BARTTER-SYNDROME REVISITED

Bartter syndrome is characterized by hyperreninemic hyperaldosteronism with hypokalemic, hypochloremic metabolic alkalosis, but with normal blood pressure. The precise mechanism and underlying defect are uncert ain; however much attention has been focused on defective chloride tra nsport at the thick ascending limb of the loop of Henle as the cause f or excessive chloride and potassium losses. A putative mutation in the angiotensin II receptor has been proposed to cause this complex in so me patients. The pathophysiology and treatment of Bartter syndrome are discussed as is the differential diagnosis of similar complexes that may be related: Gitelman syndrome and hyperprostaglandin E syndrome. I n addition, the differential diagnoses of hyperaldosteronism and metab olic alkalosis are reviewed. Finally, the outcome of a patient with Ba rtter syndrome following renal transplantation is presented. The case provider insight into the mechanism of Bartter syndrome, indicating an Intrinsic renal defect.