International Urology and Nephrology

, Volume 52, Issue 1, pp 121–128 | Cite as

Adjunctive acetazolamide therapy for the treatment of Bartter syndrome

  • Mojgan Mazaheri
  • Farahnak AssadiEmail author
  • Simin Sadeghi-Bojd
Nephrology - Original Paper



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.


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.


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.


Our data define a new physiologic and therapeutic role of acetazolamide for the management of children with Bartter syndrome.


Acetazolamide Bartter syndrome Children Hypokalemia Metabolic alkalosis 



Authors are grateful to the patients and their families for participating in this study. The authors would also like to thank the nursing staff of the participating medical centers for their contributions and administrative support during this study period.

Authors’ contributions

MM contributed significantly to analysis and interpretation of data, drafting the article. FA contributed to the design and conception of the study, analysis and interpretation of data and providing intellectual content of critical importance to the work submitted. SS contributed to acquisition, analysis, and interpretation of data. All authors read and approved the final version of the work to be published and accept the responsibility of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.


This research did not receive any specific grant from funding agencies in the public, commercial, or non-for-profit sectors.

Compliance with ethical standards

Conflict of interest

Authors acknowledge that the submitted article is original, has not been published previously in whole or part, and is not currently under review elsewhere. Authors further declare that there is no financial support or relationships that may pose conflict of interest regarding the content of this article.

Ethical approval

All procedures performed in this study were in accordance with ethical standards of the appropriate institutional research ethics committee on human experimentation and has been performed in accordance with the ethical standards as laid in the 1964 Declaration of Helsinki and its later amendments as revised in 2013 and followed the CONSORT 2010 checklist guidelines for reporting the randomized clinical trial (Clinical Trial Registration Reference: IR.ZAUMS.REC.1397.492).

Informed consent

Written parental informed consent was obtained for all participants prior to the study.


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Copyright information

© Springer Nature B.V. 2019

Authors and Affiliations

  1. 1.Department of Pediatrics, Section of NephrologySemnan University of Medical ScienceSemnanIran
  2. 2.Department of Pediatrics, Division of NephrologyRush University Medical CenterChicagoUSA
  3. 3.Department of Pediatrics, Division of NephrologyZahedan University of Medical SciencesZahedanIran

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