The Plasma Amino Acid Profile and its Relationships to Standard Quantities of Liver Function in Infants and Children with Extrahepatic Biliary Atresia and Preterminal Liver Cirrhosis

The Plasma Amino Acid Profile and its Relationships to Standard Quantities of Liver Function in... 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 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Clinical Chemistry and Laboratory Medicine de Gruyter

The Plasma Amino Acid Profile and its Relationships to Standard Quantities of Liver Function in Infants and Children with Extrahepatic Biliary Atresia and Preterminal Liver Cirrhosis

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Publisher
de Gruyter
Copyright
Copyright © 2009 Walter de Gruyter
ISSN
1434-6621
eISSN
1437-4331
DOI
10.1515/cclm.1993.31.4.197
Publisher site
See Article on Publisher Site

Abstract

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

Journal

Clinical Chemistry and Laboratory Medicinede Gruyter

Published: Jan 1, 1993

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