{"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 258]], "word_ranges": [[0, 39]], "text": "This is a grade IIa ulcer (Forrest classification), with a high risk of recurrence. Therefore, endoscopic treatment and hospitalization with intravenous treatment with PPIs (in our setting it is usually omeprazole) for at least 72 hours is clearly indicated."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[259, 344]], "word_ranges": [[39, 51]], "text": "Surgery is indicated in massive bleeding or bleeding refractory to medical treatment."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "This is a grade IIa ulcer (Forrest classification), with a high risk of recurrence. Therefore, endoscopic treatment and hospitalization with intravenous treatment with PPIs (in our setting it is usually omeprazole) for at least 72 hours is clearly indicated. Surgery is indicated in massive bleeding or bleeding refractory to medical treatment.", "full_answer_no_ref": "This is a grade IIa ulcer (Forrest classification), with a high risk of recurrence. Therefore, endoscopic treatment and hospitalization with intravenous treatment with PPIs (in our setting it is usually omeprazole) for at least 72 hours is clearly indicated. Surgery is indicated in massive bleeding or bleeding refractory to medical treatment.", "full_question": "A 52-year-old man with no concomitant diseases comes to the emergency department for melena of 24 hours evolution without hemodynamic repercussions. He denies taking nonsteroidal anti-inflammatory drugs. The hematocrit is 33% and the rest of the laboratory tests are normal. The upper gastrointestinal endoscopy performed urgently 6 hours after admission shows a normal stomach, without blood or hematic debris and an excavated ulcer of 8 mm in diameter in the anterior face of the duodenal bulb with \"visible vessel\" at its base and without active bleeding. Which of the following statements is true?", "id": 274, "lang": "en", "options": {"1": "In the initial endoscopy it is indicated to apply an endoscopic therapy and subsequently to establish endovenous treatment with high doses of a proton pump inhibitor. This strategy has been shown to reduce the risk of hemorrhagic recurrence and mortality.", "2": "In the initial endoscopy, given the absence of active bleeding, endoscopic therapy is not indicated. Subsequently, to reduce the risk of hemorrhagic recurrence, intravenous treatment with high doses of a proton pump inhibitor should be started.", "3": "n initial endoscopy, endoscopic therapy is indicated. Subsequent treatment with high doses of a proton pump inhibitor has not been shown to be of any additional benefit.", "4": "Since this is a complicated ulcer (hemorrhage) the best therapeutic option, once the hemorrhagic episode is resolved, is a vagotomy and pyloroplasty.", "5": NaN}, "question_id_specific": 73, "type": "DIGESTIVE SYSTEM", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0519_5512", "title": "Treatment of nonvariceal upper gastrointestinal bleeding.", "score": 0.017170228445099484, "content": "The etiology, pathophysiology, prognostic factors, pharmacologic treatment, and pharmacoeconomic considerations of nonvariceal upper-gastrointestinal-tract bleeding (UGB) are reviewed. UGB is associated with substantial morbidity and mortality. While UGB can be caused by a wide variety of medical conditions, 50% of UGB cases are caused by peptic ulcers. Approximately 80% of all UGB episodes stop bleeding spontaneously. Recurrence of gastrointestinal hemorrhage is associated with an increased mortality rate, a greater need for surgery, blood transfusions, a prolonged length of hospital stay, and increased overall health care costs. All patients with UGB should be evaluated for signs and symptoms of hemodynamic instability and active hemorrhage. Endoscopy within the first 24 hours of a UGB episode is considered the standard of therapy for the management of the initial hemorrhage. However, approximately 20% of patients will experience a rebleeding episode. Acid-suppressive therapy with proton-pump inhibitors (PPIs) in addition to endoscopic hemostasis is effective in reducing the frequency of rebleeding, the need for surgery, transfusion requirements, and the length of hospital stay, but not mortality rates. There are multiple dosing options for administration of PPIs in this setting. More studies are necessary to elucidate the best therapeutic approach to manage UGB. Acid-suppressive therapy is beneficial in the management of UGB. It reduces the frequency of rebleeding, the need for surgery, transfusion requirements, and the length of hospital stay. To date, no pharmacologic intervention has demonstrated a reduction in the mortality rates of patients with UGB. An optimal acid-suppressive regimen has not yet been clearly established."}, {"id": "pubmed23n0847_9971", "title": "[Gastrointestinal bleeding].", "score": 0.016576819407008087, "content": "In the Digestive Disease Week in 2015 there have been some new contributions in the field of gastrointestinal bleeding that deserve to be highlighted. Treatment of celecoxib with a proton pump inhibitor is safer than treatment with nonselective NSAID and a proton pump inhibitor in high risk gastrointestinal and cardiovascular patients who mostly also take acetylsalicylic acid. Several studies confirm the need to restart the antiplatelet or anticoagulant therapy at an early stage after a gastrointestinal hemorrhage. The need for urgent endoscopy before 6-12 h after the onset of upper gastrointestinal bleeding episode may be beneficial in patients with hemodynamic instability and high risk for comorbidity. It is confirmed that in Western but not in Japanese populations, gastrointestinal bleeding episodes admitted to hospital during weekend days are associated with a worse prognosis associated with delays in the clinical management of the events. The strategy of a restrictive policy on blood transfusions during an upper GI bleeding event has been challenged. Several studies have shown the benefit of identifying the bleeding vessel in non varicose underlying gastric lesions by Doppler ultrasound which allows direct endoscopic therapy in the patient with upper GI bleeding. Finally, it has been reported that lower gastrointestinal bleeding diverticula band ligation or hemoclipping are both safe and have the same long-term outcomes."}, {"id": "pubmed23n0619_522", "title": "Proton pump inhibitors and recurrent bleeding in peptic ulcer disease.", "score": 0.016348592019217935, "content": "Peptic ulcer disease (PUD) is one of the main lesions responsible for upper gastrointestinal (GI) bleeding, as well as esophageal varices and Mallory-Weiss tear. Helicobacter pylori and non-steroidal anti-inflammatory drugs (NSAIDs)/aspirin are the major responsible causes. In cases of upper GI bleeding, urgent endoscopy is performed after stabilization of vital signs. There are several modalities for controlling bleeding in PUD, such as ethanol injection or hypertonic saline with epinephrine. Recurrent bleeding occurs in 20% of patients after endoscopic therapy. The combination of endoscopic intervention and a proton pump inhibitor (PPI) is necessary to achieve hemostasis of active bleeding. It has been reported that high-dose omeprazole (80 mg bolus injection, then 8 mg/h continuous infusion for 72 h, then 40 mg/day orally for 1 week) can reduce recurrent bleeding, the need for surgery and mortality from hemorrhagic shock in patients with high-risk peptic ulcer bleeding, as compared with standard-dose omeprazole. The metabolism of PPIs is dependent upon P450 2C19 genotypes and the clinical usefulness of genotypic analysis remains to be determined."}, {"id": "pubmed23n0717_12878", "title": "Management of patients with ulcer bleeding.", "score": 0.016101207590569294, "content": "This guideline presents recommendations for the step-wise management of patients with overt upper gastrointestinal bleeding. Hemodynamic status is first assessed, and resuscitation initiated as needed. Patients are risk-stratified based on features such as hemodynamic status, comorbidities, age, and laboratory tests. Pre-endoscopic erythromycin is considered to increase diagnostic yield at first endoscopy. Pre-endoscopic proton pump inhibitor (PPI) may be considered to decrease the need for endoscopic therapy but does not improve clinical outcomes. Upper endoscopy is generally performed within 24h. The endoscopic features of ulcers direct further management. Patients with active bleeding or non-bleeding visible vessels receive endoscopic therapy (e.g., bipolar electrocoagulation, heater probe, sclerosant, clips) and those with an adherent clot may receive endoscopic therapy; these patients then receive intravenous PPI with a bolus followed by continuous infusion. Patients with flat spots or clean-based ulcers do not require endoscopic therapy or intensive PPI therapy. Recurrent bleeding after endoscopic therapy is treated with a second endoscopic treatment; if bleeding persists or recurs, treatment with surgery or interventional radiology is undertaken. Prevention of recurrent bleeding is based on the etiology of the bleeding ulcer. H. pylori is eradicated and after cure is documented anti-ulcer therapy is generally not given. Nonsteroidal anti-inflammatory drugs (NSAIDs) are stopped; if they must be resumed low-dose COX-2-selective NSAID plus PPI is used. Patients with established cardiovascular disease who require aspirin should start PPI and generally re-institute aspirin soon after bleeding ceases (within 7 days and ideally 1-3 days). Patients with idiopathic ulcers receive long-term anti-ulcer therapy."}, {"id": "pubmed23n1151_11639", "title": "[Peptic ulcer disease and H. pylori gastritis: key advances in clinical management].", "score": 0.015852130325814534, "content": "Helicobacter pylori (H. pylori) gastritis and non-steroidal anti-inflammatory drug (NSAID) intake are the most important risk factors for peptic ulcer disease (PUD) and ulcer bleeding. H. pylori infection was shown to increase the risk of ulcer bleeding in patients with PUD who are taking NSAID, aspirin, or another antiplatelet drug. H. pylori-positive patients on combined platelet aggregation inhibition are at the highest risk of bleeding. Evidence-based interdisciplinary treatment recommendations for the safe use of NSAID have been released. For patients with a moderate risk of PUD, the combination of NSAID and a proton pump inhibitor (PPI) or a monotherapy with a selective cyclooxygenase-2 (COX-2) inhibitor is recommended, whereas patients with a high risk of bleeding should receive a combination of a selective COX-2 inhibitor and a PPI. According to a recent randomized trial, hemodynamically stable patients with signs of upper gastrointestinal bleeding and an increased risk of death (Glasgow-Blatchford Score ≥ 12) undergoing endoscopy 6-24 after consultation do not have any disadvantage in terms of 30-day mortality compared to patients receiving endoscopy within 6 hours. After successful endoscopic hemostasis, additional prophylactic angiographic embolization does not reduce the risk of recurrent bleeding. Successful H. pylori eradication reduces the risk of developing gastric cancer (GC) in first-degree relatives of patients with GC by 73 %. In patients with successful endoscopic treatment of early GC, H. pylori testing with subsequent eradication also halves the rate of metachronous GC. Clarithromycin-based triple therapy for H. pylori eradication shows a decreasing effectiveness due to increasing antibiotic resistance, especially against macrolides. Accordingly, bismuth-containing quadruple therapy is widely recommended as the standard empiric first-line therapy."}, {"id": "pubmed23n0913_18587", "title": "Effect of scheduled second-look endoscopy on peptic ulcer bleeding: a prospective randomized multicenter trial.", "score": 0.015198408312147674, "content": "This study aimed to investigate the effectiveness of scheduled second-look endoscopy (EGD) with endoscopic hemostasis on peptic ulcer rebleeding and to identify the risk factors related to the need for second-look EGD. We prospectively randomized patients who had endoscopically confirmed bleeding peptic ulcer with stigmata of active bleeding, visible vessel, or adherent clot into 2 groups between August 2010 and January 2013. Hemoclip application or thermal coagulation and/or epinephrine injection were allowed for initial endoscopic therapy. The same dosage of proton pump inhibitor was injected intravenously. The study group received scheduled second-look EGD 24 to 36 hours after the initial hemostasis, and further therapy was applied if endoscopic stigmata persisted, as above. Those patients who developed rebleeding underwent operation or radiologic intervention despite the additional endoscopic therapy. Outcome measures included rebleeding, amount of transfusion, duration of hospitalization, and mortality. After initial endoscopic hemostasis, 319 eligible patients were randomized into 2 groups. Sixteen (10.1%) and 9 (5.6%) patients developed rebleeding (P = .132), respectively. There was also no difference in surgical intervention (0, 0% vs 1, .6%, P >.999) or radiologic intervention (3, 1.9% vs 2, 1.2%, P = .683), median duration of hospitalization (6.0 vs 5.0 days, P = .151), amount of transfusion (2.4 ± 1.7 vs 2.2 ± 1.6 units, P = .276), and mortality (2, 1.3% vs 2, 1.2%, P > .999) between the 2 groups. Multivariate analysis showed that grades 3 to 4 of endoscopists' estimation to success of initial hemostasis, history of nonsteroidal anti-inflammatory drug (NSAID) use, and larger amounts of blood transfusions (≥4 units of red blood cells) were the independent risk factors of rebleeding. A single EGD with endoscopic hemostasis is not inferior to scheduled second-look endoscopy in terms of reduction in rebleeding rate of peptic ulcer bleeding. Repeat endoscopy would be helpful in the patients with unsatisfactory initial endoscopic hemostasis, use of NSAIDs, and larger amounts of transfused blood. (Clinical trial registration number: KCT0000565; 4-2010-0348.)."}, {"id": "pubmed23n0844_2936", "title": "Diagnosis and management of nonvariceal upper gastrointestinal hemorrhage: European Society of Gastrointestinal Endoscopy (ESGE) Guideline.", "score": 0.014854426619132503, "content": "This Guideline is an official statement of the European Society of Gastrointestinal Endoscopy (ESGE). It addresses the diagnosis and management of nonvariceal upper gastrointestinal hemorrhage (NVUGIH). Main Recommendations MR1. ESGE recommends immediate assessment of hemodynamic status in patients who present with acute upper gastrointestinal hemorrhage (UGIH), with prompt intravascular volume replacement initially using crystalloid fluids if hemodynamic instability exists (strong recommendation, moderate quality evidence). MR2. ESGE recommends a restrictive red blood cell transfusion strategy that aims for a target hemoglobin between 7 g/dL and 9 g/dL. A higher target hemoglobin should be considered in patients with significant co-morbidity (e. g., ischemic cardiovascular disease) (strong recommendation, moderate quality evidence). MR3. ESGE recommends the use of the Glasgow-Blatchford Score (GBS) for pre-endoscopy risk stratification. Outpatients determined to be at very low risk, based upon a GBS score of 0 - 1, do not require early endoscopy nor hospital admission. Discharged patients should be informed of the risk of recurrent bleeding and be advised to maintain contact with the discharging hospital (strong recommendation, moderate quality evidence). MR4. ESGE recommends initiating high dose intravenous proton pump inhibitors (PPI), intravenous bolus followed by continuous infusion (80 mg then 8 mg/hour), in patients presenting with acute UGIH awaiting upper endoscopy. However, PPI infusion should not delay the performance of early endoscopy (strong recommendation, high quality evidence). MR5. ESGE does not recommend the routine use of nasogastric or orogastric aspiration/lavage in patients presenting with acute UGIH (strong recommendation, moderate quality evidence). MR6. ESGE recommends intravenous erythromycin (single dose, 250 mg given 30 - 120 minutes prior to upper gastrointestinal [GI] endoscopy) in patients with clinically severe or ongoing active UGIH. In selected patients, pre-endoscopic infusion of erythromycin significantly improves endoscopic visualization, reduces the need for second-look endoscopy, decreases the number of units of blood transfused, and reduces duration of hospital stay (strong recommendation, high quality evidence). MR7. Following hemodynamic resuscitation, ESGE recommends early (≤ 24 hours) upper GI endoscopy. Very early (< 12 hours) upper GI endoscopy may be considered in patients with high risk clinical features, namely: hemodynamic instability (tachycardia, hypotension) that persists despite ongoing attempts at volume resuscitation; in-hospital bloody emesis/nasogastric aspirate; or contraindication to the interruption of anticoagulation (strong recommendation, moderate quality evidence). MR8. ESGE recommends that peptic ulcers with spurting or oozing bleeding (Forrest classification Ia and Ib, respectively) or with a nonbleeding visible vessel (Forrest classification IIa) receive endoscopic hemostasis because these lesions are at high risk for persistent bleeding or rebleeding (strong recommendation, high quality evidence). MR9. ESGE recommends that peptic ulcers with an adherent clot (Forrest classification IIb) be considered for endoscopic clot removal. Once the clot is removed, any identified underlying active bleeding (Forrest classification Ia or Ib) or nonbleeding visible vessel (Forrest classification IIa) should receive endoscopic hemostasis (weak recommendation, moderate quality evidence). MR10. In patients with peptic ulcers having a flat pigmented spot (Forrest classification IIc) or clean base (Forrest classification III), ESGE does not recommend endoscopic hemostasis as these stigmata present a low risk of recurrent bleeding. In selected clinical settings, these patients may be discharged to home on standard PPI therapy, e. g., oral PPI once-daily (strong recommendation, moderate quality evidence). MR11. ESGE recommends that epinephrine injection therapy not be used as endoscopic monotherapy. If used, it should be combined with a second endoscopic hemostasis modality (strong recommendation, high quality evidence). MR12. ESGE recommends PPI therapy for patients who receive endoscopic hemostasis and for patients with adherent clot not receiving endoscopic hemostasis. PPI therapy should be high dose and administered as an intravenous bolus followed by continuous infusion (80 mg then 8 mg/hour) for 72 hours post endoscopy (strong recommendation, high quality evidence). MR13. ESGE does not recommend routine second-look endoscopy as part of the management of nonvariceal upper gastrointestinal hemorrhage (NVUGIH). However, in patients with clinical evidence of rebleeding following successful initial endoscopic hemostasis, ESGE recommends repeat upper endoscopy with hemostasis if indicated. In the case of failure of this second attempt at hemostasis, transcatheter angiographic embolization (TAE) or surgery should be considered (strong recommendation, high quality evidence). MR14. In patients with NVUGIH secondary to peptic ulcer, ESGE recommends investigating for the presence of Helicobacter pylori in the acute setting with initiation of appropriate antibiotic therapy when H. pylori is detected. Re-testing for H. pylori should be performed in those patients with a negative test in the acute setting. Documentation of successful H. pylori eradication is recommended (strong recommendation, high quality evidence). MR15. In patients receiving low dose aspirin for secondary cardiovascular prophylaxis who develop peptic ulcer bleeding, ESGE recommends aspirin be resumed immediately following index endoscopy if the risk of rebleeding is low (e. g., FIIc, FIII). In patients with high risk peptic ulcer (FIa, FIb, FIIa, FIIb), early reintroduction of aspirin by day 3 after index endoscopy is recommended, provided that adequate hemostasis has been established (strong recommendation, moderate quality evidence). "}, {"id": "pubmed23n0727_12350", "title": "Effect of intravenous proton pump inhibitor regimens and timing of endoscopy on clinical outcomes of peptic ulcer bleeding.", "score": 0.014685679094225211, "content": "The most effective schedule of proton pump inhibitor (PPI) administration and the optimal timing of endoscopy in acute peptic ulcer bleeding remain uncertain. The aim of this study was to determine the most efficient PPI regimen and optimal timing of endoscopy.   Consecutive patients with suspected bleeding peptic ulcers were enrolled and randomized to receive either a standard regimen or a high-dose intensive intravenous regimen. Only patients with bleeding peptic ulcers diagnosed at initial endoscopy continued the study. High-risk patients received endoscopic hemostasis. The primary outcome measure of recurrent bleeding was compared between the two dosage regimens and between early and late endoscopy. Secondary outcome measures compared included need for endoscopic treatment, blood transfusion, hospital stay, surgery and mortality. A total of 875 patients completed the study. Recurrent bleeding occurred in 11.0% in the standard regimen group, statistically higher than that in the intensive regimen group (6.4%, P=0.02). Mean units of blood transfused and duration of hospital stay were also higher in the standard regimen group (P<0.001 for each compared to intensive regimen group). However, no significant differences were noted between the two groups in the need for endoscopic hemostasis, need for surgery, and mortality. Recurrence of bleeding was similar between the early and late endoscopy groups. Units of blood transfused and length of hospital stay were both significantly reduced with early endoscopy. High-dose PPI infusion is more efficacious in reducing rebleeding rate, blood transfusion requirements and hospital stay. Early endoscopy is safe and more effective than late endoscopy."}, {"id": "pubmed23n0984_11238", "title": "Management of acute upper gastrointestinal bleeding.", "score": 0.014664943328693576, "content": "Upper gastrointestinal bleeding (UGIB) is a common medical emergency, with a reported mortality of 2-10%. Patients identified as being at very low risk of either needing an intervention or death can be managed as outpatients. For all other patients, intravenous fluids as needed for resuscitation and red cell transfusion at a hemoglobin threshold of 70-80 g/L are recommended. After resuscitation is initiated, proton pump inhibitors (PPIs) and the prokinetic agent erythromycin may be administered, with antibiotics and vasoactive drugs recommended in patients who have cirrhosis. Endoscopy should be undertaken within 24 hours, with earlier endoscopy considered after resuscitation in patients at high risk, such as those with hemodynamic instability. Endoscopic treatment is used for variceal bleeding (for example, ligation for esophageal varices and tissue glue for gastric varices) and for high risk non-variceal bleeding (for example, injection, thermal probes, or clips for lesions with active bleeding or non-bleeding visible vessel). Patients who require endoscopic therapy for ulcer bleeding should receive high dose proton pump inhibitors after endoscopy, whereas those who have variceal bleeding should continue taking antibiotics and vasoactive drugs. Recurrent ulcer bleeding is treated with repeat endoscopic therapy, with subsequent bleeding managed by interventional radiology or surgery. Recurrent variceal bleeding is generally treated with transjugular intrahepatic portosystemic shunt. In patients who require antithrombotic agents, outcomes appear to be better when these drugs are reintroduced early."}, {"id": "wiki20220301en003_42443", "title": "Peptic ulcer disease", "score": 0.014552152889707575, "content": "Early endoscopic therapy can help to stop bleeding by using cautery, endoclip, or epinephrine injection. Treatment is indicated if there is active bleeding in the stomach, visible vessels, or an adherent clot. Endoscopy is also helpful in identifying people who are suitable for hospital discharge. Prokinetic agents such as erythromycin and metoclopramide can be given before endoscopy to improve endoscopic view. Either high- or low-dose PPIs are equally effective in reducing bleeding after endoscopy. High-dose intravenous PPI is defined as a bolus dose of 80 mg followed by an infusion of 8 mg per hour for 72 hours—in other words, the continuous infusion of PPI of greater than 192 mg per day. Intravenous PPI can be changed to oral once there is no high risk of rebleeding from peptic ulcer."}, {"id": "pubmed23n0509_2706", "title": "Management of bleeding peptic ulcer: current status of intravenous proton pump inhibitors.", "score": 0.014463241436925647, "content": "Over 80% of ulcer bleeding stops spontaneously, but associated mortality and morbidity remain high. Occurrence of re-bleeding increases mortality 10-fold. Endoscopic findings in those that have bled predict the risk of recurrent bleeding. Patients whose ulcers show a 'flat dot' or clean base (Forrest Class 3) rarely rebleed or need hospitalization. However, actively bleeding ulcers or those with evidence of recent hemorrhage (Forrest Classes 1 and 2) are likely to re-bleed and may need intensive care. Meta-analyses indicate that endoscopic hemostasis has reduced re-bleeding and surgical intervention by over 60% and mortality by 45%. Beyond these reductions, data indicate that (unlike H2-receptor antagonists use, largely devoid of benefits in this area) continuous intravenous infusions of high doses of proton pump inhibitors reduce rebleeding, re-endoscopy, blood transfusion and surgical intervention, but have little effect (beyond endoscopic therapy) on associated mortality, much of it due to conditions other than rebleeding."}, {"id": "pubmed23n0657_25148", "title": "Proton pump inhibitors after endoscopic hemostasis in patients with peptic ulcer bleeding.", "score": 0.014361300075585788, "content": "Peptic ulcer bleeding is a common and potentially fatal condition. For patients with bleeding peptic ulcers that display major endoscopic stigmata of recent hemorrhage, a combination of endoscopic and pharmacologic therapy is the current standard management. To show our experience with management of peptic ulcer bleeding. Patients who presented with gastrointestinal bleeding caused by peptic ulcer or recent history (< 24 h before presentation) of hematemesis and/or melena admitted to our hospital emergency departments, and patients whose ulcer hemorrhage started after hospitalization for an unrelated medical or surgical condition. Patients with actively bleeding ulcers and those with nonbleeding visible vessel or adherent clot were treated with epinephrine injection and/or endoscopic hemoclips, and randomized to receive intravenous pantoprasole according to the continuous regimen (dose of 5 x 40 mg in continuous infusion of 8 mg/h for 72 h) or the standard regimen (40 mg bolus of PPI twice daily for 3 days). After the infusion, all patients were given 40 mg PPI twice daily orally. The primary end point was the in-hospital rebleeding rate, as discovered by the repeated endoscopy. Bleeding recurred in 5 of 34 patients (14.7%) receiving the intensive regimen, and in 8 of 35 (22.8%) patients receiving the standard regimen. Hemoglobin (g/l) rate in standard regimen group was 93.5 +/- 23.8, and in intensive regimen group 106.6 +/- 22.4 (P = 0.042). Mean units of blood transfused for all patients in group were 71.8 +/- 45.8 in the intensive and 45.3 +/- 50.2 in the standard regimen group (P = 0.0257). The duration of hospital stay was 6.4 +/- 2.8 in standard group and 5.8 +/- 2.8 in the intensive group (P = 0.40). In patients with bleeding peptic ulcers with successful endoscopic hemostasis the standard PPI regimen had advantage on transfusion requirements, but no advantage with respect to in-hospital rates of rebleeding rates, need for surgery, length of hospital stay, or death, which corresponds with recent studies."}, {"id": "wiki20220301en030_8104", "title": "Gastrointestinal bleeding", "score": 0.014317032794953246, "content": "The benefits versus risks of placing a nasogastric tube in those with upper GI bleeding are not determined. Endoscopy within 24 hours is recommended, in addition to medical management. A number of endoscopic treatments may be used, including: epinephrine injection, band ligation, sclerotherapy, and fibrin glue depending on what is found. Prokinetic agents such as erythromycin before endoscopy can decrease the amount of blood in the stomach and thus improve the operators view. They also decrease the amount of blood transfusions required. Early endoscopy decreases hospital and the amount of blood transfusions needed. A second endoscopy within a day is routinely recommended by some but by others only in specific situations. Proton pump inhibitors, if they have not been started earlier, are recommended in those in whom high risk signs for bleeding are found. High and low dose PPIs appear equivalent at this point. It is also recommended that people with high risk signs are kept in hospital"}, {"id": "pubmed23n0870_16286", "title": "High-dose omeprazole infusion compared with scheduled second-look endoscopy for prevention of peptic ulcer rebleeding: a randomized controlled trial.", "score": 0.014296791703014441, "content": "Previous studies have shown that both scheduled second-look endoscopy and high-dose continuous omeprazole infusion are effective in preventing peptic ulcer rebleeding. The aim of this noninferiority trial was to compare the efficacy of these two strategies for the prevention of rebleeding following primary endoscopic hemostasis. Consecutive patients who received endoscopic treatment for bleeding peptic ulcers (actively bleeding, with nonbleeding visible vessels) were randomized to two treatment groups following hemostasis. One group (second-look endoscopy group) received the proton pump inhibitor (PPI) omeprazole as an intravenous bolus every 12 hours for 72 hours and a second endoscopy within 16 - 24 hours with retreatment for persistent stigmata of bleeding. The other group (PPI infusion group) received continuous high-dose omeprazole infusion for 72 hours. Patients who developed rebleeding underwent surgery if repeat endoscopic therapy failed. The primary outcome was the rebleeding rate within 30 days after initial hemostasis. The margin for noninferiority was set at 5 %. A total of 153 patients were randomized to the PPI infusion group and 152 to the second-look endoscopy group. Rebleeding occurred within 30 days in 10 patients (6.5 %) in the PPI infusion group and in 12 patients (7.9 %) in the second-look endoscopy group (P = 0.646). Surgery was required for rebleeding in six patients from the PPI infusion group and three patients in the second-look endoscopy group (P = 0.32). Intensive care unit stay, transfusion requirements, and mortality were not different between the groups. Patients in the second-look endoscopy group were discharged 1 day earlier than those in the PPI infusion group (P < 0.001). After endoscopic hemostasis, high-dose PPI infusion was not inferior to second-look endoscopy with bolus PPI in preventing peptic ulcer rebleeding. ClinicalTrials.gov (NCT: 00164931)."}, {"id": "pubmed23n0643_25160", "title": "[Guidelines of treatment for bleeding peptic ulcer disease].", "score": 0.01418725159732354, "content": "Peptic ulcer (PU) bleeding is the main cause of non-variceal gastrointestinal bleeding. Negative outcomes include re-bleeding and death, and many of the deaths are associated with decompensation of coexisting medical conditions precipitated by acute bleeding event. Accurate analysis of risk for clinical features can help physician to decide treatment modality. Endoscopy can detect bleeding stigmata and perform therapeutic hemostasis. Proton pump inhibitor (PPI) compared with placebo or H2RA reduces mortality following PU bleeding among patients with high-risk endoscopic findings, and reduces re-bleeding rates and surgical intervention. PPI treatment initiated prior to endoscopy in upper gastrointestinal (UGI) bleeding significantly reduces the proportion of patients with stigmata of recent hemorrhage (SRH) at index endoscopy but does not reduce mortality, re-bleeding or the need for surgery. The strategy of giving oral PPI before and after endoscopy, with endoscopic hemostasis for those with major SRH, is likely to be the most cost-effective. The treatment of H. pylori infection was found to be more effective than anti-secretory therapy in preventing recurrent bleeding from PU. H. pylori eradication alone and eradication followed by misoprostol (with switch to PPI, if misoprostol is not tolerated) are the two most cost-effective strategies to prevent ulcer bleeding among H. pylori-infected NSAID users, although the data cannot exclude PPIs also being cost-effective treatment. This review focuses specifically on the current treatment of patients with acute bleeding from a peptic ulcer."}, {"id": "pubmed23n0952_7274", "title": "Comparison of High Dose and Standard Dose Proton Pump Inhibitor before Endoscopy in Patients with Non-Portal Hypertension Bleeding.", "score": 0.014085159616364732, "content": "Gastrointestinal bleeding with non-portal hypertension bleeding (non-PHT) is the most common cause of gastrointestinal emergencies with high mortality rate. The majority of non-PHT patient stem from acid related disease. The practice guideline recommends using pre-endoscopic proton pump inhibitors (PPIs). However, the dose and route of PPIs administration were still unclear according to the Association for Gastroenterology. To compare the efficiency of PPIs between high dose and standard dose before endoscopy in patients suffering with gastrointestinal bleeding due to non-PHT. The present study was designed as a prospective, randomized controlled trial. The patients were randomly categorized into two groups, the first group received intravenous pantoprazole 80 mg bolus then continuously drip 8 mg per hour (high dose group) and the other group received intravenous pantoprazole 40 mg twice daily before endoscopy (standard dose group). Baseline characteristics, Blatchford score, endoscopic findings, morbidity, and other complications were recorded. One hundred thirteen patients were recruited. Fifty-eight patients were in the high dose group and 55 patients in the standard dose group. Blatchford scores in the high dose group were slightly higher than the standard dose group but there was no statistically significant difference (12.49+3.29 and 12.38+4.06, respectively, p = 0.876). Twenty-two patients were high-risk for peptic ulcer bleeding from endoscopy. There were significantly less numbers of patient who were high-risk of peptic ulcer bleeding in the high dose group compared to the standard dose group (10 patients [17.24%] and 12 patients [21.82%], respectively, p = 0.025). There was no difference between the two groups in average time of hospital stay (3.03 and 2.89 days, respectively, p>0.05), mean unit of blood transfusion (1.79 and 1.63 units, respectively, p>0.05), and the complications after endoscopy such as recurrent bleeding (0 and 1 patient, respectively, p>0.05), recurrent bleeding and death (0 and 1 patient, respectively, p>0.05). The Blatchford score greater than 10, 11, and 12 showed high sensitivity of 100%, 95%, and 95% respectively with negative predictive values (NPV) of 100%, 97%, and 97% respectively, in predicting high-risk peptic ulcer bleeding. The high dose of PPIs administration before endoscopy reduced the chance of high-risk peptic ulcer bleeding compared to the standard dose. Both high dose and standard dose of PPIs did not affect the time of hospital stay, unit of blood transfusion, the complications after endoscopy, and mortality rate. Standard dose PPIs can be considered using in patients with Blatchford scores lower than 10. High dose PPIs would be beneficial in patients who have Blatchford scores between 10 and 12. For patients who have Blatchford scores greater than 12, high dose PPIs are recommended."}, {"id": "pubmed23n0725_606", "title": "Randomized controlled trial of high dose bolus versus continuous intravenous infusion pantoprazole as an adjunct therapy to therapeutic endoscopy in massive bleeding peptic ulcer.", "score": 0.013921786629284092, "content": "After therapeutic endoscopy is performed in high-risk patients with peptic ulcer bleeding, rebleeding occurs in about 25 to 30%. High dose intravenous proton pump inhibitors (PPI) have been recommended for the use in high-risk patients to prevent rebleeding following successful therapeutic endoscopy. Compare the efficacy between pantoprazole high dose bolus injections and continuous intravenous infusion to prevent rebleeding in peptic ulcer patients after initial hemostasis is achieved by the therapeutic endoscopy. A clinical block randomized control trial was conducted at Maharaj Nakorn Chiang Mai Hospital in massive peptic ulcer bleeding patients. All patients underwent endoscopic diagnosis and treatment within six hours of admission. Hemostasis was achieved by therapeutic endoscopy in 28 patients who received 80 mg pantoprazole as a loading dose before intervention. They were randomized into two groups. The first group was given a high dose of pantoprazole, 40 mg bolus injections twice daily for seven days (n = 13). The second group was given continuous intravenous infusion of pantoprazole, 8 mg per hour for the first three days, followed with a 40 mg bolus injection twice daily similar to the first group from day 4 until day 7 (n = 15). After the seventh day, both groups were given 20 mg of oral pantoprazole once daily for two months. The data was analyzed by Fisher's exact test to compare the frequency of rebleeding within seven days after therapeutic endoscopy. The frequency of recurrent bleeding between the high dose pantoprazole bolus injection group and the continuous intravenous infusion group was not significantly different, 30.8% and 33.3% respectively (p = 1.0). Three patients in the high dose bolus group and five in the continuous infusion group underwent surgery (p = 0.68). There was no statistically significant difference between the two groups by volume of blood transfusion, length of hospital stay, or mortality. In the present study, both PPI drug administration methods showed an equally effective for massive peptic ulcer bleeding. Further studies with a larger sample size are recommended."}, {"id": "wiki20220301en021_42384", "title": "Upper gastrointestinal bleeding", "score": 0.013847209122867188, "content": "Significant upper gastrointestinal bleeding is considered a medical emergency. Fluid replacement, as well as blood transfusion, may be required. Endoscopy is recommended within 24 hours and bleeding can be stopped by various techniques. Proton pump inhibitors are often used. Tranexamic acid may also be useful. Procedures (such as TIPS for variceal bleeding) may be used. Recurrent or refractory bleeding may lead to need for surgery, although this has become uncommon as a result of improved endoscopic and medical treatment. Upper gastrointestinal bleeding affects around 50 to 150 people per 100,000 a year. It represents over 50% of cases of gastrointestinal bleeding. Depending on its severity, it carries an estimated mortality risk of 11%."}, {"id": "wiki20220301en030_8098", "title": "Gastrointestinal bleeding", "score": 0.013761097659402744, "content": "Peptic ulcers Based on evidence from people with other health problems crystalloid and colloids are believed to be equivalent for peptic ulcer bleeding. Proton pump inhibitors (PPI) may reduce mortality in those with severe disease as well as the risk of re-bleeding and the need for surgery among this group. Oral and intravenous formulations may be equivalent; however, the evidence to support this is suboptimal. In those with less severe disease and where endoscopy is rapidly available, they are of less immediate clinical importance. There is tentative evidence of benefit for tranexamic acid which inhibits clot breakdown. Somatostatin and octreotide, while recommended for varicial bleeding, have not been found to be of general use for non variceal bleeds. After treatment of a high risk bleeding ulcer endoscopically giving a PPI once or a day rather than as an infusion appears to work just as well and is less expensive (the method may be either by mouth or intravenously)."}, {"id": "pubmed23n0557_9528", "title": "Does adding misoprostol to standard intravenous proton pump inhibitor protocol improve the outcome of aspirin/NSAID-induced upper gastrointestinal bleeding?: a randomized prospective study.", "score": 0.013343937787203958, "content": "Aspirin and nonsteroidal anti-inflammatory drug (NSAID)-induced gastrointestinal bleeding is recognized as an important health problem. We performed a single-center randomized clinical trial to compare the effect of high-dose intravenous proton pump inhibitor (omeprazole) alone (group 1) with omeprazole in combination with a low-dose prostaglandin analog (misoprostol; group 2) on clinical outcomes in patients with aspirin/NSAID-induced upper gastrointestinal bleeding. Additionally, we evaluated the contribution of Helicobacter pylori eradication therapy on the late consequences. Patients were recruited to the study if they had upper gastrointestinal bleeding with history of taking aspirin or other NSAIDs within the week before the onset of bleeding. All were evaluated in terms of probable risk factors. After the standard treatment protocol, patients with histologically proven H pylori infection were prescribed a triple eradication therapy for 14 days. The primary end points were recurrent bleeding, surgery requirement, and death rates before discharge and at the end of follow-up period. This study lasted for 2 years. A total of 249 patients with upper gastrointestinal bleeding were admitted, and 49.7% of these patients were users of aspirin/NSAIDs. There were 67 patients in group 1 and 56 in group 2. The distributions for gender, age, comorbidity, H pylori infection, and high-risk ulcer rate were similar in both groups. Among aspirin/NSAID users, endoscopy revealed duodenal ulcer in 47 (38.2%), gastric ulcer in 10 (8.1%), and erosive gastropathy in 33 (26.8%). The overall rebleeding occurred in 12.2%, death in 2.4% of the patients. The in-hospital death (P=.414), rebleeding (P=.925), and surgery (P=.547) rates were similar in both treatment groups. After the follow-up period of 3 months, overall rebleeding occurred in 4.1%, and death in 4.8% of the patients. The overall mortality rate was highest in those >65 years old, who were chronic low-dose aspirin users with comorbidity. One died of transfusion-related graft-versus-host disease. In this pilot study, we indicated that adding misoprostol (600 microg/day) to standardized proton pump inhibitor treatment did not improve or change the rebleeding or mortality rates of patients with upper gastrointestinal bleeding related to aspirin/NSAID use. Other prospective studies on higher doses of misoprostol are needed to establish the coeffect. One should bear in mind that all blood products must be irradiated before transfused to the host."}, {"id": "pubmed23n0736_23869", "title": "Management of non-variceal upper gastrointestinal bleeding.", "score": 0.013341913341913342, "content": "Upper gastrointestinal bleeding (UGIB) is a critical condition that demands a quick and effective medical management. Non-variceal UGIB, especially peptic ulcer bleeding is the most significant cause. Appropriate assessment and treatment have a major influence on the prognosis of patients with UGIB. Initial fluids resuscitation and/or transfusion of red blood cells are necessary in patients with clinical evidence of intravascular volume depletion. Endoscopy is essential for diagnosis and treatment of UGIB, and should be provided within 24 hours after presentation of UGIB. Pre-endoscopic use of intravenous proton pump inhibitor (PPI) can downstage endoscopic signs of hemorrhage. Post-endoscopic use of high-dose intravenous PPI can reduce the risk of rebleeding and further interventions such as repeated endoscopy and surgery. Eradication of Helicobacter pylori and withdrawal of non-steroidal anti-inflammatory drugs are recommended to prevent recurrent bleeding."}, {"id": "pubmed23n0477_23763", "title": "Diagnostic and therapeutic options in the management of nonvariceal upper gastrointestinal bleeding.", "score": 0.013131835012486007, "content": "Upper gastrointestinal bleeding from peptic ulcers is common. Advances in prognostication, therapeutic endoscopy, and medical management have evolved rapidly. Patients most likely to rebleed after therapy can now be identified and monitored more closely, and patients with ulcers of low risk for rebleeding can be managed on an outpatient basis. High-risk patients include those with ulcers containing a visible vessel or who are actively bleeding. Endoscopic therapy is mandatory in high-risk patients and involves at least two hemostatic techniques. Second-look endoscopy and repeated hemostasis should be performed promptly in patients who rebleed. Adjunctive treatment includes intravenous proton pump inhibitor administered in high doses for the first 72 hours after endoscopic therapy. Further studies are needed to determine the optimal combination of hemostatic techniques to better target patients who are at risk for ulcer rebleeding."}, {"id": "pubmed23n0494_22487", "title": "Initial factors predicting rebleeding and death in bleeding peptic ulcer disease.", "score": 0.012922589520196755, "content": "Bleeding peptic ulcer constitutes approximately half of the cases admitted with upper gastrointestinal bleeding. Although the bleeding episode stops spontaneously in most of them, rebleeding occurs in as much as 10-30% of them and has a mortality rate of 5-10%. In this study, we have evaluated the possible significant predictors associated with this adverse outcome. The records of 205 patients admitted to gastrointestinal bleeding unit (GIBU) in Riyadh Central Hospital, during the period May 1996 through to April 1999, with endoscopic confirmed diagnosis of bleeding peptic ulcer disease were reviewed for demography, clinical presentation, hematology, biochemistry, initial blood pressure, nasogastric lavage color, co-morbid disease and endoscopic findings. All the significant factors found initially (P<0.05) were entered into odds ratio and its 95% confidence interval and finally the unconditioned logistic regression model was used to find out the significant independent predictors for both rebleeding and mortality in these patients. The majority of patients (85%) were males and below the age of 60 (73%). Duodenal ulcer was the source of bleeding in 84%. Endoscopy was performed in all patients within 24 hours of admission. Only 15% were actively bleeding at the time of initial endoscopy. Thirty-six patients (17%) rebelled, majority within 72 hours of initial hemostasis. Overall, 11 patients (5%) died, 6 of them were rebleeders. Initial presentation of systolic blood pressure <100 mm Hg, blood in nasogastric tube and visible vessel within the ulcer in endoscopy were independent predictors of rebleeding while initial systolic blood pressure <100 mm Hg and age >60-years were independent predictors of mortality. Improvement of outcome in patients with bleeding peptic ulcer disease can be achieved by early detection of those patients who are at risk of adverse outcome. Patients with the above mentioned independent predictors of rebleeding and mortality are best managed in the intensive care unit with endoscopic hemostasis and proton pump inhibitor (PPI) therapy for a minimum of 5-days of admission."}, {"id": "pubmed23n0521_13701", "title": "[Endoscopic treatment of bleeding ulcers: has everything been said and done?].", "score": 0.012694145758661887, "content": "Endoscopic treatment reduces bleeding recurrence, the need for surgery and mortality in patients with bleeding ulcers. However endoscopic treatment fails in 10-15% of patients, leading to high morbidity and mortality. The therapeutic measures with demonstrated effectiveness in reducing the risk of hemorrhagic recurrence and its complications are combined endoscopic treatment (adrenaline plus a second hemostatic intervention) and proton pump inhibitors. Also useful, although there is less evidence, are immediate resuscitation and <<second look>> endoscopy. Some studies suggest that activated recombinant factor VII infusion or supra-selective arterial embolization can be useful in severe hemorrhage. Further studies are required to determine optimal treatment according to the characteristics of each patient."}, {"id": "wiki20220301en021_42396", "title": "Upper gastrointestinal bleeding", "score": 0.012559630292188432, "content": "The benefits versus risks of placing a nasogastric tube in those with upper gastrointestinal bleeding are not determined. Endoscopy within 24 hours is recommended. Prokinetic agents such as erythromycin before endoscopy can decrease the amount of blood in the stomach and thus improve the operators view. Early endoscopy decreases hospital time and the amount of blood transfusions needed. Proton pump inhibitors, if they have not been started earlier, are recommended in those in whom high risk signs for bleeding are found. It is also recommended that people with high risk signs are kept in hospital for at least 72 hours."}, {"id": "pubmed23n0994_16011", "title": "The recurrent bleeding risk of a Forrest IIc lesion at the second-look endoscopy can be indicated by high Rockall scores ≥ 6.", "score": 0.012343820562998646, "content": "The Forrest classification is widely applied to guide endoscopic hemostasis for peptic ulcer bleeding. Accordingly, practice guidelines suggest medical treatment only for ulcer with a Forrest IIc lesion because it has low rebleeding risk even without endoscopic therapy, ranging from 0 to 13%. However, the risk ranges widely and it is unclear who is at risk of rebleeding with such a lesion. This study assessed whether the Rockall score, which evaluates patients holistically, could indicate the risk of recurrent bleeding among patients with a Forrest IIc lesion at the second-look endoscopy. Patients who had peptic ulcer bleeding with Ia-IIb lesions received endoscopic hemostasis at the primary endoscopy, and they were enrolled if their Ia-IIb lesions had been fading to IIc at the second-look endoscopy after 48- to 72-h intravenous proton pump inhibitor (PPI) infusion. Primary outcomes were rebleeding during the 4th-14th day and 490% of cases. Inoculation of synovial fluid into bottles containing liquid media for blood cultures increases the yield of a culture, especially if the pathogen is a fastidious organism or the patient is taking an antibiotic. NAA-based assays for bacterial DNA, when available, can be useful for the diagnosis of partially treated or culture-negative bacterial arthritis."}, {"id": "pubmed23n0798_15491", "title": "Microorganisms and their sensitivity pattern in septic arthritis of north Indian children: a prospective study from tertiary care level hospital.", "score": 0.010732323232323232, "content": "Background. Septic arthritis is a true orthopaedic emergency. Important factors determining outcome are rapid diagnosis and timely intervention. Changing trends in microbiological spectrum and emerging drug resistance poses big challenge. Present study evaluates bacterial strains and their sensitivity pattern in septic arthritis of North Indian children. Methods. Fifty children with septic arthritis of any joint were evaluated. Joint was aspirated and 2 cc of aspirated fluid was sent for gram stain and culture. Blood cultures were also sent for bacteriological evaluation. Results. Fifty percent cases had definite radiological evidence of septic arthritis whereas ultrasound revealed fluid in 98% cases. Aspirated fluid showed isolates in 72% cases. The most common organism was Staphylococcus aureus (62%) followed by Streptococcus pneumoniae and Gr. B Streptococcus. Blood culture could grow the organism in 34% cases only. The bacterial strain showed significant resistance to common antibiotic cocktail in routine practice. Resistance to cloxacillin and ceftriaxone was 62% and 14% respectively. No organisms were resistant to vancomycin and linezolid. Conclusion. S. aureus is still the most common organism in septic arthritis. Though a significant resistance to common antibiotic cocktail is noticed, the strain is susceptible to higher antibiotics. We recommend using these antibiotics as an empirical therapy till culture and sensitivity report is available. "}, {"id": "pubmed23n1115_611", "title": "Septic Arthritis: Diagnosis and Treatment.", "score": 0.010498759988977681, "content": "Septic arthritis must be considered and promptly diagnosed in any patient presenting with acute atraumatic joint pain, swelling, and fever. Risk factors for septic arthritis include age older than 80 years, diabetes mellitus, rheumatoid arthritis, recent joint surgery, hip or knee prosthesis, skin infection, and immunosuppressive medication use. A delay in diagnosis and treatment can result in permanent morbidity and mortality. Physical examination findings and serum markers, including erythrocyte sedimentation rate and C-reactive protein, are helpful in the diagnosis but are nonspecific. Synovial fluid studies are required to confirm the diagnosis. History and Gram stain aid in determining initial antibiotic selection. Staphylococcus aureus is the most common pathogen isolated in septic arthritis; however, other bacteria, viruses, fungi, and mycobacterium can cause the disease. After synovial fluid has been obtained, empiric antibiotic therapy should be initiated if there is clinical concern for septic arthritis. Oral antibiotics can be given in most cases because they are not inferior to intravenous therapy. Total duration of therapy ranges from two to six weeks; however, certain infections require longer courses. Consideration for microorganisms such as Neisseria gonorrhoeae, Borrelia burgdorferi, and fungal infections should be based on history findings and laboratory results."}, {"id": "wiki20220301en000_195395", "title": "Gram stain", "score": 0.010439951229424912, "content": "Medical Gram stains are performed on body fluid or biopsy when infection is suspected. Gram stains yield results much more quickly than culturing, and are especially important when infection would make an important difference in the patient's treatment and prognosis; examples are cerebrospinal fluid for meningitis and synovial fluid for septic arthritis. Staining mechanism"}, {"id": "article-29494_15", "title": "Sternoclavicular Joint Infection -- Evaluation", "score": 0.010225885225885226, "content": "The gold standard for diagnosing sternoclavicular joint septic arthritis remains arthrocentesis. Aspirated joint fluid should undergo evaluation for crystals, cell count, percentage of neutrophils, and the presence of bacteria using gram stain and culture. A total nucleated cell count greater than 50000 with over 90% neutrophils with no crystals is concerning for infection. A positive gram stain or culture is virtually diagnostic regardless of the other synovial fluid analyses."}, {"id": "article-17855_12", "title": "Gonococcal Arthritis -- Evaluation", "score": 0.010213735862039306, "content": "In patients who demonstrate joint swelling and effusion, arthrocentesis and synovial fluid analysis should be performed. Typically, a synovial fluid analysis will show a white blood cell (WBC) count of 50,000 cells/mm3 or more; although, a cell count below 10,000 cells/mm3 is not uncommon. This may be associated with reduced glucose concentration and elevated LDH levels but these findings are non-diagnostic and of limited value. In patients who present with localized purulent arthritis, N. gonorrhoeae will be isolated in only about 50% (range of 25% to 75%) of synovial fluid specimens. This is even less in patients who present with the arthritis-dermatitis syndrome. NAAT of synovial fluid is more sensitive (greater than 75%) than culture and should be performed if available."}, {"id": "InternalMed_Harrison_10407", "title": "InternalMed_Harrison", "score": 0.010211357270180799, "content": "True gonococcal septic arthritis is less common than the DGI syndrome and always follows DGI, which is unrecognized in one-third of patients. A single joint such as the hip, knee, ankle, or wrist is usually involved. Synovial fluid, which contains >50,000 leukocytes/μL, can be obtained with ease; the gonococcus is evident only occasionally in Gram-stained smears, and cultures of synovial fluid are positive in fewer than 40% of cases. Blood cultures are almost always negative."}]}}}} {"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 135]], "word_ranges": [[0, 21]], "text": "Severe aplastic anemia and not a candidate for allogeneic transplant because of his age. Antisuppressants seem to be the most suitable."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Severe aplastic anemia and not a candidate for allogeneic transplant because of his age. Antisuppressants seem to be the most suitable.", "full_answer_no_ref": "Severe aplastic anemia and not a candidate for allogeneic transplant because of his age. Antisuppressants seem to be the most suitable.", "full_question": "A 71-year-old man presenting with severe pancytopenia without presence of immature cells and with a bone marrow study suggestive of severe aplastic anemia. What would be the fundamental therapeutic approach?", "id": 235, "lang": "en", "options": {"1": "Treatment with methylprednisolone at a dose of 1 g/kg/day for 5 days.", "2": "Study of siblings and if any of them is HLA compatible, allogeneic transplantation of hematopoietic progenitors.", "3": "Immunosuppressive therapy with cyclosporine and antithymocyte immunoglobulin.", "4": "Hemotherapy support.", "5": "Chemotherapy and if response autologous transplantation of hematopoietic progenitors."}, "question_id_specific": 107, "type": "HEMATOLOGY", "year": 2014, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0413_21773", "title": "Antithymocyte globulin and cyclosporine for severe aplastic anemia: association between hematologic response and long-term outcome.", "score": 0.017720530835284937, "content": "In most patients, aplastic anemia results from T-cell-mediated immune destruction of bone marrow. Aplastic anemia can be effectively treated by stem cell transplantation or immunosuppression. To assess long-term outcomes after immunosuppressive therapy. Cohort of 122 patients (31 were < or =18 years and 91 were >18 years) with severe aplastic anemia, as determined by bone marrow cellularity and blood cell count criteria, were enrolled in a single-arm interventional research protocol from 1991 to 1998 at a federal government research hospital. A dose of 40 mg/kg per day of antithymocyte globulin administered for 4 days, 10 to 12 mg/kg per day of cyclosporine for 6 months (adjusted for blood levels), and a short course of corticosteroids (1 mg/d of methylprednisolone for about 2 weeks). Survival, improvement of pancytopenia and transfusion-independence, relapse, and evolution to other hematologic diseases. Response rates were 60% at 3 months after initiation of treatment, 61% at 6 months, and 58% at 1 year. The blood cell counts of patients who responded no longer satisfied severity criteria and they were transfusion-independent. Overall actuarial survival at 7 years was 55%. Survival was associated with early satisfaction of response criteria (86% vs 40% at 5 years; P<.001) and by blood counts at 3 months (reticulocyte count or platelet count of >50 x 10(3)/ microL predicted survival at 5 years of 90% [64/71] vs 42% [12/34] for patients with less robust recovery [P<.001 by log-rank test]). There were no deaths among responders more than 3 years after treatment. Relapse was common, but severe pancytopenia usually did not recur. Relapse did not influence survival. Thirteen patients showed evolution to other hematologic diseases, including monosomy 7. Approximately half of patients with severe aplastic anemia treated with antithymocyte globulin and cyclosporine have durable recovery and excellent long-term survival. These outcomes were related to the quality of hematologic recovery."}, {"id": "pubmed23n0371_15932", "title": "[Second allogenic bone marrow transplantation after late graft rejection in a patient with severe aplastic anemia].", "score": 0.017105263157894735, "content": "Allogeneic bone marrow transplantation (BMT) is the treatment of choice in young patients (pts) with severe aplastic anemia (SAA) who have an HLA identical sibling donor. Late graft rejection to following allogeneic BMT for SAA is a significant clinical problem and is associated with a high risk of mortality. The optimal treatment strategy for patients with late graft rejection after first BMT is still an open question. We report 12-year-old patient with acquired SAA who underwent BMT from his HLA identical sister. BMT was first-line treatment within 3 months of diagnosis. Preparative therapy was Cyclophosphamide (Cy) 200 mg/kg body mass (BM) during 4 days. Graft versus host disease (GVHD) was prevented with Methotrexate (MTX), Methylprednisolone (MPDN) and Cyclosporin A (CsA). After 17 months, during which patient was with normal blood counts and full donor chimaerism, graft rejection occurred. The patient was re-engrafted from the same donor after conditioning with Cy 200 mg/kg BM plus horse antithymocyte globulin (ATG)--2 vials (á 25 mg)/10 kg BM over 4 days. Before the collection, donor's bone marrow was activated with low dose rhGM-CSF (3 micrograms/kg one day). Following a secondary BMT engraftment has sustained. The patient is alive with full donor chimaerism 26 months from secondary transplantation, without acute or chronic GVHD."}, {"id": "pubmed23n0537_19197", "title": "[Hematopoietic stem-cell transplantation in aplastic anemia].", "score": 0.01625226625226625, "content": "Severe aplastic anemia is a rare syndrome characterized by bone marrow failure with cytopenias and hypocellular bone marrow biopsy (usually 10-15%), without blasts or myelodysplasia. The first choice treatment for these patients is allogeneic bone marrow transplantation from a sibling matched for HLA-A, HLA-B and HLA-DR. Unfortunately only 30% of patients have an HLA-matched sibling (a 25% chance per sibling). The alternative treatment for severe aplastic anemia for the rest of the patients (70%) is immunosuppression with antithymocyte globuline and cyclosporine. The evolution of bone marrow transplantation since 1970's has been positive in terms of survival and transplant success (initial overall survival 43% vs. 90% lately, and graft rejection of 29% vs. 4%). The favorable outcome of bone marrow transplantation for severe or very severe aplastic anemia is due to: the use of conditioning with antithymocyte globuline and cyclophosphamide, the use of graft-vs.-host disease prophylaxis with short curse methotrexate and cyclosporine and the use of filtrated and irradiated blood products. For those patients without an HLA-matched related donor the first treatment to use is the immunosuppression with antithymocyte globuline and cyclosporine. Another option emerged in the late 80's is the unrelated bone marrow transplantation, with survival hardly half of the HLA-identical related bone marrow transplants. In our country, the first allogeneic bone marrow transplant was done in the Instituto Nacional de Ciencias Médicas y Nutrición, Salvador Zubirán, in a patient with aplastic anemia, making possible to perform this procedure safely in our country."}, {"id": "pubmed23n0520_21898", "title": "Transplantation of CD34+ selected peripheral blood to HLA-identical sibling patients with aplastic anaemia: results from a single institution.", "score": 0.01599337326366764, "content": "We evaluated the use of CD34+ selected allogeneic peripheral blood as a source of hematopoietic progenitors for allogeneic transplantation in 11 patients with aplastic anemia (AA). The median age was 17 years (range, 6--9), and the median time between diagnosis and transplant 1 month (range, 1--4). Conditioning consisted of cyclophosphamide (50 mg/kg per day) on days--7 to--4 and antithymocyte globulin (30 mg/kg per day) on days--4 to--2 in nine patients. Total lymphoid irradiation was added to the preparative regimen for two. Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporine A and prednisone. Median doses of CD34+ and CD3+ cells infused were 3.91 x 10(6) and 0.3 x 10(6)/kg, respectively. The median time taken to achieve a neutrophil count >0.5 x 10(9)/l was 12 days and to recover a platelet count >20 x 10(9)/l, 13 days. Two patients developed acute GVHD grade I--II and one developed limited chronic GVHD. There were two treatment-related deaths. At a median follow-up of 44 months (range, 4--3), nine patients were alive with sustained and complete engraftment. This is a promising procedure in patients with AA, resulting in a rapid hematopoietic recovery, a low transplant-related mortality, and a low incidence of GVHD."}, {"id": "pubmed23n0652_7664", "title": "Allogeneic hematopoietic stem cell transplantation is superior to immunosuppressive therapy in Indian children with aplastic anemia--a single-center analysis of 100 patients.", "score": 0.015710259639023807, "content": "The authors compared the outcome in 100 children (61 boys, 39 girls; median age of 10.1 +/- 3.4 years) with aplastic anemia who underwent either immunosuppressive therapy (IST; n = 70) or hematopoietic stem cell transplantation (HSCT; n = 30) between 1998 and 2007. Conditioning regimes for HSCT were a combination of either cyclophosphamide (Cy) with antilymphocyte globulin (ALG) or fludarabine (Flu) with Cy or busulfan (Bu) +/- antithymocyte globulin (ATG). Stem cell source was bone marrow in 20 and peripheral blood stem cells (PBSCs) in 10. Patients undergoing IST received either equine ALG or ATG in combination with steroids and cyclosporine. Primary engraftment was seen in 25 children (83.3%), with acute graft-versus-host disease (aGvHD) in 5 (16.6%). The day 100 transplant-related mortality (TRM) was 30% and at a median follow up of 36 months (range: 6-197), the overall and disease-free survival is 70%. Among children who received IST, 60 children received ALG while 10 received ATGAM. Responses were seen in 27 children (43.5%), which was complete (CR) in 12 and partial (PR) in 15. At a median follow up of 38 months (range: 1-84), the overall survival is 37.1%, with 81.4% survival among responders and <10% survival among non-responders. HSCT would be the treatment of choice in children with severe aplastic anemia who have a human leukocyte antigen (HLA)-matched related donor and is superior to IST in this series from India."}, {"id": "pubmed23n0258_12043", "title": "Intensive immunosuppression with antithymocyte globulin and cyclosporine as treatment for severe acquired aplastic anemia.", "score": 0.014636752136752137, "content": "Immunosuppressive therapy can produce hematologic improvement in a large proportion of patients with severe aplastic anemia. Antithymocyte globulin (ATG) is the current treatment of choice for patients who do not have histocompatible sibling donors or who are otherwise inegligible for allogeneic bone marrow transplantation. About 50% of patients respond to an initial course of ATG, and many nonresponders can be salvaged by subsequent treatment with cyclosporine (CsA). To determine whether simultaneous administration of these agents could further improve response rates, we enrolled 55 patients in a therapeutic trial of 4 days of ATG and 6 months of CsA. Among the 51 patients who had not received previous courses of ATG or CsA, 67% had responded by 3 months, and 78% had responded by 1 year (response was defined as an increase in peripheral blood counts sufficient that a patient no longer met the criteria for severe disease). There was a high incidence of relapse (36% actuarial risk at 2 years), but most relapsed patients responded to additional courses of immunosuppression, and relapse was not associated with a significant survival disadvantage. Evolution to myelodysplastic syndromes and acute leukemia was rare (1 of 51 patients), but the later appearance of paroxysmal nocturnal hemoglobinuria was more common (5 of 51 patients). Actuarial survival was 86% at 1 year and 72% at 2 years. These data support the use of a combination immunosuppressive regimen containing both ATG and CsA as first-line therapy for severe aplastic anemia."}, {"id": "pubmed23n0672_20342", "title": "[The use of allogeneic bone marrow transplantation and immunosuppressive therapy in the treatment of patients with acquired aplastic anemia].", "score": 0.013722424874066354, "content": "To evaluate the efficiency of related and unrelated allogeneic bone marrow transplantation (alloBMT) versus immunosuppressive therapy (IST) in patients with aplastic anemia (AA) having no HLA-compatible bone marrow donor. The study covered 61 patients (34 men and 27 women) diagnosed as having acquired AA. Of them, 51 patients were diagnosed as having severe AA, 5 had supersevere AA, and 5 had non-severe AA. Combined IST (antithymocyte globulin (ATG) + cyclosporin A (CsA)) was used in 43 patients; allo-BMT was performed in 18. The basic types of ATG (ATGAM (Pfizer), thymoglobulin (Genzim), ATG (Fresenius), and goat antilymphocyte globulin (ALG) (Research Institute of Gerontology, Ministry of Health of the Russian Federation) were administered. CsA was given in a dose of 5 mg/kg/day. The standard conditioning regimen (ATGAM + cyclophosphanum) and fludarabine-containing (fludarabine + cyclophosphanum + ATG; busulfan + fludarabine + ATG) programs were used in the allo-BMT group. A combination of CsA and metothrexate was given to prevent a graft-versus-host reaction. Among the IST-receiving patients, overall survival (OS) was 71%. After the first course of IST by follow-up month 6, the response rate was 74%. The second course of IST was performed in 7 patients unresponsive after the first-line IST and in 8 patients with recurrent AA. After the second course of IST, the response rate was 46.7%. Four patients who failed to achieve remission after 2 courses of IST received its third course. A complete response was obtained in 3 patients. In 18 patients following allo-BMT (related and unrelated), OS was 86%; event-free survival was 65. In 12 patients after related allo-BMT, OS was 91.7%. Related allo-BMT is the method of choice if there is a HLA-compatible sibling. If there are contraindications to it or no related donor, IST with ATG + CsA is indicated. Ineffective IST is an indication for unrelated allo-BMT that may be recommended as life-saving therapy for young patients under 40 years of age."}, {"id": "wiki20220301en010_97423", "title": "Aplastic anemia", "score": 0.013241844227759722, "content": "Treatment Treating immune-mediated aplastic anemia involves suppression of the immune system, an effect achieved by daily medicine or, in more severe cases, a bone marrow transplant, a potential cure. The transplanted bone marrow replaces the failing bone marrow cells with new ones from a matching donor. The multipotent stem cells in the bone marrow reconstitute all three blood cell lines, giving the patient a new immune system, red blood cells, and platelets. However, besides the risk of graft failure, there is also a risk that the newly created white blood cells may attack the rest of the body (\"graft-versus-host disease\"). In young patients with an HLA-matched sibling donor, bone marrow transplant can be considered as a first-line treatment. Patients lacking a matched sibling donor typically pursue immunosuppression as a first-line treatment, and matched, unrelated donor transplants are considered second-line therapy."}, {"id": "pubmed23n0053_15533", "title": "Treatment of adults with severe aplastic anemia: primary therapy with antithymocyte globulin (ATG) and rescue of ATG failures with bone marrow transplantation.", "score": 0.01276179291446467, "content": "To evaluate a policy of immunosuppression with antithymocyte globulin (ATG) as primary therapy for adults with severe aplastic anemia (SAA) regardless of the availability of an HLA-identical bone marrow donor. Thirty-one consecutive adults with SAA who satisfied the age criteria for allogeneic bone marrow transplantation (BMT) (age less than 51 years) were treated with ATG 20 mg/kg/day for 10 days along with high-dose corticosteroids. Patients with an HLA-identical donor received a transplant if they did not respond to ATG or if they developed life-threatening complications during or soon after ATG administration. Eight patients with no response to ATG were also treated with oral cyclosporine 12.5 mg/kg/day. Eleven patients had a complete and five a partial response to ATG; two patients improved with cyclosporine treatment, resulting in an overall response rate of 58% to immunosuppression. Nine of 14 patients with donors received a BMT: seven because they did not respond to ATG and two because of serious infections. Seven grafts were obtained from related and two from unrelated donors. There was no significant difference in survival between those with and without a related HLA-identical donor (log-rank p value = 0.969). At a median follow-up of 58 months, 26 of 31 are alive with an actuarial survival of 80% at 5 years. Two patients died of infection, two died from complications of BMT, and one remains transfusion-dependent. One patient died of refractory leukemia at 30 months; one patient relapsed with hypoplasia 95 months after initial therapy with ATG. He showed a complete response to treatment with cyclosporine. No other late hematologic events have occurred. This treatment approach resulted in the restoration of hematopoiesis and independence from transfusion in 80% of patients with SAA entered into the study. The efficacy of allogeneic BMT in salvaging cases in which ATG failed does not appear to be compromised. Follow-up for the development of clonal hematologic disorders remains an important part of this treatment policy."}, {"id": "pubmed23n0741_17217", "title": "[Outcome of allogeneic hematopoietic stem cell transplantation from HLA-matched sibling donor for 41 cases of severe aplastic anemia].", "score": 0.012059002978860854, "content": "To evaluate the outcome of allogeneic hematopoietic stem cell transplantation (allo-HSCT) from HLA-matched sibling donor (MSD allo-HSCT) for severe aplastic anemia (SAA). The clinical data of 41 SAA patients received MSD allo-HSCT from May. 2003 to Aug. 2011 were analyzed retrospectively. 24 patients were male, 17 were female. Median age was 23 (5 - 43) years old. 28 patients had SAA-I, 9 had SAA-II, and 4 had post-hepatitis aplastic anemia. 17 patients received allogeneic bone marrow (BM) transplantation (allo-BMT), and 24 received allogeneic peripheral blood stem cell (PBSC) transplantation (allo-PBSCT). The conditioning regimens: 20 patients received cyclophosphamide (CY) + anti-thymocyte globulin (ATG) + fludarabine (Flu), 21 received CY + ATG + Flu+ cytarabine (Ara-C) ± busulfan (Bu)/melphalan (Mel). Prophylaxis for graft-versus-host disease (GVHD): 25 patients received cyclosporine (CSA) plus short-term methotrexate (MTX), 16 received tacrolimus (FK506) plus short-term MTX. The median number of infused CD34(+) cells were 3.48 (2.39 - 4.80)×10(6)/kg in allo-BMT and 2.95 (1.27 - 5.98)×10(6)/kg in allo-PBSCT, respectively. Hematopoietic reconstitution was observed in all 41 patients (100%). The median time of neutrophils (ANC) reached to 0.5×10(9)/L and platelets (PLT) reached to 20×10(9)/L were 14 (10 - 23) days and 19 (8 - 38) days, respectively. 12 patients developed acute GVHD (aGVHD), out of which 11 developed grade I-II aGVHD, and one developed grade IV. 2 patients occurred chronic GVHD (cGVHD), out of which one with local cGVHD and the other with extensive. 4 patients occurred graft rejection (GR), all of them recovered haemopoiesis and survived after donor PBSC infusion. 5 patients (12.2%) died, out of which one died of extensive cGVHD, and 4 died of invasive fungal infections (IFI). Median follow-up time was 23 (3 - 79) months. 36 patients survived. 5-year estimated overall survival (OS), disease free survival (DFS), and transplant-related mortality (TRM) was (81.1 ± 9.0)%, (68.4 ± 11.0)%, and (18.9 ± 9.0)%, respectively. Univariate analysis showed that lover OS had significant correlation with receiving PBSCT, occurrence of aGVHD, the number of infused CD34(+) cells no more than 2.5×10(6)/kg, the number of red blood cell (RBC) transfusion before transplant more than 30 U and occurrence of IFI after transplantation (P = 0.034, 0.001, 0.006, 0.000, 0.001, respectively). Occurrence of aGVHD had significant correlation with the disparity between donor and recipient ABO blood groups, the number of PLT transfusion more than 100 U, and the number of RBC transfusion more than 30 U before transplantation, the number of infused CD34(+) cells no more than 2.5× 10(6)/kg (P = 0.019, 0.038, 0.005, 0.005, respectively). The occurrence of GR had significant correlation with the number of PLT transfusion more than 100 U before transplantation (P = 0.038). MSD allo-HSCT is an effective therapy for patients with SAA. Lower number of blood transfusion before transplantation, use of BMT, more number of infused CD34(+) cells can effectively prevent and treat aGVHD and IFI after transplantation, which may improve the efficacy of MSD allo-HSCT for SAA."}, {"id": "wiki20220301en010_97447", "title": "Bone marrow", "score": 0.012023552678892484, "content": "Donation and transplantation In a bone marrow transplant, hematopoietic stem cells are removed from a person and infused into another person (allogenic) or into the same person at a later time (autologous). If the donor and recipient are compatible, these infused cells will then travel to the bone marrow and initiate blood cell production. Transplantation from one person to another is conducted for the treatment of severe bone marrow diseases, such as congenital defects, autoimmune diseases or malignancies. The patient's own marrow is first killed off with drugs or radiation, and then the new stem cells are introduced. Before radiation therapy or chemotherapy in cases of cancer, some of the patient's hematopoietic stem cells are sometimes harvested and later infused back when the therapy is finished to restore the immune system."}, {"id": "wiki20220301en022_39005", "title": "Hematopoietic stem cell transplantation", "score": 0.011937007874015748, "content": "Nonmyeloablative A newer treatment approach, nonmyeloablative allogeneic transplantation, also termed reduced-intensity conditioning (RIC), uses doses of chemotherapy and radiation too low to eradicate all the bone-marrow cells of the recipient. Instead, nonmyeloablative transplants run lower risks of serious infections and transplant-related mortality while relying upon the graft versus tumor effect to resist the inherent increased risk of cancer relapse. Also significantly, while requiring high doses of immunosuppressive agents in the early stages of treatment, these doses are less than for conventional transplants. This leads to a state of mixed chimerism early after transplant where both recipient and donor HSC coexist in the bone marrow space."}, {"id": "pubmed23n0592_4512", "title": "[Treatment of acquired severe aplastic anemia in pediatric patients with immunosuppression and allogeneic stem cell hematopoietic transplant].", "score": 0.01186714180057823, "content": "Severe acquired aplastic anemia (SAA) is an uncommon disease of childhood. Patients with SAA receive supportive care with transfusions and timely treatment of opportunistic infections, along with specific therapies, which may be allogenic stem cell transplantation (SCT) from a matched sibling or immunosuppressive therapy (IT). To report the experience in the management of SAA. Twenty five children with acquired SAA were treated from July 1992 to September 2005. Patients with full matched sibling donors received allogenic SCT after conditioning with a cyclophosphamide containing regimen. The other patients received immune suppression with cyclosporine plus methylprednisolone (n= 18) plus ATG (n=17). All received supportive care until recovery of hematopoietic function. Those who had severe opportunistic infections at diagnosis or did not respond to two cycles of ATG were evaluated for unrelated donor SCT. Seven patients received sibling donor SCT and 18 IT, which was repeated in six. Three patients received mismatched related (1) or unrelated (2) SCT. Nineteen patients survived with a median follow up time of 4 years, 14 with full hematologic recovery. Six patients died: four due to infections after IT or SCT, one due to intracranial hemorrhage and one with secondary myelodysplasia 12 years after IT. Most children with SAA can be treated successfully with sibling donor SCT or IT. Patients without a histocompatible sibling who fail to respond to IS have a worse prognosis."}, {"id": "wiki20220301en010_97407", "title": "Aplastic anemia", "score": 0.01127651100268844, "content": "Aplastic anemia can be definitively diagnosed by bone marrow biopsy. Normal bone marrow has 30–70% blood stem cells, but in aplastic anemia, these cells are mostly gone and are replaced by fat. First-line treatment for aplastic anemia consists of immunosuppressive drugs—typically either anti-lymphocyte globulin or anti-thymocyte globulin—combined with corticosteroids, chemotherapy, and ciclosporin. Hematopoietic stem cell transplantation is also used, especially for patients under 30 years of age with a related, matched marrow donor. Aplastic anemia is known to have caused the deaths of Eleanor Roosevelt and Marie Curie. Signs and symptoms Anemia may lead to fatigue, pale skin, severe bruising, and a fast heart rate. Low platelets are associated with an increased risk of bleeding, bruising, and petechiae, with lower blood counts that impact the ability of the blood to clot. Low white blood cells increase the risk of infections."}, {"id": "pubmed23n0254_8127", "title": "Allogeneic transplantation combining mobilized blood and bone marrow in patients with refractory hematologic malignancies.", "score": 0.010976158344579396, "content": "Mobilized blood stem cells have been used successfully in autologous transplant recipients to reduce the complications of pancytopenia due to dose-intensive chemotherapy. Reports of cytokine-mobilized blood progenitor cells in allogeneic transplant recipients are rare. This is a pilot trial of six patients. Patients with advanced hematologic malignancy received bone marrow (median total 2.6 x 10(8) mononuclear cells/kg) followed by four daily transfusions of blood (median total 9.5 x 10(8) mononuclear cells/kg) from HLA-matched sibling donors who were mobilized with recombinant human granulocyte-colony-stimulating factor (5 micrograms/kg/day subcutaneously for 5 days). All patients received cyclosporine and prednisone for graft-versus-host disease (GVHD) prophylaxis. An absolute neutrophil count greater than 500 per mm3 was achieved on Day 12, and platelet transfusion independence was achieved on Day 16. The median day of hospital discharge was Day 23 after transplant. All patients achieved 100-percent donor cell engraftment. Acute > or = Grade III GVHD did not develop in any patients, but all patients developed Grade I (n = 4) or Grade II (n = 2) acute GVHD. Chronic extensive GVHD developed in four of six patients. One patient died of pneumonia 263 days after transplant while undergoing immune-suppressive therapy for chronic GVHD. The transfusion of blood stem cells in patients undergoing allogeneic bone marrow transplant is well tolerated soon after transplant, but the development of chronic GVHD may limit the general usage of unmanipulated blood stem cells."}, {"id": "pubmed23n0793_4907", "title": "First successful allogeneic hematopoietic stem cell transplantation for a sickle cell disease patient in a low resource country (Nigeria): a case report.", "score": 0.010902759939492537, "content": "Sickle cell disease (SCD) has a prevalence of 2-3% in Nigeria (population: over 150 million). We present our first allogeneic hematopoietic stem cell transplantation (HSCT) for a 7-year-old patient with severe sickle cell anemia and debilitating right-sided hemi-paraparesis. Conditioning was with (Reduced Intensity Conditioning (FLU/BU).[Fludarabine 160 mg/m2 (days -6 to -2) and Busulphan 16 mg/kg (4×25 mg 6 hly days -5 to -2) and Anti-thymocyte globulin(ATG)(ATGAM) total dose 500 mg (days -6 to -4)]. Graft versus Host Disease (GVHD) prophylaxis was with Cyclosporine A (2×50 mg daily) and Mycophenolate Mofetil (2×500 mg/day). Stem cell source was bone marrow harvested on the 28 September 2011 with 9.8×108 nucleated cells/kg in a total volume of 900 mL from his 14-year-old HLA-matched sibling (6/6). Neutrophil and platelet engraftment was day +18 and +21, respectively. At day +70 full blood count was a total white blood cell count of 3100/µl, absolute Neutrophil count 1200/µl, Hemoglobin (Hb) 11.3 g/dl, Platelet 198,000/µl, Hemoglobin phenotype AA, and no acute or chronic GVHD. He is clinically stable with a Chimerism at 2 years post-HSCT of 95% and responding to physiotherapy. We have successfully performed a stem cell transplanted in a 7-year-old Sickle Cell Anemia case. With the assistance of Government and improved Health Insurance Policy, we could make HSCT available as a cure for many Nigerians with both malignant and non-malignant disorders."}, {"id": "pubmed23n0778_10651", "title": "Management of the refractory aplastic anemia patient: what are the options?", "score": 0.01038905400701529, "content": "Refractory aplastic anemia (AA) is defined as a lack of response to first-line immunosuppressive therapy (IST) with antithymocyte globulin and cyclosporin and is manifested as persistence of severe cytopenias at 6 months after IST. Although supportive care is critical for AA patients, it is of paramount importance for refractory disease in view of the longer duration of pancytopenia and susceptibility to life-threatening infections due to IST. Improvements in supportive care have largely contributed to better outcome over the past 2 decades, with 5-year overall survival reaching 57% during 2002 to 2008 for patients with AA unresponsive to initial IST. Exclusion of hypocellular myelodysplastic syndrome and constitutional BM failure masquerading as apparent idiopathic AA should be done in conjunction with centers of excellence. Hematopoietic stem cell transplantation is indicated if refractory AA patients are fit and have a suitably matched donor, either a sibling (>40-50 years) or unrelated donor. Patients lacking a fully matched donor should be considered for a second course of antithymocyte globulin plus cyclosporin, although response in the refractory setting is only ∼30% to 35%. Response may also occur with alemtuzumab or the thrombopoietin mimetic eltrombopag in refractory AA. The emerging data for alternate donor (cord or haploidentical) transplantation in AA has provided additional therapeutic choices to consider in refractory disease."}, {"id": "pubmed23n0770_5876", "title": "Management of the refractory aplastic anemia patient: what are the options?", "score": 0.010334958660165358, "content": "Refractory aplastic anemia (AA) is defined as a lack of response to first-line immunosuppressive therapy (IST) with antithymocyte globulin and cyclosporin and is manifested as persistence of severe cytopenias at 6 months after IST. Although supportive care is critical for AA patients, it is of paramount importance for refractory disease in view of the longer duration of pancytopenia and susceptibility to life-threatening infections due to IST. Improvements in supportive care have largely contributed to better outcome over the past 2 decades, with 5-year overall survival reaching 57% during 2002 to 2008 for patients with AA unresponsive to initial IST. Exclusion of hypocellular myelodysplastic syndrome and constitutional BM failure masquerading as apparent idiopathic AA should be done in conjunction with centers of excellence. Hematopoietic stem cell transplantation is indicated if refractory AA patients are fit and have a suitably matched donor, either a sibling (>40-50 years) or unrelated donor. Patients lacking a fully matched donor should be considered for a second course of antithymocyte globulin plus cyclosporin, although response in the refractory setting is only ∼30% to 35%. Response may also occur with alemtuzumab or the thrombopoietin mimetic eltrombopag in refractory AA. The emerging data for alternate donor (cord or haploidentical) transplantation in AA has provided additional therapeutic choices to consider in refractory disease. "}, {"id": "wiki20220301en022_39004", "title": "Hematopoietic stem cell transplantation", "score": 0.010137666229631016, "content": "Myeloablative The chemotherapy or irradiation given immediately prior to a transplant is called the conditioning regimen, the purpose of which is to help eradicate the patient's disease prior to the infusion of HSCs and to suppress immune reactions. The bone marrow can be ablated (destroyed) with dose-levels that cause minimal injury to other tissues. In allogeneic transplants, a combination of cyclophosphamide with total body irradiation is conventionally employed. This treatment also has an immunosuppressive effect that prevents rejection of the HSCs by the recipient's immune system. The post-transplant prognosis often includes acute and chronic graft-versus-host disease that may be life-threatening. In certain leukemias, though, this can coincide with protection against cancer relapse owing to the graft-versus-tumor effect. Autologous transplants may also use similar conditioning regimens, but many other chemotherapy combinations can be used depending on the type of disease."}, {"id": "pubmed23n1021_38", "title": "Successful Bone Marrow Recovery After an Immunoablative Regimen With Autologous Cord Blood Transplant in a Child With Idiopathic Severe Aplastic Anemia: A Case Report.", "score": 0.009900990099009901, "content": "Aplastic anemia is a rare disease that manifests as bone marrow failure. The current treatment options include immunoablative therapy or allogeneic hematopoietic stem cell transplantation. We report a successful immunoablative regimen with autologous umbilical cord blood (auto-UCB) transplant in a 3-year-old boy with severe aplastic anemia. The immunoablation procedure consisted of 5 × 3.75 mg/kg antithymocyte globulin (Thymoglobulin) (total 18.75 mg/kg), methylprednisolone for 4 days, and cyclosporine A. The patient received auto-UCB containing 0.3 × 1025: 2 points Hemoglobin (g/dl) >13.5: 0 points 11–13.5: 1 point <11: 2 points Sodium (mmol/l) <135: 2 points Creatinine (umol/l) >141: 2 points Glucose (mmol/l) >10: 1 point"}, {"id": "Pharmacology_Katzung_5601", "title": "Pharmacology_Katzung", "score": 0.011361853049182385, "content": "A 65-year-old man undergoes cystoscopy because of the presence of microscopic hematuria in order to rule out urologic malignancy. The patient has mild dysuria and pyuria and empirically receives oral therapy with cip-rofloxacin for presumed urinary tract infection prior to the procedure and tolerates the procedure well. Approxi-mately 48 hours after the procedure, the patient presents to the emergency department with confusion, dysuria and chills. Physical exam reveals a blood pressure of 90/50, pulse of 120, temperature of 38.5° C and respira-tory rate of 24. The patient is disoriented but the physical exam is otherwise unremarkable. Laboratory test shows WBC 24,000/mm3 and elevated serum lactate; urinalysis shows 300 WBC per high power field and 4+ bacteria. What possible organisms are likely to be responsible for the patient’s symptoms? At this point, what antibiotic(s) would you choose for initial therapy of this potentially life-threatening infection?"}, {"id": "wiki20220301en003_151657", "title": "Bornholm disease", "score": 0.011361165470754511, "content": "Physical exam findings In a studied case of Bornholm disease the chest pain was unable to be reproduced on palpation and failed to improve with changes in position. The pain was made worse during deep inhalation. A pleural rub was present, however lung auscultation was clear and rashes were absent. Laboratory findings and imaging In a prior case of Bornholm disease the laboratory results showed the white blood cell count, hemoglobin, hematocrit, creatinine, liver function test (LFT), troponin, and creatine kinase (CK) were all within normal limits. The chest x-ray showed bilateral pleural effusions which resolved after infection. The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were found to be elevated. The electrocardiogram (EKG) did not show any abnormalities related to ischemia."}, {"id": "pubmed23n0352_16377", "title": "[Septicemic melioidosis in Southern Taiwan: a case report].", "score": 0.010822194273011787, "content": "The patient was a 56 year-old man, a resident of Hen-Tsueng Township in Ping-Tung County. He worked as a ranger at Ken-Ting Farm in southern Taiwan, and had been to Thailand for sight-seeing 5 years ago. He came to our outpatient department about one month prior to hospitalization for intermittent fever of one week duration. At that time, complete blood count was within normal limits and a chest roentgenogram was unremarkable. He was given erythromycin without showing any clinical improvement. Two days prior to admission, he noted pain in the left hip. The next day, severe dyspnea developed suddenly and chest x-ray film revealed bilateral nodular lesions. Physical examination on hospitalization revealed an acutely ill and jaundiced male with a temperature of 37.4 degrees C, blood pressure: 110/47 mmHg, pulse rate: 137/min, and respiratory rate: 26/min. There were rales in both lungs, but no lymphadenopathy or organ enlargement. Laboratory study showed WBC: 1,470/mm(3), platelet count: 47,000/mm(3), blood sugar: 226 mg/dL, mildly elevated transaminases and bilirubin, and BUN: 69 mg/dL, Cr: 4.3 mg/dL. Arterial blood gas analysis indicated an acute metabolic acidosis with PaO2 of 32 mmHg. Despite the initial impression of melioidosis and administration of ceftazidime plus gentamicin, his condition rapidly deteriorated and expired 18 hours after admission. Two sets of blood cultures grew Burkholderia pseudomallei. Melioidosis has been called a great imitator of diseases and culture results are frequently misinterpreted. The mortality is high even with suggested therapy with ceftazidime, cotrimoxazole, amoxicillin-clavulanate, chloramphenicol, and/or tetracycline. There has been a total of 10 cases reported in southern Taiwan and 2 of them were clearly indigenous. Melioidosis should be included in the reportable diseases, and its prevalence in Taiwan also should be investigated."}, {"id": "First_Aid_Step2_497", "title": "First_Aid_Step2", "score": 0.010635958885285549, "content": "■Signs and symptoms are similar to those of cystitis but show evidence of upper urinary tract disease. ■ Symptoms include f ank pain, fever/chills, and nausea/vomiting. Dysuria, frequency, and urgency are also possible. ■UA and culture: Results are similar to those of cystitis, but with WBC casts. Send blood cultures to rule out urosepsis. Pyelonephritis is the most common serious medical complication of pregnancy. Twenty to thirty percent of patients with untreated bacteriuria will develop pyelonephritis. When in doubt, admit a patient with pyelonephritis and administer IV antibiotics. Urosepsis must be considered in any elderly patient with altered mental status. SIRS = two or more of the following: 1. Temperature: Either < 36°C or > 38°C (i.e., hypothermia or fever). 2. Tachypnea: > 20 breaths per minute or PaCO2 < 32 mmHg on ABG. 3. Tachycardia: HR > 90 bpm. 4. Leukocytosis/leukopenia: WBC < 4000 cells/mm3 or > 12,000 cells/mm3. CBC: Reveals leukocytosis."}, {"id": "pubmed23n0352_7387", "title": "[A case of microscopic polyangiitis with severe cardiac and respiratory muscle involvement].", "score": 0.009900990099009901, "content": "A 66-year-old female was admitted to our hospital in January, 1998, complaining of low grade fever and muscle weakness of her legs. Physical examination revealed muscle weakness of her neck (4/5) and proximal skeletal muscles of her bilateral legs (3/5-4/5). She showed proteinuria and microhematuria. Her serum levels of ureanitrogen, creatinine, aspartate aminotransferase, alanine aminotransferase, creatinekinase, aldolase and myoglobin were all within the normal ranges. Antinuclear antibodies were negative, but her serum levels of pANCA (743 EU) and C reactive protein (18.0 mg/dl) were elevated. Neuroconduction velocity of her left common peroneal nerve was decreased to 40.8 m/sec and electric myograph showed neurogenic changes. Magnetic resonance images (MRI) of her bilateral thigh depicted high signal intensity in quadriceps by T 2 weighed images, but the signals were not enhanced by gadolinium injection. Muscle and renal biopsies revealed necrotizing vasculitis of the small arteries. Crescentic glomerulonephritis was also observed by renal biopsy. These findings supported the diagnosis of microscopic PN. On 16 th admission day, she developed acute cardiac and respiratory failures due to cardiac and respiratory muscle involvements with PN, and was assisted by mechanical ventilation. She was treated with methylprednisolone pulse therapy (500 mg/day, three consecutive days) on 18 th admission day, followed by 40 mg of oral prednisolone daily. However, her symptoms deteriorated, and herserum creatinine levels increased to 2.4 mg/dl. On 24 th admission day, intravenous cyclophosphamide pulse therapy (500 mg/day) was instituted. Her cardiac wall motion on echocardiography and serum creatinine levels gradually improved, but her skeletal and respiratory muscle weakness did not improve. On 38 th admission day, she was complicated with respiratory infection by methicillin resistant Staphylococcus aures. On 62 th admission day, she died of endotoxic shock. This is the first report describing respiratory muscle involvement with PN, and the second report describing MRI findings of muscle involvement by PN. Therefore, our case provides important clinical information for the diagnosis and treatment of the disease."}, {"id": "pubmed23n0643_24996", "title": "Myxedema coma associated with combination aripiprazole and sertraline therapy.", "score": 0.00980392156862745, "content": "To describe a case of myxedema coma (MC) associated with combination aripiprazole and sertraline therapy. A 41-year-old male presented to the emergency department with confusion, right-sided numbness and tingling, slurred speech, dizziness, and facial edema. His blood pressure was 160/113 mm Hg, with a pulse of 56 beats/min and temperature of 35.4 degrees C. Initial abnormal laboratory values included creatine kinase (CK) 439 U/L; serum creatinine 1.6 mg/dL; aspartate aminotransferase 85 U/L; and alanine aminotransferase 35 U/L. Repeat cardiac markers revealed an elevated CK level of 3573 U/L with a CK-MB of 24 ng/mL. Thyroid function tests showed thyroid-stimulating hormone 126.4 microIU/mL and free thyroxine 0.29 ng/dL. Home medications of unknown duration were sertraline 200 mg and aripiprazole 20 mg daily. He was admitted to the intensive care unit and initially treated with intravenous levothyroxine and dexamethasone. By hospital day 4, the patient was clinically stable and discharged to home. Myxedema coma, the most significant form of hypothyroidism (HT), is a rare but potentially fatal condition. The known precipitating causes of MC were ruled out in this patient, which left his home medications as the likely cause. Cases of HT caused by certain atypical antipsychotics and antidepressants are found in the literature, but none was reported with aripiprazole therapy. There are also no reported cases of sertraline or aripiprazole inducing MC. Use of the Naranjo probability scale indicates that the combination of aripiprazole and sertraline was a probable inducer of MC in this patient. Due to the widespread use of psychotropic medications, clinicians should be reminded of the rare, yet life-threatening, occurrence of MC when treating patients, especially with combination therapies such as sertraline and aripiprazole."}, {"id": "pubmed23n1033_11783", "title": "Case 282.", "score": 0.009708737864077669, "content": "HistoryA 63-year-old woman with a history of left mastectomy for breast cancer and partial gastrectomy with Roux-en-Y reconstruction for nonhealing peptic ulcer presented to the emergency department and reported a 1-month history of abdominal distention, fevers, chills, and flu-like symptoms. She was initially suspected of having flu, and she completed a course of oseltamivir; however, she had continued fatigue, fever, chills, abdominal bloating, and loss of appetite. She reported no contact with a sick person or recent travel. At admission, laboratory studies revealed leukocytosis, with a white blood cell count of 15.1 × 1038.3°C (101°F) on at least two occasions 2. Illness duration of ≥3 weeks 3. 4. Diagnosis that remains uncertain after a thorough history-taking, physical examination, and the following obligatory investigations: determination of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level; platelet count; leukocyte count and differential; measurement of levels of hemoglobin, electrolytes, creatinine, total protein, alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, creatine kinase, ferritin, antinuclear antibodies, and rheumatoid factor; protein electrophoresis; urinalysis; blood cultures (n = 3); urine culture; chest x-ray; abdominal ultrasonography; and tuberculin skin test (TST). The range of FUO etiologies has evolved over time as a result of changes in the spectrum of diseases causing FUO, the widespread Percentage of Cases Due to Indicated Cause"}, {"id": "pubmed23n1164_4820", "title": "Case 312.", "score": 0.009523809523809525, "content": "A 29-year-old woman was referred for US of bilateral breasts during evaluation for noncyclical mastalgia predominantly in the left breast of 8 months duration. She had been taking selective serotonin receptor inhibitors for the past 6 months for a clinical diagnosis of generalized anxiety disorder. A detailed medical history revealed breast cancer in the patient's mother and grandmother. There was no history of weight loss or appetite loss, nor was there a history of any altered bowel or bladder habits. The patient was overweight, with a body mass index of 26.8 kg/m 38.9 °C (102.02 °F) Systolic blood pressure < 90 mmHg Diffuse macular erythroderma Desquamation (especially of the palms and soles) 1–2 weeks after onset Involvement of three or more organ systems: Gastrointestinal (vomiting, diarrhea) Muscular: severe myalgia or creatine phosphokinase level at least twice the upper limit of normal for laboratory Mucous membrane hyperemia (vaginal, oral, conjunctival) Kidney failure (serum creatinine > 2 times normal) Liver inflammation (bilirubin, AST, or ALT > 2 times normal) Low platelet count (platelet count < 100,000 / mm3) Central nervous system involvement (confusion without any focal neurological findings) Negative results of: Blood, throat, and CSF cultures for other bacteria (besides S. aureus) Negative serology for Rickettsia infection, leptospirosis, and measles"}]}}}} {"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 342]], "word_ranges": [[0, 53]], "text": "Porphyria Cutanea Tarda: 60% of patients with PCT are male, many of them drink alcohol in excess, women who develop it are usually treated with drugs containing estrogens. Most are males with signs of iron overload, this overload reduces the activity of the enzyme uroporphyrinogen decarboxylase, which leads to the elevation of uroporphyrins."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Porphyria Cutanea Tarda: 60% of patients with PCT are male, many of them drink alcohol in excess, women who develop it are usually treated with drugs containing estrogens. Most are males with signs of iron overload, this overload reduces the activity of the enzyme uroporphyrinogen decarboxylase, which leads to the elevation of uroporphyrins. HCV and HIV infections have been implicated in the precipitation of acquired PCT. There is a hereditary form with AD pattern. Patients with PCT present with blistering of photoexposed skin, most frequently on the dorsum of the hands and scalp. In addition to fragility, they may develop hypertrichosis, hyperpigmentation, cicatricial alopecia and sclerodermal induration.", "full_answer_no_ref": "Porphyria Cutanea Tarda: 60% of patients with PCT are male, many of them drink alcohol in excess, women who develop it are usually treated with drugs containing estrogens. Most are males with signs of iron overload, this overload reduces the activity of the enzyme uroporphyrinogen decarboxylase, which leads to the elevation of uroporphyrins. HCV and HIV infections have been implicated in the precipitation of acquired PCT. There is a hereditary form with AD pattern. Patients with PCT present with blistering of photoexposed skin, most frequently on the dorsum of the hands and scalp. In addition to fragility, they may develop hypertrichosis, hyperpigmentation, cicatricial alopecia and sclerodermal induration.", "full_question": "A 62-year-old man with a history of significant alcohol abuse, carrier of hepatitis C virus, treated with Ibuprofen for tendinitis of the right shoulder, goes to his dermatologist because after spending two weeks on vacation at the beach he notices the appearance of tense blisters on the dorsum of his hands. On examination, in addition to localization and slight malar hypertrichosis. The most likely diagnosis is:", "id": 16, "lang": "en", "options": {"1": "Epidermolysis bullosa acquisita.", "2": "Porphyria cutanea tarda.", "3": "Phototoxic reaction.", "4": "Contact dermatitis.", "5": "Acute intermittent porphyria."}, "question_id_specific": 131, "type": "DERMATOLOGY", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0263_17953", "title": "Relationship between porphyria cutanea tarda (PCT) and viral hepatitis.", "score": 0.01960972796308757, "content": "Recent reports have revealed the high prevalence of serological markers of viral hepatitis in porphyria cutanea tarda (PCT). We present two cases of PCT associated with hepatitis C and discuss the relationship between PCT and viral hepatitis. Case 1: A 50-year-old Japanese male noticed blisters, erosions, and fragility on sun-exposed areas of his skin in November of 1990. He had no history of excessive alcohol intake. He had been taking analgesics for eighteen years. Case 2: A 64-year-old Japanese male was referred in October of 1989 because of pigmentation on sun-exposed areas of his skin. He had been drinking alcohol excessively for 43 years. The hepatitis C virus (HCV) antibody was present in each case. Tests for the HCV antibody and hepatitis B serological markers were run in 5 other patients. HCV antibody was present in 3 of them. The two cases negative for the HCV antibody exhibited the hepatitis B antibody. We speculated that viral hepatitis infection may play an important role in precipitating PCT in cases with a history of a long term excessive intake of alcohol or chemicals."}, {"id": "pubmed23n0662_7071", "title": "Familial and sporadic porphyria cutanea tarda: clinical and biochemical features and risk factors in 152 patients.", "score": 0.01905453225660103, "content": "Porphyria cutanea tarda is the most frequent porphyria and occurs in both sporadic and familial forms. We conducted the current study in a series of 152 consecutive patients with porphyria cutanea tarda attending the Porphyria Unit of the Hospital Clinic of Barcelona, Spain, to update the clinical manifestations of the disease and to study the sex differences, the proportion of familial forms, and the role of different risk factors in this population. Patients were classified as familial and sporadic cases according to erythrocyte uroporphyrinogen-decarboxylase activity and uroporphyrinogen-decarboxylase genotyping. In our cohort, skin fragility and blisters on the hands were the most frequent clinical manifestations. Women more frequently had facial hypertrichosis (84.8%; p = 0.004), affected areas other than the hands and face (33.3%; p = 0.008), and pruritus (27.3%; p = 0.041) compared with men. Of our patients, 11.8% did not present the typical clinical onset of the disease, with facial hypertrichosis and hyperpigmentation the more frequent complaints in these cases. Analysis of risk factors showed a high prevalence of hepatitis C virus infection (65.8%) and alcohol abuse (59.9%), both being more frequent in men (p < 0.001). Hepatitis C virus infection was the only risk factor that showed differences between the sporadic and familial forms in the logistic regression model (odds ratio, 0.05; 95% confidence interval, 0.006-0.46). In conclusion, atypical forms of presentation of porphyria cutanea tarda should be considered in order to prevent delayed diagnosis. We note the sustained role of hepatitis C virus infection in the precipitation of sporadic porphyria cutanea tarda. Therefore, in countries with a high prevalence of hepatitis C virus infection, the absence of such infection in a patient with porphyria cutanea tarda may suggest a possible familial case."}, {"id": "pubmed23n0783_4454", "title": "Hepatitis C- and HIV-induced porphyria cutanea tarda.", "score": 0.018957771787960466, "content": "Male, 47 FINAL DIAGNOSIS: Porphyria cutanea tarda Symptoms: Chills • cough dry • thumb swelling - Clinical Procedure: - Specialty: Metabolic Disorders and Diabetics. Challenging differential diagnosis. Porphyria cutanea tarda (PCT) is the most common type of the porphyria. It occurs due to the deficiency of enzyme uroporphyrinogen decarboxylase (UROD), which is the fifth enzyme in the biosynthesis of heme and catalyzes the conversion of uroporphyrinogen to coproporphyrinogen. The risk factors for PCT include hereditary hemochromatosis, hepatitis C infection, ethanol abuse, estrogen use, HIV, smoking, chlorinated polycyclic aromatic hydrocarbons, and hemodialysis. A 47-year-old Hispanic man presented with right thumb swelling, redness, and pain for approximately 1 week. Past medical history included HIV/AIDS, hepatitis C infection, alcohol abuse, heroin abuse, and CMV retinitis. Skin examination revealed blistering and hypo/hyper pigmented lesions over the dorsal aspects of the hands and other sun-exposed areas. Serum porphyrins were discovered to be elevated. The quantitative urine porphyrins revealed elevation of uroporphyrins, heptacarboxyl-porphyrins, hexacarboxy-porphyrins, pentacarboxyl-porphyrins and coproporphyrin. Genetic mutation of UROD was not detected. Due to the classic cutaneous lesions, laboratory findings, and associated risk factors, we were able to confirm our suspicion of the sporadic (type 1) form of PCT. A strong correlation has been demonstrated between the sporadic (type 1) form of PCT and hepatitis C virus (HCV) infection in multiple studies. The mechanism through which HCV infection may cause or trigger PCT is unknown. PCT has been described for many years, but still eludes the differential diagnosis in a patient with cutaneous findings. The uniqueness of our case is the possibility that combined risk factors have an effect on PCT."}, {"id": "pubmed23n0339_9604", "title": "Porphyria cutanea tarda. Don't forget to look at the urine.", "score": 0.017699115044247787, "content": "Diagnosis of porphyria cutanea tarda is usually fairly straightforward because of the characteristic clinical findings. Blisters and erosions develop acutely on sun-exposed skin, sometimes accompanied by hypertrichosis, abnormal pigmentation, and milia formation. Iron stores are usually elevated, and liver transaminases and blood glucose levels are often above normal as well. Gross examination of the urine can provide a valuable clue, since urine of porphyria cutanea tarda patients is red to brown in natural light and pink to red in fluorescent light. Biopsy of a bullous lesion is useful to rule out other diseases. Confirmation of porphyria cutanea tarda requires measurement of porphyrin levels in a 24-hour urine collection. Once the diagnosis is confirmed, it appears reasonable to screen all patients with porphyria cutanea tarda for hepatitis C and possibly B, but especially those less than 30 years old who have extremely high liver transaminase levels. Therapeutic measures for porphyria cutanea tarda include avoidance of exacerbating factors, especially ultraviolet light, ethanol, and certain medications. Phlebotomy or chloroquine therapy is reserved for patients in whom conservative measures fail."}, {"id": "pubmed23n0771_10378", "title": "[Sclerodermatous changes in porphyria cutanea tarda: six cases].", "score": 0.01759259259259259, "content": "The clinical features of porphyria cutanea tarda (PCT) are usually distinctive and include blistering on sun-exposed areas, fragile skin, hypertrichosis and hyperpigmentation. Sclerodermatous changes are much less common, and may either reveal PCT or else appear later. We carried out a retrospective study of the files of six female patients presenting such lesions. Six women (age: 45 to 72 years) were referred for sclerodermatous lesions on sun-exposed areas of the upper body. In four patients, these lesions revealed PCT and in the remaining two patients they were indicative of previously treated but relapsing PCT. Four had sclerodermatous skin changes mimicking morphea of the neck and neckline, the top of the back and the face, while one presented more diffuse facial and cervical sclerosis. Associated alopecia was seen in three patients. The last patient presented isolated sclerodermiform alopecia. Associated malar hypertrichosis was seen in five cases and facial hyperpigmentation was noted in three cases. Four exhibited no blisters, cutaneous fragility, milia or photosensitivity. Histological findings were consistent with morphea or scleroderma in all cases. All patients presented abnormal liver tests: cirrhosis was present in four cases (primitive biliary cirrhosis, alcoholic cirrhosis and hepatitis C) and fatty liver in two cases. In four cases, there was excessive alcohol intake. Uroporphyrin levels were above the normal range in all cases. Local corticosteroid therapy associated with phlebotomy and/or low-dose hydroxychloroquine resulted in complete normalisation of porphyrin levels in four patients, with complete resolution of the cutaneous lesions in two patients and partial improvement in the other two. Sclerodermatous changes are uncommon in PCT. They are not always late and secondary to the process of healing of blisters but can in fact constitute the first cutaneous symptom of the disease while revealing the underlying liver disease. Even in the absence of blisters, photosensitivity or cutaneous fragility, a diagnosis of PCT must be suspected in a setting of sclerodermatous changes distributed on the neck and face, or the neckline, or scarring alopecia, if associated with abnormal liver tests. Skin biopsy to confirm the diagnosis of scleroderma may delay the diagnosis, which is in fact based on porphyrin level. Normalization of the latter parameter under treatment allows regression of lesions."}, {"id": "Biochemistry_Lippincott_1009", "title": "Biochemistry_Lippinco", "score": 0.016609706083390293, "content": "1.2. A 50-year-old man presented with painful blisters on the backs of his hands. He was a golf instructor and indicated that the blisters had erupted shortly after the golfing season began. He did not have recent exposure to common skin irritants. He had partial complex seizure disorder that had begun ~3 years earlier after a head injury. The patient had been taking phenytoin (his only medication) since the onset of the seizure disorder. He admitted to an average weekly ethanol intake of ~18 12-oz cans of beer. The patient’s urine was reddish orange. Cultures obtained from skin lesions failed to grow organisms. A 24-hour urine collection showed elevated uroporphyrin (1,000 mg; normal, <27 mg). The most likely diagnosis is: A. acute intermittent porphyria. B. congenital erythropoietic porphyria. C. erythropoietic protoporphyria. D. hereditary coproporphyria. E. porphyria cutanea tarda."}, {"id": "pubmed23n0934_9712", "title": "Total Corneal Melt in Patient with Porphyria Cutanea Tarda in Presence of Another Risk Factor.", "score": 0.01505752312203925, "content": "200ml is considered a positive result. Bear in mind, however, that this number does not apply to children, and that it can differ depending on the patient's native result; small patient's with pulmonary fibrosis, restrictive lung disease etc. will have a measurably lower FEV1 than healthy average-sized adults. This can give a false positive result of the test. References Pulmonary function testing"}]}}}} {"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 258]], "word_ranges": [[0, 40]], "text": "The correct answer is 1. This is because hysterosalpingography determines if there is any obstruction of the tubes and, therefore, fertilization does not occur. This is not the case in our patient, where fertilization does occur and even implantation occurs."}, "2": {"exist": true, "char_ranges": [[259, 310]], "word_ranges": [[40, 49]], "text": "The rest of the diagnostic tests would be included."}, "3": {"exist": true, "char_ranges": [[259, 310]], "word_ranges": [[40, 49]], "text": "The rest of the diagnostic tests would be included."}, "4": {"exist": true, "char_ranges": [[259, 310]], "word_ranges": [[40, 49]], "text": "The rest of the diagnostic tests would be included."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The correct answer is 1. This is because hysterosalpingography determines if there is any obstruction of the tubes and, therefore, fertilization does not occur. This is not the case in our patient, where fertilization does occur and even implantation occurs. The rest of the diagnostic tests would be included.", "full_answer_no_ref": "[HIDDEN] This is because hysterosalpingography determines if there is any obstruction of the tubes and, therefore, fertilization does not occur. This is not the case in our patient, where fertilization does occur and even implantation occurs. The rest of the diagnostic tests would be included.", "full_question": "A 36-year-old woman comes to the clinic because she has had three miscarriages in the first trimester. She has not had any full-term pregnancies. The studies that you will request in the first term are NOT included:", "id": 444, "lang": "en", "options": {"1": "Hysterosalpingography.", "2": "Peripheral blood karyotyping of both partners.", "3": "Determination of antiphospholipid antibodies.", "4": "Vaginal ultrasound.", "5": NaN}, "question_id_specific": 104, "type": "GYNECOLOGY AND OBSTETRICS", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0301_20870", "title": "Future pregnancy outcome in unexplained recurrent first trimester miscarriage.", "score": 0.01827436582109479, "content": "The future pregnancy outcome of 201 consecutive women, median age 34 years (range 22-43), with a history of unexplained recurrent first trimester miscarriage (median 3; range 3-13), was studied. All women and their partners had normal peripheral blood karyotypes; none had antiphospholipid antibodies and none hypersecreted luteinizing hormone (LH). No pharmacological treatment was prescribed and early pregnancy supportive care was encouraged. Women aged < or = 30 years had a subsequent miscarriage rate of 25% (14/57) which rose to 52% (13/25) in women aged > or = 40 years (P = 0.02). After three consecutive miscarriages, the risk of miscarriage of the next pregnancy was 29% (34/119) but increased to 53% (9/17) after six or more previous losses (P = 0.04). A past history of a livebirth did not influence the outcome of the next pregnancy. Supportive care in early pregnancy conferred a significant beneficial effect on pregnancy outcome. Of 160 women who attended the early pregnancy clinic, 42 (26%) miscarried in the next pregnancy compared with 21 out of 41 (51%) who did not attend the clinic (P = 0.002). After thorough investigation, women with unexplained recurrent first trimester miscarriage have an excellent pregnancy outcome without pharmacological intervention if offered supportive care alone in the setting of a dedicated miscarriage clinic."}, {"id": "pubmed23n1144_10403", "title": "Evaluation of basal hormone levels and androgen receptor gene mutations in individuals with recurrent abortion.", "score": 0.01333169754222386, "content": "A recurrent miscarriage is at least two consecutive miscarriages in the first trimester of pregnancy. Due to the dependence of pregnancy on endocrine changes in the menstrual cycle, its disorders can also affect the outcome of pregnancy. In addition to hormonal disorders, genetic changes are essential factors in recurrent miscarriage. The development and maturation of ovulation depend on the molecular signaling pathways that respond to androgens. Hundreds of mutations leading to resistance to androgen receptor (AR) gene function have been recorded, including the 5'UTR polymorphic region. Therefore, considering the role of androgen receptors and hormonal changes in recurrent miscarriage, this study was performed to investigate the relationship between hormonal changes and AR gene mutations in patients with recurrent miscarriage. In this regard, a case-control study was performed on 150 patients with miscarriage referred to the infertility center. Hysterosalpingography, parental karyotype, vaginal ultrasound, antiphospholipid antibody measurement, anticardiolipin antibody, history and physical examination were performed to evaluate the possible causes of recurrent miscarriage. Hormone levels of LH, FSH, TSH, and Prolactin were measured and compared in two groups with known and unknown causes. Blood samples were also taken from patients, and after DNA extraction, the PCR method was used to determine AR gene mutations. The mean age was 30.2 ± 7.1 years, the mean number of abortions was 2.6 ± 1.2, and the mean duration of marriage was 6.1 ± 2.1 years. The mean of hormones in the two groups with known and unknown causes was compared, that TSH was significantly lower in the group with unknown cause (P = 0.031) and prolactin was higher in recurrent miscarriage patients with polycystic ovaries (P = 0.048). Regarding genetic evaluation, in the 5'UTR region of the androgen receptor gene, deletion of T nucleotide was observed in the +25 position, but no significant difference was found between the two groups. Generally, the findings of this study showed that thyroid dysfunction and hyperprolactinemia should be considered as an endocrine disorder in people with recurrent miscarriage, and genetic evaluation showed that the AR gene mutation was not associated with recurrent miscarriage."}, {"id": "wiki20220301en036_28912", "title": "Recurrent miscarriage", "score": 0.012246808930067517, "content": "Immune factors A common feature of immune factors in causing recurrent pregnancy loss appears to be a decreased maternal immune tolerance towards the fetus. Antiphospholipid syndrome The antiphospholipid syndrome is an autoimmune disease that is a common cause of recurrent pregnancy loss. Around 15% of the women who have recurrent miscarriages have high levels of antiphospholipid antibodies. Women who have had more than one miscarriage in the first trimester, or a miscarriage in the second trimester, may have their blood tested for antibodies, to determine if they have antiphospholipid syndrome. Women diagnosed with antiphospholipid syndrome generally take aspirin or heparin in subsequent pregnancies, but questions remain due to the lack of high quality trials. Thyroid antibodies Anti-thyroid autoantibodies are associated with an increased risk of recurrent miscarriage with an odds ratio of 2.3 with a 95% confidence interval of 1.5–3.5. Increased uterine NK cells"}, {"id": "pubmed23n0276_18307", "title": "The prevalence of lupus anticoagulant and anticardiolipin antibodies in women with a history of first trimester miscarriages.", "score": 0.011920302301614566, "content": "To determine the prevalence of lupus anticoagulant and raised anticardiolipin antibodies in women with a history of two or more miscarriages in the first trimester of pregnancy. A prospective study of lupus anticoagulant and anticardiolipin antibody levels in unselected women with a history of two or more first trimester miscarriages. The prepregnancy clinic and miscarriage antenatal clinic in a tertiary referral centre. Two hundred and forty-three women, of whom 113 (47%) had a past history of two miscarriages, and 130 (53%) had three or more miscarriages. Quantitative detection of lupus anticoagulant and anticardiolipin antibodies; number of miscarriages in women in the normal and the abnormal groups. Of the 243 women tested, 41 (16.8%) had an abnormality of lupus anticoagulant or anticardiolipin antibodies. This was significantly different from the normal population as previously reported. Sixteen women (6.6%) were positive for lupus anticoagulant, 20 (8.2%) had elevated anticardiolipin antibodies, and five (2%) had both abnormalities. The most frequently positive test for lupus anticoagulant was the dilute Russel viper venom time, and IgG was the most frequently elevated anticardiolipin antibody. Of the women with a history of only two miscarriages, 15% had an abnormality of lupus anticoagulant or anticardiolipin antibodies, compared with 18.5% of those with a history of three or more miscarriages. This did not reach statistical significance. There were 117 (48%) primary miscarriers and 126 (52%) secondary miscarriers. Of the primary miscarriers, 17% had an abnormality, compared to 18% of the secondary miscarriers. These findings provide further evidence of an association between lupus anticoagulant and anticardiolipin antibodies and early pregnancy loss. It is not known if these are the cause of miscarriage, markers for miscarriage, or if antiphospholipid antibodies develop as a result of a noncontinuing pregnancy. Further studies comparing various treatments are required before women with these antibodies can be optimally managed."}, {"id": "wiki20220301en345_32233", "title": "Lupus and pregnancy", "score": 0.01076923076923077, "content": "Of live births, approximately one third are delivered prematurely. Miscarriage Lupus causes an increased rate of fetal death in utero and spontaneous abortion (miscarriage). The overall live-birth rate in somebody with lupus has been estimated to be 72%. Pregnancy outcome appears to be worse in those with lupus whose disease flares up during pregnancy. Miscarriages in the first trimester appear either to have no known cause or to be associated with signs of active lupus. Later losses appear to occur primarily due to the antiphospholipid syndrome, in spite of treatment with heparin and aspirin. All women with lupus, even those without previous history of miscarriage, are recommended to be screened for antiphospholipid antibodies, both the lupus anticoagulant (the RVVT and sensitive PTT are the best screening battery) and anticardiolipin antibodies."}, {"id": "pubmed23n0302_20693", "title": "[A case of antiphospholipid antibody syndrome successfully treated with prednisolone and low-dose aspirin, with subsequent normal pregnancy].", "score": 0.010502232758145432, "content": "We reported a 26-year-old female who had experienced 2 miscarriages associated with a possible cause of antiphospholipid antibody. She consulted to Kainan Municipal Hospital with major complaints of miscarriages and positive antiphospholipid antibody. No evidence of obstetric disorder was apparent and routine haematological parameters were normal. Serological studies and clinical evidence did not show SLE. While biologically false positive (reaction of serological test for syphilis) and lupus anticoagulant was negative, IgG anticardiolipin antibody was positive. She was diagnosed as possible antiphospholipid antibody syndrome based on positive anticardiolipin antibody and the history of miscarriages. In 1993, she was started on prednisolone and aspirin therapy; antiphospholipid antibody returned to negative. Treatment with prednisolone (7.5 mg/day) and aspirin (40 mg/day) was continued. She was able to be pregnant. She gave birth successfully at our hospital in August 1995. She and her newborn showed no abnormalities."}, {"id": "wiki20220301en012_1890", "title": "Antiphospholipid syndrome", "score": 0.010405976276283443, "content": "Antiphospholipid syndrome, or antiphospholipid antibody syndrome (APS or APLS), is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. APS provokes blood clots (thrombosis) in both arteries and veins as well as pregnancy-related complications such as miscarriage, stillbirth, preterm delivery, and severe preeclampsia. Although the exact etiology of APS is still not clear, genetics is believed to play a key role in the development of the disease. The diagnostic criteria require one clinical event (i.e. thrombosis or pregnancy complication) and two positive blood test results spaced at least three months apart that detect lupus anticoagulant, anti-apolipoprotein antibodies, or anti-cardiolipin antibodies."}, {"id": "pubmed23n0931_5853", "title": "Spontaneous first trimester miscarriage rates per woman among parous women with 1 or more pregnancies of 24 weeks or more.", "score": 0.009900990099009901, "content": "The purpose of this study was to quantify spontaneous first trimester miscarriage rates per woman among parous women. A vast amount of data has accumulated regarding miscarriage rates per recognized pregnancy as well as about recurrent miscarriage. This is the second study of miscarriage rates per woman in a parous population and the first study of recurrent and non-recurrent, spontaneous first trimester miscarriage rates per woman in a large parous population. Extraction of the following variables from all delivery room admissions from both Hadassah Medical Centers in Jerusalem Israel, 2004-2014: # of first trimester spontaneous miscarriages, # live births; # living children; age on admission, pre-pregnancy height and weight, any smoking this pregnancy, any alcohol or drug abuse this pregnancy, blood type, history of ectopic pregnancy, history of cesarean surgery (CS) and use of any fertility treatment(s). Among 53,479 different women admitted to labor and delivery ward, 43% of women reported having had 1 or more first trimester spontaneous miscarriages; 27% reported having had one, 10% two, 4% three, 1.3% four, 0.6% five and 0.05% reported having 6-16 spontaneous first trimester miscarriages. 18.5% had one or more first trimester miscarriages before their first live birth. Eighty-one percent of women with 11 or more living children experienced one or more first trimester miscarriages. First trimester miscarriage rates rose with increasing age, increasing parity, after previous ectopic pregnancy, after previous cesarean surgery, with any smoking during pregnancy and pre-pregnancy BMI ≥30. Miscarriages are common among parous women; 43% of parous women report having experienced one or more first trimester spontaneous miscarriages, rising to 81% among women with 11 or more living children. One in every 17 parous women have three or more miscarriages. Depending on her health, nutrition and lifestyle choices, even a 39 year old parous woman with a history of 3 or more miscarriages has a good chance of carrying a future pregnancy to term but she should act expediently."}, {"id": "pubmed23n0283_7463", "title": "Ultrasonographic detection of a live fetus in recurrent spontaneous abortion during the first trimester.", "score": 0.009900990099009901, "content": "To examine whether recurrent spontaneous abortion (RSA) can be distinguished from repeated sporadic spontaneous abortion, the clinical course of 38 cases with three or more consecutive and unexplained first trimester RSAs were retrospectively investigated in this study. For comparison with controls, the clinical course was examined of 38 fertile females, who had had sporadic abortions. In 19 (50%) RSAs and 6 (16%) controls, fetal cardiac activity was demonstrated by ultrasound during the course of pregnancy. The rate of detection of live fetus during pregnancy or at 8 weeks +/- 7 days gestation, was significantly greater in the RSA group compared to the control. The rate of vaginal bleeding before spontaneous abortion was significantly less in the RSA group than in the control group. There was no difference between the two groups in age or gestational age at spontaneous abortion. The patients with RSA were all examined for antiphospholipid antibodies in their sera and these were detected in eight of them. However, there was no difference in the rate of positive fetal cardiac activity between the RSA patients who tested positive or negative for antibody. These results reveal that the clinical course of RSA is very different from the course of sporadic abortion. Although sporadic abortion is a common complication of pregnancy, RSA is not a random repeated abortion, but rather a separate disease from sporadic abortion in normal fertile females."}, {"id": "pubmed23n1101_5555", "title": "Placenta increta presenting with threatened miscarriage during the first trimester in rhesus-negative mother: a case report.", "score": 0.00980392156862745, "content": "Placenta accreta is known to be associated with significant maternal morbidity and mortality-primarily due to intractable bleeding during abortion or delivery at any level of gestation. The complications could be reduced if placenta accreta is suspected in a patient with a history of previous cesarean delivery and the gestational sac/placenta is located at the lower part of the uterus. Then, a proper management plan can be instituted, and complications can be reduced. The diagnosis of placenta accreta in the first trimester of pregnancy is considered uncommon. A 34-year-old Malay, gravida 4, para 3, rhesus-negative woman was referred from a private hospital at 13 weeks owing to accreta suspicion for further management. She has a history of three previous lower-segment cesarean sections. She also had per vaginal bleeding in the early first trimester, which is considered to indicate threatened miscarriage. Transabdominal ultrasound revealed features consistent with placenta accreta spectrum. She was counseled for open laparotomy and hysterectomy because of potential major complication if she continued with the pregnancy. Histopathological examination revealed placenta increta. A high index of suspicion of placenta previa accreta must be in practice in a patient with a history of previous cesarean deliveries and low-lying placenta upon ultrasound examination during early gestation."}, {"id": "pubmed23n0309_12009", "title": "A prospective study of fifty-three consecutive calcium heparin treated pregnancies in patients with antiphospholipid antibody-related fetal loss.", "score": 0.00980392156862745, "content": "In this study the efficacy and safety of calcium heparin administered alone for the prevention of fetal loss related to antiphospholipid antibodies (aPL) were evaluated. Fifty-three consecutively ascertained pregnancies were followed in 53 patients who had a history of at least 2 consecutive miscarriages during the first trimester and/or 1 fetal death during the second or third trimesters. In addition, all patients had at least 2 positive aPL tests more than 8 weeks apart before pregnancy, or a positive aPL test at the beginning of pregnancy. They were treated with calcium heparin alone, self-administered subcutaneously 3 times daily at dosages varying between 15,000 and 37,500 units. Treatment was started soon after a sonogram demonstrated a live embryo and was continued throughout pregnancy until the end of puerperium. All pregnancies terminated favourably between the 25th and 40th weeks (mean +/- SD: 36.69 +/- 2.91) with planned caesarean section in 27 cases and vaginal delivery in 26. Delivery was brought forward due to maternal and/or fetal complications in 18 cases (33.96%). Calcium heparin was associated with intravenous immunoglobulin therapy in 2 patients with fetal problems unresponsive to anticoagulant treatment alone. The newborns, 30 females and 25 males, had a mean birth weight of 2,828.3 g +/- 706.5 and a mean Apgar score at 5 minutes of 9.60 +/- 0.68. No malformations were observed. Thirty of the 37 examined placentas (81.08%) showed signs of thrombotic events. Only minor side effects of calcium heparin were observed during treatment. Our study suggests that calcium heparin administered alone using the dosages and timing described here is effective in achieving the delivery of viable infants, and that it is well tolerated."}, {"id": "pubmed23n0847_19569", "title": "Higher levels of procoagulant microparticles in women with recurrent miscarriage are not associated with antiphospholipid antibodies.", "score": 0.00978281668580176, "content": "Are the levels of circulating cell-derived microparticles (cMPs) in patients with recurrent miscarriage (RM) associated with the antiphospholipid syndrome (APS)? cMPs in women with RM are not associated with antiphospholipid antibodies (aPLs). Previous studies have focused on cMP levels in RM patients. Most studies have shown higher levels of cMPs in RM patients whereas others have reported lower levels. Data regarding cMPs in patients with the APS are scanty in the literature. A case-control study including three groups of patients. A total of 154 women were prospectively recruited from September 2009 to October 2013. Four patients refused to participate. The APS group consisted of 50 women that had been previously diagnosed with primary APS and had had ≥3 consecutive first trimester miscarriages. The uRM group included 52 couples with ≥3 consecutive first trimester miscarriages of unknown etiology. The fertile control (FER) group was composed of 52 healthy fertile women with no history of pregnancy losses. Miscarriage was defined as intrauterine pregnancy loss at <10 weeks' size on ultrasound. Venous blood samples for coagulation studies and cMP determinations were obtained. All patients underwent a thrombophilia study. cMP levels were significantly higher in the APS and uRM groups versus the FER group (P < 0.0001 and P = 0.009, respectively) (cMP number × 10(3)/ml plasma [mean ± SD]: APS: 18.5 ± 13.6; uRM: 16.3 ± 13.8; FER: 9.7 ± 4.6). There were no statistically significant differences in cMP levels between the APS and uRM groups. The sample size was arbitrarily decided according to previous studies analyzing cMPs in RM patients. Different cMP subtypes were not investigated. The present study adds further data on the subject showing that patients with RM, irrespective of testing positive for aPLs, have increased levels of cMPs compared with healthy fertile controls. The presence of elevated cMPs in RM women may reflect an ongoing systemic pathological, albeit asymptomatic, status that can become deleterious in the setting of pregnancy. This study was supported in part by grant from FIS-PI11/01560 within the 'Plan Nacional de I+D+I' and co-funded by the 'ISCIII-Subdirección General de Evaluación' and the 'Fondo Europeo de Desarrollo Regional (FEDER)'. The authors have no competing interests to disclose. Not applicable."}, {"id": "pubmed23n0494_24041", "title": "Antiphospholipid syndrome and second- or third-trimester fetal death: follow-up in the next pregnancy.", "score": 0.009708737864077669, "content": "To evaluate the efficacy of a uniform management protocol in antiphospholipid-antibody-positive obstetric patients with at least one second- or third-trimester intra-uterine fetal death. A prospective study of 33 successive pregnancies in antiphospholipid-antibody-positive patients, diagnosed after an intra-uterine fetal death. The management included treatment by a combination of aspirin and low-molecular-weight heparin, and a close follow-up with at least clinical examination, ultrasonography, uterine, and umbilical artery Doppler monthly from the first trimester. In the absence of any anomaly, delivery was induced between 37 and 38 weeks' gestation. In this high risk population, seven recurrences of vascular pathology occurred: five cases of mild, isolated fetal growth retardation and one of preeclampsia associated with fetal growth retardation requiring preterm delivery. Eight patients were delivered before 37 weeks. No recurrence of second- or third-trimester fetal death was observed. Uterine artery Doppler was informative as early as the first trimester (12-15 weeks): a bilateral notch was associated with a lower birthweight (2626+/-688 g versus 3178+/-353 g, respectively, p = 0.01), despite similar gestational age. The negative predictive value of uterine Doppler was more than 92% at 12-15 weeks' gestation and remained high throughout pregnancy. Although intra-uterine fetal death is considered at high risk of recurrence in case of antiphospholipid syndrome (APS), a uniform management protocol including aspirin and heparin and close obstetrical follow-up led to a favorable outcome in most cases."}, {"id": "wiki20220301en012_1892", "title": "Antiphospholipid syndrome", "score": 0.009678230266465561, "content": "Signs and symptoms The presence of antiphospholipid antibodies (aPL) in the absence of blood clots or pregnancy-related complications does not indicate APS (see below for the diagnosis of APS). Antiphospholipid syndrome can cause arterial or venous blood clots, in any organ system, or pregnancy-related complications. In APS patients, the most common venous event is deep vein thrombosis of the lower extremities, and the most common arterial event is stroke. In pregnant women affected by APS, there is an increased risk of recurrent miscarriage, intrauterine growth restriction, and preterm birth. A frequent cause of such complications is placental infarctions. In some cases, APS seems to be the leading cause of mental and/or development retardation in the newborn, due to an aPL-induced inhibition of trophoblast differentiation. The antiphospholipid syndrome responsible for most of the miscarriages in later trimesters seen in concomitant systemic lupus erythematosus and pregnancy."}, {"id": "pubmed23n1143_10470", "title": "High-level mosaicism for 45,X in 45,X/46,XX at amniocentesis in a pregnancy with a favorable outcome and postnatal progressive decrease of the 45,X cell line.", "score": 0.009615384615384616, "content": "We present prenatal diagnosis of high-level mosaicism for 45,X in 45,X/46,XX at amniocentesis in a pregnancy with a favorable outcome and postnatal progressive decrease of the 45,X cell line. A 32-year-old, gravida 2, para 1, woman underwent amniocentesis at 17 weeks of gestation because of the abnormal first-trimester maternal serum screening result indicating a 1/34 risk for Down syndrome. Amniocentesis revealed a karyotype of 45,X[27]/46,XX[15]. Simultaneous array comparative genomic hybridization (aCGH) on uncultured amniocytes revealed 12% mosaicism for monosomy X. Prenatal ultrasound was normal. The pregnancy was carried to term, and a 2780-g phenotypically normal female baby was delivered. The cord blood had a karyotype of 45,X[12]/46,XX[28]. At age one month, the peripheral blood had a karyotype of 45,X[13]/46,XX[27]. Interphase fluorescence in situ hybridization (FISH) analysis on the buccal mucosal cells revealed 2% (2/102 cells) mosaicism for monosomy X, compared with 1% (1/100 cells) in the normal control. When follow-up at age one year, she was doing well with normal physical and psychomotor development. Her body weight was 9.9 Kg (50th - 85th centile), and her body height was 75 cm (50th - 85th centile). The peripheral blood had a karyotype of 45,X[4]/46,XY[36]. High-level mosaicism for 45,X in 45,X/46,XX at amniocentesis can be associated with a favorable outcome and postnatal progressive decrease of the 45,X cell line."}, {"id": "Gynecology_Novak_6279", "title": "Gynecology_Novak", "score": 0.009615384615384616, "content": "For a patient to be diagnosed with antiphospholipid antibody syndrome, one or more clinical and one or more laboratory criteria must be present: 1. One or more confirmed episode of vascular thrombosis of any type: Venous Arterial Small vessel 2. Pregnancy complications: of gestation with exclusion of maternal anatomic and hormonal abnormalities and exclusion of paternal and maternal chromosomal abnormalities One or more unexplained deaths of a morphologically normal fetus at or beyond 10 weeks of gestation (normal fetal morphology documented by ultra sound or direct examination of the fetus) One or more premature births of a morphologically normal neonate at or before 34 weeks of gestation secondary to severe preeclampsia or placental insufficiency Testing must be positive on two or more occasions with evaluations 12 or more weeks apart: 1. Positive plasma levels of anticardiolipin antibodies of the IgG or IgM isotype at medium to high levels 2."}, {"id": "pubmed23n0987_2922", "title": "A case of incidental endometrial adenocarcinoma diagnosed in early pregnancy and managed conservatively.", "score": 0.009523809523809525, "content": "We report a 29-year old nulliparous woman diagnosed with a grade 1 endometrioid adenocarcinoma of the endometrium arising from an atypical polypoid adenomyoma, while being investigated for a suspected threatened miscarriage at 7 weeks gestation. She presented complaining of vaginal bleeding and a small amount of soft tissue in the cervical os was found and sent for histology. An ultrasound scan was performed, which confirmed an intrauterine ongoing pregnancy. The patient had no further episodes of unscheduled bleeding. After the confirmed histological diagnosis an MRI scan was requested, and there were no evidence of myometrial invasion or distant metastasis. The patient was seen at each trimester, remained asymptomatic throughout the pregnancy and had a normal delivery at term. There was no evidence of any residual endometrioid adenocarcinoma in the post-delivery specimen. Six weeks post-natally an endometrial biopsy was performed, which was normal. She is still in remission over a period of 8 years follow-up. Endometrial adenocarcinoma in young pregnant women is a rare clinical circumstance. This case shows that conservative management in young women is possible including in a case of an incidental diagnosis in pregnancy."}, {"id": "pubmed23n0922_2637", "title": "Two miscarriages, consecutive or non-consecutive, does it change something?", "score": 0.009523809523809525, "content": "To assess the rate of anomalies in the etiological evaluation of patients presenting recurrent early miscarriages (RM) according to miscarriage chronology (number of miscarriages, history of live birth and succession of RM). Retrospective single centre study including RM, defined as at least 2 miscarriages at less than 14 weeks of gestation (WG) between the 1st January 2012 and the 31st December 2015. Clinical data and etiological evaluation include blood glucose levels, screening for antiphospholipid syndrome (APS), endocrine assessment, vitamin levels, pelvic imaging, karyotyping of both partners, chronic endometritis and thrombophilia screening. Two hundred and eighty-eight patients were included over this period, 118 (41%) patients had no history of live birth. Two hundred and twenty-three (77%) patients had consecutive RM and 65 (22%) patients had non-consecutive RM. For consecutive RM, 62,8% had thrombophilic disorders versus 69,8% for non-consecutive RM (P>0,05); 44,7% had endocrine disorders or vitamin deficiencies versus 39,7%; 34,6% of patients with consecutive RM had uterine anomalies versus 45,5% respectively. No difference was found depending on the recurrence of RM or the history of live birth (P>0.05) apart from the age of the patient. Fifty-nine (17.4%) patients had uterine anomalies. There are 24 chronic endometritis on 31 biospsies performed. Seventy-eight (27%) patients were offered treatment. Ninety-four (90%) patients showed good therapy compliance. Eighty-one (78%) patients became pregnant. An etiological evaluation provides, for over half of the cases, an etiology or the identification of risk factors responsible for RM, as well as in some cases offering an adapted, efficient, therapeutic approach. This evaluation should be offered regardless of the obstetric history of the patient."}, {"id": "pubmed23n0832_4364", "title": "Autoimmune diseases and pregnancy: analysis of a series of cases.", "score": 0.009433962264150943, "content": "An autoimmune disease is characterized by tissue damage, caused by self-reactivity of different effector mechanisms of the immune system, namely antibodies and T cells. All autoimmune diseases, to some extent, have implications for fertility and obstetrics. Currently, due to available treatments and specialised care for pregnant women with autoimmune disease, the prognosis for both mother and child has improved significantly. However these pregnancies are always high risk. The purpose of this study is to analyse the fertility/pregnancy process of women with systemic and organ-specific autoimmune diseases and assess pathological and treatment implications. The authors performed an analysis of the clinical records and relevant obstetric history of five patients representing five distinct autoimmune pathological scenarios, selected from Autoimmune Disease Consultation at the Hospital of Braga, and reviewed the literature. The five clinical cases are the following: Case 1-28 years old with systemic lupus erythematosus, and clinical remission of the disease, under medication with hydroxychloroquine, prednisolone and acetylsalicylic acid, with incomplete miscarriage at 7 weeks of gestation without signs of thrombosis. Case 2-44 years old with history of two late miscarriages, a single preterm delivery (33 weeks) and multiple thrombotic events over the years, was diagnosed with antiphospholipid syndrome after acute myocardial infarction. Case 3-31 years old with polymyositis, treated with azathioprine for 3 years with complete remission of the disease, took the informed decision to get pregnant after medical consultation and full weaning from azathioprine, and gave birth to a healthy term new-born. Case 4-38 years old pregnant woman developed Behcet's syndrome during the final 15 weeks of gestation and with disease exacerbation after delivery. Case 5-36 years old with autoimmune thyroiditis diagnosed during her first pregnancy, with difficult control over the thyroid function over the years and first trimester miscarriage, suffered a second miscarriage despite clinical stability and antibody regression. As described in literature, the authors found a strong association between autoimmune disease and obstetric complications, especially with systemic lupus erythematosus, antiphospholipid syndrome and autoimmune thyroiditis."}, {"id": "pubmed23n0546_3636", "title": "Complement as a predictor of further miscarriage in couples with recurrent miscarriages.", "score": 0.009433962264150943, "content": "The clinical significance of complement is unclear in patients with unexplained recurrent miscarriage, though low levels of complement 3 (C3) and/or complement 4 (C4) are reported to be associated with the antiphospholipid syndrome (aPS). We therefore investigated whether C3 and C4 have a predictive value for subsequent miscarriages. In total, 215 patients with a history of two consecutive first-trimester miscarriages and no abnormal chromosomes in either partner, no uterine anomalies and no antiphospholipid (aPL) antibodies were examined. Blood tests for C3, C4, total haemolytic complement (CH50), immunoglobulin G (IgG), immunoglobulin A (IgA) and immunoglobulin M (IgM) were performed before subsequent pregnancy. Patients were then followed up without treatment, and their pregnancy outcomes were compared with their previous blood test results. From 215 pregnant patients, 45 subsequently miscarried, whereas the remainder had a live birth. There was no relation with serum CH50, IgG, IgA and IgM levels, but C3 and C4 levels in patients with subsequent miscarriage were significantly higher than in those whose pregnancy was successful. In patients with two previous miscarriages, C3 and C4 levels were higher in those women who had a third miscarriage, than in women that went on to have a live birth."}, {"id": "pubmed23n0973_20949", "title": "[Comparison of the etiological constitution of two and three or more recurrent miscarriage].", "score": 0.009345794392523364, "content": "70/min presence of nasal flaring and/or grunting severe chest wall recession (Hoover's sign) bluish skin Causes"}, {"id": "pubmed23n1110_12836", "title": "A 2-Month-Old with Kawasaki Disease with Coronary Artery Dilation in the Pre-COVID-19 Era.", "score": 0.009900990099009901, "content": "BACKGROUND Kawasaki disease (KD) is an acute inflammatory vasculitis, which occurs mostly in childhood, predominantly between the ages of 6 months and 5 years. The incidence of coronary artery abnormalities associated with KD has decreased from 25% to 4% as a result of timely diagnosis and treatment with intravenous immunoglobulin (IVIG). Infants ≤6 months of age are the most likely to develop prolonged fever without the other clinical criteria for KD, and diagnosis can sometimes be challenging or delayed. They are therefore at particularly high risk of developing coronary artery abnormalities. CASE REPORT A 2-month-old male infant with no significant medical history initially presented with a history of nasal congestion, right conjunctivitis, red lips, and 1 loose stool in the pre-COVID-19 era. He was diagnosed with otitis media and was started on oral amoxicillin. By day 7 of fever, he had developed symptoms and signs and laboratory findings consistent with Kawasaki disease, which is rare in this age group. His echocardiogram showed dilated proximal left anterior descending and right coronary arteries. He was successfully treated, and his most recent echocardiogram, performed 17 months after his treatment, showed remarkable improvement in the coronary arteries. CONCLUSIONS Kawasaki disease in children less than 6 months of age is still rare, and the presentation can sometimes make the diagnosis somewhat challenging. Increased clinical suspicion is required for recognition in the youngest patients, as they are more likely to present with few features of KD. Early diagnosis and treatment are needed to prevent or minimize the risk of significant coronary artery abnormalities."}, {"id": "pubmed23n0987_21108", "title": "Kawasaki disease triggered by parvovirus infection: an atypical case report of two siblings.", "score": 0.00980392156862745, "content": "There are reports of the familial occurrence of Kawasaki disease but only a few reports described Kawasaki disease in siblings. However, the familial cases were not simultaneous. In these patients the idea of infective agents as trigger must be considered. We describe two siblings with atypical presentations of Kawasaki disease; the sister was first diagnosed as having parvovirus infection with anemia and the brother was diagnosed as having myocarditis. The first patient was a 9-month-old Caucasian girl with fever, conjunctivitis, rash, and pharyngitis, and later she had cervical adenopathy, diarrhea and vomiting, leukocytosis, and anemia, which were explained by positive immunoglobulin M against parvovirus. However, coronary artery lesions with aneurysms were documented at day 26 after fever onset. An infusion of intravenous immunoglobulin and high doses of steroids were not efficacious to resolve the coronary lesions. She was treated with anakinra, despite a laboratory test not showing inflammation, with prompt and progressive improvement of coronary lesions. Her 7-year-old Caucasian brother presented vomiting and fever at the same time as she was unwell, which spontaneously resolved after 4 days. Four days later, he again presented with fever with abdominal pain, associated with tachypnea, stasis at the pulmonary bases, tachycardia, gallop rhythm, hypotension, secondary anuria, and hepatomegaly. An echocardiogram revealed a severe hypokinesia, with a severe reduction of the ejection fraction (20%). He had an increase of immunoglobulin M anti-parvovirus, tested for the index case of his sister, confirming the suspicion of viral myocarditis. He received dopamine, dobutamine, furosemide plus steroids, with a progressive increase of the ejection fraction to 50%. However, evaluating his sister's history, the brother showed a myocardial dysfunction secondary to Kawasaki shock syndrome. We report on familial Kawasaki disease in two siblings which had the same infectious trigger (a documented parvovirus infection). The brother was diagnosed as having post-viral myocarditis. However, in view of the two different and simultaneous evolutions, the girl showed Kawasaki disease with late coronary artery lesions and aneurysms, whereas the brother showed Kawasaki shock syndrome with myocardial dysfunction. We stress the effectiveness of anakinra in non-responder Kawasaki disease and the efficacy on coronary aneurysms."}, {"id": "article-31346_3", "title": "Wheezing -- Introduction", "score": 0.00980392156862745, "content": "The presence of wheezing does not always mean that the patient has asthma, and a proper history and physical exam are required to make the diagnosis. [1] [2] [3] [4]"}, {"id": "Pharmacology_Katzung_4939", "title": "Pharmacology_Katzung", "score": 0.009708737864077669, "content": "A 45-year-old man is brought to the local hospital emer-gency department by ambulance. His wife reports that he had been in his normal state of health until 3 days ago when he developed a fever and a productive cough. Dur-ing the last 24 hours he has complained of a headache and is increasingly confused. His wife reports that his medical history is significant only for hypertension, for which he takes hydrochlorothiazide and lisinopril, and that he is allergic to amoxicillin. She says that he developed a rash many years ago when prescribed amoxicillin for bron-chitis. In the emergency department, the man is febrile (38.7°C [101.7°F]), hypotensive (90/54 mmHg), tachypneic (36/min), and tachycardic (110/min). He has no signs of meningismus but is oriented only to person. A stat chest x-ray shows a left lower lung consolidation consistent with pneumonia. A CT scan is not concerning for lesions or elevated intracranial pressure. The plan is to start empiric antibiotics and perform a"}, {"id": "article-107983_11", "title": "Paramyxovirus -- History and Physical", "score": 0.009708737864077669, "content": "Measles : Typically, measles presents with the classic three “C”s: cough, coryza (inflammation of the mucous membranes lining the nasal cavity) and conjunctivitis. Fevers are common, along with a generalized maculopapular rash. The three “C”s usually appear the first 10 – 12 days after infection, followed by the rash. On physical exam, the Koplik spots, tiny grains of white papules surrounded by red halo on the buccal mucosa, can be noted a day or two prior to the maculopapular rash on the skin. The rash spreads on the face first, then caudally. The disease usually resolves on its own within a week after the onset of rash. [4]"}, {"id": "pubmed23n0352_3396", "title": "Persistent cough in an adolescent.", "score": 0.009615384615384616, "content": "Jessica, a 14-year-old girl with a history of asthma, went to her pediatrician's office because of a persistent cough. She had been coughing for at least 3 months with occasional cough-free periods of less than a few days. The cough was nonproductive and was not accompanied by fever, rhinorrhea, or facial or chest pain. Jessica and her mother observed that the cough increased with exercise and typically was not present during sleep. She has used two metered-dose inhalers--albuterol and cromolyn--without any change in the cough pattern. For the past 5 years, Jessica has had mild asthma responsive to albuterol. She enjoys running on the cross-country team, soccer, and dancing. She is an average student and denies any change in academic performance. She has never been hospitalized or had an emergency department visit for asthma or pneumonia. There has been no recent travel or exposure to a person with a chronic productive cough, tobacco smoke, or a live-in pet. Jessica lives with her mother and younger sister in a 10-year-old, carpeted apartment without any evidence of mold or recent renovation. In the process of taking the history, the pediatrician noticed that Jessica coughed intermittently, with two or three coughs during each episode. At times, the cough was harsh; at other times, it was a quiet cough, as if she were clearing her throat. She was cooperative, without overt anxiety or respiratory distress. After a complete physical examination with normal findings, the pediatrician interviewed Jessica and her mother alone. Jessica's parents had been divorced for the past 6 years. She lived with her mother but visited her father, and his new family with two young children, every weekend. She spoke about this arrangement comfortably and said that she loved her father and mother but didn't like the tension she experienced at her father's home. \"I don't like adults arguing when kids are around.\" When asked why she thought the cough persisted so long, she commented in a neutral tone, \"I don't know. It's never been like this before.\" Jessica's pediatrician prescribed an inhaled steroid with the albuterol. When the cough did not respond after 1 week, he ordered a chest radiograph (normal) and a tuberculin skin test (purified protein derivative-negative), and he added montelukast (a leukotriene inhibitor) and monitored airway resistance with a peak flow meter. The cough persisted, and the peak flow recording showed normal airway resistance. At this time, Jessica's pediatrician suspected a conversion reaction and contemplated the next best therapeutic strategy."}, {"id": "article-18645_19", "title": "Acute Bronchitis -- History and Physical", "score": 0.009615384615384616, "content": "Following an episode of acute bronchitis, the cough typically persists for 10 to 20 days, with a median duration of 18 days. [20] Occasionally, bronchitis cough may extend beyond 4 weeks. Paroxysms of cough accompanied by an inspiratory whoop or posttussive emesis should raise concerns for pertussis infection."}, {"id": "InternalMed_Harrison_14683", "title": "InternalMed_Harrison", "score": 0.009538950715421303, "content": "In children, adenoviruses cause a variety of clinical syndromes. The most common is an acute upper respiratory tract infection, with prominent rhinitis. On occasion, lower respiratory tract disease, including bronchiolitis and pneumonia, also develops. Adenoviruses, particularly types 3 and 7, cause pharyngoconjunctival fever, a characteristic acute febrile illness of children that occurs in outbreaks, most often in summer camps. The syndrome is marked by bilateral conjunctivitis in which the bulbar and palpebral conjunctivae have a granular appearance. Low-grade fever is frequently present for the first 3–5 days, and rhinitis, sore throat, and cervical adenopathy develop. The illness generally lasts for 1–2 weeks and resolves spontaneously. Febrile pharyngitis without conjunctivitis has also been associated with adenovirus infection. Adenoviruses have been isolated from cases of whooping cough with or without Bordetella pertussis; the significance of adenovirus in that disease is"}, {"id": "pubmed23n0652_12320", "title": "Adult Kawasaki's disease with myocarditis, splenomegaly, and highly elevated serum ferritin levels.", "score": 0.009523809523809525, "content": "Kawasaki's disease is a disease of unknown cause. The characteristic clinical features of Kawasaki's disease are fever> or =102 degrees F for> or =5 days accompanied by a bilateral bulbar conjunctivitis/conjunctival suffusion, erythematous rash, cervical adenopathy, pharyngeal erythema, and swelling of the dorsum of the hands/feet. Kawasaki's disease primarily affects children and is rare in adults. In children, Kawasaki's disease is more likely to be associated with aseptic meningitis, coronary artery aneurysms, and thrombocytosis. In adult Kawasaki's disease, unilateral cervical adenopathy, arthritis, conjunctival suffusion/conjunctivitis, and elevated serum transaminases (serum glutamic oxaloacetic transaminase [SGOT]/serum glutamate pyruvate transaminase [SGPT]) are more likely. Kawasaki's disease in adults may be mimicked by other acute infections with fever and rash, that is, group A streptococcal scarlet fever, toxic shock syndrome (TSS), and Rocky Mountain Spotted Fever (RMSF). Because there are no specific tests for Kawasaki's disease, diagnosis is based on clinical criteria and the syndromic approach. In addition to rash and fever, scarlet fever is characterized by circumoral pallor, oropharyngeal edema, Pastia's lines, and peripheral eosinophilia, but not conjunctival suffusion, splenomegaly, swelling of the dorsum of the hands/feet, thrombocytosis, or an elevated SGOT/SGPT. In TSS, in addition to rash and fever, there is conjunctival suffusion, oropharyngeal erythema, and edema of the dorsum of the hands/feet, an elevated SGOT/SGPT, and thrombocytopenia. Patients with TSS do not have cervical adenopathy or splenomegaly. RMSF presents with fever and a maculopapular rash that becomes petechial, first appearing on the wrists/ankles after 3 to 5 days. RMSF is accompanied by a prominent headache, periorbital edema, conjunctival suffusion, splenomegaly, thrombocytopenia, an elevated SGOT/SGPT, swelling of the dorsum of the hands/feet, but not oropharyngeal erythema. We present a case of adult Kawasaki's disease with myocarditis and splenomegaly. The patient's myocarditis rapidly resolved, and he did not develop coronary artery aneurysms. In addition to splenomegaly, this case of adult Kawasaki's disease is remarkable because the patient had highly elevated serum ferritin levels of 944-1303 ng/mL; (normal<189 ng/mL). To the best of our knowledge, this is the first report of adult Kawasaki's disease with highly elevated serum ferritin levels. This is also the first report of splenomegaly in adult Kawasaki's disease. We conclude that Kawasaki's disease should be considered in the differential diagnosis in adult patients with rash/fever for> or =5 days with conjunctival suffusion, cervical adenopathy, swelling of the dorsum of the hands/feet, thrombocytosis and otherwise unexplained highly elevated ferritin levels."}, {"id": "wiki20220301en315_19584", "title": "Elisha Bartlett", "score": 0.009523809523809525, "content": "During his tenure he was faced with the challenges of the Lowell Mill Girls strike in 1836, and the Panic of 1837. Writings An Address of the Birth of Spurzheim (1838) The history, diagnosis, and treatment of typhoid and of typhus fever, with an essay on the diagnosis of bilious remittent and of yellow fever (1842). An sssay on the philosophy of medical science, (1844). The history, diagnosis, and treatment of the fevers of the United States, (1847). An inquiry into the degree of certainty in medicine: and into the nature and extent of its power over disease, (1848). The history, diagnosis, and treatment of edematous laryngitis, (1850). References Bibliography"}, {"id": "pubmed23n1113_1240", "title": "Acute Appendicitis Associated With Kawasaki Disease: Case Report and Review of the Literature.", "score": 0.009433962264150943, "content": "Acute appendicitis is a rare complication of Kawasaki disease in the setting of the absence of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We experienced a rare case of acute appendicitis associated with Kawasaki disease. The patient is a six-year-old male who was brought to the emergency department by his mother with a pruritic rash, nausea, vomiting, and abdominal pain. Given fever, tenderness in the right lower quadrant on physical examination, leukocytosis with bandemia, and a non-compressible and dilated appendix on ultrasound, he was diagnosed with acute appendicitis and was treated with a laparoscopic appendectomy. He developed persistent fevers after surgery with new lip swelling, mucositis, and bilateral conjunctival injection. Kawasaki disease was suspected and intravenous gammaglobulin and aspirin were administrated. He made a full recovery. This case suggests that careful examination is needed for accurate diagnosis, especially in patients with postoperative persistent fever without signs of intra-abdominal complications. We performed a PubMed literature search and reviewed eight cases of appendicitis associated with Kawasaki disease. Of note, this case was seen in 2018 before the SARS-CoV-2 pandemic and the description of multisystem inflammatory syndrome in children (MIS-C)."}, {"id": "wiki20220301en155_33242", "title": "Eduard Bloch", "score": 0.009433962264150943, "content": "Hitler family doctor The first member of the Hitler family Bloch was to see was Adolf Hitler. In 1904, Hitler had become seriously ill and was bedridden due to a serious lung ailment. Due to this, he was allowed to abandon his school career and return home. However, after checking Hitler's files, Bloch later maintained that he had treated the youth for only minor ailments, cold or tonsilitis and that Hitler had been neither robust nor sickly. He also stated that Hitler did not have any illness whatsoever, let alone a lung disease."}, {"id": "pubmed23n1045_11514", "title": "A 16-Year-Old Boy With Cough and Fever in the Era of COVID-19.", "score": 0.009345794392523364, "content": "A 16-year-old white boy with a history of chronic lung disease of prematurity, cough-variant asthma, and incidental lung nodules presented to the emergency center in spring 2020 with acute onset dry cough, shortness of breath, and fever. An initial history, gathered from his mother because of the patient's respiratory distress, revealed no recent travel. However, his mother is a health care worker at a hospital, and sick contacts included ongoing contact with a friend with cold-like symptoms. He had a variety of animals at home, including a dog, cats, fish, rodents, and reptiles. He had a history of vaping tobacco products >6 months ago. Fever and respiratory symptoms were associated with fatigue, chest tightness, abdominal pain, and myalgias. On examination, he was ill appearing and had tachycardia, tachypnea, borderline hypoxia with an oxygen saturation of 91% on room air, diminished breath sounds at the lung bases, and unremarkable abdominal examination results. A chest radiograph was consistent with the lung examination, revealing bilateral lower lobe hazy infiltrates. He showed initial improvement for 48 hours with antibiotics, intravenous fluid resuscitation, oxygen via nasal cannula, albuterol, and prednisone. Subsequently, he worsened with persistent high fever, increasing respiratory distress with pulmonary findings, and severe persistent epigastric pain, which added a layer of diagnostic complexity. As this patient's clinical course evolved and further history became available, pulmonary medicine and infectious diseases services were consulted to guide diagnostic evaluation and treatment of this patient early in the era of coronavirus disease 2019."}, {"id": "wiki20220301en495_24326", "title": "Édouard Rist", "score": 0.009345794392523364, "content": "Selected works Études bactériologiques sur les infections d'origine otique, 1898 – Bacteriological studies on the origin of otic infections. Précis de pathologie exotique, (with Édouard Jeanselme), 1909 – Specifics of exotic pathology. La tuberculose, 1927 – Tuberculosis. Séméiologie élémentaire de l'appareil respiratoire, 1934 – Elementary respiratory symptomatology. Les Symptômes de la tuberculose pulmonaire (clinique, physiologie, pathologique, thérapeutique), 1943 – Symptoms of pulmonary tuberculosis. 25 portraits de médecins français, 1900-1950, (1955) – 25 portraits of French doctors, 1900-1950. References 1871 births 1956 deaths People from Strasbourg French military doctors Tuberculosis researchers French pulmonologists"}, {"id": "pubmed23n0940_1675", "title": "Severe desquamation in Kawasaki disease: Is it somehow protective?", "score": 0.009259259259259259, "content": "Kawasaki disease is a common vasculitis that typically affects children between one and five years of age. We report a 12-year-old boy who presented following a presumed diagnosis of pharyngitis associated with nondesquamating skin rash and conjunctivitis. Despite treatment with amoxicillin for seven days his fever persisted for ten days and then remitted. Two weeks later, he developed full thickness extensive desquamation of his palms and soles that mandated a visit to emergency department in our tertiary health centre. Physical examination revealed full thickness desquamation of his palms and soles with absence of erythema or swelling and he had unremarkable systemic examination. Laboratory tests showed thrombocytosis and high erythrocytes sedimentation rate. Throat culture and Anti-streptolysin-O titer were negative. Aspirin, anti-platelets dose, was initiated. Echocardiography was performed in the first visit and repeated three times later: at four weeks, six weeks and at three months of the illness revealing normal coronary arteries. Follow up complete blood count and sedimentation rate were normal after six weeks, therefore, aspirin was discontinued. Full thickness desquamation, not as it would be expected, might be somehow protective against the involvement of coronary arteries in Kawasaki disease."}, {"id": "article-18645_23", "title": "Acute Bronchitis -- History and Physical", "score": 0.009174311926605505, "content": "During a physical examination, lung auscultation may reveal the presence of wheezing. In some instances, the detection of rhonchi may improve or clear with coughing, suggesting that the airway secretions or obstruction causing the sound can be alleviated through coughing efforts."}, {"id": "pubmed23n0492_11383", "title": "[The probability of Kawasaki diseases in young patients with cardiac disorders--retrospective studies].", "score": 0.00909090909090909, "content": "Kawasaki disease (KD) is the leading cause of acquired heart disease in children in some countries. In Poland the diagnosis of KD is still rarely reported. KD is an acute systemic febrile illness of unknown aetiology, predominantly affecting children under five years of age. Delay in diagnosis and treatment is associated with an increased risk of coronary artery aneurysms and late cardiac complications. The aim of this study was to find out whether Kawasaki disease in childhood was the cause of cardiac disorders in young patients. We retrospectively studied 50 patients (ranging in age from 20 to 47 years; 34 males and 16 females) diagnosed in 1 Cardiac Department within one year. The patients were divided into three groups: group I--patients with ischemic heart disease, myocardial infarction and congestive heart failure; group II--patients with cardiac arrhythmia and conduction abnormalities; group III--patients with congestive cardiomyopathy. Eighteen patients had rubella in childhood and in this group one person was under 5 years of age. Nineteen patients suffered from measles and in this group there were two persons under 5 years of age. Twenty-three persons in the study group confirmed acute febrile illness under 5 years of age lasting over 5 days. No cardiac symptoms occurred during the childhood febrile or exanthema illnesses. Five criteria (symptoms of Kawasaki disease) were fulfilled by 4 persons, 4 criteria--by 4 persons, 3 criteria--by 1 person, 2 criteria--by 3 persons, 1 criterion--by 6 persons. Nobody in the study population had signs of measles, rubella or scarlet fever. Nobody had coronary artery abnormalities detected by echocardiography. Twenty-six of 50 persons had coronary angiography--no coronary artery aneurysms or ectasia were detected. Completing a more precise past history with the help of patient's parents was often impossible (death, distant place of residence). The verification of diagnosis on the basis of the obtained data is impossible. The most reliable data about diseases affecting children under 5 years of age would have been produced by additional investigations. They would have facilitated the retrospective recognition of the Kawasaki disease."}, {"id": "wiki20220301en323_3245", "title": "Health of Frédéric Chopin", "score": 0.00909090909090909, "content": "On 17 October 1849, at 2 a.m., after a sudden coughing fit, Chopin died at the age of 39. His physician, Jean Cruveilhier, confirmed his death by holding a mirror to Chopin's mouth and by illuminating his pupils with light from a candle. Pursuant to Chopin's will, Dr. Cruveilhier, a renowned professor of pathology, carried out an autopsy. The postmortem findings were also communicated to Chopin's sister Ludwika, Adolphe Gutmann and Jane Stirling. The postmortem report was destroyed either in the Paris fire of 1871 or during World War II. The death certificate stated the cause of Chopin's death as tuberculosis of the lungs and larynx. However, Wojciech Grzymała, in a letter to Auguste Leo dated October 1849, wrote that the autopsy had not confirmed tubercular pulmonary changes and that his actual disease was unknown to contemporary medicine."}, {"id": "InternalMed_Harrison_14625", "title": "InternalMed_Harrison", "score": 0.00907082608113536, "content": "The most common clinical manifestations of rhinovirus infections are those of the common cold. Illness usually begins with rhinorrhea and sneezing accompanied by nasal congestion. The throat is frequently sore, and in some cases sore throat is the initial complaint. Systemic signs and symptoms, such as malaise and headache, are mild or absent, and fever is unusual in adults but may occur in up to one-third of children. Illness generally lasts for 4–9 days and resolves spontaneously without sequelae. In children, bronchitis, bronchiolitis, and bronchopneumonia have been reported; nevertheless, it appears that rhinoviruses are not major causes of lower respiratory tract disease in children. Rhinoviruses may cause exacerbations of asthma and chronic pulmonary disease in adults. The vast majority of rhinovirus infections resolve without sequelae, but complications related to obstruction of the eustachian tubes or sinus ostia, including otitis media or acute sinusitis, can develop. In"}, {"id": "article-20073_6", "title": "Cough -- Etiology", "score": 0.009056378937960826, "content": "Pertussis, also known as whooping cough, is an illness with a classic clinical finding of paroxysmal episodes of intense coughing lasting up to several minutes followed by a loud gasp for air. It is an infection of the respiratory tract by Bordetella pertussis, where the bacterium induces mucopurulent sanguineous exudate formation within the respiratory tract. The overall course of pertussis infection lasts up to six weeks and is characterized by three stages: a catarrhal phase, a paroxysmal phase, and a convalescent phase. The catarrhal phase is characterized by rhinorrhea, sneezing, low fever, tearing, and nasal congestion. The paroxysmal phase occurs within two weeks of colonization and is characterized by classic coughing episodes followed by post-tussive vomiting. The convalescent phase is a condition of chronic coughing that may last for weeks. This illness is a serious diagnosis that requires prompt attention as it remains one of the highest causes of infant morbidity and mortality. [2]"}]}}}} {"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 390]], "word_ranges": [[0, 56]], "text": "The femoral nerve (although rare) may be damaged in a cesarean section, hysterectomy or lower abdominal operation. This nerve injury produces hypoesthesia and weakness along its distribution. Femoroneuropathy is manifested by paralysis (25% of cases) of the quadriceps muscles, abolition of patellar reflexes and hypoesthesia of the anterior and inner thigh. Therefore, answer 1 is correct."}, "2": {"exist": true, "char_ranges": [[391, 654]], "word_ranges": [[56, 104]], "text": "The femoral cutaneous or lateral femoral cutaneous nerve (L2,L3) is only sensitive and is responsible for the sensitivity of the skin of the lateral and anterior aspect of the thigh up to the knee, so it would not explain the motor problem of gait and 2 is false."}, "3": {"exist": true, "char_ranges": [[655, 1060]], "word_ranges": [[104, 170]], "text": "The obturator nerve (L2-L4) is a mixed nerve. At the motor level, it invests the obturator externus, pectineus, short approximator, the approximators (adductors) and gracilis. At the sensory level the capsule of the coxofemoral joint, medial aspect of the thigh above the knee. Therefore neither affects the sensitivity of the anterior aspect of the thigh nor the extension of the knee so that 3 is false."}, "4": {"exist": true, "char_ranges": [[1061, 1356]], "word_ranges": [[170, 230]], "text": "A lesion of the sciatic nerve at motor level affects to the flexion of the knee not to its extension and at distal level to this one (they do not count anything of distal clinic) and at sensitive level also it is distal in the external face of the leg and in the foot reason why 4 also is false."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The femoral nerve (although rare) may be damaged in a cesarean section, hysterectomy or lower abdominal operation. This nerve injury produces hypoesthesia and weakness along its distribution. Femoroneuropathy is manifested by paralysis (25% of cases) of the quadriceps muscles, abolition of patellar reflexes and hypoesthesia of the anterior and inner thigh. Therefore, answer 1 is correct. The femoral cutaneous or lateral femoral cutaneous nerve (L2,L3) is only sensitive and is responsible for the sensitivity of the skin of the lateral and anterior aspect of the thigh up to the knee, so it would not explain the motor problem of gait and 2 is false. The obturator nerve (L2-L4) is a mixed nerve. At the motor level, it invests the obturator externus, pectineus, short approximator, the approximators (adductors) and gracilis. At the sensory level the capsule of the coxofemoral joint, medial aspect of the thigh above the knee. Therefore neither affects the sensitivity of the anterior aspect of the thigh nor the extension of the knee so that 3 is false. A lesion of the sciatic nerve at motor level affects to the flexion of the knee not to its extension and at distal level to this one (they do not count anything of distal clinic) and at sensitive level also it is distal in the external face of the leg and in the foot reason why 4 also is false.", "full_answer_no_ref": "The femoral nerve (although rare) may be damaged in a cesarean section, hysterectomy or lower abdominal operation. This nerve injury produces hypoesthesia and weakness along its distribution. Femoroneuropathy is manifested by paralysis (25% of cases) of the quadriceps muscles, abolition of patellar reflexes and hypoesthesia of the anterior and inner thigh. Therefore, answer 1 is [HIDDEN]. The femoral cutaneous or lateral femoral cutaneous nerve (L2,L3) is only sensitive and is responsible for the sensitivity of the skin of the lateral and anterior aspect of the thigh up to the knee, so it would not explain the motor problem of gait and 2 is [HIDDEN]. The obturator nerve (L2-L4) is a mixed nerve. At the motor level, it invests the obturator externus, pectineus, short approximator, the approximators (adductors) and gracilis. At the sensory level the capsule of the coxofemoral joint, medial aspect of the thigh above the knee. Therefore neither affects the sensitivity of the anterior aspect of the thigh nor the extension of the knee so that 3 is [HIDDEN]. A lesion of the sciatic nerve at motor level affects to the flexion of the knee not to its extension and at distal level to this one (they do not count anything of distal clinic) and at sensitive level also it is distal in the external face of the leg and in the foot reason why 4 also is [HIDDEN].", "full_question": "A woman presenting with difficulty walking after gynecologic surgery. She has mild thigh pain and leg failure on support. On examination she has weakness in hip flexion and knee extension, and dysesthesias in the anterior aspect of the thigh. What is the most probable diagnosis of suspicion:", "id": 467, "lang": "en", "options": {"1": "Femoral nerve neuropathy.", "2": "Meralgia paresthetica of the femoral cutaneous nerve.", "3": "Obturator nerve neuropathy.", "4": "Neuropathy of the sciatic nerve.", "5": NaN}, "question_id_specific": 92, "type": "ORTHOPEDIC SURGERY AND TRAUMATOLOGY", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en624_29739", "title": "Femoral nerve dysfunction", "score": 0.01960972796308757, "content": "Those with femoral nerve dysfunction may present problems of difficulties in movement and a loss of sensation. The patient, in terms of motor skills, may have problems such as quadriceps wasting, loss of knee extension and a lesser extent of hip flexion given the femoral nerve involvement of the iliacus and pectineus muscles. One may experience numbness and tingling in any part of the leg, typically in the front and the inside of one’s thighs and down to the feet. They may also experience a dull ache in the genital region given that the inguinal ligament is actually divided into the femoral and genital branches. Feelings of the patient’s leg and knees giving out may also be prevalent due to lower extremity muscle weakness and quadriceps weakness. In terms of sensory skills, patients may observe a decrease in sensation over the front and medial sections of the thigh and medial aspects of the lower legs and feet due to their involvement of the anterior and medial cutaneous nerves of the"}, {"id": "wiki20220301en624_29744", "title": "Femoral nerve dysfunction", "score": 0.017740429505135387, "content": "The diagnosis of femoral neuropathy can be done through physical examinations, several imaging techniques and electrodiagnostic studies. Provided patients do not suffer from haemorrhage, physical examinations is the first line of diagnosis. These examinations are carried out in order to evaluate whether nerves of the lower back, lower limbs and hips are functioning well. They can also help determine whether it is strictly an injury in the femoral nerve or a systemic disorder. Other than questioning about possible recent injuries, surgeries, and medical history, inspection of asymmetry or atrophy of the quadriceps muscles, muscle stretch reflexes, and sensory testing through pinpricks and light touches are conducted. By looking at the asymmetry or atrophy of the quadriceps muscles, weaknesses in knee extension or hip flexion can be observed. Furthermore, physicians palpate over the inguinal ligament to inspect the anterior and medial leg, anterior thigh, and quadriceps reflex. In"}, {"id": "wiki20220301en624_29745", "title": "Femoral nerve dysfunction", "score": 0.016657638136511378, "content": "in knee extension or hip flexion can be observed. Furthermore, physicians palpate over the inguinal ligament to inspect the anterior and medial leg, anterior thigh, and quadriceps reflex. In addition, comparison of quadriceps strength to adductor strength help point towards femoral neuropathy. However, given that the diagnosis of femoral neuropathy through physical examination is subject to how severe the injury is, additional imaging testing such as computed tomography, magnetic resonance imaging, ultrasounds and nerve conduction studies and electromyography are also done."}, {"id": "wiki20220301en347_22187", "title": "Hip arthroscopy", "score": 0.014968082764692935, "content": "Damage to nerves around the hip joint can occur, sometimes because of direct damage with the surgical instruments, or as a result of the traction needed to gain access to the joint. For this reason, surgeons prefer to apply as little traction as possible, for as short a time as possible, in order to gain safe access to the joint. The most common nerve to be injured is the lateral cutaneous nerve of the thigh. This nerve supplies feeling to the upper, outer thigh. Other nerves that may be affected are the sciatic (leading to a weakness lifting the foot – 'foot-drop'), femoral (weak thigh muscles), obturator (numbness in the inner thigh and weakness of those muscles), and pudendal nerves. The pudendal nerves supply feeling to the reproductive organs."}, {"id": "pubmed23n0869_7500", "title": "Ultrasound-Guided Diagnosis and Treatment of Meralgia Paresthetica.", "score": 0.013846415222562011, "content": "Meralgia paresthetica refers to the entrapment of the lateral femoral cutaneous nerve at the level of the inguinal ligament. The lateral femoral cutaneous nerve - a purely sensory nerve - arises from the L2 and L3 spinal nerve roots, travels downward lateral to the psoas muscle, and then crosses the iliacus muscle. Close to the anterior superior iliac spine, the nerve courses in contact with the lateral aspect of the inguinal ligament and eventually innervates the lateral thigh. The entrapment syndrome is usually idiopathic but can also ensue due to trauma/overuse, pelvic and retroperitoneal tumors, stretching of the nerve due to prolonged leg/trunk hyperextension, leg length discrepancies, prolonged standing, external compression by belts, weight gain, and tight clothing. The diagnosis of Meralgia paresthetica is usually clinical, i.e., based on the following symptoms: paresthesia, numbness, burning sensation, dysesthesia, and pain over the anterolateral aspects of the thigh. These complaints may be worsened by walking or prolonged standing and typically disappear after weight loss, abdominal muscle strengthening, or elimination of the underlying cause. Although there are several reports on the confirmatory role of electrodiagnostic studies in the diagnosis of Meralgia paresthetica, electromyographers would usually prefer/suggest not to perform nerve conduction studies in daily clinical practice. Herewith, due to its several advantages, ultrasound imaging has been proposed as an alternative diagnostic method in the recent literature. It not only confirms the entrapment morphologically, but also uncovers a likely underlying cause and provides immediate interventional guidance. The pertinent sonographic findings would be hypoechoic and swollen lateral femoral cutaneous nerve."}, {"id": "pubmed23n0559_6872", "title": "Meralgia paresthetica caused by hip-huggers in a patient with aberrant course of the lateral femoral cutaneous nerve.", "score": 0.013306982872200264, "content": "\"Hip-huggers\" may be a precipitating factor for meralgia paresthetica (MP), especially in thin persons with an aberrant pathway of the lateral femoral cutaneous nerve (LFCN). We describe a 25-year-old woman with a long-standing history of MP caused by an abnormal course of the LFCN and tight trousers, specifically hip-huggers. Ultrasonography was useful for detecting the lesion site and the abnormal pathway of the LFCN. After neurectomy of the LFCN, most of the symptoms of MP were relieved, but mild hypesthesia remained in the lateral thigh."}, {"id": "InternalMed_Harrison_31210", "title": "InternalMed_Harrison", "score": 0.013276434329065907, "content": "2692 LATERAL FEMORAL CUTANEOUS NEUROPATHY (MERALGIA PARESTHETICA) The lateral femoral cutaneous nerve arises from the upper lumbar plexus (spinal levels L2/3), crosses through the inguinal ligament near its attachment to the iliac bone, and supplies sensation to the anterior lateral thigh. The neuropathy affecting this nerve is also known as meralgia paresthetica. Symptoms and signs consist of paresthesias, numbness, and occasionally pain in the lateral thigh. Symptoms are increased by standing or walking and are relieved by sitting. There is normal strength, and knee reflexes are intact. The diagnosis is clinical, and further tests usually are not performed. EDx is only needed to rule out lumbar plexopathy, radiculopathy, or femoral neuropathy. If the symptoms and signs are classic, EMG is not necessary. Symptoms often resolve spontaneously over weeks or months, but the patient may be left with permanent numbness. Treatment consists of weight loss and avoiding tight belts. Analgesics"}, {"id": "pubmed23n0905_23173", "title": "[A Case of Meralgia Paresthetica Treated with Neurolysis].", "score": 0.013254850664922609, "content": "A 60-year-old woman presented with a 1-year history of pain and numbness in the left anterolateral thigh. The symptoms aggravated on walking and standing. Her visual analogue scale(VAS)score was 7.1/10. Tinel's like sign was positive over the lateral femoral cutaneous nerve(LFCN), in the inguinal ligament region. LFCN block at the trigger point, in the inguinal ligament, resulted in relief of the symptoms and we diagnosed meralgia paresthetica(MP), which is the entrapment neuropathy of the LFCN. Initially, we performed observation therapy with oral medication and LFCN blocks. However, these treatments failed to relieve the symptoms. Therefore, we performed neurolysis with a microscope under local anesthesia. The symptoms improved immediately after surgery and her VAS score of thigh symptom improved from 7.1 to 1.9 after 3 months. Conservative and surgical treatment for MP generally yield good outcome and we should pay attention to the MP as a differential diagnosis for thigh numbness and pain."}, {"id": "pubmed23n0322_12572", "title": "Bilateral femoral neuropathy after vaginal hysterectomy.", "score": 0.01307497283592901, "content": "A case of bilateral femoral neuropathy as a complication of vaginal hysterectomy is presented. A 45-year-old woman developed weakness of both quadriceps, absence of bilateral knee jerks, and numbness over bilateral anteromedial thighs and medial lower legs after a vaginal hysterectomy. Electromyographic examination revealed evidence of denervation in the bilateral quadriceps. A nerve conduction study showed prolonged distal latencies and markedly reduced amplitude of the compound muscle action potentials in bilateral femoral nerves. It is suggested that this complication is caused by a microvascular and/or local mechanical injury of the femoral nerve, which is compressed beneath the tough inguinal ligament in a sustained posture with the hip joint in an extreme abduction and external rotation position. The prognosis was excellent with almost complete recovery within 10 weeks. The complication may be preventable by minimizing operating time, changing the patient's posture, and limiting the degree of flexion, abduction, and external rotation of the hip."}, {"id": "article-21681_25", "title": "Anatomy, Bony Pelvis and Lower Limb: Thigh Femoral Nerve -- Clinical Significance", "score": 0.012968478530652767, "content": "Clinically, patients will have quadriceps wasting, loss of knee extension, and to a lesser extent, hip flexion due to the involvement of the iliacus and pectineus muscles. On the sensory side, there will be a loss of sensation over the anterior and medial thigh due to the involvement of the anterior and medial cutaneous nerves of the thigh. Furthermore, loss of sensation will occur over the medial aspect of the lower leg and foot due to the involvement of the saphenous nerve. Although the prognosis in most cases is satisfactory, some cases necessitate nerve repair or grafting, and some cases cause permanent residual neurologic deficits."}, {"id": "InternalMed_Harrison_31213", "title": "InternalMed_Harrison", "score": 0.0127390408943807, "content": "Sciatic neuropathies commonly complicate hip arthroplasty, pelvic procedures in which patients are placed in a prolonged lithotomy position, trauma, hematomas, tumor infiltration, and vasculitis. In addition, many sciatic neuropathies are idiopathic. Weakness may involve all motions of the ankles and toes as well as flexion of the leg at the knee; abduction and extension of the thigh at the hip are spared. Sensory loss occurs in the entire foot and the distal lateral leg. The ankle jerk and on occasion the internal hamstring reflex are diminished or more typically absent on the affected side. The peroneal subdivision of the sciatic nerve is typically involved disproportionately to the tibial counterpart. Thus, patients may have only ankle dorsiflexion and eversion weakness with sparing of knee flexion, ankle inversion, and plantar flexion; these features can lead to misdiagnosis of a common peroneal neuropathy."}, {"id": "wiki20220301en503_1201", "title": "List of neuromuscular disorders", "score": 0.012726481127968117, "content": "Lower extremity deep peroneal mononeuropathy at the fibular neck common fibular mononeuropathy at the hip deep fibular mononeuropathy at the ankle superficial fibular mononeuropathy sciatic mononeuroapthy at the hip or thigh piriformis syndrome proximal tibial mononeuropathy tarsal tunnel syndrome interdigital neuropathy (Morton's Neuroma) sural mononeuropathy femoral mononeuropathy saphenous mononeuropathy lateral femoral cutaneous neuropathy ilioinguinal neuropathy iliohypogastric neuropathy genitofemoral neuropathy posterior femoral cutaneous neuropathy obturator neuropathy neuropathy of gluteal nerves"}, {"id": "InternalMed_Harrison_31556", "title": "InternalMed_Harrison", "score": 0.012266337999237022, "content": "The symptoms of lateral femoral cutaneous nerve entrapment, commonly known as “meralgia paresthetica,” include sensory loss, pain, and dysesthesia in part of the area supplied by the nerve (Fig. 463e-3E). There is no motor component to the nerve, and therefore weakness is not a part of this syndrome. Symptoms often are worsened by standing or walking. Compression of the nerve occurs where it enters the leg near the inguinal ligament, usually in the setting of tight-fitting belts, pants, corsets, or recent weight gain, including that of pregnancy. The differential diagnosis of these symptoms includes hip problems such as trochanteric bursitis."}, {"id": "pubmed23n1049_9587", "title": "Clinical Presentations of Lumbar Disc Degeneration and Lumbosacral Nerve Lesions.", "score": 0.012152777777777776, "content": "Lumbar disc degeneration is defined as the wear and tear of lumbar intervertebral disc, and it is mainly occurring at L3-L4 and L4-S1 vertebrae. Lumbar disc degeneration may lead to disc bulging, osteophytes, loss of disc space, and compression and irritation of the adjacent nerve root. Clinical presentations associated with lumbar disc degeneration and lumbosacral nerve lesion are discogenic pain, radical pain, muscular weakness, and cutaneous. Discogenic pain is usually felt in the lumbar region, or sometimes, it may feel in the buttocks, down to the upper thighs, and it is typically presented with sudden forced flexion and/or rotational moment. Radical pain, muscular weakness, and sensory defects associated with lumbosacral nerve lesions are distributed on lower extremities, the buttock, lower abdomen, and groin region. A lumbosacral plexus lesion presents different symptoms in the territories of the lumbar and sacral nerves. Patients with lumbar plexus lesion clinically present with weakness of hip flexion, knee extension, thigh adduction, and sensory loss in the lower abdomen, inguinal region, and over the entire medial, lateral, and anterior surfaces of the thigh and the medial lower leg, while sacral plexus lesion presents clinical symptoms at nerve fibers destined for the sciatic nerve, common peroneal nerve, and pudendal nerve. Weakness of ankle inversion, plantar flexion, and foot drop are the main clinical manifestations of the sacral plexus lesion area. Numbness and decreased sensation are also present along the anterolateral calf and dorsum of the foot. On examination, foot eversion is usually stronger than foot dorsiflexion. The patients may also present with pain and difficulty of bowel movements, sexual dysfunction assessments, and loss of cutaneous sensation in the areas of the anal canal, anus, labia major, labia minor, clitoris, penis, and scrotum."}, {"id": "wiki20220301en430_36822", "title": "Local anesthetic nerve block", "score": 0.01211548839719374, "content": "3-in-1 nerve block is indicated for pain relief for hip fractures. The femoral nerve block is indicated for femur, anterior thigh, and knee surgery. It is performed slightly inferior to the inguinal ligament, and the nerve is under the fascia iliaca. The sciatic nerve block is done for surgeries at or below the knee. The nerve is located in the gluteus maximus muscle. The popliteal block is done for ankle, achilles tendon, and foot surgery. It is done above the knee on the posterior leg where the sciatic nerve starts splitting into the common peroneal and tibial nerves. The saphenous nerve block is often done in combination with the popliteal block for surgeries below the knee. The saphenous nerve is numbed at the medial part of the lower thigh under the sartorius muscle."}, {"id": "wiki20220301en071_57955", "title": "Iliopsoas", "score": 0.01163660543595711, "content": "It is a typical posture muscle dominated by slow-twitch red type 1 fibers. Since it originates from the lumbar vertebrae and discs and then inserts onto the femur, any structure from the lumbar spine to the femur can be affected directly. A short and tight iliopsoas often presents as externally rotated legs and feet. It can cause pain in the low or mid back, SI joint, hip, groin, thigh, knee, or any combination. The iliopsoas gets innervation from the L2-4 nerve roots of the lumbar plexus which also send branches to the superficial lumbar muscles. The femoral nerve passes through the muscle and innervates the quadriceps, pectineus, and sartorius muscles. It also comprises the intermediate femoral cutaneous and medial femoral cutaneous nerves which are responsible for sensation over the anterior and medial aspects of the thigh, medial shin, and arch of the foot nerves. The obturator nerve also passes through the muscle which is responsible for the sensory innervation of the skin of"}, {"id": "article-145849_57", "title": "Foot Drop in Obstetrics -- Evaluation -- Tests for Obturator Internus Neuropathy", "score": 0.011318813239581546, "content": "The patient lies supine with the thigh flexed at the hip and the leg flexed at the knee. The examiner has one hand on the medial side of the knee and the other on the ankle. The patient then adducts the thigh against resistance to determine weakness or pain due to what appears to be an obturator neuropathy. The other side is tested for comparison purposes."}, {"id": "wiki20220301en079_43607", "title": "Hip dislocation", "score": 0.011, "content": "Posterior dislocation For posterior dislocation, the affected limb will be in a position of flexion, adduction, and internal rotation. This is to say, the affected leg will be bent upwards at the hip, while being shifted and pointed towards the middle of the body. Sciatic nerve injury is also present in 8%-20% of cases, conferring numbness and weakness to aspects of the lower leg. Anterior dislocation For anterior dislocation, the affected limb will be in a position of abduction and external rotation. The degree of flexion depends on whether it is a superior or inferior dislocation, with the former resulting in hip extension and the latter, hip flexion. This is to say that with superior and inferior anterior dislocations, the affected leg will be bent at the hip backwards and upwards respectively, while being shifted and pointed away from the body. Femoral nerve palsies can also be present, conferring leg numbness and weakness, however are uncommon. Diagnosis"}, {"id": "InternalMed_Harrison_31554", "title": "InternalMed_Harrison", "score": 0.010945134720089348, "content": "Lesions of the proximal femoral nerve are relatively uncommon but may present dramatically with weakness of hip flexion, quadriceps atrophy, weakness of knee extension (often manifesting with leg-buckling falls), and an absent patellar reflex. Adduction of the thigh is spared as these muscles are supplied by the obturator nerve, thereby distinguishing a femoral neuropathy from a more proximal lumbosacral plexus lesion. The sensory loss found is in the distribution of the femoral nerve sensory branches including the anterior part of the thigh (Fig. 463e-3D). Compressive lesions from retroperitoneal hematomas or masses are common, and a CT of the pelvis should be obtained in all cases of femoral neuropathy to exclude these conditions. Bleeding into the pelvis resulting in hematoma can occur spontaneously, following trauma, or after intrapelvic surgeries such as renal transplantation. In intoxicated or comatose patients, stretch injuries to the femoral nerve are seen following prolonged,"}, {"id": "pubmed23n1113_5399", "title": "A cook with 'burning in the thigh' and a 'hotspot' in the groin!", "score": 0.010610751477624542, "content": "Meralgia paresthetica (MP) is a condition characterised by abnormal sensations on the anterolateral aspect of the thigh due to the dysfunction of the lateral femoral cutaneous nerve. Here, I present a case of a 64-year-old female cook who attended the General Medicine clinic with 2 months of persistent numbness and 'burning' sensation over the right anterolateral thigh. Subsequent physical examination revealed the diagnosis of meralgia paresthetica. The significance of good history taking and thorough physical examination in reaching the diagnosis of meralgia paresthetica cannot be overemphasized. In most typical presentations, advanced imaging and neurodiagnostic testing do not add value to confirm the diagnosis. If the clinical diagnosis is doubtful, nerve conduction study and magnetic resonance imaging may still be performed to exclude other mimicking pathologies. Increasing awareness of MP among doctors unfamiliar with this condition will prevent the ordering of excessive investigations."}, {"id": "wiki20220301en624_29740", "title": "Femoral nerve dysfunction", "score": 0.010504549214226635, "content": "a decrease in sensation over the front and medial sections of the thigh and medial aspects of the lower legs and feet due to their involvement of the anterior and medial cutaneous nerves of the thigh and the saphenous nerve respectively."}, {"id": "Neurology_Adams_10931", "title": "Neurology_Adams", "score": 0.0103791030975497, "content": "This nerve is formed from the second, third, and fourth lumbar roots. Within the pelvis it passes along the lateral border of the psoas muscle and enters the thigh beneath the Poupart ligament, lateral to the femoral artery. Branches arising within the pelvis supply the iliacus and psoas muscles. Just below the Poupart ligament the nerve splits into anterior and posterior divisions. The former supplies the pectineus and sartorius muscles and carries sensation from the anteromedial surface of the thigh; the posterior division provides the motor innervation to the quadriceps and the cutaneous innervation to the medial side of the leg from the knee to the internal malleolus. The distinction between femoral neuropathy and a third lumbar root lesion is made by detecting weakness of the hip adductor (innervated by the obturator nerve) in the case of the root lesion."}, {"id": "wiki20220301en071_22782", "title": "Meralgia paraesthetica", "score": 0.010373984951203159, "content": "The nerve may become painful over a period of time as weight gain makes underwear, belting or the waistband of pants gradually exert higher levels of pressure. Pain may be acute and radiate into the rib cage, and into the groin, thigh, and knee. Alternately, weight loss or aging may remove protective fat layers under the skin, so the nerve can compress against underwear, outer clothing, and—most commonly— by belting. Long periods of standing or leg exercise that increases tension on the inguinal ligament may also cause pressure. The lateral cutaneous nerve of the thigh can occasionally be damaged during laparoscopic hernia repair, or scarring from the operation can lead to meralgia paraesthetica. Diagnosis Diagnosis is largely based on patient description and relevant details about recent surgeries, hip injuries, or repetitive activities that could irritate the nerve. Examination checks for sensory differences between the affected leg and the other leg."}, {"id": "Neurology_Adams_10933", "title": "Neurology_Adams", "score": 0.010357259914782039, "content": "The most common cause of femoral neuropathy is diabetes. The nerve may also be involved by pelvic tumors. Not uncommon is injury to the nerve during pelvic operations. Usually this is the result of improper placement of retractors, which may compress the nerve directly or indirectly by undue pressure on the psoas muscle. Bleeding into the iliacus muscle or the retroperitoneum, observed in patients receiving anticoagulants and in hemophilia patients, is a relatively common cause of isolated femoral neuropathy (Goodfellow et al). The presenting symptom of iliacus hematoma is pain in the groin spreading to the lumbar region or thigh, in response to which the patient assumes a characteristic posture of flexion and lateral rotation of the hip. A palpable mass in the iliac fossa and the signs of femoral nerve compression (quadriceps weakness and loss of knee jerk) follow in a day or two. Infarction of the nerve may occur in the course of diabetes mellitus and polyarteritis nodosa. Not"}, {"id": "article-21681_26", "title": "Anatomy, Bony Pelvis and Lower Limb: Thigh Femoral Nerve -- Clinical Significance -- Femoral Neuropathy in Obstetric Patients Undergoing Vaginal Delivery", "score": 0.01020844107660828, "content": "Femoral neuropathies may occur during vaginal delivery. Commonly, patients are put in the dorsal lithotomy position, in which the thigh is flexed at the hip, and the leg is flexed at the knee. This position compresses the femoral nerve against the inguinal ligament, compromising the blood supply to the nerve. At the end of the delivery, the patient attempts to stand and falls to the floor because the quadriceps femoris muscle is not functional due to the loss of its innervation by the femoral nerve. The quadriceps femoris muscle inserts onto the patella. The patellar ligament attaches to the tibial tuberosity. When the quadriceps femoris muscle contracts, it extends the leg at the knee. This action is crucial to prevent the leg from buckling. Thus, if one is assisting the patient in arising from a gurney after delivery, one must be able to catch the patient if she falls due to femoral neuropathy. [17]"}, {"id": "InternalMed_Harrison_31212", "title": "InternalMed_Harrison", "score": 0.010100193923723336, "content": "Femoral neuropathies can arise as complications of retroperitoneal hematoma, lithotomy positioning, hip arthroplasty or dislocation, iliac artery occlusion, femoral arterial procedures, infiltration by hematogenous malignancy, penetrating groin trauma, pelvic surgery including hysterectomy and renal transplantation, and diabetes (a partial form of lumbosacral diabetic plexopathy); some cases are idiopathic. Patients with femoral neuropathy have difficulty extending their knee and flexing the hip. Sensory symptoms occurring either on the anterior thigh and/or medial leg occur in only half of reported cases. A prominent painful component is the exception rather than the rule, may be delayed, and is often self-limited in nature. The quadriceps (patellar) reflex is diminished."}, {"id": "wiki20220301en085_25580", "title": "Lateral cutaneous nerve of thigh", "score": 0.009998080982536942, "content": "Nerve block The lateral cutaneous nerve of the thigh can be blocked with local anaesthetic. Ultrasound is used to guide needle insertion. This is used for procedures in the supplied area of skin, such as surgical incisions over the outer thigh, and skin grafts. Meralgia paraesthetica Entrapment of the lateral cutaneous nerve of the thigh is caused by compression of the nerve near the anterior superior iliac spine and the inguinal ligament. This causes meralgia paraesthetica (Bernhardt-Roth syndrome). This may be diagnosed with ultrasound, which changes the morphology of the nerve. Changes can include general enlargement, and a hypoechoic appearance. In patients who only have meralgia paraesthetica on one side, ultrasound scans are performed on both thighs to compare the appearance of the nerve. History The lateral cutaneous nerve of the thigh may also be known as the lateral femoral cutaneous nerve. Additional images"}, {"id": "wiki20220301en071_22779", "title": "Meralgia paraesthetica", "score": 0.009900990099009901, "content": "Meralgia paresthetica or meralgia paraesthetica is numbness or pain in the outer thigh not caused by injury to the thigh, but by injury to a nerve that extends from the spinal column to the thigh. This chronic neurological disorder involves a single nerve—the lateral cutaneous nerve of the thigh, which is also called the lateral femoral cutaneous nerve (and hence the syndrome lateral femoral cutaneous neuropathy). The term \"meralgia paraesthetica\" combines four Greek roots to mean \"thigh pain with anomalous perception\". The disorder has also been nicknamed bikini brief syndrome and skinny pants syndrome, because it can be caused by wearing tight clothing."}, {"id": "pubmed23n0534_14732", "title": "Meralgia paresthetica: clinical and electrophysiological diagnosis in 120 cases.", "score": 0.009855265663683107, "content": "We report the results of clinical and electrophysiological examinations in 131 cases of meralgia paresthetica (MP) among 120 unselected patients, 69 men and 51 women, aged 15-81 years. All patients experienced permanent or intermittent pain, and all but one had permanent sensory impairment of the thigh. The lateral aspect of the thigh was solely involved in 88 cases and the anterior aspect was also or exclusively involved in 32 cases. The right thigh was involved 62 times and the left 58 times. Symptom duration varied from 2 weeks to 20 years. The initial diagnosis was meralgia paresthetica in 47 cases (39%), root disease in 35 cases, and osteoarthritis in 6 cases; no diagnosis was proposed in the 32 remaining cases. Two cases had undergone previous spine surgery for disk herniation, with no benefit. A precise cause could explain the lateral femoral cutaneous nerve (LFCN) lesion in 46 cases, the other 74 cases being considered idiopathic (25% of patients were obese). Only one case required surgery to relieve symptoms. LFCN conduction was studied orthodromically, distally from the anterior superior iliac spine. The side-to-side amplitude ratio (ssRatio) was greater than 2.3 in 118 of 120 patients (98.3%) and was a better index to confirm a lesion of the LFCN than SNAP amplitude, which was abnormal (less than 3 microV) in 88 cases (73.3%). Only two of the 11 bilateral cases had an ssRatio lower than 2.3 (they were both 2.0). An ssRatio of 2.3 or more and a SNAP amplitude lower than 3 microV provided a specificity of 98.75% or more. The mean axonal loss was 88%. These clinical and electrophysiological data highlight the central role the neurophysiologist should play in diagnosing MP by means of an LFCN conduction study."}, {"id": "wiki20220301en578_15790", "title": "Furcal nerve", "score": 0.009674957349375953, "content": "It gives branches to the femoral nerve, lumbosacral trunk, and obturator nerve. Clinical significance The furcal nerve is found at various locations and can give problems in the diagnosis of a radiculopathy or sciatic pain. It is the main cause of atypical symptoms of sciatic and radicular pain, that are often due to lumbar disc herniation. A study found evidence of double-rooted abnormality in most of the cases looked at. References Nerves of the lower limb and lower torso"}, {"id": "pubmed23n0568_9290", "title": "Postoperative femoral motor neuropathy: diagnosis and treatment without neurologic consultation or testing.", "score": 0.009615384615384616, "content": "To review the diagnosis and treatment of postoperative femoral motor neuropathy without neurologic consultation or testing. A retrospective review of 6 consecutive patients with postoperative femoral motor neuropathy following gynecologic surgery. Diagnosis was made on clinical evaluation: history of falling during postoperative ambulation, quadriceps weakness, straight leg raise weakness, diminished knee jerk response, and no evidence of psoas hematoma or abscess. Neurologic consultation, electromyography, nerve conduction study and radiologic imaging, such as computed tomography, were not obtained. Instead, a physical therapy consultation was obtained for a knee orthotic and rehabilitation. Four postoperative femoral motor neuropathies developed following 3,014 cases of major gynecologic surgery (0.1%). Two additional cases were seen in consultation. The median age was 57 years. All patients fell while attempting ambulation on postoperative day 1. Recovery occurred at a median of 3 months (1-4). At a median follow-up of 4 years, no patient had developed additional neurologic sequelae. A history of prior postoperative femoral motor neuropathy was noted in 2 of 6 patients (33%). This was the first study of diagnosis and treatment of postoperative femoral motor neuropathy following gynecologic surgery without neurologic consultation or testing. Because of the significant expense of neurologic consultation and testing, patients with postoperative femoral motor neuropathy can have the condition diagnosed by the gynecologist and be referred directly to physical therapy without adversely affecting outcome. This also was the first study to elicit a prior history offemoral neuropathy in 33% of patients. Thus, a prior history may be a risk factor for recurrence."}, {"id": "pubmed23n1074_26559", "title": "Meralgia Paresthetica Caused by Surgery in the Park-Bench Position.", "score": 0.009523809523809525, "content": "Meralgia paresthetica (MP) is an entrapment neuropathy of the lateral femoral cutaneous nerve (LFCN). We report a rare case of MP after microvascular decompression (MVD) surgery in the park-bench position in a patient with hemifacial spasm. The patient was a nondiabetic 46-year-old woman (height: 155 cm, weight: 42 kg) who consumed alcohol infrequently. After a first MVD for right hemifacial spasm, the symptom recurred and she underwent a second MVD procedure in the park-bench position, after which hemifacial spasm resolved. However, she reported right anterolateral thigh pain and dysesthesia without motor weakness. The pain was limited to the LFCN area, and a pelvic compression test elicited a positive Tinel-like sign. Our preliminary diagnosis was MP. Because conservative therapy was ineffective, she underwent LFCN block 9 months after the second MVD procedure. Her pain improved dramatically and we made a definitive diagnosis of MP. There has been no recurrence after 30 months of observation, although she reported persistent mild dysesthesia in the LFCN area. MP is a rare complication after MVD surgery in the park-bench position. LFCN block can resolve symptoms and hasten diagnosis."}]}}}} {"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[0, 221]], "word_ranges": [[0, 36]], "text": "The most frequent side effects of the use of SGLT2 inhibitors are genitourinary tract infections. Since our patient presents with recurrent UTIs, the least indicated drug for the control of her diabetes would be option 4."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The most frequent side effects of the use of SGLT2 inhibitors are genitourinary tract infections. Since our patient presents with recurrent UTIs, the least indicated drug for the control of her diabetes would be option 4.", "full_answer_no_ref": "The most frequent side effects of the use of SGLT2 inhibitors are genitourinary tract infections. Since our patient presents with recurrent UTIs, the least indicated drug for the control of her diabetes would be [HIDDEN].", "full_question": "A 55-year-old woman, type 2 diabetic and obese, on treatment with metformin, with Hb A1c of 8%, has a history of repeated urinary tract infections. Which of the following therapeutic options to associate with metformin do you consider the LEAST appropriate?", "id": 423, "lang": "en", "options": {"1": "DPP4 inhibitors.", "2": "GLP1 analogs.", "3": "Basal insulin.", "4": "SGLT2 inhibitors.", "5": NaN}, "question_id_specific": 89, "type": "ENDOCRINOLOGY", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0761_16217", "title": "Clinical experience with exenatide in obese North Indian patients with type 2 diabetes mellitus.", "score": 0.017193034238488784, "content": "To share our clinical experience with exenatide in obese North Indian subjects with type 2 diabetes. We share our experience with use of exenatide in 74 patients treated at Indraprastha Apollo Hospital, a tertiary care centre in New Delhi, India Subjects included obese / overweight subjects (mean weight and BMI; 97.67 ± 5.6 kg and 34.56 kg/m(2)) with known history of type 2 DM (Mean: 9 ± 5.6 years) and maintaining suboptimal glycemic control (HbA1c >7%) on oral antidiabetic agents, with or without basal insulin. Metformin and sulphonylureas were continued (with dose adjustment if indicated), as was basal insulin (glargine / detemir). TZDs and DPP4 inhibitors were discontinued. The dose of exenatide was increased to 10 mcg twice a day after 4-12 weeks. 56 patients completed minimum 3 month therapy. 42 patients completed 6 months, 32, 9 months and 25 completed 12 months. Data of patients who had completed at-least 3 months of therapy was included for analysis. 69.77, 67.44, and 13.95% of the patients were receiving metformin, secretagogues or thiazolidinediones alone or in combination; 17.76% of the patients were on basal insulin. The change in fasting and post-prandial blood glucose levels were significant at 3, 6, 9 and 12 months with p-value <0.05. The mean weight loss at one, three, and six months and one year was 1.7 ± 1.3, 3.8 ± 2.5, 6.3 ± 3.4, and 8.3 ± 4.3 kg, respectively (P <0.05). The mean HbA1c (baseline: 8.8 ± 1.3%) at 3, 6 months and at one year was 7.8 ± 0.9, 7.7 ± 0.8 and 7.2 ± 0.8 (P <0.05). Thirty-five percent of the patients had a 'good' A1c value (< 7%) at the end of 12 months. 13 patients discontinued exenatide (three due to lack of response, six due to cost of therapy and four due to severe nausea). Nausea was the most common side effect, occurring in 95% patients within 1 month, although the incidence declined with passage of time. Clinical use of Exenatide is associated with significant improvement in glycemic control and major weight loss (8.3±4.3 kg at 1 year) in obese subjects with type 2 diabetes. Nausea is the most common side effect. In conclusion, exenatide is a effective and useful option for treatment of type 2 diabetes in obese Indian subjects."}, {"id": "pubmed23n1165_15121", "title": "Antihyperglycemic Drug Use in Long-Stay Nursing Home Residents with Diabetes Mellitus.", "score": 0.015201976794155567, "content": "About 29.2% of American adults ≥ 65 years of age have diabetes mellitus, but details regarding diabetes management especially among nursing home residents are dated. Evaluate the prevalence of antihyperglycemic agents in residents with diabetes mellitus and describe resident characteristics using major drug classes. cross-sectional study. virtually all United States nursing homes. 141,636 residents with diabetes mellitus. Minimum Data Set (2016) and Medicare Part D claims determined use of metformin, sulfonylureas, meglitinide analogs, alpha-glucosidase inhibitors, TZDs, DPP4 inhibitors, SGLT2 inhibitors, GLP1 agonists, as monotherapy and with basal insulin. Seventy-two percent received antihyperglycemic drugs [most common: basal insulins (53.9% total; 46.9% with other non-insulin agents), metformin (35.5% total; 14.2% monotherapy), sulfonylureas (19.6% total; 6.3% monotherapy), and DPP4 inhibitors (12.2% total; 2.2% monotherapy)]. Sixty-three percent of meglitinide monotherapy versus 34.1% of metformin monotherapy users; and 38.3% meglitinide-basal insulin versus 22.2% metformin-basal insulin users were ≥85 years. Obesity was greater among users of GLP1 agonists compared to those receiving other agents (monotherapy: 60.5% versus 33-42%; with basal insulin: 76.2% versus 50-58%). End-stage renal disease was least prevalent among metformin users (monotherapy: 6.6%; with basal insulin: 8.8%) and most common among meglitinide monotherapy (19.6%) and GLP1 agonists with basal insulin (22%) users. There is heterogeneity of diabetes treatment in nursing homes. Use of antihyperglycemic drugs with a higher risk of hypoglycemia, such as insulin with sulfonylureas or meglitinides, continue in nursing home residents."}, {"id": "pubmed23n1005_21300", "title": "Insulin Therapy in Type 2 Diabetes.", "score": 0.014198274132048966, "content": "Since the discovery of insulin, it was the only drug available for the treatment of diabetes until the development of sulfonylureas and biguanides 50 years later. But even with the availability of oral glucose-lowering drugs, insulin supplementation was often needed to achieve good glucose control in type 2 diabetes. Insulin NPH became the basal insulin therapy of choice and adding NPH to metformin and/or sulfonylureas became the standard of care until basal insulin analogs were developed and new glucose-lowering drugs became available. The advantages in cost-benefit of insulin analogs and their combination with new glucose-lowering drugs are still a matter of debate. There is no general agreement on how to avoid inertia by prescribing insulin therapy in type 2 diabetes when really needed, as reflected by the diversity of recommendations in the current clinical practice guidelines. When necessary for this review, a systematic search of the evidence was done in PubMed and Cochrane databases. Adding new oral glucose-lowering drugs to insulin such as DPP-4 inhibitors lead to a modest HbA1c reduction without weight gain and no increase in hypoglycemia. When SGLT-2 inhibitors are added instead, there is a slightly higher HbA1c reduction, but with body weight and blood pressure reduction. The downside is the increase in genital tract infections. GLP-1 receptor agonists have become the best alternative when basal insulin fails, particularly using fixed ratio combinations. Rapid-acting insulins via the inhaled route may also become an alternative for insulin supplementation and/or intensification. \"Smart insulins\" are under investigation and may become available for clinical use in the near future. Aggressive weight loss strategies together with the new glucose-lowering drugs which do not cause hypoglycemia nor weight gain should limit the number of patients with type 2 diabetes needing insulin. Nevertheless, because of therapeutic inertia and the progressive nature of the disease, many need at least a basal insulin supplementation and insulin analogs are the best choice as they become more affordable. Fixed ratio combinations with GLP1 receptor agonists are a good choice for intensification of insulin therapy."}, {"id": "pubmed23n1061_4543", "title": "[Short-term Glucose Lowering Effects of Sodium-glucose Cotransporter 2 Inhibitors Confirmed by Flash Glucose Monitoring in Two Outpatients with Type 1 Diabetes].", "score": 0.014102564102564103, "content": "Case 1 was a 41-year-old man with type 1 diabetes. He presented with poor glycemic control [hemoglobin A1c (HbA1c) of 8-9%] despite treatment with more than 20 units/day of insulin and 150 mg of miglitol. Before administration of sodium glucose cotransporter 2 (SGLT2) inhibitor, hyperglycemia was noted mainly at night by Flash Glucose Monitoring (FGM). Administration of ipragliflozin at 50 mg improved the hyperglycemia mainly at night (mean blood glucose, before administration: 205 mg/dl, day 6 of treatment: 119 mg/dl). Two months later, the HbA1c improved to 7.2% without hypoglycemia or ketosis. Case 2 was a 46-year-old woman with type 1 diabetes. She was morbidly obese and presented with poor glycemic control (HbA1c: 9-11%) although she was being treated with more than 50 units/day of insulin and 2,250 mg of metformin. Before administration of SGLT2 inhibitor, hyperglycemia was noted to be mainly nocturnal by FGM. Administration of dapagliflozin at 5 mg improved the hyperglycemia mainly at night on day 2 with improvement in the mean blood glucose level from 188 mg/dl before administration to 128 mg/dl on day 5. Four months later, the HbA1c improved to 8.0% without hypoglycemia and ketosis, and her body weight decreased from 92.1 to 89.8 kg. The hypoglycemic effect of SGLT2 inhibitors is independent of insulin. These agents also have various other effects, including weight loss, improvement of blood pressure and lipid metabolism. Here we report the short-term glucose lowering effects of two SGLT2 inhibitors, as confirmed by FGM, in two outpatients with type 1 diabetes."}, {"id": "pubmed23n1032_243", "title": "Approach Toward Diabetes Treatment in the Elderly.", "score": 0.013900062572628945, "content": "Concomitant diseases in elderly individuals with diabetes (renal failure, heart failure, ischemic heart disease, stroke, urinary incontinence, cognitive impairment, dementia, sarcopenia, and osteoporosis) make diabetes management difficult. Therefore, other comorbid conditions should be taken into account in elderly diabetics when considering a treatment approach. The use of oral antidiabetic agents in individuals older than 75 years may be limited. Although the diabetes treatment is not any different in healthy elderly patients, hypoglycemia is one of the most feared conditions, especially in the elderly. Therefore, metformin, DPP-IV inhibitors, and SGLT2 inhibitors should be considered in the first place with less risk of hypoglycemia. Low-dose sulfonylureas may also be used in selected cases. The use of new antidiabetic drugs, such as GLP-1 anologues and SGLT2 inhibitors, has strengthened our ability to cope with the risk of hypoglycemia and cardiovascular events, which are the two most important drawbacks in the treatment of elderly people with diabetes. Insulin treatment should be individualized, and the most rare injection regimens should be used. In case of failure of OAD, basal insulin should be added to the current treatment, and if necessary, a basal + plus regimen should be planned by adding bolus insulin 1/2/3 times per day to the meals. As a result, in elderly diabetics, an inadequate treatment or excessive treatment and individualizing the treatment should be the most appropriate approach."}, {"id": "pubmed23n0989_7923", "title": "Fournier Gangrene Associated With Sodium-Glucose Cotransporter-2 Inhibitors: A Review of Spontaneous Postmarketing Cases.", "score": 0.013743564191994684, "content": "Use of sodium-glucose cotransporter-2 (SGLT2) inhibitors has been associated with Fournier gangrene (FG), a rare urologic emergency characterized by necrotizing infection of the external genitalia, perineum, and perianal region. To describe and compare reported cases of FG in diabetic adults receiving treatment with SGLT2 inhibitors or other antiglycemic agents. Descriptive case series. U.S. Food and Drug Administration (FDA) Adverse Event Reporting System and published case reports. Adults receiving SGLT2 inhibitors or other antiglycemic agents. Clinical and laboratory data. The FDA identified 55 unique cases of FG in patients receiving SGLT2 inhibitors between 1 March 2013 and 31 January 2019. The patients ranged in age from 33 to 87 years; 39 were men, and 16 were women. Time to onset after initiation of SGLT2-inhibitor therapy ranged from 5 days to 49 months. All patients had surgical debridement and were severely ill. Reported complications included diabetic ketoacidosis (n = 8), sepsis or septic shock (n = 9), and acute kidney injury (n = 4). Eight patients had fecal diversion surgery, 2 patients developed necrotizing fasciitis of a lower extremity that required amputation, and 1 patient required a lower-extremity bypass procedure because of gangrenous toes. Three patients died. For comparison, the FDA identified 19 FG cases associated with other antiglycemic agents between 1984 and 31 January 2019: metformin (n = 8), insulin glargine (n = 6), short-acting insulin (n = 2), sitagliptin plus metformin (n = 2), and dulaglutide (n = 1). These patients ranged in age from 42 to 79 years; 12 were men, and 7 were women. Two patients died. Inability to establish causality or incidence, variable quality of reports, possible underreporting, and confounding by indication. FG is a newly identified safety concern in patients receiving SGLT2 inhibitors. Physicians prescribing these agents should be aware of this possible complication and have a high index of suspicion to recognize it in its early stages. None."}, {"id": "pubmed23n1126_3434", "title": "Self-Induced Euglycemic Diabetic Ketoacidosis: When to Stop the Drip.", "score": 0.013466223698781837, "content": "Diabetic ketoacidosis (DKA) is a well-known, serious complication that many patients with type 1 and 2 diabetes face due to either a relative or absolute insulin deficiency. Sodium-glucose cotransporter 2 (SGLT-2) inhibitors have gained increased popularity due to their diabetic, cardiovascular, and renal benefits. An associated complication of SGLT2 inhibitors is euglycemic DKA. A 56-year-old male with a history of type 2 diabetes mellitus and peripheral neuropathy presented with right foot pain secondary to a diabetic foot ulcer. The ulcer was present for one year, but the patient noticed increased pain and purulent discharge over the three days prior to presentation. While being treated inpatient for the foot ulcers, the patient repeatedly refused to receive standard hospital diabetes management of insulin injections. He instead insisted to take his home medications against medical advice, which were metformin and Glyxambi® (empagliflozin/linagliptin, Boehringer Ingelheim, Ingelheim am Rhein, Germany). His diabetic foot ulcer was medically managed with IV antibiotics.  On day 4 of admission, his anion gap increased to 23 mEq/L, and serum bicarbonate (CO2) decreased to 8 mEq/L, raising concerns of diabetic ketoacidosis. His glucose was 141 mg/dL, his beta-hydroxybutyrate was high at 5.5 mmol/L. An arterial blood gas (ABG) test demonstrated anion gap metabolic acidosis with secondary respiratory alkalosis. A urinalysis demonstrated glucose 1000 mg/dL and ketones of 150 mg/dL. The patient was diagnosed with euglycemic DKA. Due to the patient having normal glucose levels, an insulin drip and a 5% dextrose with 0.45% normal saline drip were started. Basic metabolic profiles were ordered every four hours, with glucose checks every hour. Once the anion gap was closed and his urinary ketones disappeared, the patient transitioned to subcutaneous insulin. He was able to be discharged home with basal subcutaneous insulin and metformin with instructions to avoid SGLT2 inhibitors in the future.  Unfortunately, there are currently no guidelines from endocrinology or internal medicine societies regarding the management of euglycemic DKA. As the typical DKA diagnostic criteria of elevated blood glucose level are not present, it is easy to overlook euglycemic DKA. As these SGLT2 inhibitors become more prevalent, careful monitoring of all potential side effects as well as the contraindications are prudent to successful management of complex disease states."}, {"id": "wiki20220301en278_14584", "title": "Canagliflozin", "score": 0.01312316715542522, "content": "Canagliflozin, sold under the brand name Invokana among others, is a medication used to treat type 2 diabetes. It is a third-line medication to be tried after metformin, a first-line medication for type 2 diabetes. It is used together with exercise and diet. It is not recommended in type 1 diabetes. It is taken by mouth. Common side effects include vaginal yeast infections, nausea, constipation, and urinary tract infections. Serious side effects may include low blood sugar, Fournier's gangrene, leg amputation, kidney problems, high blood potassium, and low blood pressure. Diabetic ketoacidosis may occur despite nearly normal blood sugar levels. Use in pregnancy and breastfeeding is not recommended. Canagliflozin is a sodium-glucose cotransporter-2 (SGLT2) inhibitor. It works by increasing the amount of glucose lost in the urine."}, {"id": "wiki20220301en008_126432", "title": "Type 2 diabetes", "score": 0.013075593273613077, "content": ", there is insufficient data to recommend nonnutritive sweeteners, which may help reduce caloric intake. Medications Blood sugar control There are several classes of anti-diabetic medications available. Metformin is generally recommended as a first line treatment as there is some evidence that it decreases mortality; however, this conclusion is questioned. Metformin should not be used in those with severe kidney or liver problems. A second oral agent of another class or insulin may be added if metformin is not sufficient after three months. Other classes of medications include: sulfonylureas, thiazolidinediones, dipeptidyl peptidase-4 inhibitors, SGLT2 inhibitors, and glucagon-like peptide-1 analogs. As of 2015 there was no significant difference between these agents. A 2018 review found that SGLT2 inhibitors and GLP-1 agonists, but not DPP-4 inhibitors, were associated with lower mortality than placebo or no treatment."}, {"id": "wiki20220301en595_5849", "title": "Empagliflozin/linagliptin/metformin", "score": 0.013063775318123635, "content": "Empagliflozin/linagliptin/metformin, sold under the brand name Trijardy XR, is a drug combination used for the treatment of type 2 diabetes. It is a combination of empagliflozin, linagliptin, and metformin. Empagliflozin/linagliptin/metformin was approved for use in the United States in January 2020. Adverse effects To lessen the risk of developing ketoacidosis (a serious condition in which the body produces high levels of blood acids called ketones) after surgery, the FDA has approved changes to the prescribing information for SGLT2 inhibitor diabetes medicines to recommend they be stopped temporarily before scheduled surgery. Empagliflozin should each be stopped at least three days before scheduled surgery. Symptoms of ketoacidosis include nausea, vomiting, abdominal pain, tiredness, and trouble breathing. History The combination preparation was developed and marketed by Boehringer Ingelheim Pharmaceuticals, Inc. and Eli Lilly and Company. References External links"}, {"id": "pubmed23n1035_10113", "title": "Dapagliflozin-associated euglycemic diabetic ketoacidosis in a patient with type 2 diabetes mellitus: A case report.", "score": 0.012738608374384237, "content": "Rare cases of euglycemic diabetic ketoacidosis (eu-DKA) have been reported after the administration of sodium-glucose cotransporter-2 (SGLT-2) inhibitors. No reports have described eu-DKA complicated by hypernatremia due to SGLT-2 inhibitors. A 76-year-old woman with a 40-year history of type 2 diabetes mellitus (DM), for which metformin (1000 mg/day) and dapagliflozin (10 mg/day) were prescribed, presented with malaise, fever, and oliguria. On presentation, her white blood cell count (11,800/μL), serum creatinine (3.2 mg/dL), and C-reactive protein (54 mg/L) were abnormal. Bilateral pyeloureteritis and diffuse paralytic ileus were present. She received intravenous antibiotics and total parenteral nutrition, and was asked to fast. Her renal function and ileus briefly improved. Oral hypoglycemic agents, metformin and dapagliflozin, along with enteral feeding were reinstituted on day 3 of hospitalization. However, on day 6 of hospitalization, the patient developed an altered state of consciousness including confusion, lethargy, and stupor. Several laboratory abnormalities suggestive of ketoacidosis with euglycemia were noted. The patient was diagnosed with eu-DKA accompanied by severe hypernatremia (corrected serum Na concentration, 163 mEq/L) and hypokalemia following dapagliflozin re-administration. The patient was treated with indicated intravenous fluid therapy. Dapagliflozin use was discontinued. The patient's mental status and laboratory findings improved gradually, and she was discharged on maintenance doses of insulin and metformin on day 14 of hospitalization. Acute illnesses such as diffuse paralytic ileus and urinary tract infection, and dietary restrictions or fasting in patients with DM can be considered potential predisposing factors for SGLT-2 inhibitor-associated eu-DKA. For patients with diabetes in the setting of acute morbidity, timely resumption of the SGLT-2 inhibitor therapy should be carefully determined. In addition, eu-DKA due to SGLT-2 inhibitor use may be accompanied by electrolyte disturbances such as hypernatremia and hypokalemia."}, {"id": "pubmed23n1149_18690", "title": "SGLT2-inhibitors are effective and safe in the elderly: The SOLD study.", "score": 0.012704933622427632, "content": "Sodium-glucose co-transporter-2 inhibitors (SGLT2i) may have important benefits for the elderly with type 2 diabetes (T2D), however some safety concerns still limit their use in patients over 70 years of age. The SOLD study (SGLT2i in Older Diabetic patients) is a multicenter study, aimed to evaluate the effectiveness and safety of SGLT2i in the older diabetic patients in a real-life setting. We analyzed a population of 739 adults (mean age 75.4 ± 3.9 years, M/F 420/319) with T2D, which started a SGLT2i-based treatment after the age of 70, with at least one year of follow-up. Data were collected at baseline, at 6 and 12 months of follow-up. SGLT2i (37.5% Empagliflozin, 35.7% Dapagliflozin, 26.1% Canagliflozin, 0.7% Ertugliflozin) were an add-on therapy to Metformin in 88.6%, to basal insulin in 36.1% and to other antidiabetic drugs in 29.6% of cases. 565 subjects completed the follow up, while 174 (23.5%) discontinued treatment due to adverse events which were SGLT2i related. A statistically significant reduction of glycated hemoglobin (baseline vs 12 months: 7.8 ± 1.1 vs 7.1 ± 0.8%, p < 0.001) and body mass index values (baseline vs 12 months: 29.2 ± 4.7 vs 28.1 ± 4.5 kg/mStaphylococcus aureus (MRSA) in a young healthy man.", "score": 0.008620689655172414, "content": "An otherwise healthy 24-year-old man presented with 1 week of fever, facial pain and swelling. He initially sought care at an outside hospital, where he was diagnosed with folliculitis and sent home with oral antibiotics. On arrival at our institution, CT neck was ordered, which demonstrated diffuse submental phlegmon, prompting incision and drainage. After initial improvement, the patient experienced high fevers and increased swelling just 12 hours later. The decision was made to take the patient for operative exploration, and wide debridement was performed due to suspicion for necrotising fasciitis intraoperatively that was ultimately confirmed on final pathology. Final speciation of intraoperative culture demonstrated a clindamycin-resistant and methicillin-resistant strain of 75% are associated with an increased likelihood of PCP compared to tuberculosis or bacterial pneumonia in patients with HIV. [11]"}, {"id": "InternalMed_Harrison_11627", "title": "InternalMed_Harrison", "score": 0.01140081799591002, "content": "presenting manifestations The presentation of pneumococcal pneumonia does not reliably distinguish it from pneumonia of other etiologies. In a subset of cases, pneumococcal pneumonia is recognized at the outset as associated with a viral upper respiratory infection and is characterized by the abrupt onset of cough and dyspnea accompanied by fever, shaking chills, and myalgias. The cough evolves from nonpurulent to productive of sputum that is purulent and sometimes tinged with blood. Patients may describe stabbing pleuritic chest pain and significant dyspnea indicating involvement of the parietal pleura. Among the elderly, the presenting clinical symptoms may be less specific, with confusion or malaise but without fever or cough. In such cases, a high index of suspicion is required because failure to treat pneumococcal pneumonia promptly in an elderly patient is likely to result in rapid evolution of the infection, with increased severity, morbidity, and risk of death."}, {"id": "wiki20220301en070_12377", "title": "Cryptogenic organizing pneumonia", "score": 0.009909099990010987, "content": "Diagnosis On clinical examination, crackles are common, and more rarely, patients may have clubbing (<5% of cases). Laboratory findings are nonspecific. Almost 75% of people have symptoms for less than two months before seeking medical attention. A flu-like illness, with a cough, fever, a feeling of illness (malaise), fatigue, and weight loss heralds the onset in about 40% of patients. Doctors do not find any specific abnormalities on routine laboratory tests or on a physical examination, except for the frequent presence of crackling sounds (called rales) upon auscultation with a stethoscope by the care provider. Pulmonary function tests usually show that the amount of air the lungs can hold is below normal. The amount of oxygen in the blood is often low at rest and is even lower with exercise. Imaging"}, {"id": "pubmed23n0544_9156", "title": "[Syphilitic aortic aneurysm. A case report].", "score": 0.009900990099009901, "content": "The incidence of tertiary syphilis has declined in recent years owing to the early recognition of the disease and use of antibiotics. As a result, syphilitic aortic aneurysms are rarely encountered nowadays. We report the case of a 65 years old man, who was admitted to our hospital in June 2004 for dyspnea, cough and chest discomfort. On physical examination, blood pressure was 130/80 mmHg with no significant laterality, pulse rate was 70 per minute and there was a decrease of breath sounds over the right lung. Laboratory findings revealed a slight elevation of the erythrocyte sedimentation rate. Serological studies for syphilis showed a positive venereal disease laboratory test (VDRL) at 1/32 and a positive Treponema pallidum hemagglutination test (TPHA) at 1/2560. The chest radiography showed a right para cardiac opacity measuring 16 x 12 cm. Fiber optic bronchoscopy showed an extrinsic compression of the right upper lobar bronchus. Gadolinium-enhanced magnetic resonance angiography and 16 multidetector-row spiral computed aortography showed a huge partially thrombosed saccular aneurysm of the ascending aorta measuring 132 mm in diameter. The circulating lumen measured 53 mm in its largest diameter. This aneurysm involved the innominate artery. There was no other arterial involvement. The patient was given a three week course of intravenous penicillin followed by a successful surgical procedure in September 2004 with ascending aortic replacement and innominate artery reimplantation. This case illustrates well a formerly common, but now extremely rare disease."}, {"id": "pubmed23n0799_21446", "title": "Acute respiratory distress in a silversmith.", "score": 0.009708737864077669, "content": "A 25-year-old young male patient presented in casualty department with severe respiratory distress on the fourth day from onset of symptoms. The patient was nonsmoker and had no antecedent medical or drug history. Prior to admission, patient had dry cough and bilateral pleuritic chest pain for the last three days. He was in severe respiratory distress with use of accessory muscles of respiration. On examination, he had heart rate of 120 beats/min, blood pressure (BP) of 150/80, respiratory rate of 48-52/min and central cyanosis present. On systemic examination, reduced intensity of breath sounds with extensive rhonchi and crepitation was found in both lung fields, with other examination being within normal limits. On pulse oximetry, oxygen saturation was 28% on room air, which increased up to 36% with the help of 4 L oxygen via nasal prongs. PaO2/FiO2 ratio was 100. Chest X-ray analysis was suggestive of non-cardiac pulmonary edema in view of bilateral fluffy opacity without cardiomegaly. In view of 2/3 positive criteria, his provisional diagnosis was Acute Respiratory Distress Syndrome (ARDS). He required mechanical ventilatory support and was gradually weaned over a period of 10 days. The patient was treated with broad spectrum antibiotics and other supportive measures. On re-evaluation of history, we found that he was a goldsmith by occupation, smelting silver and gold for the past 8-10 years. On the day of onset of symptoms, while smelting silver he was exposed to golden yellow fumes for around 15 minutes, with the quantum of exposure more than any other day earlier. From previous experience and analysis of similar silver metals, he was able to tell us that the silver was adulterated with large amount of cadmium on that day than before. Serum level of cadmium was 2.9 μg/L 6 days after initial exposure. At the time of discharge, he had residual opacities in the chest radiograph and resting oxygen saturation was 94% on room air. "}, {"id": "pubmed23n0069_9667", "title": "[A case of sarcoidosis presenting with high fever and acute respiratory failure].", "score": 0.009708737864077669, "content": "A 55-year-old man was admitted with complaints of remittent fever (39 degrees C) and dyspnea on exertion which began ten days previously. His family and past histories were non-contributory for diagnosis except his occupation as a stone mason for 26 years. The chest X-ray film taken on admission showed diffuse small nodular shadows associated with small amounts of pleural effusion and bilateral hilar adenopathy. Arterihl blood gas analysis showed severe hypoxemia and hypocapnea (Pao2 32.2 Torr, Paco2 31.6 Torr). The serum level of LDH was 985 IU/L and ACE was 49.0 IU/L, lysozyme was 28.8 micrograms/ml. Biopsied materials of the lung obtained by TBLB, liver and bone marrow showed non-caseating epithelioid granuloma without caseating necrosis. T-lymphocyte ratio increased in BALF. The patient was diagnosed to have sarcoidosis. The administration of prednisolone was initiated, which resulted in a marked improvement of clinical data including chest X-ray films, BGA, LDH, ACE and lysozyme."}, {"id": "pubmed23n0560_6547", "title": "[Spontaneous pneumothorax in pregnancy. Case report].", "score": 0.009523809523809525, "content": "Spontaneous pneumothorax is a rare condition during pregnancy. The most common cause is the rupture of a subpleural apical bulla or bleb, due to the increased respiratory demand of the peripartum period. The main risk for the mother is respiratory compromise; fetal risks include reduction in oxygen supply and preterm labor. The risk of recurrence is 30-40%, particularly during labor. Treatment is based on the magnitude of pneumothorax. Up to 75% of patients are treated with chest tube drainage. We present the case report of a previously healthy patient. Our patient was a 22-year-old female at 24.2 weeks of her second pregnancy. The patient was a non-smoker, had no history of any drug addictions, and no history of previous pulmonary disease. The patient presented with sudden onset of pleuritic right-sided pleuritic chest pain associated with dyspnea. Chest examination was notable for decreased breath sounds and hyperresonance over the right hemithorax. Chest radiography showed right spontaneous pneumothorax with total lung collapse. Diagnosis of pneumothorax should be considered in any pregnant woman with chest pain and dyspnea. The presented case was successfully treated with closed intercostal chest tube thoracostomy for 7 weeks."}, {"id": "pubmed23n1024_15211", "title": "Pneumocystis Jirovecii Pneumonia in Newly Diagnosed HIV Infection: A Challenging Case Report.", "score": 0.009433962264150943, "content": "Pneumocystis jirovecii is a potentially life-threatening opportunistic pathogen particularly affecting the lungs, mainly in immunosuppressed individuals and HIV-infected patients with a low CD4 cell count. A 50-year-old man presented with a 1-week history of pleuritic chest pain and fever. He was also hypoxic with oxygen saturation of 86% on room air. Detailed clinical history revealed that he had fatigue, dyspnea, night sweats, generalized bone pain and a loss of about 10 kg in weight over the past six months without intention. Chest imaging showed diffuse bilateral infiltrates. Diagnostic bronchoscopy was performed. Transbronchial biopsy and bronchoalveolar lavage were received simultaneously. The presence of P. jirovecii was suspected in hematoxylin-eosin-stained slides, and Gomori's methenamine silver stain was used to confirm the diagnosis. A blood test revealed dyslipidemia, hypothyroidism, increased plasma levels of the gonadotropins and positive HIV antibodies with a CD4+ cell count of 48/μL. CMV co-infection was found with CMV viral load of 6738 copies/ml in plasma. Herein, we present a case with Pneumocystis jirovecii pneumonia (PCP) that led to a new diagnosis of Human immonudeficiency virus. As in our case, diagnosis of disease through the pathological examination of tissues (biopsy samples) or bodily fluids could lead to the recognition of an unrevealed HIV-infection."}, {"id": "InternalMed_Harrison_13317", "title": "InternalMed_Harrison", "score": 0.009433962264150943, "content": "parenchymal lesions or pleural disease. Extensive disease may produce dyspnea and, in rare instances, adult respiratory distress syndrome. Physical findings are of limited use in pulmonary TB. Many patients have no abnormalities detectable by chest examination, whereas others have detectable rales in the involved areas during inspiration, especially after coughing. Occasionally, rhonchi due to partial bronchial obstruction and classic amphoric breath sounds in areas with large cavities may be heard. Systemic features include fever (often low-grade and intermittent) in up to 80% of cases and wasting. Absence of fever, however, does not exclude TB. In some cases, pallor and finger clubbing develop. The most common hematologic findings are mild anemia, leukocytosis, and thrombocytosis with a slightly elevated erythrocyte sedimentation rate and/or C-reactive protein level. None of these findings is consistent or sufficiently accurate for diagnostic purposes."}, {"id": "pubmed23n0941_15930", "title": "Report of a lung carcinoma extended to the left atrium through pulmonary vein.", "score": 0.009345794392523364, "content": "Lung cancers may extend along or grow through the pulmonary veins to invade or lie within the left atrium (LA). A 62-year-old man, previously healthy, presented with 1-month ventilatory-independent right hemithorax back pain, dry cough and large effort dyspnea. He also referred weight loss of 12 kg in 10 months and denied hemoptysis. As antecedents, he smoked for 40 years and moderate daily alcoholism. On physical examination, the patient was in good general condition, hydrated and regular respiration at rest [blood pressure (BP) =120/80 mmHg; heart rate (HR) =90 bpm; respiratory rate (RR) =16 rpm]. Cardiac auscultation revealed two standard rhythmic sounds without murmurs. Pulmonary auscultation revealed a slightly diminished vesicular murmur in the lower 1/3 of the right hemithorax without adventitious noises. Chest radiography showed a mass over the right lower lung. A CT scan confirmed the radiography image with the mass extending along the right inferior pulmonary vein and a tumor in the LA. Transthoracic and transesophageal echocardiography revealed large mass within the LA (occupying almost the entire cavity), measuring about 10 cm × 3 cm at its largest diameter, prolapsing into the left ventricle. Bronchoscopy, head CT scan, and whole-body bone scintigraphy investigation did not show any distant metastasis. The patient was successfully operated removing the intracardiac and inferior pulmonary vein tumor with the aid of cardiopulmonary bypass, followed by a right inferior lobectomy carried out after 25 days. After 30 days from surgery presented seizures associated a brain metastasis evidenced by CT when adjuvant radio and chemotherapy was started. During the next 90 days, the clinical conditions worsened, and the patient died 4 months after the surgical treatment. The case report has two primary justifications, even considering the poor outcome: (I) rarity and (II) the possibility of the surgical treatment."}, {"id": "pubmed23n0753_3228", "title": "Case 1/2013: 69-year-old male patient with sudden back and lower right limb pain and shock.", "score": 0.009345794392523364, "content": "The patient, MSM, a 69-year-old man, sought medical care due to left dorsal and right lower limb pain. The chest x-ray showed mediastinal enlargement. He was undergoing examination when he lost consciousness and went into shock. Subcutaneous emphysema was observed in the left hemithorax, as well as abolition of breath sounds at auscultation. Tracheal intubation was performed with draining of blood-tinged fluid from the left hemithorax. Echocardiography showed left ventricle with 44/29 mm; septum, 12 mm; posterior wall, 13 mm; mild aortic root dilation, dissection of the lamina and periaortic hematoma. The valves and pericardium were normal. The patient was transferred to Instituto do Coraçao - InCor. Physical examination (21 Oct 2004: 10:45) showed that the patient was sedated with tracheal intubation, pale, heart rate at 90 bpm, blood pressure 130 x 80 mmHg, bloody drainage in the chest tube. Electrocardiogram - frequency 90 bpm, sinus rhythm, low voltage in the frontal plane and decreased voltage in left leads (Fig. 1). Computed tomography showed bilateral subcutaneous emphysema, thoracic aorta with inaccurate borders in its descending portion (from the subclavian artery to the middle portion), collapsed left lung and extensive collection of hematic characteristics in same hemithorax and middle and posterior mediastinum. Small right pneumothorax; small right pleural effusion with underlying parenchymal alterations. The analysis of the heart was impaired by the presence of hemothorax. While undergoing computed tomography, the patient showed no pulse, mydriasis, with asystole unresponsive to resuscitation and died (21 Oct 2011; 15:00 h)."}, {"id": "pubmed23n1126_7223", "title": "A Rare Case of Plasma Cell Leukemia Presenting as Dyspnea.", "score": 0.009259259259259259, "content": "A 74-year-old man presented to the ED with acute chronic exertional dyspnea of 5-day duration. As part of his previous evaluation, 5 months earlier, he had undergone cardiopulmonary stress testing, routine laboratory evaluation, and chest radiography that were unremarkable. Over the subsequent months, he had waxing and waning exercise capacity until his incident hospitalization; the exercise was limited to < 40 meters. He reported associated nonproductive cough, 15-pound unintentional weight loss over the past 5 months, night sweats, easy fatigability, and early satiety. A chest radiograph was performed that showed a left hilar, mass-like consolidation with loss of the left heart border that was associated left-sided pleural effusion and left lower lung zone consolidation. On physical examination, he was afebrile and normotensive with a sinus tachycardia of 125 beats per minute. He was noted to be tachypneic with a respiratory rate of 24 breaths per minute and saturation of 95% on room air. Examination of the chest showed diminished breath sounds over left lower lung fields with scattered end expiratory wheezing."}, {"id": "wiki20220301en013_148122", "title": "Acute bronchitis", "score": 0.009259259259259259, "content": "A variety of tests may be performed in people presenting with cough and shortness of breath: A chest X-ray is useful to exclude pneumonia which is more common in those with a fever, fast heart rate, fast respiratory rate, or who are old. A sputum sample showing neutrophil granulocytes (inflammatory white blood cells) and culture showing that has pathogenic microorganisms such as Streptococcus species. A blood test would indicate inflammation (as indicated by a raised white blood cell count and elevated C-reactive protein). Decreased breath sounds, crackles, wheezing, and rhonchi that clears with coughs may be heard in the chest. Dullness to percussion and pleural rub suggest disease extension beyond the bronchi such as seen with pneumonia. Paroxysms of cough followed by inspiratory whoop and vomiting suggests pertussis."}, {"id": "pubmed23n1093_2274", "title": "Spontaneous Hemoptysis in a Patient With COVID-19.", "score": 0.009174311926605505, "content": "A 65-year-old man presented with shortness of breath, gradually worsening for the previous 2 weeks, associated with dry cough, sore throat, and diarrhea. He denied fever, chills, chest pain, abdominal pain, nausea, or vomiting. He did not have any sick contacts or travel history outside of Michigan. His medical history included hypertension, diabetes mellitus, chronic kidney disease, morbid obesity, paroxysmal atrial fibrillation, and tobacco use. He was taking amiodarone, carvedilol, furosemide, pregabalin, and insulin. The patient appeared to be in mild respiratory distress. He was afebrile and had saturation at 93% on 3 L of oxygen, heart rate of 105 beats/min, BP of 145/99 mm Hg, and respiratory rate of 18 breaths/min. On auscultation, there were crackles on bilateral lung bases and chronic bilateral leg swelling with hyperpigmented changes. His WBC count was 6.0 K/cumm (3.5 to 10.6 K/cumm) with absolute lymphocyte count 0.7 K/cumm (1.0 to 3.8 K/cumm); serum creatinine was 2.81 mg/dL (0.7 to 1.3 mg/dL). He had elevated inflammatory markers (serum ferritin, C-reactive protein, lactate dehydrogenase, D-dimer, and creatinine phosphokinase). Chest radiography showed bilateral pulmonary opacities that were suggestive of multifocal pneumonia (Fig 1). Nasopharyngeal swab for SARS-CoV-2 was positive. Therapy was started with ceftriaxone, doxycycline, hydroxychloroquine, and methylprednisolone 1 mg/kg IV for 3 days. By day 3 of hospitalization, he required endotracheal intubation, vasopressor support, and continuous renal replacement. Blood cultures were negative; respiratory cultures revealed only normal oral flora, so antibiotic therapy was discontinued. On day 10, WBC count increased to 28 K/cumm, and chest radiography showed persistent bilateral opacities with left lower lobe consolidation. Repeat respiratory cultures grew Pseudomonas aeruginosa (Table 1). Antibiotic therapy with IV meropenem was started. His condition steadily improved; eventually by day 20, he was off vasopressors and was extubated. However, on day 23, he experienced significant hemoptysis that required reintubation and vasopressor support."}, {"id": "pubmed23n1083_22289", "title": "Persistent Dyspnea in a 74-Year-Old Man With Normal Spirometry and Lung Volumes.", "score": 0.009174311926605505, "content": "A 74-year-old man was referred to a pulmonologist for evaluation of a 1-year history of nonproductive cough and progressive exertional dyspnea. He was initially evaluated by his primary care physician, where he had spirometry that was negative for any obstructive or restrictive lung disease. An echocardiogram showed a normal left ventricular ejection fraction, with no wall motion abnormality or valvular heart disease. He had an outpatient chest radiograph performed (Fig 1), and he was subsequently treated empirically for a COPD exacerbation with 5 days of oral prednisone and azithromycin. He was eventually referred to a pulmonologist because of a lack of clinical improvement. On seeing his pulmonary physician, he described the same exertional dyspnea and a nonproductive cough. A review of systems was negative for fever, chills, wheezing, angina, arthralgia, myalgia, rash, or leg swelling. He denied any medical illness and was not taking any medications. He was currently retired and had worked as a cashier his entire adult life. He had no occupational exposure to asbestos, coal dust, beryllium, silica dust, or dust from hard metal objects, such as cobalt. However, he had smoked approximately 1 to 2 packs of cigarettes per day and had done so for the past 50 years. His vital signs were unremarkable, aside from an oxygen saturation of 94% on room air. His physical examination revealed bibasilar \"velcro-like\" inspiratory crackles on lung examination. There was no digital clubbing, nor was there peripheral edema in his lower extremities. He had no muscle tenderness and demonstrated normal muscle strength against resistance."}, {"id": "pubmed23n0978_5162", "title": "Clinical Management of Pneumonitis in Patients Receiving Anti-PD-1/PD-L1 Therapy.", "score": 0.00909090909090909, "content": "30 mmol/L (>600 mg/dL) Serum osmolality >320 mOsm/kg Profound dehydration, up to an average of 9L (and therefore substantial thirst (polydipsia)) Serum pH >7.30 Bicarbonate >15 mEq/L Small ketonuria (~+ on dipstick) and absent-to-low ketonemia (<3 mmol/L) Some alteration in consciousness BUN > 30 mg/dL (increased) Creatinine > 1.5 mg/dL (increased) Imaging Cranial imaging is not used for diagnosis of this condition. However, if MRI is performed, it may show cortical restricted diffusion with unusual characteristics of reversible T2 hypointensity in the subcortical white matter."}, {"id": "wiki20220301en086_253", "title": "Hyperosmolar hyperglycemic state", "score": 0.014218651259366253, "content": "Signs and symptoms Symptoms of high blood sugar including increased thirst (polydipsia), increased volume of urination (polyuria), and increased hunger (polyphagia). Symptoms of HHS include: Altered level of consciousness Neurologic signs including: blurred vision, headaches, focal seizures, myoclonic jerking, reversible paralysis Motor abnormalities including flaccidity, depressed reflexes, tremors or fasciculations Hyperviscosity and increased risk of blood clot formation Dehydration Weight loss Nausea, vomiting, and abdominal pain Weakness Low blood pressure with standing Cause The main risk factor is a history of diabetes mellitus type 2. Occasionally it may occur in those without a prior history of diabetes or those with diabetes mellitus type 1. Triggers include infections, stroke, trauma, certain medications, and heart attacks."}, {"id": "wiki20220301en086_256", "title": "Hyperosmolar hyperglycemic state", "score": 0.014077425842131725, "content": "Differential diagnosis The major differential diagnosis is diabetic ketoacidosis (DKA). In contrast to DKA, serum glucose levels in HHS are extremely high, usually greater than 40-50 mmol/L (600 mg/dL). Metabolic acidosis is absent or mild. A temporary state of confusion (delirium) is also more common in HHS than DKA. HHS also tends to affect older people more. DKA may have fruity breath, and rapid and deep breathing. DKA often has serum glucose level greater than 300 mg/dL (HHS is >600 mg/dL). DKA usually occurs in type 1 diabetics whereas HHS is more common in type 2 diabetics. DKA is characterized by a rapid onset, and HHS occurs gradually over a few days. DKA also is characterized by ketosis due to the breakdown of fat for energy. Both DKA and HHS may show symptoms of dehydration, increased thirst, increased urination, increased hunger, weight loss, nausea, vomiting, abdominal pain, blurred vision, headaches, weakness, and low blood pressure with standing. Management"}, {"id": "wiki20220301en008_125514", "title": "Diabetic coma", "score": 0.014040163376446563, "content": "Nonketotic hyperosmolar coma Nonketotic hyperosmolar coma usually develops more insidiously than diabetic ketoacidosis because the principal symptom is lethargy progressing to obtundation, rather than vomiting and an obvious illness. Extremely high blood sugar levels are accompanied by dehydration due to inadequate fluid intake. Coma occurs most often in patients who have type 2 or steroid diabetes and have an impaired ability to recognize thirst and drink. It is classically a nursing home condition but can occur in all ages. The diagnosis is usually discovered when a chemistry screen performed because of obtundation reveals an extremely high blood sugar level (often above 1800 mg/dl (100 mM)) and dehydration. The treatment consists of insulin and gradual rehydration with intravenous fluids."}, {"id": "pubmed23n0030_10003", "title": "[Two cases of nonketotic hyperosmolar coma in neurosurgery (author's transl)].", "score": 0.014010674799847503, "content": "Hyperosmolar nonketotic coma is characterized by hyperglycemia, hyperosmolarity and dehydration in the absence of ketoacidosis. Two cases of hyperosmolar nonketotic coma, in which both the patients recovered, were presented. One of the cases was a 59-year-old female who had suffered from a metastatic brain tumor. After removal of the tumor, the patient's condition improved for a period. This was followed by a period of frequent vomiting, subsequently followed by coma. The laboratory data showed the absence of ketoacidosis in the blood sugar measured at 672 mg/dl and serum osmolarity at 343.1 mOsm./kg. The other case was a 74-year-old female who was admitted to the clinic because of cerebral thrombosis. Her caloric in-take was restricted and insulin was administered because of a mild diabetes mellitus which occured after admission. Then she entered a hyperosmolar non-ketotic coma. The laboratory data revealed blood sugar to be 1068 mg/dl and serum osmolarity to be 418 mOsm./kg. Immediately after large amounts of intravenous drip infusion and insulin were administerd, she recovered from the syndrome. The clinical observations and the pathogenesis of this syndrome were discussed."}, {"id": "pubmed23n0707_22077", "title": "Severe hyperosmolarity and hypernatremia in an adipsic young woman.", "score": 0.013910428121622256, "content": "Combined deficits in arginine vasopressin secretion (AVP) and thirst sensation can result in life threatening hyperosmolality and hypernatremia. Complications include seizures, profound volume contraction and renal failure. Fortunately, this is an uncommon clinical condition, with approximately 70 cases reported in the literature over the past 47 years [1]. Defects in AVP secretion and/or synthesis produce central diabetes insipidus (DI), polyuria with polydipsia, hypernatremia and hyperosmolality. Most awake and alert patients with an intact thirst stimulus will \"drink\" themselves back to a normal serum sodium and osmolality. However, if there is concomitant destruction of the osmoreceptors that regulate thirst, osmolal and volume homeostasis cannot be maintained. The relationships between urine osmolarity and serum osmolarity and plasma vasopressin levels are vital for distinguishing a reset osmostat from central DI. After obtaining approval from our institutional review board, we retrospectively reviewed the medical record of a 37-year-old patient who presented to our institution with a serum sodium of 176 mEq/l. Admission laboratory examination revealed: hemoglobin 12.8 g/dl; white blood cell count 4.7 × 103/µl, with a normal differential; random serum glucose 91 mg/dl ; sodium 176 mEq/l; plasma osmolality 366 mOsm/kg; BUN 33 mg/dl; serum creatinine 1 mg/dl; calcium 9.5 mg/dl; urine specific gravity 1.032; and urine osmolality 1,172 mOsm/kg. An MRI with contrast of the sella/ pituitary revealed an enhancing mass centered within the suprasellar cistern and anterior third ventricle, measuring 3.0 × 3.9 × 3.4 cm. The lesion appeared to involve the hypothalamus and displaced the optic chiasm inferiorly. Evaluation of pituitary function revealed normal serum levels of thyroid stimulating hormone, AM cortisol, luteinizing hormone, follicle stimulating hormone and prolactin. Figure 1 illustrates the relationship between measured serum AVP levels and serum osmolality. Figure 2 shows the relationship between measured urine and serum osmolality. If the serum AVP levels were not available, it would appear as though the patient had a reset osmostat. The kidneys appear to appropriately generate maximally concentrated urine at a serum osmolality above 348 but are unable to below this value. When compared with the normal curve, our patient's AVP levels were lower than expected for the corresponding osmolality. This pattern is consistent with a partial central DI. She does not have a reset osmostat. In the presence of significant volume contraction and a reduced GFR, her kidneys produced more concentrated urine despite markedly decreased central vasopressin production. As the volume contraction abated and the GFR improved, polyuria recurred, despite persistent hyperosmolarity and hypernatremia."}, {"id": "pubmed23n0583_2153", "title": "Metformin-induced lactic acidosis: a case series.", "score": 0.013131313131313133, "content": "Unlike other agents used in the treatment of type 2 diabetes mellitus, metformin has been shown to reduce mortality in obese patients. It is therefore being increasingly used in higher doses. The major concern of many physicians is a possible risk of lactic acidosis. The reported frequency of metformin related lactic acidosis is 0.05 per 1000 patient-years; some authors advocate that this rate is equal in those patients not taking metformin. We present two case reports of metformin-associated lactic acidosis. The first case is a 77 year old female with a past medical history of hypertension and type 2 diabetes mellitus who had recently been prescribed metformin (3 g/day), perindopril and acetylsalicylic acid. She was admitted to the emergency department two weeks later with abdominal pain and psychomotor agitation. Physical examination revealed only signs of poor perfusion. Laboratory evaluation revealed hyperkalemia, elevated creatinine and blood urea nitrogen and mild leukocytosis. Arterial blood gases showed severe lactic acidemia. She was admitted to the intensive care unit. Vasopressor and ventilatory support was initiated and continuous venovenous hemodiafiltration was instituted. Twenty-four hours later, full clinical recovery was observed, with return to a normal serum lactate level. The patient was discharged from the intensive care unit on the sixth day. The second patient is a 69 year old male with a past medical history of hypertension, type 2 diabetes mellitus and ischemic heart disease who was on metformin (4 g/day), glycazide, acetylsalicylic acid and isosorbide dinitrate. He was admitted to the emergency department in shock with extreme bradycardia. Initial evaluation revealed severe lactic acidosis and elevated creatinine and urea. The patient was admitted to the Intensive Care Unit and commenced on continuous venovenous hemodiafiltration in addition to other supportive measures. A progressive recovery was observed and he was discharged from the intensive care unit on the seventh day. We present two case reports of severe lactic acidosis most probably associated with high doses of metformin in patients with no known contraindications for metformin prescription. In both patients no other condition was identified to cause such severe lactic acidosis. Although controversial, lactic acidosis should be considered in patients taking metformin."}, {"id": "pubmed23n0294_1926", "title": "[Rhabdomyolysis related-acute renal failure in a patient with hyperosmolar nonketotic diabetic coma (HNKC): demonstration of myoglobin casts after normalization of renal function].", "score": 0.013036093418259023, "content": "We report a patient with rhabdomyolysis secondary to hyperosmolar nonketotic diabetic coma (HNKC), who progressed to acute renal failure. A 43-year-old male with diabetes mellitus for three years was admitted to our hospital because of loss of consciousness. The laboratory findings at admission were as follows: serum glucose 1792 mg/dl, serum Na 129 mEq/1, BUN 71 mg/d1, serum creatinine 3.3 mg/d1, CPK 715 IU/1, plasma osmolality 370 mOsm/1, and negative urine ketone bodies. A diagnosis of HNKC was made. On the 2nd day, he had oliguria and the serum creatinine increased despite adequate treatment of HNKC by the administration of intravenous fluid and insulin. On the 4th day, CPK reached 47,300 IU/1, and serum myoglobin was also increased, indicating rhabdomyolysis. His renal function improved gradually and was almost normalized on the 20th day. Renal biopsy on the 23rd day showed myoglobin at the distal renal tubules, which appeared to be involved in the pathogenesis of renal failure by rhabdomyolysis. However, we found little abnormality association with diabetic nephropathy in the renal tissue. Since HNKC is known to induce acute renal failure rarely without diabetic nephropathy, these findings suggested that the acute renal failure was caused mainly by the rhabdomyolysis. Acute renal failure induced by rhabdomyolysis in patients with HNKC is rare, but fatal. The present study showed that the measurement of serum CPK and urine myoglobin was helpful for early diagnosis. Only 12 cases have been reported to have developed renal failure due to rhabdomyolysis among patients with HNKC. To our knowledge, we demonstrated for the first time that myoglobin at the distal renal tubules after renal function was normalized."}, {"id": "wiki20220301en292_9714", "title": "Complications of diabetes", "score": 0.01250683433570257, "content": "Nonketotic hyperosmolar coma (HNS) is an acute complication sharing many symptoms with DKA, but an entirely different origin and different treatment. A person with very high (usually considered to be above 300 mg/dl (16 mmol/L)) blood glucose levels, water is osmotically drawn out of cells into the blood and the kidneys eventually begin to dump glucose into the urine. This results in loss of water and an increase in blood osmolarity. If fluid is not replaced (by mouth or intravenously), the osmotic effect of high glucose levels, combined with the loss of water, will eventually lead to dehydration. The body's cells become progressively dehydrated as water is taken from them and excreted. Electrolyte imbalances are also common and are always dangerous. As with DKA, urgent medical treatment is necessary, commonly beginning with fluid volume replacement. Lethargy may ultimately progress to a coma, though this is more common in type 2 diabetes than type 1. Hypoglycemia"}, {"id": "pubmed23n1087_25042", "title": "COVID-19 and Combined Diabetic Ketoacidosis and Hyperglycemic Hyperosmolar Nonketotic Coma: Report of 11 Cases.", "score": 0.012453806356245382, "content": "We report 11 cases of combined diabetic ketoacidosis (DKA) and hyperglycemic hyperosmolar nonketotic coma (HHNK) in coronavirus 2019 patients who presented to our institution in New Jersey, USA. The median age was 47 years (range 12-88 years). Out of the 11 patients, 7 were male and 4 were female. Out of 11 patients, 8 had type 2 diabetes mellitus (DM), 2 had undiagnosed DM, and 1 had type 1 DM. Presenting complaints included altered mental status, weakness, shortness of breath, cough, fever, vomiting, abdominal pain, chest pain, and foot pain. Out of 11 patients, pneumonia was diagnosed at presentation in 8 patients, while in 3 patients, chest X-ray was clear. Median value of initial glucose on presentation was 974 mg/dL (range 549-1556 mg/dL), and hemoglobin A1c on presentation was 13.8%. The median value of anion gap was 34 mEq/L. Out of the 11 patients, ketonemia was moderate in 6 patients, large in 3, and small in 2 patients. Acute kidney injury (AKI) occurred in 9 patients and 2 patients required renal replacement therapy. Out of the 11 patients, 6 required mechanical ventilation and 7 patients died. All the 6 patients requiring mechanical ventilation died. Our case series shows COVID-19 infection can precipitate acute metabolic complications in known DM patients or as first manifestation in undiagnosed DM patients. Patients can present with DKA/HHNK symptoms and/or respiratory symptoms. Mechanical ventilation is a poor prognostic factor. Further studies are needed to characterize prognostic factors associated with mortality in this vulnerable patient population."}, {"id": "pubmed23n0604_10809", "title": "Diabetic non ketotic hyperosmolar state: a special care in aged patients.", "score": 0.012259980660312197, "content": "The hyperosmolar hyperglycemic nonketotic state (HHNS) is an acute metabolic complication occurring characteristically in elderly type-2 diabetic patients. It may account for 10 up to 47% of cases of severe hyperglycemia with or without ketoacidosis. Many factors associated with advanced age may explain the predilection of both elderly subjects in general and older diabetics in particular to develop hyperosmolar coma, including reduced glomerular filtration rate and elevated renal threshold for glucose (which fail to correct hyperglycemia by osmotic diuresis), lack of thirst appropriate to the state of hydratation and some iatrogenic factors. In HHNS the age of the patients is the best known prognostic indicator. The increased mortality rate in the elderly diabetics depends on the severity of precipitating acute diseases (gastrointestinal hemorrhage, cardiovascular accident, pneumonia, pancreatitis, etc.), but the frequent compromises of the hemodynamic state and renal function of aged subjects substantially contributes. However, the role of erroneous management is not negligible and difficulties may be encountered in conciliating correction of metabolic disorder with treatment of precipitating illness. Insulin, water and electrolytes are the most important therapeutical tools for the treatment of hyperglycemic emergencies. In HHNS, the aggressive fluid replacement with isotonic or hypotonic NaCl solutions have first priority. Such a type of strategy is difficult to perform in patients suffering from cerebral stroke (which needs of anti-edema therapy) or congestive heart failure (necessitating to avoid fluid excess). According to the literary data, in our experience these two precipitating factors are frequent causes of death. We outline the validity of prefixed protocols of management; on the other hand, we think that the pathophysiological understanding of HHNS in the single patient is essential to decide the proper corrections and to permit a successful outcome. The primary way aiming at diminishing mortality by HHNS is its prevention; it is fundamental to warrant an appropriate fluid intake and to utilize with caution some drugs (thiazides, steroids, phenytoin, etc.) in aged diabetics, especially when nephropathic or unable, or living in nursing homes."}, {"id": "wiki20220301en002_195785", "title": "Diabetic ketoacidosis", "score": 0.012217786343612334, "content": "If cerebral edema is suspected because of confusion, recurrent vomiting or other symptoms, computed tomography may be performed to assess its severity and to exclude other causes such as stroke. Criteria Diabetic ketoacidosis is distinguished from other diabetic emergencies by the presence of large amounts of ketones in blood and urine, and marked metabolic acidosis. Hyperosmolar hyperglycemic state (HHS, sometimes labeled \"hyperosmolar non-ketotic state\" or HONK) is much more common in type 2 diabetes and features increased plasma osmolarity (above 320 mosm/kg) due to profound dehydration and concentration of the blood; mild acidosis and ketonemia may occur in this state, but not to the extent observed in DKA. There is a degree of overlap between DKA and HHS, as in DKA the osmolarity may also be increased."}, {"id": "pubmed23n0393_19935", "title": "[Metformin-associated lactic acidosis with acute renal failure in type 2 diabetes mellitus].", "score": 0.0119857133044852, "content": "An 83-year-old patient was admitted to our hospital because of gastrointestinal symptoms, mental confusion and dysarthria. The patient suffered from type 2 diabetes mellitus and was taking metformin. A mild renal insufficiency was known. On admission, we found impaired consciousness, Kussmaul breathing, a body temperature of 32.1 degrees C, and hemodynamic instability. Laboratory testing revealed lactic acidosis (pH 6.71, base excess--30, standard bicarbonate 4.0 mmol/l, lactate 24.4 mmol/l) and acute renal failure with a creatinine of 10.6 mg/dl and blood urea nitrogen of 134 mg/dl. Electrolytes were not altered; the blood glucose was elevated (147 mg/dl). According to history, physical examination, and laboratory testing the diagnosis metformin-induced lactic acidosis with acute renal failure was made. This diagnosis was confirmed by an elevated level of metformin. As soon as possible a bicarbonate hemodialysis was initiated. After 8 hours of hemodialysis the acid-base metabolism was almost balanced and the vigilance of the patient normalized. No further sessions of hemodialysis were needed and insulin therapy was started. Metformin-induced lactic acidosis is a common side effect in patients with renal insufficiency. For an early diagnosis, clinical symptoms of intoxication should be well known by physicians and patients. First-line therapy for correction of lactic acidosis and effective elimination of metformin is bicarbonate hemodialysis. Sodium bicarbonate infusions are not able to correct the acid-base metabolism sufficiently. For prevention the renal function should be monitored closely and metaformin therapy should be stopped, if a deterioration of renal function is observed."}, {"id": "InternalMed_Harrison_27965", "title": "InternalMed_Harrison", "score": 0.01131808058311399, "content": "HYPERGLYCEMIC HYPEROSMOLAR STATE Clinical Features The prototypical patient with HHS is an elderly individual with type 2 DM, with a several-week history of polyuria, weight loss, and diminished oral intake that culminates in mental confusion, lethargy, or coma. The physical examination reflects profound dehydration and hyperosmolality and reveals hypotension, tachycardia, and altered mental status. Notably absent are symptoms of nausea, vomiting, and abdominal pain and the Kussmaul respirations characteristic of DKA. HHS is often precipitated by a serious, concurrent illness such as myocardial infarction or stroke. Sepsis, pneumonia, and other serious infections are frequent precipitants and should be sought. In addition, a debilitating condition (prior stroke or dementia) or social situation that compromises water intake usually contributes to the development of the disorder."}, {"id": "pubmed23n0518_8809", "title": "Hyperosmolar hyperglycemic state.", "score": 0.01119554679926197, "content": "Hyperosmolar hyperglycemic state is a life-threatening emergency manifested by marked elevation of blood glucose, hyperosmolarity, and little or no ketosis. With the dramatic increase in the prevalence of type 2 diabetes and the aging population, this condition may be encountered more frequently by family physicians in the future. Although the precipitating causes are numerous, underlying infections are the most common. Other causes include certain medications, non-compliance, undiagnosed diabetes, substance abuse, and coexisting disease. Physical findings of hyperosmolar hyperglycemic state include those associated with profound dehydration and various neurologic symptoms such as coma. The first step of treatment involves careful monitoring of the patient and laboratory values. Vigorous correction of dehydration with the use of normal saline is critical, requiring an average of 9 L in 48 hours. After urine output has been established, potassium replacement should begin. Once fluid replacement has been initiated, insulin should be given as an initial bolus of 0.15 U per kg intravenously, followed by a drip of 0.1 U per kg per hour until the blood glucose level falls to between 250 and 300 mg per dL. Identification and treatment of the underlying and precipitating causes are necessary. It is important to monitor the patient for complications such as vascular occlusions (e.g., mesenteric artery occlusion, myocardial infarction, low-flow syndrome, and disseminated intravascular coagulopathy) and rhabdomyolysis. Finally, physicians should focus on preventing future episodes using patient education and instruction in self-monitoring."}, {"id": "pubmed23n0754_13149", "title": "Diabetic ketoacidosis: evaluation and treatment.", "score": 0.01098901098901099, "content": "Diabetic ketoacidosis is characterized by a serum glucose level greater than 250 mg per dL, a pH less than 7.3, a serum bicarbonate level less than 18 mEq per L, an elevated serum ketone level, and dehydration. Insulin deficiency is the main precipitating factor. Diabetic ketoacidosis can occur in persons of all ages, with 14 percent of cases occurring in persons older than 70 years, 23 percent in persons 51 to 70 years of age, 27 percent in persons 30 to 50 years of age, and 36 percent in persons younger than 30 years. The case fatality rate is 1 to 5 percent. About one-third of all cases are in persons without a history of diabetes mellitus. Common symptoms include polyuria with polydipsia (98 percent), weight loss (81 percent), fatigue (62 percent), dyspnea (57 percent), vomiting (46 percent), preceding febrile illness (40 percent), abdominal pain (32 percent), and polyphagia (23 percent). Measurement of A1C, blood urea nitrogen, creatinine, serum glucose, electrolytes, pH, and serum ketones; complete blood count; urinalysis; electrocardiography; and calculation of anion gap and osmolar gap can differentiate diabetic ketoacidosis from hyperosmolar hyperglycemic state, gastroenteritis, starvation ketosis, and other metabolic syndromes, and can assist in diagnosing comorbid conditions. Appropriate treatment includes administering intravenous fluids and insulin, and monitoring glucose and electrolyte levels. Cerebral edema is a rare but severe complication that occurs predominantly in children. Physicians should recognize the signs of diabetic ketoacidosis for prompt diagnosis, and identify early symptoms to prevent it. Patient education should include information on how to adjust insulin during times of illness and how to monitor glucose and ketone levels, as well as information on the importance of medication compliance."}, {"id": "wiki20220301en008_125508", "title": "Diabetic coma", "score": 0.010561247971319914, "content": "Diabetic coma is a life-threatening but reversible form of coma found in people with diabetes mellitus. Three different types of diabetic coma are identified: Severe low blood sugar in a diabetic person Diabetic ketoacidosis (usually type 1) advanced enough to result in unconsciousness from a combination of a severely increased blood sugar level, dehydration and shock, and exhaustion Hyperosmolar nonketotic coma (usually type 2) in which an extremely high blood sugar level and dehydration alone are sufficient to cause unconsciousness."}, {"id": "wiki20220301en008_126400", "title": "Type 2 diabetes", "score": 0.010250335983235007, "content": "Type 2 diabetes (T2D), formerly known as adult-onset diabetes, is a form of diabetes that is characterized by high blood sugar, insulin resistance, and relative lack of insulin. Common symptoms include increased thirst, frequent urination, and unexplained weight loss. Symptoms may also include increased hunger, feeling tired, and sores that do not heal. Often symptoms come on slowly. Long-term complications from high blood sugar include heart disease, strokes, diabetic retinopathy which can result in blindness, kidney failure, and poor blood flow in the limbs which may lead to amputations. The sudden onset of hyperosmolar hyperglycemic state may occur; however, ketoacidosis is uncommon. Type 2 diabetes primarily occurs as a result of obesity and lack of exercise. Some people are more genetically at risk than others."}, {"id": "Pathology_Robbins_4628", "title": "Pathology_Robbins", "score": 0.010232769669657254, "content": "Type 2 diabetes also may manifest with polyuria and polydipsia. In some cases, medical attention is sought because of unexplained weakness or weight loss. Most frequently, however, the diagnosis is made after routine blood or urine testing in asymptomatic individuals. In the decompensated state, patients with type 2 diabetes may develop hyperosmolar nonketotic coma. This syndrome is engendered by severe dehydration resulting from sustained osmotic diuresis and urinary fluid loss due to chronic hyperglycemia. Typically, the affected individual is an older adult diabetic who is disabled by a stroke or an infection and is unable to maintain adequate water intake. The absence of ketoacidosis and its symptoms (nausea, vomiting, respiratory difficulties) delays recognition of the seriousness of the situation until the onset of severe dehydration and coma."}, {"id": "article-40870_20", "title": "Case Study: 60-Year-Old Female Presenting With Shortness of Breath -- Diagnosis", "score": 0.010050778463217088, "content": "Myxedema coma or severe hypothyroidism Pericardial effusion secondary to myxedema coma COPD exacerbation Acute on chronic hypoxic respiratory failure Acute respiratory alkalosis Bilateral community-acquired pneumonia Small bilateral pleural effusions Acute mild rhabdomyolysis Acute chronic, stage IV, renal failure Elevated troponin I levels, likely secondary to Renal failure Diabetes mellitus type 2, non-insulin-dependent Extreme obesity Hepatic dysfunction"}]}}}} {"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[959, 1213]], "word_ranges": [[159, 202]], "text": "IVUS seems to me to be one of the best performing options. It will give us diagnostic clarity of obstruction, its magnitude and its renal repercussions. It has long been considered the examination of choice for the study of patients with renal lithiasis."}, "3": {"exist": true, "char_ranges": [[1482, 1667]], "word_ranges": [[246, 279]], "text": "Contrast-enhanced CT seems to me to be the most efficient option. It serves to discover lithiasis that other tests cannot find and gives us a global image of the rest of the structures."}, "4": {"exist": true, "char_ranges": [[2201, 2393]], "word_ranges": [[364, 392]], "text": "Ultrasound is a fast, cheap and innocuous test, but very radiologist-dependent. It gives great renal information on obstructive repercussions, but less quality in terms of finding a lithiasis."}, "5": {"exist": true, "char_ranges": [[2829, 2889]], "word_ranges": [[454, 462]], "text": "Doppler echocardiography has no use in urological diagnosis."}}, "full_answer": "In this question I have more doubts as to which is the correct answer, since we must interpret which is the most useful test, with which we can obtain more performance. However, this situation also depends on the radiology services of each center and their preferences. I will focus on the urological part, leaving aside the rest of the symptomatology. - Abdominal X-ray is the first imaging study to be performed because it is fast, simple and practically innocuous (except in children and pregnant women). 90% of the stones are radiopaque and therefore visible, but the sensitivity of this test, used in isolation, decreases to 45-59%. It is a mandatory test in any suspicion of colic pain. However, it has a lower yield than the others. Although the urological history speaks of uric lithiasis (radiotransparent), with an acid pH that reaffirms the diagnosis of uric origin, we can never rule out calcium oxalate lithiasis that can appear synchronously. - IVUS seems to me to be one of the best performing options. It will give us diagnostic clarity of obstruction, its magnitude and its renal repercussions. It has long been considered the examination of choice for the study of patients with renal lithiasis. It is a test with high sensitivity and specificity (87-90% and 94-100%, respectively), relatively innocuous, and available in all hospitals. It informs us of the number, size, shape and location of the stone, as well as the functional status of the affected kidney. - Contrast-enhanced CT seems to me to be the most efficient option. It serves to discover lithiasis that other tests cannot find and gives us a global image of the rest of the structures. Great performance but also more expensive, more time, more radiation. It has been confirmed as a high sensitivity and specificity scan, surpassing previous tests (94-100% and 92-100%, respectively, for the detection of ureteral calculi) and with its numerous advantages it has become the reference test and is expected to replace IVUS and ultrasound worldwide. It performs a helical scan with 5 mm slices allowing the detection of stones up to 2 mm. With the administration of contrast, it allows the evaluation of renal function. - Ultrasound is a fast, cheap and innocuous test, but very radiologist-dependent. It gives great renal information on obstructive repercussions, but less quality in terms of finding a lithiasis. Ultrasonography only detects calculi larger than 4 mm, located in the pyelo-ureteral junction or the uretero-vesical junction, while the lumbar and pelvic ureter is not very accessible due to the interposition of the intestinal loops. Therefore, ultrasound performed in isolation is not very sensitive (20-45%) for the detection of calculi, but associated with simple abdominal radiography increases its sensitivity and specificity. - Doppler echocardiography has no use in urological diagnosis. My answer would be CT with ev contrast and excretory phase (answer (c)), in terms of performance, sensitivity and specificity. There are radiology services more skilled or predisposed to one or the other. Without forgetting that abdominal X-ray should always be done in the suspicion of colic.", "full_answer_no_ref": "In this question I have more doubts as to which is the correct answer, since we must interpret which is the most useful test, with which we can obtain more performance. However, this situation also depends on the radiology services of each center and their preferences. I will focus on the urological part, leaving aside the rest of the symptomatology. - Abdominal X-ray is the first imaging study to be performed because it is fast, simple and practically innocuous (except in children and pregnant women). 90% of the stones are radiopaque and therefore visible, but the sensitivity of this test, used in isolation, decreases to 45-59%. It is a mandatory test in any suspicion of colic pain. However, it has a lower yield than the others. Although the urological history speaks of uric lithiasis (radiotransparent), with an acid pH that reaffirms the diagnosis of uric origin, we can never rule out calcium oxalate lithiasis that can appear synchronously. - IVUS seems to me to be one of the best performing options. It will give us diagnostic clarity of obstruction, its magnitude and its renal repercussions. It has long been considered the examination of choice for the study of patients with renal lithiasis. It is a test with high sensitivity and specificity (87-90% and 94-100%, respectively), relatively innocuous, and available in all hospitals. It informs us of the number, size, shape and location of the stone, as well as the functional status of the affected kidney. - Contrast-enhanced CT seems to me to be the most efficient option. It serves to discover lithiasis that other tests cannot find and gives us a global image of the rest of the structures. Great performance but also more expensive, more time, more radiation. It has been confirmed as a high sensitivity and specificity scan, surpassing previous tests (94-100% and 92-100%, respectively, for the detection of ureteral calculi) and with its numerous advantages it has become the reference test and is expected to replace IVUS and ultrasound worldwide. It performs a helical scan with 5 mm slices allowing the detection of stones up to 2 mm. With the administration of contrast, it allows the evaluation of renal function. - Ultrasound is a fast, cheap and innocuous test, but very radiologist-dependent. It gives great renal information on obstructive repercussions, but less quality in terms of finding a lithiasis. Ultrasonography only detects calculi larger than 4 mm, located in the pyelo-ureteral junction or the uretero-vesical junction, while the lumbar and pelvic ureter is not very accessible due to the interposition of the intestinal loops. Therefore, ultrasound performed in isolation is not very sensitive (20-45%) for the detection of calculi, but associated with simple abdominal radiography increases its sensitivity and specificity. - Doppler echocardiography has no use in urological diagnosis. My answer would be CT with ev contrast and excretory phase (answer (c)), in terms of performance, sensitivity and specificity. There are radiology services more skilled or predisposed to one or the other. Without forgetting that abdominal X-ray should always be done in the suspicion of colic.", "full_question": "A 45-year-old male patient with a history of uric lithiasis with repeated expulsive nephritic colic for 25 years, comes to the ED referring palpitations and intense right lumbar pain since 2 hours before. Urinalysis showed a pH of 5.5 and leukocyturia without proteinuria. The ECG confirms a previously unknown atrial fibrillation. Blood biochemistry shows a creatinine of 0.9mg/dl, a calcium of 11mg/dl and an LDH of 950 U/l. What is the most useful diagnostic maneuver to perform?", "id": 159, "lang": "en", "options": {"1": "Simple abdominal X-ray.", "2": "IVU.", "3": "CT with contrast.", "4": "Abdominal echography.", "5": "Doppler echocardiography."}, "question_id_specific": 111, "type": "UROLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0501_7673", "title": "Acute renal embolism. Forty-four cases of renal infarction in patients with atrial fibrillation.", "score": 0.01690340909090909, "content": "Acute renal embolus is rarely reported in the medical literature; thus, accurate data regarding presentation, laboratory tests, diagnostic techniques, and treatment are lacking. To better define this condition, we examined the medical records of all patients admitted to Kaplan Medical Center and Sheba Medical Center in central Israel from 1984 to 2002 who had a diagnosis of renal infarction and atrial fibrillation. We noted demographic, clinical, and laboratory parameters; method of diagnosis; treatment received; and patient outcome. We identified 44 cases of renal embolus: 23 females and 21 males, with an average age of 69.5 +/- 12.6 years. Thirty (68%) patients had abdominal pain, and 6 (14%) had a previous embolic event. Nine patients were being treated with warfarin on admission, 6 (66%) of whom had an international normalized ratio (INR) < 1.8. Hematuria was present in 21/39 (54%), and 41 (93%) patients had a serum lactate dehydrogenase (LDH) level > 400 U/dL. The mean LDH was 1100 +/- 985 U/dL. Diagnostic techniques included renal isotope scan, which was abnormal in 36/37 cases (97%); contrast-enhanced computed tomography (CT) scan, which was diagnostic in 12/15 cases (80%); and ultrasound, which was positive in only 3/27 cases (11%). Angiography was positive in 10/10 cases (100%). Twenty-three (61%) of 38 patients had normal renal function on follow-up. The 30-day mortality was 11.4%. Renal embolus was diagnosed mainly in patients aged more than 60 years, some of whom had a previous embolic event. Most of those receiving anticoagulant therapy had a subtherapeutic INR. Abdominal pain was common, as well as hematuria and an elevated LDH. These patients are at risk of subsequent embolic events to other organs. The most sensitive diagnostic technique in this population is a renal isotope scan, but contrast-enhanced CT scan requires further assessment."}, {"id": "pubmed23n0343_20329", "title": "Heightened suspicion and rapid evaluation with CT for early diagnosis of partial renal infarction.", "score": 0.0166052895882156, "content": "Although renal infarction has been well studied and clearly defined, it remains greatly underdiagnosed, resulting in significant morbidity. Acute segmental renal infarction is a diagnosis even more problematic, as the problem can appear insidiously and masquerade as other entities including stone, infection, and even tumor. The clinical manifestations and evaluation of partial renal infarction in our patients were reviewed. Seven patients presenting to the emergency department who were subsequently found to have partial renal infarction were identified from the 5-year records of a single institution. Patients were evaluated for presenting complaints, physical findings, temperature, and blood pressure. Laboratory analysis consisted of a complete blood count (CBC); measurements of creatinine, lactate dehydrogenase (LDH), aspartate transaminase/alanine transaminase (AST/ALT), and alkaline phosphatase; and urinalysis. The sequence of the work-up was recorded, as well as time to diagnosis. The etiology of infarction was identified for all patients. All seven patients were eventually discovered to have partial renal infarction as a result of dysrhythmia (N = 4), mural thrombus (N = 2), or septic emboli (N = 1). The average time to diagnosis was 65.2 hours with a range of 9.5 to 168 hours. The chief complaint was flank pain (N = 3), nonspecific abdominal pain (N = 2), left lower-quadrant pain (N = 1), and mental status change (N = 1). The presenting signs and symptoms included abdominal tenderness (N = 4), nausea and vomiting (N = 4), temperature >100.5 degrees F (N = 3), and hypertension (N = 3). Laboratory studies revealed a white cell count >11,000/microL in six, microhematuria in four, proteinuria in four, elevated LDH in all patients, elevated AST/ALT in two, and elevated alkaline phosphatase in one. The work-up varied by presentation, but definitive diagnosis was made by CT in all five patients scanned and by angiography in two. Angiography confirmed the CT findings in four of the five patients. In evaluating partial renal infarction, a strong clinical suspicion is necessary. We found a history of dysrhythmia or other cardiac disease, the presence of abdominal or flank pain, fever with an elevated white cell count, and an elevated LDH to be clinically significant, and their presence should alert the clinician to the possibility of renal infarction. Once a degree of suspicion exists, early evaluation with CT should speed the diagnosis and effect decreased morbidity."}, {"id": "pubmed23n0406_14754", "title": "The clinical spectrum of acute renal infarction.", "score": 0.015628686010851614, "content": "Acute renal infarction is an oft-missed diagnosis. As a result, its true incidence, although presumed to be low, is actually unknown. Surprisingly, the medical literature on the subject, other than anecdotal case reports, is scarce. To increase physician awareness of the diagnosis and to identify predictive clinical and laboratory features of the entity. Between 1 November 1997 and 31 October 2000, 11 cases of acute renal infarction in 10 patients were diagnosed in our center by contrast-enhanced computerized tomography. The medical charts of these patients were reviewed regarding risk factors, clinical presentation, possible predictive laboratory examinations, and outcome. During the 36 month observation period, the incidence of acute renal infarction was 0.007%. The mean age of the patients (5 men and 5 women) was 67.4 +/- 21.1 (range 30-87 years). In four cases the right and in five the left kidney was involved; in the other two cases bilateral involvement was seen. In 7/10 patients, an increased risk for thromboembolic events was found. Six had chronic atrial fibrillation and one had a combined activated protein C resistance and protein S deficiency. Three patients had suffered a previous thromboembolic event. Two cases were receiving anticoagulant therapy with an INR of 1.6 and 1.8, respectively. On admission, flank pain was recorded in 10/11, fever in 5 and nausea/vomiting in 4 cases. Hematuria was detected in urine reagent strips in all cases. Serum lactate dehydrogenase and white blood cell count were elevated in all cases (1,570 +/- 703 IU/L and 12,988 +/- 3,841/microliter, respectively). In no case was the diagnosis of acute renal infarction initially entertained. The working diagnoses were renal colic in 2, pyelonephritis in 3, renal carcinoma, digitalis intoxication, and suspected endocarditis in one patient each, and an acute abdomen in 3. Time from admission to definitive CT diagnosis ranged from 24 hours to 6 days. Three patients were treated with intravenous heparin and another with a combination of i.v. heparin and renal intra-arterial urokinase infusion with, in the latter case, no recovery of function of the affected kidney. With the exception of this one patient (with a contralateral contracted kidney) who required maintenance dialysis, in all other cases serum creatinine levels remained unchanged or reverted to the baseline mean of 1.1 mg/dl (0.9-1.2). Acute renal infarction is not as rare as previously assumed. The entity is often misdiagnosed. Unilateral flank pain in a patient with an increased risk for thromboembolism should raise the suspicion of renal infarction. In such a setting, hematuria, leucocytosis and an elevated LDH level are strongly supportive of the diagnosis."}, {"id": "pubmed23n1085_24991", "title": "Case 294.", "score": 0.015335772707705553, "content": "History A 50-year-old woman presented to the emergency department of our hospital with a 2-day history of lower limb pain associated with unusual asthenia and diffuse arthralgia over the past 3 weeks. She was a native of Guinea and had lived in France for most of her life, working as a personal care assistant. Her only medical history of note was an occurrence of fetal death at 12 weeks gestation when she was 35 years old. She had bilateral lower limb swelling, without changes in skin temperature or color. All proximal and distal arterial pulses were felt. General physical examination findings were otherwise unremarkable. Her laboratory tests showed a decreased hemoglobin concentration of 8.9 g/dL (normal range, 12-16 g/dL), a decreased platelet count of 45 × 1070 years with no previous history of nephrolithiasis Atrial fibrillation C-reactive protein >0.23 mg/dL Serum LDH >500 IU/L (especially with normal serum aminotransferases) More likely to be diabetic and hypertensive Negative urinalysis for RBCs Negative non-contrast CT scan"}, {"id": "pubmed23n0307_17528", "title": "Intravenous urography revisited in the age of ultrasound and computerized tomography: diagnostic yield in cases of renal colic, suspected pelvic and abdominal malignancies, suspected renal mass, and acute pyelonephritis.", "score": 0.012240494084381277, "content": "The aim of our study was to assess the diagnostic yield of intravenous urography (IVU) compared to ultrasound (US) and computerized tomography (CT) in cases of renal colic, suspected pelvic and abdominal malignancies, suspected renal mass, and acute pyelonephritis. We retrospectively analyzed the case charts of 216 consecutive patients. The patients had been referred to the Department of Radiology by different hospital departments and local general practitioners. All had undergone clinical examination, US and IVU, in that order. When deemed necessary, conventional tomography was performed. Patients with renal masses also underwent CT. In cases without renal colic and normal US examination, the subsequent IVU failed to detect any further important pathology. Hydronephrosis was equally well detected using US and IVU, however, the level of obstruction was better determined using delayed X-ray films. In 24% of cases of renal colics the initial US was normal, however, the IVU revealed ureteric obstruction. Repeat US 8-12 h later always showed hydronephrosis. In 6 of 34 solid renal masses, IVU and conventional tomography failed to make the correct diagnosis, but never could the patient be spared a subsequent CT. IVU is only indicated if US shows hydronephrosis. In cases of renal colic, repeat US is necessary to diagnose the possibly developing hydronephrosis. Clinical history, US and a plain abdominal image will suffice to make the diagnosis. Renal masses always require CT. In these cases, IVU is not necessary. There is no indication left for conventional renal tomographies."}, {"id": "pubmed23n0746_13357", "title": "Aortic dissection or renal infarction--multislice computed tomographic angiography can tell.", "score": 0.01142857142857143, "content": "Acute renal infarction as a consequence of renal artery occlusion often goes unrecognized, mostly due to the non-specific clinical features. A quick diagnosis, ideally within three hours of presentation, is a key to renal function recovery. A 62-year-old male patient was admitted with a sudden abdominal pain, right flank pain and nausea. He had a diastolic hypertension at admission and his previous medical history showed atrial fibrillation. Initial clinical diagnosis was aortic dissection. Laboratory findings included elevated lactate dehydrogenase (LDH) and serum creatinine levels. There were no signs of aortic dissection or aneurismatic lesions registered during a multislice computed tomographic (MSCT) angiography. However, MSCT angiography demonstrated left \"upper\" renal artery thrombosis and renal infarction--avascular area of the upper two thirds of the left kidney sharply demarcated from the surrounding parenchyma. Both kidneys excreted the contrast. Anticoagulant therapy was initiated, along with antiarrythmic and antihypertensive medications. The follow-up by computed tomography was performed after nine weeks, and it showed a partial revascularization of the previously affected area. Concomitant presence of flank/abdominal pain, an increased risk for thromboembolism and an elevated LDH suggested a possibility of renal infarction. MSCT angiography is a non-invasive and accurate method in the diagnosis of renal artery occlusion and the resulting renal infarction."}, {"id": "article-143520_38", "title": "Renal Infarction -- Evaluation -- Imaging", "score": 0.010081521266795294, "content": "The initial investigation in patients presenting with acute flank pain, vomiting, and hematuria is usually a non-contrast CT scan of the abdomen and pelvis to rule out urolithiasis and pyelonephritis, which are the most common reasons for such a clinical presentation. [42] If a non-contrast CT of the abdomen and pelvis is non-contributory, a contrast-enhanced CT of the abdomen should be done to rule out renal infarction as a cause of flank pain. [15] [16] Domanovits et al. suggested that a contrast-enhanced CT should be performed within 24 hours in all patients presenting with the triad of a high risk of thromboembolic events, persistent flank/abdominal/lower back pain, and high LDH levels and/or hematuria. [25]"}, {"id": "pubmed23n1007_17839", "title": "[Clinical characteristics of patients with acute renal infarction: an analysis of 52 patients in a single center].", "score": 0.009900990099009901, "content": "To investigate the clinical characteristics of patients with acute renal infarction (ARI) and explore the possible clinical and/or laboratory parameters relative to hematuria. Medical records of 52 patients hospitalized with radiologic proven ARI were retrospectively reviewed. Clinical characteristics, including demographic data, risk factors for thromboembolism, initial clinical presentations, laboratory data, diagnosis, treatment programs and outcomes were evaluated and compared between hematuria(+) and hematuria(-) patients. The mean age of the patients (34 men and 18 women) was (56.3±14.8) years. The left, right, and bilateral kidneys were involved in 44.2%, 34.6% and 21.2% of the patients, respectively. Focal, multiple and massive infarctions were involved in 36.5%, 50.0% and 13.5% of the patients. The prevalence of concurrent thromboembolic events was 38.5%. Atrial fibrillation was complicated in 44.2% of the patients. ARI often presented with nonspecific symptoms, including abdominal/flank pain (71.2%), nausea (55.8%), lumbar pain (53.9%), vomiting (48.1%), fever (48.1%), and diarrhea (21.2%). Percussion tenderness over kidney region was the most common sign (40.4%). The levels of serum lactate dehydrogenase, white blood cell count and C-reactive protein were elevated in 86.5%, 67.3%, and 54.5% of cases, respectively. Hematuria was detected in only 38.5% of the cases on admission. Elevation of serum D-dimer was only noted in 56.5% of the patients. The median duration from hospital presentation to diagnosis was 41.5 h (range: 2-552 h). Contrast-enhanced computed tomography was diagnostic in 47 (90.4%) cases. Angiography was positive in the other 5 (9.6%) cases. Anticoagulation was the most common therapy. During a mean follow-up of (39.4±35.8) months, renal functions of most patients were stable. Four patients needed permanent dialysis and one patient died of heart failure. There was no statistical significance between hematuria (+) group and hematuria (-) group for all the parameters except the level of serum lactate dehydrogenase, which was higher in hematuria(+) group [773.5 IU/L (range: 153.0-3 159.0 IU/L) vs. 488.0 IU/L (range: 137.0-3 370.0 IU/L), P=0.041]. Thromboembolism due to heart disease is the main etiology of ARI. Early contrastenhanced computed tomography scan should be considered for high-risk patients with persisting abdominal or lumbar pain and elevated serum level of lactate dehydrogenase. Hematuria is not a sensitive clue for diagnosis and is not relative to prognosis. Whether it is present may be determined by the severity of infarction."}, {"id": "pubmed23n1102_22675", "title": "Case 294: Catastrophic Antiphospholipid Syndrome.", "score": 0.00980392156862745, "content": "History A 50-year-old woman presented to the emergency department of our hospital with a 2-day history of lower limb pain associated with unusual asthenia and diffuse arthralgia over the past 3 weeks. She was a native of Guinea and had lived in France for most of her life, working as a personal care assistant. Her only medical history of note was an occurrence of fetal death at 12 weeks gestation when she was 35 years old. She had bilateral lower limb swelling, without changes in skin temperature or color. All proximal and distal arterial pulses were felt. General physical examination findings were otherwise unremarkable. Her laboratory tests showed a decreased hemoglobin concentration of 8.9 g/dL (normal range, 12-16 g/dL), a decreased platelet count of 45 × 10 55 years white blood cell count > 16000 cells/mm3 blood glucose > 11.1 mmol/L (> 200 mg/dL) serum AST > 250 IU/L serum LDH > 350 IU/L At 48 hours Calcium (serum calcium < 2.0 mmol/L (< 8.0 mg/dL) Hematocrit fall >10% Oxygen (hypoxemia PO2 < 60 mmHg) BUN increased by 1.8 or more mmol/L (5 or more mg/dL) after IV fluid hydration Base deficit (negative base excess) > 4 mEq/L Sequestration of fluids > 6 L The criteria for point assignment is that a certain breakpoint be met at any time during that 48 hour period, so that in some situations it can be calculated shortly after admission. It is applicable to both gallstone and alcoholic pancreatitis. Alternatively, pancreatitis can be diagnosed by meeting any of the following:[2] Alternative Ranson score Ranson's score of ≥ 8 Organ failure Substantial pancreatic necrosis (at least 30% glandular necrosis according to contrast-enhanced CT)"}, {"id": "pubmed23n0623_9786", "title": "[Liver rupture of a subcapsular haematoma after pharmacologic revascularization (Streptokinase) for acute myocardial infarction--case report].", "score": 0.009009009009009009, "content": "We report the case of a 56 years old male patient, smoker, obese, with untreated arterial hypertension, hospitalized on 16.02.07 with the diagnosis of inferior acute myocardial infarction, for which he received thrombolysis with streptokinase, followed by anticoagulation with non fractioned heparin. Two days later he started to complain of acute abdominal pain, and laboratory findings showed a low hemoglobin level. Imaging findings (ultrasonography and CT scan) showed evidence of subcapsular liver haematoma, caused by bleeding at hepatic and splenic level. He received red blood packed cells, fresh frozen plasma, cryoprecipitate, activated factor VII and was transferred by helicopter to Fundeni Clinical Institute--Intensive care unit (ICU). On admission, the patient was conscious, anxious, dyspneic, with mild hypoxia, with no signs of low cardiac output and with a painful abdomen. ECG, echocardiography and elevated myocardial necrosis enzymes confirmed myocardial infarction. Shortly after admission there was a worsening of his clinical condition, with a decrease in hemoglobin level despite red blood packed cells administration (Hb=7.8 g/dl) and thrombocytopenia (82000/mmc), with normal coagulation tests, thus suggesting active intraabdominal bleeding. Echography and CT scan confirmed bleeding. Emergency surgery was performed, showing massive haemoperitoneum (approx 4.5 L of blood), due to spontaneous rupture of a subcapsular hematoma in the liver. The surgical hemostasis was performed on the liver parenchyma laceration. Duration of surgery was 4 hours. There were no significant cardiac events during surgery (no signs of ischemia on ECG, no ST elevation), despite the need for inotropic agent. After surgery, the patient was referred to the ICU, intubated and ventilated, with inotropic support - dobutamine. Sequential ECG's, enzymatic trend and echocardiographies were performed to monitor myocardial ischemia. The outcome was favourable, no further bleeding and no postoperative myocardial infarction occurred. Secondary prevention was started early (thromboprophylaxis, selective beta-blocker, angiotensin inhibitors and statins). The patient had a favorable outcome and was discharged from the ICU the fourth day after surgery. He had a total length of stay in hospital of seven days, with a follow-up in the cardiology department."}, {"id": "article-143520_39", "title": "Renal Infarction -- Evaluation -- Imaging", "score": 0.009009009009009009, "content": "The only findings of renal infarction in a non-contrast CT abdomen may be some perinephric stranding and mild renal swelling, but these can easily be misinterpreted as signs of a recently passed kidney stone. The standard test for a suspected renal infarction is a contrast-enhanced CT scan of the abdomen which should be done in cases of possible renal infarction with negative non-contrast studies, especially in patients with elevated LDH, atrial fibrillation, increased risk, or history of thrombosis, or elevated age (>70)."}, {"id": "pubmed23n0302_17256", "title": "Resolution of proteinuria secondary to bilateral renal vein thrombosis after treatment with systemic thrombolytic therapy.", "score": 0.008928571428571428, "content": "A case of significant proteinuria occurred as a result of bilateral renal vein thrombosis secondary to dehydration, which resolved after treatment with urokinase. The patient developed nausea and vomiting from viral gastroenteritis with subsequent volume contraction. He later noted the onset of aching lower abdominal and flank pain. On admission, he was noted to have a serum creatinine of 1.7 mg/dL, and 4+ proteinuria on urinalysis. A 24-hour urine collection showed 2.34 g protein. A renal venogram showed bilateral renal vein thrombosis (RVT) without involvement of the inferior vena cava. Therapy was initiated with heparin at 1,000 U/hr, followed by intravenous (IV) urokinase, 4,400 U/kg bolus, followed by 4,400 U/kg/hr with continuous infusion for 12 hours. A repeat renal venogram done at this time showed partial resolution of thrombosis bilaterally. A second 12-hour infusion of urokinase at 5,000 U/kg/hr was performed; at this time, the patient reported resolution of his flank and abdominal pain. A repeat 24-hour urine collection showed 60 mg protein with a normal creatinine clearance. Levels of antithrombin III, protein C, and protein S were all normal. A renal biopsy was performed and showed normal histology on light, immunofluorescent, and electron microscopic evaluation. The patient has done well on no therapy and has had no recurrence of thrombosis or proteinuria after 2.5 years. This is a US government work. There are no restrictions on its use."}, {"id": "pubmed23n0626_25257", "title": "[Acute renal failure as atypical complication of atrial fibrillation].", "score": 0.008928571428571428, "content": "We present a case of a 76-year-old woman with persistent atrial fibrillation, recent diagnosis of ischemic stroke and embolic occlusion of the iliac artery, who was admitted to our department with symptoms of acute renal failure. Using the data from medical history, physical examination, ECG, laboratory tests (urinalysis, LDH in serum and urine) and radiologic studies (color Doppler ultrasound, angio-CT) we diagnosed renal artery embolus and infarct of solitary functioning kidney. We presumed that this complication was related to the inadequate anticoagulation in the course of atrial fibrillation. Patient received intravenous heparin followed by oral warfarin and started renal replacement therapy."}, {"id": "pubmed23n0639_23104", "title": "[Right-sided prosthetic cardiac valve thrombosis: value of cinefluoroscopy in the diagnosis and follow-up of thrombolytic treatment].", "score": 0.008849557522123894, "content": "A 33-year-old woman (Pt. A) with a prosthetic cardiac valve in the pulmonary position [CarboMedics bileaflet valve, diameter 23 mm] as part of the repair of a tetralogy of Fallot 4 years previously, and a 51-year-old woman (Pt. B) with a prosthetic cardiac valve [St. Jude Medical bileaflet valve, diameter 31 mm] inserted in tricuspid position as replacement of a degenerated Hancock bioprosthetic valve inserted 15 years previously, 10 years after an episode of endocarditis, were admitted to hospital with dyspnea and chest pain and dyspnea and tachycardia, respectively. Pt. A had a 3 - 4/6 crescendo-decrescendo systolic murmur and a 2/6 early diastolic decrescendo murmur over the 2nd to 4th right intercostal space (ICS), while Pt. B had a 3/6 holosystolic murmur and a 2 - 3/6 diastolic murmur over the 4th right ICS. Closing click was missing in both patients. Blood tests demonstrated an elevated LDH (404 U/l) in Pt. A and an elevated GGT (108 U/l) and fibrinogen (449 mg/dl) in Pt. B. Anticoagulation was below the therapeutic level, with an INR value of 1,65 and 1,93, respectively. The electrocardiogram showed sinus rhythm, right bundle branch block and an isoelectric ST-segment (Pt. A) and a typical high-frequency atrial flutter with a 2:1 block, right bundle branch block and terminal T-wave inversions in leads V1 to V5 (Pt. B). Cinefluoroscopy showed rigid and hypomobile leaflets as a result of prosthetic cardiac valve thrombosis. Doppler echocardiography confirmed the stenosis of the prosthetic valve in the pulmonary position (peak gradient 73 mm Hg, mean gradient 34 mm Hg) and the tricuspid position (mean gradient 8.48 mm Hg, peak gradient 16.73 mm Hg). Both patients were treated with unfractionated heparin and urokinase single-bolus injection of 4400 U/kg over 10 min followed by an infusion of 4400 U/kg/h over 12 h. Both patients had an abnormal opening angle, which improved to a normal opening and closing angle. Doppler echocardiography demonstrated decreased peak (18.0 and 6.6 mm Hg, respectively) and median gradients (9.0 and 2.6 mm Hg, respectively). No further complications (such as bleeding, embolism, delayed surgical treatment, rethrombosis) had occurred, and both patients became asymptomatic. After oral anticoagulation in a therapeutic INR range for 12 and 4 months, respectively, prosthetic heart valve function continued to be normal in both patients. Thrombolysis appears to be an efficacious and safe treatment in patients with thrombosis of a prosthetic cardiac valve in the pulmonary or tricuspid position, and it may be used as first-line therapy. Cinefluoroscopy is a simple and accurate method both in the diagnosis of prosthetic cardiac valve thrombosis and in following the response to thrombolytic treatment."}]}}}} {"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[0, 151]], "word_ranges": [[0, 22]], "text": "The relative mildness of the symptoms, which do not seriously interfere with daily activities, excludes in principle the diagnosis of major depression."}, "2": {"exist": true, "char_ranges": [[152, 226]], "word_ranges": [[22, 35]], "text": "There is no evidence of hypomanic phases to justify a bipolar II disorder."}, "3": {"exist": true, "char_ranges": [[677, 979]], "word_ranges": [[108, 158]], "text": "The adaptive disorder appears within 3 months after the stressful event and does not last more than 6 months if the stressful event or its consequences have ceased (which is not the case because the brother of the imaginary patient is still dead). Mild anxious-depressive symptoms fit such a diagnosis."}, "4": {"exist": true, "char_ranges": [[227, 279]], "word_ranges": [[35, 44]], "text": "Dysthymia requires a duration of at least two years."}, "5": {"exist": true, "char_ranges": [[280, 630]], "word_ranges": [[44, 100]], "text": "Cyclothymia is a very rare diagnosis nowadays (because, as Castilla del Pino said in an interview, what used to be cyclothymics are now called bipolar, contributing to one of the multiple psychiatric epidemics of our time), which consists of oscillations between the depressive pole and the manifest, mild. But there are no data on such oscillations."}}, "full_answer": "The relative mildness of the symptoms, which do not seriously interfere with daily activities, excludes in principle the diagnosis of major depression. There is no evidence of hypomanic phases to justify a bipolar II disorder. Dysthymia requires a duration of at least two years. Cyclothymia is a very rare diagnosis nowadays (because, as Castilla del Pino said in an interview, what used to be cyclothymics are now called bipolar, contributing to one of the multiple psychiatric epidemics of our time), which consists of oscillations between the depressive pole and the manifest, mild. But there are no data on such oscillations. The correct answer is, in all probability, 3. The adaptive disorder appears within 3 months after the stressful event and does not last more than 6 months if the stressful event or its consequences have ceased (which is not the case because the brother of the imaginary patient is still dead). Mild anxious-depressive symptoms fit such a diagnosis. One more example of how to conceptualize as illness what are facts (albeit painful) of life. Surely the imaginary patient takes some antidepressant because maybe something helps (1).", "full_answer_no_ref": "The relative mildness of the symptoms, which do not seriously interfere with daily activities, excludes in principle the diagnosis of major depression. There is no evidence of hypomanic phases to justify a bipolar II disorder. Dysthymia requires a duration of at least two years. Cyclothymia is a very rare diagnosis nowadays (because, as Castilla del Pino said in an interview, what used to be cyclothymics are now called bipolar, contributing to one of the multiple psychiatric epidemics of our time), which consists of oscillations between the depressive pole and the manifest, mild. But there are no data on such oscillations. [HIDDEN] The adaptive disorder appears within 3 months after the stressful event and does not last more than 6 months if the stressful event or its consequences have ceased (which is not the case because the brother of the imaginary patient is still dead). Mild anxious-depressive symptoms fit such a diagnosis. One more example of how to conceptualize as illness what are facts (albeit painful) of life. Surely the imaginary patient takes some antidepressant because maybe something helps [HIDDEN].", "full_question": "A 36-year-old patient from another culture who has been living in Spain for the last 4 years presents at a National Health Service office. He reports presenting with anxious symptoms and depressive mood for the last 10 months. This condition moderately interferes with her daily activities. She has no previous psychiatric history. This situation is the result of the death, in a car accident, of an older brother with whom he was very close. Indicate which of the following diagnoses is appropriate:", "id": 52, "lang": "en", "options": {"1": "Major depression.", "2": "Bipolar II disorder.", "3": "Adjustment disorder.", "4": "Dysthymia.", "5": "Cyclothymia."}, "question_id_specific": 144, "type": "PSYCHIATRY", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0952_21732", "title": "When Dementia Kills Before One Passes Away: Case Report of Cotard's Syndrome.", "score": 0.018283519786274835, "content": "Cotard's Syndrome (CS), among the noncognitive changes in the forms of dementia, is a seldom-found manifestation (1). This syndrome is characterized by the delusion of having lost organs (the individual experiences huge changes in the body and believes that he/she no longer has one or more organs) and by nihilistic delusion (the individual believes he/she or everyone in the world has died or been destroyed) (2). In 1880, Jules Cotard (1840-1889) described a clinical condition that he believed corresponded to a new subtype of depression, which he called anxious melancholia. He proposed that a state of acute depression and morbid anxiety could foster the development of structured delusions of hypochondria. Two years later he referred to the same clinical condition using the term délires des négations for the first time (3). The eponym CS was only introduced in 1893 by Emil Regis, who stated that Cotard had not described a new clinical entity but rather a syndrome - a cluster of symptoms that could also be found in other mental illnesses apart from depression and in which anxiety was the central characteristic (4). The most prominent symptoms found in an analysis of one hundred cases were: depressed mood (89%), nihilistic delusion (69%), anxiety (65%), delusion of guilt (63%), delusion of immortality (55%) and hypochondriacal delusions (58%) (12). Currently, CS is not classified as an isolated disorder in the Diagnostic and Statistical Manual of Mental Disorders (DSM-5) of the American Psychiatric Association or in the International Statistical Classification of Diseases and Related Health Problems (ICD-10). There is a growing consensus to consider it a secondary syndrome of an underlying disorder, of which the most associated disorders are: unipolar depression, bipolar depression and primary psychotic disorders. Other conditions have also been described, such as dementia, severe intellectual disability, cerebrovascular accident, brain tumor, and Parkinson's disease, among others (5)."}, {"id": "pubmed23n0286_1070", "title": "[Importance of DSM IV (APA) and ICD-10 (WHO) in diagnosis and treatment of mood disorders].", "score": 0.015234917987211567, "content": "Since 1993, with ICD-10 (WHO), and 1994, with DSM IV (APA), practitioners have had at their disposal two (practically compatible) classifications of mental disorders containing operational criteria for diagnosis, and helpful in guiding clinical and therapeutic approach. Moreover, the use of one of these classifications (ICD-10) is compulsory in French state psychiatric institutions. We shall try to convince that these manuals are useful, and indeed unavoidable, henceforth, not only for researchers but also for practitioners, for the following reasons: \"Any form of order is preferable to chaos\" (Lévi Strauss); An implicit classification is always at work, even where the clinician feels he is working by intuition and treating each patient as a unique and individual case. It is not necessarily a bad thing to compare one's own stereotypes with currently held beliefs; All efforts to evaluate treatments, both psychotherapeutic and chemotherapeutic, are now based on these clinical definitions and models. Particularly as regards mood disorders, DSM III and DMS IV have managed to rid us of the uncertainties and contradictions surrounding etiopathogenesis (endogenous? psychogenic? reactive? defensive? adaptive? biological? etc.) which previously ruled out any explanatory classification. There still remain a number of pathological pictures of proven existence but with different levels of significance and different treatments. The bipolar/unipolar distinction (BP/UP) has been strengthened. Major depression (actual \"depression\", of moderate severity) remains the central model, and exists in both the unipolar and bipolar forms. There is also chronic (more than 2 years) progressive dysthymia (UP) which corresponds almost exactly to \"Depressive personality\". For bipolar disorders, the distinction between bipolar I disorders and bipolar II disorders, which is now well-documented, has been retained. Cyclothymia (lasting over 2 years) is in a sense the bipolar equivalent of dysthymia. Mixed disorders are distinguished from rapid-cycling bipolar disorders. It is now known that the range of bipolar disorders requires treatment with thymoleptic drugs and that antidepressants should be used only in unipolar disorders, and occasionally in bipolar forms to treat certain acute episodes of depression. Furthermore, many specific clinical forms are defined: postnatal depression, seasonal depression, adaptive difficulties with depressive mood or anxiety and depression, iatrogenic depression or depression related to medication or to intercurrent illnesses. Moreover, criteria are also suggested \"for future studies\" of post-psychotic depression in schizophrenic patients, minor depression, brief recurrent depressive episodes and anxio-depressive syndrome."}, {"id": "pubmed23n0605_21592", "title": "Transfers to psychiatry through the consultation-liaison psychiatry service: 11 years of experience.", "score": 0.014174311926605506, "content": "There are only a few reports on issues related to patient transfer from medical and surgical departments to the psychiatric ward by the consultation-liaison psychiatry service, although it is a common practice. Here, we present a study assessing the factors that influence such transfers. We examined the demographic and clinical backgrounds of a group of patients transferred from internal medicine and surgery to the psychiatric ward over an 11-year period. A comparison was made of this data with data obtained from a group of non-transferred patients, also seen by the same consultation-liaison psychiatry service. According to our findings, the typical transferred patient, either female or male, is single, divorced or widowed, lives alone, belongs to a lower socioeconomic class, presents initially with (on the whole) a disturbed and disruptive behaviour, has had a recent suicide attempt with persistent suicidal ideas, suffers from a mood disorder (mainly depressive and dysthymic disorders), has a prior psychiatric history as well as a prior psychiatric inpatient treatment, and a positive diagnosis on axis II of the five axis system used for mental health diagnosis. The transfer of a patient to the psychiatric ward is a decision depending on multiple factors. Medical diagnoses do not seem to play a major role in the transfer to the psychiatric ward. From the psychiatric diagnosis, depressive and dysthymic disorders are the most common in the transferred population, whilst the transfer is influenced by social factors regarding the patient, the patient's behaviour, the conditions in the ward she/he is treated in and any recent occurrence(s) that increase the anxiety of the staff."}, {"id": "pubmed23n1115_2407", "title": "The Occurrence of Funeral Mania After Bereavement: A Case Report.", "score": 0.010477267830209008, "content": "Stressful or traumatic life events can lead to emergence of mood episodes. Events such as migration, relocation, job loss, bankruptcy, economic loss, divorce, natural disasters, accidental injury, or the loss of a loved one can trigger the first episode of bipolar disorder. After such life events, symptoms of depressive episodes often appear. Funeral mania, on the other hand, is defined as the emergence of manic episodes following the death of a close family member. Information on funeral mania, which occurs shortly after the loss of a loved one, is limited with a few case reports. In this study, a 26-year-old female patient who presented with the symptoms of a manic episode for the first time after her father's death and who had no previous psychiatric disease or treatment history was presented in the light of findings in the literature. It is noteworthy that the patient, who was followed up with the diagnosis of bipolar disorder (mania period) according to DSM-5 diagnostic criteria, had a temporal closeness between her mood symptoms and her father's death, and had not developed such a reaction to previous traumatic life events. Therefore, the diagnosis was evaluated as funeral mania. It should be kept in mind that, although rare, symptoms of mania can be seen among possible grief reactions."}, {"id": "wiki20220301en027_68257", "title": "List of people with bipolar disorder", "score": 0.00993051106401853, "content": "J Jesse Jackson, Jr., former member of the United States House of Representatives, has stated he's been diagnosed with bipolar II disorder. Kay Redfield Jamison, American clinical psychologist, professor of psychiatry and writer, has written extensively about her personal experiences with bipolar disorder, including in An Unquiet Mind. Jang Keun Suk, South Korean actor and singer. Jill Janus, American heavy metal singer. Adam Jasinski, winner of the U.S. series Big Brother 9. Andrew Johns, Australian rugby league player. Publicly announced his condition following retirement. \"Fast\" Eddie Johnson, American basketball player, was diagnosed with manic depression. Daniel Johnston, musician, singer-songwriter and visual artist. Lucia Joyce, daughter of writer James Joyce, was diagnosed with cyclothymia. Sarah Joyce, British singer–songwriter. Helmi Juvonen, American artist and painter, hospitalized and diagnosed with manic-depression."}, {"id": "wiki20220301en000_123512", "title": "Major depressive disorder", "score": 0.009900990099009901, "content": "To confirm major depressive disorder as the most likely diagnosis, other potential diagnoses must be considered, including dysthymia, adjustment disorder with depressed mood, or bipolar disorder. Dysthymia is a chronic, milder mood disturbance in which a person reports a low mood almost daily over a span of at least two years. The symptoms are not as severe as those for major depression, although people with dysthymia are vulnerable to secondary episodes of major depression (sometimes referred to as double depression). Adjustment disorder with depressed mood is a mood disturbance appearing as a psychological response to an identifiable event or stressor, in which the resulting emotional or behavioral symptoms are significant but do not meet the criteria for a major depressive episode. Bipolar disorder, previously known as manic–depressive disorder, is a condition in which depressive phases alternate with periods of mania or hypomania. Although depression is currently categorized as a"}, {"id": "pubmed23n0240_12918", "title": "Darvon dependence: three case studies.", "score": 0.009900990099009901, "content": "Several shared symptoms are evident among the subjects described here. First of all, each subject complained of feelings of depression, consistently and frequently. In other words, in each case, the subject described him/herself as \"being depressed.\" The detailed examination of the case notes kept by the counselors who treated these individuals indicates a more specific symptomology associated with their depression. On a more specific level, each of the subjects expressed feelings of worthlessness, hopelessness, despair, and self-destruction. In at least two of the cases, self-destructive feelings were translated into potentially suicidal actions. In addition, each of the three subjects experienced pronounced mood swings. Alternating between periods of deep depression (associated with stagnant or regressive behavior) and periods of relative optimism when at least temporary progress in therapy was evident. During these apparent periods of improvement, the subjects often related major plans of action intended to improve their lot, including new jobs, furthering their education, withdrawing entirely from drug use, patching up marital and family disorder, etc. In each case before these major improvements could be initiated, the subjects would relapse into depressed states and their plans would dissolve like so many fantasies. These cyclical mood swings and their accompanying polar manifestations would seem, superficially, indicative of a type of manic-depressive illness. It should be noted, however, that at no time did any of the subjects undergo thorough psychological testing. Such testing is planned for Subject B, who remains in treatment at this time. Each subject complained of experiencing acute anxiety attacks during his periods of depression.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en039_23366", "title": "Dysthymia", "score": 0.00980392156862745, "content": "In children and adolescents, mood can be irritable, and duration must be at least one year, in contrast to two years needed for diagnosis in adults. Early onset (diagnosis before age 21) is associated with more frequent relapses, psychiatric hospitalizations, and more co-occurring conditions. For younger adults with dysthymia, there is a higher co-occurrence in personality abnormalities and the symptoms are likely chronic. However, in older adults suffering from dysthymia, the psychological symptoms are associated with medical conditions and/or stressful life events and losses. Dysthymia can be contrasted with major depressive disorder by assessing the acute nature of the symptoms. Dysthymia is far more chronic (long lasting) than major depressive disorder, in which symptoms may be present for as little as 2 weeks. Also Dysthymia often presents itself at an earlier age than Major Depressive Disorder."}, {"id": "wiki20220301en112_43941", "title": "Death of John Carthy", "score": 0.00980392156862745, "content": "Background John Carthy, born 9 October 1972, was the only son of John and Rose Carthy. He had one sister, Marie, who was two years his junior. He was an avid Handballer and member of Abbeylara Handball Club. His father, with whom he was very close, died on 12 April 1990. In 1992 John was diagnosed with clinical depression and, subsequently with bipolar affective disorder. John's general employment was in the construction industry. He resided with his mother in an old three-bedroom house in Toneymore, Abbeylara. She and John were due to move from this old house to a new home that had been built on their land by Longford County Council as part of a rural housing scheme. The old house was due to be demolished. On 19 April 2000, the day prior to his death, John stayed in the family home all day with his mother. His mother's testimony to the Barr Tribunal indicated that John was very hostile to the move and that this was the primary topic of their discussions that day."}, {"id": "pubmed23n0853_1036", "title": "\"Is It Her Hormones?\": Psychiatric Diagnoses and Polycystic Ovarian Syndrome.", "score": 0.009708737864077669, "content": "Beth, whom you have cared for in your primary care practice since she was born, is a 15-year-old adolescent girl with no prior psychiatric history who developed significant symptoms of clinical depression, associated with self-injurious behavior (cutting on wrists, arms, and thighs). She denied any known precipitant for her depression.She is a ninth grade honors student in the gifted program at a local high school and is described as a talented musician, playing multiple musical instruments as well as soccer and basketball. She has good family support, was sociable, and had several close friends. She denied any history of trauma and denied ever using recreational drugs or other mood-altering substances.At this visit, she reported feeling \"sad and anxious.\" Family history was significant for maternal depression, which persisted through her teens and twenties. Her older sister had been diagnosed with Social Anxiety Disorder. Beth reported anhedonia, fatigue and irritable mood, lack of motivation, impaired concentration, and anxiety related to failing grades.You decide to begin medication because of the severity of her symptoms, and 1 week after starting fluoxetine 10 mg, she reportedly overdosed on an unknown quantity of acetaminophen. Within a few days of switching to escitalopram (due to persistent gastrointestinal complaints while taking fluoxetine), she developed homicidal ideation. She reported feeling grandiose, empowered, invincible, elated, and \"crazy,\" although she never demonstrated or endorsed psychotic symptoms. She became fixated upon the idea that she could kill someone and \"get away with it.\" At the time she tried to suffocate a peer with her hands, she was described as having \"a glazed over look in her eyes.\" Moods were now described as alternating between depressed and elated, with mood shifts occurring every few days. These symptoms did not improve after the antidepressant medication was discontinued.Subsequently, patient was admitted for acute psychiatric care, at which time she was described as depressed, but with an \"expansive and irritable\" mood, and with obsessive suicidal ideation. She had developed a plan to hang herself in the home. She started to believe that her mother was trying to give her \"poison.\" She reported panic attacks and said that she wanted to be in the hospital where she could feel \"safe.\" She claimed to have an \"entity inside of her body who was a bully\" and who was \"taking over her body\" and stated that he put her hand over her peer's mouth, as she watched.Psychological testing included the Minnesota Multiphasic Personality Inventory-Adolescent, showed significant paranoia, bizarre mentation, and poor reality testing. Along with interview and observation, it was determined that patient met criteria for the DSM-IV-TR clinical diagnosis of Other Specified Bipolar Disorder, with psychotic features.Aripiprazole was initiated at a dosage of 2 mg; however, moods were still described as fluctuating between extremes every few hours. It was discontinued after reaching 5 mg due to affective blunting. Risperidone 0.5 mg twice daily helped patient to feel and act \"more like herself\"; however, she continued to report significant depression. The addition of lamotrigine 25 mg daily, in addition to individual Dialectical Behavior Therapy, finally led to improvement of mood and a gradual return of her normal baseline, with reportedly stable emotional, social, and academic functioning.The patient's mother remained convinced that this adolescent's mood instability was caused by underlying hormonal problems so you refer her to endocrinology. Beth developed puberty at age 8, with menses occurring on average of twice yearly. She was found to have elevated free testosterone level of 8.6 (reference range, 1.2-7.5). She was of normal weight (body mass index = 21.85 kg/m) and did not manifest acne, male pattern hair thinning, or hirsutism. Thyroid functions, 17-OH progesterone, follicle-stimulating hormone/luteinizing hormone, and estradiol were within normal limits. Prolactin elevation (46.3) was assumed to be due to Risperidone. Patient refused ovarian ultrasound.After starting oral contraceptives to establish monthly menses, patient's emotional and behavioral symptoms continue to remain stable. After Beth decided on her own to discontinue psychotropic medications, she continued for 17 months following her initial visit to remain free of neuropsychiatric symptoms.Now that her symptoms seem resolved; you wonder what the medical diagnosis for Beth was? You wonder if \"hormones\" may have caused or contributed to her psychiatric presentation."}, {"id": "pubmed23n0532_15902", "title": "[Bipolarity among unipolar affective disorder patients--uniDEP-BI national multi-site study].", "score": 0.009708737864077669, "content": "The main objective of the study was to evaluate the occurrence of bipolarity among outpatients with a recurrent major depressive disorder. The uniDEP-BI study was conducted throughout Poland by 96 psychiatrists from 16 sites, who were trained in the study instruments. The sample was selected from the population of outpatients with at least one depressive episode (n = 880). The final study group included 246 working age adults (75.2% of females) treated for recurrent unipolar disorder. The study questionnaire consisted in the DSM-IV criteria of major depressive episode, (hypo)manic episode, criteria for bipolar spectrum disorder by Ghaemi et al. Unipolar mood disorder was confirmed in 32.9% cases, bipolar I disorder was found in 19.5%, bipolar II in 35% and bipolar spectrum disorder in 12.6% of the assessed patients. Patients with bipolar compared to the unipolar mood disorder had significantly more frequently a family history of bipolar disorder, short (hypo)manic episodes after antidepressive treatment, premorbid cyclo-, hyperthymic or impulsive (borderline type) personality, recurrent depressive episodes, atypical depressive symptoms, early onset of depression (< 25 yrs), distracted attention and panic attacks. Subjects with confirmed recurrent depressive disorder were significantly more often working (37.2% vs. 22.7%). Duration of the illness was significantly shorter and the number of previous depressive episodes was significantly lower in this group. The findings showed that the bipolarity features are more common among patients with unipolar mood disorders. It also points to a need of proper and deeper diagnostics of affective disorders and verification of rules and period of antidepressive and normothymic treatment."}, {"id": "wiki20220301en506_23519", "title": "Outline of bipolar disorder", "score": 0.009615384615384616, "content": "Bipolar spectrum – Bipolar I – bipolar disorder with at least one manic episode (with or without psychosis), possibly with hypomanic and/or depressive episodes as well Bipolar II – bipolar disorder with at least one depressive and at least one hypomanic episode, without any full mania Cyclothymia – \"mild\" bipolar disorder, with symptoms of hypomania and depression not severe enough to be classified as bipolar I or II Dysthymia – akin to depression, with symptoms that are long-lasting but less severe Major depressive disorder – a mood disorder involving low mood, low energy, poor self-esteem, lack of interest in enjoyable activities, and/or aches and pains Schizoaffective disorder – mood swings combined with psychosis; has subtypes bipolar type and depressive type Mania – a state of hyperactivity, heightened mood (euphoric or irritable), low sleep, pressured speech, grandiosity, and/or racing thoughts; may include psychotic features like delusions or hallucinations"}, {"id": "pubmed23n0106_1382", "title": "An 11-year follow-up study of 110 depressed patients.", "score": 0.009615384615384616, "content": "In 1972 the World Health Organization organized a cross-cultural five-centre study of depressive disorders. This report is concerned with data collected, after an 11-year follow-up period, in the sample of 110 depressed patients in Montreal, Canada. Eighty-five percent were traced and 59% were interviewed. Of 93 patients, 20 were dead at the follow-up date, 11 by suicide. Fifty-two percent of patients were receiving psychiatric treatment at follow-up, but there was no relation between psychiatric morbidity and treatment-seeking. Moderate or severe impairment of social functioning was present in 32%; in women, a trend linking the presence of social impairment and the time spent in episodes was observed. Of the episodes of psychiatric illness recorded after the index episode, 86% were diagnosed as depressive, 14% as unspecified affective disorder. The mean durations of the index and four subsequent episodes were 10, 11, 7, 11, and 2 months respectively. At least one recurrence after the index episode was reported by 78%, at least four recurrences by 19%. Episodes lasted at least one year in 5%, 4%, 6%, and 6% in the first, second, third and fourth episodes respectively. Sixteen percent were depressed for at least one year and 31% for at least 2 years. There was a marked trend from inpatient to outpatient treatment and from ECT to drug therapy over time. Twenty-two percent reported either moderate or severe problems with alcohol or substance abuse. There was a statistically significant association between the amount of time patients spent in depressive episodes and the number of life events they reported.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en302_12578", "title": "Cyclothymia", "score": 0.009523809523809525, "content": "Diagnosis Cyclothymia is classified in DSM-5 as a subtype of bipolar disorder. The criteria are: Periods of elevated mood and depressive symptoms for at least half the time during the last two years for adults and one year for children and teenagers. Periods of stable moods last only two months at most. Symptoms create significant problems in one or more areas of life. Symptoms do not meet the criteria for bipolar disorder, major depression, or another mental disorder. Symptoms are not caused by substance use or a medical condition."}, {"id": "pubmed23n0053_12484", "title": "[Triangular reflections on manic-depressive disorder. Apropos of 72 cases].", "score": 0.009523809523809525, "content": "In 72 cases of unipolarity or bipolarity depression followed at least three years the subsequent elements are questioned. What is the proportion of the different diagnostic types and what have been the most frequent life events of the first access, deaths, occupational and emotional failures, pregnancy, births, etc? In this population of patients between 23 and 80 years old reluctance or ignorance of the life events are important. We also investigated the level of our patients socio-professional insertion. As for the hetero ou auto aggressive acting out we tried to quantify them according to the chosen classification and the therapy followed by the patients of this group. In the hypothesis of a possible link with the hospital management evaluation and computer program we have briefly analyzed the incidence of lithium therapy on the number of hospitalisation days, without being able to draw a final conclusion as to the interest of this therapy in this very specific issue."}, {"id": "pubmed23n1017_5978", "title": "When to Raise Our White Flag-A Discussion of Scope of Practice in a Resource Scarce World.", "score": 0.009433962264150943, "content": "Thomas is a 13-year-old boy with autism spectrum disorder, attention deficit hyperactivity disorder (ADHD), generalized anxiety disorder, separation anxiety disorder, and major depressive disorder who presented for a follow-up to his developmental and behavioral pediatrician (DBP). His mother describes an increase in symptoms of anxiety and depression for the last 6 weeks, accompanied by suicidal ideation and thoughts of self-mutilation.Before this increase in symptoms, he had been doing well for the last several months with the exception of increasing weight gain, and Abilify was decreased from 5 mg to 2.5 mg at his last visit. Other medications at that time included Zoloft 100 mg twice daily, Focalin XR 40 mg every morning, and Focalin 5 mg every night. Without seeking the guidance of our developmental and behavioral pediatrics clinic, his mother increased his intake of Zoloft to 150 mg each morning and continued 100 mg each evening because of worsening anxiety and depression.Religion is very important to Thomas and his family. He acknowledges that he does not want to die and feels badly because \"suicide is against our religion.\"Helping Thomas receive appropriate care has been a challenge. He was diagnosed with ADHD and Asperger disorder at the age of 5. Thomas is homeschooled and is very attached to his mother. His parents have very different parenting styles, with his mother being more permissive and his father more authoritarian. At the time of initial diagnosis, the behavioral health services (BHS) in Thomas' community, which is about an hour away from the DBP, were limited to older children, and he was followed by a DBP for ADHD medication management. At the age of 11, he expressed passive suicidal ideation and described that he imagined his mother as \"the devil with fire coming out of her eyes\" when she corrected him. He was evaluated by BHS, diagnosed with anxiety disorder, and started on Lexapro. BHS linked to the DBP were out of network for his insurance. The family was unable to pay out of pocket, so care was subsequently transferred to a DBP clinic that was in network. Soon after, Thomas developed auditory hallucinations, and Abilify was added after consultation with BHS.Over the last few years, Thomas' symptoms have waxed and waned. He did well for a short time and then again developed auditory hallucinations, worsening symptoms of anxiety and depression, and increasing somatic symptoms including vomiting and penile pain. Medications were adjusted with input from BHS, and further attempts were made to link him to local BHS but were unsuccessful. With his current concerns of suicidal ideation and self-mutilation, what would be your next steps?"}, {"id": "pubmed23n1105_12868", "title": "Dienogest-induced major depressive disorder with suicidal ideation: A case report.", "score": 0.009345794392523364, "content": "Dienogest is a type of progestin used for the treatment of endometriosis (EM). However, a significant adverse effect of dienogest is depression; therefore, assessing for a history of mood disorders is recommended before prescribing the drug. Herein, we present the case of a patient with no history of psychiatric disorders who was diagnosed with dienogest-induced major depressive disorder. This case emphasizes the importance of close monitoring for negative mood changes in patients taking dienogest. A 41-year-old woman underwent surgery for EM. Postoperatively, her gynecologist prescribed dienogest (2 mg/d) to control EM symptoms. Two months after the initiation of dienogest, she manifested insomnia almost daily, gradually became depressed, lost interest in all activities, had incessant cries, and repeatedly thought of death. She had no history of major physical or psychiatric disorders. Major depressive disorder, single episode, severe. A psychiatric consultation was recommended, an antidepressant was prescribed, and dienogest was discontinued. Two weeks later, there was significant improvement in the symptoms, and after 4 weeks, she remained in a stable mood with no suicidal thoughts. She was followed up for 13 months with a maintenance dose of escitalopram (5 -10mg/d), until the psychiatrist recommended treatment discontinuation, with a confirmed state of remission. This was a case of dienogest-induced depression in a patient with no history of mood disorders. Clinicians should be aware of the possibility of the occurrence of severe depression in progestin users regardless of their previous history."}, {"id": "pubmed23n0353_1801", "title": "Late-life chronic depression: a 399-case study in private practice.", "score": 0.009345794392523364, "content": "Aims of the study were to find the prevalence of chronic depression in elderly patients compared with younger patients, and to compare chronic depression between elderly and younger patients, to find if there were clinical differences. A major feature of the study was the inclusion of a large number of bipolar II patients, usually not included in previous studies. Three hundred and ninety-nine consecutive unipolar (N=200) and bipolar II (N=199) depression outpatients were interviewed with the Structured Clinical Interview for DSM-IV and depression rating scales in a private practice. Chronic depression was more common in elderly patients than in younger patients (53.6% vs 40.1%, p=0.0299). Late-life chronic depression patients had later age at onset, longer duration of illness, fewer bipolar II cases, more unipolar cases and more relapses than younger chronic depression patients. Results suggest that late-life depression is more likely to be chronic than depression in younger patients. The subtyping of chronic depression according to age seems supported by a different age at onset and some clinical differences."}, {"id": "wiki20220301en302_12571", "title": "Cyclothymia", "score": 0.009259259259259259, "content": "Cyclothymia, also known as cyclothymic disorder, is a mental and behavioural disorder that involves numerous periods of symptoms of depression and periods of symptoms of elevated mood. These symptoms, however, are not sufficient to indicate a major depressive episode or a manic episode. Symptoms must last for more than one year in children and two years in adults. The cause of cyclothymia is unknown. Risk factors include a family history of bipolar disorder. Cyclothymia differs from bipolar in that major depression and mania are not found. Treatment is generally achieved with counseling and mood stabilizers such as lithium. It is estimated that 0.4-1% of people have cyclothymia at some point in their life. The disorder's onset typically occurs in late childhood to early adulthood. Males and females are affected equally often."}, {"id": "pubmed23n0570_15021", "title": "'Interrupted by fits of weeping': Cicero's major depressive disorder and the death of Tullia.", "score": 0.009259259259259259, "content": "The letters of Marcus Tullius Cicero (106-43 Bc), the Roman statesman, lawyer, orator and author, were analysed as part of a larger study that systematically examined ancient Greek and Roman literature to recover descriptions of mental illness. A degree of necessary caution was exercised, but the wealth of material revealed in the letters about Cicero's physical and emotional state enable a diagnosis of Major Depressive Disorder to be made with some certainty according to the DSM-IV-TR. Cicero appears to have experienced increasingly severe bouts of suicidal depression that seriously impaired his relationships with his friends, family and political colleagues, and possibly shortened his life. His last depressive episode following the death of his daughter Tullia is addressed here in some detail."}, {"id": "wiki20220301en189_14661", "title": "Bipolar II disorder", "score": 0.00909090909090909, "content": "BP-II is diagnosed according to the criteria established in the American Psychiatric Association's Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5). In addition, alternative diagnostic criteria is established in the World Health Organization's International Classification of Diseases-10th Revision (ICD-10). The diagnostic criteria are established from self-reported experiences from patients or their family members, the psychiatric assessment, and the mental status examination. In addition, Screening instruments like the Mood Disorders Questionnaire are helpful tools in determining a patient's status on the bipolar spectrum. In addition, certain features have been shown to increase the chances that depressed patients have a bipolar disorder, including atypical symptoms of depression like hypersomnia and hyperphagia, a family history of bipolar disorder, medication-induced hypomania, recurrent or psychotic depression, antidepressant refractory depression, and"}, {"id": "pubmed23n0422_504", "title": "[Suicide attempts in elderly people. Data from the Hospital Italiano de Buenos Aires].", "score": 0.00909090909090909, "content": "[corrected] The aim of this paper is to analyze some characteristics of 25 patients, over 60, who attempted suicide and were hospitalized in the Department of Psychiatry of a general hospital in Buenos Aires. This is a retrospective study using data from the medical records of patients (1999 to 2002); based on a protocol comprising an analysis of attempted suicides, demographic and clinical variables. Patients were diagnosed following the DSM-IV criteria by two trained GPs and were confirmed by MMPI and Rorschach. 72% were women. The average age was 73,8 % were divorced, 20% never married, 32% widowed and 32% married. The most frequent diagnosis was Major Depressive Disorder of late onset followed by Personality Disorder ( 96%; 48%). The most frequent method was intoxication, (68.75% BDZ). Almost half of the attempts were highly severe. We may infer that the elderly person who attempts suicide: is female, 68-78 years old, does not have a partner, lives with someone, has Major Depressive Disorder (at least half of them had Personality Disorder) and a clinical disease. This is her first attempt, is alone at home, and she does not advise others. Reduced hearing is a very frequent co-morbidity."}, {"id": "wiki20220301en028_34785", "title": "Depression (mood)", "score": 0.009009009009009009, "content": "A number of psychiatric syndromes feature depressed mood as a main symptom. The mood disorders are a group of disorders considered to be primary disturbances of mood. These include major depressive disorder (MDD; commonly called major depression or clinical depression) where a person has at least two weeks of depressed mood or a loss of interest or pleasure in nearly all activities; and dysthymia, a state of chronic depressed mood, the symptoms of which do not meet the severity of a major depressive episode. Another mood disorder, bipolar disorder, features one or more episodes of abnormally elevated mood, cognition, and energy levels, but may also involve one or more episodes of depression. When the course of depressive episodes follows a seasonal pattern, the disorder (major depressive disorder, bipolar disorder, etc.) may be described as a seasonal affective disorder."}, {"id": "pubmed23n0043_12531", "title": "[Selected demographic and clinical characteristics of patients with affective disorder treated in the Tworki Hospital in the years 1919- 1938 and 1947-1990].", "score": 0.009009009009009009, "content": "On the basis of medical records of 353 patients with affective disorder from the years 1919-1938 and 1947-1990 it was found that some demographic and clinical changes occurred. Amongst others there was observed a rise in the percentage of patients with depression from 1/3 to 2/3 and a fall in the number of patients suffering from mania."}, {"id": "wiki20220301en002_146953", "title": "Mood disorder", "score": 0.008928571428571428, "content": "Bipolar I is distinguished by the presence or history of one or more manic episodes or mixed episodes with or without major depressive episodes. A depressive episode is not required for the diagnosis of Bipolar I Disorder, but depressive episodes are usually part of the course of the illness. Bipolar II consisting of recurrent intermittent hypomanic and depressive episodes or mixed episodes. Cyclothymia is a form of bipolar disorder, consisting of recurrent hypomanic and dysthymic episodes, but no full manic episodes or full major depressive episodes. Bipolar disorder not otherwise specified (BD-NOS), sometimes called \"sub-threshold\" bipolar, indicates that the patient has some symptoms in the bipolar spectrum (e.g., manic and depressive symptoms) but does not fully qualify for any of the three formal bipolar DSM-IV diagnoses mentioned above."}, {"id": "pubmed23n0068_14392", "title": "A retrospective case-note study of bipolar disorder in old age.", "score": 0.008928571428571428, "content": "In a replication of an earlier published study, case notes of 75 elderly in-patients with bipolar affective disorder were examined. Few of the patients had experienced a manic episode before the age of 40. Mean age of onset of affective disorder was 46 years, and first manic episode at 60 years. Cerebral insults before the first manic attacks were recorded in a substantial number of cases, and a family history of mental illness was less common among this group. Bipolar affective disorder is relatively common as a reason for admission of elderly patients."}, {"id": "pubmed23n0386_15020", "title": "[Late onset first manic episode: role of lithium].", "score": 0.008849557522123894, "content": "This case reports on a first manic episode occurring to a 68 year old patient. Until now this male patient had been diagnosed with a recurrent depressive disorder. This depressive illness started 29 years ago and was punctuated by several depressive episodes. During one of these episodes associated to psychotic features, following the lithium discontinuation, the patient committed a homicide-suicide. He was found not to be responsible for his crime and treatment was subsequently restarted. For the next fifteen years the patient was stabilized using the association of antidepressant plus lithium, then lithium alone. Until the current manic episode subsequent to a further lithium discontinuation, the patient thanks to the mood stabilizer, could enjoy a good quality of life with a very satisfying social and professional adjustment. Following the case report, an analysis of published data on epidemiological parameters and risk factors associated was conducted. Findings show that perpetrators of murder suicides are mainly males (> 85%) suffering from depression (40% to 75%). Cases of homicide-suicide more frequently involve individuals committing a violent suicide shortly after (minutes or hours) committing one or more homicides. These cases often occur within a disturbed family context with drugs or alcohol abuse, social or cultural stresses such as poor social level or unemployment being other risk factors. Fire-arms are the most frequently used in suicides. Reported annual incidence is similar every year, ranging from 0.2 to 0.3 per 100,000 in the United States and other countries. Due to suicide but also to other disorders, mortality and morbidity rates are higher with patients suffering from mood disorders. A long term treatment with lithium results in a decrease of morbidity rate and suicidal risk in the general population."}, {"id": "wiki20220301en039_23356", "title": "Dysthymia", "score": 0.008771929824561403, "content": "\"At least three-quarters of patients with dysthymia also have a chronic physical illness or another psychiatric disorder such as one of the anxiety disorders, cyclothymia, drug addiction, or alcoholism\". Common co-occurring conditions include major depression (up to 75%), anxiety disorders (up to 50%), personality disorders (up to 40%), somatoform disorders (up to 45%) and substance use disorders (up to 50%). People with dysthymia have a higher-than-average chance of developing major depression. A 10-year follow-up study found that 95% of dysthymia patients had an episode of major depression. When an intense episode of depression occurs on top of dysthymia, the state is called \"double depression.\" Double depression"}, {"id": "pubmed23n0612_282", "title": "[Clinical particularism of bipolar disorder: unipolar mania. About a patient's study in Tunesia].", "score": 0.008771929824561403, "content": "Although present classifications (CIM, DSM) have not included the notion of a unipolar disorder to characterise the recurrence of the same type of episode, this concept conserves its pertinence for many people. Unipolar mania, in particular, is a clinical reality in our daily practice, and a predominant form of bipolarity expression. These assertions have led us to question this notion and its nosographical place: is it a subtype, distinguished by certain characteristics, or a particular category in the bipolar disorder? We conducted a retrospective, descriptive and comparative study on medical briefs of patients with type I bipolar disorder (DSM-IV criteria), who were interned for the first time between 1997 and 2001 in the Psychiatry \"E\" service of the Razi hospital of Tunis, and were followed up for at least five years. Two groups were identified: Group 1 or \"unipolar mania\": patients who presented at least two manic episodes without depression, and Group 2: the rest of the sample; and then were compared based on their sociodemographical profile, familial psychiatric antecedents, premorbid temperament, comorbidity and clinical and progressive characteristics. Seventy-two patients were included. The average age was 36. The sex ratio was three men to two women. The first episode was a manic episode in 56.9% of the cases. The average duration of illness progression was 11.6 years. Unipolar mania represented 65.3% of the sample. Between 1997 and 2001, 92% of bipolar patients interned were hospitalised for mania. Concerning recurrences, we observed nine times as many cases of manic episodes as depression. Depressive episodes of light to medium intensity had probably not been well assessed due to the families' tolerance. The high rates of both manic episodes and unipolar mania observed in this study were also found by other authors, showing the differences of bipolarity expression between the West and the other parts of the world, and in particular Africa. There was no significant difference concerning the sociodemographical features. We noticed similar results in the literature. The two groups were comparable in familial psychiatric past history and premorbid temperament. Substance abuse or dependence was observed in 5.6% of the patients. This rate was less than others found in the literature, due to the fact that it is considered as an offence in our country. We found twice as many cases of toxic consumption in bipolar as in unipolar manic patients. A recent Tunisian study has shown the absence of substance abuse in unipolar manic patients. This is probably because of the fact that substance abuse is more related to depressive manifestations. The sample starting age was 24.6 years and was significantly more precocious in the unipolar manic group (27.6 years versus 23 years, p=0.001). A significant difference in both groups was found concerning the first episode season: two extremities were observed: \"summer-autumn\" in Group 1 (63.6% G1 versus 29.4% G2) and \"winter-spring\" in Group 2 (73.6% G2 versus 36.4% G1), p=0.03. The seasonal influence on mood disorders is dealt with by other authors. Unipolar manic patients presented less affective recurrences than the rest of the group (0.37 versus 0.49 on average per year), p=0.056. Unipolar mania is still considered as a clinical variety of bipolar disorder, which is distinguished by certain features. It is a debated notion because it is based on retrospective studies that may be insufficient, although it appears as a clinical evidence and a predominant progressive variety of bipolar disorder in Tunisia."}, {"id": "wiki20220301en223_8342", "title": "Minor depressive disorder", "score": 0.008695652173913044, "content": "Minor depressive disorder differs from major depressive disorder in the number of symptoms present with 5 or more symptoms necessary for a diagnosis of major depressive disorder. Both disorders require either depressed mood or loss of interest or pleasure in normal activities to be one of the symptoms and the symptoms need to be present for two weeks or longer. Symptoms also must be present for the majority of the length of a day and present for a majority of the days in the two-week period. Diagnosis can only occur if the symptoms cause \"clinically significant distress or impairment\". Dysthymia consists of the same depressive symptoms, but its main differentiable feature is its longer-lasting nature as compared to minor depressive disorder. Dysthymia was replaced in the DSM-5 by persistent depressive disorder, which combined dysthymia with chronic major depressive disorder."}, {"id": "pubmed23n0114_5870", "title": "Comparison of depressed patients with and without suicide attempts in their past history.", "score": 0.008695652173913044, "content": "Forty-eight inpatients with the diagnosis of a Brief or Prolonged Depressive Reaction according to ICD-9 who had attempted suicide just before the admission were compared to 24 inpatients with the same diagnosis but no history of previous suicide attempts. The variables investigated included sociodemographic characteristics, family history, life events within the year prior to admission, social functioning, social support, and personality factors. The comparison of these two groups revealed that alcoholism and suicide attempts in first degree relatives, and divorce or separation of the patients' parents predispose for a depressive reaction associated with the suicidal behavior. No differences between the two patient groups were found for personality factors, number and quality of life events in the year before index admission, social functioning, and social support during the last 4 weeks. Due to the small number of patients in both groups the conclusions drawn are preliminary."}, {"id": "wiki20220301en423_14949", "title": "List of medical mnemonics", "score": 0.008620689655172414, "content": "Indiscretion (DSM-IV's \"excessive involvement in pleasurable activities\") Grandiosity Flight of ideas Activity increase Sleep deficit (decreased need for sleep) Talkativeness (pressured speech) Mania: diagnostic criteria Must have 3 of MANIAC: Mouth (pressure of speech)/ Moodl Activity increased Naughty (disinhibition) Insomnia Attention (distractibility) Confidence (grandiose ideas) Parasomnias: time of onset Sleep terrors and Sleepwalking occur during Slow-wave sleep (stages 3 & 4).Nightmare occurs during REM sleep (and is remembered). Psychiatric review of symptoms \"Depressed patients seem anxious, so call psychiatrists\": Depression and other mood disorders (major depression, bipolar disorder, dysthymia) Personality disorders (primarily borderline personality disorder) Substance abuse disorders Anxiety disorders (panic disorder with agoraphobia, obsessive-compulsive disorder)"}]}}}} {"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[370, 564]], "word_ranges": [[60, 87]], "text": "Microsporum gypseum does not usually manifest dissemination of infection of a lymphangitic aspect, in general keratinophilic fungi (dermatophytes) do not have any ability to invade deep tissues,"}, "2": {"exist": true, "char_ranges": [[594, 835]], "word_ranges": [[93, 130]], "text": "Staphylococcus aureus infection can affect superficial and deep tissue layers, they can spread following a lymphangitic path, but it is not usual such a long evolution, it is usually acute (days) and usually accompanied by systemic symptoms,"}, "3": {"exist": true, "char_ranges": [[1969, 2210]], "word_ranges": [[316, 355]], "text": "Patients are usually infected by exposure to soil by means of a sharp object (thorns, stems, animal claws, etc.) and have a slow evolution, affect deep tissues and their dissemination typically follows a pathway as described in the question."}, "4": {"exist": true, "char_ranges": [[1393, 1627]], "word_ranges": [[220, 261]], "text": "Our patient has no history of exposure to fish or water (they don't say), so considering that usually the diagnosis is based on a high index of suspicion and specifically asking for this history, our case is far from this possibility."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The patient carries out an activity in contact with soil and plants (which are not provided free of charge). The symptoms are chronic, which started distally (left thumb) and are affecting the ipsilateral arm (reddish striae). There were no systemic symptoms. They also comment that the diagnosis was made on the basis of a skin biopsy. Of the microorganisms discussed, Microsporum gypseum does not usually manifest dissemination of infection of a lymphangitic aspect, in general keratinophilic fungi (dermatophytes) do not have any ability to invade deep tissues, so we ruled out this option. Staphylococcus aureus infection can affect superficial and deep tissue layers, they can spread following a lymphangitic path, but it is not usual such a long evolution, it is usually acute (days) and usually accompanied by systemic symptoms, so we discard this option. Mycobacterium marinum (and not Mycobacterum marinum) is a non-tuberculous mycobacterium characterized by being environmental, opportunistic and photochromogenic. It has a slow growth, from 2 to 8 weeks, at a temperature ranging from 30 to 37 °C. It affects different species of fish in cold, warm, fresh or salt water, especially stagnant waters of fish tanks and pools without chlorine. Infection in humans occurs by direct contact with fish or contaminated water in the presence of a loss of continuity in the skin of the host. Our patient has no history of exposure to fish or water (they don't say), so considering that usually the diagnosis is based on a high index of suspicion and specifically asking for this history, our case is far from this possibility. The Sporothrix schenckii complex is the causative agent of Sporotrichosis, this dimorphic fungus is located in the soil, the complex is formed by 5 species, S. schenckii sensu strictu, S. brasiliensis and S. globosa, capable of causing human and animal disease; the other two are S. mexicana and S. albicana not associated with any disease. Patients are usually infected by exposure to soil by means of a sharp object (thorns, stems, animal claws, etc.) and have a slow evolution, affect deep tissues and their dissemination typically follows a pathway as described in the question.", "full_answer_no_ref": "The patient carries out an activity in contact with soil and plants (which are not provided free of charge). The symptoms are chronic, which started distally (left thumb) and are affecting the ipsilateral arm (reddish striae). There were no systemic symptoms. They also comment that the diagnosis was made on the basis of a skin biopsy. Of the microorganisms discussed, Microsporum gypseum does not usually manifest dissemination of infection of a lymphangitic aspect, in general keratinophilic fungi (dermatophytes) do not have any ability to invade deep tissues, so [HIDDEN]. Staphylococcus aureus infection can affect superficial and deep tissue layers, they can spread following a lymphangitic path, but it is not usual such a long evolution, it is usually acute (days) and usually accompanied by systemic symptoms, so [HIDDEN]. Mycobacterium marinum (and not Mycobacterum marinum) is a non-tuberculous mycobacterium characterized by being environmental, opportunistic and photochromogenic. It has a slow growth, from 2 to 8 weeks, at a temperature ranging from 30 to 37 °C. It affects different species of fish in cold, warm, fresh or salt water, especially stagnant waters of fish tanks and pools without chlorine. Infection in humans occurs by direct contact with fish or contaminated water in the presence of a loss of continuity in the skin of the host. Our patient has no history of exposure to fish or water (they don't say), so [HIDDEN]. The Sporothrix schenckii complex is the causative agent of Sporotrichosis, this dimorphic fungus is located in the soil, the complex is formed by 5 species, S. schenckii sensu strictu, S. brasiliensis and S. globosa, capable of causing human and animal disease; the other two are S. mexicana and S. albicana not associated with any disease. Patients are usually infected by exposure to soil by means of a sharp object (thorns, stems, animal claws, etc.) and have a slow evolution, affect deep tissues and their dissemination typically follows a pathway as described in the question.", "full_question": "A 58-year-old man reported a weeks-long history of progressive, mildly painful skin lesions on his left arm. It had started as an erythematous lesion on his left thumb. He had visible reddish striae as connecting lines between the lesions. The patient had no fever or other general symptoms. He had been working in his garden but did not remember any injuries. The etiologic diagnosis was made by culture of a skin biopsy. What is the most likely causative agent of this process?", "id": 297, "lang": "en", "options": {"1": "Dermatophytosis by Microsporum gypseum.", "2": "Cutaneous infection by Staphylococcus aureus.", "3": "Sporotrichosis.", "4": "Cutaneous infection by Mycobacterium marinum.", "5": NaN}, "question_id_specific": 50, "type": "INFECTIOUS DISEASES", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "article-29407_8", "title": "Sporotrichosis -- History and Physical", "score": 0.0105491063202506, "content": "Sporotrichosis has an incubation period of several days to 3 months after exposure. Infections can be divided into several syndromes including cutaneous, pulmonary, and disseminated. Cutaneous syndrome arises from minor trauma of the fungus into the skin, which is most common in rose gardeners. The initial lesions are erythematous papulonodular lesions that may be smooth or verrucous and involve lymphatic channels. [4] Lesions are typically painless even after ulceration. In this syndrome, the patient will not have systemic symptoms, and laboratory exams will be normal."}, {"id": "pubmed23n0541_18283", "title": "Disseminated sporotrichosis mimicking sarcoidosis.", "score": 0.009900990099009901, "content": "A 40-year-old Caucasian man presented to the dermatology clinic at Baylor College of Medicine, Houston, Texas, in February 2003, for the evaluation of three nonhealing ulcers. The patient's past medical history was significant for hypothyroidism and pulmonary sarcoidosis, the diagnosis of which was made in June 2000. In March 2000, the patient had complained of cough and shortness of breath. A purified protein derivative (PPD) (Mantoux text) was negative. Computed tomography (CT) scans of the chest revealed diffuse hilar and mediastinal adenopathy and bilateral interstitial and alveolar infiltrates. Although consistent with sarcoidosis, these findings were insufficient to exclude other etiologies, including disseminated fungal infection. Cultures and stains of subsequent bronchoscopy specimens failed to reveal any organisms, and histopathologic evaluation of the specimens was nondiagnostic. Based on the imaging studies and the negative cultures, a diagnosis of sarcoidosis was made, and the patient was started on therapy with prednisone. Before coming to our clinic, the patient had been on several courses of prednisone. In May 2002, the patient had presented to a private dermatologist with a 1-year history of a nonhealing 2.4 cm x 2.0 cm ulcer on the left medial forearm. Two biopsies were reported as nondiagnostic. The patient's presentation was interpreted as most consistent with Mycobacterium marinum infection, and so he was empirically treated with minocycline. This treatment was continued for almost 3 months without improvement in the ulcer. A few months after the minocycline had been discontinued, the patient was treated empirically for 2 months with ciprofloxacin. This treatment was also unsuccessful in ameliorating the ulcer. In between the two courses of antibiotics, specimens from the lesion were sent for bacterial and fungal cultures, which revealed normal skin flora. In January 2003, the patient returned to his private dermatologist with three ulcerations. In addition to the nonhealing ulcer on his left forearm, which he had acquired several months earlier, he had also developed a 3.0 cm ulcer on his right arm and a 3.0 cm ulcer on his central back. The patient refused biopsies at this visit. Given the patient's previous diagnosis of pulmonary sarcoidosis, it was thought that the skin lesions might represent ulcerative cutaneous sarcoidosis. Pyoderma gangrenosum was also considered to be a likely diagnosis. Therefore, the patient was started on a course of oral prednisone, an effective therapy for both sarcoidosis and pyoderma gangrenosum. Despite 1 month of treatment with 60 mg/day of prednisone, the ulcers increased, and the patient was subsequently referred to our clinic. Physical examination at the time of presentation revealed steroid acne on the trunk and upper extremities and three non-tender ulcers with erythematous, undermined borders (Figs 1-3). On the left arm, there was an adjacent nodule which the patient attributed to a scar from a previously healed ulcer. Histologic examination of biopsy specimens from all three sites showed similar findings. The lesion contained diffuse, suppurative, granulomatous, inflammatory infiltrates with extensive central necrosis. The infiltrates were composed of histiocytes, multinucleated foreign-body-type giant cells, plasma cells, lymphocytes, neutrophils, and neutrophil fragments. No organisms were seen in the initial, routinely stained sections. However, periodic acid-Schiff (PAS) staining demonstrated small fungal spores (Fig. 4) morphologically consistent with sporotrichosis, within the cytoplasm of multinucleated histiocytic giant cells (Fig. 5). Additional stains for bacteria and acid-fast organisms were negative. Cultures of the biopsy specimens from all three sites grew Sporothrix schenckii. Further questioning of the patient failed to reveal an obvious source of the infection. The patient denied any history of traumatic skin inoculation and did not engage in gardening or other outdoor activities that are classically associated with sporotrichosis. The patient did admit to blackberry picking on detailed retrospective questioning. Once the diagnosis of sporotrichosis was made, the patient was given 200 mg/day of itraconazole. After 2 months, the patient's ulcers were almost completely healed. The patient's pulmonary complaints were also much improved."}, {"id": "article-22848_8", "title": "Herpetic Whitlow -- History and Physical", "score": 0.009900990099009901, "content": "Patients will often experience pain and tingling in the finger before any skin changes (prodromal phase). [12] This will be followed by local tenderness, erythema, and edema with an initial crop of vesicles which are most common along the pulp and lateral aspect of the finger. The infection usually involves just one finger but has rarely been noted to involve several fingers. Vesicles usually coalesce into large, honeycomb-like bullae. They may spread proximally and may involve the nail bed where hemorrhagic or purpuric lesions may be noted. Patients will often report a disproportionate intensity of pain, particularly if there is nailbed involvement. [2] Fever, lymphadenitis, epitrochlear and axillary lymphadenopathy may be present. [13] Rarely, lymphedema of the hand and forearm may be present, although reports favor bacterial superinfection in most of these cases."}, {"id": "pubmed23n1018_23596", "title": "Dermoscopy of Mycobacterium marinum Skin infection: A Challenging Diagnosis.", "score": 0.00980392156862745, "content": "Dear Editor, Mycobacterium (M.) marinum is a slow-growing atypical mycobacterium found mainly in saltwater environments. Infection occurs following inoculation of a skin lesion and manifests as a localized granuloma; in fact, the most common cause of infection with M. marinum is the exposure of traumatized skin to affected aqueous environments (1), and it most commonly involves individuals with occupational and recreational exposure to non-chlorinated water (2). An erythematous or bluish 0.5 to 3.0 cm nodule usually develops at the inoculation site, while ulceration can occur later and subsequent lesions may be present along the lymphatic drainage. We present the first case in the literature describing the dermatoscopic characteristics of a microbiologically proven Mycobacterium marinum skin infection, although more cases are certainly needed to identify the main dermatoscopic features of this infection. In January 2019, a 66-year-old patient was referred to our Dermatological Clinic reporting the appearance of two purplish nodules about 2 months earlier, located on the back of the hand and on the left thumb (Figure 1) and of erythematous purplish appearance and quite painful to palpation. Based on the clinical presentation, infection with atypical mycobacteria, botryomycosis, fungal infection (Cryptococcus neoformans, Histoplasma capsulatum) and infection with Francisella tularensis were considered in the differential diagnosis. The patient was asked if he had an aquarium at home and he confirmed this by telling us of his passion for aquariums, which made the diagnosis easier. Dermatoscopic examination performed on the two lesions at the center of the first nodule located on the hand showed a whitish area surrounded by an erythematous background with fine scaling and dotted vessels and orange-whitish central areas with looped concentric monomorphic vessels (Figure 2, a). However, the thumb lesion had a purplish background with multiple structured rounded areas with orangish appearance surrounded by looped vessels arranged in a crown-like shape (Figure 2, b). It is interesting to note the dermoscopic-histological correlation in this disease: the orangish areas in fact correspond to a granulomatous dermatitis, characterized by inflammatory nodular infiltrate within the dermis (tuberculoid granulomas) (3). Clinically and dermoscopically, the nodules had two different sets of features because they were in different stages of development: the nodule of the thumb was older than the other one on the hand, which the patient reported was of recent onset, also confirmed by the presence of suppuration and ulceration. Both lesions had orange areas in the context of an erythematous background which led us to investigate a granulomatous disease. A deep culture examination and a skin biopsy were thus performed, showing Mycobacterium marinum infection. Oral therapy with clarithromycin 500 mg twice daily for 4 weeks was started and healing occurred in about 21 days. We present this case to emphasize the role of dermoscopy in differential diagnosis of granulomatous disease and to show dermoscopic clues that have not yet been described and that can be used in the future to establish very early diagnosis of this infection, reducing the diagnostic delay."}, {"id": "InternalMed_Harrison_10340", "title": "InternalMed_Harrison", "score": 0.00980392156862745, "content": "(Table 156-1) Impetigo contagiosa is caused by S. pyogenes, and bullous impetigo is due to S. aureus. Both skin lesions may have an early bullous stage but then appear as thick crusts with a golden-brown color. Epidemics of impetigo caused by MRSA have been reported. Streptococcal lesions are most common among children 2–5 years of age, and epidemics may occur in settings of poor hygiene, particularly among children in lower socioeconomic settings in tropical climates. It is important to recognize impetigo contagiosa because of its relationship to poststreptococcal glomerulonephritis. Rheumatic fever is not a complication of skin infection caused by S. pyogenes. Superficial dermatophyte infection (ringworm) can occur on any skin surface, and skin scrapings with KOH staining are diagnostic. Primary infections with dimorphic fungi such as Blastomyces dermatitidis and Sporothrix schenckii can initially present as crusted skin lesions resembling ringworm. Disseminated infection with"}, {"id": "wiki20220301en422_28458", "title": "Microsporum gallinae", "score": 0.009708737864077669, "content": "Humans Microsporum gallinae has been isolated from the scalp, and smooth skin in human populations. Microsporum gallinae infections are most commonly tinea capitis and tinea corporis. Very few human cases of M. gallinae infection have been reported, none of which were life-threatening. Of the reported cases, individuals ranged from 3–96 years old. They had cutaneous lesions on the glabrous skin or the scalp. These localized lesions are frequently accompanied by itching. The cutaneous manifestations are very similar to those of Microsporum canis therefore many cases of Microsporum gallinae could have been unreported. In rare cases, immunocompromised individuals form severe dissemination on the skin, instead of small localized lesions as a result of handling infected animals. Only one case of extensive dermatophytosis was reported involving M. gallinae infection of a person with AIDS."}, {"id": "pubmed23n0567_16074", "title": "A rose by any other name.", "score": 0.009708737864077669, "content": "A 40-year-old man visited Haiti in the winter. His visit was uneventful, and he went swimming in the ocean. A week after his return he developed a small \"pimple\" on his right fifth finger. This condition progressed for several weeks, with new lesions developing over the extensor surface of his forearm and in the antecubital fossa. He had tender axillary adenopathy. The patient started a new job when he returned from Haiti, working 4 days per week in a greenhouse. He denied any fever, chills, or night sweats. He was in good health without any underlying chronic health problems. Physical examination revealed a small eschar over the distal phalanx of the patient's right fifth finger (Figure 1). There were 2 erythematous nodules over the extensor surface of his right forearm (Figure 2) as well as over the antecubital fossa (Figure 3). A punch biopsy was performed and results showed suppurative granulomatous dermatitis. Sporothrix schenckii was grown from the specimen."}, {"id": "pubmed23n0697_18550", "title": "An unusual cutaneous manifestation of Crohn's disease.", "score": 0.009615384615384616, "content": "A 61-year-old man with a 12-year history of quiescent Crohn's disease on mesalamine presented to his gastroenterologist in April 2009, complaining of abdominal cramping, diarrhea, and a 25-lb weight loss over 6 weeks. He did not respond to prednisone 50 mg and 6-mercaptopurine 100 mg daily. Abdominal computed tomography findings revealed diffuse submucosal edema consistent with extensive colitis. Colonoscopy demonstrated diffuse inflammation with erythema, friability, and shallow ulcerations in the rectum and colon. Biopsies were consistent with Crohn's colitis. He was admitted for infliximab infusion for his unremitting diarrhea. Five days before admission, the patient noted mild swelling and redness of the left lower eyelid, which progressed to involve the right lower eyelid with frank pus draining from both eyes. He had no visual impairment or eye pain. Two days before admission, an ophthalmologist prescribed a steroid eyedrop with no relief. He also complained of seropurulent painful skin lesions on his face and scalp, which spread to involve his upper trunk and proximal arms. On admission to the hospital, dermatology, ophthalmology, and infectious disease consultations were obtained to rule out disseminated infection before initiation of infliximab therapy. The patient was afebrile and hemodynamically stable. His oral mucosa was normal. He had prominent bilateral lower eyelid edema, erythema, and superficial erosions with hemorrhagic crusting and frank green purulent drainage from both eyes, with crusting along the lower lash line and bilateral sclera injection (Figure 1). On his scalp, face, trunk, and proximal extremities, he had 25 to 30 erythematous, 4- to 8-mm papulopustules with narrow red halos, some with central necrosis and crusting (Figure 2). Cultures from the purulent ocular drainage and pustules on the trunk and arms were all negative for bacteria, virus, and fungi. Gram stain from the eye drainage showed polymorphonuclear leukocytes without organisms. Tissue cultures were negative for bacterial, fungal, and mycobacterial infection. Skin biopsy taken from the central upper back demonstrated subcorneal pustules with areas of eroded epidermis and collections of neutrophils in the superficial dermis (Figure 3). Special stains were negative for organisms. He received infliximab infusion 5 mg/kg for a total dose of 420 mg over 2 hours. Within 48 hours of infusion, there was notable decrease in size of lesions, in addition to reduction of purulent drainage from both eyes. The patient was discharged home following infliximab infusion. His skin lesions resolved during a period of 2 weeks, leaving small pink atrophic scars. He received his second infusion of infliximab 2 weeks after discharge with continued improvement in his gastrointestinal symptoms."}, {"id": "wiki20220301en002_4247", "title": "Correlation does not imply causation", "score": 0.009615384615384616, "content": "Example 5 A historical example of this is that Europeans in the Middle Ages believed that lice were beneficial to your health, since there would rarely be any lice on sick people. The reasoning was that the people got sick because the lice left. The real reason however is that lice are extremely sensitive to body temperature. A small increase of body temperature, such as in a fever, will make the lice look for another host. The medical thermometer had not yet been invented, so this increase in temperature was rarely noticed. Noticeable symptoms came later, giving the impression that the lice left before the person got sick."}, {"id": "pubmed23n1074_13028", "title": "Complicated cutaneous leishmaniasis caused by an imported case of Leishmania tropica in Japan: a case report.", "score": 0.009523809523809525, "content": "Leishmaniasis is not endemic in Japan, and imported cases are rare. However, there are increasing concerns regarding imported cases of cutaneous leishmaniasis from endemic countries to Japan. This report describes a case of imported cutaneous leishmaniasis that was diagnosed and treated in Japan. A 53-year-old Pakistani man presented with skin lesions on both malleoli of his right ankle and the dorsum of the left foot. The skin lesions manifested as erythematous nodules surrounding an ulcer in the center of the lesion. The lesions of the malleoli of his right ankle each measured 3 × 3 cm, and the lesion on the top of his left foot measured 5 × 4 cm. He had been living and working in Japan but had a history of a visit to Pakistan for about 2 months in 2018. The skin lesions were biopsied. Giemsa and hematoxylin and eosin staining of biopsy samples showed amastigotes of Leishmania in macrophages, and the presence of Leishmania was confirmed by skin tissue culture. Polymerase chain reaction using biopsy specimens identified Leishmania parasites, and DNA sequence analysis revealed that the species was Leishmania tropica. The patient was treated with intravenous liposomal amphotericin B for 6 days. The erythema disappeared, and the erythematous nodules resolved within 3 weeks. This is the first report of imported cutaneous leishmaniasis caused by L. tropica from Pakistan, and it is interesting that all three testing modalities showed positive results in this case."}, {"id": "wiki20220301en061_11936", "title": "Exanthem", "score": 0.009523809523809525, "content": "The four viral exanthema have much in common, and are often studied together as a class. They are: Scarlet fever, or \"second disease\", is associated with the bacterium Streptococcus pyogenes. Fourth disease, also known as \"Dukes' disease\" is a condition whose existence is not widely accepted today. It was described in 1900 and is postulated to be related to the bacterium Staphylococcus aureus. In 1979 and 2001 a possible \"seventh diease\" was postulated following reports of a condition in Japan also referred to as acute febrile infantile mucocutaneous lymph node syndrome (MCLS)."}, {"id": "pubmed23n0624_9301", "title": "Chronic nonhealing ulcer of the right thumb with multiple subcutaneous nodules.", "score": 0.009433962264150943, "content": "A 60-year-old man presented with a 3-month history of nonhealing ulcer over the tip of his right thumb. The ulcer started as a blister over the tip of the thumb that later ruptured and spread proximally to cover the whole pulp area of the thumb. There was no history of trauma, fever, weight loss, or loss of appetite. He is a pensioner and an avid gardener. He has a few cats as pets. The patient initially presented to a private orthopedic surgeon with a nonhealing ulcer of the right thumb. Multiple debridements were unsuccessful in ameliorating the ulcer. Three months after the onset of the initial lesion, multiple painless erythematous nodules had developed on his right arm, and one on the right thigh. All routine blood investigations were nondiagnostic. Swab culture from the ulcer failed to grow any organism and a course of antibiotics did not resolve the problem. Cultures of the biopsy specimen using Sabouraud's dextrose agar and potato carrot medium grew dark brown plaques that microscopically appeared to be branching hyphae. A diagnosis of sporotrichosis of the right upper limb was made and the patient was started on antifungal treatment immediately (T. Itraconazole [Sporanox] 200 mg BD). One month after commencement of antifungal treatment, the ulcer began to dry up and at 3 months all the lesions including the one on the right thigh had healed."}, {"id": "wiki20220301en338_30972", "title": "Miliary fever", "score": 0.009433962264150943, "content": "Miliary fever was a medical term in the past (Wolfgang Amadeus Mozart's death report showed this term), used to indicate a general cause of infectious disease that cause an acute fever and skin rashes similar to the cereal grain called proso millet. After subsequent advances in medicine, this term fell into disuse, supplanted by other more specific names of diseases, for example the modern miliary tuberculosis. External links . Annals of the Rheumatic Diseases, 1991; Vol.50: pp. 963–964 Obsolete medical terms"}, {"id": "pubmed23n0673_12084", "title": "Nodular lymphangitis: Report of a case with presentation of a diagnostic paradigm.", "score": 0.009345794392523364, "content": "A 54-year-old man with asthma, mitral valve prolapse, and a back injury developed erythematous nodules that progressed along the lymphatic drainage of his right arm. Skin biopsy revealed granulomatous inflammation with microabscess formation. Culture confirmed Mycobacterium marinum infection. The patient was treated with clarithromycin, ethambutol, rifampin, and topical silver sulfadiazine. Oral doxycycline hyclate was later added because of slow healing. Mycobacterium marinum is one of a group of infectious agents that can cause nodular lymphangitis. Sporotrichoid lesions most commonly develop after cutaneous inoculation with Sporothrix schenckii, Leishmania species, Nocardia species, and Mycobacterium marinum. A thorough clinical history and physical examination can narrow the differential diagnosis by eliciting information about the etiologic setting, incubation time, clinical appearance of the lesions, and presence or absence of systemic involvement for each of the causative organisms. Skin biopsy and microbiological tissue cultures are essential for diagnostic confirmation. The differential diagnosis and a suggested diagnostic paradigm will be reviewed."}, {"id": "article-25158_10", "title": "Miliaria -- History and Physical", "score": 0.009345794392523364, "content": "Miliaria rubra is the most prevalent form of miliaria. The obstruction of eccrine ducts occurs in the deeper layers of the skin and involves an inflammatory response. This results in larger, erythematous papules and vesicles. A critical clinical diagnostic feature that helps differentiate miliaria rubra from folliculitis is minimal follicular involvement. If pustules are present, then miliaria rubra is called miliaria pustulosa and may indicate a bacterial infection. Because an inflammatory response is involved, patients may experience pruritic and painful symptoms. These symptoms may worsen during perspiration, causing more irritation. In neonates usually between the ages of 1 to 3 weeks, the groin, axilla, and neck are the most commonly affected areas. In adults, miliaria rubra is most likely seen in places where clothes rub on the skin such as the trunk and extremities. The face is usually spared. Superinfection with staphylococci may occur, and when impetigo or multiple abscesses are involved, the condition is called periporitis staphylogenes. [19]"}, {"id": "pubmed23n0626_4228", "title": "Sporotrichoid atypical cutaneous infection caused by Mycobacterium marinum.", "score": 0.009259259259259259, "content": "A case of a sporotrichoid cutaneous infection caused by Mycobacterium marinum is reported. A 53- year-old male patient presented with red, partly purulent nodular lesions on the back of his left hand, forearm, and upper medial arm that had developed consecutively during the past 4 weeks. A mycobacterial infection with M. marinum was confirmed by molecular methods in a lesional skin biopsy. The patient was treated systemically with rifampicin (750 mg/day) and clarithromycine (1,000 mg/day), and topically with sulmycin (gentamicin sulfate). After 12 weeks of treatment the nodules regressed, leaving behind erythematous patches. M. marinum is a waterborne mycobacterium that commonly infects fish and amphibians worldwide. Transmissions to humans occur occasionally, in most cases as a granulomatous infection localized to the skin, typically following minor trauma to the hands. For this reason, infections are especially common among aquarium keepers."}, {"id": "InternalMed_Harrison_10338", "title": "InternalMed_Harrison", "score": 0.009259259259259259, "content": "Rickettsialpox begins after mite-bite inoculation of Rickettsia akari into the skin. A papule with a central vesicle evolves to form a 1to 2.5-cm painless crusted black eschar with an erythematous halo and proximal adenopathy. While more common in the northeastern United States and the Ukraine in 1940–1950, rickettsialpox has recently been described in Ohio, Arizona, and Utah. Blistering dactylitis is a painful, vesicular, localized S. aureus or group A streptococcal infection of the pulps of the distal digits of the hands."}, {"id": "pubmed23n0687_14602", "title": "Inflammatory skin metastasis as a first sign of progression of lung cancer--a case report.", "score": 0.009174311926605505, "content": "Skin metastases are present in 1-9% of cancer patients. In rare cases, skin metastases can manifest as lesions with signs of inflammation and are diagnosed as inflammatory cutaneous metastases (ICM). ICM in lung cancer are extremely rare and often misdiagnosed. We report on a 55-year-old man with metastatic lung adenocarcinoma and bone metastases in the axial skeleton and left humerus diagnosed in August 2008. He underwent 6 cycles of palliative chemotherapy with cisplatin and gemcitabine, obtaining a minor response. Five months later, he experienced increasing pain in his left arm, with erythematous oedematous lesion with poorly defined margins and an inflammatory appearance. A diagnosis of skin infection was made and he was treated by antibiotic therapy without improvement. Skin biopsy revealed skin infiltration by poorly differentiated carcinoma compatible with a primary lung tumour. He was started on second line therapy with docetaxel, however, the patient's status deteriorated rapidly and he died two months after the first appearance of ICM. Metastasis of lung carcinoma could be one of the causes of inflammatory skin lesions in cancer patients and these metastases should be considered in cancer patients with persisting cutaneous lesions with signs of inflammation and no response to antibiotic therapy."}, {"id": "InternalMed_Harrison_11802", "title": "InternalMed_Harrison", "score": 0.009174311926605505, "content": "fever and chills. Erysipelas tends to occur on the malar area of the face (often with extension over the bridge of the nose to the contralateral malar region) and the lower extremities. After one episode, recurrence at the same site—sometimes years later— is not uncommon. Classic cases of erysipelas, with typical features, are almost always due to β-hemolytic streptococci, usually GAS and occasionally group C or G. Often, however, the appearance of streptococcal cellulitis is not sufficiently distinctive to permit a specific diagnosis on clinical grounds. The area involved may not be typical for erysipelas, the lesion may be less intensely red than usual and may fade into surrounding skin, and/or the patient may appear only mildly ill. In such cases, it is prudent to broaden the spectrum of empirical antimicrobial therapy to include other pathogens, particularly S. aureus, that can produce cellulitis with the same appearance. Staphylococcal infection should be suspected if cellulitis"}, {"id": "wiki20220301en263_32977", "title": "Erosio interdigitalis blastomycetica", "score": 0.009151501222289547, "content": "Erosio interdigitalis blastomycetica (EIB) is a skin condition caused by a Candida albicans infection, characterized by an oval-shaped area of macerated white skin on the web between and extending onto the sides of the fingers. Signs and symptoms It is common among bartenders and homemakers. EIB can be found on both the hands and feet.It is most common between the middle and ring finger and sometimes found between toes. Cause It is believed that EIB is caused by working with water often. It is not known exactly how long the infection will last since it varies often between people who have gotten infected. The most common symptoms are pruritus and discomfort while on rare occasions some do experience pain. History EIB was first discovered by 2 two scientists, Gougerot and Goncea in 1915. It was later named by another scientist Johannes Fabry in 1917. See also Candidiasis Skin lesion References Mycosis-related cutaneous conditions"}, {"id": "pubmed23n0366_19996", "title": "Disseminated cutaneous histoplasmosis and AIDS: case report.", "score": 0.00909090909090909, "content": "Histoplasmosis is caused by the dimorphic fungus Histoplasma capsulatum. It manifests by the presence of fever as the only symptom in most individuals. The disease may present as self-limited pneumonia, or as an hematogenous widespread fungal infection with a potentially fatal outcome in elderly individuals and people with compromised T-cell mediated immunity. Here, we report a case of disseminated cutaneous histoplasmosis in a patient with AIDS. The patient was a 33 year old male homosexual, intravenous drug user, who had been diagnosed with HIV infection 5 years earlier. He was in good health, but had erythematous papules and pustules in the skin of the scalp, face, back, thighs, abdomen, palms, and soles. He was placed on anti-retroviral therapy, fluconazole for mucosal candidiasis, trimethoprim/sulfamethoxazole for pneumocystis prophylaxis, and antibiotics for the skin pustules. The skin lesions improved remarkably within 14 days. He was discharged and soon lost to follow-up. After his discharge, skin biopsy and fungal culture results revealed H. capsulatum. He was seen again 1 year later. The interim history revealed that he had taken fluconazole 100 mg/day for 1 month and fluconazole 150 mg/week for 7 months. He had not continued anti-retroviral therapy, nor taken other antifungal drugs. The clinical evolution of the disease was exceptional in that there was disappearance of all the skin lesions attributed to histoplasmosis with fluconazole. Although itraconazole remains the drug of choice for histoplasmosis. Cutaneous histoplasmosis should be considered in the differential diagnosis of atypical cutaneous lesions in individuals infected with HIV."}, {"id": "wiki20220301en026_50868", "title": "Janeway lesion", "score": 0.00909090909090909, "content": "Diagnosis Janeway lesions present as red, painless macules and papules on the palms and soles. They are not common and are frequently indistinguishable from Osler's nodes. Rarely, they have been reported in cases of Systemic lupus erythematosis (SLE), Gonococcemia (disseminated gonorrhoea), haemolytic anaemia and typhoid fever. They may last days to weeks before completely resolving. History Janeway lesions are named after Edward Janeway (1841–1911), a prominent American physician, pathologist and contemporary of Sir William Osler, who initially described \"peculiar skin lesions\" in some people with endocarditis, in a paper published in 1899. The term was first used by internist and pathologist Emanuel Libman, who reported the lesions in his paper of 1906 and explained his reasoning for using the term \"Janeway lesions\" in a footnote in 1923. Osler never mentioned Janeway lesions. The inclusion into Osler's 1925 textbook came six years after Osler died."}, {"id": "wiki20220301en075_44493", "title": "Sporotrichosis", "score": 0.009009009009009009, "content": "It has been reported in cats, mules, dogs, mice and rats. Signs and symptoms Cutaneous or skin sporotrichosis This is the most common form of this disease. Symptoms of this form include nodular lesions or bumps in the skin, at the point of entry and also along lymph nodes and vessels. The lesion starts off small and painless, and ranges in color from pink to purple. Left untreated, the lesion becomes larger and look similar to a boil and more lesions will appear, until a chronic ulcer develops. Usually, cutaneous sporotrichosis lesions occur in the finger, hand, and arm. Pulmonary sporotrichosis This rare form of the disease occur when S. schenckii spores are inhaled. Symptoms of pulmonary sporotrichosis include productive coughing, nodules and cavitations of the lungs, fibrosis, and swollen hilar lymph nodes. Patients with this form of sporotrichosis are susceptible to developing tuberculosis and pneumonia"}, {"id": "InternalMed_Harrison_1671", "title": "InternalMed_Harrison", "score": 0.009009009009009009, "content": "CHAPTER 25e Atlas of Rashes Associated with Fever Figure 25e-51 Smallpox is shown with many pustules on the face, becoming confluent (A), and on the trunk (B). Pustules are all in the same stage of development. C. Crusting, healing lesions are noted on the trunk, arms, and hands. (Reprinted from K Wolff, RA Johnson: Color Atlas and Synopsis of Clinical Dermatology, 6th ed. New York, McGraw-Hill, 2009.) CHAPTER 26 Fever of Unknown Origin fever of unknown origin Chantal P. Bleeker-Rovers, Jos W. M. van der Meer DEFINITION Clinicians commonly refer to any febrile illness without an initially obvious etiology as fever of unknown origin (FUO). Most febrile ill-nesses either resolve before a diagnosis can be made or develop distin-26 guishing characteristics that lead to a diagnosis. The term FUO should be reserved for prolonged febrile illnesses without an established etiology despite intensive evaluation and diagnostic testing. This chapter focuses on classic FUO in the adult patient."}, {"id": "wiki20220301en077_45664", "title": "Lupus vulgaris", "score": 0.008928571428571428, "content": "Lupus vulgaris (also known as tuberculosis luposa) are painful cutaneous tuberculosis skin lesions with nodular appearance, most often on the face around the nose, eyelids, lips, cheeks, ears and neck. It is the most common Mycobacterium tuberculosis skin infection. The lesions may ultimately develop into disfiguring skin ulcers if left untreated. Signs and symptoms It begins as painless reddish-brown nodules which slowly enlarge to form irregularly shaped red plaque. Cause Lupus vulgaris often develops due to inadequately treated pre-existing tuberculosis. It may also develop at site of BCG vaccination. Rarely, it has been shown to be associated with tattoo marks. Histopathology Histologically, it shows presence of epithelioid cell granulomas with Langhans giant cells with or without central caseation necrosis in the dermis. Diagnosis On diascopy, it shows characteristic \"apple-jelly\" color. Biopsy will reveal tuberculoid granuloma with few bacilli. Mantoux test is positive."}, {"id": "wiki20220301en084_15627", "title": "Royal touch", "score": 0.008928571428571428, "content": "The touch was originally meant to cure tuberculous cervical lymphadenitis (commonly referred to as scrofula or the King's Evil), rheumatism, convulsions, fevers, blindness, goitre and other ailments. Since the reign of Elizabeth I (r. 1558–1603), however, the touch was applied only to people suffering from scrofula. The Henrician practice was rarely modified, and the changes were minor; Elizabeth I made the sign of the cross above the infected person's head, while her squeamish successor, James I (r. 1603–1625), made stroking motions above the abscesses instead of actually touching them. Frequency"}, {"id": "pubmed23n0925_17637", "title": "Missed opportunity to diagnose subungual melanoma: potential pitfalls!", "score": 0.008849557522123894, "content": "Subungual melanoma, an uncommon form of acral melanoma that arises within the nail matrix, accounts for 1%-3% of all cutaneous melanoma in Caucasians. As subungual melanoma presents in a more disguised manner than cutaneous lesions, increased vigilance is required. It most commonly presents as a discolouration of the nail, nail splitting or nail-bed bleeding. Black pigmentation of the adjacent nail fold, termed Hutchinson's sign, may be a diagnostic clue. Treatment of subungual melanoma remains surgical with wide local excision and amputation primary modalities. We present the case of a 61-year-old man with an 18-month history of a left thumb nail-bed abnormality and a 6-week history of left axillary lymphadenopathy. One year earlier, he presented to the emergency department with a purulent discharge from his left thumb but declined nail-bed ablation. He was referred to the 'Hand and Plastic Injuries Clinic' by his general practitioner and diagnosed with a chronic traumatic-induced nail-bed injury. As his symptoms did not improve, he was referred to the 2-week wait Skin Cancer Clinic. The left thumb nail-bed was excised as a nail unit down to bone, and the diagnosis of melanoma was rendered. Left axillary lymphadenopathy was confirmed as metastatic melanoma. He underwent amputation of his left thumb at the interproximal phalangeal joint, and a left axillary node dissection was performed. No residual melanoma was identified in his thumb. Microscopically, his left axillary dissection confirmed 9 out of 36 positive nodes for metastatic melanoma with extracapsular spread. He was staged at IIIC disease. This case report demonstrates missed opportunities to diagnose subungual melanoma and acts as a cautionary tale in considering this pathology in the differential diagnosis of nail-bed lesions with prompt referral for further investigation."}, {"id": "wiki20220301en570_31877", "title": "Myrnie Gifford", "score": 0.008849557522123894, "content": "She began to conduct skin tests on all patients suffering from valley fever; and found that whilst some were symptomless, they were all positive for coccidioidomycosis. Gifford was the first person to recognise that desert fever and valley fever were caused by the coccidioides fungus. This work received national recognition. She was the first to demonstrate that valley fever were the primary stages of the coccidioidomycosis infection. In 1938, Gifford joined E. C. Dickson to explain that the infection resembles primary tuberculosis and a full clinical recovery is possible. She continued to work on coccidioidomycosis and found that it occurred more often in men than in women and people of ethnic minorities. Over 80% of the patients who died had been engaged in agriculture or work where dust could have been involved."}, {"id": "pubmed23n1035_7042", "title": "Mycobacterium chelonae cutaneous infection: An opportunistic disease in an immunosuppressed patient with myasthenia gravis.", "score": 0.008771929824561403, "content": "30 mEq/L). Causes Acute Acute respiratory acidosis occurs when an abrupt failure of ventilation occurs. This failure in ventilation may be caused by depression of the central respiratory center by cerebral disease or drugs, inability to ventilate adequately due to neuromuscular disease (e.g., myasthenia gravis, amyotrophic lateral sclerosis, Guillain–Barré syndrome, muscular dystrophy), or airway obstruction related to asthma or chronic obstructive pulmonary disease (COPD) exacerbation."}, {"id": "wiki20220301en040_1133", "title": "Respiratory acidosis", "score": 0.012727898176814585, "content": "Diagnosis Diagnoses can be done by doing an ABG (Arterial Blood Gas) laboratory study, with a pH <7.35 and a PaCO2 >45 mmHg in an acute setting. Patients with COPD and other Chronic respiratory diseases will sometimes display higher numbers of PaCO2 with HCO3- >30 and normal pH. Terminology Acidosis refers to disorders that lower cell/tissue pH to < 7.35. Acidemia refers to an arterial pH < 7.36. See also Acidosis Alkalosis Arterial blood gas Chemical equilibrium pCO2 pH pKa Metabolic acidosis Metabolic alkalosis Respiratory alkalosis References External links Acid–base disturbances"}, {"id": "wiki20220301en054_12513", "title": "Respiratory alkalosis", "score": 0.012720786056848753, "content": "Diagnosis The diagnosis of respiratory alkalosis is done via test that measure the oxygen and carbon dioxide levels (in the blood), chest x-ray and a pulmonary function test of the individual. The Davenport diagram allows clinicians or investigators to outline blood bicarbonate concentrations (and blood pH) after a respiratory or metabolic acid-base disturbance Classification There are two types of respiratory alkalosis: chronic and acute as a result of the 3–5 day delay in kidney compensation of the abnormality. Acute respiratory alkalosis occurs rapidly, have a high pH because the response of the kidneys is slow. Chronic respiratory alkalosis is a more long-standing condition, here one finds the kidneys have time to decrease the bicarbonate level. pH Alkalosis refers to the process due to which there is elevation of blood pH. Alkalemia refers to an arterial blood pH of greater than 7.45."}, {"id": "pubmed23n0048_354", "title": "[Respiratory regulation system].", "score": 0.012713675213675214, "content": "In 1874, Kussmaul described \"featiful terminal dyspnea\" in a case of severe diabetic coma. Probably, this was the first sign of respiratory regulation for metabolic acidosis. After this first case, about 40 years has been needed to establish the theory of acid-base equiribration, namely Henderson-Hasselbalch's equation. pH = 6.1 + log [HCO3-]/[H2CO3] This equation indicates that plasma pH is determined by the ratio of HCO3 concentration and H2CO3 concentration. Because of linear relationship between H2CO3 and PaCO2, pH depend on the ratio of HCO3- and PaCO2. In the state of metabolic acidosis, increase of [H+] stimulates ventilation and decreases the PaCO2. Inversely, in the state of metabolic alkalosis, increase of PaCO2 occurs. These reactions are called \"respiratory compensation\" or \"respiratory regulation\". The respiratory regulation system will not retern pH to normal (7.4), but compensation has some limitation which is shown as \"SIGNIFICANCE BAND\". In this paper, physiological and clinical importance of respiratory regulation and significance band is discussed."}, {"id": "article-17837_27", "title": "Arterial Blood Gas -- Clinical Significance", "score": 0.011847553419772117, "content": "Acid-base balance can be affected by the aforementioned respiratory system abnormalities. For instance, acute respiratory acidosis and alkalemia result in acidemia and alkalemia, respectively. Additionally, hypoxemic hypoxia leads to anaerobic metabolism, which causes metabolic acidosis that results in acidemia. Metabolic system abnormalities also affect acid balance, as acute metabolic acidosis and alkalosis result in acidemia and alkalemia. [25] Patients with diabetic ketoacidosis, septic shock, renal failure, drug or toxin ingestion, and gastrointestinal or renal HCO 3 loss exhibit metabolic acidosis. [28] Conditions such as kidney disease, electrolyte imbalances, prolonged vomiting, hypovolemia, diuretic use, and hypokalemia cause metabolic alkalosis. [36]"}, {"id": "article-17837_19", "title": "Arterial Blood Gas -- Results, Reporting, and Critical Findings", "score": 0.011827558520165524, "content": "Example 1 [28] : ABG: pH = 7.39, PaCO 2 = 51 mm Hg, PaO 2 = 59 mm Hg, HCO 3 = 30 mEq/L and SaO 2 = 90%, on room air. pH is in the normal range, so use 7.40 as a cutoff point, in which case it is < 7.40, and acidosis is present. The elevated PaCO 2 indicates respiratory acidosis, and the elevated HCO 3 indicates a metabolic alkalosis. The value consistent with the pH is PaCO 2 . Therefore, this is a primary respiratory acidosis. The acid-base that is inconsistent with the pH is the elevated HCO3, indicating a metabolic alkalosis, so there is compensation signifying a non-acute primary disorder because it takes days for metabolic compensation to be effective. Last, the decreased PaO 2 indicates an abnormality with oxygenation. However, a history and physical will help delineate the severity and urgency of required interventions, if any. Example 2 [28] : ABG: pH = 7.45, PaCO 2 = 32 mm Hg, PaO 2 = 138 mm Hg, HCO 3 = 23 mEq/L, the base deficit = 1 mEq/L, and SaO 2 is 92%, on room air."}, {"id": "pubmed23n0053_19826", "title": "A case of Cushing's syndrome associated with chronic respiratory failure due to metabolic alkalosis.", "score": 0.011783575005728127, "content": "A case of Cushing's syndrome associated with chronic respiratory failure is presented. Although arterial blood gas analysis showed severe metabolic alkalosis, hypoxemia and mild hypercapnia, the patient had no evidence of pulmonary disease or neuromuscular disorder. Voluntary hyperventilation and inhalation of 100% oxygen (O2) revealed normalized arterial oxygen tension (PaO2). Following the recovery from metabolic alkalosis by the treatment with potassium chloride, PaO2 was elevated and arterial carbon dioxide tension (PaCO2) was lowered. Therefore, it was strongly suggested that the main cause of chronic respiratory failure was compensatory alveolar hypoventilation as a response to metabolic alkalosis."}, {"id": "wiki20220301en054_12514", "title": "Respiratory alkalosis", "score": 0.011715462638968862, "content": "pH Alkalosis refers to the process due to which there is elevation of blood pH. Alkalemia refers to an arterial blood pH of greater than 7.45. Treatment Respiratory alkalosis is very rarely life-threatening, though pH level should not be 7.5 or greater. The aim in treatment is to detect the underlying cause. When PaCO2 is adjusted rapidly in individuals with chronic respiratory alkalosis, metabolic acidosis may occur. If the individual is on a mechanical ventilator then preventing hyperventilation is done via monitoring ABG levels. In popular culture In The Andromeda Strain, one of the characters is exposed to contamination, but saves himself by increasing his respiratory rate to induce alkalosis. See also References Further reading External links Acid–base disturbances"}, {"id": "wiki20220301en147_46232", "title": "Respiratory compensation", "score": 0.011702458761282291, "content": "In metabolic alkalosis, chemoreceptors sense a deranged acid-base balance with a plasma pH of greater than normal (>7.4). The chemoreceptors send afferent fibers to the brainstem respiratory centers. The brainstem respiratory centers decrease alveolar ventilation (hypoventilation) to create a rise in arterial carbon dioxide () tension, resulting in a decrease of plasma pH. The respiratory brainstem centers can only compensate for metabolic acid-base disturbances (metabolic acidosis and metabolic alkalosis). Renal compensation is needed to balance respiratory acid-base syndromes (respiratory acidosis and respiratory alkalosis). The kidneys can compensate for both, respiratory and metabolic acid-base imbalances. References Acid–base disturbances"}, {"id": "wiki20220301en028_78627", "title": "Multiple organ dysfunction syndrome", "score": 0.011646351644681916, "content": "Four clinical phases have been suggested: Stage 1: the patient has increased volume requirements and mild respiratory alkalosis which is accompanied by oliguria, hyperglycemia and increased insulin requirements. Stage 2: the patient is tachypneic, hypocapnic and hypoxemic; develops moderate liver dysfunction and possible hematologic abnormalities. Stage 3: the patient develops shock with azotemia and acid-base disturbances; has significant coagulation abnormalities. Stage 4: the patient is vasopressor dependent and oliguric or anuric; subsequently develops ischemic colitis and lactic acidosis. Definition Multiple dysfunction syndrome is the presence of altered organ function in acutely ill patients such that homeostasis cannot be maintained without intervention. It usually involves two or more organ systems. It calls for an immediate intervention."}, {"id": "wiki20220301en074_45830", "title": "Base excess", "score": 0.011617749825296994, "content": "Interpretation Base excess beyond the reference range indicates metabolic alkalosis if too high (more than +2 mEq/L) metabolic acidosis if too low (less than −2 mEq/L) Blood pH is determined by both a metabolic component, measured by base excess, and a respiratory component, measured by PaCO2 (partial pressure of carbon dioxide). Often a disturbance in one triggers a partial compensation in the other. A secondary (compensatory) process can be readily identified because it opposes the observed deviation in blood pH. For example, inadequate ventilation, a respiratory problem, causes a buildup of CO2, hence respiratory acidosis; the kidneys then attempt to compensate for the low pH by raising blood bicarbonate. The kidneys only partially compensate, so the patient may still have a low blood pH, i.e. acidosis. In summary, the kidneys partially compensate for respiratory acidosis by raising blood bicarbonate."}, {"id": "InternalMed_Harrison_3726", "title": "InternalMed_Harrison", "score": 0.011544655022915892, "content": "pneumonia) leading to respiratory acidosis or alkalosis. Patients with underlying pulmonary disease (e.g., chronic obstructive pulmonary disease) may not respond to metabolic acidosis with an appropriate ventilatory response because of insufficient respiratory reserve. Such imposition of respiratory acidosis on metabolic acidosis can lead to severe acidemia. When metabolic acidosis and metabolic alkalosis coexist in the same patient, the pH may be normal or near normal. When the pH is normal, an elevated anion gap (AG; see below) reliably denotes the presence of an AG metabolic acidosis at a normal serum albumin of 4.5 g/dL. Assuming a normal AG of 10 mmol/L, a discrepancy in the ∆AG (prevailing minus normal AG) and the ∆HCO3 (normal value of 25 mmol/L minus abnormal HCO3 in the patient) indicates the presence of a mixed high-gap acidosis— metabolic alkalosis (see example below). A diabetic patient with ketoacidosis may have renal dysfunction resulting in simultaneous metabolic"}, {"id": "wiki20220301en040_1132", "title": "Respiratory acidosis", "score": 0.011389759665621734, "content": "Acute respiratory acidosis: HCO3− increases 1 mEq/L for each 10 mm Hg rise in PaCO2. Chronic respiratory acidosis: HCO3− rises 3.5 mEq/L for each 10 mm Hg rise in PaCO2. The expected change in pH with respiratory acidosis can be estimated with the following equations: Acute respiratory acidosis: Change in pH = 0.08 X ((40 − PaCO2)/10) Chronic respiratory acidosis: Change in pH = 0.03 X ((40 − PaCO2)/10) Respiratory acidosis does not have a great effect on electrolyte levels. Some small effects occur on calcium and potassium levels. Acidosis decreases binding of calcium to albumin and tends to increase serum ionized calcium levels. In addition, acidemia causes an extracellular shift of potassium, but respiratory acidosis rarely causes clinically significant hyperkalemia. Diagnosis"}, {"id": "wiki20220301en040_1130", "title": "Respiratory acidosis", "score": 0.011311064063357641, "content": "Physiological response Mechanism Metabolism rapidly generates a large quantity of volatile acid (H2CO3) and nonvolatile acid. The metabolism of fats and carbohydrates leads to the formation of a large amount of CO2. The CO2 combines with H2O to form carbonic acid (H2CO3). The lungs normally excrete the volatile fraction through ventilation, and acid accumulation does not occur. A significant alteration in ventilation that affects elimination of CO2 can cause a respiratory acid-base disorder. The PaCO2 is maintained within a range of 35–45 mm Hg in normal states. Alveolar ventilation is under the control of the respiratory center, which is located in the pons and the medulla. Ventilation is influenced and regulated by chemoreceptors for PaCO2, PaO2, and pH located in the brainstem, and in the aortic and carotid bodies as well as by neural impulses from lung stretch receptors and impulses from the cerebral cortex. Failure of ventilation quickly increases the PaCO2."}, {"id": "wiki20220301en267_19616", "title": "Winters' formula", "score": 0.011164274322169059, "content": "Winters' formula, named for Dr. R.W. Winters, is a formula used to evaluate respiratory compensation when analyzing acid–base disorders and a metabolic acidosis is present. It can be given as , where HCO3− is given in units of mEq/L and pCO2 will be in units of mmHg. Winters' formula gives an expected value for the patient's PCO2; the patient's actual (measured) PCO2 is then compared to this: If the two values correspond, respiratory compensation is considered to be adequate. If the measured PCO2 is higher than the calculated value, there is also a secondary respiratory acidosis or mixed acid base disorder. If the measured PCO2 is lower than the calculated value, there is also a secondary respiratory alkalosis or mixed acid base disorder. References Respiratory therapy Mathematics in medicine"}, {"id": "wiki20220301en071_55445", "title": "Metabolic alkalosis", "score": 0.011062397099358086, "content": "To calculate the expected pCO2 in the setting of metabolic alkalosis, the following equations are used: pCO2 = 0.7 [HCO3] + 20 mmHg ± 5 pCO2 = 0.7 [HCO3] + 21 mmHg Treatment To effectively treat metabolic alkalosis, the underlying cause(s) must be corrected. A trial of intravenous chloride-rich fluid is warranted if there is a high index of suspicion for chloride-responsive metabolic alkalosis caused by loss of gastrointestinal fluid (e.g., due to vomiting). Terminology Alkalosis refers to a process by which the pH is increased. Alkalemia refers to a pH which is higher than normal, specifically in the blood. See also Hypokalemia Metabolic acidosis Respiratory acidosis Respiratory alkalosis References External links Acid–base disturbances"}, {"id": "wiki20220301en019_114904", "title": "Alkalosis", "score": 0.011025596679349705, "content": "Metabolic alkalosis can be caused by repeated vomiting, resulting in a loss of hydrochloric acid in the stomach contents. Severe dehydration, and the consumption of alkali, are other causes. It can also be caused by administration of diuretics and endocrine disorders such as Cushing's syndrome. Compensatory mechanism for metabolic alkalosis involve slowed breathing by the lungs to increase serum carbon dioxide, a condition leaning toward respiratory acidosis. As respiratory acidosis often accompanies the compensation for metabolic alkalosis, and vice versa, a delicate balance is created between these two conditions. See also References External links Acid–base disturbances"}, {"id": "First_Aid_Step1_679", "title": "First_Aid_Step1", "score": 0.010841064945542558, "content": "Features of renal disorders Key: • = compensatory response. [HCO3 −]Henderson-Hasselbalch equation: pH = 6.1 + log 0.03 P2 Predicted respiratory compensation for a simple metabolic acidosis can be calculated using the Winters formula. If measured Pco2 > predicted Pco2 Ž concomitant respiratory acidosis; if measured Pco2 < predicted Pco2 • concomitant respiratory alkalosis: Pco2 = 1.5 [HCO3–] + 8 ± 2 Acidosis and alkalosis Check arterial pH pH < 7.35 Pco2 > 44 mm Hg HCO3 – < 20 mEq/L Acidemia Respiratory acidosis Metabolic acidosis Hypoventilation Airway obstruction Acute lung disease Chronic lung disease Opioids, sedatives Weakening of respiratory muscles •Anion gap MUDPILES: Normal anion gap Check anion gap Methanol (formic acid) Uremia Diabetic ketoacidosis Propylene glycol Iron tablets or INH Lactic acidosis Ethylene glycol (oxalic acid) Salicylates (late) HARDASS:= Na+ – (CI + HCO3 )– _"}, {"id": "article-40870_20", "title": "Case Study: 60-Year-Old Female Presenting With Shortness of Breath -- Diagnosis", "score": 0.010815503003003003, "content": "Myxedema coma or severe hypothyroidism Pericardial effusion secondary to myxedema coma COPD exacerbation Acute on chronic hypoxic respiratory failure Acute respiratory alkalosis Bilateral community-acquired pneumonia Small bilateral pleural effusions Acute mild rhabdomyolysis Acute chronic, stage IV, renal failure Elevated troponin I levels, likely secondary to Renal failure Diabetes mellitus type 2, non-insulin-dependent Extreme obesity Hepatic dysfunction"}, {"id": "pubmed23n0544_11458", "title": "Acid-base balance in heart failure.", "score": 0.010647216633132126, "content": "In end-stage heart failure, various acid-base disorders can be discovered due to the renal loss of hydrogen ions and hydrogen ion movements into cells, the reduction of the effective circulating volume, hypoxemia and renal failure. This justifies the occurrence of metabolic alkalosis, metabolic acidosis, respiratory alkalosis, as well as respiratory acidosis alone or in combination. Several studies have been published on the acid-base state in heart failure. In a 1951 study, Squires et al analyzed the distribution of body fluid in congestive heart failure by taking into consideration the abnormalities in serum electrolyte concentration and in acid-base equilibrium. A recent study by Milionis et al, analyzed 86 patients with congestive heart failure receiving conventional treatment; the majority of these patients exhibited hypokalemia, hyponatremia, hypocalcemia and hypophosphatemia. Disorders in acid-base balance were noted in 37.2% of patients. In a recent study, 70 patients with severe congestive heart failure before heart transplantation showed high-normal pH, slightly reduced pCO 2 and a slight loss of hydrogen ions. After heart transplantation, stability of blood pH and hydrogen ion concentrations was found. In contrast, bicarbonate and pCO 2 increased significantly. The data led us to formulate the diagnosis of a mixed acid-base disorder that includes respiratory alkalosis and metabolic alkalosis before heart transplantation. In heart failure, the presence of acid-base imbalance associated with the activation of mechanisms that lead to salt and water retention reveals evidence concerning the pivotal role of the kidney in determining the outcome of these patients."}, {"id": "wiki20220301en040_1093", "title": "Metabolic acidosis", "score": 0.010586767458074933, "content": "Signs and symptoms Acute metabolic acidosis Symptoms are not specific, and diagnosis can be difficult unless patients present with clear indications for arterial blood gas sampling. Symptoms may include palpitations, headache, altered mental status such as severe anxiety due to hypoxia, decreased visual acuity, nausea, vomiting, abdominal pain, altered appetite and weight gain, muscle weakness, bone pain, and joint pain. People with acute metabolic acidosis may exhibit deep, rapid breathing called Kussmaul respirations which is classically associated with diabetic ketoacidosis. Rapid deep breaths increase the amount of carbon dioxide exhaled, thus lowering the serum carbon dioxide levels, resulting in some degree of compensation. Overcompensation via respiratory alkalosis to form an alkalemia does not occur. Extreme acidemia can also lead to neurological and cardiac complications:"}, {"id": "wiki20220301en019_114896", "title": "Acidosis", "score": 0.010538641686182671, "content": "Acid consumption from poisoning such as methanol ingestion, elevated levels of iron in the blood, and chronically decreased production of bicarbonate may also produce metabolic acidosis. Metabolic acidosis is compensated for in the lungs, as increased exhalation of carbon dioxide promptly shifts the buffering equation to reduce metabolic acid. This is a result of stimulation to chemoreceptors, which increases alveolar ventilation, leading to respiratory compensation, otherwise known as Kussmaul breathing (a specific type of hyperventilation). Should this situation persist, the patient is at risk for exhaustion leading to respiratory failure. Mutations to the V-ATPase 'a4' or 'B1' isoforms result in distal renal tubular acidosis, a condition that leads to metabolic acidosis, in some cases with sensorineural deafness."}, {"id": "pubmed23n0349_3075", "title": "Impairment of ventilatory response to metabolic acidosis in insulin-dependent diabetic patients with advanced nephropathy.", "score": 0.009900990099009901, "content": "Sudden cardiopulmonary arrest due to a defective respiratory reflex is observed in diabetic patients. Impaired ventilatory response in diabetic patients to acute hypoxia or hypercapnia induced by the inhalation of an artificial gas has been reported. Little is known regarding the respiratory compensatory ability for mild to moderate metabolic acidosis due to renal failure in insulin-dependent diabetic subjects. Arterial blood pH, HCO3-, PaCO2 and PaO2 were measured in 13 insulin-dependent diabetic subjects with advanced nephropathy and in 33 non-diabetic subjects with end-stage renal failure. The diabetic group consisted of six predialysis patients and seven on regular hemodialysis (HD) and the non-diabetic group, ten predialysis patients and 23 on HD. Differences between measured partial arterial pressure of carbon dioxide (PaCO2) and predicted PaCO2 determined from HCO3- were examined. PaCO2 was significantly higher in the diabetic than in non-diabetic group (40.0 +/- 7.4 versus 31.1 +/- 5.1 mmHg, p < 0.05 in predialysis, 42.0 +/- 6.4 versus 36.0 +/- 2.6 mmHg, p < 0.05 in HD), though plasma pH was essentially the same for either. Differences in measured PaCO2 and predicted PaCO2 were significantly larger in the diabetic group than in non-diabetic group. Ventilatory response to uremic acidosis may thus be considered impaired in subjects with advanced diabetic nephropathy."}, {"id": "wiki20220301en010_32316", "title": "Respiratory failure", "score": 0.00985014985014985, "content": "Respiratory failure results from inadequate gas exchange by the respiratory system, meaning that the arterial oxygen, carbon dioxide, or both cannot be kept at normal levels. A drop in the oxygen carried in the blood is known as hypoxemia; a rise in arterial carbon dioxide levels is called hypercapnia. Respiratory failure is classified as either Type 1 or Type 2, based on whether there is a high carbon dioxide level, and can be acute or chronic. In clinical trials, the definition of respiratory failure usually includes increased respiratory rate, abnormal blood gases (hypoxemia, hypercapnia, or both), and evidence of increased work of breathing. Respiratory failure causes an altered mental status due to ischemia in the brain. The typical partial pressure reference values are oxygen Pa O2 more than 80 mmHg (11 kPa) and carbon dioxide Pa CO2 less than 45 mmHg (6.0 kPa). Cause"}, {"id": "wiki20220301en142_42526", "title": "Davenport diagram", "score": 0.009826570242399892, "content": "Respiratory and metabolic acid-base disturbances One of the most important features of the Davenport diagram is its usefulness in depicting movement from one point on the equilibrium surface to another following changes in respiration and/or metabolism. Four fundamental changes may occur that affect acid-base balance in the body: respiratory acidosis, respiratory alkalosis, metabolic acidosis and metabolic alkalosis. Additionally, a respiratory and a metabolic disturbance may occur simultaneously, such as respiratory acidosis followed by a compensatory shift towards metabolic alkalosis. Respiratory disturbances"}, {"id": "pubmed23n0103_9640", "title": "Hypophosphatemia and acute respiratory failure in a diabetic patient.", "score": 0.00980392156862745, "content": "A previously healthy 48-year-old male developed diabetic ketoacidosis and severe hypophosphatemia. Within a few hours, acute respiratory insufficiency developed with a marked discrepancy between the pulmonary pathology and the very poor oxygenation seen. We argue that this was due to the effect of hypophosphatemia on respiratory muscle- and heart function and P50, leading to impaired oxygen delivery."}]}}}} {"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 102]], "word_ranges": [[0, 17]], "text": "Since it is the first episode of atrial fibrillation, it is the ideal candidate for catheter ablation."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Since it is the first episode of atrial fibrillation, it is the ideal candidate for catheter ablation.", "full_answer_no_ref": "Since it is the first episode of atrial fibrillation, it is the ideal candidate for catheter ablation.", "full_question": "A 76-year-old patient with hypertension and diabetes mellitus comes to the emergency department because he has been experiencing palpitations and decreased capacity to exert himself for 72 hours. On arrival, atrial fibrillation with ventricular response of around 120 bpm was documented. Which of the following options is FALSE?", "id": 288, "lang": "en", "options": {"1": "This patient should be orally anticoagulated for life, unless contraindicated.", "2": "If we decide to perform cardioversion on arrival at the ED, it would be necessary to perform transesophageal echocardiography beforehand.", "3": "Being the first episode of atrial fibrillation, it is the ideal candidate for catheter ablation.", "4": "Beta-blockers may be used to slow the heart rate.", "5": NaN}, "question_id_specific": 227, "type": "CARDIOLOGY AND VASCULAR SURGERY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0397_8310", "title": "Management of the older person with atrial fibrillation.", "score": 0.01838731443994602, "content": "Atrial fibrillation (AF) is associated with a higher incidence of mortality, stroke, and coronary events than is sinus rhythm. AF with a rapid ventricular rate may cause a tachycardia-related cardiomyopathy. Immediate direct-current (DC) cardioversion should be performed in patients with AF and acute myocardial infarction, chest pain due to myocardial ischemia, hypotension, severe heart failure, or syncope. Intravenous beta blockers, verapamil, or diltiazem may be given to slow immediately a very rapid ventricular rate in AF. An oral beta blocker, verapamil, or diltiazem should be used in persons with AF if a fast ventricular rate occurs at rest or during exercise despite digoxin. Amiodarone may be used in selected patients with symptomatic life-threatening AF refractory to other drugs. Nondrug therapies should be performed in patients with symptomatic AF in whom a rapid ventricular rate cannot be slowed by drugs. Paroxysmal AF associated with the tachycardia-bradycardia syndrome should be treated with a permanent pacemaker in combination with drugs. A permanent pacemaker should be implanted in patients with AF and with symptoms such as dizziness or syncope associated with ventricular pauses greater than 3 seconds that are not drug-induced. Elective DC cardioversion has a higher success rate and a lower incidence of cardiac adverse effects than does medical cardioversion in converting AF to sinus rhythm. Unless transesophageal echocardiography has shown no thrombus in the left atrial appendage before cardioversion, oral warfarin should be given for 3 weeks before elective DC or drug cardioversion of AF and should be continued for at least 4 weeks after maintenance of sinus rhythm. Many cardiologists prefer, especially in older persons, ventricular rate control plus warfarin rather than maintaining sinus rhythm with antiarrhythmic drugs. Digoxin should not be used to treat patients with paroxysmal AF. Patients with chronic or paroxysmal AF at high risk for stroke should be treated with long-term warfarin to achieve an International Normalized Ratio of 2.0 to 3.0. Patients with AF at low risk for stroke or with contraindications to warfarin should receive 325 mg of aspirin daily."}, {"id": "pubmed23n0407_20287", "title": "Atrial fibrillation in the elderly: facts and management.", "score": 0.018304351443547716, "content": "Although atrial fibrillation is not widely known by the general public, in developed countries it is the most common arrhythmia. The incidence increases markedly with advancing age. Thus, with the growing proportion of elderly individuals, atrial fibrillation will come to represent a significant medical and socioeconomic problem. The consequences of atrial fibrillation have the greatest impact. The risk of thromboembolism is well known; other outcomes of atrial fibrillation are less well recognised, such as its relationship with dementia, depression and death. Such consequences are responsible for diminished quality of life and considerable economic cost. Atrial fibrillation is characterised by rapid and disorganised atrial activity, with a frequency between 300 and 600 beats/minute. The ventricles react irregularly, and may contract rapidly or slowly depending on the health of the conduction system. Clinical symptoms are varied, including palpitations, syncope, dizziness or embolic events. Atrial fibrillation may be paroxysmal, persistent or chronic, and a number of attacks are asymptomatic. Suspicion or confirmation of atrial fibrillation necessitates investigation and, as far as possible, appropriate treatment of underlying causes such as hypertension, diabetes mellitus, hypoxia, hyperthyroidism and congestive heart failure. In the evaluation of atrial fibrillation, cardiac exploration is invaluable, including electrocardiogram (ECG) and echocardiography, with the aim of detecting cardiac abnormalities and directing management. In elderly patients (arbitrarily defined as aged >75 years), the management of atrial fibrillation varies; it requires an individual approach, which largely depends on comorbid conditions, underlying cardiac disease, and patient and physician preferences. This management is essentially based on pharmacological treatment, but there are also nonpharmacological options. Two alternatives are possible: restoration and maintenance of sinus rhythm, or control of ventricular rate, leaving the atria in arrhythmia. Pharmacological options include antiarrhythmic drugs, such as class III agents, beta-blockers and class IC agents. These drugs have some adverse effects, and careful monitoring is necessary. The nonpharmacological approach to atrial fibrillation includes external or internal direct-current cardioversion and new methods, such as catheter ablation of specific foci, an evolving science that has been shown to be successful in a very select group of atrial fibrillation patients. Another serious challenge in the management of chronic atrial fibrillation in older individuals is the prevention of stroke, its primary outcome, by choosing an appropriate antithrombotic treatment (aspirin or warfarin). Several risk-stratification schemes have been validated and may be helpful to determine the best antithrombotic choice in individual patients."}, {"id": "pubmed23n0935_5333", "title": "Emergency medicine considerations in atrial fibrillation.", "score": 0.01827849783981389, "content": "Atrial fibrillation (AF) is an abnormal heart rhythm which may lead to stroke, heart failure, and death. Emergency physicians play a role in diagnosing AF, managing symptoms, and lessening complications from this dysrhythmia. This review evaluates recent literature and addresses ED considerations in the management of AF. Emergency physicians should first assess patient clinical stability and evaluate and treat reversible causes. Immediate cardioversion is indicated in the hemodynamically unstable patient. The American Heart Association/American College of Cardiology, the European Society of Cardiology, and the Canadian Cardiovascular Society provide recommendations for management of AF. If hemodynamically stable, rate or rhythm control are options for management of AF. Physicians may opt for rate control with medications, with beta blockers and calcium channel blockers the predominant medications utilized in the ED. Patients with intact left ventricular function should be rate controlled to <110 beats per minute. Rhythm control is an option for patients who possess longer life expectancy and those with AF onset <48 h before presentation, anticoagulated for 3-4 weeks, or with transesophageal echocardiography demonstrating no intracardiac thrombus. Direct oral anticoagulants are a safe and reliable option for anticoagulation. Clinical judgment regarding disposition is recommended, but literature supports discharging stable patients who do not have certain comorbidities. Proper diagnosis and treatment of AF is essential to reduce complications. Treatment and overall management of AF include rate or rhythm control, cardioversion, anticoagulation, and admission versus discharge. This review discusses ED considerations regarding AF management."}, {"id": "pubmed23n0620_23588", "title": "Management of atrial fibrillation in the elderly.", "score": 0.018108816781383152, "content": "Atrial fibrillation (AF) is associated with a higher incidence of mortality, stroke, and coronary events than is sinus rhythm. AF with a rapid ventricular rate may cause a tachycardia-related cardiomyopathy. Immediate direct-current (DC) cardioversion should be performed in patients with AF and acute myocardial infarction, chest pain due to myocardial ischemia, hypotension, severe heart failure, or syncope. Intravenous beta blockers, diltiazem, or verapamil may be administered to slow immediately a very rapid ventricular rate in AF. An oral beta blocker, verapamil, or diltiazem should be used in persons with AF if a fast ventricular rate occurs at rest or during exercise despite digoxin. Amiodarone may be used in selected patients with symptomatic life-threatening AF refractory to other drugs. Digoxin should not be used to treat patients with paroxysmal AF. Nondrug therapies should be performed in patients with symptomatic AF in whom a rapid ventricular rate cannot be slowed by drugs. Paroxysmal AF associated with the tachycardia-bradycardia syndrome should be treated with a permanent pacemaker in combination with drugs. A permanent pacemaker should be implanted in patients with AF and symptoms such as dizziness or syncope associated with ventricular pauses greater than 3 seconds which are not drug-induced. Elective DC cardioversion has a higher success rate and a lower incidence of cardiac adverse effects than does medical cardioversion in converting AF to sinus rhythm. Unless transesophageal echocardiography has shown no thrombus in the left atrial appendage before cardioversion, oral warfarin should be given for 3 weeks before elective DC or drug cardioversion of AF and continued for at least 4 weeks after maintenance of sinus rhythm. Many cardiologists prefer, especially in older patients, ventricular rate control plus warfarin rather than maintaining sinus rhythm with antiar-rhythmic drugs. Patients with chronic or paroxysmal AF at high risk for stroke should be treated with long-term warfarin to achieve an International Normalized Ratio of 2.0 to 3.0. Patients with AF at low risk for stroke or with contraindications to warfarin should be treated with aspirin 325 mg daily."}, {"id": "pubmed23n0395_18245", "title": "Atrial fibrillation.", "score": 0.017862838915470493, "content": "The prevalence and incidence of atrial fibrillation increase with age. Atrial fibrillation is associated with a higher incidence of coronary events, stroke, and mortality than sinus rhythm. A fast ventricular rate associated with atrial fibrillation may cause tachycardia-related cardiomyopathy. Management of atrial fibrillation includes treatment of underlying causes and precipitating factors. Immediate direct-current cardioversion should be performed in persons with atrial fibrillation associated with acute myocardial infarction, chest pain due to myocardial ischemia, hypotension, severe heart failure, or syncope. Intravenous beta-blockers, verapamil, or diltiazem may be used to immediately slow a fast ventricular rate associated with atrial fibrillation. An oral beta-blocker, verapamil, or diltiazem should be given to persons with atrial fibrillation if a rapid ventricular rate occurs a rest or during exercise despite digoxin. Amiodarone may be used in selected persons with symptomatic life-threatening atrial fibrillation refractory to other drug therapy. Nondrug therapies should be performed in persons with symptomatic atrial fibrillation in whom a rapid ventricular rate cannot be slowed by drug therapy. Paroxysmal atrial fibrillation associated with the tachycardia-bradycardia syndrome should be managed with a permanent pacemaker in combination with drugs. A permanent pacemaker should be implanted in persons with atrial fibrillation in whom symptoms such as dizziness or syncope associated with non-drug-induced ventricular pauses longer than 3 seconds develop. Elective direct-current cardioversion has a higher success rate and a lower incidence of cardiac adverse effects than medical cardioversion in converting atrial fibrillation to sinus rhythm. Unless transesophageal echocardiography shows no thrombus in the left atrial appendage before cardioversion, oral warfarin should be given for 3 weeks before elective direct-current or drug cardioversion of atrial fibrillation and continued for at least 4 weeks after maintenance of sinus rhythm. Many cardiologists prefer the treatment strategy of ventricular rate control plus warfarin rather than to maintain sinus rhythm with antiarrhythmic drugs, especially in older patients. Digoxin should not be used in persons with paroxysmal atrial fibrillation. Patients with chronic or paroxysmal atrial fibrillation who are at high risk for stroke should be treated with long-term warfarin to achieve an International Normalized Ratio (INR) of 2.0 to 3.0. Persons with atrial fibrillation who are at low risk for stroke or who have contraindications to warfarin should receive 325 mg aspirin daily."}, {"id": "pubmed23n0344_3857", "title": "Management of the older person with atrial fibrillation.", "score": 0.01720573032048442, "content": "To review the management of the older person with atrial fibrillation (AF). A computer-assisted search of the English language literature (MEDLINE) database followed by a manual search of the bibliographies of pertinent articles. Studies on the management of persons with AF were screened for review. Studies of persons older than age 60 and recent studies were emphasized. Pertinent data were extracted from the reviewed articles. Emphasis was placed on studies involving older persons. Relevant articles were reviewed in depth. Available data about the management of persons with paroxysmal or chronic AF were summarized Management of AF includes treatment of the underlying disease and precipitating factors. Immediate direct-current cardioversion should be performed in persons with AF associated with an acute myocardial infarction, chest pain caused by myocardial ischemia, hypotension, severe heart failure, or syncope. Intravenous verapamil, diltiazem, or beta-blockers should be used to slow a very rapid ventricular rate associated with AF immediately. Oral verapamil, diltiazem, or a beta-blocker should be given if a rapid ventricular rate occurs at rest or during exercise despite digoxin. Amiodarone may be used in selected persons with symptomatic life-threatening AF refractory to other drug therapy. Nondrug therapies should be performed in persons with symptomatic AF in whom a rapid ventricular rate cannot be slowed by drug therapy. Paroxysmal AF associated with the tachycardia-bradycardia syndrome should be treated with a permanent pacemaker in combination with drugs. A permanent pacemaker should be implanted in persons with AF who develop cerebral symptoms such as dizziness or syncope associated with ventricular pauses greater than 3 seconds that are not drug-induced. Elective cardioversion of AF should not be performed in asymptomatic older persons with chronic AF. Unless transesophageal echocardiography has shown no thrombus in the left atrial appendage before cardioversion, oral warfarin should be given for 3 weeks before elective direct-current or drug cardioversion of AF and continued for at least 4 weeks after maintenance of sinus rhythm. Many cardiologists prefer the treatment strategy, especially in older persons, of ventricular rate control plus warfarin rather than maintaining sinus rhythm with antiarrhythmic drugs. Digoxin should be avoided in persons with sinus rhythm who have a history of paroxysmal AF. Older persons with chronic or paroxysmal AF who are at high risk for stroke or who have a history of hypertension and no contraindications to warfarin should receive long-term warfarin to achieve an International Normalized Ratio of 2.0 to 3.0. Older persons with AF who are at low risk for stroke or who have contraindications to warfarin should receive 325 mg of aspirin daily."}, {"id": "pubmed23n0317_7323", "title": "Treatment strategies for atrial fibrillation.", "score": 0.017158294392523366, "content": "Atrial fibrillation is the most common arrhythmia observed in clinical practice, occurring in 0.4% of the general population and in up to 4% of people greater than 60 years old. It is often associated with other cardiovascular disorders, such as hypertension, coronary artery disease, or cardiomyopathy. Critical evaluation and management of patients with atrial fibrillation requires knowledge of etiology, prognosis, and treatment options of this arrhythmia. On initial presentation, emergency electrical cardioversion should be performed if the patient is hemodynamically unstable. If the patient is stable, initial rate control is recommended, using atrioventricular nodal blocking agents. Further treatment mainly depends upon the duration of the episode. Patients who are in atrial fibrillation <48 hours can be safely cardioverted. Patients who are in atrial fibrillation for >48 hours are commonly anticoagulated for 3 to 4 weeks before and after cardioversion because of the risk of thromboembolism formation in the left atrial appendage. An alternate strategy, which is especially attractive when immediate cardioversion is desired, is transesophageal echocardiography to exclude left atrial thrombus followed by prompt cardioversion. After cardioversion, sinus rhythm can be maintained with class I and III drugs, such as flecainide and propafenone or amiodarone and sotalol. New treatment options, such as atrial defibrillation, atrioventricular junctional ablation, or modification of atrial pacing to prevent atrial fibrillation, are currently under investigation. Although atrial fibrillation is so common in clinical practice, it still remains difficult to treat. Conversion and maintenance to sinus rhythm with antiarrhythmic drug therapy has not shown any improvement in mortality, and some patients may benefit more from ventricular rate control. This review article discusses different treatment strategies for patients with atrial fibrillation."}, {"id": "pubmed23n0273_6468", "title": "Optimal management of older patients with atrial fibrillation.", "score": 0.016993087557603686, "content": "Long term oral warfarin should be administered to elderly patients with atrial fibrillation who are at high risk for developing thromboembolic stroke and who have no contraindications to anticoagulant therapy. Oral aspirin (acetylsalicylic acid) 325mg daily may be given to elderly patients with chronic atrial fibrillation who have contraindications to anticoagulant therapy or who are not at high risk for developing thromboembolic stroke. Management of atrial fibrillation includes treatment of the underlying disease and precipitating factors. If patients have paroxysmal atrial fibrillation with a very rapid ventricular rate associated with hypotension, severe left ventricular failure or chest pain due to myocardial ischaemia, immediate direct-current cardioversion should be performed. Intravenous verapamil, diltiazem or a beta-blocker should be used for immediate slowing of a very rapid ventricular rate associated with atrial fibrillation. If a rapid ventricular rate associated with atrial fibrillation persists at rest or during exercise despite digoxin, then oral verapamil, diltiazem or a beta-blocker should be added. Low dosages of oral amiodarone (200 to 400 mg/day) may be used in selected patients with symptomatic life-threatening atrial fibrillation refractory to other therapy. No medication which depresses atrioventricular conduction should be given to patients with atrial fibrillation and a slow ventricular rate. Cardioversion should not be performed in asymptomatic elderly patients with chronic atrial fibrillation. This author would use a beta-blocker for control of ventricular arrhythmias and following conversion of atrial fibrillation to sinus rhythm. Should atrial fibrillation recur, beta-blockers have the additional advantage of slowing the ventricular rate."}, {"id": "pubmed23n0906_1992", "title": "Atrial Fibrillation: The New Epidemic of the Ageing World.", "score": 0.016736694677871148, "content": "The prevalence of atrial fibrillation (AF) increases with age. As the population ages, the burden of AF increases. AF is associated with an increased incidence of mortality, stroke, and coronary events compared to sinus rhythm. AF with a rapid ventricular rate may cause a tachycardia-related cardiomyopathy. Immediate direct-current (DC) cardioversion should be performed in patients with AF and acute myocardial infarction, chest pain due to myocardial ischemia, hypotension, severe heart failure, or syncope. Intravenous beta blockers, diltiazem, or verapamil may be administered to reduce immediately a very rapid ventricular rate in AF. An oral beta blocker, verapamil, or diltiazem should be used in persons with AF if a fast ventricular rate occurs at rest or during exercise despite digoxin. Amiodarone may be used in selected patients with symptomatic life-threatening AF refractory to other drugs. Digoxin should not be used to treat patients with paroxysmal AF. Nondrug therapies should be performed in patients with symptomatic AF in whom a rapid ventricular rate cannot be slowed by drugs. Paroxysmal AF associated with the tachycardia-bradycardia syndrome should be treated with a permanent pacemaker in combination with drugs. A permanent pacemaker should be implanted in patients with AF and symptoms such as dizziness or syncope associated with ventricular pauses greater than 3 seconds which are not drug-induced. Elective DC cardioversion has a higher success rate and a lower incidence of cardiac adverse effects than does medical cardioversion in converting AF to sinus rhythm. Unless transesophageal echocardiography has shown no thrombus in the left atrial appendage before cardioversion, oral warfarin should be given for 3 weeks before elective DC or drug cardioversion of AF and continued for at least 4 weeks after maintenance of sinus rhythm. Many cardiologists prefer, especially in elderly patients , ventricular rate control plus warfarin rather than maintaining sinus rhythm with antiarrhythmic drugs. Patients with chronic or paroxysmal AF at high risk for stroke should be treated with long-term warfarin to achieve an International Normalized Ratio of 2.0 to 3.0. Patients with AF at low risk for stroke or with contraindications to warfarin should be treated with aspirin 325 mg daily."}, {"id": "pubmed23n0344_2804", "title": "Management of atrial fibrillation: out-of-hospital approach.", "score": 0.016108164057445983, "content": "Atrial fibrillation is increasingly common with advancing age and is responsible for 10% of the half-million strokes that occur annually in the United States. When a patient presents with atrial fibrillation, the physician's first task is to use the history, physical examination, and electrocardiogram to determine whether hospitalization is necessary. Factors indicating a need for hospital care include evidence of infarction or ischemia, congestive heart failure, hypotension or hypoperfusion, excessive rate, or pre-excitation. In addition, if the episode began within 48 hours, consider early cardioversion, which also requires hospitalization. Next, the need for control of the ventricular rate should be assessed. A heart rate under 90 beats/min at rest and under 120 beats/min after 1 minute of step exercise is a reasonable goal. Dixogin usually controls the resting rate, but sometimes beta-blockers or calcium channel blockers are needed to control the exercise rate. The need for anticoagulation is determined by the presence of clinical risk factors such as valvular heart disease, previous thromboembolism, hypertension, age over 65 years, congestive heart failure, and left atrial enlargement. An echocardiogram is necessary to complete this assessment. Patients having one or more of these risk factors are most effectively treated with warfarin, as evident from several clinical trials. Although patients over age 65 demonstrate reduced thromboembolism with warfarin therapy, they also are more prone to cerebral hemorrhage, thus, their international normalization ratio (INR) should be kept at the lower end of the therapeutic range [2,3]. Other patients can be treated with aspirin, although stroke reduction in these patients may be more related to reduction of arterial thrombosis than thromboembolism. Patients under age 65 with no risk factors have a very low annual risk of stroke without therapy (approximately 1%). If symptoms persist or if this is a first episode in someone without left atrial enlargement, cardioversion can be considered after 3 weeks of warfarin therapy with INR in the therapeutic range. Otherwise, warfarin should be continued indefinitely. Prevention of recurrence with antiarrhythmic drugs is somewhat problematic because of incomplete efficacy (30% recurrence at 1 year) and the potential for inducing other, life-threatening arrhythmias."}, {"id": "pubmed23n0480_9205", "title": "Management of newly detected atrial fibrillation: a clinical practice guideline from the American Academy of Family Physicians and the American College of Physicians.", "score": 0.01597082494969819, "content": "The Joint Panel of the American Academy of Family Physicians and the American College of Physicians, in collaboration with the Johns Hopkins Evidence-based Practice Center, systematically reviewed the available evidence on the management of newly detected atrial fibrillation and developed recommendations for adult patients with first-detected atrial fibrillation. The recommendations do not apply to patients with postoperative or post-myocardial infarction atrial fibrillation, patients with class IV heart failure, patients already taking antiarrhythmic drugs, or patients with valvular disease. The target physician audience is internists and family physicians dedicated to primary care. The recommendations are as follows: RECOMMENDATION 1: Rate control with chronic anticoagulation is the recommended strategy for the majority of patients with atrial fibrillation. Rhythm control has not been shown to be superior to rate control (with chronic anticoagulation) in reducing morbidity and mortality and may be inferior in some patient subgroups to rate control. Rhythm control is appropriate when based on other special considerations, such as patient symptoms, exercise tolerance, and patient preference. Grade: 2A. RECOMMENDATION 2: Patients with atrial fibrillation should receive chronic anticoagulation with adjusted-dose warfarin, unless they are at low risk of stroke or have a specific contraindication to the use of warfarin (thrombocytopenia, recent trauma or surgery, alcoholism). Grade: 1A. RECOMMENDATION 3: For patients with atrial fibrillation, the following drugs are recommended for their demonstrated efficacy in rate control during exercise and while at rest: atenolol, metoprolol, diltiazem, and verapamil (drugs listed alphabetically by class). Digoxin is only effective for rate control at rest and therefore should only be used as a second-line agent for rate control in atrial fibrillation. Grade: 1B. RECOMMENDATION 4: For those patients who elect to undergo acute cardioversion to achieve sinus rhythm in atrial fibrillation, both direct-current cardioversion (Grade: 1C+) and pharmacological conversion (Grade: 2A) are appropriate options. RECOMMENDATION 5: Both transesophageal echocardiography with short-term prior anticoagulation followed by early acute cardioversion (in the absence of intracardiac thrombus) with postcardioversion anticoagulation versus delayed cardioversion with pre- and postanticoagulation are appropriate management strategies for those patients who elect to undergo cardioversion. Grade: 2A. RECOMMENDATION 6: Most patients converted to sinus rhythm from atrial fibrillation should not be placed on rhythm maintenance therapy since the risks outweigh the benefits. In a selected group of patients whose quality of life is compromised by atrial fibrillation, the recommended pharmacologic agents for rhythm maintenance are amiodarone, disopyramide, propafenone, and sotalol (drugs listed in alphabetical order). The choice of agent predominantly depends on specific risk of side effects based on patient characteristics. Grade: 2A."}, {"id": "wiki20220301en293_5632", "title": "Management of atrial fibrillation", "score": 0.014520902700702921, "content": "The main risk of cardioversion is systemic embolization of a thrombus (blood clot) from the previously fibrillating left atrium. Cardioversion should not be performed without adequate anticoagulation in patients with more than 48 hours or unknown duration of AF. Anticoagulation is adequate if warfarin is given with target INR between 2 and 3 for three to four weeks prior to cardioversion, and continued for at least four weeks after cardioversion. Cardioversion may be performed in instances of AF lasting more than 48 hours if a transesophogeal echocardiogram (TEE) demonstrates no evidence of clot within the heart. Whichever method of cardioversion is used, approximately 50% of patients relapse within one year, although the continued daily use of oral antiarrhythmic drugs may extend this period. The key risk factor for relapse is duration of AF, although other risk factors that have been identified include the presence of structural heart disease, and old age."}, {"id": "pubmed23n1084_2592", "title": "Acute Tachycardia-Induced Cardiomyopathy: A Case Report.", "score": 0.013492063492063493, "content": "BACKGROUND Tachycardia from atrial fibrillation or flutter can lead to left ventricular systolic dysfunction. Some patients deteriorate quickly, and there is an acute drop in their left ventricular systolic function; however, they tend to normalize rapidly after treatment of the underlying arrhythmia. The aim of publishing the present case is to maintain awareness that tachycardia is one of the etiologies of acute systolic heart failure, which is potentially reversible by treatment when recognized. CASE REPORT An 88-year-old woman with a history of hypertension and diabetes presented to the emergency department with shortness of breath and new-onset atrial fibrillation. The physical examination revealed jugular vein distention, an irregular heart rate of approximately 140 beats/min, bilateral basal lung crackles, and no murmurs. One week before this presentation, she underwent electrocardiography, which showed she was in sinus rhythm, and transthoracic echocardiography, which indicated an ejection fraction of 65%. After hospital admission, she was started on beta-blockers for heart rate control and diuretics for heart failure management. As her symptoms persisted, she underwent a transesophageal echocardiography-guided cardioversion, where her ejection fraction was 30%. A repeat transthoracic echocardiography 3 days after the cardioversion indicated the ejection fraction had normalized to 60%. She was followed up every month in the Outpatient Cardiology Clinic and has remained asymptomatic for 1 year to date. CONCLUSIONS Although most literature describes tachycardia-induced cardiomyopathy as a chronic process, it can be acute. Patients benefit from rhythm control, and with early diagnosis and appropriate management, the prognosis is good."}, {"id": "pubmed23n0837_1889", "title": "Treatment of Atrial Fibrillation.", "score": 0.013233752620545073, "content": "Atrial fibrillation is a common arrhythmia that affects more than 2.5 million people in the United States and causes substantial morbidity and mortality, especially regarding the increased risk of stroke. To summarize atrial fibrillation treatment exclusive of stroke prevention. An Ovid MEDLINE comprehensive literature search was performed on atrial fibrillation therapy excluding anticoagulation and emphasizing studies published within the last 5 years through April 2015 (N = 5044 references). The 2014 atrial fibrillation guideline from the American Heart Association, the American College of Cardiology, and the Heart Rhythm Society also was reviewed. Reversible causes of atrial fibrillation should be identified. Risk factor modification, including weight loss and treatment of hypertension, diabetes, and obstructive sleep apnea can reduce atrial fibrillation episodes. Appropriate anticoagulation is necessary for patients at substantial stroke risk regardless of rate or rhythm treatment strategy. Sinus rhythm is often needed to control symptoms; however, an alternative strategy for atrial fibrillation is appropriate rate control. Rate control is safe in older patients (those who are about age ≥65 years) followed up for a few years, but no such safety data exist for patients younger than 60 years or for those followed up for longer periods. Thus, selection of therapy is individualized, taking into account present and future medical problems for the patient. Choice of an antiarrhythmic drug is based on safety first vs efficacy. Catheter ablation is an effective nonpharmacological alternative that is often, but not always, the second-line treatment. Reduction of the frequency and duration of atrial fibrillation episodes that result in a significant improvement in quality of life is a good marker of drug treatment success and complete elimination of atrial fibrillation is not required in many patients. Rate control is usually achieved with a β-blocker or non-dihydropyridine calcium channel blockers. It is important to assess adequate rate control during both rest and activity. If the ventricular rate goes uncontrolled for a prolonged period, tachycardia-mediated cardiomyopathy can occur. Therapy for atrial fibrillation includes prevention and modification of inciting causes and appropriate anticoagulation. Rate control is necessary for all patients. Maintenance of sinus rhythm with drugs or catheter ablation should be considered based on the individual needs of each patient."}, {"id": "pubmed23n1032_14924", "title": "[Combined Approach for Management of the Chronic Heart Failure with Preserved Left Ventricular Ejection Fraction and Permanent Atrial Fibrillation: a Case Report].", "score": 0.0132150930272996, "content": "The article described a clinical case of a patient with chronic heart failure (CHF) with preserved ejection fraction (CHF-PEF) and permanent normosystolic atrial fibrillation (AF). A 73 year-old man (body mass index, 26.4 kg /m2) with permanent normosystolic AF (duration, 10 years) was hospitalized for augmenting of CHF symptoms. The patient had NYHA II-III functional class CHF and a history of long-standing arterial hypertension. The patient received chronic therapy according to the effective guidelines (angiotensin receptor blockers, diuretics, beta-blockers, and new oral anticoagulants). Transthoracic echocardiography showed a normal ejection fraction (EF) (57 %), a moderate enlargement of the left atrium (48 mm), and moderate left ventricular (LV) hypertrophy. Radiofrequency catheter ablation (RFCA) of left atrial AF was performed. For preparation to the RFCA, the patient was administered propanorm two weeks prior to the procedure. Following external electrical cardioversion (ECV) after RFCA, sinus rhythm did not recover. The patient was prescribed amiodarone, and repeat ECV was performed in a month, which resulted in successful recovery of sinus rhythm. However, due to an increase in serum thyrotropic hormone, amiodaron was replaced with the sotalol therapy (240 mg/day). This resulted in development of symptomatic sinus bradycardia and AF relapse at 3 days after ECV. A dual-chamber cardioverter defibrillator was implanted to the patient; in another three months, repeat AF RFCA was performed with successful recovery of sinus rhythm. During the cardioverter testing for one year, the patient had one more AF episode, which was stopped by external ECV. Also, a 6-hour AF episode occurred at three months after the repeat RFCA. Symptoms of CHF disappeared by the 12th month. The combination therapy administered to the patient with normosystolic permanent AF and preserved EF, which included a pathogenetic therapy for CHF, antiarrhythmic drugs, implantation of a dual-chamber ECV, two sessions of AF RFCA, and repeat external ECVs, provided considerable improvement of CHF symptoms and stable sinus rhythm during a one-year follow-up. The return to sinus rhythm after 10 years of permanent AF necessitated changing the arrhythmia diagnosis to long-standing, persistent AF."}, {"id": "pubmed23n0906_2236", "title": "Cardioversion in Acute Atrial Fibrillation Without Anticoagulation.", "score": 0.013146551724137931, "content": "A major concern in cardioversion of newly detected atrial fibrillation is the risk of thromboembolic events. The vast majority of these events occur in the first week following cardioversion. Transesophageal echocardiography has demonstrated that thrombus and dense spontaneous echo contrast may occur in the left atrium and left atrial appendage in patients with acute atrial fibrillation (<48 hours) scheduled for cardioversion. Moreover, atrial function may become impaired immediately following successful cardioversion. The risk of thromboembolic events increases with the presence of stroke risk factors, such as heart failure, hypertension, diabetes, prior stroke, female sex and age above 65-75 years. Thus, the current guidelines of the ESC and ACC/AHA/Heart Rhythm Society recommend that patients with acute atrial fibrillation should undergo cardioversion under cover of unfractionated or low-molecular weight heparin followed by oral anticoagulation for at least 4 weeks in patients in patients at moderate-to-high risk for stroke. In line with the guidelines, new evidence from a large patient population suggests that after successful cardioversion of acute atrial fibrillation, patients have a low overall risk of thromboembolic events without any anticoagulation when they have no risk factors for thromboembolism. In contrast, the risk is in the range of 10% in patients with multiple classic risk factors for thromboembolism."}, {"id": "wiki20220301en293_5610", "title": "Management of atrial fibrillation", "score": 0.013116424182611188, "content": "The management of atrial fibrillation (AF) is focused on preventing temporary circulatory instability, stroke and other ischemic events. Control of heart rate and rhythm are principally used to achieve the former, while anticoagulation may be employed to decrease the risk of stroke. Within the context of stroke, the discipline may be referred to as stroke prevention in atrial fibrillation (SPAF). In emergencies, when circulatory collapse is imminent due to uncontrolled rapid heart rate, immediate cardioversion may be indicated. The primary factors determining AF treatment are duration and evidence of circulatory instability. Cardioversion is indicated with new onset AF (for less than 48 hours) and with circulatory instability. If rate and rhythm control cannot be maintained by medication or cardioversion, it may be necessary to perform electrophysiological studies with ablation of abnormal electrical pathways."}, {"id": "pubmed23n0309_15266", "title": "[Anticoagulation and electrical cardioversion of chronic atrial fibrillation: proposal for an abbreviated protocol].", "score": 0.012565525747764416, "content": "Several weeks of prophylactic anticoagulation are routinely prescribed before and after electrical cardioversion of atrial fibrillation. Recent studies have supported the use of transesophageal echocardiography to guide early cardioversion: patients in whom no thrombus is observed are treated with heparin followed by one month of warfarin therapy after the procedure. This kind of treatment requires hospital admission during heparin infusion, because of the need for monitoring partial thromboplastin time. To evaluate if a short at-home treatment (three days) with warfarin is sufficient to reach a good level of anticoagulation, in order to permit safe electrical cardioversion in day-hospital for patients who show no thrombi on transesophageal echocardiography. One hundred twenty-four patients with atrial fibrillation, who were candidates for cardioversion, were treated with warfarin: 10 mg the first and second day, 5 mg the third day in group A patients (n = 79); 15 mg the first day, 10 mg the second and third day in group B patients (n = 45). On the fourth day, INR value was measured and if it was < 2, warfarin therapy was prolonged until patients reached a good level of anticoagulation. Transesophageal echocardiography was performed when the INR was > or = 2, and patients were cardioverted with DC shock if there were no thrombi. The patients were discharged on the same day of the procedure, and warfarin therapy was continued for 4 weeks there-after. If a thrombus was detected, patients repeated transesophageal echocardiography after 6 weeks of warfarin therapy, and were cardioverted if the thrombus disappeared. Otherwise, cardioversion was deferred and they received prolonged warfarin treatment. If there was poor visualization of the left atrial appendage, patients received conventional warfarin therapy for 3 weeks before and 4 weeks after electrical cardioversion. Mean INR value after three days of warfarin treatment was 2.41 in group A patients and 3.02 in group B patients. Twenty-one patients from group A and 3 patients from group B required anticoagulant therapy for a mean of 3.3 and 5.1 days, respectively, before reaching a good level of anticoagulation (INR value > or = 2). Eight patients reverted spontaneously to sinus rhythm before transesophageal echocardiography. Eighteen thrombi (15.5%) were identified on the transesophageal echocardiography, all of which were in the left atrial appendage. In 11 cases, thrombus disappeared after 6 weeks of warfarin therapy. In 7 patients (6%), the atrial appendage was not sufficiently visualized. Electrical cardioversion was performed on 109 patients and was successful in 88 (80.7%). None of them experienced a clinical thromboembolic event. In the majority of patients in atrial fibrillation, a short at-home warfarin treatment is sufficient to reach a good level of anticoagulation in order to permit safe electrical cardioversion in a day-hospital situation. Larger initial doses can achieve even better results. This treatment algorithm minimizes the anticoagulation period, hospital stay, overall duration of atrial fibrillation and the time required for the mechanical function of the left atrium to return."}, {"id": "pubmed23n0502_13591", "title": "Antithrombotic therapy in atrial fibrillation: the Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy.", "score": 0.012403993855606758, "content": "This chapter about antithrombotic therapy in atrial fibrillation (AF) is part of the Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy: Evidence Based Guidelines. Grade 1 recommendations are strong and indicate that the benefits do, or do not, outweigh risks, burden, and costs. Grade 2 suggests that individual patients' values may lead to different choices (for a full understanding of the grading see Guyatt et al, CHEST 2004; 126:179S-187S). Among the key recommendations in this chapter are the following (all vitamin K antagonist [VKA] recommendations have a target international normalized ratio [INR] of 2.5; range, 2.0 to 3.0): In patients with persistent or paroxysmal AF (PAF) [intermittent AF] at high risk of stroke (ie, having any of the following features: prior ischemic stroke, transient ischemic attack, or systemic embolism, age > 75 years, moderately or severely impaired left ventricular systolic function and/or congestive heart failure, history of hypertension, or diabetes mellitus), we recommend anticoagulation with an oral VKA, such as warfarin (Grade 1A). In patients with persistent AF or PAF, age 65 to 75 years, in the absence of other risk factors, we recommend antithrombotic therapy with either an oral VKA or aspirin, 325 mg/d, in this group of patients who are at intermediate risk of stroke (Grade 1A). In patients with persistent AF or PAF < 65 years old and with no other risk factors, we recommend aspirin, 325 mg/d (Grade 1B). For patients with AF and mitral stenosis, we recommend anticoagulation with an oral VKA (Grade 1C+). For patients with AF and prosthetic heart valves, we recommend anticoagulation with an oral VKA (Grade 1C+); the target INR may be increased and aspirin added depending on valve type and position, and on patient factors. For patients with AF of > or = 48 h or of unknown duration for whom pharmacologic or electrical cardioversion is planned, we recommend anticoagulation with an oral VKA for 3 weeks before and for at least 4 weeks after successful cardioversion (Grade 1C+). For patients with AF of > or = 48 h or of unknown duration undergoing pharmacologic or electrical cardioversion, an alternative strategy is anticoagulation and screening multiplane transesophageal echocardiography (Grade 1B). If no thrombus is seen and cardioversion is successful, we recommend anticoagulation for at least 4 weeks (Grade 1B). For patients with AF of known duration < 48 h, we suggest cardioversion without anticoagulation (Grade 2C). However, in patients without contraindications to anticoagulation, we suggest beginning IV heparin or low molecular weight heparin at presentation (Grade 2C)."}, {"id": "wiki20220301en248_20356", "title": "Atrial fibrillation", "score": 0.011924853437555517, "content": "If a blood clot is seen on TEE, then cardioversion is contraindicated due to the risk of stroke, and anticoagulation is recommended. Ambulatory Holter monitoring A Holter monitor is a wearable ambulatory heart monitor that continuously monitors the heart rate and heart rhythm for a short duration, typically 24 hours. In individuals with symptoms of significant shortness of breath with exertion or palpitations regularly, a Holter monitor may be of benefit to determine whether rapid heart rates (or unusually slow heart rates) during atrial fibrillation are the cause of the symptoms."}, {"id": "wiki20220301en009_184622", "title": "Beta blocker", "score": 0.01190172032021802, "content": "Hyperthyroidism Abrupt withdrawal can result in a thyroid storm. Bradycardia or AV block Unless a pacemaker is present, beta blockers can severely depress conduction in the AV node, resulting in a reduction of heart rate and cardiac output. One should be very cautious with the use of beta blockers in tachycardic patients with Wolff-Parkinson-White Syndrome, as it can result in life-threatening arrhythmia in certain patients. By slowing the conduction through the AV node, preferential conduction through the accessory pathway is favored. If the patient happens to develop atrial flutter, this could lead to a 1:1 conduction with very fast ventricular rate, or worse, ventricular fibrillation in the case of atrial fibrillation."}, {"id": "pubmed23n0839_2918", "title": "Rate control versus rhythm control in atrial fibrillation: lessons learned from clinical trials of atrial fibrillation.", "score": 0.011817688204669583, "content": "Ample evidence supports the statement that in patients with atrial fibrillation in whom treatment is warranted, either rhythm control or rate control are acceptable primary therapeutic options. If a rhythm control strategy is chosen, it is important to consider that recurrence of atrial fibrillation is not treatment failure per se. Occasional recurrence, with cardioversion if necessary, may be quite acceptable. The latter will depend on the frequency, duration and symptoms associated with recurrence, and may require a change in the rhythm control therapy, e.g., change the antiarrhythmic drug, or initiate or redo atrial fibrillation ablation. And a rhythm control strategy should include careful attention to and treatment of comorbidities (hypertension, heart failure, diabetes, etc.). If a rate control strategy is chosen, treatment with a beta blocker or nondihydropyridine calcium channel blocker is almost always required to achieve adequate rate control. Digoxin is often useful to obtain satisfactory rate control in combination with a beta blocker or nondihydropyridine calcium channel blocker. Digoxin may be useful as primary therapy in the presence of hypotension or heart failure. Satisfactory ventricular rate control is usually a resting rate less than 110 beats per minute, although resting rates below 90 beats per minute are probably wiser. Finally, when pursuing a rhythm control strategy, because recurrence of atrial fibrillation is common, rate control therapy should be a part of the treatment regimen. "}, {"id": "pubmed23n0356_3911", "title": "Systematic review of the management of atrial fibrillation in patients with heart failure.", "score": 0.011611219830397913, "content": "To systematically review the management of atrial fibrillation (AF) in patients with heart failure. Studies investigating the management of AF in patients with heart failure published between 1967 to 1998 were identified using MEDLINE, the Cochrane register and Embase databases. Reference lists from relevant papers and reviews were hand searched for further papers. Eight studies pertaining to acute and twenty-four pertaining to chronic AF were identified. For patients with acute AF ventricular rate control, anticoagulation and treatment of heart failure should be pursued simultaneously before cardioversion is attempted. Digoxin is relatively ineffective at controlling ventricular response and for cardioversion. Intravenous diltiazem is rapidly effective in controlling ventricular rate and limited evidence suggests it is safe. Amiodarone controls ventricular rate rapidly and increases the rate of cardioversion. There are insufficient data to conclude that immediate anti-coagulation, trans-oesophageal echocardiography to exclude atrial thrombi followed by immediate cardioversion is an appropriate strategy. Patients with chronic AF should be anti-coagulated unless contra-indications exist. It is not clear whether the preferred strategy should be cardioversion and maintenance of sinus rhythm with amiodarone or ventricular rate control of AF combined with anticoagulation to improve outcome including symptoms, morbidity and survival. Electrical cardioversion has a high initial success rate but there is also a high risk of early relapse. Amiodarone currently appears the most effective and safest therapy for maintaining sinus rhythm post-cardioversion. Digoxin is fairly ineffective at controlling ventricular rate during exercise. Addition of a beta-blocker reduces ventricular rate and improves symptoms. Whether digoxin is required in addition to beta-blockade for the control of AF in this setting is currently under investigation. If pharmacological therapy is ineffective or not tolerated then atrio-ventricular node ablation and permanent pacemaker implantation should be considered. There is a paucity of controlled clinical trial data for the management of AF among patients with heart failure. The interaction between AF and heart failure means that neither can be treated optimally without treating both. Presently treatment should be on a case by case basis."}, {"id": "pubmed23n0526_24559", "title": "Atrial fibrillation and heart failure comorbidity.", "score": 0.011610688564313118, "content": "Atrial fibrillation and heart failure have in common that they mainly occur in older patients and the patients have similar underlying heart diseases. The prevalence of atrial fibrillation in heart failure patients varies from 10% to 30%. There are conflicting data whether the presence of atrial fibrillation is an independent predictor for an increased mortality in heart failure. Optimal medical heart failure therapy can improve outcome and may influence the relationship between atrial fibrillation and survival. Keystones for the management of atrial fibrillation in heart failure patients are the optimal treatment of heart failure, the use of oral anticoagulation, the case-adjusted decision of rhythm or rate control, and the primary prevention of sudden cardiac death. Heart failure patients with atrial fibrillation should receive long-term oral anticoagulation. The two options to treat atrial fibrillation are rhythm control and rate control. Given the findings of randomised trials, rhythm control of atrial fibrillation with the aim to improve survival is not justified in heart failure patients because of uncertainty about the role of atrial fibrillation as a predictor of worse outcomes and the safety of antiarrhythmic drugs. Rhythm control can be attempted, if rate control is chosen and symptoms persist. The indications for rhythm control are to control symptoms, including a deterioration of heart failure related to a loss of atrial contraction. Amiodarone seems to be the drug of choice to maintain sinus rhythm in patients with paroxysmal atrial fibrillation as well as in patients who returned to sinus rhythm after cardioversion. New non pharmacologic approaches for rhythm control such as catheter-based techniques seem to be highly effective. Rate control to prevent rapid atrial fibrillation is an acceptable approach in otherwise asymptomatic heart failure patients. Slowing of the ventricular rate often leads to a moderate improvement in left ventricular function in many patients. Standard therapy for rate control in heart failure patients consists of partial atrioventricular (AV) node blockade with digoxin and a beta-blocker. Amiodarone is also highly effective to reduce ventricular rate in patients with atrial fibrillation. When rate control remains refractory to medical therapy, rate control is achieved with AV node ablation and subsequent pacemaker implantation. Non pharmacological treatments for the primary prevention of sudden cardiac death are the implantation of a defibrillator."}, {"id": "pubmed23n0536_2362", "title": "Atrial fibrillation.", "score": 0.01138425519461516, "content": "In 2000, some 2.3 million Americans were affected by atrial fibrillation, and that number is expected to rise as our population ages. Atrial fibrillation is both a reflection of active physiologic stressors on the body and a marker of future cardiac disease progression. The disorganized atrial activity that characterizes atrial fibrillation affects cardiac function, metabolic demand, and quality of life. However, our understanding of the etiology and treatment of this condition continues to advance with the result of recent large-scale clinical trials. Diabetes, hypertension, congestive heart failure, valvular disease, and myocardial infarction are all risk factors in the development of atrial fibrillation. And the diagnosis confers a five-fold increase in the incidence of stroke. (Patients at increased risk for stroke include those with congestive heart failure, hypertension, age greater than 75, diabetes, and previous stroke.) Anticoagulation is a critical action in most cases of atrial fibrillation, as data show a 68% relative risk reduction of stroke when patients are treated with warfarin. Prior to recent trials, achieving sinus rhythm was thought to invariably improve symptoms, cardiac function, and mortality. The adverse effects of antiarrhythmic medications are now being recognized, and treatment strategies emphasizing ventricular rate control have been recommended in recent clinical practice guidelines. This shift in thinking is influencing both outpatient and emergency department management. Controlling the ventricular rate in atrial fibrillation increases cardiac output, decreases the metabolic demand of the heart, and avoids the potentially dangerous side effects of rhythm-control drugs. Rate-control agents should be selected based on the clinical profile of individual patients. A well-chosen subset of patients may benefit from either chemical or electrical cardioversion; this appears to be a reasonably safe procedure and can be accomplished on an outpatient basis. Understanding causal etiologies, managing risk for stroke (and need for anticoagulation), addressing rate, and assessing the risks of cardioversion are key elements in a comprehensive approach to atrial fibrillation."}, {"id": "article-17962_19", "title": "Atrial Fibrillation -- Treatment / Management", "score": 0.011257328990228013, "content": "The management of atrial fibrillation in the acute setting depends on hemodynamic stability and risk stratification. In cases where the patient is hemodynamically unstable, it is recommended to carry out immediate cardioversion with anticoagulant therapy. Although TEE is recommended before any cardioversion; however, if the patient is hemodynamically unstable due to a rapid ventricular response, cardioversion may be indicated without prior TEE. If there is evidence of rapid ventricular response, a beta-blocker or calcium-channel blocker should be commenced for rate control. These options can be used in the intravenous (IV) form for rapid response. Usually, a bolus is administered to the patient and then started on a drip if symptoms do not resolve. Digoxin can be considered for rate control but is not advised as a first-line agent pertaining to its adverse effects and tolerance. Amiodarone can also be given as a rhythm control agent but is also not a first-line option in the acute setting. In any case, if the decision to start amiodarone is made, cardiology should be consulted before its administration."}, {"id": "pubmed23n0259_8610", "title": "Presentation and management of patients admitted with atrial fibrillation: a review of 291 cases in a regional hospital.", "score": 0.011127940403696948, "content": "Two hundred and ninety one patients admitted with atrial fibrillation through the emergency room of a regional hospital in the year 1993 were reviewed to evaluate the presenting features and in-hospital treatment of patients with symptomatic atrial fibrillation. The incidence of atrial fibrillation increased with age (mean age was 73 +/- 12 years) and the ratio of female to male was 1.8:1. The commonest presenting features were palpitation (42.3%), dyspnoea (38.1%) and heart failure (16.4%). The most frequently associated cardiac conditions were hypertension (28.9%), atherosclerotic cardiovascular disease (24.7%) and rheumatic heart disease (17.5%). Pulmonary diseases (18.6%), diabetes mellitus (12.7%) and thyrotoxicosis (6.2%) were the principal associated non-cardiac conditions. Thromboembolic complications were found in 15 patients at presentation (5.2%). Cardiac enzyme assessment was investigated in two thirds of the patients (68.1%), while thyroid function test (59.5%) and echocardiography (29.6%) were less commonly investigated. Digoxin was still the most popular drug used for ventricular rate control, and cardioversion was performed in only 6.9% of patients. Antithrombotic therapy was used in 5.8% of patients only although it was clinically indicated in more than half of the patients (52%). Contraindications of anticoagulation were found in 23 patients (7.9%), including a history of gastrointestinal or cerebrovascular bleeding, active bleeding, chronic renal failure and poor drug compliance. The mean hospital stay was 5 +/- 4 days, compared to a mean stay of 2.7 days for other medical patients. Fourteen patients (4.8%) died during hospitalisation.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en248_20377", "title": "Atrial fibrillation", "score": 0.010727813508910188, "content": "In those with chronic AF either beta blockers or calcium channel blockers are recommended. In addition to these agents, amiodarone has some AV node blocking effects (in particular when administered intravenously) and can be used in individuals when other agents are contraindicated or ineffective (particularly due to hypotension). Cardioversion Cardioversion is the attempt to switch an irregular heartbeat to a normal heartbeat using electrical or chemical means. Electrical cardioversion involves the restoration of normal heart rhythm through the application of a DC electrical shock. The exact placement of the pads does not appear to be important. Chemical cardioversion is performed with medications, such as amiodarone, dronedarone, procainamide (especially in pre-excited atrial fibrillation), dofetilide, ibutilide, propafenone, or flecainide."}, {"id": "wiki20220301en293_5635", "title": "Management of atrial fibrillation", "score": 0.01064897298847044, "content": "Catheter ablation In patients with AF where rate control drugs are ineffective and it is not possible to restore sinus rhythm using cardioversion, non-pharmacological alternatives are available. For example, to control rate it is possible to destroy the bundle of cells connecting the upper and lower chambers of the heart – the atrioventricular node – which regulates heart rate, and to implant a pacemaker instead. This \"ablate and pace\" technique has an important place in the treatment of AF< as it is the only reliably effective method for relieving the symptoms of the arrhythmia and can be used when other methods have failed (as they do in up to 50% of cases of persistent AF). Although this procedure results in a regular (paced) heart rhythm it does not prevent the atria from fibrillating and therefore long-term warfarin anticoagulation may still be required."}, {"id": "pubmed23n0562_7121", "title": "Atrial fibrillation.", "score": 0.010434782608695653, "content": "The incidence and prevalence of atrial fibrillation are increasing because of both population ageing and an age-adjusted increase in incidence of atrial fibrillation. Deciding between a rate control or rhythm control approach depends on patient age and comorbidities, symptoms and haemodynamic consequences of the arrhythmia, but either approach is acceptable. Digoxin is no longer a first-line drug for rate control: beta-blockers and verapamil and diltiazem control heart rate better during exercise. Anti-arrhythmic drugs have only a 40%-60% success rate of maintaining sinus rhythm at 1 year, and have significant side effects. The selection of optimal antithrombotic prophylaxis depends on the patient's risk of ischaemic stroke and the benefits and risks of long-term warfarin versus aspirin, but is independent of rate or rhythm control strategy. Ischaemic stroke risk is best estimated with the CHADS2 score (Congestive heart failure, Hypertension, Age > or = 75 years, Diabetes, 1 point each; prior Stroke or transient ischaemic attack, 2 points). For patients with valvular atrial fibrillation or a CHADS(2) score > or = 2, anticoagulation with warfarin is recommended (INR 2-3, higher for mechanical valves) unless contraindicated or annual major bleeding risk > 3%. Aspirin or warfarin may be used when the CHADS(2) score = 1. Aspirin, 81-325 mg daily, is recommended in patients with a CHADS(2) score of 0 or if warfarin is contraindicated. Stroke rate is similar for paroxysmal, persistent, and permanent atrial fibrillation, and probably for atrial flutter."}, {"id": "wiki20220301en293_5633", "title": "Management of atrial fibrillation", "score": 0.010246164092317938, "content": "Rate control Rate control is achieved with medications that work by increasing the degree of block at the level of the AV node, effectively decreasing the number of impulses that conduct down into the ventricles. This can be done with: Beta blockers (preferably the \"cardioselective\" beta blockers such as metoprolol, bisoprolol, atenolol) Calcium channel blockers (i.e. diltiazem or verapamil) Cardiac glycosides (i.e. digoxin) – have limited use, apart from in the sedentary elderly patient In addition to these agents, amiodarone has some AV node blocking effects (particularly when administered intravenously), and can be used in individuals when other agents are contraindicated or ineffective (particularly due to hypotension). Diltiazem has been shown to be more effective than either digoxin or amiodarone. Drugs used to control the rate of AF may cause side effects, especially fatigue and dyspnea. These are avoided by the more radical \"ablate and pace\" treatment (see below)."}, {"id": "pubmed23n0686_23671", "title": "Canadian Cardiovascular Society atrial fibrillation guidelines 2010: management of recent-onset atrial fibrillation and flutter in the emergency department.", "score": 0.010204347260184824, "content": "Atrial fibrillation (AF) is the most common arrhythmia managed by emergency physicians. There is increasing evidence that most patients with recent-onset AF or atrial flutter (AFL) can be safely managed in the emergency department (ED) without the need for hospital admission. The priorities for ED management of recent-onset AF/AFL include rapid assessment of potential hemodynamic instability and identification and treatment of the underlying or precipitating cause. A careful evaluation of the patient's history should be performed to determine the time of onset of the arrhythmia. All patients should be stratified using a predictive index for the risk of stroke (eg, CHADS(2)). For stable patients with recent-onset AF/AFL, a strategy of either rate control or rhythm control could be selected based on multiple factors including the duration of AF and the severity of symptoms. If a strategy of rhythm control has been selected, either electrical or pharmacologic cardioversion may be used. Before proceeding to cardioversion in the absence of systemic anticoagulation, physicians must be confident that the duration of AF/AFL is clearly <48 hours and that the patient is not at a particularly high risk of stroke. When the duration of AF/AFL is >48 hours or uncertain, rate control should be optimized first and the patients should receive therapeutic anticoagulation for 3 weeks before and 4 weeks after planned cardioversion. Adequate follow-up of patients with recent-onset AF/AFL is recommended to identify structural heart disease and evaluate the need for long-term antithrombotic or antiarrhythmic therapy."}]}}}} {"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 96]], "word_ranges": [[0, 16]], "text": "Everything points to adhesive capsulitis, as you say. The age, the sex, the clinic... the DM...."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Everything points to adhesive capsulitis, as you say. The age, the sex, the clinic... the DM....", "full_answer_no_ref": "Everything points to adhesive capsulitis, as you say. The age, the sex, the clinic... the DM....", "full_question": "A 60-year-old, insulin-dependent diabetic woman presents with right-sided, nocturnal-predominant omalgia of several weeks' duration. She does not report any trauma. Physical examination shows active and passive limitation of all the arcs of movement of the shoulder. Which clinical picture do you suspect as the first diagnostic possibility?", "id": 460, "lang": "en", "options": {"1": "A malignant tumor located in the proximal epiphysis of the humerus.", "2": "Septic arthritis of the shoulder.", "3": "Adhesive capsulitis.", "4": "A degenerative acromioclavicular arthrosis process.", "5": NaN}, "question_id_specific": 174, "type": "TRAUMATOLOGY AND ORTHOPEDICS", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0876_11975", "title": "Simultaneous acute shoulder arthritis and multiple mononeuropathy in a newly diagnosed type 2 diabetes patient - First case report.", "score": 0.01873249299719888, "content": "Diabetes is a common disorder that leads to the musculoskeletal symptoms such as the shoulder arthritis. The involvement of peripheral nervous system is one of the troublesome for the patients as it provokes chronic sensory symptoms, lower motor neuron involvement and autonomic symptoms. In the course of the disease there has been several types of neuropathies described. A 41-year-old male patient was admitted to the internal medicine department because of the general weakness, malaise, polydypsia and polyuria since several days. The initial blood glucose level was 780mg/dl. During the first day the continuous insulin infusion was administered. On the next day when he woke up, the severe pain in the right shoulder with limited movement, right upper extremity weakness and burning pain in the radial aspect of this extremity appeared. On examination right shoulder joint movement limitation was found with the muscle weakness and sensory symptoms in the upper limbs. The clinical picture indicated on the right shoulder arthritis and the peripheral nervous system symptoms such as the right musculocutaneous, supraspinatus, right radial nerve and left radial nerve damage. We present a first case report of simultaneous, acute involvement of the shoulder joint and multiple neuropathy in a patient with newly diagnosed type 2 diabetes, presumably in the state of ketoacidosis."}, {"id": "pubmed23n0367_19689", "title": "Adhesive capsulitis of shoulder and treatment with protease inhibitors in patients with human immunodeficiency virus infection: report of 8 cases.", "score": 0.017699115044247787, "content": "To describe our experience with human immunodeficiency virus (HIV) infected patients receiving protease inhibitor therapy who presented with adhesive capsulitis of the shoulder. Between July 1996 and December 1999, 8 HIV-infected patients (7 male) treated with protease inhibitors who presented with adhesive capsulitis of the shoulder were retrospectively identified. Diagnosis of adhesive capsulitis relied on clinical features including shoulder pain and both active and passive restricted range of motion (ROM). All available clinical and radiographic data were reviewed. Onset of symptoms was insidious, and at presentation, patients complained of shoulder pain, which was bilateral in 4 of the 8 cases. Physical examination showed global restriction of active and passive ROM of the glenohumeral joint. The mean delay between initiation of HIV protease inhibitors and onset of shoulder pain was 14 months (range 2 to 36). The protease inhibitor therapy always included indinavir. No underlying condition associated with secondary adhesive capsulitis of the shoulder, including shoulder trauma, diabetes mellitus, thyroid disease, pulmonary or cardiac diseases could be identified. In all 8 patients, despite continuation of therapy with indinavir, both shoulder pain and restricted ROM completely resolved, after a mean disease course of 7.4 months. Adhesive capsulitis of shoulder seems to be a new adverse event of HIV protease inhibitor therapy. In all reported cases, patients were treated with indinavir. Further observations will be necessary to confirm adhesive capsulitis as a side effect."}, {"id": "pubmed23n0797_17093", "title": "Acromioclavicular osteoarthritis: a common cause of shoulder pain.", "score": 0.015570095722767477, "content": "Osteoarthritis of the acromioclavicular joint is a frequent cause of shoulder pain and can result in significant debilitation. It is the most common disorder of the acromioclavicular joint and may arise from a number of pathologic processes, including primary (degenerative), posttraumatic, inflammatory, and septic arthritis. Patients often present with nonspecific complaints of pain located in the neck, shoulder, and/or arm, further complicating the clinical picture. A thorough understanding of the pertinent anatomy, disease process, patient history, and physical examination is crucial to making the correct diagnosis and formulating a treatment plan. Initial nonoperative management is aimed at relieving pain and restoring function. Typical treatments include anti-inflammatory medications, physical therapy, and injections. Patients who continue to exhibit symptoms after appropriate nonsurgical treatment may be candidates for operative resection of the distal clavicle through either open or arthroscopic techniques. "}, {"id": "wiki20220301en038_55141", "title": "Adhesive capsulitis of the shoulder", "score": 0.013858115457438664, "content": "Physical exam findings include restricted range of motion in all planes of movement in both active and passive range of motion. This contrasts with conditions such as shoulder impingement syndrome or rotator cuff tendinitis in which the active range of motion is restricted but passive range of motion is normal. Some exam maneuvers of the shoulder may be impossible due to pain. Causes The causes of adhesive capsulitis are incompletely understood; however, there are several factors associated with higher risk. Risk factors for secondary adhesive capsulitis include injury or surgery leading to prolonged immobility. Risk factors for primary, or idiopathic adhesive capsulitis include many systemic diseases, such as diabetes mellitus, stroke, lung disease, connective tissue diseases, thyroid disease, heart disease, autoimmune disease, and Dupuytren's contracture. Both type 1 diabetes and type 2 diabetes are risk factors for the condition. Primary"}, {"id": "pubmed23n0594_7070", "title": "Chronic shoulder pain: part I. Evaluation and diagnosis.", "score": 0.013313871196036801, "content": "Shoulder pain is defined as chronic when it has been present for longer than six months. Common conditions that can result in chronic shoulder pain include rotator cuff disorders, adhesive capsulitis, shoulder instability, and shoulder arthritis. Rotator cuff disorders include tendinopathy, partial tears, and complete tears. A clinical decision rule that is helpful in the diagnosis of rotator cuff tears includes pain with overhead activity, weakness on empty can and external rotation tests, and a positive impingement sign. Adhesive capsulitis can be associated with diabetes and thyroid disorders. Clinical presentation includes diffuse shoulder pain with restricted passive range of motion on examination. Acromioclavicular osteoarthritis presents with superior shoulder pain, acromioclavicular joint tenderness, and a painful cross-body adduction test. In patients who are older than 50 years, glenohumeral osteoarthritis usually presents as gradual pain and loss of motion. In patients younger than 40 years, glenohumeral instability generally presents with a history of dislocation or subluxation events. Positive apprehension and relocation are consistent with the diagnosis. Imaging studies, indicated when diagnosis remains unclear or management would be altered, include plain radiographs, magnetic resonance imaging, ultrasonography, and computed tomography scans. Plain radiographs may help diagnose massive rotator cuff tears, shoulder instability, and shoulder arthritis. Magnetic resonance imaging and ultrasonography are preferred for rotator cuff disorders. For shoulder instability, magnetic resonance imaging arthrogram is preferred over magnetic resonance imaging."}, {"id": "wiki20220301en038_55143", "title": "Adhesive capsulitis of the shoulder", "score": 0.0125, "content": "Under the microscope, the appearance of the affected shoulder joint capsule tissue is very similar to the appearance of the tissue which stops the fingers from moving in Dupuytren’s contracture, a fairly common condition where the little finger curls into the palm. Diagnosis Adhesive capsulitis can be diagnosed by history and physical exam. It is often a diagnosis of exclusion, as other causes of shoulder pain and stiffness must first be ruled out. On physical exam, adhesive capsulitis can be diagnosed if limits of the active range of motion are the same or similar to the limits to the passive range of motion. The movement that is most severely inhibited is external rotation of the shoulder."}, {"id": "article-21982_18", "title": "Frozen Shoulder -- Differential Diagnosis", "score": 0.012083145561063541, "content": "Adhesive capsulitis, particularly in early (freezing) stage might be a diagnostic challenge as it may mimic subacromial pathology and rotator cuff tendinopathy. Presentations mentioned above may result in the delay in diagnosis of AC in the early phases. Regarding shoulder impingement and rotator cuff pathology, patients report predominantly pain with less pronounced passive range of motion. However, several facets help to distinguish frozen shoulder from other shoulder disorders. Regarding the causes other than AC, patients often state lifting a heavy object or performing repetitive overhead movements. In contrast, frozen shoulder patients usually describe spontaneous onset without an apparent cause or a history of overuse activity. Extra precaution should be paid in case of the history of malignancy. Common conditions that may mimic early adhesive capsulitis:"}, {"id": "wiki20220301en038_55155", "title": "Adhesive capsulitis of the shoulder", "score": 0.010815192218619731, "content": "Prognosis Most cases of adhesive capsulitis are self limiting, but may take 1 to 3 years to fully resolve. Pain and stiffness may not completely resolve in 20 to 50 per cent of affected people. Epidemiology Adhesive capsulitis newly affects approximately 0.75% to 5.0% percent of people a year. Rates are higher in people with diabetes (10–46%). Following breast surgery, some known complications include loss of shoulder range of motion (ROM) and reduced functional mobility in the involved arm. Occurrence is rare in children and people under 40. with the highest prevalence between 40 and 70 years of age. The condition is more common in women than in men (70% of patients are women aged 40–60). People with diabetes, stroke, lung disease, rheumatoid arthritis, or heart disease are at a higher risk for frozen shoulder. Symptoms in people with diabetes may be more protracted than in the non-diabetic population. See also Calcific tendinitis Milwaukee shoulder syndrome References"}, {"id": "wiki20220301en038_55139", "title": "Adhesive capsulitis of the shoulder", "score": 0.010378138462207911, "content": "The condition often resolves itself over time without intervention but this may take several years. While a number of treatments, such as NSAIDs, physical therapy, steroids, and injecting the shoulder at high pressure, may be tried, it is unclear what is best. Surgery may be suggested for those who do not get better after a few months. About 4% of people are affected. It is more common in people 40–60 years of age and in women. Signs and symptoms Symptoms include shoulder pain and limited range of motion although these symptoms are common in many shoulder conditions. An important symptom of adhesive capsulitis is the severity of stiffness that often makes it nearly impossible to carry out simple arm movements. Pain due to frozen shoulder is usually dull or aching and may be worse at night and with any motion."}, {"id": "wiki20220301en201_1344", "title": "Capsule of the glenohumeral joint", "score": 0.010369426751592357, "content": "Clinical significance Adhesive capsulitis or frozen shoulder syndrome is a common shoulder capsule pathology that results when injury, surgery, or other chronic health conditions such as diabetes and arthritis damage or loosen the shoulder joint. As a result of the damage, the shoulder capsule becomes inflamed and taut, leading to stiffness, pain, and limited range of motion in the shoulder area. Range of motion can usually be restored by treating the affected area with heat and non-steroidal anti-inflammatory drugs (NSAIDs) in addition to light stretching and or a disciplined physical therapy program. In extreme cases where medication and physical therapy prove to be ineffective in treating the condition, arthroscopic surgery (known as Arthroscopic Capsular Release) can be performed to loosen the capsule by removing regions of thickened scar tissue within and around the shoulder joint. References Upper limb anatomy"}, {"id": "pubmed23n0617_17711", "title": "Septic arthritis of the sternoclavicular joint and osteomyelitis of the proximal clavicle caused by prevotella melaninogenicus: a case with several features delaying diagnosis.", "score": 0.009900990099009901, "content": "A 50-year-old woman with noninsulin-dependent diabetes and cirrhosis of the liver from hepatitis-B infection presented with right-sided neck and severe shoulder pain. Minimal tenderness and swelling of the right sternoclavicular joint were noted. After 8 days, extensive studies, and several attempts at therapy to relieve the shoulder pain, the right sternoclavicular joint had become more swollen, extremely tender, warm, and erythematous. An arthrotomy of the right sternoclavicular joint revealed pyoarthosis of the joint and osteomyelitis of the adjacent clavicle. Both tissue and blood cultures grew Prevotella melaninogenicus. A site of origin for the infection was never found. The patient had an uneventful recovery after treatment with open drainage and parenteral antibiotics. Although this anaerobic organism is known to cause infection at other joint sites, this seems to be the first report of infection of the sternoclavicular joint and proximal clavicle by Prevotella melaninogenicus.This case illustrates the following: 1) neck and shoulder pain may be the presenting symptoms of occult septic arthritis of the sternoclavicular joint, 2) clinical signs of infection, such as fever and leukocytosis, may be absent in the setting of anaerobic joint infections, 3) an arthrotomy should be performed as soon as an infection of the sternoclavicular joint is suspected, 4) anaerobic as well as aerobic cultures should be taken when evaluating septic arthritis 5) 2 or more weeks may be required for identification of an anaerobic organism, such as Prevotella melaninogenicus."}, {"id": "pubmed23n0081_9719", "title": "The clinical picture of the painful diabetic shoulder--natural history, social consequences and analysis of concomitant hand syndrome.", "score": 0.009900990099009901, "content": "Sixty diabetic patients with shoulder pain were followed in order to trace the natural history of the disease. The triad of painful shoulder, hand syndrome and restricted hip joint mobility was strongly correlated to the duration of diabetes and retinopathy. Painful shoulder with restricted mobility (58%) and tendinitis (28%) predominated. Hand syndrome was found in 62% and restricted hip joint mobility in 42%. Ninety percent of painful shoulders with restricted mobility had difficulties in the activities of daily living in the acute phase. There was functional limitation of shoulder mobility in 17% of painful shoulders with restricted mobility at the end of the study. The duration of diabetes and the duration of shoulder symptoms were correlated. In 25%, working capacity was affected by the painful shoulder. A serious risk of developing shoulder symptoms persisting for more than 2 years was associated with insulin treatment, diabetes lasting more than 10 years, proliferative retinopathy and painful shoulder with restricted mobility."}, {"id": "pubmed23n0795_25074", "title": "Atypical presentation of carcinoid tumor with unresolved right shoulder pain: a case report.", "score": 0.00980392156862745, "content": "Carcinoid tumors are variants of neuroendocrine tumors that typically arise from the gastrointestinal tract and the bronchus, but they can involve any organ. Unresolved right shoulder pain manifesting as the first clinical presentation of carcinoid tumor with unknown primary origin is a rare clinical entity. To the best of our knowledge, herein we present the first case report describing metastasis to the right shoulder joint in a patient who presented with bone pain as the first clinical manifestation of metastatic carcinoid tumor of unknown primary origin. Metastasis to the right scapula as the first presentation of an underlying carcinoid tumor in the primary bronchus has been reported previously. A 72-year-old Caucasian woman presented with pain in her right shoulder after a fall. She delayed seeking medical attention for 4 weeks for personal reasons. Her physical examination revealed no erythema or swelling of the right shoulder. However, tenderness was noted on the right subacromial bursa and the right acromioclavicular joint. Her drop arm test was positive. An X-ray of the right upper extremity showed no fracture. She did not respond to methylprednisolone injections or physical therapy. Because of the unresolved right shoulder pain with disturbance of her daily activities, magnetic resonance imaging of the right shoulder was ordered, which revealed permeative destruction of the right scapula. Because the permeative destruction of the bone could have been an osteolytic malignant feature, positron emission tomography-computed tomography was performed, which produced a scan showing osseous metastasis to the right scapula, multiple liver metastases and a 1.7 cm right-lower-lobe pulmonary nodule. Her serotonin and chromogranin A levels were significantly elevated. The patient was treated with palliative cisplatin and etoposide chemotherapy followed by locoregional treatments for metastatic carcinoid tumor. She had mild improvement in her right shoulder pain, as well as better range of motion and improved quality of life, before she died less than 2 years after her diagnosis. Our present case report emphasizes the protean manifestations of carcinoid tumors with the importance of early diagnosis of bone metastases from these tumors, because early diagnosis plays a major role in choosing the therapeutic regimen and prognosticating the course of the disease. The treatment goals for high-grade, poorly differentiated carcinoid tumors of unknown origin are decreasing the tumor load while controlling symptoms with chemotherapy and local modality treatments."}, {"id": "pubmed23n0818_12059", "title": "Calcific tendinitis of the shoulder.", "score": 0.009708737864077669, "content": "Calcific tendinitis is a common disease that predominantly affects individuals aged between 40 and 60 years. Women seem to be more affected than men. Various factors have been suggested to play a role in this condition, such as abnormal activity of the thyroid gland, metabolic diseases (e.g. diabetes), and genetic predisposition. Various etiological hypotheses have been advanced: the degenerative and multiphasic theories are the two most accredited ones. Clinically, calcific tendinitis is characterized by severe, disabling pain which occurs spontaneously, usually in the morning. There can be concomitant stiffness, giving rise to a frozen shoulder-like clinical picture. Conventional radiography of the shoulder is the most appropriate imaging approach. Most cases resolve spontaneously. Many conservative treatments have been reported in the literature, showing varying levels of evidence of efficacy. Arthroscopic surgery is the orthopedic specialist's last option. It is to be noted that post-surgical pain can persist for many weeks after the operation. Finally, it is important not to forget the variant characterized by osteolytic involvement of the greater tuberosity, which has been associated with a worse clinical outcome, both after conservative treatment and after surgery. "}, {"id": "pubmed23n0614_22773", "title": "Translational manipulation after failed arthroscopic capsular release for recalcitrant adhesive capsulitis: a case report.", "score": 0.009615384615384616, "content": "This article reports the use of translational manipulation after failed arthroscopic capsular release for adhesive capsulitis. The patient was a 40-year-old woman, insulin-dependent diabetic with the insidious onset of right shoulder adhesive capsulitis. The patient underwent physical therapy 3 times a week for 6 weeks with minimal changes in her range of motion or pain. After failing physical therapy, the patient had arthroscopic capsular release and long-lever arm rotational manipulation of the right shoulder. The patient participated in physical therapy again, failing to regain her range of motion. Subsequently, the patient underwent interscalene block and translational manipulation by the same therapist followed by physical therapy. The patient's range-of-motion measures, strength testing, pain scale measurements, and functional scoring were recorded throughout her rehabilitation. She returned 2 years postdischarge for the same tests and measurements. Adhesive capsulitis in association with diabetes mellitus poses a serious treatment dilemma. Arthroscopic release may have limited benefits secondary to limited release and/or postoperative pain limiting rehabilitation. Translational manipulation under interscalene block may be considered in this difficult treatment group."}, {"id": "pubmed23n0227_7945", "title": "Rheumatologic aspects of painful conditions affecting the shoulder.", "score": 0.009615384615384616, "content": "Patients with shoulder arthritis present to the orthopedic surgeon due to joint pain and loss of shoulder motion. A differential diagnosis is established, based on the history and physical examination and selected laboratory tests and roentgenograms. Synovial fluid analysis is often very helpful in the diagnosis of shoulder arthritis and critical for differential diagnosis of inflammatory, degenerative, and septic arthritis. Shoulder involvement in primary osteoarthritis is uncommon. The shoulder is rarely the initial joint involved in rheumatoid arthritis. Several uncommon conditions, e.g., amyloid arthropathy and reflex sympathetic dystrophy syndrome, may present early and frequently in the form of shoulder pain. The results of treatment are determined by etiology of shoulder joint disease. Patients with shoulder involvement in rheumatoid arthritis generally respond to the basic management for rheumatoid arthritis. Physical therapy to improve the range of motion of the shoulder and anti-inflammatory medications, including intra-articular corticosteroids, are helpful in most cases."}, {"id": "wiki20220301en132_17874", "title": "Subacromial bursitis", "score": 0.009523809523809525, "content": "Early / initial Middle / intermittent Late / return to function Prognosis In 1997 Morrison et al. published a study that reviewed the cases of 616 patients (636 shoulders) with impingement syndrome (painful arc of motion) to assess the outcome of non-surgical care. An attempt was made to exclude patients who were suspected of having additional shoulder conditions such as, full-thickness tears of the rotator cuff, degenerative arthritis of the acromioclavicular joint, instability of the glenohumeral joint, or adhesive capsulitis. All patients were managed with anti-inflammatory medication and a specific, supervised physical-therapy regimen. The patients were followed up from six months to over six years. They found that 67% (413 patients) of the patients improved, while 28% did not improve and went to surgical treatment. 5% did not improve and declined further treatment."}, {"id": "pubmed23n0645_2011", "title": "DISCUSSION ON THE PAINFUL SHOULDER.", "score": 0.009523809523809525, "content": "Three or four definite types are separated from among the many examples of painful shoulder and their treatment is debated:- (1) ADHESIONS AROUND THE JOINT.: Characterized by limitation of movement at the shoulder-joint, through the outer half of its range. Curable by manipulative surgery. (2) TENDONITIS.: Characterized by painful movements through a small arc in the middle of the normal range. Curable in the hyperacute patient by operation, in the acute by rest in partial abduction with the assistance of time. (3) OSTEO-ARTHRITIS OF THE SHOULDER-JOINT.: Characterized by painful extremes of movement. Incurable but capable of alleviation by physiotherapy. (4) SUBACUTE ARTHRITIS OF THE SHOULDER-JOINT.: Characterized by muscle spasm at the commencement of movement. To be treated by rest on an abduction splint, and the eradication of septic foci."}, {"id": "pubmed23n1046_19670", "title": "Isolated spontaneous biceps abscess causing septic shock in a diabetic patient: A rare case report.", "score": 0.009433962264150943, "content": "Intramuscular abscesses, particularly in the biceps brachii, are an extremely rare phenomenon. When present they are usually secondary to trauma, intramuscular injections, or systemic disease. A 56 year old diabetic woman presented to our emergency department with a 3 day history of fever, cough, and a painful left shoulder. Although she had a mechanical fall 2 weeks prior, she denied any pain in her shoulder immediately after the fall. She also denied any history of drug abuse or recent intramuscular injection. On examination she looked acutely unwell and was in acute septic shock requiring inotropic support. Computed tomography of her shoulder showed a large intramuscular abscess in her left biceps brachii muscle. She was immediately taken to the operating theatre for open exploration and washout of the abscess. The multiloculated abscess was tracking into the glenohumeral joint. Post operatively she showed significant clinical improvement and after a 2 week course of intravenous antibiotics recovered well and was discharged from hospital. Intramuscular abscesses are usually seen in patients who are immunocompromised. Intramuscular needle injections and haematomas secondary to trauma are also risk factors. To the best of our knowledge, there have only been 4 published reports in the English literature of intramuscular abscess formation in the biceps brachii. We report a case of a seemingly spontaneous intramuscular biceps abscess in a diabetic patient presenting with septic shock."}, {"id": "pubmed23n0124_15175", "title": "The painful diabetic shoulder.", "score": 0.009433962264150943, "content": "Different types of shoulder affection were studied in 62 diabetic patients with shoulder pain. Three groups of shoulder joint disorder were found: painful shoulder with restricted mobility (62%), tendinitis without mobility restriction (27%), and a small group with mixed diagnoses. Sixty per cent had hand symptoms and 38% had restricted mobility of their hip joints. High frequencies of retinopathy and neuropathy were found. Affection of the shoulder joint was seen with almost the same frequency in insulin-dependent as in non-insulin-dependent patients, but after a shorter duration of diabetes in the latter. A group of patients with the triad shoulder pain, hand symptoms and restricted mobility of the hip joints had a significantly higher frequency of proliferative retinopathy than patients with shoulder pain only. The long duration of diabetes, the high frequency of insulin treatment and classical late complications indicate that diabetic patients with painful shoulder and restricted mobility are suffering from clinically advanced diabetes mellitus."}, {"id": "pubmed23n0917_16007", "title": "Addressing neurodynamic irritability in a patient with adhesive capsulitis: a case report.", "score": 0.009345794392523364, "content": "Patients with adhesive capsulitis are commonly seen by physical therapists. Pain and limited shoulder motion from adhesive capsulitis have at times been linked to neural irritation. The purpose of this case is to describe the examination and intervention of a patient with adhesive capsulitis who appeared to have a coexisting, underlying neural irritation. This paper emphasizes how the neurological component must initially be identified and addressed for a successful outcome. A 47-year-old female presented with reduced shoulder motion and function, upper extremity neural irritation, diffuse weakness, altered sensation in the involved extremity, and symptoms reproduced with upper limb neurodynamic testing. Her reduced shoulder range of motion was accompanied by limited glenohumeral glides and a report of local neck stiffness. Symptoms began several months earlier after an apparent electrical shock injury to the arm that caused symptoms and guarding of the shoulder. Intervention initially addressed the underlying neural component with spinal mobilizations while avoiding further irritation. Interventions were progressed to include mobilization and exercise to address shoulder mobility. The patient's neurodynamic irritability, distal symptoms, and neck stiffness were normalized within the first weeks of care. Subsequently, interventions were directed at the shoulder. Outcomes over an 12-week time frame included reduced pain from 10/10 to 2/10. Passive range of motion increases included flexion from 121 to 160°, abduction from 71 to 121°, and external rotation from 18 to 60°. Disability scores on Disabilities of the Arm, Shoulder, and Hand (DASH) dropped from initially 68·3 to 18·3% at discharge. She ultimately regained full upper extremity function. Therapists should be cognizant of possible neural irritation in shoulder disorders, which may contribute to conditions such as adhesive capsulitis. Identifying neural irritation is critical when determining which interventions will achieve optimal outcomes without aggravating the condition."}, {"id": "pubmed23n0369_10205", "title": "[The frozen shoulder].", "score": 0.009345794392523364, "content": "Painful stiffness of the shoulder is an ill-defined clinical entity that is difficult to assess and delicate to treat. The nomenclature used is broad and includes terms such as frozen shoulder, adhesive capsulitis, focal algodystrophy, stiff shoulder, contracted shoulder, and others. Apart from its idiopathic form, the disease can be initiated by trauma, infection, tumour, radiation, systemic and local metabolic disturbances. Pathoanatomically, the common denominator is an inflammatory vascular proliferation followed by thickening, scarring, and retraction of the joint capsule. The inflammatory process often starts at the rotator interval and may extend to the subacromial space. Clinical diagnosis is based on history and physical examination. Generally the onset of pain precedes the perception of a reduced range of motion by weeks or months. In early stages of the disease, the inflammatory type of pain dominates, i.e., the patient's main complaint ist pain at night. In the later stage, range of motion gradually decreases. Patients do not often complain about reduced motion, probably because of its slow onset. Treatment options are a combination of mobilisation exercises with intra-articular steroids, hydraulic distension of the joint capsule, manipulation under anaesthesia, arthroscopic and/or open arthrolysis. The appropriate choice of protocol is just as important as its correct timing. In the inflammatory phase, aggressive treatment protocols are probably contraindicated. Complications of invasive protocols are rare but deleterious and therefore have to be taken into consideration. New anti-anglogenetic agents may enhance functional results and shorten the rehabilitation phase."}, {"id": "InternalMed_Harrison_26273", "title": "InternalMed_Harrison", "score": 0.009306317525495607, "content": "Often referred to as “frozen shoulder,” adhesive capsulitis is characterized by pain and restricted movement of the shoulder, usually in the absence of intrinsic shoulder disease. Adhesive capsulitis may follow bursitis or tendinitis of the shoulder or be associated with systemic disorders such as chronic pulmonary disease, myocardial infarction, and diabetes mellitus. Prolonged immobility of the arm contributes to the development of adhesive capsulitis. Pathologically, the capsule of the shoulder is thickened, and a mild chronic inflammatory infiltrate and fibrosis may be present."}, {"id": "pubmed23n0636_476", "title": "Chiropractic management of a 46-year-old type 1 diabetic patient with upper crossed syndrome and adhesive capsulitis.", "score": 0.009259259259259259, "content": "To discuss the treatment of a patient with type 1 diabetes presenting with chronic neck and shoulder pain by using chiropractic manipulation and an active rehabilitation program with emphasis on correcting postural imbalances. A 46-year-old insulin dependant (type1) diabetic female presented with neck and right shoulder pain of 6 to 8 months duration. Her history included similar left-sided complaints 2 years prior at which time she underwent 3 months of rehabilitation at a local medical center, which improved her condition. Over time her pain resolved but the residuals of restricted left shoulder range of motion remained. The patient had postural changes consisting of forward head posture, rounded shoulders and internally rotated arms. Treatment included spinal manipulation, ultrasound and active rehabilitation consisting of at home exercises initially and followed with in office low-tech rehabilitation. Rehabilitation was primarily aimed at improving postural abnormalities, muscle imbalances and abnormal movement patterns. The patient improved with this course of treatment. Chiropractic care including active rehabilitation may be helpful in treating diabetic patients suffering from chronic neck and shoulder problems."}, {"id": "pubmed23n0887_16923", "title": "Range of motion of diabetic frozen shoulder recovers to the contralateral level.", "score": 0.009259259259259259, "content": "Objective To determine whether frozen shoulder heals equally well in patients with and without diabetes and whether dependency on insulin affects the outcome. Methods We retrospectively examined 178 patients with idiopathic frozen shoulder; 27 patients had diabetes. We evaluated range of motion, pain, and functional results. The mean follow-up was 9.7 years (SD, 7.1 years). Results In the presence of frozen shoulder, range of motion did not differ between patients with and without diabetes. At follow-up, range of motion in all directions of both the affected and unaffected shoulders of patients with diabetes was inferior to that of patients without diabetes. Among patients with diabetes, range of motion of the once-frozen shoulder reached the level of the unaffected shoulder. Patients with and without diabetes experienced similar pain except during exertion. The Constant-Murley score was not significantly different between the two groups, and insulin dependency did not lead to worse outcomes. Conclusion Frozen shoulder heals well in patients with diabetes."}, {"id": "pubmed23n0238_945", "title": "Fracture-dislocation of the shoulder in a 32-month-old child.", "score": 0.009174311926605505, "content": "A case of fracture-separation and dislocation of the proximal humeral epiphysis, a rare injury, is reported. The patient, a 32-month-old girl suspected of being an abused child, was reported to have fallen from her crib 2 days earlier. She had a painful, swollen, slightly ecchymotic left shoulder, which she held in the neutral position and refused to move. Radiographs showed a healing, nondisplaced radial fracture and fracture-separation of the proximal humeral epiphysis, which was dislocated into an anterior subglenoid location. Surgery under general anesthesia showed the humeral head to lie in a subglenoid position and the distal fragment of the separated epiphysis to have penetrated the posterior lateral capsule. The reduction was stabilized with a single, percutaneous, smooth Kirschner wire and shoulder immobilized in a Velpeau cast for 3 weeks. At 2 year follow-up, the patient has painless, full range of motion, the epiphysis is open, and the length of the humerous appears normal."}, {"id": "pubmed23n0237_538", "title": "[Rapid destructive arthropathy of the shoulder].", "score": 0.009174311926605505, "content": "Within the general context of rapid destruction of the humeral head, destructive arthropathy of the shoulder, described here in six cases, is a diagnosis of elimination. Being neither infectious inflammatory, microcrystalline, nor neurological, this curious variety of degenerative pathology of the shoulder involves the following: 1) a particular group of sufferers: women aged 65 to 81 years; 2) prior signs, at least radiological, of deterioration in the rotator cuff; 3) rapid erosive osteolysis of the head of the humerus reducing its radiological area by 25 per cent in less than six months; 4) early narrowing of the scapulo-humeral joint space (Ist to 9th month); 5) transient appearance of calcium debris in the area of the joint; 6) a synovial effusion in some cases, often bloody. The destruction phase is associated with pain lasting from two months to two years. However at the stage of stable sequelae, pain is moderate or minimal. Differential diagnosis with destructive arthropathy due to articular chondrocalcinosis and necrosis of the head of the humerus is particularly discussed. The cause of rapid destruction is unknown. It may be multifactorial: advanced age (constant), osteoporosis, fragility of articular cartilage as evidenced by multiple localizations of osteoarthrosis (4 cases out of 6), enzymes in the bloody effusion, trauma (3 cases out of 7), and intra-articular injections of corticosteroid derivatives, in particular fluorinated (3 cases out of 7) may possibly play a role."}, {"id": "pubmed23n1131_14631", "title": "Brodie's Abscess of the Proximal Humerus Metaphysis: A Case Report.", "score": 0.00909090909090909, "content": "Primary subacute pyogenic osteomyelitis, or Brodie's abscess was initially documented by Sir Benjamin Brodie in 1832. We present a case report with a 6-months follow-up period, demonstrating the successful diagnosis and surgical treatment of a focal lesion of the proximal metaphysis of the right humerus in a 21-years-old female. The pathology of hematologic osteomyelitis and its role in the development of a subacute abscess along with a review of literature and an in detail description of the pathogenesis of Brodie's abscess is discussed and submitted. A 21- years -old healthy female with a history of fall sustaining injury to the right shoulder one 1 year back followed by which she presented to the outpatient clinic with a swelling over her right shoulder. The patient was managed conservatively with analgesics and was relieved of pain over a course of one 1 week of medications, the patient now presents with pain and swelling in the right shoulder joint on and off since the episode of fall one 1 year back, which had increased over a period of past one 1 week. A week before the most recent presentation she started experiencing some discomfort and pain in her right shoulder. No recent trauma was reported. A mild swelling appeared over the proximal part of the humerus. There were no constitutional symptoms of fever or any illness reported. On examination, there was noted a painful restriction of ROM at the right shoulder joint with no rotator cuff injury. Laboratory investigations were suggestive of raised inflammatory markers. Radiograph of the right shoulder taken in the true antero-posterior view with the shoulder in the neutral rotation was suggestive of an oval lucency with surrounding sclerosis in the proximal metaphyseal region of the humerus. Magnetic resonance imaging MRI of the right shoulder joint showed features consistent with Brodie's abscess in the proximal metaphyseal region of the humerus. Surgical debridement of the abscess was planned. The right shoulder of the patient was immobilized by a universal shoulder immobilizer for 3 days post -operatively and then Pphysiotherapy for shoulder range of movements was started. Infectious parameters decreased and there were no complications in the postoperative period. Regular follow follow-up for two 2 weeks showed clinical improvement. At 6 months follow- up, the patient had made full recovery with radiographic improvement. Brodie's abscess was first described by Sir Benjamin Brodie in 1832. Primary hematogenous subacute osteomyelitis is rarely seen in the proximal metaphysis of the humerus. With appropriate surgical debridement and aggressive antibiotic cover, a near 100% success rate is observed in the treatment of Brodie's abscess with no residual deformities in the affected bones or restrictions in the range of movements in the neighboring joints."}, {"id": "wiki20220301en244_30372", "title": "Over My Shoulder (Mike + The Mechanics song)", "score": 0.00909090909090909, "content": "Track listings UK CD1 \"Over My Shoulder\" – 3:34 \"Something to Believe In\" – 4:19 \"Always the Last to Know\" – 4:10 UK CD2 \"Over My Shoulder\" – 3:34 \"Something to Believe In\" – 4:19 \"Word of Mouth\" – 3:55 \"Over My Shoulder\" (live) – 5:25 UK cassette and limited-edition 7-inch single \"Over My Shoulder\" – 3:34 \"Something to Believe In\" – 4:18 Charts and certifications Weekly charts Year-end charts Certifications Release history References 1994 songs 1995 singles Atlantic Records singles Mike + The Mechanics songs Song recordings produced by Christopher Neil Songs written by Mike Rutherford Songs written by Paul Carrack Virgin Records singles"}, {"id": "pubmed23n0704_21285", "title": "Acromioclavicular joint pain in patients with adhesive capsulitis: a prospective outcome study.", "score": 0.009009009009009009, "content": "Diagnosis of adhesive capsulitis is a clinical diagnosis based on history and physical examination. Afflicted patients exhibit active and passive loss of motion in all planes and a positive capsular stretch sign. The effect of adhesive capsulitis on acromioclavicular biomechanics leading to tenderness has not been documented in the literature. This study reports on the incidence of acromioclavicular tenderness in the presence of adhesive capsulitis. Furthermore, we note the natural history of such acromioclavicular joint pain in relation to that of adhesive capsulitis. Over a 2-year period (2005-2007), 84 patients undergoing initial evaluation for adhesive capsulitis were prospectively examined with the use of validated outcome measures and physical examination. Acromioclavicular joint tenderness results were compared and analyzed on initial evaluation and final follow-up of at least 1 year. Forty-eight patients (57%) with adhesive capsulitis had acromioclavicular joint pain on examination. At final follow-up, as range of motion improved, a significant increase in American Shoulder and Elbow Surgeons/Penn shoulder score and decrease in number of patients with acromioclavicular pain was noted with only 6 patients with residual pain (P<.05). In the presence of adhesive capsulitis, there is not only compensatory scapulothoracic motion but also acromioclavicular motion. This often results in transient symptoms at the acromioclavicular joint, which abate as the frozen shoulder resolves and glenohumeral motion improves. This is important to recognize to avoid unnecessary invasive treatment of the acromioclavicular joint when the patient presents with adhesive capsulitis."}, {"id": "pubmed23n0528_2569", "title": "Tuberculosis of the shoulder joint.", "score": 0.009009009009009009, "content": "Skeletal tuberculosis is less common than the pulmonary form. The involvement of the shoulder joint is infrequent. We report our experience treating tuberculosis of the shoulder in 11 patients. There were seven men and four women, ranging in age from 19 to 55 years (average 28.09 years). The duration of their complaints at presentation ranged from 3 to 24 months. The most common presentation was pain, which was seen in 10 joints. All of the patients had mild to moderate restriction of motion of the shoulder. On laboratory examination, the erythrocyte sedimentation rate was increased mildly. No patient had an active tuberculosis lesion or history of pulmonary disease. The diagnosis was based on the clinical picture and radiographic features, and was confirmed by open biopsy. The diagnosis was not confirmed by biopsy in one patient, but the family history and clinical and radiological features were highly suggestive of tuberculosis. Surgical debridement was done in two patients and open biopsy in eight patients in order to obtain samples for pathology. Arthrodesis was done in only one patient. In all patients, treatment began with a four-drug regimen for 2 months, followed by a two-drug regimen for 10 months. The mean follow-up period after the end of treatment was 28.72 months (range, 22-52 months). At the time of the last visit, all the lesions had healed without recurrence. Five cases had a painless, mobile shoulder, while three had mildly restricted shoulder motion without pain, and three had residual limitation of motion of the affected shoulder. Tuberculosis of the shoulder can be difficult to diagnose in the early stages. If not diagnosed early, bony tuberculosis may reduce the quality of life. Therefore, tuberculosis should be suspected in cases of long-standing pain in the shoulder. It is necessary to keep tuberculosis in the differential diagnosis of several osseous pathologies. Arthrodesis should be reserved only for lesions that fail to heal after adequate chemotherapy and rehabilitation."}, {"id": "article-143116_15", "title": "Shoulder Arthrogram -- Introduction", "score": 0.008992010878803332, "content": "The American College of Radiology, ACR, has criteria for selecting the imaging modalities of choice for patients with traumatic or atraumatic shoulder pain. Imaging examination choices are based upon the etiology of the shoulder pain, whether traumatic or atraumatic, the duration of symptoms, the age of presentation, and any clinical or radiographic suspicions for a particular condition. [15] Again, the initial imaging modality of choice for traumatic or atraumatic shoulder pain should be radiography. Indications for conventional radiographs include evaluation for dislocation or fracture following trauma, evaluation of calcific tendinitis, crystal deposition disease, osteoarthritis, suspicion of a bony neoplasm, particularly with a patient with a non-diagnosis of cancer, suspicion for septic arthritis, or suspected humeral head avascular necrosis. The next imaging choice should be guided by the clinical scenario and findings from the plain films. [16]"}]}}}} {"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[225, 466]], "word_ranges": [[34, 70]], "text": "Thyroglobulin is synthesized in the follicular cells and then secreted into the circulation, therefore, after total thyroidectomy it should disappear, if this does not occur we must think about the existence of remnants of metastatic tissue."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The verification of the presence of thyroglobulin in blood and the development of techniques by which it can be determined have provided a new procedure for monitoring patients with papillary or follicular thyroid carcinoma. Thyroglobulin is synthesized in the follicular cells and then secreted into the circulation, therefore, after total thyroidectomy it should disappear, if this does not occur we must think about the existence of remnants of metastatic tissue.", "full_answer_no_ref": "The verification of the presence of thyroglobulin in blood and the development of techniques by which it can be determined have provided a new procedure for monitoring patients with papillary or follicular thyroid carcinoma. Thyroglobulin is synthesized in the follicular cells and then secreted into the circulation, therefore, after total thyroidectomy it should disappear, if this does not occur we must think about the existence of remnants of metastatic tissue.", "full_question": "A 35-year-old woman consults for a right thyroid nodule detected incidentally one morning when she observed in the mirror a lump on the anterior aspect of the neck. After the pertinent studies, it was decided to operate on the patient, performing a total thyroidectomy with emptying of the central lymph node component. The pathologist's report was that the 2.3 cm thyroid nodule was entirely occupied by a papillary thyroid carcinoma, tall cell variant, without vascular infiltration but with capsular infiltration. Thyroglobulin concentrations 24 h after thyroidectomy are 14 ng/mL. What would be the next step you would take in this patient?", "id": 333, "lang": "en", "options": {"1": "Refer patient back to surgeon for right laterocervical lymph node stripping.", "2": "Initiate treatment with a dose of TSH-suppressing levothyroxine and schedule for review 6 months later with a new analytical determination of thyroglobulin and cervical ultrasound.", "3": "Request a PET-CT scan for lymph node involvement.", "4": "Delay initiation of TSH replacement-suppressive treatment with levothyroxine and refer the patient to the Nuclear Medicine service for administration of an ablative dose of 100 mCi of I131.", "5": NaN}, "question_id_specific": 90, "type": "ENDOCRINOLOGY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en558_13582", "title": "Computed tomography of the thyroid", "score": 0.014617971231085373, "content": "The thyroid cancer recurrence rate is reported to range from 7 % to 14 %. Recurrence is usually detected within the first decade after initial disease diagnosis. Large lymph node metastasis is considered the strongest predictor for thyroid cancer recurrence. Post-treatment surveillance for recurrent disease depends on cancer type and staging. Patients with DTC are usually treated with total thyroidectomy and RAI ablation. Patients should have baseline neck US evaluation at 6–12 months after the RAI ablation and then periodically, depending on the patient's risk for recurrent disease and thyroglobulin (Tg) status. After the first post-operative RAI ablation, further RAI imaging is not necessary if the patient has normal neck US, undetectable Tg level under TSH stimulation, and negative antithyroglobulin (TgAb). Annual neck US with or without FNA, along with measurement of serum Tg and serum TgAb, is usually sufficient for post-treatment surveillance in those patients. Moreover, annual"}, {"id": "wiki20220301en558_13569", "title": "Computed tomography of the thyroid", "score": 0.013619298666027638, "content": "The American College of Radiology (ACR) flowchart and recommendations for ITNs detected by CT or MRI offer general guidance and are not applicable to all patients. The recommendations are primarily based on the presence or absence of suspicious features, nodule size, patient's age, patient's life expectancy, and patient's comorbidities. Suspicious features that can be detected on CT scans include signs of local invasion and abnormal lymph nodes. Abnormal lymph nodes may show cystic components, calcifications, and/or increased enhancement. Mere nodal enlargement is less specific for thyroid cancer metastasis; however, further evaluation should be considered if the ITN has ipsilateral jugulo-digastric lymph nodes > 1.5 cm on the short axis or > 1 cm for other groups. Cervical Level IV and VI lymphadenopathies raise a higher suspicion of thyroid carcinoma metastasis. Almost all patients with ITNs and suspicious imaging features should be evaluated with a neck ultrasound. Patients with"}, {"id": "pubmed23n0821_22760", "title": "Recurrence of papillary thyroid cancer after optimized surgery.", "score": 0.013392667938122483, "content": "Recurrence of papillary thyroid cancer (PTC) after optimized surgery requires a full understanding of the disease, especially as it has changed in the last 15 years, what comprises optimized surgery, and the different types and implications of disease relapse that can be encountered. PTC has evolved to tumors that are much smaller than previously seen, largely due to various high quality imaging studies obtained for different reasons, but serendipitously identifying thyroid nodules that prove to be papillary thyroid microcarcinomas (PTMC). With rare exception, these cancers are cured by conservative surgery without additional therapy, and seldom result in recurrent disease. PTC is highly curable in 85% of cases because of its rather innocent biologic behavior. Therefore, the shift in emphasis from disease survival to recurrence is appropriate. As a result of three technologic advances-high-resolution ultrasound (US), recombinant TSH, and highly sensitive thyroglobulin (Tg)-disease relapse can be discovered when it is subclinical. Endocrinologists who largely control administration of radioactive iodine have used it to ablate barely detectable or even biochemically apparent disease, hoping to reduce recurrence and perhaps improve survival. Surgeons, in response to this new intense postoperative surveillance that has uncovered very small volume disease, have responded by utilizing US preoperatively to image this disease, and incorporated varying degrees of lymphadenectomy into their initial treatment algorithm. Bilateral thyroid resection-either total or near-total thyroidectomy-remains the standard for PTC >1 cm, although recent data has re-emphasized the value of unilateral lobectomy in treating even some PTC measuring 1-4 cm. Therapeutic lymphadenectomy has universal approval, but when lymph nodes in the central neck are not worrisome to the surgeon's intraoperative assessment, although that judgment in incorrect up to 50%, whether they should be excised has reached a central point of controversy. Disease relapse can occur individually or in combination of three different forms: lymph node metastasis (LNM), true soft tissue local recurrence, and distant disease. The latter two are worrisome for potentially life-threatening consequences whereas nodal metastases are often persistent from the initial operation, and mostly comprise a biologic nuisance rather than virulent disease. A moderate surgical approach of bilateral thyroid resection, with usual central neck nodal clearance, and lateral internal jugular lymphadenectomy for node-positive disease can be performed safely, and with about a 5% recurrence rate. "}, {"id": "wiki20220301en097_40049", "title": "Papillary thyroid cancer", "score": 0.013065362510263093, "content": "Arguments for total thyroidectomy are: Reduced risk of recurrence, if central compartment nodes are removed at the original surgery. 30-85% of papillary carcinoma is multifocal disease. Hemithyroidectomy may leave disease in the other lobe. However, multifocal disease in the remnant lobe may not necessarily become clinically significant or serve as a detriment to patient survival. Ease of monitoring with thyroglobulin (sensitivity for picking up recurrence is increased in presence of total thyroidectomy, and ablation of the remnant normal thyroid by low dose radioiodine 131 after following a low iodine diet (LID). Ease of detection of metastatic disease by thyroid and neck node ultrasound. Post-operative complications at high-volume thyroid surgery centers with experienced surgeons are comparable to that of hemithyroidectomy. Arguments for hemithyroidectomy:"}, {"id": "wiki20220301en072_8800", "title": "Thyroid disease", "score": 0.012991339107261825, "content": "Surgery Thyroid surgery is performed for a variety of reasons. A nodule or lobe of the thyroid is sometimes removed for biopsy or because of the presence of an autonomously functioning adenoma causing hyperthyroidism. A large majority of the thyroid may be removed (subtotal thyroidectomy) to treat the hyperthyroidism of Graves' disease, or to remove a goiter that is unsightly or impinges on vital structures. A complete thyroidectomy of the entire thyroid, including associated lymph nodes, is the preferred treatment for thyroid cancer. Removal of the bulk of the thyroid gland usually produces hypothyroidism unless the person takes thyroid hormone replacement. Consequently, individuals who have undergone a total thyroidectomy are typically placed on thyroid hormone replacement (e.g. Levothyroxine) for the remainder of their lives. Higher than normal doses are often administered to prevent recurrence."}, {"id": "Surgery_Schwartz_10900", "title": "Surgery_Schwartz", "score": 0.012572590011614402, "content": "of nodal metastatic disease.52Imaging After the first posttreatment scan, lowand some intermediate-risk patients with negative TSH-stimulated Tg and cervical ultrasound do not require routine diagnostic whole-body radioiodine scans. However, diagnostic whole-body scans 6 to 12 months after remnant ablation may be of value in the follow-up of patients with highor intermediate-risk patients with higher risk features. Other scenarios for follow-up scans include patients with abnormal uptake outside the thyroid bed on posttherapy scan, those with poorly informative postablation scans (e.g., due to high thyroid bed uptake), and patients with Tg antibodies.Cervical ultrasound be performed to evaluate the thyroid bed and central and lateral cervical nodal compartments at 6 and 12 months after thyroidectomy and then annually for at least 3 to 5 years, depending on the patient’s risk for recurrent disease and Tg status. Sonographically suspicious nodes ≥8 to 10 mm on the smallest diameter"}, {"id": "wiki20220301en558_13586", "title": "Computed tomography of the thyroid", "score": 0.012194337194337195, "content": "In cases of elevated thyroglobulin with negative neck US and iodine whole body scintigraphy (WBS), fluorodeoxyglucose (FDG) positron emission tomography (PET) is the next modality of choice. Dedifferentiated thyroid carcinoma usually has avid FDG-PET uptake and a negative radioiodine scan, typically does not respond to RAI therapy, and has a poorer prognosis. There is not yet consensus in the research literature on whether cross-sectional imaging (CT or MRI) or an 18FDG-PET/CT scan should be performed as the first-line imaging modality for such patients. Enhanced CT scan was thought to be more sensitive for detection of lymph node metastases. Nonetheless, scans using modern PET/CT equipment are as reliable as a proper routine staging CT scan. Many lesions can be found on 18FDG-PET/CT scanning despite the lack of IV contrast injection. However, differentiation between local recurrence versus lymph node metastases and detection of direct involvement of the aerodigestive axis or vascular"}, {"id": "pubmed23n0534_17061", "title": "[Diagnosis, treatment and follow-up in the case of differentiated thyroid cancer].", "score": 0.011894288630045395, "content": "For early diagnosis of thyroid cancer, ultrasonography (US) and US-guided fine-needle aspiration biopsy are the methods of choice. Thyroid scintigraphy using Tc-99m pertechnetate can underline the necessity of surgery in case of hypofunctioning nodules. Treatment of thyroid cancer includes total thyroidectomy and staging lymphadenectomy, in the case of lymph node metastases, radical neck dissection of the ipsilateral side. Four weeks after surgery, if TSH exceeds a value of 50 mU/l, with the exception of papillary thyroid cancer pT1a (TNM 1997), radioiodine remnant ablation using activities between 2960 and 3700 MBq I-131 is performed in all other cases. As growth of benign and malignant thyroid cells depends on TSH stimulation, thyroid hormone therapy using TSH suppressive doses (TSH, <0.03 mU/l) follows radioiodine remnant ablation. Additional fractionated external radiation therapy (50 Gy) may be administered in advanced cases (e.g., pT4 N1M0; TNM 1997). Standard follow-up of differentiated thyroid cancer includes measurement of serum thyroglobulin, US of the neck and I-131 whole-body scintigraphy (I-131 WBS). With about 98% the sensitivity of thyroglobulin is very high under TSH stimulation. In case of elevated thyroglobulin, US is the method of choice to detect local recurrences and lymph node metastases of the neck. At defined intervals of follow-up or in case of increasing thyroglobulin, I-131 WBS will be performed under TSH stimulation. With the availability of recombinant TSH (exogenous TSH stimulation) the need to withdraw thyroid hormone over a period of 3-4 weeks (endogenous TSH stimulation) is no longer necessary to perform I-131 WBS. However, in about 20-40% of cases or in the course of disease after several radioiodine therapies, recurrences or metastases may be or become iodine negative. In this case, cationic complexes such as Tc-99m Sestamibi or Tc-99m Tetrofosmin are available to detect less differentiated metastases. In the course of dedifferentiation of malignant thyroid cells, the ability of iodine uptake decreases and uptake of glucose increases. This elevated glucose metabolism can be imaged using FDG PET. Today the combination of PET (metabolic imaging) and CT (morphologic imaging) using PET/CT fusion imaging is the method of choice to image iodine-negative metastases."}, {"id": "Surgery_Schwartz_10881", "title": "Surgery_Schwartz", "score": 0.011823361823361822, "content": "patients will have benign adenomas. Total thyroidectomy is recommended by some surgeons in older patients with follicular lesions >4 cm because of the higher risk of cancer in this setting (50%) and certainly should be performed in patients with atypia on FNA, a family history of thyroid cancer, or a history of radiation exposure. Intraoperative frozen-section examination usually is not helpful, but it should be performed when there is evidence of capsular or vascular invasion or when adjacent lymphadenopa-thy is present. Total thyroidectomy should be performed when thyroid cancer is diagnosed. There is debate among experts about whether patients with minimally invasive follicular can-cers should undergo completion thyroidectomy because the prognosis is so good in these patients. A diagnosis of frankly invasive carcinoma or follicular carcinoma with angioinvasion, with or without capsular invasion, necessitates completion of total thyroidectomy primarily so that 131I can be used to"}, {"id": "wiki20220301en097_40038", "title": "Papillary thyroid cancer", "score": 0.011810916971420832, "content": "Papillary thyroid carcinomas are also discovered when a hard nodule is found in multinodular goiter, when enlarged cervical lymph nodes are detected, or when there are unidentified metastatic lesions elsewhere in the body. Expanding lesions found in the thyroid gland, especially if they are painful, should be examined as they may indicate the presence of papillary thyroid carcinoma. Other clinical signs that could indicate papillary thyroid are fixation to the trachea, a firm neck mass, damage to recurrent laryngeal or cervical sympathetic nerves. Five percent of the population can have thyroid nodules, and the majority will be benign. Appropriate workup includes an ultrasound of the neck, followed by lab studies. Patients will usually meet with both an endocrinologist and a surgeon (head and neck surgeon or endocrine surgeon)."}, {"id": "Surgery_Schwartz_10899", "title": "Surgery_Schwartz", "score": 0.011654511654511654, "content": "Further measure-ments are guided by response to therapy. Patients are considered to have an excellent response to treatment if suppressed Tg is <0.2 ng/mL and stimulated Tg is <1 ng/mL with negative imag-ing. In these patients, Tg levels can be followed every 12 to 24 months while on thyroid hormone as their risk of recurrence is low (1–4%). Patients with structurally or biochemically incomplete (negative imaging but suppressed Tg ≥1 ng/mL or stimulated Tg ≥10 ng/mL or rising anti-Tg levels) or indeter-minate responses (nonspecific imaging findings, suppressed Tg detectable but <1 ng/mL, and stimulated Tg detectable but <10 ng/mL or stable or declining anti-Tg levels) require addi-tional investigations.40 Tg measurements in FNAB aspirates have also been shown to be useful in the detection of nodal metastatic disease.52Imaging After the first posttreatment scan, lowand some intermediate-risk patients with negative TSH-stimulated Tg and cervical ultrasound do not require routine"}, {"id": "wiki20220301en184_6403", "title": "Thyroid nodule", "score": 0.011645858504497248, "content": "Malignancy Only a small percentage of lumps in the neck are malignant (around 4 – 6.5%), and most thyroid nodules are benign colloid nodules. There are many factors to consider when diagnosing a malignant lump. Trouble swallowing or speaking, swollen cervical lymph nodes or a firm, immobile nodule are more indicative of malignancy, whereas a family history of autoimmune disease or goiter, thyroid hormonal dysfunction or a soft, painful nodule are more indicative of benignancy. The prevalence of cancer is higher in males, patients under 20 years old or over 70 years old, and patients with a history of head and neck irradiation or a family history of thyroid cancer. Solitary thyroid nodule"}, {"id": "wiki20220301en558_13575", "title": "Computed tomography of the thyroid", "score": 0.011011778061586491, "content": "Role of imaging Surgery is the primary mode of treatment for DTCs. Post total thyroidectomy radioactive iodine (RAI) ablation is an option, especially in patients with distant metastasis, tumours larger than 4 cm, or extra-thyroidal disease extension. Ultrasound examination is usually adequate in evaluating primary tumours and cervical lymph nodes. Preoperative cross-sectional imaging with CT or MRI is indicated if there is a concern for local invasion that may alter the patient's staging as well as surgical approach (Figs. 4, 55 and 6)6) . Some thyroid primaries may be small, diffuse, or multifocal and therefore may be occult on imaging (Fig. 4) ."}, {"id": "Surgery_Schwartz_10892", "title": "Surgery_Schwartz", "score": 0.010957154088050314, "content": "to be discontinued for 2 weeks to allow TSH levels to rise before treatment. Levels >30 mU/L are considered optimal, based on noncontrolled studies. A low-iodine diet also is recommended during this 2-week period. The usual protocol involved administering a screening dose of 1 to 3 mCi and measuring uptake 24 hours later. After a total thy-roidectomy, this value should be <1%. A “hot” spot in the neck after initial screening usually represents residual normal tissue in the thyroid bed. Some investigators recommend omitting the scanning dose altogether to minimize thyrocyte “stunning” and subsequent requirement for higher treatment doses. Others recommend scanning only if the size of the remnant cannot be determined by the operative report or ultrasound, or if the results would alter the decision to treat or the dose to be administered. Current guidelines recommend using either 123I or low-activity 131I (1to 3-mCi dose) and delivering a therapeutic dose within 72 hours.The recommended"}, {"id": "wiki20220301en262_820", "title": "Thyroid cancer", "score": 0.010654160654160656, "content": "Treatment Thyroidectomy and dissection of central neck compartment is initial step in treatment of thyroid cancer in the majority of cases. Thyroid-preserving operations may be applied in cases, when thyroid cancer exhibits low biological aggressiveness (e.g. well-differentiated cancer, no evidence of lymph-node metastases, low MIB-1 index, no major genetic alterations like BRAF mutations, RET/PTC rearrangements, p53 mutations etc.) in patients younger than 45 years. If the diagnosis of well-differentiated thyroid cancer (e.g. papillary thyroid cancer) is established or suspected by FNA, then surgery is indicated, whereas watchful waiting strategy is not recommended in any evidence-based guidelines. Watchful waiting reduces overdiagnosis and overtreatment of thyroid cancer among old patients."}, {"id": "wiki20220301en097_40048", "title": "Papillary thyroid cancer", "score": 0.010634138712956447, "content": "Gross disease (diameter over 1.0 centimeters) - total thyroidectomy, and central compartment lymph node removal is the therapy of choice. Additional lateral neck nodes can be removed at the same time if an ultrasound guided FNA and thyroglobulin TG cancer washing was positive on the pre-operative neck node ultrasound evaluation."}, {"id": "wiki20220301en097_40041", "title": "Papillary thyroid cancer", "score": 0.009945981458500619, "content": "The so-called lateral aberrant thyroid is usually a lymph node metastasis from a papillary thyroid carcinoma. Papillary microcarcinoma is a subset of papillary thyroid cancer defined as measuring less than or equal to 1 cm. The highest incidence of papillary thyroid microcarcinoma in an autopsy series was reported by Harach et al. in 1985, who found 36 of 101 consecutive autopsies to have an incidental microcarcinoma. Michael Pakdaman et al. report the highest incidence in a retrospective surgical series at 49.9 percent of 860 cases. Management strategies for incidental papillary microcarcinoma on ultrasound (and confirmed on FNAB) range from total thyroidectomy with radioactive iodine ablation to observation alone. Harach et al. suggest using the term \"occult papillary tumor\" to avoid giving patients distress over having cancer. It was Woolner et al. who first arbitrarily coined the term \"occult papillary carcinoma\" in 1960, to describe papillary carcinomas ≤ 1.5 cm in diameter."}, {"id": "pubmed23n0731_12169", "title": "Update in utility of secondary node dissection for papillary thyroid cancer.", "score": 0.009900990099009901, "content": "Detection of recurrent/persistent thyroid cancer has improved significantly in the past decade. Disease is detected earlier in recently treated patients and localized in patients long out from initial treatment. This update reviews recent literature regarding the utility of secondary node dissection for papillary thyroid carcinoma. Outcomes include disease-free status measured biochemically and clinically. The utility of secondary node dissection as measured by clinically detectable disease exceeds 70% for all series and 90% for most. The utility as measured biochemically is more modest, with rates of biochemical cure ranging from 27-81% depending upon strictness of definition and patient selection. In predominately radioiodine scan-negative patients, using the strictest definition of biochemical cure, undetectable stimulated thyroglobulin (Tg) of less than 0.5 ng/ml, a rate of 27% is reported. Biochemical cure rates are reportedly 30-51% for stimulated Tg of less than 2 ng/ml and 56-71% for basal Tg of less than 2 ng/ml, with higher preoperative Tg levels less likely to achieve biochemical cure. Radioiodine-avid disease appears more amenable to cure, with 81% of patients achieving negative stimulated Tg after repeat I131 treatment and radio-assisted surgery. Complication rates of secondary nodal surgery appear similar to initial surgery in experienced hands; however, bilateral reoperative central neck dissection is associated with significantly higher complication rates than unilateral. Surgical resolution of clinically detectable disease is likely. Biochemical cure rates are more modest, with the greatest likelihood of biochemical cure occurring in patients with radioiodine-avid disease. In radioiodine-negative patients, there may be a higher likelihood of biochemical cure for those with lower preoperative detectable Tg levels."}, {"id": "pubmed23n0608_14808", "title": "Exophytic and ulcerated local recurrence of papillary thyroid carcinoma with spectacular response to external beam radiotherapy.", "score": 0.00980392156862745, "content": "A 60-year-old female patient with a history of multifocal papillary thyroid carcinoma was referred to the endocrinology clinic for an exophytic and ulcerated lesion at the right base of the neck. Twelve years earlier she had undergone a total thyroidectomy for multinodular goiter. Four years after the total thyroidectomy she had a lymph node dissection of the neck and was then treated twice with 100 mCi 131I. Five years after the second dose of 131I she was lost to followup. Before she was lost to followup the serum thyroglobulin (Tg) after recombinant TSH administration was 84 ng/ml (normal values < 2 ng/ml) and there was no uptake on a radioiodine whole body scan. Neck ultrasonography and MRI revealed a 1.7 by 1.7 cm mass in the region where the right lobe of the thyroid gland is. Surgical removal of the lesion was not possible since the neoplasm infiltrated the adjacent vital structures. The disease was treated with external beam radiation therapy. Two years later the patient is still alive and local control of the disease is achieved. In selected patients where other therapeutic modalities such as surgery, levothyroxine suppression therapy or radioactive iodine are not an option or ineffective, external beam radiotherapy can likely prolong survival."}, {"id": "Surgery_Schwartz_10768", "title": "Surgery_Schwartz", "score": 0.00980392156862745, "content": "regarding risk of malignancy. Ultrasound is also especially help-ful for assessing cervical lymphadenopathy (Fig. 38-11) and to guide FNAB. An experienced ultrasonographer is necessary for the best results.Computed Tomography/Magnetic Resonance Imaging Scan CT and magnetic resonance imaging (MRI) studies provide excellent imaging of the thyroid gland and adjacent nodes and are particularly useful in evaluating the extent of large, fixed, or substernal goiters (which cannot be evaluated by ultrasound) and their relationship to the airway and vascular structures. Noncontrast CT scans should be obtained for patients who are likely to require subsequent RAI therapy. If contrast is necessary, therapy needs to be delayed by several months. Combined PET-CT scans are increasingly being used for Tg-positive, RAI-negative tumors.Benign Thyroid DisordersHyperthyroidism. The clinical manifestations of hyperthy-roidism result from an excess of circulating thyroid hormone. Hyperthyroidism may arise"}, {"id": "pubmed23n0733_17821", "title": "Euthyroid status after total thyroidectomy due to functioning lung metastases from a clear cell variant of papillary thyroid carcinoma.", "score": 0.009708737864077669, "content": "Although functioning thyroid cancer metastases have been reported, they have almost never been reported for the clear cell variant of papillary thyroid carcinoma (PTC). Here we describe a patient with disseminated lung metastases of the clear cell variant of PTC who presented in the euthyroid state despite discontinuance of levothyroxine after total thyroidectomy. A 49-year-old woman underwent total thyroidectomy for the clear cell variant of PTC in March 2002. Levothyroxine replacement was initiated after total thyroidectomy, but the patient was lost to follow-up 5 years after the operation. She did not take any levothyroxine for 4 years. Upon presentation to our institution, her initial thyroid function tests were a serum thyroid-stimulating hormone (TSH) of 4.51 mIU/L (0.30-5.00), total triiodothyronine of 82 ng/dL (60-181), and free thyroxine of 1.21 ng/dL (0.89-1.76). The results of workups, including thyroid ultrasonography, chest computed tomography (CT) scan, and fluorine-18 fluorodeoxyglucose positron emission tomography (18F-FDG PET)/CT, revealed that she had multiple metastases in the cervical lymph nodes and both lungs. She received 0.9 mg of recombinant human TSH (rhTSH) for 2 consecutive days followed by administration of 200 mCi 131I. A therapeutic whole body scan after 131I administration demonstrated intense uptake in the whole lung fields, suggesting functioning lung metastases. It is extremely rare for metastatic PTC, even though it is a well-differentiated thyroid carcinoma, to produce a sufficient amount of thyroid hormones to result in euthyroid state after total thyroidectomy. To our knowledge, this is the first report of functioning lung metastases of the clear cell variant of PTC after total thyroidectomy that produced enough thyroid hormone to restore a euthyroid state. Functioning metastases from recurred PTC, particularly of the clear cell variant, are very rare. When they occur, rhTSH is required to prepare these patients for treatment with ablative doses of radioactive iodine (131I)."}, {"id": "pubmed23n0895_6150", "title": "Radioguided sentinel lymph node biopsy in patients with papillary thyroid carcinoma.", "score": 0.009708737864077669, "content": "The ATA guidelines do not recommend prophylactic central compartment neck dissection in patients with T1-T2 papillary thyroid carcinoma (PTC) with no clinical evidence of lymph node metastasis, however patients' staging is recommended. Lymph node metastasis may be present also in small PTC, but preoperative ultrasound identifies suspicious cervical lymphadenopathy in 20-30% of patients. The role of sentinel lymph node biopsy (SLNB) remain open to debate. It has been shown that the identification rate of SLN in PTC patients is improved using a radiotracer compared to a dye technique. The aim of this systematic review was to evaluate the role of radioguided SLNB (rSLNB) in the treatment of PTC patients. A systematic search was performed in the PubMed and Embase database to identify all original articles regarding the application of rSLNB in PTC patients. The primary outcome was false negative rate (FNR) of the rSLNB; the secondary outcomes were SLN intraoperative identification rate (IIR), site of lymph node metastasis, and persistent disease during follow up. Twelve studies were included. Most of PTC patients were T1-T2. The overall SLN IIR, SLN metastatic rate, and FNR were 92.1%, 33.6%, and 25.4%, respectively. Overall, lymph node metastasis were found in the central compartment (23.0%) and in the lateral compartments (10.6%). The persistent disease in patients who underwent SLNB associated to lymph node dissection (LND) in the same compartment of the SLN regardless of the SLN status was 0.6%. In all PTC patients, also in T1-T2 stage, due to the high FNR the SLNB performed alone should be abandoned and converted into a technique to guide the lymphadenectomy in a specific neck compartment (i.e., central or lateral) based on the radioactivity, regardless of the SLN status, for better lymph node staging and selection of patients for postoperative radioiodine ablation."}, {"id": "wiki20220301en203_29014", "title": "Thyroid lymph nodes", "score": 0.009683794466403162, "content": "The thyroid lymph nodes are deep anterior cervical lymph nodes found near the thyroid gland on the neck. Lymphatics of the head and neck"}, {"id": "pubmed23n0628_4027", "title": "Identification of a neck lump as a lymph node metastasis from an occult contralateral papillary microcarcinoma of the thyroid: key role of thyroglobulin assay in the fine-needle aspirate.", "score": 0.009615384615384616, "content": "Thyroglobulin (Tg) assay of material from fine-needle aspiration of neck masses can help distinguish neck masses of thyroid origin from other masses. We describe its utility in a patient with an unusual constellation of findings, a neck lump identified as a lymph node metastasis from a contralateral occult papillary thyroid carcinoma (PTC). A 56-year-old woman was referred to our center for evaluation of a 15-mm right lateral cervical neck mass which was strongly hypoechoic, not homogenous and contained several microcalcifications. There was no family history of thyroid disease, the patient was euthyroid and was not taking medications for thyroid disorders. On physical examination the thyroid was slightly enlarged and was normal on ultrasound except for a 1 x 3 mm hypoechoic nodule in the middle of the left lobe. Ultrasound-guided fine-needle aspiration biopsy (FNAB) of the right lateral cervical mass was performed with the Tg concentration of the FNAB washout liquid being >300 ng/mL and the cytology showing lymphoid elements mixed with polymorphous epithelial cells with atypical nuclei, suggesting lymph node metastasis from a cancer of epithelial origin. A lymph node metastasis from a papillary thyroid microcarcinoma (micro-PTC) was the presumptive diagnosis with the preoperative staging being Tx N1b. The patient underwent total thyroidectomy and bilateral lymph node dissection. At pathology, the right cervical mass was confirmed as lymph node metastasis of a PTC, and a unifocal micro-PTC was found in the middle left lobe. The patient was readmitted for a therapeutic (131)I dose (4810 MBq). At the time of (131)I administration, the whole-body scan showed only minimal thyroid bed uptake and serum Tg was <1 ng/mL. She was maintained on l-thyroxine treatment (150 microg/d). Five year later she did not have evidence of recurrent or residual PTC. We describe the first case of contralateral lymph node metastasis from a unifocal micro-PTC identified by the detection of high Tg levels in the wash-out liquid of FNAB."}, {"id": "pubmed23n0255_5934", "title": "[\"Occult\" carcinoma of the thyroid: clinical, morphological, and biologic characteristics for a correct therapeutic regime].", "score": 0.009615384615384616, "content": "The \"occult\" carcinoma of the thyroid is still a highly controversial topic. The controversies not only regard its diagnosis, treatment, natural history and, therefore, its biological potential, but also aspects of a nosological nature in that there is still no unequivocal acceptance of its originality, thus leading to discussions focused on its precise definition. On the basis of our experience and other published data, we have reached the following conclusions: a) the term \"occult\" carcinoma of the thyroid must be used to describe a neoplasia which does not exceed 1.5 cm in diameter, irrespective of the presence or otherwise of laterocervical adenopathy, and leaving aside the fact that it can be identified using clinical and instrumental tests; b) high-resolution echography and echo-guided FNA are fundamental instruments for a correct and early preoperative diagnosis; c) the clinical, morphological and, above all, biological (a very slowly evolving neoplasia) characteristics make the occult carcinoma of the thyroid seem to be a tumour with its own nosological identity; d) on the strength of the latter, and in particular in view of its natural history, conservative surgery (lobectomy with isthmectomy), in the differentiated forms, might play a primary role in the treatment of occult carcinoma of the thyroid in the very near future. However, at present complete thyroidectomy represents the treatment of choice even in differentiated forms, whereas lobectomy is only reserved for incidental cases of occult carcinoma discovered during the final histological test; e) lymphadenectomy is indicated in the event of lymph node involvement, not to achieve a longer survival rate but to reduce the incidence of recidivation in the form of lymph node metastases. In these cases, even the mere removal of macroscopically damaged lymph nodes is sufficient to ensure the virtual absence of recidivation on which, it is worth noting, metabolic radio-iodotherapy is efficacious in the large majority of cases."}, {"id": "pubmed23n0887_6738", "title": "Management of thyroid cancer: United Kingdom National Multidisciplinary Guidelines.", "score": 0.009523809523809525, "content": "This is the official guideline endorsed by the specialty associations involved in the care of head and neck cancer patients in the UK. This paper provides recommendations on the management of thyroid cancer in adults and is based on the 2014 British Thyroid Association guidelines. Recommendations • Ultrasound scanning (USS) of the nodule or goitre is a crucial investigation in guiding the need for fine needle aspiration cytology (FNAC). (R) • FNAC should be considered for all nodules with suspicious ultrasound features (U3-U5). If a nodule is smaller than 10 mm in diameter, USS guided FNAC is not recommended unless clinically suspicious lymph nodes on USS are also present. (R) • Cytological analysis and categorisation should be reported according to the current British Thyroid Association Guidance. (R) • Ultrasound scanning assessment of cervical nodes should be done in FNAC-proven cancer. (R) • Magnetic resonance imaging (MRI) or computed tomography (CT) should be done in suspected cases of retrosternal extension, fixed tumours (local invasion with or without vocal cord paralysis) or when haemoptysis is reported. When CT with contrast is used pre-operatively, there should be a two-month delay between the use of iodinated contrast media and subsequent radioactive iodine (I131) therapy. (R) • Fluoro-deoxy-glucose positron emission tomography imaging is not recommended for routine evaluation. (G) • In patients with thyroid cancer, assessment of extrathyroidal extension and lymph node disease in the central and lateral neck compartments should be undertaken pre-operatively by USS and cross-sectional imaging (CT or MRI) if indicated. (R) • For patients with Thy 3f or Thy 4 FNAC a diagnostic hemithyroidectomy is recommended. (R) • Total thyroidectomy is recommended for patients with tumours greater than 4 cm in diameter or tumours of any size in association with any of the following characteristics: multifocal disease, bilateral disease, extrathyroidal spread (pT3 and pT4a), familial disease and those with clinically or radiologically involved nodes and/or distant metastases. (R) • Subtotal thyroidectomy should not be used in the management of thyroid cancer. (G) • Central compartment neck dissection is not routinely recommended for patients with papillary thyroid cancer without clinical or radiological evidence of lymph node involvement, provided they meet all of the following criteria: classical type papillary thyroid cancer, patient less than 45 years old, unifocal tumour, less than 4 cm, no extrathyroidal extension on ultrasound. (R) • Patients with metastases in the lateral compartment should undergo therapeutic lateral and central compartment neck dissection. (R) • Patients with follicular cancer with greater than 4 cm tumours should be treated with total thyroidectomy. (R) • I131 ablation should be carried out only in centres with appropriate facilities. (R) • Serum thyroglobulin (Tg) should be checked in all post-operative patients with differentiated thyroid cancer (DTC), but not sooner than six weeks after surgery. (R) • Patients who have undergone total or near total thyroidectomy should be started on levothyroxine 2 µg per kg or liothyronine 20 mcg tds after surgery. (R) • The majority of patients with a tumour more than 1 cm in diameter, who have undergone total or near-total thyroidectomy, should have I131 ablation. (R) • A post-ablation scan should be performed 3-10 days after I131 ablation. (R) • Post-therapy dynamic risk stratification at 9-12 months is used to guide further management. (G) • Potentially resectable recurrent or persistent disease should be managed with surgery whenever possible. (R) • Distant metastases and sites not amenable to surgery which are iodine avid should be treated with I131 therapy. (R) • Long-term follow-up for patients with differentiated thyroid cancer (DTC) is recommended. (G) • Follow-up should be based on clinical examination, serum Tg and thyroid-stimulating hormone assessments. (R) • Patients with suspected medullary thyroid cancer (MTC) should be investigated with calcitonin and carcino-embryonic antigen levels (CEA), 24 hour catecholamine and nor metanephrine urine estimation (or plasma free nor metanephrine estimation), serum calcium and parathyroid hormone. (R) • Relevant imaging studies are advisable to guide the extent of surgery. (R) • RET (Proto-oncogene tyrosine-protein kinase receptor) proto-oncogene analysis should be performed after surgery. (R) • All patients with known or suspected MTC should have serum calcitonin and biochemical screening for phaeochromocytoma pre-operatively. (R) • All patients with proven MTC greater than 5 mm should undergo total thyroidectomy and central compartment neck dissection. (R) • Patients with MTC with lateral nodal involvement should undergo selective neck dissection (IIa-Vb). (R) • Patients with MTC with central node metastases should undergo ipsilateral prophylactic lateral node dissection. (R) • Prophylactic thyroidectomy should be offered to RET-positive family members. (R) • All patients with proven MTC should have genetic screening. (R) • Radiotherapy may be useful in controlling local symptoms in patients with inoperable disease. (R) • Chemotherapy with tyrosine kinase inhibitors may help in controlling local symptoms. (R) • For individuals with anaplastic thyroid carcinoma, initial assessment should focus on identifying the small proportion of patients with localised disease and good performance status, which may benefit from surgical resection and other adjuvant therapies. (G) • The surgical intent should be gross tumour resection and not merely an attempt at debulking. (G)."}, {"id": "wiki20220301en126_880", "title": "Cervical lymph nodes", "score": 0.00943468296409473, "content": "Clinical significance Infectious mononucleosis (glandular fever) affects the cervical lymph nodes which become swollen. The characterization of cancerous lymph nodes on CT scan, MRI or ultrasound is difficult, and usually requires confirmation by other nuclear imaging techniques such as PET scans. Tissue diagnosis by fine needle aspiration (which has a high rate of accuracy), may also be required. Involvement of the cervical lymph nodes with metastatic cancer is the single most important prognostic factor in head and neck squamous cell carcinoma and may be associated with a halving of survival. Where the cancer has penetrated the capsule of the lymph gland (extracapsular extension) survival may be decreased by a further 50%. Other important factors are the level, the number of nodes and their size, which are also correlated with the risk of distant metastases. Cervical lymph node metastasis is also a common feature of papillary thyroid carcinoma. Additional images References"}, {"id": "wiki20220301en097_40052", "title": "Papillary thyroid cancer", "score": 0.009433962264150943, "content": "Patients are administered hormone replacement levothyroxine for life after surgery, especially after total thyroidectomy. Chemotherapy with cisplatin or doxorubicin has proven limited efficacy, however, it could be helpful for patients with bone metastases to improve their quality of life. Patients are also prescribed levothyroxine and radioiodine after surgery. Levothyroxine influences growth and maturation of tissues and it is involved in normal growth, metabolism, and development. In case of metastases, patients are prescribed antineoplastic agents which inhibit cell growth and proliferation and help in palliating symptoms in progressive disease. After successful treatment, 35 percent of the patients may experience a recurrence within a 40-year span. Also, patients may experience a high incidence of nodule metastasis, with 35 percent cases of cervical node metastases. Approximately 20 percent of patients will develop multiple tumors within the thyroid gland."}, {"id": "pubmed23n1125_10230", "title": "How Many Nodes to Take? Lymph Node Ratio Below 1/3 Reduces Papillary Thyroid Cancer Nodal Recurrence.", "score": 0.009433962264150943, "content": "Papillary thyroid carcinoma (PTC) accounts for the majority of thyroid malignancies; risk of PTC recurrence over a 30-year period is approximately 30%, of which 70% occur as nodal metastases. Patients with nodal disease who are treated with therapeutic dissection are at higher risk for recurrence, but optimal nodal yield has not been defined. We aim to determine variables predictive of nodal recurrence of PTC within the first 5 years of surgery, with a focus on lymph node ratio (LNR), to inform clinical decision-making. Retrospective chart review identified 41 patients with nodal recurrence of PTC and 284 without nodal recurrence following thyroid surgery from 2000 to 2015. Cohorts were compared with regards to clinical history, surgical findings, and tumor characteristics. The fraction of the patients who underwent therapeutic central or lateral lymph node dissection was significantly higher in the nodal recurrence cohort. Maximum tumor size, presence of extrathyroidal extension, largest lymph node focus, LNR, postoperative thyroglobulin level, and administration of postoperative radioactive iodine were significantly increased in the PTC nodal recurrence group. LNR greater than 0.3 held the highest level of significance as a binary cutoff and captured the larger proportion of patients in the nodal recurrence cohort (68.3%). This study demonstrates characteristics to help assess risk of nodal recurrence of PTC and suggests LNR of lower than 0.3 is optimal to reduce risk of recurrence. The next steps include cohort studies to validate findings and weight variable analysis to optimize the extent of surgical therapeutic dissection. 4 Laryngoscope, 132:1883-1887, 2022."}, {"id": "pubmed23n0919_1806", "title": "The Role of Intraoperative Thyroglobuline Level of Lymph Node in the Management of Papillary Thyroid Cancer (Determination of a Cutoff Point).", "score": 0.009345794392523364, "content": "Some studies have shown that a preoperative high concentration of thyroglobulin (Tg) in wash out of fine-needle aspiration cytology of cervical lymph nodes mandate therapeutic lymph node dissection. However, there is disagreement about the minimum concentration of Tg which could have diagnostic value. Hence, according to our literature review, this study is the first one which designed to do intraoperatively. Therefore, this study was conducted and aimed to determine the clinical diagnostic value of Tg lymph nodes in the diagnosis of metastatic thyroid cancer. In a cross-sectional study, 65 patients with papillary thyroid carcinoma (PTC) who were thyroidectomy candidates were chosen and during surgery, before the removal of lymph nodes in the neck, fine-needle sampling was performed and the level of Tg in the samples, nature of the sample sent for biopsy and Tg levels in affected and unaffected lymph nodes were determined. The mean levels of washout Tg in malignant and nonmalignant lymph nodes were 622.1 ± 66.2 and 1.38 ± 0.43 ng/ml, respectively, and the difference between the two groups was significant (0.25 mmol/l (>1 mg/dl) higher than the upper limit of normal or >2.75 mmol/l (>11 mg/dl) Renal insufficiency: creatinine clearance <40 ml per minute or serum creatinine >1.77 mol/l (>2 mg/dl) Anemia: hemoglobin value of >2g/dl below the lowest limit of normal, or a hemoglobin value <10g/dl Bone lesions: osteolytic lesions on skeletal radiography, CT, or PET/CT Bone pain"}, {"id": "pubmed23n0324_15103", "title": "[Aetiologic features of osteoporosis in male patients aged less than 50 years: study of 28 cases with a comparative series of 30 patients over the age of 50].", "score": 0.009345794392523364, "content": "To study the aetiologic factors of osteoporosis (OP) in young male patients, we conducted a retrospective study of male osteoporosis observed in our department during the past 20 years. Patients included in the study were over 16 years of age and had a fracture or a decreased bone mineral density with a T score (assessed at the spine or at the spine and femoral neck) below -2.5 SD. The age and circumstances of diagnosis, serum and 24-hour urinary calcium and phosphorus investigations, hormonal measurements (including parathyroid hormone, thyroid hormones, cortisol and testosterone), bone biopsy and the final diagnosis were analyzed. One hundred and nineteen patients responded to the defined diagnosis criteria. Twenty-eight were less than 50 years of age (group I or young male osteoporosis). Data were compared with those of 30 patients more than 50 years of age (group II). In group I, an aetiology was found in 17 out of 28 cases (60.7%) (secondary osteoporosis: OP II), while 11 out of 28 patients (39.3%) had primary or idiopathic osteoporosis (OP I). In group II, the frequency of OP I was 19 out of 30 cases (63.3%) and 11 out of 30 patients (36.6%) had OP II. However, the frequency of OP II did not differ significantly between the two groups (P = 0.11). The aetiology in group I was either hypogonadism (6 out of 17), alcoholism (2 out of 17), mastocytosis (2 out of 17), primary biliary cirrhosis (1 out of 17), osteogenesis imperfecta (1 out of 17), idiopathic hypercalciuria (1 out of 17), corticosteroid treatment (1 out of 17) or ankylosing spondylitis (1 out of 17). Multiple causes were found in 5 out of 17 cases. The causes of osteoporosis in group II included hypogonadism (2 out of 11), alcoholism (2 out of 11), idiopathic hypercalciuria (2 out of 11), glucocorticosteroid therapy (6 out of 11) and rheumatoid arthritis (1 out of 11). Our results were compared to those of the various series reported in the literature which, though focusing on male osteoporosis, often included elderly patients. To determine whether osteoporosis in young male is more often OP II, further prospective studies are required."}, {"id": "wiki20220301en020_65250", "title": "Hypocalcemia", "score": 0.009259259259259259, "content": "Mechanism Physiologically, blood calcium is tightly regulated within a narrow range for proper cellular processes. Calcium in the blood exists in three primary states: bound to proteins (mainly albumin), bound to anions such as phosphate and citrate, and as free (unbound) ionized calcium; all of these forms are ionised. Only the unbound calcium is physiologically active. Normal blood calcium level is between 8.5 to 10.5 mg/dL (2.12 to 2.62 mmol/L) and that of unbound calcium is 4.65 to 5.25 mg/dL (1.16 to 1.31 mmol/L). Diagnosis Because a significant portion of calcium is bound to albumin, any alteration in the level of albumin will affect the measured level of calcium. A corrected calcium level based on the albumin level is: Corrected calcium (mg/dL) = measured total Ca (mg/dL) + 0.8 * (4.0 - serum albumin [g/dL]). Since calcium is also bound to small anions, it may be more useful to correct total calcium for both albumin and the anion gap."}, {"id": "pubmed23n0029_10841", "title": "[The diagnostic value of radiological, nuclear medicine and biochemical methods for detection of bone metastases in carcinoma of the prostate (author's transl)].", "score": 0.009259259259259259, "content": "785 combined diagnostic procedures are reported which were carried out on 353 patients with microscopically proven carcinoma of the prostate in order to detect metastases. X-ray films of the thorax, spine and pelvis were taken. Also bone-scintigraphy was done with 87MSr or 99MTc-polyphosphate. Additionally the alcaline, acid and prostate phosphatases were determined. A diagnostic coincidence between radiological results and bone-scan was found in 95.1% of cases. The bone-scan was false negative in 3.7%. Only in 1.2% metastases were detected earlier by the bone-scan than by x-ray examinations. Referring to the number of studies identical results were found. In roentgenologically detected metastases an increased serum level of the alcaline phosphatase was found in 61%, of the acid phosphatase in 31% and of the prostate-phosphatase in 25%."}, {"id": "pubmed23n0971_4376", "title": "Treatment approach for the older, unfit patient with myeloma from diagnosis to relapse: perspectives of a European hematologist.", "score": 0.009174311926605505, "content": "Mrs. A. is a 73-year-old woman who has developed increasing fatigue and lower back pain over the past year. The pain limits her exercise tolerance such that she can now walk only 1 block. She is a retired schoolteacher who does volunteer efforts in her community but has limited her activities due to fatigue. Karnofsky performance status is 70%. She has a history of chronic hypertension treated with a diuretic, adult-onset diabetes mellitus treated with metformin, and hypothyroidism treated with levothyroxine. Initial evaluation reveals anemia, renal dysfunction, an elevated total protein, and an L2 compression fracture on lumbosacral radiographs. Results of initial and subsequent evaluation are shown below, and she is referred to a hematologist for further evaluation, which revealed the following: calcium 9.0 mg/dL, creatinine 3.2 mg/dL with estimated creatinine clearance using the Modification of Diet in Renal Disease equation of 15 mL/min, hemoglobin 9.6 g/dL, total protein 11 g/dL, albumin 3.2 g/dL, immunoglobulin A (IgA) λ M protein 6.8 g/dL, total IgA 7.2 g/dL, IgG 0.4 g/dL, IgM 0.03 g/dL, free κ <0.01 mg/L, free λ 1000 mg/L, free light chain ratio <0.01, β-2-microglobulin 4.2, viscosity 3.0, lactate dehydrogenase 200 U/L, urine protein electrophoresis: 125 mg/dL with 30% M protein, and urine immuno-electrophoresis: λ light chain. Skeletal bone survey showed lytic lesions in femurs and humeri and diffusely in ribs bilaterally as well as compression fractures at T4, T6, and L2. Bone marrow biopsy revealed λ-restricted plasma cells comprising 50% of the bone marrow core. Fluorescence in situ hybridization testing on marrow showed that del(17p) was present in 80% of the plasma cells. Mrs. A. is informed of the diagnosis of multiple myeloma and the need for therapy. She requests consultation with 2 of the leading world experts. However, she wants to be treated near her home and does not want treatment on a clinical trial."}, {"id": "pubmed23n1014_25813", "title": "[Hypophosphatemic osteomalacia caused by urinary mesenchymal tumor: A case report].", "score": 0.009174311926605505, "content": "This case report concerns a 34-year-old woman who had been diagnosed with ankylosing spondylitis (AS), fibromyalgia syndrome (FMS), osteoarthritis (OA), lumbar disc herniation and the like in different hospitals during the past 18 months. She had progressive osteoarthrosis, significant muscle weakness, gait abnormalities in weightbearing areas, however without typical inflammatory low back pain, while the treatment with non-steroidal anti-inflammatory drugs (NSAIDs) was invalid, with normal inflammation index, negative results for rheumatic factor (RF) and human leukocyte antigen (HLA)-B27, and normal erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). She had hyphosphatemia, normal serum calcium, 1,25-(OH)2-D3 reduction, elevated alkaline phosphatase (ALP) and normal parathyroid hormone (PTH), however with elevated urinary phosphorus. Finally, the medial thigh nodule was found in the subcutaneous of her inner leg by careful examination and imaging scans including B-ultrasound and PET/CT. The final pathology confirmed that the nodule was phosphate urinary mesenchymal tumors. After the tumor was removed, the patient was treated with anti-osteoporosis and phosphorus supplementation. The symptoms of bone pain and muscle weakness were alleviated, and hypophosphatemia was corrected. It was confirmed that the patient had low-phosphorus osteomalacia due to tumor. Tumor-induced hypophosphatemia osteomalacia (TIO) was a rare paraneoplastic syndrome which was caused by excessive phosphorus excretion induced by the tumor, and was thus categorized as an acquired hypophosphatemic osteomalacia. TIO had an occult onset and was associated with a high rate of misdiagnosis, although TIO has some typical clinical features. Early diagnosis, correctly positioning of the tumor, and surgical resection can achieve good outcomes."}, {"id": "pubmed23n0971_4377", "title": "Approach to the treatment of the older, unfit patient with myeloma from diagnosis to relapse: perspectives of a US hematologist and a geriatric hematologist.", "score": 0.00909090909090909, "content": "Mrs. A. is a 73-year-old woman who has developed increasing fatigue and lower back pain over the past year. The pain limits her exercise tolerance such that she can now walk only 1 block. She is a retired schoolteacher who does volunteer efforts in her community but has limited her activities due to fatigue. Karnofsky performance status is 70%. She has a history of chronic hypertension treated with a diuretic, adult-onset diabetes mellitus treated with metformin, and hypothyroidism treated with levothyroxine. Initial evaluation reveals anemia, renal dysfunction, an elevated total protein, and an L2 compression fracture on lumbosacral radiographs. Results of initial and subsequent evaluation are shown below, and she is referred to a hematologist for further evaluation, which revealed the following: calcium 9.0 mg/dL, creatinine 3.2 mg/dL with estimated creatinine clearance using the Modification of Diet in Renal Disease equation of 15 mL/min, hemoglobin 9.6 g/dL, total protein 11 g/dL, albumin 3.2 g/dL, immunoglobulin A (IgA) λ M protein 6.8 g/dL, total IgA 7.2 g/dL, IgG 0.4g/dL, IgM 0.03 g/dL, free κ <0.01 mg/L, free λ 1000 mg/L, serum free light chain ratio <0.01, β-2-microglobulin 4.2, viscosity 3.0, lactate dehydrogenase 200 U/L, urine protein electrophoresis: 125 mg/dL with 30% M protein, and urine immunofixation: λ light chain. Skeletal bone survey showed lytic lesions in femurs and humeri and diffusely in ribs bilaterally as well as compression fractures at T4, T6, and L2. Bone marrow biopsy revealed λ-restricted plasma cells comprising 50% of the bone marrow core. Fluorescence in situ hybridization testing on marrow showed that del 17p was present in 80% of the plasma cells. Mrs. A. is informed of the diagnosis of multiple myeloma and the need for therapy. She requests consultation with 2 of the leading world experts. However, she wants to be treated near her home and does not want treatment on a clinical trial."}, {"id": "pubmed23n0090_5567", "title": "[Hypophosphatemic osteomalacia in adults].", "score": 0.00909090909090909, "content": "Hypophosphataemic osteomalacia occurred in a 38-year-old woman. The leading clinical symptom was severe bone pain. X-ray studies demonstrated fractures of the iliac crest and pubic and ischiadic bone, as well as Looser's zones and demineralization of the skeleton. Computerized densitometry of the bone revealed a 31% reduction of hydroxyapatite. Histological evaluation showed nearly absence of osteoclasts and extensive demineralisation of the bone. Hypophosphataemia (0.48 mmol/l), increased urinary phosphate clearance (36 ml/min), reduced renal-tubular reabsorption for phosphate (73%) and increased alkaline phosphatase (355 U/l) were present. Parathyroid hormone and 1,25-dihydroxyvitamin D were normal. No inborn errors, disturbances of the calcium metabolism or paraneoplastic signs could be detected. Defective renal tubular reabsorption of phosphate is likely to be the underlying cause of the disease. Phosphate supplementation and intermittent vitamin D administration remains the therapy of choice."}, {"id": "pubmed23n0552_13957", "title": "Traumatic fracture in a healthy man: benign or pathologic?", "score": 0.009009009009009009, "content": "To describe the challenge of determining the correct diagnosis in a healthy adult male patient with a recent femoral fracture and a history of multiple bone fractures. We present clinical, radiologic, laboratory, and histopathologic details in a patient with a history of recurrent fractures associated with minimal trauma. Moreover, the various types of osteopetrosis are reviewed. A 34-year-old African American man was in his usual state of good health when he fell hard on concrete. Immediately after the fall, he was able to bear weight, although pain prompted him to seek medical care. Besides a personal history of multiple fractures, he had no other medical problems. He had never smoked, denied illicit drug use, and had no family history of bone disorders or recurrent fractures. Findings on physical examination were unremarkable. Radiography disclosed an incomplete femoral fracture and osteosclerosis. Bone survey revealed diffuse, symmetric osteosclerosis of both the axial and the appendicular skeleton. The long bones showed areas of almost complete obliteration of the medullary canal, along with prominent hyperostosis. Additionally, a \"bone-within-bone\" appearance to the thickened endosteum was noted. A bone scan demonstrated numerous areas of symmetric radiotracer uptake. Laboratory analyses were unremarkable, including a complete blood cell count, electrolytes, serum protein electrophoresis, thyrotropin, and parathyroid hormone. Total alkaline phosphatase was mildly elevated at 162 U/L (normal range, 35 to 130). Seven needles were broken during attempts to perform a bone biopsy. Histologic examination showed normal bone marrow with \"woven\" bone and areas of primary spongiosa within mature osteoid. Autosomal dominant osteopetrosis type 2 was diagnosed on the basis of his clinical presentation and the radiologic and pathologic findings. The preliminary diagnosis for this patient's condition was Paget's disease, and determining the correct diagnosis of osteopetosis prevented the administration of inappropriate therapy. In addition, this case report reminds the clinician that genetic disease may manifest in adulthood."}, {"id": "pubmed23n0510_7049", "title": "[Different forms of clinical presentation of an autosomal dominant hypophosphatemic rickets caused by a FGF23 mutation in one family].", "score": 0.008928571428571428, "content": "In this report we describe different forms of clinical presentation of an autosomal dominant hypophosphatemic rickets (ADHR) in 4 members of the same family as well as the treatment used in these patients and their response to it. Patient No 1: a 60 year old female who consulted for bone pain: Bone densitometry showed osteoporosis. Laboratory assays showed hypophosphatemia with low renal phosphate threshold, high total alkaline phosphatase, normal intact PTH and normal serum calcium. With neutral phosphate and calcitriol, the biochemical parameters normalized and bone densitometry improved significantly in less than a year. Patient No 2 her grand daughter consulted at 1 year and 8 months of age for growth retardation (height at percentile 3) and genu varum. Laboratory assays showed low serum phosphate and high total alkaline phosphatase; thickening and irregular epiphyseal borders of the wrists were observed radiologically. She began treatment with calcitriol and phosphorus with normalization of laboratory parameters and increase in growth (height increasing to percentile 50 after 20 months of therapy). Patient No 3: mother of patient No 2, she had no clinical manifestations and normal densitometry but presented low serum phosphate (1.9 mg/dl) that normalized with neutral phosphate therapy. Patient No 4: he was the youngest son of Patient No 1, who had had hypophosphatemic rickets, by age 5; his serum phosphate normalized without treatment At age 29, he presented normal serum phosphate and bone densitometry. Genomic DNA analysis performed in patient No 3, showed missense mutation with substitution of arginine at position 179 for glutamine. The family was catalogued as having autosomal dominant hypophosphatemic rickets/osteomalacia."}, {"id": "pubmed23n0134_16811", "title": "[Prostatic osteocondensing metastases. The value of examining for osteomalacia].", "score": 0.008928571428571428, "content": "Bone pains observed in patients undergoing estrogen therapy, and presenting with osteoblastic metastases from prostatic cancer are usually related to unsuccessful treatment. In some patients, these pains may result from osteomalacia--ie incomplete mineralization of the new bone--because of the drainage of calcium by the osteoblastic metastases. A clinical, biological and histomorphometric study of bone specimens without decalcification was conducted in ten patients with osteoblastic disease secondary to prostatic carcinoma, who were under estrogen therapy, and for whom a change of therapy was contemplated. The study reports three cases of osteomalacia. Their bone pains were more intense, more diffuse and more permanent than those registered by patients without osteomalacia. All three had had previous fractures of the neck of the femur and a low urinary and serum calcium and phosphorus content. The discovery of osteomalacia by histomorphometric study is important because it allows effective, etiological treatment of the bone pains in these patients."}, {"id": "wiki20220301en178_38961", "title": "Ranson criteria", "score": 0.008849557522123894, "content": "Acute pancreatitis not secondary to gallstones At admission: Blood glucose > 11.11 mmol/L (> 200 mg/dL) Age > 55 years Serum LDH > 350 IU/L Serum AST > 250 IU/L WBC count > 16000 cells/mm3 Within 48 hours: Serum calcium < 2.0 mmol/L (< 8.0 mg/dL) Hematocrit decreased by > 10% Oxygen (hypoxemia with PaO2 < 60 mmHg) BUN increased by 1.8 or more mmol/L (5 or more mg/dL) after IV fluid hydration Base deficit (negative base excess) > 4 mEq/L Sequestration of fluids > 6 L Acute pancreatitis secondary to gallstones At admission: Glucose > 220 mg/dl Age > 70 years LDH > 400 IU/L AST > 250 IU/ 100 ml WBC count > 18000 cells/mm3 Within 48 hours: Serum calcium < 8 mg/dL Hematocrit decreased by > 10% Base deficit > 4 mEq/L BUN increased by > 2 mg/dL Sequestered fluid > 6L"}, {"id": "pubmed23n0297_7819", "title": "[Bone density and laboratory parameters of bone metabolism in patients with terminal heart disease].", "score": 0.008849557522123894, "content": "The purpose of this study was to assess bone mineral density (BMD) and parameters for bone metabolism in patients with end-stage heart disease awaiting heart transplantation to determine whether these patients are at increased risk of bone disease. 39 adult men (mean age 52.3, range 21-65 years) with ischemic (41%), idiopathic (43.5%), valvular (2.5%) and diverse (13%) end-stage heart disease were studied prior to heart transplantation. BMD was measured using dual-energy x-ray absorptiometry at the lumbar spine (L2-L4) and at the upper femur (Ward's triangle and femoral neck). Renal and bone biochemical profiles, parathyroid hormone (PTH), 25-OH-vitamin D3, testosterone, alkaline bone phosphatase, osteocalcin, and deoxypyridinoline and calcium excretion were measured. No fractures were found. Mean z-scores were -1.06 +/- 2.02 (mean +/- SD) at the lumbar spine, -1.12 +/- 2.03 at the femoral neck and -0.25 +/- 1.06 at Ward's triangle. Significantly decreased values were seen at the lumbar spine and at the femoral neck compared to age matched healthy controls. Mean values of serum creatinine and parathyroid hormone were elevated (114.4 +/- 29.5 mumol/l and 84.3 +/- 67.8 ng/l, respectively). 7 (18%), 10 (26%) and 22 (56%) of the 39 patients had values beneath the normal range of 25-OH-vitamin D3, testosterone and osteocalcin respectively. Mean urinary deoxypyridinoline/creatinine ratios were elevated (9.46 +/- 8.52 nmol/mmol, normal range 2.5-5). No correlation existed between osteocalcin and urinary deoxypyridinoline/creatinine ratio. Using a multiple linear regression model, serum PTH and cardiac ejection fraction (EF) weakly predicted BMD at the femoral neck (r2 = 0.26). (1.) Lumbar spine and femoral neck BMD are low in patients awaiting heart transplantation. (2.) A low EF and a high PTH weakly correlate with a decrease in BMD at the femoral neck. (3.) In patients with end-stage heart failure the coupling of bone formation to bone resorption is frequently disturbed. (4.) Testosterone and 25-OH-vitamin D3 levels are low in a number of patients and in such cases replacement therapy may be appropriate."}, {"id": "pubmed23n0059_19216", "title": "Fluoride therapy in postmenopausal osteopenic women: effect on vertebral and femoral bone density and prediction of bone response.", "score": 0.008771929824561403, "content": "Fifty-two postmenopausal women (mean age 60 +/- 5 years) with low BMD (less than -2SD of young adult values) but who had not experienced previous crush fracture were treated with 50 mg of sodium fluoride (NaF), 1 g of calcium and 400 IU of vitamin D2 per day for 2 years. Repeated vertebral and femoral BMD measurements were made and compared with those of a control group consisting of 16 untreated women. Serum alkaline phosphatase and osteocalcin, blood and urinary fluoride levels were measured regularly to determine their predictive value on bone response. 18 of 52 (35%) of the treated patients experienced side effects (29% gastric, 4% lower extremity pain syndrome) but only in 6 cases (12%) was it necessary to discontinue treatment. In neither of the two groups was any fracture recorded (vertebral or otherwise). Among the 43 women who were treated for at least 2 years, 21 (49%) were considered to have responded (i.e., with an increase of vertebral BMD greater than 0.043 g/cm2). There was a mean linear increase in BMD in this group of 0.0041 g/cm2 per month (i.e., 5.5% per year). On the other hand in the non-responder group and in the control group, vertebral BMD either remained stable or decreased. However no difference was detected between the two groups (treated and controls) at the femoral site after 2 years; both groups showed a significant decrease in BMD. The responders had a lower initial osteocalcin level and treatment led to a relatively greater increase in osteocalcin.(ABSTRACT TRUNCATED AT 250 WORDS)"}]}}}} {"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 108]], "word_ranges": [[0, 21]], "text": "The first step is to detect the mutation and once detected, if the mother is a carrier, to proceed with PGD."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The first step is to detect the mutation and once detected, if the mother is a carrier, to proceed with PGD.", "full_answer_no_ref": "The first step is to detect the mutation and once detected, if the mother is a carrier, to proceed with PGD.", "full_question": "An 8-year-old girl (index case) is clinically diagnosed as having neurofibromatosis type 1 (NF1) or Von Recklinghausen's disease with multiple neurofibromas, café-au-lait spots, and Lisch nodules. Her father (not diagnosed with NF1) died in a traffic accident at the age of 38 years. On examination, the mother presents two café-au-lait spots and attends the genetic counseling consultation with her new partner where a preimplantation genetic diagnosis (PGD) is proposed. Is PGD indicated in this case?", "id": 107, "lang": "en", "options": {"1": "Yes, as the mother has 2 café-au-lait spots, she is a carrier and PGD is indicated with these data.", "2": "It is indicated after detection of the causative mutation in the index case and eventually in his mother.", "3": "It is not indicated because NF1 responds to mutations in the neurofibromin gene (17q11.2), with recessive inheritance.", "4": "No, two café-au-lait spots are not diagnostic and your new partner is very unlikely to be a carrier (NF1 is a rare disease).", "5": "With these data, PGD is indicated, consisting of selecting embryos in vitro, to implant in the maternal uterus those without the mutation."}, "question_id_specific": 206, "type": "GENETICS AND IMMUNOLOGY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en021_104825", "title": "Preimplantation genetic diagnosis", "score": 0.013717783992139672, "content": "Germany Before 2010, the usage of PGD was in a legal grey area. In 2010, the Federal Court of Justice of Germany ruled that PGD can be used in exceptional cases. On 7 July 2011, the Bundestag passed a law that allows PGD in certain cases. The procedure may only be used when there is a strong likelihood that parents will pass on a genetic disease, or when there is a high genetic chance of a stillbirth or miscarriage. On 1 February 2013, the Bundesrat approved a rule regulating how PGD can be used in practice. Hungary In Hungary, PGD is allowed in case of severe hereditary diseases (when genetic risk is above 10%). The preimplantation genetic diagnosis for aneuploidy (PGS/PGD-A) is an accepted method as well. It is currently recommended in case of multiple miscarriages, and/or several failed IVF treatments, and/or when the mother is older than 35 years. Despite being an approved method, PGD-A is available at only one Fertility Clinic in Hungary."}, {"id": "wiki20220301en021_104751", "title": "Preimplantation genetic diagnosis", "score": 0.01284229788902686, "content": "PGD is also now being performed in a disease called hereditary multiple exostoses (MHE/MO/HME). In addition, there are infertile couples who carry an inherited condition and who opt for PGD as it can be easily combined with their IVF treatment. Pregnancy chances Preimplantation genetic profiling (PGP) has been suggested as a method to determine embryo quality in in vitro fertilization, in order to select an embryo that appears to have the greatest chances for successful pregnancy. However, as the results of PGP rely on the assessment of a single cell, PGP has inherent limitations as the tested cell may not be representative of the embryo because of mosaicism. Furthermore, a study found that diagnoses of the biopsies from the same embryos at two separate laboratories matched up only 50% of the time."}, {"id": "wiki20220301en021_104744", "title": "Preimplantation genetic diagnosis", "score": 0.012408012408012408, "content": "The world's first PGD was performed by Handyside, Kontogianni and Winston at the Hammersmith Hospital in London. Female embryos were selectively transferred in five couples at risk of X-linked disease, resulting in two twins and one singleton pregnancy. The term preimplantation genetic screening (PGS) refers to the set of techniques for testing whether embryos (obtained through IVF/ICSI) have abnormal chromosomes' number. In other words, it tests if an embryo is aneuploid or not. PGS is also called aneuploidy screening. PGS was renamed preimplantation genetic diagnosis for aneuploidy (PGD-A) by Preimplantation Genetic Diagnosis International Society (PGDIS) in 2016. The PGD allows studying the DNA of eggs or embryos to select those that carry certain mutations for genetic diseases. It is useful when there are previous chromosomal or genetic disorders in the family and within the context of in vitro fertilization programs."}, {"id": "wiki20220301en021_104758", "title": "Preimplantation genetic diagnosis", "score": 0.01070230688480938, "content": "the disease. Males on the other hand only require one copy of the mutant X allele for the disease to occur in one's phenotype and therefore, the male offspring of a carrier mother has a 50% chance of having the disease. Reasons may include the rarity of the condition or because affected males are reproductively disadvantaged. Therefore, medical uses of PGD for selection of a female offspring to prevent the transmission of X-linked Mendelian recessive disorders are often applied. Preimplantation genetic diagnosis applied for gender selection can be used for non-Mendelian disorders that are significantly more prevalent in one sex. Three assessments are made prior to the initiation of the PGD process for the prevention of these inherited disorders. In order to validate the use of PGD, gender selection is based on the seriousness of the inherited condition, the risk ratio in either sex, or the options for disease treatment."}, {"id": "wiki20220301en021_104800", "title": "Preimplantation genetic diagnosis", "score": 0.010352880124545927, "content": "Preimplantation genetic haplotyping Preimplantation genetic haplotyping (PGH) is a PGD technique wherein a haplotype of genetic markers that have statistical associations to a target disease are identified rather than the mutation causing the disease. Once a panel of associated genetic markers have been established for a particular disease it can be used for all carriers of that disease. In contrast, since even a monogenic disease can be caused by many different mutations within the affected gene, conventional PGD methods based on finding a specific mutation would require mutation-specific tests. Thus, PGH widens the availability of PGD to cases where mutation-specific tests are unavailable."}, {"id": "pubmed23n0890_15646", "title": "Novel phenotypes of NF1 patients from unrelated Chinese families with tibial pseudarthrosis and anemia.", "score": 0.009900990099009901, "content": "Neurofibromatosis type 1 (NF1) is an autosomal dominant, multi-system, neurocutaneous disorder, manifested with neurofibromas and Cafe´-au-lait spots. Germline mutations in NF1 gene are associated with Neurofibromatosis type 1. NF1 gene encodes neurofibromin, a RAS-specific GTPase activating protein. In our study, we present a clinical molecular study of four Chinese probands with NF1 from four unrelated families, showing extreme phenotypic variation with rare phenotype. In family 1, the proband is a 16 months old girl with multiple café-au-lait spots throughout her whole body. In family 2, the proband is a 6 months old girl with several café-au-lait spots mostly in her trunk and in lower limbs. In family 3, the proband is a 4 months old boy with several café-au-lait spots, tibial pseudarthrosis, and chronic iron deficiency anemia. In family 4, the proband is a 14 years old boy with multiple café-au-lait spots of variable sizes. Targeted exome capture based next generation sequencing and Sanger sequencing identified a novel mutation and three previously reported mutations in these four probands. These four mutations in NF1 gene were causing disease phenotypes in these four probands and was absent in unaffected family members and in healthy controls. According to the variant interpretation guideline of American College of Medical Genetics and Genomics (ACMG), these four mutations, are classified as \"likely pathogenic\". Our result expands the mutational spectrum of the NF1 gene associated with neurofibromatosis type1."}, {"id": "wiki20220301en021_104754", "title": "Preimplantation genetic diagnosis", "score": 0.009861317721579731, "content": "Cancer predisposition A more recent application of PGD is to diagnose late-onset diseases and (cancer) predisposition syndromes. Since affected individuals remain healthy until the onset of the disease, frequently in the fourth decade of life, there is debate on whether or not PGD is appropriate in these cases. Considerations include the high probability of developing the disorders and the potential for cures. For example, in predisposition syndromes, such as BRCA mutations which predispose the individual to breast cancer, the outcomes are unclear. Although PGD is often regarded as an early form of prenatal diagnosis, the nature of the requests for PGD often differs from those of prenatal diagnosis requests made when the mother is already pregnant. Some of the widely accepted indications for PGD would not be acceptable for prenatal diagnosis."}, {"id": "pubmed23n0958_20223", "title": "[A case of growth hormone deficiency combined with neurofibromatosis Type 1 and its gene analysis].", "score": 0.00980392156862745, "content": "Neurofibromatosis Type 1 (NF1) is an autosomal dominant genetic disorder caused by NF1 gene mutations. Café au lait spots, neurofibromatosis, Lisch nodules, axillary freckling, dermal neurofibromas and skeletal dysplasia are the most common manifestations for this disease. A 11-year-old boy visited Third Xiangya Hospital, Central South University due to growth-retardation. He was eventually diagnosed as NF1 with growth hormone deficiency. A novel heterozygous splicing mutation c.6579+2 T>C (IVS 34+2 T>C) of NF1 gene was identified in the patient and his mother. Considering NF1 may present with short stature due to growth hormone deficiency, all children with short stature combined with café au lait spots should be screened for NF1, which may assist the clinical diagnosis and the genetic counseling."}, {"id": "pubmed23n0623_2360", "title": "[Mastenbroek controversy or how much ink is spilled on preimplantation genetic screening subject].", "score": 0.00980392156862745, "content": "Preimplantation genetic screening (PGS) of in vitro fertilization (IVF) embryos has been used for advanced maternal age, repeated miscarriages and repeated implantation failure indications. Several non-randomized studies have been published, showing increased implantation rates, decreased miscarriages and trisomy rates. So PGS seemed to improve prognosis for this particular population. In 2004, a prospective randomized study tempered those results, being unable to demonstrate any significant difference of live birth rate with and without PGS in case of advanced maternal age. In July 2007, another multicenter randomized double-blind trial definitely reopened the controversy, reporting that PGS did not increase but instead significantly reduced pregnancy and live birth rates after IVF in women 35 years of age or older. The debate about efficiency and usefulness of PGS is ongoing and other powered randomized studies will be needed to conclude about real PGS usefulness."}, {"id": "pubmed23n0838_1940", "title": "[Anxiety disorders in type 1 neurofibromatosis: A case report].", "score": 0.009708737864077669, "content": "Neurofibromatosis type 1 (NF1), also known as Von Recklinghausen disease, is one of the most frequent human genetic diseases, with a prevalence of one case in 3000 births, an autosomal dominant mode of inheritance, and a high rate of new mutations. NF1 has markedly variable clinical expression, with manifestations ranging from mild lesions to several complications and functional impairment. The complications are age-specific. Psychiatric disorders are more frequent in NF1 than in the general population, especially in children. They include dysthymia, depressive mood, anxiety, and personality disorders. Bipolar mood disorders or schizophrenia are rather rare. The majority of studies have focused on physical health and neurocognitive function in NF1, whereas psychiatric disorders associated with this disease remain unclear and poorly documented. This report is based on a clinical case and discusses the relationship between neurofibromatosis type 1 and psychiatric disorders, particularly anxiety disorders. This case concerns a 13-year-old girl, the first child of healthy and non-consanguineous parents. The patient's history showed normal psychomotor and psychoaffective development. Her father and paternal grandmother had isolated café-au-lait spots. In June 2013, a subcutaneous mass appeared in her right thigh. She consulted a neurologist and was explored. The physical examination revealed signs of NF1. She had café-au-lait spots on the trunk and extremities, and a neurofibroma in the right thigh. Bilateral ophthalmic examination revealed multiple Lish nodules. After 1 month, a psychiatric consultation was requested for sad mood and night terrors. Obsessive compulsive disorder and generalized anxiety disorder were diagnosed according to the criteria of the Diagnostic and Statistical Manual of Mental Disorders, 4th edition. The current psychiatric literature does not provide full explanations of anxiety symptoms associated with NF1. Some authors have tried to explain the link between NF1 and psychiatric disorders, and several etiopathogenic hypotheses have been discussed. In our case, a concomitant diagnosis of NF1 and anxiety disorders was made at the age of 13. However, anxiety symptoms started to appear before age 4; they increased gradually and occupied the foreground. This would strengthen the hypothesis of genetic determinism in NF1 patients. The question that arises is: is it a fortuitous association of psychiatric disorders and NF1 or are they psychiatric manifestations induced by a multisystem disease? More detailed investigations are necessary to clarify the etiopathogenic and psychopathological mechanisms that would cause psychiatric comorbidity associated with NF1."}, {"id": "pubmed23n0719_14238", "title": "[Ethical aspects of preimplantation genetic diagnosis (PGD)].", "score": 0.009708737864077669, "content": "The controversy surrounding PGD has not abated in recent times. This is especially the case for PGD-based tissue typing, which is used to select a future child who could serve as a stem cell donor for an older sick sibling. We examine three types of ethical argument cited against PGD in general, and specifically against tissue-typing PGD. These arguments focus on the moral status of the early embryo, the eugenics issue, and the charge that the future child is being exploited. We conclude that none of these three arguments is unassailable, and that it is the reproductive freedom of couples considering PGD that should prevail."}, {"id": "pubmed23n0986_22293", "title": "Next Generation Sequencing Identified a Novel Multi Exon Deletion of the NF1 Gene in a Chinese Pedigree with Neurofibromatosis Type 1.", "score": 0.009615384615384616, "content": "Neurofibromatosis type 1 (NF1) is a genetic disease involving neurocutaneous abnormalities. Neurofibromatosis type 1 is an autosomal dominant disorder characterized by the neurofibromas and café-au-lait spots. Mutation in the NF1: c.5392C>T Mutation.", "score": 0.009523809523809525, "content": "Neurofibromatosis type 1 (NF1) is a neurocutaneous syndrome caused by mutations on the 2 units but variable (except in children) Plasma proteins show characteristic changes. Plasma albumin level is low, but plasma globulins are raised due to an increased formation of antibodies. Unconjugated bilirubin is hydrophobic, so cannot be excreted in urine. Thus, the finding of increased urobilinogen in the urine without the presence of bilirubin in the urine (due to its unconjugated state) suggests hemolytic jaundice as the underlying disease process. Urobilinogen will be greater than 2 units (i.e., hemolytic anemia causes increased heme metabolism; exception: infants where gut flora has not developed). Conversely, conjugated bilirubin is hydrophilic and thus can be detected as present in the urine — bilirubinuria — in contrast to unconjugated bilirubin which is absent in the urine."}, {"id": "wiki20220301en577_5317", "title": "Bilirubin glucuronide", "score": 0.011494252873563218, "content": "At birth, infants don't develop enough ability to conjugate bilirubin. Up to 8% to 11% neonates will develop hyperbilirubinemia in the first week of their lives. Hemolytic jaundice In jaundice owing to hemolysis (Prehepatic (or hemolytic) jaundice), the pathophysiology is that overproduction of bilirubin from the extravascular or intravascular hemolysis overwhelms the capacity of the liver to excrete it. The bilirubin present in the plasma is largely unconjugated in this setting as they haven't been taken up and conjugated by the liver. In this case, total serum bilirubin increases while the ratio of direct bilirubin to indirect bilirubin remains 96 to 4 as up to 96%-99% of bilirubin in the bile are conjugated mentioned above. Brain damage"}, {"id": "wiki20220301en107_51180", "title": "Neonatal cholestasis", "score": 0.011433664374840845, "content": "Neonatal cholestasis defines persisting conjugated hyperbilirubinemia in the newborn with conjugated bilirubin levels exceeding 15% (5.0 mg/dL) of total bilirubin level. The disease is either due to defects in bile excretion from hepatocytes or impaired bile flow. General presentations in neonates include abdominal pain and general gastrointestinal upset. Physical examination may show palpable liver and enlarged spleen. Differential diagnosis typically presents with a host of possibilities, many of them not treatable. Histopathology shows dilated bile duct system at all levels and bile duct proliferation in response to back pressure. The incidence has been found to be about 1 in 2,500 live births. See also Neonatal jaundice References Neonatology"}, {"id": "wiki20220301en059_49919", "title": "Neonatal jaundice", "score": 0.011324849561014108, "content": "A bilirubin level more than 34 μmol/l (2 mg/dL) may be visible. For the feet to be affected level generally must be over 255 μmol/l (15 mg/dL). Complications Prolonged hyperbilirubinemia (severe jaundice) can result in chronic bilirubin encephalopathy (kernicterus). Quick and accurate treatment of neonatal jaundice helps to reduce the risk of neonates developing kernicterus. Infants with kernicterus may have a fever or seizures. High pitched crying is an effect of kernicterus. Exchange transfusions performed to lower high bilirubin levels are an aggressive treatment. Causes In newborns, jaundice tends to develop because of two factors—the breakdown of fetal hemoglobin as it is replaced with adult hemoglobin and the relatively immature metabolic pathways of the liver, which are unable to conjugate and so excrete bilirubin as quickly as an adult. This causes an accumulation of bilirubin in the blood (hyperbilirubinemia), leading to the symptoms of jaundice."}, {"id": "article-18589_12", "title": "Breast Milk Jaundice -- Evaluation", "score": 0.011269644334160462, "content": "The evaluation of a patient presenting with hyperbilirubinemia must include a work-up to rule out pathological causes of hyperbilirubinemia before making the breast milk jaundice diagnosis. First, both unconjugated and conjugated bilirubin levels must be measured. Conjugated bilirubin levels higher than 1 mg/dL or 20% of the total bilirubin level indicate conjugated hyperbilirubinemia (also known as cholestasis or direct hyperbilirubinemia). Once cholestasis is suspected, disorders such as biliary atresia, neonatal hepatitis, and other bilirubin excretion disorders. Both breast milk jaundice and hemolytic anemias cause elevated unconjugated bilirubin levels. Hemolytic causes for hyperbilirubinemia include ABO incompatibility, G6PD deficiency, hereditary spherocytosis, and other antibody-mediated hemolysis. Hemolysis assessment should consist of direct Coombs’ testing, measurement of hemoglobin, hematocrit, and reticulocyte count, a peripheral blood smear, and genetic testing."}, {"id": "wiki20220301en003_62164", "title": "Jaundice", "score": 0.011227973694461631, "content": "Posthepatic pathophysiology Posthepatic jaundice (obstructive jaundice) is due to a blockage of bile excretion from the biliary tract → increased conjugated bilirubin and bile salts. In complete obstruction of the bile duct, conjugated bilirubin cannot access the intestinal tract → no further bilirubin conversion to urobilinogen → no stercobilin or urobilin. Instead, excess conjugated bilirubin is filtered into the urine without urobilinogen in obstructive jaundice. Conjugated bilirubin in urine (bilirubinuria) gives urine an abnormally dark brown color. Thus, the presence of pale stool (stercobilin absent from feces) and dark urine (conjugated bilirubin present in urine) suggest an obstructive cause of jaundice. Because these associated signs are also positive in many hepatic jaundice conditions, they cannot be a reliable clinical feature to distinguish obstruction versus hepatocellular jaundice causes. Diagnosis"}, {"id": "wiki20220301en059_49923", "title": "Neonatal jaundice", "score": 0.011218568665377175, "content": "Post-liver Biliary atresia or bile duct obstruction Alagille syndrome Choledochal cyst Non-organic causes Breastfeeding jaundice \"Breastfeeding jaundice\" (or \"lack of breastfeeding jaundice\") is caused by insufficient breast milk intake, resulting in inadequate quantities of bowel movements to remove bilirubin from the body. This leads to increased enterohepatic circulation, resulting in increased reabsorption of bilirubin from the intestines. Usually occurring in the first week of life, most cases can be ameliorated by frequent breastfeeding sessions of sufficient duration to stimulate adequate milk production."}, {"id": "wiki20220301en003_62150", "title": "Jaundice", "score": 0.01120199146514936, "content": "Treatment of jaundice is typically determined by the underlying cause. If a bile duct blockage is present, surgery is typically required; otherwise, management is medical. Medical management may involve treating infectious causes and stopping medication that could be contributing to the jaundice. Jaundice in newborns may be treated with phototherapy or exchanged transfusion depending on age and prematurity when the bilirubin is greater than 4–21 mg/dl (68-360 μmol/L). The itchiness may be helped by draining the gallbladder, ursodeoxycholic acid, or opioid antagonists such as naltrexone. The word \"jaundice\" is from the French jaunisse, meaning \"yellow disease\". Signs and symptoms"}, {"id": "wiki20220301en003_84271", "title": "Bilirubin", "score": 0.011164372336938708, "content": "Very high levels of bilirubin may be caused by: Neonatal hyperbilirubinemia, where the newborn's liver is not able to properly process the bilirubin causing jaundice Unusually large bile duct obstruction, e.g. stone in common bile duct, tumour obstructing common bile duct etc. Severe liver failure with cirrhosis (e.g. primary biliary cirrhosis) Crigler–Najjar syndrome Dubin–Johnson syndrome Choledocholithiasis (chronic or acute). Cirrhosis may cause normal, moderately high or high levels of bilirubin, depending on exact features of the cirrhosis. To further elucidate the causes of jaundice or increased bilirubin, it is usually simpler to look at other liver function tests (especially the enzymes alanine transaminase, aspartate transaminase, gamma-glutamyl transpeptidase, alkaline phosphatase), blood film examination (hemolysis, etc.) or evidence of infective hepatitis (e.g., hepatitis A, B, C, delta, E, etc.). Jaundice"}, {"id": "wiki20220301en624_24990", "title": "Hemolytic jaundice", "score": 0.010928553647000249, "content": "As one of the three categories of jaundice, the most obvious sign of hemolytic jaundice is the discolouration or yellowing of the sclera and the skin of the patient, but additional symptoms may be observed depending on the underlying causes of hemolysis. Hemolytic causes associated with bilirubin overproduction are diverse and include disorders such as sickle cell anemia, hereditary spherocytosis, thrombotic thrombocytopenic purpura, autoimmune hemolytic anemia, hemolysis secondary to drug toxicity, thalassemia minor, and congenital dyserythropoietic anemias. Pathophysiology of hemolytic jaundice directly involves the metabolism of bilirubin, where overproduction of bilirubin due to hemolysis exceeds the liver's ability to conjugate bilirubin to glucuronic acid."}, {"id": "wiki20220301en059_49924", "title": "Neonatal jaundice", "score": 0.010825499090570422, "content": "Breast milk jaundice Whereas breastfeeding jaundice is a mechanical problem, breast milk jaundice is a biochemical occurrence and the higher bilirubin possibly acts as an antioxidant. Breast milk jaundice occurs later in the newborn period, with the bilirubin level usually peaking in the sixth to 14th days of life. This late-onset jaundice may develop in up to one third of healthy breastfed infants."}]}}}} {"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[92, 222]], "word_ranges": [[13, 33]], "text": "Elderly patient with clinical dyspnea and syncope, and systolic murmur radiating to carotids and tip, all typical of this disease."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Very easy question. They are describing the \"portrait\" of a degenerative aortic stenosis... Elderly patient with clinical dyspnea and syncope, and systolic murmur radiating to carotids and tip, all typical of this disease.", "full_answer_no_ref": "Very easy question. They are describing the \"portrait\" of a degenerative aortic stenosis... Elderly patient with clinical dyspnea and syncope, and systolic murmur radiating to carotids and tip, all typical of this disease.", "full_question": "An 81-year-old patient consults for an episode of syncope. He reports dyspnea on exertion for a year. On cardiac auscultation there is a 3/6 systolic murmur on the left sternal border radiating to the carotids and tip. Which pathology seems most likely?", "id": 87, "lang": "en", "options": {"1": "Third-degree atrioventricular block.", "2": "Degenerative aortic stenosis.", "3": "Mitral valve insufficiency.", "4": "Hypertrophic cardiomyopathy.", "5": "Dilated cardiomyopathy."}, "question_id_specific": 54, "type": "CARDIOLOGY AND VASCULAR SURGERY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "First_Aid_Step1_294", "title": "First_Aid_Step1", "score": 0.0179549114331723, "content": "Auscultation of the heart (eg, physiologic murmur) Aortic valve sclerosis Where to listen: APT M 111234567777777 Left sternal border: Aortic regurgitation Pulmonic regurgitation cardiomyopathy PA M T Aortic area: Systolic murmur Pulmonic area: Tricuspid area: Mitral area (apex): Crescendo-decrescendo systolic ejection murmur and soft S2 (ejection click may be present). LV >> aortic pressure during systole. Loudest at heart base; radiates to carotids. “Pulsus parvus et tardus”—pulses are weak with a delayed peak. Can lead to Syncope, Angina, and Dyspnea on exertion (SAD). Most commonly due to age-related calcification in older patients (> 60 years old) or in younger patients with early-onset calcification of bicuspid aortic valve."}, {"id": "pubmed23n0080_2252", "title": "[Mitral valve prolapse as an initial clinical feature of dilated cardiomyopathy: report of two cases].", "score": 0.0172823023290313, "content": "Two cases with mitral valve prolapse (MVP), without any other cardiac abnormalities at the initial evaluation, developed the clinical features mimic to dilated cardiomyopathy (DCM) during follow-up period. Case 1. A 40-year-old man visited our hospital in May 1982 to evaluate a heart murmur. A standard 12-lead electrocardiogram (ECG) showed an abnormal Q wave in lead III. Echocardiography revealed MVP, but neither dilatation nor wall motion abnormality of the left ventricle (LV) were observed. Thallium-201 scintigraphy revealed an abnormal thallium uptake at the apex and inferior wall. He had no episode of acute myocardial infarction or myocarditis, but complete right bundle branch block developed, thus, he was hospitalized in October 1984. He had no coronary artery lesions, and had only mild mitral regurgitation on left ventriculography. The motion of the interventricular septum and apex was reduced on echocardiogram and a persistent perfusion defect was observed at the inferior wall and the interventricular septum on Tl-201 scintigrams. In December 1985, he experienced an Adams-Stokes attack due to complete atrioventricular block. Echocardiographically, the left ventricle enlarged, and the wall motion abnormalities and a perfusion defect on Tl-201 scintigrams were relatively severe. Case 2. A 46-year-old woman occasionally experienced palpitation of short duration and chest oppression since 1977. She was admitted to our hospital because of cardiac symptoms in 1982. A heart murmur of Levine II was heard and a standard 12-lead ECG showed single supraventricular extrasystole and T wave inversion in lead III and aVF. Echocardiography revealed MVP and mild mitral regurgitation, but neither dilatation nor wall motion abnormality of the LV was observed. During 6-year follow-up period, permanent atrial fibrillation developed and LV developed dilatation and wall motion abnormalities progressed. Thus, during follow-up periods, DCM-like features developed in two cases who had had MVP as a sole echocardiographic abnormality with systolic murmur and non-specific ECG changes. We consider that these two may be important cases who may show a relation between cardiomyopathic process and MVP."}, {"id": "wiki20220301en063_19521", "title": "Valvular heart disease", "score": 0.017027417027417027, "content": "Medical signs of aortic stenosis include pulsus parvus et tardus, that is, diminished and delayed carotid pulse, fourth heart sound, decreased A2 sound, sustained apex beat, precordial thrill. Auscultation may reveal a systolic murmur of a harsh crescendo-decrescendo type, heard in 2nd right intercostal space and radiating to the carotid arteries. Aortic regurgitation Patients with aortic regurgitation may experience heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea, palpitations, and angina pectoris. In acute cases patients may experience cyanosis and circulatory shock."}, {"id": "pubmed23n0203_11719", "title": "[beta-Blocking therapy in obstructive hypertrophic cardiomyopathy. Long term results (author's transl)].", "score": 0.016797541823147834, "content": "34 patients have been controlled after beta-blocking therapy, for a mean period of 5 years. Symptoms and evolution: syncope disappeared, angoy passed from 47% to 23%, dyspnea from 65% to 47%, dizziness from 70% to 54%, weakness from 30% to 37%. A systolic murmur was present in 75% of the cases. Two patients died by heart failure. Phonocardiogram: the systolic murmur was unchanged, like the carotid pulse. Paradoxical splitting of the 2 degrees sound was more frequent, atrial sound unimodified, isometric contraction shortened (60%) and the Q-1 degree sound interval prolonged (90%). Electrocardiogram: 1 degree A/V block appeared in 24% of the cases, complete A/V block in 9%, atrial fibrillation in 3%. Left atrial enlargement was more frequent; left ventricular hypertrophy unchanged. Heart catheterization (10 cases, after a mean period of 5.5 years): left ventricular pressure gradient passed from 80% to 90%; a low cardiac index from 20% to 30%; telediastolic pressure of left ventricle was unmmodified in 10% of cases, more elevated in 50%, less elevated in 40%. Chest X ray: cardiac size was unchanged in 65% of cases, enlarged in 32%; smaller in 3%. In conclusion, symptoms improved in most of the patients; no case of sudden death was observed. Some data however show that the evolution of the myocardiopathy goes on to congestive heart failure and arise doubts on the real usefullness of beta-blocking drugs in the disease."}, {"id": "pubmed23n0006_10965", "title": "[Various etiologies of systolic murmurs radiating from the apex of the heart to the neck].", "score": 0.016508152173913042, "content": "The authors studied systolic murmurs in 89 cases, 50 of aortic stenosis, 14 cases of obstructive cardiomyopathy and 20 cases of mitral incompetence. This systolic murmur is characterised by its exceptional intensity, its raspy character at the base, becoming softer at the apex and in the axilla, the presence of a thrill and irradiation into the neck in 50 to 75% of cases. The etiological diagnosis was ensured precisely by 1) pharmaco-dynamic tests: amyl nitrite accentuates the systolic ejection murmurs and attenuates murmurs due to mitral regurgitation. 2) careful analysis of diastole: a systolic murmur extending into early diastole, a third sound or an opening snap and a low-pitched diastolic murmur, suggest mitral incompetence. A high-pitched diastolic murmur is in favour of aortic stenosis. 3) the carotid arteriogram and catheterisation show the characteristic abnormalities of the carotid arteriogram found in aortic valve disease and the existence of a trans-aortic or intra-ventricular pressure gradient, when there is an obstruction to left ventricular jection. The F wave of the apex cardiogram or left atrial reflux of the contrast medium during cineangiocardiography, confirm mitral incompetence. The main phono-hemodynamic and phono-anatomical correlations have been emphasized: 1. The intensity of the systolic murmur is directly proportional to the degree of obstructive cardiomyopathy or mitral incompetence, but does not parallel the degree of the sub-valvular apparatus. 3. The maximum intensity of the murmur occurs all the later when the stenosis is tight, whilst it is earlier in severe obstructive cardio-myopathy. 4. The lozange shape of the murmur of mitral incompetence on phono-cardiography is, above all, due to those cases with lesions of the sub-valvular apparatus. Finally, a study of the sound recorded by the Allard-Laurens micromanometer permitted us to determine the mechanism of this irradiating systolic murmur."}, {"id": "pubmed23n0748_20080", "title": "[Clinical features of five patients with delayed third degree atrioventricular block after ethanol septal ablation for hypertrophic obstructive cardiomyopathy].", "score": 0.015297202797202798, "content": "To analyze the clinical features of patients with delayed third degree atrioventricular block after ethanol septal ablation for hypertrophic obstructive cardiomyopathy. The clinical data of cases with delayed third degree atrioventricular block after septal ablation for hypertrophic obstructive cardiomyopathy at our hospital from 2000 to 2011 were collected. Five out of 235 patients (2.1%) developed delayed third degree atrioventricular block. Delayed third atrioventricular block occurred at 32 h post ablation (28 - 120 h). Their average age is 46 (33 - 64) years old, there are 4 males and 1 female. Left ventricular outflow gradient before ablation was 70 - 100 mm Hg (1 mm Hg = 0.133 kPa). Intraprocedural third degree atrioventricular block occurred in 4 patients. The average injected dose of Ethanol was 1.8 (1.4 - 4.3) ml. Syncope occurred in 3 patients. Temporary pacemaker was reimplanted to all 5 patients and removed after an average of 8 d (3 - 18 d). All 5 patients were in normal sinus rhythms at discharge without the need of implanting permanent pacemaker. There was no syncope in these 5 patients after discharge during the telephone follow up for an average of 9 (1 - 72) months. The incidence of delayed third degree atrioventricular block after septal ablation is rare. Prolonged electrocardiography monitoring and prophylactic temporary pacemaker backup post ablation are necessary to detect this event and to prevent syncope related to delayed third degree atrioventricular block after septal ablation."}, {"id": "pubmed23n0821_13278", "title": "[Echographic diagnosis of systolic murmur among 280 young French militaries. Implications for the expertise in military medicine].", "score": 0.014663511010495987, "content": "Clinically discovering a systolic murmur is frequent among the young military population. When this murmur does not sound benign, a transthoracic echocardiography (TTE) is made to detect any cardiopathy, which could cause sudden cardiac death. The aim of this study was to evaluate the interest of systematic TTE in the assessment of any cardiac systolic murmur (CSM) among militaries. We ran a retrospective monocentric study in the \"Clermont-Tonnerre\" military hospital in Brest. We included all patients sent for TEE, aged 15 to 30 years old, from the 1st January 2010 until the 31st July 2013. Two hundred and eighty TTES assessing CSM were performed. We found 28/280 (10%) echocardiographic abnormalities: 13 were bicuspid aortic valves (4.6%), 6 were ventricular septal defects (2.15%), 3 were atrial septal defects (1.07%), 4 were mild mitral regurgitations (1.43%), one mild pulmonary stenosis (0.35%) and one aortic stenosis (0.35%). No hypertrophic cardiomyopathy was found. Concerning military expertise, 11 (3.92%) patients among these 28 with abnormal TEE were considered unfit for work or \"fit for work with limitations\". Assessing a cardiac systolic murmur with TEE lead to the diagnosis of a cardiomyopathy in 10% of the case. This study enhances the importance of systematic TEE when a CSM is detected in the young military, in order to determine if those soldiers can still fulfill their military duty."}, {"id": "pubmed23n0505_1348", "title": "[Aortic valve stenosis].", "score": 0.012861869313482217, "content": "Aortic valve stenosis is the most frequent reason for prosthetic valve replacement in adults. Its incidence increases with age. Development of the most frequent form, degenerative-calcific aortic stenosis, is related to atherosclerotic risk factors. The narrowing of the aortic valve orifice leads to creation of a systolic pressure drop, the gradient, between left ventricle and ascending aorta. The pressure overload from aortic stenosis causes concentric left ventricular hypertrophy and later heart failure. Typical symptoms of severe aortic stenosis include dyspnea, angina, and dizziness or syncope. On auscultation, a loud systolic murmur over the base of the heart is apparent, which is transmitted to the carotids. The ECG often shows left ventricular hypertrophy. The most important diagnostic technique is echocardiography, which allows to measure the gradient and to calculate the orifice area, which determine the degree of severity. The development of symptoms or impaired left ventricular function in severe aortic stenosis should prompt surgical treatment by valve replacement. Truly asymptomatic patients with preserved left ventricular function should be followed conservatively."}, {"id": "wiki20220301en010_85067", "title": "Aortic stenosis", "score": 0.012677484787018255, "content": "An easily heard systolic, crescendo-decrescendo (i.e., 'ejection') murmur is heard loudest at the upper right sternal border, at the 2nd right intercostal space, and radiates to the carotid arteries bilaterally. The murmur increases with squatting and decreases with standing and isometric muscular contraction such as the Valsalva maneuver, which helps distinguish it from hypertrophic obstructive cardiomyopathy (HOCM). The murmur is louder during expiration but is also easily heard during inspiration. The more severe the degree of the stenosis, the later the peak occurs in the crescendo-decrescendo of the murmur."}, {"id": "pubmed23n0117_703", "title": "[Regression of mitral valve prolapse to a state masquerading as dilated cardiomyopathy: a case report].", "score": 0.012662337662337663, "content": "A 40-year-old man was admitted to our hospital in May 1982 for evaluation of a heart murmur. A standard 12-lead electrocardiogram (ECG) showed an abnormal Q wave in lead III. Echocardiography revealed prolapse of the anterior mitral valve leaflet (MVP), but neither dilatation nor wall motion abnormalities of the left ventricle (LV) were observed. Thallium-201 scintigraphy revealed an abnormal thallium uptake at the apex and inferior wall. He had no episode of acute myocardial infarction or myocarditis, but complete right bundle branch block suddenly appeared, and he was hospitalized in October 1984. He had no coronary artery lesions, and only mild mitral regurgitation on left ventriculography. The motion of the interventricular septum and apex was reduced on echocardiography and a persistent perfusion defect was observed at the inferior wall and the interventricular septum on T1-201 scintigraphy. In December 1985, he experienced an Adams-Stokes attack due to complete atrioventricular block. Echocardiographically, the left ventricle became enlarged and the wall motion abnormality and a perfusion defect on T1-201 scintigrams were of relatively severe degree. Thus, left ventricular dilatation and wall motion abnormality may progress in some cases of MVP as it did in this one. We consider this case a very interesting one in speculating on the relationship between MVP and DCM."}, {"id": "wiki20220301en011_153781", "title": "Heart murmur", "score": 0.012366513583799036, "content": "Anatomic sources Systolic Aortic valve stenosis typically is a crescendo/decrescendo systolic murmur best heard at the right upper sternal border sometimes with radiation to the carotid arteries. In mild aortic stenosis, the crescendo-decrescendo is early peaking whereas in severe aortic stenosis, the crescendo is late-peaking, and the S2 heart sound may be obliterated. Stenosis of Bicuspid aortic valve is similar to the aortic valve stenosis heart murmur, but a systolic ejection click may be heard after S1 in calcified bicuspid aortic valves. Symptoms tend to present between 40 and 70 years of age."}, {"id": "wiki20220301en063_19522", "title": "Valvular heart disease", "score": 0.012253907042825248, "content": "Medical signs of aortic regurgitation include increased pulse pressure by increased systolic and decreased diastolic blood pressure, but these findings may not be significant if acute. The patient may have a diastolic decrescendo murmur best heard at left sternal border, water hammer pulse, Austin Flint murmur, and a displaced apex beat down and to the left. A third heart sound may be present Mitral stenosis Patients with mitral stenosis may present with heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea, palpitations, chest pain, hemoptysis, thromboembolism, or ascites and edema (if right-sided heart failure develops). Symptoms of mitral stenosis increase with exercise and pregnancy"}, {"id": "pubmed23n0126_5478", "title": "[Mitral valve prolapse with myocardial disarrangement and familial hypertrophic cardiomyopathy: a case report].", "score": 0.012236271274426194, "content": "This is a report of a patient with mitral valve prolapse (MVP) and myocardial abnormalities on endomyocardial biopsy in whose relatives hypertrophic cardiomyopathy (HCM) was identified. A 19-year-old woman was admitted to our hospital for evaluation of a heart murmur. A systolic ejection murmur was audible in the third intercostal space at the left sternal border, and a standard 12-lead electrocardiogram showed ST-T wave changes in leads II, III and aVF. Echocardiography revealed prolapse of the anterior leaflet of the mitral valve, but no left ventricular hypertrophy. Endomyocardial biopsy disclosed mild hypertrophy and disarrangement of the myocardium. The family study revealed asymmetrical septal hypertrophy in her mother, who had no history of hypertension. Her younger sister had mild hypertrophy of the interventricular septum on echocardiography, and her histopathological findings suggested a diagnosis of HCM. This case was clinically regarded as MVP, but development of left ventricular hypertrophy as noted in her mother may occur in the future."}, {"id": "pubmed23n0248_2494", "title": "[Mitral valve prolapse syndrome].", "score": 0.012106962380685008, "content": "Within one and a half year 24 patients with arrhythmias or chest pain were investigated to detect a mitral valve prolapse syndrome which was found in 9 cases by echocardiography. Within this group 6 patients complained of fatigue, dizziness, dyspnea or syncope, 6 had chest pain, 7 paroxysmal tachycardia and 2 patients premature beats. Auscultation revealed in 3 cases a systolic click, in 1 case a systolic click with late systolic murmur and in 5 cases a systolic murmur only. The ECG showed premature ventricular contractions in 2 patients, ST-T abnormalities in 6 patients. Echocardiography showed a late systolic prolapse in 6 and a pansystolic prolapse in 3 patients. In 3 cases also an angiography was performed and in this way a mitral valve prolapse detected; hemodynamics and coronary arteries were normal in all 3 cases but in one case a mitral insufficiency and in one case an asynergy of the anterior wall was found. Pathophysiology, clinical symptoms and phonocardiographic, echocardiographic and angiographic findings in mitral valve prolapse syndrome are discussed."}, {"id": "pubmed23n0620_22542", "title": "Revelation of an obstructive hypertrophic cardiomyopathy in an elderly patient.", "score": 0.012049941927990708, "content": "Hypertrophic cardiomyopathy (HCM) is classified as a primary cardiomyopathy. HCM is a clinically heterogeneous but relatively common autosomal dominant genetic heart disease that probably is the most frequently occurring cardiomyopathy. HCM is characterized morphologically and defined by a hypertrophied, nondilated left ventriculum (LV) in the absence of another systemic or cardiac disease that is capable of producing the magnitude of wall thickening evident (e.g., systemic hypertension, aortic valve stenosis). Most HCM patients have the propensity to develop dynamic obstruction to LV outflow under resting or physiologically provocable conditions, produced by systolic anterior motion of the mitral valve with ventricular septal contact. The phenotypic features of HCM may develop at any age from infancy to adulthood, and are characterized by a great heterogeneity in the extent, magnitude, and distribution of left ventricular hypertrophy. Hypertrophic obstructive cardiomyopathy (HOCM) often leads to heart failure, severe ischemia, severe symptoms and death. Determination of the exact site of the hypertrophy and of the obstruction of the left ventricular outflow tract, in asymmetric septal hypertrophy, establishes which is the best treatment strategy. In the treatment of HOCM, drug therapy with negatively inotropic drugs, percutaneous transluminal septal myocardial ablation by alcohol-induced septal branch occlusion, surgical myectomy and DDD pacemaker therapy are considered the therapeutical options. We present a case of an obstructive hypertrophic cardiomyopathy in an 84-year-old Italian woman with a left ventricular outflow tract (LVOT) peak gradient with the Valsalva maneuver of 188 mm Hg and with a history of first episode of syncope."}, {"id": "First_Aid_Step2_9", "title": "First_Aid_Step2", "score": 0.011955566712689845, "content": "Systolic murmurs: Aortic stenosis: Harsh systolic ejection murmur; radiation to carotids. Mitral regurgitation: Holosystolic murmur; radiation to axillae or to carotids. Mitral valve prolapse: Midsystolic or late-systolic click. Flow murmur: Very common, and does not imply cardiac disease. Diastolic murmurs: Always abnormal. Aortic regurgitation: Early decrescendo murmur. Mitral stenosis: Midto late, low-pitched murmur. Heart auscultation locations from the upper right sternal border, upper left sternal border, lower left sternal border, and apex: All (Aortic) People (Pulmonic) Try (Tricuspid) McDonald’s (Mitral). Causes of CHF— Hypertension Endocrine Anemia Rheumatic heart disease Toxins Failure to take meds Arrhythmia Infection Lung (pulmonary Gallops: S3 gallop: Dilated cardiomyopathy (floppy ventricle), mitral valve disease; often normal in younger patients and in high-output states (e.g., pregnancy)."}, {"id": "InternalMed_Harrison_17775", "title": "InternalMed_Harrison", "score": 0.011422337014606342, "content": "Figure 271e-12 A 70-year-old patient with known cardiac murmur and progressive shortness of breath and a recent episode of syncope. Echocardiography shows severe calcific aortic stenosis. A heavily calcified aortic valve (arrow) is shown in the parasternal long-axis views (top panels) and short-axis view (bottom left). Doppler interrogation shows a peak transaortic velocity of 5.2 m/s consistent with a peak instantaneous gradient of 109 mmHg and a mean gradient of 66 mmHg, and a corresponding aortic valve area of <0.6 cm2 (lower right). Ao, aorta; LA, left atrium; LV, left ventricle; RV, right ventricle. (See Videos 271e-8, 271e-9, and 271e-10.)"}, {"id": "pubmed23n0070_12638", "title": "[Progression from hypertrophic obstructive cardiomyopathy to dilated cardiomyopathy. Apropos of 4 cases].", "score": 0.011354186656431908, "content": "Four cases of hypertrophic obstructive cardiomyopathy diagnosed on clinical, phonomechanographic, echocardiographic and haemodynamic criteria progressing to dilated cardiomyopathy are reported. This evolution was observed over a number of years (up to 20 years) and was accompanied by a clinical aggravation in all cases with 2 deaths and atrial fibrillation in 3 of the 4 cases. The signs of intraventricular obstruction [systolic murmur, bulge on the carotid pulse tracing, systolic anterior motion of the mitral valve (SAM) and intraventricular pressure gradient] disappeared as the left heart chambers dilated with a reduction in ventricular wall motion and parietal thinning but no change in myocardial mass."}, {"id": "InternalMed_Harrison_17778", "title": "InternalMed_Harrison", "score": 0.010947890606624329, "content": "Figure 271e-15 A 34-year-old woman with known cardiac murmur and syncope with a family history of sudden cardiac death. Echocardiogram shows classic findings of hypertrophic cardiomyopathy, including marked left ventricular wall thickness, particularly in the interventricular septum, notable in the parasternal long-axis view (upper left) and apical view (upper right). Note reverse septal curvature in the apical view (upper left). There is substantial flow acceleration through the left ventricular outflow tract (lower left) with evidence of a late peaking systolic gradient (arrow, lower right) caused by outflow tract obstruction. Ao, aorta; IVS, interventricular septum; LA, left atrium; LV, left ventricle; PW, posterior wall; RV, right ventricle. (See Videos 271e-13, 271e-14, and 271e-15.)"}, {"id": "wiki20220301en243_24570", "title": "Syncope (medicine)", "score": 0.010611470878976782, "content": "Typically, tachycardic-generated syncope is caused by a cessation of beats following a tachycardic episode. This condition, called tachycardia-bradycardia syndrome, is usually caused by sinoatrial node dysfunction or block or atrioventricular block. Obstructive cardiac lesion Blockages in major vessels or within the heart can also impede blood flow to the brain. Aortic stenosis and mitral stenosis are the most common examples. Major valves of the heart become stiffened and reduce the efficiency of the hearts pumping action. This may not cause symptoms at rest but with exertion, the heart is unable to keep up with increased demands leading to syncope. Aortic stenosis presents with repeated episodes of syncope. Rarely, cardiac tumors such as atrial myxomas can also lead to syncope."}, {"id": "pubmed23n0026_5383", "title": "Mitral valve prolapse.", "score": 0.010577188621927644, "content": "Mitral valve prolapse is a condition that is being recognized with increased frequency. It is not known whether its incidence is increasing, or whether we are better able to diagnose it today. In the idiopathic or familial variety, the mitral valve pathology is almost always that of myxomatous degeneration. Some authors have suggested the presence of a cardiomyopathy because of significant left ventricular dysfunction in many cases. Idiopathic prolapse occurs predominantly in females, often at a young age, and may be associated with chest pain, dyspnea, fatigue, presyncope, syncope, and/or sudden death. The clinical findings are variable and typically consist of a nonejection click and/or late systolic murmur, heard best at the cardiac apex. Diagnosis can be confirmed by echocardiography and/or ventricular cineangiography, the latter permitting accurate recognition of the anatomy of the prolapsed leaflets. The complications of infective endocarditis, severe mitral insufficiency, and life-threatening ventricular arrhythmias represent the major problems of management. It is important to distinguish the idiopathic form of mitral valve prolapse from that due to coronary artery disease and to realize that mitral valve prolapse may occur in Marfan's syndrome, Turner's syndrome, or in association with secundum atrial septal defect or ruptured chordae tendineae. Typical clicks and/or murmurs have also been described in patients with a history of rheumatic fever and in hypertrophic cardiomyopathy. Although much descriptive knowledge has accumulated over the past 15 years, many unanswered questions remain regarding the idiopathic type of prolapse. What is the nature and cause(s) of myxomatous degeneration? What is the relation of the valve pathology to the left ventricular dysfunction? What is the relation of both of these factors to disabling chest pain, electrocardiographic changes, and life-threatening arrhythmias? Hopefully, answers to these and other important questions regarding mitral valve prolapse will be forthcoming."}, {"id": "wiki20220301en063_19520", "title": "Valvular heart disease", "score": 0.010243328100470957, "content": "Minor tricuspid insufficiency is common in healthy individuals. In more severe cases it is a consequence of dilation of the right ventricle, leading to displacement of the papillary muscles which control the valve's ability to close. Dilation of the right ventricle occurs secondary to ventricular septal defects, right to left shunting of blood, eisenmenger syndrome, hyperthyroidism, and pulmonary stenosis. Tricuspid insufficiency may also be the result of congenital defects of the tricuspid valve, such as Ebstein's anomaly. Signs and symptoms Aortic stenosis Symptoms of aortic stenosis may include heart failure symptoms, such as dyspnea on exertion (most frequent symptom), orthopnea and paroxysmal nocturnal dyspnea, angina pectoris, and syncope, usually exertional."}, {"id": "wiki20220301en128_37806", "title": "Mitral valve replacement", "score": 0.009997157998562281, "content": "Mitral stenosis and regurgitation The most common cause of mitral stenosis is rheumatic fever, seen mostly in the developing world. Other causes are mitral degenerative disease, severe calcification (elderly), congenital deformities, malignant carcinoid syndrome, neoplasm, left atrial appendage thrombus, endocarditic vegetations, certain inherited metabolic diseases, or complications of previous procedures at the aortic valve. Mitral stenosis causes left atrial pressure to increase, which, if left untreated, can lead to ventricular dilation, hypertrophy, atrial fibrillation, and thrombus creation. Symptoms include shortness of breath (dyspnea) on exertion, when lying flat (orthopnea) or during the night (paroxysmal nocturnal dyspnea), and fatigue."}, {"id": "InternalMed_Harrison_2927", "title": "InternalMed_Harrison", "score": 0.009957528094454266, "content": "DIASTOLIC HeART MuRMuRS early Diastolic Murmurs (Fig. 51e-1E) Chronic AR results in a high-pitched, blowing, decrescendo, early to mid-diastolic murmur that begins after the aortic component of S2 (A2) and is best heard at the second right interspace (Fig. 51e-6). The murmur may be soft and difficult to hear unless auscultation is performed with the patient leaning forward at end expiration. This maneuver brings the aortic root closer to the anterior chest wall. Radiation of the murmur may provide a clue to the cause of the AR. With primary valve disease, such as that due to congenital bicuspid disease, prolapse, or endocarditis, the diastolic murmur tends to radiate along the left sternal border, where it is often louder than appreciated in the second right interspace. When AR is caused by aortic root disease, the diastolic murmur may radiate along the right sternal border. Diseases of the aortic root cause dilation or distortion of the aortic annulus and failure of leaflet"}, {"id": "wiki20220301en100_5617", "title": "List of MeSH codes (C14)", "score": 0.009900990099009901, "content": "– heart failure, congestive – cardiomyopathy, dilated – dyspnea, paroxysmal – edema, cardiac – heart neoplasms – heart rupture – heart rupture, post-infarction – ventricular septal rupture – heart valve diseases – aortic valve insufficiency – aortic valve stenosis – aortic stenosis, supravalvular – williams syndrome – aortic stenosis, subvalvular – cardiomyopathy, hypertrophic – discrete subaortic stenosis – heart murmurs – heart valve prolapse – aortic valve prolapse – mitral valve prolapse – tricuspid valve prolapse – mitral valve insufficiency – mitral valve stenosis – pulmonary atresia – pulmonary valve insufficiency – pulmonary valve stenosis – leopard syndrome – pulmonary subvalvular stenosis – tricuspid atresia – tricuspid valve insufficiency – tricuspid valve stenosis"}, {"id": "wiki20220301en018_68857", "title": "Palpitations", "score": 0.009830915642612953, "content": "Positive orthostatic vital signs may indicate dehydration or an electrolyte abnormality. A mid-systolic click and heart murmur may indicate mitral valve prolapse. A harsh holo-systolic murmur best heard at the left sternal border which increases with Valsalva may indicate hypertrophic obstructive cardiomyopathy. An irregular rhythm indicates atrial fibrillation or atrial flutter. Evidence of cardiomegaly and peripheral edema may indicate heart failure and ischemia or a valvular abnormality. Blood tests, particularly tests of thyroid gland function, are also important baseline investigations (an overactive thyroid gland is a potential cause for palpitations; the treatment, in that case, is to treat the thyroid gland over-activity)."}, {"id": "InternalMed_Harrison_18477", "title": "InternalMed_Harrison", "score": 0.00980392156862745, "content": "Death in patients with severe AS occurs most commonly in the seventh and eighth decades. Based on data obtained at postmortem examination in patients before surgical treatment became widely available, the average time to death after the onset of various symptoms was as follows: angina pectoris, 3 years; syncope, 3 years; dyspnea, 2 years; congestive heart failure, 1.5–2 years. Moreover, in >80% of patients who died with AS, symptoms had existed for <4 years. Among adults dying with valvular AS, sudden death, which presumably resulted from an arrhythmia, occurred in 10–20%; however, most sudden deaths occurred in patients who had previously been symptomatic. Sudden death as the first manifestation of severe AS is very uncommon (<1% per year) in asymptomatic adult patients. Calcific AS is a progressive disease, with an annual reduction in valve area averaging 0.1 cm2 and annual increases in the peak jet velocity and mean valve gradient averaging 0.3 meters/s and 7 mmHg, respectively"}, {"id": "pubmed23n0300_16467", "title": "Does this patient have an abnormal systolic murmur?", "score": 0.009730401177769599, "content": "Our objective was to review the available evidence of the precision and accuracy of the clinical examination for abnormal systolic murmurs. We conducted a MEDLINE search, manually reviewed all reference lists, and contacted authors of published studies. Each study was independently reviewed by 2 observers and graded for methodologic quality. We found that most studies were conducted using cardiologist examiners. In the clinical setting, the reliability of detecting systolic murmurs was fair (kappa, 0.30-0.48). The most useful findings for ruling in aortic stenosis are a slow rate of rise of the carotid pulse (positive likelihood ratio, 2.8-130), mid to late peak intensity of the murmur (positive likelihood ratio, 8.0-101), and decreased intensity of the second heart sound (positive likelihood ratio, 3.1-50). The most useful finding for ruling out aortic stenosis is the absence of murmur radiation to the right carotid artery (negative likelihood ratio, 0.05-0.10). Smaller, lower-quality studies indicate that cardiologists can accurately rule in and rule out mitral regurgitation, tricuspid regurgitation, hypertrophic cardiomyopathy, and echocardiographic mitral valve prolapse. We conclude that the clinical examination by cardiologists is accurate for detecting various causes of abnormal systolic murmurs. Studies of the clinical examination by noncardiologists are needed."}, {"id": "pubmed23n0632_24946", "title": "[Clinical characteristics of cardiac syncope in children].", "score": 0.009715750232991613, "content": "To explore the clinical characteristics of cardiac syncope (CS) in children, and understand their significance in predicting the cardiac syncope. Twenty-three patients were referred to our department for evaluation of syncope. The diagnosis of the above cases was cardiac syncope. Each patient was interviewed using a standard questionnaire. The clinical histories and standard baseline electrocardiogram were analyzed to identify the variables contributing to the diagnosis of CS in children. A cardiac cause was identified in 23 syncopal patients presenting to the Department of Pediatrics, Peking University First Hospital: sick sinus syndrome in 7, congenital long QT syndrome in 4, third degree atrioventricular block in 2, supraventricular tachycardia in 2, ventricular tachycardia in 1, atrial fibrillation in 1, pacemaker dysfunction in 1, idiopathic pulmonary hypertension in 3, hypertrophic cardiomyopathy in 1, and dilated cardiomyopathy in 1. The average age of CS patients was 9 years. In totally 23 patients, exertion related syncope spells were found in 14 cases (60.9%), syncope spells at various position 7/23 (30.4%), absence of prodromes in 12/23 (52.2%), syncope spells with incontinence in 4/23 (17.4%), history of heart disease in 4/23 (17.4%). Abnormal standard baseline electrocardiogram was found in 21 cases (91.7%). The children with cardiac syncope have overt clinical features, especially abnormal findings in electrocardiogram and exertion related syncope spells are the most common clinical features."}, {"id": "wiki20220301en011_153783", "title": "Heart murmur", "score": 0.009708737864077669, "content": "Tricuspid valve regurgitation presents as a holosystolic (pansystolic) murmur at the left lower sternal border with radiation to the left upper sternal border. Prominent v and c waves may be seen in the JVP (jugular venous pressure). The murmur will increase with inspiration. Hypertrophic obstructive cardiomyopathy (or hypertrophic subaortic stenosis) will be a systolic crescendo-decrescendo murmur best heard at the left lower sternal border. Valsalva maneuver will increase the intensity of the murmur, as will changing positions from squatting to standing. Atrial septal defect will present with a systolic crescendo-decrescendo murmur best heard at the left upper sternal border due to increased volume going through the pulmonary valve, and is associated with a fixed, split S2 and a right ventricular heave."}, {"id": "pubmed23n1122_16738", "title": "Aortic Coarctation Associated With Hypertrophic Cardiomyopathy in a Woman With Hypertension and Syncope: A Case Report With 8-Year Follow-Up.", "score": 0.009708737864077669, "content": "Coarctation of the aorta (CoA) is a common congenital cardiovascular malformation with aortic narrowing in the region of the ligamentum arteriosum. Hypertrophic cardiomyopathy (HCM) is a primary cardiomyopathy that is characterized by left ventricular wall thickening and likely left ventricular outflow tract (LVOT) obstruction. They are two irrelevant diseases, and their coexistence has not been reported before. Here, we described a young female patient who concurrently has CoA and HCM. The patient has had hypertension since 18-years old and complained of chest discomfort on effort and fatigue thereafter. Initially, she was diagnosed as having hypertrophic cardiomyopathy and primary hypertension. The presence of CoA was not found until she was 35 years old when she had an onset of paroxysmal supraventricular tachycardia (PSVT) and presented with syncope. Failure of the ablation procedure 600 mg/dL). DKA usually occurs in type 1 diabetics whereas HHS is more common in type 2 diabetics. DKA is characterized by a rapid onset, and HHS occurs gradually over a few days. DKA also is characterized by ketosis due to the breakdown of fat for energy. Both DKA and HHS may show symptoms of dehydration, increased thirst, increased urination, increased hunger, weight loss, nausea, vomiting, abdominal pain, blurred vision, headaches, weakness, and low blood pressure with standing. Management"}, {"id": "wiki20220301en008_125514", "title": "Diabetic coma", "score": 0.014245014245014245, "content": "Nonketotic hyperosmolar coma Nonketotic hyperosmolar coma usually develops more insidiously than diabetic ketoacidosis because the principal symptom is lethargy progressing to obtundation, rather than vomiting and an obvious illness. Extremely high blood sugar levels are accompanied by dehydration due to inadequate fluid intake. Coma occurs most often in patients who have type 2 or steroid diabetes and have an impaired ability to recognize thirst and drink. It is classically a nursing home condition but can occur in all ages. The diagnosis is usually discovered when a chemistry screen performed because of obtundation reveals an extremely high blood sugar level (often above 1800 mg/dl (100 mM)) and dehydration. The treatment consists of insulin and gradual rehydration with intravenous fluids."}, {"id": "wiki20220301en086_255", "title": "Hyperosmolar hyperglycemic state", "score": 0.013898601398601398, "content": "Diagnosis Criteria According to the American Diabetes Association, diagnostic features include: Plasma glucose level >30 mmol/L (>600 mg/dL) Serum osmolality >320 mOsm/kg Profound dehydration, up to an average of 9L (and therefore substantial thirst (polydipsia)) Serum pH >7.30 Bicarbonate >15 mEq/L Small ketonuria (~+ on dipstick) and absent-to-low ketonemia (<3 mmol/L) Some alteration in consciousness BUN > 30 mg/dL (increased) Creatinine > 1.5 mg/dL (increased) Imaging Cranial imaging is not used for diagnosis of this condition. However, if MRI is performed, it may show cortical restricted diffusion with unusual characteristics of reversible T2 hypointensity in the subcortical white matter."}, {"id": "wiki20220301en002_195785", "title": "Diabetic ketoacidosis", "score": 0.01371326042378674, "content": "If cerebral edema is suspected because of confusion, recurrent vomiting or other symptoms, computed tomography may be performed to assess its severity and to exclude other causes such as stroke. Criteria Diabetic ketoacidosis is distinguished from other diabetic emergencies by the presence of large amounts of ketones in blood and urine, and marked metabolic acidosis. Hyperosmolar hyperglycemic state (HHS, sometimes labeled \"hyperosmolar non-ketotic state\" or HONK) is much more common in type 2 diabetes and features increased plasma osmolarity (above 320 mosm/kg) due to profound dehydration and concentration of the blood; mild acidosis and ketonemia may occur in this state, but not to the extent observed in DKA. There is a degree of overlap between DKA and HHS, as in DKA the osmolarity may also be increased."}, {"id": "pubmed23n0622_23937", "title": "[An elderly case of hyperglycemic hyperosmolar syndrome with hyperketonemia].", "score": 0.013322884012539185, "content": "A 72-year-old man had been treated for type 2 diabetes mellitus and gastric cancer. He had been receiving insulin and chemotherapy because of diabetes mellitus and terminal gastric cancer. The dose of insulin was decreased due to the appetite loss, but his general condition deteriorated with disturbed consciousness (JCS I-3), so he was admitted to our hospital in November 2006. On admission, he showed abnormal laboratory data such as WBC 11,070/microl, Hb 10.2 g/dl, serum BUN 64.1 mg/dl, serum Cr 2.23 mg/dl, serum CRP 16.78 mg/dl, plasma glucose 830 mg/dl, serum osmolarity 360 mOsm/l, and serum total keton body 5,490 micromol/l. However, serum Na (142 mEq/l), serum K (4.5 mEq/l), arterial blood pH (7.368), and the anion gap (15 mEq/l) were within the normal range. He was given a diagnosis of hyperglycemic hyperosmolar syndrome with hyperketonemia. Immediately treatment was started with physiologic saline and regular insulin infusion. After treatment, glucose level and serum osmolarity ameliorated. Though elderly cases with hyperglycemic hyperosmolar syndrome and hyperketonemia are rarely reported, it is important to be aware that elderly patients often have very atypical signs and symptoms. We report this case to show diverse nature of elderly patients."}, {"id": "pubmed23n0117_8853", "title": "Clinical features of hyperosmolar hyperglycemic nonketotic diabetic coma associated with cardiac operations.", "score": 0.013283305300383835, "content": "Hyperosmolar hyperglycemic nonketotic diabetic coma after cardiac operations was reviewed in a total of 12 patients from the literature and from my experience in an attempt to determine the clinical features of this condition. Among the unique features of this disease were the following: The mortality is high (42%). The morbidity and mortality are higher in patients with no previous history of diabetes mellitus (67% and 50%) than in those with such a history (33% and 25%). Polyuria is usually a heralding symptom. There is an average time lag of 6 days between the onset of polyuria and the established diagnosis of hyperosmolar hyperglycemic nonketotic diabetic coma. The time lag in patients who died was 7.5 +/- 0.8 days (mean +/- standard error of the mean), significantly longer than in survivors (4.5 +/- 0.8 days). Polyuria usually emerges after the stormy immediate postoperative days have passed (on postoperative day 5.3 on the average). Polyuria is generally regarded as a favorable sign not suggestive of complicating hyperosmolar hyperglycemic nonketotic diabetic coma. Therapies known to precipitate this disorder are continued even after development of polyuria. Gastrointestinal bleeding can be a precipitating factor. Hyperalimentation or elemental diet may cause dehydration and trigger hyperosmolar hyperglycemic nonketotic diabetic coma. A high or rising serum sodium concentration and/or blood urea nitrogen level with polyuria may be a warning sign of this complication. Too hasty correction of the hyperosmolar state can be dangerous. Pulmonary dysfunction may be involved in the symptoms of hyperosmolar hyperglycemic nonketotic diabetic coma."}, {"id": "pubmed23n0936_9329", "title": "Hyperosmolar Hyperglycemic State.", "score": 0.013128205128205127, "content": "Hyperosmolar hyperglycemic state is a life-threatening emergency manifested by marked elevation of blood glucose and hyperosmolarity with little or no ketosis. Although there are multiple precipitating causes, underlying infections are the most common. Other causes include certain medications, nonadherence to therapy, undiagnosed diabetes mellitus, substance abuse, and coexisting disease. In children and adolescents, hyperosmolar hyperglycemic state is often present when type 2 diabetes is diagnosed. Physical findings include profound dehydration and neurologic symptoms ranging from lethargy to coma. Treatment begins with intensive monitoring of the patient and laboratory values, especially glucose, sodium, and potassium levels. Vigorous correction of dehydration is critical, requiring an average of 9 L of 0.9% saline over 48 hours in adults. After urine output is established, potassium replacement should begin. Once dehydration is partially corrected, adults should receive an initial bolus of 0.1 units of intravenous insulin per kg of body weight, followed by a continuous infusion of 0.1 units per kg per hour (or a continuous infusion of 0.14 units per kg per hour without an initial bolus) until the blood glucose level decreases below 300 mg per dL. In children and adolescents, dehydration should be corrected at a rate of no more than 3 mOsm per hour to avoid cerebral edema. Identification and treatment of underlying and precipitating causes are necessary."}, {"id": "pubmed23n0093_14734", "title": "[Central pontine myelinolysis followed by frequent hyperglycemia and hypoglycemia--report of an autopsy case].", "score": 0.013118279569892474, "content": "A case of central pontine myelinolysis (CPM) followed by hyperglycemia and hypoglycemia was reported. The case was 53-year-old female. Diabetes mellitus was found when she was 32 years old, insulin therapy was started at 37 years of age. Since she was 50 years old, proteinuria and ankle edema had developed and she was admitted to The Keihin Hospital. The peritoneal dialysis (PD) was performed next year, followed by the hemodialysis (HD). In January 1978, strange movements and the disturbance of her consciousness were occurred during PD, then blood glucose level showed over 1,800 mg/dl and serum osmolarity was over 390 mosm/KgH2O. Then she was diagnosed as non-ketotic hyperosmolar coma. After that, during HD and PD, hyperglycemia (approximately 1,200 mg/dl) and hypoglycemia (approximately 40 mg/dl) developed frequently. She died soon after HD on 19th December 1979. The autopsy disclosed bilateral atrophic kidneys due to diabetic changes and atrophic pancreas. Gross neuropathological findings revealed a few small infarcts at the putamen and the globus pallidus, however, other area were observed to be normal. The most remarkable change in microscopical finding was nearly symmetrical demyelinative lesion in the center of the basis pontis. The nerve cells and axon cylinders were relatively well preserved in the demyelinative lesion. The hyaline degeneration was observed in the arterial wall, however, any arterial obstruction was not found. Recent studies would suggest that the electrolyte disturbance, such as hyponatremia, may lead to CPM, particularly when this disturbance was rapidly corrected. On the other hand, CPM induced by diabetic coma has been reported, however, its pathogenesis has been unclear.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n1035_10113", "title": "Dapagliflozin-associated euglycemic diabetic ketoacidosis in a patient with type 2 diabetes mellitus: A case report.", "score": 0.013105205678922462, "content": "Rare cases of euglycemic diabetic ketoacidosis (eu-DKA) have been reported after the administration of sodium-glucose cotransporter-2 (SGLT-2) inhibitors. No reports have described eu-DKA complicated by hypernatremia due to SGLT-2 inhibitors. A 76-year-old woman with a 40-year history of type 2 diabetes mellitus (DM), for which metformin (1000 mg/day) and dapagliflozin (10 mg/day) were prescribed, presented with malaise, fever, and oliguria. On presentation, her white blood cell count (11,800/μL), serum creatinine (3.2 mg/dL), and C-reactive protein (54 mg/L) were abnormal. Bilateral pyeloureteritis and diffuse paralytic ileus were present. She received intravenous antibiotics and total parenteral nutrition, and was asked to fast. Her renal function and ileus briefly improved. Oral hypoglycemic agents, metformin and dapagliflozin, along with enteral feeding were reinstituted on day 3 of hospitalization. However, on day 6 of hospitalization, the patient developed an altered state of consciousness including confusion, lethargy, and stupor. Several laboratory abnormalities suggestive of ketoacidosis with euglycemia were noted. The patient was diagnosed with eu-DKA accompanied by severe hypernatremia (corrected serum Na concentration, 163 mEq/L) and hypokalemia following dapagliflozin re-administration. The patient was treated with indicated intravenous fluid therapy. Dapagliflozin use was discontinued. The patient's mental status and laboratory findings improved gradually, and she was discharged on maintenance doses of insulin and metformin on day 14 of hospitalization. Acute illnesses such as diffuse paralytic ileus and urinary tract infection, and dietary restrictions or fasting in patients with DM can be considered potential predisposing factors for SGLT-2 inhibitor-associated eu-DKA. For patients with diabetes in the setting of acute morbidity, timely resumption of the SGLT-2 inhibitor therapy should be carefully determined. In addition, eu-DKA due to SGLT-2 inhibitor use may be accompanied by electrolyte disturbances such as hypernatremia and hypokalemia."}, {"id": "pubmed23n0374_23449", "title": "Hyperosmolar diabetic non-ketotic coma, hyperkalaemia and an unusual near death experience.", "score": 0.013038548752834465, "content": "Generally, cardiac arrest due to pulseless electrical activity has a poor outcome, except when reversible factors such as acute hyperkalaemia are identified and managed early. Hyperosmolar diabetic non-ketotic coma may lead to acute hyperkalaemia. Hyperosmolar diabetic non-ketotic coma is a metabolic emergency usually seen in elderly non-insulin dependent diabetics, characterized by severe hyperglycaemia, volume depletion, altered consciousness, confusion and less frequently neurological deficit. Cerebrovascular accident or transient ischaemic attack may be mistakenly diagnosed, particularly if the patient has no history of diabetes mellitus. Delays in diagnosis and management of glycaemic emergencies presenting as a constellation of neurological abnormalities can be avoided by routine early measurement of blood glucose. Hyperosmolar diabetic non-ketotic coma should be considered in any patient with altered consciousness or neurologic deficit in conjunction with hyperglycaemia. As hyperosmolar diabetic non-ketotic coma results in severe fluid depletion, electrolyte disturbance, profound hyperglycaemia and an altered mental state, the guiding principles of therapy include aggressive rehydration, insulin therapy, correction of electrolyte abnormalities and treatment of any underlying illnesses. Treatment of acute hyperkalaemia includes calcium ions, insulin with dextrose, salbutamol and haemodialysis."}, {"id": "pubmed23n0524_21709", "title": "Hyperglycemic hyperosmolar non-ketotic syndrome in children with type 2 diabetes*.", "score": 0.012817362817362819, "content": "Hyperglycemic hyperosmolar non-ketotic (HHNK) syndrome is thought to be a rare entity in the pediatric population, associated with significant mortality based on case reports in the literature. As obesity and type 2 diabetes in childhood grow in prevalence, such related complications may also increase. This study will serve to provide updated information regarding typical clinical course and sequelae of HHNK syndrome in childhood. Patients diagnosed with type 2 diabetes at Children's Hospital of Philadelphia (CHOP) over a period of 5 yr were screened retrospectively for any laboratory evidence of previous episodes of HHNK syndrome. The standard diagnostic criteria of blood glucose >600 mg/dL and serum osmolality >330 mOsm/L with only mild acidosis (serum bicarbonate >15 mmol/L and small ketonuria 15 mg/dL or less) were utilized. The records of all patients with type 2 diabetes mellitus (DM) diagnosed over a 5-yr period were reviewed (n=190). Seven patients were found to have one episode of HHNK syndrome by diagnostic criteria (five males, mean age at presentation 13.3 yr, age range 10.1--16.9 yr), yielding a frequency of 3.7%. All were African-American. HHNK syndrome was the clinical presentation at diagnosis of new onset diabetes for all seven children. Three of seven children had a previously diagnosed developmental delay. The average Glasgow Coma Scale (GCS) score at presentation was 13 (range 9--15). Mean body mass index (BMI) at presentation was 32.7 kg/m(2) (n=6). Mean serum osmolality was 393 mOsm/L (n=7), and mean blood glucose was 1604 mg/dL (n = 7). The average time until mental status returned to baseline among survivors was 3 d (range 1--7 d). The average number of hospital days for survivors was 10 (range 5--24 d). Four of seven patients had an uncomplicated course. One patient developed multisystem organ failure and died on hospital day 4. The case fatality rate was 14.3% (one of seven). Survivors had no appreciable neurodevelopmental sequelae. This retrospective chart review provides updated information regarding the entity of HHNK syndrome in children. This study supports the need for increased awareness of type 2 diabetes in children so that morbidity and mortality related to HHNK syndrome can be prevented."}, {"id": "pubmed23n0080_6371", "title": "[Hyperglycemic hyperosmolar nonketotic coma].", "score": 0.012800124122415733, "content": "Ten patients with the syndrome of non-ketotic hyperosmolar coma are described. The mean age of the patients was 62.3 +/- 17.12 years. One patient was 16 years old. In 9 cases the patients had type II diabetes, one had type I diabetes. In 7 cases the coma was the first sign of diabetes. The factor predisposing in most cases was infection. In the treatment-acting insulin and hypotonic solutions were given. In 2 cases clinical signs of the DIS syndrome were observed manifesting themselves with local changes, including mental disturbances. Heparin was given with good effect. Three patients (30%) died in hospital. The cause of death was serious disease associated with this coma: pancreatitis and myocarditis, purulent bronchopneumonia, myocardial infarction."}, {"id": "wiki20220301en008_125508", "title": "Diabetic coma", "score": 0.012511740238830001, "content": "Diabetic coma is a life-threatening but reversible form of coma found in people with diabetes mellitus. Three different types of diabetic coma are identified: Severe low blood sugar in a diabetic person Diabetic ketoacidosis (usually type 1) advanced enough to result in unconsciousness from a combination of a severely increased blood sugar level, dehydration and shock, and exhaustion Hyperosmolar nonketotic coma (usually type 2) in which an extremely high blood sugar level and dehydration alone are sufficient to cause unconsciousness."}, {"id": "pubmed23n0975_8119", "title": "Hypertension Despite Dehydration in an Adolescent with Diabetic Ketoacidosis.", "score": 0.012138092290764046, "content": "In general, information on blood pressure changes in diabetic ketoacidosis in paediatric population is very scarce. Our aim was to report a case of severe DKA in an adolescent girl who unexpectedly had hypertension rather than hypotension.A 17-year-old girl presented in our Children's Emergency Unit with complaints of excessive eating for 6 weeks, excessive urination for 2 weeks, fever for 1 week, vomiting for 4 days, difficulty with breathing for one day and unresponsiveness to calls for 3 hours. She had moderated to severe dehydration but no hypotension. Laboratory findings included hyperglycaemia (random blood glucose 20.8 mmo/L; 347 mg/dl), acidosis (serum bicarbonate 5 mmol/L),  ketonuria 2+; glycosuria 2+, and urine  specific gravity of 1.015. At admission, the blood pressure was 100/60 mmHg but progressively rose to 140-180/80-100 mmHg by the third day from admission. A significant hypertension can occur in children and adolescents admitted for severe DKA despite the presence of dehydration. Therefore, the attending physician should be aware of this possibility."}, {"id": "wiki20220301en002_195772", "title": "Diabetic ketoacidosis", "score": 0.011710662525879916, "content": "On physical examination there is usually clinical evidence of dehydration, such as a dry mouth and decreased skin turgor. If the dehydration is profound enough to cause a decrease in the circulating blood volume, a rapid heart rate and low blood pressure may be observed. Often, a \"ketotic\" odor is present, which is often described as \"fruity\" or like \"pear drops\". The smell is due to the presence of acetone. If Kussmaul respiration is present, this is reflected in an increased respiratory rate. Small children with DKA are relatively prone to brain swelling, also called cerebral edema, which may cause headache, coma, loss of the pupillary light reflex, and can progress to death. It occurs in about 1 out of 100 children with DKA and more rarely occurs in adults."}, {"id": "pubmed23n0518_8809", "title": "Hyperosmolar hyperglycemic state.", "score": 0.011402107952730757, "content": "Hyperosmolar hyperglycemic state is a life-threatening emergency manifested by marked elevation of blood glucose, hyperosmolarity, and little or no ketosis. With the dramatic increase in the prevalence of type 2 diabetes and the aging population, this condition may be encountered more frequently by family physicians in the future. Although the precipitating causes are numerous, underlying infections are the most common. Other causes include certain medications, non-compliance, undiagnosed diabetes, substance abuse, and coexisting disease. Physical findings of hyperosmolar hyperglycemic state include those associated with profound dehydration and various neurologic symptoms such as coma. The first step of treatment involves careful monitoring of the patient and laboratory values. Vigorous correction of dehydration with the use of normal saline is critical, requiring an average of 9 L in 48 hours. After urine output has been established, potassium replacement should begin. Once fluid replacement has been initiated, insulin should be given as an initial bolus of 0.15 U per kg intravenously, followed by a drip of 0.1 U per kg per hour until the blood glucose level falls to between 250 and 300 mg per dL. Identification and treatment of the underlying and precipitating causes are necessary. It is important to monitor the patient for complications such as vascular occlusions (e.g., mesenteric artery occlusion, myocardial infarction, low-flow syndrome, and disseminated intravascular coagulopathy) and rhabdomyolysis. Finally, physicians should focus on preventing future episodes using patient education and instruction in self-monitoring."}, {"id": "InternalMed_Harrison_27965", "title": "InternalMed_Harrison", "score": 0.011389759665621734, "content": "HYPERGLYCEMIC HYPEROSMOLAR STATE Clinical Features The prototypical patient with HHS is an elderly individual with type 2 DM, with a several-week history of polyuria, weight loss, and diminished oral intake that culminates in mental confusion, lethargy, or coma. The physical examination reflects profound dehydration and hyperosmolality and reveals hypotension, tachycardia, and altered mental status. Notably absent are symptoms of nausea, vomiting, and abdominal pain and the Kussmaul respirations characteristic of DKA. HHS is often precipitated by a serious, concurrent illness such as myocardial infarction or stroke. Sepsis, pneumonia, and other serious infections are frequent precipitants and should be sought. In addition, a debilitating condition (prior stroke or dementia) or social situation that compromises water intake usually contributes to the development of the disorder."}, {"id": "pubmed23n0783_2102", "title": "How to prevent and treat pharmacological hypoglycemias.", "score": 0.010902145166029973, "content": "A 58 year-old woman with type 2 diabetes diagnosed 3 years before came to our clinic. Her treatment was metformin 850 mg every 12 hours and glimepiride 4 mg every 24 hours. After the initiation of glimepiride 9 months before her weight has increased 5 kg, and she suffers frequent hypoglycemias which have affected her while driving. Her BMI is 35.5 kg/m². She has a normal eye fund exam. She has hypertension treated with telmisartán and hidroclorotiazide with adequate control, and also hypercholesterolemia treated with atorvastatine 40 mg every 24 hours. Her blood test shows an HbA1c of 7.0%, normal values of microalbuminuria, total cholesterol 149 mg/dl, HDL cholesterol 52 mg/dl, LDL cholesterol 98 mg/dl and triglycerides 123 mg/dl. Her blood pressure is 129/81 mmHg, there was no orthostatic hypotension, and her peripheral neurological examination shows normal results. In summary, our case is a young woman with type 2 diabetes and obesity, without chronic complications and which has frequent hypoglycaemia. How must this woman be evaluated and treated?"}, {"id": "pubmed23n1061_25282", "title": "Central pontine myelinolysis during treatment of hyperglycemic hyperosmolar syndrome: a case report.", "score": 0.010528940733146768, "content": "Central pontine myelinolysis (CPM) is a non-inflammatory demyelinating lesion of the pons. CPM and extrapontine demyelination (EPM) are together termed osmotic demyelination syndrome (ODS), a known and serious complication of acute correction of hyponatremia. Conversely, hyperglycemic hyperosmolarity syndrome (HHS) develops in patients with type 2 diabetes who still have some insulin secretory ability due to infection, non-compliance with treatment, drugs, and coexisting diseases, and is often accompanied by ketosis. HHS represents a life-threatening endocrine emergency (mortality rate, 10-50%) associated with marked hyperglycemia and severe dehydration. HHS may develop ODS, and some cases have been associated with hypernatremia. The patient was an 87-year-old woman with hyperglycemia, dehydration, malnutrition, and potential thrombus formation during long-term bed rest. HHS was suspected to have developed due to progression of hyperglycemia and dehydration caused by pneumonia. Furthermore, ketoacidosis developed from ketosis and prerenal renal failure associated with circulating hypovolemia shock, which was also associated with disseminated intravascular coagulation. Treatment was started with continuous intravenous injection of fast-acting insulin and low-sodium replacement fluid. In addition, ceftriaxone sodium hydrate, heparin sodium, thrombomodulin α, human serum albumin, and dopamine hydrochloride were administered. Blood glucose, serum sodium, serum osmolality, and general condition (including vital, infection/inflammatory findings, and disseminated intravascular coagulation) improved promptly, but improvements in disturbance of consciousness were poor. Diffusion-weighted imaging of the brain 72 h after starting treatment showed no obvious abnormalities, but high-intensity signals in the midline of the pons became apparent 30 days later, leading to definitive diagnosis of CPM. Fluctuation of osmotic pressure by treatment from hyperosmolarity due to hyperglycemia and hypernatremia in the presence of risk factors such as malnutrition, severe illness, and metabolic disorders may be a cause of CPM onset. When treating HHS with risk factors, the possibility of progression to ODS needs to be kept in mind."}, {"id": "wiki20220301en082_40294", "title": "List of ICD-9 codes 240–279: endocrine, nutritional and metabolic diseases, and immunity disorders", "score": 0.010197820797630382, "content": "Diseases of other endocrine glands (249–259) Note: for 249–259, the following fifth digit can be added: (250.x0) Diabetes mellitus type 2 (250.x1) Diabetes mellitus type 1 (250.x2) Diabetes mellitus type 2, uncontrolled (250.x3) Diabetes mellitus type 1, uncontrolled Secondary diabetes mellitus Secondary diabetes mellitus without mention of complication Secondary diabetes mellitus with ketoacidosis Secondary diabetes mellitus with hyperosmolarity Secondary diabetes mellitus with other coma Secondary diabetes mellitus with renal manifestations Secondary diabetes mellitus with ophthalmic manifestations Secondary diabetes mellitus with neurological manifestations Secondary diabetes mellitus with peripheral circulatory disorders Secondary diabetes mellitus with other specified manifestations Secondary diabetes mellitus with unspecified complications Diabetes mellitus Diabetes mellitus without mention of complication Diabetes with ketoacidosis"}, {"id": "wiki20220301en008_125513", "title": "Diabetic coma", "score": 0.010147159047883485, "content": "In the early to middle stages of ketoacidosis, patients are typically flushed and breathing rapidly and deeply, but visible dehydration, pale appearance from diminished perfusion, shallower breathing, and a fast heart rate are often present when coma is reached. However these features are variable and not always as described. If the patient is known to have diabetes, the diagnosis of diabetic ketoacidosis is usually suspected from the appearance and a history of 1–2 days of vomiting. The diagnosis is confirmed when the usual blood chemistries in the emergency department reveal a high blood sugar level and severe metabolic acidosis. Treatment of diabetic ketoacidosis consists of isotonic fluids to rapidly stabilize the circulation, continued intravenous saline with potassium and other electrolytes to replace deficits, insulin to reverse the ketoacidosis, and careful monitoring for complications."}, {"id": "wiki20220301en292_9714", "title": "Complications of diabetes", "score": 0.010069272637308262, "content": "Nonketotic hyperosmolar coma (HNS) is an acute complication sharing many symptoms with DKA, but an entirely different origin and different treatment. A person with very high (usually considered to be above 300 mg/dl (16 mmol/L)) blood glucose levels, water is osmotically drawn out of cells into the blood and the kidneys eventually begin to dump glucose into the urine. This results in loss of water and an increase in blood osmolarity. If fluid is not replaced (by mouth or intravenously), the osmotic effect of high glucose levels, combined with the loss of water, will eventually lead to dehydration. The body's cells become progressively dehydrated as water is taken from them and excreted. Electrolyte imbalances are also common and are always dangerous. As with DKA, urgent medical treatment is necessary, commonly beginning with fluid volume replacement. Lethargy may ultimately progress to a coma, though this is more common in type 2 diabetes than type 1. Hypoglycemia"}, {"id": "Pathology_Robbins_4628", "title": "Pathology_Robbins", "score": 0.00992063492063492, "content": "Type 2 diabetes also may manifest with polyuria and polydipsia. In some cases, medical attention is sought because of unexplained weakness or weight loss. Most frequently, however, the diagnosis is made after routine blood or urine testing in asymptomatic individuals. In the decompensated state, patients with type 2 diabetes may develop hyperosmolar nonketotic coma. This syndrome is engendered by severe dehydration resulting from sustained osmotic diuresis and urinary fluid loss due to chronic hyperglycemia. Typically, the affected individual is an older adult diabetic who is disabled by a stroke or an infection and is unable to maintain adequate water intake. The absence of ketoacidosis and its symptoms (nausea, vomiting, respiratory difficulties) delays recognition of the seriousness of the situation until the onset of severe dehydration and coma."}]}}}} {"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 105]], "word_ranges": [[0, 15]], "text": "The treatment of choice for Clostridioides difficile infection is vancomycin orally 125mg/6h for 10 days."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The treatment of choice for Clostridioides difficile infection is vancomycin orally 125mg/6h for 10 days. Metronidazole has been relegated to situations where vancomycin is not available.", "full_answer_no_ref": "The treatment of choice for Clostridioides difficile infection is vancomycin orally 125mg/6h for 10 days. Metronidazole has been relegated to situations where vancomycin is not available.", "full_question": "Patient diagnosed with acute diverticulitis, treated with amoxicillin/clavulanic acid. After 5 days he starts with fever and diarrhea. He was diagnosed with Clostridium difficile colitis, amoxicillin/clavulanic acid was discontinued and metronidazole was prescribed. After 4 days he has not responded to metronidazole, but is clinically stable. The next step of treatment is:", "id": 512, "lang": "en", "options": {"1": "Change metronidazole for oral vancomycin (125 mg, four times a day for 10 days).", "2": "Change metronidazole to piperacillin/ tazobactam (4 g piperacillin/0.5 g tazobactam every 8 hours).", "3": "Indicate a subtotal colectomy.", "4": "Loop ileostomy with antegrade polyethylene glycol lavage.", "5": NaN}, "question_id_specific": 153, "type": "DIGESTIVE", "year": 2021, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0726_1644", "title": "Acute fulminant pseudomembranous colitis which developed after ileostomy closure and required emergent total colectomy: a case report.", "score": 0.018653479090751345, "content": "Pseudomembranous colitis is known to be caused by Clostridium difficile; and, in 3% to 8% of patients, it lapses into an aggressive clinical course that is described as fulminant. We present here a case of extremely rapid and fatal fulminant pseudomembranous colitis that developed after ileostomy closure, a minor surgical procedure. To the best of our knowledge, this is the first case report of fatal fulminant pseudomembranous colitis after closure of a diversion ileostomy in an adult. A 69-year-old Japanese man, who had previously undergone low anterior resection and creation of a diverting ileostomy for stage III rectal carcinoma was admitted for ileostomy closure. Preoperatively, he received oral kanamycin and metronidazole along with parenteral cefmetazole. His surgery and postoperative course were uneventful until the third postoperative day, when fever and watery diarrhea became apparent. The next day he presented with epigastric and left lower abdominal pain. Computed tomography revealed a slightly distended colon. Later that night, his blood pressure fell and intravenous infusion was started. In the early morning of the fifth postoperative day, his blood pressure could be maintained only with a vasopressor. Follow-up computed tomography demonstrated severe colonic dilation. A colonoscopy confirmed the presence of pseudomembranous colitis, and so oral vancomycin was administered immediately. However, within three hours of the administration, his condition rapidly deteriorated into shock. Although an emergent total colectomy with creation of an end ileostomy was performed, our patient died 26 hours after the surgery. The histopathological examination was consistent with pseudomembranous colitis. It is important to recognize that, although rare, there is a type of extremely aggressive pseudomembranous colitis in which the usual waiting period for medical treatment might be lethal. We consider that colonoscopy and computed tomography are helpful to decide the necessity of emergent surgical treatment without delay."}, {"id": "pubmed23n0408_6712", "title": "[Clostridium difficile infection in a Department of Internal Medicine. A consecutive series of 45 patients].", "score": 0.016771929824561403, "content": "A retrospective study of 45 patients with Clostridium difficile infection over a 4-year period in a department of Internal Medicine. Mean age was 79 years; sex-ratio (F/M)=1.5; 38% of the patients had neurological or severe psychiatric disorders; 20% had a neoplastic disease. Ninety-three percent of cases had received one or more antibiotics before onset of diarrhea, prescribed mainly for a pulmonary infection. Amoxicillin clavulanic acid and cephalosporins were the most frequently used treatments, respectively in 48% and 40% of cases. For 25 patients (56%) Clostridium difficile-associated diarrhea was considered as a nosocomial infection, and as community-acquired diarrhea in 20 cases (44%). Treatment included isolation of the patient as soon as bacteriological diagnosis was known and specific therapy was instituted by metronidazole or vancomycin for a mean of 18 days. The addition of Saccharomyces boulardii was used in of cases. The clinical course was rapidly favorable for 80% of patients. Five patients died with complications of severe colitis in 2 cases. Mean hospital stay was 49 days (annual mean of the department=10 days). Clostridium difficile diarrhea concerns above all elderly patients with one or more underlying pathologies. Amoxicillin clavulanic acid and third-generation cephalosporins are the most frequently prescribed antibiotics in these cases and have the highest correlation with this infectious complication. This medical problem requires greater knowledge as it causes significant morbidity and increases the risk of prolonged hospital stays."}, {"id": "pubmed23n1072_7767", "title": "Determining the optimal surgical timing of fulminant Clostridium difficile colitis by using four objective factors and computed tomography findings: A case report.", "score": 0.014867237217467633, "content": "Clostridium difficile colitis is increasingly seen in everyday clinical situations, and most cases are treated with antibiotics. Fulminant C. difficile colitis (FCDC) is rare; however, it is extremely virulent, and understanding its appropriate surgical treatment is critical. The surgical timing is controversial because of the lack of concrete decision-making factors. We report a case of FCDC with a favourable outcome, which was achieved by using four objective factors and computed tomography (CT) findings. A patient with head trauma developed pneumonia at 2 days post-admission. He was prescribed with antibiotics. Fever and leucocytosis persisted on hospital day 10. Clostridium was detected in the stool on day 12, and metronidazole was administered. His condition did not improve; thus, he was started on vancomycin on day 14. The marked deterioration in the four laboratory parameters (white blood cell, albumin [Alb], creatinine, and body temperature) on day 15 and CT findings contributed to the decision to perform emergency subtotal colectomy and ileostomy. His condition improved dramatically postoperatively. Many factors of FCDC are already suggested for surgical intervention in the guidelines; however, they are often seen at the late stage of FCDC. Early detection of FCDC is the key to favourable surgical outcome. Following the trend of these objective factors guides in making appropriate surgical decisions. Focusing on the four objective factors and CT findings of FCDC could help surgeons detect FCDC at an early stage and decide the optimal surgical timing."}, {"id": "pubmed23n0326_1826", "title": "[Probiotic therapy of pseudomembranous colitis. Combination of intestinal lavage and oral administration of Escherichia coli].", "score": 0.014595825779761075, "content": "An 82-year-old woman was admitted because of acute left heart failure with pulmonary oedema. There was a right basal pneumonia with congestion which was treated with amoxycillin and clavulanic acid. Severe watery diarrhoea with more than 10 stools daily occurred. Clostridium difficile was not isolated. Coloscopy as far as the right flexure revealed proximally increasing whitish coatings, like those in severe pseudomembranous colitis (PMC), revealed as acute erosive colitis histologically. The plate-like incomplete erosions contained fibrin and granulocyte deposits so typical of PMC. Clostridium difficile could again not be demonstrated. As intestinal lavage, already undertaken in preparation of the coloscopy, markedly improved the symptoms it was repeated for therapeutic purposes. This brought about further improvement and a fall in granulocyte count from 29,500 G/l to 10,700 G/l. Subsequently live E. coli bacteria (nissle 1917 strain) were administered. After 5 days stool frequency had fallen to one or two daily, each soft or partly formed. Stools were normal after a further week. Coloscopy 3 weeks after onset of treatment showed almost complete healing of the PMC. The successful treatment of PMC with intestinal lavage and physiological E. coli administration agrees with the results of animal experiments and clinical experience. Whether it is an effective alternative to primary standard treatment with vancomycin or metronidazole remains to be tested by further experience, preferably properly controlled therapeutic trials."}, {"id": "article-17152_11", "title": "Acute Diverticulitis -- Treatment / Management", "score": 0.012271307452030342, "content": "Upon clinical presentation, acute diverticulitis can be managed with either outpatient or inpatient care. According to American Society of Colon and Rectal Surgeons, a patient who cannot tolerate oral intake, is excessively vomiting, shows signs of peritonitis, is immunocompromised, or at an advanced age should be hospitalized. In the absence of these conditions, and if appropriate prompt follow-up can be established, acute diverticulitis can be managed on an outpatient basis. It is reported that success rate of outpatient management is about 94% to 97%. The standard of outpatient care includes bowel rest, increase fluid intake, and oral antibiotic therapy (single or multiple drug regimen) that covers gram-negative rods and anaerobic bacteria. The most common regimen used in the United States consists of quinolones (ciprofloxacin) or sulfa drugs (trimethoprim/sulfamethoxazole) in combination with metronidazole  (or clindamycin, if the patient is intolerant to metronidazole) or single agent amoxicillin-clavulanate for 7 to 10 days. [8] [3] [9]"}, {"id": "article-19724_15", "title": "Colon Diverticulitis -- Treatment / Management", "score": 0.011258474785965259, "content": "The medical management of acute non-complicated diverticulitis includes oral or intravenous antibiotics, pain control, hydration, and restricted oral intake. Medical management of diverticulitis with antibiotics remains the standard of care. However, antibiotic choice, duration of treatment, and route of administration remain controversial. [18] Most cases of uncomplicated diverticulitis are successfully treatable in the outpatient setting. Oral antibiotics, such as ciprofloxacin and metronidazole or amoxicillin-clavulanate, are prescribed for 7 to 10 days with recommendations to minimize oral intake until the pain has resolved. [15]"}, {"id": "pubmed23n0833_3293", "title": "Antibiotic Regimen after a Total Abdominal Colectomy with Ileostomy for Fulminant Clostridium difficile Colitis: A Multi-Institutional Study.", "score": 0.011211607311098548, "content": "Fulminant Clostridium difficile colitis (fCDC) is a highly lethal disease with mortality rates ranging between 12% and 80%. Although often these patients require a total abdominal colectomy (TAC) with ileostomy, there is no established management protocol for post-operative antibiotics. In this study we aim to make some recommendations for post-operative antibiotic usage, while describing the practice across different institutions. Multi-institutional retrospective case series including fCDC patients who underwent a TAC between January 1, 2007, and June 30, 2012. We first analyzed the complete cohort and consecutively performed a survivor analysis, comparing different antibiotic regimens. Additionally we stratified by time interval (antibiotics for ≤7 d, or ≥8 d). Primary outcome was in-hospital mortality. Additional secondary outcomes included hospital length of stay (HLOS), ICU LOS, number of ventilator-free days, and occurrence of intra-abdominal complications (proctitis, abscess, sepsis, etc.). A total of 100 fCDC patients that underwent a TAC were included across five institutions. Four different antibiotic regimens were compared; A (metronidazole IV+vancomycin PO), B (metronidazole IV), C (metronidazole IV+vanco PO and PR), and D (metronidazole IV+vancomycin PR). The combination of IV metronidazole with or without PO vancomycin showed superior outcomes in terms of a shorter ICU length of stay and more ventilator-free days. However, when comparing metronidazole alone vs. metronidazole and any combination of vancomycin, no significant differences were found. Neither the addition of vancomycin enema, nor the time interval changed outcomes. Patients, after a TAC for fCDC, may be placed on either IV metronidazole or PO vancomycin depending upon local antibiograms, and proctitis may be treated with the addition of a vancomycin enema (PR). There was no data to support routine treatment of more than 7 d."}, {"id": "pubmed23n0291_22982", "title": "[Antibiotic-associated pseudomembranous colitis: retrospective study of 48 cases diagnosed by colonoscopy].", "score": 0.011134969325153374, "content": "Pseudomembranous colitis (PMC) is a rare but potentially severe complication of antibiotic treatment, which is characterized by the proliferation of the bacterium Clostridium difficile in the colon. In this retrospective study, 48 cases of endoscopically confirmed PMC were included. The following variables were analysed: characteristics of the patients, antibiotics, clinical, biological and endoscopic features of PMC and its treatment. The antibiotic treatment was often ambulatory (83 per cent) for a broncho-pulmonary infection (42 per cent). In 90 per cent of the cases, the treatment included a -lactam, frequently amoxicillin with clavulanic acid, and in 25 per cent of the cases, a fluoroquinolone. The PMC generally occurred after more than 4 days of treatment and was associated with diarrhoea, abdominal pain, fever and rarely vomiting (23 per cent). The complications were hypokalaemia (37 per cent), renal failure (27 per cent) and/or hypoproteinaemia (50 per cent). Pseumembranes were found between the rectum and the left angle of the colon. All patients recovered after one week of oral treatment with metronidazole and/or vancomycin, often in association with Saccharomyces boulardii."}, {"id": "wiki20220301en166_44924", "title": "WHO Model List of Essential Medicines", "score": 0.009900990099009901, "content": "Antischistosomals and other antinematode medicines Praziquantel Triclabendazole Oxamniquineα Cysticidal medicines Albendazoleα Mebendazoleα Praziquantelα Antibacterials Access group antibiotics Amikacin Amoxicillin Amoxicillin/clavulanic acid (amoxicillin + clavulanic acid) Ampicillin Benzathine benzylpenicillin Benzylpenicillin Cefalexin Cefazolin Chloramphenicol Clindamycin Cloxacillin Doxycycline Gentamicin Metronidazole Nitrofurantoin Phenoxymethylpenicillin (penicillin V) Procaine benzylpenicillin Spectinomycin Sulfamethoxazole/trimethoprim (sulfamethoxazole + trimethoprim) Trimethoprim Watch group antibiotics Azithromycin Cefixime Cefotaxime Ceftriaxone Cefuroxime Ciprofloxacin Clarithromycin Piperacillin/tazobactam (piperacillin + tazobactam) Vancomycin Ceftazidimeα Meropenemα Vancomycinα"}, {"id": "wiki20220301en521_16277", "title": "WHO Model List of Essential Medicines for Children", "score": 0.00980392156862745, "content": "Antifilarials Albendazole Diethylcarbamazine Ivermectin Antischistosomals and other antinematode medicines Praziquantel Triclabendazole Oxamniquine Antibiotics Access group antibiotics Amikacin Amoxicillin Amoxicillin/clavulanic acid (amoxicillin + clavulanic acid) Ampicillin Benzathine benzylpenicillin Benzylpenicillin Cefalexin Cefazolin Chloramphenicol Clindamycin Cloxacillin Doxycycline Gentamicin Metronidazole Nitrofurantoin Phenoxymethylpenicillin (penicillin V) Procaine benzylpenicillin Sulfamethoxazole/trimethoprim (sulfamethoxazole + trimethoprim) Watch group antibiotics Azithromycin Cefixime Cefotaxime Ceftriaxone Cefuroxime Ciprofloxacin Clarithromycin Piperacillin/tazobactam (piperacillin + tazobactam) Vancomycin Ceftazidime Meropenem Vancomycin Reserve group antibiotics Ceftazidime/avibactam (ceftazidime + avibactam) Colistin Fosfomycin Linezolid Polymyxin B Antileprosy medicines Clofazimine Dapsone Rifampicin"}, {"id": "wiki20220301en014_2042", "title": "Cholecystitis", "score": 0.009750923073784704, "content": "Other Supportive measures may be instituted prior to surgery. These measures include fluid resuscitation. Intravenous opioids can be used for pain control. Antibiotics are often not needed. If used they should target enteric organisms (e.g. Enterobacteriaceae), such as E. coli and Bacteroides. This may consist of a broad spectrum antibiotic; such as piperacillin-tazobactam, ampicillin-sulbactam, ticarcillin-clavulanate (Timentin), a third generation cephalosporin (e.g.ceftriaxone) or a quinolone antibiotic (such as ciprofloxacin) and anaerobic bacteria coverage, such as metronidazole. For penicillin allergic people, aztreonam or a quinolone with metronidazole may be used."}, {"id": "wiki20220301en613_24692", "title": "Ranitidine bismuth citrate", "score": 0.009708737864077669, "content": "Inside, regardless of food intake, 2 times a day (morning and evening). In the treatment and prevention of benign gastric and duodenal ulcers, as well as for the eradication of Helicobacter pylori and the relief of concomitant symptoms of dyspepsia, the following combination therapy regimens are used. Within 7 days - 3 drugs: Scheme 1. 2 times a day - ranitidine bismuth citrate 400 mg, clarithromycin 500 mg, metronidazole 400 mg or amoxicillin 1 g. Scheme 2. 2 times a day - ranitidine bismuth citrate 400 mg, clarithromycin 250 mg, metronidazole 400 or 500 mg or tinidazole 500 mg. Scheme 3. Ranitidine bismuth citrate 400 mg 2 times a day, metronidazole 500 mg 3 times a day, tetracycline 500 mg 4 times a day. Scheme 4. 2 times a day - ranitidine bismuth citrate 400 mg, tinidazole 500 mg, amoxicillin 1 g. Within 14 days - 2 drugs: Scheme 5. Ranitidine bismuth citrate 400 mg 2 times a day and clarithromycin 500 mg 2 or 3 times a day. Scheme 6. Ranitidine bismuth citrate 400 mg 2 times a"}, {"id": "pubmed23n0876_2333", "title": "Outpatient treatment in uncomplicated acute diverticulitis: 5-year experience.", "score": 0.009708737864077669, "content": "Most cases of diverticular inflammation are mild and require only medical treatment with liquid diet and antibiotics. Until recently, this treatment required admission to hospitals, which consequently entailed costs. In most cases, treatment was conservative, and less than a quarter of patients admitted actually underwent surgery. In the last year, the outpatient treatment of these patients with uncomplicated diverticulitis has proven effective and safe. The aim of the present study was to describe our experience after 5 years of outpatient treatment with oral antibiotics. We conducted a retrospective revision study between January 2010 and December 2014. We included all patients admitted to the Emergency Department of the University General Hospital of Elche with a diagnosis of uncomplicated acute diverticulitis based on medical history, physical examination and abdominopelvic computed tomography (CT) scanning. Outpatient treatment consisted of oral antibiotics for 10 days (metronidazole 500 mg/8 h and ciprofloxacin 500 mg/12 h), a liquid diet and oral analgesics (acetaminophen 1 g/6 h). During the period from January 2010 to December 2014, 224 patients were treated on an outpatient basis at a success rate of over 92%. Only 18 patients (8%) required admission after outpatient treatment. Outpatient treatment of uncomplicated acute diverticulitis was demonstrated to be safe and effective."}, {"id": "wiki20220301en236_2738", "title": "Tolevamer", "score": 0.009630419331911869, "content": "History Termination of development In early 2008, a noninferiority study versus vancomycin or metronidazole for Clostridium difficile associated diarrhea (CDAD) found that about half of the patients in the tolevamer group did not complete the treatment, versus 25% in the vancomycin and 29% in the metronidazole groups. CDAD recurrence in patients reaching clinical success was reduced significantly by tolevamer (6% recurrence rate), vancomycin (18%) and metronidazole (19%). However, the good result of tolevamer is partly due to the high drop-out rate in this group. Since tolevamer did not reach its primary endpoint in this study, its development was halted. References Antidiarrhoeals Sulfonic acids"}, {"id": "pubmed23n0349_15849", "title": "Piperacillin/tazobactam compared with cefuroxime/ metronidazole in the treatment of intra-abdominal infections.", "score": 0.009615384615384616, "content": "To assess the effect of piperacillin/tazobactam compared with cefuroxime/metronidazole in the treatment of patients with intra-abdominal infections. Randomised open study. 16 Swedish and 6 Norwegian hospitals. 269 patients with intra-abdominal infections were randomised and treated with at least one dose of each study drug. 205 patients, 105 treated with piperacillin/tazobactam and 100 with cefuroxime, were clinically evaluable for follow up (had been given the full course of treatment). Patients were given piperacillin 4g/tazobactam 0.5 g every 8 hours or cefuroxime 1.5 g every 8 hours plus metronidazole 1.5 g every 24 hours. Each patient was to be treated for a minimum of 3 days and not more than 10 days. Clinical evaluation of infection at the end of and 4-6 weeks after treatment. Evaluation of safety and tolerance to the drugs and bacteriological susceptibility to the treatment drugs. In the intention to treat analysis treatment was equally successful for piperacillin/ tazobactam (103/140, 74%) and the cefuroxime/metronidazole groups (90/129, 70%) (p = 0.6). Corresponding figures for the clinically evaluable group were 102/105 (97%) and 94/100 (94%) for piperacillin/tazobactam and cefuroxime/metronidazole groups, respectively, at the end of treatment. At late follow up, 92/105 (88%) and 83/100 (83%) in the two groups, respectively, remained free of infection. The side effects of the treatment were mild and evenly distributed between the two groups. Most pathogens were susceptible to the drugs in both treatment groups. Both piperacillin/tazobactam and cefuroxime/metronidazole are well suited to the treatment of patients with intra-abdominal infections, and we found no significant difference between the two. The drugs were safe and well tolerated in the regimens used."}, {"id": "pubmed23n0339_3359", "title": "Management of uncomplicated acute diverticulitis: results of a survey.", "score": 0.009615384615384616, "content": "A survey was conducted to document current medical treatment of patients with uncomplicated acute diverticulitis. A survey was mailed to 667 fellows of The American Society of Colon and Rectal Surgeons certified by the American Board of Colon and Rectal Surgery. Queries were based on a clinical scenario of a patient with uncomplicated diverticulitis. Three hundred seventy-three surveys (56 percent) were returned completed. The majority (66 percent) chose an abdominal computed tomographic scan as the initial diagnostic test. One-half used a single intravenous antibiotic with second-generation cephalosporins (27 percent) and ampicillin/sulbactam (16 percent) being the most common. Oral antibiotics given at discharge were ciprofloxacin (18 percent), amoxicillin/clavulanate (14 percent), metronidazole (7 percent), and doxycycline (6 percent). Combinations chosen were ciprofloxacin/metronidazole (28 percent) and metronidazole/trimethoprim sulfamethoxazole (6 percent), whereas 21 percent chose a variety of other antibiotics. The majority (74 percent) prescribed oral antibiotics for 7 to 10 days. Dietary recommendations at discharge were low residue (68 percent), regular (21 percent), and high residue (10 percent). Half of those surveyed believed avoidance of seeds and nuts were of no value. Follow-up examinations chosen included sigmoidoscopy and barium enema (29 percent), colonoscopy (25 percent), sigmoidoscopy (17 percent), barium enema (13 percent), and other (16 percent). Sixty-five percent of colon and rectal surgeons claim to handle more than half of their patients with uncomplicated diverticulitis on an outpatient basis. Variations in the management of uncomplicated sigmoid diverticulitis are noted among colon and rectal surgeons, especially in terms of antibiotic choice, discharge instructions, and follow-up outpatient studies. The survey results are compared with the conclusions reached in The American Society of Colon and Rectal Surgeons practice parameters. Documentation of practice pattern variation may serve as an educational tool for physicians to improve their quality and cost of medical care. Consideration should be given to better publicize already existing American Society of Colon and Rectal Surgeons practice parameters for this common entity."}, {"id": "wiki20220301en337_7070", "title": "Anaerobic infection", "score": 0.009523809523809525, "content": "The available parenteral antimicrobials for most infections are metronidazole, clindamycin, chloramphenicol, cefoxitin, a penicillin (i.e. ticarcillin, ampicillin, piperacillin) and a beta-lactamase inhibitor (i.e. clavulanic acid, sulbactam, tazobactam), and a carbapenem (imipenem, meropenem, doripenem, ertapenem). An antimicrobial effective against Gram-negative enteric bacilli (i.e. aminoglycoside) or an anti-pseudomonal cephalosporin (i.e. cefepime ) are generally added to metronidazole, and occasionally cefoxitin when treating intra-abdominal infections to provide coverage for these organisms. Clindamycin should not be used as a single agent as empiric therapy for abdominal infections. Penicillin can be added to metronidazole in treating of intracranial, pulmonary and dental infections to provide coverage against microaerophilic streptococci, and Actinomyces."}, {"id": "pubmed23n1033_22911", "title": "Update on the management of uncomplicated acute diverticulitis at our centre. Equally effective, more efficient.", "score": 0.009523809523809525, "content": "The current trend in the treatment of non-complicated diverticulitis is the outpatient management with antibiotic or even anti-inflammatory regimens in selected cases. We present a comparison of the results in our hospital with different protocols applied in 2016 and 2017. All patients selected for this study were diagnosed with diverticulitis grade Ia of Hinchey's classification according to radiological findings on abdominal CT. We have analyzed two retrospective cohorts: 100 patients treated in 2016 according to the old protocol and 104 patients treated in 2017 with a new protocol. In 2016, the candidates for ambulatory treatment remained under observation for 24 hours before being discharged. The treatment consisted of 14 days of ciprofloxacin and metronidazole. In 2017, only patients with more acute symptoms were observed 24 hours and amoxicillin-clavulanic acid was prescribed for only 5 days. The persistence of the disease in 2016 was 6% and in 2017 was only 5.77% (p = 0.944). Recurrence during the first year was 13% in the first group, while in the second it was 5.7%, although this difference was not statistically significant. Likewise, a significant reduction in health costs was achieved. Outpatient treatment of acute uncomplicated diverticulitis with oral treatment seems to be a safe and effective therapeutic strategy in selected patients with low comorbidity."}, {"id": "wiki20220301en035_79525", "title": "Peritonsillar abscess", "score": 0.009433962264150943, "content": "Diagnosis Diagnosis is usually based on the symptoms. Medical imaging may be done to rule out complications. Medical imaging may include CT scan, MRI, or ultrasound is also useful in diagnosis. Treatment Medical treatment with antibiotics, volume repletion with fluids, and pain medication is usually adequate, although in cases where airway obstruction or systemic sepsis occurs, surgical drainage may be necessary. Steroids may also be useful. Admission to hospital is generally not needed. Medication The infection is frequently penicillin resistant. There are a number of antibiotics options including amoxicillin/clavulanate, clindamycin, or metronidazole in combination with benzylpenicillin (penicillin G) or penicillin V. Piperacillin/tazobactam may also be used."}, {"id": "pubmed23n0893_17578", "title": "Flare-Up Diverticulitis in the Terminal Ileum in Short Interval after Conservative Therapy: Report of a Case.", "score": 0.009433962264150943, "content": "Diverticulitis in the terminal ileum is uncommon. Past reports suggested that conservative therapy may be feasible to treat terminal ileum diverticulitis without perforation; however, there is no consensus on the therapeutic strategy for small bowel diverticulitis. We present a 37-year-old man who was referred to our hospital for sudden onset of abdominal pain and nausea. He was diagnosed with diverticulitis in the terminal ileum by computed tomography (CT). Tazobactam/piperacillin hydrate (18 g/day) was administered. The antibiotic treatment was maintained for 7 days, and the symptoms disappeared after the treatment. Thirty-eight days after antibiotic therapy, he noticed severe abdominal pain again. He was diagnosed with diverticulitis in terminal ileum which was flare-up of inflammation. He was given antibiotic therapy again. Nine days after antibiotic therapy, laparoscopy assisted right hemicolectomy and resection of 20 cm of terminal ileum were performed. Histopathology report confirmed multiple ileal diverticulitis. He was discharged from our hospital 12 days after the surgery. Colonoscopy was performed two months after the surgery and it revealed no finding suggesting inflammatory bowel disease. Surgical treatment should be taken into account as a potential treatment option to manage the diverticulitis in the terminal ileum even though it is not perforated."}, {"id": "pubmed23n1130_13802", "title": "Polymicrobial bacteraemia with Clostridioides difficile and Pseudomonas aeruginosa in an elderly man following antibiotic use.", "score": 0.009345794392523364, "content": " 2 g/day), hypertension, smoking, hyperlipidemia, older age, familial disease and elevated creatinine concentrations are markers of a poor outcome. Frank hematuria has shown discordant results with most studies showing a better prognosis, perhaps related to the early diagnosis, except for one group which reported a poorer prognosis. Proteinuria and hypertension are the most powerful prognostic factors in this group. There are certain other features on kidney biopsy such as interstitial scarring which are associated with a poor prognosis. ACE gene polymorphism has been recently shown to have an impact with the DD genotype associated more commonly with progression to kidney failure."}]}}}} {"correct_option": 4, "explanations": {"1": {"exist": true, "char_ranges": [[0, 155]], "word_ranges": [[0, 29]], "text": "If they wanted us to answer that it is a TTP (answer 1) they would probably associate anemia and in the smear they would surely tell us about schistocytes."}, "2": {"exist": true, "char_ranges": [[239, 324]], "word_ranges": [[43, 59]], "text": "The other two answers, you do not have to hesitate with them. Nothing points to that."}, "3": {"exist": true, "char_ranges": [[239, 324]], "word_ranges": [[43, 59]], "text": "The other two answers, you do not have to hesitate with them. Nothing points to that."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "If they wanted us to answer that it is a TTP (answer 1) they would probably associate anemia and in the smear they would surely tell us about schistocytes. Not to say that the patient's symptoms would probably be much more than epistaxis. The other two answers, you do not have to hesitate with them. Nothing points to that.", "full_answer_no_ref": "If they wanted us to answer that it is a TTP (answer 1) they would probably associate anemia and in the smear they would surely tell us about schistocytes. Not to say that the patient's symptoms would probably be much more than epistaxis. [HIDDEN]. Nothing points to that.", "full_question": "An 18-year-old patient comes to the emergency department with epistaxis of several days' evolution, with no personal or family history of interest. On examination he is afebrile, multiple ecchymoses are observed, no splenomegaly is palpable. Laboratory tests: leukocytes 7.2 x103/μL, Hb 12.3 g/dL, platelets 6.0 x103/μL. Thrombocytopenia is confirmed in the smear, where platelets of increased size are observed. Coagulation and biochemistry studies are normal. What do you consider the most probable diagnosis?", "id": 493, "lang": "en", "options": {"1": "Thrombotic thrombocytopenic purpura.", "2": "Disseminated intravascular coagulation.", "3": "Thrombocytopenia induced by infection.", "4": "Primary immune thrombocytopenia.", "5": NaN}, "question_id_specific": 107, "type": "HEMATOLOGY", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en016_42764", "title": "Immune thrombocytopenic purpura", "score": 0.016569667921944538, "content": "Despite the destruction of platelets by splenic macrophages, the spleen is normally not enlarged. In fact, an enlarged spleen should lead to a search for other possible causes for the thrombocytopenia. Bleeding time is usually prolonged in ITP patients. However, the use of bleeding time in diagnosis is discouraged by the American Society of Hematology practice guidelines and a normal bleeding time does not exclude a platelet disorder. Bone marrow examination may be performed on patients over the age of 60 and those who do not respond to treatment, or when the diagnosis is in doubt. On examination of the marrow, an increase in the production of megakaryocytes may be observed and may help in establishing a diagnosis of ITP. An analysis for anti-platelet antibodies is a matter of clinician's preference, as there is disagreement on whether the 80 percent specificity of this test is sufficient to be clinically useful."}, {"id": "pubmed23n0724_12865", "title": "Thrombocytopenia.", "score": 0.014687032685323648, "content": "Thrombocytopenia is defined as a platelet count of less than 150 × 10(3) per µL. It is often discovered incidentally when obtaining a complete blood count during an office visit. The etiology usually is not obvious, and additional investigation is required. Patients with platelet counts greater than 50 × 10(3) per µL rarely have symptoms. A platelet count from 30 to 50 × 10(3) per µL rarely manifests as purpura. A count from 10 to 30 × 10(3) per µL may cause bleeding with minimal trauma. A platelet count less than 5 × 10(3) per µL may cause spontaneous bleeding and constitutes a hematologic emergency. Patients who present with thrombocytopenia as part of a multisystem disorder usually are ill and require urgent evaluation and treatment. These patients most likely have an acute infection, heparin-induced thrombocytopenia, liver disease, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, disseminated intravascular coagulation, or a hematologic disorder. During pregnancy, preeclampsia and the HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome are associated with thrombocytopenia. Patients with isolated thrombocytopenia commonly have drug-induced thrombocytopenia, immune thrombocytopenic purpura, pseudothrombocytopenia, or if pregnant, gestational thrombocytopenia. A history, physical examination, and laboratory studies can differentiate patients who require immediate intervention from those who can be treated in the outpatient setting. Treatment is based on the etiology and, in some cases, treating the secondary cause results in normalization of platelet counts. Consultation with a hematologist should be considered if patients require hospitalization, if there is evidence of systemic disease, or if thrombocytopenia worsens despite initial treatment."}, {"id": "pubmed23n1009_24869", "title": "Teetering on a liver's edge: a case report highlighting clinical decision-making in thrombocytopenia.", "score": 0.01432815794517922, "content": "This report illustrates the importance of a detailed history and physical exam and careful analysis of hematologic parameters when diagnosing ITP. This case demonstrates that even with subtle deviations from typical ITP findings one must promptly reevaluate the diagnosis. This case also highlights the importance of peripheral smear review by an expert in pediatric hematopathology. A previously healthy 10 year-old Asian boy presented with 2 months of easy bruising. Review of systems was negative for any constitutional symptoms. On examination, he appeared well but had numerous large ecchymoses. He had no appreciable lymphadenopathy or splenomegaly. The liver was palpable 1.5 cm below the costal margin. A complete blood count (CBC) showed: platelets = 17 × 109/L, hemoglobin = 128 g/L, white blood cell count = 5.43 × 109/L, and neutrophils = 1.63 × 109/L. A blood smear was reported as normal. Urate was 370 umol/L and lactate dehydrogenase (LDH) was 803 U/L. The child was admitted with a presumptive diagnosis of immune thrombocytopenic purpura (ITP) and treated with intravenous immunoglobulin. The following day, the blood smear was reviewed by a hematopathologist who identified blasts. A bone marrow aspiration (BMA) confirmed the diagnosis of precursor B-cell acute lymphoblastic leukemia. In children presenting with suspected ITP, leukemia should always be considered. A BMA was historically performed on all patients with presumed ITP to rule out leukemia. In 2011, the American Society of Hematology (ASH) stopped recommending routine BMA in patients suspected of having ITP. ASH advises in cases with unusual findings on history, physical examination or CBC, it is reasonable to perform a BMA. Our patient had mild hepatomegaly, which may have qualified him for a BMA. He also had an elevated LDH and urate, which are not listed as criteria for BMA by ASH but were considered atypical for ITP by the clinical team. A literature search did not reveal any primary data assessing these markers. While corticosteroids are a first line treatment in ITP, they must be reserved for when clinicians are confident that the patient does not have leukemia. Steroid administration prior to diagnosing leukemia results in delayed diagnosis and may increase the risk of complications and decrease survival."}, {"id": "wiki20220301en020_74480", "title": "Thrombocytopenia", "score": 0.014219443323920937, "content": "Thrombocytopenia is a condition characterized by abnormally low levels of platelets, also known as thrombocytes, in the blood. It is the most common coagulation disorder among intensive care patients and is seen in 20% of medical patients and a third of surgical patients. A normal human platelet count ranges from 150,000 to 450,000 platelets per microliter of blood. Values outside this range do not necessarily indicate disease. One common definition of thrombocytopenia requiring emergency treatment is a platelet count below 50,000 per microliter. Thrombocytopenia can be contrasted with the conditions associated with an abnormally high level of platelets in the blood: thrombocythemia (when the cause is unknown), and thrombocytosis (when the cause is known). Signs and symptoms"}, {"id": "wiki20220301en003_74321", "title": "Blood cell", "score": 0.012719106482769586, "content": "The normal range (99% of population analyzed) for platelets is 150,000 to 450,000 per cubic millimeter. If the number of platelets is too low, excessive bleeding can occur. However, if the number of platelets is too high, blood clots can form thrombosis, which may obstruct blood vessels and result in such events as a stroke, myocardial infarction, pulmonary embolism, or blockage of blood vessels to other parts of the body, such as the extremities of the arms or legs. An abnormality or disease of the platelets is called a thrombocytopathy, which can be either a low number of platelets (thrombocytopenia), a decrease in function of platelets (thrombasthenia), or an increase in the number of platelets (thrombocytosis). There are disorders that reduce the number of platelets, such as heparin-induced thrombocytopenia (HIT) or thrombotic thrombocytopenic purpura (TTP), that typically cause thromboses, or clots, instead of bleeding."}, {"id": "wiki20220301en299_31048", "title": "Gestational thrombocytopenia", "score": 0.012421065173358753, "content": "Diagnosis Gestational thrombocytopenia will become evident during the mid-second trimester through the third trimester of pregnancy and it is diagnosed based on exclusion. For example, women with a history of immune thrombocytopenia or thrombocytopenia, prior to pregnancy, will not be diagnosed with gestational thrombocytopenia. Patients with low platelet counts, lower than 70,000 / μL, will be difficult to diagnose. The reason is because low platelet counts maybe due to gestational thrombocytopenia or immune thrombocytopenia. In such cases, a treatment of immune thrombocytopenia therapy (corticosteroids, or intravenous immunoglobulin) will be instructed. If there is an improvement in the platelet levels, the patient will be diagnosed with immune thrombocytopenia, and if not the patient will be diagnosed with severe gestational thrombocytopenia In order for the physician to determine the underlying cause of the gestational thrombocytopenia, the following tests are conducted -"}, {"id": "wiki20220301en020_74481", "title": "Thrombocytopenia", "score": 0.012358655836916706, "content": "Signs and symptoms Thrombocytopenia usually has no symptoms and is picked up on a routine complete blood count. Some individuals with thrombocytopenia may experience external bleeding such as nosebleeds, or bleeding gums. Some women may have heavier or longer periods or breakthrough bleeding. Bruising, particularly purpura in the forearms and petechiae in the feet, legs, and mucous membranes, may be caused by spontaneous bleeding under the skin. Eliciting a full medical history is vital to ensure the low platelet count is not secondary to another disorder. Ensuring that the other blood cell types, such as red blood cells and white blood cells are not also suppressed, is also important. Painless, round, and pinpoint (1 to 3 mm in diameter) petechiae usually appear and fade, and sometimes group to form ecchymoses. Larger than petechiae, ecchymoses are purple, blue, or yellow-green areas of skin that vary in size and shape. They can occur anywhere on the body."}, {"id": "pubmed23n0753_19757", "title": "Idiopathic thrombocytopenic purpura associated with splenic tuberculosis: case report.", "score": 0.011648145668764227, "content": "Tuberculosis is still one of the most prevalent and fatal infectious diseases in spite of considerable improvements in medical science. Splenic tuberculosis is a rare form of extrapulmonary tuberculosis. There are limited numbers of cases in which immune thrombocytopenia is associated with splenic tuberculosis. We report a case of immune thrombocytopenic purpura due to splenic tuberculosis. Our case was a 58-year-old female with headache, gum bleeding, redness in legs, and ecchymoses on the arms for 10 days. On admission to hospital, laboratory tests were as follows: platelet count 6.000/mmc (150 000-450 000), haemoglobin: 12 g/dl, WBC: 8000/mm3, erythrocyte sedimentation rate: 58 mm/h and C-reactive protein was in normal ranges. After standard laboratory tests, the patient was diagnosed with idiopathic thrombocytopenic purpura. The patient presented abdominal lymphadenopathies and spleen in normal size in radiological examinations. Diagnostic laparotomy and splenectomy and lymph node excision was performed and splenic tuberculosis was detected in pathologic and microbiologic examination. The patient was successfully treated with apheresis platelets suspension, intravenous immunoglobulin and antituberculous therapy. In conclusion, splenic tuberculosis should be suspected in patients who have fever, abdominal lymphadenopathies and immune thrombocytopenic purpura. Histopathological examination is still an ideal method to confirm the diagnosis, suitably aided by microbiological examination."}, {"id": "InternalMed_Harrison_9124", "title": "InternalMed_Harrison", "score": 0.011292696543673552, "content": "The history and physical examination, results of the CBC, and review of the peripheral blood smear are all critical components in the initial evaluation of thrombocytopenic patients (Fig. 140-2). The overall health of the patient and whether he or she is receiving drug treatment will influence the differential diagnosis. A healthy young adult with thrombocytopenia will have a much more limited differential diagnosis than an ill hospitalized patient who is receiving multiple medications. Except in unusual inherited disorders, decreased platelet production usually results from bone marrow disorders that also affect red blood cell (RBC) and/or white blood cell (WBC) production. Because myelodysplasia can present with isolated thrombocytopenia, the bone marrow should be examined in patients presenting with isolated thrombocytopenia who are older than 60 years of age. While inherited thrombocytopenia is rare, any prior platelet counts should be retrieved and a family history regarding"}, {"id": "wiki20220301en046_51369", "title": "Schistocyte", "score": 0.011236309692895245, "content": "Schistocyte count A normal schistocyte count for a healthy individual is <0.5% although usual values are found to be <0.2%. A schistocyte count of >1% is most often found in thrombotic thrombocytopenic purpura, although they are more often seen within the range of 3–10% for this condition. A schistocyte count of <1% but greater than the normal value is suggestive of disseminated intravascular coagulation, but is not an absolute diagnosis. The standard for a schistocyte count is a microscopic examination of a peripheral blood smear."}, {"id": "wiki20220301en020_74489", "title": "Thrombocytopenia", "score": 0.011224201300537178, "content": "Immune thrombocytopenic purpura Many cases of immune thrombocytopenic purpura (ITP) also known as idiopathic thrombocytopenic purpura, can be left untreated, and spontaneous remission (especially in children) is not uncommon. However, counts under 50,000 are usually monitored with regular blood tests, and those with counts under 10,000 are usually treated, as the risk of serious spontaneous bleeding is high with such low platelet counts. Any patient experiencing severe bleeding symptoms is also usually treated. The threshold for treating ITP has decreased since the 1990s; hematologists recognize that patients rarely spontaneously bleed with platelet counts greater than 10,000, although exceptions to this observation have been documented."}, {"id": "InternalMed_Harrison_9237", "title": "InternalMed_Harrison", "score": 0.010845896147403685, "content": "The mortality ranges from 30 to >80% depending on the underlying disease, the severity of the DIC, and the age of the patient. The diagnosis of clinically significant DIC is based on the presence of clinical and/or laboratory abnormalities of coagulation or thrombocytopenia. The laboratory diagnosis of DIC should prompt a search for the underlying disease if it is not already apparent. There is no single test that establishes the diagnosis of DIC. The laboratory investigation should include coagulation tests (aPTT, PT, thrombin time [TT]) and markers of fibrin degradation products (FDPs), in addition to platelet and red cell count and analysis of the blood smear. These tests should be repeated over a period of 6–8 h because an initially mild abnormality can change dramatically in patients with severe DIC."}, {"id": "wiki20220301en299_31042", "title": "Gestational thrombocytopenia", "score": 0.01043955012298391, "content": "Gestational (incidental) thrombocytopenia is a condition that commonly affects pregnant women. Thrombocytopenia is defined as the drop in platelet count from the normal range of 150,000 –400,000 / μL to a count lower than 150,000 / μL. There is still ongoing research to determine the reason for the lowering of platelet count in women with a normal pregnancy. Some researchers speculate the cause to be dependent on dilution, decreased production of platelets, or an increased turnover event. Although women with normal pregnancy experience a low platelet count, women experiencing a continuous drop in platelet will be diagnosed with thrombocytopenia and women with levels greater than 70,000 / μL will be diagnosed with gestational thrombocytopenia. Thrombocytopenia affects approximately 7-10% of pregnant women and of the 7-10%, within that population; approximately 70-80% have gestational thrombocytopenia"}, {"id": "wiki20220301en083_19322", "title": "Thrombocytopenic purpura", "score": 0.010408970402885827, "content": "Thrombocytopenic purpura are purpura associated with a reduction in circulating blood platelets which can result from a variety of causes, such as kaposi sarcoma. Types By tradition, the term idiopathic thrombocytopenic purpura is used when the cause is idiopathic. However, most cases are now considered to be immune-mediated. Another form is thrombotic thrombocytopenic purpura. Diagnosis Diagnosis is done by the help of symptoms and only blood count abnormality is thrombocytopenia. Treatment See also Aspirin Hematopoietic ulcer Thrombocyte References External links Vascular-related cutaneous conditions Coagulopathies"}, {"id": "article-30096_27", "title": "Thrombocytopenia in Pregnancy -- History and Physical", "score": 0.00992843554669807, "content": "A physical exam provides clues towards determining the etiology of thrombocytopenia. Patients with gestational thrombocytopenia are often healthy, asymptomatic women who are found to have low platelets on lab investigations. [6] [5] [4] On the contrary, a pregnant woman with TMA is usually a gravely-ill patient often presenting to the emergency room or admitted to high-risk obstetric units. Patients with low platelet count usually present with mucocutaneous bleeding. However, joint bleed or severe bleeding should prompt a workup towards severe coagulopathies like DIC. Physical exam should also evaluate for hepatomegaly and/or splenomegaly (cirrhosis, lymphoproliferative disorders, etc.), skeletal deformities (like absent radius, humeral abnormality and sometimes phocomelia seen in thrombocytopenia absent radii syndrome) and skin exam (like petechiae or purpura seen commonly with ITP, or, eczema seen in Wiskott-Aldrich Syndrome). [7] [29]"}, {"id": "wiki20220301en020_74485", "title": "Thrombocytopenia", "score": 0.009900990099009901, "content": "Increased destruction Abnormally high rates of platelet destruction may be due to immune or nonimmune conditions, including: Immune thrombocytopenic purpura Thrombotic thrombocytopenic purpura Hemolytic–uremic syndrome Disseminated intravascular coagulation Paroxysmal nocturnal hemoglobinuria Antiphospholipid syndrome Systemic lupus erythematosus Post-transfusion purpura Neonatal alloimmune thrombocytopenia Hypersplenism Dengue fever Gaucher's disease Zika virus Medication-induced These medications can induce thrombocytopenia through direct myelosuppression: Valproic acid Methotrexate Carboplatin Interferon Isotretinoin Panobinostat H2 blockers and proton-pump inhibitors Other causes Lab error, possibly due to the anticoagulant EDTA in CBC specimen tubes; a citrated platelet count is a useful follow-up study Snakebite Niacin toxicity Lyme disease Thrombocytapheresis (also called plateletpheresis) Niemann–Pick disease Diagnosis"}, {"id": "pubmed23n0286_11225", "title": "Efficient diagnosis of thrombocytopenia.", "score": 0.009900990099009901, "content": "Thrombocytopenia may be a benign, incidental finding in an asymptomatic patient or the sign of a potentially life-threatening disorder. The history, physical examination and peripheral blood smear can assist the physician in determining the diagnosis and treatment. After the initial blood count is repeated to help eliminate the possibility of laboratory error, a thorough history, complete physical examination, complete blood cell count and appropriate laboratory tests are required. The history may reveal related illnesses, risk factors such as infection or drug use, or a family history suggestive of congenital thrombocytopenia. The physical examination should concentrate on the lymphatic and hepatosplenic systems, with the physician looking for jaundice, fever or petechiae. With the review of a complete blood cell count and a peripheral smear examination, the initial work-up is completed and may prevent additional, unnecessary testing. Etiology-specific tests follow if needed. Serious spontaneous bleeding is usually a risk only in patients with platelet levels under 20,000 per mm3."}, {"id": "wiki20220301en011_19172", "title": "Coagulation", "score": 0.00980392156862745, "content": "Decreased platelet numbers (thrombocytopenia) is due to insufficient production (e.g., myelodysplastic syndrome or other bone marrow disorders), destruction by the immune system (immune thrombocytopenic purpura/ITP), or consumption (e.g., thrombotic thrombocytopenic purpura/TTP, hemolytic-uremic syndrome/HUS, paroxysmal nocturnal hemoglobinuria/PNH, disseminated intravascular coagulation/DIC, heparin-induced thrombocytopenia/HIT). Most consumptive conditions lead to platelet activation, and some are associated with thrombosis. Coagulation factor disorders The best-known coagulation factor disorders are the hemophilias. The three main forms are hemophilia A (factor VIII deficiency), hemophilia B (factor IX deficiency or \"Christmas disease\") and hemophilia C (factor XI deficiency, mild bleeding tendency)."}, {"id": "article-30093_24", "title": "Thrombocytopenia -- History and Physical", "score": 0.00980392156862745, "content": "History. Obtaining a thorough history helps to identify the etiology of thrombocytopenia. Patients with platelets greater than 50000/mL, rarely have symptoms. Patients with platelets under 20000/mL most likely have spontaneous bleeding."}, {"id": "wiki20220301en004_59256", "title": "Lemierre's syndrome", "score": 0.00976699648898819, "content": "Production of bacterial toxins such as lipopolysaccharide leads to secretion of cytokines by white blood cells which then both lead to symptoms of sepsis. F. necrophorum produces hemagglutinin which causes platelet aggregation that can lead to diffuse intravascular coagulation and thrombocytopenia. Diagnosis Diagnosis and the imaging (and laboratory) studies to be ordered largely depend on the patient history, signs and symptoms. If a persistent sore throat with signs of sepsis are found, physicians are cautioned to screen for Lemierre's syndrome. Laboratory investigations reveal signs of a bacterial infection with elevated C-reactive protein, erythrocyte sedimentation rate and white blood cells (notably neutrophils). Platelet count can be low or high. Liver and kidney function tests are often abnormal."}, {"id": "wiki20220301en010_97374", "title": "Platelet", "score": 0.009708737864077669, "content": "Disorders Adapted from: The three broad categories of platelet disorders are \"not enough\"; \"dysfunctional\"; and \"too many\". Thrombocytopenia Immune thrombocytopenias (ITP) – formerly known as immune thrombocytopenic purpura and idiopathic thrombocytopenic purpura Splenomegaly Gaucher's disease Familial thrombocytopenia Chemotherapy Babesiosis Dengue fever Onyalai Thrombotic thrombocytopenic purpura HELLP syndrome Hemolytic–uremic syndrome Drug-induced thrombocytopenic purpura (five known drugs – most problematic is heparin-induced thrombocytopenia (HIT) Pregnancy-associated Neonatal alloimmune associated Aplastic anemia Transfusion-associated Pseudothrombocytopenia idiopathic thrombocytopenic purpura Vaccine induced immune thrombocytopenia Gilbert's syndrome"}, {"id": "article-30093_32", "title": "Thrombocytopenia -- History and Physical", "score": 0.009708737864077669, "content": "Check for a family history of thrombocytopenia or bleeding disorders."}, {"id": "wiki20220301en141_24158", "title": "Neonatal alloimmune thrombocytopenia", "score": 0.009615384615384616, "content": "Related conditions Immune thrombocytopenic purpura (), sometimes called idiopathic thrombocytopenic purpura is a condition in which autoantibodies are directed against a patient's own platelets, causing platelet destruction and thrombocytopenia. Anti-platelet autoantibodies in a pregnant woman with immune thrombocytopenic purpura will attack the patient's own platelets and will also cross the placenta and react against fetal platelets. Therefore, is a significant cause of fetal and neonatal immune thrombocytopenia. Approximately 10% of newborns affected by will have platelet counts <50,000 μL−1 and 1% to 2% will have a risk of intracerebral hemorrhage comparable to infants with ."}, {"id": "pubmed23n0068_5112", "title": "[The analysis of national examination for the license of medical practice in Japan (reference to the hematological clinical laboratory data)].", "score": 0.009615384615384616, "content": "It seems to us an important task to analyze the problems appeared in the national examinations of the license of medical practice in respect of clinical laboratory data. The national examination of medical license in Japan begun from 1946, about 45 years ago. In those days, clinical examination is not so developed, a pastoral era, so clinical pathology was not clearly distinguished from internal medicine. Recently it is widely emphasized that national examination for the practical medical practice should be focused on the primary care medicine, so on, which has evidently influenced the appearance rate and number of miscellaneous kinds of clinical laboratory data in the examination problems. On the other hand, We have studied the tendency and mode of appearance of clinical laboratory data in the question of above mentioned examination for several years, in general, and especially in the areas of hematology. The cardinal questions of hematological field were blood cell count (RBC, hematocrit, hemoglobin, WBC, white cell differential, PLATELET), blood sedimentation rate, coagulation and fibrinolysis factor, bone marrow findings in miscellaneous diseases, classification and staging of malignant lymphomas, so on. In conclusion, the rate of appearance and significance of clinical laboratory data in the examination mentioned is more and more increased. However, comprehensive education of clinical pathology now is improved but not accomplished, which reflects tendency and styles of the problems of the examination. So more efforts will be needed to those who continuously study clinical pathology and educate them to the medical students."}, {"id": "wiki20220301en043_23414", "title": "Plateletpheresis", "score": 0.009523809523809525, "content": "Platelet transfusion Platelet transfusions are traditionally given to those undergoing chemotherapy for leukemia, multiple myeloma, those with aplastic anemia, AIDS, hypersplenism, idiopathic thrombocytopenic purpura (ITP), sepsis, bone marrow transplant, radiation treatment, organ transplant or surgeries such as cardiopulmonary bypass. Platelet transfusions should be avoided in those with thrombotic thrombocytopenic purpura (TTP) because it can worsen neurologic symptoms and acute renal failure, presumably due to creation of new thrombi as the platelets are consumed. It should also be avoided in those with heparin-induced thrombocytopenia (HIT) or disseminated intravascular coagulation (DIC). In adults, platelets are recommended in those who have levels less than 10,000/µL, or less than 20,000/µL if a central venous catheter is being placed, or less than 50,000/µL if a lumbar puncture or major surgery is required. Whole blood platelets"}, {"id": "pubmed23n0416_22915", "title": "[The diagnosis of ITP].", "score": 0.009523809523809525, "content": "Idiopathic thrombocytopenic purpura(ITP) is a hematologic disorder which causes thrombocytopenia. The diagnosis of ITP is based on the history, physical examination and, complete blood count, and examination of the peripheral smear. The diagnostic criteria of ITP established by the Ministry of Health, Welfare, and Labor in Japan requires the bone marrow examination and the measurement of platelet associated IgG, but those tests are not always necessary according to the guidelines developed by the American Society of Hematology. The appropriate strategies for the diagnosis of ITP need to be established. In this paper, some new examinations which may help the diagnosis of ITP are also demonstrated."}, {"id": "InternalMed_Harrison_9126", "title": "InternalMed_Harrison", "score": 0.009451135423370415, "content": "The physical examination can document an enlarged spleen, evidence of chronic liver disease, and other underlying disorders. Mild to moderate splenomegaly may be difficult to appreciate in many individuals due to body habitus and/or obesity but can be easily assessed by abdominal ultrasound. A platelet count of approxi- FIGURE 140-2 Algorithm for evaluating the thrombocytopenic mately 5000–10,000 is required to maintain vascular integrity in the patient. DIC, disseminated intravascular coagulation; RBC, red blood microcirculation. When the count is markedly decreased, petechiae cell; TTP, thrombotic thrombocytopenic purpura."}, {"id": "wiki20220301en141_24159", "title": "Neonatal alloimmune thrombocytopenia", "score": 0.009433962264150943, "content": "Mothers with thrombocytopenia or a previous diagnosis of should be tested for serum anti-platelet antibodies. A woman with symptomatic thrombocytopenia and an identifiable anti-platelet antibody should be started on therapy for their which may include steroids or . Fetal blood analysis to determine the platelet count is not generally performed as -induced thrombocytopenia in the fetus is generally less severe than . Platelet transfusions may be performed in newborns, depending on the degree of thrombocytopenia. Other conditions causing a low platelet count Other conditions that can cause a low platelet count in the neonate include bacterial and viral infection, disseminated intravascular coagulation and other rare congenital conditions associated with a low platelet count."}, {"id": "pubmed23n0965_5066", "title": "[Management of thrombocytopenia].", "score": 0.009433962264150943, "content": "Thrombocytopenia is defined by a platelet level below 150 G/l. However, the limit of 100 G/l seems more appropriate to determine which thrombocytopenia will require further investigation. Initially, a thorough medical history should be performed as well as screening for any signs of bleeding. After having excluded the presence of platelet aggregates, it should be determined whether thrombocytopenia is isolated or associated with other abnormalities (cytopenias, coagulation disorder, abnormal renal or liver tests). Causes of thrombocytopenia along with the biological tests to achieve diagnosis, will be detailed in this article. We will then determine the medical emergencies that will need to be addressed to a reference center : active bleeding, biological signs of disseminated intravascular coagulation, acute renal failure, platelet count < 30 G/l (or < 50 G/l if the patient is on anticoagulation or antiplatelet therapy), significant and/or brutal onset pancytopenia. Outside these situations where vital prognosis is engaged, the patient should be rapidly addressed in case of platelet count between 30 and 50 G/l without any concomitant anticoagulation or antiplatelet therapy. Platelet levels between 50 and 100 G/l will require investigation, without any urgency, in outpatient haematology clinic."}, {"id": "wiki20220301en299_31052", "title": "Gestational thrombocytopenia", "score": 0.009345794392523364, "content": "Women who have platelet levels lower than 70,000 / μL, during pregnancy, maybe experiencing severe gestational thrombocytopenia or immune thrombocytopenia. In such cases, if the treatment of immune thrombocytopenia therapy (corticosteroids, or intravenous immunoglobulin) does not improve the platelet count, the patient will be diagnosed with severe gestational thrombocytopenia. Severe gestational thrombocytopenia may pose a risk for complications with the use of epidural or general anesthesia during delivery. Prognosis Those who have no previous history of thrombocytopenia, besides the occurrence in previous pregnancies (gestational thrombocytopenia), the platelet levels will go back to a normal range 1–2 months after the delivery. Post delivery, approximately 1–3 months later, women with gestational thrombocytopenia should have a complete blood test conducted. Lastly, gestational thrombocytopenia is a disorder that may reoccur in future pregnancies"}, {"id": "article-20610_7", "title": "Disseminated Intravascular Coagulation -- Evaluation", "score": 0.009345794392523364, "content": "No single history, physical exam, or laboratory component can lead to a diagnosis of or rule out DIC; therefore, a combination of both subjective, objective, and laboratory findings should be utilized to make a diagnosis of DIC. Laboratory findings suggestive of DIC include both an increased prothrombin time (PT) and an increased partial thromboplastin time (PTT), as well as a decreased fibrinogen level as widespread activation and consumption of the clotting cascade occurs. The overall platelet count and hematocrit level may be reduced as well. Schistocytes or fragmented erythrocytes are also commonly seen on a peripheral blood smear. The presence of fibrin split products additionally has a high sensitivity but low specificity for the presence of DIC. A specific scoring system to assess for the presence of DIC was established in 2007. This score includes platelet count, fibrin markers such as D-dimer, prolonged PT, and fibrinogen level, with a score over five indicating a high likelihood for overt DIC. [18] [19] [20] [21]"}, {"id": "pubmed23n0319_6679", "title": "[Idiopathic thrombocytopenic purpura in children].", "score": 0.009333962749978542, "content": "Idiopathic (immune) thrombocytopenic purpura (ITP) is the most frequent hemorrhagic disease in children. It represents the acquired megakaryocytic thrombocytopenia with the shortened life of platelets because of immunologic damage (antibodies absorbed by platelets). In the case of this acquired hemorrhagic disorder, in spite of compensatory increased function of the bone marrow, there is a reduced number of platelets because of their increased destruction by the reticuloendothelial system (destructive thrombocytopenia). There are three forms of ITP: acute, chronic and intermittent. The acute form occurs in 80-90% of cases with bleeding episodes lasting a few days or weeks, but no longer than 6 months. The chronic form occurs in 10-15% of children, while the rarest-intermittent form is characterized by periods of normalization in regard to the number of platelets but also with relapse in intervals of 1-3 months. The disease is caused by an immunological disorder in the sense of an imbalanced immune response. Immunologic damages of platelets cause shortening of the opsonized platelets life span. The most frequent platelet opsonins are the immumoglobulin G (IgG) antibodies directed at the platelet membrane in the form of autoantibodies, alloantibodies or possibly absorbed antigen caused by microorganism infection or drug intake. It is typical for the phenomenon of bleeding that it starts suddenly and without any other sign of illness. The most frequent acute form appears between the second and fourth year, and is characterized by seasonal occurrence usually after acute viral infections. Children older than 10 years of age, like adults, often have the chronic form associated with other immunologic disorders. The disease affects girls more often than boys (about three times more often) with moderate and constant increase of antiplatelet antibodies. Hemorrhagic manifestations include: petechiae, purpura, epistaxis, gastrointestinal and genitourinary bleeding. They depend on the grade of thrombocytopenia, although there is no strict correlation between the number of platelets and volume of bleeding. Low mortality of the acute ITP is almost exclusively due to intracranial hemorrhage. LABORATORY STUDIES: Thrombocytopenia represents a decrease in the number of blood platelets being a basic abnormality of the blood count. The half-life of platelets in ITP is shortened. Detection of antiplatelet antibodies is connected with technical difficulties, so they are established in about 30% of cases. Bleeding time is prolonged and so is the coagulum retraction which may be completely missed. The Rumpel-Leede test is positive. Clinical differentiation of drug-induced thrombocytopenia is not possible. However, other differential diagnostic possibilities are thrombotic-thrombocytopenic purpura and hemolytic-uremic syndrome. A child with aplastic anemia or acute leukemia, beside thrombocytopenia, has a characteristic finding of white and red blood cell count. Thrombocytopenia with absent radii syndrome is associated with skeletal system abnormalities. New knowledge about the role of the immune system in ITP determines the modern therapeutic modalities. In cases of acute ITP in children, there are two therapeutic options or therapies of choice: corticosteroids and high doses of intravenous immunoglobulin. Immunosupressive therapy means anti Rh(D) immunoglobulin, cyclosporine, cytostatics, danazol, loaded platelets. In cases of distinctive hemorrhagic syndrome there are also indications for platelet transfusion. Nowadays splenectomy is more restricted, because one third of cases is unsuccessful, whereas plasmapheresis is rarely used in children because of possible complications. ITP is the most frequent hemorrhagic disease in children. The disease is basically caused by an immunologic disorder with platelet destruction due to increased immunoglobulin on their membrane. (ABSTRACT TRUNCATED)"}]}}}} {"correct_option": 2, "explanations": {"1": {"exist": true, "char_ranges": [[0, 225]], "word_ranges": [[0, 37]], "text": "A glomerular filtration rate of less than 15 ml/min is classified as stage 5 (G5), indicating that the renal disease is end-stage and the initiation of replacement therapy should be considered (option 2 true, option 1 false)."}, "2": {"exist": true, "char_ranges": [[0, 225]], "word_ranges": [[0, 37]], "text": "A glomerular filtration rate of less than 15 ml/min is classified as stage 5 (G5), indicating that the renal disease is end-stage and the initiation of replacement therapy should be considered (option 2 true, option 1 false)."}, "3": {"exist": true, "char_ranges": [[226, 367]], "word_ranges": [[37, 60]], "text": "In this situation, good glycemic control is not expected to reverse renal damage, although it may help to delay progression (false choice 3)."}, "4": {"exist": true, "char_ranges": [[368, 492]], "word_ranges": [[60, 80]], "text": "These patients usually present stage A3 albuminuria (above 300 mg/24 h), and may reach the nephrotic range (false choice 4)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "A glomerular filtration rate of less than 15 ml/min is classified as stage 5 (G5), indicating that the renal disease is end-stage and the initiation of replacement therapy should be considered (option 2 true, option 1 false). In this situation, good glycemic control is not expected to reverse renal damage, although it may help to delay progression (false choice 3). These patients usually present stage A3 albuminuria (above 300 mg/24 h), and may reach the nephrotic range (false choice 4).", "full_answer_no_ref": "A glomerular filtration rate of less than 15 ml/min is classified as stage 5 (G5), indicating that the renal disease is end-stage and the initiation of replacement therapy should be considered ([HIDDEN]). In this situation, good glycemic control is not expected to reverse renal damage, although it may help to delay progression ([HIDDEN]). These patients usually present stage A3 albuminuria (above 300 mg/24 h), and may reach the nephrotic range ([HIDDEN]).", "full_question": "A 67-year-old woman with chronic kidney disease secondary to diabetic nephropathy. She currently has a creatinine of 3.2 mg/dL with estimated glomerular filtration rate (CKD-EPI) 14 ml/min. Which of the following is true?", "id": 566, "lang": "en", "options": {"1": "He presents stage 3 chronic kidney disease and should initiate phosphorus chelators.", "2": "He has stage 5 chronic kidney disease and renal replacement therapy should be considered.", "3": "Good glycemic control can reverse renal failure.", "4": "It is unlikely to present proteinuria higher than 500 mg/24 h.", "5": NaN}, "question_id_specific": 149, "type": "NEPHROLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en013_55993", "title": "Kidney failure", "score": 0.012782220509361852, "content": "Diagnostic approach Measurement for CKD Stages of kidney failure Chronic kidney failure is measured in five stages, which are calculated using the person's GFR, or glomerular filtration rate. Stage 1 CKD is mildly diminished renal function, with few overt symptoms. Stages 2 and 3 need increasing levels of supportive care from their medical providers to slow and treat their renal dysfunction. People with stage 4 and 5 kidney failure usually require preparation towards active treatment in order to survive. Stage 5 CKD is considered a severe illness and requires some form of renal replacement therapy (dialysis) or kidney transplant whenever feasible. Glomerular filtration rate"}, {"id": "wiki20220301en043_69809", "title": "Diabetic nephropathy", "score": 0.012281942479962282, "content": "Diabetic nephropathy, also known as diabetic kidney disease, is the chronic loss of kidney function occurring in those with diabetes mellitus. Diabetic nephropathy is one of the leading causes of chronic kidney disease (CKD) and end-stage renal disease (ESRD) globally. Protein loss in the urine due to damage to the glomeruli may become massive, and cause a low serum albumin with resulting generalized body swelling (edema) and result in the nephrotic syndrome. Likewise, the estimated glomerular filtration rate (eGFR) may progressively fall from a normal of over 90 ml/min/1.73m2 to less than 15, at which point the patient is said to have end-stage renal disease. It usually is slowly progressive over years."}, {"id": "wiki20220301en025_100469", "title": "Chronic kidney disease", "score": 0.012274871355060033, "content": "It may also be useful at an earlier stage (e.g. CKD3) when urine albumin-to-creatinine ratio is more than 30 mg/mmol, when blood pressure is difficult to control, or when hematuria or other findings suggest either a primarily glomerular disorder or secondary disease amenable to specific treatment. Other benefits of early nephrology referral include proper education regarding options for kidney replacement therapy as well as pre-emptive transplantation, and timely workup and placement of an arteriovenous fistula in those people with chronic kidney disease opting for future hemodialysis. Renal replacement therapy At stage 5 CKD, kidney replacement therapy is usually required, in the form of either dialysis or a kidney transplant."}, {"id": "wiki20220301en002_195610", "title": "Dialysis", "score": 0.011694386694386695, "content": "In medicine, dialysis (from Greek διάλυσις, dialysis, \"dissolution\"; from διά, dia, \"through, and λύσις, lysis, \"loosening or splitting\") is the process of removing excess water, solutes, and toxins from the blood in people whose kidneys can no longer perform these functions naturally. This is referred to as renal replacement therapy. The first successful dialysis was performed in 1943. Dialysis may need to be initiated when there is a sudden rapid loss of kidney function, known as acute kidney injury (previously called acute renal failure), or when a gradual decline in kidney function chronic kidney disease reaches stage 5. Stage 5 chronic renal failure is reached when the glomerular filtration rate is 10–15% of normal, creatinine clearance is less than 10 mL per minute and uremia is present."}, {"id": "wiki20220301en019_111576", "title": "Glomerular filtration rate", "score": 0.011484256533761485, "content": "The severity of chronic kidney disease (CKD) is described by six stages; the most severe three are defined by the MDRD-eGFR value, and first three also depend on whether there is other evidence of kidney disease (e.g., proteinuria): 0) Normal kidney function – GFR above 90 mL/min/1.73 m2 and no proteinuria 1) CKD1 – GFR above 90 mL/min/1.73 m2 with evidence of kidney damage 2) CKD2 (mild) – GFR of 60 to 89 mL/min/1.73 m2 with evidence of kidney damage 3) CKD3 (moderate) – GFR of 30 to 59 mL/min/1.73 m2 4) CKD4 (severe) – GFR of 15 to 29 mL/min/1.73 m2 5) CKD5 kidney failure – GFR less than 15 mL/min/1.73 m2 Some people add CKD5D for those stage 5 patients requiring dialysis; many patients in CKD5 are not yet on dialysis. Note: others add a \"T\" to patients who have had a transplant regardless of stage."}, {"id": "wiki20220301en013_55980", "title": "Kidney failure", "score": 0.011452216254623728, "content": "Causes of acute kidney failure include low blood pressure, blockage of the urinary tract, certain medications, muscle breakdown, and hemolytic uremic syndrome. Causes of chronic kidney failure include diabetes, high blood pressure, nephrotic syndrome, and polycystic kidney disease. Diagnosis of acute failure is often based on a combination of factors such as decreased urine production or increased serum creatinine. Diagnosis of chronic failure is based on a glomerular filtration rate (GFR) of less than 15 or the need for renal replacement therapy. It is also equivalent to stage 5 chronic kidney disease."}, {"id": "InternalMed_Harrison_28029", "title": "InternalMed_Harrison", "score": 0.011254825287010578, "content": "FIGURE 419-3 Time course of development of diabetic nephropathy. The relationship of time from onset of diabetes, the glomerular filtration rate (GFR), and the serum creatinine are shown. (Adapted from RA DeFranzo, in Therapy for Diabetes Mellitus and Related Disorders, 3rd ed. American Diabetes Association, Alexandria, VA, 1998.) 2426 Improved glycemic control reduces the rate at which microalbuminuria appears and progresses in type 1 and type 2 DM. However, once macroalbuminuria is present, it is unclear whether improved glycemic control will slow progression of renal disease. During the later phase of declining renal function, insulin requirements may fall as the kidney is a site of insulin degradation. As the GFR decreases with progressive nephropathy, the use and dose of glucose-lowering agents should be reevaluated (see Table 418-5). Some glucose-lowering medications (sulfonylureas and metformin) are contraindicated in advanced renal insufficiency. Many individuals with type 1"}, {"id": "wiki20220301en023_76153", "title": "Assessment of kidney function", "score": 0.011158706361105162, "content": "The most relevant assessments in a renal ultrasound are renal sizes, echogenicity and any signs of hydronephrosis. Renal enlargement usually indicates diabetic nephropathy, focal segmental glomerular sclerosis or myeloma. Renal atrophy suggests longstanding chronic renal disease. Chronic kidney disease stages Risk factors for kidney disease include diabetes, high blood pressure, family history, older age, ethnic group and smoking. For most patients, a GFR over 60 (mL/min)/(1.73 m2) is adequate. But significant decline of the GFR from a previous test result can be an early indicator of kidney disease requiring medical intervention. The sooner kidney dysfunction is diagnosed and treated the greater odds of preserving remaining nephrons, and preventing the need for dialysis."}, {"id": "pubmed23n0652_6097", "title": "Large kidneys predict poor renal outcome in subjects with diabetes and chronic kidney disease.", "score": 0.011116229080300938, "content": "Renal hypertrophy occurs early in diabetic nephropathy, its later value is unknown. Do large kidneys still predict poor outcome in patients with diabetes and Chronic Kidney Disease (CKD)? Seventy-five patients with diabetes and CKD according to a Glomerular Filtration Rate (GFR, by 51Cr-EDTA clearance) below 60 mL/min/1.73 m2 or an Albumin Excretion Rate above 30 mg/24 H, had an ultrasound imaging of the kidneys and were cooperatively followed during five years by the Diabetology and Nephrology departments of the Centre Hospitalier Universitaire de Bordeaux. The patients were mainly men (44/75), aged 62 +/- 13 yrs, with long-standing diabetes (duration:17 +/- 9 yrs, 55/75 type 2), and CKD: initial GFR: 56.5 (8.5-209) mL/min/1.73 m2, AER: 196 (20-2358) mg/24 H. Their mean kidney lenght (108 +/- 13 mm, 67-147) was correlated to the GFR (r = 0.23, p < 0.05). During the follow-up, 9/11 of the patients who had to start dialysis came from the half with the largest kidneys (LogRank: p < 0.05), despite a 40% higher initial isotopic GFR. Serum creatinine were initially lower (Small kidneys: 125 (79-320) micromol/L, Large: 103 (50-371), p < 0.05), but significantly increased in the \"large kidneys\" group at the end of the follow-up (Small kidneys: 129 (69-283) micromol/L, Large: 140 (50-952), p < 0.005 vs initial). The difference persisted in the patients with severe renal failure (KDOQI stages 4,5). Large kidneys still predict progression in advanced CKD complicating diabetes. In these patients, ultrasound imaging not only excludes obstructive renal disease, but also provides information on the progression of the renal disease."}, {"id": "wiki20220301en043_69821", "title": "Diabetic nephropathy", "score": 0.010927102238354507, "content": "It is recommended that diabetics have their albumin levels checked annually, beginning immediately after a diagnosis of type 2 diabetes and five years after a diagnosis of type 1 diabetes. Medical imaging of the kidneys, generally by ultrasonography, is recommended as part of a differential diagnosis if there is suspicion of urinary tract obstruction, urinary tract infection, kidney stones or polycystic kidney disease. Conformation kidney biopsy should only be performed if non-diabetic kidney disease is suspected. Urine analysis in patients with diabetic kidney disease is often bland. In cases of severely increased microalbuminuria, hematuria might be present. fat bodies might be present in patients who develop nephrotic-range proteinuria. Staging To stage the degree of damage in this (and any) kidney disease, the serum creatinine is determined and used to calculate the estimated glomerular filtration rate (eGFR). Normal eGFR is equal to or greater than 90ml/min/1.73 m2."}, {"id": "wiki20220301en025_100459", "title": "Chronic kidney disease", "score": 0.01061831250510496, "content": "Stage 3: Moderate reduction in GFR (30–59 ml/min/1.73 m2):. British guidelines distinguish between stage 3A (GFR 45–59) and stage 3B (GFR 30–44) for purposes of screening and referral. Stage 4: Severe reduction in GFR (15–29 ml/min/1.73 m2) Preparation for kidney replacement therapy. Stage 5: Established kidney failure (GFR <15 ml/min/1.73 m2), permanent kidney replacement therapy, or end-stage kidney disease."}, {"id": "wiki20220301en025_100442", "title": "Chronic kidney disease", "score": 0.01031088621633799, "content": "Chronic kidney disease (CKD) is a type of kidney disease in which there is gradual loss of kidney function over a period of months to years. Initially there are generally no symptoms; later, symptoms may include leg swelling, feeling tired, vomiting, loss of appetite, and confusion. Complications include an increased risk of heart disease, high blood pressure, bone disease, and anemia. Causes of chronic kidney disease include diabetes, high blood pressure, glomerulonephritis, and polycystic kidney disease. Risk factors include a family history of chronic kidney disease. Diagnosis is by blood tests to measure the estimated glomerular filtration rate (eGFR), and a urine test to measure albumin. Ultrasound or kidney biopsy may be performed to determine the underlying cause. Several severity-based staging systems are in use."}, {"id": "wiki20220301en023_76154", "title": "Assessment of kidney function", "score": 0.010289908514635904, "content": "The severity of chronic kidney disease (CKD) is described by six stages; the most severe three are defined by the MDRD-eGFR value, and first three also depend on whether there is other evidence of kidney disease (e.g., proteinuria): 0) Normal kidney function – GFR above 90 (mL/min)/(1.73 m2) and no proteinuria 1) CKD1 – GFR above 90 (mL/min)/(1.73 m2) with evidence of kidney damage 2) CKD2 (mild) – GFR of 60 to 89 (mL/min)/(1.73 m2) with evidence of kidney damage 3) CKD3 (moderate) – GFR of 30 to 59 (mL/min)/(1.73 m2) 4) CKD4 (severe) – GFR of 15 to 29 (mL/min)/(1.73 m2) 5) CKD5 kidney failure – GFR less than 15 (mL/min)/(1.73 m2) Some people add CKD5D for those stage 5 patients requiring dialysis; many patients in CKD5 are not yet on dialysis. Note: others add a \"T\" to patients who have had a transplant regardless of stage."}, {"id": "wiki20220301en044_86530", "title": "Kidney transplantation", "score": 0.010073260073260072, "content": "Indications The indication for kidney transplantation is end-stage renal disease (ESRD), regardless of the primary cause. This is defined as a glomerular filtration rate below 15 ml/min/1.73 m2. Common diseases leading to ESRD include renovascular disease, infection, diabetes mellitus, and autoimmune conditions such as chronic glomerulonephritis and lupus; genetic causes include polycystic kidney disease, and a number of inborn errors of metabolism. The commonest 'cause' is idiopathic (i.e. unknown)."}, {"id": "wiki20220301en000_269861", "title": "Kidney", "score": 0.010059691011235956, "content": "Kidney injury and failure Generally, humans can live normally with just one kidney, as one has more functioning renal tissue than is needed to survive. Only when the amount of functioning kidney tissue is greatly diminished does one develop chronic kidney disease. Renal replacement therapy, in the form of dialysis or kidney transplantation, is indicated when the glomerular filtration rate has fallen very low or if the renal dysfunction leads to severe symptoms. Dialysis"}, {"id": "pubmed23n0652_15277", "title": "Continuously evolving management concepts for diabetic CKD and ESRD.", "score": 0.009900990099009901, "content": "During the past 50 years, a global pandemic of kidney failure attributed to diabetes mellitus provoked continuously changing treatment strategies based in the belief that micro- and macrovascular complications of diabetes may be preventable. Both patient and physician have revised, and sometimes reversed drug regimens based on inferences extracted from prospective, controlled, properly populated trials. Illustrating this dilemma is a newly relaxed target for glycosylated hemoglobin (HbA1c) of 7%, introduced because of the greater rate of cardiovascular complications noted when striving to reduce attained HbA1c to < or = 6.5%. Our concept of the natural history of kidney disease in diabetes has repeatedly been modified by a rising mean age of those developing uremia (now 64.5 years). Underscoring the reality that the majority of diabetic kidney failure patients fall within the geriatric age group. An encouraging finding first reported in 2005 and continuing through 2009 is a declining incidence rate of irreversible advanced kidney failure in individuals known to have diabetes. That this \"good news\" results from appropriate renoprotective treatment is as yet unsubstantiated wishful thinking."}, {"id": "wiki20220301en422_14318", "title": "Mesoamerican nephropathy", "score": 0.009882416647327301, "content": "Suspected disease: Estimated GFR (eGFR) < 60 ml/min/1.73m2 Absence of diabetes, hypertension, autoimmune disease, glomerular disease, congenital kidney disease, obstructive kidney disease as a clear cause of kidney disease (these comorbidities may be present but cannot account for disease development) Residing in a hotspot region Proteinuria < 2g/24h or 2g/g urine creatinine Likely disease: All of the above, plus eGFR < 60 ml/min/1.73m2 on 3 month or longer repeat measurement Relative hypokalemia and/or hyperuricemia Kidney ultrasound with loss of corticomedullary differentiation or bilateral small kidney size, without cystic disease or large stone burden Kidney biopsy showing primary tubulointerstitial disease without alternative cause of kidney disease in evidence"}, {"id": "wiki20220301en081_57288", "title": "Secondary hyperparathyroidism", "score": 0.009815449942838478, "content": "Extended Release Calcifediol was recently approved by the FDA as a treatment for secondary hyperparathyroidism (SHPT) in adults with stage 3 or 4 chronic kidney disease (CKD) and low vitamin D blood levels (25-hydroxyvitamin D less than 30 ng/mL). It can help treat SHPT by increasing Vitamin D levels and lowering parathyroid hormone or PTH. It is not indicated for people with stage 5 CKD or on dialysis. In the treatment of secondary hyperparathyroidism due to chronic kidney disease on dialysis calcimimetics do not appear to affect the risk of early death. It does decrease the need for a parathyroidectomy but caused more issues with low blood calcium levels and vomiting. Most people with hyperparathyroidism secondary to chronic kidney disease will improve after renal transplantation, but many will continue to have a degree of residual hyperparathyroidism (tertiary hyperparathyroidism) post-transplant with associated risk of bone loss, etc."}, {"id": "wiki20220301en019_111568", "title": "Glomerular filtration rate", "score": 0.00980392156862745, "content": "The CKD-EPI equation performed better than the MDRD (Modification of Diet in Renal Disease Study) equation, especially at higher GFR, with less bias and greater accuracy. When looking at NHANES (National Health and Nutrition Examination Survey) data, the median estimated GFR was 94.5 mL/min per 1.73 m2 vs. 85.0 mL/min per 1.73 m2, and the prevalence of chronic kidney disease was 11.5% versus 13.1%. Despite its overall superiority to the MDRD equation, the CKD-EPI equations performed poorly in certain populations, including black women, the elderly and the obese, and was less popular among clinicians than the MDRD estimate. The CKD-EPI equation is: where SCr is serum creatinine (mg/dL), k is 0.7 for females and 0.9 for males, a is −0.329 for females and −0.411 for males, min indicates the minimum of SCr/k or 1, and max indicates the maximum of SCr/k or 1."}, {"id": "pubmed23n0773_18932", "title": "Delayed progression to dialysis with early and intensive management of predialysis chronic kidney disease: a case-based approach.", "score": 0.00980392156862745, "content": "In addition to hypertension and diabetes, disorders in mineral metabolism and bone disease (e.g. affecting phosphorus, calcium, parathyroid hormone, and vitamin D) are common complications of chronic kidney disease (CKD) and contribute to morbidity and mortality. Consequently, CKD requires multifactorial treatment to slow CKD progression and avoid end-stage renal disease. CKD progression and treatment outcomes are monitored by measuring the estimated glomerular filtration rate (eGFR), which decreases by 2-12 ml/min/1.73 m(2) per year depending on the stage of CKD and comorbidities, such as diabetes. This paper presents representative case studies illustrating the delay and reversal of CKD progression with comprehensive, individualized treatment regimens, including non-calcium phosphate binders, antihypertensives, lipid-lowering drugs, calcimimetics, and other drugs as required, to treat each component of CKD including CKD-mineral and bone disorder. Four patients are included, with an average age of 70-81 years and CKD stage 3 or 4 accompanied by various comorbidities, most notably diabetes and hypertension. The range of treatment and follow-up durations was 6-7 years. In each case, there was evidence of slowing or prevention of CKD progression, according to eGFR and serum creatinine, regardless of the patient's age or CKD stage. Despite a baseline eGFR of <20 ml/min/1.73 m(2) in 1 female patient, after 6 years of follow-up, her eGFR had stabilized and was maintained at >15 ml/min/1.73 m(2). These observations reinforce the value of early nephrology referral and comprehensive management of CKD and underlying conditions (hypertension and diabetes) beginning at eGFR <60 ml/min/1.73 m(2). "}, {"id": "wiki20220301en019_111565", "title": "Glomerular filtration rate", "score": 0.009708737864077669, "content": "Modification of Diet in Renal Disease (MDRD) formula Another formula for calculating the GFR is the one developed by the Modification of Diet in Renal Disease Study Group. Most laboratories in Australia, and the United Kingdom now calculate and report the estimated GFR along with creatinine measurements and this forms the basis of diagnosis of chronic kidney disease. The adoption of the automatic reporting of MDRD-eGFR has been widely criticised. The most commonly used formula is the \"4-variable MDRD\", which estimates GFR using four variables: serum creatinine, age, ethnicity, and gender. The original MDRD used six variables with the additional variables being the blood urea nitrogen and albumin levels. The equations have been validated in patients with chronic kidney disease; however, both versions underestimate the GFR in healthy patients with GFRs over 60 mL/min. The equations have not been validated in acute renal failure. For creatinine in μmol/L: For creatinine in mg/dL:"}, {"id": "pubmed23n0876_24183", "title": "[Diabetic Kidney Disease 3rd stage - laboratory markers of mineral bone disorder].", "score": 0.009708737864077669, "content": "Diabetes mellitus is the most common cause of end stage kidney disease in the developed countries. Chronic kidney disease-mineral and bone disorder (CKD-MBD) develops with deteriorating of the renal functions. Diabetic patients on hemodialysis are characterized by low bone turnover, higher prevalence of severe and progressive vascular calcification with increased cardiovascular morbidity and mortality. The main factor which causes vascular calcification in patients with diabetic kidney disease (DKD) is poor glycemic control. The recent trial findings describe an inverse correlation between intact parathyroid hormone (iPTH) serum levels and glycemic control in a group of diabetic patients on hemodialysis. The objective of the proposed project is to access the difference of the laboratory markers MBD in the group of patients with 3rd stage DKD depending on glycemic control. We focused on the relationship between the glycemic compensation of diabetes (HbA1c) and iPTH serum level. Ninety one patients with 3rd stage DKD were investigated. There were 46 women (50.5 %) and 45 men (49.5 %), average age of patients was 71.2 ± 7.0 years, with creatinine level 128 ± 30 μmol/l and estimated glomerular filtration (eGF, MDRD) 0.82 ± 0.16 ml/s. There were 60 patients with better glycemic control of diabetes (HbA1c < 7 %) vs 29 patients with poorly controlled diabetes (HbA1c > 7 %). MBD markers were compared in both groups. Patients were further stratified into subgroups based on the serum level of iPTH (iPTH < 35 pg/ml vs iPTH > 35 pg/ml) and MBD markers compared. Statistical analysis was performed using and Mann-Whitney test. We have found the statistical significance in the serum phosphate and proteinuria levels in between groups with HbA1c < 7 % vs patients with HbA1c > 7 %. Diabetics with better glycemic control had significant reduction in serum phosphate level (1.14 ± 0.20 vs 1.23 ± 0.18 mmol/l, p = 0.038) and in 24 hrs proteinuria level (0.56 ± 1.35 vs 1.30 ± 1.61 g/day, p = 0.007). In the group of presumed low bone turnover (iPTH < 35 pg/ml) we have found the trend towards increased serum calcium level (2.49 ± 0.12 vs 2.43 ± 0.10 mmol/l, p = 0.063) and increased HbA1c value (7.5 ± 1.8 vs 6.4 ± 1.6 %, p = 0.023). Our results suggest the closer relationship between glycemic control of diabetes and mineral-bone disorder in earlier stages of DKD. diabetes mellitus type 2 (DM2T) - chronic kidney disease (CKD) - mineral and bone disorder (MBD)."}, {"id": "wiki20220301en090_31250", "title": "Phosphate nephropathy", "score": 0.009650446372438073, "content": "Diagnosis Phosphate nephropathy can be diagnosed via different types of assessment, most of which are also used to detect acute kidney injury and chronic kidney disease. Most phosphate nephropathy incidents are diagnosed weeks or months after taking OSP, due to its clinical silence. For example, these assessments include the measurement of serum phosphorus with an elevation of more than 3 mmol/L, the finding of an elevated serum creatinine level and a decrease in glomerular filtration rate (GFR), urine microscopy for crystallization detection, the image of calcium phosphate crystals deposited through CT scanning, urinalysis, renal biopsy specimens with histochemical staining for calcium phosphate. These assessments are generally carried out within the laboratory environment, in which longer waiting time is required to attain the results."}, {"id": "pubmed23n0800_2883", "title": "Effect of glycemic control on intact parathyroid hormone level in end stage renal disease patients on maintenance hemodialysis.", "score": 0.009615384615384616, "content": "We evaluated the relationship between iPTH levels and glycemic control in patients with diabetes and end stage renal disease (ESRD) on maintenance hemodialysis (MHD). The study included 98 subjects with ESRD and type 2 diabetes aged 30-75 years who were on MHD. These were divided into two groups--patients with HbA1c >7.0 (53 mmol/mol) (poor glycemic control group) and patients with HbA1c <7.0 (53 mmol/mol) (good glycemic control group). All patients had been on regular bicarbonate haemodialysis for more than 6 months using polysulfone membrane dialyzer; 4 h per episode 3 times/week, with a dialysis fluid of 3.0 mEq/L of calcium concentration. 1-α-(OH)D3 and calcium carbonate were used routinely in all patients. The contribution of each relevant biological parameter to serum iPTH level was assessed using multiple regression test. Poor glycemic control was associated with reduced serum iPTH level and good glycemic control with higher serum iPTH. The serum HbA1c level was significantly correlated with the serum iPTH level (p=0.0003). Glycemic control is a significant determinant of iPTH level in diabetic ESRD patients on MHD."}, {"id": "wiki20220301en019_111559", "title": "Glomerular filtration rate", "score": 0.009523809523809525, "content": "A common mistake made when just looking at serum creatinine is the failure to account for muscle mass. Hence, an older woman with a serum creatinine of 1.4 mg/dL may actually have a moderately severe chronic kidney disease, whereas a young muscular male can have a normal level of renal function at this serum creatinine level. Creatinine-based equations should be used with caution in cachectic patients and patients with cirrhosis. They often have very low muscle mass and a much lower creatinine excretion rate than predicted by the equations below, such that a cirrhotic patient with a serum creatinine of 0.9 mg/dL may have a moderately severe degree of chronic kidney disease. Estimated GFR (eGFR) is now recommended by clinical practice guidelines and regulatory agencies for routine evaluation of GFR whereas measured GFR (mGFR) is recommended as a confirmatory test when more accurate assessment is required."}, {"id": "pubmed23n0327_22144", "title": "[Current therapy of chronic renal failure].", "score": 0.009523809523809525, "content": "The course of chronic renal failure is generally progressive and mediated by several factors that operate in combination. Several extrarenal events which may cause transient or permanent deterioration of renal function, are important, because their correction may slow the progression of renal disease e.g. volume disorders, infection, nephrotoxic agents. In progression of chronic renal disease leading factors are hypertension, proteinuria and high protein/phosphorus intake. Number of evidence suggests that ameliorating hypertension, reducing proteinuria slow the progression of chronic renal failure. Clinical studies in diabetic nephropathy demonstrated that the renoprotective effect of ACE inhibitors was independent of their effect of systemic blood pressure. In ESRD patients access for renal replacement therapy should be obtained as early as possible. An A-V fistula may take several weeks to mature especially in diabetic or elderly patients. Early dialysis has been advocated in diabetic patients. In general, patients can start ESRD therapy when residual kidney function drops to 5-10% of normal value. High quality of dialysis should be provided to the uremic patient with respect of successful renal transplantation."}, {"id": "pubmed23n0595_6553", "title": "Survival of patients on maintenance haemodialysis over a twenty-year period.", "score": 0.009433962264150943, "content": "Patient survival is a key index of the overall adequacy of treatment in most chronic diseases. Analyses of survival of patients undergoing haemodialysis is very important, as it may offer clues and ideas for prolonging survival of patients with end-stage renal disease (ESRD). The aims of this study were to describe the characteristics of the patients on maintenance haemodialysis therapy over a period of 20 years, to determine the survival rate of these patients according to ages at the onset of haemodialysis, the primary renal diseases, and the cause of death, and to determine the survival rate at five, ten, fifteen and twenty years of haemodialysis treatment at our centre. The charts of 518 unselected patients, 282 male and 236 female, treated with maintenance haemodialysis therapy in a period of 20 years (1985-2005) were reviewed. At the time of evaluation, 164 patients were currently being treated, and 354 patients overall had been diseased. Statistical analysis was performed to evaluate the relationship between survival and patient characteristics such as age, gender, primary renal disease, and age at dialysis onset. Actual survival rates were determined by the Kaplan-Meier method. The survival rate of our patients treated with maintenance haemodialysis was 60% at 5 years, 37% at 10 years, 25% at 15 years and 9% at 20 years. Female patient survival was superior to male. Patients aged under 40 at the start of dialysis had a better survival probability compared to older patients. Patients with diabetes mellitus and nephroangiosclerosis, had a lower survival rate compared to patients with glomerulonephritis and with adult dominant polycystic kidney disease. Cardiac death was the most common cause of death in patients involved in the study. About 52% of the patients died from cardiovascular disease. Death is the most severe consequence of inadequate dialysis and can be used as an index of the adequacy of the dialysis therapy. Treatment factors that may improve outcomes include an early start of dialysis therapy, a high dose of dialysis (Kt/V over 1.2), correction of anemia, adequate protein and caloric intake, control of calcium and phosphate metabolism, and the use of biocompatible dialyzers."}, {"id": "pubmed23n0491_13883", "title": "Early referral to a nephrologist is associated with better outcomes in type 2 diabetes patients with end-stage renal disease.", "score": 0.009345794392523364, "content": "End-stage renal disease (ESRD) requiring renal replacement therapy (RRT) is a late complication of type 2 diabetes. The correlation between pre-ESRD medical care and outcome has been rarely studied in France. Community-based study of case-incIdent ESRD patients. Medical care practices were described retrospectively when starting RRT. Medical status, mortality, morbIdity, and quality-of-life were recorded prospectively. One hundred and fourty-eight ESRD patients with type 2 diabetes were included. Factors independently correlated with mortality within 3 Months of RRT onset were presence of physical impairment of ambulation at onset of RRT [odd ratio (OR): 5, (95%CI: 1.9-13.3)], and starting RRT in life-threatening circumstances [OR: 3.6, (95%CI: 1.2-10.7)]. Factors independently correlated with \"poor outcome\" 1 Year after the onset of RRT were BMI less than 20 kg/m2 [OR: 13.4, (95%CI: 1.5-120.2)] and presence of 2 [OR: 2.7, (95%CI: 0.9-8.4)], or 3 or more comorbId conditions [OR: 4, (95% CI: 1.4-11)]. Three Months after the first RRT session, survival was 16.4% better for patients who had had regular nephrological care versus none, and 9.1% better for those who had had late nephrological care versus none. Type 2 diabetes patients starting RRT in an emergency setting had had significant less regular nephrological care. Length of their first hospital stay was significantly longer. They were more likely to have lower resIdual renal function, gastrointestinal symptoms, lower serum albumin, lower hematocrit, lower serum calcium, and higher serum phosphorus. During the course of chronic renal failure in type 2 diabetes patients, early implementation of nephrological well-established guIdelines is associated with better outcome after starting RRT."}, {"id": "wiki20220301en000_269835", "title": "Kidney", "score": 0.009259259259259259, "content": "Chronic kidney disease (CKD) has been recognized as a leading public health problem worldwide. The global estimated prevalence of CKD is 13.4%, and patients with kidney failure needing renal replacement therapy are estimated between 5 and 7 million. Procedures used in the management of kidney disease include chemical and microscopic examination of the urine (urinalysis), measurement of kidney function by calculating the estimated glomerular filtration rate (eGFR) using the serum creatinine; and kidney biopsy and CT scan to evaluate for abnormal anatomy. Dialysis and kidney transplantation are used to treat kidney failure; one (or both sequentially) of these are almost always used when renal function drops below 15%. Nephrectomy is frequently used to cure renal cell carcinoma."}, {"id": "pubmed23n0423_3114", "title": "The current state of chronic dialysis treatment in Japan (as of December 31, 2000).", "score": 0.009259259259259259, "content": "The annual statistical survey conducted at the end of 2000 by the Japanese Society for Dialysis Therapy collected responses from 3358 (99.94%) of 3360 institutions. Japan's total dialysis patient population at the end of the year 2000, as identified by this survey, was 206,134, an increase of 8921 (4.5%) over 1999. This translates to 1624.1 patients per million population. The annual crude mortality rate was 9.4% for the period starting at the end of the year 1999 and ending at the end of the year 2000. The mean patient age at the initiation of dialysis treatment was 63.8 (+/- 13.9; +/- SD) years; the mean age of the overall dialysis patient population was 61.2 years (+/- 13.3). Both these mean ages, which had been increasing since 1983, again continued to increase. Among the primary diagnosis, the prevalence of diabetic nephropathy had continued to increase again since 1999, to 36.6%, whereas that of chronic glomerulonephritis had continued to decline, down to 32.5%, during the same one-year period since the 1999 survey. The 2000 years-end survey incorporated the following additional variables for the first time: usage of oral antihypertensives, pre- and post-dialysis systolic and diastolic blood pressures, serum HDL cholesterol level, types and dosage of oral Vitamin D analogs administered, dosage of oral calcium carbonate administered, history of intervention for peripheral vascular disease (bypass surgery, synthetic graft replacement, stenting), history of coronary artery bypass grafting (CABG), history of percutaneous transluminal coronary angioplasty (PTCA), whether stenting had been previously performed for the treatment of ischemic heart disease, number of cigarettes smoked, the type of vascular access used at the initiation of dialysis, and the year and month the vascular access was created. The survey results indicate that 60.9% of the total dialysis patient population was using oral antihypertensives. The patients' mean serum HDL cholesterol level was 47.65 +/- 18.47 mg/dL, showing positive correlation with serum albumin level and reverse correlation with body mass index. 1.6% of all dialysis patients had previously undergone amputation, and 0.7% had a history of bypass surgery for peripheral vascular disorder. 4.5% of hemodialysis patients had a history of cardiac infarction, 1.6% had previously undergone CABG, and 2.8%, PTCA. At the time the survey was conducted, 2.0% of all dialysis patients were undergoing oral Vitamin D analog pulse therapy, and 6% were undergoing intravenous Vitamin D analog pulse therapy. A history of amputation, myocardial infarction, cerebral infarction, and cerebral bleeding were identified as high-risk factors of vital prognosis. Additionally, high mortality risk was associated with the following: glutamic-pyruvic transaminase levels exceeding 20 IU/L; positive HCV antibody status; comorbid conditions such as hepatic cell carcinoma and liver cirrhosis; platelet counts below 100,000/mL or equal to or greater than 200,000/mL; C-reactive protein levels of 0.2 mg/dL and higher, leukocyte counts of less than 3000/mL or equal to or greater than 8000/mL; and body mass index of below 22 kg/m2, as well as total serum cholesterol levels of below 160 mg/dL or equal to or greater than 260 mg/dL."}, {"id": "pubmed23n0928_6495", "title": "Clinical features of patients treated by peritoneal dialysis for over a decade.", "score": 0.009174311926605505, "content": "Peritoneal dialysis (PD) is well-established as renal replacement therapy in end stage renal disease and has survival rates similar or better than hemodialysis (HD) for the initial years on dialysis therapy. However retention rate is lower due to higher technique failure rates than in HD and few patients stay on PD for more than 10 years (PDShigella sonnei Bacteremia Presenting with Profound Hepatic Dysfunction.", "score": 0.014681295715778475, "content": "Worldwide, Shigellosis is a significant public health issue, associated with nearly one million deaths annually. About half a million cases of 15%) See Table 58-1 Drugs Rifampicin Probenecid Inherited disorders Dubin-Johnson syndrome Rotor syndrome 1. Viral serologies Hepatitis A IgM Hepatitis B surface antigen and core antibody (IgM) Hepatitis C RNA 2. Toxicology screen Acetaminophen level 3. Ceruloplasmin (if patient < 40 years of age) 4. ANA, SMA, SPEP Inherited disorders Gilbert's syndrome Crigler-Najjar syndromes Hemolytic disorders Ineffective erythropoiesis Bilirubin and other liver tests elevated Hepatocellular pattern: ALT/AST elevated out of proportion to alkaline phosphatase See Table 58-2 Cholestatic pattern: Alkaline phosphatase out of proportion ALT/AST See Table 58-3 Dilated ducts Extrahepatic cholestasis CT/MRCP/ERCP Liver biopsy Liver biopsy MRCP/Liver biopsy Results negativeResults negative Additional virologic testing CMV DNA, EBV capsid antigen Hepatitis D antibody (if"}, {"id": "InternalMed_Harrison_23380", "title": "InternalMed_Harrison", "score": 0.011057042443903757, "content": "FAMILIAL DEFECTS IN HEPATIC EXCRETORY FUNCTION Dubin-Johnson Syndrome (DJS) This benign, relatively rare disorder is characterized by low-grade, predominantly conjugated hyperbilirubinemia (Table 359-2). Total bilirubin concentrations are typically between 34 and 85 μmol/L (2 and 5 mg/dL) but on occasion can be in the normal range or as high as 340–430 μmol/L (20–25 mg/dL) and can fluctuate widely in any given patient. The degree of hyperbiliru-2003 binemia may be increased by intercurrent illness, oral contraceptive use, and pregnancy. Because the hyperbilirubinemia is due to a predominant rise in conjugated bilirubin, bilirubinuria is characteristically present. Aside from elevated serum bilirubin levels, other routine laboratory tests are normal. Physical examination is usually normal except for jaundice, although an occasional patient may have hepatosplenomegaly."}, {"id": "InternalMed_Harrison_23303", "title": "InternalMed_Harrison", "score": 0.010977056962025316, "content": "Review drug list Hepatitis C antibody Hepatitis B surface Ag Iron, TIBC, ferritin ANA, SPEP Ceruloplasmin (if patient < 40) Ultrasound to look for fatty liver <15% Direct Gilbert’s syndrome Isolated elevation of the bilirubin Hepatocellular pattern (see Table 358-1) W/U negative W/U negative W/U negative Dilated ducts W/U positive Isolated elevation of the alkaline phosphatase Cholestatic pattern (see Table 358-1) Consider liver biopsy ERCP/Liver Bx CT/MRCP/ERCP Liver Bx Ducts not dilated Dilated ducts AMA positive AMA negative Alkaline phos. of liver origin Alkaline phos. of bone origin Bone Eval Ducts not dilated and/or AMA positive MRCP Evaluation for hemolysis Dubin-Johnson or Rotor syndrome Hemolysis Fractionate bilirubin >15% Direct Check AMA Review drugs Ultrasound Liver Tests Fractionate the alkaline phosphatase or check GGT or 5' nucleotidase to assess origin of alkaline phosphatase Ultrasound Review drug list Check AMA Liver biopsy R/O Celiac disease Consider other"}, {"id": "pubmed23n0283_20435", "title": "[Broad beans as a cause of acute hemolytic anemia].", "score": 0.010755391663873059, "content": "A previously healthy 17-year-old Greek boy suddenly developed jaundice of sclerae and skin. In addition, physical examination revealed a pale appearance. He also reported feeling tired and weak. The haemoglobin level was 9.6 g/dl, lactate dehydrogenase activity 335 U/l, bilirubin concentration 3.2 mg/dl (direct bilirubin 0.7 mg/dl, indirect bilirubin 2.5 mg/dl), haptoglobin concentration 48.8 mg/dl. As haemolytic anaemia was assumed, direct questioning elicited the fact that the patient had, for the first time in his life, eaten 300 g of broad beans (Vicia faba) on each of two days, namely 3 and 2 days before the appearance of jaundice. Absence of glucose-6-phosphate dehydrogenase activity in the red blood corpuscles confirmed the diagnosis of favism. On symptomatic treatment both the enzyme activities and the bilirubin level fell to normal within one week, and the haemoglobin level was 15.7 g/dl after 4 weeks."}, {"id": "wiki20220301en068_42784", "title": "Dubin–Johnson syndrome", "score": 0.010493428140486965, "content": "Dubin–Johnson syndrome is a rare, autosomal recessive, benign disorder that causes an isolated increase of conjugated bilirubin in the serum. Classically, the condition causes a black liver due to the deposition of a pigment similar to melanin. This condition is associated with a defect in the ability of hepatocytes to secrete conjugated bilirubin into the bile, and is similar to Rotor syndrome. It is usually asymptomatic, but may be diagnosed in early infancy based on laboratory tests. No treatment is usually needed. Signs and symptoms Around 80 to 99% of people with Dubin-Johnson syndrome have jaundice, abnormal urinary color, biliary tract abnormality, and conjugated bilirubinemia. Around 30 to 79% of people with the disorder have abnormality of the gastric mucosa. Other rare symptoms include fever and fatigue."}, {"id": "wiki20220301en015_7346", "title": "Gilbert's syndrome", "score": 0.010458002645502645, "content": "Dubin–Johnson syndrome and Rotor syndrome are rarer autosomal recessive disorders characterized by an increase of conjugated bilirubin. Viral hepatitis associated with increase of conjugated bilirubin can be excluded by negative blood samples for antigens specific to the different hepatitis viruses. Cholestasis can be excluded by normal levels of bile acids in plasma, the absence of lactate dehydrogenase, low levels of conjugated bilirubin, and ultrasound scan of the bile ducts."}, {"id": "InternalMed_Harrison_3253", "title": "InternalMed_Harrison", "score": 0.010410385556698471, "content": "Laboratory Tests A battery of tests are helpful in the initial evaluation of a patient with unexplained jaundice. These include total and direct serum bilirubin measurement with fractionation; determination of serum aminotransferase, alkaline phosphatase, and albumin concentrations; and prothrombin time tests. Enzyme tests (alanine aminotransferase [ALT], aspartate aminotransferase [AST], and alkaline phosphatase [ALP]) are helpful in differentiating between a hepatocellular process and a cholestatic process (Table 358-1; Fig. 58-1)—a critical step in determining what additional workup is indicated. Patients with a hepatocellular process generally have a rise in the aminotransferases that is disproportionate to that in ALP, whereas patients with a cholestatic process have a rise in ALP that is disproportionate to that of the aminotransferases. The serum bilirubin can be prominently elevated in both hepatocellular and cholestatic conditions and therefore is not necessarily helpful in"}, {"id": "wiki20220301en011_17419", "title": "Liver function tests", "score": 0.01028059184217542, "content": "The increase in predominantly unconjugated bilirubin is due to overproduction, reduced hepatic uptake of the unconjugated bilirubin and reduced conjugation of bilirubin. Overproduction can be due to the reabsorption of a haematoma and ineffective erythropoiesis leading to increased red blood cell destruction. Gilbert's syndrome and Crigler–Najjar syndrome have defects in the UDP-glucuronyl-transferase enzyme, affecting bilirubin conjugation. The degree of rise in conjugated bilirubin is directly proportional to the degree of hepatocyte injury. Viral hepatitis can also cause the rise in conjugated bilirubin. In parenchymal liver disease and incomplete extrahepatic obstruction, the rise in conjugated bilirubin is less than the complete common bile duct obstruction due to malignant causes. In Dubin–Johnson syndrome, a mutation in multiple drug-resistance protein 2 (MRP2) causes a rise in conjugated bilirubin."}, {"id": "article-20746_8", "title": "Dubin-Johnson Syndrome -- History and Physical", "score": 0.010245976758678837, "content": "Most patients with DJS are often young adults and are asymptomatic. Hyperbilirubinemia is found as an incidental finding while undergoing routine testing or being tested for some other unrelated problem. They may rarely present with mild icterus, weakness, and/or upper abdominal pain. Pruritus is not a symptom of DJS since serum total bile acid levels are normal. In women, the condition may be subclinical and discovered because of hyperbilirubinemia or obvious jaundice once started on oral contraceptives or becoming pregnant, at which time development of jaundice may lead to the establishment of the diagnosis. Except for mild icterus, physical examination results are within normal limits. [7] [4]"}, {"id": "article-18282_6", "title": "Impaired Bilirubin Conjugation -- History and Physical", "score": 0.010200429491768075, "content": "Gilbert syndrome presents in patients as a mild, intermittent, and prolonged unconjugated hyperbilirubinemia without hemolysis. It also presents with normal LDH and haptoglobin  levels and reticulocyte counts in addition to normal liver function tests. Liver ultrasound is normal . In fact, Gilbert syndrome is most commonly asymptomatic, and it is often found incidentally on routine blood testing conducted for other reasons. Upon physical exam, this syndrome can manifest as jaundice. Nonspecific symptoms of abdominal cramps, fatigue, and malaise may also present with the disease. A thorough history should be taken to ascertain whether the patient is taking drugs that are metabolized through glucuronidation such as tolbutamide, rifamycin, HIV protease inhibitors, gemfibrozil, and statins, which could contribute to drug toxicity."}, {"id": "wiki20220301en003_62168", "title": "Jaundice", "score": 0.009894093904595218, "content": "Laboratory results for liver panels are frequently compared by the magnitude of their differences, not the pure number, as well as by their ratios. The AST:ALT ratio can be a good indicator of whether the disorder is alcoholic liver damage (above 10), some other form of liver damage (above 1), or hepatitis (less than 1). Bilirubin levels greater than 10 times normal could indicate neoplastic or intrahepatic cholestasis. Levels lower than this tend to indicate hepatocellular causes. AST levels greater than 15 times normal tend to indicate acute hepatocellular damage. Less than this tend to indicate obstructive causes. ALP levels greater than 5 times normal tend to indicate obstruction, while levels greater than 10 times normal can indicate drug (toxin) induced cholestatic hepatitis or cytomegalovirus infection. Both of these conditions can also have ALT and AST greater than 20 times normal. GGT levels greater than 10 times normal typically indicate cholestasis. Levels 5–10"}, {"id": "pubmed23n0706_7706", "title": "Severe jaundice due to coexistence of Dubin-Johnson syndrome and hereditary spherocytosis: a case report.", "score": 0.009855514010063241, "content": "Dubin-Johnson syndrome is a chronic, benign, intermittent jaundice, mostly of conjugated hyperbilirubinemia. The level of bilirubin is not expected to be more than 20 mg/dl in this syndrome. In this article, we report a patient who was evaluated for hyperbilirubinemia and liver function test abnormalities and diagnosed with Dubin-Johnson syndrome coexisting with hereditary spherocytosis. We suggest that other diseases should be investigated if patients with Dubin-Johnson syndrome present with severe hyperbilirubinemia. Dubin-Johnson syndrome accompanied by hemolytic diseases might also have high coproporphyrin levels (as in Rotor's syndrome) than expected in pure Dubin-Johnson syndrome."}, {"id": "pubmed23n0590_4739", "title": "[A young man with acute generalised jaundice and intermittent epigastric pain].", "score": 0.00980392156862745, "content": "A 24-year-old Iraqi was admitted to our hospital with acute generalised jaundice and intermittent epigastric pain. His family doctor suspected a viral hepatitis. Two days prior admission the patient had consumed large quantities of alcohol and had subsequently taken analgetic dosages of paracetamol and acetylsalicylic acid. Besides an otherwise inconspicuous physical examination the laboratory results revealed a distinct hemolysis with macrocytic, hyperchromic anaemia and negative Coombs-test. Indirect bilirubin was initially 25.2 mg/dl, LDH 2367 U/l and reticulocytes 4.4 %; haptoglobin and transferrine levels were correspondingly low. A hemoglobinopathies was excluded by hemoglobin-electrophoresis and a blood-smear. DIAGNOSIS, TREATMENT AND FURTHER COURSE: A glucose-6-phosphate dehydrogenase deficiency (G6PDD) was suspected and subsequently confirmed within three days. Acetylsalicylic acid, the most probable trigger, and other possible triggers like ciprofloxacin, metamizole, and cotrimoxazole were avoided; the jaundice faded rapidly, and the laboratory-parameters almost normalized. In patients with acute jaundice, abdominal pain, and signs of hemolysis collection of accurate anamnestic information is essential. In case of a young male with positive family-history, applicable ethnical origin, and intake of potential oxidative stressors a G6PDD should be suspected and result in requesting specific tests. Analgetic therapy with metamizole must be strictly avoided."}, {"id": "pubmed23n0581_16754", "title": "[Fulminate liver failure in a 39-year-old female patient with leukocytosis, unclear fever, and arthralgic pain].", "score": 0.009708737864077669, "content": "Fulminate liver insufficiency can have many causes and is a challenge for differential diagnosis. A 39-year-old woman was admitted because of a nonitching macular-papular exanthema on both thighs with spreading to the trunk. In addition, the patient complained of dysphagia, symmetrical arthralgias, myalgias, fever of 38 degrees C, and night sweats. An outpatient treatment with nonsteroidal antirheumatics, antihistamines and penicillin was started for 3 days before admission. On admission, a neutrophilic leukocytosis (23.6 Gpt/l), an increase in C-reactive protein (185 mg/l), and a ferritin level of 1,740 microg/l were found. Liver enzymes were increased (alanine aminotransferase 1.03 micromol/l.s, aspartate aminotransferase 1.06 micromol/l.s, gamma-glutamyltransferase 2.73 micromol/l.s, and lactate dehydrogenase 12.48 micromol/l.s). Sonographic examination showed a mild hepatosplenomegaly, but otherwise normal findings. X-rays of the lungs, hands, and ankles were normal. An echocardiography was within normal limits. Extensive serologic investigations including assays for hepatitides A, B and C as well as repeated blood cultures were negative. Antibiotic therapy was continued without any improvement. In addition, acetaminophen (4 x 1,000 mg/day) and ibuprofen (3 x 600 mg/day) were given. Liver function worsened and an icterus developed. The patient was transferred to the authors' university hospital. Because of the clinical findings of fever episodes, a typical macular exanthema, lymphadenopathy, hepatosplenomegaly, arthralgias, myalgias, dysphagia, and the presence of neutrophilic leukocytosis, fever, an increase in ferritin, but negative serologic titers and no bacteremia, a working diagnosis of Still's disease was made. The patient was treated with high-dose methylprednisone (250 mg/day for 3 days, then 100 mg/day). Liver biopsy revealed subacute hepatitis with necrosis and accompanying cholangitis. The prednisone therapy induced a fast remission and improvement of liver function, liver transplantation was not necessary. The patient is, 16 months after the incident, without symptoms under prednisone 3 mg/day, and the liver function is normal. The etiology of Still's disease is unknown and the disease is characterized by fever episodes, a typical macular-papular exanthema, lymphadenopathy, hepatosplenomegaly, and arthralgias. A mild to moderate increase in liver enzymes is often found as part of this disease. Rarely, a fulminate liver failure has been described, particularly in the presence of co-administration of nonsteroidal antirheumatics or acetaminophen. Still's disease must be considered as part of the differential diagnosis of acute liver failure, because an early diagnosis and consequent therapy with prednisone may prevent the need for liver transplantation."}, {"id": "InternalMed_Harrison_24286", "title": "InternalMed_Harrison", "score": 0.00969744622995397, "content": "exists. Serum bilirubin levels ≥342.0 μmol/L (20 mg/dL) should suggest the possibility of neoplastic obstruction. The serum alkaline phosphatase level is almost always elevated in biliary obstruction. A rise in alkaline phosphatase often precedes clinical jaundice and may be the only abnormality in routine liver function tests. There may be a twoto tenfold elevation of serum aminotransferases, especially in association with acute obstruction. Following relief of the obstructing process, serum aminotransferase elevations usually return rapidly to normal, while the serum bilirubin level may take 1–2 weeks to return to normal. The alkaline phosphatase level usually falls slowly, lagging behind the decrease in serum bilirubin."}, {"id": "wiki20220301en068_42787", "title": "Dubin–Johnson syndrome", "score": 0.009625409591897527, "content": "In post mortem autopsy, the liver will have a dark pink or black appearance due to pigment accumulation. Plentiful canalicular multiple drug-resistant protein causes bilirubin transfer to bile canaliculi. An isoform of this protein is localized to the apical hepatocyte membrane, allowing transport of glucuronide and glutathione conjugates back into the blood. High levels of gamma-glutamyl transferase (GGT) help in diagnosing pathologies involving biliary obstruction. Differentiation from Rotor syndrome Dubin–Johnson syndrome is similar to Rotor syndrome, but can be differentiated by:"}, {"id": "wiki20220301en015_7343", "title": "Gilbert's syndrome", "score": 0.009615384615384616, "content": "Because of its effects on drug and bilirubin breakdown and because of its genetic inheritance, Gilbert's syndrome can be classed as a minor inborn error of metabolism. Diagnosis People with GS predominantly have elevated unconjugated bilirubin, while conjugated bilirubin is usually within the normal range or is less than 20% of the total. Levels of bilirubin in GS patients are reported to be from 20 μM to 90 μM (1.2 to 5.3 mg/dl) compared to the normal amount of < 20 μM. GS patients have a ratio of unconjugated/conjugated (indirect/direct) bilirubin commensurately higher than those without GS. The level of total bilirubin is often further increased if the blood sample is taken after fasting for two days, and a fast can, therefore, be useful diagnostically. A further conceptual step that is rarely necessary or appropriate is to give a low dose of phenobarbital: the bilirubin will decrease substantially."}, {"id": "pubmed23n0702_20431", "title": "[Acute pancreatitis and acalculous cholecystitis associated with viral hepatitis A].", "score": 0.009615384615384616, "content": "We report the case of a 14 year-old male from Lima. He is a student with a history of bronchial asthma since age 4 receives conditional salbutamol, corticosteroids used for asthma attacks (a crisis in 2010, 1 month ago) Refuses surgery or transfusions. He presented with a two weeks for abdominal pain, nausea, fever, and jaundice. Epigastric pain is colicky and radiated back to righ upper quadrant, refers in addition to nausea and fever, for ten days notice jaundice of skin and sclera. On examen he was lucid, with jaundice of skin and mucous membranes. There was no palpable lymph nodes, abdomen with bowel sounds, soft, depressible, liver span of 15cm, positive Murphy, no peritonitis. The laboratory findings showed hemoglobin 13gr, MCV 90, platelets 461.000/mm3, WBC 4320/mm, lymphocytes 1700 (39%). total bilirubin: 8.8, B Direct: 7.6, ALT (alanine aminotransferase): 3016, AST (aspartate aminotransferase): 984, alkaline phosphatase: 250, albumin: 3.34gr%, globulin: 2.8, amylase: 589 (high serum amylase), TP: 17, INR: 1.6, VHA IgM positive. 89 mg glucose, urea 19 mg%, creatinine 0.5 mg Hemoglobin 13gr, MCV 90 Platelet 461000/mm3, WBC 4320/mm, Lymphocytes 1700 (39%). The nuclear magnetic resonance showed hepatomegaly associated with thickening of gallbladder wall without stones up to 11mm inside. No bile duct dilatation, bile duct 4mm, pancreas increased prevalence of body size. Mild splenomegaly and free fluid in the space of Morrison and right flank. Abdominal ultrasound revealed a gallbladder wall thickness (11mm), without stones in his light. Pancreas to increase volume with peripancreatic fluid free perivesicular with a volume of 430 cc. Findings consistent with acute acalculous cholecystitis and acute pancreatitis. CT-scan showed enlarged pancreas with predominance of body and tail with peripancreatic edema; the gallbladder was thickening. We report this case because the extrahepatic manifestations of viral hepatitis A infection are uncommon, specially the associated with acute acalculous cholecystitis and acute pancreatitis simultaneous."}]}}}} {"correct_option": 2, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": true, "char_ranges": [[0, 292]], "word_ranges": [[0, 48]], "text": "In this question, we are presented with a patient with systemic lupus erythematosus presenting with autoimmune hemolytic anemia. For this, the best option would be to request reticulocytes, however, as it is not among the options, we should request a Coombs' test, so the correct answer is 2."}, "3": {"exist": true, "char_ranges": [[293, 524]], "word_ranges": [[48, 93]], "text": "Although they want to confuse us with a MCV 108, so that we think of a megaloblastic anemia, the MCV is not excessively high, the onset of anemia in our patient has been rapid and would not fit with SLE or the form of presentation."}, "4": {"exist": true, "char_ranges": [[525, 599]], "word_ranges": [[93, 104]], "text": "Antinuclear antibodies are used for diagnosis, but not for monitoring SLE."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "In this question, we are presented with a patient with systemic lupus erythematosus presenting with autoimmune hemolytic anemia. For this, the best option would be to request reticulocytes, however, as it is not among the options, we should request a Coombs' test, so the correct answer is 2. Although they want to confuse us with a MCV 108, so that we think of a megaloblastic anemia, the MCV is not excessively high, the onset of anemia in our patient has been rapid and would not fit with SLE or the form of presentation. Antinuclear antibodies are used for diagnosis, but not for monitoring SLE.", "full_answer_no_ref": "In this question, we are presented with a patient with systemic lupus erythematosus presenting with autoimmune hemolytic anemia. For this, the best option would be to request reticulocytes, however, as it is not among the options, we should request a Coombs' test, so [HIDDEN]. Although they want to confuse us with a MCV 108, so that we think of a megaloblastic anemia, the MCV is not excessively high, the onset of anemia in our patient has been rapid and would not fit with SLE or the form of presentation. Antinuclear antibodies are used for diagnosis, but not for monitoring SLE.", "full_question": "26-year-old woman diagnosed with systemic lupus erythematosus, on treatment with hydroxychloroquine, who consults for a feeling of generalized weakness that has progressively set in over the last 15 days. The physical examination reveals cutaneous pallor and the CBC shows Hb 7.4 g/dL, Hct 31%, MCV 108. Which of the following determinations will be most useful in deciding the course of action?", "id": 597, "lang": "en", "options": {"1": "Haptoglobin.", "2": "Coombs test.", "3": "Vitamin B12.", "4": "Antinuclear antibodies.", "5": NaN}, "question_id_specific": 180, "type": "RHEUMATOLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1098_18165", "title": "Immune-mediated Coombs negative intravascular haemolysis in systemic lupus erythematosus (SLE).", "score": 0.01906318082788671, "content": "A 27-year-old woman presented with a history of excessive hair loss, loss of appetite, loss of weight, amenorrhoea and loss of axillary and pubic hair for 6 months followed by fever and vomiting for 5 months and abdominal pain for 1 month. During the course of her illness, the patient developed intravascular haemolysis as evidenced by a drop in haemoglobin, indirect hyperbilirubinaemia, raised lactate dehydrogenase (LDH) and haemoglobinuria. Examination revealed severe pallor, mild icterus, elevated jugular venous pressure, generalised lymphadenopathy and hyperpigmentation. Investigations revealed severe anaemia, indirect hyperbilirubinaemia, raised LDH and negative Coombs test. Antinuclear antibody and anti-dsDNA, anti-Sm and anti-SS-A/Ro antibodies were positive and complement C3 was low. The patient was diagnosed to have systemic lupus erythematosus and immune-mediated intravascular haemolysis and was treated with prednisolone and hydroxychloroquine. Haemolysis resolved following steroid therapy, and during follow-up, there were no further episodes of haemolysis."}, {"id": "pubmed23n0060_1206", "title": "[An elderly case of systemic lupus erythematosus associated with herpes zoster, anemia, and hemiparesis].", "score": 0.018637309292649097, "content": "An elderly case of systemic lupus erythematosus (SLE) with suspected hemolytic anemia was experienced. A 70 year-old female was admitted to our hospital on December 31 with complaints of herpetic eruption. She complained of arthralgia since 3 month prior to her admission. The positive findings on examination were skin eruption in the left chest, a systolic heart murmur and a palpable elastic hard liver. Laboratory data showed raised erythrocyte sedimentation rate of 149 mm per hour, decreased Hb (10.1 g/dl), decreased hematocrit (30.0%), increased reticulocytes (33%1000), decreased thrombocytes (73,000/mm3), increased gamma-globulin (33%) and positive rheumatoid factor. During admission, she developed anemia. A stool test for occult blood was negative. The haptoglobin was 38.8 mg/dl and bone marrow aspiration showed increased erythropoiesis, suggesting features of immune hemolytic anemia, except she was negative on Coomb'test. Eye fundi were similar to case of typical bleeding observed in SLE. Concerning immunological findings, the antinuclear factor was x 1280 and the anti-dsDNA antibody was x 80, on which a diagnosis of SLE was based. She experienced numbness of the left arm and developed left hemiparesis 2 days later. Therapy with 15 mg/day prednisone obtained a good response and anemia, abnormal immunological findings and hemiparesis disappeared."}, {"id": "pubmed23n1102_1154", "title": "Late-Onset Systemic Lupus Erythematosus Associated with Autoimmune Hemolytic Anemia and Sixth Cranial Nerve Palsy.", "score": 0.018543956043956044, "content": "BACKGROUND Patients with late-onset systemic lupus erythematosus (SLE) do not present with typical SLE symptoms or serology, and this can lead to a major delay in diagnosis. We report a complex case of an older woman who developed autoimmune hemolytic anemia and sixth cranial nerve palsy that posed considerable challenges in diagnosing late-onset SLE. CASE REPORT A 78-year-old Japanese woman presented with polyarthritis associated with generalized fatigue for 2 months, who later developed diplopia. Physical examination revealed conjunctival pallor, polyarthritis, and subsequent development of sixth cranial nerve palsy. Laboratory data revealed a decreased white blood cell count; macrocytic anemia; elevated levels of lactate dehydrogenase, indirect bilirubin, and erythrocyte sedimentation rate; hypocomplementemia; positive Coombs test; antinuclear antibodies (ANAs, 1: 40); and positive anti-double-strand DNA antibodies. Lymphoma, cerebral venous sinus thrombosis, and varicella-zoster virus infection were unlikely based on head computed tomography, brain magnetic resonance imaging, and cerebrospinal fluid analysis. She was diagnosed with late-onset SLE associated with autoimmune hemolytic anemia and sixth cranial nerve palsy. The patient was successfully treated with prednisone and hydroxychloroquine. CONCLUSIONS The difficulty in diagnosing late-onset SLE with atypical presentations and uncommon complications must be recognized. SLE cannot be excluded based on a low titer of ANA in a particular subgroup such as the elderly, and the prozone effect should be considered responsible for low ANA titers. In this case, late-onset SLE was diagnosed by considering multisystem pathologies despite low ANA titers."}, {"id": "pubmed23n0479_1305", "title": "[Successful treatment of intravenous cyclophosphamide pulse therapy for systemic lupus erythematosus complicated with steroid-resistant hemolytic anemia].", "score": 0.01816545290892726, "content": "(Case 1) A 13-years-old female had multiple arthralgia and butterfly rush, when she admitted in our hospital in May 2001. Nephropathy, hemolytic anemia (Hb 6.3 g/dl and direct Coombs 3+) and high titers of antinuclear antibodies and anti-ds-DNA antibody were disclosed and she was diagnosed as systemic lupus erythematosus (SLE). Although combination therapy of PSL 60 mg/day with a steroid pulse therapy, cyclosporine or an immunosorbent treatment, severe hemolytic anemia remained. However, monthly cyclophosphamide pulse therapy (IV-CY), which was started for the steroid-resistant hemolytic anemia, has gradually become effective and Hb improved up to 11.4 g/dl after 6 courses of IV-CY. (Case 2) A 53-years-old woman diagnosed as SLE in 1978 and she had PSL 5 mg for over 10 years. Severe anemia (Hb 5.9 g/dl) was disclosed with a slight fever in June 2001, and she admitted in our hospital for further examinations. Progressive hemolytic anemia was revealed with marked decrease of Hb (3.4 g/dl) and high titer of direct Coombs (3+). Neither PSL (50 mg/day) nor steroid pulse therapy were effective against hemolytic anemia. In contrast, 3 courses of monthly IV-CY (500 mg/day) resulted in the resolution of hemolysis. It is well known that the steroid-resistant hemolytic anemia is extremely hard to treat and leads to miserable prognosis, but we here propose IV-CY as an alternative and invaluable choice for the treatment of refractory hemolytic anemia complicated with SLE."}, {"id": "pubmed23n0297_3204", "title": "[Autoimmune hemolytic anemia with eosinophilia in elderly patient].", "score": 0.01811772421708477, "content": "A 70-year-old woman was admitted to our hospital in November 1992 for evaluation of anemia. Physical examination revealed anemia, jaundice, swelling of axial and inguinal lymph nodes, and splenomegaly. Abnormal hematological findings were as follows: Hb of 3.9 g/dl, reticulocyte count of 58.2% (61.7 x 10(4)/microliters), hyperplasia of normal erythroblasts in bone marrow, and eosinophilia (21.0%, 2352/microliters) in peripheral blood. Routine laboratory examinations revealed polycolonal hypergammaglobulinemia 3.0 g/dl, a high level of serum LDH (797 IU/I) and a total bilirubin of 2.4 mg/dl (indirect, 1.6 mg/dl). The serum haptoglobin level was very low (< 5 mg/dl). Results of serological examinations were as follows: IgG of 3366 mg/dl, CH50 of 16.0 U/ml, positive Coombs test 2+, and positive tests for antinuclear antibody, rheumatoid factor, and cold agglutinin. CRP was negative. PHA-stimulated lymphocyte blast formation, NK activity, and ADCC activity were found to be suppressed, and the percentage of CD4-positive lymphocytes in peripheral blood was also low. An axillary lymph node biopsy revealed reactive lymphadenitis. No signs or history suggested allergy, collagen disease, or parasitic infection. Autoimmune hemolytic anemia (AIHA) complicated by immunologic abnormalities and eosinophilia was diagnosed. Oral prednisolone markedly reduced the hemolytic anemia, eosinophilia, lymph node swelling, and splenomegaly, but NK activity remained low."}, {"id": "pubmed23n0847_23036", "title": "Elderly female with Autoimmune hemolytic anemia.", "score": 0.01743750935488699, "content": "Autoimmune hemolytic anemia (AIHA) is a rare disease with an estimated prevalence of around 17/100,000. It is often difficult to diagnose and treat AIHA, especially in elderly. A 60-year-old female was admitted with the complaints of low grade fever, on-off for 6 months, progressive fatigue and dyspnea on exertion. She was transfused with three units of blood within these 6 months. Examination revealed pallor, edema, hemic murmur, and palpable liver. Hb was 2.9 gm%, T Bil 5.2 mg/dl, ESR 160 mm, and reticulocyte count 44.05%. Direct Coombs test was positive, anti-nuclear antibody (ANA) and Anti ds DNA were positive. A diagnosis of systemic lupus erythematosus (SLE) with AIHA was considered and patient was transfused with two units of packed red cells and put on steroid (prednisolone) at 1 mg/kg body weight daily. After 3 weeks, her Hb had increased to 10.4 gm% with gross clinical improvement. "}, {"id": "pubmed23n0688_12771", "title": "Frequency of anaemia in patients with systemic lupus erythematosus at tertiary care hospitals.", "score": 0.016567656765676567, "content": "To analyze the frequency and causes of anaemia in systemic lupus erythematosus (SLE) patients attending in department of medicine at tertiary care hospitals. This retrospective, descriptive and analytical study was planned to analyze the frequency and causes of anaemia in SLE patients attending the department of medicine at (MMC) and (LUMHS) hospitals during the period of Jan 2006 to Nov 2008. The criteria used in this study were from the American College of Rheumatology. Investigations recorded were blood complete picture, absolute values, peripheral smear, and reticulocyte count in all patients of anaemia. These investigations were necessary to analyse the cases of anaemia in SLE. All investigations were not done in all cases. Patients with hypochromic microcytic anaemia were advised to have serum iron and ferritin levels, seven patients with macrocytic anaemia were advised to have direct and indirect coomb's test, LFTs, serum LDH, serum B12 and folate levels. Patients with normochromic and normocytic anaemia were considered to have anaemia of chronic disease. Bone marrow aspiration and Hb electrophoresis were done in two patients with anaemia of chronic disease. Thirty adult patients were included in this study. Special proforma were prepared to record the information from case sheets of patients including basic information, symptomatology and laboratory investigations. Severity and various types of anaemias were recorded. Anaemia was graded according to severity, as mild (Hb 10-12 G/dl), Moderate (Hb 8-10 G/dl) and severe (Hb < 8 G/dl). Haemoglobinopathies and other types of anaemias were excluded from study. Thirty adult diagnosed patients of SLE, were included. Their ages ranged from twenty years to fifty years at time of presentation. The mean age +/- SD (range) was 28 +/- 6.22 (20-50) years and median age was 31 years. Out of thirty patients, twenty seven (90%) were females and three (10%) were males. Twenty eight (93.33%) patients presented with anaemia, 14 (46.66%) patients were of mild anaemia, 8 (26.66%) patients were of moderate grade anaemia and 6 (20%) patients had severe anaemia. Iron deficiency anaemia was found in 9 (30%) patients, 12 (40%) patients had anaemia of chronic disease and 7 (23.33%) patients had haemolytic anaemia, out of theses 7 patients, 5 (16.66%) patients had Coomb's positive haemolytic anaemia. All thirty patients had ANA positive titres > 1:80; and nineteen (63.33%) patients had anti ds DNA positive, titres > 1:10. Haematologic abnormalities are common manifestations in patients with SLE. Most patients exhibit anaemia at some point during their disease course."}, {"id": "pubmed23n0265_17510", "title": "[A case of systemic lupus erythematosus presenting with myelofibrosis as a cause of pancytopenia].", "score": 0.014060606060606062, "content": "A 54 year-old woman who had a 6 month history of polyarthralgias, oral ulcers, weight loss and fatigue was admitted to the Urawa Municipal Hospital. She developed high fever, dyspnea and thrombocytopenia. Chest radiograph revealed massive right pleural effusion. At this time, laboratory investigations gave the following results: hemoglobin 12.7 g/dl, WBC 7700/microliters and platelet count 9.2 x 10(4)/microliters. Antibody to DNA was negative. Antinuclear antibody was positive at a titer of 320x in a centromere pattern; Anti-RNP and anti-Sm antibodies were negative. CH50 was 18.6 u/ml. C3 was 42.9 mg/dl. C4 was 11.5 mg/dl. Circulating immune complex (Clq) was 30.5 micrograms/ml. Circulating lupus anti-coagulant and anticardiolipine antibodies were positive. Thoracocentesis was performed; the material was a straw-colored exudate with over two thousands white cell per ul and showed marked reduction of complement titiers and elevated immune complex levels. She was then diagnosis as having SLE. Two weeks after admission, progressive leukopenia and anemia succeedingly occurred and resulted in severe pancytopenia. Bone marrow biopsy demonstrated marked marrow fibrosis and increased reticulin content with no evidence of malignancy. Steroid pulse therapy for 3 days started, and subsequently she was treated with 60 mg/day of prednisolone. Three weeks after starting on steroids, the massive pleural effusion was completely disappeared and complement titiers were normalized. Circulating immune complex has not been detected any more. After 8 weeks, the peripheral blood count was normalized. The dose of prednisolone was reduced progressively. On this occasion, the biopsy showed normocullular marrow with a marked reduction in the amount of reticulin.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0501_23829", "title": "Klinefelter's syndrome presenting with leg ulcers.", "score": 0.014052540081128067, "content": "A 54-year-old man of Persian origin presented to our department with a 1-year history of ulcers on the right leg that had been unresponsive to numerous topical treatments, accompanied by lymphedema of the right leg. Medical history included hypergonadotropic hypogonadism, which had not been further investigated. He was treated for 20 years with testosterone IM once monthly, which he stopped a year before the current hospitalization for unclear reasons. The patient reported no congenital lymphedema. Physical examination revealed two deep skin ulcers (Figure 1) on the right leg measuring 10 cm in diameter with raised irregular inflammatory borders and a boggy, necrotic base discharging a purulent hemorrhagic exudate. Bilateral leg pitting edema and right lymphangitis with lymphadenitis were noted. He had low head hair implantment, sparse hair on the body and head, hyperpigmentation on both legs, onychodystrophia of the toenails (mainly the large toe and less prominent on the other toes), which was atrophic lichen-planus-like in appearance and needed no trimming (Figure 2), normal hand nails, oral thrush, and angular cheilitis. Other physical findings were gynecomastia, pectus excavatum, small and firm testicles, long extremities, asymmetrical goiter, systolic murmur 2/6 in left sternal border, and slow and inappropriate behavior. The patient's temperature on admission was 39 degrees C. Blood cultures were negative for bacterial growth. Results of laboratory investigations included hemoglobin (11.2 g/dL), hematocrit (26.8%), normal mean corpuscular volume and mean corpuscular hemoglobin volume, and red blood cell distribution width (16%). Blood smear showed spherocytes, slight hypochromia, anisocytosis, macrocytosis, and microcytosis. Blood chemistry values were taken for iron (4 micro g/dL [normal range 40-150 micro g/dL]), transferrin (193 mg/dL [normal range 220-400 mg/dL]), ferritin (1128 ng/mL [normal range 14-160 ng/mL]), transferrin saturation (1.5% [normal range 20%-55%]), serum folate (within normal limits), and vitamin B12 (within normal limits). Direct Coombs' test equaled positive 2 + IgG. All these values indicated anemia of chronic diseases combined with hemolytic anemia. Further blood work-up tested antinuclear antibody (positive <1:80 homogeneous pattern), rheumatoid factors (143 IU/mL [positive >8.5 IU/mL]), C-reactive protein (286 mg/L [normal range 0-5 mg/L]), anticardiolipin IgM antibody (9.0 monophosphoryl lipid U/mL [normal range 0-7.00 MPL U/mL]) and antithrombin III activity (135% [normal range 74%-114%]). Results of other blood tests were within normal limits or negative, including lupus anticoagulant, beta2 glycoprotein, anticardiolipin IgG Ab, anti-ss DNA Ab, C3, C4, anti-RO, anti-LA, anti-SC-70, anti-SM Ab, P-ANCA, C-ANCA, TSH, FT4, anti-T microsomal, antithyroglobulin, protein C activity, protein S free, cryoglobulins, serum immunoelectrophoresis, VDRL, hepatitis C antibodies, hepatitis B antigen, and human immunodeficiency virus. Endocrinological work-up examined luteinizing hormone (22.9 mIU/mL [normal range for adult men 0.8-6 mIU/mL]), follicle stimulating hormone (49.7 mIU/mL [normal range for adult men 1-11 mIU/mL]), testosterone (0.24 ng/mL [normal range for adult men 2.5-8.0 ng/mL]), bioavailable testosterone (0.02 ng/mL [normal range for adult men >0.6 ng/mL]), and percent bioavailable test (8.1% [normal value >20%]). These results indicate hypergonadotropic hypogonadism. Plasminogen activator inhibitor 1 was 6 U (normal value 5-20 U/mL). Karyotyping performed by G-banding technique revealed a 47 XXY karyotype, which is diagnostic of Klinefelter's syndrome. Doppler ultrasound of the leg ulcers disclosed partial thrombus in the distal right femoral vein. X-rays and bone scan displayed osteomyelitis along the right tibia. Histological examination of a 4-mm punch biopsy from the ulcer border revealed hyperkeratosis, acanthosis, hypergranulosis, and mixed inflammatory infiltrate containing eosinophils compatible with chronic ulcer. Multiple vessels were seen, compatible with a healing process. Direct immunofluorescence of the biopsy revealed granular IgM in the dermo-epidermal junction. Indirect immunofluorescence was negative. Thyroid function tests showed normal thyroid stimulating hormone and free throxine4. Multinodular goiter was seen on thyroid scan and ultrasound. Thyroid fine needle aspiration was compatible with multinodular goiter (normal follicular cells, free colloid, macrophages with pigment). IV treatment with amoxicillin-clavulanic acid 1 g t.i.d. was administered for 2 weeks, with a decrease in temperature and normalization of the leukocyte level. Oral antibiotic treatment with amoxicillin-clavulanic acid was continued for 10 more days, followed by 25 days of ciprofloxacin for the osteomyelitis. Local treatment included saline soakings followed by application of Promogran (Johnson & Johnson, New Brunswick, NJ) and Kaltostat (ConvaTec Ltd., a Bristol-Myers Squibb Company, New York, NY) with slight improvement. At the same time, the patient was treated with warfarin sodium due to deep vein thrombosis under international normalized ratio 2-3. The patient was treated with IM testosterone once monthly for 1 year, which resulted in a reduction in the diameter and depth of the leg ulcers (Figure 3). Blood tests were not performed for follow-up of the immune state."}, {"id": "pubmed23n1019_11883", "title": "When systemic lupus erythematosus affects vision: a rare presentation of this condition.", "score": 0.013817663817663818, "content": "A 23-year-old woman with fever, oral ulcers, arthalgias and weight loss of 2-week duration suddenly developed blurred vision, with reduced visual acuity, cotton wool exudates and retinal vascular tortuosity. Laboratory testing revealed anaemia, lymphopaenia, positive antinuclear antibody and high anti-dsDNA antibody titre with low complement components. There was no evidence of infection, clinching the diagnosis of lupus retinopathy. Steroid therapy alone was highly effective and was also accompanied by a normalisation of haemoglobin and lymphocyte counts, after which azathioprine was added. Hydroxychloroquine was introduced after resolution of retinal changes. Immunosuppressive therapy was progressively tapered over the course of 12 months and then discontinued, and the patient remains in remission 48 months after the initial presentation. Our patient exemplifies a very rare manifestation of systemic lupus erythematosus. We emphasise the importance of its early detection and complexity of treatment in order to reduce visual morbidity."}, {"id": "pubmed23n1004_25990", "title": "Rowell Syndrome in Nigeria: Systemic Lupus Erythematosus Presenting as Recurrent Erythema Multiforme in a Young Woman.", "score": 0.013670776552022496, "content": "Dear Editor, Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by diverse patterns of auto-antibody production with multi-organ affectation. Cutaneous involvement, either alone or in association with other systemic illnesses, is one of its most common manifestations (1). Dermatologic disorders like malar and discoid rashes are quite suggestive of SLE. However, the occurrence of non-specific skin lesions like erythema multiforme (EM) in patients with SLE (Rowell syndrome) can rarely occur (1). In such patients, a diagnosis of SLE may be missed or delayed in the absence of other overt clinical features of lupus. Herein we report a case of recurring EM-like eruptions as the cardinal cutaneous manifestation of previously undiagnosed, active SLE in a young Nigerian woman. A 26-year-old Nigerian woman presented with a three-day history of non-pruritic, generalized, and target-like, erythematous annular patches and plaques which mostly affected the trunk. A few lesions had presented with crusting and erosions at the time of examination (Figure 1). Associated symptoms included oral painful ulcers, low grade fever, and malaise. The patient had no other systemic symptoms and her prior drug history was not remarkable. Her erythrocyte sedimentation rate (ESR) was 66 mm/hour using the Westergren method. Screening for HIV and hepatitis B and C was negative. Herpes simplex, cytomegalovirus, and Epstein Barr viruses could not be screened for. Other baseline investigations (complete blood count, electrolytes, urea and creatinine as well as urinalysis) were within normal limits. The patient was managed as a case of EM of an unidentified inciting agent and her symptoms resolved with supportive care and antibiotics. However, she developed a recurrence about 5 weeks later, with more extensive and coalescent skin lesions (Figure 2). Additionally, there was a new onset of alopecia and pain in the small joints of the hands as well as both knees and ankles. At this time, the patient's ESR had gone up to 112 mm/h and she had developed significant proteinuria, with a protein creatinine ratio of 1.3 g/g (reference <0.5 g/g). Her antinuclear antibody (ANA) titer was high (1:320) with a speckled pattern. Anti-Smith antibody was also positive. A renal biopsy was declined. A tentative diagnosis of Rowell syndrome was made. The patient was started on high-dose steroids and hydroxychloroquine 200 mg twice daily. Subsequent care included the use of mycophenolate mofetil 1 g twice daily for 6 months. This was then changed to azathioprine at 50 mg twice daily. Follow-up after 6 months showed sustained clearance of skin lesions, resolution of fever and joint pains, as well as improvement in the renal profile, with a urine protein-creatinine ratio of 0.77 g/g. The presence of systemic lupus erythematosus, EM-like lesions, and a speckled pattern of antinuclear antibody in our patient fulfils the revised diagnostic criteria for RS put forward by Zeitouni et al. at the turn of the twenty-first century (2). Considering the rarity of EM-like lesions in SLE and the possibility of constitutional symptoms in EM, a diagnosis of RS may be readily overlooked in patients like the one described, whose major cutaneous manifestation of severe active SLE was EM-like lesions. In contrast to classic EM, where skin lesions are concentrated in the extremities, a predominant truncal distribution of EM-like lesions as found in our patient may favor a clinical consideration of RS (3). However, some authors have challenged the existence of Rowell syndrome as a distinct clinical laboratory entity. Arguments put forward in this regard include the fact that none of the immunological markers that have been described in RS are specific to any disorder. Additionally, the annular polycyclic dermatosis seen in sub-acute cutaneous lupus erythematosus (SCLE) can be difficult to clinically and histologically differentiate from EM (4,5). Patients with SLE also have a higher likelihood of developing adverse drug reactions (6). The inherent complexity of SLE may make for delayed and oftentimes difficult diagnosis, especially in a country where immunologic tests are expensive and rheumatologists are scarce. When patients do occasionally present with recurrences of skin lesions in the spectrum of EM, Steven-Johnson syndrome, and toxic epidermal necrolysis in the absence of a definite inciting agent, undiagnosed lupus may indeed be present in some of these individuals and should be considered in the differential diagnosis. In conclusion, while it is very rare, SLE may present first with recurrent episodes of EM-like rash. Despite the various possibilities which underlie their association, prompt identification and treatment of SLE in patients presenting with EM is important to prevent death or irreversible organ damage."}, {"id": "pubmed23n0273_11418", "title": "[Lupus erythematosus in old age].", "score": 0.013403361344537814, "content": "Over a period of 2 months an 88-year-old man developed progressively more severe breathing-related pain under the right shoulder blade, loss of appetite, general weakness, depressive mood, sub-febrile temperature and nocturnal sweating. Various inflammation parameters were raised (sedimentation rate 43 mm in the first hour; C-reactive protein 26 mg/dl; white cell count 12,500/microliters). There also were pleural effusion and signs of mild nonspecific hepatitis. Antibiotics were administered because bacterial pneumonia was suspected. But the patient's condition deteriorated and he developed nightly periods of disorientation. There was no evidence for any advanced malignancy. Immunological tests pointed towards older-onset systemic lupus erythematosus: titre for antinuclear antibodies markedly raised to 1:20 480; anti-DNA titre moderately raised to 1:125 IU/ml. The patient's general condition and the pleuritic pain improved within 2 days under treatment with prednisone (50 mg daily); the depression, disorientation and fever receded within a week. The anti-DNA titre fell to 47 IU/ml after 8 weeks. He was able to resume his usual social activities and was kept on a maintenance prednisone dose of 5.0 mg daily."}, {"id": "pubmed23n0771_4606", "title": "A puzzle of hemolytic anemia, iron and vitamin B12 deficiencies in a 52-year-old male.", "score": 0.013071895424836603, "content": "A 52-year-old male with no significant past medical history reports increasing generalized fatigue and weakness for the past 2 weeks. Physical examination reveals jaundice and pallor without organomegaly or lymphadenopathy. His hemoglobin was 5.9 g/dL with a mean corpuscular volume of 87.1 fL and elevated red blood cell distribution width of 30.7%. His liver function test was normal except for elevated total bilirubin of 3.7 mg/dL. Serum LDH was 701 IU/L, and serum haptoglobin was undetectable. Further investigation revealed serum vitamin B12 of <30 pg/mL with elevated methylmalonic acid and homocysteine level. In addition, serum ferritin and transferrin saturation were low. The patient was diagnosed with hemolytic anemia secondary to vitamin B12 deficiency with concomitant iron deficiency anemia. "}, {"id": "pubmed23n0553_3168", "title": "Lymphoma in a patient with systemic lupus erythematosus.", "score": 0.013042943275501415, "content": "A 40-year-old woman with a 10-year history of systemic lupus erythematosus (SLE) presented with fever, lymphadenopathy and fatigue. Before that time, her SLE symptoms had been controlled with hydroxychloroquine, NSAIDs, and an occasional short course of moderate-dose prednisone. Two months before presentation, she experienced fevers ranging from 38.3 to 39.7 degrees C, but she had no specific symptoms that suggested local infection. Physical examination, multiple blood cultures, and laboratory investigations that included the following tests: hemoglobin concentration; erythrocyte sedimentation rate; C-reactive protein level; serum lactate dehydrogenase level; aspartate aminotransferase level; alanine aminotransferase level; serum complement C3 and C4 levels; white-blood-cell count; platelet count; urinalysis; serum creatinine level; CT of the chest and abdomen; bone-marrow biopsy; serum electrophoresis; and tests for Epstein-Barr virus, cytomegalovirus, hepatitis B virus, hepatitis C virus, HIV-1, antinuclear antibodies, antibodies to Smith antigen, antibodies to double-stranded DNA, and antibodies to Ro and La. Stage IVB diffuse large B-cell lymphoma with marrow and liver involvement concurrent with SLE. The patient promptly underwent chemotherapy, receiving three courses of 3 mg/m(2) vindesine on day 1, 1,500 mg/m(2) cyclophosphamide and 80 mg/m(2) doxorubicin on day 2, and 50 mg/m(2) prednisolone on days 1-5."}, {"id": "pubmed23n0870_7365", "title": "Paroxysmal Nocturnal Haemoglobinuria in the Differential Diagnosis of Unresponsive Iron Deficiency Anemia: A Case Report.", "score": 0.012710505212510024, "content": "A 16-year-old male patient who was on oral iron treatment for iron deficiency anemia for the last one year was seen at the Haematology clinic with complaints of weakness, pallor, and jaundice. A complete blood count revealed Hb of 4.2 mmol/L, Hct of 0.14, and MCV of 76 fl. A blood smear showed 50% neutrophils, 40% lymphocytes, and 10% monocytes with anisocytosis, poikilocytosis, polichromasia in erythrocytes and normoblasts. Reticulocyte count was under 1%. There was a slight erythroid hyperplasia in the bone marrow aspiration. Biochemical examinations showed total bilirubin of 3.9 mg/dL, indirect bilirubin of 3.4 mg/dL, and lactate dehydrogenase (LDH) of 6085 U/L (220-450). In re-evaluating the history of the patient, he was seen to be complaining of dark discoloration of morning urine. Perl's reaction was found to be positive for hemosiderin in the urine sediment. Because Ham's test was positive, the levels of CD55, 58, and 59 proteins on erythrocyte membranes were found to be lower. The patient was started 32 mg of methylprednisolone and his anaemia was improved by the 14th day of treatment. When evaluating iron deficiency anemia resistant to iron supplementation, PNH should be kept in mind. "}, {"id": "InternalMed_Harrison_1673", "title": "InternalMed_Harrison", "score": 0.011592988337174384, "content": "Fever >38.3°C (101°F) on at least two occasions 2. Illness duration of ≥3 weeks 3. 4. Diagnosis that remains uncertain after a thorough history-taking, physical examination, and the following obligatory investigations: determination of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level; platelet count; leukocyte count and differential; measurement of levels of hemoglobin, electrolytes, creatinine, total protein, alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, creatine kinase, ferritin, antinuclear antibodies, and rheumatoid factor; protein electrophoresis; urinalysis; blood cultures (n = 3); urine culture; chest x-ray; abdominal ultrasonography; and tuberculin skin test (TST). The range of FUO etiologies has evolved over time as a result of changes in the spectrum of diseases causing FUO, the widespread Percentage of Cases Due to Indicated Cause"}, {"id": "pubmed23n0496_5632", "title": "[Trypanosomiasis in a woman from Cameroon mimicking systemic lupus erythematosus].", "score": 0.010113650874728642, "content": "A 27-year-old woman from Cameroon was admitted because of arthralgia, myalgia and severe thrombocytopenia (20,000/ micro l). She had been suffering from weakness, recurrent febrile episodes, generalized lymphadenopathy and pancytopenia for 2 years. Having a typical autoantibody constellation and fulfilling four (pleurisy, autoimmune-hemolytic anemia, antinuclear antibodies (ANA), anti-Sm antibodies) of the American College of Rheumatology (ACR) classification criteria, systemic lupus erythematosus (SLE) had been diagnosed at another hospital. Treatment with corticosteroids and azathioprine did not improve her condition. Abnormal laboratory findings were pancytopenia, elevated markers of inflammation and extreme hypergammaglobulinemia (70 %) with polyclonal IgM (73 g/l). Antinuclear antibodies (ANA), anti-Sm-, anti-Scl 70-, anti-U1-RNP-, anti-histo-, anti-leukocyte- and IgM anticardiolipin antibodies were detected. A bone marrow biopsy showed polyclonal B-cell and plasma cell infiltrates. Examination of peripheral blood smears disclosed trypanosoma brucei infection. After the diagnosis of stage 2 West African trypanosomiasis (sleeping sickness) specific treatment was initiated leading to subsequent remission of the disease. This case report underlines the importance of a thorough differential diagnosis in cases of suspected autoimmune disease. Induction of autoantibodies during infectious diseases may be misleading. The use of the ACR criteria for SLE must be restricted to the classification of proven connective tissue diseases."}, {"id": "pubmed23n0715_15091", "title": "Coexistence of Crohn's disease in a patient with systemic lupus erythematosus.", "score": 0.009708737864077669, "content": "The concurrence of inflammatory bowel disease with systemic lupus erythematosus (SLE) is rare. The concomitant diagnosis of Crohn's disease and SLE is even more rare. The patient, a 40-year-old woman, was admitted to our hospital because of relapsing episodes of abdominal pain, diarrheas upper and lower extremities arthralgias, Raynaud's phenomenon with positive antinuclear antibodies, and fever for the last 2 years. The patient was diagnosed elsewhere with SLE and treated with hydroxychloroquine. Her medical history also included tonsillectomy and total hip replacement after a car accident. Family history was unremarkable. Physical examination was unremarkable except of very mild pain at lower left abdominal quadrant. Laboratory tests showed erythrocyte sedimentation rate at 32 mm/h, C-reactive protein at 36 mg/dl, positive rheumatoid factor, and increased C3, C4, positive antinuclear antibodies with the presence of anti-Sm and anti-RNP antibodies. Ileocolonoscopy revealed colonic inflammation with ulcers and pseudopolyps. Subsequent biopsies were diagnostic of Crohn's disease. Patient was diagnosed with Crohn's colitis concomitant to systemic lupus erythematosus and was started on therapy with azathioprine 2 mg/Kg, methylprednisolone 16 mg/d with slow tapering, mesalazine 1.5 g/day, and hydroxychloroquine. Patient is in excellent health status on the six-month follow-up."}, {"id": "pubmed23n0558_18224", "title": "[Relations beetwen severity of anemia and certain antiphospholipid antibodies presence in systemic lupus erythematosus patients].", "score": 0.009615384615384616, "content": "Anaemia is a common hematological abnormality in systemic lupus erythematosus (SLE). Autoimmune hemolytic anaemia (AHA) is one of the types of anaemia in SLE. Other reasons of anaemia in SLE are: chronic inflammatory diseases, insufficient level of elements necessary for erythropoiesis and chronic renal disease. The aim of the study was to examine the relations between severity of anemia and presence of certain antiphospholipid antibodies (aPL) in SLE. The study group consisted of 24 patients (one man and 23 women) with active lupus. Anticardiolipin antibodies (aCL), anti-02 glycoprotein I antibodies (anti-beta2 GPI IgM and IgG) and red cell parameters: haemoglobin level (Hb), erythrocytes (RBC), mean corpuscular volume (MCV), mean corpuscular haemoglobin mass (MCH), mean corpuscular haemoglobin concentration (MCHC) and ferritin level were measured before beginning or intensification of treatment. The activity of SLE was measured by SLEDAI and C3 complements. The patients were divided into two subgroups: subgroup with aCL-IgG above 20 u/ml and below 20 u/ml. In patients with aCL-IgG above 20 u/ml the mean Hb level was lower, and the mean ferritin level was significantly higher than in patients with aCL-IgG level below 20 u/ml. We observed a negative, significant correlations between Hb and aCL-IgG (p = 0.01; R = -0.47) and between Hb and anti-32 GPI IgG (p = 0,0003; R = -0,67) and a negative correlation between Hb and activity of SLE estimated by SLEDAI (p = 0.02; R = -0.45) and positive between Hb and C3 complement (p = 0.02; R = +0.5). antiphospholipid antibodies presence in patients with SLE can influence the severity of anaemia."}, {"id": "pubmed23n0754_14208", "title": "Subacute cutaneous lupus erythematosus induced and exacerbated by proton pump inhibitors.", "score": 0.009523809523809525, "content": "Subacute cutaneous lupus erythematosus (SCLE) can be induced by numerous drugs. We report 3 cases of SCLE induced by proton pump inhibitors (PPIs). To highlight a rare cutaneous side effect induced by a frequently prescribed drug such as a PPI. Case 1 was a 30-year-old man who developed multiple annular plaques over the trunk and lower limbs 1 month after the initiation of pantoprazole. Antinuclear antibodies (ANA) were positive with anti-Ro/SSA and anti-La/SSB antibodies, and histology confirmed the diagnosis. Clinical improvement was achieved 8 weeks after the discontinuation of pantoprazole and the introduction of a treatment combining topical steroids and hydroxychloroquine. Lesions relapsed when pantoprazole was accidentally rechallenged. The second case was a 31-year-old woman, 28 weeks pregnant, who presented erythematous annular plaques over the trunk 7 weeks after starting esomeprazole. ANA and anti-Ro/SSA antibodies were positive, and the histology was compatible with SCLE. Fetal ultrasound was normal. She was treated with topical and oral steroids and hydroxychloroquine. Clinical improvement was achieved 4 weeks after the discontinuation of esomeprazole. The third case was a 57-year-old woman with systemic erythematosus lupus presenting annular and psoriasiform lesions on the trunk for 15 months. She was treated successively with hydroxychloroquine, azathioprine, mycophenolate mofetil and methotrexate with prednisone. A review of her drug history revealed the introduction of omeprazole a few weeks before the first appearance of skin lesions and omeprazole was contraindicated. SCLE should systematically be suspected in case of eruption after the introduction of PPI. The risk of fetal cardiac complications is important in pregnant women."}, {"id": "pubmed23n0298_13034", "title": "[Sarcoidosis in a patient with autoimmune hemolytic anemia].", "score": 0.009523809523809525, "content": "A 65-year-old woman was admitted to our hospital because of severe anemia. A skin biopsy was done in January 1994 and sarcoidosis was diagnosed. Diffuse reticular shadows were seen in both lung fields on a chest X-ray film and mediastinal lymph node swelling was seen on a chest CT scan. She was followed as an outpatient and was not treated. She suddenly experienced vertigo and general fatigue in March 1995. Laboratory findings on admission were as follows: Hb 6.2 g/dl, MCV 115.9 fl, Ret 198%, LDH 732 IU/L, I-Bil 1.9 mg/dl, and Coombs' test was positive. Autoimmune hemolytic anemia was diagnosed, and she was treated with prednisolone (1 mg/kg). As of the time of this writing, she has no relapse of hemolytic anemia though prednisolone was discontinued 6 months ago."}, {"id": "pubmed23n0834_13001", "title": "Borderline tuberculoid leprosy in childhood onset systemic lupus erythematosus patient.", "score": 0.009433962264150943, "content": "Leprosy is a contagious and chronic systemic granulomatous disease caused by the bacillus Mycobacterium leprae. To our knowledge, no case of leprosy in a childhood-onset systemic lupus erythematosus (c-SLE) patient has been reported. For a period of 31 years, 312 c-SLE patients were followed at the Pediatric Rheumatology Unit of our University Hospital. One of them (0.3%) had tuberculoid leprosy skin lesions during the disease course and is here reported. A 10-year-old boy from Northwest of Brazil was diagnosed with c-SLE based on malar rash, photosensitivity, oral ulcers, lymphopenia, proteinuria, positive antinuclear antibodies, anti-double-stranded DNA, anti-Sm and anti-Ro/SSA autoantibodies. He was treated with prednisone, hydroxychloroquine and intravenous cyclophosphamide, followed by mycophenolate mofetil. At 12-years-old, he presented asymmetric skin lesions characterized by erythematous plaques with elevated external borders and hypochromic center with sensory loss. Peripheral nerve involvement was not evidenced. No history of familial cases of leprosy was reported, although the region where the patient resides is considered to be endemic for leprosy. Skin biopsy revealed a well-defined tuberculoid form. A marked thickening of nerves was observed, often destroyed by granulomas, without evidence of Mycobacterium leprae bacilli. At that time, the SLEDAI-2K score was 4 and he had been receiving prednisone 15 mg/day, hydroxychloroquine 200 mg/day and mycophenolate mofetil 3 g/day. Paucibacillary treatment for leprosy with dapsone and rifampicine was also introduced. In conclusion, we have reported a rare case of leprosy in the course of c-SLE. Leprosy should always be considered in children and adolescents with lupus who present skin abnormalities, particularly with hypoesthesic or anesthesic cutaneous lesions. "}, {"id": "pubmed23n0575_6282", "title": "[Hematological abnormalities in systemic lupus erythematosus and clinical significance thereof: comparative analysis of 236 cases].", "score": 0.009433962264150943, "content": "To investigate the hematological abnormalities and their relationship to the disease activity of systemic lupus erythematosus (SLE). The clinical data of 236 SLE patients, 29 males and 207 females with the age of confirmed diagnosis of 33.4, were divided into 3 groups: anemia group, with the hemoglobin (Hgb) < 100 g/L, including 2 subgroups, i.e. subgroup of anemia caused by chronic diseases (ACD) and subgroup of hemolytic anemia; low white blood cell group 1 (Group WBC1) with the WBC count < 4.0 x 10(9)/L, white blood cell group 2 (Group WBC2) with the WBC count 3.0 approximately 3.9 x 10(9)/L, immune thrombopenic purpura group (ITP group) with a platelet count < 100 x 10(9)/L, and control group without hematological changes. 72 patients underwent morphologic characterization of their bone marrow. The hematological data and the relationship thereof to the disease activity in different groups were analyzed. Among the 236 SLE patients 168 (71.18%) had hematological abnormalities and 68 of them (28.82%) without hematological abnormalities. 123 of the 168 patients with hematological abnormalities (52.1%) had anemia, 82 of which (66.7%) had characteristics of anemia caused by chronic diseases, 18 (14.6%) had hemolytic anemia, 8 (6.6%) had hematopoietic abnormalities, and the remaining 15 patients (12%) had anemia caused by unknown reasons. 73 of the 236 SLE patients (30.9%) had a WBC count < 4.0 x 10(9)/L and 57 patients (24.2%) had a platelet count < 100 x 10(9)/L. In the groups with hemolytic anemia, WBC count < 3.0 x 10(9)/L and thrombocytopenia, the complement levels were significant lower, and the levels of C-reactive protein (CRP) and positive anti-dsDNA antibody were significantly higher than those of the controls (all P < 0.05) The rate of positive antiphospholipid antibody of the hemolytic anemic patients and patients with thrombocytopenia were 22.2% and 15.8% respectively, both significantly higher than that of the controls (2.9%, both P < 0.05). 49 of the 72 patients undergoing morphologic characterization of bone marrow had normal cell morphology and a normal appearing bone marrow, 10 had varying degrees of pathologic hematopoietic changes, 2 lacked megakaryocytes, 9 expressed decreased proliferation in all three hematopoietic lineages, and 2 had only a decrease in erythropoiesis. The reason of the high proportion of anemia among the SLE patients in China may be the higher proportion of anemia caused by ACD in comparison with that abroad. Although SLE patients have lower rates of hemolytic anemia, HA is an important index of SLE disease activity. Thrombocytopenia and a WBC count < 3.0 x 10(9)/L are related to SLE disease activity. Abnormalities of hemopoiesis by bone marrow is one of the reasons of sever hematological changes in part of the SLE patients."}, {"id": "pubmed23n0740_15281", "title": "Prevalence of hematinics deficiency amongst female students and its correction.", "score": 0.009345794392523364, "content": "Nutritional anemia (NA) is common in India. While iron deficiency (ID) is a well recognized cause of NA, prevalence of deficiencies of other hematinics is not systematically investigated. Seventy students of a junior class of a polytechnic and 202 inmates of girl students home were taken up for study. Students were given a questionnaire to elicit anemia related symptoms. Blood was collected for complete blood count (CBC), serum ferritin, folic acid and vitamin B12. Students of polytechnic received hematinic at bed time during their menstrual periods whereas inmates of students home received hematinic at bed time, 3 days in a week. After 6 months blood tests were repeated in those who completed the treatment. CBC was done on Coulter counter and ferritin, folic acid and vitamin B12 were assayed by chemiluminescence. Students were divided into three groups-(1) Control group with Hb 12.0 g/dl or more and ferritin 15.0 ng/ml or more; (2) ID Group with Hb 12.0 g/dl or more and ferritin less than 15.0 ng/ml; and (3) Iron Deficiency Anemia (IDA) group with Hb less tha 12.0 g/dl and ferritin less than 15.0 ng/ml. Basal parameters of three groups were compared using students t test. Change in parameters with treatment was compared using paired students t test. Median age-16 years (range 10-25). Anemia ( Hb < 12.0 g/dl)-94 (34.6%); MCV < 80 fl-153 (56.3%); MCH < 27 pg-167 (61.4%); Ferritin < 15.0 ng/ml-161 (59.2%); Folic acid < 3.5 ng/ml-34 (12.5%); Vitamin B12 < 258 pg/ml-133 (48.9%) Pre-therapy: (1) Hb, MCV, MCH and ferritin significantly lower in ID and IDA Groups compared to control group. (2) Hb, MCV, MCH and Ferritin significantly lower in IDA Group as compared to ID Group. POST-THERAPY: (1) IDA group showed significant increase in Hb, MCV, MCH, ferritin, folic acid and vitamin B12. (2) final Hb (11.26+1.07) and ferritin (7.46+4.81) in IDA Group were subnormal. (3) MCV, MCH, ferritin, folic acid and vitamin B12 increased significantly in ID Group and control group. (1) Nutritional anemia is common amongst asymptomatic young female students. (2) Deficiencies of iron, folic acid and vitamin B12 are common and coexist. (3) 105 mg elemental iron for 3 days in a week for 6 months is not adequate to correct IDA. (4) 105 mg iron for 3 days in a week is enough to correct ID. (5) Non-anemic individuals with ID have iron deficient erythropoiesis. (6) Non-anemic individuals without ID, in this cohort, also had iron deficient eryhtropoiesis."}, {"id": "pubmed23n0956_21239", "title": "Hardness and Painful Lesion of the Breast.", "score": 0.009259259259259259, "content": "Dear Editor, Lupus panniculitis or lupus profundus is a rare inflammatory complication found in patients with systemic lupus erythematosus (SLE), or discoid lupus erythematosus (DLE) (1). When the breast is involved, the term lupus mastitis (LM) is used. This disease involving the breast is rare, and the lesions may precede, coincide with, or occur later than the onset of other lupus lesions. Tissue biopsy is required to confirm the suspected diagnoses of LM. We report a case of a patient with lupus mastitis due to the important differential diagnosis. A 60-year-old woman presented with a painful nodular lesion in her left breast that had appeared 15 days ago (Figure 1, a). She had been previously diagnosed with discoid lupus erythematosus 3 years ago. Physical examination revealed a deep and firm erythematous subcutaneous nodule without overlying skin involvement in the lower-central portion of the left breast. Laboratory findings were positive for antinuclear antibodies (1:80) and double-stranded deoxynucleic acid antibodies (1:10). Mammography and ultrasounds showed an area of increased density and irregular breast tissue along with an important thickening of the overlying skin (Figure 1, b). On suspicion of malignancy, a needle biopsy of the breast lesion was performed and showed vacuolar alteration and lymphocytic infiltrate in the basal layer. Subcutaneous fat showed a lobular panniculitis with a prominent lymphocytic infiltrate and hyalinization of the fat lobules (hyaline fat necrosis). Direct immunofluorescence of the face biopsy revealed IgA, IgG, IgM, and C3 granular deposition. Based on these results, a diagnosis of lupus mastitis associated with DLE was established. Antimalarial therapy resulted in complete resolution of the clinical features. Three years later, the patient presented with a disfiguring atrophy with retraction in the damaged areas of the breast (Figure 2). Lupus mastitis is a very unusual disease that most commonly affects middle-aged women. The first case of LM was described by Tuffanelli in 1971. The lesions usually present following the diagnosis of SLE/DLE; however, on rare occasions they may be observed earlier (2). The histophysiology of this disease remains unclear, but the predominant theory suggests an autoimmune-related etiology. Corroborating evidence for this theory includes the finding of immune complexes, both at the basement membrane of the dermal-epidermal junction and in the blood vessels in the areas of panniculitis (3). Lupus mastitis may be present in the breast as single or multiple subcutaneous nodules that may be tender or painful and can progress to chronic ulcers over time or resolve, leaving atrophic scars. The overlying skin can be normal, erythematous, poikilodermic or ulcerated. When skin changes are prominent, the lesion may clinically and radiologically mimic inflammatory breast carcinoma. Mammographic and ultrasounds findings include an ill-defined breast density with or without associated microcalcifications (4). Histologically, this disease is characterized by lobular lymphocytic panniculitis and predominantly involves the fat lobule and the presence of anucleated adipocytes in a background of a glassy-appearing collagenous stroma (hyaline fat necrosis). Fibrinoid necrosis of the vessel wall has also been reported, but is usually absent (5). Differential diagnosis of lupus mastitis includes inflammatory breast carcinoma, primary medullary carcinoma, and other immune-mediated inflammatory conditions such as diabetic mastopathy. The first line of treatment the use of antimalarial drugs such as hydroxychloroquine. Systemic steroids and cyclophosphamide have also been used. Surgical treatment should be considered only in patients who do not respond to management with medications. In summary, we reported a case of lupus mastitis in a patient with discoid lupus erythematosus. This dermatosis should be considered in the differential diagnosis of breast lesions in lupus patients, and a biopsy of the breast lesion is essential to reject suspected malignancy. If the disease is left untreated, unsightly atrophy will appear; it is thus important to diagnose early on. The course of the disease tends to be chronic with remission and flares, so patients should be followed-up regularly due to the risk of recurrences in the same area or in a different location."}, {"id": "pubmed23n0223_13278", "title": "[Central nervous system involvement in systemic lupus erythematosus].", "score": 0.009174311926605505, "content": "Three cases are presented, in two of which the CNS lesions revealed the presence of systemic lupus erythematosus (SLE). The diagnosis of SLE was certain according to the criteria of the ARA, and it was further confirmed by results of renal needle puncture biopsy. Case 1: A 16-year-old adolescent developed choreic movements followed, one month later, by psychotic symptoms suggesting a mixed hebephrenic-catatonic schizophrenic affection. Cutaneous lesions and signs of renal insufficiency 3 months later established that these disorders were related to SLE. A favourable outcome was observed rapidly for the systemic signs, recovery from neuropsychic symptoms being obtained after 3 months only but then in a few days. This course suggests the diagnosis of a \"functional psychosis\" of lupus origin. Case 2: A 24-year-old woman developed left hemiparesis followed by febrile coma. The slowly favourable course of the disease led to the appearance of a progressive dementia, with numerous epileptic seizures. Although tests for antinuclear antibodies were negative and the ESR was normal, several minor biological anomalies were suggestive of a systemic disease and the diagnosis of SLE was finally established. Corticotherapy produced only slight transient improvement. This progression towards dementia with progressive cerebral atrophy is most probably related to cerebral lupus lesions, the initial coma in the absence of any other apparent cause possibly being the first sign. Case 3: A 47-year-old woman developed simultaneously or separately episodes of arthralgia and uveitis of unknown origin over a 12-year period, and attacks of regressive multilocular neurological deficiency over a 15-year period.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0053_2677", "title": "[Interstitial lupus nephritis].", "score": 0.009174311926605505, "content": "A 17-year old-male presented with a 6-week history of weight loss, lassitude and calf pains. On examination he was very pale. Laboratory tests showed a very high erythrocyte sedimentation rate (155 mm in the first hour), anaemia (haemoglobin 10.1 g/dl), and a raised serum creatinine of 1.54 mg/dl. Microhaematuria (5-10 erythrocytes/microliter) and pronounced pyuria (500 leucocytes/microliter) were present, but the urine was sterile and there was no increase in albumin excretion. The serum IgG was raised to 75.7 g/l, suggesting an autoimmune disorder. Anti-nuclear antibodies (titre 1 : 1920) and anti-double-stranded DNA antibodies (31 U/ml) were present, while the serum complement C4 was decreased to 0.11 g/l. Renal histology showed an interstitial nephritis without glomerular involvement, while the bone marrow showed vasculitis accompanied by a prominent plasma-cell infiltrate. A diagnosis of interstitial nephritis associated with systemic lupus erythematosus was made, with asymptomatic cardiac and hepatic involvement. Renal function recovered rapidly with prednisolone therapy (initial dose 2 mg/kg.d). While glomerulonephritis is the most common lupus-associated renal disorder, isolated interstitial nephritis may occur in some cases, often with an absence of proteinuria."}, {"id": "pubmed23n0274_11480", "title": "[Renal and cerebral infarctions in a patient with systemic lupus erythematosus without antiphospholipid antibodies].", "score": 0.00909090909090909, "content": "Renal artery infarction is a very rare complication in patients with systemic lupus erythematosus (SLE), even in patients with antiphospholipid syndrome which often causes thromboembolism: Renal infarctions have only been reported in 4 SLE patients with antiphospholipid antibodies (aPL). Here we report a case of SLE without aPL who accompanied by renal and cerebral infarctions. A 42-year old Japanese woman with 8 year history of SLE manifested by arthralgia, central nervous system symptoms, positive-antinuclear and anti-DNA antibodies was admitted to our hospital for the treatment of progressive lupus nephritis. Physical examinations revealed hypertension (130-160/80-110 mmHg) without pitting pretibial edema. Laboratory evaluations showed proteinuria (3.7 g/day), normal serum creatinine level (0.9 mg/dl), low serum albumin level (2.3 g/dl) and high cholesterol level (317 mg/dl). Old cerebral infarctions were recognized by magnetic resonance imaging. However, hematological and immunological studies revealed that this case has neither a prolonged activated partial thromboplastin time, lupus anticoagulant nor anticardiolipin antibodies. Prednisolone was increased from 30 mg/every other day to 30 mg/day, and oral azathioprine, 50 mg/day, was started for the treatment of lupus nephritis. On the 11th day, she suddenly complained severe abdominal pain, which gradually localized on the right side. Computed tomography of the kidney suggested right renal infarctions, and arteriography of right renal artery confirmed both an obstruction of the ventral branch and a narrowing of the dorsal branch of right renal artery. No intra-cardiac thrombus was demonstrated by echocardiography. Following to the treatment with fibrinolytic agent and anticoagulant, her symptoms have improved.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0717_244", "title": "[Hematologic and immunologic signs of lupus: the experience of the hospital of Dakar].", "score": 0.00909090909090909, "content": "The systemic erythematosus lupus (SEL) or lupic disease is a systemic auto-immune pathology, characterized primarily by the presence of antibodies directed against native antibodies anti-DNA. The circumstances of discovery are variable and polymorphic. The hematologic signs and the immunological disorders constitute criteria of diagnosis of lupic disease. It is a multicentric retrospective study from January 1, 1997 to September 30, 2006. Patients were followed up in Internal medicine of Dakar. We appreciate the hematologic and immunological aspects appreciate their prognosis prevalence and their implications with the course the lupic disease. 142 lupic patients were included with 125 women and 17 men; the sex ratio is 0.13. The average age was 34 years with extremes of 6 and 72 years. Our patients had hematologic manifestations average in 32,4 % of the cases and immunological in 76,8 % of the cases. The immunological tests showed the presence, of antinuclear antibodies in 97,9 % of the cases, of native antibody anti-DNA in 45,7 % of the cases, the anti-ECT in 86,95 % (with the anti-RNP in 78,3 % of the cases, anti-Sm in 56,5 % and of anti-SSA in 87 % of the cases). Antibodies anti-DNA and anti-ECT were associated with the hematologic demonstrations respectively in 92,0 % and 95 % of the cases (p = 0,08). Total survival in 96 % of the cases is estimated to 7 years. The circumstances of discovery of the lupic disease are variable. The hematologic signs constitute criteria diagnosis of lupic disease. The accessibility of the hematologic and immunological assessment is necessary for an early diagnosis and an early treatment."}, {"id": "pubmed23n0311_6890", "title": "[A man with systemic lupus erythematosus presenting with spastic paraplegia].", "score": 0.009009009009009009, "content": "We report a 38-year-old man systemic lupus erythematosus who presented with an acute onset of paraplegia and urinary retention. The man had a 12-year history of nodular cutaneous mucinosis and arthralgia. In 1994, he was admitted to our hospital with a sudden onset of weakness and numbness of the right leg followed by an emergence of similar symptoms in the left leg. His elder sister had died at 16 years of age after suffering from systemic lupus erythematosus for 6 years. On examination, the patient had skin rash on his chest, back, head, forehead, and extremities. The neurological examination revealed that his tongue deviated to the right on protrusion. The muscle power was reduced to 2-3/5 in the right leg and to 4/5 in the left leg. The sensory disturbance was noted in the lower extremities with predominant involvement of the right leg. Reflexes were increased in the right biceps, triceps, both patellas, and Achilles tendons. Babinski sign was noted bilaterally. Urinary retention and constipation were also noted. The results of the blood cell count and hepatic and renal function tests were normal. Serum levels of C-reactive protein and complements (C3, C4, CH50) were also normal. Serological examinations showed increased anti-DNA antibody (14 U/ml, [normal, < 6]). Antinuclear antibody was positive at a titer of 1:1380. CSF study showed an increased protein concentration of 83 mg/dl and an IgG level of 14 mg/dl with a normal number of cells. MR images revealed a T1-low, T2-high signal lesion at the upper part of the left ventral medulla. MR images of the brain and spinal cord were normal. The patient was diagnosed as having SLE. High-dose intravenous methylprednisolone (1 g/day) pulse treatment that was started 25 days after the onset of neurological symptoms, produced partial relief. Our case presented with paraplegia with a focal lesion in the left upper ventral part of the medulla on MR images. The incidence of male SLE is low, and paraplegia is a rare complication of SLE. Thus, the medullary lesion in SLE observed in our case appears to be rare. SLE should be considered as a cause of acute onset paraplegia or myelopathy."}, {"id": "pubmed23n0998_19836", "title": "Rowell's Syndrome Triggered by Omeprazole.", "score": 0.008849557522123894, "content": "Dear Editor, Rowell's syndrome is a rare disease, characterized by the appearance of erythema multiforme (EM)-like lesions in patients with lupus erythematosus. It was initially reported by Rowell (1) in 1963 and its existence as a separate clinical entity is currently under debate (2,3). A few cases may have been induced by drugs such as systemic antimycotics, antibiotics, anticonvulsants, and more recently proton pump inhibitors (PPIs). CASE REPORT We present the case of a 67-year-old woman with subacute cutaneous lupus erythematosus (SCLE) and EM-like lesions who fulfilled all the criteria for Rowell's syndrome. The patient had lupus arthritis for two years and was treated with oral methylprednisolone 8 mg/day and hydroxychloroquine 200 mg/day. She started receiving 20 mg of omeprazole daily for gastroprotection. The patient also had arterial hypertension with no current treatment, osteoporosis, and an L1 vertebral fracture. The dermatological examination revealed multiple erythematous infiltrated plaques involving mainly the sun-exposed areas (neck, chest, upper back, and shoulders). Cutaneous lesions had an annular or target pattern and a tendency to form hemorrhagic crusts and scales at the margins (Figure 1, A). The mucous membranes were unaffected. Histological examination (hematoxylin and eosin ×200) found epidermal atrophy, vacuolar degeneration of the basal layer, and sparse perivascular lymphocytic infiltrate in the dermis - features corresponding to lupus erythematosus (Figure 2, A). Single eosinophilic necrotic keratinocytes characteristic for erythema multiforme were observed in the epidermis (Figure 2, B). Direct immunofluorescence (IF) from lesional skin showed granular deposits of C3 on the dermo-epidermal junction. Lupus band test from sun-protected, nonlesional skin was negative. On indirect IF a speckled pattern antinuclear antibodies (ANA) with >1:1280 titers were detected. Anti-Ro (>200 U/mL) and anti-La (>200 U/mL) antibodies were also positive. The blood cell count and differential analysis were within reference ranges. The 24-hour urine protein test showed a non-significant proteinuria - 0.36 g/24h. Photo-testing was impossible considering the extent of the skin lesions. The therapeutic approach consisted of increasing the hydroxychloroquine dose to 400 mg/day, substituting PPI with famotidine 20 mg/day p.o. and ceftriaxone 2 g/day for the superinfection with Ps. aeruginosa, which led to a clinical improvement (Figure 1, B). The methylprednisolone dose remained unchanged due to already existing severe adverse effects. DISCUSSION The diagnosis was based on Zeitouni et al.'s classification (4). The three main criteria are as follows: lupus erythematosus, EM-like lesions, and speckled pattern of ANA. Our patient met all three major and one minor criteria, namely the presence of anti-Ro and anti-La antibodies. As for the other minor criteria, RF was negative and no chilblains were found. Although there was a continuous time lapse (more than 1 year) between the initiation of omeprazole intake and the diagnosis of Rowell's syndrome, we suggest that the connection is probable. For instance, the latency differs depending on the incriminated medication in drug induced SCLE. Longer periods are reported for diuretics and calcium blockers, while the time interval is shorter for chemotherapeutic drugs and antimycotics (5). Our suspicions were further confirmed by the fact that the lesions improved promptly within a month after discontinuation of omeprazole and doubling the dose of hydroxychloroquine. PPIs are reported to be a major cause of drug-induced SCLE (6,7). According to Laurinaviciene et al., the most common drugs involved are PPIs, thiazide diuretics, antifungals, chemotherapeutics, statins, and antiepileptics (6). However, very few cases of Rowell's syndrome are found to be drug-related. The culprit drugs include: oral terbinafine (8,9), norfloxacin (10), sodium valproate (11) and esomeprazole (12) (Table 1). CONCLUSION Despite the common clinical and immunological features shared between SCLE, drug-induced SCLE and EM, Rowell's syndrome seems to be a separate entity rather than a coincidental association. Finally, according to our knowledge this case would be the second of Rowell's syndrome due to PPIs."}, {"id": "pubmed23n0988_18060", "title": "Indications for bone marrow examinations in rheumatology.", "score": 0.008849557522123894, "content": "Hematologic involvement or hematologic malignancies are frequently encountered during the course of rheumatic diseases. Bone marrow (BM) aspiration and/or biopsy examinations may have a diagnostic role in explaining hematologic findings detected in rheumatology clinical practice. Our aim was to describe the indications for BM examinations and to share our BM aspiration/biopsy results. We analyzed 140 BM aspiration/biopsy results of patients conducted at the Department of Rheumatology from 2010 to 2018. Demographics, complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) values, serum biochemistry test results including lactate dehydrogenase (LDH), organomegaly, indications for BM examinations and BM examination results for each patient, and mortality rates were recorded. Out of the 140 patients, 63.6% were female, and the median (Q1-Q3) age was 53 (39.5-65) years. One hundred fifteen (82.1%) patients were diagnosed as having primary rheumatic disease, and 25 (17.8%) were admitted due to musculoskeletal symptoms. Rheumatoid arthritis (RA) (n = 34, [29.5%]), and systemic lupus erythematosus (SLE) (n = 21, [18.2%]) were the most common rheumatic diseases. Cytopenia was the most common indication for BM aspiration/biopsy (n = 83, [59.3%]). Thirty-nine (47%) of 83 patients had drug-induced cytopenia. A pathology was detected in 40 (28.5%) of the 140 BM examinations. Patients with pathologic BM results had either a hematologic malignancy (n = 38, [95%]) or metastasis to a solid organ (n = 2, [5%]). The group of patients with pathologic BM biopsy results had significantly higher rates of lymphadenopathy, splenomegaly, and monoclonal gammopathy compared with the group with non-pathologic results (p = 0.001, p = 0.011, and p = 0.023, respectively). Likewise, LDH concentrations of those with pathologic results were higher than in patients with non-pathologic results [737 (range 577-1420) IU/L vs. 541 (range 306-840) IU/L, p = 0.019]. In this study, cytopenia or CBC abnormalities accompanied by elevated LDH values or anemia along with increased ESR were the most common indications for BM aspiration/biopsy. Further prospective studies are needed to determine the indications of BM aspiration/biopsy and establish the parameters that predict abnormal BM results in rheumatology practice."}]}}}} {"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[226, 311]], "word_ranges": [[32, 41]], "text": "Primary adrenal insufficiency produces hypovolemic hyponatremia (incorrect option 1)."}, "2": {"exist": true, "char_ranges": [[562, 927]], "word_ranges": [[75, 135]], "text": "With natremia above 125 mmol/l it is not indicated to treat with HSS unless the patient has severe neurological symptoms, which does not seem to be the case (they describe a confusional picture that would be a moderate hyponatremic encephalopathy), and it is not indicated to restore natremia as soon as possible (due to the risk of osmotic demyelination syndrome)."}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[312, 425]], "word_ranges": [[41, 56]], "text": "Diabetes insipidus is the opposite of this pathology, usually presenting with hypernatremia (option 4 incorrect)."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "We are presented with a patient with moderate euvolemic hyponatremia, suggesting a syndrome of inadequate antidiuresis (SIAD) secondary to sertraline, although it would be necessary to have urinary osmolality to confirm this. Primary adrenal insufficiency produces hypovolemic hyponatremia (incorrect option 1). Diabetes insipidus is the opposite of this pathology, usually presenting with hypernatremia (option 4 incorrect). The question remains whether to treat aggressively with hypertonic saline, or to be more conservative by indicating hydric restriction. With natremia above 125 mmol/l it is not indicated to treat with HSS unless the patient has severe neurological symptoms, which does not seem to be the case (they describe a confusional picture that would be a moderate hyponatremic encephalopathy), and it is not indicated to restore natremia as soon as possible (due to the risk of osmotic demyelination syndrome). Therefore, the correct answer would be 3. What I do not like about this answer is that the second part (\"if natraemia does not rise, switch to slow infusion of saline\") is incorrect, since in this case the use of furosemide, tolvaptan or urea should be considered, not saline (and it does not indicate tonicity either, since isotonic is not the same as hypertonic). In any case, I do not see it as very likely to be overridden.", "full_answer_no_ref": "We are presented with a patient with moderate euvolemic hyponatremia, suggesting a syndrome of inadequate antidiuresis (SIAD) secondary to sertraline, although it would be necessary to have urinary osmolality to confirm this. Primary adrenal insufficiency produces hypovolemic hyponatremia ([HIDDEN]). Diabetes insipidus is the opposite of this pathology, usually presenting with hypernatremia ([HIDDEN]). The question remains whether to treat aggressively with hypertonic saline, or to be more conservative by indicating hydric restriction. With natremia above 125 mmol/l it is not indicated to treat with HSS unless the patient has severe neurological symptoms, which does not seem to be the case (they describe a confusional picture that would be a moderate hyponatremic encephalopathy), and it is not indicated to restore natremia as soon as possible (due to the risk of osmotic demyelination syndrome). Therefore, [HIDDEN]. What I do not like about this answer is that the second part (\"if natraemia does not rise, switch to slow infusion of saline\") is [HIDDEN], since in this case the use of furosemide, tolvaptan or urea should be considered, not saline (and it does not indicate tonicity either, since isotonic is not the same as hypertonic). In any case, I do not see it as very likely to be overridden.", "full_question": "A 75-year-old woman on sertraline treatment who comes to the emergency department with confusional symptoms. There is no evidence of edema and blood pressure is 130/70. The blood test shows Na 126 mEq/l and K 4 mEq/l, natriuria is 45 mEq/l and diuretic intake has been ruled out. Which of the following is the most correct approach?", "id": 571, "lang": "en", "options": {"1": "It is Addison's disease and corticosteroids should be administered immediately.", "2": "Administer hypertonic saline to restore natremia as soon as possible.", "3": "Indicate water restriction and if natremia does not rise, switch to slow infusion of saline.", "4": "Request brain MRI, since it is probably diabetes insipidus.", "5": NaN}, "question_id_specific": 170, "type": "NEPHROLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0955_2875", "title": "A challenging coexistence of central diabetes insipidus and cerebral salt wasting syndrome: a case report.", "score": 0.0196078431372549, "content": "Combined central diabetes insipidus and cerebral salt wasting syndrome is a rare clinical finding. However, when this happens, mortality is high due to delayed diagnosis and/or inadequate treatment. A 42-year-old white man was referred to neurosurgery due to a non-functional pituitary macroadenoma. He underwent a partial resection of the tumor on July 2, 2015. On the day following surgery he presented polyuria with sodium 149 mEq/L, plasma osmolality 301 mOsm/kg, and urine osmolality 293 mOsm/kg. He started nasal desmopressin 0.05 mg/day with good response. He was already on dexamethasone 4 mg and levothyroxine 75 mcg due to hypopituitarism after surgery. On July 9 he became confused. Cerebral computed tomography was performed with no significant changes. His natremia dropped to 128 mEq/L with development of polyuria despite maintenance of desmopressin dose. His hemoglobin and hematocrit rose from 9.1 g/L to 11.6 g/L and 27.5 to 32.5, respectively. His thyroid function was normal and he was on hydrocortisone 30 mg/day. At 12 p.m. 150 mg/hydrocortisone infusion was initiated, but sodium did not increase. Plasma and urine osmolality were 264 mOsm/kg and 679 mOsm/kg, respectively. At 4 p.m. hydrocortisone was increased and hypertonic saline replacement started. Two hours later he was dehydrated with polyuria and vomiting, and natremia of 124 mEq/L. Hyponatremia was very resistant to treatment despite hypertonic saline replacement, hence desmopressin was suspended. The following day, urine spot analysis showed that natriuresis was 63 mEq/L with serum sodium 132 mEq/L. This was interpreted as a cerebral salt wasting syndrome and control was achieved with aggressive hypertonic saline replacements and fludrocortisone 0.1 mg/three times a day. We present a rare case of a patient with diabetes insipidus and cerebral salt wasting syndrome, who was successfully treated. Hyponatremia in a patient with diabetes insipidus may erroneously be interpreted as inadequate diabetes insipidus control or as syndrome of inappropriate antidiuretic hormone secretion, leading to therapeutic errors. Thus, all clinical and analytical data should be evaluated together for early and correct diagnosis."}, {"id": "pubmed23n0618_5686", "title": "Severe hyponatremia due to rosiglitazone use in an elderly woman with diabetes mellitus: a rare cause of syndrome of inappropriate antidiuretic hormone secretion.", "score": 0.016680509413067552, "content": "To describe the first case of syndrome of inappropriate antidiuretic hormone secretion with life-threatening hyponatremia due to rosiglitazone therapy. We describe the clinical, laboratory, and imaging findings of the study patient. An 89-year-old woman with a 5-year history of type 2 diabetes mellitus was admitted to the emergency department because of unconsciousness. She had reported generalized weakness for 15 days and nausea and vomiting for 3 days. Findings from laboratory analysis showed severe hyponatremia (sodium, 110 mEq/L). She had normal renal, cardiac, and adrenal function, and she did not have edema or volume depletion. The cause of hyponatremia was syndrome of inappropriate antidiuretic hormone secretion. We did not find any cause for her condition other than rosiglitazone, an antihyperglycemic drug that is increasingly being used in patients with type 2 diabetes mellitus. According to her medical history, rosiglitazone was prescribed 1 month previously after withdrawal of gliclazide. We stopped the rosiglitazone and administered hypertonic saline infusion to treat the hyponatremia. Saline infusion was stopped and blood sodium levels were stabilized in the normal range after 2 days. The patient's plasma sodium concentration has remained in the reference range at follow-up visits. This is the first reported case of syndrome of inappropriate antidiuretic hormone secretion as an adverse effect of rosiglitazone, and this drug should possibly be considered for addition to the list of drugs that cause this condition."}, {"id": "pubmed23n0694_15534", "title": "Evaluation of a protocol for hypertonic saline administration in acute euvolemic symptomatic hyponatremia: A prospective observational trial.", "score": 0.01488095238095238, "content": "Acute symptomatic hyponatremia is a frequent yet poorly studied clinical problem. To develop a non-weight based protocol for the treatment of acute symptomatic hyponatremia. Observational study in a Multi-disciplinary Intensive Care Unit of an urban tertiary care hospital. Patients aged >18 years, admitted with euvolemic acute symptomatic severe hyponatremia (defined as serum sodium <120 meq/l with symptoms <24 hours), formed the study population. On confirmation of euvolemic status clinically and by laboratory investigations, patients were administered 100 ml of 3% NaCl over a period of 4 hours irrespective of the weight of the patient, followed by reassessment of serum Na at the end of 4 hours. The volume of hypertonic saline (in ml) required to increase serum Na by 8 meq/l was calculated using the formula: 100 × 8/increment in serum Na observed with 100 ml hypertonic saline. This volume was infused over the next 20 hours. To monitor renal water diuresis which may contribute to overcorrection, the urine specific gravity was monitored every 4 hours for sudden decrease of ≥ 0.010 from the baseline value. Measurement of serum Na was repeated if a fall in the urine specific gravity was observed and subsequently repeated every 4 hours. If no fall occurs in urine specific gravity, serum Na measurement was repeated at 12, 20 and at 24 hours (0 hour being the initiation of 100 ml hypertonic saline). The volume of infusate was adjusted if an excessive increment of serum Na (greater than 3 meq at 8 hours, 4 meq at 12 hours, 5 meq at 16 hours and 6 meq at 20 hours) was observed. Baseline characteristics were compared using chi-square test and Mann-Whitney U test. 58 patients formed the study cohort. The mean age was 58 years. The mean serum Na on admission was 114 meq/l. Administration of 100 ml hypertonic saline resulted in a mean increase in serum Na of 2 meq/l. The mean increase in serum Na over 24 hours was 9 meq/l and mean volume of hypertonic saline required for a serum Na increment of 8 meq/l was 600 ml. The non-weight based protocol with monitoring for water diuresis is reasonably an effective strategy in the treatment of acute euvolemic symptomatic hyponatremia."}, {"id": "pubmed23n1077_6276", "title": "Hyponatremia and Encephalopathy in a 55-Year-old Woman with Syndrome of Inappropriate Antidiuretic Hormone Secretion as an Isolated Presentation of SARS-CoV-2 Infection.", "score": 0.014294134983790157, "content": "BACKGROUND During the coronavirus disease 2019 (COVID-19) pandemic of 2020, varied presentations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been reported. The present report is of a case of hyponatremia and encephalopathy due to the syndrome of inappropriate antidiuretic hormone secretion (SIADH) as the main presentation of SARS-CoV-2 infection in a 55-year-old woman. CASE REPORT A 55-year-old woman with type II diabetes mellitus presented with confusion and slurring of speech, with a temperature of 38.5°C, heart rate of 120 bpm, blood pressure of 159/81 mmHg, and oxygen saturation of 98% on room air. She did not have edema on examination. Laboratory testing showed a low sodium level of 116 mEq/L (reference range, 135-145 mEq/L) with urine osmolarity of 364 mOsm/kg, urinary sodium of 69 mEq/L, urinary potassium of 15.6 mEq/L, and serum osmolarity of 251 mOsm/kg. The patient had normal serum thyroid-stimulating hormone and cortisol levels. A chest X-ray should no pulmonary infiltrates nor did a lumbar puncture reveal signs of infection. A real-time SARS-CoV-2 polymerase chain reaction assay was positive for COVID-19. Brain imaging with computed tomography was negative for acute infarct, intracranial hemorrhage, and mass effect. Based on findings from laboratory testing and physical examination, a diagnosis of SIADH was made. The patient was treated with 3% hypertonic saline, followed by salt tablets and fluid restriction, with improvement in her clinical symptoms and serum sodium level. CONCLUSIONS The present report is of a rare but previously reported association with SARS-CoV-2 infection. Encephalopathy and hyponatremia associated with SIADH without pneumonia or other symptoms of infection should be an indication for testing for SARS-CoV-2 infection."}, {"id": "article-29810_28", "title": "Syndrome of Inappropriate Antidiuretic Hormone Secretion -- Treatment / Management", "score": 0.01349873843566022, "content": "Patients presenting with severe symptoms such as seizures, confusion, or delirium need urgent initial correction with hypertonic saline infusion for the first few hours rather than just water restriction. A 100 mL bolus of 3% hypertonic saline is given in the first 3 to 4 hours, and sodium levels are measured within 2 to 3 hours so that further doses can be adjusted to avoid correcting too rapidly. A rise of 3 to 4 mEq/L within the first few hours in such distressing conditions can be justified. If the patient's mental status does not improve, more boluses of 100 mL hypertonic saline can be given in the same way as above until symptoms get better."}, {"id": "Neurology_Adams_9178", "title": "Neurology_Adams", "score": 0.013204187117230596, "content": "The desired volume of normal saline can then be determined by keeping in mind that its sodium concentration is 154 mEq/L and that of 3 percent (hypertonic) saline solution is 513 mEq/L. If hypertonic saline is administered, it is usually necessary to simultaneously reduce intravascular volume with furosemide, beginning with a dose of 0.5 mg/kg intravenously, and to increase the dosage until a diuresis is obtained. (As a rule of thumb, 300 to 500 mL of 3 percent saline, infused rapidly intravenously, will increase the serum sodium concentration by about 1 mEq/L/h for 4 h.) Guidelines to prevent an overly rapid correction of Na are elaborated further on in relation to central pontine myelinolysis (no more rapidly than 10 mmol/L in the first 24 h). Although the syndrome of SIADH is usually self-limiting, it may continue for weeks or months, depending on the type of associated brain disease."}, {"id": "pubmed23n0399_7901", "title": "[Hyponatremia in clinical practice].", "score": 0.01290028206404513, "content": "Hyponatremia is defined as serum sodium level below 135 mEq/l; this electrolyte disorder can be associated with low, normal or high plasma tonicity. Hyponatremia with normal plasma osmolality, pseudohyponatremia, has little clinical value. Hyponatremia with increased plasma osmolality results from hyperglycemia or mannitol infusion. Patients with hyposmotic hyponatremia may be normovolemic, hypovolemic or hypervolemic; it is most important to know clinical history, physical examination that focuses on volume assessment and laboratory evaluation that includes urine osmolality and urine sodium concentration. Severe hyponatremia is associated with neurological complications and occasionally with mortality; for mild hyponatremia water restriction is usually sufficient, but in serious cases hypertonic saline infusion should be administered. Rapid correction of severe hyponatremia can cause brain demyelination; to prevent brain damage the rate of correction should be no more than 0.5 mEq/l/h (10-15 mEq/l/24 h)."}, {"id": "wiki20220301en030_8771", "title": "Electrolyte imbalance", "score": 0.012637637637637637, "content": "Treatment Considerations for treatment include symptom severity, time to onset, volume status, underlying cause, and sodium levels. If the sodium level is <120 mEq/L, the person can be treated with hypertonic saline as extremely low levels are associated with severe neurological symptoms. In non-emergent situations, it is important to correct the sodium slowly to minimize risk of osmotic demyelination syndrome. If a person has low total body water and low sodium they are typically given fluids. If a person has high total body water (such as due to heart failure or kidney disease) they may be placed on fluid restriction, salt restriction, and treated with a diuretic. If a person has a normal volume of total body water, they may be placed on fluid restriction alone."}, {"id": "wiki20220301en040_16516", "title": "Saline (medicine)", "score": 0.012248407939385489, "content": "Hypertonic saline may be used in perioperative fluid management protocols to reduce excessive intravenous fluid infusions and lessen pulmonary complications. Hypertonic saline is used in treating hyponatremia and cerebral edema. Rapid correction of hyponatremia via hypertonic saline, or via any saline infusion > 40 mmol/L (Na+ having a valence of 1, 40 mmol/L = 40 mEq/L) greatly increases risk of central pontine myelinolysis (CPM), and so requires constant monitoring of the person's response. Water privation combined with diuretic block does not produce as much risk of CPM as saline administration does; however, it does not correct hyponatremia as rapidly as administration of hypertonic saline does. Due to hypertonicity, administration may result in phlebitis and tissue necrosis. As such, concentrations greater than 3% NaCl should normally be administered via a central venous catheter, also known as a 'central line'. Such hypertonic saline is normally available in two strengths, the"}, {"id": "wiki20220301en086_257", "title": "Hyperosmolar hyperglycemic state", "score": 0.011972376403769202, "content": "Management Intravenous fluids Treatment of HHS begins with reestablishing tissue perfusion using intravenous fluids. People with HHS can be dehydrated by 8 to 12 liters. Attempts to correct this usually take place over 24 hours with initial rates of normal saline often in the range of 1 L/h for the first few hours or until the condition stabilizes. Electrolyte replacement Potassium replacement is often required as the metabolic problems are corrected. It is generally replaced at a rate 10 mEq per hour as long as there is adequate urinary output. Insulin Insulin is given to reduce blood glucose concentration; however, as it also causes the movement of potassium into cells, serum potassium levels must be sufficiently high or dangerously low blood potassium levels may result. Once potassium levels have been verified to be greater than 3.3 mEq/l, then an insulin infusion of 0.1 units/kg/hr is started. The goal for resolution is a blood glucose of less than 200 mg/dL. References"}, {"id": "wiki20220301en040_16518", "title": "Saline (medicine)", "score": 0.011887387101550436, "content": "3% NaCl has 513 mEq/L of Na and Cl. 5% NaCl has 856 mEq/L of Na and Cl. NaCl solutions that are less commonly used are 7% (1200 mEq/L) and 23.4% (approx 4000 mEq/L), both of which are used (also via central line), often in conjunction with supplementary diuretics, in the treatment of traumatic brain injury. Dextrose (glucose) 4% in 0.18% saline is used sometimes for maintenance replacement."}, {"id": "pubmed23n0421_14460", "title": "Dural sinus thrombosis with severe hypernatremia developing in a patient on long-term lithium therapy.", "score": 0.010997311097011995, "content": "Dural sinus thrombosis has not been described in a patient with hypernatremia resulting from lithium-induced nephrogenic diabetes insipidus. A 63-year-old man on chronic lithium therapy for schizoaffective disorder was transferred to the Emergency Department with dehydration and signs of central nervous system dysfunction after a 3-week isolation in a room in a psychiatric hospital due to exacerbation of psychiatric disorder, during which he refused to eat. Laboratory examination revealed hypertonic hypernatremia (osmolality, 359 mOsm/kg and Na, 171 mEq/L) and hyposthenuria (specific gravity, 1.010 and osmolality, 249 mOsm/kg), with normal serum endogenous vasopressin concentration (2.3 pg/mL). The serum lithium concentration was within the therapeutic range (0.94 mEq/L). Cranial computed tomography demonstrated subarachnoid hemorrhage and suggested dural sinus thrombosis. Although treatment with indomethacin (25 mg parenterally at 8-hour intervals) was somewhat effective in restoring renal concentrating capacity, he died of massive hemorrhagic infarction on the sixth hospital day, probably secondary to dural sinus thrombosis. The clinical diagnosis was confirmed by postmortem examination. Physicians should be alert for the possibility of dural sinus thrombosis as a complication of hypernatremia resulting from lithium-induced nephrogenic diabetes insipidus."}, {"id": "InternalMed_Harrison_3447", "title": "InternalMed_Harrison", "score": 0.010549039681303274, "content": "The therapeutic goals in hypovolemia are to restore normovolemia and replace ongoing fluid losses. Mild hypovolemia can usually be treated with oral hydration and resumption of a normal maintenance diet. More severe hypovolemia requires intravenous hydration, tailoring the choice of solution to the underlying pathophysiology. Isotonic, “normal” saline (0.9% NaCl, 154 mM Na+) is the most appropriate resuscitation fluid for normonatremic or hyponatremic patients with severe hypovolemia; colloid solutions such as intravenous albumin are not demonstrably superior for this purpose. Hypernatremic patients should receive a hypotonic solution, 5% dextrose if there has only been water loss (as in diabetes insipidus), or hypotonic saline (1/2 or 1/4 normal saline) if there has been water and Na+-Cl– loss. Patients with bicarbonate loss and metabolic acidosis, as occur frequently in diarrhea, should receive intravenous bicar bonate, either an isotonic solution (150 meq of Na+-HCO3 in 5%"}, {"id": "Surgery_Schwartz_655", "title": "Surgery_Schwartz", "score": 0.010507917789757413, "content": "of acute symp-tomatic hypernatremia. Even slower correction should be under-taken for chronic hypernatremia (0.7 mEq/h) because overly rapid correction can lead to cerebral edema and herniation. The type of fluid used depends on the severity and ease of correc-tion. Oral or enteral replacement is acceptable in most cases, or IV replacement with halfor quarter-normal saline can be used. Caution also should be exercised when using 5% dextrose in water to avoid overly rapid correction. Frequent neurologic evaluation as well as frequent evaluation of serum sodium levels also should be performed. Hypernatremia is less common than hyponatremia, but has a worse prognosis, and is an independent predictor of mortality in critical illness.29Hyponatremia Most cases of hyponatremia can be treated by free water restriction and, if severe, the administration of sodium. In patients with normal renal function, symptomatic hyponatremia usually does not occur until the serum sodium level is ≤120 mEq/L."}, {"id": "wiki20220301en032_83058", "title": "Syndrome of inappropriate antidiuretic hormone secretion", "score": 0.01008753849511385, "content": "Diagnosis Diagnosis is based on clinical and laboratory findings of low serum osmolality and low serum sodium. Urinalysis reveals a highly concentrated urine with a high fractional excretion of sodium (high sodium urine content compared to the serum sodium). A suspected diagnosis is based on a serum sodium under 138. A confirmed diagnosis has seven elements: 1) a decreased effective serum osmolality - <275 mOsm/kg of water; 2) urinary sodium concentration high - over 40 mEq/L with adequate dietary salt intake; 3) no recent diuretic usage; 4) no signs of ECF volume depletion or excess; 5) no signs of decreased arterial blood volume - cirrhosis, nephrosis, or congestive heart failure; 6) normal adrenal and thyroid function; and 7) no evidence of hyperglycemia (diabetes mellitus), hypertriglyceridemia, or hyperproteinia (myeloma)."}, {"id": "Neurology_Adams_4476", "title": "Neurology_Adams", "score": 0.01008753849511385, "content": "Treatment of SIADH If hyponatremia has been of several days or more duration, the rapid restitution of serum sodium to normal or above-normal levels carries a risk of producing osmotic demyelination (also called central pontine myelinolysis; see Chap. 40). Our usual procedure in patients with serum sodium concentrations of 117 to 125 mEq/L is to slowly correct the sodium concentration by restricting water to 400 to 800 mL/d and to verify the desired urinary loss of water by checking the patient’s weight and serum sodium until it reaches approximately 130 mEq/L. If there is drowsiness, confusion, or seizures that cannot be confidently attributed to the underlying neurologic illness, or if the serum sodium is in the range of 100 to 115 mEq/L, isotonic or 3 percent NaCl should be infused over 3 to 4 h and furosemide 20 to 40 mg administered to prevent fluid overload. In order to avoid osmotic demyelination, a safe clinical rule is to raise the serum sodium by no more than 12 mEq/L in the"}, {"id": "pubmed23n0535_10827", "title": "Therapeutic approach in patients with dysnatraemias.", "score": 0.010078302601667088, "content": "Rapid correction of dysnatraemias is frequently associated with increased morbidity and mortality. Therefore, it is important to estimate the proper volume and type of infusate required to change the serum sodium concentration predictably. The aim of this study is to evaluate the utility or/and the accuracy of the Adrogue-Madias formula in managing patients with hyponatraemia and hypernatraemia. Among the 317 patients who either on admission to our internal medicine clinic or during their hospitalization were found to have hyponatraemia or hypernatraemia, we studied 189 patients (59.6%) in whom the administration of intravenous solutions was required for the correction of dysnatraemias. Twelve hours after starting the administration of intravenous solutions the anticipated as well as the achieved serum sodium concentration were as follows: in volume depleted patients 130.2+/-4.1 vs 131.3+/-5.2 meq/l (n = 45; P = NS), in syndrome of inappropriate antidiuretic hormone secretion (SIADH) patients 127.4+/-5.7 vs 128.9+/-5.9 meq/l (n = 10; P = NS), in patients with diuretic-induced hyponatraemia 123.8+/-6 vs 125.5+/-5.6 meq/l (n = 29; P = NS), in patients with primary polydipsia 122.5+/-0.7 vs 129+/-1.4 meq/l (n = 2; P = 0.02), while in patients with hypernatraemia 153.6+/-7.5 vs 156.5+/-8.9 meq/l (n = 92; P = 0.021). Furthermore, 24 h from the initiation of the therapeutic intervention the expected and the achieved serum sodium concentrations were 130+/-4 vs 135.6+/-3.3 meq/l (n = 15; P = 0.002) in patients with volume depletion, 128.1+/-4.8 vs 130+/-4.5 meq/l (n = 15; P = NS) in patients with diuretic-induced hyponatraemia and 151.5+/-6.4 vs 153.3+/-8.3 meq/l (n = 67; P = NS) in patients with hypernatraemia. The formula that has been proposed by Adrogue and Madias predicted with relative accuracy the changes in serum sodium concentration in almost all patients. Thus, it should be considered as a very useful tool for the management of dysnatraemias. However, special attention should be paid when this equation is used in patients with hyponatraemia due to extracellular volume depletion after euvolaemia's restoration and primary polydipsia in order to avoid rapid correction of hyponatraemia."}, {"id": "pubmed23n0262_1451", "title": "[Hyponatremia and hypernatremia in the elderly].", "score": 0.009900990099009901, "content": "The study aimed at evaluating an incidence of hypo- and hypernatremia in the elderly and the results of therapy. Hyponatremia. The studies involved 18 patients aged 69.8 +/- 5.9 years with hyponatremia of 126.8 +/- 2.7 mmol/L. The main causes of hyponatremia were: diuretics, diarrhoea, and vomiting. Sodium deficit was calculated prior to the treatment in all patients. An analysis of hyponatremia incidence indicates that hyponatremia was diagnosed in 1.39% of patients over 60 years, hospitalized within 1989-1990. Sodium deficit in this group was 495.5 +/- 167.7 mmol. Sodium chloride solution was given intravenously to 12 patients, according to the \"free correction\" principle (a mean increase in serum sodium level was 0.17 +/- 0.07 mmol/L per hour). Mortality in such treated patients was 33%. Sodium chloride was not given to 6 out of examined patients. In 12 patients (66.6%) hyponatremia developed prior to hospitalization, in 6 patients (33.3%) during hospitalization. Mortality rate was 16.6% and 50%, respectively. This confirms higher mortality rate of the rapidly developing hyponatremia in the hospitalized elderly patients. In some cases hyponatremia may constitute iatrogenic complication, especially in the elderly given diuretics in an uncontrollable way. Own experience suggests that elderly patients with a risk of hyponatremia require close monitoring and early compensation of the electrolyte disorders. Hypernatremia. The studies involved 20 patients aged 71.4 +/- 7.7 years with hypernatremia of 155.6 +/- 8.4 mmol/L. A total water deficit (DH20) was calculated in this group. An analysis of hypernatremia incidence showed that this state was diagnosed in 1.55% of patients treated at the Department of Arterial Blood Hypertension within 1989-1990. Total water deficit was 3.9 +/- 1.9 L. A 5% glucose was given intravenously to 15 patients whereas oral fluid therapy was carried out in 5 patients. A mean corrected DH2O in the first day was 46.0 +/- 21.0%. Mortality rate in this group was 65%. It is worth mentioning that 37% of patients with chronic hypernatremia which developed prior to hospitalization died while in case of the acute hypernatremia developed in the hospital mortality rate was 83%. A significant effect on the results of therapy plays an early correction of hypernatremia. Mortality rate in case of DH2o supplementation below 30% during the first 24 hours is about 66%., if DH2o supplementation is 31-60%, a mortality rate is 63%, and in DH2o supplementation over 60% mortality rate is 100%. The obtained results suggest that hypernatremia in the elderly is related to the high mortality rate (65%). An early decrease of water deficit increases mortality rate in patients with hypernatremia."}, {"id": "pubmed23n0638_22516", "title": "Diagnosis and management of hyponatraemia in hospitalised patients.", "score": 0.009716981132075472, "content": "Hyponatraemia is a commonly encountered electrolyte abnormality in hospitalised patients and is associated with significant morbidity and mortality. The fact that most cases of hyponatraemia are the result of water imbalance rather than sodium imbalance underscores the role of antidiuretic hormone (ADH) in the pathophysiology. Hyponatraemia can be classified according to the measured plasma osmolality as isotonic, hypertonic or hypotonic. Hyponatraemia with a normal plasma osmolality usually indicates pseudohyponatraemia, while hyponatraemia because of a high plasma osmolality is typically caused by hyperglycaemia. After excluding isotonic and hypertonic causes, hypotonic hyponatraemia is further classified according to the volume status of the patient as hypovolaemic, hypervolaemic or euvolaemic. Hypovolaemic hyponatraemia is accompanied by extracellular fluid (ECF) volume deficit, while hypervolaemic hyponatraemia manifests with ECF volume expansion. The syndrome of inappropriate ADH (SIADH) should be suspected in any patient with euvolaemic hyponatraemia with a urine osmolality above 100 mOsm/kg and urine sodium concentration above 40 mEq/l. In the management of any hyponatraemia regardless of the patient's volume status, it is advised to restrict free water and hypotonic fluid intake. Hypertonic saline and vasopressin antagonists can be used to correct symptomatic hyponatraemia. The rate of correction is dependent upon the duration, degree of hyponatraemia and the presence or absence of symptoms. Symptomatic acute hyponatraemia (< 48 h) is a medical emergency requiring rapid correction to prevent the worsening of brain oedema. In asymptomatic patients with chronic hyponatraemia (> 48 h or unknown duration), fluid restriction and close monitoring alone are sufficient, while a slow correction by 0.5 mEq/l/h may be attempted in symptomatic patients. Excessive rapid correction should be avoided in both acute and chronic hyponatraemia, because it can lead to irreversible neurological complications including central osmotic demyelination."}, {"id": "pubmed23n0832_1297", "title": "[The most frequent electrolyte disorders in the emergency department : what must be done immediately?].", "score": 0.009708737864077669, "content": "Hyponatremia is the most common form of electrolyte disorder in the emergency room. The symptoms are unspecific and include nausea, dizziness and often falls. Typical symptoms of severe hypernatremia are vomiting, cerebral seizures, somnolence and even coma. The specific initial laboratory diagnostics include measurement of serum electrolytes, serum glucose, serum and urine osmolarity and sodium in urine. The main aim of the clinical examination is to estimate the volume status. If a patient has hypovolemia an infusion of isotonic sodium chloride solution (0.9 %) is the method of choice. If the patient is euvolemic the syndrome of inappropriate antidiuretic hormone secretion (SIADH) or (neurotropic) drugs might be the cause. In these cases the primary measure is restriction of fluid intake. As a rapid correction of sodium levels can lead to pontine myelinolysis, the increase in sodium concentration must not be less than 10 mmol/l within the first 24 h and 18 mmol/l within the first 48 h. Clinical symptoms of hyperkalemia include neurological (e.g. muscle weakness, paresis, hyperreflexia, cramps and dysesthesia), gastrointestinal (e.g. nausea, vomiting and diarrhea) and cardiac symptoms (e.g. dysrhythmia and conductance disorders). Calcium injection stabilizes cardiac rhythm disorders immediately. For a rapid drop in potassium by shifting the potassium to the intracellular space, administration of glucose with insulin and high-dose inhalative administration of betamimetics can be used. Potassium elimination is achieved by infusion of isotonic sodium choride (0.9 %) with i.v. administration of furosemide, ion exchange resins and hemodialysis."}, {"id": "pubmed23n0252_15548", "title": "[Water intoxication following preparation for barium enema].", "score": 0.009615384615384616, "content": "Induction of water intoxication from tap water enemas was reported a few years ago. Its treatment is still debated. A 4 1/2 year-old boy was admitted because he suffered from coma grade I. A barium enema had been prescribed for fecal incontinence and the patient had been given orally about 4 liters of water during the 24 hours preceding this investigation. Blood examination showed;: Na 122 mEq/l; K 3 mEq/l; Cl 87 mEq/l. Brain CT scan was normal. The patient was placed under restriction of fluid and was given i.v. 5.8% NaCl solution (2 mM/kg) for 3 hours. Convulsions appeared despite this treatment requiring intubation and ventilation plus increasing doses of NaCl: 20% solution (2 mM/kg) for 30 minutes followed by 2 mM/kg for 3 hours, associated with mannitol and furosemide infusion. Use of hypertonic saline solutions in the treatment of water intoxication is discussed. Acute hyponatremia must be rapidly corrected using hypertonic saline solution plus restriction of fluid and diuretic."}, {"id": "pubmed23n0704_13781", "title": "[Hyponatremia--should it be ignored or diagnosed?--Case 8/2011].", "score": 0.009615384615384616, "content": "HISTORY, CLINICAL FINDINGS: A 72-year-old dehydrated female was admitted to our emergency department. She presented with a decreased level of consciousness and had experienced a fall. Her medication included hydrochlorothiazide and amiloride. Laboratory findings showed a severe hyponatremia with a serum sodium concentration of 107 mmol/l and a reduced serum osmolality. Urine sodium and potassium excretion were > 30 mmol/l. A CT scan of the head did not show any signs of trauma. Using a diagnostic algorithm, the diagnosis of a hypotonic hypovolemic hyponatremia due to the intake of diuretics was confirmed. By intravenous infusion of physiological sodium chloride solution and cessation of diuretics, serum sodium concentration was raised gradually. Hereby, the patient`s state of consciousness completely normalized. Hyponatremia represents the most frequent electrolyte disturbance of hospitalized patients. It correlates with neurological deficits, proneness to falling and intrahospital mortality. Due to diagnostic insecurity of many physicians, the finding of a hyponatremia is often ignored or misclassified. Standardized approaches using diagnostic algorithms improve diagnostic accuracy. The here presented algorithm is based on only few parameters: serum and urine osmolality, urine sodium and potassium. Besides gradual raise of serum sodium, therapy of the underlying cause is essential, for example cessation of diuretics. For patients with syndrome of inadequate secretion of antidiuretic hormone (SIADH; hypotonic isovolemic hyponatremia), selective arginin-vasopressin-receptor 2-antagonists (vaptans) are a new therapeutic option. However, due to high costs, we only see an indication for patients with SIADH who are not able to consequently comply with fluid restriction."}, {"id": "wiki20220301en027_84144", "title": "Congenital adrenal hyperplasia due to 21-hydroxylase deficiency", "score": 0.009523809523809525, "content": "When brought to a hospital, the 1- to 3-week-old infant will be both underweight and dehydrated by appearance. Blood pressure may be low. Basic chemistries will reveal hyponatremia, with a serum Na+ typically between 105 and 125 mEq/L. Hyperkalemia in these infants can be extreme—levels of K+ above 10 mEq/L are not unusual—as can the degree of metabolic acidosis. Hypoglycemia may be present. This is termed a salt-wasting crisis and rapidly causes death if not treated. As ill as these infants can be, they respond rapidly to treatment with hydrocortisone and intravenous saline and dextrose quickly restores blood volume, blood pressure, and body sodium content, and reverses the hyperkalemia. With appropriate treatment, most infants are out of danger within 24 hours."}, {"id": "pubmed23n0089_20984", "title": "[Mechanism and therapy of hyponatremia with central origin].", "score": 0.009523809523809525, "content": "The effect of therapy for hyponatremia with central origin (cerebrovascular disease and head injury) was investigated in order to examine contributing factors. Out of a total of 58 subjects admitted to the hospital during the previous three years with cerebrovascular disease (49 cases), and head injuries (9 cases), hyponatremia with central origin occurred within 2 weeks. Special treatment for hyponatremia was not given in 30 of the 58 cases (control group). The group (28 cases) which underwent therapy was optionally selected in terms of the following-SIADH, natriuretic polypeptide involvement and sick cells resulting from Na-K pump disorder. These 28 cases were classified into subgroups: water restricted (7 cases), hypertonic NaCl load (9 cases), glucose/insulin/potassium (GIK) therapy (4 cases), phenytoin administration (8 cases). In all of the 58 patients, the serum sodium, potassium and osmolarity and urinary sodium and potassium were measured daily. The balance of water, sodium and potassium were calculated on hyponatremic phase. Plasma levels of such hormones as antidiuretic hormone, aldosterone and cortisol were measured on hyponatremic phase. For each group, onset day and duration of hyponatremia and lowest sodium value were investigated for the sake of comparison. No significant difference for onset day and lowest sodium value was found between each group. Duration was as follows: control group 9.4 +/- 3.3 days, water restricted 7.4 +/- 2.1 days, hypertonic NaCl load 3.3 +/- 1.4 days, GIK therapy 7.3 +/- 2.9 days and phenytoin administration 8.9 +/- 3.7 days. Hypertonic NaCl load indicated a significantly shorter duration compared with the other groups. Hypertonic NaCl load was found to be most effective for hyponatremia with central origin.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en053_51835", "title": "Ringer's lactate solution", "score": 0.009433962264150943, "content": "In a large-volume resuscitation over several hours, LRS maintains a more stable blood pH than normal saline. Chemistry One liter of Ringer's lactate solution contains: 130–131 mEq of sodium ion = 130 mmol L−1 109–111 mEq of chloride ion = 109 mmol L−1 28–29 mEq of lactate ion = 28 mmol L−1 4–5 mEq of potassium ion = 4 mmol L−1 2–3 mEq of calcium ion = 1.5 mmol L−1 Ringer's lactate has an osmolarity of 273 mOsm L−1 and a pH of 6.5. The lactate is metabolized into bicarbonate by the liver, which can help correct metabolic acidosis. Ringer's lactate solution alkalinizes via its consumption in the citric acid cycle, the generation of a molecule of carbon dioxide which is then excreted by the lungs. They increase the strong ion difference in solution, leading to proton consumption and an overall alkalinizing effect."}, {"id": "pubmed23n1164_2349", "title": "Extreme Hyponatremia Complicated by Osmotic Demyelination in a Previously Healthy Young Individual.", "score": 0.009433962264150943, "content": "Severe hyponatremia can lead to dramatic complications whether it is treated or not. At times, it may be very severe (serum Na concentration: Na 50 yrs Neck circumference: Neck circumference >40 cm Gender: Gender male Use STOP-BANG to decide if high probability of moderate-severe disease. High risk if ‘YES’ to ≥ 3 items Low risk if ‘YES’ to < 3 items Items."}, {"id": "InternalMed_Harrison_19551", "title": "InternalMed_Harrison", "score": 0.010694344380403458, "content": "Independent of obesity, hypertension occurs in >50% of individuals with obstructive sleep apnea. The severity of hypertension correlates with the severity of sleep apnea. Approximately 70% of patients with obstructive sleep apnea are obese. Hypertension related to obstructive sleep apnea also should be considered in patients with drug-resistant hypertension and patients with a history of snoring. The diagnosis can be confirmed by polysomnography. In obese patients, weight loss may alleviate or cure sleep apnea and related hypertension. Continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP) administered during sleep is an effective therapy for obstructive sleep apnea. With CPAP or BiPAP, patients with apparently drug-resistant hypertension may be more responsive to antihypertensive agents."}, {"id": "pubmed23n1090_15618", "title": "The differential impact of respiratory event scoring criteria on CPAP eligibility in women and men.", "score": 0.010473788111427944, "content": "Obstructive sleep apnea is more prevalent and severe in men than women. The American Academy of Sleep Medicine offers 2 definitions for scoring hypopneas: \"acceptable\" = associated with a ≥ 4% oxygen desaturation, adopted by Center for Medicare and Medicaid Services (CMS), and \"recommended\" = associated with a ≥ 3% oxygen desaturation and/or an arousal. We hypothesized that CMS vs American Academy of Sleep Medicine scoring criteria would differentially impact continuous positive airway pressure eligibility in women and men. We conducted a retrospective review of adult diagnostic in-lab polysomnography at an urban academic institution. All polysomnographies were scored by both CMS and American Academy of Sleep Medicine scoring criteria, and an analysis by sex was performed that considered demographics and other polysomnography variables. Of 969 polysomnographies reviewed, 674 (69.6%) were in women. Women were younger (51.5 vs 53.3 years old) and had a higher body mass index (38.6 kg/m30 events per hour The Wisconsin Sleep Cohort Study, a longitudinal study of the natural history of obstructive sleep apnea (OSA), found that of a random sample (602 employed men and women, 30–60 years old) the prevalence of OSA (5 or more events/hr) was 9% for women and 24% for men. However, the study found that among sleepy patients in this group, 2% of women and 4% of men met criteria for obstructive sleep apnea syndrome (OSAS). Those who snored habitually, were more likely to have an AHI of 15 or more."}, {"id": "pubmed23n0805_14504", "title": "Daytime sleepiness in patients with obstructive sleep apnea and severe obesity: prevalence, predictors, and therapy.", "score": 0.009174311926605505, "content": "We sought to determine prevalence and predictors of excessive daytime sleepiness in patients with severe obesity with a body mass index (BMI) > 35 kg/m(2) and obstructive sleep apnea (OSA) with an apnea-hypopnea index > 15/h. The study population consisted of 245 obese OSA patients with a BMI > 35 kg/m(2), who were retrospectively recruited from 3256 consecutive patients who underwent polysomnography at our sleep laboratory between 2006 and 2009. Baseline clinical characteristics and polysomnography results of these 245 patients were compared between patients with and without excessive daytime sleepiness, which was diagnosed in the presence of an Epworth Sleepiness Scale score (ESS) ≥ 11. A total of 123 of 245 study patients (50.2 %) had an ESS ≥ 11. Patients with an ESS ≥ 11 were younger and less often unemployed or retired compared with patients with an ESS < 11. Polysomnography revealed a longer total sleep time (TST), higher sleep efficiency, and shorter sleep latency in patients with ESS ≥ 11. In addition, obstructive apneas during TST as well as oxygen saturations < 80 % occurred significantly more often in patients with versus without an ESS ≥ 11. Improvement of daytime sleepiness after initiation of continuous positive airway pressure (CPAP) therapy occurred more often in patients with versus without ESS ≥ 11 (93 versus 73 %, p < 0.01). Obese patients with OSA and excessive daytime sleepiness are characterized by younger age, longer TSTs, more frequent obstructive apneas, and oxygen desaturations < 80 % compared with patients without excessive daytime sleepiness. Excessive daytime sleepiness can be improved in more than 90 % of patients using CPAP therapy."}, {"id": "pubmed23n0684_21600", "title": "Obstructive sleep apnea: a clinical review.", "score": 0.009174311926605505, "content": "Obstructive sleep apnea is a relatively common disorder which is being recognized and diagnosed with increasing frequency. Patients with this disorder are frequently overweight and usually present with longstanding history of heroic snoring and excessive daytime sleepiness. The diagnosis is established with an overnight sleep study, although the decision as to who should be sent to a sleep laboratory must be made on an individual basis, particularly for those whose main complaint is snoring. The major factor in the pathogenesis of this disorder is a narrow and floppy pharyngeal airway. Of the several treatment modalities available at the present time, the most successful is application of continuous positive airway pressure during sleep."}, {"id": "pubmed23n0312_20110", "title": "[Therapy with nasal CPAP (continuous positive airway pressure) in patients with obstructive sleep apnea syndrome (OSAS). I: Long-term acceptance of nasal CPAP].", "score": 0.00909090909090909, "content": "Nocturnal ventilation with nCPAP has been established as the safest and most efficient nonsurgical treatment for OSAS. Long-term results, however, are determined by the patients' compliance with therapy. The aim of this study was the objective measurement of long-term acceptability of nCPAP therapy in all patients receiving this treatment in our sleep laboratory between January 1990 and March 1995. We prospectively investigated 41 patients (36 male, 5 female) with moderate to severe OSAS who received nCPAP therapy. Mean time of follow-up was 20.6 months, ranging from 1.2 to 53.5 months. Therapy was indicated when OSAS was confirmed by cardiorespiratory polygraphy and either (1) the patient complained of daytime sleepiness or (2) the patient possessed an apnea-hypopnea index greater than 30/h or when the mean oxygen desaturation was below 80% regardless of the presenting symptoms. The compliance with treatment was defined as a mean rate of use of over 5 hours per night calculated from the time counter on the nCPAP machine. 33 patients (88.5%) have continued using nCPAP until the present time but only 24 patients (59%) met our criteria for long-term acceptance and this group was identified as responders. We found no significant differences in age, body mass index, apnea-hypopnea index, and nCPAP-pressure between responders and non-responders. Although nCPAP is the safest treatment for OSAS, there is still a large group of patients with moderate to severe OSAS who are not efficiently treated with nCPAP because of the low long-term acceptability of this therapy. With respect to this group of patients, surgical approaches have to be considered as an alternative therapy."}, {"id": "pubmed23n0374_6598", "title": "[Comparative analysis of the clinical history and polysomnography in sleep disorders. Diagnostic relevance of polysomnography].", "score": 0.00909090909090909, "content": "The incidence of associate pathologies has been studied during the sleep, as well as the diagnostic efficiency of the clinical history. Patients (n = 136) remitted by diverse services, have been studied. It has been carried out a complete polysomnography, as well as other supplementary studies (anxiety and depression tests, excessive daytime sleepiness Epworth's test, EEG and sleep notebook). The most common symptom turned out to be the primary snores, followed by the excessive daytime sleepiness and apneas. The results of the excessive daytime sleepiness Epworth's test and the anxiety and depression tests were not useful to differ among pathologies, not even between pathologies and patients with normal sleep. The percentage of diagnosis of suspicion confirmed by the polysomnography was of 39.7%, while in 11% of the total of patients it was observed the existence of more than a pathology of the sleep. In 49.3% of the cases the polysomnographic diagnosis was completely different from the diagnosis of suspicion. Among the patients with clinic suspicion of apnoea, in 48.3% of the cases the existence of the same one was verified, although in 14.6% it was associated with other pathologies. In 51.7% of the patients it was not possible to confirm this pathology. The clinical history is not enough for the diagnosis of the pathologies of the sleep. On the other hand, the existence of associate pathologies diminishes the value of several 'screening-methods'. Therefore, it is fundamental to carry out a complete polysomnography in all the patients that present any sleep disorder on the part of doctors that approach the problem of the sleep in a global way and not only thinking in the possible existence of syndrome of sleep apnoea."}, {"id": "pubmed23n0681_3032", "title": "[Predictive value of clinical features and nocturnal oximetry for the detection of obstructive sleep apnea syndrome].", "score": 0.009009009009009009, "content": "Obstructive sleep apnea syndrome (OSA) is an important cause of morbidity and mortality in adults. To evaluate the diagnostic value of clinical features and oximetric data to screen for obstructive sleep apnea before performing polysomnograpy or respiratory polygraphy. We studied 328 consecutive adult patients referred for snoring or excessive daytime sleepiness to a sleep clinic in whom a standardized questionnaire and the Sleepiness Epworth Scale were performed and body mass index (BMI), cervical circumference (CC), and nocturnal oximetry were measured. Fifty three percent (n = 173) had evidence of clinically significant OSA (apnea/hypopnea index (AHI) > 15 events/h). Patients with OSA were more likely to be male, obese (BMI ≥ 26 kg/m²), smokers, to have a thick neck (CC > 41 cm), and to have a significant greater prevalence of relative reported apneas and excessive daytime sleepiness, as determined by Epworth scale. Male gender (Odds ratio (OR): 4.00; 95% confidence intervals (CI): 1.59-10.0, p = 0.003), BMI ≥ 26 kg/m² (OR: 3.68; 95%CI: 1.59-8.49, p = 0.002), smoking (OR: 2.29; 95% CI: 1.17-4.47, p = 0.015), Epworth index > 13 (OR: 2.65; 95% CI: 1.35-5.23, p = 0.005) and duration of symptoms over 2 years (OR: 2.35; 95% CI: 1.20-4.58, p = 0.012) were significant independent predictors of OSA. In nocturnal oximetry, the lowest SpO2 (SpO2 min) and the length of registries below 90% (CT-90) were independent predictors of OSA and both correlated significantly with AHI (r = -0.49 and r = 0.46 respectively, p < 0.001). No single factor was usefully predictive of obstructive sleep apnea. However, combining clinical features and oximetry data may be appropriate to detect clinically significant OSA patients."}, {"id": "pubmed23n0371_5026", "title": "[Remission of nocturnal pathological respiratory patterns after orthotopic heart transplantation. A case report and overview of current status of therapy].", "score": 0.008928571428571428, "content": "Cheyne-Stokes respiration is characterized by recurrent phases of central apneas during sleep alternating with a crescendo-decrescendo hyperventilation. This abnormal respiratory pattern is often observed in patients with severe congestive heart failure and associated with fragmentation of sleep, excessive daytime sleepiness, and a relatively high mortality. Increased peripheral and central chemosensitivity, prolonged circulation time, and reduced blood gas buffering capacity are the major factors contributing to the pathology. However, the exact pathophysiologic mechanisms are not clear yet. Respiratory stimulants, oxygen and continuous or bilevel positive airway pressure (CPAP or BiPAP) might reduce the severity of Cheyne-Stokes respiration but have little effect on daytime sleepiness and cardiac function. There is only limited data supporting the assumption that intensive heart failure therapy has an effect on Cheyne-Stokes respiration. A 55-year-old male patient with dilative cardiomyopathy (NYHA IV) suffered excessive daytime sleepiness (Epworth Sleepiness Scale: 24 points). The patient was a heavy snorer with a normal body mass index. Treatment was initiated including ACE-inhibitors, beta-receptor blockers, diuretics and digoxin. The patient underwent sleep analysis with a Somno-Check system which demonstrated Cheyne-Stokes breathing (Respiratory Disturbance Index RDI: 40/h, lowest desaturation 76%) and body position dependent snoring. Oxygen therapy (21/min) had no effect on daytime sleepiness. Due to the cardiac condition, the patient was accepted for heart transplantation. Three weeks after transplantation sleep analysis was repeated and demonstrated a lack of evidence for periodic breathing (RDI 1/h, no desaturations below 90%), while snoring remained unchanged. Daytime sleepiness improved significantly (Epworth Sleepiness Scale: 6 points). Three weeks after normalizing left ventricular function a complete recovery from severe Cheyne-Stokes respiration was observed. Adequate therapy of the underlying cause of Cheyne-Stokes breathing such as end-stage congestive heart failure might sufficiently abolish any breathing abnormalities."}]}}}} {"correct_option": 3, "explanations": {"1": {"exist": true, "char_ranges": [[43, 61]], "word_ranges": [[6, 10]], "text": "1 is not effective,"}, "2": {"exist": true, "char_ranges": [[63, 133]], "word_ranges": [[10, 21]], "text": "2 is not effective for an organic lesion with normal electromyography,"}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[134, 204]], "word_ranges": [[21, 34]], "text": "4 is not necessary since the lesion is only of the external sphincter,"}, "5": {"exist": true, "char_ranges": [[205, 288]], "word_ranges": [[34, 50]], "text": "5 is not indicated in a lesion that can be repaired with surgery with good results."}}, "full_answer": "The answer is surgical sphincteroplasty 3. 1 is not effective, 2 is not effective for an organic lesion with normal electromyography, 4 is not necessary since the lesion is only of the external sphincter, 5 is not indicated in a lesion that can be repaired with surgery with good results.", "full_answer_no_ref": "The answer is surgical sphincteroplasty [HIDDEN]. [HIDDEN], [HIDDEN], [HIDDEN], [HIDDEN].", "full_question": "A 26-year-old woman presenting with fecal incontinence after a prolonged instrumental delivery. An endoanal ultrasound was performed showing a section of the external anal sphincter of 30º amplitude. The electrophysiological study shows normal innervation. What is the most indicated treatment?", "id": 91, "lang": "en", "options": {"1": "Medical treatment with hygienic and dietary norms.", "2": "Sphincter biofeedback.", "3": "Surgical sphincteroplasty.", "4": "Surgical repair of the pelvic floor.", "5": "Artificial anal sphincter."}, "question_id_specific": 35, "type": "GENERAL SURGERY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en009_180416", "title": "Fecal incontinence", "score": 0.018543956043956044, "content": "Parks describes post anal repair, a technique to reinforce the pelvic floor and EAS to treat idiopathic cases. Endoanal ultrasound is invented in 1991, which starts to demonstrate the high number of occult sphincter tears following vaginal deliveries. In 1994, the use of an endoanal coil during pelvic MRI shows greater detail of the anal canal than previously. During the last 20 years, dynamic graciliplasty, sacral nerve stimulation, injectable perianal bulking agents and radiofrequency ablation have been devised, mainly due to the relatively poor success rates and high morbidity associated with the earlier procedures."}, {"id": "pubmed23n0505_7300", "title": "Randomized clinical trial of intra-anal electromyographic biofeedback physiotherapy with intra-anal electromyographic biofeedback augmented with electrical stimulation of the anal sphincter in the early treatment of postpartum fecal incontinence.", "score": 0.01786843769765971, "content": "The purpose of this study was to compare intra-anal electromyographic biofeedback alone with intra-anal biofeedback that was augmented with electrical stimulation of the anal sphincter in the treatment of postpartum fecal incontinence. A secondary aim was to examine the impact of the treatment on continence-related quality of life. Sixty symptomatic women were assigned randomly to receive intra-anal electromyographic biofeedback or electrical stimulation of the anal sphincter once weekly for 12 weeks and to perform daily pelvic floor exercises between treatments. Therapeutic response was evaluated with a symptom questionnaire to determine continence score, anal manometry, and endoanal ultrasound scanning. Quality of life was assessed before and after treatment with a validated questionnaire. Fifty-four women completed the treatment; 52 women (96%) had ultrasonic evidence of an external anal sphincter defect. After the treatment, both groups demonstrated significant improvement in continence score (P < .001) and in squeeze anal pressures (P < .04). Resting anal pressures did not alter significantly. Quality of life improved after the completion of physiotherapy, but there were no differences in outcome between intra-anal electromyographic biofeedback and electrical stimulation of the anal sphincter. Intra-anal electromyographic biofeedback therapy was associated with improved continence and quality of life in women with altered fecal continence after delivery. The addition of electrical stimulation of the anal sphincter did not enhance symptomatic outcome."}, {"id": "pubmed23n0324_13420", "title": "[Adult fecal incontinence due to anal sphincter lesions: which preoperative preparations? Which surgical solutions?].", "score": 0.017713490099009903, "content": "Management of fecal incontinence due to anal sphincter lesions involves a good preoperative evaluation. This tends to confirm the incontinence, to search its mechanism, and to classify it according to the type of sphincter lesion owing to manometry, ultrasonography, and defecography. The surgical option is discussed after failure of medical treatment and biofeedback. The aim of surgery is to restore the incontinence and to maintain the exemption function. The surgical procedures include the sphincter repairs, the sphincter substitution, or even colostomy. Among the procedures of sphincter repairs, the direct repair is performed for obstetrical ruptures or postsurgical lesions of the anal sphincter, and the pelvic floor repairs are performed for fecal incontinence with intact but poorly functioning sphincter. Failures of these conservative methods lead the surgeons to develop new techniques for anal sphincter substitution. Dynamic graciloplasty and artificial sphincter (both under evaluation) constitute currently the promising alternatives to colostomy."}, {"id": "pubmed23n0597_2997", "title": "[External anal sphincter repair using the overlapping technique in patients with anal incontinence and concomitant pudendal nerve damage].", "score": 0.01743396226415094, "content": "No single surgical technique has so far emerged as the optimal approach to treat defects of the anal sphincter in patients with postpartum fecal incontinence. Our approach is to repair the external sphincter using the overlapping technique to optimize morphological and clinical outcome. The results were correlated with preoperatively determined pudendal nerve function. Thirty-five patients were followed up for three years after repair of the external anal sphincter. The patients had grade 2 (n = 29) or grade 3 (n = 6) fecal incontinence. Nineteen (54 %) patients had a concomitant defect of the internal anal sphincter and 28 (80 %) had abnormal pelvic floor EMG findings. Before surgery, all patients underwent conservative treatment with biofeedback and electrostimulation. The muscle ends were overlapped with Vicryl 4-0 sutures. A standardized protocol was used for the perioperative management in all patients. Of the 35 patients who underwent overlapping repair of the external anal sphincter, 32 (91 %) had a satisfactory result at 3-year follow-up based on sonomorphological criteria. These 32 patients were continent for solid and liquid stools. Six of the 35 patients (17 %) continued to have flatus incontinence. Two (6 %) patients were improved and one patient (3 %) had unchanged incontinence. Pudendal nerve damage had no effect on the outcome of surgery. Our findings at 3-year follow-up show good results for the overlapping repair of the external anal sphincter in terms of morphology and clinical symptoms. This outcome depends on an adequate preoperative pelvic floor conditioning, optimal perioperative management, and use of a standardized operative technique. Surgical repair of the morphological defect is recommended even in patients with pudendal nerve damage."}, {"id": "pubmed23n0298_13996", "title": "[Diagnostic and therapeutic procedures in fecal incontinence in general practice of the surgically educated proctologist].", "score": 0.017105263157894735, "content": "Age related, about 10% of the general population suffer from faecal incontinence. In a surgical, proctological office diagnosis is possible with carefully taken history, physical examination, digital examination of the anorectum, rigid rectosigmoidoscopy, and anoscopy. Together with special examinations (endoanal ultrasound, electromyography, pudendal nerve terminal motor latency [PNTML], anorectal manometry, defaecography, transit time of the colon) the plan for medical and surgical treatment can be made. The basic medical conservative therapy consists of regulating the form of stool (high fibre diet and/or loperamid), training of the sphincter and pelvic muscles electrical stimulation or biofeedback training. Outpatient surgery is possible for small prolapsing tumors of the lower rectum or anal canal, hemorrhoids grade 2 or segmental anal prolapse. Inpatient surgery is needed for any form of reconstruction of the sphincter or the sensitive area of the anal canal, post- and preanal repair, anal and rectal prolapse, (dynamic) gracilis sphincteroplasty, or for a terminal stoma in those patients, whose uncontrolled incontinence cannot be managed otherwise. After surgery it is needed to continue the medical therapy (regulating the bowel movements, biofeedback training, electrical stimulation of the sphincter)."}, {"id": "pubmed23n0280_12053", "title": "Etiology and management of fecal incontinence.", "score": 0.01646787747357007, "content": "Fecal incontinence is a challenging condition of diverse etiology and devastating psychosocial impact. Multiple mechanisms may be involved in its pathophysiology, such as altered stool consistency and delivery of contents to the rectum, abnormal rectal capacity or compliance, decreased anorectal sensation, and pelvic floor or anal sphincter dysfunction. A detailed clinical history and physical examination are essential. Anorectal manometry, pudendal nerve latency studies, and electromyography are part of the standard primary evaluation. The evaluation of idiopathic fecal incontinence may require tests such as cinedefecography, spinal latencies, and anal mucosal electrosensitivity. These tests permit both objective assessment and focused therapy. Appropriate treatment options include biofeedback and sphincteroplasty. Biofeedback has resulted in 90 percent reduction in episodes of incontinence in over 60 percent of patients. Overlapping anterior sphincteroplasty has been associated with good to excellent results in 70 to 90 percent of patients. The common denominator between the medical and surgical treatment groups is the necessity of pretreatment physiologic assessment. It is the results of these tests that permit optimal therapeutic assignment. For example, pudendal nerve terminal motor latencies (PNTML) are the most important predictor factor of functional outcome. However, even the most experienced examiner's digit cannot assess PNTML. In the absence of pudendal neuropathy, sphincteroplasty is an excellent option. If neuropathy exists, however, then postanal or total pelvic floor repair remain viable surgical options for the treatment of idiopathic fecal incontinence. In the absence of an adequate sphincter muscle, encirclement procedures using synthetic materials or muscle transfer techniques might be considered. Implantation of a stimulating electrode into the gracilis neosphincter and artificial sphincter implantation are other valid alternatives. The final therapeutic option is fecal diversion. This article reviews the current status of the etiology and incidence of incontinence as well as the evaluation and treatment of this disabling condition."}, {"id": "pubmed23n0651_10644", "title": "Sacral nerve stimulation is a valid approach in fecal incontinence due to sphincter lesions when compared to sphincter repair.", "score": 0.015159747880895457, "content": "Anal sphincter lesions represent the major cause of fecal incontinence, particularly in women. Sphincteroplasty with overlap is the traditional treatment, but a significant reduction in benefits within 5 years of surgery has been reported. More recently, sacral nerve stimulation has been suggested following sphincteroplasty or as primary treatment. Overall, 24 women with fecal incontinence in the presence of anal sphincter lesions underwent sphincteroplasty (14 patients, mean age 47.6 +/- 15.6 years, range 26-70) or definitive implant of sacral nerve stimulation (10 patients, mean age 60.7 +/- 17.6 years, range 26-73), using identical selection criteria. At baseline, patients were studied with clinical evaluation, 3-dimensional endoanal ultrasound, and anorectal manometry (ARM), repeated at follow-up (median 60.0 months, range 6-96 in sphincteroplasty group; median 33.0 months, range 6-84 in sacral nerve stimulation group). At baseline, both groups presented similar characteristics. Two sphincteroplasty patients (14.3%) experienced relapse of fecal incontinence at 6 and 19 months after treatment, whereas good to excellent continence was observed in all of the sacral nerve stimulation patients. Compared to baseline, both groups showed a significant improvement in clinical parameters, and ARM data remained unchanged. In 12 of 14 sphincteroplasty patients, the repaired sphincter at endoanal ultrasound was found to overlap. At follow-up, comparison between sphincteroplasty and sacral nerve stimulation showed no significant differences in clinical and ARM parameters, if related to lesion of internal, external, or both sphincters. These data appear to confirm that sacral nerve stimulation could represent a valid alternative in the treatment of fecal incontinence patients presenting with sphincter lesion that was not preceded by sphincteroplasty."}, {"id": "wiki20220301en009_180415", "title": "Fecal incontinence", "score": 0.014634839443023587, "content": "While the first mention of urinary incontinence occurs in 1500 BC in the Ebers Papyrus, the first mention of FI in a medical context is unknown. For many centuries, colonic irrigation was the only treatment available. Stoma creation was described in 1776, FI associated with rectal prolapse in 1873 and anterior sphincter repair in 1875. During the mid 20th Century, several operations were developed for instances where the sphincters were intact but weakened. Muscle transpositions using the gluteus maximus or the gracilis were devised, but did not become used widely until later. End-to-end sphincteroplasty is shown to have a high failure rate in 1940. In 1971, Parks and McPartlin first describe an overlapping sphincteroplasty procedure. Biofeedback is first introduced in 1974. In 1975, Parks describes post anal repair, a technique to reinforce the pelvic floor and EAS to treat idiopathic cases. Endoanal ultrasound is invented in 1991, which starts to demonstrate the high number of"}, {"id": "pubmed23n0419_17025", "title": "[Diagnosis and conservative therapy of anal fecal incontinence].", "score": 0.014578947368421052, "content": "The main diagnostic tool for patients with anal incontinence is the anorectal physiology. The anorectal sphincter can be reliably tested. Due to the fact that an obstetric damage is the most common cause of anal incontinence, the transanal endosonography is the imaging method of choice for detecting a muscle defect. With high frequency ultrasound probes (preferable 10 Mhz) the pathomorphology of the sphincter can be studied in details. If conservative treatment is failing, the neurophysiology testing is helpful in deciding which surgical method (sphincter repair in case of an intact nervus pudendus, artificial sphincter or sacral stimulation in case of neuropathy). The conservative treatment includes increasing the internal sphincter muscle tonus by applying Phenylephrine locally or Loperamid per os. The biofeedback is basically a physical muscle training of the external sphincter with a visual or acoustic feedback of the muscle function to the patient. However, newer studies show that by this method the sensory function and the coordination of the anal sphincter are improving as well. With these conservative treatment options most of the patients suffering from anal incontinence can be treated satisfactorily."}, {"id": "pubmed23n0479_21743", "title": "[The treatment of fecal incontinence].", "score": 0.014266435319066899, "content": "The treatment of faecal incontinence includes: the education of the patient, medical therapy, biofeedback and sphincteric exercises, surgical therapy. Conservative, non-surgical treatment is almost always the initial therapeutic approach, except in those cases in which an evident defect of the sphincter muscle is present. Surgical treatment has seen a noteworthy increase in the last fifteen years as a consequence of the development of new surgical techniques. These techniques include: external anal sphincter plasty, pelvic floor plasties, sacral neuromodulation, muscular transpositions with or without electrostimulation, artificial anal sphincter. These procedures may be employed as first or second level treatment depending on the type of pathology considered and its aetiology. The 1st results achieved by surgical treatment authorise us to believe that reconversion with artificial sphincter is a valid alternative to graciloplasty, notwithstanding the fact that its costs are higher. Attentive pre- operative assessment of patients is important. Patients must be strongly motivated and able to manage the new condition. Although further studies are necessary, the degree of satisfactory of the 1st patients operated is the best stimulus for pursuing the development of this technique."}, {"id": "pubmed23n0614_26026", "title": "[Reconstruction of anal sphincters following fecal incontinence and assessment of functional results].", "score": 0.014143920595533498, "content": "The aim of the study was to assess functional results of surgical sphincter reconstructions for anal incontinence. From August 1999 to January 2007, 52 patients (females 50, males 2), 45 y.o.a. on average (24-69), underwent secondary anal sphincters reconstructions for fecal incontinence, resulting from birth injuries, event. in combination with sphincter weakening in pudendal neurophathy, or for post-anorectal surgery incontinence. Duration of the incontinence symptoms prior to the surgery was 2 months to 19 years. The overlap technique in combination with anterior levatorplasty was used in most subjects (n = 31). In 12 subjects, anterior sphincters and levators plication was performed. Four patients underwent overlap reconstructions only and five patients underwent complete sphincter reconstructions. Protective colostomy was performed in six patients. Six patients underwent additional postoperative biofeedback. Endoanal ultrasound was performed in all patients prior to their procedures. Terminal motor latency examination of the pudendal nerve was indicated in all patients with sphincter dysfunction without localized defects. Anal manometry was recorded prior and post-operatively. Incontinence was assessed using the St. Mark's incontinence score (0-13). The patients assessed the reconstruction results based on the Likert scale. The reconstruction was successfull in 46 patients (88.5 %), full continence was recovered in 20 (38.5 %) patients and improvement of incontinence was recorded in 26 (50 %) subjects. In six subjects (11.5 %), the reconstruction failed. The mean incontinence score reduction following the procedure was from 11.8 to 2.4. The short-term sphincter reconstruction results were successful in the majority of the subjects, the long-term results will be assessed in another study."}, {"id": "wiki20220301en009_180410", "title": "Fecal incontinence", "score": 0.01405656395103419, "content": "Surgery may be carried out if conservative measures alone are not sufficient to control incontinence. There are many surgical options, and their relative effectiveness is debated due to a lack of good quality evidence. The optimal treatment regime may be a both surgical and non-surgical treatments. The surgical options can be considered in four categories: restoration and improvement of residual sphincter function (sphincteroplasty, sacral nerve stimulation, tibial nerve stimulation, correction of anorectal deformity), replacement / imitation of the sphincter or its function (anal encirclement, SECCA procedure, non-dynamic graciloplasty, perianal injectable bulking agents), dynamic sphincter replacement (artificial bowel sphincter, dynamic graciloplasty), antegrade continence enema (Malone procedure), and finally fecal diversion (e.g. colostomy). A surgical treatment algorithm has been proposed. Isolated sphincter defects (IAS/EAS) may be initially treated with sphincteroplasty and if"}, {"id": "wiki20220301en399_33268", "title": "Surgical management of fecal incontinence", "score": 0.013982004234297813, "content": "improves FI symptoms in the short term in most (50-80%) patients with sphincter defects. Thereafter, continence deteriorates. Most who undergo this operation are incontinence again after 5 years. Poor results with this procedure may be related to pelvic floor denervation (nerve damage). Primary sphincter repair is inadequate in most women with obstetric ruptures following vaginal delivery. Residual sphincter defects remain in most and around 50% remain incontinent. Residual sphincter defect following the operation (as demonstrated by endoanal ultrasonography) then the procedure may be repeated."}, {"id": "wiki20220301en399_33264", "title": "Surgical management of fecal incontinence", "score": 0.013966030749416405, "content": "The relative effectiveness of surgical options for treating fecal incontinence is not known. A combination of different surgical and non-surgical therapies may be optimal. A surgical treatment algorithm has been proposed for FI, although this did not appear to include some surgical options. Isolated sphincter defects may be initially treated with sphincteroplasty and if this fails, the patient can be assessed for sacral nerve stimulation. Functional deficits of the external anal sphincter (EAS) and/or internal anal sphincter (IAS), i.e. where there is no structural defect, or only limited EAS structural defect, or with neurogenic incontinence, may be assessed for sacral nerve stimulation. If this fails, neosphincter with either dynamic graciloplasty or artificial anal sphincter may be indicated. Substantial muscular and/or neural defects may be treated with neosphincter initially."}, {"id": "pubmed23n0359_6191", "title": "Are sphincter defects the cause of anal incontinence after vaginal delivery? Results of a prospective study.", "score": 0.013739961936591933, "content": "Anal incontinence affects approximately 10 percent of adult females. Damage to the anal sphincters has been considered as the cause of anal incontinence after childbirth in the sole prospective study so far available. The aims of the present study were to determine prospectively the incidence of anal incontinence and anal sphincter damage after childbirth and their relationship with obstetric parameters. We studied 259 consecutive females six weeks before and eight weeks after delivery. They were asked to fill in a questionnaire assessing fecal incontinence. Anal endosonography (7-10 MHz) was then performed. Two independent observers analyzed internal and external anal sphincters. A total of 233 patients (90 percent) were assessed, of whom 31 had cesarean section. De novo sphincter defects were observed in 16.7 percent (14 percent external, 1.7 percent internal, and 1 percent both) in the postpartum period only after vaginal delivery. These disruptions occurred with the same incidence after the first and the second childbirth. Independent risk factors (odds ratio; 95 percent confidence interval) for sphincter defect were forceps (12; 4-20), perineal tears (16; 9-25), episiotomy (6.6; 5-17), and parity (8.8; 4-19) as revealed by multivariate analyses. The overall rate of anal incontinence was 9 percent and independent risk factors (odds ratio; 95 percent confidence interval) involved forceps (4.5; 1.5-13), perineal tears (3.9; 1.4-10.9), sphincter defect (5.5; 5-15), and prolonged labor (3.4; 1-11). Among these patients only 45 percent had sphincter defects. Anal incontinence after delivery is multifactorial, and anal sphincter defects account for only 45 percent of them. Primiparous and secundiparous patients have the same risk factors for sphincter disruption and anal incontinence. Because external anal sphincter disruptions are more frequent than internal anal sphincter damage, surgical repair should be discussed in symptomatic patients."}, {"id": "pubmed23n0367_16572", "title": "Postanal repair for fecal incontinence--is it worthwhile?", "score": 0.013499064421277733, "content": "Patients with idiopathic or neurogenic incontinence without an isolated sphincter defect may be suitable candidates for a postanal repair. The aim of this study was to assess the results of postanal repair in patients with idiopathic or neurogenic fecal incontinence and to evaluate the role of various parameters, including preoperative physiologic testing on outcome. Postanal repair was offered by a single surgeon to patients meeting the following criteria: incontinence score of at least 12 of 20, absence of an isolated anterior external anal sphincter defect, and failed conservative, medical, and biofeedback management. Physiologic investigation and clinical findings of female patients who had postanal repair for fecal incontinence between 1992 and 1998 were reviewed. Physiologic investigation included anorectal manometry, pudendal nerve terminal motor latency, concentric needle electromyography, and endoanal ultrasonography. Follow-up was obtained by telephone questionnaire; moreover, patients were asked to grade the outcome of their surgery as excellent or good (success) or as fair or poor (failure). Twenty-one patients of median age 68 (range, 40-80) years had a mean duration of fecal incontinence before postanal repair of 6.8 (range, 0.5-22) years. Twenty patients (95 percent) were available for at least one year of follow-up. Seventeen patients (80.9 percent) had at least one prior vaginal delivery, and prior sphincteroplasty had been performed in 10 patients (47.6 percent). The morbidity and mortality rates were 5 and 0 percent, respectively. After a mean follow-up period of three (range, 1-7.5) years, seven patients (35 percent) considered surgery to be successful and had a statistically significant decrease in their incontinence score. Neither prolongation of pudendal nerve terminal motor latency nor external sphincter damage as noted on electromyography or any of the preoperative manometric parameters correlated with outcome. Furthermore, patients' ages at surgery did not correlate with the degree of postoperative improvement in continence scores nor did the duration of the patients' symptoms, number of vaginal deliveries, or a history of previous surgery for fecal incontinence. None of the factors assessed was demonstrated to be predictive of outcome after postanal repair; moreover, the currently available preoperative testing has not altered the success rate, which remains low (35 percent). Despite the low success rate, the absence of any mortality and the low morbidity suggest that postanal repair may be a valid therapeutic approach. However, it should be offered only to selected patients with persistent, severe fecal incontinence despite an anatomically intact external anal sphincter who are not candidates for or refuse all other operative modalities."}, {"id": "pubmed23n0735_12953", "title": "[The artificial sphincter: therapy for faecal incontinence].", "score": 0.013251651025373653, "content": "Faecal incontinence (FI) challenges a patient's professional, social and sexual life. Often the patient becomes depressive and socially isolated. If able to break open for therapy the patient should receive as first line a conservative treatment (like dietary measures, pelvic re-education, biofeedback, bulking agents, irrigation). When is the time to implant an artificial anal sphincter? If conservative therapy fails as well as surgical options (like a sphincteroplasty - if indicated a reconstruction of the pelvic floor if insufficient, or a sacral nerve stimulation) an ultimo surgical procedure should be offered to appropriate and compliant patients: an artificial anal sphincter. Worldwide, there are two established devices on the market: the artificial bowel sphincter® (ABS) from A. M. S. (Minnetonka, MN, USA) and the soft anal band® from A. M. I. (Feldkirch, Austria). How to implant the artificial anal sphincter? Both devices consist of a silicon cuff which can be filled with fluid. Under absolute aseptic conditions this cuff is placed in the lithotomy position by perianal incisions around the anal canal below the pelvic floor. A silicon tube connects the anal cuff with a reservoir (containing fluid) which is placed either behind the pubis bone in front of the bladder (ABS) or below the costal arch (anal band). With a pump placed in the scrotum/labia (ABS) or by pressing the balloon (anal band) in both types operated by the patient the fluid is shifted forth and back between the anal cuff and the reservoir closing or opening the anal canal. Both systems are placed completely subcutaneously. Both devices improve significantly the anal continence. Both systems have a high rate of reoperations. However, the causes for the redos are different. The ABS is associated with high infection and anal penetration rates of the cuff leading to an explantation rate to up to 60 % of the implants. This kind of complication seems to be much lower with the anal band. The major problem in the anal band is a defunctioning valve which occasionally has to be replaced. Despite these problems both types of artificial anal sphincters improve faecal incontinence significantly and, thus, quality of life of incontinent patients."}, {"id": "wiki20220301en009_180397", "title": "Fecal incontinence", "score": 0.013061540628080551, "content": "FI is generally treatable with conservative management, surgery or both. The success of treatment depends upon the exact causes and how easily these are corrected. Treatment choice depends on the cause and severity of disease, and the motivation and general health of the person affected. Commonly, conservative measures are used together, and if appropriate surgery carried out. Treatments may be attempted until symptoms are satisfactorily controlled. A treatment algorithm based upon the cause has been proposed, including conservative, non-operative and surgical measures (neosphincter refers to either dynamic graciloplasty or artificial bowel sphincter, lavage refers to retrograde rectal irrigation). Conservative measures include dietary modification, drug treatment, retrograde anal irrigation, biofeedback retraining anal sphincter exercises. Incontinence products refer to devices such as anal plugs and perineal pads and garments such as diapers/nappies. Perineal pads are efficient and"}, {"id": "wiki20220301en009_180411", "title": "Fecal incontinence", "score": 0.01295191295191295, "content": "procedure), and finally fecal diversion (e.g. colostomy). A surgical treatment algorithm has been proposed. Isolated sphincter defects (IAS/EAS) may be initially treated with sphincteroplasty and if this fails, the person can be assessed for sacral nerve stimulation. Functional deficits of the EAS and/or IAS (i.e. where there is no structural defect, or only limited EAS structural defect, or with neurogenic incontinence) may be assessed for sacral nerve stimulation. If this fails, neosphincter with either dynamic graciloplasty or artificial anal sphincter may be indicated. Substantial muscular and/or neural defects may be treated with neosphincter initially."}, {"id": "wiki20220301en399_33267", "title": "Surgical management of fecal incontinence", "score": 0.012355110642781875, "content": "antibiotics. Once the patient is under anesthesia, an incision is made in front of the anus (the anterior perineum). Scar tissue is removed and the mucosa of the anal canal separated from the damaged sphincter. The sphincter is cut and its ends overlapped and then stitched back together. The exact method of the procedure varies, e.g. the cut sphincter may be stitched back end to end, rather than overlapped, or the IAS and EAS may be repaired as separate stages. Sphincter repair may sometimes be combined with an anterior levatorplasty (an operation to tighten the pelvic floor). A surgical drain is left to prevent buildup of fluid. After the operation, sitz baths are recommended to maintain hygiene during healing, and laxatives prescribed to avoid hard stool. Overlapping anterior sphincteroplasty improves FI symptoms in the short term in most (50-80%) patients with sphincter defects. Thereafter, continence deteriorates. Most who undergo this operation are incontinence again after 5"}, {"id": "pubmed23n0481_13279", "title": "Primary sphincter repair: are the results of the operation good enough?", "score": 0.012284644194756555, "content": "This study was designed to evaluate the clinical outcome of primary anal sphincter repair caused by obstetric tears and to analyze possible risk factors associated with sphincter rupture during vaginal delivery. A total of 52 females with a third-degree or fourth-degree perineal laceration during vaginal delivery were examined. The symptoms of anal incontinence were obtained by a standard questionnaire. In addition to a clinical examination, endoanal ultrasound, anal manometry, and pudendal nerve terminal motor latency examinations were performed. A control group consisted of 51 primiparous females with no clinically detectable perineal laceration after vaginal delivery. After primary sphincter repair, 31 females (61 percent) had symptoms of anal incontinence. Fecal incontinence occurred in 10 females (20 percent). According to Hardcastle and Parks' and Jorge and Wexner's classifications, the study group had more severe symptoms of anal incontinence than the control group (P<0.001 in both classification groups). In endoanal ultrasound examination, a persistent defect of the external anal sphincter was found in 39 females (75 percent) in the rupture group compared with 10 females (20 percent) in the control group (P<0.001). Anal sphincter pressures were significantly lower in the rupture group than in the control group. An abnormal presentation was the only risk factor for anal sphincter rupture during vaginal delivery. After primary sphincter repair, persistent external anal sphincter defect and symptoms of anal incontinence are common in females who have had a primary sphincter repair after vaginal delivery. The means of improving the results of primary repair should be studied further."}, {"id": "wiki20220301en399_33262", "title": "Surgical management of fecal incontinence", "score": 0.012219477584485204, "content": "In fecal incontinence (FI), surgery may be carried out if conservative measures alone are not sufficient to control symptoms. There are many surgical options described for FI, and they can be considered in 4 general groups. Restoration and improvement of residual sphincter function sphincteroplasty (sphincter repair) Correction of anorectal deformities that may be contributing to FI Sacral nerve stimulation Replacement / imitation of the sphincter or its function Narrowing of anal canal to increase the outlet resistance without any dynamic component Anal encirclement (Thiersch procedure) Radiofrequency ablation (\"Secca procedure\") Nondynamic graciloplasty (\"bio-Thiersch\") Implantation/injection of microballoons, carbon-coated beads, autologous fat, silicone, collagen. Dynamic sphincter replacement Implantation of artificial bowel sphincter (neosphincter) Dynamic graciloplasty Antegrade continence enema (ACE)/ antegrade colonic irrigation"}, {"id": "Surgery_Schwartz_8380", "title": "Surgery_Schwartz", "score": 0.012164817383263984, "content": "and defecography can detect rectal prolapse. Endoanal ultrasound is invaluable in diagnosing sphincter defects (Fig. 29-8).Therapy depends on the underlying abnormality. Diarrhea should be treated medically (fiber, antidiarrheal agents). Even in the absence of frank diarrhea, the addition of dietary fiber may improve continence. Some patients may respond to bio-feedback and this approach may be considered in patients who fail dietary modification. Many patients with a sphincter defect are candidates for an overlapping sphincteroplasty. Sacral nerve Brunicardi_Ch29_p1259-p1330.indd 126923/02/19 2:28 PM 1270SPECIFIC CONSIDERATIONSPART IIstimulation been shown to decrease episodes of fecal incon-tinence and has proven durability in the long term (5 years). The artificial bowel sphincter may be useful in patients who fail other interventions. Other options include radiofrequency energy to the anal canal (SECCA procedure), magnetic anal sphincter, and injectable submucosal bulking"}, {"id": "pubmed23n0344_13364", "title": "Pudendal neuropathy and severity of incontinence but not presence of an anal sphincter defect may determine the response to biofeedback therapy in fecal incontinence.", "score": 0.01205519244734931, "content": "It has been suggested that the severity of fecal incontinence, the presence of pudendal neuropathy, or an external anal sphincter defect does not preclude clinical improvement with biofeedback therapy. A discrepancy, however, is frequently found between subjective improvement and objective results after biofeedback therapy. Our aim was to assess whether severity of fecal incontinence, presence of pudendal neuropathy, or an external anal sphincter defect could influence the results of manometric parameters after biofeedback therapy in patients with fecal incontinence. Biofeedback therapy was used to treat 27 patients with fecal incontinence (25 women; mean age, 53; range, 29-74 years), according to a strict protocol. Manometry, pudendal nerve terminal motor latency, and anal ultrasound were performed in all patients before biofeedback therapy. Manometric evaluation of external anal sphincter function was performed after the biofeedback sessions. Eight of 27 patients had a good clinical response to biofeedback, but with no significant difference in their mean amplitude and duration of squeeze pressure before and after biofeedback. There was no relationship between the clinical results of biofeedback therapy and the initial severity of fecal incontinence, pudendal neuropathy, or external sphincter defect. Patients with severe incontinence (incontinence to solids) and pudendal neuropathy failed to improve the amplitude and duration of their maximum voluntary contraction after biofeedback therapy. Patients with mild fecal incontinence (incontinence to flatus, liquids, or both) (P<0.04), without pudendal neuropathy (P<0.02), or with (P<0.05) and without (P<0.05) external sphincter defect improved their external anal sphincter function after biofeedback therapy. In patients with fecal incontinence, the severity of symptoms and pudendal neuropathy should be considered as two factors of poor prognosis of favorable manometric results after biofeedback therapy. Improvement, on the other hand, may be expected after biofeedback therapy despite an external anal sphincter defect."}, {"id": "pubmed23n0521_17435", "title": "Diagnosis of anal sphincter tears to prevent fecal incontinence: a randomized controlled trial.", "score": 0.011970730321113954, "content": "Maternal anal sphincter tears after vaginal delivery are frequently not diagnosed clinically and are associated with subsequent fecal incontinence. This study examined whether diagnosis of these tears by ultrasonography, followed by immediate surgical repair, reduces the occurrence of incontinence. We conducted a randomized trial involving 752 primiparous women without a clinically evident anal sphincter tear to evaluate the benefit of adding endoanal ultrasonography immediately after vaginal delivery to the standard clinical examination of the perineum. When a sphincter tear was diagnosed, the perineum was surgically explored and the sphincter sutured. The main outcome evaluated was fecal incontinence 3 months postpartum graded by the Wexner incontinence scale, which measures incontinence to flatus and liquid or solid stools, need to wear a pad, and lifestyle alterations. Among women assessed by ultrasonography, 5.6% had a sphincter tear. Severe incontinence was reported 3 months after childbirth by 3.3% of women in the intervention group compared with 8.7% in the control group (risk difference -5.4%; 95% confidence interval -8.9 to -2.0; P = .002). The benefit of the intervention persisted 1 year after delivery, with 3.2% severe incontinence in the intervention group compared with 6.7% in the control group (risk difference -3.5%; 95% confidence interval -6.8% to -0.3%; P = .03). Ultrasonography needs to be performed in 29 women to prevent 1 case of severe fecal incontinence. Ultrasound examination of the perineum after childbirth improves the diagnosis of anal sphincter tears, and their immediate repair decreases the risk of severe fecal incontinence. I."}, {"id": "wiki20220301en328_9020", "title": "Anorectal manometry", "score": 0.011961722488038277, "content": "Medical Uses Fecal Incontinence After eliminating structural causes of fecal incontinence from the differential diagnosis, anorectal manometry may be used to evaluate deficiencies in sphincter function or anorectal sensation. An abnormal resting pressure or squeeze pressure may indicate problems with either the internal anal sphincter or the external anal sphincter respectively. Both increased and decreased anorectal sensation has also been detected in individuals with fecal incontinence. The use of HD-ARM can allow recognition of pressure asymmetry within the anorectum. Some patients with fecal incontinence benefit from muscle strength training which may make use of anorectal biofeedback."}, {"id": "pubmed23n0420_22488", "title": "Direct repair vs. overlapping sphincter repair: a randomized, controlled trial.", "score": 0.011241953210865128, "content": "The aim of this study was to compare the results of two surgical techniques (direct end-to-end vs. overlapping) of delayed repair of a localized anterior defect of external anal sphincter after an obstetric trauma. During a five-year period, 23 patients were randomly assigned to direct end-to-end repair (n = 12) or overlapping sphincter repair (n = 11), using 2-0 PDS sutures. Two patients from each group had an internal anal sphincter defect that also was repaired. All patients had a normal pudendal nerve terminal motor latency preoperatively. Evaluations included endoanal ultrasound, anorectal manometry, and neurophysiologic evaluation. Continence was assessed by the Cleveland Clinic Continence Score (0-20; 0, perfect continence; 20, complete incontinence). The two groups were comparable with regard to age (median, 45 years), past history of sphincter repair (n = 2), and posterior vaginal repair. There was no major morbidity. The wound-healing rate was identical between the two groups. However, of the patients undergoing overlapping repair, two had fecal impaction, and one had a urinary retention. Median preoperative continence score was 17 in both the direct-repair group (score, 8-20) and the overlap group (score, 7-20). At a median follow-up of 18 months, the improvement in continence was similar between the two surgical groups, with a median continence score of 3, respectively. In both surgical groups there was a significant and similar improvement in maximum squeeze pressure and in the functional anal canal length postoperatively (P < 0.05), but the mean resting pressure was relatively unchanged. In the overlap group, one patient developed a unilaterally prolonged pudendal nerve terminal motor latency that was persistent 22 months after surgery, and two patients had impaired fecal evacuation postoperatively. This randomized, controlled study suggests that the outcome is similar whether direct end-to-end or overlapping repair of a sphincter defect is performed. Overlapping repair might be associated with more difficulties with fecal evacuation and a prolonged pudendal nerve terminal motor latency postoperatively."}, {"id": "pubmed23n0781_17087", "title": "[Surgical treatment of anal incontinence].", "score": 0.01101301810564118, "content": "An epidemiologic assessment of the frequency of anal incontinence and the assessment of the social and economic impact of this handicap allowed progress of the surgery: muscular repair by myorraphy, mainly posterior myorraphy (postanal repair) or sphincteroplasty by direct suture of external anal sphincter, development of an invasive surgery by implantation of a neosphincter (artificial anal sphincter), development of mini-invasive surgery by sacral nerve stimulation, failure of mini-invasive procedures by injection of a bulking agent or radiofrequency, development of cellular therapy by injection of autologous myoblasts. In the same time, progress of digestive functional explorations (anorectal manometry, electrophysiological tests, endoanal ultra sonography, MRI, colonic transit time) and a better knowledge of colonic and ano-rectal physiology improved the surgical thought."}, {"id": "Surgery_Schwartz_8379", "title": "Surgery_Schwartz", "score": 0.010910962429233145, "content": "most common Figure 29-8. A. Endoanal ultrasonography showing the normal layers of the anal canal. B. Endoanal ultrasonography with anterior sphincter defect from birthing injury. EAS = external anal sphincter; IAS = internal anal sphincter. (Used with permission from Charles O. Finne III, MD, Minneapolis, MN.)ABtraumatic cause of incontinence is injury to the anal sphincter during vaginal delivery. Other causes include anorectal surgery, impalement, and pelvic fracture.After a thorough medical evaluation to detect underly-ing conditions that might contribute to incontinence, evalua-tion focuses on assessment of the anal sphincter and pudendal nerves. Pudendal nerve terminal motor latency testing may detect neuropathy. Anal manometry can detect low resting and squeeze pressures. Physical examination and defecography can detect rectal prolapse. Endoanal ultrasound is invaluable in diagnosing sphincter defects (Fig. 29-8).Therapy depends on the underlying abnormality. Diarrhea should be"}, {"id": "wiki20220301en399_33266", "title": "Surgical management of fecal incontinence", "score": 0.010903794678003464, "content": "This operation aims to repair sphincter defects (which may be of unknown cause) or damage from trauma (usually caused by obstetric damage). Where the sphincter has separated from a tear, this procedure brings these ends back together. Primary sphincteroplasty is repair carried out soon after the trauma has occurred, whilst other repairs may be carried out years after the original trauma (secondary or delayed sphincter repair), usually because the trauma went unrecognised. Usually, sphincter defects are in the anterior position on the sphincter, when an anterior sphincteroplasty may be carried out. Where the sphincter defect is laterally or posteriorly placed, this carries a less successful outcome. Overlapping anterior sphincteroplasty is preceded by a bowel preparation and possibly antibiotics. Once the patient is under anesthesia, an incision is made in front of the anus (the anterior perineum). Scar tissue is removed and the mucosa of the anal canal separated from the damaged"}, {"id": "pubmed23n1144_8224", "title": "Multimodal Management of Fecal Incontinence Focused on Sphincteroplasty: Long-Term Outcomes from a Single Center Case Series.", "score": 0.010843268137697846, "content": "The management of patients with fecal incontinence and an external anal sphincter (EAS) defect remains controversial. A retrospective series of overlapping anal sphincteroplasties performed between 1985-2013 from a single center, supplemented by selective puborectalis plication and internal anal sphincter repair is presented. Patients were clinically followed along with anorectal manometry, continence scoring (Cleveland Clinic Incontinence Score-CCS) and patient satisfaction scales. Patients with a suboptimal outcome were managed with combinations of biofeedback therapy (BFT), peripheral tibial nerve stimulation (PTNS), sacral nerve stimulation (SNS) or repeat sphincteroplasty. There were 120 anterior sphincter repairs with 90 (75%) levatorplasties and 84 (70%) IAS repairs. Over a median follow-up of 120 months (IQR 60-173.7 months) there were significant improvements in the recorded CCIS values (90.8% with a preoperative CCIS &gt; 15 vs. 2.5% postoperatively; ALT); may result in death D. Cirrhosis is a complication of long-term, chronic alcohol-induced liver damage; occurs in 10-20% of alcoholics V. A. Fatty change, hepatitis, and/or cirrhosis that develop without exposure to alcohol (or other known insult) B. Associated with obesity C. Diagnosis of exclusion; ALT > AST VI. HEMOCHROMATOSIS"}, {"id": "pubmed23n0246_1005", "title": "Nonalcoholic steatohepatitis: Mayo Clinic experiences with a hitherto unnamed disease.", "score": 0.011185474375658425, "content": "Nonalcoholic steatohepatitis is a poorly understood and hitherto unnamed liver disease that histologically mimics alcoholic hepatitis and that also may progress to cirrhosis. Described here are findings in 20 patients with nonalcoholic steatohepatitis of unknown cause. The biopsy specimens were characterized by the presence of striking fatty changes with evidence of lobular hepatitis, focal necroses with mixed inflammatory infiltrates, and, in most instances, Mallory bodies; Evidence of fibrosis was found in most specimens, and cirrhosis was diagnosed in biopsy tissue from three patients. The disease was more common in women. Most patients were moderately obese, and many had obesity-associated diseases, such as diabetes mellitus and cholelithiasis. Presence of hepatomegaly and mild abnormalities of liver function were common clinical findings. Currently, we know of no effective therapy."}]}}}} {"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[103, 235]], "word_ranges": [[20, 39]], "text": "Proto-oncogene carriers usually have early thyroid tumors so radical thyroidectomy is usually recommended before the age of 5 years."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "The correct answer is 4. It seems to me to be more of an endocrine question than a pediatric question. Proto-oncogene carriers usually have early thyroid tumors so radical thyroidectomy is usually recommended before the age of 5 years. Obviously I had to look this up...it is not very common in our routine practice.", "full_answer_no_ref": "[HIDDEN] It seems to me to be more of an endocrine question than a pediatric question. Proto-oncogene carriers usually have early thyroid tumors so radical thyroidectomy is usually recommended before the age of 5 years. Obviously I had to look this up...it is not very common, in our routine practice.", "full_question": "A 5-year-old girl, completely asymptomatic, who comes to the pediatrician for a regular check-up. She consults because her mother was operated on for a thyroid tumor and a grandmother died 10 years ago from a pheochromocytoma. The priority therapeutic attitude we will adopt will be:", "id": 50, "lang": "en", "options": {"1": "Exhaustive physical examination with blood pressure control to assess whether the child may have a familial disease.", "2": "Biochemical analysis with calcitonin. If it is normal, no further control is necessary at this age.", "3": "Annual monitoring of calcitonin levels with pentagastrin stimulation and, if elevated, indication of prophylactic thyroidectomy.", "4": "Genetic study of the RET c634 proto-oncogene mutation, and if positive, prophylactic radical thyroidectomy will be performed at this age.", "5": "Genetic study of the RET c634 proto-oncogene mutation, calcitonin levels and FNA (fine needle aspiration) and if positive, radical thyroidectomy will be performed."}, "question_id_specific": 160, "type": "PEDIATRICS", "year": 2011, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0551_7266", "title": "Late-onset medullary carcinoma of the thyroid: need for genetic testing and prophylactic thyroidectomy in adult family members.", "score": 0.019232547387887194, "content": "Sporadic late-onset medullary carcinoma of the thyroid is quite rare. Usually, the patient presents with a thyroid mass or neck node metastasis along with high levels of calcitonin and preoperative fine needle aspiration biopsy suggestive of medullary carcinoma of the thyroid. The role of genetic testing in such individuals, along with testing of other family members, remains somewhat unclear at this stage, especially in patients presenting with familial medullary thyroid carcinoma. Genetic testing with RET proto-oncogene mutational studies is very popular in familial medullary thyroid carcinoma, especially in children, with routine prophylactic thyroidectomy. However, its indications in adults remain unclear at this time. Recently, a 69-year-old woman presented with a thyroid mass and underwent total thyroidectomy and central compartment dissection. She was found to have medullary carcinoma of the thyroid. The patient had four children, three of whom were found to have a RET mutation similar to their mother's, V804M. In view of the RET mutation, the three children were offered prophylactic thyroidectomy at ages 42, 45, and 47. The patient's son was noted to have extensive C-cell hyperplasia in both lobes of the thyroid. The other two individuals had benign pathology with no evidence of C-cell hyperplasia. There is no definite consensus of opinion about the need for prophylactic total thyroidectomy in adults with RET mutation. The rarely reported 804 mutation is, however, a predictor of medullary carcinoma of the thyroid. One individual in this group had extensive C-cell hyperplasia, suggesting that he would have developed medullary carcinoma of the thyroid in the future. Prophylactic thyroidectomy should be recommended in patients with RET mutation and a family history of medullary carcinoma of the thyroid; however, its role in adult family members needs to be evaluated with larger registry of prophylactic thyroidectomy. Whether these adults with rare 804-mutation could be observed and followed with serial calcitonin, ultrasound, or calcitonin stimulation tests remains to be studied."}, {"id": "pubmed23n0531_3894", "title": "[Prophylactic thyroidectomy in children and young people with hereditary medullary thyroid carcinoma: a Chilean experience].", "score": 0.018978233495232956, "content": "With the availability of the RET proto-oncogene genetic testing, it is possible to perform prophylactic total thyroidectomy among carriers of RET mutation. To evaluate the histological findings and the effects of the prophylactic total thyroidectomy in first-degree relatives of Chilean patients with multiple endocrine neoplasia type 2 (MEN 2) based on the Ret proto-oncogene analysis. Nineteen patients belonging to 11 MEN 2 families underwent total thyroidectomy. Of these, 16 either with C cell hyperplasia (CCH) or microscopic medullary thyroid carcinoma (MTC) were selected for the final analysis. The age at the moment of thyroidectomy ranged from 3 to 24 years (median 9.5). The most common mutation was located in codon 634 (69%) followed by codon 620 (25%). Histopathology revealed MTC in 13 patients (81%, youngest 3 years, oldest ones 19 and 24 years) and CCH in 3. A significant correlation was observed between basal preoperative serum calcitonin/tumor size (r = 0.53, P < 0.05) and age/tumor size (r = 0.56, P < 0.03), but not between basal preoperative serum calcitonin and age. Stimulated preoperative calcitonin levels were confounding and not useful for differentiating CCH from MTC. None of patients in whom cervical dissection was done (9/16) presented lymph node metastases, including the oldest ones. All patients but the older ones were biochemically cured after a mean of 5 years of follow-up. Prophylactic total thyroidectomy should be done early in life because there is an age-dependent progression from HCC to MTC. MTC often precedes biochemical detection of the disease."}, {"id": "pubmed23n0320_6041", "title": "Prophylactic thyroidectomy in MEN IIA: does the calcitonin level correlate with tumor spread?", "score": 0.018031189083820662, "content": "The fate of patients with multiple endocrine neoplasia of type II A (MEN II A) is determined by medullary thyroid carcinoma, which occurs in all cases. This has led to the therapeutic concept of prophylactic thyroidectomy in affected family members with the goal of removing the thyroid before the manifestation of carcinoma. We investigated a prophylactically thyroidectomized MEN II A population to determine whether the highly specific and sensitive tumor marker calcitonin correlates with tumor spread. Fifteen patients with MEN II A (aged 4-24 years) who had undergone prophylactic thyroidectomy since 1990 were included in the study. Baseline and pentagastrin-stimulated calcitonin levels were preoperatively determined in all cases. The indication for surgery was established on the basis of pathologic calcitonin levels in the first seven patients and on the basis of detected RET proto-oncogene mutation in the other eight patients. Bilateral central lymphadenectomy was performed in all patients in addition to thyroidectomy. Histology demonstrated C-cell hyperplasia in five patients (aged 4-13 years), unilateral medullary microcarcinoma in six (aged 9-17 years) and a bilateral medullary microcarcinoma in three cases (aged 17-24 years). One 9-year-old boy with bilateral microcarcinoma already had a lymph node metastasis. The mean baseline calcitonin level correlated with the histologic findings (r=0.71, P=0.003) but there was no correlation between pentagastrin-stimulated calcitonin levels and histology (r=0.21, P=0.47). In MEN II A patients undergoing prophylactic thyroidectomy, baseline but not stimulated calcitonin levels already correlate with the histologic tumor stage at the stage of clinically occult C-cell hyperplasia or medullary microcarcinoma. However, biochemical screening cannot reliably discriminate the transition from C-cell hyperplasia to invasive microcarcinoma. Individuals with MEN IIA should therefore undergo early prophylactic thyroidectomy once the diagnosis is confirmed by molecular genetic testing."}, {"id": "pubmed23n0629_23522", "title": "When is prophylactic thyroidectomy indicated for patients with the RET codon 609 mutation?", "score": 0.017549261083743842, "content": "Mutations in the RET proto-oncogene cause multiple endocrine neoplasia type 2A (MEN2A), and prophylactic thyroidectomy has generally been recommended before the age of 5 years. Patients with codon 609 mutations develop MTC at a later age and therefore the timing of prophylactic thyroidectomy is less clear. We report a three-generation family with C609Y RET mutation where members having prophylactic or therapeutic thyroidectomy call the current recommendations for age at thyroidectomy into question. Sixteen family members underwent thyroidectomy, for which clinical, laboratory, and pathological data were analyzed. A literature review of RET codon 609 mutations was carried out. Data were collected from 16 patients from this 38-member kindred. None of these affected members had pheochromocytoma, and one had a parathyroid adenoma. Nine of 16 patients had MTC (mean age 44.7 years, range 29-59 years) and elevated basal calcitonin levels; 6 of these 9 had lymph node metastases. Two patients had C-cell hyperplasia (CCH) at ages 18 and 37 years, and five patients had normal thyroid pathology (mean age 16 years, range 5-37 years). In the literature, a family with C609Y mutation was reported, with 15 members having MTC (mean age 42 years, range 21-59 years), and 6 with CCH (mean age 24 years, range 15-37 years). The youngest patient with C609Y RET mutation and MTC was 21 years old, and the youngest patient with CCH was 15 years old at diagnosis. These data suggest that patients with RET C609Y mutations can delay thyroidectomy until 10-15 years of age, with annual calcitonin screening prior to thyroidectomy."}, {"id": "pubmed23n0547_166", "title": "Very early prophylactic thyroid surgery for infants with a mutation of the RET proto-oncogene at codon 634: evaluation of the implementation of international guidelines for MEN type 2 in a single centre.", "score": 0.017419787091491105, "content": "Genetic diagnosis available since 1993 established germinal mutations of the RET proto-oncogene at codon 634 as the main cause of inherited medullary thyroid carcinoma (MTC). International guidelines established in 1999 recommend that children with such mutations undergo a total thyroidectomy before age 5, with unspecified cervical neck dissection. Since 1993, only 41 of 275 thyroidectomies reported in RET 634 children were performed before age 5 (15%). The aim of this study was to evaluate the implementation of these guidelines in a single centre. Genetic diagnosis was proposed to the parents of all eight children born after 1992 from two RET 634 families. Total thyroidectomy was proposed before age 5 if the child carried a mutation. Genetic diagnosis was performed in all the children (aged 1-3) and thyroidectomy in the five who carried a mutation (aged 2-5). Cervical lymph node dissection varied from lymphadenectomy of central and lateral compartments in the eldest child to pickings in the youngest. There was no permanent hypoparathyroidism or recurrent nerve paralysis. C-cell hyperplasia, medullary thyroid carcinoma and lymph node metastasis were present in 5/5, 3/5 and 0/5, respectively. Undetectable pentagastrin-stimulated CT levels were achieved and maintained postoperatively in all five children (average follow-up 3.6 years). MEN 2 guidelines on thyroidectomy can be efficiently and safely implemented by a multidisciplinary team operating in a single centre. The lack of guidelines on cervical neck dissection remains a problem; this could be solved by determining an age under which this procedure would be deemed unnecessary."}, {"id": "pubmed23n0758_807", "title": "[Clinical diagnosis and treatment of familial medullary thyroid carcinoma caused by a p.C618Y RET proto-oncogene mutation in a Chinese pedigree].", "score": 0.017322718561108653, "content": "To explore the clinical characteristics, therapeutic and clinical significance for RET proto-oncogene screening in a pedigree with familial medullary thyroid carcinoma. Comprehensive medical history was obtained from 19 members in a 4-generate southern Chinese family. Systemic clinical investigations including biochemical testing, imaging examinations and germline RET screening. RET screening showed heterozygous missense mutations of TGC to TAC at codon 618 on exon 10 in 8 cases (p.C618Y) completely consistent with the clinical manifestations. The clinical data of 7 patients with medullary thyroid carcinoma (MTC) and 2 carriers of asymptomatic RET mutation from were analyzed. Single/bilateral multi-centric MTC with lymph node metastases was confirmed in 6 cases by histopathology and 1 case by clinical examination. There were 1 male and 6 females with an initial mean diagnostic age was 49.6 years (range: 24 - 78). All had palpable neck masses. And the mean maximum diameter of MTC was 2.6 cm (range 1.4 - 4.4). Seven patients underwent thyroidectomy except a 78-year-old female patient: right total and left subtotal thyroidectomy (n = 1), right total thyroidectomy (previous left total thyroidectomy for benign mass) (n = 1) and total thyroidectomy (n = 4) were performed. All procedures were accompanied by at least bilateral level VI lymph node dissection and/or with modified single/bilateral neck dissection. After the first operation, 6 patients still presented a high value of calcitonin: 1 patient died of metastasis 64 months postoperatively; 3 patients underwent reoperation at 6 months after initial operation, the calcitonin levels dropped to normal in 2/3 cases and stayed higher in 1 case; another two cases presented bilateral thyroid gland residua, local lymph node enlargement on imaging examination and elevated levels of calcitonin at 214 and 60 months postoperation respectively. However, 1/2 asymptomatic with elevated pre-operative calcitonin subjects underwent total thyroidectomy and histopathological examination showed bilateral C cell hyperplasia. The other carriers, without surgery, with normal neck images, close monitoring and a 10-month follow-up, still presented undetectable calcitonin. Based on family survey, integrated RET screening and serum levels of calcitonin facilitate an early diagnosis and normalize surgery to improve the prognosis. For asymptomatic RET mutation carriers, their levels of calcitonin shall guide the individualized regimen of prophylactic thyroidectomy or strict monitoring and follow-ups."}, {"id": "pubmed23n0526_13432", "title": "Prophylactic thyroidectomy in multiple endocrine neoplasia type 2A.", "score": 0.01709901351845086, "content": "Medullary thyroid carcinoma is the most common cause of death in patients with multiple endocrine neoplasia (MEN) type 2A (MEN-2A) or type 2B or familial medullary thyroid carcinoma. We sought to determine whether total thyroidectomy in asymptomatic young members of kindreds with MEN-2A who had a mutated allele of the RET proto-oncogene could prevent or cure medullary thyroid carcinoma. A total of 50 patients 19 years of age or younger who were consecutively identified through a genetic screening program as carriers of a RET mutation characteristic of MEN-2A underwent total thyroidectomy. Five to 10 years after the surgery, each patient was evaluated by physical examination and by determination of plasma calcitonin levels after stimulation with provocative agents. In 44 of the 50 patients, basal and stimulated plasma calcitonin levels were at or below the limits of detection of the assay (proportion, 0.88; 95 percent confidence interval, 0.76 to 0.95). Two patients had basal and stimulated plasma calcitonin levels above the normal range. Stimulated plasma calcitonin levels had increased but remained within the normal range in four patients. The data suggest that there was a lower incidence of persistent or recurrent disease in children who underwent total thyroidectomy before eight years of age and in children in whom there were no metastases to cervical lymph nodes. In this study, young patients identified by direct DNA analysis as carriers of a RET mutation characteristic of MEN-2A had no evidence of persistent or recurrent medullary thyroid carcinoma five or more years after total thyroidectomy. A longer period of evaluation will be necessary to confirm that they are cured."}, {"id": "pubmed23n0946_10674", "title": "Child with RET proto-oncogene codon 634 mutation.", "score": 0.017043847241867045, "content": "İnce D, Demirağ B, Ataseven E, Oymak Y, Tuhan H, Karakuş OZ, Hazan F, Abacı A, Özer E, Mutafoglu K, Olgun N. Child with RET proto-oncogene codon 634 mutation. Turk J Pediatr 2017; 59: 590-593. Herein we reported a 7-year-old child with RET proto-oncogene c634 mutation. Her mother had been diagnosed with medullary thyroid carcinoma (MTC), and treated six years ago. Heterozygous mutation of the RET proto-oncogene at c634 had been detected in her mother. Genetic analysis showed the presence of the same mutation in our patient. Thyroid functions were normal. Serum calcitonin level was found mildly elevated. Parathormone (PTH) and carcinoembrionic antigen (CEA) levels were normal. Prophylactic thyroidectomy and sampling of cervical lymph nodes were performed. Histopathologic examination revealed hyperplasia in thyroid C cells, and reactive lymphadenopathy. The risk of MTC has been reported 100% through the life of patients with RET proto-oncogene mutation. It has been reported that particularly patients with c634 mutation have more risk of occurence of metastatic and progressive/recurrent MTC. Prophylactic `thyroidectomy, cervical lymph node dissection` before 5-years-of-age should be considered for these patients."}, {"id": "pubmed23n0638_2415", "title": "Long-term outcome of \"prophylactic therapy\" for familial medullary thyroid cancer.", "score": 0.01655982905982906, "content": "About one quarter of all medullary thyroid cancers (MTC) are determined genetically due to a mutation in the RET proto-oncogene. The most common site of mutation is in codon 634. Therapeutic approaches toward patients at risk for the development of MTC identified by family screening programs range from total thyroidectomy to total thyroidectomy with lymphadenectomy of all 4 compartments. We report 17 patients (median age, 13 years; range, 4-36) carrying a mutation in codon 634 of the RET proto-oncogene who were operated on prophylactically at our department. All patients underwent thyroidectomy with bilateral cervicocentral lymphadenectomy. Current calcitonin level, overall survival, and disease-free survival were analyzed by contacting general practitioners and patients. Tumor classification was as follows: C-cell hyperplasia, 18% (n = 3); T1 (<1 cm), 71% (n = 12); and T1 (>1 cm), 12% (n = 2). Only 2 patients had lymph node metastases (12%). These patients developed recurrent disease (median observation time, 147 months; range, 90-181). In 1 patient, the calcitonin level normalized after unilateral cervicolateral lymphadenectomy. The other patient (9 years old at primary operation) still has a persistently increased serum calcitonin level after 140 months of follow-up despite several operations for MTC. Total thyroidectomy with bilateral cervicocentral lymphadenectomy is sufficient as routine \"prophylactic therapy\" for patients with mutations in codon 634 of the RET proto-oncogene. Cervicolateral lymphadenectomy is indicated if calcitonin remains elevated after primary surgery. Prophylactic thyroidectomy should be performed before the development of lymph node metastases."}, {"id": "pubmed23n0402_19785", "title": "Surgical strategy in a kindred with a rare RET protooncogene mutation of variable penetrance with regard to multiple endocrine neoplasia.", "score": 0.016498316498316498, "content": "Prophylactic thyroidectomy is recommended for carriers of RET protooncogene mutations owing to their nearly complete penetrance for medullary thyroid carcinoma (MTC). However, this guideline is challenged by mutations exhibiting variable penetrance of C-cell pathology. A 38-year-old woman presented with pathologic basal and pentagastrin-stimulated calcitonin levels. Genetic analysis revealed a heterozygous RET protooncogene germline mutation in codon 791 (exon 13) (TAT(Tyr)-->TTT(Phe)), followed by thyroidectomy and systematic central lymph node dissection. Histology showed C-cell hyperplasia (CCH) only. Three additional carriers were identified among family members. The 71-year-old father refused surgery despite pathologic calcitonin levels. The index patient's 37-year-old sister had normal basal and stimulated calcitonin levels, and her 6-year-old son had a 10-fold rise of calcitonin after pentagastrin stimulation. Both patients underwent the same operation as the index patient. The sister had 25 hyperplastic C-cells, but the her son had extensive CCH without MTC. The eldest uncle of the index patient had died of metastatic MTC at the age of 52 with unknown carrier status. Despite variable penetrance, each carrier of a RET protooncogene germline mutation should undergo thyroidectomy, even if basal and stimulated calcitonin levels are normal because at present no test can exclude or predict the age of development of MTC. Moreover, pathologic calcitonin levels cannot differentiate between CCH and MTC. Central lymph node dissection is recommended, as lymph node metastases occur early, significantly worsening the prognosis."}, {"id": "pubmed23n0770_16014", "title": "Early, Prophylactic Thyroidectomy in Hereditary Medullary Thyroid Carcinoma: A 26-year Monoinstitutional Experience.", "score": 0.01626651050816579, "content": "Prophylactic thyroidectomy has been encouraged for children with REarranged during Transfection (RET) germline mutations to prevent the onset, persistence, or recurrence of medullary thyroid carcinoma (MTC). The American Thyroid Association (ATA) recently published guidelines on the timing of prophylactic thyroidectomy. Our aim here was to seek information on the optimal timing of surgery for carriers of RET gene mutations with no clinical evidence of disease, bearing in mind the ATA recommendations. From 1986 to 2012, total thyroidectomy was performed at our institute on 31 carriers of RET gene mutations, 28 of them found on family screening in the post-RET era, and the other 3 under 20 years of age and classified as \"early cases\" in the pre-RET era. The following parameters were studied: age at surgery, MTC risk, basal calcitonin (bCT) and pentagastrin-stimulated calcitonin (sCT), surgery outcomes, and persistence of disease. By family, the most prevalent mutation was codon 634 (30%) RET mutation. The youngest MTC patient was 5 years old. Overall, MTC was found in 68% of cases; 52% of the sample had a normal bCT and 25% had an sCT unresponsive to pentagastrin. The only factor predicting the risk of MTC at final histology was an ATA-RET risk level C. On receiver oparating curves analysis, a cutoff at age over 24 years predicted (P=0.06) a yield of MTC in the resected specimen. Interestingly, none of the patients with MTC had nodal involvement (0/21 patients with MTC). Yet, none of the patients had permanent nerve palsy, and only 1 patient had permanent hypocalcemia. bCT was normal postoperatively and during the follow-up in all but 3 patients. It is noteworthy that the yield of cancer in removed thyroid was 100% for codon 634 (9/9 patients, 5 families) and for codons 891 and 768 (2/2 patients in each of the 2 families with those codon mutations), followed by 67% for codon 609 (4/6 patients, 1 family), and 60% for codon 618 (3/5 patients in 4 families) RET mutation. In cases of ATA-RET levels B and C, waiting for an increase in bCT and/or sCT may not guarantee that prophylactic surgery is performed before MTC develops (which would assure patients a life free of diseases and a less-invasive surgical procedure, without any need for central lymph-node dissection)."}, {"id": "pubmed23n0712_23274", "title": "The timing of total thyroidectomy in RET gene mutation carriers could be personalized and safely planned on the basis of serum calcitonin: 18 years experience at one single center.", "score": 0.01583820662768031, "content": "Medullary thyroid carcinoma (MTC) is a calcitonin (CT)-producing C-cell tumor. In hereditary cases, a germline RET mutation is found in 98% of families. Because MTC is cured only if intrathyroidal, prophylactic thyroidectomy is recommended in the gene carrier (GC). The aim was to determine whether thyroidectomy performed when stimulated CT becomes detectable is as safe as prophylactic thyroidectomy and to identify the serum CT cutoff able to distinguish intrathyroidal from extrathyroidal MTC. Eighty-four GC were prospectively enrolled; 53 of the 84 underwent total thyroidectomy, one refused surgery, and 30 with normal basal and stimulated CT were under surveillance. The follow-up ranged from 2 to 18 yr. GC operated on for elevated stimulated CT included 27 GC with a positive peak CT at the screening and four cases who became positive after 4 yr. All of them had intrathyroidal MTC and no node metastases; all were cured after a mean follow-up of 7.5 yr. Among those operated on for detectable basal CT, intrathyroidal tumors were found when CT was below 60 pg/ml, whereas either node metastases or larger tumors were observed when CT was above 60 pg/ml. No correlation among serum CT, age, and type of RET mutation was observed. Thirty GC were still biochemically negative at the annual control. The time of thyroidectomy in GC with negative CT could be personalized and safely planned when stimulated CT becomes positive, independent of the type of RET mutation and patient's age. In this series, a basal CT below 60 pg/ml was always associated to an intrathyroidal localization of MTC."}, {"id": "pubmed23n0789_20416", "title": "Prophylactic thyroidectomy for asymptomatic 3-year-old boy with positive multiple endocrine neoplasia type 2A mutation (codon 634).", "score": 0.01569676181617348, "content": "The multiple endocrine neoplasia type 2A (MEN 2A) syndrome, comprising medullary thyroid carcinoma (MTC), pheochromocytoma and primary hyperparathyroidism (PHPT) is most frequently caused by codon 634 activating mutations of the RET (rearranged during transfection) proto-oncogene on chromosome 10. For this codon-mutation carriers, earlier thyroidectomy (before the age of 5 years) would be advantageous in limiting the potential for the development of MTC as well as parathyroid adenomas. This is a case report of 3-year-old boy from the MEN 2A family (the boy's father and grandmother and paternal aunt) in which cysteine substitutes for phenylalanine at codon 634 in exon 11 of the RET proto-oncogene, who underwent thyroidectomy solely on the basis of genetic information. A boy had no thyromegaly, thyroidal irregularities or lymphadenopathy and no abnormality on the neck ultrasound examination. The pathology finding of thyroid gland was negative for MTC. Two years after total thyroidectomy, 5-year-old boy is healthy with permanent thyroxine replacement. His serum calcitonin level is < 2 pg/ml (normal < 13 pg/ml), has normal serum calcium and parathyroid hormone levels and negative urinary catecholamines. Long-term follow-up of this patient is required to determine whether very early thyroidectomy improves the long-term outcome of PHPT. Children with familial antecedents of MEN 2A should be genetically studied for the purpose of determining the risk of MTC and assessing the possibilities of making prophylactic thyroidectomy before the age of 5 years."}, {"id": "pubmed23n1034_22137", "title": "HEREDITARY ENDOCRINE TUMOURS: CURRENT STATE-OF-THE-ART AND RESEARCH OPPORTUNITIES: Early thyroidectomy in multiple endocrine neoplasia: a four decade experience.", "score": 0.015497737556561086, "content": "Forty years ago, physicians caring for the J-kindred, a 100+ member family with multiple endocrine neoplasia type 2A (MEN2A), hypothesized that early thyroidectomy based on measurement of the biomarker calcitonin could cure patients at risk for development of medullary thyroid carcinoma (MTC). We re-evaluated 22 family members with proven RET proto-oncogene mutations (C634G) who underwent thyroidectomy and central lymphadenectomy between 1972 and 1994 based on stimulated calcitonin abnormalities. Current disease status was evaluated by serum calcitonin measurement and neck ultrasound in 18 of the 22 prospectively screened patients. The median age of the cohort at thyroidectomy was 16.5 years (range 9-24). The median duration of follow-up at the time of examination was 40 years (range 21-43) with a median current age of 52 years (range 34-65). Fifteen of the 18 patients had no detectable serum calcitonin (<2 pg/mL). Three had detectable serum calcitonin measurements, inappropriately elevated following total thyroidectomy. None of the 16 patients imaged had an abnormal ultrasound. Survival analysis shows no MTC-related deaths in the prospectively screened patients, whereas there were many in prior generations. Early thyroidectomy based on biomarker testing has rendered 15 of 18 MEN2A patients (83%) calcitonin-free with a median follow-up period of 40 years. There have been no deaths in the prospectively screened and thyroidectomized group. We conclude that early thyroidectomy and central lymph node dissection is an effective prophylactic treatment for hereditary MTC."}, {"id": "pubmed23n0658_19846", "title": "[Prophylactic thyroidectomy in multiple endocrine neoplasia syndrome].", "score": 0.015407319952774497, "content": "Multiple endocrine neoplasia (MEN) 2a consists on medullary thyroid carcinoma, pheochromocytoma and hyperparathyroidism. The identification of the RET proto-oncogene in 1993 has changed the prognosis of the disease. We have retrospectively studied the patients diagnosed of MEN 2a in our centre for the last 7 years in order to establish the most adequate age to undergo surgery. We present ten patients diagnosed with MEN 2a, whose ages ranged from 1.5 to 11 years old. Mean age at time of operation: 6,4 years An ultrasound study, calcitonin determinations and cathecholamines and urinary metanephrine levels were obtained before surgery. The surgical treatment is based on total total thyroidectomy, in selected cases lymph node resection in the central zone lf the neck. The most frequent RET mutation is the one affecting codon 634 (exon 10), which was found in children. Both of them had an alteration in codon 611 (exon11). No complications appeared after surgery and hospital discharge took place in the 2nd-4th day after surgery. Pathological findings were medullary thyroid microcarcinoma (MTMC) in 3 out of 10 patients, calcitonin preoperative tests were high in one of them. No tumoral cells were found in the lymph nodes. During the follow up period, 9 out of 10 from the operated patients, maintained normal calcitonin, CEA, PTH, calcium, cathecholamines and urinary metanephrine levels. Since there are 3 cases of MTC in patients between 3 and 6 years old, and diagnostic test data are not conclusive, we thoroughly recommend prophyilactic thyroidectomy at early ages, from 3 to 4 years old."}, {"id": "pubmed23n0376_17717", "title": "Importance of early screening and prophylactic thyroidectomy in asymptomatic nonindex RET germline carriers.", "score": 0.01508295625942685, "content": "Genetic testing for RET germline mutations affords rapid identification of germline carriers, offering the prospect of cure before C-cell hyperplasia (CCH) has progressed to medullary thyroid carcinoma (MTC). Although nonindex RET mutation carriers have a better prognosis than do the index patients, it remains to be ascertained whether age represents a risk factor for MTC when screening patients. The current institutional study (October 1994 through June 1999) was set up to compare asymptomatic nonindex patients who were grouped by age: < 20 years and > or = 20 years. Inclusion criteria were confirmed RET mutations in the germline, with no MTC being more advanced than pT1pN1M0. Adult patients (> or = 20 years) had MTC significantly more often (84% vs. 43%), significantly larger tumors (5 mm vs. 3 mm), and significantly higher basal calcitonin levels preoperatively (78.0 vs. 9.7 pg/ml) than their pediatric/adolescent counterparts (< 20 years). There was a close correlation between pT1 MTC and an elevated basal serum calcitonin level (r = 0.67; Spearman's rho). All three patients with lymph node metastases from MTC had elevated basal calcitonin levels. The two groups did not differ in terms of multifocality of MTC (pT1b), lymph node involvement (pN1) or bilateral lymph node metastasis (pN1b), or preoperative stimulated and postoperative basal and stimulated serum calcitonin. Prophylactic thyroidectomy should not be postponed beyond the age of 20, and it should be performed before basal serum calcitonin has turned positive. Pathologic conversion of stimulated serum calcitonin obviously marks the time in carriers of RET germline mutations when surgery should be scheduled at the latest to be prophylactic."}, {"id": "pubmed23n0498_12002", "title": "RET germline mutation in codon 791 in a family representing 3 generations from age 5 to age 70 years: should thyroidectomy be performed?", "score": 0.01501052398360474, "content": "To describe a kindred with a rare RET germline mutation in codon 791 and discuss potential management strategies. We present clinical and biochemical data as well as results of mutation analysis in our study subjects and provide an overview of related published reports. Multiple endocrine neoplasia type 2 (MEN 2) is a familial cancer syndrome characterized by the development of medullary thyroid carcinoma (MTC), pheochromocytoma, and parathyroid hyperplasia or adenoma. Germline mutations in RET are responsible for this autosomal dominant syndrome. Familial MTC is a variant of MEN 2A and can be caused by RET mutations in codon 791. Deaths from gene carriers with mutations in these codons have not yet been reported. In general, gene carriers with these RET mutations have late-onset MTC. Because only a few kindreds with this specific mutation have been identified and no long-term follow-up data are available, management of these patients can be a challenge. We illustrate the difficulties with decisions about not only when to perform thyroidectomy in these patients but also whether thyroidectomy should even be considered in such gene carriers with a benign course. Our reported kindred included four carriers with a codon 791 RET germline mutation, one of whom had the rare concomitant occurrence of acromegaly and MEN 2A. The 70-year-old mother had acromegaly and hyperparathyroidism but normal serum calcitonin levels and normal findings on thyroid ultrasound examination. She refused pentagastrin testing and any surgical intervention. The 37-year-old daughter had hypothyroidism, a small thyroid gland, and negative results of pentagastrin stimulation testing of calcitonin. The 18-year-old grandson also had a negative pentagastrin test result and normal thyroid ultrasound findings. The 5-year-old granddaughter had normal results of thyroid ultrasonography. In all patients, we recommended thyroidectomy. Prospective studies are needed to clarify which patients with codon 791 RET germline mutation should undergo thyroidectomy."}, {"id": "pubmed23n0681_11533", "title": "Preventive thyroidectomy in patients with hereditary medullary thyroid carcinoma found heterozygote for mutant RET proto-oncogene.", "score": 0.014810275266840305, "content": "The currently available genetic tests for identification of the RET proto-oncogene mutation offer the possibility of prospective successful therapy before the hyperplasia of C-cells evolve to Medullary Thyroid Carcinoma. We present our experience regarding the preventive thyroidectomy of family members with history of Medullary Thyroid Carcinoma, who were found to be heterozygote for mutant RET proto-oncogene. We have retrospectively reviewed 19 members of 6 families with history of Medullary Thyroid Carcinoma, who were heterozygote for mutant RET protooncogene and underwent prophylactic thyroidectomy. All patients included in this series were below twenty years of age. The Medullary Thyroid Carcinoma was asymptomatic and the mutation of RET protooncogene has been also documented pre-operatively in all of them. All patients had undergone total thyroidectomy, while 1 with pheochromocytoma had undergone also left epinephridectomy. Fourteen patients (73.68%) had undergone lymph-nodes resection (in 10 of them the resection was central, in 3 unilateral and in 1 bilateral). Although none of our patients suffered from hyperparathyroidism, 7 parathyroid glands have been also resected from 3 patients, while auto-transfusion has been performed in one. In all patients, preoperative measurement of the calcitonin blood levels before and after stimulation with pentagastrin has been performed."}, {"id": "pubmed23n0603_12533", "title": "[Is immediate prophylactic thyroidectomy indispensable in familiar medullary thyroid carcinoma?].", "score": 0.014801938474504846, "content": "To emphasize the importance of genetic studies in family members and early prophylactic thyroidectomy in oncogene mutation carriers in the management of familiar medullary thyroid carcinoma. A retrospective review of families with familiar medullary thyroid carcinoma treated at our center in the last 7 years was performed. We identified a total of 7 families who has isolated prevalences with thyroid malignancies. Forty members of the 7 families were screened for gene RET mutations. Prophylactic total thyroidectomy was performed in every RET mutation gene carriers. In all families the index case were patients with medullary thyroid carcinoma presenting at a mean age of 37.25 years (range 23-42). The RET oncogen mutation was in codon 634 in exon 11 (multiple endocrine neoplasia type 2A) in all these patients. Fourteen gene carriers were identified with a mean age of 20 years (range 7-37), eleven of whom had medullary thyroid carcinoma at the time of surgery. Five of the gene carriers were children, with a mean age of 11 years (range 7-16), four of whom had microcarcinoma and one had metastatic carcinoma at the time of surgery. After surgery no hypoparathyroidism or recurrent nerve paralysis were documented. No pediatric patient has presented with phaeochromocytoma or hypoparathyroidism to date Four of the five children have normal calcitonin levels (< 2 pg/ml) and they are free of disease. The one who presented metastatic carcinoma has recurrent disease and is awaiting surgical treatment. Genetic studies of family members related to patients with familiar medullary thyroid carcinoma and RET mutations is indispensable. The RET mutation in codon 634 exon 11 was found to be the most frequent association. Prophylactic thyroidectomy is the only curative treatment and has minimal complications when performed by expert surgeons. Early thyroidectomy is recommended since distant metastatic spread can occur at early age."}, {"id": "pubmed23n0567_16318", "title": "Codon Y791F mutations in a large kindred: is prophylactic thyroidectomy always indicated?", "score": 0.014663511010495987, "content": "RET proto-oncogene mutations are associated with medullary thyroid carcinomas usually requiring preventive thyroidectomy in gene carriers. We present a large kindred with the Y791F mutation in the RET proto-oncogene that did not have medullary thyroid carcinomas. Eight members of a Danish family with the Y791F mutation participated. All gene carriers underwent pentagastrin testing, and measurements of serum calcitonin. In the index person, exons 10, 11, and 13-16 of the RET proto-oncogene were screened. In the rest of the individuals only exon 13 was analysed. Mutation analysis was done by direct bidirectional sequencing of PCR products on an ABI 3100 Genetic Analyzer (Applied Biosystems, Foster City, CA, USA). The index person was screened genetically due to goitre at a young age. A total of 27 members of the family underwent genetic testing. Twelve (44.4%, 95% CI: 25.5%-64.7%) had the mutation (age range 13-84 years). None of these had abnormal pentagastrin tests (0%, 95% CI: 0%-26.5%). We found no significant differences in basal serum calcitonin concentrations between gene carriers (mean +/- SD: 0.16 +/- 0.54 pmol/l, n = 11) and non-gene carriers (0.55 +/- 0.86 pmol/l, n = 6, 2 P = 0.29). None showed signs of primary hyperparathyroidism or phaeochromocytoma. The Y791F RET proto-oncogene mutation may have a low penetrance. In selected cases, prophylactic thyroidectomy may be replaced by watchful waiting with repeated pentagastrin testing, provided careful evaluation of risks and benefits is performed."}, {"id": "pubmed23n0416_17894", "title": "Acceptable age for prophylactic surgery in children with multiple endocrine neoplasia type 2a.", "score": 0.014388120839733742, "content": "Germline mutated RET proto-oncogene, causing multiple endocrine neoplasia (MEN)-2a syndrome is the indication for prophylactic total thyroidectomy. Literature regarding the risk and the extent of early surgical intervention is scarce and the optimum age for surgery is still controversial. To optimize management in these young children we evaluate our experience and results. From 1990 to 2001 preventive total thyroidectomy was performed in 13 MEN-2a gene carriers (4 boys and 9 girls; median age 7 (4-14) years). Preoperative assessment, surgical procedure, pathological examination, postoperative complications and treatment results were studied. Surgery existed of a total thyroidectomy alone (n=3) in children with normal basal calcitonin and in combination with tracheo-esophageal exploration (n=6) or central compartment dissection (n=4) in case of abnormal calcitonin levels. Eight children presented with medullary thyroid cancer (MTC), three (median: 5 (4-12) years) with microscopic MTC and five (median 6 (4-14) years) with frank invasive MTC. Four of these five patients were younger than 6 years. Except for long-lasting hypoparathyroidism in one patient there were no complications. At a median follow-up of 6.5 years all patients are disease free. MTC in RET mutated MEN-2a gene carriers in childhood are found at the age of 4 years. Therefore, DNA testing should be done preferably before that age. Preventive surgery can be performed safely at that age and may be limited to total thyroidectomy when baseline calcitonin levels are normal."}, {"id": "Surgery_Schwartz_10912", "title": "Surgery_Schwartz", "score": 0.014308498519024836, "content": "MTC for RET point mutations has largely replaced using provocative testing with pentagastrin or calcium-stimulated calcitonin levels to make the diagnosis. Calcitonin and CEA are used to identify patients with persistent or recurrent MTC. Calcitonin is a more sensitive tumor marker, but CEA is a better predictor of prognosis.Treatment. The ATA published revised guidelines for the man-agement of medullary cancers in 2015.59 A neck ultrasound is recommended to evaluate the central and lateral neck compart-ments and the superior mediastinum. Serum calcitonin, CEA, calcium levels should also be measured, and RET proto-onco-gene mutation testing should be performed. Pheochromocy-tomas need to be excluded. If patients are found to have a pheochromocytoma, this must be operated on first. Primary hyperparathyroidism, if present, is treated at the time of thyroidectomy. These tumors are generally (>50%) bilateral. Total thyroidectomy is the treatment of choice for patients with MTC because of"}, {"id": "Surgery_Schwartz_10911", "title": "Surgery_Schwartz", "score": 0.0137126066195976, "content": "and amyloid. Marked heterogeneity is present; cells may be polygonal or spindle shaped. The presence of amyloid is a diagnostic finding, but immunohistochemistry for calcitonin is more commonly used as a diagnostic tumor marker. These tumors also stain positively for CEA and calcito-nin gene–related peptide.Diagnosis. The diagnosis of MTC is established by history, physical examination, raised serum calcitonin, or CEA levels, and FNAB cytology of the thyroid mass. Attention to family history is important because about 25% of patients with MTC have familial disease. Because it is not possible to distinguish sporadic from familial disease at initial presentation, all new patients with MTC should be screened for RET point mutations, pheochromocytoma, and HPT. Screening of patients with famil-ial MTC for RET point mutations has largely replaced using provocative testing with pentagastrin or calcium-stimulated calcitonin levels to make the diagnosis. Calcitonin and CEA are used to identify"}, {"id": "pubmed23n0693_6694", "title": "[Phenotype of the C634Y mutation in the RET proto-oncogene in MEN2A: report of a family].", "score": 0.013428262436914203, "content": "Genetic testing of RET proto-oncogen allows an early diagnosis of Multiple Endocrine Neoplasia syndrome type 2 and establish a correlation between genotype and clinical manifestations. The purpose of this study was to demonstrate the benefits of an early diagnosis with genetic testing followed by prompt surgery on the cure of MTC versus a later diagnosis with serum calcitonin. Retrospective descriptive study of 8 members of a MEN 2A family by C634Y mutation. We performed serum calcitonin screening until 1999 and subsequently RET genetic testing was obtained. Carriers underwent total thyroidectomy and periodic determination of calcitonin, urinary metanephrines, calcium, phosphorus and neck and abdominal imaging techniques. Five patients were diagnosed by calcitonin familial screening and all of them have high calcitonin by now. Three patients were diagnosed by genetic testing (an adult and two children) and they are free of disease. Calcitonin was closely monitored in children and they underwent surgery when it started to raise, at 6 and 10 years old respectively, finding nodular C-cell hyperplasia in both. Of 8 carriers 3 developed pheochromocytomas, bilateral and asynchronous, one-half had normal urinary metanephrines and two of them were simultaneous with MTC. No patient had biochemical data suggesting hyperparathyroidism although in one patient multiple parathyroid adenomas were found at thyroidectomy. RET genetic analysis has achieved an early diagnosis and treatment with no development of MTC in our patients, adjusting the time and type of surgery and allowing a genotype-phenotype correlation. It demonstrates how a genetic alteration is associated with a pathology that we can prevent and manage improving the prognosis of our patients."}, {"id": "pubmed23n0319_2315", "title": "Prophylactic thyroidectomy in 75 children and adolescents with hereditary medullary thyroid carcinoma: German and Austrian experience.", "score": 0.013313871196036801, "content": "When mutations of the RETproto-oncogene were found in 1993 to account for hereditary medullary thyroid carcinoma (MTC), surgeons obtained the opportunity to operate on patients prophylactically (i. e., at a clinically asymptomatic stage). Whether this approach is justified, and, if so, when and to which extent surgery should be performed remained to be clarified. A questionnaire was sent to all surgical departments in Germany and Austria. All of the patients who fulfilled the following criteria were enrolled: (1) preoperatively proved RET mutation; (2) age </= 20 years, (3) clinically asymptomatic thyroid C cell disease; and (4) TNM classification pT0-1/pNX/pN0-1/M0. Seventy-five patients were identified, and fifteen mutations were detected in six codons. Two adolescents had unilateral pheochromocytomas as part of the multiple endocrine neoplasia II (MEN-II) syndrome. No hyperparathyroidism was noted. All patients underwent total thyroidectomy, and 57 patients went on to have lymph node dissection. Parathyroid glands were removed in 34 patients and autografted in 11. Histopathology revealed MTC in 46 patients (61%, youngest 4 years); C cell hyperplasia (CCH) only was detected in the other 29 patients. Three patients had lymph node metastases (LNMs) the youngest being age 14 years. Calcitonin levels were not useful for differentiating between CCH and MTC, but in all patients with LNMs at least the stimulated calcitonin levels were assayed. After surgery, five patients (6.7%) sustained permanent hypoparathyroidism, and one patient (1.3%) had a permanent unilateral recurrent nerve palsy. All but three patients (96%) were biochemically cured. In conclusion, prophylactic total thyroidectomy can be performed safely in experienced centers. We recommend prophylactic total thyroidectomy at age 6. Cervicocentral lymph node dissection should be included when calcitonin levels are elevated or if patients are older than 10 years. Bilateral lymph node dissection should be performed if LNMs are suspected or when patients with elevated calcitonin are older than 15 years."}, {"id": "Surgery_Schwartz_10919", "title": "Surgery_Schwartz", "score": 0.012984496124031008, "content": "children who are RET-positive and calcitonin-negative with a normal ultrasound examination. When the cal-citonin is increased or the ultrasound suggests a thyroid cancer, a prophylactic central neck dissection is indicated.Postoperative Follow-Up and Prognosis. Patients are fol-lowed by annual measurements of calcitonin and CEA levels, in addition to history and physical examination. Other modalities used to localize recurrent disease include ultrasound, CT, MRI, and more recently, FDG-PET/CT scans. Prognosis is related to disease stage. The 10-year survival rate is approximately 80% but decreases to 45% in patients with lymph node involvement. Survival also is significantly influenced by disease type. It is best in patients with non-MEN familial MTC, followed by those with MEN2A, and then those with sporadic disease. Progno-sis is the worst (survival of 35% at 10 years) in patients with MEN2B. Performing prophylactic surgery in RET oncogene mutation carriers not only improves"}, {"id": "pubmed23n0569_20094", "title": "Medullary thyroid carcinoma: surgical treatment advances.", "score": 0.012946876044102907, "content": "Since medullary thyroid cancer (MTC) was first recognized as a distinct tumor in 1959, it became clear that MTC is more difficult to cure than papillary thyroid cancer and has higher rates of recurrence and mortality. MTC represents 5-8% of thyroid cancers. It derives from parafollicular cells of the ultimobranchial body derived from the neural crest. MTC secretes calcitonin and other hormonal peptides and is considered part of the amine precursor uptake and decarboxilation system. MTC may occur either as a hereditary or nonhereditary entity. Hereditary MTC can occur either alone as the familial MTC or as the thyroid manifestation of multiple endocrine neoplasia (MEN) type 2 syndromes (MEN 2A MEN 2B). Activating point mutations of the RET proto-oncogene have demonstrated to be causative of the familial form of medullary thyroid cancer, both isolated familial MTC and associated with MEN 2A and 2B. In the last 10 years, major improvements and new technologies have been proposed and applied in thyroid surgery; among these are molecular diagnosis with genetic screening and mini-invasive video-assisted thyroidectomy. The history of thyroid surgery starts with Billroth, Kocher and Halsted, who developed the technique for thyroidectomy between 1873 and 1910. Prophylactic surgery for patients carrying a positive RET proto-oncogene has proven to be highly effective in curing those likely to experience the development of MTC. Video-assisted procedures with central compartment dissection have proved feasible for patients carrying a positive RET proto-oncogene. This paper reviews relevant medical literature published in the English language on surgery of MTC in well-controlled trials. We discuss the particular ethical and legal issues that thyroid prophylactic surgery raises. Searches were last updated in February 2007."}, {"id": "pubmed23n0339_18957", "title": "Predictive DNA testing for multiple endocrine neoplasia 2: a therapeutic challenge of prophylactic thyroidectomy in very young children.", "score": 0.012594268476621418, "content": "Patients with multiple endocrine neoplasia (MEN) type 2 are at risk for early medullary thyroid carcinoma (MTC). Recently, the cloning of the ret oncogene has made it possible to identify patients at risk for MEN 2 syndrome with a high degree of reliability before presenting any symptoms. Children of families with MEN 2 were screened genetically if one of the parents was a known gene carrier of the RET proto-oncogene. If they were carriers, thyroidectomy was performed. The authors report five children with MEN 2 who underwent prophylactic thyroidectomy irrespective of the results of calcitonin screening tests after genetic screening had shown that they were carrier of the RET proto-oncogene. Apart from a temporary hypocalcemia in one, the operations were uneventful. Pathology results showed MTC in three children of one family with MEN 2A at age 2, 3, and 6 years. In two families with MEN 2B the thyroidectomy specimen showed bilateral MTC in a 1-year-old and a 3-year-old child. These findings show that MTC occurs at very young age in children with MEN 2. The authors advocate performing prophylactic thyroidectomy in the first year of life in children with MEN 2B and at age 2 years in children with MEN 2A to obtain an optimal cure rate."}, {"id": "pubmed23n0639_22226", "title": "Prophylactic thyroidectomy in ethnic Chinese patients with multiple endocrine neoplasia type 2A syndrome after the introduction of genetic testing.", "score": 0.012375591754575616, "content": "To evaluate the impact of genetic testing in the management of familial multiple endocrine neoplasia 2A patients. Retrospective study. University teaching hospital, Hong Kong. Twenty-two patients from eight multiple endocrine neoplasia 2A families underwent prophylactic total thyroidectomy based on a positive RET mutation genetic testing. All mutations were located at codon 634 of exon 11. Nineteen patients had preoperative basal serum calcitonin measured, and the 12 with normal levels had pentagastrin stimulation tests. Preoperative thyroid ultrasound examination was performed for 17 patients. There were 13 females and 9 males with a median age of 25.1 (range, 6.1-71.9) years. Histopathology revealed medullary thyroid carcinoma in 17 (77%), C-cell hyperplasia in four (18%), and normal pathology in one (5%) of the patients. Five patients with either C-cell hyperplasia or normal pathology were among the youngest (age range, 6-9 years). The youngest patient with medullary thyroid carcinoma was nearly 9 years old. The median size of medullary thyroid carcinomas was 8.3 (range, 0.1-18) mm, but there were no lymph node metastases. Of 15 patients with normal basal calcitonin levels, 10 had medullary thyroid carcinoma, though two tested negative with the pentagastrin-stimulated calcitonin assay. Five of six patients with normal preoperative ultrasonographic examinations had medullary thyroid carcinoma. Three (14%) of the patients were prescribed long-term calcium and vitamin D supplementation. After a median follow-up of 49 (range, 13-128) months, no patient had recurrence of medullary thyroid carcinoma. Genetic testing has replaced conventional biochemical and radiological modalities to identifying multiple endocrine neoplasia 2A carriers, in order to offer them prophylactic thyroidectomy. Chinese multiple endocrine neoplasia 2A patients with codon 634 mutation seem to have less aggressive forms of medullary thyroid carcinoma, for whom prophylactic thyroidectomy can be considered at the age of 8 years."}, {"id": "pubmed23n0401_16358", "title": "Prophylactic thyroidectomy in MEN 2A syndrome: experience in a single center.", "score": 0.012281618635209796, "content": "Genetic study of the RET proto-oncogene has modified the management, treatment, and prognosis of medullary thyroid carcinoma (MTC), multiple endocrine neoplasia 2A (MEN 2A), for patients with less advanced tumor stages. Classically, the diagnosis was based on an increase in basal and poststimulus peak calcitonin (bCT and pCT). Prophylactic thyroidectomy, based on results of genetic testing, may reduce recurrences in MTC. Of 82 MTC (MEN 2A) patients genetically diagnosed and surgically treated at our center, 22 received a prophylactic thyroidectomy (RET +, bCT and pCT with normal values and asymptomatic). We analyzed age, gender, phenotype, RET mutation, cervical ultrasound, laboratory tests (bCT, pCT, and CEA), surgery, histologic data, TNM, and followup. The 22 patients belonged to 8 families with MTC (MEN 2A). Mean age was 15.2 years (range 5 to 36 years). The RET mutation in 21 patients was Cys-->Tyr and in the remaining patient both in codon 634 in exon 11. The median values of bCT and pCT were 38 pg/mL (range < 15 to 75 pg/mL) and 148.5 pg/mL (range < 15 to 250 pg/mL), respectively. Total thyroidectomy was performed in 8 patients (age < or = 10 years) and associated central neck dissection in 14 patients (age> 10 years). Histologic study showed 7 C-cell hyperplasias and 15 MTCs (8 bilateral); the median size was 0.2 cm (range < 0.1 to 0.7cm); 1 patient had metastatic adenopathies. According to TNM, 7 were stage 0, 14 were stage I, and 1 was stage III. Postsurgery bCT and pCT values were normal in all patients, with a curative rate of 100%. MTC patients compared with C-cell hyperplasia patients were older on average, had higher mean bCT, mean pCT, and mean CEA. Prophylactic thyroidectomy based on genetic testing allows identification and treatment of patients at an early stage of the disease and decreases recurrence rates. pCT values above the upper limit of normal may be markers for the presence of MTC and should be considered in selecting operative procedures for these patients."}, {"id": "Pediatrics_Nelson_3697", "title": "Pediatrics_Nelson", "score": 0.012153639005031535, "content": "Treatment includes total thyroidectomy, selective regional node dissection, and radioablation with 131I for residual or recurrent disease. The prognosis is usually good if the disease is diagnosed early. Medullary carcinoma of the thyroid may be asymptomatic except for a mass. Diagnosis is based on the presenceof elevated calcitonin levels, either in the basal state or after pentagastrin stimulation (difficult to obtain) and histologicexamination. This tumor most often occurs with multipleendocrine neoplasia 2a or 2b (MEN), possibly in a familial pattern. In some families, the presence of mutations ofthe RET proto-oncogene is predictive of the development of medullary carcinoma of the thyroid. The location of the mutation can help determine when removal of the thyroid iswarranted. Genetic screening of other members of the family is indicated after a proband is recognized. Prophylacticthyroidectomy is indicated for the family members with thesame allele."}, {"id": "pubmed23n0815_5898", "title": "Characterization of V804M-mutated RET proto-oncogene associated with familial medullary thyroid cancer, report of the largest Turkish family.", "score": 0.01204225352112676, "content": "Analysis of the RET proto-oncogen is very important for diagnosis and prognosis of medullary thyroid cancer (MTC). Genotype-phenotype correlation is also well known. Here we report features of the largest known family in Turkey with the V804M-mutated RET proto-oncogene. Thirty members from three generations were evaluated. A RET proto-oncogen mutation, calcitonin (Ct) measurement and thyroid ultrasound were performed on all individuals. Seventeen members had V804M mutation. Fourteen of these patients underwent total thyroidectomy and additional central lymph node dissection for five subjects. The mean age of patients with MTC was 46.5 (30-61) years. The mean calcitonin level of RET positive members was 13.27 pg/mL (1-49.8 pg/mL). Three had a basal Ct level above normal limits. Seven of the 14 patients were diagnosed with MTC, and two were diagnosed with papillary thyroid cancer without MTC. One patient had central neck metastasis. Hyperparathyroidism or pheochromocytoma was not detected in any case. Patients who were RET negative, had normal Ct levels and no suspected nodule on ultrasound examination. Our study revealed a relatively good prognosis in patients with V804M mutation. Despite the surgery was performed in older age no advance disease was observed."}]}}}} {"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[3, 194]], "word_ranges": [[1, 29]], "text": "In the case of suspected compartment syndrome, the urgent invasive measure to perform would be to measure the intracompartmental pressure and, depending on the result, proceed with treatment."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "3. In the case of suspected compartment syndrome, the urgent invasive measure to perform would be to measure the intracompartmental pressure and, depending on the result, proceed with treatment.", "full_answer_no_ref": "3. In the case of suspected compartment syndrome, the urgent invasive measure to perform would be to measure the intracompartmental pressure and, depending on the result, proceed with treatment.", "full_question": "A 35-year-old man suffers a high-voltage electrical burn (3000 volts) from direct contact with wire with his left hand. On admission he presents flexion contracture of the hand, pallor of the fingers and absence of radial and ulnar pulse on palpation What is the emergency invasive measure to be performed?", "id": 326, "lang": "en", "options": {"1": "Axillary block with catheter.", "2": "Decompressive scarotomy.", "3": "Intracompartmental pressure monitoring.", "4": "Scarectomy.", "5": NaN}, "question_id_specific": 173, "type": "CRITICAL, PALLIATIVE AND EMERGENCY CARE", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0720_6992", "title": "14,000 volt electrical injury to bilateral upper extremities: a case report.", "score": 0.017494270435446907, "content": "Electrical burns are among the most devastating of burn injuries. High voltage electrical injuries result in extensive deep tissue damage and are associated with multiple complications, long term morbidity, and a high mortality rate. We describe the case of a 47 year-old electric company linesman who suffered a high voltage electrical injury (HVEI) of 14,000 volts to bilateral hands and wrists managed by the Division of Plastic and Reconstructive Surgery at the McGill University Health Center in Montreal, Quebec, Canada. His management included multiple operative procedures, including escharotomies, fasciotomies, serial debridements, and bilateral pedicle groin flaps, and amputation of his left hand."}, {"id": "pubmed23n0914_16121", "title": "[Effects of flow-through descending branch of lateral circumflex femoral artery flap on repairing high-voltage electrical burn wounds of wrist of patients].", "score": 0.016023626995098655, "content": "300 mg of protein in a 24-hour urine sample). Severe pre-eclampsia involves a blood pressure greater than 160/110, with additional medical signs and symptoms. HELLP syndrome is a type of pre-eclampsia. It is a combination of three medical conditions: hemolytic anemia, elevated liver enzymes and low platelet count. Eclampsia This is when tonic-clonic seizures appear in a pregnant woman with high blood pressure and proteinuria. Pre-eclampsia and eclampsia are sometimes treated as components of a common syndrome. Treatment"}, {"id": "pubmed23n0937_14908", "title": "Home blood-pressure monitoring in a hypertensive pregnant population.", "score": 0.01532878344708049, "content": "The majority of patients with chronic or gestational hypertension do not develop pre-eclampsia. Home blood-pressure monitoring (HBPM) has the potential to offer a more accurate and acceptable means of monitoring hypertensive patients during pregnancy compared with traditional pathways of frequent outpatient monitoring. The aim of this study was to determine whether HBPM reduces visits to antenatal services and is safe in pregnancy. This was a case-control study of 166 hypertensive pregnant women, which took place at St George's Hospital, University of London. Inclusion criteria were: chronic hypertension, gestational hypertension or high risk of developing pre-eclampsia, no significant proteinuria (≤ 1+ proteinuria on dipstick testing) and normal biochemical and hematological markers. Exclusion criteria were maternal age < 16 years, systolic blood pressure > 155 mmHg or diastolic blood pressure > 100 mmHg, significant proteinuria (≥ 2+ proteinuria on dipstick testing or protein/creatinine ratio > 30 mg/mmol), evidence of small-for-gestational age (estimated fetal weight < 10 160/110) Thrombocytopenia Increased or rapidly elevating levels of creatinine in the blood Increased liver enzymes Pulmonary edema New or persistent headaches that do not respond to pain medication Blurred or altered vision If a woman with preeclampsia has any of these signs of significant organ damage, then her condition is classified as preeclampsia with severe features. This diagnosis can be made even if the patient does not have proteinuria. Women with preeclampsia with severe features are encouraged to deliver the child after 34 weeks of gestation to minimize the risks of the severe complications. Preeclampsia can also present with seizures in the pregnant mother. In this case, the patient would be diagnosed with eclampsia."}, {"id": "wiki20220301en017_69073", "title": "Pre-eclampsia", "score": 0.014042257195026518, "content": "Immune factors may also play a role. Diagnosis Testing for pre-eclampsia is recommended throughout pregnancy via measuring a woman's blood pressure. Diagnostic criteria Pre-eclampsia is diagnosed when a pregnant woman develops: Blood pressure ≥140 mmHg systolic or ≥90 mmHg diastolic on two separate readings taken at least four to six hours apart after 20 weeks' gestation in an individual with previously normal blood pressure. In a woman with essential hypertension beginning before 20 weeks' gestational age, the diagnostic criteria are an increase in systolic blood pressure (SBP) of ≥30 mmHg or an increase in diastolic blood pressure (DBP) of ≥15 mmHg. Proteinuria ≥ or more of protein in a 24-hour urine sample or a SPOT urinary protein to creatinine ratio ≥0.3 or a urine dipstick reading of 1+ or greater (dipstick reading should only be used if other quantitative methods are not available)."}, {"id": "wiki20220301en030_22840", "title": "Gestational hypertension", "score": 0.01382547471730318, "content": "Gestational hypertension or pregnancy-induced hypertension (PIH) is the development of new hypertension in a pregnant woman after 20 weeks' gestation without the presence of protein in the urine or other signs of pre-eclampsia. Gestational hypertension is defined as having a blood pressure greater than 140/90 on two occasions at least 6 hours apart. Signs and symptoms No single diagnostic test currently exists to predict the likelihood of developing gestational hypertension. High blood pressure is the major sign in diagnosing gestational hypertension. Some women with gestational hypertension may present asymptomatic, but a number of symptoms are associated with the condition. Symptoms Edema Sudden weight gain Blurred vision or sensitivity to light Nausea and vomiting Persistent headaches Increased blood pressure"}, {"id": "pubmed23n0034_5365", "title": "Blood pressure, edema and proteinuria in pregnancy. 9. Proposal for classification.", "score": 0.013267993466013268, "content": "The frequency distributions of blood pressures in large populations fail to show two groups, one normotensive and the other hypertensive. In the spectrum of pressures, some people merely have higher levels than others and division of abnormal from normal is artificial and arbitrary, although it is useful for prognosis. The blood pressure of 140/90 as the conventional dividing line does not seem to be appropriate in pregnant women. From the standpoint of fetal prognosis, a level of 125/75 before the thirty-second week and 125/85 thereafter seems more reasonable. Moreover, those levels are close to the 120/80 that Robinson and Brucer specified as the upper limit of normal for all adults and are close to the 130/70 and 120/80 that the eminent British authority, F.J. Browne, used successively in the diagnosis of hypertensive disorders in pregnancy. If the standard of 125/75 were adopted, however, a quarter of all pregnant women would be hypertensive in the second trimester and half in the last month, which are disturbingly high proportions. For the diagnosis of preeclampsia, a rise in blood pressure probably is more significant than an arbitrary level. The usual blood pressure in midpregnancy merely defines the patient's place in the spectrum. Figure 9-1 indicates that in white nulliparas the diastolic pressure rises an average of 10 mm. Hg in the middle of the third trimester. If the mean and median are close together, greater increases would occur in half of the women. The classification of the American Committee on Maternal Welfare and of the Committee on Terminology of the American College of Obstetricians and Gynecologists specify increases of 30 mm. Hg or more in the systolic or 15 mm. Hg or more in the diastolic pressures as criteria of preeclamptic hypertension. pperhaps the rise in diastolic pressure should be set at some greater value. Our analysis of data made thus far cannot decide that issue. The next phase of the study will include analyses in individual women of the times, magnitudes, persistence or transience, and the like of changes in blood pressure, edema, and proteinuria. Such data will afford much more information than can be derived from the preliminary studies reported here. Although edema of the hands and face may be more common in preeclamptic than in normal women, such edema is so common in normal pregnancy as to suggest that it usually is normal. In our data, edema seems to bear no relation to hypertension or proteinuria. The triad of signs -- hypertension, proteinuria, and edema -- is generally accepted as characteristic, though far from specific for preeclampsia. Our data support Hytten's conclusion that edema should by dropped from the triad. There is some indication, however, that some edema is abnormal and that it is associated with an adverse effect when it coincides with proteinuria late in pregnancy."}, {"id": "wiki20220301en464_20964", "title": "Hypertensive disease of pregnancy", "score": 0.013170924935630818, "content": "Because chronic hypertension can progress to more severe forms of disease, it is important to accurately diagnose the condition early, ideally prior to pregnancy, and initiate management to control parental blood pressure. This is often difficult, as many reproductive individuals may not regularly visit the doctor and, when pregnant, may initially present for prenatal care in the second trimester. Pre-eclampsia and eclampsia Preeclampsia is a medical condition which usually develops after 20 weeks of gestation and traditionally involves both newly increased blood pressure (blood pressure > 140/90 mmHg) and proteinuria. Preeclampsia is a leading cause of fetal complications, which include low birth weight, preterm birth, and stillbirth. Women with preeclampsia are encouraged to deliver the child after 37 weeks of gestation to minimize the risks of the severe complications."}, {"id": "wiki20220301en464_20960", "title": "Hypertensive disease of pregnancy", "score": 0.013105413105413105, "content": "Risks Some women have a greater risk of developing hypertension during pregnancy. These are: Women with chronic hypertension (high blood pressure before becoming pregnant). Women who developed high blood pressure or preeclampsia during a previous pregnancy, especially if these conditions occurred early in the pregnancy. Women who are obese prior to pregnancy. Pregnant women under the age of 20 or over the age of 40. Women who are pregnant with more than one baby. Women with diabetes, kidney disease, rheumatoid arthritis, lupus, or scleroderma. Diagnosis There is no single test to predict or diagnose preeclampsia. Key signs are increased blood pressure and protein in the urine (proteinuria). Other symptoms that seem to occur with preeclampsia include persistent headaches, blurred vision or sensitivity to light, and abdominal pain."}, {"id": "pubmed23n0403_5191", "title": "Prognostic value of office and ambulatory blood pressure measurements in pregnancy.", "score": 0.013035315787609365, "content": "With the objective to assess the prognostic value of office values as compared with ambulatory monitoring in pregnancy, we analyzed 2430 blood pressure series systematically sampled from 403 untreated pregnant women for 48 consecutive hours every 4 weeks from the first visit to the hospital until delivery. Women were divided into 5 groups: \"detected\" gestational hypertension, women with office blood pressures >140/90 mm Hg after 20 weeks of gestation and hyperbaric index (area of blood pressure excess above the upper limit of a time-specified tolerance interval) consistently above the threshold for diagnosing hypertension in pregnancy; \"undetected\" gestational hypertension, office values <140/90 mm Hg but hyperbaric index above the threshold for diagnosis; normotension, both office values and hyperbaric index below the thresholds for diagnosis; white coat hypertension, women with recorded diagnosis of gestational hypertension but hyperbaric index consistently below the threshold for diagnosis; and preeclampsia, defined as gestational hypertension and proteinuria. Results indicate small and nonsignificant differences in 24-hour mean of ambulatory pressures between \"detected\" and \"undetected\" gestational hypertension at all stages of pregnancy, in contrast with highly significant differences between these two groups and normotensive pregnancies. Average office blood pressure values were similar for preeclampsia, \"detected,\" and \"undetected\" gestational hypertension. The hyperbaric index was, however, significantly higher for women with preeclampsia after 20 weeks of gestation as compared with all other groups and higher for women with either \"detected\" or \"undetected\" gestational hypertension as compared with normotensive pregnant women. The incidence of preterm delivery and intrauterine growth retardation were similar for \"detected\" and \"undetected\" gestational hypertension but significantly lower for normotensive women. In pregnancy, the hyperbaric index derived from ambulatory monitoring is markedly superior to office measurements for diagnosis of what should be truly considered gestational hypertension, as well as for prediction of the outcome of pregnancy."}, {"id": "wiki20220301en464_20961", "title": "Hypertensive disease of pregnancy", "score": 0.012755993569581323, "content": "All of these sensations can be caused by other disorders; they can also occur in healthy pregnancies. Regular visits are scheduled to track blood pressure and level of protein in urine, to order and analyze blood tests that detect signs of preeclampsia, and to monitor fetal development more closely. Classification A classification of hypertensive disorders of pregnancy uses 4 categories as recommended by the U.S. National High Blood Pressure Education Program Working Group on High Blood Pressure in Pregnancy: Chronic hypertension; Preeclampsia-eclampsia; Preeclampsia superimposed on chronic hypertension; Gestational hypertension (transient hypertension of pregnancy or chronic hypertension identified in the latter half of pregnancy)."}, {"id": "pubmed23n0818_25015", "title": "Preeclampsia: an update.", "score": 0.012572936926924145, "content": "Preeclampsia was formerly defined as a multisystemic disorder characterized by new onset of hypertension (i.e. systolic blood pressure (SBP) ≥ 140 mmHg and/or diastolic blood pressure (DBP) ≥ 90 mmHg) and proteinuria (> 300 mg/24 h) arising after 20 weeks of gestation in a previously normotensive woman. Recently, the American College of Obstetricians and Gynecologists has stated that proteinuria is no longer required for the diagnosis of preeclampsia. This complication of pregnancy remains a leading cause of maternal morbidity and mortality. Clinical signs appear in the second half of pregnancy, but initial pathogenic mechanisms arise much earlier. The cytotrophoblast fails to remodel spiral arteries, leading to hypoperfusion and ischemia of the placenta. The fetal consequence is growth restriction. On the maternal side, the ischemic placenta releases factors that provoke a generalized maternal endothelial dysfunction. The endothelial dysfunction is in turn responsible for the symptoms and complications of preeclampsia. These include hypertension, proteinuria, renal impairment, thrombocytopenia, epigastric pain, liver dysfunction, hemolysis-elevated liver enzymes-low platelet count (HELLP) syndrome, visual disturbances, headache, and seizures. Despite a better understanding of preeclampsia pathophysiology and maternal hemodynamic alterations during preeclampsia, the only curative treatment remains placenta and fetus delivery. At the time of diagnosis, the initial objective is the assessment of disease severity. Severe hypertension (SBP ≥ 160 mm Hg and/or DBP ≥ 110 mmHg), thrombocytopenia < 100.000/μL, liver transaminases above twice the normal values, HELLP syndrome, renal failure, persistent epigastric or right upper quadrant pain, visual or neurologic symptoms, and acute pulmonary edema are all severity criteria. Medical treatment depends on the severity of preeclampsia, and relies on antihypertensive medications and magnesium sulfate. Medical treatment does not alter the course of the disease, but aims at preventing the occurrence of intracranial hemorrhages and seizures. The decision of terminating pregnancy and perform delivery is based on gestational age, maternal and fetal conditions, and severity of preeclampsia. Delivery is proposed for patients with preeclampsia without severe features after 37 weeks of gestation and in case of severe preeclampsia after 34 weeks of gestation. Between 24 and 34 weeks of gestation, conservative management of severe preeclampsia may be considered in selected patients. Antenatal corticosteroids should be administered to less than 34 gestation week preeclamptic women to promote fetal lung maturity. Termination of pregnancy should be discussed if severe preeclampsia occurs before 24 weeks of gestation. Maternal end organ dysfunction and non-reassuring tests of fetal well-being are indications for delivery at any gestational age. Neuraxial analgesia and anesthesia are, in the absence of thrombocytopenia, strongly considered as first line anesthetic techniques in preeclamptic patients. Airway edema and tracheal intubation-induced elevation in blood pressure are important issues of general anesthesia in those patients. The major adverse outcomes associated with preeclampsia are related to maternal central nervous system hemorrhage, hepatic rupture, and renal failure. Preeclampsia is also a risk factor for developing cardiovascular disease later in life, and therefore mandates long-term follow-up."}, {"id": "wiki20220301en464_20970", "title": "Hypertensive disease of pregnancy", "score": 0.01241327905510005, "content": "Gestational hypertension Gestational hypertension is a provisional diagnosis that involves newly increased blood pressure in a pregnant woman that usually develops after 20 weeks of gestation, but does not currently show any signs of proteinuria or other features associated with preeclampsia. Up to 50% of gestational hypertension patients go on to develop some form of preeclampsia. Gestational hypertension will normally resolve by 12 weeks postpartum. In this case, the diagnosis of gestational hypertension will be updated to be transient hypertension of pregnancy. If the increased blood pressure does not resolve by 12 weeks postpartum, then the diagnosis of gestational hypertension will be updated to be chronic hypertension."}, {"id": "wiki20220301en464_20963", "title": "Hypertensive disease of pregnancy", "score": 0.012245254963701565, "content": "The diagnostic criteria for chronic hypertension are typically considered to be at least two separate blood pressure readings taken at least four hours apart with systolic blood pressure ≥ 140mmHg, diastolic blood pressure ≥90 mmHg, or both, identified before pregnancy, before 20 weeks gestation, or persisting at least 12 weeks after giving birth. However, there is some controversy over the utility of adopting lower thresholds for diagnosis of chronic hypertension, which is more consistent with recent recommendations from the American College of Cardiology and the American Heart Association for the diagnosis of hypertension in adults. Chronic hypertension in pregnancy is now considered mild if blood pressures do not exceed 159 mmHg systolic and 109 mmHg diastolic and severe if pressures are ≥ 160 mmHg systolic or 110 mmHg diastolic, although controversy also exists as to the most appropriate cutoffs for this definition."}, {"id": "Obstentrics_Williams_4871", "title": "Obstentrics_Williams", "score": 0.012214137214137215, "content": "C, FIGURE 40-1 Schematic shows normal reference ranges for mean arterial blood pressure changes across pregnancy. Patient A (blue) has mean blood pressures near the 20th percentile throughout pregnancy. Patient B (red) has a similar pattern with mean pressures at the 25th percentile until approximately 36 weeks when her blood pressure begins to rise. By term, it is substantively higher and in the 75th percentile, but she is still considered \"normotensive.\" 25th percentile until 32 weeks. These begin to rise in patient B, who by term has substantively higher blood pressures. However, her pressures are still < 140/90 mm Hg, and thus she is considered to be \"normotensive.\" We use the term delta hypertension to describe this rather acute rise in blood pressure. Some of these women will go on to have obvious preeclampsia, and some even develop eclamptic seizures or HELLP (hemolysis, devatedliver enzyme levels, low 2latelet count) syndrome while still normotensive."}, {"id": "pubmed23n0350_4599", "title": "[Hypertensive disorders in pregnancy].", "score": 0.012025442258000398, "content": "Hypertension in pregnancy is defined by a systolic blood pressure > or = 140 mm Hg and a diastolic blood pressure of > or = 90 mm Hg or by a rise in blood pressure of systolic > or = 30 mm Hg and diastolic > or = 15 mm Hg. High blood pressures are found in 5-10% of all pregnancies. The outcome of pregnancy is influenced by the fact whether there occurs a proteinuria in addition to hypertension. While the prognosis of an isolated hypotension is good, the combination of hypertension and proteinuria leading to preeclampsia is the primary cause of maternal death in many countries and is responsible for 20-25% of perinatal mortality. A simple classification divides between chronic hypertension, preeclampsia, preeclampsia superimposed on chronic hypertension and transient hypertension. With chronic hypertension pregnancy outcome is determined by a preexisting nephropathy and the occurrence of a superimposed preeclampsia. Preeclampsia and superimposed preeclampsia are pregnancy induced multiorganic diseases, endangering both the mother and the fetus. Transient hypertension is a benign pathology, which occurs toward the end of pregnancy usually on the basis of a latent essential hypertension, which is laid open through pregnancy. While a severe chronic hypertension in pregnancy must be treated to prevent a hypertensive maternal encephalopathy, a less severe chronic hypertension should not be treated as the risk of a superimposed preeclampsia and the maternal and fetal outcome cannot be influenced by antihypertensive therapy. The incidence of preeclampsia is 3-5% in nulliparae and 0.5% in multiparae. Preeclampsia is a severe and dangerous pathology with an unknown etiology. Pregnancy termination is the only causal therapy. At present it is still recommended to terminate a severe preeclampsia after stabilizing the mother, irrespective of gestational age. In less severe preeclampsia occurring before 32 weeks of gestation, termination of pregnancy can be postponed under intensive monitoring and a prophylaxis with magnesium sulfate in order to accelerate the fetal lung maturation with glucocorticoids. A conservative management in the case of a HELLP-syndrome (Haemolyis, Elevated Liver enzymes, Low Platelets), which is a very severe form of preeclampsia, is not recommended because it hasn't been validated in prospective controlled studies. The most dangerous complication of preeclampsia is eclampsia, which is defined by general tonic-clonic convulsions before or after birth. The most effective prophylaxis of eclamptic attacks is the intravenous therapy with magnesium sulfate. A primary prohylaxis for preeclampsia doesn't exist. Treatment with low-dose aspirin in high-risk patients, i.e. after a severe preeclampsia, in cases of chronic hypertension, in cases of nephropathy and in cases with antiphospholipid-syndrome++ can be recommended. The prophylactic use of low-dose heparin, which has lead to a significant decreased incidence of preeclampsia in retrospective analysis, is now the object of a randomized, controlled trial in our hospital. All women who suffered from a preeclampsia should have a check-up after 3-6 months. Preexisting pathologies are found in up to 40% of patients, mostly in multiparae, i.e. chronic hypertension, nephropathy, endocrine pathologies, anomalies of blood coagulation and antiphospolipid-syndrome."}, {"id": "wiki20220301en464_20966", "title": "Hypertensive disease of pregnancy", "score": 0.012013469647786913, "content": "Preeclampsia can also present with seizures in the pregnant mother. In this case, the patient would be diagnosed with eclampsia. There is no proven way to prevent preeclampsia/eclampsia. Most women who develop signs of preeclampsia, however, are closely monitored to lessen or avoid related problems. The only way to \"cure\" preeclampsia/eclampsia is to deliver or abort the baby. Eclampsia Eclampsia is one particularly concerning form of preeclampsia in which a pregnant woman who previously presented with signs of newly increased blood pressure begins to experience new generalized seizures or coma. Up to 70% of patients with eclampsia experience complications associated with pregnancy. These complications can include HELLP syndrome, acute kidney injury, and disseminated intravascular coagulation among others."}, {"id": "wiki20220301en067_43551", "title": "Hypertensive emergency", "score": 0.011860992193402165, "content": "Diagnosis The term hypertensive emergency is primarily used as a specific term for a hypertensive crisis with a diastolic blood pressure greater than or equal to 120 mmHg or systolic blood pressure greater than or equal to 180 mmHg. Hypertensive emergency differs from hypertensive urgency in that, in the former, there is evidence of acute organ damage. Both of these definitions had collectively been known as malignant hypertension, although this medical term is replaced. In the pregnant patient, the definition of hypertensive emergency (likely secondary to pre-eclampsia or eclampsia) is only a blood pressure exceeding 160 mmHg systolic blood pressure or 110 mmHg diastolic blood pressure."}, {"id": "article-23221_10", "title": "Hypertension In Pregnancy -- Evaluation", "score": 0.011836123738773699, "content": "The criteria for pre-eclampsia can also be met in the absence of proteinuria if one has new-onset hypertension with thrombocytopenia(platelets less than 100,000 x10(9)/L, renal insufficiency(double of baseline serum creatine or serum creatine >1.1mg/dL), pulmonary edema, impaired liver function (AST/ALT greater than twice upper limit of normal), or new-onset headache unresponsive to medications with no alternative cause.Pre-eclampsia can be superimposed with chronic hypertension, or as advancement along the spectrum of gestational hypertensive disease. Per ACOG guidelines, systolic blood pressure of greater than 160mmHg or diastolic blood pressure greater than 110mmHg on two separate readings 4 hours apart or any severe range pressure that requires antihypertensive medication which by treatment guidelines is severe pressures seperated by minutes(10-30 minutes). [15]"}, {"id": "pubmed23n1077_9235", "title": "Transient gestational hypertension and pre-eclampsia: Two case reports and literature review on the need for stringent monitoring.", "score": 0.011421911421911422, "content": "Transient gestation hypertension is a contributor to adverse pregnancy outcomes particularly when it progresses to pre-eclampsia (PE). This requires frequent monitoring. We illustrate the need for stringent monitoring of gestational hypertension, transient gestational hypertension (TGH) and PE without severe features and conducted a brief rapid review of the literature. Two cases are presented: Firstly, a 25-year-old primigravida at 30 gestational weeks who had an isolated TGH with high blood pressure (BP) of 141/87 mmHg, which was not investigated. Four weeks later, she presented with a BP of 202/128 mmHg, imminent eclampsia and intrauterine foetal death and had an uncomplicated induction of labour and delivered a 1400 g macerated male stillborn. Secondly, a 30-year-old primigravida at 30 gestational weeks who developed PE but her monitoring was compromised initially by inadequate healthcare capacity including unavailability of hospital bed-space for inpatient care and later by poor clinic attendance as a result of poor finances. At 32 gestational weeks, she presented with decreased foetal movement and was diagnosed as haemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome and intrauterine foetal death. She was stabilised, had induction of labour and delivered a 1400 g male macerated stillborn. Thereafter, the need for her to go home to complete the cultural burial rites of her baby and the pressure from her workplace resulted in an inadequate postpartum follow-up care. In conclusion, transient gestational hypertension is associated with adverse maternal and foetal outcomes, including foetal demise. Unavailability of hospital bed-space and poor personal finances interfere with stringent monitoring of hypertensive disorders and can be associated with adverse pregnancy outcomes. Stringent laboratory monitoring in these cases is defined by the authors as testing at least blood levels of serum Creatinine, Haemoglobin concentration, Alanine transaminase and Platelet count (abbreviated as 'CHAP') weekly."}, {"id": "wiki20220301en464_20975", "title": "Hypertensive disease of pregnancy", "score": 0.011343323108158807, "content": "There is limited evidence to suggest that calcium supplementation may reduce the risk of pre-eclampsia or stillbirth but it is unclear if it has other benefits. Prognosis The effects of high blood pressure during pregnancy vary depending on the disorder and other factors. Preeclampsia does not in general increase a woman's risk for developing chronic hypertension or other heart-related problems. Women with normal blood pressure who develop preeclampsia after the 20th week of their first pregnancy, short-term complications, including increased blood pressure, usually go away within about six weeks after delivery. Some women, however, may be more likely to develop high blood pressure or other heart disease later in life. More research is needed to determine the long-term health effects of hypertensive disorders in pregnancy and to develop better methods for identifying, diagnosing, and treating women at risk for these conditions."}, {"id": "wiki20220301en017_69074", "title": "Pre-eclampsia", "score": 0.011273448773448772, "content": "Suspicion for pre-eclampsia should be maintained in any pregnancy complicated by elevated blood pressure, even in the absence of proteinuria. Ten percent of individuals with other signs and symptoms of pre-eclampsia and 20% of individuals diagnosed with eclampsia show no evidence of proteinuria. In the absence of proteinuria, the presence of new-onset hypertension (elevated blood pressure) and the new onset of one or more of the following is suggestive of the diagnosis of pre-eclampsia: Evidence of kidney dysfunction (oliguria, elevated creatinine levels) Impaired liver function (noted by liver function tests) Thrombocytopenia (platelet count <100,000/microliter) Pulmonary edema Ankle edema (pitting type) Cerebral or visual disturbances"}, {"id": "wiki20220301en059_2554", "title": "Complications of pregnancy", "score": 0.011248926116838488, "content": "High blood pressure Potential severe hypertensive states of pregnancy are mainly: Preeclampsia – gestational hypertension, proteinuria (>300 mg), and edema. Severe preeclampsia involves a BP over 160/110 (with additional signs). It affects 5–8% of pregnancies. Eclampsia – seizures in a pre-eclamptic patient, affect around 1.4% of pregnancies. Gestational hypertension HELLP syndrome – Hemolytic anemia, elevated liver enzymes and a low platelet count. Incidence is reported as 0.5–0.9% of all pregnancies. Acute fatty liver of pregnancy is sometimes included in the preeclamptic spectrum. It occurs in approximately one in 7,000 to one in 15,000 pregnancies. Venous thromboembolism Deep vein thrombosis (DVT), a form of venous thromboembolism (VTE), has an incidence of 0.5 to 7 per 1,000 pregnancies, and is the second most common cause of maternal death in developed countries after bleeding."}, {"id": "wiki20220301en464_20959", "title": "Hypertensive disease of pregnancy", "score": 0.011214543861570475, "content": "Signs and symptoms Although many pregnant women with high blood pressure have healthy babies without serious problems, high blood pressure can be dangerous for both the mother and baby. Women with pre-existing, or chronic, high blood pressure are more likely to have certain complications during pregnancy than those with normal blood pressure. However, some women develop high blood pressure while they are pregnant (often called gestational hypertension). Chronic poorly-controlled high blood pressure before and during pregnancy puts a pregnant woman and her baby at risk for problems. It is associated with an increased risk for maternal complications such as preeclampsia, placental abruption (when the placenta separates from the wall of the uterus), and gestational diabetes. These women also face a higher risk for poor birth outcomes such as preterm delivery, having an infant small for his/her gestational age, and infant death."}, {"id": "pubmed23n0797_17240", "title": "[Atypical preeclampsia: case report].", "score": 0.011177119059284665, "content": "Preeclampsia that occurs at < 20 weeks of gestation is rare and has been usually reported with molar or hydropic degeneration of the placenta and antiphospholipid syndrome. To describe the clinical presentation of atypical preeclampsia of a patient of 37 years old at her first gestation who developed this entity at 18.5 weeks of gestation. She had history of pre-existing hypertension and infertility. This pregnancy was obtained through in vitro fertility. She reported a severe headache and was admitted to our hospital secondary to elevated blood pressure of 160/110 mm Hg. The laboratory evaluation revealed platelet count 51,000, alanine aminotransferase of 331 UI/L, aspartate aminotransferase of 285 UI/L, lactate dehydrogenase 421 UI/L and urinalysis with +2 proteinuria, soluble fms-like tyrosine kinase-1/placental growth factor ratio 895.5. The diagnosis of chronic hypertension and superimposed preeclampsia and incomplete HELLP syndrome was supported. After termination of pregnancy, the patient improved rapidly. She was discharged home on postoperative day 7 with a blood pressure of 120/70 mm Hg with normal laboratory. Clinicians should consider the diagnosis of preeclampsia and HELLP syndrome before 20 weeks of gestation in women presenting with clinical or laboratory abnormalities consistent with this disease."}, {"id": "Obstentrics_Williams_4875", "title": "Obstentrics_Williams", "score": 0.011066852422345622, "content": "TABLE 40-1. Classification and Diagnosis of Pregnancy-Associated Hypertension Gestational hypertension Preeclampsia: Hypertension plus • 2300 mg/24 h, or Urine protein: creatinine ratio 20.3, or Platelet count < 1 OO,OOO/.LL 1.1 mg/dL or doubling of baselineb Headache, visual disturbances, convulsions aRecommended only if sole available test. bNo prior renal disease. CAST (aspartate transaminase) or ALT (alanine transaminase). BP = blood pressure. Modified with permission from American College ofObstetricians and Gynecologists; Task Force on Hypertension in Pregnancy: Hypertension in pregnancy. Report ofthe American College ofObstetricians and Gynecologists'Task Force on"}, {"id": "wiki20220301en017_69079", "title": "Pre-eclampsia", "score": 0.011053779679503314, "content": "Differential diagnosis Pre-eclampsia can mimic and be confused with many other diseases, including chronic hypertension, chronic renal disease, primary seizure disorders, gallbladder and pancreatic disease, immune or thrombotic thrombocytopenic purpura, antiphospholipid syndrome and hemolytic-uremic syndrome. It must be considered a possibility in any pregnant woman beyond 20 weeks of gestation. It is particularly difficult to diagnose when pre-existing conditions such as hypertension are present. Women with acute fatty liver of pregnancy may also present with elevated blood pressure and protein in the urine, but differ by the extent of liver damage. Other disorders that can cause high blood pressure include thyrotoxicosis, pheochromocytoma, and drug misuse."}, {"id": "wiki20220301en464_20974", "title": "Hypertensive disease of pregnancy", "score": 0.011047979797979798, "content": "For all hypertensive disorders of pregnancy, a major component of care is management of the associated hypertension. This involves use of antihypertensive medication as well as restricting activity to lower blood pressure to reduce the risk of stroke. In women with preeclampsia or eclampsia, magnesium sulfate is often prescribed to prevent the occurrence of seizures in the gestational parent. Treatment should be continued from the time of diagnosis to several weeks postpartum given the increased risk of medical complications immediately following delivery of the fetus. Prevention Blood pressure control can be accomplished before pregnancy. Medications can control blood pressure. Certain medications may not be ideal for blood pressure control during pregnancy such as angiotensin-converting enzyme (ACE) inhibitors and angiotensin II (AII) receptor antagonists. Controlling weight gain during pregnancy can help reduce the risk of hypertension during pregnancy."}, {"id": "wiki20220301en017_69045", "title": "Pre-eclampsia", "score": 0.010996703247075825, "content": "Risk factors for pre-eclampsia include obesity, prior hypertension, older age, and diabetes mellitus. It is also more frequent in a woman's first pregnancy and if she is carrying twins. The underlying mechanism involves abnormal formation of blood vessels in the placenta amongst other factors. Most cases are diagnosed before delivery. Commonly, pre-eclampsia continues into the period after delivery, then known as postpartum pre-eclampsia. Rarely, pre-eclampsia may begin in the period after delivery. While historically both high blood pressure and protein in the urine were required to make the diagnosis, some definitions also include those with hypertension and any associated organ dysfunction. Blood pressure is defined as high when it is greater than 140 mmHg systolic or 90 mmHg diastolic at two separate times, more than four hours apart in a woman after twenty weeks of pregnancy. Pre-eclampsia is routinely screened for during prenatal care."}, {"id": "wiki20220301en107_1588", "title": "Hypertensive encephalopathy", "score": 0.010995410813706991, "content": "Hypertensive encephalopathy (HE) is general brain dysfunction due to significantly high blood pressure. Symptoms may include headache, vomiting, trouble with balance, and confusion. Onset is generally sudden. Complications can include seizures, posterior reversible encephalopathy syndrome, and bleeding in the back of the eye. In hypertensive encephalopathy, generally the blood pressure is greater than 200/130 mmHg. Occasionally it can occur at a BP as low as 160/100 mmHg. This can occur in kidney failure, those who rapidly stop blood pressure medication, pheochromocytoma, and people on a monoamine oxidase inhibitor (MAOI) who eat foods with tyramine. When it occurs in pregnancy it is known as eclampsia. The diagnosis requires ruling out other possible causes."}]}}}} {"correct_option": 4, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": true, "char_ranges": [[0, 239]], "word_ranges": [[0, 35]], "text": "Mentonian paresthesias (Roger's sign) are associated with bone metastases (it is not paraneoplastic but direct infiltration). In this case what you describe is an oral tumor growing locally and infiltrating the mandibular canal, I believe."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Mentonian paresthesias (Roger's sign) are associated with bone metastases (it is not paraneoplastic but direct infiltration). In this case what you describe is an oral tumor growing locally and infiltrating the mandibular canal, I believe.", "full_answer_no_ref": "Mentonian paresthesias (Roger's sign) are associated with bone metastases (it is not paraneoplastic but direct infiltration). In this case what you describe is an oral tumor growing locally and infiltrating the mandibular canal, I believe.", "full_question": "Faced with a patient presenting with an ulcerovegetating lesion on the mandibular gingiva at the level of the molar region of 5 months of evolution, the appearance of hypoesthesia in the mentonian region requires ruling out:", "id": 531, "lang": "en", "options": {"1": "Paraneoplastic neuropathy.", "2": "Diagnosis of sarcoidosis with \"on the scale\" facial paralysis.", "3": "Herpes virus infection in immunosuppressed patient.", "4": "Diagnosis of carcinoma with infiltration of the mandibular canal.", "5": NaN}, "question_id_specific": 58, "type": "ONCOLOGY", "year": 2021, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en001_99055", "title": "Ramsay Hunt syndrome type 2", "score": 0.013817065287653522, "content": "Ramsay Hunt Syndrome Type 2 is estimated to account for 12% of all facial nerve paralysis. It occurs in both immunocompetent and immunocompromised individuals with immunocompromised patients often having more severe disease presentation. RHS may occur in any age group with cases reported in patients ranging in age from 3 months to 82 years. The affected ganglion is responsible for the movements of facial muscles, the touch sensation of a part of ear and ear canal, the taste function of the frontal two-thirds of the tongue, and the moisturization of the eyes and the mouth. The syndrome specifically refers to the combination of this entity with weakness of the muscles activated by the facial nerve. In isolation, the latter is called Bell's palsy. However, as with shingles, the lack of lesions does not definitely exclude the existence of a herpes infection. Even before the eruption of vesicles, varicella zoster virus can be detected from the skin of the ear."}, {"id": "pubmed23n1165_5713", "title": "[The neuropathy of marginal mandibular branch of the facial nerve].", "score": 0.012650674304809643, "content": "To analyze the diagnosis, treatment and rehabiltation of patients with marginal mandibular branch of the facial nerve (MMB). We have collected 6 patients (mean age 40 [33.8; 44] years) with isolated lesion of MMB that innervates the depressor labii inferioris and chin muscle. The illness duration without any improvement was 35 [13; 44] days. Diagnosis and treatment were carried out according to the special algorithm developed and implemented at the N.V. Sklifosovsky Research Institute of Emergency Medicine. With needle myography of the muscle that lowers the lower lip, the change in the ratio of the maximum amplitudes of the interference pattern (MAIP) in all patients exceeded 15%, and in 2 cases it was more than 90%. Comparing with the healthy face side, a change of the MAIP ratio less than 90% was considered as the biomarker of favorable prognosis, with conservative treatment recommendations, e.g. the set of exercises with targeted effects on depressor labii inferioris. With regular exercises, patients noted positive dynamics of restoring the symmetry of the smile in 1-2 months of the disease, full recovery - in 4-5 months. In case of exercises rejection, there was no positive dynamics. A change in the MAIP ratio more than 90% or the absence of motor unit potentials was considered as the biomarker of an unfavorable outcome and an indication for surgical treatment. After surgical treatment, the improvement occurred within 4-5 months. In conservative treatment group, there were no positive changes even with regular exercises. The diagnosis of an isolated lesion of MMB is established clinically using a protocol of step-by-step assessment of facial muscle function, and tactics is determined by needle myography with depressor labii inferioris. Even with favorable myographic predictors, spontaneous recovery may not occur, exercises with a targeted effect on the depressor labii inferioris are required, and in the presence of unfavorable predictors, surgical treatment is reccomended."}, {"id": "InternalMed_Harrison_2695", "title": "InternalMed_Harrison", "score": 0.012413168227121716, "content": "involving the maxillary and mandibular divisions of the trigeminal nerve (e.g., maxillary sinusitis, neuroma, and leukemic infiltrate) is distinguished from ordinary toothache by the neuropathic quality of the pain. Occasionally, phantom pain follows tooth extraction. Pain and hyperalgesia behind the ear and on the side of the face in the day or so before facial weakness develops often constitute the earliest symptom of Bell’s palsy. Likewise, similar symptoms may precede visible lesions of herpes zoster infecting the seventh nerve (Ramsey-Hunt syndrome) or trigeminal nerve. Postherpetic neuralgia may follow either condition. Coronary ischemia may produce pain exclusively in the face and jaw; as in typical angina pectoris, this pain is usually reproducible with increased myocardial demand. Aching in several upper molar or"}, {"id": "wiki20220301en002_155434", "title": "Bell's palsy", "score": 0.01199742615771011, "content": "One disease that may be difficult to exclude in the differential diagnosis is involvement of the facial nerve in infections with the herpes zoster virus. The major differences in this condition are the presence of small blisters, or vesicles, on the external ear, significant pain in the jaw, ear face, and/or neck and hearing disturbances, but these findings may occasionally be lacking (zoster sine herpete). Reactivation of existing herpes zoster infection leading to facial paralysis in a Bell's palsy type pattern is known as Ramsay Hunt syndrome type 2. The prognosis for Bell's Palsy patients is generally much better than for Ramsay Hunt Syndrome Type 2 patients. Treatment Steroids have been shown to be effective at improving recovery in Bell's palsy while antivirals have not. In those who are unable to close their eyes, eye protective measures are required. Management during pregnancy is similar to management in the non-pregnant."}, {"id": "wiki20220301en002_155431", "title": "Bell's palsy", "score": 0.011927727850185855, "content": "One study found that 45% of patients are not referred to a specialist, which suggests that Bell's palsy is considered by physicians to be a straightforward diagnosis that is easy to manage. Other conditions that can cause similar symptoms include herpes zoster, Lyme disease, sarcoidosis, stroke, and brain tumors. Differential diagnosis Once the facial paralysis sets in, many people may mistake it as a symptom of a stroke; however, there are a few subtle differences. A stroke will usually cause a few additional symptoms, such as numbness or weakness in the arms and legs. And unlike Bell's palsy, a stroke will usually let patients control the upper part of their faces. A person with a stroke will usually have some wrinkling of their forehead."}, {"id": "Neurology_Adams_11049", "title": "Neurology_Adams", "score": 0.011882572227399813, "content": "Bell’s palsy may be bilateral, but only rarely is the involvement on the two sides simultaneous. The truly contemporaneous appearance of bilateral facial paralysis (facial diplegia) is most often a manifestation of the Guillain-Barré syndrome (GBS) and may also occur in Lyme disease and rarely, with HIV infection. There are numerous other causes of bilateral facial palsy, all of them infrequent. Keane (1994) listed the idiopathic (now presumably mainly viral) variety, GBS, and meningeal infiltration by tumor as the most common causes, but also found 2 cases of syphilis among 43 patients. The bilateral syndrome has been reported in approximately 7 of every 1,000 patients with sarcoidosis, although our impression is that it is more frequent. When acute in onset and associated with parotid gland swelling from sarcoidosis, it has been referred to as uveoparotid fever, or Heerfordt syndrome. In typical cases of sarcoidosis, the paralysis on each side tends to be temporally separated by"}, {"id": "InternalMed_Harrison_30739", "title": "InternalMed_Harrison", "score": 0.011542012927054477, "content": "Pterygopalatine Clinical Manifestations The onset of Bell’s palsy is ganglion fairly abrupt, with maximal weakness being attained by 48 h as a general rule. Pain behind the ear may precede the paralysis for a day or two. Taste sensation may be lost unilaterally, and hyperacusis may be present. In some cases, there is mild cerebrospinal fluid lymphocytosis. Magnetic resonance imaging (MRI) may reveal swelling and uniform enhancement of the geniculate ganglion and facial nerve and, in some cases, entrapment of the swollen nerve in the temporal bone. Approximately 80% of patients recover within a few weeks or months. Electromyography may be of some prognostic value; evidence of denervation after 10 days indicates there has been axonal degeneration, that there will be a long delay (3 months as 2648 Pathophysiology In acute Bell’s palsy, there is inflammation of the facial nerve with mononuclear cells, consistent with an infectious or immune cause. Herpes simplex virus (HSV) type 1 DNA was"}, {"id": "InternalMed_Harrison_30730", "title": "InternalMed_Harrison", "score": 0.011433477064757183, "content": "Isolated sensory loss over the chin (mental neuropathy) can be the only manifestation of systemic malignancy. Rarely, an idiopathic form of trigeminal neuropathy is observed. It is characterized by numbness Nasopharyngeal carcinoma Trauma Guillain-Barré syndrome Sjögren’s syndrome Collagen-vascular diseases Sarcoidosis Leprosy Drugs (stilbamidine, trichloroethylene) Idiopathic trigeminal neuropathy and paresthesias, sometimes bilaterally, with loss of sensation in the territory of the trigeminal nerve but without weakness of the jaw. Gradual recovery is the rule. Tonic spasm of the masticatory muscles, known as trismus, is symptomatic of tetanus (Chap. 177) or may occur in patients treated with phenothiazines. (Fig. 455-2) The seventh cranial nerve supplies all the muscles concerned with facial expression. The sensory component is small (the nervus intermedius); it conveys taste sensation from the anterior Trigeminal Neuralgia, Bell’s Palsy, and Other Cranial Nerve Disorders"}, {"id": "wiki20220301en293_24686", "title": "Heerfordt syndrome", "score": 0.011428218433407463, "content": "Diagnosis In patients that have already been diagnosed with sarcoidosis, Heerfordt syndrome can be inferred from the major symptoms of the syndrome, which include parotitis, fever, facial nerve palsy and anterior uveitis. In cases of parotitis, ultrasound-guided biopsy is used to exclude the possibility of lymphoma. There are many possible causes of facial nerve palsy, including Lyme disease, HIV, Melkersson–Rosenthal syndrome, schwannoma, and Bell's palsy. Heerfordt syndrome exhibits spontaneous remission. Treatment Treatments for sarcoidosis include corticosteroids and immunosuppressive drugs. Prevalence In the United States, sarcoidosis has a prevalence of approximately 10 cases per 100,000 whites and 36 cases per 100,000 blacks. Heerfordt syndrome is present in 4.1 to 5.6% of those with sarcoidosis."}, {"id": "pubmed23n0814_18529", "title": "Massive mandibular destruction and alveolar nerve infiltration without lower lip paresthesia in primary intraosseous carcinoma: report of two cases and critical appraisal of diagnostic criteria.", "score": 0.009900990099009901, "content": "To report two cases of solid type primary intraosseous carcinoma (PIOC) with a critical appraisal of one of the WHO diagnostic criteria. Both patients had radiographic and histopathologic findings showing massive mandibular destruction as well as the involvement of the inferior alveolar nerve, without lip or chin paresthesia. Patients were treated through hemimandibulectomy followed by reconstruction through fibula free flap and forearm flap. Lip and/ or chin paresthesia are rather frequent in metastatic and salivary gland tumors but not in primary tumors of the jaws. Reasons for such a discrepancy are mostly unknown. A few hypotheses are put forward here. It is the opinion of the authors that most of the diagnostic criteria for solid type PIOC are acceptable. However, the criterion \"absence of ulcer formation on the overlying mucosa\" mainly depends on the dimension of the tumor at diagnosis."}, {"id": "wiki20220301en379_13062", "title": "Human mouth", "score": 0.00980392156862745, "content": "all the lower teeth (inferior dental plexus). The oral mucosa of the gingiva (gums) on the facial (labial) aspect of the maxillary incisors, canines and premolar teeth is innervated by the superior labial branches of the infraorbital nerve. The posterior superior alveolar nerve supplies the gingiva on the facial aspect of the maxillary molar teeth. The gingiva on the palatal aspect of the maxillary teeth is innervated by the greater palatine nerve apart from in the incisor region, where it is the nasopalatine nerve (long sphenopalatine nerve). The gingiva of the lingual aspect of the mandibular teeth is innervated by the sublingual nerve, a branch of the lingual nerve. The gingiva on the facial aspect of the mandibular incisors and canines is innervated by the mental nerve, the continuation of the inferior alveolar nerve emerging from the mental foramen. The gingiva of the buccal (cheek) aspect of the mandibular molar teeth is innervated by the buccal nerve (long buccal nerve)."}, {"id": "article-49275_20", "title": "Facial Nerve Anatomy and Clinical Applications -- Clinical Significance -- Lower Motor Neuron Lesions after the Nerve Exits from the Brainstem", "score": 0.00980392156862745, "content": "After its exit from the brainstem, clinical features of lower motor neuron lesions depend on which branches of the facial nerve are affected. If the motor axons of the facial nerve are affected, the result is facial muscle weakness. Damage to the nerve to stapedius results in hyperacusis; damage to the greater petrosal branch manifests as loss of lacrimation, and damage to the chorda tympani results in loss of taste from the anterior two-thirds of the tongue as well as the loss of function of the sublingual and submandibular salivary glands. Causes of lower motor neuron lesions beyond the brainstem include infections such as the herpes simplex virus (Bell's Palsy), varicella-zoster virus (Ramsay-Hunt syndrome), Lyme disease, and HIV, inflammatory conditions such as sarcoidosis, demyelinating conditions such as Guillain Barre Syndrome, vascular conditions such as hemifacial spasm due to neurovascular compression, trauma such as temporal bone fractures, and neoplasms (both benign and malignant). [14] [1] [2]"}, {"id": "wiki20220301en475_27049", "title": "Retrocuspid papilla", "score": 0.009708737864077669, "content": "Retrocuspid papilla (RCP) is a small elevated nodules mostly behind the lower canine teeth in humans(Fig.1,2). It is sometimes associated with reactive arthritis. Epidemiology The RCP are first reported in 1947 and 1965. In a Swedish population it was first reported 1994. Among 1150 consecutively examined patients aged 20 –75 years, 10 showed RCP. Among 2000 biopsy cases from 1989 - 1992 in Department of Oral Pathology Lund University, 15 biopsies met the criteria of RCP. Clinical appearance The lesions are bilaterally situated in the attached gingiva or close to the border of the mucosa lingual to the two mandibular canines (Fig.1). However, they could in a few individuals also be seen simultaneously in the molar region and on the lingual side (Fig.2). They were 2–3 mm wide and high and covered with normal mucosa. Their tips were erected or could be folded down, mimicking the entrance of a periodontal abscess, but no duct was present."}, {"id": "pubmed23n0303_17386", "title": "[Diagnosis and treatment of facial palsy].", "score": 0.009708737864077669, "content": "The topographic diagnosis of facial nerve lesions is based on the symptoms that accompany paralysis, allowing lesions to be located in the protuberance, pontocerebellar angle, facial channel or trajectory distal to the stylomastoid foramen. Most cases of peripheral facial palsy have no apparent cause (idiopathic, or Bell's, peripheral facial palsy). However, facial palsy can sometimes be a manifestation of neuroborreliosis, multiple sclerosis, diabetes, HIV infection or neurinoma. Neurophysiologic studies complement physical examination to establish a prognosis; after the fifth day axonal degeneration related to incomplete recovery can be recognized. Magnetic resonance identifies nerve lesions but is useful only in atypical cases. Prednisone 1 mg/kg over 5 days, with gradual weaning, is the most widely accepted treatment for Bell's palsy. Acyclovir is indicated in Ramsay-Hunt syndrome. Early surgical decompression in cases with poor prognosis is not generally considered beneficial. Cases of permanent facial palsy have serious consequences, particularly because facial expression is altered."}, {"id": "pubmed23n0084_21071", "title": "[Neuropathy of the chin associated with neoplasms. Presentation of 5 cases and a review of the literature].", "score": 0.009615384615384616, "content": "Mental neuropathy is a paraneoplastic syndrome characterized by hypoesthesia or anesthesia in the area of mental nerve inervation. Its ethyology is not well known, and in some cases would be secondary to metastasis in the mandible, meningeal carcinomatosis or infiltration of the ganglion of Gasser. We report five new cases; three of them we have studied the presence of circulating antibodies against nervous tissue of ganglion of Gasser, and it was negative in all of them. Likewise we have reviewed the literature."}, {"id": "wiki20220301en127_1155", "title": "Odontogenic myxoma", "score": 0.009523809523809525, "content": "Patients afflicted with an odontogenic myxoma generally notice a painless, slowly enlarging expansion of the jaw with possible tooth loosening or displacement. As the tumor expands, it frequently infiltrates adjacent structures. Maxillary lesions frequently enter the sinuses while mandibular tumors often extend into the ramus. Diagnosis Radiographically, odontogenic myxomas appear most commonly as multilocular radiolucencies with ill-defined borders, though unilocular cyst-like tumors can occur, especially when associated with impacted teeth or when discovered in childhood. Ideally, the septa that cause the multilocular feature are thin and straight, producing a tennis racket or stepladder pattern. In reality, the majority of the septa visible in the tumor are curved and coarse, causing a \"soap bubble\" or \"honeycomb\" appearance, though locating one or two straight septa can aide in the diagnosis of this tumor."}, {"id": "First_Aid_Step1_597", "title": "First_Aid_Step1", "score": 0.009523809523809525, "content": "B . Ataxic GAAit. CN V motor lesion Jaw deviates toward side of lesion due to unopposed force from the opposite pterygoid muscle. CN X lesion Uvula deviates away from side of lesion. Weak side collapses and uvula points away. CN XI lesion Weakness turning head to contralateral side of lesion (SCM). Shoulder droop on side of lesion (trapezius). The left SCM contracts to help turn the head to the right. CN XII lesion LMN lesion. Tongue deviates toward side of lesion (“lick your wounds”) due to weakened tongue muscles on affected side. Facial nerve lesions Bell palsy is the most common cause of peripheral facial palsy A . Usually develops after HSV reactivation. Treatment: corticosteroids +/– acyclovir. Most patients gradually recover function, but aberrant regeneration can occur. Other causes of peripheral facial palsy include Lyme disease, herpes zoster (Ramsay Hunt syndrome), sarcoidosis, tumors (eg, parotid gland), diabetes mellitus."}, {"id": "pubmed23n0053_16130", "title": "[The incorporation of acyclovir into the treatment of peripheral paralysis. A study of 45 cases].", "score": 0.009433962264150943, "content": "The relation between use of acyclovir and facial nerve palsy prognosis was studied. In a randomised study, steroids or steroids + acyclovir (oral doses for Bell's palsy, and intravenous doses for Ramsay Hunt's syndrome) were given to 45 patients with facial palsy. There was a significant reduction of sequelae in patients treated with acyclovir in the group of Ramsay Hunt's syndrome (n = 15) (p less than 0.05). There were no significant differences in the group of Bell's palsy (n = 30) (p greater than 0.05), treated with acyclovir compared with steroids."}, {"id": "wiki20220301en061_34724", "title": "Dental follicle", "score": 0.009345794392523364, "content": "Clinical features The dentigerous cyst is often found in areas where unerupted teeth are found. These areas, in decreasing order of frequency, are mandibular third molars, maxillary third molars and maxillary canines. The cyst may grow to a large size, replace the tooth with which they are associated, or hardly cause resorption of adjacent tooth roots. Diagnosis Clinical and radiographic assessments are required to diagnose dentigerous cysts. A cyst is present when the follicular space exceeds 5mm from the crown. However, it is possible that keratocysts and ameloblastomas mimic the radiographical appearance of follicular cysts. Aspiration can be used to differentiate the lesions. Treatment - Marsupialization This procedure is partial removal of associated tooth. The advantage of this procedure is that it maintains the vitality of teeth and is less invasive. The disadvantage is that it required substantial after care and heals very slowly. - Enucleation"}, {"id": "InternalMed_Harrison_30744", "title": "InternalMed_Harrison", "score": 0.00932857991681521, "content": "Laboratory Evaluation The diagnosis of Bell’s palsy can usually be made clinically in patients with (1) a typical presentation, (2) no risk factors or preexisting symptoms for other causes of facial paralysis, (3) absence of cutaneous lesions of herpes zoster in the external ear canal, and (4) a normal neurologic examination with the exception of the facial nerve. Particular attention to the eighth cranial nerve, which courses near to the facial nerve in the pontomedullary junction and in the temporal bone, and to other cranial nerves is essential. In atypical or uncertain cases, an ESR, testing for diabetes mellitus, a Lyme titer, angiotensin-converting enzyme and chest imaging studies for possible sarcoidosis, a lumbar puncture for possible Guillain-Barré syndrome, or MRI scanning may be indicated. MRI often shows swelling and enhancement of the facial nerve in idiopathic Bell’s palsy (Fig. 455-3)."}, {"id": "wiki20220301en353_17095", "title": "Cysts of the jaws", "score": 0.009259259259259259, "content": "Odontogenic cysts have histologic origins in the cells of the dental structures. Some are inflammatory while others are developmental. Radicular cyst is the most common (up to two thirds of all cysts of the jaws). This inflammatory cyst originated from a reaction to dental pulp necrosis. Dentigerous cyst, the second most prevalent cyst, is associated with the crown of non-erupted tooth. Odontogenic keratocyst This lesion may be associated with the Nevoid basal cell carcinoma syndrome. Buccal bifurcation cyst which appears in the buccal bifurcation region of the mandibular first molars in the second half of the first decade of life. Eruption cyst; a small cyst in the gingiva as a tooth erupts, forming from the degenerating dental follicle Primordial cyst; previous thought to be a unique entity. Most primordial cysts have proven to be Keratocystic odontogenic tumors Orthokeratinized odontogenic cyst; a variant of the Keratocystic odontogenic tumor"}, {"id": "pubmed23n0323_13898", "title": "[Heerfordt's syndrome remitting without corticosteroid therapy].", "score": 0.009259259259259259, "content": "A 25-year-old woman presented with low-grade fever, uveitis, and bilateral swelling of the parotid glands. Her older sister had a history of sarcoidosis. A chest X-ray film showed bilateral hilar lymphadenopathy. The low-grade fever persisted after admission. Ga scintigraphy showed abnormal uptake in the parotid glands and hilar lymph nodes bilaterally. Sarcoidosis was diagnosed histologically after epithelioid cell granulomas without caseous necrosis were found in a specimen obtained by transbronchial biopsy. Heerfordt's syndrome was the final diagnosis. Six days after admission, left facial and left trigeminal nerve paralysis developed. The symptoms remitted without steroid therapy and, as of eight months after discharge there had been no evidence of recurrence."}, {"id": "wiki20220301en096_40537", "title": "Nevoid basal-cell carcinoma syndrome", "score": 0.009174311926605505, "content": "Signs and symptoms Some or all of the following may be seen in someone with Gorlin syndrome: Multiple basal-cell carcinomas of the skin Odontogenic keratocyst: Seen in 75% of patients and is the most common finding. There are usually multiple lesions found in the mandible. They occur at a young age (19 yrs average). Rib and vertebrae anomalies Intracranial calcification Skeletal abnormalities: bifid ribs, kyphoscoliosis, early calcification of falx cerebri (diagnosed with AP radiograph) Distinct faces: frontal and temporoparietal bossing, hypertelorism, and mandibular prognathism Bilateral ovarian fibromas 10% develop cardiac fibromas Cause Mutations in the human homologue of Drosophila patched (PTCH1), a tumor suppressor gene on chromosome 9, were identified as the underlying genetic event in this syndrome. Diagnosis Diagnosis of NBCCS is made by having 2 major criteria or 1 major and 2 minor criteria."}, {"id": "pubmed23n0944_7694", "title": "Numb chin syndrome: A reflection of malignancy or a harbinger of MRONJ? A multicenter experience.", "score": 0.009174311926605505, "content": "Numb chin syndrome (NCS) or mental neuropathy (MN) is a disorder characterized by sensory neuropathy on the distribution of the inferior alveolar nerve or mental nerve. The most frequent causes are of odontogenic origin (infections, wrong therapies). Other etiologies are related to primary tumor, metastasis, osteoradionecrosis and medication-related osteonecrosis of the jaw (MRONJ). The aim of this study is to highlight the clinical importance of NCS as one of the first symptoms of cancer or as consequence of drug therapy. The present study was conducted from 2010 to 2016 by recruiting patients who present NCS as one of the symptoms, having excluded those in which it depends on a clear odontogenic cause, on systemic degenerative diseases or metabolic disorders. Data collection included suspected diagnosis at the time of presentation of the symptom, final diagnosis, mandibular localization, treatment performed and diagnostic delay between the first medical examination and the definitive diagnosis. This study included 29 patients in which NCS had not a clear odontogenic cause. NCS was the first symptom of malignancy in 11 cases and the clinical sign of metastasis in 4 cases. In a single patient, it was the first symptom of an immune-mediated disease. In the remaining 13 patients, NCS represented the symptom of MRONJ. NCS can be the first symptom of malignancy, especially in patients with a previous history of cancer, but also a prodromal sign of MRONJ. It should be recognized in order to require deeper examinations for early diagnosis of the disease."}, {"id": "pubmed23n1096_12601", "title": "Metastasis in the mandible involving gingiva: An intriguing case with a perplexing pathology.", "score": 0.00909090909090909, "content": "Oral metastasis, although rare, tends to involve jawbones, particularly the posterior region of the mandible, and involvement of oral soft tissues, even when less likely, is most often seen on the gingiva and tongue. Clinically, the soft-tissue masses tend to mimic pyogenic granuloma, peripheral giant cell granuloma or an epulis and thus are difficult to diagnose and identify. The jaw bone is preferred by prostate carcinoma as a metastatic target. Prostate malignancy, which is more common in Western countries than in India, may be adenocarcinomas or carcinomas. Oftentimes, metastatic lesions develop in the alveolar region and are a cause for tooth mobility, yet, they tend to be detected only after extraction of the affected tooth. In such cases, the symptomatic presentation therefore, is vague and indicative of tooth mobility secondary to periodontal pathology unless, a detailed history and follow-up is done. We report a case of a male patient who presented to our department with a proliferative, painful, swelling postextraction of the left first molar region, and the lesion was seen at the extraction site as well as in the mandibular anterior tooth region. The swelling was associated with palpable lymph nodes. Orthopantomogram showed an irregular, radiolucent lesion extending from the lower left central incisor to the left first molar region in the mandibular alveolus. Incisional biopsy tissue came with provisional diagnosis of osteomyelitis or squamous cell carcinoma as the patient was a habitual bidi smoker for more than 20 years. Histologically, it was an undifferentiated tumor with tumor cells seen in deep connective tissue with a lack of lineage differentiation. An undifferentiated malignant tumor represents either a metastasis of unknown origin or a primary neoplasia without obvious cell line of differentiation. Immunohistochemistry (IHC) of undifferentiated tumors helps to categorize them into small round blue cell tumors or large cell tumors. The oral pathologist was perplexed as there was no mention of any other malignancy in the patient's history, which, however, was noted by the surgeons few days later. Hence, initially, a hematopoietic malignancy was suspected which was ruled out by IHC, and later, staining with cytokeratin 7 (CK7), CK-high molecular weight and P63 confirmed prostate metastases as all three were negative."}, {"id": "wiki20220301en486_23584", "title": "Actinomyces bovis", "score": 0.009009009009009009, "content": "Role in disease Clinical presentation and diagnosis Lumpy jaw (actinomycosis) in cattle can present itself in two main ways. One is as soft-tissue abscesses in the mouth and on the tongue. The other explains its common name, lumpy jaw, as A. bovis bacteria infect the mandibular bone, causing osteomyelitis and the formation of periosteal new bone, giving it the classical lumpy jaw appearance. It is caused by sharp objects, such as coarse feed materials or sticks, causing penetrative lesions in the mouth when ingested or by complication of periodontitis. Deeper lesions are required to cause infections in the bone, while shallower lesions are more likely to cause mouth abscesses. The bony lumps are usually not painful and can develop over weeks to months, causing facial distortion, dyspnea, and occasionally loose teeth. The abscesses within the bony lumps or soft tissue commonly consist of purulent granulation tissue, often gritty in texture, surrounded by a fibrous capsule."}, {"id": "pubmed23n0614_21813", "title": "Periradicular burkitt's lymphoma: a report of 2 cases from Nigeria and review of the literature.", "score": 0.008928571428571428, "content": "This article reports two cases of periradicular Burkitt's lymphoma from Nigeria, to emphasize the difficulties in differentiating the early lesion from other periradicular lesions with similar clinical and radiological findings Case 1, is a 4-year-old boy who presented with a one-month history of a painless, hard, posterior mandibular swelling (right), which was causing loosening and displacement of deciduous teeth (84 and 85). Histopathological examination of periradicular tissues from extracted tooth (85), confirmed the diagnosis of early periradicular B urkitt's lymphoma. Case 2, is a 6-year-old boy who presented with one-week history of a loose, extruded right mandibular first molar tooth (46) and an exfoliated left mandibular first molar tooth (36). After two weeks of follow-up, the patient developed bilateral mandibular swelling at the molar region, as well as peri-orbital and bilateral pedal oedema. Incisional biopsy of the oral lesion at the region of exfoliated left first mandibular molar (36) was useful for histopathological diagnosis of early Burkitt's lymphoma of the jaw. In the face of limited diagnostic tools such as clinico-radiological assessment, cytology or incisional biopsy for incipient periradicular lesions, a high index of suspicion of Burkitt's lymphoma of the jaw may be helpful in early diagnosis and treatment of a lesion, presenting in a child as periradicular radiolucency or mixed radiolucency and radiopacity, with associated loosening and displacement of teeth."}, {"id": "pubmed23n0728_1087", "title": "Orofacial granulomatosis: clinical study of 20 patients.", "score": 0.008928571428571428, "content": "The objective of this study was to analyze the clinical features of a series of patients with orofacial granulomatosis (OFG). Twenty patients diagnosed with OFG at Bellvitge Hospital (Barcelona, Spain) from 1985 to 2010 were included in the study. All of our patients (9 men and 11 women, median age 48.1 years) presented with labial swelling. Six patients presented with recurrent orofacial swelling, 12 with permanent swelling, and 2 with progressive swelling. Fissured tongue was observed in 9 cases, and 2 patients presented with recurrent episodes of peripheral facial paralysis. The median follow-up time was 65.1 months, ranging from 4 to 300 months. None of our patients developed sarcoidosis or Crohn disease. In the south of Europe, OFG does not appear to be as frequently associated with Crohn disease as in northern Europe. Although several treatments may achieve transient control of the orofacial swelling, there is no curative treatment for OFG and some patients may develop embarrassing lesions."}, {"id": "pubmed23n0555_12365", "title": "[Ramsay-Hunt syndrome associated to unilateral recurrential paralysis].", "score": 0.008922443376801648, "content": "Varicella herpes zoster (VZV) virus reactivaction can produce multiple neuropathies of the cranial and cervical region being the peripheral facial paralysys the most common one. We report one case of Ramsay-Hunt syndrome with eruption of vesicles on left auricular pinna and face besides facial palsy which associated to ipsilateral laryngeal or recurrential paralysis nonexisting previously. Our patient was treated by oral aciclovir (800 mg, 5 times daily) for 1 week. 3 months later she returned to Emergencies due to another cause and the ENT exploration showed a recovery in the mobility of the left cord but it persisted the affectation of VII pair, specially the inferior branch or cervicofacial. It is advised that the larynx should be examined in all cases of herpes zoster that involve the head and neck."}, {"id": "wiki20220301en054_63433", "title": "Human tooth development", "score": 0.008849557522123894, "content": "Primary dentition starts on the arrival of the mandibular central incisors, usually at eight months, and lasts until the first permanent molars appear in the mouth, usually at six years. The primary teeth typically erupt in the following order: (1) central incisor, (2) lateral incisor, (3) first molar, (4) canine, and (5) second molar. As a general rule, four teeth erupt for every six months of life, mandibular teeth erupt before maxillary teeth, and teeth erupt sooner in females than males. During primary dentition, the tooth buds of permanent teeth develop below the primary teeth, close to the palate or tongue."}, {"id": "pubmed23n0621_6752", "title": "[Isolated mandibular B-cell lymphoma revealed by inferior alveolar nerve anesthesia].", "score": 0.008849557522123894, "content": "Mandibular lymphomas are rare and most often revealed by painless swelling. The authors report the case of a mandibular lymphoma revealed by an isolated lesion of the inferior alveolar nerve evolving for eight months. A 41-year-old male patient was followed for left mandibular pain, with progressive hypoesthesia of the left inferior alveolar nerve. The radiological assessments remained normal during eight months. Then a vestibular tumor developed in front of tooth 34. The biopsy revealed a B-cell lymphoma. No other localization was found. The patient was in complete remission two years after polychemotherapy. Our observation is unusual in its clinical presentation. Mandibular lymphomas most often present as a painless swelling, sometimes ulcerated in the mouth. They are very rarely diagnosed after an isolated hypoesthesia of V3. Lymphomas are the second most frequent head and neck lymphomas after epidermoid carcinomas, but the frequency seems to be increasing. In almost all the cases, they present as B-cell tumours of the DLBCL subtype in the WHO classification. Mandibular localizations account for only 0.6% of the cases. They are often misdiagnosed as a dental problem. The complete remission rate after chemotherapy ranges from 60 to 80% at one year. Nevertheless, the prognosis remains bad with a survival rate of only 50% at five years."}, {"id": "wiki20220301en301_40254", "title": "Herpes esophagitis", "score": 0.008771929824561403, "content": "Herpes esophagitis is a viral infection of the esophagus caused by Herpes simplex virus (HSV). While the disease most often occurs in immunocompromised patients, including post-chemotherapy, immunosuppression with organ transplants and in AIDS, herpes esophagitis can also occur in immunocompetent individuals. Signs and symptoms People with herpes esophagitis experience pain with eating and trouble swallowing. Other symptoms can include food impaction, hiccups, weight loss, fever, and on rare occasions upper gastrointestinal bleeding as noted in the image above and tracheoesophageal fistula. Frequently one can see herpetiform lesions in the mouth and lips. Diagnosis"}]}}}} {"correct_option": 5, "explanations": {"1": {"exist": true, "char_ranges": [[29, 172]], "word_ranges": [[6, 30]], "text": "They are explaining erythema nodosum, so we would recommend relative rest (but antidepressants do not make any sense, so we also rule out (1))."}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[173, 360]], "word_ranges": [[30, 62]], "text": "Although erythema nodosum can be secondary to a malignant process, it is more frequent that it is due to Crohn's disease itself (moreover, in a 17-year-old boy it would be extremely rare),"}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": true, "char_ranges": [[365, 488]], "word_ranges": [[63, 84]], "text": "if the picture is also accompanied by a flare-up of inflammatory bowel disease, the last option (5) seems the most correct."}}, "full_answer": "2 and 4 are discarded alone. They are explaining erythema nodosum, so we would recommend relative rest (but antidepressants do not make any sense, so we also rule out (1)). Although erythema nodosum can be secondary to a malignant process, it is more frequent that it is due to Crohn's disease itself (moreover, in a 17-year-old boy it would be extremely rare), so if the picture is also accompanied by a flare-up of inflammatory bowel disease, the last option (5) seems the most correct.", "full_answer_no_ref": "2 and 4 are discarded alone. They are explaining erythema nodosum, so we would recommend relative rest (but antidepressants do not make any sense, so we also rule out (1)). Although erythema nodosum can be secondary to a malignant process, it is more frequent that it is due to Crohn's disease itself (moreover, in a 17-year-old boy it would be extremely rare), so if the picture is also accompanied by a flare-up of inflammatory bowel disease, the last option (5) seems [HIDDEN].", "full_question": "A 17-year-old boy with Crohn's disease with colonic involvement of 2 years of evolution, in maintenance treatment with azathioprine, consults for the appearance for 5 days of subcutaneous purplish red, hot, painful, bilateral, pretibial localization nodules, associated with an increase in the number of stools and abdominal pain. The most appropriate approach in this case is:", "id": 93, "lang": "en", "options": {"1": "Recommend relative rest and warm cloths on both legs and add antidepressant treatment.", "2": "Biopsy skin areas away from the injured areas and prescribe entry opioid analgesics.", "3": "Suspect the existence of a malignant intestinal tumor lesion as a trigger of the cutaneous process.", "4": "Suspect bilateral lower extremity ischemia of drug origin.", "5": "Adjustment of bowel disease treatment."}, "question_id_specific": 31, "type": "DERMATOLOGY, VENEREOLOGY AND PLASTIC SURGERY", "year": 2012, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1152_8812", "title": "Atypical Forms of Pyoderma Gangrenosum in Inflammatory Bowel Disease: Report of Four Cases and Literature Review.", "score": 0.01568627450980392, "content": "Cutaneous involvement is the second-most frequent extraintestinal manifestation of inflammatory bowel disease, with pyoderma gangrenosum (PG) a particularly relevant form because of its frequency, morbidity, and recurrence. The limited number of clinical trials involving PG increases the challenge to gastroenterologists in the management of this condition. Four cases of atypical presentations of PG are reported. A 25-year-old patient with ulcerative colitis presented an extensive chronic ulcerative lesion on her left leg that was associated with significant bleeding; the intestinal disease was in remission under the use of azathioprine. The patient was on long-term use of 60 mg corticosteroid with no improvement in the skin disease; however, initiation of cyclosporine induced remission. In the second case, a 52-year-old woman was a carrier of Crohn's disease, with a history of partial colectomy. The patient's skin condition had evolved with a cutaneous lesion localized in the perineal region, buttocks, and colostomy pouch, simulating a case of impetigo, and this had been treated with antibiotic cycles without improvement. Lesion biopsy suggested a diagnosis of PG. Consequently, the patient was started on biological therapy with infliximab, and the PG regressed. In the third case, a 38-year-old woman with a history of pancolitis presented a picture of PG with an extensive and deep ulcerative lesion in the right breast. The lesion regressed after treatment with oral corticosteroid. The final case was a 44-year-old woman with Crohn's disease suffering from Crohn's disease pancolitis. The patient's condition evolved with a mixed pattern with pustules, bullae, and ulcerative lesions in the vulva, oral cavity, gluteus, right auricular region, scalp, and left flank, and was resolved by administration of adalimumab. PG is an important and frequent manifestation of inflammatory bowel disease, with a spectrum of clinical variants, significant morbidity, and requiring a variety of therapeutic approaches."}, {"id": "pubmed23n1073_10285", "title": "Case Report: Systemic Small-Vessel Vasculitis in an Adolescent With Active Ulcerative Colitis.", "score": 0.015083679778275237, "content": " 100,000 Gram negative bacilli. He is admitted to the hospital, receives antibiotics, insulin and restarts acenocoumarol with good evolution. He is stable and wants to go home. The ward nurse says he is unable to self-administer insulin. Which of the following is the next step in determining the patient's safety at home?", "id": 390, "lang": "en", "options": {"1": "Refer to Primary Care and social work in the area to determine safety at home.", "2": "Formal evaluation of the capacity to make decisions concerning their health.", "3": "Evaluation to rule out dementia.", "4": "Evaluation to rule out depression.", "5": NaN}, "question_id_specific": 188, "type": "PSYCHIATRY", "year": 2016, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "wiki20220301en132_34108", "title": "House call", "score": 0.011458083113139563, "content": "The purpose of such visit is primary diagnostics and prescription of treatment and mode of conduct as well as prescribing blood, urine and other tests to be carried out at the polyclinic. The doctor also supplies the patient with a sick leave from work or study for a number of days and the leave is to be closed by the same doctor or his/her substitute and sealed at the clinic on the patient's recovery and checkout. If need be, the GP may arrange a visit to the sick person from one of specialist physicians from his/her clinic and of his/her nurse for giving injections. There are two identical state systems of outpatient clinics running parallel – for adults and for children. With the rise of private enterprise since 1990, city dwellers may place a phone order for a house call from a private medical facility (to be paid for out of patient's own money). See also Doctor's visit Home health nursing References Practice of medicine"}, {"id": "pubmed23n0500_8807", "title": "[A consideration through the support to resident diabetic patient and care giver].", "score": 0.010152545866831581, "content": "Our hospital is a National General Hospital with 585 beds. We began the visiting care service from 1990 and four visiting staffs are working at present. The number of targets was 69 in 2002 including 32 patients over 70 years old and 20 care-givers over 70 years old. Visiting care has been conduced to a 72-year-old female with diabetes since July 2000. The patient is in bedridden condition and at the beginning of visiting, she was taking oral medication but the condition was worsen by poor glycemic control and changed to insulin injection from June 2002 after admission to the hospital repeatedly. The patient undergoes the measurement of blood sugar daily and takes meals with 1,200 to 1,400 kcal prepared by her husband. The care-giver is a 71 year-old husband. He was an \"all-work, no-play\" type of person and had never done the housework, but he started to manage both housework and nursing because of bedridden of his wife. He is a reticent theorist, hates illogical behavior, and does not swayed by other opinions. He has accepted the things which need new knowledge and techniques such as measurement of blood glucose and insulin injection. However, for meals, he only bought side dishes and placed them. Care such as keeping the patient clean was in a same state. The patient consulted and admission to the hospital repeatedly because conditions were not stable. Visiting nurse supported daily life of patient and care giver especially in nutrition instruction to continue home life. As the result, attitude and behavior toward nursing of care-giver were changed and the patient could continue home life. Therefore we reported here."}, {"id": "pubmed23n0892_24815", "title": "Care under the Influence.", "score": 0.009900990099009901, "content": "A forty-year-old man is brought to the emergency room by his wife at five in the morning, two hours after he fell down the stairs at home, hitting his head and injuring his arm. He tells the ER physician that he got up to get a drink of water and tripped in the dark. His speech is slurred, and he smells strongly of alcohol. Lab results reveal elevated liver enzymes, and his blood alcohol level is 0.1. His medical history is unremarkable. When asked about his alcohol consumption, he says he usually has one or two drinks a night with dinner but that he drinks more on holidays and special occasions. He admits he had more to drink than usual last night because it had been a stressful day at work, but he is vague about how much he drank. His wife takes the ER physician aside and describes a very different situation. She says that her husband regularly has three or four drinks a night. She always goes to bed before he does and thinks he stays up later so he can continue to drink. She says that he often has no memory of conversations they had the night before and is concerned because he makes work-related calls at night. When asked what he does for a living, she hesitates, and then answers that he is an internist. He does not work at this hospital but works at one of its affiliated clinics. The ER doctor is concerned that his patient is an impaired physician. Yet when the admitting hospitalist, to whom he explains the situation, asks if he really wants to \"go there,\" he shrugs his shoulders. \"I suppose,\" she replies, \"you might as well call an ethics consult.\""}, {"id": "pubmed23n0038_5105", "title": "Relative contributions of history-taking, physical examination, and laboratory investigation to diagnosis and management of medical outpatients.", "score": 0.009900990099009901, "content": "To evaluate the relative importance of the medical history, the physical examination, and laboratory investigations in the diagnosis and management of medical outpatients some physicians recorded their diagnosis and a prediction of the method of managementafter reading the patient's referral letter, again after taking the history, and againafter performing the physical examination. These diagnoses and predictions were compared with the diagnosis and method of management which had been adopted two months after the patient's initial attendance. A diagnosis that agreed with the one finally accepted was made after reading the referral letter and taking the history in 66 out of 80 new patients; the physical examination was useful in only seven patients, and the laboratory investigations in a further seven. In only one of six patients in whom the physician was unable to make any diagnosis after taking the history and examining the patient did laboratory investigations lead to a positive diagnosis."}, {"id": "pubmed23n0648_15276", "title": "Who rules the roost?", "score": 0.00980392156862745, "content": "Adam's mother was concerned about her 3-year-old son's hyperactivity, violence, and activity level. Adam and his mom had recently moved into a shelter for pregnant women. The rest of the residents are primarily in their early 20s, whereas Adam's mom is 42. She had found about 3 months ago that she was pregnant. This was her fourth pregnancy, second with this father, and he had recently left her when she refused an abortion. Her other children are 22 and 24 and live out of state. She has a history of opioid addiction. She had been on methadone during Adam's gestation and had recently started on buprenorphine to treat her addiction during this pregnancy as well. Adam is here today for his 3-year-old checkup and you had not seen him for a year. Mom states that he has been healthy but has become progressively active over the last year. He is very angry about his dad leaving, and according to Adam's mother \"blames her\" for sending him away. They are living in 1 room at the shelter, and mom is finding it increasingly difficult to keep him busy all day. When she goes out looking for a job, he is very challenging at the shelter, and she constantly receives complaints that he is \"too loud\" in the common rooms. She feels like she is at the end of her rope with him, he is constantly climbing, bolting from her, and taking risks.When you examine Adam, you find a robust, healthy young boy. His eye contact is good, and he is socially related but does actively explore your office. When he begins taking the instruments off your wall, his mother sits passively watching him. When he begins playing with the faucet, she half heartedly tells him to \"stop\" but he looks at her and continues splashing. He then begins flicking the light switch on and off in the room with no response from mom. When you ask about discipline, mom states \"nothing works.\" When you ask about supports, she states \"I have nobody except Adam and the new baby now.\"Adam was born after an uneventful full-term pregnancy with his mother on 100 mg methadone daily. She denies cigarette smoking, drugs, alcohol, or other medications. Urine testing throughout was positive only for opioids. Motor milestones were achieved at the appropriate time. Language milestones at the 2-year-old visit consisted of 10 single words. Now, he has a 50 single-word vocabulary but no 2-word combinations. He primarily takes whatever he wants and has a tantrum if mom cannot figure out what he desires. Adam's medical history is unremarkable. Family history is significant for drug abuse by her father and mother; mental illness in the father's family consisting of bipolar disorder in several uncles. Where do you go from here?"}, {"id": "pubmed23n0351_22540", "title": "[Short-stay units depending on Internal Medicine].", "score": 0.00980392156862745, "content": "Wetry to establish the utility that a Short Stay Unit depending on Internal Medicine has for a third level hospital. This unit manages the patients under the \"appropriate stay\" concept. Several clinical and epidemic variables and sanitary indicators were studied in 867 patients. Cost was measured as the origin by average stays, explorations and readmission. Effectiveness was considered as the percentage of discharges that stay in the hospital for three days or less. The average age of the patients was 65.05 years. 55% were males. 82.24% had any previous disease. The most common diagnosis (ICD-9) were respiratory diseases, nervous system diseases and digestive diseases. The average stay of the patients was 57 hours, 2,259 explorations were ordered, it supposes an average of 0.328 urgent explorations and 2,276 UCE explorations. 310 explorations were no received when the patient was sent home. 36.56% of the patients required no explorations. 62.4% of the patients were sent home. Explorations not received had a bad influence in the average stay and in the discharges. Readmissions were 9.36%. We got hay 62.4% of the patients had a stay of 2,375 days in the hospital, With a reasonably low cost in readmissions and explorations. However it wasn't possible to establish which patient coming to the Emergency Service is appropriate for this Short Stay Unit."}, {"id": "pubmed23n0763_14041", "title": "History of head trauma in a 6-year-old boy: maybe more than meets the eye (and head).", "score": 0.009708737864077669, "content": "It is spring and you are meeting for the first time, Eddie, a recently turned 6-year-old boy who moved to the area in September of the previous year and is here for his 6-year-old health maintenance visit. Eddie's mother is concerned that although he is \"only\" in kindergarten, he is not retaining any information at school. His mother reports he knew some of his letters before kindergarten. Currently, when he is trying to write a word, for example, \"daddy\" he will need to ask his mother: \"what letter is the letter D?\" Before kindergarten, he knew his numbers 1 to 10. At times now, Eddie will forget these numbers. For example, \"if he is counting he will forget what comes after 4 and what comes after 9.\" Mother reports he will start crying for no apparent reason and if she asks why, he will say \"I don't know why.\" Mother is worried that Eddie is sad, although she denies suicidal ideation. She reports he used to like making noise with other kids, and now he cannot stand when the children are noisy. Eddie will comment he does not want to go to school because the kids make lots of noise and his head hurts. He complains of headaches as often as 2 to 3 times a month. She next states, \"This was not an issue before his head trauma.\" At this point, she reveals to you that in August, before the family relocated, Eddie fell from a 7-foot deck onto concrete while playing. He struck his head on the left side and lost consciousness for several seconds until shaken awake. He was nauseous and disoriented initially but without emesis or incontinence. He was taken to the local emergency department where he was admitted for 1 day and diagnosed with closed head injury, left frontal epidural hematoma, and question of postconcussive syndrome. Eddie has gone back to see the neurosurgeon twice over the last 6 months for scheduled visits and since the accident has had no further treatment.Eddie's mother reports that before the accident, if she read him a story from a book, he could remember the details from the story. Currently, he does not have good memory recall. Before the trauma, he did not attend a preschool program but stayed home with his mother full time. Eddie's first formal schooling has been kindergarten this year. When they moved, the neurosurgeon recommended he start school at the end of September given his head trauma in August. He had a recent computed tomography completed 3 months ago showing the epidural hematoma had completely resolved and the study was otherwise normal. His mother reports he had an evaluation the spring before the accident for a kindergarten screening test and was reported as \"excellent.\" Eddie's birth and medical history are otherwise unremarkable except for some seasonal allergies. He has not had a loss in language skill, although his mother reports he did not speak during his hospitalization. He would just stare and nod his head if someone would ask him a question. No family history of any learning or behavioral difficulties on either side of the family. Eddie has 2 older brothers, 10 and 7 years of age, with no learning issues.What would you do next?"}, {"id": "pubmed23n0126_7968", "title": "[Preventive medical examination in the aged; pros and cons].", "score": 0.009708737864077669, "content": "Preventive medical examination in aged people has some disadvantages when executed as a form of screening of the general population. There are almost no diseases to be found in aged persons, which meet the criteria for screening. It is likely that more needless disturbance is caused than actual case-finding achieved. Non-response in aged people will be high. And at last multiple pathology in aged people brings about that the total health-state is more important than the occurrence of specific diseases. These disadvantages can be partly solved by way of pre-selection of high risk aged persons. This increases the predictive value of the diagnostic tests; partly precluding non-response by means of having the first preventive examination at home; and determining the state of health in first instance by asking questions about ADL, house-keeping abilities, state of mental health, nutrition, a.o. The possibility of the community nurse paying home visits in stead of preventive medical examination will be discussed."}, {"id": "pubmed23n0956_4804", "title": "Autism Spectrum Disorder and Mental Health Comorbidity Leading to Prolonged Inpatient Admission.", "score": 0.009615384615384616, "content": "Sam is a 6-year-old boy with a diagnosis of autism spectrum disorder (ASD) who recently relocated and has an appointment with you, his new pediatric clinician, to establish care. He was previously followed by a psychiatrist for 2 years for additional diagnoses of insomnia, bipolar disorder, anxiety, attention deficit hyperactivity disorder, and intellectual disability. He has tried and (apparently) failed multiple psychotropic trials including stimulants, nonstimulants, mood stabilizers, atypical antipsychotics, and nonbenzodiazepine hypnotics. He has a delayed sleep onset and frequent night awakenings each night for the past 3 months, during which he \"screams, cries, and thrashes and can stay up for over an hour.\" His behaviors are described as irritable, self-injurious, and aggressive with no clear pattern of triggers according to his mother. He is nonverbal and communicates by leading and rarely pointing. The patient's current medication regimen includes clonidine 0.2 mg at night, lorazepam 1.5 mg as needed at night, olanzapine 5 mg twice daily, and diphenhydramine as needed for sleep/agitation. His mother is concerned that he is developing \"tolerance\" to the regimen and wants to wean him off some of the medications. His mother is struggling to take care of the patient given his worsening behavior and body habitus (body mass index >99%; z = 3.41).There is a family history of depression, anxiety, bipolar disorder, and autism. He has a 3-year-old sister, who is also diagnosed with ASD, though she is not as severely impacted. His mother's partner recently moved in along with 2 children of his own, aged 3 and 4 years. Sam attends a specialized school, where he receives behavior therapy and occupational therapy. He has undergone inpatient pediatric hospitalization twice, 1 time for 3 weeks and the other for 6 days, for aggressive behavior, and in both instances, he was discharged before inpatient psychiatric placement because of a lack of available beds.After urgent consultation with your local developmental and behavioral pediatrician, a slight reduction was made in the lorazepam because of concerns about tolerance and side effects. However, within a week of this, he was brought to the emergency department for continued self-injurious behavior and increased trouble with sleeping. His mother voiced concerns about his safety in the home, which were particularly related to aggression toward his younger sister. He was admitted to the pediatric inpatient floor for observation, and medication adjustment (increasing olanzapine), which was initially helpful in improving behavior, but mostly behavioral/environmental strategies were used to soothe him, including frequent wagon rides through the hospital corridors.Despite the patient being stable from the medical standpoint, Sam's mother did not feel comfortable taking him home. Social work contacted local community mental health services to pursue outpatient resources and respite care options and sought inpatient pediatric psychiatry. After several failed attempts to find placement, he remained in pediatric inpatient care for 1 and a half months with no acute medical interventions other than his oral medications.He was finally accepted to the in-state pediatric psychiatric facility when a bed was available. During his week-long stay, he had further medication adjustments with a decrease in olanzapine and optimization of his clonidine dose. During his psychiatric hospital stay, care coordination succeeded in arranging center-based applied behavior analysis interventions and respite care and parent training for his family. Sam began to show improvement in his overall agitation and aggression, requiring less clonazepam, and his mother then maintained outpatient follow-up.The day before discharge, you visit him in the hospital, and a medical student asks you why he was in the hospital for so long. How would you answer the question?"}, {"id": "pubmed23n0358_12691", "title": "[\"What an ideal clinical microbiological laboratory should be\"--from the position of patient].", "score": 0.009615384615384616, "content": "To think about the ideal clinical microbiological laboratory in patient's place, there are three important problems in present medical treatment. The first one, for patients, it is necessary to wait many hours to take a medical advice from a doctor. The second, patients should be checked many examinations. The third, the heavy patient's share in his medical expenses. To settle a bit these matters, the clinical laboratory should try to accept the rapid and economical examinations for patients."}, {"id": "pubmed23n0658_23905", "title": "The language and culture of delay.", "score": 0.009523809523809525, "content": "Satish is a 3 (1/2)-year-old boy you are seeing in your primary care office for a \"sick visit\" due to parental concerns about his language development. He is the only child of a couple who immigrated to the United States from India shortly before his birth. He received early intervention services for speech and language delays for a few months before he attained 2 years of age. However, services were discontinued when the family moved back to India for a year. After the family returned to the United States, they lived in a different state for several months before moving again recently to his current home, so he is relatively new to your practice. Satish's mother is concerned not only about his communication skills but also about his attention and social skills. She notes that he often plays alone or in parallel with other children. She was also told by his first pediatrician that Satish had \"a limited imagination.\" His parents feel that he has pretend play, in that he will pretend to get his haircut, talk on the phone, or ride on a train. Satish was born at term without complications. He passed his newborn hearing screen and a repeat hearing test at the age of 2 years. He has had no medical problems and takes a daily multivitamin. His parents are both of Indian descent. Satish's father is an engineer and had a history of being a late talker. His mother graduated from high school and is a homemaker. They are expecting their second child. Satish's developmental history is significant for language delays. He babbled at 6 months but did not have single words until he was 2 years. When he was 2 (1/2) years, he had 2 to 3 word sentences. He responded to his name at 15 months and could follow single step commands by the age of 2 years. Currently, Satish is noted to have difficulty with \"back and forth conversation.\" He sometimes repeats what others are saying.The family speaks Hindi, their native language, exclusively at home. When Satish speaks, he usually speaks in Hindi. His parents describe him as using \"odd language\" in that he will often mix up his pronouns. Satish is in an English-speaking preschool. His preschool teachers report concerns that he seems to \"withdraw into his own world,\" and does not interact well with the other children. They also report attentional problems and poor eye contact.In the office, Satish makes good eye contact with the examiner and his parents. He looks to his parents for approval when completing a task requested of him. He seems to like an Elmo toy that is in the room but holds it and looks at it closely rather than pretend to do anything with it. You ask him to feed Elmo, and he says, \"Feed Elmo.\" Because it is not clear whether he understands the verbal cues given to him, his parents repeat English directions to him in Hindi several times. He eventually complies but then leaves his chair to explore the room. His parents continue to translate your questions to him with variable results. He becomes increasingly difficult to engage, despite repeated attempts, in both English and Hindi, to attract his attention. Where do you go from here?"}, {"id": "wiki20220301en641_12006", "title": "Reta Mays", "score": 0.009523809523809525, "content": "In June 2015, Mays began working as a nursing assistant at the Louis A. Johnson Veterans Medical Center in Clarksburg, West Virginia with no certification or license to care for patients. Nursing assistants at the VAMC are not qualified or authorized to administer any medication to patients, including insulin. Mays was assigned to work overnight shifts on Ward 3A of the hospital's medical surgical unit in July 2017, when elderly patients began suffering mysterious, acute drops in blood sugar levels. Over the course of eleven months, hospital staff eventually attributed the deaths of several patients on the ward to hypoglycemia. Many of the deaths were of individuals who were not insulin-dependent. Four of the deaths occurred within a sixteen-day period. One patient, Archie Edgell, an 84-year-old Korean War veteran suffered a drop in his blood sugar to 24 (a reading of less than 70 is low and can be harmful) and was able to be stabilized by staff, only to die following another plummet"}, {"id": "pubmed23n0725_25452", "title": "Disclosure of diagnosis: to tell or not to tell?", "score": 0.009433962264150943, "content": "Jimmy is an 8-year-old boy with hepatitis B, e antigen (HBeAg)-positive, HIV and hepatitis C negative, who was adopted from Vietnam when he was 5 years and has been followed in your primary care practice since that time. Before adoption, he lived in an orphanage, where he was placed soon after birth. Jimmy currently lives with his adoptive mother and grandparents. His adoptive father has amyotrophic lateral sclerosis and recently moved to a nursing home due to a need for more intensive care. Jimmy continues to see him regularly.Jimmy's mother presents today upset about a recent encounter with his hepatologist. During this visit, Jimmy's doctor was insistent that Jimmy should be told about his illness immediately. He felt that Jimmy \"had a right to know\" and that it was important for the protection of other children. Jimmy's family practices universal precautions and Jimmy is compliant with these safety measures. Jimmy's mother has chosen not to share his diagnosis with the school and in addition has not felt the time was right to disclose the diagnosis to Jimmy. He is asymptomatic, takes no medications, and is followed yearly by a hepatologist. His mother is concerned that Jimmy would have difficulty managing this information and maintaining a \"secret.\" However, she also worries that he may feel his trust has been violated if she delays telling him.Jimmy is currently 8 years old, in second grade, and is struggling academically with math and reading. Socially, he is reported to have difficulty making friends and reading social cues. For example, he displays inappropriate boundaries, often standing too close or touching others, which has resulted in children avoiding him. During your annual visit, Jimmy presented as a friendly and engaging boy. He maintained conversation about school and some of his interests, but he was often distractible, impulsive, at times grabbing things, and fidgety, frequently standing up and then sitting back down. Jimmy's mother reports that this behavior is similar to what he exhibits in the classroom. He is currently receiving English as a Second Language services and is enrolled in a weekly \"lunch bunch.\" What advice would you give the family?"}, {"id": "pubmed23n0360_16804", "title": "[Criteria for evaluation in family medicine].", "score": 0.009433962264150943, "content": "For successful evaluation and the follow up of the family medicine team work it is necessary to develop evaluation criteria. Monitoring and evaluation criteria are numerous and different among countries depending on the health care development and the economic power of the country. In the present circumstances, taking in consideration health care reform through Primary Health Care (PHC) through family medicine team, the following criteria are proposed: equity, availability, cost-effectiveness, efficiency, efficacy, quality and satisfaction of user and health services providers. Indicators for the above mentioned criteria are also defined to estimate criteria achieved development. First indicator group relates on the medical-social indicator (for example number of families, structure of families and structures of concerning population) and the others social-economic indicators (GNP, expenditure in the health and social sector per capita, etc.). Second indicator group follows up the content of family medicine team work as follows: indicator of active and passive health care (for example number of doctor services, number of doctor and nurses home visits, consultation services, diagnostic procedures etc.). Third indicator group relates on the economic aspect, meaning the cost of implementation of such program, according indicator on content of work. Suggested criteria with indicators present the basic minimum databases, which would give the possibilities to have continuous overview in family medicine team functioning as well in its quality of work."}, {"id": "pubmed23n0772_17523", "title": "Bullying and ADHD: which came first and does it matter?", "score": 0.009345794392523364, "content": "Aiden, a 13-year-old boy in the sixth grade who is relatively new to your practice, is seen for follow-up after his routine physical last month when you noted concerns for possible attention-deficit hyperactivity disorder (ADHD) and gave the family Vanderbilt Scales to complete. Aiden has a family history of ADHD, specific learning disabilities, and mood disorder.His mother reports that she is concerned about how Aiden is doing at school; his teachers are complaining that he is not doing his work, and she is worried that he may be kept back in school. Aiden first began having trouble in the third grade. He was retained in the fourth grade for academic and behavioral reasons. Now his mother has been receiving calls about him not paying attention, distracting others, and staring at his paper. At home, he does not want to do homework and gets very frustrated. In fifth grade, he had a psychoeducational evaluation and was found not eligible for services. His achievement testing showed average scores in reading, math, and writing. Cognitive testing demonstrated average scores for verbal and nonverbal abilities and memory but was significantly below average for processing speed. Aiden continues to have problems now in into the sixth grade.You speak with Aiden in the office and ask him about school. He says, \"It's bad. I'm failing.\" He believes his major problems at school are that he is not doing his homework, he easily becomes frustrated, and he argues with the teachers. He has supportive relationships with his family and friends at school. He gets along well with some of his teachers, noting that he loves his science teacher even though she is tough and \"gives hard homework.\" He describes his history teacher as \"annoying.\" When you ask what he means he states this teacher \"Can be not nice and says mean things. She picks on me a lot.\" His description is consistent with the use of shaming as a behavior he experiences at school.You review the completed parent and teacher Vanderbilt forms; both are consistent and concerning for combined type ADHD. You discuss the diagnosis of ADHD with his mother and both agree to revisit pharmacotherapy in September when the school year resumes. You give her resources on ADHD and classroom accommodations and discuss requesting a 504 plan at school. You also discuss behavioral therapy to better address his self-regulation skills.A week later, you receive a telephone call from Aiden's mother. \"Aiden got home today and he is more upset than I have ever seen him! His teacher told him in front of the class that he would probably stay back a year and now he is saying there is no point in going to school.\" She is not aware if retention has been recommended for Aiden.What would you say to Aiden's mother? What would you do next?"}, {"id": "pubmed23n0546_2106", "title": "Technical notes: when all things are not equal.", "score": 0.009345794392523364, "content": "This article addresses 2 questions. First, how useful is adult patients' information about health and healthcare when they use the Internet for a \"health checkup\"? We find that patietns' reports are very strongly associated with medical record information for blood pressure, cholesterol, and blood glucose. Second, what are the biases in information from Internet respondents? Although we find that \"health checkup\" Internet users seem to be representative for patients in actual practice, much more research will be needed to fully address this question."}, {"id": "pubmed23n0895_1202", "title": "Bullying and ADHD: Which Came First and Does it Matter?", "score": 0.009259259259259259, "content": "Aiden, a 13-year-old boy in the sixth grade who is relatively new to your practice, is seen for follow-up after his routine physical last month when you noted concerns for possible attention-deficit hyperactivity disorder (ADHD) and gave the family Vanderbilt Scales to complete. Aiden has a family history of ADHD, specific learning disabilities, and mood disorder.His mother reports that she is concerned about how Aiden is doing at school; his teachers are complaining that he is not doing his work, and she is worried that he may be kept back in school. Aiden first began having trouble in the third grade. He was retained in the fourth grade for academic and behavioral reasons. Now his mother has been receiving calls about him not paying attention, distracting others, and staring at his paper. At home, he does not want to do homework and gets very frustrated. In fifth grade, he had a psychoeducational evaluation and was found not eligible for services. His achievement testing showed average scores in reading, math, and writing. Cognitive testing demonstrated average scores for verbal and nonverbal abilities and memory but was significantly below average for processing speed. Aiden continues to have problems now in into the sixth grade.You speak with Aiden in the office and ask him about school. He says, \"It's bad. I'm failing.\" He believes his major problems at school are that he is not doing his homework, he easily becomes frustrated, and he argues with the teachers. He has supportive relationships with his family and friends at school. He gets along well with some of his teachers, noting that he loves his science teacher even though she is tough and \"gives hard homework.\" He describes his history teacher as \"annoying.\" When you ask what he means he states this teacher \"Can be not nice and says mean things. She picks on me a lot.\" His description is consistent with the use of shaming as a behavior he experiences at school.You review the completed parent and teacher Vanderbilt forms; both are consistent and concerning for combined type ADHD. You discuss the diagnosis of ADHD with his mother and both agree to revisit pharmacotherapy in September when the school year resumes. You give her resources on ADHD and classroom accommodations and discuss requesting a 504 plan at school. You also discuss behavioral therapy to better address his self-regulation skills.A week later, you receive a telephone call from Aiden's mother. \"Aiden got home today and he is more upset than I have ever seen him! His teacher told him in front of the class that he would probably stay back a year and now he is saying there is no point in going to school.\" She is not aware if retention has been recommended for Aiden.What would you say to Aiden's mother? What would you do next?"}, {"id": "pubmed23n0050_1938", "title": "[Diabetes in elderly subjects].", "score": 0.009259259259259259, "content": "Problems raised by diabetes in subjects above 65 years of age are of three kinds: diagnostic, therapeutic and social. Passed the age of 40, there is a regular physiological increase of glycaemia, and this must be taken into account to diagnose diabetes and to set the threshold for therapeutic intervention. The treatment of elderly people has its specific imperatives, due to feeding habits and age-related changes in hepatic and renal functions. The presence of complications, sometimes multiple, explains that the patient often takes several drugs, which increases the risk of interaction between these drugs and oral antidiabetics. The therapeutic objectives must be adjusted to these imperatives, and neither the diabetic... nor his doctor must be afraid of insulin. The social status of elderly patients must not be neglected: the help of private nurses and the training in diabetology of the patient's entourage often permit to postpone the date of institutionalization."}, {"id": "pubmed23n1009_24025", "title": "Toddler Sleep Challenges: All in a Day's Work.", "score": 0.009174311926605505, "content": "Leo is a 26-month-old boy who you are seeing for an urgent care visit due to \"sleep difficulty,\" particularly sleep onset. Since age 1, he screams, hits, and kicks his mother every day, starting after she gets home from work at 5 PM (or before the family's dinnertime on her days off) and escalating over the course of the evening until he \"wears himself out\" and falls asleep in a crib in his own room around 9 to 10 PM Once asleep, he sleeps well through the night and wakes easily around 7 AM in a pleasant mood; his mother leaves for work soon after he awakens. He naps after lunch for 2 to 3 hours on weekdays at an in-home child care with 1 to 2 adult caregivers and 5 other children aged 0 to 5 years. He refuses to nap at home.Leo goes to bed easily when his father puts him to bed if his mother is not at home, but his mother feels that evenings are the only time she can spend with Leo, and so, she tries to put him to bed most nights. However, because of Leo's behaviors at bedtime with her, she feels inadequate, depressed, and guilty; when she tries to disengage or allow her husband to help, Leo screams, \"Mommy, mommy!\" and tries to gain access to her and resists his father putting him to bed until his mother returns. Both parents worry that \"he would not grow out of this,\" and his mother now avoids coming home from work for fear of Leo's behavior. Both parents feel that this situation is causing marital strain.Leo was born healthy at full-term and is an only child; pregnancy was complicated by hyperemesis gravidarum. Leo has been healthy and meeting developmental milestones. His parents describe his temperament as \"like his father at that age,\" \"easy, but never able to self-soothe,\" \"intense\" in his emotional reactions, persistent, \"strong-willed and serious,\" and \"shy and observant, withdrawn at first and then getting more pleasant after a while\" in novel situations. Behaviorally, he engaged in noninjurious head-banging at home when upset between 12 and 15 months; bit children a few times at child care between 20 and 24 months; and lately refuses to share or will push other children at child care every few weeks. His parents recently read a book about parenting \"spirited\" children but did not find it helpful. What would you do next?"}, {"id": "pubmed23n0574_2710", "title": "[Impact of peer visits].", "score": 0.009174311926605505, "content": "To measure the impact of an intervention on the general practitioners (GP) of Reunion in order to improve the management of patients with type 2 diabetes, in conforming with the recommendations of the Anaes. Randomised intervention study on a random sample of 120 practitioners out of a total of 630 GP in Reunion, 60 in the intervention group (IG) and 60 in the control group (CG). Each doctor of the IG received 2 visits by a \"visiting GP\" which have had specific training. The period of observation included the 12 months before, and the 6 months after the date of the intervention. Data were collected retrospectively, at the end of 18 months of observation; in medical records of 25 diabetics seen consecutively in consultation, the GP collected the dates of performance of six procedures under surveillance: HbA1c, examination of the feet, fundoscopy, ECG, estimation of the creatinine clearance, level of micro-albuminuria. Outcome measures were delays in performance of the procedures conforming to the recommendations. 42 GP out of 60 in the IG, and 40 out of 60 in the CG participated to the study. Patients included, 792 in the IG and 789 in the CG, were comparable for age, sex-ratio and profession. The distribution of delays in performance before the intervention was comparable in the two groups. The comparison between the groups after the intervention showed a significantly important improvement in the IG for 4 to 6 of the procedures: examination of the feet, fundoscopy, creatinine clearance and micro-albuminuria. In the short-term, a \"outreach visit\" or \"academic detailing\" improves the delay in performance of most of the surveillance procedures in type 2 diabetes."}, {"id": "pubmed23n0796_16550", "title": "Attention deficit hyperactivity, fetal alcohol spectrum disorder, or something else: the broad differential of kindergarten suspension.", "score": 0.00909090909090909, "content": "Thomas is a 5-year 6-month-old boy whose parents requested an urgent care appointment because he has recently been suspended from kindergarten stating \"and his doctor must see him before he can come back.\" His suspension from kindergarten was due to kicking and biting his classmates, but he has also become increasingly aggressive at home. His teacher reported that he has always had a high activity level and difficulty shifting attention between tasks, as well as noncompliance with rules and directions. He is noted to have learning challenges and is showing difficulties in the concept of numbers and letter sounds. The practice has followed Thomas since his healthy birth. He has a history of delayed language development, and he received early intervention services from 2 years of age. He spoke his first word at 2 years 6 months. He started a half-day preschool program at 3 years of age. He had difficulty acclimating to preschool, interacting with peers, and was described as \"hyperactive\" by his teachers. His program was modified to decrease his time having to sit in a circle time, and he often required the support of the paraprofessional in the classroom. His parents have always described him as a \"difficult child.\" He gets frustrated easily and can tantrum for up to 2 hours multiple times in a week when his immediate needs or requests are not met. He has difficulty falling asleep, has frequent night awakenings, and often has trouble getting back to sleep. His self-help skills are poor, and he has difficulty with activities such as brushing his teeth and dressing. His parents report that he does not seem to remember rules from day to day. He was evaluated at 5 years of age and diagnosed with Attention Deficit Hyperactivity Disorder, but his response to stimulants has been limited. Thomas is an only child. His parents are college educated and professionally employed. They deny drug use, domestic violence, and guns in the home. They reported that prior to the pregnancy, they enjoyed \"partying\" with friends on the weekends, but Thomas's mother reported that she stopped drinking as soon as she realized she was pregnant. All are wondering whether this child might have a fetal alcohol spectrum disorder, although he seems to have no clear facial dysmorphology. It is unsure what the next step might be and if there is value added in pursuing this diagnosis. What do you do next?"}, {"id": "wiki20220301en026_106322", "title": "Claus von Bülow", "score": 0.00909090909090909, "content": "In 1982, Bülow was arrested and tried for the attempted murders of Sunny on two occasions in two consecutive years. The main medical and scientific evidence against him was that Sunny had low blood sugar, common in many conditions, but a blood test showed a high insulin level. The test was not repeated. A needle was used as evidence against Bülow in court, with the prosecution alleging that he had used it and a vial of insulin to try to kill his wife. His mistress of two years, the soap opera actress Alexandra Isles, testified \"He said that they had been having a long argument, talk, about divorce that had gone on late into the night. She had drunk a great deal of egg nog. And then he said, ‘I saw her take the Seconal.’ And then he said that the next day when she was unconscious that he watched her knowing that she was in a bad way, all day, and watched her and watched her. And finally, when she was at the point of dying he said that he couldn’t go through with it and he called (the"}, {"id": "pubmed23n0481_5488", "title": "[A consideration through the support to resident diabetic patient and care-giver].", "score": 0.009076142131979695, "content": "Our hospital is a National General Hospital with 585 beds. We began the visiting care service from 1990 and four visiting staffs are working at present. The number of targets was 69 in 2002 including 32 patients over 70 years old and 20 care-givers over 70 years old. Visiting care has been conducted to a 72-year-old female with diabetes since July 2000. The patient is in bedridden condition and at the beginning of visiting, she was taking oral medication but the condition was worsen by poor glycemic control and changed to insulin injection from June 2002 after admission to the hospital repeatedly. The patient undergoes the measurement of blood sugar daily and takes meals with 1,200 to 1,400 kcal prepared by her husband. The care-giver is a 71-year-old husband. He was an \"all-work, no-play\" type of person and had never done the housework, but he started to manage both housework and nursing because of bedridden of his wife. He is a reticent theorist, hates illogical behavior, and does not swayed by other opinions. He has accepted the things which need new knowledge and techniques such as measurement of blood glucose and insulin injection. However, for meals, he only bought side dishes and placed them. Care such as keeping the patient clean was in a same state. The patient consulted and admission to the hospital repeatedly because conditions were not stable. Visiting nurse supported daily life of patient and care-giver especially in nutrition instruction to continue home life. As the result, attitude and behavior toward nursing of care-giver were changed and the patient could continue home life. Therefore we reported here."}, {"id": "pubmed23n0704_22995", "title": "Parents seek early intervention services for a two-year-old without autism.", "score": 0.009009009009009009, "content": "Sam is a 27-month-old boy who you have followed since birth. He lives with his parents in a small resort town approximately 90 miles outside a major city. Both his parents are professionals in their late 30s and have been highly involved in his care since birth. At the 12-month visit, they were concerned about his difficulty regulating. He was not sleeping through the night and had significant difficulty with baths. His physical examination and growth were normal. His eye contact was good, although it was difficult to see him smile. He had 1 or 2 words and was beginning to walk independently.At the 15-month checkup, they continued to be concerned about his poor regulation. He napped sporadically, and he was very difficult to take out on errands as he did not like his car seat. He now had approximately 10 single words, was using his fingers to point, and very clearly waved \"bye bye\" as soon as you entered the room.At the 18-month checkup, they state that he has not yet learned the word \"no.\" He will follow a 1-step command when he wants to but now has 15 single words without any combinations. He points for his needs and to show them something. He has become increasingly \"shy\" around strangers and prefers to play with one other child as opposed to a larger group. He does not like loud noises and prefers to go barefoot constantly. His physical examination was again normal as was his growth. He is referred for a full hearing evaluation, which is also normal. The family was referred to early intervention, and he began receiving speech and language therapy and occupational therapy for his sensory challenges as well as a play group.At the 24-month checkup, his language continued to consist of single words-now approximately 30. When the parents do not understand what he wants, he will often tantrum and has started banging his head on the floor when frustrated. He has no repetitive behaviors and is starting to demonstrate imaginative play. Bath time has becoming increasingly challenging because he does not like the sensation of soap and the water temperature must be \"just right.\" You refer the child to a Developmental and Behavioral Pediatrician for evaluation and at 28 months he is seen. During his testing visit, he had decreased eye contact and followed his own agenda but improved significantly as testing progressed. As he got more comfortable, he began making good eye contact, social referenced, and exhibited joint attention with his parents and the examiner. He did not meet criteria for an autism spectrum disorder or specifically pervasive developmental disorder-not otherwise specified (PDD-NOS). He was given a diagnosis of mixed receptive and expressive language delay and disruptive behavior disorder with sensory processing problems.The parents come to you a month after their evaluation visit asking you to give him a \"listed diagnosis of PDD-NOS\" that could be removed when he turns 3 years so that he may qualify for increased hours of services-up to 15 hours per week-as well as applied behavioral analysis therapy. A behavioral therapist through early intervention has told the family that he would benefit from this increased intervention, specifically applied behavioral analysis but the only way he can receive it is with a \"medical diagnosis\" on the autism spectrum. What do you do next?"}, {"id": "pubmed23n0086_10203", "title": "[Why are blood pressure values higher in the doctor's office than at home?].", "score": 0.009009009009009009, "content": "Blood pressure values measured by the patients at home are lower than those measured during medical consultation. To test whether the person measuring the blood pressure is responsible for this difference, the blood pressure of 127 patients was measured first by the doctor and then by the patients themselves during the consultation. There was a good agreement and no significant difference between the two measurements. Values taken at home were however significantly lower. Our results indicate, that difference between clinic and home blood pressure values does not depend on the person performing the measurement."}, {"id": "pubmed23n0839_18211", "title": "Electrocardiogram before starting stimulant medications: to order or not?", "score": 0.008928571428571428, "content": "D is an 8-year-old boy brought to his paediatrician for evaluation. His mother is concerned as his teacher has been frequently complaining that he is very restless and often disturbs the rest of the class by getting up on some pretext or the other. He is unable to concentrate on his work and gets distracted very easily. He makes many careless mistakes and can hardly finish his tasks on time. He is frequently reprimanded for talking during class. He often answers out of turn or before the question has been completed; however, so far, he has been managing to get passing grades. At home, he is constantly on the go while he is awake. If he is forced to sit, like at mealtimes, he fidgets a lot. He also needs to be constantly nagged to do everything, even his daily activities such as brushing his teeth, or he forgets to do them or leaves them incomplete. He takes ages to finish his food. It is a major job to get him to do his homework. His mother says that at home he has been like that since the last 2 to 3 years, but now she is concerned because of the difficulties he is experiencing at school as well. After obtaining his medical history, examination, and getting response from parents and teachers--using Vanderbuilt Assessment Scales--the paediatrician diagnoses him to have attention deficit hyperactivity disorder. Besides behavioural interventions, he considers medications for his management. The paediatrician is debating the merits of performing electrocardiogram and/or referring the boy to a cardiologist before starting stimulant medications. If you were caring for this patient, how would you proceed?"}, {"id": "pubmed23n0070_12917", "title": "Screening elderly people in primary care: a randomized controlled trial.", "score": 0.008928571428571428, "content": "A randomized controlled trial was carried out to test the effectiveness of a screening programme carried out by nurses for elderly people aged 75 years and over in a general practice. A total of 151 people were randomly allocated to the test group and 145 to the control group. The test group received a home visit from a nurse at which an assessment lasting 45 minutes was made of: activities of daily living, social functioning, sensory functions, mental and emotional problems, current medical problems, blood pressure, urinalysis, haemoglobin level and compliance with medication. Both groups completed a selection of items from four health indices before and 20 months after the intervention. At follow up, the test group scored significantly better than the control group on a morale scale. However, this trial provided no evidence for better resolution of physical problems or finding activities of daily living easier in the test group compared with the control group. It is suggested that the main benefit of such a screening process is that the special attention and education provided improves adaptation to old age and awareness of the support systems available. The government has proposed an annual review of elderly people in their own home and this study suggests that the objectives of this scheme should be clarified."}, {"id": "pubmed23n0349_16016", "title": "A school-aged child with delayed reading skills.", "score": 0.008849557522123894, "content": "During a health supervision visit, the father of a 7.5-year-old African American second-grader asked about his son's progress in reading. He was concerned when, at a recent teacher-parent conference to review Darren's progress, the teacher remarked that Darren was not keeping up with reading skills compared with others in his class. She said that he had difficulty sounding out some words correctly. In addition, he could not recall words he had read the day before. The teacher commented that Darren was a gregarious, friendly child with better-than-average verbal communication skills. His achievement at math was age-appropriate; spelling, however, was difficult for Darren, with many deleted letters and reversals of written letters. A focused history did not reveal any risk factors for a learning problem in the prenatal or perinatal periods. Early motor, language, and social milestones were achieved on time. Darren had not experienced any head injury, loss of consciousness, or chronic medical illness. He had several friends, and his father denied any behavioral problems at home or at school. His teacher completed a DSM-IV-specific behavioral survey for attention-deficit/hyperactivity disorder (ADHD). It did not show any evidence of ADHD. Darren's father completed 1 year of college and is currently the manager of a neighborhood convenience store. His mother had a high school education; she recalled that she found it difficult to complete assignments that required reading or writing. She is employed as a waitress. Darren does not have any siblings. The pediatrician performed a complete physical examination, the results of which were normal, including visual acuity, audiometry, and a neurological examination. It was noted that Darren seemed to pause several times in response to questions or commands. On two occasions, during finger-nose testing and a request to assess tandem gait, directions required repetition. Overall, he was pleasant and seemed to enjoy the visit. His pediatrician concluded that he had a learning problem but she was uncertain about the next step. She asked herself, \"Is there anything else I can do in the office to evaluate Darren's problem with learning? Should I quickly refer him for educational testing or encourage a reading tutor? What questions can I ask his teacher that would be helpful? Am I missing a medical disorder?\""}, {"id": "pubmed23n0359_3460", "title": "[Effects of written intervention on the number of laboratory studies per patient].", "score": 0.008849557522123894, "content": "In the context of economic measures in health care we followed over a period of nine months the consequences of a written intervention on the attitude of house staff to prescribe laboratory tests. Since it is well known that these tests have a major impact on health costs several studies have been conducted to test whether costs can be reduced without sacrifice of treatment quality. The study was undertaken in 1997. It had three phases of three months duration each: one for observation, one with the intervention and a follow-up phase. The laboratory tests requested by eleven physicians for their patients during the first month after the initial visit were analyzed. During the intervention--phase six physicians chosen at random were informed about their own average as well as that of the entire group (mean of the entire observation period of all physicians). Unexpectedly the hypothesis that the number of laboratory tests requested per patient would drop only in the group of informed physicians and should stay the same for the physicians without this intervention did not materialize. The number of performed tests dropped in both groups."}, {"id": "pubmed23n0659_1451", "title": "Scott: an 11-year-old boy with repetitive lying.", "score": 0.008771929824561403, "content": "Scott, an 11-year-old boy in the fifth grade, is brought to his pediatrician, Dr. Lewis, by his maternal grandparents with the principle concern that \"he lies constantly.\" Scott lived with his maternal grandparents since he was 2 years old, and they have full custody. His mother and father had serious substance abuse problems. The grandparents provide a stable home for Scott and his 15-year-old sister. Scott has had no contact with his mother in more than 6 years and sees his father infrequently. During the last visit with his father, he was so inebriated that he was thrown out of the movie theatre and barely avoided several car accidents on the way home. He left the children at the curb of their home and made them promise that they would lie to their grandparents about the reasons for the early return. Scott was diagnosed with attention-deficit hyperactivity disorder (ADHD) in second grade. Methylphenidate (36 mg) provides improvement in attention and concentration. His grandfather describes Scott as highly unpredictable. When he is the \"good Jake,\" he is eager to help, polite, and caring. When Scott gets behind in school or is avoiding his chores and assignments, he lies by saying that he got it all done, even though he knows his grandfather will discover the lie and punish him. When confronted with reports from school, Scott often lies and may develop more elaborate confabulatory stories. His grandfather admits that he becomes irate at these moments. He responds by removing Scott's privileges. When he planned to take Scott to see his favorite sport team in the playoffs, Scott was caught in a lie the day of his departure. His grandfather offered him a chance to fess up, pay a small price in extra chores, and save the trip. Scott stubbornly refused to admit that he lied and lost the trip. His grandfather worries that Scott has no \"moral compass.\" He takes things that do not belong to him and violates household curfew rules. He has never been physically aggressive or has never stole items from a store. He takes his sister's CD player or his grandfather's cell phone even when he has been told not to. He will then lie that he did not take it. Even when it is pulled out of his backpack, he will say he did not put it in there. His grandfather is a businessman with high moral integrity. He loves his grandson and is eager to help him. He asks Dr. Lewis what they should do about Scott's persistent lying."}, {"id": "wiki20220301en054_78737", "title": "Lot (unit)", "score": 0.008771929824561403, "content": "A lot is an old unit of weight used in many European countries since the Middle Ages until the beginning of the 20th century. Most often it was defined as either or of a pound (or more precisely of whatever mass value one local pound had at the time). Recorded values range from 10 to 50 grams. In the Imperial and US customary systems of measurement, a lot is of a pound, or an ounce, making it exactly 14.174 761 562 5 grams if derived from the international pound. See also Old Polish units of measurement Obsolete Finnish units of measurement Obsolete Russian units of measurement Obsolete Tatar units of measurement External links :sk:Libra (hmotnosť) an extensive (20+) list of values (in grams) a pound had at various times and places in Europe :bg:Фунт (единица) a historical value of lot in Bulgaria Obsolete units of measurement Units of mass"}, {"id": "pubmed23n1007_19220", "title": "Maintaining Safety and Planning for the Future.", "score": 0.008695652173913044, "content": "Kevin is a 12-year-old boy with autism spectrum disorder, intellectual disability (nonverbal IQ scores in mid-40s), and attention-deficit/hyperactivity disorder who has been followed up by a developmental-behavioral pediatrician (DBP) and a child psychologist for medication and behavioral management since he was 4 years old. Kevin was placed in the care of his great-great-aunt shortly after he turned 2 years of age because of concerns of neglect. She is now his legal guardian.Kevin is predominately nonverbal but does use a few single words to make requests or label items. He attends a public school and receives full-time special education support. He has a personal care assistant (PCA) who provides in-home support 5 to 6 days/wk for 3 to 4 hours at a time. The PCA is working on toilet training, using a \"clock-training\" approach, and also takes Kevin outdoors to play or on short outings during her visits. In his free time, Kevin prefers to watch cooking shows on television.Over the past year, Kevin's behaviors have become more concerning. There have been several episodes of Kevin waking up during the early morning hours and going to the kitchen to \"cook.\" After one of these episodes, his guardian was not aware that Kevin had woken up until the next morning when she found a concoction of corn starch, coffee grounds, cottage cheese, and powdered drink mix in the blender. Kevin had also woken up during the night and ventured out of the house into the back yard. His guardian had woken up immediately as the alarm system sounded when he opened the outer door from the house to the yard.A door alarm was added to Kevin's bedroom door so that his guardian would be alerted when he leaves his bedroom; however, the alarm is not used consistently because there are times when the alarm cannot be found at bedtime. Kevin's guardian was able to obtain a GPS device for him to wear on his shoe from the local police department. He wears this without resistance every day.Kevin's guardian is in her mid-70s, and she has had several health issues over the past 2 to 3 years. There are no other family members who are willing or able to care for Kevin if his guardian were no longer able to. The DBP and child psychologist have encouraged Kevin's guardian to explore long-term residential care options with the state agency that provides support for individuals with intellectual disabilities and with Kevin's insurance provider, but the guardian is very reluctant to do this. She fears that Kevin will be removed from her care or placed in a \"home\" where someone will \"do bad things to him.\"What else would you recommend or actions would you take to support Kevin's guardian in ensuring Kevin's safety and planning for his future care?"}]}}}} {"correct_option": 3, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": true, "char_ranges": [[0, 231]], "word_ranges": [[0, 31]], "text": "They describe a characteristic case of CADASIL (Cerebral Autosomal Dominant Arteriopathy with Sub-cortical Infarcts and Leukoencephalopathy). It is a genetic disease of autosomal dominant inheritance by mutation of the NOTCH3 gene."}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "They describe a characteristic case of CADASIL (Cerebral Autosomal Dominant Arteriopathy with Sub-cortical Infarcts and Leukoencephalopathy). It is a genetic disease of autosomal dominant inheritance by mutation of the NOTCH3 gene. This mutation causes vascular involvement of small vessel that generates severe leukoencephalopathy early, which generates various symptoms. Migraines, cerebrovascular disease and cognitive impairment at early ages stand out, as shown in the clinical case of the question.", "full_answer_no_ref": "They describe a characteristic case of CADASIL (Cerebral Autosomal Dominant Arteriopathy with Sub-cortical Infarcts and Leukoencephalopathy). It is a genetic disease of autosomal dominant inheritance by mutation of the NOTCH3 gene. This mutation causes vascular involvement of small vessel that generates severe leukoencephalopathy early, which generates various symptoms. Migraines, cerebrovascular disease and cognitive impairment at early ages stand out, as shown in the clinical case of the question.", "full_question": "A 45-year-old male brought to the consultation by his relatives for a one-year history of memory loss, which has progressed to the point of needing help with some activities of daily living. He has a history of migraines and some episode of self-limited neurological focality for which he has never consulted. The neuropsychological examination is compatible with an initial dementia and the brain MRI shows a severe leukoencephalopathy. Which test should be ordered?", "id": 577, "lang": "en", "options": {"1": "Lumbar puncture to analyze amyloid and tau protein in CSF.", "2": "Genetic study for presenilin 1 (PSEN1).", "3": "Genetic study for NOTCH3.", "4": "Genetic study for progranulin.", "5": NaN}, "question_id_specific": 105, "type": "NEUROLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0617_13878", "title": "Three-year follow-up of a patient with early-onset Alzheimer's disease with presenilin-2 N141I mutation - case report and review of the literature.", "score": 0.018311036789297658, "content": "Autosomal dominant early-onset Alzheimer disease (EOAD) is a heterogeneous condition that has been associated with mutations in 3 different genes: the amyloid precursor protein (APP), presenilin 1 (PSEN1), and presenilin 2 (PSEN2) genes. Most cases are due to mutations in the PSEN1 gene, whereas mutations in the APP and PSEN2 genes are rare. Mutation analysis of the APP, PSEN1 and PSEN2 genes was performed. We herein report the case of a German EOAD patient with a family history of dementia and a missense mutation at codon 141 (N141I) of the PSEN2 gene. To our knowledge, this is the first German EOAD patient without a Volga-German ancestry and a positive family history for dementia carries the mutation PSEN-2 N141I. The patient came to our clinic for the first time when she was 47 years old. During the following 3 years, her Mini-Mental State Examination (MMSE) score dropped from 28 to 0. Mild cognitive impairment (MCI) was an early symptom that was already present during the first consultation. The concentration in cerebrospinal fluid (CSF) of tau-protein (1151 pg/ml) was increased, whereas the concentration of beta-amyloid protein (Abeta1-42) was decreased (335 pg/ml). Magnetic resonance imaging (MRI) revealed only slight changes in the early stage of the disease and positron emission tomography with (18F) fluoro-2-deoxy-D-glucose (18F-FDG PET) demonstrated glucose reduction left parietal and in the precuneus region. Follow-up MRI and 18F-FDG PET studies showed progression of atrophy of the left entorhinal cortex with relative sparing of the hippocampus and progressive hypometabolism of both temporoparietal lobes and left frontal lobe."}, {"id": "pubmed23n0323_3218", "title": "Early-onset Alzheimer's disease due to mutations of the presenilin-1 gene on chromosome 14: a 7-year follow-up of a patient with a mutation at codon 139.", "score": 0.01801948051948052, "content": "Mutations in the presenilin-1 gene (PS-1 gene) on chromosome 14 have recently been identified as a cause of familial early-onset Alzheimer's disease (EOAD). To our knowledge, only two German EOAD patients with mutations in the PS-1 gene have been identified thus far. Herein we report the case of a German EOAD patient with a family history of dementia and a missense mutation at codon 139 (M139V) of the PS-1 gene. The patient came to our clinic for the first time when he was 44 years old. During the following 7 years, his Mini-Mental State Examination (MMSE) score dropped from 24 to 0. Myocloni were an early neurological symptom that was already present during the first consultation. We could demonstrate that myoclonic activity was of cortical origin using a back-averaging method. Magnetic resonance imaging (MRI) revealed only slight changes in the early stage of the disease. Follow-up MRI studies showed progression of bitemporal ventricular enlargement and progressive frontal and temporal cortical atrophy. Although the majority of EOAD patients belong to the sporadic (non-genetic) type of AD, early-onset dementia, early myocloni and a familial history of AD should direct attention to the possibility of a genetic form of AD."}, {"id": "pubmed23n1143_18527", "title": "SORL1 gene mutation and octapeptide repeat insertion in PRNP gene in a case presenting with rapidly progressive dementia and cerebral amyloid angiopathy.", "score": 0.016372627639668538, "content": "Cerebral amyloid angiopathy (CAA) has been associated with a variety of neurodegenerative disorders, included prion diseases and Alzheimer's disease; its pathophysiology is still largely unknown. We report the case of an 80-year-old man with rapidly progressive dementia and neuroimaging features consistent with CAA carrying two genetic defects in the PRNP and SORL1 genes. Neurological examination, brain magnetic resonance imaging (MRI), electroencephalographic-electromyographic (EEG-EMG) polygraphy, and analysis of 14-3-3 and tau proteins, Aβ40, and Aβ42 in the cerebrospinal fluid (CSF) were performed. The patient underwent a detailed genetic study by next generation sequencing analysis. The patient presented with progressive cognitive dysfunction, generalized myoclonus, and ataxia. Approximately 9 months after symptom onset, he was bed-bound, almost mute, and akinetic. Brain MRI was consistent with CAA. CSF analysis showed high levels of t-tau and p-tau, decreased Aβ42, decreased Aβ42/Aβ40 ratio, and absence of 14.3.3 protein. EEG-EMG polygraphy demonstrated diffuse slowing, frontal theta activity, and generalized spike-waves related to upper limb myoclonus induced by intermittent photic stimulation. Genetic tests revealed the presence of the E270K variant in the SORL1 gene and the presence of a single octapeptide repeat insertion in the coding region of the PRNP gene. The specific pathogenic contribution of the two DNA variations is difficult to determine without neuropathology; among the possible explanations, we discuss the possibility of their link with CAA. Vascular and degenerative pathways actually interact in a synergistic way, and genetic studies may lead to more insight into pathophysiological mechanisms."}, {"id": "pubmed23n0419_19675", "title": "Presenilin 1 mutation in an african american family presenting with atypical Alzheimer dementia.", "score": 0.015914786967418545, "content": "Alzheimer disease (AD) is characterized by memory and visuospatial deficits with relative sparing of personality. Mutations in 3 genes (presenilin 1 and 2 and amyloid precursor protein) are associated with presenile AD. Presenilin 1 gene mutations have not been described in African Americans. We studied an African American family with autosomal dominant rapidly progressive dementia and psychosis occurring early in the fifth decade of life. We performed neurologic evaluations, psychometrics, and neuroimaging. We sequenced the amyloid precursor protein gene, presenilin 1 and 2, and tau in affected and unaffected family members. One patient underwent a brain biopsy and subsequent autopsy. Personality change, auditory and visual hallucinations, delusions, memory impairment, word-finding difficulties, and subsequent rigidity, dystonia, myoclonus, and mutism developed in 2 brothers. Neuropsychometric testing in one was consistent with frontotemporal dementia or atypical AD. Neuroimaging studies showed diffuse cortical involvement. A clinical diagnosis of familial non-Alzheimer dementia was made. However, results of temporal lobe biopsy in one revealed amyloid neuritic plaques, and autopsy results confirmed the diagnosis of AD. Gene sequencing revealed a presenilin 1 point mutation (M139V) cosegregating with the disease. A tau polymorphism in exon 7 (A178T) was found in an affected brother and unaffected relatives. We report the first documented presenilin mutation in African American patients presenting with early personality change, psychosis, and memory loss with preserved praxis. The M139V mutation can present differently between kindreds, with some features suggestive of a frontal lobe syndrome. The M139V mutation can lead to atypical AD, and genetic background may have a role in determining the phenotype of genetically defined AD."}, {"id": "pubmed23n1106_14554", "title": "Hereditary cerebral amyloid angiopathy mimicking CADASIL syndrome.", "score": 0.014553770986017985, "content": "Small vessel disease (SVD), and most specifically hereditary forms like CADASIL and cerebral amyloid angiopathy (hCAA), are conditions of increasing clinical importance. We report a rare case of hCAA in a Greek family that presented with a CADASIL clinical and neuroimaging phenotype. A 65-year-old man was admitted with recurrent transient episodes of right leg numbness. The patient's medical history started at the age of 50 years with depression and behavioral disorders. His family history was positive for stroke (father), dementia (father and brother), migraine (daughter) and depression (father and daughter). Neurological examination disclosed anomic aphasia with severely impaired cognitive status, and brisk reflexes. Brain computed tomography and magnetic resonance imaging showed CADASIL-like leukoencephalopathy (hyperintense lesions in bilateral temporopolar area, external capsule, thalami, centrum semiovale and superior frontal regions) with occipital calcifications and cerebral microbleeds. Screen for variants in NOTCH3 gene was negative. Exome sequencing revealed a novel pathogenic mutation for hCAA. We report a novel amyloid precursor protein mutation which results in a CADASIL-like clinical phenotype (progressive cognitive and motor decline, stroke, migraine and behavioral disorders) and CADASIL-leukoencephalopathy coupled with occipital calcifications. Earlier recognition and swift hCAA diagnosis may prompt rational preventive and potential disease-modifying interventions."}, {"id": "pubmed23n0489_6062", "title": "When sporadic disease is not sporadic: the potential for genetic etiology.", "score": 0.013165021039036787, "content": "Approximately 2% of Alzheimer disease cases and 10% to 15% of prion disease cases are due to mutations in autosomal dominant genes. Mutations have been found in patients without family histories of neurological disease. To emphasize the need for consideration of a genetic etiology of prion disease and early-onset Alzheimer disease, regardless of the absence of a significant family history, as well as the need for pretest genetic counseling of all patients or their families. Three case reports. Patient 1, a 53-year-old man with possible Creutzfeldt-Jakob disease, was enrolled in a research study that included sequencing of the prion protein gene. Although there was no family history of neurological disease, an E200K mutation was found. This unexpected result caused the family significant distress. Patient 2, a 55-year-old woman with biopsy-proven Creutzfeldt-Jakob disease, participated in a prion disease research study. Her family was counseled about the possibility of hereditary Creutzfeldt-Jakob disease, despite the lack of family history. After assessing the ramifications, the family decided not to learn about the patient's genetic test results. Patient 3 was a 54-year-old man with a 6-year history of memory loss. A diagnosis of probable Alzheimer disease was given, and the patient and his family were counseled on the availability of presenilin 1 testing, although there was no known family history of dementia. The family agreed to testing, and a presenilin 1 mutation was identified. Certain neurodegenerative diseases may have a genetic etiology, despite the lack of a positive family history. Revealing a newly discovered hereditary cause of Creutzfeldt-Jakob disease or Alzheimer disease can have a profound effect on families. Pretest counseling on genetic issues is essential to better prepare families and to allow them to make an informed choice about learning genetic test results."}, {"id": "pubmed23n0685_22406", "title": "Pre-dementia clinical stages in presenilin 1 E280A familial early-onset Alzheimer's disease: a retrospective cohort study.", "score": 0.013043658870934241, "content": "Mild cognitive impairment (MCI) and pre-MCI have been proposed as stages preceding Alzheimer's disease (AD) dementia. We assessed descendants of individuals with a mutation in presenilin 1 (PSEN1) that causes familial AD, with the aim of identifying distinct stages of clinical progression to AD dementia. We retrospectively studied a cohort of descendants of carriers of the PSEN1 E280A mutation. Pre-dementia cognitive impairment was defined by a score 2 SD away from normal values in objective cognitive tests, and was subdivided as follows: asymptomatic pre-MCI was defined by an absence of memory complaints and no effect on activities of daily living; symptomatic pre-MCI was defined by a score on the subjective memory complaints checklist higher than the mean and no effect on activities of daily living; and MCI was defined by a score on the subjective memory complaints checklist higher than the mean, with no effect on basic activities of daily living and little or no effect on complex daily activities. Dementia was defined according to the diagnostic and statistical manual of mental disorders, fourth edition. Reference mean scores were those of participants who did not carry the PSEN1 E280A mutation. We used the Turnbull survival analysis method to identify ages at onset of each stage of the disease. We measured the time from birth until onset of the three pre-dementia stages, dementia, and death, and assessed decline in cognitive domains for each stage. Follow-up was from Jan 1, 1995, to Jan 27, 2010. 1784 patients were initially identified, 449 of whom were PSEN1 E280A carriers who had complete clinical follow-up. Median age at onset was 35 years (95% CI 30-36) for asymptomatic pre-MCI, 38 years (37-40) for symptomatic pre-MCI, 44 years (43-45) for MCI, and 49 years (49-50) for dementia. The median age at death was 59 years (95% CI 58-61). The median time of progression from asymptomatic to symptomatic pre-MCI was 4 years (95% CI 2-8), from symptomatic pre-MCI to MCI was 6 years (4-7), from MCI to dementia was 5 years (4-6), and from dementia to death was 10 years (9-12). The cognitive profile was predominantly amnestic and was associated with multiple domains. Affected domains showed variability in initial stages, with some transient recovery in symptomatic pre-MCI followed by continuous decline. Clinical deterioration can be detected as measurable cognitive impairment around two decades before dementia onset in PSEN1 E280A carriers. Onset and progression of pre-dementia stages should be considered in the investigation and use of therapeutic interventions for familial AD. Departamento Administrativo de Ciencia, Tecnología e Innovación, COLCIENCIAS, Republic of Colombia."}, {"id": "pubmed23n0357_14932", "title": "Familial British dementia with amyloid angiopathy: early clinical, neuropsychological and imaging findings.", "score": 0.01261574074074074, "content": "Familial British dementia with amyloid angiopathy (FBD) is an autosomal dominant condition characterized by a dementia, progressive spastic tetraparesis and cerebellar ataxia with onset in the sixth decade. A point mutation in the BRI gene has been shown to be the genetic abnormality. Genealogical work with the large family originally reported by Worster-Drought and updated by Plant has identified nine generations dating back to the late eighteenth century. The pedigree now includes six living affected patients, 35 historical cases, and 52 descendants at risk of having inherited the disease. A common ancestor has been identified between the large pedigree and a case report of 'familial cerebellar ataxia with amyloid angiopathy'. An autopsy case from a separate family with an identical condition is described but no common ancestor with the large pedigree has been found. Case histories have been researched and updated in each pedigree. Eleven individuals at risk of FBD, aged between 44 and 56 years, agreed to undergo a clinical and neuropsychological assessment along with MRI brain imaging in order to clarify early diagnostic features. Five of the eleven were thought to show early clinical signs of the disease. Neurological examination was abnormal in three, with limb and gait ataxia and mild spastic paraparesis. Three had impaired recognition and recall memory and another had mild impairment of delayed visual recall. All affected individuals had an abnormal MRI of the brain, consisting of deep white-matter hyperintensity (T(2)-weighted scans) and lacunar infarcts, but no intracerebral haemorrhage. The corpus callosum was affected particularly, and in one patient it was severely atrophic."}, {"id": "pubmed23n0552_21345", "title": "Enhanced brain activity may precede the diagnosis of Alzheimer's disease by 30 years.", "score": 0.01242772396185327, "content": "Presenilin 1 (PSEN1) mutations cause autosomal dominant familial Alzheimer's disease (FAD). PSEN1 mutation carriers undergo the course of cognitive deterioration, which is typical for sporadic Alzheimer's disease but disease onset is earlier and disease progression is faster. Here, we sought to detect signs of FAD in presymptomatic carriers of the PSEN1 mutation (C410Y) by use of a neuropsychological examination, functional MRI during learning and memory tasks and MRI volumetry. We examined five non-demented members of a FAD family and 21 non-related controls. Two of the five family members were carrying the mutation; one was 20 years old and the other 45 years old. The age of clinical manifestation of FAD in the family studied here is approximately 48 years. Neuropsychological assessments suggested subtle problems with episodic memory in the 20-year-old mutation carrier. The middle-aged mutation carrier fulfilled criteria for amnestic mild cognitive impairment. The 20-year-old mutation carrier exhibited increased, while the middle-aged mutation carrier exhibited decreased brain activity compared to controls within memory-related neural networks during episodic learning and retrieval, but not during a working-memory task. The increased memory-related brain activity in the young mutation carrier might reflect a compensatory effort to overcome preclinical neural dysfunction caused by first pathological changes. The activity reductions in the middle-aged mutation carrier might reflect gross neural dysfunction in a more advanced stage of neuropathology. These data suggest that functional neuroimaging along with tasks that challenge specifically those brain areas which are initial targets of Alzheimer's disease pathology may reveal activity alterations on a single-subject level decades before the clinical manifestation of Alzheimer's disease."}, {"id": "wiki20220301en231_17468", "title": "Early-onset Alzheimer's disease", "score": 0.012389139036703507, "content": "Causes Familial AD is inherited in an autosomal dominant fashion, identified by genetics and other characteristics such as the age of onset. Genetics Familial Alzheimer disease is caused by a mutation in one of at least three genes, which code for presenilin 1, presenilin 2, and APP. PSEN1 – Presenilin 1 The presenilin 1 gene (PSEN1 located on chromosome 14) was identified by Sherrington (1995) and multiple mutations have been identified. Mutations in this gene cause familial Alzheimer's type 3 with certainty and usually under 50 years old. This type accounts for 30-70% of EOFAD. This protein has been identified as part of the enzymatic complex that cleaves amyloid beta peptide from APP."}, {"id": "article-19177_9", "title": "Cerebral Autosomal Dominant Arteriopathy -- Evaluation", "score": 0.012203897187922747, "content": "CADASIL should be suspected in patients with a strong family history of early strokes and dementia, keeping in mind that CADASIL is likely under-diagnosed. Though definitive diagnosis is via genetic testing or skin biopsy, a CADASIL scale has been proposed by Pescini et al. as a less expensive initial clinical screening measure. [4] This scale includes typical symptoms of the disease such as migraines and TIA, typical imaging features, and family history, all with various weightings. In patients where CADASIL is strongly suspected, definitive diagnosis begins with serum genetic testing for a NOTCH3 mutation. Approximately 4% of patients with CADASIL will have a negative genetic test, likely related to unidentified genetic mutations. Thus, for those patients with a high clinical suspicion, the next step is a skin biopsy with histopathologic examination for GOM accumulation. [5]"}, {"id": "pubmed23n0799_23918", "title": "Impairments in Episodic-Autobiographical Memory and Emotional and Social Information Processing in CADASIL during Mid-Adulthood.", "score": 0.011715538503699644, "content": "Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) - is the most common genetic source of vascular dementia in adults, being caused by a mutation in NOTCH3 gene. Spontaneous de novo mutations may occur, but their frequency is largely unknown. Ischemic strokes and cognitive impairments are the most frequent manifestations, but seizures affect up to 10% of the patients. Herein, we describe a 47-year-old male scholar with a genetically confirmed diagnosis of CADASIL (Arg133Cys mutation in the NOTCH3 gene) and a seemingly negative family history of CADASIL illness, who was investigated with a comprehensive neuropsychological testing battery and neuroimaging methods. The patient demonstrated on one hand severe and accelerated deteriorations in multiple cognitive domains such as concentration, long-term memory (including the episodic-autobiographical memory domain), problem solving, cognitive flexibility and planning, affect recognition, discrimination and matching, and social cognition (theory of mind). Some of these impairments were even captured by abbreviated instruments for investigating suspicion of dementia. On the other hand the patient still possessed high crystallized (verbal) intelligence and a capacity to put forth a façade of well-preserved intellectual functioning. Although no definite conclusions can be drawn from a single case study, our findings point to the presence of additional cognitive changes in CADASIL in middle adulthood, in particular to impairments in the episodic-autobiographical memory domain and social information processing (e.g., social cognition). Whether these identified impairments are related to the patient's specific phenotype or to an ascertainment bias (e.g., a paucity of studies investigating these cognitive functions) requires elucidation by larger scale research. "}, {"id": "pubmed23n0684_18091", "title": "Phenotypic heterogeneity of the GRN Asp22fs mutation in a large Italian kindred.", "score": 0.01128273582506992, "content": "The Asp22fs(g.63_64insC) mutation in progranulin gene (GRN) has been so far reported in one patient who developed frontotemporal dementia (FTD) at the age of 65. Here, we describe the clinical heterogeneity associated with the GRN Asp22fs mutation in a large Italian family. Clinical and instrumental workup of two symptomatic carriers in two generations has been carried out, together with genetic analysis of probands and of nine asymptomatic family members. The first proband was a 47-year old male clinically diagnosed with FTD. Family history was positive and suggestive of an autosomal dominant pattern of inheritance. Evaluation of plasma GRN levels was consistent with the presence of a mutation in its encoding gene, that was demonstrated by sequencing [Asp22fs(g.63_64insC)]. Brain MRI showed multiple T2 and FLAIR hyperintense areas in the frontal lobe white matter and right hemisphere cortical atrophy. The second proband was his 79 year old uncle, presenting with mild cognitive impairment. Brain MRI showed small T2 hyperintense lesions and widespread cortical atrophy. Cerebrospinal fluid amyloid-β, tau, and phosphotau protein levels were in both cases in the range of normality. Additional nine asymptomatic family members were studied. This family's description expands the spectrum of clinical presentations of frontotemporal lobar degeneration caused by GRN mutations, suggesting that the diagnosis could be missed in some individuals with an atypical presentation, and points up the importance of GRN plasma level evaluation."}, {"id": "wiki20220301en318_25498", "title": "Variably protease-sensitive prionopathy", "score": 0.011142663127353541, "content": "Diagnosis is difficult, as pathognomonic signs on MRI such as cortical ribboning or hockey stick sign, periodic sharp wave complexes on EEG, and tests for 14-3-3 protein and tau protein are usually not helpful, and no mutations have been observed in the coding region of the PrP gene, unlike CJD and Variant CJD. The diagnosis can be made on pathological examination. There are unique microscopic and immunohistochemical features, and the prions cannot be digested using proteases. Because 8 out of 10 patients had a positive family history of dementia in the original study, a genetic cause was suspected. Some have suggested the disease may be a sporadic form of Gerstmann–Sträussler–Scheinker syndrome (GSS)."}, {"id": "pubmed23n0421_18899", "title": "[Autosomal dominant Alzheimer's disease. Study of a Moroccan family].", "score": 0.011020589542290031, "content": "The authors describe in this paper a Moroccan family presenting Alzheimer's disease with early onset and rapid course (6 members, of whom 3 died at 34 and 40 years old in a severe picture of dementia). Among these 6 members, Mr M.K., 36 years old, was admitted in the University Psychiatric Department for 4 years of depressive syndrome, -memory impairment and cognitive deficit. In the literature, the cases of Alzheimer's disease begining before 60 years have been reported since 1991; the transmission of this syndrome is autosomal dominant. The genetic studies showed a multifactorial determinism. For the familial cases with early onset, the mutation occurs on the amyloïd precursor protein and on the presenilin 1 and 2. In this case, the result of the familial investigations was compatible with Alzheimer's disease, a dominant autosomic disorder, with early onset between 32 and 40 years old. The clinical course evoked a mutation of presenilin 1. The identification of such mutation in one of his sisters living in France confirmed the genetic transmission. Despite progress made in understanding the pathogenesis, the development of a curative treatment in Alzheimer's disease remains difficult. Selective inhibitors of cholinesterase can improve patients with mild to moderate Alzheimer's disease forms. In Morocco, only donepezil is available, but it is inaccessible for patients who need this treatment because of its high price. For Mr M.K., who still has a professional activity, symptomatic treatment, cognitive and psychological supports may allow him to maintain an adequate life for years. The family support is essential."}, {"id": "pubmed23n0689_10353", "title": "First report of a pathogenic mutation on exon 24 of the NOTCH3 gene in a CADASIL family.", "score": 0.010457516339869282, "content": "Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetically transmitted small vessel disease clinically characterized by migraine, recurrent subcortical strokes, and cognitive and mood disorders. Pathogenic mutations are located on any of the exons of the NOTCH3 gene coding for epidermal-growth factor (EGF)-like repeats of the extracellular domain of the NOTCH3 receptor. Because the gene is large and the mutations cluster on some exons, many laboratories restrict the analysis to these exons. We report the first missense mutation involving exon 24 and causing CADASIL in a 64-year-old man. The patient was admitted to the hospital for a loss of consciousness accompanied by profuse sweating. On examination, some parkinsonian features were present. Over the last 4 years, he had developed postural instability and gait disturbances with repeated falls, behavioral disorders, and cognitive impairment. A diagnostic hypothesis of atypical parkinsonism had been advanced. The presence of multiple subcortical lacunar infarcts and leukoencephalopathy extended to the external capsule on cerebral MRI suggested the presence of CADASIL. The diagnosis was confirmed by finding a heterozygous mutation leading to a cysteine substitution on exon 24 of the NOTCH3 gene. One proband's brother, who had progressive gait disturbances, unilateral action tremor and bradykinesia, and an asymptomatic niece also resulted affected. This report underlines that when CADASIL is suspected the genetic analysis should be performed on all the NOTCH3 exons coding for EGF-like repeats including exon 24 and confirms that CADASIL may have heterogeneous phenotypes."}, {"id": "wiki20220301en395_2883", "title": "Rudolph E. Tanzi", "score": 0.010007378712414684, "content": "In 1995, Dr. Tanzi collaborated with Dr. Peter Hyslop and Dr. Jerry Schellenberg to discover the two other EO-FAD genes, presenilin 1 and 2 (PSEN1 and PSEN2). He has published many key studies characterizing the role of the EO-FAD genes in health and disease. All three genes remain among the most highly studied drug targets in the field of AD, especially with regard to therapeutic strategies aimed at reducing beta-amyloid deposition. In 1993, Dr. Tanzi also first discovered the gene for the neurodegenerative disease, Wilson’s disease, published in Nature Genetics. In that same year, he contributed significantly to the discovery of the first familial amyotrophic lateral sclerosis (ALS) gene, SOD1, by providing the key genetic and physical mapping data for chromosome 21 used to find the gene defect, published in Nature, 1993."}, {"id": "wiki20220301en318_24059", "title": "Alzheimer's Disease Neuroimaging Initiative", "score": 0.009900990099009901, "content": "ADNI uses a variety of techniques to study its participants. After obtaining informed consent, participants undergo a series of initial tests that are repeated at intervals over subsequent years (Table 2): a clinical evaluation to assess overall health and relevant history such as education neuropsychological tests to assess aspects of brain function affected by AD such as memory, executive function, and the ability to perform activities of daily living. genetic testing for the major AD genetic risk factor, APOE ε4, the gene for a form of apolipoprotein E, and other studies lumbar puncture to collect cerebrospinal fluid (CSF) which is tested for the AD biomarkers β-amyloid, the main component of amyloid plaques, and tau protein, which forms Alzheimer's brain tau tangles. Magnetic resonance imaging (MRI) scans to assess brain structure, connectivity, and the extent of white matter disease"}, {"id": "pubmed23n0339_98", "title": "CADASIL: a review with proposed diagnostic criteria.", "score": 0.009900990099009901, "content": "Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) can be considered as a new disease predominantly affecting the small vessels of the brain with an autosomal dominant transmission linked to chromosome 19. This review includes an historical perspective showing how the disease was identified from the spectrum of vascular leukoencephalopathies. More than two hundred patients have now been described, belonging to at least 30 unrelated pedigrees in Europe, America and Asia. The clinical features include four major neurological presentations associated in variable degrees during the course of the disease: migraine with or without aura, strokes or stroke-like episodes, major psychiatric symptoms and dementia. The patients are free of the classical vascular risk factors. The disease has a progressive or stepwise course with age at onset in the forties and a mean duration of 13.6 +/- 10.7 years. Death occurs in the fifties in a characteristic condition associating a pseudo-bulbar syndrome and subcortical dementia. Cerebral magnetic resonance imaging (MRI) is highly contributive to the diagnosis, showing a diffuse leukoencephalopathy with subcortical infarcts in the basal ganglia and white matter. Pathological data show macroscopic lesions similar to Binswanger's disease but different lesions of the small vessels including thickening of the media, characteristic PAS+ granular material and narrowing of the lumen. Skin biopsy may be a valuable diagnostic tool, showing ultrastructural alterations of skin vessels similar to those of brain vessels. The disease is highly homogeneous on a genetic basis and the identification of the gene Notch 3 on chromosome 19 has opened new avenues for research and genetic counselling. The pathogenesis of the disease has still to be elucidated. A definite diagnosis relies on genetical or pathological data. Diagnostic criteria are proposed to recognize the disease on clinical and imaging parameters. So far, no treatment has been reported to be successful for CADASIL. Copyright Lippincott-Raven Publishers"}, {"id": "pubmed23n0741_19695", "title": "Brain imaging and fluid biomarker analysis in young adults at genetic risk for autosomal dominant Alzheimer's disease in the presenilin 1 E280A kindred: a case-control study.", "score": 0.00980392156862745, "content": "We have previously characterised functional brain abnormalities in young adults at genetic risk for late-onset Alzheimer's disease. To gain further knowledge on the preclinical phase of Alzheimer's disease, we sought to characterise structural and functional MRI, CSF, and plasma biomarkers in a cohort of young adults carrying a high-penetrance autosomal dominant mutation that causes early-onset Alzheimer's disease. Between January and August, 2010, 18-26-year-old presenilin 1 (PSEN1) E280A mutation carriers and non-carriers from the Colombian Alzheimer's Prevention Initiative Registry in Medellín Antioquia, Colombia, had structural MRI, functional MRI during associative memory encoding and novel viewing and control tasks, and cognitive assessments. Consenting participants also had lumbar punctures and venepunctures. Outcome measures were task-dependent hippocampal or parahippocampal activations and precuneus or posterior cingulate deactivations, regional grey matter reductions, CSF Aβ(1-42), total tau and phospho-tau(181) concentrations, and plasma Aβ(1-42) concentrations and Aβ(1-42):Aβ(1-40) ratios. Structural and functional MRI data were compared using automated brain mapping algorithms and search regions related to Alzheimer's disease. Cognitive and fluid biomarkers were compared using Mann-Whitney tests. 44 participants were included: 20 PSEN1 E280A mutation carriers and 24 non-carriers. The carrier and non-carrier groups did not differ significantly in their dementia ratings, neuropsychological test scores, or proportion of apolipoprotein E (APOE) ɛ4 carriers. Compared with non-carriers, carriers had greater right hippocampal and parahippocampal activation (p=0·001 and p<0·014, respectively, after correction for multiple comparisons), less precuneus and posterior cingulate deactivation (all p<0·010 after correction), and less grey matter in several parietal regions (all p<0·002 uncorrected and corrected p=0·009 in the right parietal search region). In the 20 participants (ten PSEN1 E280A mutation carriers and ten non-carriers) who had lumbar punctures and venepunctures, mutation carriers had higher CSF Aβ(1-42) concentrations (p=0·008) and plasma Aβ(1-42) concentrations (p=0·01) than non-carriers. Young adults at genetic risk for autosomal dominant Alzheimer's disease have functional and structural MRI findings and CSF and plasma biomarker findings consistent with Aβ(1-42) overproduction. Although the extent to which the underlying brain changes are either neurodegenerative or developmental remain to be determined, this study shows the earliest known biomarker changes in cognitively normal people at genetic risk for autosomal dominant Alzheimer's disease. Banner Alzheimer's Foundation, Nomis Foundation, Anonymous Foundation, Forget Me Not Initiative, Boston University Department of Psychology, Colciencias, National Institute on Aging, National Institute of Neurological Disorders and Stroke, and the State of Arizona."}, {"id": "pubmed23n0317_1639", "title": "A 50-year perspective of a family with chromosome-14-linked Alzheimer's disease.", "score": 0.00980392156862745, "content": "A Swedish family with two generations suffering from presenile dementia with an unusually severe Alzheimer encephalopathy was first reported in 1946. The hypothesis that the disease was inherited through a dominant gene is strongly supported by the follow-up 50 years later of three additional generations and molecular genetic findings of a novel presenilin-1 gene mutation in the family. The pedigree contains six cases with well-documented dementia in four consecutive generations. The Alzheimer encephalopathy was unusually severe in the three cases studied post-mortem, with a pronounced involvement of the central grey structures, such as the claustrum, the nuclei around the third ventricle, the central thalamic nuclei and the brain stem. There were no vascular lesions and little amyloid angiopathy. All six affected cases showed the typical temporoparietal symptom pattern and other core symptoms of Alzheimer's disease, such as logoclonia, myoclonic twitchings and major motor seizures. Other predominant features were psychomotor slowness, increased muscular tension, a stiff stooped gait and a rapid loss of weight. The symptom pattern is convincingly explained by the consistent and severe involvement of cortical and central grey structures and is probably linked to the presenilin-1 gene mutation."}, {"id": "wiki20220301en471_17533", "title": "Epigenetics of neurodegenerative diseases", "score": 0.009708737864077669, "content": "Neurodegenerative diseases of the central nervous system Alzheimer’s Disease (AD) Alzheimer’s disease (AD) is the most prevalent form of dementia among the elderly. The disease is characterized behaviorally by chronic and progressive decline in cognitive function, beginning with short term memory loss, and neurologically by buildup of misfolded tau protein and associated neurofibrillary tangles, and by amyloid-beta senile plaques amyloid-beta senile plaques. Several genetic factors have been identified as contributing to AD, including mutations to the amyloid precursor protein (APP) and presenilins 1 and 2 genes, and familial inheritance of apolipoprotein E allele epsilon 4. In addition to these common factors, there are a number of other genes that have shown altered expression in Alzheimer's disease, some of which are associated with epigenetic factors. Epigenetic factors"}, {"id": "pubmed23n0681_20940", "title": "PICOGEN: five years experience with a genetic counselling program for dementia.", "score": 0.009708737864077669, "content": "We describe the 5 year experience of a genetic counselling program for familial dementias (the PICOGEN program). The neurologist selected the candidates for genetic testing in the screening visit based on family history and phenotype (Alzheimer disease-AD, frontotemporal lobar degeneration-FTLD, or prion disease). Asymptomatic subjects who decided to know their genetic status were evaluated within a structured protocol by the psychiatrist and psychologist prior to entering the program and followed up afterwards. A total of 87 patients from 72 families were candidates for the genetic study, 20 of the 72 families had a family history of autosomal dominant early-onset dementia (ADEOD). A pathogenic mutation was found in 22 patients (8 PSEN1, 1 PSEN2, 1 APP, 4 MAPT, 8 PRNP), 5 of which had not been previously described. All positive cases, except for 1 PSEN1 (12.5%) and 4 PRNP (50%) showed ADEOD. In 3 ADEOD cases (15%) no pathogenic mutation was found. After individual genetic counselling, 24/54 asymptomatic subjects at risk decided to have the pre-symptomatic study, of whom 10 (42%) were carriers of the pathogenic mutation. In the follow up, no major psychiatric complication was observed. In our series, family history of ADEOD was a sensitive criterion for the detection of pathogenic mutations in AD and FTLD but not in prion diseases. No genetic anomalies were detected in 15% of the ADEOD cases using conventional diagnostic procedures, and 43% of pre-symptomatic subjects at risk who received individual genetic counselling decided to have the study. The pre-symptomatic diagnosis proved to be safe under these conditions."}, {"id": "pubmed23n0350_12344", "title": "A follow-up study of the family with the Swedish APP 670/671 Alzheimer's disease mutation.", "score": 0.009615384615384616, "content": "To study the progression of Alzheimer's disease (AD) at a very early stage and to evaluate clinical markers of presymptomatic AD. Longitudinal study at a university hospital. A Swedish family harboring a double mutation at codons 670/671 of the APP gene on chromosome 21 was followed longitudinally for 3 years. Both mutation carriers and noncarriers participated. Results from clinical investigations, electroencephalography, neuropsychological and neuroradiological examinations including magnetic resonance imaging, single-photon emission computed tomography and positron emission tomography were assessed and compared on two or more occasions. During follow-up, 1 initially asymptomatic mutation carrier who was near the expected age of onset for this family, developed cognitive symptoms, and at the end of the follow-up fulfilled the diagnostic criteria for AD. One mutation carrier with cognitive symptoms at the first examination showed clinical deterioration and was diagnosed with AD. One demented mutation carrier died and was shown to have typical AD neuropathology at autopsy. The two remaining asymptomatic mutation carriers, as well as all the noncarriers were asymptomatic. These mutation carriers who were near the expected age of onset of AD but without clinical signs of the disease, did not show changes in either electrophysiological parameters or volumes of the temporal lobes. However, in these 2 individuals the blood flow in the temporal lobe showed intermediate values between the symptomatic mutation carriers and healthy noncarriers. Two neuropsychological tests showed a deterioration that paralleled clinical symptoms in 1 of the mutation carriers who was close to the expected age of onset and who at the end of the follow-up had clinical signs of AD. In the same subject, brain glucose metabolism was pathologically reduced in the temporal lobes before other clinical symptoms were obvious. In this familial form of AD a reduced temporal lobe glucose metabolism was indicative of AD before the expected clinical onset. Reduced glucose metabolism even preceded the development of subjective or objective cognitive dysfunction and might therefore serve as a clinical marker for AD before the onset of clinical symptoms. Reduced cerebral blood flow in the temporal lobes and cognitive deterioration paralleled the clinical decline in the early stage of the disease. Copyrightz1999S.KargerAG,Basel"}, {"id": "pubmed23n0727_13114", "title": "[Auditory hallucination as an initial sign of frontotemporal dementia].", "score": 0.009523809523809525, "content": "A 58-year-old right-handed man presented with a 9-year history of stereotyped behaviors and auditory hallucinations. At 49 years of age, he began to complain about auditory hallucinations that said offensive things about him, and around the same time, he began exhibiting stereotyped behaviors. For example, he traveled the same long route from his office to home every evening. Disinhibitory behavior occurred at 53 years of age, and he was forced to retire at 54 years of age. After retirement, the patient stayed in bed or showed a stereotyped behavior of repeatedly going to a nearby shrine every day. The complaint of auditory hallucinations disappeared around this time. At 57 years of age, he began using a day service, and soon after, several stereotyped behaviors appeared in this setting as well. Brain magnetic resonance imaging at 59 years of age showed severe atrophy and knife-blade-like appearance in the bilateral temporal poles. A clinical diagnosis of frontotemporal dementia (FTD) was made based on the neurobehavioral, neuropsychological, and neuroimaging findings. FTD patients have been reported to show hallucinations very rarely. However, recent studies have reported that frontotemporal lobar degeneration (FTLD) patients with ubiquitin-positive and transactive response-DNA-binding protein-43 (TDP-43)-positive pathologies (FTLD-U-TDP) and FTD patients with progranulin gene mutations often develop hallucinations. The current patient may belong to this group of patients with similar neuropathological and molecular biological bases."}, {"id": "pubmed23n0608_15456", "title": "[Frontotemporal dementia: a review].", "score": 0.009433962264150943, "content": "Frontotemporal dementia (FTD) is a neurological disorder characterised by the progressive degeneration of the frontal and anterior temporal cortex. FTD, as well as nonfluent progressive aphasia and semantic dementia, belongs to the more generic entity of frontotemporal lobe degeneration. Considering the involvement of the frontal lobe, the initial clinical presentation of FTD may be psychiatric, such as changes in personality or behavioural disorders. Psychiatrists, therefore, have to establish the differential diagnosis with late-onset schizophrenia or affective disorders. An accurate history of the onset of symptoms, thanks to the patient and especially to his/her family, is essential to recognize this dementia. In addition to behavioural changes, memory impairment, and speech disturbances are often present from the beginning. Consensus criteria have been proposed in 1998 that help to bring this diagnosis to mind in clinical practice. The progressive occurrence of personality changes or inappropriate social conducts in the fifth or sixth decade must prompt cognitive evaluation. NEUROCOGNITIVE AND BRAIN IMAGING DATA: A brief cognitive evaluation, such as the frontal assessment battery (FAB) may help to identify a dysexecutive syndrome and to prompt a thorough neuropsychological evaluation. The pattern of neuropsychological impairment reflects the involvement of the frontal lobe and appears different from that of other degenerative diseases, such as Alzheimer's dementia, which involves hippocampal damage. Additional investigations should however be made to detect a potentially curable dementia. Cerebral imaging is essential to the differential diagnosis and also shows evidence for the positive diagnosis of FTD. Structural MRI may initially not show the bilateral atrophy of the frontal lobe, but functional imaging may be helpful in the early stages of the illness by showing evidence of abnormalities in the anterior cerebral hemisphere. PATHOPHYSIOLOGICAL FINDINGS: In recent years, significant advances in the understanding of the pathological characteristics of FTD were made with genetic contribution, especially the discovery of the tau protein involvement. In fact, neuropathological examination with immunohistochemical analysis defines Pick's disease with Pick bodies that belong to tauopathies. Ubiquitinated intraneuronal inclusions may also be found, and some types of FTD have no distinctive pathological feature. However, although a definite diagnosis would only be established after postmortem pathological examination, the clinical, neuropsychological and imaging data enable the early identification of patients with FTD and, subsequently, the appropriate management. Although the prevalence of FTD reaches 1 Alzheimer's disease (AD) to 1.6 FTD in the general population between 45- and 64-year old, only few studies have focused on the treatment of FTD. Some evidence supports the positive effect of serotonergic agents, especially with regard to behavioural symptoms. Selective serotonin reuptake inhibitors or trazodone should therefore be prescribed in preference to acetylcholinesterase medications as in AD. However, no drug yet has the ability to stop or slow down the degenerative process. The management of daily life also bears specificities related to the younger age of these patients and to their behavioural disorders. Caregivers should receive some education about the characteristics of this dementia and should be helped in social management. As concerns aggressive behaviour, neuroleptics should generally be avoided because of poor tolerance. Finally, the outcome is characterized by a rapid loss of autonomy and sometimes by a premature institutionalisation."}, {"id": "pubmed23n0981_7638", "title": "Diagnostic Approach of Early-Onset Dementia with Negative Family History: Implications from Two Cases of Early-Onset Alzheimer's Disease with De Novo PSEN1 Mutation.", "score": 0.009433962264150943, "content": "For early-onset Alzheimer's disease (EOAD) cases with unclear family history, most cases are sporadic. Some cases are positive in genetic findings, that is, either incomplete penetrance or de novo mutation. We aimed to focus on EOAD cases with de novo mutations. Case reports and literature review were performed. The implication for diagnostic approach of early-onset dementia with negative family history was developed. We reported two Chinese EOAD cases with de novo mutations. The genotype PSEN1 G206S appeared to correlate with the phenotype of EOAD with pure cognitive problems. The second case had a PSEN1 M233V mutation with an earlier age of onset of 25 with cognitive decline, parkinsonism, and epilepsy. Although EOAD due to de novo mutations is not common, it should be considered in patients with a phenotype of progressive cognitive decline and amyloid positivity on PET or CSF analysis."}, {"id": "pubmed23n0850_11556", "title": "[Skin Biopsy is a Useful Tool for the Diagnosis of Atypical CADASIL: A Case Report].", "score": 0.009345794392523364, "content": "A 57-year-old man developed migraine at the age of 25 years. Thereafter, he developed depression at the age of 50 years, and was admitted to a psychiatric hospital at the age of 54 years because of deteriorating depression. He returned to his work after receiving treatment for depression; however, he made mistakes several times in his work. He was referred to our hospital for further neurological evaluation. The results of the neurological examination performed on admission were unremarkable. His Mini Mental State Examination (MMSE) score was 24/30, and neuropsycological evaluations revealed executive dysfunction. There was no family history of dementia or cerebral infarction. Magnetic resonance fluid attenuated inversion recovery (MR FLAIR) image of the brain showed hyperintense lesions around the lateral ventricle without involvement in the temporal pole and external capsule. Despite a lack of family history of dementia and cerebral infarction and non-specific brain MRI findings, his history of headache and depression were suggestive of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Therefore, skin biopsy was performed; electron microscopy of the biopsied sample revealed granular osmiophilic material deposits. Genetic analysis of the NOTCH3 gene showed a missense mutation with substitution of R427C in exon 8, i.e., out of the hot-spot, exon 3, and 4. Thus, skin biopsy is a useful tool for diagnosing atypical CADASIL."}, {"id": "pubmed23n0045_8553", "title": "[Two kindreds with familial Alzheimer's disease--analysis of the APP717 mutation and the mutated genes for the prion protein].", "score": 0.009345794392523364, "content": "Numerous Caucasian familial Alzheimer's disease (FAD) pedigrees have been described in the literature, while only 21 Japanese FAD families have been reported to date. Here we report the clinical findings and the result of molecular genetic analysis of 4 patients from two FAD kindreds, OS-2 and OS-3. The proband in OS-2 family has developed loss of recent memory and place disorientation age at 43. A brain CT showed severe diffuse cortical atrophy. Her younger brother had dementia at 42 years and her mother and other 3 siblings had also dementia symptoms suspected to be Alzheimer's disease. The proband in OS-3 family showed declining recent memory at 49 years and developed dysphagia, gait disturbance and emotional incontinent with cerebral atrophy at 52 years. His father and elder brother demonstrated dementia signs at 60 and 54 years old, respectively. Recently it was reported that affected members from 2 Caucasian kindreds with FAD had missense mutation in exon 17 of the gene for amyloid precursor protein (APP). Patients from three different Japanese kindreds with FAD also showed the same mutation on the APP gene. Amino acid substitution (Val-Ile) at codon 717 by this mutation is responsible for FAD in at least some kindreds. We used genomic DNA from 4 affected members of 2 families to determine whether the disease in these families is associated with a APP717 mutation and the mutated codons, 102, 117, 129, 178 and 200, on the gene for protease-resistance prion protein (PrP) which cause transmissible dementia, Creutzfelt-Jacob disease (CJD) and Gerstmann-Strausler syndrome (GSS).(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "pubmed23n0715_25332", "title": "Behavioral genetics of neurodegenerative disorders.", "score": 0.009259259259259259, "content": "Alzheimer's disease (AD) is the most common cause of dementia in the elderly, and is typically characterized by memory loss. In addition, during the disease progression, most patients develop behavioural and psychiatric symptoms of dementia (BPSD). Frontotemporal Lobar Degeneration (FTLD) is the most frequent neurodegenerative disorder with a presenile onset. It is characterized mainly by behavioural disturbances, whereas memory is conserved. The two major neuropathologic hallmarks of AD are extracellular Amyloid beta (Ab) plaques and intracellular neurofibrillary tangles (NFTs). Conversely, in FTLD the deposition of tau has been observed in a number of cases, but in several brains there is no deposition of tau but instead a positivity for ubiquitin. In some families these diseases are inherited in an autosomal dominant fashion. Genes responsible for familial AD include the Amyloid Precursor Protein (b-APP), Presenilin 1 (PS1)and Presenilin 2 (PS2). The majority of mutations in these genes are often associated with a very early onset (40–50 years of age). Regarding FTLD, the first mutations described are located in the Microtubule Associated Protein Tau gene(MAPT). Tau is a component of microtubules, which represent the internal support structures for the transport of nutrients, vesicles, mitochondria and chromosomes within the cell. Mutations in MAPT are associated with an early onset of the disease (40–50 years), and the clinical phenotype is consistent with Frontotemporal Dementia (FTD). Recently, mutations in a second gene, named progranulin(GRN), have been identified in some families with FTLD. The pathology associated with these mutations is most frequently characterized by the immunostaining of TAR DNA Binding Protein 43 (TDP-43), which is a transcription factor. The clinical phenotype associated with GRN mutations is highly heterogeneous,including FTD, Progressive Aphasia, Corticobasal Syndrome, and AD. Age at disease onset is variable, ranging from 45 to 85 years of age. The majority of cases of AD and FTLD are however sporadic, and likely several genetic and environmental factors contribute to their development. Concerning AD, it is known that the presence of the e4 allele of the Apolipoprotein E gene is a susceptibility factor,increasing the risk of about 4 fold. A number of additional genetic factors,including cytokines, chemokines, Nitric Oxide Synthases, contribute to the susceptibility for the disease. Some of them also influence the risk to develop FTLD.Variability in serotonin transporter gene could influence the development of BPSD. In this chapter, current knowledge on molecular mechanisms at the basis of AD and FTLD, as well as the role of genetics, will be presented and discussed."}, {"id": "pubmed23n1140_17128", "title": "Evidence of beta amyloid independent small vessel disease in familial Alzheimer's disease.", "score": 0.009259259259259259, "content": "We studied small vessel disease (SVD) pathology in Familial Alzheimer's disease (FAD) subjects carrying the presenilin 1 (PSEN1) p.Glu280Ala mutation in comparison to those with sporadic Alzheimer's disease (SAD) as a positive control for Alzheimer's pathology and Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) bearing different NOTCH3 mutations, as positive controls for SVD pathology. Upon magnetic resonance imaging (MRI) in life, some FAD showed mild white matter hyperintensities and no further radiologic evidence of SVD. In post-mortem studies, total SVD pathology in cortical areas and basal ganglia was similar in PSEN1 FAD and CADASIL subjects, except for the feature of arteriosclerosis which was higher in CADASIL subjects than in PSEN1 FAD subjects. Further only a few SAD subjects showed a similar degree of SVD pathology as observed in CADASIL. Furthermore, we found significantly enlarged perivascular spaces in vessels devoid of cerebral amyloid angiopathy in FAD compared with SAD and CADASIL subjects. As expected, there was greater fibrinogen-positive perivascular reactivity in CADASIL but similar reactivity in PSEN1 FAD and SAD groups. Fibrinogen immunoreactivity correlated with onset age in the PSEN1 FAD cases, suggesting increased vascular permeability may contribute to cognitive decline. Additionally, we found reduced perivascular expression of PDGFRβ AQP4 in microvessels with enlarged PVS in PSEN1 FAD cases. We demonstrate that there is Aβ-independent SVD pathology in PSEN1 FAD, that was marginally lower than that in CADASIL subjects although not evident by MRI. These observations suggest presence of covert SVD even in PSEN1, contributing to disease progression. As is the case in SAD, these consequences may be preventable by early recognition and actively controlling vascular disease risk, even in familial forms of dementia."}, {"id": "pubmed23n0395_16097", "title": "[Amnesic presentations of the compulsive obsessional confusions (about 3 patients appearing in a consultation of memory)].", "score": 0.009174311926605505, "content": "Disorders or complaints of memory are a frequent cause of consultation in depression, major anxiety and psychiatry disease with personality disorders. We report 3 patients with obsessive compulsive disorder (OCD), without diagnosis and treatment, examined in a specialized memory consultation. They always had OCD with cognitive checking. Diagnosis of transient global amnesia and temporal complex seizure were discussed in 2 cases. Psychometric impairment only was observed in first free recall of a verbal memory task and was no specific. Behavioural during testing seemed to be very important to analyse. First, a 49-year-old man consulted because he had stereotyped transient amnesia lasted one minute, 2 or 3 times a week, since 6 months. He was a teacher. Transient amnesia always occurred during lessons. Suddenly he didn't know where he was or what he was speaking about. Episodes lasted one minute. After them, he had no confusion and no difficulty in concentration but intense anxiety. In an another hand, when he was in his car, after lessons, he could forget where he was during some minutes. CT scan and EEG were normal. Neuropsychological tests only objectived impairment in first free recall of Grober and Buschke's words. Patient explained that he could not prevent to check responses. He told us checking obsessive compulsive disorder during since long time ago. We discussed clear differences which existed between seizure and ruminations or preoccupations. Secondly, a 55-year-old woman was afraid of her memory performances. She was medical secretary and had no problem in her work but she would like a memory consultation to reassure herself. She was neither depressed nor anxious. She presented curious production in fluency task. She had to produce as many animals's names as possible: she could say 35 names which was an excellent performance but only in alphabetic order! Neuropsychological tests objectived impairment in her first free recall of Grober and Buschke's words. She tried in her first free recall to remember words in alphabetic order. She explained how she was bound to range everything in alphabetic order! She had a lot of rituals. She thought that she had an obsessive compulsive disorder but never consulted about this. The observation illustrated suspiscions about memory operations which could be observed in patients group with obsessive compulsive disorders. Finally, a 62-year-old man told us that he had presented a transient global amnesia during 4 hours. He had an important appointment and was upset about that. He didn't go to it and wandered in his flat. He always asked the same questions and forgot everything. He had no neurological deficit. He was anxious, sad and cried several times. He perfectly remembered the episod and thought that he had a panic attack! Verbal memory tests only objectived difficulties in his first free recall of Grober and Buschke words as the two others patients. He had a story of obsessive compulsive disorder with checking and rituals. In this observation, we discussed clear differences which existed between panic attacks and global transient amnesia. We analyzed patterns of neuropsychological performances which illustrated clinical features of obsessive compulsive disorder. These three patients impaired in their first free recall of verbal memory task. It is not a specific result. We observed during psychometric evaluation, strategic processing which impaired episodic memory: patients tried to check their performances. Memory complaints only were observed in checking obsessive compulsive disorder. It is a difficulty or a doubt about memory capacities. Difficulties could be due to particular cognitive processes who pertubate normal memory capacities."}]}}}} {"correct_option": 1, "explanations": {"1": {"exist": true, "char_ranges": [[0, 234]], "word_ranges": [[0, 38]], "text": "Answer 1 correct: The Thessaly test is a meniscal exploration maneuver. The statement focuses exclusively on this part of the examination and also explains that it is indeed positive, which leads to the suspicion of a meniscal lesion."}, "2": {"exist": true, "char_ranges": [[1289, 1454]], "word_ranges": [[218, 246]], "text": "Answer 2 incorrect: At no time do they describe any exploration maneuvers of the anterior cruciate ligament, nor do they mention any trauma that may have injured it."}, "3": {"exist": true, "char_ranges": [[1455, 1623]], "word_ranges": [[246, 274]], "text": "Answer 3 incorrect: At no time do they describe any exploration maneuvers of the posterior cruciate ligament, nor do they mention any trauma that could have injured it."}, "4": {"exist": true, "char_ranges": [[1624, 1761]], "word_ranges": [[274, 299]], "text": "Wrong answer 4: Although it could occur, it is not to be expected in a 41 year old person with an acutely established clinical condition."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Answer 1 correct: The Thessaly test is a meniscal exploration maneuver. The statement focuses exclusively on this part of the examination and also explains that it is indeed positive, which leads to the suspicion of a meniscal lesion. \"The Thessaly test is a dynamic reproduction of load transmission in the knee joint and is performed at 5° and 20° of flexion. The examiner supports the patient by holding his or her outstretched hands while the patient stands flatfooted on the floor. The patient then rotates his or her knee and body, internally and externally, three times, keeping the knee in slight flexion (5°). Then the same procedure is carried out with the knee flexed at 20°. Patients with suspected meniscal tears experience medial or lateral joint-line discomfort and may have a sense of locking or catching. The theory behind the test is that, with this maneuver, the knee with a meniscal tear is subjected to excessive loading conditions and almost certainly will have the same symptoms that the patient reported. The test is always performed first on the normal knee so that the patient may be trained, especially with regard to how to keep the knee in 5° and then in 20° of flexion and how to recognize, by comparison, a possible positive result in the symptomatic knee.\" Answer 2 incorrect: At no time do they describe any exploration maneuvers of the anterior cruciate ligament, nor do they mention any trauma that may have injured it. Answer 3 incorrect: At no time do they describe any exploration maneuvers of the posterior cruciate ligament, nor do they mention any trauma that could have injured it. Wrong answer 4: Although it could occur, it is not to be expected in a 41 year old person with an acutely established clinical condition.", "full_answer_no_ref": "[HIDDEN]: The Thessaly test is a meniscal exploration maneuver. The statement focuses exclusively on this part of the examination and also explains that it is indeed positive, which leads to the suspicion of a meniscal lesion. \"The Thessaly test is a dynamic reproduction of load transmission in the knee joint and is performed at 5° and 20° of flexion. The examiner supports the patient by holding his or her outstretched hands while the patient stands flatfooted on the floor. The patient then rotates his or her knee and body, internally and externally, three times, keeping the knee in slight flexion (5°). Then the same procedure is carried out with the knee flexed at 20°. Patients with suspected meniscal tears experience medial or lateral joint-line discomfort and may have a sense of locking or catching. The theory behind the test is that, with this maneuver, the knee with a meniscal tear is subjected to excessive loading conditions and almost certainly will have the same symptoms that the patient reported. The test is always performed first on the normal knee so that the patient may be trained, especially with regard to how to keep the knee in 5° and then in 20° of flexion and how to recognize, by comparison, a possible positive result in the symptomatic knee.\" [HIDDEN]: At no time do they describe any exploration maneuvers of the anterior cruciate ligament, nor do they mention any trauma that may have injured it. [HIDDEN]: At no time do they describe any exploration maneuvers of the posterior cruciate ligament, nor do they mention any trauma that could have injured it. [HIDDEN]: Although it could occur, it is not to be expected in a 41 year old person with an acutely established clinical condition.", "full_question": "A 41-year-old male consults for gonalgia of several days' evolution. During the examination, the Thessaly test is performed (pain with internal and external rotation movements with the knee flexed), which is positive. Which of the following lesions is more probable?", "id": 615, "lang": "en", "options": {"1": "Meniscal injury.", "2": "Injury due to rupture of the anterior cruciate ligament.", "3": "Injury due to rupture of the posterior cruciate ligament.", "4": "Injury due to degenerative arthropathy.", "5": NaN}, "question_id_specific": 118, "type": "TRAUMATOLOGY", "year": 2022, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n0540_6354", "title": "Surgical treatment of multiple knee ligament injuries in 44 patients: 2-8 years follow-up results.", "score": 0.017105263157894735, "content": "The purpose of the study was to evaluate the mid-term results of surgical treatment in different groups of patients with multiple knee ligament injuries. Review of our patients' records revealed that 48 acute and chronic patients were surgically treated for combined knee injury. Due to severe capsular damage in these injuries, open techniques were used. In our treatment protocol, avulsed ligaments and tears of the posterolateral and posteromedial corner were repaired if possible, whereas midsubstance tears of cruciate ligaments and chronic cases were reconstructed with autografts. Postoperatively, an accelerated program of rehabilitation was introduced, aiming to progressively mobilize the joint and improve muscle endurance. For the follow-up evaluation we designed a protocol composed of two parts. In the first part, anatomical lesions were recorded and in the second part, clinical evaluation was performed using the Lysholm score, the Tegner rating system, the IKDC evaluation form, and the KT1000. Student's t tests and chi-square tests were used for data analysis. Forty-eight patients (mean age 28.6+/-11.9 years; 41 males) were classified according to the specific anatomical structures involved. Group A included 12 anterior cruciate ligament (ACL) and medial structure injuries, group B included 11 ACL or posterior cruciate ligament (PCL) ruptures combined with posterolateral injuries, and group C consisted of 25 knee dislocations (ACL and PCL ruptures which might be combined with damage of the collateral ligaments). Thirty-eight patients were surgically treated during the acute phase and ten patients were treated chronically. Forty-four patients (91.6%) were followed up at a mean of 51.3+/-29.9 months. Average Lysholm score was 87+/-12.3; average Tegner score was 5.09+/-2.19 before accident and 4.34+/-2.12 in re-examination; IKDC score was A in 10 cases, B in 22, C in 6, and D in 6. The mean range of motion was 129.9 degrees +/-12.5 degrees . The average loss of extension and flexion were 1.6 degrees +/-2.5 degrees and 7.6 degrees +/-7.9 degrees , respectively. The side-to-side difference in corrected anterior and posterior translation in quadriceps neutral angle and in anterior translation in 30 degrees angle was <3 mm for about 65% of our patients. Surgical treatment of multiple knee ligament injuries, using autografts, provided satisfactory stability, range of motion, and subjective functional results. However, despite the improvement of the quality of life, the preinjury patients' activity level was not fully obtained in re-examination. Patients underwent surgical treatment during the acute phase had better scores in several points, but finally there was no statistical significance between acute and chronic patients. Moreover, no statistically significant differences were observed among the groups with specific damaged anatomical structures."}, {"id": "pubmed23n0065_2466", "title": "Injuries to the posterior cruciate ligament of the knee.", "score": 0.01609322974472808, "content": "The posterior cruciate ligament (PCL) is the strongest ligament about the knee and is approximately twice as strong as the anterior cruciate ligament. Its main function is to prevent the posterior dislocation of the tibia in relation to the femur, providing 95% of the strength to resist the tibial posterior displacement. Along with the anterior cruciate ligament (ACL) the PCL controls the passive 'screw home' mechanism of the knee in terminal knee extension. It also provides mechanical support for the collateral ligaments during valgus or varus stress of the knee. PCL ruptures are uncommon apparently due to its strong fibre structure. The most frequent injury mechanism in isolated PCL tears is a direct blow on the anterior tibia with the knee flexed thus driving the tibia posteriorly. Automobile accidents (in which the knee hits the dashboard) and soccer injuries (in which an athlete receives a blow to the anterior surface of the tibia during knee flexion) characteristically produce this type of injury. In other PCL injury mechanisms (hyperextension, hyperflexion or rotational injuries with associated valgum/varum stress), other knee structures are also often damaged. The most characteristic diagnostic finding in a knee with a PCL rupture is the 'posterior sag sign' meaning the apparent disappearance of the tibial tubercle in lateral inspection when the knee is flexed 90 degrees. This is due to gravity-assisted posterior displacement of the tibia in relation to the femur. A positive posterior drawer test performed at 90 degrees of flexion and a knee hyperextension sign are sensitive but nonspecific tests. False negative findings are frequent, especially in acute cases. If necessary, the clinical diagnosis of the PCL tear can be verified by magnetic resonance imaging, examination under anaesthesia, arthroscopy, or a combination of these modalities. If a PCL avulsion fragment has been dislocated, surgical treatment is recommended. In isolated, complete midsubstance tears of the PCL the majority of the recent studies recommend conservative treatment, since abnormal residual posterior laxity1 in most of these knees is consistent with functional stability and minimal symptoms. This has been the case even in athletes. In isolated PCL tears, the outcome seems to depend more on the muscular (quadriceps) status of the knee than on the amount of residual posterior laxity. Therefore, the conservative treatment protocol emphasises intensive quadriceps exercises, and only a short (under 2 weeks) immobilisation period followed by early controlled activities and early weightbearing.(ABSTRACT TRUNCATED AT 400 WORDS)"}, {"id": "pubmed23n0107_3961", "title": "[Examination of the knee joint. The value of clinical findings in arthroscopic control].", "score": 0.014646464646464647, "content": "Purely clinical examination of the knee joint can, at best, only be regarded as a \"screening procedure\". Diagnosis with the aid of apparatus (sonography, arthrography, CT, NMR) produces better results. However, arthroscopy performed by an experienced examiner confirms the diagnosis in cases of suspected meniscus injury or isolated lesions of the cruciate ligaments and leads to early and therefore optimal therapy. In a retrospective study 300 arthroscopies performed in 1985 were selected and evaluated. In 1986/87, a further 300 patients were clinically examined prospectively, according to the same criteria, and findings were compared with the arthroscopy performed the following day. Clinically, in 287 patients with multiple diagnoses, internal meniscus lesions were diagnosed in 162 cases (54%), external meniscus lesions in 38 (13%), chondropathia patellae in 54 (18%), and old ruptures of the cruciate ligaments in 46 (15%). In 13 patients no diagnosis could be established. Arthroscopically, pathology of the internal meniscus was found in 98 (33%) of the 300 patients, of the external meniscus in 40 (13%), cartilage damage in 103 (34%), old cruciate ligament ruptures in 51 (17%), and recent anterior cruciate ligament ruptures in 156 (52%); in 40 cases findings were normal. At 78%, the highest positive predictive value (proportion of tentative clinical diagnoses confirmed by arthroscopy) was found in cases of old ruptures of the anterior cruciate ligament, followed by external meniscus lesions (61%) and internal meniscus lesions (55%); i.e., only 55 out of 100 clinically suspected internal meniscus lesions are diagnosed by arthroscopy.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en108_37995", "title": "Anterior cruciate ligament injury", "score": 0.013807890222984561, "content": "Most ACL injuries can be diagnosed by examining the knee and comparing it to the other, non-injured knee. When a doctor suspects ACL injury in a person who reports a popping sound in the knee followed by swelling, pain, and instability of the knee joint, they can perform several tests to evaluate the damage to the knee. These tests include the pivot-shift test, anterior drawer test, and Lachman test. The pivot-shift test involves flexing the knee while holding onto the ankle and slightly rotating the tibia inwards. In the anterior drawer test, the examiner flexes the knees to 90 degrees, sits on the person's feet, and gently pulls the tibia towards him or herself. The Lachman test is performed by placing one hand on the person's thigh and the other on the tibia and pulling the tibia forward. These tests are meant to test whether the ACL is intact and therefore able to limit the forward motion of the tibia. The Lachman test is recognized by most authorities as the most reliable and"}, {"id": "pubmed23n0764_20185", "title": "[Surgical treatment of posteromedial corner injury combined with cruciate ligament rupture of knee].", "score": 0.013405054644808744, "content": "To investigate the methods and effectiveness of surgical treatment for posteromedial corner (PMC) injury combined with anterior cruciate ligament (ACL) and posterior cruciate ligament (PCL) ruptures. Between February 2009 and February 2012, 15 patients (15 knees) with PMC injury combined with ACL and PCL ruptures underwent PMC repair with suture anchor and ACL and PCL reconstruction. There were 7 males and 8 females with an average age of 39 years (range, 15-59 years). The causes of injury were traffic accident injury in 6 cases, sport injury in 7 cases, and sprain injury in 2 cases. The disease duration was 3-15 days with an average of 7 days. All patients presented positive results of anterior drawer test, posterior drawer test and valgus stress test, and dysfunction of knee joint. Of 15 cases, 3 had ACL and PCL ruptures, 5 had ACL rupture, 3 had ACL injury at the attachment point of the condyles crest, and 4 had PCL rupture; 9 had PMC tear at the femur insertion, 5 had PMC tear at the tibia insertion, and 1had PMC tear in the body area. All incisions healed by first intention with no complication of infection or stiffness of knee. All cases were followed up 18.4 months on average (range, 10-36 months). At last follow-up, 14 cases had normal knee flexion and extension ranges, but 1 case had 10 degree limitation of the knee extension. Except 1 case which had weakly positive valgus stress test, the other patients showed negative results of anterior drawer test, posterior drawer test, and valgus stress test. Based on the improved Lysholm classification standard, the results were excellent in 8 cases, good in 5 cases, and fair in 2 cases; the excellent and good rate was 86.7%. Early repair of the PMC and reasonable reconstruction of cruciate ligament can effectively restore the knee stability for patients with PMC injury combined with ACL and PCL ruptures."}, {"id": "pubmed23n1004_724", "title": "[Mid-term effectiveness of arthroscopic anterior cruciate ligament reconstruction combined with meniscus allograft transplantation].", "score": 0.013325281803542673, "content": "To summarize the mid-term effectiveness of arthroscopic anterior cruciate ligament (ACL) reconstruction combined with meniscus allograft transplantation. A clinical data of 21 patients treated with arthroscopic ACL reconstruction and meniscus allograft transplantation and followed up more than 5 years between February 2007 and December 2014 was retrospectively analyzed. There were 12 males and 9 females, aged from 18 to 45 years, with an average age of 23.5 years. The cause of injury was sport sprain in 15 cases, falling in 4 cases, and traffic accident in 2 cases. The time from injury to operation ranged from 2 to 36 months, with an average of 12 months. Among them, 15 patients underwent previous meniscectomy, with an average interval of 1.6 years (range, 3 months to 6.5 years). All patients were primary ACL reconstruction. Preoperative anterior drawer test, Lachman test, and pivot shift test were positive. Lysholm score was 43.6±10.2. International Knee Documentation Committee (IKDC) score was 60.50±14.06. Of the 21 patients, 10 were gradeⅠ-Ⅱcartilage injuries and 11 were grade Ⅲ cartilage injuries according to MRI. All patients were followed up 5.1-7.8 years, with an average of 5.5 years. There were 2 cases of numbness of lower extremity, 3 cases of slight exudation of incision, 2 cases of articular movement bounce, 5 cases of mild joint swelling and pain after exercise. At last follow-up, Lachman tests were negative in 18 cases and positive in 3 cases; anterior drawer tests were negative in 19 cases and positive in 2 cases; pivot shift tests were negative in all cases. Lysholm score was 84.5±16.5 and IKDC score was 85.25±4.60, which were significantly higher than those before operation ( An increased Q angle and hormonal"}, {"id": "pubmed23n0874_15497", "title": "[CLINICAL OBSERVATION OF ONE-STAGE ARTHROSCOPIC RECONSTRUCTION AND STRICT IMMOBILIZATION FOR TREATMENT OF KNEE DISLOCATION].", "score": 0.01110277569392348, "content": "To investigate the effectiveness of one-stage arthroscopic reconstruction and strict immobilization for 6 weeks for treatment of knee dislocation. Between August 2010 and May 2013, 22 cases (22 knees) of knee dislocation were treated with one-stage reconstruction and strict immobilization for 6 weeks. There were 15 males and 7 females, aged 21-54 years (mean, 31.5 years). The left knee and right knee were involved in 8 cases and 14 cases respectively. The disease causes were traffic accident in 12 cases, falling from height in 6 cases, and sports injury in 4 cases. The time between injury and operation was less than 2 weeks in 6 cases, 2-3 weeks in 10 cases, and more than 3 weeks in 6 cases. The results of anterior drawer test, posterior drawer test, and Lachman test were positive in all patients. The posterior displacement of the tibia was more than 10 mm. The results of valgus stress test and varus stress test were positive in 13 cases and 11 cases respectively. The preoperative knee range of motion was (58.2 ± 28.4)°, Lysholm score was 39.7 ± 4.6. All patients had anterior cruciate ligament rupture and posterior cruciate ligament rupture; combined injuries included medial collateral ligament rupture in 11 cases, lateral collateral ligament rupture in 9 cases, both medial and lateral collateral ligament rupture in 2 cases, femoral condylar avulsion fracture in 2 cases, and meniscus injury in 7 cases. No nerve or blood vessel injury was observed. All cases obtained primary healing of incision without infection. All the patients were followed up 12-48 months (mean, 27.8 months). At 12 months after operation, the results of the anterior drawer test, posterior drawer test, Lachman test, valgus stress test, and varus stress test were all negative; the knee range of motion increased was significantly to (121.3 ± 7.9)° (t = 30.061, P = 0.000); Lysholm score was 87.2 ± 6.1, showing significant difference when compared with preoperative score (t = 24.642, P = 0.000). A combination oathogopi osta ge reconstruction and strict immobilization for treatment of knee dislocation is a safe and effective method, good stability and joint function can be achieved."}, {"id": "pubmed23n0648_9380", "title": "[Primary clinical results of double-bundle anterior cruciate ligament reconstruction with semitendinosus allografts].", "score": 0.010799942553497057, "content": "To evaluate the primary clinical results of double-bundle anterior cruciate ligament reconstruction (ACLR) with semitendinosus allografts. From March 2006 to October 2006, 33 patients underwent double-bundle ACLR with semitendinosus allografts. The complete followed-up data of 31 patients was analyzed retrospectively. There were 24 males and 7 females aged 18-35 years old (average 25 years old). The injury was caused by sports accidents in 23 cases and traffic accidents in 8 cases, involving the left knee in 18 cases and the right knee in 13 cases. Anterior cruciate ligament rupture were confirmed by MRI and arthroscopy in all the patients, without lateral collateral ligaments injuries and posterior cruciate ligament injuries. The time from injury to operation was 1-43 months (average 11 months). The knee was fixed at 0 degree position after operation for 2 weeks and got knee joint rehabilitation exercises gradually. The incision of 2 patients showed effusion 4 and 7 days after operation, respectively, and healed after symptomatic treatment. The incision of 29 patients healed by first intention. There were no complications such as stiffness of knee joint, neurovascular injuries and joint infections. All the patients were followed up for 24-29 months (average 26 months). MRI displayed the anterior cruciate ligament grafts presented with good connection and signal similar to the normal 2 years after operation. There was significant difference between the preoperational value and the final follow-up value in terms of bilateral knee joint difference of prior laxity, Lachman test, and pivot shift test (P < 0.05 ). The circumference difference between the injured and the normal was (11.6 +/- 7.9) mm before operation and (5.0 +/- 3.1) mm at the final follow-up (P < 0.05). The Tegner score, Lysholm score, and International Knee Documentation Committee score was 3.83 +/- 1.15, 64.38 +/- 6.81, and 41.42 +/- 6.30, respectively, before operation, and 6.29 +/- 0.64, 94.45 +/- 3.03, and 95.72 +/- 3.10, respectively, at the final follow-up. There was a significant difference between before and after operation (P < 0.05). The primary clinical results of double-bundle ACLR with semitendinosus allografts are satisfactory and the allogeneic semitendinosus are good grafts for double-bundle ACLR."}, {"id": "wiki20220301en022_48996", "title": "Posterior cruciate ligament", "score": 0.010715684628728107, "content": "An additional test of posterior cruciate ligament injury is the posterior sag test, where, in contrast to the drawer test, no active force is applied. Rather, the person lies supine with the leg held by another person so that the hip is flexed to 90 degrees and the knee 90 degrees. The main parameter in this test is step-off, which is the shortest distance from the femur to a hypothetical line that tangents the surface of the tibia from the tibial tuberosity and upwards. Normally, the step-off is approximately 1 cm, but is decreased (Grade I) or even absent (Grade II) or inverse (Grade III) in injuries to the posterior cruciate ligament. The posterior drawer test is one of the tests used by doctors and physiotherapists to detect injury to the PCL. Patients who are suspected to have a posterior cruciate ligament injury should always be evaluated for other knee injuries that often occur in combination with an PCL injuries. These include cartilage/meniscus injuries, bone bruises, ACL"}, {"id": "pubmed23n0761_359", "title": "[Anatomical reconstruction of posterolateral complex in treatment of multi-ligament injury of knees].", "score": 0.010669362084456423, "content": "To evaluate the short-term effectiveness after static anatomical reconstruction of posterolateral complex (PLC) in the treatment of traumatic multi-ligament injury of the knee. Between June 2007 and July 2011, 23 cases of multi-ligament injury of the knee were treated. There were 15 males and 8 females with an average age of 41 years (range, 19-56 years). The injury was caused by traffic accident in 9 cases, sprain in 7 cases, bruise in 3 cases, and falling from height in 4 cases. The time between injury and operation was 13-78 days (mean, 32 days). The results of posterior drawer test and Lachman test were positive, and all cases complicated by varus and external rotation instability. The Lysholm score of the knee was 43.4 +/- 5.7. According to International Knee Documentation Committee (IKDC) scoring, all were rated as grade D. According to Fanelli typing, all were classified as type C. The X-ray films showed that load-induced posterior motion of the knee was (13.3 +/- 4.2) mm; the lateral joint space was (15.1 +/- 2.4) mm. Anterior cruciate ligament/posterior cruciate ligament and PLC were reconstructed simultaneously with auto-semitendinosus, gracilis tendon, and allogeneic tendon. All incisions healed by first intention, and no complication occurred. All patients were followed up 12-56 months (mean, 28 months). At last follow-up, the results of posterior drawer test and Lachman test were negative; 3 cases had varus instability, and 2 cases had external rotation instability. The Lysholm score of the knee was 85.6 +/- 16.7, showing significant difference when compared with preoperative score (t=-11.469, P=0.000). According to IKDC scoring, 7 cases were rated as grade A, 12 as grade B, and 4 as grade C; significant difference was found when compared with preoperative value (Z=4.285, P=0.000). The load-induced posterior motion of the knee was (5.1 +/- 4.4) mm, the lateral joint space was (3.2 +/- 2.8) mm, showing significant differences when compared with preoperative ones (P < 0.05). In the treatment of traumatic multi-ligament injury of the knee, the anatomical reconstruction of the PLC using auto-semitendinosus, gracilis tendon, or allogeneic tendon can obtain good short-term effectiveness."}, {"id": "wiki20220301en200_11449", "title": "Meniscus tear", "score": 0.009900990099009901, "content": "A meniscus can tear due to an internally or externally rotated knee in a flexed position, with the foot in a flexed position. It is not uncommon for a meniscal tear to occur along with injuries to the anterior cruciate ligament ACL and the medial collateral ligament MCL — these three problems occurring together are known as the \"unhappy triad,\" which is seen in sports such as football when the player is hit on the outside of the knee. Individuals who experience a meniscal tear usually experience pain and swelling as their primary symptoms. Another common complaint is joint locking, or the inability to completely straighten the joint. This is due to a piece of the torn cartilage preventing the normal functioning of the knee joint."}, {"id": "pubmed23n0316_2255", "title": "[Effect of external extra-articular ligament plasty on the results of anterior cruciate ligament reconstruction with patellar tendon, a 4 years follow-up].", "score": 0.009900990099009901, "content": "The purpose of this study was to compare the functional results obtained when an external extra-articular plasty was added to an anterior cruciate ligament (ACL) reconstruction using an autologous bone tendon-bone patellar tendon graft. The authors analyzed two consecutive series of 60 and 50 patients operated by the same surgeon for a chronic rupture of the anterior cruciate ligament, one by reconstruction of the cruciate ligament with a free graft of the patellar tendon supplemented by an external extra-articular plasty made with a quadriceps tendon graft and the second with an isolated free patellar tendon graft. Anterior laxity was measured before and after surgery, by dynamic X-rays and by the Medmetric KT-1000 arthrometer. Functional results were evaluated four years after operation, with the French A.R.P.E.GE score based on sport activity level and intensity. Anterior laxity was not different before operation in both groups and there was no difference between males and females. Medmetric KT-1000 arthrometer showed the same negative differential laxity immediately after surgery in both groups and the same evolution during the first 4 years, without any significant difference on laxity on the middle aspect of the knee. Radiological results were different. After a 4 years follow-up, anterior laxity did not show significant difference on the medial compartment of the knee (5.3 +/- 2.3 mm and 5.5 +/- 1.7 mm), but there was a significant minor laxity in the lateral compartment for the lateral extra-articular plasty group (11.0 +/- 2.3 mm against 14.8 +/- 3.8 mm)(p = 0.002). Functional results and sport activity were similar in both groups. Examination showed 4 positive pivot shift tests (2 \"sliding\" and 2 positive) in the group with extra-articular plasty, even though 8 positive pivot shift tests in the isolated ACL group (5 \"sliding\" and 3 positive) were found. This study, as well as five others studies found in literature, was not randomized. In all these series, the surgical techniques, the rehabilitation programs and the functional score evaluation were too different to allow any pertinent comparison. Extra-articular plasty helps to control the laxity of the lateral compartment of the knee which is incompletely controlled by ACL reconstruction, particularly in chronic cases. This is proved by radiological measurements and pivot shift tests. Jensen in 1983, about 205 patients with a 4 year follow-up and Noyes, which used an allograft patellar tendon, found an advantage to do extra-articular plasty. But Strum (in 1989), as O'Brien (in 1991) and Roth (in 1987), did not found any advantage with extra-articular plasty. It is therefore obvious, after a four-year follow-up, that extra-articular supplementation presents an advantage for reconstruction of the ACL. by a free graft of the patellar tendon in chronic cases. Further randomized study will confirm that isolated ACL reconstruction is possible in some well defined categories of anterior laxity."}, {"id": "pubmed23n0050_3857", "title": "[Diagnosis of anterior cruciate ligament injury of the knee joint].", "score": 0.00980392156862745, "content": "From January, 1979 to May, 1989, 107 patients with problems related to anterior cruciate ligament (ACL) were treated in our hospital. 100 of the patients had anterior cruciate ligament injury confirmed by arthrotomy or arthroscopy. The remaining 7 patients were found to be normal either by arthroscopy or arthrotomy. 29 patients had fresh ACL injury and 71 old. All the patients had history of trauma of the knee joint. Swelling and pain in the affected knee joint took place in fresh cases and few of them complained of instability or deformity of the knee. On examination, floating patella test was positive in the majority of the fresh cases. It was shown that accurate diagnosis could be made by Lachman test rather than by conventional anterior drawer test in dealing with fresh injury, but with old ones, Lachman test didn't show the advantages. Examination under anesthesia or arthroscopy helped a lot in diagnosing fresh ACL injury. Anterior drawer test (ADT) was significant in determining the existence of ACL injury. When ADT was positive, ACL injury was found in the majority of the cases, however, injured ACL couldn't be ruled out by negative ADT only. Positive valgus stress test on 0 degrees position suggests possibility of ACL injury, even ADT was negative. Despite the negative anterior drawer test positive posterior drawer test on three directions indicated the injury of the posterior cruciate ligament and the anterior cruciate ligament. The positive rate of ADT was higher than that of pivot shift test in dealing with anterior cruciate ligament injury. Positive pivot shift test suggests ACL injury."}, {"id": "pubmed23n0616_18003", "title": "[The ACL tear from the pre-operative analysis to a 2-year follow-up, influence of the graft choice on the subjective and objective evaluation].", "score": 0.009708737864077669, "content": "This study is a synthesis of three series. The first study was prospective on 418 patients with an anterior cruciate ligament (ACL) tear (group I). Two population of ACL ruptures were identified. One population with a postero-lateral bundle preserved in 16%, the mean medial anterior tibial translation side to side was 4.97 mm, the Lachman test was delayed in 40% with no or glide pivot shift in 73%. The second population with a complete ACL tear had a mean medial anterior tibial translation side to side of 7.93 mm, the Lachman test was soft in 98% with gross pivot shift in 80%. The second study was a retrospective study on 258 patients (group II) at 26 months follow-up, it correlated the impact of the type of graft on the clinical objective and subjective results. Twenty-eight percent had anterior knee pain, 33% for the patellar tendon and 25% for the hamstrings, the subjective IKDC was significantly lower for the painful knees, and 68% of the patellar tendon had a hypoesthesia and only 32% for the hamstrings. The ability to walk on the knee was 68% for the hamstrings and 35% for the patellar tendon. The third study was retrospective on 127 patients, 24 months after ACL reconstruction (group III), all were tested on a isokinetic machine for the extensor, the flexor and the internal rotator. In the total population, a 10% extensor and flexor deficit and a 5% rotator deficit was noted. A significant difference between patellar tendon and hamstrings in terms of muscular recovery was found. It pointed out that a more specific rehabilitation should be done on the hamstring group. The muscular recovery was correlated to the highest subjective score. This study allowed the surgeon to be more specific in the ACL tear definition, to adapt the graft choice to the type of sport activity but also to the type of work the patient does and finally to modify the rehabilitation protocol for the hamstring technique."}, {"id": "pubmed23n0978_5069", "title": "Open knee dislocation with a patellar tendon rupture: Result of staged surgical repair.", "score": 0.009615384615384616, "content": "Knee dislocation with concomitant multiligament injury is a rare and devastating injury. We report the successful repair of a rare case of open knee dislocation with concomitant multiligament injury and patellar tendon rupture of an 18-year-old male due to a motorcycle accident. The patient presented with an open wound running parallel to the knee joint line and patellar tendon rupture with full exposure of the cartilage of the distal femur. Staged surgical management including the application of a ring-type external fixator with a hinged joint, lateral collateral ligament repair, medial collateral ligament reconstruction using autogenous hamstring tendon, and joint release was performed. Range of movement was recovered to 0 degrees of knee extension and 80 degrees of knee flexion, and extension lag was negative. The Lysholm score of the patient was recovered to 92. The patient was able to return to work in the construction field 2 years after sustaining the injury. The patient had no complaint of pain and was able to resume construction work, even though reconstruction of the anterior cruciate ligament and posterior cruciate ligament was not performed. The application of a hinged ring-type external fixation device might play a key role in early range of movement restoration and to maintain the reduced position and acceptable recovery of the posterior cruciate ligament injury without the need for reconstructive surgery. This report is the first to describe the safety and effectiveness of staged surgical management for the repair of open knee dislocation with concomitant multiligament injury and patellar tendon rupture. However, further studies with longer follow-up periods will be needed to observe the development of osteoarthritis or weakness of the knee. Staged surgical management is a safe and effective procedure for repairing an open knee dislocation with concomitant multiligament injury and patellar tendon rupture."}, {"id": "pubmed23n0751_15183", "title": "[Evaluation of the clinical results in patients with symptomatic partial tears of the anterior cruciate ligament diagnosed arthroscopically].", "score": 0.009615384615384616, "content": "The study presents a retrospective evaluation of clinical data and arthroscopic findings in a group of our patients with symptomatic knee instability due to a partial tear of the anterior cruciate ligament (ACL). The group included 31 patients diagnosed with symptomatic partial ACL tears, i.e. an isolated tear of the posterolateral (PL) or the anteromedial (AM) bundle. The patients' average age was 26.5 years. A side-to-side difference in ventral knee laxity was assessed using the anterior drawer test and the Lachman test under general anaesthesia before arthroscopy was commenced; rotational knee laxity was evaluated by the pivot shift test. An objective evaluation of side-to-side ventral laxity differences in both knees was performed on the GNRB® arthrometer with an applied pressure of 134 N and 250 N in the conscious patient. During arthroscopic examination, findings on the two ACL bundles were recorded. All 31 patients were diagnosed with symptomatic partial ACL tears, of them 22 had a PL bundle lesion and nine had an AM bundle tear. All patients with PL bundle lesions only reported problems in association with pivot sports, and all patients with AM bundle tears had problems regardless of any sports activities. In all patients with isolated AM bundle tears, the lesion was located close to its femoral attachment. In the patients with PL bundle tears, femoral location was found in 68% and tibial location in 32% of the patients. In the patients with partial PL bundle lesions, + and ++ results in the pivot shift test were recorded in 32% and 68% of the treated patients, respectively. The Lachman test showed + and ++ results in 71% and 9% of the patients, respectively. The anterior drawer test had negative results in 87% and positive + results in 13% of the patients. The side-to-side difference on the GNRB arthrometer ranged from 0.4 to 2.3 mm at a pressure of 134 N and from 1.2 to 4.2 mm at 250 N in the patients with isolated PL bundle lesions. In the patients with AM bundle lesions, the results were as follows: pivot shift test, 89% negative. 11% positive +; Lachman test, 56% negative, 44% positive +; anterior drawer test, 89% +, 11% ++; GNRB test, 2.2 to 4.4 mm at 134 N, and 4.3 to 7.1 at 250 N. The diagnosis of partial ACL lesions, i.e., isolated tears of the AM or the PL bundle, requires accurate knowledge of knee anatomy and its biomechanics. In accordance with other authors our results showed that an arthroscopic examination of both bundles of the ligament as well as knee laxity evaluation under general anaesthesia are most essential for making the definite diagnosis in partial ACL tears. They also confirmed that, in isolated AM bundle lesions, ventral laxity is present more often particularly at a higher degree of knee flexion while, in PL bundle lesions, rotational laxity is more frequent and ranges from 0 to 30 degrees of knee flexion. To make the definite diagnosis of partial ACL tears, patient medical history, clinical knee examination including instability type and degree assessment under general anaesthesia and, most importantly, arthroscopic findings on both ACL bundles are necessary."}, {"id": "wiki20220301en058_57566", "title": "Drawer test", "score": 0.009523809523809525, "content": "The drawer test is used in the initial clinical assessment of suspected rupture of the cruciate ligaments in the knee. The patient should be supine with the hips flexed to 45 degrees, the knees flexed to 90 degrees and the feet flat on table. The examiner positions himself by sitting on the examination table in front of the involved knee and grasping the tibia just below the joint line of the knee. The thumbs are placed along the joint line on either side of the patellar tendon. The tibia is then drawn forward anteriorly. An increased amount of anterior tibial translation compared with the opposite limb or lack of a firm end-point may indicate either a sprain of the anteromedial bundle or complete tear of the ACL. If the tibia pulls forward or backward more than normal, the test is considered positive. Excessive displacement of the tibia anteriorly suggests that the anterior cruciate ligament is injured, whereas excessive posterior displacement of the tibia may indicate injury of"}, {"id": "pubmed23n0068_3577", "title": "The long-term course after treatment of acute anterior cruciate ligament ruptures. A 9 to 16 year followup.", "score": 0.009523809523809525, "content": "Acute total ACL (N = 60) and concomitant medial collateral ligament (N = 46) ruptures were repaired in 60 patients (mean age, 28 years) without augmentation. Menisci were removed in 23 knees. Fifty-three (88%) of the patients were reexamined 9 to 16 years later with special emphasis on manual and instrumented stability testing (Stryker, Genucom), knee function score (Lysholm), and activity level (Tegner). Standing roentgenograms (30 degrees of knee flexion) were taken in 69% of the patients. At followup, an ACL reconstruction had been performed in seven patients (12%) due to symptomatic instability. Sixty-four percent of the knees had a positive Lachman sign and 40% a positive pivot shift. Sagittal laxity difference was +3 mm or more in 57%. Knee function score was a mean of 86 +/- 12 points. The mean activity level had changed from recreational team sports (Level 7) to recreational individual sports (Level 5). Only patients with good knee stability were able to perform demanding sports and could continue at their desired activity level. Osteoarthritis of slight to moderate degree (Fairbank I/II) was found in 58% of the patients younger than 35 years of age at the time of trauma and in 87% of the older patients. Knees with intact menisci had less osteoarthritis than knees with removed menisci (P less than 0.05)."}, {"id": "wiki20220301en099_32461", "title": "Cruciate ligament", "score": 0.009433962264150943, "content": "In animals the two cruciate ligaments that cross the inside of the knee joint are referred to as the cranial cruciate (equivalent to anterior in humans) and the caudal cruciate (equivalent to the posterior in humans). The cranial cruciate ligament prevents the tibia from slipping forward out from under the femur. Stifle injuries are one of the most common causes of lameness in rear limbs in dogs, and cruciate ligament injuries are the most common lesion in the stifle joint. A rupture of the cruciate ligament usually involves a rear leg to suddenly become so sore that the dog can barely bear weight on it. How a rupture can occur: There are several ways a dog can tear or rupture the cruciate ligament. Young athletic dogs can be seen with this rupture if they take a bad step while playing too rough and injure their knee. Older dogs, especially if overweight, can have weakened ligaments that can be stretched or torn by simply stepping down off the bed or jumping."}, {"id": "pubmed23n0395_17127", "title": "[Ligamentoplasty of the anterior cruciate ligament after 40 years: a series of 41 patients].", "score": 0.009433962264150943, "content": "Ligamentoplasty for tears of the anterior cruciate ligament (ACL) is generally thought to be unreliable after the age of 40 years. The purpose of this retrospective analysis was to assess the five-year outcome after ACL plasty in patients aged over 40 at the time of surgery. Between 1990 and 1997, 41 patients aged 44.5 +/- 4.5 years (28 men, 25 right side) underwent intra-articular reconstruction of the ACL. Clinical and radiological assessment using the IKDC criteria was obtained at a mean 62 months postoperatively using manual instrumental measurements with KT-1000. The indication for reconstruction was instability in daily life activities for 40 patients and difficulties encountered in sports activities in one; there were three cases of remodeling. Arthroscopy was used in all cases to perform a patellar tendon graft (bone-tendon-bone in 30 cases associated with extra-articular lateral reinforcement in eleven). There were no significant complications. Among 12 patients who initially participated in competition sports, seven were able to resume their activity at their former level. At last follow-up, global IKDC score was A for 12, B for 25, and C for three and D for one. All patients scored C or D had a poor IKDC symptom score, basically because of pain. Motion was not modified. The IKDC radiology score was A for 25, B for 15 and C for one, but the three cases of remodeling did not progress. Prognostic factors for overall IKDC result were: age of the patient at the time of reconstruction (under 45 years), and the delay to surgery for accident victims (less than one year). The following criteria had no effect at last follow-up: gender, sport practiced, type of initial laxity (anterior alone or global anterior), presence of meniscal damage, preservation of the medial and/or lateral meniscus, chondral injury observed peroperatively, and use of a lateral reinforcement. Radiographic remodeling observed in this group of 41 patients was related to delay from accident to surgery (p=0.0007) and preservation of the medial meniscus (p=0.03). Age, gender, type of activity before surgery, degree of initial and residual laxity had no statistically significant effect on remodeling. Age over 40 years is not a contraindication for arthroscopic free patellar tendon graft for the treatment of chronic anterior laxity. Using rigorous preoperative assessment criteria (delay from accident to surgery, absence of joint space narrowing on the AP and lateral view before intervention, symptomatic instability in daily life activities and motivated patient) this type of procedure can be performed safely and provides good functional outcome at five years. The current follow-up is insufficient to judge potential joint degradation. Age is not a contraindication if certain precautions are taken."}, {"id": "pubmed23n0761_4542", "title": "Diagnosis of ACL and meniscal injuries: MR imaging of knee flexion versus extension compared to arthroscopy.", "score": 0.009345794392523364, "content": "The aim of the study was to evaluate whether MR Imaging of the knee at 30° and 55° of flexion can improve the diagnosis of anterior cruciate ligament and menisci injuries compared to arthroscopy and imaging during extension of the knee joint. Knee joints from 40 patients with clinical suspicion of an anterior cruciate ligament (ACL) rupture were examined using MRI while the knee joint was either extended or flexed at 30° and 55° of knee flexion. A standard MR knee coil was used at extension, whereas at 30° and 55° of flexion a non-metallic positioning device and a flexible surface coil was placed ventral to the patella. Sagittal T2-weighted TSE sequences were acquired. In 29 of 40 patients, arthroscopy results were compared to the MRI examinations. Image quality of MRI examinations was evaluated using a three-point rating scale in a blinded fashion. Images were compared between groups and rated as better quality, same quality, or worse quality. Additionally, each angle MRI was compared to arthroscopy results. Partial ACL ruptures were diagnosed with 63% accuracy using MR imaging at 30° and 55° of knee flexion compared to 50% accuracy during knee extension. MRI imaging of complete ACL ruptures resulted in 83% accuracy of diagnosis when imaged at 30° flexion, 93% accuracy at 55° flexion, and 83% accuracy at extension. The accuracy of diagnosing medial meniscus lesions was 73% at extension, 64% at 30° flexion and 73% at 55° of flexion. MR imaging was only able to diagnose lateral meniscus tears with 55% accuracy in all three knee positions. The diagnosis of meniscal tears was more difficult due to small peripheral tears. The improved results in the diagnosis of ACL tears in response to 30° flexion and in particular in response to 55° flexion were based on the fact that the anterior cruciate ligament moved further away from the intercondylar roof with increased knee flexion. During flexion the ligament tension decreased, which causes the anterior cruciate ligament to have cylindrical shape and therefore made visualization of the injury easier. In conclusion, MR Imaging of the knee at 55° of flexion and less at 30° of flexion allows an improved diagnosis of injuries to the anterior cruciate ligament as compared to MRI examinations at extension. The diagnosis of meniscal injuries, however, was not superior at both flexion positions compared to commonly performed examinations at knee extension. "}, {"id": "pubmed23n0270_627", "title": "[Results of follow-up of conservatively treated isolated fresh anterior cruciate ligament rupture].", "score": 0.009345794392523364, "content": "35 patients with an arthroscopically confirmed isolated fresh anterior cruciate ligament rupture were subjected to follow-up examination after an average period of four years. In all the 35 patients a conservative treatment schedule had been followed post-arthroscopically, with a physiotherapy on a neurophysiological basis. Of the 35 isolated anterior cruciate ligament ruptures, 24 were complete and 11 partial ruptures. The follow-up examination results are based on subjective scores (O'Donoghue Score, Lysholm Score) and on one objective score (objective ODonaghue Score), as well as on the Lachman Test and the Pivot Shift Sign. In the partial anterior cruciate ligament ruptures we obtained mostly good to satisfactory results with the subjective and objective scores and a lower incidence of surgery; the original performance ratings at sports were largely maintained. On the other hand, the complete isolated anterior cruciate ligament ruptures yielded mainly satisfactory to poor results with the objective scores and a high rate of repeat surgery especially in the case of meniscus tears, and a major setback in the original sports performance ratings."}, {"id": "wiki20220301en058_57568", "title": "Drawer test", "score": 0.009336459961785653, "content": "See also Anterior cruciate ligament injury Posterior cruciate ligament injury Lachman test References Examination of the knee"}, {"id": "pubmed23n0335_3918", "title": "[Rupture of the pes anserinus superficialis and partial rupture of the patellar ligament. Rare concomitant injures in complex knee injuries].", "score": 0.009259259259259259, "content": "We present a rare case of combined knee joint lesions in a 25 year old patient. Besides the commonly reported injuries of the knee joint due to directly applied valgus force, forceful quadriceps muscle contraction, external rotation at flexed knee causing combined lesions such as rupture of the anterior cruciate ligament, rupture of the posterior cruciate ligament and rupture of the medial collateral ligament, a rare combination of the above mentioned lesions and a rupture of the lateral meniscus, an osteochondral fracture of the lateral femur condylus, a rupture of the medial patellofemoral retinacula as well as a complete rupture of the superficial pes anserinus and a partial rupture of the patellar ligament was encountered."}, {"id": "pubmed23n1102_14643", "title": "[Early Operative Treatment of Anterior Cruciate Ligament Lesions: 3-Year Experience].", "score": 0.009259259259259259, "content": "PURPOSE OF THE STUDY The aim of this study is to show new possibilities for the treatment of anterior cruciate ligament injuries. For many years, the long-established rules have been followed. However, throughout history, treatment strategies have been gradually modified. The advent of new modern technologies now makes it possible to perform reconstructive procedures already in the acute phase, and thus shorten the treatment time significantly. MATERIAL AND METHODS 85 patients were followed after an acute knee injury with an isolated anterior cruciate ligament (ACL) lesion. There were 51 men and 34 women with the average age of 32 years. All patients underwent an acute knee arthroscopy with subsequent ACL reconstruction according to the type of lesion. The results were evaluated according to the clinical examination, Lysholm and Tegner activity scores. We compared 2 surgical techniques (Internal Brace, All-Inside ACL reconstruction) and assessed the possible occurrence of complications. RESULTS The injury was most often observed in patients aged 20-40 years, when mainly sports activities put a major strain on the knee joint. After performing ACL reconstruction, all patients reported a subjective improvement in the overall condition. The number of complications was very low - only in connection with a slower warm-up of the knee joint after surgery. DISCUSSION All patients achieved full stabilization of the knee joint, which is objectified by an increase in the Lysholm score. The improvement in patient's subjective feelings is also confirmed by the increase in Tegner activity scores. Only a few patients experienced a slight restriction of movement in the 8th week after surgery, but thanks to intensive rehabilitation the function of the knee joint was completely restored within 3 months of the operation. CONCLUSIONS Our study proves that if the set rules are followed, it is possible to perform the ACL reconstruction already in the acute phase. However, in order to choose this approach, the patient's motivation, gently performed procedure and early intensive rehabilitation are necessary. When meeting these criteria, the patient is able to return to normal life, including sports activities, quite early and there is no increased risk of complications. Key words: Internal Brace, ACL reconstruction, arthroscopy, early, rehabilitation, All-Inside."}, {"id": "pubmed23n0224_14097", "title": "Acute combined posterior cruciate and posterolateral instability of the knee.", "score": 0.009174311926605505, "content": "This report concerns 13 consecutive patients (13 knees) who underwent operative treatment for acute combined posterior cruciate and posterolateral instability due to combined injury to the posterior cruciate ligament and the arcuate ligament complex. Our purpose was to examine the method of diagnosis and the results in these patients. There were 12 males and 1 female (average age, 26 years). Five patients were injured in a motor vehicle accident, four in sports activities, and four in nonsports activities. The mechanism of injury was an anteromedial blow to the flexed knee in six patients, a fall onto the knee in two, and unknown in five patients. Eleven patients were available for follow-up evaluation (average, 56 months), and in each the result was rated as good, fair, or poor. In 10 patients (90%) the results were rated as good subjectively, in 11 (100%) as good functionally, and in 8 (73%) as good objectively. Injury to both the posterior cruciate ligament and the arcuate ligament complex can result from rotational force that can be due to a blow to the anteromedial aspect of the knee. Diagnosis can be made by a combined positive response to the posterior drawer test, the anterior drawer test performed with the tibia in internal rotation, the abduction and adduction stress tests performed with the knee in full extension, the posterolateral drawer test, and the external rotation-recurvatum test. In a knee with concomitant injury to the posterior cruciate ligament and the arcuate ligament complex that requires surgical repair, all injured structures should be explored and repaired to ensure a subjectively, objectively, and functionally good result."}, {"id": "pubmed23n0726_24935", "title": "An extra-articular procedure improves the clinical outcome in anterior cruciate ligament reconstruction with hamstrings in female athletes.", "score": 0.009174311926605505, "content": "A positive glide is a common finding after ACL reconstructions, especially in women. The aim of this study was to prospectively evaluate the role of Cocker-Arnold's extra-articular procedure in reducing the incidence of a residual postoperative rotational knee laxity. Sixty patients affected by an ACL injury with a +2 (clunk) or +3 (gross shift) pivot-shift test entered this prospective study; they were randomly assigned to group A (control group, hamstrings) or group B (study group, hamstrings plus Cocker-Arnold). Thirty-two patients entered group A and 28 group B. At follow-up, patients underwent clinical evaluation, KT-1000 arthrometer and Lysholm, Tegner, VAS and subjective and objective IKDC form. At a mean follow-up of 44.6 months, the same expert surgeon reviewed 55 patients (28 group A and 27 group B). The comparison of the results of the evaluation scales used and of the KT-1000 arthrometer did not show statistically significant differences (p>0.05). Lachman test was negative (S/S) in all the patients of both groups (100 %). A residual positive pivot-shift (glide) was found in 16 patients (57.1 %) of group A and in five patients (18.6 %) of group B (p<0.05). The extra-articular MacIntosh procedure modified by Cocker-Arnold in combination with ACL reconstruction significantly reduces the rotational instability of the knee."}, {"id": "pubmed23n0294_3525", "title": "[Biomechanical correlations of lesions associated with traumatic diseases of the anterior cruciate ligament. Analysis with magnetic resonance].", "score": 0.009009009009009009, "content": "To investigate the correlations between traumatic injuries of the anterior cruciate ligament and other ligamentous, meniscal and bone traumatic injuries, a series of 193 patients with anterior cruciate ligament injuries studied with MRI between January 1992 and December 1994, was retrospectively reviewed. MR results were compared with arthroscopic and/or surgical findings in most (181) patients; in the remaining 12 patients, clinical follow-up was performed. We used two 0.5 superconductive MR units, with dedicated coils and T1-weighted spin-echo and T2*-weighted gradient-echo sequences on the axial, sagittal and coronal planes. Anterior cruciate ligament injuries were associated with other ligamentous, meniscal and bone injuries in 78% of patients. The patients were classified in 5 groups depending on biomechanics and the association of injuries: -group I: isolated injury of the anterior cruciate ligament (41 patients), most frequently caused by forced extension stress associated with \"kissing contusions\" of the anterior portion of the lateral femoral condyle and of the lateral tibial plateau; this type of injury is less frequently caused by forced flexion stress associated with avulsion fracture of the tibial eminence; -group II: associated injury of the anterior cruciate ligament and medial compartment (62 patients), caused by forced flexion-external rotation stress (abduction, valgism and external rotation). The classic association of this mechanism was the injury of the anterior cruciate ligament, medial collateral ligament and medial meniscus (O'Donoghue triad) (9 patients). Valgus stress and the pivot-shift phenomenon can impact the tibial and femoral articular surfaces, with consequent osteochondral contusion; -group III: associated injury of the anterior cruciate ligament and lateral compartment (26 patients), caused by forced flexion-internal rotation stress (adduction, varism and internal rotation). This mechanism can cause, as a typical bone lesion, Segond fractures; -group IV: associated injury of the anterior cruciate ligament, lateral and medial compartments observed in 52 patients with different associations of varus-valgus and rotatory stress; -group V: in 5 patients, anterior cruciate ligament injury was associated with traumatic injury of the posterior cruciate ligament; in this case, posterior displacement of the tibia and knee hyperextension were the most common mechanisms of injury. In conclusion, our results demonstrate that anterior cruciate ligament injuries due to traumatic sprains of the knee are rarely isolated (21%). Thus, it is important to know the biomechanics of knee trauma to read MR images in order to detect possibly associated injuries. The final goal is to assess the actual extent of the traumatic damage for best subsequent clinical-therapeutic management."}, {"id": "pubmed23n0263_7093", "title": "[Mid-term results of 173 cases of surgery of the anterior cruciate ligament using the Mac Intosh procedure, reinforced by the Kennedy Ligament Augmentation Device].", "score": 0.009009009009009009, "content": "This is a retrospective study of 173 chronic laxities of the knee operated on between May 1985 and December 1988 using the Mac Intosh procedure, reinforced by the Kennedy L.A.D. Follow-up was between 4-8 years and the same surgeon operated on all the knees. 171 patients were operated on (113 men and 58 women) aged between 15-49 years (average age 26.5 years). The vast majority were sportsmen both at competition level (51 cases) and at recreational level (119 cases). The average time-span between accident and intervention was 15 months (1-240 months). In the pre-operative assessment, 80 cases (46.2 per cent) were found to have a grade II Lachman test and 91 cases (52.6 per cent), a grade III Lachman test; a positive pivot shift was found in more than 95 per cent of cases. There were lesions of the medial meniscus in 101 cases (58.4 per cent), of the lateral meniscus in 94 cases (54.4 per cent) and there was no meniscal lesion in only 41 cases (23.8 per cent). Finally there were 22 chondral lesions of the medial femoral condyle (12.7 per cent) and 7 of the lateral condyle (4.1 per cent). These were based on 159 cases (14 lost to follow-up before the third post-operative month), but only 107 (61.8 per cent) were reviewed by one of us (i.e. not the original surgeon). Tolerance of the L.A.D. was excellent. There was no sign of synovitis or joint effusion persisting beyond 1 year. Furthermore, there were no serious complications (no sepsis, only 4 cases (2.3 per cent) of joint stiffness, 2 cases of reflex sympathetic dystrophy and 5 cases of sural phlebitis (2.9 per cent). Moreover, the few minor complications that arose resolved with the appropriate treatment. Anatomical results, evaluated using. Lachmans test (maximum manual Lachman measured with the Bercovy Laximeter) showed 24 cases (22.4 per cent) with zero differential residual laxity, 54 cases (50.5 per cent) with a differential laxity of between 0-2 mm; 20 cases (18.7 per cent) with a laxity of between 2-4 mm and in 9 cases (8.4 per cent) a laxity of greater than 4 mm. Functional results, using the Arpège C.L.A.S. system showed good results in 83 per cent (score > and unsatisfactory results in 17 per cent of cases. The long time-span between intervention and subsequent review with the C.L.A.S. system should be taken into account. However, the proportion of competitive sportsmen to recreational sportsmen was practically identical when comparing pre-traumatic figures (C = 29.5 per cent, R = 68.8 per cent) to figures at the time of follow-up (C = 21 per cent, R = 69.5 per cent). Radiological results (A.P. and Schuss films) showed 63 knees (58.9%) with no signs of either medial or lateral, preliminary or full-blown, femoro-tibial osteo-arthritis. With an average follow-up of > 5 years (4-8 years), it is possible to say that ligamentoplasty of the anterior cruciate ligament using the Mac Intosh procedure reinforced with the Kennedy L.A.D. is not causative of any iatrogenic problems. The anatomical results were totally satisfactory (almost normal) in 72.9 per cent of cases; the radiological results showed \"\"normal'' knees in 58.9 per cent of cases and functional results were identical to results obtained using many other techniques."}, {"id": "pubmed23n1051_2265", "title": "The influence of a meniscal bucket handle tear on the Posterior Cruciate Ligament Angle in Anterior Cruciate Ligament Rupture - A case report.", "score": 0.008928571428571428, "content": "Chronic anterior cruciate ligament (ACL) tear might be difficult to diagnose on MRI. Indirect signs might be a typical meniscal or cartilage lesion, or a spontaneous anterior drawer visualized by a decreased angle of the posterior cruciate ligament (PCL). A 27-year-old former ballet dancer was admitted to the emergency department for a locked left knee, without never having experienced previous symptoms of giving way or locking. The MRI performed revealed a medial meniscus bucket handle tear, without traumatic bone marrow oedema or ligament injury. The PCL angle was 130°. A former MRI of her left knee performed 1 year previously to investigate on the recurrent catching of her left knee showed a grade III medial meniscal tear of the posterior horn, and buckling of the PCL angle of 100°, as a sign of chronic ACL rupture. During arthroscopy and medial meniscal repair, the ACL showed complete loss of tension, and was therefore reconstructed simultaneously to enable proper meniscal healing. Chronic ACL insuffiency is a major risk factor for subsequent medial meniscus tear, especially bucket handle tear. The locked knee might unable proper pre-operative clinical examination. The preoperative MRI therefore being the only possibility to diagnose concomitant ligamentous injury. This is the first case reported in literature showing, that a positive PCL angle sign might be falsely negative due to a locked medial meniscus bucket handle tear."}, {"id": "pubmed23n0049_19299", "title": "A modified repair for the anterior cruciate ligament deficient knee.", "score": 0.008928571428571428, "content": "A retrospective analysis of 48 sportsmen and women from an original series of 76 consecutive patients who had undergone a modified McIntosh repair was carried out to establish whether or not the procedure could provide a satisfactory recovery and return to previous ability. The type and level of sport before injury was compared with that after operation. Symptoms of pain and giving way, and examination findings of pivot shift, and Lachman's test were compared before and after operation. Of the 48 patients assessed, 28 (58%) returned to full sporting capacity; 17 (35%) patients were participating in different sports or lower levels of their previous sports, and three patients did not participate in any sport. The more severely symptomatic knees did not perform so well after operation. The degree of preoperative anterior draw and Lachman's test did not influence the final result and the pivot shift, present in all before operation, was abolished in all but one case, which remained badly symptomatic. Concurrent meniscal injury or medial or lateral laxity did not influence return to sport once a full postoperative recovery was made. No deterioration was noticed in the level of sporting ability achieved thereafter. In this study it has been shown that the modified McIntosh repair is a swift extra-articular reconstruction for the anterior cruciate ligament deficient knee, which is less elaborate than previously described Ellison and McIntosh procedures, and which has produced a comparable result."}]}}}} {"correct_option": 4, "explanations": {"1": {"exist": true, "char_ranges": [[582, 787]], "word_ranges": [[93, 127]], "text": "Also, since we need a significant drop in intraocular pressure because the numbers are high (34 and 31), we can also rule out carbonic anhydrase inhibitors and alpha-2 agonists (options 1 and 3 incorrect)."}, "2": {"exist": true, "char_ranges": [[499, 581]], "word_ranges": [[80, 93]], "text": "Since the patient is asthmatic, we should rule out beta-blockers (wrong answer 2)."}, "3": {"exist": true, "char_ranges": [[582, 787]], "word_ranges": [[93, 127]], "text": "Also, since we need a significant drop in intraocular pressure because the numbers are high (34 and 31), we can also rule out carbonic anhydrase inhibitors and alpha-2 agonists (options 1 and 3 incorrect)."}, "4": {"exist": true, "char_ranges": [[60, 219]], "word_ranges": [[10, 34]], "text": "In the past, beta-blockers were the first choice, but nowadays they have been displaced by the group that lowers intraocular pressure the most: prostaglandins."}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "We are asked about the first choice treatment for glaucoma. In the past, beta-blockers were the first choice, but nowadays they have been displaced by the group that lowers intraocular pressure the most: prostaglandins. Their efficacy and more convenient dosage (once a day) generally make them the group of choice. Beta-blockers would remain as a second choice in cases in which the side effects of prostaglandins (change in eye color, increase in eyelash size) make us initially leave them aside. Since the patient is asthmatic, we should rule out beta-blockers (wrong answer 2). Also, since we need a significant drop in intraocular pressure because the numbers are high (34 and 31), we can also rule out carbonic anhydrase inhibitors and alpha-2 agonists (options 1 and 3 incorrect). And opt for the group that can lower intraocular pressure the most. Answer 4 correct. And then a couple of nuances of the statement. It would be more correct to talk about intraocular pressure and not \"ocular pressure\". And the wording could be improved so as not to repeat the word \"as\" in the last sentence.", "full_answer_no_ref": "We are asked about the first choice treatment for glaucoma. In the past, beta-blockers were the first choice, but nowadays they have been displaced by the group that lowers intraocular pressure the most: prostaglandins. Their efficacy and more convenient dosage (once a day) generally make them the group of choice. Beta-blockers would remain as a second choice in cases in which the side effects of prostaglandins (change in eye color, increase in eyelash size) make us initially leave them aside. Since the patient is asthmatic, we should rule out beta-blockers ([HIDDEN]). Also, since we need a significant drop in intraocular pressure because the numbers are high (34 and 31), we can also rule out carbonic anhydrase inhibitors and alpha-2 agonists ([HIDDEN]). And opt for the group that can lower intraocular pressure the most. [HIDDEN]. And then a couple of nuances of the statement. It would be more correct to talk about intraocular pressure and not \"ocular pressure\". And the wording could be improved so as not to repeat the word \"as\" in the last sentence.", "full_question": "A 67-year-old man, diabetic and asthmatic, with primary open-angle glaucoma in both eyes. He has an ocular pressure of 34 mmHg in his right eye and 31 mmHg in his left eye. Which of the following pharmacological groups is the most indicated as first choice as hypotensive treatment?", "id": 456, "lang": "en", "options": {"1": "Carbonic anhydrase inhibitors.", "2": "Beta-blockers.", "3": "Alpha-2 agonists.", "4": "Prostaglandin derivatives.", "5": NaN}, "question_id_specific": 200, "type": "OPHTHALMOLOGY", "year": 2018, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "pubmed23n1006_648", "title": "[Evaluation of the effect of prostaglandin analogues and a carbonic anhydrase inhibitor/β-blocker combination on the ocular blood flow in patients with primary open-angle glaucoma].", "score": 0.0170995670995671, "content": "To assess the effect of latanoprost and fixed combination of dorzolamide/timolol on ocular hemodynamics in patients with primary open-angle glaucoma (POAG). The study examined 34 patients (56 eyes) aged 51 to 69 years (average - 62.4±9.7 years) diagnosed with POAG in the initial (31 eyes) or advanced stage (25 eyes). Patients of the first group (20 patients, 36 eyes) were receiving latanoprost (Glauprost, Rompharm Company, Romania). The second group (14 patients, 20 eyes) was assigned to receive a fixed combination of dorzolamide/timolol (Dorzopt plus, Rompharm Company, Romania). Patient examination before and at 6 and 12 months included tonometry (ICare PRO), perimetry (Heidelberg Edge Perimeter), HRT and OCT of the optic nerve (Heidelberg Retina Tomograph 3 and Spectralis OCT2), as well as measurement of the density of surface (SVL) and deep (DVL) vascular plexus, and the Bruch's membrane opening minimum rim width (BMO-MRW). Additionally, we evaluated microcirculation in the choroid (MC) according to our original technique that uses Spectralis OCT2, and calculated rheographic index (RI) and stroke volume using transpalpebral rheoophthalmography. The decrease in IOP in the Latanoprost group was on average 27% and in the control group receiving timolol and dorzolamide - on average 22% and remained stable until the end of the study. The value of the area and volume of the disc rim band, BMO-MRW did not exhibit statistically significant changes in both groups by the 12th month of observation. A tendency to increase the RI from 51.5±24.5 to 62.2±19 (p=0.084) and the median of the index of microcirculation of the choroid (MC) from 16476 up to 23767 (p=0.062) in 1st group was observed. The study confirms the feasibility of using latanoprost and a fixed combination of dorzolamide and timolol in ocular hypotensive therapy of glaucoma thanks to their pronounced hypotensive effect. Patients were noted to have a tendency for improvement of ocular hemodynamics when using latanoprost."}, {"id": "pubmed23n0606_17148", "title": "Pharmacological management of primary open-angle glaucoma: second-line options and beyond.", "score": 0.016102551149280123, "content": "Glaucoma is one of the leading causes of blindness worldwide. Increased intraocular pressure (IOP) is considered to be the most important risk factor. Major outcome studies from recent years have shown that lowering IOP is beneficial in primary open-angle glaucoma and ocular hypertension. The introduction of new classes of IOP-lowering drugs (alpha(2)-adrenoceptor agonists, topical carbonic anhydrase inhibitors and hypotensive lipids) in the last decade has contributed to a change in the drug prescription pattern. Together with beta-adrenoceptor antagonists (beta-blockers), these drugs are now considered to be first-choice classes, giving ophthalmologists ample opportunities to choose from a broad spectrum of IOP-lowering drugs. The number of possible medical treatment combinations has increased likewise.We review medical treatment combinations of two, three or four drugs from the four major first-choice glaucoma drug classes and provide an overview of the scientific evidence for IOP efficacy of second-line medical options when first-line therapy has been effective but additional IOP lowering is necessary. A systematic search of the literature initially revealed 2729 publications. After a thorough selection process, 42 studies were found to be eligible for inclusion in the review. Publications were excluded if the primary endpoint of the study was not IOP or if glaucoma topics other than IOP lowering of drugs were studied. In addition, studies that reported results for monotherapies only were excluded. The vast majority of study arms reported on combinations of a beta-blocker with either a carbonic anhydrase inhibitor or a hypotensive lipid. For a number of treatment combinations no eligible studies were available or could be included.This review shows that combining drugs from the different first-choice classes results in an additional IOP decrease. The exact magnitude of this additional decrease and the patients to whom it applies remain unclear. In many studies, no information on IOP before the run-in phase was available. However, such data are important in order to determine whether patients with high untreated IOP or patients non-responsive to the run-in drug(s) were preferentially included. Another issue that hampers interpretation is the fact that the timepoints of measurements of IOP before and after adding a drug should be related to the peak and trough times of the drugs. Finally, differences between concomitant use and fixed combined use of drugs may have consequences for the interpretation of results."}, {"id": "wiki20220301en387_296", "title": "Glaucoma medication", "score": 0.015839300886029858, "content": "An initial reduction in the intraocular pressure of 20% from baseline is suggested. However, reduction of IOP to the target pressure range does not guarantee that progression will not occur. Therefore, the target pressure range needs to be constantly reassessed and changed as dictated by IOP fluctuations, optic nerve changes, and/or visual field progression. Medical uses Medications are divided into several groups based on chemical structure and pharmacologic action. Agents in common clinical use include: Prostaglandin analogs Parasympathomimetic (miotic) agents, including cholinergic and anticholinesterase agents Carbonic anhydrase inhibitors (oral and topical) Adrenergic antagonists (nonselective and selective Beta1-antagonists) Alpha 2 agonists Hyperosmotic agents When comparing people with primary open-angle glaucoma and ocular hypertension, medical intraocular pressure lowering treatment slowed down the progression of visual field loss. Comparison table"}, {"id": "pubmed23n0515_9578", "title": "Glaucoma and its treatment: a review.", "score": 0.015617403986387944, "content": "A review of glaucoma and its treatment is presented. Glaucoma is a common eye disease that can cause irreversible blindness if left undiagnosed and untreated. Glaucoma is a leading cause of blindness in the United States and other industrialized countries. In most cases, the symptoms of early-stage glaucoma are minimal or nonexistent. There are several different types of glaucoma, and they have been classically divided into the categories of primary or secondary open-angle or angle-closure glaucoma. Secondary forms of glaucoma are caused by various ocular or systemic diseases. Every available treatment to prevent progressive glaucomatous optic neuropathy has potential adverse effects and involves a certain amount of risk and financial expense. Conventional first-line treatment of glaucoma usually begins with the use of a topical selective or nonselective beta-blocker or a topical prostaglandin analog. Second-line drugs of choice include alpha-agonists and topical carbonic anhydrase inhibitors. Parasympathomimetic agents, most commonly pilocarpine, are considered third-line treatment options. For patients who do not respond to antiglaucoma medications, laser trabeculoplasty and incisional surgery are further methods that can be used to lower intraocular pressure. The results of clinical trials have reaffirmed the utility of antiglaucoma medications in slowing the progression of the disease. Glaucoma is a common eye disease that is usually associated with an elevated intraocular pressure. Treatment options for patients with glaucoma include medications, laser therapy, and incisional surgery. The risks and benefits of each type of treatment must be carefully considered to maximize the treatment's benefits while minimizing adverse effects."}, {"id": "pubmed23n0595_3469", "title": "The one-eye trial and fellow eye response to prostaglandin analogues.", "score": 0.015617403986387944, "content": "To determine if the intraocular pressure (IOP) reduction in one-eye trial correlates with the IOP reduction in the fellow eye when IOP variability during office hours is taken into account. This retrospective observational case series includes 50 high tension (primary open-angle glaucoma, exfoliative glaucoma and ocular hypertension; Group 1) and 21 normal tension (Group 2) glaucoma patients. Charts of bilateral open-angle glaucoma patients at Glaucoma Unit, School of Medicine, Adnan Menderes University who had pretreatment baseline office IOP measurements at 9:30, 11:30 and 15:30; were put on a one-eye trial with prostaglandin analogues, and then subsequently received the same medication in the fellow eye were reviewed. Pretreatment baseline office IOP measurements and reductions in the first and second eyes were compared to determine variability and correlation, respectively. Both groups showed significant variability in pretreatment baseline office IOP measurements. Peak IOP level was before noon in at least 70% of eyes in Group 1, 50% in Group 2. IOP dropped a mean of 8.8 +/- 4.8 mmHg (34%, P = < 0.0001) in Group 1 and 4.9 +/- 2.4 mmHg (30%, P = < 0.0001) in Group 2 during the one-eye trial. Following bilateral use of the medication a mean drop of 7.5 +/- 4.0 mmHg (31%, P = < 0.0001) in Group 1 and 2.9 +/- 1.9 mmHg (18%, P = < 0.0001) in Group 2 were obtained. The IOP reduction between fellow-eye pairs were significantly correlated in Group 1 (r = 0.541, P = < 0.0001) but not Group 2 (r =-0.310, P = 0.171). One-eye trial is a feasible method to determine the effectiveness of prostaglandin analogues and to predict the amount of IOP reduction in the fellow eye in patients with high tension glaucoma."}, {"id": "wiki20220301en040_16258", "title": "Eye drop", "score": 0.014196706720071207, "content": "Glaucoma Eye drops used in managing glaucoma help the eye's fluid to drain better and decrease the amount of fluid made by the eye which decreases eye pressure. They are classified by their active ingredient and they include: prostaglandin analogs, beta blockers, alpha agonists, and carbonic anhydrase inhibitors. There are also combination drugs available for those patients who require more than one type of medication. Mydriatic eye drops These make the eye's pupil widen to maximum, to let an optometrist have the best view inside the eyeball behind the iris. Afterwards in sunny weather they can cause dazzling and photophobia until the effect of the mydriatic has worn off."}, {"id": "wiki20220301en003_136762", "title": "Glaucoma", "score": 0.013900990099009901, "content": "Diagnosis is made by looking for cupping of the optic nerve. Prostaglandin agonists work by opening uveoscleral passageways. Beta-blockers, such as timolol, work by decreasing aqueous formation. Carbonic anhydrase inhibitors decrease bicarbonate formation from ciliary processes in the eye, thus decreasing the formation of aqueous humor. Parasympathetic analogs are drugs that work on the trabecular outflow by opening up the passageway and constricting the pupil. Alpha 2 agonists (brimonidine, apraclonidine) both decrease fluid production (via inhibition of AC) and increase drainage. Developmental glaucoma Developmental glaucoma (Q15.0) Primary congenital glaucoma Infantile glaucoma Glaucoma associated with hereditary or familial diseases"}, {"id": "pubmed23n0345_13409", "title": "Prostaglandin analogues in the treatment of glaucoma.", "score": 0.013634290925672594, "content": "Prostaglandin (PG) analogues are a new class of ocular hypotensive drugs that have been developed for the treatment of open angle glaucoma. Two of these drugs, latanoprost and unoprostone, are presently commercially available. Latanoprost was introduced in 1996 in the US and Europe. Presently it enjoys the most widespread use and is the most well documented drug of this group. It reduces the intraocular pressure (IOP) by a mechanism of action different from other drugs; namely by increasing the uveoscleral outflow. The aqueous inflow is not affected. The optimal dose regimen is one drop of 50 microg/ml once daily, which reduces the IOP by approximately 30% in patients with glaucoma. A more pronounced ocular hypotensive effect is demonstrated when latanoprost is combined with other glaucoma therapies, including beta-blockers, adrenergic and cholinergic agonists or carbonic anhydrase inhibitors. Latanoprost is well tolerated. The drug reaches a plasma concentration below that needed for stimulation of the FP-receptor, which may explain its favourable systemic tolerability profile. The major ocular adverse effect is increased iris pigmentation, which is due to increased synthesis of melanin in the melanocytes of the iris stroma. It is most frequently seen in green-brown eyes and it is probably permanent. A low frequency of cystoid macular oedema has also been reported, predominantly in predisposed eyes. Unoprostone was launched in Japan in 1994, but there is little experience with this drug outside the Japanese market and the documentation is more limited. Its main mechanism of action is on outflow, but this is not yet fully elucidated. The recommended dosage regimen is 1 drop of 1.2 mg/ml twice daily. No comparative studies in humans between the 2 drugs have yet been published."}, {"id": "pubmed23n0285_13228", "title": "[Efficacy and tolerability of 2 presentations of eyedrops combining carteolol 2% and pilocarpine 2% in primary open-angle glaucoma and simple ocular hypertension].", "score": 0.013306982872200264, "content": "The aim of the study was to compare 2 combinations of eye drops containing 2% carteolol and 2% pilocarpine: LCM 1010: ready to use eye drops CBS 341A: eye drops to be reconstituted (freeze-dried powder + solvent). Ninety-seven patients with primary open angle glaucoma or simple ocular hypertension were included in a randomized, double-blind multicentric study comparing 2 parallel groups of treatment. Intra-ocular pressure was greater than 21 mmHg with beta-blocker alone. One instillation of 2% carteolol-2% pilocarpine combination was given twice a day for one month. Before and after this treatment, intra-ocular pressure was measured at 9 am (12 hours after evening instillation) and at 11 am (2 hours after morning instillation). Both treatments reduced intra-ocular pressure by a comparable amount and there was no significant difference between groups at either measure: at 9 am: 2.11 +/- 2.39 mmHg (mean +/- SD) for LCM 1010 1.79 +/- 1.73 mmHg for CBS 341 A p = 0.25 at 11 am: 3.75 +/- 3.83 mmHg for LCM 1010 3.40 +/- 1.69 mmHg for CBS 341 A p = 0.42. Both eye drops were generally well tolerated. Efficacy and safety of ready to use eye drops 2% carteolol-2% pilocarpine combination proved to be comparable to that of eye drops to be reconstituted in the treatment of ocular hypertension poorly controlled by beta-blocker eye drops alone."}, {"id": "wiki20220301en596_17212", "title": "Bimatoprost/timolol", "score": 0.012872208436724566, "content": "Bimatoprost/timolol, sold under the brand name Ganfort, is a medication for the treatment of certain conditions involving high pressure in the eyes, specifically open angle glaucoma and ocular hypertension. It is available as eye drops. It was approved for medical use in the European Union in May 2006. Medical uses Bimatoprost/timolol is used for the treatment of open angle glaucoma or ocular hypertension in people for whom single-component eye drops such as prostaglandin analogs or beta blockers are insufficient. Contraindications Because of the timolol component, which is a beta blocker, the drops are contraindicated in people with lung problems such as asthma or severe chronic obstructive pulmonary disease, or with heart problems such as sinus bradycardia (slow heartbeat), sick sinus syndrome, sino-atrial block, or severe atrioventricular block."}, {"id": "wiki20220301en060_68339", "title": "Timolol", "score": 0.012748689219277454, "content": "In its eye drop form it is used to treat open-angle and, occasionally, secondary glaucoma. The mechanism of action of timolol is probably the reduction of the formation of aqueous humor in the ciliary body in the eye. It was the first beta blocker approved for topical use in treatment of glaucoma in the United States (1978). When used by itself, it depresses intraocular pressure (IOP) 18–34% below baseline within first few treatments. However, there are short-term escape and long-term drift effects in some people. That is, tolerance develops. It may reduce the extent of the diurnal IOP curve up to 50%. The IOP is higher during sleep. Efficacy of timolol in lowering IOP during the sleep period may be limited. It is a 5–10× more potent β-blocker than propranolol. Timolol is light-sensitive; it is usually preserved with 0.01% benzalkonium chloride (BAC), but also comes BAC-free. It can also be used in combination with pilocarpine, carbonic anhydrase inhibitors or prostaglandin analogs."}, {"id": "Pharmacology_Katzung_963", "title": "Pharmacology_Katzung", "score": 0.012413833391751372, "content": "is associated with acute and painful increases of pressure, which must be controlled on an emergency basis with drugs or prevented by surgical removal of part of the iris (iridectomy). The open-angle form of glaucoma is a chronic condition, and treatment is largely pharmacologic. Because intraocular pressure is a function of the balance between fluid input and drainage out of the globe, the strategies for the treatment of open-angle glaucoma fall into two classes: reduction of aqueous humor secretion and enhancement of aqueous outflow. Five general groups of drugs—cholinomimetics, α agonists, β blockers, prostaglandin F2α analogs, and diuretics—have been found to be useful in reducing intraocular pressure and can be related to these strategies as shown in Table 10–3. Of the five drug groups listed in Table 10–3, the prostaglandin analogs and the β blockers are the most popular. This popularity results from convenience (onceor twice-daily dosing) and relative lack of adverse effects"}, {"id": "wiki20220301en079_23203", "title": "Ocular hypertension", "score": 0.012404155653835082, "content": "Ocular hypertension is the presence of elevated fluid pressure inside the eye (intraocular pressure), usually with no optic nerve damage or visual field loss. For most individuals, the normal range of intraocular pressure is between 10 mmHg and 21 mmHg. Elevated intraocular pressure is an important risk factor for glaucoma. One study found that topical ocular hypotensive medication delays or prevents the onset of primary open-angle glaucoma. Accordingly, most individuals with consistently elevated intraocular pressures of greater than 21mmHg, particularly if they have other risk factors, are treated in an effort to prevent vision loss from glaucoma. Pathophysiology The pressure within the eye is maintained by the balance between the fluid that enters the eye through the ciliary body and the fluid that exits the eye through the trabecular meshwork."}, {"id": "InternalMed_Harrison_2337", "title": "InternalMed_Harrison", "score": 0.011587301587301587, "content": "Chronic angle-closure glaucoma and chronic open-angle glaucoma are usually asymptomatic. Only acute angle-closure glaucoma causes a red or painful eye, from abrupt elevation of intraocular pressure. In all forms of glaucoma, foveal acuity is spared until end-stage disease is reached. For these reasons, severe and irreversible damage can occur before either the patient or the physician recognizes the diagnosis. Screening of patients for glaucoma by noting the cup-to-disc ratio on ophthalmoscopy and by measuring intraocular pressure is vital. Glaucoma is treated with topical adrenergic agonists, cholinergic agonists, beta blockers, and prostaglandin analogues. Occasionally, systemic absorption of beta blocker from eyedrops can be sufficient to cause side effects of bradycardia, hypotension, heart block, bronchospasm, or depression. Topical or oral carbonic anhydrase inhibitors are used to lower intraocular pressure by reducing aqueous production. Laser treatment of the trabecular"}, {"id": "wiki20220301en221_36870", "title": "Brimonidine/timolol", "score": 0.011538461538461539, "content": "Brimonidine/timolol, sold under the brand name Combigan, is a fixed-dose combination medication eye drop indicated for the treatment of glaucoma. It is a combination of brimonidine (an α2 adrenergic agonist) and timolol (a β adrenergic blocker), in concentrations of 0.2% and 0.5% respectively. Both substances work by decreasing the synthesis of aqueous humor. Combigan is marketed and sold by Allergan. It has been approved by regulatory authorities in Europe, Canada, and the United States for reduction of intraocular pressure (IOP) in patients with glaucoma or ocular hypertension. Although clinical studies showed that the IOP-lowering effect was slightly greater (1-2 mmHg) with concomitant administration of 0.5% timolol twice daily and 0.2% brimonidine tartrate three times daily than with Combigan twice daily, the safety profile of latter was more favorable."}, {"id": "wiki20220301en112_51914", "title": "Brinzolamide", "score": 0.011059718367766471, "content": "Brinzolamide (trade names Azopt, Alcon Laboratories, Befardin, Fardi Medicals, ) is a carbonic anhydrase inhibitor used to lower intraocular pressure in patients with open-angle glaucoma or ocular hypertension. Brinzolamide was approved as a generic medication in the United States in November 2020. Chemistry Brinzolamide is a carbonic anhydrase inhibitor (specifically, carbonic anhydrase II). Carbonic anhydrase is found primarily in erythrocytes (but also in other tissues including the eye). It exists as a number of isoenzymes, the most active of which is carbonic anhydrase II (CA-II). Indications Use for the treatment of open-angle glaucoma and raised intraocular pressure due to either excess aqueous humor production or inadequate drainage of the humor via the trabecular meshwork."}, {"id": "article-35536_23", "title": "Steroid-Induced Glaucoma -- Treatment / Management", "score": 0.010562812449604903, "content": "Medical management : medical management of this condition is similar to that of primary open-angle glaucoma. The agents which may be used include beta-blockers, prostaglandin analogs, alpha-2 agonists, and carbonic anhydrase inhibitors. Beta-blockers tend to be the first-line agents in this condition. [45]"}, {"id": "pubmed23n0326_5888", "title": "Prognostic factors for hypotensive effects of isopropyl unoprostone in eyes with primary open-angle glaucoma.", "score": 0.010471204188481676, "content": "It has been reported that isopropyl unoprostone, a prostaglandin-related compound, has potent effects in lowering intraocular pressure and that its hypotensive effect is an increase of uveoscleral outflow. In the present study, we investigated the clinical characteristics of the hypotensive effects of this novel antiglaucoma drug in 115 primary open-angle glaucoma (POAG) eyes. The mean intraocular pressure (+/- standard deviation) before the addition of isopropyl unoprostone to the current regimens was 21.3 +/- 4.4 mmHg. The values at 1 month, 3 months, and 6 months after treatment were, respectively, 20.2 +/- 3.9 mmHg, 19.4 +/- 3.4 mmHg, and 18.4 +/- 2.5 mmHg. In POAG, the outflow pressure difference (delta OP), which is determined as (pretreatment pressure - posttreatment pressure)/(pretreatment pressure - 10) x 100 (%), was reduced by more than 20% in 36 (31%) of 115 eyes, 35 (36%) of 97 eyes, and 33 (53%) of 62 eyes, respectively at 1, 3, and 6 months. We defined the \"early success\" group as eyes with a significant reduction in delta OP (> or = 20%) at 1-month posttreatment. To identify the prognostic factors related to the significant reduction in intraocular pressure occurring after the administration of this drug, we carried out a statistical analysis by logistic regression analysis. Statistical analysis revealed significant prognostic factors: history of cataract surgery (P = 0.0084) and pretreatment pressure levels (P = 0.0105) at 1-month posttreatment. Also, further statistical analysis showed a significant influence of pretreatment pressure levels (P = 0.0010) at 3 months posttreatment. Our study shows an interindividual difference in the responsiveness of hypotensive effects on POAG eyes and some prognostic factors (history of cataract surgery and pretreatment pressure levels) prior to the use of this drug."}, {"id": "wiki20220301en009_184480", "title": "Medication", "score": 0.010449070750578288, "content": "For pain The main classes of painkillers are NSAIDs, opioids and local anesthetics. For consciousness (anesthetic drugs) Some anesthetics include benzodiazepines and barbiturates. For musculo-skeletal disorders The main categories of drugs for musculoskeletal disorders are: NSAIDs (including COX-2 selective inhibitors), muscle relaxants, neuromuscular drugs, and anticholinesterases. For the eye General: adrenergic neurone blocker, astringent. Diagnostic: topical anesthetics, sympathomimetics, parasympatholytics, mydriatics, cycloplegics. Antibacterial: antibiotics, topical antibiotics, sulfa drugs, aminoglycosides, fluoroquinolones. Antiviral drugs. Anti-fungal: imidazoles, polyenes. Anti-inflammatory: NSAIDs, corticosteroids. Anti-allergy: mast cell inhibitors. Anti-glaucoma: adrenergic agonists, beta-blockers, carbonic anhydrase inhibitors/hyperosmotics, cholinergics, miotics, parasympathomimetics, prostaglandin agonists/prostaglandin inhibitors, nitroglycerin."}, {"id": "wiki20220301en033_38421", "title": "Ciliary body", "score": 0.009947447447447447, "content": "Alpha-adrenergic agonists work by decreasing production of fluid and increasing drainage. Brimonidine and Apraclonidine are two commonly prescribes alpha agonists for glaucoma treatment. Alphagan P uses a purite preservative, which is better tolerated those who have allergic reactions than the older BAK preservative in other eye drops. Furthermore, less selective alpha agonists such as [epinephrine] may decrease the production of aqueous humor through vasoconstriction of the ciliary body (only for open-angle glaucoma). Carbonic anhydrase inhibitors also decrease fluid production. They are available as eye drops (Trusopt and Azopt) and pills (Diamox and Neptazane). This may be helpful if using more than one type of eye medication."}, {"id": "pubmed23n0994_10107", "title": "[Initial combination therapy for primary open-angle glaucoma].", "score": 0.009900990099009901, "content": "To study the efficacy and safety of initial combination therapy in patients with primary open-angle glaucoma (POAG). The study included 111 patients (161 eyes) with stages II-III b-c of POAG aged 46 to 89 years (mean age 70.8±8.5 years). In the test group, therapy began with a fixed combination of prostaglandin (PG) (latanoprost) and beta-blocker (BB) (timolol). In patients of the control group, POAG therapy began with administration of the analogue of prostaglandin (latanoprost). When the target IOP was not achieved, a second drug was added to the treatment - instillations of 0.5% solution of timolol maleate 2 times a day. In patients of the test group the treatment started with a combination of BB + PG, the target IOP in the 'worst' eyes was achieved in 33 cases (64%) in 1.5±0.6 visits, and in the 'best' eyes - in 17 cases (81%), in 1.2±0.4 visits. In the control group with a 'step-by-step' selection of antihypertensive therapy, the target IOP in the 'worst' eyes was achieved in 35 cases (59%), in 2.5±0.7 visits, and in the 'best' eyes - in 22 cases (76%), in 2.2±0.5 visits (p<0.05). In both study groups, there were no cases of drug withdrawal attributed to their side effects. Patients noted conjunctival hyperemia in 5% (4 eyes) and 7% (6 eyes) of cases in the test group and the control group, respectively (p>0.05). Hyperemia decreased significantly in 2 weeks of latanoprost administration or fixed combination and did not cause withdrawal. Initial combination therapy is possible in patients with newly diagnosed POAG in some clinical situations, namely in stages II-III POAG with high IOP, unavailability of regular observation of patients for various social and economic reasons, absence of somatic and local contraindications to individual components of the combined treatment, etc."}, {"id": "wiki20220301en112_52222", "title": "Dorzolamide", "score": 0.009847205990563997, "content": "Medical uses Dorzolamide hydrochloride is used to lower excessive intraocular pressure in open-angle glaucoma and ocular hypertension. This drug is able to cross the cornea, reach the ciliary body of the eye, and produce systemic effects on the carbonic anhydrase enzyme within the eye. Side effects Ocular stinging, burning, itching and bitter taste. It causes shallowing of the anterior chamber and leads to transient myopia. As a second generation carbonic anhydrase inhibitor, Dorzolamide avoids systemic effects associated with first generation carbonic anhydrase inhibitors such as Acetazolamide, Methazolamide, and Dichlorphenamide. Pharmacodynamics It lowers IOP by about 20%. Carbonic Anhydrase can convert H2CO3 into HCO3 (bicarbonate) and H+. The H+ is then exchanged for sodium (Na) which allows you to make aqueous humor. By blocking carbonic anhydrase, the Na/H exchanger can't work, which will decrease Na in the cell and prevent aqueous humor production."}, {"id": "pubmed23n0906_23906", "title": "[Ocular hypotensive effect of systemic beta-blockers in patients with primary glaucoma and arterial hypertension].", "score": 0.00980392156862745, "content": "to evaluate the ocular hypotensive efficacy of systemic beta-blockers in primary glaucoma patients suffering from arterial hypertension (AH). The study included 29 patients with POAG (58 eyes) aged from 47 to 83 years. Patients with stage I-III POAG received instillations of prostaglandin analogs and carbonic anhydrase inhibitors. All POAG patients also suffered from arterial hypertension and were prescribed selective beta-blockers (metoprolol, bisoprolol, or nebivalol) as monotherapy or as part of combination therapy (if the target arterial pressure had not been achieved under the initial treatment). After the start of oral beta-blockers therapy, the patients were re-examined at 2 and 4 weeks, 3 months, 6 months, and 1 year. A clinically significant reduction of IOP in the most seriously affected eye - by 3.3 mmHg (p<0.05), or 14% - occurred four weeks after the start of selective beta-blockers. Over three months of combination therapy, IOP in the 'worst' eye decreased by 4.4 mmHg (18.5%). At 1 year, IOP in the 'worst' eye was 6.2 mmHg (26%) lower than at baseline (p<0.05). Aged and senile patients with primary glaucoma usually suffer from polypathy (on average, they have 6.3±0.6 concurrent somatic diseases). To reduce the risk of polypharmacy and the frequency of side effects in the treatment of POAG and AH patients, it is advised that the treatment includes oral selective beta-blockers able to provide target levels of arterial pressure and IOP. In this study, oral beta-blockers in POAG and AH patients enabled IOP reduction as great as 18.5%-26% of baseline values over a 1-year follow-up period."}, {"id": "pubmed23n0838_25641", "title": "Carbonic anhydrase inhibitors as fourth drug in primary glaucomas: Is it worth it?", "score": 0.00980392156862745, "content": "To evaluate the effectiveness of carbonic anhydrase inhibitors as the fourth drug regarding intraocular pressure (IOP) control in patients with primary glaucomas. Single-centre, prospective study. Twenty-five eyes from 25 patients with primary glaucomas treated concomitantly with a topical prostaglandin analogue, a β-blocker, an α-adrenergic agonist, and a carbonic anhydrase inhibitor. Patients followed at the Federal University of São Paulo were enrolled from August to November 2013 and were initially submitted to an ophthalmologic examination where the IOP was measured at 8 am, 10 am, and 12 pm. Afterward, patients underwent a 15-day washout of the carbonic anhydrase inhibitor and had their IOP measured again. Most patients were female, white, and with a mean age of 66.4 ± 9.7 years. The removal of the fourth drug had a statistically significant effect on the IOP peak (increase of 1.20 mm Hg, p < 0.01) and mean (increase of 1.23 mm Hg, p < 0.01), but it did not interfere significantly with morning fluctuation of the IOP (p = 0.83). After discontinuation of the fourth drug, the IOP increased ≥2 mm Hg in 32% of the patients, and there was a significant increase of the IOP (defined as an IOP change ≥20%) in only 5 patients (20%). Age older than 60 years was associated with 20% of the documented IOP change (R(2) = 0.19, p = 0.03). The removal of a fourth medication does not appear to have a clinically significant impact on IOP control in most patients with glaucoma. However, 32% of the patients experienced an IOP increase ≥2 mm Hg, with age older than 60 years being the only significant predictive factor."}, {"id": "pubmed23n0393_21487", "title": "[New medical treatments of glaucoma. New strategies?].", "score": 0.009708737864077669, "content": "Several new classes of ocular hypotonia medications have recently become available. These include the topical treatments carbonic anhydrase inhibitors, the alpha-2 agonists, the prostaglandin analogues, and a fixed combination of beta-blockers and carbonic anhydrase inhibitors. With an equal or superior efficacy than beta-blockers, these new treatments must be reconsidered as first-line therapy in glaucoma. The long-term safety profile of antiglaucomatous drugs plays an important role in the new trends of medical treatment. These data, better known and detailed over time by clinical experience, are changing our habits and advancing therapeutic strategies for a more effective treatment, better suited to the individual, thus allowing the best quality of life possible."}, {"id": "pubmed23n0685_16152", "title": "Intraocular pressure changes in the contralateral eye after topical treatment: does an \"ophthalmotonic consensual reaction\" exist?", "score": 0.009615384615384616, "content": "The existence of \"ophthalmotonic consensual reaction,\" a contralateral change in intraocular pressure in the fellow eye induced by treatment of the first eye only, was suggested in 1924. Since then, the validity of this mechanism has been controversial. To assess intraocular pressure changes in the contralateral fellow eyes of patients treated with IOP-lowering medication in one eye, and investigate the existence of an ophthalmotonic consensual reaction. The study population included 38 patients with newly diagnosed bilateral ocular hypertension or early open angle glaucoma. One eye of each patient was randomly treated with one of five compounds: prostaglandin analogues, beta-blockers, alpha-2 agonists, carbonic anhidrase inhibitors and a combination therapy: dorzolamide hydrochloride-timolol maleate (Cosopt, Merck Sharpe & Dohme). The eye with the higher baseline IOP was selected to be the treated eye. After 3 weeks a masked examiner measured the IOP in both the treated and untreated eye. Mean IOP of the treated eyes at baseline was 26.1 +/- 4.2 mmHg and at follow-up 20.2 +/-2.9 mmHg, a reduction of IOP from baseline of -6 +/- 3.8 mmHg, a mean percent reduction of -22 +/- 10.1%. In the contralateral eyes, the mean IOP at baseline was 24.2 +/- 3 mmHg and 23.1 +/- 3.1 mmHg at follow-up; IOP reduction from baseline was -1.2 +/- 1.8 mmHg, or mean percent reduction -4.7 +/- 7.1%. A major contralateral IOP decrease was seen only in the beta-blockers and the combination (Cosopt) treatment groups (-6.1 +/- 8.3% and -12.3 +/- 8.3% mean percent reduction, respectively, P < 0.05). The contralateral eyes in the prostaglandin analogues, CAI or alpha2-agonist groups showed only a small change in IOP (-2.6 +/- 4.6%, -3.2 +/- 2.6%, +0.7 +/- 3.3%, mean percent reduction, respectively, P < 0.05). The existence of an ophthalmotonic consensual reaction was not supported."}, {"id": "pubmed23n0728_14323", "title": "Intraocular pressure-lowering combination therapies with prostaglandin analogues.", "score": 0.009615384615384616, "content": "Intraocular pressure (IOP) reduction is currently the only therapeutic approach demonstrated to preserve visual function in patients with glaucoma. The first line of glaucoma treatment consists of topical IOP-lowering medications, usually initiated as monotherapy. A significant proportion of patients require more than one medication to reach a target IOP at which optic nerve damage will not progress. As prostaglandin analogues (PGAs) are the most effective class for reducing IOP, one of the other commonly used classes (β-adrenoceptor antagonist [β-blocker], carbonic anhydrase inhibitor or α(2)-adrenoceptor agonist) is frequently combined with a PGA. In the last decade, the use of fixed combinations containing two medications in a single bottle has steadily increased. Fixed combinations have the potential to simplify the dosing regimen, increase patient adherence, avoid the washout effect of the second drop on the first medication instilled, decrease exposure to preservatives and, sometimes, reduce the cost of treatment. Clinical trials have evaluated PGA-based fixed combinations versus unfixed combinations (individual components administered concomitantly) or versus individual monotherapies; however, any advantage that the fixed combinations may have in terms of IOP-lowering efficacy is still debated. For these reasons, the PGA-based fixed combinations are not approved by regulatory authorities in some countries, such as the US. We review the published studies evaluating the efficacy and tolerability of the IOP-lowering unfixed and fixed combination therapies with PGAs. Regarding unfixed combinations, the review shows that α(2)-adrenergic agonists-PGA and carbonic anhydrase inhibitor-PGA combinations seem to be at least as effective at reducing IOP as the β-blocker-PGA combinations. As for the fixed combinations, the review shows that the three PGA-timolol fixed combinations are more effective than their component medications used separately as monotherapy and are better tolerated than the three respective prostaglandins. The three PGA-timolol fixed combinations are less effective at reducing IOP than the unfixed combinations but are better tolerated. The advantage of the fixed combinations in terms of patient adherence and persistence is supported by a very small number of studies and remains to be more accurately determined. Most studies, but not all, seem to show that PGA-timolol fixed combinations are more effective than other available β-blocker fixed combinations (dorzolamide-timolol fixed combinations) at reducing IOP and are similarly tolerated."}, {"id": "wiki20220301en626_24070", "title": "Uveitis–Glaucoma–Hyphema syndrome", "score": 0.0094875184695544, "content": "Treatment Initially, topical and systemic medication to control inflammation and raised IOP is appropriate, but the definitive treatment is an IOL reposition or exchange. Topical corticosteroids may be used to control anterior inflammation. Raised IOP can be lowered using topical and systemic anti-glaucoma medications such as prostaglandin analogs, beta-adrenergic antagonists, alpha-adrenergic agonists, and carbonic anhydrase inhibitors etc. History The UGH Syndrome was originally described by Ellingson in 1978 and classically included uveitis, glaucoma, and hyphaema in the setting of an anterior chamber IOL. References Glaucoma Disorders of iris and ciliary body Syndromes affecting the eyes"}, {"id": "pubmed23n1059_4922", "title": "Peripheral vascular disease - a new vascular disease associated with normal tension glaucoma: a case report.", "score": 0.009433962264150943, "content": "Normal-tension glaucoma is known as a multifactorial optic neuropathy. A number of lines of evidence suggested that vascular factors played a significant role in the development of normal-tension glaucoma. The mechanisms underlying the abnormal ocular blood flow in normal-tension glaucoma are still not clear. Peripheral vascular disease seems to be associated with glaucoma populations independent of other cardiovascular risk factors. We found this presentation, for the first time, to our knowledge, as another probable vascular abnormality related to our patient with normal-tension glaucoma, although it is necessary to confirm its pathological effect in future studies. Our patient was a 48-year-old Spanish man without any personal and family history of interest except for circulatory problems of the lower limbs with repetitive ulcers at the frontal and lateral aspects of his legs. His chief complaint was vision loss when he came to consult us. In exploration, his best corrected visual acuity was 20/20 in both eyes; initial intraocular pressure in the right eye was 14-16 mmHg and in the left eye was 16-18 mmHg, with a mild sclerosis of the lens in slit-lamp examination. No inflammation or pigmented lesion was detected in the anterior chamber. Open angle confirmed by Goldman four quadrants gonioscopy. Funduscopic examination revealed a vertical cup disc ratio of 0.6 in the right eye and 0.8 in the left eye. The patient's neuroretinal rim was normal in the right eye, and superior thinning in the left eye was determined. Examination of the patient's visual field showed inferior mild probable nasal scotoma in the right eye and an inferior deep arcuate scotoma defect in the left eye. His optical coherence tomography examination revealed thinning of the peripapillary nerve fiber layer thickness in the left eye and superior loss of macular retinal ganglion cells in the left eye. Normal intraocular pressure values were measured on the intraocular pressure curve without treatment (maximum value, 18-20 mmHg), discarding higher intraocular pressures measured out of office. Ultrasonic pachymetry measured 515/520 μm, and normal intraocular pressure measured with a PASCAL tonometer ruled out probable corneal biomechanical underestimations. The patient's polysomnography study was normal and excluded sleep apnea syndrome. The patient's serial mean blood pressure was normal, especially in the lower limbs (mean value, 125/70 mmHg), ruling out the possibility of systemic hypotension. Thyroidal and coagulation abnormalities, autoimmune disease, and inflammatory disease were excluded. Normal immunologic study and normal vascular biopsy were observed, as well as normal brain magnetic resonance imaging and a normal carotid vascular study. The primary diagnosis was moderate medium peripheral arterial disease in the lower limbs, which was confirmed by echography after ruling out other probable vascular abnormalities related to normal-tension glaucoma. After ruling out other systemic diseases and vascular abnormalities related to normal-tension glaucoma, we found peripheral arterial disease as a probable vascular abnormality related to normal-tension glaucoma in our patient. To our knowledge, this is the first time such a case has been reported. Thus, further research is needed to determine the relevance of these results to the general population."}, {"id": "pubmed23n0499_18274", "title": "How prostaglandins have changed the medical approach to glaucoma and its costs: an observational study of 2228 patients treated with glaucoma medications.", "score": 0.009433962264150943, "content": "The aim of the present study was to investigate how the medical treatment of glaucoma changed between 1997 and 2002, since the advent of prostaglandin derivatives, with regard to drug prescriptions and pharmaceutical costs. A study was made of medical prescriptions for 2228 patients with glaucoma and/or ocular hypertension, in order to investigate the following: (i) the antiglaucoma drugs most commonly prescribed in 1997 and 2002, and any differences between the drugs prescribed in these 2 years; (ii) the number of drugs used per patient in 1997 and 2002, respectively, and (iii) any increase in the prescribing of antiglaucoma drugs and their relative costs from 1997 to 2002. From 1997 to 2002 there was a sharp drop in the prescribing of beta-blockers (79% in 1997 and 55% in 2002). A marked increase in the use of prostaglandin derivatives (0% in 1997 and 18% in 2002) was registered and a marked increase in the prescribing of carbonic anhydrase inhibitors (5% in 1997 and 14% in 2002) was also noted. From 1997 to 2002 there was a trend towards drug addition rather than substitution, so that the number of drugs used per patient increased. The number of patients treated increased enormously (by 98%) from 1997 to 2002. The cost of medical therapy from 1997 to 2002 rose dramatically, with an increase of 148.9% per patient. The availability of prostaglandin derivatives has strongly influenced the medical approach to glaucoma. This class of drugs will soon become the type most commonly prescribed for patients with glaucoma and/or ocular hypertension. The increased number of treatments also suggests that the approach of ophthalmologists towards these diseases has changed. Ocular hypertension, as well as glaucoma, is now treated more aggressively. Given the increase in the prescription of prostaglandin derivatives, the pharmaceutical cost of treatment has risen dramatically."}, {"id": "wiki20220301en003_136799", "title": "Glaucoma", "score": 0.00939039408866995, "content": "still today - despite numerous new innovations in diagnostics - is considered the gold standard of determining this crucial pathogenic factor. In the late 20th century, further pathomechanisms beyond elevated IOP were discovered and became the subject of research like insufficient blood supply – often associated with low or irregular blood pressure – to the retina and optic nerve head. The first drug to reduce IOP, pilocarpine, was introduced in the 1870s; other major innovations in pharmacological glaucoma therapy were the introduction of beta blocker eye drops in the 1970s and of prostaglandin analogues and topical (locally administered) carbonic anhydrase inhibitors in the mid-1990s.. Early surgical techniques like iridectomy and fistulating methods have recently been supplemented by less invasive procedures like small implants, a range of options now widely called MIGS (micro-invasive glaucoma surgery)."}, {"id": "wiki20220301en111_14207", "title": "Carbonic anhydrase inhibitor", "score": 0.009259259259259259, "content": "Methazolamide is also a carbonic anhydrase inhibitor. It has a longer elimination half-life than acetazolamide and is less associated with adverse effects to the kidney. Dorzolamide is a sulfonamide and topical carbonic anhydrase II inhibitor. It is indicated for the reduction of elevated intraocular pressure in patients with open-angle glaucoma or ocular hypertension and who are insufficiently responsive to beta-blockers. Inhibition of carbonic anhydrase II in the ciliary processes of the eye decreases aqueous humor secretion, presumably by slowing the formation of bicarbonate ions with subsequent reduction in sodium and fluid transport."}]}}}} {"correct_option": 1, "explanations": {"1": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "2": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "3": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "4": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}, "5": {"exist": false, "char_ranges": [], "word_ranges": [], "text": ""}}, "full_answer": "Technically the patient is in a grade II hemorrhage according to the ATLS classification, in principle this means a loss of between 750 and 1500mL of blood and the indicated action is resuscitation with crystalloids; however, in the guidelines for the care of severe polytraumatized patients, the early use of blood products is increasingly recommended, especially in borderline cases like this one, and in young patients, who may not be able to translate the severity of the blood loss until it becomes much more significant. I would therefore hesitate between options 1 and 2.", "full_answer_no_ref": "Technically the patient is in a grade II hemorrhage according to the ATLS classification, in principle this means a loss of between 750 and 1500mL of blood and the indicated action is resuscitation with crystalloids; however, in the guidelines for the care of severe polytraumatized patients, the early use of blood products is increasingly recommended, especially in borderline cases like this one, and in young patients, who may not be able to translate the severity of the blood loss until it becomes much more significant. I would therefore hesitate between options 1 and 2.", "full_question": "An 18-year-old man arrives at the hospital emergency department transported by a basic life support unit. He has been hit by a car while riding a bicycle. On arrival he has a heart rate of 115 bpm, a blood pressure of 110/75 mmHg, a palpable radial pulse, a capillary refill of 2.5 seconds and a respiratory rate of 25 rpm. What do these data tell us:", "id": 484, "lang": "en", "options": {"1": "The patient has lost 15 to 30% of blood volume and requires volume replacement.", "2": "She has lost more than 30% of volemia and needs volume and blood replacement.", "3": "He has lost more than 40% of blood volume and will require surgical treatment.", "4": "Has lost more than 50% of volemia and requires volume and blood replacement.", "5": NaN}, "question_id_specific": 175, "type": "CRITICAL CARE", "year": 2020, "rag": {"clinical_case_options": {"MedCorp": {"RRF-2": [{"id": "Surgery_Schwartz_990", "title": "Surgery_Schwartz", "score": 0.017948717948717947, "content": "blood volume. However, substantial volumes of blood may be lost before the classic clinical manifestations of shock are evident. Thus, when a patient is significantly tachycardic or hypotensive, this represents both significant blood loss and physiologic decompensation. The clinical and physiologic response to hemorrhage has been classified according to the magnitude of volume loss. Loss of up to 15% of the circulat-ing volume (700 to 750 mL for a 70-kg patient) may produce little in terms of obvious symptoms, while loss of up to 30% of the circulating volume (1.5 L) may result in mild tachycar-dia, tachypnea, and anxiety. Hypotension, marked tachycardia (i.e., pulse greater than 110 to 120 bpm), and confusion may not be evident until more than 30% of the blood volume has been lost; loss of 40% of circulating volume (2 L) is immedi-ately life threatening and generally requires operative control of bleeding (Table 5-5). Young healthy patients with vigor-ous compensatory mechanisms may"}, {"id": "wiki20220301en031_37316", "title": "Hypovolemic shock", "score": 0.01694324362013666, "content": "Class 1: Volume loss up to 15% of total blood volume, approximately 750 mL. Heart rate is minimally elevated or normal. Typically, there is no change in blood pressure, pulse pressure, or respiratory rate. Class 2: Volume loss from 15% to 30% of total blood volume, from 750 mL to 1500 mL. Heart rate and respiratory rate become elevated (100 BPM to 120 BPM, 20 RR to 24 RR). Pulse pressure begins to narrow, but systolic blood pressure may be unchanged to slightly decreased. Class 3: Volume loss from 30% to 40% of total blood volume, from 1500 mL to 2000 mL. A significant drop in blood pressure and changes in mental status occur. Heart rate and respiratory rate are significantly elevated (more than 120 BPM). Urine output declines. Capillary refill is delayed."}, {"id": "wiki20220301en211_14093", "title": "Volume expander", "score": 0.0166532366371206, "content": "A volume expander is a type of intravenous therapy that has the function of providing volume for the circulatory system. It may be used for fluid replacement or during surgery to prevent nausea and vomiting after surgery. Physiology When blood is lost, the greatest immediate need is to stop further blood loss. The second greatest need is replacing the lost volume. This way remaining red blood cells can still oxygenate body tissue. Normal human blood has a significant excess oxygen transport capability, only used in cases of great physical exertion. Provided blood volume is maintained by volume expanders, a rested patient can safely tolerate very low hemoglobin levels, less than 1/3 that of a healthy person."}, {"id": "article-28971_8", "title": "Shock Resuscitation -- Indications", "score": 0.016533442645350824, "content": "In Class 2 shock, the patient has lost 750 mL to 1500 mL of blood (between 15% and 30% of total blood volume) and is beginning to become symptomatic. They may start to appear more pale or diaphoretic, with mild tachycardia (100-120 bpm), the respiratory rate may increase slightly (20-30 bpm), and their urine output may drop slightly (20-30 mL/hr). It is important to note that even outside the realm of shock management, urine output remains the single most important indicator for monitoring fluid status in a patient. Additionally, blood pressure cannot be adequately relied upon to detect the beginning of shock, as the body’s compensatory mechanisms will keep blood pressure typically within normal limits until up to 30% of total blood volume has already been lost. Finally, the Class 2 shock patient may demonstrate a slight decrease in pulse pressure, which may be the first sign of the body failing to compensate for the sudden blood loss."}, {"id": "pubmed23n0376_16392", "title": "Efficacy of acute normovolemic hemodilution assessed as a function of fraction of blood volume lost.", "score": 0.01642068873819971, "content": "It has been recommended that intraoperative acute normovolemic hemodilution (ANH) be considered for patients expected to experience surgical blood loss of 20% or more of their blood volume. Previous mathematical analyses have not evaluated the potential efficacy of ANH in terms of fraction of blood volume lost. Since decrease of oxygen-carrying capacity is a function of erythrocyte loss relative to blood volume, the purpose of this analysis was to provide an assessment of ANH applicable to all blood volumes and to determine whether this recommendation is appropriate. Equations were developed to describe the fractional blood volume loss (blood volume loss/blood volume; VReM/VBld) required to reduce hematocrit below a \"trigger\" hematocrit with maintenance of isovolemia. This is also the minimum fractional blood volume loss required for initial erythrocyte savings by any conservation technique. Equations were also developed to describe the fractional surgical blood volume loss for which ANH will obviate the need for transfusion of erythrocytes from any source other than those removed by ANH, and the fractional surgical blood volume loss required for ANH to save a defined volume of erythrocytes. Acute normovolemic hemodilution can extend the allowable fractional surgical blood loss before erythrocyte transfusion is required. The VRem/VBld required to initiate erythrocyte savings is approximately 0.5-0.9. The efficacy of ANH in terms of erythrocytes saved cannot be expressed as a function of the fractional blood volume lost alone. To save 1 unit of erythrocytes requires a fractional surgical blood loss of approximately 0.7-1.2 for the usual surgical patient when the transfusion trigger hematocrit is 0.18-0.21. This analysis suggests that surgical blood loss should be 0.50 or more for ANH to begin to \"save\" erythrocytes and 0.70 or more of the patient's blood volume for ANH to save 1 unit erythrocytes, for the usual surgical patient with an initial hematocrit of 0.32-0.36 and a transfusion \"trigger\" hematocrit (the value at which transfusion is initiated) of 0.18-0.21."}, {"id": "wiki20220301en211_14094", "title": "Volume expander", "score": 0.015268065268065269, "content": "The body detects the lower hemoglobin level, and compensatory mechanisms start up. The heart pumps more blood with each beat. Since the lost blood was replaced with a suitable fluid, the now diluted blood flows more easily, even in the small vessels. As a result of chemical changes, more oxygen is released to the tissues. These adaptations are so effective that if only half of the red blood cells remain, oxygen delivery may still be about 75 percent of normal. A patient at rest uses only 25 percent of the oxygen available in their blood. In extreme cases, patients have survived with a hemoglobin level of 2 g/dl, about 1/7 the normal, although levels this low are very dangerous."}, {"id": "wiki20220301en008_58142", "title": "Bleeding", "score": 0.013532322919888647, "content": "Class III Hemorrhage involves loss of 30-40% of circulating blood volume. The patient's blood pressure drops, the heart rate increases, peripheral hypoperfusion (shock) with diminished capillary refill occurs, and the mental status worsens. Fluid resuscitation with crystalloid and blood transfusion are usually necessary. Class IV Hemorrhage involves loss of >40% of circulating blood volume. The limit of the body's compensation is reached and aggressive resuscitation is required to prevent death."}, {"id": "article-28971_9", "title": "Shock Resuscitation -- Indications", "score": 0.013306543331564183, "content": "In Class 3 hemorrhagic shock, the patient has lost 1500-2000 mL of blood (30-40% total blood volume), they will be clearly symptomatic, confused and will be tachycardic (120-140 bpm), tachypneic (30-40 breaths/min), with blood pressure and pulse pressure decreased causing a drop in renal perfusion (urine output decreased to 5-15 mL/hr). Class 4 shock is the most severe case with acute blood loss of over 2000 mL (or over 40% total blood volume). The patient’s heart rate will be tachycardic, over 140 bpm, with nonpalpable or thready peripheral pulses. Their respiratory rate will have increased to over 35 breaths/min, and their blood pressure and pulse pressure will subsequently be severely decreased. Urine output will be negligible, and symptomatically they will be more lethargic with a likely altered mental status."}, {"id": "pubmed23n0056_11457", "title": "[The hemodynamic effects of various hydroxyethyl starch solutions in heart surgery patients].", "score": 0.013071895424836603, "content": "Blood conservation is gaining more and more interest because of the increasing risks involved in homologous blood transfusions. Acute normovolemic hemodilution (ANH) is becoming an established technique even in cardiac surgery patients. The \"optimal\" kind of volume replacement, however, is still controversial. Thus, this study was carried out to investigate the hemodynamic response of 6 different hydroxyethyl starch (HES) solutions as volume replacement. METHODS. In 60 patients undergoing elective aortocoronary bypass surgery, acute, preoperative hemodilution was performed (10 ml/kg) and HES with different concentrations, molecular weight, and substitution was infused according to a randomized sequence: 1. 6% HES 450,000/0.7; 2. 10% HES 200,000/0.5; 3. 3% HES 200,000/0.5%; 4. 6% HES 40,000/0.5; 5. 6% HES 200,000/0.5; 6. 6% HES 200,000/0.62. All patients were monitored using a new pulmonary artery catheter that allows measurement of the right ventricular ejection fraction (RVEF), right ventricular enddiastolic volume (RVEDV), and right ventricular end systolic volume (RVESV) in addition to standard hemodynamic parameters. RESULTS. Immediately after finishing ANH the typical hemodynamic changes of hemodilution (HD) were apparent (decrease in peripheral resistance and increase in cardiac index (CI]. All 6 solutions investigated were effective in hemodynamic stabilization (no changes in mean arterial pressure (MAP), filling pressures (PCP, RAP), or heart rate (HR]. Forty min after ANH, however (before beginning extracorporeal circulation (ECC], there were significant differences between the groups: in groups 3 and 4 the increase in CI had already disappeared, and SVI in group 3 was even lower than the baseline values (-8%). In the other groups, a higher CI level remained even 40 min after ANH, which was most pronounced in groups 2 (+40%) and 5 (+43%). Right ventricular performance was not changed by ANH (RVEF unchanged in all groups). Forty min after hemodilution RVEDVI (-8%) and RVESVI (-16%) decreased significantly only in group 4, whereas in the other groups these parameters were still elevated. The most pronounced positive fluid balance after the end of ECC was found in group 4 (+850 ml); in these patients paO2 decreased significantly (-150 mmHg). CONCLUSIONS. The guarantee of stable hemodynamic conditions is a prerequisite when performing ANH in coronary surgery patients. The different physiochemical attributes of various HES solutions seem to be important, thus influencing their hemodynamic response. In this study, low-concentration (3% HES 200/0.5) and low-molecular (6% HES 40/0.5) HES solutions were less effective in stabilizing hemodynamics until the beginning of ECC. Additionally, their negative influence on fluid balance during ECC, followed by a deterioration in pulmonary function led to the conclusion that other solutions are preferable; in particular, 10% HES seems to be of advantage in these situations."}, {"id": "wiki20220301en294_20685", "title": "Don't Cry for Me, Albuquerque", "score": 0.01138888888888889, "content": "Mary is rushed to the hospital; she has lost a large volume of blood, is unconscious and goes into cardiac arrest in the ambulance; though she is revived, she does not regain consciousness due to low blood volume. Marshall is the first to arrive as she is being wheeled into emergency, and he pleads with her not to die; after being barred from coming into the trauma room with her, he crumples to the floor in tears as Stan arrives, sobbing that she still wasn't breathing."}, {"id": "wiki20220301en020_27928", "title": "Hypovolemia", "score": 0.010389132783230285, "content": "Medical personnel should immediately supply emergency oxygen to increase efficiency of the patient's remaining blood supply. This intervention can be life-saving. The use of intravenous fluids (IVs) may help compensate for lost fluid volume, but IV fluids cannot carry oxygen the way blood does—however, researchers are developing blood substitutes that can. Infusing colloid or crystalloid IV fluids also dilutes clotting factors in the blood, increasing the risk of bleeding. Current best practice allow permissive hypotension in patients suffering from hypovolemic shock, both avoid overly diluting clotting factors and avoid artificially raising blood pressure to a point where it \"blows off\" clots that have formed. Hospital treatment Fluid replacement is beneficial in hypovolemia of stage 2, and is necessary in stage 3 and 4. See also the discussion of shock and the importance of treating reversible shock while it can still be countered."}, {"id": "wiki20220301en020_27927", "title": "Hypovolemia", "score": 0.010216601978214598, "content": "Stages Untreated hypovolemia can lead to shock (see also: hypovolemic shock). Most sources state that there are 4 stages of hypovolemia and subsequent shock; however, a number of other systems exist with as many as 6 stages. The 4 stages are sometimes known as the \"Tennis\" staging of hypovolemic shock, as the stages of blood loss (under 15% of volume, 15–30% of volume, 30–40% of volume and above 40% of volume) mimic the scores in a game of tennis: 15, 15–30, 30–40 and 40. It is basically the same as used in classifying bleeding by blood loss. The signs and symptoms of the major stages of hypovolemic shock include: Treatment Field care The most important step in treatment of hypovolemic shock is to identify and control the source of bleeding. Medical personnel should immediately supply emergency oxygen to increase efficiency of the patient's remaining blood supply. This intervention can be life-saving."}, {"id": "pubmed23n0250_2745", "title": "[Control of the ratio of the flow rate of the substitution fluid to the blood removal rate during preoperative normovolemic hemodilution].", "score": 0.009900990099009901, "content": "Twenty-one patients (mean age 46 +/- 13 years) due to undergo abdominal or ENT surgery, presumed to give rise to an important blood loss were included in this study. None had any contra-indication to the use of normovolaemic haemodilution (NH). Mean initial haematocrit was 40.3 +/- 1.8%. Their estimated total blood volume was 4,867 +/- 857 ml. The patients were anaesthetized with thiopentone, fentanyl, vecuronium or atracurium. Maintenance was carried out with isoflurane (0.5% during NH). Usual haemodynamic monitoring was used throughout. The required haematocrit was decided on before starting NH. The amount of blood to be removed was calculated with usual mathematical formulae. A radial artery cannula (n = 7), or a subclavian or femoral venous cannula (n = 14) was used to remove blood, which was collected within a bag containing CPC-adenine. Six % hydroxyethyl starch (Elohes) was given through a short venous cannula some distance from the first one. An antiparallel double line set in a roller pump was used to carry out the NH. A mean 1,341 +/- 405 ml of blood were withdrawn so as to reach a mean haematocrit of 30.6 +/- 2.4%. NH was completed within 17 +/- 6 min. No major haemodynamic changes occurred during the procedure. No significant differences were observed between expected and observed final haematocrits. There was no effect of the volume of blood withdrawn on the error of haematocrit prediction (0.5 +/- 0.3%). However, a higher rate of blood removal could increase this error. This easy-to-use device seems to provide fast and identical rates of blood removal and replacement. The expected haematocrit may thus be reached reliably, even if this must be checked for the sake of safety."}, {"id": "article-22750_10", "title": "Hemorrhage -- Pathophysiology", "score": 0.009862887429427354, "content": "In a healthy adult, there is an average of 4.5 to 5.5 liters or 70 to 90 ml/kg of blood circulating at any given time. Most adults can tolerate losing up to 14% of their blood volume without physical symptoms or deviations in their vital signs. The severity of hemorrhage is divided into a class system organized by the percent of blood volume loss. Up to 15% of blood volume loss classifies as a Class I hemorrhage. The patient is generally asymptomatic, and vital signs are within normal limits. Class II hemorrhage is a loss of 15 to 30% of total blood volume. Common manifestations include complaints of nausea and fatigue. On physical exam, there will be pallor and cooling of the extremities. Vital signs will start to deviate from normal, with tachycardia being the first vital sign to increase (100 to 120 beats per minute), followed by an increased respiratory rate (20-24 breaths per minute). Class III hemorrhage is 30 to 40% of total blood volume loss. Common manifestations include delayed capillary refill (greater than two seconds) and changes in mental status. Drastic blood pressure deviations (less than 90 mm Hg) are generally not seen until 30% of the blood volume is lost. Vital signs may reflect a systolic less than 90 mmHg or 20 to 30% of original measurement; HR is greater than 120; changes in mental status; and narrow pulse pressure (less than 25 mm Hg). These changes represent the body attempting to maintain perfusion to the vital organs by constricting peripheral blood vessels. Class IV hemorrhage is defined as greater than 40% of total blood volume loss. There is commonly a lack of urine output, absent peripheral pulses, and further deviations in vital signs. Severe hemorrhaging can lead to shock, which occurs when the blood loss becomes significant enough that it is unable to meet the oxygen demands of the tissue. Cellular aerobic metabolism shuts down, and anaerobic metabolism begins, leading to the production of lactic acid and, ultimately, metabolic acidosis. [10] The risk is very high for organ failure, coma, and death, absent the timely implementation of life-saving interventions."}, {"id": "wiki20220301en013_31903", "title": "Hemodynamics", "score": 0.009857635893011217, "content": "Hemodilution can be normovolemic, which implies the dilution of normal blood constituents by the use of expanders. During acute normovolemic hemodilution (ANH), blood subsequently lost during surgery contains proportionally fewer red blood cells per milliliter, thus minimizing intraoperative loss of the whole blood. Therefore, blood lost by the patient during surgery is not actually lost by the patient, for this volume is purified and redirected into the patient. On the other hand, hypervolemic hemodilution (HVH) uses acute preoperative volume expansion without any blood removal. In choosing a fluid, however, it must be assured that when mixed, the remaining blood behaves in the microcirculation as in the original blood fluid, retaining all its properties of viscosity. In presenting what volume of ANH should be applied one study suggests a mathematical model of ANH which calculates the maximum possible RCM savings using ANH, given the patients weight Hi and Hm."}, {"id": "pubmed23n0318_21764", "title": "[First aid and initial medical care in injuries of the large blood vessels].", "score": 0.00980392156862745, "content": "Nowhere as in emergency medicine results of a well done first aid can be more obvious as in the cases of big blood vessels injuries. Properly done first aid and first medical aid at big blood vessels injuries save the life of the patient. The following example is the case of MM patient The patient was received in the Emergency ward after an explosive injury of the big artery vessels and badly done first aid and be was without any signs of life. The patient was treated with: Adrenalin hydrochloride 1 ml intracardial Hydrocortisone amp a 1000 mg i.v. Sol Haemaccel 3 x 500, o ml i.v. Sol Hartman 3 x 500 ml i.v. Na-bicarbonate amp a 40 mg i.v. (2 x 20 mg) 9 bottles of fresh blood (300 ml each) After the intensive cardiopulmonal reanimation the patient after 3 hours he was in a stable general condition and sent to the operation theatre. For proper first aid the methods and activities are very simple but they should be known and implemented. In the opposite case a patient can lose all the blood and die in a hemorrhagic state."}, {"id": "wiki20220301en031_37317", "title": "Hypovolemic shock", "score": 0.009708737864077669, "content": "Class 4: Volume loss over 40% of total blood volume. Hypotension with narrow pulse pressure (less than 25 mmHg). Tachycardia becomes more pronounced (more than 120 BPM), and mental status becomes increasingly altered. Urine output is minimal or absent. Capillary refill is delayed."}, {"id": "pubmed23n0015_12344", "title": "[The demand for blood in open-heart surgery (author's transl)].", "score": 0.009708737864077669, "content": "Because of the continous increase of utilization of open heart surgery, whenever possible a more conservative use of blood is desirable. During 1977, 262 consecutive patients were operated upon using the hemodilution technique of cardiopulmonary bypass. Thirtyone operative deaths occurred (11.8%) and were not related to lack of blood administration. Mean hematocrit values were 32.4% two hours after surgery and between 31 and 32.5% in the following postoperative course. Hemoglobin concentration averaged from 9.9 to 10.4 g/100 ml during the first postoperative week. The estimated amount of blood transfused averaged 872 +/- 66 ml (+/- SE) per patient. Sixtyone patients (23%) didn't receive any transfusion. A conservative attitude towards blood administration is justified by transfusion related many risks and by the current shortage of blood."}, {"id": "pubmed23n0635_22558", "title": "[Compensation for circulating blood volume in fatal blood loss].", "score": 0.009615384615384616, "content": "Despite the extended potentials of modern medicine, noncompensated blood loss remains one of the leading causes of death in the able-bodied population all over the world. At present, there is no uniform policy of transfusion maintenance and intensive care in victims with severe decompensated blood loss. The purpose of the study was to assess the results of compensation for acute blood loss in victims with concomitant injury and wounds at various sites, which were attended by the loss of a circulating blood volume (CBV) with the use of new technologies. The authors retrospectively assessed the specific features of the course of anesthesia and compensation for blood loss in 66 male victims (mean age 37 +/- 1.5 years) with concomitant injury and various wounds complicated by acute CBV loss. CBV was compensated for by the currently available infusion media; globular blood volume deficit was restored via intraoperative instrumental reinfusion of autoblood and donor blood. The proposed procedure for infusion-transfusion therapy made it possible to stabilize the patient's condition and to perform emergency surgical treatment in victims with fatal blood loss. Total mortality in the chosen intensive care modality was 62.1%."}, {"id": "pubmed23n0315_16348", "title": "Volume therapy in the critically ill: is there a difference?", "score": 0.009523809523809525, "content": "There are still several concerns about the extensive and prolonged use of hydroxyethylstarch solution (HES) in critically ill patients. The effects of volume replacement with HES over 5 days on hemodynamics, laboratory data, and organ function were compared with volume therapy using human albumin (HA). Prospective, randomized study. Clinical investigations on a surgical intensive care unit (ICU) of a university hospital. 150 traumatized patients (injury severity score > 15) and 150 postoperative patients with sepsis were analyzed. Either 10% low-molecular weight HES (HES-trauma, n = 75; HES-sepsis, n = 75) or 20% HA (HA-trauma, n = 75; HA-sepsis, n = 75) was given for 5 days to maintain the pulmonary capillary wedge pressure (PCWP) between 12 and 15 torr. The entire management of therapy of the patients was performed by physicians who were not involved in the study and blinded to the infusion regimen. In addition to extensive cardiorespiratory monitoring, several routine laboratory parameters for assessing pulmonary, renal, hepatic, and coagulation function were analyzed from arterial blood samples on the day of admission to the ICU and on the day of sepsis diagnosis, respectively (\"baseline\" value) and daily over the following 5 days. Mortality during and after the study did not differ significantly between the infusion groups. There were also no differences between the incidence of pulmonary, renal, or hepatic failure in the two subgroups. Mean arterial pressure, heart rate, and PCWP were similar in both subgroups, whereas cardiac index, oxygen delivery index, oxygen consumption index, and the ratio between the partial pressure of oxygen in arterial blood and fractional inspired oxygen were higher in the HES- than in the HA-treated groups. Standard coagulation parameters did not differ, albumin concentration increased significantly in both HA groups, and lactate concentrations decreased only in the HES-sepsis patients (from 2.8 +/- 0.5 to 1.5 +/- 0.4 mg/dl). Volume replacement using albumin was significantly (p < 0.001) more costly than therapy with HES. Volume therapy with 10% HES for 5 days in the ICU patient showed no disadvantages compared with an infusion regimen using 20% albumin. Volume replacement using HES may even be associated with improved hemodynamics. HES appears to be a valuable and significantly cheaper alternative to albumin--even for prolonged volume therapy in the critically ill patient."}, {"id": "pubmed23n0046_23193", "title": "[A case of acute right ventricular infarction and life-saving right ventricular assistance following emergency coronary revascularization and resection of a left ventricular aneurysm--discussion of indication and proper assist flow volume].", "score": 0.009523809523809525, "content": "Right ventricular assistance (RVA) using centrifugal pump in combination with IABP was used to treat a patient who was difficult to wean from a cardiopulmonary bypass following emergency coronary revascularization and resection of a ventricular aneurysm performed to treat acute right ventricular infarction due to a PTCA complication. After 131 hours of RVA at 3.2 to 4.8 l/min, it was possible to remove the pump. No heparin was administered during this time, changing the pump head twice, was used for 64 and 50 hour period, no thrombi were detected either time. After being weaned from RVA, the patient developed severe respiratory dysfunction, but on the 10th postoperative day (POD) IABP was weaned, and on the 13th POD the artificial respirator was withdrawn. The results of the postoperative cardiac catheterization were favorable, the patient was discharged on the 57th POD, and has returned to society at the present time. The indications for RVA include a central venous pressure > 20 mmHg and a cardiac index < 1.8 l/min/m2, and tissue perfusion pressure and general preoperative condition should severe as guides. The higher the assisted flow volume the more efficacious in relieving ventricular load, but, since there is a limit to how much the left ventricle and lungs can withstand, it should not exceed levels which ensure the maintainance of cardiac output and tissue perfusion pressure."}, {"id": "pubmed23n0092_7003", "title": "[Preoperative normvolemic hemodilution in heart surgery. Pulmonary changes with the use of new technics].", "score": 0.009433962264150943, "content": "Interest in preoperative hemodilution (HD) has intensified perceptibly again, because of the increasing risk of adverse reactions to donor blood. However, in coronary surgery patients the use of HD is still the subject of controversy, as it may possibly influence organ function and especially lung water content. New techniques, including membrane oxygenation, extracorporeal circulation (ECC) with only \"partial\" bypass due to 2-stage cannulation, and hemoconcentration with cell separators, have significantly modified patient management in the field of cardiac surgery. Therefore, the influence of moderate hemodilution (12 ml/kg) on extravascular lung water (EVLW) was investigated under these conditions in 45 patients with coronary artery disease. Volume replacement was performed either with hydroxyethyl starch solution (HD-HES group, n = 15, ratio of replacement 1:1) or with Ringer's lactate (HD-RL-group, n = 15, ratio 2.5:1); 15 patients not subjected to HD served as controls. ECC was carried out with membrane oxygenators only in partial bypass. Both during and after ECC, blood was concentrated by means of a cell-saving system allowing separation and reinfusion of the red cells while the plasma is discarded. EVLW was measured using a double-indicator dilution technique with indocyanine green. Starting from comparable baseline values, EVLW was not significantly changed by hemodilution. After ECC, however, the HD-RL group showed a significant increase in lung water content (means: +2.49 ml/kg equal 42.6%), whereas this was not significantly changed in the other groups. By 5 h after ECC, the lung water content had returned to baseline values and no more differences could be observed between the groups.(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en082_15110", "title": "Blunt trauma", "score": 0.009345794392523364, "content": "Recently, criteria have been defined that might allow patients with blunt abdominal trauma to be discharged safely without further evaluation. The characteristics of such patients include: absence of intoxication no evidence of lowered blood pressure or raised pulse rate no abdominal pain or tenderness no blood in the urine. To be considered low risk, patients would need to meet all low-risk criteria. Treatment When blunt trauma is significant enough to require evaluation by a healthcare provider, treatment is typically aimed at treating life-threatening injuries, which requires ensuring the patient is able to breathe and preventing ongoing blood loss. If there is evidence that the patient has lost blood, one or more intravenous lines may be placed and crystalloid solutions and/or blood will be administered at rates sufficient to maintain the circulation. Some patients may require a surgical operation called an exploratory laparotomy to repair internal injuries."}, {"id": "pubmed23n0052_5250", "title": "[A volume-variable reservoir-pump for a manually operated cardiopulmonary bypass circuit for emergency].", "score": 0.009345794392523364, "content": "We developed a manually operated portable cardiopulmonary bypass circuit for resuscitation. The circuit is composed of, in turn, a venous drainage catheter, one-way valve, self-inflating reservoir, one-way valve, artificial lung, and an arterial catheter. These components are interlocked with conducting tubes with quick connectors. The priming volume of the circuit is about 300 ml including the self-inflating reservoir of 120 ml in capacity. For a patient with small stature, stroke volume is easily controlled by changing manual compression of the reservoir, but dilution of circulating blood with the priming solution is inevitable. For a controllable reduction of the reservoir volume, we incorporated a thin-walled balloon, which is inflatable from the outside, into the room of the reservoir. If the balloon is inflated with some amount of liquid, the same volume of functional capacity of the reservoir is lost. Thus the reservoir volume is adjusted, the hemodilution with a priming solution is minimized, and an excessive stroke volume with an inadvertent compression of the reservoir-pump is prevented as well. This innovation will make our standard size bypass circuit applicable to almost all patients, except for a newborn or infant who requires a special size of bypass circuit, and improve the survival rate of cardiopulmonary resuscitation."}, {"id": "pubmed23n0081_3362", "title": "[Comparison of volume substitutes 5 percent human albumin and 6 percent hydroxyethyl starch (40,000/0.5) in pediatric anesthesia].", "score": 0.009259259259259259, "content": "Human albumin 5% (HA), frequently used in paediatric anaesthesia as a human plasma substitute, could be replaced by hydroxyethyl starch 6% (HES) 40,000/0.5 provided its use would not entail any disadvantages but rather advantages instead. This problem was studied by examining 30 children (mean body weight 32.5 kg) in general anaesthesia. During about 3 hours of surgery the patients lost up to 15 per cent (approx. 400 ml) of blood volume. In a randomised study the blood loss was compensated either via HA or HES with 14 ml/kg body weight each, respectively. Blood pressure and pulse rate remained within the normal range. Haemodilution was represented by a drop in the erythrocyte count to about 3 million/microliter, haematocrit (HCT) to about 30% and haemoglobin to approx. 10 g/100 ml. Slight metabolic acidosis (Bodansky unit = -4 mmol/l) was found to be statistically not significant (= n.s.) with both methods, as was the difference in serum albumin and during serum electrophoresis. Serum [Na+] was reduced in those children who had been treated with HES, to 137.33 +/- 33.30 mmol/l; however, in those children who received HA with low sodium content (statistically significant difference = s.s.) the corresponding level was 134.15 +/- 2.36 mmol/l. Serum creatinine rose in each case from 60 to 80 mmol/l (s.s.), renal function being slightly impaired probably due to the anaesthesia and surgery. The value according to Quick's test and the partial thromboplastin time (PTT) remained in the normal range both with HA and HES treatment (in each case over 70% and below 25s, respectively).(ABSTRACT TRUNCATED AT 250 WORDS)"}, {"id": "wiki20220301en013_31909", "title": "Hemodynamics", "score": 0.009259259259259259, "content": "For example, if Hi is 0.30 or less it is not possible to save a red cell mass equivalent to two units of homologous PRBC even if the patient is hemodiluted to an Hm of 0.15. That is because from the RCM equation the patient RCM falls short from the equation giving above. If Hi is 0.40 one must remove at least 7.5 units of blood during ANH, resulting in an Hm of 0.20 to save two units equivalence. Clearly, the greater the Hi and the greater the number of units removed during hemodilution, the more effective ANH is for preventing homologous blood transfusion. The model here is designed to allow doctors to determine where ANH may be beneficial for a patient based on their knowledge of the Hi, the potential for SBL, and an estimate of the Hm. Though the model used a 70 kg patient, the result can be applied to any patient. To apply these result to any body weight, any of the values BLs, BLH and ANHH or PRBC given in the table need to be multiplied by the factor we will call T"}, {"id": "wiki20220301en011_140983", "title": "Venipuncture", "score": 0.00909090909090909, "content": "The volume of the blood sample collection is very important in experimental animals. All nonterminal blood collection without replacement of fluids is limited up to 10% of total circulating blood volume in healthy, normal, adult animals on a single occasion and collection may be repeated after three to four weeks. In case repeated blood samples are required at short intervals, a maximum of 0.6 ml/kg/day or 1.0% of an animal's total blood volume can be removed every 24 hours. The estimated blood volume in adult animals is 55 to 70 ml/kg body weight. Care should be taken for older and obese animals. If blood collection volume exceeds more than 10% of total blood volume, fluid replacement may be required. Lactated Ringer's solution (LRS) is recommended as the best fluid replacement by National Institutes of Health (NIH). If the volume of blood collection exceeds more than 30% of the total circulatory blood volume, adequate care should be taken so that the animal does not suffer from"}, {"id": "pubmed23n0826_18468", "title": "[Not Available].", "score": 0.00909090909090909, "content": "Bulb suction drains have long been used in various surgical procedures. The purpose of this study was to evaluate 4 commonly used bulb suction devices in vitro to explore the nonlinear changes in draining ability and efficiency along with the conformation changes of the device throughout the draining processes. Under a designed simulated scenario using pure water as the desired draining substance, the relative function of the J-VAC 100 cc (JV100) (Ethicon, Inc, Somerville, USA); the EVACUATOR 125cc (EP125) (Pacific Hospital Supply, Taiwan); the Bulb Reservoir 150 cc (BH150) (Hosmed, Inc, Miami, FL); and the HemoVac 400 cc (HV400) (Zimmer, Inc, Warsaw, IN) drains were compared. The maximum collection capacities and the dead space at maximum compression of each bulb drain were recorded and compared. The collected fluid weight was recorded along with time, and collection speeds were calculated and compared. The maximum collected weight of the 4 drains were 110.07 ± 0.54 g (JV100), 122.7 ± 06.51 g (EP125), 140.8 ± 03.78 g (BH150), and 335.07 ± 04.24 g (HV400). The dead spaces under maximum compression were 15.63 ± 01.32 ml, 19.80 ± 03.37 ml, 34.23 ± 06.77 ml, and 82.83 ± 05.51 ml, respectively. The collecting speed-volume curves were generated from the authors' tested devices. Although slightly different individually, typical characteristics, such as tendency to reach maximum collection speed at the very beginning of the collection phase; rapid decline to about 65% of peak collection speed when approximately 30% of the total collection volume had been achieved; and inefficient collection speed in the later collection phase were noted. Among all the bulb drains tested in this study, all of them performed well in vitro. Although using bulb drains continues to be an effective and economic draining method after operation, clinicians should be aware of the nonlinear features of suction efficiency during the drainage process to avoid unexpected function deterioration. ."}, {"id": "wiki20220301en463_30839", "title": "Blood pressure measurement", "score": 0.009009009009009009, "content": "Palpation A minimum systolic value can be roughly estimated by palpation, most often used in emergency situations, but should be used with caution. It has been estimated that, using 50% percentiles, carotid, femoral and radial pulses are present in patients with a systolic blood pressure > 70 mmHg, carotid and femoral pulses alone in patients with systolic blood pressure of > 50 mmHg, and only a carotid pulse in patients with a systolic blood pressure of > 40 mmHg. A more accurate value of systolic blood pressure can be obtained with a sphygmomanometer and palpating the radial pulse. Methods using constitutive models have been proposed to measure blood pressure from radial artery pulse. The diastolic blood pressure cannot be estimated by this method. The American Heart Association recommends that palpation be used to get an estimate before using the auscultatory method. Auscultatory"}, {"id": "pubmed23n0635_15807", "title": "One thousand dollar assist heart pump for patients from developing countries.", "score": 0.009009009009009009, "content": "In spite of continuous improvements in device design and applications, the profound use of heart pump has been limited because of its high price. The available clinically applied heart pump costs mostly about 100 thousands US Dollars. The author has since long tried to develop a heart pump costing only 1000 Dollars for recovery or bridge to heart transplantation therapies. The device is a radially driven centrifugal pump with a brush-less DC motor and a streamlined impeller. Its bearing is rolling bearing using 4 to 6 needles, manufactured by special wear-proof polythene with super-high-molecular weight, thus the service life achieves more than 10 years. To avoid thrombus formation, a special purge system is introduced to the bearing, allowing the saline with heparin to be infused through the bearing into the pump. The bearing, therefore, keeps working in the saline, and absolutely no thrombus will be formed along the bearing. Animal experiments demonstrated that a 30 mL fluid infusion per hour is enough to prevent thrombus formation. With these improvements, the impeller pump has continuously run for 14 months in the laboratory, and no bearing wear can be measured. The device, weighing 150 g, is fully implantable, consumes approximately 9.6 W, delivers a 9Lmin-1 blood flow against a 120 mmHg mean pressure, and reaches a highest total efficiency of 24.7% for the motor (including the controller) and the pump. The device has been used in animal experiments together with an American artificial lung for more than one month in the University of Texas and also in human trials in the Taiwan University."}, {"id": "Surgery_Schwartz_1336", "title": "Surgery_Schwartz", "score": 0.008942014615695347, "content": "response, and eye opening. Scores range from 3 (the lowest) to 15 (normal).Table 7-4Signs and symptoms of advancing stages of hemorrhagic shockCLASS ICLASS IICLASS IIICLASS IVBlood loss (mL)Up to 750750–15001500–2000>2000Blood loss (% BV)Up to 15%15%–30%30%–40%>40%Pulse rate<100>100>120>140Blood pressureNormalNormalDecreasedDecreasedPulse pressure (mmHg)Normal or increasedDecreasedDecreasedDecreasedRespiratory rate14–20>20–3030–40>35Urine output (mL/h)>30>20–305–15NegligibleCNS/mental statusSlightly anxiousMildly anxiousAnxious and confusedConfused and lethargicBV = blood volume; CNS = central nervous system.Brunicardi_Ch07_p0183-p0250.indd 19210/12/18 6:17 PM 193TRAUMACHAPTER 7patients have a progressive increase in circulating blood volume over gestation; therefore, they must lose a relatively larger vol-ume of blood before manifesting signs and symptoms of hypo-volemia (see “Special Populations”).Based on the initial response to fluid resuscitation, hypo-volemic injured"}, {"id": "wiki20220301en056_29748", "title": "Hemothorax", "score": 0.008928571428571428, "content": "When a hemothorax occurs, blood enters the pleural cavity. The blood loss from the circulation has several effects. Firstly, as blood builds up within the pleural cavity, it begins to interfere with the normal movement of the lungs, preventing one or both lungs from fully expanding and thereby interfering with the normal transfer of oxygen and carbon dioxide to and from the blood. Secondly, blood that has been lost into the pleural cavity can no longer be circulated. Hemothoraces can lead to significant blood loss – each half of the thorax can hold more than 1500 milliliters of blood, representing more than 25% of an average adult's total blood volume. The body may struggle to cope with this blood loss, and tries to compensate by maintaining blood pressure by forcing the heart to pump harder and faster, and by squeezing or constricting small blood vessels in the arms and legs. These compensatory mechanisms can be recognised by a rapid resting heart rate and cool fingers and toes."}]}}}}