123rc/medical_text huggingface dataset:
Musculoskeletal problems in association with cloacal exstrophy. The records of all thirteen patients for whom a diagnosis of cloacal exstrophy had been recorded in our hospitals were analyzed for evidence of musculoskeletal problems. All thirteen patients had spina bifida, four had congenital scoliosis, two had congenital kyphosis, and three had non-congenital scoliosis. All had a lipomeningocele, and eleven had paralysis of the lower extremities. Hydrocephalus and its associated problems were not found, but tethered-cord syndrome was diagnosed in eleven patients. Persistent diastasis of the symphysis pubis was found in all patients. Abduction and external rotation of the hips were more than normal. Mild dysplasia was seen in six of the twenty-six hips. Deformities of the foot were common, and twelve feet had been operated on for correction. Recurrent equinovarus deformity of the foot was associated with tethered-cord syndrome in two patients.
Influence of low osmolality contrast media on electrophysiology and hemodynamics in coronary angiography: differences between an ionic (ioxaglate) and a nonionic (iohexol) agent. It has recently been suggested that the addition of sodium to low osmolality contrast media may reduce the incidence of ventricular fibrillation and conduction disturbances during coronary angiography. In a randomized, double blind study of 30 patients undergoing coronary angiography we therefore examined the electrophysiological and hemodynamic effects of the two low osmolality contrast media-ioxaglate (with sodium) and iohexol (without sodium). Standard ECG, aortic blood pressure, and His bundle electrocardiogram were recorded. The contrast media were well tolerated and no serious arrhythmias were observed. Both induced a transient decrement in systolic blood pressure and reduction in heart rate 10 s following contrast injection (all P less than 0.01). Ioxaglate prolonged the QT interval at 10 s (P less than 0.01) and also when analysed for the whole observation period (120 s) (P less than 0.05), whereas iohexol did not cause any significant changes in the QT-interval. The AH-interval was prolonged by ioxaglate at 10 s (P less than 0.01), but not altered by iohexol. Thus, other factors than osmolality and sodium content might contribute to QT prolongation, since only the contrast agents with sodium (ioxaglate) induced QT prolongation in this study.
Attitudes of major employers toward the employment of people with epilepsy: a 30-year study. Beginning in 1956, major San Francisco Bay area employers were sampled at 10-year intervals for a 30-year period to assess attitudes toward the employment of epileptic workers. In this time, we documented a trend of sustained positive change that appears to validate the efforts of organizations that have focused on raising public awareness of this disorder.
Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk Ventricular dysfunction, elevated pulmonary vascular resistance, and residual distal pulmonary artery distortion contribute to early mortality after a Fontan operation; they may be transient or reversible. A baffle fenestration, allowing right-to-left shunting, maintains cardiac output and limits right atrial pressure. A baffle fenestration was surgically created at the time of a modified Fontan repair in 20 consecutive patients. Risk factors included pulmonary artery pressure of 18 mm Hg or more, end-diastolic pressure of 12 mm Hg or more, valvar regurgitation, pulmonary artery distortion, pulmonary vascular resistance of 2 Woods' units or more, ventricular outflow obstruction, and complex anatomy. Nineteen of 20 patients survived. After the operation, mean arterial oxygen saturation was 86%, mean right atrial pressure was 15 mm Hg, and mean duration of pleural effusions was 6 days. Twelve of 19 survivors tolerated early test occlusion and had permanent transcatheter umbrella closure. Four patients failed early test occlusion, with a significant decrease in venous O2 saturation and a rise in central venous pressure, due to ventricular dysfunction, pulmonary artery distortion, or aortopulmonary collaterals. Three of four had successful late closure of the fenestration after correction of these abnormalities.
Abnormalities of von Willebrand factor multimers in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. PURPOSE: To analyze and review von Willebrand factor (vWF) multimeric patterns in patients with single-episode thrombotic thrombocytopenic purpura (TTP), intermittent TTP (episodes at infrequent, irregular intervals), chronic relapsing TTP (episodes at frequent, regular intervals), and the hemolytic-uremic syndrome (HUS). PATIENTS AND METHODS: Platelet-poor plasma samples were obtained in EDTA, citrate, or citrate-hirudin-aprotinin-leupeptin from 36 patients with single-episode TTP, eight patients with intermittent TTP, four patients with chronic relapsing TTP, and 26 patients with HUS. The samples were separated by sodium dodecyl sulfate-agarose gel electrophoresis, overlaid with rabbit 125I-anti-human vWF IgG, and analyzed by autoradiography. RESULTS: Abnormalities of vWF multimers were found in platelet-poor plasma samples from 31 of 36 found in platelet-poor plasma samples from 31 of 36 patients (86%) at the onset of and during their single TTP episode. vWF multimers larger than those in normal plasma, and similar to vWF forms observed within normal human endothelial cells (unusually large vWF multimers), were demonstrated in 31% of the patients; 19% had either unusually large vWF multimers or a relative decrease in the largest plasma vWF forms in different serial samples; 36% had a relative decrease in the largest plasma vWF forms. These results imply that endothelial cell injury or intense stimulation, along with the attachment of unusually large vWF multimers and the largest plasma vWF forms to platelets, occurred during the single TTP episodes in most patients. Patterns of vWF multimers were normal in 92% of patients with single-episode TTP studied after recovery. All eight patients with intermittent TTP and the four patients with chronic relapsing TTP had unusually large vWF multimers in their plasma between episodes, and these multimers decreased or disappeared during relapses. Of 26 children and adults with HUS, 14 had a relative decrease in the largest plasma vWF multimeric forms and one had unusually large vWF multimers during the episode (vWF multimeric abnormalities in 58% of the patients). CONCLUSION: It is probable that vWF was involved in the pathophysiology of TTP in most of these patients with the single-episode, intermittent, or chronic relapsing types of TTP, and in more than 50% of the patients with HUS.
Laryngeal dystonia: a series with botulinum toxin therapy. Laryngeal dystonia is a syndrome characterized by action-induced, involuntary spasms of the laryngeal muscles. Most patients have involvement of the adductor laryngeal muscles producing uncontrolled spasms during phonation, and a "strain-strangle" speech pattern commonly termed "spastic dysphonia." Other patients have involvement of the abductor muscles producing "whispering dysphonia." Rare patients have paradoxical vocal cord motion during respiration with adductor spasms on inspiration. Over the past 5 years we have used botulinum toxin (BOTOX) to treat more than 200 patients with laryngeal dystonia. This group includes patients with adductor involvement (phonatory dystonia, recurrent laryngeal nerve section failure, respiratory dystonia) and those with abductor involvement (whispering dystonia). Patients received benefit within 24 to 72 hours, with sustained improvement for 2 to 9 months with an average of 4 months. Patients improved to an average of 90% of normal function. Clinically significant adverse effects included extended breathy dysphonia and mild choking on fluids. BOTOX has become our treatment of choice for dystonic conditions of the larynx.
Host response to mycobacterial infection in the alcoholic rat. Animals, chronically treated with alcohol, were inoculated with mycobacteria (bacillus Calmette-Guerin, 10.2 x 10(6) organisms) into the spleen to produce a granulomatous hepatitis. Before infection, chronic alcohol ingestion was associated with a depressed skin test response to phytohemagglutinin, 71.7% of baseline (P = 0.009). Mycobacterial (bacillus Calmette-Guerin) infection stimulated phytohemagglutinin skin test response to 417% of baseline in controls and 299% in alcoholics (P less than 0.001). The hepatic granuloma response was altered with smaller but more numerous granulomas (mean +/- SEM, 81.2 +/- 1.5 microns2 of area with a frequency of 1.8 granulomas per field in alcoholics vs. 129.8 +/- 5.71 microns2 and 1.2 granulomas per field in controls; P less than 0.001). These changes were associated with a 10-fold increase in colony-forming units per gram of liver (54.5 +/- 18.2 in alcoholics vs. 5.6 +/- 1.83 in controls; P = 0.0006). This model offers precise parameters for host response to infection and indicates that alcohol significantly impairs the clearing capacity for mycobacteria from the liver.
Usefulness of antithrombotic therapy in resting angina pectoris or non-Q-wave myocardial infarction in preventing death and myocardial infarction (a pilot study from the Antithrombotic Therapy in Acute Coronary Syndromes Study Group). In a prospective pilot trial of antithrombotic therapy in the acute coronary syndromes (ATACS) of resting and unstable angina pectoris or non-Q-wave myocardial infarction, 3 different antithrombotic regimens in the prevention of recurrent ischemic events were compared for efficacy. Ninety-three patients were randomized to receive aspirin (325 mg/day), or full-dose heparin followed by warfarin, or the combination of aspirin (80 mg/day) plus heparin and then warfarin. Trial antithrombotic therapy was added to standardized antianginal medication and continued for 3 months or until an end point was reached. Analysis, by intention-to-treat, of the 3-month end points, revealed the following: recurrent ischemia occurred in 7 patients (22%) after aspirin, in 6 patients (25%) after heparin and warfarin, and in 16 patients (43%) after aspirin combined with heparin and then warfarin; coronary revascularization occurred in 12 patients (38%) after aspirin, in 12 patients (50%) after heparin and warfarin, and in 22 patients (60%) after aspirin combined with heparin and then warfarin; myocardial infarction occurred in 1 patient (3%) after aspirin, in 3 patients (13%) after heparin and warfarin, and in no patient after aspirin combined with heparin and then warfarin; no deaths occurred after aspirin or after aspirin combined with heparin and then warfarin, but 1 patient (4%) died after warfarin alone; major bleeding occurred in 3 patients (9%) after aspirin, in 2 patients (8%) after heparin and warfarin, and in 3 patients (8%) after aspirin combined with heparin and then warfarin. Recurrent myocardial ischemia occurred at 3 +/- 3 days after randomization.
Increase in incidence of disease due to diagnostic drift: primary liver cancer in Denmark, 1943-85. OBJECTIVE--To examine the extent to which changes in diagnostic methods and classification are responsible for the striking increase in incidence of primary liver cancer in Denmark since 1943. DESIGN--Analysis of the time trends in sex specific, age standardised incidence of primary liver cancer and unspecified liver cancer (either secondary without known primary cancer or not specified as primary cancer) in the entire population from 1943 to 1985. By review of the 727 notifications from three periods of 5 years (1948-52, 1963-7, and 1978-82) the changes in histological diagnosis and classification were assessed. SETTING--Denmark. SUBJECTS--Notifications of liver cancer to the Danish cancer registry. RESULTS--Concomitant with the increase in primary liver cancer, the incidence of the unspecified liver cancer declined. The proportion of histologically diagnosed primary liver cancer rose from 85% to 98%, whereas the proportion for unspecified liver cancer rose from 12% to 51%. When the proportion of primary versus unspecified liver cancer obtained by histological diagnosis was extrapolated to all cases, the annual incidence of primary liver cancer was 4.4 rather than 1.6 per 100,000 population in 1948-52 and 6.0 rather than 5.5 per 100,000 in 1978-82. CONCLUSION--The increase in the incidence of primary liver cancer may be much smaller than the numbers of registered cases indicate. This example emphasises the need to consider diagnostic drift in time trend studies of disease incidence.
Asymmetric growth of the lateral cerebral ventricle in infants with posthemorrhagic ventricular dilation. Lateral cerebral ventricular volume in 36 preterm infants with or without an intraventricular hemorrhage, and with or without posthemorrhagic hydrocephalus, was measured longitudinally and compared with the ventricular index measurements of the same ventricles. A poor correlation was found (r2 = 0.67). To determine a reason for this poor relationship, we analyzed the volumes of the regions of the ventricles by a segmental volume analysis. The occipital region of the lateral cerebral ventricle enlarged at a much faster rate (1.904 +/- 0.477 ml/day) than either the anterior region (0.546 +/- 0.253 ml/day; p less than 0.01) or the middle region (-0.209 +/- 0.334 ml/day; p less than 0.01) in infants with posthemorrhagic hydrocephalus. The rate of growth of the middle region of the lateral cerebral ventricles was the same for all infants. Linear indexes, such as the ventricular index and the lateral ventricular ratio, do not allow for accurate serial estimates of ventricular size in posthemorrhagic hydrocephalus because of asymmetric growth of the lateral cerebral ventricle. We conclude that sequential volume measurements are more useful than ventricular index measurements to follow ventricular size sequentially in infants with posthemorrhagic hydrocephalus.
Bilateral malignant phyllodes tumours. We report a rare example of bilateral primary malignant phyllodes tumours. The diagnosis was supported by the identification of a benign epithelial element in each lesion. The case illustrates the typical dimorphic features of malignant phyllodes tumours. A contralateral tumour should not be regarded as metastatic without histological confirmation.
Flow cytometric DNA analysis of hepatocellular carcinoma: preliminary report. Flow cytometric DNA analysis was performed in 50 paraffin-embedded specimens of clinical hepatocellular carcinoma (HCC) after hepatic resections. The DNA distribution pattern was classified in two types, diploid and aneuploid, according to the degree of dispersion on the DNA histogram. The major DNA pattern of HCC in this report proved to be aneuploid (78%), although 22% of tumors revealed a diploid pattern. The serum alpha-fetoprotein level exceeded 40 ng/ml in 86.1% of the aneuploid tumors and in 13.9% of the diploid tumors (p less than 0.05). We found no correlation between DNA distribution and hepatitis B surface antigen positivity, the presence of liver cirrhosis or tumor size. Additionally we noted no significant correlation between the DNA pattern and survival rates in patients with HCC who underwent hepatic resection.
Effect of somatostatin analogue (octreotide) on blood flow to endocrine tumors metastatic to the liver: angiographic evaluation. The effect of an octapeptide analogue of somatostatin, octreotide, on tumor blood flow was evaluated with angiography in eight patients with hepatic endocrine tumors; one patient had primary intrahepatic gastrinoma, two patients had hepatic metastases from gastrinomas, two patients had VIPomas (vasoactive intestinal polypeptide-secreting tumor), and three patients had carcinoid tumors. Octreotide caused a marked decrease in tumor blood flow in two patients with gastrinomas and two with VIPomas. One patient could not be evaluated due to the lack of a tumor blush on a control angiogram. In patients with carcinoid tumors, octreotide caused a slight reduction in blood flow through the tumors in two patients, while there was no change in one patient. Octreotide markedly decreased gastrin and gastric acid secretion in two of three patients with gastrinomas, lowered VIP and stopped the diarrhea in patients with VIPomas, and controlled symptoms in two of three patients with carcinoid tumors. The vasoactive effect of octreotide on hepatic endocrine tumors may be a direct action on tumor blood supply or secondary to inhibition of the endocrine tumor cell secretion and consequent decreased blood flow.
Familial predisposition for herniation of a lumbar disc in patients who are less than twenty-one years old. The parents of sixty-three patients who were less than twenty-one years old and who had operatively confirmed herniation of a lumbar disc were interviewed regarding a history of sever back pain, sciatica, and herniated disc, to determine whether aggregation of herniation of a lumbar disc occurs in families of patients in this young age-group. The parents of sixty-three additional patients who had a non-spinal orthopaedic diagnosis (control group) were matched for age and sex with the study group and were given the same interview. Of the patients who had herniation of a lumbar disc and were less than twenty-one years old, 32 per cent had a positive family history for that lesion compared with 7 per cent of the control group. The relative risk of development of herniation of a lumbar disc before the age of twenty-one years is estimated to be approximately five times greater in patients who have a positive family history. The results indicate a familial basis for herniation of a lumbar disc in patients who are less than twenty-one years old.
Glucose-induced exertional fatigue in muscle phosphofructokinase deficiency BACKGROUND. The exercise capacity of patients with muscle phosphofructokinase deficiency is low and fluctuates from day to day. The basis of this variable exercise tolerance is unknown, but our patients with this disorder report that fatigue of active muscles is more rapid after a high-carbohydrate meal. METHODS AND RESULTS. To determine the effect of carbohydrate on exercise performance, we asked four patients with muscle phosphofructokinase deficiency to perform cycle exercise under conditions of differing availability of substrate--i.e., after an overnight fast, and during an infusion of glucose or triglyceride (with 10 U of heparin per kilogram of body weight) after an overnight fast. As compared with fasting and the infusion of triglyceride with heparin, the glucose infusion lowered plasma levels of free fatty acids and ketones, reduced maximal work capacity by 60 to 70 percent, and lowered maximal oxygen consumption by 30 to 40 percent. Glucose also increased the relative intensity of submaximal exercise, as indicated by a higher heart rate at a given workload during exercise. The maximal cardiac output (i.e., oxygen delivery) was not affected by varying substrate availability, but the maximal systemic arteriovenous oxygen difference was significantly lower during glucose infusion (mean +/- SE, 5.5 +/- 0.3 ml per deciliter) than after fasting (7.6 +/- 0.4 ml per deciliter, P less than 0.05) or during the infusion of triglyceride with heparin (8.9 +/- 1.3 ml per deciliter, P less than 0.05). CONCLUSIONS. In muscle phosphofructokinase deficiency, the oxidative capacity of muscle and the capacity for aerobic exercise vary according to the availability of blood-borne fuels. We believe that glucose infusion lowers exercise tolerance by inhibiting lipolysis and thus depriving muscle of oxidative substrate (plasma free fatty acids and ketones); this impairs the capacity of working muscle to extract oxygen and lowers maximal oxygen consumption.
Liver transplantation: MR angiography with surgical validation. Thirty patients (mean age, 45 years) were evaluated with magnetic resonance (MR) angiography before liver transplantation to assess the accuracy of MR angiography. A series of breath-hold, two-dimensional images were acquired and subsequently processed to form three-dimensional projection angiograms. Graphic information on blood flow in the portal vein was acquired by using presaturation bolus tracking. Correlative duplex ultrasound (US) was performed in 28 patients, and surgical or autopsy correlation was available in all cases. MR angiography demonstrated patency of the portal vein in 26 (96%) of 27 patients, made possible the diagnosis of portal venous occlusion in three of three patients, depicted reversed portal flow in one patient, and provided clear delineation of the extent of varices and specific portosystemic collateral vessels. When duplex US was successful, there was full agreement with MR angiographic results in assessing portal vein patency and flow direction. All of the MR findings were corroborated at surgical exploration or autopsy. The authors conclude that MR angiography is very accurate in the portal system and is valuable in preoperative assessment for liver transplantation.
Isolated muscular sarcoidosis causing fever of unknown origin: the value of gallium-67 imaging. An unusual case of a patient with a long-standing fever of unknown origin (FUO) is presented whose gallium-67 (67Ga) images revealed increased activity only in the calf muscles bilaterally. Other imaging modalities also failed to show chest or other abnormal findings. Subsequent biopsy of the right gastrocnemius muscle revealed noncaseating granulomas consistent with the diagnosis of sarcoidosis. When using 67Ga to evaluate a patient with a FUO, imaging of the extremities should always be included. Also, when abnormal Ga-67 uptake is present in the extremities, sarcoidosis should be included in the differential diagnosis.
Behavioral and psychosocial cancer research. Building on the past, preparing for the future. This report identifies five general conclusions that emerged from the Second Workshop on Methodology in Behavioral and Psychosocial Cancer Research. These conclusions address diverse topics, including a focus on areas other than methodology; an emphasis on the transfer of technology and knowledge to applied settings; a recognition of the role of basic behavioral research in answering clinical questions; the need to recognize and strengthen ties between the field of behavioral and psychosocial oncology and the basic behavioral and social science fields from which it emerged; and the importance of additional research on minorities and other special populations. It is suggested that meeting the challenges posed in each of these five areas is critical to continued progress in the field.
Microbiological studies of the enterocolitis of Hirschsprung's disease. The results of a prospective study of 20 cases of newly diagnosed Hirschsprung's disease (nine of whom developed enterocolitis) and 10 normal controls showed no variations in the bacterial flora (including Clostridium difficile) in the stools of the groups studied. Viral studies showed that rotavirus was present in the stools of seven of the nine cases of enterocolitis during the episode. We suggest that Hirschsprung's enterocolitis may have a complex infective aetiology and that rotavirus plays a part.