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Distal renal tubular acidosis

Disease definition

Distal renal tubular acidosis (dRTA) is a disorder of impaired net acid secretion by the distal tubule characterized by hyperchloremic metabolic acidosis. The classic form is often associated with hypokalemia whereas other forms of acquired dRTA may be associated with hypokalemia, hyperkalemia or normokalemia.

ORPHA:18

  • 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
  • OMIM: 179800  267300  602722  611590
  • UMLS: C0259810  C1704380
  • MeSH: -
  • GARD: 4667
  • MedDRA: 10045224

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