Abstract
Feeding formulations which are enriched in branch chain amino acids (BCAA) and depleted in aromatic amino acids (AAA) and methionine (Met) are used in therapy of adults with hepatic encephalopathy (HE). These formulations are also being used in children with severe liver disease. In order to document a rationale for such therapy we examined amino acid profiles in 20 infants with EBA and cirrhosis who were candidates for liver transplantation, none of whom had clinical signs of HE. Similar to reports in adult patients, we found that our patients had a low BCAA/AAA. While this ratio is lower (2.83 vs. 3.45) for normal children than for normal adults our patients mean BCAA/AAA was 1.02 ± 0.61). This was due both to decreases in BCAA (mean total BCAA was 24.57 um/dl for EBA vs. 42.16 for normals) and increases in AAA (24.51 vs. 14.93). Mean Met levels were moderately elevated in 16 patients (5.27 vs. 2.17) with striking increases in 4 patients considered endstage on the basis of bilirubin level and coagulopathy. While proline and threonine have been reported to be elevated in adults, this was not true for these children. Taurine levels were also normal. We conclude that there is justification for use of BCAA enriched, AAA and Met depleted formulations in children with EBA in the absence of HE, but that because of individual variation in disease-related disorders of amino acid metabolism such therapy requires close monitoring of the amino acid profile in these patients.
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Weisdorf, S., Fath, J., Freese, D. et al. AMINO ACIDS IN INFANTS WITH NON-CORRECTED EXTRA-HEPATIC BILIARY ATRESIA (EBA). Pediatr Res 18 (Suppl 4), 217 (1984). https://doi.org/10.1203/00006450-198404001-00746
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DOI: https://doi.org/10.1203/00006450-198404001-00746
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