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Amino acid abnormalities in infants with extrahepatic biliary atresia and cirrhosis
S A Weisdorf1, D K Freese, J J Fath
1Department of Pediatrics, University of Minnesota, Minneapolis.
Insights
Children with biliary atresia and cirrhosis show altered plasma amino acids, indicating impaired liver function. Dietary protein adjustments may improve amino acid levels before liver transplantation.
Area of Science:
- Biochemistry
- Pediatric Gastroenterology
- Hepatology
Background:
- Extrahepatic biliary atresia is a severe liver disease in children.
- Cirrhosis in children can lead to significant metabolic disturbances.
- Amino acid metabolism is primarily regulated by the liver.
Purpose of the Study:
- To analyze fasting plasma amino acid profiles in children with biliary atresia and cirrhosis.
- To compare these profiles with those of healthy children.
- To understand the implications of altered amino acids for liver function and transplantation.
Main Methods:
- Fasting plasma amino acids were measured in 26 children (6 months–5 years) with biliary atresia and cirrhosis.
- Values were compared to 95 normal control children (4 months–12 years).
- Statistical analysis was performed on amino acid concentrations and ratios.
Main Results:
- Children with cirrhosis exhibited elevated total free plasma amino acids, suggesting reduced hepatic amino acid metabolism.
- The molar ratio of branched-chain to aromatic amino acids was significantly depressed.
- Methionine levels were markedly elevated, while taurine concentrations were significantly decreased.
Conclusions:
- Altered plasma amino acid profiles in pediatric cirrhosis reflect impaired liver function.
- Nutritional strategies, including dietary protein manipulation, may be beneficial.
- Optimizing amino acid profiles prior to orthotopic liver transplantation could enhance outcomes.
Abstract:
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.