral blood smear can assist the physician in determining the diagnosis and treatment. After the initial blood count is repeated to help eliminate the possibility of laboratory error, a thorough history, complete physical examination, complete blood cell count and appropriate laboratory tests are required. The history may reveal related illnesses, risk factors such as infection or drug use, or a family history suggestive of congenital thrombocytopenia. The physical examination should concentrate on the lymphatic and hepatosplenic systems, with the physician looking for jaundice, fever or petechiae. With the review of a complete blood cell count and a peripheral smear examination, the initial work-up is completed and may prevent additional, unnecessary testing. Etiology-specific tests follow if needed. Serious spontaneous bleeding is usually a risk only in patients with platelet levels under 20,000 per mm3."}, {"id": "pubmed23n0278_1328", "title": "Amegakaryocytic thrombocytopenia with a positive direct Coombs' test.", "score": 0.00909090909090909, "content": "A 50-year-old previously well woman developed a sudden onset of ecchymoses and petechiae over the trunk and extremities and gum bleeding. There was no history of alcohol use or drug ingestion. Physical examination was normal except for the ecchymoses, purpuric rash, and gum bleeding. Complete blood count showed Hb 8.0 g/dl, leucocytes 10.9 x 10(9)/L with a normal differential, platelets 8 x 10(9)/L and reticulocytes, 4%. The bone marrow was normocellular and showed absence of megakaryocytes; there was normoblastic erythroid hyperplasia and the myelopoiesis was normal. The direct Coombs test result was positive. There was poor response to treatment with prednisone and bolus doses of vincristine. Therapy with danazol resulted in the complete normalization of the blood counts, and the patient remains well 32 months after diagnosis and is currently taking 100 mg danazol daily."}, {"id": "pubmed23n0413_21249", "title": "[Adult-onset Still-disease: survey of 18 cases].", "score": 0.00909090909090909, "content": "Adult onset Still's disease (juvenile rheumatoid arthritis with septic appearance) is rare, leading to clinical signs similar to those seen in bacterial sepsis, lymphomas, rheumatological, or systemic autoimmune diseases. The disease can present with a fever of unknown origin, and can cause difficulties in the diagnosis. It is based upon, partly, the exclusion of other diseases and on diagnostic criteria. Its characteristic feature is the rise of acute phase proteins. Exanthemata are temporary. The basis of treatment is immunosuppression, however relapses can occur. The aim of the authors was to evaluate on the most characteristic clinical signs and laboratorical data of their patients, and to examine the revealing parameters of the course of the disease. Retrospective epidemiological survey of the data obtained from 18 patients. The characteristic signs of the disease were, fever, sore throat, arthritis, joint pain, exanthemata, hepato-splenomegaly, lymphadenomegaly, pleurisy. The typical laboratorical data were: elevated CRP, low PCT, negative Waaler-Rose and ANA test, low serum iron level, leukocytosis, thrombocytosis, elevated alkalic phosphatase activity, high LDH, positive bone scintigraphy. The fever was steroid dependent. Generally, the illness was recognised after 2-3 months, and relapses were frequent. Still's disease has an important role in the differential diagnosis of fever of unknown origin. The diagnosis is based upon the evaluation of clinical signs and laboratorical data together. Prolonged immunosuppressive therapy is required."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The trick to this question is that there are 8 cervical roots for 7 vertebrae, therefore the C1 root exits ABOVE the atlas, and so on up to C8 which exits BETWEEN C7 and T1. The thoracic roots come out respectively below the vertebra with their same numbering (nerve T1 under T1, T2 under T2, etc.). Therefore, between C4 and C5 the 5th cervical nerve exits, and between T4 and T5 the 4th thoracic nerve exits. The correct answer is 4.", "full_answer_no_ref": "The trick to this question is that there are 8 cervical roots for 7 vertebrae, therefore the C1 root exits ABOVE the atlas, and so on up to C8 which exits BETWEEN C7 and T1. The thoracic roots come out respectively below the vertebra with their same numbering (nerve T1 under T1, T2 under T2, etc.). Therefore, between C4 and C5 the 5th cervical nerve exits, and between T4 and T5 the 4th thoracic nerve exits. [HIDDEN]", "full_question": "A 42-year-old woman with breast cancer has metastases at the level of the intervertebral foramina between the 4th and 5th cervical vertebrae and between the 4th and 5th thoracic vertebrae. Which spinal nerves will be injured?", "id": 64, "lang": "en", "options": {"1": "Fourth cervical and fourth thoracic nerves.", "2": "Fifth cervical and fifth thoracic nerves.", "3": "Fourth cervical and fifth thoracic nerves.", "4": "Fifth cervical and fourth thoracic nerve.", "5": "Third cervical and fourth thoracic nerve."}, "question_id_specific": 210, "type": "ANATOMY", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en038_6014", "title": "Thoracic vertebrae", "score": 0.018521679754182313, "content": "Third thoracic vertebra (T3) The thoracic spinal nerve 3 (T3) passes out underneath it. Fourth thoracic vertebra (T4) The fourth thoracic vertebra, together with the fifth, is at the same level as the sternal angle. The thoracic spinal nerve 4 (T4) passes out underneath it. Fifth thoracic vertebra (T5) The fifth thoracic vertebra, together with the fourth, is at the same level as the sternal angle. The human trachea divides into two main bronchi at the level of the 5th thoracic vertebra, but may also end higher or lower, depending on breathing. The thoracic spinal nerve 5 (T5) passes out underneath it. Sixth thoracic vertebra (T6) The thoracic spinal nerve 6 (T6) passes out underneath it. Seventh thoracic vertebra (T7) The thoracic spinal nerve 7 (T7) passes out underneath it. Eighth thoracic vertebra (T8) The eighth thoracic vertebra is, together with the ninth thoracic vertebra, at the same level as the xiphisternum."}, {"id": "wiki20220301en198_26901", "title": "Cervical spinal nerve 5", "score": 0.017773253993109927, "content": "The cervical spinal nerve 5 (C5) is a spinal nerve of the cervical segment. It originates from the spinal column from above the cervical vertebra 5 (C5). It contributes to the phrenic nerve, long thoracic nerve, and dorsal scapular nerve before joining cervical spinal nerve 6 to form the upper trunk, a trunk of the brachial plexus, which then forms the lateral cord, and finally the musculocutaneous nerve. Additional Images References Spinal nerves"}, {"id": "pubmed23n0047_10667", "title": "Variations of the ventral rami of the brachial plexus.", "score": 0.016808480959687542, "content": "We studied the variations in the ventral rami of 152 brachial plexuses in 77 Korean adults. Brachial plexus were composed mostly of the fifth, sixth, seventh and eighth cervical nerves and the first thoracic nerve (77.0%). In 21.7% of the cases examined, the fourth, fifth, sixth, seventh and eighth cervical and the first thoracic nerves contributed to the plexus. A plexus composed of the fourth, fifth, sixth, seventh and eighth cervical and the first and second thoracic nerves, and a plexus composed of the fifth, sixth, seventh eighth cervical nerves were also observed. The plexuses were classified into three groups according to cephalic limitation, and the plexus of group 2 in which the whole fifth cervical nerve enters the plexus, were observed the most frequent. The average diameter of the sixth and the seventh cervical ventral rami of the plexus was greatest and that of the fifth cervical was smallest. The largest nerve entering the plexus was the sixth or the seventh cervical nerve in about 79% of cases. The dorsal scapular nerve originated from the fifth cervical ventral ramus in 110 cases (75.8%). The long thoracic nerve was formed by joining of roots from the fifth, sixth, and seventh cervical nerves in 76.0% of cases. Also, a branch to the phrenic nerve, the suprascapular nerve, a nerve to the pectoralis major muscle and a nerve to the subscapular muscle arising from the ventral rami of the plexus were observed."}, {"id": "wiki20220301en012_15402", "title": "Spinal nerve", "score": 0.016581719334012913, "content": "The cervical nerves innervate the sternohyoid, sternothyroid and omohyoid muscles. A loop of nerves called ansa cervicalis is part of the cervical plexus. Thoracic nerves The thoracic nerves are the twelve spinal nerves emerging from the thoracic vertebrae. Each thoracic nerve T1 -T12 originates from below each corresponding thoracic vertebra. Branches also exit the spine and go directly to the paravertebral ganglia of the autonomic nervous system where they are involved in the functions of organs and glands in the head, neck, thorax and abdomen. Anterior divisions: The intercostal nerves come from thoracic nerves T1-T11, and run between the ribs. At T2 and T3, further branches form the intercostobrachial nerve. The subcostal nerve comes from nerve T12, and runs below the twelfth rib."}, {"id": "wiki20220301en120_28651", "title": "Inferior cervical ganglion", "score": 0.014052540081128067, "content": "The inferior cervical ganglion is situated between the base of the transverse process of the last cervical vertebra and the neck of the first rib, on the medial side of the costocervical artery. Its form is irregular; it is larger in size than the middle cervical ganglion, and is frequently fused with the first thoracic ganglion, under which circumstances it is then called the \"stellate ganglion.\" Structure It is connected to the middle cervical ganglion by two or more cords, one of which forms a loop around the subclavian artery and supplies offsets to it. This loop is named the ansa subclavia (Vieussenii). The ganglion sends gray rami communicantes to the seventh and eighth cervical nerves."}, {"id": "pubmed23n0369_10397", "title": "The communicating branch of the 4th cervical nerve to the brachial plexus: the double constitution, anterior and posterior, of its fibers.", "score": 0.01360544217687075, "content": "Twenty-four adult cadavers (48 sides) were used to investigate the incidence of a branch arising from the ventral ramus of the fourth cervical nerve (C4) with the phrenic nerve and subsequently joining the brachial plexus. Six brachial plexuses with spinal cords and phrenic nerves were dissected under a surgical microscope to investigate localization of fibers contained in the C4 branch to the brachial plexus. The incidence of the C4 branch was 23% (11/48 sides). Branches from C4 to the brachial plexus divided into anterior and posterior divisions on four sides (4/6 sides). On two sides, the branch did not divide but consisted entirely of an anterior division (2/6 sides). In the brachial plexus, anterior division fibers of the C4 branch were intertwined with fibers from the anterior divisions of the ventral rami of the fifth and sixth cervical nerves. They then passed to the suprascapular nerve and the anterior division of the superior trunk (6/6 sides). On the other hand, posterior division fibers of the C4 branch were intertwined with fibers from the posterior divisions of the ventral rami of the fifth and sixth cervical nerves. They then passed to the suprascapular nerve (2/6 sides) and the posterior division of the superior trunk (4/6 sides). The anterior division of the C4 branch received fibers from the ventral rootlets of the entire fourth cervical segment, whereas the posterior division received fibers from the ventral rootlets of the caudal half of the fourth cervical segment only. The fact that the suprascapular nerve received fibers from both the anterior and posterior divisions of the C4 branch was considered to support our claim that the human suprascapular nerve belongs to both the anterior and posterior divisions of the brachial plexus."}, {"id": "wiki20220301en482_1010", "title": "Cervicoaxillary canal", "score": 0.013347873500545256, "content": "The cervicoaxillary canal is the passageway that extends between the neck and the upper extremities through which the long thoracic nerve and other structures pass. Its structure is defined by being posteriorly bordered by the scapula, anteriorly by the clavicle, and medially by the first rib. The long thoracic nerve traverses this passageway in addition to axillary blood vessels and the brachial plexus. This complex nerve network arises in the neck from the fifth, sixth, seventh and eighth cervical roots, C5, C6, C7 and C8, together with the first thoracic root, T1. It then enters the canal in the axilla. References Skeletal system Upper limb anatomy Shoulder Nerves"}, {"id": "wiki20220301en122_43687", "title": "Cervical enlargement", "score": 0.013319353836595216, "content": "The cervical enlargement corresponds with the attachments of the large nerves which supply the upper limbs. Located just above the brachial plexus, it extends from about the fifth cervical to the first thoracic vertebra, its maximum circumference (about 38 mm.) being on a level with the attachment of the sixth pair of cervical nerves. The reason behind the enlargement of the cervical region is because of the increased neural input and output to the upper limbs. An analogous region in the lower limbs occurs at the lumbar enlargement. References External links - \"Vertebral Canal and Spinal Cord: Regions of the Spinal Cord\" - \"Spinal Cord, Fetus, Posterior View\" Nerves of the upper limb"}, {"id": "wiki20220301en431_1693", "title": "Outline of the human nervous system", "score": 0.01224737556237949, "content": "Intermediate nerve Greater petrosal nerve Chorda tympani (also in trigeminal? redundancy?) Vestibulocochlear nerve Vestibular nerve Cochlear nerve Glossopharyngeal nerve Tympanic nerve Tympanic plexus Lesser petrosal nerve Vagus nerve Superior laryngeal nerve Recurrent laryngeal nerve Accessory nerve Hypoglossal nerve Spinal nerves Cervical nerves Suboccipital nerve Greater occipital nerve Third occipital nerve Cervical plexus Ansa cervicalis Lesser occipital nerve Great auricular nerve Transverse cervical nerve Supraclavicular nerves Phrenic nerve Brachial plexus Supraclavicular part Dorsal scapular nerve Long thoracic nerve Subclavian nerve Suprascapular nerve Subscapular nerves Lower subscapular nerve Upper subscapular nerve Thoracodorsal nerve Medial pectoral nerve Lateral pectoral nerve Infraclavicular part Musculocutaneous nerve Medial cutaneous nerve of arm Medial cutaneous nerve of forearm Median nerve Ulnar nerve Radial nerve Axillary nerve Thoracic nerves Lumbar nerves"}, {"id": "pubmed23n0029_2513", "title": "The vertebral level of origin of spinal nerves in the rat.", "score": 0.012068739341466614, "content": "Several dissections were performed to determine the level of spinal cord termination and the vertebral level at which the dorsal and ventral roots of spinal nerves C1-S4 emerged from the spinal cord in the rat. These levels of emergence were then compared to the level of exit from the vertebral canal. The dissections demonstrated that the effect of differential growth between spinal cord and vertebral column begins in the lower cervical region and becomes progressively more pronounced throughout thoracic and lumbar levels. The disparity between the vertebral level of emergence of spinal roots from the spinal cord and their level of exit via intervertebral foramina was found to be considerably larger than was previously reported by Greene ('68). It was further noted that the spinal cord terminated at the level of the intervertebral disc between the third and fourth lumbar vertebrae, not between the fourth and fifth lumbar vertebrae as reported by Greene ('68)."}, {"id": "wiki20220301en098_11854", "title": "Posterior thoracic nucleus", "score": 0.011271929824561404, "content": "The posterior thoracic nucleus, (Clarke's column, column of Clarke, dorsal nucleus, nucleus dorsalis of Clarke) is a group of interneurons found in the medial part of lamina VII, also known as the intermediate zone, of the spinal cord. It is mainly located from the cervical vertebra C7 to lumbar L3-L4 levels and is an important structure for proprioception of the lower limb. Anatomy It occupies the medial part of the base of the posterior grey column and appears on the transverse section as a well-defined oval area. It begins caudally at the level of the second or third lumbar nerve, and reaches its maximum size opposite the twelfth thoracic nerve. Above the level of the eight thoracic nerve its size diminishes, and the column ends opposite the last cervical or first thoracic nerve."}, {"id": "wiki20220301en058_53176", "title": "Intertransversarii", "score": 0.011028589880523609, "content": "There are seven pairs of these muscles, the first pair being between the atlas and axis, and the last pair between the seventh cervical and first thoracic vertebræ. Thoracic In the thoracic region they are present between the transverse processes of the lower three thoracic vertebrae, and between the transverse processes of the last thoracic and the first lumbar. These are called the thoracic intertransversarii and are supplied by the posterior rami of the spinal nerves. Lumbar In the lumbar region they are arranged in pairs, on either side of the vertebral column, one set occupying the entire interspace between the transverse processes of the lumbar vertebrae, are the lateral lumbar intertransversarii. the other set, the medial lumbar intertransversarii, passing from the accessory process of one vertebra to the mammillary of the vertebra below."}, {"id": "Anatomy_Gray_202", "title": "Anatomy_Gray", "score": 0.009978081619872665, "content": "Nomenclature of spinal nerves There are approximately 31 pairs of spinal nerves (Fig. 2.62), named according to their position with respect to associated vertebrae: eight cervical nerves—C1 to C8, twelve thoracic nerves—T1 to T12, five lumbar nerves—L1 to L5, five sacral nerves—S1 to S5, one coccygeal nerve—Co. The first cervical nerve (C1) emerges from the vertebral canal between the skull and vertebra CI (Fig. 2.63). Therefore cervical nerves C2 to C7 also emerge from the vertebral canal above their respective vertebrae. Because there are only seven cervical vertebrae, C8 emerges between vertebrae CVII and TI. As a consequence, all remaining spinal nerves, beginning with T1, emerge from the vertebral canal below their respective vertebrae."}, {"id": "pubmed23n0641_17830", "title": "Anatomical variations of the spinal accessory nerve and its relevance to level IIb lymph nodes.", "score": 0.009900990099009901, "content": "This study was conducted to identify anatomical variations of the spinal accessory nerve (SAN) in the upper neck, the landmark of the anterior and inferior border of level IIb, and to evaluate the nerve's effect on the border and the number of lymph nodes (LNs) in level IIb. Case series with planned data collection. A total of 181 neck dissections (NDs) were prospectively enrolled in this study. The relation between the SAN and adjacent structures (internal jugular vein [IJV], sternocleidomastoid muscle [SCM], cervical plexus) and the number of LNs in level IIb was investigated. The SAN crossed the IJV ventrally in 72 cases (39.8%) and dorsally in 104 cases (57.4%), and passed through the IJV in five cases (2.8%). The SAN ran along the inner surface of the SCM and sent branches to the SCM without penetration of the muscle in 83 cases (45.9%), whereas in 98 cases (54.1%) the nerve sent branches to the SCM by penetration. Cervical plexus contribution to the SAN was seen from C2 in 96 cases (53.1%), C2 and C3 in 69 cases (38.1%), and C3 in 16 cases (8.8%). The mean number of LNs of level IIa and level IIb was 6.5 and 8.2 in cases in which the SAN crossed the IJV ventrally, and 6.8 and 5.4 in dorsally crossing cases. LNs included in the neck level IIb in ventrally crossing SAN cases were significantly larger than the dorsally crossing cases (P &lt; 0.05). Our results may help to minimize the incidence of injuring the SAN in the upper neck during ND. Neck level IIb would contain more LNs if the course of the nerve leans toward the ventral side."}, {"id": "wiki20220301en071_51054", "title": "Longus colli muscle", "score": 0.00980392156862745, "content": "It is broad in the middle, narrow and pointed at either end, and consists of three portions, a superior oblique, an inferior oblique, and a vertical. The superior oblique portion arises from the anterior tubercles of the transverse processes of the third, fourth, and fifth cervical vertebrae and, ascending obliquely with a medial inclination, is inserted by a narrow tendon into the tubercle on the anterior arch of the atlas. The inferior oblique portion, the smallest part of the muscle, arises from the front of the bodies of the first two or three thoracic vertebrae; and, ascending obliquely in a lateral direction, is inserted into the anterior tubercles of the transverse processes of the fifth and sixth cervical vertebrae. The vertical portion arises, below, from the front of the bodies of the upper three thoracic and lower three cervical vertebrae, and is inserted into the front of the bodies of the second, third, and fourth cervical vertebrae."}, {"id": "pubmed23n0705_24851", "title": "Four cases of spinal accessory nerve passing through the fenestrated internal jugular vein.", "score": 0.00980392156862745, "content": "Neck dissection (ND) is an important technique for the treatment of cervical lymph node metastasis in patients with head and neck cancer. Since the introduction of functional ND (FND), various modifications have been made to reduce the adverse effects of radical ND. Recently, many investigators have documented cases of FND with preservation of the spinal accessory nerve (SAN) and/or the sternocleidomastoid muscle, which have contributed to improve the quality of life following ND. For this type of ND, special attention must be paid to identify the SAN and the internal jugular vein (IJV). We performed 123 NDs over 2 years at the Department of Otolaryngology, Head and Neck Surgery, Kobe University Hospital. We collected data of all patients who underwent NDs by retrospectively reviewing the relevant hospital medical records and operative notes. In 4 out of 123 NDs (3.3%), an anomaly of the SAN passing through the fenestrated IJV was observed. Although this anomaly is rare, head and neck surgeons should be aware of this anomalous relationship between the SAN and the IJV in order to avoid accidental injury to these structures during ND."}, {"id": "wiki20220301en047_40153", "title": "Vertebral artery", "score": 0.009714527546937518, "content": "The vertebral artery may be divided into four parts: The first (preforaminal) part runs upward and backward between the Longus colli and the Scalenus anterior. In front of it are the internal jugular and vertebral veins, and it is crossed by the inferior thyroid artery; the left vertebral is crossed by the thoracic duct also. Behind it are the transverse process of the seventh cervical vertebra, the sympathetic trunk and its inferior cervical ganglion The second (foraminal) part runs upward through the transverse foramina of the C6 to C2 vertebrae, and is surrounded by branches from the inferior cervical sympathetic ganglion and by a plexus of veins which unite to form the vertebral vein at the lower part of the neck. It is situated in front of the trunks of the cervical nerves, and pursues an almost vertical course as far as the transverse process of the axis."}, {"id": "wiki20220301en361_18057", "title": "Vertebral column", "score": 0.009708737864077669, "content": "Lateral surfaces The sides of the vertebral column are separated from the posterior surface by the articular processes in the cervical and thoracic regions and by the transverse processes in the lumbar region. In the thoracic region, the sides of the bodies of the vertebrae are marked in the back by the facets for articulation with the heads of the ribs. More posteriorly are the intervertebral foramina, formed by the juxtaposition of the vertebral notches, oval in shape, smallest in the cervical and upper part of the thoracic regions and gradually increasing in size to the last lumbar. They transmit the special spinal nerves and are situated between the transverse processes in the cervical region and in front of them, in the thoracic and lumbar regions."}, {"id": "article-34020_13", "title": "Anatomy, Head and Neck, Posterior Cervical Region -- Nerves", "score": 0.009646502364948966, "content": "The phrenic nerve can be found deep to the posterior triangle within the prevertebral fascia as it runs along the anterior surface of the anterior scalene muscle. The spinal nerves of the fifth cervical vertebra through the first thoracic vertebra form the roots and trunks of the brachial plexus, which can also appear within the prevertebral fascia between the anterior and middle scale muscles. [11]"}, {"id": "wiki20220301en071_51090", "title": "Semispinalis muscles", "score": 0.009615384615384616, "content": "The semispinalis cervicis (or semispinalis colli), arises by a series of tendinous and fleshy fibers from the transverse processes of the upper five or six thoracic vertebrae, and is inserted into the cervical spinous processes, from the axis to the fifth cervical vertebrae inclusive. The semispinalis cervicis is thicker than the semispinalis thoracis. The fasciculus connected with the axis is the largest, and is chiefly muscular in structure. The semispinalis thoracis (or semispinalis dorsi) muscle consists of thin, narrow, fleshy fasciculi, interposed between tendons of considerable length. It arises by a series of small tendons from the transverse processes of the sixth to the tenth thoracic vertebrae, and is inserted, by tendons, into the spinous processes of the upper four thoracic and lower two cervical vertebrae. The semispinalis muscles are innervated by the dorsal rami of the cervical spinal nerves. See also List of muscles of the human body Additional images"}, {"id": "article-34020_12", "title": "Anatomy, Head and Neck, Posterior Cervical Region -- Nerves", "score": 0.009615384615384616, "content": "The superficial branches of the cervical plexus that supply cutaneous sensation to the skin overlying the cervical region emerge from the posterior aspect of the sternocleidomastoid muscle near its midpoint, at a site known as the nerve point of the neck. The anterior rami of the spinal nerves pierce the investing fascia and split to form four cutaneous nerves that run deep to the platysma. The lesser occipital nerve forms from the second cervical spinal nerve and ascends posterior to the sternocleidomastoid to innervate the skin of the scalp and neck posterior to the ear. The greater auricular nerve forms from the second and third cervical spinal nerves and ascends on the anterior surface of the sternocleidomastoid to innervate the skin over the mastoid and parotid region inferior to the ear. The transverse cervical nerve forms from the second and third cervical spinal nerves and crosses horizontally over the sternocleidomastoid to innervate the anterior aspect of the neck. The supraclavicular nerve forms from the third and fourth cervical spinal nerves and descends across the surface of the investing fascia over the posterior triangle as it branches widely to innervate the skin over the clavicle and first two ribs. [10]"}, {"id": "Anatomy_Gray_2469", "title": "Anatomy_Gray", "score": 0.009576738908867137, "content": "Middle cervical ganglion. A second ganglion inferior to the superior cervical ganglion along the course of the sympathetic trunk (the middle cervical ganglion) is encountered at about the level of cervical vertebra CVI (Figs. 8.193 and 8.194). Branches from this ganglion pass to: cervical spinal nerves C5 and C6 through gray rami communicantes, and the heart as middle cardiac nerves. Inferior cervical ganglion. At the lower end of the cervical part of the sympathetic trunk is another ganglion (the inferior cervical ganglion), which becomes very large when it combines with the first thoracic ganglion and forms the cervicothoracic ganglion (stellate ganglion). The inferior cervical ganglion (Figs. 8.193 and 8.194) is anterior to the neck of rib I and the transverse process of cervical vertebra CVII, and posterior to the first part of the subclavian artery and the origin of the vertebral artery."}, {"id": "wiki20220301en038_2724", "title": "Cervical vertebrae", "score": 0.00952921157118548, "content": "Vertebra prominens The vertebra prominens, or C7, has a distinctive long and prominent spinous process, which is palpable from the skin surface. Sometimes, the seventh cervical vertebra is associated with an abnormal extra rib, known as a cervical rib, which develops from the anterior root of the transverse process. These ribs are usually small, but may occasionally compress blood vessels (such as the subclavian artery or subclavian vein) or nerves in the brachial plexus, causing pain, numbness, tingling, and weakness in the upper limb, a condition known as thoracic outlet syndrome. Very rarely, this rib occurs in a pair. The long spinous process of C7 is thick and nearly horizontal in direction. It is not bifurcated, and ends in a tubercle that the ligamentum nuchae attaches to. This process is not always the most prominent of the spinous processes, being found only about 70% of the time, C6 or T1 can sometimes be the most prominent."}, {"id": "wiki20220301en058_53173", "title": "Interspinales muscles", "score": 0.009523809523809525, "content": "The interspinales are short muscle fascicles, found in pairs between the spinous processes of the contiguous vertebrae, one on either side of the interspinal ligament. In the cervical region the cervical interspinales are most distinct, and consist of six pairs, the first being situated between the axis and third vertebra, and the last between the seventh cervical and the first thoracic. They are small narrow bundles, attached, above and below, to the apices of the spinous processes. In the thoracic region the thoracic interspinales are found between the first and second vertebrae, and sometimes between the second and third, and between the eleventh and twelfth. In the lumbar region there are four pairs of lumbar interspinales in the intervals between the five lumbar vertebrae. There is also occasionally one between the last thoracic and first lumbar, and one between the fifth lumbar and the sacrum."}, {"id": "Neurology_Adams_987", "title": "Neurology_Adams", "score": 0.009492318420046338, "content": "the neck, third cervical; the epaulet area, fourth cervical; the deltoid area, fifth cervical; the radial forearm and thumb, sixth cervical; the index and middle fingers, seventh cervical; the little finger and ulnar border of hand and forearm, eighth cervical–first thoracic; the nipple, fourth to fifth thoracic; the umbilicus, tenth thoracic; the groin, first lumbar; the medial side of the knee, third lumbar; the medial malleolus, the fourth lumbar; the great toe, fifth lumbar; the little toe, first sacral; the back of the thigh and lateral foot, second sacral; and the genitoanal zones, third, fourth, and fifth sacral segments."}, {"id": "wiki20220301en122_50432", "title": "Cervical ganglia", "score": 0.009459924320605436, "content": "The cervical ganglia are paravertebral ganglia of the sympathetic nervous system. Preganglionic nerves from the thoracic spinal cord enter into the cervical ganglions and synapse with its postganglionic fibers or nerves. The cervical ganglion has three paravertebral ganglia: superior cervical ganglion (largest) - adjacent to C2 & C3; postganglionic axon projects to target: (heart, head, neck) via \"hitchhiking\" on the carotid arteries middle cervical ganglion (smallest) - adjacent to C6; target: heart, neck inferior cervical ganglion. The inferior ganglion may be fused with the first thoracic ganglion to form a single structure, the stellate ganglion. - adjacent to C7; target: heart, lower neck, arm, posterior cranial arteries Nerves emerging from cervical sympathetic ganglia contribute to the cardiac plexus, among other things. Unlike all other ganglia, the medial branches of the cervical ganglia are 95% postganglionic axons. Additional images References"}, {"id": "wiki20220301en470_3868", "title": "Vertebra", "score": 0.009433962264150943, "content": "The spinous process on C7 is distinctively long and gives the name vertebra prominens to this vertebra. Also a cervical rib can develop from C7 as an anatomical variation. The term cervicothoracic is often used to refer to the cervical and thoracic vertebrae together, and sometimes also their surrounding areas. Thoracic vertebrae The twelve thoracic vertebrae and their transverse processes have surfaces that articulate with the ribs. Some rotation can occur between the thoracic vertebrae, but their connection with the rib cage prevents much flexion or other movement. They may also be known as \"dorsal vertebrae\" in the human context. The vertebral bodies are roughly heart-shaped and are about as wide anterio-posteriorly as they are in the transverse dimension. Vertebral foramina are roughly circular in shape. The top surface of the first thoracic vertebra has a hook-shaped uncinate process, just like the cervical vertebrae."}, {"id": "wiki20220301en198_26899", "title": "Cervical spinal nerve 3", "score": 0.009433962264150943, "content": "The cervical spinal nerve 3 (C3) is a spinal nerve of the cervical segment. It originates from the spinal column from above the cervical vertebra 3 (C3). References Spinal nerves"}, {"id": "wiki20220301en252_28738", "title": "Spinal cord", "score": 0.009345794392523364, "content": "Within the Central Nervous System (CNS), nerve cell bodies are generally organized into functional clusters, called nuclei. Axons within the CNS are grouped into tracts. There are 31 spinal cord nerve segments in a human spinal cord: 8 cervical segments forming 8 pairs of cervical nerves (C1 spinal nerves exit the spinal column between the foramen magnum and the C1 vertebra; C2 nerves exit between the posterior arch of the C1 vertebra and the lamina of C2; C3–C8 spinal nerves pass through the IVF above their corresponding cervical vertebrae, with the exception of the C8 pair which exit between the C7 and T1 vertebrae) 12 thoracic segments forming 12 pairs of thoracic nerves 5 lumbar segments forming 5 pairs of lumbar nerves 5 sacral segments forming 5 pairs of sacral nerves 1 coccygeal segment"}, {"id": "wiki20220301en033_40391", "title": "Neuromere", "score": 0.00933641975308642, "content": "Spinal Cord Anatomy Spinal segments are the part of the spinal cord, from which ventral and dorsal roots exit to form a specific pair of spinal nerves. The spinal chord is not segmented itself, it is only made into segments by the spinal nerves as they leave. 31 deferent segments exists in a human spinal cord: 8 cervical segments Cervical nerves exit above C1 segment and below C1-C7. Note that this creates 8 nerves and segments corresponding to only 7 cervical vertebra. 12 thoracic segments Nerves exit below T1-T12. 5 lumbar segments Nerves exit below L1-L5. 5 sacral segments Nerves exit below S1-S5. 1 coccygeal segment Originally, during development there are two segments S1 and S2 which fuse. The nerves in this case exit at the coccyx. In more detail"}, {"id": "wiki20220301en038_6013", "title": "Thoracic vertebrae", "score": 0.009259259259259259, "content": "Individual thoracic vertebrae First thoracic vertebra (T1) The first thoracic vertebra has, on either side of the body, an entire articular facet for the head of the first rib, and a demi-facet for the upper half of the head of the second rib. The body is like that of a cervical vertebra, being broad, concave, and lipped on either side. The superior articular surfaces are directed upward and backward; the spinous process is thick, long, and almost horizontal. The transverse processes are long, and the upper vertebral notches are deeper than those of the other thoracic vertebrae. The thoracic spinal nerve 1 (T1) passes out underneath it. Second thoracic vertebra (T2) The thoracic spinal nerve 2 (T2) passes out underneath it. The second thoracic vertebra is larger than the first thoracic vertebra Third thoracic vertebra (T3) The thoracic spinal nerve 3 (T3) passes out underneath it."}, {"id": "pubmed23n0243_4399", "title": "The eleventh nerve in radical neck surgery.", "score": 0.009259259259259259, "content": "The greatest morbidity associated with the radical neck dissection has been the \"shoulder syndrome\" due to the sacrifice of the spinal accessory nerve. Despite the fact that the XIth nerve can be spared by a careful dissection of the postcervical triangle, to do so remains a controversial issue. The 125 radical neck dissections performed at the University of Wisconsin Clinical Science Center from 1970 through 1975 were carefully evaluated to determine the incidence of recurrent tumor in the operated-on neck. In 60 cases the spinal accessory nerve was resected with the neck specimen, and in 65 cases the nerve was preserved. A second study was then undertaken involving 245 neck dissections performed from 1975-1978 in the Wisconsin Head and neck Cancer Control Network Hospitals. In 69 instances the spinal accessory nerve was spared. The total number of neck dissections (370 cases) from both studies were analyzed. The overall rate for recurrent tumor in the neck with the classical neck dissection was 12%. When the spinal accessory nerve was spared, the recurrent rate was 6%. On the basis of these observations we propose that the classical neck dissection can be modified to preserve the spinal accessory nerve without jeopardizing the chances for a cure in elective neck dissections and selected therapeutic neck dissections."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[233, 600]], "word_ranges": [[39, 92]], "text": "The specific criterion for the diagnosis of collagenous colitis is the additional presence of an irregular band of collagen just below the surface epithelium of the colonic mucosa in continuity with the basement membrane, visible with trichrome staining (stains type I collagen fibers), which traps superficial capillaries producing lesions in the surface epithelium."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Everything we are told in this case is typical of COLLAGENOUS COLITIS. In principle, the clinical and endoscopic findings may leave us a little as at the beginning, but histology is little more than the definition of this pathology. The specific criterion for the diagnosis of collagenous colitis is the additional presence of an irregular band of collagen just below the surface epithelium of the colonic mucosa in continuity with the basement membrane, visible with trichrome staining (stains type I collagen fibers), which traps superficial capillaries producing lesions in the surface epithelium. In addition, it is accompanied by a chronic inflammatory infiltrate in the lamina propria, composed mainly of lymphocytes, plasma cells and eosinophils.", "full_answer_no_ref": "Everything we are told in this case is typical of COLLAGENOUS COLITIS. In principle, the clinical and endoscopic findings may leave us a little as at the beginning, but histology is little more than the definition of this pathology. The specific criterion for the diagnosis of collagenous colitis is the additional presence of an irregular band of collagen just below the surface epithelium of the colonic mucosa in continuity with the basement membrane, visible with trichrome staining (stains type I collagen fibers), which traps superficial capillaries producing lesions in the surface epithelium. In addition, it is accompanied by a chronic inflammatory infiltrate in the lamina propria, composed mainly of lymphocytes, plasma cells and eosinophils.", "full_question": "A 59-year-old woman presents with chronic watery diarrhea of 4 months' duration. On endoscopy, the mucosa showed no relevant features. In particular, no ulcers or friable areas were observed. A biopsy of the transverse colon was performed. Histopathology revealed a thickened area below the superficial lining epithelium, which was more evident by Masson's trichrome technique and involved epithelial atrophy and denudation. There was also a clear increase in intraepithelial lymphocyte density. The diagnosis of the intestinal lesion is?", "id": 280, "lang": "en", "options": {"1": "Chronic ulcerative colitis.", "2": "Pseudomembranous colitis.", "3": "Collagenous colitis.", "4": "Fibrosing Crohn's disease.", "5": NaN}, "question_id_specific": 32, "type": "PATHOLOGICAL ANATOMY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0313_13912", "title": "The role of mucosal biopsy in the diagnosis of chronic diarrhea: value of multiple biopsies when colonoscopic finding is normal or nonspecific.", "score": 0.01711402033204125, "content": "There are controversies about taking routine mucosal biopsy when the gross colonoscopic finding is normal. This study was conducted to determine the frequency of clinically important histological abnormalities, prospectively, in chronic diarrhea patients with grossly normal or nonspecific colonoscopic findings. One hundred and eighteen patients suffering from nonbloody diarrhea with average frequency of more than two times a day for more than 4 weeks were included. Multiple biopsies (cecum, ascending colon, mid-transverse colon, descending colon, sigmoid colon and rectum) were taken during colonoscopic examinations and each biopsy specimen was reviewed by one pathologist after H&amp;E and Masson-trichrome staining. Clinically significant abnormalities (2 collagenous colitis, 1 lymphocytic colitis, 1 eosinophilic enterocolitis, 1 ulcerative colitis and 4 melanosis coli) were observed in 9 patients (7.6%). Sixteen cases (13.6%) of borderline histological abnormalities were observed (8 cases of possible collagenous colitis and 8 cases showing some features of lymphocytic colitis). Ninety two cases (78.8%) showed nonspecific inflammation only. Clinically important histological lesions can exist in significant percentage in spite of normal or nonspecific colonoscopic findings, which can justify routine mucosal biopsy in the evaluation of chronic diarrhea patients. The clinical significance of borderline histological abnormalities needs to be determined by careful follow-up studies."}, {"id": "wiki20220301en070_39278", "title": "Microscopic colitis", "score": 0.01709090909090909, "content": "Diagnosis Colonoscopic appearances are normal or near normal. As the changes are often patchy, an examination limited to the rectum may miss cases of microscopic colitis, and so a full colonoscopy is necessary. Multiple colonic biopsies are taken in order to make the diagnosis. Histological features of colonic biopsies indicating microscopic colitis are: greater than 20 intraepithelial lymphocytes per 100 epithelial cells and, additionally, 10-20 μm of a thickened subepithelial collagen band in collagenous colitis. Inflammation of the lamina propria, with mainly mononuclear cells, may be observed in collagenous colitis."}, {"id": "pubmed23n0600_20905", "title": "Lymphocytic, collagenous and other microscopic colitides: pathology and the relationship with idiopathic inflammatory bowel diseases.", "score": 0.016931216931216932, "content": "Collagenous colitis and lymphocytic colitis are the two major conditions characterized by chronic watery diarrhoea, without endoscopic or radiological lesions, but with histological abnormalities and therefore considered as \"microscopic colitis\". The histology of colonic biopsies shows inflammation of the mucosa, and either thickening of the subepithelial collagen band or an increase of lymphocytes in the surface epithelium. Different variant forms have been reported under separate names. These are probably not specific entities. The incidence of microscopic colitis is slightly less than the incidence of chronic idiopathic inflammatory bowel diseases (IBD). Microscopic colitis and IBD are clearly different entities. The relation between both entities is weak but double. Biopsy samples from patients with IBD may mimic the features of lymphocytic or collagenous colitis, both in the initial onset and during follow-up. In the large majority of these cases, endoscopy shows or has shown mucosal lesions. In rare cases, however, a double diagnosis was made. Certain patients, usually of older age, presented first with a microscopic, usually collagenous colitis and developed subsequently genuine ulcerative colitis."}, {"id": "pubmed23n0598_13529", "title": "[Microscopic colitis - review].", "score": 0.016657638136511378, "content": "Microscopic colitis (MC) is an encompassing term for two diseases; collagenous colitis and lymphocytic colitis. The colon appears normal by colonoscopy and a diagnosis is only obtained with a biopsy. The histopathology of collagenous colitis is mainly characterized by a thickening of the subepithelial basement membrane of the colonic mucosa with a band of collagen. Lymphocytic colitis is mainly characterized by an intraepithelial lymphocytosis without the collagen thickening. Even though the two diseases have a distinctive pathology their clinical symptoms are characterized by chronic watery diarrhea without bleeding. Microscopic colitis is thought to cause about 4-13% of all chronic diarrhea but their relative frequency is much higher among older people. The mean annual incidence for collagenous and lymphocytic colitis has been increasing. Steroids are the most effective treatment for microscopic colitis and budesonide is the most studied and effective therapy for MC. The aim of this paper is to give a review of two relatively new diseases which are among the most common cause of chronic diarrhea, especially among older people."}, {"id": "pubmed23n0543_8057", "title": "Histopathological diagnosis of microscopic colitis.", "score": 0.01635260300223571, "content": "A typical symptom of microscopic colitis (MC) is chronic watery diarrhea with normal endoscopic findings and characteristic inflammatory changes in histopathology. Treatment of the disease is mainly empiric. MC has two main subtypes: lymphocytic colitis and collagenous colitis. There are also untypical histopathological forms of MC: MC with giant cells, MC not otherwise specified (NOS) and cryptal lymphocytic coloproctitis. Some other histopathological changes in MC have been observed, especially Paneth cell hyperplasia or epithelial degeneration. Eosinophilic colitis, acute colitis, amyloidosis, ulcerative colitis and Crohn's disease should be taken into consideration in differential diagnosis. The most reliable biopsy material for histopathological examination are samples obtained from transverse colon. Some studies proved that treatment of MC makes it possible to reduce not only clinical, but also histopathological, manifestations."}, {"id": "wiki20220301en070_39279", "title": "Microscopic colitis", "score": 0.0163265306122449, "content": "Pathology Microscopic colitis is characterized by an increase in inflammatory cells, particularly lymphocytes, in colonic biopsies with an otherwise normal appearance and architecture of the colon. Inflammatory cells are increased both in the surface epithelium (\"intraepithelial lymphocytes\") and in the lamina propria. The key feature is more than 20 intra-epithelial lymphocytes per 100 epithelial cells. These are the principal features of lymphocytic colitis. An additional distinguishing feature of collagenous colitis is a thickened subepithelial collagen layer, which may be up to 30 micrometres thick, that occurs in addition to the features found in lymphocytic colitis. The fact that the two types of microscopic colitis share many features including epidemiology, risk factors and, response to therapy has led to the suggestion that they are actually subtypes of the same disease."}, {"id": "pubmed23n0574_19509", "title": "[Microscopic colitis: pathogenesis].", "score": 0.016202498698594484, "content": "Microscopic colitis (MC) is a chronic inflammatory process observed in colon biopsies of patients with chronic aqueous diarrhea. It is called microscopic because diagnosis is determined by histological studies since the microscopic characteristics of the colon endoscopy are normal. Two patterns exist: Lymphocytic Microscopic Colitis and Collagenous Microscopic Colitis. Etiology is unknown, and the proposed pathogenic mechanisms indicate an immunological phenomenon. Based on this, the authors of this study hypothesize that lymphocytic infiltration of the lamina propria could be related to cytotoxic lymphocytes CD8 as causative agents of colon tissue damage. Prove hypothesis of immunological pathogenesis of MC. APPARATUS AND METHODS: Thirty eight (38) patients with diagnosed MC were recruited for the last four years in the Pathology Laboratory at Ricardo Palma University. Twenty two (22) colon biopsies with the most severe histological lesions were selected. These biopsies were obtained from 17 patients: 5 patients had 2 biopsies in 2 colonoscopy sessions. Biopsies were fixed in neutral formaldehyde, processed through the paraffin inclusion method, and stained with hematoxiline-eosine and Masson trichromic to distinguish collagenous tissue. Immunohistochemistry was conducted in 4- or 5-micron-thick histological sections processed through the immunoperoxidase method. Nineteen (19) biopsies corresponded to Lymphocytic MC and 3 to Collagenous MC. Lymphocytic MC showed intraepithelial lymphocytosis, dystrophic epithelial damage in the areas of lymphocytic infiltration, lamina propria inflammation with lymphocytes and plasma cells, and normal basement membrane. Collagenous MC showed thickened basement membrane due to the presence of a collagenous band, mild to moderate intraepithelial lymphocytosis, vacuolization,and frequent detachment of protective epithelium. Twenty two (22) biopsies were positive in the immunohistochemical studies."}, {"id": "wiki20220301en003_40015", "title": "Ulcerative colitis", "score": 0.0161907190209077, "content": "The simple clinical colitis activity index was created in 1998 and is used to assess the severity of symptoms. Endoscopic The best test for diagnosis of ulcerative colitis remains endoscopy, which is examination of the internal surface of the bowel using a flexible camera. Initially, a flexible sigmoidoscopy may be completed to establish the diagnosis. The physician may elect to limit the extent of the initial exam if severe colitis is encountered to minimize the risk of perforation of the colon. However, a complete colonoscopy with entry into the terminal ileum should be performed to rule out Crohn's disease, and assess extent and severity of disease. Endoscopic findings in ulcerative colitis include: erythema (redness of the mucosa), friability of the mucosa, superficial ulceration, and loss of the vascular appearance of the colon. When present, ulcerations may be confluent. Pseudopolyps may be observed."}, {"id": "pubmed23n0833_17920", "title": "[Evolution of ideas on microscopic colitis].", "score": 0.01595873786407767, "content": "The literature review gives the present-day views of the definition, etiology, pathogenesis, diagnosis, and treatment of microscopic colitis (MC). In the present view, MC is an inflammatory bowel disease of unknown etiology, which is characterized by chronic watery diarrhea, no macroscopic signs of large bowel involvement in the presence of specific pathomorphological changes. There are two major forms of MC, which are similar in its clinical picture, yet, heterogeneous in histological criteria: collagenous colitis (CC) and lymphocytic colitis (LC). As of now, the prevalence of MC is about 100 cases per 100,000 population, which is similar with that in other inflammatory bowel diseases, such as ulcerative colitis and Crohn's disease. MC generally prevails in women aged over 50 years. The etiology and pathogenesis of MC have not fully investigated. Watery diarrhea is as a predominant pathognomonic symptom in all the patients with MC. The major histological criterion for the diagnosis of CC is subepithelial collagen lining thickening (more than 10 pm) and that for LC is higher intraepithelial lymphocyte counts (more than 20 intraepithelial lymphocytes/100 epitheliocytes). The topical glucocorticosteroid budesonide is currently the only agent, the efficacy of which has been proven in both inducing and maintaining remission in patients with MC in many clinical trials."}, {"id": "pubmed23n0302_11357", "title": "[Are lymphocytic and collagenous colitis two forms of a single disease? Arguments taken from a biopsy quantitative study].", "score": 0.014759953776657212, "content": "Collagenous colitis and lymphocytic colitis are defined by a clinicopathologic syndrome with chronic watery diarrhea, microscopic lesions of colonic biopsies, and normal barium enema and colonoscopy. A histopathological study was performed on multiple colorectal biopsies to compare 12 cases of collagenous colitis (defined by a subepithelial collagen thicker than 10 microns) and 7 cases of lymphocytic colitis (defined by a number of intraepithelial lymphocytes more than 20 per 100 epithelial cells at least in one biopsied site). The study included a semiquantitative analysis of inflammatory infiltrate in the lamina propria, crypts distortion and epithelial detachment. The number of intraepithelial lymphocytes per 100 epithelial cells was determined in surface epithelium and crypts. The subepithelial collagen thickening was studied by computerised morphometry. The intraepithelial lymphocytes, villous atrophy and thickness of the subepithelial collagen were also determined in gastric and duodenal biopsies. In collagenous colitis, the subepithelial collagenous thickness ranged from 10 to 40 microns in the colon (median 20.99 microns). In 4 cases of collagenous colitis, no thickening of the collagen plate was seen in the rectum. We found constant epithelial detachment and mucosal distortion. In lymphocytic colitis, the thickness of the subepithelial collagen ranged from 6 to 10 microns in 4 cases and was less than 6 microns in 3 cases (median 6.24 microns). The median number of intraepithelial lymphocytes in surface epithelium was 22.35 (range 18.2 to 40) in lymphocytic colitis versus 12.22 (range 4.6 to 24.4) in collagenous colitis. In conclusion, we observed an overlap of both the collagenous plate thickness and the number of intraepithelial lymphocytes in collagenous colitis and lymphocytic colitis. This result favours a unified histogenesis for these two entities."}, {"id": "wiki20220301en126_13995", "title": "Lymphocytic colitis", "score": 0.014412216499503028, "content": "Lymphocytic colitis is a subtype of microscopic colitis, a condition characterized by chronic non-bloody watery diarrhea. Causes No definite cause has been determined. The peak incidence of lymphocytic colitis is in persons over age 50; the disease affects women and men equally. Some reports have implicated long-term usage of NSAIDs, proton pump inhibitors, and selective serotonin reuptake inhibitors, and other drugs. Associations with other autoimmune disorders suggests that overactive immune responses occur. Diagnosis The colonoscopy is normal but histology of the mucosal biopsy reveals an accumulation of lymphocytes in the colonic epithelium and connective tissue (lamina propria). Collagenous colitis shares this feature but additionally shows a distinctive thickening of the subepithelial collagen table."}, {"id": "pubmed23n0697_7483", "title": "[Microscopic colitis: histopathological review with a clinicopathological correlation].", "score": 0.014272166903745852, "content": "Microscopic colitis is a clinicopathological entity which, in addition to typical symptoms such as watery diarrhea, is characterized by its specific histopathology. Since colonoscopy yields normal findings, microscopic colitis belongs in a histological domain. The term encompasses two forms: lymphocytic and collagenous colitis. Histologically, lymphocytic colitis shows an increase in intraepithelial lymphocytes of more than 20 lymphocytes per 100 surface colonocytes, while collagenous colitis is characterized by a thickened subepithelial collagen layer of more than 10 µm. Specific stains help in the quantification of both. Since microscopic colitis does not always affect the entire colon and the number of intraepithelial lymphocytes varies physiologically, obtaining stepwise biopsies of the colon (with information on location where possible) is recommended. A thickened collagen layer is relatively specific for collagenous colitis, whereas intraepithelial lymphocytosis is also found in other diseases. Therefore, to make a correct diagnosis, it is important to correlate histological findings with clinical symptoms, including the main symptom of watery diarrhea."}, {"id": "wiki20220301en034_9032", "title": "Colitis", "score": 0.014192835592572133, "content": "An important investigation in the assessment of colitis is biopsy. A very small piece of tissue (usually about 2mm) is removed from the bowel mucosa during endoscopy and examined under the microscope by a histopathologist. It can provide important information regarding the cause of the disease and the extent of bowel damage. Types There are many types of colitis. They are usually classified by the cause. Types of colitis include: Autoimmune Inflammatory bowel disease (IBD) – a group of chronic colitides. Ulcerative colitis (UC) – a chronic colitis that affects the large intestine. Crohn's disease (CD) – another type of IBD that often leads to colitis. Unknown Microscopic colitis – a colitis diagnosed by microscopic examination of colonic tissue; macroscopically (\"to the eye\") it appears normal. Lymphocytic colitis Collagenous colitis Treatment-caused Diversion colitis Chemical colitis Chemotherapy-induced colitis Radiation colitis Checkpoint inhibitor induced colitis"}, {"id": "wiki20220301en605_24721", "title": "Segmental colitis associated with diverticulosis", "score": 0.01411988911988912, "content": "Types There are four types of SCAD, based on endoscopic appearance. Pattern A is characterized by involvement of crescentic folds and is the most common type of SCAD (52%). Pattern B has an appearance similar to mild-to moderate ulcerative colitis (30.40%), whereas pattern C appears similar to Crohn's disease (10.90%). Pattern D is the least common, and appears similar to severe ulcerative colitis (6.50%). Diagnosis SCAD is diagnosed via colonoscopy, often incidentally during examination for unrelated concerns. Colonoscopy shows erythema of the colonic mucosa, which may be characterized by friability and exudate. The descending and sigmoid colon are typically involved. Biopsies of the affected area and the unaffected rectum confirm the diagnosis. Biopsies of SCAD show evidence of chronic inflammation. Rectal biopsies show normal mucosa."}, {"id": "wiki20220301en003_40016", "title": "Ulcerative colitis", "score": 0.01409470277635837, "content": "Ulcerative colitis is usually continuous from the rectum, with the rectum almost universally being involved. Perianal disease is rare. The degree of involvement endoscopically ranges from proctitis (rectal inflammation) to left sided colitis (extending to descending colon), to extensive colitis (extending proximal to descending colon). Histologic Biopsies of the mucosa are taken during endoscopy to confirm the diagnosis of UC and differentiate it from Crohn's disease, which is managed differently clinically. Histologic findings in ulcerative colitis includes: distortion of crypt architecture, crypt abscesses, and inflammatory cells in the mucosa (lymphocytes, plasma cells, and granulocytes). Unlike the transmural inflammation seen in Crohn's disease, the inflammation of ulcerative colitis is limited to the mucosa."}, {"id": "wiki20220301en070_39276", "title": "Microscopic colitis", "score": 0.01399703374119392, "content": "Microscopic colitis refers to two related medical conditions which cause diarrhea: collagenous colitis and lymphocytic colitis. Both conditions are characterized by the presence of chronic non-bloody watery diarrhea, normal appearances on colonoscopy and characteristic histopathology findings of inflammatory cells. Signs and symptoms The main symptom is persistent non-bloody watery diarrhea, which may be profuse. People may also experience abdominal pain, fecal incontinence, and unintentional weight loss. Microscopic colitis is the diagnosis in around 10% of cases investigated for chronic non-bloody diarrhea."}, {"id": "pubmed23n0811_11290", "title": "Histology of microscopic colitis-review with a practical approach for pathologists.", "score": 0.013902282224365157, "content": "Microscopic colitis has emerged as a major cause of chronic watery non-bloody diarrhoea, particularly in elderly females. The term is used as an umbrella term to categorize a subgroup of colitides with distinct clinicopathological phenotypes and no significant endoscopic abnormalities. Lymphocytic colitis is defined by an increased number of surface intraepithelial lymphocytes, and collagenous colitis by a thickened collagen band underneath the surface epithelium. There is increased inflammation in the lamina propria, but only little or no crypt architectural distortion. Incomplete and variant forms showing less characteristic features have been reported under different names. The differential diagnosis mainly includes resolving infectious colitis and changes related to the intake of drugs such as non-steroidal anti-inflammatory drugs. Substantial clinical and histological overlap between lymphocytic and collagenous colitis has been described, raising the suspicion that the conditions are two histological manifestations of the same entity, possibly representing different manifestations during the disease course or different stages of disease development. In this review, we provide a practical approach for pathologists, with a focus on diagnostic criteria and differential diagnosis, and discuss recent insights into the pathogenesis of disease and the relationship with classic chronic inflammatory bowel disease, i.e. Crohn's disease and ulcerative colitis. "}, {"id": "pubmed23n0357_1053", "title": "Collagenous colitis and lymphocytic colitis: clinical and endoscopic findings.", "score": 0.013670776552022496, "content": "collagenous colitis (CC) and lymphocytic colitis (LC) are two entities of unknown cause, characterized by chronic watery diarrhea, grossly normal-appearing colonic mucosa and abnormal histopathological findings in colonic biopsies. The clinical features of the disease are based mainly on case reports or small uncontrolled series. Although normal colonoscopic findings are, as a rule, part of the diagnosis of CC, several cases of macroscopic colitis associated with CC have been reported, and the spectrum of endoscopic mucosal changes has not been described in large series. we present a retrospective study of all patients who underwent total colonoscopy and mucosal biopsy in our Endoscopy Unit between 1991 and 1997. Clinical and endoscopic findings in patients diagnosed as having CC or LC were recorded. of 676 patients studied, 398 suffered from chronic diarrhea. Collagenous colitis was diagnosed in 22 patients and LC in 10. Eleven per cent of the patients with CC and 20% of those with LC did not have diarrhea. Macroscopic colitis was observed in 6 out of 22 patients with CC (27%) and in 4 out of 10 with LC (40%). Macroscopic lesions included edema, erythema, abnormal vascular pattern, superficial erosions or ulcerations and hemorrhagic lacerations. In this series 7.03% of the patients with chronic diarrhea were diagnosed as having CC or LC. collagenous colitis and LC are two entities that should be considered in the differential diagnosis of chronic diarrhea. Total colonoscopy with multiple biopsies that include the right colon are mandatory. The presence of macroscopic lesions on endoscopy does not rule out a diagnosis of either entity. We identified patients who fulfilled the histopathological criteria for CC or LC but who did not have diarrhea."}, {"id": "wiki20220301en023_54770", "title": "Inflammatory bowel disease", "score": 0.013329858137543284, "content": "No disease specific markers are currently known in the blood, enabling the reliable separation of Crohn's disease and ulcerative colitis patients. The way doctors can tell the difference between Crohn's disease and UC is the location and nature of the inflammatory changes. Crohn's can affect any part of the gastrointestinal tract, from mouth to anus (skip lesions), although a majority of the cases start in the terminal ileum. Ulcerative colitis, in contrast, is restricted to the colon and the rectum. Microscopically, ulcerative colitis is restricted to the mucosa (epithelial lining of the gut), while Crohn's disease affects the full thickness of the bowel wall (\"transmural lesions\"). Lastly, Crohn's disease and ulcerative colitis present with extra-intestinal manifestations (such as liver problems, arthritis, skin manifestations and eye problems) in different proportions."}, {"id": "wiki20220301en003_39991", "title": "Ulcerative colitis", "score": 0.013211828429219733, "content": "The clinical presentation of ulcerative colitis depends on the extent of the disease process. Up to 15% of individuals may have severe disease upon initial onset of symptoms. A substantial proportion (up to 45%) of people with a history of UC without any ongoing symptoms (clinical remission) have objective evidence of ongoing inflammation. Ulcerative colitis is associated with a generalized inflammatory process that can affect many parts of the body. Sometimes, these associated extra-intestinal symptoms are the initial signs of the disease. Extent of involvement In contrast to Crohn's disease, which can affect areas of the gastrointestinal tract outside of the colon, ulcerative colitis is usually confined to the colon. Inflammation in ulcerative colitis is usually continuous, typically involving the rectum, with involvement extending proximally (to sigmoid colon, ascending colon, etc). In contrast, inflammation with Crohn's disease is often patchy, with so-called \"skip lesions.\""}, {"id": "pubmed23n0538_3047", "title": "Microscopic colitis in routine colonoscopies.", "score": 0.012933285134755192, "content": "Lymphocytic colitis (LC) and collagenous colitis (CC), both known as microscopic colitis (MC), are uncommon entities with increasing incidence as more clinicians take biopsies from macroscopically normal colons and as pathologists use more rigorous diagnostic criteria to be confident of the diagnosis. Information on the incidence of this type of colitis is limited and based on reported cases. The purpose of this work is to estimate the incidence of LC and CC in reviewed routine colonoscopies. We reviewed 2815 colonoscopies performed at a tertiary referral center with an open-access service using restricted histological criteria in order to establish the frequency rate of LC and CC in routine colonoscopic biopsy material. Cases suspicious for MC were stained with Masson's trichrome or Congo red stain and immunohistochemically for lymphocytes, where appropriate. Review of routine colonoscopic biopsies showed that MC is underreported in our colonoscopic material. Incidence rates of LC and CC (0.9 and 0.4, respectively) were based on morphological assessment of colonoscopic biopsies using stringent criteria together with clinical data and after differentiation with other lesions which can mimic MC. The 10.2% rate of this type of colitis in patients with chronic watery diarrhea indicates the necessity to consider these lesions in older individuals with diarrhea and normal endoscopical colonic mucosa."}, {"id": "pubmed23n0648_22118", "title": "Microscopic colitis in patients presenting with chronic diarrhea.", "score": 0.012743506493506494, "content": "To investigate the prevalence of microscopic colitis among patients presenting with chronic watery diarrhea. Colonic biopsies from 400 patients presenting with chronic watery diarrhea and other symptoms pertaining to lower gastrointestinal tract were studied. After a detailed clinical history and thorough physical examination full length colonoscopy was done using flexible colonoscope. Colonic biopsies were taken from abnormal and normal areas. Three to five micron thick sections were cut and stained with hematoxylin and eosin and Masson's trichrome stain to highlight sub epithelial collagen. Fifteen out of 400 (3.7%) colonic biopsies from patients presenting with chronic diarrhea had evidence of microscopic colitis. Five out of fifteen biopsies (33%) were diagnosed as collagenous colitis, 10 biopsies (67%) had evidence of lymphocytic colitis; 14/400(3.5%) histologically normal biopsies were taken as controls to compare various demographic and risk factors. Ten out of 15 patients (67%) were clinically diagnosed as irritable bowel syndrome. In the remaining five an infective etiology was suspected. On colonoscopy12/15 (80%) had no abnormality and 3/15 (20%) had mild hyperemia. A possibility of microscopic colitis should be considered while examining colonoscopic biopsy of a patient with chronic watery diarrhea and normal colonoscopy to avoid the misdiagnosis that may affect the treatment of patients."}, {"id": "pubmed23n0917_8957", "title": "Endoscopic findings and colonic perforation in microscopic colitis: A systematic review.", "score": 0.012727272727272728, "content": "Microscopic colitis (MC) is a clinical syndrome of severe watery diarrhea with few or no endoscopic abnormalities. The incidence of MC is reported similar to that of other inflammatory bowel diseases. The need for histological confirmation of MC frequently guides reimbursement health policies. With the advent of high-definition (HD) coloscopes, the incidence of reporting distinct endoscopic findings in MC has risen. This has the potential to improve timely diagnosis and cost-effective MC management and diminish the workload and costs of busy modern endoscopy units. Publications on distinct endoscopic findings in MC available until March 31st, 2017 were searched systematically (electronic and manual) in PubMed database. The following search terms/descriptors were used: collagenous colitis (CC) OR lymphocytic colitis (LC) AND endoscopy, colonoscopy, findings, macroscopic, erythema, mucosa, vasculature, scars, lacerations, fractures. An additional search for MC AND perforation was made. Eighty (n=80) articles, predominantly single case reports (n=49), were found. Overall, 1582 (1159F; 61.6±14.1 years) patients (pts) with MC and endoscopic findings were reported. The majority of articles (n=62) were on CC (pts 756; 77.5% females). We identified 16 papers comprising 779 pts (69.2% females) with LC and 7 articles describing 47 pts (72.3% females) diagnosed as MC. The youngest patient was 10 and the oldest a 97-year-old. Aside diarrhea, symptoms included abdominal pain, weight loss, bloating, flatulence, edema and others. In the study group we found 615 (38.8%) persons with macroscopic lesions in gut. Isolated linear ulcerations were identified in 7 pts (1.1%) while non-ulcerous lesions i.e. pseudomembranes, a variable degree of vasculature pruning &amp; dwindling, mucosal lacerations and abnormalities such as erythema/edema/nodularity, or surface textural alteration in 608 pts (98.1%). The location of endoscopic findings was not reported in 27 articles. The distinct endoscopic findings were described in the left (descending, sigmoid, rectum - 10/21/11 studies), right (cecum, ascending - 7/7 studies), transverse colon (n=12), as well as duodenum (n=4), and terminal ileum (n=2). In 17 (1.1%) pts colonic perforation occurred. Endoscopic findings are recognized with increased frequency in pts with MC. This could improve MC diagnosis by prompting a more extensive biopsy protocol in such cases and an earlier initiation of treatment. Procedure-related perforation has been reported in this group; therefore, cautious air insufflation is advisable when endoscopic findings are recognised."}, {"id": "wiki20220301en084_292", "title": "Collagenous colitis", "score": 0.012365626854844028, "content": "Collagenous colitis is an inflammatory bowel disease affecting the colon specifically with peak incidence in the 5th decade of life, affecting women more than men. Its clinical presentation involves watery diarrhea in the absence of rectal bleeding. It is often classified under the umbrella entity microscopic colitis, that it shares with a related condition, lymphocytic colitis. Signs and symptoms Microscopic colitis causes chronic watery diarrhea with greater than 10 bowel movements per day. Some patients report nocturnal diarrhea, abdominal pain, urgency, fecal incontinence, fatigue, dehydration and weight loss. Patients report a significantly diminished quality of life. Causes The cause of collagenous colitis is unknown."}, {"id": "pubmed23n0796_5144", "title": "Microscopic colitis: Common cause of unexplained nonbloody diarrhea.", "score": 0.012103261664472722, "content": "Microscopic colitis (MC) is characterized by chronic, watery, secretory diarrhea, with a normal or near normal gross appearance of the colonic mucosa. Biopsy is diagnostic and usually reveals either lymphocytic colitis or collagenous colitis. The symptoms of collagenous colitis appear most commonly in the sixth decade. Patients report watery, nonbloody diarrhea of a chronic, intermittent or chronic recurrent course. With collagenous colitis, the major microscopic characteristic is a thickened collagen layer beneath the colonic mucosa, and with lymphocytic colitis, an increased number of intraepithelial lymphocytes. Histological workup can confirm a diagnosis of MC and distinguish the two distinct histological forms, namely, collagenous and lymphocytic colitis. Presently, both forms are diagnosed and treated in the same way; thus, the description of the two forms is not of clinical value although this may change in the future. Since microscopic colitis was first described in 1976 and only recently recognized as a common cause of diarrhea, many practicing physicians may not be aware of this entity. In this review, we outline the epidemiology, risk factors associated with MC, its etiopathogenesis, the approach to diagnosis and the management of these individuals. "}, {"id": "pubmed23n0412_7259", "title": "[Ulcerative colitis with segmental involvement].", "score": 0.01195614936622131, "content": "Ulcerative colitis is a chronic inflammatory disease affecting areas of the colon or the full length. From the endoscopic point of view, ulcerative colitis presents lesions that stretch continuously from the rectum to variable colon segments, a characteristic that is of great value when distinguishing it from Crohn's disease. Continuous involvement, without healthy patches, justifies ending endoscopic exploration once the distal end of the lesion has been reached. To retrospectively study the frequency of segmental lesions in the colonoscopies performed in patients with ulcerative colitis. Diagnosis of ulcerative colitis and proctitis was established by clinical, endoscopic, histologic, analytical, and radiological criteria. The indication and number of endoscopies was made on the basis of the clinical criteria of diagnosis, acute episodes, refractoriness or dysplasia screening. The extent of the examination also depended on clinical criteria: the severity of the episode, tolerance to colonoscopy or the degree of cleansing. A total of 155 coloscopies were performed. In 113 colonoscopies (73%) the distal end of the lesion was reached and in 70 (45%) the cecum was reached. Of the 80 patients, 27 (33%) presented ulcerative proctitis at diagnosis. Nine of the 80 patients (11.3%) biopsies were performed in healthy colonic patches, which confirmed histological normality. Six of the 9 patients were receiving no treatment. In all patients except two, the cecum was reached in one or more of the colonoscopies. The distribution of the segmental lesions varied but these were mainly found in the periappendicular region and in the cecum in 6 of the 7 patients in whom the cecum was reached. Of the 80 patients, endoscopic evidence of rectal sparing was found in 5 (6.3%); of these, 4 were receiving systemic or topical treatment. Histological analysis confirmed the absence of inflammatory lesions in these patients. The only patient who was not receiving treatment presented microscopic lesions compatible with ulcerative colitis. Endoscopic segmental lesions in ulcerative colitis were present in 11.3% of patients. Segmental lesions were most frequently found in the cecum and periappendicular region. Endoscopic and histologic evidence of rectal sparing may be the result of systemic or topical treatment."}, {"id": "pubmed23n0080_374", "title": "Pitfalls in the diagnosis of collagenous colitis: experience with 75 cases from a registry of collagenous colitis at the Johns Hopkins Hospital.", "score": 0.011750721684496519, "content": "Collagenous colitis is a relatively rare disorder presenting mainly in middle-aged women as watery diarrhea. Endoscopic and radiographic studies of the colon are usually normal, and diagnosis must be made by biopsy. The characteristic biopsy findings are a combination of increased mucosal inflammation (collagenous colitis) as well as subepithelial collagenous thickening. The mucosal inflammatory changes include increased lamina propria plasma cells, prominent intraepithelial lymphocytes, and in some cases, numerous eosinophils. The collagenous thickening has qualitative as well as quantitative differences from normal, and may be highlighted by Masson trichrome stains. Simply quantitating the thickness of a subepithelial collagen layer is neither adequate nor necessary for the diagnosis of collagenous colitis. Major problems in diagnosing collagenous colitis arise from focusing solely on the subepithelial region without attention to inflammatory changes. For example, tangential sectioning of normal colon results in an artifactually thickened basement membrane, and such cases have been wrongly interpreted as collagenous colitis. If biopsies lack the characteristic inflammatory pattern, a tangentially cut thick basement membrane should be ignored. The key to correct diagnosis of collagenous colitis is analyzing the summation of various inflammatory changes plus subepithelial collagenization, rather than focusing on any single feature in isolation."}, {"id": "pubmed23n0511_11519", "title": "[Clinical significance of erosive and/or small ulcerative lesions in the colon and terminal ileum--short-term follow-up study].", "score": 0.011685463659147868, "content": "Various etiologies and diseases may be related to erosions and/or small ulcers without gross inflammatory changes in the surrounding mucosa found in the colon and terminal ileum during colonoscopy. However, studies on follow-up of these lesions are rare. Thus, we investigated the clinical significance of these lesions and their characteristics helpful for differential diagnosis. We reviewed the data of 183 patients with colonoscopically observed erosive or small ulcerative lesions (&lt;2 cm), and analyzed them according to the location, number, and size of lesions, histopathologic findings, chief complaints, laboratory findings, changes of symptoms, and changes in lesions during 4-12 week follow-up period. Histopathologic findings of these lesions included acute nonspecific inflammation, chronic nonspecific inflammation, Crohn's disease, tuberculous colitis, ischemic colitis, Behcet's disease, cytomegalovirus infection, eosinophilic colitis, ulcerative colitis or pseudomembranous colitis, but most of them were nonspecific (84%). In patients with nonspecific inflammation, histopathologic findings, symptoms, location and multiplicity of the lesions were not prognostic factors for the persistency of symptoms and lesions during follow-up period. Two patients with acute inflammation, who showed no improvement in symptoms and lesions, were later diagnosed as Crohn's disease. Erosive or small ulcerative lesions without macroscopic inflammatory changes in the surrounding mucosa during colonoscopy, are mainly nonspecific. However, careful follow-up is required when the symptoms and/or lesions are not improved."}, {"id": "wiki20220301en183_34351", "title": "Pancolitis", "score": 0.011586142973004285, "content": "Pancolitis, in its most general sense, refers to inflammation of the entire colon. This can be caused by a variety of things. Pancolitis or universal colitis is frequently used in a more specific fashion to denote a very severe form of ulcerative colitis. This form of ulcerative colitis is spread throughout the entire large intestine including the right colon, the left colon, the transverse colon, descending colon, and the rectum. A diagnosis can be made using a number of techniques but the most accurate method is direct visualization via a colonoscopy. Symptoms are similar to those of ulcerative colitis but more severe and affect the entire large intestine. Patients with ulcerative colitis generally exhibit symptoms including rectal bleeding as a result of ulcers, pain in the abdominal region, inflammation in varying degrees, and diarrhea (often containing blood). Pancolitis patients exhibit these symptoms and may also experience fatigue, fever, and night sweats. Due to the loss of"}, {"id": "wiki20220301en070_39282", "title": "Microscopic colitis", "score": 0.011531600407747197, "content": "Epidemiology Incidence and prevalence of microscopic colitis nears those of ulcerative colitis and Crohn's disease. Studies in North America found incidence rates of 7.1 per 100,000 person-years and 12.6 per 100,000 person-years for collagenous colitis for lymphocytic colitis, respectively. Prevalence has been estimated as 103 cases per 100,000 persons. People who develop microscopic colitis are characteristically, though not exclusively, middle-aged females. The average age of diagnosis is 65 but 25% of cases are diagnosed below the age of 45. History The condition of microscopic colitis was first described as such in 1982. Lymphocytic colitis was described in 1989. Collagenous colitis was recognised earlier, in 1976. References External links MayoClinic.com Colitis Steroid-responsive inflammatory conditions"}, {"id": "wiki20220301en070_39280", "title": "Microscopic colitis", "score": 0.01111233967271119, "content": "Differential diagnosis Differential diagnoses, which should be ruled out, include bile acid diarrhea, lactose malabsorption, celiac disease, Crohn's disease, ulcerative colitis, and infectious colitis. Treatment Lymphocytic and collagenous colitis have both been shown in randomized, placebo-controlled trials to respond well to budesonide, a glucocorticoid. Budesonide formulated to be active in the distal colon and rectum is effective for both active disease and in the prevention of relapse. However, relapse occurs frequently after withdrawal of therapy. Studies of a number of other agents including antidiarrheals, bismuth subsalicylate (Pepto-Bismol), mesalazine/mesalamine (alone or in combination with cholestyramine), systemic corticosteroids, cholestyramine, immunomodulators, and probiotics have shown to be less effective than budesonide for treating both forms of microscopic colitis."}, {"id": "wiki20220301en100_5790", "title": "List of MeSH codes (C06)", "score": 0.011024033437826541, "content": "– gastroenteritis – appendicitis – cholera morbus – colitis – colitis, ischemic – colitis, microscopic – colitis, collagenous – colitis, lymphocytic – colitis, ulcerative – proctocolitis – dysentery – dysentery, amebic – dysentery, bacillary – enteritis – duodenitis – ileitis – pouchitis – enterocolitis – enterocolitis, necrotizing – enterocolitis, neutropenic – enterocolitis, pseudomembranous – esophagitis – esophagitis, peptic – gastritis – gastritis, atrophic – gastritis, hypertrophic – inflammatory bowel diseases – colitis, ulcerative – crohn disease – mucositis – proctitis – proctocolitis"}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 16]], "word_ranges": [[0, 2]], "text": "Aortic stenosis."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Aortic stenosis.", "full_answer_no_ref": "Aortic stenosis.", "full_question": "A 78-year-old patient with no past history who consults for asthenia and dyspnea of 3 months of evolution, which has progressed to rest in the last few days. In the previous days he also refers chest pain with anginal characteristics with small efforts. On examination, blood pressure of 110/80 mmHg, heart rate of 85 bpm and auscultation with a rough systolic murmur in the right second intercostal space and crackles in both lung bases. Which of the following is the most likely diagnosis?", "id": 545, "lang": "en", "options": {"1": "Mitral insufficiency.", "2": "Aortic stenosis.", "3": "Aortic insufficiency.", "4": "Dilated cardiomyopathy of ischemic origin.", "5": NaN}, "question_id_specific": 122, "type": "CARDIOLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0402_6551", "title": "[An autopsied case with a bicuspid aortic valve who had progressive angina pectoris and heart failure during follow-up of 27 years].", "score": 0.017713490099009903, "content": "A Japanese man who died at age 85 had been followed since the age of 59, when he first presented. He had hypertension of 162/102 mmHg and a loud systolic murmur on his first visit. He had had an active daily life without any medication for the next 10 years. At the age of 72 he complained of mild chest discomfort on exercise. Although electrocardiography showed no abnormalities, echocardiogram showed calcified bicuspid aortic valve with mild stenosis. At the age of 81 the dyspnea and chest oppression were exacerbated, associated with marked ST depression on exercise electrocardiogram and restriction of aortic valve opening on echocardiograms. In the following years a gradual increase in QRS voltage and ST depression with T wave inversion were recorded on resting electrocardiograms and sharp increases in both left ventricular end-diastolic diameter and flow velocity at the aortic root were observed on echocardiograms. At the age of 85 he died of intractable heart failure with massive pleural effusion. Autopsy revealed marked hypertrophy and moderate dilatation of the heart (weight: 580 g). The bicuspid aortic valve had anterior-posterior cusps with a raphe on the anterior cusp. The mobility of the cusps was almost lost because of severe calcification and thickening. Severe stenosis was found near the orifice of the right coronary artery, but there were no significant ischemic myocardial lesions."}, {"id": "pubmed23n0495_3220", "title": "Cases from the Osler Medical Service at Johns Hopkins University.", "score": 0.01668520578420467, "content": "PRESENTING FEATURES: A 70-year-old African American man was admitted with a history of fever, chills, and malaise of several days' duration. His past medical history was notable for end-stage renal disease requiring hemodialysis, coronary artery disease, and aortic stenosis requiring a bioprosthetic aortic valve replacement. On the day of admission, the patient was noted to have a shaking chill while undergoing dialysis through his catheter and was admitted to the hospital. He complained of pain at the catheter insertion site, shortness of breath, and dyspnea on exertion, but denied chest pain. On physical examination, the patient had a temperature of 100.4 degrees F, with a heart rate of 64 beats per minute, blood pressure of 127/72 mm Hg, and an oxygen saturation of 97% on room air. He was a mildly obese man in no apparent distress. He had shotty cervical lymphadenopathy and a right subclavian dialysis catheter in place, with erythema and pus at the entry site. His jugular venous pressure was 10 cm H(2)O. Lung examination showed bibasilar rales. Heart sounds were normal, with no rub or gallop. He had a 2/6 systolic ejection murmur best heart at the left sternal border as well as a 3/6 holosystolic murmur at the apex that radiated to his left axilla. Examination of the abdomen and extremities was unremarkable. The patient's neurological examination was unremarkable, and he was alert and oriented to person, place, and time. Laboratory studies showed an elevated white blood cell count of 16,700 cells/microL. His blood urea nitrogen level was 43 mg/dL and his serum creatinine level was 4.9 mg/dL. Multiple blood cultures grew methicillin-resistant Staphylococcus aureus. An admission, chest radiograph showed no infiltrate. An admission electrocardiogram showed normal sinus rhythm with first degree atrioventricular block, left anterior fascicular block, and left ventricular hypertrophy. shows rhythm strips from lead II electrocardiograms 5 months before admission (top), on admission (middle) and 5 days after admission (bottom). What is the diagnosis?"}, {"id": "pubmed23n0909_10224", "title": "Dyspnoea on exertion in a 53-year-old woman.", "score": 0.01641641641641642, "content": "A 53-year-old woman with no previous medical history complained of easy fatigue over the last 6 months. She had a positive family history for coronary artery disease but no other risk factors. On physical examination, a 3/6 pansystolic murmur was heard over the apex, and the lung auscultation was unremarkable. Her ECG showed a left anterior fascicular block, with poor R wave progression in the anterior leads (see online supplementary image A). A subsequent echocardiogram revealed a slightly dilated for the patient's body surface area (BSA) (1.73 m<sup2</sup) left ventricle (55/35 mm), with preserved systolic function and a moderate functional mitral regurgitation. The estimated pulmonary artery pressure was 45 mm Hg. During treadmill radionuclide scintigraphy, her exercise tolerance was normal, with good inotropic response, and 96% oxygen saturation at rest and at peak exercise. A 2 mm ST segment depression was noted at peak effort, which persisted well into recovery (see online supplementary image B). The scintigraphy scan showed extensive reversible anteroapical wall ischaemia (see online supplementary image C). At this point she was referred to us for right and left heart catheterisation. Intracardiac pressures and saturations were: right atrium (RA)RA=3 mm Hg, right ventricle (RV)=26/3 mm Hg, Pulmonary artery (PA)=26/10/mean 16 mm Hg, pulmonary capillary wedge pressure (PCWP)=11 mm Hg, left ventricle (LV)=110/10 mm Hg, Aorta (Ao)=110/60/mean 80 mm Hg, Superior vena cava saturation (SVCsat)=62%, RAsat=62%, PAsat=78%, Aosat=96% and estimated pulmonary to systematic flow ratio (Qp/Qs)=1.8. Her coronary angiography and CT angiography are shown in figure 1A,B.DC1SP110.1136/heartjnl-2017-311256.supp1Supplementary material 1 DC2SP210.1136/heartjnl-2017-311256.supp2Supplementary material 2 DC3SP310.1136/heartjnl-2017-311256.supp3Supplementary material 3 heartjnl;103/17/1390/F1F1F1Figure 1Coronary and CT angiograms. What is the most likely diagnosis?Right coronary fistula to right ventricleKawasaki disease with fistulaAnomalous origin of the left coronary artery from the pulmonary arteryPersistent truncus arteriosus."}, {"id": "wiki20220301en063_19521", "title": "Valvular heart disease", "score": 0.01601246105919003, "content": "Medical signs of aortic stenosis include pulsus parvus et tardus, that is, diminished and delayed carotid pulse, fourth heart sound, decreased A2 sound, sustained apex beat, precordial thrill. Auscultation may reveal a systolic murmur of a harsh crescendo-decrescendo type, heard in 2nd right intercostal space and radiating to the carotid arteries. Aortic regurgitation Patients with aortic regurgitation may experience heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea, palpitations, and angina pectoris. In acute cases patients may experience cyanosis and circulatory shock."}, {"id": "wiki20220301en063_19522", "title": "Valvular heart disease", "score": 0.015421899096717338, "content": "Medical signs of aortic regurgitation include increased pulse pressure by increased systolic and decreased diastolic blood pressure, but these findings may not be significant if acute. The patient may have a diastolic decrescendo murmur best heard at left sternal border, water hammer pulse, Austin Flint murmur, and a displaced apex beat down and to the left. A third heart sound may be present Mitral stenosis Patients with mitral stenosis may present with heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea, palpitations, chest pain, hemoptysis, thromboembolism, or ascites and edema (if right-sided heart failure develops). Symptoms of mitral stenosis increase with exercise and pregnancy"}, {"id": "pubmed23n0788_603", "title": "Chronic dissecting aneurysm of the ascending aorta developed in a patient who had rejected surgical treatment for type II acute ascending aortic dissection three years earlier.", "score": 0.013423710792131845, "content": "A 66-year-old male patient was admitted to our clinic because of shortness of breath and chest pain. A grade 4/6 diastolic murmur was heard on auscultation. Physical examination revealed signs of congestive heart failure and poor peripheral perfusion. There was a diagnosis of type II ascending aortic dissection in the history of the patient. He had refused emergency surgical intervention three years earlier. Computed tomography revealed that the ascending aorta was dilated to about 10 cm in diameter, and there was a chronic aortic type II dissection. The patient had second- to third-degree aortic insufficiency and he had a calcified bicuspid aortic valve on echocardiography. Two-vessel disease and a 90-mmHg aortic gradient were detected on angiography. Graft replacement of the ascending aorta, serape aortic valve replacement with a mechanical valve, and coronary arterial bypass grafting were performed successfully under cardiopulmonary bypass with an open aortic technique. The patient was discharged on the 10th postoperative day with no problems. "}, {"id": "article-17749_11", "title": "Aortic Valve Disease -- History and Physical", "score": 0.013093395252837977, "content": "Aortic regurgitation when acute and/or severe can be suspected when the patient has a wide pulse pressure, and a low pitched early diastolic murmur is auscultated again over the right sternal border at the second intercostal space. Accentuated P2 may also be noticed due to elevated pressures in the pulmonary vasculature. Chronic aortic regurgitation may illicit a blowing diastolic decrescendo murmur with a positive correlation between the duration of murmur and the severity of the disease. Often auscultation of a laterally and inferiorly displaced apical impulse is present and sustained. Often there is the phenomenon of Corrigan pulse (water hammer), a bounding and forceful pulse that rapidly increases and collapses. Other less common physical exam findings include the de Musset sign, which is subtle head bobbing with a pulse, as well as the Quincke sign and Muller sign (pulsations on the fingernails and uvula, respectively). Similar physical exam findings to aortic stenosis can also be seen late in disease progression. Acute aortic regurgitation will present differently depending on the etiology. If a patient presents with tearing severe chest pain along with physical exam findings such as variation in blood pressure between the right and left extremities, consider aortic dissection as a cause. If the patient presented with a history of streptococcal infection along with fevers, swollen and tender joints, skin nodules, new onset of rash, then rheumatic heart disease would be high on the differential."}, {"id": "pubmed23n0753_3228", "title": "Case 1/2013: 69-year-old male patient with sudden back and lower right limb pain and shock.", "score": 0.012987560615644107, "content": "The patient, MSM, a 69-year-old man, sought medical care due to left dorsal and right lower limb pain. The chest x-ray showed mediastinal enlargement. He was undergoing examination when he lost consciousness and went into shock. Subcutaneous emphysema was observed in the left hemithorax, as well as abolition of breath sounds at auscultation. Tracheal intubation was performed with draining of blood-tinged fluid from the left hemithorax. Echocardiography showed left ventricle with 44/29 mm; septum, 12 mm; posterior wall, 13 mm; mild aortic root dilation, dissection of the lamina and periaortic hematoma. The valves and pericardium were normal. The patient was transferred to Instituto do Coraçao - InCor. Physical examination (21 Oct 2004: 10:45) showed that the patient was sedated with tracheal intubation, pale, heart rate at 90 bpm, blood pressure 130 x 80 mmHg, bloody drainage in the chest tube. Electrocardiogram - frequency 90 bpm, sinus rhythm, low voltage in the frontal plane and decreased voltage in left leads (Fig. 1). Computed tomography showed bilateral subcutaneous emphysema, thoracic aorta with inaccurate borders in its descending portion (from the subclavian artery to the middle portion), collapsed left lung and extensive collection of hematic characteristics in same hemithorax and middle and posterior mediastinum. Small right pneumothorax; small right pleural effusion with underlying parenchymal alterations. The analysis of the heart was impaired by the presence of hemothorax. While undergoing computed tomography, the patient showed no pulse, mydriasis, with asystole unresponsive to resuscitation and died (21 Oct 2011; 15:00 h)."}, {"id": "wiki20220301en024_55490", "title": "Aortic regurgitation", "score": 0.012530016624592083, "content": "If there is increased stroke volume of the left ventricle due to volume overload, an ejection systolic 'flow' murmur may also be present when auscultating the same aortic area. Unless there is concomitant aortic valve stenosis, the murmur should not start with an ejection click. There may also be an Austin Flint murmur, a soft mid-diastolic rumble heard at the apical area; it appears when a regurgitant jet of blood from severe aortic regurgitation partially closes the anterior mitral leaflet. Peripheral physical signs of aortic regurgitation are related to the high pulse pressure and the rapid decrease in blood pressure during diastole due to blood returning to the heart from the aorta through the incompetent aortic valve, although the usefulness of some of the eponymous signs has been questioned: Phonocardiograms detect AI by having electric voltage mimic the sounds the heart makes. Characteristics- indicative of aortic regurgitation are as follow:"}, {"id": "wiki20220301en021_58644", "title": "Chest pain", "score": 0.011873240028579833, "content": "Cocaine use: This condition is suspected when a person with few or no risk of arteriosclerosis presented with non-traumatic chest pain. Ingestion of cocaine can cause vasoconstriction of coronary arteries, thus producing chest pain similar to heart attack. Symptoms can appear within one hour of cocaine use. Aortic stenosis: This condition happens when the person has underlying congenital bicuspid valve, aortic sclerosis, or history of rheumatic fever. Chest pain usually happens during physical activity. Syncope is a late symptom. Signs and symptoms of heart failure may also present. On auscultation, loud ejection systolic murmur can be best heard at the right second intercostal space and radiates to the carotid artery in the neck. Splitting of second heart sound is heard in severe stenosis."}, {"id": "article-40408_6", "title": "Austin Flint Murmur -- History and Physical", "score": 0.011808137403780698, "content": "The Austin Flint murmur is a rumbling diastolic murmur best heard at the apex of the heart that is associated with severe aortic regurgitation and is usually heard best in the fifth intercostal space at the midclavicular line. Younger patients are more likely to have a history of a bicuspid aortic valve or rheumatic heart disease, while older patients are more likely to suffer from calcific valvular disease. A blood pressure reading on the patient will show an increased pulse pressure due to the backflow of blood through the aortic valve during diastole. An astute clinician may be able to palpate a \"water hammer\" pulse, which is also known as \"Corrigan's pulse.\" This finding demonstrates arterial swelling followed by a brisk diastolic fall. Patients may endorse a history of syncope or lightheadedness associated with an inability to maintain forward flow through the aortic valve and the significant difference between systolic and diastolic pressure. Decreasing exercise tolerance and the inability to perform activities of daily living should prompt screening for this condition."}, {"id": "article-17744_7", "title": "Aortic Stenosis -- History and Physical", "score": 0.01158008658008658, "content": "The acquired aortic stenosis manifests with exertional dyspnea, syncope, angina, and, ultimately, heart failure. [17] [18] Typically, symptoms begin at the age of 50 to 70 years in patients with the bicuspid aortic valve and in greater than 70 years in patients with tri-leaflet valve calcific stenosis. Patients progressively experience a gradual decrease in exercise tolerance, dyspnea on exertion, and fatigue. Severe exertional dyspnea, paroxysmal nocturnal dyspnea, orthopnea, and pulmonary edema show various degrees of pulmonary venous hypertension. Angina results from the combination of the need for increased oxygen in hypertrophied myocardium and reduction of oxygen delivery secondary to the excessive compression of coronary vessels. Syncope is caused by the decrease in cerebral perfusion occurring during exertion when the arterial pressure declines due to systemic vasodilation and an inadequate increase in cardiac output related to stenosis. It is also due to the malfunction of the baroreceptor mechanism in severe aortic stenosis. Non-cardiac symptoms include gastrointestinal (GI) bleeding and cerebral emboli. GI bleeding is observed in patients with severe aortic stenosis and is often associated with angiodysplasia or other vascular malformations. It manifests from shear stress-induced platelet aggregation and reduction in the von Willebrand factor. [19] Cerebral emboli occur due to microthrombi formation on thickened bicuspid valves. There is also an observed increase in the risk of infective endocarditis in patients with aortic valve disease, especially with a bicuspid valve. On examination, carotid upstroke can be observed on palpation. A slow-rising, late-peaking, and a low-amplitude carotid impulse, pulsus parvus et tardus, is an expected finding in severe aortic stenosis and, when present, is specific to aortic stenosis. On auscultation, the second heart sound may lack a split and can be heard as a single sound during inspiration. It can also become paradoxical when the closure of the aortic valve gets delayed than the pulmonic valve. A mid-systolic ejection murmur, heard best over the right second intercostal space, with radiation into the right neck. However, high-frequency components may radiate to the apex in calcified aortic valves, and this phenomenon is called the Gallavardin phenomenon. The murmur becomes softer in LV failure and when there is a fall of stroke volume."}, {"id": "pubmed23n0960_13522", "title": "Twenty-three year-old with pleuritic chest pain.", "score": 0.011458333333333334, "content": "A 23-year-old woman followed at another medical centre for congenital heart disease (CHD) presented to our emergency clinic with 3 weeks of bilateral pleuritic chest pain. She returned from holiday in Greece 6 weeks earlier where a tattoo and nasal piercing had been performed. There was no history of night sweats or fever.Her temperature was 37.5°C, heart rate 120 beats/min, oxygen saturations 94% on room air and blood pressure 110/74. Her chest was clear and there was systolic murmur on auscultation. The chest radiograph showed peripheral bilateral lower zone atelectasis. The ECG demonstrated sinus tachycardia. The haemoglobin was 11.2 g/dL, white cell count 10.18×10<sup9</sup/L, C-reactive protein 67 mg/L (normal &lt;5 mg/L) and D dimer=430 ng/mL (normal &lt;230 ng/mL).A pulmonary embolus was suspected and a CT pulmonary angiogram was performed (figure 1). Based on the CT findings, what is the most likely underlying congenital heart lesion in this patient?Bicuspid aortic valveCoarctation of the aortaFontan circulationParachute mitral valveVentricular septal defect heartjnl;105/6/464/F1F1F1Figure 1CT pulmonary angiogram (coronal views)."}, {"id": "wiki20220301en068_46138", "title": "Cardiac examination", "score": 0.011403508771929824, "content": "Finally the sacrum and ankles are checked for pitting edema which is caused by right ventricular failure in isolation or as part of congestive cardiac failure. Auscultation One should comment on S1 and S2 – if the splitting is abnormal or louder than usual. S3 – the emphasis and timing of the syllables in the word Kentucky is similar to the pattern of sounds in a precordial S3. S4 – the emphasis and timing of the syllables in the word Tennessee is similar to the pattern of sounds in a precordial S4. If S4 S1 S2 S3 Also known as a gallop rhythm. diastolic murmurs (e.g. aortic regurgitation, mitral stenosis) systolic murmurs (e.g. aortic stenosis, mitral regurgitation) pericardial rub (suggestive of pericarditis) The base of the lungs should be auscultated for signs of pulmonary oedema due to a cardiac cause such as bilateral basal crepitations."}, {"id": "InternalMed_Harrison_18477", "title": "InternalMed_Harrison", "score": 0.011368813418927313, "content": "Death in patients with severe AS occurs most commonly in the seventh and eighth decades. Based on data obtained at postmortem examination in patients before surgical treatment became widely available, the average time to death after the onset of various symptoms was as follows: angina pectoris, 3 years; syncope, 3 years; dyspnea, 2 years; congestive heart failure, 1.5–2 years. Moreover, in >80% of patients who died with AS, symptoms had existed for <4 years. Among adults dying with valvular AS, sudden death, which presumably resulted from an arrhythmia, occurred in 10–20%; however, most sudden deaths occurred in patients who had previously been symptomatic. Sudden death as the first manifestation of severe AS is very uncommon (<1% per year) in asymptomatic adult patients. Calcific AS is a progressive disease, with an annual reduction in valve area averaging 0.1 cm2 and annual increases in the peak jet velocity and mean valve gradient averaging 0.3 meters/s and 7 mmHg, respectively"}, {"id": "pubmed23n0119_16887", "title": "[Silent aortic insufficiency mimicking dilated cardiomyopathy].", "score": 0.011349730788056778, "content": "The authors report 6 cases of severe and silent aortic insufficiency having simulated in all aspects the picture of a dilated cardiomyopathy at the stage of cardiac insufficiency with primary manifestations. They insist on signs leading to the diagnosis of this clinical entity: past history of rheumatism, signs of electrical left ventricular hypertrophy in the absence of arterial hypertension, aortic calcifications and mostly presence of a discrete mitral diastolic fluttering during echocardiographic examination. Supra-sigmoid aortic angiography confirms the diagnosis of severe aortic regurgitation. In order to explain the non-perception of the murmur, they invoke the alteration of the transmission secondary to an air cushion (3 cases the association of another valvulopathy with murmur (1 case) and mainly the decrease of the leakage by increase of the left ventricular telediastolic pressure and the decrease of the diastolic aortic pressure, with diminution of the turbulences. The advantage of knowing this entity rests on the possibility of valve replacement, even at the stage of myocardial failure, since the satisfactory long-term post-operative evolution in 4 patients stands in contrast with the dangerous nature of a spontaneous evolution."}, {"id": "article-17749_10", "title": "Aortic Valve Disease -- History and Physical", "score": 0.011345515384414296, "content": "For aortic stenosis, a systolic ejection murmur can often be heard best over the right sternal border at the second intercostal space. This murmur will peak early during systole when the disease is mild, and as severity increases will peak later, it also tends to radiate to the carotid arteries along with a slowly rising carotid upstroke. Auscultation often reveals a sustained apical impulse as well. In severe disease states, a thrill can sometimes be palpated over the carotid arteries and aortic area. Additional physical exam findings due to complications and sequelae of aortic stenosis include those associated with heart failure and left ventricle remodeling, such as third and fourth heart sounds, pulmonary crackles, jugular venous distention, and pedal edema."}, {"id": "First_Aid_Step1_294", "title": "First_Aid_Step1", "score": 0.011131344738259509, "content": "Auscultation of the heart (eg, physiologic murmur) Aortic valve sclerosis Where to listen: APT M 111234567777777 Left sternal border: Aortic regurgitation Pulmonic regurgitation cardiomyopathy PA M T Aortic area: Systolic murmur Pulmonic area: Tricuspid area: Mitral area (apex): Crescendo-decrescendo systolic ejection murmur and soft S2 (ejection click may be present). LV >> aortic pressure during systole. Loudest at heart base; radiates to carotids. “Pulsus parvus et tardus”—pulses are weak with a delayed peak. Can lead to Syncope, Angina, and Dyspnea on exertion (SAD). Most commonly due to age-related calcification in older patients (> 60 years old) or in younger patients with early-onset calcification of bicuspid aortic valve."}, {"id": "InternalMed_Harrison_19088", "title": "InternalMed_Harrison", "score": 0.010928553647000249, "content": "Palpation may reveal cardiac enlargement and abnormal contraction of the cardiac impulse (left ventricular dyskinesia). Auscultation can uncover arterial bruits, a third and/or fourth heart sound, and, if acute ischemia or previous infarction has impaired papillary muscle function, an apical systolic murmur due to mitral regurgitation. These auscultatory signs are best appreciated with the patient in the left lateral decubitus position. Aortic stenosis, aortic regurgitation (Chap. 283), pulmonary hypertension (Chap. 304), and hypertrophic cardiomyopathy (Chap. 287) must be excluded, since these disorders may cause angina in the absence of coronary atherosclerosis. Examination during an anginal attack is useful, since ischemia can cause transient left ventricular failure with the appearance of a third 1582 and/or fourth heart sound, a dyskinetic cardiac apex, mitral regurgitation, and even pulmonary edema. Tenderness of the chest wall, localization of the discomfort with a single"}, {"id": "pubmed23n0920_22021", "title": "Exertional dyspnoea in a 28-year-old woman.", "score": 0.010916269887353384, "content": "A 28-year-old woman with a history of critical pulmonic stenosis, status postsurgical valvotomy and subsequent pulmonary valve replacement, presented to the cardiology clinic with 1 year of progressive exertional dyspnoea. She has a heart rate of 75 bpm and blood pressure of 110/55 mm Hg. Cardiac auscultation reveals a 1/6 systolic ejection murmur along the left sternum and an early 3/6 diastolic decrescendo murmur. A transthoracic echocardiogram is obtained (figure 1). Which of the following would be most likely found during right heart catheterisation?Ratio of pulmonary to systemic blood flow (Qp:Qs) &gt;1.5Pulmonary vascular resistance &gt;3 Wood unitsRight atrial pressure &gt;10mm HgPulmonary artery systolic pressure &gt;45mm Hg E. Pulmonary artery diastolic pressure &lt;10mm Hg."}, {"id": "pubmed23n0885_25817", "title": "ECG Case of the Month: Heart Failure in a Man from Chile.", "score": 0.010752688172043012, "content": "A 49-year-old man visiting New Orleans from Chile comes to the hospital complaining of exertional dyspnea for 2 months with the more recent onset of ankle edema. He is a slender man with a blood pressure of 91/60 mmHg, crackles at both lung bases, and markedly distended neck veins. His cardiac rhythm is irregular. A soft murmur of mitral regurgitation and a soft S-3 are heard at the left ventricular apex. All four pedal pulses are easily palpable. Epigastric tenderness is noted on palpation; although the patient has a history of peptic ulcer disease, the tenderness is probably due to an enlarged left lobe of his liver, the result of hepatic congestion from his heart failure. A chest radiograph shows generalized cardiomegaly with an especially large left ventricle. Pulmonary congestion and small bilateral pleural effusions also are noted. The Figure shows the electrocardiogram recorded on admission."}, {"id": "article-17742_11", "title": "Aortic Regurgitation -- History and Physical -- Physical Examination", "score": 0.010438877043354656, "content": "AR is associated with widened pulse pressure as a result of systolic hypertension and decreased diastolic pressure. The apical LV impulse is hyperdynamic and displaced laterally and inferiorly. A prominent systolic thrill may be palpable at the base of the heart or suprasternal notch and over the carotid arteries. It is caused by the large forward stroke volumes and low aortic diastolic pressure. S1 is normal, but S2 is increased (with a dilated aortic root) or decreased (when the aortic leaflets are thickened). A high frequency, blowing, decrescendo, diastolic murmur is heard best third intercostal space along the left sternal border. It is easier to appreciate the murmur of AR at the end of expiration while the patient is leaning forward.  The murmur increases with squatting or isometric exercise and decreases with maneuvers that decrease blood pressure. This murmur is early diastolic with mild AR and becomes holodiastolic with severe AR."}, {"id": "article-28020_14", "title": "Cardiogenic Pulmonary Edema -- History and Physical -- Physical Examination", "score": 0.010233758282128098, "content": "Cardiovascular Findings Tachycardia and hypotension may be present along with jugular venous distention. Auscultation of the heart helps to differentiate between the various causes of valvular lesions causing pulmonary edema. Auscultation typically reveals an S3 gallop in volume overload states, which may be associated with accentuation of the pulmonic component of S2. Several different types of murmurs can be heard depending on the cause of the valvular lesion. Mitral stenosis produces a low-pitched, rumbling diastolic murmur associated with an opening snap at the apex, which becomes accentuated on expiration and produces loud S1. Mitral regurgitation produces a blowing, high-pitched pan-systolic murmur best heard at the apex, radiating to the left axilla and accentuating on expiration, producing soft S1. Aortic stenosis produces a harsh crescendo-decrescendo ejection systolic murmur at the aortic area, increasing on expiration, usually radiating towards the right side of the neck. Aortic regurgitation produces a high-pitched blowing early diastolic murmur best heard in the aortic area, greatest during expiration."}, {"id": "wiki20220301en396_27354", "title": "Supravalvular aortic stenosis", "score": 0.010124691743947761, "content": "Pathophysiology Supravalvular aortic stenosis is due to diffuse or discrete narrowing of ascending aorta. The murmur associated with it is systolic murmur and is similar in character to valvular aortic stenosis murmur but commonly present at 1st Intercostal space (ICS) on the right. Individuals with this anomaly may have unequal carotid pulses, differential blood pressure in upper extremities and a palpable thrill in Suprasternal notch. Individuals with significant supravalvular AS chronically may develop left ventricular hypertrophy and also are at risk of developing coronary artery stenosis. With increased metabolic demands (e.g. exercise) such individuals may develop subendocardial or myocardial ischemia due to increased myocardial oxygen demand and seek medical help with symptoms of exercise induced angina. Diagnosis Treatment References Diseases of the aorta"}, {"id": "pubmed23n0111_15839", "title": "Chest pain in a young woman.", "score": 0.009912314144109799, "content": "Chest pain in a young person is often caused by chest wall tenderness, associated with mitral valve prolapse, or attributed to psychologic factors. Ischemic cardiac pain may be overlooked because of its rare occurrence in this age group. A 35-year-old woman complained of substernal chest pressure precipitated by exertion and relieved by rest. The symptom had been noted for 15 years. Worsening of the symptom during dancing prompted her to seek medical advice. She had no other illnesses, was taking no medications, was a nonsmoker, and had no family history of coronary disease. Physical examination disclosed a grade 1 (on the basis of 1 to 6) systolic ejection murmur, an ejection click, and a grade 2 diastolic murmur. An exercise test produced symptoms at 4 minutes. Coronary arteriography showed the absence of a left coronary ostium and filling of the entire coronary system from the right ostial injection through collateral vessels from the right coronary artery. Surgical repair was recommended. Operative intervention showed a dysplastic bicuspid aortic valve with a membrane that covered the left coronary ostium. Excision of the membrane reestablished antegrade blood flow to the left coronary system. A follow-up exercise test revealed normal findings. Because chest pain in a young person is rarely ischemic in origin, benign or noncardiac causes are usually considered; however, if the history suggests ischemic pain, the possible presence of unusual cardiovascular abnormalities should not be disregarded."}, {"id": "wiki20220301en100_5617", "title": "List of MeSH codes (C14)", "score": 0.009900990099009901, "content": "– heart failure, congestive – cardiomyopathy, dilated – dyspnea, paroxysmal – edema, cardiac – heart neoplasms – heart rupture – heart rupture, post-infarction – ventricular septal rupture – heart valve diseases – aortic valve insufficiency – aortic valve stenosis – aortic stenosis, supravalvular – williams syndrome – aortic stenosis, subvalvular – cardiomyopathy, hypertrophic – discrete subaortic stenosis – heart murmurs – heart valve prolapse – aortic valve prolapse – mitral valve prolapse – tricuspid valve prolapse – mitral valve insufficiency – mitral valve stenosis – pulmonary atresia – pulmonary valve insufficiency – pulmonary valve stenosis – leopard syndrome – pulmonary subvalvular stenosis – tricuspid atresia – tricuspid valve insufficiency – tricuspid valve stenosis"}, {"id": "InternalMed_Harrison_19098", "title": "InternalMed_Harrison", "score": 0.00989786073127724, "content": "A medical professional should be present throughout the exercise test. It is important to measure total duration of exercise, the times to the onset of ischemic ST-segment change and chest discomfort, the external work performed (generally expressed as the stage of exercise), and the internal cardiac work performed, i.e., by the heart rate–blood pressure product. The depth of the ST-segment depression and the time needed for recovery of these ECG changes are also important. Because the risks of exercise testing are small but real—estimated at one fatality and two nonfatal complications per 10,000 tests—equipment for resuscitation should be available. Modified (heart rate–limited rather than symptom-limited) exercise tests can be performed safely in patients as early as 6 days after uncomplicated myocardial infarction (Table 293-2). Contraindications to exercise stress testing include rest angina within 48 h, unstable rhythm, severe aortic stenosis, acute myocarditis, uncontrolled"}, {"id": "wiki20220301en011_153774", "title": "Heart murmur", "score": 0.009837341138982547, "content": "Timing refers to whether the murmur is a systolic or diastolic murmur. Shape refers to the intensity over time; murmurs can be crescendo, decrescendo or crescendo-decrescendo. Crescendo murmurs progressively increase in intensity. Decrescendo murmurs progressively decrease in intensity. With crescendo–decrescendo murmurs (diamond- or kite-shaped murmurs), a progressive increase in intensity is followed by a progressive decrease in intensity. Location refers to where the heart murmur is usually heard best. There are four places on the anterior chest wall to listen for heart murmurs; each of the locations roughly corresponds to a specific part of the heart and should be listened to (through the stethoscope) with the patient lying down, face up. The four locations are: Aortic region - the 2nd right intercostal space. Pulmonic region - the 2nd left intercostal spaces. Tricuspid region - the 4th left intercostal space."}, {"id": "article-25200_13", "title": "Mitral Valve Insufficiency -- History and Physical", "score": 0.009804764771652189, "content": "The majority of patients suffering from chronic MR are asymptomatic, but serial quantitative assessment and follow-up of a patient with asymptomatic MR have shown a significant impact on the survival and clinical outcome. [28] Due to the insidious and slowly progressive nature of chronic MR, patients may not report any specific symptoms and may not notice a progressive decline in functional status during the early phase of the disease by gradual modification of their activities. Bedside evaluation for valvular heart disease is very important as management highly depends on symptoms as well as the severity of valvular lesions. Patients with MR are usually referred due to symptoms of chest pain, shortness of breath, palpitations, leg swelling, or just incidental finding of systolic murmur on physical examination. A thorough history of prior bacterial or viral infections, rheumatic fever, trauma, ischemic events, invasive cardiac/noncardiac procedures and family history with a comprehensive review of systems should be obtained. A detailed physical examination with particular attention to the cardiovascular system, including pulse, apical impulse, jugular veins, edema, signs of congestive heart failure, and assessment of characteristic systolic murmur with other pertinent findings should be documented. [29] [3] A characteristic holosystolic murmur present at the apex and radiating to the left axilla can be present most of the time except in cases of MVP where the murmur appears in mid-late systole with or without midsystolic click. The murmur should not be confused with other systolic murmurs such as aortic stenosis, tricuspid regurgitation, pulmonic stenosis, ventricular septal defect, benign flow murmurs, and other cardiac disorders in the spectrum. [30]"}, {"id": "article-36084_10", "title": "Aortic Valvular Atresia -- History and Physical", "score": 0.009757312794535905, "content": "On physical examination, a precordial bulge can be present, though it is not a reliable indicator. Over 50% of patients, however, have a pulmonary systolic ejection murmur, the intensity of which can vary from grade 1 to grade 3. The murmurs can be heard clearest along the left sternal border. The second heart sound at the cardiac base is mostly single, though sometimes can have a very close split and rarely have an ejection click. Heart failure is quick to develop in about 60% of these patients, with the average age of development being 2.5 days. It is almost always associated with hepatomegaly, with about 3cm of protrusion beyond the right costal margin. An S3 heart sound can sometimes be heard, along with chest rales, edema, and palpable spleen. Blood pressure as well can be lower than average, hovering around 65/45 mmHg, and pulse pressure is generally narrow. No clinically significant pressure difference is noted between upper and lower extremity measurements. [5]"}, {"id": "pubmed23n0060_16716", "title": "[Rapid onset aortic stenosis and clinical course in adults. Clinico-hemodynamic correlations].", "score": 0.009708737864077669, "content": "In 8 patients aged 41-66 years, a second left heart catheterisation done 27-98 months after the first study, demonstrated a pressure gradient across the aortic valve, that had not previously existed, or had been trivial. No significant change of the cardiac output had occurred. All but 1 patient were hypertensive. The etiology was rheumatic in 4, degenerative in 4. Electrocardiographic, radiographic, and echocardiographic evolution could not separate the patients with a gradient greater than or equal to 70 mmHg from those whose gradient was less than or equal to 40 mmHg. The intensity of the aortic component of the second heart sound, however, decreased in all former patients, and in only 1 of the latter. Aortic valvular stenosis can arise and rapidly develop in adult patients. Concomitant rheumatic mitral valve disease, coronary artery disease and hypertension can mask and/or modify symptoms, signs and laboratory findings. Changes of the aortic component of the second sound may suggest its occurrence."}, {"id": "wiki20220301en194_20610", "title": "MYL4", "score": 0.009615384615384616, "content": "Clinical significance MYL4 expression in ventricular myocardium has shown to abnormally persist in neonates up through adulthood in patients with the congenital heart disease, tetralogy of Fallot. Altered ALC-1 expression is also altered in other congenital heart diseases, Double outlet right ventricle and infundibular pulmonary stenosis. Moreover, in patients with aortic stenosis or aortic insufficiency, ALC-1 expression in left ventricles was elevated, and following valve replacement decreased to lower levels; ALC-1 expression also correlated with left ventricular systolic pressure. Additionally, in patients with ischemic cardiomyopathy, dilated cardiomyopathy and hypertrophic cardiomyopathy, ALC-1 protein expression is shown to be reactivated, and ALC-1 expression correlates with calcium sensitivity of myofilament proteins in skinned fiber preparations, as well as ventricular dP/dtmax and ejection fraction. Interactions ALC-1 interacts with: ACTC1 MYH7 References"}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[32, 246]], "word_ranges": [[3, 30]], "text": "This is familial hypocalciuric hypercalcemia or benign familial hypercalcemia. Autosomal dominant disorder. Asymptomatic. Not cured after surgical treatment; it is due to mutation of the calcium-sensitive receptor."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Calcium/creatinine urine ratio. This is familial hypocalciuric hypercalcemia or benign familial hypercalcemia. Autosomal dominant disorder. Asymptomatic. Not cured after surgical treatment; it is due to mutation of the calcium-sensitive receptor. Difficult question since it is necessary to know data of a disease that is not usually asked in the MIR.", "full_answer_no_ref": "Calcium/creatinine urine ratio. This is familial hypocalciuric hypercalcemia or benign familial hypercalcemia. Autosomal dominant disorder. Asymptomatic. Not cured after surgical treatment; it is due to mutation of the calcium-sensitive receptor. [HIDDEN] since it is necessary to know data of a disease that is not usually asked in the MIR.", "full_question": "In a 30-year-old female patient a calcium level of 11 mg/dl (normal less than 10.5 mg/dl) is found during a routine company examination. PTH determination was 45 pg/ml (VN 10-55 pg/ml). The history is unremarkable except for the fact that the mother and paternal grandfather were diagnosed with hyperparathyroidism and underwent surgery, although they remained hypercalcemic. Which test is most useful in confirming the diagnosis?", "id": 257, "lang": "en", "options": {"1": "25-OH D.", "2": "1,25-OH 2D.", "3": "Urine calcium/creatinine ratio.", "4": "Phosphate tubular reabsorption.", "5": "PTHrP."}, "question_id_specific": 95, "type": "ENDOCRINOLOGY", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0125_6826", "title": "Late increase in serum 1,25-dihydroxyvitamin D one month after surgery for adenomatous hyperparathyroidism.", "score": 0.013844862949841507, "content": "This study was designed to follow the evolution of serum 1,25(OH)2D after surgery for primary hyperparathyroidism. Ten patients were studied before and for up to 85 d after removal of a single parathyroid adenoma. Blood and 24 h urine were obtained at various time points, for the measurement of serum or urinary phosphate and calcium indices. Before surgery, serum calcium (2.91 +/- 0.06 mmol/l; mean +/- SEM), parathyroid hormone (354 +/- 47 pg/ml) and 1,25(OH)2D (61.2 +/- 7.8 pg/ml) were elevated while serum phosphate (1.01 +/- 0.07 mmol/l) tended to be low. Relative hypoparathyroidism was evident for up to 5 d after surgery with the lowest value for serum parathyroid hormone (41 +/- 16 pg/ml) on day 1, serum calcium (2.12 +/- 0.06 mmol/l) on day 3 and highest value for serum phosphate (1.41 +/- 0.13 mmol/l) on day 5. As expected, serum 1,25(OH)2D levels decreased to 35.9 +/- 4.2 pg/ml 24 h after surgery. Stabilization of serum and urinary parameters to normal values was seen between day 5 and day 27; the only exception was serum 1,25(OH)2D, which increased again at day 27 to 57.6 +/- 5.0 pg/ml, a value as high as that before surgery. It was still elevated at day 50 (58.3 +/- 4.3 pg/ml), but returned towards normal values in three out of four patients (44 +/- 3.9 pg/ml) by day 80. No variation in 25(OH)D or 24,25(OH)2D was seen throughout the study. 1,25(OH)2D values could be related to serum parathyroid hormone values before surgery (r = 0.659, P less than 0.05) but not after. The secondary increase in serum 1,25(OH)2D could not be related to variations in serum calcium, phosphate, parathyroid hormone or diet. Further studies will be required to explain this phenomenon."}, {"id": "wiki20220301en262_2355", "title": "Familial hypocalciuric hypercalcemia", "score": 0.01339437738847478, "content": "Diagnosis As most cases of FHH are asymptomatic and benign, the diagnosis of FHH is less likely to be made. Typically, diagnosis is made in the pursuit of uncovering the etiology of hypercalcemia. Calcium levels are often in the high normal range or slightly elevated. Commonly, the parathyroid hormone level is checked and may be slightly elevated or also on the high normal end. Normally, high calcium should cause low PTH and so this level of PTH is inappropriately high due to the decreased sensitivity of the parathyroid to calcium. This may be mistaken for primary hyperparathyroidism. However, evaluation of urine calcium level will reveal a low level of urine calcium. This too is inappropriate as high serum calcium should result in high urine calcium. If urine calcium is not checked, this may lead to parathyroidectomy for presumed primary hyperparathyroidism. Additionally as the name implies, there may be a family history of benign hypercalcemia."}, {"id": "article-23201_53", "title": "Primary Hyperparathyroidism -- Evaluation -- Specific Hyperparathyroid and Hypercalcemic Disorders", "score": 0.011892029539088363, "content": "Familial hypocalciuric hypercalcemia (FHH) is an autosomal dominant inherited disorder that is sometimes easily confused with primary hyperparathyroidism. It is characterized by hypercalcemia, hypocalciuria, and high-normal to mildly increased parathyroid hormone secretion. [88] [112] Familial hypocalciuric hypercalcemia is caused by a mutation in the calcium-sensing receptor of the chief cells of the parathyroid gland. Histologically, the parathyroid glands appear normal. There is usually a known family history of the disorder. There are no clinical symptoms, and it requires no treatment. The diagnosis is usually made by history or the calcium/creatinine excretion ratio, although 24-hour urine testing showing severe hypocalciuria of <100 mg/24 hours is highly suggestive. The calcium/creatinine excretion ratio [(urinary calcium x serum creatinine) / (serum calcium x urinary creatinine)] in the urine of patients with familial hypocalciuric hypercalcemia is very low, typically <0.01 in 80% of cases, and FHH is more likely if hypercalcemia is detected before age 40. [88] Other causes of hypocalciuric hypercalcemia should be ruled out (vitamin D deficiency, severely calcium-deficient diet, mild renal failure, use of thiazide or lithium). A calcium infusion test can also help distinguish FHH from primary hyperparathyroidism. A calcium load will increase urinary calcium excretion in primary hyperparathyroidism but not familial hypocalciuric hypercalcemia. [88] A definitive diagnosis can be made in questionable cases by genetic testing. [88]"}, {"id": "pubmed23n0094_16279", "title": "Evidence that calcitonin plays a role in the postnatal increase of serum 1 alpha,25-dihydroxyvitamin D.", "score": 0.011731113251685701, "content": "To investigate the changes in the 1 alpha,25-dihydroxyvitamin D [1,25(OH)2D] level and the role of parathyroid hormone (PTH) and calcitonin (CT) during the early neonatal periods, we measured 1,25(OH)2D, 25-hydroxyvitamin D [25(OH)D], PTH specific for mid-regions (mPTH) and urinary cAMP (UcAMP) to evaluate the renal tubular responsiveness to intrinsic PTH and CT, as well as serum Ca and P in 28 mothers at term delivery and in their babies at birth and 5 days of age. Cord serum 1,25(OH)2D levels were low (28.8 +/- 9.2 pg/ml, mean +/- SD), while maternal serum 1,25(OH)2D levels were high (62.2 +/- 22.6 pg/ml). The low 1,25(OH)2D value increased 2.5 times (62.2 +/- 22.6 pg/ml) in 5-day-old infants, reaching a high normal adult value, concomitant with the increases in mPTH and urinary cAMP/creatinine ratio (UcAMP/Cr). The correlations between 1,25(OH)2D and UcAMP/Cr, and 1,25(OH)2D and mPTH in all paired samples of babies at birth and at 5 days of age were r = 0.456, n = 50, P less than 0.01 and r = 0.341, n = 50, P less than 0.05, respectively. These data suggest that the parathyroid activation after birth is a major factor in the rapid 1,25(OH)2D increment at that time. CT levels were high in all paired samples and in 5-day-old infants. CT vs 1,25(OH)2D showed a significant correlation (r = 0.473, P less than 0.05, n = 24) as well as the relative increase of 1,25(OH)2D (delta pg/ml) after birth vs CT at age 5 days (r = 0.537, P less than 0.01, n = 24).(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "article-21613_9", "title": "Familial Hypocalciuric Hypercalcemia -- Evaluation", "score": 0.011721329517939687, "content": "The diagnosis of FHH can be easy to make in an asymptomatic hypercalcemic patient with a family his­tory of hypercalcemia, a personal or family history of failed neck exploration, or normal serum PTH [1] [2] . More than 99% of the filtered calcium gets reabsorbed despite the presence of hypercalcemia. The 24 hours urinary calcium excretion is less than 100 mg/24 hours. The Ca/Cr excretion ratio is very low. It is calculated as follows: [UCa × SCr] / [SCa × UCr], where UCa is the urinary calcium con­centration, SCr is the serum creatinine, SCa is the serum calcium concentration, and UCr is the urinary creati­nine concentration, all in mg/dl. The Ca/Cr clearance ratio is less than 0.01 in 80% of cases. All patients with calcium/creatinine clearance ratio of 0.020 or less should be tested for mutations in the CaSR gene. Serum Magnesium is in the upper-normal range or mildly elevated; whereas, serum magnesium tends to be nor­mal or low in primary hyperparathyroidism."}, {"id": "InternalMed_Harrison_3703", "title": "InternalMed_Harrison", "score": 0.011598464428653107, "content": "Once true hypercalcemia is established, the second most important laboratory test in the diagnostic evaluation is a PTH level using a two-site assay for the intact hormone. Increases in PTH are often accompanied by hypophosphatemia. In addition, serum creatinine should be measured to assess renal function; hypercalcemia may impair renal function, and renal clearance of PTH may be altered depending on the fragments detected by the assay. If the PTH level is increased (or “inappropriately normal”) in the setting of elevated calcium and low phosphorus, the diagnosis is almost always primary hyperparathyroidism. Because individuals with FHH may also present with mildly elevated PTH levels and hypercalcemia, this diagnosis should be considered and excluded because parathyroid surgery is ineffective in this condition. A calcium/creatinine clearance ratio (calculated as urine calcium/ serum calcium divided by urine creatinine/serum creatinine) of <0.01 is suggestive of FHH, particularly when"}, {"id": "pubmed23n0477_6129", "title": "[Vitamin D3 overdosage due to rashly diagnosed rachitis in a child with distal tubular acidosis].", "score": 0.011023382933495293, "content": "Metabolic acidoses are diseases causing many diagnostic and therapeutic problems. Compensated metabolic acidosis can be unrecognised for a long time. This refers especially to isolated renal tubular acidosis (RTA). Unrecognised RTA causes calcium and phosphorus balance disturbances with clinical signs of improper bone mineralization. It happens that some patients with mentioned problems are \"treated\" as rachitic and take high doses of vitamin D. As a result, serum calcium and phosphates as well as urine calcium increase, without the satisfied influence on bone mineralization. We present a case of a 3.5 months old baby, who was \"treated\" as ricket in vitamin D deficiency. This baby was \"cured\" with high doses of cholecalciferol (0.0875 mg/24h for 2 weeks, then 0.175 mg/24h for 3 weeks) because of craniotabes. This treatment was carried on without any metabolic tests and caused the following disturbances: 25(OH)D serum level - 102.7 ng/ml (normal 11-54), 1,25(OH)2D serum level - 39.5 pg/ml (normal 15-70), calcaemia 2.7-2.85 mmol/l, phosphataemia 2.1 mmol/l. In this time the considerable hipercalciuria (second morning urine sample Ca/cr ratio 2.06 mmol/mmol) occurred. The other laboratory test showed as follows: serum albumins 4.5 g/dl, alkaline phosphatase 188 U/l, acid phosphatase 10.7 U l, Cl- 114.9 mmol/l, Na 146 mmol/l, K 4.8 mmol/l, HCO3a 16.6-20.1 mmol/l and pCO2 3.63-3.85 kPa, serum anion gap 11 mEq/l; pH of morning urine 6.5-7. These results suggested the presence of distal RTA aside from symptoms of vitamin D overdosage. The high serum levels of calcium and phosphates, craniotabes, rather low serum alkaline phosphatase activity and presence of metabolic acidosis the symptoms after the normalisation of calcium and phosphorus balance suggested that the distal RTA had been prior to calcium disturbances."}, {"id": "pubmed23n0944_19686", "title": "Humoral hypercalcemia of pregnancy treated with bisphosphonates.", "score": 0.009900990099009901, "content": "Hypercalcemia can be hazardous during pregnancy, most cases being due to primary hyperparathyroidism. We report a case of hypercalcemia with suppressed PTH levels necessitating treatment with bisphosphonates during pregnancy. A 38-year-old woman at the 26th week gestation was admitted because of symptomatic hypercalcemia. She did not take any medication that could influence her calcium levels. Physical examination was unremarkable. Laboratory tests on admission were: calcium 12.7 mg/dL (8.5-10.5 mg/dL), phosphorus 1.8 mg/dL (2.5-4.5 mg/dL) and PTH on 3 consecutive tests 1.2, 1.3 and 1.2 pg/mL (15-65 pg/mL). Her 24h urine calcium was 900 mg, 25-OH-D 40 ng/mL (30-58 ng/mL) and 1,25-OH-D 99 pg/mL (80-146 for women in the third trimester). Abdominal ultrasound revealed multiple hypervascular liver lesions consistent with hemangiomas by MRI. Breast and neck ultrasound were normal, and chest CT revealed few non-significant 0.3-0.7 cm pulmonary nodules with no change after an interval of 3 months. She was treated with isotonic saline, loop diuretics and calcitonin. Despite this treatment, calcium levels remained high (14.1 mg/dL), and pamidronate was initiated. On 35th week gestation, she underwent a cesarean section complicated by hypocalcemia of the newborn. Eight weeks after delivery, her calcium levels are 9.4 mg/dL and PTH 18 mg/dL. According to the extensive workup and the post-partum normalization of PTH and calcium levels, we conclude that excessive secretion of placental PTHrP was the cause of hypercalcemia in this patient. No significant adverse effect of bisphosphonate on the mother or baby were seen at the short term follow up."}, {"id": "pubmed23n0308_20854", "title": "[Adult-onset sex-linked familial hypophosphatemic osteomalacia].", "score": 0.009900990099009901, "content": "Three patients (a grandmother, her daughter and her grandson) belonging to a 23-number-kindred of five generations suffered from adult-onset, X-linked, familiar hypophosphataemic osteomalacia. According to the familiar anecdotes the great-grandmother also had the same disease. The clinical diagnosis was documented by X-ray pictures, scintigraphic and bone histological results, the laboratory diagnosis was proven by blood and urine analyses examined after phosphate loading. The youngest, 24-year-old patient was treated with daily 3 g phosphate and high doses of calcitriol for 2 years. As a new feature of our therapy, per os treatment with 1.25 micrograms calcitriol was supplemented by daily 2-4 micrograms iv. bolus calcitriol for one week every month. The osteomalacia, causing serious symptoms and complaints, healed. Our treatment seemed to be safe, as renal functions, serum total and ionized calcium and PTH levels (including midnight values) remained in normal range. On the basis of our results this disease can be treated by administration of high doses of phosphate, provided that development of hyperparathyroidism is prevented by the coadministration of high doses of calcitriol."}, {"id": "pubmed23n0232_7841", "title": "Vitamin D metabolites and parathyroid hormone in Cushing's syndrome: relationship to calcium and phosphorus homeostasis.", "score": 0.00980392156862745, "content": "We studied the effects of glucocorticoid excess on calcium and phosphorus homeostasis in relation to vitamin D metabolites and parathyroid hormone (PTH) in seven patients with spontaneous ACTH-dependent Cushing's syndrome. Remission of hypercortisolism resulted in a significant increase in tubular reabsorption of phosphate [from 76 +/- 4% to 89 +/- 2% (mean +/- SEM); P less than 0.01] and serum phosphorus (from 3.1 +/- 0.1 to 4.2 +/- 0.2 mg/dl; P less than 0.005). Serum calcium did not change, although there was a reduction in daily urinary calcium excretion from 0.23 +/- 0.02 to 0.107 +/- 0.02 mg calcium/mg creatinine. Serum immunoreactive PTH (iPTH) levels were normal during Cushing's syndrome (34 +/- 5 microleq/ml), but fell significantly after remission to 22 +/- 2 microleq/ml (P less than 0.05). This small decrease in iPTH did not correlate with the improvement of phosphate homeostasis. Plasma 25-hydroxyvitamin D (25OHD) and 1,25-dihydroxyvitamin D [1,25-(OH2)D] concentrations in Cushing's syndrome did not differ from measurements in 97 normal subjects. After treatment, 25OHD did not change, but 1,25-(OH)2D fell in each patient from a mean of 44 to 22 pg/ml (P less than 0.02). 1,25-(OH)2D was inversely correlated with serum phosphorus (r = 0.59; P less than 0.01), but did not correlate with iPTH. The known impairment of intestinal calcium absorption in Cushing's syndrome cannot be attributed to a decrease in the circulating levels of 1,25-(OH)2D. Endogenous hypercortisolism decreases tubular phosphate reabsorption and serum phosphorus, increase tubular phosphate reabsorption and serum phosphorus, increases iPTH, and results in an increase in 1,25-(OH)2D. These events may contribute to the severe loss of bone mass in such patients and may account for the calciuria and phosphaturia of Cushing's syndrome."}, {"id": "pubmed23n0279_6759", "title": "[A rare cause of hypercalcemia: familial hypocalciuric hypercalcemia].", "score": 0.00980392156862745, "content": "Familial hypocalciuric hypercalcemia is a rare disease with autosomal dominant transmission. Its basic defect is unknown and it requires no treatment. A 4 month-old girl was admitted for unexplained crying. She was found to have hypercalcemia (2.8 mmol/l) and later values of blood calcium were 3, 3.1 and 3 mmol/l. The serum free ionic calcium level was also elevated. The serum concentrations of protein, phosphorus, magnesium and the alkaline phosphatase activity were all normal. Serum concentrations of 25-(OH)-D3, 1.25-(OH)-2-D3 and PTH were also normal. The urinary calcium/creatinine ratio was normal and the urinary calcium excretion was 1.08 mg/kg/d. Screening of family members showed hypercalcemia in the father (2.8 mmol/l) and a brother aged 7 years (2.9 mmol/l). Short-term treatment with disodium etidronate lowered the serum calcium level to normal, but hypercalcemia reappeared once the treatment was discontinued. This asymptomatic familial hypercalcemia has the characteristics of familial hypocalciuric hypercalcemia. There was no associated endocrine disorder. Screening of family members is worthwhile."}, {"id": "pubmed23n0682_15895", "title": "Acromegaly as a cause of 1,25-dihydroxyvitamin D-dependent hypercalcemia: case reports and review of the literature.", "score": 0.009708737864077669, "content": "Growth hormone excess has been associated with hypercalciuria and nephrolithiasis. Hypercalcemia in acromegaly is rare and usually due to coexistent primary hyperparathyroidism. To report two cases of 1,25-dihydroxyvitamin D (1,25 (OH)(2) D)-dependent hypercalcemia in cromegaly. A 50 year-old female with 2 years history of hypercalcemia presented with features of acromegaly. Serum calcium (Ca) was 10.9 mg/dl (8.6-10.2), parathyroid hormone (PTH) 20 pg/ml (10-65), PTH-related peptide undetectable, and 1,25 (OH)(2) D 119 pg/ml (15-75). Insulin-like growth factor 1 (IGF1) was 911 ng/ml (49-292) and growth hormone (GH) 14.5 ng/ml (0.03-10). MRI showed a 1.7 cm pituitary tumor. Transsphenoidal adenectomy (TSA) resulted in normalization of IGF1, GH, Ca, and 1,25 (OH)(2) D (50 pg/ml) and complete tumor resection. A 52-year-old female was diagnosed with visual field deficits on routine exam. MRI showed a 3 cm invasive pituitary macroadenoma. IGF1 was 416 ng/ml (87-238) and GH 75.8 (0-6.0) ng/ml. Incidentally, she was found with high Ca of 10.8 mg/dl (8.9-10.3) associated with PTH 19 pg/ml and 1,25 (OH)(2) D66 pg/ml. Postoperatively, IGF1 and GH remained abnormal (440 and 12.8 ng/ml, respectively), while MRI showed parasellar tumor residue. Ca remained high (10.1-11.1 mg/dl), along with elevated 1,25 (OH)(2) D level (81.3 pg/ml). In both cases, other causes of hypercalcemia were ruled out. We present 2 cases of 1,25 (OH)(2) D-dependent hypercalcemia associated with growth hormone excess. Complete resection of tumor produced biochemical remission of acromegaly and normalization of calcium and 1,25 (OH)(2) D levels, while incomplete resection was associated with persistent 1,25 (OH)(2) D-dependent hypercalcemia. Acromegaly should be considered a cause of 1,25 (OH)(2) D-dependent hypercalcemia."}, {"id": "pubmed23n0099_13459", "title": "Familial hypocalciuric hypercalcemia: description of a new kindred with emphasis on its difference from primary hyperparathyroidism.", "score": 0.009708737864077669, "content": "In order to better understand the difference between familial hypocalciuric hypercalcemia (FHH) and primary hyperparathyroidism (PHP), 4 adults with this disease (FHH(+], from the same kindred, and spanning 2 generations, were compared with 6 patients with PHP, 10 normal controls (N) and 3 unaffected members of the same kindred (FHH(-]. Clinically speaking, the FHH(+) patients were asymptomatic while those with PHP had recurring renal stones. Various phosphocalcic balance elements were measured in blood and urine, in a fasting state, following oral loading of 1 g calcium, as well as in 24-hour urine specimens. Serum calcium levels were slightly higher (11.90 +/- .14 vs 11.37 +/- .24 mg/dl; p less than .05), in patients with FHH(+), but serum phosphorus (2.47 +/- .38 vs 2.88 +/- .40 mg/dl), serum parathyroid hormone (301 +/- 147 vs 291 +/- 224 pg/ml) and serum 1,25(OH)2D (52.8 +/- 6.7 vs 55.0 +/- 12.0 pg/ml) values were similar to those observed in patients with PHP. Urinary calcium present in FHH(+), while fasting (.064 +/- .043 mg/100 ml G.F.), after the calcium loading (.150 +/- .071) and in the 24-hour specimen (.089 +/- .016), was below the 95% confidence limit of values obtained in patients with PHP (.225 +/- .026, p less than .001; .413 +/- .066, p less than .001; 314 +/- .080, p less than .001), but similar to those obtained in N and FHH(-).(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0270_3612", "title": "A randomized trial comparing 2.5 mEq/L calcium dialysate and calcitriol to 3.5 mEq/L calcium dialysate in patients on peritoneal dialysis.", "score": 0.009615384615384616, "content": "Peritoneal dialysate containing 2.5 mEq/L of calcium has been used to prevent hypercalcemia when calcium-containing phosphate binders are given. However, worsening of hyperparathyroidism may result. Calcitriol used in conjunction with 2.5 mEq/L calcium dialysate is an attractive alternative, but has not been examined in a controlled trial. Eighteen patients were randomly assigned to either a control group (3.5 mEq/L calcium dialysate without calcitriol) or a study group (conversion to 2.5 mEq/L calcium dialysate with oral calcitriol, median dose 0.25 microgram/day). The initial mean serum calcium (9.9 vs 9.6 mg/dL), phosphate (5.4 vs 5.6 mg/dL), median n-terminal parathyroid hormone (PTH) levels (71 vs 55 pg/mL, normal &lt; 25), and median 1,25 (OH)2 vitamin D levels (4 vs 5 pg/mL, normal 15-60 pg/mL) were not different in the two groups. After 8 weeks the serum calcium and phosphate were unchanged from baseline in both groups. The 9 patients who converted to 2.5 mEq/L calcium dialysate had an insignificant fall in the PTH level, not different from the control group. The median 1,25 (OH)2 vitamin D level rose from 4 to 23 pg/mL (p = 0.003) on calcitriol, but remained unchanged in the control group (5 pg/mL). The median doses of oral calcium (0.9 vs 1.1 g/day) and the frequency of serum calcium levels greater than 11 mg/dL (4/9 vs 3/9 patients, 10% vs 8% of all values) were similar in the study and control groups. Aluminum hydroxide was required intermittently for serum phosphate control in 3 patients on 2.5 mEq/L calcium dialysate and 4 on 3.5 mEq/L calcium dialysate.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0698_11959", "title": "[Usefulness of genetic tests in familial hypocalciuric hypercalcemia with atypical clinical presentation].", "score": 0.009615384615384616, "content": "Biochemical tests related to calcium and phosphorus metabolism have traditionally been considered as a reliable tool to differentiate familial hypocalciuric hypercalcemia (FHH) from primary hyperparathyroidism (PHPT). However, diagnosis may sometimes be difficult even for experienced clinicians. Our objective was to assess the accuracy of diagnostic tests in FHH and the circumstances in which genetic studies are required. A descriptive study was conducted of two families with hypercalcemia and suspected atypical FHH. Urinary calcium excretion was measured in 24-hour urine using different tests (calcium excretion (CE), urinary calcium/creatinine clearance ratio (UCCR)), and serum PTH and 25-hydroxyvitamin D levels were tested. Index cases underwent genetic study. One patient from the first family showed overt, persistent hypercalciuria with values more consistent with PHPT than with FHH if we consider, as proposed by guidelines, a UCCR lower than 0.01 as diagnostic of FHH and a value higher than 0.02 as diagnostic of PHPT. The index case of the second family underwent surgery for a parathyroid adenoma. Both cases had a mutation c. 164C&gt;T (Pro55Leu) in exon 2 in heterozygosis. According to current clinical guidelines, definitive diagnosis of FHH requires genetic confirmation, which allowed in our case for detection of two families with FHH and atypical clinical presentations. We think that rational use of genetic tests may avoid unnecessary surgery and excess monitoring costs."}, {"id": "Surgery_Schwartz_10991", "title": "Surgery_Schwartz", "score": 0.009542447115832634, "content": "an elevated chloride-to-phosphate ratio (>33). Urinary calcium levels need not be measured routinely, except in patients who have not had previ-ously documented normocalcemia or have a family history of hypercalcemia to rule out FHH. In patients with FHH, 24-hour urinary calcium excretion is characteristically low (<100 mg/d). Furthermore, the serum calcium-to-creatinine clearance ratio (24-hour urine calcium/plasma total calcium/24-hour urine creatinine/plasma creatinine) usually is <0.01 in patients with FHH, whereas it is typically >0.02 in patients with PHPT, although there are exceptions to this. Other biochemical features of PHPT are listed in Table 38-10. Elevated levels of alkaline phosphatase may be found in approximately 10% of patients with PHPT and are indicative of high-turnover bone disease. These patients are prone to developing postoperative hypocal-cemia due to bone hunger. Serum and urine protein electropho-resis may be necessary to exclude multiple"}, {"id": "pubmed23n1030_20957", "title": "Hypercalcemia as an Early Finding of Opportunistic Fungal Pneumonia in Renal Transplantation: A Case Series Report.", "score": 0.009523809523809525, "content": "Pneumonia caused by opportunistic fungi is a serious complication in immunocompromised patients. Hypercalcemia has been described in renal transplantation associated with Pneumocystis jirovecii (PJP) or Histoplasma capsulatum (HCP) pneumonia. We describe 5 patients who underwent kidney transplant between 2014 and 2019 and developed hypercalcemia before the diagnosis of pulmonary fungal infection: 4 patients with PJP and 1 with HCP. We assessed calcium metabolism and kidney function by total and ionized calcium, phosphorus, intact parathormone (iPTH), 25-OH vitamin D, 1,25(OH)2 vitamin D, and serum creatinine levels. Mean albumin-corrected calcium and ionized calcium were 12.56 mg/dL (range, 10.8-13.8 mg/dL) and 1.57 mmol/L (range, 1.43-1.69 mmol/L). Patients were normocalcemic, at 10.12 mg/dL (range, 9.6-10.5 mg/dL), before diagnosis and resolved hypercalcemia after antifungal treatment, at 8.86 mg/dL (range, 8.0-9.5 mg/dL). All patients had low or normal iPTH values, at 29.1 pg/mL (range, &lt;3-44 pg/mL), with higher PTH levels 3 months before diagnosis and after treatment, at 147.3 pg/mL (range, 28.1-479 pg/mL) and 117.5 pg/mL (range, 18.2-245 pg/mL), respectively. The mean value for 25-OH vitamin D was 30.8 ng/mL (range, 14.6-62.8 ng/mL). This supports a PTH-independent mechanism, and we postulated an extrarenal production of 1,25(OH)2 vitamin D. In kidney transplant patients, hypercalcemia independent of PTH and refractory to treatment should alert for the possibility of opportunistic fungal pneumonia."}, {"id": "pubmed23n0361_20850", "title": "[Hypophosphatemic rickets].", "score": 0.009523809523809525, "content": "Familiar hypophosphatemic rickets (X-linked hypophosphataemia) occurs very rarely, nevertheless it makes a significant medical problem because of the fact that misdiagnosed or late diagnosed leads to physical development retardation and to severe deformities of extremities. In the case of severe deformities the surgical treatment is needed. We present the case of the 5 year old girl with X-linked hypophosphataemia, who was late diagnosed, in spite of appearance of typical clinical symptoms (short stature and deformities of extremities) and typical laboratory findings (severe hypophosphataemia, serum calcium levels at the lower normal range). This girl had been \"observed\" (by a family doctor) for 3 years because of \"varus deformity of legs\". Despite considerably abnormal developmental parameters: height and weight &lt;&lt; 3rd percentile (height &gt;-3 s.d. for age), length of lower limbs &gt;-3 s.d. for age and abnormalities in X-ray images of lower limbs (at the age of 3 5/12 presence of diaphysial deformations and abnormal metaphyses with zones of inadequate mineralization) the girl had not been diagnosed. After the admission to The Department of Pediatrics, Endocrinology and Disease of Adolescents: TRP 69.1%, PO4- 0.7-0.78 mmol/l, Ca 2.24-2.25 mmol/l, Mg 0.78 mmol/l, normal serum level of 25(OH)D (15.4 ng/ml [n. 11-54]), reduced serum level of 1 alpha 25(OH)2D (5.2 pg/ml [n. 15-70]), PTH 56 pg/ml [n. 10-70], ALP 401 U/l, ACP 9.5 U/l. 24-hour urine: PO4- 0.36-0.60 mmol/kg b.w., Ca 0.021 mmol/kg b.w. Probably, the girl will need surgical treatment because of severe bone deformities."}, {"id": "article-23201_40", "title": "Primary Hyperparathyroidism -- Evaluation", "score": 0.009474021551896809, "content": "Patients on known hypercalcemic drugs, such as lithium and thiazide, should have these medications stopped for 3 to 6 months, if possible, and their serum calcium & parathyroid hormone levels retested. Patients with low vitamin D levels should receive supplements to maintain at least 30 mg/mL. If parathyroid hormone levels are still elevated, this suggests hyperparathyroidism. Familial hypocalciuric hypercalcemia can be excluded by a 24-hour urine calcium determination (<100 mg calcium/24  hours) or by a low calcium/creatinine excretion ratio [(urinary calcium x serum creatinine) / (serum calcium x urinary creatinine)] typically <0.02. [88] It may present as early as age 30 and virtually never after age 50. [88] [89]"}, {"id": "pubmed23n1076_18599", "title": "Safety of megadose of vitamin D in patients with nephrolithiasis.", "score": 0.009433962264150943, "content": "This article describes two patients with renal lithiasis who received a megadose of 25-hydroxy vitamin D (25[OH]D) and had a good outcome. The first case reports a 74-year-old man with a long-term history of renal lithiasis and about four episodes of renal crisis. He was treated once with extracorporeal shock wave lithotripsy. He also had a history of dyslipidemia, myocardial infarction, and stroke. Laboratory tests demonstrated 25(OH)D of 28 ng/mL (normal range (nr): &gt;30 ng/mL), normal lipid levels, creatinine of 1.1 mg/dL, and homocysteine of 26.6 mcmol/L (nr: 5-15 mcmol/L); parathyroid hormone (PTH) was high at 67.3 pg/mL (nr: 10-65 pg/mL), serum total calcium was 8.6 mg/dL, 24-h urinary calcium was 139 mg/d (normal range 100-300 mg/d), and urinary sediment was normal. He received 50 000 IU per week of vitamin D for 3 mo, and 25(OH)D increased to 36.6 ng/mL. Urinary calcium was 142 mg/d, PTH was 46.7 pg/mL, and serum calcium was 9.6 mg/dL. No renal crisis was perceived. He asked for an alternative form of medication since he usually would forget to take drugs. Vitamin D in a single dose of 600 000 IU intramuscular was prescribed. He was asked to increase water intake to 2 to 3 L/d. After 3 mo his 25(OH)D was 75.0 ng/mL, serum calcium was 9.2 mg/dL, urinary calcium was 148 mg/d, and PTH was 38.7 pg/mL. He had no episodes of lithiasis renal crisis. Folic acid and methylcobalamin were added, and homocysteine normalized. At follow-up 3 y later, the patient was asymptomatic, cardiologic evaluation was stable without any other renal lithiasis crises, 25(OH)D continued to be normal at 62 ng/mL, and he received a megadose of vitamin D every 6 mo. Renal ultrasound revealed only microlithiasis. The second case reports a 52-year-old man with a long-term history of renal lithiasis experienced since he was 30 y old, with three renal crisis episodes. He was treated with an extracorporeal shock wave three times. Laboratory tests demonstrated 25(OH)D 18 ng/mL, normal biochemistry, total serum calcium of 10.2 mg/dL, 24-h urinary calcium of 154 mg/d, and normal urinary sediment. He received 50 000 IU per week of 25(OH)D for 3 mo, and 25(OH)D increased to 40.3 ng/mL. Urinary calcium was 167 mg/d, PTH was 35.3 pg/mL, and serum calcium was 10.1 mg/dL. No renal crisis was perceived. He asked for an alternative form of medication, and vitamin D in a single dose of 600 000 IU intramuscular was prescribed. He was asked to increase water intake to 2 to 3 L/d. After 3 mo, his 25(OH)D was 82.0 ng/mL, serum calcium was 9.6 mg/dL, urinary calcium was 175 mg/d, and PTH was 35.3 pg/mL. The renal ultrasound was unchanged. He had no episodes of lithiasis renal crisis. At follow-up 4 y later, the patient was asymptomatic without any other renal lithiasis crises, a renal ultrasound revealed a reduction of calculi size to microlithiasis, 25(OH)D continues normal, and he received a megadose of this vitamin every 4 mo. To the best of our knowledge, this is the first description of a megadose of vitamin D used in patients with nephrolithiasis. Furthermore, this shows the safety of this strategy in patients without hypercalciuria."}, {"id": "pubmed23n0103_9753", "title": "Parenteral nutrition for infants: effect of high versus low calcium and phosphorus content.", "score": 0.009345794392523364, "content": "Calcium (Ca) and phosphorus (P) homeostasis were determined in 18 infants (birth weight, 2,810 +/- 135 g; gestational age, 37.4 +/- 0.5 weeks; mean +/- SEM) who received high or low Ca and P content (Ca, P) parenteral nutrition (PN) with a fixed, low dose of vitamin D (25 IU/dl). Nine infants were randomized into low (standard) Ca, P (20 mg Ca and 15.5 mg P/dl) and nine into high Ca, P (60-80 mg Ca and 46.5-62 mg P/dl) PN, and then were studied for up to 6 weeks. The high Ca, P group had stable serum 1,25 dihydroxyvitamin D [1,25(OH)2D], which consistently remained within the normal range (less than 116 pg/ml). Tubular reabsorption of phosphorus (TRP) also was stable and remained consistently less than 90%. The low Ca, P group had elevated and higher 1,25(OH)2D (p = 0.03) than the high Ca, P group. The mean serum 1,25(OH)2D concentration rose from 32 to 112, 115, and 133 pg/ml over a period of 6 weeks. TRP also was higher (p = 0.02) and remained consistently greater than 90%. There were no significant differences between groups in serum parathyroid hormone, calcitonin, Ca, Mg, P, alkaline phosphatase, vitamin D binding protein, and 25 hydroxyvitamin D concentrations; urine Ca/creatinine and Mg/creatinine ratios, and fractional excretion of sodium (Na). Thus, a \"high\" Ca (60 mg/dl) and P (46.5 mg/dl) content in PN solutions can result in stable serum 1,25(OH)2D and TRP, presumably reflecting minimal stress to Ca and P homeostatic mechanisms without further increase in urinary Ca excretion."}, {"id": "pubmed23n0971_20137", "title": "Secondary Hyperparathyroidism in Patients with Biliopancreatic Diversion After 10 Years of Follow-up, and Relationship with Vitamin D and Serum Calcium.", "score": 0.009259259259259259, "content": "Secondary hyperparathyroidism (SHPT) is a matter of concern after biliopancreatic diversion (BPD). The aim of this study was to investigate the relationship between SHPT, 25(OH)D, and calcium after BPD. A retrospective analysis in obese patients after BPD performed between 1998 and 2016. Patients with at least 1 year of follow-up were included. SHPT was considered when PTH &gt; 65 pg/mL in the absence of an elevated corrected calcium. 25(OH)D (ng/mL) status was defined as: deficiency &lt; 20, insufficiency 20-29.9, and sufficiency ≥ 30. In total, 321 patients were included (76.6% women), with mean age 43.0 (10.5) years. Median follow-up was 6.0 (IQR 3.0-9.0) years. Mean body mass index was 49.8 (7.0) kg/m<sup2</sup. SHPT increased to a maximum of 81.9% in the ninth year of follow-up (95% CI: 1.5-9.1). Two years after surgery, 33.9% of patients with 25(OH)D sufficiency had SHPT (p = 0.001). Corrected calcium levels were lower in patients with PTH &gt; 65 pg/mL when compared with PTH &lt; 65 pg/mL; 1 year: 8.96 vs 9.1 mg/dL and 5 years: 8.75 vs 9.12 mg/dL (p &lt; 0.01). After surgery, patients with PTH &gt; 65 pg/mL and 25(OH)D sufficiency had lower corrected calcium levels when compared with subjects with PTH and 25(OH)D in normal range. Two years: 9.0 vs 9.2 mg/dL (p &lt; 0.05) and 4 years: 8.9 vs 9.2 mg/dL (p &lt; 0.01). Once 25(OH)D is sufficient, the increase in PTH persists associated with a decrease in serum corrected calcium. It is important to ensure a sufficient calcium intake in these patients in order to avoid SHPT and osteomalacia in the future."}, {"id": "article-21613_10", "title": "Familial Hypocalciuric Hypercalcemia -- Evaluation", "score": 0.009259259259259259, "content": "The differentiation between FHH and primary hyperparathyroidism is more difficult in the absence of a family history of hypercalcemia if PTH levels are normal and if the Ca/Cr clearance ratio is greater than 0.01 and less than 0.02. The age at diagnosis of hypercalcemia and family history is important. Detection of asymp­tomatic hypercalcemia before the age of 40 years or so favors the diagnosis of FHH. Obtaining serum calcium values from first-degree relatives in the absence of family history can be helpful."}, {"id": "pubmed23n0095_3475", "title": "Evidence of a probable role for 25-hydroxyvitamin D in the regulation of human calcium metabolism.", "score": 0.009174311926605505, "content": "1,25-Dihydroxyvitamin D [1,25-(OH)2D] is the principal mediator of the biologic effects of vitamin D. We showed previously that obese white subjects have low serum vitamin D and 25-hydroxyvitamin D (25-OHD) with increased serum-immunoreactive parathyroid hormone (PTH) and 1,25-(OH)2D, low urinary calcium, and increased urinary cyclic adenosine 3',5'-monophosphate (cyclic AMP) compared with nonobese white individuals. To determine whether 25-OHD modulates calcium metabolism, the effects of 25-OHD3, 40-100 micrograms/day for 9 days, were compared in seven obese and seven nonobese white subjects who were between the ages of 20 and 34 years. Each of them was hospitalized on a metabolic ward and given a constant daily diet that contained 400 mg calcium, 900 mg phosphate, and 18 mEq magnesium. Whereas 25-OHD3 increased mean serum 25-OHD from 7 +/- 1 to 37 +/- 5 ng/ml (P less than 0.01) and urinary calcium from 102 +/- 18 to 146 +/- 17 mg/day (P less than 0.001) and decreased mean serum 1,25-(OH)2D from 40 +/- 2 to 28 +/- 2 pg/ml (P less than 0.01) and urinary cyclic AMP from 3.23 +/- 0.57 to 2.00 +/- 0.17 nM/dl GF (P less than 0.05), it did not change mean serum calcium, ionized calcium, phosphate, magnesium, immunoreactive PTH or urinary phosphate, or creatinine clearance in the obese subjects. In contrast, 25-OHD3 increased mean serum 25-OHD from 16 +/- 1 to 46 +/- 4 pg/ml (P less than 0.001) but did not alter mean serum 1,25-(OH)2D or urinary calcium or cyclic AMP in the nonobese subjects.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0007_14242", "title": "[Evaluation of renal cyclic adenosine monophosphate, serum parathyroid hormone and phosphate reabsorption in recurrent calcium urolithiasis, healthy controls and hyperparathyroidism (author's transl)].", "score": 0.009174311926605505, "content": "In three groups (n = 12 each) of male controls (22--43 years), patients with recurring calcium urolithiasis (21--36 years) and hyperparathyroidism (HPT; 17--71 years) proven by surgery renal cyclic adenosine monophosphate (RcAMP), fractional tubular phosphate reabsorption and serum parathyroid hormone (PTH) were measured during endogenous creatinine clearance. RcAMP (muMol/g creatinine) was: controls 1.48 +/- SEM 0.27; stone formers 2.037 +/- 0.343 (not significantly different); HPT 6.234 +/- 0.454 (p less than 0.001). There is no overlap between HPT and controls. Phosphate reabsorption is least in HPT (0.84 +/- 0.015), higher in controls (0.924 +/- 0.004) and stone formers (0.941 +/- 0.007). All differences are statistically significant. Under the conditions selected (moderate hydration of individuals) Serum PHT (pg-equiv/ml) is lowest in stome formers (less than 100--339), higher in controls (less than 100--933) and HPT (400--1150). there is no overlap in PHT between the former and the latter group but a marked one between controls and HPT. For clinical purposes the resulting diagnostic uncertainty in a given patient can be overcome by additional determinations of RcAMP and ionised serum calcium: when referring to serum PTH HPT patients fall outside, RCU patients within 2 standard deviations of either parameter in control subjects. This procedure presently appears superior to those proposed in the past (urinary cAMP etc.) but requires confirmation in larger patient populations. Moreover, since HPT prevails in middle and upper age decades, their RcAMP values and those of RCU patients should be related to a range seen in closely age- and sex-matched controls."}, {"id": "pubmed23n0128_2831", "title": "Evidence for alteration of the vitamin D-endocrine system in blacks.", "score": 0.00909090909090909, "content": "As compared with values in white subjects, bone mass is known to be increased and urinary calcium to be diminished in black individuals. To evaluate the possibility that these changes are associated with alterations in the vitamin D-endocrine system, an investigation was performed in 12 black subjects, 7 men and 5 women, and 14 white subjects, 8 men and 6 women, ranging in age from 20 to 35 yr. All of them were hospitalized on a metabolic ward and were given a constant daily diet containing 400 mg of calcium, 900 mg of phosphorus, and 110 meq of sodium. Whereas mean serum calcium, ionized calcium, and phosphate were the same in the two groups, mean serum immunoreactive parathyroid hormone (350 +/- 34 vs. 225 +/- 26 pg/ml, P less than 0.01) and mean serum 1,25-dihydroxyvitamin D (1,25(OH)2D) (41 +/- 3 vs. 29 +/- 2 pg/ml, P less than 0.01) were significantly higher, and mean serum 25-hydroxy-vitamin D (25-OHD) was significantly lower in the blacks than in the whites (6 +/- 1 vs. 20 +/- 2 ng/ml, P less than 0.001). Mean urinary sodium and 24-h creatinine clearance were the same in the two groups, whereas mean urinary calcium was significantly lower (101 +/- 14 vs. 166 +/- 13 mg/d, P less than 0.01) and mean urinary cyclic AMP was significantly higher (3.11 +/- 0.47 vs. 1.84 +/- 0.25 nM/dl glomerular filtrate, P less than 0.01) in the blacks. Further, the blacks excreted an intravenous calcium load, 15 mg/kg body weight, as efficiently as the whites (49 +/- 3 vs. 53 +/- 3%, NS). Mean serum Gla protein was lower in blacks than in whites (14 +/- 2 vs. 24 +/- 3 ng/ml, P less than 0.02), and increased significantly in both groups in response to 1,25(OH)2D3, 4 micrograms/d for 4 d. There was a blunted response of urinary calcium to 1,25(OH)2D3 in the blacks, and mean serum calcium did not change. The results indicate that alteration of the vitamin D-endocrine system with enhanced renal tubular reabsorption of calcium and increased circulating 1,25(OH)2D as a result of secondary hyperparathyroidism may contribute to the increased bone mass in blacks. Their low serum 25-OHD is attributed to diminished synthesis of vitamin D in the skin because of increased pigment."}, {"id": "wiki20220301en025_72256", "title": "Hyperparathyroidism", "score": 0.009031181986453227, "content": "Diagnosis The gold standard of diagnosis is the PTH immunoassay. Once an elevated PTH has been confirmed, the goal of diagnosis is to determine whether the hyperparathyroidism is primary or secondary in origin by obtaining a serum calcium level: Tertiary hyperparathyroidism has a high PTH and high serum calcium. It is differentiated from primary hyperparathyroidism by a history of chronic kidney failure and secondary hyperparathyroidism. Hyperparathyroidism can cause hyperchloremia and increase renal bicarbonate loss, which may result in a normal anion gap metabolic acidosis. Differential diagnosis Familial benign hypocalciuric hypercalcaemia can present with similar lab changes. In this condition, the calcium creatinine clearance ratio, however, is typically under 0.01. Blood tests"}, {"id": "pubmed23n1132_7194", "title": "Occult Renal Calcifications in Patients with Normocalcemic Primary Hyperparathyroidism and Their Association with the Parathyroid Hormone-Vitamin D Axis.", "score": 0.009009009009009009, "content": "Normocalcemic primary hyperparathyroidism (NPHPT) is characterized by elevated serum levels of parathyroid hormone (PTH) with persistently normal serum calcium concentrations after excluding secondary causes of hyperparathyroidism. Urolithiasis and/or nephrocalcinosis may occur in hypercalcemic PHPT, but little is known about these complications in NPHPT. <iObjectives</i. To identify occult urolithiasis and nephrocalcinosis in asymptomatic patients with NPHPT and evaluate biochemical markers as risk predictors for the development of renal calcification (RC). <iMethods</i. Cross-sectional analysis of 34 patients with no history of urolithiasis and/or nephrocalcinosis. The diagnosis of NPHPT was as follows: elevated serum PTH (reference range: 15-65 pg/mL), normal albumin-corrected serum calcium, normal urinary calcium excretion, serum 25(OH)D &gt;30 ng/mL, eGFR (CKD-EPI) &gt; 60 mL/min/1.73 m<sup2</sup, without intestinal disease, and not on medications such as thiazide diuretics, lithium, bisphosphonates, or denosumab. Patients were categorized according to the presence or absence of RC identified by renal imaging. Their clinical and biochemical characteristics were then compared. <iResults</i. The patients had a mean age of 67.97 ± 10.45 years, predominantly postmenopausal women (88.2%); serum PTH, 119.67 ± 64.44 pg/mL; 25(OH)D, 39.00 ± 8.88 ng/dL; 1.25(OH))<sub2</subD, 74.53 ± 26.37 pg/mL; corrected serum calcium, 9.34 ± 0.62 mg/dL; and 24-hour urinary calcium, 134.87 ± 79.68 mg/day. RC was identified in 26.5% of the patients. There was no difference in anthropometric and clinical parameters, renal function, 25(OH)D, and urinary pH in patients with or without RC. Patients with RC had higher PTH values (176.22 vs. 99.32 pg/mL, <iP</i = 0.001), 1.25(OH) 2D (96.83 vs. 62.36 pg/mL, <iP</i = 0.005), and 24-hour urinary calcium (181.9 vs. 117.94 mg/day, <iP</i = 0.037). <iConclusion</i. Occult renal calcifications are common in NPHPT and are associated with increased serum PTH, 1.25(OH))<sub2</subD, and 24 h urinary calcium."}, {"id": "pubmed23n0203_5022", "title": "[Standard procedure and the diagnostic criteria for the Ellsworth-Howard test using human PTH-(1-34)].", "score": 0.009009009009009009, "content": "The present study was undertaken to establish a standard method to perform the Ellsworth-Howard test using human PTH-1-34). For this purpose we made a survey of literature concerning the Ellsworth-Howard test and then examined the data of the Ellsworth-Howard tests performed on 178 hypoparathyroid patients using human PTH-(1-34). The main items of investigation were: (i) to determine the appropriate dose of PTH for administration to adults and children; and (ii) to define the criteria of practical usefulness for the differential diagnosis of the types of hypoparathyroidism. From the analysis of the data, the following findings and conclusions were obtained. The dose of human PTH-(1-34) appropriate for diagnostic use is 100 U per person for adults and 100 U per body surface area of one square meter (100 U/m2) for children. The criteria of positive response in the Ellsworth-Howard test are defined as follows. a) phosphaturic response: (U4 + U5) - (U2 + U3) = more than 35 mg/2 h b) cyclic AMP response: U4 - U3 = more than 1 mumol/h, and U4/U3 = more than 10 times. In the above formula, U2 - U5 represent the urine samples collected hourly in order. PTH is injected at the time between U3 and U4. For the application of the criteria in children, one should use the values corrected for body surface area of one square meter. It is necessary to confirm the following conditions before the application of the criteria: the presence of hypocalcemia and hyperphosphatemia; the lack of phosphate deficiency (basal urinary phosphate excretion more than 10 mg/2 h); the accuracy of timed urine collections (ratio of creatinine excretion during 2 hours before PTH to that after PTH administration in the range from 0.8 to 1.2); and the absence of marked diurnal variation in phosphate excretion (difference in phosphate excretion between the two basal hourly urine less than 17.5 mg/h). To ensure the above conditions, medications such as phosphate-binding antacids should be withheld for at least 1 week before the test, and the test should be performed according to the standard procedure described in this paper. The diagnosis of pseudohypoparathyroidism Type II should be done cautiously. It is necessary to take account of the high basal urinary cyclic AMP excretion and the elevated serum PTH level along with the results of the Ellsworth-Howard test (positive cyclic AMP response and negative phosphaturic response) for a definite diagnosis of this entity."}, {"id": "wiki20220301en262_2352", "title": "Familial hypocalciuric hypercalcemia", "score": 0.008933022272736336, "content": "Familial hypocalciuric hypercalcemia (FHH) is an inherited condition that can cause hypercalcemia, a serum calcium level typically above 10.2 mg/dL. It is also known as familial benign hypocalciuric hypercalcemia (FBHH) where there is usually a family history of hypercalcemia which is mild, a urine calcium to creatinine ratio <0.01, and urine calcium <200 mg/day. Signs and symptoms Most cases of familial hypocalciuric hypercalcemia are asymptomatic. Laboratory signs of FHH include: High blood levels of calcium (hypercalcemia) A low amount of calcium excreted in the urine ( Ca excretion rate < 0.02 mmol/L) High blood levels of magnesium (hypermagnesemia) High normal to mildly elevated parathyroid hormone Causes Types include:"}, {"id": "wiki20220301en116_31394", "title": "X-linked hypophosphatemia", "score": 0.008932123816344395, "content": "Diagnosis Begin clinical laboratory evaluation of rickets with assessment of serum calcium, phosphate, and alkaline phosphatase levels. In hypophosphatemic rickets, calcium levels may be within or slightly below the reference range; alkaline phosphatase levels will be significantly above the reference range. Carefully evaluate serum phosphate levels in the first year of life, because the concentration reference range for infants (5.0-7.5 mg/dL) is high compared with that for adults (2.7-4.5 mg/dL). Serum parathyroid hormone levels are within the reference range or slightly elevated, while calcitriol levels are low or within the lower reference range. Most importantly, urinary loss of phosphate is above the reference range. The renal tubular reabsorption of phosphate (TRP) in X-linked hypophosphatemia is 60%; normal TRP exceeds 90% at the same reduced plasma phosphate concentration. The TRP is calculated with the following formula:"}, {"id": "InternalMed_Harrison_28586", "title": "InternalMed_Harrison", "score": 0.008930669800235018, "content": "based on the double-antibody method for PTH exhibit very high sensitivity (especially if serum calcium is simultaneously evaluated) and specificity for the diagnosis of primary hyperparathyroidism (Fig. 424-4). In summary, PTH values are elevated in >90% of parathyroid-related causes of hypercalcemia, undetectable or low in malignancy-related hypercalcemia, and undetectable or normal in vitamin D– related and high-bone-turnover causes of hypercalcemia. In view of the specificity of the PTH immunoassay and the high frequency of hyperparathyroidism in hypercalcemic patients, it is cost-effective to measure the PTH level in all hypercalcemic patients unless malignancy or a specific nonparathyroid disease is obvious. False-positive PTH assay results are rare. Immunoassays for PTHrP are helpful in diagnosing certain types of malignancy-associated hypercalcemia. Although FHH is parathyroid-related, the disease should be managed distinctively from hyperparathyroidism. Clinical features and"}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 83]], "word_ranges": [[0, 14]], "text": "CT scan is part of the diagnostic workup in case there is biochemical confirmation."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "CT scan is part of the diagnostic workup in case there is biochemical confirmation.", "full_answer_no_ref": "CT scan is part of the diagnostic workup in case there is biochemical confirmation.", "full_question": "A 58-year-old male with a history of arterial hypertension of 6 years of evolution, who consults for poor control of blood pressure figures despite receiving treatment with an angiotensin-converting enzyme inhibitor, a diuretic and a calcium antagonist. On consultation she presented with blood pressure of 149/100 mmHg. Laboratory tests: creatinine 1.2 mg/dl, potassium 2.2 mEq/l and compensated metabolic alkalosis; the rest of the biochemical study, blood count, coagulation and urinary sediment were normal. Mark the correct statement:", "id": 548, "lang": "en", "options": {"1": "The origin of hypertension in this case is excessive aldosterone secretion caused by autonomic hyperfunction of the adrenal medulla.", "2": "In most cases the anatomical substrate is bilateral hyperplasia of the adrenal cortex.", "3": "CT scan is part of the diagnostic study in case of biochemical confirmation.", "4": "Spironolactone is contraindicated in the management of this pathology.", "5": NaN}, "question_id_specific": 126, "type": "CARDIOLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0640_19248", "title": "Hypokalemia induced myopathy as first manifestation of primary hyperaldosteronism - an elderly patient with unilateral adrenal hyperplasia: a case report.", "score": 0.01906318082788671, "content": "Primary hyperaldosteronism is only rarely caused by unilateral adrenal hyperplasia. A 73-year-old hypertensive Greek man (on 10 mg amlodipine for the last ten years) presented in the emergency department with severe muscle weakness of all limbs. The initial physical and laboratory examination revealed normal blood pressure, muscle weakness, severe hypokalemia, sinus rhythm and U wave, rhabdomyolysis and metabolic alkalosis. The patient was immediately treated with intravenous administration of potassium-rich solutions, 25 mg spironolactone with progressive dose titration up to 100 mg. Because of high arterial blood pressure, irbesartan was added. On day 6, muscle weakness was completely restored with decrease of arterial blood pressure and further improvement of laboratory tests. The combination of hypokalemia with arterial hypertension raised the suspicion of primary hyperaldosteronism; therefore, we performed abdomen computed tomography scan, which revealed a nodular mass (15 mm in diameter) in the left adrenal gland. Plasma renin activity was in the lower normal range with a three-fold increase of plasma aldosterone concentration. We performed total resection of the left adrenal gland and the histopathological examination revealed hyperplasia of the left adrenal gland. This report presents a rare case of an elderly patient under antihypertensive treatment the last ten years for essential hypertension, who admitted to our emergency department with hypokalemia - induced myopathy as first manifestation of primary hyperaldosteronism due to unilateral adrenal hyperplasia."}, {"id": "pubmed23n0413_13366", "title": "[Primary aldosteronism and pregnancy: report of 2 cases].", "score": 0.015763546798029555, "content": "Based on two patients, we discuss the difficulties in diagnosing and managing primary aldosteronism in pregnancy, which derive from changes of the renin-angiotensin-aldosterone axis, from the uncertainty regarding blood pressure control along gestation and postpartum, and from the contraindication to the use of spironolactone. The first case is a 27 years old woman with a long standing refractory hypertension, a hemorrhagic stroke with left brachial hemiplegia and crural hemiparesia, two miscarriages, one stillbirth and one offspring with intrauterine growth retardation. Due to hypokalemia, a plasma aldosterone/renin activity ratio of 91, and a negative genetic screening for glucocorticoid remediable aldosteronism (GRA), a primary hyperaldosteronism with normal adrenals in CT scan was diagnosed, and good blood pressure control was attained with spironolactone. After two and a half years of normotension, a fifth pregnancy, managed with methyldopa evolved with satisfactory blood pressures, plasma potassium, fetal growth, uterine and umbilical arterial resistance indexes, and maternal endothelial function. At 37 1/2 weeks of pregnancy the patient delivered a healthy newborn weighing 2,960 g. Blood pressure rose during the 48 hours of postpartum in the absence of proteinuria and required i.v. hydralazine. The second patient is a 37 years old woman, with known refractory hypertension for 7 years, hypokalemia, plasma aldosterone/renin activity ratio greater than 40, normal adrenals in the CAT scan, and a negative genetic screening for GRA. She had normotensive pregnancies 5 and 3 years prior to the detection of hypertension, with hypertensive crisis in both postpartum periods, retrospectively considered as expressions of primary hyperaldosteronism."}, {"id": "pubmed23n0912_14559", "title": "Diagnosis and management of primary aldosteronism.", "score": 0.015668481548699334, "content": "Primary aldosteronism (PA) is the most common form of secondary hypertension (HTN), with an estimated prevalence of 4% of hypertensive patients in primary care and around 10% of referred patients. Patients with PA have higher cardiovascular morbidity and mortality than age- and sex-matched patients with essential HTN and the same degree of blood pressure elevation. PA is characterized by an autonomous aldosterone production causing sodium retention, plasma renin supression, HTN, cardiovascular damage, and increased potassium excretion, leading to variable degrees of hypokalemia. Aldosterone-producing adenomas (APAs) account for around 40% and idiopathic hyperaldosteronism for around 60% of PA cases. The aldosterone-to-renin ratio is the most sensitive screening test for PA. There are several confirmatory tests and the current literature does not identify a \"gold standard\" confirmatory test for PA. In our institution, we recommend starting case confirmation with the furosemide test. After case confirmation, all patients with PA should undergo adrenal CT as the initial study in subtype testing to exclude adrenocortical carcinoma. Bilateral adrenal vein sampling (AVS) is the gold standard method to define the PA subtype, but it is not indicated in all cases. An experienced radiologist must perform AVS. Unilateral laparoscopic adrenalectomy is the preferential treatment for patients with APAs, and bilateral hyperplasia should be treated with mineralocorticoid antagonist (spironolactone or eplerenone). Cardiovascular morbidity caused by aldosterone excess can be decreased by either unilateral adrenalectomy or mineralocorticoid antagonist. In this review, we address the most relevant issues regarding PA screening, case confirmation, subtype classification, and treatment."}, {"id": "pubmed23n0818_17244", "title": "[High doses of aldosterone antagonist is a condition of sufficient blood pressure control in bilateral adrenal hyperplasia].", "score": 0.015073212747631352, "content": "Primary aldosteronism occurs in 1-10% of hypertensive patients and is classified in adenomas or bilateral adrenal hyperplasia. Computed tomography (CT) or magnetic resonance imaging can be used to discriminate these subtypes and in guiding treatment selection. This case report describes a 65-year-old man with hypertension and hypokalaemia during 25 years. Bilateral adrenal hyperplasia was diagnosed based on a CT, and an oral sodium-loading test with measurement of renin and aldosterone confirmed the diagnosis. Blood pressure and potassium in plasma was normalized during treatment with the mineralocorticoid receptor antagonist eplerenon. "}, {"id": "pubmed23n0422_20006", "title": "[Reninoma: a rare but curable cause of high blood pressure, a case report].", "score": 0.015003030915336432, "content": "We report a case of a renin secreting tumor, which is a very rare cause of secondary high blood pressure. A 22-year-old woman was hospitalised for exploration of high blood pressure (160/110 mmHg) with severe hypokaliemia (2,7 mmol/l) and secondary hyperaldosteronism. Physical examination was normal except the high blood pressure. Bioassays show increased kaliuresis (66 mmol/24h), plasma renin (89 pg/ml in clinostastism--108 pg/ml in orthostatism), pro-renin (1207 pg/ml in clinostastism--1412 pg/ml in orthostatism) and aldosterone (210 pg/ml in clinostastism--566 pg/ml in orthostatism). The rest of the endocrine tests were normal (cortisol and ACTH at 8:00 am, urinary free cortisol, overnight 1 mg dexamethasone suppression test). Doppler ultrasound method, performed by an experienced radiologist, did not show renal artery stenosis. Abdominal computerized tomography showed a nodular formation at the upper pole of the right kidney, isodense to renal medullary. The size tumor was 15 mm. The renal vein sampling shows high values of renin on both sides whereas, for the pro-renin, the values were higher on the tumor side. In spite of treatment with CEI (Converting Enzyme Inhibitors) and calcium antagonists, the blood pressure was not controlled. Hypokaliemia persisted (3 mmol/l) in spite of high daily potassium intake (64 mmol/l of potassium chloride). After tumor resection, reninoma was diagnosed by the pathology examination and blood pressure, plasma rennin, plasma aldosterone level returned to normal."}, {"id": "pubmed23n0562_10424", "title": "[Clinical characteristics and surgery outcomes of unilateral nodular adrenal hyperplasia in primary aldosteronism: study of 145 cases].", "score": 0.014756258234519103, "content": "To investigate the clinical characteristics, differential diagnosis, and surgery outcome of unilateral nodular adrenal hyperplasia (UNAH). The clinical data of 145 patients with primary aldosteronism, 67 males and 78 females, aged 37.9 (19-60), including 78 cases of aldosterone-producing adenoma (APA), 14 cases of UNAH, and 55 cases of idiopathic bilateral adrenal hyperplasia (BAH), were collected. Radioimmunoassay was used to examine the blood and urine aldosterone and plasma rennin activity. Automatic biochemical apparatus was used to examine the blood and urine electrolytes, renal functions, and urine microalbumin. Twelve-lead electrocardiography, echocardiography, and plain scanning of enhanced CT scanning of the bilateral adrenals were conducted. Adrenal venous sampling (AVS) was conducted in 62 patients to collect blood samples from vena cava and bilateral suprarenal veins to detect the levels of aldosterone and cortisol. All UNAH patients and 3 BAH patients underwent unilateral adrenalectomy and three APA patients underwent unilateral adrenalectomy or adenoma resection. Then the patients were followed up for 39.2 months. The incidence of UNAH is 9.7% in the primary aldosteronism patients. There were no significant differences in age, gender, duration of hypertension, blood pressure (SBP, DBP), and indexes indicating damages in target organs of hypertension (left ventricular hypertrophy rate, blood creatinine, urine microalbumin, etc) among these three groups. The level of serum potassium of the APA group was significantly lower than that of the BAH group (P &lt; 0.01), and the levels of plasma and urine aldosterone of the APA group were significantly higher than those of the BAH group (P &lt; 0.05 and P &lt; 0.01). The serum potassium of the UNAH group was higher than that of the APA group and lower than that of the BAH group, and the levels of plasma and urine aldosterone of the UNAH group were both higher than those of the APA group and lower than those of the BAH group, however all not significantly (all P &gt; 0.05). The coincidence rate of CT was 50% (7/14) in the UNAH group. The accuracy of AVS for diagnosis of UNAH was 85.7% (12/14). After operation, the serum potassium and plasma aldosterone concentrations returned normal in all the UNAH patients. Blood pressure returned to normal in 50% (7/14) of the UNAH patients, and was improved in the other 50% (7/14) patients. UNAH can be cured by adrenal surgery. The diagnostic values of clinical examination and adrenal CT are limited. AVS is essential in diagnosing UNAH patients."}, {"id": "pubmed23n0547_4857", "title": "[Modern pharmacological aspects of hyperaldosteronism therapy].", "score": 0.014110965487112277, "content": "The prevalence of primary hyperaldosteronism is 5-10% of all hypertensive patients, and clearly above the estimated prevalence in the past. In nearly 30% of patients with therapy resistant hypertension, primary hyperaldosteronism is detected if they are investigated thoroughly. This will result in 1.5 to 2.5 million people in Germany suffering from primary hyperaldosteronism. Besides efficient diagnostic procedures, an effective treatment is of increasing importance. The aldosterone-producing adenoma (Conn's syndrome) is primarily cured by operation, in most cases performed endoscopically. Bilateral hyperplasia, which is found in two-thirds of primary hyperaldosteronism, is treated primarily by mineralocorticoid receptor antagonist: 12.5-50 mg/day spironolactone (in case of anti-androgenic side-effects alternatively by 50-100 mg/day eplerenone). If the blood pressure can not be lowered by this first-line treatment, an additional treatment with potassium-sparing diuretics, calcium-antagonists, ACE-inhibitors or angiotensin-2-antagonists is necessary. The start of medication should be closely monitored by serum electrolyte and creatinine controls."}, {"id": "wiki20220301en083_35405", "title": "Apparent mineralocorticoid excess syndrome", "score": 0.013878406708595387, "content": "Diagnosis Other conditions such as Liddle's Syndrome can mimic the clinical features of AME, so diagnosis can be made by calculating the ratio of free urinary cortisol to free urinary cortisone. Since AME patients create less cortisone, the ratio will much be higher than non-affected patients. Alternatively, one could differentiate between the two syndromes by administering a potassium-sparing diuretic. Patients with Liddle's syndrome will only respond to a diuretic that binds the ENaC channel, whereas those with AME will respond to a diuretic that binds to ENaC or the mineralcorticoid receptor. Treatment The treatment for AME is based on the blood pressure control with Aldosterone antagonist like Spironolactone which also reverses the hypokalemic metabolic alkalosis and other anti-hypertensives. Renal transplant is found curative in almost all clinical cases.AME is exceedingly rare, with fewer than 100 cases recorded worldwide."}, {"id": "pubmed23n1115_3220", "title": "[Chronic kidney disease after adrenalectomy in a patient with primary aldosteronism].", "score": 0.013817663817663818, "content": "We report one case of estimated glomerular filtration rate (eGFR) decline after taking unilateral adrenalectomy due to aldosterone adenoma. A 60-year-old male with 23-year history of hypertension was reported to the endocrinologist due to hypokalemia (serum potassium 3.01 mmol/L). Urine microalbumin/creatinine (ALB/CR) was 70.15 mg/g, serum creatinine was 82 μmol/L and eGFR was 89.79 mL/(min·1.73 m<sup2</sup). Random serum aldosterone was 172.2-203.5 ng/L, and random plasma rennin activity was 0-0.17 μg/(L·h). His captopril challenge test suggested that his aldosterone le-vels were suppressed by 8% (&lt; 30%) and the adrenal enhanced computed tomography scan revealed a left adrenal tumor. The patient was diagnosed with primary hyperaldosteronism (PA), aldosterone adenoma and underwent left laparoscopic adrenalectomy. Histological examination confirmed adrenal cortical adenoma. One week after the operation, his serum creatinine was increased to 127 μmol/L compared with preoperative level; eGFR was 32.34 mL/(min·1.73 m<sup2</sup). His systolic blood pressure (SBP) was 110 mmHg and diastolic blood pressure (DBP) was 60 mmHg (hypotensive drugs discontinued), and serum potassium level was 5.22 mmol/L. At the end of the 2-year follow up, the serum creatinine of this patient remained at 109-158 μmol/L and eGFR fluctuated from 63.28-40.12 mL/(min·1.73 m<sup2</sup). PA is one of the most common causes of secondary hypertension. Several studies have reported renal function deterioration of PA patients after unilateral adrenalectomy, like the patient in this article. Age, preoperative plasma aldosterone concentration, albuminuria and preoperative potassium level might be significant predictors of a decrease in the eGFR. Growing evidence suggests that aldosterone could contribute to structural kidney damage, arterial injury and hemodynamic disorder. At the same time, patients with PA exhibit glomerular hyperfiltration and glomerular vascular hypertension, leading to the misinterpretation of renal function in PA patients as subtle kidney damage may be masked by the glomerular hyperfiltration before treatment. After a unilateral adrenalectomy, glomerular hyperfiltration by aldosterone excess is resolved and renal damage can be unmasked. In conclusion, kidney function deterioration after adrenalectomy can be detected in some patients with PA. Thus, accurate evaluation of kidney function in patients with PA may be essential, especially for those with preoperative risk factors for postoperative renal impairment. After unilateral adrenalectomy, close monitoring of renal function and adequate management are required for PA patients."}, {"id": "pubmed23n0316_19475", "title": "[Primary hyperaldosteronism. Apropos of 2 cases].", "score": 0.01375534188034188, "content": "Primary hyperaldosteronism (PHA) represents less than 1 to 2% of all causes of hypertension (HT). We report 2 cases of primary hyperaldosteronism which emphasize the difficulty of distinguishing neoplastic PHA from idiopathic PHA, observed in a 60-year-old woman and a 42-year old woman, respectively. In both cases, the diagnosis of PHA was suggested by marked hypokalaemia with inappropriate potassium excretion and was confirmed by hyperaldosteronaemia and low and poorly stimulated renin activity. In the first case, computed tomography showed nodular hyperplasia of the 2 adrenal glands. The patient was treated with spironolactone and calcium channel blockers which controlled blood pressure and serum potassium. In the second case, computed tomography and magnetic resonance imaging revealed an adrenocortical adenoma confirmed by pathological examination after the operation. The diagnosis of primary hyperaldosteronism is based on three steps: detection, positive diagnosis and aetiological diagnosis. Detection is essentially based on demonstration of hypokalaemia. Positive diagnosis is based on demonstration of elevated aldosterone secretion with inhibited renin secretion. The aetiological diagnosis is dominated by the differentiation between Conn's adenoma and bilateral adrenal hyperplasia, which has therapeutic implications."}, {"id": "wiki20220301en104_37759", "title": "Pseudohyperaldosteronism", "score": 0.013663967611336033, "content": "Pseudohyperaldosteronism (also pseudoaldosteronism) is a medical condition which mimics the effects of elevated aldosterone (hyperaldosteronism) by presenting with high blood pressure (hypertension), low blood potassium levels (hypokalemia), metabolic alkalosis, and low levels of plasma renin activity (PRA). However, unlike hyperaldosteronism, this conditions exhibits low or normal levels of aldosterone in the blood. Causes include genetic disorders (e.g. Apparent mineralocorticoid excess syndrome, Liddle's syndrome, and types of Congenital adrenal hyperplasia), acquired conditions (e.g. Cushing's syndrome and mineralocorticoid-producing adrenal tumors), metabolic disorders, and dietary imbalances including excessive consumption of licorice. Confirmatory diagnosis depends on the specific root cause and may involve blood tests, urine tests, or genetic testing; however, all forms of this condition exhibit abnormally low concentrations of both plasma renin activity (PRA) and plasma"}, {"id": "pubmed23n0377_3962", "title": "Conn's syndrome and bilateral renal artery stenosis in the presence of multiple renal arteries.", "score": 0.013621142184813105, "content": "We report the case of a 42-year-old male who was admitted to our hospital after an acute hypertensive crisis despite four-way anti-hypertensive therapy. The renal scintigraphy, the excretory urogram and the biochemical profile performed two years before were unremarkable, except for slightly elevated serum creatinine and plasma aldosterone, in presence of normal aldosterone/renin ratio. The renal arterial angiography that was performed despite a second unremarkable scintigraphy revealed high-grade bilateral arterial stenosis in the presence of multiple renal arteries. Following dilatation of the left stenosis, the aldosterone/renin ratio was pathologic. Recumbent and orthostatic aldosterone values were 830 pg/ml and 1824 pg/ml, respectively, and recumbent and orthostatic renin values were 0.82 and 1.21 ng angiotensin I/ml/h, respectively. The abdominal computed tomography performed to investigate a possible concomitant Conn's syndrome resulted in the detection of a left adrenal tumor. After resection of the lesion, plasma-aldosterone levels normalized and a pronounced rise in serum potassium levels was observed. Following angioplasty of the right renal artery stenosis, blood pressure could easily be managed with combined beta and calcium channel blocker therapy. Particularly in cases of bilateral (but also in the presence of unilateral) renal artery stenosis in association with Conn's syndrome, all the available screening methods for these disorders can fail. In cases of poor response to combination hypertensive therapies, renal arteriography and a fludrocortisone-suppression test should be performed in order to rule out both renal arterial stenosis and Conn's syndrome, even in the absence of clinical and biochemical findings suspicious for either disorder."}, {"id": "pubmed23n0742_19388", "title": "[Primay hyperaldosteronism--diagnostic and treatment].", "score": 0.013469387755102041, "content": "Primary hyperaldosteronism (PHA) is characterized by an increased Aldosterone synthesis which is independent of the Renin-Angiotensin-Aldosterone-System (RAAS). The prevalence of PHA in patients who present in specialized hypertension centers is approx. 10 %. Besides patients with the classical symptoms known as \"Conn-Trias\" (hypertension, hypokalemia, metabolic alkalosis), the more frequent normokalemic patients with PHA also show a worse outcome compared to patients with essential hypertension. Identifying these patients is an important task in the evaluation of hypertension since targeted treatment options are available. Screening for PHA using the Aldosterone-Renin-Ratio (ARR) should be performed in patients with hypokalemic, severe or resistant hypertension. In addition, young patients with early onset of severe hypertension and/or positive family history should be screened. A positive screening result should be followed by a confirmatory test. The saline infusion test is the preferred clinical test for confirming a suspected PHA since it is accessible and time efficient. Other confirmatory tests are not used on a regular basis. After any confirmatory test, CT- or MRI-imaging and adrenal vein sampling (AVS) is used in order to differentiate between a unilateral adenoma, a bilateral hyperplasia or another cause of PHA. CT or MRI usually cannot discriminate smaller tumors form hyperplasia. Therefore AVS is used to detect lateralization of autonomous aldosterone production. Lateralization of aldosterone production indicates a unilateral adenoma. In these cases, laparoscopic adrenalectomy is the therapeutic option of choice with a hypertension cure rate of up to 60 %. If no lateralization is detectable, bilateral hyperplasia as the underlying cause of PHA is likely. Pharmacological inhibition of the mineralocorticoid receptor is the preferred treatment option in these cases. If Spironolactone is not well tolerated, Eplerenone and potassium-sparing diuretics should be prescribed. Often, however, in order to fully control hypertension, additional antihypertensive therapy is necessary."}, {"id": "pubmed23n0349_17935", "title": "[Primary hyperaldosteronism--12 clinical cases].", "score": 0.013450502152080344, "content": "To show clinical, biochemical, and morphological data of 12 patients with primary hyperaldosteronism: eight with an aldosterone-producing adenoma and four with adrenal hyperplasia. To compare clinical and biochemical parameters of the patients with adenoma and hyperplasia. For those with adenoma, to verify clinical and biochemical modifications after adrenalectomy. In the 12 patients with hyperaldosteronism, retrospective analysis of clinical (age, sex, blood pressure), biochemical (plasmatic and urinary potassium, plasmatic aldosterone, plasma renin activity, and plasmatic aldosterone/renin activity ratio), and morphological (computed tomography, magnetic resonance, and norcholesterol scintigraphy) data was performed. 1--In the 12 patients with hyperaldosteronism (seven female), the age was 51.0 +/- 10.2 years (mean +/- standard deviation), the systolic pressure 200.9 +/- 34.5 mm Hg and the diastolic pressure 120.0 +/- 12.3 mm Hg. Hypertension was diagnosed 12.0 +/- 10.1 years before. As biochemical evidence, we found kalaemia of 3.06 +/- 0.28 and urinary potassium of 63.4 +/- 16.5 mEq/l, renin activity 0.98 +/- 1.02 ng/ml/h, plasmatic aldosterone of 49.4 +/- 36.0 ng/dl, aldosterone/renin activity &gt; 30 in 83% of the cases. As morphological evidence, computed tomography allowed diagnosis in nine patients, suggested it in two, being doubtful in one. Performed on four patients, resonance confirmed the tomography in three and was not contributive in one. The scintigraphy performed in four patients visualized two adenomas, was negative in one adenoma and in one hyperplasia. 2--In the eight patients with adenoma (six female), the youngest age and the highest diastolic pressure compared with patients with hyperplasia were statistically significant (p &lt; 0.01 and 0.05). In the adenomas, the biochemical changes were more pronounced, but not statistically significant. The plasmatic aldosterone/renin activity ratio was also higher in the adenoma cases. 3--After the adrenalectomy, blood pressure became normal in five patients and was more easily therapeutically controlled in three. The average systolic and diastolic pressures decreased and the biochemical parameters became normal in all patients. The pre/post surgical modification of these parameters had statistical significance (systolic pressure decrease, p &lt; 0.01; diastolic pressure decrease, p &lt; 0.01; kalaemia increase, p &lt; 0.001; renin activity increase, p &lt; 0.01; aldosterone decrease, p &lt; 0.02). The plasmatic aldosterone/renine activity ratio normalized in all patients. In diagnosing primary hyperaldosteronism, biochemical (kalaemia, urinary potassium, plasmatic aldosterone, renin activity, aldosterone plasmatic/renin activity) and tomography studies were important. On comparing the patients with hyperplasia with those with adenoma, we found that the latter are younger and exhibit higher diastolic pressure, both findings with statistical significance. After adenoma surgery, blood pressure became normal in five patients and improved in three, these findings, and the improvement of the kalaemia, plasmatic aldosterone, and renin activity parameters were statistically significant."}, {"id": "pubmed23n0234_7242", "title": "A case of normoreninemic, normotensive primary aldosteronism associated with essential hypertension and nephrocalcinosis.", "score": 0.013155624568668046, "content": "A 36-year-old female case of normotensive normoreninemic primary aldosteronism with persistent hypokalemia and nephrocalcinosis is reported. She was referred to us for episodes of sudden muscle weakness during 8 years prior to admission. On the first day of admission, her blood pressure was 174/104 mmHg. On the second day of admission blood pressure normalized to 120/80 mmHg. Both of her parents were hypertensive. Arterial blood gas analysis showed metabolic alkalosis. Except an impaired urine concentration ability, renal functions were normal. Intravenous pyelogram showed numerous granular calcifications. Basal plasma renin activity was 1.0 approximately 1.5 ng/ml/hr and increased by sodium depletion. Plasma aldosterone concentration was 70 approximately 80 ng/dl and did not respond to various stimulations. Blood pressure was dependent on sodium balance. It fell on salt restriction and rose on salt loading. Blood pressure responses to vasoactive hormones were normal. Circulating plasma volume was within normal range. After removal of an adrenal adenoma, there was mild fall of blood pressure, serum potassium returned to normal level and plasma renin activity increased slightly. Histologically, there was renal tubular calcifications, and juxtaglomerular apparatus was normal. Blood pressure was elevated to 160/100 mmHg when patient was followed at out-patient clinic after discharge. We concluded that she had essential hypertension associated with primary aldosteronism. Although sodium loss and an increase in urinary kallikrein were found, they did not seem to be the cause of normoreninemic normotensive state of this patient, and the pathogenesis remains to be elucidated."}, {"id": "wiki20220301en073_38761", "title": "Hyperaldosteronism", "score": 0.012248909343688576, "content": "Primary Primary aldosteronism (hyporeninemic hyperaldosteronism) was previously thought to be most commonly caused by an adrenal adenoma, termed Conn's syndrome. However, recent studies have shown that bilateral idiopathic adrenal hyperplasia is the cause in up to 70% of cases. Differentiating between the two is important, as this determines treatment. Also, see congenital adrenal hyperplasia. Adrenal carcinoma is an extremely rare cause of primary hyperaldosteronism. Two familial forms have been identified: type I (dexamethasone suppressible), and type II, which has been linked to the 7p22 gene. Features Hypertension Hypokalemia (e.g., may cause muscle weakness) Alkalosis Investigations High serum aldosterone Low serum renin High-resolution CT abdomen Management Adrenal adenoma: surgery Bilateral adrenocortical hyperplasia: aldosterone antagonist, e.g., spironolactone"}, {"id": "InternalMed_Harrison_27001", "title": "InternalMed_Harrison", "score": 0.012175324675324676, "content": "In patients with normal adrenal morphology and family history of early-onset, severe hypertension, a diagnosis of GRA should be Disorders of the Adrenal Cortex Clinical suspicion of mineralocorticoid excess Patients with hypertension and Severe hypertension (>3 BP drugs, drug-resistant) or Family history of early-onset hypertension or cerebrovascular events at < 40 years of age Measurement of aldosterone-renin ratio (ARR) on current blood pressure medication (stop spironolactone for 4 wks) and with hypokalemia corrected (ARR screen positive if ARR >750 pmol/L: ng/ml/h and aldosterone >450 pmol/L) (consider repeat off ˜-blockers for 2 wks if results are equivocal) Negative E.g., saline infusion test (2 liters physiologic saline over 4 h IV), oral sodium loading, fludrocortisone suppression Confirmation of diagnosis Rare: Both renin and Aldo suppressed"}, {"id": "wiki20220301en019_21526", "title": "Primary aldosteronism", "score": 0.011162498572570515, "content": "Primary hyperaldosteronism has a number of causes. About 33% of cases are due to an adrenal adenoma that produces aldosterone, and 66% of cases are due to an enlargement of both adrenal glands. Other uncommon causes include adrenal cancer and an inherited disorder called familial hyperaldosteronism. Some recommend screening people with high blood pressure who are at increased risk, while others recommend screening all people with high blood pressure for the disease. Screening is usually done by measuring the aldosterone-to-renin ratio in the blood (ARR) whilst off interfering medications and a serum potassium over 4, with further testing used to confirm positive results. While low blood potassium is classically described in primary hyperaldosteronism, this is only present in about a quarter of people. To determine the underlying cause, medical imaging is carried out."}, {"id": "wiki20220301en073_38758", "title": "Hyperaldosteronism", "score": 0.010888634630053394, "content": "Hyperaldosteronism is a medical condition wherein too much aldosterone is produced by the adrenal glands, which can lead to lowered levels of potassium in the blood (hypokalemia) and increased hydrogen ion excretion (alkalosis). This cause of mineralocorticoid excess is primary hyperaldosteronism reflecting excess production of aldosterone by adrenal zona glomerulosa. Bilateral micronodular hyperplasia is more common than unilateral adrenal adenoma. Signs and symptoms It can be asymptomatic, but these symptoms may be present: Fatigue Headache High blood pressure Hypokalemia Hypernatraemia Hypomagnesemia Intermittent or temporary paralysis Muscle spasms Muscle weakness Numbness Polyuria Polydipsia Tingling Metabolic alkalosis Nocturia Blurry Vision Dizziness/Vertigo"}, {"id": "wiki20220301en024_713", "title": "Antihypertensive drug", "score": 0.010865686554381958, "content": "Sodium nitroprusside, a very potent, short-acting vasodilator, is most commonly used for the quick, temporary reduction of blood pressure in emergencies (such as malignant hypertension or aortic dissection). Hydralazine and its derivatives are also used in the treatment of severe hypertension, although they should be avoided in emergencies. They are no longer indicated as first-line therapy for high blood pressure due to side effects and safety concerns, but hydralazine remains a drug of choice in gestational hypertension. Renin inhibitors Renin comes one level higher than angiotensin converting enzyme (ACE) in the renin–angiotensin system. Renin inhibitors can therefore effectively reduce hypertension. Aliskiren (developed by Novartis) is a renin inhibitor which has been approved by the U.S. FDA for the treatment of hypertension. Aldosterone receptor antagonist Aldosterone receptor antagonists: eplerenone spironolactone"}, {"id": "wiki20220301en070_64324", "title": "Secondary hypertension", "score": 0.010347258945884012, "content": "11β-hydroxylase deficiency, aka apparent mineralocorticoid excess syndrome, involves a defect in the gene for 11β-hydroxysteroid dehydrogenase, an enzyme that normally inactivates circulating cortisol to the less-active metabolite cortisone. At high concentrations cortisol can cross-react and activate the mineralocorticoid receptor, leading to aldosterone-like effects in the kidney, causing hypertension. This effect can also be produced by prolonged ingestion of liquorice (which can be of potent strength in liquorice candy), by causing inhibition of the 11β-hydroxysteroid dehydrogenase enzyme and likewise leading to secondary apparent mineralocorticoid excess syndrome. Frequently, if liquorice is the cause of the high blood pressure, a low blood level of potassium will also be present. Cortisol induced hypertension cannot be completely explained by the activity of Cortisol on Aldosterone receptors. Experiments show that treatment with Spironolactone (an inhibitor of the aldosterone"}, {"id": "InternalMed_Harrison_26993", "title": "InternalMed_Harrison", "score": 0.010271410108155908, "content": "Diagnosis Diagnostic screening for mineralocorticoid excess is not currently recommended for all patients with hypertension, but should be restricted to those who exhibit hypertension associated with drug resistance, hypokalemia, an adrenal mass, or onset of disease before the age of 40 years (Fig. 406-12). The accepted screening test is concurrent measurement of plasma renin and aldosterone with subsequent calculation of the aldosterone-renin ratio (ARR) (Fig. 406-12); serum potassium needs to be normalized prior to testing. Stopping antihypertensive medication can be cumbersome, particularly in patients with severe hypertension. Thus, for practical purposes, in the first instance the patient can remain on the usual antihypertensive medications, with the exception that mineralocorticoid receptor antagonists need to be ceased at least 4 weeks prior to ARR measurement. The remaining antihypertensive 2319 drugs usually do not affect the outcome of ARR testing, except that beta blocker"}, {"id": "pubmed23n0115_2497", "title": "Long-term results of surgical and conservative treatment of patients with primary aldosteronism.", "score": 0.010225642363375505, "content": "The long-term results of surgical and specific drug therapy were compared in a group of 57 patients with primary aldosteronism (PA) (46 with aldosterone-producing adenoma (APA), 11 with idiopathic hyperaldosteronism (IHA) and bilateral adrenal hyperplasia). Unilateral adrenalectomy completely normalized blood pressure (BP) in 77.1% of surgically treated APA, evidently improving hypertension in remaining 22.9%. No recurrence of the adenoma in the remaining adrenal was seen in any of the surgical APA cases. In 19 of the non-surgical patients (11 with APA, 8 with IHA) monotherapy with spironolactone reduced blood pressure in 73%, though total BP normalization was an exception. The treatment normalized hypokalemia, low total exchangeable potassium, tendency to hypernatremia, and high total exchangeable sodium. Surgical as well as conservative therapy increased to normal or above-normal levels plasma renin activity suppressed prior to treatment. Pre-operatively high urine and plasma aldosterone levels normalized in all adrenalectomized patients, but remained above the normal range during spironolactone therapy in spite of a small decline in its absolute values. The disturbances of maximum renal concentrating capacity due to impaired nephron responsiveness to sufficiently high endogenous vasopressin concentrations were completely eliminated after kaliopenic nephropathy had been repaired. The other renal functions remained within normal values. Echocardiographically diagnosed left ventricular hypertrophy was seen less often than in the other types of arterial hypertension, tending to regress after APA management. Our longitudinal study (2-16 years) showed primary aldosteronism as a well curable, albeit rare, cause of hypertension. As regards BP and laboratory tests normalization, better results were achieved in surgical APA cases than in patients treated with spironolactone. Older age, longer history of hypertension and more frequent incidence of obesity, nephrosclerosis and pyelonephritis may be responsible for hypertension persisting after surgical treatment."}, {"id": "wiki20220301en086_18929", "title": "Liddle's syndrome", "score": 0.010210438875813374, "content": "Diagnosis Evaluation of a child with persistent high blood pressure usually involves analysis of blood electrolytes and an aldosterone level, as well as other tests. In Liddle's disease, the serum sodium is typically elevated, the serum potassium is reduced, and the serum bicarbonate is elevated. These findings are also found in hyperaldosteronism, another rare cause of hypertension in children. Primary hyperaldosteronism (also known as Conn's syndrome), is due to an aldosterone-secreting adrenal tumor (adenoma) or adrenal hyperplasia. Aldosterone levels are high in hyperaldosteronism, whereas they are low to normal in Liddle syndrome. A genetic study of the ENaC sequences can be requested to detect mutations (deletions, insertions, missense mutations) and get a diagnosis."}, {"id": "wiki20220301en104_37769", "title": "Pseudohyperaldosteronism", "score": 0.009969777384638065, "content": "Treatment Specific treatment of pseudohyperaldosteronism depends on the inciting cause. General management focuses on countering the effects of excess mineralocorticoid activity to achieve adequate blood pressure control and avoid end-organ damage and cardiovascular mortality. In some cases, specific antihypertensive medications may be recommended. In Liddle's syndrome, ENaC-binding potassium-sparing diuretics (e.g. amiloride or triamterene) are used to counter the excess ENaC activity. In AME, the mineralocorticoid receptor-binding potassium-sparing diuretics (e.g. spironolactone or eplerenone) are used to limit aldosterone receptor activity. Other medications such as glucocorticoids are added in AME and CAH to inhibit ACTH and further cortisol production. Lifestyle changes such as a low sodium diet are also used for managing hypertension, and cessation of licorice intake is recommended in cases of licorice overconsumption."}, {"id": "wiki20220301en016_32124", "title": "Aldosterone", "score": 0.009905288082083661, "content": "Associated clinical conditions Hyperaldosteronism is abnormally increased levels of aldosterone, while hypoaldosteronism is abnormally decreased levels of aldosterone. A measurement of aldosterone in blood may be termed a plasma aldosterone concentration (PAC), which may be compared to plasma renin activity (PRA) as an aldosterone-to-renin ratio. Hyperaldosteronism Primary aldosteronism, also known as primary hyperaldosteronism, is characterized by the overproduction of aldosterone by the adrenal glands, when not a result of excessive renin secretion. It leads to arterial hypertension (high blood pressure) associated with hypokalemia, usually a diagnostic clue. Secondary hyperaldosteronism, on the other hand, is due to overactivity of the renin–angiotensin system. Conn's syndrome is primary hyperaldosteronism caused by an aldosterone-producing adenoma."}, {"id": "pubmed23n0712_10216", "title": "Mineralocorticoid hypertension.", "score": 0.009900990099009901, "content": "Hypertension affects about 10 - 25% of the population and is an important risk factor for cardiovascular and renal disease. The renin-angiotensin system is frequently implicated in the pathophysiology of hypertension, be it primary or secondary. The prevalence of primary aldosteronism increases with the severity of hypertension, from 2% in patients with grade 1 hypertension to 20% among resistant hypertensives. Mineralcorticoid hypertension includes a spectrum of disorders ranging from renin-producing pathologies (renin-secreting tumors, malignant hypertension, coarctation of aorta), aldosterone-producing pathologies (primary aldosteronism - Conns syndrome, familial hyperaldosteronism 1, 2, and 3), non-aldosterone mineralocorticoid producing pathologies (apparent mineralocorticoid excess syndrome, Liddle syndrome, deoxycorticosterone-secreting tumors, ectopic adrenocorticotropic hormones (ACTH) syndrome, congenitalvadrenal hyperplasia), and drugs with mineraocorticoid activity (locorice, carbenoxole therapy) to glucocorticoid receptor resistance syndromes. Clinical presentation includes hypertension with varying severity, hypokalemia, and alkalosis. Ratio of plasma aldosterone concentraion to plasma renin activity remains the best screening tool. Bilateral adrenal venous sampling is the best diagnostic test coupled with a CT scan. Treatment is either surgical (adrenelectomy) for unilateral adrenal disease versus medical therapy for idiopathic, ambiguous, or bilateral disease. Medical therapy focuses on blood pressure control and correction of hypokalemia using a combination of anti-hypertensives (calcium channel blockers, angiotensin converting enzyme inhibitors, or angiotensin receptor blockers) and potassium-raising therapies (mineralcorticoid receptor antagonist or potassium sparing diuretics). Direct aldosterone synthetase antagonists represent a promising future therapy."}, {"id": "pubmed23n0996_15138", "title": "[Primary hyperaldosteronism in a population of hypertensive patients].", "score": 0.00981069174901774, "content": "The diagnosis of primary hyperaldosteronism (PHPA) has progressively increased over the last years and some authors consider it as the main cause of secondary hypertension. We studied the prevalence of PHPA in hypertensive patients followed at the Hypertension Unit from July 1999 to July 2017. A total of 2500 patients were included and diagnosis of PHPA was done in 79 of them (3.2%). It was more frequent in women (55.7%) with an increased incidence in the elderly, as compared to previous studies (27.8%). Initial diagnosis was suspected upon the presence of inappropriate kaliuria and metabolic alkalosis, associated to an aldosterone/plasma renin activity ratio &gt; 30 (ng/dl)/(ng/ml/h). After confirmation of the presence of PA, imaging techniques to determine the etiology were performed. In this way, 29 cases (36.8%) of aldosterone-producing adenoma and 5 cases of bilateral adrenal hyperplasia with nodules were identified. Computed tomography identified the adenomas and hyperplasias with bilateral cortical nodules in all patients. Adrenalectomy and/or antialdosteronics were efficient in controlling blood pressure in 69.9% of cases. Of note in this series was the remission of stage 3 chronic renal failure in two cases, the high prevalence of hypercalciuric urinary lithiasis and a case of breast carcinoma after prolonged treatment with spironolactone."}, {"id": "wiki20220301en026_2976", "title": "Congenital adrenal hyperplasia due to 17α-hydroxylase deficiency", "score": 0.009708737864077669, "content": "The adrenal cortex is hyperplastic and overstimulated, with no impairment of the mineralocorticoid pathway. Consequently, levels of DOC, corticosterone, and 18-hydroxycorticosterone are elevated. Although these precursors of aldosterone are weaker mineralocorticoids, the extreme elevations usually provide enough volume expansion, blood pressure elevation, and potassium depletion to suppress renin and aldosterone production. Some persons with 17α-hydroxylase deficiency develop hypertension in infancy, and nearly 90% do so by late childhood. The low-renin hypertension is often accompanied by hypokalemia due to urinary potassium wasting and metabolic alkalosis. These features of mineralocorticoid excess are the major clinical clue distinguishing the more complete 17α-hydroxylase deficiency from the 17,20-lyase deficiency, which only affects the sex steroids. Treatment with glucocorticoid suppresses ACTH, returns mineralocorticoid production toward normal, and lowers blood pressure."}, {"id": "pubmed23n0021_11375", "title": "[Primary hyperaldosteronism. Diagnostic procedure useful in hospital routine].", "score": 0.009708737864077669, "content": "Personal experience in the management of three cases of primary hyperaldosteronism, in which a cure was obtained by surgical removal of an adrenocortical adenoma, is was used in the elaboration of a diagnostic procedure requiring hospitalisation for 12 days. During 6 days, the patient is kept on a diet containing 100 mEq Na and K, and blood potassium values are repeatedly determined. Other causes of hypertension are ruled out. On the 6th day, baselines for blood renin and urinary aldosterone are calculated. Next, a hyposodic diet is given for 4 days, and a diuretic is administered on the last of these days, after which renin is determined \"in response to stimulation\". Lastly, two days of i.v. NaCl loading are followed by the determination of urinary aldosterone \"during inhibition\". If the picture is positive for hyperaldosteronism, the patient is discharged and followed during treatment with spironolactone, and eventually subjected to renal and adrenal arteriography to determine the site of the adenoma. Division of the procedure into increasingly complex steps enables the examination to be halted at any point when evidence in support of the suspected diagnosis fails to appear. This feature, coupled with the simplicity of the procedures adopted, enables all young subjects admitted for unexplained hypertension to be screened for hyperaldosteronism, with the assurance of obtaining certain diagnosis without an excessively long stay in hospital."}, {"id": "pubmed23n0029_9214", "title": "[Physiopathological and functional semeiologic considerations in a case of primary normoaldosteronemic hyperaldosteronism].", "score": 0.009615384615384616, "content": "Among the atypical pictures of primary aldosteronism, sometimes, normal blood and urine concentration of aldosterone have been observed in association with an adrenal aldosterone-producing adenoma. Here we report a case of atypical primary aldosteronism so characterized: -- the patient had the typical clinical findings of aldosteronism (hypertension, hypokalemic alkalosis, polyuria, etc). -- the patient exhibted all the biochemical abnormalities of primary aldosteronism: increase of exchangeable Na and of plasma volume, decrease of exchangeable K, etc. -- the patient had normal blood and urine levels of aldosterone. -- the patient's blood and urine aldosterone concentration increased following sodium depletion and K administration. Such increase was comparable with that obtained in normal subjects after the same tests. However, at the end of these tests, the patient was still in potassium depletion and sodium repletion. Therefore, it was concluded that the secretion of aldosterone, although normal in absolute values, was inappropriate to the metabolic status of the patient, since such \"normal\" values were found in association with conditions that should have produced an inhibition of aldosterone production. The catheterization of adrenal veins demonstrated the existence of a right adrenal adenoma. The blood pressure and the biochemical parameters of the patients have been normalized by right adrenalectomy."}, {"id": "pubmed23n0989_678", "title": "Hypertension due to a deoxycorticosterone-secreting adrenal tumour diagnosed during pregnancy.", "score": 0.009523809523809525, "content": "Mineralocorticoid hypertension is most often caused by autonomous overproduction of aldosterone, but excess of other mineralocorticoid precursors can lead to a similar presentation. 11-Deoxycorticosterone (DOC) excess, which can occur in 11-β hydroxylase or 17-α hydroxylase deficiencies, in DOC-producing adrenocortical tumours or in patients taking 11-β hydroxylase inhibitors, may cause mineralocorticoid hypertension. We report a 35-year-old woman who in the third trimester of pregnancy was found to have a large adrenal mass on routine obstetric ultrasound. On referral to our unit, persistent hypertension and long-standing hypokalaemia was noted, despite good compliance with multiple antihypertensives. Ten years earlier, she had hypertension noted in pregnancy which had persisted after delivery. A MRI scan confirmed the presence of a 12 cm adrenal mass and biochemistry revealed high levels of DOC and low/normal renin, aldosterone and dehydroepiandrosterone, with normal catecholamine levels. The patient was treated with antihypertensives until obstetric delivery, following which she underwent an adrenalectomy. Histology confirmed a large adrenal cortical neoplasm of uncertain malignant potential. Postoperatively, blood pressure and serum potassium normalised, and the antihypertensive medication was stopped. Over 10 years of follow-up, she remains asymptomatic with normal DOC measurements. This case should alert clinicians to the possibility of a diagnosis of a DOC-producing adrenal tumours in patients with adrenal nodules and apparent mineralocorticoid hypertension in the presence of low or normal levels of aldosterone. The associated diagnostic and management challenges are discussed. Learning points: Hypermineralocorticoidism is characterised by hypertension, volume expansion and hypokalaemic alkalosis and is most commonly due to overproduction of aldosterone. However, excess of other mineralocorticoid products, such as DOC, lead to the same syndrome but with normal or low aldosterone levels. The differential diagnosis of resistant hypertension with low renin and low/normal aldosterone includes congenital adrenal hyperplasia, syndrome of apparent mineralocorticoid excess, Cushing's syndrome, Liddle's syndrome and 11-deoxycorticosterone-producing tumours. DOC is one intermediate product in the mineralocorticoid synthesis with weaker activity than aldosterone. However, marked DOC excess seen in 11-β hydroxylase or 17-α hydroxylase deficiencies in DOC-producing adrenocortical tumours or in patients taking 11-β hydroxylase inhibitors, may cause mineralocorticoid hypertension. Excessive production of DOC in adrenocortical tumours has been attributed to reduced activity of the enzymes 11-β hydroxylase and 17-α hydroxylase and increased activity of 21-α hydroxylase. The diagnosis of DOC-producing adrenal tumours is challenging because of its rarity and poor availability of DOC laboratory assays."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[112, 175]], "word_ranges": [[17, 28]], "text": "No medication slows down or modifies the course of the disease."}, "3": {"exist": true, "char_ranges": [[234, 321]], "word_ranges": [[38, 52]], "text": "Treatment is medical and sequential, starting with the first analgesic step of the WHO."}, "4": {"exist": true, "char_ranges": [[176, 233]], "word_ranges": [[28, 38]], "text": "An MRI is not necessary, it does not contribute anything."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "It is a coxarthrosis. The surgical criteria depends on the patient's symptoms and failure of medical treatment. No medication slows down or modifies the course of the disease. An MRI is not necessary, it does not contribute anything. Treatment is medical and sequential, starting with the first analgesic step of the WHO.", "full_answer_no_ref": "It is a coxarthrosis. The surgical criteria depends on the patient's symptoms and failure of medical treatment. No medication slows down or modifies the course of the disease. An MRI is not necessary, it does not contribute anything. Treatment is medical and sequential, starting with the first analgesic step of the WHO.", "full_question": "You evaluate a 66-year-old patient with groin pain accentuated by prolonged standing for a few days per month. A plain radiograph of the hips shows narrowing of the femoroacetabular joint space, sclerosis and ostephytes.", "id": 318, "lang": "en", "options": {"1": "I make the diagnosis of coxarthrosis and send to the traumatologist to place a hip prosthesis.", "2": "Start treatment with weak opioids that have shown evidence in stopping the progression of the disease.", "3": "Treatment with paracetamol, explaining that the evolution is very variable and the surgical indication depends on the functionality and pain control.", "4": "Because of the radiological features described, I need a hip MRI before making a therapeutic decision.", "5": NaN}, "question_id_specific": 140, "type": "RHEUMATOLOGY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0298_22238", "title": "[Coxarthroses].", "score": 0.019334952363160844, "content": "Osteoarthritis of the hip is a frequent disease affecting 2 to 4% adults. The main symptoms are inguinal pain, hip movement limitation and thigh muscular atrophy. The diagnosis is usually made of on antero-posterior radiograph of the pelvis and Lequesne's lateral view that show joint space narrowing and osteophytosis. There is no correlation between radiographic lesions and clinical symptoms. The mean rate of joint space narrowing progression was shown to be of about 0.30 mm/year but inter-patients variations are very large. The evolution is more sever in older patients and when there is no osteophyte. New imaging techniques are necessary only if diagnosis is impossible on standard radiographs but not for the surgical decision. The treatment of hip osteoarthritis requires analgesic, NSAIDs and symptomatic slow actin drugs for osteoarthritis. Total hip arthroplasty is necessary when medical treatment is non effective on pain and disability."}, {"id": "pubmed23n1162_86", "title": "Editorial Commentary: Routine Preoperative Magnetic Resonance Imaging for Hip Arthroscopy Is Wasting Health Care Dollars and Delaying Surgical Intervention: Decision Making Should Be at the Discretion of the Health Care Provider Not Mandated by Health Care Insurers.", "score": 0.019246784491533265, "content": "Making an accurate preoperative diagnosis is critical to optimizing outcomes after hip arthroscopy. A detailed history, thorough physical examination, imaging studies, and diagnostic injections must all be considered in the decision-making process. In today's health care climate, it is imperative to obtain essential and indicated preoperative information while being mindful of health care dollars. Magnetic resonance imaging (MRI) of the hip has been shown to be a highly sensitive modality for hip and pelvis disorders. However, it is critical to recognize that acetabular labral tears and other hip pathology are highly prevalent in an asymptomatic young adult population. There are certainly situations when an MRI should be obtained (suspected arthritic symptoms, avascular necrosis, synovial disorders, uncommon osseous tumors); however, these patients generally present with atypical symptoms. In addition, obtaining an MRI can delay surgical intervention, which has been shown to lead to inferior outcomes in prior studies. MRI is not imperative when patients present with typical intermittent, deep anterior, lateral, groin pain with prolonged sitting, twisting and pivoting, and transitioning from sitting to standing. The typical physical examination includes positive hip impingement testing (FADIR / anterior impingement test) that recreates the patients presenting complaints. Appropriate imaging includes plain radiographs revealing adequate acetabular coverage (not significantly dysplastic) or acetabular overcoverage (pincer-type femoracetabular impingement), cam-type femoracetabular impingement, and well-maintained joint space on all views, including a false profile radiograph to further evaluate the anterior joint space. Finally, a diagnostic injection can be invaluable to further confirm the hip joint proper as the source of pain. If all of the above criteria are met, I strongly believe an MRI is unlikely to alter the surgical decision-making process. In the end, the treating clinician should determine when an MRI is necessary based on the presenting symptoms and examination, rather than insurers applying a blanket requirement for preauthorization. This physician autonomy would ultimately lead to more efficient and cost-effective patient care. Medicine is an art, and unjustified handcuffing of the artist without evidence could result in inferior results."}, {"id": "pubmed23n0779_20122", "title": "Magnetic resonance imaging of the hip: poor cost utility for treatment of adult patients with hip pain.", "score": 0.016133229247983348, "content": "Although MRI is frequently used to diagnose conditions affecting the hip, its cost-effectiveness has not been defined. We performed this retrospective study to determine for patients 40 to 80 years old: (1) the differences in hip MRI indications between orthopaedic and nonorthopaedic practitioners; (2) the clinical indications that most commonly influence treatment decisions; (3) the likelihood that hip MRI influences treatment decisions separate from plain radiographs; and (4) the cost of obtaining hip MRI studies that influence treatment decisions (impact studies). We retrospectively assessed 218 consecutive hip MRI studies (213 patients) at one institution over a 5-year interval. Medical records, plain radiographs, and MRI studies were reviewed to determine how frequently individual MRI findings determined treatment recommendations (impact study). The cost estimate of an impact study was calculated from the product of institutional MRI unit cost (USD 436) and the proportion of impact studies relative to all studies obtained either for a specific indication or by an orthopaedic/nonorthopaedic clinician. Nonorthopaedic clinicians more frequently ordered hip MRI without a clinical diagnosis (72% versus 30%, p &lt; 0.01), before plain radiographs (29% versus 3%, p &lt; 0.001), and with less frequent impact on treatment (6% versus 15%, p &lt; 0.05). Hip MRI most frequently influenced treatment when assessing for a tumor (58%, p &lt; 0.001) or infection (40%, p &lt; 0.001) and least frequently when assessing for pain (1%, p &lt; 0.002). Hip MRI impacted a treatment decision independent of plain radiographic findings in only 7% of studies (3% surgical, 4% nonsurgical). Hip MRI cost was least when assessing for a neoplasm (USD 750) and greatest when assessing undefined hip pain (USD 59,000). The cost of obtaining an impact study was also less when the ordering clinician was an orthopaedic clinician (USD 2800) than a nonorthopaedic clinician (USD 7800). Although MRI can be valuable for diagnosing or staging specific conditions, it is not cost-effective as a screening tool for hip pain that is not supported by history, clinical examination, and plain radiographic findings in patients between 40 and 80 years of age. Level IV, economic and decision analysis study. See Instructions for Authors for a complete description of levels of evidence."}, {"id": "pubmed23n0509_4494", "title": "Fate of very small asymptomatic stage-I osteonecrotic lesions of the hip.", "score": 0.013712686567164178, "content": "The prognosis for a patient with osteonecrosis of the hip is generally considered to be worse if a large volume of the femoral head is involved, the patient is symptomatic, and the stage of the lesion is advanced. In 1990, we began a prospective study to detect collapse in asymptomatic hips with a very small stage-I osteonecrotic lesion in the femoral head. We hypothesized that such patients would have a favorable prognosis. These hips were followed for a minimum of ten years after the diagnosis. A small asymptomatic stage-I osteonecrotic lesion (not seen on plain radiographs) was diagnosed with magnetic resonance imaging in forty patients (forty hips) contralateral to a hip with symptomatic osteonecrosis. The criterion for inclusion in the study was a lesion with a volume of &lt;5 cm(3) involving &lt;10% of the volume of the femoral head. Plain radiographs were made annually in six different projections for all patients. At the most recent follow-up evaluation (average, eleven years), patients with a symptomatic hip but without evidence of collapse on plain radiographs underwent a computerized tomography scan. Thirty-five (88%) of the forty hips became symptomatic, and twenty-nine (73%) demonstrated collapse. The mean interval between the diagnosis and the first symptoms was eighty months. Symptoms always preceded collapse by at least six months. The mean interval between the diagnosis and the collapse was ninety-two months (range, seventy-two to 140 months). The diagnosis of collapse could be made on only one or two of the six radiographic views obtained for each patient at each evaluation. The diagnosis of collapse for two patients was made only on a computerized tomography scan at the most recent follow-up evaluation. At the time of final follow-up, the twenty-nine hips with collapse had symptoms of intractable pain and required surgery. This study confirms that the diagnosis of collapse is difficult in hips with a very small stage-I osteonecrotic lesion. Multiple radiographic views and computerized tomography scans may be required to demonstrate small areas of collapse. Clinical and radiographic signs of progression of the disease in asymptomatic hips with a very small asymptomatic lesion progress more slowly than do those signs in hips with a large symptomatic stage-II lesion. Because hips with a small area of osteonecrosis do collapse in a large percentage of patients, such patients should be followed carefully over a long period of time. Prognostic study, Level I-1 (prospective study). See Instructions to Authors for a complete description of levels of evidence."}, {"id": "pubmed23n0757_6412", "title": "Differential diagnosis and early management of rapidly progressing hip pain in a 59-year-old male.", "score": 0.013251565314349428, "content": "Rapidly progressing degeneration of the hip joint is an uncommon condition presenting to physical therapy. Differential diagnosis can often be difficult, as clinical and radiographic findings do not always coincide leaving clinicians with difficult decision making regarding course of treatment. The purpose of this case report was to describe the differential diagnosis and early management of a patient with rapidly progressing hip pain. A 59-year-old male with a complicated medical history was referred with a diagnosis of severe bilateral hip osteoarthritis. Clinical presentation of insidious onset, severe bilateral groin and anterior thigh pain with rapid progression of functional decline lead to the differential diagnosis of bilateral avascular necrosis. The patient received seven manual physical therapy sessions over the course of one month. During this time, the patient's Lower Extremity Functional Scale score worsened from 33 to 21. The persistence of the patient's painful symptoms and continued functional decline helped determine cessation of manual therapy and referral back to his GP for further diagnostic testing and eventual correct diagnosis. This case highlights the importance of monitoring patient prognosis using outcome measures leading to a change in patient management strategies."}, {"id": "pubmed23n0362_15261", "title": "[Imaging of chronic hip pain in adults].", "score": 0.012608712164368995, "content": "Adult hip pathologies are mainly represented by the degenerative disease, so called \"osteoarthrosis, or more precisely coxarthrosis\". The means of imaging are exposed, according to their specific value: X Rays (measurement of the characteristic angles of the adult hip), Arthrography, CT Scanner, Arthro-CT Scanner, MRI, Bone Scintigraphy, Ultrasonography. Clinical findings differentiate a mechanical syndrome and an inflammatory syndrome. The coxarthrosis is the most frequent, under two forms: primary (idiopathic) coxarthrosis and secondary coxarthrosis. Primary (idiopathic) coxarthrosis has a localised narrowing of the joint space, osteophyte formation, subchondral sclerosis, cyst formation. The destruction progresses slowly, in 10 to 15 years leading to a complete destruction. Bilaterality is frequent. it is treated with total hip prosthesis. There is a rapid form (1 to 2 years) (Postel's Disease). Secondary coxarthrosis occurs after architectural vice, chondral diseases, lack of balance between the size of the head and the acetabulum as in the case of previous fracture or dislocation, avascular bone necrosis of the head of the femur, Paget's disease. Calcium pyrophosphate Deposition disease (CPPD) involves mostly aged women, and also leads to cox-arthrosis. Avascular bone necrosis of the head of the femur involves young adults. Bilateral involvement are frequent. MRI is the most sensitive and the most specific means of early diagnosis, The area of bone necrosis appears as well defined modifications of the upper head of the femur, precisely surrounded by a low signal intensity line on both Ti and T2 weighted imaging. MRI shows articular effusion, bone marrow edema. Scintigraphy gives early findings which are a characteristic, but non specific, hot spot. CT scanner is used for hip destruction evaluation. o Algodystrophy: transient osteoporosis of the hip has a cyclic course, lasting 3 to 9 months. MRI shows an inflammatory pattern in the area of the process(dark in Ti and white in T2, with positive Gadolinium response). Scintigraphy is positive. Staphylococcus location in the hip can be acute or chronic. MRI shows joint effusion, cystic formation and subchondral non specific modifications. Tuberculosis of the hip joint is relatively rare. Greater trochanteric tuberculous involvement is possible under special contexts. Chronic Inflammatory diseases are represented by Rheumatoid Arthritis, Spondylarthritis and other chronic inflammatory diseases. Synovial tumors such as Pigmented Villo Nodular Synovitis, Primary Osteochondromatosis, synovial sarcoma have special presentations. The subchondral bone can be involved by amorphous depositions such as in tophaceous gout, different varieties of lipidosis, amyloidosis, reticulo histiocytosis. Pen arthropathies are enthesopathies in the anterior rectus tendon, calcifying tendonitis (not to be confused with calcifying soft tissue tumor/chondrosarcoma). The pelvis bone and the femur are involved by primary and secondary tumors or by insufficiency fractures which can mislead to hip pathologies."}, {"id": "pubmed23n0956_8910", "title": "Idiopathic chondrolysis of hip in children: New proposal and implication for radiological staging.", "score": 0.00980392156862745, "content": "Our objective was to evaluate the radiological appearances in different stages of idiopathic chondrolysis of hip (ICH) which will be helpful in the early diagnosis and guiding appropriate treatment for this condition to prevent progression of disease. We evaluated 14 patients of ICH in varying stages: Stage 1 (<in</i = 9), Stage 2 (<in</i = 3), Stage 3 (<in</i = 2). Average age at presentation was 10-11 years. Plain radiograph and magnetic resonance imaging (MRI) was done in all these patients. In the current study, we have attempted to stage ICH based on the radiological progression of the disease, where MRI was used as the primary tool. Stage 1 showed a wedge-shaped hyperintensity in T2 weighted (T2W) and hypointensity in T1 weighted (T1W) images involving the middle one-third of the femoral head and it is the earliest and characteristic finding in MRI. Associated findings like joint space narrowing, synovial hypertrophy with joint effusion may also be observed. Stage 2 showed acetabular edema in the affected hip in addition to the above-mentioned findings. Stage 3 showed more extensive involvement of femoral head and acetabulum, with collapse of the femoral head, degenerative changes in hip, early osteoporotic changes, and ultimately loss of joint space. Imaging-based staging system proves very useful in the early diagnosis, staging, and assessing the prognosis of ICH."}, {"id": "pubmed23n0133_16112", "title": "[The natural course of osteoarthritis of the hip. Indication of conservative treatment in relation to osteophyte formation at the acetabular rim].", "score": 0.00980392156862745, "content": "One hundred and eighty-five hips from 152 patients with primary or secondary osteoarthritis were studied in an attempt to assess the degrees of hip pain in contrast to radiological and other clinical findings. In 30.8% and 26.4% of the primary and secondary osteoarthritic hips respectively, hip pain showed some gradual decrease as time elapsed. Pain relief probably in association with osteophyte formation at the craniolateral acetabular rim occurred in 62.5% and 33.3% of the primary and secondary osteoarthritic hips respectively. Significant parameters observed in the primary osteoarthritic cases of the decreasing pain group were as follows: A lesser extent of cranial displacement of the femoral head, poor capital drop development, well developed floor osteophyte. On the other hand significant parameters in the decreasing pain group of secondary osteoarthritis were as follows: Well developed floor osteophyte, a small size of cyst in both the femoral head and the acetabulum, few \"b\" or \"d\" type of bony sclerosis in the acetabulum. Careful observation of radiographic changes (cyst and sourcil) would be most important, especially in secondary osteoarthritis, to decide the indication of surgery. On the basis of histological studies of osteophyte at the craniolateral acetabular rim obtained at the operation, it was assumed that the osteophyte formation had initiated from metaplasia of the labrum or synovial membrane and progressed by a form of chondral ossification after the process of fibrous tissue formation. A well developed trabecular density in the osteophyte at the craniolateral acetabular rim was determined by the use of Muto digigrammer system, Model G-002."}, {"id": "pubmed23n0907_19827", "title": "Reliability of radiographic evaluations of the stage of osteoarthritis of the hip joint and an approach to improve the staging criteria.", "score": 0.009708737864077669, "content": "A few reports have shown that the reliability of the Japanese Orthopaedic Association stage classification of hip osteoarthritis was not high. The objective of this study was to assess the reliability of the stage classification of coxarthrosis, and to evaluate whether a modification of the classification improves reliability. We retrospectively investigated 200 hips in 100 patients with hip pain. We collected radiographs of their hip joints with the patients in both a supine and a standing position. Four orthopaedic surgeons evaluated the stage of coxarthrosis employing the JOA classification. The percentage of agreement of examiners and the value of the kappa statistic were calculated. Furthermore, to improve the reliability of classification, we modified the classification based on previous reports. Partial narrowing of the joint space and disappearance of the joint space were defined as maintained if it was 2 mm or more, and as the width of the loss of joint space that was 10 mm or more respectively. Using this classification, the same examiners assessed the stage on the same radiographs again three months after the previous evaluation. The percentages of agreement were 28.5% and 27% and the interobserver value of the kappa statistic was 0.45 and 0.44 in supine and standing position respectively. After modification of the classification, the percentages of agreement were 36.5% and 44% and the interobserver value of the kappa statistic was 0.48 and 0.56 in supine and standing positions respectively, and the intraobserver value of the kappa statistic was 0.55. The modification significantly improved the reliability only in the percentage of the agreement for the standing position. This study showed that the reliability of the JOA stage classification of coxarthrosis was not as high as previous reports have showed. Modification of the classification improved interobserver reliability."}, {"id": "pubmed23n0291_22539", "title": "[Outcome of hip shelf arthroplasty in adults after a minimum of 15 years of follow-up. Long term results and analysis of failures of 56 dysplastic hips].", "score": 0.009708737864077669, "content": "The goal of this study was to evaluate late results of hip shelf arthroplasty in adults after a minimum of 15 years follow-up. 65 hip shelf arthroplasty performed for painful hip dysplasia between 1964 and 1977 were studied retrospectively in 1992. These 65 procedures were performed in 57 patients mean aged 32 +/- 14 years [17-56]. Nine patients (9 hips) were excluded (2 deceaded, 5 lost for follow-up, and two reoperated because of severe infection). Consequently, the functional results were evaluated for 56 hips (48 patients). Before surgery, according to Merle d'Aubigné's hip rating system, all the hips were painfull (mean pain score was 2.6 +/- 1.7 [0-5]). On radiography, all the hips had a dysplastic acetabulum and arthritic changes. Arthritic changes were severe in 32 hips (57.1 per cent). The hip shelf arthroplasty was carried out according to Roy-Camille. 10 hips had additional varus femoral osteotomy. The 48 patients (56 hips) included were evaluated by means of Merle d'Aubigné's hip rating system and AP and false lateral weight-bearing Xrays. In 1992, 24 procedures were changed for total hip replacement (THR) (17 before 15 years (early failure) and 7 after 15 years of follow-up (late failure)). These 24 hips were included with their last hip rating observed just before THR. Survival analysis was performed according to Kaplan-Meier using date of revision for THR as end-point. After 16.1 +/- 5.6 years of follow-up the functional score for 56 hips was: excellent in 4 hips, very good in 7 hips, good in 10, satisfactory in 14, poor in 17, and bad in 4. The survival rate established for 65 hips was 60 per cent at 15 years and 40 per cent at 21 years. Only 39 hips shelf arthroplasties were still functional after 15 years (mean follow-up 19.1 +/- 3 years [16-28], but 18 hips (46.1 per cent) were painfree or slightly painful (pain score to 5 or 6). Among these 39 hips, the results were excellent in 4 hips, very good in 7 hips, good in 10, satisfactory in 10, poor in 6, and bad in 2. Arthritic change was the main reason for failures: the Kaplan-Meier survival rate at 21 years was 87 per cent when arthrosis was slight and only 15 per cent and 42 per cent when arthrosis was moderate to severe (p = 0.0001). The adverse effect of arthrosis was promoted by lack of congruency for early failures, and by severity of dysplasia and hip subluxation for late failures. The additional femoral varus osteotomies had no influence on functional or radiographic outcome. Our study indicated that hip shelf arthroplasty performed for painful acetabular dysplasia in adult has a 40 per cent probability survival rate at 21 years. The high rate of revision (42.8 per cent) could be related to the prevalence of severe pre-operative arthrosis. The low rate of lost for follow-up (8.7 per cent) and the long follow-up period (16.1 years) made our conclusion reliable. In spite of a high revision rate we recommend shelf athroplasty to treat acetabular dysplasia in adults. This procedure, very reliable in cases of moderate arthrosis, could be performed in cases of severe arthrosis to delay and make easier THR, but a low survival rate could be expected unless dysplasia, lack of congruency and subluxation were mild."}, {"id": "pubmed23n0394_13263", "title": "Structural effect of avocado/soybean unsaponifiables on joint space loss in osteoarthritis of the hip.", "score": 0.009615384615384616, "content": "To evaluate the structural effect of avocado/soybean unsaponifiables (ASU) in the treatment of patients with symptomatic osteoarthritis (OA) of the hip. Patients with regular painful primary OA of the hip (European League Against Rheumatism 1980 criteria) and a joint space still &gt; or = 1 mm (Kellgren grade 1 to 3, assessed by an independent observer prior to inclusion) entered a prospective, multicenter, randomized, parallel group, double-blind, placebo-controlled trial of 2 years duration. Patients had at least a 6-month history of regular pain and an algofunctional index (AFI) &gt; or = 4. The primary assessment criterion was a decrease of the joint space width (JSW) on plain anteroposterior radiographs of the pelvis performed in standing position, measured at the narrowest points by 2 independent readers, previously tested and selected and blinded to both the treatment and the time sequence. Secondary criteria were standard clinical outcome measurements (AFI, pain on a visual analog scale, consumption of nonsteroidal antiinflammatory drugs and patient's and investigator's global assessments). One hundred sixty-three patients were included: 102 men and 61 women (mean age 63.2 +/- 8.7 years). A total of 108 patients (72 men and 36 women; mean age 64 +/- 7.9 years) were radiologically evaluable at 23.7 +/- 2.6 months (ASU group; n = 55) and 23.7 +/- 3.2 months (placebo group; n = 53). Overall comparison of the evolution of JSW showed no difference between the ASU and placebo groups, from 2.35 +/- 0.93 to 1.87 +/- 1.10 mm and from 2.5 +/- 0.94 to 1.9 +/- 1.33 mm, respectively (intergroup P value at end point = 0.9). When patients were divided into 2 subgroups according to the median value of the baseline JSW (2.45 mm), the joint space loss in the most severely affected subgroup of patients (baseline JSW &lt; or = median) was significantly greater in the placebo group than in the ASU group: from 1.69 +/- 0.58 to 0.84 +/- 0.77 mm (-0.86 +/- 0.62 mm) and from 1.66 +/- 0.42 to 1.22 +/- 0.7 mm (-0.43 +/- 0.51 mm), respectively (P &lt; 0.01). The JSW decrease was identical, with no difference in ASU and placebo groups, in the less severely affected subgroup of patients (baseline JSW &gt; median). Clinical parameters in the 2 groups did not differ significantly throughout the study. This pilot randomized, double-blind, placebo-controlled trial failed to demonstrate a structural effect of ASU in hip OA. However, in a post-hoc analysis, ASU significantly reduced the progression of joint space loss as compared with placebo in the subgroup of patients with advanced joint space narrowing. These results suggest that ASU could have a structural effect but require confirmation in a larger placebo-controlled study in hip OA."}, {"id": "pubmed23n0660_20128", "title": "[Late results of conservative treatment of perthes disease.].", "score": 0.009615384615384616, "content": "The objective of the submitted work was to assess the long-term prognosis of Perthes disease. From the originally invited 142 patients 72 attended the check-up examination, complete X-ray documentation was assembled only in 32 patients with 38 affected hip joints. The time interval which had elapsed since the onset of the disease was on average 17 years. The authors evaluated the clinical and X-ray picture of the hip joints and assessed retrospectively the affection of the head according to Catterall's method, incl. signs of a head associated with risk. Treatment provided during the sixties and beginning of the seventies did not meet the principles of modern \"containment\" therapy. It comprised bed rest, application of a plaster spike and aftertreatment with a Thomas splint. During late check-up examinations Wiberg's angle, the epiphyseal index, the index of overlapping of the head, the distance of the head from the floor of the acetabulum and the height of the peak of the greater trochanter above the centre of the head of the femur were assessed. In addition to assessment of these partial X-ray parameters the authors evaluated the spherical properties of the head by Mose's method and subjectively the X-ray picture, using a three grade scale (satisfactory, feasible, poor). In the majority of assessed parameters the authors found a statistically significant correlation with the retrospective classification of the original X-ray pictures classified according to Catterall. The clinical picture was satisfactory in the majority, the authors did not find painful restriction of movement, while almost half the patients reported occasional subjective complaints as regards the hip joint. On the X-ray changes in the overgrowth of the greater trochanter were more marked than changes of the spherical character of the head and the extent of decentering. The follow-up of the group will continue to obtain a longer time interval from the onset of the disease. Key words: Perthes disease, late results, Catterall's classification."}, {"id": "pubmed23n0401_5530", "title": "[Coxarthrosis].", "score": 0.009523809523809525, "content": "Osteoarthritis of the hip joint is a very common disease. There is a minor prevalence of males. By etiology one can distinguish primary (idiopathic) from secondary osteoarthritis. Secondary are due to well-known etiologies as overweight, repetitive traumata, malposture, muscle- and tendon-imbalance etc. Osteoarthritis includes not only cartilage abnormalities, but also such of the subchondral-region, synovialis, synovial fluid and periarticular muscles. The cartilage shows in osteoarthritis typically edema and swelling, defects with tears, fibrillation, and \"baldness\" and (or) cartilagenous repair-islands and joint space narrowing as well, while subchondrally micro-edema, necrosis, ev. microfractures, \"cysts\", demineralisation followed by sclerosis, osteophyte-formation and deformity is seen. With conventional radiographs and CT joint space narrowing, subchondral cysts, sclerosis and osteophytes and deformities are well delineated, MRI however allows visualization of subtle bone marrow and cartilage abnormalities. Clinically, the diagnosis of pre-osteoarthritis becomes more and more important. This includes e.g. deformities and malpostures, labrum-pathologies and structural imbalances. There are three prognostic different types of hip-osteoarthritis depending on the migration of the head of the hip joint: the most common are the latero-cranial and the medio-caudal ones, while the central one is found very rarely. Basic imaging method are conventional radiographs, and CT, followed by MRI. The diagnosis of an \"activated osteoarthritis\" is made by bone-scintigraphy or MRI with i.v. application of contrast-media. The labrum- and cartilage diagnosis should be done with MRI or MR-arthrography. Functional computer-animated analysis will be of great diagnostic value in the near future. MRI indications are differences between clinical results and imaging, missing clinical improvement of an \"activated\" osteoarthritis under standard therapy, unclear joint-pain and before any arthroscopy."}, {"id": "pubmed23n0585_12593", "title": "Prognosis of hip pain in general practice: a prospective followup study.", "score": 0.009523809523809525, "content": "Hip pain in the elderly is the main feature of osteoarthritis of the hip. In this prospective followup study, we wanted to determine which patients with hip pain show disease progression, and what the incidence of total hip replacement (THR) is in this group of patients after 3 and 6 years of followup. Within general practices in the area of Rotterdam (The Netherlands), persons age &gt; or = 50 years with incident hip pain were included. After 3 and 6 years, progression of hip pain was assessed. A total of 224 patients were included. After 3 years disease progressed in 29 (15%) patients and 23 (12%) received a THR. After 6 years disease progressed in 45 (28%) patients and 36 (22%) received a THR. The prognostic variables for a THR after 3 and 6 years related to history taking were age &gt; or = 60 years, morning stiffness, and pain in the groin/medial thigh; variables related to physical examination were decreased extension/adduction, painful internal rotation, and a body mass index &lt; or = 30 kg/m(2); and the variable related to radiologic findings was a Kellgren/Lawrence grade of 2 or higher. In this study population, approximately 12% of patients presenting with hip pain to their general practitioner will undergo a THR within 3 years, and approximately 22% after 6 years. Using the variables obtained from history taking, physical examination, and radiologic findings enables better identification of persons at high risk for a THR."}, {"id": "pubmed23n0067_12318", "title": "[Imaging of the hip].", "score": 0.009433962264150943, "content": "Imaging is fundamental to the diagnosis and therapeutic follow-up of all hip diseases. Despite the advent of extremely sophisticated exploratory methods, the oldest imaging method, straightforward radiography, makes it possible to recognize, locate ans treat the lesion in most cases. Good interpretation of simple antero-posterior and lateral x-ray films therefore is very important. Among the new imaging methods, computerized tomography has brought the third dimension, while ultrasonography and nuclear magnetic resonance have enabled us to visualize cartilages and soft tissues. The contribution to diagnosis and follow-up of these modern methods is considerable, but since we are too near their beginning and since the number of examinations is still too small in many fields, the information they provide must be used with caution. A good knowledge of their principle, advantages and drawbacks should help us to utilize them at best by prescribing only those which are necessary and sufficient for diagnosis and treatment."}, {"id": "pubmed23n0753_10627", "title": "[The role of core decompression for the treatment of femoral head avascular necrosis in renal transplant recipients].", "score": 0.009345794392523364, "content": "Avascular bone necrosis is a relatively rare but significant complication in renal transplant recipients because it causes progressive pain and invalidity. It can be the consequence of the action of numerous causative factors, but it is mostly connected to corticosteroid treatment.The underlying pathophysiologic mechanism is a diminished blood flow to the bone leading to necrosis and bone destruction. During the past 25-years period, 570 renal transplantations and five combined kidney and pancreas transplantations were performed in our centre. A part of the patients was lost to follow-up due to the separation of Croatia from the former Republic of Yugoslavia. After transplantation, we revealed aseptic necrosis of the femoral head in five female patients. All patients had a history of treatment with pulse doses of corticosteroids. At transplantation the average age of the patients was 52.2 yrs (range 46 to 62 yrs), and dialytic treatment before transplantation lasted in average 9.2 yrs (range 2.5 to 21.2 yrs). The period between renal transplantation and the development of clinical signs of avascular bone necrosis lasted in average 1.2 yrs (range 0.3 to 2.3 yrs). We will demonstrate our 62-year old female patient with terminal renal failure caused by post-streptococcal glomerulonephritis, who was treated with peritoneal dialysis 2.5 years before renal transplantation. Twenty months before renal transplantation the patient received pulse doses of corticosteroids, together with immunoglobulins and plasmapheresis, for the treatment of an acute polyradiculoneuritis Guillaine Barré. After transplantation a standard immunosuppressive protocol was applied which included tacrolimus, mycophenolate mofetil, corticosteroids and induction with basiliximab. Four months after transplantation the patient started to feel pain in the right hip after longer standing, in addition to the earlier long-lasting problems caused by bilateral coxarthrosis. The pelvic radiograph showed subchondral radiolucencies in the lateral part of the head circumference spreading into the proximal part of the neck of the right femur, which indicated the presence of aseptic necrosis, but these changes could have also been caused by coxarthrosis. Unexpectedly, magnetic resonance imaging (MRI) did not reveal changes characteristic for avascular bone necrosis. Due to the progressively worsening of pain and the radiographic finding, the patient was submitted to decompression surgery of the femoral head. The surgical procedure was performed under diascopic guidance (C-arm) which allowed the correct positioning of a Kuerschner wire. A cannulated drill (diameter 4.0 mm) was placed over the wire and we performed two drillings of the spongiosis of the femoral head through to the subchondral area. Postoperatively, the patient was soon verticalized and advised to walk with crooks during a period of six weeks. This time is necessary to allow the mineralisation and strengthening of the bone which is now better vascularised. The patient recovered well and had no more pain. In renal transplant recipients it is most important to raise suspicion and verify the presence of avascular bone necrosis early, because timely bone decompression surgery can eliminate pain and cure the patient or it can prevent or delay bone destruction. When clinical signs of avascular bone necrosis arise and radiographic or standard MRI findings are negative, additional investigations (i.e. SPECT or MRI with contrast) should be performed to confirm or exclude the diagnosis. In latter phases of the disease, surgical decompression of the femoral head cannot lead to permanent amelioration, and it is inevitable to perform more invasive surgical procedures like \"resurfacing\" or bone grafting in younger patients, or the implantation of total hip endoprotheses."}, {"id": "pubmed23n0735_19515", "title": "Stress fracture of the femoral neck in a child:  a case report.", "score": 0.009259259259259259, "content": "A 7-year-old boy developed complaints of pain in the left groin. These complaints started spontaneously. Initial plain radiographs of the pelvis indicated no abnormalities. As the symptoms persisted for 6 weeks, the young patient and his parents visited our institution. Clinical investigation showed a slight extension deficit of the left hip. New radiographs and MRI indicated a fracture line with sclerosis along the inferior border of the left femoral neck. In retrospect, this stress fracture of the femoral neck was also visible on the initial radiographs. Seven months after the onset of complaints in the left groin and prescribed partial weight bearing with crutches, callus formation with consolidation of the femoral neck was observed on radiographs. Eleven months after onset, the patient recovered fully without any residual symptoms. After 21 months, the young patient did not have any complaints or restrictions in physical activity. Because of its highly rare nature, stress fractures of the femoral neck in children are easy to miss initially. This was also applicable in our case. Extensive differential diagnosis of a child with pain in the groin furthermore adds to the difficulty in the diagnosis of a stress fracture of the femoral neck. This case report emphasizes the importance of the evaluation of radiographs and observation in children with hip complaints. Similarly, interdisciplinary consultation and cooperation between the general practitioner, orthopaedic surgeon, radiologist and paediatrician is essential in the diagnosis, evaluation and treatment of these young patients."}, {"id": "pubmed23n0117_7415", "title": "[Study of a painful total hip prosthesis].", "score": 0.009259259259259259, "content": "The authors propose a simple routine survey to be performed in the case of a painful total hip prosthesis which is generally able to define the diagnosis and the prognosis. X-ray films are more valuable by their repetition than by their simple analysis. Certain minor signs can help predict or detect loosening of the prosthesis or other complications, but in the great majority of cases, radiology alone is not sufficient to make the diagnosis, as the films are only able to show clear spaces around the prosthesis, without being able to definitely confirm the aetiopathogenic significance of this sign. In these cases, when the decision has not been taken to re-operate, more sophisticated investigations are required (scintigraphy, dynamic tests, arthrography), although they must be used with caution as very often the clinical findings will have the last word."}, {"id": "pubmed23n1003_17020", "title": "Radiographic Prevalence of Sacroiliac Joint Abnormalities and Clinical Outcomes in Patients With Femoroacetabular Impingement Syndrome.", "score": 0.009174311926605505, "content": "To quantify the prevalence of sacroiliac joint (SIJ) abnormalities in patients undergoing hip arthroscopy for femoroacetabular impingement syndrome (FAIS) by use of various imaging modalities and to compare outcomes based on SIJ abnormalities. Plain radiographs, computed tomography (CT) scans, and magnetic resonance imaging (MRI) scans of patients who underwent primary hip arthroscopy for FAIS from January 2012 to January 2016 were identified. The exclusion criteria included patients undergoing bilateral or revision surgery, those with a history of dysplasia, and those with less than 2 years' follow-up. On radiographs, the SIJs were graded using modified New York criteria for spondyloarthropathy. CT and MRI scans were reviewed for joint surface erosion, subchondral sclerosis, joint space narrowing, pseudo-widening, bone marrow edema, and ankylosis. Patients with SIJ abnormalities were matched to patients without SIJ abnormalities in a 1:2 ratio by age and body mass index. Outcomes included the Hip Outcome Score-Activities of Daily Living (HOS-ADL), Hip Outcome Score-Sports Subscale (HOS-SS), modified Harris Hip Score (mHHS), visual analog scale (VAS) for pain, and VAS for satisfaction. Of 1,009 consecutive patients, 743 (73.6%) were included; 187 (25.2%) showed SIJ changes. Of these 187 patients, 164 (87.7%) had changes on plain radiographs, 88 (47.1%) had changes on CT, and 125 (66.8%) had changes on MRI. SIJ changes on any imaging modality were weakly correlated with pain to palpation of the SIJ (r = 0.11, P = .004) on physical examination. Pain to palpation of the SIJ on physical examination (odds ratio [OR], 1.12; P = .031) and a history of SIJ pain (OR, 1.93; P = .018) increased the odds of having an SIJ abnormality on any imaging modality. After matching, patients without SIJ abnormalities had a significantly greater HOS-ADL (95.4 vs 90.6, P = .001), HOS-SS (91.1 vs 77.5, P &lt; .001), and mHHS (91.3 vs 84.5, P &lt; .001) and a significantly lower VAS pain score (10.9 vs 25.7, P &lt; .001) than patients with abnormalities at a mean follow-up of 34.1 ± 9.7 months (range, 24-54 months). Patients without SIJ abnormalities had greater odds of achieving the minimal clinically important difference for the HOS-ADL (OR, 2.91; P = .001) and for the HOS-SS (OR, 2.83; P &lt; .001) but not for the mHHS (OR, 1.73; P = .081). A high prevalence of SIJ abnormalities (25.2%) is seen on imaging in FAIS patients. These patients may show significantly inferior clinical outcomes and persistent postoperative pain after FAIS treatment. The results of this study may allow treating orthopaedic surgeons to better inform patients with SIJ abnormalities that they may not achieve clinically significant outcome improvement after hip arthroscopy. Level III, retrospective comparative study."}, {"id": "pubmed23n0091_7137", "title": "[Save the joint? Replace the joint? Long-term results and considerations in the treatment of femur head necroses in adults].", "score": 0.009174311926605505, "content": "65 patients with necrosis of the femoral head (not caused by a trauma) in 102 hip joints were examined at an average of 9.7 years following first operation: 1st: 30 joints underwent intertrochanteric varus angulation osteotomy (follow up 11.6 y). 12/30 clinically not sufficient, 13/30 radiologically worse, only 2/30 improved range of movement. 2nd: 35 hip joints were treated using an artificial hip replacement (follow up 9.1 y): 77% got loose at an average of 5 years requiring 39 follow-up operations. 3rd: 5 arthrodesis were performed (follow up 11.4 y). 60% delayed nor non bony union. The results indicate a general osteopathy overlapping the size of osteonecrosis, and causing a lot of complications. Various therapeutic concepts should be considered with respect to radiological stage and age of the patient. The results of joint saving operations depend on the ability of the femoral bony structures to react sufficiently."}, {"id": "pubmed23n0888_25499", "title": "Treatment of mixed type femoroacetabular impingement using safe surgical hip dislocation in adults.", "score": 0.00909090909090909, "content": "This study aims to assess the experience gained in a single institution in the treatment of mixed type femoroacetabular impingement (FAI) using safe surgical hip dislocation (SSHD) technique. In this study, 22 hips of 21 patients (7 males, 14 females; mean age 33.8±10.6 years; range 19-52 years) treated by SSHD technique in our clinic between October 2009 and October 2014 were retrospectively evaluated. Preoperative and final Harris hip scores (HHS) and alpha angles were compared. Age, gender, laterality, impingement tests, preoperative HHS, cam and pincer type FAI radiographic indicators and intraoperative articular findings were assessed in terms of their influence to the final functional outcomes. Mean duration of the symptoms was 29.5 months. Groin pain, activated by flexion and internal rotation of the hip, was the main symptom. A radiographic diagnosis of \"mixed type FAI\" was made in all hips. Mean follow-up duration of 22 hips was 48 months. The difference between the mean preoperative and latest HHS was statistically significant (60.0 vs. 87.6 points, p&lt;0.001). The treatment was considered satisfactory in 17 of 22 hips (77%) having a mean HHS of 95.0 points. Hips having a preoperative HHS of less than 60 points were more prone to unsatisfactory outcome. Among the investigated patient-dependent, clinical, radiographic variables and intraoperative articular findings, coxa profunda sign in a plain radiograph was found correlated with a higher rate of unsatisfactory outcome (p=0.040). Safe surgical hip dislocation procedure has a success rate of 77% after a mean follow-up of four years. Coxa profunda sign is associated with the unsatisfactory clinical outcome. Preoperative HHS of less than 60 points seems to be a negative predictive variable on the clinical outcome."}, {"id": "pubmed23n0839_14423", "title": "[S3 Guideline. Part 1: Diagnosis and Differential Diagnosis of Non-Traumatic Adult Femoral Head Necrosis].", "score": 0.00909090909090909, "content": "Non-traumatic femoral head necrosis (FHN) is primarily a disease of the middle-aged adult. Early diagnosis, at a time with lacking or minimal clinical symptoms, is mandatory to consider conservative therapy or joint preserving operations as a therapeutic option. The new German S3 guideline about diagnosis and therapy of FHN is a cooperative effort of five professional medical societies, overall headed by the Deutsche Gesellschaft für Orthopädie und Orthopädische Chirurgie (DGOOC). This review (part I/III) cites and explains the statements of the S3 guideline as agreed on the use of imaging methods for diagnosis of FHN. A diagnostic algorithm is presented. FHN clinically has to be considered in case of equivocal pain of a hip joint with a minimum of 6 weeks duration, when risk factors can be revealed, groin pain at clinical investigation, limping, pain or limitation of movement in case of load, and no obvious differential diagnoses. Is an FHN clinically suspected, primarily radiographs of the pelvis ap and a Lauenstein projection of the hip involved should be carried out. When the radiographs are normal, an MRI of the hips should follow routinely. MRI allows the diagnosis of FNH with high accuracy. Furthermore, MRI reveals the site and the size of the necrotic area involved and evaluates the integrity of the joint surface and subchondral fractures. When ARCO stage II (ARCO: Association Research Circulation Osseous) is diagnosed and MRI does not allow one to determine the joint surface with certainty, a CT of the hip joints should be performed. The S3 guideline explains and recommends the use of the ARCO classification. Although, this classification of 1993 is still largely based on radiographs, the pragmatic use of an \"extended\" version seems reasonable. Today, classical radiographic criteria like impression of the joint surface and subchondral fractures (\"crescent sign\") are better to be evaluated by MRI, in cases of subtle findings MRI is even surpassed by CT. The extent of the necrosis in the femoral head as well as the size of the surface area involved is best revealed with MRI. Additionally, in the era of cross sectional imaging a stage \"0\" seems obsolete. The guideline also addresses practically important considerations about the differential diagnosis of misleading MRI findings. This especially holds true for bone marrow oedema in the femoral head which may be misinterpreted. The differentiating features between FHN, transient bone marrow oedema and destructive arthropathy are discussed."}, {"id": "pubmed23n1059_20869", "title": "Incidence of Femoroacetabular Impingement and Surgical Management Trends Over Time.", "score": 0.009009009009009009, "content": "Femoroacetabular impingement (FAI) is a well-known cause of hip pain in adolescents and young adults. However, the incidence in the general population has not been clearly defined. To (1) define the population-based incidence of diagnosis of FAI in patients with hip pain, (2) report the trends in diagnosis of FAI over time, and (3) determine the changes in the rate and type of surgical management over time. Cohort study; Level of evidence, 3. A geographic database was used to identify patients who were 14 to 50 years old with hip pain between the years 2000 and 2016. Chart and radiographic review was performed to determine which patients had FAI. To be included, patients had to have a triad of clinical symptoms, physical examination signs, and imaging findings consistent with FAI. Medical records were reviewed to obtain demographic information, clinical history, physical examination findings, imaging details, and treatment details. Statistical analysis determined the overall age- and sex-adjusted annual incidence of FAI diagnosis and trends over time. There were 1893 patients evaluated with hip pain, and 716 (38%; 813 hips) had diagnosed FAI. The mean ± SD age was 27.2 ± 8.4 years, and 67% were female. The incidence of FAI diagnosis was 54.4 per 100,000 person-years. Female patients had a higher incidence than male patients (73.2 vs 36.1 per 100,000 person-years; <iP</i &lt; .01). Incidence of FAI diagnosis were higher from 2010 to 2016 (72.6 per 100,000 person-years; <iP</i &lt; .01) as compared with 2005 to 2009 (45.3) and 2000 to 2004 (40.3). Hip arthroscopy, surgical hip dislocation, and periacetabular osteotomy utilization increased from the 2000-2004 to 2010-2016 periods, respectively: 1 (1%) to 160 (20%; <iP</i = .04), 2 (1%) to 37 (5%; <iP</i = .01), and 1 (1%) to 22 (3%; <iP</i = .58). The overall incidence of FAI diagnosis was 54.4 per 100,000 person-years, and it consistently increased between 2000 and 2016. Female patients had a higher incidence than male patients. The utilization of joint preservation operations, including hip arthroscopy, surgical hip dislocation, and anteverting periacetabular osteotomy, increased over time."}, {"id": "pubmed23n0818_6530", "title": "Strategy and optimization of diagnostic imaging in painful hip in adults.", "score": 0.009009009009009009, "content": "Diagnostic imaging strategy in painful hip depends on many factors, but in all cases, plain X-ray is the first investigation. It may be sufficient to reach diagnosis and determine treatment options. More effective but more expensive exploration is indicated in two circumstances: when plain X-ray is non-contributive, and when diagnosis has been established but more accurate imaging assessment is needed to guide treatment. Following radiography, the choice of imaging techniques depends not only on the suspected pathology but also on the availability of equipment and its performance. MRI is probably the technique that provides the most comprehensive results; recent improved accessibility has significantly simplified the diagnostic algorithm. CT remains invaluable, and current techniques have reduced patient irradiation to a level similar to that of standard X-ray. Finally, cost is an important consideration in choosing the means of exploration, but the overall financial impact of the various strategies for diagnosis of painful hip is not well established. This article aims to provide a simple and effective diagnostic strategy for the assessment of painful hip, taking account of the clinical situation, and to detail the most typical semiologic patterns of each disease affecting this joint. "}, {"id": "pubmed23n0802_25089", "title": "Rapidly destructive inflammatory arthritis of the hip.", "score": 0.008928571428571428, "content": "Rapidly destructive coxarthrosis (RDC) is a rare syndrome that involves aggressive hip joint destruction within 6-12 months of symptom onset with no single diagnostic laboratory, pathological, or radiographic finding. We report an original case of RDC as an initial presentation of seronegative rheumatoid arthritis (RA) in a 57-year-old Caucasian woman presenting with 6 months of progressive right groin pain and no preceding trauma or chronic steroid use. Over 5 months, she was unable to ambulate and plain films showed complete resorption of the right femoral head and erosion of the acetabulum. There were inflammatory features seen on computed tomography (CT) and magnetic resonance imaging (MRI). She required a right total hip arthroplasty, but arthritis in other joints showed improvement with triple disease modifying antirheumatic drugs (DMARD) therapy and almost complete remission with the addition of adalimumab. We contrast our case of RDC as an initial presentation of RA to 8 RDC case reports of patients with established RA. Furthermore, this case highlights the importance of obtaining serial imaging to evaluate a patient with persistent hip symptoms and rapid functional deterioration. "}, {"id": "pubmed23n0114_13440", "title": "[Long-term development of primary osteochondritis of the hip (Legg-Perthes-Calvé). Apropos of 60 hips with a follow-up of more than 30 years].", "score": 0.008928571428571428, "content": "This study concerns 60 hips in 57 patients aged between 33 and 56 years with a follow-up of 30 to 44 years since the onset of the disease. The progress of the condition was assessed by objective and subjective clinical criteria and radiographic criteria including roundness of the head, head height, head cover, abnormal head shape and the presence of arthrosis. The main prognostic feature was found to be the eventual shape of the femoral head. Normal heads (7 cases) and round heads with some flattening (20 cases) had few problems at the time of follow-up. Irregular heads (7 cases) and, most of all, very irregular heads (20 cases) progressed badly. Pain was common, often severe and the patients were disabled. Fourteen of them had consulted a doctor and seven had already been treated surgically. Arthrosis as a consequence of osteochondritis seemed to be due to joint incongruence. We consider that the prognosis can be made at the end of the progressive phase of the disease, since remodelling plays no part in the roundness of the head. These findings indicate the need for care in recommending treatment in the residual phase of the disease and suggest that it is difficult to determine what surgical treatment should be considered in adult life."}, {"id": "pubmed23n0739_14266", "title": "Surgical indications for treatment for femoroacetabular impingement with surgical hip dislocation.", "score": 0.008849557522123894, "content": "There is a lack of detailed information about the indications of surgical treatment for femoroacetabular impingement (FAI), particularly using open surgical dislocation. The purpose of this review was to systematically review the reported indications for surgical dislocation of the hip for FAI. Two databases (MEDLINE and EMBASE) were screened for clinical studies involving the treatment for FAI with surgical hip dislocation. We conducted a full-text review and the references for each included paper were hand-searched for other eligible studies. Papers published until September 2011 were included in this review. Two individuals reviewed all identified studies independently, and any disagreement was resolved through consensus. Fifteen studies met the eligibility criteria, which included a total of 822 patients. We identified a lack of consensus for clinical and radiographic indications for surgical hip dislocation to treat FAI. The most common clinical indications reported were clinical symptoms such as hip pain in 10 papers (67 %), a positive impingement sign in 9 papers (60 %), painful/reduced range of motion in 9 papers (60 %), activity-related groin pain in 4 papers (27 %), and non-responsive to non-operative treatment in 4 papers (27 %). The most commonly reported radiographic indicators for surgical hip dislocation were a variety of impingement findings from radiographs in all 15 included papers (100 %), a combination of radiographs and MRA in 5 papers (33 %) or radiographs and MRI in 3 papers (20 %). These results showed that that there was an inconsistency between the clinical and radiographic indications for surgical hip dislocation as a treatment for femoroacetabular impingement. This review suggests that there is a need for the development of standardized clinical and radiological criteria that serve as guidelines for surgical treatment for FAI. Systematic review, Level IV."}, {"id": "pubmed23n0514_21325", "title": "Surgical treatment of late developmental displacement of the hip. Results after 33 years.", "score": 0.008849557522123894, "content": "The outcome of displaced hips treated by Somerville and Scott's method was assessed after more than 25 years. A total of 147 patients (191 displaced hips) was reviewed which represented an overall follow-up of 65.6%. The median age at the index operation was two years. During the first five years, 25 (13%) hips showed signs of avascular change. The late development of valgus angulation of the neck, after ten years, was seen in 69 (36%) hips. Further operations were frequently necessary. Moderate to severe osteoarthritis developed at a young age in 40% of the hips. Total hip replacement or arthrodesis was necessary in 27 (14%) hips at a mean age of 36.5 years. Risk factors identified were high dislocation, open reduction, and age at the original operation. Two groups of patients were compared according to outcome. All the radiographic indices were different between the two groups after ten years, but most were similar before. It takes a generation to establish the prognosis, although some early indicators may help to predict outcome."}, {"id": "wiki20220301en023_61820", "title": "Juvenile idiopathic arthritis", "score": 0.008771929824561403, "content": "At the time of receiving a JIA diagnosis, children and their families often have many questions regarding prognosis. Recent therapeutic advances in the management of JIA have made inactive disease and clinical remission achievable goals for the majority of children with access to modern treatments. Clinical remission can be defined as the absence of signs and symptoms of inflammatory disease activity, including extra-articular manifestations of the disease. Differentiating subtypes of JIA helps to target treatment and leads to more positive outcomes, however subtype is not the only predictor of JIA outcome. Poor prognostic factors include arthritis of the hip, cervical spine, ankles or wrists; prolonged elevation of inflammatory markers; and radiographic evidence of joint damage including erosions or joint space narrowing. Patients with RF-positive polyarthritis often have worse outcomes associated with more aggressive disease. Despite this, the probability of this subgroup achieving"}, {"id": "pubmed23n0067_15485", "title": "The American College of Rheumatology criteria for the classification and reporting of osteoarthritis of the hip.", "score": 0.008771929824561403, "content": "Clinical criteria for the classification of patients with hip pain associated with osteoarthritis (OA) were developed through a multicenter study. Data from 201 patients who had experienced hip pain for most days of the prior month were analyzed. The comparison group of patients had other causes of hip pain, such as rheumatoid arthritis or spondylarthropathy. Variables from the medical history, physical examination, laboratory tests, and radiographs were used to develop different sets of criteria to serve different investigative purposes. Multivariate methods included the traditional \"number of criteria present\" format and \"classification tree\" techniques. Clinical criteria: A classification tree was developed, without radiographs, for clinical and laboratory criteria or for clinical criteria alone. A patient was classified as having hip OA if pain was present in combination with either 1) hip internal rotation greater than or equal to 15 degrees, pain present on internal rotation of the hip, morning stiffness of the hip for less than or equal to 60 minutes, and age greater than 50 years, or 2) hip internal rotation less than 15 degrees and an erythrocyte sedimentation rate (ESR) less than or equal to 45 mm/hour; if no ESR was obtained, hip flexion less than or equal to 115 degrees was substituted (sensitivity 86%; specificity 75%). Clinical plus radiographic criteria: The traditional format combined pain with at least 2 of the following 3 criteria: osteophytes (femoral or acetabular), joint space narrowing (superior, axial, and/or medial), and ESR less than 20 mm/hour (sensitivity 89%; specificity 91%). The radiographic presence of osteophytes best separated OA patients and controls by the classification tree method (sensitivity 89%; specificity 91%). The \"number of criteria present\" format yielded criteria and levels of sensitivity and specificity similar to those of the classification tree for the combined clinical and radiographic criteria set. For the clinical criteria set, the classification tree provided much greater specificity. The value of the radiographic presence of an osteophyte in separating patients with OA of the hip from those with hip pain of other causes is emphasized."}, {"id": "pubmed23n0826_17816", "title": "BILATERAL RAPIDLY DESTRUCTIVE HIP OSTEOARTHRITIS: A CASE REPORT.", "score": 0.008695652173913044, "content": "Although hip osteoarthritis usually shows a slow progression, a rapidly destructive osteoarthritis is observed in approximately 10% of patients. We aimed to present a case with rapidly destructive osteoarthritis in bilateral hip joints. A 78-year-old male patient was admitted due to pain in hip joints. In examination, hip movements were minimally painful and limited. The patient was able to walk independently with a cane. When he re-applied six months later, hip movements were severely limited and painful. In plain radiographs, while a slight narrowing in hip joint space, sclerosis and minimal osteophyte had been observed at the first administration, extreme narrowing, subluxation, flattening of femoral head, increased sclerosis, resorption in femoral head and acetabulum were detected six months later. We consider that hip osteoarthritis in elderly people should be monitored at frequent intervals in terms of clinic and radiological progression. "}, {"id": "pubmed23n0116_5410", "title": "A reappraisal of 'analgesic hip'.", "score": 0.008695652173913044, "content": "Nineteen patients with hip radiographs typical of 'analgesic hip' (rapidly destructive, atrophic arthropathy involving both femoral and acetabular components) have been studied. Women predominated (14:5), and all were elderly (mean age 74 years, range 64-83 years). Destructive hip disease was unilateral in all but one case. The mean interval from symptom onset to typical x ray appearance was short (one year, range three to 24 months), and persistent pain unresponsive to drug therapy was characteristic. Screening showed no metabolic or neurological disease. Contrary to previous reports, non-steroidal anti-inflammatory drugs could not be incriminated in development of the disease. Clinical and radiographic similarity to apatite associated destructive arthritis of other large joints was striking, and occurrence of the latter, uncommon condition in five patients (five shoulders, two knees) suggests that both descriptions represent a common articular response at different joint sites."}]}}}}
{"correct_option": 5, "explanations": {"1": {"exist": true, "char_ranges": [[100, 191]], "word_ranges": [[18, 31]], "text": "significant self-induced weight loss, body image distortion and amenorrhea. So we remove 1."}, "2": {"exist": true, "char_ranges": [[485, 559]], "word_ranges": [[85, 98]], "text": "There is no anxiety clinic and she also meets the criteria for depression."}, "3": {"exist": true, "char_ranges": [[244, 379]], "word_ranges": [[40, 64]], "text": "3 does not make sense in the MIR because of the clinic -in reality the history is different and in psychiatry everything is evolution-."}, "4": {"exist": true, "char_ranges": [[380, 434]], "word_ranges": [[64, 75]], "text": "4 has to meet temporal criteria that are not met here."}, "5": {"exist": true, "char_ranges": [[485, 559]], "word_ranges": [[85, 98]], "text": "There is no anxiety clinic and she also meets the criteria for depression."}}, "full_answer": "As soon as we hear about weight we go to anorexia nervosa, but anorexia nervosa has three features: significant self-induced weight loss, body image distortion and amenorrhea. So we remove 1. One loses weight when one gets sad, worries, etc... 3 does not make sense in the MIR because of the clinic -in reality the history is different and in psychiatry everything is evolution-. 4 has to meet temporal criteria that are not met here. So we are between 2 and 5. Depression or anxiety? There is no anxiety clinic and she also meets the criteria for depression. The poor girl has been diagnosed with depression. The answer is 5 (another option is that she was studying the MIR).", "full_answer_no_ref": "As soon as we hear about weight we go to anorexia nervosa, but anorexia nervosa has three features: significant self-induced weight loss, body image distortion and amenorrhea. So we remove 1. One loses weight when one gets sad, worries, etc... [HIDDEN] -in reality the history is different and in psychiatry everything is evolution-. [HIDDEN]. So we are between 2 and 5. Depression or anxiety? There is no anxiety clinic and she also meets the criteria for depression. The poor girl has been diagnosed with depression. [HIDDEN] (another option is that she was studying the MIR).", "full_question": "A 19-year-old female university student comes to the clinic accompanied by her parents, reporting feeling progressively more asthenic in the last two months, with loss of appetite and weight and with greater difficulty in concentrating on her studies. The anamnesis also highlights that she has lost interest in going out with friends, has ideas of death without self-harming ideation and pessimistic cognitions of the future. Her weight is 90% of that considered ideal for age and gender. She does not present weight phobia or body image distortion. The most appropriate diagnosis is:", "id": 147, "lang": "en", "options": {"1": "Anorexia nervosa.", "2": "Anxiety disorder.", "3": "Borderline personality disorder.", "4": "Dysthymia.", "5": "Major depressive disorder."}, "question_id_specific": 157, "type": "PSYCHIATRY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en004_7205", "title": "Growing Pains", "score": 0.014080459770114942, "content": "In 1990, Tracey Gold began group therapy in an eating disorder program but only learned more ways to lose weight. That season, her weight loss problem was touched upon slightly on her television series, when Gold is seen looking at her body in a carnival mirror and describes to another character the distorted image in her head. In 1991, she started starving herself more than ever and vomiting, and lost a massive amount of weight, to the point that she was admitted to a hospital in early 1992. Her lowest weight is estimated to have been near 80 pounds. She was suspended from the show for her skeletal appearance. Photos of Gold's emaciated body were plastered all over tabloid magazines, and she was one of the first celebrities ever to be formally outed for anorexia. She last appeared in the 1991 episode, \"Menage a Luke,\" after missing the two prior episodes where her problem is very obvious in some scenes and did not return until the last two episodes of the series in the late spring of"}, {"id": "wiki20220301en606_25015", "title": "The Truth About Size Zero", "score": 0.012858619798397518, "content": "During the course of the documentary, Redknapp visited different locations to talk to people. She visited with friend and actress Denise van Outen in Los Angeles, and they discussed van Outen's experiences with people the pressure to lose weight. She went to Rhodes Farm Clinic to meet with several young women who are recovering from anorexia or bulimia to talk about their experiences with eating disorders. She also met with Spice Girl Mel C (\"Sporty Spice\") to discuss her severe eating disorder during her time the Spice Girls and the media portrayal. She met with her school friend, \"Sophie,\" who had suffered with a severe eating disorder during her teenage years, and they talked about the experience of low caloric intake. She visited her old stage school the Italia Conti Academy of Theatre Arts to talk with students and warn them about the horrors of crash dieting. She presented Clothes Show Live in Birmingham, and while she was there, she interviewed some models, and talked to a"}, {"id": "Neurology_Adams_12101", "title": "Neurology_Adams", "score": 0.011757575757575757, "content": "Reports concerning the percentage of first-degree relatives of anorectic patients with bipolar disease are also inconsistent. An increased prevalence of neurosis or alcoholism has been noted in other members of the family. However, psychiatrists seem to agree that the patient does not have symptoms that conform to any of the major neuroses or psychoses. Certainly loss of appetite, lack of self-esteem and interest in personal appearance, and self-destructive behavior—common features of anorexia nervosa—are also symptoms of depressive illness, yet most of the patients do not look or admit to being dejected. Moreover, endogenous depression affects both sexes. The pathologic fear of becoming fat and the obsession with weight might be interpreted as a phobic or obsessional neurosis."}, {"id": "pubmed23n0876_17161", "title": "Trichotillomania: Bizzare Patern of Hair Loss at 11-Year-old Girl.", "score": 0.009900990099009901, "content": "Trichotillomania (TTM) is defined by the Diagnostics and Statistic Manual of Mental Disorders, 4th edition (DMS-IV) as hair loss from a patient`s repetitive self-pulling of hair. The disorder is included under anxiety disorders because it shares some obsessive-compulsive features. Patients have the tendency towards feelings of unattractiveness, body dissatisfaction, and low self-esteem (1,2). It is a major psychiatric problem, but many patients with this disorder first present to a dermatologist. An 11-year-old girl came to our department with a 2-month history of diffuse hair loss on the frontoparietal and parietotemporal area (Figure 1). She had originally been examined by a pediatrician with the diagnosis of alopecia areata. The patient`s personal history included hay fever and shortsightedness, and she suffered from varicella and mononucleosis. Nobody in the family history suffered from alopecia areata, but her father has male androgenetic alopecia (Norwood/Hamilton MAGA C3F3). The mother noticed that the child had had changeable mood for about 2 months and did not want to communicate with other persons in the family. The family did not have any pet at home. At school, her favorite subjects were Math and Computer Studies. She did not like Physical Education and did not participate in any sport activities during her free time. This was very strange because she was obese (body-mass index (BMI) 24.69). She was sometimes angry with her 13-year-old sister who had better results at school. The girl had suddenly started to wear a blue scarf. The parents did not notice that she pulled out her hair at home. Dermatological examination of the capillitium found a zone of incomplete alopecia in the frontoparietal and parietotemporal area, without inflammation, desquamation, and scaring. Hairs were of variable length (Figure 1). There was a patch of incomplete alopecia above the forehead between two stripes of hair of variable length (Figure 2). The hair pull test was negative along the edges of the alopecia. Mycological examination from the skin capillitium was negative. The trichoscopy and skin biopsy of the parietotemporal region of the capillitium (Figure 3) confirmed trichotillomania. Laboratory tests (blood count, iron, ferritin, transferrin, selenium, zinc, vitamin B12, folic acid, serology and hormones of thyroid gland) were negative. We referred the girl for ophthalmologic and psychological examination. Ophthalmologic examination proved that there was no need to add any more diopters. The psychological examination provided us with a picture in which she drew her family (Figure 4). The strongest authority in the family was the mother because she looked after the girls for most of the day. She was in the first place in the picture. The father had longer working hours and spent more time outside the home. He worked as a long vehicle driver. He was in the second place in the picture. There was sibling rivalry between the girls, but the parents did not notice this problem and preferred the older daughter. She was successful at school and was prettier (slim, higher, curly brown hair, without spectacles). Our 11-years-old patient noticed all these differences between them, but at her level of mental development was not able to cope with this problem. She wanted to be her sister's equal. The sister is drawn in the picture in the third place next to father, while the patient's own figure was drawn larger and slim even though she was obese. Notably, all three female figures had very nice long brown hair. It seemed that the mother and our patient had better quality of hair and more intense color than the sister in the drawing. The only hairless person in the picture was the father. The girl did not want to talk about her problems and feelings at home. Then it was confirmed that our patient was very sensitive, anxious, willful, and withdrawn. She was interested in her body and very perceptive of her physical appearance. From the psychological point of view, the parents started to pay more interest to their younger daughter and tried to understand and help her. After consultation with the psychiatrist, we did not start psychopharmacologic therapy for trichotillomania; instead, we started treatment with cognitive behavioral therapy, mild shampoo, mild topical steroids (e.g. hydrocortisone butyrate 0.1%) in solution and methionine in capsules. With parents' cooperation, the treatment was successful. The name trichotillomania was first employed by the French dermatologist Francois Henri Hallopeau in 1889, who described a young man pulling his hair out in tufts (3-5). The word is derived from the Greek thrix (hair), tillein (to pull), and mania (madness) (5). The prevalence of TTM in the general adult population ranges from 0.6% to 4%, and 2-4% of the general psychiatric outpatient population meet the criteria for TTM (2-5). The prevalence among children and adolescents has been estimated at less than 1% (5). The disease can occur at any age and in any sex. The age of onset of hair pulling is significantly later for men than for women (3). There are three subsets of age: preschool children, preadolescents to young adults, and adults. The mean age of onset is pre-pubertal. It ranges from 8 to 13 years (on average 11.3 years) (2-5). The occurrence of hair-pulling in the first year of life is a rare event, probably comprising &lt;1% of cases (5). The etiology of TTM is complex and may be triggered by a psychosocial stressor within the family, such as separation from an attachment figure, hospitalization of the child or parent, birth of a younger sibling, sibling rivalry, moving to a new house, or problems with school performance. It has been hypothesized that the habit may begin with \"playing\" with the hair, with later chronic pulling resulting in obvious hair loss (2). Environment is a factor because children usually pull their hair when alone and in relaxed surroundings. The bedroom, bathroom, or family room are \"high-risk\" situations for hair-pulling (5). Men and women also differed in terms of the hair pulling site (men pull hair from the stomach/back and the moustache/beard areas, while women pull from the scalp) (3). Pulling hair from siblings, pets, dolls, and stuffed animals has also been documented, often occurring in the same pattern as in the patient (5). Genetic factors contributing to the development of TTM are mutations of the SLITRK1 gene, which plays a role in cortex development and neuronal growth. The protein SAPAP3 has been present in 4.2% of TTM cases and patients with obsessive-compulsive disorder (OCD). It may be involved in the development of the spectrum of OCD. A significantly different concordance rate for TTM was found in monozygotic (38.1%) compared with dizygotic (0%) twins in 34 pairs (3). The core diagnostic feature is the repetitive pulling of hairs from one`s own body, resulting in hair loss. The targeted hair is mostly on the scalp (75%), but may also be from the eyebrows (42%), eyelashes (53%), beard (10%), and pubic area (17%) (3,5). There are three subtypes of hair pulling - early onset, automatic, and focused. Diagnostic criteria for TTM according to DSM-IV criteria are (2,3,5): 1) recurrent pulling of one`s hair resulting in noticeable hair loss; 2) an increasing sense of tension immediately prior to pulling out the hair or when attempting to resist the behavior; 3) pleasure, gratification, or relief when pulling out the hair; 4) the disturbance is not better accounted for by another mental disorder and is not due to a general medical condition (e.g., a dermatologic condition); 5) the disturbance causes clinically significant distress or impairment in social, occupational, or other important areas of functioning. The differential diagnosis includes alopecia areata (Table 1) (6), tinea capitis, telogen effluvium, secondary syphilis, traction alopecia, loose anagen syndrome, lichen planopilaris, alopecia mucinosa, and scleroderma (2-5). Biopsy of an involved area (ideally from a recent site of hair loss) can help to confirm the diagnosis (5). On histologic examination, there are typically increased numbers of catagen and telogen hairs without evidence of inflammation. Chronic hair pulling induces a catagen phase, and more hairs will be telogen hairs. Pigment casts and empty anagen follicles are often seen. Perifollicular hemorrhage near the hair bulb is an indicator of TTM (2). Complications of TTM are rare, but they comprise secondary bacterial infections with regional lymphadenopathy as a result of picking and scratching at the scalp. Many patients play with and ingest the pulled hairs (e.g. touching the hair to lips, biting, and chewing). Trichophagia (ingestion of the hair) can lead to a rare complication named trichobezoar (a \"hair ball\" in stomach). This habit is present in approximately 5% to 30% of adult patients, but it is less frequent in children. Patient with trichophagia present with pallor, nausea, vomiting, anorexia, and weight loss. Radiologic examination and gastroscopy should not be delayed (2,4,5). The management of the disease is difficult and requires strong cooperation between the physician, patient, and parents. The dermatologist cannot take part in the therapy, strictly speaking, but without the psychological, psychopharmacologic, and topic dermatologic treatment a vicious circle will be perpetuated. "}, {"id": "pubmed23n0906_14373", "title": "[Treatment Outcome in Female In-Patients with Anorexia nervosa and Comorbid Personality Disorders Prevalence - Therapy Drop out and Weight Gain].", "score": 0.009900990099009901, "content": "<bIntroduction</b Personality disorders (PD) are among the most common comorbid disorders in female patients with Anorexia Nervosa (AN). Recent research findings suggest that comorbid PD are associated with a higher treatment drop-out rate and a worse therapeutic outcome. However, no study to date has distinguished between certain age groups concerning these issues. <bResearch questions</b Therefore, the present study focuses on the prevalence of PD (1), treatment drop-out rates (2) and weight gain (3) in female in-patients with AN. Thereby, we differentiate among three age groups (17-24 years; 25-34 years; 35-65 years). <bMaterial &amp; Methods</b We assessed female in-patients (N=331) with AN at the Helios Clinic in Bad Grönenbach in Germany using the Eating Disorders Inventory-2 and the psychotherapeutic-medical basic documentation at the beginning and at the end of their treatment. Furthermore, we investigated the drop-out rate and weight gain by comparing anorexic patients with and without comorbid PD that were diagnosed by clinicians using ICD-10 criteria. <bResults</b In sum, our patients with AN demonstrated a prevalence rate of 34% for one or more comorbid PD. Interestingly, patients between 17-24 years showed a lower prevalence rate of 22% compared to those between 25-34 years (42%) and 35-65 years (41%). Furthermore, younger age and comorbid PD seemed to be significant predictors for treatment dropout. One of the most striking results was that younger patients (17-24) without a comorbid PD had the highest weight gain during treatment. This could not be observed in patients with a comorbid PD, who demonstrated the highest weight gain between 25 and 34 years of age. <bConclusion</b Our findings support the hypothesis that comorbid PD are related to a worse outcome in patients with eating disorders. Future studies might do well in assessing dimensional scores of personality disorders and other relevant aspects like for example the amount of social support to draw further conclusions on these associations. Our results emphasize the need for more disorder-specific interventions tailoring at patients with AN and comorbid PD to improve treatment outcome."}, {"id": "pubmed23n1141_20355", "title": "Letter to the Editor: Depression As The First Symptom Of Frontal Lobe Grade 2 Malignant Glioma.", "score": 0.00980392156862745, "content": "Dear Editor, Next to focal neurological symptoms, epileptic seizures and head aches, brain tumors can less frequently bring about cognitive changes, slowed speech, difficulty sustaining mental functioning and psychiatric symptoms of personality changes and. loss of interest in daily activities, these symptoms may be evaluated as anxiety or depression. Depression is known to be a complication of brain tumours and may sometimes be seen after the presentation of neurological symptoms linked to brain tumours, and sometimes after tumor treatment (Oğuz et al. 2005, Litofsky et al. 2004, Moise and Madhusoodanan 2006, Oreskovic M et al. 2007, Rooney A et al. 2010). The dorsolateral prefrontal, orbitofrontal and medial frontal circuits constitute the three subcortical neuronal circuits in the frontal cortex. The dorsolateral prefrontal circuit is associated with planning and operational functions and lesions on it may give rise to apathy, abulia, perseveration, personality changes and planning disorder. Lesions involving the orbitofrontal circuit, which is associated with response suppression and disinhibition, may involve emotional lability and memory problems. Whereas lesions affecting the right orbitofrontal circuit give rise to elevated mood, lesions on the left orbitofrontal circuit lead to depressed mood. In cases with medial frontal circuit involvement, akinetic mutism may result from lesions in the superior medial region and anteroretrograde amnesia and confabulation are observed with lesions in the inferior medial region (Tosun et al. 2016, Chirchiglia 2018). A diagnosis of psychiatric disorder may be given during the first examination of patieants with primary brain tumours, especially if localized in the frontal lobe. Thorough history taking and physical examination are necessary for early diagnosis. The case reported here concerns a 29-year-old university graduate female patient, living with her partner and children, who consulted the clinic with complaints of tendency to frequent crying, anhedonia, having difficulty with speech fluency, forgetfulness and distractedness that had presented suddenly, 2 months previously, without any causative stressor. In her mental status examination, she appeared having normal self-care with appearance at her actual age. She was fully conscious and oriented, not willing to cooperate with the interview, had distinct difficulty in maintaining attention and with fluency of speech. Her mood was depressive. She described loss of appetite, fatigue and energy loss. Her difficulty in paying attention was pronounced. She did not have a history of psychotropic medication use or family history of psychiatric disease. She did not smoke or use alcohol or substance. After evaluating the clinical interview, a preliminary diagnosis of major depressive disorder was considered on the basis of the DSM-5 criteria. Routine blood tests were requested. Given the continuation of her complaints, the difficulty with fluent speech and the increase in tendency to sleep at the first week follow up, cranial MRI was planned. The MRI results showed on the right, in the frontal lobe a multilocular mass with precallosal extension, undiscernable margins with the right lateral aspect of the corpus callosum genu and dispersed cystic-necrotic areas with T2 signal series. The dimensions of the mass were nearly 5 x 3 cm causing a 1-cm right-to-left shift of the midline (Figure 1) DEPRESSION AS THE FIRST SYMPTOM OF FRONTAL LOBE GRADE 2 MALIGNANT GLIOMA 2 Türk Psikiyatri Dergisi 2 Turkish Journal of Psychiatry Letter to the Editor 143 144 The patient was referred for surgery with the preliminary diagnosis of high-grade glial tumour. Pathology results identified a grade 2 glioma. It was learned that radiotherapy sessions were begun after surgery. The patient did not have any symptoms of psychopathology during the 2 monthly psychiatric interviews made after surgery. Brain tumours generally indicate their presence with headache, seizures and other neurological symptoms and very rarely with depression as seen in the case of our patient. It should be kept in mind that atypical psychiatric symptoms may have an underlying organic lesion and subtle neurological symptoms should be investigated in detail. A recent meta-analysis on 37 observational studies determined a 21.7% prevalence of depression in a total of 4518 patients with intracranial tumours. Comorbidity of depression with brain tumor was demonstrated to worsen the quality of life, increase suicidal risk and lower the chance of survival (Huang et al. 2017). The possibility of psychiatric symptoms being the clinical clues for brain cancer was noted and the necessity of neuroimaging tests in cases of recent-onset psychosis or mood disorder symptoms, atypical personality changes and anorexia without body dysmorphic disorder was emphasized (Madhusoodanan et al. 2015). Loss of interest, tendency to frequent weeping, introversion and anhedonia were the sole complaints in the case discussed here. The increase in psychomotor retardation and slowing down of movements at the very first weekly control follow up necessitated neuroimaging. Despite the reports in the literature on the frequent association of unpreventable excessive behavior, disinhibition and irritability with right frontal injury and lesions (Okumuş and Hocaoğlu 2018), depression was the dominant symptom in the case presented here. There are differences between primary major depression and depression presenting with underlying somatic diseases which is known to occur at later ages (Rouchell et al. 2002). However, our patient was aged 29 years. Also, cases of depression due to somatic disease are less associated with family history of depression and suicidal ideation and attempts, while cognitive symptoms come to the foreground during mental status examination. (Sertöz and Mete 2004, Rouchell et al. 2002). Our patient did not have suicidal ideation or attempts, or a family history of depression. In apathy, which may be explained as emotional blunting, indifference or detachment from the external world, targeted behavior is also reduced next to the lack of emotional expression. The individual discussed here was learned not to sit at the table or change the television channel unless reminded to do so. When the reason was asked, she could not think of one. The reduction in emotional expression accompanies reduced insight, abulia and lack of empathy (Sözeri Varma et al. 2019). In depression, apathy is defined as 'sorrowless depression'. Our patient cried but had very blunted mimics and gestures. She explained that she could not help weeping even at times when she did not feel internally distressed. The seriousness of apathy, as a symptom difficult to differentiate from depression, is still not understood. Neuroimaging Figure 1- Cranial MRI of the patient 145 Received: 16.08.2020, Accepted: 04.12.2020, Available Online Date: 05.10.2021 1MD., Antalya Kepez State Hospital, Department of Psychiatry, Antalya, 2MD., Ordu University Training and Research Hospital, Department of Psychiatry, Ordu, Turkey e-mail: bosbora@yahoo.com https://doi.org/10.5080/u25957 studies indicate apathy to be a reflect of impaired frontal-subcortical circuits and the functional disorder of the connections between the ventromedial prefrontal cortex and the basal ganglia (Chase 2011). Comparison of 45 individuals with depression due to aging and 43 healthy individuals showed apathy to be associated with fronto-limbic gray and white matter abnormalities which continued after antidepressant treatment. The structural anomalies of the posterior subgenual cingulate gyrus and the uncinate fasciculus were discussed (Yuen 2014). The case discussed here is presented to emphasize the importance of brain imaging methods and detailed investigation of atypical symptoms for diagnostic approaches to psychiatric disorders. Especially, complaints at young age of depression with psychomotor retardation, reduced fluency of speech and sudden onset withdrawal without stressors should be a warning of secondary depression. Yours sincerely... Şerif Bora Nazlı1 , Muhammet Sevindik2 REFERENCES Chase TN (2011) Apathy in Neuropsychiatric Disease: Diagnosis, Pathophysiology, and Treatment. Neurotox Res 19:266-78. Chirchiglia D (2018) Pseudodepression as an Anticipatory Symptom of Frontal Lobe Brain Tumors. Int J Depress Anxiety 1:007. Huang J, Zeng C, Xiao J et al (2017) Association between depression and brain tumor: a systematic review and meta-analysis. Oncotarget 8:94932-43. Litofsky NS, Farace E, Anderson F et al (2004) Depression in patients with high-grade glioma: Results of the glioma outcomes project. Neurosurgery 54:358-67. Madhusoodanan S, Ting MB, Farah T et al (2015) Pyschiatric aspects of brain tumors: A review. World J Psychiatry 5:273-85. Moise D, Madhusoodanan S (2006) Psychiatric symptoms associated with brain tumors: a clinical enigma. CNS Spectr 2006;11:28-31. Oğuz N, Ilnem C, Yener F (2005) Psychiatric symptoms in brain tumors: Case reports. Bulletin of Clinical Psychopharmacology 15:18-21. Hocaoğlu Ç, Okumuş B (2018) Psychiatric manifestations and brain tumor: A case report and brief review. The Medical Journal of Mustafa Kemal University 9:42-9. Oreskovic NM, Strother CG, Zibners LM (2007) An unusual case of a central nervous system tumor presenting as a chief complaint of depression. Pediatric Emergency Care 23:486-8. Rooney A, Carson A, Grant R (2011) Depression in cerebral glioma patients: a systematic review of observational studies. J Natl Cancer Inst103:61-76. Rouchell AM, Pounds R, Tierney JG (2002) Depression Textbook of Consultation-Liaison Psychiatry, 2nd Edition, Volume 1. MG Wise, JR Rundell (Ed), Washington DC American Psychiatric Publishing, Inc, p.307-38. Özen SÖ, Hayriye ME (2004) Bedensel Hastalıklarda Depresyon. Klinik Psikiyatri Ek 2:63-9. Sözeri Varma G , Bingöl C , Topak O et al (2019) Relationship of apathy with depressive symptom severity and cognitive functions in geriatric depression. Arch Neuropsychiatry 56:133-8. Yuen GS, Gunning FM, Woods E et al (2014) Neuroanatomical correlates of apathy in late-life depression and antidepressant treatment response. J Affect Disord 166:179-86."}, {"id": "wiki20220301en047_6071", "title": "Stacy London", "score": 0.00980392156862745, "content": "In the early 1990s, London struggled with anorexia, binge eating, and other weight issues. Standing , she was at her lightest weight and at her heaviest. In a 2007 interview with Sirens magazine, she said of the experience, \"I have been every size in my life. I've been smaller than a zero, up through a size 16. I've had lots of issues with body image and weight my whole life and it really took a great deal of work to recognize that at all those weights, no matter how I felt, I could still find a dress that made me feel sexy and powerful.\""}, {"id": "pubmed23n0824_6126", "title": "A 9-year-old girl with persistent obsessive and compulsive behaviors in a primary care pediatric practice.", "score": 0.009708737864077669, "content": "Chloe is a 9-year-old gal whose mother made an initial visit to a new pediatrician for concerns about her behavior. Chloe is apprehensive about the visit and frequently hides behind her mother.Her parents first noticed Chloe becoming angry and more emotional 3 years ago, which her parents did not initially understand. However, over the past year, she has started to have more worries and unusual behavior.Chloe and her mother report that when she walks through doorways, she will almost always go back and walks through again. At home, she will walk through doorways multiple times and at school, she will pretend she forgot something so her friends do not notice. She often will not walk downstairs and occasionally her mother has to carry her. Clothes are problematic for Chloe. If her father touches something of a specific color and then touches Chloe, she will have to change her clothes or take a shower. Sometimes, she will never be able to wear those clothes again. She had a recent episode where she could not stop tapping a red paper, because if she stopped, she said it would burst into flame. During the 2 weeks before the pediatric visit, symptoms increased to the point that she is now refusing to go to school. When she stays home, she lays in 1 place all day.Chloe is a fourth grade student. The family does not report academic concerns. She has friends. She denies any appetite or sleep problems. She endorses periods of sadness, lack of energy, and decreased interest in social activities, mostly because she worries and is embarrassed. She kept her behaviors hidden from her 5 siblings for the past year, and she talked only to her mother about them. She is worried her friends might discover her behaviors.The family history is notable for multiple paternal family members with anxiety and bipolar disorder and depression on mother's side. A few months ago, Chloe's family adopted a 7-year-old child with special needs from China.Her growth, vital signs, and physical examination are unremarkable. Her mother filled out the Short Mood and Feelings Questionnaire and the Screen for Child Anxiety-Related Emotional Disorders, which both had elevated scores."}, {"id": "wiki20220301en095_16514", "title": "Debbie Martin", "score": 0.009708737864077669, "content": "Reece-Wilmore revealed she came up with the idea for the bulimia storyline, saying \"When I came back from my Christmas holiday, I'd lost tons of weight. Everyone commented on my new figure and I thought to myself 'If there was anything wrong with me, those sorts of comments would have made me really paranoid about my weight.'\" The actress then suggested the storyline, believing it was a subject familiar with a lot of teenage girls. The Neighbours bosses were initially \"wary\" of tackling such a difficult and sensitive storyline and a spokesperson said \"We try to tackle serious issues such as bulimia as much as possible. We may not be able to wave a magic wand and make the problems go away, but by dealing with current situations they become topics for wider discussion.\" Reece-Wilmore added that she could see how eating disorders were connected to teenagers' anxieties about approaching adulthood as she used to make herself sick to avoid going to school."}, {"id": "pubmed23n0895_1203", "title": "A 9-Year-Old Girl With Persistent Obsessive and Compulsive Behaviors in a Primary Care Pediatric Practice.", "score": 0.009615384615384616, "content": "Chloe is a 9-year-old gal whose mother made an initial visit to a new pediatrician for concerns about her behavior. Chloe is apprehensive about the visit and frequently hides behind her mother.Her parents first noticed Chloe becoming angry and more emotional 3 years ago, which her parents did not initially understand. However, over the past year, she has started to have more worries and unusual behavior.Chloe and her mother report that when she walks through doorways, she will almost always go back and walks through again. At home, she will walk through doorways multiple times and at school, she will pretend she forgot something so her friends do not notice. She often will not walk downstairs and occasionally her mother has to carry her. Clothes are problematic for Chloe. If her father touches something of a specific color and then touches Chloe, she will have to change her clothes or take a shower. Sometimes, she will never be able to wear those clothes again. She had a recent episode where she could not stop tapping a red paper, because if she stopped, she said it would burst into flame. During the 2 weeks before the pediatric visit, symptoms increased to the point that she is now refusing to go to school. When she stays home, she lays in 1 place all day.Chloe is a fourth grade student. The family does not report academic concerns. She has friends. She denies any appetite or sleep problems. She endorses periods of sadness, lack of energy, and decreased interest in social activities, mostly because she worries and is embarrassed. She kept her behaviors hidden from her 5 siblings for the past year, and she talked only to her mother about them. She is worried her friends might discover her behaviors.The family history is notable for multiple paternal family members with anxiety and bipolar disorder and depression on mother's side. A few months ago, Chloe's family adopted a 7-year-old child with special needs from China.Her growth, vital signs, and physical examination are unremarkable. Her mother filled out the Short Mood and Feelings Questionnaire and the Screen for Child Anxiety-Related Emotional Disorders, which both had elevated scores."}, {"id": "pubmed23n0881_25530", "title": "", "score": 0.009615384615384616, "content": "Bulimia sufferer Joanna Goodfellow is staging an exhibition of photographs portraying the distorted perceptions she has of her body and the self-hatred she sometimes feels. Joanna has suffered from an eating disorder for the past five years and wants people to understand the 'frustration and anger' she feels when she looks at her bod)'. The exhibition, at the Barbican Theatre in Plymouth, runs until February 12."}, {"id": "pubmed23n0895_1220", "title": "Inattentive Attention-Deficit/Hyperactivity Disorder, Stimulant Medication, and Weight Loss in a 15-Year-Old Girl: Are We Enabling the Development of an Eating Disorder?", "score": 0.009523809523809525, "content": "Nicole is a 15-year-old girl presenting to the Developmental Behavioral Pediatrics Clinic with symptoms of the inattentive type of Attention-Deficit/Hyperactivity Disorder (ADHD) and declining school performance over the last year. She expressed frustration over her inability to concentrate on schoolwork. Assuming that her poor grades were secondary to lack of effort, her parents withdrew privileges. Nicole became increasingly depressed. She stopped participating in activities, she previously enjoyed, and her parents reported that she stopped singing in the shower. After talking to a cousin with ADHD, Nicole concluded that she had ADHD as well. She asked her parents to arrange for an evaluation.Nicole met DSM-5 criteria for the diagnosis of inattentive ADHD and was started on a stimulant medication (mixed amphetamine salts). She had symptoms of a coexisting depression, although she did not meet criteria for diagnosis of a depressive disorder. At a 3-week follow-up visit, she showed improvement in targeted ADHD symptoms; homework was now easier and her grades improved. At a 2-month follow-up, Nicole's weight dropped from 53 kg (47th percentile) prestimulant treatment to 49 kg (31st percentile). She reported appetite suppression after taking the stimulant but did not feel that her eating habits had changed significantly. Her father reported that she had a preference for junk food and snacks. Nicole did not enjoy exercising and did not participate in extracurricular sports.She weighed herself several times a day, as she was worried about losing too much weight. Nicole's mood continued to be low, despite the fact that her grades improved, and her parents were more understanding of her challenges. She was otherwise healthy and reported regular menstrual cycles. Nicole requested an increase in the dose of stimulant medication for greater improvement in concentration during homework and in school.Her pediatric clinician was concerned about the possibility of an eating disorder in addition to depression. She asked herself, \"Are we treating inattentive ADHD effectively or are we enabling an eating disorder?\""}, {"id": "wiki20220301en490_931", "title": "Paul Cosford", "score": 0.009523809523809525, "content": "Initially, Cosford had planned a career in general practice, but following the loss of a child to a genetic disorder, he moved to north west London to take up psychiatry and worked with people with learning difficulties and severe mental illness. In 1990, he became lecturer in psychiatry at St Mary’s. In 1992, he co-authored a paper titled \"Eating disorders in later life: A review\", which was published in the International Journal of Geriatric Psychiatry. It reported that not all anorexics were young, that the desire to be thin and happy with one's body did not lessen as women aged, and that eating disorders in older women could appear as a new problem, or recurrence or persistence of an earlier problem. It often occurred in association with severe depression or obsessive compulsive disorder, and the most common trigger for developing an eating disorder later in life, he noted, was the death of a spouse or a loved-one. He is noted to have membership of the Royal College of"}, {"id": "pubmed23n0841_23322", "title": "Inattentive Attention-Deficit/Hyperactivity Disorder, Stimulant Medication, and Weight Loss in a 15-Year-Old Girl: Are We Enabling the Development of an Eating Disorder?", "score": 0.009433962264150943, "content": "Nicole is a 15-year-old girl presenting to the Developmental Behavioral Pediatrics Clinic with symptoms of the inattentive type of Attention-Deficit/Hyperactivity Disorder (ADHD) and declining school performance over the last year. She expressed frustration over her inability to concentrate on schoolwork. Assuming that her poor grades were secondary to lack of effort, her parents withdrew privileges. Nicole became increasingly depressed. She stopped participating in activities, she previously enjoyed, and her parents reported that she stopped singing in the shower. After talking to a cousin with ADHD, Nicole concluded that she had ADHD as well. She asked her parents to arrange for an evaluation.Nicole met DSM-5 criteria for the diagnosis of inattentive ADHD and was started on a stimulant medication (mixed amphetamine salts). She had symptoms of a coexisting depression, although she did not meet criteria for diagnosis of a depressive disorder. At a 3-week follow-up visit, she showed improvement in targeted ADHD symptoms; homework was now easier and her grades improved. At a 2-month follow-up, Nicole's weight dropped from 53 kg (47th percentile) prestimulant treatment to 49 kg (31st percentile). She reported appetite suppression after taking the stimulant but did not feel that her eating habits had changed significantly. Her father reported that she had a preference for junk food and snacks. Nicole did not enjoy exercising and did not participate in extracurricular sports.She weighed herself several times a day, as she was worried about losing too much weight. Nicole's mood continued to be low, despite the fact that her grades improved, and her parents were more understanding of her challenges. She was otherwise healthy and reported regular menstrual cycles. Nicole requested an increase in the dose of stimulant medication for greater improvement in concentration during homework and in school.Her pediatric clinician was concerned about the possibility of an eating disorder in addition to depression. She asked herself, \"Are we treating inattentive ADHD effectively or are we enabling an eating disorder?\""}, {"id": "pubmed23n1048_4259", "title": "Anorexia nervosa manifesting as massive ascites, hypercholesterolemia, and sequential binge eating in an 11-year-old girl: A case report.", "score": 0.009345794392523364, "content": "Anorexia nervosa (AN) is a serious eating disorder associated with a distorted body image. Hypercholesterolemia has been found in patients with AN but the mechanism of hyperlipidemia in AN remains little known. Ascites in patients with AN has been attributed to hypoalbuminemia and liver diseases, but massive ascites without the aforementioned etiologies has never been reported in AN. An 11-year-old girl was admitted for exclusion of organic underlying diseases due to severe body weight loss (18% within 3 weeks), poor appetite, and hypercholesterolemia (274 mg/dL). She complained of heartburn sensation, nausea, vomiting, constipation, and postprandial dull abdominal pain with fullness. The patient's condition met with all 3 of the Diagnostic and Statistical Manual of Mental Disorders (DSM-5) criteria for diagnosing AN. On admission, her total cholesterol level was 337 mg/dL and hypocomplementemia (C3 55.5 mg/dL) was also found. Abdominal sonography and computed tomography scans showed massive ascites. However, neither proteinuria nor hypoalbuminemia was found. Upper gastroduodenal endoscopy showed chronic superficial gastritis and colonoscopy revealed negative findings. Ascites obtained by paracentesis demonstrated a transudate without bacterial infection, tuberculosis, or pancreatitis. Exploratory laparoscopy showed nonpurulent ascites. However, biopsies from the small intestine, mesentery, and liver showed chronic inflammation and fibrosis. The intensive nutritional therapy by increasing total energy intake stepwise with a combination of high-energy formula and her favorite foods. Her hypercholesterolemia, hypocomplementemia, and massive ascites resolved after her weight was restored. She developed binge eating with continuous weight gain after discharge. Her weight significantly increased to an obese level (body mass index [BMI] 25.9 kg/m) after loss to follow-up for 4 years until she returned to our emergency room due to suicide attempt. Diagnostic crossover between subtypes in anorexia nervosa might be a potential risk factor for illness severity and poor prognosis. AN can manifest as massive ascites with normal albumin concentrations that could possibly be due to chronic inflammation of the intestinal serosa, mesentery, and peritoneal surface of the liver."}, {"id": "wiki20220301en024_73321", "title": "List of Family Guy characters", "score": 0.009345794392523364, "content": "Jillian Russell-Wilcox (voiced by Drew Barrymore) – Brian's cute, bulimic, and dimwitted ex-girlfriend who is portrayed as a stereotypical blonde. She is quite ignorant; for example, she does not understand that Adolf Hitler died decades ago. She has a close friendship with Peter, who treats her more like a daughter than he does Meg, due to their similar levels of intelligence. The remainder of the Griffin family look on her as a figure of mockery and symbol of Brian's shallowness, although she has affection for them, involving them all in her wedding. She first appeared in \"Whistle While Your Wife Works\". She is the only girlfriend that Brian has dated for more than one episode, and was a recurring character in season 5. He stays with her purely for sex, though after they split, he felt strong feelings of love for her. In \"Prick Up Your Ears\", when Brian talks about Jillian's bulimia, he goes on to compare her to Karen from The Carpenters, who succumbed to anorexia in 1983, claiming"}, {"id": "wiki20220301en433_12403", "title": "Eating disorders and development", "score": 0.00926825319734499, "content": "Diagnostic criteria Anorexia is characterized by a significant reduction in energy intake which leads to a low body weight given age, sex, development, and physical health considerations. Individuals with anorexia also experience a significant fear of weight gain or engage in behaviors that interfere with weight gain. The illness is also characterized by a disturbance in body image, a significant focus and evaluation of self based on body weight, and/or lack of recognition of the consequences and seriousness of the current low body weight. Intense fear of gaining weight or of becoming fat, or persistent behavior that interferes with weight gain, even though at a significantly low weight. Anorexia has two subtypes: the restricting type and the purging type. This is based on whether an individual engages in or does not engage in bingeing and purging behaviors. Anorexia is characterized as mild, moderate, severe, or extreme based on the extent of weight loss."}, {"id": "pubmed23n0853_1036", "title": "\"Is It Her Hormones?\": Psychiatric Diagnoses and Polycystic Ovarian Syndrome.", "score": 0.009259259259259259, "content": "Beth, whom you have cared for in your primary care practice since she was born, is a 15-year-old adolescent girl with no prior psychiatric history who developed significant symptoms of clinical depression, associated with self-injurious behavior (cutting on wrists, arms, and thighs). She denied any known precipitant for her depression.She is a ninth grade honors student in the gifted program at a local high school and is described as a talented musician, playing multiple musical instruments as well as soccer and basketball. She has good family support, was sociable, and had several close friends. She denied any history of trauma and denied ever using recreational drugs or other mood-altering substances.At this visit, she reported feeling \"sad and anxious.\" Family history was significant for maternal depression, which persisted through her teens and twenties. Her older sister had been diagnosed with Social Anxiety Disorder. Beth reported anhedonia, fatigue and irritable mood, lack of motivation, impaired concentration, and anxiety related to failing grades.You decide to begin medication because of the severity of her symptoms, and 1 week after starting fluoxetine 10 mg, she reportedly overdosed on an unknown quantity of acetaminophen. Within a few days of switching to escitalopram (due to persistent gastrointestinal complaints while taking fluoxetine), she developed homicidal ideation. She reported feeling grandiose, empowered, invincible, elated, and \"crazy,\" although she never demonstrated or endorsed psychotic symptoms. She became fixated upon the idea that she could kill someone and \"get away with it.\" At the time she tried to suffocate a peer with her hands, she was described as having \"a glazed over look in her eyes.\" Moods were now described as alternating between depressed and elated, with mood shifts occurring every few days. These symptoms did not improve after the antidepressant medication was discontinued.Subsequently, patient was admitted for acute psychiatric care, at which time she was described as depressed, but with an \"expansive and irritable\" mood, and with obsessive suicidal ideation. She had developed a plan to hang herself in the home. She started to believe that her mother was trying to give her \"poison.\" She reported panic attacks and said that she wanted to be in the hospital where she could feel \"safe.\" She claimed to have an \"entity inside of her body who was a bully\" and who was \"taking over her body\" and stated that he put her hand over her peer's mouth, as she watched.Psychological testing included the Minnesota Multiphasic Personality Inventory-Adolescent, showed significant paranoia, bizarre mentation, and poor reality testing. Along with interview and observation, it was determined that patient met criteria for the DSM-IV-TR clinical diagnosis of Other Specified Bipolar Disorder, with psychotic features.Aripiprazole was initiated at a dosage of 2 mg; however, moods were still described as fluctuating between extremes every few hours. It was discontinued after reaching 5 mg due to affective blunting. Risperidone 0.5 mg twice daily helped patient to feel and act \"more like herself\"; however, she continued to report significant depression. The addition of lamotrigine 25 mg daily, in addition to individual Dialectical Behavior Therapy, finally led to improvement of mood and a gradual return of her normal baseline, with reportedly stable emotional, social, and academic functioning.The patient's mother remained convinced that this adolescent's mood instability was caused by underlying hormonal problems so you refer her to endocrinology. Beth developed puberty at age 8, with menses occurring on average of twice yearly. She was found to have elevated free testosterone level of 8.6 (reference range, 1.2-7.5). She was of normal weight (body mass index = 21.85 kg/m) and did not manifest acne, male pattern hair thinning, or hirsutism. Thyroid functions, 17-OH progesterone, follicle-stimulating hormone/luteinizing hormone, and estradiol were within normal limits. Prolactin elevation (46.3) was assumed to be due to Risperidone. Patient refused ovarian ultrasound.After starting oral contraceptives to establish monthly menses, patient's emotional and behavioral symptoms continue to remain stable. After Beth decided on her own to discontinue psychotropic medications, she continued for 17 months following her initial visit to remain free of neuropsychiatric symptoms.Now that her symptoms seem resolved; you wonder what the medical diagnosis for Beth was? You wonder if \"hormones\" may have caused or contributed to her psychiatric presentation."}, {"id": "wiki20220301en070_21586", "title": "Imogen Bailey", "score": 0.009259259259259259, "content": "In September 2008 Bailey revealed she had had anorexia nervosa, in an interview with UK magazine, NOW: \"I went through a minor, but still not very good, anorexic stage and actually got down to less than 40 kg\". Her weight loss occurred over about a year from 2000 to 2001 and was influenced by \"being surrounded by other anorexics and the stress of a break-up from an unfaithful boyfriend\". Family and friends intervened and she sought assistance for her condition. On the 3rd of June 2020, KISS FM Host Kyle Sandilands revealed on his show that he has a sexual relationship with Bailey for about 6 months after he was evicted from the Celebrity Big brother house. In 2016 Bailey met and became engaged to Robb Beckmann and they married in January 2018. In May 2020 they had a baby girl Odette after suffering three miscarriages prior. Imogen now works as a birth and end of life doula and runs workshops focussed on empowering women. Filmography References"}, {"id": "pubmed23n1017_5978", "title": "When to Raise Our White Flag-A Discussion of Scope of Practice in a Resource Scarce World.", "score": 0.009174311926605505, "content": "Thomas is a 13-year-old boy with autism spectrum disorder, attention deficit hyperactivity disorder (ADHD), generalized anxiety disorder, separation anxiety disorder, and major depressive disorder who presented for a follow-up to his developmental and behavioral pediatrician (DBP). His mother describes an increase in symptoms of anxiety and depression for the last 6 weeks, accompanied by suicidal ideation and thoughts of self-mutilation.Before this increase in symptoms, he had been doing well for the last several months with the exception of increasing weight gain, and Abilify was decreased from 5 mg to 2.5 mg at his last visit. Other medications at that time included Zoloft 100 mg twice daily, Focalin XR 40 mg every morning, and Focalin 5 mg every night. Without seeking the guidance of our developmental and behavioral pediatrics clinic, his mother increased his intake of Zoloft to 150 mg each morning and continued 100 mg each evening because of worsening anxiety and depression.Religion is very important to Thomas and his family. He acknowledges that he does not want to die and feels badly because \"suicide is against our religion.\"Helping Thomas receive appropriate care has been a challenge. He was diagnosed with ADHD and Asperger disorder at the age of 5. Thomas is homeschooled and is very attached to his mother. His parents have very different parenting styles, with his mother being more permissive and his father more authoritarian. At the time of initial diagnosis, the behavioral health services (BHS) in Thomas' community, which is about an hour away from the DBP, were limited to older children, and he was followed by a DBP for ADHD medication management. At the age of 11, he expressed passive suicidal ideation and described that he imagined his mother as \"the devil with fire coming out of her eyes\" when she corrected him. He was evaluated by BHS, diagnosed with anxiety disorder, and started on Lexapro. BHS linked to the DBP were out of network for his insurance. The family was unable to pay out of pocket, so care was subsequently transferred to a DBP clinic that was in network. Soon after, Thomas developed auditory hallucinations, and Abilify was added after consultation with BHS.Over the last few years, Thomas' symptoms have waxed and waned. He did well for a short time and then again developed auditory hallucinations, worsening symptoms of anxiety and depression, and increasing somatic symptoms including vomiting and penile pain. Medications were adjusted with input from BHS, and further attempts were made to link him to local BHS but were unsuccessful. With his current concerns of suicidal ideation and self-mutilation, what would be your next steps?"}, {"id": "wiki20220301en159_7366", "title": "Ellen West", "score": 0.009174311926605505, "content": "Ellen West's life was marred by thoughts related to death anxiety. Towards the end of her life, it could be said that she had a death obsession. West was given a great variety of diagnoses including melancholia, severe obsessive neurosis, and schizophrenia. While her major problem dealt with food, as what started out as a diagnosis of anorexia nervosa morphed into bulimia nervosa with the fear of becoming fat through eating. This fixation caused her great depression, as her focus day in and out was on eating or not eating. This \"obsession with death\" became \"life's only goal\" and that the \"symbolization of life and death took place around the act of eating.\" West's fear of becoming fat caused her to welcome death as an acceptable outcome, as then she wouldn't have to worry anymore. She was often quoted by her psychiatrist, Ludwig Binswanger, explaining how her life felt like a prison that could be only made better by dying. To West, her life felt empty and dull, and filling her body"}, {"id": "pubmed23n1115_8295", "title": "Anorexia nervosa, conduct disorder, and the juvenile justice system: a case of applying traditional treatment modalities in a non-traditional setting.", "score": 0.00909090909090909, "content": "Anorexia Nervosa is highly comorbid with depressive, anxiety, and obsessive-compulsive spectrum disorders. However, it has not previously been reported as comorbid with antisocial personality traits, except when substance use disorder is also identified. We present an unusual case of a patient with resistant anorexia nervosa and comorbid conduct disorder. This case was also unique in that the juvenile justice system was involved during treatment. A 13-year-old female was admitted to our pediatric hospital for the treatment of anorexia nervosa. She had a history of violent behaviors toward family members, often jeopardizing her care. During hospitalization, she physically attacked a physician on her care team shortly before she transitioned to an eating disorders treatment program. She was diagnosed with conduct disorder, and following discharge, she attacked her father in a premeditated act. This led to her entry into the juvenile justice system. While under the custody of the juvenile justice system, she was readmitted to our hospital for further treatment of anorexia nervosa. Our treatment strategy included psychotropics, positive reinforcement, close interdisciplinary coordination among the various hospital teams, and the juvenile justice system. Following discharge from her second hospitalization back to the juvenile detention system, our patient maintained a healthy weight and appeared to show improvements in the cognitive distortions related to her eating disorder. To our knowledge, this is the first reported successful treatment of an individual with resistant anorexia nervosa and conduct disorder. It was likely a combination of weight gain, psychotropic medications, and the structured milieu provided by the juvenile justice system that led to the effective treatment of our patient. This case illustrates that a non-traditional healthcare setting can be an asset to treatment through persistence and close collaboration across institutions."}, {"id": "wiki20220301en165_3853", "title": "Avoidant/restrictive food intake disorder", "score": 0.009009009009009009, "content": "Anxiety disorder Specific food avoidances could be caused by food phobias that cause great anxiety when a person is presented with new or feared foods. Most eating disorders are related to a fear of gaining weight. Those who have ARFID do not have this fear, but the psychological symptoms and anxiety created are similar. Some people with ARFID have fears such as emetophobia (fear of vomiting) or a fear of choking. Anorexia nervosa Though the physical symptoms may be similar, anorexia nervosa differs from ARFID because in ARFID the lack of food intake is not related to body image or weight concerns. Additionally, in a study analyzing the similarities between patients with AN and patients with ARFID, those with ARFID were significantly younger (10.8 vs 14.1 yrs old) with an earlier onset of illness (6.2 vs 13.7 yrs old) and a longer evolution time (61.2 vs 8.4 months). Also, a greater proportion of the ARFID patients were male rather than female (60.6% vs 6.1%)."}, {"id": "wiki20220301en174_10490", "title": "Wannarexia", "score": 0.009009009009009009, "content": "Many people who actually suffer from the eating disorder anorexia are angry, offended, or frustrated about wannarexia. Eating disorders are about using food to cope with life distress and poor body image, and they have very complex underlying causes. People with eating disorders might use weight as a measure of self-worth, and they often derive pleasure from losing weight while it doesn't feel enough. The notion of \"wannarexic\" is a very dangerous invalidation of eating disorders that are not specified based on the BMI criteria of the DSM. It can feed the eating disorder mindset of not sick enough and lead people to get too sick to validate their struggle with an eating disorder however even the most severely ill people suffering from eating disorders still struggle to feel \"sick enough\" ."}, {"id": "pubmed23n1004_9323", "title": "Sounds unrealistic: an adolescent girl with anorexia nervosa consumes 19 L of fluid in a few hours: what happens to the physiology?", "score": 0.008928571428571428, "content": "Adolescents with eating disorders (EDs) may present not only with abnormal eating behaviors but also with abnormal drinking behaviors varying widely. These behaviors include water loading to cheat on weight measurements, to feel full and suppress appetite and/or to induce vomiting; as well as restricting fluid intake in addition to food. We present a 16-year-old female adolescent with anorexia nervosa restrictive type and major depressive disorder who was hospitalized due to acute food refusal and developed generalized seizures due to dilutional hyponatremia in consequence of consuming excessive amount of water. Psychiatric diagnoses were made according to 'The Diagnostic and Statistical Manual of Mental Disorders' (5th ed.; DSM-5) criteria. After starting nutritional rehabilitation with a low calorie meal plan to avoid refeeding syndrome, a weight gain of 2 kg was noted in the second day of hospitalization. At the bedside visit, she was observed in a disoriented manner and consecutively in seconds, lost consciousness with a generalized tonic-clonic seizure lasting 2 min. Her serum sodium level was measured as 116 mEq/L, which was normal at the time of admission. It was later learned that she secretly ingested 19 L of water in a short amount of time. She regained consciousness and no further seizures were observed after intravenous sodium deficit correction and fluid restriction therapy. Her serum sodium level was normalized (137 mEq/L) within 12 h. A thorough clinical assessment of hydration and drinking behaviors as well as eating behaviors is essential for patients with EDs to avoid serious medical complications with high mortality and morbidity during follow-up. It is interesting that this amount of fluid consumption in such a short period of time did not present to the clinic with vomiting, gastric dilatation or bowel irrigation symptoms in a case with acute food refusal and restriction for a year, instead absorbed very quickly causing acute and severe symptomatic hyponatremia with generalized seizures."}, {"id": "wiki20220301en340_2320", "title": "Wintergirls", "score": 0.008928571428571428, "content": "Wintergirls (2009) is a realistic fiction novel by the American author Laurie Halse Anderson. The novel was published in 2009 by Viking. The story focuses on a girl, Lia Overbrook, who suffers from anorexia and self harm. Lia struggles to cope with her mental illness while balancing everything else going on in her life. Some months after a fall out with her best friend Cassie, Lia receives the news that she has died from bulimia. This complicates Lia's life even more and forces her to confront her own illness. Plot Lia Overbrook, an 18-year-old girl, just found out that her ex-best friend Cassie has died. Cassie had called Lia 33 times the night of her death. However, Lia never answered the phone. Cassie was found in a hotel room, killed by her illness, bulimia. Lia, who has a history of anorexia, falls into a downward spiral of self-harm and calorie counting. To hide her illness from her family, Lia's obsessive and destructive behavior worsens and recovery seems impossible."}, {"id": "pubmed23n1145_15445", "title": "Facing an eating disorder: A case of body dysmorphic disorder and avoidant/restrictive food intake disorder.", "score": 0.008849557522123894, "content": "Eating disorders can be notoriously difficult to diagnose and treat. This patient is an 18-year-old female who presents to care severely underweight and notably cachexic. For a number of years, she had experienced depressive symptoms, anxiety, and continued loss of appetite. She denied purposefully restricting foods, recognized that she was thin, and denied a fear of gaining weight. She was admitted to a disordered eating unit for refeeding and during her inpatient stay disclosed that she had a long-standing \"hatred of face.\" Ultimately, she received the diagnoses of avoidant/restrictive food intake disorder and body dysmorphic disorder. This case highlights the importance of differentiating body dysmorphia, seen in body dysmorphic disorder, and distorted body image, as seen in anorexia nervosa. This differentiation is significant as the treatment approaches to these distinct diagnoses are not the same."}, {"id": "wiki20220301en378_30061", "title": "A Counterfeit Presentment", "score": 0.008849557522123894, "content": "Anorexia Constance is referred to as a “ghost” or an “apparition” multiple times throughout the course of the play. Howells has most likely employed this characterization to indicate that Constance is anorexic. Anorexia is a disease that Howells held very dear to his heart. In 1889, his beloved daughter, Winifred, succumbed to the disease when she was only 26 years old and weighed a mere 79 pounds. Although this unfortunate event happened over a decade after the publication of this play, it could be speculated that Winnie was already showing symptoms, since anorexia was becoming increasingly prevalent amongst young women her age."}, {"id": "pubmed23n0581_15654", "title": "Anorexia, Maudsley and an impressive recovery: one family's story.", "score": 0.008791208791208791, "content": "Ann was first diagnosed with anorexia nervosa at the age of 14 years. For two years she maintained unshakeable delusions that her body was grossly obese and that she did not require food to live. Her rational thought processes were swamped by overwhelming emotions of fear, self-loathing, rage and despair. She developed obsessive and compulsive behaviours, her reactions to food were phobic, she withdrew from social contact, was flooded by suicidal thoughts and had periods of self-harming. For 16 months after diagnosis Ann was seen by a variety of medical professionals who prescribed a range of treatments, some of which were life saving but none of which, crucially, had any appreciable impact on the long-term course of her illness. She then began treatment following the family-based Maudsley Approach which encourages parents to develop and maintain a programme of weight gain for their child. Six months after starting Maudsley Ann achieved a healthy body weight and all of her psychiatric symptoms began to disappear spontaneously. Over the next five months Ann's physical and mental health continued to improve dramatically. She has made a full recovery and has shown no sign of relapse for over 12 months."}, {"id": "pubmed23n0858_15352", "title": "[Anorexia with sinus bradycardia: a case report].", "score": 0.008771929824561403, "content": "As anorexia patients always go to the psychiatric clinic, little is concerned about the occurrence of sinus bradycardia in these patients for cardiologists and psychiatrists. The aim of this paper is to discuss the relationship between anorexia and sinus bradycardia, and the feature analysis, differential diagnosis and therapeutic principles of this type of sinus bradycardia. We report a case of sinus bradycardia in an anorexia patient with the clinical manifestations, laboratory exams, auxiliary exams, therapeutic methods, and her prognosis, who was admitted to Peking University Third Hospital recently. The patient was a 19-year-old female, who had the manifestation of anorexia. She lost obvious weight in a short time (about 15 kg in 6 months), and her body mass index was 14.8 kg/m(2). The patient felt apparent palpitation, chest depression and short breath, without dizziness, amaurosis or unconsciousness. Vitals on presentation were notable for hypotension, and bradycardia. The initial exam was significant for emaciation, but without lethargy or lower extremity edema. The electrocardiogram showed sinus bradycardia with her heart rate being 32 beats per minute. The laboratory work -up revealed her normal blood routine, electrolytes and liver function. But in her thyroid function test, the free thyroid (FT) hormones 3 was 0.91 ng/L (2.3-4.2 ng/L),and FT4 was 8.2 ng/L (8.9-18.0 ng/L), which were all lower; yet the thyroid stimulating hormone (TSH) was normal 1.48 IU/mL (0.55-4.78 IU/mL). Ultrasound revealed her normal thyroid. Anorexia is an eating disorder characterized by extremely low body weight, fear of gaining weight or distorted perception of body image, and amenorrhea. Anorexia patients who lose weight apparently in short time enhance the excitability of the parasympathetic nerve, and inhibit the sympathetic nerve which lead to the appearance of sinus bradycardia, and functional abnormalities of multiple systems such as hypothyroidism. But this kind of sinus bradycardia and hypothyroidism have good prognosis. And asymptomatic sinus bradycardia with reversible causes, because of the great prognosis, they do not need special treatment. Multiple medical and psychiatric disciplines were consulted, and then, family care, nutritional support and psychiatric therapy were given, and she did not need thyroid hormone replacement therapy. The patient's overall clinical status improved gradually during her hospital stay and her heart rate was recovered to 55 beats per minute."}, {"id": "Psichiatry_DSM-5_3414", "title": "Psichiatry_DSM-5", "score": 0.008771929824561403, "content": "V62.82 (263.4) Uncomplicated Bereavement This category can be used when the focus of clinical attention is a normal reaction to the death of a loved one. As part of their reaction to such a loss, some grieving individuals present with symptoms characteristic of a major depressive episodchfor example, feel- Other Conditions That May Be a Focus of Clinical Attention 717 ings of sadness and associated symptoms such as insonmia, poor appetite, and weight loss. The bereaxed individual typically regards the depressed mood as ”normal,” al- though the individual may seek professional help for relief of associated symptoms such as insomnia or anorexia. The duration and expression of \"normal” bereavement vary con- siderably among different cultural groups. Further guidance in distinguishing grief from a major depressive episode is provided in the criteria for major depressive episode."}, {"id": "pubmed23n0534_20425", "title": "[Mental disorders associated with multiple sclerosis].", "score": 0.008695652173913044, "content": "Findings are reported in 4 patients with multiple sclerosis (MS) and various mental disorders which were directly associated with the underlying disease, with disease-related increased vulnerability, were a reactive response or had occurred independently of the MS. CASE 1: A 53-year-old woman with MS for 24 years developed cognitive disorders with markedly impaired quality of life. CASE 2: A 44-year-old man with MS for 8 years had depression and felt suicidal. CASE 3: A 58-year-old woman with MS for 35 years suffered from both depression and symptoms of a compulsive-obsessive disorder. CASE 4: A 19-year-old woman with MS for 2 years was treated by a psychiatrist for the first time when she developed acoustic hallucinations and suicidal ideation during cortisone treatment. CASE 1: The progressive mental disorder was classified as manifestation of the chronic progressive MS: it was treatment-refractory. CASE 2: The patient underwent psychotherapy as both an in- and out-patient. His depression was thought to be reactive and improved well under this treatment, despite the deterioration of the MS, requiring him to use a wheel-chair. CASE 3: The compulsive-obsessive symptoms persisted during medication for depression, for which she then received behavioral treatment. CASE 4: The initial symptoms of hallucination and suicidal ideation ceased when cortisone was stopped. A diagnosis of borderline personality was made, based on her continuing symptoms of impaired impulse control, abnormal affect, distorted self-image, a feeling of inner emptiness, agitation with suicidal tendency and factitious injuries. The renewed occurrence of acoustic hallucinations and suicidal tendency occurred independently of cortisone treatment and was interpreted as expression of inflammatory disease activity, but differential diagnosis had to consider the patient s personality disorder, too. Mental disorders in patients with MS are manifold and their causes are complex. It is important to recognize these associated diseases and initiate individualized treatment, because they considerably impair the patients quality of life."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 241]], "word_ranges": [[0, 37]], "text": "We describe the case of a patient with a hyperkalemic emergency (potassium >6.5 mmol/l with ECG alterations). The first step is the administration of iv calcium gluconate to counteract the cardiac toxicity of hyperkalemia (option 3 correct),"}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "We describe the case of a patient with a hyperkalemic emergency (potassium >6.5 mmol/l with ECG alterations). The first step is the administration of iv calcium gluconate to counteract the cardiac toxicity of hyperkalemia (option 3 correct), giving us more time to initiate hypokalemic treatments (initially saline glucose with insulin, salbutamol, furosemide, and assessing dialysis if there is no good response).", "full_answer_no_ref": "We describe the case of a patient with a hyperkalemic emergency (potassium >6.5 mmol/l with ECG alterations). The first step is the administration of iv calcium gluconate to counteract the cardiac toxicity of hyperkalemia ([HIDDEN]), giving us more time to initiate hypokalemic treatments (initially saline glucose with insulin, salbutamol, furosemide, and assessing dialysis if there is no good response).", "full_question": "In a patient with known advanced chronic kidney disease (CKD G4, eGFR 20 ml/min) who comes to the emergency department for general weakness and is found to have severe hyperkalemia (K 7 mEq/l) with electrocardiographic abnormalities. What would be the first measure to take?", "id": 530, "lang": "en", "options": {"1": "Administration of cation exchange resins.", "2": "Placement of a catheter to initiate dialysis.", "3": "Administration of intravenous calcium gluconate.", "4": "Administration of oral furosemide.", "5": NaN}, "question_id_specific": 167, "type": "NEPHROLOGY", "year": 2021, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1010_562", "title": "A case of advanced chronic kidney disease with severe hypocalcemia, how to safely manage and dialyze?", "score": 0.01761025720244072, "content": "Patients often present with advanced chronic kidney disease (CKD) complicated with severe hypocalcemia that may be accompanied by electrocardiographic changes. The management of this kind of patients may require hemodialysis (HD). However, initiation of renal replacement therapy in this scenario needs special attention to avoid complications such as cardiac arrhythmias. A 22-year-old male presented to our emergency department with severe renal failure, hypocalcemia, hyperphosphatemia, severe acidosis, and QT prolongation on electrocardiography. The patient was kept in the emergency department under cardiac monitoring. He was started on IV calcium gluconate 1 g every 6 h aiming to increase his adjusted calcium level to 1.8 mmol/L. He subsequently received the first HD session with low blood flow, increased calcium, and decreased bicarbonate dialysate bath. There were no arrhythmias or hemodynamic instability. Intravenous calcium was discontinued; adjusted calcium improved progressively after dialysis and reached 1.9 mmol/L by the time of discharge and after receiving three sessions of HD. This case describes a not so infrequent presentation of advanced renal impairment with profound hypocalcemia, hyperphosphatemia in the setting of CKD-associated mineral bone disorder. Intravenous calcium administration may promote vascular and metastatic calcification, particularly with the coexistence of hyperphosphatemia, and hence, it is best avoided. There are no guidelines to direct initiating HD in this context. However, it appears that using a high calcium bath is prudent to minimize cardiovascular complications, particularly if there is the prolongation of the corrected QT interval on electrocardiography."}, {"id": "pubmed23n1130_14872", "title": "Electrocardiography is Unreliable to Detect Potential Lethal Hyperkalemia in Patients with Non-dialysis Chronic Kidney Disease.", "score": 0.017545209696036936, "content": "Hemodialysis patients with hypercalcemia are less likely to manifest the usual electrocardiographic changes associated with hyperkalemia than in those with normal renal function. This study was conducted to determine whether electrocardiography (ECG) is a reliable indicator to detect severe life-threatening hyperkalemia in non-dialysis CKD patients. The study was conducted at three referral university hospitals between July 2017 and June 2018. Severe hyperkalemia was defined as serum potassium concentration ≥ 8.0 mEq/L. Serum potassium, sodium, bicarbonate, calcium, and creatinine concentrations were measured and simultaneous 12-lead ECG was obtained. Patients with end-stage renal disease receiving renal replacement therapy were excluded. Also excluded were patients with the usual ECG abnormalities to hyperkalemia. Of the 438 patients screened, 10 (2.3%) aged 2-14 years with severe hyperkalemia and normal ECG findings were identified. Median serum potassium level was 8.6 mEq/L (range 8.2-9.0). All had regular sinus rhythm. P, QRS, ST segment, T morphology, PR and QT interval, and QRS duration were all normal. Hyperkalemia was associated with CKD, metabolic acidosis, and hypercalcemia in all cases. Therapy with intravenous 0.9% saline, sodium bicarbonate, glucose, insulin, calcium, and salbutamol corrected the hyperkalemia in 7 patients. The remaining three patients evinced arrhythmias requiring hemodialysis. Although rare, non-dialysis CKD patients with hypercalcemia may not manifest the usual electrographic abnormalities associated with hyperkalemia. Thus, a normal ECG finding in non-dialysis CKD patients should be interpreted with caution."}, {"id": "pubmed23n0799_16990", "title": "Serum potassium in stage 5 CKD patients on their first presentation in a dialysis service of a county hospital in western Romania.", "score": 0.01731893837156995, "content": "CKD patients present deficient elimination of potassium. Ambulatory treatment with hypotensors, mainly angiotensin-renin system inhibitors, can be associated in these patients with potassium retention and risk of hyperkalemia. In pre-dialysis stage-5 CKD patients, the use of medication accompanied by hyperkalemia increases risks of developing it. Using diuretics like spironolactone also increases this risk. Serum potassium can also increase in case of inappropriate consumption of potassium-rich food (bananas). Since ambulatory care does not always rigorously control hyperkalemia in CKD patients we consider it is useful to screen patients when they are referred to dialysis services. The screening can reflect the management of ambulatory CKD patients under treatment with ACE-I and ARB hypotensors. We remark that beta-blockers are attributed a (limited) role in increasing the values of serum K. We studied a group of 477 stage-5 CKD patients referred for dialysis to The Dialysis Centre of the Emergency County Hospital Timişoara. The average age of the patients was 57.41 +/- 14.26 years. 260 were males and 217 females. All were stage-5 CKD with GFR &lt; 15 mL/min/1.73 m2, with a group average value of eGFR of 5.72 +/- 2.81 mL/min/1.73m2. Our investigations showed hypokalemia in 14 patients (2.93%). Hyperkalemia was found in 179 patients. Of these, 124 had mild hyperkalemia (5.5-6.4 mEq/L), 45 patients had medium hyperkalemia (6.5-7.5 mEq/L) and 10 (2.09%) had severe hyperkalemia (K &gt; 7.5 mEq/L). Hyperkalemia was more frequent in patients who had been treated with blockers of the renin-angiotensin system than in patients who had used other hypotensors or who had not needed hypotensors and had not taken diuretics. Severe hyperkalemia (K &gt; 7.5 mEq/L) was present both in patients treated with blockers of the renin-angiotensin system and in those treated with other hypotensors and in 1 case without hypotensor or diuretic treatment. 2 cases treated with blockers of the renin-angiotensin-aldosterone system with severe hyperpotassemia associated antialdosteronic diuretics, cumulating hyperpotassemic effects. Diuretic treatments with loop diuretics influenced the values of serum K of most patients. Hyperkalemia represents an important problem in nephrology because of the risks it induces in the treatment ofpre-dialysis CKD patients and it requires attentive monitoring."}, {"id": "pubmed23n1058_5511", "title": "Severe Persistent Hyperkalemia with Electrocardiogram Changes in a Patient with Hyperaldosteronism.", "score": 0.01710319164162015, "content": "A 62-year-old female presented to the emergency department (ED) with fatigue and generalized body weakness for the last three days. Upon arrival, initial ECG showed wide complex tachycardia with sine waves and a heart rate (HR) ranging between 100-170 bpm. She was otherwise vitally stable. The patient had a past medical history of hyperaldosteronism, type 2 diabetes mellitus (DM), chronic kidney disease (CKD) with microalbuminuria, and hypertension. She also had a history of cerebrovascular accident (CVA) and residual left-sided weakness more pronounced in the upper limb. Initial venous blood gas (VBG) analysis showed a potassium level of more than 10 mmol/L, chloride 114 mmol/L, bicarbonate 9 mmol/L, sodium 135 mmol/L, and pH of 7.1. Treatment for hyperkalemia was started immediately with calcium gluconate 1 gm that effectively narrowed her QRS complex and normalized her ECG. Salbutamol nebulization, glucose/insulin infusion, and calcium polystyrene syrup were given. Later, she was started on 100 mg sodium bicarbonate infusion, and Foley's catheter was inserted to follow urine output (UOP) strictly. However, she did not show a decrease in serum potassium levels. Then the patient underwent hemodialysis for two hours. Her first potassium reading after hemodialysis was 5.2 mmol/L. The purpose of this case report is to emphasize the importance of hemodialysis in patients with persistent severe life-threatening hyperkalemia."}, {"id": "pubmed23n0407_384", "title": "Therapeutic approach to hyperkalemia.", "score": 0.01672194582642344, "content": "The foremost step in the initial clinical management of hyperkalemia is to decide whether a hyperkalemic patient requires immediate treatment to avoid a life-threatening situation (serum potassium concentration &gt;6.0 mEq/l and EKG changes). When the decision for urgent treatment of hyperkalemia is based on EKG changes, an important caveat for clinicians is that absent or atypical EKG changes do not exclude the necessity for immediate intervention. Once an urgent situation has being handled with intravenous push of a 10% calcium salt, the initiation of short-term measures can be launched by either a single or combined regimen of the three agents that cause a transcellular shift of potassium - insulin with glucose, beta(2)-agonist (albuterol), and NaHCO(3). As the first choice among these available options, we favor an intravenous bolus of 10 units of insulin with 50 ml of 50% glucose alone or in combination with 10-20 mg of albuterol by nebulizer. These can be repeated as required until the institution of hemodialysis. The combination of insulin with glucose and NaHCO(3) as an another option needs further clarification for its additive effects. However, NaHCO(3) has lost its favor because of its poor efficacy as a potassium-lowering agent when used alone. The next step is to remove potassium from the body - diuretics (furosemide), cation exchange resin (kayexelate) with sorbitol, and dialysis (preferably hemodialysis). The final important step for the managements of hyperkalemia is a long-term plan to prevent its recurrence or worsening. In addition to every effort to elucidate underlying causes and pathophysiologic mechanisms for hyperkalemia, an extensive search must be made to uncover overt or sometimes covert medications that may have led to the development of hyperkalemia. Furthermore, one must obtain detailed dietary and medical history of hyperkalemic patients."}, {"id": "pubmed23n0832_1297", "title": "[The most frequent electrolyte disorders in the emergency department : what must be done immediately?].", "score": 0.014479166666666668, "content": "Hyponatremia is the most common form of electrolyte disorder in the emergency room. The symptoms are unspecific and include nausea, dizziness and often falls. Typical symptoms of severe hypernatremia are vomiting, cerebral seizures, somnolence and even coma. The specific initial laboratory diagnostics include measurement of serum electrolytes, serum glucose, serum and urine osmolarity and sodium in urine. The main aim of the clinical examination is to estimate the volume status. If a patient has hypovolemia an infusion of isotonic sodium chloride solution (0.9 %) is the method of choice. If the patient is euvolemic the syndrome of inappropriate antidiuretic hormone secretion (SIADH) or (neurotropic) drugs might be the cause. In these cases the primary measure is restriction of fluid intake. As a rapid correction of sodium levels can lead to pontine myelinolysis, the increase in sodium concentration must not be less than 10 mmol/l within the first 24 h and 18 mmol/l within the first 48 h. Clinical symptoms of hyperkalemia include neurological (e.g. muscle weakness, paresis, hyperreflexia, cramps and dysesthesia), gastrointestinal (e.g. nausea, vomiting and diarrhea) and cardiac symptoms (e.g. dysrhythmia and conductance disorders). Calcium injection stabilizes cardiac rhythm disorders immediately. For a rapid drop in potassium by shifting the potassium to the intracellular space, administration of glucose with insulin and high-dose inhalative administration of betamimetics can be used. Potassium elimination is achieved by infusion of isotonic sodium choride (0.9 %) with i.v. administration of furosemide, ion exchange resins and hemodialysis."}, {"id": "article-31674_13", "title": "EMS Confined Space Care -- Issues of Concern -- Crush Injury and Crush Syndrome", "score": 0.014120399381710322, "content": "Several electrolyte abnormalities may be present. Hypocalcemia should only be treated if the patient is symptomatic since the patient may later develop hypercalcemia. Start with 10 mL of intravenous calcium gluconate 10% or 5 mL of intravenous calcium chloride 10% over 2 minutes.  Hyperkalemia should be monitored and treated based on the potassium level and electrocardiographic changes.   The ECG may not change in some situations where the potassium level is dangerously elevated.  If there are severe or life-threatening electrocardiographic changes or arrhythmias such as the widening of the QRS to third-degree heart block or pulseless electrical activity, the intravenous calcium gluconate or chloride should be given immediately. It may be necessary to give several doses every 10 minutes to achieve the cardioprotective effects of calcium. If the patient is acidotic then sodium bicarbonate 1 meq/kg should be given immediately IV over several minutes.  To drive the potassium into the cells, 10 units of intravenous insulin, together with 1 to 2 ampules of D50, should be given.   The Beta agonist effect of albuterol inhalation treatments can also help transiently decrease potassium levels.  Kaexylate with sorbitol can be given orally at 25 to 50 gm, or it may be given as a retention enema.  Patients with life-threatening hyperkalemia need the potassium removed from their system.  The medications and treatments are temporizing measures to provide time for excretion of removal of potassium from the body.  The patient may require emergent dialysis.  Critiques of recent disaster responses were that there was an insufficient number of dialysis machines to treat those with renal failure secondary to crush injuries.  Blood products should be given as necessary and when indicated."}, {"id": "wiki20220301en292_25370", "title": "Hs and Ts", "score": 0.013963210702341137, "content": "A common presentation of hyperkalemia is in the patient with end-stage renal disease who has missed a dialysis appointment and presents with weakness, nausea, and broad QRS complexes on the electrocardiogram. (Note however that patients with chronic kidney disease are often more tolerant of high potassium levels as their body often adapts to it.) Several medications, for example the antibiotic trimethoprim/sulfamethoxazole or an ACE inhibitor, can also lead to the development of significant hyperkalemia. The electrocardiogram will show tall, peaked T waves (often larger than the R wave) or can degenerate into a sine wave as the QRS complex widens. Immediate initial therapy is the administration of calcium, either as calcium gluconate or calcium chloride. This stabilizes the electrochemical potential of cardiac myocytes, thereby preventing the development of fatal arrhythmias. This is, however, only a temporizing measure. Other temporizing measures may include nebulized salbutamol,"}, {"id": "wiki20220301en057_29614", "title": "Crush syndrome", "score": 0.013852813852813853, "content": "Intravenous hydration of up to 1.5 L/h should continue to prevent hypotension. A urinary output of at least 300 mL/h should be maintained with IV fluids and mannitol, and hemodialysis considered if an increase in urine is not achieved. Use intravenous sodium bicarbonate to keep the urine pH at 6.5 or greater, to prevent myoglobin and uric acid deposition in kidneys. To prevent hyperkalemia/hypocalcemia, consider the following adult doses: calcium gluconate 10% 10 mL or calcium chloride 10% 5 mL IV over 2 minutes sodium bicarbonate 1 meq/kg IV slow push regular insulin 5–10 U 50% glucose 1–2 ampules IV bolus kayexalate 25–50 g with sorbitol 20% 100 mL by mouth or rectum. Even so, abnormal heart rhythms may develop; electrocardiographic monitoring is advised, and specific treatment begun promptly. References External links Life or Limb: What happens when your leg gets trapped under a building? Injuries Early complications of trauma Nephrology Syndromes"}, {"id": "wiki20220301en026_74328", "title": "Hyperkalemia", "score": 0.01312043093497499, "content": "Initial treatment in those with ECG changes is salts, such as calcium gluconate or calcium chloride. Other medications used to rapidly reduce blood potassium levels include insulin with dextrose, salbutamol, and sodium bicarbonate. Medications that might worsen the condition should be stopped and a low potassium diet should be started. Measures to remove potassium from the body include diuretics such as furosemide, potassium-binders such as polystyrene sulfonate and sodium zirconium cyclosilicate, and hemodialysis. Hemodialysis is the most effective method. Hyperkalemia is rare among those who are otherwise healthy. Among those who are hospitalized, rates are between 1% and 2.5%. It is associated with an increased mortality, whether due to hyperkalaemia itself or as a marker of severe illness, especially in those without chronic kidney disease. The word hyperkalemia comes from hyper- 'high' + kalium 'potassium' + -emia 'blood condition'."}, {"id": "pubmed23n0580_16426", "title": "Management of hyperkalemia in dialysis patients.", "score": 0.012929596262929596, "content": "Hyperkalemia is common in patients with end-stage renal disease, and may result in serious electrocardiographic abnormalities. Dialysis is the definitive treatment of hyperkalemia in these patients. Intravenous calcium is used to stabilize the myocardium. Intravenous insulin and nebulized albuterol lower serum potassium acutely, by shifting it into the cells. Despite their widespread use, neither intravenous bicarbonate nor cation exchange resins are effective in lowering serum potassium acutely. Prevention of hyperkalemia currently rests largely upon dietary compliance and avoidance of medications that may promote hyperkalemia. Prolonged fasting may provoke hyperkalemia, which can be prevented by administration of intravenous dextrose."}, {"id": "InternalMed_Harrison_3592", "title": "InternalMed_Harrison", "score": 0.012517006802721088, "content": "Intravenous bicarbonate has no role in the acute treatment of hyperkalemia, but may slowly attenuate hyperkalemia with sustained administration over several hours. It should not be given repeatedly as a hypertonic intravenous bolus of undiluted ampules, given the risk of associated hypernatremia, but should instead be infused in an isotonic or hypotonic fluid (e.g., 150 mEqu in 1 L of D5W). In patients with metabolic acidosis, a delayed drop in plasma K+ concentration can be seen after 4–6 h of isotonic bicarbonate infusion. 3. Removal of potassium. This is typically accomplished using cation exchange resins, diuretics, and/or dialysis. The cation exchange resin sodium polystyrene sulfonate (SPS) exchanges Na+ for K+ in the gastrointestinal tract and increases the fecal excretion of K+; alternative calcium-based resins, when available, may"}, {"id": "pubmed23n1095_19114", "title": "Patiromer in a Patient with Severe Hyperkalemia on Incremental Hemodialysis with 1 Session per Week: A Case Report and Literature Review.", "score": 0.011329113924050633, "content": "Hyperkalemia is common in patients with ESRD, undergoing hemodialysis (HD), and is associated with an increase in hospitalization and mortality. Residual kidney function in long-term dialysis patients is associated with lower morbidity and mortality in HD patients. Although the 2015 National Kidney Foundation-Kidney Disease Outcomes Quality Initiate (NKD-KDOQI) guidelines allow the reduction in the weekly HD dose for patients with a residual kidney urea clearance (Kur) &gt;3 mL/min/1.73 m<sup2</sup, very few centers adjust the dialysis dose based on these criteria. In our center, the pattern of incremental hemodialysis (iHD) with once-a-week schedule (1 HD/W) has been an option for a group of patients showing very good results. This pattern is maintained as long as residual diuresis is &gt;1,000 mL/24 h, Kur is &gt;4 mL/min, and there is no presence of edema or volume overload, as well as no analytical parameters persistently outside the advisable range (serum phosphorus &gt;6 mg/dL or potassium [K<sup+</sup] &gt;6.5 mmol/L). Management of hyperkalemia in HD patients includes reduction of dietary intake, dosing of medications that contribute to hyperkalemia, and use of cation-exchange resins such as calcium or sodium polystyrene sulfonate. Two newer potassium binders, patiromer sorbitex calcium and sodium zirconium cyclosilicate, have been safely used for potassium imbalance treatment in patients with ESRD in HD with a conventional regimen of thrice weekly, but has not yet been studied in 1 HD/W schedules. We present the case of a 76-year-old woman in iHD (1 HD/W) treated with patiromer for severe HK and describe her clinical characteristics and outcomes. In addition, we review the corresponding literature. Based on these data, it can be anticipated that the use of patiromer may overcome the risk of hyperkalemia in patients with incident ESRD treated with less-frequent HD regimens."}, {"id": "pubmed23n0615_21447", "title": "[Electrolyte and acid-base balance disorders in advanced chronic kidney disease].", "score": 0.009900990099009901, "content": "1. The kidneys are the key organs to maintain the balance of the different electrolytes in the body and the acid-base balance. Progressive loss of kidney function results in a number of adaptive and compensatory renal and extrarenal changes that allow homeostasis to be maintained with glomerular filtration rates in the range of 10-25 ml/min. With glomerular filtration rates below 10 ml/min, there are almost always abnormalites in the body's internal environment with clinical repercussions. 2. Water Balance Disorders: In advanced chronic kidney disease (CKD), the range of urine osmolality progressively approaches plasma osmolality and becomes isostenuric. This manifests clinically as symptoms of nocturia and polyuria, especially in tubulointerstitial kidney diseases. Water overload will result in hyponatremia and a decrease in water intake will lead to hypernatremia. Routine analyses of serum Na levels should be performed in all patients with advanced CKD (Strength of Recommendation C). Except in edematous states, a daily fluid intake of 1.5-2 liters should be recommended (Strength of Recommendation C). Hyponatremia does not usually occur with glomerular filtration rates above 10 ml/min (Strength of Recommendation B). If it occurs, an excessive intake of free water should be considered or nonosmotic release of vasopressin by stimuli such as pain, anesthetics, hypoxemia or hypovolemia, or the use of diuretics. Hypernatremia is less frequent than hyponatremia in CKD. It can occur because of the provision of hypertonic parenteral solutions, or more frequently as a consequence of osmotic diuresis due to inadequate water intake during intercurrent disease, or in some circumstance that limits access to water (obtundation, immobility). 3. Sodium Balance Disorders: In CKD, fractional excretion of sodium increases so that absolute sodium excretion is not modified until glomerular filtration rates below 15 ml/min (Strength of Recommendation B). Total body content of sodium is the main determinant of extracellular volume and therefore disturbances in sodium balance will lead to clinical situations of volume depletion or overload: Volume depletion due to renal sodium loss occurs in abrupt restrictions of salt intake in advanced CKD. It occurs more frequently in certain tubulointerstitial kidney diseases (salt losing nephropathies). Volume overload due to sodium retention can occur with glomerular filtration rates below 25 ml/min and leads to edema, arterial hypertension and heart failure. The use of diuretics in volume overload in CKD is useful to force natriuresis (Strength of Recommendation B). Thiazides have little effect in advanced CKD. Loop diuretics are effective and should be used in higher than normal doses (Strength of Recommendation B). The combination of thiazides and loop diuretics can be useful in refractory cases (Strength of Recommendation B). Weight and volume should be monitored regularly in the hospitalized patient with CKD (Strength of Recommendation C). 4. Potassium Balance Disorders: In CKD, the ability of the kidneys to excrete potassium decreases proportionally to the loss of glomerular filtration. Stimulation of aldosterone and the increase in intestinal excretion of potassium are the main adaptive mechanisms to maintain potassium homeostasis until glomerular filtration rates of 10 ml/min. The main causes of hyperkalemia in CKD are the following: Use of drugs that alter the ability of the kidneys to excrete potassium: ACEIs, ARBs, NSAIDs, aldosterone antagonists, nonselective beta-blockers, heparin, trimetoprim, calcineurin inhibitors. Determination of serum potassium two weeks after the initiation of treatment with ACEIs/ARBs is recommended (Strength of Recommendation C). Routine use of aldosterone antagonists in advanced CKD is not recommended (Strength of Recommendation C). Abrupt reduction in glomerular filtration rate: Constipation. Prolonged fasting. Metabolic acidosis. A low-potassium diet is recommended with GFR less than 20 ml/min, or GFR less than 50 ml/min if drugs that raise serum potassium are taken (Strength of Recommendation C). In the absence of symptoms or electrocardiographic abnormalities, review of medications, restriction of dietary potassium and use of oral ion exchange resins are usually sufficient therapeutic measures (Strength of Recommendation C). If symptoms and/or electrocardiographic abnormalities are present, the usual parenteral pharmacological measures should be used (10% calcium gluconate, insulin and glucose, salbutamol, resins, diuretics) (Strength of Recommendation A). Parenteral bicarbonate and ion exchange resins in enemas are not recommended as first-line treatment (Strength of Recommendation C). Hemodialysis should be considered in patients with glomerular filtration rates below 10 ml/min (Strength of Recommendation C). 5. Acid-Base Disorders in CKD: Moderate metabolic acidosis (Bic 16-20) mEq/L is common with glomerular filtration rates below 20 ml/min, and favors bone demineralization due to the release of calcium and phosphate from the bone, chronic hyperventilation, and muscular weakness and atrophy. Its treatment consists of administration of sodium bicarbonate, usually orally (0.5-1 mEq/kg/day), with the goal of achieving a serum bicarbonate level of 22-24 mmol/L (Strength of Recommendation C). Limitation of daily protein intake to less than 1 g/kg/day is also useful (Strength of Recommendation C). Use of sevelamer as a phosphate binder aggravates metabolic acidosis since it favors endogenous acid production and therefore acidosis should be monitored and corrected if it occurs (Strength of Recommendation C). Hypocalcemia should always be corrected before metabolic acidosis in CKD (Strength of Recommendation B). Metabolic acidosis is an infrequent disorder and requires exogenous alkali administration (bicarbonate, phosphate binders) or vomiting."}, {"id": "wiki20220301en023_76154", "title": "Assessment of kidney function", "score": 0.00980392156862745, "content": "The severity of chronic kidney disease (CKD) is described by six stages; the most severe three are defined by the MDRD-eGFR value, and first three also depend on whether there is other evidence of kidney disease (e.g., proteinuria): 0) Normal kidney function – GFR above 90 (mL/min)/(1.73 m2) and no proteinuria 1) CKD1 – GFR above 90 (mL/min)/(1.73 m2) with evidence of kidney damage 2) CKD2 (mild) – GFR of 60 to 89 (mL/min)/(1.73 m2) with evidence of kidney damage 3) CKD3 (moderate) – GFR of 30 to 59 (mL/min)/(1.73 m2) 4) CKD4 (severe) – GFR of 15 to 29 (mL/min)/(1.73 m2) 5) CKD5 kidney failure – GFR less than 15 (mL/min)/(1.73 m2) Some people add CKD5D for those stage 5 patients requiring dialysis; many patients in CKD5 are not yet on dialysis. Note: others add a \"T\" to patients who have had a transplant regardless of stage."}, {"id": "wiki20220301en019_111576", "title": "Glomerular filtration rate", "score": 0.009708737864077669, "content": "The severity of chronic kidney disease (CKD) is described by six stages; the most severe three are defined by the MDRD-eGFR value, and first three also depend on whether there is other evidence of kidney disease (e.g., proteinuria): 0) Normal kidney function – GFR above 90 mL/min/1.73 m2 and no proteinuria 1) CKD1 – GFR above 90 mL/min/1.73 m2 with evidence of kidney damage 2) CKD2 (mild) – GFR of 60 to 89 mL/min/1.73 m2 with evidence of kidney damage 3) CKD3 (moderate) – GFR of 30 to 59 mL/min/1.73 m2 4) CKD4 (severe) – GFR of 15 to 29 mL/min/1.73 m2 5) CKD5 kidney failure – GFR less than 15 mL/min/1.73 m2 Some people add CKD5D for those stage 5 patients requiring dialysis; many patients in CKD5 are not yet on dialysis. Note: others add a \"T\" to patients who have had a transplant regardless of stage."}, {"id": "pubmed23n0079_7610", "title": "[Hyperkalemic emergency: causes, diagnosis and therapy].", "score": 0.009708737864077669, "content": "Eight patients with life threatening hyperkalemia were treated in the intensive care unit over a period of 2 years. Serum potassium at admission was 7.1-11.2. Two patients had to be resuscitated and 3 exhibited quadriplegia or paralyses but were alert. Seven showed marked ECG change: in 5 the QRS-complexes were extremely broadened and in one case an AV-rhythm was observed; the atrial wave was absent in all 7 patients. Renal failure was present in 7 of 8 patients. In 6 of these 8 cases drugs were also involved in the development of hyperkalemia. The following therapeutic procedure is recommended for hyperkalemia: (1) injection of calcium, (2) inhalation or injection of beta 2-mimetics (well documented in the literature; clinical experience limited), (3) insulin and glucose i.v., (4) sodium bicarbonate, but only in case of metabolic acidosis, (5) hemodialysis, (6) cation exchange resins or furosemide in non-acute situations."}, {"id": "pubmed23n0792_22080", "title": "Acute ascending muscle weakness secondary to medication-induced hyperkalemia.", "score": 0.009615384615384616, "content": "Secondary hyperkalemic paralysis is an uncommon but potentially life-threatening consequence of drug-induced disease. We report a case of a 53-year-old female with history of chronic kidney disease presenting to the emergency department with a one-day history of upper and lower extremity weakness and paresthesias. Serum potassium concentration on admission was greater than 8 mEq/L, and serum creatinine was elevated above baseline. Electrocardiogram showed first-degree atrioventricular block with peaked T waves. The patient reported compliance with daily lisinopril 10 mg, spironolactone 25 mg, and 40 mEq twice daily of potassium chloride. Symptoms and electrocardiogram returned to baseline within 24 hours of presentation and serum potassium returned to 4.2 mEq/L at approximately 36 hours without the need for dialysis. This case emphasizes the importance of including such a condition in the differential diagnosis of patients with ascending paralysis and the importance of close monitoring of patients placed on potassium-elevating agents. "}, {"id": "article-28355_16", "title": "Renal Failure -- Treatment / Management -- Acute Renal Failure", "score": 0.009523809523809525, "content": "The mainstay is treating the underlying cause and associated complications If the case of oliguria and no volume overload is noted, a fluid challenge may be appropriate with diligent monitoring for volume overload In the case of hyperkalemia with ECG changes, IV calcium, sodium bicarbonate, and glucose with insulin should be given. These measures drive potassium into cells and can be supplemented with polystyrene sulfonate, which removes potassium from the body. Hemodialysis is also an emergency method of removal. If acidosis is present, serum bicarbonate intravenous or oral versus emergency/urgent dialysis based on the clinical situation If obstructive etiology presents treat accordingly. Bladder outlet obstruction secondary to prostatic hypertrophy may benefit from Flomax or other selective alpha-blockers"}, {"id": "pubmed23n1104_15753", "title": "Association of dyskalemias with short-term health care utilization in patients with advanced CKD.", "score": 0.009433962264150943, "content": "<bBACKGROUND:</b Patients with advanced chronic kidney disease (CKD) are at high risk for dyskalemias, which may induce arrhythmias that require immediate emergent or hospital care. The association of dyskalemias with short-term hospital/emergency room (ER) visits in advanced CKD is understudied. <bOBJECTIVE:</b To assess the association of dyskalemias with short-term hospital/ER visits in an advanced CKD population. <bMETHODS:</b From among 102,477 US veterans transitioning to dialysis from 2007 to 2015, we identified 21,366 patients with 2 predialysis outpatient eGFR &lt; 30 ml/min/1.73m<sup2</sup 90-365 days apart (with the second eGFR serving as the index date) and at least 1 potassium (K) in the baseline period (1 year before index) and 1 outpatient K (oK) in the follow-up (1 year after the index but before dialysis initiation). We examined the association of time-varying hypokalemia (K &lt; 3.5 mEq/L) and hyperkalemia (K &gt; 5.5 mEq/L) vs referent (3.5-5.5 mEq/L) with separate hospital and ER visits within 2 calendar days following each oK value over the 1-year follow-up period from the index. We used generalized estimating equations with binary distribution and logit link to model the exposure-outcome relationship adjusted for various confounders. We conducted various subgroup and sensitivity analyses to test the robustness of our results. <bRESULTS:</b Over the 1-year follow-up, 125,266 oK measurements were observed, of which 6.8% and 3.7% were classified as hyper- and hypokalemia, respectively. In the multivariable-adjusted model, hyperkalemia (adjusted odds ratio [aOR] = 2.04; 95% CI = 1.88-2.21) and hypokalemia (aOR = 1.66; 95% CI = 1.48-1.86) were associated with significantly higher odds of hospital visits. Similarly, hyperkalemia (aOR = 1.83; 95% CI = 1.65-2.03) and hypokalemia (aOR = 1.24; 95% CI = 1.07-1.44) were associated with significantly higher odds of ER visits. Results were robust to subgroups and sensitivity analyses. <bCONCLUSIONS:</b In patients with advanced CKD, dyskalemias are associated with higher risk of hospital/ER visits. Interventions targeted at lowering the risk of dyskalemias might help in reducing the health care utilization and associated economic burden among patients with advanced CKD experiencing dyskalemias. <bDISCLOSURES:</b This study was supported by grant 5U01DK102163 from the National Institute of Health (NIH) to Kamyar Kalantar-Zadeh and Csaba P. Kovesdy and by resources from the US Department of Veterans Affairs. The data reported here have been supplied in part by the United States Renal Data System (USRDS). Support for VA/CMS data were provided by the Department of Veterans Affairs, Veterans Health Administration, Office of Research and Development, Health Services Research and Development, VA Information Resource Center (project numbers SDR 02-237 and 98-004). Opinions expressed in this article are those of the authors and do not necessarily represent the opinion of the Department of Veterans Affairs or the funding institution. Kovesdy has received honoraria from Akebia, Ardelyx, Astra Zeneca, Bayer, Boehringer-Ingelheim, Cara Therapeutics, Reata, and Tricida unrelated to this study. Kalantar-Zadeh has received honoraria and/or support from Abbott, Abbvie, ACI Clinical (Cara Therapeutics), Akebia, Alexion, Amgen, American Society of Nephrology, Astra-Zeneca, Aveo, BBraun, Chugai, Cytokinetics, Daiichi, DaVita, Fresenius, Genentech, Haymarket Media, Hofstra Medical School, International Federation of Kidney Foundations, International Society of Hemodialysis, International Society of Renal Nutrition &amp; Metabolism, Japanese Society of Dialysis Therapy, Hospira, Kabi, Keryx, Kissei, Novartis, OPKO, National Institutes of Health, National Kidney Foundations, Pfizer, Regulus, Relypsa, Resverlogix, Dr Schaer, Sandoz, Sanofi, Shire, Veterans Affairs, Vifor, UpToDate, and ZS-Pharma, unrelated to this study. Gatwood has received research support from AstraZeneca, Merck &amp; Co., and GlaxoSmithKline unrelated to this study. Obi has received research support from Relypsa/Vifor Pharma Inc. The remaining authors declare that they have no relevant financial interests."}, {"id": "pubmed23n0543_16958", "title": "[Successful resuscitation of intractable hyperkalemic cardiac arrest].", "score": 0.009433962264150943, "content": "We report on a 42-year-old oliguric uremic man on regular hemodialysis who developed sudden cardiac arrest, secondary to severe hyperkalemia, with a plasma potassium concentration of 9.7 mEq x l(-1). The cardiac arrest persisted after the initiation of cardiopulmonary resuscitation and intensive treatment for marked hyperkalemia for an hour and 55 minutes. Therefore a portable percutaneous cardiopulmonary support (PCPS) system had to be instituted while the patient had very prolonged refractory ventricular fibrillation. His cardiac rhythm was restored immediately after application of PCPS and he recovered without neurological sequelae. We therefore suggest that PCPS should be considered as a therapeutic option during cardiopulmonary resuscitation for life-threatening cardiac arrest secondary to severe hyperkalemia."}, {"id": "pubmed23n0677_1225", "title": "Beating the odds--surviving extreme hyperkalemia.", "score": 0.009345794392523364, "content": "Severe hyperkalemia (&gt;7 mmol/L) is a medical emergency because of possible fatal arrhythmias. We here report the case of a 58-year-old woman surviving extreme hyperkalemia (&gt;10 mmol/L). The patient with a history of congestive heart failure, a DDD pacemaker and mild chronic renal insufficiency was admitted with progressive weakness and sudden onset of hypotension and bradycardia in the absence of any pacemaker action. Laboratory tests revealed an extreme serum potassium level of 10.1 mmol/L, with a slightly elevated serum creatinine of 149 μmol/L. Treatment with norepinephrine, sodium bicarbonate, and insulin improved both the hemodynamic situation and the serum potassium with subsequent regaining pacemaker actions even before additional hemodialysis normalized the potassium level. A thorough investigation demonstrated that several mechanisms contributed to the extreme potassium level: urinalysis and a low transtubular potassium gradient in the presence of metabolic acidosis with normal anion gap pointed to preexisting interstitial nephritis, with renal tubular acidosis type IV as the predisposing factor, whereas several drugs and acute impairment of renal function contributed to the dangerous situation. Despite the odds for fatal outcome, the patient recovered completely, and long-term management was initiated to prevent recurrent hyperkalemia."}, {"id": "pubmed23n1145_3554", "title": "Recommendations for the management of hyperkalemia in the emergency department.", "score": 0.009317307276579187, "content": "Hyperkalemia, a common electrolyte disorder, is seen often in emergency departments. Patient outcomes are impacted by proper management, which requires consideration of both clinical and laboratory findings in relation to kidney function, hydration, the acid-base balance, and heart involvement. Delicate decisions about the timing of potassium level correction must be tailored in each case. For these reasons the Spanish Society of Emergency Medicine (SEMES), the Spanish Society of Cardiology (SEC), and the Spanish Society of Nephrology (SEN) joined forces to come to a consensus on defining the problem and recommending treatments that improve hospital emergency department management of hyperkalemia. Intravenous calcium, insulin and glucose, and salbutamol continue to be used to treat acute hyperkalemia. Either loop or thiazide diuretics can help patients if volume is not depleted, and dialysis may be necessary if there is kidney failure. Ion-exchange resins are falling into disuse because of adverse effects and poor tolerance, whereas novel gastrointestinal cation-exchange resins are gaining ground and may even be of some use in managing acute cases. It is essential to adjust treatment rather than discontinue medications that, even if they favor the development of hyperkalemia, will improve a patient's long-term prognosis. Valid alternative treatment approaches must therefore be sought for each patient group, and close follow-up is imperative."}, {"id": "pubmed23n0846_18791", "title": "Association between Serum Potassium and Outcomes in Patients with Reduced Kidney Function.", "score": 0.009259259259259259, "content": "Patients with CKD are more likely than others to have abnormalities in serum potassium (K(+)). Aside from severe hyperkalemia, the clinical significance of K(+) abnormalities is not known. We sought to examine the association of serum K(+) with mortality and hospitalization rates within narrow eGFR strata to understand how the burden of hyperkalemia varies by CKD severity. Associations were examined between serum K(+) and discontinuation of medications that block the renin-angiotensin-aldosterone system (RAAS), which are known to increase serum K(+). A cohort of patients with CKD (eGFR&lt;60 ml/min per 1.73 m(2)) with serum K(+) data were studied (n=55,266) between January 1, 2009, and June 30, 2013 (study end). Serum K(+), eGFR, and covariates were considered on a time-updated basis. Mortality, major adverse cardiovascular events (MACE), hospitalization, and discontinuation of RAAS blockers were considered per time at risk. During the study, serum K(+) levels of 5.5-5.9 and ≥6.0 mEq/L were most prevalent at lower eGFR: they were present, respectively, in 1.7% and 0.2% of patient-time for eGFR of 50-59 ml/min per 1.73 m(2) versus 7.6% and 1.8% of patient-time for eGFR&lt;30 ml/min per 1.73 m(2). Serum K(+) level &lt;3.5 mEq/L was present in 1.2%-1.4% of patient-time across eGFR strata. The median follow-up time was 2.76 years. There was a U-shaped association between serum K(+) and mortality; pooled adjusted incidence rate ratios were 3.05 (95% confidence interval, 2.53 to 3.68) and 3.31 (95% confidence interval, 2.52 to 4.34) for K(+) levels &lt;3.5 mEq/L and ≥6.0 mEq/L, respectively. Within eGFR strata, there were U-shaped associations of serum K(+) with rates of MACE, hospitalization, and discontinuation of RAAS blockers. Both hyperkalemia and hypokalemia were independently associated with higher rates of death, MACE, hospitalization, and discontinuation of RAAS blockers in patients with CKD who were not undergoing dialysis. Future studies are needed to determine whether interventions targeted at maintaining normal serum K(+) improve outcomes in this population."}, {"id": "pubmed23n1032_5007", "title": "Prevalence and correlates of hyperkalemia in a renal nutrition clinic.", "score": 0.009174311926605505, "content": "Hyperkalemia (HK) is a frequent complication of chronic kidney disease (CKD). Vegetable-based renal diets are considered at risk due to the high potassium (K) content. The aim of this study was to describe the prevalence and correlates of chronic hyperkalemia (HK) in CKD patients on nutritional care, and in particular, the risk of HK in patients on plant-based versus animal-based low-protein diets. We recruited adult patients affected by CKD not on dialysis, afferent to our renal nutrition clinic from November 2014 to May 2019. We evaluated a total of 870 accesses in 219 patients (172 m, 47 f, age 67 ± 13 years). HK was defined as mild when K serum level was 5.1-5.9 mEq/l, moderate when K serum level was 6.0-6.9 mEq/l, and severe HK when K serum level was ≥ 7 mEq/l. Biochemical, anthropometric data and medications were recorded. The prevalence of HK in all the renal nutrition visits was 26.1%; all but six cases were mild HK, whereas no severe HK was observed. The prevalence of HK was associated with decreased eGFR, up to 36.5% for eGFR &lt; 20 ml/min. Medications were similar in hyperkalemic and normokalemic patients, RAASi being present in up to 85% of patients. In a follow-up of 40 ± 14 months, no association was found between HK and mortality, whereas HK, at the start of follow-up, showed a trend to increased ESRD risk. Serum potassium levels and prevalence of HK were not different between patients on animal-based low-protein diet and plant-based low-protein diet. In conclusion, chronic HK is quite prevalent in a renal nutrition clinic, especially when eGFR falls down below 60 ml/min, thereby reaching the highest prevalence in CKD stage 4. Hyperkalemia is mostly mild, being moderate to severe HK quite infrequent. Hyperkalemia was not associated with higher risk of mortality, whereas a trend, although not statistically significant, was observed for lower ESRD-free survival. Plant-based low-protein diet is not associated with significant higher prevalence of HK with respect to animal-based LPD at the same residual kidney function."}, {"id": "pubmed23n0317_20227", "title": "Hyperkalemia in hospitalized patients: causes, adequacy of treatment, and results of an attempt to improve physician compliance with published therapy guidelines.", "score": 0.009174311926605505, "content": "Hyperkalemia is a common, potentially life-threatening disorder. Electrocardiograms are considered to be sensitive indicators of the presence of hyperkalemia. Since the treatment of hyperkalemia involves relatively few maneuvers and because its success can be objectively scored, we investigated how physicians manage this disorder and how successful their prescribed therapy is. We also sought to determine whether treatment could be improved by providing the treating physicians with therapy guidelines on a real-time basis. Consecutive patients with hyperkalemia were identified by review of laboratory records. During the observation-only phase of the study, demographic data, contributing causes, electrocardiogram findings, treatments used, compliance with prescribing guidelines, and patient outcome were recorded. During the subsequent notification phase of the study, treatment recommendations were sent to the patient's ward when the elevated potassium value was noted. The same outcome data were collected. There were 127 episodes of hyperkalemia during the observation-only phase and 115 during the notification phase. No patients died or had life-threatening cardiac arrhythmias. Electrocardiographic abnormalities consistent with hyperkalemia were observed in only 14% of episodes. Renal failure (77%), drugs (63%), and hyperglycemia (49%) contributed to most episodes. Treatments used were exchange resin (51%), insulin (46%), calcium (36%), bicarbonate (34%), and albuterol (4%). The agents were equally efficacious. The time to first treatment was shorter in patients with potassium levels of 6.5 mmol/L or more than in patients with lower values (2.1 +/- 2.2 vs 2.8 +/- 2.4 hours; P&lt;.05). Treatment was better in the intensive care unit than on regular wards. Only 39% of episodes during the observation-only period met the predetermined criteria for monitoring and diagnosis, initial treatment, and follow-up. During the notification period, physician performance was no better; only 42% of episodes met all criteria. The laboratory transmitted a copy of the guidelines to the patient's ward only 38% of the time. In a separate analysis of these episodes, there was no improvement in treatment. Physicians who did not receive the notification fulfilled all treatment criteria more often than physicians who did (50% vs 30%; P&lt;.05). Although treatment of hyperkalemia was frequently suboptimal, no serious arrhythmias and no deaths complicated management of 242 episodes of severe hyperkalemia. A narrowly targeted effort to improve physician management of a disorder with discrete treatment options did not improve therapy."}, {"id": "Gynecology_Novak_3540", "title": "Gynecology_Novak", "score": 0.009092911934947625, "content": "Hyperkalemia is encountered infrequently in preoperative patients. It is usually associated with renal impairment but can be seen in patients who have adrenal insufficiency, are taking potassium-sparing diuretics, and have marked tissue breakdown such as that occurring with crush injuries, massive gastrointestinal bleeding, or hemolysis. The clinical manifestations are mainly cardiovascular. Marked hyperkalemia (potassium >7 mEq/L) can result in bradycardia, ventricular fibrillation, and cardiac arrest. The treatment chosen depends on the severity of the hyperkalemia and whether there are associated cardiac abnormalities detected with electrocardiography. Calcium gluconate (10 mL of a 10% solution), given intravenously, can offset the toxic effects of hyperkalemia on the heart. One ampule each of sodium bicarbonate and D5W, with or without insulin, will cause a rapid shift of potassium into cells. Over the longer term, cation exchange resins such as sodium polystyrene sulfate"}, {"id": "pubmed23n0988_6597", "title": "Association of hyperkalemia with clinical outcomes in advanced chronic kidney disease.", "score": 0.00909090909090909, "content": "Patients with advanced chronic kidney disease (CKD) are at greatest risk of hyperkalemia (HK). The relationship between HK and negative outcomes (mortality or progression of renal insufficiency) in non-dialysis dependent CKD patients is controversial. To determine the incidence, prevalence, and factors related with HK in a cohort of CKD patients, and its relationship with mortality, hospitalization rate, CKD progression, and dialysis initiation. A retrospective, observational study in an incident cohort of adult patients with stage 4 or 5 CKD not on dialysis. Inclusion criteria were: having at least three consecutive estimated glomerular filtration rate (eGFR) measurements in a follow-up period &gt;3 months. Decline in renal function was estimated as the slope of the individual linear regression line of eGFR over follow-up time. HK was defined as serum K levels ≥5.5 meq/l. Associations of HK with outcomes were adjusted for major confounding variables in the multivariate analysis. The study group consisted of 1079 patients (574 males, mean age: 65±14 years) with mean baseline eGFR 14.8±4.5 ml/min/1,73 m<sup2</sup. Mean follow-up time was 15 months with a median of 7 serum sample determinations per patient. HK was observed at baseline in 26% of patients; in at least one serum sample during the individual follow-up period in 68%; or chronically (&gt;50% of samples) in 33% of patients. By multivariate logistic regression, the best determinants of chronic HK were: male sex (OR = 1.529; 95% CI [1.154-2.025], p = .003), serum bicarbonate (OR = 0.863 [0.829-0.900], p &lt;.0001), diuretic treatment (OR = 0.743 [0.556-0.992], p = .044), and angiotensin converting enzyme inhibitor and/or angiotensin receptor blockers (OR = 4.412 [2.915-6.678], p &lt;.0001). Patients whose serum K levels were in the upper quartile showed a significantly faster CKD progression (-4.05±5.22 vs. -2.69±5.61 ml/min/1.73 m<sup2</sup/year, p &lt;.0001), and more frequent dialysis initiation (63% vs. 57%, p = .115), though lower mortality (9% vs. 17%, p = .003) and hospitalization rates (2.68±5.94 vs. 3.16±6.77 days per year, p = .301) than the other study patients. However, in the multivariate analysis, average serum K levels were not independently associated with the clinical outcomes investigated. HK is a common biochemical finding in non-dialysis dependent CKD patients, mainly associated with prescribed medication. However, HK was not independently associated with major negative clinical outcomes."}, {"id": "pubmed23n0064_21557", "title": "[2 cases from the practice of intensive treatment].", "score": 0.00909090909090909, "content": "Two cases from the intensive treatment practice are presented: Case I--a 43-year-old woman drawn out from a state of clinical death during chronic dialysis for chronic renal failure with importunate ultrafiltration to combat the severe pulmonary edema, which led to hypoxia and cardiac arrest with functionally affected hemodynamic parameters (central venous pressure), because of centrally placed a/v fistula. Case II--a 46-year-old woman with severe drug disease and extremely critical hypotonic hyperhydration and anasarca, treated also with ultrafiltration, enhanced sodium influx and intensive application of diuretics; for 24 hours a negative fluid balance was achieved (71191 ml) until finally a relative fluid-electrolyte equilibrium was reached."}, {"id": "wiki20220301en202_5863", "title": "Xipamide", "score": 0.009009009009009009, "content": "Unlike with thiazides, only terminal kidney failure renders xipamide ineffective. Uses Xipamide is used for cardiac oedema caused by decompensation of heart failure renal oedema, chronic renal disease (but not with anuria) hepatic oedema caused by cirrhosis ascites lymphoedema hypertension in combination with chronic renal disease Pharmacokinetics After oral administration, 20 mg of xipamide are resorbed quickly and reach the peak plasma concentration of 3 mg/l within an hour. The diuretic effect starts about an hour after administration, reaches its peak between the third and sixth hour, and lasts for nearly 24 hours. One third of the dose is glucuronidized, the rest is excreted directly through the kidney (1/3) and the faeces (2/3). The total plasma clearance is 30-40 ml/min. Xipamide can be filtrated by haemodialysis but not by peritoneal dialysis. Dosage Initially 40 mg, it can be reduced to 10–20 mg to prevent a relapse."}, {"id": "pubmed23n0268_4005", "title": "Outcomes of severe hyperkalemia in cardiopulmonary resuscitation with concomitant hemodialysis.", "score": 0.009009009009009009, "content": "To investigate the efficacy of hemodialysis during cardiopulmonary resuscitation as an effective adjunct to the treatment of severe hyperkalemia. A prospective study. In hospital dialysis units and intensive care units. Renal failure patients who developed hyperkalemia induced cardiac arrest and failed to recover from conventional cardiopulmonary resuscitation (CPR) were included. Three patients entered into this study: 2 patients with chronic renal failure maintained on regular hemodialysis and one with acute renal failure who suffered from severe hyperkalemia. All three patients developed asystolic cardiac arrest with unrecordable blood pressure due to severe hyperkalemia. Aggressive CPR together with intravenous epinephrine, sodium bicarbonate and calcium chloride were instituted. External cardiac massage with cardiac defibrillation was unable to restore spontaneous heart action. After lack or response to intensive resuscitation, hemodialysis was performed concomitant with CPR to eliminate the potassium load. Sinus rhythm and blood pressure were restored in all 3 patients but one of them eventually succumbed to her underlying disease. Hemodialysis during CPR is probably an effective adjunct to the treatment of severe hyperkalemia in patients with severe hemodynamic compromise and asystolic cardiac arrest."}, {"id": "wiki20220301en085_59102", "title": "Contrast-induced nephropathy", "score": 0.008928571428571428, "content": "Decreased kidney function European guidelines classify a pre-existing decreased kidney function to be a risk factor of contrast-induced nephropathy in the following cases: Estimated glomerular filtration rate (eGFR) < 45 ml/min/1.73 m2 of body surface area before intra-arterial administration with first-pass renal exposure (not passing lungs or peripheral circulation before kidneys), or in the intensive care unit eGFR < 30 ml/min/1.73 m2 before intravenous administration or intra-arterial administration with second-pass renal exposure Known or suspected acute kidney injury To calculate estimated GFR from creatinine, European guidelines use the CKD-EPI formula in adults ≥ 18 years, and the revised Schwartz formula in children. Swedish guidelines recommends no specific formula in children because of lack of evidence, but on the other hand recommends GFR based on cystatin C rather than creatinine in those with abnormal muscle mass or liver failure or cirrhosis."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 269]], "word_ranges": [[0, 48]], "text": "They comment on the case of a young woman with symptoms of systemic disease of probable autoimmune origin (lupus) who presents with macrocytic anemia, options 2 and 4 are options to identify the autoimmune disease but what they ask us is what to do to treat the anemia,"}, "3": {"exist": true, "char_ranges": [[273, 515]], "word_ranges": [[49, 90]], "text": "we should suspect that the anemia is secondary to the autoimmune disease and therefore request a direct Coombs test, since if it is positive the patient, except in extreme cases, will not be transfused and the primary cause should be treated."}, "4": {"exist": true, "char_ranges": [[0, 269]], "word_ranges": [[0, 48]], "text": "They comment on the case of a young woman with symptoms of systemic disease of probable autoimmune origin (lupus) who presents with macrocytic anemia, options 2 and 4 are options to identify the autoimmune disease but what they ask us is what to do to treat the anemia,"}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "They comment on the case of a young woman with symptoms of systemic disease of probable autoimmune origin (lupus) who presents with macrocytic anemia, options 2 and 4 are options to identify the autoimmune disease but what they ask us is what to do to treat the anemia, so we should suspect that the anemia is secondary to the autoimmune disease and therefore request a direct Coombs test, since if it is positive the patient, except in extreme cases, will not be transfused and the primary cause should be treated. It also speaks of rapid onset, which in the case of vitamin B12 is more progressive.", "full_answer_no_ref": "They comment on the case of a young woman with symptoms of systemic disease of probable autoimmune origin (lupus) who presents with macrocytic anemia, [HIDDEN], but what they ask us is what to do to treat the anemia, so we should suspect that the anemia is secondary to the autoimmune disease and therefore request a direct Coombs test, since if it is positive the patient, except in extreme cases, will not be transfused and the primary cause should be treated. It also speaks of rapid onset, which in the case of vitamin B12 is more progressive.", "full_question": "A 26-year-old woman consults for a feeling of generalized weakness that has progressively set in over the course of three weeks, becoming particularly intense in the last two days. For the last couple of years, she reports episodes of joint pain in the hands that have required the use of anti-inflammatory drugs, as well as the appearance of an erythematous lesion of unclear cause in the neckline area, mainly in summer. In the physical examination there was only an evident cutaneous-mucosal pallor and a heart rate of 100 bpm. The hemogram shows: Hb 6 gr/dL, Hto 27 %, MCV 105 fL, 3,420 leukocytes/mm3 (2300 neutrophils/mm3, 800 lymphocytes/mm3, 250 monocytes/mm3, 50 eosinophils/mm3, 20 basophils/mm3), platelets 170,000/mm3. Biochemistry: AST 30 IU/L, ALT 35 IU/L, GGT 59 IU/L, alkaline phosphatase 105 IU/L, LDH 490 IU/L, urea 20 mg/dL, creatinine 0.8 mg/dL. Taking into account the available information, indicate which of the following additional analytical parameters you would need to know in order to make the most appropriate immediate decision:", "id": 494, "lang": "en", "options": {"1": "Vitamin B12.", "2": "Antinuclear antibodies.", "3": "Direct Coombs' test.", "4": "Anti-DNA antibodies.", "5": NaN}, "question_id_specific": 157, "type": "HEMATOLOGY", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0756_22389", "title": "[A predisposing clinical condition for disseminated tuberculosis: hairy cell leukemia].", "score": 0.015941756103684177, "content": "Hairy cell leukemia (HCL), a rare and slow-progressive B-cell lymphoproliferative disease, enhances predisposition to infectious complications, especially to disseminated mycobacterial infections. Although the association between HCL and mycobacterial disease has been established, disseminated Mycobacterium tuberculosis infection has been reported only in a few case series. In this report, a disseminated tuberculosis (TB) case who had been diagnosed as HCL with histopathologic examination of the bone marrow after being investigated for the etiology of fever of unknown origin, was presented. A 56-year-old male patient who was admitted to our clinic with the complaints of three weeks' duration fever, chills, night sweats and cough was hospitalized. On physical examination, the body temperature of the patient who appeared very ill, was 39°C. Dispersed macular rash that turned pale when pressure was applied, was detected on the legs, arms and back. There were no signs of peripheral lymphadenopathy, hepatomegaly or splenomegaly. Laboratory results revealed haemoglobin 10 g/dl, white blood cell count 1000/mm3, thrombocytes 143.000/mm3, erythrocyte sedimentation rate 41 mm/h, CRP 200 mg/L, uric acid 9.5 mg/dl, AST 118 IU/L, ALT 102 IU/L and LDH 429 IU/L. Thorax computed tomography showed mediastinal and bilateral hilary lymphadenopathy. Although preliminary diagnosis was lymphoma, examination of acid-fast bacilli in three days sequential sputum samples and sputum culture for the growth of mycobacteria (Bactec MGIT 960 TB system, Becton Dickinson, Md) were performed to rule out miliary TB. Blood cultures were also performed with non-radiometric fully automated TB hemoculture bottles (Bactec TB, Becton Dickinson, Md). Sputum cultures yielded no mycobacterial growth, however M.tuberculosis growth was detected in blood culture on the 27th day of inoculation. Bone marrow biopsy revealed HCL. However the patient died on the 14th day of hospitalization. In conclusion, disseminated tuberculosis should be considered for differentional diagnosis in patients with HCL or similar hematologic malignancies since TB is endemic in Turkey."}, {"id": "pubmed23n0660_1372", "title": "[Malignant peritoneal mesothelioma].", "score": 0.013595413595413594, "content": "The peritoneal mesothelioma is a rare pathology with unspecific symptoms reason to be a difficult diagnosis. We report a case of a 58 year old man with diabetes mellitus type 2, arterial hypertension and smoking; without precedent of asbestos exposure. The patient presented a one month history characterized by progressive increase of the abdominal volume and sensation of fullness; three weeks later they added breathlessness and hyporexia. The patient was in regular general condition; he was not presenting hepatic stigmas, edema or adenomegalies. The examination of thorax and cardiovascular it was normal. The abdomen distended by ascites, not painful, liver and spleen not examined. Laboratory: Hemoglobin 11,9 gr/dl, WBC 6840/mm3 Bands 1 %, lymphocytes 10 %, platelets 620000/mm3, PT 12 seconds, PTT 34 seconds, glucose 158 mg/dl, BUN 20,5 mg/ dl, creatinine 1,2 mg/dl, proteins 6,1 gr/dl, albumin 2,6 gr/dl. LDH 316 U/l, beta2microglobulin 2,2 mg/l (0.83-1.15 mg/l). HBV and HCV negative. Ca 19.9, CEA, AFP and PSA negative. Hemocultive negative. Ascitic fluid: ADA 20,3 U/l, serum-ascitic albumin gradient (SAAG) 1,1. Leukocytes 2237 cells/mm3, PMN 6 %, lymphocytes 90 %, mesothelial cells 4 %, proteins 4,6 gr/dl, albumin 2,34 gr/dl, glucose 44 mg/dl, LDH 1918 U/l. Gram and cultive: negatives. BAAR and cultive: negative . Cytology: mesothelial cells with changes of type reagent, Block cell for tumour cells: negative. Abdominal US: increased peritoneum and abundant ascitic fluid. Thoracic-abdominal CT: left side pleural effusion, severe ascites with thick epyplon. Upper GI endoscopy: moderate gastritis. Colonoscopy: two small sessile polyps in sigmoid colon. The finds of the laparoscopy were interpreted like carcinomatosis or peritoneal tuberculosis. The report of the peritoneal biopsy was informed as suggestive of undifferentiated carcinoma; the reappraisal with inmunohystochemic (calretinin +,cytokeratin +, vimentin +) indicated malignant peritoneal mesothelioma, type epithelial. The evolution was torpid. The patient was transferred to the Service of Oncology where they initiated chemotherapy with Cysplatin (CDDP) and died 20 days later. The malignant mesothelioma peritoneal is a unfrequent entity, with limited therapeutic options; generally detected late, with a palliative treatment."}, {"id": "wiki20220301en178_38961", "title": "Ranson criteria", "score": 0.012163433537923929, "content": "Acute pancreatitis not secondary to gallstones At admission: Blood glucose > 11.11 mmol/L (> 200 mg/dL) Age > 55 years Serum LDH > 350 IU/L Serum AST > 250 IU/L WBC count > 16000 cells/mm3 Within 48 hours: Serum calcium < 2.0 mmol/L (< 8.0 mg/dL) Hematocrit decreased by > 10% Oxygen (hypoxemia with PaO2 < 60 mmHg) BUN increased by 1.8 or more mmol/L (5 or more mg/dL) after IV fluid hydration Base deficit (negative base excess) > 4 mEq/L Sequestration of fluids > 6 L Acute pancreatitis secondary to gallstones At admission: Glucose > 220 mg/dl Age > 70 years LDH > 400 IU/L AST > 250 IU/ 100 ml WBC count > 18000 cells/mm3 Within 48 hours: Serum calcium < 8 mg/dL Hematocrit decreased by > 10% Base deficit > 4 mEq/L BUN increased by > 2 mg/dL Sequestered fluid > 6L"}, {"id": "pubmed23n0807_24550", "title": "A case of Crimean-Congo haemorrhagic fever with normal laboratory findings.", "score": 0.01211708645336964, "content": "Crimean-Congo haemorrhagic fever (CCHF) is a tick-borne disease caused by Nairovirus, of the family Bunyaviridae. This is the first case report of a confirmed CCHF case without laboratory abnormality. A 36-year-old woman was admitted to our clinic with the complaints of fever, chills, myalgia and vomiting. She was living in a CCHF-endemic region and had received a tick bite ten days previously. Her complaints had started five days after the tick bite, and bleeding of the nose and vagen followed. Under laboratory analysis, serum white blood cell (WBC) was 7300/mm3, haemoglobin (Hb)11.9 gr/dL, platelet (Plt) count 293000/mm3, aspartate transaminase (AST) was 23 U/L, alanine transaminase (ALT) 14 U/L, lactate dehydrogenase (LDH) 139 U/L, creatinine phosphokinase (CPK) 39 U/L, INR 0.8 and APTT 26.2 seconds. Based on these clinical and epidemiological findings, a diagnosis of CCHF infection was suspected, and the diagnosis of CCHF was confirmed with a blood sample tested by TaqMan-based one-step RT-PCR positivity and IgM antibody positivity. We suggest that patients from an endemic region who have typical epidemiological and clinical findings should be evaluated as a possible case for CCHF even if the laboratory findings are not compatible. "}, {"id": "InternalMed_Harrison_1704", "title": "InternalMed_Harrison", "score": 0.011940673705379587, "content": "Fever ˜38.3° C (101° F) and illness lasting ˜3 weeks and no known immunocompromised state History and physical examination Stop antibiotic treatment and glucocorticoids Obligatory investigations: ESR and CRP, hemoglobin, platelet count, leukocyte count and differential, electrolytes, creatinine, total protein, protein electrophoresis, alkaline phosphatase, AST, ALT, LDH, creatine kinase, antinuclear antibodies, rheumatoid factor, urinalysis, blood cultures (n=3), urine culture, chest x-ray, abdominal ultrasonography, and tuberculin skin test"}, {"id": "InternalMed_Harrison_1673", "title": "InternalMed_Harrison", "score": 0.009900990099009901, "content": "Fever >38.3°C (101°F) on at least two occasions 2. Illness duration of ≥3 weeks 3. 4. Diagnosis that remains uncertain after a thorough history-taking, physical examination, and the following obligatory investigations: determination of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level; platelet count; leukocyte count and differential; measurement of levels of hemoglobin, electrolytes, creatinine, total protein, alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, creatine kinase, ferritin, antinuclear antibodies, and rheumatoid factor; protein electrophoresis; urinalysis; blood cultures (n = 3); urine culture; chest x-ray; abdominal ultrasonography; and tuberculin skin test (TST). The range of FUO etiologies has evolved over time as a result of changes in the spectrum of diseases causing FUO, the widespread Percentage of Cases Due to Indicated Cause"}, {"id": "wiki20220301en033_21958", "title": "Acute pancreatitis", "score": 0.00980392156862745, "content": "At admission age in years > 55 years white blood cell count > 16000 cells/mm3 blood glucose > 11.1 mmol/L (> 200 mg/dL) serum AST > 250 IU/L serum LDH > 350 IU/L At 48 hours Calcium (serum calcium < 2.0 mmol/L (< 8.0 mg/dL) Hematocrit fall >10% Oxygen (hypoxemia PO2 < 60 mmHg) BUN increased by 1.8 or more mmol/L (5 or more mg/dL) after IV fluid hydration Base deficit (negative base excess) > 4 mEq/L Sequestration of fluids > 6 L The criteria for point assignment is that a certain breakpoint be met at any time during that 48 hour period, so that in some situations it can be calculated shortly after admission. It is applicable to both gallstone and alcoholic pancreatitis. Alternatively, pancreatitis can be diagnosed by meeting any of the following:[2] Alternative Ranson score Ranson's score of ≥ 8 Organ failure Substantial pancreatic necrosis (at least 30% glandular necrosis according to contrast-enhanced CT)"}, {"id": "wiki20220301en033_21957", "title": "Acute pancreatitis", "score": 0.009708737864077669, "content": "Practice guidelines state: 2006: \"The two tests that are most helpful at admission in distinguishing mild from severe acute pancreatitis are APACHE-II score and serum hematocrit. It is recommended that APACHE-II scores be generated during the first 3 days of hospitalization and thereafter as needed to help in this distinction. It is also recommended that serum hematocrit be obtained at admission, 12 h after admission, and 24 h after admission to help gauge adequacy of fluid resuscitation.\" 2005: \"Immediate assessment should include clinical evaluation, particularly of any cardiovascular, respiratory, and renal compromise, body mass index, chest x ray, and APACHE II score\" Ranson score The Ranson score is used to predict the severity of acute pancreatitis. They were introduced in 1974. At admission age in years > 55 years white blood cell count > 16000 cells/mm3 blood glucose > 11.1 mmol/L (> 200 mg/dL) serum AST > 250 IU/L serum LDH > 350 IU/L At 48 hours"}, {"id": "pubmed23n0276_2375", "title": "[Roles of department of laboratory medicine on the new patient clinic].", "score": 0.009708737864077669, "content": "It is now quite well accepted that laboratory test results are indispensable or of primary significance to accurately diagnose a new patient's disease. Furthermore, most doctors recently find difficulty in appropriately selecting and ordering necessary but not excess laboratory tests and to read and interpret all the given test results correctly. In our hospital the system of the outpatient clinic will be changed basically on a specialty clinic system. In order to operate such a specialty clinic system effectively, it appears quite important to set up a unit to discriminate new unspecified patients properly and to consult them to an appropriate specialty clinic. As a preliminary trial, we opened a new patient clinic in July, 1992. The aim of this clinic is to accurately diagnose the new patients' disease immediately and to send them to the specialty clinic on the day of their first visit. Prior to history taking and physical examination by the attending doctor, the patients are instructed to take a set of laboratory tests. These include urinalysis, chest X-P, ECG, hematological examinations (RBC, WBC, Ht, Hb, PLT and ESR) and biochemical tests (AST, ALT, ALP, gamma GTP, LDH, CPK, Chol, T-Bil, TP, Alb, TG, BUN, Cr, Glu, Na, K, Ca, P and CRP). These results are transferred to the clinic within one hour so that the doctor is able to make the diagnosis effectively and to refer the patients to an appropriate clinic.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0363_22997", "title": "Toxic epidermal necrolysis following combination of methotrexate and trimethoprim-sulfamethoxazole.", "score": 0.009615384615384616, "content": "A 15-year-old boy with T-cell acute lymphoblastic leukemia (ALL) (FAB L1), diagnosed in 1995, received combination chemotherapy consisting of 6 weeks of induction (vincristine, epirubicin, L-asparaginase, prednisolone) and 2 weeks of consolidation (cytosine arabinosides, etoposide). After achieving remission, for further maintenance of remission, he was treated with 14 cycles of intensive chemotherapy consisting of 6-MP, 10 mg/kg orally on the first 4 days, and cyclophosphamide, 1200 mg/m2, vincristine, 1.5 mg/m2, epirubicin, 15 mg/m2, and cytosine arabinoside, 40 mg/m2, intravenously on days 4, 11, 39, and 40, respectively. On day 18 of each cycle, he received intravenous methotrexate (MTX) infusion in a total dose of 150 mg/m2 plus oral leucovorin (30 mg/m2 ) rescue 36 h after starting MTX therapy. In addition, oral trimethoprim-sulfamethoxazole was given regularly to prevent Pneumocystis carinii infection. The patient achieved remission during the first course of treatment, but 8 months later the disease relapsed. He then received four doses of MTX (800 mg intravenously) plus leucovorin rescue in the following 4 months. During the last MTX therapy, small hemorrhagic bullae were found on the lateral side of the right ankle, but subsided after a few days. Due to partial remission of the disease, he was admitted again in January 1999 for high-dose MTX therapy. An initial hemogram on admission revealed hemoglobin 7.2 g/dL, white cell count 15,200/mm3, platelet count 153/mm3, blood creatinine 0.5 mg/dL, and alanine leucine aminotransferase (ALT) 20 U/L. He received 8500 mg of MTX (5000 mg/m2 ) as a continuous intravenous infusion for 24 h. Thirty-six hours after the start of MTX infusion, leucovorin (30 mg, intravenous) rescue was initiated every 6 h for 3 days. Another preventive measure to cover MTX toxicity included aggressive intravenous fluid replacement (4 L/m2 /day) and the addition of 25 meq/L sodium bicarbonate to the intravenous fluid to alkalinize the urine. Concurrent medication included 6-MP (50 mg) once daily and trimethoprim-sulfamethoxazole (120 mg, 600 mg) twice daily every other day. Plasma MTX levels were 52.36 micromol/L 24 h after MTX infusion, 1.87 micromol/L after 48 h, 0.57 micromol/L after 72 h, and 0.41 micromol/L after 96 h. These indicated delayed MTX plasma clearance. The blood creatinine level was mildly elevated from 0.5 mg/dL to 0.7 mg/dL. Thirty-six hours after the administration of MTX, the patient developed an erythematous painful swelling on the right middle finger. The erythema, with subsequent large bulla formation, progressed to all the fingers, toes, palms, and the soles of the feet. Some erythematous to hemorrhagic papules also appeared on the bilateral elbows. Subsequently, diffuse tender erythema with extensive erosions and focal tiny pustules developed on the back, abdomen, proximal extremities, and face (Fig. 1a,b). A positive Nikolsky's sign was also present. A biopsy specimen of the right dorsal hand lesion revealed parakeratosis, detached acanthotic epidermis with scattered necrotic keratinocytes, dyskeratotic cells and nuclear atypia, neutrophilic exocytosis, and many neutrophils in the papillary dermis (Fig. 2). The skin condition deteriorated rapidly. Toxic epidermal necrolysis-like lesions involved 90% of the total body surface on the fifth day after MTX infusion. Mucositis, diarrhea, involuntary tremor, fever, and chills were noted. The patient was then sent to the burn unit for intensive skin care. Ten days after MTX therapy, profound agranulocytosis and thrombocytopenia (white cell count 100/mm3, platelets 14,000/mm3, and hemoglobin 5.6 g/dL) were found. The patient was then started on granulocyte colony stimulation factor (G-CSF, 5 microg/kg/day), but his general condition deteriorated rapidly and he died 6 days later due to septic shock and multiple organ failure."}, {"id": "pubmed23n0058_20665", "title": "[Clinical diagnosis and laboratory data].", "score": 0.009615384615384616, "content": "Modern medicine can not be practiced without laboratory tests. Laboratory tests play a vital role from the initial stage of clinical examination. The Japanese Society of Clinical Pathology has formed a committee specifically dealing with the effective and economic use of lab tests without missing or duplicating the important tests. As is shown in table 1 in the main text \"Essential Laboratory Tests\" were initially agreed as those tests a patient should take when visiting a clinic regardless of the complaint. From an early stage, laboratory tests were done simultaneously with history-taking and physical examinations. Then the \"Initial Impression\" is obtained and \"Organ-oriented 1st and 2nd screening tests\" and confirmatory tests will be done to make the final diagnosis. To evaluate the validity of the \"Essential Laboratory Tests\", we performed the tests on 1026 patients who visited our general medicine clinic for the first time. We compared the Initial Impression with or without Essential Laboratory Tests. Cases in which a diagnosis could not be made by history-taking and physical examination were decreased from 17.4% to 8.0% by performing the essential laboratory tests. Diagnoses made without the essential laboratory tests were found to be mistaken in 10.4% and the additional use of the tests was suggested to lead to a more accurate diagnosis. In 110 cases, diseases unrelated to the chief complaints, were discovered. Even for the respiratory tract infection, CRP and WBC count, which were included in the essential laboratory tests, were very informative."}, {"id": "pubmed23n0497_5823", "title": "[Leukopenia due to anti-tuberculous chemotherapy including rifampicin and isoniazid].", "score": 0.009523809523809525, "content": "To examine the incidence rate by age and gender of leukopenia caused by chemotherapy including rifampicin (RFP) and isoniazid (INH), and to study the relationships between the leukopenia and the hepatic side effect or other haematological disorders such as thrombocytopenia. Out of the tuberculosis patients who were admitted to our hospital in 1987-88, 1991-92, and 1996-2000, 1,525 patients (1,153 men, 372 women) were chosen for our study who had the white blood cell counts (WBC) in the peripheral blood more than 3,000/mm3 before chemotherapy, and underwent haematologic examination at least twice within 3 months after starting chemotherapy. The definition of leukopenia was as follows: 1) WBC became less than 3,000/mm3 during chemotherapy for patients with pre-treatment WBC more than 4,000/mm3, or 2) WBC decreased more than 1,000/mm3 in patients with pre-treatment WBC between 3,000 and 4,000/mm3. The incidence rates of leukopenia by age, gender, and regimens of chemotherapy were calculated. The case-control study was done between the control and the leukopenia groups excluding patients suffered from agranulocytosis to clarify the hematological and biochemical characteristics of the leukopenia group. The control patients were chosen in the following way. For each patient with leukopenia, a patient with the same admission year, same gender, same regimen of chemotherapy, and the nearest age was chosen as a control patient. The changes in counts of white blood cell, granulocyte, and platelet, in hemoglobin concentration, and in hepatic enzyme levels before and during chemotherapy were compared between the leukopenia and the control groups. Thrombocytopenia was defined as platelet count less than 15 x 10(4)/mm3 and hepatic dysfunction as either asparate aminotransferase (AST) higher than 31 IU/l or alanine aminotransferase (ALT) higher than 34 IU/l. (1) Incidence rate of leukopenia The leukopenia appeared in 36 patients (14 men, 22 women), two (one man, one woman) of whom showed agranulocytosis. The incidence rate was 1.2% (14/1,153) for men and 5.9% (22/372) for women. The incidence rate of women was higher than that of men in the age groups between 20 to 79 y.o., but no difference was seen in the age groups elder than 80 y.o. There were no differences in the incidence rate among groups treated with HRE (E: ethambutol), HRS (S: streptomycin), and HREZ (Z: pyrazinamide). The chemotherapy was continued in 30 patients after the appearance of leukopenia, and the natural recovery from leukopenia was seen in 19 patients, while the leukopenic state lasted during the chemotherapy in the remaining 11 patients. In two patients who exhibited agranulocytosis all drugs were discontinued. In the remaining 4 patients one or more drugs were discontinued. (2) Case-control study between leukopenia (N = 34) and the control (N = 34) groups There were no differences in age, sputum culture positivity on admission, degree of roentgenographic extent of the disease, ratio of cavity formation, and quantity of daily doses between the two groups. There was also no difference between the days until leukopenia appeared after starting chemotherapy (47.6 +/- 29.5 days) in the leukopenia group, and the days until WBC count became minimum within 3 months after starting chemotherapy (41.7 +/- 21.0 days) in the control group. The negativity of tuberculin skin testing was higher in the leukopenia group [7/14 (50%)] than in the control group [1/10 (10%)], however, the difference was statistically not significant due to rather small size of cases. Before the starting chemotherapy, the counts of WBC (7,230 +/- 1,530 vs 5,500 +/- 1,510/mm3, p &lt; 0.001), neutrophil (5,230 +/- 1,450 vs 4,320 +/- 1,620/mm3, p &lt; 0.05), lymphocyte (1,440 +/- 830 vs 830 +/- 440/mm3, p &lt; 0.001) and platelet (34.9 +/- 12.2 vs 24.1 +/- 6.4 x 10(4)/mm3, p &lt; 0.001) in the peripheral blood and the globulin level (3.71 +/- 0.61 vs 3.35 +/- 0.61 g/dl, p &lt; 0.05) in the serum were significantly higher in the control group than in the leukopenia group. The decrements in the counts of WBC and granulocyte during chemotherapy were larger in the leukopenia group than in the control group (delta WBC: 2,880 +/- 1,530 vs 1,910 +/- 1,520/mm3, and delta Neut: 2,840 +/- 1,510 vs 1,820 +/- 1,380/mm3, p = 0.01, respectively), but the counts of lymphocyte were similar in both groups. The platelet counts also decreased in both groups, but to the mid-normal level in the control group, and to the lowest normal level in the leukopenia group, in which 15 out of 34 patients (44%) showed thrombocytopenia. The levels in the serum of hepatic enzymes such as AST, ALT, and gamma-GTP (gamma-glutamyl aminotransferase) increased during chemotherapy in the leukopenia group, while decreased in the control group, and the facts indicate that in the former not only bone marrow cells but also hepatic cells were impaired by anti-tuberculosis drugs. Leukopenia may occur in the course of treatment with anti-tuberculosis drugs, but it is not necessary to stop the chemotherapy immediately, because the WBC count recovers spontaneously or remains under stable leukopenic state during chemotherapy in most cases. But when leukopenia appears, the peripheral blood counts must be checked cautiously, and the chemotherapy should be stopped if the WBC count progressively decreases. The patients who showed leukopenia due to anti-tuberculosis drugs may have had weaker natural and acquired (cell-mediated) immunologic response to tuberculosis infection, and more vulnerable bone marrow cells and hepatic cells to anti-tuberculosis drugs than the control."}, {"id": "pubmed23n0519_1503", "title": "[Laboratory findings in patients with hemorrhagic fever with renal syndrome].", "score": 0.009523809523809525, "content": "To examine the frequency and distribution of hematologic and biochemical laboratory findings in 94 patients with hemorrhagic fever with renal syndrome (HFRS) in the epidemic year 2002. The following laboratory findings were retrospectively analyzed: erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), hemoglobin, hematocrit, leukocyte count and differential percentage (segmented neutrophils, band neutrophils, atypical lymphocytes), platelet count, coagulation tests, blood urea nitrogen (BUN), creatinine, urine, potassium, bilirubin (BIL), aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyltransferase (GT), alkaline phosphatase (ALP), and serum protein electrophoresis. The study included 94 HFRS patients treated at the Dr Fran Mihaljević University Hospital for Infectious Diseases in Zagreb during 2002. ESR increase, mostly mild to moderate, was found in 86.2% of study patients. Increased CRP was recorded in 98.9% of study patients, however, one-fourth had CRP higher than 100 mg/L. Leukocytosis was recorded in 38.3% (10.1 +/- 4.2 x 10(9)/L), thrombocytopenia in 89.4% patients (68.2 +/- 48.3 x 10(9)/L), and severe thrombocytopenia (x 10(9)/L) in six patients. Three patients had abnormal coagulation tests. Increased values of BUN and creatinine were recorded in more than a half of patients, while only four patients had mild hyperkalemia. Only three patinets required hemodialysis. Mildly to moderately increased values of aminotransferases (AST, ALT, GT) were observed in more than 2/3; hypoalbuminaemia in nearly 1/3, and elevated alpha-2 fraction in more than 2/3 of patients. The majority of patients had pathologic urine findings. First laboratory abnormalities were usually found between day 5 and 7 of the disease (increased CRP level, thrombocytopenia, leukocytosis, and elevation of hemoglobin and hematocrit). Biochemical abnormalities(elevation of cratinine and urea, increased levels of aminotransferases) usually occurred at the beginning of the second week, and ESR increase in the second week of disease. The majority of our patients had laboratory findings characteristic of HFRS. Thrombocytopenia and increased level of CRP were the most common laboratory findings during the first week of the disease. Renal and liver impairment occurred at the beginning of the second week of the disease."}, {"id": "pubmed23n0726_16420", "title": "Fulminant hepatic failure due to dengue.", "score": 0.009433962264150943, "content": "Dengue Fever (DF) is only rarely considered as a cause of acute liver failure even globally and only a few case reports of acute hepatic failure and encephalopathy occurring in DF in adults are available. We report a case of Acute Liver Failure due to Dengue during a major outbreak in 2010 in Chitwan. A 20 year old previously healthy female presented to the emergency department of Chitwan Medical College with fever, jaundice and altered sensorium. She was tested positive for Dengue IgM. Her biochemical and clinical parameters were suggestive of acute liver failure with total billirubin of 10.1 mg/dL, direct billirubin of 5.2 mg/dL, ALT 5760 IU, AST 14100 IU, alkaline phosphatase of 1250 IU, PT INR of 1.76 and platelet count of 30,000/mm3. Other causes for acute hepatic failure like acute viral hepatitis, leptospirosis, malaria, Reyes syndrome were ruled out. The patient was admitted and managed in the ICU with supportive care and platelet transfusion. With treatment she made a significant clinical and biochemical improvement with AST of 105 IU, ALT of 120 IU and platelet count of 150,000/mm3. She was discharged after 11 days of hospital stay."}, {"id": "pubmed23n0702_20431", "title": "[Acute pancreatitis and acalculous cholecystitis associated with viral hepatitis A].", "score": 0.009433962264150943, "content": "We report the case of a 14 year-old male from Lima. He is a student with a history of bronchial asthma since age 4 receives conditional salbutamol, corticosteroids used for asthma attacks (a crisis in 2010, 1 month ago) Refuses surgery or transfusions. He presented with a two weeks for abdominal pain, nausea, fever, and jaundice. Epigastric pain is colicky and radiated back to righ upper quadrant, refers in addition to nausea and fever, for ten days notice jaundice of skin and sclera. On examen he was lucid, with jaundice of skin and mucous membranes. There was no palpable lymph nodes, abdomen with bowel sounds, soft, depressible, liver span of 15cm, positive Murphy, no peritonitis. The laboratory findings showed hemoglobin 13gr, MCV 90, platelets 461.000/mm3, WBC 4320/mm, lymphocytes 1700 (39%). total bilirubin: 8.8, B Direct: 7.6, ALT (alanine aminotransferase): 3016, AST (aspartate aminotransferase): 984, alkaline phosphatase: 250, albumin: 3.34gr%, globulin: 2.8, amylase: 589 (high serum amylase), TP: 17, INR: 1.6, VHA IgM positive. 89 mg glucose, urea 19 mg%, creatinine 0.5 mg Hemoglobin 13gr, MCV 90 Platelet 461000/mm3, WBC 4320/mm, Lymphocytes 1700 (39%). The nuclear magnetic resonance showed hepatomegaly associated with thickening of gallbladder wall without stones up to 11mm inside. No bile duct dilatation, bile duct 4mm, pancreas increased prevalence of body size. Mild splenomegaly and free fluid in the space of Morrison and right flank. Abdominal ultrasound revealed a gallbladder wall thickness (11mm), without stones in his light. Pancreas to increase volume with peripancreatic fluid free perivesicular with a volume of 430 cc. Findings consistent with acute acalculous cholecystitis and acute pancreatitis. CT-scan showed enlarged pancreas with predominance of body and tail with peripancreatic edema; the gallbladder was thickening. We report this case because the extrahepatic manifestations of viral hepatitis A infection are uncommon, specially the associated with acute acalculous cholecystitis and acute pancreatitis simultaneous."}, {"id": "Obstentrics_Williams_9133", "title": "Obstentrics_Williams", "score": 0.009427742616033755, "content": "Neutrophils (x 103/mm3) 1.4-4.6 3.6-10.1 3.8-12.3 3.9-13.1 4,6,9,42 Lymphocytes (x103/mm3) 0.7-4.6 1.1-3.6 0.9-3.9 1.0-3.6 4,6,9,42 Monocytes (x103/mm3) 0.1-0.7 0.1-1.1 0.1-1.1 0.1-1.4 6,9,42 Eosinophils (x 103/mm3) 0-0.6 0-0.6 0-0.6 0-0.6 6, 9 Basophils (x103/mm3) 0-0.2 0-0.1 0-0.1 0-0.1 6,r9 200-400c 254-344 220-441 288-530 39,r42 Transferrin, saturation 22-46b Not reported 10-44 5-37 Transferrin, saturation 22-46b Not reported 18-92 9-98 Creatinine (mg/dL) 0.5-0.9d 0.4-0.7 0.4-0.8 0.4-0.9 39,r42,r45 Gamma-glutamyl 9-58 2-23 4-22 3-26 5,r42,r39,r70 transpeptidase (GGT) Lactate dehydrogenase 115-221 78-433 80-447 82-524 42,29,39,r70 Magnesium (mg/dL) 1.5-2.3 1r.6-2.2 1.5-2.2 1.1r-2.2 3,r26,r29,r39,r42,r48,r63 Osmolality (mOsm/kg H2O) 275-295 275-280 276-289 278-280 17,r63 Phosphate (mg/dL) 2.5-4.3 3.1r-4.6 2.5-4.6 2.8-4.6 3,r26,r33,r39,r42 Potassium (mEq/L) 3.5-5.0 3.6-5.0 3.3-5.0 3.3-5.1 20,r26,r29,r39,r42,r63,r66"}, {"id": "wiki20220301en117_11026", "title": "Diagnostic peritoneal lavage", "score": 0.009345794392523364, "content": "Procedure After the application of local anesthesia, a vertical skin incision is made one third of the distance from the umbilicus to the pubic symphysis. The linea alba is divided and the peritoneum entered after it has been picked up to prevent bowel perforation. A catheter is inserted towards the pelvis and aspiration of material attempted using a syringe. If no blood is aspirated, 1 litre of warm 0.9% saline is infused and after a few (usually 5) minutes this is drained and sent for analysis. Interpretation of results If any of the following are found then the DPL is positive and operative exploration is warranted: 10 cc/blood 100,000 RBCs/mm3 500 WBCs/mm3 Presence of bile, bacteria or food particle Amylase level > 175 IU/mL Bilirubin level > 0.01 mg/dL ALP > 2 IU/L See also Peritoneal washing References Bibliography External links Trauma Man: Images of DPL being performed on a simulator Digestive system procedures Trauma surgery"}, {"id": "pubmed23n0820_12236", "title": "[Let's interpret laboratory data as physical findings].", "score": 0.009345794392523364, "content": "It is an important role of a clinical laboratory to provide accurate results of examinations promptly when a physician requires them, and this may be generally achieved. What more can technologists do to improve the medical service? It is more helpful for other medical staff to report crude laboratory data with adequate comments, which can be easily and promptly used in medical treatment. In this reversed clinicopathological conference, the admission periods of patients were divided into several phases: the admission day, from the 1st to 7th days, and from the 1st to 15th days. We closely analyzed routine laboratory data in each phase, and commented on what might be useful for the medical staff to the grasp the pathological state of patients in each phase. If comments from the laboratory become commonly acceptable, clinical laboratories will come to play a more valuable role in medical practice."}, {"id": "pubmed23n0354_18950", "title": "[A case of elderly-onset systemic lupus erythematosus (SLE) complicated with severe liver dysfunction and pancytopenia due to myelofibrosis].", "score": 0.009259259259259259, "content": "A 67-year-old woman was admitted to our hospital with a fever. She had been experiencing arthralgia for about one month. On admission, she had a fever of 38.5 degrees C, was anemic and was experiencing tenderness in the joints of both hands, elbows and feet. Laboratory data revealed proteinuria, urinary cylinders, pancytopenia (WBC 900/mm3, Hb 9.5 g/dl, Plt 7.8 x 10(4)/mm3), liver dysfunction (GOT 414 IU/l, GPT 140 IU/l), and hyper-gamma globulinemia. Antibiotics and granulocyte-colony stimulating factor were administered intravenously. Bone marrow aspiration was unsuccessful, but a bone marrow biopsy revealed bone marrow fibrosis. Immunological examinations were positive for antinuclear antibodies, anti-deoxyribonucleic acid (DNA) antibodies, anti-double stranded anti- DNA antibodies, as well as a decreased level of serum complement and an increased level of serum immune complexes. Tests for viral antigens and antibodies known to cause hepatitis were negative. Based on these findings, a diagnosis of SLE accompanied by liver dysfunction and bone marrow fibrosis was made. Steroid pulse therapy was initiated, but her liver function deteriorated on the first day of steroid therapy, and she died three days later. SLE accompanied by myelofibrosis is extremely rare, and only 17 and cases have been reported to date. Among these reports, the present case is the second oldest subject and the first SLE patient to suffer from both myelofibrosis and severe liver dysfunction."}, {"id": "InternalMed_Harrison_13839", "title": "InternalMed_Harrison", "score": 0.009259259259259259, "content": "Myocarditis appears to be common in both forms of relapsing fever and accounts for some deaths. Most commonly, myocarditis is evidenced by gallops on cardiac auscultation, a prolonged QTc interval, and cardiomegaly and pulmonary edema on chest radiography. General laboratory studies are not specific. Mild to moderate normocytic anemia is common, but frank hemolysis and hemoglobinuria do not develop. Leukocyte counts are usually in the normal range or only slightly elevated, and leukopenia can occur during the crisis. Platelet counts can fall below 50,000/µL. Laboratory evidence of hepatitis can be found, with elevated serum concentrations of unconjugated bilirubin and aminotransferases; the prothrombin and partial thromboplastin times may be moderately prolonged."}, {"id": "pubmed23n0285_10584", "title": "[Undifferentiated connective tissue syndromes (UCTS) accompanied by laryngeal involvement and autoimmune hepatitis].", "score": 0.009174311926605505, "content": "Here we report a patient with undifferentiated connective tissue syndromes (UCTS) who developed hoarseness during exacerbation of autoimmune hepatitis. A 51-year-old woman was hospitalized in November 1993 because of hoarseness and liver dysfunction. She had demonstrated Raynaud's phenomenon, polyarthralgia and hoarseness since 1992. In August 1993, liver dysfunction was noted. On admission, laboratory data showed mild leukopenia, thrombocytopenia (WBC 3,900/mm3, platelet 12.4 x 10(4)/mm3), and elevations of transaminase (GOT 96 IU/l, GPT 79 IU/l) and IgG (4,556 mg/dl). Anti-nuclear antibody (ANA) and anti-smooth muscle antibody were positive. Other autoantibodies including anti-DNA antibody, anti-Scl 70 antibody were all negative. LE test and LE cells were also negative. On laryngoscopic examination, lesions that appeared similar to a bamboo-joint were noted at the middle of the bilateral vocal cords. Pathological findings of liver biopsy specimen were compatible with autoimmune hepatitis. She was treated with 30 mg of prednisolone. Polyarthralgia, hoarseness and the abnormalities of the transaminase levels improved rapidly. Laryngoscopic findings were also normalized. We considered this laryngeal involvement to be acute laryngitis accompanied by some UCTS, including a typical systemic lupus erythematosus (SLE) because of arthritis, cytopenia and ANA positivity. Involvement of the larynx in collagen disease is rarely mentioned in published reports."}, {"id": "pubmed23n0038_8022", "title": "[Evaluation of the quality of diagnostic examinations in ambulatory patients].", "score": 0.009174311926605505, "content": "100 medical records of male and female patients seen for the first time in 1972 at the Medical Outpatients Department, University of Basel (Switzerland) were reviewed retrospectively to assess the quality of history and clinical as well as laboratory diagnostic procedures. In addition, 10 patients were selected for total re-evaluation by renewal clinical and laboratory examinations. On the basis of entries made in the medical records (format DIN A5) the medical history and the clinical examination were judged to be of good quality. A minimal program for laboratory investigation (sedimentation rate, hemoglobin, leukocyte count, serum-transaminase, BUN, urine for sugar and albumin and urine sediment) and small format chest-X-ray had been carried out according to the rules and supplement by additional investigations in 87%. The retrospective check on 10 patients showed that the first examination had been well done and without omissions. Continuous checking of diagnostic procedures was recognized as important, and changes in the forms used were admitted to be necessary for improving quality."}, {"id": "pubmed23n0125_12189", "title": "[Ordering laboratory tests: their quantity and causes of variations among physicians].", "score": 0.00909090909090909, "content": "To establish whether some physicians contribute more than others to the alarming increase in laboratory related expenditure, 317 consecutive medical files established by 10 residents of an outpatient clinic were analyzed. The expense incurred in establishing a diagnosis varied, according to the physicians involved, by a factor of 4.9 for back pain and 2.6 for acute respiratory illness. The difference is even more striking when the various types of investigation are analyzed (blood count, ESR, X-rays). It is therefore possible to group physicians according to their individual tendency to order tests. This classification is not related to age, sex, former professional experience or sub-specialty in internal medicine. There is a significant correlation (Spearman's test) between this classification and the approach to medicine, as evidenced by analysis of the content of an interview on medical practice. These interviews were conducted double blind, recorded and transcribed to determine psychological traits. Physicians who order a large number of tests are characterized by a high index of uncertainty, as evidenced by the large number of hesitant expressions and a tendency to cut the interview short, showing off to compensate for lack of self-assurance, depersonalized reference to patients, few references to the interviewer, difficulty in establishing a true dialogue. These characteristics can be considered to form a coherent entity which shows that even the material aspects of medical practice are strongly influenced by the physician-patient relationship."}, {"id": "pubmed23n0812_18415", "title": "Successful treatment of massive ascites due to lupus peritonitis with hydroxychloroquine in old- onset lupus erythematosus.", "score": 0.009009009009009009, "content": "Systemic lupus erythematous (SLE) is an auto-immune disease with multiple organ involvements that occurs mainly in young women. Literature data suggest that serositis is more frequent in late-onset SLE. However, peritoneal serositis with massive ascites is an extremely rare manifestation. We report a case of old-onset lupus peritonitis treated successfully by Hydroxychloroquine. A 77-year-old Tunisian woman was hospitalized because of massive painful ascites. Her family history did not include any autoimmune disease. She was explored 4 years prior to admission for exudative pleuritis of the right lung without any established diagnosis. Physical examination showed only massive ascites. Laboratory investigations showed leucopenia: 3100/mm3, lymphopenia: 840/mm3 and trace protein (0.03 g/24 h). Ascitic fluid contained 170 cells mm(3) (67% lymphocytes), 46 g/L protein, but no malignant cells. The main etiologies of exudative ascites were excluded. She had markedly elevated anti-nuclear antibody (ANA) titer of 1/1600 and a significantly elevated titer of antibody to double-stranded DNA (83 IU/mL) with hypo-complementemia (C3 levl was at 67 mg/dL). Antibody against the Smith antigen was also positive. Relying on these findings, the patient was diagnosed with SLE and treated with Hydroxychloroquine 200 mg daily in combination with diuretics. One month later, there was no detectable ascitic fluid and no pleural effusions. Five months later she remained free from symptoms while continuing to take chloroquine. This case was characterized by old age of onset of SLE, the extremely rare initial presentation with lupus peritonitis and massive painful ascites with dramatic response to only hydroxychloroquine treatment."}, {"id": "pubmed23n0359_3460", "title": "[Effects of written intervention on the number of laboratory studies per patient].", "score": 0.009009009009009009, "content": "In the context of economic measures in health care we followed over a period of nine months the consequences of a written intervention on the attitude of house staff to prescribe laboratory tests. Since it is well known that these tests have a major impact on health costs several studies have been conducted to test whether costs can be reduced without sacrifice of treatment quality. The study was undertaken in 1997. It had three phases of three months duration each: one for observation, one with the intervention and a follow-up phase. The laboratory tests requested by eleven physicians for their patients during the first month after the initial visit were analyzed. During the intervention--phase six physicians chosen at random were informed about their own average as well as that of the entire group (mean of the entire observation period of all physicians). Unexpectedly the hypothesis that the number of laboratory tests requested per patient would drop only in the group of informed physicians and should stay the same for the physicians without this intervention did not materialize. The number of performed tests dropped in both groups."}, {"id": "pubmed23n0933_17911", "title": "Disseminated Histoplasmosis in an Indonesian HIV-Positive Patient: A Case Diagnosed by Fine Needle Aspiration Cytology.", "score": 0.008928571428571428, "content": "A 30-year-old Javanese-Indonesian man was admitted with complaints of 3 months persistent fever, weight loss, and fatigue. He had never known his past history of unprotected HIV until the admission. His only risk factor is unsafe sex. The patient seemed well nourished. Physical examination revealed blood pressure 100/60 mmHg, pulse 100 beats per minute, respiratory rate 20 times per minute, and temperature 38.8°C. Multiple cervical and inguinal lymphadenopathies were also found. Electrocardiogram showed anterolateral ischemic finding, whereas chest X-ray were normal. Laboratory test results revealed pancytopenia with hemoglobin of 8.2 g/dL, leucocyte count 2000 cells/mm3, platelet 78000 cells/mm3, hematocrit 25.8%, AST 162 IU/L, ALT 81 IU/L, decreased albumin of 2.72 g/dL. The clinical differential diagnosis were lymphoma or tuberculosis lymphadenopathy."}, {"id": "pubmed23n0304_7502", "title": "[Check up of breast cancer stages 1 and 2].", "score": 0.008928571428571428, "content": "We have studied the efficacy of investigations during follow-up of 430 operable node positive and node negative breast cancer patients. Median follow-up was eight years, and 128 patients had relapsed, 91 with metastatic disease. Eight blood analyses, chest X-ray, limited skeletal X-ray and bone scan examinations were undertaken at regular intervals. Of the patients who had relapsed, 59% had symptoms, 23% were detected by clinical examination and 18% were detected by blood analysis only. X-rays and scintigrams were of little value in proportion to the costs. The combination of three blood analyses was useful. An increase in sedimentation rate (ESR) of more than 10 mm/h, an increase in gamma-glutamyltransferase (GT) of 20 U/l and an increase in alkaline phosphatase (ALP) of 60 U/l or more. By using ESR, gamma-GT, ALP, history and clinical examination, costs could be reduced by 90% while maintaining adequate baseline screening for relapse."}, {"id": "pubmed23n0069_17938", "title": "[Comparison of symptoms and clinical and laboratory findings in the first and last weeks of typhoid fever].", "score": 0.008849557522123894, "content": "In this study we examined the clinical and laboratory findings of 80 in-patients. There is an important difference between sexes (p greater than 0.05). Comparison of ages showed that 7-30 age is more vulnerable than the older group. We found clinical symptoms of fever, chills, headache, abdominal pain, disturbances in bowel function, nausea, vomiting, anorexia, and lassitude in the first two weeks more frequently when compared with the 3rd, 4th, 5th weeks of illness (p less than 0.001). Where physical finding of rose spots, discordant pulse rate are important in the first two weeks (p less than 0.001). Abdominal discomfort is an important symptom both in the first two and in the last three weeks (% 40.3 and % 36 respectively). Hepatomegaly and splenomegaly, were found more frequently in the last three weeks. According to laboratory findings of anemia, leukopenia, increased erythrocyte sedimentation rate and positive blood and feces cultures there is no important difference between the first two and last three weeks (p greater than 0.05). Increase in polynuclear leucocytes is important for the first two weeks, and increase in lymphocytes is important in the last three weeks (p less than 0.001). Positivity of group agglutination tests is 57%, in the first two weeks and 83% in the last three weeks. This difference is found to be important."}, {"id": "pubmed23n1109_17765", "title": "[Rhabdomyolysis as the presentation form of COVID-19 infection. Report of one case].", "score": 0.008771929824561403, "content": "COVID-19 infection causes a systemic inflammatory response, which mainly presents as a febrile syndrome with respiratory involvement. We report a 37-year-old male who consulted for myalgia, nausea and epigastric pain lasting three days. On admission, he had crepitations at the lung bases. The initial laboratory showed a creatine kinase of 62,768 U/L, a LDH of 1,110 IU/L, a creatinine a 2.1 mg/dL, an aspartate aminotransferase of 1,347 IU/L, a D-dimer of 1,140 ng/mL, a ferritin of 1,201 ng/mL and a lymphocyte count of 810 cells/mm3. The chest CT scan was compatible with multifocal pneumonia, suggesting a COVID-19 infection. COVID-19 PCR was positive. The patient was managed with hydration, sodium bicarbonate, ceftriaxone, and azithromycin, with a good clinical response."}, {"id": "pubmed23n0334_16863", "title": "Is 'routine' laboratory testing a thing of the past? Current recommendations regarding screening.", "score": 0.008771929824561403, "content": "A 55-year-old man comes to you for a routine physical examination. He is a nonsmoker who takes no medications and has no signs of acute or chronic disease, and he has not seen a doctor in years. What blood work should you order for this patient? The authors of this article help you answer this question in light of recent advances in technology, restrictions in healthcare reimbursement, and increased sophistication in cost-benefit analysis for laboratory testing."}, {"id": "pubmed23n0563_16706", "title": "Blood and urine physiological values in farm-cultured Rana catesbeiana (Anura: Ranidae) in Argentina.", "score": 0.008695652173913044, "content": "A total of 302 samples of healthy farm-cultured Rana catesbeiana specimens (9-21 months-old, 50-350 g liveweight, 50% each sex) from the north-east of Argentina, were analyzed through spectrophotometry, electrophoresis, densitometry, refractometry and microscopy in order to obtain blood and urine normal values. Confidence intervals (p&lt;0.05) for PCV (28.6-31.6%), RBC (0.40-0.44 T/L), MCV (686-732 fL), hemoglobin (6.41-7.20 g/dL), MCH (151-164 pg), MCHC (22.6-24.0%), WBC (18.7-22.3 G/L), neutrophils (58.4-63.4%), lymphocytes (23.9-29.8%), monocytes (2.1-3.8%), eosinophils (4.6-7.0%), basophils (2.9-4.1%), bleeding time (289-393s), coagulation time (452-696s), prothrombin time (76-128s), urinary density (1.0061-1.0089 g/mL), urinary pH (6,38-6.96)., fibrinogen (0.59-0.99 g/dL), total protein (4.19-4.49 g/dL), albumin (1.49-1.67 g/dL), alpha-1 globulin (0.20-0.24 g/dL), alpha-2 globulin (0.48-0.54 g/dL), beta globulin (0.68-0.77 g/dL), gamma globulin (1.28-1.42 g/dL), albumin/globulin ratio (0.50-0.58), creatinine (4.09-5.56 mg/L). urea (76.1-92.4 mg/L), uric acid (11.5-15.4 mg/L), triglycerides (0.34-0.52 g/L), total cholesterol (0.56-0.67 g/L), HDL-C (0.03-0.05 g/L), LDL-C (0.34-0.44 g/L), alpha lipoprotein (6.01-8.67%). beta lipoprotein (91.3-93.9%), glucose (0.45-0.54 g/L), Na (116-121 meq/L), K (3.42-3.81 meq/L), Cl (100-116 meq/L), Ca (7.98-8.61 mg/dL). P (8.319.36 mg/dL), Mg (2.26-2.55 mg/dL), Fe (105-178 ug/dL), ALP (144-170 [U/L), ALT (10.0-14.8 IU/L), AST (42.8-53.4 IU/L), GGT (7.8-10.6 IU/L), LDH (99-135 IU/L), CHE (151-185 lU/L) and CPK (365-500 IU/L), were obtained. Some parameter ranges were similar to those obtained in amphibians, birds or mammals; others were very different. These parameters are useful to evaluate sanitary, metabolic and nutritional state on captive bullfrogs."}, {"id": "pubmed23n0834_10578", "title": "A case of brucellosis presenting with acute hepatitis and bicytopenia.", "score": 0.008695652173913044, "content": "Although liver involvement is frequently seen in brucellosis, acute hepatitis is a rare clinical entity. In its progress, haematological findings are non-specific and vary in respect to severity. In this paper, we present a case of brucellosis with acute hepatitis and bicytopenia without anaemia. A 19-year-old man presented with a 2-week history of fever, sweating, low back and leg pain, lassitude, loss appetite, nausea and vomiting. He gave a history of raw milk ingestion and animal contact. Physical examination showed signs of icteric skin and sclera, tenderness in the right hypochondriac region and hepatosplenomegaly. On admission to hospital, laboratory tests showed WBC 3500/mmc (polymorphs 63% and lymphocytes 33%), haemoglobin 13.8 g/dL, platelet 89000/mmc, erythrocyte sedimentation rate 19 mm/h, and C-reactive protein 21.7 mg/dL (N&lt;0.8 mg/dL). Biochemical tests were as follows: AST 771 U/L, ALT 471 U/L, ALP 355 U/L, GGT 432 U/L, total bilirubin 2.61 mg/dL, direct bilirubin 1.45 mg/dL and albumin 3.7 g/dL. Viral hepatitis markers were found to be negative (HBsAg, anti-HBc total, anti-HBc IgM, anti-HAV IgM, and anti-HCV). Blood culture grew Brucella melitensis. Leukopenia and thrombocytopenia returned to normal levels at the 7th and 14th day of his admission, respectively. Liver function tests improved at the 28th day. Treatment of the brucellosis was performed with antibiotics (tetracycline 500 mg orally four times daily for 6 weeks and streptomycin 1 g IM once daily for 21 days). Finally, a case of brucellosis with acute hepatitis and bicytopenia was treated with a successful outcome. In conclusion, we suggest that due consideration be taken of bicytopenia/pancytopenia and acute hepatitis in brucellosis cases in Turkey, an endemic region. "}]}}}}
{"correct_option": 5, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": true, "char_ranges": [[179, 609]], "word_ranges": [[33, 101]], "text": "the clinical picture points to a parapneumonic pleural effusion, the macroscopic characteristics of the fluid seem so and the analysis of it shows us that the effusion is also complicated, almost on the verge of being an empyema (very high LDH, cellularity with predominance of PMN, glucose consumption). In addition, with this pH (and knowing that the effusion is already loculated), the endothoracic tube is more than indicated."}}, "full_answer": "In this question there should not be much doubt either (by the way, I think it is more of Pneumology than mine, but well, with this fever my friend Emilienko has deviated), since the clinical picture points to a parapneumonic pleural effusion, the macroscopic characteristics of the fluid seem so and the analysis of it shows us that the effusion is also complicated, almost on the verge of being an empyema (very high LDH, cellularity with predominance of PMN, glucose consumption). In addition, with this pH (and knowing that the effusion is already loculated), the endothoracic tube is more than indicated.", "full_answer_no_ref": "In this question there should not be much doubt either (by the way, I think it is more of Pneumology than mine, but well, with this fever my friend Emilienko has deviated), since the clinical picture points to a parapneumonic pleural effusion, the macroscopic characteristics of the fluid seem so and the analysis of it shows us that the effusion is also complicated, almost on the verge of being an empyema (very high LDH, cellularity with predominance of PMN, glucose consumption). In addition, with this pH (and knowing that the effusion is already loculated), the endothoracic tube is more than indicated.", "full_question": "A 64-year-old male presents with fever, cough, dyspnea and right pleuritic pain of 1 week's evolution. Chest X-ray shows a loculated right pleural effusion occupying two thirds of the hemithorax. During thoracentesis, a yellowish fluid is drawn and analysis shows: leukocytes 15,000/uL, 92% neutrophils, glucose 30 mg/dL, pH 7, lactate dehydrogenase 3500 U/L, adenosine deaminase 45 U/L and absence of germs on GRAM stain. What is the most appropriate next course of action in this patient?", "id": 117, "lang": "en", "options": {"1": "Intravenous antibiotic therapy.", "2": "Intravenous antibiotic therapy and repeat diagnostic thoracentesis in 24 hours.", "3": "Intravenous antibiotic therapy and repeat diagnostic thoracentesis if there is no improvement in 48 hours.", "4": "Intravenous antibiotic therapy and evacuative (therapeutic) thoracentesis if any germ is isolated in the pleural fluid culture.", "5": "Intravenous antibiotic therapy and placement of a chest tube or catheter to drain all pleural fluid."}, "question_id_specific": 112, "type": "INFECTOLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0320_9651", "title": "Management of parapneumonic effusions.", "score": 0.019075302025615408, "content": "When a patient with a parapneumonic pleural effusion is first evaluated, a therapeutic thoracentesis should be performed if more than a minimal amount of pleural fluid is present. Fluid obtained at the therapeutic thoracentesis should be gram-stained and cultured and analyzed for glucose, pH, LDH, white blood cells, and differential cell count. If the fluid cannot be drained because of loculations, a chest tube should be inserted and thrombolytic agents administered. If the pleural fluid recurs after the initial therapeutic thoracentesis but the patient is doing well clinically and the initial pleural fluid glucose was greater than 60 mg/dL; the pH, greater than 7.2; the LDH, less than three times the upper normal limit for serum and the cultures are negative; he or she can be observed. If one or more of the aforementioned criteria are not met, a second therapeutic thoracentesis should be performed, with repeat diagnostic evaluations of the pleural fluid. If the fluid recurs a second time, a small chest tube should be placed if the pleural fluid glucose and pH were lower and the LDH higher on the second thoracentesis than on the first thoracentesis. Patients with loculated-parapneumonic effusions should be treated with tube thoracostomy and thrombolytic agents. If drainage is incomplete, thoracoscopy, with breakdown of adhesions and debridement of the pleural space, is indicated. If thoracoscopy is unsuccessful, then thoracotomy, with decortication, is indicated unless the patient is too debilitated."}, {"id": "pubmed23n0589_512", "title": "Explosive pleuritis.", "score": 0.017494270435446907, "content": "The objective of the present paper is to describe the clinical and computed tomography features of 'explosive pleuritis', an entity first named by Braman and Donat in 1986, and to propose a case definition. A case report of a previously healthy, 45-year-old man admitted to hospital with acute onset pleuritic chest pain is presented. The patient arrived at the emergency room at 15:00 in mild respiratory distress; the initial chest x-ray revealed a small right lower lobe effusion. The subsequent clinical course in hospital was dramatic. Within 18 h of admission, he developed severe respiratory distress with oxygen desaturation to 83% on room air and dullness of the right lung field. A repeat chest x-ray, taken the morning after admission, revealed complete opacification of the right hemithorax. A computed tomography scan of the thorax demonstrated a massive pleural effusion with compression of pulmonary tissue and mediastinal shift. Pleural fluid biochemical analysis revealed the following concentrations: glucose 3.5 mmol/L, lactate dehydrogenase 1550 U/L, protein 56.98 g/L, amylase 68 U/L and white blood cell count 600 cells/mL. The pleural fluid cultures demonstrated light growth of coagulase-negative staphylococcus and viridans streptococcus, and very light growth of Candida albicans. Cytology was negative for malignant cells. Thoracotomy was performed, which demonstrated a loculated parapneumonic effusion that required decortication. The patient responded favourably to the empirical administration of intravenous levofloxacin and ceftriaxone, and conservative surgical methods in the management of the empyema. This report also discusses the patient's rapidly progressing pleural effusion and offers a potential case definition for explosive pleuritis. Explosive pleuritis is a medical emergency defined by the rapid development of a pleural effusion involving more than 90% of the hemithorax over 24 h, which causes compression of pulmonary tissue and mediastinal shift to the contralateral side."}, {"id": "pubmed23n0642_14821", "title": "[Parapneumonic pleural effusions and empyema in adults:current practice].", "score": 0.01715686274509804, "content": "About 20% of hospitalized patients with bacterial pneumonia have an accompanying pleural effusion. Parapneumonic effusions (PPE) are associated with a considerable morbidity and mortality. The main decision in managing a patient with a PPE is whether to insert a chest tube (complicated PPE). Imaging (i.e., chest radiograph, ultrasound and computed tomography) and pleural fluid analysis (i.e., pH, glucose, lactate dehydrogenase, bacterial cults) provide essential information for patient management. Therefore, all PPEs should be aspirated for diagnostic purposes. This may require image-guidance if the effusion is small or heavily loculated. According to the current guidelines, any PPE that fulfills at least one of the following criteria should be drained: size &gt; or = 1/2 of the hemithorax, loculations, pleural fluid pH &lt; 7.20 (or alternatively pleural fluid glucose &lt; 60 mg/dl), positive pleural fluid Gram stain or culture, or purulent appearance. The key components of the treatment of complicated PPE and empyema are the use of appropriate antibiotics, provision of nutritional support, and drainage of the pleural space by one of the following methods: therapeutic thoracentesis, tube thoracostomy, intrapleural fibrinolytics, thoracoscopy with breakdown of adhesions or thoracotomy with decortication. The routine use of intrapleural fibrinolytic therapy remains controversial. (c) 2009 Elsevier España, S.L. All rights reserved."}, {"id": "pubmed23n0125_11771", "title": "Pleural effusions caused by infection.", "score": 0.016368354514605096, "content": "Diagnostic thoracentesis is imperative when pneumonia is accompanied by an effusion (parapneumonic effusion). Examination of the pleural fluid is the only way to differentiate empyema and complicated parapneumonic effusions from uncomplicated parapneumonic effusions, and this differentiation is vital in deciding whether chest tube drainage is needed. If the aspirated pleural fluid contains pus or bacteria, closed chest tube drainage and antibiotic therapy should be started promptly. The same management approach is indicated if the pleural fluid pH is less than 7.00 or the glucose level is less than 40 mg/ml, since these effusions almost invariably are complicated parapneumonic effusions that do not resolve without fluid drainage. If the pleural fluid pH is greater than 7.20 and glucose level is more than 40 mg/ml, antibiotic therapy alone will suffice. Management of parapneumonic effusions with a pH of 7.00 to 7.20 should be based on serial observations of clinical status and pleural fluid findings."}, {"id": "pubmed23n0362_11326", "title": "Management of pleural effusions.", "score": 0.016183107672469374, "content": "This review summarizes current strategies in the treatment of patients with pleural effusion. To determine whether a patient has a transudative or exudative pleural effusion, Light's criteria should be applied to measure the concentrations of protein and lactate dehydrogenase (LDH) in the pleural fluid and serum. If the effusion is transudative, therapy should be directed toward the underlying congestive heart failure, cirrhosis, or nephrosis. Consideration should be given to pleurodesis with a sclerosant if patients with recurrent transudative effusion have severe dyspnea due to their effusion. If the effusion is exudative, attempts should be made to define the etiology. The diagnosis of pleural malignancy is most easily established via pleural fluid cytology. If this is negative and the patient is suspected of having pleural malignancy, thoracoscopy is indicated. The concentrations of adenosine deaminase and gamma-interferon in pleural fluid are useful in the diagnosis of pleural tuberculosis. Patients with pneumonia and pleural effusion should undergo therapeutic thoracentesis; the pleural fluid should be Gram-stained and cultured, and the differential cell count, glucose and LDH concentration, and pH should be determined. Indicators of a poor prognosis include the presence of frank pus, a positive Gram-stain, a pleural glucose concentration of less than 2.2 mmol/L, a pH less than 7.00, the presence of pleural loculations, and an LDH concentration greater than three times the upper limit of normal in serum. If the pleural fluid cannot be completely evacuated because of loculations, intrapleural thrombolytic therapy should be considered. If thrombolytics are ineffective, thoracoscopy or thoracotomy with decortication should be performed. Dyspneic patients with malignant pleural effusions whose dyspnea is relieved with therapeutic thoracentesis should be considered for pleurodesis using a tetracycline derivative. Talc is not recommended because it induces acute respiratory distress syndrome in about 5% of patients, with an overall mortality of 1%."}, {"id": "pubmed23n0536_20279", "title": "Parapneumonic effusions and empyema.", "score": 0.015584415584415586, "content": "Parapneumonic effusions occur in 20 to 40% of patients who are hospitalized with pneumonia. The mortality rate in patients with a parapneumonic effusion is higher than that in patients with pneumonia without a parapneumonic effusion. Some of the excess mortality is due to mismanagement of the parapneumonic effusion. Characteristics of patients that indicate that an invasive procedure will be necessary for its resolution include the following: an effusion occupying more than 50% of the hemithorax or one that is loculated; a positive Gram stain or culture of the pleural fluid; and a purulent pleural fluid that has a pH below 7.20 or a glucose below 60, or has a lactic acid dehydrogenase level of more than three times the upper normal limit for serum. Patients with pneumonia and an effusion of more than minimal size should have a therapeutic thoracentesis. If the fluid cannot be removed with a therapeutic thoracentesis, a chest tube should be inserted and consideration be given to the intrapleural instillation of fibrinolytics. If the loculated effusion persists, the patient should be subjected to video-assisted thoracoscopic surgery, and if the lung cannot be expanded with this procedure, a full thoracotomy with decortication should be performed. The definitive procedure should be performed within 14 d."}, {"id": "pubmed23n0128_5352", "title": "Parapneumonic effusions and empyema.", "score": 0.015483339220534558, "content": "Nearly 50 per cent of patients with acute bacterial pneumonia have an accompanying pleural effusion (parapneumonic effusion). With appropriate antibiotic therapy, the pleural effusion will resolve along with the pneumonia in the majority of patients. However, in a small fraction, the pleural effusion will not resolve unless drainage of the pleural space is instituted. Such patients are said to have complicated parapneumonic effusions. It is important to identify patients with complicated parapneumonic effusions as early as possible, since tube drainage of the pleural space becomes increasingly difficult the longer its institution is delayed. The possibility of a complicated parapneumonic effusion should be considered in every patient with bacterial pneumonia. If both diaphragms cannot be distinctly identified throughout their length on the lateral chest radiograph, decubitus chest radiographs should be obtained. If the thickness of the fluid on the decubitus radiograph is greater than 10 mm, a diagnostic thoracentesis should be performed. Only pleural fluid analysis can identify patients with complicated parapneumonic effusions. Complicated parapneumonic effusions are characterized by low pleural fluid pH and glucose levels, a high pleural fluid LDH, and a positive Gram stain of the pleural fluid. Tube thoracostomy should be performed immediately in a patient with an acute bacterial pneumonia if the pleural fluid glucose is below 40 mg per 100 ml, the pleural fluid pH is below 7.00, or if the Gram stain of the pleural fluid is positive. Patients with pleural fluid pH above 7.20, pleural fluid LDH below 1000 IU per L, and pleural fluid glucose levels above 40 mg per 100 ml respond well to only the administration of appropriate antibiotics.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0831_10151", "title": "Repeated therapeutic thoracentesis to manage complicated parapneumonic effusions.", "score": 0.014755658980325787, "content": "In complicated parapneumonic effusion (CPPE), antibiotics and evacuation of the infected pleural fluid are mandatory. The first-line evacuation treatment is still controversial. The aim of this article is to highlight the usefulness of repeated therapeutic thoracentesis (RTT) as a first-line treatment. In the most recent study on RTT in CPPE, disposable pleural needles were used and the median number of thoracentesis was 3. The success rate was 81%, and only 4% of the patients were referred for thoracic surgery. The 1-year survival rate was 88%. On multivariate analysis, the observation of microorganisms in the pleural fluid after Gram staining and first thoracentesis volume at least 450 ml was associated with a higher risk of RTT failure. RTT is less invasive and can target different loculated pleural collections. Patients are less confined to beds between each procedure, and could even be ambulatory managed. The use of intrapleural fibrinolytics in association with DNase could most likely enhance the efficacy of RTT. RTT is efficient and well tolerated in the management of CPPE, including pleural empyema, and could be proposed as a first-line therapy for CPPE. This technique could be used in association with intrapleural fibrinolytics and DNase."}, {"id": "wiki20220301en025_92732", "title": "Pleural empyema", "score": 0.014685679094225211, "content": "There is no readily available evidence on the route of administration and duration of antibiotics in patients with pleural empyema. Experts agree that all patients should be hospitalized and treated with antibiotics intravenously. The specific antimicrobial agent should be chosen based on Gram stain and culture, or on local epidemiologic data when these are not available. Anaerobic coverage must be included in all adults, and in children if aspiration is likely. Good pleural fluid and empyema penetration has been reported in adults for penicillins, ceftriaxone, metronidazole, clindamycin, vancomycin, gentamicin and ciprofloxacin. Aminoglycosides should typically be avoided as they have poor penetration into the pleural space. There is no clear consensus on duration of intravenous and oral therapy. Switching to oral antibiotics can be considered upon clinical and objective improvement (adequate drainage and removal of chest tube, declining CRP, temperature normalization). Oral"}, {"id": "pubmed23n0627_18339", "title": "Thoracic empyema - a review based on three cases reports.", "score": 0.014099689548120041, "content": "Complicated parapneumonic effusion is one in which an invasive procedure is necessary for its resolution and empyema means pus in the pleural space. An early diagnosis and therapy of these conditions results in less morbidity and mortality. CT of the chest is important to study complex pleural effusions. Loculated effusions, those occupying more than 50% of the thorax, or which show positive Gram stain or bacterial culture, or a purulent effusion with a pH below 7.20, with a glucose level below 60 mg/dl or a LDH level more than three times the upper limit of normal for serum, are indications for an invasive procedure. These characteristics result from the evolution of a not well treated parapneumonic effusion, through the three stages: (1) exsudative; (2) fibrinopurulent; (3) fibrotic. Depending on the stage therapeutic methods vary from therapeutic thoracentesis, insertion of a chest tube with or without instillation of fibrinolytics, video-assisted thoracoscopic surgery, and lung decortication. A review of all these aspects are done based on a series of three cases reports with very different clinical presentation: one patient with empyema by Streptococcus pyogenes and that died rapidly due to massive hemoptysis; a patient with empyema due to acute pneumonia developing during an airflight; a patient with empyema and bacteraemia by Streptococcus pneumonia leading to the diagnosis of an unknown HIV infection."}, {"id": "wiki20220301en002_144492", "title": "Pneumonia", "score": 0.013810166441745389, "content": "Pleural effusion, empyema, and abscess In pneumonia, a collection of fluid may form in the space that surrounds the lung. Occasionally, microorganisms will infect this fluid, causing an empyema. To distinguish an empyema from the more common simple parapneumonic effusion, the fluid may be collected with a needle (thoracentesis), and examined. If this shows evidence of empyema, complete drainage of the fluid is necessary, often requiring a drainage catheter. In severe cases of empyema, surgery may be needed. If the infected fluid is not drained, the infection may persist, because antibiotics do not penetrate well into the pleural cavity. If the fluid is sterile, it must be drained only if it is causing symptoms or remains unresolved."}, {"id": "wiki20220301en015_101880", "title": "Pleural effusion", "score": 0.013730610952833176, "content": "Thoracentesis Once a pleural effusion is diagnosed, its cause must be determined. Pleural fluid is drawn out of the pleural space in a process called thoracentesis, and it should be done in almost all patients who have pleural fluid that is at least 10 mm in thickness on CT, ultrasonography, or lateral decubitus X-ray and that is new or of uncertain etiology. In general, the only patients who do not require thoracentesis are those who have heart failure with symmetric pleural effusions and no chest pain or fever; in these patients, diuresis can be tried, and thoracentesis is avoided unless effusions persist for more than 3 days. In a thoracentesis, a needle is inserted through the back of the chest wall in the sixth, seventh, or eighth intercostal space on the midaxillary line, into the pleural space. The use of ultrasound to guide the procedure is now standard of care as it increases accuracy and decreases complications. After removal, the fluid may then be evaluated for:"}, {"id": "pubmed23n0287_18501", "title": "Serial pleural fluid analysis in a new experimental model of empyema.", "score": 0.013623407109322602, "content": "Prior attempts to create an animal model of empyema by direct inoculation of bacteria alone into the pleural space have been unsuccessful. The animals either died of overwhelming sepsis or cleared the infection from the pleural space without development of an empyema. We hypothesized that injection of bacteria with a nutrient agar into the pleural space would allow the bacteria to remain in the pleural space for an extended time period, permitting an empyema to develop. The bacterium Pasteurella multocida in brain heart infusion (BHI) agar was injected into the right hemithorax of 12 New Zealand white male rabbits. Our preliminary studies showed that the animals died in less than 7 days if they were not given parenteral antibiotics. For this reason, the rabbits were given penicillin, 200,000 U, IM, every 24 h starting 24 h after bacterial injection. Pleural fluid was sampled by thoracentesis at 12, 24, 48, 72, and 96 h after bacterial injection. Pleural fluid pH, glucose, lactate dehydrogenase (LDH), leukocyte count, and Gram's stain and culture (in one half of the animals) were obtained at each time point. Pleural biopsy specimens were obtained at autopsy after 96 h. The mean pleural fluid pH reached a nadir of 7.01 at 24 h and remained less than 7.1 throughout the experiment. The mean pleural fluid glucose level reached a nadir of 10 mg/dL at 24 h. The mean pleural fluid LDH peaked at 21,000 IU/L at 24 h and the mean pleural fluid leukocyte count peaked at 12 h with a value of 67,000 cells per cubic millimeter. Gram's stains revealed organisms and cultures were positive for growth in all animals at 12 and 24 h. Some animals had positive Gram's stains and growth on cultures up to 72 h after bacterial injection. At autopsy, all rabbits injected with bacteria had gross pus in the right pleural space and had developed a thick pleural peel. Microscopic specimens of the pleura revealed large numbers of leukocytes (primarily polymorphonuclear lymphocytes) with invasion of the adjacent lung and chest wall. In conclusion, this model more closely mimics the empyema that occurs in humans, relative to previous animal models. This model appears appropriate for additional randomized studies in which different methods for the treatment of empyema can be evaluated."}, {"id": "InternalMed_Harrison_20553", "title": "InternalMed_Harrison", "score": 0.012713706172124022, "content": "2. Pleural fluid pH <7.20 3. Pleural fluid glucose <3.3 mmol/L (<60 mg/dL) 4. Positive Gram stain or culture of the pleural fluid 5. Presence of gross pus in the pleural space If the fluid recurs after the initial therapeutic thoracentesis and if any of these characteristics are present, a repeat thoracentesis should be performed. If the fluid cannot be completely removed with the therapeutic thoracentesis, consideration should be given to inserting a chest tube and instilling the combination of a fibrinolytic agent (e.g., tissue plasminogen activator, 10 mg) and deoxyribonuclease (5 mg) or performing a thoracoscopy with the breakdown of adhesions. Decortication should be considered when these measures are ineffective."}, {"id": "article-27359_16", "title": "Thoracic Empyema -- Evaluation -- Thoracocentesis", "score": 0.012618675639947122, "content": "The recommendation is for diagnostic fluid sampling via thoracentesis in all patients with pleural effusions with greater than 2 cm depth on lateral decubitus film or computed tomography, associated with a pneumonic illness, recent chest trauma, surgery or features of ongoing sepsis. [12] [13] Frank pus in the pleural space invariably necessitates surgical drainage. However, if there is uncertainty whether a turbid fluid is infected, a pH less than 7.2 measured via a blood gas analyzer warrants an invasive procedure for drainage. [14] Polymorphonucleocyte predominance, low glucose, and LDH over 1000 on biochemical analysis of pleural fluid support the diagnosis of empyema. Furthermore, fluid culture data should be used to guide appropriate antimicrobial therapy. Research shows that culture yield can be increased significantly if the pleural fluid gets injected into blood culture bottles immediately after aspiration. [15]"}, {"id": "wiki20220301en167_881", "title": "Parapneumonic effusion", "score": 0.011952572193536048, "content": "A parapneumonic effusion is a type of pleural effusion that arises as a result of a pneumonia, lung abscess, or bronchiectasis. There are three types of parapneumonic effusions: uncomplicated effusions, complicated effusions, and empyema. Uncomplicated effusions generally respond well to appropriate antibiotic treatment. Diagnosis The criteria for a complicated parapneumonic effusion include Gram stain–positive or culture-positive pleural fluid, pleural fluid pH <7.20, and pleural fluid LDH that is greater than three times the upper limit of normal of serum LDH. Diagnostic techniques available include plain film chest x-ray, computed tomography (CT), and ultrasound. Ultrasound can be useful in differentiating between empyema and other transudative and exudative effusions due in part to relative echogenicity of different organs such as the liver (often isoechogenic with empyema)."}, {"id": "InternalMed_Harrison_20552", "title": "InternalMed_Harrison", "score": 0.011676470588235295, "content": "The possibility of a parapneumonic effusion should be considered whenever a patient with bacterial pneumonia is initially evaluated. The presence of free pleural fluid can be demonstrated with a lateral decubitus radiograph, computed tomography (CT) of the chest, or ultrasound. If the free fluid separates the lung from the chest wall by >10 mm, a therapeutic thoracentesis should be performed. Factors indicating the likely need for a procedure more invasive than a thoracentesis (in increasing order of importance) include the following: 1. 2. Pleural fluid pH <7.20 3. Pleural fluid glucose <3.3 mmol/L (<60 mg/dL) 4. Positive Gram stain or culture of the pleural fluid 5. Presence of gross pus in the pleural space"}, {"id": "wiki20220301en025_92730", "title": "Pleural empyema", "score": 0.011470655326359696, "content": "Treatment Pleural fluid drainage Proven empyema (as defined by the \"golden\" criteria mentioned earlier) is an indication for prompt chest tube drainage. This has been shown to improve resolution of the infection and shorten hospital admission. Data from a meta-analysis has shown that a pleural fluid pH of <7.2 is the most powerful indicator to predict the need for chest tube drainage in patients with non-purulent, culture negative fluid. Because of the viscous, lumpy nature of infected pleural fluid, in combination with possible septation and loculation, it has been proposed that intrapleural fibrinolytic or mucolytic therapy might improve drainage and therefore might have a positive effect on the clinical outcome. Intrapleural fibrinolysis with urokinase decreased the need for surgery but there is a trend to increased serious side effects."}, {"id": "wiki20220301en031_47978", "title": "Asplenia", "score": 0.011431501477951378, "content": "In an emergency room or hospital setting, appropriate evaluation and treatment for an asplenic febrile patient should include a complete blood count with differential, blood culture with Gram stain, arterial blood gas analysis, chest x-ray, and consideration for lumbar puncture with CSF studies. None of these evaluations should delay the initiation of appropriate broad-spectrum intravenous antibiotics. The Surviving Sepsis Campaign guidelines state that antibiotics should be administered to a patient suspected of sepsis within 1 hour of presentation. Delay in starting antibiotics for any reason is associated with a poor outcome."}, {"id": "article-20929_17", "title": "Pleural Effusion -- Evaluation", "score": 0.01141233805990253, "content": "The presence of organisms by gram stain or culture leads to a diagnosis of empyema and necessitates a chest tube for drainage of pus. Cytology is necessary for determining the presence of malignant cells in the pleural fluid. The sensitivity of pleural fluid cytology in the presence of malignant effusion in the first thoracentesis is around 60%, and the yield increases with further attempts, approaching 95% by three samples on different days.  However, if a malignant effusion is strongly suspected and cytology is negative, then medical thoracoscopy with pleural biopsy can be performed after two to three thoracenteses to obtain a diagnosis."}, {"id": "article-26643_20", "title": "Parapneumonic Pleural Effusions and Empyema Thoracis -- Treatment / Management", "score": 0.011259285254723052, "content": "Treatment of parapneumonic effusion includes appropriate antibiotic therapy together with drainage of pleural fluid as indicated. Parapneumonic effusion is classified into 4 groups based on risk for the poor outcome: Category 1 (very low risk): The effusion is small (less than 10-mm thickness on decubitus) and free-flowing. No thoracentesis is indicated. Category 2 (low risk): The effusion is small to moderate (equal to 10 mm and less than half the hemithorax) and free-flowing with negative culture and Gram stain regardless of the prior use of antibiotics and pH equal to 7.20. Category 3 (moderate risk), in which one of the following criteria is present: the fluid equal to half the hemithorax, loculated effusion, thicken pleura on contrast-enhanced CT scan, positive Gram stain or culture or pH less than 7.20. Category 4 (high risk): This is when pleural fluid is in the form of pure pus."}, {"id": "wiki20220301en063_55695", "title": "Thoracentesis", "score": 0.01108047385620915, "content": "Tension pneumothorax is a medical emergency that requires emergent needle decompression before a chest tube is placed. Indications This procedure is indicated when unexplained fluid accumulates in the chest cavity outside the lung. In more than 90% of cases analysis of pleural fluid yields clinically useful information. If a large amount of fluid is present, then this procedure can also be used therapeutically to remove that fluid and improve patient comfort and lung function. The most common causes of pleural effusions are cancer, congestive heart failure, pneumonia, and recent surgery. In countries where tuberculosis is common, this is also a common cause of pleural effusions."}, {"id": "wiki20220301en063_55702", "title": "Thoracentesis", "score": 0.010934744268077601, "content": "Cultures and stains If the effusion is caused by infection, microbiological culture may yield the infectious organism responsible for the infection, sometimes before other cultures (e.g. blood cultures and sputum cultures) become positive. A Gram stain may give a rough indication of the causative organism. A Ziehl–Neelsen stain may identify tuberculosis or other mycobacterial diseases. Cytology Cytology is an important tool in identifying effusions due to malignancy. The most common causes for pleural fluid are lung cancer, metastasis from elsewhere and pleural mesothelioma. The latter often presents with an effusion. Normal cytology results do not reliably rule out malignancy, but make the diagnosis more unlikely. References External links A photo gallery of thoracentesis showing the procedure step-by-step. V. Dimov, B. Altaqi, Clinical Notes, 2005. A free PDA version. Medical tests Respiratory system procedures Veterinary diagnosis Interventional radiology 1850 introductions"}, {"id": "Surgery_Schwartz_4945", "title": "Surgery_Schwartz", "score": 0.010928553647000249, "content": "can be avoided in patients with small effusions associated with resolving pneumonia. These patients typically present with cough, fever, leukocytosis, and uni-lateral infiltrate, and the effusion is usually a result of a reactive, parapneumonic process. If the effusion is small and the patient responds to antibiotics, a diagnostic thoracentesis may be unneces-sary. If the effusion is large and compromising respiratory efforts, or if the patient has a persistent white blood cell count despite improving signs of pneumonia, an empyema of the pleural space must be considered. In these patients, early and aggressive drainage with chest tubes is required, possibly with surgical intervention.Once the decision is made to access a pleural effusion, the next step is to determine if a sample of the fluid or complete drainage of the pleural space is desired. This step is influenced by the clinical history, the type and amount of fluid present, the Brunicardi_Ch19_p0661-p0750.indd 73601/03/19"}, {"id": "article-27331_19", "title": "Pleurisy -- Treatment / Management", "score": 0.010897377318833957, "content": "A category 3 effusion may also be defined as a pH of <7.20 or a positive gram stain/culture (empyema). [13] Category 4 effusions are defined by purulent material on thoracentesis. [13] Category 3 and 4 effusions are recommended to have thoracostomy drainage. [13] Empyema, or category 4 effusions, may require surgical evacuation and decortication. Due to the high mortality of the categories 3 and 4 pleural effusions (30%), additional methods for the complete drainage of the infected pleural fluid include intrapleural fibrinolytic followed by drainage and/or surgical decortication. [14] The MIST 2 trial compared intrapleural placebo, intrapleural DNase, intrapleural tPA, and combination tPA/DNAse in patients with category 3 or 4 effusions."}, {"id": "wiki20220301en025_92727", "title": "Pleural empyema", "score": 0.010844010814623252, "content": "The most often used \"golden\" criteria for empyema are pleural effusion with macroscopic presence of pus, a positive Gram stain or culture of pleural fluid, or a pleural fluid pH under 7.2 with normal peripheral blood pH. Clinical guidelines for adult patients therefore advocate diagnostic pleural fluid aspiration in patients with pleural effusion in association with sepsis or pneumonic illness. Because pleural effusion in the pediatric population is almost always parapneumonic and the need for chest tube drainage can be made on clinical grounds, British guidelines for the management of pleural infection in children do not recommend diagnostic pleural fluid sampling."}, {"id": "wiki20220301en063_55698", "title": "Thoracentesis", "score": 0.010653293575494714, "content": "The use of ultrasound for needle guidance can minimize the complication rate. Follow-up imaging While chest X-ray has traditionally been performed to assess for pneumothorax following the procedure, it may no longer be necessary to do so in asymptomatic, non-ventilated persons given the widespread use of ultrasound to guide this procedure. Interpretation of pleural fluid analysis Several diagnostic tools are available to determine the etiology of pleural fluid. Transudate versus exudate First the fluid is either transudate or exudate. A transudate is defined as pleural fluid to serum total protein ratio of less than 0.5, pleural fluid to serum LDH ratio > 0.6, and absolute pleural fluid LDH > 200 IU or > 2/3 of the normal."}, {"id": "article-27331_18", "title": "Pleurisy -- Treatment / Management", "score": 0.010493827160493827, "content": "Category 2 pleural effusions are >10 mm but less than half of the hemithorax. [13] Category 2 to 4 pleural effusions are recommended to have diagnostic thoracentesis performed, and empyema ruled out (must have negative culture, gram stain, and a pH>7.20).  A category 3 effusion involves more than half of the hemithorax, loculations noted within the pleural space, or thickening of the parietal pleura. [13]"}, {"id": "InternalMed_Harrison_10159", "title": "InternalMed_Harrison", "score": 0.010483834603417955, "content": "P. aeruginosa, or (rarely) S. pneumoniae. Aspiration pneumonia is typically a polymicrobial infection involving both aerobes and anaerobes. A significant pleural effusion should be tapped for both diagnostic and therapeutic purposes. If the fluid has a pH of <7, a glucose level of <2. 2 mmol/L, and a lactate dehydrogenase concentration of >1000 U/L or if bacteria are seen or cultured, then it should be completely drained; a chest tube is often required and video-assisted thoracoscopy may be needed for late treatment or difficult cases."}, {"id": "wiki20220301en014_89505", "title": "Pleurisy", "score": 0.010327883498615208, "content": "Computed tomography (CT) scan A CT scan provides a computer-generated picture of the lungs that can show pockets of fluid. It also may show signs of pneumonia, a lung abscess, or a tumor. Magnetic resonance imaging (MRI) Magnetic resonance imaging (MRI), also called nuclear magnetic resonance (NMR) scanning, uses powerful magnets to show pleural effusions and tumors. Arterial blood gas In arterial blood-gas sampling, a small amount of blood is taken from an artery, usually in the wrist. The blood is then checked for oxygen and carbon-dioxide levels. This test shows how well the lungs are taking in oxygen. Thoracentesis Once the presence of an excess fluid in the pleural cavity, or pleural effusion, is suspected and location of fluid is confirmed, a sample of fluid can be removed for testing. The procedure to remove fluid in the chest is called a diagnostic thoracentesis. The doctor inserts a small needle or a thin, hollow, plastic tube in the chest wall and withdraws fluid."}, {"id": "article-26643_3", "title": "Parapneumonic Pleural Effusions and Empyema Thoracis -- Introduction", "score": 0.00982369639922307, "content": "Uncomplicated parapneumonic effusions, which are exudative,  neutrophilic effusion. Gram stain and culture are negative, glucose level greater than 60 mg/dl, pH above 7.20. Complicated parapneumonic effusions, resulting from a bacterial introduction into the pleura. In this type of parapneumonic effusion, there is a decreased glucose level, pleural fluid is below 7.20. Cultures of fluid from complicated parapneumonic effusions are negative and rapid bacterial clearance from the pleural space, or low bacterial count may explain this. The fluid termed as complicated because it necessitates drainage for resolution. Empyema thoracis in which there is frank pus in the pleural space, or there is evidence of bacterial infection of the pleural fluid by Gram stain or a positive culture. [2] [3]"}, {"id": "pubmed23n0327_9488", "title": "[Purulent pleurisy and empyema. With the exception of pleural tuberculosis].", "score": 0.00980392156862745, "content": "Purulent collections in the pleural cavity usually occur as complications of pneumonia in immunodeficient or socially underprivileged patients. The key to diagnosis, pleural aspiration is indicated in case of sufficiently abundant collections, especially in patients with fever. Exploratory puncture is a therapeutic emergency, allowing optimal antibiotic therapy when a causal germ is isolated and drainage of the purulent collection. If there is the slightest doubt, imaging techniques should be used to guide the puncture. Drainage is essential and is indicated whenever the aspiration fluid is purulent, contains, germs or the chemistry suggests major bacterial colonisation (acid pH, low glucose, high lactic acid dehydrogenase). Local injections of fibrinolytic agents improve drainage. Complete recovery without sequellae is usually achieved. Physical therapy, provided early and for a prolonged period, helps improve the prognosis. Early care reduces the risk of recurrence of this potentially severe condition."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[144, 368]], "word_ranges": [[24, 66]], "text": "Option 2 is not very credible, in fact, as much as patients with CAP may have associated diarrhea, it is unlikely that a bacterium is sensitive to an antibiotic at the beginning of the picture and stops being so after 3 days."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[14, 142]], "word_ranges": [[3, 24]], "text": "given the antibiotic treatment given and the severity of the diarrhea, it seems that we are dealing with a Clostridium infection."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "In this case, given the antibiotic treatment given and the severity of the diarrhea, it seems that we are dealing with a Clostridium infection. Option 2 is not very credible, in fact, as much as patients with CAP may have associated diarrhea, it is unlikely that a bacterium is sensitive to an antibiotic at the beginning of the picture and stops being so after 3 days.", "full_answer_no_ref": "In this case, given the antibiotic treatment given and the severity of the diarrhea, it seems that we are dealing with a Clostridium infection. Option 2 is [HIDDEN], in fact, as much as patients with CAP may have associated diarrhea, it is unlikely that a bacterium is sensitive to an antibiotic at the beginning of the picture and stops being so after 3 days.", "full_question": "A 52-year-old patient is admitted to the Hospital for severe pneumonia. With appropriate antibiotic treatment, the patient's respiratory symptoms improved. After 4 days of stay in the ward, his evolution is complicated by the appearance of a severe diarrhea. What is the most frequent microorganism responsible for this condition?", "id": 27, "lang": "en", "options": {"1": "Salmonella enterica.", "2": "The bacterium itself causing pneumonia that has become resistant to the antibiotic.", "3": "Campylobacter jejuni.", "4": "Clostridium difficile.", "5": "Yersinia enterocolitica."}, "question_id_specific": 203, "type": "INFECTIOUS", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0264_14401", "title": "[Etiology, diagnosis and course of infectious diarrhea in the Liestal canton hospital (5-year retrospective study)].", "score": 0.015564437984496124, "content": "Between 1987 and 1991 219 patients (1.3% of all hospitalized patients) with acute infectious diarrhea were investigated retrospectively. 52% of the patients were hospitalized and 48% were outpatients. In 55% the inducing diarrhea microorganism could be identified. The most frequently detected pathogens were endemic Salmonella sp. and Campylobacter jejuni (65%). Imported diarrheas (Shigella sp. and parasites) were rare, as only 15% of the patients had a history of travel. All Clostridium difficile infections were associated with antibiotic treatment. The stool examinations for bacteria were positive in 92/160 patients (57%). Stool examinations for occult blood or fecal leukocytes were highly useful in detecting the infectious agent. In 70% of the patients with positive occult blood test or 81% of the patients with fecal leukocytes, an infectious agent in the stool was found. All patients recovered with few complications. 31% of the patients were treated by antibiotics because of septic disease or pathogenic parasites."}, {"id": "pubmed23n0416_13711", "title": "A 5-year study of the bacterial pathogens associated with acute diarrhoea on the island of Crete, Greece, and their resistance to antibiotics.", "score": 0.015079865489701555, "content": "During a 5-year period (1995-1999) a total of 7090 stool samples obtained from patients with acute diarrhoea, mostly community-acquired, were examined for bacterial pathogens, in the Greek island of Crete. One or more enteric pathogens were isolated from 987 patients (14%). Salmonella enterica were the most commonly isolated bacteria (6%), followed by Campylobacter spp. (4.2%), and enteropathogenic Escherichia coli (EPEC) (1.8%). Yersinia enterocolitica (0.6%), Shigella spp. (0.3%), and Aeromonas hydrophila (0.04%), were less frequently isolated. Clostridium difficile was isolated from 65 out of 451 diarrhoeal specimens examined (14.4%). Toxin B was detected in all cases. No verotoxigenic E. coli strains were identified. Resistance to ampicillin was observed in 31.5% of the Salmonella, 58.3% of the Shigella and 31.5% of the EPEC isolates. Resistance to trimethoprim-sulfamethoxazole was observed in 4.4% of the Salmonella, 30.5% of the Shigella, and 18.5% of the EPEC isolates. High percentages of resistance to quinolones (44.5% to norfloxacin, and 40.5% to ciprofloxacin), were found among Campylobacter isolates, while resistance to erythromycin was observed in 14.9% of them. With the present study we continue the surveillance of bacterial pathogens associated with diarrhoeal disease on the island of Crete."}, {"id": "wiki20220301en029_14012", "title": "Gastroenteritis", "score": 0.014391077531930203, "content": "Toxigenic Clostridium difficile is an important cause of diarrhea that occurs more often in the elderly. Infants can carry these bacteria without developing symptoms. It is a common cause of diarrhea in those who are hospitalized and is frequently associated with antibiotic use. Staphylococcus aureus infectious diarrhea may also occur in those who have used antibiotics. Acute \"traveler's diarrhea\" is usually a type of bacterial gastroenteritis, while the persistent form is usually parasitic. Acid-suppressing medication appears to increase the risk of significant infection after exposure to a number of organisms, including Clostridium difficile, Salmonella, and Campylobacter species. The risk is greater in those taking proton pump inhibitors than with H2 antagonists."}, {"id": "pubmed23n0705_14286", "title": "Empiric antimicrobial therapy and infectious diarrhea. Do we need local guidelines?", "score": 0.013825324180015256, "content": "In the management of acute diarrhea, administration of antibiotics may be indicated. Appropriate antimicrobial therapy can shorten illness, reduce morbidity and can be life-saving in invasive infections. Emergence of microbial strains resistant to commonly used antibiotics means that treatment failures may become common. Because of changing patterns of resistance, knowledge of recent local patterns of susceptibility can guide the initial choice of antibiotics. A retrospective study was conducted to investigate the epidemiology of infective gastroenteritis in patients over 14years old in the region of Chania (Crete). We reviewed all positive stool cultures and susceptibilities of the pathogens recovered from patients with symptoms of acute diarrhea, from 2003 until October 2010. Out of 194 positive stool cultures, we observed 139 cases of Salmonella enterica and 48 cases of Campylobacter jejuni. During the last 3years of observation there was an increased incidence of C. jejuni, especially after the tap water outbreak that occurred in our region in 2009. In the vast majority of acute diarrhea in adults, antibiotics are of no benefit and overprescription may confer to side effects, costs and emergence of resistance. Antibiotics are initiated in cases of febrile diarrheas especially those believed to have moderate to severe disease. Considering the increased incidence of C. jejuni and the resistance of the great majority of isolated strains to quinolones as well as the sensitivity of Salmonella spp. to azithromycin, administration of azithromycin empirically for acute diarrhea, when indicated, could be appropriate in our region."}, {"id": "pubmed23n0360_4843", "title": "[Pneumonia caused by Haemophilus influenzae. Study in a series of 58 patients].", "score": 0.013352625051674245, "content": "Haemophilus influenzae tends to form part of the usual respiratory flora in adults, especially if they have a chronic underlying disease or are smokers. Pneumonia due to H. influenzae is frequently involved in respiratory infections and its level of resistance to ampicillin has remained stable over the last five years. Most of the literature on the subject was published more than 10 years ago. In this study, we describe the clinical features and evolution of 58 adult patients admitted to hospital for pneumonia due to H. influenzae over a 2-year period, with this group accounting for 6.5% of all the patients admitted with pneumonia during this time period. The etiological diagnosis was made using a good quality sputum sample. Forty patients (69%) were male. The mean age (+/- SD) of the group was 67 (+/-16.8) years and all the patients had at least one underlying disease. The mean duration of the symptoms was 6.7 days. All patients presented an increase in the quantity or purulence of the sputum. On admittance, respiratory failure was present in 52 patients (90%). Gram-negative coccus-bacilli were observed in the direct sputum test and H. influenzae grew in the culture. In two cases, H. influenzae was recovered from the blood culture and in one from bronchial aspiration obtained through bronchoscopy. Another pathogen was identified in 28 patients (48%). In 21 it was another pyogenic bacteria (15 S. pneumoniae, 4 M. catharralis, 1 K. pneumoniae, 1 E. coli), an atypical microorganism in 5 (3 C. pneumoniae, 2 C. burnetii) and a respiratory virus in 2 (syncytial and influenza A). Atypical bacteria and respiratory virus were detected using serological techniques. The radiographic infiltrate was unilobar in 54 of the 58 patients and all showed an alveolar pattern. The empirical treatment included the administration of a third generation cephalosporin (or a fluoroquinolone in patients allergic to penicillin). The evolution was favorable in all the cases in which H. influenzae was the only pathogen or was accompanied by an atypical microorganism or a respiratory virus. Four patients with mixed bacterial pneumonia died (2 S. pneumoniae, 1 E. coli and 1 M. catharralis). The study indicates that pneumoniae due to H. influenzae affects a population with an underlying disease, preferably pulmonary, that it has a longer clinical period than that for pneumococcal pneumonia, that it is slightly bacteremic and, that, usually, it evolves benignly with a low mortality."}, {"id": "wiki20220301en033_46336", "title": "Salmonellosis", "score": 0.013071895424836603, "content": "Signs and symptoms Enteritis After a short incubation period of a few hours to one day, the bacteria multiply in the small intestine, causing an intestinal inflammation (enteritis). Most people with salmonellosis develop diarrhea, fever, vomiting, and abdominal cramps 12 to 72 hours after infection. Diarrhea is often watery and non-bloody but may be mucoid and bloody. In most cases, the illness lasts four to seven days, and does not require treatment. In some cases, though, the diarrhea may be so severe that the patient becomes dangerously dehydrated and must be hospitalized. At the hospital, the patient may receive fluids intravenously to treat the dehydration, and may be given medications to provide symptomatic relief, such as fever reduction. In severe cases, the Salmonella infection may spread from the intestines to the blood stream, and then to other body sites, and can cause death, unless the person is treated promptly with antibiotics."}, {"id": "pubmed23n1133_15235", "title": "[Salmonella Bacteremia Accompanying COVID-19: The First Salmonella Co-Infection in the World Unrelated to Pakistan].", "score": 0.013032990974167444, "content": "Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection emerged in China at the end of 2019 and caused coronavirus disease 2019 (COVID-19). The lymphopenia seen in COVID-19 increases the incidence of susceptibility to other microorganisms and may cause co-infections. As the signs and symptoms of the diseases overlap with other infectious diseases and due to the intensity in health services, the diagnosis of co-infections becomes difficult and the treatment may be delayed. Therefore, infections accompanying COVID-19 cause an increase in morbidity and mortality.The isolation and quarantine measures taken during the COVID-19 process have reduced the number of infections transmitted from person to person. However, there was no significant decrease in diseases transmitted by food, such as salmonellosis. During the pandemic, salmonellosis continued to be a problem, especially in endemic areas such as Pakistan, and an increase in Salmonella infections associated with backyard poultry has been reported in countries such as the United States. A co-infection of COVID-19 and enteric fever associated with travel to Pakistan was reported for the first time in the literature in February 2021. In this case report, the first co-infection of COVID-19 and Salmonella in our country was presented. A 56-yearold male patient with no known systemic disease was admitted to the hospital with fever, shortness of breath, weakness and myalgia lasting for three days. SARS-CoV-2 polymerase chain reaction test was positive. The patient has been hospitalized and favipiravir, moxifloxacin, and methylprednisolone were started. Blood cultures were taken from the patient whose clinical picture worsened and fever continued despite of the medical treatment. Salmonella enterica spp. enterica was isolated and ceftriaxone treatment was started. The patient's anamnesis was deepened, but no diarrhea, abdominal pain, suspicious food consumption, travel history were determined. From the second day of the ceftriaxone treatment, the patient's fever decreased and no growth was detected in the control blood cultures. Ceftriaxone treatment was completed in 14 days and the patient was discharged on the 28th day. Approximately 87-95% of Salmonella strains isolated in our country are S.enterica spp. enterica, and S.enterica spp. enterica was also isolated in our case. Salmonella infections most commonly present as gastroenteritis, but the risk of bacteremia increases in case of immunosuppression. Although there was no additional disease in our case, it was considered that the infection in the form of bacteremia occurred due to an immunosuppression caused by COVID-19. In this context; drawing blood cultures of patients hospitalized with the diagnosis of COVID-19 is very important in terms of detecting co-infections and superinfections, and administering appropriate antibiotic therapy at appropriate treatment times. Presentation of first case of Salmonella bacteremia and simultaneous COVID-19 infection in our country was the strong side of our report. In addition, our case is also important as being the first SARS-CoV-2 and Salmonella co-infection unrelated to Pakistan in the literature. The limitation of our case was that S.enterica spp. enterica detected in the blood culture could not be subtyped and the stool culture could not be examined. However, this does not constitute a diagnostic requirement. In addition, the patient's pre-COVID-19 Salmonella carrier status was also unknown. As a result, patients become vulnerable to other infections due to the lymphopenia seen in COVID-19. Therefore, Salmonella bacteremia can be seen with SARS-CoV-2 infection without a comorbid condition. Drawing blood cultures in hospitalized patients with the diagnosis of COVID-19 is very important in terms of detecting concomitant infections in a short time. In patients whose clinical condition does not improve and fever continues despite of treatment, blood cultures should be taken, especially in the case of an advanced immunosuppresive treatment plan, and it should always be kept in mind that secondary infections and co-infections may occur."}, {"id": "wiki20220301en550_17015", "title": "2018 American salmonella outbreak", "score": 0.01295929849066853, "content": "Treatment Treatment of salmonella infections depends on age and pre-disposition of the infected person and the severity of the symptoms. One of the side-effects from salmonella infection is dehydration, making the replacement of fluids and electrolytes the main treatment for less severe infections. In severe cases, patients are treated with antibiotics, rehydrated with intravenous (IV) fluids and hospitalized. In cases where the infection spreads from the intestines to the bloodstream, quick treatment with antibiotics is required to prevent the risk of death. Possible complications include resistances to certain antibiotics in the salmonella streaks, that might require treatment with different antibiotics than commonly prescribed antibiotics. In this case, whole-genome sequencing (WGS) analysis identified no antibiotic resistance in 112 of 127 isolates. Prevention and Structural Consequences"}, {"id": "pubmed23n0302_3384", "title": "Acute diarrhea in under five-year-old children admitted to King Mongkut Prachomklao Hospital, Phetchaburi province.", "score": 0.012496658647420476, "content": "A prospective epidemiological and clinical study of acute diarrhea in children under 5 years old was done at King Mongkut Prachomklao Hospital in order to provide baseline data for health officers to make a strategic plan to reduce the diarrheal mortality and morbidity, which is one of the mid-decade goals for children. There were 105 cases of acute diarrhea patients admitted to the Pediatric ward between May 1995 and April 1996. Seventy-six per cent of them were in the younger age group (&gt; 1 month-2 years old) while 23.8 per cent were in the older age group (2-5 years old). Causative pathogens were identified in 64 patients (61%). Younger patients had a higher percentage of identifiable pathogens (66.7%) than older patients (44.4%). Rotavirus was the most common pathogen isolated (17.2%). The other common pathogens identified were Eschericia coli (14.1%), Campylobacter jejuni (14.1%), Shigella (12.5%), Entamoeba histolytica (7.8%) and Salmonella (3.1%). Mixed infections were reported in 31.3 per cent of these patients. Clinical presentations and stool characteristics were difficult to distinguish from most of the pathogens. However, Rotavirus was highly suspected if a younger child presented with fever, watery to loose stool with the predominant symptom of vomiting. Mucous, mucous-bloody stool gave a clue to the diagnosis of Shigella and Entamoeba histolytica. Most cases had at least mild to moderate dehydration, so oral rehydration solution (ORS) was successfully given in only 31.4 per cent of patients. Antibiotics were prescribed to 51.4 per cent of patients in this study. Healthcare personnel should emphasize to parents and caretakers about good hygienic behavior to reduce the episodes of diarrhea and the use of ORS every time when their children have diarrheal episodes to reduce the disease severity."}, {"id": "pubmed23n0360_22946", "title": "[Pneumonia due to Haemophilus influenzae.Study in a series of 58 patients]", "score": 0.012483530961791832, "content": "Haemophilus influenzae tends to form part of the usual respiratory flora in adults, especially if they have a chronic underlying disease or are smokers. Pneumonia due to H. influenzae is frequently involved in respiratory infections and its level of resistance to ampicillin has remained stable over the last five years. Most of the literature on the subject was published more than 10 years ago. In this study, we describe the clinical features and evolution of 58 adult patients admitted to hospital for pneumonia due to H. influenzae over a 2-year period, with this group accounting for 6.5&amp;#37; of all the patients admitted with pneumonia during this time period. The etiological diagnosis was made using a good quality sputum sample. Forty patients (69&amp;#37;) were male. The mean age (+/- SD) of the group was 67 (+/-16.8) years and all the patients had at least one underlying disease. The mean duration of the symptoms was 6.7 days. All patients presented an increase in the quantity or purulence of the sputum. On admittance, respiratory failure was present in 52 patients (90&amp;#37;). Gram-negative coccus-bacilli were observed in the direct sputum test and H. influenzae grew in the culture. In two cases, H. influenzae was recovered from the blood culture and in one from bronchial aspiration obtained through bronchoscopy. Another pathogen was identified in 28 patients (48&amp;#37;). In 21 it was another pyogenic bacteria (15 S. pneumoniae, 4 M. catharralis, 1 K. pneumoniae, 1 E. coli), an atypical microorganism in 5 (3 C. pneumoniae, 2 C. burnetii) and a respiratory virus in 2 (syncytial and influenza A). Atypical bacteria and respiratory virus were detected using serological techniques. The radiographic infiltrate was unilobar in 54 of the 58 patients and all showed an alveolar pattern. The empirical treatment included the administration of a third generation cephalosporin (or a fluoroquinolone in patients allergic to penicillin). The evolution was favorable in all the cases in which H. influenzae was the only pathogen or was accompanied by an atypical microorganism or a respiratory virus. Four patients with mixed bacterial pneumonia died (2 S. pneumoniae, 1 E. coli and 1 M. catharralis). The study indicates that pneumoniae due to H. influenzae affects a population with an underlying disease, preferably pulmonary, that it has a longer clinical period than that for pneumococcal pneumonia, that it is slightly bacteremic and, that, usually, it evolves benignly with a low mortality."}, {"id": "pubmed23n0408_6712", "title": "[Clostridium difficile infection in a Department of Internal Medicine. A consecutive series of 45 patients].", "score": 0.01226696495152871, "content": "A retrospective study of 45 patients with Clostridium difficile infection over a 4-year period in a department of Internal Medicine. Mean age was 79 years; sex-ratio (F/M)=1.5; 38% of the patients had neurological or severe psychiatric disorders; 20% had a neoplastic disease. Ninety-three percent of cases had received one or more antibiotics before onset of diarrhea, prescribed mainly for a pulmonary infection. Amoxicillin clavulanic acid and cephalosporins were the most frequently used treatments, respectively in 48% and 40% of cases. For 25 patients (56%) Clostridium difficile-associated diarrhea was considered as a nosocomial infection, and as community-acquired diarrhea in 20 cases (44%). Treatment included isolation of the patient as soon as bacteriological diagnosis was known and specific therapy was instituted by metronidazole or vancomycin for a mean of 18 days. The addition of Saccharomyces boulardii was used in of cases. The clinical course was rapidly favorable for 80% of patients. Five patients died with complications of severe colitis in 2 cases. Mean hospital stay was 49 days (annual mean of the department=10 days). Clostridium difficile diarrhea concerns above all elderly patients with one or more underlying pathologies. Amoxicillin clavulanic acid and third-generation cephalosporins are the most frequently prescribed antibiotics in these cases and have the highest correlation with this infectious complication. This medical problem requires greater knowledge as it causes significant morbidity and increases the risk of prolonged hospital stays."}, {"id": "wiki20220301en069_40742", "title": "Community-acquired pneumonia", "score": 0.012248407939385489, "content": "Treatment CAP is treated with an antibiotic that kills the infecting microorganism; treatment also aims at managing complications. If the causative microorganism is unidentified, which is often the case, the laboratory identifies the most effective antibiotic; this may take several days. Health professionals consider a person's risk factors for various organisms when choosing an initial antibiotic. Additional consideration is given to the treatment setting; most patients are cured by oral medication, while others must be hospitalized for intravenous therapy or intensive care. Current treatment guidelines recommend a beta lactam, like amoxicillin and a macrolide, like azithromycin or clarithromycin, or a quinolone, such as levofloxacin. Doxycycline is the antibiotic of choice in the UK for atypical bacteria, due to increased clostridium difficile colitis in hospital patients linked to the increased use of clarithromycin."}, {"id": "wiki20220301en550_17014", "title": "2018 American salmonella outbreak", "score": 0.011888906935195712, "content": "Signs and Symptoms Salmonella is a bacterium that produces the symptoms of diarrhoea, fever and abdominal cramps in infected individuals 12-72 hours after exposure. Most people recover without treatment and the illness usually lasts for 4-7 days. Some people may need hospitalization due to severe symptoms and in rare cases, Salmonella can cause death unless the person is treated with antibiotics promptly. Those most likely to develop severe salmonella are children under the age of 5, adults over the age of 65 and people with compromised immune systems. Treatment"}, {"id": "wiki20220301en540_24103", "title": "List of antibiotic-resistant bacteria", "score": 0.011560029417172274, "content": "Gram positive Clostridium difficile Clostridium difficile is a nosocomial pathogen that causes diarrheal disease worldwide. Diarrhea caused by C. difficile can be life-threatening. Infections are most frequent in people who have had recent medical and/or antibiotic treatment. C. difficile infections commonly occur during hospitalization. According to a 2015 CDC report, C. difficile caused almost 500,000 infections in the United States over a year period. Associated with these infections were an estimated 15,000 deaths. The CDC estimates that C. difficile infection costs could amount to $3.8 billion over a 5-year span. C. difficile colitis is most strongly associated with fluoroquinolones, cephalosporins, carbapenems, and clindamycin. Some research suggests the overuse of antibiotics in the raising of livestock is contributing to outbreaks of bacterial infections such as C. difficile.[16]"}, {"id": "pubmed23n0291_18364", "title": "[Long-term follow-up of the etiology of diarrhea with emphasis on the role of rotaviruses in hospitalized patients at the Infectious Disease Clinic of the Medical School Hospital in Plzen].", "score": 0.009900990099009901, "content": "During 1986-1994 the etiological structure of diarrhoea in hospitalized patients at the Infectious Diseases Clinic, University Hospital Plzen was analyzed. In children under four years most frequently (in 26%) rotaviruses were involved, in older patients their ratio was lower and the decisive pathogenetic organism were salmonellae. In rotavirus infections the shortest hospitalization period was recorded. These infections were encountered all round the year with a maximum prevalence in the winter months."}, {"id": "pubmed23n0226_14537", "title": "[Unusual pathology caused by Salmonella in the Greater Hospital of Milan in the 3-year period of 1977-1979].", "score": 0.00980392156862745, "content": "At the Central Laboratory of the Medical School, University of Milano, Italy, 44 Salmonella strains were isolated from morbid materials other than stools during the three-years period 1977-79 (blood 26, urine 6, pus 3, pleural fluid 4, bile 3, upper respiratory mucus 2). In the present paper, nine cases of Salmonella infections, selected for their unusual localization a/o their peculiar clinical onset, are reviewed. From most of the clinical histories it appears that, in quite a few occasions, the episode due to Salmonella has been favoured or conditioned by a preexisting disease, therefore suggesting a nosocomial-type event. It also appears that the help from the microbiologist was often requested less than promptly, and minimal requirements for its efficacy were seldom achieved."}, {"id": "wiki20220301en022_28228", "title": "Campylobacter jejuni", "score": 0.009708737864077669, "content": "Treatment Patients with Campylobacter infection should drink plenty of fluids as long as the diarrhea lasts to maintain hydration. Patients should also get rest. If he or she cannot drink enough fluids to prevent dehydration or if the symptoms are severe, medical help is indicated. In more severe cases, certain antibiotics can be used and can shorten the duration of symptoms if given early in the illness. Moreover, maintenance of electrolyte balance, not antibiotic treatment, is the cornerstone of treatment for Campylobacter enteritis. Indeed, most patients with this infection have a self-limited illness and do not require antibiotics at all. Nevertheless, antibiotics should be used in specific clinical circumstances. These include high fevers, bloody stools, prolonged illness (symptoms that last >1 week), pregnancy, infection with HIV, and other immunocompromised states."}, {"id": "pubmed23n0391_5877", "title": "[Acute Gastroenteritis in hospitalized children. 14-Year evolution].", "score": 0.009708737864077669, "content": "To analyze the etiology and evolution of patients with acute gastroenteritis hospitalized in our pediatric department and to study the clinical and laboratory differences between acute viral and bacterial gastroenteritis. We studied the children with a diagnosis of acute gastroenteritis, aged between 0 and 14 years, who were consecutively admitted between 1987 and 2000. Differences were considered statistically significant if p &lt; 0.05. A total of 2,613 patients diagnosed with acute gastroenteritis were hospitalized (10.4 % of hospital admissions). The most common pathogens isolated were rotaviruses (46.5 %), followed by Salmonella (32.6 %) and Campylobacter (19.3 %). Hospital admissions due to Salmonella (p &lt; 0.0001), other bacteria (Escherichia coli and Shigella) (p &lt; 0.002) and adenoviruses (p &lt; 0.01) significantly decreased. Rotaviruses were the most frequently detected pathogens in winter and in children aged less than 1 year (p &lt; 0.0001). The incidence of Salmonella spp was greater in summer and in children older than 2 years (p &lt; 0.0001). The incidence of hyperthermia (rectal temperature higher than 38.5 degreeC) (p &lt; 0.0001), dehydration (p &lt; 0.0005) and fecal blood (p &lt; 0.0001) was higher in bacterial diarrheas. Erythrocyte sedimentation rate (p &lt; 0.001) and leukocyte counts were higher in bacterial gastroenteritis (p &lt; 0.01). Rotaviruses were the most frequently isolated enteropathogens. The features that best distinguished between bacterial and viral diarrhea were hyperthermia and fecal blood. Hospital admissions due to Salmonella Shigella E. coli, and adenoviruses significantly decreased."}, {"id": "wiki20220301en010_74532", "title": "Methicillin-resistant Staphylococcus aureus", "score": 0.009615384615384616, "content": "Endocarditis and bacteremia Evaluation for the replacement of a prosthetic valve is considered. Appropriate antibiotic therapy may be administered for up to six weeks. Four to six weeks of antibiotic treatment is often recommended, and is dependent upon the extent of MRSA infection. Respiratory infections CA-MRSA in hospitalized patients pneumonia treatment begins before culture results. After the susceptibility to antibiotics is performed, the infection may be treated with vancomycin or linezolid for up to 21 days. If the pneumonia is complicated by the accumulation of pus in the pleural cavity surrounding the lungs, drainage may be done along with antibiotic therapy. People with cystic fibrosis may develop respiratory complications related to MRSA infection. The incidence of MRSA in those with cystic fibrosis increased during 2000 to 2015 by five times. Most of these infections were HA-MRSA. MRSA accounts for 26% of lung infections in those with cystic fibrosis."}, {"id": "wiki20220301en062_310", "title": "Pneumonia severity index", "score": 0.009523809523809525, "content": "Usage The purpose of the PSI is to classify the severity of a patient's pneumonia to determine the amount of resources to be allocated for care. Most commonly, the PSI scoring system has been used to decide whether patients with pneumonia can be treated as outpatients or as (hospitalized) inpatients. A Risk Class I or Risk Class II pneumonia patient can be sent home on oral antibiotics. A Risk Class III patient, after evaluation of other factors including home environment and follow-up, may either: be sent home with oral antibiotics be admitted for a short hospital stay with antibiotics and monitoring. Patients with Risk Class IV-V pneumonia patient should be hospitalized for treatment. Algorithm"}, {"id": "pubmed23n0894_14744", "title": "[Bacteremia caused by ciprofloxacin-resistant Salmonella serotype Kentucky: a case report and the review of literature].", "score": 0.009523809523809525, "content": "Salmonella infections can be seen in four clinical types, namely gastroenteritis, bacteremia/sepsis, enteric fever and carriage. These infections can result in uncomplicated diarrhea in most cases, but can lead to invasive disease requiring antimicrobial therapy and can be life-threatening in elderly or immunocomprimised patients. Broad-spectrum cephalosporins and fluoroquinolones are crucial options in the treatment of the invasive infections. Ciprofloxacin resistance is rarely seen in non-typhoid Salmonella enterica isolates, and only in S. Typhimurium, S. Choleraesuis and S. Schwarzengrund. In this report, we aimed to discuss a patient infected with ciprofloxacin-resistant Salmonella Kentucky under the light of data from our country and the world. A 52-year-old male patient wih acute myocardial infarction was hospitalized in intensive care unit of cardiovasculer surgery for left ventricular assist device (LVAD) implantation for the treatment of left ventricular disfunction. On the seventh day of LVAD and coronary artery bypass grafting (CABG), the patient presented high fever and productive cough. His physical examination revealed hyperemia around the insertion point of right jugular central venous catheter (CVC) and a serous discharge from the insertion point of LVAD located just below the inferior edge of sternum. Empiric IV cefoperazone/sulbactam (SCF) therapy was started with the prediagnosis of pneumonia and bloodstream infection. The blood samples taken from peripheral veins and CVC, and swabs taken from LVAD insertion point for culture when the patient was febrile, revealed the growth of bacteria with S type and lactose-negative colonies on EMB and SS media. Biochemical characteristics of the isolate were as follows: lactose fermentation negative, H<sub2</subS positive, IMVIC (-,+,-,+), urease negative, lysine/ornithine decarboxylase positive and motile. The bacteria was then identified as Salmonella enterica serotype Kentucky (8,20;i;z6) by agglutination tests. Antibiotic susceptibility tests were conducted according to CLSI guidelines and it was found as ampicillin- and ciprofloxacin-resistant. Ciprofloxacin resistance of the isolate was confirmed with E-test. Stool culture was performed to investigate the source of infection, and S. Kentucky was isolated. On the 15th day of SCF treatment, LVAD was taken out, and tissue cultures taken from the fibrillar tissues between pericardial layers during surgery, also yielded S. Kentucky growth. On the second day of SCF therapy the patient's fever returned normal and on the seventh day, CBC and CRP values were normalized. Nevertheless, the clinical situation of the patient worsened gradually and on the 40th day he was intubated due to low oxygen saturation and pleural effusion. His antibiotherapy was stopped on 42nd day as the blood cultures were negative and his clinical situation was attributed to cardiac failure. The patient died four days after the antibiotherapy has stopped due to cardiac reasons. To our knowledge, this is the first reported case infected with ciprofloxacin-resistant Salmonella Kentucky in our country."}, {"id": "pubmed23n0350_1947", "title": "Prophylactic Lactobacillus GG reduces antibiotic-associated diarrhea in children with respiratory infections: a randomized study.", "score": 0.009433962264150943, "content": "Antimicrobial treatment may disturb the colonization resistance of gastrointestinal microflora, which may induce clinical symptoms, most commonly diarrhea. The severity of antibiotic-associated diarrhea may range from a brief, self-limiting disease to devastating diarrhea with electrolyte disturbances, dehydration, crampy abdominal pain, pseudomembranous colitis, toxic megacolon, or even death. The incidence of diarrhea in children receiving a single antimicrobial treatment is unclear. In addition to more critical use of antimicrobials, adjunctive preventive measures to antibiotic-associated diarrhea are needed. The objective of this study was to evaluate the incidence of diarrhea after antimicrobial treatment in children with no history of antimicrobial use during the previous 3 months. Another aim of this study was to assess the preventive potential of Lactobacillus rhamnosus GG (Lactobacillus GG; American Type Culture Collection 53103), a probiotic strain with a documented safety record and a therapeutic effect in viral gastroenteritis on antibiotic-associated diarrhea. Oral antimicrobial agents were prescribed for the treatment of acute respiratory infections at the clinics of the Health Care Center of the City of Tampere or Tampere University Hospital, Finland, to 167 patients who were invited to participate in the study. Of the patients, 48 were lost to follow-up; therefore, the final study population consisted of 119 children from 2 weeks to 12. 8 years of age (mean: 4.5 years). All study subjects met the inclusion criteria: they had not received any antimicrobial medication during the previous 3 months, they did not suffer from gastrointestinal disorders, and they did not need intravenous antimicrobial treatment. The patients were randomized to receive placebo or 2 x 10(10) colony-forming units of Lactobacillus GG in capsules given twice daily during the antimicrobial treatment. Lactobacillus GG and placebo capsules were indistinguishable in appearance and taste. The parents kept a daily symptom diary and recorded stool frequency and consistency at home for 3 months. Diarrhea was defined as at least three watery or loose stools per day for a minimum of 2 consecutive days. In the case of diarrhea, viral (adenovirus, rotavirus, calicivirus and astrovirus) and bacterial (Salmonella, Shigella, Yersinia, Campylobacter, Clostridium difficile, Staphylococcus aureus, and yeasts) analyses were studied in fecal samples. The metabolic activity of the gut microflora was assessed by analysis of fecal urease, beta-glucosidase, and beta-glucuronidase activities. The primary outcome measure was diarrhea during the first 2 weeks after the beginning of the antimicrobial treatment, because this period most likely reflects the effects of antimicrobial use. Secondary outcome measures were the activities of fecal urease, beta-glucuronidase, and beta-glucosidase. On the entire follow-up, 80% of any gastrointestinal symptoms were reported during the first 2 weeks after the beginning of the antimicrobial treatment. The incidence of diarrhea was 5% in the Lactobacillus GG group and 16% in the placebo group within 2 weeks of antimicrobial therapy (chi(2) = 3.82). The treatment effect (95% confidence interval) of Lactobacillus GG was -11% (-21%-0%). In diarrheal episodes, the viral and bacterial analyses were positive for Clostridium difficile in 2 cases and for Norwalk-like calicivirus in 3 cases. The age of the patients with diarrhea was between 3 months and 5 years in 75% of cases in both groups. The severity of diarrhea was comparable in the study groups, as evidenced by similar stool frequency (mean: 5 per day; range: 3-6) and the duration of diarrhea (mean: 4 days; range: 2-8). The activities of fecal urease and beta-glucuronidase, but not beta-glucosidase, changed significantly after the beginning of the antimicrobial treatment in the Lactobacillus GG group and in the placebo group alike. (ABSTRACT TRUNCATED)"}, {"id": "pubmed23n0537_1936", "title": "[Oral pefloxacin in the treatment of acute gastroenteritis].", "score": 0.009433962264150943, "content": "In the case of travellers' diarrhoea, in order to reduce to a minimum the period of sickness and temporary invalidity, suitable therapy needs to be undertaken, involving the reintegration of fluids and electrolytes, as well as antibiotic treatment whose spectrum of action covers the most commonly isolated microorganisms. The aim of the study was to verify the rapidity of the antibacterial action of pefloxacin in the treatment of this type of gastrointestinal infection. The patients enrolled were treated for 5 days with pefloxacin in tablets containing 400 mg of the active agent, given orally. Treatment involved a first dose of 800 mg (2 tablets in a single administration), followed by 400 mg twice a day (1 tablet every 12 hours). Administration was carried out prevalently at mealtimes. At the end of treatment a two-week follow-up period was scheduled. At the beginning of the study, and after 5 days' treatment, a coproculture was carried out, with identification of the germ responsible for the infection. The study was carried out in accordance with the principles of the Helsinki declaration and its revisions, in particular each patient gave his/her informed consent to participate in the trial. Thirty patients enrolled in the study (16 males, 14 females), of mean age 48.2 years (18-82 min-max), mean weight of 66.3 kg (46-88 min-max), suffering from acute gastroenteritis. The coproculture carried out at the baseline for all patients led to the isolation in 10 patients, of 10 pefloxacin-sensitive GRAM-negative bacterial strains: Salmonella spp. in 6 cases, Shigella spp. in 2, Escherichia coli in 1 and Yersinia enterocolitica in 1. At the end of treatment and follow-up the coproculture indicated that all the germs isolated initially had been eradicated and no cases of superinfection or reinfection occurred. The number of daily evacuations gradually decreased from a mean of 6.8 at the baseline to 1.1 on the fifth day (p&lt;0.0001). The consistency of faeces had increased in a significant fashion by the third day and at the end of treatment 93.3% of patients had well-formed faeces. In the course of treatment abdominal pain, nausea and vomiting decreased progressively and body temperature returned within normal limits by the third day. Safety was excellent in 93.3% of patients, with one case of nausea and one of erythema, which resolved spontaneously in the course of treatment. At the end of treatment with pefloxacin, the physician expressed his final judgment of efficacy, which was excellent in 86.7% of cases (26/30) and good in 13.3% (4/30), in accordance with the clinical evaluation of recovery in 93.3% of cases (28/30) and of improvement in 6.7% (2/30). In forms of acute gastroenteritis in which coproculture shows the presence of germs sensitive to pefloxacin, the use of this quinolone guarantees a high percentage of success with a short course of treatment."}, {"id": "wiki20220301en029_32762", "title": "Hospital-acquired infection", "score": 0.009431524547803619, "content": "Types Hospital-acquired pneumonia Ventilator-associated pneumonia Urinary tract infection Gastroenteritis Puerperal fever Central line-associated blood stream infection Organisms Staphylococcus aureus Methicillin resistant Staphylococcus aureus Candida albicans Pseudomonas aeruginosa Acinetobacter baumannii' Stenotrophomonas maltophilia Clostridium difficile Escherichia coli Tuberculosis Vancomycin-resistant Enterococcus Legionnaires' disease Cause Transmission In-dwelling catheters have recently been identified with hospital acquired infections. To deal with this complication, procedures are used, called intravascular antimicrobial lock therapy that can reduce infections that are unexposed to blood-borne antibiotics. Introducing antibiotics, including ethanol, into the catheter (without flushing it into the bloodstream) reduces the formation of biofilms."}, {"id": "wiki20220301en069_48387", "title": "Blood in stool", "score": 0.009345794392523364, "content": "Inflammatory bowel Diseases causing inflammation in the GI tract can lead to blood in the stool. Inflammation can occur anywhere along the GI tract in Crohn's disease, or in the colon if a person has ulcerative colitis. Crohn's disease Ulcerative colitis Colitis Enteritis—inflammation of the small intestine, which has many causes including autoimmune conditions (e.g. Crohn's disease), certain drugs (e.g. ibuprofen), radiation therapy, and Coeliac disease. Infectious colitis Food poisoning—the bacteria that is associated with bloody diarrhea is typically E. coli Campylobacter enteritis Shigellosis Salmonellosis (Salmonella enteritis/Salmonella enterocolitis) Bacterial gastroenteritis Campylobacter jejuni Clostridium difficile Escherichia coli enteritis—most common cause of travelers' diarrhea Salmonella enterica Shigella dysenteriae see also dysentery Staphylococcus aureus Drug-induced colitis Radiation enteritis"}, {"id": "pubmed23n0090_4583", "title": "[Bacterial etiological factors of diarrhea in the stool of infants hospitalized in January 1987].", "score": 0.009345794392523364, "content": "The results are presented of the second part of the studies conducted for monitoring of the variability of bacterial factors causing diarrhoea in the youngest children. The presently reported study comprised 42 babies aged from 7 days to 18 months treated in hospital in January 1987. The number of children with diarrhoea hospitalized in this period was relatively smaller than in January 1985, while the incidence of enteropathogenic bacteria in stools was very similar. They included mainly Klebsiella and enteropathogenic Escherichia coli strains, especially in the youngest children. In only one case Salmonella arizonae was found while in 1985 a very high number of Salmonella agona strains were cultured, especially from newborns. In both parts of the study Campylobacter jejuni and Yersinia enterocolitica were never found in stools. In both parts of the study the course of bacterial diarrhoea was mostly moderately severe, loose stools continued usually for 3-4 days, but in the second part more cases of severe diarrhoea were noted, which, however, could not have been related to the bacterial species cultured from stools or to the presence of mixed bacterial flora. The importance is stressed of the carrier state of enteropathogenic bacteria which was observed in three-fourths of patients in January 1987, while in January 1985 this was present in nearly all cases without diarrhoea."}, {"id": "wiki20220301en002_144475", "title": "Pneumonia", "score": 0.009259259259259259, "content": "Antibiotics by mouth, rest, simple analgesics, and fluids usually suffice for complete resolution. However, those with other medical conditions, the elderly, or those with significant trouble breathing may require more advanced care. If the symptoms worsen, the pneumonia does not improve with home treatment, or complications occur, hospitalization may be required. Worldwide, approximately 7–13% of cases in children result in hospitalization, whereas in the developed world between 22 and 42% of adults with community-acquired pneumonia are admitted. The CURB-65 score is useful for determining the need for admission in adults. If the score is 0 or 1, people can typically be managed at home; if it is 2, a short hospital stay or close follow-up is needed; if it is 3–5, hospitalization is recommended. In children those with respiratory distress or oxygen saturations of less than 90% should be hospitalized. The utility of chest physiotherapy in pneumonia has not yet been determined."}, {"id": "pubmed23n0212_11419", "title": "Changes in the trend of shigellosis in Dhaka: family study on secondary infection, clinical manifestation and sensitivity pattern: 1980.", "score": 0.009259259259259259, "content": "The incidence of shigellosis and the death rate have increased and the resistance of shigellae to antibiotics has changed in Dhaka during our experiences. In 1980, we investigated the secondary infection and case rates, infection to case ratio, duration of illness, excretion of shigellae and antibiotic sensitivity pattern in 100 families with cases of shigellosis, culturing rectal swabs obtained by home visits for a 10-day period. Standard methods were used for culture and sensitivity tests. The over-all secondary infection rate in contacts was 27.3% and the case rate 10.7%. The rates were higher for Shigella flexneri than for Sh. dysenteriae. When the index cases were nought to four years old the secondary infection and case rates were higher than when index cases were older. Contacts aged nought to four years had highest attack rates. The average duration of excretion of Sh. flexneri was 4.5 and Sh. dysenteriae 2.6 days. Illness was one day longer for Sh. dysenteriae than for Sh. flexneri. Cases of shigellosis in hospital had higher rates of fever and blood in stool than those who were not in-patients. 40% of Sh. dysenteriae and 14% of Sh. flexneri were sensitive to tetracycline, 0 to 5% to streptomycin and 100% to sulphamethoxazole, trimethoprim and gentamicin. Incidence of Sh. flexneri had increased in 1980 but that of Sh. dysenteriae remained the same as in 1973 although Sh. dysenteriae type 1 appeared to be less infective in 1980 than in 1973."}, {"id": "wiki20220301en394_10207", "title": "Campylobacter coli", "score": 0.009174311926605505, "content": "Treatment Campylobacteriosis is often self-limiting infection, which is treated according to the symptoms, for example with electrolyte replacement and rehydration. While extra fluid is required of an infected person for as long as the symptoms lasts, antibiotics such as azithromycin or ciprofloxacin can be used to treat risk groups, including immunocompromised patients. Due to the increased antibiotic usage in both animal agriculture and human populations, Campylobacter spp. has been reported to be progressively resistant to several antibiotics, including fluoroquinolones and macrolides. Although most patients recover from the infection, severe post-infectious complications such as Guillain-Barré syndrome, a rare autoimmune condition which causes muscle weakness as a result of the immune system damaging the peripheral nervous system, have been linked to campylobacteriosis. See also List of human flora References"}, {"id": "pubmed23n0279_3297", "title": "[Life-threatening diarrhea in atypical course of legionellosis].", "score": 0.009174311926605505, "content": "After eating a meal of poultry a 41-year-old man fell ill with severe diarrhoea, persistent high fever of around 39 degrees C, dehydration and somnolence. On admission to hospital physical examination was normal except for signs of dehydration. The blood count showed a leukocytosis (13,300/microliters) with 60% stab-form neutrophils. The erythrocyte sedimentation rate was raised to 49/82 mm. Also increased were the serum concentrations of creatinine (3.5 mg/dl), creatine kinase (179 U/l), lactate dehydrogenase (298 U/l) and C-reactive protein (16.8 mg/dl). Bacteriological and virological examinations of blood and stool were negative. A normal fluid and electrolyte balance was re-established. But as there was no improvement, ampicillin was administered, 2 g three times daily, then ciprofloxacin, 500 mg two times daily, and finally combined with metronidazole, 500 mg three times daily. Despite this treatment a chest radiograph on the tenth day revealed an infiltration in the left basal lung segment, and the legionella titre became positive at 1:2045. The antibiotic treatment was changed to 150 mg roxithromycin two times daily. The fever fell within 3 days and the diarrhoea stopped after 5 days. He was discharged free of symptoms after 24 days."}, {"id": "pubmed23n0942_9136", "title": "<i>Clostridium difficile</i> Colitis Leading to Reactive Arthritis: A Rare Complication Associated With a Common Disease.", "score": 0.00909090909090909, "content": "The relationship between reactive arthritis and enteric infections caused by <iYersinia enterocolitica, Campylobacter jejuni</i, and <iSalmonella typhimurium</i is well documented. <iClostridium difficile</i colitis is a less recognized cause of reactive arthritis. We present a case of a 58-year-old woman with <iClostridium difficile</i colitis complicated by reactive arthritis. A 58-year-old woman with no significant past medical history presented to our hospital with complaints of nonbloody watery diarrhea, abdominal pain for the past 1 week, and right knee pain starting 1 day prior. The patient had recently used antibiotics for a respiratory tract infection. On examination, the patient had a swollen and erythematous right knee. While in the hospital the patient also developed a similarly painful and swollen left knee. The patient was found to be positive for <iC difficile</i toxin in stool. Synovial fluid analysis of both the knee joints revealed a sterile and inflammatory fluid, negative for crystals and showing no growth on gram stain. We diagnosed the patient with reactive arthritis secondary to <iC difficile</i colitis once all other causes of the bilateral knee joint symptoms were ruled out with appropriate laboratory and imaging studies. Treatment with oral vancomycin and an anti-inflammatory was initiated, and the patient had complete resolution of symptoms. This case illustrates the importance of recognizing <iC difficile</i colitis as a potential differential for reactive arthritis under the appropriate circumstances. The treatment of reactive arthritis is mainly supportive and treating the underlying cause, which happens to be <iC difficile</i in this case."}, {"id": "pubmed23n0172_317", "title": "[Salmonellosis in children: study of 95 cases in the Hospital Ste-Justine, Montreal, in 1963-1964].", "score": 0.00909090909090909, "content": "A retrospective study was done in children in whom salmonellosis was confirmed by laboratory findings with the aim of reviewing etiology, epidemiology, clinical manifestations and therapy. The 15 serotypes most frequently isolated from stool, and in exceptional cases from urine, are discussed. If patients with typhoid fever are excluded, only one patient (who subsequently died) had a blood culture positive for Salmonella, specifically S. enteritidis.No seasonal or other peaks of incidence were noted. Age appeared to be important; of 81 patients with gastroenteritis, 30 were less than 6 months old.Two children in the older age group developed complications; one with appendicitis required surgery.Ten strains of Salmonella out of 23 tested by the disc method showed in vitro resistance to ampicillin on primary isolation.Acquired in vitro resistance to one or more antibiotics appeared to develop with six Salmonella strains reisolated from patients after or during antibiotic treatment.In several children the stool cultures remained positive after clinical signs had disappeared. These findings strongly suggest that, even though antibiotic therapy may improve the symptoms of Salmonella infection, it does not decrease the number of carriers during the convalescent period."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 99]], "word_ranges": [[0, 20]], "text": "With the deformity and pain in the distal radius, we expect a fracture at this level, so we mark 3."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "With the deformity and pain in the distal radius, we expect a fracture at this level, so we mark 3. However, I see it as unquestionable, given that it could also have a concomitant fracture in the ulnar green stalk, although the main lesion is that of the radius.", "full_answer_no_ref": "With the deformity and pain in the distal radius, we expect a fracture at this level, so we mark [HIDDEN]. However, I see it as [HIDDEN], given that it could also have a concomitant fracture in the ulnar green stalk, although the main lesion is that of the radius.", "full_question": "A 7-year-old boy brought to the emergency department after falling off a swing onto his right hand. He has no history of interest. He presents with fork dorsum deformity of the wrist and functional impotence, with normal distal neurovascular status. What lesion would you expect to find on the urgent x-ray you request?", "id": 471, "lang": "en", "options": {"1": "Fracture - dislocation of Monteggia.", "2": "Radial head fracture.", "3": "Epiphysiolysis of the distal radius.", "4": "Fracture in green stem of ulna.", "5": NaN}, "question_id_specific": 139, "type": "ORTHOPEDIC SURGERY AND TRAUMATOLOGY", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en038_74788", "title": "Distal radius fracture", "score": 0.017973200673995025, "content": "Before the 18th century, distal radius fracture was believed to be due to dislocation of the carpal bones or the displacement of the distal radioulnar articulation. In the 18th century, Petit first suggested that these types of injuries might be due to fractures rather than dislocations. Another author, Pouteau, suggested the common mechanism of injury which leads to this type of fractures - injury to the wrist when a person falls on an outstretched hand with dorsal displacement of the wrist. However, he also suggested that volar displacement of the wrist was due to the ulnar fracture. His work was met with skepticism from colleagues and little recognition, since the article was published after he died. In 1814, Abraham Colles described the characteristics of distal end radius fracture. In 1841, Guilaume Dupuytren acknowledged the contributions by Petit and Pouteau, agreeing that the distal end radius fracture is indeed a fracture, not a dislocation. In 1847, Malgaigne described the"}, {"id": "pubmed23n0648_19629", "title": "Fracture-Dislocations You Can't Afford to Miss.", "score": 0.014071870425461586, "content": "Occult dislocations can accompany extremity fractures and may lead to serious complications if undetected and untreated. Physicians, therefore, need to watch for uncommon trauma like distal radius fractures with radioulnar joint disruption (Galeazzi fractures), proximal ulna fractures with radial head dislocation (Monteggia fractures), and midfoot dislocations with or without fracture (Lisfranc injuries). Injury recognition involves comparing important historical features like a fall onto an outstretched hand, physical exam findings such as the nature of the pain, and sometimes-subtle radiographic clues. Treatment is usually surgical."}, {"id": "wiki20220301en038_74748", "title": "Distal radius fracture", "score": 0.013265923429262812, "content": "Distal radius fractures are common, and are the most common type of fractures that are seen in children. Distal radius fractures represent between 25% and 50% of all broken bones and occur most commonly in young males and older females. A year or two may be required for healing to occur. Most children with a buckle wrist fracture experience a full recovery to their previous level of wrist function and do not have an increased chance of re-fracturing the same spot or other adverse effects. Signs and symptoms People usually present with a history of falling on an outstretched hand and complaint of pain and swelling around the wrist, sometimes with deformity around the wrist. Any numbness should be asked to exclude median and ulnar nerve injuries. Any pain in the limb of the same side should also be investigated to exclude associated injuries to the same limb."}, {"id": "article-31420_10", "title": "Wrist Sprain -- History and Physical", "score": 0.01209828853479264, "content": "A Scapholunate ligament injury commonly presents following a fall onto a dorsally extended and ulnar deviated hand. Wrist pain is usually dorsal-radial and accompanied by an inability to bear weight with the wrist extended, such as in a push-up position. The physical exam may show tenderness over the location of the scapholunate ligament, which is palpable on the dorsum of the wrist, immediately distal to the head of the radius. A scaphoid shift test can be performed as part of the physical exam to aid in the diagnosis with a positive likelihood ratio of 2.76 and a negative likelihood ratio of 0.25. [6] Dynamic imaging with a clenched fist, as well as contralateral wrist views, can improve the sensitivity of imaging with plain films. [7]"}, {"id": "pubmed23n1124_3678", "title": "Elbow Dislocation with Irretrievable Rotating-Rope Injury of the Forearm: Case Report and Literature Review.", "score": 0.011518626142045603, "content": "Simultaneous dislocation of the elbow, radioulnar joint and proximal radius fracture with rotary noose injury to the medial ulna tubercle is extremely rare. An emergency surgery was performed to reduce it. The radial head with the backbone was reset after two hammers were fixed, then the radial capitulum safety was fixed with a locking plate. After the ulnar instability was examined, two Kirschner wires were drilled percutaneously to fix the elbow flexion at 100° under closed reduction, and two Kirschner wires were drilled percutaneously to fix the ulnar joint. Good follow-up results were achieved. To the best of our knowledge, this is the first report on this particular type of injury and on this approach to treating this type of injury. We report the case of a 36-year-old male, who extended and landed on his left hand to protect his child in right arm before felling, resulting in severe pain and deformity of his left elbow and wrist and loss of movement in these joints. X-ray examination found proximal distal radioulnar joints, a proximal radial fracture and a dislocation bowstring in the ulna nodule. For a timely diagnosis in an emergency open reduction situation, accurate judgment of this injury is highly important. After 12 months of postoperative follow-up, the patient was symptom-free, and radiographs showed fracture healing. We performed emergency reduction and internal fixation of the elbow and successfully saved elbow function, no stability decrease and movement restriction. This case also provides a new reference for the treatment of this type of elbow fracture dislocation."}, {"id": "article-28166_7", "title": "Radial Head Fractures -- History and Physical", "score": 0.010779859050445104, "content": "The fracture occurs when a patient has direct traumatic to the elbow or falls with the wrist and the hand in extension. The usual signs are pain and functional impotence, there may be blockages in the elbow."}, {"id": "wiki20220301en336_36318", "title": "Ulna fracture", "score": 0.010597618267234786, "content": "Nightstick fracture is a fracture of the middle portion of the ulna without other fractures. Distal ulna fractures typically occur along with distal radius fractures. Hume fracture - a fracture of the olecranon with an associated anterior dislocation of the radial head. Monteggia fracture - a fracture of the near to elbow end of the ulna with the dislocation of the head of the radius at the elbow joint. Galeazzi fracture - not a fracture of the ulna but a displaced fracture of the radius accompanied by a dislocation of the ulna at the wrist, where the radius and ulna come together."}, {"id": "wiki20220301en022_70393", "title": "Radius (bone)", "score": 0.009900990099009901, "content": "Essex-Lopresti fracture – a fracture of the radial head with concomitant dislocation of the distal radio-ulnar joint with disruption of the interosseous membrane. Radial shaft fracture Distal radius fracture Galeazzi fracture – a fracture of the radius with dislocation of the distal radioulnar joint Colles' fracture – a distal fracture of the radius with dorsal (posterior) displacement of the wrist and hand Smith's fracture – a distal fracture of the radius with volar (ventral) displacement of the wrist and hand Barton's fracture – an intra-articular fracture of the distal radius with dislocation of the radiocarpal joint. History The word radius is Latin for \"ray\". In the context of the radius bone, a ray can be thought of rotating around an axis line extending diagonally from center of capitulum to the center of distal ulna. While the ulna is the major contributor to the elbow joint, the radius primarily contributes to the wrist joint."}, {"id": "pubmed23n0212_10517", "title": "Fractures of the distal end of the radius. An historical account.", "score": 0.009900990099009901, "content": "From the time of Hippocrates to the beginning of the nineteenth century, fractures of the distal end of the radius were mistaken for dislocations of the wrist. Clinical observations, anatomic dissections, and experiments on cadavers dispelled this error. It was not, however, until after the discovery of roentgenograms that the variety and complexity of these common injuries could be appreciated. From the large group of fractures of the distal end of the radius, three distinct varieties can be distinguished rather easily, and to them the eponyms of Colles (Pouteau-Colles), Smith, and Barton have been applied. The remainder, which consist of a substantial number, do not fit into any specific category and must be described individually."}, {"id": "pubmed23n1068_10787", "title": "An Extremely Rare Combination of Monteggia Equivalent Type 1 Lesion (diaphyseal Ulna and Radial Neck Fractures Without Dislocation) with Ipsilateral Radius Shaft and Distal Radius Fractures in a Child.", "score": 0.00980392156862745, "content": "We report an extremely rare combination of Monteggia equivalent Type 1 lesion (diaphyseal ulna and radial neck fractures without dislocation) with ipsilateral radius shaft and distal radius fractures in a 13-year-old boy. There are only a few cases of Monteggia or Monteggia equivalent injury with ipsilateral forearm fractures in children, and injury pattern being reported by us is not only rare but also the only case reported, thus far to the best of our knowledge. A 13-year-old, right-hand dominant boy presented in casualty with a history of fall 1 day back with pain, swelling and deformity in the left forearm with bleeding from the left forearm, and restriction of movement of fingers and thumb of the left hand. On examination, there was a wound of size 1.5 cm on the upper third-forearm over the ulnar aspect. No neurovascular deficit was present. X-rays were performed, which suggested Type I Monteggia fracture equivalent lesion (diaphyseal ulna and radial neck fractures without dislocation) with ipsilateral distal radius and radial shaft fractures. The patient was operated with toileting, debridement, and close reduction of proximal ulnar fracture with titanium elastic nail (TENS) Distal radius was managed by percutaneous fixation with two K-wires under the guidance of image intensifier, while the shaft of radius fracture was managed by close reduction and internal fixation with elastic TENS nail with a lateral entry point and radial neck fracture was managed by the Metaizeau technique. Follow-up of the patient showed subsequent union of all fractures with good functional outcome. We have highlighted an extremely rare combination of injuries. Early recognition and prompt surgical intervention can lead to a satisfactory outcome, even in these complex injuries."}, {"id": "wiki20220301en201_5730", "title": "List of Ashes to Ashes characters", "score": 0.00980392156862745, "content": "He is reported missing by Donna. Days later, on 8 November 1982 his body is discovered floating in the canal by DCI Gene Hunt on the day he was to have flown to Turkey. The coroner opines that the low volume of water in his lungs suggests that he was killed on dry land, and notes the blunt force trauma to his right frontal lobe. The two facts suggesting murder to DI Alex Drake; while Hunt agrees with the possibility, he offers the alternative theory that Mitchell was drunk, fell, hit his head and fell in the river. Drake and the coroner agree that the scabbing on Mitchell's wrist wounds suggest they were received at the same time as the head wound."}, {"id": "pubmed23n0562_17997", "title": "[Wrist pain following radial head fracture caused by a tear in the interosseous membrane (Essex-Lopresti lesion)].", "score": 0.009708737864077669, "content": "A 44-year-old multiple injured patient presented with several fractures including a dislocated, comminuted radial head fracture after a 4 meter fall from a ladder. He was treated with radial head resection. However, at routine follow-up he indicated pain and loss of function of his wrist due to a distal radio-ulnar dislocation with a high position of the ulna, causing loss of pronation and supination. This is also known as the Essex-Lopresti lesion. Operative treatment included reduction and fixation of the distal radio-ulnar joint after resection osteotomy of the distal ulnar shaft according to Sauvé and Kapandji."}, {"id": "pubmed23n0763_14041", "title": "History of head trauma in a 6-year-old boy: maybe more than meets the eye (and head).", "score": 0.009615384615384616, "content": "It is spring and you are meeting for the first time, Eddie, a recently turned 6-year-old boy who moved to the area in September of the previous year and is here for his 6-year-old health maintenance visit. Eddie's mother is concerned that although he is \"only\" in kindergarten, he is not retaining any information at school. His mother reports he knew some of his letters before kindergarten. Currently, when he is trying to write a word, for example, \"daddy\" he will need to ask his mother: \"what letter is the letter D?\" Before kindergarten, he knew his numbers 1 to 10. At times now, Eddie will forget these numbers. For example, \"if he is counting he will forget what comes after 4 and what comes after 9.\" Mother reports he will start crying for no apparent reason and if she asks why, he will say \"I don't know why.\" Mother is worried that Eddie is sad, although she denies suicidal ideation. She reports he used to like making noise with other kids, and now he cannot stand when the children are noisy. Eddie will comment he does not want to go to school because the kids make lots of noise and his head hurts. He complains of headaches as often as 2 to 3 times a month. She next states, \"This was not an issue before his head trauma.\" At this point, she reveals to you that in August, before the family relocated, Eddie fell from a 7-foot deck onto concrete while playing. He struck his head on the left side and lost consciousness for several seconds until shaken awake. He was nauseous and disoriented initially but without emesis or incontinence. He was taken to the local emergency department where he was admitted for 1 day and diagnosed with closed head injury, left frontal epidural hematoma, and question of postconcussive syndrome. Eddie has gone back to see the neurosurgeon twice over the last 6 months for scheduled visits and since the accident has had no further treatment.Eddie's mother reports that before the accident, if she read him a story from a book, he could remember the details from the story. Currently, he does not have good memory recall. Before the trauma, he did not attend a preschool program but stayed home with his mother full time. Eddie's first formal schooling has been kindergarten this year. When they moved, the neurosurgeon recommended he start school at the end of September given his head trauma in August. He had a recent computed tomography completed 3 months ago showing the epidural hematoma had completely resolved and the study was otherwise normal. His mother reports he had an evaluation the spring before the accident for a kindergarten screening test and was reported as \"excellent.\" Eddie's birth and medical history are otherwise unremarkable except for some seasonal allergies. He has not had a loss in language skill, although his mother reports he did not speak during his hospitalization. He would just stare and nod his head if someone would ask him a question. No family history of any learning or behavioral difficulties on either side of the family. Eddie has 2 older brothers, 10 and 7 years of age, with no learning issues.What would you do next?"}, {"id": "wiki20220301en273_20768", "title": "Judgment Day (2002)", "score": 0.009615384615384616, "content": "With his feud with Triple H now over, Chris Jericho decided to enter the King of the Ring tournament, with the winner to receive a WWE Undisputed Championship match on August 25 at SummerSlam. Jericho was scheduled to compete against Edge in the first round tournament, but Edge forfeited his match to Jericho due to a legitimate shoulder injury. Jericho advanced as far as the semi-finals, losing to Rob Van Dam. On the May 30 episode of SmackDown!, The Undertaker defeated Randy Orton to retain the WWE Undisputed Championship. Afterward, Triple H assaulted The Undertaker and demanded a title shot. During Triple H's match against Test, The Undertaker interfered, enabling Test to nail Triple H with a big boot for the pin. Afterwards, The Undertaker nailed Triple H with multiple chair shots to his elbow. On the June 6 episode of SmackDown!, Triple H became the number one contender to the WWE Undisputed Championship by defeating Hollywood Hulk Hogan. Triple H faced The Undertaker for the"}, {"id": "pubmed23n0886_18993", "title": "Ipsilateral Plastic Deformation Monteggia and Galeazzi-Type Fracture in a Child: A Case Report.", "score": 0.009523809523809525, "content": "A 7-year-old boy attended the emergency department after falling from a climbing frame onto his outstretched left wrist. On examination, there was mild swelling to the left elbow and tenderness to the antecubital fossa. There was also tenderness diffusely to the distal ulnar and radius. There was no neurovascular deficit. Radiographs revealed a plastic deformation fracture of the left radius and ulna, with dislocations of the ipsilateral radiocapitellar joint and distal radioulnar joint. A diagnosis of combined Monteggia and Galeazzi-type fractures of the left forearm was made. It is rare to find cases of combined Monteggia and Galeazzi fractures to the same forearm. Furthermore, to our knowledge, ipsilateral plastic deformation Monteggia and Galeazzi-type fractures in children have not been reported in the literature."}, {"id": "article-20616_18", "title": "Distal Radius Fractures -- History and Physical", "score": 0.009523809523809525, "content": "Patients with distal radial fractures will often complain of post-traumatic distal upper extremity pain. DR fractures can also be present as part of a larger multiple trauma presentation. In either case, it is vital not to fixate on an obvious DR injury, and to do a complete evaluation for other possible life-threatening injuries or problems. A systematic approach in trauma situations will prevent a provider from missing occult injuries. Children are also diagnostically difficult as their presentations are often subtle. Children can suffer unwitnessed falls or trauma, and they are often unable to verbalize their symptoms. They can appear well and favoring the uninjured arm may be the only sign of an underlying fracture. [10] [8]"}, {"id": "pubmed23n0895_16223", "title": "A Very Rare Presentation of Type 1 Monteggia Equivalent Fracture with Ipsilateral Fracture of Distal Forearm-approach with Outcome: Case Report.", "score": 0.009433962264150943, "content": "We report a case of Type 1 Monteggia equivalent injury with intact radio-capitellar congruity, associated with epiphyseal fracture of distal radius and distal ulna shaft in an 11-year-old boy. There are only a few cases of Monteggia or Monteggia equivalent injury with ipsilateral forearm fractures in children, and injury pattern being reported by us is not only rare but also the only case reported thus far to the best of our knowledge, Sood et al. described Type 1 equivalent with epiphyseal injuries of both radius and ulna Osada et al. also described injury pattern same as Sood et al. with epiphyseal separation in both distal radius and ulna. Our case was slightly different than above two in that distally, there was ulna shaft fracture with Salter Harris Type 2 epiphyseal separation in the radius. An 11-year-old, right-hand dominant boy presented in casualty with a history of fall one day back with pain, swelling and deformity in the left forearm with bleeding from left forearm and loss of movement of fingers and thumb of the left hand. On examination, there was a wound of size one centimeter on mid-forearm over the ulnar aspect. Extension of fingers and thumb at metacarpophalangeal joints was lost with intact sensations suggestive of posterior interosseus nerve involvement. No vascular was deficit was present. X-rays were performed which suggested type two epiphyseal separation proximal radius with fracture shaft ulna with lateral angulation in elbow and proximal forearm. Radiocapitellar joint congruity was maintained in the views performed. X-rays of wrist suggested fracture both bones distal forearm epiphysis in distal radius and distal shaft in ulna. The patient was operated with toileting, debridement, and open reduction of proximal ulnar fracture with K-wire. Proximal radius epiphyseal separation was approached by Kocher approach and fixed with two K-wires, while for distal radius epiphyseal separation open reduction and internal fixation was performed. Follow-up of the patient showed posterior interosseus nerve recovery and subsequent union of all fractures with good functional outcome. This type of lesion is rare in children probably because the annular ligament is relatively lax and the radial head dislocates more easily anteriorly, rather than occurrence of fracture as seen in our case, and associated fracture of distal forearm is a very rare injury."}, {"id": "pubmed23n0284_16885", "title": "Occult distal radial fractures.", "score": 0.009433962264150943, "content": "The radiological diagnosis of distal radial fractures is usually easy, but some fractures without displacement cannot be detected at the first examination. In this retrospective study of 626 wrist injuries diagnosed as \"wrist sprain\" we found 39 distal radial fractures which were discovered only after repeated examinations. The incidence of distal radial fractures was much higher than other wrist fractures that were diagnosed after repeated examinations. Repeat standard four-view X-ray examination, as well as other imaging methods, are necessary to diagnose these fractures."}, {"id": "wiki20220301en038_74756", "title": "Distal radius fracture", "score": 0.009345794392523364, "content": "Diagnosis Diagnosis may be evident clinically when the distal radius is deformed, but should be confirmed by X-ray. The differential diagnosis includes scaphoid fractures and wrist dislocations, which can also co-exist with a distal radius fracture. Occasionally, fractures may not be seen on X-rays immediately after the injury. Delayed X-rays, X-ray computed tomography (CT scan), or Magnetic resonance imaging (MRI) can confirm the diagnosis. Medical imaging X-ray of the affected wrist is required if a fracture is suspected. Posteroanterior, lateral, and oblique views can be used together to describe the fracture. X-ray of the uninjured wrist should also be taken to determine if any normal anatomic variations exist before surgery. A CT scan is often performed to further investigate the articular anatomy of the fracture, especially for fracture and displacement within the distal radio-ulnar joint. Various kinds of information can be obtained from X-rays of the wrist:"}, {"id": "pubmed23n0384_7887", "title": "[Who shot Erik XIV?].", "score": 0.009345794392523364, "content": "Though Sweden was still at war with Denmark a revolution took place in 1568 and King Erik XIV was dethroned by his brothers Johan (John) and Karl (Charles). The situation was chaotic and the brothers didn't know how to dispose of the King. To execute or to expatriate him was far too dangerous, consequently remained to keep him imprisoned. This solution to the problem was not without hazards since Erik had many friends who repeatedly tried to free him. The former king, however, also had many enemies, one of whom was Olof Gustafsson Stenbock a man who was responsible for the guarding of Erik. Olof Gustafsson had himself been sentenced to death by King Erik but been reprieved. His brother Abraham Gustafsson Stenbock was on the other hand executed on the order of the King. On the 19th September 1569 Olof Gustafsson visited the imprisoned king, who rushed to his feet, attacking Olof Gustafsson. Olof retreated, took out his gun and shot the King in his left forearm. The King survived 8 years with a badly wounded arm which probably was of no use to him. In this article I have tried to put together all pieces of evidence to prove that the King really was shot. This includes old tales as well as written evidence and facts brought to light during the latest examination of King Erik's remains. This investigation was done in 1958 by professor Carl-Herman Hjortsjö and his collaborators. At the opening of the sarcophagi a defect healed fracture on the left humerus was still clearly visible. Many facts in this article originate from works by L.-I. Jönsson who in a very thorough and accurate way tried to reconstruct this interesting and exciting historical crime."}, {"id": "pubmed23n1089_13206", "title": "Diagnosis and treatment of a closed and inverted metacarpal head fracture.", "score": 0.009259259259259259, "content": "This case report documents a rare inversion of a closed metacarpal head fracture in the setting of polytrauma. Although rare, hemispherical articular bones can fracture and rotate 180°. Because of the symmetry of the bone and the rarity of an inverted, metacarpal head fracture, a delay in diagnosis and subsequent treatment can occur, which can lead to a poor outcome. This is particularly true in the setting of polytrauma. A 38-year-old male, polytrauma patient presented to the emergency department (ED) after falling off a bridge and being struck by an oncoming vehicle. He presented with multiple surgical fractures of the upper and lower extremities as well as his pelvis. Three days after he was brought to the ED, x-rays were performed of his painful left hand, which revealed an extra-articular third metacarpal head fracture, for which he underwent open reduction of the closed fracture. Both collateral ligaments were intact and the head fragment had inverted within the constraints of these ligaments. Some of the ligament and capsular tissue remained attached to the head fragment along the radial and ulnar margins but was otherwise entirely covered with cartilage. The reduction maneuver was difficult but after the reduction was achieved, the fracture appeared stable and no internal fixation was used. Post reduction, the injury was splinted for 2 weeks and then early motion was allowed. The fracture has since healed, and the patient has attained near-full function of the finger and joint."}, {"id": "wiki20220301en137_9431", "title": "EFX (show)", "score": 0.009259259259259259, "content": "As with Tommy Tune, Rick Springfield initially signed a one-year contract. Despite becoming the newest victim in EFX long history of performance-related accidents, fracturing his arm and spraining his wrist early on in his run, Springfield renewed his full contract and stayed with the show until it closed permanently on December 31, 2002. The final performances of EFX Alive! were shot with several cameras. The result was a complete DVD video of EFX Alive! (compiling footage from multiple performances) released exclusively by Springfield's Gomer Records as part of a three-disc limited edition of his 2005 studio album, The Day After Yesterday and available only through the official Rick Springfield merchandise site. An original cast album of the Springfield version of EFX was not released. References"}, {"id": "pubmed23n0719_21543", "title": "Ulnar fracture with late radial head dislocation: delayed Monteggia fracture.", "score": 0.009174311926605505, "content": "Monteggia fractures are rare but commonly discussed lesions, with increasing complications due to late diagnosis. This article describes a case of a Monteggia fracture with delayed dislocation of the radial head. Previous radiographs of a 2-year 8-month-old boy show complete fracture of the distal ulna, with no radial head dislocation. The radial head remained well positioned after 4 weeks. Seven years later, he sustained another arm injury. He was diagnosed with a hematoma but was later believed to have nursemaid's elbow. He presented to our institution 5 weeks after the injury, and the radial head was found to be chronically dislocated, indicating a displacement occurring sometime during the past 7 years. After failing conservative treatment, the patient underwent surgical repair. The annular ligament was reconstructed using a harvested triceps fascia band, and an ulnar osteotomy was performed. A review of the literature found few reports of delayed Monteggia fractures, which accounted the delayed dislocations to ulnar angulation. However, our patient showed minimal ulnar angular deformity. We propose that the initial fracture disrupted the annular ligament and the radial head spontaneously relocated prior to being seen, which put the radial head at risk for later dislocation. We present an alternative hypothesis of dislocation after fracture healing and report the longest known period of delay between fracture and dislocation."}, {"id": "pubmed23n0274_1258", "title": "Brachial artery laceration with closed posterior elbow dislocation in an eight year old.", "score": 0.00909090909090909, "content": "Elbow dislocations are relatively uncommon in children. Rupture of the brachial artery associated with closed elbow dislocations in children is rare. This is a report of an 8-year-old boy, the youngest patient ever to be reported to have a closed posterior dislocation of the elbow associated with a brachial artery laceration. The boy incurred a closed elbow dislocation after a fall onto his outstretched arm. On physical examination, both radial and ulnar (ulnar) pulses were absent. Radiographs showed a posterolateral dislocation of the right elbow and distal fractures of the radius and ulna. Operative exploration of the antecubital fossa showed complete transection of the brachial artery. Repair of the vessel was performed using an interposition vein graft. The distal forearm fractures were managed by closed reduction. At the two-year postoperative follow-up examination, the patient had a normal neurovascular examination with full range of motion of his elbow and wrist. Surgical treatment should include exploration of the antecubital fossa and reconstruction of the injured vessels."}, {"id": "wiki20220301en065_40808", "title": "Reginald Dixon", "score": 0.00909090909090909, "content": "On 3 July 1958, The Bulletin newspaper reported that Dixon was to have an operation that Sunday, quoting him as saying \"A nerve in my right elbow is affecting the hand\". It was \"the result of an injury received in a motorcycle smash when I was a youth.\" The Bulletin reported that \"He wanted to postpone the operation until the end of the season, but doctors have warned him that delay might mean losing the use of the hand\". He returned to the ballroom and its Wurlitzer later in the same month, having made over 2,000 broadcasts."}, {"id": "pubmed23n1062_16231", "title": "Isolated Radial Shaft Fracture with Unreducable Posterior Dislocation of the Radial Head in Adult: A Case Report.", "score": 0.009009009009009009, "content": "Isolated fracture of the radial diaphysis with dislocation of the radial head is a rare injury, which requires careful evaluation. Combined injuries associated with forearm shaft fractures and elbow dislocations are well recognized. A 35-year-old male presented to our emergency department with a history of fall in ditch under influence of alcohol with swelling of her right elbow and deformity of right upper limb. Roentgenograms showed oblique fracture of the radial shaft and dislocation of radial head posteriorly. He underwent open reduction and internal fixation of fracture with limited contact dynamic compression plate and reduction of radial head and fixing with radiocapitellar wire. After 4 months, the fracture healed fully and he had complete full range of elbow movements. Ipsilateral radial head dislocation and radial shaft fracture are extremely rare injury in a child. A good outcome can be achieved by applying principles of the management of proximal forearm fracture-dislocation. Ipsilateral radial head dislocation and radial shaft fracture is extremely rare injury in a child. A good outcome can be achieved by applying principles of management of proximal forearm fracture-dislocation."}, {"id": "wiki20220301en248_7936", "title": "Frank Reys", "score": 0.009009009009009009, "content": "Trainer Trainer Ray Hutchins stood by Frank Reys after a horrible two years in which he had suffered a broken shoulder, two pelvis fractures, a broken cheek bone and a broken ankle, and earned himself the nickname \"Autumn Leaves\". Despite these injuries Hutchins entrusted Reys with the ride on the four-year-old gelding Gala Supreme in the 1973 Caulfield Cup, in which Gala Supreme ran second. Hutchins had long before mapped out the Melbourne Cup as his mission, and after the Caulfield Cup second he prepared Gala Supreme for the Cup."}, {"id": "pubmed23n0412_7746", "title": "Combined Monteggia and Galeazzi fractures in a child: a case report and review of the literature.", "score": 0.008928571428571428, "content": "We present an unusual case in which a combination of Monteggia and Galeazzi fractures occurred in the same forearm. The patient was a 10-year-old male who climbed up the pole of a basketball net, caught hold of the net, then lost his grip, and fell onto his right hand. On physical examination, a complete paralysis of the radial ulnar and median nerves was recognized. X-rays showed an olecranon fracture and lateral dislocation of the radial head in the elbow joint, a dorsal dislocation of the distal bone fragments due to a fracture of the distal third of the radius, and a palmar dislocation of the distal end of the ulna at the wrist joint. The injuries were diagnosed as a combination of a Bado type III Monteggia fracture and a palmar-type Galeazzi fracture of the same arm. Manual reduction and immobilization in a plaster cast were performed. Three years after the injury, both the distal and proximal radioulnar joints were maintained in the reduction position. Range of motion was reduced minimally in extension at the patient's elbow, and there was complete recovery of all three nerves. A combination of Monteggia and Galeazzi fractures in the same arm has been reported in only two pediatric patients worldwide and in eight cases total when adult patients are included, indicating that this is an extremely rare trauma."}, {"id": "wiki20220301en096_50983", "title": "Rhun ab Arthgal", "score": 0.008928571428571428, "content": "It is uncertain when Rhun's reign and life ended. One possibility is that Rhun died in 876, when Causantín seems to have been slain by Vikings. Causantín's death is dated to 876 by the Annals of Ulster. The Chronicle of the Kings of Alba appears to locate his fall in Atholl, whilst several king-lists locate his demise to a place variously called , an otherwise uncertain location that could refer to Inverdovat in Fife. If Causantín indeed enjoyed overlordship of Strathclyde at this date, Rhun could have fallen alongside him as a supporting vassal. It is likewise unknown who succeeded to the kingship of Strathclyde. If Rhun and Causantín both died in 876, Eochaid could well have inherited the British kingship in their absence. Certainly, Causantín's brother, Áed mac Cináeda, succeeded as King of the Picts, and ruled as such upon his death two years later. Whilst it is possible that the Pictish kingship was then assumed by a certain Giric, another possibility is that Eochaid succeeded to"}, {"id": "pubmed23n0722_15245", "title": "Teasing out specific language impairment from an autism spectrum disorder.", "score": 0.008849557522123894, "content": "Marcus is a handsome, sweet, 7½-year-old boy with a significant history of delayed development, specifically in speech and language skills, as well as difficulties with social interactions that have led other specialists to be concerned about a diagnosis of an autism spectrum disorder.He has been seen in our primary care practice since birth. He was born full-term after vaginal delivery weighing 6 pounds, 6 ounces. There were no pregnancy or delivery complications noted. Genetic testing revealed normal chromosomes, fragile X, and microarray testing. Marcus was a picky eater and good sleeper and had delays in toilet training.There is no family history of attention-deficit hyperactivity disorder (ADHD), autism, or substance abuse. Maternal grandmother and mother have a history of learning difficulties, and his father and a paternal uncle have a history of depression and anxiety. Marcus lives in a supportive environment with his mother, father, and sister.Marcus was noted to have significantly delayed language, stuttering, and immediate echolalia as a toddler. Gross and fine motor milestones were met on time, but he did not talk or follow directions until 4 to 5 years old. As a younger child, he would pretend to talk on the phone or mow the grass with a pretend lawn mower, but other household activities were not of interest to Marcus.Currently, he enjoys puzzles, reading, and board games. He likes to play with other children and can interact with familiar adults. Marcus is reported to initiate social interactions, although he has difficulty in understanding personal space. Imaginative play is preferred over other types. He seeks out adult attention and will bring objects over to an adult especially to share his perceived accomplishment. Marcus has difficulty in playing cooperatively with his sister.He is independent with activities of daily living. Marcus is noted to have auditory defensiveness including covering his ears to loud noises and becoming distressed. Parents feel he is immature and inattentive for his age. Marcus responds well when a routine is followed.Previous testing about 2 years ago revealed significant language deficits on the Clinical Evaluation of Language Functioning with average scores on the Woodcock Johnson Achievement Testing and Test of Nonverbal Intelligence Version 3. Marcus was not referred for early intervention and he did not attend preschool. In a regular education Kindergarten, he received speech and occupational therapy along with reading and math support.Comments from teachers or evaluator include the following: Marcus looked to his peers for clues about what he should be doing. Marcus has great difficulty in understanding requests but seems to be interested in pleasing his teacher and others. Marcus' language difficulty makes socialization with his peers problematic; however, he is interested in interacting with them and they seem to accept him willingly. Marcus has intent to communicate with others but relies on visual support to decipher social situations. Marcus has difficulty in attending to details and moves from activity to activity quickly. His short attention span is likely impacting not only learning but also his ability to socially interact with peers.On the day you see him for his 7-year-old checkup, he brought many toys over to show his father and interrupted your conversation to get your attention intermittently throughout the examination. He immediately pointed out a lit ceiling tile with Nemo illuminated to show his father. Marcus does not have any notable or significant repetitive motor mannerisms or stereotypies reported or observed. Marcus' gesture use was appropriate for age and included both symbolic (directing eye gaze and pointing) and concrete (hands up to be picked up and touching an item rather than pointing to it) gestures. Play observed today, although immature for age, was novel, imaginative, and functional. Answers to questions did not always match the question posed. He had a difficult time waiting for his turn before interrupting a conversation. Visual cues were helpful in understanding what was expected of him and what was going on socially.Marcus' speech is notable for persistent stuttering and difficulty in turn-taking in conversation. He gets frustrated easily and has a hard time being understood. He continues to confuse pronouns and makes some grammatical errors. He is able to follow simple directions but has a hard time following complex or multistep directions with accuracy. Nonverbal communication includes pointing to objects of interest in order to share the experience (\"Look mom!\"). He will point to identify an object and can follow a point across the room. He is able to use his eye contact to direct yours to moderate social interactions.Marcus has a special interest in Thomas the Tank Engine Train and Disney movies but is able to move away from those topics to engage in other play interests. Repetitive behaviors are not noted. Toe walking, hand flapping, or spinning, or unusual hand motions or observation of objects were not observed.Difficulties noted today include delays in his receptive and expressive language, poor intelligibility, dysfluency, and impaired motor planning. He recently underwent an audiogram which was normal. You decide to refer to a specialist for further evaluation."}, {"id": "wiki20220301en082_44336", "title": "Fred Olen Ray", "score": 0.008849557522123894, "content": "Evil Toons (1992) was a comedy-horror, then he co-directed another with Wynorski, Dinosaur Island (1994). Witch Academy (1994) was the last of his \"scream queen\" movies. After Attack of the 60 Foot Centerfold (1995), he made Fugitive Rage (1996), Friend of the Family II (1996), Inferno (1997), Hybrid (1997), and The Shooter, which has been referred to as Ray's best film. Dear Santa (1998) was a family film and Billy Frankenstein (1998) a comedy. 2000s to present In 2001 he made the film Critical Mass. He later said he was a \"Critical Mass kind of guy. I like to shoot things and blow stuff up. I also like comedies. Don't like erotic thrillers.\" He established a DVD company called Retromedia. Ray made a film called Bikini Airways \"on a lark and it did really well\", said Ray. This led to a series of Bikini films. In 2007 he reflected on his career:"}, {"id": "wiki20220301en028_33457", "title": "Bone fracture", "score": 0.008771929824561403, "content": "Rib fracture Sternal fracture Shoulder fracture Clavicle fracture Scapular fracture Arm fracture Humerus fracture (fracture of upper arm) Supracondylar fracture Holstein-Lewis fracture – a fracture of the distal third of the humerus resulting in entrapment of the radial nerve Forearm fracture Ulnar fracture Monteggia fracture – a fracture of the proximal third of the ulna with the dislocation of the head of the radius Hume fracture – a fracture of the olecranon with an associated anterior dislocation of the radial head Radius fracture Essex-Lopresti fracture – a fracture of the radial head with concomitant dislocation of the distal radio-ulnar joint with disruption of the interosseous membrane Distal radius fracture Galeazzi fracture – a fracture of the radius with dislocation of the distal radioulnar joint Colles' fracture – a distal fracture of the radius with dorsal (posterior) displacement of the wrist and hand"}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 159]], "word_ranges": [[0, 22]], "text": "Answer 2 incorrect: Graves' disease is characterized by hyperthyroidism and one or more of the following features: goiter, exophthalmos and pretibial myxedema."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[160, 293]], "word_ranges": [[22, 45]], "text": "Answer 4 incorrect: The most obvious symptom of subacute thyroiditis is neck pain. Sometimes, the pain may extend to the jaw or ears."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Answer 2 incorrect: Graves' disease is characterized by hyperthyroidism and one or more of the following features: goiter, exophthalmos and pretibial myxedema. Answer 4 incorrect: The most obvious symptom of subacute thyroiditis is neck pain. Sometimes, the pain may extend to the jaw or ears. Painful enlargement of the thyroid gland can last for weeks or months. And you usually have low serum thyroid stimulating hormone (TSH). High serum free T4 level. High serum thyroglobulin level. Incorrect answer 4: Very rare tumor.", "full_answer_no_ref": "Answer 2 [HIDDEN]: Graves' disease is characterized by hyperthyroidism and one or more of the following features: goiter, exophthalmos and pretibial myxedema. Answer 4 [HIDDEN]: The most obvious symptom of subacute thyroiditis is neck pain. Sometimes, the pain may extend to the jaw or ears. Painful enlargement of the thyroid gland can last for weeks or months. And you usually have low serum thyroid stimulating hormone (TSH). High serum free T4 level. High serum thyroglobulin level. [HIDDEN]: Very rare tumor.", "full_question": "A 30-year-old woman comes to the clinic reporting anxiety, weight loss of about 6 kg and a feeling of \"nervousness\" in the last three months. In the physical examination she has tachycardia, hyperreflexia and absence of goiter. In the blood test TSH values are < 0.01 microU/mL, T4 is elevated and thyroglobulin levels are low. A scintigraphy shows an absence of uptake in the thyroid region. What seems to you the most likely diagnosis?", "id": 327, "lang": "en", "options": {"1": "Factitious thyrotoxicosis.", "2": "Hyperthyroidism due to Graves' disease.", "3": "Ovarian teratoma (ovarian stromal tumor).", "4": "Subacute thyroiditis.", "5": NaN}, "question_id_specific": 81, "type": "ENDOCRINOLOGY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0534_2003", "title": "RET/PTC3 rearrangement and thyroid differentiation gene analysis in a struma ovarii fortuitously revealed by elevated serum thyroglobulin concentration.", "score": 0.019237883832778392, "content": "Struma ovarii (SO) is usually asymptomatic and only in a few cases it is associated with thyrotoxicosis. The presurgical diagnosis is very uncommon. In the majority of cases a pelvic mass is discovered at physical examination or by abdominal ultrasound. Only the hystopathologic examination is able to reveal the characteristic features of SO, with thyroid cells organized in follicles as the main tumoral tissue constituent. The histologic recognition of malignancy is not easy and usually requires an exhaustive sampling of the lesion to evaluate the extracapsular invasion. We report the case of a 59-year-old woman who came to our observation for the fortuitous finding of elevated serum thyroglobulin (Tg) levels (600-800 ng/mL). Because the thyroid function was normal and the ultrasound showed only a subcentrimetric nodule, the clinical suspicious of a SO was considered. Ultrasound examination of the abdomen showed a solid mass of 2 cm in the left ovary. A (131)I uptake was observed at scintiscan in the site of the solid mass. Three months after the resection of the left ovary serum Tg levels were markedly reduced (106 ng/mL), and its values continued to decrease down to 34 ng/mL at last control. The histology showed that the ovarian mass was mainly constituted of thyroid tissue (98%), with no malignant features. The molecular analysis of several thyroid differentiation gene mRNAs in the SO tissue showed an abundant expression of all genes but pendrin (PDS). A reduced PDS mRNA expression might explain the defective thyroxine (T(4)) production. Despite the absence of malignant features, the expression of RET/PTC3 rearrangement was found, raising the possibility of a potential malignant nature of the tumor. A cancer-free period of 3-4 years, as in our patient, is not long enough to definitively exclude a late onset metastatic disease but, unfortunately, the patient died of nonmedical reasons. In conclusion, we report a case of SO that, to our knowledge, is the first in which the clinical suspicion arose from the inappropriately elevated presurgical serum levels of Tg. A quite exhaustive molecular analysis of thyroid specific genes and oncogenes provided two interesting findings: the low PDS mRNA expression, which may explain the low hormonal production and the absence of thyrotoxicosis and the presence of a RET/PTC3 rearrangement, which prompts the possibility of a late malignant evolution."}, {"id": "pubmed23n1090_24702", "title": "First case report of papillary thyroid carcinoma arising within a functional teratoma in Graves' disease patient.", "score": 0.017022792022792024, "content": "Mature cystic teratoma is the most common kind of ovarian germ tumor. However, malignant transformation is uncommon, differentiated thyroid carcinoma is even rare. Hyperthyroidism due to coexistence of Graves' disease (GD) and struma ovarii has been reported. Functional teratoma with papillary thyroid carcinoma (PTC) in GD case has never been reported in literature. A 48-year-old woman with GD for 4 years, who visited our hospital with complaints of severe abdominal pain for 1 day. Computed tomography of the abdominal revealed a large fat-containing lesion with dense calcification, measured 8.6 × 7.2 cm in size. Laparotomy right total oophorectomy was performed, and a huge gangrenous right ovary was noted during exploration. The final pathological diagnosis was teratoma with PTC change at right ovary. We performed thyroglobulin, TTF-1 and CK19 staining in the teratoma, the results were positive, suggesting the thyroid-hormone secretion in the PTC tissue. After resection of the ovarian lesion, euthyroidism was achieved. Adjuvant thyroidectomy is not performed for no evidence of thyroid lesion or distant metastases. No GD recurrence in the 2 years after operation. The patient also does not manifest any gynecological disease symptoms, whereas the other ovary, in the follow-up ultrasound examinations, shows normal size and echo structure. PTC can arise within ovarian teratoma and may have thyroid hormone production. Surgeries of unilateral oophorectomy or cystectomy are a reasonable treatment, and follow-up of thyroid image and data is necessary."}, {"id": "pubmed23n0963_754", "title": "Hyperthyroidism due to struma ovarii: Diagnostic pitfalls and preventing thyroid storm.", "score": 0.01655805293257082, "content": "We report struma ovarii in a case that had hyperthyroidism and was treated with laparoscopic tumor resection. A 40-year-old Japanese woman presented with tachycardia, finger tremor, and weight loss. Although blood examination showed hyperthyroidism, test results for thyroid stimulating hormone receptor antibody and thyroid stimulating antibody were negative, and thyroid scintigraphy showed no abnormal findings. Because she was diagnosed with an ovarian tumor, and whole-body scintigraphy showed that iodine uptake was detected in the pelvic space, we diagnosed her with an ovarian tumor, which caused excessive thyroid hormone secretion. After controlling the thyroid hormone level, we resected the ovarian tumor laparoscopically. The thyroid hormone level was within the normal range postoperatively without any medications. Based on our experience, physicians need to remember that ovarian tumors can cause hyperthyroidism. Controlling the thyroid hormone level preoperatively by using antithyroid drugs and performing minimally invasive laparoscopic surgery is considered useful for preventing thyroid storm."}, {"id": "pubmed23n1097_19359", "title": "Hyperthyroidism associated with <i>struma ovarii</i> - a case report and review of literature.", "score": 0.016516816516816517, "content": "Hyperthyroidism is a state characterized by elevated serum level of thyroid hormones: thyroxine (T4) and triiodothyronine (T3). This is mainly related to the condition and functioning of the thyroid gland. In 60-80% of cases elevation of these hormones are caused by Grave's disease. Thyrotoxicosis is an extreme presentation of hyperthyroidism which can, in rare cases, be caused by excessive synthesis of thyroxine by tumor cells. Struma ovarii is a rare ovarian teratoma composed of thyroid tissue in more than 50%. To present a case of a 30-year-old female patient with a past history of Grave's disease treated by strumectomy 7 years prior; now presenting for the assessment of secondary amenorrhea. Pelvic ultrasound revealed bilateral solid tumors on the left and right ovary, respectively measuring 5 cm and 6 cm in diameter. Her clinical presentation was suggestive of overt hyperthyroidism, and she presented with a significantly elevated CA-125 (152.7 U/mL). The patient subsequently underwent a bilateral oophorectomy in which both masses were excised and histopathological examination confirmed teratoma maturum cysticum. Struma ovarii was noted as a component of the left ovary teratoma. Establishing a proper diagnosis of hyperthyroidism and elucidating its origin is often challenging. Struma ovarii is a rare cause of hyperthyroidism but should always be considered in case of treatment resistant hyperthyroidism. This case-report lends itself as an example of the value in maintaining gynecological-endocrinological knowledge in the setting if clinical gynecology."}, {"id": "pubmed23n0297_14548", "title": "The many causes of subclinical hyperthyroidism.", "score": 0.016443850267379677, "content": "Subclinical hyperthyroidism, defined as serum thyroid hormone levels in the reference range with low serum TSH concentration, is a well recognized clinical entity, but little information is available concerning the prevalence of the disorders that produce it. We conducted a 24-month retrospective survey of subclinical hyperthyroidism patients referred to a university hospital nuclear medicine service for diagnostic studies. Twenty-four consecutive patients were evaluated (22 outpatients and 2 inpatients). All patients had highly sensitive TSH determination, thyroid hormone levels, radioiodine uptake and scan (except for 2 postpartum women), and, selectively, TSH-receptor antibody (TRAb), serum thyroglobulin, antithyroid antibodies, T3-suppression test, and erythrocyte sedimentation rate. A TSH value of about 0.1 microIU/mL was used as the cutoff. Only one patient in the group had a nonthyroidal disorder. In 14 patients (61%) subclinical hyperthyroidism was self-limited, due to silent thyroiditis (5 patients), iodine-induced hyperthyroidism (3 patients), postpartum thyroiditis (2 patients), subacute thyroiditis (2 patients), and probable hemorrhage into a functioning nodule (2 patients). Of the non-self-limited disorders (39%), Graves' disease accounted for 6 patients and solitary or multinodular goiter for 3. Graves' disease proved difficult to diagnose because the thyroid gland was normal in size in two of the six patients, TRAb was positive in only two of six, and the radioiodine uptake and gradient were normal in all six; the T3-suppression test was positive in two of two patients. We conclude that the causes of subclinical hyperthyroidism are the disorders that commonly produce overt thyrotoxicosis in medical practice, Graves' disease being the most frequent. However, the tests used to diagnose overt Graves' disease often fail in the setting of subclinical hyperthyroidism, except possibly the T3-suppression test."}, {"id": "pubmed23n0498_12183", "title": "Coexistence of malignant struma ovarii and Graves' disease.", "score": 0.016073829605182742, "content": "To report an unusual case of hyperthyroidism from Graves' disease that was coexistent with malignant struma ovarii. We summarize the clinical history, physical findings, laboratory data, imaging studies, pathologic features, and treatment in a patient with recurrent hyperthyroidism and discuss the incidence of ovarian tumors of various histologic origins, including thyroid tissue (that is, struma ovarii). Five years after diagnosis of Graves' disease and resolution of symptoms with 1 year of antithyroid drug therapy, a 53-year-old woman had recurrence of palpitations, tremors, and weight loss. Results of thyroid function tests showed high total and free thyroxine levels and a low thyrotropin level. Thyroid radioiodine uptake was high (69% at 24 hours). Abdominal ultrasound studies disclosed a cystic mass in the right adnexal area. Total abdominal hysterectomy and bilateral oophorectomy revealed a 7.5-cm cystic right ovary that contained a 1.0-cm struma ovarii with a 0.4-cm nodule of follicular variant papillary thyroid carcinoma within it. The patient was treated with methimazole and radioiodine ablation of the thyroid. Three months later, a massive myocardial infarction resulted in her death. The concomitant presence of Graves' disease complicates the management of struma ovarii and raises interesting questions about treatment and prognosis."}, {"id": "pubmed23n0575_2431", "title": "[Particular evolution of the thyroid state in Grave's disease: two cases].", "score": 0.015763076188201577, "content": "We report two cases of Grave's disease (GD) caracterized by the succession of hypothyroid and hyperthyroid states. Case 1: A 32 years old woman, has presented initially a typical GD with hyperthyroidism. Grave's ophtalmopathy and homogenous goiter. Four months later, she presented a spontaneous hypothyroidism necessiting treatment with thyroxine and a severe myasthenia gravis. More later (6 months), she experienced symptoms of hyperthyroidism after thymectomy. The level of anti-thyrotropin-receptor antibodies (TSab) was very high (141 UI/I, NV &lt; 10). Case 2: A 29 years old woman has been treated by thyroxine (150 microg/day) for a primary hypothyroidism. Ten months later, she presented symptoms of hyperthyroidism even after stoppage of thyroxine. TSH value was decreased (TSH &lt; 0.05 microU/ml) and FT4 level was raised (FT4 = 25.5 pmol/l). The thyroid antibodies were positive. We discuss, after review of the litterature, the physiopathological mecanisms of these changes in the thyroid state, particularly the role of the blocking and stimulating anti-thyrotropin-receptor antibodies."}, {"id": "pubmed23n1080_12085", "title": "Resolution of hyperthyroidism and thyroid antibodies following struma ovarii resection: an uncommon entity.", "score": 0.014183708165347062, "content": "We report a case of 34-year-old clinically asymptomatic woman who had been followed for 6 years for hyperthyroidism with thyroid stimulating hormone &lt;0.006 uIU/mL, free T4 1.98 ng/mL, free T3 5.3 pg/mL, elevated thyroid stimulating immunoglobulin 1.70 IU/L, thyroid peroxidase antibody 38 IU/mL and thyroglobulin antibody 9.3 IU/mL. Radioiodine thyroid scan showed minimal uptake in both thyroid lobes (24-hour uptake was 0.3%). She subsequently underwent evaluation for lower abdominal pain and menstrual irregularities, which revealed a large left ovarian cyst measuring 15.9 cm × 10.8 cm × 13.2 cm and right-sided ovarian cyst measuring 2.7 cm × 3.3 cm × 3.5 cm. Laparoscopic bilateral ovarian cystectomy was performed and the final pathology revealed struma ovarii of the left ovarian cyst with the entire ovarian tumour made up of benign thyroid tissue. Thyroid function tests performed 3 months after surgical removal of struma ovarii showed euthyroidism. We present a rare case with detailed laboratory and immunological data before and after ovarian extirpation with resolution of hyperthyroidism associated with functional struma ovarii."}, {"id": "wiki20220301en000_205311", "title": "Hyperthyroidism", "score": 0.012684928340268145, "content": "Measuring specific antibodies, such as anti-TSH-receptor antibodies in Graves' disease, or anti-thyroid peroxidase in Hashimoto's thyroiditis—a common cause of hypothyroidism—may also contribute to the diagnosis. The diagnosis of hyperthyroidism is confirmed by blood tests that show a decreased thyroid-stimulating hormone (TSH) level and elevated T4 and T3 levels. TSH is a hormone made by the pituitary gland in the brain that tells the thyroid gland how much hormone to make. When there is too much thyroid hormone, the TSH will be low. A radioactive iodine uptake test and thyroid scan together characterizes or enables radiologists and doctors to determine the cause of hyperthyroidism. The uptake test uses radioactive iodine injected or taken orally on an empty stomach to measure the amount of iodine absorbed by the thyroid gland. Persons with hyperthyroidism absorb much more iodine than healthy persons which includes radioactive iodine which is easy to measure. A thyroid scan"}, {"id": "wiki20220301en001_156556", "title": "Thyroid", "score": 0.012683916793505834, "content": "An examination of the thyroid will also include observation of the person as a whole, to look for systemic signs such as weight gain or loss, hair loss, and signs in other locations – such as protrusion of the eyes or swelling of the calves in Graves' disease. Tests Thyroid function tests include a battery of blood tests, including the measurement of the thyroid hormones, as well as the measurement of thyroid stimulating hormone (TSH). They may reveal hyperthyroidism (high T3 and T4), hypothyroidism (low T3, T4), or subclinical hyperthyroidism (normal T3 and T4 with a low TSH)."}, {"id": "pubmed23n0668_12653", "title": "Expression of benign and malignant thyroid tissue in ovarian teratomas and the importance of multimodal management as illustrated by a BRAF-positive follicular variant of papillary thyroid cancer.", "score": 0.012534872167233539, "content": "The most common type of ovarian germ cell tumor is the teratoma. Thyroid tissue, both benign and malignant, may be a component of an ovarian teratoma. Here we review this topic and illustrate major features by presenting multimodal management of a patient with BRAF-positive disseminated follicular thyroid cancer arising in an ovarian teratoma. Malignant thyroid tissue is often difficult to distinguish from benign thyroid tissue arising in ovarian teratomas. Preoperatively, an elevated thyroglobulin (Tg) level, laboratory or clinical evidence of hyperthyroidism, or ultrasonography appearance of \"struma pearl\" should prompt referral to oncologist for surgical management of a possibly malignant ovarian teratoma. Postoperatively, tumor tissue should be referred to pathologists experienced with differentiating benign from malignant struma ovarii. Once diagnosed, treatment of this rare condition should be handled by a team of specialists with combined treatment modalities. We cared for woman with disseminated thyroid cancer arising in an ovarian teratoma whose history illustrates the complexity of managing ovarian teratomas with malignant thyroid tissue. At age 33 she had an intraoperative rupture of an ovarian cyst, thought to be struma ovarii. During her next pregnancy, pelvic masses were noted; biopsies revealed well-differentiated papillary thyroid carcinoma, follicular variant. She was euthyroid, but had elevated serum Tg levels. Surgical staging demonstrated widely metastatic intraabdominal dissemination. A thyroidectomy revealed no malignancy. A post-(131)I treatment scan revealed diffuse uptake throughout the abdomen. She then developed abdominal pain and, on computed tomography, was found to have multiple intraabdominal foci of disease. Serum Tg was 264 ng/mL while on L-thyroxine for hypothyroidism and to obtain thyrotropin suppression. A 18 fluorodeoxyglucose positron emission tomography scan showed no pathological uptake. The tumor was found to be BRAF mutation positive (K601E). She underwent extensive secondary debulking and a second course of (131)I with lithium pretreatment. Posttreatment scan revealed diffuse abdominal uptake. Six months posttherapy, the patient is asymptomatic with a serum Tg of 18.1 ng/mL. Aggressive multimodal management appears to be the most promising approach for malignant thyroid tissue arising in ovarian teratomas."}, {"id": "pubmed23n1116_12785", "title": "Malignant Struma Ovarii with Concurrent Thyroid Cancer: Outcomes during and after Pregnancy.", "score": 0.012237762237762238, "content": "Struma ovarii (SO) is a rare ovarian teratoma characterized by the presence of thyroid tissue in more than 50% of the tumor. Malignant transformation is rare and the most common associated malignancy is papillary thyroid carcinoma (PTC). Pregnancy may represent a stimulus to differentiated thyroid cancer (DTC) growth in patients with known structural or biochemical evidence of disease, but data about malignant SO evolution during pregnancy are rare. We present the first reported case of a pregnant patient with malignant SO and biochemical evidence of disease. A previously healthy 35-year-old female diagnosed with a suspicious left pelvic mass on routine ultrasound was submitted to laparoscopic oophorectomy which revealed a malignant SO with areas of PTC. A 15-mm thyroid nodule (Bethesda V in the fine-needle aspiration cytology) was detected by palpation and total thyroidectomy was performed. Histology revealed a 15 mm follicular variant of PTC (T1bNxMx). Subsequently, she received 100 mCi of radioactive iodine therapy (RAIT) with the whole-body scan showing only moderate neck uptake. Her suppressed thyroglobulin (Tg) before RAI was 1.1 ng/mL. She maintained biochemical evidence of disease, with serum Tg levels of 7.6 ng/mL. She got pregnant 14 months after RAIT, and during pregnancy, Tg increased to 21.5 ng/mL. After delivery, Tg decreased to 14 ng/mL but, 6 months later, rose again and reached 31.9 ng/mL on the last follow-up visit. TSH was always suppressed during follow-up. At the time of SO diagnosis, a chest computed tomography scan showed 4 bilateral lung micronodules in the upper lobes which were nonspecific, and 9 months after diagnosis, a pelvic MRI revealed a suspicious cystic nodule located on the oophorectomy bed. These lung and pelvic nodules remained stable during follow-up. Neck ultrasonography, abdominal MRI, and fluorodeoxyglucose-positron emission tomography showed no suspicious lesions. As for DTC, pregnancy seems to represent a stimulus to malignant SO growth. This can be caused by the high levels of estrogen during pregnancy that may bind to receptors in malignant cells and/or by the high levels of hCG which is known to stimulate TSH receptors."}, {"id": "wiki20220301en000_205296", "title": "Hyperthyroidism", "score": 0.012048474639715517, "content": "Graves' disease is the cause of about 50% to 80% of the cases of hyperthyroidism in the United States. Other causes include multinodular goiter, toxic adenoma, inflammation of the thyroid, eating too much iodine, and too much synthetic thyroid hormone. A less common cause is a pituitary adenoma. The diagnosis may be suspected based on signs and symptoms and then confirmed with blood tests. Typically blood tests show a low thyroid stimulating hormone (TSH) and raised T3 or T4. Radioiodine uptake by the thyroid, thyroid scan, and TSI antibodies may help determine the cause."}, {"id": "wiki20220301en024_81643", "title": "Hashimoto's thyroiditis", "score": 0.011827183970687136, "content": "Given the relatively nonspecific symptoms of initial hypothyroidism, Hashimoto's thyroiditis is often misdiagnosed as depression, cyclothymia, premenstrual syndrome, chronic fatigue syndrome, fibromyalgia, and less frequently, as erectile dysfunction or an anxiety disorder. On gross examination, a hard goiter that is not painful to the touch often presents; other symptoms seen with hypothyroidism, such as periorbital myxedema, depend on the current state of progression of the response, especially given the usually gradual development of clinically relevant hypothyroidism. Testing for thyroid-stimulating hormone (TSH), free T3, free T4, and the antithyroglobulin antibodies (anti-Tg), antithyroid peroxidase antibodies (anti-TPO, or TPOAb) and antimicrosomal antibodies can help obtain an accurate diagnosis. Earlier assessment of the person may present with elevated levels of thyroglobulin owing to transient thyrotoxicosis, as inflammation within the thyroid causes damage to the integrity"}, {"id": "InternalMed_Harrison_26822", "title": "InternalMed_Harrison", "score": 0.011514089948997991, "content": "Laboratory Evaluation As depicted in Fig. 405-10, thyroid function tests characteristically evolve through three distinct phases over about 6 months: (1) thyrotoxic phase, (2) hypothyroid phase, and (3) recovery phase. In the thyrotoxic phase, T4 and T3 levels are increased, reflecting their discharge from the damaged thyroid cells, and TSH is suppressed. The T4/T3 ratio is greater than in Graves’ disease or thyroid autonomy, in which T3 is often disproportionately increased. The diagnosis is confirmed by a high ESR and low uptake of radioiodine (<5%) or 99mTc pertechnetate (as compared to salivary gland pertechnetate concentration). The white blood cell count may be increased, and thyroid antibodies are negative. If the diagnosis is in doubt, FNA biopsy may be useful, particularly to distinguish unilateral involvement from bleeding into a cyst or neoplasm."}, {"id": "pubmed23n1162_13604", "title": "Strumal Carcinoid Tumor of the Ovary: Report of Rare Occurrence with Review of Literature.", "score": 0.011296208154846898, "content": "The primary ovarian carcinoid tumor is a very rare ovarian tumor, which accounts for approximately 0.5% to 1.7% of all carcinoids and 1% of ovarian cancer. According to its histopathological features, it can be divided into four categories: insular, trabecular, strumal, and mucinous, among which insular carcinoid is common in Western countries. By comparison, the chain-typed and trabecular carcinoid seem to be common in Asian countries. To date, about 150 cases have been reported in the world, and 40% of them are strumal carcinoid tumor of the ovary (SCTO), which is a highly specialized teratoma differentiated from the monomer, and often characterized by the coexistence of thyroid follicular tissue and carcinoid tissue with the neuroendocrine function. Preoperative diagnosis may be difficult due to the very insidious nature of the disease and its multiple imaging manifestations. We reported the case of a 39-year-old woman with a 5-year clinical history. Gynecologic examination and ultrasonic testing revealed an enlarged ovary with a diameter of about 60 mm, accompanied by a hypoechoic area, which was suspected to be a benign teratoma. Ca-125, AFP, free T4, TSH, and other diagnostic indicators were normal. During the laparoscopic oophorocystectomy of the left ovary, a smooth and solid tumor with the size of 6 × 6 × 5 cm was found in the right ovary. During the operation, a mature cystic teratoma containing a struma was frozen, then the oophorocystectomy of the left ovary was performed. According to the Federation International of Gynecology and Obstetrics (FIGO) in 2014, histopathological examination showed a mature teratoma with thyroid carcinoid stage Ic, and Douglas's cystic hygroma cytopathology was negative. One year after the operation, the patient was tumor-free, with Ca-125, FT4, and TSH being within the normal range. Specific diagnostic tools and serological monitoring of malignant tumors of the ovary have low specificity and sensitivity in the diagnosis of this rare malignant tumor of the ovary. Female patients with habitual constipation, chronic abdominal colic, diarrhea, and endocrine dysfunction also need to be alert to this rare malignant tumor of the ovary."}, {"id": "article-23323_22", "title": "I-123 Uptake -- Normal and Critical Findings -- Thyrotoxicosis of Extrathyroidal Origin", "score": 0.011151942020109221, "content": "There are multiple sources of extrathyroidal thyroid hormone, including exogenous thyroid hormone ingestion seen in factitious hyperthyroidism, thyroid hormone-producing metastatic thyroid cancer, and struma ovarii, a teratomatous ovarian mass that contains functional thyroid tissue. Another extrathyroidal cause of thyrotoxicosis includes TSH-induced thyrotoxicosis, which is caused by an autonomous TSH secreting pituitary adenoma, the only extrathyroidal cause associated with an elevated RAIU. Clinically, patients can have varying levels of thyrotoxicosis, depending on the level of excretion the exogenous source releases. Factitious hyperthyroidism can often be difficult to diagnose as the patients rarely disclose exogenous thyroid ingestion. Oftentimes the ingested thyroid hormone is T4, which may yield a higher than normal T4 to T3 ratio. Thyroid hormone production from metastatic thyroid malignancy is not very common as it requires a well-differentiated metastatic malignancy, which is usually less efficient at producing and excreting thyroid hormone. These metastases are usually seen in the setting of known thyroid cancer on I-131 imaging. Similar to metastasis, struma ovarii does not efficiently excrete thyroid hormone and rarely produces significant amounts of thyroid hormone. Struma ovarii is often found incidentally when pathology of an ovarian mass returns with thyroid tissue. RAIU of the overall thyroid gland in the neck is decreased, while the lesion may have elevated RAIU. [11]"}, {"id": "InternalMed_Harrison_26780", "title": "InternalMed_Harrison", "score": 0.01070351036827738, "content": "Primary thyrotoxicosis Features of Graves’ diseasea? T3 toxicosis Subclinical hyperthyroidism TSH low, unbound T4 high Measure TSH, unbound T4 Normal Measure unbound T3 TSH normal or increased, high unbound T4 TSH-secreting pituitary adenoma or thyroid hormone resistance syndrome Follow up in 6-12 weeks High TSH low, unbound T4 normal TSH and unbound T4 normal No further tests Graves’ disease Multinodular goiter or toxic adenomab? Yes No Toxic nodular hyperthyroidism Low radionuclide uptake? Yes No Destructive thyroiditis, iodine excess or excess thyroid hormone Rule out other causes including stimulation by chorionic gonadotropin"}, {"id": "wiki20220301en073_42718", "title": "Thyroiditis", "score": 0.01066830896376351, "content": "Diagnosis The most common and helpful way to diagnose thyroiditis is first for a physician to palpate the thyroid gland during a physical examination. Laboratory tests allow doctors to evaluate the patient for elevated erythrocyte sedimentation rates, elevated thyroglobulin levels, and depressed radioactive iodine uptake (Mather, 2007). Blood tests also help to determine the kind of thyroiditis and to see how much thyroid stimulating hormone the pituitary gland is producing and what antibodies are present in the body. In some cases, a biopsy may be needed to find out what is attacking the thyroid. Classification Thyroiditis is a group of disorders that all cause thyroidal inflammation. Forms of the disease are Hashimoto's thyroiditis, the most common cause of hypothyroidism in the US, postpartum thyroiditis, subacute thyroiditis, silent thyroiditis, drug-induced thyroiditis, radiation-induced thyroiditis, acute thyroiditis, and Riedel's thyroiditis."}, {"id": "InternalMed_Harrison_26785", "title": "InternalMed_Harrison", "score": 0.010611205432937181, "content": "Differential Diagnosis Diagnosis of Graves’ disease is straightforward in a patient with biochemically confirmed thyrotoxicosis, diffuse goiter on palpation, ophthalmopathy, and often a personal or family history of autoimmune disorders. For patients with thyrotoxicosis who lack these features, the diagnosis is generally established by a radionuclide (99mTc, 123I, or 131I) scan and uptake of the thyroid, which will distinguish the diffuse, high uptake of Graves’ disease from destructive thyroiditis, ectopic thyroid tissue, and factitious thyrotoxicosis. Scintigraphy is the preferred diagnostic test; however, TRAb measurement can be used to assess autoimmune activity. In secondary hyperthyroidism due to a 2296 TSH-secreting pituitary tumor, there is also a diffuse goiter. The presence of a nonsuppressed TSH level and the finding of a pituitary tumor on CT or magnetic resonance scan (MRI) scan suggest this diagnosis. Clinical features of thyrotoxicosis can mimic certain aspects of other"}, {"id": "wiki20220301en072_8770", "title": "Thyroid disease", "score": 0.010493465147453083, "content": "In some types, such as subacute thyroiditis or postpartum thyroiditis, symptoms may go away after a few months and laboratory tests may return to normal. However most types of thyroid disease do not resolve on their own. Common hypothyroid symptoms include fatigue, low energy, weight gain, inability to tolerate the cold, slow heart rate, dry skin and constipation. Common hyperthyroid symptoms include irritability, anxiety, weight loss, fast heartbeat, inability to tolerate the heat, diarrhea, and enlargement of the thyroid. Structural abnormalities may not produce symptoms, however some people may have hyperthyroid or hypothyroid symptoms related to the structural abnormality or notice swelling of the neck. Rarely goiters can cause compression of the airway, compression of the vessels in the neck, or difficulty swallowing. Tumors, often called thyroid nodules, can also have many different symptoms ranging from hyperthyroidism to hypothyroidism to swelling in the neck and compression"}, {"id": "Gynecology_Novak_3696", "title": "Gynecology_Novak", "score": 0.010038967043127931, "content": "Diffuse toxic goiter (Grave’s disease) is the most common cause of hyperthyroidism and results from abnormal stimulation of the thyroid gland by antithyroid antibodies. Other causes of hyperthyroidism include multinodular goiter, excess thyroid hormone, or thyroiditis. Any signs or symptoms suggestive of weight loss, tachycardia, atrial fibrillation, goiter, or proptosis should initiate a more extensive laboratory evaluation of thyroid function. Total thyroxin, free triiodothyronine (T3), free thyroxin (T4), and thyroid-stimulating hormone (TSH) tests are useful in diagnosis. In hyperthyroidism, the free T4 level will be elevated, and the TSH level will be suppressed (154). A new diagnosis of hyperthyroidism necessitates postponement of elective surgery until adequate treatment with antithyroid medication is received because of the risk of thyroid storm. Thyroid storm is associated with mortality of up to 40% (168). Stable thyroid conditions do not require any special preoperative"}, {"id": "wiki20220301en000_205310", "title": "Hyperthyroidism", "score": 0.010026737967914439, "content": "Hypersecretion of thyroid stimulating hormone (TSH), which in turn is almost always caused by a pituitary adenoma, accounts for much less than 1 percent of hyperthyroidism cases. Diagnosis Measuring the level of thyroid-stimulating hormone (TSH), produced by the pituitary gland (which in turn is also regulated by the hypothalamus's TSH Releasing Hormone) in the blood is typically the initial test for suspected hyperthyroidism. A low TSH level typically indicates that the pituitary gland is being inhibited or \"instructed\" by the brain to cut back on stimulating the thyroid gland, having sensed increased levels of T4 and/or T3 in the blood. In rare circumstances, a low TSH indicates primary failure of the pituitary, or temporary inhibition of the pituitary due to another illness (euthyroid sick syndrome) and so checking the T4 and T3 is still clinically useful."}, {"id": "Gynecology_Novak_5694", "title": "Gynecology_Novak", "score": 0.009907583034020744, "content": "Thyroid-Stimulating Receptor Antibody in Graves Disease The level of TSHRAb of the TBII class grossly parallels the degree of hyperthyroidism as assessed by the serum levels of thyroid hormones and total thyroid volume. Studies suggest that the combination of a small goiter volume (<40 mL) and a low TBII level (<30 U/L) results in a 45% chance of remission during the 5 years after completion of a 12-to 24-month course of antithyroid drug therapy (397). In contrast, the overall rate of relapse exceeded 70% in patients with a large goiter volume (>70 mL) and a higher TBII level (>30 U/L). The subgroup of patients with larger goiters and higher TBII levels had less than a 10% chance to remain in remission in the 5 years after treatment. Although it is not necessary for the diagnosis of Graves disease, except in some cases of multinodular goiter, a TSHRAb measurement may be a useful marker of disease severity. Used in combination with other clinical factors, it may contribute to initial"}, {"id": "wiki20220301en002_139837", "title": "Graves' disease", "score": 0.009900990099009901, "content": "Another sign of Graves' disease is hyperthyroidism; that is, overproduction of the thyroid hormones T3 and T4. Normal thyroid levels are also seen, and occasionally also hypothyroidism, which may assist in causing goiter (though it is not the cause of the Graves' disease). Hyperthyroidism in Graves' disease is confirmed, as with any other cause of hyperthyroidism, by measuring elevated blood levels of free (unbound) T3 and T4. Other useful laboratory measurements in Graves' disease include thyroid-stimulating hormone (TSH, usually undetectable in Graves' disease due to negative feedback from the elevated T3 and T4), and protein-bound iodine (elevated). Serologically detected thyroid-stimulating antibodies, radioactive iodine (RAI) uptake, or thyroid ultrasound with Doppler all can independently confirm a diagnosis of Graves' disease. Biopsy to obtain histiological testing is not normally required, but may be obtained if thyroidectomy is performed."}, {"id": "Gynecology_Novak_5655", "title": "Gynecology_Novak", "score": 0.009894217768233515, "content": "Because most disorders of hyperthyroidism and hypothyroidism are related to dysfunction of the thyroid gland and TSH levels are sensitive to excessive or deficient levels of circulating thyroid hormone, TSH levels are used to screen for these disorders. Current thyrotropin or TSH sandwich immunoassays are extremely sensitive and capable of differentiating low-normal from pathologic or iatrogenically subnormal values and elevations. TSH measurements provide the best way to screen for thyroid dysfunction and accurately predict thyroid hormone dysfunction in about 80% of cases (343). Reference values for TSH are traditionally based on the central 95% of values for healthy individuals, and some controversy exists regarding the upper limit of normal. Values in the upper limit of normal may predict future thyroid disease (342,344). In a longitudinal study, women with positive thyroid antibodies (TPOAbs or TgAbs), the prevalence of hypothyroidism at follow-up was 12.0% (3.0% to 21.0%; 95%"}, {"id": "wiki20220301en190_18552", "title": "Thyrotoxicosis factitia", "score": 0.00980392156862745, "content": "Patients present with hyperthyroidism and may be mistaken for Graves’ disease, if TSH receptor positive, or thyroiditis because of absent uptake on a thyroid radionuclide uptake scan due to suppression of thyroid function by exogenous thyroid hormones. Ingestion of thyroid hormone also suppresses thyroglobulin levels helping to differentiate thyrotoxicosis factitia from other causes of hyperthyroidism, in which serum thyroglobulin is elevated. Caution, however, should be exercised in interpreting thyroglobulin results without thyroglobulin antibodies, since thyroglobulin antibodies commonly interfere in thyroglobulin immunoassays causing false positive and negative results which may lead to clinical misdirection. In such cases, increased faecal thyroxine levels in thyrotoxicosis factitia may help differentiate it from other causes of hyperthyroidism. See also Foodborne illness Liothyronine References External links Thyroid disease Toxicology"}, {"id": "InternalMed_Harrison_26786", "title": "InternalMed_Harrison", "score": 0.009713551230260742, "content": "nonsuppressed TSH level and the finding of a pituitary tumor on CT or magnetic resonance scan (MRI) scan suggest this diagnosis. Clinical features of thyrotoxicosis can mimic certain aspects of other disorders, including panic attacks, mania, pheochromocytoma, and weight loss associated with malignancy. The diagnosis of thyrotoxicosis can be easily excluded if the TSH and unbound T4 and T3 levels are normal. A normal TSH also excludes Graves’ disease as a cause of diffuse goiter."}, {"id": "wiki20220301en014_128887", "title": "Thyroid-stimulating hormone", "score": 0.009705540488182875, "content": "TSH concentrations in children are normally higher than in adults. In 2002, the NACB recommended age-related reference limits starting from about 1.3 to 19 µIU/mL for normal-term infants at birth, dropping to 0.6–10 µIU/mL at 10 weeks old, 0.4–7.0 µIU/mL at 14 months and gradually dropping during childhood and puberty to adult levels, 0.3–3.0 µIU/mL. Diagnosis of disease TSH concentrations are measured as part of a thyroid function test in patients suspected of having an excess (hyperthyroidism) or deficiency (hypothyroidism) of thyroid hormones. Interpretation of the results depends on both the TSH and T4 concentrations. In some situations measurement of T3 may also be useful. A TSH assay is now also the recommended screening tool for thyroid disease. Recent advances in increasing the sensitivity of the TSH assay make it a better screening tool than free T4. Monitoring The therapeutic target range TSH level for patients on treatment ranges between 0.3 and 3.0 μIU/mL."}, {"id": "pubmed23n0755_2240", "title": "A case of simultaneous occurrence of Graves' disease and Hashimoto's thyroiditis.", "score": 0.009523809523809525, "content": "Simultaneous occurrence of Hashimoto's thyroiditis (HT), and Graves' disease (GD) is rare. We report a case of simultaneous occurrence of GD and HD, at presentation. A 60-year-old lady presented with tremulousness of hands, palpitation, and excessive sweating. She had a history of weight loss and neck-swelling. Her weight was 46 kg, BMI 17, afebrile, regular pulse rate of 110/min with fine tremor in hands. Thyroid gland was symmetrically enlarged, firm, without any bruit, but mildly tender with lobular surface. There were no occular manifestations. Initial thyroid function tests (TFT) revealed: T3: 3.80 ng/ml (0.80-2.10), T4: 12.40 ug/dl (5.10-12), thyroid stimulating hormone (TSH): 0.20 μU/L (0.70-5). Her anti thyroperoxidase (TPO) antibody: 374 IU/ml (normal [nl.] &lt;35) and TSH receptor antibody: 15 U/L (nl. &lt;1) were both strongly positive. Ultrasonogram of thyroid revealed a hypoechoic enlarged gland. 99mTc pertechnetate scan showed an enlarged gland with increased uptake of radiocontrast: 17% (nl. 0.4-4%) with some patchy defects in both lower poles. Thyroid fine needle aspiration cytology (FNAC) showed sheets of Hurthle cells with abdunce of lymphocytes indicating HT. She was observed on beta blockers. Repeat TFT, 3 months later showed: T3: 4.20 ng/ml, T4: 14.40 ug/dl, TSH: 0.001 μU/L, with increased uptake on repeat scan. HT rarely occurs following GD. Our case of an elderly lady with no eye signs, lobular, firm tender goiter with patchy uptake in both lower poles on Tc99m scan were odd points in diagnosing isolated GD. FNAC confirmed simultaneous HD with GD."}, {"id": "pubmed23n0314_8803", "title": "[A case of ovarian struma causing symptoms of hyperthyroidism].", "score": 0.009345794392523364, "content": "The authors report the case of a 46-year old woman, who went with diffuse complaints to the endocrinology department. The laboratory date showed hyperthyroidism. She had hypogastric complaints, therefore a gynecological consultation was carried out. An infant--head sized elastic formation was found on the left hand side next to a female fist sized myomatous uterus. On sonographic examination the growth proved to have a size of 12 x 14 cm. Besides the myomatous uterus the histology of the operative specimen confirmed a left sided ovarious teratoma, which was composed solely of usual thyroid tissue. After the operation it the patient's endocrine disorders became spontaneously regulated. Our attention to called the fact that especially in case of therapy resistant hyperthyroidism we should think of this infrequent clinical picture."}, {"id": "pubmed23n0927_22431", "title": "[An ovarian tumor can hide another one].", "score": 0.009259259259259259, "content": "We report the case of a 33-year-old woman who went under surgery for a cystic mature teratoma. The histological exam found two cysts, one was a mature teratoma and the other was a struma ovarii with a papillary carcinomatous element. Struma ovarii cancerization is seen in 5 to 10% of the cases usually under a papillary carcinoma type. Diagnosis is rarely made before surgery, the patients exceptionally show thyroid symptoms. Histologically, the tumour presents the same way as the one seen in the thyroid gland and BRAF mutations have been reported. The problem concerns ovarian metastases of a thyroid cancer. A normal thyroid check up and normal thyroid tissue close to the tumor in the ovary are in favor for a cancerize struma ovarii. The therapeutic care is not consensual, going from an annexectomy to hysterectomy and bilateral annexectomy. The patients must be followed on long-term with thyroglobulin quantitative analysis for at least 10 years and whole body scintigraphy with iodine 123 to detect relapse or metastases. The prognosis is usually good but precise criteria are still to define."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 166]], "word_ranges": [[0, 20]], "text": "They describe a typical hemisection or Brown-Sequard picture. Ipsilateral posterior motor and chordal involvement with contralateral pain and temperature involvement."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "They describe a typical hemisection or Brown-Sequard picture. Ipsilateral posterior motor and chordal involvement with contralateral pain and temperature involvement.", "full_answer_no_ref": "They describe a typical hemisection or Brown-Sequard picture. Ipsilateral posterior motor and chordal involvement with contralateral pain and temperature involvement.", "full_question": "A man presents on neurological examination with a thermoalgesic sensory deficit in the left leg associated with a loss of vibratory and positional sensitivity in the right leg. At the same time he presents with clumsiness and loss of distal strength in the right leg and a right plantar cutaneous reflex in extension. Which of the following statements is correct:", "id": 450, "lang": "en", "options": {"1": "It is a centromedullary syndrome type syringomyelia.", "2": "It is a hemimedullary syndrome.", "3": "It is a pattern of transverse medullary lesion.", "4": "It is a pattern of lateral bulbar lesion.", "5": NaN}, "question_id_specific": 154, "type": "NEUROSURGERY", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0353_15932", "title": "[Medial medullary infarction: report of three patients presented with central vestibular dysfunction without limb and lingual weakness].", "score": 0.016979638822936904, "content": "The purpose of this article is to draw attention to atypical presentation of medial medullary infarction (MMI). With advanced imaging techniques, small infarctions occurring in the medulla are more easily identified. It is difficult, however, to make a clinical diagnosis of MMI if both hypoglossal nerve palsy and limb weakness are absent, because motor weakness is considered a cardinal manifestation of MMI. We describe here three patients who developed central vestibular dysfunction due to MMI without limb and lingual weakness. Case 1: A 44-year-old, diabetic woman developed vomiting and numbness on her left upper limb. Examination revealed unidirectional horizontal and rotatory nystagmus beating toward the right side. There were no Horner syndrome and hypoglossal nerve palsy. Barré arm and leg signs and limb ataxia were absent. Romberg sign was negative. Hypesthesia was present on her left forearm, hand, and fingers. Thumb-localizing test was normal. Cranial MRI demonstrated an infarction in the right paramedian region of the upper medulla. MR angiography demonstrated irregularity of the basilar and the left vertebral arteries. Case 2: A 69-year-old woman suffered from dizziness and nausea. She showed unidirectional, left-beating horizontal nystagmus. There were no Horner syndrome and hypoglossal nerve palsy, Barré arm and leg signs, and limb ataxia. MRI disclosed an infarction in the left upper medial medulla. Case 3: A 47-year-old man developed vertigo when turning over in bed. He showed left-beating nystagmus without latency, when lying down. Horner syndrome and hypoglossal nerve palsy were absent MRI showed bilateral MMI, with the right lesion being larger than the left. MR angiography demonstrated a stenosis in the distal portion of the left internal carotid artery but not in the vertebral and basilar arteries and their branches. This case represents central positional vertigo. Vestibular syndrome seen in cases 1 and 2 was incomplete and incongruent, suggesting dysfunction of the central vestibular system. There have been only nine cases of MMI with horizontal nystagmus in primary position, including unidirectional horizontorotatory nystagmus. In these cases, horizontal nystagmus beats toward the side of the lesion. In sharp contrast, horizontal nystagmus typically beats away from the lesion side in cases of Wallenberg syndrome, suggesting different underlying mechanism. Unidirectional horizontal and rotatory nystagmus is generally associated with peripheral vestibular dysfunction. There has been no reported case of MMI presenting with vestibular dysfunction preserving motor power. Thus, this \"benign\" form of MMI might have been misdiagnosed as peripheral vestibular dysfunction before the era of MRI."}, {"id": "pubmed23n0326_19936", "title": "[Sensory disturbance of crossed oral-pedal topography in a case of lateral medullary infarction].", "score": 0.014565826330532213, "content": "We report a 40-year-old man with hypertension and diabetes mellitus, who had crossed oral-pedal sensory disturbance in lateral medullary infarction. He suddenly developed dysesthesia in the right mount and the left leg. His blood pressure was 150/90 mmHg. Neurological examination showed Horner's sign in the right eye and horizontal nystagmus. Sensory function revealed decreased temperature, hypalgesia and dysesthesia in the right mouth and the left leg. Vibratory and position sense were normal. T1- and T2-weighted images disclosed a low and high signal intensity area in the lateral portion of the right medulla oblongata, respectively. Brain and neck MRA using time-of-flight sequence revealed no obvious abnormal structures. We have diagnosed him as lateral medullary infarction. The unique topography of sensory dysfunction thought to be attributed to a far-lateral lesion in the medulla oblongata. Our patient suggests that lateral medullary infarction causes variable patterns of sensory disturbance. Thus, lateral medullary infarction should be warranted when we encounter patients with miscellaneous distribution of sensory impairment, such as crossed mouth-foot hypalgesia."}, {"id": "wiki20220301en025_36555", "title": "Lateral medullary syndrome", "score": 0.012568758502395458, "content": "Lateral medullary syndrome is a neurological disorder causing a range of symptoms due to ischemia in the lateral part of the medulla oblongata in the brainstem. The ischemia is a result of a blockage most commonly in the vertebral artery or the posterior inferior cerebellar artery. Lateral medullary syndrome is also called Wallenberg's syndrome, posterior inferior cerebellar artery (PICA) syndrome and vertebral artery syndrome. Signs and symptoms This syndrome is characterized by sensory deficits that affect the trunk and extremities contralaterally (opposite to the lesion), and sensory deficits of the face and cranial nerves ipsilaterally (same side as the lesion). Specifically a loss of pain and temperature sensation if the lateral spinothalamic tract is involved. The cross body finding is the chief symptom from which a diagnosis can be made."}, {"id": "InternalMed_Harrison_30029", "title": "InternalMed_Harrison", "score": 0.011364522417153996, "content": "Signs and symptoms: Structures involved 1. Medial medullary syndrome (occlusion of vertebral artery or of branch of vertebral or lower basilar artery) On side of lesion Paralysis with atrophy of one-half half the tongue: Ipsilateral twelfth nerve On side opposite lesion Paralysis of arm and leg, sparing face; impaired tactile and proprioceptive sense over one-half the body: Contralateral pyramidal tract and medial lemniscus 2. Lateral medullary syndrome (occlusion of any of five vessels may be responsible—vertebral, posterior inferior cerebellar, superior, middle, or inferior lateral medullary arteries) On side of lesion"}, {"id": "wiki20220301en157_31903", "title": "Brown-Séquard syndrome", "score": 0.011191749427043544, "content": "Pure Brown-Séquard syndrome is associated with the following: Interruption of the lateral corticospinal tracts: Ipsilateral spastic paralysis below the level of the lesion Babinski sign ipsilateral to lesion Abnormal reflexes and Babinski sign may not be present in acute injury Interruption of posterior white column: Ipsilateral loss of tactile discrimination, vibratory, and position sensation below the level of the lesion Interruption of lateral spinothalamic tracts: Contralateral loss of pain and temperature sensation. This usually occurs 2–3 segments below the level of the lesion. Treatment Treatment is directed at the pathology causing the paralysis. If the syndrome is caused by a spinal fracture, this should be identified and treated appropriately. Although steroids may be used to decrease cord swelling and inflammation, the usual therapy for spinal cord injury is expectant."}, {"id": "pubmed23n0524_11125", "title": "[Intermediate medullary infarction: a case report].", "score": 0.01113372810264002, "content": "A 68-year-old man presented with right eye pain and vertigo. Thereafter, he gradually leaned rightward, then laid down. He felt nausea and vomited. His right upper eyelid drooped and he felt dysethesia of the right hand. On neurological examination, ptosis of his right eye with slightly miotic right pupil, paresis of the right soft palate and hoarseness were noted. Arm deviation test demonstrated rightward deviation. He presented sensory ataxia of the right upper and lower extremities: finger nose test showed mild dysmetria of the right upper extremity, heel knee test demonstrated dysmetria of right lower extremity and these findings worsened when he closed his eyes. He showed mild bending of his bilateral ring and little fingers when he did rapid alternative movement. He leaned rightward when he sat and closed his eyes. Position sense of his right upper and lower extremities was decreased and sometimes he could not answer correctly when asked on which direction his finger pointed. Pinprick sensation was mildly decreased on the left side not including the face. Touch and vibration sense were normal. SEP findings on upper and lower extremity stimulation were normal. MRI of the brain showed T2 high intensity and partially T1 low intensity lesion at the right medulla (Figure). MR angiography showed no apparent lesion of major arteries such as dissection of the vertebral arteries. He complained and presented with hiccup initially. On MRI, the lesion was thought to involve the spinothalamic tract, medial lemniscus and inferior olivary nucleus. Ambiguus nucleus was in the lesion and solitary nucleus near the lesion. There is no report that seems to describe clinical features of a lesion like that in this case. Intermediate medullary infarction may present dissociated sensory disturbance like Brown-Sequard syndrome and position sensory disturbance without disturbance of vibration sense."}, {"id": "pubmed23n0085_11291", "title": "[Subacute myeloneuropathy after abuse of nitrous oxide: an electron microscopic study on the peripheral nerve].", "score": 0.010736721873215305, "content": "We have experienced a case of myeloneuropathy following habitual abuse of nitrous oxide. We report clinical and pathological findings of this case with review of literatures. A 36-year-old dentist was first admitted to our hospital on August 17, 1983 because of numbness of both lower legs and unsteady gait. He had recreationally inhaled nitrous oxide 30 to 60 minutes everyday since a year ago. Neurological examination showed ataxic broad-based gait, moderate loss of pain and touch sensation in the lower limbs up to patella. Position and vibratory senses were more severely impaired. Deep tendon reflexes increased in the upper extremities and at the knees, but diminished at ankle jerks. Muscle strength was normal. Prominent Lhermitte's sign was present. Except for reduced serum vitamin B12 level, laboratory results were normal. Needle electromyography showed high amplitude and long duration NMU in the right quadriceps and anterior tibial muscles. Motor nerve conduction velocity was 34 m/sec at the right posterior tibial nerve and could not be detected at the left. Sural nerve biopsy was performed. The density of the myelinated fibre measured on transverse section was in normal range but degenerated fibres were occasionally recognized. Single teased nerve fibre showed various degree of myelin ovoid along the fibre. Myelin loss was shown to occur in some parts of the fibre. Electron microscopy showed myelin splitting and formation of intramyelinic vacuoles containing myelin debris. Axon was almost normal at least in the early stage of degeneration. Later, axon disappeared with destruction of myelin sheath. These nerve changes largely demonstrated demyelination but it was occasionally accompanied with axonal degeneration.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "Neurology_Adams_10232", "title": "Neurology_Adams", "score": 0.010166741178613416, "content": "be more rapid or more leisurely. The initial disturbance may be of motor or sensory function and the distribution may be asymmetrical. High cervical or foramen magnum lesions produce special clinical syndromes, as described in Chap. 3 and below. With thoracic lesions, one leg usually becomes weak and stiff before the other one. Subjective sensory symptoms of the dorsal column type (tingling paresthesias) assume similar distributions. Pain and thermal senses are more likely to be affected than tactile, vibration, and position senses. Nevertheless, the posterior columns are frequently involved as the process progresses. Initially, the sensory disturbance is contralateral to the maximum motor weakness, but a sharply defined Brown-Séquard hemicord syndrome is rarely observed. The bladder and bowel usually become paralyzed coincident with paralysis of the legs. If the compression is relieved, there is recovery from these sensory and motor symptoms, often in the reverse order of their"}, {"id": "pubmed23n0595_2052", "title": "Syringo-subarachnoidal shunting to correct unilateral leg deformity in a child with terminal syringomyelia: case report.", "score": 0.009900990099009901, "content": "Among cases with terminal syringomyelia, 25% are associated with tethered cord syndrome. As it can be difficult to determine whether the neurological deficits are attributable to a syrinx or to a coexisting occult spinal dysraphism, it is not easy to determine the correct surgical strategy. We report a 19-month-old girl with an underdeveloped right leg and pes varus detected when she was 1 month old; lumbosacral magnetic resonance imaging (MRI) revealed syringomyelia. She developed recurrent urinary tract infections and consulted our department with a diagnosis of congenital neurogenic bladder. She presented with clubbed equine position, wore a short brace on her underdeveloped right leg, and exhibited limping gait due to shortening of the right leg. There was no anal reflex. The skin on her lower back was normal. MRI study showed that the lower end of the conus medullaris existed at the L3/4 level; central-type syringomyelia was recognized in the conus medullaris at the T12/L1-L2/L3 level. Computed tomography myelography detected no trabeculae causing tethering effects or influx of contrast medium into the syrinx. There was no comorbid disease like hydrocephalus or Chiari malformation. We performed syringo-subarachnoidal shunt by L1-L2 hemilaminectomy. Postoperative MRI confirmed shrinkage of the syrinx. One year later, although her perianal sensory loss and bowel and bladder dysfunction remained, her right leg had caught up with the left and at 1 year and 9 months after the procedure there is no discrepancy in her legs and she is able to run without limping."}, {"id": "pubmed23n0255_21155", "title": "Tibialis anterior R-1 response: physiological behaviour, normative data and clinical utility in L4-L5 radicular compression.", "score": 0.009900990099009901, "content": "A modified H-reflex, known as R-1 response, was recorded in healthy subjects by averaging 200 responses from the moderately contracting tibialis anterior muscle (TA) after repetitive submaximal stimulation of common peroneal nerve. Physiological changes to different modes of stimulation and recording were studied and normative data obtained at the most reliable parameters. Subsequently, the test is applied to 36 patients with clinical and myelographic evidences of L5 or L4-L5 radicular compression (with or without S1 radicular compression). Increasing the force of muscle contraction resulted in increase in amplitude up to a certain limit, with no change in latency. Increasing the current from subliminal to submaximal stimulation caused initial increase and subsequent fall in the amplitude without change in the latency. The change in the stimulus frequency from 0.5 to 3 Hz did not alter latency or amplitude but the higher frequencies sometimes did. On changing the position of the foot from plantarflexed to dorsiflexed, an increase in amplitude and reduction in duration of the response was noted. Fifty subjects were studied using stimulus intensity just enough to produce 200-400 microV M-response, stimulus duration of 1.0 msec, stimulus rate of 3 Hz and the muscle force sufficient to keep the foot in complete dorsiflexion. The tibialis anterior R-1 response (TAR-1) could be recorded reliably from both legs in all the subjects. The take-off point was sharp and in no subject was there any difficulty in measuring the latency to onset point. On repeating the test, the onset point of the response was superimposable. Latency showed linear correlation with height and age. Among the patients, 21 out of 25 cases of unilateral (84.0%) and 10 out of 11 cases of bilateral (90.9%) radicular compression had abnormal findings. Abnormalities included significant a right-left latency difference within the normal range of absolute latencies, unilateral or bilateral prolongation of latency to onset point and attenuation or absence of the response. It was concluded that the TAR-1 is an important and reliable test for the study of functional integrity of L4-L5 spinal segments in lumbo-sacral sensorimotor root lesions."}, {"id": "Neurology_Adams_1608", "title": "Neurology_Adams", "score": 0.00980392156862745, "content": "Fig. 10-3) and between the fourth and fifth lumbar vertebrae (compressing the L5 root). Lesions of the fifth lumbar root (L5) produce pain in the region of the hip and posterolateral thigh (i.e., sciatica) and, in more than half such cases, lateral calf (to the lateral malleolus), and less often, the dorsal surface of the foot and the first or second and third toes. Pain is elicited by the straight-leg raising test or one of its variants, and protective nocifensive reflexes come into play, limiting further elevation of the leg. Paresthesia may be felt in the entire territory or only in its distal parts. The tenderness is in the lateral gluteal region and near the head of the femur. Weakness, if present, involves the extensors of the big toe and foot and the foot invertors (a distinguishing feature of foot drop originating from peroneal nerve damage, which spares inversion because it is a tibial nerve function). The ankle jerk may be diminished (more often it is normal), but the knee"}, {"id": "wiki20220301en012_33055", "title": "Trigeminal nerve", "score": 0.009764010419103769, "content": "Clinical significance Trigeminal neuralgia Cluster headache Migraine Wallenberg syndrome Wallenberg syndrome (lateral medullary syndrome) is a clinical demonstration of the anatomy of the trigeminal nerve, summarizing how it processes sensory information. A stroke usually affects only one side of the body; loss of sensation due to a stroke will be lateralized to the right or the left side of the body. The only exceptions to this rule are certain spinal-cord lesions and the medullary syndromes, of which Wallenberg syndrome is the best-known example. In this syndrome, a stroke causes a loss of pain-temperature sensation from one side of the face and the other side of the body."}, {"id": "pubmed23n0272_4893", "title": "[A 64-year-old man with recurrent blurred vision and an abdominal mass].", "score": 0.009708737864077669, "content": "We report a 64-year-old man with recurrent bouts of blurred vision who died after developing an abdominal mass. He was well until June of 1985 when he was 59-years-old when he had an acute onset of loss of vision in his right eye. He was treated by prednisolone with a complete remission. In August of 1986, he had another bout of blurring of vision in his left eye. Once he lost his left vision completely, from which he showed slow recovery. In January of 1987, he developed blurring of his right eye and loss of pain and touch sensation in his right leg. Since then he repeated loss of vision in his right or left eye five times, and he was admitted to our hospital in May of 1990. On admission, he was alert and oriented. General physical examination was unremarkable. Neurologic examination revealed bilateral optic nerve atrophy. He could not discriminate light or dark by either eye. Other cranial nerves were unremarkable. He could walk in a wide-base only with support; spasticity was noted in his left leg. Muscle strength was preserved. Deep reflexes were exaggerated in both legs with extensor plantar reflex bilaterally. Pain and touch sensation was decreased in the left leg by 30%, and vibration was diminished in both feet. Position sense was preserved. Routine blood counts and chemistries were unremarkable. Cranial MRI scans revealed multiple high-signal intensity lesions in both pontine bases, basal ganglia, thalami, and in the deep cerebral white matters. He was treated with oral prednisolone, plasmapheresis, lymphocytapheresis, and then immuran. His vision showed only slight recovery to discriminate light and dark. In October of 1990, slight weakness appeared in his both legs. In December of that year, he developed nausea, and a fiber colonoscopic study revealed a stenosis in the transverse colon. In March of 1991, he developed anemia and liver dysfunction. In July of that year, jaundice appeared, and his serum bilirubin was increased. In October, his leg weakness became more prominent, and his cranial CT scans at that time revealed a low density change in the right cerebellum in the right superior cerebellar artery territory; in addition, multiple low density spots were scattered to be seen in both cerebral hemispheres including the basal ganglia and thalamic areas with ventricular dilatation and cortical atrophy.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0554_6022", "title": "Comparison of the electrically evoked leg withdrawal reflex in cerebellar patients and healthy controls.", "score": 0.009708737864077669, "content": "The aim of this study was to analyze the contribution of the cerebellum in the performance of the lower limb withdrawal reflexes. This has been accomplished by comparing the electrically evoked responses in cerebellar patients (CBL) with those in sex- and age-matched healthy control subjects (CTRL). The stimulus was applied to the subjects' medial plantar nerve in four blocks of ten trials each with switching the stimulus from one leg to the other after each block. Responses of the main muscle groups (tibial muscle: TA; gastrocnemius muscle: GA; rectus femoris muscle: RF; biceps femoris muscle: BI) of both legs were recorded during each stimulus. The group of CBL patients consisted of both focally lesioned patients (CBLf) and patients presenting a diffuse degenerative pathology (CBLd). (1) For the withdrawal reflex in CTRL subjects, responses were observed in distal and proximal muscles of the ipsilateral side and corresponding concomitant responses on the side contralateral to the stimulation, whereas in CBL patients responses were restricted primarily to distal muscles, particularly the TA of the ipsilateral, i.e. the stimulated, side. (2) The sequence of activation of the different distal and proximal muscles ipsilateral to the stimulation, derived from latencies and times-to-peak, was for the CTRL group: TA-GA-BI-RF. This sequence was found also in the CBLf patients on their unaffected side. However, on their affected side CBLf patients showed very early GA activation, almost simultaneously with TA and RF activations and before BI activation. RF activation before BI activation was also found in CBLd. In the latter group, GA was activated after RF but before BI with all responses typically delayed. (3) The general pattern of the electrically evoked lower limb reflex consisted of an early, excitatory F1 component and a later, excitatory F2 component of larger amplitude observed in the CTRL subjects and the CBLd patients. In contrast to this pattern CBLf patients exhibited large F1 components followed by small F2 components. (4) The characteristic differences in the withdrawal reflex responses of cerebellar patients depended on the type of the lesion, providing evidence for an important involvement of the cerebellum in the control of the performance of withdrawal reflexes."}, {"id": "pubmed23n0311_6890", "title": "[A man with systemic lupus erythematosus presenting with spastic paraplegia].", "score": 0.009615384615384616, "content": "We report a 38-year-old man systemic lupus erythematosus who presented with an acute onset of paraplegia and urinary retention. The man had a 12-year history of nodular cutaneous mucinosis and arthralgia. In 1994, he was admitted to our hospital with a sudden onset of weakness and numbness of the right leg followed by an emergence of similar symptoms in the left leg. His elder sister had died at 16 years of age after suffering from systemic lupus erythematosus for 6 years. On examination, the patient had skin rash on his chest, back, head, forehead, and extremities. The neurological examination revealed that his tongue deviated to the right on protrusion. The muscle power was reduced to 2-3/5 in the right leg and to 4/5 in the left leg. The sensory disturbance was noted in the lower extremities with predominant involvement of the right leg. Reflexes were increased in the right biceps, triceps, both patellas, and Achilles tendons. Babinski sign was noted bilaterally. Urinary retention and constipation were also noted. The results of the blood cell count and hepatic and renal function tests were normal. Serum levels of C-reactive protein and complements (C3, C4, CH50) were also normal. Serological examinations showed increased anti-DNA antibody (14 U/ml, [normal, &lt; 6]). Antinuclear antibody was positive at a titer of 1:1380. CSF study showed an increased protein concentration of 83 mg/dl and an IgG level of 14 mg/dl with a normal number of cells. MR images revealed a T1-low, T2-high signal lesion at the upper part of the left ventral medulla. MR images of the brain and spinal cord were normal. The patient was diagnosed as having SLE. High-dose intravenous methylprednisolone (1 g/day) pulse treatment that was started 25 days after the onset of neurological symptoms, produced partial relief. Our case presented with paraplegia with a focal lesion in the left upper ventral part of the medulla on MR images. The incidence of male SLE is low, and paraplegia is a rare complication of SLE. Thus, the medullary lesion in SLE observed in our case appears to be rare. SLE should be considered as a cause of acute onset paraplegia or myelopathy."}, {"id": "pubmed23n1012_9208", "title": "Pure Conus Medullaris Syndrome without Lower Extremity Involvement Caused by Intradural Disc Herniation at L1/2: A Case Report.", "score": 0.009615384615384616, "content": "Conus medullaris syndrome (CMS) is a rare pathology. The conus medullaris is located at the end of the spinal cord and continues to the cauda equina. Conus medullaris lesions can cause variable symptoms and neurological deficits, usually involving the lower extremities; CMS that does not affect the lower limbs is extremely rare. No reports have described isolated CMS caused by intradural disc herniation (IDH). This report describes a case of CMS without lower extremity involvement associated with IDH at L1/2. A 52-year-old man with a 10-year history of lower back pain complained of dysuria and lumbago with no leg symptoms at his first visit to the urology department. Neurological examination revealed mild perineal hypoalgesia; however, motor function and lower extremity sensation were normal with except for left ankle dorsiflexion weakness (manual muscle test, 4/5). Magnetic resonance imaging revealed conus medullaris compression by a mass, continuous with the L1/2 disc, and severe spinal canal stenosis at vertebral levels L3/4 and L4/5. Postmyelographic computed tomography indicated direct conus medullaris compression by an intradural and extramedullary mass continuous with the L1/2 disc. Without recovery of his dysuria, the patient underwent surgery, including partial laminectomy of the L1/2, incision of the dura mater, and removal of the herniated disc. Immediately after surgery, his dysuria completely resolved. More than one year postoperatively, the patient remained active with no change in his neurological condition. Although CMS without lower limb symptoms is extremely rare, we experienced an isolated case of CMS associated with IDH causing direct conus medullaris compression. Without lower extremity involvement, the CMS diagnosis was relatively easy. Surgical treatment for CMS without lower extremity involvement caused by IDH was effective."}, {"id": "article-40738_13", "title": "Lumbosacral Plexopathy -- History and Physical", "score": 0.009586442914278646, "content": "Physical examination may be normal in mild cases. Bruises may be seen in cases of trauma. A straight leg raise test is positive in more than half of the patients. Asymmetric lower limb muscle weakness may be seen with asymmetrically absent or reduced deep tendon reflexes. Knee jerk reflex is affected in lumbar plexopathy and ankle jerk is affected in sacral plexopathy. Muscle weakness in hip flexion, knee extension, or adduction suggests a possible injury to the lumbar plexus. Sensory loss may be present in a dermatomal pattern in cases of proximal LS plexopathy involving the roots, or in the nerve distribution. Sensory changes to the medial thigh, anterior thigh, and medial leg can suggest lumbar plexus involvement; posterior thigh, dorsum of the foot, and perineum are likely related to sacral plexus involvement. Spinal point tenderness may be present, especially in cases of sacral fracture or infection. A rectal exam should be performed to assess rectal tone. Saddle anesthesia and bowel or bladder incontinence are rare and may be present, making it difficult to differentiate from cauda equina and conus medullaris syndromes. The inguinal region should also be palpated for suspected hematomas."}, {"id": "pubmed23n0255_13300", "title": "[A 74-year-old man with urinary incontinence, right leg weakness and multiple cranial nerve palsies].", "score": 0.009523809523809525, "content": "We report a 74-year-old man with a lung cancer, who developed right leg weakness, neurogenic bladder, and multiple cranial nerve palsies. The patient was well until December of 1992, when he was 74-year-old, when he noted transient double vision; in February of 1993, he noted numb sensation and weakness in his right leg. Later in the same month, he developed overflow incontinence of urine and weakness in his right face. He also noted deafness in his left ear (he had a marked loss of hearing in his right ear since childhood because of otitis media). His weakness in his right leg had progressed, and he was admitted to our service on March 19, 1993. On admission, he was afebrile and BP was 130/50 mmHg. General physical examination was unremarkable. On neurologic examination, he was alert and oriented to all spheres; no dementia was noted nor were detected aphasia, apraxia, and agnosia. His optic fundi were unremarkable; ocular movement appeared normal, however, he complained of diplopia in far vision. Sensation of the face was intact. He had right facial palsy of peripheral type; he was unable to close his right eye, and Bell's phenomenon was observed on attempted eye closure. On the left side, he had facial spasm. He had marked bilateral deafness. He had no dysarthria or dysphagia. The remaining of the cranial nerves were intact. Motor wise, he was unable to stand or walk alone; weakness did not appear to account for his difficulty in gait; manual muscle testing revealed 4/5 weakness in his tibialis anterior muscle, 1/5 in the peroneus longus, 0/5 in his extensor hallucis longus and extensor digitorum longus, all on the right side. Brachioradial and quadriceps femoris reflexes were increased to 3/4; plantar response was equivocal on the right side, and flexor on the left. Sensory examination revealed loss of touch and pain sensation in the L5 and S1 distributions in his right leg: vibration and position sensations were also diminished in his right foot. He had overflow urinary incontinence with loss of bladder sensation. Marked nuchal stiffness was noted, however, no Kernig's sign or eye ball tenderness was present. Pertinent laboratory findings were as allows; WBC 8,100/microliters, Ht 42.5%, platelet 326,000/microliters, TP 6.8 g/dl, BUN 16 mg/dl, creatinine 0.54 mg/dl, glucose 95 mg/dl, Na 136 mEq/l, K 4.4 mEq/l, Cl 100 mEq/l; liver profile was normal; CEA 436.6 ng/ml, CA19-93 U/ml; urinalysis was normal.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0329_1897", "title": "[Disturbance of deep sensation in medial medullary syndrome. Topographical localization of medial lemniscus in the medulla oblongata].", "score": 0.009523809523809525, "content": "Medial medullary infarction is characterized by ipsilateral hypoglossal nerve palsy with contralateral hemiparesis and disturbance of deep and discriminative sensory perception. We examined the extent and distribution of disturbances in deep sensation and compared the findings with the lesion location in the medial lemniscus detected by MRI in 3 patients with medial medullary infarction. We classified the lesion location into 2 groups; type I and type II. Type I was ventral to the middle medial lesion of the medial lemniscus, and type II was ventral to the dorsal medial lesion. In our series, type I (Case 1) impairment of the three kinds of deep sensations was more severe in the lower extremities than in the up-per extremities. In type II (Cases 2, 3) the severity or impairment in the upper extremities was moderate or severe and nearly equal to that in the lower extremities. There was no difference in the severity of impairment for the four kinds of discriminative sensations. In the literature, type I (8 patients) impairment of position sense in deep sensation was found in 1 of 7 patients in the upper extremities and 5 of 7 patients in the lower extremities. Impairment of vibration sense was found in 1 of 7 patients in the upper extremities and in all patients in the lower extremities. In type II (14 patients) severe impairment of position and vibration sense in deep sensation was found in 3 patients in the upper extremities equal to that in the lower extremities. There was no tendency in the severity of impairment of four kinds of discriminative sensations. Including our 3 cases and 22 in the literature, impairment of deep sensation was more severe in the lower extremities than in the upper extremities in type I (9 patients) and the extent was none (7), mild or moderate (2) in the upper extremities, mild (2), moderate (1), severe (2), obscure (4) in the lower extremities, while in type II (16 patients) the severity in the upper extremities was nearly equal to that in the lower extremities and the extent was none (1), mild or moderate (1), severe (5), obscure (9) in the upper extremities, none (2), mild or moderate (1), severe (6), obscure (7) in the lower extremities. It is concluded that hemiparesis appeared with lesions located in the pyramidal tract of the medulla, hemiparesis and disturbance of deep sensation in the upper and lower extremities, predominantly in the lower extremities with the lesion of the pyramidal tract to the middle of medial lemniscus in the medulla, hemiparesis and disturbance of the upper and lower extremities deep sensation with lesions of the pyramidal tract to the whole of the medial lemniscus in the medulla. Evaluating deep sensation of the upper and lower extremities is useful for speculation of the lesion location in the medial lemniscus in medial medullary infarction."}, {"id": "pubmed23n0383_16974", "title": "Sensory symptoms in ipsilateral limbs/body due to lateral medullary infarction.", "score": 0.009433962264150943, "content": "To characterize the incidence, topography, and radiologic and pathophysiologic findings of ipsilateral sensory symptoms in the limbs/body in patients with lateral medullary infarction. Between 1994 and 2001, the author identified 12 patients with lateral medullary infarction (6.7% of all lateral medullary infarction patients) who presented with ipsilateral sensory symptoms in the limbs/body in addition to typical lateral medullary syndrome. Brain MRI, nerve conduction velocity, and electromyographic studies were performed. Twenty-four patients without ipsilateral sensory symptoms were included as a control group. Clinical and radiologic findings were compared between the two groups. The ipsilateral sensory symptoms were generally described as numbness or tightness, predominantly affecting the upper extremities, especially distal fingers. Vibration and proprioceptive sensation were occasionally impaired. None showed evidence of peripheral neuropathy or radiculopathy. The patients with ipsilateral sensory symptoms significantly more often had vertigo, nausea/vomiting, severe gait ataxia, hiccup, ipsilateral hemiparesis, and caudally located lesions than those without. The caudal lesions producing ipsilateral sensory symptoms tended to extend dorsomedially. Lateral medullary infarction associated with ipsilateral sensory symptoms in the limbs/body is an uncommon but distinct variant caused by caudal lesions extending dorsomedially that probably involve the ipsilateral dorsal column or decussating lemniscal fibers."}, {"id": "pubmed23n0653_9523", "title": "[Two cases of familial amyotrophic lateral sclerosis with a SOD1L126S mutation showing high age at onset and slow progression].", "score": 0.009345794392523364, "content": "An 80-year-old man (patient 1) was admitted to our hospital with numbness of the right leg and weakness of the lower extremities, predominantly in the right leg, for 1 year previously. Neurological examination revealed moderate weakness without atrophy, and fasciculation in the bilateral lower extremities. No hyperreflexia was noted, and the plantar response was flexor. Neither bulbar palsy nor sensory disturbance was observed. Electromyography (EMG) showed a chronic neurogenic pattern, including giant motor unit potentials and reduced interference in all four limb muscles. MRI images of the cervical and lumbar spines showed severe age-related spondylosis. The clinical and laboratory findings led to a diagnosis of spinal progressive muscular atrophy. Motor paralysis progressed slowly for the following four years, culminating in respiratory failure. A 79-year-man, the younger brother of patient 1 (patient 2), suffered from gait disturbance for 3 years before the admission to our hospital. During the following 3 years, bilateral leg weakness developed, causing difficulty walking. Neurological examination revealed a diffuse mild weakness with atrophy and fasciculation in the bilateral lower extremities; the right deltoid muscle was also mildly weak. Mild hyperreflexia was also noted on the left side, and the plantar response was extensor on the left. EMG showed acute and chronic neurogenic patterns in the four limb muscles. Because the missense mutation c.377 T &gt; C; p.L126S was found on exon 5 of the superoxide dismutase (SOD) 1 gene in this patient, a diagnosis of familial ALS was made. Eight patients reported as familial ALS with the SOD1L126S mutation, including the present cases, all developed an onset of weakness in the lower extremities, but showed few upper motor neuron signs. It is important to consider the possibility of this type of familial ALS in a case of spinal progressive muscular atrophy with late-onset and mild progression, if family history is positive."}, {"id": "article-24830_7", "title": "Medial Medullary Syndrome -- History and Physical -- Symptoms and Signs", "score": 0.009345794392523364, "content": "Contralateral paralysis of the upper and lower limb of the body due to lateral corticospinal tract involvement is a common finding. [10] [11] A contralateral decrease in proprioception, vibration, and/or fine touch sensation may occur if medial lemniscus involvement is present in medial medullary syndrome. Some patients report paresthesias or less commonly dysesthesias in the contralateral trunk and lower limb. There are no objective signs of position, touch, or vibration sense loss in many patients with sensory symptoms. In certain patients, a slight loss of position and vibration sense with proprioceptive dysfunction in the contralateral foot is observed. [12]"}, {"id": "pubmed23n0697_20231", "title": "Zoster myelitis in sickle cell anemia.", "score": 0.009259259259259259, "content": "A 17-year-old female patient, known case of sickle cell anemia was admitted to our hospital with 10 days history of fever, vomiting, and epigastric pain. On examination, her temperature was 38°C. There was a vesicular type of rash below the nipple and over the left chest involving the back. She was diagnosed as herpes zoster and was started on acyclovir with good hydration and analgesia. Three days later, she developed weakness and decreased sensation of the right leg. On the fifth day, examination revealed power of 1/5 on the right leg, and 4/5 on the left leg, there was proximal and distal increased tone and brisk reflexes and up going toe on the right side with sensory level at T4-T6. An MRI of the thoracolumbar spine showed high signal intensity at T4-T6. The CSF analysis revealed positive polymerase chain reaction for varicella zoster. She was treated with intravenous (IV) dexamethasone 4 mg, 4 times per day. After 3 days she developed left leg weakness, urine incontinence, and power in the left leg was 3/5. Reflex plantar was up going bilaterally with sensory level at the nipple, T4-T6. She was then stared on IV methylprednisolone one gm for 3 days followed by a tapering dose of prednisolone 50 mg for 2 weeks, after a week of starting medication she was able to walk. This case of transverse myelitis is related to varicella zoster infection, with sickle cell anemia, and was successfully treated with high dose IV methylprednisolone, IV acyclovir, and physiotherapy."}, {"id": "pubmed23n1072_1064", "title": "Cutaneomeningospinal Angiomatosis (Cobb Syndrome) in a Young Patient.", "score": 0.009174311926605505, "content": "Cobb Syndrome (Spinal Arteriovenous Metameric Syndrome 1-31 (SAMS 1-31)) is a rare, non-hereditary disorder. Approximately 100 cases of CS have been described to date. The actual incidence may be much higher since only symptomatic patients were documented. In particular, post mortem studies suggest a possibly higher incidence of this syndrome. The main clinical manifestations of this syndrome include skin stains of vascular nature on the torso, in combination with spinal vascular malformations localized in one and the same metameric or spinal segment. A rare diagnosis of this syndrome in patients over 18 is probably related to the fact that the disease may be asymptomatic throughout a long period of time, while patients may tend to disregard the skin lesions. As a result, most publications on this pathology are based on separate case reports. Significant variability of clinical manifestations as well as prolonged progress of the disease often cause errors in diagnosis. What follows is a case report of a young patient with Cobb Syndrome, who was admitted to a regional vascular centre with a misdiagnosis of stroke. 20 patients of young age (from 20 to 35 years old), with a diagnosis of stroke, who were admitted to a University Clinic (of the Russian National Research Medical University Named After Pirogov N.I., Moscow). Among this group of patients, a patient with Cobb syndrome was identified. Patient P., of 22 years, presented with acute, intensive cervical spinal pain, predominantly on the right, numbness and weakness in the arms and legs. About 3 weeks before admission to the hospital, the patient had ARVI with a fever of up to 37.5°C: two weeks before the onset of symptoms, he had undergone extirpation of 2 teeth, for which reason he spent over 2 hours in a forced position with his head thrown back (prolonged overextension in the cervical spine). Multiple skin angiomas on the chest spreading to the shoulder and scapula region. Tetraparesis up to 4 points: tetraparesis in hands with low muscle tone, low reflexes, tetraparesis in legs with high muscle tone, high reflexes, foot clonus when causing Achilles reflexes, tremor in the extremities and no plantar reflex pathology were detected, sensitivity disorders in the hands \"the high gloves\" and no pelvic disorders were detected. Given the presence and exacerbation of neurological symptoms and cutaneous angiomas, MRI with a contrast agent of the cervical spine was recommended. MR-image of an advanced arteriovenous malformation (AVM) of the cervical spinal cord with signs of gliosis and spinal cord oedema at the C2-C7 level. Endovascular embolization of the AVM in the cervical spinal cord was performed. The treatment led to the complete reversal of neurological symptoms. In the presence of skin lesions, the diagnosis of CS does not present particular difficulties, so in children and young patients with skin angiomatosis, it is advisable to conduct a comprehensive examination using selective spinal angiography or MR angiography to exclude arteriovenous malformations in the spinal cord."}, {"id": "pubmed23n0383_2983", "title": "The central loop of H-reflex in the S1 spinal nerve: normal values and constitutional influencing factors.", "score": 0.009174311926605505, "content": "To investigate the central loop of H reflex in the S1 nerve and the constitutional influencing factors in normal healthy individuals. The study was performed on 39 apparently healthy volunteers. To obtain central H-reflex, the cathode electrode was inserted at a point 1 cm medial to the posterior superior iliac spine, perpendicular to the frontal plane. After the needle touched the sacrum, it was slightly retracted to avoid direct contract to the sacrum. The anode electrode was placed over the anterior iliac spine. The active pick-up electrode was placed at the middle of the line connecting the popliteal crease to the medial malleolus. The reference electrode was placed 2 cm distal to it. The ground eletrode was placed near the active pick-up electrode over the calf muscles. Mean +/- SD for minimum necessary stimulation to obtain central H-reflex was 23 +/- 13 mA. This was significantly higher than the minimum necessary stimulation to obtain peripheral H reflex (p &lt; 0.0001 using paired t test). Mean central loop of H reflex was 6.9 msec with SD of 0.4 msec. There were no significant differences between the two sides. Mean amplitude of M-wave was 2.2 mV with SD of 1.5 in central loop study. Mean amplitude of H-wave was 2.4 mV with SD of 1.5 in central loop study. The Mean time interval between the onset of M-wave and the end of H-wave (M-H duration) was 18.5 msec with SD of 2.3. After stepwise reduction of variables, considering the correlation between leg length and other variables, the leg length was the only variable strongly correlating with central loop of H-reflex. Lack of major influencing factors after correction for leg length, elimination of a major fraction of the afferent segment of the loop, and its ability to differentiate peripheral from central lesions make central H-reflex studies an invaluable diagnostic tool."}, {"id": "Neurology_Adams_10231", "title": "Neurology_Adams", "score": 0.00913868957982697, "content": "Sensorimotor spinal tract syndrome The clinical picture is related predominantly to compression and less often to invasion and destruction of spinal cord tracts. The signs of compression consist of a combination of (1) an asymmetrical spastic weakness of the legs with thoracolumbar lesions and of the arms and legs with cervical lesions, (2) a sensory level on the trunk below which perception of pain and temperature is reduced or lost, (3) posterior column signs, and (4) a spastic bladder under weak voluntary control. The onset of the compressive symptoms is usually gradual and the course progressive over a period of weeks and months, frequently with back pain. With extradural tumors, paralysis usually develops over a period of days to several weeks, but the tempo of progression may be more rapid or more leisurely. The initial disturbance may be of motor or sensory function and the distribution may be asymmetrical. High cervical or foramen magnum lesions produce special clinical"}, {"id": "InternalMed_Harrison_30040", "title": "InternalMed_Harrison", "score": 0.009109609168380842, "content": "Superior cerebellar peduncle Midpontine syndrome: Medial longitudinal fasciculus 5th n. sensory nucleus 5th n. motor nucleus Spinothalamic Medial lemniscus FIGURE 446-12 Axial section at the level of the midpons, depicted schematically on the left, with a corresponding magnetic resonance image on the right. Approximate regions involved in medial and lateral midpontine stroke syndromes are shown. Signs and symptoms: Structures involved 1. Medial midpontine syndrome (paramedian branch of midbasilar artery) On side of lesion Ataxia of limbs and gait (more prominent in bilateral involvement): Pontine nuclei On side opposite lesion Paralysis of face, arm, and leg: Corticobulbar and corticospinal tract Variable impaired touch and proprioception when lesion extends posteriorly: Medial lemniscus 2."}, {"id": "pubmed23n0074_7829", "title": "[Short-latency somatosensory evoked potentials and magnetic resonance imaging in the medial medullary syndrome].", "score": 0.00909090909090909, "content": "A 57-year-old woman was admitted to Kakeyu Hospital complaining of paresis of the left upper and lower extremities which suddenly developed three years ago. Neurological examination revealed spastic paresis of the left upper and lower limbs without facial and lingual paresis. The reflexes were abnormally brisk on both sides but they were more remarkable on the left side, which also showed Babinski's sign. Sensitivity to light touch and vibration was moderately decreased and sensitivity to pinprick and joint position was minimally decreased in the left upper and lower extremities. No cerebellar sign was observed. Needle EMG disclosed large motor units with an amplitude of 4-5 mV and a duration of 8 msec in the light half of the tongue during weak contraction. MRI using a 0.5-T superconducting magnetic resonance unit detected a small, wedge-shaped infarction in the anterior medial portion of the medulla just below the pontomedullary junction. Somatosensory evoked potentials (SEPs) after median nerve stimulation with a non-cephalic reference were recorded. After stimulation of the left side, the scalp-recorded P13 was recognized at the normal latency, but the later components, N16 and N18, were apparently absent. On the other hand, SEPs in another case with thalamic hemorrhage revealed normal N16 potential with absence of N18 on the affected side. From these SEP findings and the reports on SEPs in lesions of the brain stem or thalamus, it was suggested that P13 is abnormal in lower medullary lesions and is preserved in upper medullary lesions, and that N16 is abnormal in brain stem lesions and is preserved or changed a little in thalamic lesions."}, {"id": "pubmed23n0370_10112", "title": "A case of reversible paraparesis following celiac plexus block.", "score": 0.009009009009009009, "content": "Permanent and acute reversible paraplegia following celiac plexus block (CPB) have been reported. We report a case of prolonged reversible paraparesis after alcohol celiac plexus block. A 72-year-old man with primary multicentric pancreatic tumor and multiple hepatic metastases underwent alcohol celiac plexus neurolysis for severe abdominal pain radiating to the back. The patient had complete pain relief after the block but developed paresthesia of the left leg, which then spread to the right leg. Subsequently, loss of flexion and extension of the muscles supplying the left hip, knee, and foot developed. Deep tendon reflexes were brisk on the left compared to the right, and both plantar reflexes gave flexor responses. Magnetic resonance imaging and myelography were normal. Motor-evoked potential recordings showed a spinal cord lesion with involvement of the pyramidal and spinothalamic tracts. Somatosensory-evoked potentials indicated a relative sparing of dorsal column pathways. Physiotherapy was started, the sensory changes gradually subsided, and the patient was discharged 30 days after the block with clinically insignificant neurological deficit. Paraparesis following alcohol celiac plexus block may be reversible over an extended period of time."}, {"id": "pubmed23n0732_18197", "title": "Comparison of the classically conditioned withdrawal reflex in cerebellar patients and healthy control subjects during stance: I. electrophysiological characteristics.", "score": 0.009009009009009009, "content": "The aim of this study was to demonstrate the involvement of the human cerebellum in the classically conditioned lower limb withdrawal reflex in standing subjects. Electromyographic activity was recorded from the main muscle groups of both legs of eight patients with cerebellar disease (CBL) and eight control subjects (CTRL). The unconditioned stimulus (US) consisted of electrical stimulation of the tibial nerve at the medial malleolus. The conditioning stimulus (CS) was an auditory signal given via headphones. Experiments started with 70 paired conditioning stimulus-unconditioned stimulus(CSUS) trials followed by 50 US-alone trials. The general reaction consisted of lifting and flexing the stimulated (stepping) leg with accompanying activation of the contralateral (supporting) leg. In CTRL, the ipsilateral (side of stimulation) flexor and contralateral extensor muscles were activated characteristically. In CBL, the magnitudes of ipsilateral flexor and contralateral extensor muscle activation were reduced comparably. In CTRL, the conditioning process increased the incidence of conditioned responses (CR), following a typical learning curve, while CBL showed a clearly lower CR incidence with a marginal increase, albeit, at a shorter latency. Conditioning processes also modified temporal parameters by shortening unconditioned response (UR) onset latencies and UR times to peak and, more importantly in CBL, also the sequence of activation of muscles, which became similar to that of CTRL. The expression of this reflex in standing subjects showed characteristic differences in the groups tested with the underlying associative processes not being restricted exclusively to the CR but also modifying parameters of the innate UR."}, {"id": "wiki20220301en170_30299", "title": "Middle cerebral artery syndrome", "score": 0.008928571428571428, "content": "Signs and Symptoms Hemiparesis or hemiplegia of the lower half of the contralateral face Hemiparesis or hemiplegia of the contralateral upper and lower extremities* Sensory loss of the contralateral face, arm and leg* Ataxia of contralateral extremities* Speech impairments/aphasia: Broca's area, Wernicke's or Global aphasia as a result of a dominant hemisphere lesion (usually the left brain) Perceptual deficits: hemispatial neglect, anosognosia, apraxia, and spatial disorganization as a result of a non-dominant hemisphere lesion (usually the right brain) Visual disorders: déviation conjuguée, a gaze preference towards the side of the lesion; contralateral homonymous hemianopsia Note: *faciobrachial deficits greater than that of the lower limb Diagnosis Diagnosis can be confirmed by CT Scan or MRI for advanced investigations. References External links Stroke Syndromes affecting the nervous system"}, {"id": "pubmed23n0255_6760", "title": "Medial medullary syndrome. Report of 18 new patients and a review of the literature.", "score": 0.008928571428571428, "content": "With advanced imaging techniques, infarctions occurring in the medulla are now more easily identified. To date, however, only approximately 30 cases of medial medullary infarction syndrome (MMS) have been reported, and the clinical and radiological characteristics of MMS remain to be studied. We studied 18 patients (15 men, 3 women; mean age, 62 years) who had compatible clinical and MRI findings of MMS and reviewed the previously reported cases. Seventeen patients had a unilateral lesion usually involving the upper medulla, and 1 had bilateral lesions. Fifteen patients had unilateral sensorimotor stroke, while 2 presented with pure motor stroke. The face was usually but not always spared. The degree of hemiparesis varied, and a tingling sensation with decreased vibration and position sense was the most common sensory manifestation. Two patients had lingual paresis, and none suffered respiratory difficulties. One patient presented with bilateral gait ataxia without sensorimotor dysfunction. Angiography or MR angiography performed in 9 patients showed vertebral artery disease in 6. Three patients had concurrent lateral medullary infarction, and 1 had a previous history of lateral medullary syndrome. The prognosis was generally good, although residual hemiparesis remained in patients with initially severe hemiparesis. Review of 26 previously reported cases showed that they frequently had bilateral lesions, often presenting with quadriplegia, lingual paresis, respiratory symptoms, and a grave prognosis. Our data illustrate that MMS is most often manifested as benign hemisensorimotor stroke frequently associated with tingling sensation and impaired deep sensation. This benign form of MMS should be much more common than MMS with poor prognosis and may have been frequently misdiagnosed as capsular or pontine stroke before the era of MRI."}]}}}}
{"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[0, 122]], "word_ranges": [[0, 18]], "text": "Dopler ultrasound is the most accurate test and allows subsequent non-invasive monitoring of the evolution of the disease."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Dopler ultrasound is the most accurate test and allows subsequent non-invasive monitoring of the evolution of the disease.", "full_answer_no_ref": "Doppler ultrasound is the most accurate test and allows subsequent non-invasive monitoring of the evolution of the disease.", "full_question": "65-year-old man with a history of pancreatic neoplasm undergoing chemotherapy. What diagnostic test is more cost-effective to confirm the diagnostic suspicion?", "id": 265, "lang": "en", "options": {"1": "D-dimer.", "2": "Magnetic resonance imaging.", "3": "Phlebography.", "4": "Venous Doppler ultrasound.", "5": "Helical CT."}, "question_id_specific": 137, "type": "PNEUMOLOGY", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0303_15123", "title": "Diagnostic workup of patients with suspected pancreatic carcinoma: the University of California-Los Angeles approach.", "score": 0.013549239920687376, "content": "A large number of diagnostic procedures (e.g., ultrasound, computed tomography [CT] scan, fine-needle aspiration [FNA], angiography, endoscopic retrograde cholangiopancreatography [ERCP], and laparoscopy), are available to the clinician as he/she pursues the workup of patients who are thought to have a pancreatic (periampullary) malignancy. Not all of these procedures should be used in every patient and some have been overused. Based on a current literature review and their own experience, the authors describe the rationale of the diagnostic workup in patients with suspected pancreatic carcinoma in a single institution (a university medical center). Helical CT scan provides the best overall assessment of patients with periampullary malignancies, and it is often the only test required. If the patient's history and blood test abnormalities suggest pancreatic carcinoma and the helical CT scan shows a mass in the head of the pancreas that appears to be resectable, the patient should be prepared for surgery. If no mass is apparent on the helical CT scan, a diagnostic ERCP is indicated. If microscopic proof of the diagnosis will avoid surgery, then an FNA for cytology should be performed. When unresectability appears likely and cannot be confirmed in less invasive ways, laparoscopy is indicated. In patients with periampullary malignancies, helical CT scan provides the best overall assessment. Guidelines are presented for the selective use of ultrasound, FNA, ERCP, and laparoscopy, which are important for the most cost-effective workup of these patients."}, {"id": "pubmed23n0511_9612", "title": "The value of modern ultrasonographic techniques and computed tomography in detecting and staging of pancreatic carcinoma.", "score": 0.013498112401168175, "content": "to assess the clinical value of ultrasonographic methods and computed tomography in diagnosing and staging pancreatic carcinoma. prospective clinical trial of 140 patients (64 women and 77 men; mean age 59,6) operated on for pancreatic carcinoma between 2000 and 2004. In each case helical CT, routine-, color- and power Doppler and 3-D USG were performed to detect and stage cancer. Analyses of accuracy, sensitivity, specificity, PPV and NPV of ultrasonographic methods and CT were made. 3-D USG showed the best accuracy of local staging (T): 95.6%. CT was the most accurate in lymph node assessment: 91.3%. The accuracy of CT, 3-D USG and power-Doppler at detecting vascular infiltration was 93.1%. diagnostic accuracy of modern ultrasound techniques is comparable to helical CT in detecting and staging pancreatic carcinoma. USG is recommended due to the relatively low cost, non-invasiveness and availability of the procedure."}, {"id": "wiki20220301en027_57426", "title": "Insulinoma", "score": 0.011597122302158274, "content": "Diagnostic imaging The insulinoma might be localized by noninvasive means, using ultrasound, CT scan, or MRI techniques. An indium-111 pentetreotide scan is more sensitive than ultrasound, CT, or MRI for detection of somatostatin receptor positive tumors, but not a good diagnostic tool for insulinomas. An endoscopic ultrasound has a sensitivity of 40-93% (depending on the location of the tumor) for detecting insulinomas. Sometimes, angiography with percutaneous transhepatic pancreatic vein catheterization to sample the blood for insulin levels is required. Calcium can be injected into selected arteries to stimulate insulin release from various parts of the pancreas, which can be measured by sampling blood from their respective veins. The use of calcium stimulation improves the specificity of this test."}, {"id": "wiki20220301en622_26954", "title": "Page kidney", "score": 0.009900990099009901, "content": "Diagnosis Techniques for the diagnosis of Page kidney are all imaging based, including abdominal x-ray, intravenous pyelography, angiography, renal doppler ultrasound, CT scan, and Magnetic resonance imaging (MRI). X-ray and pyelography may add in diagnosis, but are non-diagnostic when used alone. Additionally, pyelograms use potentially nephrotoxic contrast agents, further limiting their utility. Diagnosis is primarily made using ultrasound and CT. CT and MRI imaging can show the space occupying lesions but doppler ultrasound is needed to display the hemodynamic changes occurring in the kidney. Ultrasound is often the initial diagnostic test of choice but due to its low resolution further imaging may be necessary. Treatment"}, {"id": "wiki20220301en033_21944", "title": "Acute pancreatitis", "score": 0.00980392156862745, "content": "The principal value of CT imaging to the treating clinician is the capacity to identify devitalised areas of the pancreas which have become necrotic due to ischaemia. Pancreatic necrosis can be reliably identified by intravenous contrast-enhanced CT imaging, and is of value if infection occurs and surgical or percutaneous debridement is indicated. Magnetic resonance imaging While computed tomography is considered the gold standard in diagnostic imaging for acute pancreatitis, magnetic resonance imaging (MRI) has become increasingly valuable as a tool for the visualization of the pancreas, particularly of pancreatic fluid collections and necrotized debris. Additional utility of MRI includes its indication for imaging of patients with an allergy to CT's contrast material, and an overall greater sensitivity to hemorrhage, vascular complications, pseudoaneurysms, and venous thrombosis."}, {"id": "wiki20220301en071_6956", "title": "ICD-9-CM Volume 3", "score": 0.009708737864077669, "content": "Diagnostic radiology () Diagnostic radiology () Soft tissue x-ray of thorax () Other x-ray of thorax () X-ray of urinary system () Computerized axial tomography of kidney CAT scan of kidney () Other nephrotomogram () Intravenous pyelogram Diuretic infusion pyelogram () Retrograde pyelogram () Percutaneous pyelogram () Retrograde cystourethrogram () Other cystogram () Ileal conduitogram () Other x-ray of the urinary system KUB x-ray () Other diagnostic radiology and related techniques () Soft tissue x-ray of abdomen () Other x-ray of abdomen () Skeletal x-ray of extremities and pelvis () Other x-ray () Arteriography using contrast material () Angiocardiography using contrast material () Phlebography () Diagnostic ultrasound () Thermography () Other diagnostic imaging () Diagnostic imaging, not elsewhere classified () Magnetic resonance imaging of brain and brain stem () Magnetic resonance imaging of chest and myocardium"}, {"id": "pubmed23n0594_12594", "title": "Initial failure of angiography to demonstrate a bleeding pancreatic cancer: a case for provocative agents.", "score": 0.009708737864077669, "content": "Mesenteric angiography is commonly employed in the modern-day investigation of gastro-intestinal bleeding if the bleeding sites cannot be identified by endoscopic means. Angiography is optimally sensitive in the presence of active bleeding. However, vasospasm may occasionally account for a negative study shortly after bleeding. A 70-year-old lady with inoperable carcinoma of the pancreas presented with gastro-intestinal bleeding. Although upper endoscopy visualised active bleeding from the tumour, which had invaded into the duodenum, haemostasis could not be achieved endoscopically. Therefore, mesenteric angiography was arranged. The initial angiography failed to demonstrate the bleeding site, which only became obvious on a repeat study, when embolisation was performed to achieve haemostasis. Vasospasm probably accounted for the initial negative study, as the second angiography was able to demonstrate contrast extravasation without the use of any anticoagulant or thrombolytic agent. It is not our routine to give pharmacological agents to provoke bleeding after a negative angiography, but for selected patients this manoeuvre may turn out to be more cost-effective."}, {"id": "wiki20220301en547_4894", "title": "Occult fracture", "score": 0.009615384615384616, "content": "Fractures represent up to 80% of the missed diagnoses in the emergency department. Failure to recognize the subtle signs of osseous injury is one of the reasons behind this major diagnostic challenge. While occult fractures present no radiographic findings, radiographically subtle fractures are easily overlooked on initial radiographs. In both cases, a negative radiographic diagnosis with prominent clinical suspicion of osseous injury will prompt advanced imaging examination such as CT scan, magnetic resonance imaging, ultrasound, and nuclear medicine to confirm or exclude the clinically suspected diagnosis. The burden entailed in missing these fractures includes prolonged pain with a loss of function, and disability. Early detection, on the other hand, enables more effective treatment, a shorter hospitalization period if necessary, and decreased medical costs in the long run. It will also prevent inherent complications such as nonunion, malunion, premature osteoarthritis, and"}, {"id": "pubmed23n0129_11136", "title": "The radiologic diagnosis of pancreatic cancer. The effect of new imaging techniques.", "score": 0.009615384615384616, "content": "The objective of this study was to evaluate how the introduction of radiologic studies affected the diagnostic workup for pancreatic cancer, from 1955 through 1979. For 961 patients diagnosed as having pancreatic cancer at three teaching hospitals, we reviewed medical records, autopsy reports, and death certificates for results from all radiologic studies, surgical and pathologic procedures, and for the final diagnosis. The number of radiologic studies per patient increased as new studies were introduced; 1.6 for 1955-1959, 3.5 for 1975-1979 (P less than 0.0001). Depending on the cutoff level chosen, the sensitivity of the overall radiologic diagnosis increased over time, 0.17-0.43 for 1955-1959, to 0.54-0.78 for 1975-1979; CT, US, and ERCP accounted for much of the increase. As newer radiologic studies are introduced, continued use of previously accepted studies should be carefully evaluated."}, {"id": "wiki20220301en438_35958", "title": "Non-invasive procedure", "score": 0.009523809523809525, "content": "Diagnostic images Bioluminescence imaging Dermatoscopy Diffuse optical tomography Gamma camera Computed tomography Infrared imaging of the body Magnetic resonance elastography Magnetic resonance imaging Magnetic resonance spectroscopy Optical coherence tomography Posturography Radiography, fluoroscopy Ultrasonography and echocardiography Diagnostic signals Electrocardiography Electroencephalography Electromyography Photoplethysmograph Electrical impedance tomography Electroneuronography Electroretinography Electronystagmography Magnetoencephalography Evoked potentials Impedance phlebography Nuclear magnetic resonance Therapy Epidermal radioisotope therapy Radiation therapy Brachytherapy Lithotripsy Defibrillation Biofeedback Oxygen therapy Non-invasive ventilation VPAP BIPAP Neurally adjusted ventilatory assist Biphasic cuirass ventilation Therapeutic ultrasound See also Minimally invasive procedures References Medical procedures"}, {"id": "pubmed23n0215_12171", "title": "[Diagnostic algorithms. Analysis of the factors conditioning their definition. An illustrative case (bilio-pancreatic pathology)].", "score": 0.009523809523809525, "content": "A case of biliopancreatic pathology studied with traditional (the old algorithm) and the latest (new algorithm) diagnostic techniques is examined. A comparative analysis reveals the second to be simpler, less expensive and less dangerous to the patient. The hope is expressed that doctors will become better informed of the diagnostic criteria to employ for the purpose of cost reduction."}, {"id": "wiki20220301en346_41340", "title": "OPS-301", "score": 0.009433962264150943, "content": "Diagnostic procedures : Diagnostic procedures : physical examination : study of individual body systems : biopsy without incision : biopsy with incision : diagnostic endoscopy : function tests : exploratory diagnostic measures : Other diagnostic measures Radiology : Radiology : ultrasound : projection radiography : computed tomography (CT) : optical techniques : representation of the vascular system : nuclear medicine diagnostic procedure : scintigraphy : single-photon emission computed tomography (SPECT) : single-photon emission computed tomography with computed tomography (SPECT / CT) : positron emission tomography (PET) with full-ring scanner : positron emission tomography with computed tomography (PET / CT) : probe measurements and incorporation : magnetic resonance imaging (MRI) : other imaging techniques : additional information on imaging techniques"}, {"id": "pubmed23n0339_18770", "title": "[Computerized tomography of pancreatic tumors].", "score": 0.009433962264150943, "content": "Few pancreatic carcinomas (5-22%) are resectable at the time of diagnosis because this lesion is seldom diagnosed in an early stage. A considerable improvement in the rate of survival is described only for resectable tumors: it is extremely necessary to find an imaging technique for early diagnosis and for accurate staging of pancreatic carcinoma to discern operable from inoperable cancer. The sensitivity of CT in predicting that pancreatic carcinoma is unresectable has been described as approaching 100%. However the reverse is not true. More than one third of the tumors revealed with CT and interpreted as resectable cannot be excised. The major reason for errors with CT are failure to detected liver metastases, peritoneal implants, lymph node involvement and encasement of the great vessels by tumor. Significant progress has recently been made to improve the detection of these details with the recent introduction of helical CT with infusion of a bolus of contrast material and thin section collimation. Traditionally, when a single sequence of images was acquired during abdominal CT, the time of the acquisition was dominated by the requirement to scan during maximal hepatic enhancement, which unfortunately may not be optimal for evaluation of the pancreas. With the advent of helical CT, the acquisition of two sets of images after infusion of contrast material is now possible; the first one takes place during the arterial enhancement; it is useful to detect tumor vascular encasement and the maximum difference of tissue attenuation between normal greater pancreatic enhancement and hypodense pancreatic mass, less vascularizated. It appears that the peak parenchymal enhancement achieved with helical CT may improve the sensitivity of CT scanning in detecting pancreatic carcinoma, especially small tumors confined within the organ. The second phase takes place during the venous or portal enhancement and provides useful information about venous encasement and hepatic metastasis. Extraglandular extension with invasion of adjacent major arterial (celiac axis or its branches, superior mesenteric artery) and venous (portal, splenic, superior mesenteric) appear as soft-tissue attenuation thickening obscuring the perivascular fat, with deformity, thrombosis or occlusion of the vessels. In cases of venous occlusion, collateral vein can be identified. Dilatation of the small veins that surround the head of the pancreas might be used as an additional criterion of extrapancreatic extension of neoplasia. With the features of spiral CT (contrast material optimization and continuous scanning), the detection of small lesions in the liver and peritoneal implants has been increased. Helical CT seems not to detect anything else about lymph node involvement than conventional CT, limited by the same morphologic criteria. The only CT means of detection of node involvement by pancreatic carcinoma is the pathologic enlargement of lymph nodes without specificity for neoplastic or not neoplastic ones. In many cases 2D, 3D and MIP imaging are helpful to evaluate vasculature encasement, especially for visualization of vessels which lie in oblique, coronal or sagittal plane. Consequently helical CT has the potential to become an alternative angiographic technique. Many studies have been done to evaluate spiral CT potential impact and to compare the value of this technique with other ones in the initial diagnosis and staging of pancreatic carcinoma. One of these studies compares dual-phase helical CT and endoscopic endo-sonography. The Authors observe that the two techniques do not differ significant statistically in detecting pancreatic carcinoma, except endoscopic sonography is more sensitive than helical CT for tumors smaller than 15-20 mm. They found the accuracy to predict unresectable carcinoma is 100% for dual-phase helical CT and less for endoscopic endosonography (86%). (ABSTRACT TRUNCATED)"}, {"id": "wiki20220301en364_23998", "title": "Immanuel St. Joseph's", "score": 0.009345794392523364, "content": "Services Inpatient services provided include: birthing rooms, adult heart catheterization and diagnostics, hospice, pain management, cancer treatment, and psychiatric emergency services. Outpatient services include: chemotherapy, trauma center, Chiropractic treatment, dentistry care, kidney dialysis, physical rehabilitation, substance abuse treatment, and urgent care. Diagnostic equipment available include: CT scanner, a diagnostic radioisotope facility, Magnetic resonance imaging, Multislice spiral CT, Single photon emission CT, and ultrasound. References Buildings and structures in Blue Earth County, Minnesota Mankato, Minnesota Hospitals in Minnesota Hospitals established in 1898 Hospitals established in 1996 Mayo Clinic"}, {"id": "pubmed23n0019_13314", "title": "Computerized tomography, diagnostic ultrasound, and radionuclide scanning. Comparison of efficacy in diagnosis of pancreatic carcinoma.", "score": 0.009345794392523364, "content": "Forty-six patients, including 33 with proved pancreatic carcinoma, were studied with computerized tomography (CT), ultrasound (US), and radionuclide (RN) scanning. The results of each scanning procedure were compared with the surgical and clinical findings. The detection rate was 82% for CT, and 92% with US. A mass is the most important finding in the diagnosis of pancreatic carcinoma. Measurements of the pancreas with CT and US were similar, with visualization of all parts of the pancreas routinely better with CT scans. Radionuclide scans were abnormal in 96% of the patients with pancreatic carcinoma as well as in 75% of patients without pancreatic disease. A rational approach to examination of a patient with suspected pancreatic carcinoma should begin with US scan with available, because the detection rate with this method is equal to that with CT and its cost per procedure and for equipment is substantially less."}, {"id": "wiki20220301en001_185851", "title": "Ultrasound", "score": 0.009259259259259259, "content": "Medical ultrasound is an ultrasound-based diagnostic medical imaging technique used to visualize muscles, tendons, and many internal organs to capture their size, structure and any pathological lesions with real time tomographic images. Ultrasound has been used by radiologists and sonographers to image the human body for at least 50 years and has become a widely used diagnostic tool. The technology is relatively inexpensive and portable, especially when compared with other techniques, such as magnetic resonance imaging (MRI) and computed tomography (CT). Ultrasound is also used to visualize fetuses during routine and emergency prenatal care. Such diagnostic applications used during pregnancy are referred to as obstetric sonography. As currently applied in the medical field, properly performed ultrasound poses no known risks to the patient. Sonography does not use ionizing radiation, and the power levels used for imaging are too low to cause adverse heating or pressure effects in"}, {"id": "pubmed23n0921_20101", "title": "Unresectable Pancreatic Adenocarcinoma: Eight Years Later.", "score": 0.009259259259259259, "content": "Pancreatic cancer is the fourth leading cause of cancer deaths in the United States, and is considered uniformly fatal when patients present with unresectable, advanced-stage disease at the time of diagnosis. Long-term survival of patients with advanced-stage pancreatic adenocarcinoma remains rare, despite advances in adjuvant chemoradiation protocols. A 73-year-old male presented to our emergency department with abdominal pain and a history of biopsy-proven, stage III pancreatic adenocarcinoma. His initial staging CT scan and trans-duodenal ultrasound had demonstrated a stage IIa (T3, N0, Mx) lesion. On surgical exploration, he was up-staged to stage III (T4, N0, Mx), noting encasement of the superior mesenteric vessels and involvement of the portal vein. He underwent palliative choledochojejunostomy and was treated with 4 months of oxaliplatin and capecitabine, with concurrent radiation therapy (50.4 Gy), followed by 4 months of gemcitabine. After 7 months, the patient withdrew from therapy due to treatment intolerance. He then turned to self-medication with non-traditional herbal therapies. After 3 years of surveillance, he was lost to follow-up until presenting to our facility with abdominal pain 8 years after his initial diagnosis. On diagnostic CT scan during his current presentation for abdominal pain, he was found to have no evidence of pancreatic cancer. Based on our review of the literature, we present the longest known survival of a patient with surgically unresectable pancreatic adenocarcinoma. Further study of this patient's phenotypic or genotypic characteristics may provide insight into better therapeutic agents, or a predictive subset of patients who will benefit from specific chemotherapeutic options."}, {"id": "wiki20220301en056_8543", "title": "Batten disease", "score": 0.009174311926605505, "content": "Computed tomography (CT) or magnetic resonance imaging (MRI) are diagnostic imaging tests that allow physicians to better visualize the appearance of the brain. MRI imaging test uses magnetic fields and radio waves to help create images of the brain. CT scan uses x-rays and computers to create a detailed image of the brain's tissues and structures. Both diagnostic imaging tests can help reveal brain areas that are decaying, or atrophic, in persons with NCL. Measurement of enzyme activity specific to Batten disease may help confirm certain diagnoses caused by different mutations. Elevated levels of palmitoyl-protein thioesterase is involved in CLN1. Acid protease is involved in CLN2. Cathepsin D is involved in CLN10."}, {"id": "pubmed23n0288_11193", "title": "The use of spiral computed tomography in the evaluation of vessel encasement for pancreatic cancer.", "score": 0.009174311926605505, "content": "Spiral CT allows for a noninvasive evaluation of the mesenteric arterial and venous vessels. This test can be performed quicker, with less expense, and with a reduced radiation and contrast load than angiography. Comparison studies of angiography and spiral CT are needed in patients with pancreatic cancer to determine the best method of evaluating possible vessel involvement. Preoperative imaging of patients with pancreatic cancer is crucial in determining resectability and planning management. Computed tomography (CT) remains the diagnostic procedure of choice for the evaluation of the primary tumor and angiography is the gold standard to evaluate vessel encasement. This case evaluates the usefulness of spiral computed tomography in determining vessel encasement. A 53-yr-old female presented with vague abdominal complaints and evaluation revealed a mass in the pancreas. CT suggested portal vein involvement and collateralization was noted in the upper abdomen. Spiral CT revealed normal arterial anatomy and near complete obstruction of the portal vein at the superior mesenteric vein (SMV) splenic vein (SV) confluence. Operative findings confirmed the involvement of the portal vein at the confluence of the SMV and SV. Pancreatico-duodenectomy with portal vein resection and primary anastomosis was performed. The patient's postoperative course was uneventful and she was discharged home on the 13th postoperative day."}, {"id": "wiki20220301en482_25826", "title": "Pancreatic cyst", "score": 0.00909090909090909, "content": "Follow up guidelines Cysts from 1–5 mm on CT or ultrasound are typically too small to characterize and considered benign. No further imaging follow-up is recommended for these lesions. Cysts from 6–9 mm require a single follow-up in 2–3 years, preferably with magnetic resonance cholangiopancreatography (MRCP) to better evaluate the pancreatic duct. If stable at follow-up, no further imaging follow-up is recommended. For cysts from 1–1.9 cm follow-up is suggested with MRCP or multiphasic CT in 1–2 years. If stable at follow-up, the interval of imaging follow-up is increased to 2–3 years. Cysts from 2–2.9 cm have more malignant potential, and a baseline endoscopic ultrasound is suggested, followed by MRCP or multiphasic CT in 6–12 months. If patients are young, surgery may be considered to avoid the need for prolonged surveillance. If these cysts are stable at follow-up, interval imaging follow-up can be done in 1–2 years. References External links Pancreas disorders Cysts"}, {"id": "pubmed23n0072_17177", "title": "[Differential diagnosis between pancreatic ductal adenocarcinoma and chronic pancreatitis with thin slice table incremental CE-CT].", "score": 0.00909090909090909, "content": "Thin slice table incremental CE-CT was performed on 18 patients considered to have pancreatic carcinoma, 13 patients with chronic pancreatitis, and 19 patients with a normal pancreas. Each patient underwent precontrast CT scanning and delayed scanning. Computed tomography was performed with a high-speed scanner, a TCT 900s. First, precontrast CT scanning was initiated at the level of the top of the right diaphragm, and the scan sequence was performed to include the liver and pancreas at contiguous 10 mm intervals. Second, a bolus of 50-60 mL of 300 mgI/mL iopamidol was delivered intravenously by hand injection via a peripheral arm vein, and subsequent injections were administered during table incrementation with 2-second interscan delay, and the scan sequence was obtained at contiguous 5 mm intervals with a scan speed of 1-second. Delayed scanning was performed 1-2 minutes later after 12 images had been obtained by thin slice table incremental CE-CT scanning. The average CT numbers were calculated for each lesion by precontrast CT scanning, thin slice table incremental CE-CT scanning, and delayed scanning. For the patients with pancreatic carcinoma, the average CT numbers were 41.6HU +/- 6.4HU, 69.6HU +/- 10.4HU, and 86.2HU +/- 14.7HU by precontrast CT scanning, thin slice table incremental CE-CT scanning, and delayed scanning, respectively. While for the patients with chronic pancreatitis, the average CT numbers were 50.0HU +/- 5.0HU, 94.3HU +/- 13.3HU, and 82.5HU +/- 11.6HU, respectively, and for those with a normal pancreas, the average CT numbers were 51.3HU +/- 5.7HU, 93.5HU +/- 7.6HU, and 82.0HU +/- 11.6HU, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en091_7612", "title": "Nutcracker syndrome", "score": 0.009009009009009009, "content": "Diagnosis Nutcracker syndrome is diagnosed through imaging such as doppler ultrasound (DUS), computed tomography (CT), magnetic resonance imaging (MRI), and venography. The selection of the imaging modality is a step-wise process. DUS is the initial choice after clinical suspicion based on symptoms. CT and MRI are used to follow up afterwards, and if further confirmation is necessary, venography is used to confirm. Doppler Ultrasound Although its ability to detect renal vein compression is dependent on how a patient is positioned during imaging, DUS is recommended as an initial screening tool as it has a high sensitivity (69–90%) and specificity (89–100%). DUS measures the anteroposterior diameter, and a peak systolic velocity at least four times as fast as an uncompressed vein is indicative of NCS."}, {"id": "pubmed23n0278_6129", "title": "[Diagnostic modalities and treatment of cancer of the pancreas. Evolution in the population in the department of Côte d'Or from 1976 to 1988].", "score": 0.009009009009009009, "content": "There is no study establishing time trends for the diagnostic and therapeutic approaches to pancreatic cancer based on population data. The data of the Registry of Digestive Tumors of Côte-d'Or (France) were used to this end in 544 cancers diagnosed between 1976 and 1988. The proportion of the histologically confirmed cases increased annually by 13.4% (P &lt; 0.001). This was mainly due to progress in percutaneous biopsy (+25.7% per year between 1983 and 1988, P &lt; 0.001). As regards the diagnosis, ultrasonography was used more frequently (+21.9% per year, P &lt; 0.001) as well as CT scan since its introduction in 1983 (+19.6% per year, P &lt; 0.001). Pancreatic cancer was diagnosed by sonography in 16.7% of the cases in 1976 and 70.6% in 1988 (mean annual variation: +5.7%, P &lt; 0.001). The proportions for CT scan were 12.8% in 1983 and 23.5% in 1988 (mean annual variation: +3.5%, NS). There was no significant change in the use of retrograde cholangiopancreatography over time. Other diagnosis criteria were less frequently used: laparoscopy was no longer used after 1983 and intraoperative diagnosis was made less frequently (-2.5% per year, P &lt; 0.001). Endoscopy or radiographic data were rarely used as a diagnosis criterion. These changes in approaches to the diagnosis of pancreatic cancer were not accompanied by any progress in diagnosis stage, therapeutic approach or survival suggesting that when clinical symptoms become evident, pancreatic cancer is already advanced. Therapeutic advances, early diagnosis in patient at risk or identification or reasons for pancreatic cancer are the only means of progress in this problem."}, {"id": "wiki20220301en031_20492", "title": "D-dimer", "score": 0.008928571428571428, "content": "In some hospitals, they are measured by laboratories after a form is completed showing the probability score and only if the probability score is low or intermediate. This reduces the need for unnecessary tests in those who are high-probability. Performing the D-dimer test first can avoid a significant proportion of imaging tests and is less invasive. Since the D-dimer can exclude the need for imaging, specialty professional organizations recommend that physicians use D-dimer testing as an initial diagnostic. Interpretation Reference ranges The following are reference ranges for D-dimer: D-dimer increases with age. It has therefore been suggested to use a cutoff equal to patient’s age in years × 10 µg/L (or x 0.056 nmol/L) for patients aged over 50 years for the suspicion of venous thromboembolism (VTE), as it decreases the false positive rate without substantially increasing the false negative rate."}, {"id": "pubmed23n0292_3251", "title": "[Diagnostic localization of insulinoma. Experiences with 25 patients with solitary tumors].", "score": 0.008928571428571428, "content": "The most effective way to localize the mostly small ( &lt; 2 cm), benign and solitary insulinomas is still under discussion. Especially the evaluation of the different preoperative localization methods is not clarified. The aim of our study was to support the ongoing discussion in that matter. In total 25 patients have been included in our study since 1987. All showed sporadic insulinomas and underwent surgery. The following preoperative localization methods had been used: ultrasonography (US): 25 patients, computed tomography (CT): 23 patients, somatostatin receptor scintigraphy (SRS): four patients since 1990, angiography: six patients, endosonography (ES): five patients since 1995, selective portal venous sampling (PVS): two patients, magnetic resonance imaging (MRI): four patients since 1993. All 25 patients underwent a bidigital palpation in combination with intraoperative ultrasonography (IOUS). Four of the 25 patients were reoperated and had a prior unsuccessful operation elsewhere. Preoperatively 19 of 25 insulinomas were localized (76%). The following sensitivity rates had been found: ultrasonography: 56%, computed tomography: 43%, endosonography: 100%, angiography: 66%, magnetic resonance imaging: 25%, selective portal venous sampling: 100%, somatostatin receptor scintigraphy: 0%. All 25 insulinomas were detected during operation, 100% by palpation in combination with intraoperative ultrasonography and 92% by palpation on its own. After an insulinoma is biochemically proven and after exclusion of a malignant metastasizing tumor by ultrasonography, all patients should be operated on. Intraoperative ultrasonography should be performed in any case. As other preoperative localization methods did not prove a convincing cost-utility-relation, one should not consider the usage of these methods before the initial operations. Before re-operations one should consider the use of costly pre-operative methods to localize insulinomas. Here endosonography and selective portal venous sampling are recommended as the first procedures of choice."}, {"id": "wiki20220301en572_13749", "title": "Muscle–eye–brain disease", "score": 0.008849557522123894, "content": "The genome determination helps to distinguish other congenital muscular dystrophies before and after birth. However, only some laboratories provide prenatal genetic test to screen for MEB. Medical imaging MEB can be diagnosed with medical imaging by the shared patterns of brain structural abnormalities. Common practice includes magnetic resonance imaging (MRI) and computerized tomography (CT). They can show the enlargement of ventricle, absence or degeneration of septum pellucidum, pachygyric symptoms, abnormalities in corpus collosum, lissencephaly. Fetal MRI and ultrasound are used as a prenatal diagnostic tool if needed to screen for the disease. Observation like general structural malformation in the third trimester suggests congenital muscular dystrophy. Further diagnostic test is required to make confirmation."}, {"id": "pubmed23n0093_11431", "title": "[Costs in the hospital (in the diagnosis of liver and pancreatic tumors using imaging procedures)].", "score": 0.008849557522123894, "content": "The proportion of inpatients with tumours of the liver and pancreas is rather small, being only 0.25% of all the patients. Nevertheless, imaging methods may still be employed to an extent that is out of proportion to the small number of such patients. Although it is not possible at present to assess the cost arising from using the imaging methods for the diagnosis of these particular patients, an analysis of the performance data stated in the annual balance sheets of hospitals definitely shows an upward trend in respect of the use of imaging methods in diagnostic procedures in general. Despite the reductions in individual methods, such as, for example, roentgenoscopic screening or fluoroscopy, there are disproportionate increases in the total number of x-rayed patients while at the same time other imaging methods such as sonography, computed tomography and endoscopy show steep increases. It can be assumed that this applies likewise to patients with tumours of the liver and pancreas. Side by side with the tendency to extend the spectrum of use there is also an individual cost increase per case. Whereas the total cost for all Hamburg hospitals increased by 5.2% between 1985 and 1986, cost of materials and equipment rose by 6.8% and specifically in the x-ray sector by 10.2%. Seen from this point of view, the x-ray sector has become a growing cost factor. Of course that does not mean that the multitude of procedures to which the patient is subjected is always meaningful and mandatory.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en011_125004", "title": "Hydranencephaly", "score": 0.008771929824561403, "content": "Diagnosis An accurate, confirmed diagnosis is generally impossible until after birth, though prenatal diagnosis using fetal ultrasonography (ultrasound) can identify characteristic physical abnormalities. After birth, diagnosis may be delayed for several months because the infant's early behavior appears to be relatively normal. The most accurate diagnostic techniques are thorough clinical evaluation (considering physical findings and a detailed patient history); advanced imaging techniques, such as angiography, computerized tomography (CT scan), and magnetic resonance imaging (MRI); and (more rarely) transillumination. However, diagnostic literature fails to provide a clear distinction between severe obstructive hydrocephalus and hydranencephaly, leaving some children with an unsettled diagnosis."}, {"id": "pubmed23n0239_6324", "title": "[Value of sonography in the control of pancreatic cancer].", "score": 0.008771929824561403, "content": "In 152 examinations, sonography was found to be a reliable screening method in the follow-up of curative and palliative surgery of carcinoma of the pancreas. By the effected changeover from compound scanning to the high-resolution real-time B-process, it has become possible to recognize at an early date even small locoregional recurring tumours (1.5 cm diameter), metastases of lymph nodes (1-1.5 cm diameter) and hepatic filiae (1.0 cm diameter); it has also been possible to detect typical changes, such as stenoses or thromboses, at the major vessels of the upper abdomen, well in time. If the findings are not clear, CT examination will be mandatory, if necessary in conjunction with bolus injection of a contrast medium. These methods have been able to largely replace the well-known invasive procedures, such as ERCP, angiography and laparoscopy in follow-up controls."}, {"id": "wiki20220301en099_51310", "title": "List of MeSH codes (E01)", "score": 0.008695652173913044, "content": "– diagnostic techniques, cardiovascular – angiography – angiocardiography – angiography, digital subtraction – aortography – cerebral angiography – cineangiography – coronary angiography – fluorescein angiography – magnetic resonance angiography – phlebography – portography – radionuclide angiography – radionuclide ventriculography – gated blood-pool imaging – ventriculography, first-pass – angioscopy – blood circulation time – blood flow velocity – blood pressure determination – blood pressure monitoring, ambulatory – blood volume determination – capillary fragility – heart function tests – angiocardiography – ballistocardiography – cardiac output – stroke volume – cardiography, impedance – cardiotocography – coronary angiography – echocardiography – echocardiography, doppler – echocardiography, doppler, color – echocardiography, doppler, pulsed – echocardiography, stress – echocardiography, three-dimensional"}, {"id": "pubmed23n0280_13459", "title": "Cost-benefit analysis of the work-up for pancreatic cancer.", "score": 0.008695652173913044, "content": "We reviewed the records of 126 patients with pancreatic cancer to assess the value of diagnostic tests. The most commonly performed studies were computed tomography (CT) (97% of patients), endoscopic retrograde cholangiopancreatography (ERCP) (44%), and fine-needle aspiration (FNA) (41%). Of 34 patients who were found to have a mass in the body or tail of the pancreas on CT, 13 underwent ERCP; the results found by ERCP did not affect the management of the patients, whereas the results of FNA in 12 patients eliminated the need for operation. Of 14 patients with suspected metastases as evidenced by CT, the results of 3 ERCPs had no impact, whereas 5 of 7 patients who had FNAs avoided operation. Of five patients with normal results on CT, three had an ERCP that identified tumor. Of 26 patients with atypical CTs, the results of 12 of 16 ERCPs and 3 of 5 FNAs confirmed cancer. In contrast, in 48 patients with a mass in the head of the pancreas and biliary dilatation, ERCP did not alter the patients' management; only 3 of 14 patients who had FNAs avoided operation. Thus, the results of ERCP rarely altered the management of the patient when the CT showed a mass but was useful when the scan was normal or atypical. FNA was helpful in patients with cancer in the body or tail of the pancreas or with suspected metastases and in confirming the diagnosis when the CT was inconclusive."}, {"id": "pubmed23n0562_17402", "title": "[Diagnosis of venous disease].", "score": 0.008620689655172414, "content": "Venous disease of the lower extremities comprises several common conditions such as varicose veins, superficial thrombophlebitis, deep venous thrombosis and chronic venous insufficiency. The high prevalence and increasing incidence with age of these disorders impose relevant diagnostic and therapeutic concerns. Varicose veins and superficial thrombophlebitis are easily diagnosed clinically and additional diagnostic means, i. e., duplex scan, is needed for documentation or planning of surgery. Deep venous thrombosis cannot be confirmed clinically and further imaging based on clinical probability is usually required for correct diagnosis. Color-coded duplex and compression sonography have emerged as the means of choice in skilled hands. Phlebography still remains the gold standard based on its investigator-independent characteristics. However, it is becoming more and more a research tool. CT scan and magnetic resonance imaging are too expensive for broad application, but may be helpful in cases of uncertainty such as iliac vein thrombosis. CT scans, magnetic resonance imaging and sonography reveal additional information compared to phlebography about surrounding tissue and may help in establishing a differential diagnosis. Limitations of CT scan and phlebography are radiation exposure and contrast media application. Chronic venous insufficiency is basically established by medical history and clinical findings. Functional and imaging tests such as plethysmography, color-coded duplex sonography and phlebography are essential for confirming the diagnosis, evaluating a surgical intervention or defining the cause of chronic venous insufficiency."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 127]], "word_ranges": [[0, 20]], "text": "The seizures described and the EEG correspond to typical absence seizures and the initial treatment of choice is valproic acid."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The seizures described and the EEG correspond to typical absence seizures and the initial treatment of choice is valproic acid.", "full_answer_no_ref": "The seizures described and the EEG correspond to typical absence seizures and the initial treatment of choice is valproic acid.", "full_question": "A 10-year-old boy with brief episodes of distractions (< 1 minute) in which he does not respond to calls and blinks. An EEG shows spike-wave discharges at 3 cycles per second. First-line elective treatment would with:", "id": 189, "lang": "en", "options": {"1": "Valproate.", "2": "Carbamazepine.", "3": "Phenytoin.", "4": "Gabapentin.", "5": "Clonazepam."}, "question_id_specific": 71, "type": "PEDIATRICS", "year": 2013, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0516_2345", "title": "[Electroencephalography in status epilepticus in sleep (ESES) in various clinical pictures].", "score": 0.018605053651782624, "content": "Electroencephalographic epileptic status during sleep (ESES--according to Electrical Status Epilepticus in Sleep) is characterized with paroxysmal attacks of 1.5-3.5 (even to 5) Hz/s spikes and waves during NREM. A case is presented of a 7-year-old boy who had normal development until 3 years of age when epileptic attacks started. First to appear were left-sided, hemifacial twitches with orofacio-lingual deficits. Despite treatment with various types and combinations of antiepileptic medications, the attacks persisted and became more frequent. About a year after the onset of the disease, the spectrum of epileptic attacks had expanded (left-sided tonic-clonic, atonic-astatic, myoclonic, atypical absences, and then drop attacks and negative myoclonic seizures became dominant). The boy appeared mentally retarded. During the course of the disease, the diagnoses varied: hemifacial twitches, partial epilepsy, left-sided partial epilepsy, atypical benign epilepsy, Landau-Kleffner syndrome, myoclonic-astatic epilepsy. A year and a half after the onset of the disease EEG (polysomnographic whole-night recording) revealed electrical status epilepticus in sleep (ESES) with spike-wave index &gt; 85%. It appeared that this was continuous spike and wave during slow wave sleep syndrome (CSWS) with characteristic attacks, bifrontal atrophies on brain CT and right frontotemporal EEG focus. Treatment with valproate and ethosuximide combined with vigabatrin and clonazepam seemed to be effective. In the last 20 months ESES is rare and transitory, mental and neuropsychological functions have improved, but he still has several hemifacial twitches daily. The two brain MRI scans were normal. Differential diagnosis can be atypical benign epilepsy (suspected familial diagnosis). Family history of atopy, 'persistent' colds and obstructive bronchitis in the boy and treatment with antiepileptic medications (especially carbamazepine, phenobarbital and phenytoin) could have been provoking or modulating factors of the epileptic disease. So far we have not identified an epileptic syndrome because it seems that multiple clinical pictures overlap. ESES could be diagnosed using polysomnographic whole-night recording with calculated spike-wave index. Follow-up of the spike-wave index could be useful in differentiation, but not in precise diagnosis of epileptic syndrome. Clinical course could be modulated by different factors. Continuous following over a long period could be helpful in the classification of epilepsy."}, {"id": "First_Aid_Step2_628", "title": "First_Aid_Step2", "score": 0.016841369671558352, "content": "EEG typically shows 10-Hz activity during the tonic phase and slow waves during the clonic phase. Protect the airway. Treat the underlying cause if known. 1° generalized tonic-clonic seizures: Phenytoin, fosphenytoin, or valproate constitutes first-line therapy. Lamotrigine or topiramate may be used as adjunctive therapy. Secondarily generalized tonic-clonic seizures: Treatment is the same as that for partial seizures. Begin in childhood; subside before adulthood. Often familial. Hx/PE: Present with brief (5to 10-second), often unnoticeable episodes of impaired consciousness occurring up to hundreds of times per day. Patients are amnestic during and immediately after seizures and may appear to be daydreaming or staring. Eye fluttering or lip smacking is common. Dx: EEG shows classic three-per-second spike-and-wave discharges. Tx: Ethosuximide is the first-line agent."}, {"id": "pubmed23n0374_22188", "title": "Perioral myoclonia with absence seizures: a rare epileptic syndrome.", "score": 0.01632679401135777, "content": "We present the clinical and video-EEG data on an epileptic boy whose absence seizures with marked perioral movements had started at the age of 1.5 years. From age 12 years, he experienced frequent episodes of typical absence status epilepticus (ASE) lasting 1-2 hours with marked perioral myoclonia and moderate confusion. Initial therapy with carbamazepine was substituted by valproate because of worsening of the absence seizures. At the age of 17, the patient was admitted to our clinic with his usual, but long lasting ASE attack, accompanied by 2 generalized tonic-clonic convulsions. ASE was confirmed with the EEG which showed continuous 3 Hz spike and wave paroxysms with occasional normal intervals of 1-5 seconds. IV injection of clonazepam improved the clinical and EEG findings immediately. Video- EEG examination performed after a few weeks demonstrated typical absence seizures with perioral myoclonia. Based on the characteristics of seizure semiology, other clinical data and EEG findings, the patient was diagnosed as having the syndrome of \"perioral myoclonia with absence seizures\" described by Panayiotopoulos."}, {"id": "pubmed23n0759_10444", "title": "Effects of clonazepam on self-induced photoparoxysmal responses.", "score": 0.016073829605182742, "content": "A 5-year-old girl exhibited daily episodes of repetitive blinking lasting 5-10 s. Electroencephalography (EEG) revealed marked photoparoxysmal responses (PPR) at 3-20 Hz of photic stimulation and diffuse spike-wave bursts during sleep. A 24-h video EEG identified 11 episodes of repetitive blinking, 3 of which resulted in widespread spike-wave discharges. These suggested that the behavior of the patient represented self-induction of PPR. Valproate sodium was ineffective in decreasing PPR, as revealed by EEG, and the frequency of blinking episodes, but clonazepam attenuated PPR and significantly decreased the blinking behavior."}, {"id": "pubmed23n0396_4994", "title": "Gabapentin: new indication. Little impact on partial epilepsy in children between 3 and 12.", "score": 0.015811730097444382, "content": "(1) The standard treatment for partial epilepsy in children is carbamazepine. The efficacy of other antiepileptics has also been documented, either alone (phenobarbital, oxcarbazepine, valproate sodium, phenytoin), or in drug combinations (lamotrigine, topiramate). (2) A licence extension has been granted in France for gabapentin in partial epilepsy in children aged 3 to 12 years, in combination with other antiepileptics. (3) The clinical file contains no data from trials comparing gabapentin with other antiepileptics. (4) The main double-blind trial involved 247 children who were treated either with their usual treatment + gabapentin or usual treatment + placebo. Gabapentin was only moderately effective, and the overall number of responders did not differ significantly between the gabapentin and placebo groups. (5) In this trial the main adverse effects among the children on their usual treatment + gabapentin were behavioural disorders (hostility and mood swings). (6) In practice, the licensing of gabapentin for children with partial epilepsy aged between 3 and 12 years changes nothing in their practical management."}, {"id": "pubmed23n0074_6149", "title": "[Chronological change of EEG findings in a case of pyridoxine dependency seizures].", "score": 0.015110401702580475, "content": "A patient of pyridoxine dependent seizures was reported. He was born at 34 weeks' gestation and weighted 2,760 g. Apgar scores were 6 and 9 at 1 and 5 minutes, respectively. He showed the first seizure 2 hours after his birth. Phenobarbital, phenytoin, sodium valproate, diazepam and clonazepam were not effective. Pyridoxal phosphate (50 mg) was given intravenously, resulting in suppression of convulsions. However, muscle tonus was severely depressed. In EEG, a discontinuous pattern was found in quiet and indeterminate sleep on the 2nd day of life. At 5th week multifocal spikes were found, and the discontinuous pattern persisted. Ictal discharges at 13th week showed generalized, continuous, irregular and high voltage slow waves with multifocal spikes. At 27th week of life, high voltage slow waves disappeared and multifocal spike discharges decreased. At 2 years and 10 months of age, the patient was suffering from athetotic cerebral palsy and severe mental retardation. Pyridoxal phosphate at the doses of 35-40 mg/kg/day had been administered. Irritability sometimes occurred and additional 50 mg of pyridoxal phosphate controlled this irritability effectively."}, {"id": "pubmed23n0349_541", "title": "Epileptic negative myoclonus induced by carbamazepine in a child with BECTS. Benign childhood epilepsy with centrotemporal spikes.", "score": 0.014185367633643496, "content": "A 7-year-old female with benign childhood epilepsy with centrotemporal spikes developed epileptic negative myoclonus (ENM) seizures during carbamazepine (CBZ) treatment. She had experienced nocturnal partial seizures since 5 years of age. Interictal electroencephalography demonstrated typical rolandic discharges. Valproate was first initiated at 6 years of age, but the seizures were uncontrollable. Carbamazepine was added and valproate withdrawn. The frequency of partial seizures did not decrease. Moreover, she had brief episodes of tone loss in each or both arms and eye blinking several weeks after CBZ introduction. Unilateral loss of arm tone corresponded to spike-and-wave discharges in the contralateral centrotemporal region, and a loss of tone in arms was associated with bilateral synchronous discharges. Eye blinking was also related to bilateral synchronous discharges and classified as a myoclonic seizure. The ENM and myoclonic seizures disappeared soon after CBZ withdrawal. Therefore the authors concluded that CBZ induced the ENM and myoclonic seizures in this patient. CBZ sometimes induces generalized seizures in the treatment of partial epilepsy and generalized epilepsy. CBZ-induced ENM seizures should be considered when a brief lapse of tone appears during CBZ treatment."}, {"id": "pubmed23n0077_18368", "title": "Somnambulism in adults.", "score": 0.013439434129089302, "content": "We evaluated with clinical interviews and polysomnographic examinations 10 adults with the complaint of sleepwalking, often accompanied by violent behavior or self-injury. During the polysomnographic studies, 8 patients had 47 distinct somnambulistic episodes. All episodes occurred in non-REM sleep, with 91% occurring in slow-wave sleep. Contrary to previous reports, episodes were not confined to the 1st 3rd of the night. Clinical EEGs were normal in 5 of 6 patients. In the 7 patients tried on 1 or more treatment regimens, clonazepam effectively suppressed the somnambulism in 5 of 6 patients in whom it was tried, carbamazepine in 1 of 3, flurazepam in 2 of 2, and a combination of clonazepam and phenytoin in one."}, {"id": "wiki20220301en228_22021", "title": "Rolandic epilepsy", "score": 0.013315525335923587, "content": "Treatment Given the benign nature of the condition and the low seizure frequency, treatment is often unnecessary. If treatment is warranted or preferred by the child and his or her family, antiepileptic drugs can usually control the seizures easily. Carbamazepine is the most frequently used first-line drug, but many other antiepileptic drugs, including valproate, phenytoin, gabapentin, levetiracetam and sultiame have been found effective as well. Bedtime dosing is advised by some. Treatment can be short and drugs can almost certainly be discontinued after two years without seizures and with normal EEG findings, perhaps even earlier. Parental education about Rolandic epilepsy is the cornerstone of correct management. The traumatizing, sometimes long-lasting effect on parents is significant. It is unclear if there are any benefits to clobazam over other seizure medications."}, {"id": "Neurology_Adams_1482", "title": "Neurology_Adams", "score": 0.012859569980666705, "content": "Carbamazepine is effective in 70 to 80 percent of patients (600 to 1,200 mg/d), but half become tolerant over a period of several years. Other antiepileptic drugs such as phenytoin (300 to 400 mg/d), valproic acid (800 to 1,200 mg/d), clonazepam (2 to 6 mg/d), gabapentin (300 to 900 mg/d or more), pregabalin (150 to 300 mg/d), and carbamazepine in combination with other medications, suppress or shorten the duration and severity of the attacks in most patients for varying times. Baclofen may be useful in patients who cannot tolerate carbamazepine or gabapentin, but it is most effective as an adjunct to one of the anticonvulsant drugs. Capsaicin applied locally to the trigger zones or the topical instillation in the eye of an anesthetic has been helpful in some patients. By temporizing and using these drugs, one may permit a spontaneous remission to occur in perhaps 1 in 5 patients over a year or two."}, {"id": "pubmed23n0624_4104", "title": "[Idiopathic generalised epilepsies in the elderly: the viewpoint of a geriatrician].", "score": 0.012691971035251055, "content": "Long-term follow-up studies indicate a low remission rate in idiopathic generalised epilepsies (IGE) (Martinez-Juarez et al., 2006), suggesting they may persist to an advanced age. However there are few estimates of IGE frequency in the elderly. EEGs of 700 patients aged over 70 years, recorded between January 2006 and March 2007, were reviewed for anomalies consistent with IGE. We then examined the clinical history of patients with these anomalies. A persistent IGE was identified in four female patients (mean age: 79 years); in two cases it was a juvenile myoclonic epilepsy (JME) and in two an epilepsy with grand mal seizures. Seizures in three patients had begun in childhood or adolescence and in one at 40 years. Before hospitalization, few or no seizures were reported and IGE had not been diagnosed. IGE was revealed in each patient by a relatively severe event: an absence status (AS), subcontinuous myoclonic seizures or repeated convulsive generalised seizures (CGS). These events were not situation-related but in one patient the relapse of simple convulsive seizures, may have been related to the withdrawal of anti-epileptic drugs (AED) several months previously. EEG records showed generalised spikes or polyspikes and waves organised in a status epilepticus or in interictal rhythmic discharges. In one case they were evident only from a 24 hours recording. Clonazepam injection was used to suppress the AS episode and the subintrant myoclonia. After the AS, interictal generalised epileptic discharges persisted. Two of the four patients had familial history of epilepsy or febrile seizures but in no case was an epileptogenic lesion evident in brain CT scan or MRI. Clinical exams and biologic parameters were normal. All of the patients had worked and were married with children. Appropriate therapies were followed after the diagnosis of IGE. One patient with JME had been treated by Valproate which was discontinued by the general practitioner because of lethargy and replaced by Carbamazepine; seizures were aggravated under both Carbamazepine and then Lamotrigine and until the patient became seizure-free on Levetiracetam. The antiepiletic treatment was also modified in a second patient, while the two others responded well to Valproate. IGE can exacerbate in the elderly, as different types of seizures including AS, subintrant myoclonia or repeated CGS. Our data suggest persistent IGE are quite frequent in an aged population and may be underestimated due to difficulties in diagnosis. Correctly diagnosed, adjustment of AED may offer substantial clinical improvements in IGE of the elderly."}, {"id": "wiki20220301en233_6184", "title": "Panayiotopoulos syndrome", "score": 0.011637653127014829, "content": "At the age of 2 years, a girl had an autonomic status epilepticus during sleep. This was characterized by pallor, progressive impairment of consciousness, and vomiting that lasted 45 minutes. A second episode occurred after 11 months, during sleep, and consisted of impairment of consciousness, hypotonia, deviation of the eyes to the right, hypersalivation, and right-sided clonic convulsions. It was terminated after 45 minutes with rectal diazepam. Treatment with carbamazepine was initiated. After 6 months, she had a third episode similar to the previous ones, but shorter. At the age of 4 years 9 months, during an ambulatory EEG, she had another autonomic seizure with marked ictal EEG abnormalities, but again, the interictal did not show any spikes. Carbamazepine was replaced with phenobarbital. All 12 interictal EEGs during the active seizure period, 6 of them during sleep, were normal. At last follow-up at the age of 16 years, she was well, a good student, unmedicated, and"}, {"id": "pubmed23n0131_3226", "title": "Exacerbation of seizures in children by carbamazepine.", "score": 0.011597640934400844, "content": "We studied 15 children with complex partial seizures in whom one or more seizure type was exacerbated during treatment with carbamazepine. The most common seizure type that was exacerbated was generalized atypical absence (11 patients). Four patients had more frequent and severe generalized convulsive seizures. Monitoring with video-electroencephalographic telemetry suggested that the electroencephalogram can be used to predict the risk of seizure exacerbation with carbamazepine. A bilaterally synchronous spike-and-wave discharge of 2.5 to 3 cycles per second is predictive of increased atypical absence seizures with carbamazepine, whereas generalized bursts of spikes and slow waves of 1 to 2 cycles per second suggest a risk of increased generalized convulsive seizures. Carbamazepine should be used with caution in children with a mixed seizure disorder, particularly those with a generalized bilaterally synchronous discharge of 2.5 to 3 cycles per second on the electroencephalogram. The drug should be considered a possible precipitating factor in any child with an increased frequency of generalized convulsive or generalized absence seizures concomitant with administration of this anticonvulsant agent."}, {"id": "pubmed23n0481_4813", "title": "Phenytoin and carbamazepine cross reactivity: report of a case and review of literature.", "score": 0.011568627450980393, "content": "Cross reactivity between phenytoin, carbamazepine, and oxcarbazepine is reported. An 8 year old boy with partial seizures developed maculopapular rashes with itching on day 15 of carbamazepine therapy. After stopping carbamazepine, phenytoin 100 mg daily was prescribed two days later. On the 12th day of phenytoin therapy he developed cervical and axillary lymphadenopathy with fever. Lymph nodes revealed reactive hyperplasia. Oxcarbazepine 75 mg twice daily also resulted in oral and mucosa ulceration. The seizures were controlled without any side effects with sodium valproate 200 mg three times a day and gabapentin 300 mg twice a day. Due to the cross reactivity of aromatic anticonvulsants (phenytoin, carbamazepine, and oxcarbazepine), valproate or newer anticonvulsants should be used if a patient has sensitivity to these drugs."}, {"id": "wiki20220301en345_10658", "title": "Jeavons syndrome", "score": 0.011321721311475411, "content": "Management Based on anecdotal evidence, the drugs of choice are those used for other idiopathic generalized epilepsies. Valproate alone, or most probably in combination with clonazepam, levetiracetam, lamotrigine or ethosuximide, appears to be the most effective regimen. The choice of the second drug depends on the main seizure type. Clonazepam is highly efficacious in eyelid myoclonia and myoclonic jerks. Of the newer antiepileptic drugs, levetiracetam may be the most effective, because of its anti myoclonic and anti photosensitive properties. Lamotrigine is very effective in absence seizures but may exaggerate myoclonic jerks. Contra-indicated drugs are: Carbamazepine, gabapentin, oxcarbazepine, phenytoin, pregabalin, tiagabine and vigabatrin."}, {"id": "pubmed23n0358_22276", "title": "Landau-Kleffner syndrome with onset at 18 months and an initial diagnosis of pervasive developmental disorder.", "score": 0.010217113665389528, "content": "We report one of the youngest and most intensively studied cases of Landau-Kleffner syndrome, with a follow-up of 5 years. The boy developed normally until the age of 18 months when he had two attacks, possibly epileptic. Electroencephalogram (EEG) was normal. Over the next 5 months he lost his six to ten words, did not engage with other children and became mute. When he was 34 months old a child-psychiatrist suggested a diagnosis of pervasive developmental disorder or developmental dysphasia. An EEG 3 months later showed abnormalities typical of Landau-Kleffner syndrome. His non-verbal abilities were normal as well as his neurological examination and brain magnetic resonance imaging (MRI). A trial of clobazam and vigabatrin was unsuccessful. When he was 4 years and 9 months old he was treated with corticosteroids and within 3 months his vocabulary increased from the standard for 1 1/2 years of age to that for 2 1/2 years of age. His language abilities continued to improve slowly until a stagnation period at the age of 6 years and 9 months. A second course of corticosteroids improved his comprehension and vocabulary to an almost normal level, and his EEG normalized. A total of 11 EEGs were obtained; all included sleep, but continuous spike and wave during slow sleep was never documented. This report illustrates that Landau-Kleffner syndrome should be considered as an alternative diagnosis in children diagnosed with developmental dysphasia. An EEG including sleep should be considered, and in the presence of abnormalities a trial of anti-epileptic drugs or corticosteroids should be undertaken."}, {"id": "pubmed23n0380_22639", "title": "Gabapentin monotherapy: new indication. Sometimes helpful.", "score": 0.010154639175257732, "content": "(1) Gabapentin is now licensed for first-line or replacement monotherapy of partial epilepsy, in patients over 12 years of age.  (2) The assessment file concurs with current recommendations of medicines agencies.  (3) One comparative trial of first-line monotherapy showed a similar efficacy/adverse effects ratio of gabapentin and carbamazepine. Gabapentin has not been compared with valproate sodium.  (4) In one trial, involving patients with refractory epilepsy, various doses of gabapentin were added to an ongoing inadequately effective treatment, which was then gradually stopped.  Gabapentin monotherapy was considered satisfactory in a minority of patients.  (5) The adverse effects of gabapentin are limited to neuropsychological disorders, namely dizzy spells, drowsiness, fatigue and headache.  The risk of interactions is also limited.  (6) The optimal dose regimen of gabapentin is not yet established."}, {"id": "pubmed23n0305_14516", "title": "The effectiveness of clonazepam on the Rolandic discharges.", "score": 0.009970238095238095, "content": "Rolandic discharge (RD), noted in the electroencephalography (EEG) of patients with benign epilepsy in childhood with centrotemporal spikes (BECCT) has several unique features. One feature is that the amount or frequency of RDs does not correlate well with the incidence of seizures in BECCT although it is a key finding in the diagnosis of this epileptic syndrome. In this study, we examined the efficacy of antiepileptic drugs focusing on the disappearance of RDs in relationship with seizure control. Forty patients with BECCT who were not medically treated prior to this study were randomly sorted into three groups. Twenty patients were assigned for clonazepam (CZP) treatment, 10 patients for valproate (VPA) and the remaining 10 patients for carbamazepine (CBZ). Each drug was administered for 4 consecutive weeks. EEGs were recorded twice during the study, before and 4 weeks after the medication trial. The effects of each treatment on RDs were assessed. RDs disappeared in 15 of the 20 cases treated with CZP (75%) within 4 weeks while the same was observed in only one of the 10 cases treated with VPA (10%). CBZ failed to demonstrate any effect on RD. In the group treated with CZP, there were no differences in seizure incidence, seizure type and blood concentration of CZP between the patients whose RDs disappeared and those whose RDs remained."}, {"id": "pubmed23n1078_305", "title": "A 73-Year-Old Woman with Respiratory Failure and Stimulus-Induced Rhythmic, Periodic, or Ictal Discharges (SIRPIDs) in the Absence of a Detectable Brain Insult Diagnosed and Monitored by Continuous Electroencephalogram (EEG) and Treated with Valproate, Carbamazepine, and Clonazepam.", "score": 0.00980392156862745, "content": "BACKGROUND Stimulus-induced rhythmic, periodic, or ictal discharges (SIRPIDs) commonly occur in critically ill patients and can be distinguished from spontaneous epileptic seizures by continuous electroencephalogram (CEEG) monitoring. There are no current treatment guidelines for SIRPIDs. This report is of a 73-year-old woman with respiratory failure and without any detectable gross brain lesions. She had developed SIRPIDs, which were diagnosed through CEEG monitoring. She responded well to valproate, carbamazepine, and clonazepam. CASE REPORT A 73-year-old woman was admitted to the intensive care unit (ICU) with a chest infection. After 3 days, this infection was complicated by respiratory failure and coma, for which she was intubated. After that, recurrent brief episodes of abnormal head and right upper limb jerky movements with right gaze deviation occurred. Nurses noticed that these episodes occurred exclusively upon physical interaction with the patient, and lasted up to 3 minutes. No focal findings were noted on neurological examination. The brain computed tomography (CT) scan revealed no acute brain insult. CEEG revealed SIRPIDs, which abated with midazolam boluses, followed by infusion at 15 mg/hour. Later, they were controlled by valproate, carbamazepine, and clonazepam in succession, guided by CEEG data. CONCLUSIONS This report shows the importance of CEEG monitoring to diagnose SIRPIDs and monitor treatment response. It also suggests that SIRPIDs can occur even in the absence of gross brain pathology. Although there are no current treatment guidelines for SIRPIDs, the use of valproate, carbamazepine, and clonazepam can help control them, as evidenced in this case."}, {"id": "pubmed23n0310_8579", "title": "[Benign myoclonic epilepsy in childhood. A case report].", "score": 0.00980392156862745, "content": "Benign myoclonic epilepsy of childhood is a rare syndrome which appears at between 4 months and 3 years of age. The prognosis is good if diagnosed and treated early. It is characterized by many short crises (usually of 3 seconds and not more than 5-10 seconds long), proximal and cephalic jerking movements without falling to the ground, and at no particular time of the day. In the EEG polygraph background activity continues and crises coincide with generalized spike and wave or multiple spike and wave discharges of 1 to 2 seconds, accompanied by isolated myoclonic movements in the neck and deltoid muscles, which persist during NREM sleep. Benign epilepsy of childhood usually responds to monotherapy with valproic acid. In our case photosensitivity appeared at 7 years of age with persistence of generalized spikes and waves during sleep. We suggest that photosensitivity may be used as an index of the clinical course, and that treatment should continue to be given until photosensitivity disappears."}, {"id": "pubmed23n0374_22186", "title": "Epigastric sensations as an unusual manifestation of adult absence epilepsy.", "score": 0.009708737864077669, "content": "We report the case of a 39-year-old woman with onset of daily epigastric sensations associated with brief episodes of unresponsive blank stare, which have been interpreted as complex partial seizures with occasional secondary generalisation. Phenytoin as monotherapy and in combination with valproate had not been effective. During video-EEG we recorded typical absences with brief 3 second spike, and slow-wave discharges of up to 5 seconds, which were recognized by the patient herself. All absences were preceded by epigastric sensations. There was no indication of focal epilepsy. Monotherapy with valproate substantially decreased the frequency of the absences. In conclusion, this case is peculiar for several reasons: 1) late onset of absence epilepsy, 2) epigastric sensation at onset of absence seizures, 3) recognition of brief \"phantom\" absences and 4) presumable adverse effects of phenytoin."}, {"id": "pubmed23n0402_10475", "title": "A 5-year-old boy with recurrent mania successfully treated with carbamazepine.", "score": 0.009708737864077669, "content": "In the present paper the clinical symptomatology and treatment of childhood mania that was first seen in a child at 5 years of age and which re-emerged at age 7, is reported. The patient presented at the child and adolescent psychiatric outpatient clinic of Istanbul Medical University with the typical symptoms of mania such of hyperactivity, euphoria, irritability, dangerous and risky behavior, decreased sleep, and age-inappropriate sexual behavior. He was treated with carbamazepine safely and effectively without any major side-effects. Clinical phenomenology and treatment of the condition are discussed with relevant literature."}, {"id": "pubmed23n0875_25065", "title": "Vagoglossopharyngeal Neuralgia Occurred Concomitantly with Ipsilateral Hemifacial Spasm and Versive Seizure-Like Movement: A First Case Report.", "score": 0.009615384615384616, "content": "Vagoglossopharyngeal neuralgia (VGPN) is a very rare condition. VGPN with convulsive like attack is even rarer All of the cases had their head turned to the opposite side of facial pain. Hemifacial spasm occurring concurrently with VGPN has never been reported. Herein, we present the first case of VGPN that had ipsilateral hemifacial spasm and versive seizure-like movement to the same side of facial pain. We reported a 71-year-old man presenting with multiple episodes of intermittent sharp shooting pain arising on the right middle neck, followed by hemifacial spasm on right face. Then the patient became syncope while his head and gaze turned to the same side of the painful neck. Electrocardiography showed sinus arrest. Interictal Electroencephalography was normal. This patient initially responded to pregabalin for two weeks, then the symptoms became worse. Microvascular decompression and carbamazepine resulted in the complete remission of all symptoms after six months of follow-up. We could not explain the pathophysiology of unilateral versive seizure like movement."}, {"id": "pubmed23n0311_9127", "title": "Gabapentin for familial paroxysmal dystonic choreoathetosis.", "score": 0.009523809523809525, "content": "We present a 4-year-old girl with stereotyped episodes of inability to speak and dystonic posturing of the face and extremities lasting 20 minutes. An older brother and mother had similar spells in childhood. Routine and video-EEG during events were normal. The diagnosis was non-kinesigenic paroxysmal dystonic choreoathetosis since the episodes were not exacerbated by movement. Gabapentin 10 mg/kg/d eliminated most attacks."}, {"id": "pubmed23n0510_11498", "title": "Induction of epileptic negative myoclonus by oxcarbazepine in symptomatic epilepsy.", "score": 0.009433962264150943, "content": "Provocation of various seizure types including epileptic negative myoclonus and generalised atonic seizures is rarely observed in children treated with carbamazepine (CBZ). Provocation of the latter seizure types by oxcarbazepine (OXC) is not described in the literature. We report a four year-old boy with symptomatic epilepsy caused by left-sided cerebral atrophy of unknown origin who developed numerous daily drop attacks when exposed to OXC. Polygraphic analysis revealed secondary generalised precentral sharp-slow waves frequently associated with a silent period lasting for 100-150 ms in the electromyogram recorded from the deltoid and neck muscles. These seizures stopped promptly within 36 hours after discontinuation of OXC. This case demonstrates that OXC, similar to CBZ, can provoke epileptic negative myoclonus in some children with focal epilepsies. [Published with videosequences]."}, {"id": "pubmed23n0293_21369", "title": "Movement disorders associated with the use of gabapentin.", "score": 0.009345794392523364, "content": "We report two cases of unusual movement disorders associated with the use of gabapentin (GBP) in patients being treated for epilepsy who were otherwise neurologically intact. We describe two cases of unusual movement disorders associated with the use of GBP. There were significant differences in the clinical findings between the two cases. In the first case, movements were very pronounced and the patient was in oculogyric crisis. Movements in the second case were quite subtle but nonetheless problematic for the patient. In each case, discontinuation of GBP led to rapid resolution of the movements, although a single dose of lorazepam was used in the first case. Although formal electrophysiologic studies have not been performed, the movements associated with GBP use appear to be dystonic or myoclonic. Discontinuation of GBP led to rapid resolution of the movements. In severe cases, as in patients with oculogyric crisis, small doses of a benzodiazepine (BZD) appear to be efficacious and safe."}, {"id": "pubmed23n0762_5616", "title": "Antiepileptics other than gabapentin, pregabalin, topiramate, and valproate for the prophylaxis of episodic migraine in adults.", "score": 0.009174311926605505, "content": "Some antiepileptic drugs but not others are useful in clinical practice for the prophylaxis of migraine. This might be explained by the variety of actions of these drugs in the central nervous system. The present review is part of an update of a Cochrane review first published in 2004, and previously updated (conclusions not changed) in 2007. To describe and assess the evidence from controlled trials on the efficacy and tolerability of antiepileptic drugs other than gabapentin, pregabalin, topiramate, and valproate (which are the subjects of separate Cochrane reviews) for preventing migraine attacks in adult patients with episodic migraine. We searched the Cochrane Central Register of Controlled Trials (CENTRAL; The Cochrane Library 2012, Issue 12), PubMed/MEDLINE (1966 to 15 January 2013), MEDLINE In-Process (current week, 15 January 2013), and EMBASE (1974 to 15 January 2013) and handsearched Headache and Cephalalgia through January 2013. Studies were required to be prospective, controlled trials of antiepileptic drugs other than gabapentin, pregabalin, topiramate, and valproate taken regularly to prevent the occurrence of migraine attacks, to improve migraine-related quality of life, or both. Two review authors independently selected studies and extracted data. For headache frequency data, we calculated mean differences (MDs) between antiepileptic drugs and comparators (placebo, active control, or same drug in a different dose) for individual studies and pooled these across studies. For dichotomous data on responders (patients with ≥ 50% reduction in headache frequency), we calculated odds ratios (ORs) and numbers needed to treat (NNTs). We also summarised data on adverse events from placebo-controlled trials and calculated risk differences (RDs) and numbers needed to harm (NNHs). Eleven papers describing 10 unique trials met the inclusion criteria. The 10 trials reported results for nine antiepileptic drugs other than gabapentin, pregabalin, topiramate, and valproate. Six of the eight drugs investigated in placebo-controlled trials were not better than placebo in reducing headache frequency per 28-day period during treatment (clonazepam, lamotrigine, oxcarbazepine, and vigabatrin) and/or in the proportion of responders (acetazolamide, carisbamate, lamotrigine, oxcarbazepine). One prospective, randomised, double-blind, single cross-over trial of 48 patients demonstrated a significant superiority of carbamazepine over placebo in the proportion of responders (OR 11.77; 95% confidence interval (CI) 3.92 to 35.32). The NNT was 2 (95% CI 2 to 3). In a small prospective, randomised, double-blind, parallel-group trial, levetiracetam 1000 mg was significantly superior to placebo in reducing headache frequency per 28-day period during treatment (MD -2.40; 95% CI -4.52 to -0.28; 26 patients), as well as in the proportion of responders (OR 26.07; 95% CI 1.30 to 521.91; 26 patients). The NNT was 2 (95% CI 1 to 4). The same trial examined levetiracetam 1000 mg versus topiramate 100 mg and found a small but significant difference favouring topiramate in headache frequency per 28-day period during treatment (MD 1.40; 95% CI 0.14 to 2.66; 28 patients). There was no significant difference between levetiracetam and topiramate in the proportion of responders (OR 0.71; 95% CI 0.16 to 3.23; 28 patients). Finally, one trial with 75 participants examined zonisamide versus topiramate (200 and 100 mg, respectively) and found no significant difference between them in reduction of headache frequency from baseline during the third month of treatment. Adverse events for active treatment versus placebo were available for all investigated drugs except levetiracetam, vigabatrin, and zonisamide. A high prevalence of adverse events was noted for carbamazepine, with a NNH of only 2 (95% CI 2 to 4). Available evidence does not allow robust conclusions regarding the efficacy of antiepileptic drugs other than gabapentin, pregabalin, topiramate, and valproate in the prophylaxis of episodic migraine among adults. Acetazolamide, carisbamate, clonazepam, lamotrigine, oxcarbazepine, and vigabatrin were not more effective than placebo in reducing headache frequency. In one trial each, carbamazepine and levetiracetam were significantly superior to placebo in reducing headache frequency, and there was no significant difference in proportion of responders between zonisamide and active comparator. These three positive studies suffer from considerable methodological limitations."}, {"id": "pubmed23n0272_637", "title": "[Absence seizures in adulthood: four cases].", "score": 0.009174311926605505, "content": "We present four patients between 40 and 60 years of age with irregular control of seizures in adulthood, with a mean duration of history of 44 years and the following characteristics in common; a) history of childhood absence seizures beginning between age 4 and 7; b) appearance of generalized tonic-clonic seizures during adolescence; and c) persistence of typical absence seizures upon awakening during adulthood, manifested as \"clumsiness during the first half hour after awakening\". Waking and sleeping EEG polygraphs were done on all patients, including the first half hour after awakening, confirming the presence of generalized polyspike/wave during non-REM sleep and generalized spike/wave periods accompanied by simultaneous loss of consciousness, or intermittent slow generalized polyspike/wave accompanied by bradypsychia. We comment on the usefulness of valproic acid alone or in combination with ethosuximide in the treatment of these patients."}, {"id": "pubmed23n0387_23718", "title": "[Two siblings with eyelid myoclonia with absences].", "score": 0.00909090909090909, "content": "We report two siblings with eyelid myoclonia with absences. Patient 1, a 7-year-old boy, visited us because of eyelid blinking resembling a tic. He had experienced the movements since 2 years old. The diagnosis of a simple motor tic was initially made, however, the episodes worsened gradually. Patient 2, a younger brother of patient 1, was a 5-year-old boy. His eyelid blinking also began at age of 2 years. Additionally, the mother's aunt and her cousin had a history of grand mal on awakening, and the patient's cousin has febrile seizures. Their clinical features were as follows; (i) eyelid myoclonia, described as rapid, rhythmic eyelid fluttering with upward jerking of the eyes and head, lasting for 1-2 seconds; (ii) it occurred frequently each day; (iii) when it lasted for more than 2-3 seconds, it was associated with absences; (iv) both hyperventilation and photic stimulation on 18 f/c induced clinical seizures; and (v) ictal EEG revealed 3-4 c/s generalized irregular spike-waves with a duration of 1-3 seconds. Based on these characteristics, a diagnosis of eyelid myoclonia with absences was made. The present cases are the first sibling cases reported in Japan and, according to their family history, a genetic predisposition should be considered."}, {"id": "pubmed23n0075_12488", "title": "[Benign familial neonatal convulsion: clinical features of the propositus and comparison with the previously reported cases].", "score": 0.00909090909090909, "content": "A patient with benign familial neonatal convulsions was presented. The patient had the first episode of cyanosis on the second day of life. Thereafter, he also experienced focal clonic and/or multifocal clonic seizures. The interictal EEG showed no definite abnormality. Between the seizures he appeared well and physical examination was essentially normal. Treatment with phenobarbital (4 mg/kg/day, P. O.) was started and subsequently he had no further seizures until 3 months. At the age of 4 months, he was admitted to the hospital again because of generalized tonic-clonic seizures. The interictal EEG showed sporadic spikes dominantly in the right central area. The findings of ictal EEG at that time are characterized by fast spiking of increasing amplitude during the tonic phase. During the clonic phase, there are repetitive+ bursts of spikes and sharps mixed with persisting muscle potential. The termination of the convulsion is characterized by general voltage depression. Clinical characteristics such as seizure types, EEG findings, responses to antiepileptic drugs and recurrence of the seizures found in our propositus were compared with those of the patients previously reported in the literature."}, {"id": "pubmed23n0609_4702", "title": "[The effect of prophylactic therapy with valproate sodium and phenobarbital in two patients with cyclic vomiting syndrome].", "score": 0.009009009009009009, "content": "Cyclic vomiting syndrome (CVS) is a disorder characterized by recurrent, stereotypic episodes of incapacitating nausea, vomiting, and other symptoms, separated by intervals of comparative wellness. Associated symptoms include nausea, abdominal pain, headache, and motion sickness. Recently, CVS was categorized as a migraine. Case 1 was a girl aged 4 years and 11 months, who had frequent and severe episodes of vomiting since she was 3 years old. The diagnosis of CVS was established on the basis of clinical symptoms and laboratory data. Her electroencephalogram was normal. Prophylactic therapy using a single drug such as amitriptyline, carbamazepine, phenytoin, cyproheptadine, valproate sodium or phenobarbital was not effective. However, her recurring vomiting disappeared with prophylactic therapy using valproate sodium and phenobarbital. Case 2 was a boy aged 10 years and 7 months, who had frequent episodes of vomiting since he was 1 year and 10 months old. He had been receiving intravenous hyperalimentation therapy at home since infancy because of frequent vomiting and failure to thrive. His electroencephalogram showed no abnormality. Prophylactic therapy using a single drug such as diazepam, phenytoin, valproate sodium or phenobarbital was not effective. However, his recurring vomiting disappeared with prophylactic therapy using valproate sodium and phenobarbital. There were no adverse effects in both patients. The combination therapy with valproate sodium (20 - 26 mg/kg/day) and phenobarbital (4 - 5 mg/kg/day) was effective as a prophylactic therapy in these two patients. The combination therapy with valproate sodium and phanobarbital for prophylaxis of vomiting may be helpful in patients with intractable CVS."}, {"id": "article-23343_32", "title": "Idiopathic (Genetic) Generalized Epilepsy -- History and Physical -- Childhood Absence Epilepsy (CAE)", "score": 0.008930931611343982, "content": "CAE occurs in early childhood, with a peak onset between 4 to 7, usually before age 10. Clinically, CAE patients experience staring and altered awareness. They usually have only absence seizures and fewer than 20 spells per day at the time of diagnosis. The hyperventilation test provokes seizures. [34] Typical absence seizures are brief (4 to 30 seconds) vacant episodes (loss of awareness and unresponsiveness) with impairment of consciousness associated with abrupt onset and cessation, as well as behavioral arrest or staring with no post-ictal symptoms. These episodes may be associated with orofacial automatisms. [10] EEG will show typical 3 Hz spike-and-wave pattern ictally. Ethosuximide is used as first-line treatment and is effective over 50% of the time. However, ethosuximide is ineffective against non-absence seizures, so valproate is the preferred medication if another type is also present. Poor prognosis includes EEGs that have abnormal baseline slowing and generalized-tonic-clonic or myoclonic seizures as well as absence seizures. [35]"}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[229, 281]], "word_ranges": [[31, 39]], "text": "Given its benign nature, surgery is not recommended,"}, "2": {"exist": true, "char_ranges": [[229, 281]], "word_ranges": [[31, 39]], "text": "Given its benign nature, surgery is not recommended,"}, "3": {"exist": true, "char_ranges": [[229, 281]], "word_ranges": [[31, 39]], "text": "Given its benign nature, surgery is not recommended,"}, "4": {"exist": true, "char_ranges": [[229, 281]], "word_ranges": [[31, 39]], "text": "Given its benign nature, surgery is not recommended,"}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Serous cystoadenoma of the pancreas is a benign, often polycystic entity (also called microcystic adenoma) formed by collagen-producing (non-mucinous) cells. It is usually asymptomatic and is discovered as an incidental finding. Given its benign nature, surgery is not recommended, and is reserved only for symptomatic patients or those in whom the nature of the lesion is in doubt after a complete study with CT, MRI and echoendoscopy with biopsy. It is frequently sporadic, but can be associated with Von-Hippel-Lindau syndrome.", "full_answer_no_ref": "Serous cystoadenoma of the pancreas is a benign, often polycystic entity (also called microcystic adenoma) formed by collagen-producing (non-mucinous) cells. It is usually asymptomatic and is discovered as an incidental finding. Given its benign nature, surgery is not recommended, and is reserved only for symptomatic patients or those in whom the nature of the lesion is in doubt after a complete study with CT, MRI and echoendoscopy with biopsy. It is frequently sporadic, but can be associated with Von-Hippel-Lindau syndrome.", "full_question": "A 72-year-old woman with no past history of interest. After a complicated renal colic, a 2 cm cystic lesion in the tail of the pancreas, together with multiple bilateral renal cystic lesions, is found by chance in the abdominal CT scan. Endoscopic ultrasonography shows a polycystic lesion formed by multiple vesicles with central calcification in the tail of the pancreas with no connection to the duct of Wirsung. Fluid analysis is compatible with a serous cystadenoma. Of the following, which is the most correct attitude regarding the management of this patient:", "id": 508, "lang": "en", "options": {"1": "Surgical resection (corporocaudal pancreatectomy).", "2": "Endoscopic ultrasonography-guided puncture and ethanolization of the puncture.", "3": "Follow-up of the lesion by MRI.", "4": "Kidney-pancreas transplant.", "5": NaN}, "question_id_specific": 148, "type": "DIGESTIVE", "year": 2021, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0358_6063", "title": "Pancreatic lesions in von Hippel-Lindau syndrome: the coexistence of metastatic tumors from renal cell carcinoma and multiple cysts.", "score": 0.016351457840819542, "content": "Multiple cysts and benign cystadenomas of the pancreas have been documented occasionally in von Hippel-Lindau syndrome (HLS); however, the malignant involvement of the pancreas in HLS is very rare. We report a case of HLS in which metastatic tumors from renal cell carcinoma (RCC) coexisted with multiple cysts in the pancreas. A 22-year-old woman with a history of HLS had undergone a partial resection of the left kidney for RCC 3 years earlier, at which time a solid mass in the pancreatic tail and multiple pancreatic cysts were also incidentally detected by computed tomography. Over the following 3 years, the mass enlarged slightly, thus raising suspicions that it might be a primary neoplasm of the pancreas. She was referred to the Department of Surgery and Surgical Basic Science to undergo surgery. In addition to the tumor in the pancreatic tail, however, further tumors in the pancreatic head were also disclosed by preoperative celiac arteriography and intraoperative palpation and ultrasonography. A distal pancreatectomy was performed, because the enucleation of all the tumors in the pancreatic head was technically impossible and because the patient declined a total pancreatectomy. A histologic examination of the mass in the pancreatic tail revealed metastatic RCC. This case emphasizes that metastatic disease should be included in the differential diagnosis when evaluating the pancreas in a patient with HLS."}, {"id": "pubmed23n0315_18329", "title": "Chronic obstructive pancreatitis due to a pancreatic cyst in a patient with autosomal dominant polycystic kidney disease.", "score": 0.014894795127353266, "content": "Autosomal dominant polycystic kidney disease, the most frequent inherited polycystic disease, is a systemic disorder characterised by the development of numerous and bilateral kidney cysts leading to chronic renal failure. Extrarenal cysts are located mainly in the liver but also in various organs including the pancreas. To our knowledge, complications of pancreatic cysts in this disease have never been reported. The first case of painful chronic obstructive pancreatitis due to a true pancreatic cyst in a patient with autosomal dominant polycystic kidney disease is reported. Abdominal transparietal and endoscopic ultrasonography, computed tomography, and endoscopic retrograde cholangiopancreatography showed a cystic lesion in the body of the pancreas associated with upstream dilatation of the main pancreatic duct. Intraoperative ultrasonography before and after cyst fluid aspiration, and pancreatography and pathological examination of the resected distal pancreas confirmed that both main pancreatic duct enlargement and chronic pancreatitis were caused by a benign cyst. Chronic obstructive pancreatitis should be added to the extrarenal complications of autosomal dominant polycystic kidney disease."}, {"id": "pubmed23n0681_2511", "title": "[Case of von Hippel-Lindau disease diagnosed by detection of multiple pancreatic endocrine tumors and renal tumor 13 years after bilateral adrenalectomy].", "score": 0.014423076923076924, "content": "von Hippel-Lindau (VHL) syndrome is an inherited neoplastic syndrome caused by abnormity of the VHL gene found on the short arm of the chromosome 3. We reported a case of VHL disease diagnosed by the detection of multiple pancreatic endocrine tumors and renal tumor 13 years after bilateral adrenalectomy. A 40-year-old man presented with multiple pancreas tumors (maximum size 42 mm in diameter) detected by screening abdominal ultrasonography. A 23 mm renal tumor was detected by contrast computed tomography scan at that time. His past history included left retinal angioma (age 15) and bilateral adrenal pheochromocytoma (age 27). VHL was diagnosed by genetic testing. Endoscopic ultrasound-guided fine-needle aspiration biopsy of the pancreas tumor was performed, and tumor was diagnosed as an endocrine tumor. After diagnosis, distal pancreatectomy (body-tail) was performed. This was a didactic case indicating that we should suspect VHL syndrome based on past history and family history and follow such cases up strictly."}, {"id": "pubmed23n0304_9596", "title": "A case report of pancreatic mucinous cystadenoma in a patient receiving chronic hemodialysis.", "score": 0.01427555374923796, "content": "Cystic neoplasms of the pancreas are rare, accounting for about 9 to 10% of cystic pancreatic lesions. A 63-year-old man, who had been receiving chronic hemodialysis due to diabetic chronic renal failure, was admitted after the discovery of cystic masses in the pancreatic tail. Abdominal ultrasonography and computed tomography revealed multiloculated cystic tumors. Endoscopic retrograde cholangiopancreatography (ERCP) demonstrated findings consistent with malignant tumors. Preoperatively, he was thought to have a cystadenocarcinoma in the pancreatic tail for which he underwent a distal pancreatectomy and splenectomy. Histologic analysis of the resected mass established the diagnosis mucinous cystadenoma. To our knowledge, pancreatic mucinous cystadenoma occurring in a patient receiving chronic hemodialysis has not been previously reported. Therefore, we present a novel case in this report."}, {"id": "pubmed23n0678_22509", "title": "Hydatid cyst of the pancreas. An experience with six cases.", "score": 0.012734052681688353, "content": "The pancreas is an infrequent site of hydatid disease. This study aims at giving better insight into the diagnostic and managerial approach to the disease. Six patients with hydatid cysts of the pancreas. Retrospective review of the clinical records. The six patients (four men, two women) ranged in age from 18 to 68 years. Five of the cysts were primary while one had an associated cyst in the liver. Abdominal pain, vomiting, abdominal mass and dyspeptic symptoms were seen in cysts involving the body and tail. Two patients having cysts in the head of the pancreas presented with obstructive jaundice. An indirect hemagglutination test and an enzyme-linked immunoabsorbent assay were positive for the presence of specific hydatid antibodies in four patients. Abdominal ultrasonography, computed tomography and magnetic resonance cholangiopancreatography (MRCP) successfully imaged the cysts and also defined the relationship of the lesion with the pancreatic duct. All patients underwent surgical exploration. Three patients had intraoperative fine needle aspiration cytology of the cystic lesion for microscopic and electrolyte analysis. A preoperative diagnosis was possible in two patients and, in the other four, the diagnosis was made intraoperatively and confirmed on histopathological examination. with cysts located in the tail underwent a distal pancreatectomy with a splenectomy while those with cysts in the body had a pericystectomy or central pancreatectomy. Cysts of the head were treated with evacuation, partial cystectomy and tube drainage. There were no postoperative complications, and no evidence of cyst recurrence was observed during the follow-up period. All the patients were followed up at three-month intervals with a mean follow-up time of 58.7 months (rang: 4-120 months); no patient had cyst recurrence or dissemination. A hydatid cyst is an uncommon cause of cystic lesions in the pancreas and should be included in the differential diagnosis of cystic lesions of the pancreas, especially in endemic areas. Intraoperative fine needle aspirate for microscopic and electrolyte estimation seems to be an effective method for establishing a proper diagnosis. MRCP, which can depict the communication of the cystic lesion with the pancreatic duct, helps in defining the type of surgical treatment. Cysts in body and tail are best treated by resectional methods whereas, for those in the head region, a cystectomy with simple drainage is a simple, quick and effective solution."}, {"id": "pubmed23n0697_16911", "title": "Serous cystic neoplasms of the whole pancreas in a patient with von Hippel-Lindau disease.", "score": 0.012583826429980276, "content": "We describe here a case of von Hippel-Lindau (VHL) disease with a serous cystic neoplasm of the whole pancreas. The patient was a 35-year-old woman suffering from a palpable abdominal tumor. She had a history of hemangioblastomas of the cerebellum. CT revealed large solid tumors in the pancreatic head and body, and multiple cystic lesions in the whole pancreas as well as a right renal tumor. When endoscopic retrograde cholangiopancreatography (ERCP) was performed, bleeding from the duodenal papilla was detected. Since she had some distinguishing clinical features, the diagnosis of VHL disease was made. The preoperative diagnosis of the pancreatic lesion was serous cystic neoplasms with hemosuccus pancreaticus and total pancreatectomy was performed. Histological examination of the specimen revealed serous cystic neoplasms which occupied the entire pancreas. VHL cases operated on for serous cystic neoplasms of the entire pancreas are very rare."}, {"id": "wiki20220301en433_28914", "title": "Cystic lesions of the pancreas", "score": 0.012084396212547019, "content": "Cystic lesions of the pancreas are a group of pancreatic lesions characterized by a cystic appearance. They can be benign or malignant. Cystic lesions are found in 20.6% of all pancreatectomy specimens. Among this heterogeneous group, benign neoplasms predominate, particularly those with mucinous lining. Age at presentation, gender, location and tumor size are highly variable, with the exception of solid pseudopapillary tumor. Types Pancreatic intraductal papillary mucinous tumors (most common diagnosis - 52.6%) Pancreatic serous cystic tumors (20.6%) Pancreatic serous cystadenoma Pancreatic serous cystadenocarcinoma Pancreatic mucinous cystic tumors (13.4%) Pancreatic mucinous cystadenoma Pancreatic mucinous cystadenocarcinoma References Digestive system neoplasia Pancreas"}, {"id": "wiki20220301en320_14483", "title": "Pancreatic serous cystadenoma", "score": 0.01190755973364669, "content": "Pathology Treatment These lesions rarely require surgery unless they are symptomatic or the diagnosis is in question. Since these lesions do not have malignant potential, long-term observation with imaging surveillance is unnecessary. Surgery can include the removal of the head of the pancreas (a pancreaticoduodenectomy), removal of the body and tail of the pancreas (a distal pancreatectomy), or rarely removal of the entire pancreas (a total pancreatectomy). In selected cases the surgery can be performed using minimally invasive techniques such as laparoscopy. Epidemiology Serous cystadenomas of the pancreas are more common in women. SCAs are usually diagnosed in people 50-60 years of age. See also Ovarian serous cystadenoma Pancreatic mucinous cystadenoma Solid pseudopapillary neoplasm References External links Digestive system neoplasia Pancreas disorders"}, {"id": "wiki20220301en071_6934", "title": "ICD-9-CM Volume 3", "score": 0.010309532618078678, "content": "() Repair of liver () Other operations on liver () Operations on gallbladder and biliary tract () Cholecystotomy and cholecystostomy () Diagnostic procedures on biliary tract () Endoscopic retrograde cholangiopancreatography (ERCP) () Cholecystectomy () Anastomosis of gallbladder or bile duct () Incision of bile duct for relief of obstruction () Other incision of bile duct () Local excision or destruction of lesion or tissue of biliary ducts and sphincter of Oddi () Repair of bile ducts () Other operations on biliary ducts and sphincter of Oddi () Other operations on biliary tract () Operations on pancreas () Pancreatotomy () Diagnostic procedures on pancreas () Local excision or destruction of pancreas and pancreatic duct () Marsupialization of pancreatic cyst () Internal drainage of pancreatic cyst () Partial pancreatectomy () Total pancreatectomy () Radical pancreaticoduodenectomy () Transplant of pancreas () Other operations on pancreas"}, {"id": "wiki20220301en017_65286", "title": "Renal cell carcinoma", "score": 0.010106077981651376, "content": "Deciding on the benign or malignant nature of the renal mass on the basis of its localized size is an issue as renal cell carcinoma may also be cystic. As there are several benign cystic renal lesions (simple renal cyst, haemorrhagic renal cyst, multilocular cystic nephroma, polycystic kidney disease), it may occasionally be difficult for the radiologist to differentiate a benign cystic lesion from a malignant one. The Bosniak classification system for cystic renal lesions classifies them into groups that are benign and those that need surgical resection, based on specific imaging features."}, {"id": "pubmed23n0407_19902", "title": "[Pancreatic metastasis of renal cell carcinoma: report of three cases].", "score": 0.009900990099009901, "content": "The pancreas is an uncommon site of metastasis from renal cell carcinoma. Three observations are described in this review which is aimed at reporting recent data on diagnosis, prognosis and therapeutic features of this kind of metastasis 0: The average space of time between nephrectomy and the diagnosis of the metastasis was 16 years. They have been fortuitously discovered in 2 cases, in patients who did not complain of any pancreatic symptom, during abdominal ultrasonography done for another reason. In the third case, pancreatic symptoms led to the diagnosis. Endoscopic ultrasonography (EUS) was useful to diagnose multiple lesions misdiagnosed on CT-scan or MRI imaging. EUS patterns are characteristic, but histological and cytological examinations of EUS-guided needle biopsies are difficult to study according to the hypervascularized character of these metastasis. The diagnosis of pancreatic metastasis must be suggested for patients suffering from a pancreatic mass with a previous medical history of late renal cell carcinoma. According to their hypervascularized character, the negativity of EUS-guided needle biopsies could strongly direct the diagnosis. When surgery is possible, the survival rate is better than in primary pancreatic adenocarcinoma and is even better than in pancreatic metastasis from other sites."}, {"id": "wiki20220301en327_28665", "title": "Solid pseudopapillary tumour", "score": 0.00980392156862745, "content": "Diagnosis The gold standard for diagnosing solid pseudopapillary tumour of the pancreas is endoscopic ultrasound (EUS) guided fine needle aspiration (FNA) of the lesion. Management In most cases, solid pseudopapillary tumours should be resected surgically, as there is a risk of malignancy (cancer). See also Pancreatic cancer Pancreatic mucinous cystic neoplasm Serous cystadenoma of the pancreas References Digestive system neoplasia Pancreatic cancer"}, {"id": "pubmed23n0785_11552", "title": "Secondary tumors of the pancreas diagnosed by endoscopic ultrasound-guided fine-needle aspiration: a 10-year experience.", "score": 0.00980392156862745, "content": "Determining whether a pancreatic mass is a primary or secondary neoplasm is necessary for appropriate treatment. We reviewed our experience using endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) for diagnosis of pancreatic tumors to identify clinical and cytopathologic characteristics of metastatic disease. We reviewed all cases of tumors metastatic to the pancreas evaluated at The University of Texas MD Anderson Cancer Center and The Methodist Hospital in Houston, Texas during the period from 2002 to 2012. The review included cytologic specimens, clinical history, radiologic findings, primary tumor type, and clinical follow-up. We identified 66 patients with disease metastatic to the pancreas for which cytologic material was available: 38 (58%) men and 28 (42%) women, with an average age of 63 years (range, 40-89 years). Most metastases (98%) were single lesions, and nearly half were located in the head of the pancreas (30/66). The most common site of origin for these metastases was kidney (27 [41%] cases). Follow-up information was available for 65 (98%) patients, and duration of follow-up ranged from &lt;1 to 10 years (mean, 2.3 years). Thirty-three patients (50%) were alive at the time of the most recent follow-up contact. Of the 25 patients with metastatic renal cell carcinoma, clear cell type, 19 (76%) were alive at the time of the most recent follow-up. It was concluded that metastases may mimic primary pancreatic carcinomas both clinically and cytologically. Ancillary studies in conjunction with clinical history are necessary for the accurate diagnosis of FNAs of secondary pancreatic tumors."}, {"id": "wiki20220301en261_27897", "title": "Mucinous cystadenoma", "score": 0.009708737864077669, "content": "Macroscopy Microscopy Types Pancreatic Pancreatic mucinous cystadenoma or mucinous cystadenoma of the pancreas (MCN) are a type of mucinous cystic neoplasm of the pancreas. The cure rate is very high in cases on benign cystic lesions, but the case changes if malignant changes ensue. Benign cystadenomas are the most common cystic tumors of the pancreas accounting for 75% of the cases. On an average, mucinous accounts for 40%-50% of cystic tumors, and serous cytadenoma accounts for 30% of it. Mucinous cystadenomas are in the dital pancreas in about 80% of the cases and distal pancreatectomy is needed for resection. In 20% of the cases it is in the head of the pancreas. Earlier it was believed that MCN occurs exclusively in females who are middle aged. However, occasional occurrence in men have been reported, especially those who are 45 years of age or above."}, {"id": "pubmed23n0894_21477", "title": "[A Case of Multiple Pancreatic Metastases from Renal Cell Carcinoma Diagnosed Using EUS-FNA].", "score": 0.009708737864077669, "content": "The patient was a 79-year-old man, who underwent left nephrectomy for left renal cell carcinoma in 2007. In March 2015, he complained ofthirst, polydipsia, and polyuria. A slight elevation ofamylase levels was detected following laboratory testing. Abdominal CT revealed well-enhanced tumors in the pancreatic head and tail. MPD was dilated in the pancreatic body and tail. Endoscopic ultrasound-guided fine-needle aspiration(EUS-FNA)was used to obtain additional pathological findings. We diagnosed multiple pancreatic metastases from renal cell carcinoma using cell block sections from EUS-FNA ofthe pancreatic head tumor. We also identified worsening of diabetes control due to pancreatic disease. A subtotal stomachsparing pancreaticoduodenectomy and a distal pancreatectomy were performed in June 2015. Histological examination confirmed clear cell carcinoma metastases from RCC in both tumors. The patient remains alive without recurrence approximately 1 year after surgery. Glycemic control has improved with a decrease in insulin levels. Cell block sections from EUS-FNA are useful in the diagnosis of pancreatic disease. Although postoperative follow-up ofthe remnant pancreas is important, preservation ofthe pancreas should be considered for multiple pancreatic metastases when complete tumor removal is possible."}, {"id": "pubmed23n0920_16709", "title": "Pancreatic neuroendocrine tumor with complete replacement of the pancreas by serous cystic neoplasms in a patient with von Hippel-Lindau disease: a case report.", "score": 0.009615384615384616, "content": "von Hippel-Lindau disease is a dominantly inherited multi-system syndrome with neoplastic hallmarks. Pancreatic lesions associated with von Hippel-Lindau include serous cystic neoplasms, simple cysts, and neuroendocrine tumors. The combination of pancreatic neuroendocrine tumors and serous cystic neoplasms is relatively rare, and the surgical treatment of these lesions must consider both preservation of pancreatic function and oncological clearance. We report a patient with von Hippel-Lindau disease successfully treated with pancreas-sparing resection of a pancreatic neuroendocrine tumor where the pancreas had been completely replaced by serous cystic neoplasms, in which pancreatic function was preserved. A 39-year-old female with von Hippel-Lindau disease was referred to our institution for treatment of a pancreatic neuroendocrine tumor. Abdominal computed tomography demonstrated a well-enhanced mass, 4 cm in diameter in the tail of the pancreas, and two multilocular tumors with several calcifications, 5 cm in diameter, in the head of the pancreas. There was complete replacement of the pancreas by multiple cystic lesions with diameters ranging from 1 to 3 cm. Magnetic resonance cholangiopancreatography showed innumerable cystic lesions on the whole pancreas and no detectable main pancreatic duct. Endoscopic ultrasound-guided fine-needle aspiration of the mass in the pancreatic tail showed characteristic features of a neuroendocrine tumor. A diagnosis of pancreatic neuroendocrine tumor in the tail of the pancreas and mixed-type serous cystic neoplasms replacing the whole pancreas was made and she underwent distal pancreatectomy while avoiding total pancreatectomy. The stump of the pancreas was sutured as firm as possible using a fish-mouth closure. The patient made a good recovery and was discharged on postoperative day 9. She is currently alive and well with no symptoms of endocrine or exocrine pancreatic insufficiency 8 months after surgery. A pancreas-sparing resection should be considered for patients with pancreatic neuroendocrine tumors and complete cystic replacement of the pancreas to preserve quality of life after surgery."}, {"id": "pubmed23n0071_5301", "title": "[Two cases of pancreatic serous cystadenoma].", "score": 0.009615384615384616, "content": "Two cases of operated pancreatic serous cystadenoma were reported with radiological findings. A 67-year-old man was admitted to our hospital complaining of pain in the left upper quadrant. Ultrasonography revealed a mass having a mixed hypoechoic and echogenic pattern at the body of the pancreas. Computed tomography showed a honeycomb appearance. Angiogram showed hypervascularity and ERCP finding showed narrowing of the main pancreatic duct at the body of the pancreas. A 73-year-old man was detected incidentally to have a low density mass with central satellite appearance by computed tomography at the tail of the pancreas during the examination for the renal tumor. The mass showed hypervascularity and A-V shunt angiographically and narrowing of the main pancreatic duct at the tail of the pancreas by ERCP. Both patients underwent distal pancreatectomy. Histopathological findings showed microcystic pattern with single lining epithelial cells which were low cuboidal or flattened and contained intracytoplasmic glycogen."}, {"id": "pubmed23n0402_18051", "title": "Serous cystadenoma of the pancreas associated with pancreas divisum.", "score": 0.009523809523809525, "content": "Pancreas divisum is an embryologic anomaly of the pancreas that is characterized by a lack of fusion of the dorsal and ventral pancreatic ducts. It is rarely associated with pancreatic neoplasms. We report herein a rare association of pancreas divisum and serous cystadenoma of the pancreas. A 46-year-old Japanese woman presented with epigastralgia. Ultrasonography, computed tomography (CT) and magnetic resonance cholangiopancreatography revealed a multilocular cystic mass, measuring 7 cm in diameter, with a central stellate scar, in the body and tail of the pancreas. Angiography demonstrated a relatively hypervascular mass, suppressing the splenic vein. No arterial encasement was evident. Endoscopic retrograde pancreatography through the major papilla demonstrated only the duct of Wirsung; cannulation into the minor papilla was unsuccessful. In addition, CT showed a mildly dilated main pancreatic duct draining into the minor papilla. Distal pancreatectomy and splenectomy were performed, with the tentative diagnosis being serous cystic neoplasm of the pancreas, possibly malignant, and pancreas divisum. The cut surface of the resected specimen had a honeycomb-like appearance and the specimen consisted of multiple cysts of various sizes. Histopathological examination showed multiple cysts lined by a single layer of flat or cuboidal epithelial cells with glycogen in the cytoplasm. There was no evidence of malignancy. The histopathological diagnosis was serous cystadenoma of the pancreas. To the best of our knowledge, only three cases of serous cystadenoma of the pancreas associated with pancreas divisum have been reported. We report the fourth case of such an association, and briefly review the literature."}, {"id": "pubmed23n0620_17196", "title": "[Diagnosis and treatment of pancreatic metastasis from renal cell carcinoma].", "score": 0.009523809523809525, "content": "Pancreatic metastasis from renal cell carcinoma (RCC) is a rare event and has not been reported in our country. We report a series of 3 patients with metastatic RCC to the pancreas after radical nephrectomy at our institution. The published reports in the literature were reviewed, and the diagnosis, treatment as well as prognosis of this rare event were discussed. The data of 3 RCC patients with metastasis to the pancreas were reviewed retrospectively, including radical nephrectomy, metastatic interval, the second and third surgical removal. Survival of the three patients was analyzed and the reports in the literature were compared as well. The average interval from radical nephrectectomy to the comfirmed pancreatic metastasis was 6.6 years (range, 1.2 to 12 years). The pathological stage revealed T2N0M0 (n = 2) or T3N0M0 (n = 1), with right-sided tumor in 2 patients and left side in 1. One patient was asymptomatic, while the other two cases were symptomatic at presentation, including upper abdominal pain, weight loss, slight xanthochromia of the skin and titillation, clay stool (n = 1); irregular fever, weight loss and jaundice (n = 1). All pancreatic metastases were hypervascular on arterial stage of CT imaging. One patient had only a solitary pancreatic metastasis (n = 1), the another showed two metastatic lesions (n = 1), the third one had multiple lesions (n = 1). Surgical removal was accomplished in 2 patients: including pylorus-preserving pancreaticoduodenectomy in one, and pylorus-preserving pancreaticoduodenectomy together with partial tail resection in another one. The third one only received interventional therapy due to widespread extrapancreatic metastasis, and died of disseminated disease 11 months after the therapy. One of the above two surgically treated patients underwent the second removal due to local recurrence 2.5 years after the first removal of pancreatic metastasis. These two patients were still alive after follow-up of 8.6 years and 16.1 years, respectively. Renal cell carcinoma is an unpredictable tumor that may demonstrate very delayed metastasis even from early-stage of the disease. The pancreas is a rare site of metastasis from renal cell carcinoma. We advocate careful long-term follow-up of patients with a history of RCC. Aggressive surgical management of pancreatic metastatic lesions may provide a chance of long-term survival."}, {"id": "pubmed23n0944_20253", "title": "The importance of endoscopic ultrasound fine-needle aspiration in the diagnosis of solid pseudopapillary tumor of the pancreas: two case reports.", "score": 0.009433962264150943, "content": "Solid pseudopapillary tumor of the pancreas, otherwise known as solid and cystic tumor or Frantz tumor, is an unusual form of pancreatic carcinoma, with unknown etiopathogenesis, that accounts for 0.2 to 2.7% of all pancreatic tumors. It is defined as an exocrine pancreatic neoplasia that mainly affects women between the second and third decade of life, and its management is not well defined. Endoscopic ultrasound offers a key anatomical advantage in accessing the pancreas and endoscopic ultrasound fine-needle aspiration has become the gold standard method for the diagnosis of pancreatic lesions. Case 1: A 31-year-old white Hispanic woman presented with epigastric pain for 5 months. An abdominal ultrasound revealed a single 2 cm nodule in the uncinate process of her pancreas. Endoscopic ultrasound showed a regular, well-defined solid lesion with alternating cystic areas at the uncinate process of her pancreas, measuring 1.7 × 1.4 cm; endoscopic ultrasound fine-needle aspiration was then performed with cytopathological analysis compatible with solid pseudopapillary tumor. Body computed tomography confirmed the absence of metastases and she underwent conventional duodenopancreatectomy. However, she died 4 days after surgery due to postoperative surgical complications. Case 2: A 35-year-old Hispanic woman presented with left upper quadrant abdominal pain for 3 months, associated with a palpable mass at this region. A computed tomography scan showed a solitary nodule in the pancreatic body. Endoscopic ultrasound showed a regular, well-defined, homogeneous lesion with small anechoic (cystic) areas, measuring 2 × 2 cm, in between the pancreatic body and neck. Endoscopic ultrasound fine-needle aspiration was performed and cytopathological analysis was suggestive of a pseudopapillary solid tumor. She underwent a body-tail laparoscopic pancreatectomy with splenectomy. Nine months after the diagnosis, she remains asymptomatic, continuing regular follow-up in the oncology out-patient clinic. Solid pseudopapillary tumor is a rare pancreatic malignancy. Endoscopic ultrasound fine-needle aspiration is the gold standard method to characterize and diagnose this type of pancreatic lesion, making this an invaluable tool to help guide clinical management and improve the preoperative diagnostic yield."}, {"id": "pubmed23n0780_20359", "title": "[A case of pancreatic metastasis from renal cell carcinoma 27 years after nephrectomy].", "score": 0.009433962264150943, "content": "A 78-year-old man was admitted to Ogikubo Hospital for pancreatic tumors detected by computed tomography (CT). The patient had undergone right nephrectomy for renal cell carcinoma (RCC) 27 years previously. Dynamic CT revealed a hypervascular mass in the pancreatic head and a cystic mass in the pancreatic body that were approximately 35 mm and 20 mm in size, respectively. Total pancreatectomy and splenectomy were performed. Histological examination of the resected specimens revealed metastatic tumors from RCC and they were diagnosed as clear cell type. Metastatic carcinoma of the pancreas is uncommon. Pancreatic metastasis from RCC is rare; however, it could occur many years after the initial diagnosis and treatment of the primary tumor. A long and careful follow-up that includes examination of the pancreas is mandatory after nephrectomy for RCC. In this paper, we discuss a case of RCC metastasis to the pancreas and report it in the literature. "}, {"id": "pubmed23n0379_4510", "title": "Macrocystic type of serous cystadenoma with a communication between the cyst and pancreatic duct.", "score": 0.009345794392523364, "content": "A 42-year-old woman with a cystic lesion in the head of the pancreas was evaluated by using abdominal ultrasonography, a computed tomographic scan, magnetic resonance imaging and endoscopic retrograde pancreatography. Multiple cystic lesions, 5 cm in diameter, which had papillary protrusion inside the cyst in the head of the pancreas and had the communication between the cysts and pancreatic duct, were determined. Pylorus-preserving pancreaticoduodenectomy was performed under the diagnosis of mucinous cystic neoplasm of the pancreas. Although the cut surface of the tumor showed a macrocystic tumor of 3 cm in diameter, part of the cyst wall was cavernous. A histopathological examination showed single-layered cuboidal cells, which lead to the diagnosis as being serous cystadenoma of the pancreas. Serous cystadenoma is a rare, almost benign pancreatic tumor. The macrocystic subtype of serous cystadenoma is even more rare. We describe a patient who had this macrocystic subtype of serous cystadenoma with a communication between the cyst and pancreatic duct. This case illustrates the difficulty in the diagnosis of cystic lesions in the pancreas, and might support the single category of cystic lesions of the pancreas."}, {"id": "pubmed23n0977_8237", "title": "[A Patient with Multiple Pancreatic Metastases Undergoing Total Pancreatectomy 18 Years after Renal Cell Carcinoma Resection].", "score": 0.009345794392523364, "content": "A 71-year-old woman underwent right nephrectomy for the treatment of clear cell renal cell carcinoma at the age of 53. After 15 years, surgical removal of a solitary tumor was performed in the right adrenal gland and thyroid gland; both were diagnosed as metastases of renal cell carcinoma. Eighteen years after the initial resection, computed tomography(CT) showed multiple hypervascular tumors spreading across the entire area of the pancreas. She was referred to our hospital, and endoscopic ultrasound-guided fine needle aspiration biopsy(EUS-FNA)revealed that they were metastases from the renal cell carcinoma. Total pancreatectomy and splenectomy were performed, and the patient remains alive and well with no evidence of recurrent disease 7 months after the pancreatectomy. Furthermore, her blood glucose level is well controlled with insulin therapy."}, {"id": "pubmed23n0520_2639", "title": "Mucinous cystadenoma of the pancreas associated with acute pancreatitis and concurrent pancreatic pseudocyst.", "score": 0.009259259259259259, "content": "We report an unusual occurrence of a recurrent pancreatic pseudocyst caused by an underlying mucinous cystadenoma of the distal pancreas. A 54-year old female was admitted for acute pancreatitis. Her only risk factors included the use of hydrochlorothiazide and two or three glasses of wine daily. Abdominal computed tomography (CT) done a week after onset of her symptoms showed a 5-cm cystic lesion in the tail of the pancreas suspected to be a pseudocyst. Her symptoms subsequently resolved. One month later, she had another episode of pancreatitis and an abdominal CT showed an 11 x 16 cm pseudocyst along with the previously mentioned cystic lesion. Approximately 6 weeks after her initial presentation, she was taken to the operating room for an exploratory laparotomy and cyst gastrostomy for a symptomatic pseudocyst. An intraoperative frozen section of the cyst wall showed a fibrous wall with acute and chronic inflammation without an epithelial lining. Six weeks after her cyst gastrostomy, she returned with abdominal pain, early satiety, and anorexia. Abdominal CT showed reaccumulation of fluid within the pseudocyst and endoscopic retrograde cholangiopancreatography (ERCP) revealed a normal caliber pancreatic duct with an abrupt cutoff at the distal duct. She underwent exploratory laparotomy with drainage of 3 L of fluid from the pancreatic pseudocyst. After gaining access to the lesser sac, a 6-cm cystic lesion was identified in the tail of the pancreas. She underwent a distal pancreatectomy and splenectomy. The intraoperative and final pathology confirmed the presence of a benign mucinous cystadenoma. The patient had an uneventful recovery, began to tolerate oral intake, and was discharged 7 days after surgery. The differentiation between a pancreatic pseudocyst and benign cystic neoplasms of the pancreas is crucial to determine treatment options. Cystic neoplasms of the pancreas, whether mucinous or serous, have the potential to harbor malignancy, and resection is recommended."}, {"id": "pubmed23n0834_17354", "title": "Macrocystic serous cystadenoma of the pancreas: Report of 4 cases.", "score": 0.009174311926605505, "content": "Macrocystic serous cystadenomas (MaSCA) are rare benign tumor of the pancreas which represent an atypical macroscopic morphologic variant of serous cystadenomas (SCA). They are characterized by a limited number of cysts with a diameter of &gt;2 cm and share imaging features overlapping those of mucinous cystic neoplasm (MCN) and branch-duct intraductal papillary mucinous neoplasm (BD-IPMN), thus frequently making the pre-operative radiologic diagnosis difficult. Four cases of MaSCA, which were surgically treated in our structure, are reported. Two women (62 and 39 year-old) presented with upper abdominal pain and palpable mass underwent CT with evidence of a lobulated cystic neoformation (98 × 70 and 94 × 75 mm respectively) originating from the body and the tail of the pancreas respectively. They underwent distal pancreatectomy for suspected MCN. A 38 year-old woman underwent laparoscopic distal pancreatectomy because of the incidental finding of an unilocular cystic lesion in the pancreatic tail (23 mm) of indeterminate origin (MCN, SCA or metastasis). In a 40 year-old woman, admitted for acalculous acute pancreatitis, an unilocular cystic lesion in the body of the pancreas (62 mm) was detected and confirmed after 2 months at CT, therefore she underwent distal pancreatectomy for suspected pseudocyst or SCA. In all of the 4 patients the histological examination of the specimens revealed a MaSCA. Imaging techniques have a low diagnostic power in terms of differentiation of MaSCA from malignant lesions (as MCNs and BD-IPMN). In the clinical practise of MaSCA, surgery appears to gain indications that are wider than those correlated to the pathologic outcome, because of the necessity of a correct differential diagnosis from potentially malignant cystic tumors and the frequent symptoms requiring treatment."}, {"id": "pubmed23n0920_19581", "title": "Asymptomatic Pancreatic Metastasis from Renal Cell Carcinoma Diagnosed 21 Years after Nephrectomy.", "score": 0.009174311926605505, "content": "This report presents our experience with a case of pancreatic metastasis of renal cell carcinoma (RCC) at a long-term follow-up after nephrectomy. A 73-year-old man underwent nephrectomy for right RCC 21 years ago; computed tomography (CT) scanning on routine follow-up revealed a solid mass in the tail of the pancreas, and magnetic resonance imaging (MRI) showed some tumors in the head and tail of the pancreas. The patient was asymptomatic and allergic to contrast medium. Therefore we could not perform contrast CT/MRI for further examination to diagnose pancreatic tumors. We undertook endoscopic ultrasonography (EUS) and detected a hypervascular and low echoic mass; tumor tissues were obtained by EUS-guided fine-needle aspiration (EUS-FNA). Pathological diagnosis revealed pancreatic metastasis of clear cell RCC; this was similar to the pathological findings of tumor tissues initially obtained by nephrectomy. EUS-FNA was extremely useful for the definitive diagnosis of a rare type of pancreatic tumor."}, {"id": "pubmed23n0738_7497", "title": "[Management of pancreatic cystic tumors in the Alberto Sabogal Sologuren hospital].", "score": 0.00909090909090909, "content": "Cystic tumors of the pancreas comprise 1% of all neoplasms of the pancreas and 10 to 15% of pancreatic cysts. There are a variety of cystic lesions, of which 90% is made up of serous cystadenomas, mucinous cystic neoplasms, intraductal mucinous neoplasms and solid pseudopapillary neoplasms. This study describes and analyzes retrospectively the clinical, radiological, surgical, pathological and follow up of 12 patients operated on for cystic tumors of the pancreas in the hospital IV Alberto Sabogal Sologuren, in the period 2005 to 2010. we found 5 (41%) serous cystadenomas with a mean age of 66 years, localized 80% in the head and 20% body; 2 (17%) mucinous cystic neoplasms with a mean age of 54, all located in body, 2 (17%) intraductal mucinous neoplasms with a mean age of 63, all located in the head, and 3 (25%) solid pseudopapillary neoplasms with a mean age of 33 years, located in body 33% and 66% in tail with a predominance of females in a ratio of 3:1. Had abdominal pain (75%), weight loss (17%) and palpable mass (17%). Of the 2 cystic mucinous neoplasms, only one have a low-grade dysplasia, of the two mucinous intraductal neoplasms, one have grade moderate dysplasia and the other with a high degree, the rest of cystic neoplasms were benign. We realize 6 Pancreaticoduodenectomy, 4 corporocaudales pancreatectomies, 2 distal pancreatectomies; of them splenectomy realize in 4 patients (2 in corporocaudal pancreatectomies and 2 distal pancreatectomies). In all cases the preoperative diagnosis was based on abdominal TEM. in 4 patients was expanded with RMN for suspicion of mucinous tumor and in 2 patients was performed CPRE for suspected intraductal tumors. Two patients coursed with atelectasis, and one patient had pancreatic fistula grade A and other mild pancreatitis post-operative. No patient was reoperated. There was no mortality post operative. Postoperative was more for the pancreaticoduodenectomy group. In a 2 year follow up, no observed recurrence and all patients are alive. Preoperative diagnosis is crucial given the differences in natural history of the spectrum of lesions. Despite improved radiographic imaging, techniques, definitive diagnosis is only made after studying the resection sample."}, {"id": "pubmed23n0557_22515", "title": "A case of pancreatic heterotopy of duodenal wall, intraductal papillary mucinous tumor and intraepithelial neoplasm of pancreas, papillary carcinoma of kidney in a single patient.", "score": 0.00909090909090909, "content": "We report a case of the contemporaneous presence of two histologically different pancreatic neoplasms, one renal cancer and one embryogenic duodenal anomaly in a single patient. A 66-year-old man underwent ultrasound examination because of urinary disorders; a solid neoformation within the inferior pole of the left kidney was observed. Computed tomography confirmed the renal lesion, but also a heterogeneous mass within the pancreatic head appeared without bile ducts dilatation. Abdominal magnetic resonance revealed a multiloculated cystic component of the pancreatic mass. A second CT scan confirmed the renal and biliary findings, but it revealed a modest enlargement of the pancreatic asymptomatic mass. A resection of the left kidney inferior pole and a pylorus-preserving pancreaticoduodenectomy were performed. Histopathologic analysis of the surgical specimen revealed mild differentiated papillary renal carcinoma, intraductal papillary mucinous adenoma of the pancreatic head, foci of intraepithelial pancreatic neoplasm and pancreatic heterotopy of duodenal muscular and submucosal layers. The coexistence of several primaries and anomalies in one patient led us to suppose a genetic predisposition to different lesions, even in the absence of known familial genetic syndromes. The study of such cases may help to improve the investigation of molecular correlations and etiological factors of different solid tumors. Nowadays, surgery is the only effective cure."}, {"id": "pubmed23n0421_14014", "title": "[Distal pancreatic resection with splenic preservation for metastasis of renal carcinoma diagnosed 24 years later from the nephrectomy].", "score": 0.009009009009009009, "content": "Renal cell carcinoma is a malignant tumor with a singular biological behaviour, presenting in some reported cases very late metastases. This report describes a case of solitary pancreatic metastasis from kidney carcinoma, operated on 24 years before, that appears exceptional because of the long disease-free period after nephrectomy and the unusual metastatic site. The 73-year-old woman concluded the follow-up several years before; she presented aspecific abdominal pain and ultrasonographic examination and CT-scan revealed the presence of a mass in the pancreatic istmus. The mass was excised with splenic preservation and was diagnosed to be a pancreatic metastasis from clear cell renal carcinoma. We discuss the diagnostic and therapeutic features of this tumors. It appears important to obtain the diagnosis preoperatively, because good results may be obtained with surgery, justifying an aggressive surgical approach."}, {"id": "pubmed23n0410_19542", "title": "A giant retention cyst of the pancreas (cystic dilatation of dorsal pancreatic duct) associated with pancreas divisum.", "score": 0.008928571428571428, "content": "We describe a rare case of pancreas divisum associated with a giant retention cyst (cystic dilatation of the dorsal pancreatic duct), presumably formed following obstruction of the minor papilla. The patient was treated by pancreatico(cysto)jejunostomy. A 50-year-old man was admitted with complaints of increasing upper abdominal distension and body weight loss. There was no previous history of pancreatitis, gallstones, drinking, or abdominal injury. An elastic-hard tumor-like resistance was palpable in the upper abdomen. Computed tomography and ultrasound (US) examinations revealed a giant cystic lesion expanding from the pancreas head to the tail. Endoscopic retrograde cholangiopancreatography findings showed a looping pancreatic duct which drained only the head and uncinate process of the pancreas to the main papilla. A US-guided puncture to the cystic lesion revealed that the lesion continued to the main pancreatic duct in the tail of pancreas. The lesion was connected to a small cystic lesion, which was located inside the minor papilla, and ended there. The amylase level in liquid aspirated from the cyst was 37 869 IU/l, and the result of cytological examination of the liquid showed class II. A pancreatico(cysto)jejunostomy was performed, with the diagnosis being pancreas divisum associated with a retention cyst following obstruction of the minor papilla. The histological findings of a specimen from the cyst wall revealed that the wall was a pancreatic duct covered with mildly inflammatory duct epithelium; there was no evidence of neoplasm. The patient is currently well, and a CT examination 2 years after the operation showed disappearance of the cyst and normal appearance of the whole pancreas."}, {"id": "pubmed23n0938_23151", "title": "Serous Cystic Neoplasms of the Pancreas: Endoscopic Ultrasonographic Versus Computed Tomography and Magnetic Resonance Imaging Features of Surgically Removed Masses.", "score": 0.008849557522123894, "content": "Our purpose was to assess the endoscopic ultrasonography (EUS) features of serous cystic neoplasms (SCNs) of the pancreas in determining the surgical removal compared with computed tomography (CT) and magnetic resonance imaging (MRI) features. For 33 consecutive patients with 34 surgically confirmed SCNs over the past 11 years, preoperative EUS features were compared with those of CT and MRI (CT&amp;MRI). Besides the lesion size and location, a retrospective analysis of the various imaging features was performed by 2 observers to understand the characteristics that determine the need for surgical intervention in terms of multiplicity of locules, calcification, mural thickening, mural nodules, ductal communication, and main pancreatic duct dilatation in addition to the gross morphologic type: microcystic, macrocystic (&gt;1 cm), mixed, or solid. The most common gross morphologic type was mixed lesions, which consisted of microcystic and macrocystic components (15/34; 44%), followed by microcystic (38%), macrocystic (15%), and solid (3%) lesions. A minority (5/34; 18%) of the lesions showed main pancreatic duct dilatation (upstream, n = 3; downstream, n = 0; diffuse, n = 2). Mural nodules or solid components were more frequently noted in EUS (67%) than in CT&amp;MRI (25%; P = 0.001), whereas other findings showed no remarkable difference between EUS and CT&amp;MRI (P &gt; 0.05). In determining the surgical treatment of multiloculated cystic lesions, interpretation of EUS features for the presence of solid component or mural nodules should be more carefully determined, especially in the patients with suggestive features of SCN on CT or MRI to avoid unnecessary surgery."}, {"id": "pubmed23n0908_6709", "title": "Laparoscopic total pancreatectomy for multiple metastasis of renal cell carcinoma of the pancreas: a case report and literature review.", "score": 0.008849557522123894, "content": "Advances in surgical techniques and laparoscopic instruments have resulted in the development of laparoscopic pancreatic surgery. Total pancreaticoduodenectomy is performed for treating benign and borderline pancreatic disease involving the whole pancreas. Here, we report a case of metastatic renal cell carcinoma in the pancreas, treated by laparoscopic pylorus-preserving total pancreaticoduodenectomy. A 59-year-old woman was diagnosed with metastatic renal cell carcinoma. Multiple metastatic lesions were found on routine follow-up. She had a history of radical video-assisted right-nephrectomy for renal cell carcinoma (conventional type, pT1) in November 2003, without any recurrence. However, in 2014, a routine health checkup revealed multiple enhancing lesions throughout the pancreas. Positron emission tomography showed a suspicious 4-cm lesion in her left thyroid. Laparoscopic pylorus-preserving total pancreaticoduodenectomy with splenectomy was performed, along with simultaneous left total thyroidectomy with central compartment node dissection for metastatic renal cell carcinomas. The total operation time was 441 min, with an estimated blood loss of 150 ml; no transfusion was administered. Her hospital stay was 12 days. The histopath report confirmed metastatic renal cell carcinoma in the pancreas and left thyroid. Based on literature reviews, we further tried to estimate the oncologic outcome of total pancreatectomy in multiple pancreatic metastasis of renal cell carcinoma. Laparoscopic pylorus-preserving total pancreaticoduodenectomy is feasible and safe, even in cases of metastatic renal cell carcinoma."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 353]], "word_ranges": [[0, 57]], "text": "The clinical and biological picture of this patient raises suspicion of hemolytic anemia in the context of systemic lupus erythematosus. If we have to choose only one diagnostic test, and this should be the first, we are interested in confirming the presence of an autoimmune hemolytic anemia with the direct Coombs' test in order to initiate treatment."}, "2": {"exist": true, "char_ranges": [[354, 381]], "word_ranges": [[57, 62]], "text": "The other tests can wait..."}, "3": {"exist": true, "char_ranges": [[354, 381]], "word_ranges": [[57, 62]], "text": "The other tests can wait..."}, "4": {"exist": true, "char_ranges": [[354, 381]], "word_ranges": [[57, 62]], "text": "The other tests can wait..."}, "5": {"exist": true, "char_ranges": [[354, 381]], "word_ranges": [[57, 62]], "text": "The other tests can wait..."}}, "full_answer": "The clinical and biological picture of this patient raises suspicion of hemolytic anemia in the context of systemic lupus erythematosus. If we have to choose only one diagnostic test, and this should be the first, we are interested in confirming the presence of an autoimmune hemolytic anemia with the direct Coombs' test in order to initiate treatment. The other tests can wait...", "full_answer_no_ref": "The clinical and biological picture of this patient raises suspicion of hemolytic anemia in the context of systemic lupus erythematosus. If we have to choose only one diagnostic test, and this should be the first, we are interested in confirming the presence of an autoimmune hemolytic anemia with the direct Coombs' test in order to initiate treatment. The other tests can wait...", "full_question": "A 25-year-old female patient with a history of skin rash after sun exposure and occasional polyarthritis in the joints of the hands, controlled with non-steroidal anti-inflammatory drugs, presents with general malaise, progressive feeling of generalized weakness and pallor for the last 15 days. Laboratory tests showed hemoglobin 7 g/dL, MCV 108 mm/h, 150,000 platelets/mm3, 3000 leukocytes/mm3, elevated LDH, undetectable haptoglobin. In the case of choosing a single diagnostic test indicate which of the following determinations should be performed first:", "id": 151, "lang": "en", "options": {"1": "Direct Coombs test.", "2": "Antinuclear antibodies (ANA).", "3": "Vitamin B12.", "4": "Ferritin.", "5": "Folic acid."}, "question_id_specific": 70, "type": "RHEUMATOLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0060_1206", "title": "[An elderly case of systemic lupus erythematosus associated with herpes zoster, anemia, and hemiparesis].", "score": 0.01631124650926631, "content": "An elderly case of systemic lupus erythematosus (SLE) with suspected hemolytic anemia was experienced. A 70 year-old female was admitted to our hospital on December 31 with complaints of herpetic eruption. She complained of arthralgia since 3 month prior to her admission. The positive findings on examination were skin eruption in the left chest, a systolic heart murmur and a palpable elastic hard liver. Laboratory data showed raised erythrocyte sedimentation rate of 149 mm per hour, decreased Hb (10.1 g/dl), decreased hematocrit (30.0%), increased reticulocytes (33%1000), decreased thrombocytes (73,000/mm3), increased gamma-globulin (33%) and positive rheumatoid factor. During admission, she developed anemia. A stool test for occult blood was negative. The haptoglobin was 38.8 mg/dl and bone marrow aspiration showed increased erythropoiesis, suggesting features of immune hemolytic anemia, except she was negative on Coomb'test. Eye fundi were similar to case of typical bleeding observed in SLE. Concerning immunological findings, the antinuclear factor was x 1280 and the anti-dsDNA antibody was x 80, on which a diagnosis of SLE was based. She experienced numbness of the left arm and developed left hemiparesis 2 days later. Therapy with 15 mg/day prednisone obtained a good response and anemia, abnormal immunological findings and hemiparesis disappeared."}, {"id": "pubmed23n1102_1154", "title": "Late-Onset Systemic Lupus Erythematosus Associated with Autoimmune Hemolytic Anemia and Sixth Cranial Nerve Palsy.", "score": 0.013354037267080746, "content": "BACKGROUND Patients with late-onset systemic lupus erythematosus (SLE) do not present with typical SLE symptoms or serology, and this can lead to a major delay in diagnosis. We report a complex case of an older woman who developed autoimmune hemolytic anemia and sixth cranial nerve palsy that posed considerable challenges in diagnosing late-onset SLE. CASE REPORT A 78-year-old Japanese woman presented with polyarthritis associated with generalized fatigue for 2 months, who later developed diplopia. Physical examination revealed conjunctival pallor, polyarthritis, and subsequent development of sixth cranial nerve palsy. Laboratory data revealed a decreased white blood cell count; macrocytic anemia; elevated levels of lactate dehydrogenase, indirect bilirubin, and erythrocyte sedimentation rate; hypocomplementemia; positive Coombs test; antinuclear antibodies (ANAs, 1: 40); and positive anti-double-strand DNA antibodies. Lymphoma, cerebral venous sinus thrombosis, and varicella-zoster virus infection were unlikely based on head computed tomography, brain magnetic resonance imaging, and cerebrospinal fluid analysis. She was diagnosed with late-onset SLE associated with autoimmune hemolytic anemia and sixth cranial nerve palsy. The patient was successfully treated with prednisone and hydroxychloroquine. CONCLUSIONS The difficulty in diagnosing late-onset SLE with atypical presentations and uncommon complications must be recognized. SLE cannot be excluded based on a low titer of ANA in a particular subgroup such as the elderly, and the prozone effect should be considered responsible for low ANA titers. In this case, late-onset SLE was diagnosed by considering multisystem pathologies despite low ANA titers."}, {"id": "InternalMed_Harrison_1673", "title": "InternalMed_Harrison", "score": 0.012955491110830917, "content": "Fever >38.3°C (101°F) on at least two occasions 2. Illness duration of ≥3 weeks 3. 4. Diagnosis that remains uncertain after a thorough history-taking, physical examination, and the following obligatory investigations: determination of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level; platelet count; leukocyte count and differential; measurement of levels of hemoglobin, electrolytes, creatinine, total protein, alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, creatine kinase, ferritin, antinuclear antibodies, and rheumatoid factor; protein electrophoresis; urinalysis; blood cultures (n = 3); urine culture; chest x-ray; abdominal ultrasonography; and tuberculin skin test (TST). The range of FUO etiologies has evolved over time as a result of changes in the spectrum of diseases causing FUO, the widespread Percentage of Cases Due to Indicated Cause"}, {"id": "pubmed23n1072_14085", "title": "Case 291.", "score": 0.01264775413711584, "content": "History A 46-year-old woman with known mixed connective tissue disease with clinical features of scleroderma and polymyositis and who was not on specific medications was referred to our institution to assess for interstitial lung disease due to her predisposing condition. She was a nonsmoker, had no respiratory symptoms, and enjoyed good exercise tolerance. She did not have any cutaneous lesions or renal disease. There was no family history of pulmonary or systemic disease. Her routine blood test results revealed a white blood cell count of 4.6 × 10<sup9</sup/L (normal range, [4.4-10.1] × 10<sup9</sup/L), a hemoglobin level of 7.76 mmol/L (normal range, 7.26-9.18 mmol/L), a platelet count of 189 × 10<sup9</sup/L (normal range, [170-380] × 10<sup9</sup/L), a bilirubin level of 8 μmol/L (7-19 μmol/L), and a creatinine level of 63 μmol/L (45-82 μmol/L), all within normal limits. Lung function tests at presentation yielded normal results, with a diffusing capacity for carbon monoxide of 95% and a forced vital capacity of 2.29 (98% predicted value). However, this patient had an elevated serum globulin level of 47 g/L (normal range, 26-32 g/L) and an erythrocyte sedimentation rate of 36 mm/h (normal range, 0-20 mm/h), while C-reactive protein level was normal at less than 0.35 mg/dL. She was seropositive for antinuclear (titer &gt;1/720), anti-Ro, anti-La, and anti-extractable nuclear antigen antibodies. Chest radiography and CT were performed at presentation (Figs 1, 2) and 14-year follow-up (Figs 3, 4). PET/CT was performed at 7- (Fig 5, <iA</i and <iB</i) and 13-year follow-up (Fig 5, <iC</i and <iD</i). Throughout this 14-year follow-up period, she remained completely free of respiratory symptoms and continued to go for a brisk walk every day. At 14-year follow-up, there was no substantial change in serum laboratory values, but a lung function test revealed her diffusing capacity for carbon monoxide had decreased to 52%, while her forced vital capacity remained good at 95%; these findings were suggestive of interval development of restrictive lung function."}, {"id": "InternalMed_Harrison_1704", "title": "InternalMed_Harrison", "score": 0.01224433388612493, "content": "Fever ˜38.3° C (101° F) and illness lasting ˜3 weeks and no known immunocompromised state History and physical examination Stop antibiotic treatment and glucocorticoids Obligatory investigations: ESR and CRP, hemoglobin, platelet count, leukocyte count and differential, electrolytes, creatinine, total protein, protein electrophoresis, alkaline phosphatase, AST, ALT, LDH, creatine kinase, antinuclear antibodies, rheumatoid factor, urinalysis, blood cultures (n=3), urine culture, chest x-ray, abdominal ultrasonography, and tuberculin skin test"}, {"id": "pubmed23n1155_14862", "title": "Severe hemolysis with negative direct antiglobulin test: A case report.", "score": 0.012207310108509423, "content": "A 49-year-old woman with type 2 diabetes mellitus (T2DM) presented to the emergency department. Her examination showed marked pallor, exhaustion, lethargy, yellowish eyes, anorexia, nausea and vomiting. Hematuria; negative standard direct antiglobulin test (DAT); normal glucose 6 phosphate dehydrogenase (G6PD); hemoglobin (Hb), 4.8 g/dl; Mean cell volume (MCV), 91fl; platelet count, 233 × 10<sup6</sup/L; Total bilirubin, 7.0 mg/dl; Glucose, 316 mg/dl; lactate dehydrogenase (LDH), 1750U/L. Undoubtedly, therapeutic panel should have been used for hemolytic anemia. Intravenous (IV) fluids and 2 units of packed cell were transfused. Methylprednisolone with rituximab were started for the patient. After 3 weeks of the patient admission, she was discharged home with stable vital signs and Hb, 10 g/dl. We concluded in the cases that presented along with a severe drop in Hb and evidence of hemolysis which non immune hemolytic anemia is excluded in spite of negative standard DAT limited transfusion besides corticosteroids combined with rituximab, could be helpful in saving the patient."}, {"id": "wiki20220301en001_89031", "title": "Rheumatoid arthritis", "score": 0.011978933599531859, "content": "The by far most common clinical test for ACPAs is the anti-cyclic citrullinated peptide (anti CCP) ELISA. In 2008 a serological point-of-care test for the early detection of RA combined the detection of RF and anti-MCV with a sensitivity of 72% and specificity of 99.7%. Other blood tests are usually done to differentiate from other causes of arthritis, like the erythrocyte sedimentation rate (ESR), C-reactive protein, full blood count, kidney function, liver enzymes and other immunological tests (e.g., antinuclear antibody/ANA) are all performed at this stage. Elevated ferritin levels can reveal hemochromatosis, a mimic of RA, or be a sign of Still's disease, a seronegative, usually juvenile, variant of rheumatoid arthritis. Classification criteria In 2010, the 2010 ACR / EULAR Rheumatoid Arthritis Classification Criteria were introduced."}, {"id": "pubmed23n1159_5124", "title": "Severe Vitamin B12 Deficiency Presenting as Pancytopenia, Hemolytic Anemia, and Paresthesia: Could Your B12 Be Any Lower?", "score": 0.011963357943669675, "content": "Although severe vitamin B12 deficiency is rare in the United States, recent increases in the adoption of vegan lifestyles have led to a significant rise in the rates of B12 deficiency, along with its hematologic and neurologic sequelae, the latter of which is often irreversible. We describe a case of a 39-year-old male who presented with a several-month history of progressively worsening word-finding difficulties, shortness of breath, and a four-day history of bilateral hand numbness and tingling. Laboratory data revealed pancytopenia with profound anemia. Markers of hemolysis were positive, including elevated indirect bilirubin, disproportionately elevated lactate dehydrogenase (LDH), low haptoglobin, negative direct anticoagulant test, and hypoproliferative reticulocyte index. Blood smear revealed hypersegmented neutrophils and macrocytosis. Vitamin B12 levels were undetectable, and anti-intrinsic factor and parietal cell antibodies were negative. A thorough history revealed a 20-year history of strict veganism without B12 supplementation. He was transfused with packed red blood cells and started on subcutaneous B12 injections with rapid improvement of his symptoms. Early recognition of B12 deficiency causing the constellation of pancytopenia, hemolytic anemia, and neurologic symptoms is vital in preventing irreversible neurologic sequelae. This case also highlights the importance of accurate history taking to aid in early diagnosis of B12 deficiency, especially in the context of rising rates of veganism in the United States."}, {"id": "pubmed23n0354_18950", "title": "[A case of elderly-onset systemic lupus erythematosus (SLE) complicated with severe liver dysfunction and pancytopenia due to myelofibrosis].", "score": 0.01175943168494379, "content": "A 67-year-old woman was admitted to our hospital with a fever. She had been experiencing arthralgia for about one month. On admission, she had a fever of 38.5 degrees C, was anemic and was experiencing tenderness in the joints of both hands, elbows and feet. Laboratory data revealed proteinuria, urinary cylinders, pancytopenia (WBC 900/mm3, Hb 9.5 g/dl, Plt 7.8 x 10(4)/mm3), liver dysfunction (GOT 414 IU/l, GPT 140 IU/l), and hyper-gamma globulinemia. Antibiotics and granulocyte-colony stimulating factor were administered intravenously. Bone marrow aspiration was unsuccessful, but a bone marrow biopsy revealed bone marrow fibrosis. Immunological examinations were positive for antinuclear antibodies, anti-deoxyribonucleic acid (DNA) antibodies, anti-double stranded anti- DNA antibodies, as well as a decreased level of serum complement and an increased level of serum immune complexes. Tests for viral antigens and antibodies known to cause hepatitis were negative. Based on these findings, a diagnosis of SLE accompanied by liver dysfunction and bone marrow fibrosis was made. Steroid pulse therapy was initiated, but her liver function deteriorated on the first day of steroid therapy, and she died three days later. SLE accompanied by myelofibrosis is extremely rare, and only 17 and cases have been reported to date. Among these reports, the present case is the second oldest subject and the first SLE patient to suffer from both myelofibrosis and severe liver dysfunction."}, {"id": "pubmed23n0607_2166", "title": "Adult-onset Still's disease: a report of 28 cases and review of the literature.", "score": 0.011661904445478105, "content": "Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by fever, skin rash, polyarthralgias or polyarthritis, sore throat, hepatosplenomegaly, lymphadenopathy, leukocytosis, liver enzyme elevation, and high serum level of ferritin. Several kinds of skin lesions have been reported in this condition. The aim of this study was to assess the clinical and laboratory aspects of 28 patients with AOSD in central Iran. According to the diagnostic criteria of AOSD, we identified 28 patients between 2002 and 2007. We intended to describe the clinical characteristics, treatment, and outcome of our patients with AOSD. Of 28 patients with AOSD, 21 (75%) were female, 7 (25%) were male. Fever (100%), sore throat (92%), Arthralgia (92%), dermatographism (92%), typical rash (85%) and arthritis (60%) were the most common findings. The mean values of laboratory findings were as follows; C-reactive protein (CRP) level of 14.4 mg/dl, erythrocyte sedimentation rate (ESR) of 91.5 mm/h, leukocyte count of 15744.4/microl. Abnormal levels of aspartate aminotransferase and alanine aminotransferase were observed in 25 (89%) patients. Twenty patients (71%) had high ferritin values (&gt;500 ng/ml). The clinical characteristics were similar to previous series. A febrile polyarthritis was the most frequent presentation form. Dermatographism was frequently encountered phenomenon in our patients with AOSD. Being that dermatographism is a simple inducible skin reaction, along with its sensitivity in active disease, we suggest more controlled studies to validate accuracy and positive predictive value of it in convenient clinical setting in the diagnosis of AOSD and to consider including it in diagnostic criteria."}, {"id": "wiki20220301en083_35257", "title": "Autoimmune hemolytic anemia", "score": 0.011256200020244965, "content": "Laboratory investigations are carried out to determine the etiology of the disease. Following confirmation of hemolysis (seen with laboratory markers of low hemoglobin, elevated LDH, decreased haptoglobin, and elevated unconjugated bilirubin), a direct antiglobulin test (DAT)(also known as a Coomb's test) is done to show auto-immune pathogenesis with antibodies, compliment or both on the erythrocyte surface. This is followed by a monospecific DAT that identifies the specific antibody and compliment types on the erythrocyte surface. In cold agglutinin disease, the monospecific DAT is by definition positive for the compliment molecule C3d but IgM may be negative as the molecule may detach at the time of testing. The diagnosis of cold agglutinin disease is confirmed with an elevated cold agglutinin titer. A bone marrow biopsy is used in AIHA to identify a possible underlying lymphoproliferative disorder."}, {"id": "article-31170_15", "title": "Viral Arthritis -- Evaluation -- Diagnosis", "score": 0.010451335335660361, "content": "There is no single test or clinical presentation which is suggestive of viral arthritis. The diagnosis is mainly clinical, supported by other tests, including inflammatory markers, autoantibodies, and serology. A viral etiology should be suspected in a patient presenting with acute onset of arthralgias or polyarticular symptoms, especially if the duration of symptomatology has been less than six weeks. The presence of extra-articular symptoms, viz rash or lymphadenopathy, should be noted. Testing for antinuclear antibodies (ANA), rheumatoid factor (RF), and anti-citrullinated protein antibodies (ACPA) could be helpful if the features of inflammatory arthritis persist. In suspected cases of viral arthritis, the serum should be collected for serology. Serologic testing, however, must be directed against the specific viruses suspected based upon both epidemiologic and clinical data. As a general rule with serology, an IgM antibody response would be indicative of acute infection and would be converted to an IgG isotype after a few weeks. Due to the high rate of infections in the general population, for example, with EBV and hepatitis, positive IgG antibodies, especially if stable in titers, are non-diagnostic. Other laboratory features are related to the specific viral syndromes. For example, the detection of positive HBsAg elevated aminotransferases and IgM anti-HBc antibodies in a patient with recent-onset polyarthritis could provide the clue to the diagnosis of HBV-associated polyarthritis. Isolation of virus from the joint itself or viral cultures is not useful except in rare circumstances."}, {"id": "pubmed23n0303_10797", "title": "[Acute cardiac failure due to dilated cardiomyopathy in systemic lupus erythematosus with antiphospholipid antibody].", "score": 0.01044963439921423, "content": "A 33-year-old man presented malar rash in April, 1992. The rash had gradually developed and he was admitted to our hospital in February, 1994. Laboratory findings showed proteinuria of 0.5-0.8 g/ day, thrombocytopenia (4.8 x 10(4)/mm3), false positive serologic test for syphilis, anti-nuclear antibody with a speckled type at a titer of 1 : 80. Activated partial thromboplastin time was prolonged (41.3 s), and anti-beta 2-GPI antibody was strongly positive (56.6 U/ml on enzyme linked immunosorbent assay). The diagnosis of systemic lupus erythematosus with antiphospholipid syndrome was made and prednisolone 60 mg/day improved his manifestations. He could be discharged in July, 1994. Nine months after the discharge he developed dyspnea, and he was admitted to our hospital again. On admission the blood pressure was 212/170 mmHg, Levine III/VI systolic murmur was noted at the apex of heart. Significant laboratory findings showed as follows: WBC 15, 110/mm3 (Neu 73%, Lym 18%), RBC 380 x 10(4)/mm3, Hb 10.2 g/dl, Plt 20.0 x 10(4)/mm3, GOT 23 IU/l, GPT 21."}, {"id": "wiki20220301en020_68552", "title": "Paroxysmal nocturnal hemoglobinuria", "score": 0.009969604863221885, "content": "Diagnosis Blood tests in PNH show changes consistent with intravascular hemolytic anemia: low hemoglobin, raised lactate dehydrogenase, raised bilirubin (a breakdown product of hemoglobin), and decreased levels of haptoglobin; there can be raised reticulocytes (immature red cells released by the bone marrow to replace the destroyed cells) if there is no iron deficiency present. The direct antiglobulin test (DAT, or direct Coombs' test) is negative, as the hemolysis of PNH is not caused by antibodies. If the PNH occurs in the setting of known (or suspected) aplastic anemia, abnormal white blood cell counts and decreased platelet counts may be seen at this. In this case, anemia may be caused by insufficient red blood cell production in addition to the hemolysis."}, {"id": "pubmed23n1021_13220", "title": "[Meningococcemia: Different Serotypes in the Same Region].", "score": 0.009900990099009901, "content": "Meningococcal infections are important health problems causing high morbidity and mortality. Neisseria meningitidis have 13 serogroups. A, B, C, Y and W135 are the most common causes of invasive disease among those serogroups. The distribution of the serogroups differs according to the geographical regions and the age groups. In this case report, two cases of meningococcemia infected with serogroup C and Y of N.meningitidis rarely seen in our country were presented. First case was a two and a half year-old female patient who has admitted to our pediatric emergency unit with fever and rash spreading from lower extremities to her body. The patient had diffuse purpuric rash with generalized weakness and tendency to sleep at admission. The patient has been suspected as meningococcemia because of the skin rash, tendency to sleep and hypotension. Antibiotics treatment was started immediately and lumber puncture was performed. In blood tests, leukocyte count: 3600/mm3 (61% neutrophils), hemoglobin: 11.1 g/ dl, platelet count: 127.000/mm3 , C-reactive protein: 10 mg/dl, erythrocyte sedimentation rate: 6 mm/ hour, prothrombin time: 28.8 seconds (normal value= 11-16), prothrombin activity: 36%, international normalized ratio (INR): 2.13 (normal value= 1-1.5), activated partial thromboplastin time: 57.7 seconds (normal value= 25-35 sec), fibrinogen: 246 mg/dl (normal value= 200-400 mg/dl) and in cerebrospinal fluid protein: 21 mg/dl and glucose: 62 mg/dl were found. There were eight cells in the microscopic examination. Skin rashes were increased and the patient became hypotensive. No microorganisms were isolated in blood and cerebrospinal cultures. N.meningitidis serogroup C was isolated from the cerebrospinal fluid of the patient using polymerase chain reaction (PCR). The patient suffered from immune-mediated arthritis in the sixth day of treatment and nonsteroidal anti-inflammatory drugs were given. The patient has recovered with antibiotics, fresh frozen plasma and inotropic treatment. Second case was a 13 year-old male patient who has admitted three days after the first case with a pre-diagnosis of malignancy because of pancytopenia and fever. The patient had generalized weakness and a few petechial purpuric rashes at the facial region at admission. After the admission general status of the patient has worsened rapidly and he has died as a result of cardiovascular arrest. Blood tests in admission showed leukocyte count: 6000/mm3 (79% neutrophils), hemoglobin: 17.3 mg/dl, platelet count: 16.000/mm3 , C-reactive protein: 8.63 mg/dl, prothrombin time: 92.6 seconds, prothrombin activity: 10%, INR: 6.78, activated partial thromboplastin time: 231.5 seconds. Cerebrospinal fluid obtained from postmortem lumbar puncture showed no growth (protein: 95 mg/dl, glucose: 35 mg/dl) and N.meningitidis serogroup Y was detected by PCR. Two meningococcemia cases caused by two different serogroups which are rarely seen in our region in recent years were presented at the same time period in the same hospital. This case report pointed out that surveillance has a great importance in such diseases."}, {"id": "pubmed23n0771_4606", "title": "A puzzle of hemolytic anemia, iron and vitamin B12 deficiencies in a 52-year-old male.", "score": 0.00980392156862745, "content": "A 52-year-old male with no significant past medical history reports increasing generalized fatigue and weakness for the past 2 weeks. Physical examination reveals jaundice and pallor without organomegaly or lymphadenopathy. His hemoglobin was 5.9 g/dL with a mean corpuscular volume of 87.1 fL and elevated red blood cell distribution width of 30.7%. His liver function test was normal except for elevated total bilirubin of 3.7 mg/dL. Serum LDH was 701 IU/L, and serum haptoglobin was undetectable. Further investigation revealed serum vitamin B12 of &lt;30 pg/mL with elevated methylmalonic acid and homocysteine level. In addition, serum ferritin and transferrin saturation were low. The patient was diagnosed with hemolytic anemia secondary to vitamin B12 deficiency with concomitant iron deficiency anemia. "}, {"id": "pubmed23n0297_3204", "title": "[Autoimmune hemolytic anemia with eosinophilia in elderly patient].", "score": 0.00980392156862745, "content": "A 70-year-old woman was admitted to our hospital in November 1992 for evaluation of anemia. Physical examination revealed anemia, jaundice, swelling of axial and inguinal lymph nodes, and splenomegaly. Abnormal hematological findings were as follows: Hb of 3.9 g/dl, reticulocyte count of 58.2% (61.7 x 10(4)/microliters), hyperplasia of normal erythroblasts in bone marrow, and eosinophilia (21.0%, 2352/microliters) in peripheral blood. Routine laboratory examinations revealed polycolonal hypergammaglobulinemia 3.0 g/dl, a high level of serum LDH (797 IU/I) and a total bilirubin of 2.4 mg/dl (indirect, 1.6 mg/dl). The serum haptoglobin level was very low (&lt; 5 mg/dl). Results of serological examinations were as follows: IgG of 3366 mg/dl, CH50 of 16.0 U/ml, positive Coombs test 2+, and positive tests for antinuclear antibody, rheumatoid factor, and cold agglutinin. CRP was negative. PHA-stimulated lymphocyte blast formation, NK activity, and ADCC activity were found to be suppressed, and the percentage of CD4-positive lymphocytes in peripheral blood was also low. An axillary lymph node biopsy revealed reactive lymphadenitis. No signs or history suggested allergy, collagen disease, or parasitic infection. Autoimmune hemolytic anemia (AIHA) complicated by immunologic abnormalities and eosinophilia was diagnosed. Oral prednisolone markedly reduced the hemolytic anemia, eosinophilia, lymph node swelling, and splenomegaly, but NK activity remained low."}, {"id": "pubmed23n1043_7936", "title": "[A Case of Simultaneous Acute Lymphoblastic Leukemia Diagnosis with Crimean-Congo Hemorrhagic Fever].", "score": 0.009708737864077669, "content": "Crimean-Congo hemorrhagic fever (CCHF) is a zoonotic disease that can be presented with fever, fatigue, generalized joint/body pain, diarrhea and bleeding in various parts of the body. The risk of developing a severe fatal disease in humans, the possibility of being infected with aerosols and the risk of being used as a biological weapon make the disease still an important health problem all over the world as there is no a specific treatment and vaccine that has proven effective againt the virus today. The pathogenesis of the disease is not known, but vascular endothelial damage is prominent. Therefore, it progresses with thrombocytopenia, anemia, leukopenia and this hematological findings can be confused with hematological malignancies. Acute lymphoblastic leukemia (ALL) is a malignancy included in differential diagnoses and occurs as a result of mutations occuring at a stage of differentiation in the lymphoid precursor cells in the bone marrow. In this study, we present a case of ALL who was diagnosed with CCHF simultaneously. A 43-year old female patient who works in the library and does not have a chronic disease other than asthma and thyroid disorder, has admitted to our hospital with the complaints of intermittent fever, weakness, generalized joint and body pain for about 3 weeks. She had fever and the physical examination revealed bilateral cervical and right postauricular lymphadenopathies. Her aspartate aminotransferase: 77 U/L, alanine aminotransferase: 117 U/L, lactate dehydrogenase: 616 U/L, hemoglobin: 8.27 g/dl, leukocyte count: 15.690/mm3 , neutrophil count: 550/mm3 (%3.5), lymphocyte count: 6690/mm3 (%42.6), platelet count: 102.100/mm3 , C-reactive protein: 163.6 mg/L was detected and the patient was hospitalized on 5 August 2019 for further examination and treatment. Considering that the patient may have viral infection in the foreground the requested test results were detected as; anti-CMV IgM negative, anti-CMV IgG positive, anti-toxoplasma IgM negative, anti-toxoplasma IgG positive, anti-rubella IgM negative, anti-rubella IgG positive, HBsAg negative, anti-HBc IgM negative, antiHBs positive, anti-HAV IgM negative, anti-HAV IgG positive, anti-HCV negative, anti-HIV negative, EpsteinBarr virus (EBV) VCA IgM negative, EBV VCA IgG positive, EBV EBNA IgG positive. Brucella Rose Bengal and Coombs tube agglutination was found be negative. As the cytopenia of the patient deepened, the patient was accepted to have neutropenic fever and it was planned to start piperacillin-tazobactam 4 x 4.5 g/day and two units of erythrocyte replacement therapy. When the patient's history was questioned again, it was learned that she had a tick on her neck about three weeks ago and she had removed the tick herself; 4-5 days later she had the complaints of fever and flu like symptoms and also diarrhea complaints lasting for 3-4 days. Considering the current anamnesis and laboratory findings, the patient was thought to have CCHF and the patient was isolated. The serum sample taken from patient with an initial diagnosis of CCHF and sent to Department of Microbiology Reference Laboratory Public Health Agency of Turkey. The patient was referred to the Antalya Training and Research Hospital. The patient's CCHF serum result was positive. Ribavirin treatment was not initiated in the patient who was accepted to be in the convalescence period, piperacillin-tazobactam 4 x 4.5 g/day treatment was continued and supportive treatment was given. In the follow-up, as the patient's neutropenia, thrombocytopenia and lymphocytopenia still continuing, she was transferred to hematology clinic for malignancy examination and bone marrow biopsy performed by hematology and B cell ALL was diagnosed. She was accepted to be convalescent in terms of CCHF and chemotherapy was started for ALL treatment by hematology. The patient is still being followed up by the hematology clinic and allogenic hematopoietic stem cell tranplantation is planned for the patient. As a result, CCHF is a disease that can be confused with many differential diagnosis. With this case, it is aimed to draw attention to the diagnostic difficulties of CCHF and ALL and to be the first case in the literature."}, {"id": "pubmed23n0322_19509", "title": "Pancytopenia associated with 5-aminosalicylic acid use in a patient with Crohn's disease.", "score": 0.009615384615384616, "content": "We report a case of pancytopenia in a 23-year-old man with Crohn's disease who was treated with 5-aminosalicylic acid (Pentasa; Nisshin, Tokyo, Japan) 3.0 g/day. He developed fever, nausea, diarrhea, and malaise and stopped taking on the third day after commencing Pentasa. Ten days after withdrawal of Pentasa, he was admitted to hospital because of worsening symptoms. Hematologic evaluation disclosed pancytopenia: red blood cells 283 x 10(4)/mm3; white blood cells 700/mm3; and platelets 8000/mm3. Other pertinent laboratory data, including liver and renal function tests results, serology for virus infection, and serum levels of vitamin B12 and folic acids, were normal. Bone marrow examination showed a generalized hypocellular picture, suggestive of drug-induced bone marrow suppression. He received blood transfusion and recombinant human granulocyte colong-stimulating factor (filgrastim). The leucopenia and thrombocytopenia resolved on the 7th and 13th days of hospitalization, respectively. The anemia continued because of bloody stool caused by Crohn's disease. However, reticulocytes were markedly increased in number on the 13th day of hospitalization. He is well at 9 months follow-up. Excluding other causes, Pentasa-associated pancytopenia was considered. The increasing use of this agent is expected, because of the increasing number of patients with inflammatory bowel disease. Careful clinical and hematological monitoring should be performed, especially for the first 3 months, in patients beginning treatment with Pentasa. The drug should be withdrawn immediately if there is a suspicion of blood disorders."}, {"id": "pubmed23n0818_22421", "title": "[A case of autoimmune lymphoproliferactive syndrome and literature review].", "score": 0.009615384615384616, "content": "To summarize the clinical characteristics, diagnosis and treatment of a case with autoimmune lymphoproliferative syndrome (ALPS) . The patient was diagnosed as autoimmune lymphoproliferactive syndrome (ALPS) after being admitted to the Department of Rheumatism and Immunology of Tianjin Children's Hospital in February 20, 2014. The clinical characteristics, physical examination, laboratory tests, gene tests, and treatment process were analyzed and related literature was reviewed. The patient was a 16-month- old boy.Since the first month of life, he started to have repeatedly fever, diarrhea, shortness of breath, lymphadenopathy, hepatosplenomegaly, anemia (HGBmin 50 g/L) and thrombocytopenia (min 35 × 10⁹/L) . But multiple exams showed a normal peripheral blood leukocyte count, hypergammaglobulinemia (IgG 19 800 mg/L, IgA 1 710 mg/L, IgM 2 590 mg/L) and significantly increased serum vitamin B12. Flow cytometric measures showed that CD3⁺ CD4⁻ CD8⁻ T lymphocytes significantly increased ( &gt; 10%) at four times. The count of CD3⁺ TCRαβ⁺ CD4⁻ CD8⁻T lymphocytes (double negative T cells; DNTs) &gt;3% twice. The genetic test showed that 309th FAS gene area showed heterozygous mutations, the boy was diagnosed as ALPS. Added examinations of lymphocytes apoptosis induced by FAS was positive. He was treated with prednisone 15 mg once daily and immunomodulator 150 mg three times a day, while in maintaining period with normal levels of hemoglobin and platelet, the dose of prednisone was reduced gradually. Till now, the patient has been treated and observed for 8 months. We retrieved the reports of ALPS in the databases at home and abroad published in recent 10 years, more than 400 cases reported from foreign countries, but there were only 5 domestic cases. Among those, 4 had onset in infancy and 1 at 6-years of age. All the cases presented servere lymphadenopathy and hepatosplenomegaly with anemia (4 of them with hemolytic anemia) and thrombocytopenia. Three cases had a history of frequent infection, one of them had glomerulonephritis. All patient with significant high level of serum immunoglobulin ( &gt; 1.5 times upper limit of normal range), in 3 of them serum vitamin B12 was &gt; 1.5 pg/L (the other 2 cases missed the exam). In 5 cases CD3⁺ CD4⁻ CD8⁻T cells &gt; 10%, and in 2 case DNTs were 8.9% and 15.7% respectively (the other 3 cases missed the exam). Three cases were clearly detected with FAS mutations. All patients were treated with corticosteroid, 2 of them were added with mycophenolate mofetil. The therapy presented effective result in early 1-3 months, but no long-term follow-up reports were available. ALPS is a disorder of disrupted lymphocyte homeostasis caused by defective Fas-mediated apoptosis, and it is one of the primary immunodeficiency diseases. The onset of the disease occurs during infancy mainly. Clinical lymphoid hyperplasia and autoimmune phenomena are outstanding signs, which can be associated with frequent infections and allergies. The level of serum vitamin B12 &gt; 1.5 pg/L and the count of CD3⁺ CD4⁻ CD8⁻ T cell show important significance. Exact diagnosis should depend on detecting DNTs and FAS gene."}, {"id": "pubmed23n0501_23829", "title": "Klinefelter's syndrome presenting with leg ulcers.", "score": 0.009523809523809525, "content": "A 54-year-old man of Persian origin presented to our department with a 1-year history of ulcers on the right leg that had been unresponsive to numerous topical treatments, accompanied by lymphedema of the right leg. Medical history included hypergonadotropic hypogonadism, which had not been further investigated. He was treated for 20 years with testosterone IM once monthly, which he stopped a year before the current hospitalization for unclear reasons. The patient reported no congenital lymphedema. Physical examination revealed two deep skin ulcers (Figure 1) on the right leg measuring 10 cm in diameter with raised irregular inflammatory borders and a boggy, necrotic base discharging a purulent hemorrhagic exudate. Bilateral leg pitting edema and right lymphangitis with lymphadenitis were noted. He had low head hair implantment, sparse hair on the body and head, hyperpigmentation on both legs, onychodystrophia of the toenails (mainly the large toe and less prominent on the other toes), which was atrophic lichen-planus-like in appearance and needed no trimming (Figure 2), normal hand nails, oral thrush, and angular cheilitis. Other physical findings were gynecomastia, pectus excavatum, small and firm testicles, long extremities, asymmetrical goiter, systolic murmur 2/6 in left sternal border, and slow and inappropriate behavior. The patient's temperature on admission was 39 degrees C. Blood cultures were negative for bacterial growth. Results of laboratory investigations included hemoglobin (11.2 g/dL), hematocrit (26.8%), normal mean corpuscular volume and mean corpuscular hemoglobin volume, and red blood cell distribution width (16%). Blood smear showed spherocytes, slight hypochromia, anisocytosis, macrocytosis, and microcytosis. Blood chemistry values were taken for iron (4 micro g/dL [normal range 40-150 micro g/dL]), transferrin (193 mg/dL [normal range 220-400 mg/dL]), ferritin (1128 ng/mL [normal range 14-160 ng/mL]), transferrin saturation (1.5% [normal range 20%-55%]), serum folate (within normal limits), and vitamin B12 (within normal limits). Direct Coombs' test equaled positive 2 + IgG. All these values indicated anemia of chronic diseases combined with hemolytic anemia. Further blood work-up tested antinuclear antibody (positive &lt;1:80 homogeneous pattern), rheumatoid factors (143 IU/mL [positive &gt;8.5 IU/mL]), C-reactive protein (286 mg/L [normal range 0-5 mg/L]), anticardiolipin IgM antibody (9.0 monophosphoryl lipid U/mL [normal range 0-7.00 MPL U/mL]) and antithrombin III activity (135% [normal range 74%-114%]). Results of other blood tests were within normal limits or negative, including lupus anticoagulant, beta2 glycoprotein, anticardiolipin IgG Ab, anti-ss DNA Ab, C3, C4, anti-RO, anti-LA, anti-SC-70, anti-SM Ab, P-ANCA, C-ANCA, TSH, FT4, anti-T microsomal, antithyroglobulin, protein C activity, protein S free, cryoglobulins, serum immunoelectrophoresis, VDRL, hepatitis C antibodies, hepatitis B antigen, and human immunodeficiency virus. Endocrinological work-up examined luteinizing hormone (22.9 mIU/mL [normal range for adult men 0.8-6 mIU/mL]), follicle stimulating hormone (49.7 mIU/mL [normal range for adult men 1-11 mIU/mL]), testosterone (0.24 ng/mL [normal range for adult men 2.5-8.0 ng/mL]), bioavailable testosterone (0.02 ng/mL [normal range for adult men &gt;0.6 ng/mL]), and percent bioavailable test (8.1% [normal value &gt;20%]). These results indicate hypergonadotropic hypogonadism. Plasminogen activator inhibitor 1 was 6 U (normal value 5-20 U/mL). Karyotyping performed by G-banding technique revealed a 47 XXY karyotype, which is diagnostic of Klinefelter's syndrome. Doppler ultrasound of the leg ulcers disclosed partial thrombus in the distal right femoral vein. X-rays and bone scan displayed osteomyelitis along the right tibia. Histological examination of a 4-mm punch biopsy from the ulcer border revealed hyperkeratosis, acanthosis, hypergranulosis, and mixed inflammatory infiltrate containing eosinophils compatible with chronic ulcer. Multiple vessels were seen, compatible with a healing process. Direct immunofluorescence of the biopsy revealed granular IgM in the dermo-epidermal junction. Indirect immunofluorescence was negative. Thyroid function tests showed normal thyroid stimulating hormone and free throxine4. Multinodular goiter was seen on thyroid scan and ultrasound. Thyroid fine needle aspiration was compatible with multinodular goiter (normal follicular cells, free colloid, macrophages with pigment). IV treatment with amoxicillin-clavulanic acid 1 g t.i.d. was administered for 2 weeks, with a decrease in temperature and normalization of the leukocyte level. Oral antibiotic treatment with amoxicillin-clavulanic acid was continued for 10 more days, followed by 25 days of ciprofloxacin for the osteomyelitis. Local treatment included saline soakings followed by application of Promogran (Johnson &amp; Johnson, New Brunswick, NJ) and Kaltostat (ConvaTec Ltd., a Bristol-Myers Squibb Company, New York, NY) with slight improvement. At the same time, the patient was treated with warfarin sodium due to deep vein thrombosis under international normalized ratio 2-3. The patient was treated with IM testosterone once monthly for 1 year, which resulted in a reduction in the diameter and depth of the leg ulcers (Figure 3). Blood tests were not performed for follow-up of the immune state."}, {"id": "pubmed23n0083_5319", "title": "[The value of individual laboratory tests in the evaluation of inflammatory activity in rheumatoid arthritis].", "score": 0.009523809523809525, "content": "In 65 patients with definite or classical rheumatoid arthritis (RA), according to ARA criteria, and the active disease, we studied the correlation of the clinical status (the number of the swollen and tender joints) with the following laboratory parameters: sedimentation rate, C-reactive protein, serum iron level, haemoglobin content and the number of erythrocytes, leucocytes and thrombocytes in the peripheral blood. All patients received nonsteroidal antiinflammatory drugs; 25,5% of them were on small daily doses of corticosteroids, and most of them (76,5%) were receiving some of the second-line drugs. The acceleration of the sedimentation rate was the most useful laboratory parameter and was found in 96,9% of the patients, followed by an increased quantity of the alpha-2 globulin, sideropenia and a decreased level of haemoglobin. Increase in the CRP titer was found in this study to be of a rather modest value and was increased in only 40% of the RA patients."}, {"id": "wiki20220301en003_200652", "title": "Anemia", "score": 0.009494436381228835, "content": "A blood test will provide counts of white blood cells, red blood cells and platelets. If anemia appears, further tests may determine what type it is, and whether it has a serious cause. although of that, it is possible to refer to the genetic history and physical diagnosis. These tests may include: complete blood count (CBC); a CBC is used to count the number of blood cells in a sample of the blood. For anemia, it will likely to be interested in the levels of the red blood cells contained in blood (hematocrit), hemoglobin, mean corpuscular volume. determine the size and shape of red blood cells; some of red blood cells might also be examined for unusual size, shape and color. serum ferritin; This protein helps store iron in the body, a low levels of ferritin usually indicates a low levels of stored iron. serum vitamin B12; low levels usually develop an anemia, vitamin B12 is needed to make red blood cells, which carry oxygen to all parts of human body."}, {"id": "pubmed23n0266_5009", "title": "[Erythrocytosis in a 4 year-old child: diagnostic, prognostic, therapeutical problems].", "score": 0.009433962264150943, "content": "Pure erythrocytosis is rare in children. This report describes such a case. A 4 year-old boy was admitted because erythrocytosis had been found routinely before adenoidectomy. He was born in Guatemala, was adopted just after his birth, and had been living in France since that age. Clinical examination was normal. His hemogram showed: erythrocytes: 8,800,000/mm3; hemoglobin: 20.1 g/dl; hematocrit: 66.8%; reticulocytes: 262,000/mm3; platelets: 200,000/mm3; leukocytes: 6,800/mm3. The patient had been given iron salts for the past 3 months without an earlier hemogram. Total red cell mass was 1200 ml (N: 600). The myelogram was normal as was the leukocyte alkaline phosphatases, serum lysozyme and vitamin B12. Blood ferritin was low (3.5 ng/ml). In vitro cultures of erythroid precursors were normal, as was the karyotype of myeloid cells. Blood erythropoietin concentration was 20-293 mU/ml (N:4-14). All the causes of secondary polycythemia were eliminated by appropriate investigations. The patient was treated by phlebotomy in aliquots of 25 ml/kg, twice a month, for 10 months, and was given iron therapy. At the end of treatment, his hemoglobin was 14 g/dl and his hematocrit was 45%. Both progressively increased again one year later, requiring new phlebotomies. The patient was followed for 4 years but no cause for this erythrocytosis was found; it was probably congenital in origin. This case of pure erythrocytosis was associated with elevated erythropoietin production. Whether this high secretion is related to receptor dysfunction remains to be determined."}, {"id": "pubmed23n0999_21252", "title": "Adult-onset Still's Disease as a Differential Diagnosis in Prolonged Fever: Diagnosis and Treatment Experience.", "score": 0.009433962264150943, "content": "Adult onset Still's disease is a rare systemic disease that may involve many organs and may mimick many disease such as infection, autoimmune disease, and also malignancy. The diagnostic approach and treatment strategies have not been well established due to its rarity; however, there are some diagnostic criteria that may help. We present a case of 36-year old man who experienced high prolonged fever which firstly thought as infection. He also had unilateral wrist and knee joint pain and maculopapular rash. Laboratory examination showed high leukocytes count with elevated polymorphonuclear neutrophil count, high platelet count, high ferritin level, and negative results of many infection markers (typhoid antibody, procalcitonin, malaria test, blood culture, urine culture, IgM pneumonia, ASTO, syphilis test, antiHIV, HBsAg, antiHCV, etc). Chest X-ray, joint X-ray, ultrasonography, and echocardiography showed normal result. The patient was then diagnosed with Adult-onset Still's disease and received intravenous methylprednisolone and the fever was disappeared in 3 days. Six months later the arthralgia appeared again, methotrexate was administered and the pain was then relieved."}, {"id": "pubmed23n0740_15281", "title": "Prevalence of hematinics deficiency amongst female students and its correction.", "score": 0.009345794392523364, "content": "Nutritional anemia (NA) is common in India. While iron deficiency (ID) is a well recognized cause of NA, prevalence of deficiencies of other hematinics is not systematically investigated. Seventy students of a junior class of a polytechnic and 202 inmates of girl students home were taken up for study. Students were given a questionnaire to elicit anemia related symptoms. Blood was collected for complete blood count (CBC), serum ferritin, folic acid and vitamin B12. Students of polytechnic received hematinic at bed time during their menstrual periods whereas inmates of students home received hematinic at bed time, 3 days in a week. After 6 months blood tests were repeated in those who completed the treatment. CBC was done on Coulter counter and ferritin, folic acid and vitamin B12 were assayed by chemiluminescence. Students were divided into three groups-(1) Control group with Hb 12.0 g/dl or more and ferritin 15.0 ng/ml or more; (2) ID Group with Hb 12.0 g/dl or more and ferritin less than 15.0 ng/ml; and (3) Iron Deficiency Anemia (IDA) group with Hb less tha 12.0 g/dl and ferritin less than 15.0 ng/ml. Basal parameters of three groups were compared using students t test. Change in parameters with treatment was compared using paired students t test. Median age-16 years (range 10-25). Anemia ( Hb &lt; 12.0 g/dl)-94 (34.6%); MCV &lt; 80 fl-153 (56.3%); MCH &lt; 27 pg-167 (61.4%); Ferritin &lt; 15.0 ng/ml-161 (59.2%); Folic acid &lt; 3.5 ng/ml-34 (12.5%); Vitamin B12 &lt; 258 pg/ml-133 (48.9%) Pre-therapy: (1) Hb, MCV, MCH and ferritin significantly lower in ID and IDA Groups compared to control group. (2) Hb, MCV, MCH and Ferritin significantly lower in IDA Group as compared to ID Group.  POST-THERAPY: (1) IDA group showed significant increase in Hb, MCV, MCH, ferritin, folic acid and vitamin B12. (2) final Hb (11.26+1.07) and ferritin (7.46+4.81) in IDA Group were subnormal. (3) MCV, MCH, ferritin, folic acid and vitamin B12 increased significantly in ID Group and control group. (1) Nutritional anemia is common amongst asymptomatic young female students. (2) Deficiencies of iron, folic acid and vitamin B12 are common and coexist. (3) 105 mg elemental iron for 3 days in a week for 6 months is not adequate to correct IDA. (4) 105 mg iron for 3 days in a week is enough to correct ID. (5) Non-anemic individuals with ID have iron deficient erythropoiesis. (6) Non-anemic individuals without ID, in this cohort, also had iron deficient eryhtropoiesis."}, {"id": "pubmed23n0688_16038", "title": "Adult onset Still's disease: review of 41 cases.", "score": 0.009345794392523364, "content": "To describe the clinical, laboratory and radiological features, treatment and prognosis of patients with adult onset Still's disease (AOSD). Specific clinical features were retrospectively recorded in 41 patients fulfilling the Yamaguchi criteria. Patients were reviewed in two academic hospitals with a referral area of 700,000-1,000,000 inhabitants. Laboratory tests including haemogram, ferritin, biochemistry and autoimmunity were reviewed. Radiological studies, treatment and ACR functional class were determined. Forty-one patients with AOSD were identified, 25 of whom were female. Mean age at diagnosis: 38.19 years (range 17-68). Feverish polyarthritis was the most common clinical presentation. Acute phase reactants were invariably high in all patients. Serum ferritin levels were elevated in 86% of patients. Anti-cyclic citrullinated peptide antibodies (anti-CCP antibodies) were negative in all patients except one. The course of the disease was monocyclic in 44% of the patients, polycyclic in 26%, and chronic articular in 30%. ACR class was as follows: 29 (72.5%) class I, 7 (17.5%) class II, 2 (5%) class III and 2 (5%) class IV. As for the treatment received, aspirin or NSAIDs controlled the disease in eight patients (19.5%) and high-dose corticosteroids (0.5-1 mg/kg/day) in 32 (78%). Almost half of the patients (49%) required an additional diseasemodifying agent, usually methotrexate. Finally, in seven of them (17%) a biological treatment with TNF-α or specially anti-IL-1 had to be added to control the disease. The clinical and laboratory findings were similar to previous studies. Anti-CCP antibodies were almost always negative. A monocyclic course was associated with a good prognosis. Most of the patients were in ACR functional class I and II. Biological agents were required in 7 patients (17%)."}, {"id": "pubmed23n1004_25990", "title": "Rowell Syndrome in Nigeria: Systemic Lupus Erythematosus Presenting as Recurrent Erythema Multiforme in a Young Woman.", "score": 0.009259259259259259, "content": "Dear Editor, Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by diverse patterns of auto-antibody production with multi-organ affectation. Cutaneous involvement, either alone or in association with other systemic illnesses, is one of its most common manifestations (1). Dermatologic disorders like malar and discoid rashes are quite suggestive of SLE. However, the occurrence of non-specific skin lesions like erythema multiforme (EM) in patients with SLE (Rowell syndrome) can rarely occur (1). In such patients, a diagnosis of SLE may be missed or delayed in the absence of other overt clinical features of lupus. Herein we report a case of recurring EM-like eruptions as the cardinal cutaneous manifestation of previously undiagnosed, active SLE in a young Nigerian woman. A 26-year-old Nigerian woman presented with a three-day history of non-pruritic, generalized, and target-like, erythematous annular patches and plaques which mostly affected the trunk. A few lesions had presented with crusting and erosions at the time of examination (Figure 1). Associated symptoms included oral painful ulcers, low grade fever, and malaise. The patient had no other systemic symptoms and her prior drug history was not remarkable. Her erythrocyte sedimentation rate (ESR) was 66 mm/hour using the Westergren method. Screening for HIV and hepatitis B and C was negative. Herpes simplex, cytomegalovirus, and Epstein Barr viruses could not be screened for. Other baseline investigations (complete blood count, electrolytes, urea and creatinine as well as urinalysis) were within normal limits. The patient was managed as a case of EM of an unidentified inciting agent and her symptoms resolved with supportive care and antibiotics. However, she developed a recurrence about 5 weeks later, with more extensive and coalescent skin lesions (Figure 2). Additionally, there was a new onset of alopecia and pain in the small joints of the hands as well as both knees and ankles. At this time, the patient's ESR had gone up to 112 mm/h and she had developed significant proteinuria, with a protein creatinine ratio of 1.3 g/g (reference &lt;0.5 g/g). Her antinuclear antibody (ANA) titer was high (1:320) with a speckled pattern. Anti-Smith antibody was also positive. A renal biopsy was declined. A tentative diagnosis of Rowell syndrome was made. The patient was started on high-dose steroids and hydroxychloroquine 200 mg twice daily. Subsequent care included the use of mycophenolate mofetil 1 g twice daily for 6 months. This was then changed to azathioprine at 50 mg twice daily. Follow-up after 6 months showed sustained clearance of skin lesions, resolution of fever and joint pains, as well as improvement in the renal profile, with a urine protein-creatinine ratio of 0.77 g/g. The presence of systemic lupus erythematosus, EM-like lesions, and a speckled pattern of antinuclear antibody in our patient fulfils the revised diagnostic criteria for RS put forward by Zeitouni et al. at the turn of the twenty-first century (2). Considering the rarity of EM-like lesions in SLE and the possibility of constitutional symptoms in EM, a diagnosis of RS may be readily overlooked in patients like the one described, whose major cutaneous manifestation of severe active SLE was EM-like lesions. In contrast to classic EM, where skin lesions are concentrated in the extremities, a predominant truncal distribution of EM-like lesions as found in our patient may favor a clinical consideration of RS (3). However, some authors have challenged the existence of Rowell syndrome as a distinct clinical laboratory entity. Arguments put forward in this regard include the fact that none of the immunological markers that have been described in RS are specific to any disorder. Additionally, the annular polycyclic dermatosis seen in sub-acute cutaneous lupus erythematosus (SCLE) can be difficult to clinically and histologically differentiate from EM (4,5). Patients with SLE also have a higher likelihood of developing adverse drug reactions (6). The inherent complexity of SLE may make for delayed and oftentimes difficult diagnosis, especially in a country where immunologic tests are expensive and rheumatologists are scarce. When patients do occasionally present with recurrences of skin lesions in the spectrum of EM, Steven-Johnson syndrome, and toxic epidermal necrolysis in the absence of a definite inciting agent, undiagnosed lupus may indeed be present in some of these individuals and should be considered in the differential diagnosis. In conclusion, while it is very rare, SLE may present first with recurrent episodes of EM-like rash. Despite the various possibilities which underlie their association, prompt identification and treatment of SLE in patients presenting with EM is important to prevent death or irreversible organ damage."}, {"id": "pubmed23n0729_18106", "title": "Severe vitamin B12 deficiency in an exclusively breastfed 5-month-old Italian infant born to a mother receiving multivitamin supplementation during pregnancy.", "score": 0.009174311926605505, "content": "In infants, vitamin B12 deficiency may be due to an inborn error of absorption and metabolism, or nutritional problems. An exclusively breastfed 5-month-old Italian male infant, who was born after a normal full-term pregnancy to a vegan mother who was apparently daily treated with a multivitamin oral preparation during the second and third trimester, was hospitalised because of poor weight gain, feeding difficulties, severe pallor, muscle hypotonia and somnolence. Upon admission, his weight, length and head circumference were below the third percentile, he had an enlarged liver and spleen, and showed a significant delay in developmental milestones and communicative reactions. He had a hemoglobin level of 4.7 g/dL with an MCV of 84.2 fL, a white blood cell count of 4,680/mm3, and a platelet count of 45,000/mm3. His serum vitamin B12 level was 57 pg/mL (normal value 180-500 pg/mL) and serum folate level 12.8 ng/mL (normal value &gt;3 ng/mL). The results of metabolic examinations excluded a cobalamin C disorder, whereas nutritional screening showed a serum iron concentration of 9 μg/dL and serum ferritin of 4 ng/mL. Magnetic resonance imaging of the brain showed mild dilatation of the lateral ventricles with diffuse delayed myelination. The child was diagnosed as having vitamin B12 and iron deficiency due to nutritional inadequacy and was immediately treated with packed red blood cells, intramuscular vitamin B12 injections, and iron supplementation. A few days after the start of therapy, his hemoglobin levels and other hematological parameters rapidly improved, and a clinical improvement was observed within few weeks. There was an increase in his achievement of developmental milestones, but his development was still retarded seven months after the start of therapy. This case underlines the importance of adequately controlling maternal vitamin B12 intake during pregnancy by means of supplementation which, in the case of vegan mothers, should be significantly greater than that usually given. Moreover, the supplementation should be continued during lactation in order to avoid the development of signs of deficiency that may be associated with persistent neurological problems in infants. The case also highlights the need to consider vitamin B12 deficiency in infants with severe anemia even if their hematological parameters do not indicate megaloblastic anemia because the concomitant presence of substantial iron deficiency may modify the characteristics of the anemia."}, {"id": "pubmed23n0847_23036", "title": "Elderly female with Autoimmune hemolytic anemia.", "score": 0.009174311926605505, "content": "Autoimmune hemolytic anemia (AIHA) is a rare disease with an estimated prevalence of around 17/100,000. It is often difficult to diagnose and treat AIHA, especially in elderly. A 60-year-old female was admitted with the complaints of low grade fever, on-off for 6 months, progressive fatigue and dyspnea on exertion. She was transfused with three units of blood within these 6 months. Examination revealed pallor, edema, hemic murmur, and palpable liver. Hb was 2.9 gm%, T Bil 5.2 mg/dl, ESR 160 mm, and reticulocyte count 44.05%. Direct Coombs test was positive, anti-nuclear antibody (ANA) and Anti ds DNA were positive. A diagnosis of systemic lupus erythematosus (SLE) with AIHA was considered and patient was transfused with two units of packed red cells and put on steroid (prednisolone) at 1 mg/kg body weight daily. After 3 weeks, her Hb had increased to 10.4 gm% with gross clinical improvement. "}, {"id": "InternalMed_Harrison_8268", "title": "InternalMed_Harrison", "score": 0.009118156868409649, "content": "clinical features AIHA is a serious condition; without appropriate treatment, it may have a mortality of approximately 10%. The onset is often abrupt and can be dramatic. The hemoglobin level can drop, within days, to as low as 4 g/dL; the massive red cell removal will produce jaundice; and sometimes the spleen is enlarged. When this triad is present, the suspicion of AIHA must be high. When hemolysis is (in part) intravascular, the telltale sign will be hemoglobinuria, which the patient may report or about which we must enquire or test for. The diagnostic test for AIHA is the direct antiglobulin test developed in 1945 by R. R. A. Coombs and known since by this name. The beauty of this test is that it detects directly the pathogenetic mediator of the disease, i.e., the presence of antibody on the red cells themselves. When the test is positive, it clinches the diagnosis; when it is negative, the diagnosis is unlikely. However, the sensitivity of the Coombs test varies depending on the"}, {"id": "pubmed23n0334_77", "title": "A case of pancytopenia secondary to low-dose pulse methotrexate therapy in a patient with rheumatoid arthritis and renal insufficiency.", "score": 0.00909090909090909, "content": "Most reports on serious MTX toxicity have focused on hepatic abnormalities, while other effects, including hematologic reactions, have not been emphasized. We experienced a case of pancytopenia secondary to MTX therapy in a patient with RA and renal insufficiency. A 67-year-old woman with a 12-year history of active seropositive RA that was a response to non-steroidal anti-inflammatory drugs, hydroxychloroquinine and intra-articular steroid injections, had been followed up and was diagnosed as early chronic renal failure in October, 1993. Recently, because of significant morning stiffness and polyarthralgia, the decision was made to institute MTX treatment. This was begun as a single oral dose of 5mg/week. After 2 doses, the patient was admitted to the hospital with general weakness. Laboratory tests showed a hemoglobin level of 7.9 g/dl, WBC count 1800/mm3 and platelet count of 64000/mm3. The serum creatinine level was 6.1 mEq/dl and the BUN level was 82 mEq/dl. Liver function test results were normal, but the serum albumin level was 2.7 g/dl. The patient subsequently developed fever and blood transfusions, granulocyte colony stimulating factor (G-CSF) and intravenous prophylactic antibiotic therapy were required. Her condition was improved. In summary, Low-dose MTX-related adverse hematologic side effects, including fatal pancytopenia, are rare but are a cause of increasing concern in patients with RA and renal insufficiency. Close monitoring of associated risk factors, particularly impaired renal function, should be mandatory for all patients who are receiving MTX therapy."}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[759, 889]], "word_ranges": [[123, 141]], "text": "Suspecting giant cell arteritis, there are three analytical markers that are characteristically high: CRP, ESR and platelet count."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "This is a woman with abrupt, painless and severe loss (she only sees the movement of the hands) of vision in one eye. She does not give us much information from the ocular examination, only that there is an afferent pupillary defect. We have to rely on systemic symptoms to diagnose the problem. This is an elderly woman with typical symptoms of polymyalgia rheumatica, and the pain in the temporomandibular region is very suggestive. It is giant cell arteritis or temporal arteritis. The most common ocular manifestation is anterior ischemic optic neuropathy. In the fundus we would certainly see a papillary edema of that eye, with a gypsum white coloration. The afferent pupillary defect is very characteristic of these severe ischemic optic neuropathies. Suspecting giant cell arteritis, there are three analytical markers that are characteristically high: CRP, ESR and platelet count. We will first request one of these parameters and treat it often without waiting for biopsy confirmation. Therefore, option 2 is the correct one.", "full_answer_no_ref": "This is a woman with abrupt, painless and severe loss (she only sees the movement of the hands) of vision in one eye. She does not give us much information from the ocular examination, only that there is an afferent pupillary defect. We have to rely on systemic symptoms to diagnose the problem. This is an elderly woman with typical symptoms of polymyalgia rheumatica, and the pain in the temporomandibular region is very suggestive. It is giant cell arteritis or temporal arteritis. The most common ocular manifestation is anterior ischemic optic neuropathy. In the fundus we would certainly see a papillary edema of that eye, with a gypsum white coloration. The afferent pupillary defect is very characteristic of these severe ischemic optic neuropathies. Suspecting giant cell arteritis, there are three analytical markers that are characteristically high: CRP, ESR and platelet count. We will first request one of these parameters and treat it often without waiting for biopsy confirmation. Therefore, [HIDDEN].", "full_question": "A 70-year-old woman with a history of anorexia, weight loss, discomfort in the musculature and proximal joints plus pain in the temporomandibular region who comes to the emergency department for unilateral loss of vision (hand movement), sudden and painless onset (afferent pupillary defect). What test would you request first for diagnostic purposes?", "id": 404, "lang": "en", "options": {"1": "Lumbar puncture.", "2": "C-reactive protein.", "3": "Magnetic Resonance Angiography.", "4": "Carotid ultrasound.", "5": NaN}, "question_id_specific": 139, "type": "OPHTHALMOLOGY (ECTOPIC)", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0494_1430", "title": "Rapid, painless unilateral vision loss in a 37-year-old healthy woman.", "score": 0.01460697772173182, "content": "A 37-year-old woman experienced painless, progressive vision loss to no light perception in the left eye over the course of 3 days. The right eye was unaffected. On examination, the only other abnormal finding was a +4 left afferent pupillary defect. She was initially diagnosed with retrobulbar optic neuritis and admitted for treatment with intravenous methylprednisolone. Neuro-imaging revealed a large right anterior cerebral artery aneurysm that crossed the midline to compress the left optic nerve. The aneurysm was treated with coil embolization, which was technically successful but which did not lead to significant improvement in vision."}, {"id": "pubmed23n1105_22827", "title": "Sudden-onset unilateral painless vision loss.", "score": 0.013383838383838383, "content": "A 75-year-old Caucasian woman presented with sudden-onset multifocal scotomas in her right eye's central vision for 1 day. There were subtle white intraretinal foveal lesions that correlated with patchy inner retinal hyperreflectivity on optical coherence tomography, suggestive of paracentral acute middle maculopathy. Initial cerebrovascular work-up was negative. Review of systems was positive for lethargy and jaw claudication. The sedimentation rate and c-reactive protein were elevated, but platelet count was normal. The patient was started on 60 mg oral prednisone daily and underwent bilateral temporal artery that confirmed the diagnosis of giant cell arteritis."}, {"id": "InternalMed_Harrison_1431", "title": "InternalMed_Harrison", "score": 0.011568156583262323, "content": "A careful neurologic examination is an essential first step in the evaluation. In most cases, patients with an abnormal examination or a history of recent-onset headache should be evaluated by a computed tomography (CT) or magnetic resonance imaging (MRI) study. As an initial screening procedure for intracranial pathology in this setting, CT and MRI methods appear to be equally sensitive. In some circumstances, a lumbar puncture (LP) is also required, unless a benign etiology can be otherwise established. A general evaluation of acute headache might include cranial arteries by palpation; cervical spine by Pain induced by bending, lifting, cough Pain associated with local tenderness, e.g., region of temporal artery the effect of passive movement of the head and by imaging; the investigation of cardiovascular and renal status by blood pressure monitoring and urine examination; and eyes by funduscopy, intraocular pressure measurement, and refraction."}, {"id": "wiki20220301en564_9634", "title": "Acute visual loss", "score": 0.010900164403306083, "content": "Giant cell arteritis Giant cell arteritis should be considered in an older person with jaw claudication, temporal pain, and tiredness. Placing the person on steroids might save both their vision and decrease their risk of stroke. Without treatment a person can quickly go blind in both eyes. Vascular occlusions Central retinal artery occlusion: CRAO is characterized by painless, acute vision loss in one eye. Central retinal vein occlusion: CRVO causes sudden, painless vision loss that can be mild to severe. Branch retinal vein occlusion: sudden painless vision loss or visual field defect are the main symptom of BRVO. Branch retinal artery occlusion: BRAO may also cause acute painless loss of vision. Vitreous hemorrhage It is one of the most common causes of acute or subacute decrease in vision. Hyphema Blood in the anterior chamber of the eye is known as hyphema. Severe hyphema covering pupillary area can cause sudden decrease in vision. References Blindness"}, {"id": "wiki20220301en623_12323", "title": "Recurrent painful ophthalmoplegic neuropathy", "score": 0.00998256320836966, "content": "Magnetic resonance angiography (MRA) or CT angiography (CTA) can be used to examine cerebral blood vessels and to rule out vascular abnormalities, such as an aneurysm. In cases where intracranial vascular lesions, for example, subarachnoid hemorrhage cannot be completely ruled out after performing MRA or CTA, physicians may consider using traditional digital subtraction angiography (DSA) for more detailed investigation. Laboratory tests Lumbar punctures and blood tests might be performed on those with RPON to identify other possible causes of cranial neuropathy, including diabetes, inflammatory diseases, infections, tumors, and other systemic diseases that involve either the central nervous system or the peripheral nervous system. The detection of abnormalities in these tests suggests that RPON is highly unlikely to be the culprit in cranial neuropathy, and more diagnostic tests should be done to find out the underlying condition."}, {"id": "pubmed23n0810_19941", "title": "Atypical retinal vaso-occlusion with structural and functional resolution.", "score": 0.009900990099009901, "content": "The purpose is to report a patient with primary open-angle glaucoma that developed sudden painless unilateral vision loss, a sequential ophthalmoscopic appearance with features of both central retinal artery and later central retinal vein occlusion, and objective visual system dysfunction in the form of a relative afferent pupil defect, who spontaneously recovered vision along with complete resolution of the pupillary defect over several weeks. A 50-year-old woman with a long-standing history of glaucoma presented with acute, painless vision loss in one eye, a pallid retina with a cherry red macula, diffuse retinal hemorrhages, and a relative afferent pupil defect. Spectral domain optical coherence tomography and fluorescein angiography were essentially normal with neither retinal edema nor retinal ischemia to account for the visual dysfunction. Over the course of 2 months, the patient regained vision and the relative afferent pupil defect, typically a permanent manifestation of retinal destruction, resolved. Not all retinal vaso-occlusive phenomena can be completely attributed to a central retinal vein or artery occlusion. In the patient presented, there was no objective diagnostic testing that revealed a cause for the patient's vision loss or relative afferent pupillary defect. This combined with the complete recovery of vision and resolution of the relative afferent pupillary defect underscores a lack of comprehensive understanding of retinal vaso-occlusive disease."}, {"id": "pubmed23n0574_18362", "title": "[Temporal arteritis presenting with headache and abducens nerve palsy. Report of a case].", "score": 0.009900990099009901, "content": "A 71-year-old man visited our clinic with a 3-day history of severe throbbing headache and 1-day history of horizontal diplopia. He had had jaw claudication and pain in the neck and shoulder several days previously. His right eye was slightly esotropic and did not move laterally. There was no blepharoptosis, proptosis, lid edema, or conjunctival injection. The pupils were unremarkable. The remainder of the cranial nerve functions was intact. There was no limb weakness or sensory impairment. Superficial temporal arteries were swollen and tender on both sides. Laboratory examination showed elevated CRP level and high erythrocyte sedimentation rate. Cranial MR images were unremarkable. The cerebrospinal fluid was acellular with 45 mg/dl of protein. A diagnosis of temporal arteritis was made. Treatment with 50 mg of prednisolone brought about prompt disappearance of the headache. Right ocular movement fully recovered in 10 days. Temporal artery biopsy findings and response to corticosteroid were consistent with temporal arteritis. The motility pattern of the right eye was consistent with complete abducens nerve palsy, which is a rare manifestation of temporal arteritis. Although temporal arteritis is a rare cause of ophthalmoplegia in the elderly patients, swift diagnosis and treatment is necessary to avoid blindness."}, {"id": "wiki20220301en003_97415", "title": "Headache", "score": 0.009832509940956743, "content": "One recommended diagnostic approach is as follows. If any urgent red flags are present such as visual loss, new seizures, new weakness, new confusion, further workup with imaging and possibly a lumbar puncture should be done (see red flags section for more details). If the headache is sudden onset (thunderclap headache), a computed tomography test to look for a brain bleed (subarachnoid hemorrhage) should be done. If the CT scan does not show a bleed, a lumbar puncture should be done to look for blood in the CSF, as the CT scan can be falsely negative and subarachnoid hemorrhages can be fatal. If there are signs of infection such as fever, rash, or stiff neck, a lumbar puncture to look for meningitis should be considered. If there is jaw claudication and scalp tenderness in an older person, a temporal artery biopsy to look for temporal arteritis should be performed and immediate treatment should be started. Neuroimaging"}, {"id": "pubmed23n1121_13718", "title": "Atypical Leber hereditary optic neuropathy with a 34-year interval between vision loss in both eyes.", "score": 0.00980392156862745, "content": "Leber hereditary optic neuropathy (LHON) is an inherited mitochondrial disease characterized by painless vision loss affecting both eyes. The disease usually develops in both eyes within weeks to months of onset. We report a case of LHON who presented with unilateral vision loss in childhood with an interval of more than 30 years between vision loss in the two eyes. A 43-year-old man presented with a 1-month history of vision loss in his right eye. At 9 years of age, his visual acuity in the left eye declined, and he had been treated with glaucoma eyedrops bilaterally at his eye clinic. At his first visit to our hospital, his BCVA was 0.15 in the right eye and 0.1 in the left eye, and critical flicker frequency was 16 Hz in the right eye and 15 Hz in the left eye, and he was negative for a relative afferent pupillary defect. The Goldman visual field showed central scotoma in both eyes. Fundus examination revealed slight redness of the right optic disc with meandering retinal small vessels, and the left optic disc had a slight pallor. Fluorescein angiography could not be performed because of liver dysfunction. OCT showed prominent bilateral thinning of the RNFL and retinal ganglion cell layer. Enhancement of the optic nerve was not apparent on orbital gadolinium-enhanced magnetic resonance imaging. Hematologic analysis revealed macrocytic anemia and low levels of vitamin B12 and folate. His mother had a presumptive diagnosis of LHON but did not receive genetic testing. A male cousin also had severe vision loss. Based on the likely family history of LHON, we performed genetic testing, which revealed the 11778 mitochondrial point mutation associated with this condition. We report a case of LHON with 34 years interval in vision loss in the fellow eye. LHON may develop in the second eye decades after its onset in the first. Detailed medical interviews and scrutiny, such as examination of family history, are warranted in consideration of LHON."}, {"id": "First_Aid_Step2_614", "title": "First_Aid_Step2", "score": 0.00980392156862745, "content": "If a 20-year-old female develops headaches after drinking red wine, think migraine. Associated symptoms/signs: Significant findings include fever or rash (consider meningitis or other infectious causes), jaw claudication (specific for temporal arteritis), or constitutional symptoms such as weight loss (associated with neoplastic, inflammatory, or infectious conditions). Photophobia, nausea, and vomiting are associated with migraine, aneurysmal SAH, and meningitis, but neck stiffness is more likely to accompany the latter two. Neurologic sequelae: Look for diplopia, mental status changes or associated symptoms (numbness, weakness, dizziness, ataxia, visual disturbances), papilledema, or pupillary abnormalities (partial CN III palsy or Horner’s syndrome). Patient risk factors: High-risk patients are > 50 years of age, immunocompromised, or with preexisting malignancy. If SAH is suspected, obtain a head CT without contrast. If CT is , LP is mandatory. Obtain a CBC."}, {"id": "pubmed23n0995_14786", "title": "Ultrasound-Assisted Diagnosis of Optic Neuritis in the Emergency Department: A Case Report.", "score": 0.009708737864077669, "content": "Optic neuritis is a common cause of subacute unilateral vision loss, occurring in 1-5 per 100,000 persons per year. It is more common in Caucasians, women, and those from countries with northern latitudes. Those aged 20-49 years are at greatest risk. The condition arises due to inflammation of the optic nerve. Inflammation may occur due to systemic inflammatory disorders, most commonly multiple sclerosis. A 21-year-old African-American male presented to our emergency department with a complaint of painful unilateral vision loss. On examination he was found to have a relative afferent pupillary defect and red desaturation. A bedside ultrasound suggested pseudopapilledema suggestive of optic neuritis. He was admitted to Neurology for confirmation of and treatment for optic neuritis. Magnetic resonance imaging confirmed optic neuritis. The patient was treated with i.v. steroids and discharged after improvement in visual function. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Optic neuritis is a clinical diagnosis. The subtle historical components and examination findings make it a diagnostic challenge for the busy emergency physician. Early diagnosis may improve visual outcomes. Discovery of pseudopapilledema on bedside ultrasound may be seen in optic neuritis, and is another finding that emergency physicians may assess for in patient presenting with unilateral vision loss."}, {"id": "pubmed23n0314_1466", "title": "[A 62-year-old man with an acute onset of consciousness disturbances].", "score": 0.009708737864077669, "content": "We report a 62-year-old man who developed coma and died in a fulminant course. The patient was well until May 1, 1996 when he noted chillness, tenderness in his shoulders, and he went to bed without having his lunch and dinner. In the early morning of May 2, his families found him unresponsive and snoring; he was brought into the ER of our hospital. He had histories of hypertension, gout, and hyperlipidemia since 42 years of the age. On admission, his blood pressure was 120/70, heart rate 102 and regular, and body temperature 36.3 degrees C. His respiration was regular and he was not cyanotic. Low pitch rhonchi was heard in his right lower lung field. Otherwise general physical examination was unremarkable. Neurologic examination revealed that he was somnolent and he was only able to respond to simple questions such as opening eyes and grasping the examiner's hand, but he was unable to respond verbally. The optic discs were flat; the right pupil was slightly larger than the left, but both reacted to light. He showed ptosis on the left side, conjugate deviation of eyes to the left, and right facial paresis. The oculocephalic response and the corneal reflex were present. His right extremities were paralyzed and did not respond to pain Deep tendon reflexes were exaggerated on the right side and the plantar response was extensor on the right. No meningeal signs were present. Laboratory examination revealed the following abnormalities; WBC 18,400/ml, GOT 131 IU/l GPT 50 IU/l, CK616 IU/l, BUN 30 mg/dl, Cr 2.1 mg/ dl, glucose 339 mg/dl, and CRP 27.4 mg/dl. ECG showed sinus tachycardia and ST elevation in II, III and a VF leads and abnormal q waves in I, V5, and V6 leads. Chest X-ray revealed cardiac enlargement but the lung fields were clear. Cranial CT scan revealed low density areas in the left middle cerebral and left posterior cerebral artery territories. The patient was treated with intravenous glycerol infusion and other supportive measures. At 2: 10 AM on May 3, he developed sudden hypotension and cardiopulmonary arrest. He was pronounced dead at 3:45 AM. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that the patient had acute myocardial infarction involving the inferior and the true posterior walls and left internal carotid embolism from a mural thrombus. Post mortem examination revealed occlusion of the circumflex branch of the left coronary artery due to atherom plaque rupture and myocardial infarction involving the posterior and the lateral wall with a rupture in the postero-lateral wall. Marked atheromatous changes were seen in the left internal carotid, the middle cerebral and the basilar arteries; the left internal carotid and the middle cerebral arteries were almost occluded by thrombi and blood coagulate. The territories of the left middle cerebral and the occipital arteries were infarcted; but the left thalamic area was spared. The neuropathologist concluded that the infarction was thrombotic origin not an embolic one as the atherosclerotic changes were severe. Cardiac rupture appeared to be the cause of terminal sudden hypotension and cardiopulmonary arrest. It appears likely that a vegetation which had been attached to the aortic valve induced thromboembolic occlusion of the left internal carotid artery which had already been markedly sclerotic by atherosclerosis. It is also possible that the vegetations in the aortic valve came from mural thrombi at the site of acute myocardial infarction, as no bacteria were found in those vegetations."}, {"id": "pubmed23n1145_24843", "title": "Paracentral Acute Middle Maculopathy After COVID-19 Disease: Multimodal Evaluation.", "score": 0.009615384615384616, "content": "To report the case and multimodal imaging findings of a healthy young woman who developed paracentral acute middle maculopathy (PAMM) 9 weeks after COVID-19 disease. Case report. Ultra-widefield fundus photography, macular spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography (FA), and OCT-angiography (OCT-A) were performed. A 36-year-old woman who developed PAMM 9 weeks after SARS-CoV-2 infection. A 36-year-old woman went to the emergency department (ED) with sudden, painless, left eye (LE) vision loss. The only relevant past medical history was COVID-19 disease 9 weeks before. Best corrected visual acuity (BCVA) was 20/200, a LE relative afferent pupillary defect (RAPD) was present and superficial hemorrhages throughout the macular area and peripheral retina were found. Nearly four hours after admission, LE BCVA recovered to 20/20 without RAPD. Five days after presentation in the ED, the patient returned with recurrent LE vision loss, with spontaneous recovery within 12 hours. Macular SD-OCT revealed hyperreflectivity of the inner plexiform and inner nuclear layers and the diagnosis of PAMM was established. The patient started oral acetylsalicylic acid and oral prednisolone. The patient did not report any new episodes of vision loss and there was a progressive resolution of abnormal fundus findings. SARS-CoV-2 infection increases the risk of vascular thrombotic events with possible involvement of the retinal circulation, and PAMM may present as a possible complication. Ophthalmologists should be able to recognize it promptly through multimodal imaging findings."}, {"id": "pubmed23n0596_4516", "title": "Anterior ischemic optic neuropathy due to giant cell arteritis with normal inflammatory markers.", "score": 0.009615384615384616, "content": "In anterior ischemic optic neuropathy (AION), it is important not to miss the diagnosis of giant cell arteritis (GCA) because this requires immediate steroid treatment to prevent involvement of the second eye and possible blindness. A missed diagnosis also might lead to fatal systemic complications. Observational case report. A 79-year-old woman noticed decreased visual and visual field loss in the right eye. At presentation, right visual acuity was 10/20 (ETDRS chart 2000). There was a right relative afferent pupillary defect of 0.6 log units. Asked for symptoms of GCA she complained about temporal and occipital headache, jaw claudication combined with malaise, and myalgia of the upper limbs. Laboratory tests showed normal inflammatory markers. Repeated tests confirmed ESR and CRP to be within the normal range. GCA being suspected, ultrasound of the superficial temporal arteries and temporal artery biopsy were performed unilaterally on the right side. Histology showed a chronic inflammatory cell infiltrate consistent with active GCA. The patient was treated with high-dose corticosteroids (250 mg methylprednisolone, three times/day, initially) and symptoms rapidly resolved, but visual loss remained unchanged. The case presented here proves that GCA with typical related visual loss (AION) is possible even when both ESR and CRP are in the normal range. Therefore, in the presence of typical symptoms, the clinician must not rely solely on laboratory testing, but start steroid therapy immediately and order a temporal artery biopsy."}, {"id": "wiki20220301en015_138436", "title": "Sciatica", "score": 0.009523809523809525, "content": "Cancer should be suspected if there is previous history of it, unexplained weight loss, or unremitting pain. Spinal epidural abscess is more common among those with diabetes mellitus or immunocompromised or who had spinal surgery, injection or catheter; it typically causes fever, leukocytosis and increased erythrocyte sedimentation rate. If cancer or spinal epidural abscess are suspected, urgent magnetic resonance imaging is recommended for confirmation. Proximal diabetic neuropathy typically affects middle aged and older people with well-controlled type-2 diabetes mellitus; onset is sudden causing pain usually in multiple dermatomes quickly followed by weakness. Diagnosis typically involves electromyography and lumbar puncture. Shingles is more common among the elderly and immunocompromised; usually (but not always) pain is followed by appearance of a rash with small blisters along a single dermatome. Acute Lyme radiculopathy may follow a history of outdoor activities during warmer"}, {"id": "pubmed23n1025_22054", "title": "Lessons of the month 4: Giant cell arteritis with normal inflammatory markers and isolated oculomotor nerve palsy.", "score": 0.009523809523809525, "content": "Giant cell arteritis (GCA) is an important condition to suspect and treat early, as failure to do so can result in anterior ischaemic optic neuropathy and subsequent permanent visual loss.A 71-year-old woman presented to her local emergency department with a 1-week history of constant, moderate-severe global headache associated with intermittent periorbital pain. Two weeks later she developed sudden horizontal diplopia. Examination demonstrated right oculomotor nerve palsy. Her erythrocyte sedimentation rate (ESR) was 9 mm/hr. Repeat blood tests 1 month later showed an ESR of 67 mm/hr. Temporal artery biopsy was positive.A review from a cohort of 764 patients with suspected GCA who underwent biopsy found the sensitivity of an elevated ESR and c-reactive protein was 84% and 86%, respectively, but the specificity was only 30%. Therefore, inflammatory markers should only act as a guide, and caution should be taken in their interpretation especially with respect to the time of sampling in the disease evolution.Isolated oculomotor nerve palsy in association with GCA is rare. The first case series was described by miller fisher in 1959 who observed two patients presenting with diplopia, ptosis and ocular palsies. In anyone over the age of 50 who develops a new, refractory headache and cranial neuropathy, GCA should be the first consideration."}, {"id": "pubmed23n1037_7823", "title": "A tearfully painful darkness.", "score": 0.009433962264150943, "content": "A 70-year-old woman presented with new onset of left eye and facial pain. Ophthalmic and neurological examinations, magnetic resonance imaging brain, erythrocyte sedimentation rate, and C-reactive protein were unrevealing. A few days later, she developed vision loss in her left eye. Examination revealed decreased visual acuity with a relative afferent pupillary defect in the left eye and a diffuse mild swelling of the left optic nerve head. Repeat magnetic resonance imaging showed T2 hyperintensity and enhancement of the intraorbital optic nerve and surrounding tissues with no other intracranial abnormalities. Serum studies showed elevated myelin oligodendrocyte glycoprotein IgG titer. She was treated with IV methylprednisolone 1000 mg daily for 3 days and was discharged on prolonged prednisone taper with return of vision to baseline."}, {"id": "pubmed23n0334_10172", "title": "Lessons to be learned: a case study approach--a case of temporal arteritis.", "score": 0.009433962264150943, "content": "A 71-year-old male presented with a history of sudden partial visual loss in the right eye with an inferior visual field defect over the past 3-4 days. He had no history of headache or of facial pain. Clinical examination confirmed that vision on the right side was reduced to 6/18 and on the left to 6/12. The right eye showed a relative afferent pupillary defect. There was no other abnormality of the anterior segment of either eye. The right retina showed a pale swollen optic disc and a provisional diagnosis of anterior ischaemic optic neuropathy (AION) was made. An urgent erythrocyte sedimentation rate (ESR) was ordered and the patient was asked to return to the eye clinic in one month. However, 16 days later--when it was first recognised that his ESR was elevated to 75 mm in the first hour--the patient was recalled immediately in order to commence systemic steroid treatment; but regrettably, by this time, his right eye had become totally blind. In this case, although the attending doctor made a correct clinical diagnosis on presentation, he failed to act upon the result of the blood test."}, {"id": "pubmed23n1076_12839", "title": "Childhood-Onset Leber Hereditary Optic Neuropathy: Particular Features.", "score": 0.009345794392523364, "content": "Leber hereditary optic neuropathy (LHON) is an optic neuropathy of mitochondrial inheritance. Childhood-onset disease is relatively rare and there are limited data on this important patient subgroup. We present 3 particular presentations of LHON. Patient 1 was an 8-year-old boy admitted to the emergency department reporting a progressive bilateral visual loss and intermittent headaches. Neuro-ophthalmological examination revealed a bilateral pseudopapilledema. Lumbar puncture identified intracranial hypertension and the brain and orbits magnetic resonance imaging showed T2 hyperintensity in the posterior region of the left optic nerve and the optic chiasm. Patient 2 was a 12-year-old boy admitted to the emergency department reporting painless, progressive central vision loss in the right eye. Fundus examination revealed a hyperemic disc and vascular network papillary and peripapillary vascular microdilations. Three months later, the left eye presented visual loss. Patient 3 was a 6-year-old female child referred to the neuro-ophthalmology specialist due to painless central visual loss in both eyes. Her BCVA was 1/10 and counting fingers in right and left eye, respectively, and fundus examination revealed a pallor optic disc in the temporal sector. The phenotype of childhood-onset disease may present itself distinct from classical adult-onset LHON. The absence of classical clinical features could lead to initial misdiagnosis. There should exist a high index of suspicion in children presenting unexplained subnormal vision in order to avoid potential diagnostic delays."}, {"id": "pubmed23n0321_12634", "title": "Masticatory muscle pain: an important indicator of giant cell arteritis.", "score": 0.009345794392523364, "content": "Giant cell arteritis (GCA) is a polysymptomatic disease which constitutes an ophthalmic emergency because early recognition and management can prevent blindness. There is conflicting information in the literature on the validity, sensitivity, and specificity of various systemic symptoms and signs of GCA. This paper presents a review of our prospective studies on the subject, and our findings are particularly relevant to dentists. We investigated 363 patients in a prospective study. Positive temporal artery biopsy was seen in 106 patients and negative in 257 referred for diagnosis of GCA. Systemic symptoms and signs of GCA and erythrocyte sedimentation rate (Westergren-ESR) and C-reactive protein (CRP) levels were compared in these two groups of patients. The odds of having a positive temporal artery biopsy (i.e., GCA) were 9.1 times greater with jaw claudication (pain in masticatory muscles on eating), 3.4 times with neck pain, 3.2 times with CRP &gt; 2.45 mg/dL, 2.0 times with ESR 47.107 mm/hr, 2.7 times with ESR &gt; 107 mm/hr, and 2.0 times when the patients were aged &gt; or = 75 years. Other signs and symptoms did not show a significant association with a positive biopsy. Our study showed that \"normal\" ESR values do not rule out GCA but that CRP is a more useful test than ESR. Since jaw claudication is one of the most important symptoms of GCA, dentists should keep this possibility in mind when older patients come complaining of jaw pain while eating."}, {"id": "wiki20220301en053_41040", "title": "Posterior ischemic optic neuropathy", "score": 0.009259259259259259, "content": "Defective light perception in one eye causes an asymmetrical pupillary constriction reflex called the afferent pupillary defect (APD). Arteritic PION A-PION most commonly affects Caucasian women, with an average age of 73. At onset vision loss is unilateral, but without treatment it rapidly progresses to involve both eyes. Vision loss is usually severe, ranging from counting fingers to no light perception. Associated symptoms are jaw pain exacerbated by chewing, scalp tenderness, shoulder and hip pain, headache and fatigue. Perioperative PION Vision loss is usually apparent upon waking from general anesthesia. Signs observable to a bystander include long surgery duration and facial swelling. Vision loss is usually bilateral and severe, ranging from counting fingers to no light perception. Cause"}, {"id": "pubmed23n0524_20048", "title": "[Transient trochlear nerve palsy as the presenting neurological sign of panarteritis nodosa].", "score": 0.009259259259259259, "content": "Panarteritis nodosa (PAN) is a systemic vasculitis affecting small and medium-sized arteries. Neuro-ophthalmological complications of PAN are rare but numerous, and may affect the eye, the visual and the oculomotor pathways. Such complications occur mainly in patients previously diagnosed with PAN. A 51-year-old woman presented with an isolated right trochlear (IV) palsy, in the setting of headaches and fluctuating fever of unknown etiology. Erythrocyte sedimentation rate was 13 mm and full blood cell count was normal. Previous chest X-ray and blood studies were negative for an infection or inflammation. Orbital and cerebral CT scan was normal. Spontaneous recovery of diplopia ensued over four days. Two days later, paresthesia and sensory paresis of the dorsal portion of the left foot were present. Lumbar puncture revealed 14 leucocytes (76 percent lymphocytes) with elevated proteins, but blood studies and serologies were negative. A diagnosis of undetermined meningo-myelo-radiculoneuritis was made. Because of a possible tick bite six weeks previously the patient was empirically treated with 2 g intravenous ceftriaxone for 3 weeks. Fever rapidly dropped. Six weeks after the onset of diplopia, acute onset of blindness in her right eye, diffuse arthralgias and fever motivated a new hospitalization. There was a central retinal artery occlusion of the right eye. Blood studies now revealed signs of systemic inflammation (ESR 30 mm, CRP 12 mg/L, ANA 1/80, pANCA 1/40, leucocytosis 12.4 G/L, Hb 111 g/L, Ht 33 percent). Biopsy of the left sural nerve revealed arterial fibrinoid necrosis. A diagnosis of PAN was made. Transient diplopia can be the heralding symptom of a systemic vasculitis such as PAN, giant cell arteritis and Wegener granulomatosis. In this patient the presence of accompanying systemic symptoms raised a suspicion of systemic inflammation, but the absence of serologic and imaging abnormalities precluded a specific diagnosis initially. A few weeks later, the presence of a second ischemic event (retinal) and positive blood studies led to a further diagnostic procedure. Oculomotor and abducens palsies have rarely been reported in association with PAN. We report the first case of trochlear nerve paresis as the inaugural neurological sign of PAN. This case highlights the importance of considering inflammatory systemic disorders in patients with acute diplopia particularly when they are young, lack vascular risk factors or cause, and complain of associated systemic symptoms."}, {"id": "wiki20220301en019_114604", "title": "Ankylosing spondylitis", "score": 0.009174311926605505, "content": "These diagnostic criteria include: Inflammatory back pain:Chronic, inflammatory back pain is defined when at least four out of five of the following parameters are present: (1) Age of onset below 40 years old, (2) insidious onset, (3) improvement with exercise, (4) no improvement with rest, and (5) pain at night (with improvement upon getting up) Past history of inflammation in the joints, heels, or tendon-bone attachments Family history for axial spondyloarthritis or other associated rheumatic/autoimmune conditions Positive for the biomarker HLA-B27 Good response to treatment with nonsteroidal anti-inflammatory drugs (NSAIDs) Signs of elevated inflammation (C-reactive protein and erythrocyte sedimentation rate) Manifestation of psoriasis, inflammatory bowel disease, or inflammation of the eye (uveitis) If these criteria still do not give a compelling diagnosis magnetic resonance imaging (MRI) may be useful. MRI can show inflammation of the sacroiliac joint. Imaging"}, {"id": "pubmed23n0525_4392", "title": "[Blindness in both eyes due to late diagnosis of giant cell arteritis].", "score": 0.009174311926605505, "content": "Giant cell arteritis (GCA) is often diagnosed very late, variable \"facets\" of the disease exist render the diagnosis more difficult. Follow-up observations of five very old patients are reported in whom diagnosis was made too late, resulting in blindness of both eyes. Five patients (age 76-84 years, 4 women, one man) with GCA became blind in both eyes because diagnosis had been delayed (two patients) or onset of therapy was too late (three patients). In two patients who also had arterial hypertension, the symptom \"headache\" had been misleading. Symptoms of accompanying general diseases masked the real diagnosis, particularly in the second patient who had renal insufficiency, coronary artery disease, and unilateral obstruction of the internal carotid artery. Symptoms that failed to lead to the correct diagnosis were: muscle or chewing pain (three patients), circumscribed numbness around the mouth (second patient), and persistent headache despite normalization of blood pressure. Normal findings from cranial CTAs (two patients) led to the wrong reassurance of the patient. Swelling of the optic disk (two patients) was misdiagnosed by ophthalmologists, as was a retinal branch arterial occlusion (first patient). Three patients, afraid of possible side effects caused by glucocorticoids, took ineffective alternative medications. Poor vigilance led to blindness of the fifth patient with long-standing polymyalgia rheumatica. Targeted examinations at the onset of symptoms are necessary. GCA-symptoms were mis-constructed by additional diseases that disguised the correct diagnosis. The danger of bilateral blindness is particularly great in patients of great age."}, {"id": "pubmed23n1033_13514", "title": "Leber's hereditary optic neuropathy following unilateral painful optic neuritis: a case report.", "score": 0.00909090909090909, "content": "Leber's hereditary optic neuropathy (LHON) is a maternally inherited mitochondrial disease, characterized by acute or subacute, painless, bilateral visual loss. LHON is often misdiagnosed as optic neuritis at an early stage because of the similarity of their clinical presentation. To date, there has been no reported case of actual optic neuritis and LHON in one patient. A 40-year-old, healthy man was referred to our clinic with acute painful visual loss in the right eye for 2 weeks. In the right eye, visual acuity decreased to 20/40, and the Ishihara colour test score was 8/14 with a relative afferent pupillary defect. Optic disc swelling was found only in the right eye, and magnetic resonance imaging revealed enhancement of the the right optic nerve, consistent with optic neuritis. After receiving 1 g of intravenous methylprednisolone daily for three days, his ocular pain resolved, and visual acuity improved to 20/20 within 2 weeks. Seven months later, the patient developed acute painless visual loss in the right eye. Visual acuity decreased to 20/200 in the right eye. There was no response to the intravenous methylprednisolone therapy at that time. Eight months later, he developed subacute painless visual loss in the left eye. Genetic testing for LHON was performed and revealed the pathologic mtDNA 11778 point mutation. We report a case with painful unilateral optic neuritis preceding the onset of LHON. Even if a typical optic neuritis patient has completely recovered from steroid treatment once in the past, it is advisable to keep in mind the possibility of LHON if acute or subacute loss of vision subsequently or simultaneously occurs in both eyes and does not respond to steroids."}, {"id": "pubmed23n1148_299", "title": "An Unusual Case of Giant Cell Arteritis.", "score": 0.00909090909090909, "content": "Giant cell arteritis (GCA), also known as temporal arteritis (TA), is a systemic autoimmune inflammation of medium and large arteries. It is the most common vasculitis affecting adults older than 50, with an incidence of 20/100,000 and an average age of onset of 70. Typically, patients initially present with new-onset headaches, visual changes and disturbances, jaw claudication, arthralgias, and tender or swollen temporal or occipital arteries. Our patient is a 73-year-old male who presented to the emergency room with 10 days of bilateral headache radiating to the occipital area associated with fevers, persistent chills, generalized weakness, and a headache described as constant, dull, 9 out of 10 pain, and minor pain with neck flexion. Lab work revealed an elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). The patient had tender palpation to his temples and due to a high suspicion of giant cell arteritis, he was started on high-dose steroids with rapid relief of his symptoms. Biopsy showed evidence of active non-granulomatous vasculitis and confirmed bilateral temporal arteritis within the context of the clinical setting. GCA patients are more likely to be women and typically present with unilateral headache (66% of GCA), jaw claudication (50%), fevers (50%), and transient visual loss (16-54%). Here, we describe a 73-year-old male with a past medical history of cerebral vascular accident (CVA), diabetes, and cancer that presented with 10 days of bilateral headaches and fevers. Unlike the usual presentation, our patient denied any vision and joint pain changes, and the temporal arteries were not stiff to palpation. This patient presentation is unique to previous reports in the limited display of symptoms and absence of the most commonly associated manifestations. Although his presentation supported GCA, the features of elevated ESR and CRP, headache, and fever were too general to diagnose GCA exclusively, and his additional symptoms of rhinorrhea and sinus pain more likely supported infection. Our case indicates the importance of maintaining a high index of clinical suspicion for GCA in the elderly population presenting with headaches and elevated ESR and CRP. GCA, also known as temporal arteritis (TA), is a systemic autoimmune inflammation of medium and large arteries. Typically, patients initially present with new-onset headaches, visual changes and disturbances, jaw claudication, arthralgias, and tender or swollen temporal or occipital arteries. Diagnosis requires high clinical suspicion, and treatment revolves around high doses of steroids."}, {"id": "pubmed23n0536_7105", "title": "Three presentations of monocular vision loss.", "score": 0.009009009009009009, "content": "Carotid artery disease is estimated to affect 30% of persons older than 50. Risk factors include hypertension, cigarette smoking, hyperlipidemia and diabetes mellitus. Symptoms ascribed to carotid artery lesions with stenosis of the artery or plaque formation include monocular vision loss and transient ischemic attacks. Patients can present with transient monocular vision loss as their initial symptom. Three patients from a geriatric clinic in Wilmington, Delaware presented with different complaints of vision loss with similar overall outcomes. Patient A was an 87-year-old woman who presented with dimming of vision on extreme left head turn. Dilated fundus examination found a retinal arterial emboli in the left eye (O.S.). Carotid duplex examination found 50% to 79% left internal carotid stenosis with no hemodynamic stenosis of the right internal carotid. Patient B was a 78-year-old woman who presented with a right superior altitudinal defect and transient vision loss in the right eye. Dilated fundus examination found retinal arterial emboli in the right eye (O.D.). Carotid duplex examination found 50% to 79% carotid stenosis in both the left and right internal carotids. Patient C was an 84-year-old man who complained of a superior altitudinal visual field defect O.D. Dilated fundus examination found a retinal arterial emboli O.D. Carotid duplex results showed calcified atherosclerotic plaques present at the level of the common carotid artery bifurcations bilaterally, with 50% to 70% narrowing of the right internal carotid artery with no significant narrowing of the left internal carotid artery. These 3 presentations show that in patients older than 50 who present with chief complaints of monocular vision loss, a differential diagnosis of carotid artery disease must be considered. Patients who exhibit retinal arterial emboli are at increased risk for stroke and vascular death. Appropriate measures for confirming a diagnosis include duplex ultrasound imaging, magnetic resonance angiography (MRA), and carotid angiography. Surgical techniques such as carotid angioplasty and carotid endarterectomy may be recommended."}, {"id": "pubmed23n0019_2125", "title": "[Arteriitis temporalis--a major disease developing in advance age (author's transl)].", "score": 0.009009009009009009, "content": "Arteriitis temporalis, a disease that was largely unknown in the early fifties, has gained importance in recent years. In the past ten years 32 patients suffering from arteriitis temporalis were diagnosed and treated both in the eye hospital and in the rheumatic-cardiological hospital of the Berlin-Buch Municipal Hospital. The following clinical findings were essential for the diagnosis: advanced or old age of the patient, massive headache in the temporal and/or occipital regions, myalgia primarily in the shoulders and the neck that responded relatively poorly to treatment, reduced eyesight, loss of weight and decrease in vitality. Extremely high BSR, an increased amount of alpha-2 and an increased level of alkaline phosphatase were most important among the laboratory findings. Corticosteroids have proved to be the medicine of choice for treating this condition."}, {"id": "pubmed23n1030_6059", "title": "Central retinal artery occlusion as initial presentation of Moyamoya disease in a middle-aged woman.", "score": 0.008928571428571428, "content": "To present a case of central retinal artery occlusion as the first symptomatic manifestation of Moyamoya disease in a middle-aged patient. Case report of a 48-year-old female Chinese-American patient who presented with sudden onset painless unilateral vision loss. Fundus photos, optical coherence tomography, fluorescein angiography, magnetic resonance angiography, computed tomography angiography, and catheter cerebral angiogram were performed. The patient's dilated fundus examination showed classic findings of a central retinal artery occlusion. Diagnostic brain imaging demonstrated extensive stenosis of the cerebrovascular network, with almost complete unilateral occlusion of the internal carotid artery along with compensatory collateral vessels. This led to a new diagnosis of Moyamoya disease. The patient was treated with extracranial-intracranial bypass surgery. Arterial abnormalities in patients with Moyamoya disease are uncommon and have previously only been reported in younger patients in their teens and 20s. Young and middle-aged patients presenting with central retinal artery occlusions should undergo complete neurologic workup including stroke evaluation; in this case, revealing Moyamoya disease, a rare yet life-threatening condition, as the underlying etiology."}, {"id": "First_Aid_Step2_571", "title": "First_Aid_Step2", "score": 0.008928571428571428, "content": "Also called giant cell arteritis; due to subacute granulomatous infl ammation of the large vessels, including the aorta, external carotid (especially the temporal branch), and vertebral arteries. The most feared manifestation is blindness 2° to occlusion of the central retinal artery (a branch of the internal carotid artery). Risk factors include polymyalgia rheumatica (affects almost half of TA patients), age > 50, and female gender. Presents with new headache (unilateral or bilateral); scalp pain and temporal tenderness; and jaw claudication. Fever, permanent monocular blindness, weight loss, and myalgias/arthralgias (especially of the shoulders and hips) are also seen. ESR > 50 (usually > 100). Ophthalmologic evaluation. Temporal artery biopsy: Look for thrombosis; necrosis of the media; and lymphocytes, plasma cells, and giant cells."}, {"id": "pubmed23n1143_24145", "title": "Isolated Infiltrative Optic Neuropathy in an Acute Lymphoblastic Leukemia Relapse.", "score": 0.008849557522123894, "content": "Optic nerve infiltration as the first sign of isolated central nervous system relapse of acute lymphoblastic leukemia (ALL) is rare. A seven-year-old girl with standard-risk B-cell ALL who was in remission presented with sudden onset of left eye pain and loss of vision. Examination revealed no perception to light in the left eye with positive relative afferent pupillary defect. The optic disc was hyperemic and swollen with total obscuration of the disc margin associated with central retinal artery and vein occlusion. Magnetic resonance imaging of the brain and optic nerve showed left intraorbital optic nerve thickening associated with perineural enhancement and intraconal fat involvement. Lumbar puncture revealed leukemic infiltration with blast cells after a week of eye symptoms, while bone marrow aspiration was negative for malignant cells. A diagnosis of left leukemic optic nerve infiltration with central retinal artery and vein occlusion was made. A high index of suspicion with repeat cerebrospinal fluid sampling is crucial to confirm the diagnosis as vitreous biopsy may fail to reveal infiltrative cells."}, {"id": "pubmed23n0244_2773", "title": "[The Tolosa-Hunt syndrome: report of a case with recurrent (9 times) painful ophthalmoplegia (author's transl)].", "score": 0.008849557522123894, "content": "A 48-year-old woman was referred to the First Dept. of Int. Med., Nagasaki Univ. Sch. Med., in August, 1979, with a six-month history of recurrent episodes of right-sided painful ophthalmoplegia and diplopia. An epidode affected the right eye, lasted one to two weeks, and relapsed every month. On examination she had a complete ptosis on the right side and pain on the right eye. All extraocular muscle supplied by the 3rd nerve were paralysed. The pupils were equal in size both sides, reacting to light completely. Visual acuity was normal except myopia. All the other cranial nerves and the remainder of central nervous system was normal. Results of thyroid function tests and of lumbar puncture were normal. The glucose tolerance test showed a mild diabetic pattern. Blood and CSF cultures for bacteria, fungi, and acid-fast bacillus were negative. The skull, brain CT scan, and carotid angiogram were within normal limits. A tentative diagnosis of Tolosa-Hunt syndrome was made after an unproductive search for a cause for this woman's painful ophthalmoplegia and unsuccessful treatment of ophthalmoplegia with antibiotics or diet therapy for mild hyperglycemia. The patient was given prednisolone 30 mg daily orally when she had the 9th attack of painful ophthalmoplegia Pain, ptosis, and diplopia disappeared in 5 days and she did not show any recurrence of symptoms over the next 7 months."}]}}}}
{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[645, 825]], "word_ranges": [[113, 142]], "text": "I would opt for 1, because this entity fulfills the characteristics described (absence of monoclonal peak in most cases, acute renal failure and presence of kappa chains in urine),"}, "2": {"exist": true, "char_ranges": [[105, 357]], "word_ranges": [[19, 60]], "text": "Option 2, nephrotic syndrome, apart from being broad and nonspecific, would not be assessable, since they do not give us the quantification of urine protein (they only tell us that urinalysis reveals the presence of kappa light chains, not the amount)."}, "3": {"exist": true, "char_ranges": [[358, 469]], "word_ranges": [[60, 79]], "text": "Option 3 would be unlikely; in amyloidosis, the light chains that are usually deposited are of the lambda type."}, "4": {"exist": true, "char_ranges": [[470, 602]], "word_ranges": [[79, 104]], "text": "Option 4 also seems unlikely, since in IgA myeloma we would expect a monoclonal peak of this Ig, which in this case does not appear."}, "5": {"exist": true, "char_ranges": [[826, 878]], "word_ranges": [[142, 150]], "text": "being light chain myeloma a somewhat broader entity,"}}, "full_answer": "This question is mixed hemato-nephro. It is complicated, so we are going to rule out options one by one. Option 2, nephrotic syndrome, apart from being broad and nonspecific, would not be assessable, since they do not give us the quantification of urine protein (they only tell us that urinalysis reveals the presence of kappa light chains, not the amount). Option 3 would be unlikely; in amyloidosis, the light chains that are usually deposited are of the lambda type. Option 4 also seems unlikely, since in IgA myeloma we would expect a monoclonal peak of this Ig, which in this case does not appear. The doubt that arises is between 1 and 5; I would opt for 1, because this entity fulfills the characteristics described (absence of monoclonal peak in most cases, acute renal failure and presence of kappa chains in urine), being light chain myeloma a somewhat broader entity, but option 5 could also be considered valid.", "full_answer_no_ref": "This question is mixed hemato-nephro. It is complicated, so we are going to rule out options one by one. [HIDDEN], nephrotic syndrome, apart from being broad and nonspecific, would not be assessable, since they do not give us the quantification of urine protein (they only tell us that urinalysis reveals the presence of kappa light chains, not the amount). [HIDDEN]; in amyloidosis, the light chains that are usually deposited are of the lambda type. [HIDDEN], since in IgA myeloma we would expect a monoclonal peak of this Ig, which in this case does not appear. The doubt that arises is between 1 and 5; [HIDDEN], because this entity fulfills the characteristics described (absence of monoclonal peak in most cases, acute renal failure and presence of kappa chains in urine), being light chain myeloma a somewhat broader entity, but [HIDDEN].", "full_question": "A 68-year-old patient consults for edema and asthenia. Laboratory tests showed creatinine 5 mg/dL, hemoglobin 10 g/dL and marked hypogammaglobulinemia in serum at the expense of IgG, IgA and IgM. A urinalysis reveals the presence of kappa light chains. What is your diagnostic suspicion?", "id": 215, "lang": "en", "options": {"1": "Kappa light chain deposition disease.", "2": "Nephrotic syndrome.", "3": "Amyloidosis.", "4": "IgA myeloma with Bence-Jones proteinuria.", "5": "Light chain myeloma."}, "question_id_specific": 106, "type": "NEPHROLOGY", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0070_11204", "title": "[Diclonal gammopathy (IgG kappa, IgA lambda) with nephrotic syndrome terminating into IgA lambda myeloma after three years--report of a case].", "score": 0.019424799622819428, "content": "A 43-year-old man was admitted to our hospital because of legs edema and periorbital edema in Dec. 1983. Laboratory findings showed massive proteinuria (3.7 g/day), Bence Jones protein (BJP) in urine, and hypoproteinemia. Peripheral blood examinations were normal and a bone marrow aspiration showed hypocellularity with slight increase of monocytes and plasma cells. Serum immunoelectrophoresis showed two M-components (IgG kappa, IgA lambda). Serum IgG was 1,690 mg/dl, IgA 379 mg/dl and IgM 160 mg/dl. No remarkable findings were obtained in bone survey, Ga-scintigraphy and rectal biopsy, and a diagnosis of diclonal gammopathy with nephrotic syndrome was made. In Aug. 1986, serum IgA started to increase rapidly with concomitant decrease IgG. He died of pneumonia due to pancytopenia in Dec. 1986, when serum IgG was 450 mg/dl, IgA 1,014 mg/dl, and IgM less than 39 mg/dl. Immunoelectrophoresis showed two M-components (IgG kappa, IgA lambda) in serum and BEP (kappa, lambda), IgG (kappa) and IgA (lambda) in urine. An autopsy showed massive infiltration of myeloma cells which were positive for lambda light chain in bone marrow, suggesting a development of myeloma from a diclonal gammopathy in about 3 years."}, {"id": "wiki20220301en217_31734", "title": "Plasma cell dyscrasias", "score": 0.018532818532818532, "content": "Non-IgM SMM Non-IgM SMM (also termed IgG and IgA SMM because of the rarity of IgD and IgE SMM) is diagnosed in asymptomatic individuals based on criteria identical to those listed above for Non-IgM MGUS except that: their intact IgG or IgA myeloma protein levels are equal to or greater than 30 grams/liter rather than 15 grams/liter; their bone marrow shows plasma cells comprise between 10% and <60% rather than <10% of nucleated cells; and/or their 24-hour urine contains 0.5 gram or greater levels of Bence Jones, i.e. light chain myeloma, proteins. individuals must also lack evidence of more recently established multiple myeloma-defining criteria viz., CRAB features, amyloidosis, more than one solitary plasmacytoma, and/or serum or urine free light chain κ to λ or λ to κ ratios of 100 or greater."}, {"id": "wiki20220301en217_31727", "title": "Plasma cell dyscrasias", "score": 0.01793758043758044, "content": "Individuals diagnosed with light chain MGUS typically do not express detectable levels of an IgG, IgA, IgD, IgE, or IgM intact myeloma protein in their blood. Rather, they overexpress a monoclonal, aberrant free κ (i.e. kappa) or λ (i.e., lambda) immunoglobulin light chain. For diagnosis, the κ and λ free light chains are quantified by immunological methods and the ratio of κ to λ light chains is used to detect unbalanced light chain synthesis that is indicative of a monoclonal light chain plasma cell dyscrasia. Light chain MGUS is defined as a disorder in which a serum κ to λ free light chain ratio falls outside the normal range of 0.26–1.65 (mean =0.9) provided that it is not associated with: a) any of the CRAB criteria, b) a bone marrow plasma cell count of 10 or a higher percentage of nucleated cells, c) evidence of amyloid deposition (see Light chain deposition disease), and d) an accumulation of 0.5 or more grams of the monoclonal light chain in the urine over a 24-hour period."}, {"id": "wiki20220301en217_31737", "title": "Plasma cell dyscrasias", "score": 0.01722753485655887, "content": "Light chain SMM Light chain smoldering multiple myeloma (light chain SMM) was previously termed idiopathic Bence Jones proteinuria. The condition is currently diagnosed in asymptomatic individuals who have a 24-hour urinary Bence Jones, i.e. light chain myeloma protein level, that is >0.5 grams and/or bone marrow plasma cells that are 10% to <60% of nucleated cells. These individuals must also; lack detectable IgG, IgA, IgD, IgE, or IgM myeloma proteins in sera; have a free κ/λ or λ/κ light chain ratio outside of 0.26 to 1.65 range but less than 100; and/or have no evidence for the presence of any one of the CRAB criteria, amyloidosis, or end organ damage attributable to the myeloma proteins or plasma cells."}, {"id": "pubmed23n0266_1122", "title": "The clinicopathological spectrum of renal amyloidosis.", "score": 0.01721132897603486, "content": "Renal amyloidosis is an uncommon cause of nephrotic syndrome. The clinical conditions in Chinese people remain obscure. This is a retrospective review of the clinicopathological spectrum of 12 cases diagnosed in Taichung Veterans General Hospital from October 1982 to November 1993. Charts and renal pathological slides were reviewed retrospectively. A scoring system was set to evaluate the degree of amyloid deposition in the renal tissue. The clinical profile, immunological data, pathological picture and final outcomes are presented and discussed, with literature review. There were 12 cases of primary amyloidosis including 1 case of multiple myeloma. All were confirmed by renal biopsy. The cases were all male with mean age of onset as 53.3 +/- 11.3 (range: 32 to 65 years). The mean follow-up duration was 22.9 +/- 32.8 months. The initial average creatinine clearance was 66.7 +/- 42.7 ml/min; mean daily urine protein was 7.0 +/- 4.1 grams. Nephrotic syndrome was the main clinical manifestation, present in all 12 cases. Other presenting symptoms and signs included: malaise in 7 cases; hypertension and anorexia in 4 cases; limb numbness in 3 cases; low back pain, dizziness and microhematuria in 2 cases each; anemia, headache, stroke, restrictive cardiomyopathy, hepatomegaly, syncope, body weight loss, dysphagia and skin itching in one case individually. Amyloid cardiomyopathy was present in 4 of the 8 patients who received echocardiography. The mean serum albumin level was 1.9 +/- 0.7 mg/dl, globulin level 3.1 +/- 1.1 mg/dl. Urinary Bence-Jones protein examination was performed in 8 cases; none revealed positive response. The mean immunoglobulin (Ig) level for the patients included: IgG 1018 +/- 901 mg/dl, IgA 262.0 +/- 313.8 mg/dl, IgM 104.8 +/- 84.2 mg/dl. There were at least 4 cases with high levels of one Ig but depressed levels in the others. M-component was shown by immunoelectrophoresis (IEP) in 90% of the cases. IEP impression in 10 cases revealed 2 cases of IgA lambda, 2 cases IgA kappa, 3 cases IgG lambda and 1 case IgG kappa monogammopathy, 1 case free lambda myeloma and 1 case negative. The ratio of kappa to lambda chain was 3:6. Bone marrow biopsy performed in 8 cases found only 1 case with multiple myeloma, one with amyloidosis; the other 6 cases were unremarkable. Mesangium was the site of heaviest amyloid deposition, followed by tubular basement membrane, artery and interstitium. The median survival time for those whose total score was lower than 3 points was 97.5 months; 3 to 5 points, 14 months; 6 points or more, 18.5 months. The median survival time was 21.6 months and 3-year-survival rate was 32.7%. The 2 cases with long-term survival were of 111 months and 84 months. The possible reason included: 1) Organ-limited renal amyloidosis; 2) Light amyloid deposition; 3) Younger age; 4) Other undetected favorable factors. 1) Renal amyloidosis is not a frequent diagnosis of nephrotic syndrome in Taiwan, but it should be suspected in every patient over 50 years old with a recent onset of proteinuria. 2) Renal amyloidosis can be diagnosed only by renal biopsy. 3) Primary renal amyloidosis is a disease of poor prognosis."}, {"id": "pubmed23n0825_14570", "title": "Kidney failure as an unusual initial presentation of biclonal gammopathy (IgD multiple myeloma associated with light chain disease)--a case report.", "score": 0.016337285902503294, "content": "Immunoglobulin D (IgD) myeloma is a rare disease, about 2% of all myelomas, even rarer when accompanied with another multiple myeloma in biclonal gammopathy. We presented a case of biclonal gammopathy-as-sociated manifestation of IgD myeloma and light chain disease in a patient who initially had renal failure. 37-year-old male approximately one month before hospitalization began to feel malaise and fatigue along with decreased urination. Laboratory analysis revealed azotemia. A dialysis catheter was placed and hemodialysis started. The patient was then admitted to our hospital for further tests and during admission, objective examination revealed pronounced paleness with hepatosplenomegaly and hypertension (170/95 mmHg). Laboratory analysis showed erythrocyte sedimentation rate 122 mm/h, expressed anemic syndrome (Hb 71 g/L) and renal failure dialysis rank: creatinine 1,408 micromol/L, urea 31.7 mmol/L. There was two M components in serum protein electrophoresis: IgD lambda and free light chain lambda. Proteinuria was nephrotic rank (5.4 g/24 h), whose electrophoresis revealed 2 M components--massive in alpha 2 fraction of 71%; 7% in the discrete beta fraction, beta 2M / serum 110 mg / L, in urine 1.8 mg/L--extremely high; IgL kappa I lambda index 1:13 (reference value ratio 2:1). The findings pointed to double myeloma disease: IgD myeloma and Bence Jones lambda myeloma. Bone biopsy confirmed IgD myeloma lambda 100% infiltration medulla predominantly plasmablasts. The treatment continued with hemodialysis 3 times per week with chemotherapy protocol bortezomib, doxorubicin, dexamethasone. After 4 cycles of chemotherapy, there was a decrease of IgD, lamda-light chains, reduction in proteinuria (1.03 g/24 h), so hemodialysis was reduced to once per week. Six months after treatment initiation the patient underwent autologous bone marrow transplantation. In a 2-year follow-up period double myeloma disease showed complete remission. The presented rare form of double myeloma disease with initial renal insufficiency underscores the importance of careful observation and teamwork that can alter the course of this serious disease."}, {"id": "pubmed23n1090_5950", "title": "Light Chain Deposition Disease Diagnosed Using Computed Tomography-Guided Kidney Biopsy.", "score": 0.01576934392468373, "content": "Light chain deposition disease (LCDD) is characterized by the deposition of monoclonal immunoglobulin light chains in the kidney, which can cause end-stage kidney disease if not treated. While kidney biopsy is required for definitive diagnosis, choosing an appropriate biopsy method may be problematic when examining patients with atrophic kidneys. A 66-year-old Japanese man was referred to our institution with a three-month history of leg edema. Clinical investigations revealed proteinuria levels of 7.5 g/day. CT-guided percutaneous kidney biopsy was selected as the biopsy method because atrophic kidneys were poorly visualized on ultrasonography. Kidney biopsy revealed nodular glomerulosclerosis, exclusive deposition of the κ chain, and powdery electron-dense deposits, all of which were indicative of LCDD. Bence-Jones protein was detected in the urine. The patient also had an abnormal serum-free light chain ratio. Bone marrow biopsy revealed multiple myeloma; therefore, the patient was diagnosed to have LCDD with multiple myeloma. The patient was treated with daratumumab, bortezomib, cyclophosphamide, and dexamethasone. After a one-year follow-up, the patient had hematological and renal responses without any treatment-related adverse effects. Our case demonstrates the effectiveness of daratumumab as a treatment for LCDD with nephrotic-range proteinuria. Additionally, we suggest that CT-guided kidney biopsy should be considered as a diagnostic test in patients with kidney atrophy when making a definitive diagnosis."}, {"id": "pubmed23n1084_24552", "title": "Myeloma Cast Nephropathy and COVID-19: A Case Report of Multiple Myeloma Presenting as Acute Kidney Injury in the Setting of COVID-19.", "score": 0.015463337831758885, "content": "A 64-year-old African American male presented to the emergency department with subacute low back pain for two weeks and decreased urine output. He was found to have a potassium level of 9.2 mmol/L and was uremic with a creatinine level of 28.5 mg/dL and blood urea nitrogen (BUN) level of 201 mg/dL. He also tested positive for COVID-19. He was then started on continuous renal replacement therapy (CRRT). His urinalysis showed more than 500 mg/dL of protein. A workup for multiple myeloma was also conducted, and urine protein electrophoresis test was positive for free lambda light chains with a level of 17,700 mg/L and free kappa light chains with a level of 88.30 mg/L with a kappa:lambda free light chain ratio of 0.005. Additionally, serum Bence Jones protein level was elevated at 240 mg/dL, and serum beta-2 microglobulin level was elevated at 31.41 mg/L. An immunoglobulin (Ig) panel also showed low levels of IgG, IgA, and IgM. Kidney biopsy for this patient showed definite cast nephropathy and minimal chronic changes, with only one of over 20 glomeruli sclerosed and minimal interstitial deposits. The patient was started on chemotherapy with cyclophosphamide, bortezomib, and dexamethasone (CyBorD)."}, {"id": "pubmed23n0251_18578", "title": "[IgD-lambda type multiple myeloma associated with IgG-kappa type benign monoclonal gammopathy].", "score": 0.015421986216246752, "content": "A 76-year-old man was admitted to Kisen hospital because of lumbago and chest pain. Laboratory examinations revealed a chronic renal failure with marked elevation of the serum BUN (48.8 mg/dl) and creatinine levels (8.2 mg/dl). The serum electrophoresis demonstrated a hypergammaglobulinemia with M peaks. An immunoelectrophoresis demonstrated monoclonal IgD-lambda and IgG-kappa proteins in the serum, and lambda-type Bence Jones protein in the urine (0.4 g/day). Bone marrow smears revealed an abnormal proliferation of atypical plasma cells (43%). A systemic X-ray examination of the skeletal system showed systemic osteoporosis without punched out lesion. The patient was diagnosed as having IgD-lambda type multiple myeloma and IgG-kappa type benign monoclonal gammopathy by quantifying concentration of two M proteins (1,160 mg/dl in IgD, 1,179 mg/dl in IgG, respectively). A combination chemotherapy with melphalan and prednisolone was administered monthly for multiple myeloma, and hemodialysis for the renal failure was performed 3 times a week. A marked improvement of his laboratory findings including a diminution of the serum IgD-lambda M-protein was obtained. On the other hand, IgG-kappa M-protein level was unchanged. Two M-protein levels showed a different behavior after the combination chemotherapy. Although the patient died of congestive heart failure, the partial remission of multiple myeloma has been maintained for 16 months with chemotherapy."}, {"id": "pubmed23n0906_12738", "title": "Gamma 1-heavy chain deposition disease accompanied by IgG kappa in serum, urine, and bone marrow.", "score": 0.014873653907666063, "content": "A 32-year-old Japanese woman presented with hypertension, nephrotic syndrome, microhematuria, and severe hypocomplementemia. Her serum creatinine concentration increased from 1.46 mg/dL (129.0 μmol/L) to 3.46 mg/dL (305.8 μmol/L) over 1 month. Renal biopsy revealed Congo red-negative nodular glomerulosclerosis accompanied by mesangial proliferation. There was extensive staining of immunoglobulin (Ig) G in the glomerular and tubular basement membranes and expanded mesangial regions. Staining was negative for IgA, IgM, and kappa and lambda light chains and positive for the gamma 1 IgG subclass. Staining for constant domains of the gamma heavy chains showed a deletion of the first constant domain (CH1). Electron microscopy revealed electron-dense deposits in the glomerular and tubular basement membranes and mesangium. These findings indicated gamma 1-heavy chain deposition disease (HCDD). Serum and urine immunoelectrophoresis revealed an IgG kappa monoclonal band, whereas bone marrow biopsy revealed monoclonal plasmacytosis with positive staining for kappa chains. HCDD associated with kappa light chain is extremely rare. We report the first case of HCDD with IgG kappa detected in the serum, urine, and bone marrow."}, {"id": "InternalMed_Harrison_8901", "title": "InternalMed_Harrison", "score": 0.014868491680085883, "content": "The serum M component will be IgG in 53% of patients, IgA in 25%, and IgD in 1%; 20% of patients will have only light chains in serum and urine. Dipsticks for detecting proteinuria are not reliable at identifying light chains, and the heat test for detecting Bence Jones protein is falsely negative in ~50% of patients with light chain myeloma. Fewer than 1% of patients have no identifiable M component; these patients usually have light chain myeloma in which renal catabolism has made the light chains undetectable in the urine. In most of these patients, light chains can now be detected by serum free light chain assay. IgD myeloma may also present with light chain disease. About two-thirds of patients with serum M components also have urinary light chains. The light chain isotype may have an impact on survival. Patients secreting lambda light chains have a significantly shorter overall survival than those secreting kappa light chains. Whether this is due to some genetically important"}, {"id": "pubmed23n0265_12799", "title": "[A case report of light and heavy chain deposition disease (IgG2 lambda)].", "score": 0.014800410522335657, "content": "A 73-year-old male was admitted to the renal division of our hospital because of hypertension, proteinuria and bilateral pretibial edema. Eight years previously, he was diagnosed as being afflicted with interstitial pneumonia on the basis of a chest X-ray examination. Laboratory tests conducted during the current admission showed normocytic normochromic anemia, renal dysfunction and mild proteinuria. Total IgG was normal, but a high proportion of IgG2 was observed. M-protein in the serum was positive for both IgG lambda and Bence Jones protein (lambda type). A bone marrow biopsy showed the proportion of plasma cells to be 10.6%, but atypical cells were not found. We diagnosed the patient's condition as plasma cell dyscrasia. Light microscopy examination of a renal biopsy specimen showed moderate mesangial proliferation with a deposition of PAS-positive and Congo red negative materials in the mesangial area: nodular gomerulonephritis was seen in some glomeruli. Immunofluorescence revealed IgG and lambda light chains, strong linear staining along the glomerular basement membrane and tubular basement membrane and positivity in the mesangial area. Results of staining for IgA, IgM, fibrinogen and C3 were weakly positive in the mesangium area, while those for C4, Clq and free kappa were negative. Positive staining of IgG2 was seen by immunoperoxidase study, but the tissue was negative for IgG1, IgG3, IgG4. Electron microscopy demonstrated a dense granular deposition in the mesangial, subendothelial and peritubular area and a microfibrillar structure in the mesangial area. The diameter of the microfibrillar structure was 14 nm on the average.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0496_12964", "title": "A case of primary immunoglobulin light chain amyloidosis with a delayed appearance of Bence Jones protein in urine.", "score": 0.01453833066969119, "content": "We report here a case of a 58-year-old man who had nephrotic syndrome and immunoglobulin light chain (AL) amyloidosis. This patient underwent a renal biopsy to confirm the diagnosis. Treatment with permanganate before Congo red staining showed systemic secondary amyloidosis (AA) fibrils, which were sensitive to permanganate oxidation. Although this patient was initially diagnosed as having AA amyloidosis, he did not have any chronic inflammatory disease and/or malignancy. The level of amyloid A protein (7.9 microg/mL) in sera was within the normal range (0-8.0 microg/mL). Therefore, we performed an immunostaining of the precursor protein (amino terminus of constant region: kappa and lambda light chains, and AA protein) using duodenal biopsy specimens for a precise diagnosis. Immunostaining was positive for the amino terminus of constant region of the lambda light chain, and negative for the amino terminus of constant region of the kappa light chain and AA protein. No plasma cell proliferation in the bone marrow was observed. We finally diagnosed this patient as having primary AL amyloidosis. It appears that a pathological diagnosis must be performed by immunostaining the precursor proteins with the permanganate digestion technique in tissue of patients with amyloidosis. There were no abnormalities in serum and urine immunoelectrophoresis at the time of renal biopsy in this patient. During the follow-up period, after discharge, Bence Jones protein appeared in the urine, but not in the serum. It is necessary to observe patients with primary AL amyloidosis carefully to determine if they their condition will progress to multiple myeloma."}, {"id": "wiki20220301en217_31771", "title": "Plasma cell dyscrasias", "score": 0.01317995710664847, "content": "Multiple myeloma Multiple myeloma is diagnosed in patients that (except for non-secretory multiple myeloma patients) have a clonal IgG, IgA, IgD, or IgE myeloma protein in their serum and/or a clonal κ or λ light chain in their serum or urine plus either one of two sets of criteria. In the first criteria set, patients must have ≥10% bone marrow clonal plasma cells plus ≥1 of the CRAB criteria; in the second criteria set, patients must have ≥10 bone marrow clonal plasma cells plus ≥1 of the following findings, ≥60% bone marrow clonal plasma cells, a free κ/λ or λ/κ light chain ratio in serum of ≥100 (the involved clonal light chain concentration must be ≥100 milligrams/liter), and/or >1 focal bone lesion on magnetic resonance imaging. The 5 year medium survival of patients with multiple myeloma treated with currently used treatment regiments is 48.5%. Light chain multiple myeloma"}, {"id": "wiki20220301en013_91228", "title": "Multiple myeloma", "score": 0.012797619047619047, "content": "In theory, multiple myeloma can produce all classes of immunoglobulin, but IgG paraproteins are most common, followed by IgA and IgM. IgD and IgE myeloma are very rare. In addition, light and or heavy chains (the building blocks of antibodies) may be secreted in isolation: κ- or λ-light chains or any of the five types of heavy chains (α-, γ-, δ-, ε- or μ-heavy chains). People without evidence of a monoclonal protein may have \"nonsecretory\" myeloma (not producing immunoglobulins); this represents about 3% of all people with multiple myeloma. Additional findings may include a raised calcium level (when osteoclasts are breaking down bone, releasing it into the bloodstream), raised serum creatinine level due to reduced kidney function, which is mainly due to casts of paraprotein deposition in the kidney, although the cast may also contain complete immunoglobulins, Tamm-Horsfall protein and albumin."}, {"id": "pubmed23n0527_8791", "title": "Fibrillary glomerulonephritis associated with monoclonal gammopathy of undetermined significance showing lambda-type Bence Jones protein.", "score": 0.012783877274778163, "content": "A 79-year-old woman was admitted to our hospital because of leg edema due to a nephrotic syndrome. Urinary and serum immunoelectrophoresis showed positive for the lambda type of Bence Jones protein. A bone marrow aspiration test revealed mild plasmacytosis (6.4% of the total cells). These findings confirmed her diagnosis of monoclonal gammopathy of undetermined significance (MGUS). Her renal biopsy specimen revealed mild mesangial cell proliferation and an increase in the mesangial matrix. Immunofluorescence studies showed positive staining for IgG, IgA, C3, and kappa and lambda light chains in the capillary wall and mesangium area. Electron microscopy showed that the electron deposits in the thickened basement membrane were formed by randomly arranged 16- to 18-nm nonbranching fibrils. A Congo red stain for amyloid was negative. These findings corresponded with the diagnosis of fibrillary glomerulonephritis. Therefore, this case showed a rare combination of fibrillary glomerulonephritis and MGUS."}, {"id": "wiki20220301en217_31715", "title": "Plasma cell dyscrasias", "score": 0.01272838468007033, "content": "Monoclonal gammopathy of undetermined significance (MGUS), is defined as the presence in the blood or urine of a monoclonal antibody, antibody heavy chain, or antibody light chain in a person lacking symptoms or signs of a more serious plasma cell dyscrasia. The condition is typically discovered as an incidental finding when serum protein electrophoresis is done for various reasons unrelated to plasma cell dyscrasias. Protein electrophoresis generally detects one of the following patterns of monoclonal myeloma protein spikes representing: a) intact IgG, IgA, IgE, IgE, or IgM; b) intact IgG, IgA, IgE, IgD, or IgM plus high concentrations of a free (i.e. not bound to a heavy chain) κ or λ light chain; c) a free κ chain in great excess of a λ chain or a free λ chain in great excess of a κ chain; and d) free γ, δ, or μ heavy chains unbound to a light chain (free α and ε heavy chain myeloma protein spikes have not been reported). Among MGUS cases expressing an intact antibody, 70%, 15%,"}, {"id": "wiki20220301en217_31718", "title": "Plasma cell dyscrasias", "score": 0.012014180672268907, "content": "Non-IgM MGUS, commonly termed MGUS, is diagnosed in individuals who exhibit a serum IgG, IgD, IgA, or IgE monoclonal protein with or without increased levels of blood and/or urine free κ or λ light chains. These patients typically also show small increases in bone marrow plasma cells. Further requirements for the diagnosis of non-IgM MGUS are: a) bone marrow clonal plasma cells <10% of total nucleated cells; b) absence of any of the four CRAB criteria (CRAB criteria are C = Calcium serum levels >1 milligram/deciliter above normal values and/or a serum level >11 milligram/deciliter; R = Renal insufficiency as defined by a glomerular filtration rate <40 milliliter/minute and/or a serum creatinine >2 gram/deciliter due to myeloma protein-induced kidney damaged; A = Anemia, as defined by a blood hemoglobin level >2 gram/deciliter below normal and/or <10 gram/deciliter due to the plasma cell dyscrasia rather than e.g. iron deficiency or blood loss; B = Bone lesions, i.e. ≥1 lytic (i.e."}, {"id": "wiki20220301en056_72578", "title": "Bence Jones protein", "score": 0.011517893870835048, "content": "The proteins are immunoglobulin light chains (paraproteins) and are produced by neoplastic plasma cells. They can be kappa (most of the time) or lambda. The light chains can be immunoglobulin fragments or single homogeneous immunoglobulins. They are found in urine as a result of decreased kidney filtration capabilities due to kidney failure, sometimes induced by hypercalcemia from the calcium released as the bones are destroyed ,dehydration due to polyurea, amyloidosis or from the light chains themselves. The light chains have historically been detected by heating a urine specimen (which causes the protein to precipitate) and now by electrophoresis of concentrated urine. More recently, serum free light chain assays have been utilised in a number of published studies which have indicated superiority over the urine tests, particularly for patients producing low levels of monoclonal free light chains, as seen in nonsecretory multiple myeloma and AL amyloidosis. History"}, {"id": "InternalMed_Harrison_3384", "title": "InternalMed_Harrison", "score": 0.011404151404151405, "content": "PROTEINURIA ON URINE DIPSTICK Quantify by 24-h urinary excretion of protein and albumin or first morning spot albumin-to-creatinine ratio RBCs or RBC casts on urinalysis In addition to disorders listed under microalbuminuria consider Myeloma-associated kidney disease (check UPEP) Intermittent proteinuria Postural proteinuria Congestive heart failure Fever Exercise Go to Fig. 61-2 Macroalbuminuria 300-3500 mg/d or 300-3500 mg/g Microalbuminuria 30-300 mg/d or 30-300 mg/g Nephrotic range > 3500 mg/d or > 3500 mg/g + Consider Early diabetes Essential hypertension Early stages of glomerulonephritis (especially with RBCs, RBC casts)Consider Early diabetes Essential hypertension Early stages of glomerulonephritis (especially with RBCs, RBC casts) Nephrotic syndrome Diabetes Amyloidosis Minimal change disease FSGS Membranous glomerulopathy IgA nephropathy"}, {"id": "pubmed23n0027_4519", "title": "[IgD-multiple myeloma. Review of 102 cases reported in the literature (author's transl)].", "score": 0.011097000483792937, "content": "Reports on 102 patients suffering from IgD-myeloma are reviewed and analyzed. Patients with IgD-myeloma are younger than patients with myeloma producing IgG or IgA myeloma proteins.  Males are affected by this disease 3 times as often as females and 11 times as often as female patients in the group producing kappa light chain type of IgD myeloma protein. Hyperproteinaemia and extreme spikes of the monoclonal immunoglobulin occur less often. Approximately 90% of the patients have a lambda light chain myeloma protein and almost all patients excrete Bence-Jones protein. Renal insufficiency, amyloidosis, and plasma-cell leukemia are found more frequently than in other types of multiple myeloma. IgD-multiple myeloma carries a poorer prognosis, possibly related to the frequent finding of renal insufficiency."}, {"id": "wiki20220301en044_52085", "title": "Cryoglobulinemia", "score": 0.010994044892349977, "content": "Signs and symptoms due to the cryoglobulins of type I disease reflect the hyperviscosity and deposition of cryoglobulins within the blood vessels which reduce or stop blood perfusion to tissues. These events occur particularly in cases where blood cryoglobulin levels of monoclonal IgM are high in patients with IgM MGUS, smoldering Waldenström's macroglobulinemia, or Waldenström's macroglobulinemia and in uncommon cases where the levels of monoclonal IgA, IgG, free κ light chains, or free λ light chains are extremely high in patients with non-IgM MGUS, non-IgM smoldering multiple myeloma, or multiple myeloma. The interruption of blood flow to neurological tissues can cause symptoms of confusion, headache, hearing loss, and peripheral neuropathy. Interruption of blood flow to other tissues in type I disease can cause cutaneous manifestations of purpura, blue discoloration of the arms or legs (acrocyanosis), necrosis, ulcers, and livedo reticularis; spontaneous nose bleeds, joint pain,"}, {"id": "wiki20220301en217_31712", "title": "Plasma cell dyscrasias", "score": 0.01076555023923445, "content": "IgM myeloma proteins or in rare cases other myeloma proteins such as IgA, free κ light chains, or free λ light chains may increase blood viscosity, deposit in peripheral blood vessels, and thereby cause vascular occlusion and gangrene of the extremities in the syndrome termed cryoglobulinemia. Monoclonal IgM myeloma proteins operating through their effects on increasing blood hyperviscosity can reduce blood flow to the central nervous system to cause blurred vision, headaches, vertigo, ataxia, and cold-induced hemolytic anemia. IgM, IgG, and to lesser extents κ and λ free light chain myeloma proteins can cause Immune thrombocytopenic purpura with extensive bleeding tendencies ."}, {"id": "wiki20220301en217_31743", "title": "Plasma cell dyscrasias", "score": 0.010227504590899817, "content": "AL amyloidosis can occur at any stage in the plasma cell dyscrasia spectrum. Typically, patients developing this type of amyloidosis have had excess κ or λ free light chains in their urine for years before diagnosis. At diagnosis, however, they typically have a relatively small plasma cell burden (bone marrow plasma cells <5% to 7% of total nucleated cells) and in only <5% to 10% of cases do other findings indicate the presence of a malignant condition (i.e. definitive signs of multiple myeloma, Waldenström macroglobulinemia, or chronic lymphocytic leukemia associated with over-production of a clonal light chain). Nonetheless, these individuals often evidence serious involvement of the kidney (proteinuria, nephrotic syndrome) or heart (restrictive cardiomyopathy, arrhythmias) in 70% or 60% of cases, respectively, and of dysfunction in the peripheral nervous system (numbness, paresthesias) or autonomic nervous system (orthostatic hypotension) in 20% or 15% of cases, respectively. They"}, {"id": "wiki20220301en217_31716", "title": "Plasma cell dyscrasias", "score": 0.010113625232637868, "content": "chain; and d) free γ, δ, or μ heavy chains unbound to a light chain (free α and ε heavy chain myeloma protein spikes have not been reported). Among MGUS cases expressing an intact antibody, 70%, 15%, 12%, and 3% express either IgG, IgM, IgA, or two of these M proteins, respectively, with or without excessive levels of a light chain; these cases represent ~80% of all MGUS. About 20% of MGUS cases express either κ or λ light chains. As a group, these MGUS findings occur more commonly in men and are ~2-fold more common in individuals of African descent than Caucasians. MGUS cases expressing free γ, δ, or μ heavy chains are extremely rare. MGUS is categorized into the following sub-types based upon the identity and levels of the myeloma proteins detected as well as the prognoses for progressive disease indicated by these myeloma protein findings."}, {"id": "article-25248_22", "title": "Monoclonal Gammopathy of Undetermined Significance -- Differential Diagnosis", "score": 0.009925314465408805, "content": "Monoclonal gammopathy of renal significance: This disease entity is diagnosed when the patient has diagnostic criteria for MGUS as well as renal insufficiency and monoclonal immunoglobulin deposits in the kidney by immunofluorescence. [11] Light chain smoldering multiple myeloma (idiopathic Bence Jones proteinuria): The diagnostic criteria of LC-SMM include: Monoclonal light chains in the urine (Bence Jones proteinuria) No immunoglobulin heavy chain expression in the serum or urine No symptoms of PCM, WM, or amyloid light chain amyloidosis Primary (amyloid light chain) amyloidosis and light chain deposition disease: This entity of plasma cell neoplasms is associated with the pathologic deposition of monoclonal light chains in tissue. The diagnostic criteria include the presence of amyloid in tissue and evidence of plasma cell neoplasm. B cell lymphoproliferative disorder"}, {"id": "wiki20220301en013_91229", "title": "Multiple myeloma", "score": 0.00991304347826087, "content": "Other useful laboratory tests include quantitative measurement of IgA, IgG, and IgM to look for immune paresis, and beta-2 microglobulin, which provides prognostic information. On peripheral blood smear, the rouleaux formation of red blood cells is commonly seen, though this is not specific. The recent introduction of a commercial immunoassay for measurement of free light chains potentially offers an improvement in monitoring disease progression and response to treatment, particularly where the paraprotein is difficult to measure accurately by electrophoresis (for example in light chain myeloma, or where the paraprotein level is very low). Initial research also suggests that measurement of free light chains may also be used, in conjunction with other markers, for assessment of the risk of progression from MGUS to multiple myeloma."}, {"id": "pubmed23n0027_10164", "title": "Distribution of heavy chain classes and light chain types in 757 cases of monoclonal gammapathies.", "score": 0.009903174408628508, "content": "The distribution in heavy chain classes and light chain types of M components were studied in 757 cases of monoclonal gammapathies. These gammapathies were classified according to clinical and hematological data in 439 myeloma (MM), 165 Waldenström's macroglobulinemia (WM) and 152 monoclonal gammapathies occurring in other conditions. The IgG/IgA ratio differs in myeloma and in non-myelomatous gammapathies (64% IgG versus 33% IgA in myeloma and 95% IgG versus 5% in absence of myeloma). Presence of free light chains in patient's urines (Bence Jones proteinuria) was detected in about 72% of cases of MM, 48% of WM and only in 9% in others gammapathies. Concerning the sex of patients, an equal repartition between males and females is observed in MM, whereas males predominate in WM. The age distribution of the patients shows that 74% of myeloma and 79% of WM were above sixty. In addition to the 757 cases under study, 10 sera with two M Components were characterized."}, {"id": "pubmed23n1091_7037", "title": "Diagnosis of light chain cast nephropathy through immunostaining of the urine: Perfecting the \"liquid biopsy\".", "score": 0.009900990099009901, "content": "Light chain (LC) cast nephropathy is the main cause of kidney injury and an important determinant of poor survival in patients with multiple myeloma (MM). It is usually suspected when an MM patient with elevated serum concentration of free LC presents kidney failure, but it often requires confirmation by kidney biopsy. We report the case of a 73-year-old woman who presented with fatigue, weight loss, and constipation. Laboratory exams revealed anemia, hypercalcemia, and kidney failure. Urine sediment analysis demonstrated irregular crystalline \"waxy type\" casts. With the hypothesis of LC cast nephropathy, immunostaining of the urine sediment was performed. The analysis revealed several rectangular and irregular casts with intense and bright stain for λ LCs only. A myelogram was performed, showing extensive occupation of the bone marrow by plasma cells; and immunofixation in urine and serum revealed monoclonal IgG-λ component, confirming the diagnosis of IgG-λ MM. This case highlights the potential utility of the urine sediment analysis and immuno-staining as a reliable non-invasive alternative method for diagnosis of cast nephropathy in patients with monoclonal gammopathies."}, {"id": "wiki20220301en217_31741", "title": "Plasma cell dyscrasias", "score": 0.009889259635378886, "content": "Increases in the levels of free κ or λ light chains are a common feature of plasma cell dyscrasias. These increases occur in: 40% of IgM MGUS, IgM SMM, and Waldenstroms macroglbulonemia cases; 60% to 70% of non-secretory multiple myelom cases; 90% to 95% of intact immunoglobulin multiple myeloma cases; and, by definition, 100% of light chain multiple myeloma cases. There are two different types of plasma cell dyscrasia-associated amyloidosis syndromes: amyloid light chain amyloidosis (AL amyloidosis) in which amyloid deposits consist of free light chains and amyloid heavy chain amyloidosis (AH amyloidosis) in which amyloid deposits contain only free heavy chains. The deposits in a third type, AHL amyloidosis, consists of both free light chains and free heavy chains. AHL amyloidosis is here, as in some recent reports, grouped with AH amyloidosis."}, {"id": "wiki20220301en261_11844", "title": "Serum free light-chain measurement", "score": 0.00987618283101024, "content": "Guidelines In 2009, the International Myeloma Working Group published guidelines making recommendations of when serum free light-chain analysis should be used in the management of multiple myeloma. Diagnosis The serum free light-chain assay in combination with serum protein electrophoresis and serum immunofixation electrophoresis is sufficient to screen for pathological monoclonal plasmaproliferative disorders other than AL amyloidosis which requires all the serum tests as well as 24 h urine immunofixation electrophoresis. Monitoring Serial serum free light-chain measurement should be routinely performed in patients with AL amyloidosis and multiple myeloma patients with oligosecretory disease. It should also be done in all patients who have achieved a complete response to treatment to determine whether they have attained a stringent complete response."}, {"id": "pubmed23n1100_5080", "title": "Efficacy and safety of plasmapheresis in symptomatic hyperviscosity and cast nephropathy: A Multicenter Experience in Turkey.", "score": 0.00980392156862745, "content": "Cast nephropathy (CN) and hyperviscosity (HV), which we encounter in plasma cell diseases, are serious clinical manifestations that increase mortality and morbidity if not managed well in the early period. Therapeutic plasma exchange (TPE) procedures based on the removal of patient plasma is a frequently preferred treatment modality. TPE is recommended at varying levels of evidence for the treatment of CN and HV in plasma cell disorders. A total of 61 patients, 50 with multipl myeloma (MM) and 10 with Waldenström macroglobulinemia (WM), who underwent TPE for CN and HV, were included in our multicenter, and retrospective study. A statistically significant decrease was found in all disease-related biochemical markers, which were measured 1 week after the application of TPE added to standard medical treatment (IgG; p &lt; 0.001, IgM; p = 0.004, IgA; p = 0.14, kappa light chain; p &lt; 0.001, lambda light chain; p &lt; 0.001, β-2 microglobulin; p &lt; 0.001, total protein; p &lt; 0.001, albumin; p &lt; 0.001, LDH; p = 0.02, creatine; p &lt; 0.001, hemoglobin; p = 0.010). Clinically, all 11 patients who underwent TPE for HV responded. While a partial response (PR: 80 %) was obtained in 40 of 50 MM patients with CN, no response was obtained in 10 patients (non-response: 20 %). In conclusion, it was observed that TPE reduced all biochemical markers related to HV and CN, while making a significant contribution to clinical improvement. We believe that adding TPE to the standard treatment in this patient group will reduce mortality and morbidity in the early period and have a positive effect on survival in the long term."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 154]], "word_ranges": [[0, 22]], "text": "The acidosis with elevated PCO2, hypoxemia and normal bicarbonate suggests an acute respiratory acidosis of rapid onset probably due to drug intoxication."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The acidosis with elevated PCO2, hypoxemia and normal bicarbonate suggests an acute respiratory acidosis of rapid onset probably due to drug intoxication.", "full_answer_no_ref": "The acidosis with elevated PCO2, hypoxemia and normal bicarbonate suggests an acute respiratory acidosis of rapid onset probably due to drug intoxication.", "full_question": "A 24-year-old woman is found lying in the street by passers-by. On arrival of the emergency team she was found with an oxygen saturation of 88% breathing room air and on examination with pinpoint pupils. She was transferred to the emergency room of the nearest hospital, where the baseline arterial blood gases showed: pH 7.25, PaC02 60 mmHg, Pa02 58 mmHg, bicarbonate 26 mEq/1 and base excess of -1. In blood sodium was 137 mEq/1 and chloride 100 mEq/1:", "id": 264, "lang": "en", "options": {"1": "Partial respiratory failure.", "2": "Metabolic acidosis.", "3": "Pure respiratory acidosis.", "4": "Respiratory alkalosis due to lack of chloride.", "5": "Blood gases can only be venous blood."}, "question_id_specific": 135, "type": "PNEUMOLOGY", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en013_157313", "title": "Arterial blood gas test", "score": 0.016560678325384208, "content": "Respiratory alkalosis (Pa CO2 < 35 mmHg) occurs when there is too little carbon dioxide in the blood. This may be due to hyperventilation or else excessive breaths given via a mechanical ventilator in a critical care setting. The action to be taken is to calm the person and try to reduce the number of breaths being taken to normalize the pH. The respiratory pathway tries to compensate for the change in pH in a matter of 2–4 hours. If this is not enough, the metabolic pathway takes place. Under normal conditions, the Henderson–Hasselbalch equation will give the blood pH where: 6.1 is the acid dissociation constant (pKa) of carbonic acid () at normal body temperature [] is the concentration of bicarbonate in the blood in mEq/L PaCO2 is the partial pressure of carbon dioxide in the arterial blood in mmHg"}, {"id": "pubmed23n1017_8255", "title": "Can Venous Blood Gas Be Used as an Alternative to Arterial Blood Gas in Intubated Patients at Admission to the Emergency Department? A Retrospective Study.", "score": 0.016382868937048503, "content": "Blood gas analysis plays an important role in both diagnosis and subsequent treatment of critically ill patients in the emergency department and the ICU. Historically, arterial blood is predominantly used for blood gas analysis. The puncture is painful and complications may occur. The purpose of the present study was to evaluate the agreement between arterial and venous blood gas analysis and whether the sole use of venous blood gas analysis would have changed therapy. Adult patients who were intubated in the field and received an arterial and venous blood gas analysis within 15 mins after admission to the ED were eligible for inclusion. The values for pH, pCO<sub2</sub, HCO<sub3</sub-, base excess and lactate levels were collected retrospectively. Mean differences were calculated by subtracting venous from arterial values. The agreement between venous and arterial measurements was assessed using the method of Bland and Altman. Blood gases were assessed by two independent physicians using a standardized questionnaire to determine whether the use of venous blood gases would have led to a different interpretation of the situation (other diagnostic path) or a change of therapy (eg. respirator adjustment). Acceptable limits were defined before the collection of data started. Fifty patients (62% male, median age 63years) who were treated at the Emergency Department between June 1, 2014 and December 31, 2014 were included in the study. Following average differences and limits of agreement (LOA) were documented: pH 0.02312 with LOA from -0.048 to 0.094; pCO<sub2</sub -3.612 mmHg with LOA from -15 to 8.1 mmHg; BE -0.154 mmol/l with LOA from -3.7 to 3.4 mmol/l; HCO<sub3</sub-0.338 mmol/l with LOA from -2.27 to 2.9 mmol/l; Lactate -0.124 mg/dl with LOA from -2.28 to 2.03 mg/dl. Using venous blood gas results 100% of the patients with metabolic alkalosis were correctly diagnosed. Metabolic acidosis was detected with a high sensitivity (80.64%), specificity (89.47%) and positive predictive value (92.59%). The answers to lactate and acidosis due to AKI showed a specificity and positive predictive value of 100%. The respiratory adjustment showed a high sensitivity (91.89%) but a low specificity (38.46%). For pH, bicarbonate, BE and lactate venous blood gases can be used as surrogates for arterial measurements. Venous pCO<sub2</sub can be used for screening of hypercapnia and trending. Respirator adjustments may be done too often if the venous blood gas is used."}, {"id": "wiki20220301en040_1133", "title": "Respiratory acidosis", "score": 0.015802318486882246, "content": "Diagnosis Diagnoses can be done by doing an ABG (Arterial Blood Gas) laboratory study, with a pH <7.35 and a PaCO2 >45 mmHg in an acute setting. Patients with COPD and other Chronic respiratory diseases will sometimes display higher numbers of PaCO2 with HCO3- >30 and normal pH. Terminology Acidosis refers to disorders that lower cell/tissue pH to < 7.35. Acidemia refers to an arterial pH < 7.36. See also Acidosis Alkalosis Arterial blood gas Chemical equilibrium pCO2 pH pKa Metabolic acidosis Metabolic alkalosis Respiratory alkalosis References External links Acid–base disturbances"}, {"id": "wiki20220301en074_45830", "title": "Base excess", "score": 0.014755359031048736, "content": "Interpretation Base excess beyond the reference range indicates metabolic alkalosis if too high (more than +2 mEq/L) metabolic acidosis if too low (less than −2 mEq/L) Blood pH is determined by both a metabolic component, measured by base excess, and a respiratory component, measured by PaCO2 (partial pressure of carbon dioxide). Often a disturbance in one triggers a partial compensation in the other. A secondary (compensatory) process can be readily identified because it opposes the observed deviation in blood pH. For example, inadequate ventilation, a respiratory problem, causes a buildup of CO2, hence respiratory acidosis; the kidneys then attempt to compensate for the low pH by raising blood bicarbonate. The kidneys only partially compensate, so the patient may still have a low blood pH, i.e. acidosis. In summary, the kidneys partially compensate for respiratory acidosis by raising blood bicarbonate."}, {"id": "wiki20220301en013_157315", "title": "Arterial blood gas test", "score": 0.014693387975411063, "content": "In general, it is much easier to correct acute pH derangement by adjusting respiration. Metabolic compensations take place at a much later stage. However, in a critical setting, a person with a normal pH, a high CO2, and a high bicarbonate means that, although there is a high carbon dioxide level, there is metabolic compensation. As a result, one must be careful as to not artificially adjust breaths to lower the carbon dioxide. In such case, lowering the carbon dioxide abruptly means that the bicarbonate will be in excess and will cause a metabolic alkalosis. In such a case, carbon dioxide levels should be slowly diminished. See also Anion gap Radial artery puncture Chemical equilibrium Hemoximetry Arteriovenous oxygen difference References External links Online arterial blood gas calculator Diagnostic intensive care medicine Respiratory physiology Blood tests Respiratory therapy"}, {"id": "wiki20220301en198_22391", "title": "PCO2", "score": 0.014252486762013164, "content": "Medicine In medicine, the partial pressure of carbon dioxide in arterial blood is called or PaCO2. Measurement of in the systemic circulation indicates the effectiveness of ventilation at the lungs' alveoli, given the diffusing capacity of the gas. It is a good indicator of respiratory function and the closely related factor of acid–base homeostasis, reflecting the amount of acid in the blood (without lactic acid). Normal values for humans are in the range 35–45 mmHg. Values less than this may indicate hyperventilation and (if blood pH is greater than 7.45) respiratory alkalosis. Values greater than 45 mmHg may indicate hypoventilation, and (if blood pH is less than 7.35) respiratory acidosis. Aquatic Sciences"}, {"id": "article-17837_19", "title": "Arterial Blood Gas -- Results, Reporting, and Critical Findings", "score": 0.014211334926596108, "content": "Example 1 [28] : ABG: pH = 7.39, PaCO 2 = 51 mm Hg, PaO 2 = 59 mm Hg, HCO 3 = 30 mEq/L and SaO 2 = 90%, on room air. pH is in the normal range, so use 7.40 as a cutoff point, in which case it is < 7.40, and acidosis is present. The elevated PaCO 2 indicates respiratory acidosis, and the elevated HCO 3 indicates a metabolic alkalosis. The value consistent with the pH is PaCO 2 . Therefore, this is a primary respiratory acidosis. The acid-base that is inconsistent with the pH is the elevated HCO3, indicating a metabolic alkalosis, so there is compensation signifying a non-acute primary disorder because it takes days for metabolic compensation to be effective. Last, the decreased PaO 2 indicates an abnormality with oxygenation. However, a history and physical will help delineate the severity and urgency of required interventions, if any. Example 2 [28] : ABG: pH = 7.45, PaCO 2 = 32 mm Hg, PaO 2 = 138 mm Hg, HCO 3 = 23 mEq/L, the base deficit = 1 mEq/L, and SaO 2 is 92%, on room air."}, {"id": "InternalMed_Harrison_3678", "title": "InternalMed_Harrison", "score": 0.014014801110083255, "content": "The patient presented with a mixed acid-base disorder, with a significant metabolic alkalosis and a bicarbonate concentration of 44 meq/L. A venous blood gas was drawn soon after his presentation; venous and arterial blood gases demonstrate a high level of agreement in hemodynamically stable patients, allowing for the interpretation of acid-base disorders with venous blood gas results. In response to his metabolic alkalosis, the Pco2 should have increased by 0.75 mmHg for each 1-meq/L increase in bicarbonate; the expected Pco2 should have been ~55 mmHg. Given the Pco2 of 62 mmHg, he had an additional respiratory acidosis, likely caused by respiratory muscle weakness from his acute hypokalemia and subacute hypercortisolism."}, {"id": "pubmed23n0770_14610", "title": "Peripheral venous blood gases and pulse-oximetry in acute cardiogenic pulmonary oedema.", "score": 0.0139817911557042, "content": "The role of venous blood gases as an alternative to arterial blood gases in patients with severe acute heart failure has not been established. To assess the correlation between arterial and peripheral venous blood gases together with pulse-oximetry (SpO2), as well as to estimate arterial values from venous samples in the first hours upon admission of patients with acute cardiogenic pulmonary oedema. Simultaneous venous and arterial blood samples were extracted on admission and over the next 1, 2, 3, 4, and 10 hours. SpO2 was also registered at the same intervals. A total of 178 pairs of samples were obtained from 34 consecutive patients with acute cardiogenic pulmonary oedema. Arterial and venous blood gases followed a parallel course in the first hours, showing high correlation rates at all time intervals. Venous samples underestimated pH (mean difference -0.028) and overestimated CO2 (+5.1 mmHg) and bicarbonate (+1 mEq/l). Conversely, SpO2 tended to underestimate SaO2 (mean±SD: 93.1±9.1 vs. 94.2±8.4). Applying simple mathematical formulae based on these differences, arterial values were empirically calculated from venous samples, showing acceptable agreement in the Bland-Altman test. Likewise, a venous pH &lt;7.32, pCO2 &gt;51.3 mmHg, and bicarbonate &lt;22.8 mEq/l could fairly identify arterial acidosis, either respiratory or metabolic, with a test accuracy of 92, 68, and 91%, respectively. In patients with cardiogenic pulmonary oedema, arterial blood gas disturbances may be estimated from peripheral venous samples. By monitoring SpO2 simultaneously, arterial punctures could often be avoided."}, {"id": "wiki20220301en013_157309", "title": "Arterial blood gas test", "score": 0.013935117383393245, "content": "Guidelines A 1 mmHg change in PaCO2 above or below 40 mmHg results in 0.008 unit change in pH in the opposite direction. The PaCO2 will decrease by about 1 mmHg for every 1 mEq/L reduction in [] below 24 mEq/L A change in [] of 10 mEq/L will result in a change in pH of approximately 0.15 pH units in the same direction. Assess relation of pCO2 with pH: If pCO2 & pH are moving in opposite directions i.e., pCO2 ↑ when pH is <7.4 or pCO2 ↓ when pH > 7.4, it is a primary respiratory disorder. If pCO2 & pH are moving in same direction i.e., pCO2 ↑when pH is >7.4 or pCO2 ↓ when pH < 7.4, it is a primary metabolic disorder. Parameters and reference ranges These are typical reference ranges, although various analysers and laboratories may employ different ranges."}, {"id": "wiki20220301en013_157311", "title": "Arterial blood gas test", "score": 0.01387906647807638, "content": "The normal range for pH is 7.35–7.45. As the pH decreases (< 7.35), it implies acidosis, while if the pH increases (> 7.45) it implies alkalosis. In the context of arterial blood gases, the most common occurrence will be that of respiratory acidosis. Carbon dioxide is dissolved in the blood as carbonic acid, a weak acid; however, in large concentrations, it can affect the pH drastically. Whenever there is poor pulmonary ventilation, the carbon dioxide levels in the blood are expected to rise. This leads to a rise of carbonic acid, leading to a decrease in pH. The first buffer of pH will be the plasma proteins, since these can accept some H+ ions to try to maintain acid-base homeostasis. As carbon dioxide concentrations continue to increase (PaCO2 > 45 mmHg), a condition known as respiratory acidosis occurs. The body tries to maintain homeostasis by increasing the respiratory rate, a condition known as tachypnea. This allows much more carbon dioxide to escape the body through the"}, {"id": "article-26709_8", "title": "Partial Pressure of Carbon Dioxide -- Clinical Significance", "score": 0.01360146862483311, "content": "There are differences in the acute and chronic stages of respiratory acidosis or alkalosis. Acute respiratory acidosis from increased PCO2 will result in immediate changes to serum bicarbonate levels due to the bicarbonate buffer system; however, this is limited in its ability to achieve homeostasis. The kidney will gradually increase the serum bicarbonate levels in chronic cases. Chronic respiratory acidosis is when the acidemia exists for 3 to 5 days, which is approximately how long it will take the kidney to buffer the acidemia. In acute respiratory acidosis, normally, the serum bicarbonate will increase by 1 mEq/L for every 10 mmHg increase in PCO2. For chronic respiratory acidosis, the serum bicarbonate will increase by 4 to 5 mEq/L for every 10 mmHg rise in PCO2. [6] [5] The result typically causes a mild chronic acidosis or low-normal pH near 7.35. [6] In regards to respiratory alkalosis, the same timeframe applies to acute versus chronic. In acute respiratory alkalosis, for every decrease in PCO2 by 10 mmHg, the serum bicarbonate will also decrease by 2 mEq/L. In chronic respiratory alkalosis, or alkalosis lasting 3 to 5 days, for every 10mmHg drop in PCO2, it is expected that serum bicarbonate will decrease by 4 to 5 mEq/L. [5] [6]"}, {"id": "wiki20220301en013_157316", "title": "Blood gas test", "score": 0.01315844966471258, "content": "A blood gas test or blood gas analysis tests blood to measure blood gas tension values, it also measures blood pH, and the level and base excess of bicarbonate. The source of the blood is reflected in the name of each test; arterial blood gases come from arteries, venous blood gases come from veins and capillary blood gases come from capillaries. The blood gas tension levels of partial pressures can be used as indicators of ventilation, respiration and oxygenation. Analysis of paired arterial and venous specimens can give insights into the aetiology of acidosis in the newborn. Values measured"}, {"id": "wiki20220301en013_157318", "title": "Blood gas test", "score": 0.01284606866002215, "content": "Blood gas tests also measure the levels of bicarbonate and of standard bicarbonate, of base excess, of oxygen saturation, and of pH. An arterial blood gas test is more often used. Clinical significance Blood gas tests can be used in the diagnosis of a number of acidosis conditions such as lactic, metabolic, and respiratory acidosis, diabetic ketoacidosis, and also of respiratory alkalosis. Particularly, umbilical cord blood gas analysis can give an indication of preceding fetal hypoxic stress. In combination with other clinical information, normal paired arterial and venous cord blood gas results can usually provide a robust defence against a suggestion that an infant had an intrapartum hypoxic‐ischaemic event."}, {"id": "wiki20220301en040_1132", "title": "Respiratory acidosis", "score": 0.01227106227106227, "content": "Acute respiratory acidosis: HCO3− increases 1 mEq/L for each 10 mm Hg rise in PaCO2. Chronic respiratory acidosis: HCO3− rises 3.5 mEq/L for each 10 mm Hg rise in PaCO2. The expected change in pH with respiratory acidosis can be estimated with the following equations: Acute respiratory acidosis: Change in pH = 0.08 X ((40 − PaCO2)/10) Chronic respiratory acidosis: Change in pH = 0.03 X ((40 − PaCO2)/10) Respiratory acidosis does not have a great effect on electrolyte levels. Some small effects occur on calcium and potassium levels. Acidosis decreases binding of calcium to albumin and tends to increase serum ionized calcium levels. In addition, acidemia causes an extracellular shift of potassium, but respiratory acidosis rarely causes clinically significant hyperkalemia. Diagnosis"}, {"id": "article-26709_9", "title": "Partial Pressure of Carbon Dioxide -- Clinical Significance", "score": 0.012170712809917356, "content": "The regulation of PCO2 is also involved in metabolic acidosis and alkalosis, as well. In metabolic acidosis, for every 1 mEq/L drop of bicarbonate, there will be a decrease in PCO2 by 1.2 mmHg. [5] For sudden drops in bicarbonate, it will take approximately 12 to 24 hours to reach full compensation; however, this process will start in as early as 30 minutes by chemoreceptor and CSF pH changes. [7] Another way to determine the expected PCO2 and compare the value obtained on blood gas analysis with metabolic acidosis is to utilize Winter’s equation. [8] If the measured PCO2 is higher or lower than the Winter’s equation PCO2, there may be a secondary respiratory acidosis or alkalosis, respectively. This situation may be the case in underlying lung pathology or neuromuscular pathology, such as an anoxic injury-causing minute ventilation control to become reduced. Also, in the presence of very severe metabolic acidosis, there is a limit to respiratory compensation with minute ventilation. The PCO2 typically cannot fall below 8 to 12 mmHg, and the sustained increase in minute ventilation to achieve this low PCO2 will usually cause rapid respiratory fatigue. In the case of metabolic alkalosis, the expected compensation of PCO2 is to increase by 0.7 mmHg for every 1 mEq/L increase in serum bicarbonate. [5]"}, {"id": "pubmed23n0744_20632", "title": "[What you should know of the arterial blood gases during the watch].", "score": 0.012042875157629257, "content": "Gasometry is the measurement of dissolved gases in the blood, by measuring pH, carbon dioxide pressure (pCO(2)), serum bicarbonate (HCO(3-)), and lactate and serum electrolytes: sodium, potassium and chlorine you can make a diagnosis, etiology and treatment in the critically ill patient. The aim is to provide five steps for the interpretation of blood gases by: 1. The definition of acidemia or acidosis, or alkalemia or alkalosis. 2. Defining the metabolic component or respiratory. 3. To determine the anion gap; levels above 15 ± 2 determine other likely causes of excess anions (methanol, uremia, diabetic ketoacidosis, paraldehyde, ionized, lactic acidosis, ethylene glycol and salicylates. 4. Compensation, using the Winter formula. 5. The delta gap, with the formula for determining intrinsic and metabolic alkalosis. When anion gap is normal, is calculated urinary anion gap; the value is negative if the loss is extrarenal, contrary to the positive result is renal etiology."}, {"id": "wiki20220301en020_19466", "title": "Hypercapnia", "score": 0.012010932105868816, "content": "In many people a high causes a feeling of shortness of breath, but the lack of this symptom is no guarantee that the other effects are not occurring. A significant percentage of rebreather deaths have been associated with CO2 retention. The effects of high can take several minutes to hours to resolve once the cause has been removed. Diagnosis Blood gas tests may be performed, typically by radial artery puncture, in the setting of acute breathing problems or other acute medical illness. Hypercapnia is generally defined as an arterial blood carbon dioxide level over 45 mmHg (6 kPa). Since carbon dioxide is in equilibrium with carbonic acid in the blood, hypercapnia drives serum pH down, resulting in respiratory acidosis. Clinically, the effect of hypercapnia on pH is estimated using the ratio of the arterial pressure of carbon dioxide to the concentration of bicarbonate ion, . Tolerance"}, {"id": "wiki20220301en142_15270", "title": "Pulmonary gas pressures", "score": 0.01185811865053223, "content": "The alveolar pO2 is not routinely measured but is calculated from blood gas measurements by the alveolar gas equation. Partial pressure of carbon dioxide Pathology The partial pressure of carbon dioxide, along with the pH, can be used to differentiate between metabolic acidosis, metabolic alkalosis, respiratory acidosis, and respiratory alkalosis. Hypoventilation exists when the ratio of carbon dioxide production to alveolar ventilation increases above normal values – greater than 45mmHg. If pH is also less than 7.35 this is respiratory acidosis. Hyperventilation exists when the same ratio decreases – less than 35mmHg. If the pH is also greater than 7.45 this is respiratory alkalosis. See also Alveolar-arterial gradient Diffusing capacity Pulmonary alveolus References Respiratory physiology"}, {"id": "wiki20220301en071_55445", "title": "Metabolic alkalosis", "score": 0.011794033852857383, "content": "To calculate the expected pCO2 in the setting of metabolic alkalosis, the following equations are used: pCO2 = 0.7 [HCO3] + 20 mmHg ± 5 pCO2 = 0.7 [HCO3] + 21 mmHg Treatment To effectively treat metabolic alkalosis, the underlying cause(s) must be corrected. A trial of intravenous chloride-rich fluid is warranted if there is a high index of suspicion for chloride-responsive metabolic alkalosis caused by loss of gastrointestinal fluid (e.g., due to vomiting). Terminology Alkalosis refers to a process by which the pH is increased. Alkalemia refers to a pH which is higher than normal, specifically in the blood. See also Hypokalemia Metabolic acidosis Respiratory acidosis Respiratory alkalosis References External links Acid–base disturbances"}, {"id": "wiki20220301en040_1095", "title": "Metabolic acidosis", "score": 0.011762125954426744, "content": "Diagnostic approach and causes Metabolic Acidosis is defined as a reduced serum pH, and an abnormal serum bicarbonate concentration of <22 mEq/L, below the normal range of 22 to 29 mEq/L. However, if a patient has other coexisting acid-base disorders, the pH level may be low, normal or high in the setting of metabolic acidosis. In the absence of chronic respiratory alkalosis, metabolic acidosis can be clinically diagnosed by measuring serum bicarbonate levels in the blood, which is generally a standard component of blood panels. Imperatively, when weighing a metabolic acidosis diagnosis, the change in serum bicarbonate levels over time should be considered; if baseline bicarbonate results are unknown, a single set of values may be misinterpreted. Causes Generally, metabolic acidosis occurs when the body produces too much acid (e.g., lactic acidosis, see below section), there is a loss of bicarbonate from the blood, or when the kidneys are not removing enough acid from the body."}, {"id": "wiki20220301en040_1128", "title": "Respiratory acidosis", "score": 0.011716664887370557, "content": "Types Respiratory acidosis can be acute or chronic. In acute respiratory acidosis, the PaCO2 is elevated above the upper limit of the reference range (over 6.3 kPa or 45 mm Hg) with an accompanying acidemia (pH <7.36). In chronic respiratory acidosis, the PaCO2 is elevated above the upper limit of the reference range, with a normal blood pH (7.35 to 7.45) or near-normal pH secondary to renal compensation and an elevated serum bicarbonate (HCO3− >30 mEq/L). Causes Acute Acute respiratory acidosis occurs when an abrupt failure of ventilation occurs. This failure in ventilation may be caused by depression of the central respiratory center by cerebral disease or drugs, inability to ventilate adequately due to neuromuscular disease (e.g., myasthenia gravis, amyotrophic lateral sclerosis, Guillain–Barré syndrome, muscular dystrophy), or airway obstruction related to asthma or chronic obstructive pulmonary disease (COPD) exacerbation."}, {"id": "wiki20220301en010_32316", "title": "Respiratory failure", "score": 0.011581797959282363, "content": "Respiratory failure results from inadequate gas exchange by the respiratory system, meaning that the arterial oxygen, carbon dioxide, or both cannot be kept at normal levels. A drop in the oxygen carried in the blood is known as hypoxemia; a rise in arterial carbon dioxide levels is called hypercapnia. Respiratory failure is classified as either Type 1 or Type 2, based on whether there is a high carbon dioxide level, and can be acute or chronic. In clinical trials, the definition of respiratory failure usually includes increased respiratory rate, abnormal blood gases (hypoxemia, hypercapnia, or both), and evidence of increased work of breathing. Respiratory failure causes an altered mental status due to ischemia in the brain. The typical partial pressure reference values are oxygen Pa O2 more than 80 mmHg (11 kPa) and carbon dioxide Pa CO2 less than 45 mmHg (6.0 kPa). Cause"}, {"id": "wiki20220301en009_14170", "title": "Sepsis", "score": 0.011531986531986532, "content": "More specific definitions of end-organ dysfunction exist for SIRS in pediatrics. Cardiovascular dysfunction (after fluid resuscitation with at least 40 ml/kg of crystalloid) hypotension with blood pressure < 5th percentile for age or systolic blood pressure < 2 standard deviations below normal for age, or vasopressor requirement, or two of the following criteria: unexplained metabolic acidosis with base deficit > 5 mEq/l lactic acidosis: serum lactate 2 times the upper limit of normal oliguria (urine output ) prolonged capillary refill > 5 seconds core to peripheral temperature difference Respiratory dysfunction (in the absence of a cyanotic heart defect or a known chronic respiratory disease) the ratio of the arterial partial-pressure of oxygen to the fraction of oxygen in the gases inspired (PaO2/FiO2) < 300 (the definition of acute lung injury), or"}, {"id": "wiki20220301en054_12513", "title": "Respiratory alkalosis", "score": 0.011047979797979798, "content": "Diagnosis The diagnosis of respiratory alkalosis is done via test that measure the oxygen and carbon dioxide levels (in the blood), chest x-ray and a pulmonary function test of the individual. The Davenport diagram allows clinicians or investigators to outline blood bicarbonate concentrations (and blood pH) after a respiratory or metabolic acid-base disturbance Classification There are two types of respiratory alkalosis: chronic and acute as a result of the 3–5 day delay in kidney compensation of the abnormality. Acute respiratory alkalosis occurs rapidly, have a high pH because the response of the kidneys is slow. Chronic respiratory alkalosis is a more long-standing condition, here one finds the kidneys have time to decrease the bicarbonate level. pH Alkalosis refers to the process due to which there is elevation of blood pH. Alkalemia refers to an arterial blood pH of greater than 7.45."}, {"id": "wiki20220301en019_114897", "title": "Acidosis", "score": 0.010960435501117916, "content": "Mutations to the V-ATPase 'a4' or 'B1' isoforms result in distal renal tubular acidosis, a condition that leads to metabolic acidosis, in some cases with sensorineural deafness. Arterial blood gases will indicate low pH, low blood HCO3, and normal or low PaCO2. In addition to arterial blood gas, an anion gap can also differentiate between possible causes. The Henderson-Hasselbalch equation is useful for calculating blood pH, because blood is a buffer solution. In the clinical setting, this equation is usually used to calculate HCO3 from measurements of pH and PaCO2 in arterial blood gases. The amount of metabolic acid accumulating can also be quantitated by using buffer base deviation, a derivative estimate of the metabolic as opposed to the respiratory component. In hypovolemic shock for example, approximately 50% of the metabolic acid accumulation is lactic acid, which disappears as blood flow and oxygen debt are corrected. Treatment"}, {"id": "wiki20220301en074_45829", "title": "Base excess", "score": 0.010927102238354507, "content": "Base excess (or deficit) is one of several values typically reported with arterial blood gas analysis that is derived from other measured data. The term and concept of base excess were first introduced by Poul Astrup and Ole Siggaard-Andersen in 1958. Estimation Base excess can be estimated from the bicarbonate concentration ([HCO3−]) and pH by the equation: with units of mEq/L. The same can be alternatively expressed as Calculations are based on the Henderson-Hasselbalch equation: Ultimately the end result is: Interpretation Base excess beyond the reference range indicates metabolic alkalosis if too high (more than +2 mEq/L) metabolic acidosis if too low (less than −2 mEq/L)"}, {"id": "wiki20220301en267_19616", "title": "Winters' formula", "score": 0.010849002849002849, "content": "Winters' formula, named for Dr. R.W. Winters, is a formula used to evaluate respiratory compensation when analyzing acid–base disorders and a metabolic acidosis is present. It can be given as , where HCO3− is given in units of mEq/L and pCO2 will be in units of mmHg. Winters' formula gives an expected value for the patient's PCO2; the patient's actual (measured) PCO2 is then compared to this: If the two values correspond, respiratory compensation is considered to be adequate. If the measured PCO2 is higher than the calculated value, there is also a secondary respiratory acidosis or mixed acid base disorder. If the measured PCO2 is lower than the calculated value, there is also a secondary respiratory alkalosis or mixed acid base disorder. References Respiratory therapy Mathematics in medicine"}, {"id": "wiki20220301en054_12514", "title": "Respiratory alkalosis", "score": 0.010816326530612244, "content": "pH Alkalosis refers to the process due to which there is elevation of blood pH. Alkalemia refers to an arterial blood pH of greater than 7.45. Treatment Respiratory alkalosis is very rarely life-threatening, though pH level should not be 7.5 or greater. The aim in treatment is to detect the underlying cause. When PaCO2 is adjusted rapidly in individuals with chronic respiratory alkalosis, metabolic acidosis may occur. If the individual is on a mechanical ventilator then preventing hyperventilation is done via monitoring ABG levels. In popular culture In The Andromeda Strain, one of the characters is exposed to contamination, but saves himself by increasing his respiratory rate to induce alkalosis. See also References Further reading External links Acid–base disturbances"}, {"id": "wiki20220301en074_45827", "title": "Base excess", "score": 0.010696710696710698, "content": "In physiology, base excess and base deficit refer to an excess or deficit, respectively, in the amount of base present in the blood. The value is usually reported as a concentration in units of mEq/L (mmol/L), with positive numbers indicating an excess of base and negative a deficit. A typical reference range for base excess is −2 to +2 mEq/L. Comparison of the base excess with the reference range assists in determining whether an acid/base disturbance is caused by a respiratory, metabolic, or mixed metabolic/respiratory problem. While carbon dioxide defines the respiratory component of acid–base balance, base excess defines the metabolic component. Accordingly, measurement of base excess is defined, under a standardized pressure of carbon dioxide, by titrating back to a standardized blood pH of 7.40."}, {"id": "article-17093_17", "title": "Physiology, Acid Base Balance -- Related Testing", "score": 0.010574152072711151, "content": "Arterial blood gas (ABG) sampling, is a test often performed in an inpatient setting to assess the acid-base status of a patient. A needle is used to draw blood from an artery, often the radial, and the blood is analyzed to determine parameters such as the pH, pC02, pO2, HCO3, oxygen saturation, and more. This allows the physician to understand the status of the patient better. ABGs are especially important in the critically ill. They are the main tool utilized in adjusting to the needs of a patient on a ventilator. The following are the most important normal values on an ABG: pH = 7.35 to 7.45 pCO2 = 35 to 45 mmHg pO2 = 75 to 100 mmHg HCO3- = 22 to 26 mEq/L O2 Sat = greater than 95% The ability to quickly and efficiently read an ABG, especially in reference to inpatient medicine, is paramount to quality patient care."}, {"id": "pubmed23n0569_615", "title": "Comparison of arterial and venous blood gases analysis in patients with exacerbation of chronic obstructive pulmonary disease.", "score": 0.010141206675224647, "content": "To investigate whether venous blood gases (VBG) test can be replace by an arterial blood gases (ABG) in exacerbation of chronic obstructive pulmonary disease (COPD). From October 2005 to March 2006, at the Emergency Room of Kashan Beheshti Hospital, the data of 107 patients with exacerbation of COPD were assessed. Arterial blood gases and VBG samples were obtained simultaneously, and indexes of pH, carbon dioxide partial pressure (PCO2), bicarbonate (HCO3), oxygen partial pressure (PO2) and Oxygen (O2) saturation level were analyzed. The mean +/- SD of indexes in ABG and VBG samples were as follows: pH = 7.37 +/- 0.47 versus 7.34 +/- 0.047; PCO2 = 53.88 +/- 7.63 mm Hg versus 59.55 +/- 8.96 mm Hg, HCO3= 30.66 +/- 4.49 mEq/L versus 31.94 +/- 4.39 mEq/L; PO2 = 55.37 +/- 11.19 mm Hg versus 43.08 +/- 10.54 mm Hg. The average difference between indexes in ABG and VBG samples were as follows: pH = 0.0241 +/- 0.004, p&lt;0.001, r = 0.864; PCO2 = 5.673 +/- 1.126 mm Hg, p&lt;0.001, r = 0.761; HCO3 = 1.279 +/- 0.604 mEq/L, p&lt;0.001, r = 0.749; and PO2 = 12.294 +/- 2.115 mm Hg, p&lt;0.001, r = 0.702. Venous blood gases, especially pH and PCO2 levels have relatively good correlation with ABG values. In view of the fact that, this correlation is not close, VBG cannot be substitute for ABG in exacerbation of COPD."}]}}}}
{"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 160]], "word_ranges": [[0, 22]], "text": "We are presented with a case of RSV-positive bronchiolitis. According to current recommendations, it would be indicated to initiate supplemental oxygen therapy."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "We are presented with a case of RSV-positive bronchiolitis. According to current recommendations, it would be indicated to initiate supplemental oxygen therapy.", "full_answer_no_ref": "We are presented with a case of RSV-positive bronchiolitis. According to current recommendations, it would be indicated to initiate supplemental oxygen therapy.", "full_question": "A 2-month-old infant with an upper respiratory tract cold of 3 days of evolution, who begins with moderate respiratory distress and pulmonary auscultation with expiratory wheezing. Oxygen saturation is 89 %. Respiratory syncytial virus is isolated in the nasopharyngeal exudate. Which of the following treatments do you consider more indicated for this condition?", "id": 594, "lang": "en", "options": {"1": "Oral rivabirin.", "2": "Nebulized salbutamol.", "3": "Supplemental oxygen.", "4": "Intravenous corticosteroids.", "5": NaN}, "question_id_specific": 80, "type": "PEDIATRICS", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0257_18321", "title": "[Comparative study of nebulized sambutol against placebo in the acute phase of bronchiolitis in 33 infants aged 1 to 6 months].", "score": 0.01827849783981389, "content": "The therapeutic role of bronchodilators in bronchiolitis remains controversial. The aim of this study is to evaluate the safety and the clinical response to nebulized salbutamol in infants with mild acute bronchiolitis. Thirty-three infants, aged 1 month to 5 months and 22 days (mean: 92.4 days) were included in the study. Patients received either nebulized salbutamol (0.15 mg/kg per dose: 16 infants) or a placebo (normal saline aerosol: 17 infants), delivered by an oxygen propellent, three times at intervals of 1 hour, as part of a double-blind randomized trial. Effect of treatment was evaluated by measuring respiratory and heart rate, clinical scores based on the degree of retraction and wheezing, and oxygen saturation. Clinical assessment was repeated 30 minutes after each nebulization. A nasopharyngeal swab was obtained for detection of respiratory syncytial virus (VRS) antigens by immunofluorescence assay in all patients. Patients in the salbutamol group exhibited significantly greater improvement in respiratory rate (P = 0.01), accessory muscle score (P &lt; 0.001) and wheezing score (P &lt; 0.001). There was no significant difference in oxygen saturation between both groups. Infants treated with salbutamol exhibited a non-significant increase in heart rate after the three sprays; no other adverse effects were noted. VRS was identified in 78% of the children tested. Salbutamol is safe and effective in relieving the respiratory distress of young infants with acute bronchiolitis. Our study confirms previous observations that infants younger than six months of age respond as well as older children when given three doses of nebulized salbutamol. Responders could not be differentiated from non responders by personal or family histories of atopy and VRS isolation. A longitudinal study could establish a correlation between response to bronchodilator therapy and later development of asthma."}, {"id": "pubmed23n0893_7155", "title": "Respiratory Syncytial Virus Bronchiolitis in Children.", "score": 0.017216117216117217, "content": "Bronchiolitis is a common lower respiratory tract infection in infants and young children, and respiratory syncytial virus (RSV) is the most common cause of this infection. RSV is transmitted through contact with respiratory droplets either directly from an infected person or self-inoculation by contaminated secretions on surfaces. Patients with RSV bronchiolitis usually present with two to four days of upper respiratory tract symptoms such as fever, rhinorrhea, and congestion, followed by lower respiratory tract symptoms such as increasing cough, wheezing, and increased respiratory effort. In 2014, the American Academy of Pediatrics updated its clinical practice guideline for diagnosis and management of RSV bronchiolitis to minimize unnecessary diagnostic testing and interventions. Bronchiolitis remains a clinical diagnosis, and diagnostic testing is not routinely recommended. Treatment of RSV infection is mainly supportive, and modalities such as bronchodilators, epinephrine, corticosteroids, hypertonic saline, and antibiotics are generally not useful. Evidence supports using supplemental oxygen to maintain adequate oxygen saturation; however, continuous pulse oximetry is no longer required. The other mainstay of therapy is intravenous or nasogastric administration of fluids for infants who cannot maintain their hydration status with oral fluid intake. Educating parents on reducing the risk of infection is one of the most important things a physician can do to help prevent RSV infection, especially early in life. Children at risk of severe lower respiratory tract infection should receive immunoprophylaxis with palivizumab, a humanized monoclonal antibody, in up to five monthly doses. Prophylaxis guidelines are restricted to infants born before 29 weeks' gestation, infants with chronic lung disease of prematurity, and infants and children with hemodynamically significant heart disease."}, {"id": "pubmed23n1122_14232", "title": "Childhood Respiratory Conditions: Lower Respiratory Tract Infection.", "score": 0.01652780600149021, "content": "Bronchiolitis, a lower respiratory tract infection, commonly affects infants and children younger than 2 years. Respiratory syncytial virus is the most common cause. Nasal congestion, rhinorrhea, and mild fever occur in the first 1 to 3 days. Symptoms worsen for several days to include wheezing and other lower respiratory tract signs, and then resolve over days to weeks. However, some children develop hypoxemia and/or respiratory distress and require hospitalization. The diagnosis is clinical, based on the history and physical examination findings. Routine chest x-rays and blood tests are not recommended. Management is supportive, including nasal suctioning, oxygen (in cases of hypoxemia), and hydration. Use of bronchodilators (ie, albuterol, epinephrine) and systemic corticosteroids is not recommended in infants and children ages 1 to 23 months. Pneumonia, another common infection in infants and children, typically is caused by viruses or bacteria. The diagnosis is clinical, and chest x-rays and blood cultures are not required for patients well enough to be treated as outpatients. Antibiotics are not routinely required for preschool-aged children (ie, younger than 5 years) with pneumonia because most have viral infections. For immunized school-aged children with mild to moderate pneumonia suspected to be of bacterial origin, amoxicillin is recommended. In school-aged children, if atypical pathogens (eg, Mycoplasma) are suspected, a macrolide antibiotic should be prescribed."}, {"id": "pubmed23n0289_7643", "title": "[An epidemic of respiratory syncytial virus bronchiolitis among infants in northern Israel].", "score": 0.016057203788496517, "content": "In the wake of a community outbreak of bronchiolitis in northern Israel from December 1993 to March 1994, we conducted a retrospective study of 108 infants aged 2 weeks to 14 months with proven respiratory syncytial virus (RSV) infection (diagnosed by a rapid RSV antigen test in nasopharyngeal secretions). 47% of the infants were less than 8 weeks old. Mean hospital stay was 6.6 days (range 1-60). The characteristic clinical findings were: cough in all patients, dyspnea in 96%, rhinitis in 95% and fever in 55%. In those younger than 8 weeks, or in those with underlying diseases, hospitalization was longer, the disease was more serious and complications more frequent (p &lt; 0.002). 4 children (3%) died, 3 of whom had severe congenital heart defects. All children were treated with oxygen and beta-agonist inhalations. The 33% who also received corticosteroids were older and most had a history of pulmonary diseases, such as asthma or bronchopulmonary dysplasia. There was no difference between those who did or did not receive corticosteroids with regard to severity of disease or rate of complications. Ribavirin was used to treat 19 (17.5%), most of whom had underlying lung disease. The others were otherwise healthy infants younger than 8 weeks. 13 were cared for in the intensive care unit, 11 of whom required mechanical ventilation. A rapid test for detection of RSV infection enabled prompt isolation of infected patients so that the risk of nosocomial infection was reduced and Ribavirin therapy could be started early, if required."}, {"id": "pubmed23n0052_3365", "title": "The efficacy of nebulized metaproterenol in wheezing infants and young children.", "score": 0.015639481000926783, "content": "The benefit of beta-adrenergic agonists in the treatment of acutely wheezing infants and young children has not been well documented in the outpatient setting. To determine the efficacy of nebulized metaproterenol sulfate, 74 children aged 36 months or younger with acute wheezing participated in a double-masked, randomized, placebo-controlled clinical trial. Children received nebulized metaproterenol, either as an initial treatment or after a control treatment with normal saline solution. At baseline and 20 minutes after each treatment, an assessment was made that included measurements of heart rate, respiratory rate, oxygen saturation, and clinical variables related to respiratory compromise with the use of a standardized respiratory distress index (RDI). Children who received saline solution as initial therapy had no significant differences from baseline in any of the assessment measures. After metaproterenol therapy, children demonstrated an increase in heart rate ([mean +/- SD] 147 +/- 14 beats per minute vs 153 +/- 16 beats per minute), a decrease in respirations (50/min +/- 5/min vs 45/min +/- 7/min), improvement (lower scores) on the RDI (24 +/- 4 vs 15 +/- 2), and an increase in oxygen saturation (94.1% +/- 2.7% vs 95.3% +/- 3.0%). Patients aged 12 months or younger (n = 37) benefited from metaproterenol treatment (improvement in respiratory rate and RDI) but not to the same degree as children aged 24 months or older (n = 23) (improvement in respiratory rate, RDI, and oxygen saturation). Compared with assessments made before metaproterenol treatment, patients with respiratory syncytial virus infection (n = 21) had improvement in respirations (52/min +/- 7/min vs 45/min +/- 6/min) and RDI scores (22 +/- 4 vs 14 +/- 3). Based on a priori criteria (reduction in a premedication respiratory rate of 20% and an RDI score of 50%), responders to metaproterenol therapy included 45% of the entire sample and, respectively, 40% of those aged 12 months or younger, 52% of those aged 24 months or older, and 48% of patients who tested positive for respiratory syncytial virus. Although there appears to be an age-dependent degree of response, metaproterenol is effective in relieving the respiratory distress of young acutely wheezing children, including those with documented respiratory syncytial virus bronchiolitis."}, {"id": "pubmed23n0272_8356", "title": "Bronchiolitis.", "score": 0.01496621432959178, "content": "Bronchiolitis, a lower respiratory tract illness most often caused by respiratory syncytial virus, generally affects children under two years of age, commonly during the winter months. Necrosis of epithelial cells in the small airways leads to inflammation and airway obstruction, causing decreased oxygen saturation, with cough and wheezing. Hospital admission should be considered for children with pulse oximetry levels less than 95 percent at rest. Treatment consists of humidified oxygen, intravenous hydration and administration of nebulized albuterol. Infants with mild disease who are identified early in the course of illness should be reevaluated in 24 hours. Infants with congenital heart disease, bronchopulmonary dysplasia or a history of prematurity, who are at high risk for severe disease, should be treated with ribavirin."}, {"id": "pubmed23n0262_12236", "title": "Predictors of bronchodilator responsiveness in infants with wheezing associated respiratory tract infection.", "score": 0.014906832298136646, "content": "The responsiveness to bronchodilator is variable in infants with wheezing associated respiratory illness (WARI). Factors for prediction of the response will lead to more rational use of the bronchodilator in these infants. We examined the possible predictive factors in 44 children under 2 years of age who had their first episode of WARI. All of them were treated with 0.15 mg/kg of nebulized salbutamol. Thirty patients (68%) with decreasing clinical score &gt; or = 3 after treatment were considered as the responders while the remainder (14 infants) were non responders. By using Chi-square test, Fisher exact test and Mann-Whitney U test to compare the data of the 2 groups, the significant factors for the responders were older than 6 months and history of previous LRI (p &lt; 0.01). The significant factors for the non-responders included concurrent diarrhea, patchy pulmonary infiltration and positive RSV in the nasopharyngeal secretion (p &lt; 0.01). These results suggested effective bronchodilator therapy in infants older than 6 months or having a history of previous LRI. Those who had acute RSV infection or patchy infiltration in chest X-ray and associated diarrhea were less likely to respond."}, {"id": "pubmed23n0357_12098", "title": "Lower airway disease caused by respiratory syncytial virus.", "score": 0.014875027648750276, "content": "Respiratory syncytial virus (RSV) is the most common cause of lower respiratory tract disease in infants and young children. Most infections due to RSV are mild and do not require hospitalization. RSV causes both upper respiratory tract infections as well as lower respiratory tract infections. Infants with underlying disease states like bronchopulmonary dyslasia, congenital heart disease and prematurity appear more prone to develop severe infection and have a higher incidence of hospitalization. The exact pathogenesis of RSV is not well understood. The mortality associated with primary RSV infection in healthy children is estimated to be between .005% to .02%. In hospitalized children the mortality rate is estimated to be from 1% to 3%. Several treatment modalities in the form of bronchodilators, corticosteroids, ribavirin, intravenous immune gammaglobulin and antibiotics are available. Studies have failed to show the true beneficial effect of any of the above treatment modalities. Supportive care is only needed. The best treatment is the supportive care in the form of oxygen and fluids and close monitoring of the vital signs including oxygen saturation."}, {"id": "pubmed23n0324_14103", "title": "Efficacy of corticosteroids in acute bronchiolitis: short-term and long-term follow-up.", "score": 0.014681977872161921, "content": "Corticosteroids continue to be used by many physicians to treat infants with bronchiolitis. The aim of this study was to examine the short-term and long-term efficacy of oral corticosteroid therapy when added to beta2-agonists in infants with mild to moderate bronchiolitis (defined as the first episode of wheezing associated with low grade fever, rhinitis, tachypnea, and increased respiratory effort in a previously healthy infant during the winter months). Infants with mild to moderate bronchiolitis, were randomly assigned to receive either oral prednisone (2 mg/kg/day) or placebo for 3 days. All patients received nebulized albuterol q.i.d. during this period. Upon admission and after 3 days of therapy, a clinical score was assigned based on respiratory rate, use of accessory muscle, and the presence of wheeze. Oxygen saturation (SaO2) was also measured. On day 7, we inquired as to the well-being of each child. Two years later, the development of chronic respiratory symptoms was assessed. Thirty-eight infants were enrolled in the study; 20 received prednisone and 18 received placebo. Both groups were similar in terms of age, duration of illness prior to enrollment, pretrial medication use, clinical severity of bronchiolitis, history of atopy, and family history of atopy. After 3 and 7 days of treatment, both groups showed similar clinical improvement and there were no statistically significant differences between the two groups in the clinical score or in the SaO2. No major side effects were observed. Two years later, 32% of the infants continued to suffer from chronic respiratory symptoms, with a similar prevalence in both groups. We conclude that a 3-day course of oral corticosteroids is of no benefit to infants with mild to moderate bronchiolitis who are also treated with an inhaled beta2-agonist."}, {"id": "pubmed23n0529_13547", "title": "Respiratory syncytial virus infections: characteristics and treatment.", "score": 0.014666029013231309, "content": "In this review, we describe the history, epidemiology and clinical manifestations of infections attributed to respiratory syncytial virus (RSV) in children. At present, no cure exists for RSV infection but commonly employed palliative treatments include oxygen and inhaled beta(2)-adrenoceptor agonists, such as salbutamol, to relieve the wheezing and increased bronchiolar smooth muscle constriction. Adrenaline (epinephrine) has been found to be superior to the selective beta(2)-adrenoceptor agonists. Oral or inhaled corticosteroids should counteract the inflammatory response to RSV infection but their effectiveness is controversial. Inhaled ribavirin is the only licensed antiviral product approved for the treatment of RSV lower respiratory-tract infection in hospitalized children, although its use is now restricted to high-risk infants. Other treatments considered are nasopharyngeal suctioning, surfactant therapy, recombinant human deoxyribonuclease I, heliox (helium:oxygen) and inhaled nitric oxide. Prevention of infection by RSV antibodies is another strategy and, currently, palivizumab is the only safe, effective and convenient preventative treatment for RSV disease in high-risk populations of infants and young children. Its cost-effectiveness, however, has been questioned. Both live attenuated and subunit vaccines against RSV infection have been developed but so far there is no safe and effective vaccine available. Finding effective treatments and prophylactic measures remains a major challenge for the future."}, {"id": "pubmed23n0544_8255", "title": "Evaluation of the efficacy of prednisolone in early wheezing induced by rhinovirus or respiratory syncytial virus.", "score": 0.014423076923076924, "content": "The role of systemic corticosteroids in the treatment of early childhood wheezing in children is not clear. We sought to determine whether prednisolone is effective in rhinovirus-induced early wheezing. We conducted a controlled trial comparing oral prednisolone (2 mg/kg per day in three divided doses for 3 days) with placebo in 78 hospitalized children (mean age, 1.1 year; standard deviation, 0.7) experiencing their first or second episode of wheezing induced by rhinovirus or respiratory syncytial virus. Mixed viral infections were excluded. Our primary end point was the time until the patient was ready for discharge; secondary end points included oxygen saturation during hospitalization, duration of symptoms, occurrence of relapses during the next 2 months and blood eosinophil counts at discharge and 2 weeks later. In multivariate regression analysis, prednisolone did not influence the time until ready for discharge, but it decreased relapses during the subsequent 2-month period in rhinovirus-affected children (prednisolone versus placebo, 22% versus 56%; odds ratio, 19.06; 95% confidence interval, 2.52-144.03; P = 0.004) and in children with blood eosinophils &gt; or = 0.2 x 10/L (respectively, 24% versus 71%; odds ratio, 10.57; 95% confidence interval, 1.99-56.22; P = 0.006). Rhinovirus-affected children had more blood eosinophils on admission (mean, 0.44 versus 0.086 x 10/L), had a higher prevalence of atopy (44% versus 8%) and were older (mean, 1.4 versus 0.9 years, P &lt; 0.001 for all) than respiratory syncytial virus-infected children. Prednisolone reduced relapses during a 2-month period after first episodes of wheezing associated with rhinovirus infection or blood eosinophils &gt; or = 0.2 x 10/L."}, {"id": "wiki20220301en019_64025", "title": "Respiratory syncytial virus", "score": 0.014021164021164021, "content": "Treatment for severe illness is primarily supportive, including oxygen therapy and more advanced breathing support with CPAP or nasal high flow oxygen, as required. In cases of severe respiratory failure, intubation and mechanical ventilation may be required. Ribavirin is the only antiviral medication currently licensed for the treatment of RSV in children, although its use remains controversial. Signs and symptoms RSV infection can present with a wide variety of signs and symptoms that range from mild upper respiratory tract infections (URTI) to severe and potentially life-threatening lower respiratory tract infections (LRTI) requiring hospitalization and mechanical ventilation. While RSV can cause respiratory tract infections in people of all ages and is among the most common childhood infections, its presentation often varies between age groups and immune status. Reinfection is common throughout life, but infants and the elderly remain at highest risk for symptomatic infection."}, {"id": "wiki20220301en019_64027", "title": "Respiratory syncytial virus", "score": 0.013900990099009901, "content": "Bronchiolitis is a common lower respiratory tract infection characterized by inflammation and obstruction of the small airways in the lungs. While several viruses can cause bronchiolitis, RSV is responsible for about 70% of cases. It usually presents with 2 to 4 days of runny nose and congestion followed by worsening cough, noisy breathing, tachypnea (fast breathing), and wheezing. As infants work harder to breathe, they can also show signs of respiratory distress, such as subcostal retractions (when the belly pulls under the ribcage), intercostal retractions (when the muscles between the ribs pull inward), grunting, and nasal flaring. If the child has not been able to feed adequately, signs of dehydration may also be present. Fever may be present, but high-grade fever is uncommon. Crackles and wheezing can often be heard on auscultation, and oxygen saturation levels may be decreased."}, {"id": "wiki20220301en019_64029", "title": "Respiratory syncytial virus", "score": 0.01365613922010957, "content": "Adults Reinfection with RSV remains common throughout life. Reinfection in adulthood often produces only mild to moderate symptoms indistinguishable from the common cold or sinus infection. Infection may also be asymptomatic. If present, symptoms are generally isolated to the upper respiratory tract: runny nose, sore throat, fever, and malaise. In the vast majority of cases, nasal congestion precedes the development of cough. In contrast to other upper respiratory infections, RSV is also more likely to cause new onset wheeze in adults. Only about 25% of infected adults will progress to significant lower respiratory tract infection, such as bronchitis or tracheobronchitis."}, {"id": "wiki20220301en019_64057", "title": "Respiratory syncytial virus", "score": 0.01311502117953731, "content": "Treatment Supportive care Treatment for RSV infection is focused primarily on supportive care. This may include monitoring a patient's breathing or using suction to remove secretions from the upper airway. Supplemental oxygen may also be delivered though a nasal cannula or face mask in order to improve airflow. In severe cases of respiratory failure, intubation and mechanical ventilation may be required to support breathing. If signs of dehydration are present, fluids may also be given orally or through an IV."}, {"id": "pubmed23n0346_6837", "title": "Management strategies for respiratory syncytial virus infections in infants.", "score": 0.01298750188224665, "content": "Management of respiratory syncytial virus lower respiratory tract infection in infants is predominantly supportive and symptomatic. Outpatient management requires close attention to feeding, oral hydration, and monitoring of fever, behavior, and respiratory effort. In the small proportion of patients who need hospitalization, clinicians are concerned primarily with oxygenation and the possibility of oxygen desaturation. Appropriate interventions depend on the level of oxygenation as indicated by pulse oximetry or arterial blood gases. Hydration and symptomatic treatment with alpha- or ss(2 )-adrenergic agonist bronchodilators may lessen the work of breathing. The role of these agents remains controversial, however, and anticholinergic bronchodilators are considered ineffective. Current antiviral therapy is limited to aerosolized ribavirin. Immunotherapy with respiratory syncytial virus immune globulin or a monoclonal antibody has not been rewarding in terms of clinical outcome, although the antiviral effect of these agents has been impressive. There is concern about long-term pulmonary sequelae after respiratory syncytial virus lower respiratory tract infection early in life. Several recent studies, including new data reported here, suggest that ribavirin may have a beneficial effect on some of these sequelae, whereas other studies have failed to demonstrate any benefit. Future studies may help resolve this question."}, {"id": "pubmed23n0417_19985", "title": "[Respiratory syncytial infections in the elderly. Seven cases and review of the literature].", "score": 0.012983277866798142, "content": "We report 7 cases of respiratory syncytial virus (RSV) infections among elderly patients hospitalized for acute lower tract infection during the winter months. The median age was 95 years (range: 79-106 years). 6 patients were living in nursing homes. All patients had chronic cardiopulmonary conditions. Clinical symptoms included upper respiratory tract symptoms for 3 of them and lower respiratory tract symptoms for all of them. Three patients developed a severe infection with acute respiratory failure. The chest X ray showed an interstitial infiltrate in 3 cases, a consolidation in one case. All patients received oxygen, respiratory physical therapy, nebulized B-agonists and antibiotics. Corticosteroids were used for 4 patients. Progress was favorable for all patients and none died. Diagnosis was obtained in a few hours by direct antigen detection by enzyme immunoassays (EIA) in sputum. Respiratory syncytial virus infection is a common cause of respiratory tract infection in the elderly, during the winter months. Clinical manifestations are similar to influenza infections. Detection of RSV antigens from sputum specimens may be a useful and rapid diagnostic method and merits further evaluation in the elderly. Early diagnosis of respiratory syncytial virus infection should be encouraged to allow the implementation of effective infection control procedures and avoid inadequate therapies."}, {"id": "pubmed23n0401_18056", "title": "Management of bronchiolitis: current practices in Ireland.", "score": 0.01232741617357002, "content": "To establish current practice for hospital-based treatment of uncomplicated respiratory syncytial virus (RSV) infection in the Republic of Ireland. A questionnaire was sent to all consultant general paediatricians in the Republic of Ireland. The questionnaire described a clinical scenario and this was followed by a list of management questions. The scenario was of a 3-month-old infant with uncomplicated but moderately severe RSV infection requiring hospitalization. Seventy-three questionnaires were sent. 63/73 (86%) of the questionnaires were returned. With respect to management of this case almost all (61/63) the paediatricians felt that oxygen therapy was necessary (oxygen saturation described in the case was 90%). With respect to bronchodilator therapy, ipratropium bromide (38/63--60%) was chosen much more frequently than salbutamol (15/63--24%). Chest physiotherapy would have been prescribed by 8/63--13% of paediatricians. Oral steroids were infrequently chosen (1/63--2%) but nebulised steroids were selected in 7/63 (11%) cases. The routine use of RSV monoclonal antibody, palivizumab, for RSV prophylaxis was reported by 49% (31/63) of paediatricians. Prematurity with bronchopulmonary dysplasia was considered an indication for its administration by all of these but only 23% considered prematurity alone to be an indication. The management of infants with RSV bronchiolitis varies greatly among consultant paediatricians in Ireland. Evidence based guidelines may be of value in establishing a more uniform national treatment approach."}, {"id": "wiki20220301en019_64058", "title": "Respiratory syncytial virus", "score": 0.011834632148989565, "content": "Additional supportive treatments have been investigated specifically in infants hospitalized with RSV bronchiolitis. These include the following: Nebulized hypertonic saline has been shown to reduce length of hospitalization and reduce clinical severity in infants with viral bronchiolitis. It is thought that this treatment may help by reducing airway edema and mucus plugging to decrease airway obstruction. Heliox, a mixture of oxygen with helium, may improve respiratory distress within the first hour after starting treatment. It works by reducing airway resistance and decreasing the work of breathing. However, it has not been shown to impact overall illness outcomes. Chest physiotherapy has not been found to reduce disease severity and is not routinely recommended. Inhaled recombinant human deoxyribonuclease (rhDNase), an enzyme that digests the DNA that contributes to mucus plugging and airway obstruction, has not been shown to improve clinical outcomes in this group."}, {"id": "First_Aid_Step2_940", "title": "First_Aid_Step2", "score": 0.011302311373587212, "content": "Treatment is primarily supportive; treat mild disease with outpatient management using fluids and nebulizers if needed. Hospitalize in the setting of marked respiratory distress, O2 saturation of < 92%, toxic appearance, dehydration/poor oral feeding, a history of prematurity (< 34 weeks), age < 3 months, underlying cardiopulmonary disease, or unreliable parents. Treat inpatients with contact isolation, hydration, and O2. A trial of aerosolized albuterol may be attempted; continue albuterol therapy only if effective. RSV is the most common cause of bronchiolitis. Ribavirin is an antiviral drug that has a controversial role in bronchiolitis treatment. It is sometimes used in high-risk infants with underlying heart, lung, or immune disease. RSV prophylaxis with injectable polyor monoclonal antibodies (RespiGam or Synagis) is recommended in winter for high-risk patients ≤ 2 years of age (e.g., those with a history of prematurity, chronic lung disease, or congenital heart disease)."}, {"id": "wiki20220301en002_60432", "title": "Asthma", "score": 0.01103212102821969, "content": "Humidified Oxygen to alleviate hypoxia if saturations fall below 92%. Corticosteroid by mouth are recommended with five days of prednisone being the same 2 days of dexamethasone. One review recommended a seven-day course of steroids. Magnesium sulfate intravenous treatment increases bronchodilation when used in addition to other treatment in moderate severe acute asthma attacks. In adults intravenous treatment results in a reduction of hospital admissions. Low levels of evidence suggest that inhaled (nebulised) magnesium sulfate may have a small benefit for treating acute asthma in adults. Overall, high quality evidence do not indicate a large benefit for combining magnesium sulfate with standard inhaled treatments for adults with asthma. Heliox, a mixture of helium and oxygen, may also be considered in severe unresponsive cases. Intravenous salbutamol is not supported by available evidence and is thus used only in extreme cases."}, {"id": "pubmed23n0364_6565", "title": "Inhaled corticosteroids during and after respiratory syncytial virus-bronchiolitis may decrease subsequent asthma.", "score": 0.010509031198686371, "content": "Respiratory syncytial virus (RSV) bronchiolitis in infancy can lead to bronchial hyper-reactivity or recurrent obstructive bronchitis. The aim of the present study was to determine whether the type of treatment has an influence on respiratory status after RSV bronchiolitis. The study involved 117 infants (mean age 2.6 months), who needed hospital treatment because of RSV bronchiolitis. The patients were divided randomly into three groups. All received the same symptomatic treatment. Group I children received symptomatic treatment only, group II children were treated for 7 days with inhaled budesonide, 500 microg three times per day, administered via a nebulizer. Group III children received nebulized budesonide, 500 microg twice per day for two months. Follow-up consisted of out-patient check-ups 2 and 6 months after the infection, and telephone contact two years after the infection. Statistically significant differences were seen between the groups. In group I 37% of the children had asthma, in group II 18%, and in group III 12%. According to the present study it seems that inhaled corticosteroid treatment during and after the acute phase of infant RSV bronchiolitis may have a beneficial effect on subsequent bronchial wheezing tendency."}, {"id": "pubmed23n0260_19949", "title": "Seasonal respiratory viral infections. Impact on infants with chronic lung disease following discharge from the neonatal intensive care unit.", "score": 0.010248028227480282, "content": "To determine the frequency and severity of acute respiratory infections in infants with bronchopulmonary dysplasia following discharge from the neonatal intensive care unit. A prospective cohort study of 30 oxygen-dependent children who were younger than 2 years (mean age, 9.8 months; range, 3 to 24 months) were studied from September 1990 through April 1991. During the study, 101 (90.2%) of 112 visits for illness were prompted by new or worsening respiratory symptoms. Diagnoses included upper respiratory tract infection (30.4%), otitis media (26.0%), pneumonia (11.1%), acute exacerbation of bronchopulmonary dysplasia (10.4%), reactive airway disease (9.6%), and bronchiolitis (5.9%). Among these children, an increase in the fraction of inspired oxygen was necessary during 43% of visits. Ten children were hospitalized on 25 occasions for a mean of 37.6 hospital days per child (range, 1 to 107 days), and mean length of stay for each hospitalization was 15 days (median, 6 days). Five children were admitted to the pediatric intensive care unit. Respiratory viruses isolated included respiratory syncytial virus (n = 7), parainfluenza 3 virus (n = 3), and adenovirus (n = 2). No isolates of influenza A or B were detected. Anthropometrics at study entry and study end were converted to z scores as descriptors of weight for age, height for age, and weight for height. Growth improved during the 8 months of the study; however, overall, the children were leaner at study end than at study entry. In children with bronchopulmonary dysplasia, respiratory viral infections led to significant morbidity, which included long and frequent hospitalizations during the peak of the respiratory viral season. Although weight and linear growth increased throughout the study, patients were leaner at study conclusion than at study entry."}, {"id": "pubmed23n0403_20455", "title": "The impact of respiratory syncytial virus infection: a prospective study in hospitalized infants younger than 2 years.", "score": 0.010049633888643175, "content": "We analyzed the influence of respiratory syncytial virus (RSV) on the clinical course and management of infants hospitalized due to viral upper and lower respiratory tract infections (U/LRTI). Infants younger than 2 years were prospectively tested for RSV infection by antigen detection in nasopharyngeal aspirates between November 1999 and October 2000. Of 281 infants hospitalized during the study period, 58 (21%) tested RSV positive. Seasonal distribution of RSV infections showed a peak in March (45% of all U/LRTI). Infants with RSV infection (12% were preterm, 5% had congenital heart disease) were younger (p &lt; 0.001), had more severe U/LRTI (p &lt; 0.001), longer hospitalizations (p &lt; 0.001), more days with oxygen requirement (p &lt; 0.001) and respiratory support (p = 0016) and more frequent requirements for bronchodilators (p = 0.002) and corticosteroids (p = 0.02). RSV contributed to prolonged hospitalizations and more severe clinical courses of disease both in very young term and preterm infants."}, {"id": "pubmed23n0339_9624", "title": "[Is the use of ribavirin aerosols in respiratory syncytial virus infections justified? Clinical and economic evaluation].", "score": 0.009900990099009901, "content": "The controversy about the use of ribavirin aerosol for children at risk (cardiopathy, pneumopathy, premature and immunodeficient patients), in case of respiratory syncytial virus (RSV) infection, led us to stop its prescription in 1993 and study prospectively the patients admitted during the following winters. Criterias of inclusion for this study were those of the Committee on Infectious Diseases of the American Academy of Pediatrics concerning the use of ribavirin aerosol. Two cohorts of patients were studied: the first group included treated patients (ribavirin group: n = 22, ribavirin and support treatment: salbutamol aerosols, respiratory physiotherapy and oxygen-therapy; winters 1989-1990 to 1992-1993); the second group included patients with support treatment only (control group: n = 22; winters 1993-1994 and 1994-1995). The clinical gravity score at admission (4.55 vs 5.23, P = 0.46) and the risk factor scores (3.05 vs 3.27, P = 0.69) of the two groups were identical. Results showed that the children of the ribavirin group stayed much longer in hospital (14.2 vs 8.2 days, P = 0.002) and in the intensive care unit (7.2 vs 0.2 days, P &lt; 0.001) than those of the control group. More support treatment was necessary for the ribavirin group as regard respiratory physiotherapy (3.8 vs 2.7 sessions a day, P = 0.026), the duration of oxygen-therapy (7.3 vs 3.7 days, P = 0.030) and the number of children requiring respiratory assistance (4 vs 0 children, P = 0.116). Administration of ribavirin aerosols (480 US$ a dose) and the way in which such treatment was carried out meant high daily costs for the ribavirin group (1,076 vs 604 US$, P &lt; 0.001). As hospitalization was longer for children treated with ribavirin, the global cost was therefore much higher (15,552 vs 5,156 US$, P &lt; 0.001). The antiviral effect of ribavirin is undeniable. However ribavirin is known to be the cause of severe bronchospasms (two cases in our study) and can also cause moderate and long term bronchospasms, aggravating therefore the clinical evolution of the disease. Our experience shows that administration of ribavirin aerosols in case of RSV infection of inferior respiratory airways seems not to be justified."}, {"id": "pubmed23n0368_13630", "title": "[Prednisolone treatment of respiratory syncytial virus infection. A randomized, controlled trial of 147 children].", "score": 0.00980392156862745, "content": "Our objective was to evaluate the effect of systemic prednisolone as an adjunct to conventional treatment with beta 2-agonist, fluid replacement and respiratory support in hospitalized infants younger than 24 months with respiratory syncytial virus (RSV) infection. The study was randomized, double-blind and placebo-controlled. During the winter of 1995-96, 147 infants less than two years of age hospitalized with RSV infection were allocated to treatment with either systemic prednisolone mixture 2 mg/kg daily or placebo for 5 days. Our main outcome measures were: 1. Acute effect variables: duration of stay in hospital, use of medicine and supportive measures while in hospital. 2. At follow-up one month after discharge: duration of illness, start in day care center, morbidity and use of medicine. 3. At follow-up one year after discharge: morbidity, use of medicine and skin prick tests with allergens. Prednisolone treatment had no effect on any of the outcome measures. We find our results in agreement with the largest studies reported earlier; therefore, corticosteroid, whether by systemic route or by inhalation, should not be prescribed to infants with RSV infection."}, {"id": "pubmed23n0894_14746", "title": "[A case of bronchiolitis obliterans secondary to human metapneumovirus bronchiolitis].", "score": 0.009708737864077669, "content": "Human metapneumovirus (hMPV), formerly classified in Paramyxoviridae family is now moved into Pneumoviridae, which was described as a novel family. It causes upper and lower respiratory tract infections (LRTIs) usually in children younger than five years old. The recent epidemiological studies indicated that hMPV is the second most frequently detected virus in LRTIs of young children, following the respiratory syncytial virus (RSV). Bronchiolitis obliterans (BO) is a chronic obstructive lung disease characterized by fibrosis of the distal respiratory airways. It is usually a result of an inflammatory process triggered by a LRTI related to adenovirus, RSV, Mycoplasma pneumoniae, measles virus, Legionella pneumophila, influenza virus or Bordetella pertussis as a causative agent. In this report, a case of hMPV bronchiolitis complicated with BO has been reported to point out the complications and severity of the clinical progress belongs to this virus. A three-month-old female patient has admitted to our pediatric intensive care unit with the diagnosis of acute bronchiolitis and respiratory failure. She was born at term, weighing 2950 gram and had been hospitalized in newborn intensive care unit for 11 days with the diagnosis of transient tachypnea of the newborn and neonatal sepsis. On auscultation, there were bilateral crepitant rales, wheezing and prolonged expirium. Her oxygen saturation was 97-98% while respiratory support was given with a non-rebreathing reservoir mask. Complete blood count, procalcitonin and C-reactive protein levels were in normal ranges. The chest radiography yielded right middle lobe atalectasia, left paracardiac infiltration and bilateral air trapping. A nasopharyngeal swab sample was analyzed by a commercial multiplex real-time reverse transcriptase-polymerase chain reaction (Thermo Fisher Scientific®, USA) developed for the detection of 15 respiratory viruses. Her sample yielded positive result for only hMPV. On the 4th day of hospitalization, the patient was intubated because of respiratory failure and carbon dioxide retention. She was extubated on the 19th day but could not tolerate. In the thorax computed tomography (CT), bilateral hyperinflation, patchy infiltration, mosaic perfusion and atelectasis especially bilateral posterior areas were detected. Bronchoscopy was normal except mild bronchomalacia in right middle lobe bronchus. The patient was diagnosed as BO secondary to hMPV bronchiolitis, according to the clinical, virological, bronchoscopic and thorax CT results. On the 76th day of admission, she was discharged with respiratory support with home ventilation via a tracheostomy cannula and medical treatments of oral metilprednisolone, nebulized salbutamol and budesonide. In conclusion, hMPV should not be undervalued especially in infants with severe LRTI that can be complicated with BO."}, {"id": "pubmed23n0346_5794", "title": "[Childhood croup].", "score": 0.009708737864077669, "content": "Hoarseness, whooping cough and stridor are elements of a syndrome of upper airway obstruction. In childhood, acute laryngotracheobronchitis is by far the commonest cause of this syndrome. Yet, the differential diagnosis includes a number of rare and severe entities. In many cases, the traditional distinction between viral and spasmodic types is not possible. The value of humidifying therapy has not been established. In severe cases, nebulized adrenaline is of benefit but should be reserved for hospital. The effect lasts only two hours and at times a rebound effect is observed. It is now realized that some patients treated with adrenaline can safely be discharged after a two to three hours observation. There is a large body of evidence that all children arriving at the emergency department with croup should receive steroids without delay. This policy results in a much better outcome, with important reduction in hospitalizations, intensive care unit admissions and incubations. Oral dexamethasone is the drug of choice: it is as effective, easier to administer and cheaper than nebulised budesonide. In most studies, dexamethasone has been used at a dose of 0.6 mg/kg but there is some evidence that 0.15 mg/kg may be just as effective."}, {"id": "pubmed23n0482_17156", "title": "[Respiratory morbidity after hospital discharge in premature infants born at &lt; or = 32 weeks gestation with bronchopulmonary dysplasia].", "score": 0.009615384615384616, "content": "Bronchopulmonary dysplasia (BPD) is the most frequent cause of respiratory morbidity in the first 2 years of life among preterm infants who survive the first 28 days. To evaluate respiratory morbidity in the first 2 years of life in a group of preterm infants born at (32 weeks' gestation with BPD (oxygen requirement at 36 weeks' postconceptional age) by comparing it with that in preterm infants born at (32 weeks without BPD and with a control group of full term infants without neonatal morbidity. To determine whether respiratory morbidity in children with BPD decreases after the age of 2 years. Group I: preterm children with BPD (n = 29). Group II: preterm children without BPD (n = 29). Group III: children with appropriate gestational age and weight (n = 32). A cross-sectional, descriptive study of the three groups was performed over a 2-year period. In 17 children in group 1, the study was prolonged to the age of 4 years. We analyzed wheezing on at least two occasions, use of inhaled bronchodilators, use of inhaled glucocorticosteroids for more than 6 months, and hospitalization for respiratory illness. The chi-square test and Fischer's exact test were performed. At least one episode of wheezing occurred in 25 children (86.2%) in group I compared with 12 children (41.4%) in group II and 6 (18.8%) in group III. Nineteen children (65.5%) in group I and none in the remaining two groups received treatment with inhaled glucocorticosteroids for more than 6 months (p &lt; 0.001). Inhaled bronchodilators were used by 25 children (86.2%) in group I compared with 12 (41.4%) in group II and 6 (18.8%) in the control group (p &lt; 0.001). Twelve children (41.3%) in group I were hospitalized for respiratory illness compared with 8 (27.6%) in group II. There were no admissions among the control group. None of the children with BPD who received prophylaxis with palivizumab contracted respiratory syncytial virus infection. Seventeen children with BPD were evaluated until the age of 4 years. Episodes of wheezing decreased from 88.2% in the first year to 41 % between the third and fourth years (p &lt; 0.001). Treatment with inhaled glucocorticosteroids for more than 6 months was given to 88.2% in the first year, 41.2 % between the first and second year and to 0 % after the second year (p &lt; 0.001). Hospital admissions for respiratory illness decreased from 52.9% in the first year to 17.6% in the second year. None of the children were hospitalized after the age of 2 years (p &lt; 0.001). During the first 2 years of life, children with BPD showed a greater number of admissions and episodes of wheezing and a greater need for medical treatment. Respiratory morbidity improved with age, 40% showed recurrent wheezing episodes at the age of 4 years."}, {"id": "First_Aid_Step2_939", "title": "First_Aid_Step2", "score": 0.009576851472026759, "content": "Presents with low-grade fever, rhinorrhea, cough, and apnea (in young infants). Exam reveals tachypnea, wheezing, intercostal retractions, crackles, prolonged expiration, expiratory wheezing, and hyperresonance to percussion. Predominantly a clinical diagnosis; routine cases do not need blood work or a CXR. CXR may be obtained to rule out pneumonia and may show hyperinfl ation of the lungs with flattened diaphragms, interstitial infiltrates, and atelectasis. Nasopharyngeal aspirate to test for RSV is highly sensitive and specific but has little effect on management (infants should be treated for bronchiolitis regardless of whether RSV is or not). Treatment is primarily supportive; treat mild disease with outpatient management using fluids and nebulizers if needed."}, {"id": "pubmed23n0391_5035", "title": "New approaches to respiratory infections in children. Bronchiolitis and croup.", "score": 0.009523809523809525, "content": "Croup is a disease that is commonly seen in children younger than the age of 6 years. The cause is viral, with parainfluenza viruses and RSV being the two most common pathogens. Treatment consists primarily of supportive care, and parents usually have tried humidification and cool air exposure before the child presents to the ED. Children with moderate to severe croup are usually seen in the ED. The use of steroids in an oral preparation results in a clinical improvement of outpatients with mild to moderate croup and reduces the need for hospitalization. The dosage range for oral dexamethasone is 0.15 mg/kg to 0.6 mg/kg. Nebulized budesonide may also be used. Racemic or L-epinephrine, both of which are equally effective, can be used for symptomatic treatment in severe croup. After administration of racemic or L-epinephrine, hospitalization is not automatic and patients can be discharged safely from the ED after a 3-hour of observation period. There should be no respiratory distress, and the patient should have access to follow-up and emergency care if needed."}, {"id": "wiki20220301en027_63987", "title": "Infant respiratory distress syndrome", "score": 0.009433962264150943, "content": "Oxygen is given with a small amount of continuous positive airway pressure (CPAP), and intravenous fluids are administered to stabilize the blood sugar, blood salts and blood pressure. CPAP application to preterm neonates with respiratory distress is associated with a reduction in respiratory failure, mechanical ventilation and mortality. However, CPAP is associated with an increased rate of pneumothorax compared to spontaneous breathing with or without supplemental oxygen. If the baby's condition worsens, an endotracheal tube (breathing tube) is inserted into the trachea and intermittent breaths are given by a mechanical device. An exogenous preparation of pulmonary surfactant, either synthetic or extracted from animal lungs, is given through the breathing tube into the lungs. Surfactant medications can decrease the risk of death for very low-birth-weight infants who are hospitalized by 30%. Such small premature infants may remain ventilated for months. A study shows that an aerosol"}]}}}}
{"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 111]], "word_ranges": [[0, 15]], "text": "Typical of ticlopidine. Not only thrombocytopenia but also headache, schistocytes, high LDH and high bilirubin."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Typical of ticlopidine. Not only thrombocytopenia but also headache, schistocytes, high LDH and high bilirubin.", "full_answer_no_ref": "Typical of ticlopidine. Not only thrombocytopenia but also headache, schistocytes, high LDH and high bilirubin.", "full_question": "A 67-year-old female patient on ticlopidine treatment comes to the emergency department with headache, asthenia and petechiae in the lower extremities. Laboratory tests showed hemoglobin 8.2 g/dL, MCV 100 fl, platelets 25000/ul and leukocytes 7500/ul with normal formula. The reticulocyte count was elevated and the blood smear showed numerous schistocytes. Coagulation studies (APTT, PT and fibrinogen) are normal. The biochemistry shows LDH 2700 IU/l and bilirubin 2.6 mg/dl. What is the most likely diagnosis?", "id": 236, "lang": "en", "options": {"1": "Autoimmune thrombocytopenic purpura.", "2": "Thrombotic thrombocytopenic purpura.", "3": "Bone marrow aplasia.", "4": "Drug-induced thrombopenia.", "5": "Disseminated intravascular coagulation."}, "question_id_specific": 108, "type": "HEMATOLOGY", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0059_11730", "title": "[Thrombotic thrombocytopenic pupura (TTP)--remission following treatment with high-dose immunoglobulin].", "score": 0.019142700128228614, "content": "A 60-year-old man was admitted to our hospital because of fever, hemorrhagic tendency, anemia and neurological abnormality. A blood count revealed that the hemoglobin was 6.8 g/dl, the reticulocyte was 17.3 percent with 2 erythroblasts per 100 white cells, the white cell count was 7,100/microliters and the platelet count was 0.8 x 10(4)/microliters. Peripheral blood smear demonstrated marked fragmentation of red cells. Bone marrow examination disclosed the marked erythroid hyperplasia. Although the bleeding time was prolonged (14 minutes 30 seconds), the other hemostatic data were within normal limits. The serum bilirubin level was 1.57 mg/dl; LDH level, 1,437 U/l; creatinine level, 0.92 mg/dl; BUN level 14.7 mg/dl. Haptoglobin was below 10 mg/dl. Results of immunological tests were all negative except the result of PAIgG (576.6 ng/10(7) cells). The urinalysis showed proteinuria, microhematuria and trace granular and hyaline casts. A diagnosis of thrombotic thrombocytopenic purpura was made. The patient was initially treated with prednisolone (60 mg), aspirin (1,000 mg), dipyridamole (150 mg), gabexate mesilate (1.5 g), sodium oxagrel (80 mg) daily with little response. The thirty days after admission, infusion of gamma globulin (20 g, daily) was given for 3 days. The clinical state and laboratory findings became dramatically improved shortly after the administration of gamma globulin and the laboratory data came to be normalized after 1 month. After ten months of this treatment, the patient is remained asymptomatic and the hematological data are within normal range without using any drug. A trial seems justified to confirm the value of this mode of therapy."}, {"id": "pubmed23n0282_16640", "title": "[An elderly case of thrombotic thrombocytopenic purpura].", "score": 0.01879964695498676, "content": "A 78-year-old woman was admitted to our hospital because of disorientation and fever on January 21, 1992. Two days before admission she experienced vomiting, anorexia and general malaise. Laboratory examinations on admission disclosed a hemoglobin level of 11.1 g/dl and a platelet count of 8,000/microliters. The peripheral blood smear revealed anisocytosis with numerous schistocytes and poikilocytes. Polychromatophilic and nucleated red blood cells were also seen, and the reticulocyte count was 38/1000. Her serum lactate dehydrogenase (LDH) value was 2,977 WU and the total serum bilirubin level was 3.5 mg/dl with 2.7 mg/dl indirect reacting fraction. Serum creatinine was 4.7 mg/dl. Her consciousness became semicomatose after a systemic seizure which lasted approximately 15 seconds and her hemoglobin level decreased to 8.5 g/dl on hospital day 2. Therefore, we diagnosed her as having thrombotic thrombocytopenic purpura (TTP) because of the presence of all 5 features, that is, thrombocytopenia, microangiopathic hemolytic anemia, fluctuating neurologic abnormalities, renal dysfunction and fever. A plasmapheresis with fresh frozen plasma (FFP) replacement was begun on that day. She was also treated with anti-platelet agents, 80 mg/day aspirin, and 300 mg/day dipyridamole. Moreover, packed red blood cells (PRC) were infused. While also receiving diphenylhydantoin and phenobarbital to prevent convulsions, status epilepticus developed on day 3. Because of inhibited spontaneous respiration which was an adverse effect derived from diazepam and sodium thiamylal administered intravenously to treat the status epilepticus, an artificial respiration was initiated.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0681_21394", "title": "[Metastatic prostate cancer complicated with chronic disseminated intravascular coagulopathy causing acute renal failure, mimicking thrombotic thrombocytopenic purpura and hemolytic uremic syndrome: pathomechanism, differential diagnosis and therapy related to a case].", "score": 0.018614718614718615, "content": "Disseminated intravascular coagulopathy (DIC) is characterized as activation of the clotting system resulting in fibrin thrombi, gradually diminishing levels of clotting factors with increased risk of bleeding. Basically two types of DIC are distinguished: (1) chronic (compensated) - with alteration of laboratory values and (2) acute (non-compensated) - with severe clinical manifestations: bleeding, shock, acute renal failure (ARF), transient focal neurologic deficit, delirium or coma. Chronic DIC related to metastatic neoplasia is caused by pancreatic, gastric or prostatic carcinoma in most of the cases. Incidence rate of DIC is 13-30% in prostate cancer, among those only 0.4-1.65% of patients had clinical signs and symptoms of DIC. In other words, chronic DIC is developed in one of eight patients with prostate cancer. DIC is considered as a poor prognostic factor in prostatic carcinoma. The similar clinical and laboratory findings of TTP-HUS (thrombotic thrombocytopenic purpura - hemolytic uremic syndrome) and DIC makes it difficult to differentiate between them. A 71 years old male patient with known chronic obstructive pulmonary disease, benign prostatic hyperplasia, significant carotid artery stenosis, gastric ulcer and alcoholic liver disease was admitted to another hospital with melena. Gastroscopy revealed intact gastric mucosa and actually non-bleeding duodenal ulcer covered by clots. Laboratory results showed hyperkalemia, elevated kidney function tests, indirect hyperbilirubinemia, increased liver function tests, leukocytosis, anemia, thrombocytopenia and elevated international normalized ratio (INR). He was treated with saline infusions, four units of red blood cells and one unit of fresh frozen plasma transfusions. Four days later he was transported to our Institution with ARF. Physical examination revealed dyspnoe, petechiae, hemoptoe, oliguria, chest-wall pain and aggressive behavior. Thrombocytopenia, signs of MAHA (fragmentocytes and helmet cells in the peripheral blood), normal INR, elevated lactate dehydrogenase (LDH) and ARF suggested TTP-HUS. Hemodialysis and six plasmaferesis (PF) were carried out. After the fifth PF, skin manifestations of thrombotic microangiopathy occurred on the feet. Clotting analysis revealed elevated D-dimer (&gt;5 μg/mL), normal fibrinogen (3.2 g/L), a slightly raised INR (1.36) and activated partial prothrombin time (APTT) (45.8 sec), normal reticulocyte (57 G/L) and a slightly low platelet count (123 G/L), which proved to be chronic DIC. Therapeutic dose of low-molecular-weight heparin (LMWH) was started. Elevated prostate-specific antigen (PSA) (109.6 ng/mL) suggested prostatic carcinoma. Prostate biopsy revealed adenocarcinoma (Gleason: 4+4 for left lobe and 3+3 for right lobe). Elevated alkaline phosphatase suggested metastases in the bone, which were confirmed by bone scintigraphy. Combined androgen blockade (CAB) was started. After three months follow-up our patient's status is satisfactory. PSA is in the normal range (4.6 ng/mL). Thrombocytopenia of uncertain origin with normal or raised INR, APTT, elevated D-dimer, normal fibrinogen and reticulocyte count prove the diagnosis of chronic DIC. This process warrants searching for metastatic neoplasia. Due to the relatively low serum levels of circulating procoagulant factors (e.g. tissue factor), therapeutic dose of LMWH can be used with good efficiency in chronic DIC with low risk of bleeding. Severe DIC as a complication of metastatic prostate cancer can be treated by androgen deprivation therapy (ADT) or CAB in combination with ketokonazole and concomitant use of supportive treatment. Deme D, Ragán M, Kovács L, Kalmár K, Varga E, Varga T, Rakonczai E. Metastatic prostate cancer complicated with chronic disseminated intravascular coagulopathy causing acute renal failure mimicking thrombotic thrombocytopenic purpura and hemolytic uremic syndrome: pathomechanism, differential diagnosis and therapy related to a case."}, {"id": "pubmed23n0719_4304", "title": "Pseudo-thrombotic thrombocytopenic purpura: A rare presentation of pernicious anemia.", "score": 0.018575851393188854, "content": "Schistocytes are fragmented red blood cells due to the flow of blood through damaged capillaries and indicate endothelial injury. They are typical of microangiopathic hemolytic anemia seen in life threatening conditions like disseminated intravascular coagulation or thrombotic thrombocytopenic purpura/hemolytic uremic syndrome .We report a rare sub-acute presentation of pernicious anemia with hemolysis, thrombocytopenia and numerous schistocytes that was initially diagnosed as a more serious thrombotic thrombocytopenic purpura. A 31-year-old Caucasian woman presented with fatigue and paresthesia of both feet for 1 week. Past medical history included hypertension and gastro-esophageal reflux disease. Examination revealed scleral icterus and pallor. Examination of the abdomen did not show hepatosplenomegaly. Initial laboratory tests showed severe anemia, and low platelets. Indirect bilirubin and serum Lactate De Hydrogenase were elevated. Prothrombin time, partial thromboplastin time, serum fibrinogen, and serum fibrin degradation product levels were normal. Peripheral smear revealed numerous schistocytes, anisocytosis and macro-ovalocytes. Thrombotic thrombocytopenic purpura (TTP) was suspected due to the constellation of sub-acute onset of fatigue and paresthesia along with thrombocytopenia, schistocytes and an elevated LDH. Plasmapheresis was initiated for possible TTP. However, platelet count worsened despite plasmapheresis for 4 days. On re-evaluation, vitamin B(12) was found to be low. Treatment with intra-muscular vitamin B(12) led to symptomatic and hematologic improvement. Pernicious anemia was confirmed by the presence of anti-intrinsic factor antibodies, elevated serum gastrin level and atrophic gastritis. Clinicians must be aware of unusual clinical presentation of vitamin B(12) deficiency with schistocytes as the management is simple and effective."}, {"id": "pubmed23n1029_23571", "title": "Thrombotic Thrombocytopenic Purpura: What an Intensive Care Unit Doctor Needs to Know.", "score": 0.017073525138041265, "content": "Accurate and prompt diagnoses of thrombotic microangiopathy (TMA) in the emergency room (ER) and intensive care unit (ICU) setting can be challenging since its presentation involve multiple organ systems, and comorbid diseases can be deceptive for an accurate diagnosis.  Here, we present the case of a patient, who upon arrival to the ER, reported severe chest pain radiating to his left shoulder, diaphoresis, headache, and nausea. Several numbers of small petechiae on the bilateral lower extremities were also found during physical examination. Laboratory data demonstrated elevated troponin levels, platelet count of 34, and hemoglobin of  8.7 g/l. Establishing a differential diagnosis between a microvascular occlusive disorder and acute coronary syndrome was imperative to reduce further clinical complications and mortality. A peripheral smear, which is an essential test in approaching the diagnosis of thrombotic thrombocytopenic purpura (TTP), was done and it identified an increased number of schistocytes. The laboratory findings narrowed the diagnosis to an immunological process, where the dysfunctional platelets caused coronary thrombosis and further intermittent coronary ischemia. In this case report, we discuss the atypical presentation of TTP, its differential diagnosis, and management in order to develop an effective treatment in the ER and ICU settings and to reduce the mortality rate."}, {"id": "wiki20220301en046_51369", "title": "Schistocyte", "score": 0.015714943587381992, "content": "Schistocyte count A normal schistocyte count for a healthy individual is <0.5% although usual values are found to be <0.2%. A schistocyte count of >1% is most often found in thrombotic thrombocytopenic purpura, although they are more often seen within the range of 3–10% for this condition. A schistocyte count of <1% but greater than the normal value is suggestive of disseminated intravascular coagulation, but is not an absolute diagnosis. The standard for a schistocyte count is a microscopic examination of a peripheral blood smear."}, {"id": "pubmed23n0282_20398", "title": "[Emergency blood picture].", "score": 0.015268065268065269, "content": "Assessment of peripheral blood counts and blood film analysis are frequently performed as diagnostic procedures in emergency medicine. Far fewer situations exist, however, in which these analyses are the main clue in establishing an emergency diagnosis. Artifacts can lead to wrong diagnosis, e.g. pseudo-thrombocytopenia, which is defined as a low platelet count resulting from a laboratory artifact. Severe neutropenia (agranulocytosis) and extreme hyperleukocytosis, as well as suspicion of acute leukemia, require a rapid diagnostic work-up. A newly detected anemia should not necessarily be treated by packed red cell transfusions. The decision whether an anemic patient ought to receive transfusions should be based on the speed with which the anemia has developed, as well as on clinical judgement. As a rule a chronic anemia patient with hemoglobin above 70 g/l does not need transfusions. An uncritical transfusion policy can even cause emergencies, e.g. in patients with megaloblastic anemia or in anemic multiple myeloma patients with a hyperviscosity syndrome. An elevated hematocrit requires prompt further investigations. This is of utmost importance if one considers the diagnosis of polycythemia vera rubra, a disease in which patients are particularly prone to thrombotic complications. Fragmented red cells (schistocytes) on peripheral blood smears constitute a cardinal diagnostic clue for the detection of microangiopathic hemolytic anemias (MAHA), in particular for the diagnosis of the life-threatening thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Malaria is another example for a chief role of blood smears examination in achieving a rapid diagnosis. If one encounters an unexpected severe thrombocytopenia, a marrow examination reveals whether it is due to rapid peripheral destruction, or due to a marrow failure. Furthermore, in any patients with an unanticipated thrombocytopenia, a disseminated intravascular coagulation and a MAHA should be ruled out. Heparin-induced thrombocytopenia is a rare, but possibly fatal complication of therapy with heparins."}, {"id": "wiki20220301en085_21868", "title": "Thrombotic microangiopathy", "score": 0.014776444929116684, "content": "Diagnosis CBC and blood film: decreased platelets and schistocytes PT, aPTT, fibrinogen: normal markers of hemolysis: increased unconjugated bilirubin, increased LDH, decreased haptoglobin negative Coombs test. Creatinine, urea, to follow renal function ADAMSTS-13 gene, activity or inhibitor testing (TTP)."}, {"id": "pubmed23n0928_18867", "title": "Incidental Thrombotic Thrombocytopenic Purpura during Acute Ischemic Stroke and Thrombolytic Treatment.", "score": 0.014705882352941176, "content": "Intravenous tissue plasminogen activator (IV tPA) was shown to be an effective treatment for acute ischemic stroke (AIS). According to stroke guidelines, there is no need to wait for the complete blood count (CBC) and coagulation test results before application of IV alteplase if there is no suspected coagulation disorder. In this study, a patient with AIS and thrombotic thrombocytopenic purpura (TTP) symptoms during thrombolytic treatment was presented. A 33-year-old male patient was admitted at the 2.5th hour of AIS symptoms onset with right hemiparesis and sensorimotor aphasia. Cranial computed tomography (CT) and diffusion magnetic resonance imaging did not reveal any abnormality. In his medical history, the patient did not have any contraindication for thrombolytic treatment. To avoid delays to thrombolytic therapy, blood samples were taken, and after that, IV bolus alteplase treatment was applied. During maintenance treatment, agitation and vomiting developed. The result of blood samples showed less than 26,000 mm<sup3</sup platelet count and maintenance therapy was stopped. In control cranial CT, there was no hemorrhage. In the laboratory examination; anemia, low platelet count; increased indirect bilirubin, lactate dehydrogenase (LDH) levels were found, and fever was 38.4°C. Schistocytes were observed in peripheral blood smear and the patient was diagnosed as TTP. Stroke guidelines recommend not to wait for the results of CBC and coagulation tests before IV tPA treatment in patients who do not have any history of coagulopathy disorder. If possible, before applying IV tPA we may wait for the results of coagulation and CBC tests, keeping in mind the diseaes with high mortality such as TTP."}, {"id": "pubmed23n0842_9225", "title": "To treat or not to treat: a rare case of pseudo-thrombotic thrombocytopenic purpura in a Jehovah's Witness.", "score": 0.014210128495842782, "content": "Thrombotic thrombocytopenic purpura (TTP) is a rare microvascular occlusive disorder characterized by systemic intravascular aggregation of platelets, thrombocytopenia, and mechanical injury to red blood cells. We report a rare case of pernicious anemia presenting as TTP in a Jehovah's Witness. A 46-year-old Jehovah's Witness female presented with epigastric pain, vomiting, and diarrhea for 2 days and fatigue and paresthesias for 4 weeks. Initial laboratory evaluation showed severe anemia and thrombocytopenia with elevated total bilirubin and lactate dehydrogenase. Peripheral blood smear showed schistocytes, macroovalocytes, and hypersegmented neutrophils. TTP was suspected and plasmapheresis was offered. The patient refused it due to her religious beliefs. Due to the presence of macroovalocytes and hypersegmented neutrophils, vitamin B12 level was checked and found to be extremely low. Anti-intrinsic factor antibodies and anti-parietal cell antibodies were also positive; hence a diagnosis of pernicious anemia was established. Treatment with intramuscular vitamin B12 was initiated, which resulted in dramatic neurologic and hematologic improvement. Vitamin B12 deficiency can lead to elevated levels of homocysteine in the blood. Homocysteine can cause endothelial dysfunction, which can lead to formation of microvascular thrombi. Due to this phenomenon, vitamin B12 deficiency can rarely present with schistocytes and thrombocytopenia, which combined with other stigmata of vitamin B12 deficiency, can be misdiagnosed as TTP."}, {"id": "pubmed23n1041_19599", "title": "Immune Thrombocytopenia Purpura Secondary to COVID-19.", "score": 0.013900913900913902, "content": "A 73-year-old female with past medical history of essential hypertension, hyperlipidemia, seasonal allergies, and chronic back pain presented to the hospital with complaints of headaches, fevers, fatigue, generalized body aches, shortness of breath, and diarrhea. Initial complete blood count was remarkable for leukopenia with an absolute lymph count of 0.60 K/µL and severe thrombocytopenia (platelet count &lt; 3 K/µL). She was tested for COVID-19 via nasopharyngeal swab polymerase chain reaction (PCR) testing and found positive. Additional labs showed an elevated D-dimer, C-reactive protein, fibrinogen, and lactate dehydrogenase. Vitamin B12 and folate levels were obtained and found to be normal. Peripheral smear showed no schistocytes or additional hematologic abnormalities apart from thrombocytopenia. The patient was transfused one unit of platelets with no improvement in platelet count. Fibrinogen count was obtained and found in normal range at 458 mg/dL. Prothrombin time (PT), activated partial thromboplastin time (aPTT), and international normalized ratio (INR) were all found to be normal. Immune thrombocytopenia purpura (ITP) was suspected and intravenous immunoglobulin (IVIG) was administered at a dose of 1 g/kg/day for two doses. By day 4, the patient had marked response to treatment with platelet recovery to 105 K/µL and subsequently discharged by day 5 with complete resolution of symptoms and platelet count of 146 K/µL. Twenty-eight days after discharge, she presented to hematology clinic with platelet count of 8 K/µL. Repeat nasopharyngeal swab PCR COVID testing was negative and she was treated with IVIG and pulse dexamethasone with prompt response, confirming suspicion of underlying, undiagnosed ITP prior to COVID infection."}, {"id": "pubmed23n1042_13774", "title": "Thrombotic Thrombocytopenic Purpura: Revisiting a Miss and an Inevitable Consequence.", "score": 0.01382216985407414, "content": "Thrombotic thrombocytopenic purpura (TTP) is typically characterized by the symptomatic pentad of fever, thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, and renal failure. Atypical TTP is the diagnosis used to describe the subset of patients with TTP who present with symptoms that deviate from the classic pentad. We report a case an 86-year-old woman who presented to the emergency department complaining of chest pain for one day. She was reportedly on antibiotics for sinus infection. Physical examination revealed multiple bilateral superficial hematomas, predominantly on her extremities. On admission, her lab values were as follows: platelet count of 6,000/cubic millimeter, hemoglobin of 10.4 grams/deciliter, leukocyte count of 5100 cells/cubic millimeter, total bilirubin of 2.3 milligrams/deciliter, and troponin-I of 5.190 nanograms/milliliter. Peripheral blood smear was normal and did not reveal any schistocytes. The patient was admitted to the intensive care unit with a diagnosis of a non-ST-elevation myocardial infarction and a presumed diagnosis of immune thrombocytopenic purpura from antibiotic use. She was treated with intravenous solumedrol and a high-intensity statin. On the third day of her admission, the patient's mental functioning deteriorated and was intubated to protect her airway. A second peripheral smear revealed schistocytes, and subsequent laboratory studies supported the diagnosis of TTP. Plasma exchange therapy was planned. However, the patient succumbed to cardiac arrest before it could be initiated. The diagnosis was later confirmed with an ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) assay.  This case serves as an example of one of the many ways in which TTP can present, and emphasizes the importance of considering TTP as a differential diagnosis."}, {"id": "InternalMed_Harrison_9127", "title": "InternalMed_Harrison", "score": 0.012988634944423628, "content": "Platelet count < 150,000/˜L Hemoglobin and white blood count Normal Abnormal Bone marrow examination Peripheral blood smear Platelets clumped: Redraw in sodium citrate or heparin Fragmented red blood cells Normal RBC morphology; platelets normal or increased in size Microangiopathic hemolytic anemias (e.g., DIC, TTP) Consider: Drug-induced thrombocytopenia Infection-induced thrombocytopenia Idiopathic immune thrombocytopenia Congenital thrombocytopenia first appear in areas of increased venous pressure, the ankles and feet in an ambulatory patient. Petechiae are pinpoint, nonblanching hemorrhages and are usually a sign of a decreased platelet number and not platelet dysfunction. Wet purpura, blood blisters that form on the oral mucosa, are thought to denote an increased risk of life-threatening hemorrhage in the thrombocytopenic patient. Excessive bruising is seen in disorders of both platelet number and function."}, {"id": "article-30097_9", "title": "Thrombotic Thrombocytopenic Purpura -- Evaluation", "score": 0.01282051282051282, "content": "Laboratory evaluation plays a critically important role in diagnosing TTP because signs and symptoms are variable, and end-organ damage can be delayed. For diagnosis, the laboratory data must show anemia and thrombocytopenia along with an indication of active hemolysis, such as the presence of schistocytes, increased unconjugated bilirubin, increased reticulocyte count and increased lactate dehydrogenase. [23] [24] [25] As with any hemolysis in general, the serum haptoglobin decreases as it combines with the free hemoglobin released from the destroyed erythrocytes."}, {"id": "pubmed23n1073_21962", "title": "[Analysis of five children with acquired thrombotic thrombocytopenic purpura].", "score": 0.012670917037571675, "content": "<bObjective:</b To investigate the clinical characteristics, treatment and prognosis of children with acquired thrombotic thrombocytopenic purpura (TTP). <bMethods:</b The clinical manifestations, laboratory examination, treatment and prognosis of 5 children with acquired TTP hospitalized in Beijing Children's Hospital, Capital Medical University from January 2016 to July 2019 were analyzed retrospectively. <bResults:</b There were 5 children with acquired TTP including 2 males and 3 females, with the onset age of 8.9(0.8-14.5) years, while 11 children with TTP in the same period. Thrombocytopenia and microangiopathic hemolytic anemia were found in all 5 patients. Only one patient had typical pentalogy of TTP, 3 patients had nervous system symptoms and 3 patients had fever, while renal impairment was relatively rare (1 case). Laboratory examination showed severe thrombocytopenia (7(4-14) ×10<sup9</sup/L) and low level of hemoglobin (70(58-100)g/L) in all 5 children. Blood biochemical examination showed that total bilirubin (mainly indirect bilirubin) increased in 3 patients, lactate dehydrogenase increased in 5 patients, and urea nitrogen increased in 1 patient. Bone marrow smear showed megakaryocyte did not decrease. Plasma ADAMTS13 activity was 0 in all 5 patients while ADAMTS13 inhibitor was positive in 4 patients and negative in 1 patient. All 5 children received glucocorticoid therapy, rituximab was added in the early stage of the disease, and 3 children received plasma exchange. The time of platelet recovery to normal was 19 (9-29) days. One child had TTP recurrence after 9 months of treatment. The condition was stable after being treated with glucocorticoid and rituximab again. This case was finally diagnosed as systemic lupus erythematosus after more than 3 years followed up. By December 1, 2020, the follow-up time was 24(16-57) months.The clinical symptoms of all patients disappeared and the platelet level was stable at 159(125-269) ×10<sup9</sup/L. <bConclusions:</b Childhood acquired TTP is relatively rare, which can occur in all age groups. The clinical manifestations are mainly thrombocytopenia and microangiopathic hemolytic anemia, the plasma ADAMTS 13 activity and inhibitor test are helpful for the diagnosis of acquired TTP. Plasma exchange and rituximab are effective treatment. This disease requires long-term follow-up."}, {"id": "article-30113_18", "title": "Thrombotic Thrombocytopenic Purpura Evaluation and Management -- Evaluation", "score": 0.01248271092669433, "content": "Reference methods for determining the ADAMTS-13 activity are complex, and the results are not always readily available in an emergency setting. Thus, initial management should be started based on clinical presentation. Blood testing will likely show profound thrombocytopenia (counts <30x10 9 /L), microangiopathic hemolysis (schistocytes >1% in a blood smear), decreased ADAMTS-13 activity, and the presence of autoantibodies to ADAMTS-13. The direct Coombs test should be negative (except in some cases of SLE), and the coagulation test should not be prolonged. Other lab abnormalities include decreased haptoglobin, increased reticulocytes, elevated LDH, and elevated indirect bilirubin. [19] [20] [21] [22]"}, {"id": "wiki20220301en435_34729", "title": "Upshaw–Schulman syndrome", "score": 0.012375138734739178, "content": "Diagnosis A diagnosis of TTP is based on the clinical symptoms with the concomitant presence of thrombocytopenia (platelet count below 100×109/L) and microangiopathic hemolytic anemia with schistocytes on the blood smear, a negative direct antiglobulin test (Coombs test), elevated levels of hemolysis markers (such as total bilirubin, LDH, free hemoglobin, and an unmeasurable haptoglobin), after exclusion of any other apparent cause. USS can present similar to the following diseases, which have to be excluded: fulminant infections, disseminated intravascular coagulation, autoimmune hemolytic anemia, Evans syndrome, the typical and atypical form of hemolytic uremic syndrome, HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome, pre-eclampsia, heparin-induced thrombocytopenia, cancer that is often accompanied with metastasis, kidney injury, antiphospholipid antibody syndrome, and side effects from hematopoietic stem cell transplantation."}, {"id": "pubmed23n1159_13172", "title": "Rituximab for acute plasma-refractory thrombotic thrombocytopenic purpura: A case report.", "score": 0.011860897638347896, "content": "Thrombotic thrombocytopenic purpura (TTP) is a rare disease due to deficiency of ADAMTS13 which can present with anemia and thrombocytopenia. The study highlights the role of PLASMIC score in diagnosis and rituximab in the treatment of this condition. Herein, we report a case of 38 years old female who had presented with fever, irritability, and altered sensorium. On investigations, she had hemolytic anemia, and thrombocytopenia with peripheral blood smear showing occasional schistocytes and managed with steroids and plasma exchange. As her platelet, LDH, and a few other lab parameters failed to normalize and met the criteria of refractory TTP, hence she was started on 5 cycles of rituximab and her condition improved. Thrombotic thrombocytopenic purpura can be presumed based upon PLASMIC score where if the score is 5 or more while ADAMTS13 assay is required for confirmation. It is a life-threatening condition where treatment options include therapeutic plasma exchange (PEX), glucocorticoids, Rituximab, and caplacizumab. Rituximab is considered particularly in refractory cases. Thrombotic thrombocytopenic purpura can lead to complications due to low platelet counts. Hence, early diagnosis and intervention are crucial to prevent such complications."}, {"id": "article-20610_7", "title": "Disseminated Intravascular Coagulation -- Evaluation", "score": 0.011761432618373127, "content": "No single history, physical exam, or laboratory component can lead to a diagnosis of or rule out DIC; therefore, a combination of both subjective, objective, and laboratory findings should be utilized to make a diagnosis of DIC. Laboratory findings suggestive of DIC include both an increased prothrombin time (PT) and an increased partial thromboplastin time (PTT), as well as a decreased fibrinogen level as widespread activation and consumption of the clotting cascade occurs. The overall platelet count and hematocrit level may be reduced as well. Schistocytes or fragmented erythrocytes are also commonly seen on a peripheral blood smear. The presence of fibrin split products additionally has a high sensitivity but low specificity for the presence of DIC. A specific scoring system to assess for the presence of DIC was established in 2007. This score includes platelet count, fibrin markers such as D-dimer, prolonged PT, and fibrinogen level, with a score over five indicating a high likelihood for overt DIC. [18] [19] [20] [21]"}, {"id": "InternalMed_Harrison_9237", "title": "InternalMed_Harrison", "score": 0.011487122015649703, "content": "The mortality ranges from 30 to >80% depending on the underlying disease, the severity of the DIC, and the age of the patient. The diagnosis of clinically significant DIC is based on the presence of clinical and/or laboratory abnormalities of coagulation or thrombocytopenia. The laboratory diagnosis of DIC should prompt a search for the underlying disease if it is not already apparent. There is no single test that establishes the diagnosis of DIC. The laboratory investigation should include coagulation tests (aPTT, PT, thrombin time [TT]) and markers of fibrin degradation products (FDPs), in addition to platelet and red cell count and analysis of the blood smear. These tests should be repeated over a period of 6–8 h because an initially mild abnormality can change dramatically in patients with severe DIC."}, {"id": "wiki20220301en027_75422", "title": "Henoch–Schönlein purpura", "score": 0.011338655559412242, "content": "Diagnosis The diagnosis is based on the combination of the symptoms, as very few other diseases cause the same symptoms together. Blood tests may show elevated creatinine and urea levels (in kidney involvement), raised IgA levels (in about 50%), and raised C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR) results; none are specific for Henoch–Schönlein purpura. The platelet count may be raised, and distinguishes it from diseases where low platelets are the cause of the purpura, such as idiopathic thrombocytopenic purpura and thrombotic thrombocytopenic purpura."}, {"id": "InternalMed_Harrison_21351", "title": "InternalMed_Harrison", "score": 0.011331947472494042, "content": "during bicarbonate infu-ated with red blood cell fragmentation and numerous schistocytes sion. Administration of sodium bicarbonate may also lead to urinary on peripheral smear. Reticulocytosis, decreased plasma haptoglobin, precipitation of calcium phosphate, which is less soluble at alkaline and an LDH level document hemolysis. The serum bilirubin level is pH. Dialysis is often necessary and should be considered early in the usually normal or slightly elevated. The Coombs’ test is negative. The course. Hemodialysis is preferred. Hemofiltration offers a gradual, white cell count is usually normal, and thrombocytopenia (<100,000/ continuous method of removing cellular by-products and fluid. The μL) is almost always present. Most patients have a normal coagulation prognosis is excellent, and renal function recovers after the uric acid profile, although some have mild elevations in thrombin time and in level is lowered to ≤10 mg/dL. levels of fibrin degradation products. The serum"}, {"id": "InternalMed_Harrison_9154", "title": "InternalMed_Harrison", "score": 0.011101064898533252, "content": "Thrombotic thrombocytopenic microangiopathies are a group of disorders characterized by thrombocytopenia, a microangiopathic hemolytic anemia evident by fragmented RBCs (Fig. 140-1D) and laboratory evidence of hemolysis, and microvascular thrombosis. They include thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS), as well as syndromes complicating bone marrow transplantation, certain medications and infections, pregnancy, and vasculitis. In DIC, although thrombocytopenia and microangiopathy are seen, a coagulopathy predominates, with consumption of clotting factors and fibrinogen resulting in an elevated prothrombin time (PT) and often activated partial thromboplastin time (aPTT). The PT and aPTT are characteristically normal in TTP or HUS."}, {"id": "pubmed23n0817_13708", "title": "Does corticosteroid treatment cause prolonged recovery and increased total bilirubin level in severe ADAMTS-13-deficient TTP patient?", "score": 0.01057536692895809, "content": "A 41-year-old female patient complaining of fatigue, headache, mild confusion, and rush on her lower extremities was admitted to our emergency department. Laboratory tests revealed that he had anemia, thrombocytopenia, and increased levels of indirect bilirubin and lactic dehydrogenase (LDH) in blood tests. Direct and indirect Coombs tests were negative, and fragmented erythrocytes were observed in peripheral blood smears. The patient was diagnosed with thrombotic thrombocytopenic purpura (TTP). The best supportive care was provided. Therapeutic plasma exchange (TPE) and 1 mg/kg methylprednisolone treatments were administered. On the 10th day of treatment, LDH level and fragmented red blood cells in peripheral blood smear were decreased, but his direct and indirect bilirubin levels increased despite the fact that he was treated with 1 mg/kg methylprednisolone and TPE. The patient had severe ADAMTS-13 deficiency. After discontinued steroids treatment, his bilirubin level normalized within 4 days. On the 4th day after bilirubin level normalized, vincristine treatment was administered. TPE was also continued. There was no consensus about the optimal schedule for discontinuing plasmapheresis therapy, and also we observed total bilirubin level improvement with discontinued corticosteroid treatment. In this case, corticosteroid treatment was linked with the increase of total bilirubin level in severe ADAMTS-13-deficient TTP patient."}, {"id": "First_Aid_Step2_372", "title": "First_Aid_Step2", "score": 0.01041854501011801, "content": "A clinical syndrome characterized by f ve signs/symptoms: low platelet count, microangiopathic hemolytic anemia, neurologic changes (delirium, seizure, stroke), impaired renal function, and fever. Diagnosis is largely clinical. It is rare for all signs to be present, but the presence of schistocytes (broken RBCs) on peripheral smear with low platelets and rising creatinine is highly suggestive. Nucleated RBCs are also often seen in the peripheral smear. Hemolytic anemia labs include elevated indirect bilirubin, LDH, and AST along with low haptoglobin. Coagulation factors are normal. TABLE 2.7-3. Laboratory Values in DIC Overlapping conditions are HUS, HELLP syndrome, and DIC. HUS: Characterized by renal failure, hemolytic anemia, and low platelets. Severe elevations in creatinine are more typical of HUS than of TTP. HELLP syndrome: Affects pregnant women, often occurring in conjunction with preeclampsia (see the Obstetrics chapter)."}, {"id": "InternalMed_Harrison_12876", "title": "InternalMed_Harrison", "score": 0.010327883498615208, "content": "Because the clinical picture of brucellosis is not distinctive, the diagnosis must be based on a history of potential exposure, a presentation consistent with the disease, and supporting laboratory findings. Results of routine biochemical assays are usually within normal limits, although serum levels of hepatic enzymes and bilirubin may be elevated. Peripheral leukocyte counts are usually normal or low, with relative lymphocytosis. Mild anemia may be documented. Thrombocytopenia and disseminated intravascular coagulation with raised levels of fibrinogen degradation products can develop. The erythrocyte sedimentation rate and C-reactive protein levels are often normal but may be raised."}, {"id": "wiki20220301en192_930", "title": "Harrington–Hollingsworth experiment", "score": 0.01019672131147541, "content": "The Harrington–Hollingsworth experiment was an experiment that established the autoimmune nature of the blood disorder immune thrombocytopenic purpura. It was performed in 1950 by the academic staff of Barnes-Jewish Hospital in St. Louis, Missouri. Experiment The experiment was undertaken in 1950 by William J. Harrington and James W. Hollingsworth, who postulated that in patients with idiopathic thrombocytopenic purpura (ITP), it was a blood factor that caused the destruction of platelets. To test this hypothesis, Harrington received 500 ml of blood from a patient with ITP. Within three hours, his platelets dropped to dangerously low levels and he experienced a seizure. His platelet count remained extremely low for four days, finally returning to normal levels by the fifth day. Bone marrow biopsy from Harrington's sternum demonstrated normal megakaryocytes, the cells necessary for platelet production."}, {"id": "article-30097_24", "title": "Thrombotic Thrombocytopenic Purpura -- Differential Diagnosis", "score": 0.010186907609177075, "content": "Disseminated intravascular coagulation; schistocytes are relatively uncommon, there are abnormal coagulation studies, and fibrinolysis is more common in DIC than TTP. [41] [42]"}, {"id": "InternalMed_Harrison_8195", "title": "InternalMed_Harrison", "score": 0.010150858610174932, "content": "Hemolytic Anemias and Anemia Due to Acute Blood Loss 650 The laboratory features of HA are related to hemolysis per se and the erythropoietic response of the bone marrow. Hemolysis regularly produces an increase in unconjugated bilirubin and aspartate aminotransferase (AST) in the serum; urobilinogen will be increased in both urine and stool. If hemolysis is mainly intravascular, the telltale sign is hemoglobinuria (often associated with hemosiderinuria); in the serum, there is hemoglobin, lactate dehydrogenase (LDH) is increased, and haptoglobin is reduced. In contrast, the bilirubin level may be normal or only mildly elevated. The main sign of the erythropoietic response by the bone marrow is an increase in reticulocytes (a test all too often neglected in the initial workup of a patient with anemia). Usually the increase will be reflected in both the percentage of reticulocytes (the more commonly quoted figure) and the absolute reticulocyte count (the more definitive parameter). The"}, {"id": "InternalMed_Harrison_12756", "title": "InternalMed_Harrison", "score": 0.010116171481443404, "content": "Shiga toxin produced by S. dysenteriae type 1 has been linked countries, where enterohemorrhagic E. coli (EHEC) predominates as the etiologic agent of this syndrome. HUS is an early complication that most often develops after several days of diarrhea. Clinical examination shows pallor, asthenia, and irritability and, in some cases, bleeding of the nose and gums, oliguria, and increasing edema. HUS is a nonimmune (Coombs test–negative) hemolytic anemia defined by a diagnostic triad: microangiopathic hemolytic anemia (hemoglobin level typically <80 g/L [<8 g/dL]), thrombocytopenia (mild to moderate in severity; typically <60,000 platelets/μL), and acute renal failure due to thrombosis of the glomerular capillaries (with markedly elevated creatinine levels). Anemia is severe, with fragmented red blood cells (schizocytes) in the peripheral smear, high serum concentrations of lactate dehydrogenase and free circulating hemoglobin, and elevated reticulocyte counts. Acute renal failure occurs"}, {"id": "InternalMed_Harrison_9159", "title": "InternalMed_Harrison", "score": 0.009900990099009901, "content": "TTP is a devastating disease if not diagnosed and treated promptly. In patients presenting with new thrombocytopenia, with or without evidence of renal insufficiency and other elements of classic TTP, laboratory data should be obtained to rule out DIC and to evaluate for evidence of microangiopathic hemolytic anemia. Findings to support the TTP diagnosis include an increased lactate dehydrogenase and indirect bilirubin, decreased haptoglobin, and increased reticulocyte count, with a negative direct antiglobulin test. The peripheral smear should be examined for evidence of schistocytes (Fig. 140-1D). Polychromasia is usually also present due to the increased number of young red blood cells, and nucleated RBCs are often present, which is thought to be due to infarction in the micro-circulatory system of the bone marrow."}, {"id": "pubmed23n1147_11088", "title": "Thrombotic thrombocytopenic purpura as an acute complication of COVID-19.", "score": 0.009694835680751173, "content": "While disseminated intravascular coagulation (DIC) is a serious complication of COVID-19, a close differential in critically ill patients with thrombocytopenia is Thrombotic thrombocytopenic purpura (TTP). We describe the case of a middle-aged lady admitted with COVID-19 pneumonia who developed progressive thrombocytopenia, altered sensorium and renal failure. The absence of coagulation abnormalities alerted to the possibility of TTP, strengthened by presence of schistocytes in peripheral smear. This case highlights the need for high index of suspicion and to pay attention to normal tests as well that might give clues to the diagnosis. New onset thrombocytopenia in COVID-19 need not always indicate DIC. A careful examination of peripheral smear may help diagnosing TTP especially if coagulation profile is normal."}]}}}}