clinical features

Last reviewed 01/2023

About one-third of patients are jaundiced at birth. In all, jaundice is present by the end of the first week and deepens progressively.

Other features include:

  • pale stools, indicating obstruction
  • dark urine
  • pruritus
  • often hepatomegaly and splenomegaly

In untreated, or unsuccessfully treated biliary atresia there may be clubbing and skin xanthomas, a result of the raised serum cholesterol.

The occasional patient may be thriving and be without hepatomegaly but will still require prompt diagnosis if biliary cirrhosis is to be avoided.