Hypokalemic Distal Renal Tubular Acidosis

Patricia G. Vallés, Daniel Batlle*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

41 Scopus citations


Distal renal tubular acidosis (DRTA) is defined as hyperchloremic, non-anion gap metabolic acidosis with impaired urinary acid excretion in the presence of a normal or moderately reduced glomerular filtration rate. Failure in urinary acid excretion results from reduced H+ secretion by intercalated cells in the distal nephron. This results in decreased excretion of NH4 + and other acids collectively referred as titratable acids while urine pH is typically above 5.5 in the face of systemic acidosis. The clinical phenotype in patients with DRTA is characterized by stunted growth with bone abnormalities in children as well as nephrocalcinosis and nephrolithiasis that develop as the consequence of hypercalciuria, hypocitraturia, and relatively alkaline urine. Hypokalemia is a striking finding that accounts for muscle weakness and requires continued treatment together with alkali-based therapies. This review will focus on the mechanisms responsible for impaired acid excretion and urinary potassium wastage, the clinical features, and diagnostic approaches of hypokalemic DRTA, both inherited and acquired.

Original languageEnglish (US)
Pages (from-to)303-320
Number of pages18
JournalAdvances in Chronic Kidney Disease
Issue number4
StatePublished - Jul 2018


  • Acid excretion
  • Distal RTA
  • Growth failure
  • Hyperchloremic metabolic acidosis
  • Hypokalemia

ASJC Scopus subject areas

  • Nephrology


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