Long-term outcome of Bartter syndrome in 54 patients: A multicenter study in Korea.

ABSTRACT:

Introduction

Bartter syndrome (BS) is a rare salt-wasting tubulopathy caused by mutations in genes encoding sodium, potassium, or chloride transporters of the thick ascending limb of the loop of Henle and/or the distal convoluted tubule of the kidney. BS is characterized by polyuria, failure to thrive, hypokalemia, metabolic alkalosis, hyperreninemia, and hyperaldosteronism. Potassium and/or sodium supplements, potassium-sparing diuretics, and nonsteroidal anti-inflammatory drugs can be used to treat BS. While its symptoms and initial management are relatively well known, long-term outcomes and treatments are scarce.

Methods

We retrospectively reviewed 54 Korean patients who were clinically or genetically diagnosed with BS from seven centers in Korea.

Results

All pati

SUBMITTER: Choi N 

PROVIDER: S-EPMC10040751 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature