Bartter's Syndrome without Hyperplasia of the Juxtaglomerular Apparatus, Treated with Indomethacin

Abstract

ABSTRACT. The present report describes a case of potassium‐wasting nephropathy with the physiological and endocrinological findings that are typical for Bartter's syndrome (BS). However, the renal juxtaglomerular apparatus shawed no hyperplasia at two renal biopsies two years apart. The short‐term (9 days) effect of indomethacin in combination with spironolactone was a suppression of hyperreninemia and hyperaldosteronism and an increase in vascular sensitivity to angiotensin II associated with potassium and sodium retention. Subsequently, on indomethacin alone, potassium balance was obtained on a lower level with persistent hypokalemia and persistent renal potassium leakage. Hypokalemia persisted during long‐term (9 months) treatment with indomethacin despite normalization of the activity of the renin‐aldosterone system. The results indicate that indomethacin as long‐term treatment may be ineffective in maintaining a normal potassium balance in BS.

Pediatric Bartter syndrome: The therapeutic challenge

“…They also reported that infusion of albumin was associated with a rapid increase in serum potassium and a reduction in aldosterone secretion rate [8]. The work of Kornerup and colleagues suggested that longterm treatment with indomethacin may be ineffective in maintaining normal potassium balance in Bartter syndrome [9]. Konomi, et al, reported the occurrence of pseudotumor cerebri in Bartter syndrome during indomethacin therapy [14].…”

“…The use of indomethacin has been reported by many authors including Verberckmoes, et al, Zancan and colleagues, Littlewood and colleagues, Proesmans and colleagues, Dequiedt, et al, Kornerup and colleagues, Konomi, et al, Valles, et al, Floret, et al, Favre, et al, and Storm and colleagues[1,3,[9][10][11][12][13][14][15][16][17].…”