The Amino Acid Compositions of Formula for Children with Inherited Metabolic Disorder

Ryu, Kyung Hwa; Kim, Jong Soo; Lee, Eun Ha; Kwon, Il Sun; Hahn, Si Houn

Abstract

PURPOSE: This study aimed to determine the amino acids composition, safety and efficacy of formulas recently developed by Korean dairy companies for children with inherited metabolic disorder.
METHODS
The determination of amino acids concentration was performed on eight Korean formula samples. The samples were hydrolyzed with 6N HCL or performic acid and analyzed by amino acid analyzer.
RESULTS
No phenylalanine, methionine or leucine was observed in PKU-1 and PKU-2 Formulas, Methionine-Free and Leucine-Free Formula, respectively. BCAA-Free Formula was free from leucine, isoleucine, and valine and MPA Formula did not contain methionine and valine. Protein-Free formula did not include any amino acids. UCD Formula contained arginine but was free of alanine, aspartic acid, glutamic acid, glycine, histidine, proline and serine. Methionine-Free Formula contained higher amounts of cystine and tyrosine was higher in PKU-1 and 2 Formulas. The amounts of isoleucine and threonine were minimal in MPA Formula.
CONCLUSION
This study confirmed that the eight special formulas, developed for the first time by a Korean dairy company for children with inherited metabolic disorder contain appropriate amino acids with proper contents. Both the total amino acid amounts and specific amino acid concentrations of the formulas were appropriate for related diseases, which could be used safely by the patients with inherited metabolic disorder. For UCD Formula that contains arginine, we suggest that arginine be removed from the formula in order to use for any urea cycle defect patients before the specific diagnosis is made.