Which congenital condition may lead to biliary atresia?

Biliary atresia is a serious congenital condition that results in the obstruction or absence of the bile ducts, preventing bile from being properly drained from the liver into the intestine. Alagille syndrome is one of the congenital syndromes associated with biliary atresia.

Alagille syndrome is characterized by a triad of clinical manifestations: cholestasis (which can progress to biliary atresia), cardiac defects, and skeletal abnormalities. The cholestasis in Alagille syndrome can lead to bile duct paucity and the development of biliary atresia due to the abnormal morphology of the bile ducts. Children with Alagille syndrome often present with jaundice and other liver-related symptoms early in life, which can be attributed to the complications arising from these biliary issues.

Other congenital conditions like Down syndrome, Turcot syndrome, and Beckwith-Wiedemann syndrome do not have the same direct association with biliary atresia as Alagille syndrome does. While they may present a range of other developmental challenges and malformations, the primary link to biliary atresia is most clearly established with Alagille syndrome due to the specific anatomical and functional liver abnormalities it causes.