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Distal renal tubular acidosis
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Distal renal tubular acidosis

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Contributors: Abhijeet Waghray MD, Paritosh Prasad MD
Other Resources UpToDate PubMed


Renal tubular acidosis (RTA) can be classified into 3 types: proximal, distal, and hyperkalemic (hypoaldosteronism). Distal (type 1) RTA leads to a non-anion gap metabolic acidosis due to impaired distal acidification of urine. Causes of distal RTA can be divided into primary lesions (hereditary gene mutations) or secondary (eg, autoimmune, nephrotoxins, medications).
  • In adults, the most common cause is autoimmune disease, eg, Sjögren syndrome or rheumatoid arthritis, or hypercalciuria. In otherwise healthy adults with unexplained distal RTA, diagnosis of Sjögren syndrome should be considered.
  • In children, hereditary causes of distal RTA are most common and are due to genetic mutations in the basolateral chloride-bicarbonate exchanger and in the apical hydrogen-ATPase. The autosomal recessive form presents in infancy, whereas the autosomal dominant form typically presents in adolescence or adulthood.
  • Both ifosfamide and toluene ingestion can cause distal RTA in children and adults.
Hereditary distal RTA will manifest in children while secondary forms of distal RTA most often manifest in adults.


N25.89 – Other disorders resulting from impaired renal tubular function

236461000  – Distal renal tubular acidosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Chronic diarrhea – Urine pH may be inappropriately elevated in this setting; however, the urine anion gap should be negative.
  • Ileal loop urinary diversion
  • Bladder-drained pancreas transplant
  • Toluene inhalation

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Management Pearls

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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Last Reviewed: 03/20/2018
Last Updated: 03/20/2018
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Distal renal tubular acidosis
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Distal renal tubular acidosis (Adult) : Dehydration, Hypercalciuria, Metabolic acidosis, Muscle weakness, Myalgia, K decreased
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