Biliary atresia: A comprehensive review

J Autoimmun. 2016 Sep:73:1-9. doi: 10.1016/j.jaut.2016.06.005. Epub 2016 Jun 23.


Biliary atresia presents as an obliterative cholangiopathy with neonatal jaundice and pale stools. The disease exhibits aetiological heterogeneity with a multiplicity of potential causative factors, both developmental and environmental. A number of clinical variants making up a minority of all cases can be defined relatively precisely which match suggested aetiology better although in most it still remains speculative. These include the syndromic form (BASM), the cystic form and those associated with CMV IgM antibodies. We review not only the clinical evidence for a developmental or an immune-mediated aetiology perhaps triggered by perinatal viral exposure but also several other recently suggested concepts such as microchimerism, gene susceptibility and environmental toxins.

Keywords: Biliary atresia; Immune response; Inflammation; Kasai portoenterostomy; Microchimerism.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Bile Ducts / growth & development*
  • Biliary Atresia / diagnosis
  • Biliary Atresia / epidemiology*
  • Biliary Atresia / etiology*
  • Biliary Atresia / surgery
  • Biopsy
  • Chimerism*
  • Cytomegalovirus / immunology
  • Cytomegalovirus / isolation & purification
  • Environmental Exposure / adverse effects*
  • Genetic Predisposition to Disease
  • Humans
  • Immunity, Humoral*
  • Immunoglobulin M / immunology
  • Incidence
  • Infant
  • Liver / pathology
  • Liver Function Tests
  • T-Lymphocytes / immunology
  • Ultrasonography


  • Immunoglobulin M