123rc/medical_text huggingface dataset:
Family history fails to identify many children with severe hypercholesterolemia. Optimal strategies for identifying children with hypercholesterolemia have not been established. Several groups have advocated that testing of serum cholesterol levels be limited to those children who have family histories of hyperlipidemia or premature coronary heart disease. We studied the ability of comprehensive family histories to identify children with hyperlipidemia in a group of 114 children (mean age, 8 +/- 4 years) who were referred for treatment of hypercholesterolemia. A positive family history was defined according to guidelines of the American Academy of Pediatrics. The mean fasting total cholesterol in the children was 5.74 +/- 1.42 mmol/L (222 mg/dL). Family history was negative for hypercholesterolemia or premature coronary heart disease in 22 (22%) of 100 children with total cholesterol levels greater than the 75th percentile for their ages, in 13 (18.3%) of 71 children with total cholesterol levels greater than the 95th percentile for their ages, and in four (11.8%) of 34 children with presumed heterozygous familial hypercholesterolemia. Of the 78 children who had both hypercholesterolemia and positive family histories, hyperlipidemia was reported in 72 families, whereas premature heart disease was reported in only 27. We conclude that in a population of children referred because of known hypercholesterolemia, a detailed family history not only fails to identify many children with mild hypercholesterolemia, but also fails to identify a significant proportion of children with markedly elevated cholesterol levels. Additionally, in families of children with hypercholesterolemia, a history of hyperlipidemia is more common than a history of premature heart disease.
Assessment of ventricular function with an ambulatory left ventricular function monitor. Changes in ventricular function caused by activities of daily living, including standing, walking, stair climbing, and mental stress, were evaluated using a radionuclide device that recorded left ventricular function on a beat-by-beat basis. The ambulatory monitor was positioned over the patient's left ventricle after a gated blood pool scan. Monitoring revealed a 10% increase of left ventricular ejection fraction from baseline to brisk walking, an 18% increase during stair climbing, and a 6% increase with mental stress. In some subjects, however, the increase in ejection fraction during mental stress exceeded that during exercise.
Morphological and histopathological aspects of aneurysms after patch aortoplasty for coarctation. Repair of coarctation of the aorta by synthetic patch grafting has been complicated by late aneurysm formation. These aneurysms differ macroscopically from atherosclerotic thoracic aortic dilatations. Specimens for microscopic examination were taken from 14 of 20 patients undergoing aneurysm resection. Histological analysis of the specimens showed medionecrosis in 13 patients of the specimens showed medionecrosis in 13 patients (93%), foreign body reaction in 11 patients (78%), and intimal thickening in 3 patients (21%). The three layers of the aortic wall could be identified in the aneurysms. On the basis of these results, we discuss the etiologic factors and pathogenetic mechanisms involved in the development of these aneurysms.
A clinical study of acute gastrointestinal hemorrhage associated with various shock states. Gastrointestinal hemorrhage from stress ulceration is a life-threatening complication in a critically ill patient. We retrospectively studied 471 patients admitted to the Department of Traumatology of our hospital who developed shock in their clinical course. Forty-two patients (8.9%) developed gastrointestinal hemorrhage, most within 1 wk (76.2%). The hemorrhage lesion usually was located in the corpus of the stomach. The mortality rate of the shock patients with gastrointestinal hemorrhage was 33.3% (14 of 42). Comparison of different types of shock showed that the incidence, severity, and mortality rates of gastrointestinal hemorrhage were significantly higher in septic shock patients than in hemorrhagic shock patients. These findings show the importance of preventive therapy against progression of early mucosal damage and development of gastrointestinal hemorrhage in shock patients, especially those with sepsis.
Expression of the human nephron differentiation molecules in renal cell carcinomas. The authors tested frozen sections from 28 renal cell carcinomas (RCC)--21 clear, 1 eosinophilic, 4 basophilic, and 2 spindle-shaped cell type--with monoclonal antibodies (MAb) reacting against cytokeratin, vimentin, CD24, CALLA/CD10, villin, CD26, and HLA class I and class II molecules. These molecules are markers of specific segments of the mature kidney, and their loss or acquisition reflects the different steps of human nephrogenesis. KI67 MAb was used to evaluate cell-proliferating activity. All RCC cases expressed cytokeratin. Coexpression of vimentin was observed in 21 of 28 cases. Whether of clear or chromophilic type, all tumoral cells strongly expressed CD24 molecule, present on primitive blastema cells. All clear-type RCCs expressed CALLA/CD10 and 60% were also villin positive; some were faintly positive for CD26. CALLA, villin, and CD26 were not detected in basophilic cell type. HLA class I molecules were variably expressed in almost all cases, but HLA class II were never detected on tumoral cells. Except for the spindle-shaped population, cell-proliferating activity was low. These results favor the hypothesis that RCCs derive from cells that have 'recovered' the different options of metanephric differentiation. Clear cells show evidence of maturation toward proximal type, while basophilic cells do not. It would be of interest to evaluate the usefulness of serum measurements of villin and/or CALLA as markers in clear cell-type RCC.
Tension pneumothorax secondary to a gastropleural fistula in a traumatic diaphragmatic hernia. We report a case of tension pneumothorax due to a gastropleural fistula resulting from perforation of the stomach in a traumatic diaphragmatic hernia. Awareness of perforation of strangulated stomach or bowel in a diaphragmatic hernia as a cause of pneumothorax, with or without tension physiology, in a patient with a history of trauma is important so that surgical repair can be undertaken without delay.
Tricuspid valve repair for tricuspid valve endocarditis: tricuspid valve "recycling". Tricuspid valve endocarditis traditionally has been treated with either valve excision or valve replacement. To avoid implantation of foreign material in an infected field, we have applied the principles of mitral valve repair to 4 patients with tricuspid valve endocarditis. On preoperative echocardiography, all patients had 3 to 4+ tricuspid regurgitation, evidence of progressive right ventricular enlargement, and mobile vegetations. In each case, up to three quarters of the anterior leaflet was excised en bloc with infected chordae and papillary muscle heads. Surgical procedures included standard quadrangular resection, conversion to a bicuspid valve, and pericardial patch replacement of the anterior leaflet with mobilization of basal chordae to replace resected marginal chordae. On postoperative echocardiography, tricuspid regurgitation and right ventricular dimensions were reduced in 2 of 4 patients in spite of loss of leaflet tissue. All excised valve tissue demonstrated bacteria on Gram stain or culture. Nonetheless, all repaired valves were successfully sterilized without recurrent infections. Tricuspid valve repair can allow eradication of infection with potential for improving valve competency in complicated tricuspid valve endocarditis.
Postoperative hypertension: a prospective, placebo-controlled, randomized, double-blind trial, with intravenous nicardipine hydrochloride. Nicardipine hydrochloride, a new calcium channel blocker for intravenous use, has been compared with placebo in a prospective, randomized, double-blind trial on postoperative hypertension. Of 175 patients who signed preoperative consent, 24 (13.7%) met entry criteria. Hypertension was significantly better controlled in nicardipine-treated patients compared with those receiving placebo (93% versus 30%, p less than 0.05). All the placebo failures were subsequently successfully treated with nicardipine. There were no significant complications in either group. The authors conclude that nicardipine HC1 is an effective, safe therapy for postoperative hypertension. Further studies are now indicated comparing it with other drugs currently used to treat this condition.
The leukocyte count: a predictor of hypertension. In an exploratory study of 1031 persons observed to progress from normotension to essential hypertension and 1031 matched subjects who remained normotensive, the initial leukocyte count (WBC) was found to be related to the development of hypertension, with risk increased 40% (95% confidence interval 12-82%) in persons in the highest as compared to the lowest quartile of WBC. This relationship proved to be largely independent of body mass index, body fat distribution, alcohol and tobacco consumption, and parental history of hypertension. An increased WBC may reflect greater sympathetic tone or may directly increase peripheral vascular resistance by impeding circulation through small blood vessels. If confirmed, this study adds another condition to the growing list for which the WBC is predictive. This simple, cheap test should be considered for inclusion in prospective epidemiological studies of many different diseases.
Elevated plasma beta-endorphin levels in patients with congestive heart failure Recent experimental studies show that the opioid system is important to the pathophysiology of cardiovascular impairment in congestive heart failure. Plasma beta-endorphin levels were measured in 37 patients with congestive heart failure and compared with those of 21 age- and gender-matched normal subjects. The relation of plasma beta-endorphin levels and cardiac function at rest and exercise capacity was assessed in 17 of the patients with dilated cardiomyopathy. Exercise capacity was determined by symptom-limited maximal treadmill exercise with expired gas analysis. Plasma beta-endorphin levels were elevated and correlated with the patients' New York Heart Association functional cardiac status (control: 14.0 +/- 4.4 pg/ml; class II: 17.9 +/- 3.6 pg/ml; class III: 28.3 +/- 8.8 pg/ml; class IV: 46.7 +/- 14.6 pg/ml, mean +/- SD). No relation was found between plasma beta-endorphin levels and left ventricular systolic performance as assessed by M-mode and Doppler echocardiography. Plasma beta-endorphin levels were negatively correlated with cardiac output determined by Doppler echocardiography and positively correlated with systemic vascular resistance (r = -0.733, r = 0.747, respectively, both p less than 0.001), but not correlated with calf blood flow as measured by a plethysmography. A good correlation was found between plasma beta-endorphin levels at rest and exercise capacity. The correlations with peak oxygen consumption, anaerobic threshold, and peak rate-pressure product were r = -0.721, -0.672, and -0.674, respectively (p less than 0.01). The data show that plasma beta-endorphin levels are elevated in patients with congestive heart failure and reflect, to some degree, the severity of the disease.
Lipiodol computerized tomography: how sensitive and specific is the technique in the diagnosis of hepatocellular carcinoma? Computerized tomography (CT) following the intra-arterial injection of Lipiodol (Lipiodol-CT) was performed on 60 patients suspected of having a hepatocellular carcinoma (HCC). Four main patterns of uptake of the Lipiodol within the liver were seen on CT. Of the 14 well circumscribed lesions with dense homogeneous uptake of Lipiodol, 13 were confirmed to be HCCs. Of the 25 lesions with dense patchy uptake of Lipiodol at the periphery and/or in the centre, 19 were confirmed to be HCCs. In 18 patients, in whom only ill defined faint patchy uptake of Lipiodol was present in the liver, or in whom no hepatic uptake was present at all, only one patient was found later to have an HCC. Of the three hypodense lesions in the liver with no Lipiodol uptake, one was found to be necrotic HCC, one a cholangiocarcinoma and one a regenerative nodule. In the diagnosis of HCC, Lipiodol-CT had an overall sensitivity of 97.1%, an accuracy of 88.3% and a specificity of 76.9%. Of the 34 patients with HCC, only 23 were solitary at diagnosis. The size of the HCCs ranged from 0.8 cm to 11 cm in diameter with the median size at 2.2 cm. Eleven of 34 HCCs (32.3%) were resectable. We conclude that, as part of a screening programme for high risk patients. Lipiodol-CT is useful in the early detection of HCCs. The technique also plays an important role in determining whether the tumour should be resected or managed with chemotherapy. By detecting HCCs while still small, the resectability rate can also be improved.
Coronary angioplasty requiring extraordinarily high balloon inflation pressure. Two cases are presented in which extraordinarily high balloon inflation pressures (20 and 17 atm) were required to successfully dilate a saphenous vein graft stenosis and a right coronary artery stenosis. The clinical application of high balloon inflation pressures and balloon selection is discussed.
Breast self-examination in relation to the occurrence of advanced breast cancer. Two hundred nine female enrollees of the Group Health Cooperative of Puget Sound who developed advanced-stage breast cancer during the period 1982-1988 were interviewed about their practice of breast self-examination (BSE), use of other breast cancer screening modalities, and medical and reproductive histories. Each subject's description of how she performed the examination was scored according to her mention of up to 10 recommended BSE techniques. A random sample of 433 women without advanced-stage breast cancer from the same population was interviewed for comparison. Relative to women not practicing BSE, the risk of advanced-stage breast cancer among BSE users was 1.15 (95% confidence interval, 0.73-1.81). Frequency of BSE did not differ between women with advanced-stage breast cancer and control subjects, whether in all subjects or in subgroups defined by age, use of mammography, or frequency of clinical breast examinations. While self-described proficiency in BSE was generally low in both case and control subjects, the small percentage of women reporting more thorough self-examinations, regardless of frequency, had about a 35% decrease in the occurrence of advanced-stage breast cancer compared to women who did not perform BSE. These results suggest that, while carefully performed BSE may avoid the development of some advanced-stage breast cancers, BSE as practiced by most Seattle-area women is of little or no benefit.
Genetic mapping of "Lubag" (X-linked dystonia-parkinsonism) in a Filipino kindred to the pericentromeric region of the X chromosome. "Lubag" is an X-linked disorder causing dystonia and parkinsonism that has only been described in families from the Philippines, principally from the island of Panay. We have established linkage between the disease phenotype "lubag" and DNA markers which span the Xp11.22-Xq21.3 region by using a large Filipino family with 8 affected men in three generations. These DNA markers define an interval of about 20 centimorgans in the pericentromeric region of the X chromosome as the most likely site of the disease locus XDPD (X-linked dystonia-parkinsonism). XDPD has a maximum multipoint log likelihood ratio score (Zmax) of about 4.6 over the interval from Xq12 to Xq21.31 (DXS159-DXYS1X). The co-occurrence of dystonia and parkinsonism in lubag and in other known disorders suggests there may be a common pathogenetic mechanism. Identification of the genetic defect in this family may provide an important clue toward understanding the pathogenesis and pathophysiology of both dystonia and parkinsonism.
Usefulness of labetalol in chronic atrial fibrillation. Beta-adrenergic blocking agents are useful in controlling excessive ventricular rate in chronic atrial fibrillation (AF) but often reduce exercise capacity. To investigate the advantage of labetalol--a unique beta blocker with alpha-blocking property--in chronic AF, 10 patients without underlying structural heart disease were studied with treadmill test, 12-minute walk and 24-hour ambulatory electrocardiographic monitoring. Patients were randomized and crossed over to receive 4 phases of treatment (placebo, digoxin, digoxin with half-dose labetalol, and full-dose labetalol). Exercise durations were 14.1 +/- 1.5, 14.2 +/- 1.5, 16.1 +/- 1.1 and 15.6 +/- 1.1 minutes, respectively, indicating that labetalol did not reduce exercise tolerance. Although digoxin had no advantage over placebo in controlling maximal heart rate (177 +/- 2 vs 175 +/- 3 beats/min), labetalol, both as monotherapy or as an adjunct to digoxin, was advantageous (156 +/- 4 vs 177 +/- 2 beats/min, p less than 0.01, and 154 +/- 4 vs 177 +/- 2 beats/min, p less than 0.01, respectively). The rate-pressure product was consistently lowered by labetalol at rest and during exercise. At peak exercise, the addition of labetalol to digoxin reduced the maximal rate-pressure product achieved from 30,900 +/- 1300 to 24,100 +/- 2,000 mm Hg/min (p less than 0.01) and the maximal rate-pressure product was lowest with full-dose labetalol (22,300 +/- 1,600 mm Hg/min). During submaximal exercise on treadmill or during the 12-minute walk, the combination of labetalol and digoxin produced the best heart rate control, whereas labetalol monotherapy was comparable to digoxin therapy.
Spinal instability secondary to metastatic cancer. Fifty-five patients with severe pain from spinal instability secondary to metastatic cancer were referred to Hope Hospital, none being judged to be in a terminal condition. One patient had too extensive disease for surgery so 54 were treated by 55 spinal stabilisations; 49 obtained complete relief of pain and two had partial relief. There were three failures. Twenty-eight of the patients had clinical evidence of spinal cord or cauda equina compression and were decompressed at the time of stabilisation. Of these, 20 had major recovery of neurological function. Patients with pre-operative evidence of extradural tumour had 'prophylactic' decompression at the time of stabilisation; none of these patients later developed signs of cord or cauda equina compression. The results suggest that alleviation of pain and restoration of mobility are best achieved by segmental spinal stabilisation; a few patients require a combined anterior and posterior stabilisation. Postoperative radiotherapy should be given whenever possible, and the causative tumour should be treated by endocrine or chemotherapy, as indicated.
Multiple gunshot wounds of the head: an anthropological view. A decomposed body was judged at the scene to have two gunshot wounds of the thorax and three of the head. Confirmed at autopsy, the condition of the remains precluded conclusions about the precise nature of the defects. Preparation and reconstruction of the skull disclosed seven large cranial defects and a series of fractures. This preparation allowed the application of well-known principles of gunshot wound analysis. Although the analysis of specific gunshot wound defects is well covered in the literature, there are few examples of the application of gunshot wound principles to complex wound cases. Three entrances and three exits were identified. A seventh defect resulted from bullet passage. Finally, the wounds were sequenced.
Chemoprotective effects of recombinant human IL-1 alpha in cyclophosphamide-treated normal and tumor-bearing mice. Protection from acute toxicity, hematologic effects, development of late mortality, and enhanced therapeutic efficacy. In this study, recombinant human IL-1 alpha (rhIL-1 alpha) was used to protect normal and tumor-bearing BALB/c mice from the acute toxicity caused by lethal doses of cyclophosphamide (Cy) and 5-fluorouracil. Pretreatment of mice for 7 days with 10,000 U/day of rhIL-1 alpha protected 70 to 100% of mice from the acute death induced by lethal doses of both Cy (380 mg/kg) and 5-fluorouracil (250 mg/kg). In contrast, post-treatment of mice with single or multiple doses of rhIL-1 alpha was not chemoprotective. Pretreatment of mice with rhIL-1 alpha increased the acute LD90 of Cy from 380 mg/kg to greater than 500 mg/kg in normal mice, an LD90 dose-modifying effect of at least 1.25, was accompanied by a more rapid recovery from neutropenia and a less severe reduction in the number of bone marrow single lineage monocyte, myeloid, or myelomonocytic colonies. Some of the mice (10 to 50%) that were successfully protected by pretreatment with rhIL-1 alpha died after day 50. These mice consistently presented with extensive pulmonary inflammation and fibrosis at death. Mice bearing murine renal cancer (Renca) were also protected from the acute toxic effects of Cy (450 mg/kg) by pretreatment with rhIL-1 alpha. Renca-bearing mice pretreated with rhIL-1 alpha and either sublethal (300 mg/kg) or lethal (450 mg/kg) doses of Cy exhibited enhanced survival times over those of untreated Renca-bearing mice. Interestingly, the cause of death in Renca-bearing mice that ultimately failed treatment with rhIL-1 alpha plus 300 mg/kg Cy was recurrent tumor, whereas most mice treated with rhIL-1 alpha plus 450 mg/kg Cy had no detectable tumor, although several died from late pulmonary inflammation and fibrosis. Thus, the dose escalation of Cy in rhIL-1 alpha-pretreated mice results in greater antitumor effects of Cy. However, the dose escalation of some cytotoxic agents allowed by the use of myelostimulatory agents can result in late fatal complications not detected in acute toxicity testing.
Cystic hygroma: massive recurrence in adult life. Cystic hygroma is considered a disease of childhood. It may appear for the first time in adult life but recurrence by that stage is rare and may present difficulties in diagnosis due to previous treatment. The authors present a case where early pharmacological intervention may have prevented a potentially life threatening situation. The world literature is also reviewed.
Inconsistent response to divalproex sodium in hemichorea/hemiballism. We report 6 patients with hemichorea/hemiballism of vascular origin who were treated with divalproex sodium (Depakote). Four of 6 responded initially. Marked improvement was seen in 2 patients only and in 1 of these hemiballism recurred despite continuing therapy. Divalproex sodium is not uniformly effective in the treatment of hemichorea/hemiballism.
An extracorporeal perfusion system to bridge pediatric liver transplant candidates. An extracorporeal liver perfusion system was designed to maintain cadaver livers in an oxygenated, normothermic state for bridging procedures for hepatic transplantation. Nonpulsatile high flow and pulsatile low flow blood are supplied to the portal venous (PV) and hepatic arterial (HA) circulations. Controlled low blood flow (5-10 cc/kg [patient]/min) is exchanged between the high flow extracorporeal perfusion circuit (1 cc/g [liver]/min) and the patient. The system was evaluated in perfusions of fresh, excised pig livers (n = 5). The average oxygen consumption was 9 +/- 3 microliters/g/min, and bile production averaged 4.7 microliters/g/hr. Perfusion pressures and flows were normal in both the HA and PV circulations for about 4 hr. Pressures then gradually rose, especially in the HA circulation, causing flow to decrease, with subsequent mottling and discoloration of the liver. Red blood cell, platelet, and white blood cell counts fell continuously. Maintenance of liver function was assessed by clearance of an 80 mg taurocholic acid challenge. An average of 56% of injected acid was cleared from the perfused livers (n = 5) in the first half hour, compared with 90% and 25% for the in situ (n = 3) and unperfused (n = 3) control livers, respectively. The system consistently maintained livers in a moderately well functioning state through the first 4 hr of perfusion. Adequate support of animals with induced hepatic failure must now be demonstrated.