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Isolated biliary atresia

Disease definition

A rare, biliary tract disease characterized by progressive obliterative cholangiopathy of the intra- and extrahepatic bile ducts, occuring in the embryonic/ perinatal period, leading to severe and persistent neonatal jaundice and acholic stool.


Classification level: Disorder
  • Synonym(s):
    • Isolated atresia of bile ducts
    • Non-syndromic biliary atresia
  • Prevalence: 1-9 / 100 000
  • Inheritance: Multigenic/multifactorial 
  • Age of onset: Infancy, Neonatal, Antenatal
  • ICD-10: Q44.2
  • ICD-11: LB20.21
  • OMIM: 210500
  • UMLS: C0005411
  • MeSH: D001656
  • GARD: 12010
  • MedDRA: 10003650

Detailed information


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