Biliary atresia: East versus west

Semin Pediatr Surg. 2020 Aug;29(4):150950. doi: 10.1016/j.sempedsurg.2020.150950. Epub 2020 Jul 23.

Abstract

Biliary atresia (BA) is a common cause of surgical jaundice during the neonatal period. It is currently considered as a spectrum of diseases with a common final pathology characterized by obliteration of the extrahepatic biliary tract and the absence of normally branching intrahepatic ducts. Though it is a global disease that can be found in all ethnicities there are some clear differences between BA arising in the East and the West. This is likely to be related to different genetic, environmental and cultural factors. BA is more frequently found in Far Eastern infants (both Chinese and Japanese) though the syndromic associations are much less common. Many Eastern countries have national screening programmes not seen in the West possibly due to debate over its cost effectiveness in countries where incidence is low. Kasai portoenterostomy (KPE) is considered as the primary treatment of BA but its outcome still remains unsatisfactory across the region. Given the complexity of BA, it is unlikely that strategic advances could be made by the sole effort of individual countries and we believe that collaboration between the East and West is the way forward.

Keywords: Biliary atresia; Biliary atresia splenic malformation syndrome; Kasai portoenterostomy; Liver transplant.

Publication types

  • Review

MeSH terms

  • Biliary Atresia* / diagnosis
  • Biliary Atresia* / ethnology
  • Biliary Atresia* / surgery
  • Humans
  • Infant, Newborn
  • Infant, Newborn, Diseases* / diagnosis
  • Infant, Newborn, Diseases* / ethnology
  • Infant, Newborn, Diseases* / surgery
  • Liver Transplantation* / statistics & numerical data
  • Portoenterostomy, Hepatic* / statistics & numerical data