Reduction of cerebrospinal fluid phenylalanine after oral administration of valine, isoleucine, and leucine.
Pediatr Res
; 16(9): 751-5, 1982 Sep.
Article
en En
| MEDLINE
| ID: mdl-7133808
ABSTRACT
A supplement of the branched chain amino acids, valine, isoleucine, and leucine (VIL) was administered orally to patients with phenylketonuria, either together with unrestricted diet of natural protein or with a low phenylalanine diet. The VIL supplement brought about a significant reduction of the cerebrospinal fluid-serum ratio of phenylalanine from a mean value of 0.254 without VIL to 0.204 with VIL. The reduction varied from 15-40% (mean 21%). Concentrations of glycine, lysine, methionine, threonine, tryptophan, and tyrosine were within normal limits in serum and cerebrospinal fluid of infants with phenylketonuria. No amino acid imbalance was created by the supplement and no adverse effects from VIL were observed.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Fenilalanina
/
Fenilcetonurias
/
Aminoácidos de Cadena Ramificada
Límite:
Adult
/
Child
/
Female
/
Humans
/
Infant
/
Newborn
Idioma:
En
Revista:
Pediatr Res
Año:
1982
Tipo del documento:
Article