Biliary atresia: cellular dynamics and immune dysregulation

Semin Pediatr Surg. 2012 Aug;21(3):192-200. doi: 10.1053/j.sempedsurg.2012.05.003.


The cause of biliary atresia is unknown; in the past few decades, the majority of investigations related to its pathogenesis have centered on viral infections and immunity. The acquired or perinatal form of biliary atresia entails a progressive inflammatory injury of bile ducts, leading to fibrosis and obliteration of both the extrahepatic and intrahepatic bile ducts. Theories of pathogenesis include viral infection, chronic inflammatory or autoimmune-mediated bile duct injury, and abnormalities in bile duct development. This review will focus solely on human studies pertaining to a potential viral trigger of bile duct injury at diagnosis and provide insight into the interplay of the innate and adaptive immune responses in the pathogenesis of disease.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Adaptive Immunity*
  • Biliary Atresia* / immunology
  • Biliary Atresia* / virology
  • Cytomegalovirus Infections / complications*
  • Cytomegalovirus Infections / immunology
  • Humans
  • Immunity, Cellular
  • Immunity, Humoral
  • Immunity, Innate*
  • Reoviridae Infections / complications*
  • Reoviridae Infections / immunology
  • Rotavirus Infections / complications*
  • Rotavirus Infections / immunology