Medicine
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We present a 6-generation kindred of over 221 members, 43 of whom were affected with von Hippel-Lindau (vHL) disease. Through a simple screening protocol, we diagnosed vHL retrospectively in 15 cases, and for the first time in 28, 11 of whom were presymptomatic. We found many complications of vHL in previously diagnosed relatives and in new cases. ⋯ Recommendations are made for family screening, which was economical and effective. Bayesian calculations help to predict risks for genetic counseling. The molecular basis of vHL may soon be found, since it has been linked to DNA markers on the short arm of chromosome 3.
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We evaluated the clinical features of 96 cases of amebic liver abscess and 48 of pyogenic hepatic abscess. Most patients with amebic abscess were young Hispanic males. Those with pyogenic abscess were older, without any ethnic predominance. ⋯ Seven patients with pyogenic abscess died, 5 as a result of hepatic abscess. In 3 of these cases, the diagnosis was unsuspected till autopsy. Improved awareness of this disease may decrease morbidity and mortality from this treatable condition.
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Cardiac myxoma is a true intracardiac neoplasm, which is histologically benign but which on occasion may exhibit behavior suggestive of a true malignancy. It is the most common form of primary cardiac tumor, accounting for 50% of such neoplasms. Seventy-five percent of myxomas are found in the left atrium, typically arising from a stalk attached to the area of the foramen ovale. ⋯ Four cases with unusual manifestations are high-lighted in the text to illustrate the protean manifestations of this potentially curable illness. Non-invasive cardiac imaging is essential to establish the diagnosis and differentiate myxoma from the other more common illnesses it imitates. Echocardiography is highly accurate for its diagnosis, has proved invaluable for the management of these patients, and is the imaging technique of choice for initial evaluation of patients in whom the diagnosis of cardiac myxoma is suspected.
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The medical records of 507 patients with polymicrobial septicemia were examined to determine prognostic and descriptive factors. Over 50% of the episodes occurred in patients with solid tumors and 80% originated during hospitalization. Invasive procedures and immunosuppressive therapy frequently preceded development of polymicrobial septicemia, and infection was often accompanied by shock and pneumonia. ⋯ Overall response among these patients was 50%, with poorest response seen among patients with persistent neutropenia (25%), pneumonia (19%), and gram-negative bacillary infection (46%). Therapy with an antibiotic regimen to which all causative organisms were sensitive was of greatest prognostic significance. Response to appropriate therapy was 58%, whereas only 10% of those who received inappropriate therapy were cured (p less than .0001).
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The classic proposal of intracellular K+ for extracellular H+ exchange as responsible for the hyperkalemia of diabetic ketoacidosis (DKA) has been questioned because experimentally induced organic anion acidosis fails to produce hyperkalemia. It has been suggested, instead, that the elevated serum [K+] of DKA might be the result of the compromised renal function, secondary to volume depletion, that usually accompanies DKA. However, several metabolic derangements other than volume depletion and acidosis, which are known to alter potassium metabolism, also develop in DKA. ⋯ These results indicate that the endogenous ketoacidemia and hyperglycemia observed in DKA, which result primarily from insulin deficit, are the main determinants of increased [K+]p. Since exogenous ketoacidemia and hyperglycemia in the otherwise normal experimental animal do not increase [K+]p, it is postulated that insulin deficit itself may be the major initiating cause of the hyperkalemia that develops in DKA. Renal dysfunction by enhancing hyperglycemia and reducing potassium excretion also contributes to hyperkalemia.