Thelansis

  Biliary atresia (BA) is a progressive, idiopathic, necroinflammatory process initially involving a segment or all of the extrahepatic biliary tree. As the disease advances, the extrahepatic bile duct lumen is obliterated, and bile flow is obstructed, resulting in cholestasis and chronic liver damage. With time, the intrahepatic biliary system becomes involved. Two different forms of BA are recognized. The more common of the two forms is the perinatal or postnatal form, which accounts for most cases. These children typically appear healthy at birth; their weights are average, and they have pigmented stools. Jaundice develops at some variable interval postnatally; typical timing is between 2 to 6 weeks of age. Because of the frequency of breast milk jaundice, the diagnosis can be missed unless a fractionated bilirubin is obtained. The less common presentation is the embryonic or fetal form, which occurs in 10% to 35% of cases. These children are cholestatic at birth; 10% to 20% h...