Editorial Review

Distal Renal Tubular Acidosis Syndromes: A Pathophysiological Approach
Mitchell L. Halperin, Marc B. Goldstein, Robert M.A. Richardson, Bobby J. Stinebaugh

Departments of Medicine, St. Michael's Hospital and Toronto General Hospital, University of Toronto, Ont., Canada, and Baylor College of Medicine, Houston, Tex., USA

Address of Corresponding Author

Am J Nephrol 1985;5:1-8 (DOI: 10.1159/000166895)

  Key Words

• Acid base
• Ammonium
• Net acid excretion
• Renal tubular acidosis
• Metabolic acidosis
• Kidney

  Abstract

Ammonium is the most important component of renal acid excretion. A reduced rate of ammonium excretion is the common feature of the group of diseases called distal renal tubular acidosis. We have presented an alternative approach to patients with distal acidification defects based upon the pathophysiology of these disorders. Accordingly, the purpose of this review is to describe a revised classification based on our current understanding of collecting duct hydrogen ion secretion and ammonium addition to the lumen of the distal nephron. We have subdivided these defects into four groups: disorders of the collecting duct proton pump (pump defects); failure to generate and/or maintain an appropriate electrical gradient to favor hydrogen ion secretion (voltage defects); back-leak of hydrogen ions across an abnormally permeable collecting duct membrane (gradient defects), and diminished availability of NH₃ in this nephron segment (NH₃ defects). These four subtypes can be identified by measuring the urine pH and PCO₂ under appropriate circumstances and evaluating the renal excretion of ammonium and potassium.

Copyright © 001\ S. Karger AG, Basel

  Author Contacts

Dr. M.L. Halperin, Laboratory 1, Research Wing, St. Michael's Hospital, 38 Shuter Street, Toronto, Ont. M5B 1A6 (Canada)

  Article Information

Received: May 15, 1984
Accepted: June 18, 1984
Published online: October 24, 2008
Number of Print Pages : 8