| The Amino Acid Compositions of Formula for Children with Inherited Metabolic Disorder |
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Kyung Hwa Ryu, Jong Soo Kim, Eun Ha Lee, Il Sun Kwon, Si Houn Hahn |
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Department of Pediatrics, School of M edicine, Ajou University, Suwon, Korea |
| 유전성 대사이상질환 아동을 위한 특수분유의 아미노산 분석에 관한 연구 |
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유경화, 김종수, 이은하, 권일선, 한시훈 |
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아주대학교 의과대학 소아과학교실 |
Correspondence:
Si Houn Hahn, Email: omik@madang.ajou.ac.
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| 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. |
| Key Words:
Formula, Amino acid analysis, Metabolic disorder |
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