Lipoamide dehydrogenase deficiency: a new cause for recurrent myoglobinuria.
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Biomedical subjects
Publications and source records attributed to J Halevy.
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A 39 year-old patient with long-standing diagnosis of pelvic endometriosis had been treated for over twenty years with oral contraceptives (OCs). A year prior to admission to the gynecological ward, an ultrasonographic examination revealed three hepatic lesions, which were not reported in previous liver sonography. These lesions progressed during OC use, over the next six months. Liver function tests were normal. Liver scan, CT and ultrasound imaging techniques supported the diagnosis of solid hepatic tumors. These lesions developed concomitantly to long-term use of OCs, therefore discontinuation of therapy was mandatory. Six months later, the patient was hospitalized due to pelvic pain. She underwent total abdominal hysterectomy and left salpingo-oophorectomy (the right adnexa had been removed years before). Due to the patient's young age, hormone replacement therapy was indicated. The preferred preparation was transdermal estradiol due to the fact that systemic absorption has no hepatic first-pass effect and therefore exerts minimal influence on liver enzymes and functions. Serial ultrasonographic examinations, performed while under treatment with transdermal estradiol, showed complete regression of the hepatic lesions over a period of two years. Our report demonstrates regression of multifocal hepatic tumors despite transdermal estradiol replacement therapy.
A 23-year-old Ethiopian new immigrant presented with a giant lymphangioma extending from the posterior mediastinum through the retroperitoneum, ending as a herniated fluid-filled sac in the inguinal region. Chylous fluid aspirated from within the lymphangioma was cultured positive for Mycobacterium tuberculosis. Considerable regression occurred following 6 months of antituberculous treatment.
Functional adaptation of the remnant intestine after extensive resection of small bowel is well documented. The effects of partial resection of large bowel on the remaining colon have not been characterized. Transepithelial potential (VT), tissue resistance (RT), and short-circuit current (Isc) were measured in vitro across distal colonic tissue of rats three weeks after proximal hemicolectomy with ileotransversotomy and compared to the same parameters measured in the distal colon of control animals. In a second series of experiments, an in vivo perfusion technique was used to measure changes in sodium, potassium, and water transport in distal colon following proximal hemicolectomy. A 2.5-fold increase in VT (P less than 0.01), a 62% increase in RT (P less than 0.001), and a 35% increase in Isc (P less than 0.05) were observed three weeks following hemicolectomy when compared to control animals. A 64% increase in net sodium absorption (P less than 0.025), no significant change in net potassium transport, and a 115% increase in net water absorption (P less than 0.01) were demonstrated in hemicolectomized animals when compared to control. It is concluded that in the rat the distal colon is capable of functional adaptation to increase net sodium and water absorption within three weeks after proximal hemicolectomy. The mechanism responsible for this adaptive process has yet to be defined. Our findings may explain the lack of chronic diarrhea in patients undergoing right hemicolectomy.
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Seizure induced bone fractures in patients with renal osteodystrophy are uncommon. A case is described where multiple bone fractures occurred following convulsions which appeared shortly after haemodialysis.
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Whereas, in chronic type B hepatitis, the therapeutic effect of alpha-interferon has been studied extensively, data on the effect of interferon on the course and prognosis of acute hepatitis B are scarce in the literature. We report a case of acute type B hepatitis complicated by life-threatening extrahepatic manifestations where recombinant alpha-interferon facilitated clinical, biochemical, and serological recovery.
Clinical and laboratory features of 17 patients over 40 years of age (mean age: 55 years) admitted with infectious mononucleosis were compared with those of 17 adolescents (mean age: 13 years) hospitalized with this illness. Elderly patients with infectious mononucleosis were found to run a longer febrile course (13 vs. 7 days, p less than 0.01) and to have a lower peak total white blood cell count (6,600/mm3 vs. 11,000/mm3, p less than 0.001) and a lower incidence of splenomegaly (50% vs. 76%, p less than 0.05), lymphadenopathy (25% vs. 94%, p less than 0.001), and pharyngitis (25% vs. 47%, p less than 0.05), compared with young patients with infectious mononucleosis. Patients in both groups had a high prevalence of abnormal liver function tests. It is concluded that infectious mononucleosis in patients over 40 years of age is not as uncommon as previously reported, and that clinical and laboratory features differ between young and older patients suffering from this disease.
Studies were performed, using a synthetic glucocorticoid, RU 28362, that has negligible binding to aldosterone receptors, to examine whether the effect of glucocorticoids on the electrical properties of rat distal colon involves a specific mechanism or crossover binding to aldosterone receptor sites. Compared with control, RU 28362, in a dose of 70 micrograms.100 g body wt-1.day-1, increased transepithelial potential differences (VT) twofold and the equivalent short-circuit current (ISC) threefold; epithelial resistance (RT) was reduced 25%. These changes were qualitatively similar to, but less than, the changes induced by the same dose of aldosterone. The effects of dexamethasone in the same dose were indistinguishable from the changes caused by aldosterone. The results of cell-impalement studies corresponded to whole epithelial changes; RU 28362 induced a small increase in basolateral potential difference, from -43 +/- 2 to -45 +/- 1 mV, and a fall in apical potential difference, from 40 +/- 2 to 36 +/- 1 mV, but these effects on cell membrane voltage were less than caused by either aldosterone or dexamethasone. In contrast to the marked inhibitory action of amiloride to reduce VT in aldosterone- and dexamethasone-treated animals, this sodium channel blocker had no effect on VT in RU 28362-treated animals. Similarly, while spironolactone caused marked inhibition of the changes in VT, RT, and ISC induced by aldosterone, the inhibitory action on tissue from RU 28362-treated animals was slight or absent. These results suggest that glucocorticoids have a specific aldosterone receptor-independent action on the electrical properties of the large intestine.
Severe hypophosphatemia (serum phosphorus less than or equal to 0.48 mmol/L [less than or equal to 1.5 mg/dL]) was found in 120 patients admitted to a major university hospital, during a period of 16 months. Fifty-one patients (42.5%) developed hypophosphatemia postoperatively. Medications known to precipitate hypophosphatemia were a causative factor in 82% of the patients, with glucose administered intravenously, antacids, diuretics, and steroids being the most common agents associated with profound hypophosphatemia. Gram-negative septicemia was observed in 16 patients, and it was the second most common cause of severe hypophosphatemia. The mortality rate was 20% in patients with a serum phosphorus concentration between 0.36 and 0.48 mmol/L (1.1 and 1.5 mg/dL) (group A) and 30% in patients with a serum phosphorus concentration of less than or equal to 0.32 mmol/L (less than or equal to 1.0 mg/dL) (group B). The cause of death and its temporal association with the lowest observed values of phosphorus concentration indicate that severe hypophosphatemia might be a contributory factor to mortality. Our data indicate that severe hypophosphatemia in hospitalized patients is the result of a combination of factors. Surgery, followed by a period of fasting with intravenous administration of glucose, and gram-negative septicemia are the most common causes.
Severe life-threatening hypokalemia (serum potassium less than or equal to 2.5 mmol/l) was found in 0.03% of serum biochemical profiles within 13 years in a major university hospital in Israel. Out of 130 patients with hypokalemia of this magnitude, 84 (65%) were females and the mean age was 64 years. A combination of iatrogenic factors, including the administration of intravenous fluids with insufficient or no potassium (K) replacement and the use of K-depleting medications and insulin, was responsible for the hypokalemia in 68% of the patients. Gastrointestinal loss of K was the main cause of severe hypokalemia in 22% of the patients. It is concluded that severe hypokalemia in hospitalized patients is commonly the result of multiple iatrogenic factors and, therefore, can be prevented by frequent monitoring of serum K and appropriate K supplementation.
These experiments were performed to determine whether aldosterone-dependent effects in apical and basolateral membranes could be temporarily dissociated and whether aldosterone increases the maximal capacity, or maximum turnover rate of the sodium pump. Tissue from rat distal colon exposed to the action of high plasma levels of aldosterone for 4 h, 24 h and 7-10 days was compared to control tissue in a modified Ussing chamber, before and after addition of nystatin to the mucosal solution to remove the apical barrier to the cell entry of sodium. The maximum turnover rate of the sodium pump was represented by the equivalent short circuit current, Isc, after the addition of nystatin. After 4 h of aldosterone basal Isc increased 2.6-fold above control (43 +/- 34 microA.cm-2, p less than 0.05) and transepithelial PD, VT, increased 2-fold over control (-7.0 +/- 5.5 mV, lumen negative, p less than 0.05). Administration of aldosterone for 24 h caused further marked increases in Isc (11-fold) and a fall in RT to 50% of control. Similar changes were observed after 7-10 days on low sodium diet, and at all time intervals the changes were completely inhibited by amiloride (10(-4)M). Although aldosterone stimulated Isc within 4 h, there was no further increase in Isc in the presence of nystatin during the same time period compared to the control post-nystatin Isc of 419 +/- 79 microA.cm-2. However, after 24 h aldosterone caused approximately a 40% increase in the maximal turnover rate of the sodium pump, which persisted for 7-10 days.(ABSTRACT TRUNCATED AT 250 WORDS)
Clinically significant liver involvement in systemic amyloidosis, especially with cholestasis, is rare. We report a case of primary amyloidosis with severe intrahepatic cholestasis leading to terminal liver failure. The present case is the first of its kind reported involving Lambda light chains only as the associated paraprotein. Conventional treatment and a therapeutic trial with dimethyl sulphoxide were unsuccessful.
Dietary sodium depletion with elevated aldosterone levels induces electrogenic, amiloride-sensitive sodium absorption and inhibits electroneutral sodium chloride absorption in the rat distal colon. To assess the role of aldosterone in the production of these changes, unidirectional 22Na and 36Cl fluxes were performed under voltage clamp conditions across isolated distal colonic mucosa of rats given continuous aldosterone infusions for up to 12 days. Aldosterone infusion for 7-12 days produced identical changes in both electrogenic sodium absorption and electroneutral sodium chloride absorption compared with dietary sodium-depleted animals. In contrast, aldosterone at 24, 48, and 72 h produced varying changes in ion transport: electrogenic sodium absorption progressively increased, whereas electroneutral sodium chloride absorption was initially augmented but then inhibited. Aldosterone induced active potassium secretion, demonstrated by a reversed short-circuit current after addition of amiloride, in all experimental groups. These results demonstrate that the changes in ion transport observed in sodium-depleted animals are produced by aldosterone, and that aldosterone not only stimulates electrogenic sodium absorption and potassium secretion but in a time-dependent manner both stimulates and inhibits electroneutral sodium chloride absorption.
Hospital utilization by crisis-ridden chronic mentally ill persons living in the community was studied after their participation in a network therapy program, which convenes a social network of relatives and friends to provide continuing emotional and practical support for mentally ill individuals and their families. The study compared the number of psychiatric hospitalizations and total days hospitalized for 20 network therapy participants and 20 nonparticipants before and after referral to the program. In a follow-up period ranging from six months to two years, the number of hospitalizations fell by 74 percent for participants and by 19 percent for nonparticipants. The total number of days hospitalized decreased by 76 percent for participants, compared with a 112 percent increase for nonparticipants. The results suggest that participants benefit from network therapy and that its effect is enduring.
A case of cholestatic jaundice after cloxacillin treatment is presented. Hepatotoxicity due to the drug was confirmed by inadvertent rechallenge and strongly positive tests for macrophage migration inhibitory factor and mast cell degranulation. The recognition of cholestatic hepatotoxicity due to oxacillin derivatives should help to avoid unnecessary invasive procedures in the evaluation of this reversible condition.