Biliary atresia: From pathology to treatment

Abstract

Biliary atresia is a rare disease of infancy with an estimated incidence in Europe of 1 in 15,000-19,000. The disease onset is confined to the neonatal period, and there is no equivalent liver disease outside of this period. It affects all races and ethnic groups, although black mothers are 2.5 times more likely to have a baby with biliary atresia than other ethnic groups. The pathogenesis of biliary atresia is unknown; and research to further understand the underlying mechanisms of its development is disadvantaged by the rarity of disease and lack of correlation between human and animal models. A remarkable medical achievement has been the Kasai portoenterostomy surgical procedure, which along with liver transplantation (when necessary) has increased survival into adulthood with a good quality of life.