Background: Plasma amino acid patterns and the therapeutic benefits of amino acid supplements are not examined well in postoperative patients with biliary atresia (BA). This study aimed to investigate profiles of the amino acid molar ratio, the Fischer molar ratio (FR, valine + leucine + isoleucine/phenylalanine + tyrosine), and relationships with other nutritional parameters and to assess the efficacy of enteral nutrient preparations for hepatic failure in patients with BA.
Methods: In study 1 profiles of FR were analyzed in 24 patients with BA (aged 3 to 12 years) in the postoperative period and compared with liver function tests and anthropometric measures. In study 2 10 patients with BA with a FR < 2.0 were begun on a dietary regimen consisting of an ordinary diet supplemented with a branched-chain amino acid-enriched elemental diet.
Results: Study 1: In jaundiced patients with total bilirubin levels > 2.0 mg/dL the FR remained below 2.0 throughout the period of observation, in contrast with nonjaundiced patients. The FR was closely related to the levels of serum albumin and plasma rapid turnover proteins and anthropometric measures as well as biochemical data reflecting intrinsic liver function. Study 2: A significant increase in rapid turnover proteins and improvement of general status were noted concurrently with an increase of the FR.
Conclusions: The FR indicates a combination of nutritional status and intrinsic liver function in post-operative BA patients. The FR is a useful parameter in conducting nutritive therapy with a branched-chain amino acid-enriched elemental diet in those patients.