Chapter

Congenital disorders of the liver, biliary tract, and pancreas

J.A. Summerfield

in Oxford Textbook of Medicine

Published on behalf of Oxford University Press

Published in print May 2010 | ISBN: 9780199204854
Published online May 2010 | e-ISBN: 9780199570973 | DOI: http://dx.doi.org/10.1093/med/9780199204854.003.1525

Series: Oxford Textbooks

Congenital disorders of the liver, biliary tract, and pancreas

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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 ...

Chapter.  2872 words.  Illustrated.

Subjects: Gastroenterology

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