Aetiology of biliary atresia: what is actually known?

Orphanet J Rare Dis. 2013 Aug 29;8:128. doi: 10.1186/1750-1172-8-128.


Biliary atresia (BA) is a rare disease of unknown etiology and unpredictable outcome, even when there has been timely diagnosis and exemplary surgery. It has been the commonest indication for liver transplantation during childhood for the past 20 years. Hence much clinical and basic research has been directed at elucidating the origin and pathology of BA. This review summarizes the current clinical variations of BA in humans, its occasional appearance in animals and its various manifestations in the laboratory as an experimental model.

Publication types

  • Review

MeSH terms

  • Animals
  • Biliary Atresia / diagnosis*
  • Biliary Atresia / etiology*
  • Biliary Atresia / immunology
  • Disease Models, Animal
  • Humans
  • Liver Transplantation / adverse effects