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Distal renal tubular acidosis

Disease definition

A rare genetic or acquired renal tubular disease characterized by hyperchloremic metabolic acidosis. Primary distal renal tubular acidosis (dRTA) is often associated with hypokalemia, other forms with hypokalemia, hyperkalemia or normokalemia.


Classification level: Disorder
  • Synonym(s):
    • Classic RTA
    • Familial distal primary acidosis
    • Renal tubular acidosis type 1
    • dRTA
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant or Autosomal recessive or Not applicable 
  • Age of onset: All ages
  • ICD-10: N25.8
  • ICD-11: GB90.44
  • OMIM: 179800  267300  602722  611590
  • UMLS: C1704380
  • MeSH: -
  • GARD: 4667
  • MedDRA: 10045224

Detailed information


  • Clinical practice guidelines
  • English (2021) - Nephrol Dial Transplant ERN

Disease review articles

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.