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Congenital disorders of the liver, biliary tract, and pancreas 

Congenital disorders of the liver, biliary tract, and pancreas
Chapter:
Congenital disorders of the liver, biliary tract, and pancreas
Author(s):

J.A. Summerfield

DOI:
10.1093/med/9780199204854.003.1525

July 30, 2015: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

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date: 22 October 2017

Congenital disorders of the liver, biliary tract, and pancreas are rare or very rare.

Biliary atresias present with cholestatic jaundice starting after the first 2 weeks of life and may eventually cause biliary cirrhosis and liver failure. Prognosis depends on the type of atresia: (1) intrahepatic—both nonsyndromic (with cirrhosis usually developing in late childhood, and fatal without liver transplantation) and syndromic (e.g. Alagille’s syndrome, due to mutation in the ...

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