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MINI REVIEW article

Front. Endocrinol.
Sec. Renal Endocrinology
Volume 15 - 2024 | doi: 10.3389/fendo.2024.1411317
This article is part of the Research Topic Gastrointestinal and Hepatic Involvement in Cystic Fibrosis in the era of CFTR Modulator Therapies View all articles

Metabolic Alkalosis in Cystic Fibrosis: From Vascular Volume Depletion to Impaired Bicarbonate Excretion

Provisionally accepted
Manoocher Soleimani Manoocher Soleimani 1,2*
  • 1 Medicine/Nephrology, University of New Mexico, Albuquerque, OH, United States
  • 2 New Mexico Veterans Health Care Center, Albuquerque, NM, United States

The final, formatted version of the article will be published soon.

    Cystic fibrosis (CF) is the most common life-threatening genetic disease in the United States and among people of European descent. Despite the widespread distribution of the cystic fibrosis transmembrane conductance regulator (CFTR) along kidney tubules, specific renal phenotypes attributable to CF have not been well documented. Recent studies have demonstrated the downregulation of the apical Cl -/HCO3 -exchanger pendrin (Slc26a4) in kidney B-intercalated cells of CF mouse models. These studies have shown that kidneys of both mice and humans with CF have an impaired ability to excrete excess HCO3 -, thus developing metabolic alkalosis when subjected to excess HCO3 -intake.The purpose of this minireview is to discuss the latest advances on the role of pendrin as a molecule with dual critical roles in acid base regulation and systemic vascular volume homeostasis, specifically in CF. Given the immense prevalence of vascular volume depletion, which is primarily precipitated via enhanced chloride loss through perspiration, we suggest that the dominant presentation of metabolic alkalosis in CF is due to the impaired function of pendrin, which plays a critical role in systemic vascular volume and acid base homeostasis.

    Keywords: Hypokalemia, Barter-like Syndrome, Diabetes Mellitus; Nephrotoxicity, Acute Kidney Injury, Kidney Tubules

    Received: 02 Apr 2024; Accepted: 23 Jul 2024.

    Copyright: © 2024 Soleimani. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Manoocher Soleimani, Medicine/Nephrology, University of New Mexico, Albuquerque, 45267, OH, United States

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