extrahepatic biliary atresia

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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)

  • more common in females
  • more common in Asians

Approximately 20% of patients have other congenital abnormalities (2).

Reference:

  • Kelly DA, Davenport M; Current management of biliary atresia. Arch Dis Child. 2007 Dec;92(12):1132-5.
  • Hartley JL, Davenport M, Kelly DA; Biliary atresia. Lancet. 2009 Nov 14;374(9702):1704-13.

Last reviewed 01/2018

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