Melricflash/CW_MedAbstractsAlt huggingface dataset:
Dopamine beta-hydroxylase activity in cerebrospinal fluid of idiopathic torsion dystonia. Since a postmortem biochemical study and a genetic linkage study of idiopathic torsion dystonia suggested possible involvement of dopamine beta-hydroxylase (DBH), we determined CSF DBH activities of Jewish and non-Jewish patients with childhood-onset idiopathic torsion dystonia and found no differences from a control population. 

Category: nervous system diseases
Motor dysfunction in HIV-infected patients without clinically detectable central-nervous deficit. Motor tests were performed in 50 HIV-infected patients in all stages according to the current CDC classification, but without any clinically evident central nervous system deficit, and the results compared with an age-matched control group. Patients were excluded from the study if there was alcohol or drug abuse, fever and/or opportunistic cerebral infection. The parameters tested were postural tremor of the outstretched hands, most rapid voluntary alternating index finger movements (MRAM) and rise time of most rapid index finger extensions (MRC). Whereas tremor peak frequencies did not differ significantly in the patients and controls, MRAM and rise times of MRCs showed significant slowing in the patient group. Morphologically, the motor test performance of the HIV-infected patients was similar to that of patients with manifest basal ganglia disease (Parkinson's, Huntington's and Wilson's diseases). MRI scans of all patients were normal. It is concluded that in HIV-infected patients there is a very early subclinical central nervous system affection, especially of the basal ganglia, which is detectable with appropriate, quantitative motor function tests. These functional abnormalities precede the structural alterations in the MRI scans. 

Category: general pathological conditions
Use of auscultation to follow patients with mitral systolic clicks and murmurs. Mitral systolic clicks and murmurs together with associated symptoms constitute a major reason for cardiologic referral. Although echocardiography with Doppler study enables characterization of the mitral valve apparatus and quantification of regurgitation, its use has resulted in an overemphasis of the technical diagnosis of mitral valve prolapse and an undervaluation of diagnosis based on physical examination. To determine the clinical significance of an auscultatory classification of mitral systolic clicks with or without precordial systolic murmurs, 1 consultant's medical records of 291 patients with these signs were reviewed. Based on initial auscultatory findings, patients were divided into: (1) single or multiple apical systolic clicks with no murmur (n = 99); (2) single or multiple apical systolic clicks and a late systolic murmur (n = 129); and (3) single or multiple apical clicks and an apical pansystolic murmur or murmur beginning in the first half of systole (n = 63). The average duration of patient follow-up was 8 years (range 1 to 30). The prognosis was excellent for patients from all 3 classes. Two cardiac-related deaths occurred: 1 each from classes 1 and 2. Mitral valve surgery was performed in 3 class 2 patients (2%) and in 2 class 3 patients (3%). No patients developed endocarditis during follow-up. Palpitations, with varying anxiety overlay, constituted a major indication for cardiologic referral in all 3 classes. Auscultatory findings were valuable to the physician for explanation and relief of patient anxiety. For patient management, use of an auscultatory classification may be preferable to the technically generated term "mitral valve prolapse.". 

Category: general pathological conditions
Diurnal fluctuations of arterial ketone body ratio in normal subjects and patients with liver dysfunction. To explore the metabolic aspects of chronic liver disease, diurnal changes of arterial ketone body ratio (acetoacetate/3-hydroxybutyrate), reflecting hepatic mitochondrial redox potential, were investigated in normal subjects, patients with chronic liver disease (Child's class A or B), and patients with hepatic failure (Child's class C). Ketone body ratio in normal subjects increased after breakfast from 0.96 to 2.00, after lunch from 2.17 to 2.38, and after dinner from 1.23 to 2.55 with blood glucose level ranging from 103 to 141 mg/dL (5.7 to 7.8 mmol/L). By contrast, the ketone body ratio in the Child A or B group changed little and remained within a range of 0.70-1.35 despite a large change in blood glucose level from 102 to 176 mg/dL (5.7 to 9.8 mmol/L). Ketone body ratio in Child's class C remained near or below 0.4 with no response to glucose administration, despite a marked elevation in blood glucose level. These results indicate that hepatic mitochondrial redox potential undergoes diurnal changes in sharp response to meals in normal liver function but that these fluctuations are absent in patients with liver damage (Child's class A, B, and C). Furthermore, it remains at low levels in severe liver failure (Child's class C). It is also suggested that hepatic mitochondrial redox potential plays an important role in the regulation of blood glucose levels. 

Category: nervous system diseases
Effects of dietary calcium on nimodipine-sensitive calcium channel function in stroke-prone spontaneously hypertensive rats. We studied the effects of dietary Ca2+ on blood pressure, survival, and calcium channel function to investigate cardiovascular disease mechanisms in stroke-prone spontaneously hypertensive rats. Beginning at 3 weeks of age, rats were fed high sodium chloride diets (8.0%) in combination with either high (2.0%) or low (0.2%) Ca2+ diets for 8 weeks. At 12 weeks of age, survival was 90% in the high Ca2+ group and 30% in the low Ca2+ group. The higher blood pressure and lower survival in the low Ca2+ group suggest an intensification of altered vascular muscle cell mechanisms by a dietary Ca2+ deficit. Nimodipine (1-10 nM) effectively blocked L-type Ca2+ currents in isolated vascular muscle cells from both groups. Contraction of isolated cells that were not patch clamped to high potassium solutions were also blocked by 1 nM nimodipine. Disappearance of the L-type Ca2+ channel current was accelerated by holding at depolarizing potentials (positive to -50 mV) and by depolarizing steps to 0 mV. Nimodipine block of the L-type Ca2+ currents in vascular muscle is believed to contribute substantially to antihypertensive properties and stroke prevention, actions that may develop fully only in stroke-prone spontaneously hypertensive rats on a diet of at least normal Ca2+. 

Category: nervous system diseases
Enhanced expression of CD2 antigen on lung T cells. CD2 molecules not only function in the adhesion of T cells to other cell types but also in the activation of T cells via an alternative pathway. To investigate the possible roles of CD2 molecules in the activation and accumulation of lung T cells, using monoclonal antibodies and a flow cytometer we evaluated CD2 antigen expression on lung and blood lymphocytes in 10 normal subjects, 30 patients with pulmonary sarcoidosis, 7 patients with interstitial pneumonia associated with collagen vascular disease, 5 patients with farmer's lung disease, and 8 patients with Crohn's disease. The mean fluorescence intensity (MFI) of CD2 on lung T cells obtained by bronchoalveolar lavage (BAL) was significantly higher than that on blood T cells in all study groups except the control group. In patients with pulmonary sarcoidosis this enhancement of the expression of CD2 antigen was observed only on CD4+ T cells. Since ligands for CD2 are present on immunocompetent cells of other types in the local pulmonary milieu, these results suggest that increased expression of CD2 molecules could facilitate the communication and interaction of lung T cells with their environment in the lung and thereby possibly contribute to the local accumulation of T cells. 

Category: digestive system diseases
Intraoperative scintigraphy for active small intestinal bleeding. Localizing active sites of bleeding within the small intestine remains a difficult task. Endoscopic, angiographic or scintigraphic studies may point to the small intestine as the site of blood loss, but at operation, without a palpable lesion, the exact site of bleeding remains elusive. Patients are managed at laparotomy with intraoperative endoscopy, angiography, multiple enterotomies, "blind" resections, or placement of an enterostomy. We describe two patients in whom intraoperative scintigraphy accurately identified active sites of bleeding in the small intestine when other modalities failed. Intraoperative scintigraphy is rapid, easy to perform and is an effective means of identifying active sites of bleeding within the small intestine. 

Category: general pathological conditions
Cerebrospinal fluid neopterin in human immunodeficiency virus type 1 infection. We evaluated cerebrospinal fluid (CSF) concentrations of neopterin, a putative marker of activated macrophages, in 97 subjects infected with human immunodeficiency virus type 1 who had a spectrum of neurological complications. The highest CSF neopterin concentrations occurred in those with neurological opportunistic infections, primary central nervous systems lymphoma, and acquired immunodeficiency syndrome (AIDS) dementia complex. In general, the CSF concentration of neopterin was independent of CSF cell count and blood-brain barrier disruption to albumin. In the patients with AIDS dementia complex, CSF neopterin concentrations correlated with severity of disease and decreased in conjunction with clinical improvement following treatment with zidovudine. These results suggest that CSF neopterin, although not disease-specific, may be useful as a surrogate marker for the presence of AIDS dementia complex and its response to antiviral therapy. 

Category: nervous system diseases
The role of the immunogenetics laboratory in marrow transplantation. Allogeneic marrow transplants from genotypically HLA-identical siblings have become lifesaving therapy for patients with various congenital and acquired diseases of the immune and hematopoietic systems. Unfortunately, less than 30% of patients in the United States have an HLA-identical sibling; therefore, many are denied the opportunity for potentially curative treatment for otherwise fatal conditions. The results of marrow transplantation from haploidentical family members who were partially compatible for nonshared haplotypes with the patients demonstrated the relevance of HLA compatibility for engraftment and graft-vs-host disease. These results have provided the rationale for using HLA-identical unrelated volunteers as marrow donors. The recent development of a national registry of volunteer bone marrow donors (the National Marrow Donor Program in the United States) has made it possible to access a centralized file of more than 260,000 HLA-typed volunteers. Marrow transplants from an unrelated donor have become feasible. However, our preliminary clinical experience clearly demonstrated an increase in the incidence and severity of graft-vs-host disease. It is likely that standard HLA typing techniques are not adequate to provide data for sufficient donor and recipient matching. It is possible that currently available molecular technology could be applied to HLA typing and donor matching to improve on clinical results. 

Category: neoplasms
Thiazide diuretics and the risk of hip fracture. Results from the Framingham Study. Thiazide diuretics may preserve bone mass and prevent elderly women's osteopenic fractures, but studies have not distinguished between thiazide preparations or examined former users. We performed a case-control study looking at thiazide use and subsequent hip fracture in postmenopausal female members of the Framingham Study cohort. Cases who had experienced a first hip fracture (n = 176) were compared with age-matched controls (n = 672). Results showed a modest protective effect of any recent thiazide use (not significant). However, recent pure thiazide users experienced significant protection against fracture (adjusted odds ratio, 0.31; 95% confidence interval, 0.11 to 0.88), whereas recent users of combination drugs containing thiazides experienced no protection (adjusted odds ratio, 1.16; 95% confidence interval, 0.44 to 3.05). Combination drugs generally contained only 25 mg of hydrochlorothiazide, suggesting that the small amount of thiazide was insufficient to preserve bone mass. Former thiazide users were not protected against fracture. In sum, recent pure thiazide use in women protects against hip fracture. 

Category: cardiovascular diseases
Clinicopathological features of elevated lesions of the duodenal bulb. We present here our findings on patients with an elevated lesion of the duodenal bulb. All these patients were treated in our clinics between the years 1984 and 1988. These lesions were present in 36 of 8,802 patients who underwent upper gastrointestinal pan-endoscopy. Two patients had a duodenal carcinoma, 2 an adenoma, and 1 a Brunner's gland adenoma. There were 15 with a hyperplastic polyp, 3 with a heterogenic gastric mucosa, 3 with Brunner's gland hyperplasia, 6 with duodenitis, and 4 with regenerative mucosa. Among these 36 lesions, only 69% (25 lesions) were evident on the upper gastrointestinal X-ray series. Adenoma and Brunner's gland adenoma were of a pedunculated form of the gross type and had an irregular surface mucosa. Both duodenal carcinomas were detected by endoscopic biopsy and were resected. Histologically, these lesions were limited to the submucosal layer and were of the non-pedunculated polypoid form, but there were no other characteristic endoscopic features, in comparison with other elevated lesions. Thus, upper gastrointestinal endoscopy with routine observations of the duodenal bulb plus endoscopic biopsy will lead to a definite diagnosis of these elevated lesions and to the early detection and treatment of this rare malignant lesion. 

Category: digestive system diseases
Why do lean hypertensives have higher mortality rates than other hypertensives? Findings of the Hypertension Detection and Follow-up Program. Specific causes of death were analyzed for 10,908 participants in the Hypertension Detection and Follow-up Program, to explore possible explanations for the observed excess 8.3-year mortality from all causes in hypertensives with low body mass. Although the cardiovascular mortality rate among men in the lowest decile of body mass (body mass index 21.96 or less) was 50% higher than that of men in the median class (body mass index 26.4-28.8), death rate for noncardiovascular deaths was more than 2 1/2 times higher in men with lean versus median body mass. The pattern was similar among women. Among noncardiovascular causes, striking differences in mortality rates between lean hypertensives and those of average body mass were observed for cirrhotic death (relative risk of 12+ in men and 11+ in women), for nonmalignant respiratory disease in men (relative risk of 7+), for violent death (both sexes), and for malignant neoplasms in men. Prevalence of smoking was almost twice as high in the lowest compared with the median body mass group; among the lean, excess deaths, particularly noncardiovascular deaths, were concentrated among smokers. Thus, male smokers in the lowest decile of body mass constituted only 3% of the study population, but contributed 8% of all deaths, 11% of all noncardiovascular deaths, and 22% of all cirrhotic deaths. A larger proportion of deaths occurred early in follow-up in the lean versus other hypertensives, suggesting occult disease among the lean at baseline. There was no evidence that more severe or treatment-resistant hypertension was present in or could explain excess mortality among the hypertensives with low body mass. The inference from the findings is not that overweight is protective for hypertensives nor that excess risk is due to leanness per se. Rather, a reasonable hypothesis, particularly from findings on specific causes of death, is that excess mortality in lean hypertensives is due to deleterious lifestyles, particularly smoking and excess alcohol intake, contributing to both leanness and risk of death. 

Category: general pathological conditions
Cardiopulmonary perfusion and cerebral blood flow in bilateral carotid artery disease The fear of cerebral complications after cardiopulmonary bypass in patients with heart disease and severe carotid artery disease has led many authors to suggest combined approaches in these patients. The pathogenetic mechanism for stroke is based partly on the stenotic narrowing of the carotid artery. A diameter reduction of 75% is frequently considered hemodynamically significant and indicative of an increased risk for neurological morbidity. We studied the cerebral blood flow in 7 patients undergoing coronary artery bypass grafting who also had severe bilateral carotid disease. The results were compared with the results in 17 patients without carotid disease who had bypass grafting. The cerebral blood flow was measured by xenon 133 washout technique before, during, and after cardiopulmonary bypass with moderate hypothermia. Acid-base regulation was according to the alpha-stat theory, and blood pressure was kept greater than 50 mm Hg. The cerebral blood flow levels (mL.100g-1.min-1) before, during, and after cardiopulmonary bypass in the study group (30 +/- 11, 31 +/- 8, 47 +/- 20) (mean +/- standard deviation) were almost identical to those in the control group (30 +/- 11, 28 +/- 8, 47 +/- 12). The cerebral blood flow levels for the left and right hemispheres in the group with carotid disease were comparable and within normal ranges. In 2 patients, slight differences were noted between hemispheres, and this finding may indicate an increased risk for ischemia. These patients, however, did not show any signs of postoperative deficit. The flow limitations of critical carotid stenoses do not seem to imply a risk for cerebral hypoperfusion if cardiopulmonary perfusion is performed in a controlled manner. 

Category: cardiovascular diseases
Stridor: intracranial pathology causing postextubation vocal cord paralysis. During an 18-month period in a pediatric intensive care unit, nine patients with vocal cord paralysis were identified using flexible bronchoscopy. When tracheally extubated, each child was found to have stridor. The children ranged in age from 17 days to 5 1/2 years. Two patients had unilateral paralysis, but neither required tracheostomy. Seven patients displayed bilateral abductor vocal cord paralysis. Of these, six patients required tracheostomy. Surgical injury to the recurrent laryngeal nerve was the probable cause in two patients. The other seven patients had neurologic disorders with documented or suspected increases of intracranial pressure. Four of the seven patients with bilateral abductor vocal cord paralysis regained cord mobility within 4 months. Both children with unilateral cord paralysis have no stridor and vocalize well 1 year later. Cord paralysis in the setting of intracranial hypertension probably results from compression or ischemia of the vagus nerve before it exits the skull. Early visualization of the larynx should be done in patients who become stridulous when extubated, especially those with prior thoracic procedures or with neurologic disorders associated with intracranial hypertension. 

Category: general pathological conditions
Implication of specific DQB1 alleles in genetic susceptibility and resistance by identification of IDDM siblings with novel HLA-DQB1 allele and unusual DR2 and DR1 haplotypes. Genetic susceptibility to insulin-dependent diabetes mellitus (IDDM) is associated with the HLA-DR3 and DR4 haplotypes. The HLA-DR2 haplotype is negatively associated with IDDM, an association that has been interpreted as dominant protection. Here, we describe the molecular analysis of the HLA class II genes in an unusual family with three HLA-DR1/2 siblings, all of whom have IDDM. With polymerase chain reaction amplification and sequence analysis to characterize the class II alleles, we identified a novel DQB1 allele on the DR1 haplotype and an unusual DQB1 allele on the DR2 haplotype. However, the DRB1 alleles on these DR1 and DR2 haplotypes are the conventional alleles (*0101 and *1501, respectively). These results suggest that it is the conventional DQB1 allele (*0602) not the DRB1 allele (*1501) on the protective DR2 haplotype that confers protection in the general population and, furthermore, that these unusual DQB1 alleles may confer susceptibility to IDDM in this family. The unusual DQB1 allele on this DR2 haplotype encodes Asp at position 57, indicating that it is the allele DQB1*0602 and not simply the presence of this residue that is responsible for the protective effect. 

Category: general pathological conditions
Neonatal small bowel atresia, stenosis and segmental dilatation. In 8 years, 44 neonates were treated for small bowel atresia, stenosis or segmental dilatation by one surgical team. There was a single lesion in 30 infants and 14 had two or more sites of intestinal obstruction. Associated gastrointestinal abnormalities were found in 23 (52 per cent) patients. Resection of the atresia(s) and primary anastomosis were performed in 34 infants and staged procedures were carried out in ten patients with complicated lesions. Patients undergoing staged procedures had a higher complication rate than those having primary repair (six out of ten and 11 out of 34 respectively). The overall initial survival rate was 93 per cent, rising to 100 per cent in those without associated anomalies or prenatal complications, but more than a quarter (27 per cent) of survivors have long-term disabilities. 

Category: digestive system diseases
Abdominal wall pain: an alternative diagnosis. The cause of abdominal pain need not necessarily reside in the viscera; the abdominal wall is another source of symptoms. Some causes of abdominal wall pain are obvious, e.g. hernias, but not so others such as nerve entrapment syndromes. This review is concerned with causes of abdominal wall pain which, although common, may be easily overlooked. 

Category: digestive system diseases
Possible involvement of the retinoblastoma gene in undifferentiated sinonasal carcinoma. Retinoblastoma tumor formation is initiated by the loss of function of both alleles of the RB-1 gene on chromosome 13. Patients with the hereditary form of retinoblastoma carry a germ line mutation at one of the two homologous gene loci in all cells and have an increased risk for nonocular tumors (mainly osteosarcoma and other mesenchymal tumors) in later life. The authors studied a 38-year-old patient with sinonasal undifferentiated carcinoma (SNUC) who had been treated for bilateral retinoblastoma by enucleation (left eye) and irradiation (right eye), respectively. Using molecular probes for the RB-1 gene and other loci on chromosome 13, the authors detected a deletion at the RB-1 locus in metastatic SNUC cells that was not present in normal tissue. These findings indicate that somatic mutations at RB-1 locus may be involved in the formation or progression of ectodermal tumors. 

Category: neoplasms
HIV infection: clinical features and treatment of thirty-three homosexual men with Kaposi's sarcoma. The clinical findings of patients with oral Kaposi's sarcoma are reviewed. These oral findings commonly included candidiasis, hairy leukoplakia, gingivitis associated with human immunodeficiency virus (HIV), periodontitis, and other symptoms, including xerostomia. The other common symptoms of HIV disease that may be of importance in leading to a diagnosis are reviewed in this patient group. Treatment by local radiotherapy or by intralesional vinblastine of these oral Kaposi's sarcomas resulted in successful palliation, with more than 50% regression of the lesions in 80% of the patients treated. 

Category: neoplasms