The need of essential amino acids in children: An evaluation based on the intake of phenylalanine, tyrosine, leucine, isoleucine, and valine in children with phenylketonuria, tyrosine amino transferase defect, and maple syrup urine disease
1980
Kindt, Elisabeth | Halvorsen, Sverre
To evaluate the need for live amino acids to maintain normal body growth, the diets of four children (ages 3 to 6 years, 2 males, 2 females) with blocks in the metabolism of these amino acids are explored. Two children had phenylketonuria (PKU), one child had tyrosine aminotransferase defect (TATD), and the last maple syrup urine disease (MSUD). By registering the intake of phenylalanine in the PKU children, the intake of phenylalanine and tyrosine in the TATD child and isoleucine, leucine and valine in the MSUD child, the needs for these amino acids in growing children was investigated. Sufficient intake, and the difference between sufficient intake and requirement, are discussed, with comparisons to former studies on requirements.
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