[A patients with chronic renal failure due to Bartter's syndrome]

A male patient with hyperplasia of the juxtaglomerular apparatus, hypokalemia, an associated increase in plasma renin activity and hyperaldosteronism without hypertension was diagnosed as having Bartter's syndrome at the age of three. He was treated with spironolactone, indomethacin and potassium supplements. However hypokalemia, increased plasma renin activity and hyperaldosteronism persisted. Proteinuria was observed at the age of fifteen. Because of the gradual reduction of his renal function, the patient was admitted to our hospital and underwent hemodialysis at the age of twenty-one. He was normotensive. Serum potassium was within the normal range. Plasma renin activity and aldosterone concentration levels were still elevated. Renal biopsies, which had been carried out four times throughout his treatment, revealed an increase in sclerosis of the glomerular mesangial region, and interstitial fibrosis in proportion to narrowing of the small arteries in association with the juxtaglomerular hyperplasia. These findings suggested that changes in the glomeruli and interstitium of the kidney were secondary to long-standing hypokalemia, stenosis of the small arteries and the effects of the prescribed drugs for Bartter's syndrome.