Introduction Extrahepatic biliary atresia is a congenital structural defect of the hepatic biliary tree. Its frequency is estimated to be 1: 8000 to 1 :15000. It is responsible for 75 to 80% of the cases of conjugated hyperbilirubinaemia in children (1, 2). In the natural course of extrahepatic biliary atresia, 70 to 90% of cases die within 2-3 years (3). Affected patients may benefit, for a time, from hepatoportoenterostomy (4), but most eventually develop biliary cirrhosis and progressive liver failure (5, 6). The only definitive therapy appears to be liver transplantation (3, 6). Lack of Eur. J. Clin. Chem. Clin. Biochem. / Vol. 31,1993 / No. 4 donor liver and low body weight in recipients are f probl*TM> f that ,f^ and ^imal ^ tional care of the chronically malnourished transplan'at'On can*dates are vital (13). Single laboratory fmdmgs , TMU "TM?* not be indlcatlve °f. "*Pend'ng mmal llver failure m ext ^ ^epatic biliary atresia ^ '" In adult cirrhotics, plasma concentrations of the aromatic amino acids are increased and those of branched chain amino acids are decreased. Methionine can be markedly elevated (7-9). The Fischer index (the ratio of the sum of the concentrations of Byrd et
Clinical Chemistry and Laboratory Medicine – de Gruyter
Published: Jan 1, 1993
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