Gynaecomastia, neurofibromatosis and breast cancer.

Gynaecomastia commonly affects pubertal boys. We report a rare case of bilateral ductal carcinoma in situ found incidentally after breast reduction surgery in a young man with neurofibromatosis.

Postural hemodilution in nephrotic edema: a cause of spurious hemorrhage after renal biopsy.

A patient with massive edema caused by nephrotic syndrome showed a nine-point decline in hematocrit after an otherwise uneventful renal biopsy. Investigation showed no evidence of hemorrhage. The decrease in hematocrit was shown to be dilutional, occurring as a result of reabsorption of edema fluid into the vascular space that occurred during the patient's prolonged recumbency after the biopsy. The phenomenon was later reproduced in this patient under study conditions. The implications of this phenomenon are discussed in light of the pathophysiology of edema formation in the nephrotic syndrome.

Bartter's syndrome: the unsolved puzzle.

Bartter's syndrome is a congenital abnormality characterized by metabolic alkalosis [corrected], hyperreninemic hyperaldosteronism, and hypokalemia. Most patients present early in life with symptoms such as muscle weakness and polyuria, which may be attributed to potassium depletion. Despite the hyperaldosteronism, the patients tend to be normotensive, which is at least partially explained by vascular hyporesponsiveness to pressor hormones. Numerous studies have documented increased renal excretion of prostaglandins. Several different patterns of aberrant renal ion transport have been observed in patients with the syndrome, suggesting that it actually may represent a family of related but distinct tubular disorders. Therapeutic approaches to Bartter's syndrome include potassium supplementation, prostaglandin synthesis inhibitors (nonsteroidal anti-inflammatory agents), aldosterone antagonists, and converting enzyme inhibitors. During the first two decades following its initial description, Bartter's syndrome was the focus of widespread interest, based on the likelihood that its investigation might provide insight into the normal functioning of the renin-angiotensin-aldosterone and prostanoid hormone systems. During the past decade, however, little additional progress has been made in Bartter's syndrome, and its patho-physiology remains poorly understood.

Budd-Chiari syndrome in autosomal dominant polycystic kidney disease: a complication of nephrectomy in patients with liver cysts.

We report two patients with autosomal dominant polycystic kidney disease (ADPKD) who developed the Budd-Chiari syndrome following bilateral nephrectomy. Both patients had massive cystic enlargement of the liver. Neither had any other identifiable risk factors for the Budd-Chiari syndrome. We suggest that removal of the kidneys may predispose toward anatomic obstruction of the inferior vena cava or hepatic veins by liver cysts. Nephrectomy should be approached cautiously in ADPKD patients with extensive involvement of the liver by cysts.

Potassium homeostasis with indomethacin therapy in normal subjects.

In an attempt to delineate effects of prostaglandin (PG) synthesis inhibition on potassium metabolism in normal subjects, we challenged 13 young, healthy volunteers with a potassium chloride infusion before and after a 4-day course of indomethacin (25 mg orally, three times a day). The plasma potassium level was monitored at 10-minute intervals throughout the 50-minute infusion and for a total of 180 minutes. The maximal increment in plasma potassium level was 0.82 +/- 0.07 mmol/L (mEq/L) in the untreated state, and 0.86 +/- 0.08 mmol/L with indomethacin treatment. The basal potassium level before infusion was higher in the indomethacin-treated than the control state (3.83 +/- 0.07 v 3.68 +/- 0.07 mmol/L; P less than 0.01). Urinary potassium excretion over the 3-hour study period equalled the potassium load administered, and was unaffected by indomethacin therapy. Indomethacin did not alter insulin or aldosterone levels during the study. PGE2 excretion over the 3 hours was lower in the indomethacin than the control phase, although it was higher than normal in both phases. In an additional experiment, the comparative effects of a saline versus saline-potassium infusion on PG excretion were studied. No differences were seen between the excretion patterns of PGE2 or 6-keto-PGF1a with the two infusions. We conclude that (1) although basal serum potassium level is slightly higher in healthy young people during indomethacin treatment, there is little effect on handling of an acute potassium load; (2) the aldosterone response to hyperkalemia is PG-independent; (3) urinary PG excretion increases in response to a saline-based infusion, but the effect is not enhanced by acute potassium loading.

Evidence that circulating 6keto prostaglandin E1 causes the platelet defect of Bartter's syndrome.

Bartter's syndrome is associated with activation of prostaglandin metabolism. In the present study we provide several lines of evidence that a circulating metabolite of prostacyclin, 6ketoPGE1 is responsible for a defect in platelet function present in patients with Bartter's syndrome. In platelet aggregometry studies, plasma from patients contained platelet inhibitory activity which was fully neutralized by coincubation with antibody directed against 6ketoPGE1. Fractionation of lipophilic extracts of plasma by high pressure liquid chromatography yielded a platelet inhibitory fraction which comigrated with authentic 6ketoPGE1 and was neutralized by anti 6ketoPGE1 antibody. Lastly, direct measure of the plasma concentration of 6ketoPGE1 by specific radioimmunoassay indicates a 2-fold increase in patients with Bartter's syndrome (133 +/- 9.1 vs 60.7 +/- 12.3 picograms/ml; p less than 025). These studies provide firm evidence that the platelet dysfunction present in patients with Bartter's syndrome is attributable to an increase in the plasma concentration of 6ketoPGE1. In addition, these data provide further evidence in support of the centrality of activation of prostaglandin metabolism in the pathophysiology of Bartter's syndrome.

Mesangial immunoglobulin A deposits in minimal change nephrotic syndrome: a report of an older patient and review of the literature.

A 57-year-old patient with a history of monoclonal immunoglobulin A (IgA) gammopathy developed idiopathic nephrotic syndrome. Renal biopsy showed minimal glomerular changes with predominant glomerular mesangial IgA. The association of glomerular mesangial IgA with otherwise typical minimal change nephrotic syndrome has been noted before, and the literature concerning this combination of findings is reviewed. The patient herein described represents one of the two oldest patients yet reported with this syndrome and raises questions about the relationship between minimal change disease and IgA nephropathy. Severe proteinuria (and even the nephrotic syndrome) is not necessarily the harbinger of a poor prognosis in IgA nephropathy if the glomerular morphology is otherwise consistent with minimal change nephrotic syndrome. Such patients should be treated in a fashion similar to those with minimal change nephrotic syndrome. The significance of the IgA gammopathy in the pathogenesis of this case is unknown.