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Isolated polycystic liver disease

Disease definition

Isolated polycystic liver disease (PCLD) is a genetic disorder characterized by the appearance of numerous cysts spread throughout the liver and that in most cases is described as autosomal dominant polycystic liver disease (ADPCLD).

ORPHA:2924

  • Synonym(s):
    • ADPCLD
    • Autosomal dominant polycystic liver disease
    • PCLD
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant or Not applicable 
  • Age of onset: Adult
  • ICD-10: Q44.6
  • OMIM: 174050  617004
  • UMLS: C0158683
  • MeSH: C536330
  • GARD: 9457
  • MedDRA: 10010427  10048834

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