The effects of branched-chain amino acid-enriched elemental diet in patients with biliary atresia

Supplemental administrations of ED-H, branched-chain amino acid (BCAA)-enriched elemental diet for hepatic disorder, were performed in 10 postoperative biliary atresia (BA) patients. These patients were exhibiting, more or less, cirrhotic changes. The duration of ED-H administration ranged from 7 months to 3 years. initially, these patients showed lowered molar ratios, Val+Leu+Ile/Phe+Tyr, in plasma aminograms with decreased levels of plasma rapid-turnover proteins. ED-H administration induced a significant increase in molar ratio as well as increases in plasma prealbumin and retinol-binding protein levels. With an improved general status, such as activity level and play performance, there were significant increases both in weight for age and weight for height. No particular deleterious effects were observed throughout the period of ED-H administration. In conclusion, supplemental ED-H administration can be performed safely with an efficacy in postoperative BA patients who need metabolic/nutritional supports due to abnormal liver functions.