Amino acid abnormalities in infants with extrahepatic biliary atresia and cirrhosis

We measured fasting plasma amino acids in 26 children aged 6 months to 5 years with extrahepatic biliary atresia and cirrhosis and compared them with fasting values in 95 normal control children aged 4 months to 12 years. We found that the cirrhotic children had elevations of total free plasma amino acids implying reduced hepatic metabolism of amino acids and that the molar ratio of the branched chain amino acids (isoleucine, leucine, and valine) to the aromatic amino acids (phenylalanine and tyrosine) was significantly depressed. Methionine was also markedly elevated, and taurine concentrations were significantly decreased. Manipulation of the amino acid distribution in dietary protein to normalize plasma amino acids prior to orthotopic hepatic transplantation may be helpful in improving amino acid utilization.