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.