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Outcome in adulthood of biliary atresia: A study of 63 patients who survived for over 20 years with their native liver
Author(s) -
Lykavieris Panayotis,
Chardot Christophe,
Sokhn Maroun,
Gauthier Frédéric,
Valayer Jacques,
Bernard Olivier
Publication year - 2005
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.20547
Subject(s) - biliary atresia , medicine , liver transplantation , hepatology , porta hepatis , atresia , biliary cirrhosis , gastroenterology , gallstones , cirrhosis , surgery , transplantation , disease , autoimmune disease
To define the long‐term prognosis of children undergoing the Kasai operation for biliary atresia, a retrospective study was undertaken comprising 271 patients operated between 1968 and 1983. Twenty years after surgery, 63 (23%) were alive with their native liver. Serum bilirubin was normal in 21 of these patients, 12 also had normal serum aminotransferase and γ‐glutamyltransferase activities, all but 2 had signs of cirrhosis, 44 had signs of portal hypertension, 19 had late bacterial cholangitis, and 6 had gallstones. Seven female patients gave birth to 9 children, and 3 male patients fathered 6 children. After age 20, 2 patients died of liver failure and 14 underwent or are awaiting liver transplantation. Twenty‐year survival with native liver was significantly better in children with biliary atresia restricted to the hepatic ducts or with cysts at the porta hepatis. In conclusion , in the long term, less than 18% of infants with biliary atresia who are treated with corrective surgery may avoid liver transplantation, but even these patients require assiduous lifelong care. (H EPATOLOGY 2005;41:366–371.)