Bartter's syndrome comes of age.

Abstract

In 1962, Bartter and co-workers1 published a description of two patients presenting with a new syndrome characterized by hypokalemia, metabolic alkalosis, hyperaldosteronism with normal blood pressure, decreased pressor responsiveness to infused angiotensin II, and hyperplasia of the juxtaglomerular complex. Since then, many reports, both in children and in adults, have appeared in the literature under the heading of “Bartter's syndrome.” It is nowadays evident that this term does not represent a unique entity but encompasses a variety of disorders of renal electrolyte transport all characterized by a biochemical picture of hyperreninemic hypokalemic metabolic alkalosis. At the present time this ensemble of patients can be divided into three different genetic and clinical entities2: 1. Neonatal (or antenatal) Bartter's syndrome is observed in newborn infants and is characterized by polyhydramnios, premature delivery, life-threatening episodes of fever and dehydration during the early weeks of life, growth retardation, hypercalciuria, and early-onset nephrocalcinosis. The inclusion of these neonatal cases under the global heading of Bartter's syndrome has been a matter of discussion and Seyberth et al3 have proposed the term “hyperprostaglandin E syndrome” to distinguish them from other cases of Bartter's syndrome. Recent molecular biology findings have clearly demonstrated …