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Biliary atresia is a congenitally acquired condition in which the biliary tree become progressively sclerosed and occluded. The main intrahepatic ducts become obliterated and death occurs in 98% of individuals by two years old if untreated.
The cause may be a congenital or perinatal infection, for example with reoviruses.
It occurs approximately once in 15 000 live births in the UK (1)
Approximately 20% of patients have other congenital abnormalities (2).