Repository of Research and Investigative Information

Repository of Research and Investigative Information

Zahedan University of Medical Sciences

Adjunctive acetazolamide therapy for the treatment of Bartter syndrome

(2019) Adjunctive acetazolamide therapy for the treatment of Bartter syndrome. Int Urol Nephrol. ISSN 0301-1623

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PURPOSE: Bartter syndrome is a rare hereditary salt-losing tubulopathy caused by mutations of several genes in the thick ascending limb of Henle's loop, characterized by polyuria, hypokalemic metabolic alkalosis, growth retardation and normal blood pressure. Cyclooxygenase inhibitors, potassium-sparing diuretics and angiotensin-converting enzyme inhibitors are currently used to treat electrolyte derangements, but with poor response. Whether treatment with acetazolamide, a carbonic-anhydrase inhibitor, would result in better clinical outcomes is unknown. METHODS: We randomly assigned children with Bartter syndrome in a 1:1 ratio to either receive indomethacin, enalapril, and spironolactone or indomethacin, enalapril, and spironolactone plus acetazolamide once daily in the morning for 4 weeks. After 2 days of washout, participants crossed over to receive the alternative intervention for 4 weeks. The present study examines the serum bicarbonate lowering effect of acetazolamide as an adjunctive therapy in children with Batter syndrome. RESULTS: Of the 43 patients screened for eligibility, 22 (51), between the ages 6 and 42 months, were randomized to intervention. Baseline characteristics were similar between the two groups. Addition of acetazolamide for a period of 4 weeks significantly reduced serum bicarbonate and increased serum potassium levels, parallel with a reduction in serum aldosterone and plasma renin concentration. The 24-h urine volume, sodium, potassium, and chloride decreased significantly. CONCLUSION: Our data define a new physiologic and therapeutic role of acetazolamide for the management of children with Bartter syndrome.

Item Type: Article
Keywords: Acetazolamide Bartter syndrome Children Hypokalemia Metabolic alkalosis
Subjects: WJ Urogenital System > WJ 400-600 Ureter. Bladder. Urethra
Journal or Publication Title: Int Urol Nephrol
Journal Index: Pubmed
Identification Number:
ISSN: 0301-1623
Depositing User: خانم مهدیه رضائی پور

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