Pheochromocytoma in von Hippel-Lindau disease: clinical presentation and mutation analysis in a large, multigenerational kindred.

The clinical presentation and characterization of the mutation in members of a large kindred with von Hippel-Lindau disease (VHLD) and pheochromocytoma were examined. Twenty-five proven cases of VHLD occurring in four generations of a large kindred have been followed since 1964, and pheochromocytoma has occurred in 17. Symptoms of pheochromocytoma developed at an early age, on average at 12.

Circulating dihydroxyphenylglycol and norepinephrine concentrations during sympathetic nervous system activation in patients with pheochromocytoma.

Although increased plasma norepinephrine (NE) concentrations mediate vasoconstriction during episodic hypertension and hypertensive crises in patients with pheochromocytoma (Pheo), the precise origin of this circulating NE (tumor or sympathetic nerves) is not known. Dihydroxyphenylglycol (DHPG), a deaminated metabolite of NE, is formed principally in sympathetic nerve endings. Under basal conditions, plasma NE and DHPG concentrations correlate closely, and during sympathetic nervous system activation, both plasma NE and DHPG concentrations increase.

Disparate urinary catecholamine patterns in secondary hypertension due to unique sequential development of pheochromocytoma and neuroblastoma.

A 43-year-old white woman had a pheochromocytoma removed from her left adrenal gland, and one year later she developed a new left upper abdominal mass that was found to be a neuroblastoma. On both occasions, urinary vanillylmandelic acid level was elevated. However, urinary norepinephrine and epinephrine levels were increased only during the pheochromocytoma episode, while the urinary homovanillic acid level was elevated only when neuroblastoma developed.

Insertion of subclavian hemodialysis catheters in difficult cases: value of fluoroscopy and angiographic techniques.

Double-lumen hemodialysis catheters designed to be placed via a subclavian vein approach have gained rapid acceptance over the past several years. Several studies have shown a significant rate of subclavian vein stenosis or occlusion after placement of these catheters. A large number of these patients require repeat placement of catheters with access often becoming increasingly difficult to obtain.

Rabies pre-exposure prophylaxis using intradermal human diploid cell vaccine: immunologic efficacy and cost-effectiveness in a university medical center and a review of selected literature.

The authors studied the antigenicity of intradermal human diploid cell rabies vaccine administered to 40 laboratory workers considered to be at-risk at the University of Virginia Medical Center. A 1-year postvaccination serology was determined for 20 of those 40, all of whom demonstrated an antirabies titer greater than or equal to 1:50 by the raped fluorescent focus inhibition test. By 2 years' postvaccination, 5 of 40 subjects had "unprotective levels" (less than 1:5), whereas 35 had titers greater than or equal to 1:5, and none had a titer greater than or equal to 1:50.

Increased uptake and accumulation of catecholamines by red blood cells in phaeochromocytoma.

Red blood cell (RBC) and plasma catecholamine content were determined by high performance liquid chromatography in 18 patients suspected of having phaeochromocytoma on the basis of clinical manifestations, especially hypertension and laboratory findings. Eight normotensive health individuals served as controls. The fivne patients with tumours had strikingly greater RBC catecholamine concentrations (C) than in plasma and reached CRBS/Cplasma ratios approximating 5, significantly different from the ratios in controls and in hypertensive patients without tumours.

Catecholamine-secreting infratemporal fossa paraganglioma.

Extraadrenal paragangliomas are tumors of the paraganglion system, usually arising from the carotid bodies, the glomus jugulare, or the glomus tympanicum. These tumors are capable of secreting catecholamines which can cause severe hypertensive crises. This paper reports a case of a patient who suffered a cerebral vascular accident due to hypertension resulting from a catecholamine-secreting infratemporal fossa paraganglioma.

Pheochromocytoma: diagnosis, localization, and treatment.

Pheochromocytoma is curable in 90% of cases, yet its diagnosis and localization are among the most challenging problems in clinical medicine. Although only 10% of these tumors are malignant, others may be lethal because of excessive circulating catecholamines. The clinical hallmark of pheochromocytoma is hypertension, but some patients are normotensive and may even be hypotensive.

Biorhythms of the nonpregnant human uterus.

Uterine activity was monitored continuously for periods of three or five days in two subjects. Biorhythms in the spontaneous variations of intrauterine pressure and frequency of contraction were demonstrated in both phases of the menstrual cycle. Statistically, the patterns conformed to 24-hour Fourier curves, with higher values synchronized by the periods of light.

Cutaneous histoplasmosis after renal transplantation.

A 21-year-old woman with a renal allograft presented with cellulitis of the right foot as the first manifestation of disseminated histoplasmosis. Disseminated histoplasmosis is more common in immunosuppressed hosts, and there is a trend toward more frequent cutaneous manifestations in transplant recipients (47%). Biopsy of cellulitis in these patients may lead to early diagnosis and curative amphotericin therapy.

Familial pheochromocytoma, hypercalcemia, and von Hippel-Lindau disease. A ten year study of a large family.

Long-term epidemiological and laboratory studies were carried out in a kindred with familial pheochromocytoma associated with von Hippel-Lindau disease. Thirteen members were affected by the syndrome and the trait appears to be transmitted in an autosomal dominant fashion. Of 13 patients, 7 had pheochromocytoma alone.

Study of chemical sympathectomy in endotoxin-induced lethality and fibrin depostion.

Shock and the generalized Shwartzman reaction are well known features of endotoxin which have been shown to involve the sympathetic nervous system. The mechanism of sympathetic nervous system involvement with endotoxin injection was studied in rabbits chemically sympathectomized with 6-hydroxydopamine. Endotoxin, in doses producing a spectrum of morbidity and mortality in normal rabbits, was administered i.

Nephrotoxic nephritis in rabbits. The role of the sympathetic nervous system.

The sympathetic nervous system and catecholamines play a major role in fibrin deposition in organs in rabbits after endotoxin administration. Glomerular fibrin deposition is also a key factor in the pathogenesis of nephrotoxic nephritis in rabbits, but the role of the sympathetic nervous system in this type of fibrin deposition has not been defined. We investigated sympathetic nervous system involvement in nephrotoxic nephritis using a model of isolated chemical sympathectomy with 6-hydroxydopamine.

Catecholamine uptake, accumulation, and release in acute porphyria.

Hypertension and tachycardia are well known features of acute porphyria and have been shown to be related to increased circulating catecholamines. The mechanism by which circulating catecholamines are increased was studied using the isolated perfused rat heart and human platelets as a model of adrenergic neuronal function. It was found that neither delta-aminolevulinate (ALA) nor porphobilinogen (PBG) blocked uptake or caused release in the isolated perfused rat heart.

Avasucular necrosis of pheochromocytoma followed by spontaneous remission.

Following an acute spontaneous hypertensive crisis and shock a patient with pheochromocytoma was found to have an exceedingly high catecholamine excretion rate. After this episode, the patient remained normotensive and urinary excretion of catecholamines returned to normal. During surgery, a large pheochromocytoma was found and removed that showed avascualr necrosis.

Close monitoring is essential during isoniazid prophylaxis.

A three-year prospective study during the entire course of preventive therapy with isoniazid (INH) for recently infected hospital employees was undertaken to evaluate the risk of developing INH-related hepatic injury. Results of clinical and laboratory tests at 1, 2, 6, and 12 months were correlated to ascertain the significance and value of monitoring procedures. Thirty-two (32%) of 100 employees developed elevated SGOT levels: one (1%) patient developed clinical hepatitis in the first month of therapy; four (4%) subjects with SGOT elevations (82-378 units) became clinically symptomatic at one to four months and INH was discontinued; one (1%) individual with minimal increases in serum bilirubin and SGOT levels was symptomatic at eight months and the drug was stopped.

Therapy of the idiopathic nephrotic syndrome with alternate day steroids.

Eighty-one adult patients with the idiopathic nephrotic syndrome were treated with prednisone, 60 to 120 mg, on alternate days. Treatment was continued with diminishing drug doses for up to 10 years. Biopsy specimens were categorized as showing lipoid nephrosis 36 per cent, focal sclerosis 12 per cent, diffuse proliferative 22 per cent and membranous nephropathy 30 per cent.

Reversible renal failure from isolated granulomatous renal sarcoidosis.

A patient presented with a 6 month history of progessive renal failure. Serum creatinine was 7.1 mg/100 ml, creatinine clearance 4 ml/minute and hematocrit 22%.

Minimizing tuberculosis risk to hospital personnel and students exposed to unsuspected disease.

A 15-month prospective study of the personnel and students exposed to initially unsuspected active cases of tuberculosis was undertaken to define the risk of their acquiring infection. Eight of 484 (1.65%) personnel exposed to 17 initially unsuspected tuberculosis patients and 5 of 2013 (0.