Orphanet: Autosomal dominant tubulointerstitial kidney disease

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Autosomal dominant tubulointerstitial kidney disease

Disease definition

A chronic tubulointerstitial nephropathy, which belongs, together with nephronophthisis (NPH), to a heterogeneous group of inherited tubulo-interstitial nephritis, termed NPH-MCKD complex.


Classification level: Disorder
  • Synonym(s):
    • ADTKD
    • Autosomal dominant medullary cystic kidney disease
    • MCKD
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Infancy, Childhood, Adolescent, Adult
  • ICD-10: Q61.5
  • OMIM: 174000  603860  609886
  • UMLS: -
  • MeSH: -
  • GARD: 10801
  • MedDRA: -

Detailed information


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