shukoor65/lab-rag-model huggingface dataset:
Hydrops fetalis in the McKusick-Kaufman syndrome: a case report. McKusick-Kaufman syndrome is an autosomal recessive multiple malformation syndrome characterized by hydrometrocolpos and polydactyly. We report on a patient with McKusick-Kaufman syndrome and severe hydrops. This case illustrates the necessity of genetic evaluations for all patients with unexplained hydrops.
Monoclonal antibody YB5.B8 identifies the human c-kit protein product. The c-kit proto-oncogene encodes a 145- to 160-Kd transmembrane tyrosine kinase, which is a member of the platelet-derived growth factor receptor family and is allelic with the murine white spotting locus (W). W mutations affect several aspects of hematopoiesis, most notably erythroid progenitors and mast cells. A monoclonal antibody, YB5.B8, had been raised against the leukemic blasts of a patient with M1-type acute myelocytic leukemia (AML) and it precipitates a 150-Kd cell surface glycoprotein from leukemic cells. The YB5.B8 epitope is expressed on mast cells, on up to 3% of normal mononuclear bone marrow cells, and it identifies a sub-group of AML patients with a poor prognosis. In view of similarities noted between the cell surface antigen identified by YB5.B8 and the c-kit protein product, we performed experiments to determine whether they are identical. c-kit RNA expression in the cell lines HEL (human erythroleukemia) and A172 (glioblastoma) was shown to parallel the expression of the YB5.B8 epitope in these lines as measured by flow cytometry. Immunoprecipitation analysis with anti-kit serum and YB5.B8 antibody indicated that the two antibodies identified proteins of identical size in HEL (155 Kd) and A172 (145 Kd) cells, and sequential immunoprecipitations with the kit and the YB5.B8 antibodies demonstrated that the two antibodies recognize the same molecule. The proteins identified by both the anti-kit and YB5.B8 antibodies displayed in vitro autophosphorylation activity in immune complex kinase assays. In addition, YB5.B8 was able to inhibit the binding of the kit ligand to HEL cells. These studies provide evidence that the YB5.B8 antigen and the c-kit protein product are identical and raise certain hypotheses regarding the role of c-kit in AML.
Chronic periaortitis presenting as common bile duct obstruction. The case of a 67 year old woman is reported who presented with cholestatic jaundice and was found to have, in addition, an inflammatory abdominal aortic aneurysm. Only at necropsy did histopathology show chronic periaortitis as the aetiology of a pancreatic head mass which, during life, mimicked a pancreatic neoplasm obstructing the bile and pancreatic ducts.
Subependymal giant cell astrocytoma: a clinical, pathological, and flow cytometric study. Of 345 patients with tuberous sclerosis complex evaluated at the Mayo Clinic from 1950 to 1989, 23 were identified as having brain tumors. In 20 of the 23, histological or clinical evidence showed the tumor to be a subependymal giant cell astrocytoma. A search of the Mayo Clinic tissue registry yielded 73 giant cell-containing astrocytomas and intraventricular gliomas exclusive of ependymomas. Reexamination revealed no further examples of subependymal giant cell astrocytoma in patients without features of the tuberous sclerosis complex. Considerable histological variation was observed in the 15 subependymal giant cell astrocytomas subjected to critical microscopic review. It is of note that no correlation was noted between either the histological features, such as atypia, mitoses, endothelial proliferations, necrosis, or the flow cytometric characteristics and the clinical course or the survival time of the patients.
Drivers with untreated sleep apnea. A cause of death and serious injury. Three patients with untreated sleep apnea fell asleep while driving and caused serious automobile accidents. One person died, another became permanently paraplegic, and the three patients with sleep apnea were seriously injured in these crashes. This sequela of sleep apnea is not surprising, since subjects with sleep apnea may be poor drivers with a high accident rate and a high incidence of "near-miss" vehicular incidents. Because drivers with untreated sleep apnea may cause a large number of preventable automobile accidents, physicians have specific duties involving these drivers. First, physicians must try to identify impaired drivers with sleep apnea before they have an accident; routinely asking patients about loud snoring and hypersomnolence may help identify these impaired drivers. Second, physicians must consider the diagnosis of sleep apnea when examining patients who fall asleep while driving. Next, physicians must warn their patients with sleep apnea about the risks of driving with untreated sleep apnea. Finally, physicians must treat any seriously impaired driver with sleep apnea and keep these patients from driving until they can receive successful treatment.
Chronic Cryptosporidium parvum infections in congenitally immunodeficient SCID and nude mice. Severe combined immune deficient (SCID) and athymic National Institutes of Health (NIH)-III (bg/nu/xid) mice were evaluated for susceptibility to Cryptosporidium parvum infections as neonates and as adults after challenge with inocula of 5 x 10(4) and 1 x 10(6) oocysts/mouse, respectively. SCID and NIH-III nude mice developed chronic infections that persisted over 12 weeks. Cryptosporidial organisms were observed in the small and large intestines of all mice. Colonization of the gallbladder and hepatobiliary duct epithelium occurred in a majority of the NIH-III nude mice and a smaller number of the SCID mice. Severe infections in both mouse strains were associated with clinical deterioration followed by death. Signs in these mice included dehydration, icterus, and emaciation. Deaths apparently resulted from hepatic dysfunction. Control mice experienced transient infections with no clinical signs. Immunodeficient mice should prove useful in studies of disease pathogenesis and in evaluating potential anticryptosporidial agents.
Basaloid monomorphic adenomas. Monomorphic adenomas can be divided into basaloid and nonbasaloid type. With the exception of the canalicular adenoma, the basaloid monomorphic adenomas are preponderantly tumors of the major salivary glands, most often the parotid gland. The dermal analogue monomorphic adenomas, so named because of their histologic similarity to eccrine tumors of the skin, are a unique subset of the basaloid adenomas. They are often multifocal, recur frequently, may undergo malignant change, and can be part of a salivary-cutaneous tumor diathesis.
Intratumoral DNA content variability. A study of non-small cell lung cancer. Twenty-eight resected primary non-small cell lung carcinomas were studied for intratumoral DNA variability by flow cytometry (FCM). Three separate tissue specimens from each resected tumor were divided equally for FCM analysis and histologic evaluation. FCM analysis also was performed on fine-needle aspirates (FNAs) from the center of the resected tumor. Histologically, there were 8 squamous carcinomas, 19 adenocarcinomas, and 1 large cell carcinoma. Twenty-three tumors (82%) were aneuploid, and 5 (18%) were diploid. The DNA index in aneuploid neoplasms ranged from 0.91 to 3.30 (mean, 1.64). All 5 diploid and 19 (83%) of the (23) aneuploid neoplasms manifested intratumoral DNA stability. Four (17%) of the aneuploid tumors showed regional DNA heterogeneity expressed as additional stemlines in at least one sample. The FNA yield was sufficient in 21 cases and inadequate for complete analysis in 7 cases. In general, good correlation between FNA and tissue analysis was obtained. However, in three of the aneuploid neoplasms, FNA materials did not reveal an additional nondominant stemline, as noted in the tissue specimens. The authors attribute this finding to a dilutional factor in the aspiration material. The authors conclude that most non-small cell lung carcinomas express DNA stability; FNA provides adequate cellular material for FCM in most cases.
Health risks of foreign travel. Preparing adults for jaunts abroad. Preparing adult travelers for journeys abroad can be challenging and rewarding. Prevention is the cornerstone of a safe, enjoyable trip. Patient education and commonsense precautions may well prevent infection or disease. Prophylaxis for diarrhea and malaria could save one day of illness or inconvenience on an expensive trip or may save a traveler's life. And the Loa loa worm? The nurse fortunately waited until it crawled from under her cornea. Then it was gently teased from under the bulbar conjunctiva.
Diagnosis and treatment of muscle contraction (tension-type) headaches. Acute tension-type headache is a very common condition that rarely is a problem in treatment. Chronic tension-type headache, however, is often a difficult therapeutic problem. The pathogenesis is not well understood, but both peripheral muscle contraction and central pain-modulating systems are probably involved. Therapy usually works best when multiple techniques are used, including pharmacologic, psychological, and physiologic modalities.
Increased cisplatin tissue levels with prolonged arterial infusion in the rat. Prolonged arterial infusions of cisplatin (DDP) have been effective in the treatment of regionally confined malignancies. It is unclear whether the route or schedule of DDP administration was responsible for the observed therapeutic benefit. To resolve this issue, tumor and normal tissue platinum (Pt) levels were determined in rats bearing hind-limb rat mammary tumors after intravenous (IV) and intra-arterial (IA) DDP infusions of constant dose and varying lengths. Infusions of DDP at 6 mg/kg were conducted IA over 30 minutes, and 3, 6, 24, and 48 hours and IV over 30 minutes and 48 hours. After infusion, Pt concentrations in solubilized tissue homogenates were measured by flameless atomic absorption spectroscopy. Maximum tumor Pt levels were seen after 48-hour IA infusion (29.3 micrograms Pt/mg tissue). IA infusions of 24 hours or less resulted in significantly lower Pt levels. Maximum tumor Pt concentration after IV administration was only 0.98 micrograms/mg tissue (48-hour infusion). Muscle Pt levels adjacent to the tumor were highest in the IA infused extremities, but at the 48-hour interval, were 53-fold less than tumor levels. Tumor and adjacent muscle Pt levels were not significantly different from each other after IV administration. This study provides pharmacologic evidence that lengthening the duration of IA DDP infusion increases tumor levels of Pt over that of IV or rapid IA administrations. The benefit of prolonged IA DDP infusions is dependent upon both route and schedule of drug administration.
Acute and chronic hypothermia in a man with spinal cord injury: environmental and pharmacologic causes. It is well known that people with spinal cord injuries can develop hypothermia when exposed to an unusually cold environment. Hypothermia can also develop during inpatient rehabilitation, particularly as a side effect of certain medications. We present a patient with C4 incomplete spinal cord injury whose core body temperature was chronically subnormal, and who developed acute hypothermia on several occasions during inpatient rehabilitation. The results of tests of his autonomic function were abnormal. Acute hypothermia (core temperature approximately 34C) was induced when he was challenged with nifedipine. The serum level of phenytoin became elevated to toxic levels during two episodes of acute hypothermia, but the serum level of carbamazepine did not change appreciably. This case demonstrates that people with spinal cord injuries are at risk for hypothermia, that hypothermia can be induced by nifedipine, and that significant thermal challenges can occur during routine inpatient rehabilitation. In addition, hypothermia appears to affect the metabolism of phenytoin.
Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. OBJECTIVE: To characterize mortality in persons diagnosed with primary pulmonary hypertension and to investigate factors associated with survival. DESIGN: Registry with prospective follow-up. SETTING: Thirty-two clinical centers in the United States participating in the Patient Registry for the Characterization of Primary Pulmonary Hypertension supported by the National Heart, Lung, and Blood Institute. PATIENTS: Patients (194) diagnosed at clinical centers between 1 July 1981 and 31 December 1985 and followed through 8 August 1988. MEASUREMENTS: At diagnosis, measurements of hemodynamic variables, pulmonary function, and gas exchange variables were taken in addition to information on demographic variables, medical history, and life-style. Patients were followed for survival at 6-month intervals. MAIN RESULTS: The estimated median survival of these patients was 2.8 years (95% Cl, 1.9 to 3.7 years). Estimated single-year survival rates were as follows: at 1 year, 68% (Cl, 61% to 75%); at 3 years, 48% (Cl, 41% to 55%); and at 5 years, 34% (Cl, 24% to 44%). Variables associated with poor survival included a New York Heart Association (NYHA) functional class of III or IV, presence of Raynaud phenomenon, elevated mean right atrial pressure, elevated mean pulmonary artery pressure, decreased cardiac index, and decreased diffusing capacity for carbon monoxide (DLCO). Drug therapy at entry or discharge was not associated with survival duration. CONCLUSIONS: Mortality was most closely associated with right ventricular hemodynamic function and can be characterized by means of an equation using three variables: mean pulmonary artery pressure, mean right atrial pressure, and cardiac index. Such an equation, once validated prospectively, could be used as an adjunct in planning treatment strategies and allocating medical resources.
Cervical necrotizing fasciitis of odontogenic origin. Necrotizing fasciitis is a severe soft tissue infection caused by both aerobic and anaerobic bacteria and is characterized by a rapid extension along fascial planes and by necrosis of soft tissues. The disease rarely occurs in the head and neck. Three cases of necrotizing fasciitis of the neck after a dental infection are presented. The difficulty in diagnosing the early stage of this condition in relation to other soft tissue infections of odontogenic origin in the neck is discussed. The importance of an early diagnosis followed by an appropriate combination of medical, surgical, and dental treatment is emphasized.
Longitudinal study of the renin-angiotensin-aldosterone system in hypertensive pregnant women: deviations related to the development of superimposed preeclampsia. A prospective longitudinal study of 25 pregnant women (30 pregnancies) with chronic hypertension, a group prone to development of preeclampsia, was conducted to explore the relationship between the renin-angiotensin-aldosterone system and the development of superimposed preeclampsia. In women with chronic hypertension in whom preeclampsia did not develop (17 pregnancies), blood pressure decreased and the renin-angiotensin-aldosterone system was stimulated, beginning in the first trimester and continuing throughout pregnancy as found previously in normotensive pregnant women (n = 58). Plasma estradiol and progesterone levels also increased progressively. In women with chronic hypertension in whom preeclampsia developed (13 pregnancies), blood pressure decreased and the renin-angiotensin-aldosterone system was stimulated in the first trimester as in the other groups. However, later in pregnancy significant differences were observed. Blood pressure began to rise in the second trimester. Initially the renin-angiotensin-aldosterone system remained stimulated, but in the early third trimester, when preeclampsia was diagnosed, plasma renin activity and urine aldosterone excretion decreased, and atrial natriuretic factor increased. These data provide information that may be useful in the recognition of superimposed preeclampsia, and in the investigation of its pathogenesis.
An unusual presentation of salmonellal aortitis. Our patient represents what we believe to be the first documented case of Salmonella choleraesuis aortitis presenting as a salmonellal empyema in an elderly diabetic man. Although S choleraesuis often causes septicemia, its absence should not lead one to forget the pathogenic nature of and high mortality associated with this organism. Aggressive efforts must be made to search for endovascular infection, because cures can be achieved only with surgical intervention and prolonged antibiotic therapy.
Insulin resistance and hypertension. While clinicians have long recognized the apparent increased prevalence of hypertension among diabetics, sophisticated epidemiological analyses begun in the early 1970s have established that hypertensive individuals are more prone to hyperinsulinemia and glucose intolerance than normotensive individuals. Subsequently, the several hypertensinogenic effects of insulin were carefully studied in a number of laboratories. Most recently, the association of these two relatively common cardiovascular risk factors, hypertension and insulin resistance, was broadened to include lipid abnormalities, namely, increased concentrations of very low density lipoprotein triglycerides and decreased concentrations of high density lipoprotein cholesterol. These abnormalities, all of which appear in association more commonly than would be expected by chance, clearly predispose affected individuals to increased cardiovascular risk. This review summarizes our current understanding of the mechanisms underlying the relations between insulin resistance and hypertension and focuses discussion on the role of insulin as a common link between them. It concludes with recommendations, based on today's knowledge, for behavioral and therapeutic interventions aimed at the prevention of increased cardiovascular risk.
Direct mechanical ventricular actuation for cardiac arrest in humans. A clinical feasibility trial Direct mechanical ventricular actuation (DMVA) is a non-blood-contacting method of biventricular cardiac massage which may be applied expediently for total circulatory support. The purpose of this study was to assess the feasibility of DMVA application for patients suffering refractory cardiac arrest. Following informed consent, DMVA was applied in 22 patients. Vascular access for hemodynamic monitoring was possible in only 12 patients, whose outcomes serve as the basis for this report. The mean age of the patients was 48.2 +/- 4.2 years (seven men; five women). The average time from witnessed cardiac arrest to DMVA application was 81 +/- 9 minutes. Application took less than two minutes from the time of skin incision and resulted in immediate hemodynamic improvement. Systolic and diastolic blood pressures averaged 78 +/- 4 and 41 +/- 4 mm Hg, respectively, with a mean cardiac output of 3.14 +/- 0.18 L/min during a mean of 228 +/- 84 minutes of circulatory support (range, 25 minutes to 18 hours). In selected cases the device was temporarily removed for 2 to 3 minutes and open-chest cardiac massage (OCCM) performed at similar compression rates. DMVA increased arterial pressures 65 percent and cardiac output 190 percent compared to OCCM. Initial arterial pH (7.12 +/- 0.04) improved by the time the device was removed (7.24 +/- 0.05). Serum lactate levels decreased from 18.0 +/- 2.3 mumol/L to 14.9 +/- 2.9 mumol/L. Four patients were successfully defibrillated: two had inadequate cardiac function and died within 1 h, and two were successfully resuscitated, but later died from cardiac failure and respiratory insufficiency. Another patient regained normal neurologic function during DMVA and was successfully bridged to cardiopulmonary bypass for emergent coronary artery bypass grafting, but died later from myocardial infarction. There were only two complications: (1) a cardiac laceration during pericardiotomy (1/22 patients); and (2) a ventricular rupture during OCCM (1/22). No complication resulted from the device. We found DMVA to be a feasible method for acute cardiovascular stabilization in victims suffering refractory cardiac arrest. Human clinical trials employing earlier DMVA application are required to determine its resuscitative potential.
Effects of preoperative electrical stimulability and historical factors on performance with multichannel cochlear implant. We investigated the relationship between results of preoperative transtympanic electrical promontory stimulation, duration of deafness, postoperative implanted psychophysical results, and postoperative speech and speech sound recognition as indicated by a battery of five tests. Our subjects were 10 patients implanted with the Cochlear Corporation multielectrode implant, 1 year postimplantation, with a minimum of 17 active electrodes programmed in the bipolar + 1 mode. The results indicated that preoperative promontory thresholds, the slope of the threshold function, and the duration of auditory deprivation are excellent predictors of postoperative speech and speech sound recognition in the auditory (processor alone) mode. These results have significant implications for patient selection and counseling.
Diagnosing and treating common oral pathologies. When the physician is confronted with an oral pathologic condition in a child, the adage "common things happen commonly" should be applied. Congenital lesions such as palatal and alveolar cysts occur in almost 50% of newborns. Developmental conditions such as Fordyce granules and retrocuspid papillae are found in most children. Localized soft-tissue enlargements commonly seen in young children include the parulis, mucocele, papilloma, and inflammatory gingival tumors. In addition, soft-tissue pathologies and discomfort associated with herpesvirus infections or recurrent aphthous ulcerations often present as a chief complaint. The physician's knowledge and treatment recommendations for common oral pathologies should be an integral component to the overall medical management of infants, children, and adolescents.
Exercise. Practical treatment for the patient with depression and chronic fatigue. Exercise should be part of everyone's lifestyle, but may be a particularly important part of the treatment of patients with depression and chronic fatigue. It will reliably and consistently decrease feelings of tiredness and despondency, although it is seldom sufficient as a sole treatment intervention for such patients. When initiating an exercise program, a baseline assessment should be followed by an exercise prescription and an appropriate degree of patient education about exercise physiology. This should be followed, in addition to other treatment interventions, by regular follow-up visits to make appropriate adjustments; provide encouragement and motivation to continue; monitor weight, body fat, and nutrition; and assess the effectiveness of the program on the underlying problem.
Oncogene expression and oral cancer. Cancer of the oral cavity remains a major problem in the United States as well as in many other countries, some of which record as much as 30% of all their cancers to be in the oral region. Despite numerous advances in the fields of epidemiology and etiology, little is known of the molecular basis of oral cancer. Advances in the field of oncogenes have produced a tool to study the different stages of carcinogenesis, including transition from premalignant to malignant stages. These strategies have direct relevance to oral cancer where the premalignant stage is well defined. Preliminary studies into the expression and function of oncogenes suggest abnormalities including individual and multiple amplification of three or more types of these genes. This review briefly describes the concept of oncogenes, and their possible role in the development of neoplasia and specifically on studies in oral cancer.