Q. What are the clinical features of conjugated hyperbilirubinemia in infants?
A. As the liver disease progresses the clinical picture becomes overt. The classical trial of jaundice, off-white stools and off-white urine become pronounced and the child develops hepatosplenomegaly. Ascites may or may not be present. Curiously, many patients with biliary atresia have an open umbilical ring with an umbilical hernia. The advanced liver disease results in bleeding manifestations and multiorgan dysfunction and eventually in liver cell failure. Liver cell failure may precipitate hepatic encephalopathy with a rising serum ammonia level.

In addition to this generalized clinical picture, certain specific clinical features, which are associated with different disorders are summarized in Table 1.